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TEXT-BOOK OF OPHTHALMOLOGY
TEXT-BOOK OF
OPHTHALMIOLOGY
By Sir W. STEWART DUKE-ELDER
M.A., D.Sc.(St.And.), Ph.D. (Lond.), M.D., Ch.B., F.R.C.S.
VOL. I
THE DEVELOPMENT, FORM AND FUNCTION OF
THE VISUAL APPARATUS
Crown quarto, xxix and 1124 pages, with 1022 illustrations,
including 7 coloured plates
VOL. II
CLINICAL METHODS OF EXAMINATION, CON-
GENITAL AND DEVELOPMENTAL ANOMALIES,
GENERAL PATHOLOGICAL AND THERAPEUTIC
CONSIDERATIONS, DISEASES OF THE OUTER
EYE
Crown quarto, 1000 pages, with 742 illustrations, including
24 coloured plates
British Medical Journal : This first volume is in our judgment
a great book. It is great by reason of its bulk, for it exceeds a
thousand pages; it is great in its conception, and also great in
its achievement. The author may well be congratulated upon
such a piece of work—upon his ability to produce it and his
industry in producing it. Whichever way this book is examined,
its high standard is confirmed.
The second volume is fully up to the high standard set by
the first.
The American Journal of Ophthalmology : For the ground it
covers, this book has practically the value of a whole library.
It should be found in every library that purports to contain
books on ophthalmology.
An examination of Duke-Elder's second volume affords
English-speaking ophthalmologists new reasons for rejoicing.
British Journal of Ophthalmology : It goes without saying
that a book of this kind should be the indispensable possession
of the ophthalmologist who values a knowledge of his subject,
and more particularly of one whose duty it is to instruct students.
Finally, it seems almost superfluous to say that we congratulate
the author on his achievement ; we can only hope that its
production will be as great a source of satisfaction to him as it
will be of inspiration to the world of English-speaking
Ophthalmologists.
Journal of the American Medical Association : This text-book
is one of the best that has ever appeared in English.
Anglo-Saxon ophthalmology owes a real debt of gratitude
to Duke-Elder, not only for his brilliant research work but even
more for his text-book.
TEXT-BOOK OF

OPHTHALMOLOGY
BY
Sir W. STEWART DUKE-ELDER
Surgeon Oculist to H.M. The King ;
Colonel A.M.S. Consulting Ophthalmic Surgeon to the British Army ;
Ophthalmic Surgeon, St. George’s Hospital;
Honorary Research Associate, University College, London ;
Lecturer in Ophthalmology, St. George’s Hospital Medical
School, University of London, etc.
VOL. III
DISEASES OF THE INNER EYE
WITH 1140 ILLUSTRATIONS, INCLUDING
164 IN COLOUR
ST. LOUIS
THE C. V. MOSBY COMPANY
1941
Medical Library
A’z
#4
, D88
/?34ſ
V, 3
All rights reserved
MADE AND PRINTED IN GREAT BRITAIN
º>--(-
2.-*
º; :
ºw
ACKNOWLEDGMENTS
As in the previous volumes of this book, I have trespassed upon the
kindness of many of my colleagues, particularly in this country and America,
in obtaining the use of the great majority of the illustrations used in these
pages. Each is acknowledged as it occurs ; but it is a pleasure to express
my indebtedness to them and to the many journals which have so liberally
supplied me with permission for reproduction. And Once again I have
added to my great debt to Mr. R. R. James, who has so generously shared
s with me the labour of proof reading.
STEWART DUKE-ELDER.
63 HARLEY STREET,
LONDON, W.l.
IMPORTANT NOTE
In references to Volume I the first figures refer to the first edition (1932),
the figures following in brackets to the Second Impression (1938).
CONTENTS
WOLUME III
DISEASES OF THE INNER EYE
Section XIII
The Uveal Tract
The Retina
The Optic Nerve
The Lens .*
The Vitreous Body
Glaucoma and Hypotony
Intra-ocular Parasites
CHAPTER XXXV
DISEASES OF THE UVEAL TRACT
I. General Considerations
The Healing of Wounds tº & º * > . . e
1. Wounds of the iris, 2098. 2. Wounds of the ciliary body,
2099. 3. Wounds of the choroid, 2100.
II. Disturbances of the Circulation
1. Hyperaemia . e e
2. Embolism and Thrombosis
3. Haemorrhages. ſº * sº e
Disciform Degeneration of the Macula tº
(a) Senile Disciform Degeneration of the Macula
(b) Juvenile Disciform Degeneration of the Macula
III. Inflammation of the Uveal Tract : Uveitis
A. General Considerations º e
The General AEtiology of Uveitis
1. Exogenous Infections
2. Secondary Infections
3. Endogenous Infections . * e & e
(A) Inflammations due to Organismal Metastases
(B) Inflammations due presumably to Organismal
Allergy or Toxic Influences te tº
Teeth 2144. Tonsils, 2145. The nasal
sinuses, 2145. The intestinal tract, 2146.
The genito-urinary tract, 2146.
(C) Uveitis Associated with Generalized Infectious
Diseases.
PAGE
2097
2098
2101
2104
2105
2108
2115
2116
2124
2126
2126
2126
2127
2130
2136
2136
2140
2149
ix
CONTENTS
III. Inflammation of the Uveal Tract : Uveitis—contal.
A. General Considerations—contà.
(C) Uveitis Associated with Generalized Infectious
Diseases—contal.
1. The acute exanthems, 2149. 2. Acute
intestinal infections, 2150. 3. Blood infec-
tions, 2151. 4. Respiratory fevers, 2151.
5. Cerebro-spinal infections, 2151. 6. Infec-
tions of the salivary glands, 2152.
(D) Uveitis Associated with Diseases of the Skin.
(E) Uveitis due to Metabolic Conditions º te
1. Rheumatism, 2155. 2. Gout, 2155.
3. Diabetes, 2156. 4. Auto-intoxication, 2156.
5. Renal disease, 2157.
The General Pathology of Uveitis
1. Acute Purulent Inflammations .
2. Sub-acute and Chronic Uveitis .
The General Symptomatology of Uveitis . g te
Iritis, 2197. Cyclitis, 2200. Choroiditis, 2203.
The General Treatment of Uveitis g º
1. AEtiological Treatment
2. Local Treatment e
(a) Medical Treatment
(b) Surgical Treatment.
B. Special Types of Uveitis.
I. Acute Suppurative Inflammations .
1. Panophthalmitis . {e
Gas Gangrene Panophthalmitis
PAGE
2153
2154
2158
21.59
2177
2197
2206
2206
2208
2212
2216
2221
2. Suppurative Irido-cyclitis . e 4.
Recurrent Irido-cyclitis with hypopyon ©
3. Suppurative Choroiditis (Suppurative End-
ophthalmitis) © e tº tº de e
II. Exudative Inflammations
A. Non-specific Types º
1. Iritis and Irido-cyclitis *
i. Simple Acute Iritis . º
ii. Sub-acute and Recurrent Iritis
iii. Acute Irido-cyclitis
iv. Chronic Cyclitis .
2. Exudative Choroiditis.
i. Diffuse Choroiditis
ii. Disseminated Choroiditis º
iii. Circumscribed Exudative Choroiditis
3. Exudative Uveitis (Exudative Endophthalmitis)
i. Endogenous Infections. & wº º
ii. Uveitis with Haemorrhages and Neoplasms
iii. Uveitis with Retinal Detachments
iv. Atrophic Uveitis. tº gº
v. Traumatic Uveitis
222]
222I
2224
2225
2227
2228
2231
2231
2231
2232
2233
2234
2236
2237
2238
2239
2241
2251
2251
2252
2252
2253
2253
CONTENTS
xi
III. Inflammation of Uveal Tract : Uveitis—conta.
B. Special Types of Uveitis—conta.
II. Exudative Inflammations—contal.
B. Specific Infections
1. Herpes tº º
i. Herpes Simplex
ii. Herpes Zoster
2. Gonorrhoea te
i. Simple Iritis * tº we
ii. Exudative Irido-cyclitis * © e * >
iii. Plastic Irido-cyclitis
3. Syphilis . © e
A. Acquired Syphilis.
i. Roseola of the Iris * tº
ii. Syphilitic Irido-cyclitis . * •
(a) Secondary Syphilitic Irido-cyclitis
(b) Tertiary Syphilitic Irido-cyclitis
(c) The Jarisch-Herxhiemer Reaction
(d) Recurrent Syphilitic Irido-cyclitis .
iii. Syphilitic Chorio-retinitis tº e
(a) Diffuse Choroiditis g tº ©
(b) Disseminated Syphilitic Chorio-
retinitis * • e tº
iv. Syphilomata.
(a) Papules
(b) Gummata
B. Congenital Syphilis
i. Acute Irido-cyclitis
ii. Anterior Uveitis
iii. Chorio-retinitis
Pathology . tº & *
Syphilitic Atrophy of the Iris © , º
Diagnosis, 2281. Prognosis, 2282. 'Treatment,
2282.
4. Tuberculosis * e (s e • & *
History, 2285. Incidence, 2287. Mode of infec-
tion, 2288. Experimental researches, 2289.
Clinical Manifestations, 2290.
I. Tuberculous Infections
1. Acute Miliary Tuberculosis . § sº
In the iris, 2293. In the choroid, 2294.
2. Chronic Tuberculomatous Lesions . e
(a) Chronic Miliary Tuberculomata of
the Iris . ſº te .
(b) Chronic Tuberculous Cyclitis .
(c) Tuberculous Disseminated Choroiditis
(d) Chronic Tuberculous Uveitis . .
3. Conglomerate Tubercle .
(a) In the Anterior Segment.
(b) In the Posterior Segment ).
4. Diffuse Proliferative Tuberculosis).
PAGE
2254
2254
2254
2255
2257
2258
2258
2259
2261
2262
2262
2263
2263
2264
2264
2264
2266
2267
2267
2270
2271
2272
2273
2273
2273
2273
2275
2280
2284
2293
2296
2296
2298
2303
2303
2304
2303
2306
2307
xii , CONTENTs
III. Inflammation of Uveal Tract : Uveitis—conta.
B. Special Types of Uveitis—contal.
II. Exudative Inflammations—contal.
B. Specific Infections—conta.
4. Tuberculosis—contal. PAGE
II. Diffuse Allergic Inflammations
Exudative Tuberculous Uveitis 2309
(a) Acute Plastic Type te ſe . 2310
(b) Chronic or Relapsing Type . . 2310
Prognosis, 2311. Diagnosis, 2311.
Treatment, 2315.
5. Leprosy . * * . g 2320
1. Miliary Leprotic Spots in the Iris 2320
2. Isolated Lepromata g 2321
3. Diffuse Plastic Irido-cyclitis 2321
6. Trypanosomiasis * 2323
C. Syndromes of Doubtful AEtiology 2324
1. Sympathetic Ophthalmitis tº 2324
Sympathetic Irritation e * 2324
Historical, 2325. Incidence, 2325.
Predisposing Causes . # e § e * . 2326
Perforating wounds, 2327. Operative wounds, 2328.
Perforation of a corneal ulcer, 2328. Sub-conjunctival
rupture of the sclera, 2328. Contusion, 2329. Intra-ocular
malignant melanomata, 2329. Irido-cyclitis, 2329.
AEtiological Theories ge e {º & gº 2330
(a) The Infective Theory . 2331
(b) The Allergic Theory g ge * © . 2334
Clinical picture, 2336. Pathology, 2338. Diagnosis,
2348. Prophylaxis, 2349. Treatment, 2351.
2. Uveo-Parotitis (Heerfordt's Disease) . * 2355
3. Sarcoid of Boeck 2358
4. Heterochromic Cyclitis . * } e 2360
(a) Heterochromic Cyclitis (of Fuchs) 2361
(b) Neurogenic Heterochromia ſº g * tº 2362
5. Uveitis with Alopoecia, Vitiligo, Poliosis and Dysaconsia 2364
6. Acute Diffuse Serous Choroiditis (Harada’s Disease) 2366
D. Fungus Infections (Mycoses) § * * gº 2367
Blastomycosis (Monilia) 2367
Aspergillosis 2367
E. Uveitis due to Irritants * * tº & & 2368
Ophthalmia Nodosa (Irido-cyclitis Pseudo-tuberculosa) 2368
IV. Uveal Disease in Association with General Disease 2369
1. Vascular Diseases
(a) Vascular Sclerosis . * g tº * Q tº . 2369
Senile Macular Chorio-retinal Degeneration (Senile Macular
Degeneration of Haab) 2372
(b) Peri-arteritis Nodosa 2374
2. Haemopoietic Diseases 2376
(a) Leukaemia tº 2376
(b) Chloroma * tº © * & Qº * . 2379
(c) Erythraemia (Polycythaemia: Vaquez's Disease). . 2380
CONTENTS
xiii
IV. Uveal Disease in Association with General Disease—contal.
3. Metabolic Diseases
(a) Gout . e
(b) Diabetes Mellit º º º º © º e
1. CEdema and Vesiculation of the Pigment Layer of the Iris
2. Glycogenous Degeneration . © . . . g
3. Rubeosis of the Iris
4. Lipa-mia . º
(c) Diabetes Insipidus .
(d) Renal Disease º e
Albuminuric Choroiditis.
(e) Hepatic Disease . e
V. Degenerations and Atrophies in the Uveal Tract
1. Senile Changes e
1. The Iris . º e tº e º º º
(a) Atrophy and Degeneration in the Stroma . . .
(b) Depigmentation and Proliferation of the Epithelial
Layers e g e º e c e e
2. The Ciliary Body . g tº G º & e ©
(a) Atrophy . º e
(b) Epithelial Hyperplasi
3. The Choroid . e g
2. Pathological Atrophies º
A. Secondary Atrophies e e - e
1. Post-Inflammatory Atrophy . - e e &
(a) In the iris, 2398. (b) In the ciliary body, 2401.
(c) In the choroid, 2401.
. Traumatic Atrophy
. Ischaemic Atrophy .
. Glaucomatous Atrophy
. Myopic Atrophy
. Neurogenic Atrophy º
B. Primary or Essential Atrophies º e e
1. Essential (Progressive) Atrophy of the Iris.
2. Essential (Gyrate) Atrophy of the Choroid .
3. Primary Choroidal Sclerosis e e
Diffuse Choroidal Sclerosis . º
Central Areolar Choroidal Atrophy .
Massive Peri-papillary Choroidal Sclerosis
Angioid Streaks
C. Pigmentary Disturbances
(a) Melanin e 4- º
(b) Haematogenous Pigment
(c) Siderosis º e
:
3. [Degenerations of the Uveal Tract
(a) Fatty Degeneration
Cholesterol Deposits.
(b) Hyaline Degeneration .
(c) Ossification
(d) Necrosis e º º e
1. Circulatory Disturbances Q © º
2. Toxic Disturbances
T.O.-WOL. III.
PAGE
2380
2380
2383
2383
2384
2385
2386
2386
2386
2387
2388
2389
2389
2389
2389
2392
2393
2393
2394
2396
2398
2398
2398
2402
2402
2402
2403
2403
2404
2404
2407
2409
2412
2412
2413
2413
2418
2418
2420
2420
24.21
24.21
2422
2423
2423
2429
2429
2430
xiv.
CONTENTS
VI. Cysts of the Uveal Tract .
1.
2.
3.
Congenital Cysts
Parasitic Cysts
Traumatic.Inplantation Cysts.
. Pearl Tumours
Degenerative Cysts.
4. Exudative Cysts of the Epithelial Layers .
5. t; * tº
6. Idiopathic or Spontaneous Cysts º *
(a) Spontaneous Cysts of the Iris Stroma g tº
(b) Spontaneous Cysts of the Pigmentary Epithelium
The Clinical Course, Diagnosis and Treatment of Uveal Cysts .
VII. Tumours of the Uveal Tract
I. Primary Tumours
A. Epithelial Tumours
PAGE
2431
2432
2432
2432
2433
2438
2440
2440
2441
2442
2443
. . 24.46
1. Simple Melanoma of the Pigment Layer of the Iris.
2. Epithelial Hyperplasia * & º tº
3. Epithelioma (Medullo-epitheliomata)
(a) Diktyoma.
(b) Malignant Epithelioma of the Ciliary Epithelium
4. Neuroblastoma .
5. Gliosis e
B. Mesodermal Tumours
1. Myoma
2. Angioma .
3. Myeloma .
4. Lymphomata and Lymphosarcomata .
5. Endothelioma
C. Neuro-Ectodermal Tumours
1. Neurofibroma
Diffuse Neurofibromatosis (Plexiform Neuroma).
Discrete Neurofibromata (Perineural Fibroblastomata)
2. Naevus . &
In the iris, 247 l.
In the ciliary body, 2473. In the
choroid, 2473. Treatment, 2476.
3. Malignant Melanoma . gº gº
Incidence, 2478. Heredity, 2479.
Clinical Course .
(i) Circumscribed Tumours . e º e te
In the choroid, 2481. The ciliary body, 2486.
The iris, 2488.
(ii) Diffuse or Infiltrating Melanomata gº tº º
In the choroid, 2492. In the ciliary body, 2494.
The Spread of Uveal Melanomata . . . tº
Extra-Ocular Extension * -
Intra-ocular Metastases
Systemic Metastases º
Microscopic Anatomy º *
Cytology, 2504.
Stroma, 2507. Pigment, 2507.
Changes Occurring in Tumours e ge tº *
Haemorrhages, 2507. Degenerative changes, 2508.
Necrosis, 2508.
Symptoms, 2512.
Treatment, 2516.
Dermoid
Fibrosis, 2511.
Diagnosis, 2513. Prognosis, 2514.
2446
2446
2446
2447
2449
2449
2450
2453
2453
2454
2454
2456
2461
2461
2461
2461
2466
2466
2469
24.71
2477
2480
2481
2490
2495
2495
2498
2500
2503
2507
2520
CONTENTS
XV"
VII. Tumours of the Uveal Tract—contà.
II. Secondary Tumours .
A. By Direct Extension .
1. Epithelioma
2. Sarcoma .
3. Melanoma
4. Neuro-blastoma
5. Endothelioma .
B. Metastatic Tumours .
. Carcinoma
. Hypernephroma
Sarcoma . & &
. Malignant Melanoma.
. Chorion-epithelioma .
. Testicular Adeno-carcinoma
i
VII. Detachments and Displacements of the Uveal Tract
Displacements of the Iris
Detachments of the Iris. gº e e e o *
Detachment of the Posterior Pigmented Layer from the Stroma.
Displacements of the Ciliary Body .
Detachments of the Choroid . º
CHAPTER XXXVI
DISEASES OF THE RETINA
I. General Considerations
Healing of Wounds
II. Disturbances of the Circulation.
A. Anomalies of the Blood-flow
1. Hyperaemia . tº * tº
(a) Active Hyperaemia . •
(b) Passive Hyperaemia : Cyanosis
2. Anaemia : Ischaemia. tº sº
Retinal Ischaemia after Exsanguination
– 3. Obstruction of the Retinal Arteries
(a) Angiospasm tº
(b) Embolism e & * & tº
(c) Endarteritis and Arterial Thrombosis .
Retinal Arteriolar Thrombosis .
4. Capillary Disturbances . tº
Central Angiospastic Retinopathy
— 5. Obstruction of the Retinal Veins
Venous Thrombosis : Endo-phlebitis
6. CEdema .
Macular Oedema, 2592. Central serous retinopathy, 2592.
Stellate retinopathy, 2595.
7. Retinal Haemorrhages tº &
(a) Inter-retinal haemorrhages. * - *
(b) Pre-retinal (Sub-hyaloid) Haemorrhage
PAGE
25.20
2520
2520
2522
2522
2522
2522
2522
2522
2533
2534
2534
2435
2536
2537
2537
2537
2537
2537
2537
2544
2549
255.1
2551
2552
2552
2552
2555
2556
2561
2566
2569
2572
2578
2578
2578
2578
2578
2588
2595
2595
2599
xvi - CONTENTS
II. Disturbances of the Circulation—contal.
A. Anomalies of the Blood-flow—contal.
7. Retinal Haemorrhages—conta.
(c) Vitreous Haemorrhages . * > se gº e *
Recurrent. Intra-ocular Haemorrhages in Young Adults:
Eales' Disease (Angiopathia Retinalis Juvenalis) .
Tubercle, 2602. Septic foci. 2602. Thrombo-
angiitis (Buerger's disease), 2602.
Sequelae of Retinal Haemorrhages
1. Retinitis Proliferans. tº is
2. External Haemorrhagic Retinitis (Coats)
B. Anomalies of the Blood-vessels . gº
. Tortuosity of the Blood-vessels.
:
PAGE
260]
2602
2604
2604
2610
2612
2612
2613
2613
2615
2616
26.17
2618
. Varicosities of the Veins
. Anomalous Anastomoses .
Aneurysms ſº ge ſº * tº &
Miliary Aneurysms with Retinal Degeneration .
Arterio-venous (Racemose) Aneurysm
5. New-vessel Formation
III. Inflammation of the Retina : Retinitis
A. General Pathology . e e © e tº tº º ©
Inflammatory changes, 2622. Proliferative changes, 2624.
Degenerative changes, 2628. Pigmentation, 2633.
B. Non-specific Retinal Inflammation
I. Exogenous Purulent Inflammation
II. Endogenous Primary Retinitis.
(a) Acute Metastatic Retinitis
1. Metastatic Panophthalmitis sº
2. Localized Acute Metastatic Retinitis .
(b) Peri-vasculitis
(c) Peri-arteritis Nodosa * * e tº *
(d) Toxic Exudative Retinitis (Pseudo-nephritic Retinitis
of Leber : Stellate Retinitis) .
(e) Retinitis Cachecticorum (of Pick)
(f) Retinitis Septica (of Roth) & © © º *
(g) External Exudative Retinitis (of Coats) (Sero-fibrinous
Degenerative Chorio-retinitis of Leber) &
III. Secondary Retinitis
(a) Chorio-retinitis . • • º
(b) Retinitis Secondary to Irido-cyclitis
C. Specific Inflammations
1. Syphilis ſº e e e * & *
(a) Diffuse Syphilitic Neuro-retinitis º º cº { }
(b) Syphilitic Peri-vasculitis (Syphilitic Retinal Angio-
pathy) * de ; e. &’ te ge & g
(c) Toxic Exudative Syphilitic Retinitis
(d) Central Serous Syphilitic Retinitis
(e) Circumscribed Gummatous Lesions ſº º
(f) Neuritis Papulosa . iº sº ſº gº tº
(g) Syphilitic Arteriosclerosis . & tº ge tº * >
(h) Congenital Syphilitic Chorio-retinitis . ſº e gº
2622
2622
2636
2636
2637
2637
2637
2637
2639
2643
2644
2645
2646
2648
2655
2656
2656
2660
2660
2661
2663
2664
2664
2664
2664
2665
2666
CONTENTS xvii
III. Inflammation of the Retina : Retinitis—conta.
C. Specific Inflammations—contal. - PAGE
2. Tuberculosis . * > Ç & * & & tº . 2666
(a) Miliary Tubercle of the Retina . & te g . 2667
(b) Massive Tubercle of the Retina . tº {º & . 2667
(c) Exudative Tuberculous Retinitis. º tº º . 2668
(d) Tuberculous Peri-phlebitis e º • wº . 2668
(e) Tuberculous Peri-arteritis. . * & & . 2672
3. Leprosy . & g tº * e * º g . 2673
4. Actinomycosis. e & & g g & ë . 2673
5. Acute Febrile Diseases . tº e e * * * . 2673
6. Dermato-myositis . & . º 4- * * . 2674
IV. Retinopathies Associated with General Diseases . gº º tº . 2675
A. Vascular Sclerosis and Retinopathies. tº e {e tº . 2675
Retinal Manifestations of Vascular Disease gº e º . 2677
1. Translucency of the Vessel Walls e sº * e . 2677
2. The Vacular Reflex . e e g 4e e & . 2677
3. Sheathing of the Vessels . º ë. te † & . 2680
4. Arterio-venous Crossings . e * º º tºp . 2683
5. Irregularity of the Lumen e e tº {º e . 2690
6. Tortuosity and Enlargement of the Vessels. e * . 269]
7. General Attenuation of the Retinal Arteries * se . 2692
8. The Pulse ſº & & & - 2693
Complications of Vascular Sclerosis . - 2693
1. Angiospasm, 2693. 2. Aneurysms, 2694. 3. Haemorrhages,
2694. 4. Thromboses, 2694. 5. Retinopathy, 2695.
Vascular Sclerosis . e tº * * tº . 2696
1. Diffuse Atherosclerosis . * º te * tº . 2696
Diffuse Atherosclerosis with Hypertension ſº & . 2696
2. Hypertensive Sclerosis . * tº º & ge . 2698
(a) Simple Hyperpiesia . e * * * e . 2699
(b) Diffuse Hyperplastic Arteriosclerosis : Arteriolar
Sclerosis © gº & * º o g . 2700
The General Relations of Vascular Sclerosis : Retinal and
Cerebral Arteriosclerosis gº & º e g . 2704
Vascular Retinopathies . * gº sº e * sº . 2706
1. Arteriosclerotic Retinopath e . e tº g . 2707
2. Renal Retinopathy . * t gº e tº e . 2709
Trench Nephritis . , ºt g g e º & . 27 13
3. Toxaemic Retinopathy of Pregnancy . . g to . 2723
4. Malignant Hypertensive Retinopathy. * * tº . 27.26
5. Diabetic Retinopathy º e de . . . e . 2729
The Treatment of the Vascular Retinopathies . g . 2735
B. Blood Diseases • & © o * & e tº . 2737
1. The Anaemias . º ſº º e e * º . 2737
2. Erythraemia . e º ſº w; - e e . 2738
3. The Haemorrhagic Diatheses . e . 2740
Purpura haemorrhagica, 2740. Haemophilia, 2740.
4. Leukaemia e * e & o § e . 2740
5. Lipa-mia © tº tº ſº tº ſº ſº tº ... 2742
6. Haemochromatosis . * . g - tº & g . 2744
xviii CONTENTS
V. Atrophies and Degenerations
A. Senile Changes
B. Secondary Degenerations
Colloid Bodies (Drusen) *
Cystic Degeneration of the Retina . o
Peripheral cystic (cystoid) degeneration,
Cystic macular degeneration (Honeycomb macula) : Macular
holes, 2755.
Circinate Degeneration.
C. Primary Degenerations . e tº * * > tº * *
1. Primary Pigmentary Degeneration of the Retina (Retinitis
Pigmentosa) § * e c * : e
Historical, 2766. Heredity, 2766. Associated degeneracies,
2768. Incidence, 2769. Clinical picture, 2770. Symptoms,
2772. Clinical course, 2772.
Atypical Clinical Manifestations
1. Central Pigmentary Degeneration
2. Degeneratio Sine Pigmento
3. Acute Pigmentary Degeneration
AEtiology wº º e º tº
2. Degeneratio Punctata Albescens ſº e º e
3. Familial Colloid Degeneration (Honeycomb Choroiditis of
Doyne) & º º e tº º * e e
4. Heredo-macular Degeneration
5. Familial Lipoid Degenerations . g tº * & º
(a) The Infantile Form of Amaurotic Family Idiocy (Tay-
Sachs’ Disease) . gº º e t º * :
(b) Essential Lipoid. Histocytosis (Niemann-Pick’s Disease)
(c) The Juvenile Form of Amaurotic Family Idiocy : Maculo-
cerebral Degeneration (Batten-Mayou or Spielmeyer-
Vogt Disease) * & ge ë ſº te
(d) Late Infantile Form of Amaurotic Family Idiocy .
VI. Cysts of the Retina
. Congenital Cysts
. Cystic Degeneration.
. Atrophic Areas
. Traction Cysts
. Parasitic Cysts
:
VII. Tumours of the Retina
A. Primary Tumours ſº &
I. Neuro-epiblastic Tumours. * * tº & * tº
Incidence, 2813. Clinical course, 2815. Prognosis, 2826.
Treatment, 2826.
The Nature of Neuro-epiblastic Tumours
(a) Retino-blastoma
(b) Neuro-epithelioma .
(c) Medullo-epithelioma
(d) Neurocytoma & e e
(e) Astrocytomata (True Gliomata)
PAGE
2745
2745
2746
2747
2752
2754
2760
2765
2765
2773
2773
2774
2774
2777
2784
2786
2788
2792
2797
2800
2801
2805
2806
2806
2806
2807
2807
2807
2812
2812
2812
2830
2833
2836
2839
2840
2840
CONTENTS - xix
VII. Tumours of the Retina—contal. PAGE
B. Associated Tumours º º e e e e º . 2843
1. Angiomatosis (the v. Hippel-Lindau Disease) e 2843
Incidence, 2846. Clinical picture, 2848. Pathology, 2852.
Treatment, 2856.
2. Tuberous Sclerosis (Bourneville’s Disease) . º º . 2857
3. Neuro-fibromatosis (Von Recklinghausen’s Disease) . . 2860
Sarcoma e - º -> e e e º . 2863
C. Secondary Tumours. - º - * º e e . 2863
1. Metastatic Carcinoma & - dº & º & . 2863
2. Metastatic Sarcoma . e º o • e o . 2864
VIII. Foldings and Detachments of the Retina . º - e e . 2864
Retinal Grooves . º º & - e º º e . 2864
Detachment of the Retina . e {- § & sº & . 2864
AEtiology - 2867
Race, 2867. Heredity, 2867. Sex, 2867. Bilaterality, 2867.
Age, 2868. Influence of myopia, 2868. The influence of eye-
strain, 2869. Traumatism, 2869. Inflammatory lesions, 2872.
Haemorrhagic lesions, 2873. Embryonic remains, 2873.
Parasites, 2874.
Histological Features . * - • e e º . 287.4
Changes in the Vitreous Body . & º & * . 2876
Retinal Holes and Tears . º e © & 4- . 2878
The Frequency of Retinal Holes . º e e . 2878
Types of Retinal Holes and Tears • 2879
Arrow head tears, 2879. Round holes, 2879.
Dialyses, 2879. Irregular slits and rents, 2879.
Multiplicity of Holes. º º -> e • . 2881
Macular Holes . º º e • º -> . 2881
Peripheral Holes and Tears te -> e e . 2882
The Healing of Retinal Holes . º e . . . 2886
The Ocular Tension in Detachment . º e © . 2886
The Sub-retinal Fluid º -> e - • e . 2887
Experimental Detachment - º e º o . 2888
AEtiological Theories © e - e - e © . 2889
The Significance of Retinal Holes & © º e . 2893
Exudative Detachments. g º e º e º . 2896
Serous Detachments e g • e -> e e . 2897
Clinical progress, 2899. Clinical symptoms, 2891.
The Appearance of the Fundus after Re-attachment . º . 2906
Diagnosis . {e - g º & º • • . 2907
Treatment . e • • - • - e º . 2908
Prophylaxis . e - e - º º º - . 2908
Medical Methods of Treatment . º -> 42 º . 2909
Surgical Treatment. gº º * º º {} . 2911
Prognosis & © e º º p e º . 29.18
CHAPTER XXXVII
DISEASEs of THE OPTIC NERVE
I. General Considerations . e e - e º e & . 2927
The Healing of Wounds. º º e - º © . 2929
Changes in the Nerve Fibres and the Interstitial Tissue. º . 29.30
XX - CONTENTS
II. Disturbances of the Circulation . © e
Haemorrhage into the Optic Nerve Sheaths
(a) Sub-dural Haemorrhages
(b) Sub-arachnoid Haemorrhages
Papilloedema
AEtiology, 2945. Unilaterality, 2949. Pathology, 2951. Patho-
genesis, 2955. Clinical picture, 2960. Symptoms, 2962. Diagnosis,
2964. Prognosis, 2964. Treatment, 2964.
III. Inflammation of the Optic Nerves
A. Perineuritis *
1. Exudative Perineuritis
2. Purulent Perineuritis
B. Optic Neuritis e ... e
1. Purulent Optic Neuritis
2. Interstitial Optic Neuritis
(a) Papillitis * o º º
(b) Retro-bulbar Neuritis - -
1. Acute Retro-bulbar Neuritis
2. Chronic Retro-bulbar Neuritis
3. Chiasmal Neuritis
Differential Diagnosis . - e
Prognosis, 2981. Treatment, 2981.
AEtiology e º & © º © e
I. Optic Neuritis Secondary to Local Infections
(a) Intra-ocular Infections
(b) Intra-cranial Infections
(c) Intra-orbital Infections
(d) The Nasal Sinuses .
II. Central Nervous Infections gº
(a) Disseminated (Multiple) Sclerosis e
(b) Acute Disseminated Encephalo-myelitis
(c) Neuro-myelitis Optica (of Devic) e
(d) Encephalitis Periaxialis Diffusa (of Schilder)
(e) Herpes Zoster . e w tº -
III. Leber’s Disease º - - e e º •
Heredity, 2999. Incidence, 3000. Clinical course,
3001. Pathology, 3002. AEtiology, 3003.
IV. Optic Neuritis from Endogenous Toxic Conditions
1. Acute Infective Diseases
2. Septic Foci • *
3. Endogenous Toxins . tº ge e º º
Diabetes, 3005. The anaemias, 3005. Malignant
disease, 3006. Pregnancy and lactation, 3006.
Severe haemorrhage, 3006.
4. Avitaminosis . -
Optic Neuritis in Calves .
C. Toxic Amblyopia . o º -> º e
A. Poisons Producing a Central Visual Defect
1. Tobacco . e & - º * tº -> º
AEtiology, 3010. Clinical picture, 3011. Course and
prognosis, 3013. Differential diagnosis, 3014.
Pathology, 3014. Treatment, 3017.
PAGE
2938
2938
2938
2939
2944
2967
2967
2968
2970
2971
2971
2973
2976
2978
2978
2979
2979
2980
2983
2985
2985
2985
2985
2985
2989
2990
2992
2993
2997
2997
2998
3004
3004
3004
3005
3007
3008
3008
3009
3009
CONTENTS xxi
III. Inflammation of the Optic Nerve—contal.
C. Toxic Amblyopia—conta.
A. Poisons Producing a Central Visual Defect—contal. PAGE
2. Ethyl Alcohol . tº * ſº tº e tº . 3019
3. Methyl Alcohol & † tº ſº * > e . 3021
4. Carbon Disulphide $ e e e ge . 3024
5. Iodoform : Iodine . º * tº © ſº . 3025
6. Lead g te sº tº g ſe g & . 3026
7. Thallium e § * g © tº e . 3028
8. Inorganic Arsenic . tº e * & º . 3028
B. Poisons producing a Peripheral Contraction of the Field . 3029
1. Organic Arsenic Preparations . e. tº ſº . 3029
2. Quinine and Its Relations . g tº te * tº . 3031
3. Aspidium (Filix Mas) * g º º ë . 3034
4. Salicylates e sº tº * º e . 3034
5. Ergot . º º * : e & & g . 3035
6. Anilin and its Relations . tº tº * º . 3035
D. Specific Infections . º * & tº e & tº . 3036
1. Tuberculosis . tº * & - ſº tº Ç . 3036
A. Tuberculosis by Direct Spread from Neighbouring
Structures sº * tº & g * . 3036
Spread from an intra-ocular lesion, 3036; from an
orbital lesion, 3036; from the meninges, 3037.
B. Metastatic Tuberculosis * º e . 3037
1. Miliary Tubercle & º º gº © . 3037
2. Solitary Massive Tubercle . * * > ſº , 3038
2. Syphilis. * * - © e tº g º . 3041
1. Syphilitic Optic Neuritis e * * . 3041
2. Gummata * ſº e º º * * . 3044
3. Para-syphilitic (Primary) Optic Atrophy . † . 3045
Clinical course, 3045. Symptoms, 3046. Pathology, 3050.
Prognosis, 3054. Treatment, 3055.
IV. Degenerations of the Optic Nerve . e o g © ſº & - 3059
A. Degenerative Depositions & º * > g & & - 3059
1. Arachnoid Cell Nests tº g º & * & . 3059
2. Corpora Amylacea . g º * & g & . 3360
3. Corpora Arenacea . { } * º g & e . 3061
4. Hyaline Bodies (Drusen) . * º & e gº . 3062
5. Amyloid Degeneration . & * e g . . 3065
B. Vascular Degenerations . g e ſº • † : * . 3065
C. Optic Atrophy e tº * º ſº e gº e . 3067
Pseudo-glaucoma * t e gº tº tº e . 3071
W. Tumours of the Optic Nerve and its Sheaths . e º e * . 3073
Clinical Symptoms and Course tº * * * e e . 3073
(a) Intra-ocular Tumours & tº ... º * g * . 3073
(b) Intra-orbital Tumours tº tº e g se º . 3074
(c) Intra-cranial Tumours e g º º e tº . 3077
A. Primary Tumours . tº 3078
1. Essential Tumours of the Nerve : Glioma (Spongioblastoma.) 3080
2. Meningioma . tº * © * e tº & . 3089
(a) Endothelioma . * e dº tº e e . 3089
(b) Fibroma . tº º e e º ge te . 3093
xxii CONTENTS
V. Tumours of the Optic Nerve and its Sheaths—contal.
A. Primary Tumours—contal.
3. Neuro-ectodermal Tumours
(a) Neurinoma . * g © º &
(b) Naevi and Malignant Melanomata . . . tº ©
Treatment of Primary Tumours of the Optic Nerve and its
Sheaths
B. Secondary Tumours
1. Carcinomata.
2. Sarcomata
CHAPTER XXXVIII
DISEASES OF THE LENS
I. General Considerations e
The Healing of Wounds
II. The Capsule and Sub-capsular Epithelium
Capsular Exfoliation . * te iº
Senile Exfoliation of the Lens Capsule
Colloid Bodies * * & dº * º º
Sub-capsular Fpithelial Opacities (Sub-capsular Cataract)
III. Cataract
Historical tº
General AEtiology • . e
The Chemical Pathology of the Lens & g tº g º g
Proteins, 3118. Lipoids, 3120. Inorganic extractives, 3121.
Organic extractives, 3123. Water and solid content, 3123.
Reaction, 3125.
The Chemical Pathology of the Blood . g *
The Normal and Pathological Metabolism of the Lens
The Permeability of the Capsule
Experimental Cataract . e º e
1. Cataract due to Mechanical Injury
2. Cataract due to Physico-Chemical Causes
(i) Osmotic Cataract
(ii) Cold Cataract º
(iii) Heat Cataract . &
(iv) Cataract of Acidification
. Radiational Cataract tº & e *
A Decrease of the Permeability of the Capsule
An Interference with Nutrient Supplies
. Cataract in Asphyxia. º *
. Deficiency Cataracts .
. Toxic Cataract . ſº º
(a) Naphthalene Cataract
(b) Lactose and Galactose Cataract
(c) Thallium Cataract . Ç e
9. Cataract in Aparathyroidea and Tetany
i
PAGE
3095
3095
3095
. 3097
3098
3098
3101
3102
3102
3104
3104
3106
3111
3112
3.115
31.15
3I 17
3.118
3126
3128
3136
3138
3139
3139
3139
3140
3140
3140
3141
3144
3144
3145
3145
3.146
3.146
3.15.2
3153
3154
CONTENTS xxiii
III. Cataract—contal. PAGE
AEtiological Theories . tº e * tº & e tº . 31.57
Biological theories, 3157. Immunological factors, 3158. Func-
tional theories, 3159. Local disturbances, 3160. General
metabolic disturbances, 3161. General Toxaemia, 3162. Vitamin
Deficiency, 3163. The Endocrine Glands, 3163.
General Pathology * : wº e * & º gº * . 31.65
General Symptomatology . * * e * gº tº . 3172
General Treatment & sº g * . e * e tº . 3177
Clinical Types of Cataract . © © e § © tº . 31.84
A. Evolutionary Lenticular Opacities . tº & e . 31.84
Punctiform Opacities (Blue-dot Cataract) $º * . 31.84
Dilacerated Cataract . e gº * * } g . 31.85
Coronary Cataract. . te g te * & . 3186
B. Senile Changes & ge te º e * te . 3188
Physiological Changes of Senescence tº & º . 3189
Pathological Senile Changes . g * $º tº . 31.90
Vacuole Formation . g & * * * . 31.90
The Formation of Clear Clefts . * º º . 3.191
Lamellar Separation º e g sº § . 3.192
Senile Cataract . * º gº * gº de . 31.93
1. Nuclear Cataract (Hard Cataract) & tº . 3195
Lens with Double Focus . & * tº . 31.97
Black Cataract (Cataracta Brunescens or Nigra) . 31.98
2. Cortical Cataract (Soft Cataract) . . . * . 31.99
Cuneiform Cataract tº te * © . 3200
Peri-nuclear Punctate Cataract . & e . 3202
Cupuliform Cataract . * & * e . 3203
C. Pathological Cataract * “s * > * & e . 3204
1. Diabetic Cataract * te & * ºp g . 3204
Cataract of Cholera and Hunger. º & e . 3209
2. Endocrine Cataract . te * * * : º . 3209
(a) Cataract in Aparathyroidea and Tetany. g . 3210
(b) Cataract in Myotonic Dystrophy . º º . 32.13
Familial Hypertrophic Dystrophy * © . 32.17
(c) Mongolian Idiocy tº i.e. wº e . 32.17
(d) Cretinism . & e * e º & . 32.19
(e) Dermatogenous Cataract º * e e . 3220
(i) Neurodermatitis . ſº gº & . . . .3220
(ii) Sclerodermia te * ſº tº (s . 3220
(iii) Poikiloderma Atrophicans Vasculare g . 3220
(iv) Chronic Eczema . tº & te e . 3221
3. Toxic Cataract . g e tº g e e . 3221
(i.) Dinitrophenol Cataract ſº & º * . 3221
(ii) Paradichlorobenzene Cataract . e © . 3223
(iii) Ergot Cataract . tº ge tº & * . 3223
(iv) Cataracta Cachectica . &e tº g § . 3224
4. Complicated Cataract . † ſe g g g . 3224
(i) Cataract associated with Irido-cyclitis . tº . 3228
(ii) Heterochromic Cataract * º & º . 3229
(iii) Cataract complicating Severe Corneal Inflammation 3229
(iv) Choroidal Cataract . e te tº º . 3230
xxiv. CONTENTS
III. Cataract—contal.
Clinical Types of Cataract—conta.
C. Pathological Cataract—conta.
4. Complicated Cataract—conta.
(v) Cataract complicating a Detached Retina
(vi) Cataract complicating Absolute Glaucoma .
(vii) Cataract complicating an Intra-ocular Tumour
(viii) Hepato-Lenticular Degeneration (Pseudo-sclerosis).
D. Secondary Cataract (After-Cataract)
IV. Displacements of the Lens . ** tº
Sub-luxation or Partial Dislocation
Dislocation of the Lens
Tremulousness of the Lens
CHAPTER XXXIX
DISEASES OF THE WITREOUS BODY
I. General Considerations
II. Degenerations of the Vitreous Body .
1. Fluidity of the Vitreous (Synchisis)
2. Vitreous Opacities . º tº
A. Endogenous Opacities
(a) Protein Coagula
(b) Crystalline Deposits g º º e te
Asteroid Bodies : Scintillatio Albescens or Nivea
Synchisis Scintillans
B. Exogenous Opacities
(a) Protein . g
(b) Exudative Cells
(c) Blood ge *
(d) Epithelial Cells .
(e) Tumour Cells
(f) Pigment Granules
3. Specific Degenerations
(a) Senile Degeneration . º
(b) Myopic Degeneration º tº *
(c) Degeneration in Retinal Detachment.
(d) Degeneration in Inflammatory Conditions .
(e) Haemorrhages . tº
(f) Degeneration in Tumours .
III. Detachments and Displacements. ſº
Detachment of the Vitreous Body . * e
Prolapse of the Vitreous Body (Vitreous Hernia)
IV. Cysts in the Vitreous .
PAGE
3231
323I
3231
3231
3232
3238
3241
3243
3244
3245
3246
3246
3248
3248
3248
3249
3249
3252
3253
3253
3254
3255
3.256
3256
3.256
3258
3258
3260
. 3262
3262
3265
3266
. 3266
3266
3273
3278
CONTENTS
XXV
CHAPTER XL
ANOMALIES OF THE INTRA-ocular PRESSURE
A. Glaucoma
Historical
Secondary Glaucoma . e tº wº © º tº
1. Glaucoma Secondary to Intra-ocular Inflammations
(a) Inflammatory Glaucoma
(b) Post-inflammatory Glaucoma
2. Glaucoma Secondary to Trauma .
(a) Traumatic Glaucoma .
(b) Post-traumatic Glaucoma
Post-operative Glaucoma. *
Traumatic Glaucoma complicated by a Retained Foreign Body
3. Glaucoma Secondary to Changes in the Lens
(a) Deformities of the Lens • *
Microphakia (or Spherophakia).
(b) Displacements of the Lens .
(c) Intumescence of the Lens g
(d) In Morgagnian Cataract . e
(e) Senile Exfoliation of the Lens Capsule (Glaucoma Capsulare)
4. Glaucoma Secondary to Vascular Anomalies of the Retina
(a) In Venous Thrombosis : Haemorrhagic Glaucoma .
(b) In Cyanosis Retinae & *
(c) In Vascular Retinopathies .
(d) In Papillitis e te e o tº
. Glaucoma Secondary to Intra-ogular Tumours
. Glaucoma Secondary to Detachment of the Retina
. Glaucoma Secondary to Intra-ocular Haemorrhage
. Glaucoma Secondary to Atrophic and Sclerotic Conditions
(a) Essential Atrophy of the Iris © o *
(b) Choroidal, Sclerosis and Retinal Degenerations.
9. Glaucoma Secondary to Venous Obstruction in the Orbit
10. Glaucoma Secondary to Epidemic Dropsy ge § ©
11. Glaucoma Secondary to Congenital Anomalies : Buphthalmos
(a) Uncomplicated Buphthalmos e º
:
(b) Complicated by widespread Vascular or Neural Anomalies
(1) Angiomata
(2) Neurofibromatosis
12. Experimental Glaucoma . * º & *
(a) The Production of Circulatory Stasis . © e
(b) Obstruction of the Circulation of Intra-ocular Fluid
Primary Glaucoma
Incidence . & & e ©
Bilaterality, 3330. Heredity, 3331.
Predisposing Factors . º ſº
1. The Refraction
2. Mydriasis . © e *
3. The Properties of the Blood.
4. The Capillary System .
PAGE
3280
3280
3285
3287
3287
3292
3296
3296
3301
3301
3306
3307
3307
3307
3307
3309
3309
3310
3311
3311
3312
3312
33.13
3313
3315
3315
3316
3316
3.316
3.316
3317
3320
3320
3322
3322
3324
3326
3326
3327
3327
3328
3333
3333
3333
3334
3335
xxvi CONTENTS
A. Glaucoma—contal.
Primary Glaucoma—contal.
Predisposing Factors—conta. PAGE
5. Systemic Diseases tº e iº tº wº t * . 3336
6. The Neuro-vegetative System te gº ſº e º . 3337
7. The Endocrine Glands * > & de te tº © . 3338
Pathology . º & tº wº e gº e tº º . 3341
The Pathological Results of Increased Pressure . • tº . 3341
AEtiology . tº º te sº © * e tº te . 3355
The Vascular Basis . º e e te º * tº . 3357
Contributory Factors * * º & º e ge . 3358
Clinical Picture . & © & ſe te g & sº . 3364
Simple Glaucoma (Non-congestive or Compensated) . tº . 3364
Congestive or Incompensated Glaucoma . e & & . 3365
Absolute Glaucoma. * is sº {º g e . 3367
Symptomatology . * tº e e * tº © tº . 3368
Objective Symptoms © e * e o e * . 3368
The Tension and its Variations * * & e & * . 3375
Visual Symptoms {e e * & e & e . 3379
Visual Fields . wº e e º o e te e . 3380
The Light Sense . ſº gº tº & - § © . 3384
Diagnosis . º e º ę e * e e tº . 3387
Provocative Tests . e & © ſº tº • • . 3389
Treatment . ſº º g tº & tº tº wº º . 3391
A. General Principles of Treatment . ſº e e & . 3391
B. Medical Methods of Reducing Tension . * e * . 3391
I. Pharmacological Methods . * • $ ge . 3391
II. Physical Methods ". sº & © & . . . 3399
C. Surgical Methods of Relieving Tension . * tº * . 3400
Systematic Treatment . e º * o ë & . 3407
General and Hygienic Treatment e tº me e * : . 3408
1. Simple Glaucoma . & * tº * 3408
2. Congestive (Incompensated) Glaucoma . º e . 3411
3. Absolute Glaucoma cº & º & º ſº . 34.13
Prognosis wº ſº tº e e e e . * . 3414
B. Hypotension : Ophthalmomalacia e sº $º e e e . 34.17
AEtiology © º * fe ſe tº & & * . 34.18
1. Congenital º * * § & * © . 3418
2. Myopia º º * gº * º * tº . 34.18
3. Retinal Detachment . * we e e * . 3418
4. Trauma wº tº o * g & e º . 3419
5. Infections and Inflammations. º & º g . 3421
6. General Illnesses . e * § © e i.e. . 3421
Diabetic Coma . * g * tº de g . 3421
Primary Anaemia g © g ſº * de . 3422
7. Nervous Influences & e e e tº g . 3422
8. The Endocrine Glands . tº te e © e . 3423
Pathology ſº e º iº * º g e * . 3424
Clinical Symptoms . tº * sº sº o º sº . 3427
Treatment tº & * $ & º * º { } . 3428
CONTENTS
xxvii
CHAPTER XLI
INTRA-OCULAR PARASITES
A. Nemathelminthes.
Filaria
1. Filaria loa
2. Filaria bancrofti . e © tº
3. Filaria medinensis (Dracunculus medinensis)
4. The Onchocerca
In the Anterior Chamber .
In the Lens . . .
In the Posterior Segment .
Onchoceriasis.
B. Platyheminthes e
1. Cysticercus Cellulosae .
In the Anterior Chamber
In the Lens - º e e
In the Posterior Segment of the Eye .
Diagnosis, 3442. Prognosis, 3443. Treatment, 3443.
2. Echinococcus e º
C. Arthropoda e ©
Ophthalmomyiasis Interna
Ophthalmomyiasis Anterior .
Ophthalmomyiasis Posterior .
Index
I’AGE
3430
3430
3430
3430
3431
3431
34.32
3434
3434
3436
3438
3438
3440
3441
3441
3445
344.7
3447
3448
3448
3451
SECTION XIII
DISEASES OF THE INNER EYE
THE UVEAL TRACT.
THE RETINA.
THE OPTIC NERVE.
THE LENS.
THE VITREOUS BODY.
GLAUCOMA AND HYPOTONY.
INTRA-OCULAR PARASITES.
T. (). —WOI, III.
Fig. 1765.-WILLIAM Mackenzie.
(1791–1868.)

CHAPTER, XXXV
DISEASES OF THE UVEAL TRACT
It seems to me appropriate to introduce this section on Diseases of the Inner
Eye with a portrait of William Mackenzie (1791–1868) (Fig. 1765). Before the era
of the ophthalmoscope the internal eye was virtually a closed book, and the physician,
who could not be guided by observation, had to reason largely by inference. It is
difficult for us to realize to-day the precariousness of his position, and it says much
for the powers of observation and almost intuitive deduction displayed by the masters
of 100 years ago that they acquired the knowledge and achieved the results they did.
William Mackenzie, the surgeon of Glasgow, educated originally for the Church and
fortunately for us deviating into medicine, was pre-eminently one of these. Inspired
to specialize in the eye by Beer, of Vienna, he founded the Glasgow Eye Infirmary in
1824, and by the labours of a long and industrious life, was one of the foremost surgeons
to raise ophthalmology to the high place it now holds among the special branches of
medicine. The knowledge of his day, correlated by his unusually logical and lucid
appreciation and abundantly enriched by his own personal observations and original
deductions, was collected in his classical book which, translated into German (1832)
and French (1844–56), became the standard work for his generation—his “Practical
Treatise on the Diseases of the Eye,” the first English edition of which appeared in
1830, and the fourth in 1854.
I. GENERAL CONSIDERATIONS
While it can be sharply divided regionally into three distinct areas—
the iris, ciliary body and choroid—the entire uveal tract is developmentally,
structurally and functionally one indivisible whole. Formed from the
mesoderm which surrounds the optic cup, and developing into what can be
best described in general terms as a spongework of blood-vessels, this middle
tunic of the eye takes on the all-important rôle of providing nourishment
for the globe. It will be recalled that, apart from a minor contribution
from the anterior ciliary vessels which arise from the arterial twigs to the
recti muscles, the vascular supply of the uveal tract is formed by the
posterior ciliary arteries, branches of the ophthalmic artery. These pierce
the sclera near the posterior pole, the short vessels running directly into the
posterior part of the choroid, the two longer vessels travelling forwards in
the supra-choroidal space to the ciliary region where they form the major
circle of the iris from which is derived the blood supply of the ciliary body
and iris as well as that of the anterior portions of the choroid. It will be
appreciated, therefore, that the pathology of the whole richly vascularized
area is very similar, and that it is frequently involved throughout its entire
extent. If localization of a disease-process occurs, it is on a regional
2097 B 2
2098 TEXT-BOOK OF OPHTHALMOLOGY
rather than a topographical basis, following the distribution of the blood-
supply—an inflammation, for example, of the anterior or the posterior part
of the uveal tract. An iritis never exists alone without some cyclitis ; a
cyclitis never without an iritis and most usually—if not invariably—with
some anterior choroiditis. It is true that diseases of the anatomically
distinct areas give rise to different clinical signs and symptoms, have
different incidental effects, and carry a different prognosis, but, for the most
part, sharing as they do a common aetiology and a common pathology, and
merging inseparably the one into the other, it seems conducive to
economy in space and logic in treatment to consider them together.
Diseases of the uveal tract, particularly those of an inflammatory
nature, are common—a circumstance not at all surprising in view of the
extremely rich vascularity of its structure : it readily shares in general
systemic disease, it faithfully reflects toxic states, and it is greedy in acting
as an area of lodgment for circulatory metastases, organismal or otherwise.
Moreover, diseases once localized frequently assume serious proportions,
for a vascularized network is not characterized by sluggishness in its
reactions, and further, once established they frequently entail serious
effects. Since the uvea assumes the function of nourishing the eyeball,
it is obvious that if pathological processes therein are widespread, they will
have a profound and calamitous effect upon the entire metabolism of the
globe—the logical end-result is, indeed, its complete atrophy. If they
are circumscribed the same considerations obtain to a large extent,
especially if they are localized in the ciliary body which carries the heaviest
responsibility in nutrient activity. But to this general consideration
incidental effects of great importance must be added. The optical functions
of the iris may be deranged, the pupillary aperture obliterated by exudative
or other material, or the vitreous chamber filled with opacities. Moreover,
a blockage of the drainage channels at the angle of the anterior chamber or
adhesions of the normally mobile iris to the lens may impede the circulation
of the intra-ocular fluid so that a secondary glaucoma develops as the
terminal event. Finally, as we have already seen, the sentient layers of the
retina are nourished directly from the choroid, so that any lesion of the
latter invariably affects the former secondarily, to some extent in all cases
and with disastrous results in many.
The Healing of Wounds
Aseptic wounds of the iris are peculiar owing to the very small amount
of tissue-reaction which they stimulate. So much, indeed, is this the case
that there is no formation of granulation tissue or of scar tissue, and years
after it has been inflicted an iridectomy wound appears just as it did
originally without any new formation of fibrous tissue or other evidence of
reparative activity—the ragged edges remain, the endothelium on the
DISEASES OF THE U WEAL TRACT 2099
anterior surface and the pigment epithelium on the posterior make no
attempt to extend round the raw area; only the edges may be somewhat
thickened and retracted slightly backwards owing to contraction of the
posterior layers (Fuchs, 1896; Parsons, 1904; Henderson, 1907; McBurney,
1914). It is not that the iris is incapable of forming scar tissue; this, we
shall see, it frequently does when irritated by toxins, and, as we have seen
already," it does so profusely when it becomes prolapsed through a per-
forating corneal wound or ulcer. Its passivity may be due to the fact that
it is so securely protected from the irritative factors commonly affecting
other raw surfaces by being suspended in aqueous that the necessary stimuli
are lacking. Whatever the
cause, when the blood-clot
which at first fills the
wound is absorbed—and the
haemorrhage is usually small
in quantity --no further
reaction takes place.
Wounds of the ciliary
body are of clinical interest
in view of their immediate
liability to set up a
traumatic irido-cyclitis "
which may easily involve
the loss of the eye, and their
ultimate liability to excite a
sympathetic ophthalmitis.”
In simple cases, however,
uncomplicated by either of
these unpleasant alterna- Fig. 1766.-Wound of CILIARY BoDY.
tives, the healing of a - Fourteen days old perforating wound in a monkey.
--- - Equatorial section. x 25. (Parsons.)
ciliary wound does not display
the inactivity which characterizes the iris, but is associated with a definite
tissue-reaction comparable with that which occurs in the choroid (Parsons,
1903) (Fig. 1766). The process of healing takes place by cicatrization, and
occurs rapidly as might be expected in a tissue so richly vascularized. The
vascular elements of the ciliary body itself take a large share in providing
fibroblasts which permeate the blood-clot filling the wound, while more
are supplied by the episcleral tissue. The sclera remains inert, as also does
the pars ciliaris retinae except that the pigment cells show some slight
degree of proliferation. As in most reactive processes in the uvea, the
pigment-cells of this tissue also show proliferation, and pigment granules
become scattered throughout the scar, some of them intra-cellular but most
of them lying free in the meshes of the scar-tissue or engulfed in leucocytes.
* Vol. II, p. 1825. - p. 2109. * p. 2135, * p. 2324.

2100 TEXT-BOOK OF OPHTHALMOLOGY
Wounds of the choroid heal in a similar manner by changes essentially
inflammatory and reparative, and as one would expect from its rich
vascularity, this tissue forms the chief means of repair in wounds of the
Fig. 1767–Wound of RETINA AND Choroid.
Nineteen days after injury in a monkey. Retina and choroid replaced by a
fibrous scar. x 60. (Parsons.)
posterior segment of the globe which necessarily usually involve the sclera
and the retina as well. It is largely from the point of view of the latter
tissue that such wounds have received attention (Tepliaschin, 1894; Parsons,
Fig. 1768–Wound or THE RETINA.
Fifteen days after perforating injury in a monkey. The edges of the selera
are separated by new-formed fibrous tissue derived from the episclera which extends
far into the vitreous, x 25. (Parsons.)
1903). In the neighbourhood of such an uncomplicated wound the choroid
is relatively normal apart from pigmentary proliferation, but in the
immediate vicinity of the wound it shows intense activity, throwing out
fibroblasts into the blood-clot, a process in which, in perforating wounds,


DISEASES OF THE UVEAL TRACT 210]
it is assisted by the episcleral tissues : again the sclera and the retina are
relatively inert. In the simplest case the retina and choroid in the course
of the 3rd week after an injury may be replaced by a fibrous scar consisting
merely of layers of imbricated spindle-cells closely packed together in which
are foci of pigment, partly free and partly intra-cellular (Fig. 1767). Any
new-formed vessels eventually become obliterated to form fibrous strands.
and as the fibrous tissue itself degenerates and contracts into thinner
lamellae, hyaline and other forms of degeneration eventually supervene. In
many cases, however, a super-abundance of scar-tissue is formed which may
extend from the wound far into the vitreous cavity, sending out long
proliferative filaments which may spread as far afield as the ciliary body
(Fig. 1768). Such a condition of traumatic proliferating chorio-retinitis is
not uncommon, and will be dealt with more fully in the section on injuries
to the eye.”
Fuchs. B. O. G. Heidel., xxi, 179, 1896. Parsons. R. L. O. H. Rep. xv. (3), 215, 1903.
Henderson, T. O. Rev., xxvi, 191, 1907. Path. of the Eye, London, i, 286, 1904.
McBurney. A. of O., xliii, 12, 1914. Tepljaschin. A. f. Aug., xxviii, 354, 1894.
II. DISTURBANCES OF THE CIRCULATION
The clinical and pathological interest of circulatory disturbances in the
uveal tract is limited mainly to the choroid. Essentially they comprise
(1) hyperaemia and anaemia, (2) emboli and thrombi of an innocent nature
(non-infective and non-neoplastic), and (3) haemorrhages. Apart from
those due to trauma, haemorrhages in the iris and ciliary body are rare ;
while in those regions even the relatively common condition of vascular
sclerosis gives rise to few if any symptoms and has excited little pathological
interest.
Controlled as it is by the two vascular circles running round the anterior
segment of the uveal tract, the blood-supply of the iris and ciliary body
tends to react vascularly as a single richly anastomosing network. In the
choroid, however, despite its apparently rich vascularity, there is a very
definite regional characterization. Leber (1903) has shown anatomically
that the larger posterior ciliary arteries have practically no branches of
intercommunication except of a capillary nature, and that such inter-
anastomoses which do exist are confined for practical purposes to the region
around the optic nerve and to the anterior part of their distribution near
the periphery of the fundus. Hence, while the iris and ciliary body tend
to react as a whole, to a very great extent the vascular network of the
choroid reacts as a series of terminal vascular systems the individual branches
of which may be affected separately, and may show varying degrees of
liability to disease or obstruction (Nettleship, 1903; Coats, 1907; Hepburn,
1 Vol. IV.
2102 TEXT-BOOK OF OPHTHALMOLOGY
1910–12; and others). Histologically, also, sclerotic lesions in the chorio-
capillaris may occur in sharply defined areas, the change from healthy to
diseased vessels being sometimes quite abrupt (Greeves, 1912; and others).
From this point of view the main area of the choroid may be divided into
three separate regions each of them capable of showing a considerable degree
of individuality—the region of the posterior pole, the equatorial region, and
the peripheral region—so that we find a definite tendency towards a
preferential distribution of inflammatory or sclerotic lesions to one or other
of these areas. To those three one other region must be added, the juxta-
papillary region supplied separately by the vessels of the zonule of Zinn.
In each of these main areas sub-divisions occur, a small section reacting
independently. The most typical example of this is the macular region,
where sclerotic or inflammatory changes may be localized indicating the
involvement of a single semi-independent vascular bed controlled by one of
the posterior ciliary arteries.
The importance of all vascular lesions in the choroid, of course, lies in
their disastrous effects upon the retina, the sentient layers of which rely
upon the chorio-capillaris for nourishment. The changes due to a circulatory
disturbance in the retina and choroid have been thoroughly studied in
animals by experimental section of the posterior ciliary arteries and the
optic nerve (Berlin, 1871; Wagenmann, 1890; Capauner, 1893; Krückmann,
1899 ; and Nicholls, 1938). On section of the posterior ciliary arteries a
degeneration of the epithelial layers of the retina occurs, the cells of the
pigmentary epithelium become loose and necrotic, the pigment migrating
into the retina to form irregular star-shaped elements resembling bone-
corpuscles, while Bruch's membrane shows degenerative changes, sometimes
with the formation of colloid excrescences.
Clinical cases wherein such a condition is exemplified are few, since,
despite the frequency of disturbances in the choroidal circulation, the
opportunities of obtaining such eyes for anatomical examination are rare ;
moreover, it is frequently difficult to determine whether pathological
changes are due to inflammatory processes or whether they are simply
degenerative and dependent entirely on circulatory disturbances. Such
opportunities do occur, however, after operations for optico-ciliary resection
or after the removal of tumours of the optic nerve when the eyeball has
to be sacrificed subsequently (Studer, 1905; Birch-Hirschfeld, 1910;
Koyanagi, 1913; Komoto, 1915) (Fig. 1769). In the choroid the vessels
are sometimes quite obliterated by new-formed fibrous tissue which
subsequently tends to undergo hyaline degeneration, the whole tissue is
atrophied, and the pigment cells become degenerated, the normally elongated
and much-branched chromatophores assuming a rounded form. Gross
changes are seen in the pigmentary epithelium : the cells of this layer as a
whole become degenerated and lose their pigment ; some of them migrate
into the retina where considerable quantities of pigment may be found,
DISEASES OF THE UWEAL TRACT 2103
Fig. 1769.-RETINA AND CHoRoid AFTER Stoppage of THE BLooD-supply.
The retina and choroid near the optic disc. The retina is reduced to a swollen
necrotic mass. The choroid shows pigmentary degeneration, the chromatophores
being changed into round cells. (Komoto, T. O. S.)
Fig. 1770.--THE FUNDUs AFTER REs Ection of THE OPTIC NERVE 5: YEARs PREviously
sHow ING CHANGEs DUE to Stoppage of THE BLooD-supply. (Collins, T. O. S.)


2104 TEXT-BOOK OF OPHTHALMOLOGY
sometimes as free granules, at others as large circular clumps ; while at other
places, again, the pigmentary cells proliferate or become changed into
spindle-shaped cells which enter the outer layers of the retina like an out-
growth. When the posterior ciliary arteries alone are severed, the outer
half of the retina becomes thin, its normal structure is lost, and proliferation
of the neuroglia and degeneration of the rods and cones are marked. It is
noteworthy that Komoto (1915) found that when the central artery of the
retina was cut also, while in the posterior region the retina became completely
necrotic, the anterior region remained relatively vital, indicating that the
retina in this area—even its inner layers—could be kept alive by the
peripheral choroidal circulation supplied by the anterior ciliary arteries.
A case reported clinically by Collins (1912) illustrates the ophthalmoscopic
appearance of the fundus 5 years after complete destruction of the posterior blood
supply following the extirpation of an optic nerve tumour (Fig. 1770); the greatest
changes occur at the posterior pole where the retina and choroid are replaced by
scar tissue, in the anterior region a thin atrophic choroid gives a red reflex but over
the entire fundus pigmentary degeneration of the typical bone-corpuscle type is
profuse.
Berlin. K. M. Aug., ix, 278, 1871. Romoto. T. O. S., xxxv, 295, 1915.
Birch-Hirschfeld. Z. f. Aug., xxiv, 193, 1910. Koyanagi. K. M. Aug., li (1), 623, 1913.
Capauner. B. O. G. Heidel., xxiii, 45, 1893. Krückmann. A. f. O., xlvii (3), 644, 1899.
Coats. T. O. S., xxvii, 135, 1907. Leber. G.-S. Hb., II, ii (2), 39, 1903.
Collins. T. O. S., xxxii, 396, 1912; xlvi, 86, Nettleship. R. L. O. H. Rep., xv., 189, 1903.
1926. Nicholls. Brit. J. O., xxii, 672, 1938.
Greeves. R. L. O. H. Rep., xviii, 301, 1912. Schreiber. B. O. G. Heidel., xxxiii, 286, 1906.
Hepburn. R. L. O. H. Rep., xviii, 92, 1910. Studer. A. f. Aug., liii, 206, 1905.
T.O.S., xxxii, 361, 1912. Wagenmann. A. f. O., xxxvi (4), 1, 1890.
1. Hyperaemia
HYPERAEMIA, a dilatation of the small vessels in response to irritation,
is not clinically recognizable except in the iris where its occurrence is most
frequently a symptomatic condition as a precursor of inflammation. The
same causes, however, which produce an iritis, if acting in so slight a degree
as not to determine an actual inflammation, may give rise to a condition of
irritation involving a hyperaemia of the iris.
A pure hyperaemia is, however, most frequently seen in association with corneal
irritation, such as by a foreign body or in the case of a corneal ulcer. This, it will
be remembered,” is due to an unusually widespread axon reflex mediated by the
Vth. nerve, whereby, after a localized trauma, a dilatation of the small vessels is set
up throughout the uveal tract, associated with an increased permeability of the
capillaries, a rise of temperature in the anterior chamber, and an increase in the
intra-ocular pressure. Such a condition, of course, may not remain permanently in
the stage of hyperaemia ; exudation may follow with all the symptoms of iritis, even
to the formation of a hypopyon.
1 Vol. I, pp. 420 (521), 520 (529).
DISEASES OF THE UVEAL TRACT 2105
The clinical picture of hyperaemia of the iris is readily recognized. The
increase in vascularity brings on a change in appearance, more marked in
lightly coloured irides than in those of darker complexion ; a blue or grey
iris appears greenish, while a brown iris becomes tinged with yellow, the
change being seen most easily in unilateral cases when comparison can be
made with the sound eye. Qn account of the radial disposition of the
vessels their engorgement makes the pupil contract mechanically, an effect
usually augmented by a reflex spasm of the sphincter brought on by
irritation : for this reason it is less responsive in its reflexes and dilates less
readily with mydriatics. Close examination by the loupe or the slit-lamp
reveals, particularly in lightly coloured irides, the presence of distended
radial vessels as red striae, and an apparent increase in their number due to
the filling up of channels which normally are partially or completely
obliterated. Finally the hyperaemic condition becomes extended to some
degree through the anterior ciliary vessels to the sub-conjunctival circulation
where it is evident as a mild ciliary injection." The picture is accompanied
by some photophobia, some increase in lacrimation, and in more severe cases,
a reflex trigeminal neuralgia. Comfort is usually rapidly produced by the
elimination of spasm with atropine and the avoidance of bright light, and,
provided the cause can be eliminated, and provided the irritation is not
great enough to promote actual inflammation, the condition dies away
leaving no traces of its presence.
Eye-strain and uncorrected errors of refraction, as well as exposure to glare, are
frequently said to produce a hyperaemia of the choroid, an assumption for which there
seems to be no adequate evidence.
PAssIVE HYPERAEMIA of the uveal tract is a symptomatic condition of incidental
importance caused by some obstruction to the circulation, either intra-ocularly, as
by a tumour, or extra-ocularly, as by an orbital inflammation or neoplasm.
In POLYCYTHAEMIA the hyperaemia may be so pronounced as to alter the colour
of the iris from a blue to a reddish brown appearance. Goldzieher (1904) considered
this effect due to multiple minute haemorrhages, but the fact that the normal colour
re-appears after death demonstrates that its origin is an enormous distension of the
smaller vessels, particularly the venules (Baquis, 1908; Tallei, 1924).
ANAEMIA OF THE UVEAL TRACT has little clinical significance. An interesting
occurrence is a localized complete anaemia due to an obliteration of choroidal vessels
and leading to destructive retinal lesions occurring in patients with Raynaud’s disease
(Bailliart, Tillé and Laignier, 1934).
Bailliart, Tillé and Laignier. Bull. S. d’O. Goldzieher. Cb. pr. Aug., 257, 1904.
Paris, xlvi, 17, 1934. Tallei. Boll. d’Oc., iii, 545, 1924.
Baquis. A. f. O., lxviii, 117, 1908.
2. Embolism and Thrombosis
Small EMBOLIC or THROMBOTIC LESIONS in the uveal tract are common,
most of them being associated with inflammatory conditions : a benign
1 For appearance and differential diagnosis see Vol. II, p. 1500; also Plate XXI, Fig. 1352.
2106 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 1771–Blockage of Choroid AL VEssels.
Showing the resultant changes when a large number of choroidal vessels
becomes occluded. (Hepburn, T. O. S.)
Fig. 1772-CHonoidal, INEAR.cºm.
In a woman of 42 suffering from multiple thrombi following phlebitis of leg.
The effects of blockage of a large choroidal artery. (Hepburn.)


DISEASES OF THE UVEAL TRACT 2107
embolism—that is, one neither organismal nor neoplastic in nature—is rare.
Owing to the rich terminal inter-anastomoses in the anterior regions of the
uveal tract and within the individual choroidal systems, it is probable that
small incidental emboli or thromboses rarely excite clinical effects, but a
blockage of one of the larger feeding vessels of the choroid may produce a
widespread infarction, characterized by necrosis and atrophy of the area of
supply, with pigmentary disturbance and degeneration of the overlying
retina (Fig. 1771). Particularly is this evident in the central area where the
effects are, of course, disastrous. The infarct appears ophthalmoscopically
as a sharply localized scar-like lesion without outlying exudate, over which
the retina, the pigment epithelium and the choroid are destroyed (Fig. 1772).
Fig. 1773.-INFARCT of THE PostERIor CILIARY ARTERLEs.
It is obvious that such cases rarely become available for anatomical examination.
Coats (1907) has described one of more than usual interest which occurred in a patient
A–A", necrotic area in sclera. B-B', necrotic area in choroid. C-C, necrotic
area in retina. E, equatorial staphyloma. Note atrophy and cupping of the nerve.
(Coats, T. O. S.)
with scleritis and was due to a blockage of one of the entering arteries. The resulting
infarct involved the choroid and retina in complete necrosis over a wedge-shaped
area, more extensive in the retina than in the choroid (Fig. 1773). The pathological
picture resembled closely that of a pale infarction of the kidney, and around the
necrotic area was a slight degree of reactive inflammation. Such definite localiza-
tion may appear strange in the richly vascularized choroid, but it follows from the
poverty of anastomoses between the areas of distribution of the posterior ciliary
arteries. Goldstein and Wexler (1933) also described an unusual case of simultaneous

2108 TEXT-BOOK OF OPHTHALMOLOGY
emboli in the posterior ciliary and central retinal arteries derived from a thrombosis
in the innominate artery superimposed upon arteriosclerotic disease ; the clinical
picture, however, was dominated by events in the retina.
Cases of AIR EMBOLISM are occasionally met with as a part of widespread air
embolism following thoracic operations: in this event the pupil remains widely dilated
until death. A case which seemed to be characterized by repeated emboli in the posterior
ciliary arteries was described by Knapp (1868) in a patient with cardiac symptoms:
immediate blindness was followed by recovery of vision apart from Scotomata, and a
greyish opacity in the fundus gradually disappeared. In a case of embolism described
by Seefelder (1919), the patient remained blind for four days and recovered practically
completely the sight of the eye, a localized scar remaining.
THROMBOSES OF THE LARGE VENOUS CHANNELS have a similar disastrous
effect on the entire area of their drainage. Thus the events which follow a
thrombosis of a vortex vein are most vividly seen when this occurs accident-
ally during an operation for detachment of the retina by cauterization
(Black, 1932). Such a catastrophe is accompanied by intense congestion
and chemosis of the eye associated with some pain, and an ophthalmoscopic
appearance resembling a large solid-looking detachment of the retina
without inflammatory signs. At first the vision is reduced to perception
of light, but after some weeks a considerable degree of improvement may
OCCUI”.
Black. T. O. S., lii, 486, 1932. Rnapp. A. f. O., xiv. (1), 245, 1868.
Coats. T. O. S., xxvii, 135, 1907. Seefelder. Z. f. Aug., xli., 265, 1919.
Goldstein and Wexler. A. of O., x, 70, 1933.
3. Haemorrhages
H.EMORRHAGES in any region of the uveal tract may be due to three
factors (apart from trauma).
1. Over-distension of the vessels as the result of some circulatory
disturbance with the result that their walls give way. Small haemorrhages
in the iris may be caused in this way by an intra-ocular operation, the
sudden diminution of the intra-ocular pressure on opening the globe depriving
the small vessels of their usual support from without so that minute bursts
occur. Such haemorrhages can be produced experimentally with great ease
after a paracentesis in a rabbit by a sudden compression of the abdomen so
that the vascular pressure is momentarily raised.
2. Fragility of the vessel walls, whether due to general atherosclerotic
changes in old people, or to localized disease in the uveal tract. In general
terms such diseases may be atrophic or inflammatory in nature. Probably
the most frequent example of the former type is the choroidal haemorrhages
occurring in the atrophic processes of myopia, which, when they occur at
the macula, as they frequently do, may permanently abolish central
vision. The inflammatory type is most readily seen in the iris, when in
DISEASES OF THE UVEAL TRACT 2109
intense inflammations, such as in the irido-cyclitis associated with herpes
or gonorrhoea, the normal inflammatory process of diapedesis becomes
intensified into the development of petechial haemorrhages or the formation
of a hyphaema in the anterior chamber.
3. A derangement of the blood itself which produces pathological changes
in the vessel walls, such as in the anaemias, leucaemia, purpura, scurvy, or
diabetes.
HAEMORRHAGES IN THE IRIs are rarely extensive or of serious import.
It is well known that even after an iridectomy or a traumatic tear of
the iris tissue, bleeding is rare and usually small in degree. This is due
partly to the rapid retraction of the vessels in the spongy iris tissue,
partly to the activity of very efficient axon reflexes through the sympathetic
which obliterate the lumen of the vessel until a clot has been formed,” and,
in cases where the globe is intact, to the occluding action of the intra-ocular
pressure.
It is an exception for a large haematoma to be formed in the tissues after an
iridectomy, such as that described by Collins (1890) wherein the haemorrhage extended
to strip Descemet’s membrane from the cornea ; most exceptionally it is seen arising
apparently spontaneously in iritis (Ellett, 1939). Even in the operation of cyclo-
dialysis wherein anterior ciliary vessels are deliberately broken across, bleeding does
not always follow. Only rarely does excessive and uncontrollable bleeding occur,
as in haemophiliacs after an operation, when continued haemorrhage involving a
raised tension may persist until some drastic method is undertaken to control it,
such as a blood transfusion (Delord, 1935).
Little pathological disturbance follows a haemorrhage into the iris :
the blood is rapidly absorbed leaving no trace. If the quantity is large, it
collects in the anterior chamber and sinks to the bottom forming a HYPH.EMA
(iT6, below ; 21 pia, blood), wherefrom it is usually absorbed in the course of
a few days. Even when the anterior chamber is completely filled forming a
total hyphaema, absorption, although it may be delayed over some weeks,
usually occurs uneventfully without any therapeutic measures other than
keeping the patient relatively quiet. The only serious permanent result
that may ensue is blood-staining of the cornea with products of the red
corpuscles which have broken down in the anterior chamber—a rare
complication, however, which only follows haemorrhage in conditions of
raised intra-ocular pressure.” In repeated bleedings, on the other hand,
symptoms of iritis of considerable severity may occur, as have been noted,
for example, in purpura (Benedict, 1930; Werner and Adlercreutz, 1931) or
haemophilia (Jean Sédan, 1930).” In scurvy local haemorrhages may produce
small roseola-like patches, but an iritis is rare (Löwenstein, 1917).
HAEMORRHAGES IN THE CHOROID are of more clinical significance, for
if they are of any size they may produce a permanent defect in vision owing
1 Vol. I, p. 418. * Vol. II, p. 2038. 8 p. 2132.
2110 TEXT-BOOK OF OPHTHALMOLOGY
to destruction of the overlying retina, a consideration which applies
particularly to those occurring at the macula.
The traumatic type is well known and will be dealt with at a later stage.
The spontaneous type is little noticed in the literature, their occurrence,
indeed, being denied by some authorities (Venneman, 1906), while others
admit that they have never seen them clinically (Fuchs, 1925): nevertheless,
they are of not uncommon occurrence, as witness the paper of Bedell (1932)
wherein 28 personal cases are photographically described. Moreover, they
are always of grave significance indicating serious disease and entailing
permanent consequences, the smaller extravasations involving a localized
loss of vision, and the more profuse haemorrhages almost invariably leading
to loss of the eye.
A LOCALIZED CHOROIDAL HAEMORRHAGE (Plate XXXII) which confines
itself to the choroid varies much in size depending on the importance of the
vessel from which bleeding has occurred. It appears as a dark red, granular,
rounded mass with a strong tendency to assume a circular shape, over which
the retinal vessels course, and may be many times a disc-diameter in size.
Sometimes smaller masses are grouped irregularly round the main lesion ;
and as one haemorrhage begins to disappear it may be followed by others in
the same region or separated from the original site. The retinal tissue lying
upon it, deprived of its nourishment, becomes a smoky grey or whitish
colour, the delicate shading of the membrane over the red mass resembling
the bloom of a ripe plum. Meantime in the corresponding area of the visual
field there is a blur, and since other symptoms are absent the occurrence may
well escape notice unless the macular region is involved.
The process of absorption is slow and may be a question of months.
If the haemorrhage is massive it usually takes place concentrically from the
periphery, quite frequently irregularly, leaving isolated islands stranded
behind in its recession. Over it the retina is grey and translucent, merging
progressively from a central milky grey area to a stippled periphery where
patches of exudate appear. The permanent scar is considerably smaller
than the original haemorrhage, and remains as a white fibrous irregularly
pigmented plaque ; and in this region, of course, blindness is complete. On
the other hand, the haemorrhage may take the form of a thin spreading
film of blood which leaves behind a pigmented area covered by a greyish
adherent membrane through which the sclerosed choroidal vessels are visible ;
in this region vision, if not abolished, is greatly disturbed (Fig. 1775). A
choroidal haemorrhage is, in fact, more destructive to vision than a retinal
haemorrhage, and when it occurs at the macula its effects are serious indeed
(Hepburn, 1910; Foster Moore, 1910).
A differential diagnosis is easily made between pre-retinal or retinal homorrhages ;
the former obviously cover the retinal vessels. The latter are usually striate in
appearance, characteristic in shape, and the superficial vessels rarely cross over them,
PLATE XXXII.
Choroid AL HAEMoRRhAges
Fig. 1774–RECENT Choroid AL HAEMoº- Fig. 1775. -ScLERosis AND ATRoPHY AFTER
RHAGE IN PAGET's Disease. CHoRoid AL HAEMoRRHAGE.
Fig. 1776.-RECENT Choroid AL HAEMoR-
RHAGE AT MACULA.
Note radiating oedematous lines.
Fig. 1777.-Choroid AL HAEMoRRHAGEs IN Fig. 1778. Choroid AL HAEMoRRHAGE
Myopia. INvolving the MacULA.
Ten days old. (Foster Moore.)
[To face p 2110.



T)ISEASES OF THE UVEAL TRACT 21 11
while a choroidal haemorrhage is usually globular, always dark and granular and
invariably deep to the retinal vessels. A second point in diagnosis is the distinction
from choroidal pigmentation, whether post-traumatic or inflammatory : a mass of
pigment is darker in colour, black as opposed to a deep plum red, more homogeneous,
without the granular appearance, has an irregular outline, and does not vary in size
or migrate in position. The last condition, and one of considerable importance, which
must be considered is a choroidal tumour. Choroidal blood is smoother in its surface,
redder in colour, and transilluminates better ; moreover, the history of sudden onset
is typical of a haemorrhage and the causal condition (other haemorrhages, Sclerotic
changes, myopia, etc.) may point to the diagnosis. Diagnostic puncture would
probably not be sufficiently certain in its findings to justify the danger of dissemination
in the event of a tumour being present ; and it is to be remembered that hamorrhages
not uncommonly occur in small malignant melanomata. Individual cases, indeed, may
present difficulties so considerable as to justify an excision of the eye, a circumstance
to which we are indebted for the available pathological studies (Meller, 1925, “pseudo-
sarcoma of the choroid ‘’’; Corrado, 1934) (see Fig. 1781).
The aetiology of such haemorrhages is varied.
1. Acute choroiditis. Few acute inflammations of the choroid are
unaccompanied by bleeding, recent haemorrhages disappearing and new ones
appearing on the periphery of an advancing lesion. Usually, however, they
are small in amount and incidental in importance.
2. In vascular sclerosis affecting the choroid haemorrhages may occur.
The most typical cases occur in senile macular degeneration, essentially
the result of a progressive sclerosis of the small choroidal twigs supplying the
macular region. This disease is frequently complicated by a haemorrhage
which may occupy a large area in the posterior pole of the eye, a tragedy
indicated symptomatically by a sudden failure in the central vision which
has been gradually deteriorating for some time. When a haemorrhage in
this region extravasates between the pigmentary epithelium and Bruch's
membrane the picture of disciform degeneration of the macula 3 is produced.
3. In myopia, in the atrophic process of stretching, choroidal haemor-
rhages are not unusual, especially near the macula, when, of course, their
effect on central vision is immediate and serious.” Curiously the incidence
of the lesion does not run commeasurably with the degree of myopia ;
indeed, it tends to occur as frequently, if not more so, in moderate degrees
of stretching than in cases of gross disappearance of tissue and extreme
choroidal atrophy (Fig. 1777, Plate XXXII).
4. In papilloedema choroidal haemorrhages occur as a pathological
rarity ; their clinical recognition, moreover, is rare (Bedell, 1932) since the
picture is obscured by more absorbing events. Their occurrence in this
condition would appear to have no peculiar significance.
5. Apart from these specific conditions localized choroidal haemorrhages
are met with incidentally in general disease ; usually they occur with
haemorrhages elsewhere, and frequently they indicate a serious state of
1 p. 2372. * p. 2116. 3 Vol. IV.
T.O.-WOL. III. C
2112 TEXT-BOOK OF OPHTHALMOLOGY
affairs. Such conditions include arteriosclerosis, hyperpiesia, blood
dyscrasias (pernicious anaemia, purpura, leucaemia, etc.), diabetes, and
there is a residuum of cases to which no particular cause can be assigned.
MASSIVE HAEMoRRHAges FROM THE CHoRoid are much more dramatic
occurrences but are fortunately much rarer. From a large vessel the
bleeding may be very considerable indeed, rupturing the choroid extensively,
filling up the sub-choroidal space, or detaching the retina and occupying
the space between it and the choroid (Fig. 1779). Such cases occurring
spontaneously apart from injury (such as wounds, rupture or concussion)
Fig. 1779–Sub-choroidal, H.A.MoRRHAGE.
The union between selera and choroid is preserved in the posterior segment.
(Samuels, A. of 0.)
are rare, and are confined to those in which serious vascular disease is
present. The immediate result is an acute and uncontrollable secondary
glaucoma for which the only treatment is evisceration or removal of the eye
for the relief of pain.
Such a case was reported by Demicheri (1901) in a man aged 35 in a blind
staphylomatous and degenerate eye: anatomical examination showed that an exten-
sive intra-choroidal haemorrhage had become sub-choroidal. Another case reported
by Crigler (1932) occurred in a woman of 44 with a history of bleeding: a (justifiable)
diagnosis of sarcoma was made, and pathological examination of the eye showed
hyaline degeneration of the choroidal vessels and a large haemorrhage between this

DISEASES OF THE UVEAL TRACT 2113
tissue and the retina. In this case bleeding from the socket was almost uncontrollable.
Seale (1931) reported a case in a diabetic suffering from carbuncles; acute glaucoma
developed which was followed by a ring abscess and massive sloughing of the cornea.
In cases of contusion a sub-choroidal haemorrhage is not usual, and in these hypo-
tony may exist.” Such lesions, due to a rupture of the posterior ciliary arteries, were
produced experimentally by Berlin (1873), and were examined pathologically by
Collins (1916–17): a thin layer of blood lies between the choroid and sclera, compressing
the former and producing in it an ischaemia with a consequent degeneration of the
overlying retina.
Post-operative haemorrhages, however, make up by far the most common
instances of this group of cases: a haemorrhage occurs after the globe is
opened, the bursting of the vessel being facilitated by the sudden drop in
intra-ocular pressure. Quite frequently the blood remains sub-choroidal ;
in other cases an expulsive haemorrhage occurs—the choroid is ruptured
and the flow of blood carries most of the contents of the globe with it.
Such an accident occurs in the experience of every surgeon and is one of the
most dreaded happenings in ophthalmic practice. It is met with usually in
operations for glaucoma with high tension, or during or after a cataract
extraction in an old person with high blood-pressure and frail vessels, or
alternatively, on the perforation of a corneal ulcer. In the typical case of
acute glaucoma, immediately the iridectomy is performed, the lens presents
in the wound, and is often extruded to be followed by vitreous, which
inexorably and horribly oozes out, the patient meantime undergoing
excruciating pain. If things are left the anterior chamber fills with blood
and from the widely gaping wound a mass of bloody vitreous protrudes, the
pain usually persisting till the eye is eviscerated or excised. Less severe
bleeding probably accounts for many cases of so-called “malignant
glaucoma, '' wherein operation is followed by an accentuation rather than a
relief of tension. During a cataract operation, or just after it has been
finished, more rarely several hours or even days after it has been safely
and uneventfully completed, a similar sequence occurs—an uncontrollable
welling of the vitreous into the wound, sometimes even a prolapse of the
retina and choroid, with perhaps an extravasation of blood upon the
face, all to the accompaniment of severe pain. Occasionally, particularly if
enough of the ocular contents have been extruded, a shrunken eye can be
retained, but, more usually pain, vomiting, and bleeding continue until the
eye is removed.
Not being a rarity, this condition is considerably annotated in the
literature. Cases following cataract extraction are well reviewed by Ziegler
(1926) and Lundsgaard (1928) (Collins, 1914, found 1 in 518 cases): and a
series of typical cases in glaucoma is described with pathological examina-
tions by Samuels (1931). As a rule so much damage has been done that exact
pathological interpretation is difficult. The source of the bleeding in severe
1 p. 3419.
2114 TEXT-BOOK OF OPHTHALMOLOGY
cases is the long posterior ciliary arteries which become ruptured (Fuchs,
1917; Samuels, 1931) (Fig. 1780); and the blood gathers preferentially in
the anterior segment of the eye where the choroid is quite free from the
sclera, especially on the nasal and
- temporal sides away from the anchor-
- age of the vortex veins (Fig. 1779).
In minor cases the blood becomes
encapsulated within 2 weeks, and the
haemorrhage gradually disappears
without the intervention of phago-
cytes, a fibrous capsule contracting
or collapsing over it, so that finally
its walls coalesce with the supra-
choroidea into a thick dense avascular
membrane of fibrous tissue (Fig. 1781).
Fig. 1780.-Sub-choroid AL HLEMoRRHAGE. The intense pain associated with the
Rupture of a long posterior ciliary condition is presumably due to
artery at its point of entrance into the stretching of the ciliary nerves, and
peri-choroidal space. The nerve shows the -
effects of traction. (Samuels, A. of 0.) the eventual atrophy of the eye, if
it has been retained, to destruction
of the long posterior ciliary nerves and vessels.
The prevention of such haemorrhages in intra-ocular operations is a
difficult and chancy matter. In patients with whom difficulties may be
expected to arise some recommend reduction of the blood-pressure by
Fig. 1781. Sub-choroidal, HAEMoRRHAGE.
Circumscribed thickening of the supra-choroidea as a result of a small haemorrhage
–pseudo-sarcoma of choroid. (Samuels, A. of 0.)
dieting, sedatives and free bleeding from the arm before or even during
operation; previous reduction so far as possible of the intra-ocular pressure
by miotics or even paracentesis in arteriosclerotic persons with glaucoma is
well worth while, as well as taking every care to make the surgical incision
slowly and to allow a gradual escape of aqueous humour; but despite all
precautions the accident may occur unexpectedly. A preliminary trephining


DISEASES OF THE UVEAL TRACT 21 15
of the sclera at the equator with a 2 mm. trephine may be tried in cases
where a calamity may be expected, for example, if it has already occurred
in the other eye (Averbach, 1936); not only is this valuable by reason
of the reduction in tension which it brings about, but theoretically it might
provide an innocuous outlet for the blood if bleeding did occur. Once
having occurred, if the haemorrhage remains sub-choroidal, the retention of
the eye is rare. The first case reported wherein an eye with some vision
was saved after an extensive sub-choroidal haemorrhage was that of
Verhoeff (1915); the accident occurred after the performance of a sclerec-
tomy for glaucoma and the eye was saved by puncturing the sclera near the
equator to evacuate the blood : a similar attempt to combat the same
accident in the other eye at a later date, however, was unsuccessful (Verhoeff,
1931). In such cases there is nothing to lose ; and the value of this drastic
procedure was demonstrated by Vail (1938) in 2 cases, one after a cataract
and the other after a glaucoma operation wherein prompt action in perform-
ing a large posterior sclerotomy saved what would otherwise have been an
ophthalmic wreck. In most cases sacrifice of the eye is necessary for the
relief of pain ; and on the whole probably evisceration is safer than excision,
for violent haemorrhage after the latter operation may be difficult to control
and may result in necrosis owing to extravasation under pressure in the
loose tissue of the orbit and lids.
Averbach. Sov. vestm. Oft., viii, 651, 1936. Lundsgaard. Acta O., vi, 251, 1928.
Bedell. A. of O., viii, 186, 1932. Meller. Z. f. Aug., lvii, 131, 1925.
Benedict. A. of O., iv, 588, 1930. Moore, Foster. T. O. S., xxx, 165, 1910.
Berlin. K. M. Aug., xi, 42, 1873. Nettleship. T. O. S., xxix, 134, 1909.
Collins. R. L. O. H. Rep., xiii, 166, 1890. Samuels. A. of O., vi, 840, 1931.
T. O. S., xxxiv, 41, 1914; xxxvi, 204, 1916; Seale. Brit. J. O., xv, 514, 1931.
xxxvii, 112, 1917.
Corrado. A n. di Ott., lxii, 1009, 1934.
Crigler. A. of O., viii, 690, 1932.
Delord.
Demicheri. A. de Oft., H.-A., i, 573, 1901.
Ellett. A. of O., xxi, 497, 1939.
Fuchs. Z. f. Aug., lvii, l, 1925.
T. B. of Ophthal., 1917.
Hepburn. T. O. S., xxx, 82, 103, 1910.
Bull. S. d’O. Paris, xlvii, 202, 1935.
Sédan, Jean. Bull. S. fr. d’O., xliii, 222, 1930.
Vail. Am. J. O., xxi, 256, 1938.
Venneman. Encyc. franç. d’Ophtal., Paris,
vi, 1, 1906.
Verhoeff. O. Rec., xxiv, 55, 1915.
A. of O., vi, 851, 1931.
Werner and Adlercreutz.
lxxiii, 883, 1931.
Ziegler. Contrib. to Oph. Science. Wis., 1926.
Finska laik. handl.,
Löwenstein. K. M. Aug., lvi, 583, 1917.
DISCIFORM DEGENERATION OF THE MACULA
(SENILE MACULAR EXUDATIVE CHOROIDITIS)
DISCIFORM DEGENERATION OF THE MACULA is a condition, frequently
bilateral, characterized ultimately by the development of a localized mass
of organized tissue situated under the retina. Such a picture may have a
composite aetiology ; but one proved cause is the occurrence of a haemorrhage
from the chorio-capillaris extravasating between Bruch's membrane and the
pigment epithelium. In general terms the cases may be divided into two
classes, senile and juvenile ; in the first the haemorrhage is usually due to
sclerotic changes in the choroid ; in the second its aetiology is obscure.
2116 TEXT-BOOK OF OPHTHALMOLOGY
The term disciform degeneration of the macula was first used by Oeller (1905),
but cases of a similar nature had been described previously (Pagenstecher, 1875;
v. Michel, 1877; Walker, 1897; Yarr, 1899 ; Silcock, 1899 ; Jessop, 1903;
Batten, 1904; Doyne, 1904). During the next 20 years sporadic reports appeared of
the same lesion under different titles—Senile macular changes in arteriosclerosis
(Possek, 1905), tumour-like swelling at the macula (Lawford, 1911 ; Beatson Hird, 1916;
Elschnig, 1919), senile macular eacudative retinitis (Axenfeld, 1915; Hegner, 1916;
Holm, 1917; Knapp, 1919; Coppez and Danis, 1923; Feingold, 1924; Wölfflin,
1926; Davenport, 1926)—until eventually the condition was established as a clinical
entity by Junius and Kuhnt (1926) in an exhaustive monograph. Since that time
the term disciform degeneration of the macula has been retained by the great majority
of writers, and the names of Junius and Kuhnt have been closely associated with it
(Cords, 1925; Ormond, 1927; Paul, 1927; Cushman, 1928; Weizenblatt, 1928;
Holloway and Verhoeff, 1928; Barrière, 1929; Hanssen, 1930; Pallarés, 1931;
Behr, 1931; Duynstee, 1932; Adler, 1933; Soudakoff, 1934; Kahler and O’Brien,
1935; Davis and Sheppard, 1935; Verhoeff and Grossman, 1937; and others).
The typical case develops in a patient over middle life with arterial disease ;
it commences with a haemorrhage at the macula and terminates with the presence
of a tumour-like mass of organized tissue at the macular region which abolishes central
vision. Cases presenting a very similar ophthalmoscopic picture are described in the
literature occurring in persons under 40 years, which differ from the senile type in
the absence of gross haemorrhages, and the tendency to ultimate resolution with
almost complete disappearance of the lesion and the restoration of vision. Systematized
attention was first called to these by Junius (1929) and later by Davis and Sheppard
(1935), under the title of Juvenile macular eacudative retinitis, the assumption being
that the trouble was retinal in origin. In 1937, however, a significant observation
was made by Verhoeff and Grossman—a case occurring in a man of 63, free from
arteriosclerosis but with some anaemia and numerous telangiectases in the skin,
who retained good vision in the presence of the lesion (and thus resembled the juvenile
type), showed on pathological examination, instead of the sub-retinal haemorrhage
characteristic of the senile lesion, an exudate of serum. They advanced the hypothesis,
therefore, that the senile and juvenile types of lesion are extremes in a series in which
intermediate forms exist, the first being a frank haemorrhage from the chorio-capillaris
involving permanent effects occurring in persons with arterial disease, the second being
an exudation of serum dependent on Some more temporary cause and occurring in
tissues capable of showing a more active response. It is to be remembered that this
is a hypothesis resting as yet on circumstantial evidence ; but in the absence of a
more reasonable and proven explanation, we shall accept it and treat the two conditions
in association.
SENILE DISCIFORM DEGENERATION OF THE MACULA
While senile disciform degeneration of the macula is not a common
condition, it is commoner than would be supposed from the fact that only
some 130 cases have been reported in the literature ; Kahler and O’Brien
(1935), for example, met 12 cases in their clinic in one year, and Davenport
(1926) gathered a series of 17. It is definitely associated with age, occurring
most commonly in the sixth decade ; the youngest recorded case was 39
(Neame, 1923) and the oldest 83 (Kahler and O’Brien, 1935). Males are
affected more frequently than females (10 : 7), most of the patients have
arterial disease, while hypertension, nephritis, diabetes and occasionally
DISEASES OF THE UWEAL TRACT 2117
Fig. 1782.-DiscIFoRM DEGENERATIon of MACULA : EARLY HAEMoRRHAGIC STAGE.
(Kahler and O’Brien, A. of 0.)
Fig. 1783.−DiscIFoRM DEGENERATIon of MACULA : LATE FIBRous STAGE.
(Kahler and O'Brien, A. of 0.)


21 18 TEXT-BOOK OF OPHTHALMOLOGY
syphilis (3 cases) may be accompaniments. In rather more than half the
cases the condition is bilateral, and in a few the second eye has been involved
after the first has been observed.
The clinical symptoms are entirely confined to the visual phenomena—a
gross impairment or, more usually, a complete abolition of central vision,
occasionally with a central positive Scotoma (Axenfeld, 1915; Junius and
Kuhnt, 1926). Since most patients have come under observation when the
lesion is far advanced, the usual mode of onset is difficult to determine :
on the one hand a loss of vision remarked suddenly may have come on
gradually unnoticed, and on the other a gradual loss of vision may have
been due to precedent sclerotic changes at the macula. In quite a proportion
of cases, however, the onset has been sudden and a sub-retinal haemorrhage
has been observed at the first examination (Wolff, 1931 ; and others), while
in others metamorphopsia or other central disturbances have been noted
(Coppez and Danis, 1926; Junius and Kuhnt, 1926). In all cases the loss
of vision has been permanent, and in none has any noteworthy improvement
occurred.
The clinical picture, if seen in the early stages, is usually characterized
by a sub-retinal haemorrhage in the central region (Fig. 1782) while other
haemorrhages—retinal and sub-retinal—may be present in the fundus. As a
very early preliminary sign François (1936) noted minute capillary dilata-
tions at the macula before the onset of the first subjective symptoms. The
macular haemorrhage raises the retina into a mound usually, but not always,
of a disc-like shape, varying from one-half to several times the size of the
optic disc, but always located within the region of the superior and inferior
temporal vessels. Over the mound the retinal vessels are always visible, and
occasionally arterio-venous communications are present. At first it is grey,
green, or black in colour, but later it becomes white (apart from pigmentary
deposits), and as it does so it usually diminishes in size ; but the margins
always remain distinct. This ultimate appearance of a white, raised, sharply
circumscribed mass, is of course, permanent : it may reach an elevation of
5 or 6 dioptres, the surface may be irregular and dotted with haemorrhages
and pigment, and over it the retina is transparent and usually applied
closely to it, with occasionally the appearance of some fluid between it and
the scar tissue. The shape is by no means always discoid but may assume
quite irregular forms (Figs. 1783–6).
Associated appearances in the fundus, as one would expect in the type
of patient affected, are common. Arteriosclerosis of the retinal arteries is
not common (about 20% of cases), but the frequent occurrence of senile
changes of a degenerative nature at the other macula betrays choroidal
sclerosis—pigmentary changes, drusen, white exudative spots, and so on.
In all the unilateral cases which have been observed to become bilateral the
second eye showed such changes, so that they may well be a constant
precursor, Haemorrhages, both near the lesion and in other parts of the
DISEASES OF THE UWEAL TRACT 2119
Fig. 1784.—DiscIForM DEGENERATION of MACULA.
(Kahler and O'Brien, A. of 0.)
Fig. 1785. Disciformſ DEGENERATION of MACULA.
(Kahler and O’Brien, A. of 0.)


2120 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 1786.-Disciform DEGENERATIon or Macula.
(Kahler and O’Brien, A. of 0.)
Fig. 1787.-Disciform DEGENERAtion of Macula.
Showing circinate changes. (Kahler and O'Brien, A. of 0.)


DISEASES OF THE UVEAL TRACT 2121
fundus, are common. White spots of a degenerative or exudative character
are also common and may be found in any degree up to a frank circinate
retinopathy," a condition which may precede or follow the disciform lesion, or
may co-exist when it has been first discovered, or may exist in the fellow eye
(Doyne, 1904; Neame, 1923; Davenport, 1926; Lauber, 1926; Seefelder,
1928; Kuniya, 1931 ; Behr, 1931 ; Kahler and O’Brien, 1935; and many
Others) (Fig. 1787). Angioid streaks 2 are a more unusual concomitant but
their association is significant (Feingold, 1924; Coppez and Danis, 1926;
Wildi, 1926; Bonnet, 1935).
Once the tumour has formed little change usually takes place ; the
mass remains relatively constant, adding, perhaps, a few patches of pigment
or haemorrhage, or further exudative areas around its edges or arranged in
a circinate distribution. Apart from the permanent loss of central vision,
therefore, the prognosis is good, quite different, for example, from exudative
retinitis (of Coats) wherein a spread to form a pseudo-glioma, retinal detach-
ment, and secondary glaucoma is the rule. Cases of secondary glaucoma
have, it is true, been reported (Coppez and Danis, 1923; Davenport, 1926),
but these are so rare as to be incidental.
Diagnosis. The main points in the diagnosis of this condition are the size and
situation of the elevated mound, the absence of an extensively detached retina, the
frequent co-existence of sub-retinal haemorrhages and of retinal exudates of the nature
of circinate retinopathy, and the absence of inflammatory signs. The crucial point
in diagnosis is, of course, a malignant melanoma, but if these characteristic signs are
present enucleation of the eye should be delayed. The only other conditions of
importance with which difficulty may arise are the eacudative retinitis of Coats and
conglomerate twbercle. The former, however, occurs in young patients and is rarely
confined to a small central area. If it is so, in the absence of a guide from associated
sub-retinal haemorrhages an ophthalmological distinction may be impossible ; but
fortunately the lack of a specific diagnosis makes no difference to the conduct of the
case. Apart from the diagnostic signs of the degenerative lesion, tubercle is usually
differentiated by its diffuse inflammatory appearance with softer edges, by its systemic
diagnosis, and by its rapid course leading to destruction of the eye.
Pathology. Several eyes showing this condition have come under
pathological examination, most of them being removed under the diagnosis
of a tumour (v. Michel, 1877; Axenfeld, 1915; Hird, 1916; Knapp, 1919;
Elschnig, 1919; Wölfflin, 1926; Paul, 1927; Holloway and Verhoeff,
1928; Seefelder, 1928 ; Hanssen, 1930; Behr, 1931 ; Verhoeff and Gross-
man, 1937). The most recent cases are those of Verhoeff and Grossman
(1937) and Braun (1937) who are essentially in agreement. The mass is
a large haemorrhage between the pigmentary epithelium and Bruch's
membrane which had extravasated through a ragged tear in the latter from
a rupture in the chorio-capillaris (Figs. 1788–9). Gradually the haemorrhage
becomes organized, being permeated with finely fibrillated connective
1 p. 2760. * p. 24.13.
2122 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 1788–DiscipoRM Degeneration.
Hemorrhagic extravasation in macular region beneath pigment epithelium. The
latterisintact except for obviously artificial breaks. At the summit of the mound is a
small amount of serum between the pigment epithelium and the retina. x 9.
(Verhoeff and Grossman, A. of 0.)
Fig. 1789.-Disciroºm DEGENERATIow.
Rupture of the chorio-capillaris near the periphery of the haemorrhagic extra-
vasation under the pigment epithelium. x 4s. (Verhoeff and Grossman, A. of ol)
Fig. 1790.-Discipoºm Degeneration.
Section showing hºmorrhagic extravasation at the fovea. The pigment epithelium
posteriorly is of new formation and separated from Bruch's membrane by connective
tissue. X 13. (Verhoeff and Grossman, A. of on




DISEASES OF THE UVEAL TRACT 2123
tissue (Figs. 1790–1). In the meantime the pigmentary epithelium prolife-
rates forming large numbers of spindle cells, which indeed make up a great
proportion of the mass of the mound ; this has been an invariable event in
all the cases examined. Behr (1931) found elastic fibres which appeared to
have been derived from the lamina vitrea. Into the mass run small blood-
vessels from the choroid through breaks in Bruch's membrane : only
occasionally a capillary enters from the retina. More minute breaks in this
membrane admit fibroblasts from small collections in the choroid under-
neath. All observers agree that in the later stages the mound is thus formed
of a mass of fibrous tissue and proliferated pigment cells organized essentially
from the choroid. Further changes involve hyaline degeneration (Verhoeff
-
Fig. 1791.-DiscIFoRM DEGENERATION.
Showing large masses of old blood undergoing organizations in which strands of
pigment epithelium take part. Bruch's membrane is intact. x 44 (Verhoeff and
Grossman, A. of 0.)
and Grossman, 1937), the formation of cartilage (v. Michel, 1877) and even
of bone (Axenfeld, 1915; Wölfflin, 1926). Excepting general sclerotic
changes (Elschnig, 1919; Holloway and Verhoeff, 1928; Behr, 1931), the
remainder of the choroid is usually normal apart from occasional cellular
and exudative accumulations (Paul, 1927; Hanssen, 1930); and although
the retina over the mass shows degenerative and atrophic changes, these
appear to be secondary.
AEtiology. The origin of the lesion has been located in different places
by different observers. The blood vessels of the retina were considered to be
at fault by some (Axenfeld, 1915; Elschnig, 1919; Junius and Kuhnt,
1926; Ormond, 1927; Duynstee, 1932; and others); the membrane of
Bruch by others (v. Michel, 1877; Behr, 1931); but most observers agree
that the choroid is the source of the disturbance. An inflammatory origin
was postulated by Pagenstecher (1875), Axenfeld (1915), Hegner (1916),
Neame (1923), Coppez and Danis (1926) and others, and that this may

2124 TEXT-BOOK OF OPHTHALMOLOGY
*~.
sometimes be the ease is suggested by the finding of Paul (1927) of an
exudative choroiditis breaking through the membrane of Bruch, and of a
lymphocytic infiltration in the choroid by Hanssen (1930). It would seem,
however, that in most cases the mass originates from a sub-retinal haemor-
rhage in the absence of inflammatory changes, and the most probable cause
seems to be sclerotic changes in the chorio-capillaris (Possek, 1905; Cords,
1925; Pallarés, 1931; Holloway and Verhoeff, 1928; Soudakoff, 1934 ;
Verhoeff and Grossman, 1937). Hepburn (1913) suggested a thrombotic
lesion in the chorio-capillaris. As has been seen, the macular region has a
specially differentiated vascular bed in the chorio-capillaris, which may be
affected alone or in association with widespread disturbances elsewhere in
the choroid and retina, a circumstance which makes it likely that the aetiology
of disciform degeneration is essentially the same whether or not it is
associated with other conditions such as circinate retinopathy. These two
conditions may well occur together with no mutual relationship as to cause
and effect, but rather as collateral events depending on a common cause—
choroidal and retinal sclerosis. On the other hand, it is quite possible for a
lesion of angiosclerosis to be localized to this particular area ; and again,
although the age and condition of the majority of the patients suggest an
association with a sclerotic lesion, the circulatory disturbance may possibly
be of a different nature, and may, indeed, be temporary and vary in different
Ca,SéS.
JUVENILE DISCIFORM DEGENERATION OF THE MACULA
(JUVENILE EXUDATIVE MACULAR CHOROIDITIS)
Cases of a somewhat similar lesion to senile disciform degeneration of
the macula occur in younger people in the second and third decades ; they
are characterized by the same ophthalmoscopic appearance of a sub-retinal
exudative-like mass having degenerative rather than inflammatory charac-
teristics, but are differentiated by the fact that the lesion tends to resolve
leaving little impairment of vision.
Attention was first called to them by Junius (1930) under the title of
juvenile eacudative macular retinitis. Somewhat similar cases were discussed
by de Schweinitz (1926), and Davis and Sheppard (1935). When the lesion
is at its height, vision is poor, but practically complete healing may occur
in from 2 to 6 months. No pathological examinations have been made,
and Junius considered that the lesion was due to a vascular disturbance in
the retina. As we have just seen, however, the pathological examination
of a case in an old man by Verhoeff and Grossman (1937) (Figs. 1792–3),
which appeared and behaved clinically in a way identical with that
characteristic of the juvenile type suggests very strongly that the aetiology
is similar. This case occurred in a non-arteriosclerotic person, and the
DISEASES OF THE UVEAL TRACT 2125
lesion consisted of a sub-retinal serous exudate derived from the choroid
with the formation of a minimal amount of fibrous tissue. The juvenile
type is therefore probably due to some vascular disturbance in the chorio-
capillaris in this region which causes an extravasation, mainly of serum,
between the pigmentary epithelium and Bruch's membrane. The cause
Fig. 1792.-Juvenile. Disciform DEGENERATION.
Pigment epithelium elevated by serous exudate. x 10. (Verhoeff and
Grossman, A. of 0.)
Fig. 1793. –Juvex ILE Disciformſ DEGENERATIon.
Connective tissue formation between pigment epithelium and Bruch's membrane
derived from fibroblasts that have penetrated the latter. x 45. (Verhoeff and
Grossman, A. of 0.)
of the disturbance is obscure ; it is presumably a temporary oedema caused
by a vaso-neurosis of systemic or toxic origin, but probably varies in
different cases.
Adler. A. of O., x, 379, 1933. Davenport. T. O. S., xlvi, 137, 1926.
Axenfeld. A. f. O., xe, 452, 1915. Davis and Sheppard. A. of O., xiii, 960, 1935.
Barrière. A. de Oft. B.-A., iv, 223, 1929. Doyne. T. O. S., xxiv, 91, 1904.
Batten. T. O. S., xxiv, 127, 1904. Duynstee. K. M. Aug., lxxxviii, 511, 1932.
Behr. Z. f. Aug., lxxv, 216, 1931. Elschnig. K. M. Aug., lxii, 145, 1919.
Bonnet. A. d’O., lii, 225, 1935. Feingold. T. Am. O. S., xxii, 268, 1924.
Braun. A. f. Aug., ex, 534, 1937. François. Bull. S. fr. d’O., xlix, 226, 1936.
Coppez and Danis. A. d’O., x1, 129, 1923; Hanssen. Z. f. Aug., lxxii, 360, 1930.
xliii., 461, 1926. Hegner. K. M. Aug., lvii, 27, 1916.
Cords. B. O. G., Heidel.., xlv. 246, 1925. Hepburn. T. O. S., xxxiii, 86, 1913.
Cushman. Am. J. O., xi, 720, 1928. Hird, Beatson. T. O. S., xxxvi, 345, 1916.


2126
TEXT-BOOK OF OPHTHALMOLOGY
Holloway and Verhoeff. T. Am. O. S., xxvi,
206, 1928.
Holm. K. M. Aug., lix, 319, 1917.
Jessop. T. O. S., xxiii, 384, 1903.
Junius. Z. f. Aug., lxx, 129, 1930.
Junius and Kuhnt. Die Scheibenförmige
Entartung d. Netzhautmitte, Berlin, 1926.
Rahler and O’Brien. A. of O., xiii, 937, 1935.
Rnapp. A. of O., xlviii, 559, 1919.
Kuniya. Acta S. O. Japan, xxxv, 1156, 1931.
Lauber. Z. f. Aug., lx, 214, 1926.
Lawford. T. O. S., xxxi, 257, 1911.
v. Michel. A. f. O., xxiv. (I), 131, 1877.
Neame. P. R. S. Med., xvi, 11, 1923.
Pallarés. K. M. Aug., lxxxvi, 201, 1931.
Paul. Z. f. Aug., lxiii, 205, 1927.
Possek. Z. f. A ug., xiii, 771, 1905.
de Schweinitz. Atlantic Med. J., xxxi, 818,
1928.
Seefelder. A. f. O., czz, 139, 1928.
Silcock. T. O. S., xix, 69, 1899.
Soudakoff. Chinese Med. J., xlviii, 981, 1934.
Verhoeff and Grossman. A. of. O., xviii, 561,
1937.
Verhoeff and Sisson. A. of O., lv., 125, 1926.
Walker. T. O. S., xvii, 64, 1897.
Weizenblatt. Z. f. Aug., lxv, 299, 1928.
Wildi. K. M. Aug., lxxvi, 177, 1926.
Oeller. Atlas, Wiesbaden, 1905. Wolff. P. R. S. Med., xxiv, 890, 1931.
Ormond. Guy’s H. Rep., lxxvii, 16, 1927. Wölfflin. A. f. O., cxvii, 33, 1926.
Pagenstecher. Atlas, Wiesbaden, 1875. Yarr. T. O. S., xix, 68, 1899.
III. INFLAMMATIONS OF THE UVEAL TRACT : UVEITIS
We have already noted that although one or other of the sections of the uveal
tract may be inflamed separately, and that while there is a considerable degree
of individuality in the circulation to the anterior and the posterior areas differentiating
to some extent the iris, ciliary body and anterior choroid from the posterior region
of the choroid, and therefore a considerable degree of pathological localization in blood-
borne diseases into the two categories of irido-cyclitis and choroiditis, yet the anato-
mical, physiological and pathological continuity of the whole tract is so profound
and fundamental that these conditions are best considered as a whole. In this section
we shall therefore discuss the aetiology, pathology, symptomatology and therapeusis
of uveitis generally and then pay particular attention to such affections as have
specific individuality, pointing out as we go characteristics which have a regional
value.
A. General Considerations
The General AEtiology of Uveitis
An aetiological classification of the inflammatory diseases of the uveal
tract is a matter of considerable difficulty, not least because our knowledge
has by no means progressed sufficiently far to render the task possible ;
even to-day the cause of many clinical conditions is disputed, and in other
cases is quite unknown. One disconcerting factor is that this tissue shares
profoundly in a host of general systemic diseases, and in many cases its
reaction is nondescript and non-specific, so that a study of the clinical
picture provides little help in differentiation. Be that as it may, inflam-
matory conditions may be considered provisionally in certain categories.
1. ExoGENOUs INFECTIONs, derived from without, induced either by
(a) organismal infection introduced through a perforating injury or
ulcer of the cornea or sclera (wound infection);
(b) intra-ocular parasites ;
(c) poisonous chemical, animal or vegetable material.
DISEASES OF THE UVEAL TRACT 2127
2. SECONDARY INFECTIONS affecting the uveal tract by continuity or
arising from some ocular event.
(a) Uveitis of an infective nature by direct continuity from
(1) An ocular inflammation affecting the cornea, the sclera, the
retina, or the optic nerve.
(2) A peri-ocular inflammation in the orbit or meninges.
(b) Uveitis of a toxic or allergic nature associated with unusual ocular
products in the eye :
(1) With a neoplasm.
(2) With an endocular haemorrhage.
(3) In a degenerated eye : atrophic uveitis.
(4) With a detached retina.
(5) With lens protein : endophthalmitis phaco-anaphylactica.
(6) With uveal pigment. . . . . . . . -
(c) Traumatic irido-cyclitis.
(d) Sympathetic ophthalmitis.
(e) Uveitis through nerve involvement.
(1) Through the trigeminal : herpes. -
(2) Through the sympathetic : heterochromic irido-cyclitis.
3. ENDOGENOUS INFECTIONS, the uveal tract being affected primarily
from some cause operative elsewhere in the body.
(a) Inflammations due to organismal metastases.
(1) Acute suppurative metastatic lesions.
(2) Granulomatous lesions; tubercle, syphilis, leprosy.
(3) Virus infections.
(4) Fungus infections.
(b) Inflammations due (presumably) to Organismal allergy or toxic
influences.
(c) Inflammations associated with generalized febrile illnesses.
(1) The exanthemata ; measles, scarlet fever, small-pox,
chicken-pox.
(2) Acute intestinal infections : typhoid fever, cholera,
dysentery. -
(3) Blood infections : malaria, relapsing fever, Weil's disease.
(4) Respiratory infections: influenza, whooping cough.
(5) Cerebro-spinal infections : meningitis.
(6) Infections of the salivary glands : mumps, uveo-parotitis.
(d) Inflammations associated with diseases of the skin.
(e) Inflammations associated with metabolic disturbances (rheumatism,
gout, diabetes, nephritis, auto-intoxication, etc.).
1. ExOGENOUS INFECTIONS
(a) Eacogenous inflammations, caused by the introduction of organisms
through an infected perforating wound or a perforated corneal or scleral
T. O. —WOL. III. D
2128 TEXT-BOOK OF OPHTHALMOLOGY
ulcer are usually of grave significance. It is to be remembered that all
inflammatory conditions in such circumstances are not necessarily due to
organismal infection, for, as we shall see, a non-infective traumatic uveitis
may develop," or an inflammation due to the presence of unusual protein
material or a sensitivity thereto.” If, however, an actual infection does
occur, it may on occasion be confined to the anterior segment when a severe
PLASTIC IRIDO-CYCLITIS witH HYPOPYON usually results; if it is introduced
into the vitreous cavity suppuration may be confined to the posterior segment
(ENDOPHTHALMITIs) to be followed by atrophy; but if, as is usually the case,
the organisms or their toxins diffuse throughout the globe the picture of
PANOPHTHALMITIS ensues with its severe local and general symptoms,
entailing loss of the eye either by fibrosis and atrophy or by acute suppura-
tion and even rupture of the globe followed by phthisis bulbi. Particularly
is this so if the bacteria have access to lens substance for there seems no
doubt that this forms a particularly good culture medium both in vitro and
in vivo, not only for the more usual pyogenic Organisms, but also for mild
or non-pathogenic spore-bearing types, or such surface parasites as the
staphylococcus epidermidis or the xerosis bacillus which as a rule excite little
reaction (Lindner, 1914–24; Morax and Chiazzaro, 1927; Orloff, 1928;
Levitova, 1929 ; Santonastaso, 1930). As a very rare event, if such an
exogenously infected eye is not excised a generalized infection may follow
with pyaemic manifestations in other parts of the body (Deggeller, 1929).
It is interesting that the eye usually survives infection on the surface
of the vitreous, as occurs, for example, in a perforating wound into which
the vitreous prolapses, but if the organisms are introduced into the substance
of the gel, the infection runs on apace. Thus it has been shown experi-
mentally that if virulent organisms (B. pyocyaneus, Gray, 1933) are
introduced into the vitreous body, within 2 hours there is extensive engorge-
ment of the choroid, and very soon thereafter a leucocytic and eventually a
lymphocytic and histiocytic infiltration permeates the retina, until a layer
of pus surrounds the entire vitreous cavity interposing itself between the
gel and the retina. Within 24 hours a panophthalmitis may develop in acute
cases. With such organisms as the typhoid or cholera bacilli, even if
introduced into the vitreous in minute quantity, not only may panophthal-
mitis develop, but general infection and death (Axenfeld, 1894; de
Grandmont and Gasparrini, 1895). In clinical conditions an infection in
the vitreous usually attacks the retina first at its anterior and posterior
extremities, that is, at the pars ciliaris retinae and around the optic nerve
head (Fuchs, 1904); thence the inflammation spreads throughout the
retina, setting up a diffuse and intense suppurative retinitis.” Sometimes
the retina becomes detached at an early stage, and in this event the choroid
may escape for a long time, being attacked by continuity from the two
extremities whence the inflammatory process may spread relatively slowly.
1 p. 2135. * p. 2132. * p. 2637.
DISEASES OF THE UVEAL TRACT 2129
The organisms usually responsible for the most virulent cases are the pneumococcus,
the streptococcus, and the staphylococcus (Elschnig, 1915–21; Morax, 1921), more
rarely the bacillus pyocyaneus (McNab, 1904; Mauresberg, 1910; Schneider, 1926;
Safar, 1927; Garretson and Cosgrove, 1927; Shearer, 1935), the bacillus coli (Randolph
1893; Cistjakov, 1914; Pascheff, 1914; Morax and Coppez, 1926), or members of the
proteus group (Hanke, 1903). It is to be remembered also that even otherwise mild
Saprophytes or feebly pathogenic organisms if introduced into the eye may produce a
like result, as in the case of the bacillus subtilis (Hoppe, 1907; Kodama, 1910;
Butler, 1914; Greenspon, 1918; Flieringa, 1921 ; Gifford and Hunt, 1929; Hoffman,
1933), the diplobacillus of Morax Axenfeld or the ozena bacillus (Rosenhauch, 1914),
B. fluorescens (Horvath, 1923), the xerosis bacillus (Lindner, 1914–24; Scheffels, 1929)
or a pseudo-diphtheria bacillus (Baumeister, 1916).
Post-operative Infections. The most frequent instance of post-operative
infection in Ophthalmic surgery is after cataract extraction, a subject
which will be discussed more extensively in a subsequent volume. Such
an accident may occur despite the utmost precaution, although the cause
may not always be exogenous infection.
Maitland ‘Ramsay’s (1921) figures are typical—68 cases in 2,146 operations
(3.16%) of which 46 eyes (2.14%) were lost, i.e., the very high percentage of 67.7
of the infected eyes. In a series of 470 patients Lindner (1914) found 12 cases of post-
operative infection (2.5%) : 6 had irido-cyclitis and cleared up, 3 had hypopyon, of
which 2 eyes were lost, and the remaining 3 developed a destructive panophthalmitis.
It is to be noted that while such conditions usually occur at the time
of the infliction of the wound or soon thereafter, late infections may occur
in cases when a badly healed or fistulous wound remains or when a prolapse
of some uveal tissue exists. Most of these late infections complicate opera-
tions undertaken for glaucoma when a fistulous scar is intentionally made ;
they are due in most cases to the entry of the organisms responsible for an
incidental intercurrent conjunctivitis which obtain access into the eye
through a small defect in the conjunctival flap covering the fistula or
through its attenuated and devitalized tissues. When such cases do occur,
however, the prognosis, as in all exogenous infections, is not good, while,
if the eye survives, the fistulous opening frequently becomes closed and the
operation therefore rendered useless.
Different authors vary in their estimates of this occurrence : some are very
pessimistic (Pillat, 1921, who puts the percentage as 18-2 ; Meller, 1931), but in the
practice of the majority the incidence is under 1% (Butler, 1921, 3 in 150 cases;
Wessely, 1927, none in 134 cases ; I have seen 2 cases, both after a staphylococcal
conjunctivitis, in a series of 1,000 trephines, one eye being lost and the other retained
but atrophic). Reviewing the literature of 252 cases, Scardapane (1926) found late
infections to follow in 2.27% of Elliot's trephining and 1.54% of Lagrange's
Sclerectomy ; 44% retained a useful eye and in only 5% was the vision unimpaired.
Reviewing 536 trephining operations performed at Moorfields Hospital, Davenport
(1926–27) found 14 cases of late infection of which 6 retained good vision and 2
required evisceration ; and in a later study of the literature involving an analysis of
5,616 trephines, Erola (1934) found that the estimates of different surgeons varied
irregularly between 0 and 13.6%, while the average in iridencleisis was 0.3%.
D 2
2130 TEXT-BOOK OF OPHTHALMOLOGY
(b) The introduction of parasites (cysticercus, etc.) into the eye usually produces
an irritative and intractable uveitis so long as they remain. The reaction is due to
the toxins which they liberate, a question which will be considered at a later stage.”
(c) Chemical substances of poisonous nature can produce a widespread uveal
inflammation, as witness the violent and frequently destructive process which is asso-
ciated with extensive corneal necrosis by burns with acids, alkalis, or lime. The
absorption of poisonous animal substances may produce effects of a similar nature.
Thus the sting of bees, wasps, beetles or spiders may produce an irido-cyclitis with
hypopyon in association with a keratitis (Langer, 1897; Kraupa, 1911; Huwald,
1904; Koyanagi, 1920; Lundsgaard, 1928). Irritative plant juices also may have a
like effect, such as that of the Euphorbiaceae (croton oil) (Lewin and Guillery, 1905;
Samperi, 1917; Krautschneider, 1918, and others). These conditions have already
been studied in a previous chapter.” Finally the hairs of caterpillars and some plants
are in a peculiar case in that they excite a distinctive localized lesion—OPHTHALMIA
NODOSA or IRIDO-CYCLITIS PSEUDO-TUBERCULOSA. This condition has also been
discussed “and will be noted again subsequently.
Axenfeld. A. f. O., xl (3), 1 ; (4) 103, 1894. Langer. A. f. eacp. Path. Pharm., xxxviii,
Baumeister. K. M. Aug., lvii, 539, 1916. 381, 1897.
Butler. P. R. S. Med., xiv, 51, 1921. Levitova. Izv. sev. Kavkast. Univ., i, 20,
Butler, Harrison. Ophthalmoscope, xii, 13, 1929. cº
1914. Lewin and Guillery. Wirkungen, d. Arznei-
Cistjakov. Vestn. O., xxxi, 3, 1914. mitteln w. Giften auf d. Auge, Berlin,
1905.
Lindner. A. f. O., lxxxviii, 415, 1914.
Z. f. Aug., liii, 305, 1924.
Davenport. Brit. J. O., x, 474, 1926.
T. O. S., xlvii, 283, 1927.
Deggeller. K. M. Aug., lxxxiii, 483, 1929.
IClschnig. A. f. O., lxxxix, 502, 1915; cv,
599, 1921.
Z. f. Aug., xliii, 309, 1920.
Erola. Acta O., xii, 137, 1934.
Flieringa. K. M. Aug., lxix, 241, 1921.
Am. J. O., viii, 70, 1925.
Fuchs. A. f. O., lviii, 391, 1904.
Garretson and Cosgrove. J. Am. Med. 4s.,
lxxxviii, 700, 1927.
Gifford and Hunt. A. of O., i, 494, 1929.
de Grandmont and Gasparrini. Am. di Ott.,
xxiv, 343, 1895.
Gray. Brit. J. O., xvii, 15, 1933.
Greenspon. Am. J. O., i, 316, 1918.
Hanke. Z. f. Awg., x, 373, 1903.
R. M. Aug., xliii (1), 724, 1905.
Hoffman. Z. f. Aug., lxxx, 124, 1933.
Hoppe. B. O. G. Heidel., xxxiv, 343, 1907.
Horvath. Mag. Orv. A., xxiv, 121, 1923.
Huwald. A. f. O., lix, 46, 1904.
Rodama. K. M. Aug., xlviii, 624, 1910.
Royanagi. K. M. Aug., lxv, 854, 1920.
Rraupa. Cb. pr. Aug., xxxv, 321, 1911.
Rrautschneider. Wien. med. W., xxxi, 1146,
1918.
. Pillat.
Lundsgaard. Acta O., vi, 181, 1928.
Mauresberg. Z. f. Aug., xxiv, 299, 1910.
McNab. K. M. Aug., lxii (1), 54, 1904.
Meller. Augenártztliche Eingriffe, Wien, 1931.
Morax. T. O. S., xli., 375, 1921.
Morax and Chiazzaro. An. d’Oc., clxiv, 241,
1927.
Morax and Coppez. An d'Oc., clxiii, 596,
1926.
Orloff. Russ. O. J., vii, 281, 1928.
Pascheff. An. d’Oc., clii, 44, 1914.
K. M. Aug., lxvi, 525, 1921.
Ramsay. T. O. S., xli., 387, 1921.
Randolph. A. of O., xxvi, 379, 1893.
Rosenhauch. Postep. Okul., 1, 1914.
Safar. Z. f. Aug., lxi, 25, 1927.
Samperi. A. di Ott., xxiv, 265, 1917.
Santonastaso. Boll. Acad. pugl. Sci., v, 165,
1930.
Scardapane. Boll. d’Oc., iv, 866, 1926.
Scheffels. K. M. Aug., lxxxiii, 117, 1929.
Schneider. K. M. Aug., lxxvii, 103, 1926.
Shearer. A. of O., xiii, 447, 1935.
Wessely. K. M. Aug., lxxviii, 80, 1927.
2. SECONDARY INFECTIONS
Secondary inflammations affect the uveal tract by direct spread from
the neighbouring tissues, or arise as a result of some exciting event occurring
in the eye.
* Chap. XLI.
* Vol. II, p. 1815.
* Vol. II, p. 1718.
DISEASES OF THE UVEAL TRACT 2131
(a) INFLAMMATIONS ARISING BY DIRECT SPREAD are common, and
usually arise from other ocular tissues; they may, however, follow extra-
ocular lesions.
1. The most common instance of the former class is the irido-cyclitis
which is associated with a keratitis. We have already seen 1 that an irritative
vaso-dilatation of the anterior segment of the uveal tract associated with a
constricted pupil is a constant accompaniment of even the milder types of
acute keratitis; in the more severe types of suppurative keratitis (hypopyon
ulcer) the associated irido-cyclitis, due to the diffusion of toxins into the
eye and characterized by the formation of hypopyon and plastic exudates,
may assume a greater importance in the clinical picture than the disease of
the cornea itself. This condition, its aetiology, pathology, symptoms, and
treatment have already been fully discussed.”
A scleritis also invariably involves an associated uveal inflammation,
and in the deeper forms of this affection, the uveitis, which is usually of
considerable and sometimes of destructive severity, constitutes the gravest
aspect of this intractable disease. In many cases, indeed, it is difficult to
decide which of the two tissues has been primarily affected, and a more
correct term would frequently be scLERO-UVEITIS. With an anterior
scleritis there is always an irido-cyclitis and frequently a choroiditis; the
inflammation is usually plastic and exudative in nature, extensive synechiae
are common, keratic precipitates and vitreous opacities the rule, an exuda-
tive retinal detachment not a rare event, and a secondary glaucoma a
frequent and dreaded complication. Similarly posterior scleritis, while
frequently associated with an irido-cyclitis, is invariably complicated by a
severe choroiditis with massive vitreous opacities, which leaves, at the
best, large areas of chorio-retinal atrophy. The symptomatology, pathology
and treatment of these conditions have already been considered.”
An unusual occurrence is a strictly localized inflammation of the uveal tract
consequent on a localized Scleral lesion. Such a condition was reported by van Lint
(1933), wherein a localized Scleral abscess, produced traumatically, was accompanied
by a “partial iritis ‘’—an inflammation apparently localized to the corresponding
sector of the iris. Presumably such a condition is due to the direct diffusion of toxins
affecting the segment of the iris by contiguity.
It will be seen at a later stage that many retinal inflammations,
particularly those of an acute nature or due to syphilis or tubercle, spread
readily to the underlying choroid, and in severe cases may spread over the
whole uveal tract. Moreover, acute or extensive (tuberculous, syphilitic,
etc.) disease of the optic nerve, particularly when occurring near its ocular
termination, may affect the neighbouring choroidal tissues.
Uveitis, extensive and suppurative in nature, follows a suppurative
process originating in the lens or vitreous, but since these conditions are due
to the introduction of infection from without frequently with the retention
1 Vol. II, p. 1909. * Vol. II, pp. 1913, 1919. * Vol. II, pp. 2054 et seq.
2132 TEXT-BOOK OF OPHTHALMOLOGY
of a foreign body, they properly come under the heading of exogenous
inflammation (q.v.).
2. Inflammations spreading to the wea from neighbouring structures
outside the eye are rare : these include orbital abscesses, thrombo-phlebitis
of the orbital veins, or suppurative meningitis which spreads into the eye
along the sheaths of the optic nerve. In those cases panophthalmitis
develops and vision, and usually the eye, are lost. -
The organisms, of course, are various. Thus Axenfeld (1894) reported a case of
pneumococcal endophthalmitis following a pneumococcal meningitis, wherein a
bacterial infiltration was traced along the arachnoid spaces, the nerve sheath, and then
into the globe. From the orbit the most ready route of infection is by a thrombo-
phlebitis of the vortex veins (Wissmann, 1918; Mulock-Houwer, 1919).
(b) The second aetiological group includes a heterogeneous collection
of conditions all of which are characterized by UVEAL INFLAMMATION DUE
TO THE PRESENCE OF UNUSUAL OCULAR PRODUCTS IN THE EYE. These may
excite an irritation PARTLY OWING TO THE DIRECT ABSORPTION OF TOXIC
MATERIAL, BUT IN MOST CASES THERE IS A SUGGESTION THAT THE ALLERGIC
INFLUENCE OF A FOREIGN PROTEIN ENTERS INTO THE QUESTION.
1. Uveitis associated with an intra-ocular neoplasm occurs especially
when a malignant melanoma has reached, in part at any rate, a necrotic
stage. It is probably caused by the diffusion of toxins from the growth,
to which influence may perhaps be added the allergic effect of the repeated
liberation of unusual proteinmaterials."
2. An irritative iritis of comparable origin, usually less severe and more
transient, may occur in the presence of an intra-ocular hoemorrhage or in the
course of a disease characterized by massive exudation or repeated bleeding
(exudative retinitis, etc.). In this category may also be noted the relapsing
iritis occasionally seen after recurrent bleeding in haemophilia (Jean Sédan,
1930) or purpura (Benedict, 1930; Werner and Adlercreutz, 1931); in these
cases the inflammation is probably a toxic one conditioned by the absorption
of blood products from the haematoma.
3. An atrophic uveitis is a frequent and characteristic periodic reaction
seen in blind degenerated eyes. The cause of such an inflammation is
obscure, but presumably it is due partly to the effect of toxins liberated b
degenerating tissues, with, perhaps, an allergic element added. -
4. An irido-cyclitis of a similar type with a similar clinical appearance
and prognosis is met with in cases of retinal detachment of long standing. In
1 p. 2510.
DISEASES OF THE UVEAL TRACT 2133
some cases wherein the eye is degenerated, the condition is an atrophic
irido-cyclitis; but in others, signs of general degeneration are absent, and
the inflammation appears suddenly with a fall in tension in an eye which
had hitherto given little trouble. These inflammatory attacks Birch-
Hirschfeld (1912) has explained as allergic phenomena due to the presence
in the intra-ocular fluid of unusual protein constituents which reach it
through a retinal tear." -
5. Endophthalmitis phaco-anaphylactica. Endophthalmitis phaco-
anaphylactica, a concept about which everyone is by no means agreed, is a
generalized inflammation of the uveal tract occurring, it is presumed, in
sensitive persons, after lenticular proteins have been liberated into the eye
as after an extra-capsular extraction or a discission operation. The frequent
occurrence of such an inflammatory reaction is incontestable ; to a certain
limited extent it occurs in all patients, and as a general rule the ocular
reaction varies directly with the amount of lens matter left in the eye.
Such a sequence of events might well be explained as general toxic and
mechanical effects, but there remains an appreciable number of cases in
which the reaction becomes exceptionally severe, so severe, indeed, as to
justify excision of the eye after many weeks of persistent irritation and pain.
The presumption is that such cases are allergically sensitive to lenticular
protein and that the reaction is allergic in nature.
The conception that endophthalmitis phaco-anaphylactica is a definite
clinical entity occurring as an anaphylactic inflammatory reaction in
sensitive persons was first advanced by Verhoeff and Lemoine (1922) on the
basis of their finding that those persons who suffered the violent type of
Ocular symptoms when tested intra-cutaneously displayed a positive skin-
test to lenticular protein, and that these persons, when given a desensitizing
course of intra-muscular injections of lens protein, rapidly showed an
amelioration of their symptoms. The publication of this work was rapidly
followed by criticism from several workers, some of whom denied the
possibility of such a mechanism altogether (Rötth, 1925–29 ; Riehm, 1932);
others concluded that the reaction would be better explained on a simple
toxic basis without assuming an allergic element (Ellis, 1927; Braun, 1932);
while others brought forward evidence of the latter type of influence
(Lemoine and McDonald, 1924; Courtney, 1929; Burky and Woods, 1931;
Burky, 1934; Goodman, 1935). .
The question of the validity of this conception cannot be considered
settled as yet. The conclusion that it is entirely without foundation is
certainly overdrawn, and while the evidence points strongly to the interven-
tion of the factor of allergy to a considerable extent, how far such inflamma-
tions do represent typical allergic phenomena and constitute a separate
clinical entity must be settled by still further research. It certainly is the
1 p. 2887.
2134 TEXT-BOOK OF OPHTHALMOLOGY
case that while normal persons do not react intra-dermally to an injection of
lens protein, a considerable number do, and a small percentage (some
5%, Goodman, 1935) react violently; that a large proportion of these cases
do show more than usually severe inflammatory reactions to the post-
operative liberation of lens protein in the eye ; and it has been shown
in a number of cases that such a reaction does not occur or is considerably
modified after systemic desensitization by a course of injections prior to a
subsequent operation.
One of the most cogent arguments against the allergic conception was the
failure of the earlier workers (Rötth, 1926; Braun, 1932) to reproduce the sensitivity
experimentally in animals; but Burky (1934) has shown that such a result can be
attained in animals by the intermediary action of a staphylococcus toxin, repeated
injections of the toxin and lens protein being given at the same time. When such sensi-
tized rabbits were subjected to the operation of needling a violent endophthalmitis
resulted resembling clinically and histologically the condition in man, that is, of a
chronic inflammation characterized by a dense lymphocytic rather than a polymorpho-
nuclear infiltration. Burky suggests, therefore, that, in some instances at any rate,
the sensitivity may be brought about by the adjuvant action of a toxin from a focus
of infection at the time when lens matter is being absorbed after an operation. The
prophylaxis and treatment of this condition will be considered in the section dealing
with operations on the lens."
6. Uveitis associated with Sensitivity to Uveal Pigment. It has been
suggested that an endophthalmitis may arise as an allergic reaction to uveal
pigment in much the same way as endophthalmitis phaco-anaphylactica is
said to depend on sensitivity to lens protein. The pioneer work on the
immunological properties of the uveal pigment was done by Elschnig
(1910–11), who established that it could act as an antigen, and concluded
that its antigenic properties were responsible for the condition of sympathetic
ophthalmitis. His theory, which will be discussed in more detail sub-
sequently,” assumed that injury to one eye, involving a dissemination and
absorption of pigment, produced a hypersensitivity in the Organism as a
whole, especially in the fellow eye, and that continued absorption by this
eye, now rendered sensitive, resulted in an allergic intoxication manifested
clinically as sympathetic ophthalmitis, the whole reaction being parallel
to that of, for example, serum sickness. We shall see that this work
excited much controversy, and that, while certain of Elschnig's immuno-
logical postulates are undoubtedly true, the evidence available to-day
indicates that while an allergy to uveal pigment may be a factor in upsetting
the normal immunological defence-mechanism, it does not seem to be the
main aetiological factor in this disease.
However this may be, there is evidence that allergy to uveal pigment
may give rise to an endocular inflammation. The immunological properties
of the pigment have been amply confirmed by several workers, such as
Nakamura (1919), and Kodama (1922), but more particularly by Woods
1 Vol. IV. * p. 2334,
DISEASES OF THE UVEAL TRACT 2135
(1916–25). The last investigator verified the development of a complement-
fixation reaction in the blood of patients who had received an injury to the
uveal tract, and succeeded in producing a uveitis in dogs by sensitization
and intoxication with uveal pigment. It is quite possible, therefore, that in
some cases of violent uncomplicated traumatic cyclitis, this factor may enter
largely into the question. In any event it has been found that in a number
of such cases, particularly those presenting a picture of a low-grade, delayed,
recurring post-operative uveitis, a cutaneous sensitivity to uveal pigment
can be demonstrated (Woods, 1916–25; Knapp, 1925). Woods also found
that in such cases the histological picture differed from that seen in non-
specific, non-infective traumatic uveitis in that it showed the presence of
epithelioid cells and a proliferation of the pigmentary epithelium. Although
Woods has reported favourable clinical results with treatment by desensitiza-
tion by systemic injections of uveal pigment, sufficient evidence has not been
brought forward to justify a pragmatic conclusion of its value. Some effect
can be demonstrated objectively, however, by the increase of the opsonic
index so that the pigment is actively phagocyted (Henton, 1937). It
may therefore well be that, even although such a condition may not be a
clear-cut and independent clinical entity, the allergic influence of this
pigment may figure in the aetiology of a type of post-traumatic uveitis which
is delayed in its appearance, chronic, relapsing and recalcitrant in its course,
and non-infective in its appearance. The whole conception, however, is still
somewhat nebulous.
(c) TRAUMATIC UVEITIS. Quite apart from organismal infections, and
probably apart from an allergic reaction to extraneous protein material,
uveitis of an irritative type not infrequently follows injury (see Lindner,
1914–24). Traumata of all kinds enter into the aetiology of this condition,
including post-operative cases, particularly cataract extractions. The most
serious cases are those in which a foreign body is retained in the eye (these
will be discussed subsequently)," or in which some structure (iris, lens
capsule, etc.) is retained in the wound. A luxation of the lens may also
cause a similar irritative condition ; and finally an incarceration of uveal
tissue in an old wound, as in an adherent leucoma or an anterior staphyloma,
may produce such an irido-cyclitis long after the condition appears to have
settled down.” In some cases the factor of continued nerve irritation may
exert a disturbing influence, as might arise from stretching of the ciliary
nerves owing to an adhesion of the iris in the wound or a displacement of
the ciliary body or choroid (Arkhangelsky, 1936), while stimulation of
vaso-motor reflexes may exert a considerable influence on the aetiology.
SYMPATHETIC OPHTHALMITIS, an exceedingly dangerous and destructive inflamma-
tion of a chronic nature, raises up problems So peculiar that it must be dealt with
separately,” as also will the NEUROGENIC TYPES OF INFLAMMATION, herpes,” and
heterochromic irido-cyclitis.”
1 Vol. IV. * Vol. II, p. 1825. 8 p. 2327. 4 p. 2254. * p. 2361.
2136
TEXT-BOOK OF OPHTHALMOLOGY
Arkhangelsky. Sovet. vestnik. O., viii, 10,
1936.
Axenfeld. A. f. O., xl (3) l ; (4) 103, 1894.
Benedict. A. of O., iv, 588, 1930.
Birch-Hirschfeld. A. f. O., lxxxii, 241, 1912.
Braun. A. f. Aug., cvi, 99, 1932.
Burky. A. of O., xii, 536, 1934.
Burky and Woods. A. of O., vi, 548, 1931.
Courtney. Am. J. O., xii, 20, 1929.
Ellis. N. Y. State J. Med., xxvii, 1296, 1927.
Elschnig. A. f. O., lxxv, 459 ; lzxvi, 509,
1910; lxxix., 428, 1911.
Goodman. A. of O., xiv, 90, 1935.
Henton. A. of O., xvii, 113, 1937.
Rnapp. A. of O., liv, 252, 1925.
Rodama. J. Infect. Dis., xxx, 418, 1922.
Lemoine and McDonald. A. of O., liii, 101,
1924.
Lindner. A. f. O., lxxxviii, 415, 1914.
Z. f. Awg., liii, 305, 1924.
van Lint. A. d’O., l, 449, 1933.
Muloch-Houwer.
1919.
Nakamura. Festsch. Prof. Komoto, 211, 1919.
Riehm. K. M. Aug., lxxxviii, 62, 1932.
Rötth. Magyar Orvosi A., xxvi, 466, 1925.
A. of O., lv., 103, 1926.
A. f. O., czzii, 34, 1929.
Sédan, Jean. Bull. S. fr. d’O., xliii, 222, 1930
Verhoeff and Lemoine. Am. J. O., v, 737,
1922. Internat. Cong. Washington, 234,
1922.
Werner and Adlercreutz. Finska läk. handl.,
lxxiii, 833, 1931.
Wissmann. A. f. O., xcvii, 276, 1918.
Woods. A. of O., xlv, 557, 1916; xlvi, 8
283, 503, 1917; xlvii, 161, 1918.
J. Am. Med. As., lxxvii, 1317, 1921.
T. O. S., xlv. (1), 208, 1925.
Am. J. O., xix, 9, 100, 1936.
Woods and Knapp. A. of O., li, 560, 1922.
K. M. Aug., lxiii, 179,
5
3. ENDOGENOUS INFECTIONS
Endogenous inflammations, which are caused by the entrance by way of
the blood-stream of organisms or their products into the eye from some
source situated elsewhere in the body, form the great majority of inflamma-
tory conditions affecting the uveal tract. In a comparatively small number
of cases the aetiology of such affections is clear, but unfortunately in the
majority the clinical picture is of a nondescript, non-specific nature, and not
only is the causal organism a matter of doubt but also the mechanism of its
action is obscure. Around the whole subject, in fact, much difference of
Opinion exists, and our knowledge is by no means sufficiently far advanced
either in the problems of diagnosis or pathology to allow us to proceed on
sure ground. Certain definite categories, however, can be recognized.
(A) Inflammations due to Organismal Metastases
That uveitis can be caused by the actual entrance of micro-organisms
into the eye and their lodgement therein has been abundantly proved both
experimentally and clinically. This was first established by the classical
experiments of Cohnheim (1867) who injected guinea-pigs with tuberculous
material and found at autopsy tubercles in the uveal tract, a result which
has been amply confirmed by many others who injected tubercle bacilli
into the blood-stream (Haensell, 1879; F. Lagrange, 1895–98; Friedrich
and Nosske, 1899 ; Stock, 1903–07 ; Axenfeld, 1905; Rollet and Aurand,
1908; H. Lagrange, 1924; and others). Similarly, it is easy in syphilis to
recover treponemata from the eye after testicular inoculation (Bertarelli,
1906–07 ; Greeff and Clausen, 1906; Uhlenhuth and Mulzer, 1911 ;
Igersheimer, 1922; Chesney and Kemp, 1925; Clapp, 1929–33; and many
others). In the same manner streptococci, pneumococci and other organisms
have been found when injected into animals to produce uveitis, and from
DISEASES OF THE UVEAL TRACT 2137
the affected eyes the organisms have been recognized by microscopic
demonstration in sections and recovered in culture (Poynton and Paine,
1903; Selenkowsky and Woizechowsky, 1903 ; Rosenow, 1915; Irons,
Brown and Nadler, 1916 ; Haden, 1923 ; Brown, 1934 ; and others).
Such cases undoubtedly occur clinically. Metastatic bacterial emboli,
of course, account for the specifically characteristic lesions of tubercle,
syphilis, and leprosy, and they also account for the uveitis, usually of a
purulent nature and a dramatically destructive course, which complicates a
general pyaemia due to pyogenic organisms. Such cases, however, are not
common : Axenfeld (1894) compiled 69 from the literature, 27 of which were
bilateral, Seguini (1923) collated 342 of which 106 were examined bacterio-
logically, and in the literature of the subsequent 10 years Lumbroso (1933)
found a further 91. They occur along with embolic manifestations in other
parts of the body in pyaemic states, the streptococcus being the most common
Organism, and have been noted on occasion in a host of diseases. In many
of them the aetiological proof has been complete, either by the recognition
of the organisms histologically in the uveal tract (Friedenwald and Rones,
1931), or by the cultivation of the same organism from the primary focus
and the ocular tissues (Levine, 1930; Brown, 1934 ; Sédan, 1935; Laval,
1937; and many others). -
The organisms responsible for these conditions include all those which give rise
to pyaemic manifestations. In the majority of cases in the literature bacteriological
examinations are lacking : thus in Seguini’s (1923) compilation of 342 cases, 259 were
of undetermined bacterial origin. The streptococcus is the most common, and the most
fatal : thus Luttringer (1922) reported 6 cases in puerperal fever all of which died,
Brown (1934) recorded cases in septic abortion and pyelitis, and so on. On the other
hand, in the more chronic types of streptococcal infection, an incidental and mild
choroidal lesion only may result (Schüssele, 1909 : Friedenwald and Rones, 1931).
Next in numerical importance, and usually leading to loss of the eye, is the pneumo-
coccus: first noted by Axenfeld (1894) and subsequently by Peters (1901), such cases
have been described in detail clinically and pathologically by Bussola (1931), Heinomen
(1932), Lawson (1933) and Hulka (1937). They usually occur in the course of a
pneumonia, but include such diverse conditions as pneumococcal otitis media (Lawson,
1933; Howard, 1936), cholecystitis (Terrien, 1922) or a coryza (Heinomen, 1932).
The meningococcus is also frequently found (Laval, 1937), especially in children
(Jeandelize, 1925). Other organisms include the staphylococcus from a furuncle On
the skin, in the nose, or a carbuncle in the neck (Schanz, 1906; Horner and Cordes,
1931; Higuchi, 1934; Laval, 1937), from a prostatic abscess (Terrien, 1922) or a
urinary infection (Sédan, 1935), the gonococcus (Velhagen, 1937), B. coli (Oreste, 1909;
Betti, 1919; Levine, 1930; Sugimoto, 1933; François, 1935), or a mycosis (Müller,
1903; Fuchs, 1919; Verhoeff, 1926), or mould fungus (Lorenz, 1933). On the other
hand, cases which have been proved histologically to be due to metastatic suppurative
deposits have remained completely undiagnosed despite the most thorough and
exhaustive investigation both clinical and pathological (v. Hippel, 1932).
The list of infective diseases in the course of which such an accident may happen
is very large indeed, and contains practically all pyaemic states. It has been known
as a sequel of puerperal fever since the time of Tenon, and Jüngken (1836) described
the hypopyon in this condition as milk in the anterior chamber. Meckel (1854) and
2138 TEXT-BOOK OF OPHTHALMOLOGY
Müller (1856) made the first pathological examinations, and since that time clinical
studies have been extremely numerous (Röth, 1872; Mitvalsky, 1893; Berger and
Loewy, 1906; Luttringer, 1922; and very many others). Other diseases include
pneumonia (Axenfeld, 1894; Fraenkel, 1899; Peters, 1901; Touzet, 1921; McKee,
1936; Hulka, 1937; and others), meningitis (Jeandelize, 1925), influenza (Samperi,
1919; Onate, 1919; Sédan, 1935), otitis media (Lawson, 1933; Howard, 1936),
cholecystitis (Terrien, 1922), urinary infections (Levine, 1930; Brown, 1934), com-
plicated fractures (Greenwood, 1930), septic abortion (Brown, 1934), infective surgical
operations (Lumbroso, 1933), tonsillitis (v. Fleet, 1931; Asbill, 1933), endocarditis
(Blüthe, 1908; Gilbert, 1917–25; Weve, 1923; v. Herrenschwand, 1929; Friedenwald
and Rones, 1931), as well as a host of other conditions such as abscesses and boils
(Hanke, 1921; Horner and Cordes, 1931; Higuchi, 1934), the list even including a
common cold due to pneumococci (Heinomen, 1932).
As a rule these metastatic conditions make up for their rarity by their
gravity: in the majority the eye is lost, and very frequently the ocular
inflammation is an incident in a general state so profoundly serious that the
illness terminates fatally. Thus in Axenfeld’s series 66% of the patients
died. In a large number of cases, however, the patient is relatively well,
and the ocular manifestation may be the only evidence of systemic infection.
It may be that such lodgements are largely fortuitous ; but, on the other
hand, there is evidence that sometimes the embolus may be “fixed '' in the
eye owing to some coincidence which has lowered the resistance of the
tissues locally. Thus Sédan (1935) considered that a preceding retinal
haemorrhage determined the lodgement of a staphylococcus from the urinary
tract in the eye. That trauma may act as a localizing influence in this sense
was proved by the original animal experiments of Selenkowsky and
Woizechowsky (1903) and is confirmed by the not infrequent occurrence of a
suppurative uveitis, which is usually of the sub-acute type and goes on to
panophthalmitis, which appears post-operatively.” It may be argued that
some of the cases of this type are due to exogenous infection by contamina-
tion during the operation, but the occurrence of such a clinical picture
several days or even weeks after an operation in which no technical complica-
tions have occurred, in which the wound has soundly healed, and which was
undertaken with an apparently sterile conjunctival sac which has remained
so, proves beyond question that such cases may be metastatic and endogenous
(Harrison Butler, 1920–21; Gilbert, 1928; Orloff, 1928)—a comforting
thought for the surgeon.
The route of infection is not always easy to decide because the condition
may proceed with such rapidity that the eye comes under observation first
in a state of panophthalmitis. In a great many of the cases the central
artery of the retina may be the port of entry, the condition being primarily
a retinitis with a secondary uveitis. So far as the ciliary circulation is
concerned the long or short posterior ciliary arteries may be involved, and
the inflammation is at the start, and in less dramatic cases may remain, an
1 p. 2129.
DISEASES OF THE UVEAL TRACT 2139
anterior or posterior uveitis. Only rarely have the anterior ciliary twigs
from the vascular supply to the recti been proven to be the starting point
(Hulka, 1937).
Clinically such conditions may be classified into three types depending
on the virulence of the infective process.
(a) Acute purulent panophthalmitis, in which case a localized abscess in
the anterior or posterior segment of the uvea rapidly progresses to a general
infection of all the tissues.
(b) Sub-acute wweitis, which sometimes may remain limited to the
anterior or posterior segment. In the former case it appears as an irido-
cyclitis with hypopyon which may be a recurrent condition,1 and in the
latter, an endophthalmitis develops in which the vitreous cavity becomes
purulent and atrophy of the globe eventuates.
(c) Chronic weitis—an insidious and mild inflammatory condition
becoming gradually apparent. This type may be seen, for example, in the
septicaemia of chronic endocarditis (the OPHTHALMIA LENTA of Gilbert, 1925),
and in the foetus and in infants is responsible for the appearance of a pseudo-
glioma. The most mild types occurring in the posterior segment have been
designated SEPTIC CHOROIDITIs by Friedenwald and Rones (1931). These
investigators in the routine autopsy of 37 cases of septicaemia due to a variety
of organisms (streptococci, staphylococci, pneumococci, gonococci, B. coli)
found small choroidal metastatic deposits in 9 : all were bilateral, none was
acute, pathologically they were comprised of a predominantly lymphocytic
infiltration, and clinically they gave no striking ophthalmoscopic evidence of
their presence. They correspond, therefore, with the “septic retinitis of
Röth.”
About the possibility of VIRUS INFECTIONS little is yet known. Herpetic iritis
comes into this class ; but that cases of uveitis which have been classed as of unknown
aetiology may on Occasion be caused by some (unknown) virus is suggested by the
experience of Friedenwald and McKee (1938). In a case of bilateral uveitis with
papillitis these workers produced from the filtered spinal fluid a uveitis in animals
which was very similar to the “periodic ophthalmia '' of horses which Woods and
Chesney (1930) concluded was a virus infection. The possibility of a virus infection in
the aetiology of sympathetic ophthalmitis will be discussed at a later stage.
Asbill. A. of O., ix, 820, 1933. Chesney and Kemp. J. Eacp. Med., xlii, 33,
Axenfeld. A. f. O., xl (3), 1 ; (4), 103, 1894. 1925.
B. O. G. Heidel., xxv, 140, 315, 1896. Clapp. A. of O., ii, 580, 1929.
Med. Kl., i, 375, 1905. Am. J. O., xi, 527, 1928; xvi, 397, 1933.
Berger and Loewy. Ueber Augenkrankungen Cohnheim. A. path. Anat., xxxix, 49, 1867.
bei Frauen, Wiesbaden, 1906. Berlin. kl. W., iv, 63, 324, 1867.
Bertarelli. Zbl. Bakt., xli., 320, 1906; xliii, van Fleet. A. of O., vi, 426, 1931.
238, 1907. - Fraenkel. A. f. O., xlviii (2), 456, 1899.
Betti. Riforma Med., xxxv, 784, 1919. François. A. d’O., lii, 424, 1935.
Blüthe. Diss., Heidel., 1908. Friedenwald and McKee. Am. J. O., xxi,
Brown. A. of O., xii, 730, 731, 1934. 723, 1938.
Bussola. Boll. d’Oc., x, 987, 1931. Friedenwald and Rones. A. of O., v., 175,
Butler, Harrison. T. O. S., x1, 181, 1920; 1931.
xli, 391, 1921.
1 p. 2227. * p. 2646. See also periarteritis nodosa.
2140 TEXT-BOOK OF OPHTHALMOLOGY
Friedrich and Nosske. Ziegler’s Beit., xxvi, Lumbroso. Rass. It. Ott., ii, 628, 1933.
470, 1899. Luttringer. Gyméc. et Obstet., vii, 513, 1922.
Fuchs. A. f. O., lxxxiv, 201, 1913; ci, 24, McKee. A. of O., xv, 787, 1936.
9
1919. Meckel. Charité Annalen, 1854.
Gilbert. B. O. G. Heidel.., xlvii, 91, 1928. Mitvalsky. K. M. Aug. xxxi, 18, 1893.
Z. f. Aug., xxxvii, 174, 1917. Müller, H. (1856). Gesammelte Schriften,
A. f. Aug., lxxxvi, 29, 1920; xcvi, 119, Leipzig, 1872.
1925. Müller, L. K. M. Aug., xli (2), 236, 1903.
Greeff and Clausen. Dewt. med. W., xxxii, Onate. Rev. Cub. Oft., i, 328, 1919.
1454, 1906. Oreste. An. d’Oc., czlii, 373, 1909.
Green and Green. Am. J. O., ii, 607, 1919. Orloff. Russ. O. J., vii, 281, 1928.
Greenwood. A. of O., iv, 773, 1930. Peters. K. M. Aug., xxxix, 392, 1901.
Haden. A. Int. Med., xxxii, 828, 1923. Poynton and Payne. T. O. S., xxiii, 39, 1903.
Haensell. A. f. O., xxv (4), 1, 1879. Rollet and Aurand. Rev. gen. d’O., xxvii, 1,
Hanke. A. f. O., cv, 851, 1921. 1908.
Heinomen. Finska Läk. handl., lxxiv, 360, Rosenow. J. Infect. Dis., xvii, 403, 1915;
1932. xxxiii, 248, 1923; xlv, 331, 1929.
v. Herrenschwand. K. M. Aug., lxxxiii, 419, Röth. Deut. Z. f. Chir., i, 471, 1872.
1929. Samperi. A. di Ott., xxvi, 19, 1919.
Higuchi. K. M. Aug., xciii, 348, 1934. Schanz. Z. f. Aug., xvi, Erg., 58, 1906.
v. Hippel. A. f. O., czkviii, 272, 1932. Schüssele. K. M. Aug., xlvii (2), 50, 1909.
Horner and Cordes. Am. J. O., xiv, 628, Sédan. Bull. S. fr. d’O., xlviii, 140, 1935.
1931. Seguini. An. di Ott., li, 30l., 1923.
Howard. A. of O., xv, 787, 1936. Selenkowsky and Woizechowsky. A. f. Aug.,
Hulka. A. of O., xvii, 127, 1937. xlvii, 299, 1903.
Igersheimer. A. f. O., ciz, 265, 1922. Stock. K. M. Aug., xli., Beil., 17, 1903.
Irons, Brown and Nadler. J. Infect. Dis., A. f. O., lxvi, l, 1907.
xviii, 315, 1916. Sugimoto. Acta O. S. Japan, xxxvii, 1661,
Jeandelize. Médicine, vi, 276, 1925. 1933.
Jüngken. Augenkrankheiten, Berlin, 1836. Terrien. Rev. gen. d’O., xxxvi, 331, 1922.
Lagrange, F. A. d’O., xv, 170, 1895. Touzet. A. d’O., xxxvii, 372, 1921.
Bull. S. fr. d’O., xvi, 88, 1898. Uhlenhuth and Mulzer. Ber. kl. W., xlviii,
Lagrange, H. Tuberculose du tractus weal, 653, 1911.
Paris, 1924. Velhagen. K. M. Aug., xcviii, 20, 1937.
Laval. A. of O., xviii, 104, 1937. Verhoeff. A. of O., lv, 225, 1926.
Lawson. J. Am. Med. As., ci, 599, 1933. Weve. A. f. Awg., xciii, 14, 1923.
Levine. A. of O., iii, 410, 1930. Woods and Chesney. J. Eacp. Med., lii, 637,
Lorenz. Diss., München., 1933. 1930.
(B) Inflammations due presumably to Organismal Allergy or Tozic Influences
It is well to state at the beginning that the great majority of cases of
uveitis are of a nondescript, non-specific type, the aetiology of which is at
present quite uncertain. Exudative rather than purulent in nature, localized
rather than generalized in their incidence in the uveal tract, and chronic,
recurrent and benign rather than acutely destructive in character, they
differ profoundly from the type of inflammation we have just discussed as
due to the intra-ocular lodgement of bacterial metastases. They do not
occur typically in the course of an acute massive infection, there is no easily
demonstrable bacteraemia associated with them, and organismal emboli
are not found within the eye, nor do these occur simultaneously in other
organs; on the contrary they tend rather to be associated with the more
chronic type of infection and to run parallel with rheumatic affections
in joints and fascial planes. Most authorities agree that the vast majority
of such cases are infective in nature, but since pathological material
is hard to come by and since the bacteriological evidence available is
inferential rather than direct, not only is the nature of the infection in most
cases obscure, but also its mode of action is largely conjectural.
I)ISEASES OF THE UVEAL TRACT 2141
The history of the changes of opinion which have come over ophthalmological
thought in this matter is interesting. In the middle of last century, for example, the
causes of uveitis were given as syphilis, tubercle, rheumatism and gout, leaving a
large residuum of idiopathic cases. Thus Arlt (1853) gave as causes of iritis, scrofula.
30%, rheumatism 21.5%, syphilis 17% and idiopathic cases 25.5%. On the whole
syphilis and rheumatism were particularly stressed, while v. Michel (1881) emphasised
the importance of tubercle. This view held the field until some years after the opening
of the present century, and is still reflected in many of the standard text-books of to-day.
Early in the century, however, some English clinicians drew attention to the probability
of such cases being due to focal infection : one of the first recorded opinions was that
of Nettleship (1879), who considered a case of exudative choroiditis to be due to dental
infection, Campbell (1905) spoke of iritis as being due to septic teeth, Butler (1911),
in 100 cases of iritis, considered 12% to be due to oral, nasal and other sepsis, and
eventually Lang (1913) raised this figure to 40% of cases of eye disease, teaching the
revolutionary hypothesis that these conditions were “septic in origin and not due to
anaemia, rheumatism, or the gouty diathesis.” In the meantime in America Billings
(1912–21) put forward the same hypothesis, and his suggestions were enthusiastically
taken up and put to the test of elaborate experimental research by such investigators
as Irons and Brown (1916–26) and Rosenow (1915–30). While, therefore, at an
American congress in 1907 (Zentmayer, Chance, Risley and Ziegler) it was said that
85% of cases of iritis were due to syphilis and rheumatism and the residuum was due
to gout, tubercle, gonorrhoea, diabetes, nephritis, malaria, infectious fevers, anaemia,
auto-intoxication, adolescence and Senility, and at an International congress in 1913,
de Schweinitz found little mention of focal infection as a cause for uveitis, in the
following year Goulden (1914), writing in England, assessed the aetiological incidence
of focal infection in irido-cyclitis as 55% and in choroiditis at 43%, and within the
next two decades in America figures of 40% to 45% were given (Irons and Brown,
1923; Gifford, 1931 ; Holloway, 1931), whereas the influence of tubercle was put as
low as 4% (Irons and Brown, 1923) or 8% (Gifford, 1931). Contemporaneously, in the
German-speaking countries on the Continent of Europe, the proportion of cases of
uveitis of indefinite origin which are labelled as tuberculous had become correspondingly
high (50%, Hessberg, 1918; 45%, Gilbert, 1930; 50%, Löwenstein, 1931; 50%, Kahoun,
1932). The position, therefore, may be stated in general terms to be that while syphilis
as an aetiological factor is much less popular than it used to be (thus the syphilitic
percentage in iritis is given as 60–70%, v. Wecker, 1876; 26%, Baum, 1902; 30%,
Leber, 1909; 17%, Hessberg, 1910; 11.6%, Igersheimer, 1910; 12.7%, Elschnig, 1912;
14%, Gilbert, 1929; 16.9%, Gifford, 1931; 6.4 %, Kahoun, 1932), the group of
cases which used to be labelled idiopathic or due to a rheumatic or gouty diathesis,
has been transferred by English and American clinicians to the new category of focal
infections, and by Continental clinicians to the tuberculous group. On the scientific
evidence which is available it is impossible in the present state of our knowledge to
adjudicate dogmatically between the two schools of thought, but the circumstantial
evidence is strongly in favour of the fact that a large proportion of cases of uveitis is
due to focal infection, most frequently streptococcal in nature ; but, as the reader
of the previous volume of this text-book will have seen,” I think it is possible that the
majority of this somewhat indefinite group are allergic in nature rather than directly
infective, and that a tuberculous or streptococcal focus acts in their causation by a
similar mechanism, the allergy frequently being composite, and the incidence of the
inflammatory process depending more upon the constitutional and immunological
condition of the tissues than on the particular organism concerned.
Vol II. p. 1484.
2142 TEXT-BOOK OF OPHTHALMOLOGY
The mechanism of such infections has excited controversy from early
times when discussions arose—as they still do—as to whether they are due
to direct organismal infection or not. Litten (1880), Leber (1880) and
Wagenmann (1887) contended that they were due to bacterial metastases;
Röth (1872), Herrnheiser (1892) and Ischreyt (1900) thought they were toxic;
while Axenfeld (1907) held that they were usually toxic but exception-
ally might be due to organisms of attenuated virulence, a view which he
substantiated by a case of pneumococcal metastases wherein one eye was
lost with panophthalmitis while the other showed mild inflammatory
nodules containing masses of the organism (Axenfeld and Goh, 1897).
We have already discussed at length the more modern conception of the
aetiology of such lesions 1 and all that will be required here is a short
recapitulation. We have seen that it is possible that a number of them, at
any rate, are due to the occurrence in persons suffering from focal sepsis or
tuberculosis of a mild transient bacteraemia of such a type that it cannot
be looked upon as an infection of the blood in the usual pathological sense,
but represents a temporary leak of relatively non-pathogenic organisms
into the circulation followed by their rapid removal by the immune action
of the body. It has been shown, for example, that such relatively innocent
bacteraemias occur in 10% of persons suffering from streptococcal dental
infections (Okell and Elliott, 1935); and it is claimed particularly by the
Vienna school that a similar condition exists in the subjects of focal tubercu-
losis (Löwenstein, 1931; Urbanek, 1932; Meller, 1934). We have also seen
when discussing the general question of allergy " that if the eye shares in a
systemic infection, a hypersensitivity of the ocular tissue can be produced,
that later contact of the same eye with the specific bacterial products results
in inflammation, and that experimentally the greatest degree of sensitivity
arises from repeated systemic injections of an organism or from the
maintenance (as in agar) of a focal lesion in the body acting over some
time (Swift and Derick, 1928; Julianelle, 1930; Seegal and Seegal, 1930;
Schultz and Swift, 1932; Brown, 1934–35; MacLean, 1936; Berens,
Angevine, Guy and Rothbard, 1938); in the last case even non-pathogenic
organisms, such as B. xerosis, may produce a pathological effect (Marchesani,
1929; v. Szily, 1930). It has also been shown that long after sensitization
has occurred and when all traces of the specific protein had disappeared
from the aqueous, re-activation by an intravenous injection made the
specific antigen reappear in the anterior chamber (Seegal, Seegal and
Rhorazo, 1933), a phenomenon which explains recurrent attacks of
inflammation. Moreover, it has been shown experimentally that parenteral
injections of horse serum may give rise to a focal reaction in the eye accom-
panied by a lymphocytic infiltration of the uvea, a reaction which is
cumulative on repeated injections (Iga, 1930); and at least one case has been
reported in man wherein a reactive iritis of considerable severity seemed to
* Vol. II, p. 1484. - Vol. II, p. 1455.
DISEASES OF THE UVEAL TRACT 2143
be constantly associated with serum sickness (Theodore and Lewson, 1939).
This being the case, it is a plausible hypothesis, and one which in the present
unsatisfactory state of our knowledge seems more justifiable than any other,
that a number of such cases of uveitis are due to the eaccitation of a local
allergic reaction by bacterial products liberated from some focus of infection in
an immune but allergic patient.
It is true that some such cases (tuberculous, streptococcal) may be due to
direct organismal infection (Benedict, 1921; Meller, 1934; Traut, 1934), the
Organisms being present in scanty numbers or in an attenuated state so
that the systemic and immune reaction is slight. Thus, on investigating
5 patients with iritis by special cultural methods, Traut (1934) found strepto-
cocci in the blood of all of them as he had previously (1933) done in cases of
chronic arthritis; and in 1 case at least of irido-cyclitis an identical strepto-
coccus has been found in an apical dental abscess and in the anterior chamber
(Wood, 1925). Again, Urbanek and Meller (1932) claim a tuberculous
aetiology in the somewhat astonishing number of 90% of cases of inflamma-
tions of the uveal tract, stating that the bacilli are demonstrable in the blood
and in the eye itself. Whether this is the case or not, it cannot be the whole
story, for it does not explain the absence of systemic symptoms and gives
no clue as to why the eye is picked out and the other organs escape embolic
manifestations; it seems likely that it cannot reasonably be accepted as the
usual mechanism. We have already discussed 1 the explanation offered by
the theory of elective localization as propounded by Rosenow (1915–30),
Rosenow and Nickel (1932), and others, and we have found that the evidence
so far produced is by no means satisfying ; although it is possible that in
certain cases localization in the eye may be determined by preceding trauma
(Fage, 1930; and others), such an explanation cannot be general.
Other cases may be due to an involvement of the eye in a systemic
intoxication, for it has been established experimentally that uveitis can be
produced by the injection of such toxic products as bacterial filtrates
(Guillery, 1911–14; Woods, 1916) or toxins (diphtheria, staphylococcal,
streptococcal: Stock, 1903; Koske, 1905; Brown, 1932). Toxins
have been identified as arising from many organisms, including the
streptococcus, the staphylococcus, the pneumococcus, and the gonococcus,
and in this case the question of tissue-selectivity becomes more plausible, and
can be exemplified by several indisputable instances: the exotoxin of the
tetanus bacillus, for example, even when present in massive concentration
in the blood, may have a selective effect only on the anterior horn cells of the
spinal cord, and the only evidence of a diphtheritic toxaemia may be on the
heart muscle. But on the whole it would seem that a large number of
such cases at any rate are due to AN INFECTIVE ELEMENT WHICH ORDINARILY
IS A COMMON AND MINOR, EVENT OCCURRING INTERMITTENTLY IN APPARENTLY
HEALTHY PERSONS WITHOUT MISHAP AND GIVING RISE TO NO SYSTEMIC
1 Vol. II, p. 1488.
T.O.-WOL. III. E
2144 TEXT-BOOK OF OPHTHALMOLOGY
CLINICAL EVIDENCES, BUT WHICH IN THE PRESENCE OF TISSUE-ALLERGY AT
ONCE ASSUMES UNWARRANTED IMPORTANCE, THE PRIMARY AFTIOLOGICAL
FACTOR BEING THE CONDITION OF SENSITIZATION OF THE LOCAL TISSUE
RATHER THAN THE VIRULENCE OF THE CAUSAL INFECTIVE ORGANISM.
What the causal focus in such conditions may be, is too frequently
difficult or impossible to determine, and any diagnosis must usually be made on
circumstantial evidence rather than on actual matter of proof. Tuberculosis
undoubtedly is a frequent cause : a lowering of the resistance of an immune
but allergic patient allows the periodic liberation of bacilli from a focus in,
for example, a mediastinal or abdominal gland, the disintegrated products
of which excite a focal reaction in the sensitized eye. Foci of pyogenic
infection are probably a more common cause, such as are found in the teeth,
tonsils, nasal sinuses, the prostate and deep urethra, the endometrium, the
colon, the urinary tract, the gall-bladder, and so on. It is probable, indeed,
that more than one source of infection frequently exists contemporaneously,
such as a tuberculous and streptococcal One, or a mixed streptococcal One
from, perhaps, the teeth, tonsils, and colon : such a composite aetiology
receives frequent clinical support, and it has been shown experimentally
that multiple sensitization of the eye can be obtained by the injection of
different allergens, both protein and bacterial (Brown, 1935). The entire
subject is very obscure and necessarily vague, largely because concrete
clinical proof is exceedingly difficult to produce in the absence of the finding
of an incriminating organism in the eye ; indeed, such an aetiology is by no
means universally admitted, and, even when it is admitted, the probable
causal organism is frequently a matter of guesswork. In all cases there is
a large number of variables of conditions and circumstances, applying both
to the host and the invading organism, about which our knowledge is
fragmentary or non-existent ; but the essential fault probably lies with the
tissues of the host and their immunological state.
With so much unknown, and the mechanism of infection a matter of
dispute, it is obvious that any identification of a particular lesion as the
causal factor must be a matter of the assessment of probabilities. Some
remarks, however, on the more important foci may be of value.
1. TEETH
Although Nettleship (1879) had early drawn attention to the association between
an exudative choroiditis and dental infection, little attention was paid to the subject
until the papers of Butler (1911), Lang (1913) and Goulden (1914) in England and
Billings (1912) in America. Lang (1913), for example, considered that oral sepsis
accounted for 139 out of 215 cases of ocular disease which could be attributed to
sepsis, and Goulden (1914) concluded that it was the commonest cause of uveitis,
being responsible for 67 out of 194 cases. Since that time innumerable reports have
appeared in the literature of cases of uveitis in all its forms which have been associated
with dental infection and have cleared up on the removal of infected teeth
(Hartzell, 1915; Ulrich, 1915; Ackland, 1923; Lang, 1923; Ziegler, 1923; Gifford,
DISEASES OF THE UVEAL TRACT 2145
1923; de Schweinitz, 1924; Fromaget, 1924; Hensen, 1924; Elschnig, 1925;
Walter, 1925; Bjerrum, 1927; Magnasco, 1928; MacCallan, 1928–30; to mention
a few writers from various countries). Experimentally also, the number of workers
who have induced an iritis in animals by the injection of streptococci from dental
abscesses is considerable, in Several instances the organisms being recovered from the
eye (Rosenow, 1920; Benedict, 1920–21; Lewis, 1923; Haden, 1923), a positive
result which must counterbalance negative or practically negative experiences (Moody,
1916; Henrici, 1916; Levy, Steinburgher and Pease, 1917; Back, 1927). Moreover,
clinically an identical streptococcus has been recovered from a dental abscess and the
aqueous in a case of irido-cyclitis (Wood, 1925). It is to be remembered, of course,
that a very large proportion of the population has gross dental sepsis without any
uveitis, but these, it is presumed, have developed no streptococcal ocular allergy :
thus Ulrich (1915) found that in 76 patients with gross dental sepsis and general
infective manifestations, only 17 had iritis, and Levy, Steinburgher and Pease (1917),
noted 7 cases of iritis among 57 patients with obvious dental foci. Some idea, however,
of the trend of authoritative opinion on the importance of this factor in the causation
of inflammations in the uveal tract is given by the following figures taken from the
relatively recent literature giving the percentage of cases of iritis attributable to dental
causes, the first column representing American writers, the second European :
Irons and Brown (1923), 13.5% Elschnig (1925), 20%.
Bulson (1925), 32%. Back (1927), 32%.
Newton (1925), 14.6%. Gilbert (1929), 1-2%.
Gifford (1931), 12.7%.
Baeseman (1934), 28.5%.
2. TONSILS
In the opinion of many recent writers the tonsils play a part as important, if not
more so, than the teeth, and quite frequently a streptococcal infection is located in
both regions: in many cases, indeed, particularly in old people, the infection of the
tonsils may be secondary to dental disease. Goulden (1914) cited two cases of irido-
cyclitis which seemed definitely due to tonsillar infection ; Gilbert (1929) gives a
percentage in irido-cyclitis of 1-2, and Newton (1925) of 6-6. Other authors offer
higher figures—Bulson (1925), Gifford (1931) 22%, Irons and Brown (1923), 26.5%,
and Baeseman (1934) 26.2%.
3. THE NASAL SINUSES
Since Ziem (1887) first drew attention to a possible connection between
infective conditions of the nose and nasal sinuses and iritis, a considerable number of
clinicians recorded cases emphasizing an apparent relation of cause and effect (Fromaget
1893; Fage, 1895–98; Fish, 1904; Senn and Spirig, 1900; Hoffmann, 1906; Davis,
1917; and others). The long series of cases recorded by Lang (1913), de Schweinitz
(1913), and Goulden (1914) associating irido-cyclitis and choroiditis with various types
of nasal disease, especially with the retention of pus in the various sinuses (antrum,
frontal sinus, ethmoid cells, etc.), drew more attention to the subject, and to-day
the connection is generally admitted. Most authors consider that they form one of
the less frequent causes of uveitis; others would give them a greater share in the
aetiology (Mackenty, 1932). The following figures are given by more recent writers
On the aetiology of iritis :
Irons and Brown (1923), 2%. Bulson (1925), 4%.
Newton (1925), 8%. Gilbert (1929), 4%.
Gifford (1931), 6.8%.
2146 TEXT-BOOK OF OPHTHALMOLOGY
It is interesting to note in passing that Brown and Drummer (1929) produced
Ocular lesions in rabbits from intra-carotid injection of streptococci from the antrum
in a case of iritis.
4, THE INTESTINAL TRACT
The first to draw attention forcibly to the relation between the bowel and irido-
cyclitis was Elschnig (1905), who considered the ocular inflammation due to intoxica-
tion from products of perverted metabolism. The researches of de Schweinitz (1906)
and de Schweinitz and Fife (1908), however, suggested the more modern view that
any influence which the gastro-intestinal tract exercised was much more probably
as a source of bacteria or their toxic products, the bowel thus acting in the same manner
as other foci of infection. Most authors are agreed that the gut is not a common source
of infection : Elschnig, it is true, put the percentage of cases of iritis due to this
cause as high as 19 (1912) or 17.5 (1925), and Lang (1917) as 11.5, while Dwyer (1919),
studying a series of 122 cases of all types of uveitis from this point of view, ascribed a
very considerable influence to the bowel, finding marked relief of the symptoms
from changing the intestinal flora. The majority of clinicians, however, estimate the
proportion of cases which can be ascribed to this cause at a much lower figure : 4%
(Butler, 1911); 3.5% (Goulden, 1914); 6.6% (Newton, 1925), or do not mention it at
all (Gilbert, 1929; Irons and Brown, 1923; Bulson, 1925; Gifford, 1931).
As a rule the infection is streptococcal, and in this connection the undoubted
influence of a streptococcal oral sepsis extending down to, and being absorbed from the
bowel must be remembered (Elschnig, 1925). This is not, however, the invariable
rule : the bacillus coli may be responsible, and a case of irido-cyclitis wherein a
culture of pneumococci was grown from the faeces was reported by Browning (1912–13)
which cleared up immediately and permanently on the administration of a vaccine.
Cases of irido-cyclitis and choroiditis have been noted to accompany mucous colitis
and gastro-enteritis especially in children (Viciano, 1904; Goulden, 1914; and
others). Dysentery has also been associated with irido-cyclitis on several
occasions (Vossius, 1904; Houdart, 1904; Browning, 1912–13), and Mills (1926)
reported the presence of uveitis in a large proportion of sufferers from non-dysenteric
amoebiasis, the ocular lesions improving with treatment of the amoebic infection.
Finally, in connection with gastro-intestinal infections there is evidence that a chole-
cystitis or an appendicitis may act (rarely, it is true) as a focus of infection (Lang,
1913; McGuire, 1920; Greeser, 1931, and others).
5. THE GENITO-URINARY TRACT
The male genito-urinary tract undoubtedly acts as an important factor in the
aetiology of uveitis, more especially of irido-cyclitis, and in the tract the most important
region is the deep wrethra and the prostate. In this connection, of course, gonorrhoea
must first be considered. Since gonococcal inflammations tend to have a characteristic
clinical picture, we shall discuss them separately at a later stage, but in the present
argument it is to be noted that while a severe exudative, plastic, or even haemorrhagic
irido-cyclitis occurs typically some six weeks or later after the urethral infection,
usually in association with arthritis, recurrent attacks of less severe inflammation
may re-appear persistently many years after the original infection has apparently
subsided. The first type of lesion is associated with a gonococcal septicaemia, and in
one typical case the organisms have been isolated from the eye (Sidler-Huguenin,
1911), but the second type is probably of an allergic nature. In this latter type of
inflammation it is probable that a gonococcal infection is not always alone responsible
for the eye condition ; frequently it is associated with a streptococcal one, and again a
purely streptococcal prostatitis is by no means uncommon. Thus while Irons and Brown
(1923) found 9 in their series of 200 cases of irido-cyclitis due to gonorrhoeal infection,
DISEASES OF THE UVEAL TRACT 2147
they considered 6 to be due to a non-gonococcal prostatitis; Green (1926), Benedict,
v. Lackum and Mickel (1926) and Holloway (1931) called special attention to strepto-
coccal prostatitis, while Zentmayer (1926) considered prostatic infection as a factor
in the aetiology of uveitis to be more frequently non-gonococcal than otherwise.
Pelouze (1932), indeed, concluded that 35% of all men beyond the age of 35, and 72%
of men with focal infective symptoms have a prostatic infection which is usually
secondary to tonsillar and dental sepsis with only rarely any relation to gonococcal
infection. He found that uveitis was occasionally due to this cause alone, and that
the prostatitis very frequently was an adjuvant factor in a composite streptococcal
infection of such importance that the uveitis would not clear up until the local condi-
tion in the prostate was treated.
The pelvic organs in women are probably less frequently a source of focal infection
than in the male, but their influence cannot be discounted. As a source of gonococcal
infection they are much less important : thus in 109 cases of uveitis due to this organism
Byers (1907) found only 5 women. So far as other types of infection are concerned,
there is a considerable literature on the subject. Mention has already been made
of the metastatic panophthalmitis which not infrequently occurs with puerperal
sepsis,” but a non-suppurative type of irido-cyclitis and choroiditis has for long been
associated with uterine conditions. Some older writers considered the connection
to be a reflex nervous irritation or a vaso-motor disturbance in which sexual excess
played a large part, but although ocular inflammations may sometimes coincide
with the menses (Coudron, 1878; Noblot, 1889; Puech, 1889; Pargoire, 1892 ; and
others), it is probable that this influence is due to a more ready infection of the blood-
stream from a focal infective lesion at that time. Chronic endometritis has frequently
been mentioned in the literature as a cause of uveitis (Denis, 1874; Cohn, 1890,
“Oeil uterin ‘’; de Wecker, 1891, “Iritis metridue '’; Vignes and Bataud, 1896;
Berger and Loewy, 1906; Lang, 1913; Goulden, 1914; and others), but the really
well authenticated cases are comparatively few. Thus Gifford (1931) in 118 cases of
iritis traced one only to uterine infection, and Benedict, v. Lackum and Mickel (1926), in
their summary of streptococcal pelvic infections, found scleritis as a more typical
ocular manifestation than uveitis.
In both sexes, of course, infection may arise from the urinary tract, the commonest
responsible organism being the B. coli (Davis, 1917; and others).
The difficult question of the part played by these foci of infection in
uveal inflammation may be summed up somewhat as follows. In a very
large number of cases such foci can be demonstrated—almost always more
than one ; but in others none can be found. Thus Schnabel (1931), investigat-
ing 20 cases from every point of view, found multiple foci in 17 and none in 3.
The absence of a demonstrable focus may be due in some cases to a persistence
of the ocular inflammation after the primary focus has ceased to be of
importance, the local inflammation in the eye having established itself as a
separate entity and progressing independently of the original cause, but
negative findings are probably more usually due to the existence of an infec-
tive lesion so minute that its existence defies discovery by our limited resources
of clinical examination. The most common infection is streptococcal; tuberculosis
acting allergically from a focal lesion usually in a mediastinal gland probably comes
second. Of the streptococcal foci the most common are the teeth and tonsils,
1 p. 2137.
2148
TEXT-BOOK OF OPHTHALMOLOGY
thereafter and less frequently, the nasal sinuses, then the posterior urethra and
prostate and the pelvic organs in women, and only rarely the intestinal tract with
its diverticula, the gall-bladder and appendia.
Ackland. T. O. S., xliii, 417, 1923.
Arlt. Die Krankheiten, d. Auges., Prague, 1853.
Axenfeld. A. f. O., x1 (3), 1 ; (4) 103, 1894.
Die Bact, in der Augenheilkunde, Jena, 1907.
Axenfeld and Goh. A. f. O., xliii (1), 147, 1897.
Back. K. M. Aug., lxxviii, 316, 1927.
Baeseman. J. Med. S. New Jersey, xxxi, 700,
1934.
Baum. Diss., Freiburg, 1902.
Benedict. Am. J. O., iii, 860, 1920.
T. Am. O. S., xix, 335, 1921.
A. of O., l, 560, 1921.
Benedict, v. Lackum and Mickel.
O. S., xxiv, 145, 1926. -
Berens, Angevine, Guy and Rothbard.
Am. J. O., xxi, 1315, 1938.
Berger and Loewy. Ueber Awgenerkramkungen
bei Frauen, Wiesbaden, 1906.
Billings. A. Int. Med., ix, 484, 1912.
Am. Med. As. Sect. O., 17, 1921.
Bjerrum. Acta O., v, 39, 1927.
Brown. Am. J. O., xv, 19, 1932.
A. of O., xii, 730, 1934.
T. Am. O. S., xxxiii, 435, 1935.
Brown and Drummer. A. of O., ii, 573, 1929.
Browning. Brit. Med. J., ii, 1022, 1912.
O. Rev., xxxii, 101, 1913.
Bulson. T. Am. O. S., xxiii, 292, 1925.
Butler. Brit. Med. J., i, 804, 1911.
T. O. S., xliii, 448, 1923.
Byers. Study of Systemic Manifestations of
Systemic Gomorrhoea, Studies from the
Victoria Hospital of Montreal, 1907.
Campbell. Lancet, ii, 219, 1905.
Cohn. Uterus w. Auge, Wiesbaden, 1890.
Coudron. Gaz. des Hóp., 859, 1878.
Davis. T. O. S., xxxvii, 220, 1917.
Denis. Thése de Paris, 1874.
Dwyer. A. of O., xlviii, 344, 1919.
Elschnig. Wien. kl. W., xxv, 713, 1912.
R. M. Aug., xliii (2), 417, 1905; lxxiv, 783,
1925.
Fage. Rec. d’Opht., 266, 1895; 327, 1898.
A d’O., xlvii, 538, 1930.
Finnoff. Am. J. O., xiv., 127, 1931.
Fish. Am. J. O., xxi, 353, 1904.
Fromaget. Rev. internat, d. rhinol., iii, 244,
1893.
Bull. S. fr. d’O., xxxvii, 3, 1924.
Gifford. T. O. S., xliii, 455, 1923.
Am. J. O., xiv., 100, 1931.
Gilbert. Kurzes Hb. d. O., v, 2, 1930.
Goulden. R. L. O.H. Rep., xix, 328, 1914.
Green. J. Am. Med. As., lxxxvii, 1183, 1926.
Greeser. Am. J. O., xiv, 929, 1931.
Guillery. A. f. Aug., lxviii, 242,
lxxviii, 11, 1914.
Baden. A. Int. Med., xxxii, 828, 1923.
Hartzell. J. Am. Med. As., lxiv, 1055;
lxv, 1093, 1915.
T. Am.
1911;
Henrici. J. Inf. Dis., xix, 572, 1916.
Hensen. Münch. med. W., lxxi, 470, 1924.
Herrnheiser. K. M. Aug., xxx, 393, 1892.
Hessberg. K. M. Aug., xlviii, Beil., 60, 1910.
Z. f. Aug., xl, 324, 1918.
Hoffmann. Z. f. Aug., xvi, Erg., i, 1906.
IHolloway. Am. J. O., xiv, 232, 1931.
Houdart. X Internat. Cong. O. Lucerne, 168.
1904
Iga. K. M. Aug., lxxxiv, 449, 1930.
Igersheimer. A. f. O., lxxvi, 217, 1910.
Irons. Am. J. O., xiv, 1228, 1931.
Irons and Brown. J. Am. Med. As., lxvi,
1840, 1916 ; likxxi, 1770, 1923; lxxxvii,
1167, 1926.
Irons, Brown and Nadler. A. of O., xlv., 229,
1916.
Ischreyt. A. f. Aug., xli., 38, 1900.
Julianelle. J. Eacp. Med., li, 633, 643, 1930.
Rahoun. Oft. Sbornik, vii, 269, 1932.
Kolmer. Am. J. O., xiv, 217, 1931.
Koske. Arb. a. d. kaiserl. Gesundheitsamt.,
xxii, 411, 1905.
Lang. Lancet, i, 1368, 1913.
Brit. Med. J., i, 381, 1913.
P. R. S. Med., x, Sect. O., 43, 1917.
T. O. S., xliii, 426, 1923.
Leber. A. f. O., xxvi (3), 10, 1880; lxxiii, 1,
1909.
Levy, Steinburgher and Pease.
As., lxix., 194, 1917.
Lewis. J. Am. Med. As., lxxxi, 1775, 1923.
Litten. Z. f. kl. Med., ii, 378, 1880.
Löwenstein. Münch. med. W., lxxviii, 261,
1931.
MacCallan. Dental Surgeon, xxv, 569, 1928.
T. O. S., xlviii, 181, 1928.
P. R. S. Med., xxii, 14, 1929.
A. of O., iii, 673, 1930.
Mackenty. Am. J. O., xv, 27, 1932.
MacLean. T. Am. O. S., xxxiv, 324, 1936.
McGuire. A. of O., xlix, 500, 1920.
Magnasco. Riv. ot. ecc., v, 264, 1928.
Marchesani. A. f. Aug., c-ci, 606, 1929.
Meller. Z. f. Aug., lxxvii, 1, 1932.
T. O. S., liv, 467, 1934.
v. Michel. A. f. O., xxvii (2), 171, 1881.
Münch. med. W., xlvii, 853, 1900.
Z. f. Aug., xv, 13, 1906.
Mills. A. of O., lii, 525, 1923.
J. Am. Med. As., lxxxvii, 1176, 1926.
Moody. J. Inf. Dis., xix, 515, 1916.
Moore. Am. J. O., xiv, 110, 1931.
Nettleship. R. L. O. H. Rep., ix, 182, 1879.
Newton. Teacas State Med. J., xxi, 315, 1925.
Noblot. These de Bordeaua, 1889.
Okell and Elliott. Lancet, ii, 869, 1935.
O’Leary. Am. J. O., xv, 24, 1932.
Pargoire. Thése de Paris, 1892.
Pelouze. A. of O., vii, 372, 1932.
J. Am. Med.
DISEASES OF THE UVEAL TRACT
2149
Pelouze. Am. J. O., xv, 963, 1932.
Popper. Wien. kl. W., xliv, 1494, 1931.
Puech. A. d’O., viii, 410, 1889.
Rosenow. J. Infect. Dis., xvii, 403, 1915;
xxxiii, 248, 1923 ; xlv, 331, 1929.
J. Am. Med. As., lxv, 1687, 1915.
J. Dent. Res., i, 205, 1919.
J. Am. Vet. As., lxxi, 378, 1927.
T. Am. Acad. O. and Oto-Laryngol., xxxii,
41, 1927.
Internat. Clin., ii, 29, 1930.
Am. J. O., iii, 860, 1920.
Rosenow and Nickel. Am. J. O., xv, 1, 1932.
Röth. Deut. Z. f. Chir., i, 471, 1872.
Sidler-Huguenin. A. f. Aug., lxix, 346, 1911.
Stock. K. M. Aug., xli (1), 81, 228, 1903.
Swift and Derick. P. S. Eacp. Biol. Med.,
xxv, 222, 224, 1928.
J. Eacp. Med., lii, 1, 1930. -
Swift, Derick and Hitchcock. J. Am. Med.
As., xc, 906, 1928.
v. Szily. K. M. Aug., lxxxv, 21, 1930.
Theodore and Lewson. A. of O., xxi, 829,
1939. -
Traut. J. Infect. Dis., lii, 230, 1933.
Am. J. O., xvii, 106, 1934.
Ulrich. J. Am. Med. As., lxv, 1619, 1915.
Urbanek. T. O. S., lii, 227, 1932.
Schnabel. Am. J. O., xiv, 223, 1931. Urbanek and Meller. Z. f. Aug., lxxvii, 17,
Schultz and Swift. J. Eacp. Med., Iv, 591, 1932. 1932.
de Schweinitz. Am. Med. As., T. Sec. O., Viciano. A. de Oft. H.-A., 674, 1904.
158, 1902; 332, 1906.
An. of O., xv, 498, 1906.
Intermat. Cong. Med. London, Sec. O., 7,
1913.
Brit. Med. J., ii, 396, 1913.
Dental Cosmos, lxii, 565, 1920.
Bull. S. fr. d’O., xxxvii, 8, 1924.
Vignes and Bataud. A. d’O., xvi, 449, 1896.
Vossius. Die oph. Kl., ii, 17, 1904.
Wagenmann. A. f. O., xxxiii (2), 147, 1887.
Walter. J. Am. Med. As., lxxxv, 1720, 1925.
de Wecker. Semaine Méd., 86, 1891.
v. Wecker, G. S. Hb. I., iv, 485, 1876.
Wood, D. T. O. S., xlv. (2), 724, 1925.
de Schweinitz and Fife. O. Rec., xvii, 347, Woods. A. of O., xlv., 451, 1916; xvii, 1,
1908. 1937.
Seegal and Seegal. Proc. S. Eacp. Biol. Med., Zentnayer. J. Am. Med. As., lxxxvii, 1172,
xxvii, 390, 393, 1930. 1926.
J. Immunol., xxv, 221, 1933. Zentmayer, Chance, Risley and Ziegler.
Seegal, Seegal and Khorazo.
xxv, 207, 1933.
Senn and Spirig. Korresp. f. Schw. Aertze,
xxx, No. 17, 1900.
J. Immunol., Therapeutics Gazette, xxiii, 521, 1907.
Ziegler. T. O. S., xliii, 452, 1923.
Ziem. Cb. pr. Aug., xl, 358, 1887.
(C) Uveitis Associated with Generalized Infectious Diseases
A large number of acute febrile diseases are associated with inflammatory
evidences, usually of an acute exudative nature, in the uveal tract, particu-
larly in its anterior segment. Little is known of the precise aetiology of such
cases since opportunities for bacteriological studies are very difficult to
obtain, but the most probable mechanism is a direct action upon the uveal
tissues of the infective agent circulating in the blood-stream. The responsible
organisms have been isolated from the eye in cases of irido-cyclitis in
typhoid fever (de Grandmont, 1892; Mayou, 1906), in influenza (Casali,
1907), and in cerebro-spinal meningitis (Wintersteiner, 1901; Mayou,
1906; McKee, 1908; Weakley, 1916), and if the acute irido-cyclitis with
hypopyon which accompanies the febrile stage of gonorrhoea be admitted
into this category, in this disease also (Sidler-Huguenin, 1911). Presumably
in the other infections a similar mechanism is at work, a conclusion supported
by the occasional occurrence in certain of them of actual panophthalmitis
undoubtedly due to metastatic origin.
1. THE ACUTE EXANTHEMS
(i) MEASLES (MORBILLI). In measles a primary iritis does not occur. A secondary
iritis may be associated with a corneal ulcer ; and metastatic manifestations are by
no means unknown. Occasionally these may remain localized, producing, for example,
2150 TEXT-BOOK OF OPHTHALMOLOGY
a patch of choroiditis, but a generalized uveitis usually results, sometimes developing
into a pseudo-glioma (Müller, 1894; Davis, 1905; Passow, 1919; Thies, 1926).
(ii) scARLET FEVER (SCARLATINA). Scarlet fever produces no direct uveal com-
plications: in the literature, however, uveitis has been noted (Zlocisti, 1901 ; Villard,
1904), and a metastatic uveitis may follow a secondary otitis media or a meningitis
(Weeks, 1885; and others).
(iii) SMALL-Pox (VARIOLA) AND VACCINIA. A primary iritis, frequently associated
with choroiditis and vitreous opacities, is not an uncommon complication of small-
pox, occurring in 17% (Coccius, 1871) or 13% (Adler, 1874) of cases in pre-vaccination
days. The uveitis usually comes on about the 8th to the 12th day of the illness and is
generally sero-plastic in character and not of great severity (Hutchinson, 1866; v.
Graefe, 1869 ; Bouchard, 1871; Hackenburg, 1872; and others). Some considerable
time after the illness patches of trabecular atrophy may be seen in the iris, especially
in the ciliary region (50% of all cases, Russo, 1933), as well as a general disturbance
and dispersal of the iris pigment. These rounded areas of post-variolar atrophy in the
stroma are completely devoid of pigment and probably represent the effect of toxic
products carried by the blood-stream ; they have been mis-diagnosed as vitiligo.
Saltini (1894) observed a case of metastatic ophthalmia after vaccination in which
the globe had to be enucleated.
(iv) CHICKEN-Pox (VARICELLA). An acute uveitis resulting in phthisis bulbi
was noted by Hutchinson (1886–87) in a case of chicken-pox—but whether this was
incidental or not is difficult to say. Depigmented atrophic patches resembling those
seen after small-pox have been described (Löwenstein, 1932).
2. ACUTE INTESTINAL INFECTIONS
(i) TYPHOID FEVER. Uveal complications in typhoid fever are not common,
occurring only in a small proportion of cases. In 767 cases Larinow (1878) found
5 cases of iritis; in 82 cases the only ocular complication noted by Paul (1906) was a
retinal haemorrhage ; and in 100 cases Krückmann (1907) found two showing patches
of choroiditis. Iritis with hypopyon has been noted (de Grandmont, 1892; Mayou,
1906; Goldstein, 1919), choroiditis (Hersing, 1872; Hotz, 1885), and panophthalmitis
(v. Millikin, 1899; Mansilla, 1922), while the organism has been recovered from the
eye (de Grandmont, 1892; Mayou, 1906).
Irido-cyclitis has been noted following an anti-typhoid vaccination (Prélat, 1917).
(ii) CHOLERA. A toxic iritis has been noted as a rarity (Williams, 1885).
(iii) DYSENTERY. We have already seen + that BACILLARY DYSENTERY is asso-
ciated occasionally with a metastatic sclero-conjunctivitis: sometimes a severe
anterior uveitis occurs (Vossius, 1904; Houdart, 1904; Browning, 1912–13) fre-
quently but not invariably associated with inflammatory manifestations in the joints
(Morax, 1917; Cosse and Delord, 1917; Maxwell, 1918; Maxwell and Kiep, 1918;
Kiep, 1919). Usually the irido-cyclitis appears later than the sclero-conjunctivitis,
the latter commencing from the 10th to the 20th day after the onset of intestinal
symptoms, and the former from the 20th to the 40th day (Toulant and Sarrony, 1936):
an interval as short as 12 days (Maxwell and Kiep, 1918) or as long as 88 days may
elapse (Graham, 1919). As a rule the prognosis is good with control of the dysenteric
infection, although relapses may occur. The interesting feature of the condition,
however, is that, like a gonococcal infection of the urethra, an articulo-ocular syndrome
develops.
AMCEBIC DYSENTERY, on the other hand, is not usually associated with uveal
symptoms, but cases have been recorded wherein an irido-cyclitis occurred with this
type of dysenteric infection suggesting a close relationship between the two (Houdart,
1904; Luna, 1918; Pagès, 1933). The irido-cyclitis is usually acute and purulent
1 Vol. II, p. 1547.
DISEASES OF THE UVEAL TRACT 2151
with a hypopyon, and while the ocular inflammation associated with bacillary
dysentery is probably a toxic manifestation, this type resembles a direct metastatic
infection analogous to the hepatic and cerebral abscess which occur in this disease.
3. BLOOD INFECTIONS
(i) MALARIA. Castellani and Chambers (1919) have found the malarial parasite
in the choroidal vessels, but although a few cases of serous irido-cyclitis have been
noted in malaria (Adams, 1881; Selûck, 1889; Péchin, 1899; Kirkorow, 1899), it
would seem from their paucity that the infection has no special predilection for the
uvea (Maxwell, 1918). The iritis is typically a mild relapsing condition, and a chorio-
retinitis is rare (Bywater, 1922; Marin, 1923). Poncet (1878) found histological
changes in the choroid suggesting the after-effects of pigmentary emboli such as have
been noted in other organs.
(ii) RELAPSING FEVER (RECURRENT FEVER : SPIROCHAETOSIS). Relapsing fever
in its several forms has been associated with uveitis since the time of Mackenzie (1830)
who had experience of an epidemic in Dublin in 1826. The inflammation occurs in
from 2% to 12% of cases, and is probably toxic in nature ; it may be either acute or
chronic, the first type occurring preferentially in young persons, the second in old
(Logetschnikow, 1870). In 15% to 20% of cases it is bilateral. It may take the form
of an acute (Peltzer, 1872; Adamūck, 1894) or chronic iritis (Estlander, 1869), an
irido-cyclitis (Müller, 1873; Lübinsky, 1886), Ewetzky, 1897; Groenouw, 1900), a
choroiditis (Estlander, 1869 ; Trompetter, 1880), or a complete uveitis (Blessig, 1867).
(iv) SPIROCHAETOSIS ICTERO-HAEMORRHAGICA (WEIL's DISEASE). An irido-cyclitis,
usually associated with conjunctivitis, occurs occasionally in Weil's disease (Weil,
1886; Pfuhl, 1888; Herrnheiser, 1892; Hertel, 1917; Weekers and Firket, 1917;
Will, 1928); Weekers and Firket (1917) met 6 in 60 cases, and Moret (1917) 2 in 72.
Occasionally the inflammation is a serious one accompanied by hypopyon, apparently
of metastatic origin ; more usually the symptoms are slight, running parallel with the
disease, recrudescing with the frequent relapses, and not disappearing until con-
valescence is established.
4. RESPIRATORY FEVERS
(i) INFLUENZA. Inflammatory affections of the uveal tract occur in some 8% of
cases of influenza (Groenouw, 1904). They may be divided into two types, an acute,
but relatively mild uveitis, presumably of toxic origin, and a destructive purulent
inflammation of a metastatic type associated with septicaemia. The first usually takes
the form of serous irido-cyclitis, occasionally of a complete uveitis, but an exudative
choroiditis is relatively rare : it may be of considerable severity, sometimes with a
gelatinous exudate going on to seclusion and occlusion of the pupil with the production
of many vitreous opacities (Adler, 1890; Badal and Fage, 1890; Gutmann, 1890;
Pflüger, 1890; Truc, 1898; Péchin, 1900; Fraenkel, 1920; Natanson, 1901; Green
and Green, 1919). The metastatic cases of hypopyon irido-cyclitis or panophthalmitis
are rare and occur usually in exceptionally severe cases, the bacteriological findings
including the staphylococcus (Eversbusch, 1890; Lavagna, 1894), the pneumococcus
(Alfieri, 1897) and the Haemophilus Influenzae (Tanja, 1898; Casali, 1907, and others)
(see z. Nedden, 1906; Samperi, 1919; Onate, 1919; Sédan, 1935).
(ii) whoopi NG COUGH (PERTUssIs). A case of irido-cyclitis has been reported
as occurring with whooping cough, but the relation between the two was not proven
(Chronis, 1905).
5. CEREBRO-SPINAL INFECTIONS
In CEREBRO-SPINAL MENINGITIs a metastatic uveitis occurs with some frequency
(Knapp, 1865; Wilson, 1867): during a typical epidemic in 100 cases Heine (1905) en-
countered 5 instances; v. Goeppert (1905) 3 in 44; Radmann (1905) 3 in 61; and
215.2 TEXT-BOOK OF OPHTHALMOLOGY
Curtius (1905) 7 in 200. The incidence, however, varies considerably. Thus in Detroit,
Gordon (1927–31), in 1090 cases met 4 cases of irido-cyclitis, 1 of choroiditis, 1 of
uveitis, and 9 of panophthalmitis; Lazar (1936) in 575 cases met 9 cases of bilateral,
19 of unilateral uveitis, and 2 of panophthalmitis; while Lewis (1936) in 200 cases
met 15 (7.5%) of endophthalmitis of which 11 were unilateral and 4 bilateral. The
complication usually comes on towards the end of the first week of the illness. Occa-
sionally the inflammation is relatively mild in degree and exudative in type (Heine,
1905; Lazar, 1937); more usually it is frankly suppurative, but, even so, not of the
most severe type in that, starting usually as an iritis with hypopyon, after a
relatively stormy course, it resolves typically as a pseudo-glioma without involving
much shrinkage of the globe (Nettleship, 1883–85; Jones, 1884 : Collins, 1892;
Eleming, 1900; Cross, 1900; Stephenson, 1900; Mayou, 1906; Haden, 1918;
Metz-Klok, 1924; and many others). The infection is blood-borne, for positive
cultures of the meningococcus have been found in the blood (Martini and Rohde,
1905; Lewis, 1936) and in the eye (Wintersteiner, 1901; Uhthoff, 1905; Mayou,
1906; McKee, 1908; Weakley, 1916), while the optic nerve sheath is usually normal.
There is no doubt, however, that early treatment with anti-serum greatly reduces
the number of ocular complications (Lazar, 1936).
Not all uveal inflammations following meningeal infection are due to the meningo-
coccus. A panophthalmitis of this type, for example, has been noted after an influenzal
meningitis (Bajardi, 1890), or with meningitis complicating scarlet fever (Weeks, 1885)
or septic conditions (Shears, 1884; Silcock, 1900), particularly otitis media (Collins,
1892; Axenfeld, 1896; Cargill, 1898; and many others). Apart from blood-borne
metastatic infections, some of these have been pathologically proved to have resulted
from direct spread from the cranial cavity along the optic nerve (Hofmann, 1886;
Collins, 1892; and others), although this route is probably unusual (Axenfeld, 1907).
6. INFECTIONS OF THE SALIVARY GLANDS
(i) MUMPS (EPIDEMIC PAROTITIS). An iritis, usually of not a very severe nature,
has been noted to occur with mumps (Zossenheim, 1892; de Micas, 1907; Hack,
1909; Lundsgaard, 1916; Rieth, 1919; Popovici, 1929).
(ii) UVEO-PAROTITIS. Quite apart from mumps, a well-defined clinical entity of
unknown atiology occurs, characterized by a bilateral chronic uveitis and parotitis
frequently associated with paresis of the cranial nerves, particularly the Seventh ; this
will be dealt with subsequently.”
Adams. R. L. O. H. Rep., x, 214, 1881. Castellani and Chambers. Manual of Tropical
Adamück. Prozessen. Wratsch, 1041, 1894.
Adler. A. f. Derm. w. Syph., i, 175, 1874.
Weim. med. W., xl, 140, 1890.
Alfieri. A. di Ott., iv, 328, 1897.
Axenfeld. Die Bact. in der Augenheilkunde,
Jena, 1907.
M. f. Psych. Neurol., ii, 413, 1896.
A. f. O., xl (3), 1 ; (4), 103, 1894; xlii, 101,
1896. -
Badal and Fage. A. d’O., x, 136, 1890.
Bajardi. Policlinica, i, 264, 1890.
Blessig. K. M. Aug., v, 291, 1867.
Bouchard. Gaz. d. Hôp. Paris, xliv, 393, 1871.
Browning. Brit. Med. J., ii, 1022, 1912.
O. Rev., xxxii, 101, 1913.
Bywater. T. O. S., xlii, 359, 1922.
Cargill. T. O. S., xviii, 131, 1898.
Casali. Am. di Ott., xxxvi, 120, 1907.
Medicine, 1919.
Chronis. K. M. Aug., xliii (2), 663, 1905.
Coccius. De Morbis oculi humani, Leipzig,
1871.
Collins. R. L. O. H. Rep., xiii, 361, 1892.
Collins and Mayou. Path. and Bact. of the
Eye, London, 1911.
Cosse and Delord. An... d’Oc., cliv, 33, 1917.
Cross. T. O. S., xx, 117, 1900.
Curtius. Med. Kl., i, 780, 805, 1905.
Davis. Post-Graduate, N.Y. xx, 800, 1905.
Estlander. A. f. O., xv (2), 108, 1869.
Eversbusch. Münch. med. W., xxxvii. 89,
114, 1890.
Ewetzky. Westn. O., xiv, 51, 1897.
Fleming. T. O. S., xx, 130, 1900.
Fraenkel. Dewt, med. W., xlvi, 673, 1182,
1920.
1 p. 2355.
DISEASES OF THE UVEAL TRACT
2153
v. Goeppert. Berlin. kl. W., xlii, 644, 688,
Goldstein. Diss. Heidel., 1919.
Gordon. Med. Rep. of Herman Kiefer Hosp.
Detroit, 1927–31.
v. Graefe. A. f. O., xv (3), 193, 1869.
Graham. P. R. S. Med. (Sec. Med.), xiii, 23,
1919.
de Grandmont. A. d’O., xii, 623, 1892.
Green and Green. Am. J. O., ii, 607, 1919.
Groenouw. K. M. Aug., xxxviii, 224, 1900.
G.-S. Hb. II, xi (1), 618, 1904.
Gutmann. Berlin. kl. W., xxvii, 1111, 1139,
1890.
Hack. A. f. Aug., lxiv, 137, 1909.
Hackenburg. Diss. Berlin, 1872.
Baden. Am. J. O., i, 647, 1918.
Heine. Berlin. kl. W., lxii, 772, 1905.
BHerrnheiser. Prag. med. W., xvii, 492, 1892.
IIersing. A. f. O., xviii (2), 69, 1872.
Hertel. A. f. O., xciv, 28, 1917.
IHofmann. Newrol. Centb., 357, 1886.
Hotz. Am. J. O., ii, 222, 1885.
Houdart. X. Internat. Cong. O. Lucerne, 168,
1904.
Hutchinson. R. L. O. H. Rep., v, 333,
1866; vi, 146, 1867.
O. Rev., v, 241, 1886.
Jones. T. O. S., iv, 90, 1884.
Riep. T. O. S., xxxix, 298, 1919.
Kirkorow. Vest. O., xvi, 19, 1899.
Enapp. Cb. f. med. Wiss., iii, 513, 1865.
Erückmann. G-S. Hb., II, v (6), 127, 1907.
Larinow. K. M. Aug., xvi, 487, 1878.
Lavagna. Gior. d. R. Accad. Med. Torino,
117, 1894.
Lazar. A. of O., xvi, 847, 1936.
Am. J. O., xx, 928, 1937.
Lewis. T. Am. O. S., xxxiv, 284, 1936.
Logetschnikow. A. f. O., xvi (1), 353, 1870.
Löwenstein. K. M. Aug., lxxxix, 790, 1932.
Lübinsky. Westm. O., iii, 449, 1886; iv., 1,
1887.
Luna. Am. J. O., i, 658, 1918.
Lundsgaard. K. M. Aug., lvii, 393, 1916.
Mackenzie. Practical Treatise on Diseases of
the Eye, 1830.
Mansilla. Rev. Cub. de O., iv, 136, 1922.
Marin. A. de Oft., H.-A., xxiii, 663, 1923.
Martini and Rohde. Berlin. kl. W., xlii, 997,
1905.
Maxwell. Brit. J. O., ii, 406, 412, 1918.
Maxwell and Kiep. Brit. J. O., ii, 71, 1918.
Mayou. R. L. O. H. Rep., xvi, 565, 1906.
McKee. O. Rec., xvii, 438, 1908.
Metz-Klok. K. M. Aug., lxxiii, 379, 1924.
de Micas. Rec. d’O., 413, 1907.
v. Millikin. T. Am. O. S., xxxv, 535, 1899.
Morax. An. d’Oc., cliv, 45, 1917.
Moret. A. med. Belg., lxx, 1105, 1917.
Müller. Diss., Greifswald, 1873.
Diss., Breslau, 1894.
Natanson. Wratsch, xxii, 1600, 1901.
z. Nedden. K. M. Aug., xli (1), 209, 1903;
xliv (1), 479, 1906. *
Nettleship. T. O. S., iii, 36, 1883; v, 101,
1885.
Onate. Rev. Cub. de O., i, 328, 1919.
Bagès. Folia O. Orient., i, 305, 1933.
Passow. A. f. Aug., lxxxv, 277, 1919.
Paul. K. M. A ug., xliv (2), 73, 1906.
Péchin. Rec. d’O., xix, 390, 1899; xxi, 129,
1900.
Peltzer.
Pfuhl.
Pflüger.
1890.
Poncet. Am... d’Oc., lxxix, 201, 1878.
Popovici. Cluj. med., x, 465, 1929.
Brélat. A. d’O., xxxv, 742, 1917.
Radmann. Deut. med. W., xxxi,
1020, 1905.
Rieth. K. M. Aug., lxiii, 527, 1919.
Russo. A. di Ott., vi, 923, 1933.
Saltini. Rass. de Sc. Med., Modena, 1894.
Samperi. A. di Ott., xxvi, 19, 1919.
Sédan. An. d’Oc., clxxii, 375, 1935.
Selück. Russ. Med., No. 1, 1889.
Shears. Liverpool Med.-Clir. J., iv,
1884.
Sidler-Huguenin. A. f. Aug., lxix, 346, 1911.
Silcock. T. O. S., xx, 112, 1900.
Stephenson. T. O. S., xx, 121, 1900.
Tanja. Ned. Tij. v. Gen., xxxiv. (1), 735, 1898.
Thies. K. M. Aug., lxxvii, 379, 1926.
Toulant and Sarrony. A. d’O., liii, 523, 1936.
Trompetter. K. M. Aug., xviii, 123, 1880.
Truc. Rev. gen. d’O., 318, 1898.
Uhthoff. B. O. G. Heidel., xxxii, 84, 1905.
Villard. Monpellier Méd., xviii, 354, 1904.
Vossius. O. Kl., ii, 17, 1904.
Weakley. Brit. Med. J., i, 47, 1916.
Weekers and Firket. A. d’O., xxxv, 647,
1917.
Weeks. Cb. pr. Aug., ix, 129, 1885.
Weil. Deut. A. f. kl. Med., xxxix, 209, 1886.
Will. Z. f. Aug., lxiv, 222, 1928.
Williams. St. Louis Med. Surg. J., xlviii,
246, 1885.
Wilson. R. L. O. H. Rep., vi, 57, 1867.
Wintersteiner. Wien. kl. W., xiv, 996, 1901.
Zlocisti. Diss., Freiburg, 1901.
Zossenheim. Beit. z. Aug., iv, 68, 1892.
Berlin. kl. W., ix. 444, 1872.
Deut. milit. Z., xvii, 385, 1888.
Berlin. kl. W., xxvii, 601, 637, 663,
707,
436,
(D) Uveitis Associated with Diseases of the Skin
In infective conditions of the skin secondary inflammations of the
uveal tract are not uncommon. Thus in erysipelas metastatic infection may
lead to a purulent irido-cyclitis and panophthalmitis (Fortunati, 1889;
2154 TEXT-BOOK OF OPHTHALMOLOGY
Hansen, 1891; Thier, 1900), and from such eyes the streptococcus has been
recovered (de Grandmont, 1892). Staphylococcal processes, such as a
furuncle or carbuncle, may give rise to the same condition (Schanz, 1906;
Gilbert, 1920–25; Laval, 1937). In other conditions an iritic involvement
is secondary to a keratitis, as in pemphigus (Pflüger, 1878; Steffan, 1885), or
epidermolysis bullosa (Cohen and Sulzberger, 1935).
In others a toacic element may enter into the question, as the iritis in
urticaria (Peschel, 1886), erythema nodosum (Reis, 1906), erythema
exudativum multiforme (Bailey, 1931), or psoriasis (Morax, 1893), or as the
choroiditis in lichen (Schimanowsky, 1908); while in still others a parallel
lesion may affect the skin and the eye, as the involvement of the ciliary body
in erythema nodosum (Gilbert, 1920), or the choroiditis occurring in conjunc-
tion with lupus erythematosus which may be miliary in type (Abramowicz
and Delewicz, 1933; Semon and Wolff, 1933; Pillat, 1935).
Such is probably the case also in acne rosacea, wherein a nondescript iritis asso-
ciated with the corneal lesion may occur (Blancke, 1905; Doggart, 1930). Alterna-
tively, a more rare pathognomonic type of inflammation—ROSACEA IRITIS-may be
evident. The latter is characterized by swelling and abolition of the markings
of the ciliary zone of the iris with a considerable amount of neo-vascularization, while
the pupillary zone remains relatively normal : the latter portion only responds to
mydriatics. -
The most interesting condition belonging to this group, however, is the uveitis
associated with poliosis, alopoecia, vitiligo, and dysacousia, but as this presents peculiar
pathological problems it will be dealt with separately."
Abramowicz and Delewicz. An. d’Oc., clxx, Olah. K. M. Aug., xc, 61, 1933; c, 714,
599, 1933. 1938.
Bailey. A. of O., vi, 362, 1931. Peschel. Rap. di Servizi Ocul. nell’Ospizio di
Blancke. Diss., Diessen, 1905. Carita di Torino, 1886.
Cohen and Sulzberger. A. of O., xiii, 374, Pflüger. K. M. Aug., xvi, l, 1878.
1935. Pillat. A. of O., cxxxiii, 566, 1935.
Doggart. T. O. S., l, 98, 1930. Reis. K. M. Aug., xliv (2), 203, 1906.
Fortunati. Rif. Med., v., 1478, 1889. Schanz. Z. f. Aug., xvi, Erg., 58, 1906.
Gilbert. A. f. Aug., lxxxvi, 29, 50 ; lzxxvii, Schimanowsky. K. M. Aug., xlvi (1), 660,
27, 1920; xcvi, 119, 1925. 1908.
de Grandmont. A. d’O., xii, 623, 1892. Semon and Wolff. Proc. R. S. Med., xxvii,
Hansen. Nord. O. Tidschr., iv, 29, 1891. 153, 1933. t
Laval. A. of O., xviii, 104, 1937. Salus. Med. Kl., i, 865, 1936.
Morax. An. d’Oc., cir, 368, 1893. Steffan. K. M. Aug., xxiii. 214, 1885.
Thier. K. M. Aug., xxxviii, 643, 1900.
(E) Uveitis due to Metabolic Conditions
In contra-distinction to the older writers who described a large propor-
tion of cases of uveitis as being due to metabolic states such as rheumatism,
gout, diabetes, etc., present-day thought ascribes less and less to such an
aetiology and more and more to an infective origin. It is true that affections
of the uveal tract are common in these states, but their rôle would
seem to be rather that of lowering the general resistance of the patient to
infective processes and of preparing a soil suitable for the flourishing of other
1 p. 2364.
DISEASES OF THE UVEAL TRACT 2155
pathological influences which otherwise might never have gained a footing.
1. RHEUMATISM. Until comparatively recent times rheumatism has
always entered largely into the aetiology of uveitis; to-day, although the
co-existence of rheumatic manifestations and uveitis is widely recognized,
the two conditions are properly regarded, not as cause and effect, but as
parallel symptoms of the same underlying infective process. Thus rheumatic
iritis, which Krückmann (1907) in the second edition of the Graefe-Saemisch
Handbook placed first in his classification, is a term which is, or should be,
now abandoned, the articular and muscular rheumatic lesions being con-
sidered, like the iritis, as manifestations of a streptococcal infection focally
determined. Actually, uveitis is extremely rare in acute rheumatism (de
Schweinitz, 1913); the association occurs typically in the more chronic
manifestations of the disease. It is well known that, particularly in the
severe types of arthritic affections involving the limbs or the spine, an
exceedingly recalcitrant and destructive inflammation of the plastic type
is common in all parts of the uveal tract involving frequently a combination
of irido-cyclitis and choroiditis (Kunz, 1933, and many others), but although
such ocular manifestations may be called rheumatic from the clinical point
of view, they belong aetiologically to the class of allergic inflammations
usually of streptococcal and sometimes of gonococcal origin.
One interesting type of a quiet non-destructive irido-cyclitis, which leaves behind
synechiae and little else, should be noted here. It occurs usually in young children
(and hence has sometimes been diagnosed as tuberculous) who suffer from chronic
septic polyarthritis (Still's disease), and is frequently associated with a band-shaped
opacity in the cornea.” Such cases have been reported by Fuchs (1918), Uhthoff
(1918), Behmann (1921), Hauptvogel (1922), Waubke (1922), Friedländer (1933),
Walsh and Chan (1934), and Holm (1935). The irido-cyclitis usually appears some
time after joint manifestations have developed, but it may precede the arthritis by a
year or two ; its course is insidious, the fundus remains clear, and the vision usually
is ultimately good.
The combination of rheumatic fever and purpura (peliosis rheumatica of Schönlein)
has been noted to be associated with a hypopyon irido-cyclitis (Motolese, 1936).
A third association is a subacute recurringiritis with spondylarthritis ankylopoietica,
an inflammatory infection of the spine of a chronic nature occurring usually before
middle age and characterized by the formation of spurs, lips and overgrowths of the
bodies of the vertebrae (Fränkel, 1904–07; Kraupa, 1933; Teschendorf, 1933; Kunz,
1933–35; Strebel, 1935). Some authorities associate it with endocrine disturbance,
and parathyroidectomy has been claimed to have given good results (Strebel, 1935).
2. GouT. Gout as a cause of inflammatory conditions of the eye-ball
is rarely quoted to-day, but a GOUTY IRITIS (Hutchinson, 1885) or IRITIS
URICA (Krückmann, 1907) figured largely in the older literature. It may be
true that many cases classed under this head would now be considered to
be of infective origin, and that the “gouty diathesis '' was frequently a
convenient label to attach to cases wherein the origin was obscure ; but
that intensely painful and sometimes destructive inflammations of the
1 Vol. II, p. 2009.
2156 TEXT-BOOK OF OPHTHALMOLOGY
uveal tract due to gout did occur commonly in past years, and do occur
rarely to-day, is undoubted. Their peculiar clinical symptoms and pathology
will be considered at a later stage."
3. DIABETES. In the older literature DIABETES MELLITUS has always
figured in the aetiology of uveitis, particularly in inflammations of the iris.
Attention was first adequately drawn to the question by Leber (1885), and
since his time various estimates of its incidence have been given, but in
recent times these have been small. Thus in Europe von Noorden's (1927)
series of 477 diabetics, of which 58.3% showed ocular complications,
contained 2 cases only, and in America Spalding and Curtis (1927) dealing
with 307 diabetics, of which 41% had ocular complications, did not note
iritis at all. Again, in England, Foster Moore (1920), in 62 diabetic patients,
found one case only in which the iritis could not be attributed to a definite
cause other than diabetes. A diabetic choroiditis is not mentioned in the
literature. It is probable, therefore, that such inflammations as do occur
are not really diabetic in origin, but are due to organismal infection appearing
more readily in the subjects of diabetes owing to the peculiar reduction of
resistance which is associated with this disease. In the same way post-
operative infections are peculiarly liable to occur in these subjects (Uhthoff,
1908; and others). In diabetes, however, certain definite changes in the
uveal tract tend to occur—an oedema and loosening of the posterior layer
of the iris, an infiltration of the tissues with glycogen, an increased
vascularization, and, more rarely, a lipaemia, but as those are not of an
inflammatory nature, they will be discussed elsewhere.”
4. AUTO-INTOxTCATION. Although the problem had been mentioned
by Baquis (1899), the connection between uveitis and intestinal auto-
intoxication was first seriously raised by Elschnig (1905), who, on the basis
of finding indican in the urine, ascribed a large number of cases of chronic
and recurrent irido-cyclitis to the effects of a perverted metabolism and the
absorption of toxic products from the bowel. Elschnig (1912–25), in fact,
went so far as to ascribe to this influence so large a proportion as 17% to 19%
of all cases of iritis, claiming relief of the symptoms when other methods
had failed by a regulated diet and the administration of intestinal antiseptics.
Most writers, however, agree that such a proposition is inadmissible
(v. Hippel, 1911–12; Stuelp, 1911; Bernheimer, 1912). It certainly is the
case that there is no scientific evidence for the production of uveal inflamma-
tion by this mechanism, for although Wessely (1915) produced inflamma-
tion and atrophy of the choroid by the intra-ocular injection of bile salts,
such a reaction is in no way comparable. Auto-intoxication undoubtedly
predisposes to the incidence of uveitis by lowering the resistance to
infections generally, but is only admissible as a direct aetiological factor in
so far as excessive bacterial activity in the bowel may enable it to act as a
1 p. 2380. 2 p. 2383.
DISEASES OF THE UVEAL TRACT 2157
source of organismal infection in the same manner as other septic foci (de
Schweinitz, 1906–13; de Schweinitz and Fife, 1908).
5. RENAL DISEASE. An association between irido-cyclitis and nephritis
has long been remarked and a causal relationship between the two was
suggested by Leber (1885), v. Michel (1900), Baum (1902), and Gutmann
(1905). We shall see that advanced sclerotic changes may occur in the
blood-vessels throughout the entire uveal tract in this disease, and it has
been suggested that their presence is responsible for the difficult com-
plication of post-operative iritis which tends to occur in such subjects
(Gilbert, 1921 ; Mylius, 1934). Similarly, it is probable that a primary
iritis is not essentially associated with nephritis, and that the undoubted
tendency which statistics reveal for uveal inflammations to occur in these
cases, is explained adequately by the greater liability of the tissues to
succumb to a streptococcal or other infection (Löhlein, 1909).
Baquis. Settimama Med., liii, 582, 1899.
Baum. Diss., Freiburg, 1902.
Behmann. K. M. Aug., lxvi, 450, 1921.
Bernheimer. Wien. kl. W., xxv, 410, 1912.
IElschnig. Wien. kl. W., xxv, 61, 1908.
K. M. Aug., xliii (2), 417, 1905; lxxiv,
783, 1925.
Fränkel. Fortschr. d. Róstr., vii, 72, 1904;
xi, 171, 1907.
Friedländer. Ugeskrift f. Loeger., No. 44,
1190, 1933.
Fuchs. K. M. Aug., lxi, 10, 1918.
Gilbert. Münch. med. W., lxviii, 979, 1921.
Gutmann. Deut. med. W., xxxi, 1671, 1905.
Hauptvogel. K. M. Aug., lxix, 763, 1922.
v. Hippel. A. f. O., lxxix, 350, 1911;
lxxxi, 105, 1912.
Holm. T. O. S., lv., 478, 1935.
Boll. d’Ocul.., xv, 711, 1936.
Hutchinson. T. O. S., v., 1, 1885.
Fraupa. K. M. Aug., xci, 493, 1933.
Rrückmann. G.-S. Hb., II, v (6), 35, 1907;
Leber. A. f. O., xxxi (4), 183, 1885.
Löhlein. Arb. aus d. path. Instit. 2. Leipzig,
BI.4, 1909.
v. Michel. Münch. med. W., xlvii, 853, 1900.
Moore, Foster. T. O. S., xl, 15, 1920.
Motolese. Boll. d’Ocul.., xv, 711, 1936.
Mylius. B. O. G. Heidel., l, 138, 1934.
von Noorden. Zuckerkrankheit, Berlin, viii,
1927.
de Schweinitz. A. of O., xxxv, 449, 1906.
Brit. Med. J., ii, 396, 1913.
Trans. Internat. Cong. Med.,
Sec. O., 7, 1913.
de Schweinitz and Fife.
i, 2040, 1908.
Spalding and Curtis.
cxcvii, 165, 1927.
Strebel. K. M. Aug., xcv, 786, 1935.
Stuelp. A. f. O., lxxx, 548, 1911.
Teschendorf. Deut. med. W., ii, 1576, 1933.
Uhthoff. B. O. G. Heidel.., xxxv, 64, 1908.
R. M. Aug., lx, 11, 1918.
London,
J. Am. Med. Ass.,
Boston Med. Surg. J.,
vi, 12, 1907. Walsh and Chan. Am. J. O., xvii, 238, 1934.
Runz. K. M. Aug., xci, 153, 1933; xcv, Waubke. K. M. Aug., lxix, 79, 1922.
486, 1935. Wessely. A. f. Aug., lxxix, 1, 1915.
A summary of the aetiology of endogenous uveitis is not an easy matter.
In general terms, among adults in Great Britain, at any rate, THE COMMONEST
CAUSE IS WITHOUT DOUBT FOCAL INFECTION IN WEHICH BY FAR THE COM-
MONEST RESPONSIBLE ORGANISM IS THE STREPTOCOCCUs with the gonococcus
as a poor second, AND THE COMMONEST SITES THE TEETH, THEN THE TONSILS,
AND THEN THE PROSTATE. THE SECOND PLACE IS OCCUPIED BY TUBERCLE,
and since true metastatic tubercle is rare and the majority of cases are
focally determined (usually from tuberculous adenitis), there is thus a great
preponderance of the focal type of lesion. THE THIRD PLACE IS OCCUPIED BY
SYPHILIS ; THE FOURTH BY METASTATIC LESIONS ; THE FIFTH BY ACUTE
INFECTIOUS DISEASES AND EXANTHEMATA ; and THE SIXTH BY SKIN DISEASEs ;
while in a large residuum of cases the aetiology is obscure and undetermined.
2158 TEXT-BOOK OF OPHTHALMOLOGY
So far as the various parts of the weal tract are concerned the same ratio holds
good, with the exception that in the choroid the proportion due to tubercle
is higher than in the anterior segment. There is, however, a definite
variation with age : in infancy the disease is rare and at this stage pride of
place used to be, and probably still is, occupied by congenital syphilis, while
metastatic conditions come second. In adolescence tuberculosis is the most
common cause ; and as age advances focal infection gradually becomes more
important and eventually predominates. So far as sea is concerned, on the
whole both are affected equally, but the large number of women affected
after the menopause should be noted.
In different countries the incidence undoubtedly varies. In America it
seems to be much the same as in England, although most writers place syphilis
before tubercle. In central Europe tubercle is given pride of place over focal
infection, but while this may well be the case since tubercle is probably much
more prevalent among this population than in Britain and America, one
suspects that the teeth and tonsils of the Germans and Austrians are, in a
quiet way, almost as malicious as those of the Anglo-Saxons. In the tropics
syphilis is much the commonest cause, accounting for the great majority of
cases in some communities, and a percentage almost as high probably
exists among the negro population of America.
The General Pathology of Uveitis
Inflammations of the uveal tract may be divided into two pathological
types—acute purulent and sub-acute or chronic exudative inflammations.
In this section we shall discuss these in general terms, leaving those reactions
which show a specific and individualistic picture to be described in
subsequent sections.
Since the time of Meckel (1854) and Müller (1856) who made the first anatomical
examinations of panophthalmitis in cases of puerperal fever, of Virchow (1856) who
first demonstrated the presence of a capillary embolism, and later of v. Michel (1881)
who carried out the first experimental researches on inflammatory changes in the iris
after injecting silver nitrate solution into the anterior chamber of rabbits, a very large
amount of pathological work has been done upon the pathology of acute uveitis.
The most important clinical papers are by Ulrich (1882), Fuchs (1884–1925), Vossius
(1891), Collins (1892), Schirmer (1892), Herrnheiser (1893–94), Axenfeld (1894), Ginsberg
(1897–1920), Buchanan (1901–05), Kitamura (1908), Zade (1913), Raubitschek (1914),
Brückner (1919), Lindner (1920), Gilbert (1920–21), and Lamb (1938–39); while much
information can be derived from the experimental histological studies of Stoewer (1899),
Stock (1903), Selenkowsky and Woizechowsky (1903), Ulrich (1904), and Rados (1913).
Important work on experimentally produced cyclitis by the introduction of organisms
into the ciliary body was done by Straub (1912); and more recently a stimulus to
the study of aseptic exudative inflammations of the choroid has been provided by the
histological section of eyes which have been submitted to the various operative
procedures designed to replace a detached retina, whether by ignipuncture, chemical
cauterization, diathermy or electrolysis (Herzfeld, 1930; Amsler, 1930; Luntz, 1931;
Terry, 1932; Mayer, 1932; Stallard, 1933; Safar, 1933; v. Szily and Machemer,
1933–34; Campos, 1934; Cordero, 1934; Nakashima and Sakurai, 1934; and others).
DISEASES OF THE UVEAL TRACT 21.59
ACUTE PURULENT INFLAMMATIONS
Acute purulent inflammations, which are caused either by exogenous
infection, usually through a perforating wound,” or by endogenous infection
through the blood-stream by the lodgement of metastatic foci of virulent
pyogenic organisms,” tend usually to involve the entire globe. We have seen
that the uveal tract forms a closely inter-related vascular bed within the
confines of which inflammatory processes tend to spread apace ; moreover,
the immunological resistance in the chambers of the eye is extremely low,
and the circulation of the intra-ocular fluids aids the diffusion of toxins from
any localized lesion throughout the globe so that its complete involvement
is the usual sequel to the infection of any one part. The ultimate result,
therefore, tends to be the same wherever the initial lesion was located in
the uveal tract, or, for that matter, if the retina formed the primary focus,
as it frequently does in metastatic processes. Such a generalized purulent
inflammation of the inner eye is called PANOPHTHALMITIs ; but in less
virulent cases a walling off of the inflammation in the anterior or posterior
segment may determine a suppurative irido-cyclitis or choroiditis.
The first sign of inflammation is the vascular reaction—an intense
hyperaemia, a massive dilatation of the smaller vessels and their engorgement
with red corpuscles and polymorphonuclear leucocytes, the latter type of
cell escaping through the vessel walls in quantity and surrounding them as
by a cuff. In many cases of pyaemia, and also in Some cases of exogenous
infection, bacterial thrombi are seen in the vessels (v. Michel, 1881 ;
Wagenmann, 1887 ; Herrnheiser, 1892–93; Axenfeld, 1894–96 ; Goh,
1897), sometimes in considerable quantity, and occasionally in such quantity
and with so little perivascular reaction that their multiplication after death
must be assumed (Herrnheiser, 1893). Soon, however, the lumen of the
vessels becomes blocked with fibrin, pus cells, and masses of organisms,
so that the circulation is brought to a standstill ; while the vessel walls
become swollen and indefinite, and eventually appear homogeneous. At this
stage the surrounding stroma becomes packed with exudative cells—
erythrocytes, sometimes in such quantity as to constitute multiple haemor-
rhages, polymorphonuclear cells constituting the great mass of the infiltration
among which are seen less numbers of lymphocytes, mast cells, and
eosinophils, the latter, however, being more numerous in exogenous
inflammations (Fuchs, 1904).
In the meantime the stroma cells become swollen, their processes dis-
appear so that they become round, their nuclei stain poorly, and they
begin to necrose, or, alternatively, to wander out and mix with the exudative
cells (Brückner, 1919). The chromatophores also show similar disintegrative
changes; they become broken up, their pigment granules are scattered and
are found lying loose in the tissues or aggregated into clumps or engulfed
1 p. 2127. * p. 2136,
T.O.--WOL. III. F -
2160 TEXT-BOOK OF OPHTHALMOLOGY
in leucocytes. Partly owing to the cessation of the circulation following the
complete blockage of the vessels (Lindner, 1920), and partly as a direct result
of the liberation of massive doses of bacterial toxins, the entire tissue thus
becomes necrotic, until eventually no nuclei may take on a stain except the
invading army of leucocytes which pervades it throughout. These pack the
entire stroma, being especially massed in the perivascular spaces, and
eventually escape in quantity into the chambers of the eye along with a
fibrinous oedema-fluid.
This reaction occurs in all the uveal tissues with only incidental topo-
graphical differences. In the iris the whole structure becomes uniformly
thickened and necrotic, and from both aspects there pours out a fibrinous
Fig. 1794–METAst Atto ENDophth ALMITIs.
Angle of the anterior chamber packed with polymorphonuclear leucocytes,
following an infective embolism in the ciliary region (Parsons).
exudate rich in leucocytes (Fig. 1794). v. Michel (1881) found in his animal
experiments that this exudative coagulum caused an upheaval of the
endothelial layer on the surface, beneath which it gathered before the
disintegration of these cells allowed it to escape into the anterior
chamber. His observation was confirmed by Fuchs (1904), but it is probable,
largely owing to the incompleteness of this layer in man, that such an
occurrence is exceptional. Usually it exudes into the anterior chamber
directly, at first making the aqueous cloudy, and rapidly forming a
HYPopyon, the consistency of which depends on the proportion of fibrin
which encloses the leucocytes in its meshes (Figs. 1795, 1796). As a rule it is
white or yellow in colour and fluid in consistency, but if the proportion of
fibrin is high it may be grey, translucent and semi-solid (gelatinous exudate);
this may shrink as the fibrin contracts, so that in a few days it disappears,

DISEASES OF THE UVEAL TRACT 2161
leaving perhaps a thin pellicle attached to the pupillary margin. More
usually, however, the hypopyon grows, sometimes with remarkable rapidity,
filling the anterior chamber with semi-solid pus, and gumming the posterior
surface of the iris to the lens, so that in the most acute cases in which
tissue-necrosis is markedly extensive and complete, to define the boundary
between the hypopyon and the infiltrated iris may be difficult (Fig. 1797).
In the ciliary body the oedema and leucocytic infiltration rapidly
obscure the structure, although the ciliary muscle is usually less affected
than the rest of the tissues. The inflammation is most intense in the ciliary
processes and less so in the valleys between them (Fuchs, 1904), and the
Fig. 1795.-PANophth ALMITIs.
After a penetrating wound. Leucocytic infiltration of the cornea; anterior
chamber full of pus cells enmeshed in a fibrous network. The dark mass lying in
front of the iris on the right is a hyphaema. The lens and ciliary body bathed in
purulent exudate (x 7) (Parsons).
exudation appears at a very early stage particularly from the region of the
pars plana (Straub, 1896–1913; Brückner, 1919). In the first few hours a
fibrin-rich, albuminous fluid seeps through into the posterior chamber and
leucocytes begin to penetrate actively between the cells of the ciliary
epithelium (Fig. 1797). In intense inflammations this may occur so rapidly
that large vesicles form between the two layers of the epithelium, such as
occur after paracentesis (Greeff, 1894), or the entire epithelium may be raised
up in sheets, which eventually desquamate. This applies especially to the
pigmented layer, the cells of which break up, releasing their pigment
granules which are absorbed by the leucocytes, thus leaving the ciliary body
in extreme cases almost devoid of pigment as though it had been bleached.
In the meantime the exudate pours into the posterior chamber filling it with

E 2
216.2 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 1796.-PANoph THALMITIs.
Purulent irido-cyclitis showing exudate in anterior and posterior chamber
( x 10) (Parsons).
Fig. 1797. PURuLENT IRIDo-cyclºrºs.
Panophthalmitis after perforating wound. Pus and blood in the anterior
chamber. Intense congestion and infiltration of the iris, which is necrotic on the
left, and of the ciliary body on the right below (x 35) (Parsons).


DISEASES OF THE UWEAL TRACT 2163
a fibrinous coagulum rich in cells, spills forwards into the anterior chamber
to add to the hypopyon, insinuates itself between the zonular fibres and
may even cover the lens, and spreads backwards into the vitreous body
round the fibrils of which the pus cells cling in histological sections making
a beautiful picture as they radiate out fan-wise from the ciliary region.
In the choroid the same reaction occurs, first round the medium sized
and smaller vessels of the inner layers, and last in the layer of larger vessels
and the supra-choroidea. Eventually, however, the whole of the stroma
becomes packed with cells, and the hyperaemia, oedema, and infiltration
swell the tissue until it may become seven or eight times its normal
Fig. 1798.-PANophth ALMITIs.
Congestion and infiltration of the choroid and retina with perivascular infiltra-
tion of the latter (x 60) (Parsons).
thickness (Fig. 1798). If the infection is an exogenous one starting as an
abscess in the vitreous, or an endogenous one localized initially in the retina
(the usual site), the choroid remains immune for a long time, being attacked
primarily in two places, anteriorly near the pars plana, and posteriorly in the
region of the optic nerve head, whence a spread of the infection to the
remainder of the tissue may be comparatively slow. Similarly in metastatic
inflammations starting in the choroid, the membrane of Bruch holds out for
a considerable period, and, unlike its tenuous prolongation over the ciliary
body and iris, it prevents a spread of the exudation into the chambers
of the eye; the fluid is thus forced externally and distends the supra-
choroidal space, forcing the laminae apart, sometimes forming cystic spaces
among them, and at other times raising the choroid bodily up from the

2164 TEXT-BOOK OF OPHTHALMOLOGY
sclera. Eventually, however, fluid loaded with toxins filters through the
membrane of Bruch and collects underneath the retina, detaching it in
places and inhibiting its function. In the meantime marked changes are
occurring in the pigmentary epithelium ; the cells become rounded, they
lose their pigment and become cast off, the freed pigment being engulfed by
leucocytes. Finally, in severe cases Bruch's membrane begins to dis-
integrate, leucocytes stream through breaches in its continuity, the purulent
infiltration pervades the retina and destroys it, and passes thence into the
vitreous body (Fig. 1799).
Fig. 1799.-PANophth ALMITIs.
Exudates in the vitreous (x 60) (Parsons).
Of all localities the most intense infiltration is seen at the channels of
exit. The spaces round the perforating vessels become choked with pus
cells, the angle of the anterior chamber becomes similarly infiltrated, while
the perivascular infiltration spreads down the central vessels into the
optic nerve and exudate may accumulate in its meningeal sheath. Occa-
sionally in those cases there is some danger of the development of meningitis
or of cavernous sinus thrombosis. Anteriorly, the massive doses of toxins
diffusing through the cornea may cause necrosis of its central tissues, and in
an attempt to isolate and remove the resultant sequestrum, a ring infiltra-
tion of this tissue appears which ulcerates, usually with rapid perforation
of the cornea" (Collins, 1893; Fuchs, 1903; Attias, 1914; Flieringa,
1921; Gifford and Hunt, 1929; and others). The lens, too, is similarly
affected. If its capsule is intact, the inward diffusion of toxins causes
* Ring Abscess of the Cornea, Vol. II, p. 1990.

DISEASES OF THE UWEAL TRACT 21.65
death and autolysis of its cells and the formation of cataract; but if in
traumatic cases the capsule is ruptured, the excellent culture medium which
its substance provides for the growth of any type of organism rapidly
induces the formation of an abscess.
In acute cases, therefore, the entire eye becomes involved, and once the
infection becomes established in its interior, the lack of immunological
defence too frequently results in its structure being destroyed beyond
recognition. The eventual result in the most severe types is perforation
Fig. 1800.-METASTATIC ENDoPHTHALMITIs.
Meningococcal. A. Vitreous filled with pus. B. Dilatation of the choroidal
vessels. Perforation through choroid and sclera (C) to form an abscess beneath the
tendon of the internal rectus (D). EE". Anterior chamber with purulent exudate.
The ring abscess of the cornea has perforated (Mayou : R. L. O. H. Rep.).
of the globe with evacuation of its purulent contents (Fig. 1800). Quite
frequently, however, the infection becomes walled off and to some extent
localized, the entire uveal tract participating in the inflammatory reaction,
but the abscess-formation being limited to the anterior or the posterior
segment.
Exceptionally, particularly in staphylococcal infections, an isolated abscess may
occur, as in the iris (Raubitschek, 1914; Horner and Cordes, 1931), or multiple abscesses
may appear (Heine, 1923; Mylius, 1926). Also, exceptionally, isolated lesions may
occur in the posterior segment. Most of those cases of metastatic ophthalmia which
have been seen ophthalmoscopically and subsequently examined histologically were
localized in the retina or optic nerve (Goh, 1897; Grunert, 1902; v. Michel, 1902;
Spicer, 1907) : only a very few started as a focus of choroiditis–Bull (1901) in a
case of pneumonia, Hanke (1921) in a case due to furuncles on the nose and lip, and
Higuchi (1934) in a case due to a furuncle on the temple.

2166 TEXT-BOOK OF OPHTHALMOLOGY
In such cases wherein a generalized purulent reaction does not occur,
characteristics of chronic inflammation frequently exist coincidently with the
erudative reaction typical of the acute form. Presumably we are dealing with
an irritant sufficiently virulent to excite a polymorphonuclear infiltration at
the site at which it is most concentrated, but, as the toxins diffuse to other
parts, their diminished effectivity is sufficient only to give rise to the mono-
nuclear reaction characteristic of the more chronic forms of inflammation.
We may thus find the simultaneous presence of acute and chronic inflamma-
tory cells from the beginning of the process, and as time goes on the latter
gradually replace the former as the natural defences of the tissues bring the
infection under control and
healing becomes established. The
type of response thus depends
upon the intensity rather than
the nature of the infection.
The simultaneous occurrence
of the two types of inflammation,
acute and chronic, was originally
established by Fuchs (1904) and
Ruge (1904) in suppurating in-
flammations after exogenous
infection, the most typical instance
being the presence of an acute
purulent abscess in the vitreous
together with a chronic infiltration
of the ciliary body. Again, in a
suppurative retinitis which pours
Fig. 1801–Septic Expophthalmitis. out polymorphonuclear cells in
º, º quantity, the choroid may be
ciliary body (x 450) (Lamb, Am. J. O.). infiltrated with lymphocytes; or
the ciliary body itself may dis-
charge numerous pus cells into the anterior part of the vitreous chamber,
while it is itself packed almost entirely with small round cells; or dense
numbers of pus cells may infiltrate the inner layers of the choroid and at
the same time the outer layers may be occupied by an equally dense infiltra-
tion of mononuclear cells (Ginsberg, 1921; Lamb, 1938–39) (Fig. 1801). The
exudative cells thus not only come from the blood, but are partly derived
from the tissues, the iris stroma, the ciliary body, and even the corneal
endothelium (Brückner, 1919; Gilbert and Plant, 1921; Wolf, 1921
and others).
Fuchs (1913) considered that the infiltration of the uvea with round cells in these
cases predicated a beginning of sympathetic ophthalmitis, but the nature of the
conditions in which the association of the two types of infiltration appears negatives
this view. We shall see presently that in all inflammations, no matter how acute, if

DISEASES OF THE UVEAL TRACT 2167
the eye survives, the chronic factor intervenes and intensifies as the original virulence
of the aetiological agent weakens : it would seem also that the same agent from the com-
mencement may cause the appearance of acute inflammatory cells in its immediate neigh-
bourhood, and of chronic cells further afield. It is to be noted that the exudative cell
in both types is always phagocytic, since this is the only one capable of long migration
from its parent tissue. The difference is essentially one of origin in response to a call
which must be answered immediately or at leisure: the polymorphonuclear cell, or
microphage, characteristic of the acute phase, comes solely from the blood, while the
mononuclear cell, or macrophage, characteristic of the chronic reaction, is a derivative
essentially of the reticulo-endothelial system.”
If the eye survives the acute fulminating purulent process, a sub-acute
stage follows when the usual histological picture of the subsidence of a
purulent inflammation develops. In general terms this involves a gradual
retirement of the polymorphonuclear cells, their replacement by mononuclears,
Fig. 1802.-PHTHISIs BULBI.
After panophthalmitis. Total posterior synechia: anterior capsular cataract;
detachment of ciliary body; detachment of retina; massive sub-retinal coagulum :
grooves in sclera owing to pressure of recti (x 3) (Parsons).
the development of neo-vascularization and granulation tissue which is eventually
changed to fibrous tissue, and the ultimate shrinkage and degeneration of the
resultant scar with more or less complete disorganization of the globe (Fig. 1802).
As a rule this process involves the whole globe, but in those cases
where some localization is maintained to the anterior or posterior segment,
the affected part may become almost completely converted into scar-tissue
leaving the relatively free segment with its structures comparatively intact.
1 Vol. II, p. 1450.

2168 TEXT-BOOK OF OPHTHALMIOLOGY
Thus in an exogenous infection of the anterior segment, the anterior chamber
may be abolished and the cornea, iris and lens be fused into one indis-
criminate staphylomatous mass, leaving the posterior segment anatomically
intact but with the retinal function impaired. And again, a purulent process
in the vitreous cavity may organize to form a pseudo-glioma, leaving merely
synechiae to mark the associated inflammation of the anterior uvea. The
usual end-result of the first type of inflammation is the obliteration of the
anterior chamber and the development of glaucoma, of the second, the
formation of a pseudo-glioma and shrinkage of the globe.
In the anterior segment of the weal tract, the fibro-cellular exudate
rapidly glues theiris to the anterior surface of the lens. At first the adhesion
Fig. 1803–IRIs Boniº - SECLusto PUPILLE.
Extensive peripheral anterior synechia. Posterior synechia with seclusio
pupille; inflammatory pupillary membrane with occlusio pupillae; anterior capsular
cataract (Parsons).
has a homogeneous embryonic structure, but eventually organization and
fibrosis permanently bind the two tissues together by the formation of
PostERIOR syNECHLE (ovuéxew : to hold together) (Fig. 1804). The whole
circle of the pupil may be thus bound down forming an ANNULAR (RING)
SYNECHLA, completely shutting off the anterior from the posterior chamber
(SECLUSIO PUPILLE). This condition in itself is full of danger, for the
stoppage of the circulation of the intra-ocular fluid and its banking up in
the posterior chamber at first bellies the iris forwards like a sail, pro-
ducing an IRIs BoMBE (Fig. 1803), and then leads to the development of a
secondary glaucoma. The adherence of the iris to the lens may be more
complete, and becoming opposed to and united with it over its entire
extent, a total Posterior synºchta results; while the exudate filling
the posterior chamber may similarly organize, obliterating it, and, forming
an inflammatory pupillary membrane, may cover the entirelens to constitute

DISEASES OF THE UVEAL TRACT 21.69
Fig. 1804.—CHRoNIC IRIDo-cyclitis.
Total posterior synechia ; occlusio pupillae; anterior capsular cataract;
detachment of ciliary body and retina (Parsons).
the condition of occLUsio PUPILLAE (Fig. 1804). Finally, the swelling of the
tissues at the root of the iris and the anterior part of the ciliary body may
Fig. 1805.-IRIDo-CycLITIS.
Showing retraction of the iris at the periphery. C, the cornea (cut across) showing
peripheral anterior synechia. I, degenerated iris showing a fibrous membrane on its
anterior surface and ectropion of the pigment layer. The posterior chamber is
filled with almost non-cellular fibrous tissue into which the ciliary processes are
dragged. V, coagulum behind the detached atrophic retina (x 25) (Parsons).
make this region of the uvea approach the cornea, so that the angle of the
anterior chamber is bridged across by strands which eventually consolidate to
form ANTERIOR PERIPHERAL synECHLE (Junius, 1919) (Fig. 1805); by thus


2170 TEXT-BOOK OF OPHTHALMOLOGY
obliterating the angle of the anterior chamber and further impairing the
circulation of the intra-ocular fluid, this may add considerably to the danger
of a secondary rise in tension.
Meantime, in the anterior chamber itself proliferating capillaries
penetrate into the fibrin-rich exudate, and in their wake spindle-shaped
fibroblasts follow, converting not only the iris stroma into scar-tissue, but
also laying down new fibrous tissue upon its surface. In this way layers of
fibrous tissue are formed in the anterior chamber, sometimes in enormous
amount (Figs. 1805, 1806); and in exogenous infections where perforation
Fig. 1806–IRIno-ovoitºrs.
From a micro-cephalic idiot. C. Posterior lamellae of cornea. I. Inflamed and
degenerated iris. F. Laminated fibrous tissue as thick as the cornea nearly filling
the anterior chamber.
has occurred, the new fibrous tissue may, as we have seen,” bind the cornea
and the iris, and sometimes the lens together in one conglomerate scar to
form a staphyloma throughout the interstices of which the pigmentary cells
of the uvea proliferate and ramify,
In the ciliary region a cicatricial organization of the exudate occurs in a
similar manner; the polymorphonuclear cells are replaced by mononuclears,
and granulation tissue appears with the ultimate development of a mass of
fibrous scar-tissue constituting a Pseudo-GLIoMA. We have seen that the
most active exudate is formed from the pars plana of this region, whence it
spreads fan-wise into the anterior vitreous; here, as it organizes it forms a
cycliſtic MEMBRANE behind the lens (Fig. 1807). Such a membrane is
* Vol. II., p. 1819.

DISEASES OF THE UVEAL TRACT 2171
Fig. 1807.-Acute CycLITIs.
Cyclitis of fourteen days' duration showing exudate lying upon the ciliary
body, retina and lens capsule (Buchanan, T. O. S.).
Fig. 1808.-CYCLITIs. Fig. 1809.-CycLITIs.
A chronically inflamed ciliary body Above, the unpigmented ciliary epithe-
showing macrophages emigrating through lium ; below, macrophages throwing out
unpigmented ciliary epithelium (Lamb. processes preliminary to becoming fibro-
Am. J. O.). blasts (Lamb, Am. J. O.).
initially developed from macrophages which emigrate through the ciliary
epithelium (Fig. 1808), become transformed into fibroblasts or connective
tissue cells with long branching processes (Fig. 1809), and ultimately
consolidate into fibrous tissue (Fig. 1810) (Lamb, 1938). It is to be noted
that the cells of the ciliary epithelium do not participate in the process, but


2172 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 1810.-CycLITIs.
Newly formed fibroblasts in profile infiltrated
with a few macrophages. Some of the latter in
the group below and to the right have taken up
particles of melanin (Lamb, Am. J. O.).
they show a marked prolifera-
tive activity, particularly the
cells of the pars plana, so that
long branching epithelial
tubules grow into the scar-
tissue, giving the appearance of
proliferating glands (Collins,
1896), (Figs. 1812, 1813). Con-
traction of this scar-tissue in
the vitreous cavity soon
detaches the retina to which it
is adherent, and the eventual
appearance is usually that of
a pyramid-shaped fibrous
mass, its base anteriorly
stretched across the globe
behind the lens, and its apex
adhering posteriorly to the optic
dise (Fig. 1811). The fibrous tissue is at first loose and cellular, perhaps
interspersed with hyaline masses, but eventually it becomes dense and
Fig. 1811–PANopatrial mitis.
Pusand exudate in anterior chamber; inflammatory anterior capsular cataract;
eyelitic membrane behind lens; complete detachment and folding of the retina.
From a girl of six (x 8) (Parsons).


DISEASES OF THE UVEAL TRACT 2173
Fig. 1812.-TUBULEs IN CycLITIs.
Cyclitis of 20 years’ duration; to the right the sclerotic, to the left of which
non-pigmented tubules are seen growing from the pars plana into a mass of
post-cyclitic fibrous tissue. On the left this fibrous tissue is more cellular and has
undergone fatty degeneration (x 55) (Parsons).
FIG. 1813.−TUBULES IN CYCLITIs.
Cyclitis of 35 years' duration. Pigmented tubules in dense post-cyclitic fibrous
tissue (x 60) (Parsons).


2174 TEXT-BOOK OF OPHTHALMOLOGY
T
|-
-
º - º
-
- -
º - º
-
º -- -
is -
N - - __- -
- -
- - -
-
- - - - - - -
- --- - - - - - -
Fig. 1814.-FIBRosis of THE Choroid.
From an eye blind for 30 years. Almost complete fibrous degeneration of the
retina with detachment. A thick layer of hyaline fibrous tissue on the surface of the
choroid which is degenerated to form the pigmented line only (Parsons).
fibrillar (Fig. 1813), and in the process of consolidation the ciliary processes
are drawn inwards, so that the entire ciliary body may become detached
from the sclera except at the insertion of the ciliary muscle (Fig. 1802).
Fig. 1815.-Phthisis Bulb with Box.E IN Choroid.
From a man of 28 who had acute cyclitis following measles at the age of 5.
Note dome-shaped cornea resembling a buphthalmos, retracted iris, calcareous
lens, eyelitic membrane, detached retina and bone in choroid showing true medul-
lary spaces (x 3) (Parsons).

DISEASES OF THE UVEAL TRACT 2175
At the same time the choroid becomes metamorphosed by a similar
process into fibrous tissue which proliferates inwards to involve the retina
and eventually to invade the vitreous cavity (Fig. 1814). On its contrac-
tion this tissue distorts and detaches the retina; beneath it the pigment
epithelium proliferates and secretes colloid bodies on Bruch's membrane
and eventually the peri-choroidal space may become traversed and finally
obliterated by scar-tissue (Fuchs, 1904). During this stage, owing to the
destruction of the ciliary body and the other vascular regions of the eye, the
formation of intra-ocular fluid becomes disorganized, the tension falls,
Fig. 1816–FATTY DEGENERATIon IN CycLITIs.
Cyclitic exudate of 24 months duration showing fatty degeneration (x 100)
(Buchanan, T. O. S.).
general atrophy sets in, and the globe shrinks to develop the condition of
PHTHISIs BULBI (Fig. 1815).
Subsequently degenerative changes occur in the cicatricial tissue—fatty
degeneration (Buchanan, 1901; Jaensch, 1926) (Fig. 1816), the deposition of
sheaves of cholesterin crystals (Cramer and Schultze, 1894), the formation of
cartilage (Sgrosso, 1890), and the deposition of lime salts first as fine granules
and concretions (Fig. 1817), and eventually as true organized bone (Fig. 1818).
Bone-formation occurs with great frequency in the choroid,” less readily
in the ciliary body, only exceptionally in the iris; in the exudative deposits
it is somewhat rare. Here it commences as a granular deposit around a
growing blood-vessel, among which radiate osteogenic fibres and stellate
1 p. 2747. * p. 2423.
T.O.-WOL. III. G

2176 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 1817.-CALCAREous Degeneration is cycliſtis.
Cyclitic exudate after 11 years showing calcareous particles (Buchanan T.o.S.).
Fig. 1818-Bose-roºmation is Cyclitus.
Cyclitic exudate after 13 years showing fully-formed bone including cavity
lined with osteoblasts (Buchanan, T. O.S.).


DISEASES OF THE UVEAL TRACT 2177
Osteoblastic cells. The granules coalesce and become arranged into a some-
what homogeneous structure striated concentrically around the cavity in
which the central blood-vessel lies. A regular lamination then appears
centrally, but the growing edge remains ill-defined, being marked by fibres
radiating out into the neighbouring fibrous tissue around which the cells,
surrounded by a calcareous deposit, arrange themselves in columns.
Meantime the lens may be completely embedded in fibrous tissue.
Degenerative changes within its fibres lead to opacity and shrinkage,
while the capsule becomes folded in waves. Finally, the lens may become
calcareous (Fig. 1815); but so long as the capsule remains intact, it forms
an impassable barrier to Osteoblasts and true bone-formation does not occur.
SUB-ACUTE AND CHRONIC UVEITIS
Cases of SUB-ACUTE OR CHRONIC ENDOPHTHALMITIS are much more
common than those of acute panophthalmitis which we have already
considered, and they embrace a whole host of conditions of exceedingly varied
aetiology. Into this category come the common cases of iritis, irido-cyclitis,
and choroiditis which are designated clinically as “acute,” for these, despite
their sudden clinical onset and acute symptoms, have the histological character-
istics of sub-acute inflammatory processes. Into the same class also come
cases of exogenous infection of a non-purulent nature caused by mild
infection and irritation, the aetiology of which we have discussed under the
heading of traumatic iritis,” and also such metastatic conditions as do not
give rise to an acutely purulent response. Whatever their aetiology the same
general pathological picture is presented, quite different from the intense and
destructive reaction characteristic of acute purulent inflammations. The
destruction of tissue is limited and the lesion frequently remains focal, the
eacudation is less, and the invading cells are fewer, being represented essentially by
mononuclear cells of all types. Such an inflammation may heal completely
leaving a small and unimportant scar at the site of the focal lesion, or residual
tell-tale aggregations of lymphocytes, mast cells, or histiocytes to mark the
site and extent of previous lesions which had occurred years previously
(Fuchs, 1925; Zimmermann, 1925). On the other hand, in chronic or
recurrent cases, a generalized endophthalmitis of considerable severity
develops, which leaves sequelae indistinguishable from the wreck which
follows acute panophthalmitis—destruction of the drainage mechanism in
the anterior segment with the development of glaucoma, or the formation
of a cyclitic membrane in the posterior segment with subsequent shrinkage
of the globe. Indeed, this type of inflammation is sometimes worse than the
first in its final result, for the process may remain active indefinitely and
there is frequently a tendency for the development of recurrent attacks of
1 p. 2135.
21.78 TEXT-BOOK OF OPHTHALMOLOGY
a sub-acute nature, involving repeated painful illnesses and ultimately
necessitating excision of the eye-ball. -
The characteristics of such sub-acute or chronic inflammations are
essentially the same whatever their site. These may be summarized under
three heads: changes in the affected tissue, the reaction of the pigmentary
epithelium, and the formation of exudate.
The changes in the tissue involve essentially a vascular hyperaemia
followed by a cellular infiltration, which is sometimes diffuse but more
typically focal and nodular. Polymorphonuclear cells, the feature of acute
inflammations, are exceptional; lymphocytes are common but do not as a
Fig. 1819.-INFLAMMATony Punctate Deposits.
The ciliary body covered with swollen leucocytes undergoing hyaline
degeneration. There were massive keratic precipitates (x. 55) (Parsons).
rule predominate; plasma cells are also numerous, occurring on occasion in
enormous numbers; but the characteristic cells are the large mononuclear
phagocytes (histiocytes)—maerophages, clasmatocytes, epithelioid cells—
sometimes unusually large, frequently agglutinating into clumps, and
occasionally fusing together to form giant cells. The infiltration may
be followed by focal necrosis in its densest parts; but the usual result is the
formation of granulation tissue and its subsequent organization and
consolidation into scar-tissue.
The pigmentary epithelium which lines the inner aspect of the iris, ciliary
body and choroid reacts peculiarly and uniformly in all regions by a dual
process of degeneration and proliferation, in much the same manner as we
have seen it to reactin vascular lesions in the posterior segment (Wagenmann,
1890; Capauner, 1893; Krückmann, 1899; Fuchs, 1904–20; Leber, 1915).

DISEASES OF THE UVEAL TRACT 217.9
The degenerative changes involve an oedematous swelling of the cells
followed by their breaking loose and disintegration with a scattering of their
pigment which is phagocyted. The proliferation in other areas, particu-
larly the areas surrounding focal lesions, may be excessive in degree and
seems to be aimless in type, epithelial nodules being formed on the posterior
surface of the iris, long tubular prolongations growing exuberantly from the
ciliary region, and large masses being formed in the posterior segment
invading the retina and filling up all the available spaces.
The remaining characteristic is the easudation thrown off from a lesion in
any locality, but always most pronounced when the ciliary body is involved
(Fig. 1819). In the more chronic types it may be sparsein quantity, limiting
itself to microscopical dimensions on the surface of the inflamed area, and
Fig. 1820–KERATIC PRECIPITATEs (Straub, T. O. S.).
appearing as cellular precipitates on the posterior corneal surface and on the
iris, or as fine dust-like opacities in the vitreous. At other times it is more
abundant and rich in fibrin, determining adhesions which become consoli-
dated, binding the iris to the lens, obliterating the posterior chamber, or
resulting in the formation of a pseudo-glioma.
In the first case the aqueous humour becomes turbid, and cellular
elements escaping not only from the ciliary body but also from the iris and
the choroid are seen floating in its current, to be deposited eventually on the
back of the cornea as KERATIC PRECIPITATES (Fig. 1820), partly because they
are thrown there by the centrifugal force of the convection currents in the
anterior chamber, and partly because they adhere to the swollen and
oedematous endothelium.
The various forms and the fate of these deposits have already been fully
described and figured"; it will be sufficient to mention here that the deposition
is preceded by cedematous changes in the endothelium (Mayou, 1924; Vogt, 1930),
* Vol. II, p. 1864.

2180 TEXT-BOOK OF OPHTHALMOLOGY
and that they vary in size from minute and almost invisible collections of cells to
plastic masses covering large areas of the cornea, destroying the endothelium and
eventually inducing a seleral-like opacity in the substantia propria. The histological
appearances of these precipitates, first described by Schweigger (1873), de Wecker
(1876) and Knies (1880), were elaborately detailed by Fuchs (1884), whose conclusions
subsequent observers have essentially confirmed (Ridley, 1895; Groenouw, 1900;
Baas, 1903; Harms, 1904; Bartels, 1905; Straub, 1913; Mayou, 1924; and others).
Polymorphonuclear cells rarely occur in these deposits, and lymphocytes seldom
form precipitates large enough to be seen by the naked eye; in the usual case the
preponderating cells are plasma cells and large mononuclear phagocytes. These
cells readily agglutinate and adhere to the endothelium to form the typical yellowish
waxy deposit known as “mutton-fat k. p.”
It is important to remember that the first changes occur in the corneal endothelium
which, like all endothelial tissues, is extremely sensitive to toxins. At first the cells
become slightly swollen, the cement substance between them becomes loosened, and
they frequently become detached (Fig. 1821). They do not disintegrate, but on the
B. D. C. E. A.
Fig. 1821–ENDoTHELIAL CHANGEs IN CycLIris.
A. Swollen endothelial cells. B. Loosened endothelial cells becoming phago-
cytic. C. Denuded area on Descemet's membrane. D. Lymphocyte. E. Plasma
cell (Mayou, T. O. S.).
other hand often proliferate and become actually phagocytic, taking up particles
of pigment and broken up leucocytes. If this roughening of the endothelium is not
extensive, the high specific gravity of the albuminous fluid and the convection
currents in the anterior chamber tend to make the deposition of precipitates assume
the typical triangular distribution described by Ehrlich (1882) and Turk (1906); but
more usually localized roughening and destruction determines a more irregular dis-
tribution; and in extreme cases when the toxin is sufficiently powerful to destroy
large areas of the endothelium, the exudate may subsequently organize into a mass
of permanent fibrous tissue covering a correspondingly large area of the back of the
corneº-
It is to be remembered that while they are most readily apparent
clinically on the posterior surface of the cornea such deposits are seen on the
anterior surface of the iris, on the pupillary border, as well as on the ciliary
epithelium. They are most common at the pupillary border (Gilbert,
1914; Koeppe, 1917), when they are usually known as Koºppe's NoDULEs.
Much more rarely they are seen on the capsule of the lens (Spicer, 1924), on
tags of the pigment layer adherent to the lens after an iridectomy (Koby,
1930), or on isolated remnants of the pupillary membrane (Meesmann, 1927).

DISEASES OF THE UVEAL TRACT 2181
Pathologically these nodules differ from tubercles in that they consist of accumu-
lations of epithelioid cells and lymphocytes which have been deposited without in-
volving the loss of tissue. They have been studied histologically by Vogt (1923),
Derby (1928), and Busacca (1932), and may be divided into two types—those appearing
on the ectodermal layers of the iris at the pupillary border (the NoDULEs of Koeppe)
ULE Fig. 1823.−MEsoper MAL
on IRIs (Busacca, K. M. Floccule on IRIs
Aug.). (Busacca, K. M. Aug.).
(Fig. 1822), and those appearing on the anterior mesodermal layers of the iris (the
FLoccules of BusAccA) (Fig. 1823). The former are of some permanence, but the
latter are usually transient, although occasionally they may become conglomerate and
vascularized. They are composed of masses of cells deposited from the aqueous and
in structure somewhat resemble a phlycten : this, as well as the fact that they contain
no organisms (Vogt, 1923), and can be produced by the injection of dead bacilli (Derby,
1928) suggests that they may have an allergic origin.
Fig. 1824.—PUNCTATE DEPosits on THE RETINA (Lister, T. O. S.).


2182 TEXT-BOOK OF OPHTHALMOLOGY
Similarly, particularly in post-traumatic infections, exudative deposits
may also occur on the retina, clumps of white cells bound together by fibrin
lying upon its surface, sometimes forming microscopical precipitates, but at
others appearing as accumulations of enormous dimensions (Elliot, 1917;
Lister, 1921; Samuels, 1936) (Fig. 1824); while in almost all cases fine
cellular opacities crowd the vitreous (Fig. 1835).
In the iris the mononuclear infiltration is usually intense throughout the
tissue, most especially around the blood-vessels which are surrounded by a
Fig. 1825–AouTE PLAstro IRITIs.
A superficial, delicate network of fibrin on the anterior surface of the iris seen
faintly in the figure. The blood-vessels are congested and the stroma densely
infiltrated particularly in the anterior layers (x 120) (Parsons).
cuff of cells: the region of the muscles and the richly vascular pupillary
border are frequently particularly involved (Fig. 1825). The vessel walls
themselves show changes of a hyaline nature, and frequently the smaller
ones become completely blocked by endothelial proliferation. To this may
be added a proliferation of the muscularis, an infiltration of the media, and
a thickening of the external elastic lamina (Meller, 1935). If the lesion is
focal there is considerable oedema and distortion of the tissues in the affected
area caused by aggregations of round cells—a NoDULAR IRITIs (Fig. 1826).
In the central area of such a nodule necrosis may develop which is eventually
healed by the formation of scar tissue. In addition, some exudate
escapes from the surfaces, which, when it is fibrinous, determines on the
posterior aspect the formation of posterior synechiae, with, perhaps, subse-

DISEASES OF THE UVEAL TRACT 21.83
Fig. 1826.-NoDULAR IRITIs.
Chronic irido-cyclitis following influenza with a nodule of lymphocytes near
the pupillary margin and inflammatory pupillary membrane (x 60) (Parsons).
Fig. 1827-FIBRous Tissue on IRIs : Choi EstERol. IN THE ANTERIOR
CHAMBER.
Irido-cyclitis of 5 years duration after an injury. The anterior chamber is
full of a fibrous coagulum containing leucocytes, pigment cells, free pigment granules,
calcareous granules and cholesterol. Note the new fibrous tissue on the surface of
the iris and the hyaline membrane which appears to be continuous with Descemet's
membrane covering this tissue (x 55) (Parsons).


218.4 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 1828–INFLAMED Aſtrophic IRIs.
From a blind painful eye showing a false angle and hyperplasia of the endo-
thelium on the surface of the iris (x. 60) (Parsons).
Fig. 1829–HYALINE MEMBrase on IRIs.
Old anterior synechia. Descemet's membrane appears to split, a hyaline layer
passing over the surface of the iris covered by a single stratum of endothelium
(x 120) (Parsons).


DISEASES OF THE UVEAL TRACT 21.85
quent seclusion and occlusion of the pupil, and on the anterior surface may
eventually lead to the formation of fibrous tissue (Fig. 1827). Sometimes the
endothelium proliferates into several layers, which may remain cellular
(Fig. 1828); but more frequently hyaline changes take place, so that the
anterior surface of the iris may become overlaid by a hyaline layer covered,
perhaps, by a layer of endothelial cells and apparently continuous with
Descemet's membrane (Wagenmann, 1888–92; Axenfeld, 1902; Werncke,
1903; Halben, 1904; and others) (Fig. 1829).
On the posterior surface the pigment epithelium undergoes simul-
taneously the opposite processes of disintegration and proliferation which we
have just discussed, the cells in places breaking up and losing their pigment,
and at other places proliferating to form several layers or misshapen clumps
and masses, occasionally with the formation of cystic spaces between the
two layers. Meantime, the exudative adhesions to the lens also organize,
forming permanent posterior synechiae, which consolidate to such a degree
that, if they are forcibly broken down, the posterior layer of the pigment
epithelium remains attached to the lens while the anterior layer is torn
away from it, the former gradually atrophying but leaving permanent traces
of pigment upon the lens capsule. The final result may be a more or less
complete atrophy of the iris accompanied by endarteritis of the small vessels,
and the replacement to a large extent by fibrous tissue of all its elements
except the exuberant pigment epithelium, which, partly owing to the
mechanical effect of a shrinkage of the contracting scar tissue (Knies, 1876;
Birnbacher and Czermak, 1886), partly owing to the atrophy of the stroma
(Fuchs, 1883–1920), but probably largely as a result of its own overgrowth
(Gallenga, 1905; Lohmann, 1910; Siegrist, 1912) may spread round the
pupillary margin and extend for some considerable distance down the
anterior surface to form an ECTROPION OF THE PIGMENT LAYER 1 (Fig. 1805).
In the more pronounced cases, indeed, the pigment layer may eventually cover
almost the entire anterior surface of the iris when it is unhindered by the presence of
posterior synechiae (Stern, 1914). In extreme cases of atrophy, dehiscences may occur
in the iris stroma, so that actual holes are formed (Oblath, 1899), an occurrence usually
preceded by the presence of synechiae and a rise of pressure in the posterior chamber
(Hess, 1892; Franck, 1903; Harms, 1903; de la Vega, 1923). As a rule such holes
are formed in the thinner part of the iris near its ciliary attachment, so that a mom-
traumatic atrophic irido-dialysis results.
An alternative end-result is for the connective tissue on the anterior surface of
the iris to grow right over the pupillary aperture. Sometimes such a membrane
contains new-formed vessels developed from the iris stroma, and if these are in large
numbers and attain considerable size, they may give the impression of an actual
vascularization of the lens capsule itself (Bedell, 1922–24 ; Munson, 1934).
In the ciliary body the histology of the milder cases of inflammation is
less fully known than in the case of the iris or choroid, for the first, and some-
* Since the retinal layers of the iris are involved, the common term ectropion wrece is a
misnomer.
2186
TEXT-BOOK OF OPHTHALMOLOGY
Fig. 1830.-Cyclitus.
Bleached section in cyclitis of 16 days’ duration (Buchanan, T. O. S.).
Fig. 1831.-CycLITIs.
Cyclitis of 22 days duration showing large epithelioid cells between the retina
and the vitreous (x 120) (Buchanan, T. O. S.).


DISEASES OF THE UVEAL TRACT 218.7
times the only indication of its presence is the appearance of exudative cells
in the anterior chamber or in the vitreous body. A sub-acute or chronic
cyclitis is, however, accompanied by the same general tissue-changes. The
ciliary processes become swollen, sometimes sufficiently to touch the lens
(Fuchs, 1884), the swelling being due to oedema and mononuclear infiltra-
tion; as in the iris, the infiltration is essentially perivascular or aggregated
into nodular masses, especially towards the posterior part of the ciliary
region between the ciliary muscle and the epithelium. The epithelium itself
undergoes degenerative changes in some places, and at others proliferates
into long tubular branching processes, which in their earlier stages may be
Fig. 1832. –CYCLITIs.
Fibrinous exudate in cyclitis of 18 days duration (x 100) (Buchanan, T. O. S.).
unpigmented (Alt, 1877) (Fig. 1830). Between the epithelial cells the exuda-
tive cells pass forming masses lying on the surface, especially in the depres-
sions between the processes, and eventually travel into the posterior and
anterior chambers and the vitreous body (Fig. 1831). This exudation
forms the characteristic clinical indication of a cyclitis, and, depending on
whether it is relatively fluid and cellular on the one hand, or rich in fibrin and
coagulable on the other, the condition is termed SERous CYCLITIs (the old
“serous iritis '') or PLA stic cyclitis. In the serous type, as we have seen,
the exudates are limited to the deposition of keratic precipitates on the cornea,
similar depositions on the iris and sometimes on the lens capsule, and the
appearance of a fine dust-like cloud in the vitreous body. In the plastic type
of cyclitis the exudate is copious, and being rich in fibrin, it accumulates
in the posterior chamber, and, encircling the lens, it forms a cyclitic

218.8 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 1833. CycLITIs.
Fibrous tissue in the exudate from the parseiliaris retinae in cyclitis of 60 days’
duration (x 200) (Buchanan, T. O. S.).
Fig. 1834.-CycLºris.
Cyclitis of many years' duration. Corneo-scleral junction above and to right;
lens above and to left ; below the lens a mass of fibrous tissue with the ciliary body
to the right and below this a detached and folded retina (x 14) (Buchanan, T.O.S.).


DISEASES OF THE UVEAL TRACT 21.89
membrane in the anterior region of the vitreous cavity (Figs. 1832–3). This
becomes organized and eventually consolidates into fibrous tissue, consti-
tuting a pseudo-glioma and leading to phthisis bulbi by a process in every
way analogous to that forming the later stages of acute suppurative
inflammations (Fig. 1834).
One of the important complications of a cyclitis is the frequency of toxic dis-
turbances at the posterior pole of the eye whither cellular and toxic materials cast
Note the deposition of leucocytes on the swollen disc and adjacent retina
and also on the membranes in the vitreous (Straub, T. O. S.).
FIG. 1835.-PAPILLITIs IN IRIDo-cycliſtis.
off by the inflamed ciliary body into the vitreous are presumably carried by the
fluid current towards the optic disc. We shall see at a later stage" that scotoma,
macular disturbances, and optic neuritis may occur. The pathological basis of these
lesions was demonstrated by Straub (1912) who created an experimental cyclitis by
introducing organisms into the ciliary body and found the nerve head swollen, and
it and the adjacent retina besprinkled with leucocytes which were also plentiful in
the vitreous (Fig. 1835).
1 pp. 2656, 2985.

2190 TEXT-BOOK OF OPHTHALMOLOGY
In the choroid a similar picture characterizes sub-acute inflammations
as is seen in the iris and ciliary body, a picture which remains unaltered in its
essentials whether the lesion is single or multiple and disseminated, circum-
scribed or diffuse, or situated centrally or peripherally. Such a topographical
differentiation of the types of sub-acute choroiditis, indeed, has no true
pathological significance; they are all characterized by the same vascular
congestion, the same oedema and mononuclear infiltration leading to focal
necrosis and replacement by scar-tissue, the same disorganization of the
pigmentary epithelium and involvement of the retina.
The typical tissue-reaction in the choroid has been extensively studied in the
aseptic exudative choroiditis produced to promote the re-attachment of a detached
retina, whether by ignipuncture, chemical cauterization, diathermy or electrolysis."
In such cases studied experimentally on animals the choroid is found to be hyperºemic
and oedematous within an hour, when a leucocytic infiltration becomes evident ;
within 3 days connective tissue has begun to form; in 5 days the choroid and the
retina are firmly bound together and the cells of the pigmentary epithelium have
started to wander into the retina; and after 50 days the choroid is replaced by fibrous
tissue and the retina transformed into scar-tissue.
In infective cases, in the focal area and usually far beyond its
confines, the process is ushered in by a vascular dilatation, the endothelial
Fig. 1836.-Choroup1tis : EARLY STAGE.
An early patch in a syphilitic case of disseminated choroiditis showing pro-
liferation of the cells through Bruch's membrane (x 120) (Parsons).
cells of the capillaries being so swollen that they project into and
obstruct the lumen. Occasionally hamorrhages appear, which become
converted into a coagulum over which the retina ultimately suffers necrosis.
* For Bibliography see p. 2158.

DISEASES OF THE U WEAL TRACT 2191
This vascular reaction is rapidly followed by a mononuclear infiltration,
which, if it is diffuse, is usually more evident in the chorio-capillaris than
in the outer layers, but is frequently localized to form a modular choroiditis
(Fig. 1836). The infiltration obscures the stroma cells in the affected area,
but elsewhere they are seen to be rounded with their processes retracted.
The chromatophores wander to the superficial layers, crowd round the
infiltrated foci, and then begin to undergo degenerative changes, some
becoming rounded and clumped together and others breaking up, scattering
their pigment granules, many of which are phagocyted by leucocytes.
tº
SQ-
~
Fig. 1837.-Choroid ITIs: LATE STAGE ( x 15) (Buchanan, T. O. S.).
At the same time the retinal pigmentary epithelium over the infiltrated
area shows profound changes, most marked when the inner choroidal layers
are affected, and especially when Bruch's membrane is broken through. The
cells become oedematous, swell, and loosen, some of them breaking down and
scattering their pigment, others proliferating and heaping themselves up
in the sub-retinal space, at first as rounded cells devoid of pigment, but
possessed of active amoeboid movements (Krückmann, 1899). These
migrate into the retina accumulating preferentially round the vessels, and
if the continuity of the membrane of Bruch is broken, into the choroid also,
the free pigment being taken up by the vascular endothelium and phagocytic
cells (Fig. 1836).
These disintegrative changes are rapidly followed by reparative
activities, the two processes sometimes proceeding simultaneously. In the
choroid granulation tissue appears early and extensive fibrosis is the rule, the
infiltration with fibrous tissue not confining itself to this tissue alone but
T-0.-vol. III. 11

2.192 TEXT-BOOK OF OPHTHALMOLOGY
extending into the retina as well so that the two tissues are ultimately
blended into one continuous fibrous scar around which heaped-up clumps of
proliferating pigment cells are usually much in evidence, as well as irregular
masses of glial tissue (chorio-refinitis adhesiva) (Fig. 1837) (Krückmann,
1899). The fibrous tissue frequently spreads inwards, invading the vitreous
cavity (the choroiditis hyperplastica of Schöbl, 1889–90). As this tissue
passes through apertures in Bruch's membrane it may spread in the sub-
retinal space in large sheets, while the proliferating epithelium occupies
Fig. 1838.-Choroupiris : FIBRotic Stage.
Traumatic irido-cyclitis from a contusion 25 years previously. The choroid is
atrophic but vascular. There is a thick layer of vascularized fibro-cellular tissue
on its inner surface (Buchanan, T. O. S.).
every nook and cranny, appearing in histological sections as misshapen
isolated plugs and islets. As the scar-tissue contracts these may be distorted
into grotesque shapes, or the retina itself may be pulled and throttled out of
existence in some places and puckered and folded in others until it may
resemble a tumour (Paton, 1903; Parsons, 1904) (Figs. 1838–9).
A more unusual result is the ultimate development of a fibrous tissue opacity,
unconnected with the choroidal focus and having a predilection for the macular region.
It is fixed to the retina, but is not vascularized, thus differing from retinitis proliferans,
nor does it exert traction upon the retina or predispose to a detachment. Although
it may become thinner, it does not disappear, but vision through it may be surprisingly
good (Knapp, 1937).

DISEASES OF THE UVEAL TRACT 219.3
FIG. 1839.-RETINAL Folds CAUSED BY CONTRACTION OF FIBROUs TISSUE.
Note pigment proliferation (X 55) (Parsons).
FIG. 1840.-SUPRA-CHOROIDITIS.
Traumatic irido-cyclitis in which a thick layer of dense fibrous tissue formed
on the outer side of the choroid (x 55) (Parsons).


H 2
2.194 TEXT-BOOK OF OPHTHALMIOLOGY
Similarly exudation and infiltration followed by the formation of
granulation tissue and its subsequent consolidation to fibrous tissue may
spread outwards until the supra-choroidal space is occupied by dense fibrous
tissue. The chromatophores in this region show the usual changes of retrac-
tion of their processes and disintegration and liberation of their pigment, but
the most characteristic changes are seen in the endothelial cells (Fuchs,
1904). These throw out processes running parallel to each other or forming
a network, and ultimately develop into fibrous tissue showing a thick fibrillar
structure with few nuclei (Fig. 1840). Ultimately this tissue is vascularized
by new vessels derived from the ciliary arteries or the capillaries of the
FIG. I. 841.-GIANT CELLS IN RETINA.
In irido-cyclitis. The giant cells are arranged around cholesterol crystals, the
spaces from which these were dissolved out being seen in the figure (X 120) (Parsons).
sclera, and large spaces appear in it lined by endothelial cells which by their
growth detach the choroid from the sclera. The contraction of the fibrous
tissue also adds to the distortion, throwing the choroid into folds, and
ultimately this adventitious layer may become several millimetres thick,
thus meriting its description as a “fibroma of the choroid.”
At the same time, in addition to the cellular reaction, a considerable
amount of exudate, rich in protein and containing mononuclear cells, escapes
from the injured vessels, filters through the membrane of Bruch, permeates
the retina and reaches the vitreous cavity. Here it forms a cloud of dust-
like opacities, which may travel forwards through the posterior chamber
and even be deposited on the cornea. It is true that the grossest and most
numerous elements are derived from the ciliary body, but such an exudation

DISEASES OF THE UVEAL TRACT 2195
from the choroid to its inner or outer aspect is a constant phenomenon,
giving rise on occasion to a detachment of the choroid or retina, and in the
most severe cases leading to a complete detachment of the retina, its
enmeshment in a cyclitic membrane, and the formation of a pseudo-glioma
(Fig. 1811). The fluidis coagulable, greenish in colour owing to altered blood-
pigment, and frequently contains glittering cholesterol crystals, which,
reacting as foreign bodies, collect around them giant cells (Fig. 1841). Finally,
as the tissue-elements atrophy and degenerate, clumps of cholesterol crystals,
FIG. 1842, - BoME IN THE CHoRoip.
Hyaline mass in the choroid becoming calcified (x 160) (Parsons).
monocytes, pigment cells, giant cells, and fibrous elements form organized
cicatricial masses in the vitreous, the retina, and the choroid itself, tending
ultimately to undergo all types of degeneration—fatty, hyaline, cartilaginous,
and eventually in some cases ossifying (Figs. 1815, 1842).
Alt. A. f. Aug., vii, 310, 1877. Brückner. A. f. O., c. 179, 1919.
Amsler. K. M. Aug., lxxxv 835, 1930; Buchanan. T. O. S., xxi, 208, 1901; xxv,
lxxxvi, l, 1931. 121, 1905.
An. d’Oc., clºvii, 115, 1930. Bull. T. Am. O. S., xxxvii, 316, 1901.
Attias. A. di Ott., xxi, 1, 1914.
Axenfeld. B. O. G. Heidel.., xxv, 282, 1896;
xxx, 275, 1902.
A. f. O., x1 (3), 1 ; (4), 103, 1894.
Baas. Z. f. Aug., ix, 30, 1903.
Bartels. K. M. Aug., xliii (2), 15, 1905.
Bedell. J. A. m. Med. Ass., lxxix, 355, 1922;
lxxxii, 363, 1924.
Birnbacher and Czermak. A. f. O., xxxii (2),
1, 1886.
K. M. Aug., lxxxviii, 14, 1932.
Campos. An. di Ott., lxii, 360, 1934.
Capauner. B. O. G. Heidel.., xxiii, 45, 1893.
Collins. R. L. O. H. Rep., xiii, 361, 1892.
O. Rev., xii, 221, 1893.
Researches into the Anatomy and Path. of
the Eye, London, 1896.
Busacca.
Cordero. Am. di Ott., lxi, 65, 1934.
Cramer and Schultze. A. f. Aug., xxix., 288,
1894.

21.96
TEXT-BOOK OF OPHTHALMoLOGY
Derby. A. of O., lvii, 561, 1928.
Ehrlich. D. med. W., viii, 35, 1882.
Elliot. Lancet, i, 325, 361, 1917.
Flieringa. K. M. Aug., lxix, 241, 1921.
Am. J. O., viii, 70, 1925.
Franck. A. f. Aug., xlvii, 198, 1903.
Euchs, A. A. f. O., ciii, 228, 1920.
Fuchs, E. A. f. O., xxix (4), 209, 1883;
xxx (3), 139, 1884; lvi, l, 1903; lviii,
391, 1904; lxi, 365, 1905; lxxxiv, 201,
280, 1913; xcviii, 122, 1919; cv, 584,
1925.
K. M. Aug., lxiv. (1), 1, 1920.
Gallenga. A. di Ott., xii, 467, 1905.
Gifford and Hunt. A. of O., i, 494, 1929.
Gilbert. A. f. O., lxxvii, 199, 1910.
A. f. Aug., lxxxvii, 27, 59, 1920; lxxxix,
23, 1921.
B. O. G. Heidel.., xlii, 68, 1920.
Münch. med. W., lxi, 306, 1914.
Gilbert and Plant. A. f. Aug., xc, 1, 1921.
Ginsberg. A. f. O., xliv, 26, 1897; lxviii,
232, 1908; civ, 36, 1921.
K. M. Aug., lxiv. (1), 226, 1920.
Goh. A. f. O., xliii (1), 147, 1897.
Greeff. A. f. Aug., xxviii, 178, 1894.
Groenouw. K. M. Aug., xxxviii, 186, 209,
1900.
Grunert, B. O. G. Heidel.., xxx, 338, 1902.
Halben. A. f. A ug., xlix, 220, 1904.
Hanke. A. f. O., cv, 851, 1921.
Harms. K. M. Aug., xli (1), 522, 1903;
xlii (2), 25, 1904.
Heine. K. M. Aug., lxxi, 106, 1923.
Herrnheiser. K. M. Aug., xxx, 393, 1892;
xxxii, 137, 1894.
Prager. Z. f. Heilk., xiv. 1, 1893.
Herzfeld. A. of O., iv, 298, 1930.
Hess. K. M. Aug., xxx, 106, 1892.
Higuchi. K. M. Avg., xciii, 348, 1934.
Hirschberg. A. f. Aug., viii, 49, 1879.
Horner and Cordes. Am. J. O., xiv., 628,
1931.
Ischreyt. A. f. A wy., xxxvii, 305, 1898.
Jaensch. K. M. Aug., lxxvi, 476, 1926.
Junius. Z. f. Aug., xli, 247, 1919.
Kitamura. K. M. Aug., xlvi, 144, 1908.
Rnapp. A. of O., xviii, 558, 1937.
Rnies. A. f. O., xxii (3), 163, 1876.
A. f. Awg., ix, 1, 1880.
Koeppe. A. f. O., xcii, 115, 1917.
Koby. Slit-lamp Microscopy of the I.iving
Eye, London, 1930.
Rrückmann. A. f. O., xlviii (3), 237, 1899.
Lamb. Am. J. O., xxi, 137, 503, 1938;
xxii, 258, 1939.
Leber. G.-S. Hb., IT, vii (1), 1915.
Lindner. K. M. Aug., lxiv. (1), 217, 1920.
Lister. T. O. S., xli., 275, 1921.
Lohmann. K. M. Aug., xlviii (1), 75, 1910.
Luntz. Z. f. Aug., lxxiii, 380, 1931.
Nayer. A. of O., vii, 499, 1932.
Mayou. T. O. S., xliv, 81, 1924.
Meckel. Charité Annalen, v. 276, 1854.
Meesmann. B. O. G. Heidel.., xlvi, 300, 1927.
Meller. Z. f. Aug., lxxxvii, 239, 1935.
v. Michel. A. f. O., xxvii (2), 171, 1881.
Diss., Tübingen, 1902.
Müller, H. Gesammelte Schriften, Leipzig,
1856.
Munson. A. of O., xii, 99, 1934.
Mylius. A. f. Aug., lx, 147, 1926.
Nakashima and Sakurai. Acta O. S. Jap.,
xviii, 77, 1934.
Oblath. Beit. z. Aug., xli., 10, 1899.
Parsons. T. O. S., xxiv, 149, 1904.
Pathology of the Eye, London, i, 286, 336 ;
ii, 446, 1904–5.
Paton. T. O. S., xxiii, 63, 1903.
Pes. A. f. Aug., xlvii, 309, 1903.
Rados. A. f. O., lxxxv, 381; lz.xxvi, 213,
1913.
Raubitschek. K. M. Aug., lii (1), 683, 1914.
Ridley. R. L. O. H. Rep., xiv, 237, 1895.
Roth. Deut. Z. f. Chir., i, 471, 1872,
Ruge. A. f. O., lvii, 401, 1904.
Safar. Behandlung d. Netzhautabhebung,
Berlin, 1933.
Samuels. Am. J. O., xix, 493, 1936.
Sattler. A. f. O., xxii (2), 1, 1876.
Schirmer. A. f. O., xxxviii (4), 95, 1892;
lxxiv, 224, 1910.
Schweigger. Handbuch, Berlin, 1873.
Schöbl. A. f. Aug., xx, 98, 1889; xxi, 368,
1890.
Selenkowsky and Woizechowsky. A. f. Avg.,
xlvii, 299, 1903.
Sgrosso. Riforma med. Napoli, vi, 848, 1890.
Siegrist. B. O. G. Heidel., xxxviii, 303, 1912.
Spicer. Brit. J. O., Monogr. Suppl., i, 1924.
T. O. S., xxvii, 230, 1907.
Stallard. Brit. J. O., xvii, 294, 1933.
Stern. A. f. Aug., lxxvi, 80 ; lxxvii, 77, 1914.
Stock. K. M. Aug., xli., Beil., 17, 1903.
Beit. z. path. Anat., xliii, 470, 1908.
Stoewer. A. f. O., xlviii (1), 178, 1899.
Straub. A. f. O., lxxxvi, 1, 1913.
T. O. S., xxxii, 60, 1912.
B. O. G. Heidel., xxv, 108, 1896.
IX. Internat. Congr., Utrecht., 26, 1899.
v. Szily and Machemer. K. M. Aug., xc,
806, 1933 ; xcii, 44; xciii, 721, 1934.
Terry. A. of O., viii, 182, 1932.
Turk. A. f. O., lxix, 481, 1906.
K. M. Aug., xliv (1), 300, 1911.
Ulbrich. A. f. O., lviii, 243, 1904.
Ulrich. A. f. O., xxxviii (2), 239, 1882.
de la Vega. K. M. Aug., lxxi, 44, 1923.
Virchow. A. f. path. Anat., x, 1, 1856.
Vogt. A. f. O., cxi, 91, 1923.
Atlas, Berlin, II, i, 1930.
Vossius. Beit. z. Aug., ii, 62, 1891. -
Wagenrnann. A. f. O., xxxiii (2), 147, 1887;
xxxv (1), 172, 1889; xxxvi (4), 1, 1890;
xxxvii (2), 21, 1891; xxxviii (2), 91, 1892.
de Wecker. G.S. Hb. I, iv, 485, 1876.
Werncke. K. M. Aug., xli., Beil., 283, 1903.
Wolf. A. f. Aug., xc, 29, 1921.
Zade. A. f. O., lxxxv, 294, 1913.
Zimmermann. Z. f. Aug., lvii, 279, 1925.
DISEASES OF THE UWEAL TRACT 2197
The General Symptomatology of Uveitis
Depending on the type and acuteness of the inflammatory process and
the region preferentially attached, the symptoms of uveitis vary considerably
but it is convenient here to discuss the main symptoms which are common
to the majority of conditions.
THE ESSENTIAL SYMPTOMs OF IRITIS may be described in two groups:
(1) Objective symptoms of (a) Hyperaemia and ciliary injection.
(b) Exudation into the tissues and into the
chambers of the eye to form precipitates,
synechiae, and vitreous opacities.
(c) Changes in the size, shape and reaction
of the pupil.
(a) Pain.
(b) Photophobia.
(c) Increased lacrimation.
(d) Blurring of vision.
Of the objective symptoms, the HYPERAEMIA is the first to appear, and it
is usually marked ; indeed, owing to the extreme vascularity of the iris and
the looseness of its stroma, the classical signs of inflammation—dolor,
tumor, calor, rubor—are all well accentuated. It is usually first evident
as a peri-corneal ciliary injection, an engorgement essentially of the episcleral
vessels round the limbus appearing as a rose-red flush, the characteristics of
which have already been described." Frequently the conjunctival vessels
are also engorged, but the underlying ciliary injection and the absence of a
discharge indicates the nature of the condition. Sometimes the associated
oedema may belly out the mucous membrane to form a chemosis, but this is
more usually a symptom of a complicating cyclitis. The swelling of the
tissues of the iris and the blurring of its pattern may tend to obscure the
hyperaemia in this tissue itself, but close examination usually reveals the
engorged and distended radial vessels.
In acute cases the hyperaemia may be so great as to lead to the formation
of many petechial haemorrhages and merit the description HAEMORRHAGIC
IRITIS. These may be both inside the tissue and upon its surface, and
eventually blood in considerable quantity may collect in the anterior chamber
to form an IRITIS WITH HYPHAEMA (Fig. 1885), a condition associated by the
older authors essentially with gout and rheumatism (Hutchinson, 1885;
v. Michel, 1890; Reche, 1892), but which is now considered typical of
gomococcal infections (Gilbert, 1911). More rarely such bleedings are seen in
herpetic iritis (Gilbert, 1911) and in the iritis associated with erythema
modosum (Reis, 1906).
The ExUDATE within the tissues of the iris is usually so considerable as to
produce well-defined clinical changes. The iris, instead of having the
1 Vol. II, p. 1500.
(2) Subjective symptoms of
21.98 TEXT-BOOK OF OPHTHALMOLOGY
consistency of a wrung-out sponge, assumes that of a sponge engorged with
a sticky fluid. The delicate tracery of its architecture is lost, and its fine
colouring is clouded over so that it becomes sodden in appearance, like wet
blotting paper. Partly because of this and partly because of the hyperaemia,
the colour changes from blue to green, from grey to a muddy soot colour, and
from brown to a reddish yellow. Escaping from the tissues the exudation
becomes apparent in the anterior chamber, at first by a turbidity of the
aqueous owing to an increase in its protein content distinguishable with the
slit-lamp as an “aqueous flare,” 4 then by the entrance of cellular elements,
and sometimes by the appearance of a meshwork of fibrin. When the ciliary
body is involved the exudates are always more abundant, but, as we have
seen, the iris also forms a source of origin, and they are deposited in solid
clumps on the back of the cornea as keratic precipitates,” as well as on the
surface of the iris and lens, and when they are formed in quantity in acute
cases, they collect at the bottom of the anterior chamber to form the
greenish-yellow mass of a hypopyon (Fig. 1844). Finally, in plastic
cases, the exudates covering the iris glue it to the lens, forming POSTERIOR
syNECHIAE, which may appear as isolated strands, or, in cases where the
exudative element is great, lead to seclusion or occlusion of the pupil.”
Particularly in sub-acute or chronic inflammations of relatively low virulence
exudative and cellular accumulations may be found on the iris, which are termed
NoDULES: pathologically, as we have seen,” they differ from tubercles in that
they consist of accumulations of epithelioid cells and lymphocytes without the loss of
tissue. Their occurrence has been known for a considerable time (the guttate iritis
of Doyne, 1910), but the frequency of their occurrence has only been realized since the
slit-lamp came into general use. In this connection they were first adequately described
by Gilbert (1914) and later by Koeppe (1917), and are generally known as KOEPPE
NODULEs. Doyne associated their presence with gout, and many authors look upon
them as an indication of tubercle ; but they undoubtedly occur in other conditions
and are non-specific, merely indicating the presence of a low-grade uveitis. As a general
rule their presence should be associated especially with tubercle, syphilis, leprosy, and
sympathetic ophthalmitis.
Such nodules appear as small translucent grey lumps and on occasion they may
be quite numerous ; thus in a tuberculous case Finnoff (1933) counted 42, a number
which became reduced to 11 within 3 days.
As we have seen they are usually divided into two types :
(1) Ectodermal modules (the true Koeppe type) on the pupillary border (Fig. 1909).
These are the commoner type and they appear as white efflorescences which seem to
rise out of the posterior layer of the iris.
(2) Mesodermal floccules (Busacca, 1932) (Fig. 1907) on the anterior surface of the
iris. The nodules usually last some weeks, but the mesodermal floccules may be very
transient, or may occasionally become conglomerate and vascularized.
On the mesodermal part of the iris they lie sometimes within the iris tissue on
an area which has undergone ulceration, sometimes on its surface. They are usually
most numerous near the collarette ; near the periphery they may give rise to the
formation of anterior synechiae which may obliterate the angle of the anterior chamber.
1 p. 2234, Fig. 1851. 2 p. 2179. 8 p. 2168. 4 p. 2181.
DISEASES OF THE UVEAL TRACT 2199
The CHANGES IN THE PUPIL follow from the behaviour of the exudate.
At first it is small and sluggish in its reactions owing to the engorgement and
waterlogging of the tissues, an effect enhanced by the stimulation of the
nerves by irritant and toxic substances and the mechanical impairment of
the muscle fibres by cellular infiltration (Brückner, 1919). Affecting both
muscles equally, the stimulation of the more powerful sphincter overcomes its
weaker antagonist, and the scattered fibres of the dilatator are embarrassed
more readily than the compact bundles of the sphincter. At a later stage the
development of synechiae makes the pupil irregular, a deformity enhanced if
an attempt at dilatation is made by a mydriatic. When organization to
fibrous tissue has occurred, the iris becomes fixed and immovable ; but if
mydriasis is attempted before this the synechiae may be stretched, giving
the pupil an irregular festooned appearance, or the synechiae may be broken
down, leaving anchored to the lens capsule remnants of the posterior
pigmented layer of the iris which remain permanently to indicate the
presence of past inflammation.
Of the subjective symptoms pain is the most prominent, a fact not sur-
prising in view of the rich nerve supply from the trigeminal. The pain is an
acute spasmodic ciliary neuralgia superimposed upon a dull ache, and is
worst in the acute stages when the tissue is swollen and hyperaemic and the
nerve-endings are stimulated by a high concentration of toxic substances.
At a later stage exacerbations occur when synechiae add their mechanical
irritation ; as was pointed out by v. Graefe (1855), the pain therefore
decreases either when the pupil is fully contracted or (preferably) when the
adhesions are broken down. It is not confined to the eye but may radiate
all over the distribution of the trigeminal to the brow, the malar region and
the cheek, but rarely to the nose and teeth. With it are associated a reflex
photophobia and lacrimation, both evidences of trigeminal irritation ; and
to the patient’s discomfort may be added a generalized headache.
This generalized headache is taken by some authors (Heine, 1913–14; Gilbert,
1927) to indicate meningeal irritation. As the retina may be considered an out-
post of the brain, so the uvea can be looked upon as an extension of the pia-arachnoid ;
and associated with an acute iritis symptoms of meningeal disturbance, such as a
raised intra-cranial pressure, have been observed.
The remaining symptom is a diminution of vision which appears always
from the beginning of the illness. It is mainly due to exudative opacities in
the aqueous, the vitreous and in the pupillary aperture, and when these are
gross in quantity, particularly in the last situation, the reduction of vision
may lead to complete incapacity. The earlier authors considered that much
of this was due to a secondary involvement of the retina (Schnabel, 1876;
Hirschberg, 1888); but the haziness of the retina which they described as
associated with an iritis was probably mainly attributable to opacities in
2200 TEXT-BOOK OF OPHTHALMOLOGY
the media. It is true, as will be noted presently, that inflammatory com-
plications may occur at the posterior pole, but in iritis these are rare and
incidental.
A CYCLITIS of some degree, as we have seen, invariably accompanies an
iritis, and when it is marked it involves an accentuation of all the symptoms.
The hyperaemia and ciliary injection may be increased to result in chemosis,
the exudation into the chambers of the eye produces liberal quantities of
keratic precipitates and vitreous opacities, the pupil is distorted or blocked
by synechiae, and the neuralgic pain is intensified. One sign is of especial
importance—a lowering of the intra-ocular tension, which always indicates
involvement of the ciliary body.
In cases of cyclitis, however, with little or no evidence of iritis, the
symptomatology may be much less dramatic. In severe cases there is ciliary
injection involving the deeper vessels, and there may be neuralgic pain with
tenderness on pressure on the ciliary region, but quite frequently these signs
are absent. The only constant occurrence is the presence of exudate which
becomes evident as keratic precipitates and vitreous opacities, and in the
milder cases these may occur in an otherwise apparently normal eye. Since
in their earlier stages or in quiet and chronic cases these may be minimal in
degree and readily missed, the greatest care should be taken in the clinical
examination of patients who complain of indefinite symptoms such as a slight
impairment of vision without obvious cause : a minute speck on the corneal
endothelium visible only to the slit-lamp, a few vitreous opacities, or a change
in colour of the iris are sufficient to establish the diagnosis. Posterior
synechiae may only appear slowly and insidiously and may never be
prominent, and the anterior chamber is frequently deep owing partly to a
damming back of the aqueous which, with its high content of albumen, finds
its exit difficult, and partly, especially in the later stages, to retraction of
organized exudate in the posterior chamber. As an incident, an irritative
contraction of the ciliary muscle may induce myopia ; while one of the more
unobtrusive signs is that an interference with the fluid-traffic in this import-
ant region frequently induces a significant lowering in tension. In the more
severe plastic cases the clinical picture is dominated by the formation of
exudate, which may plaster the cornea with precipitates, occlude the pupil,
surround the lens, form a cyclitic membrane behind it, give rise to massive
fibrosing strands in the vitreous leading to a detachment of the retina,
and completely disorganize the ciliary body itself resulting in phthisis bulbi.
As a complication of irido-cyclitis there is, of course, frequently an
anterior choroiditis ; to this must be added the occasional occurrence of a
complicated cataract, and the involvement of the scleral and episcleral tissue
by infiltration, particularly in the region of the canal of Schlemm and the
anterior perforating vessels. Three further complications must be noted—
DISEASES OF THE UVEAL TRACT 220]
the participation of the cornea in the inflammatory process, involvement of
the retina and optic nerve, and the vagaries of the intra-ocular pressure.
In the most severe and acute cases of suppurative inflammation we
have already seen that the cornea may be gravely affected, the toxic process
bringing on a catastrophic sequence of ring abscess, massive necrosis and
perforation.” Even in the milder cases, however, the cornea is to some
extent involved. It may be that the only evidence of its share in the
inflammation is an oedematous swelling of the endothelium with a haziness
of the cells just discernible with the slit-lamp ; in other cases the damage to
the endothelium is profound and extensive, large portions being exfoliated
in sheets, leaving denuded areas which become plastered with exudate which
subsequently organizes to leave permanent opacities (Fuchs, 1883; endo-
theliogenous connective tissue, Wagenmann, 1888–96).”
The extent of the deep corneal involvement associated with the deposition of
keratic precipitates is best seen clinically by the method of deep staining (v. Hippel,
1902), wherein it will be remembered “ that if fluorescein is instilled into the con-
junctival sac and allowed to remain there for a few minutes behind the closed lids,
the dye penetrates the intact epithelium showing up endothelial defects and keratic
deposits as green dots, while damaged areas of the stroma become grass-green. In
this event three layers of opacity can be distinguished—the cloudy substantia propria,
the Smudged endothelium, and the precipitates, while an oedematous condition of the
opposite area of epithelium can sometimes be remarked.*
In addition to endothelial disturbance it is not uncommon to find some degree
of keratitis profunda especially in the central area due to toxic action ; indeed, so
marked may this be that it is sometimes difficult to decide on clinical grounds which
is the primary disease, as for example, in the keratitis and anterior uveitis associated
with syphilis " or in cases of keratitis profunda associated with uveitis of more indefinite
aetiology." Finally, corneal involvement may occur at the periphery by direct spread
through the angle of the anterior chamber, a complication most readily seen in
granulomatous lesions, such as tubercle.
Inflammatory complications involving the retina and optic nerve in
affections of the anterior segment of the uveal tract are rare, but of con-
siderable importance ; they are probably due to the diffusion of toxins
towards the posterior pole of the eye with the fluid traffic in the vitreous body.
We have already seen that the haziness described by early writers as a
retinitis (Schnabel, 1876; Hirschberg, 1888) is probably attributable to
opacities in the media, but in severe cases of irido-cyclitis a macular occlema
may be evident (Lippmann, 1921; Zeeman, 1923). A secondary retinal
phlebitis may also occur (Meller, 1921) and coincident optic neuritis (Meller,
1921 ; Gilbert, 1922; Kleinsasser, 1922; Fry, 1938). Finally, a central
scotoma may appear without visible Ophthalmoscopical changes, at first to
colours and then to white (Clegg, 1922–24). This most incapacitating
1 pp. 1990, 2164. * For clinical and pathological appearances see Vol II, pp. 1857–58.
* Vol. II, p. 1131. 4 Vol. II, p. 1862. * Vol. II, p. 1982. * Vol. II, p. 1982.
2202 TEXT-BOOK OF OPHTHALMOLOGY
symptom is probably a toxic effect, and fortunately tends to disappear with
a subsidence of the active inflammation in the anterior segment.
We have already seen + that the pathological proof of the occurrence of these
conditions has been furnished by Straub (1912), who found that an optic neuritis
followed an experimentally induced cyclitis, the optic nerve head and the adjacent
retina being sprinkled with leucocytes derived from the ciliary body continuous with
those pervading the vitreous gel (Fig. 1835).
The behaviour of the tension of the eye is frequently of great importance
in irido-cyclitis. In this connection two distinct conditions are evident.
The first occurs during the acute inflammatory stage or frequently in the
course of a relatively quiet cyclitis when a raised tension develops typically
with a deep anterior chamber. Its onset is presumably due to a disturbance
of vaso-motor reflexes, to which may be added difficulties presented to the
free circulation and drainage of the intra-ocular fluid partly owing to its
increased viscosity, loaded as it is with proteins, fibrin, and exudative cells
which clog the meshes of the filtration angle, while the possibility of the
osmotic retention of water by the abnormally concentrated intra-ocular
fluid must also be considered. The second type of raised tension is a more
simple matter : it occurs after the initial inflammatory stage and is probably
due to mechanical obstruction of the circulation of the intra-ocular fluid
either by organized exudate at the pupillary aperture or by granulation,
hyaline, or fibrous tissue blocking the angle of the anterior chamber, a
complication which may occur obviously and soon, or, particularly if
peripheral synechiae are involved, slowly and insidiously several years
later. In all these circumstances a secondary glaucoma is by no means
uncommon, and it may form a complication of great gravity, the treatment
of which presents problems of more than usual difficulty.” Conversely,
this acute calamity may be replaced by an insidious danger when the
ciliary body is involved, when interference with the processes of formation
of the intra-ocular fluid may lead to a lowering of the ocular tension, a
frequent transient event in the initial stages of cyclitis, and a common
permanent state at the terminal stage if the ciliary body becomes dis-
organized when it entails the development of phthisis bulbi.
The only conditions with which a differential diagnosis arises in this connection
are between an acute iritis or irido-cyclitis and an acute conjunctivitis or an acute
glaucoma—all conditions characterized by AN ACUTELY RED EYE. Of the two, the
differential diagnosis with glaucoma is of the utmost importance since the treatment
of the two is diametrically opposite, atropine being a necessity in iritis and disastrous
in glaucoma. Between them no difficulty should arise as will be evident from Table
LXIII, wherein the distinguishing features are annotated. If despite these points
doubt is still felt, it may be legitimate to instil homatropine (2.0%) or euphthalmine
in the conjunctival sac—atropine should never be used, since in glaucoma its effects
cannot be neutralized by eserine : in conjunctivitis the only effect is a regular and
1 p. 2189. * p. 3290.
DISEASES OF THE UVEAL TRACT 2203
normal dilatation of the pupil ; in iritis, if the pupil dilates, synechiae will be exposed,
but the tension remains normal ; but in glaucoma, while the pupil may dilate further,
it remains regular and the tension usually increases considerably.
TABLE LXIII
Acute conjunctivitis. Acute iritis. Acute glaucoma.
1. Pain Nil : some discom- || Moderate : mainly in Very severe : in eye
fort eye and 1st branch with complete Vth
Of Vth neuralgia radiating to
jaw and beyond
2. Tenderness Nil Marked Marked
3. Injection Superficial : see | Deep ciliary : see p. Deep ciliary : see p.
p. 1500 1500 1500
4. Pupil Normal Small and irregular Large and oval
5. Tension Normal Usually normal or low High
6. Media . Clear Opacities in pupil Cornea steamy
7. Secretion Muco-purulent Watery Watery
8. Vision . Good Fair Poor
9. Onset Gradual Usually gradual Sudden
10. Systemic Nil Few Prostration and
complications vomiting
THE CLINICAL SIGNS AND SYMPTOM'S OF CHOROIDITIS are few : character-
istic subjective symptoms peculiar to choroiditis do not exist, and objective
signs are limited to Ophthalmoscopic appearances. Unless in the acute
purulent forms, which are uncommon, and which may be associated with
conjunctival injection and chemosis, there is no external manifestation of the
deep-seated disease; the appearances revealed by the ophthalmoscope com-
prise changes in the fundus and opacities in the media (Plates XXXV,
XXXVI).
The clinical appearance of a patch of acute choroiditis is characterised
by a pale yellow or greyish-white area with ill-defined edges, due partly
to the accumulation of inflammatory exudates, partly to disturbance of
the overlying pigmentary epithelium, and partly to the retina becoming
cloudy and swollen Owing to the seepage of toxic exudative fluid from the
choroid through Bruch's membrane. Over these areas, however, so long as
the lesion remains choroidal, the retinal vessels course normally. This may
be called the eacudative stage. As the acute phase passes, the atrophic stage
commences. The affected area becomes more sharply defined and becomes
delineated from the rest of the fundus, while in the site of inflammation itself
two changes take place : first, the appearance of white patches, due some-
times to the formation of fibrous tissue as the exudates become organized
by the fibroblastic activity of the choroid, and at others to the thinning and
atrophy and final disappearance of choroidal tissue so that the sclera shines
through ; and second, the phenomenon of pigment heaping, due partly to
the accumulation and partly to the proliferation of the pigmentary cells, so
2204 TEXT-BOOK OF OPHTHALMOLOGY
that masses of black pigment are seen in the atrophic areas, more particularly
at their edges. This appearance—a clear-cut atrophic area with scattered
patches of pigment and surrounded by a black pigmentary zone—is the
natural termination of the lesion and remains as a permanent cicatrix.
Some vitreous opacities are the rule in choroiditis. These may be few
in number but when, as frequently happens, the continuity of Bruch's
membrane is broken and the exudates pass through the retina freely, they
may be very considerable in amount. When they are excessive in quantity,
however, the suspicion always arises that the ciliary body is also involved ;
but even without this complication, it is not uncommon for some fine
opacities to circulate forwards into the anterior chamber and be deposited
on the posterior surface of the cornea, even although the choroidal lesion is
limited to the posterior pole of the eye.
The subjective symptoms of choroiditis are limited to visual phenomena,
pain or other subjective disturbances being completely absent ; therefore it
frequently happens that, unless an important area such as the central region
is affected, the disease escapes the patient’s notice altogether at the time,
and the previous existence of the lesion may only be established after some
long interval when a healed scar is discovered accidentally during the course
of a routine examination. The symptoms comprise two types of
phenomena :
1. Abnormal subjective appearances—(a) photopsiae :
(b) vitreous opacities.
2. Disturbances in vision—(a) metamorphopsia, macropsia, micropsia.
(b) Scotomata.
At the commencement of the illness, or at the spreading periphery of an
established lesion, irritation of the rods and cones gives rise to the subjective
sensation of flashes of light (PHOTOPSIAE) in the corresponding region of the
visual field. This does not invariably occur, and it is frequently followed by
the appearance of a cloud of vitreous opacities indicating the outpouring of
exudative material. In a peripheral lesion these are the only two available
symptoms of a choroiditis, and frequently the latter is the only one to be
remarked. When the disease becomes established disturbances of vision
occur, due first to a rucking up of the retina over the area affected, and
eventually to its subsequent destruction. When the retina is raised up in an
irregular manner, the image becomes distorted (METAMORPHOPSIA); and if
the visual elements are separated the image appears smaller (MICROPSIA),
while if they are crowded together the opposite phenomenon of MACROPSIA
appears, since in the first case fewer, and in the second, more rods and cones
are stimulated. These phenomena, it will be appreciated, can rarely be
observed unless the macular area is involved. Eventually, as the retinal
damage progresses, a black spot is projected in the visual field corresponding
to the lesion (a positive scotoma), and finally this merely becomes a blank
hiatus in the field (a negative scotoma). Occurring in the macular region
DISEASES OF THE UVEAL TRACT 2205
such a scotoma, of course, is disastrous ; in the mid-region of the fundus it
may or may not be appreciated ; but in the periphery it is completely
neglected. In most cases, especially if the inflammatory process is a mild
and superficial one, the scotoma corresponds to the area of retinal
destruction ; but in cases of severe and deep inflammation, the resultant
scotoma may spread beyond its confines and extend to the periphery of the
field in a wedge-shaped or sector-shaped defect. We shall see presently 1
that the cause of these extended defects is unknown, some authorities con-
sidering that the loss of function in the sector affected is due to destruction
of nerve fibres (Groes-Petersen, 1912; v.d. Hoeve, 1914) and others attribut-
ing it to vascular influences (Jensen, 1908; Hepburn, 1912–14).
Of the complications which follow in the train of choroiditis we have
already seen that the most common is a retinitis; indeed, this complication
is probably invariable and the disease can usually be best described as a
CHORIO-RETINITIS. The membrane of Bruch offers considerable resistance
to the inflammatory process, but at an early stage toxic exudative fluid finds
its way through it making the Overlying retina cloudy and Oedematous ; and
in the later stages this membrane may become disintegrated when exudative
cells find their way freely into the retina, so that the ultimate result is a
complete fusion of the retina and choroid in a mass of organized fibrous tissue.
Other complications which frequently follow are the involvement in the
inflammatory process of the ciliary body and the iris to form a general
uveitis; of the optic nerve in a neuritis which, after the inflammation has died
down, may develop into a choroiditic atrophy ; of the lens, which, owing to a
direct toxic effect, may develop a complicated cataract ; and, finally, of the
sclera by the extension outwards of the inflammatory process, to form a
sclero-choroiditis.
Brückner. A. f. O., c, 179, 1919. Hepburn. T. O. S., xxxii, 366, 1912.
Busacca. K. M. Aug., lxxxviii, 14, 1932. R. L. O. H. Rep., xix, 398, 1914.
Clegg. Brit. J. O., vi, 118, 1922. v. Hippel. A. f. O., liv, 509, 1902.
T. O. S., xliv., 86, 1924. Hirschberg. Ophthalmoskopie in Eulenberg’s
Doyne. T. O. S., xxx, 91, 1910. Realenzyklopädia, II, xiv, 685, 1888.
Hb. d. v. d. Hoeve.
Eversbusch. Pentzoldt-Stintznig's
ges. Therapie, v, 1911.
Finnoff. A. of O., ix, 13, 1933.
Ery. A. of O., xix, 833, 1938.
|Fuchs. A. J. O., xxix (4), 209, 1883; xcii,
145, 1917.
Gilbert. K. M. Aug., xlix (1), 649, 1911.
Münch. med. W., lxi, 306, 1914.
A. f. Aug., lxxxvi, 50, 1920.
G.-S. Hb., II, v (6), 1, 1922,
B. O. G., Heidel., xlvi, 86, 116, 1927.
v. Graefe. A. f. O., ii., 187, 1855.
Groes-Petersen. K. M. Aug., l (2), 159,
1912.
Heine. Münch. med. W., lx,
1913; lxi, 2092, 1914.
1305, 2441,
K. M. Aug., liii, 487, 1914.
Hutchinson. T. O. S., v., 1, 1885.
Jensen. A. f. O., lxix, 41, 1908.
Kleinsasser. Z. f. Aug., xlviii, 61, 1922.
Roeppe. A. f. O., xcii, 115, 1917.
Lippmann. K. M. Aug., lxvii, 63, 1921.
Meller. A. f. O., cv, 299, 1921.
v. Michel. Lehrbuch d. Aug., Wiesbaden, 1890.
Reche. K. M. Aug., xxx, 176, 1892.
Reis. K. M. Aug., xliv (2), 203, 1906.
Z. f. Aug., lxix., 174, 1929.
Schnabel. A. f. Aug., v., 101, 1876.
Straub. T. O. S. xxxii, 60, 1912.
Wagenmann. A. f. O., xxxiv. (1), 211, 1888;
xxv (1), 172, 1889; xxxvii (2), 21, 1891;
xxxviii (2), 91, 1892; xlii (2), 1, 1896.
Zeeman. A. f. O., czii, 152, 1923.
1 p. 2246.
2206 TEXT-BOOK OF OPHTHALMOLOGY
The General Treatment of Uveitis
In so far as we are dealing under this heading with a multitude of clinical
conditions the aetiology of which is legion, it is obvious that treatment must
vary very considerably in individual cases, but it will be instructive as well as
economical in space, to summarize the main principles of general treatment
here, and to indicate subsequently specific modifications applicable to
particular cases when these are being individually described. These general
methods of treatment we shall discuss under four headings: aetiological,
local, systemic, and surgical.
(a) AETIOLOGICAL TREATMENT
The most important—and frequently the most difficult—problem in
dealing with a case of inflammation of the uveal tract, is to determine
promptly the cause of the lesion, and, this having been decided upon with
as much certainty as possible, to eliminate it or, alternatively, to counteract
its effects, for even although local treatment may abort a single acute attack,
it cannot be expected to act so efficiently nor can it be expected to preclude
subsequent recurrences unless the cause is dealt with. In some cases, it is
true, such as in exogenous infections, the aetiology is obvious, but in most
the reverse is the case. Again, it is frequently easy to find a possible cause ;
but after it has been eliminated the fact that the inflammation still drags on
stimulates a further search which reveals a second possible cause, a process
which may be repeated over a long period during which much valuable time
is lost and irreparable damage is done. For these reasons, therefore—the
obscurity of most cases, the possibility that the obvious cause may not be
effective, the probability of the existence of more than one focal cause, and
the tendency of such inflammations to become chronic or be recurrent with
eventual disatrous results—for these reasons it is advisable that in all cases
a complete systemic investigation of each patient should be undertaken.
Such an investigation entails not a little work. It includes a blood
examination, which involves a Wassermann reaction or other test for
syphilis, a complement fixation test for gonorrhoea, a blood count, which
frequently provides valuable information as to the type of infection present,
and a sedimentation estimation which may give some index of the degree
of general toxicity. It includes a bacteriological investigation of the
nose, naso-pharynx, throat, bowel, and urine, and, when indicated, of the
prostatic secretion or the cervix uteri. In assessing the necessity for these
last investigations the prevalence of a non-gonorrhoeal prostatitis or
vesiculitis or endometritis must be remembered. It also includes a clinical
and radiological examination of the nasal sinuses and teeth, the latter being
advisable even in edentulous patients, for in not a few of these a buried root
or an area of residual Osteitis in the alveolus forms an active and virulent
area of absorption, the neglect of which may lead to serious results. It is to be
DISEASES OF THE UVEAL TRACT 2207
noted that the same pitfall may arise with regard to tonsillar remnants
which may well be bacteriologically infected : here again, as in the nose, the
fact that an operation has been performed on an infected area does not
justify the assumption that a cure has been complete. It should also include
a skin test for the existence of allergy to tubercle, and if this is positive a
systemic examination to determine the existence and activity of any
tuberculous lesion—a most important consideration when specific treatment
is instituted. Finally, if no apparent cause can be detected, it should be
remembered that an infection of the female pelvic organs may be symptom-
less, and that these, as well as the gall-bladder and appendix, have been
inculpated in the production of uveal disease, although rarely.
This information having been gathered (and it should not occupy much
time, for at the beginning of an attack every day is of importance) an
assessment must be made ; and this frequently calls for more than usual
clinical judgment. In the case of tubercle, syphilis, gonorrhoea and other
typical infections the indications for treatment are clear ; but in many cases
when only indefinite evidence is obtained, or evidence of multiple infections,
as a streptococcal infection of the teeth, tonsils and colon, the whole aspect
of the case must be weighed. The ultimate cure of uveal inflammation
depends not only on the elimination of an infective focus, but also on the
maintenance of the patient's health and the raising of his resistance;
over-drastic mutilating surgery undertaken rather as the result of trial and
error than of reasoning on a broad medical basis may produce results upon
the physical and mental health of the patient more disastrous than the
initial disease itself. It is to be remembered, however, that if an undoubted
source of infection exists, the patient is better without it, even apart from the
uveal inflammation ; and if it is removable, the sooner it is done the better,
after the inflammation in the eye is under control. The danger of exciting a
severe relapse in uveal inflammation by the removal of such a focus at any
other than the acute initial stage, provided the patient is reasonably fit, is
frequently over-exaggerated; and provided he is not over-dosed by toxins
and debilitated by shock—as may occur, for example, after the extraction of
too many teeth at one time—any local exacerbation in the eye is exceptional
and much more alarming than dangerous. Indeed, such an occurrence rarely
causes permanent damage, and much the more usual result is a relief of the
symptoms so prompt and effective as to justify any slight risk which the
adoption of the bolder policy may entail. In many cases, of course, a focus
may be incriminated which cannot be removed, as may arise, for example,
in the nasal sinuses, the colon, the prostate, and so on. In these circum-
stances everything should be done to mitigate the intensity of the infection
by local measures (lavage, massage, etc.). At the same time in these, as well
as in the other cases where more radical measures are possible, the systemic
effect of the infection may be neutralized by the administration of vaccines.
The value of vaccine therapy in uveitis is a question on which the widest
T.O.-WOL. III, I
2208 TEXT-BOOK OF OPHTHALMOLOGY
divergencies of opinion are held ; undoubtedly the widest divergencies of
results are obtained. The theoretical basis of the treatment is the active
immunization of the patient by the stimulation of the fixed cells so that they
themselves are activated to produce immune bodies against the offending
organism. Irregularity in results is only to be expected when one remembers
that our knowledge of the complex mechanism of immunity is still very
superficial and that our technique and dosage may well be at fault, that
many patients may not be in a position to respond to such stimulation, and
that the best results can only be anticipated when an autogenous vaccine is
administered—autogenous in the sense that it is prepared from the organism
which causes the lesion—and this is an extraordinarily difficult matter to
adjudicate upon in those numerous cases where a precise aetiology is doubtful.
It is to be remembered, for example, that even if it is decided that a lesion
is streptococcal the varieties of streptococci are legion. Vaccines must
therefore be used judiciously, and their indiscriminate use without adequate
laboratory and medical control is an unjustifiable attitude of levity and
unintelligence.
(b) LOCAL TREATMENT
The local treatment of uveal inflammations involves primarily three
main desiderata—the establishment of mydriasis, the application of heat,
and the relief of pain. The advent of complications, such as the formation
of synechiae or the development of increased tension produces other
problems which may have to be solved by surgical interference.
Mydriasis is usually attained most easily by atropine, which should be
exhibited at the soonest possible moment. This drug has a three-fold
action : (1) by paralysing the intra-ocular muscles it puts the tissues at rest,
a factor of great value in combating any inflammation ; (2) by relaxing
the ciliary muscle through which the long posterior ciliary arteries pass it
increases the blood-supply to the anterior segment and by abolishing the
pull of the insertion of the muscle on the choroid it encourages congestion,
both reactions aiding hyperaemia," and (3) it dilates the pupil. The more
so is its early administration urgent in iritis, since, in addition to the rapid
relief of inflammation and pain which it produces by putting the
tissues at rest, the importance of preventing the formation of synechiae cannot
be overstressed. Granted that dilatation of the pupil is attained and main-
tained from the commencement, a case of iritis loses most of its anxieties,
and the danger of permanent damage to vision is minimized. Its early and
efficient administration, therefore, may make a world of difference for the
patient’s lifetime. With almost equal urgency this also applies to cyclitis;
and even to choroiditis, for it must be remembered that the ciliary muscle
runs backwards behind the equator, and entoptic observations show that
on its contraction the retina as far back as the fovea is appreciably pulled
* Vol. I, p. 422 (423).
DISEASES OF THE UVEAL TRACT 2209
upon. Apart, therefore, from its action in mechanically hindering the
formation of synechiae, the value of atropine lies in its effect in producing
absolute rest and encouraging hyperaemia in the inflamed tissues most
particularly in the iris and ciliary body, but also in the choroid.
Its use may produce three unpleasant effects—a distressing dryness
of the throat, symptoms of general intoxication (flushing, fever, delirium,
etc.), and symptoms of atropine irritation. The first two phenomena are
largely due to its passage down the naso-lacrimal duct and its absorption
from the throat. They may be mitigated by pressure on the canaliculus
for some minutes after the drug is instilled, or by its administration in
oily solution or as an ointment. This last is especially useful in children,
who are more susceptible to atropine poisoning than adults, and more-
over, in them its application in this way is easier as it is absorbed into
the eye if it is rubbed into the lashes. The problems of atropine irritation
have already been discussed "; it will be remembered that it is a typical
allergic reaction in the conjunctiva and surrounding skin unassociated with
general symptoms of intoxication. Although not a serious condition and
although it disappears soon after the atropine is left off, the discomfort which
it causes and the development of an acute moist eczematous irritation
resembling erysipelas, necessitate the discontinuance of the drug; it is to be
borne in mind, also, that once the state of sensitivity has developed, any sub-
sequent attempts to employ atropine, even after the lapse of years, frequently,
but not invariably result in a sharp recrudescence. If this phenomenon
appears, therefore, although the immediate irritation can be mitigated by
the application of adrenaline packs, atropine should be replaced by some
other mydriatic : hyoscine hydrobromide (0.25 to 1%), scopolamine
hydrobromide (0:25 to 1%), or duboisine sulphate (0.25 to 1%). It not
infrequently happens, however, that irritation develops to all four drugs,
in which case, as an alternative to discontinuing mydriatics altogether, an
attempt may be made to desensitize the patient (Waller, 1934). The
technique of this is described elsewhere *; but unfortunately the method
suffers from the two drawbacks that it is by no means always effective and
that the desensitization takes several weeks to develop. In chronic or recurrent
cases, however, it is well worth trying, for in these, unfortunately, time is
not measured by weeks, but by months and sometimes by years.
Atropine is usually exhibited as drops (1 or 2%) or an ointment, but if it is not
successful in dilating the pupil, a more concentrated form may be used, such as the
dusting of a few grains of powder or the insertion of a crystal into the conjunctival
sac. The addition of 2% cocaine to the solution also aids absorption, as also may the
addition of 5 to 10% dionine ; while the former, if anything, increases the patient’s
comfort, the latter cannot often be easily tolerated unless it is combined with holocaine
(2%) in which case its lymphogogue and analgesic effect may be useful. A more
effective method, which is especially applicable when adhesions have already formed
* Vol. II, p. 1681. * Vol. II, p. 1685.
2210 TEXT-BOOK OF OPHTHALMOLOGY
and require to be broken down, is to administer it as sub-conjunctival injections, when
it may be used in doses of 2 minims of a 1 in 200 solution (Perrin, 1929). A final
means by which a powerful action may be attained is by driving the drug into the
eye by iontophoresis (atropine sulphate 1 in 1,000 solution) (Rochat, 1929; de Grósz,
1936; Erlanger, 1936; and others).
The effect of atropine may well be augmented by adding cocaine and adrenaline
to the injected solution so that a synergic summation of effects is obtained by stimulating
the dilatator in addition to paralysing the sphincter. Adrenaline in some form
or other may be given alone [either as the ordinary solution of the hydrochloride
saturating a wick of cotton under the lids (Gradle, 1925), or as epinephrine bitartrate
(Thiel, 1924; Cordes and Harrington, 1935 ; Horner and Bettman, 1936) and so on],
but the most powerful effect is obtained in combination with atropine. Rodin (1926–27)
recommended atropine (2 m. of 2% solution) and adrenaline (4 m. of 1 in 1,000).
Newton (1935) used a sub-conjunctival injection of 2 m. each of 4% cocaine, 1%
atropine and 1: 1,000 adrenaline in 10 m. saline. Hamburger (1924–27) introduced,
under the trade name of lavo-glaucosan, a mixture of lavo-adrenaline combined
with methyl-amino-aceto-pyrocatechol, which is used as drops instilled into the
conjunctival sac three to five times at intervals of 15 minutes. There is a rapid and
maximal dilatation of the pupil of sufficient power to break down synechiae which would
be untouched by atropine, and—a point of importance where secondary glaucoma
has to be considered—little tendency to raise the intra-ocular pressure (Duke-Elder
and Law, 1929). The effect, however, is transient, and if a continued dilatation is
required, this drug should be combined with atropine.
The most easily used and best example of mydriatic synergy, however, of which
I have had experience is the mixture of atropine, cocaine and adrenaline which was
evolved at Moorfields Hospital under the name mydricain (Flynn, 1933). It is a
solution of atropine sulphate gr. 1/60, cocaine HCl gr. 1/10, laevo-adrenaline HCl
gr. 1/600, mixed with sodium chloride gr. 1/80 and chlorbutal gr. 1/120 in 5 m. sterile
water, and is used as a sub-conjunctival injection. This drug is extremely efficient
in producing a maximal mydriasis in an acute case, a condition which can be maintained
by atropine, and is capable of breaking down synechiae which have resisted other
methods; moreover, it has the advantage that it does not seem to cause atropine
irritation even in susceptible subjects.
Heat is of great value particularly in the treatment of inflammation of
the anterior segment of the uveal tract, a fact recognized universally since
its enthusiastic advocation by v. Graefe (1860), not only because of the
great comfort it usually gives to the patient, but also because of its thera-
peutic value in increasing the blood-flow and inducing hyperaemia. It may
be given as repeated bathings with hot water, which is most easily carried
out with the aid of a wooden spoon carrying cotton wool, or with less trouble
and continuously by a Japanese muff-warmer or an electric heater, applied
over a gauze pad to the closed lids by a bandage (Maddox, 1903, and others).
Some patients obtain more relief from dry heat, and others from moist ; and
it is frequently a good plan to alternate the two, keeping the dry heat going
all day and giving periodical hot bathings. A third method of applying heat,
and one which in severe cases may be more effective, is by medical diathermy,
either of the long-wave but preferably of the short-wave type, and in
recalcitrant or in very acutely congested cases, the intense and penetrat-
ing heat which may be generated in this way may be of the utmost value,
DISEASES OF THE UVEAL TRACT 221 1
both in controlling the inflammation and relieving pain (Cumberbatch, 1927;
Monbrun and Casteran, 1929; Poos, 1936; Bergler, 1936; Phillips, 1939,
and others). By this means a temperature of 42°C. can safely be induced in
the anterior chamber (Moncrieff, Coulter, and Holmquest, 1932), but care
should be taken in cases of hypopyon or hyphaema that the associated
congestion may not increase the exudative response to an embarrassing
degree. There are a few patients, especially in the acute phases of
inflammation, who appear to be unable to tolerate heat of any kind, and in
these the cold produced by ice-packs laid upon the eye sometimes produces
relief more quickly than anything else.
In the relief of pain heat is usually the most efficient remedy, probably
moist heat more frequently than dry. If pain and congestion are severe,
however, leeches applied to the temple close to the outer canthus, can rarely
be bettered for the rapidity of the relief which they produce ; in fact, in
recalcitrant cases which resist other forms of treatment their effect in
reducing the inflammation and promoting comfort is frequently quite
dramatic. Although perhaps somewhat more repulsive in anticipation than
they are in actual fact, they are certainly much more efficient than any
kind of artificial leech. Apart from these local measures, analgesics, such as
aspirin, salicylates or related compounds, usually suffice, although some-
times resort must be had to derivatives of the morphia group, such as
veganin, dilaudid, and so on. Finally, one of the most important factors in
ensuring comfort is the avoidance of bright light—a consideration which is
also of therapeutic value ; to remain in a dark room is unnecessary, but
shaded illumination is advisable and, in addition, the eyes should be shielded
by dark glasses.
Sub-conjunctival injections have been advocated in many quarters since the
time of Rothmund (1866) in the treatment of different types of uveitis, particularly
the chronic forms, and especially when the choroid is affected, with a view to hastening
resolution and absorbing vitreous opacities. In some cases the object is to introduce
substances into the eye, in others to cause an irritative hyperamia, and in others
again to withdraw material from the eye by osmosis (Wessely, 1900–03; Hertel,
1910; Kochmann and Römer, 1914). For the latter purpose saline as a 5% or
stronger solution has been much employed, but it is difficult to see why the con-
centrated solution should not obtain Osmotic dilution from the neighbouring orbital
tissues rather than through the Sclera from the eye ; and I, at any rate, have never
seen any value result from its use. One is tempted, in fact, to ascribe the popularity
of the method in some countries to the sense of drama it introduces in impressionable
minds. A more irritative effect is produced by drugs such as the bichloride
(1 : 2,000–4,000) or the cyanide of mercury (1 : 5,000) (Darier, 1891; Motais, 1891;
Deutschmann, 1894; and others), and a host of other substances. Iodine has had
a considerable vogue, first as potassium iodide (Gallemaerts, 1893), but a more
dramatic effect is claimed by some writers (Cowan and Jordan, 1935; Jensen, 1937)
from Pregl's solution (a complex hypo-iodite preparation) which, when injected in
doses of from 5 m. to 1 c.c., induces a considerable local reaction and liberates
nascent iodine in contact with a weak acid medium as is constantly found in
2212 TEXT-BOOK OF OPHTHALMOLOGY
inflamed tissue. It has been particularly recommended in the acute types of
suppurative lesion, especially in exogenous inflammations, post-operative or traumatic.
Local radiation to the eye has also had advocates, but the results rarely come up
to expectations. Ultra-violet light is of some value in tuberculous cases which have
iridic nodules (Flemming and Krusius, 1911; Koeppe, 1916; Gilbert, 1917; Seidel,
1917; Passow, 1921–25; Ascher, 1922; Birch-Hirschfeld, 1922), but any effect is
by no means uniform (Duke-Elder, 1927). Radium or X-rays have been employed
intermittently, although with no outstanding success, for acute (Darier, 1903),
chronic (Koster, 1911), and especially tuberculous uveitis (Koster, 1911; Koster and
Cath, 1911; Cuperus, 1914; Flemming, 1913–14; Fleming, 1925; Greeves, 1930;
Foy, 1939; and others). .
Surgical diathermy may occasionally be useful in destroying a localized lesion,
as, for example, a tuberculoma of the choroid (Weve, 1935–39).
GENERAL TREATMENT
General treatment, apart from specific remedies for particular diseases
(syphilis, tubercle, etc.) which will be discussed in subsequent sections, has
three particular functions—to assist in the elimination and neutralization
of toxins, to provide any assistance which drugs may offer to combat the
infection, and to stimulate the patient’s resistance. To obtain these ends,
however, the most important single necessity is rest in the acuter stages and
the avoidance of fatigue in the more chronic stages of the malady. Rest
includes not only that of the eyes as obtained by atropine, the avoidance of
visual effort and of glare, but bodily rest as well, and it is frequently astonish-
ing how often an acute or sub-acute inflammation which is not doing
well will at once become amenable if the patient is merely sent to bed without
other change of treatment, a phenomenon which occurs particularly with the
hospital type of patient, for whom rest at home is usually difficult. In the
more chronic stages of the disease it may be advisable to attain the same
ends by some type of spa and climatic treatment, when the patient has the
advantages of waters, eliminative measures, baths, regulated diet and
exercise, and mild discipline in pleasant circumstances. In tuberculous
cases and frequently in chronic cases of infective origin, if the stimulatory
effects of sunshine and climate cannot be obtained, ultra-violet light baths
afford a second-best substitute (Duke-Elder, 1926–28 ; Goulden, 1926;
MacCallan, 1928; Law, 1934). -
Eliminative treatment is best instituted by a smart saline purge, and
throughout the illness great attention should be devoted to the bowels. In
cases of recalcitrant constipation high colonic lavage is often effective ;
as is calomel in small doses (gr. 3 t.d.s., or oftener) both as a laxative
and as an intestinal antiseptic. The kidneys can be made to do their share
by the prescription of an abundance of bland fluid to be drunk, while a
further valuable method of elimination is through the skin by the induction
of periodic profuse sweating by hot-air baths augmented, if necessary, by
sub-cutaneous injections of pilocarpine (1/7 to 1/5 gr.).
DISEASES OF THE UVEAL TRACT 2213
Drugs are frequently a most useful adjuvant in treatment. When the
infection is known and a specific remedy exists to counteract it, its use is
obvious, but apart from a few instances, as arsenic in syphilis, sulph-
anilamide in acute streptococcal, gonococcal, or B. coli infections, such an
occasion does not frequently arise.
Of drugs which may be of value from the general point of view, the most
important and most useful is salicylate, especially in the acute or sub-acute
stages of the disease. In the more chronic types of inflammation the heavy
metals are sometimes of value. The classical remedy is mercury, given either
by mouth or inunction, especially in exudative types of inflammation ; gold is
also frequently of value, administered intravenously, and arsenic in non-
syphilitic as well as syphilitic lesions. In the later stages iodine should not
be forgotten, and it is well combined with small doses of thyroid, which
probably acts by stimulating a metabolism rendered sluggish by toxins; and
in this association the advantage of an abundance of vitamins in the food
and as pharmacological concentrates should not be overlooked. It is
frequently striking how a chronic or recurrent inflammation which tends to
go on indefinitely may clear up rapidly on the administration of thyroid
extract and vitamin concentrates, not necessarily because these substances
are deficient, although such a state may have been brought about by a
continuance of the infection, but because they stimulate the patient to
mobilize his own defences.
Sulphanilamide Compounds. Of recent years a therapeutic agent of very
great power has been introduced in the drugs of the sulphanilamide group, which
although first brought out against the streptococcus, are effective against many
organisms, such as the gonococcus, the pneumococcus, and B. coli (Whitby, 1938, and
others). Their action, which appears to depend on the para-position of an amino
radical, is not yet understood : they are not bactericidal nor antigenic, nor do they
stimulate resistance through the reticulo-endothelial system or by phagocytic activity;
they are bacterio-static, arresting the multiplication of organisms and probably killing
them by interfering with their utilization of their pabulum. They are highly diffusible,
appearing rapidly after administration in all body-fluids and are quickly eliminated,
so that their concentration must be maintained by repeated dosage. Mengel (1939)
and Pinkhof (1939) have found them in high concentration in the aqueous humour
after administration by mouth and also in considerable concentration in the vitreous,
the concentration in the aqueous in rabbits being equal to that in the blood, and
the concentration in the vitreous varying from 25 to 35% of this ; it is interesting
also that the intra-ocular concentration is higher after oral administration than after
instillation into the conjunctival sac. The ocular effects were demonstrated experi-
mentally by Rambo (1938) who showed that, whether given orally or injected into
the eye, their administration overcame an infection due to an injection of haemolytic
streptococci into the anterior chamber of rabbits, but was only able to delay the
destruction of the eye when a similar culture was injected into the vitreous. It is
obvious that such drugs are of extreme value in all types of uveitis from pan-
ophthalmitis to a relatively mild iritis, and their effects are frequently most dramatic,
particularly in streptococcal, pneumococcal and gonococcal conditions.
The Salicylates are one of the most useful drugs which can be given systemically,
2214 TEXT-BOOK OF OPHTHALMOLOGY
especially in acute and sub-acute inflammations. Their mode of action is unknown.
It has been demonstrated by S. R. Gifford (1922) that their presence can be detected
in the aqueous humour 25 minutes after their oral administration by mouth in rabbits,
and that in 40 minutes they may reach a concentration therein of 1 : 1,250. This
may be increased further by the use of dionine and hot packs (Whitham, 1913). It
is probable that their action is not directly bactericidal (Hanzlik, 1921), but that the
inflammatory changes caused by the infection are rendered less severe by them is
undoubted, while general effects of considerable importance are evident, such as an
increase of heat elimination resulting in a lowering of temperature (Barbour, 1919–21).
The best effects in acute cases are obtained when the drug is administered in large
doses, and those originally suggested by H. Gifford (1900) of 1 gr. per lb. of body-
weight per day achieve the best result if they can be borne. After 4 or 5 days this
may be cut down to 60 gr. a day, and this can be well continued until some days after
the symptoms have subsided. In such doses Some nausea and buzzing in the ears
may be evident, but need not necessitate discontinuance of the drug unless they
become troublesome ; nor need a transient albuminuria provided the kidneys are
healthy, a point which should be ascertained beforehand. If vomiting occurs the
same dosage may be given rectally, but the intravenous route (Black, 1924) seems
unnecessary in view of the rapidity with which the drug reaches the eye from the
alimentary tract. If, however, vomiting or toxic symptoms become troublesome,
some of the substitutes for salicylates may be tried—cinchophen or neocinchophen.
Gold is administered intravenously, usually as sodium gold thiosulphate, in
doses starting with 10 and increasing by 5 mgm. to a maximum of 50 mgm. 2 or 3 times a
week. Its action is slow and 25 injections form a minimum course (Benedict and
Goeckerman, 1932); but it is to be remembered that some patients become sensitive
to it, and the earliest onset of toxic symptoms (diarrhoea, skin-rashes, Oedema, etc.)
is an indication for its immediate stoppage. Its action is not understood ; it is
probably not bactericidal, but stimulates non-specifically the defence-mechanism
through its effect upon the capillaries. It has been advocated for tuberculous lesions,
and is sometimes found efficacious in the very chronic type of uveitis associated with
arthritis, in which condition also it is frequently of value.
Arsenic probably acts in a somewhat similar manner—not directly on the organism
concerned, but possibly by the elaboration of some non-specific substance in the
tissues. Apart from syphilitic lesions, where its effect is often dramatic, it is
frequently of value given as a course of intravenous injections (as neoarsphenamine,
etc.) in cases of chronic uveitis of doubtful origin and even in sympathetic ophthalmitis
(Benedict and O’Leary, 1922; Palich-Szanto, 1922; Elewaut, 1924; Lundsgaard,
1925; Browning, 1926; Lucic, 1936; and others).
Calcium in the form of the gluconate has been recommended as an intravenous
or intramuscular injection, since it is claimed to produce a rapid amelioration of pain
and inflammation (Stastnik, 1932; Theobald, 1933; and others). Weekers (1936)
suggested calcium chloride (3 gr. daily for 20 to 30 days), it being held that calcium
has an inhibiting effect on the production of transudates, diminishes the permeability
of the vessels and the excitability of the sympathetic vasomotor nerves. Sodium
thiosulphate injected intravenously has also been recommended particularly in cases
when a plastic exudate is prominent.
Stimulation of the mechanism of immunity is frequently possible by
specific measures, such as vaccines and Sera, but in the many cases wherein
the aetiology cannot be definitely established and in acute cases where delay
is contra-indicated, non-specific measures may be adopted by utilizing
general protein shock. How the foreign protein produces its therapeutic effect
DISEASES OF THE UVEAL TRACT 2215
is unknown, but that events of considerable importance occur is seen in the
severe general reaction which follows, characterized particularly by acute
malaise and fever. There is a general leucocytosis and an increase of anti-
bodies in the blood (Petersen, 1922), and in the eye itself, especially in the
ciliary body, there follow a considerable degree of vaso-dilatation, a lympho-
cytosis, and a diffuse cellular infiltrate (v. Szily, 1930), while the anti-body
content of the aqueous is increased (Brown, 1935). It would seem probable,
however, that the main effect is a general one whereby the whole mechanism
of immunity receives a fillip which stimulates it to greater activity. In
uveitis the main field of value of this method of therapy is at the commence-
ment of acute cases, especially exogenous infections following perforating
wounds, when it may have an abortive effect, and in old-standing chronic
and relapsing inflammations in cases where the patient's resistance seems to
have lost control of the situation. In these cases the immediate effect may
be dramatic, and, although it is sometimes not sustained, in an unequal or
equilibrated contest the balance may be tipped over to the side of recovery.
In all cases, however, some consideration must be given to the general
condition of the patient for these methods of treatment are drastic, involving
considerable stimulation and much evident reaction, and they may not be
easily or safely borne.
The two most favoured substances employed to produce protein shock are whole
milk and typhoid vaccine.
Milk, which was originally employed for fever therapy by Schmidt (1916) and
Saxl (1916), is given intra-gluteally, usually in an initial dose of from 5 to 10 c.c.,
increasing every 2 or 3 days, depending on the reaction produced ; it is as well not to
give a subsequent dose until the temperature has been normal for at least 12 hours,
or until the leucocytosis has disappeared. It is to be remembered that the shock-
producing qualities of milk are increased if it is bacteriologically unclean, and it is best
to use a sample some days old which has been boiled for 4 minutes and allowed to cool.
While by no means universally applicable, in many cases which hitherto had been
very recalcitrant its effect has been favourable (Müller and Thanner, 1916; Uddgren,
1918; Purtscher, 1920; Igersheimer, 1921; Gaston, 1926; Muskat, 1928). In
order to avoid the discomfort and fever of the reaction, certain milk substitutes have
been advocated—lactigen, casein (Lindig, 1919 ; Müller, 1919), aolan (Jickeli, 1925–28)
—but since the beneficial effect of this method of therapy seems directly related to
the discomfort it causes, their use seems illogical.
Typhoid vaccine is more dramatic in its effects on uveal inflammation, but more
drastic in the general symptoms it causes, producing a high temperature of 102° to
105° F. with rigors and general malaise : it is therefore more suited for younger
patients (Allen, 1925; Howard, 1927–28 ; and many others). It is given intra-
venously, an average initial dose being 25 million organisms, while subsequent doses
are prescribed, increasing in strength up to 100 million depending on the previous
reaction, allowing 24 hours normal temperature between each.
Other bacterial antigens have been employed from time to time (Mason, 1928),
among which should be mentioned Coley's fluid (toxins of the killed cultures of strep.
erysipelatis and B. prodigiosus) (Levine, 1931–35). Anti-diphtheritic serum has its
advocates (Key, 1924–26 ; Verhoeff, 1927; Cobb, 1933), while auto-servm therapy—
the intra-muscular injection of 10 c.c. of blood removed from a vein—is claimed to
2216 TEXT-BOOK OF OPHTHALMOLOGY
give good results without a violent febrile response (Wick, 1926–27 ; Guiral and
Guiral, 1930; and others).
Meesmann (1936) suggested intra-cutaneous injections of bee venom at daily
intervals (0.1 c.c. apicosan), claiming good results in iritis of the “rheumatic ’’ type.
As an alternative to protein shock a high temperature may be induced
by artificial fever therapy as produced by diathermy, or one of the several
hyperthermal cabinets which are now in use, particularly in America. By
this means a rectal temperature of 105° F (40°C.) can be reached or ex-
ceeded, easily and relatively comfortably and, it is maintained, the immune
mechanism stimulated thereby. Good results have been claimed for this
method by Whitney (1935) and Weeks and Morris (1938) in acute infective
iritis and by Cullen and Simpson (1936) in syphilitic uveitis.
It is to be remembered that many authorities—and with considerable
reason—maintain that much of the effect of vaccines is non-specific, in which
case they act in a sense as a mild degree of protein shock. Particularly is
this true of tuberculin, and it is probable that in many cases the beneficial
effects of tuberculin therapy, especially in chronic cases, is due to this
effect rather than to a specific reaction upon a tuberculous process.
SURGICAL TREATMENT
Of the surgical procedures which may be adopted in inflammations of
the uveal tract, the simplest is paracentesis, a method first employed by
Wardrop (1808) and advocated by Desmarres (1847). Apart from its
employment in cases of raised tension, or in cases wherein the size of a
hypopyon is becoming embarrassing, a simple paracentesis frequently has a
good effect, partly by producing a maximal hyperaemia (Dianoux, 1898;
zur Nedden, 1906; Wessely, 1906), and partly by flooding the chambers of
the eye with a plasmoid aqueous rich in immune bodies (Peters, 1908;
Schirmer, 1908; and others). The paracentesis can, of course, be repeated
or re-opened on subsequent days, a method of treatment which may be of
considerable value in the treatment of chronic irido-cyclitis with precipitates
(Grunert, 1922; Hamburg, 1924). In suppurative inflammations of the
anterior segment of the eye, it has been recommended that, in addition to
the paracentesis, the anterior chamber be washed out : Denig (1908) used
saline, and antiseptics, such as bichloride of mercury or hydrogen peroxide,
may be employed. As a rule, however, such procedures are disappointing
in their results. -
A further suggestion was made by Schieck (1933) in that aqueous be drawn off
and the anterior chamber be filled with the patient’s own blood freshly drawn from the
arm (auto-hoemotherapy). Since Schieck's original enthusiastic reports in cases of
tubercle, many writers have claimed good results with this treatment : the blood
remains in the anterior chamber for 2 or 3 days and as it is absorbed, in successful
cases, the keratic precipitates are seen to tend to disappear, iridic nodules to diminish,
and synechiae to be torn away. Most writers have employed the method in tubercle
(Baer, 1933; Moreu, 1933; Kyrieleis, 1934; Serr, 1934; Krasnow, 1934; Mata,
DISEASES OF THE UVEAL TRACT 2217
1935; Rollet, 1935; and others); good results may, however, accrue in cases where
tubercle does not seem to enter into the question ; but unfortunately the effect may
be only temporary. The rationale of such treatment is not quite clear. Presumably
it depends to a small extent on the introduction of anti-bodies with the blood, partly
on the local irritative and hyperaemic effect, but to a large extent, acting as a local
protein shock, the injection stimulates the protective and immunological functions of
the tissues. For these reasons the method of treatment is indicated in chronic rather
than in acute conditions, for in the latter stimulatory measures may be harmful.
An iridectomy has been advocated as a therapeutic measure in chronic,
and particularly recurrent, irido-cyclitis. Undoubtedly it sometimes has a
beneficial influence, but more usually the effect is disappointing, and the
operation should not be employed unless it is indicated on other grounds,
such as raised tension. In all cases every endeavour should be made to
operate in a quiet interval when active signs of inflammation are absent, for
in an acute phase a superimposed traumatic iritis will almost certainly make
matters worse, the operative coloboma may fill up with exudate, and an
intractable haemorrhage, which will absorb only slowly and with difficulty,
may result. The presence of fresh keratic precipitates, any suspicion of a
lowering of the tension, or the presence of much thickening or atrophy of
the iris are contra-indications. It is true that even in those circumstances
excellent results are occasionally achieved, but more often it is found that
the diseased eye will not tolerate operative procedures, and the final result
may well be a malignant exacerbation of the inflammation, or, especially
when the eye is soft, intractable haemorrhages and shrinkage of the globe.
Circumstances do arise, however, when an iridectomy is indicated : to
forestall the formation of ring synechiae, to establish communication between
the posterior and the anterior chambers when these have formed, to make an
artificial pupil when vision is destroyed by the occlusion of the pupillary
aperture with exudate, and, above all, for the relief of raised tension. The
first indication is important, for once total posterior synechiae have formed
and the iris has become atrophic, friable and matted down to the organized
exudate on its posterior surface, an iridectomy is a difficult operation at best
and is rarely successful. In these circumstances a timely iridectomy,
choosing that portion of the iris for excision which is least changed and
least bound by adhesions, may forestall eventual destruction of the eye and
ultimate loss of vision. Where complete ring synechiae have formed and
especially when a condition of iris bombé develops, an iridectomy or an
iridotomy is essential ; and the same applies when tension becomes
uncontrollably high.
In these circumstances an iridectomy may not be possible owing to the
difficulty of introducing a knife into an anterior chamber almost obliterated by an
iris bombé. In this event a transfia-ion of the iris as suggested by Fuchs (1896) may be
attempted. In this operation a slender Graefe knife is thrust through the cornea at
the temporal limbus, and threaded through the bulging iris in a straight linemaking four
punctures on its way, until it makes a counter-puncture at the opposite limbus. The
22 18 TEXT-BOOK OF OPHTHALMOLOGY
four iridotomies thus made usually establish communication between the two chambers
so that the iris becomes flattened, allowing an iridectomy to be performed in more
favourable circumstances in a few days' time before the small openings have become
closed with exudate.
An alternative procedure, and one which is preferable in most cases, is to cut
into the anterior chamber at the limbus under a conjunctival flap, the perforation
being made gradually by repeated incisions from the outside, and then when the iris
presents to perform an iridectomy. This idea was introduced by Gayet (1884), Dianoux
(1884) and Dehenne (1888), and the technique was further elaborated by Elschnig
(1928), Lundsgaard (1928), Foroni (1928) and Salzmann (1930), the last of whom
called the operation iridectomia ab eacterno. Such an operation is applicable to most
cases in which an iridectomy should be done, is simple to perform, it can be done
when the shallowness of the anterior chamber renders the classical operation impractic-
able, and is usually efficacious in its results (Horner, 1936). -
Finally, when all ameliorative measures have failed and the eye is
shrinking or hard and painful, recourse may be had to a retro-bulbar injection
of alcohol (Grüter, 1918; Jaensch, 1925; Weekers, 1936), or the final resort
of evisceration or eaccision performed.
A word about the difficult problem of THE CONTROL OF THE MOST
UNPLEASANT COMPLICATION.—A RISE OF TENSION. Two distinct conditions
arise—the increase in tension occurring in the active stages of an irido-cyclitis,
and that resulting from the structural sequelae of this condition. The best
way to deal with a secondary glaucoma of the first type is to combat the
irido-cyclitis which produces it, and if structural changes have not developed
to obstruct the circulation of fluid in the eye, it is surprising how frequently
this can be done. In the acute phase, therefore, every chance should be
given to medicinal treatment, but what this treatment should be has excited
no little controversy. Frequently a very difficult dilemma presents itself as
to whether the irido-cyclitis should be treated with mydriatics or the
glaucoma by miotics. Some authorities would consistently give mydriatics
in every (or nearly every) case (Larsen, 1924; and others), others would be
equally indiscriminate with miotics (Malling, 1923; Hagen, 1925; and
others), while others again are inconstant in their allegiance to either
(Greeves, 1928; Lawson, 1928; and others). To legislate on a condition in
which the picture may change from day to day, and in which each case must
be considered on its merits is impossible, but on the whole, where it is
practicable, atropinization gives the best results. Certainly in those cases
when the inflammation is acute and when the anterior chamber is deep, the
urgent necessity is to establish dilatation of the pupil with the attendant
decongestion of the anterior segment of the uveal tract and the breaking
down of existing synechiae. If this can be achieved reduction of the hyper-
tension usually follows. The best method to attain it is by atropine in
massive doses, a course which frequently requires courage. In these
circumstances many authorities advise the exhibition of eserine in the hope
of lowering the tension and then resort to atropine, but as a general rule
DISEASES OF THE UVEAL TRACT 2219
miotics tend to have an unfavourable influence upon irido-cyclitis, irritating
the ciliary and iridic musculature, aggravating congestion, increasing the
formation of exudates, and favouring the production of adhesions. It seems
preferable in this type of case to remain logical throughout, to push atropine,
preferably combining it with adrenaline and cocaine as a sub-conjunctival
injection to attain synergic mydriasis, and control any dangerous initial rise
of tension by repeated paracenteses. Such treatment is, of course, to be
combined with the most active local and general medical measures, leeches,
heat, cathartics, massive doses of Salicylates, and so on. Such a method may
not be without its anxieties so that it frequently requires hospitalization and
constant observation until equilibrium has been established, but for the
general treatment of a serious condition in its acute stages this is not at all a
bad thing and is usually followed by the most satisfactory results.
If such a course does not succeed in lowering the tension or in those
cases where the anterior chamber is shallow and where the anatomical
configuration raises suspicions of a tendency to glaucoma, atropine may make
matters worse. Some of these latter cases do well on miotics, but as a general
rule it is best to resort to operative measures, a course which should usually
be adopted if the anterior chamber is shallow and the irido-cyclitis is quiet.
As a general rule, therefore, if the clinical appearance indicates that the rise
of tension is due to active inflammation, particularly if the anterior chamber
is deep, atropine should be given a persistent trial ; if the appearance suggests
that the primary cause is embarrassment at the filtration angle, operation
should be considered, which should take the form of a large trephining or
some other fistulizing procedure.” When the raised tension is due to obvious
anatomical changes, such as pupillary synechiae, early operation is, of course,
necessary, an iridectomy being then indicated ; and also in the late cases
where tension develops slowly and insidiously some long time after the acute
inflammation has passed, presumably owing to changes at the angle of the
anterior chamber, a fistulization operation should again be done. These
Questions will be discussed in more detail when dealing with secondary
glaucoma.
Allen. T. Sect. O., Am. Med. AS., 135, 1925.
Ascher. K. M. Aug., lxvii, 619, 1921.
IBaer. K. M. Aug., xc, 485, 1933.
Barbour et al. A. Int. Med., xxiv, 617, 624,
1919.
J. Pharm. and Earp. Ther., xviii, 165, 1921.
Benedict and Goeckerman. A. of O., viii,
250, 1932.
Benedict and O’Leary. T. Pacific Coast O. S.,
44, 1922.
Bergler. Z. f. Aug., xc, 6, 1936.
Birch-Hirschfeld. 100 Vers. deut. Natur-
forsch. w. Aertze. Leipzig, 1922.
Black. Am. J. O., vii, 773, 1924.
Brown. T. Am. O. S., xxxiii, 435, 1935.
Browning. T. O. S., xlvi, 249, 1926.
Cobb. J. Iowa Med., S., xxiii, 250, 1933.
Cordes and Harrington. Am. J. O., xviii,
451, 1935.
Cowan and Jordan. Pennsylvania Med. J.,
xxxviii, 704, 1935.
Cullen and Simpson. A. of O., xv, 625, 1936.
Cumberbatch. Diathermy. London, 1927.
Cuperus. A. f. Aug., lxxvii, 1, 1914.
Darier. A. d’O., xii, 449, 1891.
La clin. Opht., ix, 315, 1903.
Dehenne. An. d’Oc., c, 120, 1888.
Denig. O. Rec, xxvii, 119, 1908.
A. of O., xiv, 860, 1935.
1 p. 3402.
2220
TEXT-BOOK OF OPHTHALMOLOGY
Desmarres. Traité theoretique et pratique d.
maladies d. yewar. Paris, 1847.
Deutschmann. Beit. z. Aug., xv, 73, 1894.
Dianoux. Bull. S. fr. d’O., ii, 44, 1884.
Am... d’Oc., exx, 248, 1898.
Duke-Elder. Brit. Med. J., i, 891, 1926.
T. O. S., xlvi, 213, 1926 ; xlviii, 264, 1928.
Brit. J. O., xi, 67, 1927; xii, 289, 353, 1928.
Duke-Elder and Law. Brit. Med. J., i, 590,
1929.
Elewaut. A. med. Belges, lxxvii, 356, 1924.
Elschnig. K. M. Aug., lxxx, 382, 1928.
Erlanger. Brit. J. O., xx, 213, 1936.
Fleming. Am. J. O., viii, 730, 1925.
Flemming. A. f. O., lxxxiv, 345, 1913.
Strahlentherapie, iv, 681, 1914.
Flemming and Krusius. B. O. G. Heidel.,
xxxvii, 107, 1911.
Flynn. Brit. J. O., xvii, 298, 1933.
Foroni. Am. di ott., lvi, 82, 1928.
Foy. T. O. S., lix, 458, 1939.
Fuchs. B. O. G. Heidel., xxv, 178, 1896.
Gallemaerts. Bull. Acad. roy. de Méd. Belge,
vii, 490, 1893.
Gaston. Am. J. O., ix, iii, 1926.
Gayet. Bull. S. fr. d’O., ii, 41, 1884.
Gifford, H. J. Amer. Med. As., xxxiv, 341,
1900.
Gifford, S. R. Am. J. O., v, 948, 1922.
Gilbert. A. f. Aug., lxxxii, 179, 1917.
Goulden. T. O. S., xlvi, 207, 1926.
Gradle. J. Am. Med. As., lxxxiv, 675, 1925.
v. Graefe. A. f. O. iii (2), 180, 1860.
Greeves. T. O. S., xlviii, 45, 1928; 1, 11 1,
1930.
de Grósz. A. d’O., liii, 25, 1936.
Grunert. B. O. G., Jena, xliii, 178, 1922.
Grüter. B. O. G. Heidel., xli., 85, 1918.
Guiral and Guiral. A. de Oft. H.-A., xxx, l,
1930.
Hagen. A. f. O., exv, 521, 1925.
Acta O., iii, 96, 1925.
Hamburg. Z. f. Aug., liii, 55, 136, 1924.
Hamburger. K. M. Aug., lxxii, 47, 541, 774,
1924; lxxiv, 150, 1925; lxxvi, 27, 400,
546, 1926; lxxix, 232, 1927.
Z. f. Aug., liii, 127, 1924; lv., 109, 112, 1926.
A. of O., lv, 533, 1926.
Hanzlik et al. J. Am. Med. As., lxxvi, 1728,
1921.
Hertel. Z. f. Aug., xxiii, 73, 1910.
Horner, A. of O., xv, 70, 1936.
Borner and Bettman. Am. J. O., xix, 311,
1936.
Howard. China Med. J., xli., 395, 1927.
Am. J. O., xi, 685, 1928.
Igersheimer. Therap. Halbmonatsch., xxxv,
104, 1921.
Jaensch. Z. f. Aug., lviii, 2, 1925.
Jensen. North West Med., xxxvi, 247, 1937.
Jickeli. K. M. Aug., lxxv, 434, 1925; lxxx,
221, 1928.
Rey. J. Am. Med. As., lxxxii, 183, 1924.
Am. J. O., ix, 351, 1926.
Rochmann and Römer. A. f. O., lxxxviii,
528, 1914. -
Roeppe. A. f. O., xcii, 115, 1916.
Roster. Ned. tij. v. Gen., ii, 633, 702, 1911.
Strahlentherapie, iii, 583, 1913.
Koster and Cath. Ned. tij. v. Gen., ii, 633,
702, 1911.
Krasnow. Sov. vestm. Oft., v, 222, 1934.
Ryrieleis. Z. f. Aug., lxxxv, 16, 1934.
Larsen. A. f. O., czv, 144, 1924.
Law. Ultra-violet Therapy in Eye Disease.
London. 1934.
Lawson. T. O. S., xlviii, 60, 1928.
Levine. A. of O., vi, 75, 1931; xiv, 554, 1935.
Lindig. Münch. med. W., lxvi, 921, 1919.
Lucic. A. of O., xv, 826, 1936.
Lundsgaard. Acta O., ii, 189, 1925; vi, 311,
478, 1928.
MacCallan. T. O. S., xlviii, 287, 1928.
Maddox. O. Rev., xxii, 52, 1903.
Malling. Acta O., i, 97, 345, 1923.
Mason. Am. J. O., xi, 702, 1928.
Mata. A. de Oft. H.-A., xxxv, 597, 1935.
McMullen. T. O. S., xlviii, 51, 1928.
Meesmann. K. M. Aug., xcvii, 727, 1936.
Mengel. A. of O., xxii, 407, 1939.
Monbrun and Casteran. La haute frequence
en Ophtalmologie. Paris, 1929.
Moncrieff, Coulter, and Holmquest.
J. O., xv, 194, 1932.
Moreu. A. de Oft., H.-A., xxxiii, 699, 1933.
Motais. Rev. gen. d’O., x, 245, 1891.
Müller, E. F., Münch. med. W., lxvi, 1233,
1919.
Müller, L. Wien. kl. W., xxxi, 933, 1918.
Müller, L., and Thanner. Med. Kl., xii, 1120,
1916.
Muskat. Am. J. O., ii, 539, 1928.
z. Nedden. B. O. G. Heidel., xxxiii, 215, 1906.
Newton. A. of O., xiv, 618, 1935.
Palich-Szanto. K. M. Aug., lxix, 799, 1922.
Passow. Strahlenther., xii, 441, 1921.
A. f. Aug., xc, 123, 1921 ; xciii, 95, 1923;
xciv, i, 1924 ; xcvii, 74, 1925.
Perrin. A. d’O., xlvi, 672, 1929.
Peters. K. M. Aug., xlvi, 456, 1908.
Petersen. Protein. Therapy and Non-specific
Resistance. N.Y., 1922.
Phillips. T. O. S., lix, 193, 1939.
Pinkhof. Ophthalmologica, xcvii, 356, 1939.
Poos. J. Missouri Med. As., xxxiii, 388, 1936.
Purtscher. Z. f. Aug., xliii, 100, 1920.
Bambo. Am. J. O., xxi, 739, 1938.
Am.
Rochat. Ned. tij. v. Gen., ii, 641, 647,
1912.
XIII Internat. Cong. Amsterdam, i, 199,
1929.
Rodin. Am. J. O., ix, 24, 1926.
A. of O., lvi, 277, 1927.
Rollet. A. of O., xiv, 860, 1935.
Rothmund. K. M. Aug., iv, 161, 1866.
Salzmann. Z. f. Awg., lxxii, 127, 1930.
Saxl, Wien. kl. W., xxix, 1043, 1916.
Schieck. K. M. Aug., xc, i, 1933.
Z. f. Aug., lxxxiii, 257, 1934.
Schirmer. K. M. Aug., xlvi, 456, 1908.
Schmidt. Med. Kl., xii, 171, 1916.
Seidel. A. f. O., xciii, 357, 1917.
DISEASES OF THE UVEAL TRACT 2221
Serr. B. O. G. Heidel., 1, 41, 60, 1934. Weeks and Morris. Am. J. O., xxi, 664, 1938.
Stastnik. Oft. Sbornik, vii, 216, 1932. Wessely. B. O. G. Heidel., xxviii, 69, 1900;
v. Szily. K. M. Aug., lxxxv, 21, 1930. xxxiii, 143, 1906. -
Theobald. Am. J. O., xvi, 975, 1933. A. f. eacp., Path. u. Pharm., xlix, 417, 1903.
Thiel. K. M. Aug., lxxii, 534, 1924. Weve. Ned. tij. v. Gen., i, 754, 1935.
Uddgren. Milchinjektionen in d. Ophthal. T. O. S., lix, 43, 1939.
Stockholm, 1918. Whitby. Lancet, ii, 1905, 1938.
Verhoeff. A. of O., lvi, 28, 1927. Whitham. Ophthalmoscope, xi, 71, 1913.
Waller. T. O. S., liv, 96, 1934. Whitney. J. Am. Med. As., civ, 1794, 1935.
Wardrop. Essays on the Morbid Anatomy Wick. K. M. Aug., lxxvii, 487, 1926.
of the Human Eye. London, 1808. A. f. O., cxviii, 221, 1927.
Weekers. A. d’O., liii, 166, 1936.
B. Special Types of Uveitis
I. Acute Suppurative Inflammations
In discussing the pathology of inflammations of the uveal tract 1 we
saw that as a general rule an acute suppurative process spreads rapidly
throughout the uveal tract and readily involves the whole eye in a pan-
ophthalmitis; more rarely it remains limited to the anterior or posterior
segment to constitute an irido-cyclitis with hypopyon or an acute
endophthalmitis. -
1. PANOPHTHALMITIS
A panophthalmitis is due to the arrival and settlement in the eye of
virulent pyogenic Organisms, and we have seen in our discussion of the
general aetiology of uveal inflammation that the infection may be exogenous *
or endogenous.” In the first place the organisms are introduced into the
eye through a perforating wound or ulcer ; in the second they arrive by
metastatic deposition from some infective lesion elsewhere, either a local focus
of infection, as a furuncle or abscess, or a general infective disease, as
pneumonia, puerperal fever, influenza, scarlet fever, measles, meningitis,
and so on. The organisms met with and the associated infections have
already been detailed," but on the whole it may be said that, other things
being equal, the most intense inflammations are due to exogenous infections,
perhaps because the virulence of endogenous organisms may become
attenuated from contact with anti-bodies in the blood-stream. In either
event the clinical picture produced is one of the most dramatic in ophthal-
mology, for once the infection obtains a foothold symptoms develop with
uncontrollable rapidity culminating frequently in irretrievable loss of sight
within twenty-four hours.
A typical acute case usually starts with pain in the eye and general
malaise—a rise in temperature, headache, drowsiness, and sometimes
vomiting. An oedematous swelling, frequently with some venous engorge-
ment, appears on the lids, particularly the upper, an intense conjunctival and
ciliary injection develops rapidly into an extreme chemosis, and the cornea
* p. 2159. * p. 2127. * p. 2136. * p. 2137.
2222 TEXT-BOOK OF OPHTHALMOLOGY
becomes hazy and lustreless. From an early stage a view through the hazy
cornea may be difficult; the irisis muddy and grey, the aqueous turbid, and
the posterior surface of the cornea may become rapidly plastered with pre-
cipitates, a process which may soon develop into an ever-increasing hypo-
pyon (Fig. 1846, Plate XXXIII). If at the beginning an internal examina-
tion is possible, the retinal veins are found to be dilated, white areas rapidly
increasing in size are scattered over the fundus, a vitreous haze increases
Fig. 1843–PANophth ALMITIs (Asbill, A. of 0.).
apace untileverything becomes obscured, while eventually a yellow pupillary
reflex develops due to pus in the vitreous cavity. At this stage trans-
illumination gives a uniformly diminished intensity of reflex, indicating that
the globe is filling with purulent exudate. Depending on the virulence of the
organism this stage may persist for some hours or days with the symptoms
gradually becoming more violent (Fig. 1843); the tension rises, the eye becomes
fixed and proptosed as the sclera becomes involved, the general symptoms
increase and the temperature rises, and the pain becomes more and more
unbearable until relief is obtained by surgical measures or when a perforation
of the globe occurs (Fig. 1800). In the latter case the outer coat gradually


DISEASES OF THE UVEAL TRACT 2223
becomes infiltrated until it disintegrates and finally ruptures, allowing the
escape of the pus and intra-ocular contents, a process which occurs either
through a sloughing away of the cornea after the development of a ring
abscess," or most frequently just behind the limbus and sometimes more
posteriorly. Thereupon the pain immediately subsides, and after prolonged
suppuration the end result is a shrunken and completely disorganized eye
(Fig. 1802). As a rule, such an eye shrinks to a condition of phthisis,
and, as such, usually gives rise to very little trouble.
The pathology of these cases of acute inflammation and their occasional resolution
through a sub-acute stage to organization and fibrosis is discussed on p. 2159.
The prognosis with regard to the eye and vision is almost invariably bad.
Some Organisms, such as the staphylococcus, may remain content with a
more localized but nevertheless intensely necrotic destruction, and in
the case of others, such as the meningococcus, especially in children,
the process may result in fibrosis and the development of a pseudo-
glioma,” but even then a disorganized sightless eye is the only result
to be expected. The loss of the eye is the rule in the more common
streptococcal or pneumococcal infections. In fact, the main variation in
prognosis rests solely on the rate of destruction ; but occasionally even a
virulent exogenous infection, such as the B-pyocyaneus, while usually
causing a fulminating panophthalmitis (Sattler, 1891 ; Garretson and
Cosgrove, 1927; Lanou, 1935), may assume a more chronic form and
respond to treatment
It is to be remembered, however, that in endogenous cases the patient
may already be gravely ill, and a fatal termination of an illness in which the
Ocular infection is merely an incident is by no means unknown. Thus in
streptococcal puerperal septicaemia Luttringer (1922) reported 6 cases, all
of which died, and in Axenfeld’s (1894) series of 69 cases, 66% were fatal.
On the other hand, the ocular infection may be the only obvious evidence of
systemic infection, and the patient may remain relatively well (Asbill, 1933),
and survive even if the panophthalmitis is bilateral (Hirschberg and
Henius, 1885; Fischer, 1897, and others). As a rare event an exogenous
panophthalmitis may serve as the origin for pyaemic metastases
and lead to general infection and death (Axenfeld, 1894; Gasparrini,
1895).
The treatment of panophthalmitis usually resolves itself into a question
of cutting the process short surgically, At the beginning, however, every
attempt should be made to control the inflammation. The patient should
be put to bed and purgatives and drugs for the relief of pain administered.
If it can be determined, the causal organism should be attacked by specific
methods, as by serum, or drugs (as, for example, sulphanilamide in the case
1 Vol. II, p. 1990. * p. 2168.
T.O. —WOL. III.
2224 TEXT-BOOK OF OPHTHALMOLOGY
of the streptococcus). Alternatively, protein shock by milk or typhoid
vaccine may be tried. Local treatment involves atropine, heat as hot
bathings, and leeches, while ice packs frequently give much relief. Sub-
conjunctival injections, for example, of Pregl's solution (Cowan and Jordan,
1935; Jensen, 1937) have been advocated. If, however, these measures
fail and pus becomes evident, although paracentesis or vitreous puncture
(zur Nedden, 1922) may be attempted, the more usual necessity is eviscera-
tion of the contents of the globe or excision of the eye. Of the two alterna-
tives an excision results in the most rapid healing ; but it involves two
dangers—bursting of the eye during the operation with an escape of pus
into the orbital tissues, and a spread of infection along the severed nerve-
sheaths with the possible development of meningitis—a rare event. For the
first reason as little pressure as possible should be put upon the globe during
excision, and the method is rarely possible in exogenous infections involving
a perforation. Moreover, it should not be attempted if the surrounding
orbital tissues are infected, and probably should not be when a suppurative
process which has started posteriorly has reached a fulminating stage. The
safer alternative is to open the cornea freely by a crucial excision, scoop out
the contents of the globe, and swab the interior of the sclera with
perchloride or some other antiseptic ; if there is any accumulation of
inflammatory products behind the sclera, this region should be drained.
Later, when all signs of suppuration have ceased the shrunken remains of the
globe may be excised or they may be allowed to remain.
GAS GANGRENE PANOPHTHALMITIS
The panophthalmitis caused by the B. welchii is worthy of a special
note, partly because of its special clinical features, and partly because of
the urgent necessity of radical treatment. A number of cases have now been
reported in the literature (Chaillous, 1904; Darier, 1906; James, 1910 ;
Heath, 1929; Ridley, 1929; Hamilton, 1930; Berry, 1932; Walker,
1934–38; Rieger, 1936), and in each the infection was exogenous through
perforation by a foreign body, in each a fulminating panophthalmitis
developed in which the characteristics were more than usually severe pain,
an early rise of tension, the presence of blood or of a thin coffee-coloured
discharge, and the eventual formation of gas bubbles in the anterior chamber.
These signs, particularly the last two, are sufficient to establish a diagnosis
apart from laboratory findings. Treatment consists of enucleation or
evisceration, and the administration of B. welchiiserum. It is interesting that
all the reported cases have recovered uneventfully whether the eye was
eviscerated or enucleated, healing being usually as prompt and kindly as in
uninfected cases; in no case was there any spread of the infection nor was
there any mortality—a striking contrast to the results of this type of
infection in other parts of the body.
PLATE XXXIII
Suppurative U vertis
Fig. 1844.-Suppu RAtivº IRIDo-cyclitis. Fig. 1845. SUPPURATIVE METAstatic
CHoRoibitis.
Fig. 1847-PHTHIsis BULB AFTER Fig. 1848.-Pseudo-GLIoMA AFTER
PANophthal Mitis (Neame). . Expophth ALMiris.
[To face p. 2224.


DISEASES OF THE UVEAL TRACT 2225
Asbill. A. of O., ix, 820, 1933. Hirschberg and Henius. Cb. pr. Aug., ix, 84,
Axenfeld. A. f. O., xl (3), 1 ; (4) 103, 1894. I885.
Berry. Am. J. O., xv, 1022, 1932. James. O. Rev., xxix, 161, 1910.
Chaillous. La Clin. opht., x, 328, 1904. T. O. S., xxx, 179, 1910.
Cowan and Jordan. Penn. Med. J., xxxviii, Jensen. North West Med., xxxvi, 247, 1937.
704, 1935. Lanou. Am. J. O., xviii, 950, 1935.
Darier. La Clim. opht., xii, 227, 1906. Luttringer. Gymécol. et Obstet., v, 513, 1922.
Fischer. Cb. pr. Aug., xxi, 173, 1897. z. Nedden. Uber Glaskörperabsaugung.
Garretson and Cosgrove. J. Am. Med. As., Berlin. 1922.
lxxxviii, 700, 1927. Ridley. T. O. S., xlix, 221, 1929.
Gasparrini. Am. di Ott., xxiv, 343, 1895. Rieger. K. M. Aug., xcvi, 548, 1936.
Hamilton. Brit. J. O., xiv, 452, 1930. Sattler. B. O. G. Heidel.., xxi, 201, 1891.
Heath. Brit. J. O., xiii, 574, 1929. Walker. J. Am. Med. As., cii, 1561, 1934.
A. of O., xix, 406, 1938.
2. SUPPURATIVE IRIDO-CYCLITIS
While, as we have seen, an acute suppurative inflammation in the
anterior segment of the uveal tract is usually the prelude to a general
panophthalmitis, in the less virulent types of infection the inflammatory
process may be localized and spend itself out. Sometimes it leaves
remarkably few sequelae and useful vision, but more usually a disorganized
anterior chamber, an occluded pupil and total posterior synechiae, a condition
which may eventually lead to atrophy of the globe even though the choroid
has been little affected and the posterior segment of the globe has remained
comparatively normal.
Such inflammations occur either exogenously or endogenously. In
eacogenous cases they result from infection introduced from an ulcer or a
perforating injury involving the anterior part of the globe, a category which
includes post-operative infections. As a rule, in such cases the lips of the
wound become oºdematous and necrotic ; the iris becomes thickened and
swollen and intensely hyperaemic, the tissues of the iris and ciliary body
become densely infiltrated with inflammatory cells and extravasated
blood, even in places becoming necrotic, and from them is poured
out a copious inflammatory exudate, coagulating on the surface of
the iris, spreading over the lens capsule to cover the pupillary area, coating
the back of the cornea, filling up the anterior chamber to form a hypopyon,
the posterior chamber to surround the lens, and spreading into the anterior
part of the vitreous body (Fig. 1844, Plate XXXIII). This clinical picture is
associated with a hazy cornea, intense injection of the globe and usually
chemosis, and frequently a swelling and Oedema of the lids, while it is
accompanied by severe pain and general malaise.
If the inflammatory process continues to be progressive and spreads to
the posterior segment a panophthalmitis develops ; but it may remain
localized and resolution set in. In this case macrophages invade the exudate
and organization and fibrosis result." The exudate in the anterior chamber
may thus become converted into fibrous tissue, new-formed vessels growing
in from the iris, a tissue which becomes adherent to the cornea in front at its
1 For pathology see p. 2166.
2226 TEXT-BOOK OF OPHTHALMOLOGY
periphery, and to the lens behind so that the posterior chamber is obliterated.
Sometimes the organized exudate spreads over the posterior surface of the
cornea ; and occasionally the cornea, the iris and the lens become welded
together in an adherent staphyloma.” Meantime, atrophy of the iris tissue
progresses *; it becomes thin and loses its architectural demarcations, its
stroma is converted into rigid fibrillary connective tissue, hyaline degenera-
tion appears affecting particularly the blood-vessels, and the branched
chromatophores become converted into round cells, proliferating and massing
together into clumps. Simultaneously, in the ciliary region the exudate
organizes to form a ciliary membrane, which, embedding the ciliary processes
as it organizes and contracts, reduces them to a state of atrophy and draws
them into the interior of the eye, while the epithelium, both pigmented and
unpigmented, proliferates into the organized fibrous tissue as long finger-like
processes. The lens, also, encircled and pressed upon by fibrous tissue,
becomes cataractous and shrinks, while if much exudate has escaped into
the anterior vitreous, its subsequent contraction may result in atrophy of
the globe.
It is to be remembered, however, that the subsidence of the acute
inflammation and the development of atrophy does not by any means
indicate the end of the patient’s troubles, for, in contra-distinction to a
phthisical eye, which, being reduced to little more than a fibrotic mass, usually
remains quiet, an atrophic eye tends to develop periodic attacks of irritation
and inflammation. This condition of relapsing atrophic uveitis may, indeed,
be associated with so much recurrent pain and discomfort that in many cases
excision is preferable to the retention of the eye.”
These acute inflammations of exogenous origin are not limited to actual
organismal infections, for we have already seen * that the diffusion of the toxins of
organisms through the cornea can produce the same effect : the most typical clinical
picture which arises in this way is the irido-cyclitis with hypopyon which accompanies
an ulcus serpens,” but a somewhat similar clinical picture is seen in diplo-
bacillary ulcers" and in the reaction to mycotic keratitis". We have also seen * that
chemical substances of a poisonous nature, whether organic or of animal or vegetable
origin, are liable to excite the same clinical reaction—extensive necrotic burns, the
stings of insects, the juices of plants, and so on. The clinical picture of irido-
cyclitis with hypopyon produced in all these cases conforms to type in its general
characteristics.
The endogenous cases run a similar course, the majority developing a
panophthalmitis. One or more isolated abscesses with a localized tumour-
like appearance and surrounded by acute inflammatory changes of the most
violent character may occur in the iris and ciliary body in metastatic
staphylococcal infections (Raubitschek, 1914; Heine, 1923 ; Mylius, 1926;
Horner and Cordes, 1931), but even in these cases the fulminating irido-
cyclitis produced may well necessitate excision. A characteristic of such
* Vol. II, p. 1825. * p. 2398. * Vol. II, p. 1232. * p. 2131.
* Vol. II, p. 1934. * Vol. II, p. 1939. 7 Vol. II, p. 1941. 8 p. 2130.
DISEASES OF THE UVEAL TRACT 2227
conditions is the intense necrosis which becomes evident ; this is always seen
locally and is sometimes so widespread as to involve the entire iris, an effect
due not only to the concentration of toxins but also to the widespread
blockage of the vessels by massive thrombosis (Lindner, 1920). On the
other hand, if the infection be less virulent, the eye may survive, sometimes,
however, to show subsequent relapses in the condition of recurrent irido-
cyclitis with hypopyon.
RECURRENT IRIDo-cycliſtis witH Hypopyon (Hypopyon RECIDIVANs,
IRIDO-CYCLITIS RECIDIVANS PURULENTA, IRIDO-CYCLITIS SEPTICA,
oPHTHALMIA LENTA) is a condition in which a purulentirido-cyclitishabitually
recurs, sometimes over a period of years and frequently in both eyes, usually
resulting in disorganization of the eye and loss of vision. The disease occurs
usually in young male adults towards the end of a long illness when other
infective complications are in evidence–swellings of the joints (v. Herren-
schwand, 1929), furunculosis, metastatic abscesses, empyema of the
maxillary antrum (Weve, 1923), orchitis
(Blüthe 1908), stomatitis and
pyodermia (Zeeman, 1936), and so on.
Most of these cases seem to be
septicæmic in origin, but Weve (1923),
reasoning from the persistence of the
recurrences and the relative mildness
and shortness of the attacks when
compared with the usual metastatic
lesion, advanced the view that they
were an expression of allergy to a
staphylococcal infection. On the other - -
hand, cases have been associated with Fig. 1849.-RECURRENT PURULENT IRIDo-
erythema nodosum (Reis, 1906; Gilbert, CYCLITIS.
1925; Blobner, 1937), while others C *..."p"ºn", º:
have been attributed to tuberculosis ...iii. *" º º,
(Stähli, 1922; Urbanek, 1929–32, (weve, A. f. Aug.).
with a positive blood-culture;
Zeeman, 1936). An analogy may be developed here with patients with
phlyctenules who are allergic to tubercle and harbour an abundance of
staphylococci. Finally, despite the most thorough search including the
microscopical examination of an eye, cases occur wherein no cause can be
discovered (von Hippel, 1932).
Whatever the aetiology, the clinical history is usually a miserable story;
a sudden attack of acute iritis occurs in one eye, a hypopyon appears and
after reaching considerable dimensions, usually disappears in a few days,
but permanent damage, synechiae, exudates on the lens capsule and vitreous
opacities are left. Quite unexpectedly in a few weeks or a few months a

2228 TEXT-BOOK OF OPHTHALMOLOGY
recurrence takes place, and so the story goes on ; sooner or later the other eye
is usually involved, exudates in the fundus and optic neuritis are frequent
complications, and too often the end-result is an atrophic iris, an occluded
pupil and bare perception of light or a completely disorganized or shrunken
eye (Fig. I849).
Several such eyes have been pathologically examined (Blüthe, 1908;
Gilbert, 1920; Weve, 1923; Nakayama, 1926; van Heuven, 1930;
v. Hippel, 1932; Blobner, 1937). The usual histological picture is one of
recent and old inflammation of a non-specific type characterized by
polymorphonuclear cells and erythrocytes, followed by round- and plasma-
celled infiltration over the entire uveal tract, with advanced atrophy and
pigmentary disturbance. In the end the choroid and retina are usually
found in an indiscriminate mass behind the lens in which Ossification may
occur, and most of the intra-ocular tissues are destroyed.
The treatment is as described for acute irido-cyclitis generally, parti-
cular attention being paid to increasing the patient’s immunity to the
offending organism. Blobner (1937) got good results temporarily from
blood transfusions. The prognosis, however, is usually bad, for if the
patient survives the underlying infection, and if the eye is saved from
disorganization, as a rule some degree of optic atrophy with narrowing of
the retinal vessels permanently affects the vision.
Blobner. Z. f. Aug., xci, 129, 1937. Mylius. K. M. Aug., lxxvi, 881, 1926.
Blüthe. Diss., Heidel., 1908. Nakayama. A. f. O., czvi, 249, 1926.
Gilbert. A. f. Aug., lxxxvi, 29, 1920; xcvi, Raubitschek. K. M. Aug., lii (1), 683, 1914.
119, 1925. Reis. K. M. Aug., xliv, (2), 203, 1906.
Heine. K. M. Aug., lxxi, 106, 1923. Ståhli. K. M. Aug., lxix., 721, 1922.
v. Herrenschwand. K. M. Aug., lxxxiii, Urbanek. Z. f. Aug., lxix., 174, 1929; lxxvii,
419, 1929. 17, 1932.
van Heuven. T. O. S., 1, 568, 1930. Weve. A. f. Aug., xciii, 14, 1923; civ, 192,
v. Hippel. A. f. O., czkviii, 272, 1932. 1931.
Horner and Cordes. Am. J. O., xiv, 628, Zeeman. Berens’ The Eye and its Diseases,
1931. p. 662. London. 1936.
Lindner. K. M. Aug., lxiv. (1), 217, 1920.
3. SUPPURATIVE CHOROIDITIs (SUPPURATIVE ENDOPHTHALMITIs)
In a suppurative inflammation of the choroid the main feature is the out-
pouring of pus into the vitreous cavity ; the pathology of the process is
therefore analogous to that which we have just considered, but the results
are usually more disastrous, for, while a hypopyon in the anterior chamber
can in favourable circumstances disappear to leave few traces behind, pus
in the vitreous cavity invariably entails permanent and serious damage and
usually loss of the eye. There is first, as we have already described, an
intense engorgement of the choroid, a leucocytic and histiocytic infiltration,
a spread into and a disorganization of the retina, and a pouring of exudate
into the vitreous to form therein an abscess. If the eye survives, this is
1 p. 2163.
DISEASES OF THE UVEAL TRACT 2229
transformed into fibrous tissue, which, contracting and degenerating,
detaches the retina and forms a pseudo-glioma or more usually results in
an atrophic globe.
As with purulent inflammations of the anterior segment of the uveal
tract, an endophthalmitis of this type may be due to exogenous and endo-
genous causes. The cases of eacogenous infection which start in the posterior
segment of the globe are usually due to infected foreign bodies penetrating
into the vitreous cavity. We have already discussed how the resulting
vitreous abscess primarily spreads therefrom to the retina, and then affects
the choroid secondarily, the suppurative process usually establishing itself
first at the periphery and the optic disc and spreading thence towards the
equator. The endogenous cases are due to the lodgment of virulent bacterial
metastases in the choroidal blood-vessels,” the site of predilection being
the smaller vessels in the anterior region. Quite frequently the process
results in a panophthalmitis; sometimes a localized endophthalmitis develops,
and rarely, especially in meningitis in children, the course of the disease may
be comparatively mild and some degree of vision may be retained.
The clinical picture varies with the severity of the infection. In the
most severe cases which start as a DIFFUSE PURULENT CHOROIDITIs and run
a panophthalmitic course, the suppuration in the posterior parts of the
globe is rapidly obscured by events anteriorly ; and cases which remain
confined to the deeper parts of the eye are relatively rare. In this event,
however, the absence of sensory nerves in the choroid allows even a purulent
inflammation of the posterior segment to be painless and symptomless, and
indeed, the eye may appear quite normal from the outside until the yellow
mass of purulent exudate behind the lens is seen through the dilated pupil
(amaurotic cat’s eye) (Jaeger, 1831) (Fig. 1848, Plate XXXIII). At an early
stage, of course, vision is rapidly reduced and ultimately is completely and
irrevocably destroyed, the common result being a complete destruction and
atrophic degeneration of the globe with subsequent shrinkage.
Occasionally it happens that the suppurative process in metastatic
cases remains localized—CIRCUMSCRIBED METASTATIC PURULENT CHOROIDITIs
—in which case the eye may survive and vision may be unimpaired apart
from the development of an absolute scotoma corresponding to the lesion,
the functional importance of which varies with its site. In such milder cases
the choroidal lesion may be seen through a vitreous haze, either as a large
hazy yellow patch with indefinite outlines, sometimes small in extent
(Fig. 1845), but more usually covering a considerable area and resembling
in its general appearance diffuse or conglomerate tuberculous disease of the
choroid (Hanke, 1921 ; Higuchi, 1934); over it the retina is usually cloudy
and oedematous for a considerable area. Frequently the site of the
metastasis is in the anterior region of the choroid, in which case, if recovery
1 p. 2163. * p. 2136.
22:30 TEXT-BOOK OF OPHTHALMOLOGY
ensues, no symptomatic damage may be done. Sometimes the site of the
lesion is near the disc when the clinical picture is characterized by an
appearance of intense papillitis with much exudation extending into the
neighbouring retina (Gérard, 1926; and others); or alternatively it may be
at the macula in which case the exudative reaction in the overlying retina
may assume the well-known and typical star-figure arrangement (Paton,
1912) (Fig. 1850). Occasionally small embolic lesions are scattered diffusely
over the fundustoform the picture of Disseyſin ATED METASTATIC CHORomoltis
Fig. 1850.-Metastatic Choroupiris.
There is very extensive exudation over the metastatic focus at the macula
(Paton, T. O. S.).
(Axenfeld, 1896; Prümm, 1900; Maier, 1902; and others). In all those
localized cases, if the eye survives, the final picture presented by the local
lesion is that of a white and completely atrophic area wherein the retina and
choroid have been entirely destroyed, one of the characteristics of which
is the lack of pigmentary proliferation owing to the acute nature of
the destruction. It is to be noted that even in those localized cases the
presence of keratic precipitates and the frequently accidental discovery of
inconspicuous posterior synechiae indicate that to some extent the entire
uveal tract has been involved.
Pathologically these lesions originate in an infective embolus in the smaller vessels
of the chorio-capillaris, as has been determined histologically by Axenfeld (1896)
Goh (1897), Bull (1901), Hanke (1921), and Higuchi (1934). Thence an acute suppura-
tive process spreads throughout the thickness of the choroid and involves the retina,

DISEASES OF THE UVEAL TRACT 2231
to be followed in favourable cases by a chronic inflammatory infiltration and fibrosis
in the manner already described." It is to be remembered that the majority
of such metastatic cases are primarily retinal and it is frequently impossible clinically
to differentiate the two (Fränkel, 1899; Peters, 1901; Bull, 1901; and others); but in
any event the point is of somewhat academic interest since the clinical course, the
prognosis and the treatment are identical in both.
The treatment of such lesions is on the lines already indicated,” a
determination of the aetiology and any specific measures that can be taken
against the offending organism, local applications such as atropine, heat and
leeches to the temple, general eliminative and tonic treatment and, if the
inner eye fills up with pus, evisceration or enucleation according to the
principles already laid down.
Axenfeld. B. O. G. Heidel.., xxv, 140, 315, Jaeger. Ueber Markschwamm d. Auges w.
1896. amaurotische Katzenauge. Würzburg,
Bull. T. Am. O. S., ix, 316, 1901. 1831.
Fränkel. A. f. O., xlviii (2), 456, 1899. Maier. Diss., Tübingen, 1902.
Gérard. La Clin. O., xv, 132, 1926. Paton. T. O. S., xxxii, 174, 1912.
Goh. A. f. O., xliii (1), 147, 1897. Peters. K. M. Aug., xxxix (1), 392, 1901.
Hanke. A. f. O., cv, 851, 1921. Prümm. Diss., Giessen, 1900.
Higuchi. K. M. Aug., xciii, 348, 1934.
II. Exudative Inflammations
A. Non-specific Types
1. IRITIS AND IRIDO-CYCLITIS
Although it may be taken as agreed that any inflammatory process affecting the
iris or the ciliary body involves to some degree the whole of the anterior segment of
the uveal tract, and that therefore to talk of iritis or cyclitis is incorrect in the strictly
pathological sense, yet from the clinical point of view the frequent localization of the
symptoms preponderantly in one or other region justifies a topographical differentiation.
It must not be assumed, however, that the classification here adopted describes
separate entities sharply partitioned the one from the other ; they merely form the
more dramatic types chosen for convenience in description from the continuously
varying series which the clinic affords ; and it is to be remembered that the variations
met with are not only those of nature depending on the site of the most intense inflam-
mation, but also of severity, so that a whole series of syndromes are produced depending
on the massiveness of the infection, its virulence, and the response and resistance of the
individual. Again it is to be remembered that although many of these inflammations
are called acute—and rightly so for their symptomatology and course may be exceed-
ingly acute—in the pathological sense this, too, is an inaccuracy, for, in contra-
distinction to the suppurative processes which we have just studied, the exudative
inflammations of the uvea are all characterized by the mononuclear infiltration associated
with sub-acute and chronic processes. The treatment of the subject in the following
pages must therefore be viewed with these reservations. The reader will also remember
that the general principles of the aetiology, pathology, symptomatology and treatment
have already been fully discussed in general terms, so that it will suffice at this stage
merely to indicate the appropriate references without detailed descriptions.
1 p. 2175. * p. 2206.
2232 TEXT-BOOK OF OPHTHALMOLOGY
i. SIMPLE ACUTE IRITIS
Simple acute iritis is a common disease and, although characterized by
acute symptoms and considerable distress, is not of itself of serious conse-
quence provided it is expeditiously and adequately treated, and provided the
cause is eliminated so that subsequent relapses—the most serious aspect of
the condition—are avoided. It is sometimes due to exogenous influences, as
for example, when associated with a keratitis or corneal injury which does
not involve the heavy organismal infection of the inner eye which we have
seen to give rise to a suppurative inflammation ; but in the vast majority
of cases, the aetiology is a systemic endogenous infection which, differing
from the metastatic processes we have just studied, causes an intra-ocular
inflammation either by the deposition in the eye of a few bacteria of miti-
gated virulence or by their toxic or allergic effects."
The clinical course of the illness varies with its severity, but the average
case lasts from 3 to 6 weeks. A typical attack comes on suddenly with the
classical symptoms the rationale of which we have already explained " : a
rose-red ciliary injection (Fig. 1852, Plate XXXIV; Fig. 1352, Plate XXI), a
blurring of the texture of the iris, a contracted inactive pupil, and at this
early stage, an oedema of the corneal endothelium, these being associated
with neuralgic pain in the eye and trigeminal region, photophobia, lacrima-
tion and blurring of vision. This symptom-complex, as we have seen, is
associated pathologically with a focal infiltration by mononuclear cells such
as is characteristic of sub-acute inflammations. In the usual case of
acute iritis such a focus lies in the substance of the iris ; centrally there
develops an area of necrosis, around it there is much occlema and distortion
of the tissues, and opposite it on the posterior surface fibrinous exudate
pours out by which the iris becomes bound to the capsule of the lens. When
the acute phase subsides, the focal lesion heals by fibrosis producing a
permanent defect in the stroma of the iris, and unless treatment has been
adequate, the exudative adhesion becomes converted into an Organized
synechia (Fig. 1854, Plate XXXIV).
If the patient is seen early and adequate treatment instituted the
symptoms usually diminish rapidly, and in most cases the condition clears
up happily ; the iris returns to normal, the exudates disappear, and recent
adhesions are broken down, leaving perhaps a small tag of the pigmentary
epithelium attached to the lens at the level of the contracted pupillary
margin as a permanent relic of the inflammatory attack (Fig. 1854). If,
however, treatment has not been adequate or timely, the story may be
very different, for synechiae become permanent, an iris bombé appears
(Fig. 1853, Plate XXXIV), a continuance of the inflammation leads to an
increase in exudation which may rapidly organize, and with the aetiological
factor still operative, recurrences lead to ever-increasing damage.
1 p. 2140. * p. 2197.
DISEASES OF THE UVEAL TRACT 2233
Prompt treatment is therefore the vital factor in the prognosis—rest, full
atropinization at the earliest possible moment, local heat, massive doses of
salicylates, and—most important of all—a thorough search for the
aetiological factor and its elimination, or, if that is impossible, its neutraliza-
tion as far as may be.
ii. SUB-ACUTE AND RECURRENT IRITIs
This type of inflammation may appear at first to be of less serious
import than the acute cases of iritis we have just considered, but although
the initial attack may be of comparatively slight severity and may pass
away leaving few sequelae, subsequent recurrences appearing persistently
at frequent intervals eventually tend by their cumulative effects to add up a
score of damage much more formidable than that resulting from a single and
more violent infection. In the vast majority of cases the aetiology is obscure.
They are, however, endogenous in origin and most probably allergic in
nature ; the rheumatic type of iritis comes into this category, and it is
possible that the infection in most cases is a focal streptococcal one (teeth,
tonsils, etc.)," while a considerable proportion may well be of a tuberculous
allergic nature. In any event the clinical picture suggests a relatively
weak noxious agent associated with poor resistance in the tissues, the two
factors carrying out a relatively equally balanced but interminable duel
wherein now one and now the other gains the upper hand for a time.
The clinical picture is quite non-specific and frequently less dramatic
than in a frankly acute iritis. Each attack may be associated with a varying
degree of ciliary injection and pain, but it may well happen that a gradual
loss of vision draws attention to an eye which shows undoubted evidence of
having suffered a series of sub-acute attacks to which little attention had
been paid—a contracted pupil which dilates poorly, small scars in the iris
or fluffy translucent grey infiltrative masses on its surface or nodules on the
pupillary margin, fine adhesions between the iris and the lens, a few cells
in the aqueous, numerous small keratic deposits and a generalized disturb-
ance of the corneal endothelium.
Pathologically each attack is characterized by the development of a small
focus of mononuclear infiltration of almost microscopic size which eventually
undergoes necrosis and fibrosis. In time an enormous number of these
become scattered throughout the tissue. Each attack may not last many
days, but in the absence of efficient treatment the disease almost invariably
becomes bilateral and recurrences persist at intervals of a few days or weeks
or months, until eventually the iris becomes widely scarred and atrophic
and completely bound down to the lens, the pupil becomes occluded by a
connective tissue membrane, and cataract or glaucoma results.
The treatment consists essentially of determining and eliminating the
aetiological factor, and on its success the whole prognosis depends. Each
1 p. 2147.
2234 TEXT-BOOK OF OPHTHALMOLOGY
attack is usually relatively easily controlled by atropine and heat, the
permanent damage caused by a series of attacks may demand surgical
methods of relief,” but the elimination of the aetiological factor is frequently
a matter of the greatest difficulty. A complete search for focal sepsis is
essential, and in the event of a focus being found a prolonged course of auto-
genous vaccine to desensitize the patient thoroughly is usually advisable in
the attempt to prevent recurrences. Clinical examination and a skin-test
may reveal the possibility of a tuberculous allergy, in which case tuberculin
over a period of one or two years is indicated. And if nothing is found
general anti-rheumatic measures may well be instituted together, perhaps,
with a course of tuberculin injections which may be effective in a non-
specific capacity.
iii. Acute. IRIDo-cycliſtis
An acute irido-cyclitis corresponds in its aetiology and its clinical course
with an acute iritis, but with a greater portion of the uveal tract involved,
every feature is accentuated, the duration of the acute phase is longer, and the
potential permanent damage greater (Fig. 1853, Plate XXXIV). The attack
may come on acutely and reach a fulminating stage rapidly, the ciliary
injection is intense frequently with chemosis, and a characteristic sign is
oedema of the upper lid; the aqueous is
frankly turbid (Fig. 1851), the cornea
hazy, while the precipitates on its
internal surface may be numerous and
large, a vitreous haze indicates an out-
pouring of exudates behind the lens, and
when exudation is massive (PLASTIC
IRIDo-cycliſtis), the tendency for the
development of total posterior synechiae
and a complete seclusion of the pupil may be great (Fig. 1855). In addition
to severe local pain and radiating neuralgia there is added tenderness in
the ciliary region, while the diminution of vision caused by the haziness of
the media may be accentuated by some degree of optic neuritis and oedema
in the macular region.
Complications, too, are more frequently in evidence, particularly in the
initial stages that of an increased tension. As we have seen * this may be
partially due to vaso-motor instability and dilatation, and partly to diffi-
culties in drainage encountered by the viscous, protein-rich intra-ocular
fluid in its passage through exit-channels clogged with fibrin and cellular
elements. In this event to the distress of an acute inflammatory condition
is added the greater distress of an acute secondary glaucoma, with its
mounting increase of pain and rapid diminution or (although rarely) abolition
Fig. 1851.--THE Aqueous IN Acute
Cyclºris (slit-lamp).
p. 2216. * p. 2202.

DISEASES OF THE UVEAL TRACT 2235
of vision. The sequelae are also more grave; abolition of the posterior
chamber and seclusion of the pupil are a commonplace, some posterior
lenticular opacities are frequent and degeneration in this tissue may
readily involve the formation of a complete secondary cataract, widespread
endothelial damage may lead to the development of permanent corneal
Opacities, the vitreous is usually disorganized to some degree, the extent of
which varies from the appearance of a cloud of dust-like opacities to the
formation of dense floating membranes, the toxic effects at the posterior pole
of the eye may leave permanent changes at the macula or some degree of
optic atrophy, or a long continued raised tension may have impaired the
vitality of the nerve, while the shrinkage of cyclitic membranes and the
disorganization of the ciliary body may result in the eventual diminution of
ocular tension and a gradual shrinking and atrophy of the globe.
The participation of the ciliary body in the inflammation thus not only
makes the disease much more violent and the complications more serious,
but also makes the prognosis more grave in that it may leave sequelae which
are not amenable to amelioration. The damage of an iritis is largely confined
to the anterior chamber and can usually be rectified surgically, but the
inflammatory products of a cyclitis invading the posterior segment of the
eye, in so far as they are not absorbed spontaneously (a prolonged and
partially successful process at the very best), are not susceptible of removal
and are a permanent liability. Partly owing to this, and partly owing to the
disorganization of the central seat of nourishment of the globe, the ultimate
and logical end-result of a severe and long continued cyclitis is atrophy of
the entire eye, a calamity which never follows an iritis.
Treatment, therefore, should be as prompt and thorough as possible,
for the only hope of a good prognosis in a well-established case is control of
the inflammation before irremediable damage has accumulated, and an
elimination of the cause before the acute phase has passed into a chronic one
or a tendency to relapses has been acquired. The most thorough aetiological
search should be followed by the immediate removal of the causal focus of
infection if that is possible, and the administration of any specific treatment
which is available against the offending organism ; if no such treatment
suggests itself, intensive salicylate therapy or non-specific measures (milk or
typhoid injections) may be tried. Locally everything should be done to
relieve congestion and pain (hot bathings, leeches, etc.), while every
endeavour should be made to attain and maintain mydriasis. In this
connection a close watch must be kept upon the tension, for if it becomes
raised the continued administration of mydriatic drugs may give rise to
anxiety, and the future of the eye may depend upon the prompt and judicious
adoption of the alternative methods of dealing with this complication which
have already been detailed."
1 p. 2218.
2236 TEXT-BOOK OF OPHTHALMOLOGY
iv. CHRONIC CYCLITIS
Chronic cyclitis is frequently a most unpleasant condition ; it may be
so insidious and devoid of dramatic symptoms that it may have progressed
so far as to have established itself beyond easy control before it is discovered ;
it may be so chronic and recalcitrant in its evolution as to become bilateral
and to progress with exacerbations and remissions over a period of years
despite every known method of treatment ; its persistent course may be
interrupted by complications, such as the insidious onset of a secondary
glaucoma, which may be difficult and dangerous to control; and the
wreckage left by its end-results may finally, after a disheartening struggle
extending over many years, amount to complete disorganization and
shrinkage of the eye.
The aetiology is always some endogenous infection, it may be an infective
focus, usually streptococcal, or it may be tuberculous ; but usually it is
hard and too frequently quite impossible to determine with any certainty.
When the causal factor is discoverable and when it can be eliminated or its
influence neutralized, the prognosis may be good ; but when it is not, the
issue ultimately depends on the resistance of the patient. In young adults
(in these the question of tubercle should be considered, although quite
frequently no cause can be found : Thomson, 1928) a serviceable eye may
well be retained ; but unfortunately the disease is characteristic of those
past middle life who are of a debilitated type, particularly women between
50 and 60, and in these the story is frequently different. Here we are
probably dealing with a toxin of low virulence but of long standing and well
established (such as in the mouth, throat and bowel), and it is working on
tissues whose immunological resilience is poor and will not respond to
stimulation. In these the story may be a sad one—that of a long and
insidious course varied by relapses and intermission until after many years
the eye (and sometimes both) becomes soft, tender and phthisical.
The clinical picture presented by the slighter and most insidious cases
(the SEROUS IRITIS of older authors) presents a minimum of symptoms and
signs (Fig. 1856, Plate XXXIV). There may, or may not, be a barely detect-
able ciliary flush, a few keratic precipitates and a few dust-like opacities in a
fluid vitreous, and perhaps some change of colour in the iris and an unusually
deep anterior chamber ; all of them slight and all of them readily missed.
There need be no pain or tenderness or any other indication of the mischief
progressing in the eye except a gradual diminution of vision which may be
periodically intensified. The visual change may be explained partly by the
development of myopia due to irritation of the ciliary muscle, but more
usually is accounted for by the haze in the media ; sometimes it results from
the slow development of cataractous changes at the posterior pole of the eye;
and not infrequently it is a symptom of an intermittent or insidiously
progressive secondary glaucoma which now dominates the picture and may
PLATE XXXIV
IRIDo-cyclitis
Fig. 1853.-Acute. IRIDo-cyclitis with IRIs
BoM.B.E.
Fig. 1856.-CHRoxic CycLITIs.
Fig. 1855. CHRosic Exudative IRIDo-
cyclitis showing NEw VEssels over
THE IRIs AND Pupil LARY ExupATE. [To face p. 2236.

DISEASES OF THE UVEAL TRACT 2237
have been present for some considerable time before the patient sought
relief.1
More active cases show a more obvious symptomatology. The ciliary
flush may be evident, there may be oedema of the upper lid, the keratic
precipitates may be conspicuous, the vitreous haze dense, and the vision
greatly diminished during the acute phases to recover considerably in the
intervals, while some neuralgia may be present in the eye and over the
forehead. Posterior synechiae, however, are not usually a conspicuous
feature at the beginning ; but in the end they tend to form and to organize.
A cyclitic membrane eventually appears, the vitreous gel breaks down com-
pletely and is replaced by a cloud of punctate and membranous opacities, and
after vicissitudes extending over many years, the globe becomes progressively
more soft, more tender and eventually shrunken and atrophic, a condition
in which excision for the relief of constantly recurring irritation may be
advisable.
A rare early complication is retinal detachment (Thomson, 1928; Beigelman,
1930), possibly associated with liquefaction of the vitreous gel ; as a late complication
owing to traction by organized exudate, it is, of course, common. The occurrence
of macular disease and optic neuritis has already been noted.
Apart from the usual local measures—in which the effect of atropine on
the tension must be carefully observed—treatment consists essentially of
eliminating the cause if it can be found and stimulating the resisting powers
of the patient in every possible way. Unfortunately both of them are
frequently matters of considerable difficulty, for the aetiology is too often
obscure and the patients of the type whose response is negligible. A full
discussion of both of these problems will be found in the previous sections.”
2. Exu DATIVE CHOROIDITIS
The classification of exudative inflammations of the choroid is most unsatis-
factory in the majority of treatises on the subject, different writers adopting entirely
different methods; in some cases there is a failure to differentiate inflammatory from
degenerative and vascular lesions, and in others a topographical classification is
adopted which treats the same disease-process as several separate entities according to
its location. The first policy merely leads to confusion and the second seems to com-
plicate a subject already sufficiently involved, especially when pushed to its logical
conclusion ; in the American Encyclopedia of Ophthalmology, for example, twenty-six
different forms of choroidal inflammation are given separate headings. On the other
hand, it would seem more advisable to follow the teaching of those writers (Hepburn,
1912–24 ; Rönne, 1915; de Schweinitz, 1921 ; Friedenwald, 1924; and others)
who press for the recognition of the identity of lesions with a similar pathology no
matter whether they occur at the macula, near the optic disc or in the general body of
the fundus, and to divide the varieties of this type of choroiditis into three classes only :
diffuse, disseminated, and circumscribed. All of them, whatever the size, number,
or site of the lesions may be, have the same aetiology which has already been
1 p. 3288. * p. 2206.
2238 TEXT-BOOK OF OPHTHALMOLOGY
fully discussed,” that of endogenous infection in which an organism or toxin
(syphilitic, tuberculous, streptococcal, etc.) finds its way to the choroid through the
blood-stream from some other part of the body. All of them have the same general
pathology which again has been fully discussed,” an inflammatory infiltration of the
mononuclear type, which during its acute exudative stage destroys the affected
area and usually the Over-lying retina, giving rise to a varying amount of exudation and
abolishing the function of the region. All of them resolve in the same way, passing
into an atrophic stage and leaving a scarred area, the interstices of which may be filled
up with pigment, and round the margins of which pigment proliferates to a varying
degree.
i. DIFFUSE CHOROIDITIS
A diffuse exudative inflammation of the choroid is relatively rare
(Fig. 1860, Plate XXXV): as will be noticed later it occurs in syphilis and
FIG. 1857.-DIFFUSE CHOROIDITIS.
The field of Fig. 1862, Plate XXXV.
tuberculosis, other cases appear to be due to focal infections or acute
illnesses such as measles or malaria (Marin-Amat, 1923), while others again
prove incapable of aetiological explanation. At the commencement one or
more ill-defined yellow or dirty grey plaques appear on the fundus which
tends to become obscured by a developing vitreous haze ; these gradually
spread, often creeping over the fundus until they coalesce with each other,
while new patches appear so that in time the greater part of the fundus,
particularly at its posterior pole, may become involved, the entire retina
being Oedematous and cloudy. As time goes on Organization gradually
occurs, fresh exudates appearing coincidently with the progressive resolution
of the older ones, but eventually nothing but white scarred areas are left in
1 p. 2140. ? p. 2190.

DISEASES OF THE UVEAL TRACT 2239
which the larger choroidal vessels may form a net-work, over which the
retinal vessels may run unchanged, and between which lie areas of normal
fundus (Fig. 1861, Plate XXXV). In the meantime there is much disturb-
ance and migration of the retinal pigment, which becomes agglutinated into
clumps and variously shaped aggregates, a distribution which is frequently
obvious towards the periphery, where an appearance simulating primary
pigmentary degeneration * may be produced (Fig. 1862). Such a condition
may be termed SECONDARY PIGMENTARY DEGENERATION OF THE RETINA,
R G H T
FIG. 1858.-SECONDARY RETINITIS PIGMENTOSA.
A case of old disseminated choroiditis with much pigmented atrophy of a
feathery type in the mid-periphery. Night-blindness 4 or 5 years, vision 6/60
(Hepburn, T. O. S.).
As a rule there is a scotoma in the field corresponding to the affected area.
(Fig. 1857), but in the milder cases where tissue-destruction has not been
extensive, the functional disability may be a concentric ring Scotomata and
hemeralopia (Hersing, 1872; Hepburn, 1908; Augstein, 1915–16) (Fig. 1858).
ii. DISSEMINATED CHOROIDITIS
Disseminated choroiditis is a much more common lesion (Fig. 1863,
Plate XXXV); here again the cause is often syphilis, which is frequently
congenital, or tubercle, but other cases occur wherein a non-specific infective
aetiology must be postulated. The clinical picture is that of a large number
of isolated inflammatory foci scattered over the fundus ; a recent focus
1 p. 2765.
T.O. — W () I.. I : I. L

2240 TEXT-BOOK OF OPHTHALMOLOGY
appears ophthalmoscopically as around yellowish fluffy spotlying underneath
the retinal vessels, but over which the retina is cloudy and oedematous owing
to the percolation of fluid through Bruch's membrane. As organization
proceeds the exudative area is replaced by scar tissue, Bruch's membrane
is destroyed, and the retina and choroid are bound together in a single scar
within and around which is much proliferation of pigment. Since the
exudative stage is of short duration, the lesion is observed usually only after
activity has subsided and the atrophic stage has been reached; this is
attained in the matter of a few weeks. This stage persists permanently,
Fig. 1859.-Dissºminated Choroid ITIs: ATRoPHic STAGE.
the lesion appearing as a white area bespattered with pigment, with sharp
heavily pigmented edges (Fig. 1859). In the meantime fresh foci appear,
sometimes over a period of many months, and although in the milder cases
they may remain relatively few in number, in the more severe cases they
may multiply until the entire fundus becomes peppered with them.
In the active stage when exudative activity is proceeding there are
always some vitreous opacities; but in the less severe cases these may
be eventually absorbed. At the beginning there is also an oedema and
hyperaemia of the disc, which later may become grey and atrophic (choroiditic
atrophy), while the retinal vessels become much constricted. A further
sequel in severe cases is the development of a complicated cataract at the
posterior pole of the lens owing to faulty nutrition and toxic absorption.
As in all cases of choroiditis the symptoms are entirely visual. During
exudative activity the vitreous haze and retinal oedema may lower the

PLATE XXXV
DIFFUse AND Disse MINATED CHoRoidITIs
Fig. 1860.-DIFFuse ChoroidITIs (early stage).
Fig. 1861.-DIFFUse Choroid its (atrophic Fig. 1862–DIFFuse SUPERFIGIAL Choko-
stage). DITIs (pigmented stage) (see Fig. 1857).
- * -
- ºf . -
-** ºl. º * -ºs.
ºf . º -
º º º - º
-
Fig. 1863. DissEMINATED CHoRoibitis. Fig. 1864.—METASTATIC CHoRoipitrs (part
of a pan-uveitis).
[To face p. 2240.











DISEASES OF THE UVEAL TRACT 2241
vision considerably, while the irritability and disturbance of the retina is
indicated by photopsiae. Provided the macular region is spared, however,
central vision may eventually be unimpaired, although changes in the
optic nerve or the lens may produce a deleterious effect. Around the focal
areas retinal distortion is marked, and in the corresponding areas of the
field scotomata are permanent—these may be innumerable and minute,
forming a legacy which, when the macula is involved, is calamitous
(Fig. 1865).
Disseminated choroiditis is usually diffuse in its topographical distribution over the
fundus but sometimes is limited to a relatively restricted area. The most common such
area is the periphery—ANTERIOR DISSEMINATED CHOROIDITIs (Fig. 1896, Plate XXXIX)
—a condition most frequently met with in association with the anterior uveitis which
R J C. H. T.
FIG. 1865.-DIssEMINATED CHOROIDITIs (Hepburn, T. O. S.).
accompanies syphilitic interstitial keratitis. The posterior pole may also be singled
out ; and in this region a peculiar form was described by Förster as AREOLAR
CHOROIDITIs—a condition to be distinguished from the sclerotic lesion of central areolar
choroidal atrophy." This type of choroiditis is peculiar in that the first focus is in
the region of the macula while subsequent ones appear at ever-increasing distances
from it, so that the more peripheral foci are the more recent. The evolution of the
pigmentation of the individual spots is also peculiar : at first they are uniformly black,
but as organization proceeds they progressively depigment from the centre outwards,
so that they eventually appear as dark circles with a white interior. Such behaviour
is the opposite of the usual gradual increase in pigmentation of choroiditic patches.
iii. CIRCUMSCRIBED ExUDATIVE CHOROIDITIS
In this form one or more patches of exudative choroiditis occur, in
which, while each is usually considerably larger in size than the average
1 p. 24.12.

L 2
2242 TEXT-BOOK OF OPHTHALMOLOGY
focus in a disseminated lesion, the appearance and the evolution of the
individual patches are, however, the same. There is a raised yellowish
area of exudative inflammation with indistinct and fluffy edges, over it
the retina is oedematous, and its development is associated with the formation
of a fibrous pigmented scar. Hepburn (1912) has divided these cases into
two categories according as to whether they lie superficially or deeply in
the choroid, and since there are considerable differences in the clinical
course and effects of the lesion according as to whether the smaller vessels
only or the entire tissue is involved in the inflammatory process, we shall
adopt his classification.
In the suPERFICIAL TYPE OF CHOROIDITIs (Plate XXXVI) there is
frequently no history available, for the inflammation may come on, evolve
and organize without the knowledge of the patient. An exudative inflam-
matory patch appears which is fairly sharply differentiated from the rest of
the fundus ; only occasionally are keratic precipitates seen, and the vitreous
haze is slight. Within a few weeks the inflammation dies down, leaving an
atrophic area in which there is little development of fibrous tissue but much
pigment proliferation, the greater bulk of which is retinal, which may be
heaped up in great masses. Over the patch normal retinal vessels are seen
to cross, and on its floor the larger choroidal vessels may be visible indicating
that these arteries are unharmed.
Such patches may be single, but are usually multiple, several appearing
simultaneously, or one following another (Figs. 1869–70). In shape they may
be round or oval, or quite irregular ; their size varies, but frequently they
are large and may have a diameter 3 or 4 times that of the disc. Sometimes
they are found in large numbers (Fig. 1868), especially in the mid-peripheral
region, but they always leave a considerable part of the fundus unaffected
and are never so widely distributed as in disseminated choroiditis.
Over the lesion the layer of rods and cones is destroyed, so that an
absolute Scotoma in the field of vision is produced which corresponds exactly
with the area involved. When multiple lesions exist the loss of retinal
function therefore shows itself in numerous scotomatous areas but, unlike
deep inflammations, these are circumscribed and do not extend beyond the
area of actual inflammatory destruction.
In contra-distinction to the superficial type, a DEEP CHOROIDITIS is a
much more obvious and serious disease. Adequate attention was first
drawn to it by Hill Griffith (1898), and its significance has been fully described
by Friedenwald (1902–24), Hepburn (1912–14), Heath (1939), and others; the
first of these described 100 cases. The aetiology is almost certainly varied
and embraces most forms of endogenous infection, occurring mainly in young
adults of either sex. Syphilis seems to be an unimportant factor (Verhoeff,
1916; Knapp, 1920; Zeeman, 1921; 1 out of 100 cases, Friedenwald, 1924);
tubercle is a favourite diagnosis (v. der Hoeve, 1914; Shertlin, 1916; Gilbert,
PLATE XXXVI
SUPERFICIAL CHoRoibitis
Fig. 1866.-CIRCUMscribed SUPERFICIAL Fig. 1867.-CIRCUMsCRIBED SUPERFICIAL
CHoRoibitis : SUB-Acute STAGE. ChoroidITIs: ATRoPHIC STAGE.
Figs. 1869–70.-ChoroidITIC PATCHES IN PERIPHERY witH A Juxta-PAPILLARY LEsion.
Fig. 1869-A fresh juxta-papillary lesion. Fig. 1870.-The same case 4 months later.
[To face p. 2242.


DISEASES OF THE UVEAL TRACT 2243
1923), but the evidence for this has often been inconclusive, the apparent
response to tuberculin being frequently extravagantly interpreted in view
of the tendency of the disease to spontaneous cure (Rönne, 1915). In the
majority of cases the aetiology is obscure and probably depends on latent
foci of infection.
The intensity of the symptoms varies with the severity of the lesion, but
the characteristic signs of the disease are :-
1. The immediate and sudden appearance of a fine haze of vitreous
Fig. 1871.-PERIPHERAL SolITARY CHoRoibitis.
A localized inflammatory scar in the periphery (Hepburn, T. O. S.).
opacities, always sufficiently numerous to cause visual disability, and some-
times obscuring the ophthalmoscopic view of the fundus.
2. The early appearance of fine keratic precipitates and the late
development of coarser depositions.
3. The appearance of an inflammatory patch in the fundus.
4. The complete absence of all external signs of inflammation, the iris
being healthy and the pupil active.
The clinical course varies considerably with the intensity of the
inflammation. The onset is always sudden and marked by a dramatic
decrease of vision due to the cloud of vitreous opacities; sometimes there is

2244 TEXT-BOOK OF OPHTHALMOLOGY
pain or discomfort on moving the eyes, and occasionally an ophthalmic
migrainous attack (Blessig, 1910; Friedenwald, 1924), or a general
malaise, but these subjective symptoms are rare. Apart from the keratic
precipitates the external eye appears normal, but if the fundus can be seen,
ophthalmoscopic examination reveals one or more patches of choroiditis. In
contrast to the superficial lesion these are usually single (Fig, 1881) or few in
number (not more than 3 or 4). In its acute stage (Fig. 1871, Plate XXXVII)
it has the usual yellow oedematous appearance with a fluffy outline, but as
it heals it becomes greyish in colour: pigmentation is usually scanty,
Fig. 1872.-RECURRENT Patches or Chorzoi Diris.
amounting in most cases to a stippling of the affected area which becomes
demarcated from the rest of the fundus by a pigmented line, but fibrous
tissue formation may eventually be prolific, and a retinitis proliferans may
become established. Such patches vary in size from a small circular lesion,
smaller than the disc, to an area many times the size of the disc. The
intensity of the inflammation varies very considerably, but, unlike the
superficial lesion, its course is slow and it usually takes months and some-
times years to subside. Moreover, if the aetiological factor persists, it has a
strong tendency to recur either in the same place or elsewhere in the same
eye or in the other (Fig. 1872); thus Friedenwald (1924) found that 40%
of his cases showed fresh lesions at the site of old patches, or old lesions
p. 2604,

DISEASES OF THE UVEAL TRACT 224.5
elsewhere in the fundus of the same or the fellow eye. Bilateral cases are,
however, rare (van der Hoeve, 1914; Rönne, 1915; Zentnayer, 1922;
Friedenwald, 1924). After the acute stage has passed, in the majority
of cases the keratic precipitates disappear, the vitreous opacities slowly clear
up, and the vision returns to normal apart from the scotoma which persists
permanently. The immediate prognosis, therefore, depends on the site of
the lesion and the importance of the resultant scotoma. Some cases, how-
ever, show macular changes even when the lesion is some considerable
distance away—oedema, star-shaped figures, or pigmentary disturbances.
Others of great severity wherein the fundus is entirely obscured by
abundant keratic precipitates, a turbid aqueous and vitreous Opacities, may
L.E. F. T O t. E. F. r O
FIG. 1873.−SECTOR DEFECT IN DEEP FIG. 1874.—SECTOR DEFECT IN DEEP
CHOROIDITIS. CHOROIDITIS.
Early stage. Case of Fig. 1881, Late stage. Field of Fig. I882
Plate XXXVII. (Hepburn).
progress to the development of irido-cyclitis, cataract, glaucoma, retinal
detachment and phthisis bulbi.
The occurrence of keratic precipitates is almost universal although they may be
extremely fine and dust-like. They may not be seen in extremely mild cases, but, since
they frequently only remain during the acute exudative stage, it is possible that they
have been missed. The presence of precipitates in the absence of iritis should always
suggest the possibility of a choroiditis for evidence of which the fundus and fields
should be searched. It is usually said that they indicate an associated cyclitis (Hepburn,
1912–14), a condition termed serous uveitis by Schieck (1920), but this seems an
unwarrantable assumption, as also does the conclusion that the deposition of similar
exudative cells along the pupillary margin provides evidence of a hypothetical super-
ficial iritis limited to the pupillary border (Schieck, 1920). On the other hand, the
complete absence of signs of inflammatory trouble in the iris and ciliary body, and the
appearance of the precipitates with the Onset of the choroidal lesion and their dis-
appearance with the cessation of its activity suggest that, like the exudates in the
vitreous, these cells are derived from the posterior segment.

2246 TEXT-BOOK OF OPHTHALMOLOGY
The scotoma resulting from a deep choroiditis is frequently peculiar and
characteristic. Sometimes it shares the characteristics of the defect caused
by a superficial inflammation in that it corresponds in extent to the confines
of the lesion itself, but more usually it is sector-shaped, extending right to the
periphery, while in some cases the retina actually over the affected area
remains functional (Figs. 1873–4). For example, with a lesion near the disc an
enlargement of the blind spot only may be apparent, or a localized scotoma
may result, or a complete sector defect ; or a localized Scotoma may in a
few months develop into a sector defect. Moreover, similar lesions in the
two eyes may produce a circular localized scotoma in one and a sector-defect
in the other ; or a central lesion may give rise to a sector defect running
out to the periphery, while a peripheral lesion may fail to blot out the small
area of the field beyond its confines.
The causation of the sector-shaped field defect is disputed. Most authors ascribe
it to destruction of the nerve fibres (Groes-Petersen, 1912; v. d. Hoeve, 1914 ; and
others); others attribute it to a blockage of the deep vessels and consequent destruction
of the retinal neuro-epithelium in the more peripheral parts (Jensen, 1908; Hepburn,
1912–14 ; and others). Until more pathological work is done, however, the question
must remain undecided.
Some doubt has arisen whether the disease may be primarily a retinitis or a
choroiditis, a doubt reflected in the names proposed from time to time retino-
choroïditis (Groes-Petersen, 1912), neuro-fibrilitis (v. d. Hoeve, 1914), neuritis
retinoe (Zeeman, 1921), and others. Larsen (1918) and Fleischer (1922) regarded it as
primarily retinal, the latter associating it with multiple sclerosis ; and Fuchs (1921)
examined one case pathologically wherein the lesions were confined to the retina
without any choroidal involvement. The clinical appearances, however, indicate that
in the majority of cases the choroid is the essential site of the lesion although the
retina is undoubtedly involved as well.
The main points of difference between the superficial and deep types of
inflammation may be summarized thus:—
SUEPERFICIAL DIEEE’
1. May occur without the knowledge of 1. Sudden onset with dimness of vision.
the patient.
2. Few vitreous opacities and rarely k. p. 2. Many vitreous opacities and k.p. the
rule.
3. Sharply defined inflammatory patch. 3. Hazily defined inflammatory patch.
4. Clears up in a few weeks. 4. Active for many months.
5. Much pigment proliferation. 5. Little pigmentation.
6. Little fibrous tissue reaction. 6. Much fibrous tissue reaction.
7. Choroidal vessels cross the floor of the 7. All choroidal vessels destroyed.
patch.
8. Scotomata correspond to affected area. 8. Wedge-shaped Scotomata running to
periphery common.
DISEASES OF THE UVEAL TRACT 2247
Fig. 1875.-JuxTA-PAPILLARY CHoRoibitis (Abraham, A. of O.).
The site of the lesion is very varied, any part of the fundus being
susceptible. Friedenwald’s (1902–24) statistics for 100 cases showed the
following distribution —
Near the disc . - - - - . 29 cases
Near the macula - - - - , 29 , ,
In the periphery - - - - 37 , ,
Extending from disc to periphery - - 1 case
Extensive lesion only dimly seen . - - 4 cases
Fig. 1876–FIELD of JuxTA-PAPILLARY CHoRoidITIs Due to A SMALL Patch AT THE
Border of THE Disc.


2248 TEXT-BOOK OF OPHTHALMOLOGY
Depending on the topographical site the lesion has received different names,
a practice which, as we have seen, has little justification (Hepburn, 1914;
Rönne, 1915; Friedenwald, 1924). Its occurrence at two sites, however,
may be specially noted ; near the disc and at the macula.
Its occurrence near the disc, which is accounted for by an involvement of
the vessels of the circle of Zinn, was originally noted by Griffith (1898) and
Friedenwald (1902), but was subsequently described by Jensen (1908) who
published four cases (occurring in 62,800 patients) under the heading of
Fig. 1877–CENTRAL CHoRoibitis (Hepburn).
CHORomorris JuxTA-PAPILLARIs, and by this name it is frequently alluded to
(Figs. 1869–70, 1875, 1879–80). Many such cases have been observed (Blessig,
1910; Patterson, 1910; Hepburn, 1912–14; Appleman, 1914; v. d. Hoeve,
1914; Ham, 1914; Rönne, 1915; Schertlin, 1916; Gertz, 1916:
Lodberg, 1916; Fleischer, 1922; Abraham, 1929–32; and others). The
aetiology, course, prognosis, and treatment are the same as for a deep
choroiditis elsewhere in the fundus, the only noteworthy point from the
clinical aspect being the possibility of confusing the diagnosis in the
active stage with an optic neuritis (Fig. 1869). In these cases, of course,

DISEASES OF THE UVEAL TRACT 2249
the sector-shaped defect in the field may be very large indeed considering
the actual size of the choroidal lesion (Fig. 1876).
A MACULAR (CENTRAL) CHoRoidITIs (Figs. 1877–8)is also common, and it is
important because of the great disability resulting from the central scotoma:
it is to be noted, however, that loss of function is usually limited to the
macula. Moreover, while the general pathology of the lesion is the same as
occurs elsewhere in the fundus, the peculiar anatomical conditions at the
macula endow a lesion situated here with certain characteristic attributes.
It will be remembered that the macular region is supplied by a relatively
self-contained choroidal circulation, and for this reason the inflammatory
Fig. 1878.-CENTRAL CHoRoibitis.
A localized inflammatory scar at the macula (Hepburn, T. O. S.).
area is comparatively large and almost always circular ; moreover, since
the vessel-system is localized, a single lesion is the rule. Again, the choroid
here is thick and the overlying retina relatively thin ; it therefore quite fre-
quently occurs that the inflammatory process assumes a characteristically
severe character, and is usually associated with considerable oedema and
exudation into the neighbouring retina. In this way a retinitis circinata 1
may be produced, and indeed the dramatic and striking changes in the
retina may tend to obscure events in the choroid underneath, although the
latter may be of primary importance. The amount of cicatricial tissue
remaining at the macula varies considerably—sometimes there are large
masses of fibrous tissue (Fig. 1877), at other times only a small crinkled scar;
and at the same time the associated retinitis may resolve or cicatrize,
1 p. 2760.

2250 TEXT-BOOK OF OPHTHALMOLOGY
leaving a certain amount of permanent scar-tissue behind, varying from a
few scattered patches in the region round about to a circle occupying the
entire central region of the fundus (Fig. 1878), (see Gunn, 1894; Spicer,
1894 ; Lawford, 1896 ; Butler, 1910; Collins, 1911 ; McMullen, 1911 ;
Hepburn, 1912; Zentmayer, 1922; Kraupa, 1936; and many others).
The treatment of exudative choroiditis should be along the lines we have
already fully discussed," but since, despite any type of treatment, the lesion
involves a complete and irremediable destruction of the area involved,
and since, once having occurred, the tendency is for it to heal spon-
taneously without the widespread and far-reaching complications which
characterize inflammations of the anterior part of the uveal tract, heroic
therapeutic measures are not usually indicated. The most important
element in the treatment, indeed, is to determine and eliminate the
aetiological factor ; the immediate damage has been done and every care
should be taken that repetitions do not occur. In the acute stages rest in
bed in a darkened room is indicated, and locally atropine and heat, perhaps
as diathermy, are helpful. Free opening of the bowels, general eliminative
measures, salicylates in large doses, and general stimulating treatment will
hasten resolution ; in the more chronic stages mercury and iodine may aid
the healing process. But everything should be made secondary to the
attack by any specific or non-specific measures which may be indicated to
combat the activity of the responsible organism.
Abraham. A. of O., ii, 452, 1929; viii, 503,
1932.
Appleman. O. Rec., xxiii, 221, 1914.
Augstein. K. M. Aug., lv., 474, 1915; lvii,
272, 1916.
Beigelman. J. Am. Med. As., 1658, 1930.
Blessig. A. f. O., lxxiv, 284, 1910.
Butler. Ophthalmoscope, viii, 409, 1910.
Collins. T. O. S., xxxi, 112, 1911.
Fleischer. B. O. G. Heidel., xliii, 140, 1922.
Friedenwald. T. Am. O. S., xxxviii, 577,
1902.
T. O. S., xliv, 308, 1924.
Fuchs. A. f. O., cvii, 15, 1921.
Gertz. K. M. Aug., lvi, 301, 1916.
Gilbert. Z. f. Aug., l, 267, 1923.
Griffith. Norris and Oliver's System of
Diseases of the Eye, iii, 355, Phila., 1898.
Groes-Petersen. K. M. Aug., l (2), 159, 1912.
Gunn. T. O. S., xiv, 99, 1894.
Ham. K. M. Aug., lii, 484, 1914.
Heath. Brit. J. O., xxiii, 289, 1939.
Hepburn. T. O. S., xxviii, 255,
xxxii, 366, 1912.
R. L. O. H. Rep., xviii, 92, 1910; xix, 398,
1914.
Brit. J. O., viii, 401, 1924.
1908;
Bersing. A. f. O., xviii (2), 69, 1872.
M
v. d. Hoeve. K. Aug., liii, 487,
1914.
Jensen. A. f. O., lxix, 41, 1908.
Knapp. T. Am. Acad. Oph. Oto-Lary., xxxv,
132, 1920.
Rraupa. Z. f. Aug., lxxxix, 204, 1936.
Larsen. K. M. Aug., lx, 650, 1918.
Lawford. T. O. S., xvi, 87, 1896.
Lodberg. K. M. Aug., lvi, 301, 1916.
McMullen. T. O. S., xxxi, 266, 1911.
Marin-Amat. A. de Oft. H. - A., xxiii, 633,
1923.
Patterson. O. Rec., xix, 260, 1910.
Rönne. K. M. Aug., liv, 455, 1915.
Schertlin. K. M. Aug., lvii, 60, 1916.
Schieck. Z. f. Aug., xliii, 625, 1920.
de Schweinitz. Diseases of the Eye. Phila.,
1921.
Sédan. Marseille méd., lix, 871, 1922.
Spicer. T. O. S., xiv, 132, 1894.
Thomson. Brit. J. O., xii, 189, 1928.
Verhoeff. T. Am. O. S., xiv, 568, 1916.
Zeeman. A. f. O., cvi, l, 1921.
Zentnayer. Am. J. O., v, 486, 1922.
1 p. 2206.
PLATE XXXVII
DEEP Chokolbit is
Figs. 1879–80.-JuxTA-PAPILLARY CHORoibitis.
Fig. 1879–Early stage. Fig. 1880.-Same case in late stage (Goulden).
º-D - Avv. HEAD --
Figs. 1881–82.-DEEP CHoRoi DITIs.
Fig. 1881–Early stage. Fig. 1882.-Same case in late stage (Hepburn).
[To face p. 2250.


DISEASES OF THE UVEAL TRACT 2251
3. ExUDATIVE UVEITIS
Exudative inflammations affecting the whole of the tissues of the uveal
tract, sometimes termed CHRONIC IRIDO-CHOROIDITIS, are less common than
those associated primarily with the anterior or posterior segments ; but as
would be expected, the prognosis is more grave, associated as they are with
all the misfortunes which follow a chronic inflammation of each individual
part. Such a widespread inflammation may, of course, be the end-result
of an illness which began as an iritis, a cyclitis, or a choroiditis, but here we
shall deal with those conditions which from the start have the character of a
complete ExUDATIVE ENDOPHTHALMITIS.
The aetiology of such inflammations is extremely varied. They may be
due to exogenous infections such as are not sufficiently virulent to give rise
to a suppurative reaction; more usually the cause is a systemic endogenous
infection, the most common probably being streptococcal or tuberculous ;
and a third somewhat heterogeneous group of cases follows as the result of
the absorption of some toxic material or of some irritative influence in the
eye itself.
In the ENDOGENOUS CASEs the symptoms are usually comparatively
mild, and the illness assumes a slow chronic form in which tissue-response
seems to be minimal, so that, without any dramatic interludes, the eye—and
usually both are affected—slowly goes blind. In the young such a picture
suggests a tuberculous or syphilitic aetiology, but the majority of patients
are past middle age, particularly women. In most cases of the latter type
the infection is probably streptococcal, and it is interesting that the great
majority suffer from rheumatic affections of all degrees up to completely
crippling arthritis. On the other hand many of the patients appear, even
on the most thorough examination, to be perfectly healthy.
The disease usually starts insidiously without marked pain or injection,
and the patient presents himself because of gradually failing vision at the
stage when posterior synechiae have already been formed, fine dust-like
keratic precipitates are present, a vitreous haze is obvious, and the trouble
has become well established. Pathologically, the entire uveal tract is
infiltrated with histiocytes, lymphocytes, and sometimes plasma cells, usually
agglutinated into clumps," and these in exudative form produce a haziness
in all the media. The synechiae slowly increase, a thin membrane covers
the pupil, the iris becomes atrophic early, the vitreous, cloudy with
opacities, rapidly degenerates into a muddy fluid filled with membranes,
and frequently, especially at the posterior pole, a sudden diminution of
vision is found to be due to a patch of chorio-retinitis dimly made out
through the turbid media. Sooner or later secondary changes appear in
the lens and the formation of a complete cataract is seldom long delayed;
1 p. 2177.
2252 TEXT-BOOK OF OPHTHALMOLOGY
and sometimes after a course of years a termination of the disease may be
brought about either by the development of secondary glaucoma or by
shrinkage and atrophy of the eye and a detachment of the retina.
The treatment of such cases—always a matter of difficulty, and fre-
quently of despair—has already been fully discussed."
Those cases which are due to the absorption of some toxic material in
the eye tend to have a similarly bad prognosis. A typical picture is seen
after the occurrence of repeated INTRA-ocular HAMoRRHAGEs or in the case
of INTRA-ocular NEoPLAs Ms especially when they have reached a necrotic
stage. In this event a relapsing irritant iritis, characterized pathologically
by actual necrosis of the uveal tissue (Fuchs, 1910) and clinically by marked
signs of inflammation including intense
ciliary injection and the appearance of
fibrinous and even purulent exudate,
may necessitate excision apart altogether
from the new growth. Such an occur-
rence in a blind eye associated with pain
and a detached retina should always
suggest the possibility of a necrotic
neoplasm ; nor should the presence of
keratic precipitates absolutely preclude
the neoplastic rather than the inflamma-
tory nature of a tumour the diagnosis
of which is in doubt. These questions
- will be discussed more fully at a later
Fig. 1883.-CHRonic Exudative stage.”
-
-
Uveitis,
Uveitis in retinal detachment with - - - - - - -
complicated cataract. A similar relapsing irritative condi-
tion may be seen in cases of long-
standing DETACHMENT OF THE RETINA, which is quite possibly due, as was
suggested by Birch-Hirschfeld (1912), to the irritative action of unusual
protein constituents in the intra-ocular fluid which reach it through the
retinal tear from the sub-retinal fluid. The inflammation frequently comes
on suddenly, as if some allergic element were present, associated with a
sudden fall in tension in an eye which has hitherto remained quiescent.
Pathologically a lymphocytic infiltration predominates (Fuchs, 1913); and
eventually a plastic exudate produces seclusion of the pupil (Fig. 1883),
or a sub-luxation of the lens results in a rise of tension. Treatment has little
effect, and, since recurrences invariably ensue, recourse must usually be had
to excision of the globe. This condition will be further noted in dealing
with retinal detachments.”
1 p. 2206. * p. 2511. * p. 2887.


DISEASES OF THE UWEAL TRACT 2253
ATROPHIC UWEITIS
An atrophic uveitis, which is presumably caused by the toxins liberated
from necrosis of the tissues in a blind degenerated eye, comes into the same
category and gives rise to a similar clinical picture. The inflammatory
reaction comes on periodically with exacerbations and remissions, the clinical
signs being frequently not particularly violent, but the pain and irritation
being unusually severe. Ciliary injection is associated with neuralgic pain
and lacrimation ; there is hyperaemia and sometimes a greenish discolora-
tion of the iris with the formation of synechiae, the anterior chamber is
usually deep owing to falling backwards of the lens, the aqueous muddy, and
the tension low. Eventually loss of perception of light indicates that the
choroid and retina are similarly involved. Since in most of these cases
subsequent atrophy of the globe is imminent, since treatment produces little
amelioration of the distressing symptoms which tend to persist indefinitely
or to recur periodically, and since a small but definite proportion of such
blind and painful eyes on pathological examination have been found to
contain a neoplasm, the most satisfactory treatment is to excise a useless
and troublesome organ.
TRAUMATIC UWEITIS
We have seen that even though no organismal infection appears to
have been sustained, traumata of all types may be followed by a recalcitrant
º --- º º º-
- º - º --- º *.
*…º.º.º.º.º.º.º. ºf
- --- ------- º - -
º -
Sºś
ſº-º- ºº:-
º
Round-celled infiltration in the choroid in post-traumatic uveitis after extrac-
tion of cataract (Collins, T. O. S.).
º -
-º-º- ---
º
--- ---
Fig. 1884.—CHRoxic UVEITIs.
1 p. 2135,





2254 TEXT-BOOK OF OPHTHALMOLOGY
and relapsing inflammation of the uveal tract of an irritative nature. Since
traumata are usually sustained by the anterior segment of the globe, the
inflammatory response is frequently, in its initial stages at any rate, confined
to an anterior uveitis, and the resulting irritative irido-cyclitis may be so
severe as to justify excision of the globe. In fact, some of the most serious
types of uveitis belong to this category, many cases developing a refractory
secondary glaucoma or rapidly running on to atrophy without showing the
stubborn resistance and capacity for resolution which so frequently
characterize the inflammations due to Organismal infections or constitutional
causes. The characteristic pathological appearance is the presence of
groups of small round cells throughout the entire uveal tract (Fig. 1884)—
the weitis maligna of older writers (Collins, 1891). Clinically the
inflammation is usually serous in type (the post-traumatic serous iritis of
Fuchs, 1913); and a very rare complication is the formation of a con-
siderable amount of fibrous tissue (Wood, 1932). While the eye is constantly
weak, watery and irritable, the most annoying characteristic is the
persistent occurrence of acute exacerbations, the symptoms clearing up only
to relapse again, each recurrence being accompanied by the same acute
pain and irritation, until eventually the eye is removed, a decision frequently
hastened by the fear of sympathetic inflammation in the other eye.
It is possible that some of these traumatic cases may be rendered more intractable
by an allergic sensitivity acquired to uveal pigment * or to lenticular proteins
(endophthalmitis phaco-anaphylactica) * : the possible aetiological influence of these
factors has already been discussed.
Birch-Hirschfeld. A. f. O., lxxxii, 241, Fuchs. A. f. O., lxxvii, 304, 1910; lxxxiv,
1912. 201, 1913.
Collins. R. L. O. H. Rep., xiii, 166, 1891. Wood. Brit. J. O., xvi, 546, 1932.
B. Specific Infections
1. HERPES
A full discussion of the aetiology of both forms of herpes and of our knowledge of
the virus (or viruses) concerned will be found in the section on corneal herpes.”
HERPEs simplex is frequently associated with an iritis which in many
cases is probably an irritative condition consequent on the corneal involve-
ment. In the most severe cases when deep ulcers are formed, the irido-
cyclitis may be correspondingly grave and may be associated with a
hypopyon ; while in the rare event of perforation a destructive irido-cyclitis
may ensue (Levin, 1933). The fact, however, that intra-ocular injection of
infective material derived from a diseased cornea produces an intense
irido-cyclitis and uveitis (v. Szily, 1924–27) suggests that the more severe
cases of intra-ocular inflammation which are observed from time to time are
determined by the virus itself, although an associated streptococcal infection
may occasionally be implicated. It is also of interest that after infecting
1 p. 2134. * p. 2133. * Vol. II, p. 1890.
DISEASES OF THE UVEAL TRACT 2255
the ciliary body of one eye of a rabbit with the virus, v. Szily (1924) noted the
development of a herpetic endocular inflammation in the other. The iritis
may be very acute initially, even haemorrhagic in type (Fig. 1885), the
tension may give rise to considerable anxiety, the inflammation may
be prolonged and obstinate in its course, and it has a tendency to relapse
without any corneal involvement (Gilbert, 1912).
In HERPEs zoster some iritic irritation and hyperaemia is the rule, but
when keratitis supervenes an irido-cyclitis, which may assume very severe
proportions, is invariable; occasionally it occurs without corneal involve-
ment, coming on a week after the skin eruption. It is typically exudative
and plastic in type, and keratic precipitates and synechiae are profuse.
Sometimes a very recalcitrant secondary glaucoma develops (Erdmann,
1909; Dubois, 1912; Weeks, 1917; Veasey, 1919), but more frequently the
Fig. 1885.-HERPET1c IRITIs.
tendency is towards hypotonia, while in the worst cases shrinkage of the
globe supervenes so that the eye may ultimately have to be excised for the
relief of pain (Paton, 1926).
More characteristic, but more rare, is the occurrence of eruptive lesions on
the iris corresponding to those on the skin, which were first described by
Machek (1895) (HERPEs IRIDIs): these are circumscribed swollen areas
characterized by acute vascular dilatation and complicated frequently by a
hyphaema. Their development is associated with considerable pain, there is
a violent iritis which takes a long time—sometimes 6 to 12 months—to heal,
and they leave as sequelae small white atrophic scars (VITILIGoIRIDIs) wherein
the stroma is thinned or even absent (Gilbert, 1910; Igersheimer, 1927;
Saint-Martin, 1929). Here again, probably owing to the vasomotor
instability associated with the herpetic lesion, an excess or diminution of
tension may give rise to much anxiety. Quite frequently an increase in the
severity of the inflammation accompanied by more profuse exudation and
T.O.-WOL. III. M

2256 TEXT-BOOK OF OPHTHALMOLOGY
a vitreous haze suggests a coincident infection of the ciliary body. The
clinical picture of the lesion is fairly characteristic, the main features being
the acute trigeminal pain, the localized iritic lesion and the hyphaema
(Machek, 1895; Bellwinkel, 1913 ; Roelofs, 1920; Schöppe, 1923; Löwen-
stein, 1923; and others).
Pathological eacaminations of these cases have been carried out by
Sattler (1874), Meller (1920) and Gilbert (1921), the characteristic lesion
being a marked and widespread infiltration around the ciliary nerves and
vessels. The peri-neuritis and peri-arteritis lead to a complete local necrosis
to which the haemorrhages are due, and it may be that the violence of the
inflammation is a reaction to the tissue-necrosis as well as to the infective
agent (Meller, 1920).
We have already seen that a sequel to herpes, even in cases which involved no
iridic inflammation, may be the persistence of a dilated immobile or sluggish pupil.
Wyss (1871), who found haemorrhages in the iridic musculature of such an eye, ascribed
the condition to muscular paresis following thrombo-phlebitis, but it seems more
probable that the majority of cases are due to neuritic involvement which may have its
seat outside the eye in the ciliary ganglion or more centrally (Baranov, 1922; Browne,
1931 ; Genet, 1934). -
Since there is no specific treatment for herpes, the conduct of these
cases should be on general lines as for iritis.” It sometimes happens, however,
that if the condition is caught early, a daily injection of 1 c.c. of pituitrin
sub-cutaneously for the first few days may abort or considerably mitigate
the disease (Niles, 1932; Somers and Pouppirt, 1935). Ruggles (1931)
claimed good results with sodium iodide. Protein shock has many
advocates, and sometimes is efficacious ; auto-haemotherapy is the most
popular remedy (Beeson, 1928; Barksdale, 1937), diphtheria anti-toxin
(5,000 units) sometimes produces an amelioration of the symptoms (Walker
and Walker, 1938), while Gifford (1937) recommended injections of
convalescent serum.
Baranov. T. O. S., xlii, 167, 1922. Machek. A. f. Aug., xxxi, 1, 1895.
Barksdale. Virgina Med. Monthly, lxiv, 378, Meller. Z. f. Aug., xliii, 450, 1920.
1937. Niles. N. Y. State J. Med., xxxii, 773, 1932.
Beeson. A. of Derm. and Syph., xviii, 573, Paton. Brit. J. O., x, 305, 1926.
1928. Roelofs. Ned. tij. v. Gen., lxiv (2), 2876, 1920.
Bellwinkel. Diss., München, 1913. Ruggles. A. of Derm. and Syph., xxiii, 472,
Browne. Med. J. Awstralia, ii, 621, 1931. 1931.
Dubois. K. M. Aug., 1 (1), 601, 1912. Saint-Martin. Bull. S. d’O. Paris, xli., 39, 1929.
Erdmann. Z. f. Aug., xxii, 30, 1909. Sattler. K. M. Aug., xii, 352, 1874.
Genet. Bull. S. d’O. Paris, xlvi, 234, 1934. Schöppe. A. f. Aug., xcii, 208, 1923.
Gifford. Handbook of Ocular Therapeutics. Somers and Pouppirt. California and West.
Phila., 1937.
Gilbert. K. M. Aug., xlviii (2), 24, 1910;
xlix (1), 649, 1911.
B. O. G. Heidel., xxxviii, 101, 1912.
A. f. Avg., lxxxix, 23, 1921.
Igersheimer. K. M. Aug., lxxix, 647, 1927.
Levin. Sov. vest. Oft., iii, 403, 1933.
Löwenstein. K. M. Aug., lxiv, 15, 1920;
lxxi, 313, 1923.
* Vol. II, p. 1905.
Med., xlii, 370, 1935.
v. Szily. B. O. G. Heidel., xliv, 61, 1924.
R. M. Aug., lxxviii, Beil. 11, 1927.
Veasey. T. Am. O. S., xvii, 297, 1919.
Walker and Walker. A. of O., xx, 304, 1938.
Weeks. A. of O., xlvi., 460, 1917.
Wyss. A. f. Heilk., xii, 261, 1871.
* p. 2206.
DISEASES OF THE UWEAL TRACT 2257
2. GONORRHOEA
The occurrence of a severe iritis during an attack of gonorrhoea was first
pointed out by Sir William Lawrence (1830), whose observations were con-
firmed in minute and exact detail by Mackenzie (1833). From this early date
clinical observations were recorded so numerous as to put the association of
the two beyond doubt, even before the discovery of the gonococcus by
Neisser in 1879 : the sequence of gonorrhoea, arthritis and iritis was too
frequent to be coincidental, while the re-appearance of iritis with subsequent
gonorrhoeal infections in the same individual (Ruckert, 1886) pointed
unmistakably to the same conclusion. A further notable clinical observation
was made by Griffith (1900) who suggested that not only might iritis be a
complication of this infection but also a late and sole sequel ; and the matter
has finally been clinched by the production of bacteriological proof by
Sidler-Huguenin (1911) who isolated gonococci from the prostatic secretion,
the blood and the exudate from the anterior chamber in a typical acute case
of the disease.
Incidence. There is no doubt that gonorrhoeal iritis is a common
condition, forming a considerable proportion particularly of the recurrent
irites which used to be associated with rheumatism. The estimate of its
incidence varies ; most authors conclude that it accounts for about 5% of
all cases of iritis (3% Gilbert, 1929; 5% Irons and Brown, 1923; Bulson,
1925; Gifford, 1931; 6% Butler, 1911 ; 8% Yeld, 1901), but some would
put it higher—16 out of 37 cases of iritis (Goulden, 1914). It is almost
exclusively a disease of males, and thus corresponds in its incidence with
arthritis. In Byers’ (1907) series of 109 cases of gonorrhoeal uveitis only 4
were females, a proportion which is borne out in the experience of all other
observers, and is probably dependent on a causal association with infection
of the prostate and seminal vesicles.
The time of onset is also of importance. The eyes become affected only
when the disease has spread to the deep urethra, and iritis is usually a
complication of a chronic prostatitis or vesiculitis; it never occurs during
the acute stage of anterior urethritis (Sidler-Huguenin, 1911; v. Hippel,
1917; Browning, 1920; and others). It therefore does not occur until
some weeks (4 to 6) or months after infection, and then it usually follows
closely on other evidences of systemic spread, particularly affections of the
larger joints, especially the knees and ankles. It need not, however, be
associated with arthritis and may precede it (10 out of 109 cases, Byers,
1907). On the other hand, there seems to be no limit to the time when
an attack or a relapse can occur subsequent to infection ; a period of 5 to 10
or more years is quite common before the initial attack, and Kravitz (1936)
reported an undoubted case wherein the trauma of a cataract extraction
flared up an irido-cyclitis in the smears from which gonococci were
discovered 60 years after the original infection. Moreover, the frequency
M 2
2258 TEXT-BOOK OF OPHTHALMOLOGY
of persistent recurrences over an indefinite period of years indicates the
presence of a source from which infective materialisintermittently supplied,
and is probably accounted for by the astonishing viability of the gonococcus
in the posterior urethra and its associated organs. This peculiarity is
perhaps associated with the intra-cellular habitat of the organism which
thus lives symbiotically in the tissues. It may, indeed, be said that the
chronicity of the uro-genital infection is such that it is without time-limit,
and one case at least has been authenticated of an acute exacerbation of a
latent urethritis following a prostatectomy 50 years after the original
infection (Fraser and Dye, 1935).
Once one eye has been affected the tendency is for relapses to occur
and for the other to be involved also. It may occur simultaneously in both
eyes, and relapses may be bilateral (Byers, 1907); but this is not the rule.
The ocular involvement is part of a
systemic infection but there is some doubt
as to its precise nature. Many writers
infer that, like the associated arthritis, it
is metastatic in nature, and in support
of this it is advanced that endogenous
gonococcal conjunctivitis has been proved
to be on occasion metastatic (Morax,
1894; Sidler-Huguenin, 1911; Davids,
1914)," while in one case at least the
organism has been recovered from the
eye (Sidler-Huguenin, 1911). It is to be
remembered, however, that in the hands
of others, cultures have been negative
Fig. lºº (Byers, 1907; Browning, 1920) and that
this case was one of septicaemia with a
haemorrhage in the anterior chamber. On the other hand, the frequency
with which a fulminating attack resolves leaving few sequelae, and the
rapid cures which may on occasion follow treatment suggest that the
inflammation may be toxic in nature.
Gonorrhoeal infections may assume several forms, the most typical of
which are as follows:–
i. SIMPLE IRITIS. This is a typical attack of acute iritis with all the
characteristic symptoms of this disease except that corneal precipitates are
rarely seen and the tendency to form synechiae is slight. Even in the
absence of efficient treatment the adhesions usually disappear leaving only
a few dots of uveal pigment on the lens. Relapses, however, are the rule.
ii. Exudativº IRIDo-cycliſts. This forms a clinical picture which
may be said to be characteristic of gonorrhoea; it is the type ofiritis originally
described by Mackenzie (1833), but is not the most common (13 cases in 112,
* Vol. II, p. 1546.

DISEASES OF THE U WEAL TRACT 2259
Byers, 1907). There is a violent irido-cyclitis usually without corneal
precipitates, but characterized by a profuse exudation into the anterior
chamber sometimes almost filling it. Most typically this is fibrinous in
nature and forms a grey translucent gelatinous mass appearing somewhat like
a dislocated lens (Fig. 1886). More rarely exudative cells are sufficiently
in evidence to form a small hypopyon, and more frequently the extreme
hyperaemia of the iris leads to the formation of actual haemorrhages into the
anterior chamber and the development of a haemorrhagic iritis. In either
case the immediate prognosis under efficient treatment is good, for by the
end of 6 to 8 weeks all traces of exudation are usually gone, the media are
clear and restoration of vision is complete. This unexpected completeness
of recovery is a characteristic feature of this type of iritis; but unfortu-
nately the tendency to relapses, especially of the plastic type, is very
considerable and in the absence of treat-
ment almost invariable ; rarely complica-
tions such as a papillitis or an acute
choroiditis introduce a disturbing element
(Thomasson, 1923).
iii. PLASTIC IRIDo-CYCLITIs. This, the
type originally described by Lawrence
(1830), is the most common, the most
stubborn, and carries with it the worst
prognosis. It is a violent irido-cyclitis
characterized by the absence of keratic
precipitates and the rapid development of
broad and extensive synechiae, which even
under the most energetic treatment may
prevent pupillary dilatation (Fig. 1887).
Recurrences are the rule, and ultimate seclusion of the pupil and involve-
ment of the ciliary body frequently result in nutritional changes and
eventual shrinkage of the globe.
Fig. 1887.-PLASTIC GonococcAL
IRIDocyclitis.
iv. A mild iritis associated with an endogenous conjunctivitis is a rare event."
The conjunctival complication is itself uncommon (1% of gonococcal cases, Heerfordt,
1910), and of these about one third show a diffuse corneal opacity, a hyperaemia of the
iris, and a turbidity of the aqueous (Byers, 1907). Exceptionally, more severe mani-
festations occur, such as a ring abscess and hypopyon (Apetz, 1903).
The diagnosis of gonorrhoeal iritis can often be made clinically by the
pathognomonic picture presented by the exudative type of inflammation ;
and it should be suggested in every case of recurrent iritis in the male. The
points upon which importance should be stressed are the history of previous
gonorrhoea, the frequent (but not invariable) association with gonorrhoeal
Vol. II, p. 1546.

2260 TEXT-BOOK OF OPHTHALMOLOGY
rheumatism, the recurrence or exacerbation of symptoms after prostatic or
vesicular massage, and the frequently good reaction to gonococcal vaccines.
In suspicious cases the diagnosis is clinched by the demonstration of
gonococci in the deposit of the centrifugalized urine or in the prostatic
discharge, while supplementary evidence—although a negative result cannot
be accepted as conclusive—may be obtained by a complement-fixation test
of the blood (Gault, 1935).
Local treatment on the usual lines (heat, leeches, atropine, etc.") should
be prompt and energetic, but the most important element is the possibility
of cutting short the attack and preventing subsequent relapses by systemic
measures. The most efficient drug to employ is sulphanilamide, which will
sometimes clear up a violent inflammation in a few days; but this is only a
temporary measure and a cure with any hopes of permanency must be based
on specific treatment of the disease. Immediate treatment by vaccine is
sometimes dramatic in its effects (v. Hippel, 1917; Browning, 1920; and
others), and it should be given in large quantity at the earliest possible
moment (50 to 500 millions as an initial dose). After the acute stage has
been passed every endeavour should be made to eliminate the source of the
infection in the region of the deep urethra and thereby prevent the usual
history of persistent and ever-recurring relapses. At the least this involves
a long course of prostatic massage and a very prolonged course of vaccine :
it is useful to remember that if prostatic massage tends to excite focal
relapses in the eye, these may be aborted by the prophylactic administration
of sulphanilamide. Finally, after the local conditions appear to have com-
pletely subsided, periodical examinations should be made by a urogenital
specialist, and on the least sign of further trouble treatment should be
conscientiously repeated. It is well to insist again that there is no limit to
the chronicity of a latent gonococcal infection, and if recurrences of an iritis
are to be forestalled there must be no limit to the period of vigilance.
Attention has already been drawn to the occurrence of a non-gonococcal
prostatitis giving rise to an iritis º. B. coli have been found (Browning, 1920), but
streptococci are more common (Irons and Brown, 1923; Green, 1926; Benedict,
v. Lackum and Nickel, 1926). Some authors, indeed, particularly in America, go so
far as to say that of cases of iritis due to prostatic infection the non-gonococcal cases
are more frequent than the gonococcal (Zentnayer, 1926), an Opinion vigorously denied
by others (Browning, 1920).
The literature up to 1872 has been collected by Koeniger, up to 1907 by Byers,
up to 1914 by Goulden.
Münch. med. W., l, 1340, 1903.
T'. Am.
Apetz.
Benedict, v. Lackum and Nickel.
O. S., xxiv, 145, 1926.
Browning. Brit. J. O., iv., 102, 1920.
Bulson. T. Am. O. S., xxiii, 292, 1925.
Butler. Brit. Med. J., i, 804, 1911.
Byers. The Ocular Manifestations of Systemic
Gomorrhoea. Studies from the Victoria
Hospital, Montreal, ii, No. 2, 1907.
1 p. 2206.
Davids. A. f. O., lxxxvii, 160, 1914.
Fraser and Dye. J. Am. Med. As., cv, 269,
1935.
Gault. Bull. S. d’O. Paris, xlvii, 273, 1935.
Gifford. Am. J. O., xv, 100, 1931.
Gilbert. Kurzes Hb. d. O., Berlin, v, 2, 1929.
Goulden. R. L. O. H. Rep., xix, 354, 1914.
Green. J. Am. Med. As., lxxxvii, 1183, 1926.
Griffith. T. O. S., xx, 83, 1900.
* p. 2146.
DISEASES OF THE UVEAL TRACT 2261
Beerfordt. A. f. O., lxxii, 344, 1909; lxxvii, Mackenzie. Treatise on Diseases of the Eye.
145, 1910. 1833.
v. Hippel. A. f. O., xciv, 355, 1917. Morax. Thése de Paris, 1894.
Irons and Brown. J. Am. Med. As., lxxxi, Ruckert. K. M. Aug., xxiv, 339, 1886.
1770, 1923; lxxxvii, 1167, 1926. Sidler-Huguenin. A. f. Aug., lxix, 346, 1911.
Koeniger. Diss., Berlin, 1872. Thomasson. A. of O., lii, 546, 1923.
Kravitz. Am. J. O., xix, 328, 1936. Yeld. Brit. Med. J., ii, 1331, 1901.
Lawrence. Treatise on Venereal Dis. of the Zentnayer. Am. Med. As., T. Sec. O., 216,
Eye, London, 1830. 249, 1926.
3. SYPHILIS
Although—in Great Britain at any rate—the manifestations of syphilis
are not so common as they used to be, largely because of the great improve-
ment in the methods of treatment and the education of the population in
availing itself at an early stage of its advantages, yet this disease must be
classed as one of the common causes of uveitis ; in some countries, particu-
larly in the tropics, and among some classes, such as negro populations, it is
probably the commonest cause. The most varied statistics as to its
incidence are given in the literature.
Thus in England, Butler (1911) claimed a luetic aetiology in 22% of cases of iritis,
Lang (1913) 6%, and Goulden (1914) 9% ; in America, Irons and Brown (1923) gave
19%, Bulson (1925) 33%, Newton (1925) 13%, Gifford (1931) 16.9%, O'Leary (1932)
6%, and Woods and McCormick (1937) 8% ; while on the Continent, Arlt (1853) gave
17%, Elschnig (1925) 20%, and Gilbert (1930) 16.6%. Some much higher estimates
have been claimed : Campbell (1905) and Bruns (1925) 70%, and Clapp (1921) 82% of
hospital cases of iritis and 69% of private cases. Weiss (1925) estimated that syphilitic
iritis constituted 0.34% of all eye diseases, 13% of all syphilitic eye diseases, and 55% of
all cases of iritis. The aetiology is, of course, not always clear ; thus Irons and Brown
(1923), who concluded that 6% of cases of iritis were certainly syphilitic, found another
13% of their cases where syphilis was probably acting in combination with other factors,
i.e., that 19% occurred in syphilitics. Holloway (1931) put this figure at 20 to 35%.
Igersheimer (1928), who made a very thorough study of the subject, found 28.9% of
cases of acute iritis, and 8.1% of chronic irido-cyclitis in syphilitics.
Conversely, the proportion of syphilitics who develop uveitis is relatively high.
Groenouw (1904) found that 3.2% of syphilitics have ocular involvements and of these
50% had uveitis : while Moore (1931) found iritis in 5% of all patients with early
secondary syphilis, and almost twice as frequently with recurrent secondary syphilis
(9%) and again frequently with late syphilis, in which it occurred in 3% of cases.
With adequate treatment these figures are considerably lowered but not abolished.
Thus Stokes (1932) found the early cases reduced to 0.4%, but it is of great interest
that with inadequate treatment this figure rose to 2.6%. It is somewhat more common
in male than in female patients (53 : 47% Weiss, 1925), and twice as common among
black than white syphilitics (Clapp, 1921 ; Zimmermann, 1924).
Eacperimental animal eacperiments upon the incidence of uveal syphilis have not had
very noteworthy results. As we have already noted,” a considerable amount of research
has been done since the original experiments of Bertarelli (1906), particularly on the
pathology of interstitial keratitis, after intra-testicular or intra-arterial inoculation
with treponemes, the most important recent papers being those of Igersheimer (1922),
Brown (1922), Chesney and Kemp (1925), Löwenstein (1927–29), Shibata (1929),
1 Vol. II, p. 1966.
2262 TEXT-BOOK OF OPHTHALMOLOGY
Grigoriew (1929), Clapp (1929–33), and Funabashi (1930–33). The majority found that
an iritis was neither constant nor marked, but on the other hand Brown and Pearce
(1921) produced an iritis as frequently as a keratitis. While the lesion may be severe,
with a hyphaema, much exudation, and the formation of syphilomatous nodules, it is
usually fleeting, and seems to depend essentially on damage to the blood-vessels, the
walls of which become infiltrated with lymphocytes and plasma cells. Experimental
work is also interesting in indicating that in the chorio-retinal manifestations the
primary seat of infection is the choroid, but here again detailed observations are few
(Nichols, 1914; Igersheimer, 1918; Cattaneo, 1924), for infections of the posterior
segment are rare in experimental syphilis.
In general terms it may be said that syphilitic affections of the uveal
tract follow the same course as those of the body generally. The whole
question of immunity in syphilis has been widely studied both experi-
mentally (Chesney, 1927–30; Zinsser, 1931) and by the observation of
large numbers of patients, treated and untreated (Zimmermann, 1924;
Brunsgaard, 1929; Moore, 1931; and others). The clinical manifestations
of the disease can be divided into two categories, early and late. The early
symptoms correspond with the generalized systemic infection which con-
stitutes the secondary stage, and, as we have seen, uveal inflammation occurs
in about 5% of these cases. Once this stage has passed, as a rough average
it may be said that patients who have received no treatment may be divided
into four approximately equal categories. In 25%, after the early generalized
outbreak a complete cure seems to be achieved in that no further clinical
evidences develop and the Wassermann reaction remains negative. In
25% no further clinical evidences develop but serological evidence of
infection exists in that a positive Wassermann reaction remains. In 25%
tertiary fibrotic and degenerative parenchymatous lesions develop affecting
especially the cardio-vascular and central nervous systems; while the
remaining 25% seem to become sensitized so that, in the presence of few
organisms, late violent inflammatory reactions occur : to this last group
belongs the majority of cases of late uveal syphilis.
From the clinical point of view uveal syphilis presents a great variety
of heterogeneous pictures, but the pathology underlying them all is very
similar. Nor is there any really fundamental difference between the
manifestations of the inherited and the acquired type of the disease. It is
convenient, however, to study these conditions in separate categories although
it must be remembered that the variations are quantitative rather than
qualitative, one type frequently merges into the other imperceptibly, and
that more than one may co-exist.
A. Acquired Syphilis
i. ROSEOLA OF THE IRIS
Roseolae of the iris are rare, but they may form the first manifestation
of syphilis in the eye, and indeed they may be the first indication of the
PLATES XXXVIII AND XXXIX
|UVEAL SYPHILIS
[To face p. 2262.
PLATE XXXVIII
Syphills of THE ANTERior Segment
Fig. 1888.-EARLY Roseola. Fig. 1889.-LATE Roseola.
Fig. 1890–CILLAR v Gumnia. Fig. 1891–ATRoetry Arrºr a
GRANULoMatous LEston.
Fig. 1892–Papuan IRIris (Neame).



PLATE XXXIX
SYPHILIs of THE PostERIon Segment
FIG. 1893.-EARLY Disseyſis ATED FIG. 1894.—LATE Dissesſinated
CHOROIDITIs, CHoRoibitis (same case as Fig. 1893).
Fig. 1896.-PERIPHERAL CHoRoidITIs. Fig. 1897.-ATRoPHIC STAGE of
DIsse MINATED CHoRoidITIs.


DISEASES OF THE UVEAL TRACT 2263
generalized systemic infection. Essentially they are a mild and transient
vascular and tissue-reaction to small treponemal capillary emboli. They
occur in the initial stages of the secondary period, perhaps as early as the
6th week after the primary sore (Krückmann, 1907), and are frequently
associated with or followed by a macular eruption on the skin.
THE EARLY RosBOLA of the iris (Fig. 1888, Plate XXXVIII) is merely a
hyperaemia affecting the already existing superficial vascular loops of the
iris, producing a bright red engorgement which contrasts with the copper
tint of the skin eruption. Situated preferentially about the middle third of
this tissue, the hyperaemia appears quickly, lasts usually a few days without
producing any symptoms or visual loss, and then disappears leaving no
trace behind. The earliest, least severe, and most fleeting of specific
manifestations in the eye, it is probably usually unobserved, and is perhaps
seen more frequently by the dermatologist than the ophthalmologist.
RECURRENT ROSEOLAE. In contra-distinction to the early roseola of the
iris, a similar hyperaemia may appear at a later stage, up to the second year
after infection—RECURRENT ROSEOLAE (Fig. 1889, Plate XXXVIII). In place
of being a generalized vaso-dilatation they form localized patches and are
frequently associated with inflammatory symptoms, exudation, and the
formation of synechiae, and they may persist to develop into typical papules.
ii. SYPHILITIC IRIDO-CYCLITIS
Syphilitic irido-cyclitis occurs typically at two epochs in the natural
history of the infection—at the early secondary stage and as a late tertiary
lesion. In addition, it occurs in association with arsenical treatment of the
disease, either as a flare-up following the first injection of the drug (the
Jarisch-Herxheimer reaction), or as a recurrent irido-cyclitis (recidive
reaction) following inadequate treatment.
(a) SECONDARY syPHILITIC IRIDO-CYCLITIS. A severe and acute irido-
cyclitis is not uncommon in the early secondary stages of syphilis : thus
among 10,000 syphilitics studied by Moore (1931) at the Johns Hopkins
Hospital, the expectancy of its development with the generalized secondary
outbreak was 4.5%. Only exceptionally does it appear before the third
month after infection (Krückmann, 1907), although Widder (1881) reported
a case at 5 weeks ; the maximum incidence is between the 4th and 6th
months. In three-quarters of the cases it is associated with a skin rash,
usually of the folliculo-papular or maculo-papular type, and in about one
quarter of the cases it is the earliest manifestation to appear after the
chancre, antedating the generalized outbreak by 1 to 14 days (Moore, 1931).
The affection is usually unilateral but in a large proportion of cases (45%)
both eyes are attacked, although rarely simultaneously. The inflammation
is severe as a rule and may indeed be fulminating in intensity or haemorrhagic
2264 TEXT-BOOK OF OPHTHALMOLOGY
in character (Rollet and Rosnoblet, 1923), but although permanent damage
may occasionally be done, the prognosis is generally good, the persistence of
sequelae such as impairment of vision being unusual and blindness being an
exceptional result. Nor, once it has occurred and has been adequately
treated, does the inflammation tend to relapse.
(b) TERTIARY syPHILITIC IRIDO-CYCLITIs, occurring in the tertiary
stage of the infection is somewhat rarer than the early secondary type, but
nevertheless in Moore's (1931) series of cases it was about equally common—
109 instances out of 249 cases of iritis in 10,000 syphilitics. It may occur in
the first or second year of the infection, and its appearance may be delayed
for 20 or more years (50 years, Schubert, 1881), but its onset averages
10 years after the primary chancre. Some authorities state that it is
usually an isolated phenomenon of the disease (Igersheimer, 1918;
Zimmermann, 1924), but in his comprehensive series Moore (1931) found that
approximately half the cases were complicated by other syphilitic mani-
festations such as tertiary skin or mucosal lesions, bone lesions, cardio-
vascular disease, or neuro-syphilis. The inflammation may be mild or very
severe indeed, and, in contrast to the early type, relapses are common even
when adequate treatment is administered. The prognosis is considerably
more serious than in the former variety for permanently damaging sequelae
are not unusual, and, especially when several relapses have occurred, resultant
blindness is not unknown.
(c) THE JARISCH-HERxHEIMER REACTION is a relatively frequent
phenomenon consisting of the development of an acute iritis in a previously
uninvolved eye 24 to 48 hours after the first therapeutic injection of arsenic.
The reaction is part of a sudden general systemic intensification of the
syphilitic lesion. It is assumed to be due to the death of a large number of
treponemes with the liberation of their endotoxins, or perhaps, to the
sudden release of inflammatory products from infected tissues in the course
of resolution. It is of no great clinical importance in so far as it always
clears up rapidly on continued anti-syphilitic treatment.
(d) RECURRENT SYPHILITIC IRIDO-CYCLITIs (RECIDIVE REACTION) is a
relatively rare phenomenon largely because recurrent syphilis itself is rare : it
occurred 29 times in Moore's (1931) series of 249 cases of irido-cyclitis in
10,000 syphilitics. It is usually unilateral, but occurs in both eyes in about
17% of cases. The inflammation follows inadequate anti-syphilitic treat-
ment and comes on as a rule some 4 to 6 months after its cessation, although
its appearance has been delayed 44 years after the last arsenical injection
(Moore, 1931). It does not usually follow an early secondary iritis but rather
appears as a new phenomenon, and it is extremely rare for a recurrent iritis
to be followed by a subsequent relapse. It is unusual for it to be accom-
DISEASES OF THE UVEAL TRACT 2265
panied by muco-cutaneous lesions, but some 15% of cases are associated
with evidence of neuro-syphilis and meningeal involvement. The resultant
inflammation is as serious—but no more so—than the early irido-cyclitis;
50% are mild, 50% are severe, and occasionally they are of fulminating
onset and devastating severity so that blindness or phthisis bulbi may result.
It has been argued that this recurrent inflammation is due to a fresh generalization
of organisms with a fresh invasion of a previously uninvaded eye ; but the facts that
irido-cyclitis is relatively twice as frequent a manifestation of recurrent syphilis as of
secondary syphilis, that it is usually unilateral and unassociated with signs of general
systemic infection but frequently with meningeal infection, and that at the time of
the relapse the blood Wassermann is usually negative, suggest that the ocular and
meningeal inflammations are of a similar nature. Zimmermann (1928) supposed that
sub-sterilizing treatment with arsenic destroys all or most of the treponemes except
those in the central nervous system or the eye, for into these two regions this drug
enters only with difficulty.” Normally, if an infection is allowed to run its natural
course, the patient develops a certain degree of immunity and spontaneously heals all
his secondary lesions, so that he enters into a stage of latency which persists for a short
or indefinite period, or even for a lifetime. The rapidity with which the anti-Spirochaetal
drugs kill off the organisms, however, prevents the patient from developing his own
immunity as would occur in an untreated patient, so that the foci or organisms left in
the eye and the central nervous system are at liberty to multiply with as little hindrance
as if they had been freshly introduced from without. The consequence is that after a
suitable incubation period, an irido-cyclitis ensues.
The typical case of syphilitic irido-cyclitis is ushered in by headache,
acute pain in the eye and rapid loss of vision, the eye becomes tender and
congested, and the iris is acutely hyperaemic, swollen, sodden and oedematous.
The inflammation is a diffuse one affecting the entire tissue of the iris,
superficial and deep, and involves much swelling ; but its main character-
istic is the profuseness of the fibrinous exudate (FIBRINOUs IRITIs). This
sometimes shows itself in the form of fine fibrinous threads lying on the iris
or as a thin coating over its surface, and always gives rise to a muddy
aqueous and a profuse deposition of fine star-shaped precipitates on the
posterior surface of the cornea. Fibrinous adhesions usually bind the iris
to the lens, and occasionally the inflammation may be still more violent so
that a gelatinous exudate may fill the anterior chamber (Schmidt, 1871) or
a hypopyon (Schmidt, 1872), or a hyphaema (v. Schroeder, 1880) may form.
Pathologically, as we shall see presently, the iritis is always of the
nodular type, but usually the nodules are so small as not to reveal themselves
on clinical examination. Sometimes in the boggy Oedematous tissue, small
nodules are seen with the slit-lamp (Swatikowa, 1927), and, much more
significantly, localized discoloured swellings near the pupillary margin in
association with which are broad thick synechiae formed, not by an adhesion
of the posterior epidermal layer with the lens capsule, but of the mesodermal
tissue. This is a peculiarity of syphilitic iritis and when the pupil is dilated
1 Vol. I, p. 439 (442).
2266 TEXT-BOOK OF OPHTHALMOLOGY
it gives it a characteristically notched appearance. More rarely, in some
5% of cases, these localized nodular lesions assume much larger proportions
and bulge out of the iris tissue to form typical syphilomata.
While many of the cases run an uncomplicated course, complications
are not at all uncommon. Some vitreous opacities, indicating a ciliary
inflammation, are the rule except in the mildest cases, and quite frequently
the inflammation does not confine itself to the anterior segment of the uveal
tract but spreads to become a complete wweitis. An anterior choroiditis is
common, and although pupillary exudates and a vitreous haze make
examination of the fundus in most cases impossible, an involvement of the
entire uveal tract is indicated by the presence of large scotomata in the
visual field which can be mapped out with strong light stimuli (Schnabel,
1876). Moreover, the occasional presence of a ring scotoma continuous with
the blind spot betrays a coincident affection of the nerve fibres (Förster,
1874). If the fundus can be seen choroiditic changes are obvious, and with
them may be associated a secondary neuro-retinitis, or a swelling of the
nerve-head indicating the presence of an optic neuritis which may result in
some degree of secondary optic atrophy. Syphilitic vascular changes in the
retina are also not uncommon. Corneal involvement in the form of an
interstitial keratitis, taking the form of a diffuse opacity infiltrating especially
the marginal regions, is an occasional feature, (Coccius, 1870; Schmidt,
1871–72; and many others), and, much more rarely, a localized deep
punctate keratitis (Mauthner, 1875; Baumert, 1927; Bryn, 1927; Weill,
1927; and others)." The marginal interstitial keratitis is avascular and
responds well to treatment. Cataract may also occur, and, finally, a
generalized and massive exudation may lead to a detachment of the retina
and shrinkage and loss of the eye (Nettleship, 1871 ; Delafield 1871 ; and
many others).
Late complications affecting the retina and optic nerve some months or years after
a syphilitic irido-cyclitis may sometimes present a characteristic picture called
NEURITIS PAPULOSA” by A. Fuchs (1926). The vitreous is full of opacities, and a grey
exudative mass projects into it from the disc, producing a Scotoma in the field varying
from an enlarged blind spot to a complete central defect (Fuchs, 1916; Satanowsky,
1932). Histological examination of such eyes shows the characteristic changes of
chronic irido-cyclitis in the anterior segment, and in the posterior segment a round-
celled infiltration associated especially with the retinal vessels when the arteries are
encircled by a cuff of lymphocytic elements and their lumen obstructed by proliferating
endothelium. In the retina and over its surface there is much fibrinous exudate which
becomes converted into fibrous tissue arranged as in a proliferating retinitis.
iii. SYPHILITIC CHORIO-RETINITIS
Choroiditis, or CHORIO-RETINITIs, since the retina is almost invariably
affected in association with the choroid, sometimes secondarily, sometimes
1 Vol. II, p. 1986. * p. 2664.
DISEASES OF THE UVEAL TRACT 2267
simultaneously, and, indeed, sometimes primarily (Fuchs, 1916), is relatively
common in acquired syphilis. Such cases, however, probably constitute a
relatively small proportion of the total incidence of choroiditis in adults—
some 8% (Gilbert, 1930) to 16% (Igersheimer, 1928)—a sharp contrast to the
fact that the vast majority (83%) of cases of choroiditis occurring in children
are due to congenital lues. Clinically there are no pathognomonic features
in the picture presented by the disease, and almost every type of choroiditis
may have this origin.
(a) A DIFFUSE CHOROIDITIS has been described by Krückmann (1903) occurring
early after infection, sometimes as early as the first month. He described it as
characterized by a widespread grey disturbance of the fundus, which disappears On
treatment leaving no trace except perhaps fleck-form areas of superficial choroidal
atrophy, and compares it to a roseola-like rash.
The common and typical lesions, however, occur in the late secondary
and tertiary stages of the disease. They are circumscribed in distribution,
being either disseminated or localized.
(b) DISSEMINATED SYPHILITIC CHORIO-RETINITIS. This, the most
common manifestation of chorio-retinal syphilis, originally noted by
Jacobson (1859), was first adequately described by Förster (1874) in a
classical work upon which all subsequent writing has been based. It appears
typically in the late secondary stages of the disease, but its onset may be
delayed for 10 or more years after infection. In about half the cases it is
bilateral, the involvement of one eye following quickly that of the other.
Starting with a diminution of vision due to the formation of a cloud of
vitreous opacities, and visual phenomena of metamorphopsiae and
photopsiae, greyish-yellow areas of acute inflammation are seen in the fundus,
while with difficulty through the hazy media, retinal oedema and a haziness
of the disc may be made out. The inflammatory foci may be few or
numerous, and in many cases these become confluent, occupying large areas
of the fundus and assuming the most polymorphic shapes (Figs. 1893–4,
Plate XXXIX). As a rule the posterior segment of the fundus is
preferentially affected, and a characteristic of syphilitic disease is the
frequency of an involvement of the area round the disc, leaving eventually
a ring-like atrophic areola round its margin.
The almost invariable involvement of the retina makes the proliferation
of pigment of the retinal type a prominent feature. The pigment may be
found in great irregular or grouped masses, or in feathery and corpuscular
aggregations resembling pigmentary degeneration, or arranged in ring-
formation, and quite often it is gathered round the larger vessels. A final
feature which is seen occasionally in the later stages of the disease is the
presence of white fibrous tissue, sometimes in large sheets, lying between
the choroid and the retina, over which the retinal vessels course normally
(Doyne and Stevenson, 1905).
It is to be noted that there is no pathological or clinical difference
2268 TEXT-BOOK OF OPHTHALMOLOGY
between the smaller discrete lesions and the larger confluent areas, and
it is quite possible, as Rönne (1934) suggested, that in all cases there is
a diffuse infiltration of the chorio-capillaris which in certain localities
becomes more intense so that it breaks through Bruch's membrane, destroys
the pigmentary epithelium and becomes ophthalmoscopically visible. Such
areas may be small or large, and may attain their maximum activity at
different times so that the visible areas of inflammation may appear to
develop in sequence or in crops. Fuchs (1916) obtained histological evidence
for this view in a case which showed clinically a few sparse disseminated
areas, but on microscopical examination showed a complete infiltration over
the choroid. In such an event Krückmann’s (1903) contention that the
|FIG. 1898.--SYPHILITIC CHOROIDITIS.
inflammatory areas in the choroid are analagous to isolated papules, is
incorrect. According to Rönne (1934), this accounts for the great variations
seen in the final fundus picture, which depends on the places and extent of the
perforations by the choroidal infiltration, on the degree of consequent
pigment proliferation, and on the subsequent atrophy and vascular sclerosis
in the choroid.
The course and development of the disease are extremely varied. Some-
times it dies down rapidly leaving little or no defect apart from localized
scotomata, which, provided the macular region is untouched—as it often is-
do little harm. On the other hand, activity may continue over a long period,
fresh lesions appearing or old lesions spreading. Moreover, a cyclitis may
develop, and a complete and recalcitrant uveitis result (the syPHILITIC
UVEITIs of Förster, 1874). A feature of syphilitic chorio-retinitis is the
definite tendency to recurrences after the disease has lain dormant for a

DISEASES OF THE UVEAL TRACT 2269
year or more ; and occasionally the recurrences are more serious than the
initial illness in that they may be complicated with an anterior uveitis.
For this reason the prognosis should always be guarded.
In the acute stages the damage to the retina may produce a much more
widespread scotoma than the Ophthalmoscopic picture would seem to
warrant, and a very typical phenomenon is the appearance of a ring scotoma
(Fig. 1898) indicating involvement of the nerve fibres (Förster, 1874;
Rönne, 1934). Such a scotoma in choroiditis, resembling rather that
associated with an optic neuritis or neuro-retinitis, is characteristic of
syphilitic disease in the acute stage, and it may be sufficient to suggest the
diagnosis even when the ophthalmoscopically visible changes in the
fundus may not be sufficiently typical to do so. As a rule the ring scotoma
does not persist, for the fields of old cases tend rather to resemble those of
non-specific fundus diseases, the Scotomata corresponding to the areas of
atrophy. If diffuse atrophy of the retina results, the peripheral field may
be completely abolished and only a small central area left around the
fixation point, a condition associated with night-blindness, which, being
usually characterized by much pigmentary degeneration, may be termed
a secondary retinitis pigmentosa. Finally, vision may be seriously impaired
by the development of some degree of optic atrophy, which, although it
does not occur as often in acquired as in congenital syphilis, complicates
quite a number of cases. -
(c) A PERIPHERAL (ANTERIOR) CHOROIDITIS is much rarer in acquired
syphilis than the type wherein the lesions are situated in the region of the
posterior pole. It usually occurs as small fleck-form areas in conjunction
with an irido-cyclitis and especially with an interstitial keratitis, a condition
usually, but not invariably, associated with inherited disease.
(d) The LOCALIZED TYPES OFSYPHILITIC CHOROIDITIs are rarer and usually
occur at a later stage in the history of the disease than the disseminated.
They have no clinical attributes to distinguish them from similar lesions
caused by other organisms. They may occur anywhere in the fundus,
including the region of the disc (CHOROIDITIs JUXTA-PAPILLARIs) (Fig. 1869,
Plate XXXVI) (Verhoeff, 1916; Köhne, 1920; Knapp, 1920; Zeeman, 1921;
Stoewer, 1924; Seefelder, 1924; and others), or of the macula (CENTRAL
CHOROIDITIs) (Bader, 1858; Hutchinson, 1859; 1874; Bergmeister, 1874;
Juler, 1882; Fehr, 1902; Fuchs, 1918–21 ; Kitahara, 1933; and others)
(Fig. 1877).
PIGMENTARY DEGENERATION of the retina resembling that seen in primary pig-
mentary degeneration (retinitis pigmentosa), as a result of a diffuse chorio-retinitis is
common, but the occurrence of scattered pigmentation of this type arranged in discrete
masses overlying the retinal vessels without other gross choroidal lesions is relatively
rare. We shall see presently that such a picture forms one of the commoner mani-
festations of congenital syphilis, but in the acquired type of the disease its appearance
1 p. 2273.
2270 TEXT-BOOK OF OPHTHALMOLOGY
is confined to the later tertiary period, and the recorded cases have occurred together
with optic atrophy in association with general paralysis (Nagel, 1898; Stein, 1903)
or tabes (Friedenwald, 1930). The occurrence of such a lesion, which is usually
characteristic of congenital syphilis, in late neuro-syphilis would seem to indicate a
resemblance between the immunological reaction of the body in those two aspects of
the infection ; and it is of interest that under treatment the progress of the retinal
pigmentation may cease or its amount even decrease (Friedenwald, 1930).
iv. SYPHILOMATA
We have noted that the typical syphilitic infiltration is nodular in type
even when clinically the inflammation seems diffuse in character, and that
it frequently happens that these nodules become aggregated together to
constitute a granulomatous tumour. The question of the nature of these
syphilitic nodules was first discussed by Widder (1881) who concluded that
two types existed :—
(a) PAPULEs or condylomata—a multiple variety, small in size, richly
vascular, and tending to complete resolution, appearing in the early secondary
stages.
(b) GUMMATA—a solitary variety, large in size, poor in vessels, and
liable to degeneration and necrosis, associated with the late stage of the
disease.
Apart from the fact that the terms papule and condyloma are
unfortunate in that they are usually applied to patches on epithelial surfaces
in the formation of which the epithelium takes a part, subsequent observa-
tions have shown that these two types of lesion do not by any means fall
into two distinct categories. It is true that the evanescent nodules are a
characteristic accompaniment of the iritis associated with the early
secondary stage of the disease, but the solitary granulomata are by no
means characteristic of the tertiary stage, and may occur at any time from
the early stage onwards. All syphilomata, indeed, tend to appear early ;
thus Ewetzky (1904) found that 43% occurred in the first half-year, 22% in
the second, and 8% in the third half-year after infection. Actually there is
histological evidence that both types of tumour are merely aggregations of
the nodules characteristic of the diffuse inflammation, and are accompanied
by smaller but clinically invisible nodules scattered throughout the surround-
ing tissue ; moreover, it is found histologically that many of the solitary
granulomata are richly vascularized. It is obvious, indeed, that these
“solitary " tumours are in fact multiple, and that they cannot be classed
according to the period of the disease in which they occur or according to
their vascularity or tendency to necrose. While, therefore, it must be
accepted that the granulomatous lesions occurring in uveal syphilis are all of
the same essential nature and represent aggregations of the modular infiltration
typical of the disease, and that the single term syPHILOMA would probably
be the more correct universal appellation, the facts that multiple, vascular,
DISEASES OF THE UVEAL TRACT 22 71
transient granulomata are common in the early secondary stage, and that
larger granulomata occur at any stage, which may or may not necrose according
to the presence or absence of an obliterative endarteritis, justify the clinical
differentiation of the two ; and in this event it is convenient to retain the
classical terms, papules and gummata.
PAPULES (Fig. 1892, Plate XXXVIII), which were originally noted by
Beer (1817) and were first adequately described by Widder (1881) and
Alexander (1888), are relatively common in association with a syphilitic
iritis : Igersheimer (1918) noted them in 31 out of 78 cases of syphilitic
iritis, Gilbert (1930) in 29 out of 83 cases, and Moore (1931) in 16 out of
249 cases, of which 6 occurred in 111 cases of secondary iritis, 3 in 29 cases
of relapsing iritis, and 7 occurred in 109 cases of tertiary iritis. They
appear as round or pyramidal swellings varying in size from a hemp-seed
to a pea and in colour from yellow to reddish-yellow or reddish-brown.
They occur usually at the pupillary or ciliary borders of the iris and rarely
in the intervening tissue, and vary in number from one to several ; appearing
rapidly in the midst of a considerable inflammatory disturbance, they may
persist for some days or some weeks, and then usually absorb and disappear
leaving merely a depigmented and atrophic area in the iris. Occasionally,
however, papules at the ciliary border of the iris become converted into
granulation tissue which projects as a red mass into the angle of the anterior
chamber ; but these also tend to disappear and the area becomes atrophic.
Such circular circumscribed areas of atrophy in the iris affecting the anterior
mesodermal layer and occasionally revealing the pigmented layers of the
posterior surface on their floor, situated preferentially in the region of the
sphincter Or at the root of the iris, and especially if they are associated
with broad-based synechiae, typical of are syphilitic irido-cyclitis (Fig. 1891,
Plate XXXVIII). (Krückmann, 1907; de la Vega, 1923; Licsko, 1923).
As occurring in the early form of irido-cyclitis they may be superficial
or deep. The SUPERFICIAL EARLY PAPULEs (Fig. 1889, Plate XXXVIII)
occur near the pupillary margin and, as we have seen, are developed
from persistent roseola. They are therefore small, only slightly raised
up from the surface, highly vascularized and light red in colour.
DEEP EARLY PAPULEs, on the other hand, are associated with the rich
capillary plexus round the sphincter muscle ; they therefore start deep in
the mesodermal tissue which they thrust aside as they grow and rise beyond
the surface (Fig. 1892, Plate XXXVIII). Their development is associated
with much inflammatory disturbance and oedema in the surrounding tissue
which becomes heaped up to adhere to the lens capsule as the typical broad-
based syphilitic synechia. Usually they are few in number, but occasionally
in severe and neglected cases they may form a more or less complete wall
round the pupil (v. Michel, 1902), the one merging into the other.
Eventually they die down and disappear, giving place to the fibrinous
exudative iritis.
T.O. —WOL. III. N
2272 TEXT-BOOK OF OPHTHALMOLOGY
LATE PAPULEs are deeply situated, preferentially in the sphincter region
or near the ciliary border, but differ from those appearing at the early
secondary stage in being associated with much less hyperaemia, oedema, and
inflammatory disturbance. They are therefore more grey or yellow in
appearance, more opaque and solid in texture ; moreover, they remain
static and isolated, and although they may occur in groups, do not become
confluent. As a rule also they persist longer, and although they may rarely
become necrotic and bleed (Despagnet, 1898), they usually absorb slowly,
leaving no trace except a depigmented and atrophic area in the iris.
GUMMATA. Granulomatous masses of a gummatous nature are said to
be associated with syphilis of an unusually severe character (Tooke, 1903);
they usually occur fairly early in the infection, quite a large proportion
(42.8%, Ewetzky, 1904) in the first six months—the gomme précoce of
Fournier (1859)—but their appearance may be delayed until late in the
tertiary period (10 years or more). The vast majority occur in the ciliary
body and appear clinically in the angle of the anterior chamber (Fig. 1890,
Plate XXXVIII), while as a rarity they may start primarily in the iris or the
choroid. Ciliary gummata occur most commonly when the choroid is the
primary seat of the disease, in which case the ciliary region may become
diffusely infiltrated with granulation tissue and inflammatory exudates, or is
occupied by a localized granulomatous mass. This undergoes necrosis involv-
ing a structural disintegration of the iris, the cornea and angle of the anterior
chamber, and eventually fills the entire globe with progressively necrosing
tissue, or breaks through the sclera in the ciliary region, and appears on the
surface as a violet-coloured staphylomatous protrusion which eventually
develops into a widely sloughing ulcer. The process is usually accompanied by
the most violent irido-cyclitis with abundant plastic exudates, which may
necessitate excision owing to pain. With active anti-syphilitic treatment,
however, the lesion tends to resolve, but if the process has advanced to any
great extent before treatment is inaugurated, the extensive damage may
well lead to blindness even although the eye may be retained.
Ciliary gummata are relatively rare, but many cases are on record since their
original description by Arlt (1856). The early cases have been annotated by Ostwalt
(1896), Coppez (1898), Tooke (1903) and Ewetzky (1904), the last of whom collected
67 cases; and in the literature up to 1930 Berberov (1934) gathered 147 cases, adding
6 of his own. Interesting examples are those described by Brixa (1899), Hanke (1899),
Morton and Parsons (1902), Parsons (1903), Brückner (1910), Uhthoff (1918), Hoch-
welker (1922), Cattaneo (1924), Wachtler (1925) and Reganati (1925).
Gummata of the iris are much more rare, for although the iris is frequently invaded
secondarily from the ciliary body, large granulomata starting in this tissue are excep-
tional (Gräfe and Colberg, 1861; Benoit, 1898 ; Rumschewitsch, 1903; Greeves,
1915, and others).
Gummata of the choroid are also rare, if, indeed, they can be said to exist. Apart
from the extension of a ciliary granuloma into the anterior region of the choroid or of
the very rare extension of a gumma of the optic nerve into the posterior region, only
diffuse gummatous infiltration of the choroid has been observed, which, however, may
DISEASES OF THE UVEAL TRACT 2273
become extremely extensive and undergo necrosis (Schöbl, 1888; Fiahlo, 1901;
Stock, 1905; Verhoeff, 1916; Hanssen, 1916; Uhthoff, 1918).
B. Congenital Syphilis
The first writer to note the occurrence of ocular inflammation in
congenital syphilitics was Lawrence (1830) who described a case of acute
iritis. Five other such cases were annotated by early writers (Walker, 1845;
Maunsell and Evanson, 1847; Jacob, 1849; and Dixon, 1855), until
eventually Jonathan Hutchinson (1858–73), in his classical series of papers,
described in detail and with a wealth of clinical observation at least five
clinical entities occurring in this condition—acute iritis, interstitial keratitis
with anterior uveitis, chronic irido-cyclitis, choroiditis, and vitreous opacities.
(i) ACUTE IRIDO-CYCLITIS. An acute irido-cyclitis is not uncommon in
hereditary syphilis; in fact, it can be said with a fair degree of certitude that
this is by far the commonest aetiology of such an inflammation in infants. It
may occur in the foetus or be present at birth (Reis, 1907; Seefelder, 1908),
but usually comes on when the child is some 5 to 6 months old. The
inflammation, which may be either unilateral or bilateral, is usually of the
acute fibrinous type, and may clear up fairly rapidly on treatment, but
quite frequently an exudative membrane occludes the pupil, or massive
exudation in the vitreous leads to the formation of a pseudo-glioma and the
development of phthisis bulbi (Hutchinson, 1858–59 ; Nettleship, 1883;
Dorrell, 1911; and many others), or even panophthalmitis (Vernon, 1869).
(ii) The anterior uveitis which accompanies, or is perhaps the essential lesion in
tinterstitial keratitis, has already been fully discussed.* An irido-cyclitis with a
fibrinous exudation is invariable, and the frequency of a peripheral choroiditis of the
disseminated type may be assessed from the statistics of Cunningham (1922) who, in
82 cases of interstitial keratitis of syphilitic origin, found choroiditis in 56.
(iii) CHORIO-RETINITIS. Chorio-retinitis is common in hereditary
syphilis, assuming many different forms and presenting a heterogeneous
collection of clinical pictures. Sometimes it is associated with other stigmata
of hereditary syphilis,” very frequently with interstitial keratitis, but, again,
it may be the only obvious clinical manifestation of the disease. It usually
occurs very early in life, probably sometimes before birth, and is noted,
frequently incidentally, after the active inflammation is over and healing
has taken place ; it is, indeed, rare to observe active disease and then it is
usually superimposed upon much old and quiescent disease (Maclachlan and
Carr, 1926). The more typical ophthalmoscopical appearances may be
divided into the following groups (Hirschberg, 1895; Antonelli, 1897;
Sidler-Huguenin, 1901) :-
(a) The finely pigmented or pepper-and-Salt fundus, a picture character-
1 Vol. II, p. 1963. * Vol. II, p. 1965.
2274 TEXT-BOOK OF OPHTHALMOLOGY
istic of hereditary lues, wherein the entire fundus is dusted with innumerable
small bluish pigmented spots between which lie rounded depigmented areas
of a yellowish-red colour (Fig. 1895, Plate XXXIX). Frequently the changes
are most accentuated towards the periphery, which assumes a general
leaden hue. This appearance remains permanently unchanged, and is
frequently associated with atrophy of the disc and narrowing of the retinal
vessels.
(b) Isolated pigment spots, usually two or three in number, and often
surrounded by a bright halo, are scattered in the peripheral fundus.
(c) Grossly pigmented areas form large black masses in the fundus, some-
times in circumscribed areas, and sometimes throughout the entire periphery.
(Fig. 1896, Plate XXXIX.)
(d) White atrophic areas may be scattered over the fundus, either in
disseminated form, especially in the peripheral regions, or forming large
confluent patches in the central area. As a rule they are less in diameter
than the disc, and pigmentation, either as a central spot or a marginal ring,
is sparse. (Fig. 1897, Plate XXXIX.)
While these types occur they must not be taken as definite clinical
entities, for frequently mixed pictures appear, different areas of the fundus
showing different characteristics. Sometimes the changes are widespread
in their distribution, the entire fundus being occupied by pathological
alterations ; frequently they are confined to the posterior region, or,
especially when associated with interstitial keratitis, to the periphery :
while occasionally the macula alone is involved in a central choroiditis
(Kraupa, 1936). The underlying lesion is probably in most cases in the
chorio-capillaris so that the pigmented epithelium is readily affected, some-
times to produce a diffuse mottling, sometimes being stimulated to proli-
ferate, and sometimes being totally destroyed over an area of inflammation
leaving an atrophic patch. At other times fibrous tissue formation is
evident lying underneath the retinal vessels (Nettleship, 1882, and others),
and it is probable that the characteristic white patches are fibrous scars
which constitute the end-result of an obliterative endarteritis in the choroid.
Such masses of new tissue between the retina and choroid may be very
extensive, occupying, for example, the whole posterior area and embracing
the disc and the macula (Lawford, 1902), and not infrequently proliferation
may extend into the vitreous body (Nettleship, 1883). Again, malformations
of the disc may be left as a permanent legacy—dimples on its surface,
irregularities of its margin, and the formation of membranes extending over
it (Nicolau, 1930). Finally, such choroiditic changes are very frequently
associated with waxy-like pallor of the optic disc, a narrowing of the calibre
of the retinal vessels, and a considerable diminution in function of the eye,
the visual acuity and the light sense being lowered, and the fields con-
stricted, a condition sometimes complicated by the development of a
secondary cataract.
DISEASES OF THE UVEAL TRACT 2275
Pathology. The pathological changes in uveal syphilis are in no way
characteristic, for with the exception of advanced gummatous lesions, the
picture is essentially that presented by any nondescript chronic inflammation.
Nor do the changes in the acquired form differ essentially from those met
with in hereditary syphilis, except that in the latter the infiltration tends to
be more diffuse and to comprise myeloid cells, the primitive and immature
elements with which the foetus tends to respond to any injurious stimulation.
Essentially, the infiltration consists of lymphocytes and plasma cells, a
peculiar attribute being that, while it may be diffuse in distribution, it has a
strong tendency to become aggregated into modular form ; moreover, there is the
same tendency to fibrous tissue formation in atrophic areas as occurs in
non-specific inflammations generally. Vascular changes are by no means
characteristic, and while some writers have stressed the importance of
FIG. 1899. –SYPHILITIC IRITIS.
There are two iridic nodules, one at the root and one at the peripheral end of
the sphincter (Greeves, T. O. S.).
peri-vascular infiltrations of round cells, homogeneous hyaline degeneration
of the vessel walls, and proliferative endothelial overgrowth resulting in
endarteritis obliterans, (v. Michel, 1881–1907; and others), in the experience
of most observers, these changes are slight or entirely absent (Edmunds and
Brailey, 1880; Scher, 1892; Benoit, 1898; Nagel, 1898; Hanke, 1899;
Brixa, 1899; Greeves, 1915; and many others). Even in congenital
syphilis specific vascular changes cannot be said to occur invariably
(Schlimpert, 1906; Rumbaeur, 1921). In fact, changes of the nature of a
small celled infiltration of the adventitia and an endothelial overgrowth are
more characteristic of the extra-ocular portions of the posterior ciliary
arteries (Baas, 1897 : Rumschewitsch, 1903).
The earliest cases of diffuse iritis to be investigated histologically were
by v. Michel (1881) who examined portions removed by iridectomy in an
active stage, and Fuchs (1884) who described an eyeball removed in an
advanced stage. This description has been amply confirmed by subsequent

2276 TEXT-BOOK OF OPHTHALMOLOGY
writers (Igersheimer, 1913–28; Fuchs, 1918; Weekers, 1919; Yoshida,
1919; Rumbauer, 1921; Schwenker, 1922; Li, 1924; Tome and Bona,
1936; and others). The infiltration of lymphocytes and plasma cells is
almost invariably gathered into nodular form, the nodules being preferen-
tially near the pupillary border or the root of the iris and situated deeply in
its posterior layers (Fig. 1899).
Similarly in the choroid a non-specific infiltration with round cells
occurs with a strong tendency to nodular aggregations. The earliest records
of Hutchinson (1859), Pagenstecher (1862), Nettleship (1871-83) and
Cowell (1872) have been corroborated by the later work of Fuchs (1884–1918),
Fig. 1900.-SYPHILITIC ChoroidITIs.
Fibrillar tissue between choroid and retina on the surface of the intact membrane
of Bruch. The dark areas are tubes of pigmentary epithelial cells (Greeves, T.O.S.).
Brixa (1899), Ståhli (1913), Greeves (1915), Igersheimer (1918), Spicer (1924),
Ling (1929), and many others. While the outer layers of the choroid are
usually—except in the most advanced cases–relatively untouched, the inner
layers are mainly affected, the chorio-capillaris being densely infiltrated
until few of the capillaries remain to suggest its original structure. Quite
frequently, corresponding to the appearance noted clinically, the inflam-
matory infiltration is intensified at the posterior pole near the disc,
where the whole thickness of the choroid may be destroyed. Over most of
the fundus the membrane of Bruch may be intact, but the pigment
epithelium lying upon it may have disappeared in part, or be stimulated
to form dense proliferative masses. A further feature which has been

DISEASES OF THE UVEAL TRACT 2277
noted on several occasions is the formation of wide areas of dense
avascular fibrillated tissue between the choroid and the retina lying
on the surface of Bruch's membrane, through which tubes of pigmented
epithelium sometimes proliferate (Nettleship, 1883; Greeves, 1915;
Ling, 1929; and others) (Fig. 1900); it seems most likely that this
tissue is not derived from the fibroblasts of the choroid as the result of
post-inflammatory fibrous organization, but is probably formed from the
cells of the pigmentary epithelium by a process of proliferation and
metaplasia.
The retina is usually affected in some degree. It is frequently extremely
Fig. 1901,–DIssFMINATED SYPHILITIC CHoRIo-RETINITIs (Parsons).
atrophic and adherent to the choroid, the rods and cones may have
disappeared, the two nuclear layers and the ganglion-cell layer are atrophied,
and sometimes the arrangement of its layers can be made out only with
difficulty or not at all; lymphocytic infiltration may be marked in the nerve-
fibre layer and around the retinal vessels. Then retinal changes are often
more generalized than the choroidal, but here and there Bruch's membrane
is broken down, and an exudative infiltration from the choroid followed by
newly formed blood-vessels, penetrates into the retinal tissue, exactly as in
cases of non-specific choroiditis, so that the two are eventually welded
together in an atrophic patch (Fig. 1901). Not infrequently also there are
chronic inflammatory changes in the arachnoid sheath of the optic nerve,
with a mononuclear infiltration, so that the nerve is swollen and oedematous

2278 TEXT-BOOK OF OPHTHALMOLOGY
in the acute stages and atrophic in the chronic stages, sometimes with the
formation of new-formed connective tissue on the nerve-head.
SYPHILoMATA. In a considerable number of cases the nodular
distribution of the infiltration becomes accentuated, and gathering together
Fig. 1902–SYPHILITIC GRANULomatous Nobute on the PECTINATE LIgAMENT
(Greeves, T. O. S.).
Fig. 1903—SYPHILITIC GRANULoMA At Root or Iris (Greeves, T. O. S.).
in larger aggregations, granulomatous masses are formed. We have already
noted that there is no essential qualitative difference between the smaller
nodules and the larger masses. The nodules are made up of plasma cells
and lymphocytes supported in a reticulum, with masses of epithelioid cells
arranged frequently in the central parts so as collectively to resemble


DISEASES OF THE UVEAL TRACT 2279
a multi-nuclear giant cell; at other times typical giant cells are seen.
In contra-distinction to the avascular nodules of tubercle, these syphilitic
**.*. º'-
-- - -
Fig. 1904.—SYPHILITIC GRANULoMA.
A small granuloma in the posterior part of the iris. The anterior pigmented
layer is proliferating into it (Greeves, T. O. S.).
Fig. 1905.-CILIARY GUMMA (x 8) (Parsons).
nodules are supplied with newly-formed capillaries, but when they occur
in avascular sites, such as on the pectinate ligament, they may be avascular,


2280 TEXT-BOOK OF OPHTHALMOLOGY
or alternatively are supplied by vessels from neighbouring structures such
as the iris (Fig. 1902).
Such nodules occur plentifully on the iris, preferentially in the posterior
layers of the stroma close against the pigment epithelium ; thereon they
tend to grow forwards through the stroma to appear on the anterior surface
(Fig. 1903); sometimes the pigment epithelium is stimulated to proliferate
into their substance (Fig. 1904). In the choroid the chorio-capillaris is the
favourite site. An aggregation of such nodules into a mass of granulation
tissue may be said to form a papule when it retains this structure and its
vascularity, in which event it can disappear entirely, leaving an area of
atrophy at its site. Alternatively, changes of the nature of endarteritis
obliterans may ensue especially if the granuloma is of a large size, so that
necrosis sets in centrally and spreads throughout the mass, forming what
may be called a gumma ; some of these, particularly in the ciliary region,
may reach such dimensions either as a tumour-like mass or a diffuse infiltra-
tion as completely to fill the globe with necrotic tissue which eventually
perforates through the sclera (Fig. 1905).
A considerable number of pathological examinations of these syphilomata is in the
literature since the original description of Virchow and Billroth (see v. Graefe, 1860)
and Gräfe and Colberg (1861), the first of whom described a granuloma (which may
possibly have been tuberculous) removed from the iris of a child one year old, and the
latter a highly vascularized granuloma removed by iridectomy. Granulomata of the
fresh papular type in an iritis papulosa were examined by Benoit (1898), Andersen
(1913) and Weekers (1919), and tumours of the iris of a gummatous type by V.
Hippel (1867), Liebrecht (1891), Rumschewitsch (1903) and Greeves (1915).
Syphilomata of the ciliary body are much more common ; in Ewetzky’s (1904)
monograph wherein 67 cases are collected, 21 were examined histologically. Interesting
histological descriptions of gummata or gummatous infiltrations are those of Scherl
(1892), Brixa (1899), Hanke (1899), Fiahlo (1901), Morton and Parsons (1902), Parsons
(1903), Brückner (1910), Uhthoff (1918) and v. Hippel (1921).
In the choroid the usual change is a diffuse infiltration which assumes large
dimensions and usually spreads from the ciliary region (v. Hippel, 1867; Schöbl,
1888; Baas, 1897; Fiahlo, 1901; and others); isolated tumour-like granulomata are
usually near the disc associated with disease of the optic nerve (Stock, 1905; Verhoeff,
1916; Hanssen, 1916; Jacoby, 1921 ; and others).
It is curious that treponemes have rarely been demonstrated in the uveal tissue
and then only sparsely in congenital cases (Bab, 1906; Schlimpert, 1906; Krückmann,
1907; Sabrazès and Dupérie, 1908); the eye thus differs from most internal organs
where in such cases these organisms may be found in enormous numbers.
SYPHILITIC ATROPHY OF THE IRIS
In syphilitic cases several types of atrophy of the iris are met with which it is
convenient to summarize here.
1. Localized areas of atrophy corresponding to the site of a granulomatous lesion
which has resolved (de la Vega, 1923; Licsko, 1923; and others) (Fig. 1891,
Plate XXXVIII). In congenital syphilis they may be very numerous; thus Harman
(1910) in an old case of kerato-iritis noted 22 such areas on the right iris and 13 on the
left.
DISEASES OF THE UVEAL TRACT 2281
2. Small isolated white patches devoid of pigment seen in the secondary stage,
Comparable to leucodermia, and called LEUKIRIDIA by Krückmann (1907).
3. A generalized atrophy of the iris tissue in the tertiary stage associated with
central neuro-Syphilis and usually with pupillary paresis (Igersheimer, 1918; Behr,
1921 ; Barkan, 1921). Such an atrophy associated with tabes or general paralysis is
of a trophic and not an inflammatory nature ; it will be considered in greater detail
at a later stage.”
Diagnosis. The diagnosis of syphilitic uveitis is not always easy, for
except for cases of typical iritis papulosa, gummatous formations, and certain
cases of acute chorio-retinitis wherein the occurrence of choroidal lesions
associated with oedema of the retina and optic disc, a cloud of fine vitreous
Opacities and a ring scotoma which may suggest a specific aetiology,
most cases of syphilitic inflammation present a picture which cannot be
diagnosed with absolute certainty from inflammations due to other causes.
The diagnosis must therefore usually rest on a study of the patient as a
whole. At the same time it must be remembered that every iritis or
choroïditis occurring in a syphilitic subject is not necessarily due to this disease ;
Other causes must therefore be excluded, and in cases where a mixed aetiology
offers itself, the diagnosis of syphilis can be presumed if the ocular lesion is
accompanied by parallel syphilitic lesions elsewhere and if it responds to
anti-Syphilitic treatment.
A history of syphilitic infection is of value, especially in early cases, but
no reliance can be placed on its absence, particularly in females in whom its
occurrence can be so readily missed. Much more important is a complete
physical examination. In early syphilis over 95% of the cases of ocular
disease are associated with evidence of syphilis elsewhere, either a healed
chancre, most commonly muco-cutaneous lesions, more rarely alopoecia,
arthritis, periostitis, or meningeal symptoms (Moore, 1931). In the late
tertiary stages associated lesions are not so common, but nevertheless they
are encountered in 50% of cases—cardio-vascular, neurological, Osseous, or
dermal and mucosal lesions being in evidence. In congenital syphilis other
stigmata of the disease are common, and the diagnosis should depend, not
alone upon examination of the child, but also when it is possible, of the
parents.
The basis of the diagnosis, however, is serological, and most reliance is
placed upon the Wassermann reaction of the blood. It must not, however,
be taken by any means as a constant indication. In early syphilis it is a
fairly good index, but in the later stages of the disease the results are
more irregular. In syphilitic iritis, for example, Moore (1931) found the
blood Wassermann positive in 97% in early cases, but in late cases only in
81% ; in recurrent syphilis, occurring when the immunity had been dis-
turbed by inadequate treatment, it was positive in only 55% of cases. In
these last, of course, the history is, or should be, clear. The cerebro-spinal
1 p. 2403.
2282 TEXT-BOOK OF OPHTHALMOLOGY
fluid findings are no more reliable, since neuro-syphilis is not a frequent
concomitant of ocular syphilis (Zimmermann, 1924). Thus Moore (1931)
found a positive cerebro-spinal fluid in 20% of early and 26% of late cases
of iritis. It is noteworthy, however, that in recurrent affections, where, as
we have seen, neuro-recurrences frequently coincide with ocular recurrences,
this percentage rises to 41. The majority of syphilologists consider that the
flocculation tests, such as the Kahn test or the Kline test, are more sensitive
than the complement-fixation tests, but these also are by no means
universally reliable. It is evident, therefore, that while these serological
tests are a good diagnostic guide in the secondary stage of the disease,
reliance upon them alone will lead to the mis-diagnosis of some 20% of the
later cases.
The prognosis of uveal syphilis varies considerably with the nature of
the lesion and the date of its onset after infection, but at any stage it can be
potentially dangerous to vision. In iritis occurring in the secondary stage
the prognosis with adequate treatment is usually good, although some 10%
of cases are left with considerably damaged vision. In the tertiary stage,
however, the prognosis is worse, largely because of the late development of
glaucoma ; almost half the cases do well, and of the remainder who have
permanently impaired vision, some 10% eventually become completely
blind. The prognosis of a choroiditis depends largely on the site of the lesion,
and when the central area is affected it is always bad. When it is not
affected, the prognosis should again be guarded, for recurrences are frequent,
and damage to the optic nerve and retina may leave the function of the eye
permanently impaired.
The treatment of syphilitic diseases of the uveal tract should combine
the usual methods of therapy applicable locally to the inflammation in
question 1 with systemic treatment of the general disease ; and this is a
question so bound up with possible present or future associations with
syphilis elsewhere in the body that it is always well that it be placed in the
hands of a syphilologist. It cannot be undertaken without a thorough
knowledge of the patient as a whole, of the bewildering complexities of the
disease, and of the range, the possibilities, and the dangers of the drugs
employed when applied to the individual case of the patient concerned—
whether they be mercury, iodides, arsenic or bismuth. In all cases of uveal
disease treatment should be begun as soon as the diagnosis is made or is
considered probable, and it should be carried out intensively and vigorously,
for any delay may produce disastrous permanent effects ; this should be
done regardless of the possibility of the occurrence of a Herxheimer reaction,
for if treatment is persisted in, this carries a good prognosis. Moreover, the
best modern opinion insists that treatment should ideally be continued
without intermission for at least a year after the eye has quietened, the
patier * is asymptomatic, and is serologically normal ; to stop treatment
p. 2206.
DISEASES OF THE U WEAL TRACT
2283
when the ocular inflammation has subsided is merely to court future disaster,
both in the eye and elsewhere.
It is to be noted that if the local condition calls for it, a syphilitic eye
tolerates operation relatively well. If emergency measures are called for, an
intra-ocular operation can be undertaken at any time, and if an emergency
does not exist, so far as the syphilitic element is concerned, it can be done with
safety after a course of arsenical treatment lasting 6 to 8 weeks, which, of
course, should be continued as soon as practicable after operation.
Alexander. Syphilis w. Auge, 1888.
Neue Erfahrungen ü. luetische Augenkrank-
wngen, 1894.
Andersen. A. f. O., lxxxiv, 172, 1913.
Antonelli. Les Stigmates ophtal. rudi-
mentaères de la Syphilis hereditaire, Paris,
1897.
Arlt. Die Krankheiten d. Auges, Prag., 1853.
Krankheiten, d. Auges, ii, 67, 82, 1856.
Baas. A. f. O., xliv, 642, 1897; (3) 642,
1898.
Bab. D. med. W., xxxii, 1945, 1906.
Bader. R. L. O. H. Rep., i, 245, 1858.
Barkan. K. M. Aug., lxvi, 528, 1921.
Baumert. K. M. Aug., lxxix., 782, 1927.
Beer. Lehre v. d. Augenkrankheiten, Wien,
1817.
Behr. K. M. Aug., lxvi, 363, 1921.
Benoit. A. d’O., xviii, 189, 1898.
Berberov. Sov. vest. Oft., v, 565, 1934.
Bergmeister. A. f. O., xx (2), 95, 1874.
Bertarelli. Zbl. Bakt., xli., 320, 1906; xlii,
238, 1907.
Brixa. A. f. O., xlviii (1), 123, 1899.
Brown. A. of O., li, 408, 1922.
Brown and Pearce. Proc. Soc. Eacp. Biol.
Med., xviii, 255, 258, 1921.
Brückner. B.O. G. Heidel., xxxvi, 400, 1910.
Brunnsgaard. A. f. Derm. u. Syph., clvii,
309, 1929.
Bruns. A. of O., liv, 462, 1925.
Bryn. K. M. Aug., lxxviii, Beil., 89, 1927.
Bulson. T. Am. O. S., xxiii, 292, 1925.
Butler. Brit. Med. J., i, 804, 1911.
Campbell. Lancet, ii, 219, 1905.
Brit. Med. J., ii, 231, 1910.
Cattaneo. An. di Ott., lii, 299, 414, 1924.
Chesney. Immunity in Syphilis, Baltimore,
1927.
Am. J. Syph., xiv, 289, 1930.
Chesney and Kemp. J. Eacp. Med., xlii, 33,
1925.
Clapp. A. of O., ii, 580, 1929.
Am. J. O., iv, 194, 1921; xi, 527, 1928;
xvi, 397, 1933.
Coccius. Die Heilanstalt für arme Augen-
kranke, w.s.w.., Leipzig, 1870.
Coppez. A. d’O., xviii, 376, 1898.
Cowell. R. L. O. H. Rep., vii, 335, 1871–72.
Cunningham. T. O. S., xlii, 44, 1922.
Delafield. T. Am. O. S., 69, 1871.
Despagnet. Rec. d’O., xx, 636, 1898.
Dixon. A Guide to the Practical Study of
Diseases of the Eye, pp. 145, 149, 1855.
Dorrell. T. O. S., xxxi, 47, 1911.
Doyne and Stevenson. T. O. S., xxv, 110,
1905.
Edmunds and Brailey. R. L. O. H. Rep., x,
132, 1880.
Elschnig. K. M. Aug., lxxiv, 783, 1925.
Ewetzky. Syphilom, d. Ziliarkorpers, Berlin,
1904.
Fehr.
Fiahlo.
Cb. pr. Aug., xxvi, 233, 1902.
A. f. O., lii (3), 446, 1901.
Förster. A. f. O., xx (1), 33, 1874.
Fournier. J. d’O., i, 495, 543, 1859.
Friedenwald. Am. J. O., xiii, 943, 1930.
Fuchs, A. Z. f. Aug., lviii, 315; lix, 213,
1926.
Fuchs, E. A. f. O., xxx (3), 139, 1884;
xcvii, 85, 1918 ; cvii, 15, 1921.
Cb. pr. Aug., xl, 105, 1916.
Funabashi. Lºwes, v, 70, 1930; ix, 26, 63,
201 ; x, 18, 1933.
Gifford. Am. J. O., xiv., 100, 1931.
Gilbert. Kurzes Hb. d. O., Berlin, v, 125,
1930.
Goulden. R. L. O. H. Rep., xix, 328, 1914.
v. Graefe. A. f. O., vii (2), 35, 1860.
Gräfe, A. and Colberg. A. f. O., viii (1), 288,
1861.
Greeff and Clausen. D. med. W., xxxii, 1454,
1906.
Greeves. T. O. S., xxxv, 277, 1915.
Grigoriew. Derm. W., lxxxix, 1122, 1929.
Groenouw. G.-S. Hb., II., xi (I), 737, 1904.
Hanke. A. f. O., xlviii (2), 300, 1899.
Hanssen. K. M. Aug., lvi (1), 66, 1916.
Harman. T. O. S., xxx, 103, 1910.
v. Hippel. A. f. O., xiii (1), 65, 1867; cv,
1037, 1921.
Hirschberg. Cb. pr. Aug., xix, 75, 1895.
Bochwelker. A. d’O., xxxix, 171, 1922.
Bolloway. Am. J. O., xiv, 232, 1931.
Hutchinson, R. L. O. H. Rep., i, 191, 226,
1858; ii, 54, 258, 1859; vii, 42, 1873.
Igersheimer. A. f. O., lxxxiv, 48, 1913;
cix, 265, 1922.
Zb. g. O., xii, 193, 1924; xxvi, l, 1932.
Syphilis w. Auge, Berlin, 1918, 1928.
Irons and Brown. J. Am. Med. As., lxxxi,
1770, 1923.
Jacob. Treatise on Inflammations of the
Eye-ball, p. 97, 1849.
2284
TEXT-BOOK OF OPHTHALMOLOGY
Jacobson.
1859.
Jacoby. Z. f. Aug., xlvi, 133, 1921.
Juler. T. O. S., ii, 61, 1882.
Ritahara. Acta O. S. Japan, xxxvii, 2094,
1933.
Rnapp. T. Amer. Ac. Oph. Oto. Larg., xxv,
132, 1920.
Röhne. K. M. Aug., lxv, 882, 1920.
Kraupa. Z. f. Awg., lxxxix, 204, 1936.
Frückmann. B. O. G. Heidel., xxx, 117,
1902; xxxi, 51, 1903;
G.-S. Hb., II, vi, 40, 1907.
Lang. Lancet, i, 1368, 1913.
Brit. Med. J., i, 381, 1913.
Lawford. T. O. S., xxii, 161, 1902.
Lawrence. Treatise on Venereal Dis. of the
Eye, London, p. 306, 1830.
Li. A. of O., liii, 531, 1924.
Licsko. K. M. Aug., lxxi, 456, 1923.
Liebrecht. K. M. Aug., xxix, 184, 1891.
Ling. A. f. O., i, 207, 1929.
Löwenstein. K. M. Aug., lxxviii, Beil., 73,
1927; lxxxii, 64, 1929.
Maclachlan and Carr. T. O. S., xlvi, 358,
1926.
Maunsell and Evanson. Practical Treatise on
the Management and Dis. of Children.
Dublin, p. 534, 1847.
Mauthner. Zeisel's Lhb. d. Syphilis, ii, 1875.
v. Michel. A. f. O., xxvii (2), 171, 1881.
B. O. G. Heidel.., xxx, 132, 1902.
Z. f. Aug., xviii, 295, 1907.
Moore. Am. J. O., xiv, 110, 1931.
Morton and Parsons. T. O. S., xxii, 266,
1902.
Nagel. A. f. Aug., xxxvi, 369, 1898.
Nettleship. R. L. O. H. Rep., vii, 204, 370,
374, 576, 1871–72.
T. O. S., ii, 60, 1882; iii, 36, 1883.
Newton. Teacas State Med. J., xxi, 315, 1925.
Nichols. J. Eacp. Med., xix, 362, 1914.
Nicolau. Cluj. Med., xi, 420, 1930.
O’Leary. Am. J. O., xv, 24, 1932.
Ostwalt. Rev. gem. d’O., 97, 1896.
Pagenstecher. Würzburg. med. Z., 402, 1862.
Parsons. R. L. O. H. Rep., xv (3), 292, 1903.
Reganati. Atti d. Congr. S. Ital. di oft., 231,
1925.
Reis. A. f. O., lxvi, 201, 1907.
Rochon-Duvigneaud. A. d’O., xv, 764, 1895.
Rollet and Rosnoblet. A. d’O., x1, 688,
1923.
Rönne. Acta O., xii, 1, 1934.
Rosenstein. K. M. Aug., lxxvi, 657, 1926.
Rumbauer. K. M. Aug., lxvi, 61, 1921.
Rumschewitsch. K. M. Aug., xli (1), 27,
1903.
Rćnigsberg. med. Jahrb., i, 283,
Sabrazès and Dupérie. Réunion biol. de
Bordeaua, 10th Nov., 1908. See Nagel's
Jahrb., xl, 698, 1909.
Satanowsky. A. de Oft. B. A., vii, 279, 1932.
Scherl. A. f. Aug., xxv, 287, 1892.
Schlimpert. D. med. W., xxxiv, 1942, 1906.
Schmidt. K. M. Aug., x, 94, 1871.
Berlin. kl. W., ix, 273, 1872.
Schnabel. A. f. Aug., v, 50, 1876.
Schöbl. Cb. pr. Aug., xii, 321, 1888.
v. Schroeder. Diss., Dorpat., 1880.
Schubert. Ueber Syph. Augenkrankheiten,
Berlin, 1881.
Schwenker. K. M. Aug., lxix., 9, 1922.
Seefelder. D. med. W., xxxiv. 1233, 1908.
Derm. W., lxxix, 1034, 1924.
Shibata. A. Derm., Kioto, xiii, 69, 1929.
Sidler-Huguenin. Habilitationsschrift, Zürich,
1901.
Soewarno. K. M. Aug., lxiii (2), 285, 1919.
Spicer. Brit. J. O., Monogr. Supp. I, 1924.
Ståhli. A. f. Aug., lxxiv, 13, 1913.
Stein. A. f. O., lvi, 463, 1903.
Stock. K. M. Aug., xliii (1), 640, 1905.
Stoewer. Z. f. Aug., lii, 76, 1924.
Stokes. Ven. Dis. Inform., xiii, 253, 1932.
Swatikowa. K. M. Aug., lxxviii, 688, 1927.
Tome and Bona. Rev. Cub. Oto.-Neuro.-O.,
v, 89, 1936.
Tooke. K. M. Aug., xli., Biel., 158, 1903.
Uhthoff. A. f. O., xxxix (1); (3) 126, 1893.
K. M. Aug., lxi (1), 20, 1918.
Vancea. Cluj. Med., xi, 117, 1930.
Z. f. Aug., lxxiii, 117, 1930.
de la Vega. K. M. Aug., lxxi, 44, 1923.
Verhoeff. T. Am. O. S., xiv, 568, 1916.
Vernon. R. L. O. H. Rep., vi, 292, 1869.
Virchow and Billroth. See v. Graefe: A. f. O.,
vii (2), 35, 1860.
Wachtler. K. M. Aug., lxxiv, 725, 1925.
Walker. Provincial Med. and Surg. J., 293,
1845.
Weekers. A. d’O., xxxvi, 600, 1919.
Weill. Bull. S. d’O. Paris, xxxix, 332, 1927.
Weiss. Med. kl., xxi, 1395, 1925.
Widder. A. f. O., xxvii (2), 99, 1881.
Willett. O. Rec., xxv, 153, 1915.
Woods and McCormick. Wisconsin Med. J.,
xxxvi, 268, 1937.
Yoshida. Nippon Gank. Zash., 1919, Ref.
K. M. Aug., lxv, 140, 1919.
Zeeman. A. f. O., cvi, 1, 1921.
Zimmermann. A. of O., liii, 549, 1924; lvii,
509, 1928.
Zinsser. Resistance to Infectious Diseases,
N.Y., 1931.
4. TUBERCULOSIS
Since attention was first vividly drawn to its prevalence by v. Michel
(1881–90), the subject of tuberculosis of the uveal tract has probably excited
more controversy—which is far from finished yet—than any other question
DISEASES OF THE UVEAL TRACT 2285
in the whole of ophthalmology. The reasons for this are the extraordinary
pleomorphism with which tubercle manifests itself in the uvea, owing to
variations in the virulence and massiveness of the infection and the degree
and nature of the resistance put up by the host, depending on his state of
immunity and allergy, and the fact that, although certain forms, such as
those characterized by clinically visible tubercles (which incidentally are
rare), are readily recognized and agreed upon, much more frequently the
inflammatory reaction excited is nondescript in character and shows nothing
in its clinical picture indicative of its pathology ; the consequence is that the
diagnosis of tubercle is greeted by the observer and his critics with enthusiasm
or scepticism or violent disagreement according to their clinical bias.
The history of uveal tuberculosis is lengthy but interesting, dating back for more
than a hundred years. Maitre-Jan (1707) is usually credited with describing the first
case, that of a soldier in whose eye an excrescence of the iris sloughed through the
cornea ; nearly a century later Autenrieth (1807) described similar choroidal tumours
in a man who died later of the disease we know to-day as generalized tuberculosis, and
Jaeger (1831) annotated a case wherein the disease spread from the ciliary body to
perforate the globe and end in phthisis bulbi. In the interval several such descriptions
had appeared by Saunders (1811), Demours (1818), Delarne (1820), Ritterich (1827),
Lawrence (1830), Lincke (1833) and Mackenzie (1854). Shortly after the advent of the
ophthalmoscope, choroidal tubercle was observed clinically by Jaeger (1855), and soon
thereafter these ophthalmoscopic appearances were proved to be tubercles micro-
scopically by Manz (1858). The whole subject, which hitherto had been somewhat
vaguely conceived by pathologists, was then put on a firm clinical and experimental
basis by Cohnheim (1867) in an important and classical monograph, wherein he showed
that in cases of miliary tuberculosis the choroidal nodules seen clinically were patho-
logically identical with the tubercles seen elsewhere in the body, and demonstrated
that the same appearance with the same histological picture could be produced after
the inoculation of guinea-pigs with tuberculous material. His work, proving that
miliary tuberculosis of the choroid was a metastatic infection and part of a systemic
disease, was confirmed by E. Graefe and Leber (1868) and Waldenburg (1869) in
Germany, and Wells (1868) in England, and shortly thereafter the first microscopic
examination of a tuberculous iritis by Gradenigo (1869) in a patient who died later of
acute miliary tuberculosis put disease of this tissue upon the same sound basis, an
observation rapidly confirmed (Berthold, 1871; Perls, 1873; Saltini, 1875; Manfredi,
1875; Weiss, 1877 ; Parinaud, 1879; and others).
A new chapter was ushered in by the discovery of the tubercle bacillus by Koch
(1882), the presence of which was first demonstrated in a tuberculous lesion in the eye
by Haab (1884). Following the discovery of tuberculin by Koch (1890), while a new
field of therapeusis was introduced, its wide use subsequently as a diagnostic agent in
uveal tuberculosis, particularly in central Europe, greatly extended the type of disease
which came to be considered tuberculous (v. Hippel 1904; and others), a conviction
strengthened by improvements in clinical and radiological examination which
demonstrated the extreme prevalence among the general population of healed or
quiescent tuberculous lesions in the pulmonary and lymphatic systems. The con-
ception that non-specific types of uveal inflammation might well be tuberculous was
strengthened by the response of many of these cases to treatment by tuberculin, and
was confirmed on several occasions by the histological demonstration of tubercles in
eyes which had shown no clinical evidence of a specific pathology (Stock, 1907;
Ogilvy, 1909; Verhoeff, 1929–30; Finnoff, 1931; Meller, 1934; Woods, 1938 ; and
2286 TEXT-BOOK OF OPHTHALMOLOGY
others). The result is that there has arisen a considerable school whose main centre
of inspiration is Vienna (Löwenstein, 1931; Meller, 1932–36), which claims a tubercu-
lous aetiology not only for the classical specific lesions in which obvious tubercles can
be demonstrated, but also in a whole host—even the majority—of non-specific inflam-
mations, especially those of a recurrent or chronic type, and would also include in the
same category almost every chronic inflammation of the uveal tract the aetiology of
which is in doubt (such as sympathetic ophthalmitis, uveo-parotitis, and so on). Since
eyes of this type are rarely available for pathological examination, since a large
proportion of the population of every country and a great majority in some have been
infected with tubercle, and since the response to tuberculin may well be non-specific
in many cases, the question is not susceptible of clinical proof, and still remains a
matter of opinion and therefore of controversy.
It follows that the most diverse estimates are given in the literature
as to the frequency of uveal tuberculous disease, a question upon which we
have already touched." Since they retained the diagnosis for cases showing
clinically recognizable tubercles, the earlier writers considered the infection
rare ; thus Horner (1883) found one case of tuberculous iritis in 4,000 eye
cases, Terson (1890) 2 in 30,000, and Machek (1894) 3 in 40,000. More recent
writers, on the other hand, have varied between two extremes—those
unwilling to admit a tuberculous aetiology unless it is proven, and those who
have found reason to place into this category most uveal inflammations, the
nature of which is doubtful. As representative of the first class we may cite
the following percentages of the incidence of tuberculous irido-cyclitis
among all cases of iritis : Butler (1911) 6%, Lang (1913) 11%, Goulden
(1914) 2%, Irons and Brown (1923), 4%, Bulson (1925) nil, Newton (1925)
2.6%, Gifford (1931) 8.5%. An intermediate class is represented by Baum
(1902) 15%, Elschnig (1912) 28%, Irons (1931) 16%, and Woods and
McCormick (1937) 28% ; and the opposite extreme by Haas (1898) 48%,
Wilmer (1928) 40%, and Gilbert (1929) 45%. It is obvious that these
statistics are matters of faith rather than fact, and as such are quite valueless.
A study of its incidence in tuberculous patients produces information of
more value. All writers are generally agreed that in patients with active
and frank pulmonary tuberculosis, ocular tuberculosis is rare ; I have seen
only one such case, a tuberculoma of the choroid occurring a year after the
pulmonary lesion had been clinically healed after thoracoplasty (Fig. 1917,
Plate XLI).
Denig (1895) was the first to stress this point particularly ; at Würzburg he found
only 5 cases of ocular metastases in 60 cases of tuberculosis of the lungs, 90 of the bones
and joints, and 20 of the lymphatic glands, while of 86 cases of tubercle of the anterior
uveal tract, 67 showed no evidence of systemic disease. The literature from other
countries bears out this incidence. Löwenstein (1923) in central Europe found an
incidence of ocular tubercle in only 0.1% of 40,000 sanatoria patients; the reports of
Glover (1930) and Goldenburg and Fabricant (1930) from America include 1573
patients with pulmonary phthisis, and of these 1.5% had ocular tubercle, while Moore
(1931) found a similarly negligible amount ; in Russia, Witkina and Maklakova (1926)
* p. 2141.
DISEASES OF THE UVEAL TRACT 2287
found 2 cases only in 19,000 sanatoria patients; in Italy, de Benedetti (1930) found
4 in 21,293.; Werdenberg (1935) in Switzerland found a higher percentage, encountering
active pulmonary lesions in 10% of cases of ocular tubercle, and radiological signs of
disease in 30% ; but Grönholm (1928) found the incidence very small in the sanatoria
of Scandinavia. The incidence of ocular tuberculosis in patients with surgical tubercle
(of bone, kidney, etc.) is probably higher than in pulmonary tubercle ; I have seen
only one such case—a ciliary tuberculoma—in 10 years’ attendance at a hospital for
surgical tubercle for children, but Rohrschneider (1933) reports an incidence of 7%.
While active pulmonary tuberculosis is therefore not usually found with
ocular tubercle, it is generally agreed that healed tuberculosis of the lungs or
infection of the lymphatic glands, particularly those of the hilus, forms a
common association. This view has been ably enunciated by Krückmann
(1928) after extensive histological studies, and was supported by Axenfeld
(1930) and others. -
Thus in a study of 100 cases of ocular tuberculosis, Grönholm (1928) found clinical
and X-ray evidence of old or active pulmonary tubercle only in 18%, but of hilus gland
disease in 55%. Similarly, Werdenberg (1935) in 500 cases found barely detectable
hilus shadows in 60%, X-ray evidence of pulmonary tubercle with heavy hilus shadows
but without clinical signs in 30%, and clinically detectable pulmonary disease in 10%.
The first category he found had severe ocular disease associated with the slight chest
lesion (“normal ‘’), the second slight ocular disease and severe chest disease
(“inverse ’’) and the third showed “parallelism ‘’ in that both lesions were severe.
Lower figures are given by Woods (1938); in 150 patients with ocular tubercle, only
2 had active intra-thoracic tubercle, and in only 28% were signs of present or past
tubercle detectable.
It may be taken, therefore, that with the eacception of acute miliary tubercu-
losis, ocular tuberculosis occurs with the greatest rarity in the presence of
active tuberculosis elsewhere, but rather in apparently normal, healthy,
well-mourished individuals with a HEALED OR BENIGN TUBERCULOUS INFECTION
in some organ, usually the lymphatic system in the chest, more rarely in the lungs,
the glandular depôt, even although it is usually slight, quiescent, clinically healed
or calcified, being capable of disseminating infection after the primary pulmonary
focus has healed, presumably by the occurrence of an intermittent bacillaemia
which is usually without systemic significance.
The incidence of ocular tubercle is typical of tuberculosis generally. So
far as age is concerned it generally affects young adults, although it may be met
with at almost any time of life; several cases have been reported in infancy,
and Ogilvy (1909) described a histologically verified case in a man of 72 years
of age. In 32 cases of tuberculosis of the iris Griffith (1890) found the average
age of incidence to be 12 years; the youngest was 4 months and the oldest
52 years. Péchin (1899) found it most common between 5 and 25 years.
The seases are about equally affected, the literature on the whole showing a
preponderance of females. In the miliary forms of the disease both eyes
are usually affected, but in solitary tuberculoma one eye is usually involved,
more frequently the right, a fact which may be accounted for by its more
T.O. —WOL. III. O
2288 TEXT-BOOK OF OPHTHALMOLOGY
direct blood-supply. In the sub-acute and chronic types of plastic and
exudative inflammation the disease is again usually bilateral, although
involvement of the fellow eye may not occur for some years.
Mode of Infection. Since, as we have seen, ocular tubercle occurs
typically in the apparently strong and robust and rarely in those gravely ill
with tuberculosis elsewhere, many of the earlier writers considered that it
must be a primary infection derived from the air or from contact with
infected material. Two schools of thought therefore existed towards the
latter part of the nineteenth century, the advocates of a primary ocular
infection headed by Parinaud (1879), Hirschberg (1882), Poncet (1888),
Denig (1895), v. Michel (1900), and Terson (1904), and those who considered
it secondary in origin (de Wecker, 1876; Fuchs, 1891; Leber, 1891 ;
Axenfeld, 1905). Their opinions, however, gradually became reconciled at the
beginning of the present century by the advances in radiological methods
in the diagnosis of quiescent tubercle in remote parts of the body, which
indicated the extreme prevalence of this disease even in unsuspected cases
and explained the anomaly which confused the earlier workers. The final
proof of the latter contention was the detailed experimental work on animals,
which we shall study presently, demonstrating that uveal tuberculosis was
a blood-borne disease, and that a primary infection of the conjunctiva spread
not into the inner eye but by way of the lymphatic system to the neck and
chest (Lagrange, 1924); consequently to-day it is generally accepted that
wveal tubercle is a secondary infection derived through the blood-stream from
some focus of infection elsewhere in the body, the focus being as a rule a quiescent
and apparently healed lesion of considerable standing, usually in a mediastinal
gland, sometimes in the lungs. The prevalence of such lesions may be
gathered from the fact that microscopically visible evidences of old tubercu-
lous disease could be demonstrated post-mortem in 42% of men of the best
physique in England who died from rapidly fatal injuries in the Great War
(Whitehead, 1922). It is generally conceded also that, with the exception
of the acute miliary form which is part of a general dissemination all over the
body, the mode of infection of the eyes is not by a flooding of the blood-
stream with large numbers of bacilli, but rather as a mild transient bacillaemia.
We have already seen,” that although such an event is probably not so
common as is claimed by some workers (Löwenstein, 1931 ; Popper, 1931;
Urbanek, 1932; Meller, 1934), it certainly does occur; and although it is
claimed by some that a certain degree of organotropism and selective
sensitization must be postulated to explain the frequency of ocular tubercu-
losis and its tendency to bilaterality (Löwenstein, 1931 ; Riehm, 1931), it is
probable that the localization of the bacilli in the eye is largely accidental, and
that their dramatic effects are frequently due to allergic sensitization, the uvea
being a favourable nidus owing to the prevalence in it of elements of the
reticuloendothelial system.”
1 Vol. II, p. 1488. * Vol. II, p. 1457.
DISEASES OF THE UVEAL TRACT 2289
Although endogenous in origin, it is quite possible (although rare) for a tuberculous
inflammation to be flared up or a quiescent lesion to be excited into activity by trauma
or an intra-ocular operation (Block, 1921 ; Black and Haessler, 1930; Lloyd, 1930 ;
Fralick, 1931 ; and others).
In rare cases uveal tubercle may arise from direct eactension from neighbouring
structures such as the cornea, conjunctiva or sclera (Schultze, 1896) or the Optic nerve
or its sheaths (Vejdovsky, 1933), and again it is possible for a miliary lesion to affect
both the conjunctiva and uveal tract simultaneously (Neame, 1922).
Ea:perimental Researches. A very large amount of experimental work
has been done upon the mode of infection of uveal tuberculosis, and it has
thrown valuable light upon the whole subject, not only by establishing
the haematogenous method of infection but in explaining the rationale of the
different forms which the disease may present clinically in different
individuals.
As we have seen, the first experimental work was that of Cohnheim
(1867), carried out before the tubercle bacillus was discovered, wherein he
showed that uveal tubercles were produced after the systemic or intra-
ocular inoculation of guinea-pigs with tuberculous material, findings which
were corroborated by Haensell (1879), Hosch (1882), and others. After the
discovery of the tubercle bacillus these results were put on a more scientific
basis by the demonstration by Felix Lagrange (1898) that an intra-carotid
injection of the bacilli caused miliary tuberculosis of the uvea, a result con-
firmed by Friedrich and Nosske (1899), Schieck (1900–21), Axenfeld (1905),
Stock (1905–07) and Daels (1907), who demonstrated that the reaction could
be either nodular or plastic in type : Finnoff (1923–24), indeed, has shown
that a lesion of any tissue in the eye except the lens can be produced by
injecting the bacilli into the carotid arteries. Similarly, Rollet and Aurand
(1908), Straub (1912), and Ohmart (1937) showed that the intra-ocular
injection of the bacilli produced a like result, in some cases acute and miliary,
and in others chronic and massive. -
A more elaborate analysis of the type of inflammatory reaction produced
was inaugurated by the work of Henri Lagrange (1924) who found that quite
a different tissue-response resulted when normal or previously infected
animals were employed. It will be remembered 1 that it had been estab-
lished at a very early date by Koch (1891) that animals inoculated sub-
cutaneously with tubercle bacilli reacted differently according as to whether
they had previously been infected or not : the normal animals showed a
slowly progressive proliferative reaction with the formation of tubercles,
while the re-inoculated animals showed an acute exudative or necrotic
response. Lagrange found the same phenomenon in the eye, for after the
injection of bacilli into the eye, normal animals showed an insidiously
progressive reaction with tubercle formation and abundant bacilli in
the local lesions, while tuberculous animals immediately developed violent
1 Vol. II, p. 1476.
2290 TEXT-BOOK OF OPHTHALMOLOGY
caseating lesions. These findings were verified by Montalti (1927) who used
tuberculin as well as living tubercle bacilli, and by Guillery (1927) who used
tuberculo-protein—in both cases previously infected animals showed violent
necrotizing lesions. That these allergic reactions could be obtained with
dead bacilli or living was shown by Petroff and Stewart (1925), and since the
defatted bacilli appear to be innocuous (O'Rourke, 1925), it appears that
the lipo-protein of the organism is responsible for them. A further important
observation was supplied by Finnoff (1924) who showed that when dead
bacilli were used, early lesions characterized by tubercle formation appeared,
but were sometimes followed after several weeks by a recrudescence of
activity characterized by caseation, as is typical of the reaction
produced in re-inoculated animals; while Samoilov (1930–31) and Long
(1937) demonstrated that injections of tuberculin in previously affected
animals could light up the initial choroidal lesions into renewed activity,
producing a non-specific inflammatory reaction.
These experimental results, which have been amply confirmed (Frieden-
wald, 1933; Woods, Burky and Friedenwald, 1938; and others), demon-
strate the mechanism of the various forms which tubercle assumes in animals.
The reaction depends on the virulence and massiveness of the infection and
On whether the infection is an original one or a super-infection, that is,
whether the animal is allergic or not. If the animal has not been previously
infected, tubercle bacilli reaching the eye produce a slowly progressive ocular
tuberculosis with tubercle formation. Depending on the quantity of bacilli
reaching the eye these may be miliary or few ; and depending on the viru-
lence of the bacilli these may heal or progress to an intense exudative
inflammation and may ultimately result in caseation and perforation of the
globe. If, on the other hand, the animal has been previously infected, quite a
different picture results from a second infection either with living or dead
bacilli or even with tuberculo-protein—an acute caseating lesion appears
which is quite non-characteristic of tubercle, which in some cases is self-limited
and heals, usually with subsequent recurrences, and in others, depending on
the number and virulence of the bacilli and the resistance of the animal,
progresses with an intense caseating reaction which leads to perforation.
Clinical Manifestations. Based on these experimental researches, we
can classify the clinical manifestation of uveal tuberculosis on a scientific
basis. We can divide them into two main groups depending on the immuno-
logical reaction of the patient, the first group reacting as to an original
infection, the second as to a superimposed infection in an already sensitized
individual. The first is a simple tuberculous infection, the characteristic of
which is a chronic proliferation without marked inflammation ; the second
an allergic reaction, the characteristic of which is an acute and highly
inflammatory response with marked exudation. Each of these can be divided
into two sub-groups depending on the virulence of the organism and the
degree of immunity and allergy displayed by the patient. Since, as we have
DISEASES OF THE UVEAL TRACT 2291
seen, the vast majority of cases of uveal tuberculosis occurs in patients with
clinically quiescent old-standing disease in the thorax, it follows that the
greater proportion of clinical cases falls into the second group, while examples
of the first are rarer.
I. TUBERCULOUS INFECTIONs. This group comprises those relatively
rare cases which are clinically typical of tubercle. They show a slowly
progressive uveal lesion with tubercle formation, and occur essentially in
young individuals and frequently affect one eye only. Clinically, they tend
to progress steadily and without remission for months or at most a year,
finally terminating in cure or caseation and perforation of the eye. Patho-
logically they are proliferative in type showing characteristic tubercles with
lymphocytes, monocytes and plasma cells, and in them tubercle bacilli can
frequently be demonstrated.
1. ACUTE MILIARY LESIONs. If the infection is due to a massive
bacillaemia in an individual who has advanced and progressive
tuberculous disease, that is, in an individual whose resistance is low, an
acute miliary tuberculosis of the uveal tract occurs ; and since the degree of
allergy is low owing to the massiveness of the infection, such lesions show
little tendency to confluence or caseation. If the individual survives the mass
infection, which he rarely does, the degree of allergy may rise so that the
miliary tubercles may tend to coalesce and caseate resulting in a diffuse type
of the disease (2, c) by which the eye may be destroyed. •'
2. CHRONIC TUBERCULOMATOUS LESIONs. While acute miliary tubercu-
losis is the result of a bacillaemia in an individual whose resistance is low,
chronic tuberculomata, as we have seen, result when the infection is less
massive and the resistance high ; although differing in clinical history, the
two lesions are essentially the same, but in the latter the tissues are given
the opportunity of putting up a proliferative defensive response by the
formation of a slowly progressive granulomatous lesion. We have noted
that the earliest cases of ocular tubercle to be described before the advent
of the microscope or the ophthalmoscope were of this nature. The lesions
may be single or multiple, and, depending on whether the infection has
arrived by the long or short posterior ciliary arteries, it appears first in and
is usually limited to the anterior or posterior segment of the globe. In each
locality we may get three conditions depending on the severity of the
infection and the resistance offered by the patient –
(a) A relatively benign and chronic miliary lesion which does not tend
to progress—MULTIPLE CHRONIC TUBERCULOMATA OF THE IRIs ;
CHRONIC TUBERCULOUS CYCLITIS ; DISSEMINATED TUBERCULOUS
CHOROIDITIS ; CHRONIC TUBERCULOUS UVEITIS.
(b) A slowly but steadily progressive lesion with a tendency to con-
fluence resulting in a massive and frequently ultimately destruc-
tive CoNGLOMERATE TUBERCLE of the iris and ciliary body or the
choroid.
2292 TEXT-BOOK OF OPHTHALMOLOGY
(c) A rapidly progressive and destructive lesion—DIFFUSE PROLIFERA-
TIVE TUBERCULOSIs. In these cases resistance is low and an
exudative caseating inflammation develops which spreads easily
to the deeper structures of the eye involving them in necrosis.
Such cases are rare and involve rapid loss of vision and usually
loss of the eye.
II. DIFFUSE ALLERGIC INFLAMMATIONs. This group comprises the
great mass of cases of uveal tuberculosis, and these are clinically non-specific
in character, showing nothing characteristic of tuberculosis. They occur in
persons who have had tuberculosis previously and have developed a high
allergic sensitization. They may be divided into two types:–
(a) An ACUTE PLASTIC INFLAMMATION, rapidly localizing and self-
limiting in character.
(b) A CHRONIC AND RECURRENT ExUDATIVE INFLAMMATION, which
pursues a slow non-destructive course, and is the more common
type.
The first occurs with a relatively massive infection in an individual
with a low resistance and a moderate degree of allergy, the second with a
minor infection in an individual whose resistance is high and whose allergy
is marked. As a rule the first occurs in young adults, the second in middle
age. Between the two extremes there is, of course, no hard and fast line,
but a gradual variation depending on the number and virulence of the
organisms and the degree of immunity and allergy of the host. Histologically
typical tubercles are not regularly formed, although small hidden nests of
epithelioid cells are met with in the deeper parts of the eye, tubercle bacilli
can rarely be demonstrated, and caseation and perforation are extremely
uncommon. At any later period, provided the state of allergy remains high,
re-infection with tubercle bacilli or their products is liable to light up the
condition again so that a recurrence of the inflammation occurs.
While these more or less definite types may be said to occur and form
a useful basis for the classification of the clinical types of tuberculous disease,
it must not be imagined that they form hard and fast groups. One type may
merge into another or be met with in company with another so that a mixed
picture is formed ; and in the course of years the disease may change its
clinical characteristics and gradually assume different attributes, depending
sometimes on a change in the virulence of the infection and at others either
on an alteration of the patient’s general immunological state or on the
degree of allergy attained by the ocular tissues. Thus after the initial
infection of the eye by tubercle bacilli the slow response of tubercle formation
occurs, but at the same time the diffusion of tuberculo-protein may sensitize
the tissues. Once this has been established a further diffusion of tuberculo-
protein may produce a rapidly spreading allergic inflammatory reaction
characterized by necrosis and caseation. With neutralization of the tissue
reagin, this allergic reaction subsides only to flare up again if re-sensitization
DISEASES OF THE UVEAL TRACT 2293
occurs. Such re-sensitization could occur from the local lesion itself by the
continued diffusion of tubercle-protein, or as easily from a remote lesion
elsewhere in the body. And at any time if the infection becomes massive
or the general resistance falls, allergy may diminish and the whole clinical
picture may change. In general terms it may be said that in youth
(10–20 years) the proliferative and caseating types predominate, in early
adult life (20–30 years) when allergy is moderate, the acute plastic types of
inflammation, and in later life (30–60 years) when allergy has become
marked, the non-specific progressive exudative types are most common.
ACUTE MILIARY TUBERCULOSIs. Acute miliary tuberculosis of the
uveal tract is merely an incident in the general systemic metastases which
occur when an actively progressive lesion in some distant part of the body
gains access to the blood-stream by necrosing through the wall of a blood-
vessel ; it is therefore associated with similar disseminated lesions elsewhere,
usually in the meninges and most of the internal organs, and is thus a
terminal event in a patient already gravely ill. Occasionally it is the first
evidence of disseminated tuberculosis and remains the only evidence for some
weeks (Cargill and Mayou, 1906), when it forms a sign of considerable
diagnostic importance. The tubercles may be found in any part of the uveal
tract, but are only rarely seen in the iris, being much more common in the
choroid, particularly in its posterior half in the region of distribution of the
posterior ciliary arteries.
First seen ophthalmoscopically by Jaeger (1855), they were studied extensively
by Bouchut (1866-75) and Galezowski (1867), while their common occurrence and mode
of formation was demonstrated both clinically and experimentally by Cohnheim
(1867); indeed, miliary choroidal tubercles are seen in some 50% (Carpenter and
Stephenson, 1901), 75% (Litten, 1877), or 80% (Bock, 1883) of all cases of
disseminated tuberculosis. Galezowski (1867) associated them particularly with
tuberculous meningitis, believing that the infection followed the sheath of the optic
nerve along the retinal vessels. Experimental work, however, demonstrated their
haematogenous origin, and it is to be noted that although a miliary tuberculosis of the
uvea frequently occurs with a similar meningeal lesion, and although the vaginal
space of the optic nerve may be affected, the two are parallel but independent
phenomena (Tooke, 1936).
In the iris acute miliary tuberculosis (Fig. 1908, Plate XL) is not
commonly seen clinically and is generally associated with a similar
involvement of the whole uveal tract. The iris tissue, especially near the
root, is peppered with small greyish-yellow or reddish nodules clearly
visible with the slit-lamp, this being superimposed on a typical picture of
a moderately severe iritis with pericorneal injection and photophobia, and
usually a cloud of fine keratic precipitates. Quite frequently a diffuse iritis
appears first, indistinguishable from a non-specific inflammation, in the
midst of which the nodules appear subsequently. As a rule the patient
dies before the disease has run its course. Occasionally the condition may
2294 TEXT-BOOK OF OPHTHALMOLOGY
improve and relapse later, as in the case recorded by Samuelsohn (1893) of a
boy of 6 years in whom the tubercles disappeared only to reappear with
meningitis and death. In the few cases which do recover, the eye either
becomes blind and atrophic, or the process continues, the nodules become
confluent, and the globe ruptures and becomes phthisical.
In the choroid similar nodes are seen as greyish-white spots with blurred,
indistinct edges under an area of retinal oedema, over which the retinal
vessels bulge as they pass (Fig. 1914, Plate XLI). The nodules vary in size
Fig. 1906–MILLARY TUBERCLE or THE Choroid.
A recent tubercle with dense circumscribed leucocytic infiltration
(Tooke, Brit, J. O.).
from pale pin-point specks to a lesion 0-5 to 2 mm. in diameter, to which
size they may attain in a little over a week (Botteri, 1909); occasionally the
confluence of smaller foci may lead to larger formations. Any part of the
choroid may be attacked, but the site of election is at the posterior pole around
the disc, although they have been found pathologically further forward than
the limits of ophthalmoscopical examination. They are usually relatively
few in number, some three or four being the most common, but quantities up
to 52 (Cohnheim, 1867), 60–70 (Fraenkel, 1870) and 70 have been observed
(Morton, 1891). As a rule this picture remains until death; but in those

PLATES XL AND XLI
UWEAL TUBERCULOSIS
[To face p. 2294.
PLATE XL
TUBERCLE of THE ANTERior segment
Fig. 1907–Floccule
on IRIs.
º
Fig. 1910–ConglomeRATE Tubercle of
THE IRIs.
Fig. 1908.-MILIARY TUBERCLE of IRIs.
Fig. 1909.-NoDULE
on IRIs.
Fig. 1911.-Cosglovenate Tubercle OF
THE IRIs.
Fig. 1912–BLAstro Tuberculous
IRudo-cycliſtis.
Fig. 1913–Tuberculous Sciero-
- ERATITLs.







PLATE XLI
TUBERCLE or THE Post ERIoR SEGMENT
Fig. 1914.—MILIARY TUBERCUI ous Fig. 1915.--DissEMINATED TUBERCULouis
CHoRoidITIs. CHoRoibitis.
Fig. 1916.-SolITARY TUBERCULous Fig. 1917.-CoxglovieRATE TUBERCLE.
ChoroidITIs.


DISEASES OF THE UVEAL TRACT 2295
cases where death is delayed or the patient recovers, the tubercles may
coalesce and necrose so that the lesion progresses to destruction of the eye.
Rarely in milder cases, about the third week, they may begin to heal,
becoming bright yellow or white in colour with an aggregation of pigment
as a ring round their borders or a dark spot in the centre, producing a picture
of disseminated choroiditis such as is seen in the chronic form of this disease
to be noted presently (Fig. 1915, Plate XLI).
Pathology. Shortly after the first miliary tubercles of the choroid were seen
ophthalmoscopically by Jaeger (1855), their first microscopical examination was
Fig. 1918.-ChoroidAL TUBERCLE.
Chronic tuberculous formation showing encapsulation with numerous epithelioid
cells, giant cells and marked coagulation necrosis (Tooke, Brit. J. O.).
published by Manz (1858), whose findings have been amply confirmed by subsequent
writers (Busch, 1866; Cohnheim, 1867; E. Graefe and Leber, 1868 ; Poncet, 1875;
Dinkler, 1889; Margulies, 1898–1903; Finnoff, 1931; Tooke, 1936; and many
others). The lesion may be described as a tuberculous area rather than as a sharply
defined tubercle; there are numbers of giant cells with peripheral nuclei,
surrounded by epithelioid cells with a peripheral zone of lymphocytes; in the larger
tubercles the centre is often caseous. In the iris they appear in the stroma and
involve little change in the anterior border layer; later the pigment disappears
and the nodule begins to protrude through the surface. In the choroid (Figs, 1906,
1918) they are found usually in the chorio-capillaris, although they occur in the middle

2296 TEXT-BOOK OF OPHTHALMOLOGY
layers developing from the adventitia of the larger vessels (Manz, 1858), or may even
Surround the large vessels of the outer layer ; nor do they always project inwards,
but sometimes invade the supra-choroidea and invade the sclera. In the affected
area the choroid disappears except for pigment remnants which may be seen engulfed
in the giant cells ; the blood-vessels are obliterated, their walls degenerate and the
endothelium proliferates, although sometimes in the centre a large patent vessel
remains (Manz, 1858–63; Margulies, 1898).
Over the smallest lesions the pigment epithelium may remain unchanged, but
when they attain any considerable size the pigment disintegrates. Apart from Oedema,
the overlying retina remains normal (Fig. 1906). The choroid surrounding the nodules
is hyperamic but usually shows few inflammatory changes apart from some lympho-
cytic infiltration ; more marked generalized changes occasionally occur (Weiss, 1877),
but are rare.
Tubercle bacilli are frequently found in these lesions but in very varying numbers.
Sometimes they are very numerous, especially in the caseous areas (Penman and
Wolff, 1932), sometimes they are seen in the vessel walls (Margulies, 1903; Botteri,
1909) or within the lumen of the vessels (Wedl and Bock, 1886), and at other times they
are seen only sparingly or not at all.
CHRONIC TURERCULOMATOUS I,ESIONS.
(a) Chronic Miliary Tuberculomata of the Iris. Chronic miliary
tuberculomata of the iris can be described as a chronic and relatively benign
form of miliary tuberculosis, the essential difference between this and the
acute form being perhaps that the infection is benign and attenuated, or
more probably that the patient has sufficient resistance to allow the tissues
to put up a proliferative defence to the invasion. Occasionally the whole
stroma of the iris and ciliary body are studded with innumerable nodular
granulomata, but more frequently they are aggregated in the region of the
lesser arterial circle near the pupillary margin (Fig. 1933), or near the angle of
the anterior chamber (Fig. 1919). Their appearance is sometimes preceded
clinically by a diffuse exudative iritis of considerable severity, sometimes
with haemorrhagic extravasations, more often with profuse exudation, which
may form a hypopyon ; this, indeed, forms one type of “iritis with
recurring hypopyon.” ". In mild cases, however, inflammatory processes
may be little in evidence, the whole illness being accompanied by little or
no pain, and the eye remaining extraordinarily quiet. At first, they show up
as small grey elevations in the stroma but gradually as they grow to 1 to
3 mm. in size, the colour changes to yellow and they become superficially
vascularized. -
The clinical course varies in different cases but a termination either in
resolution or loss of the eye is attained in a few months. Sometimes in milder
cases the process subsides and the nodules undergo complete absorption—
the attenuated tubercle of Leber (1891). Indeed, this may occur before the
nodule has broken through the surface in which case no clinical evidence of
disease may be left. Alternatively, when a considerable amount of tissue
destruction has occurred, an atrophic patch is left in the stroma in addition
1 p. 2227.
DISEASES OF THE UWEAL TRACT 2297
FIG. 1919.-TUBERCULOU's IRITIs.
Specific infiltration of root of iris (i) with little involvement of the ciliary body (c),
a is the cornea. This is a common starting-point of uveal tuberculosis (Igersheimer).
Fig. 1920.-TUBERCULouis CYCLITIs,
The cyclitic type of tuberculosis of the anterior uvea, showing infiltration of
the iris with commencement of thinning of the corneo-sclera at the limbus (1)
(Igersheimer).


2298 TEXT-BOOK OF OPHTHALMOLOGY
to a minute scar (v. Michel's flecks), and if the pigment epithelium has been
involved a translucent area remains on transillumination. At the other
extreme the process may progress, the nodules grow and fuse so that the
greater part of the iris gradually becomes converted into a mass of granulo-
matous tissue (Fig. 1921), or an immense confluent mass is formed in one
area, usually surrounded by satellites to constitute the picture of
conglomerate tubercle (vide infra).
(b) Chronic Tuberculous Cyclitis. The ciliary body is frequently the
seat of a relatively benign and very chronic form of tuberculosis (Fig. 1920).
Fig. 1921–Tuºrºcutious IRITs.
The iris is thickened by granulomatous infiltration. There is also the tuberculous
interstitial keratitis with dense infiltration of the deep corneal lamellae (X 10)
(Parsons).
Since the tuberculous nodules are not seen in the earlier stages, the nature
of the disease can only be inferred when it has spread to other parts of
the eye. Sometimes it starts with a severe plastic iritis; but quite
frequently the symptoms are so mild and the process so quiet that it is not
detected until it has advanced far. All that is to be seen is a slight ciliary
injection, a few large mutton-fat keratic precipitates, and perhaps one or
two small translucent nodules on the pupillary margin (Fig. 1922).
These nodules, originally described by Gilbert (1914) and Koeppe (1917) and
histologically examined by Vogt (1923), Derby (1928), and Busacca (1932), have
already been discussed." It will be remembered that they may either appear on the
ectodermal layer of the pupillary margin (nodules), or on the mesodermal tissue of the
p. 2180.

DISEASES OF THE UWEAL TRACT 2299
anterior surface of the iris (floccules) (Figs. 1907–1909), and that they are partly
exudations from the stroma and partly depositions from the aqueous. Although
they occur in syphilis, leprosy and sympathetic ophthalmitis, they are most charac-
teristic of tubercle.
The illness may go on for some time in this way, perhaps causing some
deterioration of vision, but sometimes escaping notice altogether until the
development of secondary glaucoma, which proves unamenable to treatment,
first indicates that something is seriously at fault; and it is from the
examination of such eyes after they have been enucleated for this reason
that we have gathered most of our knowledge of early ciliary tuberculosis
(Bergmeister, 1927; Finnoff, 1931). The clinical signs which should excite
Fig. 1922.-TUBERCULous NoDULEs on THE PUPILLARY MARGIN AND THE
ANTERIoR LEN's CAPsule. IN TUBERCULouis CYCLITIs (v. Szily, K. M. Aug.).
suspicion in such quiet but progressive cases are (a) mutton fat keratic
precipitates, (b) Koeppe nodules on the pupillary margin, (c) vitreous
opacities, and (d) a tendency to a rise in tension.
The disease usually starts in the inner vascular layer which rests upon
the inner surface of the ciliary muscle in the region of the pars plana or
corona (Fig. 1923). Here tuberculous nodules form, which as they grow
push inwards to destroy the epithelium and open into the posterior chamber
(Fig. 1924). Thence spread occurs by two routes. In the first place infection
is carried forwards by the current of the aqueous, first of all depositing the
typical keratic deposits (Fig. 1925), and then involving the iris, the angle of
the anterior chamber (Fig. 1926) and the cornea in a progressive kerato-
iritis (Fig. 1927). It is probable that this anterior spread is by way of
the posterior chamber, the pupil, the anterior chamber, and through its angle
to the sclera, episcleral tissue and the conjunctiva (Fig. 1928) (Verhoeff,
1910–30), and is comparable to the diffuse meningitis which follows a
solitary caseous lesion in the brain or meninges. Simultaneously, infection

2300 TEXT-BOOK OF OPHTHALMOLOGY
Figs. 1923 and 1924–Tuberculous Nodules being Formed rºom the CILIARY
Body (v. Szily, K. M. Aug.).
Fig. 1925. – C or N E A L Fig. 1926–ANGLE of the ANTERior
PR E c IP 1 T. A. T E IN CHAMBER (v. Szily, K. M. Aug.).
TUBERCULouis CycLitis.


DISEASES OF THE UVEAL TRACT 2301
Fig. 1927.-UveAL TUBERCULos Is.
The cornea (c), showing keratitis profunda, with much thickening behind
Descemet's membrane (m) (Igersheimer).
is carried backwards through the vitreous along the perivascular lymph
spaces of the retinal veins (Meller, 1921; Finnoff, 1931), setting up a peri-
vasculitis which results in recurrent haemorrhages (Fig. 1929), and some-
times to the optic nerve to form a papillitis (Fig. 1930)."
- - - º
-- - * *** * - º -
ºº: Sºº -- º
º
- ..º. sº tº ººººº...
5- ºr-. "Tºw. º º: -----
*ś
Hºº
Spread from the root of the iris along anterior ciliary vessels to selera and
conjunctiva (Igersheimer).
Fig. 1928.-UVEAL TUBERCULosis.
1 p. 21.89.







2302 TEXT-BOOK OF OPHTHALMOLOGY
v. Szily (1931) has indeed demonstrated histologically the presence of migrating
clumps of cells, particularly of giant cells (which he called wander-tubercle) on the lens
capsule, the iris and the posterior surface of the cornea as well as in the angle of the
º
º
-
Fig. 1929–UVEAL TUBERoulosis.
The retina, showing infiltration around retinal vessels with an extension through
the internal limiting membrane (A) and a tubercle on the inner surface (B)
(Igersheimer).
Fig. 1930.-UVEAL TUBERCULosis.
Showing papillitis and peri-vascular infiltration (Igersheimer).
anterior chamber and the vitreous, and it seems that these represent the vehicle of the
dissemination of infection. It will be remembered also, as we have already seen,” that
Straub (1912) and Ohmart (1937) showed experimentally that by inoculating the
+ p. 21.89.




DISEASES OF THE UVEAL TRACT - 2303
ciliary body with tubercle bacillian optic neuritis developed, sometimes of considerable
severity, apparently by dissemination through the vitreous (Fig. 1835).
Tuberculomata may also have their origin in the perichoroidal lymph space
between the ciliary muscle and the sclera; the ciliary muscle itself is relatively immune.
Thence the disease spreads easily forwards to the scleral spur, the pectinate ligament
and the base of the iris, or backwards into the choroid, or outwards through the sclera,
in which case a sclerosing keratitis' develops; in severe cases this results in
perforation. - -
(c) Tuberculous Disseminated Choroiditis. In the choroid single or
multiple small tuberculomata (sometimes called chronic miliary tubercu-
losis without general miliary tuberculosis) are probably not so rare as is
commonly supposed. Such lesions occur most usually in the second
* - -
sº sº… 3.…..:
º - - - - - -- ---
sºsºº:: **** º
º - - - - º -
. - - - - - - -
*ś
Fig. 1931.-UVEAL TUBERCULosis.
Infiltration of the choroid with atrophy of the pigment epithelium and the
external layer of the retina to form a chorio-retinal lesion (Igersheimer).
and third decades of life and are rare after middle age. Although not simul-
taneously affected as a rule, both eyes are generally involved in some degree.
The condition is characterized by the appearance of yellowish-grey round
nodules in the choroid over which the retina becomes necrotic, and is
associated with vitreous opacities and often with an accompanying cyclitis.
After the process subsides there are left atrophic pigmented areas in the
fundus, which produce the clinical picture of tuberculous disseminated
choroiditis (Figs. 1931, 1914-15, Plate XLI); (v. Michel, 1890; Bach,
1893; Schultz-Zehden, 1905; Ginsberg, 1910; Gilbert, 1919).
(d) Chronic Tuberculous Uveitis. Not infrequently the whole uveal
tract is involved in a low-grade uveitis, which persists either with
periodic exacerbations and remissions which nothing controls, or runs a
quiet symptomless course for years until eventually vision is seriously
interfered with by exudation in the pupil, the slow development of a com-
plicated cataract, or an involvement of the macula. In some such cases a
1 Vol. II, p. 2058.
T-0.-WOL-III- p


2304 TEXT-BOOK OF OPHTHALMOLOGY
loss of the pigment in the iris produces the picture of heterochromic cyclitis.”
An important feature of these cases is the frequency with which the milder
type of lesion persists over years without claiming attention to itself and
without serious symptoms except a slight loss of vision (the ºritis obturams
of Schieck, 1920). An occasional nodule on the iris margin, a minute precipi-
tate on the cornea, a few vitreous opacities, or some mild changes at the
posterior pole of the lens may persist almost indefinitely without serious
effects; or a disseminated choroiditis starting in youth may remain practi.
cally stationary for years until, when middle life is past, relapses become
more numerous and recalcitrant and vision is gradually lost; or until
suddenly a fresh nodular lesion develops, indicating a lowering of the general
resistance, and perhaps coinciding with a recrudescence of the primary
lesion or preceding a generalized systemic dissemination.
CONGLOMERATE TUBERCLE
(a) In the Anterior Segment. We have already seen that in unfavourable
cases a tuberculous module on the iris or in the ciliary body may proliferate
and grow, sometimes gathering into itself adjacent modules to become a
Fig. 1932–TUBERCULoMA or IRIS FILLING ANTERIOR CHAMBER
(Finnoff, Am. J. O.).
confluent granuloma of considerable size, usually surrounded by satellites
(Figs. 1910–11, Plate XL). Such a conglomerate tubercle appears as a
yellow mass growing from the iris stroma and may easily be mistaken for
a malignant tumour. It becomes heavily vascularized by blood-vessels
passing over its surface but not penetrating into its substance, and tends
slowly to progress until it may entirely fill the anterior chamber (Fig. 1932).
During this proliferative period the mildness of the pain and subjective
symptoms are quite improportionate to the objective appearances, but
1 p. 2360.

DISEASES OF THE UVEAL TRACT 2305
sero-fibrinous exudates, haemorrhages, and the formation of a caseous
hypopyon may occur at any time. If the angle of the anterior chamber
is involved, however, a very resistant secondary glaucoma may develop.
Even at this stage progress may stop, the growth may subside and
eventually disappear, leaving behind a scarred and atrophic iris; but
frequently progression is continuous. The direction of growth is always
forwards, for as was pointed out by Lagrange (1901), the ciliary muscle
presents a sufficient barrier to backward extension to protect the choroid
and the supra-choroidal space. Instead, the infection tends to confine
Fig. 1933.-TUBERCULou's IRIDo-cyclitis.
On the left the iris has prolapsed through a tuberculous perforation at the limbus
forming a large granulomatous mass outside the globe. On the right a typical
tubercle in the iris near the pupillary margin ( x 8) (Parsons). -
itself to the anterior segment, the infiltration follows the channels of drainage
of the intra-ocular fluid, infiltrates the spaces of Fontana at the angle of the
anterior chamber, and invades the substantia propria of the cornea to
produce a tuberculous sclerosing keratitis. Occasionally a growth near the
pupillary margin will extend across the anterior chamber and, approaching
the cornea, will produce an interstitial keratitis in the central region, or
even may invade the cornea directly, rupturing Descemet's membrane
(Finnoff, 1931; Siegrist, 1934). The story is completed by perforation
of the globe which occurs through the region of the angle of the anterior
chamber, granulomatous tissue tracking obliquely through the sclera past
the canal of Schlemm along the anterior ciliary veins (Fig. 1933). Finally,
a large fungating mass appears on the surface of the globe, which, after

P 2
2306 TEXT-BOOK OF OPHTHALMOLOGY
continuing to grow for some time, tends to caseate and shrink, involving
the development of phthisis bulbi or panophthalmitis. In such cases the
prognosis is frequently extremely bad, the disease progressing in spite of all
treatment, demanding on occasion excision of the eye for the relief of pain,
a calamity which has been bilateral (Butler, 1930). It frequently happens,
however, in these severe cases that general dissemination occurs before this,
and the patient dies of miliary tuberculosis.
(b) In the posterior segment a conglomerate or solitary tubercle of the
choroid is rare ; thus zur Nedden (1903) found only 34 cases in the literature,
adding 4 of his own, and Natanson (1910) collected another 12. The lesion
may occur anywhere in the choroid, most usually near the posterior pole,
Sometimes at the macula, sometimes near the disc (juxta-papillaris),but often
equatorially or near the ora. In its earliest stages it appears as a whitish-
grey area with indistinct margins, which gradually grows until it resembles
an intra-ocular tumour projecting as a round white or yellow globular mass
into the cavity of the eye (Fig. 1917, Plate XLI). Frequently, however,
round its edges white fluffy masses indicate its inflammatory origin. Over
it the retinal vessels course normally, but occasionally in the active stage
small haemorrhages are formed on the surface, while the retina itself is pulled
tight in crinkled folds, until eventually this tissue may become detached
and the fundus details become obliterated. Less frequently it spreads
diffusely in the choroid, forming an extensive flat growth (Manfredi, 1875;
Poncet, 1875; Hirschberg, 1882; Wagenmann, 1888; and others). In this
way it may progress slowly for months, accompanied sometimes by a
cloud of vitreous opacities, and occasionally by a mild and chronic anterior
uveitis as indicated by a few keratic precipitates and nodular excrescences
at the pupillary margin, appearances which are shown histologically to be
due to small collections of lymphocytes without epithelioid cells. Occasion-
ally, also, an optic neuritis may occur as a complication (Cummins,
1924).]
Such tubercles may progress for several months and then regress with
the development of a white scar involving the choroid and the retina, usually
surrounded by a densely pigmented zone. In this state it may remain
Quiescent throughout the patient’s life, but sometimes recrudescences occur,
when activity becomes evident usually at one point on the margin of the
scar where a fresh nodule appears, a process which may be repeated several
times (Jackson, 1919; Lloyd, 1930; Finnoff, 1931). More frequently,
however, progress continues, Bruch's membrane is attacked and the retina
is involved, the vitreous opacities increase, a proliferating retinitis may
ensue, an irido-cyclitis develops, at first, perhaps, of the quiet type,
characterized by Koeppe's nodules on the pupil and a few keratic deposits,
but frequently developing into a frank exudative inflammation with
synechiae, while lenticular opacities appear at the posterior pole of the lens
1 p. 3036.
DISEASES OF THE UVEAL TRACT 2307
and slowly progress. Just as the ciliary muscle acts as a barrier against the
backward spread of an iridic tuberculoma, so it tends for a long time to
confine a choroidal granulomatous process to the posterior segment
(Lagrange, 1924; Eltore, 1934), so that finally the sclera is involved, and
perforation results which is followed by phthisis bulbi or by pyogenic
infection and panophthalmitis.
In some of the cases of nodular uveitis, particularly of the benign miliary type,
symptoms of cerebral disturbance, such as headache (Gilbert, 1927) and increased
cerebro-spinal pressure (Heine, 1914) suggest a similar meningeal involvement.
DIFFUSE PROLIFERATIVE TUBERCULOSIS
This is a rare type of inflammation, usually coming on acutely and
characterized by intense inflammatory signs, rapid spread, and a tendency
FIG. 1934.—TUBERCULOU's UVEITIs.
Note spontaneous dislocation of the lens (x 3) (Parsons).
to destruction of the eye. Such a process may be confined to the anterior or
posterior segments or may involve the whole uveal tract (Fig. 1934). In the
iris there is a generalized confluent infiltration of the tissue with great
thickening and a filling of the anterior chamber with material resembling
pus (Edmunds and Brailey, 1882). In the clinical picture there are no
pathognomonic signs to distinguish this type of tuberculous disease, but
sometimes the appearance of nodules in the stroma makes the diagnosis clear.
In the choroid a similar diffuse and intense inflammation occurs, usually
affecting a large area at the posterior pole. Sometimes this forms a lenticular-

2308 TEXT-BOOK OF OPHTHALMOLOGY
shaped mass and then settles down, but frequently the membrane of Bruch
is destroyed, the retina is involved and great masses of granulation tissue
invade the vitreous chamber so that the greater part of the globe is filled
with necrotic tuberculous material constituting a sub-acute panophthalmitis
(Fig. 1935), the appearance sometimes resembling a glioma, and being
clinically visible to the naked eye (amaurotic cat’s eye) (Fig. 1848). If
the process does not become quiescent the optic nerve may be invaded, but
this is rare; more usually the sclera is infiltrated first and perforation occurs
either at the angle of the anterior chamber, under the conjunctiva at the
Fig. 1935-Tube Roulous ChoroidITIs.
There has been a perforation of the sclera and the retina and large areas of the
choroid are necrotic. From a girl aged 1 (x 3) (Parsons).
perforations of the anterior ciliary vessels, or at the equator near the exits
of the vortex veins. In the latter event a yellow tumour appears under the
conjunctiva, which when it bursts or is incised, demonstrates itself as a
pus-containing cavity continuous with the vitreous chamber. It is interesting
that the discharge from such a panophthalmitic eye often contains tubercle
bacilli in abundance (Koyanagi, 1934; Katznelson, 1936; and others).
Sometimes a mixed infection occurs (Demaria, 1905), or the process runs rapidly
apace in the puerperium (Lüttge, 1903; Kellermann, 1908).
Pathologically such granulomata have the usual tuberculous structure. The first
examination to be recorded was by v. Graefe (1855) in the eye of a pig." Gradenigo
* Uveal tubercle is not uncommon in animals: cattle (Hess, 1891; Moncet, 1895,
Schmidt, 1899; Lothermoser, 1905; Spörer, 1905; and others); cats (Coats, 1912; Lawford
and Neame, 1932).

DISEASES OF THE UVEAL TRACT 2309
(1870) made the first contribution to the histology of modular iritis, a description
followed by many others (Berthold, 1871; Saltini, 1875; Manfredi, 1875; Weiss,
1877; Haab, 1879; Parinaud, 1879; Poncet, 1882; Swanzy, 1882; Liebrecht, 1890;
Derkac, 1924; Petrovic, 1926; Brüsselmanns, 1926; and others). The granulomata,
wherever they occur, consist of groups of giant cells with epithelioid cells surrounded
by lymphocytes generally with extensive caseation, the many nodules making up the
tumour mass being united by dense lymphocytic infiltration. In the surrounding
area there is usually great vaso-dilatation (Rüter, 1881).
Histological descriptions of tuberculous disseminated choroiditis show a similar
granulomatous picture of chronic inflammation (Schultz-Zehden, 1905; Ginsberg,
1910; Gilbert, 1919; and Meller, 1932) (Figs. 1906 and 1918).
Fig. 1936.-TUBERCLE of THE CHoRoid.
There is an absence of typical tuberculous systems, especially of epithelioid
cells (x 120) (Parsons).
The diffuse type of reaction is characterized by an intensive infiltration with
lymphocytes and epithelioid cells throughout the involved tissue; there is also
scattered through it an abundance of giant cells, but typical tubercle systems do not
usually occur (Fig. 1936). Large areas undergo caseation and tissue necrosis is a
prominent feature. Sometimes when the choroid is affected the necrosis is limited to
the retina, and in a few cases a peculiar layer of epithelioid and giant cells is formed
between it and the choroid associated with necrosis on its surface (Lüttge, 1903;
Botteri, 1908; Weigelin, 1910; Brown, 1912; Meller, 1913; v. Hippel, 1917–18–26;
Stock, 1923; Kägi, 1924 Igersheimer, 1933).
EXUDATIVE (ALLERGIC) TUBERCULOUS UVEITIS
This form of uveitis is quite atypical, and for this reason, as we have
seen, the diagnosis is usually a matter of doubt and controversy. It occurs
in adults, usually affecting both eyes, and is non-destructive in the sense that

2310 TEXT-BOOK OF OPHTHALMOLOGY
the inflammatory attacks are self-limited and cause damage only in a non-
specific manner. It is in the great chronicity and recurrent habit of the
inflammation that its danger lies. It may be divided into two types, an
acute plastic type occurring generally in young adults, and a chronic and
relapsing type occurring typically in older people.
(a) The acute plastic type is a highly inflammatory condition which,
appearing as an irido-cyclitis, is characterized by considerable pain, some
plastic exudate and the rapid development of posterior synechiae; an
occasional complication is a sclerosing keratitis (Fig. 1913, Plate XL).
Granulomatous nodules are absent but one feature of considerable
diagnostic value may be present—the occurrence of small translucent
Roeppe nodules on the pupillary margin of the iris. On rare occasions the
iritis has died down and recurred at a later period with typical nodular
formations, thus indicating the diagnosis (Jacqueau and Bujadoux, 1924).
In the choroid a typical exudative patch of choroiditis occurs, which is
quite frequently single, with perhaps some satellite patches round about it.
It may occur in any part of the fundus with a preference for the periphery,
and is usually about the size of the optic disc or sometimes larger (Fig. 1916,
Plate XLI). At first there may be only a fine haze of vitreous opacities,
but later these usually become intensified into a dense cloud, while the
development of keratic precipitates and posterior synechiae frequently
indicates involvement of the anterior segment of the uveal tract. After the
process subsides there is left an atrophic pigmented area of the fundus, which,
however, shows some tendency to undergo relapses, either at its edges or in
its near vicinity.
(b) The chronic or relapsing type occurs typically in older people,
particularly in women about 50 (Fig. 1912, Plate XL). The irido-cyclitis is
highly exudative, large mutton-fat deposits plastering the posterior surface
of the cornea, massive synechiae and inflammatory exudation binding the iris
firmly to the lens and occluding the pupil ; while, if a view can be obtained
through the pupil, a dense cloud of opacities is seen to fill the vitreous and
obscure the fundus. Such a condition is frequently bilateral, exceedingly
chronic and prone to relapse, and may well end in blindness. In the
choroid a similar type of lesion may occur, but probably not so commonly,
although it is considered so by many Continental writers (Gilbert, 1929).
The typical lesion is a disseminated choroiditis which by reason of its
recurrent nature may eventually leave only relatively minute areas of the
fundus unaffected.
While there is nothing in the clinical picture presented by these exuda-
tive lesions to prove, or sometimes even to suggest, a tuberculous aetiology,
the histological appearances show nothing specific. The difficulties are
increased by the rarity with which these eyes come to pathological examina-
tion. Tubercle formation is not regularly found, caseation is extremely
rare, and the bacilli cannot be demonstrated. Occasionally in the deeper
DISEASES OF THE UVEAL TRACT 2311
parts of the eye are small hidden nests of epithelioid cells (Igersheimer,
1933), but more usually non-specific infiltration followed by non-specific
scarring constitutes the entire pathological picture.
So much so is this the case that, as we have seen, the tuberculous aetiology of such
cases is frequently strenuously denied ; but that an inflammation which clinically
appears quite non-specific may in fact be tuberculous has been demonstrated. Thus
Verhoeff (1910–30) sectioned the eyes of 7 patients with non-specific irido-cyclitis and
choroiditis, all of whom showed histologically a tuberculous inflammation with the
acute exudative response typical of an allergic reaction ; he concluded, indeed, that
all cases of localized exudative choroiditis were either tuberculous or syphilitic.
Similarly in 10 eyes examined at the Wilmer Institute showing a completely non-
specific clinical picture, in four of which tubercle was not suspected, Woods (1938)
showed histologically demonstrable tuberculosis. Other such cases have been
described by Stock (1907), Ogilvy (1909), Finnoff (1931), Bonnet and Colrat (1935),
and others.
Prognosis. From the preceding paragraphs it will be gathered that the
prognosis of uveal tuberculosis is on the whole not good. The acute miliary
form is usually a prelude to a fatal general dissemination, and the ocular
prognosis of the specific granulomatous types is generally bad, although
many of the milder cases completely recover leaving scarred and atrophic
patches either in the iris or the choroid. Cases which progress beyond a
few months’ duration, however, especially when a tendency to become
conglomerate exists, may well result in destruction of the eye even in spite
of treatment. Many of the cases in this proliferative group, indeed, are
only slightly, if at all, amenable to treatment ; and the seriousness of the
outlook is greater in involvements of the anterior segment than the posterior
because of the danger of the development of a secondary glaucoma, and the
consequent necessity to enucleate the eye because of pain. The quieter
types of cyclitis, on the other hand, especially in younger people, are usually
amenable to treatment. In the exudative type of reaction the disease is less
dramatic in its course, but the strong tendency to chronicity and the liability
to interminable relapses should always make the prognosis guarded,
especially in older people. Recovery, it is true, is the rule, but the vision is
usually considerably impaired to a degree depending on the amount of
structural damage done before the inflammation is controlled. It is to be
remembered also, that the soft degenerated eyes resulting from long-
standing tuberculous inflammation tolerate operative interference badly :
as a general rule they should be left alone or enucleated.
Diagnosis. As we have seen, the diagnosis of uveal tubercle is frequently
so difficult as to present a quite insoluble problem ; indeed, in many cases
a tentative diagnosis is merely adopted after a process of exclusion and must
remain a surmise. The problem may be attacked from several angles.
(a) The clinical appearance of the eye in the granulomatous type of
lesion is sometimes sufficiently distinctive to allow a diagnosis to be made
without more ado. Miliary tubercles are pathognomonic. Tuberculomata
2312 TEXT-BOOK OF OPHTHALMOLOGY
of the anterior segment can be confused with few conditions except
syphilomata," lepromata,” ophthalmia nodosa º and neoplasms such as
retinoblastomata, which it is to be remembered in children, may grow
exuberantly on the surface of the iris or in the angle of the anterior chamber 4
(Jung, 1891; Behr, 1919; Meisner, 1921 ; Sijpkens, 1922; Pascheff, 1924);
while tuberculomata of the choroid bring up the differential diagnosis with
melanomata.” It is in the non-specific plastic and exudative types of
inflammation that the greatest difficulties arise. In these the features
which may most commonly suggest—but cannot prove—tuberculosis are
a clinical course characterized by an insidious onset, a slowly progressive
evolution, an intractability to treatment, and a tendency to relapses even when
healed for a considerable time—all this with an absence of severe pain and
marked subjective symptoms.
(b) Clinical evidence of tuberculosis elsewhere in the body is of the greatest
importance since uveal tubercle is a secondary disease. It is to be remem-
bered, however, that especially in the exudative types of inflammation,
active tuberculous lesions are the exception ; much more common is a healed,
Quiescent, scarred, pulmonary lesion, or an apparently quiet gland, usually
at the hilus. Such evidences are exceedingly common, but frequently may be
extremely difficult to find even with careful clinical and radiological
technique.
To give one example, a cat with ocular tubercle was examined pathologically by
Coats (1912) with completely negative results until a small tuberculous lesion of a
mammary gland was discovered.
(c) Skin Tests. A great deal has been written and much controversy
waged over the question of the reliability of the skin sensitivity to tuberculin.
It is to be remembered that these tests do not indicate active tuberculosis,
but only the presence of a sensitivity of the skin to tuberculo-protein which
follows an antecedent tuberculous infection somewhere in the body.
It is true that it has been shown experimentally that the cutaneous
sensitivity gives a fairly reliable index of the ocular sensitivity in systemi-
cally affected animals (Woods, Burky and Friedenwald, 1938), although
probably the ocular sensitivity is much higher than the dermal (Krasso,
1935), but it gives no direct indication of the activity or quiescence of any
ocular lesion. It must be remembered on the one hand that a tuberculous
infection at some time in life is practically universal, at any rate in urban
populations, almost 100% of whom have some degree of cutaneous sensi-
tivity to tubercle-protein (Hetherington and others, 1931–35, in America);
and on the other, that such a sensitivity quite frequently disappears when
the state of allergy is low, as in active or massive tuberculous infections,
during intercurrent infections, or in the moribund.
1 p. 2270. * p. 2321. 8 p. 2368.
* p. 2816. * p. 2514.
DISEASES OF THE UVEAL TRACT 2313
Of the four main diagnostic tests generally employed, the sub-cutaneous and
Calmette’s conjunctival tests should not be used in cases of uveal tuberculosis because
of the danger of a possible flare-up in the local lesion. The von Pirquet test, although of
value when used in graduated doses (Browning, 1929), is too delicate ; it is probably
of value in children under 5 years in whom a negative reaction may be taken to preclude
tuberculosis. The intra-cutaneous Mantouac test, when regarded as a qualitative
reaction in doses of 0.001, 0-01 and 0.1 mg. old tuberculin, is the most generally valuable:
the enormous literature on the subject has been summarized by Hart (1932). But
even so its value must not be exaggerated, and the view cannot be held with safety
that a strongly positive test is shown by patients with ocular tuberculosis and that a
weakly positive test is evidence against a tuberculous inflammation in the eye. Thus
Braune(1930) found the incidence of positive reactions only 20% higher in cases of
tuberculous compared with non-tuberculous eye disease. Hrankovicova (1932)
found only 75 strongly positive reactions in 214 cases of presumed ocular tuberculosis;
Biozzi (1933) 19 in 70 cases; and Woods (1938) in only 53.4% of cases, while 5%
required a dose as high as 0.1 mg. or were insensitive, a percentage not far removed from
that obtaining among normal city dwellers. Similarly, Friedenwald and Dessoff (1935)
found that in 10 cases of histologically proved ocular tubercle, only 6 reacted to 0-001
mg., 2 to 0-01, 1 to 0-1, and 1 to 1-0 mg., while in 36 cases of histologically proved
non-tuberculous cases 5 reacted to 0-001 mg. and the remainder varied from a
positive response to 0-01 mg. or less to insensitivity. Finally the question arises
as to the necessary specificity of the tuberculin reaction, for it has been shown that
identical cutaneous reactions can be obtained in tuberculous subjects by means of a
filtrate of B. coli or an extract of culture-free bouillon (Bordet, 1932; Lagrange, 1933).
It is not suggested that the determination of the skin sensitivity is
valueless, but it must be viewed in its proper perspective and in the know-
ledge that it is shown by many normal individuals. Negative reactions to
0.001 mg. or to weaker doses are without diagnostic value, but the pre-
sumption in favour of an ocular lesion being of a tuberculous nature in a
suspected patient is greatly strengthened by a high degree of skin sensitivity
to 0-001 mg. of tuberculin ; indeed, in correlation with the clinical picture
and with the exclusion of other aetiological factors, this forms the most
valuable criterion on which a diagnosis of tubercle can be made.
(d) The production of a focal reaction in the eye after the sub-cutaneous
injection of old tuberculin, in addition to a general febrile response and a
local reaction at the site of inoculation, has been said to establish positively
the diagnosis of tubercle, and for this reason is sometimes still applied in.
suspected uveal tuberculosis (Samoilov and Tihomirova, 1935; and others)
Such tuberculin reactions, however, may be extremely dangerous. In a
diffuse irido-cyclitis the iris becomes more hyperaemic, the pupil contracts,
circum-corneal injection is increased, the aqueous and vitreous become
more turbid, and secondary glaucoma may cause permanent damage. In
the nodular type, the size of the granulomata may be increased and they
become more vascular. A choroidal reaction may be even more hazardous :
the lesion becomes larger, vitreous opacities may increase, an outpouring
of exudate may lead to a retinal detachment and haemorrhages may lead
eventually to the formation of scar tissue. A diagnostic method which has
2314 TEXT-BOOK OF OPHTHALMOLOGY
led to the permanent loss of vision (Finnoff, 1925; Wilmer, 1928) is surely
unjustifiable. The more so is this the case since it cannot be considered
specific, for, on the one hand, a similar focal reaction can be obtained in
tuberculous lesions with such other proteins as milk (Löwenstein, 1923) or
casein (Tobias, 1922), and on the other, a prepared inflammatory focus
caused by a non-specific inoculum such as B. coli filtrate will be actuated
by an injection of tuberculin (de Witt, 1913–14).
(e) Serological methods of diagnosis are all quite unreliable. A comple-
ment fixation reaction against antigens derived from the tubercle bacillus
has led to results of no value (Carrère, 1923; Bachmann, 1924; Waltis,
1925; Hambresin and Bessemans, 1925; Arnould, 1931 ; Biozzi, 1933; and
others). Nor are changes in the blood picture or sedimentation rate of any
real diagnostic significance (Young, 1929; Fontana, 1933; Rossi, 1933;
Tertsch, 1935; and others).
(f) Histological evidence of tuberculosis is a guide of great value in
diagnosis, but it is only occasionally that the opportunity crops up, such as
when a piece of iris is removed for iridectomy, or one eye is excised when
the other shows a similar clinical picture. In many cases, moreover, as we
have seen, the histological picture is by no means distinctive and merely
indicates the presence of a chronic exudative inflammation.
(g) The recognition of the bacilli themselves is the only foolproof method
of diagnosis ; but the possibility of its attainment is rare. The opportunity
is generally limited to the case of eyes which have been excised for advanced
typical disease, and it is to be remembered that negative findings are of no
value in ruling out the diagnosis. In these cases histological recognition is
so much a matter of chance that the biological test is more satisfactory, the
material being inoculated sub-cutaneously or intra-peritoneally into a guinea-
pig which will develop generalized tuberculosis in from 6 to 12 weeks, or
sub-conjunctivally in a rabbit (Cosmettatos, 1930). We have already seen
they may be found in quantity in the discharge from a perforated eye ; but
it is only on the very rarest occasions that they can be obtained by
paracentesis of the aqueous (Stephenson, 1908).
Caramazza (1934) claimed results by injecting aqueous into the peri-bronchial
lymph glands of a guinea-pig, re-injecting these emulsified into a second, and so on,
obtaining finally changes he believed to be tuberculous.
The cultivation of tubercle bacilli from the blood-stream has been practised by
Löwenstein (1931), but in the hands of others the technique has so far proved unreliable
and negative (Tiedemann, 1931; Malek and Reimova, 1933; Takagi, 1934; Bencini,
1934; and others). Moreover, even if the bacilli are present in the blood-stream,
although providing presumptive evidence, this does not prove that they are
responsible for the ocular lesion, for in the type of chronic non-destructive inflamma-
tion in which the investigation is claimed to have positive results, the bacillaemia has
no constitutional symptoms and appears to affect no other organ ; the mere finding of
them does not prove that they affect the eye.
DISEASES OF THE UVEAL TRACT 2315
(h) Finally, the therapeutic response to specific treatment by tuberculin
may give a clue to the diagnosis ; but it is to be remembered that tuberculin
may act non-specifically, nor is the effect usually sufficiently dramatic to
allow conclusions of great certainty to be drawn.
Treatment. The appropriate local treatment should always be
administered in cases of uveal tuberculosis (mydriatics, heat, and so on),
but unfortunately, while allaying the inflammatory symptoms and tending
to prevent complications, this has little influence on the tuberculous
process. Since direct attack upon the bacilli has so far proved unsuccessful,
the most hopeful method of treatment is directed towards increasing the
immunity of the patient so that by his own endeavours he destroys the
bacillus and encapsulates the lesion, and at the same time towards decreasing
his allergic sensitivity so that destructive inflammatory effects are avoided
and the tissues are given the opportunity to heal themselves.
Since we know nothing whatever of the mechanism of immunity, the first
end can be achieved only by indirect means—a promotion of the general
powers of resistance by a freedom from strain, an adequate and vitamin-rich
diet, abundance of fresh air and sunshine, and—first and last—REST. Of
them all the most important is rest, without which a tuberculous lesion will
rarely get well, and with which alone it may be cured. Rest is as important
as in the case of pulmonary tuberculosis ; in ocular lesions it is often
forgotten, and quite frequently the condition only begins to improve when
it is insisted upon.
If a disciplined and care-free régime cannot be maintained, sanatorium treatment
becomes ideal ; but it would seem that high altitudes are not of the same value
as in pulmonary lesions. Sunlight is most important, and if it cannot be obtained
naturally it should be administered artificially (Koeppe, 1919; Lundsgaard,
l923; Duke-Elder, 1926–29 ; Goulden, 1926 ; Stein, 1926 ; Bentzen, 1927; and
others).
Local immunity can be increased by flooding the eye with a plasmoid
aqueous rich in immune bodies by a paracentesis, a procedure frequently of
dramatic value, especially in active tuberculous lesions of the anterior
segment ; and it is probable that it may be made more effective by injecting
the patient’s own blood into the anterior chamber (auto-haemotherapy :
Schieck, 1932). Such a procedure may be followed by a prompt subsidence
of the local inflammation (Baer, 1933; Krasnow, 1934; Miranda, 1934;
Serr, 1934; Kyrieleis, 1934; Denig, 1935 ; Mata, 1935; Rollet, 1935;
and others).
The attainment of a specific immunity has been suggested by inoculation with an
attenuated tubercle bacillus—B. Calmette-Guérin (BCG); but it has not yet received
general adoption ; and the increase in allergy which follows its administration probably
rules it out for uveal tuberculosis.
1 p. 2208.
2316 TEXT-BOOK OF OPHTHALMOLOGY
Desensitization is achieved by the use of one or other of the various
types of tuberculin,” which in uveal tuberculosis is of great value. The older
method of using tuberculin was to give fairly large doses in order to excite
repeated focal reactions in the hope of thereby producing a local immunity.
Very frequently this was disastrous, a circumstance which probably accounts
for many of the unfavourable reports of tuberculin therapy to be found
in the literature—of which there are many, varying in opinion from useless-
ness to dangerousness * (Igersheimer, 1920; Derby and Carvill, 1927;
Rollet and Colrat, 1927; and others). On the other hand there are more
authors who consider its use of great value (Clarke and Mayou, 1908;
Clarke and Wright, 1908; v. Hippel, 1914; Török, 1919; Weigelin, 1921 ;
Hartig, 1923; Wilmer, 1928; Woods and Rones, 1928; King, 1929;
Lawson, 1929; Woods and Randolph, 1937; and many others). It is
probable that the best results are obtained when tuberculin is regarded
purely as a means to achieve and maintain desensitization so as to allow the
forces of immunity to act unhindered by allergic influences. In this view
several important considerations follow. It is not a curative treatment but an
adjuvant to the essential general treatment which it cannot replace. It must be
given in minimal doses never eacceeding the patient’s reaction point, even
although the initial doses are of the order of 1/10,000,000 mg. We have seen
that the ocular sensitivity is greater than that of the skin ; a skin reaction
at the site of inoculation should therefore be regarded as an indication for
reduction of the dose. If an ocular lesion is actively progressive, suggesting
a high sensitivity and a poor immunological response, even greater care
should be taken, as otherwise tuberculin becomes dangerous (Werdenberg,
1935): in these cases dosage should be exceedingly small and the treatment
is well preceded by a paracentesis and auto-haemotherapy with a view to
increasing the local immunological defence and minimizing the danger
of a focal reaction (Woods, 1938). Finally, treatment should be continued until
full desensitization has been attained, preferably for a year after ocular
activity has ceased, and if sensitization recurs thereafter as indicated by
subsequent skin reactions, a further course should be inaugurated. Used
with these guiding principles it will be found that tuberculin forms a
therapeutic agent of great value in uveal tuberculosis.
Other methods of treatment have been advocated from time to time of which two
may be specially mentioned—gold intravenously (as gold sodium thiosulphate *) and
radiation. Various types of radiation are claimed to be of value, particularly in
the granulomatous types of disease. Ultra-violet light has been advocated (Koeppe,
1919; Nicolau, 1929), but results are by no means consistent (Duke-Elder, 1928;
Woods and Randolph, 1937). X-rays have received good reports (Scheerer, 1925;
Stock, 1926; Wetterstrand, 1926; Richter, 1927; Lloyd, 1930; Diacicov, Muntéanu
and Gavrilescu, 1930; Odnasheva and Justchenko, 1931 ; Werdenberg, 1935; and
others), as also has radium (Kumer and Sallmann, 1929), but again the effects have so
1 Vol. II, p. 1479. * Vol. II, p. 1479. p. 2214
DISEASES OF THE UWEAL TRACT
2:317
FIG. 1937.
Fig. 1938.
FIGs. 1937–38.-PERIPHERAL TUBERCULouis ChoroidITIs TREATED BY DIATHERMy.
Fig. 1937 shows the case before treatment; Fig. 1938 in the cicatricial stage
after diathermic coagulation (Weve, T. O. S.).
far been inconsistent, although Woods (1939) reported good results with the beta rays
of radium.
Surgical treatment has been suggested in the case of solitary tubercle of the choroid:
thus Petrovic (1926) after a Krönlein surgical approach excavated a caseous tumour
with a sharp spoon with good results; while Weve (1935–39) suggested coagulation by
diathermy (Figs. 1937–38).
Arnould, An. d’Oc., clºviii, 624, 1931.
Autenrieth. Versuche f. d. prakt. Heilkunde,
Tübingen, i, 309, 1807.
Axenfeld. Med. kl., i, 375, 1905.
K. M. Aug., lxxxv, 465, 1930.
Bach. A. f. Aug., xxviii, 36, 1893.
Bachmann. A. f. Hygiene, xciv, 228, 1924.
Baer. K. M. Aug., xe, 485, 1933.
Baum. Diss., Freiburg, 1902.
Behr. K. M. Aug., lxiii, 277, 1919.
Bencini. Boll. d’Oc., xiii, 1309, 1934.
de Benedetti. Boll. spec. Med. Chir. Pavia, iv.
374, 1930.
Bentzen. Acta O., v, 29, 1927.
Bergmeister. Die tub. Erkrankungen d.
Auges, Berlin, 1927.
Berthold. An. d’Oc., lxvi, 85, 1871.
Biozzi. Rass. It. d’Ott., ii, 1355, 1933.
Black and Haessler. Am. J. O., xiii, 139,
1930.
Block. K. M. Aug., lxvii, 581, 1921.
Bock. A. f. path. Anat., xei, 434, 1883.


23 18
TEXT-BOOK OF OPHTHALMOLOGY
Bonnet and Colrat. A. d’O., lii, 385, 1935.
Bordet. Internat. Tuberculosis Cong., Hague,
1932.
Botteri. A. f. O., lxix., 1, 1908.
K. M. Aug., xlvii (1), 490, 1909.
Bouchut. Gaz. d. Hôp. de Paris, xxxix, 225,
1866; xlviii, 338, 347, 1875.
Braun. A. f. O., czziv, 182, 1930.
Brown. A. f. O., lxxxii, 300, 1912.
Am. J. O., xix, 668, 1936.
Browning. T. O. S., xlix, 64, 1929.
Brüsselmanns. A. d’O., xliii, 37, 1926.
Bulson. T. Am. O. S., xxiii, 292, 1925.
Busacca. K. M. Aug., lxxxviii, 14, 1932.
Busch. A. f. path. Amat., xxxvi, 448, 1866.
Butler. Brit. Med. J., i, 804, 1911.
T. O. S., l, 608, 1930. -
Caramazza. Ras. It. di Ott., iii, 871, 1934.
Cargill and Mayou. T. O. S., xxvi, 101, 1906.
Carpenter and Stephenson. P. R. S. Med.,
Dis. Children, i, 169, 1901.
Carrère. Rev. gen. d’O., xxxvii, 476, 1923.
Clarke and Mayou. T. O. S., xxviii, 190, 1908.
Clarke and Wright. T. O. S., xxviii, 192,
1908.
Coats. T. O. S., xxxii, 126, 1912.
Cohnheim. A. f. path. Amat., xxxix, 49, 1867.
Berlin. kl. W., iv, 63, 324, 1867.
Cosmettatos. Rev. gen. d’O., xliv, 57, 1930.
Cummins. Brit. J. O., viii, 112, 1924.
Daels. A. f. path. Amat., cze, Beiheft, 90,
1907.
Delarne. Cours complet d. maladies des yewa,
Paris, p. 206, 1820.
Demaria. K. M. Aug., xliii, Beil., 90, 1905.
Demours. Traité d. Maladies des Yewa,
Paris, 1818.
Denig. A. f. Aug., xxxi, 359, 1895.
A. of O., xiv, 860, 1935.
Derby. A. of O., lvii, 561, 1928.
Derby and Carvill. T. Am. O. S., xxv, 104,
1927.
Derkac. Z. f. Aug., liv, 212, 1924.
Diacicov, Muntéanu and Gavrilescu.
Med., xi, 496, 1930.
Dinkler. A. f. O., xxxv. (4), 309, 1889.
Duke-Elder. Brit. Med. J., i, 891, 1926.
T. O. S., xlvi, 213, 1926; xlviii, 262, 1928;
xlix, 72, 1929.
Brit. J. O., xi, 67, 1927;
1928; xiii, l, 1929.
Edmunds and Brailey. T. O. S., ii, 269, 1882.
Elschnig. Wien. kl. W., xxv, 713, 1912.
R. M. Aug., lxxiv, 783, 1925.
Eltore. An. di Ott., lxii, 779, 1934.
Finnoff. T. Am. O. S., xx, 291, 1922; xxi,
87, 1923.
A. of O., liii, 130, 1924.
Am. J. O., vii, 365, 1924; viii, 241, 1925;
xiv, 127, 1208, 1931.
Fontana. Boll. d’Oc., xii, 598, 1933.
Fraenkel. Jhrb. f. Kinderheilk., ii, 1870.
Fralick. A. of O., vi, 421, 1931; ix, 13, 1933.
Friedenwald. A. of O., ix, 165, 1933.
Friedenwald and Dessoff. Bull.
Hopkins H., lvii, 148, 1935.
Cluj.
xii, 289, 353,
Johns
Friedrich and Nosske.
xvi., 470, 1899.
Fuchs. Lehrb. d. Aug., Leipzig, 1891.
Galezowski. A. génér., x, 258, 1867.
Gifford. Am. J. O., xiv., 100, 1931.
Gilbert. Münch, med. W., lxi, 306, 1914.
A. f. Awg., lxxxii, 179, 1917; lxxxiv, 153,
1919.
B. O. G. Heidel., xlvi, 86, 116, 1927.
Kurzes Hb. de O., Berlin, v, 2, 1929.
Ginsberg. A. f. O., lxxiii, 538, 1910.
Glover. Am. J. O., xiii, 411, 1930.
Goldenburg and Fabricant. T. Sect. O. Amer
Med. As., 135, 1930.
Goulden. R. L. O. H. Rep., xix, 328, 1914.
T. O. S., xlvi, 207, 1926.
Gradenigo. Am. d’Oc., lxiv, 174, 1870.
v. Graefe. A. f. O., ii (1), 210, 1855.
Graefe, E., and Leber. A. f. O., xiv. (1), 183,
1868.
Griffith. T. O. S., x, 84, 1890.
Grönholm. Acta O., vi, 297, 1928.
Guillery. D. med. W., liii, 740, 1927
Haab. A. f. O., xxv (4), 163, 1879.
R. M. Aug., xxii, 391, 1884.
Haas. Diss., Würzburg, 1898.
Haensell. A. f. O., xxv (4), 1, 1879.
Hambresin and Bessemans. C. R. S. Biol.,
xcii, 219, 1925.
Hart. Med. Res. Council Rep., London,
clxiv, 1932.
Hartig. Z. f. Aug., l, 79, 1923.
Heine. Münch. med. W., lx, 1305, 2441,
1913; lxi, 2092, 1914.
Hess. Schweiz. A. f. Tierheilk., xxx, 175,
1891.
Hetherington et alia. A. Int. Med., xlviii,
734, 1931; lv, 709, 1935.
v. Hippel. A. f. O., lix, 1, 1904;
193, 1914; xcii, 421, 1917;
1918; evii, 606, 1926.
Hirschberg. A. f. path. Amat., xc, 1, 1882.
Horner. Die Krankheiten, d. Auges im Kinde-
salter, 1883.
Hosch. A. d’O., ii, 508, 1882.
Hrankovicova. Oft. Sbornik, vii, 81, 1932.
Igersheimer. B. O. G. Heidel., xlii, 235, 1920.
XIV Internat. Cong. Madrid, i, 1, 1933.
A. of O., xi, 119, 1934.
Irons. Am. J. O., xiv, 1228, 1931.
Irons and Brown. J. Am. Med. As., lxvi,
1840, 1916; lxxxi, 1770, 1923; lxxxvii,
1167, 1926.
Jackson. Contrib. to Med. and Biological
Research, i, 616, 1919.
Jacqueau and Bujadoux.
cxxxiv, 96, 1924.
Jaeger. Ueber Markschwamm d. Auges w.
Amaurotisches Katzenauge, Würzburg,
p. 78, 1831.
Z. f. pr. Heilk., ii, 9, 1855.
Jung. A. f. O., xxxvii (4), 125, 1891.
K. M. Aug., lxxi, 70, 1923.
Kägi. Z. f. Avg., lii, 155, 1924.
Katznelson. Sovet. vest. O., ix, 205, 1936.
Rellermann. Z. f. Avg., xx, 464, 1908.
Ziegler's Beiträge,
lxxxvii,
xcv, 255,
S. d’O. Lyons,
DISEASES OF THE UVEAL TRACT
23 19
King. A. of O., i, 713, 1929.
Roch. Berlin. kl. W., xix, 221, 1882.
Dewt. med. W., xvi, 5, 1890; xvii, 1189,
1891.
Roeppe. A. f. O., xcii, 115, 1917.
Münch. med. W., lxvi, 743, 1919.
Royanagi. K. M. Aug., xciii, 37, 1934.
Krasnow. Sov. vestm. Oft., v, 222, 1934.
Krasso. Z. f. Aug., lxxxv, 271, 1935.
FCrückmann. B. O. G. Heidel., xlvii, 64, 1928.
Z. f. Aug., lxvii, 176, 1929.
Kumer and Sallmann. Die Radiumbehandlung
$n d. Augenheilkunde, Wien, 1929.
Ryrieleis. Z. f. Aug., lxxxv, 16, 1934.
Lagrange, F. A. d’O., xv, 170, 1895.
Bull. S. fr. d’O., xvi, 88, 1898.
Tumeurs de l’oeil, Paris, i, 1901.
Lagrange, H. La Tuberculose du tractus uveal,
Paris, 1924.
A. d’O., xli., 80, 1924.
An. d’Oc., clxx, 561, 1933.
Brit. J. O., xvii, 679, 1933.
Lang. Lancet, i, 1368, 1913.
Lawford and Neame. Brit. J. O., vii, 305,
419, 1923.
Lawrence. Treatise on Diseases of the Eye,
London, p. 593, 1830.
Lawson. T. O. S., xlix, 45, 1929.
Leber. B. O. G. Heidel., xxi, 44, 1891.
Leoz. A. de Oft., H.-A., xxxv, 230, 1935.
Liebrecht. A. f. O., xxxvi (4), 224, 1890.
Lincke. De fungo medullari oculi, Leipzig,
i, 52, 1833.
Litten. Sammlung klin. Vorträge v. Volkmann,
cxix, Inn. Med., No. 43, 1061, 1877.
Lloyd. Am. J. O., xiii, 753, 1930.
Long. A. of O., xvii, 191, 1937.
Lothermoser. Z. f. Veterinóirkwnde,
1905.
Löwenstein. Zb. f. d. ges. O., viii, 209, 1923.
Münch. med. W., lxxviii, 261, 1931.
Lundsgaard. Acta O., i, 39, 1923.
Lüttge. A. f. O., Iv, 53, 1903.
Machek. Wien. med. W., xliv, 1065, 1121, 1894.
Mackenzie. Practical Treatise on Diseases of
the Eye, 1854. -
Maitre-Jan. Traité d. maladies des yeux,
Troyes, p. 456, 1707.
Malek and Reimova. Ceskoslovenska Oft., i,
184, 1933.
Manfredi. Am... di Ott., iv, 265, 1875.
109,
Manz. A. f. O., iv (2), 120, 1858; ix (2), 133,
1863.
Margulies. Beit. z. Kenntniss d. Miliar-
tuberculose d. Choroidea, Königsberg,
1898.
Z. f. kl. Med., xlviii, 238, 1903.
Mata. A. de Oft., H.-A., xxxv, 270, 1935.
Meisner. K. M. Aug., lxvii, 388, 1921.
Meller. Z. f. Aug., xxx, 246, 379, 1913;
lxxix, 95, 110, 1932; lxxxix, i, 1936.
A. f. O., cv, 299, 1921.
Wien. kl. W., xlv, 33, 1932.
T. O. S., liv, 467, 1934.
v. Michel. A. f. O., xxvii (2), 232, 1881.
Lehrb. d. Aug., Wiesbaden, 1890.
T.O. —WOL. III.
Z. f. Aug., ii, l, 1908.
Münch. med. W., xlvii, 853, 1900.
Miranda. A. de Oft. H.-A., xxxiv, 349, 1934.
Moncet. Rev. Vétérinaire, 329, 1895.
Montalti. An... dº Ott., lv, 555, 1927.
Moore, W. Am. J. O., xiv, 596, 1931.
Morton. Brit. Med. J., ii, 169, 1891.
Natanson. K. M. Aug., xlviii (1), 113, 1910.
Neame. Brit. J. O., vi, 204, 1922.
zur Nedden. K. M. Aug., xli (2), 351, 1903.
Newton. Teacas State Med. J., xxi, 315, 1925.
Nicolau. S. Român di Oft. Bueuresti, xi, 33,
1929.
Odnasheva and Justchenko.
xiii, 484, 1931.
Ogilvy. T. O. S., xxix, 8, 1909.
Ohmart. Am. J. O., xvi, 773, 1937.
O’Rourke. Am. J. O., viii, 715, 1925.
Parinaud. Gaz. d’Opht., i, 118, 1879.
Pascheff. K. M. Aug., lxxiii, 126, 1924.
Péchin. A. d’O., xix, 696, 1899.
Penman and Wolff. Proc. R. S. Med., xxvi,
315, 1932.
Perls. A. f. O., xix (1), 221, 1873.
Petroff and Stewart. J. Immunol., x, 677,
1925.
Petrovic. K. M. Aug., lxxvii, 791, 1926.
Poncet. Gaz. med. de Paris, iv, 81, 1875.
Progrès méd., i, 467, 1882.
Rec. d’O., x, 577, 1888. -
Popper. Wien. kl. W., xliv, 1494, 1931.
Rich and McCordock. Bull. Johns Hopkins
Hosp., xliv, 273, 1929.
Richter. K. M. Aug., lxxviii, 91, 1927.
Riehm. A. f. Aug., cv, 82, 1931.
Ritterich. Järliche Beit. z. Vervollkommung
d. Aug., Leipzig, i, 37, 1827.
Rohrschneider. Z. f. Aug., lxxxi, 197, 1933.
Rollet. A. of O., xiv, 860, 1935.
Rollet and Aurand. Rev. gén. d’O., xxvii, l,
1908.
Rollet and Colrat.
Paris, 1927.
Rossi. A. di Ott., xxxix, 439, 1933.
Rüter. A. f. Aug., x, 147, 1881.
Saltini. An. di Ott., iv., 127, 1875.
Samoilov. Russ. O. J., xii, 145, 1930; xiii,
471, 1931.
Samoilov and Tihomirova. Am. d’Oc., clxxii,
993, 1935.
Samuelsohn. B. O. G. Heidel., xxiii, 75, 1893.
Saunders. Treatise on Some practical points
relating to Diseases of the Eye, London,
p. 119, 1811.
Scheerer. K. M. Aug., lxxv, 27, 1925.
Schieck. A. f. O., l, 247, 1900; cw, 257, 1921.
D. med. W., 729, 1911.
B. O. G. Heidel.., xxxvii, 201, 191 l ; xlix,
183, 1932.
K. M. Aug., lxv, 414, 1920.
Z. f. Aug., xliii, 625, 1920.
Schmidt. A. f. Tierheilk., xxv, 206, 1899.
Schultze. A. f. Aug., xxxiii, 145, 1896.
Schultz-Zehden. Z. f. Awg., xiv, 213, 1905.
Serr. B. O. G. Heidel., 1, 41, 1934.
Siegrist. K. M. Aug., xciii, 289, 1934.
Rum. O. J.,
Tuberculose de l’Oeil,
Q
2320
TEXT-BOOK OF OPHTHALMOLOGY
Sijpkens. K. M. Aug., lxix., 27, 1922.
Spörer. W. f. Tierheilk., 487, 1905.
Stein. K. M. Aug., lxxvii, 203, 1926.
Stephenson. T. O. S., xxviii, 35, 1908.
Stock. K. M. Aug., xli., Beil., 17, 1903;
lxx, 356, 1923; lxxvi, 542, 1926.
B. O. G. Heidel.., xxxii, 297, 1905.
A. f. O., lxvi, l, 1907.
Straub. T. O. S., xxxii, 60, 1912.
Sudzuki. Acta O. S. Japan, xxxv, 111, 1931.
Suker and Cushman. Am. J. O., xiii, 781,
1930.
Swanzy. T. O. S., ii, 259, 1882.
v. Szily. K. M. Aug., lxxxvii, 13, 1931.
Takagi. Acta S. O. Japan, xxxviii, 1444, 1934.
Terson. A. d’O., x, 7, 1890.
An. d’Oc., czzxi, 119, 1904.
Tertsch. Z. f. Aug., lxxxv, 314, 1935.
Tiedemann. Zb. Bakt., czzii, 483, 1931.
Tobias. Kl. W., i, 515, 1922.
Tooke. Brit. J. O., xx, 23, 1936.
Török. A. of O., xlviii, 242, 1919.
Urbanek. T. O. S., lii, 227, 1932.
Valtis. Am. d’Inst. Pasteur, xxxix, 365, 1925.
Vejdovsky. Ceskoslovenska Oft., i, 28, 1933.
Verhoeff. T. Am. O. S., xii, 366, 1910.
J. Am. Med. As., lxiii, 13, 1914 ; xcv, 583,
1930.
A. of O., i, 63, 1929.
Vogt. A. f. O., czi, 91, 1923.
Wagenmann. A. f. O., xxxiv. (4), 145, 1888.
Waldenburg. Tuberculose, Berlin, 1869.
de Weeker. G. S. Hb., I, iv, 504, 1876.
Wedl and Bock. Path. Anat. d. Auges, Wien
1886.
Weigelin. A. f. O., lxxv, 411, 1910.
R. M. Awg., lxvi, 641, 1921.
Weiss. A. f. O., xxiii (4), 57, 1877.
Wells. T. Path. S. London, xix, 359, 1868.
Werdenberg. Schw. med. W., lix, 126, 1929.
R. M. Aug., lxxv, 545, 1925; c, 477; ci,
641, 1938.
A. of O., xiii, 303, 1935.
Beit. 2. Kl. d. Tub., lxxxix, 705, 1937.
Wetterstrand. Acta Radiol., vii, 639, 1926.
Weve. Ned. tij. v. Gen., lxxix, 754, 1935.
T. O. S., lix (1), 43, 1939.
Whitehead. Brit. J. O., vi, 529, 1922.
de Witt. J. Inf. Dis., xii, 58, 1913; xiii, 378 ;
xiv, 498, 1914.
Wilmer. A. of O., lvii, 1, 1928.
Witkina and Maklakova. Beil. z. Kl. d. Tub.,
lxiii, 119, 1926.
Woods. Am. J. O., xxi, 359, 1938.
A. of O., xxii, 735, 1939.
Woods, Burky and Friedenwald.
xix, 229, 236, 245, 1938.
Woods and McCormick. Wisconsin Med. J.,
xxxvi, 268, 1937.
Woods and Randolph. A. of O., viii, 510,
1937.
Woods and Rones.
I928.
Young. T. O. S., xlix, 58, 1929.
A. of O.,
South Med. J., xxi, 613,
5. LEPROSY
There is a considerable difference of opinion as to incidence of leprotic
infections of the uveal tract, so much so, indeed, that one is driven to the
conclusion that variations probably exist in different localities. Most
authors, however, agree that irido-cyclitis is common and choroiditis
rarer ; according to Lopez (1890) the uveal tract is affected in half the cases
of leprosy, Borthen and Lie (1899) estimate its implication in 37% of the
anaesthetic type and 74% of the tuberous, and, indeed, ciliary infections are
the commonest cause of blindness in this disease.
It is well known that the lepra bacilli are present usually in enormous numbers
in leprotic lesions ; the iris and ciliary body form no exception to this rule (Hansen,
1880; Neisser, 1879), where indeed they may be found without giving rise to any ocular
symptoms or any histological reaction (Fuchs, 1937). They may also be found in the
region of the ora serrata, but their discovery in the choroid is rarer (Wintersteiner
1895; Doutrelepont and Wolters, 1896; Franke and Delbanco, 1900; Pendergast, 1940).
In the anterior segment of the uveal tract three manifestations of leprosy
may appear.
1. MILIARY LEPROTIC SPOTS IN THE IRIs associated with an insidious
and chronic iritis are probably the commonest occurrence and form a
characteristic and pathognomonic picture, which is resembled by no other
DISEASES OF THE UVEAL TRACT 2321
ocular condition (Fig. 1939). The eye is relatively quiet, but keratic
precipitates demonstrate the presence of cyclitis. Dusted irregularly over
the iris between the collarette and the ciliary margin are numerous small
white dots, held by some to be exudative in nature (Kirwan, 1929) and by
most to be small foci of infection (Yudkin, 1918; Morax, 1924; Bhaduri,
1926; Tiscornia, 1927; King, 1936; and others). Exudative nodules
containing leucocytes and bacilli do occur (Peter, 1924), but the more
usual appearance is of small lepromata occurring in the substance and on
the surface of the iris, which may leave areas of local atrophy behind
(Valettas, 1916). While no other changes may
exist, other signs of chronic irido-cyclitis may
appear—posterior synechiae going on to occlusion
of the pupil, atrophy of the stroma and depig-
mentation leading to a condition of heterochromic
cyclitis, a secondary cataract, and generalized
uveal involvement eventually resulting in a soft
and degenerated eye.
2. Isolated LEPROMATA are rarer, and indeed
their occurrence has been denied by some ob-
servers. They spring from a general infiltration
of the ciliary body and appear at the angle of
the anterior chamber usually associated with
an interstitial keratitis. Their gradual progress
to involve the ciliary body, iris, choroid and
sclera leads to the eventual destruction of the
eye.
Fig. 1939.-NoDULAR LEPRotic
IRITIs (King).
Such lesions have been produced experimentally
by the injection of leprous material or the bacilli into
the anterior chamber of animals (Damsch, 1883; Vossius, 1884; Chorine, Guilliny
and Montestruc, 1934).
3. An acute DIFFUSE PLASTIC IRIDo-CYCLITIS is not very common, and
it differs in no way from a non-specific infection. It is accompanied by
considerable pain and ciliary injection, a hyperaemic and muddy iris, and is
usually exudative in type associated with extensive synechiae and vitreous
opacities. It would seem difficult, however, to differentiate this from an
incidental infective inflammation.
More difference of opinion exists as to leprotic manifestations in the
posterior segment. Some observers consider that the choroid is immune
apart from the spread of disease directly from the ciliary body (Franke and
Delbanco, 1900; Neve, 1900); this, however, may be extreme in degree
involving an infiltration of the entire globe and the production of a pseudo-
glioma. More systematic investigations, however, show that such is not

Q 2
2322 TEXT-BOOK OF OPHTHALMOLOGY
the case: Trantas (1899) observed punctate lesions similar to those seen in
the iris together with pigmentary proliferation in 68% of his cases, and
Rubert (1904), in a systematic search, discovered fundus changes 47 times
among 202 lepers both of the tuberous and anaesthetic type, but particularly
in the former.
The lesions may be unilateral or bilateral, and appear as white, isolated,
scar-like areas with some pigmentary proliferation. They may be few or
numerous, varying in size from a pin's head to a disc-diameter; usually they
are in the peripheral fundus, so near the ora that their ophthalmoscopic
Fig. 1940,-LEPRotic ChorolorTIs (Hoffmann).
observation is difficult, and they are rarely found behind the equator, so that
a typical picture of disseminated choroiditis may be produced (Penichet,
1929). Hoffmann (1928–30), indeed, considers that a disseminated choroiditis
of this type (chokomorris LEPRosa PRAEcox), indistinguishable from that
usually called tuberculous and tending to attack the posterior region of the
fundus as well as the periphery, frequently occurs in latent leprosy, that is,
in an infected individual who exhibits no clinical symptoms over a long
period (Fig. 1940). The course of the choroiditis is benign, vitreous opacities
are few, and vision is unimpeded.
Pathologically leprous lesions of the uveal tract correspond with those seen else-
where in the body—a granulomatous infiltration of mononuclear cells, often packed
with bacilli, without focal arrangement as in tubercle, but with a tendency to aggregate
into large multi-nucleated giant lepra-cells (Hansen and Bull, 1873; Poncet, 1888;

DISEASES OF THE UVEAL TRACT 2323
Philippson, 1893; Wintersteiner, 1895; Doutrelepont and Wolters, 1896; Babes,
1898; Jeanselme and Morax, 1898 ; Borthen and Lie, 1899 ; Franke and Delbanco,
1900; Pendergast, 1940; and others).
The treatment of leprosy, in addition to the usual local measures, depends
On the administration of chaulmoogra oil, generally as the ethyl ester.”
The initial injections should be small owing to the danger of severe focal
reactions in the eye. Schnaudigel (1923) and Hoffmann (1928) report good
results with gold therapy (Krysolgan). It is noteworthy that if complications,
such as iris bombé or cataract, develop, a leprotic eye tolerates surgical
interference well (Pinkerton, 1927–37), although the iris tissue is frequently
so rotten that iridectomy may fail (Wood, 1925). In no case of leprosy,
however, is the ultimate prognosis good ; the utmost that can be done is to
postpone a final tragedy.
Babes. Untersuchungen ü. d. Leprabacillus w. Kirwan. Trans. VII Cong. Far East Trop.
d. Histol. d. Lepra, 1898.
|Bhaduri. Indian Med. Rec., xlvi, 269, 1926.
Borthen and Lie. Die Lepra des Auges,
Leipzig, 1899.
Chorine, Guilliny and Montestruc.
Biol., crvi, 1261, 1934.
Damsch. A. f. path. Amat., xcii, 20, 1883.
Doutrelepont and Wolters. A. f. Derm. Syph.,
xxxiv, 55, 1896.
Franke and Delbanco. A. f. O., l, 380, 1900.
Fuchs. K. M. Aug., xcviii, 728, 1937.
Plansen. A. f. path. Amat., lxxix, 32, 1880;
xc, 542, 1882.
Hansen and Bull.
London, 1873.
Hoffmann. Z. f. Immun. Forsch., lix, 297,
1928.
Dermat. W., lxxxvi, 394, 1928.
J. Tropical Med. Hyg., xxxii, 328, 1929.
J. dos clinicos. Rio de Jan., xi, 225, 1930.
Jeanselme and Morax. An. d’Oc., czz, 312,
1898.
King. Brit. J. O., xx, 561, 1936.
C'. R. S.
Leprous Dis. of the Eye,
Med., i, 289, 1929.
Lopez. A. f. Aug., xxii, 318, 1890.
Morax. A m. d’Oc., Czli, 811, 1924.
Neisser. Breslauer àrztl. Z., i, 200, 214, 1879.
Neve. Brit. Med. J., i, 1153, 1900.
Pendergast. A. of O., xxiii, 112, 1940.
Penichet. Rev. Cub. de Oft., i, 320, 1929.
Peter. A. of O., liii, 258, 1924.
Pinkerton. A. of O., xliii, 42, 1927.
Laryngoscope, xliii, 991, 1933.
Am. J. O., xx, 715, 1937.
Philippson. Beit. z. Aug., xi, 31, 1893.
Poncet. Prog. méd., vii, 33, 1888.
Rubert. Mitt. Augenklinik v. Fejer, ii, 1904.
Schnaudigel. Münch. med. W., lxx, 1047, 1923.
Tiscornia. A. de Oft., B. A., ii, 321, 1927.
Trantas. Bull. S. fr. d’O., xvii, 275, 1899.
A. d’O., xxxii, 193, 1912.
Valettas. K. M. Aug., lvi, 472, 1916.
Vossius. B. O. G., Heidel.., xvi, 27, 1884.
Wintersteiner. Wien. kl. W., viii, 350, 1895.
Wood. Brit. J. O., ix, 1, 1925.
Yudkin. Am. J. O., i, 303, 1918.
6. TRYPANOSOMIASIS
We have already seen * that trypanosomiasis is not uncommonly complicated by a
keratitis with which is associated an irido-cyclitis. Sometimes, however, an irido-
cyclitis which has certain definite characteristics occurs alone, it attacks one eye only,
is of short duration, is benign in type, and tends to recur. It is possible that
many of these cases are toxic in nature (Daniels, 1918), but when actual invasion
of the eye by the organism occurs, the inflammation may be associated with small
haemorrhages on the iris or a hyphaema (Bargy, 1928). Such symptoms can be brought
on experimentally in animals (Woods and de Schweinitz, 1917); and a choroiditis has
been reported (Collins, 1915). The ocular symptoms coincide with a general dissemina-
tion of the organism and are therefore associated with constitutional symptoms of
fever, headaches, poly-adenitis, papular eruptions and Oedematous patches on the skin,
1 Vol. II, p. 1480. * Vol. II, p. 1980.
2324 TEXT-BOOK OF OPHTHALMOLOGY
tachycardia, tremulousness of the tongue and extremities, delayed pain after deep
muscular pressure, and general asthenia. The diagnosis is made by the recognition
of the parasite in the blood, while sometimes it may be recovered from the anterior
chamber (Neame, 1927; Bargy, 1928); treatment is effective by atoxyl, soamin or
germanin.
Bargy. Bull. S. fr. d’O., xli., 43, 1928. Neame. Brit. J. O., xi, 209, 1927.
Collins. Proc. R. S. Med., ix, 5, 1915. Woods and de Schweinitz. T. Am. O. S., xv,
Daniels. Proc. R. S. Med., ix, 2, 1915. 106, 1917.
Brit. J. O., ii, 83, 1918.
C. Syndromes of Doubtful AEtiology
1. SYMPATHETIC OPHTHALMITIS
SYMPATHETIC OPHTHALMITIS is a specific bilateral inflammation of the
entire weal tract of unknown aetiology, characterized clinically by an insidious
onset, a progressive course with exacerbations and usually a disastrous
termination, and pathologically by a modular or diffuse infiltration of the uveal
tract with lymphocytes and epithelioid cells, which almost invariably follows
a perforating wound involving uveal tissue. The disease appears in the
injured eye (or ExCITING EYE)' at a variable time after injury and
synchronously or shortly afterwards affects the other (or syMPATHIZING)
eye ; but the clinical and pathological picture presented by the two eyes is
identical.
SYMPATHETIC IRRITATION
Sympathetic ophthalmitis is to be sharply differentiated from sympathetic irritation
—a term traditionally used to designate certain disturbances, presumably of a reflex
nature, seen in the sound eye in association with injury or disease of its fellow. The
exciting eye is always painful, irritable and inflamed, and it is frequently shrunken.
In the fellow eye there is a complete absence of signs of intra-ocular inflammation—
immobility of the iris, keratic precipitates, cells in the aqueous, and so on—but a large
number of somewhat indefinite symptoms may appear which have received a con-
siderable amount of attention in the older literature, all of which usually show marked
variations corresponding to exacerbations and remissions in the condition of the diseased
eye.
Objectively there is usually some peri-corneal injection, lacrimation is a constant
symptom, and sometimes blepharospasm. Subjectively photophobia is a prominent
feature, brought on by stimuli which seem ridiculously small ; the eye is painful and a
radiating trigeminal neuralgia may occur. Visual symptoms are frequent, the most
common being a lowering of the visual acuity (sympathetic amblyopia): but the visual
disturbances are varied and alter with the condition of the exciting eye ; central vision
may deteriorate considerably, a concentric contraction may appear in the fields, or the
whole field of vision may periodically cloud over. Rapid onset of visual fatigue is
conspicuous, and paresis of accommodation common.
* The Continental terminology is somewhat confusing when compared with the English
and should be noted : the exciting eye is called syMPATHISANT, SYMPATHISIERENDE, sym-
PATIzzANTE ; the sympathising eye, SYMPATHISE, syMPATHISIERTE, SIMPATIzzATO. Gifford
(1920) suggested the term syMPATHOGENIC for the exciting eye.
DISEASES OF THE UVEAL TRACT 2325
The cause of these indefinite irritative symptoms inspired much speculation in
the early literature, and it was generally concluded that they were due to an irritation
of the ciliary nerves (Schirmer, 1900); because of this an optico-ciliary neurotomy was
advocated. It is to be remembered, however, that neither this nor even excision of
the exciting eye is a certain cure of the condition, for it has occurred in association with
a badly fitting artificial eye, painful scars in the socket, or after Mules’ operation.
Indeed, all the symptoms can be produced by the presence of a foreign body in one eye.
It seems much more probable that the whole symptom-complex is merely a reflea.
effect of no particular significance which will disappear only when the stimulus is
removed. Most of the symptoms are explicable by reflex trigeminal and vaso-motor
irritation, and the “reflex ‘’ amblyopia or amaurosis is almost certainly of hysterical,
Or at any rate psychogenic, origin. There are certainly no grounds for the belief that it
forms a forerunner of sympathetic ophthalmitis. Fuchs (1905), for example, sectioned
16 eyes, causing sympathetic irritation and found none of the characteristic signs of
Sympathetic inflammation. At the same time any appearance of irritation, especially
after a perforating injury, should stimulate the most careful clinical examination and
thorough search for signs of real danger in the exciting eye—sometimes a matter calling
for the greatest clinical experience and judgment—lest signs interpreted as irritative
may be really the prelude to a true inflammation.
Historical. The conception of sympathetic inflammation is one of very consider-
able antiquity ; probably the first reference in literature may be considered to be the
note from Agathias in the anthology compiled from Constantius Cephalis (A.D. 1000):
“ the right eye when diseased often gives its suffering to the left.” In the oldest
German text-book of ophthalmology, Bartisch (1583) remarked that when one eye is
injured “the other good eye is besides also in great danger,” a calamity noted by Le
Dran (1696) in his reflections on war wounds, and also understood by Duddell (1729),
who recorded the fact that in many cases both eyes were lost although only one was
originally injured. Nearly a hundred years elapsed before a clearer conception was
introduced by Demours (1818) and Wardrop (1819), both of whom used the term
“sympathetic,” but the history of the disease really commences with the masterly
and comprehensive clinical description of Mackenzie (1835) who called the disease
sympathetic ophthalmia. Wardrop (1819) had already drawn attention to the fact that
veterinary surgeons had been in the habit of destroying the injured eye of a horse with
lime or a nail in order to save the good one, but Prichard (1851) was the first to practise
excision as a therapeutic measure. Twelve years later at the first Heidelberg Congress,
Critchett (1863) showed that excision was ineffective once the inflammation had broken
Out, and thus regularized prophylactic excision as it is practised to-day. The next
incidents of importance in the history of our knowledge of the disease were the critical
survey and experiments of Schirmer (1900) and the classical histological studies of
Fuchs (1905) who demonstrated that it was a separate disease-entity distinct from any
other affecting the eye ; since their time the literature has been full of attempts to
determine the aetiology of this condition—a problem so baffling that our ideas upon it
to-day are no more ordered than they were then.
Incidence. The general incidence of sympathetic ophthalmitis is
difficult to assess, since the cases seen by single observers are few, and
pathological proof of the condition is usually lacking so that the diagnosis
in many reported cases remains presumptive. It is, however, a relatively
rare disease, and owing to the improvements in the surgical technique of
the treatment of wounds is undoubtedly becoming rarer. The average of
most writers brings the percentage up to between 0.1 and 0.15 of clinic
2326 TEXT-BOOK OF OPHTHALMOLOGY
patients (Mooren, 1869 ; Becker, 1888; Ohlemann, 1890; Cohn, 1897;
Schirmer, 1900; Peters, 1919; de Grósz, 1926; Theobald, 1930).
The sea, incidence shows a great preponderance of males (about double
the proportion of females in the literature), a fact probably explained by the
greater exposure of the former to injury.
The age incidence is interesting in that it has always been held that the
young are especially susceptible (Reis, 1907; Domann, 1912; and
others); Schirmer (1900) explained this on the ground of the frequency of
perforating wounds among children. Taking the very carefully collected
and proved statistics of Fuchs (1905), Verhoeff (1927), Joy (1935) and
Woods (1936), it is found that 20.6% of cases occurred in the first decade,
13-3 in the second, 16-1 in the third, 11.0 in the fourth, 11.0 in the fifth, 13.3
in the sixth, and 14.7% above the age of 60. The interesting thing is that
these figures correspond very closely to the average age distribution of the
general population, so that there does not appear to be a very marked pre-
dilection so far as age is concerned. On the other hand it is quite certain
that no age is immune ; thus Fuchs (1905) found 6 out of 35 cases over
60 years of age, and Joy (1935) 8 out of 41. Curiously there is a seasonal
incidence, the majority of the cases occurring in the winter months
(Waldmann, 1935; Trowbridge, 1937).
Predisposing Causes. Quite a number of conditions may be responsible
for exciting sympathetic ophthalmitis. In general terms it may be said that
of these much the most common are perforating wounds which account for
some 65% of the cases in the literature ; operative wounds are responsible
for another 25%, while the remaining 10% are made up of cases which follow
non-perforating contusions with sub-conjunctival scleral rupture, perforating
corneal ulcers, and intra-ocular malignant tumours. With the exception
of those cases following intra-ocular malignant melanomata (and in many
of the reported cases perforation had occurred either by extension of the
neoplasm or by operative interference), it is the rarest thing for sympathetic
inflammation to arise without rupture of the globe. It is true that exceptional
cases have been reported after contusions without obvious perforation which
have been followed by a low-grade traumatic uveitis, or after phthisis bulbi
had developed as a consequence of uveitis. Analysing the literature
critically up to his time, however, Morax (1932) came to the conclusion
that, despite the many published cases there were only two above suspicion
(Milles, 1882; Deutschmann, 1889) wherein a proved sympathetic disease
occurred without perforation of some kind; and in one case, at any rate,
which gave no clinical evidence whatever of a perforation but seemed to
be a simple contusion, complete serial sectioning revealed a minute scleral
puncture (Andersson, 1938).
It is a very old observation that suppuration in the injured eye is rarely
followed by sympathetic disease, so old, indeed, that pre-Listerian surgeons
intentionally produced a “beneficent * suppuration in a badly injured eye
DISEASES OF THE UVEAL TRACT 2327
by passing a seton through it, believing that the purulent infection destroyed
the factors responsible for the condition or prevented infection passing up
the optic nerve by sealing the lymph spaces (Gifford, 1886). As a general
rule this is undoubtedly true ; but it must be remembered that several
instances have been reported following a post-traumatic panophthalmitis
(Gunn, 1886, 4 cases ; Schirmer, 1900, 2 cases ; Peters, 1919, listed 18 cases ;
Samuels, 1933–38, 3 cases out of 101 ; Woods, 1936; Trowbridge, 1937,
3 out of 32 cases).
(a) PERFORATING wounds. Ever since the time of Mackenzie (1835)
it has been universally recognized that the major cause of sympathetic
ophthalmitis is a perforating injury of the globe involving uveal tissue, and
in the vast majority of cases rapid and reactionless healing of the wound is
interfered with by some complication such as incarceration of the iris, ciliary
body or lens capsule, or the retention of a foreign body in the eye. The
most common exciting condition is that of a sub-acute inflammation in a soft
shrunken eye in which delayed or incomplete healing of the wound is present.
It has always been generally considered that wounds in the ciliary region
(the “danger zone *) are the most dangerous, but they do not form a pre-
ponderating majority ; thus in Fuchs’ (1905) series 23 out of the 35 cases,
and in Trowbridge's (1937) 21 out of 32 were not in the ciliary region. It
may be that any predilection for the ciliary region is explained not topo-
graphically but because the resultant incarcerative cyclitis readily retards
healing. Corneal wounds are relatively immune but when injuries of the
limbus are included, they form a large proportion of the whole (46.9%,
Trowbridge); even when they are centrally situated sympathetic inflamma-
tion may follow, especially when they are complicated by an incarceration
of the iris.
The statistical incidence of sympathetic ophthalmitis after perforating wounds of
the globe is of interest. Its assessment is somewhat difficult owing to lack of adequate
proof of the condition in many instances, but an average from the literature may be
taken at about 2%. The following figures may be quoted :— -
At Berlin (1869–1904) 30 cases in 1,291 perforating injuries (2.32%) (Steindorff,
1905).
At Bonn he wo 16 cases in 500 perforating injuries (1.2%) (Reis, 1911).
At Breslau (1897–1907) 17 cases in 577 (2.94%) (Kitamura, 1907), and (1908–23)
24 cases in 980 perforating injuries (2.45%) (Hentschel, 1923).
At Tübingen (1896–1908) 12 cases in 1,150 perforating injuries (1.0%) (Weigelin,
1910).
At º (1905–12) 12 cases in 387 perforating injuries (3.1%) (Domann, 1912).
At Illinois (1909–29) 8 cases in 1,465 perforating injuries (0.54%) (Theobald, 1930).
At Vienna, among 200 badly injured eyes which had to be excised, Fuchs (1905)
found changes characteristic of sympathetic disease in 35. -
It is interesting and says much for advances in surgery that while sympathetic
ophthalmitis was very common in the American Civil War (16.14% in 254 cases of
destruction of the eye-ball) and in the Franco-German War (the German returns gave
55–56% of all cases of injury to the eye-ball and the French 50%), and was still
relatively common (5% of eye injuries) in the Russo-Japanese War (Oguchi, 1913), such
2328 TEXT-BOOK OF OPHTHALMOLOGY
cases were rare during the World War (1914–18). Therein there were only 13 cases
reported in the German literature : Reis (1931) saw no instance in 2,000 perforating
injuries, Dimmer (1916) found it similarly negligible, and Schieck (1916), after circu-
larizing 150 serving surgeons, collected 8 cases only. Morax (1917) was able to collect
only 35 cases in the French army and himself saw one case in 1,500 serious ocular
wounds, the exception being an eye pierced with a splinter and subsequently operated
on for traumatic cataract. Similarly, Weekers (1917) had no instance in 800 ocular
injuries and de Lapersonne (1916) none in 1,000 injuries. Most English surgeons neither
saw nor heard of a case (Jessop, 1915); and in the Official Medical Returns of the
entire United States Army (1924) one case only gave rise to suspicion, and it cleared
up in a few days after enucleation of the injured eye and the administration of
salicylates.
(b) OPERATIVE wounds come second in numerical importance, and of
these slightly more than half follow operations for cataract ; it is to be noted
that in these the type of disease is usually severe. Here, again, healing has
been complicated by some misfortune such as incarceration of the iris or the
lens capsule. Adequate attention was first drawn to the occurrence of this
catastrophe by Critchett (1863), but there is no doubt that its incidence has
fallen considerably with the improved aseptic surgical technique of later
times. Thus, while Milles (1882) reported 11 cases after cataract extraction
at Moorfields Hospital in the years 1880–81, modern statistics are very much
lower indeed.
In the literature Schirmer (1900) gathered some 100 cases up to his time; Peters
(1919) found only these reports subsequently : Nance (1910), Brav (1912) and Mansilla
(1913). Eversbusch and Pemerl (1884) met 2 cases in 1,420 extractions (0.14%);
Bäuerlein (1884) none in 860, Schirmer (1900) 1 in 550 (0.18%) Domann (1912) 8 in
3,820 (0.21%); Theobald (1930) 2 in 7,444 extractions (0.027%).
Sympathetic ophthalmitis following other operations is rarer—
iridectomy, especially if the tension remains sub-normal (Deutschmann,
1889; Fehr, 1900; Fuchs, 1905; Ihumi, 1910 ; Theobald, 1930 ;
Trowbridge, 1937; Samuels, 1937), sclerectomy (Fuchs, 1905; Meller,
1914; Nagel, 1915; Schönenberger, 1930; Samuels, 1937), iridencleisis
(Trowbridge, 1937), iridodesis (v. Graefe, 1863; Gunn, 1886), discission
(Mooren, 1869; Gunn, 1886), tattooing of the cornea (Panas, 1878;
Trousseau, 1899), excision of a staphyloma (Fuchs, 1905); or after incom-
plete exenteration or excision when remnants of the eye are left behind
(Lawson, 1868; Mooren, 1869 ; Trousseau, 1899 ; Snellen, 1910; Schieck,
1912), more especially when a glass or metal ball is inserted, as in Mules’
operation (Cross, 1887–97; Gifford, 1908; and others).
(c) The PERFORATION OF A CORNEAL ULCER is a rare cause of sympathetic
inflammation, but several cases are to be found in the literature : Gunn
(1886), Weeks (1894), Fuchs (1905), Steindorff (1905), Thomson (1909),
Kitamura (1907), Weigelin (1910), Hentschell (1923), Lindemann (1925),
Verhoeff (1927), Joy (1935), Woods (1936), Groenouw (1936), and others.
(d) A SUB-CONJUNCTIVAL RUPTURE OF THE SCLERA is a rare cause, but in
DISEASES OF THE UVEAL TRACT 2329
many cases it is very difficult to exclude an unnoticed minute wound or
abrasion, in which case the condition becomes an open wound. Several
cases, however, are on record, among which may be noted those of Alt (1877),
Gunn (1886), Schirmer (1900, who collected 27 reported cases of which 7
were anatomically proved), Fuchs (1905), Gifford (1908), Domann (1912),
Hussels (1914), Ribstein (1915), Harman (1915), Joy (1935), and Woods
(1936). An interesting case was described by Zorab (1915) in which a
contusion-rupture at the posterior pole was followed by sympathetic
ophthalmitis.
(e) CONTUSION without rupture of the globe has been recorded as a very
rare cause, especially if it has been associated with a low-grade traumatic
uveitis or a persistent hyphaema, but the proof of the absence of a minute
perforation or of the presence of a definite sympathetic ophthalmitis has not
always been given (Delaney, 1931 ; Lamb, 1932; Joy, 1935; Trowbridge,
1937). Again, serial sectioning has demonstrated a minute puncture which
provided no clinical evidence of its existence and would have been
impossible otherwise to detect (Andersson, 1938).
(f) INTRA-OCULAR MALIGNANT MELANOMATA undoubtedly cause
sympathetic ophthalmitis, although here again several cases in the lit-
erature are complicated by perforation of the globe by extension of the
neoplasm, usually anteriorly but sometimes posteriorly (Meller, 1909) or by
operative interference. On a critical review very few of the recorded cases
give absolute proof of its development in the absence of perforation
(Morax, 1932). The occurrence, however, is well attested in the literature
and it seems to be associated especially with necrotic tumours (Milles, 1882;
Nettleship, 1886; Deutschmann, 1889; Nieden, 1894; Schirmer, 1900;
Fuchs, 1905 ; Meller, 1909 ; Reis, 1911 ; Butler, 1927 : Schwartz, 1927 ;
Daniels, 1931 : Joy, 1935 ; Melanowski, 1936 ; Trowbridge, 1937 ; and
others). -
(g) An IRIDO-CYCLITIS, particularly one which has progressed to phthisis
bulbi, has been said occasionally to give rise to sympathetic disease, for
example, in phthisis after small-pox (Nettleship, 1886) or after measles
(Gunn, 1886; Joy, 1935), or an inflammation of unknown aetiology (Fuchs,
1905; Kitamura, 1907; Weigelin, 1910; Meller, 1913; and others). Here
again most of the cases are difficult to assess ; in a few an iridectomy had been
performed which vitiates the argument, in others the involvement of the
second eye might have been a parallel infection (not at all an unusual
phenomenon), and in yet others the interval has been so long as not to
exclude a separate infection (Kitamura, 4 years ; Weigelin, 16 years).
The interval between injury and the onset of inflammation is also of
importance. Again, on this question the greatest diversity of opinion
exists in the literature. Nettleship (1886) gives the shortest interval as
9 days and the longest as 20 years. Schirmer's (1900) extremes found in the
2330 TEXT-BOOK OF OPHTHALMOLOGY
literature up to his date were 14 days to 42 years. Among the 200 cases
collected by the Ophthalmological Society of the United Kingdom, 18
occurred within 4 weeks, 170 after 4 weeks and within the 1st year, and
12 later than a year (Nettleship, 1886); and Woods (1936), reviewing
modern literature and confining his figures to proved cases, found 1 case
(Joy, 1935) in 9 days, 5 cases in the 2nd and 3rd weeks, the greater
number (61.2%) from 3 weeks to 2 months, a considerable number (24%)
from 2 months to 1 year, and a definite residuum of 10% after 1 year.
The following are among the extreme figures in the literature : Schirmer (1900)
reported a case after 15 years, Fuchs (1905) after 20 years, Gunn (1886) after 25 years,
Vignaux (1877) after 25 years, Sulzer (1907) after 37 years, Hentschell (1923) after
40 years, Sander (1931) after 40 years, Weeks (1894) after 42 years, Knapp (1871) after
45 years, Rockliffe (1907) after 50 years, and Joy (1935) 7 cases after an interval of
over 5 years and one after 48 years. -
With these long intervals, however, the diagnosis becomes very
problematical owing to the difficulty of eliminating a fortuitous irido-
cyclitis, or the possibility of the occurrence of an unnoticed second injury.
In nearly all of them the exciting eye has been shrunken and has become
painful and inflamed, perhaps spontaneously, perhaps by some trauma
shortly before sympathetic inflammation began, and then anatomical
investigation has revealed recent inflammatory changes. In general terms,
therefore, it may be said that cases occur eacceptionally before an interval of
2 weeks, that 65% occur before an interval of 2 months, 80% before an interval
of 3 months and 90% before the lapse of a year, the most dangerous time being
from the 4th to the 8th week. It follows that we may assume that the
chances of its development after 3 months are few, but that the possibility of its
incidence eacists indefinitely.
AEtiological Theories
Despite researches extending over a hundred years, the pathogenesis
of sympathetic ophthalmitis is unknown, but of the many theories which
have been put forward only two remain outstanding—the infective theory
and the allergic theory. The earlier ones require only passing mention.
Nerve Theories. The oldest writers (Mackenzie, 1835, and others) considered that
a state of inflammation was propagated along the optic nerves and chiasma from one
eye to the other ; Mooren (1869) suggested that the channel of transmission was the
trigeminal nerve ; while H. Müller (1858) promulgated the theory that irritating
impulses arising in the ciliary nerves were transmitted to those on the opposite side and
set up the condition of inflammation. In established sympathetic ophthalmitis there
are frequently demonstrable inflammatory changes in the ciliary nerves, which were
stressed by the advocates of this theory (Goldzieher, 1877; Krause, 1881; Uhthoff,
1883; and others); but this is what would be expected in a generalized inflammation
and need not be of aetiological significance. There is, moreover, some experimental
evidence that irritation of one eye results in reflex irritation of the other (Jesner, 1880;
Wessely, 1900), but no evidence germane to the point. Nor are the modifications of the
DISEASES OF THE UVEAL TRACT 2331
theory on any more sure foundation. Meyer’s (1891) suggestion that ciliary reflex
irritation set up sympathetic irritation in the Second eye if it was normal and
sympathetic ophthalmitis if it contained micro-organisms, or Schmidt-Rimpler’s (1892),
that ciliary irritation prepared the way for an unknown infective agent which normally
would have been ineffective, or Arkhangelsky’s (1935), bringing the same theory up
to the modern physiological concepts of the regulation of the tissue-metabolism by
the nervous system, are all equally lacking in proof.
1. The Infective Theory. Although the clinical behaviour and
pathological appearance of the disease strongly suggest an infective origin,
no bacteriological confirmation has resulted from innumerable researches.
The hypothetical organism, its route of entrance into the exciting eye, and
its method of attacking the sympathizing eye are alike unknown.
With regard to the organism many suggestions have been made, dating
from the early papers of Ayres and Alt (1887), Nordenson (1888) and
Randolph (1890). In the early days of bacteriology staphylococci,
streptococci and other types of undifferentiated cocci or minute particles
(Raehlmann, 1904) were from time to time hailed as aetiological agents, but
none has stood the test of time. An infective origin was suggested by the
experiments of Greeff (1893) and Schirmer (1900) who inoculated portions of
both an exciting and a sympathizing eye into the anterior chamber of rabbits
and set up a chronic uveitis, but the inflammatory reaction appeared in the
inoculated eye only. The resemblance of the blood-count in affected
patients to that found in many protozoal diseases and the occasional
response of the disease to arsenical treatment, prompted the suggestion that
it was associated with this class of infection (Browning, 1926; Hepburn,
1926); but for this there is no evidence. To-day two suggestions occupy
the centre of the stage, one, that the disease is a tuberculous manifestation,
and the other, that it is a virus infection.
(a) The Tubercle Bacillus. The granulomatous nature of the inflammation
and its general resemblance to tuberculosis have led many authors to
believe that sympathetic ophthalmitis is a tuberculous manifestation
(Meller, 1914–34; v. Hippel, 1917–34; Hentschel, 1923; Peters, 1925;
Urbanek, 1932; Riehm, 1933; and others). Guillery (1924–35) introduced
capsules containing tubercle bacilli and other toxins into the vitreous of
rabbits and produced a uveitis resembling sympathetic inflammation with
a similar involvement in the other eye, from which he concluded that the
disease is a manifestation of the activity of tuberculo-toxins rather than that
of the bacilli acting in conjunction with the catabolic products of tissue
destruction liberated by the trauma ; his work, however, was not confirmed
by v. Szily (1924–26), and his conclusions were questioned by Meesmann
and Volmer (1927), Marschesani (1928), and Poos and Sartorius (1930). The
demonstration by Löwenstein (1931), however, of the frequency of a
systemically innocuous tuberculo-bacillaemia, of its occurrence in the blood
in cases of sympathetic ophthalmitis (Meller, 1932–34), and finally, the
2332 TEXT-BOOK OF OPHTHALMOLOGY
demonstration of the bacilli in the eye in such a case (Meller, 1936), added
strong arguments to this view ; but these findings again have so far lacked
general confirmation," and it may be argued that if the bacilli are innocuous
in the blood-stream and in other organs, they may be innocuous, and
therefore not causative, even if present in the eye. It must certainly be
admitted that Koch’s postulates have not yet been fulfilled.
(b) A virus infection is obviously suggested by the failure of bacteriology
to discover a causal organism. Such an aetiology was originally suggested by
zur Nedden (1905), who put forward the very attractive theory of a causal
virus inhabiting the conjunctival sac and gaining entrance to the exciting
eye through trauma. This suggestion was supported by the work about to
be mentioned of the proven facility with which the herpes virus travels from
one eye to the other ; but most attempts at reproducing the inflammation
by the inoculation of filtrates from affected eyes have proved negative
(Marchesani, 1925; Undelt, 1926; Meesmann and Volmer, 1927; and
others). It is true that Gifford and Lucic (1929) obtained a positive result
with three negative ones, and that v. Szily (1934) produced a nodular
infiltration in the eyes of monkeys and chicks from an extract of the ground up
ciliary body of a human case ; and it is possible that experimental failures
can be explained on the supposition that the hypothetical virus is short lived,
or requires peculiar culture-media, or is indifferent to experimental animals.
The question of how the infection lights up in the injured eye is also
unanswered. The older view is that it is eacogenous, presumably conjunctival,
and finds a portal of entry at the perforating wound, a view supported by the
occasional observation of a much more active and intense infiltration here
than elsewhere indicating the existence of a primary lesion (the INOCULATION
CHANCRE of Redslob) (Redslob, 1921 ; Marchesani, 1925; Samuels, 1933;
Felsenthal, 1934). The fact that such a primary lesion cannot always be
demonstrated (Fliri, 1925) does not rule out the possibility that a primary
infection may well occur without producing a primary lesion, or at any
rate a lesion capable of histological observation. On the other hand a
school of thought believes that the infection is endogenous, and that the fact
of trauma and the consequent impairment of the vitality of the tissue are
sufficient to localize organisms already circulating in the blood (Meller,
1914–35; Fliri, 1925). This contention has been forcibly supported by
Meller (1935) from the histological view-point with reference to the marked
changes occurring in the blood-vessels, particularly in the major circle of the
iris. Such a contention cannot be refuted, and seems to be the readiest
explanation of those very rare cases wherein sympathetic disease occurs
without perforation of the globe—if, indeed, such cases do occur ; but then
again it may be argued that a lowering of the vitality of the tissues by
1 p. 2143.
DISEASES OF THE UVEAL TRACT 2333
contusion or disease may facilitate the entrance of an organism without an
actual solution of continuity.
The route of infection from the earciting to the sympathizing eye is also a
matter of speculation. Most authorities consider that the condition is a
metastatic one carried by the blood-stream, a supposition mentioned by
Mackenzie (1835) but first strongly advocated by Berlin (1880). This was
at first bitterly opposed, notably by Leber (1881–1904), on the ground that
a metastatic deposition in one organ was absurd. More recent advances in
bacteriology, however, have deprived this argument of much of its force, by
postulating an organism which in the circulation and in other tissues is
non-virulent, but has gained a selective affinity for uveal tissue (Gifford,
1908; Parsons, 1908), or is activated in the eye by allergic influences. In
this event the organism could either be exogenous, and only enter the
circulation after its introduction into the first eye, or it could be endogenous,
and be localized in the first eye by trauma and in the second by selective
affinity or allergic sensitization. It is obvious that most of the obscure
facts of sympathetic ophthalmitis can be explained on this theory: the
latent period being the interval of propagation in the exciting eye, the value
of excision before general dissemination has occurred, and the possibility of
an indefinite period of dormancy (e.g., by encapsulation) and eventual
reactivation, perhaps by a slight injury, at a very much later date.
A second theory suggests that the spread of infection is directly from
eye to eye. Passing mention need only be made of Arnold's (1891) view that
bacterial transmission occurred by reverse venous flow through the cavernous
sinus. More is to be said for the theory of bacterial transmissions by the
lymph channels in the intervaginal spaces of the optic nerves and chiasma—
the OPHTHALMIA MIGRATORIA of Deutschmann (1882–99). Leber (1881–1904)
and Deutschmann (1882) put forward this view after experimental work
in which they found inflammatory changes in the optic nerves and a
papillitis in the other eye by injecting irritants and toxins into the vitreous
of one eye, results, however, which most other experimentalists were unable
to confirm (Mazza, 1887; Gifford, 1888; Randolph, 1890–92; Schirmer,
1892; Angelucci, 1896; and others). At a later date Deutschmann (1927)
claimed to have verified his earlier work by experiments on monkeys, and
Rrug and Rohdenburg (1932) found that oils and particulate matter followed
the same route. Although this claim was denied by Berliner and Nomidez
(1932), the former authors found that tuberculin excited a bilateral response
along this path, an observation rendered more interesting by the fact that
tubercle bacilli did not, since the reaction produced by them blocked up the
lymph spaces—a possible explanation of the immunity of suppurating eyes.
Clinical evidence in favour of this hypothesis is, however, slight.
Deutschmann (1893) found post-mortem in a case of sympathetic
ophthalmitis which died of carcinoma of the stomach that both optic
* Wide infra.
2334 TEXT-BOOK OF OPHTHALMOLOGY
nerves and the chiasma were infiltrated with round cells while the brain and
meninges were normal, an observation confirmed by A. Fuchs (1924). The
possibility of spread along the direct nerve path has also received ample
corroboration by the inoculation of one eye in rabbits with herpes virus, the
observation of the development of inflammation in the second eye, and the
histological demonstration of continuous infiltration along the optic nerves
and chiasma (Mariani, 1924; v. Szily, 1924–27 ; Grüter, 1925; Gifford and
Lucic, 1926; Abe, 1926; Velhagen, 1927; and others). This, of course,
does not prove that sympathetic disease is an expression of herpes, but it
seems to indicate that a virus can travel from one eye to the other along this
path.
2. The Allergic Theory. The basis of the allergic theory of the aetiology
of sympathetic ophthalmitis was the demonstration by Elschnig (1910–11)
that uveal pigment was capable of acting as an antigen,” from which he
assumed that injury to the exciting eye resulted in an absorption and general
dissemination of the pigment, which produced a hypersensitivity especially
in the homologous organ so that continued absorption resulted in an allergic
intoxication of the sensitized tissue of the second eye, a state which
ultimately became clinically manifest as sympathetic inflammation. This
theory has received a considerable amount of experimental confirmation
from Woods (1918–33) who showed that it was possible to produce uveitis in
the second eye after intra-ocular sensitization of dogs with uveal pigment
and subsequent intoxication by intra-peritoneal injection. He also showed
(1921) that a complement fixation reaction to uveal pigment occurred in the
blood after injury to the eye, the complement binding substances being
usually present in patients whose eyes healed normally, and being absent in
those who developed sympathetic ophthalmitis—a finding confirmed by Fodor
(1927). Woods (1925–33) also found that patients who developed sympa-
thetic inflammation usually demonstrated a skin hypersensitivity to pigment
which was absent in normal individuals, and that the disease responded well
to therapeutic injections of uveal pigment. Friedenwald (1934) also showed
that the intra-dermal reaction in sensitive individuals had all the histo-
logical characteristics of the inflammatory reaction in the eye. Woods
claimed, therefore, that the normal healing of a wound was associated with
the appearance in the blood-stream of anti-bodies specific for uveal pigment,
but that when protracted inflammation occurred those anti-bodies did not
appear, and that, when sympathetic ophthalmitis developed, not only were
these anti-bodies lacking but a cellular hypersensitivity for uveal pigment
developed. These clinical observations have been repeated, but not with
uniform results, by Verhoeff (1927), Berens (1927), Gifford (1928) and Gill
(1930).
That allergy may well enter into the picture is supported not only by
4 p. 2134.
DISEASES OF THE UVEAL TRACT 2335
the work of Woods but also by the collateral findings of others. Thus
Marchesani (1929) produced an inflammation of the fellow eye after repeated
inoculations of B. subtilis in rabbits, apparently by the development of a
paired-organ sensitivity. It is true that bacterial metastases could well have
been responsible for the involvement of the second eye (Iga, 1929; Kiyosawa,
1931), but that sensitivity may enter into the matter is suggested by the
experiments of Riehm (1928–30) who reached the conclusion that a foreign
protein absorbed from one eye produced an elective sensitivity in the fellow
eye in pigmented rabbits but not in albinos.
At the same time it seems impossible that sympathetic ophthalmitis can
be a simple allergic reaction, a fact, indeed, insisted upon by Woods
(1925–33) in his later writings. In the first place, the pathological findings
in no way resemble any known manifestation of allergic response, although
the early and widespread destruction of pigment may be significant
(Friedenwald, 1934; Oguchi, 1934). Again, considerable disturbances of
uveal pigment constantly occur after contusions or disease 1 without
deleterious results ; the liberation of pigment, therefore, does not seem to be
the only exciting factor. Further, optic neuritis is sometimes an early
and prominent symptom and the optic nerve contains no pigment. Again,
one would necessarily require two separate disturbances, the first to provide
the sensitizing, the second the shocking dose; if, after the original dis-
turbance has subsided, a small anomaly occurring subsequently in the uvea
be supposed to elicit the reactive process, it is difficult to understand the
prophylactic value of excising the exciting eye for the slightest disturbance
in the remaining eye ought to provide a similar shocking dose which would
thus equally endanger it for weeks, for years, or sometimes for life. Since
experience shows that this does not occur, it would seem that sympathetic
Ophthalmitis must have some other factor as its essential cause.
While the aetiology of sympathetic ophthalmitis thus remains uneluci-
dated, it seems almost certain that it is infective in origin of the possible
infections the most attractive hypothesis is that of an unknown virus,
probably one inhabiting the conjunctival sac. Reviewing the immense
amount of research expended on the question and gathering all the various
hypotheses put forward from time to time, it seems most probable that such
an infection is exogenous, that it eventually becomes systemic but excites no
symptoms, and that it is localized in the other eye by allergic sensitization,
or because the uvea forms the only favourable nidus for its development.
On the other hand, a direct spread from eye to eye is by no means impossible.
It is to be remembered, however, that although the possibility of either
mechanism has been demonstrated, no specific proof to support one or the
other view has yet appeared. -
1 p. 2418.
T.O.-WOL. III. R
2336 TEXT-BOOK OF OPHTHALMOLOGY
Clinical Picture
In the eacciting eye no clearly cut clinical picture forms an invariable prelude
to sympathetic disturbance in the other. As a rule it is an eye which has
received a perforating wound in the ciliary region, which does not clear up
readily but remains angry, injected and irritable, developing the picture of a
persistent, indolent, low-grade uveitis usually with periodic painful
exacerbations, which has a thickened, unresponsive iris with nodules on
the pupillary margin and on its surface, a capsular clouding of the lens, and
eventually shows a tendency to phthisis bulbi. The most important sign of
all is the appearance of precipitates on the back of the cornea—always a
sign of danger. While this clinical picture of a low-grade, recurrent traumatic
uveitis with a tendency to phthisis is typical, it is by no means invariable,
for sympathetic inflammation may follow almost any type of condition—on .
the One hand a clean wound that has apparently healed rapidly and without
complications, and on the other a violently acute irido-cyclitis, or even,
on rare occasions, a purulent panophthalmitis.
In the sympathizing eye a similarly diverse clinical picture may be seen,
so much so, indeed, that the older writers (v. Graefe, 1866; Mooren, 1869 ;
Randolph, 1890; Schirmer, 1900; and others) spoke of several sympathetic
syndromes—sympathetic serous iritis, plastic iritis, papillo-retinitis, optic
atrophy, cataract, detached retina, scleritis, and so on. It is true that the
disease may start either in the anterior or posterior segment—usually the
former—and it is true that it varies very considerably in its clinical type ;
but on the whole it ultimately resolves into the same clinical picture of
complete uveitis characterized generally by an insidious onset with the
appearance of keratic precipitates and early visual failure, an absence of
objective symptoms and pain in the early stages, a long drawn-out course
interpolated with exacerbations, an immobility and thickening of the iris,
sometimes with nodular formation and always with posterior synechiae which
eventually cover the pupillary aperture and become vascularized forming
a membrane continuous with the iris, a tendency to the development of
secondary glaucoma, and eventually to shrinkage and phthisis bulbi.
As a rule prodromal symptoms of a sensitiveness to light and transient
obscurations of vision due to accommodative failure or the develop-
ment of transient myopia such as occurs in early cyclitis 1 (Ellett,
1922; Marchesani, 1925; Swab, 1928) may occur intermittently for
some time, associated with the objective development of some slight
lacrimation, a mild ciliary injection and tenderness on pressure. These
should excite suspicion ; for although they may disappear under treatment
(PRECOCIOUS SYMPATHETIC OPHTHALMITIs ; Vogt, 1921 ; Damel, 1932), they
are usually followed by the early symptoms of a mild serous cyclitis, charac-
terized by floating elements in the aqueous and retro-lental space, a fine,
1 p. 2200.
DISEASES OF THE UVEAL TRACT 2337
diffuse oedema of the corneal endothelium, the presence of a few precipitates,
a clouding of the aqueous, some vitreous haze, a lustreless iris and a slight
ciliary injection—symptoms so slight that they may well pass unnoticed
until visual failure calls attention to a condition already well established.
Fundus examination may reveal peripheral choroiditis or slight papillitis
with blurring of the margin of the disc, or general retinal oedema. Some-
times the inflammation recedes at this stage ; but much more usually, after
a month or two a plastic irido-cyclitis of considerable severity develops. In
other cases this plastic form is evident from the beginning. The keratic
precipitates increase in number, the vitreous becomes turbid and the lens
capsule clouds over, the iris becomes thickened frequently showing large
flat nodules on its surface, new vessels appear, posterior synechiae rapidly
develop and spread until the entire pupillary margin is bound down to the
clouded lens, and eventually the thickened lens capsule is covered by an
inflammatory membrane continuous with and vascularized from the iris.
For some months in the worst cases the tension rises while a complete
cataract and total posterior synechiae become established, and before the
ultimate relapse into a condition of lowered tension and eventual phthisis,
all vision has usually been lost.
On the other hand, but much more rarely, the disease may start in the
posterior segment without evidence of an irido-cyclitis but with swelling
and congestion of the optic nerve and diffuse retinal oadema most marked
at the posterior pole. A preliminary symptom may be a sudden hyper-
metropia due to thickening of the choroid at the posterior pole. A
characteristic feature of sympathetic choroiditis is the presence of small
white spots, somewhat resembling drusen, due to changes in the pigmentary
epithelium (DALÉN's SPOTS) (Hirschberg, 1879). Eventually small exudative
patches appear in the peripheral area of the fundus, clouding of the vitreous
rapidly obscures the picture, opacities develop in the lens, and the inflam-
mation spreads to the anterior uvea producing the characteristic picture
already described.
The occurrence of a simple optic neuritis, as described by some of the older authors
(Hirschberg, 1874; Vignaux, 1877; Eversbuch and Pemerl, 1884; Brailey, 1884;
and others) is questionable ; it is usually the prelude or the sequel of a generalized
uveitis. An exudative retinal detachment is not uncommon and may even be an
initial symptom (Axenfeld, 1909; Aykia, 1920). Some scleral involvement is a
common pathological finding, but it is not usually clinically evident although intra-
scleral nodules may lead to rupture of the sclera and extra-ocular proliferation
(Schirmer, 1900; Blascheck, 1903 ; Ruge, 1904); and the keratitis which has been
noted exceptionally is probably a symptomatic manifestation. A bleaching of the eye-
lashes and even the eyebrows is another curious phenomenon which occurs, occa-
sionally associated with deafness (Schenkl, 1873; Nettleship, 1884; Bock, 1890;
Tay, 1892; Vogt, 1906; Cramer, 1912; Soriano, 1929; and others).
General systemic symptoms suggesting meningeal involvement are also rare ; a
1 p. 2364.
23:38 TEXT-BOOK OF OPHTHALMOLOGY
high temperature (Widmark, 1908; Zuntz, 1915; and others), severe headache and
meningitis (Snellen, 1881; Risley, 1885; Deutschmann, 1889; Lugue, 1936; and
others).
Although the course of sympathetic ophthalmitis is variable, it must
always be looked upon as a very serious disease. Cases undoubtedly occur,
especially the serous type of cyclitis, which run a mild and short course
to recovery—(2 in 35 cases Fuchs, 1905; 9 in 41, Joy, 1935; 6 in 28, Wood,
1936). As a rule, however, the disease runs a chromic course with a decided
tendency to relapses, an average duration of the cases which quieten being
somewhere between 9 and 12 months. Thereafter subsequent relapses with
sharp exacerbations of acute inflammation are common. Occasionally these
are remarkably well borne (Villard, 1931); an example is a case reported
by Verhoeff (1927) which had 8 relapses in 8 years, the patient still being
under treatment and still retaining good vision. More usually, however, the
eye is unable to survive this ordeal and vision is lost. In such cases the
inflammation usually remains active for a year or more, and even thereafter,
when blindness had resulted, persistent activity may give rise to periodic
exacerbations of the inflammation which recur indefinitely and
monotonously until the eye is removed.
Pathology
It has been definitely established that both the eacciting and the
sympathizing eye, apart from post-traumatic changes in the former, show
fidentical pathological changes, and since the habit of excising the former has
been a routine for many years, the wealth of material thus provided has given
ample opportunity for the full investigation of the characteristic histological
changes. Essentially, these comprise a massive round-celled infiltration of
the whole wreal tract and particularly the choroid, with a preponderance of
epithelioid cells and some giant cells, and with a definite tendency towards the
formation of nodular aggregations (Fig. 1958, Plate XLII).
Most of the pathological studies have naturally been made upon the exciting eye.
Although earlier histological investigations of considerable importance had been
made (Brailey, 1881; Krause, 1881 ; and others), the first comprehensive pathological
description was published by Schirmer (1892), after which a considerable series of
papers followed (Pincus, 1894; Fehr, 1900; Gurnert, 1900; Ruge, 1904; Dalén,
1904; and others) until the classical paper of Fuchs (1905) describing 35 cases appeared;
since then there has been little to add to the picture. Subsequent papers of importance
are those of Brown (1907), Lenz (1907), Fuchs (1909), Gilbert (1910), Weigelin (1910),
Reis (1911), Domann (1912), Goldzieher (1913), Meller (1913–20), v. Hippel (1917),
Brückner (1917), Redslob (1921), Wood (1927), Jäger (1927), Schreiber (1930), Theobald
(1930), Samuels (1933–37), Woods (1936), Trowbridge (1937), Andersson (1938), and
others. Studies of the sympathizing eye are fewer (Pagenstecher, 1873; Becker 1882;
Milles, 1882; Deutschmann, 1893; Zimmermann, 1895; Schirmer, 1900; Grunert,
1900; Asayama, 1902; Blascheck, 1903; Ruge, 1904 Lenz, 1907 : Wagenmann,
1910; Weigelin, 1910; Pollot, 1912; Bräutigam, 1912; Krailsheimer, 1914; Meller,
1914; Jäger, 1927; Wood, 1930). -
DISEASES OF THE UVEAL TRACT 2339
Fig. 1941. THE TYPICAL INFILTRATION IN SYMPATHETIC OPHTHALMITIs.
In the choroid at the point of perforation of the sclera by a metallic foreign
body (Samuels, A. of O.).
Throughout the uveal tract the initial infiltration is composed of
lymphocytes and plasma cells, but these are soon followed by the masses
of large epithelioid cells characteristic of the disease, which in the more
chronic stages coalesce to form multi-nucleated giant cells (Fig. 1941);
Fig. 1942. –SYMPATHETIC OPHTHALMITIs.
Nodular infiltration in the iris (Parsons).


2340 TEXT-BOOK OF OPHTHALMOLOGY
-
Fig. 1943.-SYMPATHE Tic Ophth ALMITIs.
Section through the iris showing giant cells, epithelioid cells, plasma cells,
lymphocytes (x 300) (D. J. Wood, Brit. J. O.).
occasionally eosinophils are numerously represented. These elements at
first have a nodular distribution but eventually form a uniform and con-
tinuous lamina. The typical stages in the infiltration are:-
1. Focal infiltration of lymphocytes around and in the larger veins,
especially of the choroid.
Fig. 1944–Sympathetic Ophthalmitis.
Infiltration of the iris with plasma cells which fill the anterior chamber and
appear again behind the line of the pigmentary epithelium (x 140) (D. J. Wood,
Brºt, J. O.).


DISEASES OF THE UVEAL TRACT 2341
2. Typical nodules of epithelioid cells with giant cells in the centre
surrounded by lymphocytes.
3. Tumour-shaped masses appearing in the iris and a uniform diffuse
infiltration in the choroid, especially in its outer layers.
Fig. 1945. SYMPATHETIC OPHTHALMITIs.
The typical infiltration in the ciliary body (Theobald, Am. J. O.).
Fig. 1946.-SYMPATHETIC OPHTHALMITIs.
Infiltration in the ciliary region, showing a prolapse of infiltrated tissue (Parsons).
In the iris the infiltration commences in the posterior layers, sometimes
behind the sphincter, where large nodular masses of cells are formed
(Fig. 1942), while the anterior layers may remain free from infiltration for a
long time. These cellular masses prefer to spread posteriorly; they invade


2342 TEXT-BOOK OF OPHTHALMOLOGY
and tend to break through the pigment epithelium at a relatively early
stage and spread out over the anterior capsule of the lens giving it its cloudy
appearance, and eventually they form the characteristic membrane-like
synechiae which give the appearance of a confluence of the iris and lens
capsule. Eventually, however, the iris becomes completely infiltrated
(Fig. 1943) and may ultimately form a tumour-like mass (Fig. 1944). The
ciliary body shows involvement first in its posterior part between the
Fig. 1947. Sympath Etic Ophthal Mitis.
A lymphocytic nodule in the choroid (x 200) (D. J. Wood, Brit. J. O.).
retinal epithelium and the ciliary muscle, which resists invasion for a long
time, the infiltration remaining confined to the vascular layers (Fig. 1945):
eventually, however, the entire tissue becomes involved (Fig. 1946).
The choroid is usually the site of the most intense inflammation especially
towards its posterior parts, and may become three or four times its normal
thickness (Fig. 1947), and may even reach such proportions as almost to
fill the cavity of the globe in the short space of a few weeks. The first
deposits occur in the external layer especially along the veins in the layer of
large vessels; even in the later stages when the infiltration has become
diffuse, the chorio-capillaris remains relatively free. The tendency is rather

DISEASES OF THE UVEAL TRACT 2343
Fig. 1948. SYMPATHETIC OPHTHALMITIs: ExcITING EYE.
Non-specific traumatic exudate. Specific sympathetic inflammation (A. C.
Woods, Am. J. O.).
to invade and fill the supra-choroidal space. In the posterior part of the
uvea as in the anterior, the early cellular deposits are nodules of lympho-
cytes, and as the infiltration spreads and becomes diffuse they become
interspersed with masses of epithelioid cells in the centres of which lie giant
Fig. 1949. Sympath ETIC INFILTRATIon of THE PRollapsed IRIs AT THE SITE
of INJURY (Samuels, A. of 0.).


2344 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 1950–Sympathetic Ophthalmitis.
Dalén-Fuchs area. Epithelioid cells replacing the pigment epithelium of the
ciliary body (Theobald, Am. J. O.).
Fig. 1951. DALEx-Fuchs Nobuie is Fig. 1952–DALéN-Fuchs Nobuº is
Sympathetic OPHTHALMITIs (A. C. Sympathetic Ophthal Miris.
Woods, Am. J. O.). Higher magnification showing pig-
ment phagocytosis (A. C. Woods, Am.
J. O.).


DISEASES OF THE UVEAL TRACT 2345
cells. Eventually the stroma disintegrates and becomes depigmented while
the epithelioid and giant cells phagocytose the pigment, Bruch's membrane
disappears, the vessels, first the veins and then the arteries, succumb, and
finally, often only after the lapse of years, the whole tissue is replaced by a
shrunken layer of pigmented connective tissue.
In the exciting eye it is to be remembered that this characteristic
picture may to some extent be obscured by that of an exudative
inflammation, the result of the traumatic endophthalmitis that has followed
the injury, the plastic endophthalmitis and the sympathetic infiltration
going on side by side (Fig. 1948). In this eye, also, in some cases but by
Fig. 1953.−SYMPATHETIC OPHTHALMITIs.
Leucocytic invasion of the retina with destruction of nerve-fibres and ganglion
cells (x 140) (Wood, Brit. J. O.).
no means in all, a peculiarly intense reaction occurs at the site of the wound
(Redslob, 1921; Marchesani, 1925; Samuels, 1933; Trowbridge, 1937).
Prolapsed portions of the uveal tract are almost always heavily infiltrated
and may form greyish-blue staphylomatous-like masses at the site of the
wound (Figs. 1946 and 1949).
The reaction of the pigment epithelium on the posterior surface of the
iris, and overlying the ciliary body and the choroid, is peculiar (Dalén, 1904;
Fuchs, 1905). There is frequently a general disintegration of the pigment
epithelium associated with an invasion by the infiltrate from the stroma
(Fig. 1950). More characteristically, in isolated areas groups of the cells of
the pigment epithelium swell and proliferate to form a nodule (THE DALÉN-
Fuchs NoDULE), which may increase considerably in size, the cells elongating
into a spindle-shape (Figs, 1951 and 1952). Eventually they undergo
autolysis and depigmentation and are invaded sometimes by lymphocytes

2346 TEXT-BOOK OF OPHTHALMOLOGY
and sometimes by epithelioid cells from the underlying stroma which
phagocytose the liberated pigment.
The remaining tissues show much less massive changes. In the retina
there is always cellular perivascular infiltration especially round the veins
(SYMPATHETIC PERIvasculits of Meller, 1921; Echevarría, 1922), a condi-
tion which may be associated with retro-bulbar neuritis (Fig. 1953). Typical
profuse infiltration is rare (Ruge, 1904), but it may occur to such an extent
that the vitreous is heavily invaded (Wood, 1930) (Fig. 1954). Occasionally,
in the anterior type of the disease the retinal changes are more marked than
the choroidal, but in this case the disturbance is essentially a periphlebitis
Fig. 1954–SYMeathetic Ophthal Miris.
Lymphocytic infiltration of the retina. The cells have penetrated the internal
limiting membrane and entered the vitreous (x 140) (D. J. Wood, Brit. J. O.).
derived from the ciliary region, the origin of which we have already traced
(Schreiber 1932). The optic disc is usually little affected beyond a peri-
vascular infiltrate, but there is frequently a dense infiltration behind the
lamina cribrosa which rapidly diminishes further back (Fig. 1955), although
nodular formations along both nerves and across the chiasma have been
described by Deutschmann (1893) and A. Fuchs (1924).
A spread of the infiltration is more common outwards, when a mantle
is formed round the perforating vessels, especially the emissary veins
(Fig. 1956). Thence masses of cells infiltrate the inner layers of the sclera,
and may even appear as nodules which proliferate at the points of exit of
the vessels (Schirmer, 1892; Blascheek, 1903; Ruge, 1904; Woods, 1936;
Trowbridge, 1937). At other times in severe cases a diffuse internal scleritis
p. 2201.

DISEASES OF THE UVEAL TRACT 234.7
º
º
-
-E.
º
º
-
-
-
-
Fig. 1955.-SYMPATHETIC OPHTHALMITIs.
Involvement of the pial and arachnoid sheaths of the optic nerve (Samuels,
4. of 0.).
Fig. 1956.-SYMPATHETIC OPHTHALMITIs.
Infiltration around the perforating vessels of the selera (A. C. Woods, Am. J. O.).















2348 TEXT-BOOK OF OPHTHALMOLOGY
develops, large areas posteriorly being uniformly and heavily infiltrated, or
circumscribed nodules may form distinct from the perforating vessels
(Samuels, 1933).
Finally, infiltration may be found in the sub-conjunctival tissues
(Collins, 1914), in the surrounding orbital tissues, and even in the external
muscles quite a distance from the globe (Samuels, 1933).
Diagnosis
The diagnosis—especially the early diagnosis—of sympathetic
ophthalmitis is one of the most important in ophthalmology, for its
establishment usually means the sacrifice of One eye, and failure to establish
it may well mean the loss of both. It may be approached from three
aspects—clinical, serological and pathological.
The clinical diagnosis depends essentially on minute and accurate
observation with the slit-lamp. The essential early phenomenon is the
occurrence in the aqueous, the retro-lental space, and the vitreous of free floating
particles, often in large quantity, which ultimately are deposited as pellucid,
granular keratic precipitates on the back of the cornea, and as small patches
of exudate on the pupillary margin' or sometimes on the iris itself
(Vogt, 1921). It has been claimed that in their absence sympathetic
ophthalmitis does not exist (Butler, 1926). In association with the history
of injury the appearance of keratic precipitates indicates a very dangerous
eye ; but before the typical picture of a low-grade, insidious uveitis with
visual failure develops, the diagnosis should have been made.
The Serological diagnosis is more open to question. The blood-count is considered
to be of value by Browning (1926), who claimed a characteristic excess of large mono-
nuclear lymphocytes and a concomitant decrease in polymorphonuclear leucocytes;
but this picture is neither universal nor specific. The intra-dermal pigment test
already discussed has been advocated by Woods (1925), and it certainly is the case,
whether its aetiological significance be incidental or not, that a positive reaction occurs
in many cases. The test, however, can by no means be considered obligatory ; a
negative result is valueless, and a positive result can only be accepted as confirmatory
of other evidence.
Pathological diagnosis is frequently possible because of the routine
habit of excising a suspicious exciting eye ; the presence in it of specific
changes thus establishes the diagnosis for purposes of treatment of the other.
It is interesting that Fuchs (1905) was able to separate accurately his series
of 200 injured eyes into those which had and those which had not excited
sympathetic disease from pathological evidence alone without reference to
the case history. The special points of diagnostic importance are :—
1. The tendency to general uniform infiltration of the entire uveal
tract, and the early infiltration of the posterior layers of the iris.
2. The early infiltration, chiefly of lymphocytes, which first appears
1 pp. 2180, 2198.
PLATE XLII
Fig. 1957. HETERochRoxſic IRIDo-cyclitis (RIGHT Eye).
Fig. 1958.-SYMPATHETIC OPHTHALMITIs.
Advanced infiltration of entire uveal tract (Samuels, A. of 0.).
[To face p. 23.48.


DISEASES OF THE UVEAL TRACT 2349
as focal accumulations in the walls of veins, invading and occluding their
lumen, and the early infiltration of the emissary veins. This is a distin-
guishing point even in the absence of epithelioid or giant cells.
3. The early destruction of the pigment epithelium, the early
phagocytosis of pigment, and the presence of Dalén-Fuchs nodules.
4. The absence of tissue destruction, caseation and necrosis.
From the pathological point of view sympathetic ophthalmitis has to be commonly
differentiated from three conditions.
1. Infective endophthalmitis.” While sympathetic ophthalmitis is a proliferation
and infiltration within the uvea itself, especially the choroid, infective endophthalmitis
affects primarily the tissues surrounding the vitreous cavity—the retina, ciliary body
and iris, while the choroid is affected secondarily, the infection spreading from the
anterior and posterior parts. It is an exudative inflammation characterized primarily
by polymorphonuclear cells.
2. Chronic traumatic weitis.” Here the same tissues are preferentially attacked,
and the infiltration is mononuclear.
3. Tuberculosis. Here, instead of becoming uniform, the infiltration remains
focal and nodular, the anterior layers of the iris are preferentially affected, involvement
of the emissary veins occurs at a late stage, pigment phagocytosis is rare, and the
surrounding tissues tend to be destroyed by caseation and necrosis.
The prognosis of sympathetic ophthalmitis is very difficult to assess,
for the literature includes many cases of questionable diagnosis, more which
were incompletely followed up, and still more which came under observation
when the eye was hopelessly involved in pathological changes. While in
advanced disease the prognosis is generally very bad, if the case is seen early
and adequate treatment instituted it is by no means necessarily so. An
adequate idea of the expectation of a good prognosis—that is, subsidence
of the inflammation, and retention of useful vision—is given by the recent
American statistics reported by Post (1934), Joy (1935), Verhoeff and Irvine
(1936), and Woods (1936)—a total of 125 cases in which the diagnosis was
histologically proved and the final result observed. Of these 51.2% showed
a favourable, and 48.8% an unfavourable outcome, so that the chances
appear to be about even. If, however, only early cases are considered
(74 of these cases), the favourable results rise to a figure of 66%.
Prophylazis
In the first place prophylaxis should concern itself with the adequate
toilet of perforating wounds. The two most common precursors of sympa-
thetic disease are delayed healing owing to entanglement of the iris, ciliary
body or lens capsule in the wound and the retention of a foreign body : every
endeavour should therefore be made to eliminate these at the soonest
possible moment, for upon the success of these measures the ultimate fate
of both eyes may depend. In the surgery of a wound an undoubted prophy-
1 p. 2251. * p. 2135.
2350 TEXT-BOOK OF OPHTHALMOLOGY
lactic measure of importance is its immediate covering with a large
conjunctival flap, and it is probable that protein shock therapy as soon
as possible after the injury is of value. If, despite these precautions,
the eye continues to remain irritable with photophobia, lacrimation, and
ciliary injection, more drastic steps should be contemplated. It is now
generally recognized and universally acted upon that the most valuable and
important method to prevent the Onset of sympathetic disease is enucleation of
the injured eye before the characteristic pathological changes have become
established. It is an idea, as we have already seen, borrowed from veterinary
surgery by Wardrop (1819), put into practice by Prichard (1851), and fully
established in ophthalmological routine as a measure of proven value by
Critchett (1863).
Other alternatives have been suggested but none of them is justifiable. Bunge
(1880) in v. Graefe’s clinic suggested exenteration, but cases rapidly accumulated
wherein sympathetic disease appeared in the second eye in spite of it, an occurrence
demonstrated anatomically to be due to remnants of uveal tissue left behind (Pflüger,
1896; Schmidt-Rimpler, 1900; Ruge, 1904; and others). Through the stimulus
provided by the migratory theory of transmission up the optic nerves, an optico-ciliary
neurotomy was at one time practised, first by Bouchéron (1876–79), but this was
abandoned after extensive trials. To-day the only recognized method is enucleation,
and, owing to the possibility of extra-ocular spread and to our ignorance of the
method of transmission to the other eye, it is probably best to remove as much of
the surrounding tissue including the muscular insertions and as large a portion of the
optic nerve as is practicable ; indeed, Samuels (1933) has suggested the advisability
of radiation of the socket with a view to destroying any potentially active material
left behind.
The ideal time to enucleate a suspicious eye is early, before pathological
changes commence, and the literature shows that, with the rarest exceptions
(Dor, 1931; A. Fuchs, 1932; Joy, 1935), enucleation within 14 days after
the injury protects the second eye against the onset of sympathetic disease.
If there is any doubt, this should be done. Although it seems peculiarly
effective in the rarer type of posterior disease, enucleation after this period
cannot be regarded as an absolute preventive, since the trouble may have
already started. Thus Schieck (1918) gathered 80 cases in the literature
in which sympathetic ophthalmitis followed enucleation, and the subsequent
literature contains further cases (Verhoeff, 1927, 3 cases or 8.6% ; Joy,
1935, 6 cases or 14.6% ; Woods, 1936, 4 cases or 14.3%). Usually in such
cases the disease starts within 2 weeks in the remaining eye, during which
time a real danger therefore exists ; but the onset has occasionally been
delayed for longer (7 months, although the injured eye was enucleated
6 days after injury, A. Fuchs, 1932). It may be said that if definite signs
of disease are seen in the injured eye, the other is not safe. Once definite
signs of disease have started in the second eye the question of enucleation
of the injured eye becomes more debatable, and many consider that in this
event enucleation is valueless or even inadvisable, since the exciting eye
DISEASES OF THE UWEAL TRACT 2351
may ultimately have the better vision of the two. Quite a number of cases
have been reported wherein useful vision has resulted in both eyes (Gifford,
1920; Dor, 1931, and others), and it has even occurred that the sympa-
thizing eye has to be enucleated because of pain while the injured eye
retained good vision (Wood, 1930; Davids, 1931). Certainly in advanced
cases it is usually inadvisable to remove the exciting eye; but in incipient
cases there is much evidence to show that rapid enucleation, even if some
considerable time has elapsed since the injury, results in a more favourable
course than the average. On the other hand there are reports of enucleations
which have been done immediately after suspicious signs had been observed
in which pathological examination showed only slight and early infiltration
of the exciting eye, and yet have been followed by complete blindness in the
other eye (Samuels, 1936). No case of sympathetic disease runs to order,
and the point is difficult to argue since there is no means of knowing how the
disease would have progressed otherwise ; but enucleation is probably
indicated in the first few days after symptoms have appeared in the second
eye, and certainly and at any time if the injured eye is blind or practically
useless.
Treatment
In a disease the aetiology of which is unknown and the course so
remorseless, it is understandable that innumerable therapeutic methods have
been attempted. So far as localtreatmentis concerned the most essential point
is to attain and maintain pupillary dilatation from the commencement in
an attempt to forestall the rapid development of complete synechiae. This
should be done by every possible means," but apart from this, other methods
of local treatment—heat, sub-conjunctival injections, and so on—are of
little value. -
The treatment of the common complication of increased tension is
difficult. Atropine should be stopped, for the raised tension is usually due
to embarrassment of the filtration angle ; and miotics encourage further
synechiae. Operative interference is usually unsuccessful, and if not, is
almost invariably of very temporary value. Probably the safest method
is to attack the only tissue in the eye unaffected by disease and perform
repeated paracenteses through the cornea, a procedure more legitimate in
view of the fact that hypertension is usually temporary, and hypotony the
usual end-result.
Innumerable general methods of treatment have been introduced, and
for each of them good results have been claimed. With regard to drugs, the
early method was by intensive mercury treatment by inunction to the point
of salivation, and it undoubtedly produced a percentage of successes
(Nettleship, 1886), and is still used with effect (de Grósz, 1926). In more
p. 2208.
T.O. —WOL. III. - S
2352 TEXT-BOOK OF OPHTHALMOLOGY
recent times the two drugs which have yielded the most favourable
results have been salicylates in massive doses (Gifford, 1900–14) or arsenicals
(Gifford, 1908; Jones and Browning, 1911; Browning, 1926; Hepburn,
1926). Of the two, salicylates, if given in large enough doses, are probably
the better.
Of the other heavy metals silver has been the most popular. Following the
suggestion of da Gama Pinto (1906), electro-colloids (Elecktrargol, Elektrokuprol,
Elektroselenium) by intravenous injection were used considerably on the Continent
in the Great War (Schlösser, 1914; Herrmann, 1916; Schieck, 1916; Wessely, 1917;
IHentschell, 1923; Villard, 1928).
Non-specific therapy has been advocated, particularly diphtheria anti-
toxin by Verhoeff (1924–27) : this must be given in large doses after the
sensitivity has been tested and desensitization carried out if necessary, an
average being 20,000 units daily until improvement or anaphylactic reactions
occur. Milk injections have also been advocated (Heine, 1923; Fradkine,
1924), typhoid vaccine (Shannon, 1937), and auto-serum (Guiral and Guiral,
1923–30). Inoculation with malaria has been tried (A. Fuchs, 1932),
and rapid improvement has been noted after the intervention of an acute
attack of scarlet fever (Undelt, 1926), influenza (Sexe, 1931) or malaria
(Montéaunu, 1938). -
Specific therapy may be said to have been tentatively introduced by Dor (1897) who
advocated the sub-conjunctival injections of an extract of ciliary body, and zur Nedden
(1905) who attempted to employ immune sera. The most important suggestion in this
direction, however, arose from Woods (1925–33) who employed extracts of uveal
pigment to desensitize patients who showed evidence of hypersensitivity, and then to
stimulate anti-bodies by active immunization. That an increase in opsonic index to
uveal pigment occurs has been demonstrated (Henton, 1937). In Woods’ hands some
degree of success appears to have been achieved.
On the supposition that sympathetic ophthalmitis was a form of tuberculosis,
tuberculin has been widely employed in its treatment (Bernheimer, 1911; Wood, 1918;
Nakamura, 1932; and others), but the results are by no means uniform : Meller (1921),
and Nakamura and Uchida (1937) prescribe it prophylactically.
Operative procedures to improve vision should be postponed until the
eye has been quiet for at least a year and should be undertaken only then if
they are necessary, for operative interference has a strong tendency to light
up the inflammation, not usually as an acute exacerbation, but by starting
a slow plastic process which gradually obliterates any good which may have
been done. When the eye has been quiet for a long time an optical iridec-
tomy may do good in milder cases, but it is frequently a difficult technical
procedure owing to the friability of the tissue, and too often the coloboma
is filled by exudate at a later date ; and an extraction of the lens can only
be justifiably undertaken when the patient has little to lose in the event of
an unfortunate result. At the same time, after a proper interval, heroic
procedures can be tolerated sometimes with success—as an iridectomy
DISEASES OF THE UVEAL TRACT
2353
(which was a failure), followed by an extraction (after which the pupil
closed), followed by an iridotomy (which remained patent with fair vision)
(Fisher, 1933).
For résumés of the enormous literature see Schirmer (1900), Parsons (1908),
Peters (1919) and Gifford (1920).
Abe. A. f. O., czvii, 375, 1926.
Alt. A. f. Aug., vii, 310, 1877.
Andersson. Acta O., xvi, 119, 1938.
Angelucci. A. di Ott., iv, 75, 1896.
Arkhangelsky. Sov. vest. Oft., vii, 737, 1935.
Arnold. A. f. path. Anat., czziv, 3, 1891.
Asayama. A. f. O., liv, 444, 1902.
Axenfeld. K. M. Aug., xlvii (1), Beil., 113,
1909.
Aykia. Nippon Gank. Zass., 1920: Ref. K. M.
Aug., lxvi (1), 951, 1921.
Ayres and Alt. Am. J. O., iv, 29, 1887.
Bartisch. Ogbóo. AplošovXeto., Dresden, 1583.
Bäuerlein. Bericht i. d. 157. Wirksamkeit usw.
Awgenkl. in Würzburg, 1884.
Becker. A. f. Psy. w. Newr, xii, 250, 1882.
Die Universitäts Avgenkl. in Heidelberg,
Wiesbaden, 1888.
Berens et alia. Atlantic Med. J., xxx,
1927.
Berlin. A. f. Psy. w. Newr., xi (1), 273,
1880
547,
Berliner and Nonidez. A. of O., viii, 695,
1932.
Bernheimer. A. f. Awg., lxx, 1, 1911.
Blascheck. Z. f. Aug., ix, 434, 1903.
Bock. K. M. Aug., xxviii, 484, 1890.
Bouchéron. Gaz. méd. de Paris, v, 442, 1876.
C. R. S. Biol., lxxxix, 647, 1879.
Brailey. Internat. Cong. Med., London, 35,
1881.
T. O. S., iv, 87, 1884.
Bräutigam. Diss., Rostock, 1912.
Brav. Ophthalmology, viii, 492, 1912.
Brown. A. of O., xxxvi, 173, 1907.
Browning. T. O. S., xlvi, 249, 1926.
Brückner. Z. f. Aug., xxxviii, 139, 1917.
Bunge. Diss., Halle, 1880.
Eutler. T. O. S., xlvi, 261, 1926.
Brit. J. O., xi, 230, 1927.
Cephalis. Anthology, xi, 352, 1000.
Cohn. Dreissig Jahre augenörz. Tätigkeit,
Breslau, 1897.
Collins. T. O. S., xxxiv, 18, 1914.
Cramer. Abriss d. Unfall-. w. Invaliditäts-
kunde des Sehapparates. Stuttgart,
1912.
Critchett. B. O. G. Heidel., i, 440, 1863.
Cross. T. O. S., vii, 149, 1887; xvii, 300,
1897.
Dalén. Mitt. Augenkl. Carol. med.-chir.
Instit., Stockholm, vi, 1, 1904.
Damel. A. de Oft. B. A., vii, 133, 1932.
Daniels. Z. f. Aug., lxxiv, 146, 1931.
K. M. Aug., lxxxvi, 526, 1931.
Davids. A. f. Awg., cv, 13, 1931.
Delaney. A. of O., v, 781, 1931.
Demours. Traité d. Maladies d. Yewa, Paris,
1818. g
Deutschmann. A. f. O., xxviii (2), 291, 1882;
xxix (1), 292, 323; (4), 261, 1883;
xxx (3), 77, 331, 1884; xxxi (2), 277,
1885; czix, 347, 1927.
Ophthalmia Migratoria, Leipzig, 1889.
Beit. z. Aug., i, 771; ii, 104, 1893-95.
Cb. pr. Aug., xxiii, 110, 1899.
Dimmer. K. M. Aug., lvii, 257, 1916.
Domann. Diss., Leipzig, 1912.
Dor. Prov. Méd. Lyons, xi, 265, 1897.
An. d’Oc., czviii, 49, 1897.
A. d’O., xlviii, 811, 1931. .
Le Dran. Traité en Refleasions tirées de la
Pratique sur les Plaies d’Armes de Few,
Amsterdam, 1696.
Duddell. Treat. of the Horny Coat of the Eye,
London, 1729.
Echevarria. Z. f. Aug., xlviii, 203; xlix,
100, 1922.
Ellett. T. Am. Acad. Oph-Oto-Lar., xxvii, 203,
1922.
Elschnig. A. f. O., lxxv, 459 ; Ixxvi, 509,
1910 ; lxxviii, 549; lxxix, 428, 1911.
Eversbusch and Pemerl. A. f. Aug., xiii, 396,
1884.
Fehr. Cb. pr. Aug., xxiv, 240, 1900.
Felsenthal. An. d’Oc., clxxi, 944, 1934.
Fisher. Brit. J. O., xvii, 35, 1933.
Fliri. Z. f. Awg., lv., 27, 1925.
Fodor. K. M. Aug., lxxix, 759, 1927.
Fradkine. La Clin. Opht., xiii, 322, 1924.
Friedenwald. Am. J. O., xvii, 1008, 1934.
Fuchs, A. Z. f. Aug., lvi, 275, 1924.
A. de Oft., B. A., vii, 67, 1932.
Fuchs, E. A. f. O., lxi, 365, 1905; lxx, 465,
1909; czv, 584; cvi, 168, 1925.
‘Gifford, H. A. f. Aug., xvii, 14, 1886.
A. of O., xvii, 362, 1888.
J. Amer. Med. As., xxxiv, 341, 1900.
O. Rec., xvii, 129, 584, 1908 ; xxiii, 64,
349, 1914.
R. M. Aug., xlviii (I), 588, 1910.
Amer. Encyc. Oph., Chicago, xvi, 12369,
1920.
Gifford, S. R.
1928.
Gifford, S. R., and Lucic.
Med. As., 20, 1926.
A. of O., i, 468, 1929.
Gilbert. A. f. O., lxxvii, 199, 1910.
Gill. South. Med. J., xxiii, 885, 1930.
Goldzieher. K. M. Aug., xv, 405, 1877.
A. f. path. Anat., ccxiii, 335, 1913.
Nebraska Med. J., xiv, 432,
T. Sect. O., Am.
S 2
2354
TEXT-BOOK OF OPHTHALMOLOGY
v. Graefe. A. f. O., ix. (3), 199, 1863 ; xii (2),
149, 1866.
Greeff. A. f. Aug., xxvi, 274, 1893.
Groenouw. K. M. Aug., xevi, 742, 1936.
de Grósz. T. O. S., xlvi, 271, 1926.
Grunert. K. M. Aug., xxxviii, Beil., 1, 1900.
Grüter. A. f. Awg., xcv, 180, 1925.
Guillery. K. M. Aug., xlix (2), 49, 1911.
A. f. Aug., lxviii, 242, 1911 ; likxii, 99,
1912; lxxiv, 132, 1913; xciv, 143,
1924; cir, 139, 474, 1935.
Z. f. Tuber., xxxviii, 1, 1923.
Münch. med. W., lxxii, 298, 1925.
Guiral and Guiral. A. de Oft. H.-A., xxiii,
563, 1923; xxx, 1, 1930.
La Clin. Opht., xii, 665, 1923.
Gunn. R. L. O. H. Rep., xi, 78, 1886.
Harman. West London Med. J., xx, 114, 1915.
Heine. K. M. Aug., lxxi, 70, 1923.
Henton. A. of O., xvii, 113, 1937.
Hentschel. K. M. Aug., lxx, 403; lxxi, 434,
I923.
Hepburn. T. O. S., xlvi, 242, 1926.
Herrmann. Diss., Rostock, 1916.
v. Hippel. A. f. O., xcii, 421, 1917.
B. O. G. Heidel., l, 144, 1934.
Hirschberg. Klin. Beobachtungen,
35, 1874.
A. f. Aug., viii, 55, 1879.
Hobby. O. Rev., vi, 238, 1887.
Hussels. K. M. Aug., liii, 221, 1914.
Iga. K. M. Aug., lxxxiii, 195, 1929.
Ihumi. Nipp. Gank. Zas., March, 1910:
Ref. K. M. Aug., xlix (1), 120, 1911.
Jäger. K. M. Aug., lxxviii, 613, 1927.
Jesner. A. f. d. g. Phys., xxiii, 14, 1880.
Jessop. T. O. S., xxxv, 1, 1915.
Jones and Browning. Brit. Med. J., i, 1426,
1911.
Joy. A. of O., xiv, 733, 1935;
1937.
Kitamura. K. M. Aug., xlv. (2), 211, 1907.
Kiyosawa. Acta S. O. Jap., xxxiv, 151, 1930:
xxxv, 102, 1931. -
Knapp. A. f. Aug., ii, 133, 1871.
Rºrailsheimer. Deut. med. W., xl, 323, 1914.
Rrause. A. f. Aug., x, Erg., 629, 1881.
Krug and Rohdenburg. A. f. O., viii, 72,
1932.
Lamb. A. of O., vii, 97, 1932.
de Lapersonne. A. d’O., xxxv, 129, 1916.
Lawson, G. R. L. O. H. Rep., vi, 123, 1868.
Leber. A. f. O., xxvii (1), 325, 1881; lviii (2),
324, 1904.
Lenz. K. M. Aug., xlv. (2), Beil., 229, 1907.
Lindemann. K. M. Aug., lxxiv, 775, 1925.
Löwenstein. Münch. med. W., lxxviii, 261,
1931.
Lugue. A. de Oft., H.-A., xxxvi, 90, 1936.
Mackenzie. Treatise on Diseases of the Eye,
II,’ 1835. -
Mansilla. A. de Oft., H.-A., xiii, 194, 1913.
Marchesani. Z. f. Aug., lvii, 44, 1925.
B. O. G. Heidel., xlv., 107, 1925; xlvii,
309, 326, 1928.
A. f. Aug., xcvii, 575, 1926.
Wien,
xvii, 677,
XIII Internat. Cong. Oph., Amsterdam,
ii, 531, 1929.
Mariani. A. f. Derm. Syph., czlvii, 259, 1924.
Mazza. A. di Ott., xvi, 17, 1887.
Meesmann and Volmer. A. f. Aug., xcviii,
271, 1927.
Melanowski. K. M. Aug., xcvii, 52, 1936.
Meller. Z. f. Aug., xxx, 246, 379, 1913;
lxxix, 95, 110, 1932; lxxxvii, 239, 1935;
lxxxix, l, 1936.
A. f. O., lxxii, 167, 1909; lxxxviii, 282;
lxxxix, 39, 248, 427, 437, 1914-15; cii,
122, 1920; cv, 299, 1921.
K. M. Aug., lii (1), 1, 1914.
An. of O., xxiv, 217, 1915.
Wien. kl. W., xlv, 33, 1932.
T. O. S., liv, 467, 1934.
Meyer. B. O. G. Heidel., xx, 104, 1891.
Milles. R. L. O. H. Rep., x (3), 325, 1882.
Montéaunu. Acta méd. Rom., v.-vi., 405, 1938.
Mooren. Ueber Sympath. Gesichtsstörungen,
Berlin, 1869.
Morax. An. d’Oc., cliv, 363, 426, 1917;
clxix, 637, 1932.
Müller, H. A. f. O., ii (1), 368, 1858.
Nagel. Calif. State Med. J., xiii, 227, 1915.
Nakamura. K. M. Aug., lxxxix, 43, 1932.
Acta S. O. Japan, xxxviii, 69, 1934.
Nakamura and Uchida. A. f. O., czzxvii,
233, 1937.
Nance. O. Rec., xix, 331, 1910.
zur Nedden. A. f. O., xlii, 193, 1903, 1905.
Nettleship. T. O. S., iv, 76, 83, 85, 1884.
Nettleship et alia. T. O. S., vi, 170, 1886.
Nieden. A. f. Aug., xxix, 339, 1894.
Nordenson. Cb. pr. Aug., xii, 20, 1888.
Oguchi. Beit. z. Aug., ix, 75, 1913.
Acta S. O. Japan, xxxviii, 98, 1934.
Ohlemann. A. f. Awg., xxii, 94, 1890.
Pagenstecher. K. M. Aug., xi, 123, 1873.
Panas. Gaz. de Hôp. Paris, li, 673, 1878.
Parsons. Path. of the Eye, London, iv., 1229,
1908.
Peters. G.-S. Hb., III, Die Sympath. Augen-
kramkung, 1919.
Klin. W., iv., 1529, 1925.
Pflüger. Korrespondenz-Bl. f. Schw. Aerzte,
xxvi, 3, 1896.
Pincus. A. f. O., x1 (4), 231, 1894.
Pinto, da Gama. Affections sympathiques,
Enc. fr. d’O., v, 1906.
Pollot. A. f. O., lxxxi, 264, 1912.
Poos and Sartorius. A. f. O., czziv, 565, 1930.
Post. South. Med. J., xxvii, 421, 1934.
Prichard. Provincial Med. dº Surg. J., xv, 66,
1851.
Raehlmann. Deut. med. W., xxx, 449, 1904.
Randolph. A. f. Aug., xxi, 159, 1890.
A. of O., xxi, 367, 1892.
Redslob. Am. d’Oc., clviii, 659, 1921.
Reis. B. O. G. Heidel.., xxxiv, 313, 1907.
A. f. O., lxxx, 69, 1911.
Kurzes Hb. der O., Berlin, iv, 590, 1931.
Ribstein. Diss., Strassburg, 1915.
Riehm. K. M. Aug., lxxxi, 867, 1928; xc,
477, 1933. *.
DISEASES OF THE UVEAL TRACT
2355
Deut. med. W., lv, 907, 1134, 1929.
A. f. O., czziii, 361, 1930.
Risley. J. Am. Med. As., iv, 43, 1885.
Rockliffe. T. O. S., xxvii, 88, 1907.
Ruge. A. f. O., lvii, 401, 1904.
Samuels. A. of O., ix, 540 ; x, 185, 1933;
xv, 59, 1936; xvii, 1031, 1937; xx, 804,
1938.
Sander. Brit. J. O., xv, 25, 1931.
Schenkl. A. f. Derm. Syph., v, 137, 1873.
Schieck. K. M. Aug., xxix., 196, 1912.
B. O. G. Heidel., x1, 160, 1916.
A. f. O., xcv, 322, 1918.
Schirmer. A. f. O., xxxviii (4), 95, 1892.
G.-S. Hb. II, vi (2), 37, 1900.
Schlösser. Münch. med. W., xli (2), 2357,
1914.
Schmidt-Rimpler. A. f. O., xxxviii (1), 199,
Deut. med. W., xxvi, 429, 451, 1900.
Schönenberger. A. f. O., czkv, 29, 1930.
Schreiber. B. O. G. Heidel.., xlviii, 334, 1930.
A. f. O., cxxix, 127, 1932.
Schwartz. Am. J. O., x, 35, 1927.
Sexe. Bull. S. fr. d’O., xliv, 353, 1931.
Shannon. A. of O., xvii, 186, 1937.
Snellen. T. Internat. Med. Cong., London,
31, 1881.
Ned. tij. v. Gen., ii, 1211, 1910.
Soriano. A. de Oft. B. A., iv, 557, 1929.
Steindorff. Beit. z. Aug., Fest. Hirschberg,
277, 1905.
Sulzer. An. d’Oc., czzxvii, 141, 1907.
Swab. Am. J. O., xi, 461, 1928.
v. Szily. K. M. Aug., lxxii, 593, 1924;
lxxviii, Beil., 11, 1927; xciii, 145, 1934.
Deut. med. W., lii, 1598, 1926.
Tay, Waren. T. O. S., xii, 29, 1892.
Theobald. Am. J. O., xiii, 597, 1930.
Thomson. An. of O., xvii, 641, 1909.
Trousseau. An. d’Oc., cxxi, 185, 1899.
Trowbridge. Am. J. O., xx, 135, 1937.
Uhthoff. A. f. O., xxix (2), 167, 1883.
Undelt. K. M. Aug., lxxvi, 825, 1926.
lurbanek. T. O. S., lii, 227, 1932.
Velhagen. A. f. O., czix, 325, 1927.
Verhoeff. A. of O., liii, 517, 1924; lvi, 28,
1927.
Verhoeff and Irvine.
xxxvi, 63, 1936.
Vignaux. De d’Ophtalmie
Paris, 1877.
Villard. A. d’O., xlv., 490, 1928; xlviii, 197,
1931.
Vogt. K. M. Aug., xliv (1), 228, 1906.
Atlas, Berlin, 1921.
Wagenmann. A. f. O., lxxiv, 489, 1910.
Waldmann. A. f. Aug., ciz, 441, 1935.
Wardrop. Morbid Anat. of the Human Eye,
London, 1819.
Weekers. An. d’Oc., cliv, 196, 1917.
Weeks. N.Y. Eye and Ear Infirmary, ii, 30,
1894.
Weigelin. A. f. O., lxxv, 411, 1910.
Wessely. A. f. O., l, 123, 1900.
Münch. med. W., 1, 1621, 1917.
Widmark. Mit. a. d. Augenkl. d. Carol. med.-
chºir. Inst., Stockholm, ix, 111, 1908.
Wood, D. J. Brit. J. O., xi, 217, 1927; xiv,
291, 1930.
Wood, Russ. Brit. J. O., ii, 27, 1918.
Woods. A. of O., xlv, 557, 1916; xlvi, 8,
283, 503, 1917; xlvii, 161, 1918; ix, 200,
1933.
J. Am. Med. As., lxxvii, 1317, 1921.
T. O. S., xlv., 208, 1925.
Am. J. O., xix, 9, 100, 1936.
Woods and Little. A. of O., ix, 200, 1933.
Zimmermann. A. f. O., xlii (2), 39, 1895.
Zorab. Ophthalmoscope, xiii, 597, 1915.
N. Y. State Med. J.,
sympathique,
Zuntz. Diss., Marburg, 1915.
2. UVEO-PAROTITIS
UVEO-PAROTITIS (UVEO-PAROTID FEVER : HEERFORDT’s DISEASE) is a
well-defined clinical and pathological entity of unknown aetiology charac-
terized by a chronic bilateral parotitis and uveitis, often associated with paresis
of the cranial nerves and other general symptoms, and having a tendency towards
spontaneous resolution.
The syndrome was first defined as a clinical entity by Heerfordt (1909) (febris
wvea-parotidea sub-chronica), who considered it an atypical form of mumps, calling
attention to three similar reports in the literature (Daireaux, 1899; Péchin, 1901 ;
Collomb, 1903). Since then about 100 cases have appeared of which Merrill and
Oakes (1931) reviewed 30, Savin (1934) 66, and Kruskal and Levitt (1935) 64.
The disease, which occurs characteristically in young people usually
between 10 and 30 years of age (the extremes in the literature are 6 and
65 years), may start with a prodromal period of malaise, sometimes with a
2356 TEXT-BOOK OF OPHTHALMOLOGY
gastric upset, and usually slight fever. Thereafter the characteristic
features are a painless chronic swelling of the parotids which resolves
without suppuration, a generalized uveitis, frequently of a nodular type,
tending to the formation of synechiae and vitreous opacities, and a paresis
of some of the cranial nerves, usually the seventh. Other occasional ocular
symptoms include keratitis, optic-neuritis, cataract and glaucoma, while
general symptoms, such as skin rashes, erythema nodosum, enlargement of
lymph glands, rheumatic pains, dryness of the mouth and polyuria may be
present. The symptoms may last for a variable period, usually some
months, but the disease is ultimately self-limiting and the only permanent
disability is the resulting visual impairment which may occasionally entail
blindness. Several of the reported cases ended fatally, and in each instance
necropsy disclosed a widespread miliary tuberculosis of a sub-acute type.
The important symptoms may appear in almost any order. In about
half the published cases parotid swelling, and in the other half uveitis is
the initial occurrence ; rarely the syndrome is ushered in by facial paralysis
or skin lesions. The interval between the uveitis and the parotitis is usually
short, but a period of 4 years is recorded (Foster, 1938). The parotid
swellings, which are interstitial in type, have lasted from 6 weeks to 2 years,
and they may be of enormous size. It is to be noted that in all the patho-
logical reports in the literature there was histological evidence of tubercle
(Reimers, 1926; Dorrell, 1932; Kruskal and Levitt, 1935). The sub-
maxillary glands are occasionally enlarged (6 cases), and also the lacrimal
(9 cases).
The nervous lesions are various and are best explained as a widespread
peripheral polyneuritis, although the cerebro-spinal axis seems to have been
occasionally involved. Facial paralysis is the commonest manifestation,
and that it is not due to strangulation by the swollen parotid is shown by
the fact that it may appear after the gland has subsided (Rieth, 1919), and
that it is sometimes accompanied by an abolition of taste (Daireaux, 1899 ;
Mohn, 1933), indicating an affection above the origin of the chorda tympani.
Palatal paralysis, ptosis, diplopia, recurrent laryngeal paralysis, vagal
paralysis and a spastic condition of the lower extremities with extensor
plantar reflexes have been occasionally noted : so also have absence of the
knee and ankle-jerks, ataxia, deafness, numbness and parasthesias.
Joints (knees, ankles, wrists) have been swollen in some 10% of cases :
lymphatic glands (cervical, pre-auricular, epitrochlear, mediastinal, axillary
and inguinal) in 20% ; enlargement of the spleen (3 cases) or Orchitis
(2 cases) is exceptional.
When they have been examined, urine, blood and cerebro-spinal fluid
have usually been normal. Indeed, the only positive finding is histological
evidence of tubercle, and in the four autopsies which have been published it
has been uniformly present. Pathological examinations of the eyes have been
few, but they have shown somewhat indefinite nodular accumulations with
DISEASES OF THE UVEAL TRACT 2357
giant cells but without caseation (Lehmann, 1916; Ohno, 1920; Bahr,
1938).
There is no specific treatment known ; and cases should be dealt
with symptomatically on general principles.”
Aºtiology
Most of the discussion in the literature has been concerned with the
aetiology. The theory of mumps as an aetiological factor can be ruled out
since many cases have had this disease previously and it confers an immunity,
since the facial paresis sometimes precedes parotid involvement, and since
there is no evidence of its contagiousness or its occurrence in association
with epidemic parotitis. The majority of authors have inclined towards
a tuberculous aetiology (Lehmann, 1916; Gjessing, 1916; Thomsen,
1919; Schall, 1923; Cavara, 1928; Souter, 1929; Mohn, 1933; Garland
and Thompson, 1933–34 ; Igersheimer, 1934; Davies, 1934; Tanner
and McCurry, 1934; Haferkorn, 1935; Cogan, 1935; Kruskal and
Levitt, 1935; Nowkirischky, 1935; and others). The evidence for this is
the nodular appearance of the iritis in many cases, the frequent signs of
tubercle elsewhere in the body or the development of tubercle subsequently,
the histological appearance in the iris and the parotid of tubercle-like systems,
the demonstration of the tubercle bacillus in the parotid on biopsy (Tanner
and McCurry, 1934) or in a sub-maxillary gland (v. d. Hoeve, 1927), and the
finding of generalized tuberculosis in the few cases which have come to
necropsy. For this reason the name UVEO-PAROTID TUBERCULOSIS has been
suggested (Garland and Thompson, 1933). As against this, however, may
be cited the fact that evidence of tubercle elsewhere is sometimes absent
(Doyne, 1937; and others), the constant absence of caseation in the parotid
which is unusual in tubercle, and the usually benign and favourable
course and prognosis of the disease. Moreover, the involvement of the
nervous system, the gastric and skin manifestations, and the other systemic
features of the disease bear a very slight resemblance indeed to systemic
tuberculosis, and are almost impossible to reconcile with meningeal or
cerebral tuberculosis. Finally, skin sensitivity to tuberculin has been
negative more often than positive in those cases wherein it has been reported
(11 out of 17 cases, Berg, 1931). The high incidence of tubercle is, of course,
significant and must weigh heavily in an assessment of the question, but it
is not conclusive proof and might be explained by a lighting up of a latent
tuberculosis by an intercurrent infection (Savin, 1934). Weve (1918–26)
suggested that a toxic agent is involved, which, from the tubercle-like
histological findings, might be called para-tuberculosis. Bang (1918),
on the other hand, considered the syndrome a variant of Mikulicz’s
disease, and pseudo-leucaemic in nature, and Torben (1937), a benign
lymphogranulomatosis. In most cases, however, this can be ruled out
1 p. 2208.
2358 TEXT-BOOK OF OPHTHALMoLOGY
(Kaitz, 1931; and others), as also can syphilis and other known infections.
The only rational course to adopt in the present state of our knowledge is
to admit ignorance, and while remembering the very strong possibility of a
benign and unusual type of systemic tuberculosis, to consider the syndrome
as the effect of some unknown toxin affecting particularly the parotid and
uveal tissue, with a tendency to cause a peripheral neuritis and skin
lesions (Feiling and Viner, 1922; MacBride, 1923; Atland, 1924; Critchley
and Phillips, 1924; Parker, 1926; Hamburger and Schaffer, 1928; Merrill
and Oaks, 1931; Tait, 1934; Cohen and Rabinowitz, 1935; Folger, 1936;
and others).
Atland. Z. f. Aug., liii, 113, 1924.
Bahr. Acta O., xvi, 101, 1938.
Raitz. Brit. J. O., xv, 36, 1931.
Kruskal and Levitt. Am. J. O., xviii, 735,
Bang. Ugesk. f. Laeger, lxxx, 571, 1918.
Berg. Hygiea, lxxxv, 401, 1923.
A. d’O., xlviii, 568, 1931.
Brewerton. T. O. S., xxx, 148, 1910.
Cavara. Boll. d’Oc., vii, 925, 1928.
Cogan. Am. J. O., xviii, 637, 1935.
Cohen and Rabinowitz. J. Am. Med. As., cv,
497, 1935.
Collomb. Rev. Méd. de la Swisse Rom., xxiii,
43, 1903.
Coppez. Bull. S. Belge d’O., vi, 118, 1922.
Critchley and Phillips. Lancet, ii, 906, 1924.
Daireaux. Bull. med. Paris, xiii, 227, 1899.
Davies. Lancet, ii, 746, 1934.
Dorrell. Roy. Berkshire Hosp. Rep., i, 91,
1932.
Doyne. P. R. S. Med., xxx, 947, 1937.
Feiling and Viner. J. Neurol. and Psycho-
path., ii, 353, 1922.
Folger. A. of O., xv, 1098, 1936.
Foster. T. O. S., lviii, 433, 1938.
Garland and Thompson. Quart. J. Med., ii,
157, 1933.
Lancet, ii, 743, 1934.
Gjessing. K. M. Aug., lvi, 252, 1916; lx,
249, 1918.
Haferkorn. M. f. Kinderh., lxiii, 161, 1935.
Hamburger and Schaffer. Am. J. Dis. Child.,
xxxvi, 434, 1928.
Heerfordt. A. f. O., lxx, 254, 1909.
v. d. Hoeve. K. M. Aug., lxxix, 672, 1927.
1935.
Lehmann. Hospitaltid., lix, 117, 137, 1916.
MacBride. J. Newrol. and Psychopath., iv, 242,
1923.
Mackay. Brit. J. O., i, 612, 1917.
T. O. S., xxxvii, 208, 1917; xli., 209, 1921.
McCulloch. T. O. S., xlvii, 410, 1927.
Merrill and, Oaks. Am. J. O., xiv, 15, 1931.
Mohn. Acta O., xi, 397, 1933.
Muirhead. T. O. S., lviii, 435, 1938.
Müller. K. M. Aug., lxiv, 387, 1920.
Nowkirischky. K. M. Awg., xev, 620, 1935.
Ohno. K. M. Aug., lxv, 155, 1920.
Parker. Bristol Med. Chir. J., xliii, 73, 1926.
An. Int. Med., x, 92, 1936.
Péchin. Rev. gén. d’O., xx, 445, 1901.
Ramsay. T. O. S., xli., 194, 1921.
Reimers. Z. f. Aug., lx, 30, 1926.
Rieth. K. M. Aug., lxiii (2), 527, 1919.
Savin. T. O. S., liv, 549, 1934.
Schall. K. M. Awg., lxx, 350, 1923.
Souter. T. O. S., xlix, 113, 1929.
Tait. Lancet, ii, 748, 1934.
Tanner and McCurry. Brit. Med. J., ii, 1041,
1934.
Thomsen. Ugesk. f. Laeger, lxxxi, 1517, 1919.
Torben. Acta O., xv., 104, 1937.
Toulant and Morard. A. d’O., liii, 321, 1936.
Weve. Ned. tij. v. Gen., i, 544, 1918.
Z. f. Aug., lx, 68, 1926.
K. M. Aug., lxxviii, 83, 1927.
Igersheimer. A. of O., xi, 119, 1934.
3. SARCOID OF BOECK
This disease (BENIGN MILIARY LUPOID : MULTIPLE SARCOID OF BOECK,
1899) is characterized by the formation of multiple nodules in the skin and
subcutaneous tissues, as well as in bones, glands and the viscera, having a
benign, symptomless and torpid course showing a progressive tendency
with spontaneous remissions, and usually terminating favourably with
resolution of the lesions into scar-like areas. We have seen that it may
attack the conjunctiva and the external eye 1 ; as a rare event it may
involve the uveal tract.
* Vol. II, p. 1637.
DISEASES OF THE UVEAL TRACT 2359
Cases of iritis are not common, and since the first recorded cases by Schumacher
(1909) and Bering (1910) some 30 cases have been reported, but since many of them
occur in dermatological literature the ocular records have frequently been vague. No
single observer has seen many of them, although Bloch (1915) described 5. It may
probably be taken that it occurs in between 5 to 10% of cases (Osterberg, 1939).
Clinically the iritis closely resembles a tuberculous condition, and is with
few exceptions bilateral. It is a slow and painless inflammation with a
tendency to the formation of multiple nodules which may reach considerable
dimensions (Bering, 1910; Favauge-Bruyel, 1922; Nordin, 1937; Oster-
berg, 1939). There is little tissue-reaction or tissue-necrosis and the disease
very frequently subsides with great rapidity spontaneously, even large
nodules disappearing to leave scarcely any scar. As a rare event there
occur periods of widespread dissemination of the nodules, to be followed by
long quiescent phases, the eye eventually becoming phthisical (Mylius,
1928; Walsh, 1939). Occasionally the posterior segment is affected also,
and whitish-yellow masses of histologically typical sarcoid structure may
be scattered over the fundus (Mylius and Schürmann, 1929; Walsh,
1939). Papilloedema, to be followed by optic atrophy, due to involvement
of the optic nerve, may also occur (Reis and Rothfeld, 1931).
The pathological picture of the nodular lesions is singularly uniform,
the chief features being an accumulation of epithelioid cells interspersed
with a few giant cells and surrounded by a sparse infiltration of lymphocytes,
but without necrosis or sclerosis : it is, in fact, the picture of benign
tubercle.
Morphologically an individual case cannot be distinguished from
tubercle, but Boeck's disease differs in being less painful, more torpid, less
destructive, and more proliferative than the former. The differential
diagnosis can only be made on the (usually) negative or faintly positive
cutaneous reaction to tuberculin, and after a review of the general condition,
noting especially the presence of similar lesions in the other organs most
usually affected.
The aetiology of the disease is unknown, but the great majority of authorities
consider it of a tuberculous nature but of benign character—either an atypical form (the
para-tuberculosis of Weve, 1926), or an attenuated form in which few organisms of
little virulence are associated with a slight degree of allergy and a high degree of
resistance (Pinner, 1938). The arguments in favour of such an aetiology are the
histological appearances and the frequent development of frank tuberculosis in many
of these patients together with a disappearance of the sarcoid lesions, the finding of
tubercle post-mortem in about half the necropsies, and the finding of an acid-fast
bacillus once in an early lesion (Kyrle, 1920). Many authors also associate it with
Heerfordt's disease,” itself, as we have seen, sometimes considered tuberculous, for
the parotid gland is frequently affected in multiple sarcoid (Walsh, 1939; and others):
The matter, however, can by no means be considered settled.
1 p. 2355.
2360
TEXT-BOOK OF OPHTHALMOLOGY
Bering. Derm. Z., xvii, 404, 1910.
Bloch. A. f. Derm. Syph., czix (1), 133, 1915.
Boeck. J. Cutan. and Genito-Urimary Dis.,
xvii, 543, 1899.
Norsk Mag. f. Laegev., xiv, 1321, 1899.
Favauge-Bruyel. Ned. tij. v. Gen., lxvi, 11,
1061, 1922.
Mylius and Schürmann. Beit. z. Klin. d. Tub ,
lxxiii, 166, 1929.
Nordin. Acta derm.-vener., xviii, 3, 245, 1937.
Osterberg. Brit. J. O., xxiii, 145, 1939.
Pinner. Am. Rev. Tub., xxxvii, 690, 1938.
Reis and Rothfeld. A. f. O., czzvi, 357, 1931.
Schumacher. Münch. med. W., ii, 2664, 1909.
Walsh. A. of O., xxi, 421, 1939.
Ryrle. A. f. Derm. Syph., czzv, 481, 1920.
- Weve. Z. f. Awg., lx, 68, 1926.
Mylius. Z. f. Aug., lxv, 71, 1928.
4. HETEROCHROMIC CYCLITIS
We have already touched upon this subject in a previous volume,”
when it was pointed out that two types of heterochromia existed—SIMPLE
HETEROCHROMLA, a hereditary condition affecting the development of the
iris and without pathological significance, and COMPLICATED HETERO-
CHROMIA, wherein the alteration in colour is due to pathological processes.
It is true that this view is not universally accepted, Lutz (1908–28), for
example, holding that all cases can be explained on the basis of an anomaly
in unilateral inheritance leading to an abiotrophy, but it seems the most
rational explanation of the facts. Again, although it is also disputed, we
will divide complicated heterochromia into three types : (1) an ATROPHIC
condition following post-traumatic or inflammatory states or the result of
senility—a group which will be considered under the heading of atrophies,”
(2) HETEROCHROMIC CYCLITIs, and (3) NEUROGENIC HETEROCHROMIA, wherein
the depigmentation follows a lesion of the sympathetic nerve, and, although
probably not an inflammation in the true sense, is most conveniently
considered here.
The history of heterochromia provides the easiest way to present the problem.
Although the phenomenon of a difference in colour between the two irides was known
to the ancients,” the first scientifically reported cases were those of Jonathan Hutchinson
(1869) who noted the occurrence of cataract in eyes which were of lighter colour than
their fellows. The occurrence of heterochromia with cyclitis and cataract was recorded
subsequently by several observers (Sym, 1889; Gunn, 1889; Malgat, 1889–99; and
others), until the coincidence became recognized, and eventually Weill (1904), corre-
lated the syndrome by suggesting that some circulatory disturbance in foetal life
interfered with the development of the uveal pigment and led to cyclitis and cataract.
Thereafter, as in so many diseases of the eye, a classical paper appeared by Fuchs
(1906) who, after an extensive study of 38 cases, concluded that the essential feature
in the syndrome was a cyclitis characterized by a change in the colour of the iris, the
absence of synechiae, the presence of precipitates, and the preservation of vision except
for the common development of a complicated cataract. Since the physiological
hardening of the lens nucleus did not occur, he concluded that the condition was due
to some unknown toxin acting in intra-uterine or infantile life. From that date
heterochromic cyclitis came to be regarded as a clinical entity and many subsequent
authors, following Heine (1912–23), concluded that infection was the sole origin of the
phenomenon and that the toxin concerned was tubercle. Some years previously, how-
ever, it had been shown that extirpation of the cervical sympathetic resulted in pig-
1 Vol. II, p. 1402. ? p. 2389. 8 Vol. II, p. 1402.
DISEASES OF THE UVEAL TRACT 2361
mentary deficiency of the iris of the same side (Angelucci, 1893; Bistis, 1898), a
phenomenon confirmed by Lutz (1908–10), and noted after a birth injury by Mayou
(1910); similar clinical cases rapidly appeared wherein decoloration of the iris was
associated on the same side with a typical Horner's syndrome of miosis, enophthalmos
and facial hemiatrophy (Galezowski, 1910; Bistis, 1912; Metzner and Wölfflin,
1914–16; Scalinci, 1915; Koeppe, 1918; and others), and eventually v. Herrenschwand
(1918) suggested that this type of heterochromia should be classed as a clinical entity.
This suggestion was enthusiastically taken up by Streiff (1919), Kraupa (1924), and
more especially by Bistis (1928) who held that heterochromic cyclitis did not exist, that
the cloudy lens and vitreous and the keratic precipitates were not cyclitic in Origin but
were the result of cellular deposits from dilated blood-vessels deprived of their sym-
pathetic control and the nutritional changes consequent thereon. Two schools of
thought thus arose, one that the clinical phenomena are due to mild infective cyclitis,
the other that they are neurogenic and nutritional in Origin, although to Some extent
they are reconciled by the admission of the second school that, although the phenomena
are primarily nutritional in origin, the state of the eye may allow the development of
infections so mild that normally they would be ineffective (Scalinci, 1915; Bistis,
1934). It seems probable, however, that both are right and that both factors, the toxic
and the neurogenic, are operative in different cases.
Complicated heterochromia is not an uncommon condition. Several
hundred cases are reported in the literature, and individual authors have
reported large numbers—38 cases, Fuchs (1906), 100, Lutz (1908–28), 90,
Heine (1912–23), 30, Szekely (1931), and so on. Butler (1911) reported
0.03% of patients in England, Lutz (1928) 0.2% in Switzerland, and Berg
(1924) 0.19% in Scandinavia. The average age of observation is between
20 and 35, the extremes being 13 and 64 years. The occurrence in males
is some 10% higher than females. It is almost invariably unilateral,
although bilateral cases have been reported, both eyes becoming light in
colour and showing cyclitic changes (Berg, 1925; Raia, 1931). It occurs
in pigmented races, in whom, however, it is not so obvious (e.g., Chinese, Lan-
Ting, 1932); and a heterochromic cyclitis may develop in the darker of two
eyes, the lighter remaining normal (Lloyd, 1931).
(a) HETEROCHROMIC CYCLITIs (OF FUCHS)
This type of heterochromia is relatively common (33 out of the 38 cases
of Fuchs, 1906) (Fig. 1957, Plate XLII). It is associated with signs of cyclitis
of an insidious origin and a very slow and chronic course which runs on for
many years without any of the ordinary objective signs of inflammation and
without subjective pain. The iris tissue presents a peculiar appearance : the
architecture of the trabeculae is lost, the relief of the anterior surface is
diminished, the radial markings on the pupillary zone are less prominent,
and at the pigment border the deep pigmentary layer appears defective and
moth-eaten in places, or may become ragged, coming away in tags. With
the slit-lamp the whole tissue shows a rarefaction and transparency entirely
different from the appearance after any other type of cyclitis; the impression
is given of looking through to the pigment epithelium which itself appears
2362 TEXT-BOOK OF OPHTHALMOLOGY
riddled with holes like a sieve. Frequently the cyclitis is associated with a
cataract which is not of a typically complicated appearance, but is charac-
terized initially by fine dust-like opacities in the posterior cortex frequently
accompanied by striae in the peripheral parts of the lens : this is rapidly
followed by a generalized diffuse opacity, until eventually the lens becomes
completely opaque. Precipitates are formed on the posterior corneal surface
with great constancy, and the endothelium has been noted to be bedeved
and oedematous : the precipitates are small, non-pigmented, non-confluent
and are easily overlooked, but sometimes they are absent, even in the
presence of cataract. The aqueous humour is sometimes clear and at other
times it shows a definite haze in the beam of the slit-lamp, and the vitreous
body is not uncommonly clouded with dust-like opacities. A peripheral
choroiditis has been noted, and a quiet and insidious type of glaucoma has
supervened in a considerable number of cases (Sym, 1889; Fuchs, 1906;
Rraupa, 1923; Lutz, 1928; 4 out of 13 cases, Gradle, 1929; Lloyd, 1931;
and many others).
Microscopic examination of the iris (Fuchs, 1906; Schlippe, 1910; Franke,
1917) has never shown very profound changes : there is a lack of chromatophores, a
connective tissue overgrowth in the stroma, a thickening of the vessel walls, and an
infiltration with plasma cells and lymphocytes with occasional eosinophils and mast
cells. Nests of epithelioid cells have been noted but these may be incidental to the
picture. The changes in the chromatophores give evidence rather of failure of develop-
ment than of Subsequent destruction, and consequently the unknown aetiological agent
postulated by Fuchs must act in intra-uterine or very early infantile life.
These changes proceed without showing any variation for many years.
If the visual acuity is affected by opacities in the lens, its extraction is easy
and the operative prognosis is good (Fuchs, 1906; Edgerton, 1933; and
many others): the most unfortunate occurrence is a rise of tension. The
whole character of the disease is so unlike any other cyclitis that it is small
wonder that many believe its origin to be vasomotor rather than toxic—
the absence of synechiae, the uniform diffuse involvement of the entire iris,
the early appearance and rapid development of cataract, the absence of
subjective symptoms, and the fact that in almost every case it is confined
to one eye. Fuchs, as has already been noted, hypothesized an unknown
toxin acting in early (or intra-uterine) life, a view which has considerable
support (Schlippe, 1910; Haessler, 1929; and others); while in the opinion
of several observers the aetiological factor is tuberculosis (Heine, 1923;
King, 1927; Szekely, 1931; Lloyd, 1931; and others).
(b) NEUROGENIC HETEROCHROMIA
The possibility of changes in colour in the iris following a sympathetic
lesion is founded on a sound experimental basis. After excision of the superior
cervical ganglion Angelucci (1893) produced a depigmentation in the uveal
tract of dogs and rabbits, Abelsdorff (1904) produced heterochromia in the cat,
DISEASES OF THE UVEAL TRACT 23.63
Calhoun in rabbits (1919), and more extended experiments were conducted by
Bistis (1913–28), Lazarescu (1933), Passow (1933–34) and others. Cases
ranking as human experiments were reported by Bistis (1928) wherein a man
aged 20 developed Horner's syndrome with heterochromia a year after a
surgical injury during a cervical operation, and by Lazarescu (1933) after a
bayonet wound in the neck. Cases have been reported after a similar injury
at birth (Mayou, 1910–16; Kranz, 1924) or in early or adult life (Lazarescu,
1933; Bistis, 1934), and many cases after pathological sympathetic paralyses.
In the majority of cases the lesion has been paralytic : an irritative lesion
has also been associated with the phenomenon (Galezowski, 1910), while
Streiff (1919) considered that an asymmetrical sympathetic tone is sufficient
cause. Some of this group of cases showed corneal precipitates and lens
changes, while in others the eye appeared otherwise normal. According
to Herrenschwand (1918–24) this type of heterochromia differs from the
cyclitic type in the fact that the intimate architecture and trabeculae of the
deeper layers of the iris remain definitely and clearly demarcated.
Although it is considered by some that the sympathetic plays no part
in the anomaly but that infective causes are invariably present, the frequent
occurrence of heterochromia with a typical Horner's syndrome is suggestive,
as also its occurrence with vasomotor disturbances in the nose (Muck,
1934), and appears conclusive when the experimental and traumatic
production of the condition is borne in mind.
It is to be remembered that a multiplicity of lesions in addition to trauma may
cause such a disturbance ; thus v. Herrenschwand (1918) that out of 13 cases 11 had
an enlarged thyroid. Giannantoni and Possente (1933) made the interesting observa-
tion correlating this neurogenic type with tuberculosis, that the depigmented iris was
frequently on the side of the most marked pulmonary lesions and might be accompanied
by a large pupil and longer lashes, pointing to sympathetic irritation. Passow (1933–34)
associated it with the “ Status dysraphicus' wherein many structural anomalies are
seen in the skeletal system, and considered the probable cause central in origin, such as
latent syringomyelia or syringobulbia.
It is supposed by some that the heterochromia is directly due to the
influence of the sympathetic on the chromatophores themselves *; but
the more general opinion is that the primary anomaly is vascular. The
interdependence of the development of pigment and blood-vessels has
already been referred to,” and it seems necessary to explain the keratic
precipitates and cataract on vaso-dilatatory and nutritional grounds unless
an additional toxin be hypothesized ; and it has been experimentally
demonstrated that section of the sympathetic causes a vaso-dilatation with
increased permeability of the capillaries in the eye. It is possible that in
some cases a hereditary element may be involved (Terrien, 1932); and it is
also possible that, on the basis of the nutritional disturbance, benign infec-
tions, to which a normal eye would not have succumbed, readily obtain a
footing and induce a cyclitis of an unusually mild type.
1 Vol. I, p. 378. * Vol. I, p. 376.
2364
TEXT-BOOK OF OPHTHALMOLOGY
Abelsdorff. A. f. O., lix, 376, 1904.
Angelucci. A. di Ott., i, 1, 1893.
Berg. K. M. Aug., lxxiii, 788, 1924.
Acta O., ii, 361, 1925.
Bistis. Cb. pr. Aug., xxii, 136, 1898.
A. d’O., xxxii, 578, 1912; xiv, 569, 1928.
A. f. Awg., lxxv, 302, 1913.
A. of O., xliv, 433, 1915. -
B. O. G. Heidel., xlv., 88, 1925; xlvi, 360,
- 1927. -
Z. f. Aug., lxxxii, 180, 1934.
Butler. Ophthalmoscope, ix, 501, 1911.
Calhoun. Am. J. O., ii, 255, 1919.
Edgerton. Am. J. O., xvi, 1076, 1933.
Eranke. K. M. Aug., lviii, 165, 1917.
Fuchs. Z. f. Aug., xv, 191, 1906.
A. f. O., xciii, 381, 1917.
Galezowski. Art. d’Oc., czliv, 169, 1910.
Giannantoni and Possente. An. dº Ott., lxi,
823, 1933.
Gradle. Am. J. O., xii, 547, 1929.
Gunn. O. Rev., viii, 225, 1889.
Koby. An. d’Oc., clx, 119, 1923.
Koeppe. A. f. O., xcvii, 198, 1918.
Krº, K. M. Aug., lxxii, 802; lxxiii, 695,
1924.
Kraupa. K. M. Aug., lxxi, 200, 1923; lxxii,
670, 1924.
Lan-Ting. Chinese Med. J., xviii, 963, 1932.
Lazarescu. An. d’Oc., clxx, 767, 1933.
Lloyd. Am. J. O., xiv, 287, 1931.
Lutz. Z. f. Aug., xix, 208, 1908.
Deut. med. W., xxxvi, 1125, 1910.
A. f. O., czz, 653, 1928.
Malgat. Rec. d’O., ix, 321, 1889; xvii, 449,
1895; xxi, 326, 1899.
Mayou. T. O. S., xxx, 196, 197, 1910; xxxvi,
411, 1916.
Metzger. K. M. Aug., lxix, 838, 1922.
Metzner and Wölfflin. A. f. O., lxxxix, 308,
1914; xci, 167, 1916.
Muck. K. M. Aug., xciii, 341, 1934.
Passow. A. f. Awg., cviii, 137, 1933.
Münch. med. W., lxxxi, 1243, 1934.
Haessler. A. of O., ii, 333, 1929.
Heine. Med. Kl., viii, 1777, 1912.
Rl. W., ii, 345, 1923. -
v. Herrenschwand. K. M. Aug., lx, 467, 1918.
Rl. W., xxiii, 1059, 1923. Streiff. K. M. Aug., lxii, 353, 1919.
A. f. Aug., xlv., 103, 1924. Sym. O. Rev., viii, 202, 1889.
Hutchinson. R. L. O. H. Rep., vi, 44, 277, Szekely. Z. f. Aug., lxxiv, 329, 1931.
1869. Terrien. A. d’O., xlv., 544, 1932.
Ring. T. Am. O. S., xxv, 380, 1927. Weill. Cb. pr. Aug., xxviii, 186, 1904.
Raia. Am. J. O., xiv, 299, 1931.
Scalinci. A. d’O., xxix, 560, 1909.
A. de Ott., xxii, 57, 1915.
Schlippe. A. f. Aug., lxvii, 97, 1910.
5. UVEITIS WITH ALOPOECLA, VITILIGO, POLIOSIS, AND DYSACOUSIA
It is now recognized that a clinical syndrome occurs wherein a chronic
bilateral uveitis is associated with widespread disturbances affecting especially
the skin, the pilous system, and the ear. The uveitis is a generalized affection
characterized essentially by a diffuse exudative choroiditis frequently leading
to a detachment of the retina, and by an exudative irido-cyclitis. In the
skin a patchy depigmentation occurs (vitiligo), the hair may be lost in
patches (alopoecia) or become whitened (poliosis), the affection involving
not only the hair of the head but also including the lashes, the eyebrows,
and the hair of the axillae; and in the ear there may be deafness and tinnitus.
We have already seen that a depigmentation of the lashes in association with
chronic uveitis, particularly sympathetic ophthalmitis, is an observation of considerable
age (Schenkl, 1873; Jacobi, 1874; Nettleship, 1884; Bock, 1890; Tay, 1892; and
others). Other isolated associations were noted sporadically with cases of uveitis—a
patchy depigmentation of the skin in irido-cyclitis by Gilbert (1910), with chorio-
retinitis by Erdmann (1911), while Cramer (1913) described a case of sympathetic
inflammation the first attack of which was accompanied by deafness and the second
with a whitening of the hair of the head and axillae and partial baldness. The first to
describe the complete syndrome was Vogt (1906), and some time thereafter there
followed a number of cases from Japan where the condition appears to be quite common
(Komoto, 1911–12 ; Tsugi, 1918; Hata and Harada, 1918; Masuda, 1926; Harada,
1926; Koyanagi, 1929; Tagami, 1931; and Hamada, 1934). Other reports have
followed from Europe and America (Wexler, 1928; Soriano, 1929; Parker, 1931;
Rones, 1932; Salus, 1932; Bunge, 1933; Davies, 1935) and from China (Luo, 1936).
DISEASES OF THE UVEAL TRACT 2365
The disease occurs in young adults, usually in the third decade, but a
case as young as 10 years has been reported (Rones, 1932). In all cases
there is bilateral and widespread chronic uveitis appearing in both eyes
at the same time, without obvious subjective symptoms except gross visual
disturbance : it is of the exudative type, producing at first the fundus picture
of an oedematous retinitis with profuse vitreous opacities together with an
exudative irido-cyclitis. Thereafter, if the fundus can be seen, a diffuse
exudative choroiditis may result in a complete retinal detachment, while
the irido-cyclitis progresses to the formation of synechiae and seclusion of the
pupil. The appearance, indeed, resembles a severe bilateral sympathetic
ophthalmitis. A rise in tension is rare, for a tendency to hypotony is the
more common condition. The inflammation, however, tends to be self-
limiting, and although at the height of the affection the vision is almost
invariably reduced to mere perception of light, the final visual result is
frequently surprisingly good, some 30% of the cases recovering normal
vision. Permanent loss of vision is common, however, and operative
attempts to improve it, as by iridectomy, may not succeed (Parker, 1931).
When the process finally recedes and the fundus is visible, the retina is
depigmented and appears of a diffuse yellowish colour with a few pigmented
foci and atrophic areas.
Alopoecia areata, involving a patchy baldness of hair, eyebrows and
lashes occurs invariably, and poliosis in about 90% of cases. Vitiligo and
temporary deafness occur in about half of the cases. The changes in the
skin and hair appear in from 3 weeks to 3 months after the onset
of ocular symptoms, and in a large number of cases the hair becomes normal
again in from 5 to 8 months. The impairment of hearing and the vitiligo,
on the other hand, come on soon after or practically simultaneously with the
eye symptoms and may last 4 to 6 weeks, the causative lesion being
apparently either in the labyrinth or the central nervous system. Occa-
sionally general symptoms indicating meningeal involvement occur—
headaches, vomiting and vertigo, with an increase in pressure and cell-
content of the cerebro-spinal fluid (Hamada, 1934). In its most complete
form, therefore, the syndrome involves meningeal, vestibular, cochlear, skin
and eye changes.
The treatment of the condition is entirely symptomatic."
The aetiology of the disease is unknown. On the clinical findings in
the vast majority of cases the factors of syphilis and tubercle, as suggested
by Demaria (1917), can be excluded. Some cause must be found for the
peculiar exudative uveal inflammation and the depigmentation and atrophic
changes in the skin. To link the two it seems necessary to postulate either
an endocrine or a toxic factor. Recalling that the suprarenals control
pigmentation, that ovarian hypofunction leads to vitiligo and that the skin
1 p. 2208.
2366 TEXT-BOOK OF OPHTHALMOLOGY
of eunuchs is unpigmented, Koyanagi (1929) suggested an endocrine origin:
but the only clinical case substantially bearing out this hypothesis is that of
Jess (1932) of a girl who had adiposo-genital dystrophy with derangement
of the pituitary, ovaries, thyroid and suprarenals. It seems most probable,
therefore, that most cases must be referred to a toacic cause, and in this
connection the resemblance of the disease to sympathetic ophthalmitis must
be noted. The most obvious supposition is that some unknown virus is
concerned ; but here again there are advocates of the parallel allergic theory,
Elschnig (1910) and Peters (1912) ascribing the occurrence of vitiligo and
alopoecia to the anaphylactic effect of uveal pigment, accounting in this way
for their occasional occurrence in sympathetic disease as well, a view sup-
ported by Davies (1935). Hamada (1934) also claims a common aetiology
embracing the two ; but the question must in the meantime remain subjudice.
It is possible that the two diseases to be noted next, both of which involve a
similar exudative choroiditis, are related aetiologically to this syndrome, possibly
representing a milder form : in any event, their aetiology is also unknown.
Bock. K. M. Aug., xxviii, 484, 1890.
Bunge. A. f. Aug., cviii, 212, 1933.
Cramer. K. M. Aug., li (2), 205, 1913.
Davies. A. of O., xiv, 239, 1935.
Demaria. A. of Oft., H.-A., xvii, 355, 1917.
Elschnig. A. f. O., lxxvi, 509, 1910.
Erdmann. K. M. Aug., xlix (1), 129, 1911.
Gilbert. K. M. Aug., xlviii, 24, 1910.
Hamada. Acta S. O. Jaſp., xxxviii, 109, 127,
1934.
Harada. Nipp. Gank. Zass., xxx, 356, 1926.
Hata and Harada. Jikken Gank. Zass., ii,
199, 1918.
Jacobi. K. M. Aug., xii, 153, 1874.
Koyanagi. K. M. Aug., lxxxii, 194, 1929.
Luo. Chima Med. J., l, 1409, 1936.
Masuda. Jikken Gank. Zass., ix, 431, 1926.
Nettleship. T. O. S., iv, 83, 1884.
Parker. Am. J. O., xiv, 577, 1931.
Peters. D. med. W., xxxviii, 1361, 1912.
Rones. A. of O., vii, 847, 1932.
Salus. K. M. Aug., lxxxix, 84, 1932.
Schenkl. A. f. Derm. Syph., v, 137, 1873.
Soriano. A. de Oft. B. A., iv, 557, 1929.
Tagami. Acta S. O. Jap., xxxv, 114, 1931.
Tay. T. O. S., xii, 29, 1892.
Tsugi. Nippon Gank. Zass., xxii, 359, 1918.
Vogt. K. M. Aug., xliv, 228, 1906.
Wexler.
Jess. B. O. G. Leipzig, xlix, 469, 1932.
Komoto. K. M. Aug., xlix (1), 139, 1911;
(2), 129, 1912.
A. of O., lvii, 393, 1928.
6. ACUTE DIFFUSE SEROUS CHOROIDITIs (HARADA’s DISEASE)
This is a peculiar type of acute diffuse eacudative choroïditis of unknown oetiology and
of good prognosis, described as occurring in Japan by Harada (1926). It occurs in
adults of 30 to 50 years of age and apparently of good health, and comes on suddenly
with a serious and progressive diminution of vision. Initially it is characterized by a
diffuse yellowish Oedematous opacity over the whole of the fundus and a general
vitreous haze without clinical evidence of involvement of the anterior segment of the eye ;
detachment of the retina then occurs, and extends so that the entire retina becomes
detached in a funnel-shape dome to the papilla, while there is a diffuse atrophy of the
pigment layer. At this stage perception of light may be lost. After several weeks the
detachment becomes flatter, and eventually complete re-attachment takes place so
that the fundus assumes its normal colour apart from the presence of irregularly placed
' pigment spots, the process being accompanied by a return of the vision to normal.
During the first two or three weeks there is headache, loss of appetite, nausea and
vomiting, but after two or three weeks as the changes in the fundus reach their height,
the general symptoms disappear.
DISEASES OF THE UVEAL TRACT 2367
Tagami (1931) has reproduced the disease in animals' eyes by the injection of fluid
aspirated from the sub-retinal space. He produced a uveal infiltration with lympho-
cytes and large mononuclear cells, resembling that found in sympathetic inflammations
except for the absence of giant cells. There was much serous and fibrinous exudation,
detaching the retina, but the viability of the neuro-epithelium remained unimpaired,
a fact which explains the remarkable recovery of vision after so severe an inflammatory
reaction.
A picture with some affinities to the preceding but limited in extent to the macular
region has been described as occurring quite commonly in Japan—CHORIO-RETINITIS
CENTRALIS SEROSA. It may be related to the preceding condition, but it will be
discussed together with angiospastic retinopathies.”
Harada. Nipp. Gank. Zass., xxx, 356, 1926. Tagami. Acta O. S. Jap., xxxv, 114, 1931.
D. Fungus Infections (Mycoses)
Fungus infections of the uveal tract are extremely rare, only sporadic
cases appearing in the literature. It is not exceedingly uncommon for a
mycotic keratitis to cause an intra-ocular inflammation, but cases of
endogenous metastatic mycotic uveitis are exceptional. Thus Verhoeff
(1926) described histologically a case of such a nature which ended fatally
with hepatic and endocarditic lesions where the infecting organism resembled
ACTINOMYCOSIS, an organism which gives rise to a slight iritis on inoculation
into the anterior chamber (Ponfick, 1881); and there is an undoubted case
of BLASTOMYCOSIS in the literature and several of ASPERGILLOSIS.
BLASTOMYCOSIs (MONILIA)
A uveal infection with blastomycetes * is a very great rarity. Two cases of intra-
Ocular infection which appeared to be exogenous, occurring after the entrance of
organisms through a corneal ulcer have been reported by Churchill and Stober (1914)
and Lundsgaard (see McKee, 1930). The possibility, however, of metastatic
infection was proved by Stock (1908) who produced tubercle-like lesions in the iris
and choroid after the intra-venous injection of the fungus in animals. Only one such
case appears in the literature (Schwartz, 1931), which had a heavy and fatal systemic
infection involving the skin, sub-cutaneous tissues, lungs, spleen, knee-joint, and eye.
A tumour appeared on the iris associated with a violent irido-cyclitis, which eventually
discharged pus into the anterior chamber ; it was tentatively diagnosed as tubercle,
but on withdrawal the pus gave a pure culture of blastomycetes. Necropsy showed an
intense polymorphonuclear infiltration with giant cells and blastomycetes, the centre of
the affected area of the iris being necrotic. The diagnosis can only be made by the
microscopic identification of the monilia ; treatment should consist of large doses of
iodides.
ASPER GILLOSIS
We have already seen * that an intractable and necrotic ulcer of the cornea is not
uncommonly produced by the fungus aspergillus fumigatus, and that with the keratitis
a severe toxic irido-cyclitis results. Occasionally an ulcer perforates, directly infecting
the uvea (Castroviejo and Urra, 1921), and at other times the fungus may penetrate
1 p. 259 . * Vol. II, p. 1661. * Vol. II, p. 1941.
T.O.-WOL. II. T
2368 TEXT-BOOK OF OPHTHALMOLOGY
into the inner eye, usually causing a vitreous abscess and a panophthalmitis (Schirmer,
1896; Nobbe, 1898; Kampherstein, 1903; Best—in a blackbird, 1905). More rarely
the absence of obvious superficial infection and the presence of the fungus elsewhere
in the body suggest an endogenous origin (Dimmer, 1913; Stock, 1926; Huber, 1933;
Lorenz, 1933). Histological findings in such cases (Huber, 1933) reveal an abscess in a
shrunken vitreous with an acute necrotic irido-cyclitis. The diagnosis is by recognition
of the fundus pathologically ; and the usual treatment is excision of the eye.
Best. D. med. W., xxxi, 1820, 1905. Lundsgaard. Quoted McKee, A. of O., iii,
Castroviejo and Urra. A. de Oft. H.-A., xxi, 301, 1930.
453, 1921. Nobbe. A. f. O., xlv. (3), 700, 1898.
Churchill and Stober. A. Imt. Med., xiii, 568, Ponfick. Virchow's Festsch., Berlin, 1881.
1914. Schirmer. A. f. O., xlii (1), 131, 1896.
Dimmer. K. M. Aug., li (2), 194, 1913. Schwartz. A. of O., v, 581, 1931.
Huber. Schw. med. W., lxiii, 181, 1933. Stock. Beit. z. path. Amat., xliii (3), 470, 1908.
Rampherstein. K. M. Aug., xli (1), 151, 1903. K. M. Aug., lxxvi, 49, 1926.
Lorenz. Diss., München, 1933. Verhoeff. A. of O., Iv, 225, 1926.
E. Uveitis due to Irritants
That a severe irido-cyclitis may be caused by irritant chemical
substances, whether of inorganic, animal or vegetable origin, we have already
seen when discussing diseases of the conjunctiva." The subject need not be
stressed further here except to point out that in many cases the clinical
reaction from necrotic burns, the juices of poisonous plants or the stings of
insects may be ultimately more serious in its effects upon the internal eye
than because of the damage done to the superficial tissues. In these cases
the irido-cyclitis conforms to the general type; but the destructive reaction
which follows the introduction into the eye of the hairs of caterpillars or
some plants calls for some remark.
OPHTHALMIA NODOSA (IRIDO-CYCLITIS PSEUDO-TUBERCULOSA)
Ophthalmia nodosa has already been discussed among diseases of the conjunctiva,”
and it will be sufficient to note the fact here that if the hairs lodge in the cornea a severe
toxic irido-cyclitis with a hypopyon may develop, while if they enter the iris the
typical nodular lesion composed essentially of lymphocytes and giant cells, may be
produced (see especially Wagenmann, 1890; Stargardt, 1903; de Schweinitz and
Shumway, 1904; Teutschländer, 1908; Dejean and Harant, 1934; Villard and
Dejean, 1934). Occasionally the hairs wander far into the eye, even to the optic disc
(Ogin, 1907). The irido-cyclitis is most recalcitrant, the hypopyon may be massive,
and after the usual history of repeated exacerbations and intermissions, vision may be
gravely affected by extensive iridic adhesions and vitreous opacities. One or more iridic
nodules may develop, which may give the clinical appearance of tubercles, and on their
resolution they leave cicatrized and deformed areas ; exceptionally the eye may be
lost from phthisis bulbi (v. Hippel, 1918). Apart from the usual treatment of irido-
cyclitis, nodules on the iris should be dealt with by removal by an iridectomy.
Dejean and Harant. S. med. Biol. Mont- Stargardt. A. f. O., Iv, 469, 1903.
pellier, xv, 539, 1934. Teutschländer. A. f. Aug., lxi, 117, 1908.
v. Hippel. A. f. O., xcvi, 364, 1918. Villard and Dejean. A. d’O., li, 719, 1934.
Ogin. K. M. Aug., xlvi, 484, 1907. Wagenmann. A. f. O., xxxvi (1), 126, 1890.
de Schweinitz and Shumway. T. Am. O. S.,
x, 237, 1904.
1 Vol. II, p. 1707. * Vol. II, p. 1718.
DISEASES OF THE UVEAL TRACT 2369
IV. UWEAL DISEASE IN ASSOCIATION WITH GENERAL DISEASE
1. Vascular Diseases
(a) VASCULAR SCLEROSIs
Sclerosis of the uveal vessels is a much more common condition than
might be realized clinically because it cannot be diagnosed (apart from its
pathological recognition) until it has progressed to a very advanced degree :
in the choroid, for example, the vessels are normally hidden from ophthal-
moscopic examination until the overlying pigment epithelium has been
destroyed. The condition may be either primary or secondary. The
secondary type follows any inflammatory or degenerative condition of
sufficient seriousness to cause an endarteritis and thrombosis ; such sclerotic
islands are therefore a constant sequel of most disease-processes and need not
detain us further here.
PRIMARY VASCULAR SCLEROSIS, on the other hand, is due to sclerotic
processes originating in the vessel walls themselves, and as such has the
same aetiology as arteriosclerotic lesions elsewhere.” The atherosclerotic
type of change, indeed, may be said to be a constant age-change (Müller,
1872; Friedenwald, 1930): thus Kerschbaumer (1892) found their presence
in the uvea in some degree in 50% of people between 40 to 50 years of age,
in about 75% of those between 50 and 60, and to some extent in all cases
above 60. These findings were confirmed by Friedenwald (1930), who
concluded that arteriolar sclerosis in the choroid usually started at the age
of 40, and, as occurs in the spleen, progresses slowly without special relation
to general vascular disease : moreover, choroidal sclerosis seems to have
no parallelism to cerebral sclerosis (Wood, 1915). In the presence of
advanced generalized arterial disease, however, such changes are more
accentuated, and they occur in their most decided form in arteriosclerosis
complicated by renal disease.
The pathological changes described are many and various, and conform to the
histological picture found elsewhere * (Müller, 1872; Kerschbaumer, 1892; Garnier,
1892; Siegrist, 1896–99; Ewetzky, 1898; Hofmann, 1902; Parsons, 1904; Schmidt,
1905; Krückmann, 1906; Attias, 1912; Coats, 1913; Ginsberg, 1912–28 ; Monauni,
1919; Rones, 1938; and many others) (Figs. 1959–60). The senile atherosclerotic
type includes in its picture a deposition of lipoids in the intima associated with
fibrous-tissue proliferation and followed by hyaline degeneration which may be
extreme, sometimes with the formation of large atheromatous plaques. In this senile
change the thickening and hyalinization of the adventitia and the media may be so
marked that it may be difficult to differentiate these structures from the hyalinized
connective tissue surrounding the vessels; but there is little narrowing of the
lumen or tendency to obliteration (Fig. 1981). In the more arteriosclerotic types
there is connective-tissue proliferation of the media with reduplication of the internal
elastic lamina so that eventually extreme narrowing or even obliteration of the
1 Vol. II, p. 1430. * Vol. II, p. 1431.
2370 TEXT-BOOK OF OPHTHALMOLOGY
lumen may follow (Fig. 1960). The ultimate result may be complete occlusion of
the vessel with advanced hyaline or lipoid degeneration and complete degeneration
and disorganization of the cells of its walls.
Fig. 1959.-ScLERosis or VEssels of IRIs.
Note the emigration of pigment towards the anterior surface of the iris : chronic
glaucoma (Castroviejo, A. of O.).
These arteriosclerotic changes are found most frequently and most far
advanced at the posterior pole of the eye. Some authorities, indeed, hold
that they are confined to the region of the choriod behind the equator,
---> e-e="
º - º - - - -
-- - - º-
- --- -
Fig. 1960–ARTERIolar ScLERosis IN CHoRoip (Friedenwald, A. of 0.).
that is, to the area of distribution of the short posterior ciliary arteries;
nevertheless they do occur in the periphery of the choroid, more rarely in
the ciliary body, and stillmore rarely in the iris. In the latter situation they


DISEASES OF THE UVEAL TRACT 2371
may be seen in cases of chronic glaucoma with generalized arteriosclerosis
(Castroviejo, 1931) (Fig. 1959), but are usually met with in cases of arterio-
sclerosis associated with renal disease : and in these they occur but seldom
(Gilbert, 1928–34). Some writers consider that they give rise to the
“primary ‘’iritis met with in nephritics (Leber, 1885–1915; v. Michel
1900–06), and occasionally in arteriosclerotics (Adams, 1920) (the IRIDO-
scLEROSIs of Gilbert, 1921),” and they may possibly account for some cases
of post-operative iritis. In the ciliary body and the anterior part of the
choroid such changes are more common : thus Mylius (1934) found them
markedly in 21 out of 86 cases of renal disease. Sometimes their clinical
recognition is rendered possible by the detection with the slit-lamp of a
slight diffuse clouding of the aqueous humour denoting an interference with
the normal permeability of the vessel walls.
It is in the posterior region of the choroid, however, that sclerotic
changes are common and most especially marked (Fig. 1960). The posterior
ciliary arteries may be grossly affected both in their extra- and intra-scleral
course, the choroidal arteries may become obliterated to be replaced by
hyaline and fibrous tissue strands, the chorio-capillaris may have disappeared
over large areas, less marked changes may occur in the choroidal veins, and
the perivascular spaces bordering the vortex veins may be filled with
wandering cells or proliferated endothelial cells or obliterated by fibrous
tissue. Ophthalmoscopically the characteristic changes seen are the senile
crescent around the optic disc, an exaggerated tigroid appearance of the
fundus, colloid excrescences, and areas of disturbed choroidal pigmentation.
Such changes are found most typically in association with general vascular
sclerotic states especially in old age ; they are, however, seen particularly
in renal disease 2 (Gilbert, 1935), and in this condition, of course, the prognosis
is not good. Cohen (1922), indeed, considers that choroidal arteriolar
sclerosis plays an important part in the development of renal retinitis.
Somewhat similar changes, involving fibrous tissue proliferation, hyaline degenera-
tion and proceeding to obliteration of the vessels are met with in lead poisoning (Oeller,
1881); while sclerotic changes and choroidal haemorrhages with subsequent retinal
degeneration are found with some frequency in osteitis deformans (Paget’s disease)
(Coppez, 1912; Ormond, 1931 ; Cohen, 1938). As a rarity, extensive sclerotic changes
of this nature are found, presumably as a causal factor, in haemorrhagic glaucoma
(Kümmell, 1909).
We have seen that arteriosclerotic changes in the choroid assume their
greatest importance by reason of their effects on the vitality of the retina ;
and this, of course, is of greatest moment in the central area. Certain
retinal diseases affecting this region, particularly circinate retinopathy, may in
certain cases be due more to choroidal than retinal events, a conclusion
suggested by the fact that the exudates may be found only in the external
layers of the retina and very extensively in the choroid (Ammann, 1897;
1 p. 2157. * p. 2157.
237.2 TEXT-BOOK OF OPHTHALMOLOGY
Seefelder, 1929 ; Evans, 1933); how far these can be considered secondary
to choroidal disease will be considered subsequently." The foveal region
itself, however, lacking a retinal circulation and entirely dependent upon the
underlying chorio-capillaris, is a frequent and direct sufferer from such
choroidal changes, and we shall now consider in this section the frequent
degenerative changes which occur there as a direct consequence of arterio-
sclerotic disease in the choroid and usually in association with general
systemic arterial disease.
SENILE MACULAR CHORIO-RETINAL DEGENERATION
(SENILE MACULAR DEGENERATION OF HAAB)
SENILE MACULAR DEGENERATION, first described as a clinical entity
by Haab (1885), is a common cause of failure in central vision in old people.
It is characterized by the presence of degenerative changes, usually punctate
in nature, occurring bilaterally, limited to the region of the macula, and due to
sclerosis and obliteration of the chorio-capillaris in the central area (Plate XLIII,
Figs. 1961–66).
Clinically the changes may appear too minute to warrant the disastrous
visual effects which they produce. Frequently they are difficult to make
out at all, and when on clinical examination no obvious cause can be
found for visual deterioration in an old person, careful examination of
this region after the pupil has been dilated may reveal fine pigmentary
disturbances. This appearance is considerably enhanced if red-free light
is employed. The first change is usually a ring of pigment dots, the stippled
appearance being sharply defined on the foveal side and tailing off peri-
pherally into a normal fundus. The fovea itself gradually becomes paler
in colour and the stippling denser, until gradually the progressive failure
in central vision develops into an absolute scotoma, which despite all
treatment is permanent. The condition usually starts unilaterally, but after
some time—it may be many months—the other eye is almost invariably
affected ; and at any time the normal slow progress of the disease may be
dramatically accelerated by the development of both retinal and choroidal
haemorrhages (Fig. 1964). In the retina these may leave round yellowish-
white patches, and in the choroid large irregular areas of sclerosis and
atrophy may develop or the picture of disciform degeneration * may result.
The pathology of the condition has been investigated by several authors
(Nagel, 1875; Harms, 1904; Friedenwald, 1930; Rones, 1938; and
others) (Fig. 1967). The primary changes are found in the choroidal circula-
tion, which shows marked sclerotic changes and hyaline degeneration. The
whole thickness of the choroid is affected, but the most advanced changes
are in the chorio-capillaris where most of the channels disappear. Colloid
1 p. 2760. * p. 2115.
PLATE XLIII
SENILE MACULAR DEGENERATIos
Various stages of senile macular degeneration due to arteriosclerotic disease of the
choroid. The most advanced case (Fig. 1966) was complicated by arteriopathic
dementia.
Fig. 1961. Fig. 1962.
Fig. 1963. Fig. 1964.
Fig. 1965. Fig. 1966.
[To face p. 2372.
-
tº /2
*fcº.



DISEASES OF THE UWEAL TRACT 2373
bodies are frequently seen on Bruch's membrane, and the pigmentary
epithelium overlying may be raised by exudative material; the pigment
cells show atrophy or proliferation with a dispersal or a heaping up of
pigment. The deeper layers of the retina, deprived of their nourishment,
become disorganized and atrophied, a process which, indeed, may finally
extend throughout the thickness of this tissue, until in the foveal region
only a few degenerated cells remain lying close to the internal limiting mem.
brane, the cones being ill-formed or entirely absent. It is to be noted that
since the sclerotic changes in the choroid may sometimes be widespread,
-
Fig. 1967.-SENILE MACULAR DEGENERATION showING ADVANCED ARTERIo-
ScLERosis (Rones, Am. J. O.).
the limitation of the retinal changes to the macula indicates the greater
vulnerability of this region.
While the sclerotic origin of senile macular degeneration is generally accepted, van
der Hoeve (1918–20) considered that the action of light might be a contributory cause.
This was suggested to him by the observation that the condition occurred rarely in
conjunction with senile cataract, in which case, of course, the opaque lens would
absorb the light and protect the posterior pole. His statistical evidence was corro-
borated by Gjessing (1925), but may be no more than an interesting coincidence.
The treatment of sclerotic lesions of the choroidal vessels, as of all
processes of this nature, is extremely unsatisfactory. The condition is due
essentially to processes outside medical control—the gradual attrition
of age and the stresses of living, the influence of hereditary and constitutional
factors, and the accumulated effects of low-grade pathological processes
acting over a long time. Moreover, in all such cases we are dealing with
tissues of low vitality which are in no condition to respond to therapeutic or
stimulatory measures. In view of the fact that toxic influences of a chronic
nature do predispose to such changes, the elimination of any septic focus
is advisable; and it is significant that a very large number of such cases




2374. TEXT-BOOK OF OPHTHALMOLOGY
suffer from a chronic and long-standing streptococcal toxaemia, originating,
for example, from the teeth, the throat and the bowel. For the rest, a suitable
mode of life and diet, with perhaps mild cardio-vascular stimulants and the
administration of iodine and small doses of thyroid is the most that can be
done.
Optical appliances in cases of macular degeneration may, it is true,
tide the patient over for a little time—strong convex lenses, telescopic
glasses, and so on—but slowly and irrevocably the central vision diminishes,
all fine work and reading become more and more difficult and then impossible,
and although field vision, and with it the ability to get about the world,
remains, such people can only be helped in so far as every effort should be
made to assist them to accommodate their interests with philosophy and
resignation to their limited visual efficiency.
Adams. Brit. J. O., iv, 297, 1920. Hofmann. A. f. Aug., xliv, 339, 1902.
Ammann. A. f. Aug., xxxv, 123, 1897. Rerschbaumer. A. f. O., xxxviii (1), 127,
Attias. A. f. O., lxxxi, 405, 1912. 1892.
Castroviejo. A. of O., v., 189, 1931. Krückmann. B. O. G. Heidel.., xxxiii, 65,
Coats. T. O. S., xxxiii, 30, 1913. 1906.
Cohen. T. O. Sec. Am. Med. As., 61, 1922. Kümmell. A. f. O., lxxii, 86, 1909.
Leber.
A. of O., xix, 487, 1938.
Coppez. A. d’O., xxxii, 380, 529, 1912.
Evans. Brit. J. O., xvii, 257, 1933.
Ewetzky. K. M. Aug., xxxvi, 381, 1898.
Friedenwald, J. S. Path. of the Eye, Balti-
more, 1930.
Friedenwald, H. T. O. S., I, 452, 1930.
Garnier. Cb. pr. Aug., xvi, 9, 1892.
Gilbert. Münch. med. W., lxviii, 979, 1921.
R. M. Aug., xciv, 335, 1935.
Ginsberg. A. f. O., lxxxii, l, 1912.
Hb. sp. path. Anat. w. Histol., xi (1), 489,
1928.
Gjessing. Z. f. Awg., lvi, 79, 1925.
Acta O., ii, 341, 1925.
Haab. Cb. pr. Aug., ix, 383, 1885.
Harms. K. M. Aug., xlii (2), 50, 1904.
v. d. Hoeve. A. f. O., xcviii, l, 39, 49, 1918.
K. M. Aug., lxi, 140, 1918.
Am. J. O., iii, 178, 1920.
A. f. O., xxxi (4), 183, 1885.
G.-S. Hb., II, vii (1), 865, 872, 1915.
v. Michel. Münch. med. W., xlvii, 853, 1900.
Z. f. Aug., xv, 13, 1906.
Monauni. A. di Ott., xxvi, 263, 1919.
Müller, H. Gesammelte w. hinterlassene
Schriften, Leipzig, 1872.
Mylius. B. O. G. Heidel., I, 138, 1934.
Nagel. K. M. Aug., xiii, 388, 1875.
Oeller. A. f. path. Anat., lxxxvi, 329, 1881.
Ormond. T. O. S., li, 258, 1931.
Parsons. Pathology of the Eye, i, London,
1904.
Rones. Am. J. O., xxi, 239, 1938.
Schmidt. Zb. f. path. Anat., xvi, 49, 1905.
Seefelder. A. f. Awg., c-ci, 334, 1929.
Siegrist. B. O. G. Heidel.., xxv, 83, 1896.
IX. Internat. Cong. O., Utrecht, 36, 1899.
Wood. Ophthalmoscope, xiii, 374, 1915.
(b) PERI-ARTERITIs NoDOSA
PERI-ARTERITIS NoDOSA, a somewhat rare disease described originally
by Kussmaul and Maier (1866) and elucidated particularly by Gruber (1925),
is characterized by the formation of multiple modules on the smaller arteries.
It is found usually in young persons and probably represents the reaction of
the arterial system to a generalized toacic process of a severe and chronic nature
for which no particular organism can be held responsible ; the streptococcus
and the staphylococcus are most frequently found. The nodules are of an
inflammatory character ; there is a massive infiltration of the adventitia,
particularly with leucocytes, lymphocytes and plasma cells, as well as
DISEASES OF THE UVEAL TRACT 2375
degeneration of the media and proliferation of the intima. Eventually fibrous
tissue develops, the wall gives way as an aneurysmal dilatation, and finally
hyaline degeneration and extensive thrombosis occur. The disease, after
Fig. 1968. PERI-ARTERITIs NoDos A.
Peri-arterial nodule in a short ciliary artery just outside the sclera (Friedenwald
and Rones, A. of O.).
Fig. 1969.-PERI-ARTERITIs NoDos A.
Peri-arterial nodule in the choroid (Friedenwald and Rones, A. of O.).
running a course characterized by exacerbations and remissions, is usually
fatal, most frequently ending in a terminal nephritis.
Despite the vascularity of the eye, observations of the occurrence of
typical nodes are few, although they date from the time of Müller (1899),
who noted changes of this type in the retinal vessels. Böck (1929) and v.


2376 TEXT-BOOK OF OPHTHALMOLOGY
Herrenschwand (1929) found typical nodules in the short ciliary and other
orbital arteries, but the first to find pathological changes in the choroidal
circulation were Christeller (1926), Goldstein and Wexler (1929) and Helpern
and Trubek (1933) in cases of endocarditis with multiple emboli. A typical
case was also examined by Friedenwald and Rones (1931) wherein nodules
of fibrous thickening with intense mononuclear infiltration were evident in
the short ciliary arteries and also in the choroid (Figs. 1968 and 1969).
Ophthalmologically the patient showed a picture of advanced arterio-
sclerosis with multiple haemorrhages and exudates. King (1935) also
described a typical case in which a transitory papilloedema occurred in one
eye, while in the other a recurrent intense sub-acute uveitis associated with
peri-arterial retinal infiltration led to secondary glaucoma which necessitated
excision of the globe.
In a less typical case Friedenwald and Rones (1931) found histologically similar
nodules in the choroid which appeared ophthalmoscopically during life as rounded
yellow elevations underneath the retina. Similar inflammatory nodules were
noted pathologically in the choroid by Hanssen and Knack (1917) in nephritis with
albuminuric retinitis; they attributed the lesions to this disease, but it is to be noted
that many of their cases also had manifestations of a chronic infective nature as well,
to which the nodular arteritis might well have been due. Tertsch (1935) reported a
case without ocular involvement but with peri-arterial infiltration in the extra-ocular
muscles.
Belpern and Trubek. A. f. Path., xv, 35.
Böck. Z. f. Aug., lxix, 225, 1929.
Christeller. A. f. Verdauungskr., xxxvii, 249, 1933.
1926. v. Herrenschwand. K. M. Aug., lxxxiii, 419,
Friedenwald and Rones. A. of O., v., 174, 1929.
1931. King. T. O. S., Iv, 246, 1935.
Goldstein and Wexler. A. of O., ii, 288, 1929. Kussmaul and Maier. D. A. f. kl. Med., i,
Gruber. A. f. path. Amat., cclviii, 441, 484, 1866.
1925. Müller. Festsch. Stadt. Krankenh., Dresden,
Hanssen and Knack. K. M. Aug., lix, 263, 458, 1899.
1917. Tertsch. Z. f. Aug., lxxxvii, 294, 1935.
2. Haemopoietic Diseases
(a) LEUKAEMIA
In this disease there is a decrease in the red cells and an abnormal increase in the
white cells of the blood, either the polymorphonuclear leucocytes and their corre-
sponding myelocytes (MYELOID LEUKAEMIA) or the lymphocytes (LYMPHOID LEUKAEMIA);
in rare cases wherein the lymphocytes are relatively increased without an increase in
the total number of leucocytes, the contradictory term ALEUKAEMIA is employed. In
either case the disease is usually chronic, lasting a year or two, but may run an acute
course ; and in neither case is the aetiology known, some aspects being in favour of
an infective origin, but more pointing to a neoplastic origin. As would be expected
with changes in the blood of so profound a nature, the nutrition and permeability of
the capillary endothelium suffers, so that both cellular elements and fluid readily
escape into the perivascular spaces and the tissues, and there is evidence that this
process is intensified by the multiplication of the leucocytes after they have left the
vessels. The escape of the formed elements of the blood may also be aided by actual
DISEASES OF THE UVEAL TRACT 2377
destruction of the vessel walls after their invasion by leucocytes (Murakami, 1901;
Goldstein and Wexler, 1935). It follows, therefore, that both types of leukaemia,
but especially the lymphatic type, are characterized by the accumulation of immense
numbers of leucocytes in many organs—usually the spleen and lymphatic glands,
and more rarely other organs such as the liver, kidneys, intestine, skin, conjunctiva,"
and the uveal tract. Retinal changes are common and will be noted subsequently.
In general terms two types of pathological change are evident in the
uveal tract in leukaemia : (1) an immense engorgement of the blood-vessels
with white cells leading to a marked stretching of their walls and dilatation
of their lumen, associated with few changes in the stroma (Fig. 1970); and
(2) a massive invasion of the tissues with white cells to form a lymphomatous
infiltration, sometimes of a tumour-like nature, while the vascular system
remains relatively normal (Fig. 1971). The various types of leukaemia are
not always differentiated in the literature, but with few exceptions the first
Fig. 1970. THE CHoRoid IN LEUKAEMIA.
Showing vascular engorgement with slight nodular infiltration of the tissues (N)
(Goldstein and Wexler, A. of O.).
change is usually characteristic of myeloid leukaemia, and the second of the
lymphatic type of the disease (Koyanagi, 1914; Kümmell, 1918).
As a general rule the posterior part of the uveal tract is more affected
than the anterior; usually the iris and ciliary body, and frequently the
anterior part of the choroid are free, while the posterior part of the choroid
is immensely thickened. Occasionally, however, an infiltration of the iris
occurs. Rarely it is nodular (v. Michel, 1878–81), sometimes it appears as
an exudative inflammation (Bab, 1922, in aleukaemia), or is characterized
by a hypopyon (Weve, 1932, in myeloid leukaemia), or the iris is intensely
hyperaemic (Triebenstein, 1920, in aleukaemia), or spontaneous hæmorrhages,
although very much less common than in the retina, may lead to the forma-
tion of a hyphaema (Sorger, 1898). Typically the iris is grey and its archi-
tecture is lost, the hypopyon, which is composed of leucocytes, is greyish-
white rather than yellow as in purulent conditions, synechiae are absent,
and the surface of the iris is rich in superficial vessels.
The ciliary body is also rarely affected, but occasionally it is infiltrated,
sometimes even more than any other part of the globe (Deutschmann, 1892;
Vol. II, p. 1796.

2378 TEXT-BOOK OF OPHTHALMOLOGY
Kerschbaumer, 1895; Bäck, 1899; Meller, 1905; Weve, 1932). It is in
the choroid, however, especially in its posterior region, that the most marked
changes usually occur, either as an immense vascular engorgement in the
myelogenous type (Schultz-Zehden, 1907; Verderame, 1910; Koyanagi,
1914; Cohen, 1918; Kümmell, 1918), or as a lymphomatous infiltration
in the lymphatic type (Roth, 1870; Oeller, 1878; Osterwald, 1881;
Deutschmann, 1892; Bäck, 1899; Meller, 1905–07; Stock, 1906; Hudson,
1910; Kambe, 1914; Koyanagi, 1914; Kümmell, 1918; Goldstein and
Wexler, 1935; and others) (Fig. 1971). Haemorrhages into the tissue are
Fig. 1971–THE Choroin is Lºukaemia.
Showing massive diffuse infiltration with great thickening of the choroid (Foster
Moore, Medical Ophthalmology).
not uncommon (Parsons, 1908; Hudson, 1910). The infiltration with
mononuclear cells may be very dense, presenting the histological appearance
of a LYMPHoMA * : it is most marked in the layer of large vessels and leaves
the chorio-capillaris relatively free; usually it is diffuse, but sometimes is
localized to form a discrete lymphoma; and the cells need not all be of the
same type as those found in the blood. Thus in a case of Koyanagi's (1914),
the majority of cells in the choroidal infiltration were small lymphocytes
while the preponderant cell in the blood was the large lymphocyte. In acute
cases there may be immense karyokinetic activity (Hudson, 1910). At this
stage the fundus assumes the yellowish or pale green colour characteristic of
leukaemic retinopathy,” but the details may become obscured by oedema, a
vitreous haze or even by a detachment of the retina.
* Vol. II, p. 1796. * p. 2741.

DISEASES OF THE UVEAL TRACT 23.79
There is still some difference of opinion with regard to the origin of the infiltrating
cells. Most authorities agree with the view expressed by Stock (1906) and Hudson
(1910) that the infiltration is a passive one, the cells escaping from the circulation and
thereafter multiplying in the tissues. Kerschbaumer (1895) considered the infiltration
orbital in origin derived from extra-bulbar vessels, extending along the perivascular
lymph-sheaths of the posterior choroidal vessels, a view supported by Borgeson and
Wagener (1929). Türk (1903) and Meller (1905–07), on the other hand, held that the
cells represented a true lymphatic new-growth arising from lymphatic elements in the
tissues—in this case wandering cells or histiocytes in the uvea. The question cannot
be taken as definitely settled, but in view of the absence of lymphatic elements in the
uvea, the first view seems the more acceptable.
The prognosis of leukaemia is bad ; acute cases may die in a few days,
and chronic ones linger on, sometimes with remissions, for one or more years.
No treatment is really effective but temporary improvement may be obtained
by X-rays. Thus Alvis (1938) reported a case of the myeloid type in a child
which rapidly progressed to bilateral retinal detachment and blindness owing
to the intense choroidal infiltration of the posterior segments of the eyes,
which, after two X-ray treatments to each eye, recovered temporarily, the
retinae re-attaching themselves and some vision returning for 3 months before
the death of the patient.
Alvis. Am. J. O., xxi, 31, 1938. v. Michel. D. A. f. kl. Med., xxii, 295, 1878.
Andrews. T. O. S., xliii, 128, 1923. A. f. O., xxvii (2), 237, 1881.
Bab. Z. f. Awg., xlvii, 231, 1922. Murakami. K. M. Aug., xxxix (1), 136,
Bäck. Z. f. Aug., i, 234, 1899. 1901.
Borgeson and Wagener. Am. J. Med. Sc., Oeller. A. f. O., xxiv. (3), 239, 1878.
clxxvii, 663, 1929. Osterwald. A. f. O., xxvii (3), 203, 1881.
Cohen. A. of O., xlvii, 61, 1918. Parsons. Path. of the Eye, iv, 1317, 1908.
Deutschmann. Beit. z. Aug., iv, 42, 1892. Roth. A. f. path. Amat., xlix, 441, 1870.
Goldstein and Wexler. A. of O., xiii, 26, 1935. Schultz-Zehden. Med. Kl., iii, 663, 1907.
Hudson. R. L. O. H. Rep., xviii, 153, 1910. Sorger. Münch. med. W., xlv., 1120, 1898.
Kambe. K. M. Aug., lii, 79, 1914. Stock. K. M. Aug., xliv (1), 328, 1906.
Kerschbaumer. A. f. O., xli., 99, 1895. Triebenstein. K. M. Aug., lxiv, 825, 1920.
Koyanagi. K. M. Aug., liii, 152, 1914. Türk. Wien. kl. W., xvi, 1073, 1903.
Rümmell. A. f. O., xcv, 105, 1918. Verderame. A. f. path. Amat., ce, 667,
Meller. A. f. O., lxii, 130, 1905. 1910.
Z. f. Aug., xv, 538, 1906. Weve. A. f. Aug., cv, 710, 1932.
R. M. Aug., xlv., 491, 1907.
(b) CHLOROMA
This sarcomatous-like proliferation of the tissues of the bone-marrow
associated with leukaemic changes in the blood, usually lymphaemic but
sometimes myelaemic, rarely causes conspicuous lesions in the uveal tract.
Although dissemination may take place into the choroid (Gallemaerts, 1914),
the tissues are rarely infiltrated, but the blood-vessels contain a large
proportion of leukaemic cells (Hudson, 1911).
Gallemaerts. Bull. S. fr. d’O., xxxi, 486, 1914. Hudson. R. L. O. H. Rep., xviii, 181, 1911.
23.80 TEXT-BOOK OF OPHTHALMOLOGY
(c) ERYTHREMIA (Polycyth ºvna RuBRA VERA: VAQUEZ's DisPASE)
In this disease of the haemopoietic system characterized by an enormous
increase in the erythrocytes, the uveal vessels, particularly the veins, share
in the general vasodilatation. So much so may this be the case that the
choroid may be swollen to several times its normal size (Fig. 1972) (Foster
Moore, 1925). The venous engorgement may also change the colour of the
iris from a blue to a reddish-brown hue, a phenomenon which is not due to
Fig. 1972.--THE Choroid IN Polycyth ºria.
A, retina. B, choroid. C, selera. The chief ophthalmoscopic sign was
engorgement of the retinal veins (Foster Moore, Medical Ophthalmology).
multiple haemorrhages as was suggested by Goldzieher (1904), since it
disappears on death (Baquis, 1908; Tallei, 1924). There may also be a
degeneration and scattering of the iris pigment (Tirelli, 1928).
Baquis. A. f. O., lxviii, 117, 1908. Tallei. Boll. d’Oc., iii, 545, 1924.
Goldzieher. Cº. pr. Aug., xxviii, 257, 1904. Tirelli. A. dº Ott., xxxv, 567, 1928.
Moore, Foster. Medical Ophthalmology, II,
102, 1925.
3. Metabolic Diseases
(a) Gout
GOUTY IRITIS
How extensively the gouty diathesis figures in the aetiology of iritis
has always been a matter of dispute. The older physicians, who found their
expression in the forceful teaching of Jonathan Hutchinson (1871-85), were
liberal in the share they allotted to it, but even before his time other authori-
ties considered that the term goutyiritis was too loosely used (Mackenzie,
1833). Even although it seems certain that gout is now much less common

DISEASES OF THE UVEAL TRACT 2381
than it used to be in the days when over-indulgence in food and drink were
the necessary attributes of a gentleman, it is quite obvious to-day that the
terms “gout ’’ and “gouty rheumatism ‘’ used to be employed in far too
facile a manner, and that many cases of ocular inflammation were so labelled
on quite unconvincing evidence ; but at the same time it is as obvious that
many of the cases described were true examples of a specific clinical entity
and that similar instances are observed at the present time. The condition
is, however, rare : Lang (1913) estimated it as constituting 3.5% of all cases
of iritis, and Gilbert (1929) as 1.0%, but most writers on statistics of incidence
make no mention of it.
A typical attack of gouty or uratic iritis generally has characteristic
clinical features (Hutchinson, 1885; Krückmann, 1907; Chance, 1923;
Savin, 1938). As a rule it is preceded by or associated with an inflammatory
involvement of the outer coats, the extent of which varies from an
episcleritis fugax to a deep scleritis with chemosis. The attack usually
comes on suddenly, frequently after a dietetic indiscretion and often at
night, and may precede or accompany the involvement of a joint ; the eye
becomes intensely red and exceedingly painful, the cornea is steamy, the
aqueous cloudy, and the tension is frequently raised. The picture, indeed,
may simulate an acute glaucoma and may be mistaken for it. If the uvea
is alone involved, pain may be slight (Hutchinson, 1885; Wood, 1936), but
in the typical attack of ocular gout which involves the outer coats of the eye
as well and is associated with an intense purplish-red injection of the deep
scleral and ciliary vessels, the pain is usually excruciating and out of all
proportion to the objective evidences of intra-ocular inflammation. Quite
frequently corneal precipitates are much in evidence or a gelatinous exudate
may fill the anterior chamber, while a light cloud of vitreous opacities is not
ll.I] COIN).II) OI). -
In the ordinary case of iritis the condition runs a fulminating course
and clears up in from 4 to 10 days’ time, leaving remarkably few clinical
traces of its occurrence, keratic precipitates disappear in a few days or weeks,
and the gelatinous exudate vanishes when the acute phase has passed; indeed,
even after many recurrent attacks the only evidence of past inflammation
may be the presence of a few pigmentary tags on the lens, and on this
account the patient’s history of the violence and repetition of the trouble
may be doubted. This clinical feature is borne out by pathological findings,
for even in eyes which have had to be excised on account of the persistence of
attacks the uveal tissue may show remarkably little change, the stroma
being packed with lymphocytes but showing no gross destruction or necrosis
(Wood, 1936) (Figs. 1973-74). It may happen, however, particularly in
arteriosclerotic individuals, that as time goes on recurrences become more
recalcitrant, exudate persists and adhesions become permanent, until
eventually the entire uveal tract becomes involved and the vision is destroyed.
In the typical case, however, the sudden onset, the short duration, and the
2.382 TEXT-BOOK OF OPHTHALMOLOGY
relatively complete resolution differentiate these cases from infective
inflammations and indicate their probable chemical origin.
- - -
-
- --- ---
---
---
|-
- -
***.**-
Fig. 1973.-Gouty IRITs.
Despite scores of attacks there is comparatively little damage in the tissues
(D. J. Wood).
In contra-distinction to the crystalline deposits which may be found in
the selera and cornea (Weve, 1924; Scheffels, 1925; Axenfeld, 1930;
Fig. 1974–Gouty Uveitis.
The choroid (above) contains
many lymphocytes, and the
sub-retinal space is full of
coagulum containing large oval
pigmented cells (D. J. Wood).
Wood, 1936), urates are not deposited in the
uveal tissues: Niyazi and Vefik (1929),
however, described a typical case of a gouty
man in whose anterior chamber during
typical ocular attacks white precipitates
formed which on examination gave the
murexide reaction. They seemed to be thus
analogous to tophielsewhere.
Other conditions have been ascribed to gout
—the quiet iritis of Hutchinson (1885), the
rheumatic and gouty cyclitis of Bouchéron (1887),
the guttate iritis of Doyne (1910)-but any
aetiological relation can only be accepted with great
reservation.
The diagnosis should be suggested by the
clinical course of the condition–its sudden
onset, acute symptoms and rapid resolution.
It is supported by the diagnosis of the gouty
diathesis which rests upon the following
points: a family history of the disease; a
personal history of acute attacks particularly
if following provoking factors; the presence
of tophi; X-ray evidence of punched-out
areas in the bones of an affected joint; a
raised blood uric acid during attacks; a diminution of uric acid secretion
in the urine; and a rapid therapeutic response to colchicum.
The local treatment should include atropine and abundance of heat
which brings relief most readily; but the essential treatment liesin an attack


DISEASES OF THE UVEAL TRACT 2383
on the underlying diathesis. General measures, which should be most rigidly
enforced, include a light diet, avoidance of alcohol, abundance of bland
fluids, and the exhibition of colchicum or atophan. Almost as important
are the prophylactic steps which may be taken, rearranging the patient’s life
and habits to ensure that his metabolism is put into a more satisfactory
condition.
Axenfeld. K. M. Aug., lxxxv, 493, F930.
Bouchéron. Am. d’Oc., xcvii, 233, 1887.
Chance. Am. J. O., vi, 286, 1923.
Doyne. T. O. S., xxx, 91, 1910.
Gilbert. Kurzes H.-B. d. O., Berlin v, i, 1929.
Hutchinson. R. L. O. H. Rep., vii, 287, 455,
1871 ; xii, 61, 1888.
Lancet, i, 1, 1873; i, 14, 1876.
T. O. S., i, 32, 1881; v, 1, 1885.
Brit. Med. J., ii, 995, 1884.
Frückmann. G.-S. H.-B. II, v (6), 35, 1907.
Lang. Lancet, i, 1368, 1913.
Brit. Med. J., i, 381, 1913.
Mackenzie. Treatise on Diseases of the Eye,
1833.
Niyazi and Vefik. Turk. O. Gaz., vi, 179,
1929.
Savin. T. O. S., lviii, 149, 1938.
Scheffels. K. M. Aug., lxxiv, 510, 1925.
Weve. B. O. G. Heidel., xliv, 236, 1924.
Over keratitis Urica, Rotterdam, 1925.
Wood. Brit. J. O., xx, 510, 1936.
(b) DIABETES MELLITUs
We have seen that although it is claimed in the older literature that
diabetes mellitus figured in the aetiology of iritis (Leber, 1885), this view is
not now generally accepted. Iritis does occur in this disease (2 cases in
477 diabetics, v. Noorden, 1927; nil in 307 diabetics, Spalding and Curtis,
1927), but when it does so it is probably due to an intercurrent infection,
the pathogenicity of which may be increased by the diabetic diathesis, a
circumstance well seen in the liability of such patients to develop post-
operative iritis (Uhthoff, 1908). There are, however, certain pathognomonic
changes which are seen particularly in the iris in this condition, all of which
are probably toxic effects.
1. CEDEMA AND VESICULATION OF THE PIGMENT LAYER OF THE IRIs
It is an old observation that in the operation for iridectomy in diabetic
patients, iris pigment is readily set free and makes the aqueous dark and
cloudy, or alternatively, in an operation for cataract a part of the pigment
layer may come away with the lens. The cause of the phenomenon was
first noted histologically by Becker (1883) in his extensive studies on cataract
and was studied in detail by Kamocki (1887–89) who found it to be based
upon an enormous swelling and sponginess of the two layers of the pigmentary
epithelium (Fig. 1975); his histological observations have been amply
confirmed (Deutschmann, 1887; Goerlitz, 1894; Koch, 1899; Reis, 1903;
Uhthoff, 1908; Hoffmann, 1913; Kurz, 1937). Such changes, a swelling,
cystic formation, detachment and necrosis of the pigmentary epithelium,
have been produced experimentally in rabbits by Villani (1934).
1 p. 2156.
T.O.-WOL. III. U
2384 TEXT-BOOK OF OPHTHALMOLOGY
This is a fairly common phenomenon in diabetes. The pigment cells
themselves are enlarged and elongated, so that they appear to become
proliferated to form several layers, the nucleus with the pigment is pressed
to one side of the cell body which thus becomes depigmented, vacuoles appear
and eventually the cells break down to form cyst-like vesicles of considerable
size (10 × 1.5 mm.) containing fragmented nuclei, free pigment, and
sometimes particles of glycogen. The iris stroma is usually normal, but
may be oedematous (Becker, 1883) or atrophic (Deutschmann, 1887). The
changes are usually confined to the iris but the depigmentation and vesicu-
lation may extend backwards over the ciliary epithelium (Kamocki, 1892)
and may even affect the ora serrata (Deutschmann, 1887; Kurz, 1937).
Fig. 1975.--THE PIGMENTARY EPITHELIUM or THE IRIs IN DIABETEs
( × 55) (Parsons).
These phenomena, while very evident pathologically, produce changes
which can be detected with the slit-lamp. The general disturbance of the
pigmentary cells may produce a scattering of free pigment which may be
seen on the anterior surface of the iris, particularly after operations (Augstein,
1904; Vossius, 1910). The cystic and depigmented areas on the posterior
surface can be appreciated as lacunae by retro-illumination (Villani, 1934);
while the swelling of the cells may be seen at the pupillary margin under
high magnifications (x 40) where the pigment border is exuberant and
its surface shows a multitude of tiny pits (Vogt, 1922).
2. GLYCOGENOUs DEGENERATION
A glycogenous degeneration affecting especially the pigmentary
epithelium is an almost constant accompaniment of the changes just
described (Reis, 1903); but frequently the phenomenon is more widespread,
affecting the stroma and particularly the sphincter and dilatator muscles,
and more rarely the ciliary muscle and choroid (Hoffmann, 1913). It has
also been met with in the corneal epithelium, the corneal nerves, the retina,
and the optic nerve (Best, 1905; Hoffmann, 1913).
It is to be noted that such a glycogenous degeneration is also met with in chronic
inflammations, intra-ocular tumours (Hoffman, 1913; Maruo, 1913) and leukaemia
(Verderame, 1910).

DISEASES OF THE UVEAL TRACT 2385
3. RUBEOSIS OF THE IRIS
This is a rare condition characterized by a peculiar non-inflammatory
vascular proliferation affecting particularly the iris in diabetics, and usually
associated with glaucoma. The
condition was first described by
Salus (1928), who applied the term
employed by v. Noorden (1927) to
describe the reddish appearance of
the skin of the face in diabetics
due to enlargement of the inter-
mediate portion of the capillary
loops; and several other cases
have appeared in the literature
since his original paper (Axenfeld,
1929; Villani, 1934; Waldstein,
1934; Sallmann, 1935; Lawrence
and Levy, 1936; Motolese, 1936;
Kurz, 1937). The appearance of
the iris is very striking, the surface
being festooned with new-formed
vessels, especially near the sphincter
Fig. 1976.-RuBEosis DIABETICA
(Kurz, A. f. Aug.).
region where the individual branches anastomose to give the appearance of
a ring (Fig. 1976). On gonioscopic examination Kurz (1937) found large
Fig. 1977. RuBEosis
DLABETICA.
Peripheral synechiae seen with
the gonioscope (Kurz, A. f. Aug.).
vessels running peripherally into the angle,
where they broke up again into innumerable
branches connecting directly with Schlemm's
canal and forming extensive peripheral
synechiae (Fig. 1977). A recurrent hyphaema
is common and quite frequently a recalcitrant
glaucoma results which yields only temporarily
or not at all to operative treatment. A peculiar
feature is that the vessels sometimes tend to
disappear spontaneously, but the glaucoma
associated with a recurrent hyphaema may
rapidly destroy vision, while the eye may have
to be removed because of pain.
A histological examination conducted by
Kurz (1937) showed that the new vessels were
unassociated with inflammation or with newly
formed tissue, but lay in and upon the iris itself.
The choroidal vessels were also enormously
dilated, and in both regions there were
numerous extravasations of blood into the


U 2
2386
TEXT-BOOK OF OPHTHALMOLOGY
tissues.
A frequent concomitant is a haemorrhagic diabetic retinopathy;
and in association with the anomaly in the iris a similar proliferation of new
vessels has been noted on both surfaces of the cornea (Lawrence and Levy,
1936) and on the optic disc (Kurz, 1937).
4. A lipaemia of the vessels of the uveal tract has been histologically established
by Reis (1903) and Hoffmann (1913).
Augstein. LXXVI dewt. Naturforsch.-Wers.,
Breslau, 1904.
Axenfeld. K. M. Aug., lxxxiii, 334, 1929.
Becker. Zur Anat. d. gesunden u. kranken
Linse, Wiesbaden, 1883. *
Best. B. O. G. Heidel., xxxii, 315, 1905.
Deutschmann. A. f. O., xxxiii (2), 229, 1887
Goerlitz. Diss., Freiburg, 1894.
Hoffmann. A. f. Aug., lxxiii, 261, 1913.
Kamocki. A. f. Aug., xvii, 247, 1887; xxv,
209, 1892.
Koch. Diss., Marburg, 1899.
Kurz. A. f. Aug., cz, 284, 1937.
Lawrence and Levy. Brit. J. O., xx, 198,
1936.
Maruo. Diss., München, 1913.
Motolese. Atti Cong. S. O. Ital.., xxxiii, 439,
1936.
v. Noorden. Zuckerkrankheit, Berlin, VIII,
1927.
Reis. A. f. O., lv., 437, 1903.
Sallmann. Z. f. Aug., lxxxv, 262, 1935.
Salus. Med. Kl., i, 256, 1928.
Spalding and Curtis. Boston Med. Surg. J.,
cxcvii, 165, 1927.
Uhthoff. B. O. G. Heidel., xxxv, 64, 1908.
Verderame. A. f. path. Anat., co, 667, 1910.
Villani. Am... dº Ott., lxii, 763, 881, 977, 1934.
Vogt. Z. f. Aug., xlix, 64, 1922.
Vossius. B. O. G. Heidel., xxxvi, 333, 1910.
Waldstein. A. of O., xii, 419, 1934.
Leber. A. f. O., xxxi (4), 183, 1885.
(c) DIABETES INSIPIDUs
An irido-cyclitis in diabetes insipidus is a great rarity (Stoewer, 1912; Hommel,
1931). In the two reported cases the disease occurred in young adult males, there was
marked ciliary injection, little exudate and the pupil dilated readily ; in Hommel’s
case a few yellowish-grey nodules appeared near the periphery of the iris, in Stoewer’s
an associated optic neuritis was present, and in both no alternative aetiology could be
demonstrated, while after lasting some months the condition cleared up with an
improvement in the diabetic state. The association is suggestive, but not proven.
Hommel. K. M. Aug., lxxxvii, 492, 1931. Stoewer. K. M. Aug., l (2), 624, 1912.
(d) RENAL DISEASE
We have already seen that advanced arteriosclerotic changes through-
out the uveal tract are apt to occur in renal disease, and that as a consequence
intercurrent infections (Löhlein, 1907) or post-operative inflammations
(Gilbert, 1921 ; Mylius, 1934) are prone to occur in such subjects. It is
probable that nephritis acts in this way as an adjuvant factor in the aetiology
of such conditions rather than as a directly causative agent, as was suggested
by the older writers (Leber, 1885; v. Michel, 1900–06; Baum, 1902). In
advanced renal cases, however, the general sclerotic process in the choroid
may show characteristic features which entitle it to be considered as a
clinical entity.
1 p. 2371.
DISEASES OF THE UVEAL TRACT 2387
ALBUMINURIC CHOROIDITIS
In cases of advanced nephritis, always associated with albuminuria
and usually with an extremely high blood pressure, choroidal changes may
become ophthalmoscopically visible to constitute a clinical picture of
albuminuric choroiditis (Figs. 1978–79). These appearances are usually a
terminal event in a protracted illness and are frequently, but not invariably,
masked by an associated albuminuric retinitis with retinal cedema, and
are clinically evident as changes in the pigmentary epithelium which can be
ascribed to vascular sclerosis in the choroid underneath (Siegrist, 1899;
Elschnig, 1904; Köppl. 1927; Koyanagi, 1928; A. Fuchs, 1930).
Fig. 1978. –ELscHNIG's Spots IN Fig. 1979.-SIEGRIST's Spots IN
ALBUMINURIC Choroiditis. ALBUMINURIC CHoRoibitis.
Clinically the lesions can be classified in four groups.
1. Pale yellow or reddish plaques sometimes surrounded by pigmentary
deposits situated in the periphery of the fundus.
2. Black isolated flecks of pigment associated with a bright yellow or
red halo (ELSCHNIG's spots, 1904) (Fig. 1978).
3. Chains of pigmented spots arranged like a string of beads along a
white and sclerosed choroidal vessel (sIEGRIST's spots, 1899) (Fig. 1979).
4. Large patches of chorio-retinal atrophy.
Pathologically in these eyes there is an extensive lymphocytic infiltra-
tion in the choroid and sometimes in the external layers of the retina with
advanced peri-arteritis and obliterative sclerosis of the smaller vessels
(Elschnig, 1904; Fuchs, 1930). The peculiar fleck-like lesions are due to a
patchy distribution of the sclerotic process in the chorio-capillaris, the
overlying pigment disappearing in certain areas to give rise to the red or
yellow patches and halos, and accumulating in other areas, sometimes with

2388 TEXT-BOOK OF OPHTHALMOLOGY
the formation of heaped-up bunches of proliferated cells. The chain-like
formations described by Siegrist mark the course of sclerosed choroidal
vessels over which the pigmentary epithelium has become correspondingly
affected. In all cases the prognosis is thoroughly bad—much worse than
that of albuminuric retinopathy. Especially is this so in the patchy oblitera-
tion of the chorio-capillaris which becomes ophthalmoscopically visible as
Elschnig's Spots, an appearance which may be taken as an indication of
impending death. On the other hand the changes in the larger vessels
which give rise to Siegrist's Spots are not of so serious an import and are
compatible with good vision (Köppl, 1927).
More advanced pathological changes occur throughout the uvea
associated with an albuminuric retinopathy, but the clinical picture is
obscured by retinal oedema, infiltration and haemorrhage—gross sclerosis and
advanced hyaline, amyloid and fatty degeneration of the vessels (Theodor,
1887; Ewetzky, 1898; Hofmann, 1902; Schmidt, 1905; v. Michel, 1906;
Lauber and Adamūk, 1909; Ginsberg, 1912; Gilbert, 1921; and others).
Baum. Diss., Freiburg, 1902. Leber. A. f. O., xxxi (4), 183, 1885.
Elschnig. Wien. med. W., liv, 445, 494, 1904. Löhlein. Arb. aus d. path. Instit. z. Leipzig,
Ewetzky. K. M. Aug., xxxvi, 381, 1898. H. 4, 1907.
Fuchs, A. K. M. Aug., lxxxiv, 39, 1930. v. Michel. Münch. med. W., xlvii, 853, 1900.
Gilbert. Münch. med. W., lxviii, 979, 1921. Z. f. Aug., xv, 13, 1906.
Ginsberg. A. f. O., lxxxii, 1, 1912. Mylius. B. O. G., Heidel., 1, 138, 1934.
Hofmann. A. f. Aug., xliv, 339, 1902. Schmidt. Zb. path. Anat., xvi, 49, 1905.
Köppl. Z. f. Aug., lxii, 131, 1927. Siegrist. IX International Cong., Utrecht, 36,
Koyanagi. K. M. Aug., lxxx, 436, 1928. 1899.
K. M. Aug., xciv, 335 1935. Theodor. Beit. 2. path. Anat. d. Auges bei
Lauber and Adamük. A. f. O., lxxi, 429, Nierenleiden, Wiesbaden, 1887.
1909.
(e) HEPATIC DISEASE
Prolonged disease of the liver—such as leads to jaundice, cirrhosis or cholangitis—
is well known to give rise to subjective symptoms of hemeralopia and concentric
field defects, and to the development of xerosis of the conjunctiva. Occasionally
degenerative changes are seen in the choroid and retina somewhat resembling those
Seen in primary pigmentary degeneration with diffuse atrophy, but unassociated with
night blindness and field contraction (Hartshorne, 1934). A few pathological studies
occur in the literature correlating these symptoms with degenerative changes in the
uveal tract, which were termed OPHTHALMIA HEPATICA by the older writers. That
such changes were probably related as cause and effect was suggested by the experi-
ments of Dolganoff (1897) who, on ligating the bile-ducts of dogs, found after an
interval of 2 to 3 months, exudative and infiltrative changes in the choroid and
degeneration of the pigmentary epithelium and the retina.
The pathological findings differ very considerably. Baas (1894), in a case of
jaundice of 11 years’ duration, found widespread vascular degeneration of the sclerotic
type throughout the entire uveal tract, atrophy of the stroma with connective tissue
Overgrowth, a round-celled infiltration, and degeneration of the chromatophores
and pigmentary epithelium—a picture which he called CIRRHOSIs of THE CHOROID.
In more acute cases Hori (1895) and Purtscher (1900) found no vascular degeneration
DISEASES OF THE UVEAL TRACT 2389
but changes in the pigment epithelium ; a layer which Koyanagi (1920) found to be
infiltrated with lipoid material in a young boy who had suffered from jaundice for a
year and from hemeralopia for a month.
Baas. A. f. O., I (5), 212, 1894. Hori. A. f. Aug., xxxi, 393, 1895.
Dolganoff. A. f. Aug., xxxiv, 196, 1897. Koyanagi. K. M. Aug., lxiv, 836, 1920.
Hartshorne. Am. J. O., xvii, 945, 1934. Purtscher. A. f. O., 1, 83, 1900.
W. DEGENERATIONS AND ATROPHIES IN THE UWEAL TRACT
1. Senile Changes
Degenerative and atrophic changes are of universal occurrence in the
uveal tract in old age. We have already touched upon the question of how
far such changes can be considered as purely senile," when it was pointed
out that age is not a question of years, for “many grow old before they
arrive at age " (Sir Thomas Browne, 1646); the processes of senescence
should rather be calculated in terms of intensity of living, of heredity, and
of the cumulative effects of rude shocks or quietly progressive toxaemias.
It is only by virtue of their practical universality that certain changes can
be considered as the concomitants of senescence; and the same changes
are frequently seen in early life after disease. Whatever view is taken
of the matter, two main changes
occur in the later years of life
in the uveal tract with great
constancy—an atrophy of the
mesodermal stroma, and a
degeneration and depigmentation
running side by side with an
occasional tendency to prolifera-
tion of the ectodermal epithelium.
1. THE IRIS
(a) ATRoPHY AND DEGENERA-
TION IN THE STROMA
In the stroma of the iris the
general tendency to senile atrophy
is seen in a thinning of the entire
structure and a flattening out of
- - - - - FIG. 1980.-S D -
its delicate architecture with a dis- IG * * * *
appearance of the crypts. This Slit-lamp. In the focal band are seen
occurs especially in the pupillary granules of pigºent indigºing pigmentºry
degeneration. On the left gaps in the
zone, where - the anterior leaf pigmentary epithelium are seen by trans-
with the iris frill and the circulus illumination (Koby, Slit-lamp Microscopy).
* Vol. II, p. 1434.

23.90 TEXT-BOOK OF OPHTHALMOLOGY
minor may entirely disappear. As a result the brown sphincter muscle
becomes visible, especially by retro-illumination, and the pigmented epithe-
lium may shine through, altering the colour of the iris to a greyer or bluer
hue (Fig. 1980). The essential change is in the blood-vessels, for it will be
remembered that the anterior layers of the iris are almost entirely composed
of these, supported by a little connective tissue; they are seen to be
largely converted into white lines, and only a few of them are seen to
Fig. 1981—SENILE Vascular Chasses Is THE IRIs (Rones, Am. J. o.).
contain blood. The connective tissue itself is largely replaced by hyaline
material, which frequently accumulates in the region of the pupillary margin
and behind the sphincter to such a degree that an elevated hyaline ring may
be clearly visible (HYALINE DEGENERATION of THE PUPILLARY MARGIN of
Fuchs, 1884) (Fig. 1982). It is this change which is largely responsible for
the usual presence of senile miosis and rigidity of the pupil.
Pathologically the most prominent feature is the selerosis of the blood-vessels,
the adventitia of which may be so thickened and hyalinized that it is difficult to
differentiate it from the surrounding hyalinized connective tissue: the media partakes
in this change, but the lumen remains patent (Fig. 1981) (Kerschbaumer, 1888–92;


DISEASES OF THE UVEAL TRACT 2391
Attias, 1912; Monauni, 1919; Rones, 1938). In the stroma itself there is an increased
development of collagenous fibres (Fuchs, 1885), and a general hyalinization of the
stroma (Meller, 1904), a process particularly evident near the pupillary margin and
behind the sphincter (Fuchs, 1885; Meller, 1904; Seefelder, 1909; Axenfeld, 1911;
Attias, 1912; Rones, 1938) (Fig. 226). Meller (1904) suggested that this hyaline
deposit was laid down by the endothelium, but it seems more probable that it is
secondary to the vascular changes which initiate a similar degeneration in the sur-
rounding tissue (Seefelder, 1909). The muscle fibres of the sphincter are relatively
little changed, but the inter-fascicular septa become hyalinized ; and a hyaline
degeneration of the dilatator fibres is more common. The endothelium covering the
FIG. 1982.-SENILE ATRoPHY of THE IRIs.
Atrophy of the pigmentary epithelium, and hyalinization of the connective
tissue beneath the sphincter (Rones, Am. J. O.).
anterior surface may become prominent, a phenomenon seen more distinctly in patho-
logical conditions, and it may even deposit a fine hyaline membrane and lose its
cellular form (Figs. 1828 and 1829). Fatty or calcareous degeneration, however, in
simple senile conditions, is rare.
As a rule these atrophic changes are general over the iris, but frequently
localized areas may be especially affected. Most commonly this is seen near
the pupillary margin when the atrophy of the tissues may result in the
formation of a furrow—the sulcus CIRCUM-MARGINALIs of Vogt (1921). At
other times patches of varying size may appear on the surface of the iris
where all that is left of the stroma are a few fibres looking as if they had
been teased out so that the pigment epithelium is exposed, occasionally
giving the appearance of a pigmented tumour. Sometimes these fibrils

2392 TEXT-BOOK OF OPHTHALMOLOGY
become free, when they curl and float in the anterior chamber, a condition
which may be seen all round the iris (Dollfus, 1927); and at other times
all the mesodermal tissue may disappear entirely, forming a partial coloboma
and exposing completely the retinal layers, a phenomenon usually confined
to the peripheral area so that the sphincter region is left (Koby, 1930).
The trabeculae of the angle of the anterior chamber share in the senile
sclerosis to a marked degree (Fig. 1984). Here the deposition of collagenous
material may be very evident and the whole of the delicate meshwork of the
angle tends to become thickened and more impervious by the laying down
of a considerable amount of hyaline and fibrous tissue in the lamellae
(Henderson, 1907). It is probable that this process figures in the aetiology
of glaucoma.
(b) DEPIGMENTATION AND PROLIFERATION IN THE EPITHELLAL LAYERS
Some degree of depigmentation of the epithelial layers of the iris occurs
in most persons over 50, 90% of persons over 60, and is constant over
80 years of age; it is usually more pronounced in light irides than in dark
(Hinnen, 1921; Kaminskaja-Pavlova, 1927), and affects particularly the
Fig. 1983–SENILE PRodiºration of PIGMENTARY EPITHELIUM or IRIs
(Rones, Am. J. O.).
lower part of the iris (Axenfeld, 1911; Höhmann, 1912; Koeppe, 1917;
Soewarno, 1919; Vogt, 1922; Jess, 1923). It is most easily seen as it
affects the pupillary margin. Here the pupillary ruff becomes ragged,
moth-eaten and irregular in patches, or cuneiform sectors and radial fissures
devoid of pigment may appear, untileventually the whole of the pigment may
be lost; such cases are always associated with a considerable degree of
atrophy of the stroma and hyaline degeneration of the pupillary border, so
that the normally pigmented ruff is replaced by a grey pellucid border. At

DISEASES OF THE UVEAL TRACT 23.93
the same time examination of the iris by light reflected from the lens shows
that the marginal defects may be carried far down the posterior surface,
and may reveal translucent areas indicating irregular lacunae where the
pigment is lacking (Fig. 1980). These changes are usually associated with
a scattering of the pigment over the structures surrounding the anterior
chamber, the anterior surface of the iris being sprinkled with granules as
if by black pepper, as well as the capsule of the lens, the posterior surface
of the cornea, and particularly the meshes of the pectinate ligament. It
will be seen at a later stage that this deposition has been associated with the
aetiology of glaucoma (Levinsohn, 1922),
At other times the opposite process of proliferation of the pigment
epithelium may occur, and at certain points of the pupillary margin irregu-
larities are formed by deeply pigmented cellular proliferations forming
isolated dark excrescences (Fig. 1983).
2. THE CILIARY BODY
Very similar changes occur in the ciliary body (Donders, 1855; Müller,
1856; Kuhnt, 1881; Fuchs, 1884; Kerschbaumer, 1888; Hess, 1910;
Attias, 1912; Rones, 1938). The ciliary processes themselves increase in
FIG. 1984.—SENILE DEGENERATION OF THE CILIARY BoDY.
Atrophy of the ciliary muscle with increase of connective tissue between the fibres.
Complete hyalinization of the ciliary processes. The pectinate ligament is sclerosed
and in it are enmeshed numerous pigment granules (Rones, Am. J. O.).

2394 TEXT-BOOK OF OPHTHALMOLOGY
bulk and become elongated and more branched, coming right up to the
margin of the lens and running inwards and forwards towards the iris
(Fig. 1984). The posterior chamber is thus narrowed and the root of the
iris is pushed towards the cornea, thus embarrassing the already sclerosed
filtration angle and perhaps tending to predispose to glaucoma. Corre-
sponding to these macroscopic changes, the stroma of the processes becomes
denser, the loose cellular tissue giving place to more compact connective
tissue. Hyaline degeneration then appears, at first in the walls of the
blood-vessels, which become enormously thickened as those in the iris, and
then in the stroma, so that in advanced cases the stroma of the processes
may be converted into homogeneous hyaline masses, in which areas of fatty
(Attias, 1912; Hanssen, 1922–23) or calcareous (Kadletz, 1920) degeneration
may be seen (Fig. 1984). The elastic tissue in the ciliary body also increases,
more especially in the posterior parts and immediately anterior to the ciliary
muscle, in which latter situation elastic fibres may form a ring on the inner
side of the angle of the anterior chamber, a phenomenon most easily seen
in myopic eyes of the aged in whom the ciliary muscle is poorly developed
(Stutzer, 1898 ; Prokopenko, 1903; Herbert, 1929). At the same time the
basal hyaline membrane becomes thicker and loses its homogeneous appear-
ance, the outer part frequently becoming granular and showing calcium
deposits, and the fibres of the inner network becoming thicker (Müller,
1872).
A considerable degree of atrophy occurs in the ciliary muscle, where the
fibres are thinner, the nuclei sparser, and the inter-fibrillar tissue becomes
increased and eventually hyalinized with occasional deposits of fatty
droplets or calcareous granules (Herbert, 1929). In more advanced cases
atrophic and degenerative changes affect the muscle fibres themselves, which
may become impregnated with fatty droplets. The earliest changes in the
muscle are seen, according to Attias (1912), about the age of 40 so that the
phenomena may play a considerable part in the development of presbyopia ;
while it is considered by some to be a factor in the aetiology of glaucoma
owing to the failure of the muscle to open out adequately the drainage
passages at the angle of the anterior chamber (Herbert, 1929).
In the ciliary epithelium both layers are affected. In a general sense
the non-pigmented epithelium shows degenerative changes, the most common
of which is the deposition of fatty droplets, an occurrence also seen in the
pigmented epithelium after it has been bleached (Attias, 1912; Busacca,
1927) : these changes are probably dependent on the sclerosis of the blood-
vessels underneath.
The most interesting change, however, is the occurrence of a SENILE
HYPERPLASIA OF THE NON-PIGMENTED LAYER OF CELLs, especially on the
pars plana, in which process the pigmented cells may take part (Fig. 1985).
This hyperplasia, which is often pre-senile, starting at the age of 40 (Kersch-
baumer, 1888), may be extreme in degree, but, causing no clinical symptoms,
DISEASES OF THE UVEAL TRACT 23.95
-
Fig. 1985.-SENILE HYPERPLAs.I.A of CILIARY EPITHELIUM.
Cross section of a ciliary process showing both pigmented and non-pigmented
epithelium participating in the hyperplasia (Rones, Am. J. O.).
Fig. 1986.-SENILE CYSTIC FoEMATION IN CILIARY BoDY.
Section of a ciliary process showing marked proliferation of the non-pigmented
epithelium with commencing cyst-formation (Rones, Am. J. O.).




2396 TEXT-BOOK OF OPHTHALMOLOGY
is usually observed unexpectedly in histological studies. The earliest change
is the appearance of karyokinesis and proliferation in the non-pigmented
cells which become convex towards the vitreous and form either large flat
excrescences in a festoon-like arrangement, or pedunculated or sessile knobs,
or wart-like growths (Fig. 1986) (Kuhnt, 1881; Kerschbaumer, 1888;
Hanke, 1899; Salzmann, 1912; Archangelsky, 1926; Rones, 1938).
Quite frequently the pigmented epithelium participates in the process and
proliferating pigmented cells can be seen interspersed with the non-pig-
mented cells (Fig. 1985). At other times the central cells in the knob-like
elevations degenerate so that cysts are formed which may reach considerable
size l ; and at other times a definite and massive tumour is formed which
may even become clinically apparent.”
Although it is undoubtedly the case that the epithelial hyperplasia is a
physiological senile phenomenon, it is not so certain that the formation of
cysts and tumours can be placed in the same category. The hyperplasia
is presumably due to the stimulation caused by irritative substances in the
sclerotic processes of senescence ; and it may be that the frankly neo-
plastic changes are merely an extension of the same process. Moreover, it
is probable that no sharp line of demarcation can be drawn between these
changes and the more rapid hyperplastic changes characterized by the
formation of tubular outgrowths of cells which percolate into a cyclitic
membrane in cases of long-standing inflammation.”
3. THE CHOROID
In the choroid, as in other parts of the uveal tract, the essential senile
changes occur in the blood-vessels (Kerschbaumer, 1892; de la Fontaine
Verwey, 1927; and others). In the arteries there is a fatty deposition in
the intima, a degeneration, largely fatty in nature, of the muscular coat
which is finally replaced by fibrous tissue, until eventually the whole vessel
wall may become hyalinized, the lumen being much restricted or even
obliterated, so that the vessel takes on the appearance of a white opaque
tube with thick striated walls. These changes are usually most accentuated
in the posterior region ; and they are accompanied by somewhat similar
changes in the veins—a thickening of the walls due to increase of connective
tissue, and a loading of the perivascular spaces with endothelial cells packed
with fatty droplets. These changes are reflected in the chorio-capillaris,
the walls of the small vessels becoming thickened, the lumen narrowed
or occluded, and the intercapillary spaces narrowed or obliterated. Since
these capillary changes are secondary to degeneration in the supplying
arteries they tend to occur in patches, areas of atrophy being interspersed
with areas wherein the capillaries appear to be compensatorily dilated.
This patchy sclerosis may be quite evident ophthalmoscopically, especially
in lightly pigmented individuals, when the thickening in the arterial walls may
1 p. 2440. * p. 2447. 8 p. 2173.
DISEASES OF THE UVEAL TRACT 2397
become visible; pathologically the corresponding area of the chorio-capillaris
is found to be atrophied producing an irregularity with many variations in
calibre in the capillary network. All these changes are particularly evident
near the posterior pole, and in the region immediately round the disc they
may together constitute the picture of SENILE CIRCUM-PAPILLARY CHOROIDAL
ATROPHY (THE SENILE PERI-PAPILLARY HALO) (Bücklers, 1928).
These degenerative processes are naturally reflected in the tissues
dependent upon the choroid for nourishment. As a result changes partly
of a degenerative and partly of a proliferative nature are evident in the
pigment epithelium of the retina, the cells of which deposit colloid material
on the retinal portion of Bruch's membrane (COLLOID BODIES or DRUSEN); 1
while in the retina itself, especially in the macular region, secondary degenera-
tive processes may appear (SENILE DEGENERATION OF THE MACULA).”
The pigmentary epithelium is an early sufferer from the atrophic changes
in the choroid, both regressive and hyperplastic changes occurring earlier
and more extensively in the peripheral region of the fundus (Donders, 1855;
Müller, 1856–72 ; Kerschbaumer, 1892; Coats, 1905; Attias, 1912;
Monauni, 1919). They may, indeed, be said to be a pre-senile condition,
for they are seen to some extent, according to Kerschbaumer (1892), in one-
third of eyes examined between 40 and 50, and in two-thirds between 50
and 60 years of age (Fig. 2351). The cells lose their hexagonal shape,
becoming swollen and elongated, so that the layer loses its regular appearance.
The pigment granules lose their normal rod-shaped form and become small
and round, and are irregularly distributed in the cells ; some parts are devoid
of them, in others they are heaped up, while in yet others complete
depigmentation may occur. The cytoplasm becomes vacuolated and
infiltrated with fat, the nuclei stain feebly and disappear, and finally the
cells disintegrate, scattering their pigment over the basal membrane. Such
irregularity and clumping of pigment is frequently visible ophthalmos-
copically.
Coincidentally, as occurs in the iris and ciliary body, an opposite process
of hyperplasia may occur. Many cells contain two nuclei, and in an irregular
process of hyperplasia new ill-formed cells appear. Associated with this
proliferation is an increase in the deposition of hyaline material on the
cuticular part of Bruch's membrane (Hanssen, 1929–30), which in places is
so accentuated as to form macroscopically visible excrescences (colloid bodies
or drusen).
Archangelsky. Russ. O. J. (see K. M. Aug., Busacca. K. M. Aug., lxxviii, 529, 1927.
lxxvii, 739, 1926). Coats. R. L. O. H. Rep., xvi, 164, 1905.
Attias. A. f. O., lxxxi, 405, 1912. Dollfus. Bull. S d’O. Paris, xxxvii, 170, 1927.
Axenfeld. B. O. G. Heidel., xxxvii, 255, Donders. A. f. O., i (2), 107, 1855.
1911. Fuchs. A. f. O., xxx (3), 123, 1884; xxxi (3),
Browne, Sir Thomas. Pseud. Epi., iv, 217, 39, 1885 ; likviii, 534, 1908.
1646. Hanke. A. f. O., xlvii (3), 474, 1899.
Bücklers. A. f. O., czki, 243, 1928.
1 p. 2747. * p. 2372.
2398 TEXT-BOOK OF OPHTHALMOLOGY
Hanssen. K. M. Aug., lxviii, 391, 1922; Levinsohn. K. M. Aug., lxi, 174, 1918;
lxx, 732, 1923; lxxxiii, 108, 1929. lxviii, 471, 1922.
2. f. Aug., lxxii, 360, 1930. * Meller. A. f. O., lix, 221, 1904.
Henderson. O. Rev., xxvii, 255, 1907. Monauni. A. di Ott., xxv-vi, 263, 1919.
PIerbert. T. O. S., xlvii, 159, 1927. Müller, H. A. f. O., ii (2), 1, 1856.
Brit. J. O., xiii, 289, 337, 1929. Gesawsmelte w. hinterlassene Schriften,
Hess. A. f. Aug., lxvii, 341, 1910. Leipzig, 1872.
Hinnin. Z. f. Aug., xlv., 129, 1921. Prokopenko. A. f. O., lv, 94, 1903.
Höhmann. A. f. Aug., lxxii, 60, 1912. Rones. Am. J. O., xxi, 239, 1938.
Jess. K. M. Aug., lxxi, 175, 1923. Salzmann. Amat. w. Histol. d. menschlichen
Radletz. K. M. Aug., lxiv, 566, 1920. Awges, Wien, 1912.
Raminskaja-Pavlova. Russ. A. O., iii, 476, Seefelder. Z. f. Aug., xxi, 289, 1909.
1927. . Soewarno. K. M. Aug., lxiii, 275, 1919.
Kerschbaumer. A. f. O., xxxiv, 16, 1888; Stutzer. A. f. O., xlv., 328, 1898.
xxxviii, 127, 1892. Verwey, de la Fontaine. K. M. Aug., lxxix,
Koby. Slit-lamp Microscopy of the Living 148, 1927.
Eye, London, 1930. - Vogt. Atlas, Berlin, 1921.
Roeppe. A. f. O., xciii, 151, 173, 275, 1917. A. f. O., cvii, 196; cviii, 182; ciz, 154,
Ruhnt. B. O. G. Heidel., xiii, 49, 1881. 404, 1922.
Wilmer. A. of O., i, 42, 1929.
2. Pathological Atrophies
In addition to senile atrophy, which we have just considered, atrophic
and degenerative processes are of common occurrence in the uveal tract.
These may be classified as (a) secondary atrophies, due to (1) inflammatory
processes, (2) trauma, (3) ischaemia, (4) glaucoma, (5) myopia, or (6) neuro-
genic influences, as in tabes, or (b) primary or essential atrophies.
(A) Secondary Atrophies
1. POST-INFLAMMATORY ATROPHY
POST-INFLAMMATORY ATROPHY is a common condition, the essential
features of which we have already discussed in dealing with uveitis.
(a) In the Iris
Any inflammation of long standing is followed by atrophic changes of
a more or less pronounced degree in the iris, the pathology of which have
already been described. Usually this is diffuse and affects the whole tissue,
in which case the iris loses its delicate relief and lustre, and becomes grey and
dull, resembling a piece of moist blotting-paper. The final result is a dis-
coloured flat iris, wherein the normal blood-vessels are replaced by sclerosed
white lines, and on the surface of which may be seen clumps of dilated new
vessels, aggregations of pigment and occasionally cholesterol crystals (Fig.
1987). f
In contra-distinction to senile atrophy, if the iris has been immobilized
for a long time by adhesions, the sphincter, like any other disused muscle,
eventually tends to suffer an atrophy of disuse. The atrophic process,
which, like senile atrophy, is essentially due to sclerosis of the vessels,
DISEASES OF THE UVEAL TRACT 2399
with the added factor of pressure by
the contraction of new-formed fibrous
tissue as the result of the organization
of exudates, causes a general degenera-
tion of the stroma cells which first
lose their long ramifying processes
and then disappear entirely in large
numbers. In extreme cases actual
lacunae and holes appear in the iris,
especially near the thin ciliary margin,
a process perhaps accentuated by a
rise of pressure in the posterior chamber
caused by an occluded pupil (Oblath,
1899; Franck, 1903; Harms, 1903).
The pigmentary epithelium is
usually very resistant, but, as occurs
in senile conditions, the pigment may
eventually become irregular in patches,
disappearing in some places and be-
coming heaped up in others, and in
extreme cases it may disappear entirely
so that a red reflex may be obtained
Fig. 1987.-Post-INFLAMMATory
VARIcose VEssels on IRIs.
(Ballantyne and Michaelson, Brit. J. O.).
through the iris. Alternatively the pigment layer may seem to become
more exuberant, appearing round the pupillary margin and extending
Fig. 1988—ATRoPHIc EcºRoPros of Uvea. Pig MENT.
Note the distortion of the sphincter muscle (Parsons).
T.O.-WOL. III.


2400 TEXT-BOOK OF OPHTHALMOLOGY
downwards over the anterior surface of the iris, sometimes almost to the
ciliary root (Stern, 1914). This phenomenon of ECTROPION OF THE PIGMENT
LAYER" (Lawford, 1886; Nettleship, 1886) is due partly to atrophy of the
stroma (Fuchs, 1883–1920), partly to the contraction of organized exudates
on the surface of the iris (Kries, 1876; Birnbacher and Czermak, 1886),
and partly to a hyperplasia of the pigment layer itself (Gallenga, 1905;
Lohmann, 1910; Siegrist, 1912) (Fig. 1805); and in pronounced cases
where there is much contraction of fibrous tissue, the distortion bends over
the entire structure of the iris so that the sphincter appears hook-shaped
on radial section (Fig. 1988). Such an ectropion frequently indicates a
serious affection of the posterior segment.
Histologically the atrophic process affects all the tissues. The blood.
vessels may show a high degree of sclerosis, giving the usual picture of thick
hyaline rings (Fig. 1959), or may have almost entirely disappeared, the only
vascularization present being that by wide vessels of new formation
(Fig. 1987). Most of the stroma may be filled with flat cells with stunted
processes which replace the chromatophores, amongst which lie masses of
hyaline material ; but the muscle fibres usually persist longer before they
are ultimately replaced by hyalinized connective tissue. The endothelium
may be unchanged but it is frequently proliferated into several layers and
may show warty excrescences (Fig. 1828); and occasionally it secretes a
hyaline membrane which appears to be continuous with Descemet's
membrane (Fig. 1829) (Wagenmann, 1892).
Not infrequently the atrophy is limited to circumscribed areas. In the
ordinary types of infective irido-cyclitis (streptococcal, etc.) such patches are
found either near the greater or lesser circle of the iris or near the pupillary
margin, that is, in the regions where capillary anastomoses are greatest
(Mayou, 1910–25) (Fig. 1989, Plate XLIV). It is probable that they
represent the site of embolic lesions, and correspond with the common
type of circumscribed choroiditis. The most marked patches, however, are
those which we have already noted following the nodular and localized lesions
of tubercle, syphilis, leprosy, herpes, small-pox, and other conditions. In
this event the atrophy may be partial or complete (Fig. 1891, Plate XXXVIII),
the ectodermal layers being apparent either blackly pigmented or greyish
in colour, either because the pigment has disappeared or because they are
covered with fibrous tissue ; while exceptionally both layers may disappear
so that a hole in the iris is formed.
Such a hole was described by Bietti (1933) after small-pox. The pupil was
occluded and the tension high ; and he concluded that the final rupture was mechanical
owing to pressure in the posterior chamber. A hyaline membrane covered the angle
of the anterior chamber and extended through the hole to spread over the ciliary
body. De la Vega (1923) reported the development of atrophic holes in syphilis.
1 p. 2185.
PLATE XLIV.
ATRoPHIEs of THE Uvea.
Fig. 1989.-Post-INFLAMMAtoRY ATRophy Fig. 1990.-GLAU comatous ATRophy.
of THE IRIs (after Irido-cyclitis). (After an iridectomy; note marked
pigmentary degeneration).
Fig. 1992.-EARLY Gyrate ATRophy. Fig. 1993.
LATE GvRATE ATRophy.
[To face p. 2400.


DISEASES OF THE UVEAL TRACT 2401
(b) In the Ciliary Body
In the ciliary body similar atrophic changes follow prolonged inflam-
mation. There is atrophy of all the tissues which are replaced by a connective
tissue hyperplasia, frequently showing hyaline degeneration. The vessels
become sclerosed, and the muscular fibrillae are thinned and interwoven with
hyalinized connective tissue, while the whole ciliary body is flatter and
smaller. The processes are reduced in number, thinned and shrunken,
frequently being drawn out into the vitreous (Figs. 1812–13). When marked
in degree these changes are associated with a diminished intra-ocular tension
and consequent malnutrition of the whole eye.
(c) In the Choroid
Two types of post-inflammatory atrophy are found in the choroid. A
choroiditis, as we have seen, finds its natural termination in the replacement
Fig. 1994.-ExTREME Chorro-RETINAL ATRoPHY AFTER AN INFLAMMATony LEsion.
(x 28.) (Parsons).
of the normal vascular tissue by fibrous tissue, which eventually forms a mass
in which the retina and choroid are fused, and which may subsequently
undergo hyaline degeneration or even ossification (Fig. 1994). Such fibrous
scars frequently associated with pigmentary proliferation, and sometimes with
complete disappearance of all the tissue so that the sclera is exposed, form
the usual ophthalmoscopic picture of the “ atrophic stage" of a choroiditis."
Occasionally such a process may be very widespread, so that an almost
complete atrophy of the choroid and retina results with disastrous visual
results (Fig. 1991, Plate XLIV) (Paton, 1930). In such cases the chorio-
capillaris entirely disappears and only the more gross choroidal vessels
remain, while the sole ophthalmoscopic evidences of the existence of the
retina are the fine threads of retinal vessels and a few scattered pigment
granules. The presence of the large choroidal vessels differentiates this
condition from choroideremia,” a condition usually considered as a congenital
anomaly ; it is to be remembered, however, that it has been claimed that
1 p. 2203. * Vol. II, p. 1329, Plate XV, Fig. 1217.

2402 TEXT-BOOK OF OPHTHALMOLOGY
choroideremia is not a congenital absence of the choroid but represents
the ultimate stage of its post-natal pathological dissolution (Bedell, 1937).
Alternatively after an exudative cyclilis when the globe has shrunken,
the choroid is pulled and folded up by cicatricial organized cyclitic exudates.
A process of atrophy rapidly sets in, excessive quantities of new fibrous
tissue being formed, which may ultimately undergo hyaline degeneration
and ossification."
2. Traumatic ATRoPHY
The effects of trauma will be considered in a later section ; but it may be well to
note here the marked degree of atrophy and depigmentation which may be associated
with a dislocated or sub-luxated lens (Santori, 1929, and others). From his observa-
Fig. 1995, Atrophy or THE IRIs AND Pianºst PRourºnation.
Proliferation of pigment with ectropion in glaucoma (Castroviejo, A. of 0.).
tions on a bilateral case, Velhagen (1927) concluded that such a condition was associated
with an atrophy of the zonule, ciliary body, and choroid, and was part of a symptom-
complex comprising an anterior uveal dystrophy.
3. Ischiºmic ATRoPHY
An atrophy of the uveal tissues ensues when the blood supply to a particular
area is diminished or cut off, a process best seen in the choroid. It occurs, for example,
if the posterior ciliary arteries are cut experimentally,” or if the supplying choroidal
vessels are sclerosed and obliterated by disease.”
4. Glaucomatous ATRoPhy
An extreme degree of atrophy of the entire uveal tract is the usual condition in
long-standing glaucoma whether congenital or acquired. It presumably results from
p. 2423. * p. 2102. * p. 2107.

DISEASES OF THE UVEAL TRACT 2403
continued pressure and ischaemia. As a rule the iris is uniformly thinned (Fig.
1995) and ectropion of the pigment layer is common (Kries, 1876; Birnbacher and
Czermak, 1886; Licsko, 1923). As a rule the process is widespread, but small spots
of intense atrophy may occur in the stroma and defects in the pigment epithelium
may show a patchy distribution, while the process may be evident only in large isolated
plaques. The atrophy is almost invariably associated with much pigmentary dis-
turbance * (Fig. 1990, Plate XLIV), and will be dealt with more fully in the chapter
on glaucoma.
5. MYOPIC ATROPHY will be dealt with under the heading of myopia in the following
volume.
6. NEUROGENIC ATROPHY
An atrophy and depigmentation of the iris of neuropathic origin is seen
after lesions of the ciliary ganglion and in neuro-syphilis, especially associated
with TABEs (Dupuy-Dutemps, 1905; Igersheimer, 1918; Behr, 1921 ;
Kaminskaja-Pavlova, 1927; Koby, 1930). It would seem that the causative
lesion must be in the peripheral neuron and not central. Perhaps the earliest
sign is a slight depigmentation, which is appreciable only in dark irides.
Thereafter atrophy of the stroma develops, sometimes generalized and
sometimes patchy, and usually associated with pupillary paresis ; it is
characterized by thinning of the trabeculae which appear as whitish
filaments, effacement of the crypts, and depigmentation of the retinal layers
especially near the pupil. When it is patchy in distribution it is usually
associated with an irregular pupil, the part of the pupil corresponding to the
atrophic zone having the greatest radius of curvature. There is some doubt
as to whether the atrophy, which must depend on a lower neuron lesion, and
the disturbance of the pupillary reflex in tabes are cause and effect, or whether
they are two associated but parallel phenomena. Dupuy-Dutemps (1905)
considered that the atrophy runs pari passu with the pupillary paresis ; but
Lodato (1924) claimed that it may precede paresis, and may indeed be a very
early premonitory sign of tabes ; Vancea (1931) found that it was not
necessarily associated with loss of the pupillary reflexes, nor with irregularity
or inequality of the pupil ; while McGarth (1932) considered that the paresis
was a direct result of the atrophy. This subject will be more fully discussed
in the section of neurology, but most authoritative opinion ascribes the motor
anomalies to a central lesion in the mid-brain.
Barkan. K. M. Aug., lxvi, 528, 1918. Gallenga. A. di Ott., xii, 467, 1905.
Bedell. A. of O., xvii, 444, 1937. Hambresin. Boll. S. fr. d’O., xlii, 310, 1929.
Behr. K. M. Aug., lxvi, 363, 1921. Harms. K. M. Aug., lxi (1), 522, 1903.
Bietti. Boll. d’Oc., xii, 172, 1933. Helbron. Z. f. Aug., iv, 200, 1900.
Birnbacher and Czermak. A. f. O., xxxii (2), Igersheimer. Syphilis u. Auge, Berlin, 1918.
1, 1886. Kaminskaja-Pavlova. Russ. A. O., iii, 476,
Dupuy-Dutemps. An. d’Oc., czzxiv, 190, 1927.
1905. Koby. Microscopy of the Living Eye, London,
Franck. A. f. Aug., xlvii, 198, 1903. p. 213, 1930.
Fuchs. A. f. O., xxix (4), 209, 1883; ciii, Kries. A. f. O , xxii (3), 163, 1876.
297, 1920. Lawford. T. O. S., vi, 163, 1886.
1 p. 3343.
2404
TEXT-BOOK OF OPHTHALMOLOGY
Licsko. K. M. Aug., lxxi, 456, 1923.
Lodato. Riv. Oto-Neuro-Oſt., i. 557, 1924.
Atti S. It, Oto-Neuro-oſt., 276, 1926.
Lohmann. K. M. Aug., xlviii (1), 75, 1910.
Mayou. Brit, Med. J., ii, 1324, 1910.
Brit. J. O., ix, 7, 1925.
McGarth. J. Mental Sc., lxxviii. 362, 1932.
Nettleship. T. O. S., vi, 165, 1886.
Oblath. Beit. 2. Aug., xli., 10, 1899.
Santori. Boll. d’Oc., viii, 31, 1929.
Siegrist. B. O. G. Heidel.., xxxviii, 303, 1912.
Stern. A. f. Aug., lxxvi, 80; lxxvii, 77, 1914.
Vancea. Z. f. Aug., lxiii, 254, 1931.
de la Vega, K. M. Aug., lxxi, 44, 1923.
Velhagen. K. M. Aug., lxxviii. Beil., 156,
1927.
Vossius. B. O. G. Heidel.., xxxvi, 225, 1910.
Wagenmann. A. f. O., xxxviii (2), 91, 1892.
Paton. Proc. R. S. Med., xxiii, 289, 1930.
(B) Primary or Essential Atrophies
1. Essex.TIAL (PROGREssive) ATRoPHY of THE IRIs
This is a unilateral disease of unknown actiology characterized by a slowly
progressive atrophic change in the tissues of the iris which leads to the complete
Fig. 1996.-Essential ATRoPHY or THE IRIs.
In the stage of absolute glaucoma (Aitchison, T. O. S.).
disappearance of large portions of the tissue, and ultimately ends in blindness
from absolute glaucoma.
The disease is a rare one which was first clearly defined by Harms (1903), who
included in the group of cases he described those of Lindsay Johnson (1886) and Hess
(1892). Subsequent cases include those of Hepburn (1907), Casey Wood (1910), de
Schweinitz (1915), Lane (1917), Zentmayer (1918), Feingold (1918), Larsson (1920),
Arnold (1923), Liesko (1923), Rochat and Mulder (1924), Kreiker (1926–28), Griscom

DISEASES OF THE UVEAL TRACT 2405
(1927), Waite (1928), Mohr (1928), Almeida (1931), Klauber (1932), Kadlicky (1932),
Jeaneon (1933), Bietti (1933), Barr (1934), von Grosz (1936), McKeown (1937) and
Aitchison (1938). The case of Fine and Barkan (1937), which was noted at the age of
5 and was bilateral, had many affinities with buphthalmos.
The disease usually occurs in early adult life, but has been reported at
54 years (Griscom, 1927). It starts insidiously with the development of an
eccentric position of the pupil, which gradually becomes distorted and
displaced far to the side, usually showing an ectropion of the pigment
epithelium, until it becomes connected to the ciliary body merely by shrivelled
remains of the iris (Fig. 1996). On the opposite side large holes appear in
Fig. 1997. Essº NTIAL ATRoPHY of THE IRIs.
Horizontal section through a large hole and the pupil (Rochat and Mulder,
Brit. J. O.).
the iris which enlarge and coalesce so that the entire tissue atrophies until
only a few strands of stroma remain with large apertures between. Indeed,
the pupillary ring usually retains more of its original form than any other
portion. Eventually, usually after some years, when the greater part of the
iris has vanished, the tension rises and the symptoms of increased intra-ocular
pressure become conspicuous. Up to this development there have been no
clinical symptoms; but the glaucoma which develops is usually very
recalcitrant, responding little to miotics and only rarely to operation, and
the common end-result is blindness. Some comfort, however, may be
derived from the fact that all the reported cases have been invariably
unilateral.
Pathological examinations have been confined to eyes removed for

2406 TEXT-BOOK OF OPHTHALMOLOGY
absolute glaucoma (Bentzen and Leber, 1895; Wood, 1910; Feingold, 1918;
Iicsko, 1923; Rochat and Mulder, 1924; Ellett, 1928) (Fig. 1997). All
the cases show an extensive soldering of the root of the iris to the cornea with
compact tissue forming extensive peripheral synechiae. In Rochat and
Mulder’s case the tissue in the angle was so abundant and richly cellular as
to resemble a neoplasm of the root of the iris, and over it ran a regular layer
of endothelium which had secreted a hyaloid membrane.
The cause of the disease is unknown. Bentzen and Leber (1895) con-
sidered that the atrophy was the result of a primary glaucoma ; but that
this cannot be so is seen in that several cases have been carefully observed
in the earlier stages when it was obvious that atrophy was progressing and
symptoms of glaucoma were yet completely absent (de Schweinitz, 1915;
Rochat and Mulder, 1924; Waite, 1928; McKeown, 1937); while in
Waite’s (1928) case in which sclerectomy controlled the glaucoma, atrophy
proceeded for four years although the tension never rose above 12 mm.
Schiótz. It would seem, therefore, that the atrophy is primary and the
glaucoma secondary.
The cause of the atrophy is obscure. Cases showing inflammatory symptoms are
exceptional and probably anomalous (Harms, 1903; Wood, 1910; Aitchison, 1938).
Some would ascribe it to a congenital anomaly—a congenital vascular disturbance of
the lesser vascular circle (Feingold, 1918), or a continuance of the same biological
process which is responsible for the atrophy of the pupillary membrane (Kreiker,
1923–28 ; Jeancon, 1933). It is difficult to understand, however, why the iris remains
normal for so many years and then atrophy should again become evident late in life.
Lane (1917) and Zentmayer (1918) considered vascular sclerosis the causal factor, and
others have ascribed it to some constitutional toxic disturbance (de Schweinitz, 1915);
de la Vega (1923), for example, found the formation of a hole in the iris in syphilis
without inflammatory signs. In most of the cases, however, detailed systemic examina-
tion has proved negative. Pathological studies indicate that the principal factor in
producing the initial deformity is traction caused by the shrinking of the new-formed
tissue at the angle of the anterior chamber, and that the pulling over of the iris leads to
atrophy and tearing of the opposite segment ; while the ultimate complete obliteration
of the angle, perhaps with the added factor of the great reduction in the absorbing
surface of the iris, determines the final glaucoma which destroys vision. There is no
clue, however, to the cause of the circumscribed obliteration of the angle. The most
obvious hypothesis is some toxic factor ; but on the other hand, von Grosz
(1936) considers it a primary heredo-degenerative process. Any decision, however,
must rest until an opportunity is provided for the pathological examination of an
early case.
Until our knowledge has been increased it would seem probable that treatment
will remain ineffective and the prognosis bad. An operation should be done in an
attempt to relieve the tension : a sclerectomy controlled the tension in Waite's (1928)
case for 4 years, and 2 years after two cyclodialyses von Grosz's (1936) case had
normal tension and good vision.
Aitchison. T. O. S., Iviii (1), 430, 1938. Bentzen and Leber. A. f. O., xli (3), 229,
Almeida. Am. Oc. Rio, ii, 303, 1931. 1895.
Arnold. K. M. Aug., lxxi, 723, 1923. Bietti. Boll. d’Oc., xii, 172, 1933.
Barr. A. of O., xii, 567, 1934. Ellett. T. Am. O. S., xxvi, 306, 1928.
DISEASES OF THE UVEAL TRACT 2407
Feingold. Am. J. O., i, 1, 1918. Lane. O. Rec., xxvi, 285, 1917.
Fine and Barkan. Am. J. O., xx, 277, 1937. Larsson. K. M. Aug., lxiv, 510, 1920.
Gifford. Am. J. O., ix, 548, 1926. Licsko. K. M. Aug., lxxi, 456, 1923.
Griscom. Am. J. O., x, 647, 1927. McKeown. A. of O., xviii, 347, 1937.
v. Grosz. A. f. Aug., cz, 111, 1936. Mohr. Ned. tij. v. Gen., ii, 4322, 1928.
Harms. K. M. Aug., xli (2), 522, 1903. Rochat and Mulder. Brit. J. O., viii, 362,
Hepburn. T. O. S., xxvii, 84, 1907. 1924.
Hess. K. M. Aug., xxx, 103, 1892. de Schweinitz. T. Am. O. S., xiv, 250,
Jeancon. Am. J. O., xvi, 813, 1933. 1915.
Johnson. O. Rev., v, 57, 1886. de la Vega. K. M. Aug., lxxi, 44, 1923.
Radlicky. Oft. Sbornik, vii, 68, 1932. Waite. Am. J. O., xi, 187, 1928.
Klauber. Brat. Lek. Listy., xii, 152, 1932. Wood. Ophthalmoscope, viii, 858, 1910.
Kreiker. K. M. Aug., lxxiii, 141, 1924; Zentmayer. Am. J. O., i, 510, 1918.
lxxvi, 575, 1926; lxxx, 492, 1928.
2. EssFNTIAL (GYRATE) ATROPHY OF THE CHOROID.
GYRATE ATROPHY is a disease of unknown origin characterized by a pro-
gressive primary atrophy of the choroid, the pigment epithelium and the retina,
frequently of familial incidence, starting usually with an irregular patchy
distribution in the periphery and tending ultimately to involve the greater part
of the fundus.
The term “atrophia gyrata choroideae et retinae '’ was first employed by Fuchs
(1896) in describing a case with his usual accuracy and detail, correlating it with 3
cases described from his clinic by his pupil Cutler (1895). Similar cases had been
described previously by Laurence and Moon (1866), Jacobsohn (1888), and Hutchinson
(1900) and subsequent cases are those of Wernicke (1908), Komoto (1914), Mori (1914),
Arganaraz (1917), Böhn (1919), Zorn (1920), Werkle (1931), Volovic (1931), McGuire
(1932), Lyle (1932) and Kapuscinski (1934). The earlier of these were summarized
by Nettleship (1907) and the later by Usher (1935). Analogous cases are found in the
literature, as those of Bednarski (1900) which was of inflammatory origin, and Ohashi
(1932) which was anomalous in that it was stationary, non-familial, was unaccompanied
by other anomalies, and gave rise to a peculiar form of night-blindness. McGuire's
(1932) case (aged 11) was also anomalous in that over the period of observation
(2 years) it was non-progressive and there was no night-blindness ; but it could well be
interpreted as a very early stage in the disease. No pathological examination has been
recorded in the literature.
The disease usually comes under observation in the second and third
decades of life (from 6 to 29 years), the symptoms attracting attention being
a progressive diminution of vision and night blindness. The appearance of
the fundus is characteristic. Starting in the periphery atrophic areas appear,
usually of irregular and sometimes of fantastic shape, between which lie areas
of normal fundus, and over which run the larger choroidal and smaller retinal
vessels (Fig. 1992, Plate XLIV). Presumably in those areas the chorio-
capillaris and the pigment epithelium have entirely disappeared. The
atrophic areas vary in size, shape and distribution ; some are small and
round, while others are large, polygonal, or irregularly bizarre in form. Over
them there are usually deposited clumps of pigment, and the remainder of
the retina is also frequently mottled and diffusely stippled in appearance.
As the disease progresses the atrophic areas increase in size, fuse and spread
2408 TEXT-BOOK OF OPHTHALMOLOGY
inwards from the periphery, while a peri-papillary atrophy usually develops
coincidently (Fig. 1993, Plate XLIV); and in the final stage practically all
the fundus has disappeared with preservation only of the macula, a part
which persists longest doubtless because of the denser chorio-capillary
network. Eventually this also may become atrophic, or may show
pigmentary degeneration.
There are frequently other complications. In a quarter of the recorded
cases the retinal vessels were contracted and narrowed and the disc pale. In
one half of the cases in which a record has been made some cataract has been
present. In three-quarters of the cases myopia of varying degree has
occurred (up to −20 D), and it has usually been progressive. Finally, other
I * Q

FIG. 1998.-GYRATE ATROPHY.
Werkle's heredity of 3 generations.
deformities may be present—webbing of the fingers, infantilism, ataxias,
and so on.
The subjective symptoms include a gradually increasing visual failure
which may progress to mere perception of light. Night-blindness has been
present in every case as a prominent symptom from the commencement,
with one exception (McGuire, 1932). The fields of vision have been normal
in 20% of the recorded cases; usually there is progressive concentric
contraction ; a ring scotoma has occurred, but a central scotoma is unknown.
The progress of the condition is extremely slow ; no treatment is
known ; and the prognosis depends on the rate of development.
The disease is definitely hereditary, and consanguinity of the parents is
frequently mentioned. Six pedigrees have been given in the literature ; in
four the affected individuals were in the same sibship (Laurence and Moon,
1866; Cutler, 1895; Mori, 1914; Lyle, 1932), in one in 2 generations
(Jacobsohn, 1888), and in one in 3 (Werkle, 1931) (Fig. 1998). In two instances
DISEASES OF THE UVEAL TRACT 2409
it occurred with primary pigmentary degeneration of the retina in the same
pedigree (Böhn, 1919; Zorn, 1920), and in another two with choroideremia
(Zorn, 1920; Werkle, 1931).
The nature and aetiology of the disease is still in dispute, but it seems undoubtedly
to be a heredo-degenerative condition. Komoto (1914) considered the retinal neuro-
epithelium as the first tissue to be affected, but most authors agree with Fuchs (1896)
that the atrophy is primarily choroidal and that the constantly enlarging spots indicate
that the process begins at the centre of individual vascular areas. Fuchs considered
it related to primary pigmentary degeneration of the retina, a view upheld by
Nettleship (1907), who included in a loosely related group gyrate atrophy, retinitis
pigmentosa, retinitis pigmentosa sine pigmento, retinitis punctata albescens, congenital
night-blindness, and choroideremia. Its relationship to choroideremia seems closest,
the latter being regarded by some as the ultimate stage of the disease (Böhn, 1919;
Werkle, 1931). It will be remembered 1 that there are reasons for assuming that this
condition is not congenital (Bedell, 1937). The strongest argument in favour of this is
the occurrence of the two conditions in the same pedigree, while in one of Werkle's cases
One eye showed the picture of choroideremia while the other showed a moderately
advanced gyrate atrophy. It will also be remembered that no case of choroideremia
has been seen younger than 14 years; but at the same time no one has yet seen a
case of gyrate atrophy develop into this condition. In view of our unsatisfactory
knowledge of both conditions and in the complete absence of pathological studies, the
question must be left open ; indeed it is questionable whether either condition forms a
homogeneous group.
Arganaraz. A. de Oft. H.-A., xvii, 665, 1917. Lyle. Am. J. O., xv, 1165, 1932.
Bedell. A. of O., xvii, 444, 1937. McGuire. A. of O., viii, 372, 1932.
Bednarski. A. f. Aug., xl, 420, 1900. Mori. Nip. Gank. Zas., xviii, 262, 1914.
Böhn. K. M. Aug., lxiii, 381, 1919. Nettleship. R. L. O. H. Rep., xvii, 151, 333,
Cutler. A. f. Aug., xxx, 117, 1895. 1907.
Fuchs. A. f. Aug., xxxii, 113, 1896. Ohashi. Acta S. O. Japan, xxxvi, 53, 1932.
Hutchinson. A. of Surg., xi, 118, 1900. Usher. T. O. S., lv., 170, 1935.
Jacobsohn. K. M. Aug., xxvi, 202, 1888. Volovic. Russ. O. J., xiv., 142, 1931.
Kapuscinski. B. O. G. Heidel., l, 12, 28, 1934. Werkle. K. M. Aug., lxxxvii, 173, 1931.
Komoto. K. M. Aug., lii, 416, 1914. Wernicke. A. f. Aug., lxii, 239, 1908.
Laurence and Moon. O. Rev., ii, 32, 1866. Zorn. A. f. O., ci, 1, 1920.
3. PRIMARY CHOROIDAL SCLEROSIs
Choroidal sclerosis is a degenerative vascular change occurring as a FOCAL
lesion in the choroid associated with a secondary depigmentation and degenera-
tion of the retina. It occurs in elderly people although it has been noted
exceptionally in the young (8 years, Bednarski, 1900; 11 years, Knapp,
1907). The lesion is not necessarily associated with general arterial disease
or even retinal disease, although cardiac disease (Altnow, 1927) or coronary
sclerosis may occasionally co-exist (Yater and Wagener, 1929; Cohen, 1938).
Its importance depends upon the effects upon the tissues lying on its inner
surface which rely upon it for nourishment ; these include Bruch's membrane,
the pigment epithelium, and the retina.
The typical reaction in Bruch's membrane is the formation on its inner
hyaline layer of excrescences (colloid bodies or drusen).” In the pigment
1 Vol II, p. 1330. * p. 2747.
2410 TEXT-BOOK OF OPHTHALMOLOGY
layer depigmentation and atrophy occur, resulting in a lightening of the
colour of the fundus so that the structure of the choroid is readily seen.
º
- - ---
Fig. 1999.-PRIMARy Choroidal ScLERosis.
The choroid is atrophic showing fibrosis of the arterial walls without any marked
thickening or encroachment on the lumen of the vessels (x 120) (Martin Cohen,
A. of 0.).
Fig. 2000–PRiMARy Choroidal Sclerosis.
A choroidal artery from Fig. 1999 showing fibrotic wall without narrowing of
lumen (x 240) (Martin Cohen, A. of 0.).
Alternatively, the pigment cells may proliferate resulting in a darkening of
the colour, the changes occurring irregularly so that the pigmentation varies






DISEASES OF THE UWEAL TRACT 241]
in intensity in different areas. Moreover, there is usually an actual migration
of the pigment into the retina itself. Frequently the granules tend to become
deposited in the perivascular spaces of the veins so that these are mapped
out over some of their length in black, but a more usual appearance is the
formation of small irregular star-shaped masses of jet-black pigment taking
a form resembling bone-corpuscles lying in the inner layers of the retina,
superficial to and obscuring the blood-vessels. In the retina, degenerative
changes of a more or less widespread nature may occur and its function is
always impaired.
In the choroid itself the sclerosis is limited essentially to the arteries;
the veins are relatively unaffected, but the sclerotic process produces
Fig. 2001.-CHoRoidAL SCLERosis.
From the same case as Figs. 1999, 2000; showing a relatively normal branch of the
central retinal artery (X 240) (Martin Cohen, A. of O.).
degenerative changes in the intervascular stroma so that the vessels are
exposed. In a typical case of focal choroidal sclerosis occurring in Paget’s
disease, unassociated with retinal but accompanied by coronary disease,
Cohen (1938) found that the walls of the arteries were not thickened but the
muscular coat was completely replaced by fibrous a-cellular tissue; the
elastica stained poorly, but the endothelium was preserved and the lumen
was normal (Figs. 1999, 2000). These changes were sharply limited to a
focal area of the choroid and were entirely degenerative in nature, affecting
essentially the muscular coat. The pathological picture, without thickening
of the wall or encroachment on the lumen, is thus quite different from that
presented by the hyperplastic changes resulting from hypertension or
associated with nephritis (compare Fig. 1960).
-

24 12 TEXT-BOOK OF OPHTHALMOLOGY
Choroidal sclerosis, while always essentially due to the same patho-
logical process, gives rise to varied clinical pictures depending on its regional
distribution. It may affect the entire choroid as diffuse choroidal sclerosis,
showing usually, however, an area in the posterior region where the process
is more advanced regionally. Alternatively, it may be sharply limited to a
circumscribed area of the fundus; of these the two most typical are the
central area (central senile areolar choroidal atrophy) and the region around
the optic disc (peri-papillary choroidal sclerosis).
DIFFUSE CHOROIDAL SCLEROSIS
This condition, described by Morton (1885) under the name of atrophy
of the choroid, is characterized by a general obliteration of the choroidal
vessels. It usually occurs in old people about the sixth or seventh decade,
but may appear in young adults. It is not hereditary but may appear
familially (Sorsby, 1935–39), and is usually not associated with widespread
or serious vascular disease either systemically or in the retina or the cerebral
circulation (Geis, 1911). Syphilis or other general disease does not figure in
the aetiology.
Ophthalmoscopically the fundus appears tigroid and brownish and the
most marked changes are in the posterior part of the globe (Plate XLV,
Fig. 2002). Atrophy of the pigment epithelium allows the choroid to be
seen ; and disappearance of the chorio-capillaris reveals the sclerosed
larger vessels outlined on the sclera. Some of them have greyish-white,
grossly thickened walls and a small central blood-stream, while others are
obliterated altogether, appearing as white solid bands. The process starts
near the disc and may be limited to this region ; more usually it becomes
widespread, but generally islands of greater sclerosis are evident wherein
atrophy of the choroid has exposed the white sclera extensively. A favourite
place for this accentuation is on the temporal side of the disc ; on the other
hand, and much more rarely, the circulus arteriosus nervi optici may escape
and the circumpapillary zone remain free. The retinal vessels course normally
over the fundus and may quite well be free of disease, but this tissue frequently
shows corpuscular accumulations of pigment ; while the optic disc may have
a waxy atrophic appearance.
The symptoms are a progressive deterioration of vision with a concentric
contraction of the visual field and a diminution of the light sense with night-
blindness; these tend to be slowly progressive until eventually function may
be limited to an appreciation of hand-movements only, or a bare perception
of light.
CENTRAL AREOLAR CHOROIDAL ATROPHY
Central areolar choroidal atrophy, described originally by Nettleship (1884),
and noted by Retze (1901) Thompson (1901–05), and others, is a bilateral condition
occurring in old people characterized by the development of a sharply circumscribed
oval area of extreme choroidal sclerosis extending from the disc to well beyond the
PLATE XLV.
CHoRoidAL ATRoPHIEs
Fig. 2002.-DIFFUSE CHORoid AL SCLE Rosis.
Fig. 2003.−CENTRAL AREola R SCLERosis Fig. 2004.—PERI-PAPILLARY SCLE Rosis.
Fig. 2005.-ANGLord STREAks.
To face p. 24.12.



DISEASES OF THE UVEAL TRACT 2413
macula (Fig. 2003, Plate XLV). Its familial occurrence has been noted (Sorsby,
1935–39). In the affected area the retinal pigment has entirely gone, as also have the
smaller choroidal vessels : only the larger ones remain and these are always narrowed
and frequently obliterated. The retinal vessels remain intact ; but there is a central
absolute Scotoma.
MASSIVE PERI-PAPILLARY CHOROIDAL SCILEROSIS
In this condition the sclerotic process occurs in the area round the disc, the
vessels involved being those derived from the circulus vasculosus nervi optici (Fig.
2004). Usually, however, the sclerotic process radiates to a variable and sometimes
considerable extent peripherally in all directions (Harman, 1902; Cuperus, 1903;
Guglianetti, 1908–09 ; Pillat, 1930; Di Marzio, 1938). In a small degree it is not
unlike the common age-change known as the halo senilis.” Like the other types of
choroidal sclerosis it may show a familial incidence (Sorsby, 1936–39).
Altnow. A. Int. Med., xl, 757, 1927. Rnapp. K. M. Aug., xlv. (1), 171, 1907.
Bednarski. A. f. Awg., x1, 420, 1900. Morton. T. O. S., v., 142, 1885; xiii, 282,
Cohen. A. of O., xix, 487, 1938. 1893.
Cuperus. A. f. Aug., xlviii, 235, 1903. Nettleship. T. O. S., iv, 165, 1884.
DiMarzio. Fundus Oculi, Rome, Figs. 194–6, Pillat. Am. J. O., xiii, 1, 1930.
1938. Retze. Beit. z. Aug., v, 94, 1901.
Evans. Brit. J. O., xvii, 257, 1933. Sorsby. P. R. S. Med., xxviii, 526, 1935;
Geis. K. M. Aug., lxix (1), 1, 1911. xxx, 386, 1936.
Guglianetti. A. di Ott., xv, 469, 1908; xvi, Brit. J. O., xxiii, 433, 1939.
1, 1909. Thompson. T. O. S., xxi, 66, 1901; xxv,
Harman. T. O. S., xxii, 156, 1902. 118, 1905. -
Rerschbaumer. A. f. O., xxxviii (1), 127, Yater and Wagener. Am. J. Med. Sc.,
1892. clxxviii, 105, 1929.
ANGIOID STREAKs
ANGIOID STREAKs are a peculiar and characteristic appearance in the
fundus first described by Doyne (1889) and named as a clinical entity by
Plange (1891). The condition is characterized by a fairly uniform picture
consisting of a network of streaks varying in colour from reddish to dark
brown which emanate from a more or less circular ring round the disc
and radiate irregularly to the peripheral parts of the retina, sometimes
associated with a mosaic of spots (Fig. 2005, Plate XLV ; Fig. 2156,
Plate XLVIII; Figs. 2006–7). The streaks vary in width, being
sometimes broad and sometimes narrow, but are usually irregular and
ragged somewhat suggesting swept-up accumulations of pigmentary débris
or a line of wrack left on the sea-shore. In the later stages they are
sometimes bounded by a whitish border which has variously been interpreted
as resulting from fibrous tissue formation, or the refraction of light from the
edges of folds or fissures. The streaks lie underneath the retinal vessels and
above the choroidal vessels, but show no connection with or relationship to
the distribution of either. Many cases remain stationary indefinitely but in
a large proportion other lesions, sometimes most incapacitating in their
severity, are present in the fundus—haemorrhages particularly at the macula,
1 p. 2397.
2414 TEXT-BOOK OF OPHTHALMOLOGY
colloid degeneration, exudative choroidal masses particularly in the central
region (disciform degeneration), areas of choroidal atrophy, and so on. In
the recorded cases, indeed, haemorrhages occur in some 35% and macular
lesions in over 50%. While, therefore, the condition itself causes no
symptoms and is usually discovered accidentally, these associated lesions
are frequently of disastrous functional import. The streaks themselves,
however, do not alter much even when observed for long periods (22 years,
Fig. 2006.-ANGioid STREAks.
A typical case. Note the choroidal spot interrupting the streak on the nasal
side (Rayner Batten, Brit. J. O.).
Köhne, 1918), but the ultimate prognosis because of the coincident macular
degeneration is poor.
Thus Coppez and Danis (1925) first saw a case in 1900 which remained unaltered
for 8 years with normal vision in each eye; 9 years later there was a central scotoma
in one eye, and in 1923 there was complete degeneration of the central area in both.
Although not rare, angioidstreaks are not a common condition; only some
150 cases are reported in the literature. Both eyes are always involved
although the lesions are not symmetrical. It is more common in males than
in females, and patients usually come under observation in middle age,
between 30 and 50, 50% being over 40 years of age. It is to be noted
that the streaks are frequently associated with the development of
subsidiary degenerative or haemorrhagic lesions, and the streaks have been

DISEASES OF THE UVEAL TRACT 24.15
Fig. 2007.-ANgroup STREAks.
Showing streaks of enormous dimensions. Note the large choroidal spots
interrupting them (Rayner Batten, Brit. J. O.).
-
noted several times in youth or adolescence (Holloway, 1927; 9 years,
Bachsinian-Frenkel, 1927). An interesting feature is that several published
reports indicate a sporadic familial tendency, the lesion being transmitted
- - * -- - * - - --
-- - -- --- - ---
************* ****
- - - -
Fig. 2008.-ANgroup STREAks.
Showing break in Bruch's membrane with pigmentary disturbance (Hagedoorn,
A. of O.).
T.O.-W.O.L. 111.
Y


2416 TEXT-BOOK OF OPHTHALMOLOGY
as a recessive hereditary character (Spicer, 1914; Lindner, 1914; Lederer,
1923; Wildi, 1926; Clay, 1932; Hartung, 1932; Sugg and Stetson, 1934;
Blobner, 1935; Böck, 1935 ; Matras, 1935; Franceschetti and Roulet,
1936; Goedbloed, 1938). Trauma enters into the history in a large
number of cases (Marchesani and Wirz, 1931).
Pathological examinations have with few exceptions been inconclusive
since the histological findings had not been correlated with the ophthal-
moscopic picture. Lister (1903) described an appearance of new-formed
vessels in the outer layers of the retina, Magitot (1911) recorded regressive
changes in old haemorrhages, and Verhoeff (1928) a case of extensive fibrosis
of the choroid which, by contraction, produced folds in the pigmentary
epithelium. It is not clear, however, if any of these cases represented the
condition in question. In the two eyes seen ophthalmoscopically and
examined histologically different pictures were presented : Hagedoorn
(1937–39) found thickenings of Bruch's membrane with numerous small
ruptures and large sections where it was completely absent, together with
a thickening of the arterial walls in the choroid with a disappearance of
the elastic tissue (Fig. 2008); while Law (1938) found this structure and
the pigment epithelium normal, but the retina itself was thrown into
sharp folds in which the internal limiting membrane took no part and under
which was accumulated a quantity of pigmentary débris and degenerated
cells.
Many theories as to the aetiology of angioid streaks have been put
forward.
1. Haemorrhagic Theories. That the streaks are haemorrhagic in origin was
originally suggested by Doyne (1889) and Plange (1891), a view which received wide
support among the older writers (Knapp, 1892; Holden, 1895; de Schweinitz, 1896;
Pagenstecher, 1908; Spicer, 1914; Calhoun, 1927–28 ; and others), and was
elaborated most fully by Collins (1923). Collins believed that the haemorrhage was
intra- or sub-choroidal and that haematogenous pigment was deposited in the peri-
vascular spaces, particularly around the posterior ciliary vessels. The early redder
stage was taken to represent a deposition of haemoglobin, and the later darker stage of
haemosiderin and haematoidin.
2. Vascular Theories. The formation of supernumerary new vessels was sug-
gested by Lister (1903), Schrader (1906), Pagenstecher (1908), Zentmayer (1909),
Wassenaar (1933), Jacoby (1934) and Denti (1937) as a cause, and thrombosis of
anomalous posterior veins by Clay (1932): neither of these views can be substantiated.
Such a conception is disproved by a case described by Spicer (1914) in which a streak
was interrupted sharply by a patch of chorio-retinal atrophy through which the sclera
was visible (see Figs. 2006 and 2007): if the streak represented a vessel it could not
run undisturbed on either side of a complete break in its continuity. Batten (1931)
postulated a familial vascular disease of the degenerative type affecting the choroid
which gives rise to haemorrhages and exudates to be followed by choroidal atrophy or
scattering of pigment.
3. A folding of the inner tunics of the eye, particularly of the pigment epithelium,
was suggested by Walser (1895), Alt (1909), Guist (1921), and Coppez and Danis
(1924–25), the folding being probably produced by some exudative process in the
DISEASES OF THE UWEAL TRACT 24.17
choroid ; we have already seen that Verhoeff (1928) postulated a similar folding by
fibrous contraction. A plication of the retina itself with the accumulation under it
of pigmentary débris is more fully authenticated by the histological findings of Law
(1938).
4. Degeneration of the elastic elements of Bruch's membrane leading to the
formation of fissures was suggested as the aetiological factor by Kofler (1917)
and Lohmann (1922). Hughes (1929) considered that this was due to a
congenital deficiency in the pigment epithelium, but the theory attracted
little attention until the association of angioid streaks with pseudo-acanthoma
elasticum was pointed out by Grönblad (1929) (the GRöNBLAD-STRANDBERG
syNDROME). This is a rare disease of obscure aetiology affecting the elastic
tissue throughout the body, and becoming clinically evident in the skin by
the appearance of buff or yellow patches particularly on the neck and in the
flexures and sometimes on the abdomen and chest. The essential histological
change is an increase in amount and a degeneration of the dermal elastic
fibres. Since this original observation the great majority of published cases
in the literature have shown the presence of the two conditions—when they
have been specifically looked for (57 out of 67 cases, Goedbloed, 1938)
(Marchesani and Wirz, 1931; Poos, 1931 ; Grönblad, 1932–33; Hartung,
1932; Bonnet, 1933–35; Dykman, 1934; Benedict and Montgomery, 1935;
Denti, 1937; and others). It is noteworthy that angioid streaks are also,
although more rarely, associated with the somewhat similar dermal condi-
dition of senile elastosis (Jacoby, 1934; Franceschetti and Roulet, 1936), and
also with Paget’s disease (Batten, 1931; Verhoeff, 1931; Terry, 1934;
Franceschetti and Roulet, 1936; Goedbloed, 1938; Lambert, 1939).
As we have just seen changes in the elastic tissue of Bruch's membrane were
found histologically by Hagedoorn (1937). This association, particularly
in the case of pseudo-xanthoma elasticum, can scarcely be incidental, and it
is possible that similar lesions in the walls of the blood-vessels may account
for the tendency to haemorrhages in these cases not only in the eye but
elsewhere in the body (see Fig. 1774, Plate XXXII).
Until more pathological investigations have been made it is difficult in
the present state of our knowledge to give a dogmatic opinion on the matter.
The most likely provisional explanation would seem to be that angioid
streaks are associated with a degeneration of Bruch's membrane in which
ruptures occur, and that these are associated with arterial disease and
intra-ocular haemorrhages which, if they are recurrent, are prone to give rise
to other and more damaging lesions, particularly at the macula. It is
probable, too, that in many cases these ocular lesions are associated with
systemic disease which may cause haemorrhages elsewhere, one of which is
undoubtedly pseudo-xanthoma elasticum.
The treatment of the condition is unsatisfactory and, since the striae
themselves are symptomless, devolves into the amelioration or prevention
of the usual haemorrhagic and degenerative complications. Calcium and
Y 2
24l 8
TEXT-BOOK OF OPHTHALMOLOGY
vitamins B1 and C have been recommended by Franceschetti and Roulet
(1936) and Frydman (1938) in the early stages, but their effect in the later
stages is nil, nor do they prevent recurrent haemorrhages.
Gold as
intravenous injections has also been tried, but with by no means uniformly
successful results.
Alt. Am. J. O., xxvi, 171, 1909.
Bachsinjan-Frenkel. Russ. A. O., iii, 498,
1927.
|Batten. Brit. J. O., xv, 279, 1931.
Benedict. J. Am. Med. As., ciz, 473, 1937.
Benedict and Montgomery. Am. J. O.,
xviii, 205, 1935.
Blobner. K. M. Aug., xcv, 12, 1935.
Böck. Z. f. Aug., lxxxvi, 238, 1935.
Bonnet. A. d’O., i, 721, 1933; lii, 225, 1935.
Calhoun. T. Am. O. S., xxv, 209, 1927.
Am. J. O., xi, 91, 1928.
Clay. A. of O., viii, 334, 1932.
Collins. T. O. S., lxiii, 273, 1923.
Coppez and Danis. A. d’O., xli., 115, 1924;
xlii, 649, 1925
Denti. Am. di Ott., lxv, 93, 1937.
Doyne. T. O. S., ix, 128, 1889.
Dykman. A. of O., xi, 283, 1934.
Franceschetti and Roulet. A. d’O., liii, 401,
1936.
Frydman. An. d’Oc., clxxv, 154, 1938.
Goedbloed. A. of O., xix, 1, 1938.
Grönblad. Acta O., vii,329, 1929; x, Suppl.
1, 1932; xi, 461, 1933.
Guist. Z. f. Aug., xlv, 61, 1921.
Hagedoorn. Ned. tij. v. Gen., 3003, 1937.
A. of O., xxi, 746, 935, 1939.
Hartung. K. M. Aug., lxxxviii, 43, 1932.
Holden. A. of O., xxiv, 147, 1895.
Holloway. T. Am. O. S., xxv, 173, 1927.
Jacoby. A. of O., xi, 828, 1934.
Knapp. A. of O., xxi, 289, 1892.
Kofler. A. f. Aug., lxxxii, 134, 1917.
|Köhne. A. f. O., xev, 97, 1918.
Lambert. A. of O., xxii, 106, 1939.
Law. T. O. S., lviii, 191, 1938.
Lederer. K. M. Aug., lxxi, 767, 1923.
Lindner. A. f. O., lxxxviii, 230, 1914.
Lister. O. Rev., xxii, 151, 1903.
Lohmann. A. f. Aug., xc, 203, 1922.
Magitot. An. d’Oc., czlv, 12, 1911.
Marchesani and Wirz. A. f. Aug., civ, 522,
1931.
Matras.
Pagenstecher.
175, 1908.
Plange. A. f. Aug., xxiii, 78, 1891.
Poos. K. M. Aug., lxxxvii, 734, 1931.
Schrader. A. f. Aug., xvi, Erg., 80, 1906.
de Schweinitz. T. Am. O. S., vii, 650, 1896.
Spicer. P. R. S. Med., Sect. O., viii, 33, 1914.
Sugg and Stetson. J. Am. Med. As., cii, 1369,
1934.
Terry. T. Am. O. S., xxxii, 555, 1934.
Verhoeff. T. A m. Med. As., Sect. O., 243,
I928.
J. A. m. Med. As., xovii, 1873, 1931.
Walser. A. f. Aug., xxxi, 345, 1895.
Wassenaar. Am. J. O., xvi, 759, 1933.
Wildi. K. M. Aug., lxxvi, 177, 1926.
Zentnayer. T. Am. O. S., xii, 267, 1909.
|Wien. kl. W., xlviii, 198, 1935.
A. f. O., lxvii, 298; lxviii,
Hughes. A. of O., i, 551, 1929.
3. Pigmentary Disturbances
(a) MELANIN
Most of the conditions, the majority of which are atrophic in nature,
which are associated with pigmentary disturbances have been already noted.
The most common is SENILITY, for in senile atrophy, which must be con-
sidered physiological, disintegration of pigment is a constant phenomenon
occurring to some extent in about 90% of persons over 50 years and
universally between 60 and 70 years of age (Hinnen, 1921; Vogt, 1921). We
have already seen that the depigmentation affects both the stroma and the
posterior ectodermal layers, in the former by a destruction of the chromato-
phores in the process of hyalinization and sclerosis, and in the latter, where
the phenomenon is much more marked, by a disintegration of the pupillary
border and by the appearance of small lacunae or gross defects. Frequently
also, as we have noted, localized accumulations of pigment may be observed
side by side with the depigmentation giving an irregular, jagged appearance
DISEASES OF THE UVEAL TRACT 2419
to the pupillary border; while the pigment granules swarm over the stroma,
sometimes in considerable quantity, where they lie superficially on the
trabeculae and in the crypts of the iris, and are deposited on the capsule of
the lens, on the posterior surface of the cornea where a cuneiform distribution
may appear (Krukenberg's spindle *), and most numerously among the
trabeculae and the meshes of the pectinate ligament in the angle of the
anterior chamber. These changes are of pathological interest in that they
FIG. 2009.-PIGMENT IN ANGLE of THE ANTERIOR CHAMBER.
After a traumatic irido-cyclitis. The angle is filled with cells from the pigmentary
epithelium forming a dense mass with some leucocytes and erythrocytes. There is a
similar pigmented mass behind the iris. The iris is necrotic (x 55) (Parsons).
have been associated with the aetiology of cataract (Axenfeld, 1911) and
glaucoma (Levinsohn, 1922).”
An unusual degree of this type of pigmentary disturbance is seen in
pathological conditions, more especially TRAUMA, GLAUCoMA, DLABETES,
RETINAL DETACHMENT, POST-INFLAMMATORY ATROPHIES, and MALIGNANT
MELANoMATA (Fig. 2009). Each of them is described in their several
sections. Not only may the depigmentation be general, but it may occur
in local areas, especially in localized inflammations; in this connection the
white atrophic spots of VITILIGo * after variola and scarlatina should be
noted. Two special forms of depigmentation are of importance; the great
disintegration of the posterior pigment layer which occurs in complicated
HETEROCHROMA, where, in addition to disappearance of the pigment border,
large confluent lacunae appear in the posterior layer; and the LEUKIRIDIA
* Vol. II, p. 2036. * p. 3359. * p. 2150.

2420 TEXT-BOOK OF OPHTHALMOLOGY
which occurs in about 10% of cases of secondary syphilis, especially in
women with brown irides (Krückmann, 1907; Soewarno, 1919). This has
many affinities with leucoplakia ; depigmentation begins near the ciliary
border in the neighbourhood of the crypts, where large depigmented areas
appear which may become confluent, and later may become pigmented
again.
(b) HAEMATOGENOUs PIGMENT
Haematogenous pigment may bring about a change in colour of the iris
after bleeding, whether traumatic or inflammatory, or a similar appearance
may perhaps be the result of prolonged inflammatory hyperaemia. Such a
post-traumatic heterochromia occurs a considerable time after the injury—
usually some 10 (Koby, 1930) to 20 years (Vogt, 1921). As a rule the
injured eye exhibits a type of pigmentation similar to the other, only darker,
the superficial layers of the stroma being especially affected and showing a
diffuse brownish colouration. At other times the iris takes on a greenish-
yellow hue, frequently with the addition of localized darker areas. It is
interesting that in those cases the lens has usually been destroyed or
needled ; indeed, Vogt has seen the condition 16 years after a needling
operation undertaken for myopia.
For some considerable time after an injury masses of haematogenous
pigment, usually engulfed by leucocytes, may be caught in the meshes of
the pectinate ligament.
(c) SIDEROSIS
When an iron foreign body is retained in the eye, after a latent period lasting
from Some weeks to some years, pigmentary and degenerative changes, which are
grouped together under the term SIDEROSIs, take place. This condition will be
dealt with in detail when considering injuries of the eye, and it will be sufficient here
to summarize the most marked pigmentary changes which occur in the uveal tract
(Mayou, 1925–26 ; Wolff, 1934). The iris assumes a brown colour which is seen
pathologically to be due to impregnation of its superficial layers with iron. It is
noteworthy that the sphincter and dilatator muscles are heavily impregnated, whereas
the ciliary muscle is relatively unaffected : this may have some association with the
epithelial origin of the former. The pigmentary epithelium on the posterior surface
of the iris is usually little affected, although in severe cases it may show some degenera-
tion ; but the ciliary epithelium becomes heavily loaded with iron, particularly the
inner layer. Eventually the outer layer loses its pigment and becomes bleached and
highly degenerate, a circumstance which may account for the usual termination of
cases of untreated siderosis in glaucoma, by allowing the dialysation of a plasmoid
aqueous. The choroid, however, is little affected, presumably because it is protected
by the retina.
Axenfeld. B. O. G. Heidel., xxxvii, 1911. Mayou. T. O. S., xlv., 274, 1925; xlvi, 167,
Hinnen. Z. f. Aug., xlv., 129, 1921. 1926.
Koby. Microscopy of the Living Eye, London, Soewarno. K. M. Aug., lxiii, 275, 1919.
1930. Vogt. Atlas, Berlin, 1921.
Krückmann. G.-S. Hb. II, vi, 40, 1907. A. f. O., cvi, 90, 1921.
Levinsohn. K. M. Aug., lxviii, 471, 1922. K. M. Aug., lxxxii, 433, 1929.
Wolff. P. R. S. Med., xxvii, 691, 1934.
DISEASES OF THE UVEAL TRACT 24.21
4. Pathological Degenerations
(a) FATTY DEGENERATION
FATTY DEGENERATION occurs in the uveal tract both in senility and in
pathological states, in which latter case it may occur even in young children
(vitreous abscess, metastatic ophthalmitis, necrotic retino-blastoma, etc.).
It is not, however, a common condition and is rarely marked in degree, partly,
perhaps, because there is a strong tendency to carry away the fatty deposits
in the veins (Jaensch, 1935): they have thus disappeared before pathological
examination takes place. The fat may in some cases be brought by the
blood-stream and stored in the tissues, or alternatively, it may represent
the remnants of degenerated and necrotic tissue-cells. It may be found in
two situations, both types of degeneration occurring together in the same
eye ; the one affects the blood-vessels (arteries, arterioles and capillaries),
and the other the tissues, wherein the fatty globules may be extra- or intra-
cellular, in the stroma, in the chromatophores, the muscle cells, or the
epithelium. In a general sense such changes may be found in any part of
the uvea, but are seen most frequently in the ciliary body and less so in the
iris and choroid.
In the iris fatty changes may be present in the vessel walls. In the
atherosclerosis of senility this is very rare, but in pathological conditions it
sometimes does occur, as in phthisis bulbi following panophthalmitis, irido-
cyclitis or ulcus serpens, in chronic irido-cyclitis, particularly tuberculous
(Landman, 1925), in secondary glaucoma, or in association with a malignant
tumour ; in the last case this degeneration may be entirely localized to the
immediate vicinity of the tumour (Jaensch, 1935). More commonly is the
fat found free in the iris stroma or in fat-laden histiocytes, the latter being
especially numerous near the perivascular spaces or in the chromatophores.
A degeneration of the sphincter muscle usually occurs in cases where the
stroma is little affected. At first a few globules are seen in the muscle
cells, the nuclei then become obscured by them, globules then appear between
the fibres, and finally the whole region of the sphincter stains uniformly with
sudan.
In the ciliary body fatty changes are more frequent. They are seen
commonly in senility, when the arteries may show considerable fatty deposits
in their walls (Attias, 1912; Hanssen, 1922–23), the fibres of the ciliary
muscle may contain fatty droplets (Herbert, 1929), and the ciliary epithelium,
first and particularly the non-pigmented layer, shows considerable fatty
degeneration (Attias, 1912; Busacca, 1927). In long-standing pathological
conditions this process is intensified, extra-cellular fat accumulating
preferentially under the epithelium (Jaensch, 1935), attacking the ciliary
muscle (Villani, 1934), and eventually impregnating almost all the connective
tissue in the ciliary processes. In the epithelium the cells may eventually
2422 TEXT-BOOK OF OPHTHALMOLOGY
contain more fat than pigment in such conditions as primary glaucoma
(Ischreyt and Reinhard, 1901), secondary glaucoma following injury
(Meesmann, 1924; Jaensch, 1930), retino-blastoma (Busacca, 1927 ), and in
inflammatory conditions of long-standing (Jaensch, 1935).
In the choroid fatty degeneration occasionally occurs in the walls
of the smaller arteries, and here again the perivascular spaces may be
packed with histiocytic cells undergoing foamy degeneration. This is
intensified in pathological conditions, but usually the fatty changes in the
tissues are more accentuated than in the vessels. Such changes Jaensch
(1935) found in 10% of the eyes he examined. The fatty globules may be
Fig. 2010–INTRA-ocular CholestERoi Tumour (Williamson-Noble, T. O. S.).
seen sporadically in a single histiocyte or a single chromatophore, or may be
so marked that the entire thickness of the choroid may be sudan-positive.
It has already been noted that accumulations of lipoid material may be formed
in the uveal tract in experimental lipoidosis brought about by a cholesterin-rich diet
(Jess, 1925; Rohrschneider, 1925–27; Löwenstein, 1936; and others).
CHOLESTEROL DEPOSITS
Occasionally, after prolonged inflammation or after a haemorrhage, cholesterol
crystals are seen lying on the surface of an atrophie iris or accumulating with other
débris in a fibrous coagulum in the anterior chamber (Fig. 1827); and—also rarely—
large masses of crystals may accumulate in tumour formation. These may occur in
the choroid or at the angle of the anterior chamber, where the mass of crystals may be
bound together and enclosed by proliferating endothelium (Williamson-Noble, 1922;
Heath, 1933) (Fig. 2010).
* Vol. II, p. 1436.

DISEASES OF THE UWEAL TRACT 2423
Attias. A. f. O., lxxxi, 405, 1912. Jess. B. O. G. Heidel.., xlv., 212, 1925.
Busacca. K. M. Aug., lxxviii, 529, 1927. Landman. A. of O., liv, 261, 1925.
Hanssen. K. M. Aug., lxviii, 391, 1922; Löwenstein. K. M. Aug., xcvi, 765, 1936.
lxx, 732, 1922; lxxxiii, 108, 1929. Meesmann. A. f. Aug., xciv, 56, 1924.
Heath. A. of O., x, 342, 1933. Rohrschneider. K. M. Aug., lxxiv, 93, 1925.
Berbert. Brit. J. O., xiii, 289, 337, 1929. A. f. O., czv, 535, 1925; exviii, 131,
Ischreyt and Reinhard. A. f. Aug., xliii, 133, 1927.
I901. Villani. An. di Ott., lxii, 483, 1934.
Jaensch. B. O. G. Heidel.., xlviii, 265, 1930. Williamson-Noble. T. O. S., xlii, 189, 1922.
A. f. O., czzxiii, 517, 1935.
(b) HYALINE DEGENERATION.—involving primarily changes in the blood-vessels
and later in the stroma wherein large areas particularly near the pupillary margin and
in the ciliary processes may undergo almost complete hyalinization—has already been
sufficiently noted in the sections on senile and pathological atrophies."
(c) Ossi FICATION
We have already noted * that calcification is a frequent end-change in
degenerating tissues which are accessible to the body-fluids, the change being
probably a physical one depending on a low carbon-dioxide tension due to
metabolic inactivity ; and that when blood-vessels are present, the process
tends to become one of Ossification, wherein the deposition becomes organized
in the definite architecture of Haversian systems controlled by osteoblastic
cells. Such a process is relatively common in the uveal tract of degenerated
eyes, particularly in the organized exudate from the ciliary body and
choroid resulting from a long-continued plastic inflammation. Bone-
formation is not confined to post-inflammatory degenerative conditions,
however ; it may arise, although rarely, in non-inflammatory exudates, as
in cases of angioma, microphthalmos with cysts (Collins, 1927), in hydro-
phthalmos (Dürr and Schlegtendal, 1889), or in extreme degrees of atrophy
of the choroid when the walls of the blood-vessels are almost without
structure (Bresgen, 1935).
The study of intra-ocular ossification has a very long history. It was investigated
more than 100 years ago by Schön (1838) and later by Alt (1847), while the occurrence
of true bone in the eye was proved by Hulke (1857). Thereafter the detailed studies
of Pagenstecher (1860), Knapp (1871) and Schiess-Gemuseus (1873) gave a sound
observational basis for our knowledge, which has been amplified particularly by Reid
(1888), Buchanan (1901), Snowball (1903) and others.
Eacperimental studies have been few and inconclusive. After injecting bile salts
into the vitreous (Wessely, 1915) discovered bone-formation in the atrophic chorcid.
v. Szily (1924), in his herpetic inoculations, found bone in both eyes. Imai (1930),
after injecting formic acid into the vitreous of rabbits, produced a phthisis bulbi which
in 46% of cases showed ossification ; and v. Herrenschwand (1934), after introducing
diseased skin (molluscum contagiosum, lupus pernio, Boeck's sarcoid, lichen, etc.)
into the anterior chamber of guinea-pigs, produced bone in the vascularized exudative
adhesions of the iris.
1 p. 2390. * Vol. II, p. 1442.
24.24
TEXT-BOOK OF OPHTHALMOLOGY
Since it constitutes the final phase of the degeneration of organized
plastic inflammatory deposits, ossification is usually found at a late stage
Fig. 2011.-Box E-
FORMATION IN
THE CHoRoid.
The bone almost
fills the globe
(Lawson).
in degenerating shrunken eyes. It may be met with at
any period of life (from 11 to 102 years), but several years
(some 10 or 12) are usually required to lapse after the
inflammatory activity before it makes its appearance.
There are, however, exceptions. Thus Schiess-Gemuseus
(1873) found bone in an eye which had been functioning
normally 10 months previously, Buchanan (1901) found
it in a boy 10 weeks after an injury, and in his experi-
ments v. Herrenschwand (1934) observed bone usually in
from 4 to 7 months, the shortest period being 1 month.
Usually the affected eye is blind, shrunken and painful,
and has been so for some considerable time; but rare
cases have occurred wherein bone has been found in an eye
which still retained some vision (Wölfflin, 1926). Clinically, bone in the
choroid can only exceptionally be seen ophthalmoscopically (Laqueur, 1877)
owing to the general degeneration of the media, but its presence may some-
Fig. 2012.-Bone-formation IN THE Choroup - EARLY STAGE.
Calcareous and bony deposits in the choroid close to the dise pushing the retina
forwards (x. 50) (Parsons).
times beinferred by the production of deep-seated pain on pressure (Dunn,
1925); the only satisfactory method of clinical diagnosis, however, is by
X-rays, relatively soft rays being used as the bone is spongy in texture
(del Duca, 1930; Shapira, 1932; and others).


DISEASES OF THE UVEAL TRACT 2425
- -
-- º
---
- ---
Fig. 2013.-Bon E-ForMATION IN THE CHOROID.
A bony cast of the choroid, large areas of true bone being formed in connective
tissue (Crawley, T. O. S.).
It has been claimed by Panas (1868) that bone can be primarily formed
in the iris, his claim being based on the microscopic demonstration of osteo-
blasts in a piece of iris removed by iridectomy in a man of 36; and we have
Fig. 2014.—Box.E-Fort MATIon IN CYCLITIS.
of 17 years' standing. True bone in the curve of the displaced ciliary body
(x 15) (Buchanan, T. O. S.).


2426 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2015.-CALCIUM DEPosits IN CycLITIC MEMBRANE (Crawley, T. O. S.).
seen that it can occur in iritic adhesions (v. Herrenschwand, 1934). The
essential sites, however, for the occurrence of ossification are (1) the inner
layers of the choroid, especially in its posterior parts where it is laid down in
the exudates from the chorio-capillaris which lie between this tissue and the
-
-
* -
-
Fig. 2016–TRUE Box.E-Formatios Is Cycliſtic MEMBRANE (Crawley, T. O. S.).


DISEASES OF THE UWEAL TRACT 24.27
retina, and (2) in exudative cyclitic membranes. Thence it has spread to
every tissue of the eye except the cornea (Fig. 2011). Although it may
start anywhere in the inner layers of the choroid and remain patchy in its
distribution, the process usually starts at the posterior pole round the disc
(Fig. 2012), pieces of bone being first irregularly distributed, and then
forming compact bars interconnecting with one another, the interspaces
being filled with granulation tissue and degenerated remnants of the
choroidal stroma. This may develop into a solid plaque at the posterior
pole, and then eventually the entire choroid may be converted into a solid
shell of bone, usually thicker at the posterior end where a hole remains to
admit the optic nerve (Fig. 2013) (Crawley, 1927; Kassner, 1933; and
many others). Occasionally bone dips over the edge of the optic disc,
covering it also (Moretti, 1928, in the cupped disc of a secondary glaucoma).
Usually the process stops anteriorly at the ora serrata, but it may proceed
further into the ciliary processes (Fig. 2014), the entire vitreous cavity
may be filled (Dejean, 1928), and if its capsule is ruptured 1 the lens may
be invaded. In regions where vascularization is not available, as in avascular
cyclitic membranes, the process does not progress beyond calcification
(Fig. 2015), but even here in the presence of blood-vessels, true bone is
laid down (Fig. 2016).
As a rule, as first pointed out by Knapp (1871), Ossification commences
in the region of the chorio-capillaris, and occurs in those cases where, as
a result of long-continued plastic inflammation, the capillary bed has
disappeared and has been replaced by exudates organized into fibrous tissue
(Schiess-Gemuseus, 1873). If the chorio-capillaris has not been destroyed,
the bone is found in fibrous tissue lying between this layer and the membrane
of Bruch where exudate has been poured out, and if this membrane has
been destroyed and the retina is fused to the choroid, the bone appears to
lie internal to the choroid, but is external to the pigmented epithelium if it
has survived. If the retina has become detached the pigment layer either
becomes degenerated and depigmented, or disappears entirely. The bone
fis thus developed in the fibrous tissue which replaces the capillary layer, or has
formed on its inner aspect as the result of the organization of ea:udates.
If, therefore, the membrane of Bruch survives, it is usually internal to the layer
of bone, but quite a number of cases have been reported wherein this relation has been
reversed (Schiess-Gemuseus, 1873; Brailey, 1882; Fontan, 1883). It is probable
that the presence of bone within the membrane is explained on the supposition that
it has been ruptured at an early stage of the inflammatory process, that the choroidal
exudation has spread between it and the retina, and that in this situation it is invaded
by the process of ossification (Lagrange, 1901). This seems a more reasonable explana-
tion than that depending on a hypothetical origin from the retinal pigmentary layer
(Brailey, 1882) Occasionally in the choroid small isolated areas of ossification may
be due to the calcification of masses of hyaline material, while bone may develop in
colloid bodies which are first permeated with fibrous tissue and then ossify.”
1 p. 2177. * p. 2747.
2428 TEXT-BOOK OF OPHTHALMOLOGY
The intimate nature of the process of ossification is not yet completely
known. Bone in the eye is never derived from cartilage; it is always
periosteal in type and heteroplastic in nature with the typical structure of
ordinary long bone. The fibrous tissue in which it is formed first assumes a
granular, denser appearance as a deposit of lime salts appears (the osteoid
stage). The cells assume a more angular shape, and then arrange
themselves in small trabeculae enclosing spaces lined by a layer of osteo-
blasts, which gradually lay down true bony lamellae. These lamellae with
their bone corpuscles are arranged concentrically round the blood-vessels to
Fig. 2017–Bose-Formation IN THE Choroid.
Irido-eyelitis of 23 years standing. True bone medulla with fat in choroid
(x 55) (Parsons).
form Haversian systems, and eventually, although rarely, a true medulla
may be formed composed of adipose tissue (Fig. 2017) (Parsons, 1905;
Margotta, 1931). A unique case has been reported by Stock (1918) wherein
the medullary tissue thus formed assumed neoplastic activity so that a
myeloma developed, which in turn destroyed most of the bone.
The origin of the osteoblasts is not yet clear. The old view that they are embryonic
cells transported to the choroid from some periosteal tissue, as of the orbit, cannot be
substantiated (Kassowitz, 1879: Whiting, 1890). Another theory suggested that
they represent embryonic cells remaining in the choroid from foetal life, which, being
stimulated into activity by long-standing inflammation and irritation, form fibrous
tissue and then bone (Grandclément, 1898). A third, a more probable view, is that
the connective tissue cells themselves become converted into bone corpuscles (Pagen-

DISEASES
2429
OF THE UVEAL TRACT
stecher, 1860 ; Buchanan, 1901).
But whether the connective tissue cells in question
arise from the fixed cells of the choroid or from the cells of the capillary walls is as yet
unknown.
Alt. Prager Vierteljahrschrift., iv, 2, 1847.
Brailey. R. L. O. H. Rep., x, 405, 1882.
Bresgen. Z. f. Aug., lxxxv, 217, 1935.
Buchanan. T. O. S., xxi, 232, 1901.
Collins. T. O. S., xlvii, 124, 1927.
Crawley. T. O. S., xlvii, 447, 1927.
Dejean. Bull. S. fr. d’O., xli., 218, 1928.
del Duca. Saggi di Oft., v, 547, 1930.
Dunn. Lancet, i, 229, 1925.
Dürr and Schlegtendal. A. f. O., xxxv (2),
88, 1889.
Fontan. Rec. d’O., iii, 205, 1883.
Grandclément. Lyon Méd., lxxxvii, 481,
1898.
v. Herrenschwand. Z. f. Aug., lxxxiv, 265,
1934.
Hulke. Med. Times dº Gaz., xiv., 127, 1857.
Knapp. A. of O., ii, 1, 1871.
Lagrange.
Laqueur.
Tumeurs de l’oeil, i, Paris, 1901.
A. f. Aug., vi, 29, 1877.
Margotta. Am. di Ott., lix, 416, 1931.
Moretti. Z. f. Aug., lxvi, 239, 1928.
Pagenstecher. A. f. O., vii (1), 99, 1860.
Panas. Gaz. des Hóp. de Paris, xli., 563, 1868.
Parsons. Path. of the Eye, ii, 480, 1905.
Pavia. Semama med., xxxiii, 606, 777,
1926.
Reid. Glasgow Med. J., xxix, 248, 1888.
Schiess-Gemuseus. A. f. O., xix (1), 202,
1873.
Schön. An... d’Oc., i, 381, 1838.
Shapira. Am. J. O., xv, 721, 1932.
Snowball. T. O. S., xxiii, 217, 1903.
Stock. K. M. Aug., lxi, 14, 1918.
Imai. A. f. O., czzv, 267, 1930. v. Szily, K. M. Aug., lxxii, 593, 1924.
Kassner. Z. f. Aug., lxxxii, 139, 1933. Wessely. A. f. Aug., lxxix., 1, 1915.
Kassowitz. Pratok Obs. Morsk. Vrach., xvi, Whiting. ...A. of O., xix, 361, 1890.
25, 1879. Wölfflin. A. f. O., exvii, 33, 1926.
(d) NECROSIs
NECROSIs, or acute death of the tissue-cells, differs from atrophy in that,
instead of the tissues being thin, condensed and cellular, they are swollen,
oedematous and ghost-like owing to absence of their nuclei. It may occur in
two main conditions—when the circulation is seriously interfered with
causing death of the tissues, and when the tissues are killed by an intense
toxaemia (Samuels, 1929).
1. Circulatory disturbances
(a) An irido-dialysis, by cutting off the branches of the greater arterial
circle, may cause a necrosis of the corresponding ciliary region of the iris.
(b) After experimental section (Wagenmann, 1890), or embolic occlusion
(Coats, 1907) of the posterior ciliary arteries, the corresponding area of the
choroid and the outer layers of the retina suffer necrosis (Fig. 1773).
(c) Glaucoma. While chronic glaucoma leads to atrophy, an acute
fulminating glaucoma may lead to necrosis of the pupillary margin of the
iris, that is, the part farthest away from the blood-supply which is impeded
by strangulation at the root.
(d) After localized thrombosis.
(e) With intra-ocular tumours, as a pressure phenomenon.
(f) After severe concussion injuries, in which case the entire iris may
become necrotic.
2430 TEXT-BOOK OF OPHTHALMOLOGY
2. Toaric disturbances
(a) In acute and massive bacterial infections, particularly perforating
wounds and metastatic inflammations, the necrosis usually becoming
evident two or three days after the advent of infection.
(b) In association with necrotic tumours.
In the iris the changes are definite and sometimes very striking
(D’Oswaldo, 1924; Edeskuty, 1925; Birnbacher, 1928; Samuels, 1929).
Fig. 2018–Necrosis or Iris with Pianºstaev Chaºs.
(a) Accumulation of pigmented cells on posterior surface of necrotic iris,
surrounded by a delicate membrane (b) forming a capsule: to the left disintegration
of the pigmented layer. (c) Deposition of pigmented cells on anterior surface of iris.
(Samuels, T. O. S.).
The stroma becomes oedematous, few nuclei are seen, the chromatophores
mostly disappear or assume a round contour, and although the sphincter
muscle tends to be preserved, it also may disappear in part or, rarely,
entirely. The pigment epithelium is usually greatly altered (Fig. 2018). In
severe cases the cells may be completely killed and cast off. More usually,
however, the process is less drastic and they tend to accumulate in large
masses on the posterior surface or at the pupillary margin, or alternatively,
they may migrate into the stroma or even accumulate on its anterior surface.
In the ciliary body the unpigmented epithelium may disappear without
leaving any trace, but the pigment layer is more resistant especially in the
furrows. In severe cases, however, the apices of the ciliary processes may

DISEASES OF THE UWEAL TRACT 2431
dissolve entirely away, but the ciliary muscle generally is preserved (Samuels,
1929).
In the choroid the vessels almost entirely disappear, and nuclei in the
stroma are sparse. The choroidal pigment becomes scattered in irregular
clumps throughout the stroma, and the retinal pigment tends to disappear
(Coats, 1907).
Birnbacher. Z. f. Aug., lxiv, 227, 1928. Edeskuty. Z. f. Aug., lv., 38, 1925.
Coats. T. O. S., xxvii, 135, 1907. Samuels. T. O. S., xlix, 421, 1929.
D’Oswaldo. Z. f. Aug., liv, 60, 1924. Wagenmann. A. f. O., xxxvi (4), 1, 1890.
WI. CYSTS OF THE UWEAL TRACT
Cystic formations in the uveal tract are limited to the anterior segment—
the iris and the ciliary body. Since the first authoritative paper on the
subject by Hulke (1869), who described 19 cases, various systems of classi-
fication have been proposed, based either upon morphological or topo-
graphical considerations (Collins, 1895; Terrien, 1901 ; Wintersteiner, 1906;
Villard and Dejean, 1933; and others). Our knowledge of the subject is not
yet sufficiently advanced to allow us to classify them on an aetiological basis,
for with the exception of the simplest forms—traumatic implantation cysts
and parasitic cysts—we have no certain and completely proven explanations
to offer for their occurrence. The following, however, is a provisional
aetiological classification :-
1. Congenital cysts. -
Developmental cysts (a) in the stroma.
(b) in the epithelium.
Traumatic implantation cysts.
Retention or exudative cysts (a) in the stroma,
(b) in the epithelium.
2.
:
;
Degenerative cysts.
Parasitic cysts.
*
In addition to these PSEUDO-CYSTs may occur, formed by the shutting off of
portions of the anterior or posterior chambers usually by inflammatory adhesions, as
for example, in a localized condition of iris bombé when the anterior wall of a cyst-
like formation may be comprised by the bellied-out iris and the posterior wall by the
lens capsule and adhesions between it and the ciliary processes.
Such a classification, however, is by no means satisfactory. As we have
seen a large proportion of congenital cysts are implantations, either of the
surface or neural ectoderm into the stroma ; the remainder, occurring
between the epithelial layers, represent a persistent dilatation of the primary
optic vesicle. Developmental cysts, which are usually termed spontaneous
cysts, probably have the same origin, the process becoming apparent at a
1 Vol. II, p. 1321.
T.O. — WOL. III. 7.
2432 TEXT-BOOK OF OPHTHALMOLOGY
later stage. Moreover, the aetiology of the endothelial cysts of the iris
stroma, which are usually believed to be retention-cysts, is very different
from the epithelial cysts of the posterior layers which result from exudative
or traction processes. It would be logical, therefore, to divide implanta-
tion cysts into two sub-divisions—developmental and traumatic. Again,
traumatic cysts may be implantation- or retention-cysts. From the
clinical point of view, however, (1) congenital, (2) parasitic and (3) traumatic
implantation cysts form distinct classes; (4) exudative and degenerative
cysts of the epithelial layers are usually secondary and incidental phenomena
resulting from inflammatory or pressure conditions, while the remainder can
be classified as (5) spontaneous or idiopathic cysts, which comprise (a) cysts
in the stroma, whether endothelial or epithelial in Origin, and (b) cysts in the
pigmentary epithelium.
For purposes of convenience we shall describe them in this latter order.
1. ConCENITAL Cysts. These have been already considered."
2. PARASITIC CYSTs. These will be dealt with in a later chapter.”
3. TRAUMATIC IMPLANTATION CYSTS
Implantation cysts are formed after trauma by the transplantation into
the eye of epithelial elements from the cornea or conjunctiva either as
separate islands or by the active ingrowth of the epithelium along the track
of a perforating wound, whether traumatic or surgical. They are by no
means uncommon. We have already seen that such implantations occur
in the cornea,” and that the entire anterior chamber may be converted into
a cyst lined by epithelium ; sometimes the epithelium is carried deeper and
is implanted into the iris. Occasionally a foreign body is introduced into the
eye at the same time, or an eyelash is carried into the anterior chamber,
when the epithelium of its root-sheath may become implanted, eventually
giving rise to a cyst-like proliferation.
The literature contains a large number of such cases. Mackenzie (1830) described
one in detail, and sporadic cases were recorded by Wharton Jones (1852), v. Graefe
(1857–64), Hulke (1869), and others, until the paper of Rothmund (1871–72) appeared
which established the pathology of the condition. He collected 36 cases of which 28
followed perforating wounds, 2 followed cataract operations, and 3 the inclusion of an
eyelash, and established the theory of the mechanical transference of epithelial
elements from the surface into the inner eye (see further Greeff, 1892; Meller, 1901;
Streiff, 1904; Kümmell, 1907; Urmetzer, 1908 ; Collins, 1914; and others).
Such cysts have been produced eacperimentally on several occasions in animals
by introducing into the anterior chamber foreign bodies, skin, mucous membrane,
or hairs (Dooremaal, 1873; Goldzieher, 1874; Masse, 1881–85; Hosch, 1885; Corrado,
1931 ; Suzuki, 1934; Accardi and Rossi, 1936; Perera, 1938; and others).
* Vol. II, p. 1321. * p. 3438. * Vol. II, p. 2042.
DISEASES OF THE UVEAL TRACT 2433
The implantation cysts thus formed may be of two distinct types: solid
looking round or oval tumours—pearl cysts; or translucent cysts with thin
walls.
PEARL TUMoURs appear as solid greyish-white, round or oval bodies,
usually situated in the peripheral parts of the iris stroma or in the angle of
FIG. 2019.-PEARL TUMoUR OF IRIs.
From an implanted lash (Collins).
the anterior chamber. Their nature was first clearly demonstrated by
Monoyer (1872); they may become evident a few months (Silva, 1905) or a
few years (Früchte, 1906) after injury, and sometimes they are associated
with an eyelash or a foreign body. The walls are formed of stratified or
Fig. 2020.-PEARL-LIKE IMPLANTATION Cyst of THE IRIs.
After the implantation of an eyelash. A, cyst-wall. B, keratinized cyst-
contents. C, iris stroma. D, Pars iridica retinae (Foster Moore, Brit. J. O.),
cubical epithelium, sometimes closely resembling that of the cornea or
conjunctiva, and the cells may be continued throughout arranged in
concentric lamellae like the layers of an onion. Frequently, however, the
central cells undergo degeneration ; the nuclei disappear, globules of fat or
cholesterol crystals are deposited often in considerable quantity, together


7, 2
2434 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2021.-Cyst of THE IRIs.
Cystic degeneration of the epithelium in an implantation tumour of the iris
containing wood fibres (Pickard, T. O. S.).
with indiscriminate granular material (Figs, 2019–20). Finally, the
degeneration may progress to the formation of cystic spaces, so that an
actual cyst may eventually be formed (Fig. 2021) (Strawbridge, 1878:
Fig. 2022.-Cyst of THE IRIs.
Cyst of the posterior chamber following a perforating injury 9 years previously
(Krug, Am. J. O.).


DISEASES OF THE UVEAL TRACT 2435
Snell, 1881; Cross and Collins, 1893; Wintersteiner, 1900; Wallner,
1905; Silva, 1905; Früchte, 1906; Pickard, 1906).
TRUE CYSTs are a more common formation—they may arise a few weeks
(Blaschek, 1905), or may not be noted for many years (14, Griscom, 1929)
FIG. 2023.-Cyst of THE PostERIOR CHAMBER.
The same case as Fig. 2022 showing recurrence after operation (Krug, Am. J.O.).
FIG. 2024.-Cyst of Post ERIoR CHAMBER.
The same case as Fig. 2023 in section (Krug, Am. J. O.).
after an injury (Figs. 2022–24). The cyst is composed of a relatively thin
wall, usually of ill-developed or irregular epithelium and contains a yellowish
or slightly turbid fluid ('' serous cysts"). The wall may, indeed, be so thin
as to be readily transparent clinically, and in one case v. Hippel (1913) saw
a hypopyon-like deposit which kept a horizontal level when the head was


2436 TEXT-BOOK OF OPHTHALMOLOGY
moved. The cyst usually occurs in the periphery of the iris stroma, the
anterior layers of which are stretched over it to become thin and atrophic ;
Fig. 2025.-EPITHELIAL Cyst of PostFRior CHAMBER.
The iris is adherent to the cornea and the space bounded by the iris, the ciliary
processes and the lens is lined by stratified epithelium (× 12) (Parsons).
as growth proceeds it advances into the anterior chamber and may become
adherent to the cornea (Fig. 2025), while the stretched tissues of the iris
Fig. 2026.-Cyst of THE
EYE.
Large epithelial cyst in a
shrunken eye (Collins).
anteriorly or the pigmented epithelium posteriorly
may seem to take part in the formation of its
walls (Stölting, 1885; Claiborne, 1903). Alterna-
tively, it may be more deeply placed and bulge
into the posterior chamber, appearing eventually
as a dark mass in the pupil; or it may develop
deeply situated in the ciliary region (Früchte,
1906; Chance and Goldberg, 1909; Marbourg,
1925; Tooker, 1933; Krug, 1938) (Fig. 2024).
Sometimes it is not limited to the iris alone,
for this may form the posterior wall and the
cornea the anterior ; or it may extend on to the
capsule of the lens (Fig. 2025). In one case in
which the lens was wounded, the epithelium of
the cystinvaded it also (Wintersteiner, 1900). Any
gradation, in fact, may occur up to the formation of a complete cyst of the
anterior chamber," while on rare occasions the cyst may grow until it fills
* Vol. II, p. 1817.


DISEASES OF THE UWEAL TRACT 2437
the cavity of a shrunken eye (Collins, 1891) (Fig. 2026). On the other hand,
the cyst wall may be continuous with the surface epithelium along the track
of the wound (Stölting, 1885), or the cavity of the cyst may protrude under
Fig. 2027.-EPITHELIAL Cyst of ANTERIOR CHAMBER,
Two and a half years after a corneal wound with prolapse of the iris. The cyst
lined with multi-layered epithelium occupies the entire anterior chamber, being con-
tinuous over the iris, and protrudes under the conjunctiva (x 5) (Parsons).
-º-
-
-
--
Fig. 2028.-INCLUsion Cyst of IRIs.
Following the implantation of an eyelash.
the conjunctiva (Fig. 2027). Finally, several cysts may occur together, some
purely corneal, some purely iridic, and some bounded by various portions of
the anterior chamber (Clausnitzer, 1911).


2438 TEXT-BOOK OF OPHTHALMOLOGY
The accidental intrusion of an eyelash into the interior of the eye is not uncom-
monly associated with an implantation cyst, either a pearl-tumour (v. Graefe, 1857 ;
Monoyer, 1872; Wintersteiner, 1900; Moore, 1930 ; Bonnet and Paufique, 1934) or
a serous cyst. The eyelash itself is sometimes seen in the cyst wall (Roth and
Geiger, 1925; Marbourg, 1925), but quite frequently lies some distance from it
(Moore, 1930) (Fig. 2028).
All kinds of wounds are aetiological factors, particularly accidental perforating
wounds with steel instruments, glass, and so on (even a lead pencil, Roth and Geiger,
1925). Foreign bodies of all kinds entering the eye have been the transplanting agent,
such as pieces of metal (Ahlström, 1903; Blaschek, 1905) or wood (Pickard, 1906).
Operative wounds involving perforation have frequently figured in the aetiology, and
in one case the perforation of the globe by a stitch in an operation for strabismus
was the immediate cause (which incidentally led to loss of the eye) (Brownlie and
Neame, 1923). It is important when considering the aetiology of “spontaneous * cysts
to realize how readily a small injury can be missed, for in several of the cases the
history has been quite negative although the presence of a foreign body in the eye
has proved the contrary.
The clinical course, prognosis and treatment of these cysts will be con-
sidered at the end of this section.”
Accardi and Rossi. Rass. It. d’O., v, 3, 1936.
Ahlström. Cb. pr. Aug., xxvii, 257, 1903.
Blaschek. Z. f. Aug., xiii, Erg., 804, 1905.
Bonnet and Paufique. A. d’O., li, 5, 1934.
Brownlie and Neame. Brit. J. O., vii, 497,
Marbourg. Am. J. O., viii, 147, 1925.
Masse. C. R. Acad. Sc., xcii, 797, 1881 ;
xcvi, 202, 1883.
Rystes, Tumeurs perlés et Tumeurs
dermoides de l’Iris, Paris, 1885.
1923. Meller. A. f. O., lii (3), 436, 1901.
Chance and Goldberg. O. Rec., xix, 201, 1909. Monoyer. Am. d’Oc., lxvii, 249, 1872.
Claiborne. T. Am. O. S., x, 588, 1903. Moore. Brit. J. O., xiv, 496, 1930.
Clausnitzer. K. M. Aug., xlix, 434, 1911. Perera. Am. J. O., xxi, 605, 1938.
Collins. T. O. S., xi, 133, 1891 ; xxxiv, 21, Pickard. T. O. S., xxvi, 62, 1906.
1914. Roth and Geiger. Am. J. O., viii, 870, 1925.
R. L. O. H. Rep., xiv., 12, 1895. Rothmund. K. M. Aug., ix, 397, 1871 ; x,
Corrado. An. di Ott., lix, 706, 1931. 189, 1872.
Cross and Collins. T. O. S., xiii, 199, 1893. Salus. K. M. Aug., lxxviii, 368, 1927.
Dooremaal. A. f. O., xix (3), 359, 1873. Silva. K. M. Aug., xliii (2), 450, 1905.
Früchte. K. M. Aug., xliv (2), 42, 1906. Snell. R. L. O. H. Rep., x, 208, 1881.
Goldzieher. A. f. earp. Path. u. Pharm., ii, Stölting. A. f. O., xxxi (3), 99, 1885.
388, 1874. Strawbridge. T. Am. O. S., ii, 385, 1878.
v. Graefe. A. f. O., iii (2), 412, 1857;
vii (2), 39, 139, 1860; x (1), 211, 1864.
Greeff. A. f. Aug., xxv, 395, 1892.
Griscom. Am. J. O., xii, 400, 1929.
Handmann. K. M. Aug., lxxii, 111, 1924;
lxxiv, 218, 1925.
v. Hippel. K. M. Aug., li (1), 520, 1913.
Hosch. A. f. path. Amat., xcix, 446, 1885.
IHudelo. A. d’O., lii, 410, 1935.
Hulke. R. L. O. H. Rep., vi, 12, 1869.
Jones, Wharton. Lancet, i, 568, 1852.
Krug. Am. J. O., xxi, 413, 1938.
Rümmell. K. M. Aug., xlv. (2), 421, 1907.
Mackenzie. Diseases of the Eye, 1830.
Streiff. A. f. Aug., l, 56, 1904.
Suzuki. Acta O. S. Jap., xxxviii, 553, 1934.
Terrien. A. d’O., xxi, 651, 1901.
Tooker. T. Am. O. S., xxxi, 187, 1933;
A m. J. O., xvii, 41, 1934.
Urmetzer. A. f. O., lxviii, 494, 1908.
Vail. A. of O., xv, 270, 1936.
Villard and Dejean. A. d’O., l, 91, 194. 272,
1933.
Wallner.
Wintersteiner.
I900.
Lubasch-Ostertag Ergeb. Path., x, Erg., 1906.
Diss., München, 1905.
B. O. G. Heidel.,
xxviii, 4,
4. ExuTATIVE CYSTS OF THE EPITHELLAL LAYERS
Cysts which are exudative in nature, and usually associated with
inflammatory conditions, are readily formed by a separation of the two
1 p. 2443.
DISEASES OF THE UVEAL TRACT 2439
layers of the retinal epithelium. They are quite commonly found in the
iris of eyes examined pathologically after long-standing inflammation: the
posterior layer of the epithelium is glued to the lens or to cyclitic fibrous
tissue and the anterior layer of the epithelium is separated from it and
together with the stroma is pressed forwards, the separation being presum-
ably due partly to the accumulation of fluid between the two layers owing
to obstruction of the circulation of the tissue-fluids and the increased
permeability of the vessels, and partly to the pull of the dilatator muscle on
the synechia. In addition to these two mechanical influences the factor
of active epithelial proliferation must also be considered, since the cyst wall
Fig. 2029.-Cyst of PIGMENTED EPITHELIUM of IRIs.
Five years after injury. Seclusio and occlusio pupillae; separation of the two
epithelial pigmented layers of the iris with the formation of numerous cysts. Anterior
capsular cataract and detached retina (x 7) (Parsons).
is much thicker than could be explained by a passive stretching of the
epithelium ; moreover, a frequent association is the formation of small solid
darkly pigmented nodules on the pupillary border (Weeks, 1914). A single
large cyst may be formed in this way which occupies the whole posterior
surface of the iris and may cause the clinical appearance of a pronounced
iris bombé (Fig. 2029), or appears as a dark mass above the pupillary
margin (Collins, 1890–96; Wilmer, 1929), or innumerable small cystic bodies
may surround the pupil. Thus in a case of chronic uveitis, Wilmer (1929)
observed 20 minute pedunculated translucent cysts at the pupillary border
the largest of which was not over 1 mm. in diameter. A similar cystic forma-
tion may occur in the ciliary epithelium involving both layers, as in a case

2440 TEXT-BOOK OF OPHTHALMOLOGY
of old chorio-retinitis described by Coats (1908), in which the eye was
excised under the diagnosis of ciliary sarcoma, or the post-traumatic cases
noted by Greefſ (1892) and Mayer (1899).
Cysts of a similar type between the two ectodermal layers are met with
in conditions other than inflammation where the circulation is impeded by
pressure. The most common is glau-
coma (Eales and Sinclair, 1896; Vogt,
1921–23), and the pressure of a neoplasm
on the root of the iris has produced a
like result (Collins, 1896) (Fig. 2030).
(Edema, without stasis determined
Fig. 2030 cºst or powest Epithelium by pressure, as a cause of cystic formation
- of IRIs. is exemplified in the posterior layers of
º º". º the iris and sometimes in the ciliary
containing a malignant melanoma of the body in diabetes, a subject which has
ciliary body (Collins). already been discussed."
Finally, when increased transudation from the capillaries is encouraged,
either by a sudden lowering of the intra-ocular pressure as by paracentesis
(Greeff, 1894; Carlini, 1910; Carrère, 1923; Samojloff, 1925–27),” or
when the permeability of the small vessels is increased in acute inflammation,”
cyst-like vesicles appear between the two layers of epithelium overlying the
ciliary body, and sometimes between those on the posterior surface of the
IITIS.
Carlini. A. f. O., lxxvii, 96, 1910. Mayer. Münch, med, W., xlvi. 854, 889,
Carrère. C. R. S. Biol., lxxxviii. 475, 1923. 1899.
Coats. R. L. O. H. Rep., xvii, 143, 1908. Samojloff. K. M. Aug., lxxv. 382, 1925.
Collins. R. L. O. H. Rep., xiii, 41, 1890. A. f. O., exviii, 391, 1927.
Researches, London, 1896. Vogt. K. M. Aug., lxvii. 330, 1921.
Eales and Sinclair. T. O. S., xvi. 56, 1896. A. f. O., exi, 91, 1923.
Greeff. A. f. Aug., xxv, 395, 1892; xxviii, Weeks. A. of O., xliii, 483, 1914.
178, 1894. Wilmer. A. of O., i, 162, 1929.
5. DEGENERATIVE cysts are seen in the cystic formations which occur in the ciliary
epithelium in senile atrophy (Kuhnt, 1881), a question of which we have already dis-
cussed." It will be remembered that they may beformed by a simple detachment of the
ciliary epithelium or by actual proliferation of the cells (Brailey, 1882–1907), and that,
while they are usually small and invisible clinically, they may on occasion, especially in
post-inflammatory states, assumesuch a size that they become apparent in the pupillary
aperture (Greeff, 1892), and have led to the excision of an eye under the diagnosis of a
malignant growth (Schieck, 1904).
Brailey. T. O. S., ii, 63, 1882; xxvii, 95, 1907. Kuhnt. B. O. G. Heidel.., xiii, 38, 1881.
Greeff. A. f. Aug., xxv, 395, 1892. Schieck. K. M. Aug., xlii (2), 341, 1904.
6. IDIOPATHIC or Spont'ANEous Cysts
When the causes which we have already considered have been
eliminated, a considerable number of cases of cysts remain which have
p. 2383. * Vol. I, p. 511 (520). * p. 2161. 4 p. 2396.

DISEASES OF THE UVEAL TRACT 2441
not been accounted for, and have occurred in the absence of trauma,
inflammation, glaucoma or other predisposing conditions. Thus Tertsch
(1914), collecting from the literature 51 cases of non-traumatic cysts of the
iris, concluded that 41 were spontaneous. They may occur in two situations
—in the stroma or between the two epithelial layers on the posterior
surface of the iris.
(a) SPONTANEOUS CYSTS OF THE IRIs STROMA
Spontaneous cysts of the iris stroma are not common ; the great
majority occurs in young persons, half of them before the tenth
year and two-thirds before the twentieth—strong evidence in favour of
their probable developmental aetiology. The oldest case on record was
58 years of age. They appear almost invariably in normal eyes as grey or
dark spots in the iris stroma which grow slowly to form semi-transparent
vesicles, exciting little or no reaction, over which the stretched and atrophic
iris remnants may appear only in the form of flecks of pigmented or
vascularized tissue. They rarely give rise to irritation or increased tension.
Since the original reports of Bosteels (1864), Krükow (1875) and Schröter (1877),
relatively few cases of this type have been recorded : indeed, a study of the literature
reveals only some 41 cases which can unequivocally be accepted as such. Tertsch
(1914) reviewed 37 cases in detail ; but some of them were probably not spontaneous.
Since his paper the following cases have appeared—Böhm (1916), Rados (1919),
McMullen (1920), Koch (1921), Wood (1925), Filippow (1930) and Shunway (1931).
The histology and nature of these cysts have given rise to much con-
troversy ; nor can the question be yet decided. Many of the older
authors, and some present-day writers, consider that a number are
mesodermal in origin, their frail walls being composed of endothelial cells.
Sattler (1874) first suggested that their formation was excited by some
unnoticed irritant, as a microscopic foreign body, and Schmidt-Rimpler
(1889), who endorsed their endothelial nature, considered that they repre-
sented the closure of crypts of the iris by the endothelium lining the surface.
This view was supported by Collins (1890) and Engelen (1900), while others
suggested that the endothelium may introduce itself between the two leaves
of the mesodermal stroma (cleft-cysts) (Ginsberg, 1895; Terrien, 1901 ;
Monzon, 1902; Strieff, 1904). The view is still advocated that such endo-
thelial cysts occur after trauma as mesodermal retention cysts (Hudelo, 1935).
There is no doubt, however, that other similar cysts are ectodermal in
origin, and have a developmental basis ; and it is claimed that the cells
interpreted as endothelial in the so-called mesodermal retention cysts, are
in reality altered ectodermal cells. In some of them the wall is composed of
several layers of cubical or cylindrical cells, sometimes with goblet cells,
resembling those of the surface epithelium ; in others the epithelium is
frailer and has some resemblance to the epithelium of the iris, occasionally
2442 TEXT-BOOK OF OPHTHALMOLOGY
being pigmented. These epidermal cells appear to have migrated in
embryonic life either from the surface ectoderm, as occurs normally
in the case of the lens vesicle, or from the anterior pigment layer, as occurs
normally in the case of the pupillary musculature and the clump cells,
with which layer, indeed, some cysts have had anatomical continuity. Nadal
(1911) suggested the third possibility of a migration of cells secondarily from
the lens vesicle. Implanted into their new medium, these cells undergo
proliferation to form developmental implantation cysts (Rosenweig, 1894;
Lagrange, 1900; Bardelli, 1906; Gallemaerts, 1907; Juselius, 1908;
Lauber, 1908 ; Puccioni, 1908 ; Passera, 1909; Worth, 1910 ; Böhm, 1916;
Rados, 1919 ; Koch, 1921 ; Butler, 1921 ; Hudelo, 1935; and others).
Such cysts certainly do occur ; but whether they represent the only origin
of this type of cyst, or whether both types, retention and implantation,
exist, cannot yet be decided on our present knowledge.
Bardelli. A m. di Ott., xxxv, 660, 1906. Monzon. These de Paris, 1902.
Böhm. K. M. Aug., lvi, 70, 1916. Nadal. A. d’O., xxxi, 363, 19 l l .
Bosteels. An. d’Oc., lii, 175, 1864. Passera. Ophthalmologica, i, 503, 1909.
Butler. T. O. S., xli., 452, 1921. Puccioni. La Clin. Oc., ix, 3181, 1908.
Collins. R. L. O. H. Rep., xiii, 41, 1890. Rados. A. f. O., xcix, 152, 1919.
Engelen. Diss., Marburg, 1900. Rosenweig. Beit. z. Aug., xiv, 34, 1894.
Filippow. K. M. Aug., lxxxiv, 247, 1930. Sattler. K. M. Aug., xii, 127, 1874.
Gallemaerts. A. d’O., xxvii, 689, 1907. Schmidt-Rimpler. A. f. O., xxxv (1), 147,
Ginsberg. Cb. pr. A ug., xix, 338, 1895. 1889.
Hudelo. -4. d’O., lii, 410, 1935. Schröter. K. M. Aug., xvi, 417, 1877.
Juselius. K. M. Aug., xlvi (2), 300, 1908. Shunway. Am. J. O., xiv, 792, 1931.
Roch. K. M. Aug., lxvii, 407, 1921. Strieff. A. f. Aug., l, 56, 1904.
Krükow. A. f. O., xxi, 213, 1875. Terrien. A. d’O., xxi, 651, 1901.
Lagrange. A. d’O., xx, 272, 1900. Tertsch. A. f. O., lxxxviii, 72, 1914.
Lauber. A. f. O., lxviii, l, 1908. Wood. Brit. J. O., ix. 450, 1925.
McMullen. Proc. R. S. Med., Sect. O., xiii, Worth. R. L. O. H. Rep., xviii, 52, 1910.
77, 1920.
(b) sponTANEOUs CYSTs OF THE PIGMENTARY EPITHELIUM
It is now generally accepted that spontaneous cysts appearing in
the pigmentary epithelium of the iris are explicable on a congenital basis
and represent a persistence of the cavity of the primary optic vesicle.” The
persistence of the annular sinus at the anterior termination of the vesicle for
some time after the remainder of the cavity has disappeared accounts for
the occurrence at the pupillary margin of smaller cysts (Worth, 1910 ;
Bliedung, 1921 ; Vogt, 1921–23), which may be found in any gradation in
size down to small cystic flocculi,” and also probably explains the occasional
presence of free cystic bodies in the anterior chamber.” It is not to be
thought, however, that these are formed entirely passively ; the densely
thick walls of some and the tendency of most to grow in size indicate
proliferative activity on the part of the epithelial cells, while the possibility
of the cellular wall secreting fluid is suggested by the observation of Fuchs
(1885) of a free ectodermal cyst in the anterior chamber which intermittently
1 Vol. II, p. 1321. * Vol. II, p. 1319. Vol. II, p. 1298.
DISEASES OF THE UVEAL TRACT 2443
emptied and filled itself. It is interesting that such cysts may be multi-
locular or multiple and may occur both in the iris and ciliary body
(Pagenstecher, 1910; Stephenson, 1916; Braunstein, 1925).
Such cysts, in contra-distinction to spontaneous cysts in the stroma,
are met with in adult life, and although they may remain quiescent for a
long time, the tendency is for them to grow steadily, ultimately bringing on
secondary glaucoma and loss of vision. -
Although relatively rare, a considerable literature has built itself upon
the subject since the early observations of Wharton Jones (1852), Bosteels
(1862) and Hulke (1867). Representative cases occurring in the iris which
have been pathologically examined are those of Collins (1891), Schieck
(1904), Wintersteiner (1906), Gilbert (1910), Pagenstecher (1910), Fischer
(1920), Bliedung (1921), Braunstein (1925), Granström (1930), and Town
(1933). In the ciliary epithelium the cases of Rabitsch (1904), Lauber
(1908), Ichikawa (1909), Pagenstecher (1910), Remky (1923), and Elschnig
(1925) are noteworthy. In most cases the eyes have been removed for
absolute glaucoma or because a diagnosis of a malignant tumour had been
made, by which time the cyst had grown to some considerable size; the
posterior wall is usually formed by heavily pigmented epithelium, but the
anterior wall may be formed of unpigmented epithelial cells, which may
be closely associated with stretched-out stroma. In his case Hanssen (1918)
considered the anterior wall made up of deformed dilatator cells.
Bliedung. K. M. Aug., lxvii, 401, 1921. Pagenstecher. A. f. O., lxxiv, 290, 1910.
Bosteels. A n. S. méd. Anvers, xxv, 42, 1862. Rabitsch. Cb. pr. Aug., 321, 1904.
Braunstein. A. f. O., czv, 380, 1925. Remky. K. M. Aug., lxx, 347, 1923.
Collins. R. L. O. H. Rep., xiii, 41, 1891. Schieck. K. M. Aug., xlii (2), 341, 1904.
Elschnig. K. M. Aug., lxxiv, 783, 1925. Stephenson. T. O. S., xxxvi, 270, 1916.
Fischer. K. M. Aug., lxv, 876, 1920. Tertsch. A. f. O., lxxxviii, 72, 1914.
Fuchs. A. f. Aug., xv, 7, 1885. Town. A m. J. O., xvi, 790, 1933.
Gilbert. K. M. Aug., xlviii (1), 149, 1910. Vogt. K. M. Aug., lxvii, 330, 1921.
Granström. Acta O., viii, 35, 1930. A. f. O., czi, 91, 1923.
Hulke. R. L. O. H. Rep., vi, 12, 1867. Wintersteiner. B. O. G. Heidel.., xxxiii, 345,
Ichikawa. K. M. Aug., xlvii (1), 73, 1909. 1906.
Jones, Wharton. Lancet, i, 568, 1852. Worth. R. L. O. H. Rep., xviii, 52, 1910.
Lauber. A. f. O., lxviii, 1, 1908.
The Clinical Course, Diagnosis and Treatment of Uveal Cysts
Occasionally—especially in the case of cysts in the stroma—cysts of
the iris cease to grow, and may remain unchanged for an indefinite period.
In exceptional cases they may even become smaller or disappear (Fehr,
1902; Früchte, 1906; Wolfrum, 1914—all in traumatic implantation
cysts); but if they do rupture they almost invariably fill up again, and
the more usual course is that of a slow and steady increase in size. In
this growth three stages occur :— .
1. A symptom-free period, when no discomfort or visual disturbance
8, TISéS.
2444 TEXT-BOOK OF OPHTHALMOLOGY
2. An irritative period when, especially in traumatic cysts, an irido-
cyclitis may develop.
3. A period of raised tension which results in absolute glaucoma, vision
being completely destroyed and excision of the eye being usually
necessary because of pain.
It is important, therefore, as soon as the diagnosis of an intra-ocular cyst is
made, to arrange for adequate treatment when it is yet small, especially if
it is seen to be growing in size, since the only ultimate result which can be
legitimately expected if events are allowed to take their course is loss of the
eye.
The diagnosis, however, of these cysts is not always an easy matter ;
and in few conditions is care in taking the history and meticulous exactitude
in making the clinical examination of more importance. The history should
sift the questions of trauma, how long the cyst has been present, whether
other people have noted it, and if it has been observed to grow. The clinical
examination should include a minute search for a perforating wound, a
determination of the mobility of the pupil and any question of infiltration of
the tissues, the presence of new blood-vessels or unusual pigment prolifera-
tion, and, above all, the transparency. This last should be investigated by
dia-scleral illumination (Plate IX, Fig. 1065) and with the direct beam of the
slit-lamp. Exceptionally the diagnosis is facilitated by the cyst altering its
position over the pupillary margin when the head is moved (Stevenson,
1919). In summary, the features which may indicate the diagnosis of a cyst
in contra-distinction to a tumour are therefore—multiplicity, tremulousness,
mobility, the presence of a notched margin, and, above all, translucency.
It is to be remembered that a cyst can itself cause an irido-cyclitis, and
consequently, in the case of cysts of the stroma, nodular inflammations, such
as tubercle, syphilis, and so on, must be eliminated. The most important
differential diagnosis, however, concerns a neoplasm, which almost invariably
means a malignant tumour, a problem which is most difficult to clear up in the
case of cysts in the posterior chamber. The difficulties may be considerably
aggravated by the fact that a cyst in this position may have walls so thick
that transillumination is impossible, but it is to be remembered that
translucency can be obtained with the slit-lamp even when dia-scleral
illumination would indicate a solid growth (Lindemeyer, 1927; Wissmann,
1927). Even so, the list of cysts in the literature which have been excised
as tumours by competent observers is significant (Schieck, 1904 ;
Wintersteiner, 1906; Coats, 1908; Pagenstecher, 1910; Stephenson, 1916;
Remky, 1923; Marburg, 1925; Roth and Geiger, 1925). If doubt is felt
as to the presence of a neoplasm, an iridectomy should be done if the mass is
excisable, and if not a diagnostic puncture may be performed, which, if
negative, should be followed by removal of the eye. Frequently this is an
easy and valuable procedure (Fuchs, 1911); occasionally it leads to a mishap,
as in a case described by Juler (1911) wherein puncture was followed
DISEASES OF THE UVEAL TRACT 2445
by a hyphaema, whereupon excision of the eye revealed a malignant
melanoma.
The treatment of election in dealing with cysts of the iris is their complete
excision in an iridectomy. This can only be attempted with hope of success,
however, if the cyst is small, localized towards the pupillary edge of the iris
leaving the root of this tissue clear (a point best demonstrated by gonioscopy),
and if the pupil is mobile and the tissues are not infiltrated. When these
ideal conditions do not obtain, or the cyst is so large that an iridectomy
is impossible, several courses are open, but probably the most satis-
factory procedure is to remove as much as possible surgically—and a great
deal can be removed if a sufficiently large corneal section is folded back
under complete akinesia—followed by radiation. It is quite definitely the
case that several cysts have not recurred after what must have been incom-
plete surgical removal (Verhoeff, 1938); but unfortunately this experience
is not invariable. On the other hand, a cure has followed the simple expedient
of collapsing the cyst and pulling it out with forceps (Butler, 1921). On the
whole, however, if radical surgical excision appears impossible other methods
may be considered in conjunction with it, the most promising of which is
radiation.
Radiation by mesothorium was first attempted by Axenfeld (1918) and
Jendralski (1922) with good results, particularly in implantation cysts.
|X-rays and radium have been shown to kill experimentally induced epithelial
ingrowths in animals (Suzuki, 1934); and the clinical successes reported
with their use justifies the risk of their employment, especially as an
adjuvant measure after incomplete surgical removal (Handmann, 1924–25;
de la Vega and Magdalena, 1926; Salus, 1927; Custodis, 1933; Wail,
1936; Federici, 1937; and Perera, 1938). An alternative procedure is to
aspirate the cyst and inject its cavity with iodine (Schöler, 1911; Alger,
1932) or wash it out with pure carbolic acid (Wright, 1925). This manoeuvre
is best done by inserting two needles into the cyst, one for aspiration and one
for injection ; or alternatively leaving the one needle in during the manipula-
tions, since entrance into a collapsed cyst is impossible. A final suggestion is
to puncture the cyst with an electrolytic or diathermy needle and thus
destroy it (Villard and Dejean, 1933).
The older methods of seeking to rupture the cyst by alternate extreme contraction
and dilatation of the pupil (Eversbusch, 1893), puncture or transfixation of the cyst,
or of its sub-conjunctival drainage are occasionally successful (Reid, 1928), but more
usually not.
Alger. A. of O., vii, 984, 1932. Fehr. Cb. pr. Aug., xxvi, 232, 1902.
Axenfeld. B. O. G. Heidel.., xli., 312, 1918. Früchte. K. M. Aug., xliv (2), 42, 1906.
Butler. T. O. S., xli., 451, 1921. Fuchs. T. Am. O. S., xii, 884, 1911.
Coats. R. L. O. H. Rep., xvii, 143, 1908. Handmann. K. M. Aug., lxxii, 111, 1924;
Custodis. K. M. Aug., xc, 361, 1933. lxxiv, 218, 1925.
Eversbusch. B. O. G. Heidel., xxiii, 216, 1893. Jendralski. K. M. Aug., lxviii, 175, 1922.
Federici. Boll. d’Oc., xvi, 112, 1937. Juler. T. O. S., xxxi, 44, 131, 1911.
244t;
TEXT-BOOK OF OPHTHALMOLOGY
Lindemeyer. K. M. Aug., lxxix, 650, 1927.
Marburg. Am. J. O. viii, 147, 1925.
Pagenstecher. A. f. O., lxxiv, 290, 1910.
Perera. Am. J. O., xxi. 605, 1938.
Reid. T. O. S., xlviii. 88, 1928.
Remky. K. M. Aug., lxx, 347, 1923.
Z. f. Aug., li, 113, 1923.
Roth and Geiger. Am. J. O., viii. 870, 1925.
Salus. K. M. Aug., lxxviii. 368, 1927.
Schieck. K. M. Aug., xlii (2), 341, 1904.
Suzuki. Acta O. S. Jap., xxxviii. 553, 1934.
Vail. A. of 0., xv, 270, 1936.
de la Vega and Magdalena. A. de Oft., B.A.,
i, 143, 1926.
Verhoeff. Am. J. O., xxi, 419, 1938.
Villard and Dejean. A. d’O., 1, 91, 194, 272,
1933.
Wintersteiner. B. O. G. Heidel. xxxiii, 345,
1906.
Wissmann. K. M. Aug., lxxix., 649, 1927.
Schöler. K. M. Aug., xlix (1), 703, 1911. Wolfrum, Lubarsch-Ostertag's Ergeb., xvi,
Stephenson. T. O. S., xxxvi, 270, 1916. Erg. Bd., 1914.
Stevenson. Brit. J. O., iii. 555, 1919. Wright. Brit. J. O., ix, 454, 1925.
VII. TUMIOURS OF THE UWEAL TRACT
I. Primary Tumours
A. Epithelial Tumours
1. SIMPLE MELANoMA of THE PIGMENT LAYER of THE IRIs
A simple hyperplasia of the pigmented layers on the posterior surface
of the iris sometimes assumes dimensions which entitle it to be classed
among tumours (Fuchs, 1882; Anargyros, 1903; Stock, 1905; Gilbert,
1910–21; Schmidt, 1934) (Fig. 2031). The growth may be solid or assume
Fig. 2031.-SIMPLE MELANoMA of THE IRIs.
(After Anargyros.)
a cystic shape, and may appear over the pupillary border as a brown mass
(Stock, 1905) or be seen through the iris stroma giving the appearance
of a naevus (Gilbert, 1910); after the pupillary margin the most common
site is opposite the peripheral edge of the sphincter where the epithelium in
any event tends to invade the stroma. The melanoma consists of masses
of deeply pigmented cells in the substance of the iris continuous with the
posterior epithelium, sometimes without any stroma (Anargyros, 1903),
sometimes accompanied by blood-vessels (Stock, 1905), and sometimes by
a sparse amount of connective tissue (Gilbert, 1910–21). Collins (1926)
reported an extensive growth of the pigmentary cells forwards into the stroma
following an injury which involved a partial iridodialysis. Occasionally a
melanoma starts in association with the origin of the dilatator fibres, which,
of course, are ultimately derived from the retinal epithelium (Klien, 1936).

DISEASES OF THE UVEAL TRACT 2447
Such melanomata are comparable to the congenital hyperplasia of the pigment
border of the iris which may be localized in flocculi" and are normally present in the
horse.* A similar formation is frequently seen as an epiblastic hypertrophy at the
edge of a coloboma (Mann, 1924).
Anargyros. A. f. Aug., xlvi. 62, 1903. Klien. A. of O., xv, 985, 1936.
Collins. T. O. S., xlvi. 86, 1926. Mann. T. O. S., xliv, 161, 1924.
Fuchs. A. f. Aug., xi, 435, 1882. Schmidt. A. f. Aug., cviii, 457, 1934.
A. f. O., lxviii, 534, 1908. Stock. K. M. Aug., xliii (1), 503, 1905.
Gilbert. K. M. Aug., xlviii (1), 149, 1910.
A. f. Aug., lxxxviii, 143, 1921.
2. EPITHELLAL HYPERPLASIA
We have already seen that in degenerative or inflammatory conditions
the epithelial layers, both on the posterior surface of the iris and over the
ciliary body, may show hyperplastic activities. It will be recalled, for
Fig. 2032–EPITHELIAL HYPERPLAs.I.A of THE CILIARY Body (Whiting, T. O. S.).
example, that such a hyperplasia may result in the proliferation of long
tubules of ciliary epithelium into the substance of a cyclitic membrane, and
that in old age a hyperplasia of both epithelial layers, but especially of the
non-pigmented one, is of constant occurrence forming small excrescences
projecting into the vitreous (Kuhnt, 1881; Kerschbaumer, 1888). In old
age, especially when associated with long-continued inflammation, this
hyperplastic process may be so marked as to produce an epithelial tumour,
the stimulus presumably being prolonged and continued irritation (Fig. 2032)
(Hanke, 1899; Parsons, 1903; Coats, 1908; Meller, 1913; Velhagen,
* Vol. II, p. 1319. * Vol. I, p. 74.
T.O.-WOL. III. A. A

2448 TEXT-BOOK OF OPHTHALMOLOGY
1917; Wunderlich, 1921; Whiting, 1922; Szabo, 1932). Sometimes small
compact tumours may be formed in this way, the bulk of them being com-
posed of cells resembling the unpigmented
epithelium, the pigmented layer playing
a secondary and more passive part
(Fig. 2033); exceptionally the growth is
derived almostentirely from the epithelium
of the pigmented layer (Zentmayer, 1936).
Hyaline degeneration is common, and
depigmentation of the inner layer of cells
may occur. As a rule such tumours give
rise to no clinical symptoms and are only
discovered pathologically by accident.
More rarely they may attain a size such as
to bulge out the iris so that their presence
is inferred (Zentnayer, 1936), while ex-
ceptionally they may attain large dimen-
sions causing a detached retina, local pain
and epibulbar congestion, so that the eye
- - - - --- º
- --- -
--- º º
º
---
Fig. 2033.-EPITHELLAL HYPERPLAs.I.A.
of the CILLARY Body.
There is an external lining of pig-
mented epithelium on the inner
surface of which the unpigmented
epithelium proliferates into the cavity
of the cyst in the form of folds. These
appear as strands of cells placed base
to base without connective tissue or
blood-vessels (Coats, R. L. O. H.
Rep.).
has been excised under the diagnosis of
malignancy (Stewart, 1931). At other
times pigmented and non-pigmented
epithelium may proliferate irregularly to
fill the globe almost entirely, producing a
condition which might well be considered
locally malignant (Schlipp, 1899)."
In the older literature there was no
unanimity as to the nature of these tumours.
Misled by the tubular form which the epithelium frequently adopted, many of the
early writers classified them as ADENoMATA (Fuchs, 1883; Collins, 1891–96: Pergens,
1896; Alt, 1898); but no true glandular structure is present. Hanke (1899) con-
sidered his case an epithelioma, Lagrange (1901) an endothelioma, and finally Parsons
(1903) compared it to a papillomatous hyperplasia. This view has now been generally
adopted, and the term employed by Fuchs (1908) of BENIGN EPITHELIoMA of the
ciliary epithelium is most usually employed.
Alt. Am. J. O., xv. 321, 363, 1898. Meller. A. f. O., lxxxv, 191, 1913.
Coats. R. L. O. H. Rep., xvii, 143, 1908. Parsons. R. L. O. H. Rep., xv. 375, 1903.
Collins. T. O. S., xi, 61, 1891; xiv, 83, 1894. Pergens. A. f. Aug., xxxii, 293, 1896.
R. L. O. H. Rep., xiii, 58, 1890. Schlipp. A. f. O., xlviii (2), 353, 1899.
Researches, London, 1896. Stewart. T. O. S., li, 616, 1931.
Fuchs. A. f. O., xxix. (4), 209, 1883; lºviii, Szabo. K. M. Aug., lxxxviii, 201, 1932.
534, 1908. Velhagen. K. M. Aug., lviii (1), 239, 1917.
Hanke. A. f. O., xlvii (3), 474, 1899. Whiting. T. O. S., xlii, 101, 1922.
Kerschbaumer. A. f. O., xxxiv. (4), 28, 1888. Wunderlich. K. M. Aug., lxvi. (1), 217, 1921.
Kuhnt. B. O. G. Heidel.., xiii, 49, 1881. Zentnayer. A. of 0., xvi, 677, 1936.
Lagrange. Tumeurs de l'OEil, Paris, i, 1901.
p. 2450.

DISEASES OF THE UVEAL TRACT 2449
3. EPITHELIoMA (MEDULLO-EPITHELIoMATA)
Primary malignant epithelial tumours are limited in the uveal tract to
the ciliary epithelium, and are thus essentially retinal.
Anomalous cases, it is true, have been reported occurring in association with the
pigment epithelium of the iris (Robertson, 1895; Hirschberg and Birnbacher, 1896),
but these are susceptible of other explanations (Emanuel, 1900; Wintersteiner,
1906; Fuchs, 1908).
The number of malignant epithelial tumours which have been reported
as occurring in the ciliary epithelium is small, so small, indeed, as to make
them great rarities and to render their classification and appreciation
difficult. This is the more unfortunate since the neoplasms which have been
described differ considerably from one another. The classical paper on the
subject is that of Fuchs (1908), who divided such neoplasms into two main
FIG. 2034.—DYKTYoMA of THE CILLARY BoDY.
General view of the neoplastic tissue which filled the entire ocular cavity (x 24)
(Soudakoff, A. of 0.).
groups, the one resembling embryonic retina (DIKTYoMATA), and the other
retaining the simple structure of the ciliary epithelium (MALIGNANT
EPITHELIoMATA of THE CILLARY Body). The entire group may be classed
as MEDULLO-EPITHELIoMATA in so far as they arise from cells corresponding
to the medullary epithelium of the primitive neural tube (Grinker, 1931)."
(a) DIKTYoMA
DIRTYoMATA are epithelial tumours growing from the ciliary body
which have the structure of embryonic retina before its layers are differen-
tiated. They occur in young children, usually quite young, the oldest
recorded cases being 16 years (Boeck, 1929) and 28 years (Soudakoff, 1936);
in the latter case, however, the tumour was in a very advanced stage.
* p. 2839.

2450 TEXT-BOOK OF OPHTHALMOLOGY
Clinically a whitish, irregular, unpigmented growth is seen arising from the
ciliary body, spreading over the posterior surface of the iris and the anterior
surface of the lens, while small nodules usually grow from its surface.
Progress may be slow—thus one case was observed for 7 years (Spicer and
Greeves, 1914)—but eventually the neoplasm may fill the entire globe and
lead to perforation through the sclera (Soudakoff, 1936). The tumour is
locally malignant, systemic metastases being unknown.
Histological examinations have been made by Badal and Lagrange
(1892), Emanuel (1900), Verhoeff (1904), Kuthe and Ginsberg (1905),
Spicer and Greeves (1914), Velhagen (1917), Satanowsky (1928), Boeck
Fig. 2035. Dyktyoma or THE CILiary Body.
From the same specimen as Fig. 2034, showing folding and interlacing of bands
of tumour cells to form tubules and rosettes with little connective tissue between.
M. indicates mitotic figures (Soudakoff, A. of 0.).
(1929), and Soudakoff (1936) (Figs. 2034–35), The tumour is made up of con-
voluted bands of cells arranged in one or several rows formed in intricate
convolutions upon folded and interlacing membranes. The histological
picture may be thus one of extreme complexity, sections showing at one
place cylindrical or oval tubes and at another round rosette formations.
The cells arranged in bands and columns resemble the non-pigmented
epithelium, while those lying free are like those of embryonic retina. Glial
fibres are not present. Frequently the cells become vacuolated, and there
may be groups of solitary cells undergoing active karyokinesis, or individual
cells invading the tissues around.
(b) MALIGNANT EPITHELIoMA of THE CILLARY EPITHELIUM
In contra-distinction to diktyomata, malignant epitheliomata of the
ciliary epithelium occur in adults, and usually in eyes which have had severe

DISEASES OF THE UVEAL TRACT 2451
inflammation. The clinical appearance is that of a small, dark, non-translucent
nodule bulging out the iris from behind and eventually appearing over the
pupillary margin (Fig. 2036). An iridectomy at this stage may be easily
done leaving the tumour in situ behind the coloboma (Heine, 1920) (Fig. 2037)
Fig. 2036.-PRIMARY EPITHELIoMA of THE CILIARY Body.
With dilated pupil (Heine, T. O. S.).
Fig. 2037.-PRIMARY EPITHELIoMA of THE CILLARY BoDY.
The growth seen in the coloboma of an iridectomy (Heine, T. O. S.).
Eventually, however, after intermittent attacks of raised tension, the growth
may completely fill the eye and infiltrate its coats (Snell, 1899).
Pathological studies on these tumours reveal a considerable diversity of structure,
but in general terms a single layer of cells is present, as in the ciliary epithelium, but
arranged in intricate convolutions (Figs. 2038–39) (Collins, 1894; Schlipp, 1899,
Fuchs, 1908; Greeves, 1911; Meller, 1913; Heine, 1920; Mårtens, 1921; Merkel, 1926:


2452 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2038. PRIMARY EPITHELIoMA of THE CILLARY Body.
A low-power view of the tumour of Figs. 2036 and 2037 extending forwards from
the ciliary process beneath the lens (Heine, T. O. S.).
Barrow and Stallard, 1932). Fuchs (1908) divided them into three sub-groups accord-
ing to their intimate structure, the first consisting of cell-membranes and tubules
(Collins, 1891–99), the second consisting of cell-tubules only (Fuchs, 1908), and the
Fig. 2039.-PRIMARY EPITHELIoMA of THE CILLARY Boby.
High-power view of the tumour in Fig. 2038 showing tubules surrounded by a
layer of columnar cells which in places appear duplicated owing to oblique cutting
(Heine, T. O. S.).


DISEASES OF THE UVEAL TRACT
2453
third of a quite irregular arrangement (Schlipp, 1899).
It is questionable, however,
if such a classification is of much value, as subsequent cases have refused to fit easily
into such a scheme, and their classification would necessitate almost as many sub-
groups as cases.
Badal and Lagrange. A. d’O., xii, 143, 1892.
Barrow and Stallard. Brit. J. O., xvi, 98,
1932.
Boeck. Z. f. Aug., lxix., 17, 1929.
Collins. T. O. S., xi, 55, 1891 ; xiv, 83, 1894;
xix., 100, 1899.
Emanuel. A. f. path. Anat., clxi, 338, 1900.
Fuchs. A. f. O., lxviii, 534, 1908.
Greeves. T. O. S., xxxi, 261, 1911.
Grinker. A. of O., v, 920, 1931.
Heine. T. O. S., xl, 146, 1920.
Mårtens.
A. f. Aug., lxxxix, 1, 1921.
Meller.
A. f. O., lxxxv, 191, 1913.
Merkel. A. f. Aug., xcvii, 308, 1926.
Michel. A. f. O., xxiv. (1), 131, 1878.
Robertson. O. Rev., xiv, 374, 1895.
Satanowsky. Semana méd., ii, 1017, 1928.
Schlipp. A. f. O., xlviii, 353, 1899.
Snell. T. O. S., xix, 99, 1899.
Soudakoff. A. of O., xv, 680, 1936.
Spicer and Greeves. P. R. S. Med., viii, 9,
1914.
Hirschberg and Birnbacher.
xx, 289, 1896.
Kuthe and Ginsberg. Beit. z. Aug., Festschr.
J. Hirschberg, 127, 1905.
Cb. pr. Aug., Velhagen. K. M. Aug., lviii (1), 239, 1917.
Verhoeff. T. Am. O. S., x, 351, 1904.
Wintersteiner. B. O. G. Heidel.., xxxiii, 345,
1906.
4. NEUROBLASTOMA
While, as we shall see,” it is relatively common for an extension of a retinal neuro-
blastoma to invade the uveal tract, the primary origin of such a tumour in the pro-
longation of the retina over the ciliary body and iris is rare. Axenfeld (1907), Weekers
(1908), and Pascheff (1924) have reported such tumours in the iris, and Hirschberg and
Happe (1870) and Emanuel (1900) described cases which they interpreted as gliomata
of the pars ciliaris retinae. Helfreich (1875) also described a gliomatous proliferation
of the ciliary processes in a microphthalmic eye. These tumours will be considered
in detail in the section on diseases of the retina.”
Axenfeld. B. O. G. Heidel., xxxiv, 300, 1907. Hirschberg and Happe. A. f. O., xvi (1),
Emanuel. A. f. path. Anat., clxi, 338, 296, 1870.
1900. Pascheff. B. O. G. Heidel., xliv, 261, 1924.
Helfreich. A. f. O., xxi (2), 236, 1875. Weekers. K. M. Aug., xlvi (1), 26, 1908.
5. GLIOSIS
Small masses of glial cells are sometimes met with as a histological accident in
the choroid or lying between this tissue and the retina, particularly in myopic eyes
or in old inflammatory lesions. These may be interpreted as proliferations of glial
tissue through a tear in the basement membrane caused either by stretching or by
inflammatory destruction ; but their occurrence apart from myopia and inflammation
and their association with epithelial pigmented cells led Kreibig (1933) to believe that
they might be formed by a direct metaplasia of the pigmentary epithelium.
Seefelder (1921) recorded a curious case which seemed to lie midway between a
melanoma and a gliosis of the epithelial layers of the iris.
Kreibig. Z. f. Awg., lxxx, 23, 1933. Seefelder. A. f. O., cv, 271, 1921.
1 p. 2817. * p. 2839.
24.54 TEXT-BOOK OF OPHTHALMOLOGY
B. Mesodermal Tumours
1. MYoMA
Although myomata of the iris are probably epiblastic in nature, con-
forming to the origin of the iris musculature, it is convenient to consider all
myomata together. In the iris they usually form sharply circumscribed
tumours, appearing as yellowish-grey nodules without clinical symptoms,
which grow slowly and are relatively benign, but if not completely excised,
may recur (Verhoeff, 1936). Clinically a myoma is quite indistinguishable
from a malignant melanoma, and for this reason the eye has usually been
enucleated.
While a myoma is impossible to diagnose clinically, its pathological
Fig. 2040.-LEIoMyoma or THE IRIs.
Low-power view showing interlacing bundles of tumour cells invading the
sphincter (Frost, Am. J. O.).
recognition is by no means easy. The neoplasm is characterized by closely
packed bundles of long spindle-shaped cells with an eosinophilic cytoplasm
and rod-shaped nuclei arranged in palisade-like rows (Fig. 2040); these are
bound together with myoglial fibrils coursing along the cells and coalescing
to form large fibres at their terminal processes (Fig. 2041). The picture
resembles a spindle-celled malignant melanoma, the difference being that
the cells of the latter are rarely distinctly spindle-shaped but terminate in,
or send off laterally, ill-defined processes anastomosing with those of neigh-
bouring cells to form a syncytium.
It is only possible to distinguish between the two by differential staining
(Mallory's phosphotungstic haematoxylin) and by the demonstration of the

DISEASES OF THE UWEAL TRACT
2455
Fig. 2041.-LEIoMYoMA of THE IRIs.
High-power view showing rod-shaped nuclei with palisade arrangement
(Frost, Am. J. O.).
2042.-LEIoMyo MA of IRIs.
FIG.
Gold-impregnation staining showing myoglial fibres (Frost, Am. J. 0
.).


2456 TEXT-BOOK OF OPHTHALMOLOGY
myoglial fibres by gold-impregnation; and since these methods are com-
paratively recent, the cases reported by the older writers must be viewed with
suspicion. Four early cases are in the literature which may be examples of
leiomyoma of the iris: Dreshfeld (1875), Thompson (1899), Helleberg (1899),
and van Duyse (1911); but the only three to be unquestionably demon-
strated are those of Verhoeff (1923), Frost (1937), and Ellett (1939) (Fig.
2042), they seemed to be associated with the sphincter muscle.
There are more reports of myomata occurring in the ciliary body. The early
cases of Iwanoff (1876), Solomon (1882), Mules (1888), Deutschmann (1890), Lange
(1890), and Lagrange (1901), and that of Guaita (1895) which extended into the choroid,
may well all have been spindle-celled melanomata ; and even the later cases of Velhagen
(1933) affecting the ciliary body, and Bossalino (1934) affecting both the ciliary body
and iris, were not completely demonstrated to be of this nature.
Bossalino. Bol. d’Oc., xiii, 332, 1934. Lagrange. Tumeurs de l'OEil, Paris, i, 1901.
Deutschmann. Beit. 2. Aug., i, 39, 1890. Lange. A. f. O., xxxvi (3), 247, 1890.
Dreshfeld. Lancet, i. 82, 1875. Mitwalsky, A. f. Aug., xxviii, 321, 1894.
v. Duyse. A. d’O., xxxi, 13, 1911. Mules. T. O. S., viii, 67, 1888.
Ellett. A. of O., xxi, 497, 1939. Solomon. T. O. S., ii, 263, 1882.
Frost. Am. J. O., xx, 347, 1937. Thompson. T. O. S., xix, 49, 1899.
Guaita. Am. di Ott., xxiv, 25, 1895. Velhagen. K. M. Aug., xei, 456, 1933.
Helleberg. Mitt, aus d. Augenkl, Karol. Verhoeff. A. of O., lii, 132, 1923; xvi. 893.
med.-chir. Instit. Stockholm, ii, 1899. 1936.
Iwanoff. G.-S. Hb., I, iv, 6, 1876.
2. ANGIoMA
Angiomata of the uveal tract are rare, and of the anterior parts of the
uveal tract exceedingly rare.
Several cases have been reported clinically as being hapmangiomata of
Fig. 2043.-HAEMANgioma of THE CILLARY Booy (Daily, Am. J. O.).
the iris ; these were localized and highly vascular tumours on the surface of
the iris which gave rise to periodic bleeding. It is probable, however, that
some of them were granulomata and others malignant melanomata; perhaps

DISEASES OF THE UVEAL TRACT 2457
Fig. 2044. HAEMANgioma of THE CILIARY Body (Daily, Am. J. O.).
three can be considered clinically as angiomata—those of Wolfe (1880),
Hönig (1912), and Bleisch (1921). Only one case has undergone the test of
microscopical examination (Rodin, 1929): it occurred as a spot in the iris of
a boy aged 4 years, and continued to bleed intermittently until the eye was
Fig. 2045.-ANGIoMA of THE CHOROID.


2458 TEXT-BOOK OF OPHTHALMOLOGY
excised because of raised tension and pain. Knapp (1928) and Tyson (1932)
described angiomatous changes in the iris in association with buphthalmos
and a naevus flammeus of the face, with in the first case a similar condition
of the gums and palate, trunk and upper extremities, and in the second a
calcified vascular growth of the occipital lobe.
Haemangiomata of the ciliary body are similarly rare, two cases being
in the literature, Griffith (1892) and Daily (1931) (Figs. 2043–44). The
latter occurred in a child of 5 months and the eye was excised for recurrent
hyphaemata with pain and raised tension. Histologically the whole ciliary
body was replaced by an angiomatous tumour, which projected with a
necrotic head into the angle of the anterior chamber from which point the
bleeding took place.
Haemangiomata of the choroid are more common, but still rare (Fig.
2045). Reports of some 25 cases are in the literature, but very few of them
have been observed ophthalmoscopically. Of recent years provisional
clinical diagnoses have been made (Reis, 1911; Salus, 1913; Henderson,
1920; Lamma, 1931; Lindenmeyer, 1932; Mulock Houwer, 1933; Ludwig,
1935; Veil, 1936; and Brons, 1936), but in the majority of cases the eye
has been excised under the diagnosis of malignant melanoma or the tumour
has been found accidentally in an eye excised for absolute glaucoma.
Although really congenital, and occurring usually in young people, the
tumour has occasionally not attracted attention until adult life (69 years in
one case); indeed, an eye has been passed as normal at 14 years of age
and shown pathological changes at 25 which assumed the proportions of a
tumour at 36 (Lindenmeyer, 1932). Clinically such tumour-formation is
usually situated near the disc (24 out of 25 cases, Mulock Houwer, 1925–33);
it has well-defined steep margins and is of slow growth, it is early associated
with a detachment of the retina, and it may have a peculiar greenish-yellow
or greyish-blue hue which may excite suspicion that it is not a malignant
melanoma. Of great importance is a sector-shaped defect in the field of
vision which does not correspond with the site of the tumour (Reis, 1911 ;
Mulock Houwer, 1925–33; Morax and Depouilly, 1930; Jaensch, 1932;
Lindenmeyer, 1932); and of still greater importance the presence of dilated
vessels in the fundus or of naevi elsewhere, especially on the face (naevus
flammeus). Finally, it is of slow evolution (Veil, 1936, observed one
clinically for 7 years) with a tendency to peri-papillary extension, and
pigmentary degenerative changes are common at the macula, presumably
owing to the proximity of the tumour to the disc. It must be recognized,
however, that the diagnosis is by no means certain, although recurrent
hyphaemata and increased tension progressing to absolute glaucoma may be
suggestive.
Pathological studies show that the neoplasm is an ordinary cavernous
angioma with large endothelial-lined spaces filled with blood and separated
* p. 2460.
DISEASES OF THE UVEAL TRACT 2459
by very delicate fibrous tissue (Figs. 2046–47) (Panas and Remy, 1879;
Milles, 1884; Lawford, 1885; Nordenson, 1885; Snell, 1886; Schiess-
Gemuseus, 1888; Giulini, 1890; Tailor, 1894; Wagenmann, 1900;
Fig. 2046.-HAEMANGIoMA of THE CHoRoid (Brons, K. M. Aug.).
Steffens, 1902; Beltman, 1904: Fehr, 1905; Meller, 1907; Stoewer,
1908; Quackenboss and Verhoeff, 1908; Reis, 1911; Bergmeister, 1911;
Love, 1914; de Haas, 1928; Jahnke, 1930; v. Hippel, 1931 : Jaensch,
1932; Brons, 1936; Evans, 1937; Evans and Evans, 1939). A peculiar
feature of several of these neoplasms is the presence on the inner side of a
band of fibrous-looking tissue which frequently contains plaques of bone
(Nordenson, 1885; Wagenmann, 1900; Steffens, 1902; Fehr, 1905; Paton
and Collins, 1919). The fibrous proliferation has usually been considered
mesodermal, but v. Hippel (1931) suggested that it is ectodermal and


2460 TEXT-BOOK OF OPHTHALMOLOGY
derived from the pigmentary epithelium, a view supported by the fact that
frequently Bruch's membrane itself seems hypertrophied and the pig-
mentary epithelium may consist of several layers of cells (Mulock Houwer,
1925; Kern, 1932; Brons, 1936).
The frequency of the occurrence of angiomata with a maevus flammeus on the same
side of the face has already been mentioned. This, as will be seen, is a common
accompaniment of buphthalmos or glaucoma, a relationship first described anatomically
by Milles (1884) and subsequently verified by a number of authors." In the same
The communication of the intra-ocular tumour along the path of a vortex
vein through the sclera to the orbital veins (Jameson Evans, Brit. J. O.).
Fig. 2048.-Choroid AL ANGIovia.
syndrome may also be included angiomatous changes in the brain, a relationship first
suggested by Sturge (1879) and Horrocks (1883) and confirmed anatomically by
Cushing and Bailey (1928) and radiologically by Jahnke (1931), O'Brien and Porter
(1933), and Evans and Evans (1939). In his case of an angioma of the choroid with
a naevus flammeus, Jahnke (1930) found a tortuous network of the diploic veins of the
skull. A cerebral angioma has been noted radiologically and has produced defects
in the visual fields in association with an angiomatous-like hypertrophy of the blood-
vessels of theiris (Tyson, 1932). In cases of intra-ocular angiomata, therefore, an X-ray
of the skull is advisable since an associated intra-cerebral lesion may exist.
Beltman. A. f. O., lix (3), 502, 1904.
Bergmeister. A. f. O., lxxix., 285, 1911.
Bleisch. K. M. Aug., lxvi. 926, 1921.
Brons. K. M. Aug., xovii, 43, 1936.
Cushing and Bailey. Tumours arising from
the Blood Vessels of the Brain, Springfield,
Ill., 1928.
Daily. Am. J. O., xiv, 653, 1931.
Evans. A. of 0., xviii, 193, 1937.
Evans and Evans. Brit. J. O., xxiii, 95, 1939.
Fehr. Cb. pr. Aug., xxix., 161, 1905.
Giulini. A. f. O., xxxvi (4), 247, 1890.
Griffith. Med. Chronicle, xvi. 1, 1892.
de Haas. Ned, tid. v. Gen., ii., 4326, 1928.
Henderson, Brit. J. O., iv, 373, 1920.
v. Hippel. A. f. O. cxxvii, 46, 1931.
Hönig. Cb, pr. Aug., xxxvi, 229, 1912.
Horrocks. T. O. S., iii, 106, 1883.
Houwer, Mulock. K. M. Aug., lxxv, 657,
1925; we, 58, 1933.
Jaensch. K. M. Aug., lxxxviii. 622, 1932.
Jahnke. Z. f. Aug., lxxii, 354, 1930; Ixxiv,
165, 1931.
Kern. Z. f. Aug., lxxix., 21, 1932.
Knapp. A. of O., lvii, 219, 1928.
Lamma. Boll. d’Oc., x, 974, 1931.
Lawford. T. O. S., v, 136, 1885.
Lindenmeyer. K. M. Aug., lxxxviii, 339,
1932.
Love. A. f. O., xliii, 607, 1914.
Ludwig. K. M. Aug., xev, 168, 1935.
Meller. Z. f. Aug., xvii, 50, 1907.
Milles. T. O. S., iv, 168, 1884.
Morax and Depouilly. An d'Oc., clvii, 537,
1930.
Nordenson. A. f. O., xxxi (4), 59, 1885.
O'Brien and Porter. A. of O., ix. 715, 1933.
Panas and Remy. Anat. Path. de l'ºil,
Paris, 1879.
Paton and Collins. T. O. S., xxxix, 157, 1919.
Quackenboss and Verhoeff. An of O., xvii,
654, 1908.
p. 33.22.

DISEASES OF THE UVEAL TRACT 2461
Reis. Z. f. Aug., xxvi, 308, 1911. Sturge. T. Clin. S., xii, 162, 1879.
Rodin. A of O., ii, 679, 1929. Tailor. An... dº Ott., xxxiii, 18, 1894.
Salus. Z. f. Awg., xxx, 317, 1913. Tyson. A. of O., viii, 365, 1932. -
Schiess-Gemuseus. A. f. O., xxxiv (3), 226, Veil. Bull. S. d’O. xlviii, Paris, 302, 1936.
1888. Wagenmann. A. f. O., li (3), 532, 1900.
Snell. Brit. Med. J., ii, 68, 1886. B. O. G. Heidel., xxxi, 287, 1903.
Steffens. K. M. Aug., xl (2), 113, 1902. Wolfe. Med. Times and Gazette, i, 504, 1880.
Stoewer. K. M. Aug., xlvi (2), 323, 1908. Zamenhof and Arkin. Kl. Ocz., vii, 164, 1929.
3. MYELOMA
Stock (1918) described a unique case of a myeloma in the choroid. It occurred
in a degenerated eye with a detached retina as a richly cellular, heavily vascularized
tumour between the choroid and the sclera, stretching from the region of the disc
almost to the lens. It was growing in a plaque of Ossification, and the tumour had
itself destroyed most of the bone.
Stock. K. M. Aug., lxi, 14, 1918.
4. LYMPHOMATA and LYMPHO-SARCOMATA in the uveal tract have already been
discussed."
5. ENDOTHELIOMA
Endotheliomata in the uveal tract are extremely rare—so rare as to make their
assessment difficult. They may occur in the anterior part of the ciliary body, where
they may be associated with the endothelium of the pectinate ligament (Butler and
Assinder, 1923; Lewitzkaja, 1925) or in the choroid (Mackay and Collins, 1910;
Giannini, 1934). The tumour is composed of large flattened polygonal cells, with
large nuclei and cytoplasmic vacuoles, sometimes pigmented and grouped in spaces
bounded by delicate strands of tissue ; but clinically it is quite undiagnosable.
Many authors have described naevi, particularly those on the anterior surface of
the iris, and malignant melanomata, particularly the so-called flat or ring sarcomata,
as endotheliomata : these, however, will be discussed under the heading of neuro-
epithelial tumours.
Butler and Assinder. Brit. J. O., vii, 549, Lewitzkaja. Russ. O. J., iv, 765, 1925.
1923. Mackay and Collins. T. O. S., xxx, 281, 1910.
Giannini. Am. di Ott., lxii, 852, 1934.
C. Neuro-ectodermal Tumours
At the present time our ideas as to the nature and classification of neuro-
ectodermal tumours are somewhat in a state of flux. Until quite recently
the chaotic state of our knowledge of neuro-fibrous neoplasms was reflected
in the confusion of the terminology employed, and in the contradictory
views expressed as to whether they were ectodermal and neural in origin or
arose from the endoneural connective tissue and were therefore mesodermal.
The latter view was generally held, particularly of neuro-sarcomata, until
the researches of Verocay (1910) showing that they were derived from
proliferation of the cells of the sheath of Schwann, and it may be taken as
practically certain that these are ectodermal (Harrison, 1924). Somewhat
1 p. 2376
2462 TEXT-BOOK OF OPHTHALMOLOGY
later there appeared the classical studies of Masson (1926–32) of Montreal,
on cutaneous naevi, whose work revolutionized all previous conceptions of
neural neoplasms and greatly extended their scope. His researches have
now been amply confirmed (Stewart and Copeland, 1931; Foot, 1932;
Laidlaw, 1932; Laidlaw and Murray, 1933; Geschickter, 1935), and are
generally accepted to-day.
It will be remembered that the cutaneous nerves end in two ways:
on the one hand, the neurolemma or sheath of Schwann becomes differen-
tiated into a capsule—the Meissner corpuscle—in the derma, and on the
other, non-medullated fibres enter the basal layers of the epithelium to
ramify round two special types of cell—those of Merkel-Ranvier and
chromatophores. Masson (1926) proved conclusively that cutaneous naevi
arose from a neoplastic proliferation of these specialized end-organs,
demonstrating every transitional stage between the normal end-organ to
the characteristic groups of naevus cells which have no resemblance in
structure to the original neural cells, but with which are closely associated
both medullated and non-medullated nerve fibres." In certain tumours
only the dermal element participates, in others only the epithelial, and in
others both. Moreover, the neural cell is capable of differentiating to pig-
ment-formation, forming the normal chromatophore of the tissue. In the
eye, it will be remembered 4 that pigment formation, as indicated by a
positive dopa reaction (Meischer, 1933), is normally limited to embryonic
life ; but in the malignant degeneration of neoplastic formation the cells
return to their embryonic activity and again become dopa-positive.
Pigment-formation is thus merely an ea pression of changed metabolic activity
and is in no way essential, a fact in accordance with the variation in pigment
content from complete absence to intense concentration which is met with
both in primary and in metastatic tumours.
From the histological point of view the similarity between neural
malignant tumours (the neurogenic sarcomata) and malignant tumours of
connective tissue origin (sarcomata) is very great and has been responsible
for much confusion. Both usually have a marked fibrillary structure and
show many spindle-shaped cells, and both show every gradation from the
most benign to the most malignant forms. But in the neurogenic tumour
the spindle-cell, although predominant, has not a monopoly, for ovoid and
other shaped cells are found with it, and as a rule it is characterized by a
larger and more vesicular type of nucleus than is characteristic of the true
sarcoma cell. According to Masson (1932), the collagen in these tumours is
also derived from the neural ectoderm, for the connective tissue endo- and
peri-neurium take no part in the evolution of the tumour proper; in fact,
it develops independently of connective tissue although fibrous proliferation
may be stimulated secondarily, sufficiently sometimes, indeed, to stifle the
neoplastic cells of Schwann.
* Vol. II., p. 1805. Vol. I, p. 373.
DISEASES OF THE UVEAL TRACT 2463
In general terms, therefore, it appears that a number of tumours which
used to be described in the literature under such names as neurofibroma,
plexiform neuroma, ganglionic neuroma, naevus, melanoma, and some types
of sarcoma, are neurogenic in origin, the fundamental cell of origin being the
cell of Schwann.
So far as the uveal tract is concerned, our knowledge is not so
unequivocal, partly because the intimate nerve-arrangements have not
been thoroughly worked out, and partly because simple tumours, in which
the neurogenic structure is most easily demonstrable, are rare and difficult
to obtain for investigation. There is, however, evidence that the choroid
is richly supplied with vasomotor and sensory nerves, derived particularly
from the ciliary nerves, which ramify to form plexuses containing ganglion
cells, especially in the outer layers (Bietti, 1897–99; Agababow, 1904–12;
Fig. 2049.-NAEvus of LoNg Post ERIoR CILLARY NERVE AND CHoRoid.
Low-power section (Theobald, A. of O.).
Salzmann, 1912; Axenfeld, 1925). It is significant, as we shall see, that
malignant tumours in the choroid arise in its external layers where these
nerves are most numerous. And it is also of great interest that both
anatomical and physiological evidence points to the close association of the
chromatophores of the uveal tract with nerve fibres and their partial control
by them.”
In the uveal tract these neurogenic tumours are represented by three
varieties—the neurofibromata of von Recklinghausen,” benign naevi" and the
malignant tumours generally known as sarcomata, and usually believed to
be of mesodermal origin, but which are more probably neuro-ectodermal in
nature. These we will classify as malignant melanomata. It may be over-
simplification, in the present state of our knowledge, to consider all repre-
sentatives of these as related ; but it is significant that the neurofibromata
are sometimes pigmented and associated with naevi (Goldstein and Wexler,
1930)," and that benign naevi may occur not uncommonly in the same eye
as malignant melanomata (Heine, 1924; v. d. Hoeve, 1924; Houwer, 1928;
Schappert-Kimmyser, 1929; Heinsius, 1931), and have been observed to
* Vol. I, p. 56, Fig. 76. * Vol. I, p. 378.
* p. 2466. * p. 2471. * p. 2471.
T.O.-WOL. III. a B.

2464 TEXT-BOOK OF OPHTHALMOLOGY
degenerate into the malignant type of neoplasm both in the iris 1 and the
choroid.” It is noteworthy also that Theobald (1937) has produced con-
clusive histological evidence that a naevus may be associated with the ciliary
Fig. 2050.-NAEvus of THE LoNg PostERIoR CILLARY NERVE.
Detail of tumour shown in Figs. 2049 and 2075 showing the proliferation of
tumour cells from the cells of the sheath of Schwann and the accompanying pigment
cells. Underneath is the ciliary nerve entering the tumour and above are the tumour
cells (Theobald, A. of O.).
nerves both in the choroid and the scleral canal (Figs. 2050–51), and that
reports are now coming to hand demonstrating the connection of malignant
melanomata with the cells of the sheath of Schwann. Cases have been
Fig. 2051.-NAºvus of the Long Posterior CILLARY NERVE.
The nerve is seen in a tumourous condition while running in the scleral canal
(Theobald, A. of O.).
recorded wherein the histological picture of a choroidal “ sarcoma 'suggested
very strongly that it represented a malignant degeneration of a neuro-
fibroma (Schubert, 1925; Nitsch, 1929), while Wätzold and Gyotoku (1928),
after a study of 27 cases, concluded that the alveolar structure and poly-
morphous cellular forms of uveal “ sarcomata º indicated an ectodermal
p. 2473. * p. 2474.


DISEASES OF THE UVEAL TRACT 2465
origin. Berger and Vaillancourt (1934) also discovered sensory corpuscles
in a similar tumour, and concluded that it was a ganglio-neuroma corre-
Fig. 2052.-MALIGNANT MELANoMA of THE CHoRoid.
N. course of long posterior ciliary nerve. At 1 and 2 the cells of Schwann are
proliferating into sheets and bundles of tumour cells (for detail see Fig. 2053) (Theobald,
A. of 0.).
sponding in every way to a neuro-naevus of the skin. They observed incon-
testibly the differentiation of the tumour cells into two types—typical
Fig. 2053.-MALIGNANT MELANoMA or THE CHoRoid.
Proliferation of Schwannian cells into bundles of tumour cells growing at right
angles to the nerve: detail of Fig. 2052 (Theobald, A. of O.).
spindle-shaped Schwann cells and pigment cells free of all Schwannian
appearance. It would appear, therefore, that pigmented cells can represent


B B 2.
2466 TEXT-BOOK OF OPHTHALMOLOGY
a peculiar differentiation of neural elements, which is assumed by them in
neoplastic conditions. Much more conclusively, Theobald (1937), in the
histological study of seven typical malignant tumours of the choroid,
demonstrated in each case a neural origin from the proliferating Schwann
cells of the sheaths of the ciliary nerves traversing this tissue (Figs. 2052
and 2053). We shall take it, therefore, in the meantime that these three
tumours are neurogenic in nature, and consider “ sarcomata” as malignant
melanomata.
Agababow. A. f. mik. Anat., lxiii, 701, 1904.
A. f. O., lxxxiii, 317, 1912.
Axenfeld. K. M. Aug., lxxv, 602, 1925.
Berger and Vaillancourt. Bull. Assoc. fr. p.
l'étude de cancer, xxiii, 275, 1934.
Bietti. A. di Ott., xxvi, 215, 1897.
A. f. O., xlix, 190, 1899.
Foot. Am. J. Path., viii, 309, 321, 1932.
Geschickter. Am. J. Cancer..., xxv, 377, 1935.
Goldstein and Wexler. A. of O., iii, 288, 1930.
Harrison. J. Comp. Neurol.., xxxvii, 123,
1924.
Heine. A. f. O., exiii, 417, 1924.
A. f. O., crxvii, 458, 1931.
Ned tº. v. Gen., lxviii, 1434,
Heinsius.
v. d. Hoeve.
1924.
Houwer. T. O. S., xlviii, 167, 1928.
Laidlaw. Am. J. Path., viii. 477, 1932.
Laidlaw and Murray. Am. J. Path., ix, 827,
1933.
Masson.
1926.
Am. J. Path... viii. 367, 1932.
Meischer. A. f. mik. Anat., xevii, 326, 1933.
Nitsch. Z. f. Aug., lxix., 117, 1929.
Salzmann. Anat, and Histol. of the Human
Eye, Chicago, 1912.
Schappert-Kimmyser.
1929.
Schubert. Z. f. Aug., lvi, 252, 1925.
Stewart and Copeland. Am. J. Cancer., xv,
1235, 1931.
Theobald. A. of O., xviii. 971, 1937.
Verocay. Beit. 2. path. Anat., xlviii, 1, 1910.
Wätzold and Gyotoku. A. f. O., exk, 209,
1928.
Am. d’Anat. Path., iii, 417, 657,
A. f. Aug., e-ci, 46,
1. NEUROFIBROMA
DIFFUSE NEUROFIBROMAtosis (PLExIFoRM NEUROMA) of the choroid is
rare, and is usually associated with evidences of von Recklinghausen's
Fig. 2054.—NEURoºibroMatosis.
Showing, involvement of the iris and ciliary body. Note the blockage of the
filtration angle and the complete atrophy of the iris on the left (F. A. Davis, 4. of 0.).
disease elsewhere. The disease can occur locally in the eye in the absence of
any similar lesions elsewhere in the body (Freeman, 1934; Meeker, 1936),

DISEASES OF THE UWEAL TRACT 24.67
but more usually pigmented nodules are found in the skin or more widespread
involvement is present, such as neurofibromatous lesions in the lids, orbit,
zygomatic and temporal regions and the optic nerve (Wheeler, 1937). As a
rule the condition occurs with buphthalmos (Sachsalber, 1897; Snell and
Collins, 1903; Collins and Batten, 1905; Sutherland and Mayou, 1907;
Weinstein, 1909; Murakami, 1913; Weiner, 1925; Knight, 1925; Davis,
1939), but again this is not an invariable relationship (Snell and Collins,
1903; Freeman, 1934; Meeker, 1936). It is probable that the buphthalmic
condition is determined by a failure in development at the angle of the
Fig. 2055.-NEUROFIBRoºſatosis. Fig. 2056.-NEUROFIBRomatosis.
Showing the filtration angle in Showing involvement of a long ciliary
the case of Fig. 2054. Note the nerve penetrating the sclera. There is
anterior synechia of the iris to the neurofibromatosis of the ciliary body.
cornea (F. A. Davis, A. of O.). Compare with Fig. 2051 (F. A. Davis,
A. of 0.).
anterior chamber, the iris being sometimes adherent to the corneal margin
and the disc excavated even when the eye is normalin size (Figs. 2055). In
non-buphthalmic cases the condition, although it is congenital, may not be
noted until adult life, when glaucoma may supervene (41 years, Meeker,
1936), or alternatively no attention may be drawn to it until death (at
56 years; Freeman, 1934).
Histologically the choroid and sometimes the ciliary body may be
enormously increased in thickness, and be occupied by dense highly nucleated
fibrous-like tissue arranged in layers which may replace all the normal
elements; the cells of which it is composed are difficult to distinguish from
fibroblasts, but they are probably proliferated cells of Schwann (Figs. 2056).
There are quantities of greatly thickened non-medullated nerve fibres
forming a dense honeycombed mesh-work, numerous foci of ganglion cells,

2468 TEXT-BOOK OF OPHTHALMOLOGY
and large areas of spindle-shaped cells containing pigment. Scattered
throughout there are oval laminated structures arranged in whorls with
Fig. 2057.-NEURoºibºo Matosis or THE Choroid.
Note the numerous ovoid bodies (F. A. Davis, A. of 0.).
laterally arranged nuclei; some of them are probably of the same nature as
Meissnerian or Pacinian corpuscles which are found normally in the skin of
the hands, feet, lips and genitals, and appear abnormally in neurogenic
Fig. 2058.-Ovoid Body of Choroid.
Resembling a sensory end organ (Meissnerian Corpuscle) (F. A. Davis, A. of 0.).
tumours (Figs. 2057–58). Knight (1925) described them as hyalinized whorls
of fibroblasts; while a similar appearance may be given by enlarged
proliferated non-myelinated nerve fibres cut transversely (Davis, 1939)
(Fig. 2059).


DISEASES OF THE UVEAL TRACT 2469
The iris is usually unaffected in neurofibromatosis, but occasionally it
has been involved. Frequently, as we have seen, it is ill-developed and
adherent to the cornea, and it may indeed be reduced to a small ring of
fibrous tissue in which the only recognizable element is the sphincter
(Wheeler, 1937). Occasionally, however, small brown spots the size of a
Fig. 2059.-Ovoid Body of Choroid.
An enlarged non-myelinated nerve.
pin's head (Sakurai, 1935), or nodules resembling those seen in the skin
(Lisch, 1937), or melanomata (Goldstein and Wexler, 1930) have been
observed (Fig. 2072).
DiscRETE NEUROFIBROMATA (PERINEURAL TIBROBLASTOMATA) are much
rarer, but these solitary and encapsulated tumours arising from nerve-
sheaths and most commonly found in association with the acoustic and
spinal nerves, have been noted arising from the ciliary nerves (Axenfeld,
1925). They are quite unassociated with v. Recklinghausen's disease.
Nitsch (1929) reported two cases; a tumour described as a neurinoma by
Kyrieleis (1928) is probably of this nature; Callender and Thigpen (1930)
found two in one eye, one in the ciliary region, where it appeared clinically as
a small yellow spot in the iris, and one posteriorly, the neoplasms being in
association respectively with the anterior and posterior ciliary nerves; and
Papoleczy (1932) reported an intra- and retro-bulbar tumour associated with
a posterior ciliary nerve. A flat neurofibroma was described by Freeman
(1934) originating from a perivascular nerve plexus in the choroid, and a
typical tumour a little way from the disc arising from a posterior ciliary nerve
was noted by Stough (1937). Characteristically these tumours are made up
of spindle cells arranged in columns or palisades; they are of extremely slow
growth and are non-malignant (Figs. 2060–61). Moorhouse (1939)

2470 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2060-NEUROFIBRoma of Choroup (Moorhouse, T. o. S.).
Fig. 2061.
NEUROPIBRoma of Choroid.
Showing interlacing bundles of fibrous tissue cells with elongated nuclei (x 140)
(Moorhouse, T. O. S.).


DISEASES OF THE UVEAL TRACT
24.71
recorded a similar tumour just behind the ciliary body in a patient who
showed some evidence of von Recklinghausen's disease.
Two NEUROMAs of the ciliary nerves composed almost exclusively of medullated
fibres were observed by Fuchs (1927) : in one case there was a tumour on one of the
ciliary nerves in the choroid.
Axenfeld. K. M. Aug., lxxv, 602, 1925.
Callender and Thigpen. Am. J. O., xiii, 121,
1930.
Collins and Batten. T. O. S., xxv, 248, 1905.
Davis. A. of O., xxii, 761, 1939.
Freeman. A. of O., xi, 641, 1934.
Fuchs. A. f. O., cxviii, 697, 1927.
Goldstein and Wexler. A. of O., iii, 288, 1930.
Murakami. K. M. Aug., li (2), 514, 1913.
Nitsch. Z. f. Aug., lxviii, 117, 1929.
Papoleczy. A. f. O., cxxviii, 326, 1932.
Sachsalber. Beit. z. Aug., xxvii, 1, 1897.
Sakurai. Acta O. S. Japan, xxxix, l 1, 1935.
Snell and Collins. T. O. S., xxiii, 157, 1903.
Stough. A. of O., xviii, 540, 1937.
Sutherland and Mayou. T. O. S., xxvii, 179,
Knight. Am. J. O., viii, 791, 1925. 1907.
Kyrieleis. A. f. O., czix, I 19, 1928. Weinstein. K. M. Aug., xlvii (2), 577, 1909.
Lisch. Z. f. Aug., xciii, 137, 1937. Wheeler. A m. J. O., xx, 368, 1937.
Meeker. A. of O., xvi, 152, 1936. Wiener. A. of O., liv, 481, 1925.
Moorhouse. T. O. S., lix, 416, 1939.
2. NAFVUS
In the first volume of this text-book we have already discussed the vexed question
of the aetiology of pigmented tumours, and we have seen that while in the past there
has been much controversy as to whether they are ectodermal, mesodermal or
endothelial in origin, it is now generally accepted that in the skin and conjunctiva
they are neuro-epithelial derived from the end-apparatus of sensory nerves. They
thus bear a relation to neurofibromatosis, in which condition, as we have just seen,
hyper-pigmentation and melanomata occur in the uvea. We have also concluded
that, although the application of this conception to uveal and meningeal melanomata
is still speculative, there seems little reason to doubt that these tumours have the same
Origin.
In the Iris. We have already seen that melanomata may occur in the
iris formed by proliferation of the ectodermal epithelium *; a second type
occurs not uncommonly, especially in blue and grey irides, wherein the
tumour is situated in the stroma, sometimes in its substance, but quite
frequently raised up upon its surface. Such naevi are congenital ; their
usual habit is to grow very slowly in the earlier years of life whereupon they
enter on a period of indefinite quiescence, sometimes ultimately to undergo
atrophy. They may occur with multiple naevi in the skin (Grimsdale, 1899),
and, as we have noted,” in association with neuro-fibromatosis (Goldstein
and Wexler, 1930). They may also be associated with other congenital
anomalies, such as a hyaline membrane on the iris and ectropion of the
pigment layer (Wood, 1928).
Such naevi may be of any size, from that of a pin-point, to occupy a large
segment of the iris, or to merge into a condition of generalized melanomatosis”
(Plate XLVI, Figs. 2065, 2066). Sometimes they may be highly vascu-
p. 2461. * p. 2446.
* Vol. II, p. 1302.
* Vol. II, p. 1805. 2 * pp. 2463, 2469.
2.472 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2062.-MELANoMA of the IRIs at SIMPLE STAGE.
(Mayou, Brit. J. O.).
Fig. 2063.-MELaNoMA or the IRIs AT MALIGNANT Stage.
The same case as Fig. 2062, 13 years later. The growth has increased in size,
blocked the filtration angle and the tension was raised (Mayou, Brit. J. O.).


PLATE XLVI
Tumours of THE ANTERIor SEGMENT.
Fig. 2064.-NAEvus V.Asculosus. Fig. 2065.-NAEvus Fig. 2066.-LARGE NAEvus of THE
of THE IRIs. IRIs.
Fig. 2067-UNPIGMENTED MALIGNANT Fig. 2068.-MALIGNANT MELANoMA of
MELANoMA (“LEUco-sarco MA ’’). THE IRIs.
º
ºf
Figs. 2069–70.-MALIGNANT MELANoMA of THE Fig. 2071.-ExTRA-ocular ExtENsios
CILIARY Body. of MALIGNANT MELANoMA of
Fig. 2069 with undilated; Fig. 2070 with dilated pupil. CILIARY Body.
To face p. 2472.





DISEASES OF THE UVEAL TRACT 2473
larized and almost angiomatous in type (naevus vasculosus) (Plate XLVI,
Fig. 2064). They have been observed to remain without appreciable
change for long periods (10 years, Chandler, 1929; 37 and 49 years, Ridley,
1921); but occasionally they show evidences of continual growth, a feature
which raises the question of their malignancy. Spread occurs not only
over the surface of the iris but peripherally into the ciliary body and into
the angle of the anterior chamber, a circumstance which is usually followed
by glaucoma (Figs. 2062–63). The occurrence of glaucoma in an eye with
a maevus should therefore always be looked upon with the greatest suspicion.
A typical case was reported by Stirling (1920) wherein a speck in the iris the
size of a pin's head remained stationary for 20 years, and then suddenly and rapidly
spread almost over the entire iris, when the eye had to be excised for glaucoma. Simi-
larly Mayou (1930) observed a stationary navus for 13 years, and then, on its sudden
increase of growth with the development of raised tension, it was found to be malignant.
In a series of 26 cases of innocent naevi, Coats (1912) found that 7 became malignant
while under observation. In the literature there are numerous reports of such mel-
anomata which had been observed for some years and then had grown, showing on
histological examination malignant characteristics, occasionally parts of the tumour
of a malignant nature lying side by side with parts still benign (Collins, 1889–93, 1926;
Coats, 1912; Wood and Pusey, 1902; Wolfrum, 1909; Raubitschek, 1914; Heine,
1924; Mayou, 1930; Knapp, 1930; and others). It will also be remembered that
a large proportion of the cases of melanosis bulbi are ultimately associated with the
development of a malignant tumour."
In the ciliary body naevi are not visible clinically, but they have
occasionally been seen on histological examination. Definite tumours
were encountered by Fuchs (1917) in 3 cases and by Alexander (1932); a
group of pigmented cells forming a mass amongst the fibres of the ciliary
muscle was described by Foster Moore (1914), and long streaks of pigmented
cells in the stroma of the ciliary body between the muscle fibres were seen
by Sattler (1929) and Schappert-Kimmyser (1929). It is to be noted that
a malignant tumour was present elsewhere in the eye in two of these cases.
Histological examinations of such naevi have revealed many different pictures
(Fuchs, 1882; Collins, 1889–1926; Verhoeff, 1911; Raubitschek, 1914; Gilbert, 1921 ;
Knapp, 1930 ; Tooke, 1938; and others) (Fig. 2072). Those on the surface have
been described as being composed of heavily pigmented endothelial-like cells,
sometimes forming structures of considerable thickness lying upon the surface of
the iris. Others are composed of rounded or spindle-shaped cells without processes,
which have been compared to clump cells ; others have had cell-processes resembling
in appearance the branched chromatophores of the stroma, ; and in others the cellular
content is mixed, but there is no cellular infiltration or evidence of active cell-division.
It is probable, however, that all of them are Schwannian in nature.”
In the Choroid. Choroidal naevi present a striking clinical picture, and
are probably more common than the relatively meagre literature suggests
(Collins, 1899; de Schweinitz and Shumway, 1905; Nettleship, 1912;
1 Vol. II, p. 1395. * p. 2461.
2474 TEXT-BOOK OF OPHTHALMOLOGY
Moore, 1914–26; Fuchs, 1917 : Usher, 1926; Johnston, 1929; Wagener and
Wellbrock, 1930). It appears as a roughly circular bluish or slate-grey
patch of homogeneous texture with somewhat feathered edges, usually about
Fig. 2072.-NAºvus or THE IRIs (Goldstein and Wexler, A. of 0.).
the size of the optic disc, but sometimes larger, and usually situated near
the posterior pole of the eye (Figs. 2073 and 2077, Plate XLVII). Overit the
retinal vessels course normally, and it gives rise to no symptoms. Such
Fig. 2073.-SIMPLE MELANoMA of THE CHoRoip AT THE Disc (Reese, A. of 0.).
formations are, of course, congenital, and have been observed unchanged for
many years; at the same time, like navielsewhere, they may assume malig-
nant characteristics. This possibility was suggested by de Schweinitz and


DISEASES OF THE UVEAL TRACT 2475
Shumway (1905), Wolfrum (1909), Nettleship (1912), Foster Moore (1914),
and Mulock Houwer (1928), and was proved by Mulock Houwer (1929) and
Wagener and Wellbrock (1930). The latter two examined histologically
Fig. 2074.—NAEvus of THE CHoRoid.
P. pigment epithelium. C. choroid. S. sclera (Johnston, Brit. J. O.).
malignant melanomata comprised of undifferentiated and actively growing
cells which arose from the centre of a typical choroidal naevus seen by them
in the first case 24, and in the second 4 years previously. It is interesting
Fig. 2075. NAºvus of LoNg Post ERIOR CILIARY NERVE AND CHoRoid.
High-power section showing involvement of the ciliary nerve, from the sheaths
of which the navus springs (Theobald, A. of 0.). Same case as in Fig. 2049.
also that naevi and malignant tumours are not uncommonly met with in the
same eye (Heine, 1924; v. d. Hoeve, 1924; Houwer, 1928; Heinsius, 1931).
Schappert-Kimmyser (1929), for example, found 5 separate naevi in 50
eyes with, and 2 in 50 eyes without malignant melanomata.


24.76 TEXT-BOOK OF OPHTHALMOLOGY
Histological studies have been recorded by several authors (de Schweinitz and
Shumway, 1905; Foster Moore, 1914–17; Magitot, 1916; Fuchs, 1917; v. Szily,
1920; Usher, 1926; Velhagen, 1928; Johnston, 1929; Schappert-Kimmyser and
Houwer, 1929) (Figs. 2074 and 2075). The growth lies in the outer layers of the choroid,
the retinal pigment being undisturbed. By the older writers the cells have been
described as spindle-shaped and lacking the processes characteristic of choroidal
chromatophores, but Schappert-Kimmyser and Houwer (1929) compare them to
atypical navus cells.
Treatment. Since the vast majority of naevi remain stationary, no
treatment is required ; but a periodical watch should be kept upon them
lest signs of growth appear. If evidence of this does occur, particularly in
the case of naevi of the iris, or if glaucoma supervenes, they should be regarded
seriously. In the case of iris tumours, excision in an iridectomy should be
performed if it is certain that the neoplasm can be removed in its entirety.
If, however, this seems impossible, it is best not to attempt it, as there is
evidence, as we have seen to occur in conjunctival navi, that incomplete
eaccision leads to rapid and uncontrollable eatension. In this connection a case
reported by Greenwood (1929) may be mentioned wherein partial excision
resulted in a rapid filling up of the globe with melanotic tissue and its
extension to the lids. If the case does not therefore seem suitable for
excision radiation may be tried. This has frequently given most satis-
factory results, for example, in Craig's (1934) case, causing complete dis-
appearance of the tumour in six months. Failing this, and in choroidal
tumours, excision of the globe is the only alternative if signs of growth give
cause for alarm.
Alexander. A. of O., vii, 521, 1932. Moore. R. L. O. H. Rep., xix, 411, 1914.
Chandler. A. of O., i, 649, 1929. Brit. J. O., i, 26, 1917.
Coats. T. O. S., xxxii, 165, 1912. T. O. S., xlvi, 120, 1926.
Collins. R. L. O. H. Rep., xii, 273, 1889. Nettleship. P. R. S. Med., Sect. O., vi, 1, 1912.
T. O. S., xiii, 128, 1893; xix, 53, 1899; Raubitschek. K. M. Aug., lii, 683, 1914.
xl, 145, 1920; xlvi, 86, 1926.
Craig. T. O. S., liv, 620, 1934.
Fuchs. A. f. Awg., xi, 435, 1882.
A. f. O., xxxi (3), 39, 1885; lxxxvi, 155,
1913; xciv, 43, 1917.
Gilbert. A. f. Aug., lxxxviii, 143, 1921.
Goldstein and Wexler. A. of O., iii, 288,
1930.
Greenwood. A. of O., i, 650, 1929.
Grimsdale. T. O. S., xix, 61, 1899.
Heine. A. f. O., cziii, 417, 1924.
Heinsius. A. f. O., czzvii, 458, 1931.
v. d. Hoeve. Ned. tij. v. Gen., lxviii, 1434,
1924.
Houwer, Mulock. T. O. S., xlviii, 167, 1928.
Ned, tij. v. Gen., i, 1649, 1929.
Johnston. Brit. J. O., xiii, 498, 1929.
Rnapp. A. of O., iv, 720, 1930.
Littwin. A. of O., iv, 154, 1930.
Magitot. An. d’Oc., cliii, 93, 1916.
Mayou. Brit. J. O., xiv, 152, 1930.
Ridley. T. O. S., xli., 451, 1921.
Sattler. Die Bösartigen Geschwälste d. Augen,
Leipzig, 1929.
Schappert-Kimmyser.
1929.
Schappert-Kim myser and Mulock Houwer.
A. f. Aug., c-ci, 21, 1929.
de Schweinitz and Shumway. T. Am. O. S.,
x, 439, 1905.
Stirling. T. O. S., xl, 143, 1920.
v. Szily. B. O. G. Heidel., xlii, 313, 1920.
Tooke. Brit. J. O., xxii, 153, 1938.
Usher. T. O. S., xlvi, 131, 1926.
Velhagen. K. M. Aug., lxxx, 73; lxxxi, 392,
1928.
Verhoeff. A. of O., xl, 486, 1911.
Wagener and Wellbrock. A. of O., iv, 509,
1930.
Wolfrum. A. f. O., lxxxi, 195, 1909.
Wood. Brit. J. O., xii, 140, 1928.
Wood and Pusey. A. of O., xxxi, 323, 1902.
A. f. Awg., c-ci, 46,
DISEASES OF THE UVEAL TRACT 2477
3. MALIGNANT MELANOMA
The natural history of malignant melanomata has been known for well over a
century, the first clear disquisition being that of Laennec (1819) who called pigmented
growths melanoses. Their malignant nature was stressed by Lawrence (1845),
while Sichel (1851) distinguished between the benign and malignant varieties. The
entire subject was first put on a sound scientific basis by Virchow (1863), who included
in his studies a series of ocular tumours derived from v. Graefe’s clinic ; he first laid
stress on the fact, generally admitted to-day, that the real criterion of the nature of the
tumour lay in its intimate histological structure, showing that the presence of pigment
was relatively unimportant, and gave these growths their first systemic classification
into benign melanomata, melano-sarcomata, and melano-carcinomata. Almost
immediately thereafter there appeared the classical papers of v. Graefe (1868) and
Knapp (1868) confirming these histological data and giving so clear and exact an account
of the clinical course of these neoplasms that it can only be modified to-day in un-
essential details. There followed, among others, two important series of papers by
Hirschberg (1868–80) and Nettleship (1872–76), each a series of valuable contributions;
and then the classical and detailed monograph of Fuchs (1882) appeared in which he
collated and detailed all the information on the subject and discussed 259 cases,
including 22 of his own. Other valuable pathological collections towards the end of
the century were compiled by Lawford and Collins (1891) of 103 cases from London,
Panas and Rochon-Duvigneaud (1898) from France, Parwel (1899) of 100 cases from
Balle, and Kerschbaumer (1900) of 67 cases from Leipzig. The classical treatises of
Lagrange (1901) and Parsons (1905) collected and criticized all this wealth of material,
and there our knowledge has essentially remained until comparatively recent times.
At this time great ingenuity was displayed as to systems of classification. The
old distinction of melanotic and leuco-sarcomata, while retained as a useful
clinical description, was generally dropped on Virchow's authority. Fuchs (1882)
described 14 types—spindle- and round-celled sarcoma, sarcoma, with areolar pig-
mentation, endothelial sarcoma, cavernous sarcoma, fibro-sarcoma, alveolar sarcoma,
giant-celled sarcoma, myxosarcoma, chondrosarcoma, Osteosarcoma, myosarcoma,
and so on. Kerschbaumer (1900) used the classification—angiosarcoma, melanotic
sarcoma, leucosarcoma, spindle-celled sarcoma, combined tumours, and degenerating
sarcoma. Lagrange (1901) divided those tumours into two groups—melanotic and
leuco-sarcomata, each with three divisions depending on (1) the form of their cells
(round, spindle-shaped, or giant), (2) endothelial origin and grouping of the cells
(haemangiosarcoma, lymphangiosarcoma, alveolar, and tubular), and (3) the state of
the blood-vessels (telangiectatic and cavernous). Coppez (1901) suggested three
groups : endotheliomata, peritheliomata, and Sarcomata, growing from the proper
cells of the choroid. The various systems of classification proposed since then are no
more definitive, and the only 1ogical attitude was that adopted by Parsons (1905) who
refused to introduce classification into a subject so complex morphologically regarding
the aetiology of which absolute ignorance prevailed.
There followed a controversy as to the histogenesis of these tumours, and of
the available cells from which they might be derived the chromatophores and the
endothelium came most under discussion. The discussion has never been settled,
since the very commencement of a tumour is an impossible histological finding, and the
normal structures are very quickly disorganized once the tumour attains any size.
v. Recklinghausen (1882) put forward the view that the essential neoplastic cells were
endothelial ; Ribbert (1898–1911) and Acton (1922) concluded that they were meso-
blastic chromatophores and that the cells which resembled endothelium and were
arranged in quasi-alveoli, were round or polygonal and non-pigmented merely because
2478 TEXT-BOOK OF OPHTHALMOLOGY
they were immature : Ribbert therefore proposed the term chromatophoroma. A
third view suggested a dual origin—partly from endothelium and partly from meso-
blastic chromatophores (Fuchs, 1882; Coppez, 1901; Collins, 1926). A final com-
plication was the theory that the essential pigmented cells were ectodermal in origin
and had migrated inwards from the retinal layers (Unna, 1893; Krompecher, 1908–23;
Wolfrum, 1909; Bloch, 1917–21 ; Kornfeld, 1920–21 ; Dawson, 1925). Thus
matters stood in considerable confusion, the majority of opinion considering that
these neoplasms were mesodermal Sarcomata, largely because of their clinical behaviour,
since they did not involve lymphatic channels or glands in their metastasis but spread
by means of blood-vessels. At this point the work of Masson (1926–31), which was
verified by Foot (1932), Laidlaw (1932), Laidlaw and Murray (1933) and others,
appeared on the origin of melanomata of the skin," ascribing to these neoplasms a
neurogenic origin ; and, as we have seen, especially in view of the researches of
Theobald (1937), there is every reason to believe that the malignant tumours of the
uveal tract, whether pigmented or not, are essentially neoplastic developments from
the cells of Schwann and are therefore neuro-ectodermal in origin (Figs. 2052–53).
Incidence
The literature now contains so many studies of large numbers of cases of malignant
melanomata that a very fair assessment can be made of their incidence. The first
collation of note was that of Fuchs (1882) of 259 cases. In London the cases seen at
Moorfields Hospital from 1871–90, 103 in number, were studied by Lawford and
Collins (1891), from 1891–98, 58 in number, by Marshall (1899), while Davenport
(1927) studied a further 35 cases and collated the total 345 cases dealt with at this
hospital from 1871 to 1925. Pawel (1899) published 100 cases from Halle, Kersch-
baumer (1900) 67 cases from Leipzig, and Wintersteiner (1902) collected 881 cases
from the general literature. More recent studies include those of Arganaraz (1916)
on 30 cases from Buenos Aires, Jaensch (1930) on 68 cases at Breslau, v. Hippel (1933–
36) on 118 cases at Göttingen, Terry and Johns (1935) on 94 cases at Boston, Denecke
(1936) on 36 cases from Greifswald, and Kronenberg (1938) on 188 cases from New
York.
The general incidence shows it to be a rare disease. On the average
it occurs in from 0.02 to 0.06% of all eye-patients, that is, about 2 to 6 per
10,000 patients. It would appear from records kept for long periods (1871–
1925 at Moorfields) that this proportion is relatively constant, neither
showing an increase nor a decrease over a period of half a century.
There is no special sea incidence. Since the findings of Fuchs (1882)
(137 males; 116 females) and Lawford and Collins (1891) (59 males;
44 females) it has been usually stated to be more frequent among males.
Subsequent statistics have not borne this out. Thus the complete Moorfields
statistics (1871–1925) give 167 males and 175 females.
The average age incidence is about 50 years, some 45% occurring in the
5th and 6th decades of life. Cases in the 2nd decade are very rare, and in
the first exceptional—Fuchs (1882) recorded 11 of them. The youngest
recorded case is that of Iwumi (1910) in which the eye was excised at the
age of 3 when the tumour had already perforated the sclera, and in which
symptoms had been noted at 9 months. Cases above 80 years are likewise
* p. 2461.
DISEASES OF THE UVEAL TRACT 2479
exceptionally rare. The average age of tumours of the iris is lower than
of the ciliary body and choroid—40 instead of 50 years.
The eye affected is sometimes said to be preferentially the left, but there
is no statistical evidence for this statement. Bilateral cases are very
exceptional. The first reported case was by Carter (1874). Fuchs (1882)
found records of 5, Wintersteiner (1902) of 4, and in reporting a case wherein
the iris of one eye and the choroid of the other were involved, Shine (1930)
could only collect 7 instances in the literature.
With regard to the part of the eye affected the vast majority occur in the
choroid. Subsequent statistics generally approximate those originally found
O Known cases of malignant melanoma.
@ Suggestive history.
The numerals give the ages of the individuals when seen.
FIG. 2076.-HEREDITY OF MALIGNANT MELANOMA OF THE CHOROID
(Davenport, Brit. J. O.).
by Fuchs (1882): choroid 85%, ciliary body 9%, iris 6%. Among the
choroidal tumours the greater proportion are situated posteriorly, especially
on the temporal side (51%, Kronenberg, 1938); thereafter the anterior and
equatorial segments are about equally represented (19.8% and 16.2%),
while the remainder are generalized. In the iris in the majority of cases the
lower half is affected ; the upper half comes next, then the inner and last the
outer segments—Parsons’ (1905) figures for these locations are 35 lower,
13 upper, 5 inner and 2 outer.
The co-existence of malignant disease elsewhere—usually a carcinoma—
is very rare and merely a fortuitous coincidence. Cases have been reported
by Fisher and Box (1900), Parsons (1905), Valentine (1917), Grimsdale
(1930) and Wagenmann (1934).
Hereditary influences are little in evidence. Cases can always be found
wherein near relatives died of “ tumours,” of which coincidences, for
T.O. —WOL. III. C C

24.80 TEXT-BOOK OF OPHTHALMOLOGY
example, Lawford and Collins (1891) collected 14 instances. A definite
familial tendency, however, is occasionally observed, as that involving
7 individuals in four generations of a family reported upon by Nettleship
(1872–79), Lawford and Collins (1891), Marshall (1899), Parsons (1905), and
Davenport (1927), and illustrated in Fig. 2076.
The question of injury in the aetiology is a vexed one. There are, of
course, many clinical records of a neoplasm being preceded by an injury
until the incidence of which the eye was stated to be perfectly healthy.
Fuchs (1882), indeed, found such a history in 11% of his cases, Leber and
Krahnstover collected 34 cases, and in a study of 507 cases in the literature
Lane (1934) found an injury mentioned as a possible incriminating agent
66 times. Although from a medico-legal standpoint claims for compensa-
tion from this point of view are increasing and awards are becoming more
frequent, in all the literature there is no scientific evidence, either experi-
mental or pathological, that the relationship between the two events is more
than incidental. The utmost concession which can justifiably be made is
that traumatism may be responsible for exciting into obvious activity a
tumour which hitherto had been pursuing a slow latent course (Chance,
1934).
There is, indeed, more evidence that prolonged inflammation may be a
determining factor in some cases, owing to the occurrence of malignant
melanomata in shrunken phthisical eyes. In the majority of cases the
phthisis bubi probably supervened upon the tumour and was dependent
upon the toxic effect of necrotic changes in it ; but in others it would appear
that the inflammation preceded the development of the neoplasm. Thus
Leber and Krahnstüver (1898) collected 34 cases of the first category and
22 of the second. Such a preceding inflammation is usually plastic and
rarely purulent, and the subsequent degeneration may have progressed to
the formation of bone (Fig. 2129) (Margotta, 1931 : Chance, 1934). It will
be realized, as will be seen later, that the clinical picture thus presented
complicates considerably the diagnosis of a neoplasm.
Clinical Course
Following the suggestion of Knapp (1868), it is classical to describe the
clinical evolution of malignant melanomata in four stages: (1) a symptomless
stage, (2) a stage of glaucoma, (3) a stage of extra-ocular extension, and
(4) the stage of metastases. While a large number of tumours may conform
to this ordered course, such behaviour is by no means invariable. A rise of
tension may never occur ; extra-ocular extension may occur before signs of
secondary glaucoma are evident ; and metastases may be widespread at
any stage, sometimes in the first stage and very frequently indeed before the
third. It is questionable if such a scheme is of much value, and it is mis-
leading in so far as it suggests a false security in that development occurs
in a sequential order.
PLATE XLVII
Tumours or THE Posterior SEGMENT.
Fig. 2077.-NAEvus of THE CHoRoid. Fig. 2078.-EARLY MALIGNANT MELANoMA
of THE Choroid.
Fig. 2079–MALIGNANT MELANoMA or THE CHoRoid
(showing much pigmentation) (Doherty).
Fig. 2080–MALIGNANT MELANoMA of THE Fig. 2081–MALIGNANT MELANoMA OF
CHoRoid (showing no pigmentation). THE Choroid (showing retinal detach-
ment).
[To face p. 2480.



DISEASES OF THE UVEAL TRACT 2481
The very early stages of a neoplasm are difficult to come upon, but
early growths have been found accidentally in eyes enucleated for other
reasons (Hensius, 1931), and also when the tumour is located directly at the
macula (Fuchs, 1900) (Figs. 2082, 2078, Plate XLVII). The tumour com-
mences in the outer layers of the choroid, in Haller's or Sattler's layer of
vessels, and sometimes in the supra-choroidea (Fuchs, 1882; Derby, 1903;
Schultz-Zehden, 1905), in the corresponding part of the ciliary body, or in the
-
º
----------
--- º -
º º --
Fig. 2082–MALIGNANT MELANoMA of Choroid . VERY EARLY STAGE ( x 120)
(Parsons).
anterior layers of the iris. It is interesting from the point of view of their
neurogenic origin that it is in the outer layers that the nerve plexuses are
most common." As growth proceeds it may assume one of two character-
istics, usually forming a discrete circumscribed tumour, or alternatively, and
much more rarely, infiltrating the tissues in a diffuse manner.
(i). CIRCUMSCRIBED TUMoURs
In the Choroid
The typical circumscribed malignant melanoma appears first as an
elliptical or lenticular-shaped neoplasm in the outer layers of the choroid
(Figs. 2083, 2078, Plate XLVII), a shape which is probably imposed upon
it by the resistance of the sclera and the membrane of Bruch which inhibits
growth in thickness and allows lateral spread more readily. Soon, however,
Bruch's membrane is burst through, whereupon, since resistance is greatly
relieved, vigorous growth in all directions proceeds equally in the sub-
retinal space, so that a characteristic globular head is formed connected
1 p. 2463.
C. C. 2






2482 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2083.-MALign ANT MELANoMA or Choroid : EARLY STAGE.
The tumour (1 × 6 mm.) was found accidentally on microscopic examination.
Note exudative detachment of the retina above it (Rönne, T. O. S.).
Fig. 2084.-MALign ANT MELANoMA of Choroid.
In an early stage showing typical mushroom shape. The broken ends of Bruch's
membrane are curled up at the end of the neck (x 9) (Parsons).


DISEASES OF THE UVEAL TRACT 2483
to the main mass by a narrow neck at the level of Bruch's membrane, giving
the whole the appearance of a mushroom (Fig. 2084). A globular or lenti-
cular shape therefore indicates that the tumour is still confined to the
choroid, while a mushroom shape is proof of its having perforated Bruch's
membrane. At this stage the retina is raised up and detached; it
usually rests upon the knob-like head around which is a wide exudative
detachment, the sub-retinal space being filled by a richly albuminous
fluid formed partly as the result of venous obstruction and partly by
irritative reaction (Figs. 2079–81, Plate XLVII). Quite frequently
this fluid gravitates to the most dependent part of the globe, and there
Fig. 2085.-MALIGNANT MELANoMA of THE CHoRoid : LATE STAGE.
The tumour fills the globe and extends into the orbit (x 24) (Parsons).
it may form a retinal detachment quite apart from the tumour ; such a
remote detachment may be large even when the tumour itself is yet small
(Fig. 2092). Occasionally a very shallow detachment occurs in the early
stages far out in the peripheral retina, the rationale of which is not under-
stood (E. Fuchs, 1917; A. Fuchs, 1935). Meantime the tumour increases
in size gradually filling up the cavity of the globe and eventually occupying
all the available space (Fig. 20.85); but as a rule, before this has occurred,
the clinical picture is dominated by the incidence of glaucoma or an
irido-cyclitis.
The retina itself is not invaded until a very late stage, when it becomes
completely disorganized ; but after Bruch's membrane is perforated the
pigment epithelium is always involved. The area overlying the tumour

2484 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2086–MALignant MELANoMA of THE Choroid.
The neoplasm broke through the retina and proliferated freely in the vitreous
obscuring the optic disc. It pushed the nerve-fibres aside without injury so that a
corresponding defect was produced in the visual field. The tumour is cavernous in
structure (Rönne, T. O. S.).
Fig. 2087,-MALignant MELAsoma or Choroid.
Atypical form (x 24) (Parsons).


DISEASES OF THE UVEAL TRACT 2485
Fig. 2088. Juxta-PAPILLARY MALIGNANT MELANoMA
(Rönne, T. O. S.).
becomes adherent to it, the pigment cells at one part degenerating and at
another proliferating, the cells in the latter event frequently growing into
the neoplasm itself. At the same time the part of the retina which overlies
the tumour usually undergoes a marked degree of necrosis. A very excep-
Fig. 2089.-Juxta-PAPILLARY MALIGNANT MELANoMA
The melanoma of the choroid simulates clinically a tumour of the disc. a. Ter-
mination of lamina vitrea. b. Part of the tumour appearing to grow from the disc.
c. Retina. d. Tumour in choroid extending round the disc to the opposite side.
e. Optic nerve (Reese, A. of O.).


2486 TEXT-BOOK OF OPHTHALMOLOGY
tional occurrence is for a tumour to burst right through the retina without
detaching it and project and proliferate nakedly into the vitreous (Fig. 2086).
This typical mode of evolution may be modified if other channels of diminished
resistance are opened out for the growth of the neoplasm. If access to a neural or
vascular channel through the sclera is obtained early, or perhaps if the neoplasm
starts in association with the sheath of a ciliary nerve while yet in the sclera, this
tissue may be split up and infiltrated, so that a large flat growth is formed, burrowing
along the sclera and involving the choroid over a wide area without invading the
cavity of the globe or even causing a retinal detachment (Fig. 2087) (Lawson and
Parsons, 1905 ; Goulden and Stallard, 1932).
Juarta-papillary tumours may also present an anomalous appearance. Beginning
in the choroid near the disc, the growth takes the path of least resistance around the
termination of Bruch's membrane and through the intermediary tissue of Kuhnt so
that it lobulates over the nerve head, appearing clinically to arise from this structure
as a mushroom-shaped tumour (Figs. 2088–89) (Speciale-Cirincione, 1925; Charamis,
1930; Reese, 1933; Rönne, 1936; Prevec, 1937; and others).
In the Ciliary Body
In the ciliary body malignant melanomata are relatively rare: Fuchs
(1882) collected 22 cases, Mules (1888) 27, and Groenouw (1899) 50 cases
from the literature; Lawford and Collins (1891) found 6 cases in their series
Fig. 2090.-MALIGNANT MELaNoMA or CILLARY Body.
(The location of electrodes at the upper part of the globe is incidental) (Coppez,
T. O. S.).
of 103 cases of uveal malignant melanomata, Kerschbaumer (1900) 8 in 67,
Denecke (1936).4 in 36, and Kronenberg (1938) 13 in 188 cases. The progress
of growth is the same as that characteristic of a choroidal tumour; it

DISEASES OF THE UVEAL TRACT 2487
Fig. 2091.-MALIGNANT MELANoMA of CILLARY BoDY.
Showing invasion of the anterior chamber. The iris is embedded in the growth,
at the anterior part of which is seen the pupillary edge with the sphincter. The
lens has been pushed aside (x 10) (Parsons).
Fig. 2092.-MALIGNANT MELANoMA or CILIARY BoDY.
Horizontal section showing neoplasm (A) on outer side with a large degenerated
area; it is invading the anterior chamber and is extending along the anterior perforating
vessels to form several episcleral nodules. At B there is a remote retinal detachment.
From a woman aged 50 (x 3) (Parsons).


2488 TEXT-BOOK OF OPHTHALMOLOGY
commences as a lenticular tumour (Fig. 2090) and then, if in the posterior
part of the ciliary body, forms a rounded projection into the vitreous and
infiltrates backwards into the choroid and forwards into the posterior chamber.
If situated anteriorly it invades the posterior chamber at an early stage,
pushing theiris forwards, and appearing in the angle of the anterior chamber
to produce an effect resembling irido-dialysis (Fig. 2069, Plate XLVI). It
then progresses to fill the anterior chamber, the iris being invaded by con-
tinuity and perhaps being embedded in the growth (Fig. 2091). The lens
is pushed aside and often grossly distorted and deformed, rapidly becoming
cataractous; it is only rarely that its capsule is ruptured and its substance
invaded by tumour cells (Lawford, 1887). Detachment of the retina only
occurs late; but extra-ocular extension along the perforating scleral canals
is usually early and obvious (Groenouw, 1899) (Fig. 2071, Plate XLVI,
Fig. 2092).
In the Iris
In the iris malignant melanomata are still more rare. The first micro-
scopically investigated case was by Waren Tay (1866), and the recorded
cases in the literature (some 150 in number) have been assessed by Knapp
(1879), Fuchs (1882) (16 cases), Ewetzky (1896), Werther (1896), Wood and
Fig. 2093.-MALIGNANT MELANoMA of IRIs.
A spindle-celled neoplasm showing little pigment (x 60) (Parsons).
Pusey (1902) (83 cases), Laven (1913) (130 cases), and Zentmayer (1931)
(reviewing 65 recent cases); more recent informative cases include those of
Meyer and Kubik (1933), Patterson (1935), Rosenbaum (1938). A large
number have been observed to arise from naevi which have suddenly shown
signs of active growth: Coats (1902), for example, gathered 26 cases of

DISEASES OF THE UVEAL TRACT 2489
innocent naevi, of which 7 became malignant while under observation, and
it would seem probable that this is the usual origin (Hirschberg, 1883,
Fig. 2094.-MALIGNANT MELANoMA of IRIs.
Showing extensive involvement of ciliary body, retina and suspensory ligament
with deformation of the lens (Mayou, Brit. J. O.).
º
Fig. 2095.-MALIGNANT MELANoMA of IRIs.
Showing method of spread. The growth is beginning to involve the canal of
Schlemm and the ciliary region (Mayou, Brit. J. O.).
3 times in 16 cases; Wood and Pusey, 1902, 10 times in 90 cases; Kayser,
1903; Mayou, 1930 (Figs. 2062–63). The growth is frequently nodular and
practically always vascular, and is usually densely pigmented and uniformly
1 p. 2473.


2490 TEXT-BOOK OF OPHTHALMOLOGY
black (Fig. 2068, Plate XLVI). Non-pigmented tumours (“leuco-
sarcomata ??) are rare ; since the original reports of Le Brun (1869) and
St. John Roosa (1869), Duke-Elder and Stallard (1930) in describing a case
(Fig. 2067, Plate XLVI) were able to gather and analyse 26 reports in the
literature, while a subsequent case of interest is that of Zentmayer (1931).
Such tumours are yellowish-white in colour, richly and prominently
vascularized, showing on the surface numerous vascular loops, and they
usually stand out from the tissue of the iris as prominent nodules.
The tumour usually grows more slowly than a choroidal growth, but
eventually fills up the substance of the iris (Fig. 2093), spreads laterally over
its surface, bulges out into the anterior chamber, eventually filling it
(Fig. 2094), quite frequently taking on a sudden accession of rapid growth
(Fleischer, 1908; Franke, 1909). Usually at an early stage it invades the
ciliary body, the pectinate ligament, the walls of Schlemm's canal, and
spreads along the posterior surface of the cornea (Fig. 2095). In this, as in
ciliary tumours, extra-ocular spread is early, and obvious, while the rapid
involvement of the drainage channels at the filtration angle may produce
a secondary glaucoma unexpectedly soon.
(ii) DIFFUSE OR INFILTRATING MELANOMATA
This type of neoplasm is characterized by its tendency to infiltrate the
tissues of the uvea diffusely without the formation of a definite tumour. The
differentiation between the circumscribed and the diffusely infiltrating types
of growth was first made by Fuchs (1882); Mitvalsky (1894) introduced the
term Flächensarcom ; in French literature the term sarcome en mappe ou
en plaque (Lagrange, 1901) is employed ; while Parsons (1904) employed
the term commonly used in English literature of FLAT SARCOMA for choroidal
tumours. Ewetzky (1898), in describing the first case of the kind, suggested
the term RING SARCOMA to describé those affecting the ciliary body and iris.
Parsons (1904) explained the diffuse nature of these growths by the theory
that they were endotheliomata originating in the lining cells of the lymph
spaces through which they proliferated, a view suggested by the presence of
their large round or polygonal cells distributed in an alveolar or plexiform
arrangement. In most cases, however, spindle cells predominate and there
is little cytological difference between them and other tumours (Greeves and
Holmes, 1914; Neame, 1922; Mayou, 1930; and others). Greeves and
Holmes (1914) drew attention to the fact that in comparison with the
circumscribed tumours, these neoplasms are relatively avascular, and there-
fore, being dependent on pre-formed vessels rather than on new-formed
vessels for nourishment, they tend to spread along vascular and lymphatic
sheaths, a habit evident both in the primary tumour and its metastases.
Such tumours are not common : Lawford and Collins (1891) found 1 case in their
series of 103 cases; Parsons (1904) collected 31 in the literature, 18 being of the flat
choroidal type and 13 being of the ring type ; Greeves and Holmes (1914), in describing
DISEASES OF THE UVEAL TRACT 2491
a flat choroidal growth, added a further 11 cases, 9 of which were choroidal and 2 of
which infiltrated the ciliary body and iris. Subsequent cases of the latter type were
described by Laven (1913), Pindikowski (1915), Li (1923), Déjean (1924), Bruner
(1924), Szymanski (1928), Mayou (1930), Adams (1930), and others. A minute study
of the flat type of choroidal growth was made by Neame (1922) and Merrill (1933).
The essential features in the evolution of this type of neoplasm distin-
guishing it from the circumscribed variety are its slow growth with long
Fig. 2096,-DIFFUSE MALIGNANT MELANoMA of CHOROID.
Showing thickening of the choroid and extra-bulbar nodules (X 1-5) (Parsons).
Fig. 2097.-FLAT MALIGNANT MELANoMA of CHOROID.
A. Retina. B. Choroid infiltrated with growth. C. Sclera (Neame, Brit. J. O.).
preservation of vision and lack of objective signs, its diffuse infiltration of the
uveal tract and the sclera resulting in early and multiple extra-ocular
extensions, and the extent of its metastatic spread. The most conspicuous
feature is the length of the history, from 7 months to 10 years.


2492 TEXT-BOOK OF OPHTHALMOLOGY
In the choroid the neoplasm slowly infiltrates the entire tissue (Figs. 2096
and 2097), especially the outermost layers. Bruch's membrane is usually
intact, and the retina is not attacked early, nor, owing to the flat nature of
the growth, is it detached until the disease is advanced. Ophthalmological
recognition is, therefore, extremely difficult, and visual deterioration may
go on for one (Mackay, 1910) or several years (Mitwalsky, 1894) before any
objective disturbance can be seen. Even then the appearances are
anomalous, and nothing abnormal may be visible except irregular choroidal
pigment (Kipp, 1905), or a patch resembling chorio-retinitis (Cargill and
Fig. 2098. DIFFusº MALignant MELANoMA or Chobolu.
Section through the centre of the growth. A. Bruch's membrane and the under-
lying chorio-capillaris. B. Large choroidal vessels separated by tumour cells. C. Sclera.
D. The growth traversing the sclera along the course of a ciliary nerve from which it
has probably arisen (Greeves and Holmes, T. O. S.).
Mayou, 1907) or choroidal sclerosis (Merrill, 1933), or, after 5 years, a vague
oedema suggesting neuro-retinitis (Wescott, 1912); indeed, death from
multiple metastases may occur without anything whatever being seen
with the ophthalmoscope (Greeves and Holmes, 1914), and the first objective
evidence in the eye may be its extra-ocular spread, at which stage and for
some considerable time thereafter, the vision may still be good (Neame,
1922). Meantime, however, penetration of the sclera is taking place, partly
along the channels of the vessels and nerves, and partly by direct infiltration
of the tissue between the bundles of fibres (Fig. 2098), so that extra-scleral
nodules are formed. Direct invasion of the optic nerve is not common; but
eventually infiltration of the angle of the anterior chamber leads to glaucoma.

DISEASES OF THE UVEAL TRACT 2493
Fig. 2099,-RING-shaped MALIGNANT MELANoMA of IRIs.
Note the appearance of crypts of the iris, the floors of which are black; synechiae
in the pupillary area; and the neoplastic swellings at the periphery (Mayou, Brit. J. O.).
-
-
Fig. 2100.-RING-shaped MALIGNANT MELANoMA of IRIs.
To show involvement of the iris and the angle of the anterior chamber (see
Figs. 2101 and 2102 (x 42) (Adams, Brit. J. O.).


2494 TEXT-BOOK OF OPHTHALMOLOGY
In the ciliary body and iris the ring-shaped tumours have a similar long
history; for example, that reported by Kopetzky (1901) had lasted 12 years.
The growth may start either in the ciliary body or in the iris and infiltrates
Fig. 2101,–Ring-shaped MALIGNANT MELANoMA of IRIs.
Detail of the tumour of Fig. 2100. The growth extends on both sides of Descemet's
membrane (x 330) (Adams, Brit. J. O.).
Fig. 2102.-Ring-shaped MALignant MELANoMA.
Detail of tumour of Fig. 2100 to show the distribution and cellular structure of
the growth in the ciliary region. The anterior chamber is on the left( x 250) (Adams,
Brit. J. O.),
circumferentially, tending at the same time to invade the anterior part of
the choroid. In the iris the clinical appearances may be deceptive.
Occasionally at one point, presumably the starting point of the tumour, a lump


DISEASES OF THE UWEAL TRACT 2495
is seen (Fig. 2099), and the entire tissue may be more heavily pigmented,
areas of pigment appearing all over the stroma. In the meantime it
infiltrates the ciliary body, and, extending over the angle of the anterior
chamber, tends to spread over the inner surface of the cornea, sometimes on
either side of Descemet's membrane (Figs. 2101). While this infiltration is
going on the clinical picture may resemble that of an irido-cyclitis degenerating
into a secondary glaucoma. Similarly in the ciliary body the neoplasm may
occupy most of the tissues, flattening out the processes but usually leaving
the epithelium intact (Fig. 2102), invading the iris profusely, usually its
anterior layers at first, but eventually the entire tissue (Fig. 2103), and
Fig. 2103.−RING-shAPED MALIGNANT MELANoMA of IRIs AND CILIARY BoDY.
To show the involvement of the region of the canal of Schlemm, and of the ciliary
processes, as well as a flat involvement of the iris (x. 20) (Werner, T. O. S.).
infiltrating the angle of the anterior chamber, before symptoms of irido-
cyclitis and secondary glaucoma develop. In a case of my own which had
been treated for a long time for glaucoma secondary to irido-cyclitis, it was
only the presence of pigmented keratic precipitates and a questionable area of
non-translucency at one part of the ciliary circle that led to enucleation
of the eye being performed whereupon a complete ring infiltration of the
entire ciliary region was found.
The Spread of Uveal Melanomata
These tumours spread by three methods—direct extra-ocular extension
through the sclera, local metastases in the eye, and general metastases
throughout the body.
ExTRA-ocular EXTENSION may take place at a very early stage in the
evolution of the tumour (when the tumour was 2 × 3 mm. Schultz-Zehden,
T.O.-WOL. III. D. D.

2496 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2104.—FLAT MALIGNANT MELANoMA: To show THE Route of Extra-
BULBAR. ExtENsion.
A. The cornea. B. Epibulbar growth over lower part of cornea. C. Track of
growth connecting the epibulbar and intra-ocular portions of the neoplasm.
D. Extension of growth on posterior surface of cornea. E. Root of iris densely
infiltrated with growth. F. Ciliary body densely infiltrated with growth (× 3.0)
(Neame, Brit. J. O.).
Fig. 2105-MALignant MELANoMA or Choroid :
NERVE.
Ns. Melanoma showing necrotic changes. Pte. Pigmented tumour cells infiltrating
the optic nerve (Samuels, T. O. S.).
to show Extension into THE OPTIC


DISEASES OF THE UWEAL TRACT 2497
1905). As a rule it does not occur directly through the sclera but takes the
path of least resistance along the channels made by the perforating vessels
and nerves. The neighbouring scleral fibres are rapidly infiltrated, and once
the neoplastic tissue reaches the outside, owing to the absence of tension, it
usually grows apace. If extension occurs through the ciliary region, the
extra-ocular growth is readily seen and recognized ; it usually appears
externally on the sclera somewhat posterior to the limbus at the site of the
exits of the emissaries (Fig. 2104), and therefore some considerable way
-
Fig. 2106.-FUNGATING MALIGNANT MELANoMA (Maitland Ramsay).
behind the site of election of a tuberculous perforation which occurs preferen-
tially at the level of Schlemm's canal and the limbus itself (Fig. 1928).
There is usually direct continuity of the cells along the scleral canals, but
occasionally the most exhaustive search fails to reveal this, raising the
question whether the cells may not sometimes be carried by the lymph
stream (Groenouw, 1899). It has happened that these extra-ocular exten-
sions have been noted before the parent intra-ocular tumour (Neame, 1922),
and they may attain considerable dimensions, ultimately forming ulcerating
and fungating growths upon the globe (Fig. 2106).
If it occurs equatorially or posteriorly, extra-ocular extension may

D D 2
2498 TEXT-BOOK OF OPHTHALMOLOGY
remain undiagnosed until exophthalmos or chemosis sets in. Quite
frequently a small amount of extension occurs at an early stage, associated
sometimes, perhaps, with the Origin of the tumour from the sheath of a nerve
traversing the sclera (Fig. 2098); but as we shall see immediately, this
does not necessarily indicate a hopeless prognosis, for there is evidence that
after enucleation these orbital infiltrating cells may disappear (Wätzold,
1930). In any event, cells of malignant melanomata are frequently left
in the orbit after enucleation and orbital recurrences are relatively rare
(v. Hippel, 1930; Terry and Johns, 1935; Denecke, 1936). At the
same time it must be remembered that such cells may remain dormant
for long, or alternatively proliferate extremely slowly, for orbital recurrences
have delayed their appearance for many years after enucleation (10 years,
Freudenthal, 1891). If events are allowed to take their course, how-
ever, the end-result can only be described as horrible, for extension proceeds,
filling up the entire orbit, breaking through the roof into the cranial cavity,
or infiltrating into the ethmoidal region with eventual involvement of the
nose and palate.
The lamina cribrosa usually resists infiltration for a long time, but
extension through it and up the optic nerve is not unknown (Jaensch, 1932)
(Fig. 2105). Terry and Johns (1935) found such extension in 12 out of their
94 cases, in two of which the cranial cavity along the nerve was grossly
invaded, the chiasma was destroyed and a temporal hemianopia produced
in the other eye. Such an occurrence, however, cannot be called common ;
a case, for example, was recorded by Charamis (1930) wherein a juxta-papillary
tumour filled the entire globe and yet no penetration of the sclera or of the
lamina had occurred.
The most advanced cases of extra-ocular spread are rare in civilized countries
to-day, but yet they do occur, as witness the case reported by Appleman (1932) in
America, in which the patient did not seek advice until the eye was “three times the
normal size '’ and the whole orbit was filled with neoplastic tissue. A parallel case
was described by Cardell (1935) in England wherein a large fungating tumour pro-
truded from the eye, the cornea was invaded along new vessels, the globe was
filled and greatly distended, the orbit was occupied, and extension had occurred up
the optic nerve, the patient eventually dying of a cerebral seizure. The lesser degrees
of extra-Scleral perforation are, however, frequent, for, as would be expected con-
sidering the neural origin of these tumours, this may occur at an early stage. Thus
Kerschbaumer (1900) found that 23 cases out of 58 had extra-ocular extension ;
Jaensch (1930), in a study of 68 cases, found the sclera involved in 13, in 12 of which
perforation had occurred, and in 5 of which the orbit was filled with new growth ;
while Kronenberg (1938) found extra-ocular extensions in 36.1% of his cases.
Intra-ocular Metastases. Local metastases in the eye are rare, a very
striking contrast to their great frequency in retino-blastomata. They occur
most commonly with ciliary tumours, and are usually found in the anterior
parts of the eye. The posterior surface of the cornea (Fig. 2107), the
anterior surface of the iris (Fig. 2108), and the angle of the anterior chamber
DISEASES OF THE UWEAL TRACT 2499
may be sown with isolated aggregations of tumour cells, which may also
permeate the substance of the iris and the ciliary processes. It is a common-
place for pigmented cells to accumulate in the angle of the anterior chamber,
but these usually remain inert and do not lead to metastatic deposits, although
Fig. 2107.-MALIGNANT MELANoMA of CHoRoid : Corne AL METAstases.
Showing deposits of tumour cells on the posterior surface of the cornea (x 120)
(Coats, R. L. O. H. Rep.).
they may obstruct the drainage of fluid. It is possible that these metastatic
deposits reach the anterior chamber by way of the root of the iris or the
corneo-iridic angle, but the presence of free tumour cells in the posterior
chamber in a ciliary melanoma suggests that local dissemination through the
Fig. 2108.-MALIGNANT MELANoMA of CHoRoid : METASTAses IN IRIs AND CILIARY Body.
A. Layer of tumour cells on the anterior surface of the iris deposited from the
anterior chamber and not derived from the underlying tissue. B. (and elsewhere).
Isolated tumour cells. C. Pigmentary epithelium artificially separated. D. Ciliary
process infiltrated with discrete pigmented neoplastic cells (x 120) (Coats, R. L. O. H.
Rep.).
pupil is possible (Coats, 1912), a view confirmed by the observation that
these free cells in the anterior chamber may show mitotic activity
(Bergmeister, 1910). It is also interesting that the posterior surface of the
cornea and the anterior surface of the iris have been studded with metastatic
deposits 15 months before a ciliary tumour was detected (Fehr, 1902).


2500 TEXT-BOOK OF OPHTHALMOLOGY
Such cases are not particularly common. Primary tumours of the ciliary body
giving rise to intra-ocular metastases have been recorded by Hirschberg (1893), Panas
and Rochon-Duvigneaud (1898), Groenouw (1899), Kamocki (1900), Wagenmann
(1900), Fehr (1902), Parsons (1903), and Coats (1912); and of the iris by Argyll
Robertson and Knapp (1873), Solomon (1882), and Axenfeld and Kayser (1903).
Metastases in the sub-retinal space are less common, and have been
observed on few occasions only. The outer aspect of the retina and the
inner aspect of the choroid may be sown with nodules (Williams and Knapp,
1874; Coats, 1912), the outer aspect of the retina may be affected (Ewetsky,
1896) or the choroid alone may be invaded (Mitwalsky, 1894) (Figs. 2109
and 2110).
Fig. 2109.-MALIGNANT MELANoMA of Fig. 2110.-MALIGNANT MELANoMA or
CHonoid : Choroid AL Metastases. THE Choroid : RETINAL METAstases.
Showing local metastases on the inner Invading the outer aspect of a
surface of the choroid. (; 120) (Coats, detached retina near the ora (x 120)
R. L. O. H. Rep.). (Coats R. L. O. H. Rep.).
The presence of free cells in the vitreous has been observed on a few
occasions—by Mitvalsky (1894), Ewetsky (1896) and Neame (1922)—all in
the flat type of growth when it had invaded the ciliary body.
The few cases on record wherein more than one tumour occurs in the
same eye raise the question as to whether they are of independent develop-
ment, corresponding to multiple naevi, or are metastatically derived the one
from the other. Thus Fuchs (1882) observed five small tumours in the choroid
all separated from one another by normal tissue, and other cases have been
described with two (Velhagen, 1920; Rochat, 1926). Owing to the paucity
of histological evidence the matter cannot yet be definitely answered.
Systemic Metastases. Extra-ocular metastases form the usual fatal
termination of malignant melanomata. They may occur at the very earliest
stage in the evolution of the tumour (Hensius, 1931), they may cause

DISEASES OF THE UVEAL TRACT 2501
symptoms before any attention is drawn to the eye (Greeves and Holmes,
1914), but quite frequently their occurrence is delayed until a fairly advanced
stage is reached. The pre-auricular and neighbouring glands are not
affected ; for the metastases are blood-borne, the cells readily breaking into
the wall-less channels in the growth and probably less frequently invading
the normal blood-vessels.
Much the most fertile field for metastases is the liver (Fig. 2111), and
thereafter almost any organ in the body may be affected (Fig. 2112). The
Fig. 2111.-MALign ANT MELANoMA Fig. 2112.-MALIGNANT MELANoMA or
of CHoRoid : METASTAs Es IN Choroid : METAst Ases IN INTEs.TINE
Live R. (Lediard, T. O. S.) (Lediard, T.O.S.)
following are two series—an old and a recent—in the literature. Fuchs
(1882) gives: liver 3, stomach 7, sub-cutaneous tissue 4, heart, kidney,
bones, mucous membranes, each 3, lungs 3, spine, lymph glands 2, spleen,
pancreas, intestine, epiglottis, brain, each 1. Terry and Johns (1935) give :
liver 15, general 3, spine 2, lungs, stomach, kidney, lymph glands, hip, brain,
orbit, each 1. Metastases in the central nervous system may be extremely
widespread in the infiltrating type of growth, tumour tissue sometimes
extending uniformly down to the dorsal and ventral roots of the cord (Greeves
and Holmes, 1914; Neame, 1922) (Figs. 2113–15). The pigment content
of the neoplasm has no relation to metastasis.
With regard to location, choroidal tumours, especially when situated posteriorly,
most readily give rise to metastases, while tumours of the iris are much less malignant.
With regard to cellular types, Callender and Wilder (1935) and Terry and Johns (1935)
found that pure spindle-celled tumours with ill-defined nucleoli have a good prognosis
from this point of view, while the so-called epithelioid cell is responsible for the highest

Figs. 2113-15-MALIGNANT MELANoMA of Choroid : Metastases IN CENTRAL NER vous
SystEM.
Fig. 2113-Metastases in brain: a large tumour in the right temporal lobe;
metastases on the ventral surfaces of the pons, medulla and cord; a nodular tumour
on the right oculo-motor nerve. The ocular tumour was of the flat type.
Fig. 2114-Meta- Fig. 2115.-Transverse section of the 3rd lumbar segment
stases in cord : the showing diffuse infiltration of the pia, large nodules of growth
ventral surface of cord at the entrance of the dorsal and ventral roots, and extensions
showing nodular along the perivascular spaces (Greeves and Holmes, T. o. S.).
tumours and diffuse
discolouration of the
pia.


DISEASES OF THE UVEAL TRACT 2503
mortality rate. Callender and Wilder (1935) also found that a heavy reticulum was of
good prognosis, while those tumours wherein no fibrils were present except in the
inter-lobular stroma were invariably and rapidly fatal.
As a rule the main tumour and its metastases have essentially the same
structure, although minor differences are common. A different cellular
arrangement may be evident due to the infiltration of pre-existent tissue-
spaces; the form of the cells may differ in proportion, a primarily spindle-
celled growth in the eye giving rise to a metastatic tumour characterized
mainly by round cells. Specialized structure in a part of the primary
growth may be universal in the secondary deposits, and the latter may be
non-pigmented when the former is melanotic ; indeed, some nodules in the
liver may be pigmented and lie side by side with others which are not.
Microscopic Anatomy
The general histological structure of malignant melanomata varies
very much, not only among different tumours but in different parts of the
same tumour. The microscopical appearances have been the basis of
-º
:
:
-§
Fig. 2116.-MALIGNANT MELANoMA of Choroid.
Differentiation of the tumour into cavernous form. The stroma is made up of small
fusiform cells as seen in the choroid (x 80) (Susman, Brit. J. O.).
several attempts at classification, but are probably not of fundamental
significance. In most cases the determining factor in the general arrange-
ment is the blood-vessels, the majority of which are merely endothelial tubes
with a wide lumen, or channels between the cells with no proper wall. It is
for this reason that haemorrhages and metastases are so common. When the


2504 TEXT-BOOK OF OPHTHALMOLOGY
growth is very vascular and the cells are directed longitudinally to the axes of
the vessels, the tumour may have a cavernous appearance (Fig. 2116); when
this arrangement becomes closely packed and regular a tubular structure is
produced (“angio-sarcomata”). When the normal tissue spaces in the
uvea become filled with tumour cells so that the fibrous and elastic tissue-
stroma is pushed apart, an alveolar appearance is produced; and this
structure may again be simulated by a profuse division and anastomoses of
vessels marking out areas occupied by neoplastic cells. Finally, if the cells
are grouped around endothelial-lined spaces which do not contain blood, the
term endothelioma or lymph-angiosarcoma has been applied.
º
sº
- -
- -
- -- -
- º :: - -
- -- . -
-
-
---
-------
Fig. 2117,-MALignant MELANoMA or Choroid.
Showing spindle-celled structure (x 80) (Susman, Brit. J. O.).
Cytology. Many types of cells are found in malignant melanomata, and
frequently the same tumour contains several varieties in various parts.
Various classifications have been given from time to time but perhaps the
most useful is that of Callender (1931).
1. SPINDLE-CELLs (Figs. 2117, 21.21). These are much the commonest
and their appearance led originally to the inclusion of these tumours in the
category of sarcomata. They resemble closely the embryonic connective-
tissue cells associated with such neoplasms, but the long oval nucleus is large
and more vesicular. They are arranged in closely packed bundles in sheets
or whorls or quite irregularly, and their ends terminate in drawn-out fibres.
Callender divided them into sub-types depending on their nuclear structure:
(A) with a delicate reticulum and ill-defined nucleolar material, and (B) with a coarse
deeply staining nuclear network and a dense round central nucleolus.








DISEASES OF THE UWEAL TRACT 2505
2. FASCICULAR CELLs. These are elongated oval or spindle cells but
sometimes assume a rounded or oval shape, arranged distinctively in
columns or fasciculi radiating in palisade form from a central lymphatic or
capillary blood-vessel. Tumours with this distinctive cell were formerly
classed as peritheliomata (Fig. 2118).
--- - -
****
º
-
- -º-º: * & tºº, Tº
sº
ºfeºfºº º, ø, ºr ſº.
ºes ºs º- º º º
Eºº. º:
ºf cº.º.
º º
Fig. 2118.-FAscicular CELLs IN FIG. 2119.-EPITHELIOID CELLS IN A
MALIGNANT MELANoMA of CHOROID. MALIGNANT MELANoMA.
Fascicular cells arranged in cylin- From an iridic tumour showing
drical columns resembling al endotheliomatous-like cells (x : 300)
perithelioma (Parsons). (Collins).
Fig. 2120. GIANT CELL IN MALIGNANT MELANoMA of Choroid. (Mayou.)
3. EPITHELIoID CELLs. Large polygonal or rounded cells with an oval
nucleus. They usually lie in a well-marked recticular stroma, and were
formerly classed as endotheliomata (Fig. 21.19).
In addition to these, giant cells are occasionally found (Fig. 2120). Their signi-
ficance is not understood ; but they are probably evidence of rapid cell-division
and are without significance.
On the basis of this cellular study Callender (1931) divided malignant melanomata


2506 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2121.-SPINDLE-cºlled MALignant Fig. 2122--THE REriculin or a
MELANoMA. MALIGNANT MELANoMA.
From a choroidal tumour stained with The same section as Fig. 2121. Stained
haematoxylin and eosin to show the cellular for reticulin by Wilder's silver method.
structure (King). Areas with and without fibrils are approxi-
mately equal (King).
Fig. 2123.-The RETICULIN or a Fig. 2124.—THE REriculin or a
MALIGNANT MELANoMA. MALIGNANT MELANoMA.
Stained as in Fig. 2122 : areas with Stained as in Fig. 2122. The sclera up
fibrils predominate (King). and to the left ; the tumour down and to
the right. No fibrils are present except in
the sclera and the adjacent parts of the
tumour (King).


DISEASES OF THE UVEAL TRACT 2507
into four groups: (1) spindle-cell type with its two sub-groups A and B, (2) fascicular
type, (3) epithelioid type, and (4) mixed type. It is of prognostic interest and
importance that group 1A has the best prognosis and the mixed type the worst, the
epithelioid cells, whether occurring alone or in company, being responsible for the
highest mortality rate, a finding on which there is general agreement (Fuchs, 1882;
Callender and Wilder, 1935; Terry and Johns, 1935).
Stroma. Melanomata are usually very cellular and frequently the cells
appear to have no stroma between them, except perhaps in association with
the nutrient vessels. We have already seen " that there is no reason to
doubt that the collagenous fibres are, like the cells, of neurogenic origin
(Masson, 1931). The distribution of reticulin (argyrophil fibres) is very
irregular, varying in different parts of the same tumour from complete
absence to a dense network embracing each cell (Callender and Wilder, 1935;
King, 1937) (Fig. 2122). These fibres are demonstrated by the silver
impregnation method of Bielschowsky ; occasionally a tumour shows
no reticulum of any kind (Fig. 2124); others show an extremely dense
network (Fig. 2123); while yet others show areas wherein the network
is dense and interspersed with free areas (Fig. 2122). Callender and
Wilder (1935) attach considerable prognostic importance to its density,
metastases being much less frequent when it is abundant and inevitable
when it is absent ; it would, indeed, be reasonable to assume that a highly
developed stroma might act as a mechanical barrier to the embolic escape
of cells.
Pigment. Although the presence of pigment forms so striking a clinical
and histological characteristic of these tumours, it has generally been
admitted since the time of Virchow (1863) that it has no fundamental
significance. It is mainly melanin, and may occur in all types of cell and
occasionally as isolated or clumped granules between the cells. As has
already been discussed at length,” although much controversy has raged
and is still being raged over its origin, there seems little doubt that its forma-
tion is the result of an incidental metabolic activity of the neurogenic cells
which constitute the tumour, demonstrating a reversal to their embryonic
character. The rod-shaped retinal pigment is occasionally seen when Bruch's
membrane has been perforated and the epithelial cells invade the growth
(v. Hippel, 1894; Leber, 1897 : Kerschbaumer, 1900). Haematogenous
pigment is also frequently found indicating the occurrence of haemorrhage,
the pigment in this case being frequently contained in leucocytes.
Changes occurring in Tumours
Haemorrhages are of frequent occurrence, usually small and sometimes
recurrent (v. Hippel, 1894); indeed, a haemorrhage into the anterior chamber
* Foot (1925–28) modified Bielschowsky’s original method by using ammonium hydroxide
and silver nitrate ; Wilder (1935) employed preliminary sensitization by uranium nitrate ; and
King (1937) found it advisable to bleach all sections first.
2 S).
p. 2461.
2508 TEXT-BOOK OF OPHTHALMOLOGY
or the vitreous (Rönne, 1936) may be the first clinical evidence of the
presence of a tumour, and this by causing glaucoma may draw attention to the
condition. A case was reported by Mayou (1932) wherein the first symptom
was a haemorrhagic discoloration of the iris. Extensive haemorrhages are
not at all uncommon (Fig. 2125); thus Verhoeff (1904) found comparatively
large extravasations in 25 out of 55 neoplastic cases examined. Indeed, a
spontaneous intra-ocular haemorrhage should always raise the question of a
malignant tumour in the diagnosis. It may be that in some cases the bleed-
ing is secondary to necrosis, and in others that it causes necrosis; but it is
Fig. 2125.-DEGENERATING MALIGNANT MELAN oxia.
A mass near the posterior pole consisting of a large haemorrhage detaching the
retina with a few trabeculae of degenerated neoplasm (Mayou, T. O. S.).
not surprising that such incidents occur when the fragility or absence of
walls in the vascular channels is recalled.
Degenerative changes are relatively common, and become the rule in
extensive neoplasms. Hyaline degeneration occurs frequently, affecting first
the vessel walls and later the cells themselves. The deposition of glycogenous
material (Best, 1898–1901; Kerschbaumer, 1900) or fatty globules in the
cells is rarer (Ginsberg, 1912); but the most interesting and typical change
is necrosis.
Necrosis, with its accompanying inflammation and uveitis, is a common
and important phenomenon in malignant melanomata (Fig. 2126). It has
been intensively studied by Leber and Krahnstºver (1898), Parsons (1905),
Fuchs (1910), and Samuels (1929–34). It may occur in some cases as the
result of haemorrhages (Verhoeff, 1904), or of a local lack of blood supply

DISEASES OF THE UVEAL TRACT 2509
due to compression producing a deficiency of nutrition (Ewetsky, 1898;
Neese, 1907); at other times it may be the result of endogenous microbic
cLEMENT CLARKE
Fig. 2126.-NEC Rotic MALIGNANT MELANoMA.
A. Mushroom-shaped melanoma. B. Necrotic area. C. Rupture in Bruch's
membrane. D. Base of neoplasm. E. Sclera (Goulden and Stallard, T. O. S.).
infection acting on susceptible tissue (Leber and Krahnstover, 1898;
Parsons, 1905), or again, of virulent cyto-toxins being diffused throughout the
Fig. 2127.-NEcRoric MALIGNANT MELANoMA (x 3) (Parsons).
tissues of the eye (Ewetsky, 1898; Palich-Szantó, 1919; Samuels, 1934).
In any event it is a common phenomenon: Leber and Krahnstover (1898)


2510 TEXT-BOOK OF OPHTHALMOLOGY
collected 34 cases, Fuchs (1882) found partial necrosis in 62 out of 150 cases,
of which 19 were completely necrotic. Samuels (1934) met some degree of
necrosis in 84 out of 106 tumours examined, of which 24 were completely
necrotic. Terry and Johns (1935) found necrosis present in 67 out of
94 tumours. Usually the necrosis is patchy, in which case no symptoms
may arise; but at other times it is massive, the whole tumour mass appearing
Fig. 2128-REsults or Comple:TE NECRosis of A MALIGNANT MELANoMA.
T. The tumour. Pus formed as a result of the necrosis has caused a perforation
(P). The retina is everywhere necrotic and has fallen to pieces at R. The anterior
chamber is filled with blood and pus and the lens (L) has been dislocated by the
tumour (Samuels, T. O. S.).
histologically like a broken-down mass of blood-clot (Parsons, 1905) (Fig.
2127). When it is at all massive, a more or less severe uveitis is invariable,
and since the cyto-toxins diffuse throughout the globe, every tissue is
attacked, as a rule the anterior part of the globe more severely than the
posterior (Fig. 2128). The ciliary body is always severely affected, the
iris follows suit, and the choroid too is involved; the retina may disintegrate
away; the cornea and sclera are not exempt; and the lens turns cataractous.
Even in the less advanced cases a severe plastic inflammation develops, and a
hypopyon or a hyphaema is common. One striking feature, often of
diagnostic importance, is the appearance of a vascularized membrane on

DISEASES OF THE UVEAL TRACT 25.11
the iris. The clinical picture may be abruptly changed at any time by the
development of a fulminating glaucoma; while, on the other hand, a pre-
ceding glaucoma may have prepared the way for a rapid disintegration of
the tissues. Such cases, indeed, are usually not suspected as being malignant,
and the eyes are frequently excised for absolute glaucoma. On the other
hand, these cases may be diagnosed as panophthalmitis (Samuels, 1934) or
as a vitreous abscess (Butler and Assinder, 1924), or be excised for a recalci-
trant irido-cyclitis with secondary glaucoma (Williamson-Noble, 1925), or
may even proceed to a purulent perforation (v. Graefe, 1862; Samuels,
1933). In other less acute cases the eyeball shrinks, so that the clinical
Fig. 2129.-SHRUNREN GLoBE containing A MALIGNANT MELANoMA (Chance, T. Am. O. S.).
entity of a malignant melanoma in a phthisical eye is found as a relatively
common event, which on rare occasions and in totally necrotic tumours,
may result in a spontaneous local cure (Fuchs, 1910) (Fig. 2129).
The question of the development of sympathetic ophthalmitis in such cases has
already been fully discussed."
Fibrosis is not uncommonly seen in the neighbourhood of these tumours, the
proliferation of connective tissue around them being presumably the result of irritative
products, in this case less virulent than those causing widespread necrosis and acute
inflammation (Nettleship, 1904; Collins, 1929).
Glaucoma is a feature of a large number of cases at some stage of the
disease; indeed, it is frequently the first symptom noticed (Terry, 1939).
It is important to remember, however, that in the earlier stages the tension
is not necessarily raised. The older authors concluded that the tension
1 p. 23.29.
T-0.-W.O.L. 111. E. F.

2512 TEXT-BOOK OF OPHTHALMOLOGY
was never reduced unless the ciliary body was involved (Marshall, 1899) or
unless a plastic inflammation was present (Leber and Krahnstover, 1898);
but more recent records show that a definite hypotony may eacist initially
(Franz, 1920; Francis, 1920), and indeed, that when compared with the
other eye, an initial drop in intra-ocular pressure is the rule in the early
stages whether the neoplasm affects the ciliary body or the choroid
(Donnington, 1938). Eventually, however, the reverse relationship holds,
and the tension tends to rise. In most cases this is probably due to
obstruction of the venous return from the ciliary body and choroid inducing
circulatory congestion and stasis (Evans, 1939), to which factor may be
added obstruction to the circulation of the intra-ocular fluid at the channels
of exit. In some cases this is due to cellular accumulations at the angle of
the anterior chamber ; in others to actual infiltration of the angle, so that
glaucoma is an early symptom in infiltrating tumours in this neighbourhood ;
in others it may be due to the formation of a vascularized membrane on the
iris ; and in others to recurrent haemorrhages ; but the classical conception
advocated by Priestley Smith (1891) that the rise of tension, particularly with
tumours of the posterior segment, was volumetric and was due to a displace-
ment forwards of the vitreous and lens forcing the periphery of the iris
against the cornea, is probably rarely if ever operative. A final influence may
be the liberation of histamine-like substances by the tumour in the earlier
stages of necrosis. When the eye is almost filled with new growth, glaucoma
is practically invariable. Conversely, the destruction and atrophy of the
ciliary processes in the later stages may lead to hypotony and shrinkage
of the globe.
Symptoms
From what has been already said it will be apparent that the symptoms
of malignant melanomata can be very various and confusing, and indeed,
may be altogether absent until the occurrence of extra-bulbar nodules or
until metastatic disturbance in the central nervous system or abdomen draws
attention to the case. We have also seen that, although it is often true,
the sequential progress in four stages made classical by early writers, is
misleading—a first symptomless stage lasting one or several years, a
glaucomatous stage lasting generally less than a year, a third stage of extra-
ocular extension, and a final metastatic stage. In view of the fact that
metastases may occur while the tumour is still in its earliest stages, the
paramount importance of early recognition is amply evident ; but unless
the tumour is in the iris and the patient or his friends are sufficiently
observant to notice it, or is situated at the macula or in its vicinity so that
an early retinal detachment involves the macula and thus disturbs central
vision, it is all too common for growth to progress until toxic or pressure
symptoms appear and draw attention to the condition. If by good fortune
the macula is affected, the early symptoms are a change in colour values
DISEASES OF THE UVEAL TRACT 2513
(usually a poor discrimination for green, Déjean and Varnier, 1934),
micropsia due to separation of the retinal elements, an increase in hyper-
metropic refraction, and finally a central scotoma. When the tumour is
situated elsewhere visual symptoms are lacking until a spreading retinal
detachment occurs, or until the growth obstructs the visual axis. Occa-
sionally, especially with ciliary tumours, a certain amount of referred ciliary
pain occurs, but this is not by any means constant ; and in these also a
localized scleral telangiectasis with an engorgement of the episcleral veins in
One quadrant may be significant.
Apart from this, the symptomatology is essentially indirect. We have
seen that one of the most common clinical pictures is that of secondary
glaucoma. The other common manifestation is the appearance of toxic
symptoms—usually an irido-cyclitis, more rarely an optic neuritis (Vancea,
1930–31). We have seen that it is not at all uncommon for eyes to be
excised for a stubborn and painful irido-cyclitis with secondary glaucoma,
or in a shrinking phthisical state, when an unsuspected neoplasm is discovered
pathologically. Another rare occurrence is an intra-ocular haemorrhage,
frequently associated with an intractable glaucoma ; a haemorrhagic absolute
glaucoma should always be looked upon with suspicion. Two points which
may be of considerable significance are a powdering of the iris and cornea
with pigment (Fehr, 1902; Purtscher, 1906), and an anaesthesia of a section
of the cornea (Sédan, Ourgand and Morenon, 1934; Sédan, 1935), an
indication perhaps of the origin of the neoplasm in the sheaths of the ciliary
Iner VeS.
In the symptomatology therefore the following points are suggestive—
a unilateral rise of tension without evidence of the usual signs of chronic
glaucoma, especially if preceded by a period of low tension, and more
particularly if associated with a detachment of the retina or a naevus of the
iris which appears to have grown ; a unilateral uveitis ; a unilateral disturb-
ance of ocular pigment ; and an atypical detachment of the retina, especially in
a hypermetropic eye in the absence of a retinal hole or a choroiditis and
without a history of injury. In any detachment, points in favour of the
presence of a tumour, which, however, are by no means invariably present,
are an absence of undulations and waviness, a regularity of outline, the
presence of pigment deposits, and the appearance of a second system of
vessels separate from the retinal vessels. A sudden and extensive retinal
detachment with a retinal tear is rarely associated with a neoplasm ; and a
small circumscribed detachment, especially near the macula, usually is. A
detachment with raised tension is suggestive, but a normal or sub-normal
tension is by no means exclusive. A further point is the presence of a
yellowish tinge in neoplastic detachments, which is sometimes quite obvious
and is probably due to the richly albuminous sub-retinal fluid (Rönne,
1936). It is to be remembered also that the extent of a detachment gives
no index to the size of a tumour, nor necessarily does its site, since it may be
E E 2
2514 TEXT-BOOK OF OPHTHALMOLOGY
remote. Finally, a blind and painful phthisical eye so frequently contains a
tumour, that on this ground alone it can be argued with a considerable
amount of justification that all such eyes should be excised (Chance, 1934;
Terry, 1939). A similar generalization should be made in the case of eyes
with absolute glaucoma in which the fundus cannot be seen ; thus Neame
and Kahn (1925) found pathologically that as high a proportion as 10% of
402 such eyes contained an unsuspected malignant tumour.
Two special diagnostic methods can be employed in the objective examination
of the eye. The first and most useful is transillumination (diaphanoscopy).” Trans-
pupillary transillumination may be of value in investigating tumours of the anterior
segment ; but the more generally useful method is trans-scleral transillumination,
either by studying the pupillary reflex, or, usually more usefully in choroidal tumours,
by the direct method of Lindahl (1920) when the observer simultaneously examines
the fundus with the ophthalmoscope. If the neoplasm is situated behind the equator
no hesitation should be felt in introducing the transilluminator into Tenon's capsule
when very definite information can be obtained especially by switching the trans-
illuminator off and on and comparing alternately the appearance by simple ophthalmo-
scopy with that by ophthalmoscopic transillumination (Lindahl, 1920; Holth, 1926;
Shoemaker and de Long, 1926; Rönne, 1928–36; Klein, 1929 ; and others). It is
to be remembered, however, that the findings by transillumination are not always
unequivocal : heavily pigmented eyes may prevent transillumination, a small tumour
may allow the light past it and fail to throw a shadow, a haemorrhage or inflammatory
mass may obstruct the light, a non-pigmented growth may not, and a cyst with thick
pigmented walls may appear solid.
Diagnostic puncture has occasionally been resorted to, either of the anterior
chamber or the sub-retinal space, the fluid being centrifuged and examined for neo-
plastic cells (Ruben, 1906; Meisner, 1923; v. Grósz, 1929). Moreover, in the last
resort between the diagnosis of a solid or cystic mass, an exploratory puncture may
be made ; but in these cases the danger of encouraging metastatic spread should be
remembered, and preparations should be made for immediate excision should the results
point to the presence of a neoplasm.
From the point of view of differential diagnosis the most common conditions
which ought to be remembered are :—In the iris and ciliary body—inflammatory
granulomata, especially tubercle and syphilis, secondary tumours and cysts. In the
choroid—inflammatory conditions, especially tubercle, syphilis, exudative retinitis,
disciform macular degeneration, haemorrhages, especially sub-choroidal haemor-
rhages, simple detachments of the retina and choroid, retinal tumours, especially
neuro-blastomata and angiomata, simple choroidal tumours, especially navi or
angiomata, secondary choroidal tumours, retinal cysts and parasitic cysts (cysticercus).
The prognosis of a malignant melanoma is thoroughly bad if it is
allowed to develop, death usually being due to general metastases or
occasionally to local spread in the cranial and nasal cavities ; the occurrence
of spontaneous healing by necrosis (Fuchs, 1910) is so rare and exceptional
that it cannot be considered in the assessment of a case. Even if the eye
is removed the prognosis is very uncertain and sometimes, although very
rarely, the operation may be rapidly followed by a generalized dissemination
(Lange, 1913). So far as structure is concerned, the spindle-celled type with
1 Vol. II, p. 1153.
DISEASES OF THE UVEAL TRACT 251 5
a delicate nuclear structure gives the best prognosis ; and the round-celled
epithelioid type the worst ; the less dense the inter-cellular reticulum the
worse the prognosis ; while infiltrating growths are more dangerous than
the discrete variety. Since metastases may occur at any time, the sooner
the eye is removed the better ; but even if enucleation has been performed
at a very early stage, metastatic spread is not excluded. Its occurrence,
indeed, is a mere matter of chance, and there is statistical evidence that
enucleation during the second stage is only slightly less effective than during
the first (Wätzold, 1930 ; v. Hippel, 1933–36). After extra-ocular extension
has occurred, however, the incidence of fatalities increases rapidly ; but
again not invariably.
Thus Holmes Spicer (1912) recorded an absence of metastases after 13 years,
although at the time of enucleation the sclera had been perforated and the orbit
invaded. A similar case with a similar time interval was reported by Denecke (1936)
even although exenteration of the orbit was not performed. Parsons (1905) recorded
a case with extensive extra-bulbar growth which showed a local recurrence, but
was still alive and well 7 years after exenteration of the orbit : Denecke (1936)
reported a similar case also well 124 years later. It must therefore be accepted that
those cases wherein cells are found in the emissaria, although undoubtedly giving rise
to anxiety, are not necessarily doomed : v. Hippel (1930) reported 12 such which were
well 5 years subsequently.
An expression of the prognosis in percentages after enucleation is
difficult, since most of the earlier writers accepted much too short a period
of observation in their estimation of a case. Most local recurrences and
metastases certainly occur within the first year, but they also occur quite
frequently between the 5th and 10th years, while longer periods have been
reported (17 years, Foster Moore, 1914). There is, therefore, no time-limit
of safety.
Largely because of the short time-limit involved the older statistics are too
favourable. Fuchs (1882) found death due to general dissemination in 18.5%. Law-
ford and Collins (1891) traced 79 cases of whom 40 were dead, 26 being due to metastases
(the average duration of life being 2–3 years) and 1 being due to local recurrences, 6 of
which took place within 7 months ; 25% of the patients were alive 3 years after
enucleation. Among later statistics v. Grósz (1929) found that 50% died of metastases;
Denecke (1936) found that of 36 cases 14 died of metastases, 11 within the first 5 years,
and one 143 years later ; and v. Hippel (1933–36) that 62% lived 5 years and 30%
10 years after enucleation. His figures were as seen in Table LXIV.
TABLE LXIV

sº. * Of Cases | Metastases ID “tº Other Patients alive
|
I 61 23 14 24
II 48 25 10 13
III | 9 8 --- I
| :
2516 TEXT-BOOK OF OPHTHALMOLOGY
Extra-ocular recurrences are rather the exception than the rule, even
although the escape of tumour cells by the emissaria must be very common ;
while recurrence in the orbit is rare, figures from the literature show that
some 15 to 30% of cases show extra-ocular extensions : Lawford and Collins
(1891) 21 cases in 103 ; Marshall (1899) 6 in 58 ; Kerschbaumer (1900) 15
in 58 ; Pawel (1899) 21 in 100 ; Byers and MacMillan (1935) 5 in 51. It is
probable that the paucity of these recurrences indicates a considerable degree
of local immunity to small and isolated neoplastic elements (Wätzold,
1930), a factor the existence of which is suggested by the very long time
which occasionally elapses before the recurrence becomes evident (12 years,
Steiner, 1922; 13 years, Lukens, 1932, and Newton, 1938; 30 years, Pawel,
1899). -
A serum test modified by Klein (1935) from the technique of Freund and Neuberg
has been suggested to aid in prognosis (v. Hippel, 1936). It depends on the possession
of the serum of persons not affected by malignant tumours of a histiolytic property
for malignant cells. The presence of this property (a negative Klein reaction) indicates
the existence of a defensive power against neoplastic growth and the absence of a
tumour. Patients, therefore, with a uveal tumour should give a positive reaction,
and after enucleation in the absence of metastases should give a negative test. The
reaction, however, is delicate and complicated, and the results so far have not
invariably been correct.
Treatment
As soon as a malignant melanoma is diagnosed the treatment of election
is enucleation of the eye. One exception to this is the case of certain
tumours of the iris wherein eaccision by iridectomy may be considered ; the
indications for this less mutilating procedure are that the tumour be small,
well-defined, situated at or near the pupillary margin and not encroaching
on the ciliary border, when the iris is mobile, the tension is normal, the
vision good, and when there is every evidence that complete removal can be
attained by iridectomy, a circumstance which should be proved by immediate
pathological examination of the excised tissue. When these conditions do
not prevail the eye should be enucleated ; a course which is safer in all cases.
It is to be remembered that incomplete excision frequently leads to rapid
local extension and presumably favours general dissemination. A second
exception may perhaps be made on rare occasions when special circumstances
exist (as the occurrence of a tumour in an only eye) when electro-coagulation
by diathermy may be considered, but not without misgivings (Weve, 1939)
(Figs. 2130–31).
Enucleation, in the case of choroidal tumours, should involve as much
of the orbital tissue at the posterior portion of the globe as possible,
and the maximum length of optic nerve. The tissue thus excised should
be examined pathologically for retro-bulbar nodules, and if any evidences
of extra-ocular extension are found, the safest course is to proceed to an
eacenteration of the orbit. In this operation the merits of electro-coagulation
DISEASES OF THE UVEAL TRACT 2517
should be considered, in so far as it controls haemorrhage, permits of the
most thorough removal of the orbital contents, and by reducing trauma
to a minimum and by coagulating the orbital vessels at the limits of
the field, should theoretically minimize the danger of operative
dissemination. A more pleasant alternative to this is radiation. Some
authors (Wätzold and Gyotoku, 1928) have advocated exenteration as
a routine measure, but, in view of the relative rarity of such recurrences, this
course seems unjustifiable, especially if enucleation is followed by radiation.
Fig. 2130.-MALIGNANT MELANoMA of Choroid TREATED BY DIATHERMY.
The original tumour. (See further Fig. 2131.)
If extra-ocular extension has occurred by way of the anterior perforating
vessels, it is probably sufficient to excise a very liberal amount of conjunctiva,
and follow this up by radiation. If the orbital walls and accessory sinuses
have been invaded, wide and extensive operations (Golovine, 1909; Filatov,
1923) may be attempted, followed by radiation as an alternative to a very
painful and nauseating death.
The value of radiation as a post-operative measure is undoubted; but
as a primary method of treatment it is extremely precarious and in the present
state of knowledge is not justified. It has been very rarely attempted;
and its use may lead to a reaction so severe that the eye has had

25.18
TEXT-BOOK OF OPHTHALMOLOGY
to be excised because of pain (Gifford, 1918).
Reports of a marked diminu-
tion of growth over a period of years have been recorded after X-rays (Salzer,
1921), or after the insertion of radon needles (5 millicuries) into the neoplasm
Fig. 2131.-MALign ANT MELANoMA or Choroid TREATED BY DIATHERMY.
The same case as Fig. 2130 in the atrophic stage 2 years after diathermic
coagulation (Weve, T. O. S.).
itself (Foster Moore, 1930); but any such attempts should be limited to
cases wherein an only eye is affected or when other very special indications
exist.
Ind. J. Med. Res., ix, 464, 1922.
Brit. J. O., xiv. 448, 1930.
Appleman. A. of O. viii. 618, 1932.
Arganarez. El Sarcome de la Coroides, Buenos
Aires, 1916.
Axenfeld and Kayser. B. O. G. Heidel. xxxi,
277, 1903.
Bergmeister. A. f. O., lxxv, 474, 1910.
Best. Beit. … path. Amat., Xxiii. 253, 1898.
B. O. G. Heidel.., xxix, 63, 1901.
Bloch. Z. f. phys. Chem..., xeviii. 226, 1917.
A. f. Derm. Syph... cxxxv, 77, 1921.
Bruner. A. of O., liii, 162, 1924.
Butler and Assinder. Brit. J. O., viii. 321,
1924.
Acton.
Adams.
Byers and MacMillan.
1935.
Callender. T. Am. Acad. O. Oto-Lary.
xxxvi, 131, 1931.
Callender and Wilder.
251, 1935.
Cardell. Proc. R. S. Med., xxix., 228, 1935.
Cargill and Mayou. T. O. S., xxvii, 149, 1907.
Carter. T. Clin. S., London, vii. 60, 1874.
Chance. Am. J. O., xvii, 48, 1934.
Charamis. A. d’O., xlvii. 833, 1930.
Coats. T. O. S., xxxii, 165, 1902.
R. L. O. H. Rep., xviii, 284, 1912.
Collins. T. O. S., xlvi. 86, 1926; xlix, 166,
1929.
A. of 0., xiv, 967,
Am. J. Cancer, xxv,

DISEASES OF THE UVEAL TRACT
2519
Coppez. A. d’O., xxi, l, 141, 1901.
Davenport. Brit. J. O., xi, 443, 609, 1927.
Dawson. Edin. Med. J., xxxii, 501, 1925.
Déjean. A. d’O., xli., 420, 1924.
Déjean and Varnier. A. S. de Sc. med. biol.
Montpellier, xv., 144, 1934.
Denecke. K. M. Aug., xcvii, 594, 1936.
Derby. K. M. Aug., xli., Beil., 124, 1903.
Donnington. A. of O., xx, 359, 1938.
Duke-Elder and Stallard. Brit. J. O., xiv,
158, 1930.
Evans. Brit. J. O., xxiii, 745, 1939.
Ewetzky. A. f. O., xlii (1), 170, 1896; xlv. (3),
563, 1898.
Fehr. Cb. pr. Aug., xxvi, 129, 1902.
Filatov. A. f. Aug., xciii, 40, 1923.
Fisher and Box. T. O. S., xx, 140, 1900.
Fleischer. B. O. G. Heidel.., xxxv, 343, 1908.
Foot. Am. J. Path., i, 341, 1925; iii, 401,
1927; iv., 525, 1928; viii, 309, 321, 1932.
Francis. Am. J. O., iii, 872, 1920.
Franke. A. f. O., lxx, 332, 1909.
Franz. K. M. Aug., lxiv, 348, 1920.
Freudenthal. A. f. O., xxxvii (1), 137, 1891.
Fuchs, A. K. M. Aug., xcv, 1, 1935.
Fuchs, E. Das Sarcom d. Uvealtractus, Wien,
1882.
B. O. G. Heidel., xxviii, 197, 1900.
A. f. O., lxxvii, 304, 1910; lxxxi, 556, 1912;
xciv, 43, 1917.
Gifford. A. of O., xlvii, 241, 1918.
Ginsberg. A. f. O., lxxxii, 1, 1912.
Golovine. An. d’Oc., czlii, 413, 1909.
Goulden and Stallard. T. O. S., lii, 481, 1932.
v. Graefe, A. A. f. O., xiv. (2), 103, 1868.
Greeves and Holmes. T. O. S., xxxiv, 113,
1914.
Grimsdale. Brit. J. O., xiv., 410, 1930.
Groenouw. A. f. O., xlvii (2), 282, 398, 1899.
v. Grósz. A. f. Aug., c-ci, 236, 1929.
Pianke. A. f. O., xlvii (3), 463, 1899.
Hensius. A. f. O., czzvii, 458, 1931.
v. Hippel. A. f. O., x1 (1), 123; (4), 266, 1894;
cxxiv, 206, 1930 ; exxix, 552, 1933;
cxxxv, 67, 79, 1935 ; cz.xxv, 67, 79,
1936.
Hirschberg. K. M. Aug., vi, 163, 1868;
vii, 65, 1869.
A. f. O., xvi (1), 296, 1870; xxii (1), 135,
1876; xxix (1), 1, 1883.
A. f. Aug., viiii, 189, 1879; x, l, 1880.
Berlin. kl. W., xxx, 1215, 1893.
Holth. K. M. Aug., lxxvi, 510, 1926.
Ischreyt. A. f. O., lxxxi, 220, 1912.
Iwumi. K. M. Aug., xlviii (2), 619, 1910.
Jaensch. K. M. Aug., lxxxiv, 649, 1930;
lxxxviii, 622, 1932.
Ramocki. Z. f. Awg., iii, 32, 1900.
Kayser. K. M. Aug., xli., Beil., 136, 1903.
FCerschbaumer. Das Sarcom d. Auges,
Wiesbaden, 1900.
King. T. O. S., lvii, 557, 1937.
Ripp. T. A. m. O. S., x, 432, 1905.
Klein. K. M. Aug., lxxxiii, 489, 1929.
Wissensch. Woche zu Frankfurt, ii, 39, 1935.
Knapp. A. of O., viii, 82, 1879.
Die intraocularen Geschwälste, Carlsruhe,
1868.
Kopetzky. A. f. O., lii (2), 330, 1901.
Kornfeld. Anat. Anz., liii, 216, 1920–21.
Krompecher. Beit. z. path. Anat., xliv, 51, 88,
1908; lxxii, 163, 1923.
Kronenberg. A. of O., xx, 290, 1938.
Laennec. De l'Auscultation, Paris, 1819.
Lagrange. Tumeurs de l’Oeil, Paris, i, 1901.
Laidlaw. Am. J. Path., viii, 477, 1932.
Laidlaw and Murray. Am. J. Path., ix, 827,
1933.
Lane. A. of O., xii, 980, 1934.
Lange. K. M. Aug., li (2), 537, 1913.
Laven. K. M. Aug., li (2), 493, 1913.
Lawford. R. L. O. H. Rep., xi, 422, 1887.
Lawford and Collins. R. L. O. H. Rep., xiii,
104, 1891.
Lawrence. London Med. Gaz., i, 961, 1845.
Lawson and Parsons. T. O. S., xxv, 257,
1905. .
Leber. A. f. O., xliv (3), 683, 1897.
B. O. G. Heidel.., xxvi, 53, 1897.
Leber and Krahnstäver. A. f. O., xlv., 164,
231, 467, 1898.
Le Brun. Am. d’Oc., ix, 209, 1869.
Li. Am. J. O., vi, 545, 1923.
Lindahl. K. M. Aug., lxv, 11, 1920.
Lukens. Am. J. O., xv, 434, 1932.
Mackay. T. O. S., xxx, 281, 1910.
Margotta. An. di Ott., lix, 416, 1931.
Marshall. R. L. O. H. Rep., xv, 51, 1899.
Masson. A m. d’Amat. Path., iii, 417, 1926.
Am. Surg., xciii, 218, 1931.
Mayou. Brit. J. O., xiv, 152, 1930.
T. O. S., lii, 244, 1932.
Meisner. K. M. Aug., lxx, 722, 1923.
Merrill. K. M. Aug., xci, 598, 1933.
Meyer and Kubik. Am. J. O., xvi, 893, 1933.
Meyerhof. K. M. Aug., xxxix (1), 913, 1901.
Mitvalsky. A. f. Aug., xxviii, 321, 1894.
Moore, Foster. R. L. O. H. Rep., xix, 411,
1914.
Brit. J. O., xiv., 145, 1930.
Mules. T. O. S., viii, 67, 1888.
Neame. Brit. J. O., vi, 493, 537, 1922.
Neame and Kahn. Brit. J. O., ix, 618, 1925.
Neese. Westm. O., l, 1907.
Nettleship. R. L. O. H. Rep., vii, 343, 389,
1872; viii, 264, 1875; ix, 40, 1879.
T. O. S., xxiv, 93, 1904.
Newton. Am. J. O., xxi, 668, 1938.
Palich-Szantò. A. f. Aug., lxxxiv, 118, 1919.
Panas and Rochon-Duvigneaud. Researches
anat. et clim. Sur le Glaucome et les
Néoplasmes intra-oculaires, Paris, 1898.
Parsons. A. f. O., Iv, 350, 1903.
A. of O., xxxiii, 101, 1904.
T. O. S., xxv, 193, 279, 1905.
O. Rev., xxiv, 28, 1905.
Path. of the Eye, London, i, 326, 365; ii,
493, 1905.
Patterson. Am. J. O., xviii, 651, 1935.
Pawel. A. f. O., xlix (1), 71, 1899.
Pindikowski. K. M. Aug., liii, 516, 1914,
Prevec. K. M. Aug., xcix, 513, 1937.
2520
TEXT-BOOK OF OPHTHALMOLOGY
Purtscher. Cb. pr. Aug., xxx, 139, 1906.
v. Recklinghausen. Ueber d. multiplen
Fibrome d. Haut, Berlin, 1882.
Reese. A. of O., ix, 560, 1933.
Reis. Z. f. Aug., xxviii, 426, 1912.
Ribbert. Beit. 2. path. Amat., xxi, 471, 1898.
Geschwillste Bonn, 1904.
Lhb. alg. Path. u. path. Anat., iv, 262, 1911.
Robertson, Argyll and Knapp. A. of O.,
iii (2), 106, 1873.
Rochat. K. M. Aug., lxxvi, 651, 1926.
Rönne. B. O. G. Heidel., xlvii, 241, 1928.
T. O. S., lvi, 270, 1936.
Rosenbaum. Am. J. O., xxi, 1360, 1938.
Ruben. A. f. O., lxxxi, 199, 1912.
St. John Roosa. T. Am. O. S., i, 14, 1869.
Salzer. Münch. med. W., lxviii (1), 203, 1921.
Samuels. T. O. S., xlix, 421, 1929 ; liii, 520,
1933.
A. of O., xi, 998, 1934.
Schultz-Zehden. K. M. Aug., xliii (2), 150,
1905.
Sédan. A. d’O., lii, 595, 1935.
Sédan, Ourgand and Morenon.
Paris, xlvi, 623, 1934.
Shine. N.Y. Eye and Ear Infirmary, Clim.
Rep., i, 31, 1930.
Shoemaker and de Long.
Wiscon., p. 146, 1926.
Sichel. An... d’Oc., xxvi, 1851.
Smith, Priestley. On the Path. and Treatment
Bull. S. d’O.
Contrib. O. Sc.,
Steiner. K. M. Aug., lxix., 266, 1922.
Susman. Brit. J. O., xxii, 722, 1938.
Szymanski. Bull. S. fr. d’O., xli., 38, 1928.
Tay. R. L. O. H. Rep., v, 227, 1866.
Terry. A. of O., xix, 989, 1939.
Terry and Johns. Am. J. O., xviii, 903, 1935.
Theobald. A. of O., xviii, 971, 1937.
Unna. Berl. kl. W., xxx, 14, 1893.
Valentine. Brit. J. O., i, 540, 1917.
T. O. S., xxxvii, 229, 1917.
Vancea. Cluj. Med., xi, 16, 1930.
A. d’O., xlviii, 43, 1931.
Velhagen. K. M. Aug., lxiv, 252, 1920.
Verhoeff. A. of O., xxxiii, 241, 1904.
Virchow. Die krankhafter Geschwälste,
Berlin, 1863.
Wagenmann. Deut. med. W., 262, 1900.
B. O. G. Heidel., 1, 303, 1934.
Wätzold. A. f. O., exxiv, 139, 1930.
Wätzold and Gyotoku. A. f. O., c2(x, 209,
1928.
Werther. A. f. Aug., xxxii, 297, 1896.
Wescott. T. A. m. O. S., xiii, 183, 1912.
Weve. T. O. S., lix, 43, 1939.
Wilder. A m. J. Path., xi. 817, 1935.
Williams and Knapp. A. f. Aug., iv, S3, 1874.
Williamson-Noble. T. O. S., xlv, 251, 1925.
Wintersteiner. Enzyklopädie d. A ugenheilk'.,
Leipzig, 1902.
Z. f. Aug., xix, 388, 1908.
A. f. O., lxix., 75, 1908.
of Glaucoma. London. 1891. Wolfrum. A. f. O., lxxi, 195, 1909.
Solomon. T. O. S., ii, 257, 1882. Wood and Pusey. A. of O., xxxi, 323, 1902.
Speciale-Cirincione. An. di Ott., liii, 849, 1925. Zentmayer. A. of O., v, 219, 1931.
Spicer. T. O. S., xxxii, 245, 1912.
DERMOID
A unique case of a dermoid in the choroid was described by Follin (1861):
it occurred in a woman aged 70, and its epithelial wall, which contained hair-
follicles, enclosed a mass of dense connective tissue with fat cells.
Follin.
Bull. S. de Chir. Paris, ii, 1 16, 1861.
II. Secondary Tumours
A. By Direct Eartension
The extension of epibulbar tumours through the sclera to involve the
uveal tract is rare ; but such an occurrence may be met with both in
carcinomata and sarcomata.
1. EPITHELIOMA
The dense tissues of the cornea and sclera prevent the intra-ocular
spread of epibulbar carcinomata for a considerable time.
It will be remem-
DISEASES OF THE UVEAL TRACT 2521
bered that the great majority of them have their origin at the limbus;
thence they may infiltrate the cornea, reach Schlemm's canal, and from this
point involve the interior of the eye (Whitehead, 1906) (Fig. 2132). Most
usually, however, entry occurs by pushing columns of cells through the
scleral trabeculae and along the peri-vascular and peri-neural lymphatics
at the limbus (Heyder, 1887; Parsons, 1904; Collins, 1915; Fuchs, 1916;
Sattler, 1921; Michail, 1922; Beauvieux and Pesme, 1923; and others).
Fig. 2132.-INTRA-ocular SPREAD of AN EPIBULBAR EPITHELIoMA.
Note the tumour of the iris and the neoplastic replacement of the ciliary body
(Whitehead, T. O. S.).
From this point the iris and ciliary body become involved, or the supra-
choroidal space is invaded and the choroid infiltrated ; while in either case,
as occurs in other lymph spaces, cells may become detached and set up new
intra-ocular metastatic foci.
Reis (1903) described a large orbital carcinoma which surrounded the greater
part of the posterior half of the globe, in association with which a metastatic adeno-
carcinoma extended almost throughout the choroid.
In such cases the clinical appearance, symptoms, and treatment are dominated
by the parent extra-bulbar tumour.
Beauvieux and Pesme. A. d’O., x1, 233, Michall. Cluj. Med., iii. 222, 1922.
1923. Parsons. Pathology of the Eye, i, 145, 1904.
Collins. T. O. S., xxxv, 215, 1915. Reis. K. M. Aug., xli (2), 416, 1903.
Fuchs. K. M. Aug., lvii. 1, 1916. Sattler. A. f. O., ev. 1207, 1921.
Heyder. A. f. Aug., xvii, 294, 1887. Whitehead. T. O. S., xxvi, 58, 1906.
* Vol. II, p. 1872.

2522 TEXT-BOOK OF OPHTHALMOLOGY
2. SARCOMA
Penetration of the globe by epibulbar sarcomata is rare, and only a few cases
are to be found in the literature. Kolaczek (1880) reported a case wherein the entire
globe was filled with sarcomatous tissue. Adamūk (1881) described a small penetration
near the equator. v. Michel (1899) noted a sarcoma extending from the equator to
the cornea which had invaded the choroid and iris throughout. Kerschbaumer (1900)
recorded 3 cases, in 1 of which a round-celled sarcoma at the limbus penetrated to the
canal of Schlemm and the pectinate ligament and thence had invaded the ciliary body
and iris ; and in the other 2 the globes had collapsed. Verhoeff and Loring (1903)
described a spindle-celled sarcoma which started as a small red spot above the cornea
and 2 years later there was a diffuse neoplastic involvement of the iris, ciliary body
and choroid as far back as the disc.
Adamük. A. f. Aug., xi, 19, 1881. v. Michel. IX Internat. Cong., Utrecht, 1899.
Kerschbaumer. Das Sarcom des Auges, Verhoeff and Loring. A. of O., xxxii, 97,
Wiesbaden, 1900. 1903.
Kolaczek. D. Z. f. Chºir., xii, 75, 1880.
3. MELANOMA
A most interesting case was observed by Mulock Houwer (1926) in which a large
pigmented tumour was adherent to the posterior part of the eye over an extensive
area ; it had multiple intra-ocular extensions all associated with the posterior ciliary
nerves, along which ran strands of tumour tissue. It formed a beautiful example of
the association of such tumours with ciliary nerves.
Houwer, Mulock. T. O. S., xlvi, 122, 1926.
4. NEURO-BLASTOMA
It is a commonplace for a neuro-blastoma of the retina to invade the choroid,
ciliary body or iris, a matter which will be discussed in connection with these tumours.”
5. END OTHELIOMA
Wintersteiner (1899) reported a case in which an endothelioma of the optic nerve
appeared to have given rise to extensions of a similar neoplastic nature in the choroid ;
while Cushing and Eisenhardt (1938) found a meningioma in a girl of 7 years of age
which had penetrated from the optic nerve into the choroid.”
Cushing and Eisenhardt. Meningiomas, Ill., Wintersteiner. Verhandl. LXXI, Vers. dtsch.
287, 1938. Naturf. u. Aerzte in München, ii (2), 330,
1899.
B. Metastatic Tumours
1. CARCINOMA
Although carcinomata are the most common secondary tumours affecting
the inner eye, nevertheless their occurrence is rare. This is probably due
1 p. 2816. * p. 3089.
DISEASES OF THE UVEAL TRACT 2523
to the fact that the metastases are blood-borne (Fig. 2138), and since the
ophthalmic artery leaves the internal carotid at right angles, it is not
readily entered ; it is easier for malignant emboli coming by this route to
travel straight on and lodge themselves in the minute circulation of the
brain and meninges. It is possible, however, that such deposits are con-
siderably more frequent than the literature would indicate, for in most of
the cases the patient is already gravely ill, sometimes more or less moribund,
and the clinical picture is dominated by his general condition while he
himself does not notice what may be a minor defect in one eye moreover,
uveal metastases are of slower growth than those in the brain and elsewhere,
so that the patient may well have died before attention is attracted to the
Ocular condition, and routine ophthalmoscopic or pathological examination
of the eye is usually omitted in such cases of terminal carcinomatosis.
Few surgeons have recorded more than 1 case—Fehr (1918), Greenwood (1922),
and Usher (1923–26) have recorded 4 each. Uhthoff (1904) gave a ratio of 1 : 30,000
patients; Sattler (1926) 2 : 300,000 ; Payne (1932) 2: 70,000 ; Suker (1932) 1 :
100,000; and Stallard (1933) in the Moorfields records 1 : 140,000. Michaïl (1932)
reports 1 case in 48,000 eyes examined histologically.
The first case recorded in the literature was that of Perls (1872) of a man of 43
who had a choroidal carcinoma secondary to a primary growth in the lung. Hirschberg
(1882) followed with a case of carcinoma of the breast ; and thereafter many reports
have appeared. The subsequent literature has been collated and reviewed by several
authors—Marshall (1897) who collected 24 cases; Parsons (1903) 33 cases, and (1905)
50 cases ; Krukenberg (1903) 37 cases ; Suker and Grosvenor (1909) 62 cases; Steichele
(1919), Ullmann (1921) 66 cases; Behr (1922); Usher (1923) 110 cases; Clapp (1926)
118 cases ; de Long (1933) 135 cases ; Hudson (1933) 149 cases ; Ask (1934) 211 cases,
in 59 of which the diagnosis was not histologically proved ; and Lemoine and McLeod
(1936) 229 cases, of which 156 were histologically proved. Résumés of all the individual
cases were made by Usher (1923), Ask (1934) and Lemoine and McLeod (1936).
The site of the tumour is of considerable importance. As one would
expect, the vast majority of emboli travel up the 20 odd short posterior
ciliary arteries rather than the 2 long posterior or the 5 anterior
arteries; the posterior region of the choroid is therefore the site of election,
more especially its temporal side near the macula where the short
ciliary arteries are most numerous and largest, while involvement of the
ciliary body and iris is rare. Moreover, since such emboli are usually
liberated in numbers, multiple tumours are not uncommon, and in 20.8%
of the cases both eyes are affected, not usually simultaneously, but one
following the other ; simultaneous involvement of the brain is common.
Moreover, the left eye is more commonly affected than the right (13 out of
21 cases, Parsons, 1905), an incidence comparable to the great frequency of
left-sided cerebral and ocular embolism and doubtless due to the more direct
pathway by the left carotid artery.
Involvement of the anterior segment of the uveal tract is thus rare :
Usher (1923) could collect only 8 cases (Ewing, 1890; Lagrange, 1901;
2524 TEXT-BOOK OF OPHTHALMOLOGY
Briehn, 1902; Cutler, 1903 ; Paul, 1905; Chiarini, 1907 ; Proctor and
Verhoeff, 1907; Toulant, 1916); Ask (1934) brought the total in the
literature up to 23 ; and Sanders (1938) to 27, of which 17 were micro-
scopically proved to be metastatic carcinomata. Involvement of the ciliary
body alone is very rare (Knapp, 1929, in a carcinoma of the breast ; Orr and
Johnstone, 1935, of the thyroid); while there are only four cases wherein
the growth has been shown histologically to be limited to the iris only
(Proctor and Verhoeff, 1907, carcinoma of the breast ; Bollack, Bertillon
and Roques, 1927, of the breast ; Larsen, 1933, of the oesophagus ; and
Sanders, 1938, in which the primary site was not determined). In Toulant's
(1916) case, which was not histologically investigated, the involvement of
the iris was bilateral.
The site of the primary growth in the vast majority of cases is the female
breast (65%, Hegner, 1911; 65 out of 90 cases in both sexes, and 62 out of
71 cases in females, Usher, 1923; 50% of cases, Lemoine and McLeod, 1936);
a unique case secondary to carcinoma of the male breast was reported by
Giri (1939). The second most common site is the lungs (about 10% of cases),
then the alimentary tract (about 7%), and thereafter the thyroid and
the liver. Exceptional sites are the prostate (Greenwood and Southard,
1903), ovary (Holden and Rusk, 1904), and parotid (Dimissianos, 1932).
It is somewhat difficult to account for the preponderance of breast
tumours in the aetiology, since, while they are responsible for over 50% of
uveal metastases, their general incidence is only 13.5%, while carcinoma of
the stomach with a relative frequency of 36.5% gives rise only to 3.3% of
uveal metastases. Ask (1934) suggested that this was due to a varying
predilection for haematogenous metastases in the primary growth, a factor
presumed to be high in breast tumours ; but it is probable also that the
uvea provides more suitable nutritional conditions for some tumour-cells
than for others.
Owing to the preponderance of breast cancer as the causal lesion,
metastatic tumours are much more common in females than in males
(73 females to 29 males, Usher, 1923). Most cases occur from 40–50 years of
age, and below 30 and above 70 they are rare.
While intra-ocular metastases usually occur as part of a general dis-
semination of the disease, they not infrequently occur quite early in its
evolution ; indeed, many cases have been reported even within compara-
tively recent years, wherein the visual disturbance was the first symptom
noticed by the patient, and has led to a systemic examination in search of
the parent growth (Arisawa, 1914; Usher, 1923; Dimissianos, 1932;
Michail, 1932; Moore and Stallard, 1932; Siegrist and Cramer, 1933;
Sanders, 1938; and many others). The importance of diagnosing these
ocular tumours is therefore obvious. The most common sites wherein a
primary growth may be latent in this way are the intestinal canal, the lungs,
and the thyroid (Orr and Johnstone, 1935).
DISEASES OF THE UVEAL TRACT 2525
Venco (1935), reviewing the literature of 6 cases of metastatic adeno-carcinomata
of the thyroid, drew attention to this difficulty and pointed out the slow course of the
metastases which may be delayed 10 or 15 years after an operative attempt to remove
the original tumour.
Clinical Course
The typical ophthalmoscopic appearance of a metastatic carcinoma of
the choroid is somewhat distinctive; indeed it is quite frequently diagnosable
Fig. 2133.-METAstATIC CARCINoMA of THE CHoRoid.
From an adeno-carcinoma of the breast (Evans, Brit. J. O.).
from its appearance alone. In contra-distinction to the usual form of a
malignant melanoma, it is rarely circumscribed but rather appears as a
flat thickening of the choroid, usually thickest at the posterior pole and
thinning off anteriorly, over which there is a shallow retinal detachment
(Figs. 2133–35). The swelling is of a pale grey colour, the surface usually
displays a grey mottling, and the edges are not sharply defined. Small
haemorrhages are not uncommon on its surface, or vessels of new formation,
but the vitreous is rarely disturbed. Increase of growth occurs rapidly,
more rapidly than in a flat melanoma, glaucoma is late in appearing, but
pain is earlier and more pronounced than in primary tumours and frequently

2526 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2134-METAstatic CARCINoMA or THE Choroid : EARLY STAGE.
From the lung (Foster Moore and Stallard, Brit. J. O.).
involves the necessity of enucleation. At any time, of course, a large retinal
detachment may occur obscuring the details of the clinical picture, and its
occurrence, particularly its bilateral occurrence, in a carcinomatous patient
is always suggestive.
Fig. 2135-METAstatic CARCINoMA of the Choroid : L'Are Stage.
Showing involvement of the choroid, selera and retro-bulbar tissue in a metastasis
from the breast (Cohen, A. of 0.).


DISEASES OF THE UVEAL TRACT 2527
O
ºne ºwn
- - - - -º
FIG. 2136.-METAstATIC CARCINoMA or CILIARY Body.
From a carcinoma of the thyroid. A. Retina. B. Sub-choroidal haemorrhage.
C. Ciliary process (Orr and Johnstone, Brit. J. O.).
A metastatic carcinoma of the ciliary body is a pathological curiosity
(Fig. 2136); there are no diagnostic features which distinguish it from other
types of neoplasm in this region.
A metastatic carcinoma of the iris appears as a small shallow swelling of
this tissue, or a circular or oval nodule, or an irregular raised mass, but is
-
Fig. 2137.-METASTATIc CARCINoMA of IRIs.
The neoplasm has filled half the anterior chamber and invaded the cornea
(Sanders, Am. J. O.).
T.O.-WOL. III.
F. F.


2528 TEXT-BOOK OF OPHTHALMOLOGY
usually fairly sharply defined (Fig. 2137). The colour is variable—dull
grey, reddish, or greyish-brown—and frequently it is vascular. It may
appearin any quadrant, or in any part—the pupillary area, the intermediate
zone, or the root; as a rule, except at the site of the tumour, the iris retains
its normal appearance and its mobility. In all types, choroidal, ciliary and
iridic, signs of inflammation are usually lacking, but evidences of irido-
cyclitis may be present which are liable to suggest an erroneous diagnosis of
syphilis or tuberculosis.
The tumour starts from a malignant embolus arrested in the small
vessels (Fig. 2138); the vessel is blocked, the cells proliferate and burst
Fig. 2138-CARoisonia or Choroid.
Metastatic deposits of carcinoma cells from a carcinoma of the breast in an
enlarged choroidal vessel (Cohen, A. of 0.).
through, infiltrating the perivascular spaces. Such emboli in the posterior
ciliary arteries were seen by Schultze (1890), Abelsdorff (1896) and Cohen
(1937), and in the anterior arteries by Lagrange (1901). In a case described
by Smoleroff and Agatston (1935) the infiltration around the vessels in the
scleral canal was continuous with the main mass in the eye. Rarely
miliary metastatic deposits have been found in the chorio-capillaris, as
in a bilateral case secondary to a bronchial carcinoma in which, in addition
to the intra-ocular growths, multiple deposits in each orbit led to
exophthalmos (Kreibig, 1931). Having reached the perivascular
spaces, the tumour infiltrates between the planes of the choroidal
stroma, taking the path of least resistance; and for this reason
most of the growth is lateral with a minimum of increase in thickness.
Bruch's membrane is thus usually left intact and the overlying retina

DISEASES OF THE UVEAL TRACT 2529
uninvolved, although it may suffer degenerative changes; occasionally,
however, it is invaded from the disc (Lagrange, 1898; Oatman, 1903;
Oeller, 1905; Gelpke, 1905; Steichele, 1919). The infiltration is essentially
lateral and it may be extremely widespread involving the iris and ciliary
body and the angle of the anterior chamber; out-lying nodules are also
frequent, which may sometimes be separate primary metastases, or may
Fig. 2139.-CHoRoidAL METAstasis FROM A CARCINoMA of THE BREAST.
The choroidal tumour resembles a carcinoma mammae. The same tumour as in
Figs. 2135 and 2138 : for detail of part A see Fig. 2140 (Cohen, A. of 0.).
be local metastases carried along the tissue spaces. The ciliary body and
iris may indeed be found on microscopical examination to be heavily
infiltrated without any clinical evidence of the process (Brewitt, 1903;
v. Hippel, 1911; Ishihara, 1914). The sclera is sometimes eroded, but extra-
ocular spread is relatively uncommon, probably because death has usually
supervened before this can occur; several cases are, however, on record
(Oeller, 1907; Lafon, 1908; Usher, 1923; Cohen, 1937; and others).

F E 2
25.30 TEXT-BOOK OF OPHTHALMOLOGY
Extension up to the optic nerve may also occur, infiltration taking place
by way of the lymph paths into the vaginal and perivascular spaces where
the neoplasm proliferates, destroying the nerve fibres and occasionally
replacing them entirely with new-growth (Schöler, 1883; v. Michel, 1905;
MacMillan, 1922; Usher, 1923; Davis, 1932; McDannald and Payne,
1934). The nerve itself does not seem to be readily attacked by direct
continuity even if the tumour is situated closely around the optic disc
(Parsons, 1905; Benedict, 1932).
|- - -- - *
*** ****
-
Fig. 21.40.-Choroid AL METAstasis From A CARCINoMA or THE BREAst.
The part marked A in Fig. 2139 showing a cellular nest (Cohen, A. of 0.).
Histology. The histology of the tumour depends upon the nature of
the primary growth but varies considerably independently of it. In tumours
secondary to carcinoma of the breast, the neoplasm usually consists of alveoli
of various sizes containing large round or polygonal cells with single large
nuclei; sometimes the stroma between the alveoli is sparse, and at other
times dense masses of fibrous tissue are present (Figs. 2138–39). The
choroidal tissue is compressed and degenerated and the chromatophores
disintegrated, the escaped pigment massing into clumps.
When the tumour is derived from glandular tissue (stomach, bowel,
liver, thyroid) or from the lungs it is adeno-carcinomatous in type, with the
character of a papilliferous alveolar carcinoma, the papillae consisting of
columnar or cubical cells in a single row supported by a sparse stroma
disposed in branching trabeculae projecting into the alveoli (Figs. 2141–42).



DISEASES OF THE UVEAL TRACT 2531
Fig. 2141.-CHoRoidAL METASTAsis of A CARCINoMA of THE STOMACH.
Showing a papilliferous alveolar structure (Usher, Brit. J. O.).
Fig. 2142.-CHoRoipal METAstasis of A CARCINoMA of THE LUNG.
A. Adeno-papilliferous arrangement of carcinoma cells. B. Area of cell
degeneration. C. Connective tissue stroma. D. Hemorrhage (Foster Moore
and Stallard, Brit. J. O.).


2532 TEXT-BOOK OF OPHTHALMOLOGY
Some of the cubical cells are usually arranged in irregular clumps and
infiltrate the spaces of the choroid.
Degenerative changes are common and frequently well marked—vacuolation of
the cells, hyaline degeneration, and necrosis ; while multiple haemorrhages are the
rule.
The prognosis in these cases is invariably bad ; they always end fatally,
usually within a short time, owing to metastases. Usher (1923), in his
review of 110 cases, found that the average duration of life after the ocular
lesion has been noted was 8 months, the longest interval being 2 years and
the shortest 4 weeks. The great majority of patients, indeed, are already
cachectic, and multiple metastases co-exist in many organs of the body.
The treatment is therefore purely palliative, and is unnecessary unless the
eye is giving rise to pain. In this event enucleation is usually performed,
although relief may be obtained by radiation which may produce a cessation
of activity and necrosis, a possibility demonstrated histologically post-
mortem by Lemoine and McLeod (1936).
Abelsdorff. A. f. Aug., xxxiii, 34, 1896.
Arisawa. K. M. Aug., lii, 659, 1914.
Ask. Acta O., xii, 308, 1934. .
Behr. K. M. Aug., lxix., 788, 1922.
Benedict. A. of O., viii, 232, 1932.
Bollack, Bertillon and Roques. An. d’Oc.,
clxiv, 336, 1927.
Brewitt. Diss., Göttingen, 1903.
Briehn. Diss., Königsberg, 1902.
Chiarini. Boll. R. Accad. Med. Roma, xxxiii,
5, 1907.
Clapp. Am. J. O., ix, 513, 1926.
Cohen. A. of O., xviii, 604, 1937.
Cutler. T. Am. O. S., x, 451, 1903.
Davis. A. of O., viii, 226, 1932.
De Long. Am. J. O., xvi, 712, 1933.
Dimissianos. K. M. Aug., lxxxix, 356, 1932.
Ewing. A. f. O., xxxvii, 120, 1890.
Fehr. K. M. Aug., lx, 741, 1918.
Gelpke. K. M. Aug., xliii (1), 492, 1905.
Giri. Schw. med. W., lxix, 319, 1939.
Goldstein and Wexler. A. of O., xiii, 207,
1935.
Greenwood. Am. J. O., v, 912, 1922.
Greenwood and Southard. Boston Med. and
Surg. J., czlix, 286, 287, 1903.
Hagiwara. Acta S. O. Jap., xxxvi, 138,
1932.
Hegner. K. M. Aug., xlix (2), 31, 1911.
v. Hippel. K. M. Aug., xlix (1), 743, 1911.
Hirsch. Prag. med. W., xxxvi, 49, 1911.
Hirschberg. Cb. pr. Aug., vi, 376, 1882.
Holden and Rusk. A. of O., xxxiii, 322,
1904.
Hudson. P. R. S. Med., xxvii, 304, 1933.
Ishihara. K. M. Aug., liii, 127, 1914.
Knapp. A. of O., i, 604, 1929.
Kreibig. Z. f. Aug., lxxiv, 362, 1931.
Krukenberg. K. M. Aug., xli., Beil., 145,
1903.
Lafon. Rec. d’O., xxx, 193, 1908.
Lagrange. A. d’O., xviii, 30, 1898.
Tumeurs de l’Oeil, Paris, 1901.
Larsen. Acta O., xi, 386, 1933.
Lemoine and McLeod. A. of O., xvi, 804,
1936.
MacMillan. A. of O., li, 227, 1922.
Marshall. R. L. O. H. Rep., xiv., 415, 1897.
McDannald and Payne. A.
1934.
Micháil. An. d’Oc., clzix, l 19, 1932.
v. Michel. Z. f. Aug., xiv. 421, 1905.
Moore and Stallard. Brit. J. O., xvi, 532,
1932.
Oatman. Am J. Med. Sc., cli, 375, 1903.
Oeller. A. f. Aug., lii, 121, 1905.
Orr and Johnstone. Brit. J. O., xix,
1935.
Parsons. R. L. O. H. Rep., xv, 286, 1903.
Pathology of the Eye, ii, 533, 1905.
Paul. A. f. Aug., liii, 1, 1905.
Payne. Teacas State J. Med., xxviii, 277,
1932.
Perls. A. f. path. Anat., lvi, 437, 1872.
Proctor and Verhoeff. A. of O., xxxvi, 47,
1907.
Sanders. Am. J. O., xxi, 646, 1938.
Sattler. Die bósartigen Geschwillste d. Auges,
Leipzig, 1926.
of O., xii, 86,
593,
Schöler. Cb. pr. Aug., vii, 236, 1883.
Schultze. A. f. Aug., xxi, 319, 1890; xxvi,
19, 1893.
Siegrist and Cramer. Am... d’Oc., clz.x, 116,
1933.
Smoleroff and Agatston. A. of O., xiv, 809,
1935.
Stallard. P. R. S. Med., xxvi, 1042, 1933.
Steichele. A. f. Aug., lxxxiv, 201, 1919.
Suker. A. of O., viii, 232, 1932.
DISEASES OF THE UVEAL TRACT 25.33
Suker and Grosvenor. Ophthalmology, v, Ullmann. Diss., Heidel., 1921.
580, 1909. Usher. Brit. J. O., vii, 10, 1923; x, 180,
Toulant. A. d’O., xxxv, 44, 1916. 1926.
Uhthoff. D. med. W., xxx, 1423, 1904. Venco. An. di Ott., lxiii, 401, 1935.
B. O. G. Heidel., xxxiii, 143, 1906.
2. HYPERNEPHRoma
A metastatic hypernephroma in the eye is a rarity. A bilateral case was recorded
by Chance (1906) affecting the iris and ciliary body, forming discrete tumours which
were clinically diagnosed as sarcomata. Stock (1923) reported bilateral uveal
metastases in the choroid and ciliary body; choroidal tumours have been recorded
METASTATIC HYPERNEPHRow A of IRIs (Hudson and Lister).
FIG. 2143.
by Small (1908), Hird (1921) and Kreibig (1931), while Ginsberg (1928) found
bilateral intra-vascular aggregations of cells as well as extra-vascular tumours in the
choroidal tissues. Hudson and Lister (1934) also described a nodular, yellow, vas-
cularized new-growth on the iris which on section proved to be a hypernephroma: it
was associated with widespread disorganization and glycogenic infiltration of the
tissues and signs of irido-cyclitis (Fig. 2143). Histologically the cells are seen to be
large and angular with a tendency to become columnar and assume a palisade arrange-
ment on the surface (Fig. 2144).
Chance. T. Am. O. S., xi, 178, 1906. Hudson and Lister. P. R. S. Med., xxvii,
Ginsberg. HB. spez. Path. Amat. Histol., 1613, 1934.
xi (1), 567, 1928. Kreibig. Z. f. Aug., lxxv, 326, 1931.
Hird. T. O. S., xli., 457, 1921. Small. O. Rec., xvii. 55, 1908.
Stock. K. M. Aug., lxxviii, Beil. 116, 1923.

2534 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2144.—METAstATIC HYPERNEPHRoma of IRIs.
The tumour of Fig. 2143 showing elongated columnar cells arranged in palisade
formation on the surface and polyhedral cells elsewhere (Hudson and Lister).
3. SAR.com.A
Metastatic sarcomata in the choroid are great rarities, but several reports have
appeared in the literature. Two of the cases were considered to arise from round-
celled sarcomata of the mediastinum which had given rise to widespread metastases
affecting the brain and internal organs (Meigs and de Schweinitz, 1894; Wiener, 1902):
in each case the uveal involvement was multiple and bilateral. It is possible that they
may have been malignant lymphomata. Neese (1907) described a choroidal tumour
as an alveolar sarcoma with round and spindle cells which he considered to be
secondary to a sarcoma of the breast (cysto-sarcoma proliferans phyllodes). Finally,
Elschnig (1926) described a spindle-celled sarcoma of the iris in a woman who died of
general metastases from a primary spindle-celled sarcoma of the ovary.
Elschnig, H. H. A. f. O., exvii, 316, 1926. Neese. Westm. O., 1, 1907.
Meigs and de Schweinitz. Am. J. Med. Sc., Wiener. J. Am. Med. As., xxxix., 1158,
cviii, 193, 1894. 1902.
4. MALIGNANT MELANoMA
Many authorities state that secondary malignant melanomata do not occur in
the uveal tract (Fuchs, 1882; Parsons, 1905). If they do occur they are exceptionally
rare, a fact which seems surprising in view of the widespread character of a generalized
melanomatosis, but conforms to Virchow's (1863) dictum that “ organs which
exhibit a marked tendency to protopathic tumour formation present a very slight
inclination to metastatic deposits.” There are, however, cases wherein a naevus of
the skin has been observed for a long time and then, on suddenly becoming malignant,
has given rise to a widespread melanomatosis, involving the internal organs and the

DISEASES OF THE UVEAL TRACT 2535
central nervous system, in the midst of which a uveal melanoma has become obvious
(Pflüger, 1885; ten Doesschate, 1919; Cordes and Horner, 1930; Corrado, 1931;
Fry, 1933); the obvious deduction from them is that the general dissemination had
involved the eye. The early case of Brömser (1870) may be more equivocal; but the
occurrence of multiple nodules in one case (Fry, 1933), and the appearance in another
of a tumour in the optic nerve-head (Schiess-Gemuseus and Roth, 1879), a site not in
keeping with a primary choroidal neoplasm, support the contention; while in a
third the metastases were bilateral, associated with nodules in the optic nerve as well
as in the brain and all the organs in the body except the liver and spleen (Cordes and
Horner, 1930). A further case described by Adamiik (1909) may be noted, wherein,
after an epi-bulbar and palpebral melanoma had been excised together with the eye,
the excision was followed by a general melanomatosis of the skin and the development
of a melanoma in the ciliary body of the other eye : it may be suggested that if such a
tumour had appeared in any other organ it would have been considered a secondary
manifestation. At the same time it is impossible to prove that the ocular tumour was
not the primary one, or that two separate navi had not synchronously assumed
malignant characteristics.
Adamūk. Z. f. Aug., xxi, 505, 1909. Fuchs. Das Sarcom d. Uvealtractus, Wien,
Brömser. Diss., Berlin, 1870. 1882.
Cordes and Horner. J. Am. Med. As., xev, Parsons. Pathology of the Eye, ii, 533, 1905.
655, 1930. Pflüger. A. f. Aug., xiv., 129, 1885.
Corrado. A. di Ott., xxxviii, 508, 1931. Schiess-Gemuseus and Roth. A. f. O.,
ten Doesschate. Ned. Tij. v. Gen., ii, 1432, xxv (2), 177, 1879.
1919. Virchow. Die Krankhaften Geschwälste,
Fry. A. of O., ix, 248, 1933. Berlin, 1863.
5. CHORION-EPITHELIOMA
Metastases of chorion-epitheliomata in the eye are exceptional. A case has been
reported in a woman by Simidu (1935); and cases in men by MacDonald (1936) and
Reichling (1938) who had teratomatous mixed tumours of the testicle. In MacDonald's
case there was marked endocrine disturbances and female characteristics, and part of
Fig. 2145.-Chorios-EPITHELIoMA of CHoRoid (MacDonald, A. of 0.).

2536 TEXT-BOOK OF OPHTHALMOLOGY
the testicular tumour was a carcinoma and part a typical chorion-epithelioma (Fig.
2145). All the metastases, including a massive but circumscribed choroidal tumour
which necessitated enucleation of the eye because of pain before death, were of the
Fig. 2146.-CHorton-EPITHELIoMA or Choroid.
The tumour of Fig. 2145 showing syncytial masses (Sy) with Langhan's cells
surrounding blood-containing spaces (MacDonald, A. of 0.).
chorion-epitheliomatous type with the typical polyhedral cells and large nucleated
syncytial cells (Fig. 2146).
MacDonald. A. of 0., xvi, 672, 1936. Simidu. Acta O. S. Jap., xxxix., 2023, 1935.
Reichling. A. of O., xix, 156, 1938.
6. TESTICULAR ADENo-CARCINoMA
A case of intra-ocular metastasis of an adeno-carcinoma of the testis in a child
of 4 who died of general metastases was reported by Goldstein and Wexler (1935).
This embryonal tumour of a teratoid nature is distinguished by its rapid growth. It
was, like chorion-epitheliomata, circumscribed rather than infiltrating, but was limited
to the supra-choroidea. It did not involve the choroid proper which was detached
over it, but growth proceeded in the direction of the sclera, while widespread necrosis
of its outer portion led to symptoms of a deep scleritis which had originally called
attention to the condition. The tumour itself was composed of small groups of deeply
staining polyhedral cells separated by cellular connective tissue.
Goldstein and Wexler. A. of 0., xiii, 207, 1935.

DISEASES OF THE UVEAL TRACT 2537
VIII. DETACHMENTS AND DISPLACEMENTS OF THE UVEAL TRACT
Detachments and displacements of portions of the uveal tract are not
uncommon, but in most cases they are only of incidental interest.
A DISPLACEMENT OF THE IRIS FORWARDS is seen in many hypermetropic
eyes especially in advanced age ; the condition is due to its being pushed
forwards by the relatively large lens and the hypertrophied ciliary body,"
a displacement which is important since it inclines to the formation of
anterior peripheral synechiae and the development of glaucoma.” In
pathological states a similar condition may occur more acutely, the iris being
pushed forward by an intumescent cataract, a tumour in the ciliary body,
or a herniation of the vitreous gel through a traumatically ruptured zonule.
In cases of perforation of the cornea, either traumatic, ulcerative, or opera-
tive, the loss of the anterior chamber always allows the structures behind the
iris to press it forward owing to uncompensated pressure, while at the time
of perforation the escaping aqueous may carry the iris with it into the
corneal wound or outside of the globe altogether to form an anterior synechia
or a prolapse of the iris & ; eventually the iris may be inextricably fused
with the cornea in a scar forming an adherent leucoma which, if it becomes
ectatic, may develop into an anterior staphyloma 4 (see Fuchs, 1918). The
condition of iris bombé, when in the presence of complete ring-synechiae the
accumulated pressure in the posterior chamber bulges the iris forwards, has
already been considered.” -
A DISPLACEMENT OF THE IRIS BACKWARDs with a consequent deep
anterior chamber may occur normally in long myopic eyes. It also follows
a perforation or rupture of the posterior segment of the globe involving a
loss of vitreous ; while a luxation of the lens has a similar effect. Such
backward displacement may result in a complete inversion of the iris in
which case it is folded backwards against the ciliary body. The anatomy of
this condition has been described by Groenouw (1919) and Fuchs (1925); it
is a traumatic effect following rupture of the posterior segment of the globe
or, more frequently, a cataract operation wherein vitreous has escaped into
the anterior chamber. Clinically its appearance resembles a coloboma of
the iris, but the continuous structure of the iris as well as the absence of cut
margins make the diagnosis clear. No treatment is required or should be
attempted.
DETACHMENTS OF THE IRIs always occur at its root at the junction with
the ciliary body; if localized they are termed IRIDO-DIALYSEs, and since such
lesions are essentially due to trauma they will be considered elsewhere." It
may be noted here, however, that the accident has been said to occur after
strong contraction of the pupil due to sudden exposure to a very bright light
(Mandicevski, 1935). It may occur apparently spontaneously (Perlia, 1912)
1 p. 2393. 2 p. 3359. 8 Vol. II, p. 1819.
* Vol. II, p. 1825. 5 p. 2168. 6 Vol. IV.
2.538 TEXT-BOOK OF OPHTHALMOLOGY
or in conditions of advanced atrophy.” A total detachment, or TRAUMATIC
ANIRIDIA, is rare and may be associated with an extensive rupture of the
sclera.”
A DETACHMENT OF THE POSTERIOR PIGMENTED LAYER FROM THE STROMA
is a rare occurrence, seen most usually after injuries or an iridectomy when
the two leaves float freely in the anterior chamber. In inflammatory
conditions localized separations occur with considerable frequency, with the
result that cystic spaces are formed preferentially in the middle region of the
iris. This may occur occasionally in association with an inflammatory
ectropion of the pigment border, or with extensive anterior peripheral
synechiae in glaucoma, but the fact that in most cases apposition of the two
layers resists mechanical separation suggests that these detachments depend
upon pre-existing conditions such as unequal growth, so that the posterior
layer is thrown into folds over the anterior (Corrado, 1934).
DISPLACEMENTS OF THE CILIARY BODY are rare and usually incidental
results in a pathological picture dominated by more serious events. We
have already studied the gross displacement of the ciliary processes which
may be due to an intra-uterine inflammation which has resulted in the
formation of congenital anterior staphyloma.” A similar change may be
found pathologically, induced by the organization of haemorrhages or
exudates following severe inflammation or trauma. Some degree of dis-
placement may also follow a dislocation of the lens (Fuchs, 1929), and is a
frequent but incidental concomitant of a malignant tumour in this region.
DETACHMENT OF THE CHOROID
The choroid is detached not infrequently from the sclera, either by
traction from within or by the accumulation of fluid, either serous or
sanguineous, in the peri-choroidal space. Such an occurrence is only to be
expected when it is remembered that the uveal tract is only loosely attached
to the sclera except at the scleral spur, at the posterior pole, and in the
region of the vortex veins. Especially in the anterior region of the choroid
and ciliary body is the attachment negligible, for here the long, thin supra-
choroidal lamellae run outwards and backwards in a very oblique manner ;
indeed, in this region there may be said to exist normally a lymph space.
DETACHMENT OF THE CHOROID BY THE TRACTION OF ORGANIZED
INFLAMMATORY Tissue is a common pathological finding in eyes which have
been lost through plastic irido-cyclitis. The condition is, of course, not
recognizable clinically, and since such eyes are already blind, its only interest
lies in the fact that continued traction may involve the ciliary body also and
thus cause severe irritation.
A SEROUS DETACHMENT is produced by the accumulation of fluid under
the choroid. This is probably a common occurrence in a minor degree,
1 p. 2398. ? Vol. IV. 8 Vol. II, p. 1324.
DISEASES OF THE UVEAL TRACT 2539
for in the normal eyes of man (such as those removed for an orbital tumour.
O'Brien, 1935) or of monkeys, some serous fluid may be found quite
frequently in the peri-choroidal space. In mechanically congestive conditions,
as in the case of a neoplasm of the outer layers of the choroid, for example,
the disturbance to the circulation produces an outflow of serous fluid into
this space, which may cause an extensive detachment. A similar occurrence
may be associated with inflammatory congestive conditions, as scleritis
(Purtscher, 1938) or an episcleral abscess (Muirhead, 1934). Such a detach-
FIG. 2147-SERous DETACHMENT of THE CHoRoid.
After a cataract extraction, showing detachment of the posterior part of the
ciliary body and choroid with wide separation of the spaces of the lamina supra-
choroidea which are filled with granular coagulum (Collins, T. O. S.).
ment, however, occurs most typically whenever a perforating lesion has
occurred (Hudson, 1914), a class of case which, of course, includes intra-
ocular operations, particularly those undertaken for glaucoma (iridectomy,
and especially frequently after Elliot's trephining or Lagrange's sclerectomy),
or for cataract (Fig. 2147).
The first detachment of the choroid after a cataract extraction was reported by
Knapp (1868), in which case the eye was excised and examined under the mistaken
diagnosis of a neoplasm. Thereafter reports were few (Reuling, 1870; Groenouw, 1889;
Haab, 1893; Lindemann, 1893; Marshall, 1896; Velhagen, 1897) and the condition was
considered a great rarity until Fuchs (1904) experience of 6 cases observed within a
period of 7 months led him to suggest that it was a relatively common complication
after extractions which was usually overlooked. Subsequent reports of note were made
by Augstein (1901), Meller (1911), Collins (1914) and Verhoeff (1915). Fuchs had

2540 TEXT-BOOK OF OPHTHALMOLOGY
given an incidence of 4.7% of all extractions, a proportion corroborated by Barkan
(1915), but Hagen (1921) reported 11 detachments in 50 consecutive extractions, an
incidence of 22%. It is to be remembered that these observers considered that the
detachment occurred some days after operation, but, by immediate post-operative
observation, O’Brien (1936) found it to occur almost invariably : in 140 consecutive
extractions, both by the intra- and extra-capsular method, in which there were no
complications, the fundus was visible in 92, and in 86 of these an immediate detach-
ment of the choroid was observed, that is, an incidence of more than 93%. In other
operations, particularly those for glaucoma, the incidence is probably as high : Fuchs
(1900) who found a 4.7% incidence after cataract extraction, saw 10% some days
after iridectomy, Meller (1911) found 22% after sclerectomy, Bothman and Blaess
(1935) found 14%, and Hagen (1921), who noted 22% after cataract extractions,
found 76% after the trephine operation.
The aetiology of these detachments has excited some controversy.
Rnapp (1868) believed that the condition was due to a serous scleritis, Haab
(1893) considered the appearance due to cysts in the peripheral retina,
Velhagen (1897) to a vesicular formation similar to that described by
}reeff in the ciliary epithelium after paracentesis of the anterior chamber,"
while Fuchs (1900) attributed it to slight detachments of the ciliary body at
the angle of the anterior chamber and a seepage of aqueous humour into
the peri-choroidal space. It would seem, however, that there is little doubt
that the mechanism is that originally hypothecated by Marshall (1896) and
maintained with slight modifications by Meller (1911), Hudson (1914), Hagen
(1921), Verhoeff (1925), O’Brien (1935) and others, a mechanism which was
verified by experimental studies on rabbits by Meesmann (1921); owing to a
sudden lowering of the intra-ocular pressure and the abolition of the anterior
chamber allowing the lens and vitreous to move forwards, there is a transudation
of fluid from the choroidal veins into the peri-choroidal space. It is to be
remembered that these veins, especially in the anterior region, are numerous
and large, and are anatomically blood-sinuses with a single endothelial wall
with no muscular coat and a minimum of connective-tissue fibrillae ; a
sudden release of external pressure will lead to their engorgement, and
transudation through their thin walls will be easy. Moreover, fluid with-
drawn from beneath such a detachment is albuminous, containing fibrin
and some red blood corpuscles, and coagulates rapidly (O'Brien, 1935); it is
therefore derived from the blood and not from the aqueous.
An additional point was suggested by Lindner (1936). In a leaking eye the
aqueous humour is initially replaced partly by fluid from the vitreous gel, and this is
made good by abnormal plasmoid intra-ocular fluid. He considered that this fluid
seeping through the vitreous causes it to shrink by chemical action, and that the
factor of vitreous shrinkage has much to do with the choroidal detachment.
Choroidal detachments associated with intra-ocular operations may be
described in three classes : (1) Operative detachments occurring at or
immediately after operation, (2) post-operative detachments occurring or
* Vol. I, p. 437 (440).
DISEASES OF THE UVEAL TRACT 2541
persisting some days after operation, and (3) delayed detachments occurring
some months or years later. The first type, as we have seen, is almost
universal, the second is common and the third rare.
OPERATIVE DETACHMENTs occurring at the time of operation are usually
flat, the border of the detached area appearing as a dark, gently curved,
elevated line with the convexity directed towards the nerve head. The
fundus in the affected area has a light, somewhat translucent hue, and the
appearance of elevation is accentuated by the arching forwards of the
retinal vessels at its edge. They are almost invariably peripheral and usually
in the inferior or infero-temporal or infero-nasal quadrants ; their occurrence
in the upper fundus is rare. More than one may be present, and occasionally
large areas may be affected occupying approximately half of the fundus and
reaching backwards to the disc. The prognosis of these detachments is
good, the choroid usually re-attaching itself with closure of the corneal
section and vision being unaffected. Alternatively the condition persists,
and assumes the characteristics of the second type.
Post-OPERATIVE CHOROIDAL DETACHMENTS persisting or occurring some
days after operation are due to delayed closure of the wound or the subse-
quent rupture of its loosely adherent margins, whether it be a corneal section
or the conjunctival flap of an operation for glaucoma. In the latter opera-
tion they are more common than in the former. The formation of a new
detachment is thus associated with a diminution or abolition of the anterior
chamber, a lowering of the ocular tension, and (usually) the appearance of
folds in Descemet's membrane. Frequently some leakage from the wound
can be demonstrated by the presence of a green stain after the instillation
of fluorescein. Such a detachment may be visible by oblique illumination .
as a dark brown mass behind the lens, and ophthalmologically it appears
as a dark grey, globular, rounded swelling with a smooth surface extending
hemispherically into the vitreous. The borders are dark and well-defined
and over the surface course normal retinal vessels. It is situated peri-
pherally, anterior to the equator, preferentially medially and laterally, but
frequently more than one exist. Sometimes there are adjoining elevations
separated by deep creases ; sometimes they are widely separated, as on the
temporal and nasal sides ; and occasionally massive detachments may
extend around most or all of the globe, abolishing vision by mechanical
obstruction ; they rarely, if ever, reach the macula or the disc, and a
secondary detachment of the retina does not occur.
The usual history of such detachments is that they disappear in several
days ; sometimes they persist for some weeks, but rarely for over a month
(6 weeks, O’Brien, 1935; 6 months, Maschler, 1920 ; 8 months when
operative interference was successfully undertaken, Bothman, 1937).
Central vision is unaffected, the visual fields become normal, and the
function of the eye is unimpaired. If the separation is of short duration no
ophthalmoscopically visible traces remain, but if it has persisted long,
2542 TEXT-BOOK OF OPHTHALMOLOGY
pigmentary disturbances are sometimes seen, the affected area appearing
moth-eaten and showing granules and streaks and clumps of pigment which,
so far as is known, are permanent (Schur, 1913; Lindner, 1914; Plocher,
1917; Fuchs, 1918). Verhoeff (1931) examined two such eyes, and con-
sidered the effect to be due to a ridge-like hyperplasia of the pigment
epithelium, the streaks corresponding to the creases present in the choroid
--
º sº
* Gºº
º
Fig. 2148,-Choroid AL STREAK AFTER DETACHMENT.
A pigmented streak is shown in cross-section in the centre with a large cell
almost free from pigment (Verhoeff).
during detachment (Fig. 2148). If, despite treatment, the condition
remains for a very long time, degenerative changes may occur, and the
prognosis usually becomes unfavourable.
Even to this, however, there are exceptions. Thus a case reported by Tillmann
(1933) lasted 150 days after a trephine operation; a spontaneous re-attachment
lasting 85 days was followed by a further detachment which healed spontaneously in
57 days, and a year later the function of the eye and the fields were normal.
DELAYED DETACHMENTs of the choroid, occurring some months or even
years after operation, are rare, but may involve serious consequences. They
are probably always due to a re-opening of the wound, perhaps by unnoticed
trauma (Meller, 1911). A sudden diminution of vision is remarked, the eye
becomes soft, the anterior chamber becomes shallow or disappears, per-
sistent weeping is prominent, and on examination the typical globular
appearance is seen ophthalmoscopically.
Marshall (1896) reported a case coming on suddenly 6 months after a cataract
extraction in which a detachment involved all quadrants: the eye remained soft and
irritable, and had to be removed. In Meller's (1911) case a trauma 3 years after
extraction was followed by a similar sequence, when the wound was found to have
remained fistulous owing to epithelial ingrowths. Not all cases, however, are of such
seriousness. Derby (1930) saw a case wherein a detachment developed spontaneously
8 months after a trephine operation, the tension re-establishing itself, the anterior
chamber re-forming, and the detachment disappearing spontaneously six weeks
later: the duration of the detachment coincided with the temporary appearance of a
conjunctival bleb along the line of the incision. Lambert (1930) also recorded a case
discovered 3 years after an extraction which disappeared spontaneously, and Löhlein
(1931) one 9 months after an extraction, which resolved a week after the closure of
a fistula in the wound by cauterization.







DISEASES OF THE UVEAL TRACT 2543
The differential diagnosis is important and embraces two conditions—
a detachment of the retina and a tumour. The diagnosis from detached
retina is easy, the solid appearance of a choroidal detachment, its smooth
surface (not wavy or mobile as in a retinal detachment), and the appearance
of normal retinal vessels with their colour unchanged, being sufficiently
apparent points of differentiation. With regard to the question of the
presence of a tumour, transillumination of a choroidal detachment shows an
exaggerated lighting of the sclera over the detached area (Hagen, 1921) and
a luminous pupil, while the history of recent operation is usually suggestive.
The treatment of such detachments should be confined to an attempt to
ensure closure of the operation wound, either by light cauterization or by
turning down a conjunctival flap ; as we have seen, the leaking area may be
indicated by staining with fluorescein. When this has been satisfactorily
accomplished, it can be taken that the detachment will resolve spontaneously
and the patient need not be further immobilized or the eye bandaged. Only
occasionally, despite efficient closure of the wound, the tension of the globe
remains so low that the choroid is not pushed back ; in two such cases
Verhoeff (1930) obtained reposition after a scleral puncture. A definite
attempt at repair should be made if the detachment persists for a period of
three months, especially in the case of fistulization operations for glaucoma,
the most recalcitrant cases to deal with. If the simple proceedings mentioned
fail, Bothman’s (1937) suggestion may be tried of turning down a strip of the
tendon of the superior rectus sub-conjunctivally over the operation wound.
Cases of unexplained (“spontaneous '') detachment of the choroid occur, the
nature of which is not clear (Verhoeff and Waite, 1925); they are, however, extremely
rare and may be complicated by a detachment of the retina (Elschnig, 1931). The
condition has been noted in association with a tumour of the lacrimal gland, the
detachment disappearing on surgical removal of the tumour (Ziporkes, 1937).
Augstein. Z. f. Aug., v, 268, 1901. Lindner. A. f. O., lxxxviii, 230, 1914;
Barkan, H. J. Am. Med. As., lxv, 1520, 1915.
Bothman. A. of O., xviii, 65, 1937.
Bothman and Blaess. Am. J. O., xix, 1072,
1935.
Collins. T. O. S., xxxiv, 41, 1914.
Corrado. An. di Ott., lxii, 573, 1934.
Derby. A. of O., iv, 530, 1930.
Elschnig, H. H. K.M. Aug., lxxxvi, 595, 1931.
Fuchs, A. Am. J. O., viii, 601, 1925.
Fuchs, E. A. f. O., li, 199, 1900 ; liii, 375,
1902; lviii, 391, 1904; xcv, 215, 1918;
civ, 247, 1921 ; cxxii, 86, 1929.
R. M. Aug., lx, 797, 1918.
Groenouw. A. f. Aug., xx, 69, 1889.
A. f. O., xcviii, 252, 1919.
Haab. Beit. 2. Aug., i, 103, 1893.
Hagen. K. M. Aug., lxvi, 161, 1921.
Hudson. R. L. O. H. Rep., xix, 301, 1914.
Knapp, H. Die intraocularen Geschwälste,
Karlsruhe, 1868.
Lambert. A. of O., iv, 755, 1930.
Lindemann. Beit. z. A ug, i, 338, 1893.
T.O.-WOL. III.
Cxxxv, 332, 462, 1936.
Löhlein. K. M. Aug., lxxxvii, 825, 1931
Mandicevski. K. M. Aug., xciv, 668, 1935.
Marshall. T. O. S., xvi, 98, 1896.
Maschler. K. M. Aug., lxiv, 549, 1920.
Meesmann. A. f. Aug., xc, 69, 1921.
Meller. A. f. O., lxxx, 170, 1911.
Muirhead. T. O. S., liv, 578, 1934.
O’Brien. Am. J. O., xiv, 527, 1935 ; xvi, 655,
1936.
Perlia. K. M. Aug., l (2), 739, 1912.
Plocher. K. M. Aug., lix, 610, 1917.
Purtscher. Z. f. Aug., xciv, 12, 141, 1938.
Reuling. A. f. Aug., i, 186, 1870.
Schur. K. M. Aug., li (2), 372, 1913.
Tillman. K. M. Aug., xci, 528, 1933.
Velhagen. Cb. pr. Aug., xxi, 363, 1897.
Verhoeff. O. Rec., xxiv, 55, 1915.
A. of O., liv, 20, 1925; iv., 755, 1930.
J. A. m. Med. As., xcvii, 1873, 1931.
Verhoeff and Waite. T. Am. O. S., xxiii, 120,
1925.
Ziporkes. A. of O., xviii, 933, 1937.
G G
CHAPTER XXXVI
DISEASES OF THE RETINA
I am introducing this chapter with the photographs of two English ophthal-
mologists who have influenced the development of this branch of our subject pro-
foundly : the one, who belonged to the earlier school of the latter days of the last
century, enriched it by his unusually shrewd and painstaking clinical observations;
the other, whom we in 1940 may consider a member of the middle school of the beginning
of the present century, contributed no less lavishly to our pathological knowledge of
the subject.
Marcus Gunn (1850–1909) (Fig. 2149), who came of old Scandinavian stock, and
was associated with Moorfields Hospital and the National Hospital for Nervous Diseases
in London, will always be remembered as one of the masters of ophthalmoscopic
examination and description. Among many wider interests of comparative biology,
his original contributions to our knowledge, more particularly of diseases of the retina
and optic nerve, are particularly outstanding. Probably the pieces of work for which
he will be most remembered are embodied in his classical paper on the ophthalmoscopic
evidences of retinal arterial Sclerosis as a sign of changes in the arteries in the brain and
other organs, and that on the clinical manifestations and prognosis of renal retinitis.
George Coats (1876–1915) (Fig. 2150) earned for himself in a comparatively short
time, by hard and unremitting work undertaken with the enthusiasm and zest that
befitted a great adventure, a place in the scientific and pathological aspects of ophthal-
mology which has been generously recognized all the world over. We have already
come across his name on many occasions in these pages, particularly in association
with the subject of developmental anomalies, but his most classical work is undoubtedly
associated with the vascular diseases of the retina, one of which—exudative retinitis—
he established as a clinical entity. It is indeed one of the losses of ophthalmology that
he died at the early age of 39 years.
I. GENERAL CONSIDERATIONS
The diseases of the retina form a very complicated chapter, but a more
than usually interesting and important one. As the key-tissue of the eye the
retina carries the primary responsibility of the visual function, and the
destruction of its tissues by any pathological process involves irreparable
blindness. Moreover, its destruction is easy, partly because of the delicacy
and complexity of its structure which rapidly falls a victim to noxious
influences, and partly because of the intensity of its metabolism which is
unable to support the deprivation of essential supplies with impunity for
any length of time. It will be remembered 1 that, although the metabolism
of the retina is still largely a mystery, we do know that it has a very
high oxidative capacity and a glycolytic activity of unusual intensity, about
* Vol. I, p. 426 (427).
2544
DISEASES OF THE RETINA
25.45
Fig. 2149.-MARCUs GUNN, 1850–1909.

2546 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2150–GEoRGE CoATs, 1876–1915.

DISEASES OF THE RETINA 2547
twice that of the iris which is approximately equal to that of muscular
tissue, and comparable, indeed, with that of malignant tumours (Warburg,
1924–25; Warburg, Posener and Negelein, 1924; Negelein, 1925;
Nakashima, 1929; Adler, 1930–31). Once an adequate supply of oxygen
is cut off, or once the tissue is exposed to the lytic action of leucocytes,
exudates, or bacteria, death rapidly sets in. Thus 6 to 7 hours after the
establishment of an experimental embolus of the central artery, Birch-
Hirschfeld (1900) found gross pathological changes in the neural elements,
and Guist (1926) observed permanent damage 15 minutes after section of
both the central retinal and posterior ciliary arteries. In such a case, in
the absence of toxic elements, the destruction of the tissue is by autolysis,
while in the presence of toxins or inflammatory processes, heterolysis occurs.
As a result the retina rapidly becomes swollen and opaque and eventually
suffers total atrophy, only a reticulum of the supporting frame-work of
nerve tissue and strands of the connective tissue associated with the blood-
vessels remaining, while glial proliferation is stimulated.
Autolysis occurs when, in the injured or dying tissues, anabolism cannot maintain
its equilibrium with catabolism with the result that acids are formed in excess (Galante,
1921 ; Sala, 1933; Krause, 1936). The enzymes which in the normal alkaline retina
take part in its metabolism, continue to break up metabolites to form acid products
which cannot be dealt with, the increased osmotic activity of which leads to the
imbibition of water and a swelling and translucency of the tissues. The proteolytic
enzymes then begin to act, hydrolysing the intra-cellular proteins into smaller and
smaller fragments, and converting the large molecular aggregates into simpler products
which diffuse away, eventually leaving only the supporting tissue behind.
An adequate understanding of the pathological processes in the retina
must be based upon a consideration of the reaction of its various elements to
noxious stimuli. These elements are of two very different types—neural
ectodermal tissue, the primary constituent of the retina, and mesodermal
elements interpolated with the blood-vessels.
With regard to the primary nervous tissue, we have already seen that
it is made up of three neurons. The first is the neuro-epithelial layer, com-
posed of sensory end-organs (the rods and cones), their nuclei, and terminal
fibres, which corresponds in the somaesthetic system to the sensory end-
organs in the skin. This neuron forms a district in itself devoid of blood-
vessels, nourished by diffusion from the chorio-capillaris. The second neuron
is comprised essentially of the bipolar cells, minute structures with their nuclei
in the inner nuclear layer and their processes extending from the outer to the
inner reticular layer, which nevertheless are homologous with the far-flung
dorsal spinal root cells, the processes of which extend from the skin peripherally
to the nuclei of the medulla. The third neuron, which is homologous with the
arcuate fibres which run from the medulla through the mesial fillet to the
thalamus, is represented by the ganglion cells of the retina, the axons of
Vol. I, p. 243.
2548 TEXT-BOOK OF OPHTHALMOLOGY
which traverse the nerve-fibre layer, the optic nerve, chiasma and tract,
and terminate in the primary visual centres. The bipolar cell, therefore,
with its processes is a true peripheral nerve, the ganglion cells form a
spread-out nucleus of the central nervous system, and the nerve-fibre layer
and its proximal continuations are analogous to tracts in the brain-stem,
sharing with them the pathological processes to which they are prone. It is
to be remembered, also, that these layers—the cerebral layers of the retina-
are nourished differently from the neuro-epithelium, and analogously to the
brain—by end-arteries, which, being a secondary mesodermal invasion into
Fig. 2151.-RETINA or Rabbit 17 DAY's AFTER SEction of Posterior
CILIARY ARTERLEs.
Showing the border of the area of degeneration. Note the replacement by
glial tissue and the leucocytic infiltration at the extreme right (Nicholls, Brit. J. O.).
ectodermal tissue, are insulated from the nervous elements among which
they lie by connective tissue and neuroglial structures.
Diseases of the retina may therefore be divided into two classes—those
affecting the nervous elements and those affecting the mesoblastic tissues.
The diseases affecting the nervous elements may again be classified according
to the neuron which they attack. Thus in the first neuron primary degener-
tions may occur, such as primary pigmentary degeneration or tapeto-retinal
degeneration, death from poisoning as by the hypo-iodite preparations,
destruction of the nervous elements may be followed by gliosis, or secondary
lesions follow diseases of the choroid (chorio-retinitis) whence this neuron
draws its nourishment. The second neuron, since it lies within the two
ºapillary nets of the retina, is peculiarly prone to lesions originating from

DISEASES OF THE RETINA 2549
the blood-vessels, and forms the common site for the deposition of haemor-
rhages, transudates, and exudates determined by the effect of toxins on the
capillary walls, while developmental anomalies are typified in the growth of
retinoblastomata. The third neuron may show developmental anomalies,
as in amaurotic idiocy, but like the nuclei of the brain-stem, the ganglion
cells are peculiarly prone to the action of poisons (methyl alcohol, tobacco,
etc.), while a secondary degeneration of the nerve-fibres and ganglion cells
is a necessary consequence of any interruption of the nerve-fibres in the optic
nerve by mechanical, inflammatory, or atrophic processes.
Moreover, the retina is by no means a self-contained entity in its
liability to disease. We shall see that infective processes in the vitreous
body readily affect it, while inflammations of the uveal tract rarely leave it
unscathed. We have already seen that inflammations of the anterior
segment of the uvea frequently involve retinal complications through the
diffusion of toxins to the posterior segment of the eye, while choroidal
inflammations and degenerations of any severity invariably do so. More
especially does this affect the sentient layer which is nourished directly from
the chorio-capillaris. This influence is best illustrated by the destructive
effects of a failure in the choroidal circulation, a question which we have
already discussed "; the complete degeneration of the neural tissues and their
eventual replacement by glial and scar tissue are well illustrated in Figs. 1769
and 2151 (Berlin, 1871; Wagenmann, 1890; Komoto, 1915; Nicholls, 1938).
It is interesting, however, that even although the central retinal artery, as well as
the posterior ciliaries, are cut, while the degeneration of the retina at the posterior pole
is complete, it gets much less marked towards the equator and the entire retina,
including the inner layers, is normal anteriorly (Komoto, 1915). Since the only blood-
supply available in this case is the anterior ciliaries, this indicates that the whole
retina in its anterior part can be nourished from the choroid, a fact which explains
many clinical phenomena, for example, the limitation of the Oedema and necrosis to
the posterior region in obstruction of the central artery.
Adler. T. Am. O. S., xxviii, 301, 1930. Kubowitz. Bioch. Z., ceiv, 475, 1929.
A. of O., vi. 901, 1931. Nakashima. Bioch. Z., ceiv, 479, 1929.
Berlin. K. M. Aug., ix, 278, 1871. Negelein. Bioch. Z., clviii, 121 : clv, 122,
Birch-Hirschfeld. A. f. O., i, 166, 1900. 1925. *
Galante. Boll. S. Ital. di biol. sper., ii, 337, Nicholls. Brit. J. O., xxii, 672, 1938.
1921. - Sala. Boll. d’Oc., xii, 161, 1933.
Guist. Erholungsfähigkeit d. Netzhaut mach Tamija. Bioch. Z., clz.xxix, 114, 1927.
Unterbrechung.d. Blutzirkulation, Berlin, Wagenmann. A. f. O., xxxvi (4), 1, 1890.
1926. Warburg. Bioch. Z., clii, 51, 1924; clz, 140,
Romoto. T. O. S., xxx, 295, 1915. 1925.
Rrause. A. of O., xvi, 425, 1936. Warburg, Posener and Negelein. Bioch. Z.,
Fºrebs. Bioch. Z., clxxxix, 57, 1927. clii, 309, 1924.
THE HEALING OF WOUNDS
In traumatic cases, since a wound of the retina alone never occurs in
practice, the picture presented by the healing of a wound in the posterior
1 p. 2101.
2550 TEXT-BOOK OF OPHTHALMOLOGY
segment of the eye is dominated much more by the reparative activity of the
choroid, and usually of the episcleral tissues as well, than by that of the
retina. The general picture presented in this process when uncomplicated
by infection has already been described." It will be remembered that the
tract of such an uncomplicated wound is rapidly filled with blood-clot which
soon becomes organized into a fibrous scar, the fibroblasts being derived
essentially from the highly vascular choroid and to a less extent from the
episcleral tissues; occasionally the process is exuberant in its intensity and
long fibrous strands extending into the vitreous determine the appearance
of a traumatic proliferating chorio-retinitis.
In this process the retina takes a relatively small part. So far as the
nervous elements are concerned, the changes are entirely degenerative in the
Fig. 2152.-A RETINAL TEAR (Lister, Brit. J. O.).
immediate neighbourhood of the wound (Tepliaschin, 1894; Parsons, 1903);
in the nerve-fibre layer peripheral to the wound, the ganglion cells show
degenerative changes and round cells make their appearance, while degenera-
tion appears in the fibres of the optic nerve. Some authorities consider that
there is evidence of proliferation of the glial tissue which may produce a
profuse secondary gliosis or in some cases a tumour-like mass (Krückmann,
1905; v. Hippel, 1918), but this is questioned by others (Parsons, 1903–05;
Collins, 1928). The latter authorities claim that the active reparative factors
—and they certainly form the essential ones—are the mesodermal and
endothelial elements of the blood-vessels, which, in association with those
in the choroid, provide the main constituents in the process of the
organization of the final fibrous scar.
1 p. 2100.

DISEASES OF THE RETINA 2551
Wounds involving the retina alone uncomplicated by trauma to other
structures, are very difficult to produce experimentally, but they are com-
monly seen clinically as tears in the retina. It is notorious that such
ruptures do not become closed or bridged across by cicatricial tissue ;
instead, the margins of the wound show only degenerative changes in the
nerve elements with no indication of proliferative activity (Lister, 1924;
Collins, 1928; Gonin, 1934; Vogt, 1936; Arruga, 1936; and many others)
(Fig. 21.52). In all their pathological changes, indeed, the neural elements of
the retina degenerate, and never regenerate ; and if they make abortive
attempts at proliferation (Tepljaschin, 1894; Schreiber and Wengler, 1910;
Weil and Meyer, 1940), these are only pathological curiosities without
essential significance in a reparative process.
Arruga. Detachment of the Retina, N.Y., Parsons. R. L. O. H. Rep., xv (3), 215, 1903.
1936. Path. of the Eye, London, ii, 457, 1905.
Collins. T. O. S., xlviii, 107, 1928. Schreiber and Wengler. A. f. O., lxxiv, I,
Gonin. Le Décollement de la Retime, Lausanne, 1910.
1934. Tepljaschin. A. f. Aug., xxviii, 354, 1894.
v. Hippel. A. f. O., xcv, 173, 1918. Vogt. Die operative Therapie w. d. Path. d.
Krückmann. A. f. O., lx, 350, 452, 1905. Netzhautablósung, Stuttgart, 1936.
Lister. Brit. J. O., viii, l, 1924. Weil and Meyer. A. f. O., xxiii, 591, 1940.
II. DISTURBANCES OF THE CIRCULATION
A. Anomalies of the Blood-flow
Clinical methods for the observation of the variations in the calibre and blood-
pressure of the retinal vessels are of considerable importance in this connection, and
although most of the methods have already been noted, it may be well to summarize
them here. We have already seen that in measurements of the calibre of the vessels,
a considerable degree of accuracy can be obtained by projecting ophthalmoscopically a
graticule (Morgan, 1927 ; Lambert, (1932), or a scale (Lambert, 1934–35), or a
system of lines of graded thickness (Neame, 1936) on the retina. Fundus
photography with subsequent micro-measurements on the plate also gives
results of value (Haessler and Squier, 1932). Greater accuracy is obtained
by the Mossokular of Lobeck (1934–37) in which the principle of the helio-
meter is applied in obtaining double images by altering the optical system of the
Gullstrand ophthalmoscope. This gives relative measurements which can be com-
pared with the diameter of the disc (1.5 mm.) (Kühn, 1937; Badtke, 1937). Such
measurements show that normally the arteries are slightly narrower than the veins,
the proportions being—calibre of artery : vein : diameter of the disc = 1 : 1-1 to
1.4 : 11 to 17. Translated into absolute measurements Lobeck (1937) gives the normal
width of the retinal arteries near the disc at 0-134 to 0-088 mm., of the veins as 0- 176
to 0-097. In diseased conditions, such as hyperpiesia or renal retinopathy, this
proportion is disturbed.
A clinical estimate of the blood-pressure in the retinal arteries can be made by
observing the pulsation of the vessels while external pressure is made upon the globe.”
The most widely used instrument for this purpose is the ophthalmo-dynamometer of
Bailliart (1917), the pressure registered at the commencement and cessation of pulsation
being taken as indicating the diastolic and systolic pressures : Bailliart's measurements
average 30–35/60–70 mm. Hg. It has already been pointed out at length * that, con-
1 Vol. II, p. 1178. * Vol. I, p. 403. * Vol. I, p. 404.
2552 TEXT-BOOK OF OPHTHALMOLOGY
sidered in the absolute sense, these measurements are highly inaccurate ; but in this
respect they differ little from the results of most clinical methods. Although in the
Scientifically physiological sense they can only be accepted with the greatest reserve,
their clinical value is undoubted. The diastolic pressure is the most important
measurement ; it should be approximately half that of the diastolic pressure registered
by the sphygmomanometer in the brachial artery, and is normally some 15–20 mm.
Hg above the intra-ocular pressure as registered tonometrically. If a difference of
this order does not obtain, whether it be due to a high intra-ocular pressure or a low
blood-pressure, there is a tendency for a failure of the capillary circulation, with a
consequent loss of conductivity or even eventual atrophy of the nerve fibres. If the
difference is greater, a state of hypertension, with its accompanying dangers, exists.
Badtke. K. M. Aug., xcix, 535, 1937. Lambert. A. of O., vii, 440, 1932; xii, 868,
Bailliart. An. d’Oc., cliv, 257, 648, 1917; 1934.
clv, 433, 1918; clzv, 321, 1928. Am. J. O., xviii, 1003, 1935.
Haessler and Squier. A. of O., vii, 280, Lobeck. A. f. O., cxxxiii, 152, 1934; cz.xxvi,
1932. 439, 1937.
Kühn. A. f. O., cxxxviii, 129, 1937. Morgan. Brit. J. O., xi, 339, 1927.
Neame. T. O. S., lvi, 155, 1936.
HYPERAEMIA
(a) ACTIVE HYPERAEMIA
An active hyperaemia in the retina, wherein the arterial system is
engorged, is characterized by a fullness and tortuosity of the arteries, and is
recognized clinically most readily from the pinkish appearance of the optic
disc with a veiling or blurring of its margin due to unusual capillary conges-
tion. It occurs from general causes when the blood-pressure is raised, and
from local causes in conditions of retinal or uveal inflammation. It has
also been claimed that a similar result follows cervical sympathectomy.”
None of these conditions is of great clinical significance. In a more
indefinite sense a hyperaemia of the retina is said by many writers to result
from continual visual irritation, persistent exposure to strong light, or
from the effects of refractive errors, and to lead to a super-sensitive state
of retinal hyperaesthesia characterized by photophobia, lacrimation,
blepharospasm, neuralgia, and lack of visual endurance (Jäger, 1876). In
addition to visual causes, reflex conditions in the naso-pharynx, and general
disorders of the physical and mental health have been included in the
aetiology. The whole subject is extremely vague and indefinite and rests
upon little or no pathological foundation, and as a rule the symptom-
complex is probably best viewed in the light of an element in a complicated
neurosis.
(b) PASSIVE HYPERAEMIA : CYANOSIS
Passive hyperaemia or venous congestion is a commoner condition and
more readily recognized since the veins become dilated and tortuous to a
greater extent than the arteries. It is due to an obstruction of the venous
* Vol. I, p. 416 (417).
DISEASES OF THE RETINA 2553
return from any cause, whether it be general conditions such as cardiac
insufficiency (Geigel, 1897), a mediastinal tumour (Schlepegrell, 1881),
emphysema, coughing, or convulsive seizures as in epilepsy (Gowers, 1904),
and so on. Obstructions in the orbit have a similar effect, whether neoplastic
or inflammatory, and the most intense effect in this region is seen in
aneurysmal communications between the carotid artery and the cavernous
sinus." In the eye itself any obstruction at the nerve-head has a similar
FIG. 2153.-CYANosis of THE RETINA.
In a boy of 18 with polycythaemia compensatory to cardiac insufficiency
(Kronfeld, Am. J. O.).
result, as is seen in papilloedema due to increased intra-cranial pressure;
while in the retina obstruction of the veins in thrombosis has a like result.”
The condition of papilloedema will be fully discussed in a later chapter, and it is
sufficient to note here the intensity of the venous engorgement which it may entail.
It is interesting that, unless in terminal states, the greatest degree of stagnation
appears to occur with moderate intra-cranial pressures of about 40 mm. Hg, for at this
level the difference between the arterial and venous pressures in the retina is minimal:
above this pressure the general blood-pressure rises to overcome the cerebral anaemia
and the circulation proceeds at a higher level (Lindberg, 1935). However that may
be, the stasis can be extreme ; not only does it involve a venous engorgement, but, if
it persists any length of time, the disordered capillary permeability may result in a
generalized retinal oedema with star-shaped exudative formations at the macula,
producing a picture closely resembling renal retinopathy (stELLATE RETINopATHY).”
The exudates may disappear on the relief of the intra-cranial pressure by lumbar
puncture, to return again with a subsequent rise in pressure (Colrat, 1930; Puscariu
and Nitzulescu, 1931; Merkulow, 1937).
1 Vol. IV. * p. 2578. * p. 2595.

2554 TEXT-BOOK OF OPHTHALMOLOGY
CYANosis of THE RETINA is the apt term applied when engorgement
becomes so marked as to change the colour of the fundus (Fig. 2155.
Plate XLVIII). The ophthalmological picture of intensely congested
and tortuous veins engorged with dark blood, of arteries resembling
normal veins in their appearance, of occasional haemorrhages, and of a
generalized purplish hue of the fundus forms part of a general cyanosis
(Fig. 2153). Such a condition is usually associated with congenital heart
disease with stenosis of the pulmonary artery or with one or other of the
types of polycythaemia; " and since the former is frequently associated
with a compensatory polycythaemic condition of the blood, it is
Fig. 2154–Cyanosis of the RETINA.
The same case as Fig. 2153, showing a section through a vein near the dise
(Kronfeld, Am. J. O.).
probable that this, as well as the mechanical engorgement, is responsible
for the very peculiar and striking ophthalmological picture (de Schweinitz
and Woods, 1925). Cyanosis can, however, occur in the absence of any
polycythaemic changes in cases of acquired heart failure and emphysema
with generalized venous stasis (Jancke, 1936–37), or in erythromelalgia
(Calhoun, 1926).
The condition as associated with congenital heart disease was first briefly noted
by v. Ammon (1841), and was fully described by Knapp (1861), since whose time it
has been repeatedly remarked (Nagel, 1886; Hirschberg, 1904; Posey, 1905; Krämer,
1910; and others).
p. 2738.

DISEASES OF THE RETINA 2555
In some cases there is no functional disability, and in these histological
examination reveals enormously dilated blood-vessels but no other patho-
logical changes in the eye (Knapp, 1861); Brailovskij and Glekler, 1927;
Kronfeld, 1931) (Fig. 2154); but in others visual deterioration may be
serious, and these are characterized by marked endarteritis, multiple
arterial thrombotic occlusions with secondary changes in the veins and
massive haemorrhages, and usually terminate disastrously in secondary
glaucoma (Goldzieher, 1904; Baquis, 1908; Ginzburg, 1928). In such
cases, of course, the treatment depends on the general condition of which the
ocular changes form merely a local symptom.
v. Ammon. Klin. Darstellung, iii, 75, 1841. Knapp. Verhandl. d. natur.-med. Vereins zu
Baquis. A. f. O., lxviii (2), 177, 1908. Heidelberg, ii, 54, 1861.
Brailovskij and Glekler. Russ. O. J., vi, 267, Krämer. Z. f. Aug., xxiii, 27, 1910.
1927. Kronfeld. Am. J. O., xiv, 1108, 1931.
Calhoun. Contrib. to Oph. Science, Wiscon., Lindberg. A. f. O., cxxxiii, 191, 1935.
29, 1926. 2- Merkulow. Acta O., xv, 406, 1937.
Colrat. A. d’O., xlvii, 773, 1930. * Nagel. Mitt. aus d. O. Klin. in Tübingen,
Geigel. Münch. med. W., xliv, 222, 1897. ii (3), 411, 1886.
Ginzburg. K. M. Aug., lxxxi, 591, 1928. Posey. T. Am. O. S., x, 634, 1905.
Goldzieher. Cb. pr. Aug., xxviii, 257, 1904. Puscariu and Nitzulescu. Brit. J. O., xv, 697,
Gowers. Medical Ophthalmology, p. 191, 1904. 1931.
Hirschberg. A. f. O., lix, 131, 1904. Schlepegrell. Diss., Göttingen, 1881.
Jäger. Erg. d. Untersuchung mit d. Augen- de Schweinitz and Woods. T. Am. O. S.,
spiegel, Wien, 1876. xxiii, 90, 1925.
Jancke. K. M. Aug., xcvi, 605, 1936; xcix,
756, 1937.
ANAEMIA : ISCHAEMIA
ANAEMIA OF THE RETINA, like hyperaemia, may be due to general or local
causes. In the first case the ocular condition is merely a part of a general
syndrome, as occurs, for example, in general anaemia, cerebral anaemia,
syncope, or on a sudden fall in the general blood-pressure (Wordsworth, 1863,
and others). In heart failure the retinal circulation may indeed fail to such
a degree that transient blindness results (Rosenstein, 1925–27). Such
anaemic conditions are especially prone to occur in the eye, particularly when
a condition of arterial hypotony exists whether it be due to disease or
functional conditions, since the blood reaching the retina has to overcome
the intra-ocular pressure ; and if the symptoms are persistent and assume
alarming proportions involving repeated transient attacks of blindness, in
the event of it being impossible to raise the general blood-pressure and tone
up the vaso-motor control, it may be legitimate to attempt to lower the
intra-ocular pressure by miotics or even by operation (Lauber, 1937).
The extreme lability which the ocular circulation may display in this respect is
seen, for example, in the change in the retinal blood-pressure with the position of the
body. Thus Kamogawa (1936–37) found that in normal persons the change from the
erect to the horizontal position involved an alteration in pressure of from 9 to 11 mm.
Hg in the central retinal artery measured dynamometrically ; and it was noted by
very early writers that in unstable persons temporary total blindness might follow the
assumption of the erect posture (Schmall, 1888; Hutchinson, 1892).
2556 TEXT-BOOK OF OPHTHALMOLOGY
A more pronounced and lasting degree of anaemia, classically referred
to as ISCHAEMIA OF THE RETINA (Gräfe, 1861) is seen in states of general
collapse, when complete blindness, which may last for some days or weeks or
until death, may occur, accompanied by a fixed dilated pupil, and sometimes
followed, if the patient survives, by optic atrophy (Fig. 2156, Plate XLVIII).
The first conditions of this type to be annotated were the profound anaemias
observed by Haddaeus (1865) in the comatose stage of typhus, by v. Graefe
(1866) in the collapse stage of cholera, and by Knapp (1880) in a unilateral
case in whooping cough ; but the most common example is after prolonged
and profuse haemorrhage, such as is met with in parturition, gastric and
duodenal ulcers, pulmonary tuberculosis, and much more rarely, after severe
wounds.
RETINAL ISCHAEMIA AFTER EXSANGUINATION is a rare event considering
the common occurrence of profound losses of blood. It is, however, a well-
known clinical catastrophe which was alluded to by Hippocrates, and has
received ample mention in the literature ; thus Terson (1922) was able to
review 250 publications on the subject. It is interesting that visual symptoms
usually follow bleeding from medical conditions—a haematemesis, a melaena,
a renal haemorrhage, a haemoptysis, or a uterine haemorrhage—and relatively
rarely from wounds or surgical procedures. It is interesting also that
recurrent haemorrhages are the most prone to affect the vision, several small
haemorrhages being more effectual than a single large One although the
amount of blood lost is the same or even less. The resulting blindness is
usually bilateral, but is unilateral in some 15% of cases, and it may be
permanent or transitory. It usually comes on between the 3rd and the
7th day after bleeding but may be delayed for some time. Terson's
(1922) statistics of its incidence are these : during the haemorrhage, 8.3% ,
immediately after, 11.6% ; within 12 hours, 14.2% ; within 2 days, 19.2% ;
between 3 and 16 days, 39.2% ; and later than 16 days after the haemorrhage,
7.5%.
Typical examples will be found in the following papers. Bleeding from the
stomach or bowel : Alt (1912); Hegner (1912); Pincus (1919); Görlitz (1920);
Grimminger (1925).
From the uterus : Calhoun (1913); Puppel (1924). After childbirth or abortion :
Bistis (1908); Pincus (1919); Terrien (1921); Fink (1924); Pines (1931).
From the nose : Bistis (1908); Harbridge (1924); Grimminger (1925).
From wounds : Pröll (1907); Pincus (1919); Terrien (1921).
Clinically the pupil is always dilated and fixed, but the ophthalmoscopic
picture may be varied. Sometimes the fundus appears normal; at other
times all that can be remarked is an attenuation of the vessels. A pallor of
the disc is common, and occasionally there is a suggestion of the picture of
optic neuritis with peri-papillary oedema. Not uncommonly retinal haemor-
rhages occur; sometimes there are white fluffy patches of oedema resembling
those found in renal retinopathy (Fig. 2166), but only rarely is the entire
PLATE XLVIII
RETINAL VAscular ANoMALIEs
Fig. 2155.-CYANosis of RETINA. Fig. 2156.-Ischlºria AFTER Exsang UINATION.
A girl of 17 years with morbus cordis Following haematemesis. Note optic atrophy,
and a coloboma of the fundus. pigmentary disturbances and angioid streaks.
Fig. 2157.-OBSTRUCTIox of CENTRAL RETINAL ARTERy.
Fig. 2158.-VENous THRowſhosis. Fig. 2159.-VENous THRow Bosis.
End result of a rt, sup, temp. thrombosis.
Note tortuous new vessels.
[To face p. 2556.



DISEASES OF THE RETINA 2557
retina oedematous with a cherry-red spot at the macula such as is seen in
complete obstruction of the arterial circulation," while in the worst cases a
complete optic atrophy, frequently with some pigmentary disturbances in
the retina, may result (Fig. 2156, Plate XLVIII). Not uncommonly there
are associated evidences of cerebralischaemia—sensory and motor anomalies,
aphasia, deafness, amnesia, nystagmus, polyneuritis, and coma.
The occasional unilaterality of the incidence of symptoms is interesting, a feature
which may characterize even the worst cases. Thus Bonnet and Blanc (1934) recorded
a uniocular case which presented the typical picture of embolism of the central artery "
Fig. 2160.-RETINAL Isch AEMIA.
After a miscarriage (Pines, Brit. J. O.).
in which all perception of light was lost, the vision 2 weeks later being only and
limited to a small area around the fixation point.
The prognosis of the condition is very difficult to assess, for some of the
cases which seem mildly affected at the time retain their visual defect, while
others in whom all vision is lost recover to a remarkable degree. Terson
(1922) found that on the average 50% of the cases remained permanently
blind, while of the 50% which showed improvement only some 10 to 12%
recovered normal vision. Haemorrhages, of course, may do permanent
damage, and when these occur at the macula the disability is extreme; and
when any degree of atrophy has set in the prognosis is necessarily bad.
1 p. 2561. * p. 2569.

2558 TEXT-BOOK OF OPHTHALMOLOGY
The pathology of the conditions has not been frequently studied, since
opportunities for examining such eyes have been rare. Such investigations
as have been made, however, show that in the earlier stages there is papillary
stasis with general retinal oedema and degeneration of the ganglion cells and
their fibres without any inflammatory evidences ; in the later stages the
picture of complete optic atrophy is presented (Hirschberg, 1881 ; Ziegler,
1887; Raehlmann, 1889; Görlitz, 1920). These histological changes were
verified experimentally on dogs by Uhthoff (1922) and are very similar to the
effects of such poisons as quinine." The essential pathology of the ischaemia
is thus a preferential degeneration of the third neuron. Several considerations,
however, point to the probability that something more than an ischaemia
enters into the question. The rarity of the condition, its occasional
unilaterality, the tendency for the delay in its onset, and its preferential
incidence after repeated haemorrhages, have suggested the possible influence
of some haemoclastic shock or anaphylactic crisis or a toxaemia due to profound
haemolysis (Pincus, 1919; Terson, 1922).
The treatment of such a condition should be directed first of all to combat
systemically the effects of the loss of blood—to stop the haemorrhage and to
maintain the circulation by transfusions of saline, or better, of blood. So
far as local measures are concerned every effort should be made to maintain
the circulation. Hot compresses and irritative sub-conjunctival injections
have been advocated, but undoubtedly the most effectual procedure is to
lower the intra-ocular pressure and thus induce a local vaso-dilatation by a
paracentesis which can be re-opened more than once in order to prolong its
effect.
A similar condition of ischaemia may follow local causes in the orbit—
trauma to the central retinal artery either by accident (Knapp, 1868;
Pagenstecher, 1869; Parsons, 1903; and others), or operatively as in the
removal of tumours of the optic nerve or an optico-ciliary resection (Studer,
1905; Birch-Hirschfeld, 1910; Collins, 1912; Koyanagi, 1913; Komoto,
1915), or by ligature of the carotids (Siegrist, 1900). In such cases, of course,
the posterior ciliary arteries are also sectioned ; the profound pathological
changes which result have already been described ” as well as the experi-
mental researches conducted on the problem (Wagenmann, 1890; Komoto,
1915; Nicholls, 1938; and others). Pressure on the entering artery may
have a like result, such as haemorrhage into the orbit (Pagenstecher, 1884) or
into the nerve sheath (Nettleship, 1895), an orbital abscess (Wirths, 1910), or
tumour (v. Graefe, 1864). Inflammatory conditions, such as facial erysipelas
(Carl, 1884; Jones, 1884; and others), or orbital or nasal suppurations
(Baas, 1893; Weiss, 1903; Hairi, 1921 ; Muloch Houwer, 1925) may
have a similar effect through impeding the circulation by intra-arterial
thrombotic processes, while more extensive thrombotic lesions involving the
1 p. 3031. * p. 2102.
DISEASES OF THE RETINA 2.559
middle cerebral artery or the carotid may be associated with hemiplegia of
the opposite side (Hughlings Jackson, 1864; Gowers, 1875; Elschnig, 1892;
Siegrist, 1898; Guthrie and Mayou, 1908; and others). The picture
presented by such a lesion is at the time usually that of a white translucent
retina swollen with cedema, frequently with a red spot at the macula where
the choroid shines through the thinner tissues, a picture resembling that of an
Fig. 2161.-Division of THE CENTRAL VEssels AND NERVE BEHIND THE GLoBE.
From an orbital wound. Note the central white area of intense oedema and
necrosis with the dark red spot at the macula (Parsons).
embolism " (Fig. 2161). The oedema, as we have seen, is due to an avid
imbibition of fluid caused by the osmotic attraction of catalytic products;
and, as we have already noted in experimental conditions,” the region of the
posterior pole usually suffers most severely. As the oedema disappears and
is replaced by fibrosis the picture changes to that of a dead-white disc with
sharply defined edges, minute and thread-like arteries and similarly
attenuated veins, so that after a time the vessels can only be traced for a
1 p. 2561. * p. 2549.
T.O.-WOL. II. H. H.

2560 TEXT-BOOK OF OPHTHALMOLOGY
short way beyond the disc where they are continued by white lines, while here
and there, especially along the course of the vessels, lie the remains of old
exudates. The pupil also is dilated and all vision permanently lost, as the
retina has ceased to be a functioning organ but has become little more than
an Organizing scar. -
Finally, in the eye itself an ischaemia of sudden onset occurs in conditions
of occlusion of the central retinal artery ; these will be discussed immedi-
ately. A more gradual anaemia is seen in prolonged disease of the vessel
walls and in all atrophic conditions. In these cases the vessels tend to
become greatly attenuated, their walls become thickened and appear as white
lines bordering the blood-stream until eventually the arteries may be com-
pletely transformed into tenuous white strands or even disappear.
It may be noted in passing that Leber (1915) considered that the lesions of arterio-
sclerotic and renal retinopathy were essentially due to ischaemia resulting from
peripheral arteriolar contraction, while Volhard (1929) attributed to spasmodic
vascular contraction the primary rôle in the genesis of hypertensive (malignant
nephro-sclerotic) retinopathy. Such an ischaemia would involve deficient oxygenation,
and consequently nutritional troubles would be expected to arise in the vessel walls
and the parenchymatous tissues. These questions, however, will be taken up at a
later stage.
Alt. Am. J. O., xxix, 74, 1912. Rnapp. A. f. O., xiv. (1), 218, 1868.
Baas. K. M. Aug., xxxi, 75, 1893. A. f. Aug., v, 203, 1880.
Birch-Hirschfeld. Z. f. Aug., xxiv, 193, 1910. Komoto. T. O. S., xxxv, 295, 1915.
Bistis. A. d’O., xxviii, 34, 1908. Eoyanagi. K. M. Aug., li (1), 623, 1913.
Bonnet and Blanc. Bull. S. d’O. Paris, xlvi, Lauber. XV Internat. Congr. O., Cairo, ii, 17,
52, 1934. 1937.
Calhoun. Georgia Med. J., iii, 74, 1913. Leber. G.-S. Hb. II, vii (a), 45, 1915.
Carl. K. M. Aug., xxii, 113, 1884. Nettleship. O. Rev., xiv, 97, 1895.
Collins. T. O. S., xxxii, 396, 1912; xlvi, 86, Nicholls. Brit. J. O., xxii, 672, 1938.
1926. Pagenstecher. A. f. O., xv (1), 223, 1869.
Elschnig. A. f. Aug., xxiv, 65, 1892.
Fink. Zb. f. Gymäk., xlviii, 1188, 1924.
Görlitz. K. M. Aug., lxiv, 763, 1920.
Gowers. Lancet, ii, 794, 1875.
v. Graefe. A. f. O., x (1), 201, 1864; xii (2),
207, 1866.
Gräfe. A. f. O., viii (1), 143, 1861.
Grimminger. Z. f. Aug., lvii, 106, 1925.
Guthrie and Mayou. T. O. S., xxviii, 104,
1908.
Hairi. Rev. gen. d’O., xxxv, 525, 1921.
Harbridge. Am. J. O., vii, 192, 1924.
Heddaeus. K. M. Aug., iii, 285, 1865.
Begner. K. M. Aug., I (1), 119, 1912.
Hirschberg. B. O. G. Heidel., x, 53, 1877;
xiii, 69, 1881.
Beit. 2. Aug., iii, 18, 1878.
Cb. pr. Aug., xvi, 257, 1892.
Houwer. Ned. Tij. v. Gen., lxix (1), 1667,
1925.
PHutchinson. A. of Surg., iv., 184, 1892.
Jackson. R. L. O. H. Rep., iv, 35, 1864.
Jones. Brit. Med. J., i, 312, 1884.
Ramogawa. K. M. Aug., xcvii, 611, 1936;
xcviii, 54, 1937.
A. f. Aug., xiii, 143, 1884.
Parsons. R. L. O. H. Rep., xv, 362, 1903.
Pincus. A. f. O., xcviii, 152, 1919.
Pines. Brit. J. O., xv, 75, 1931.
Pröll. Diss., Freiburg, 1907.
Puppel. Mon. f. Geburt. u. Gyndik., lxv, 351,
1924.
Raehlmann. Fortsch r. d. Med., vii, 928, 1889.
Rosenstein. K. M. Aug., lxxv, 357, 1925;
lxxviii, 248, 1927.
Schmall. A. f. O., xxxiv. (1), 37, 1888.
Siegrist. B. O. G. Heidel., xxvii, 10, 1898.
A. f. O., l, 511, 1906.
Studer. A. f. Awg., liii, 206, 1905.
Terrien. A. d’O., xxxviii, 263, 1921.
Terson. Am. d’Oc., clix, 23, 1922.
TJhthoff. B. O. G. Heidel.., xliii, 204, 1922.
Volhard. Zb. f. d. ges. O., xxi, 129, 1929.
Wagenmann. A. f. O., xxxvi (4), 1, 1890.
Weiss. Z. f. Aug., x, 16, 91, 1903.
Wirths. Z. f. Aug., xxiii, 506, 1910.
Wordsworth. R. L. O. H. Rep., iv. (1), l l 1,
1863.
Ziegler. Beit. 2. path. Amat., ii, 57, 1887.
DISEASES OF THE RETINA 2561
OBSTRUCTION OF THE RETINAL ARTERIES
The most dramatic and severe type of ischaemia is brought about by an
obstruction of the retinal artery, a condition which may be caused by
several factors acting sometimes singly and sometimes in combination. In
the first place the obstruction may be functional and due to spasm of healthy
arteries; in the second it may be caused by organic disease—either the
sudden lodgment of an embolus in a normal retinal vessel or the closure of a
diseased artery by endarteritis or thrombosis. In all cases the symptoms
come on suddenly ; in some they may be fleeting, so that normal or impaired
vision may be regained ; and in others blindness may be complete and
permanent. Since in all cases the mechanism involved is the same, it will be
convenient to deal first with the general clinical and pathological picture
before studying each condition separately.
The clinical picture presented by a complete obstruction of the retinal
artery is eminently dramatic (Fig. 2157, Plate XLVIII). The larger arteries
are reduced to threads and the smaller vessels may become almost invisible
and sometimes difficult to trace a little way from the disc. The blood-
column, particularly in the veins, is frequently broken up in segments in
the so-called “ cattle-truck ’’ way, a phenomenon usually referred to as bead-
fing or fragmentation, sections of the blood-column moving jerkily and
swinging to and fro in different directions in various parts of the system until
eventually it becomes stationary as a fine, beaded, dark thread." Meantime,
and occasionally with great rapidity, the retina loses its transparency. During
the first day cloud-like striae following the general arrangement of the nerve-
fibres appear in the neighbourhood of and overlying the main vessels, and these
are succeeded during the second day by a denser pallor, the retina becoming
opaque and milky white especially in the central area, so that the outlines
of the disc, which is abnormally pale, become obscured. The most dramatic
effect, however, is seen at the macula, which appears as a bright cherry-red
spot standing out prominently in the hazy white background, around which
minute vessels usually unnoticed ophthalmoscopically, are frequently
rendered visible by contrast.
In some cases, even when a complete obstruction appears to have
occurred, an area of relatively normal retina may remain in the central area.
This is due to the presence of a cilio-retinal artery, which may supply the
macular region and thus preserve central vision (Fig. 2162). Conversely,
in rare cases, a cilio-retinal artery alone may become blocked (Hirsch, 1896;
Meller, 1909; Levy, 1909) (Fig. 2164). In other cases the obstruction may
not affect the main artery, but may be limited to one of its branches, as when
an embolus is arrested at a bifurcation or near the periphery (Fig. 2163); in
this event identical changes are limited to the sector affected. Of all the
* This phenomenon of fragmentation, which is also apparent on death, can be induced
tººls by clamping the arteries and veins supplying the head (Wolf and Davies,
H H 2
2562 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2162.-Embolism of THE CENTRAL RETINAL ARTERY witH RETAINED PATENcy
of A CILLARY RETINAL WEssed.
Fig. 2163–EMBolism or A BRANch ARTERY or the RETINA.
The embolus blocks the inferior branch in which an opaque white plug appears
just at the dise margin.


DISEASES OF THE RETINA 2563
branches the superior temporal is the most frequently involved, a circum-
stance which also applies to venous thromboses (Minton, 1936).
In some cases the obstruction is not complete, in which event the
arteries remain small but can be made to pulsate vigorously on pressure
upon the globe, a phenomenon first noted by Knapp (1869), and usually
associated with an increased beading effect (Friedenwald, 1893). In no case,
indeed, are the vessels ever completely empty, although their occlusion might
be expected as a result of the intra-ocular pressure, for some blood is always
dammed back at the venous exits at the lamina cribrosa where the elastic
fibres constrict the vessel walls. In all cases, moreover, some circulation is
regained, a fact explained by Schnabel and Sachs (1885) by postu-
lating an incomplete blockage, but more probably due to the relieving effect
of the anastomoses between the ciliary and retinal circulations in the region
of the nerve-head, the normal anastomotic vessels increasing in size and new
º -
-
Fig. 2164.—OBstruction of CILIo-RETINAL ARTERY (Pines, Brit. J. O.).
channels being rapidly formed which may, indeed, be ophthalmoscopically
visible (Knapp, 1869 ; Nettleship, 1891; Gonin, 1905; Coats, 1905–13)
(Fig. 2201). After one or two days the haziness begins to disappear and the
vessels show up more clearly, and eventually after a few weeks the appearance
of the retina gradually becomes normal again ; but although it regains its
translucency, it remains completely atrophic. The vessels remain small,
the reduction being due in part to accommodation to the greatly reduced
blood-flow, and probably largely to nutritional and degenerative changes.
After some months their walls may appear as white lines, probably as a result
of a degenerative periarteritis and periphlebitis explicable on nutritional
grounds; and eventually they may be reduced entirely to white threads.
At this stage the disc has become white and atrophic, while degenerative
patches, sometimes pearly and vesicular and sometimes pigmented, may
appear in the retina, marking the remains of haemorrhages and areas of
gross degeneration (Reimar, 1903).

2564 TEXT-BOOK OF OPHTHALMOLOGY
The pathology underlying these general changes is by no means agreed
upon. Two views have been put forward to account for the milky opacity
of the retina, but the difficulty of obtaining early specimens uncom-
plicated by post-mortem changes has made the conclusions drawn from
pathological examination somewhat inconclusive. Nettleship (1874),
Hirschberg (1884), Manz (1891), Ridley (1895), Nuel (1896), Siegrist (1898),
Früchte (1908), Shoemaker (1908) Rupert (1911) and Meinshausen (1920) all
found changes, which were, however, comparatively slight, indicating a
condition of oedema as well as degeneration in the ganglion-cell layer; they
attributed the opacity essentially to the former. Elschnig (1892), Hancock
(1908) and Coats (1913), however, regarded the retinal opacity as due to
ischaemic necrosis involving albuminous coagulation. The late pathological
picture is that of complete atrophy of the nerve-fibre and ganglion-cell layer with
Fig. 2.165-EMBolism of CENTRAL RETINAL ARTERY.
Showing complete atrophy of the nerve fibres and ganglion-cell layer (Coats,
R. L. O. H. Rep.).
fatty degeneration of the cells and varicose degeneration of the fibres with
relative normality of the outer layers of the retina, which receive their nourish-
ment from the choroid (Fig. 2165) (Coats, 1913; Meinshausen, 1920; Möhr
and Böhm, 1921; Engelbrecht, 1924; Karbe, 1924; Schall, 1925; and others).
Experimentally, both changes of oedema and degeneration have been
described (Birch-Hirschfeld, 1900; Shiba, 1906). While ischaemic necrosis
is probably the cause of the dense white opacity which may appear in the
later stages, several clinical observations make it certain that, in the earlier
phases at any rate, oedema is the causal factor. This is shown by its early
onset; thus de Schweinitz (1912) observed the typical picture with a cherry-
red spot 10 minutes after the initial stoppage, and intervals of 2 to 3 hours
are common (Coverdale, 1929; and others); it is impossible for a nervous
degeneration to become effective so quickly. Moreover, in cases of tem-
porary occlusion, the visualfield corresponding to an area of translucent retina
can be retained (Coverdale, 1929), while full visual recovery may follow the

DISEASES OF THE RETINA 2565
development of the typical fundus picture (Werner, 1931; Young, 1932);
such a recovery from a degenerative process is unthinkable. It would seem,
therefore, that while the third neuron is an early victim of ischaemic necrosis,
the ophthalmoscopic changes are essentially oadematous in mature, and in their
initial stages are reversible. They are due, as we have already discussed," to
the imbibition of water owing to the increased osmotic attraction initiated
by the catalytic breaking down of the retinal tissues by enzymes which
flourish in the acid medium created by the state of anoxaemia, a process which
culminates in autolysis and terminates in complete atrophy. The red spot
at the macula is due to a retained transparency in this region, for the thin-
ness of the tissues limits the Oedema and allows the choroid to shine through.
The symptoms of obstruction of the retinal artery are entirely visual.
In their most dramatic form they com-
prise sudden, complete and permanent
blindness, but if the obstruction is of rela-
tively short duration, a considerable degree
or the whole of vision may be recovered.
Even in the worst cases, however, some
visual field may be retained. Most com-
monly this is near the disc especially on
its temporal side (Fischer, 1891 ; Coats,
1905–13; de Schweinitz and Holloway,
1908), a phenomenon almost certainly due
to cilio-retinal anastomoses or collateral
vessels at the nerve-head (Fig. 2166).
Occasionally, also, some of the peripheral Fig. 2166. Expoliss of CENTRAL
field remains especially on the temporal RETINAL ARTERY.
side, a circumstance which can best be ex- Area of the visual field retained
& e e 14 days after complete obstruction
plained On the assumption that the anterior of the central artery. 10330 white.
retina can obtain sufficient nourishment
from the choroid to maintain its viability (Hancock, 1908).” As a rule the
macula, owing probably to its precarious blood-supply and highly specialized
nature, is early affected, and remains permanently functionless unless the
obstruction has been of comparatively short duration. If the anaemia
exists only for an hour or so, central vision may return (Wood-White, 1882),
an event which still may occur, although exceptionally, after a lapse of some
days (Werner, 1931 ; Young, 1932); but after an occlusion which could
not have lasted longer than five-and-a-half hours, a permanent central
scotoma has persisted while the remainder of the retina has resumed its
function (Coverdale, 1929). When a branch vessel is occluded a sector
defect arises, and it is interesting that it is typically bounded by a straight
line even although the oedematous retina presents an irregular border
(Bedell, 1937) (Fig. 2167). This tendency may even apply to the central
1. * p. 2549.
p. 2547.

2566 TEXT-BOOK OF OPHTHALMOLOGY
region where a paracentral scotoma taking the form of an altitudinal macular
hemianopia may result from the blockage of a branch of the inferior temporal
artery supplying the macula (Butler, 1927).
The complications of arterial obstruction are few. Small haemorrhages
occasionally occur especially near the disc, perhaps the result of the rupture
of newly distended anastomoses. Retinal haemorrhages may also follow the
resumption of the circulation through capillaries damaged by ischaemia.
More profuse and massive haemorrhages have been noted suggesting in their
extent a haemorrhagic infarction (Knapp, 1869 ; Landesberg, 1874;
Löwenstein, 1878; and others); it is probable that they are usually deter-
mined by thrombosis in a stagnant vein obstructing the re-established
circulation (Coats, 1913). A secondary glaucoma has rarely occurred ; in
some cases it has been due to inflammatory and neo-vascular changes at the
FIG. 2167.-Soon after the obstruction. FIG. 2168.—After injection of acetyl-
choline (Campbell Orr).
FIGS. 2167–68.-EMBOLISM OF RT, INF. TEMP. ARTERY.
filtration angle, and in others it has been associated with a co-existent venous
thrombosis.
(a) ANGIOSPASM
Although a spasmodic contraction of the retinal arteries was suspected
by Mauthner (1868), and was suggested as an explanation of their small size
in chronic Bright's disease by Gowers (1876), and despite the fact that it was
described clinically by Raynaud (1874), it is only within comparatively
recent times that it has been accepted as a relatively common cause of
temporary obscurations of vision (AMAUROSIS FUGAX) or even permanent
defects. The condition can, indeed, be produced experimentally by the
injection of adrenalin (Redslob, 1926; Cohen and Bothman, 1927), the
serum of hypertensive individuals (Agatston, 1933), or in susceptible persons
by cold or the inhalation of tobacco smoke (Cusick and Herrell, 1939). The

DISEASES OF THE RETINA 2567
classically accepted opinion was that all arterial obstruction was due to
organic processes—embolism, endarteritis and thrombosis—but the actual
observation of spasmodic contractions coming on suddenly, of fleeting dura-
tion, appearing and disappearing in different parts of the fundus, associated
with similar vascular phenomena elsewhere, and coming and going within a few
minutes or hours to leave no trace behind, have fully established the condi-
tion as a clinical entity. There is, indeed, a tendency to go to the other
extreme and to consider spasm much more common than organic obstruction
(Abadie, 1925).
AEtiologically cases of retinal angiospasm can be divided into two
classes; those occurring usually in young individuals who frequently present
other evidences of cardio-vascular irritability, and those occurring in the
middle-aged or elderly who have pronounced organic retinal arterial disease
and are usually the subjects of arteriosclerosis or syphilis (Uhthoff, 1925).
The first type is frequently associated with evidences of vaso-motor
instability elsewhere, such as migraine, which probably results from a similar
condition in the brain (Parisotti, 1898; Thompson, 1902; Löhlein, 1922;
Lister, 1931), or menstrual and ovarian disturbances (Rosenstein, 1927;
Marucci, 1936). Reflex factors, such as vaginal douching (Priestley Smith,
1884) or washing out the antrum (Hirsch, 1920; Weiss, 1921 ; Swett, 1935),
or even a change of posture such as bending down (Harbridge, 1906 :
Traguair, 1933) may precipitate an attack. Toxic influences are also a
cause (Davenport, 1931 : Schousboé, 1937), either endogenous toxins as
influenza (Griffith, 1931), malaria (Testelin, 1866; Königstein, 1875;
Landsberg, 1885; Schnabel, 1885; Sédan, 1929) or the toxaemia of preg-
nancy (Mylius, 1928; Freeman, 1933; Selinger, 1937), or exogenous poisons
as tobacco," alcohol % or quinine.” It may occur also in lead poisoning, in a
case of which, after the sudden development of bilateral blindness, Elschnig
(1898) watched spasmodic contractions of the retinal arteries for some
days. In still other cases no evidence of vascular or organic disease can be
found (Ormond, 1918; Traquair, 1933). Quite frequently, however,
evidences of angiospasm in other regions are noted in addition to migraine :
epilepsy (Hughlings Jackson, 1863; Allbutt, 1871), temporary loss of hearing
(Bailliart and Rollin, 1935), hemicrania (Schiff-Wertheimer and Bailliart,
1935), or even generalized spasms in the cortex, meninges and extremities
which have proved fatal, demonstrating an unusually intense excitability
of the entire vaso-motor system (Sédan and Jayle, 1936).
The association of retinal angiospasm with Raynaud's disease is interesting. This,
it will be remembered, is an angiospastic condition of unknown astiology, but usually
considered as a vasomotor neurosis, characterized by the three stages of local syncope,
asphyxia, and eventual gangrene, affecting usually the extremities. An associated
spastic condition of the retina is rare : thus Allen and Brown (1932) noted only 1 case
in 147 subjects. Nevertheless there is a considerable number of cases in the literature
1 p. 3009. 2 p. 3019. 8 p. 3031.
2568 TEXT-BOOK OF OPHTHALMOLOGY
(Stevenson, 1890; Batten, 1910; Weiss, 1912; Shinkle, 1924; Dunphy, 1932;
Bailliart, Tillé and Laignier, 1934; Jean-Gallois and Roulier, 1934 ; Bailliart, 1936;
Anderson and Gray, 1937; Carpenter, 1938; and others). Most of the cases have
been transient and have left little or no sequelae, but occasionally a milky retina, an
atrophic nerve, and a visual acuity reduced to bare perception of light results (Wagener
and Gipner, 1927; Dunphy, 1932; Andersen and Gray, 1937).
The second type associated with Organic vascular disease tends to occur
in elderly persons who have a high blood-pressure. It occurs in arterio-
sclerotics (Wagenmann, 1897) and syphilitics (Kraupa and Hahn, 1921 ;
Werner, 1931 ; Kravitz, 1934 ; Schousboé, 1937 ; and others). In these
subjects migrainous symptoms are absent, but there is a significant associa-
tion with hemi-paresis and angina pectoris (Farid Bey, 1930; Dejean, 1932).
It will be remembered that angiospasm is closely associated with arteriolar
disease and is credited by some authorities as being the essential aetiological
factor in hypertensive retinopathy (Volhard, 1929), and certainly the occur-
rence of acute retinal angiospasm is common in severe hypertensive and renal
disease (Wagener, Barker and Burke, 1933) * : the narrowing or complete
closure may be transient with a return to the normal calibre, but permanent
changes may result if it is too persistent in its duration or too frequent in its
TeCUITTen CeS.
The clinical picture presented by spasm of the retinal arteries depends
on the extent and duration of the occlusion. In the most intense cases the
pupil is dilated and fixed, and the arteries involved are reduced to thin white
lines so narrowed as to be difficult to trace for any distance beyond the
disc. The entire circulation may cease, segmentation of the blood-column
with a beaded appearance in the veins being very apparent, until after some
time the arteries fill up again in a moment and the veins suddenly resume
their normal size. If the condition is prolonged, however, the clinical
picture of obstruction * may appear with a milky-white retina and a cherry-
red spot at the macula, a phenomenon which has indeed been observed
10 minutes after the original stoppage (de Schweinitz, 1912).
These spasmodic constrictions may occur with startling rapidity
(Bardsley, 1917), with great variability as to site (Lange and Lange, 1928),
and with such intensity that the vessel may be ophthalmoscopically invisible
(Wagener and Gipner, 1927). The spasm may behave in a clonic way, the
artery emptying and filling up intermittently ; it may be very temporary
(lasting 20 minutes, Swett, 1935) and recur frequently : thus Bruner
(1921) observed ten different attacks in one day. Sometimes the effect
seems to be delimited at the bifurcations of the arteries. Thus Benson
(1906) observed the bloodless portion to creep slowly up towards the peri-
phery, and when it reached a bifurcation the vessel filled up suddenly so that
the fundus appeared immediately normal. Again, spasmodic waves of
contraction in peristaltic form may run along the arteries producing a
1 p. 2720. 2 p. 2561.
DISEASES OF THE RETINA 2569
bloodless ring of constriction (Wagenmann, 1897; Mylius, 1928); or an
ampulla-like enlargement filled with blood with a constriction at either end
may move peripherally like a wave (Harms, 1906); while Weiss (1912) saw,
as the white streak-like vessels filled up, a club-like swelling at the peripheral
end of the advancing column of blood thrusting in front of it a fine thread-
like prolongation into the white portion of the vessel. Carpenter (1938),
in a case of complete spasm, watched an artery every few moments suddenly
shoot full of blood up to its division, to be followed immediately by another
and then another like the spokes of a cart-wheel, the phenomenon going on
rapidly for over 2 hours, and being still apparent but at longer intervals in
2 weeks’ time. Cases have been observed in which spasms of this nature
recurred intermittently for 2 or 3 years (Bailliart and Rollin, 1935).
The only ocular symptom in such cases is the visual defect which varies
according to the area affected from a partial scotoma to sudden and painless
blindness, an event sufficiently dramatic in those cases where the spasm is
bilateral. Usually the attack is transitory and full restoration of function
occurs (Minton, 1936), a happy resolution which may be attained even after
the development of considerable retinal oedema and the appearance of a
cherry-red spot at the macula (Werner, 1931). Cases have been reported
which show no disability after fifty or more attacks (Traguair, 1933). Fre-
quently, however, if the attacks are of long duration or repeatedly recur,
permanent damage is done (Kravitz, 1934), and in cases which have gone on
for some years, the field may be eventually limited to the fixation point
(Bailliart and Rollin, 1935). In other cases irregular nerve-fibre bundle
defects persist in the field. In the arteriosclerotic cases, of course, the
prognosis is worse, for in these a late re-opening is not always accompanied
by a return of vision ; remaining narrow and thread-like, the arteries
develop a secondary sclerosis and perivasculitis, haemorrhages and exudates
appear, and the nerve becomes atrophic.
(b) EMBOLISM
The first case of embolism of the central artery was described clinically
by v. Graefe (1859), the accuracy of the diagnosis being proved histologically
by Schweigger (1864). For long this was accepted as the usual cause of
occlusion of the retinal artery, and although it was suggested by Loring
(1874), Priestley Smith (1884), Nettleship (1887), and others that many
cases were more easily explicable on the basis of an obstruction due to local
disease in the smaller vessels than to an embolus imported from a distance,
it was not until the work of Reimar (1899), Coats (1905–13) and Harms
(1905–14) that the relative rarity of a true embolus was recognized. At a
later date, as we have also seen, the importance of angiospasm became
appreciated. That cases of embolism are relatively rare is indeed to be
expected when the difficulty of a chance entry into the retinal artery is
2570 TEXT-BOOK OF OPHTHALMOLOGY
remembered in view of the two right-angled bends in its approaches, one at
the origin of the ophthalmic artery from the internal carotid, and the other
where the central artery leaves the ophthalmic or one of its branches.
There is a considerable number of cases in the literature in which the
lodgment of an embolus in a relatively normal retinal artery has been
observed clinically and proved anatomically (Schweigger, 1864; Manz,
1891; Marple, 1895; Velhagen, 1905; Coats, 1905–13; Früchte, 1908;
Engelbrecht, 1924; Karbe, 1924; and others). The mass may entirely
block the lumen of the vessel (Fig. 2169). Alternatively the embolic block
may be partial and the lumen become completely obstructed subsequently
by thrombosis (Fig. 2170). Proximal to the obstruction the artery becomes
collapsed and obliterated by proliferation and adhesion of its endothelium
(Fig. 2.173); the vein also shows concentric narrowing owing to thickening of
its walls; and distal to the obstruction the vessel is occupied by an organized
thrombus in which lumina may ultimately be formed by the entrance of
collaterals (Fig. 21.71).
The most frequent site of embolic obstruction is in the region of the
lamina cribrosa where the artery becomes narrowed just before it enters the
eye. If the embolus finally lodges here, blindness is complete, but not
infrequently it passes on into one or other of the main branches, and
of all the branches the superior temporal is the most frequently involved
(Minton, 1936), so that a partial recovery of the field may result. Here it
may be seen with the ophthalmoscope as a clear-cut opaquely white area in
the artery before which the blood-column stops abruptly (Fig. 2163); it is
interesting that the white body at the site of the obstruction seems larger
than the diameter of the blood-column, its apparent size being probably due
to the resultant opacity in the wall of the vessel (Werner, 1913). Cases,
indeed, have been observed clinically when it has passed to the site of the
first bifurcation where it has remained for some time, and then, perhaps
after dilatation of the vessels by a paracentesis or the inhalation of amyl
nitrite, has passed on to a fork in the far periphery when the resultant blind
sector in the field entails little functional disability (Mules, 1888; Butler,
1927; Coverdale, 1929; Orr and Young, 1935) (Figs. 2167–68). More rarely
the embolus is caught in a small macular branch (Lloyd, 1918; Engelbrecht,
1924; Schall, 1925) or a cilio-retinal artery (Hirsch, 1896; Zentnayer, 1906;
Levy, 1909; Meller, 1909).
As one would expect, emboli may occur simultaneously elsewhere. Thus in the
eye embolic obstruction has been shown anatomically to be associated with a similar
blockage of the vessels of the circle of Zinn in a case of cardiac pseudo-myxoma (Reichling
1934); while simultaneous involvement of the posterior ciliary arteries as well as the
cerebral and brachial arteries was demonstrated by Goldstein and Wexler (1933), the
emboli being detachments from a thrombosis in the innominate artery superimposed
upon an arteriosclerotic lesion of this vessel occurring in a young boy suffering from
chronic lipoid nephrosis. Simultaneous cerebral involvement, producing aphasia or
hemiplegia, is not very uncommon (Sichel, 1872; Gowers, 1875; Elschnig, 1892;
DISEASES OF THE RETINA
2571
Figs. 21.69–73.−OBstruction of CENTRAL RETINAL ARTERY (Coats).
Fig. 21.69. The embolus in the central artery at the level of the lamina:
the two branches of the vein (to the right) are occluded.
- ºss
º
- -- -- - -
7--- - --
- -- - -
- º- Nº.
tº sº º
- "º º º - º - º - - º: º
sº sº
Fig. 2170.-Above the level of the lamina.
The obstruction (A) does not completely block
the vessels, the rest of the channel being
blocked by thrombus (B). The vein lumen
is very small and to it is adherent organizing
thrombus (J).
º-º-º:
º
***sº sº. Yºº,
Fig. 2172.-Higher up the nerve at the
highest point to which the obstruction (A)
reaches; B. thrombus.
Fig. 2171,-Below the level of the ob-
struction. The artery is occupied by organized
thrombus in which two small lumina (D) have
been formed. The central vein (G) is small
but patent, and its inferior temporal branch
is partially thrombosed near the point of
junction.
-- -- - - - -
\º J. "Sº, ºr -º
- ---- - - - - - -
- - - - *- º *-------
- - º **. - - º
- ----- - -- - - -
º * * - . - - - - -
º
- * > .
- -
Fig. 2173.−The artery (to the left) and the
vein above the obstruction. The wall of the
artery is normal; that of the vein is thick and
infiltrated.





















2572 TEXT-BOOK OF OPHTHALMOLOGY
Barms, 1905; Velhagen, 1905; Whiting, 1934; and others), while multiple emboli
throughout the body may occur (Galezowski, 1902; Früchte, 1908).
The origin of such emboli is varied. Most cases show endarteritis (Möhr and
Böhm, 1921 ; Schall, 1925) or arteriosclerotic or atheromatous lesions in the aorta
(Farid Bey, 1931), the innominate (Goldstein and Wexler, 1933), or carotid arteries
(Elschnig, 1892). Fischer (1891), indeed, found that 70% of cases had marked disease
of the heart or larger arteries. Cases of chorea provide most of the examples occurring
in young children (Swanzy, 1875; Leber, 1877; Knapp, 1918; Bride, 1937;
and others). Childbirth is another cause (Knapp, 1873; Snell, 1886; and others),
septic processes (Engelbrecht, 1924; Mylius, 1924), or acute illnesses, as pneumonia
(Barkan, 1873), diphtheria (Davidsohn, 1902), malaria (Bywater, 1922), and so on.
Blood diseases, such as polycythaemia may involve this as a complication (Elschnig
and Nonnenbruch, 1932), and syphilis is a common cause. It has also followed an
arsenical injection (van Lint, 1928).
It is to be noted, of course, that emboli may not always be simple. A malignant
embolus may occur, although very rarely ; * but infective emboli lodge more frequently
in the retinal arteries than in the uveal circulation.” Fat emboli have been observed
after wounds (Hosch, 1906; Utgenannt, 1921 ; Bernhard, 1925); paraffin emboli
after cosmetic operations on the nose and face (Leiser, 1902; Hurd and Holden,
1903 ; Rohmer, 1905; Zahn, 1910); or air emboli (Schapringer, 1904–06; Stargardt,
1913).
(c) ENDARTERITIS AND ARTERIAL THROMBOSIS
A large number of cases of arterial obstruction are undoubtedly due to
a slow and progressive obliteration of the lumen of the artery by a prolifera-
tive endarteritis, the process being usually completed abruptly by a terminal
thrombosis or spasm. Such a sequence was first suggested by Loring
(1874), Priestley Smith (1884) and Nettleship (1887), and its occurrence was
amply proved pathologically by Schnabel and Sachs (1885), Raehlmann
(1889), Wagenmann (1894), Nuel (1896), Siegrist (1898), Haab (1898–1900),
Reimar (1899), v. Michel (1899), Hertel (1901), Galinowsky (1901),
Coats (1905–13), and Harms (1905–14). The obstruction may occur within
the lamina cribrosa (Fig. 2174) or in the retina (Figs. 2309–10). The
lumen of the vessel may be of normal diameter, but may be almost com-
pletely blocked by endarteritic proliferation, a small eccentric cleft being
left through which a precarious circulation can be maintained, which is liable,
however, to be shut off at any time. Probably owing to the maintenance of
this circulation the vessel proximal to the lesion usually remains patent
(Fig. 2175), a condition quite different from that associated with a complete
embolic blockage (Fig. 2.173).
Such a lesion is the most common cause of arterial obstruction
in old people, more particularly when arterial disease is present. Endarteritis
is also probably in question in those patients who give a history of repeated
prodromal obscurations of vision before the final attack of blindness, and
also in those in whom bilateral occlusion occurs, either simultaneously or
1 p. 2863. * p. 2138.
DISEASES OF THE RETINA 2573
Fig. 2.174.—ENDARTERITIC OBSTRUCTIon of THE CENTRAL ARTERY.
At the level of the lamina the artery to the left almost occluded by an endarteritic
nodule; in it the dark granular mass is calcareous, and a small branch is blocked by
endothelial proliferation. The lumen of the vein is encroached upon by fibrous tissue
(Coats).
FIG. 2175.-END ARTERITIC OBstruction of THE CENTRAL ARTERY.
Behind the level of the lamina in the case of Fig. 2174. The arterial wall shows
no endarteritis and the lumen is full of blood. The vein is very small (Coats).


2574 TEXT-BOOK OF OPHTHALMOLOGY
nearly so. The usual clinical picture is that of retinal embolism, but some-
times massive extravasations of blood occur, presenting a picture akin to
that of venous obstruction (Pines, 1929) (Fig. 2176). It is to be remembered,
moreover, from the clinical point of view, that appearances may sometimes
be deceptive, for endarteritic changes may occur without any ophthalmo-
scopic evidence of their presence. Thus in 27 necropsies Bridgett (1926)
found that 7 only were histologically normal, whereas careful ophthalmo-
Fig. 2176-THRombosis of A BRANCH or THE SUP. NASAL ARTERy
(Pines, Brit. J. O.).
scopic examination before death had established the fact that 17 cases had
clinically normal vessels.
A widespread and destructive thrombosis may also occur in young
subjects, sometimes involving both arteries and veins, due to an infective or
toxic peri-arteritis. Thus Bender (1935) reported a case in a woman of 35
who sustained a bilateral complete occlusion of all the retinal arteries and
veins in association with general infective symptoms after the removal of an
infected tooth. Other similar cases show nothing whatever in their history
or general state (Kalt, 1935). The end-result of such a catastrophe, of

DISEASES OF THE RETINA 2575
course, is complete blindness, with an atrophic retina, usually bespattered
with white spots and pigmented areas especially at the posterior pole.
Cases have occurred wherein a traumatic aetiology of the thrombosis seems obvious
(Moorhouse, 1927; Arkle, 1937).
Closure of the retinal arteries as well as the veins by thrombosis have been observed
in cases of THROMBO-ANGIITIS OBLITERANS (BUERGER's DISEASE, 1917), a malady of
obscure aetiology affecting almost exclusively male Russian, Polish or Rumanian Jews
of 30–50 years of age. It is characterized by an acute inflammatory process affecting
all the vascular coats of the arteries and veins, a process which is followed by extensive
thromboses, which, however, may eventually become canalized. The closure is
usually permanent and may be associated with gangrene of the extremities (Gresser,
1932; Lisch, 1937; Uyama, 1937)."
The differential diagnosis between these conditions is not by any means
always an easy matter. Many cases of spasm are obvious, but it cannot be
assumed that every white break in an artery is an organic embolus ; the
subsequent behaviour of the case, however, usually makes the matter clear,
and if the spasm is of sufficient duration to produce permanent defects, the
diagnosis is largely academic. Similarly a diagnosis between embolism,
endarteritis and arterial thrombosis is frequently impossible, although the
type of patient, the presence of arterial disease, either in the eye or elsewhere,
and the history of prodromal attacks are suggestive of the latter process.
The treatment in all cases of arterial obstruction is essentially the same,
and comprises an immediate attempt to restore the circulation by dilating
the artery before retinal ischaemia has advanced to autolysis and necrosis,
followed by any measures which may seem practicable to prevent a recur-
rence of the incident by stabilizing the general vaso-motor system.
Frequently in cases of spasm local treatment is effective ; occasionally in
cases of embolism a vaso-dilatation sufficient to allow the obstruction to pro-
gress to a more peripheral and less dangerous position can be attained ; but in
cases of arterial disease when obliterative endarteritis is present, although
vaso-dilatators may give temporary relief, the patient is in a precarious
state in which little can be done to help him.
Among the simplest and most immediately effective methods of pro-
ducing a vaso-dilatation is the inhalation of amyl nitrite (Samelsohn, 1881)
and the immersion of the patient in a hot bath (Neame, 1933). The retro-
bulbar injection of drugs such as acetyl-choline sometimes, but by no means
invariably, has a beneficial effect (Tirelli, 1931; Tillé and Héry, 1934; Orr
and Young, 1935; and others) (Figs. 2167–68). Tichomiroff (1932) employed
retro-bulbar injections of atropine. As a general vaso-dilatator Viallefont
(1935) suggested cobra venom ; while Gifford and Marquardt (1938) found
1 p. 2602.
T.O.--WOL. III. I I
2576 TEXT-BOOK OF OPHTHALMOLOGY
intravenous injections of typhoid vaccine (10 to 25 millions) or papaverine
hydrochloride (# gr.) most effective. Of the surgical methods, massage of
the globe as originally suggested by Wood-White (1882) and Mauthner (1883)
is the easiest to apply and is sometimes efficacious. A paracentesis which
dilates the vessels by a sudden lowering of the intra-ocular pressure, and
may be re-opened more than once, is, however, of more value (Secondi,
1864–77; Priestley Smith, 1884). v. Graefe (1861) performed an iridectomy,
while the heroic procedure of attempting to move an embolus at the disc by
detaching the internal rectus and massaging the optic nerve was advocated
by Oppenheimer (1926). .
In addition to these local measures, in recurrent spastic cases every
endeavour should be made to decrease the excitability of the vaso-motor
system. Sedatives may help hyper-emotional cases, as well as the
avoidance of psychical traumata. Tobacco should be forbidden and
exposure to cold and fatigue avoided, general metabolic disturbances
should be corrected, and careful observations kept upon the peripheral
circulation.
Abadie. Presse méd., xxxiii, 1026, 1925.
Agatston. Am. J. O., xvi, 327, 1933.
Allbutt. On the Use of the Ophthalmoscope in
Diseases of the Central Nervous System,
1871.
Allen and Brown.
1472, 1932.
Anderson and Gray. A. of O., xvii, 662, 1937.
Arkle. T. O. S., lvii, 353, 1937.
Bailliart. Bull. S. d’O. Paris, xlviii, 296, 1936.
|Bailliart and Rollin. Bull. S. d’O. Paris, xlvii,
612, 1935.
Bailliart, Tillé and Laignier.
Paris, xlvi, 17, 1934.
Bardsley. Brit. J. O., i, 239, 1917.
Barkan. A. f. Awg., iii, 175, 1873.
Patten. T. O. S., xxx, 238, 1910.
Bedell. Am. J. O., xx, 237, 1937.
Bender. Am. J. O., xviii, 148, 1935.
Bernhard. Münch. med. W., lxxii (2), 1590,
1925.
Birch-Hirschfeld. A. f. O., i, 166, 1900.
Bride. T. O. S., lvii, 346, 1937.
Bridgett. Am. J. O., ix, 725, 1926.
Bruner. Am. J. O., iv, 503, 1921.
Buerger. Am. J. Med. Sc., cliv, 319, 1917.
Butler. Brit. J. O., xi, 559, 1927.
Bywater. T. O. S., xlii, 359, 1922.
Carpenter. A. of O., xix, 111, 1938.
Coats. R. L. O. H. Rep., xvi, 262, 1905;
xix, 45, 71, 78, 1913.
T. O. S., xxxiii, 80, 1913.
A. f. O., lxxxvi, 341, 1913.
Cohen and Bothman. Am. J. Phys., lxxx,
665, 1927.
Coverdale. Brit. J. O., xiii, 529, 1929.
Cusick and Herrell. A. of O., xxi, 111, 1939.
Davenport. T. O. S., li, 115, 1931.
Davidsohn. Diss., Freiburg, 1902.
J. Am. Med. As., xcix,
Bull. S. d’O.
Dejean. A. S. Méd. biol. Montpellier, xii,
505, 1932.
Dunphy. T. Am. O. S., xxx, 420, 1932.
Elschnig. A. f. Aug., xxiv, 65, 1892.
Wien. med. W., xlviii, 1305, 1411, 1898.
Elschnig and Nonnenbruch. K. M. Aug.,
lxxxviii, 433, 1932.
Engelbrecht. Z. f. Aug., lii, 85, 1924.
Farid Bey. Brit. J. O., xiv, 402, 1930; xv,
467, 1931.
Fischer. Ueber die Embolie d. Art. cent. Ret.,
Leipzig, 1891.
Freeman. Am. J. O., xvi, 341, 1933.
Friedenwald. O. Rev., xii, 161, 1893.
Früchte. K. M. Aug., xlvi (1), 245, 1908.
Galezowski. Rec. d’O., xxiv, 273, 1902.
Galinowsky. A. f. Aug., xliii, 183, 1901.
Gifford and Marquardt. A. of O., xxi, 211,
1938.
Goldstein and Wexler. A. of O., x, 70, 1933.
Gonin. Am. d’Oc., czzxiii, 167, 1905.
Gowers. Lancet, ii, 794, 1875.
Brit. Med. J., ii, 743, 1876.
v. Graefe. A. f. O., v. (1), 136, 1859; viii (1),
143, 1861.
Gresser. Am. J. O., xv, 235, 1932.
Griffith. T. O. S., li, 122, 1931.
Haab. Cor. f. Schw. Aertz, xxviii, 338, 1898.
B. O. G. Heidel., xxviii, 209, 1900.
Hancock. R. L. O. H. Rep., xvii, 428, 1908.
Harbridge. Ophthalmology, ii, 647, 1906.
Harms. A. f. O., xli., 1, 245, 1905; lxxxiv,
104, 1913; lxxxvii, 334, 354, 1914.
B. O. G. Heidel.., xxxiii, 253, 1906.
Hertel. A. f. O., lii, 191, 1901.
Hirsch. A. f. Aug., xxxiii, Erg., 139, 1896.
K. M. Aug., lxv, 625, 1920.
Hirschberg. Cb. pr. Aug., viii, l, 70, 1884.
Hosch. A. f. Aug., liv, 162, 1906.
DISEASES OF THE RETINA
2577
IHurd and Holden. Med. Rec., lxiv, 53, 1903.
Jackson. R. L. O. H. Rep., iv., 10, 1863.
Jean-Gallois and Roulier. Bull. S. d’O.
Paris, xlvi, 32, 1934.
Ralt. Bull. S. d’O. Paris, xlvii, 15, 1935.
Rarbe. A. f. Aug., xciv, 190, 1924.
Enapp. A. of O., i, 64, 1869 ; xlvii, 459,
1918.
A. f. Aug., i, 29, 1869; iii, 178, 1873.
Rönigstein. K. M. Aug., xiii, 333, 1875.
FGraupa and Hahn. K. M. Aug., lxvi, 829,
1921.
Fravitz. Am. J. O., xvii, 741, 1934.
Kubik. K. M. Aug., lxviii, 361, 1922.
Landesberg. A. f. Aug., iv, 110, 1874.
Landsberg. A. f. Aug., xiv, 87, 1885.
Lange and Lange. Kl. W., vii, 2286, 1928.
Leber. G.-S. Hb. I, v, 544, 1877; II, vii (A1),
143, 1915.
Leiser. Deut. med. W., xxviii, Vereins-Beil.,
110, 1902.
Levy. T. O. S., xxix, 130, 1909.
v. Lint. A. d’O., xlv., 425, 1928.
Lisch. K. M. Aug., xcix, 812, 1937.
Lister. T. O. S., li, 121, 1931.
Lloyd. Am. J. O., i, 406, 1918.
Löhlein. K. M. Aug., lxviii, 390, 1922.
Loring. Am. J. Med. Sc., lxvii, 313, 1874.
Löwenstein. K. M. Aug., xvi, 270, 1878.
Lundie. O. Rev., xxv, 129, 1906.
Manz. Fest, v. Helmholtz, 1, 1891.
Marple. N. Y. Eye and Ear Inf. Rep., iii, 1,
1895.
Marucci. An. di Ott., lxiv, 671, 1936.
Mauthner. Lhb. d. Ophthal., Wien, 1868.
Allg. Wien. med. Blatter, vi, 258, 1883.
Meinshausen. K. M. Aug., lxv, 199, 1920.
Meller. A. f. O., lxxii, 456, 1909.
v. Michel. Z. f. Aug., ii, 1, 1899.
Minton. P. R. S. Med., xxx, 285, 1936.
Möhr and Böhn. K. M. Aug., lxvi, 812, 1921.
Moorhouse. T. O. S., xlvii, 421, 1927.
Mules. T. O. S., viii, 151, 1888.
Mylius. Z. f. Aug., liv, 157, 1924.
Z. f. Aug., Abhand., x, l, 1928.
Neame. T. O. S., liii, 123, 1933.
Nettleship. R. L. O. H. Rep., viii, 9, 251,
1874; xi, 268, 1887.
Fest. v. Helmholtz, 7, 1891.
Nuel. A. d’O., xvi, 166, 1896.
Oppenheimer. K. M. Aug., lxxvii, 192, 1926.
Ormond. Brit. J. O., ii, 273, 1918.
Orr and Young. Brit. Med. J., i, 1119, 1935.
Parisotti. Am. d’Oc., crix, 321, 1898.
Pines. Brit. J. O., xiii, 161, 1929.
Raehlmann. Fortschr. d. Med., vii, 928,
1889.
Raynaud. A. gén. de Méd., xxviii, 5, 169,
1874.
Redslob. Am. d’Oc., clziii, 169, 1926.
Reichling. B. O. G. Heidel., 1, 329, 1934.
IReimar. A. f. Aug., xxxviii, 209, 291, 1899.
A. f. O., xxxii, 552, 1903.
Bidley. R. L. O. H. Rep., xiv, 265, 1895.
Rohmer. An. d’Oc., czzxiv, 163, 1905.
Fosenstein. K. M. Aug., lxxviii, 248, 1927.
Rupert. K. M. Aug., xlix (2), 721, 1911.
Samelsohn. Cb. pr. Aug., v, 200, 1881.
Schall. K. M. Aug., lxxv, 247, 1925.
Schapringer. Cb. pr. Aug., xxviii, 282, 1904;
xxx, 358, 1906.
Schiff-Wertheimer and Bailliart.
d’O. Paris, xlvii, 615, 1935.
Bull. S.
Schnabel. A. f. Aug., xv, 311, 1885.
Schnabel and Sachs. A. f. Aug., xv, 11,
1885.
Schousboč. Bull. S. d’O. Paris, xlix, 112, 1937.
Schweigger. Vorlesungen ü. d. Gebrauch d.
Augenspiegels, Berlin. p. 140, 1864.
de Schweinitz. T. Am. O. S., xiii, 814, 1912.
de Schweinitz and Holloway. T. Am. O. S.,
xi, 471, 1908.
Secondi. Giorn. d’Oft. It., vii, 97, 1864.
An... dº Ott., vi, 5, 1877.
Sédan. Am... d’Oc., clxvi, 705, 1929.
Sédan and Jayle. An. d’Oc., clxxiii, 609, 865,
1936; clzxiv, 73, 1937.
Selinger. Am. J. O., xx, 56, 1937.
Shiba. A. f. O., lxiii, 393, 1906.
Shinkle. J. Am. Med. As., lxxxiii, 355,
1924.
Shoemaker. T. Am. O. S., xi, 502, 1908.
Sichel. A. de Physiol., iv, 33, 207, 1872.
Siegrist. B. O. G. Heidel., xxvii, 10, 294,
1898.
Smith, Priestley. O. Rev., iii, 1, 1884.
Snell. T. O. S., vi, 341, 1886.
Stargardt. Beit. z. Kl. d. Tub., xxviii, 479,
1913.
Stevenson. Lancet, ii, 917, 1890.
Swanzy. R. L. O. H. Rep., viii, 181, 1875.
Swett. Am. J. O., xviii, 359, 1935.
Testelin. Am. d’Oc., lvi, 317, 1866.
Thompson. T. O. S., xxii, 177, 1902.
Tichomiroff. Sov. vestm. O., i, 98, 1932.
Tillé and Héry. Bull. S. d’O. Paris, xlvi,
290, 1934.
Tirelli. A. d. Ott., xxxviii, 227, 1931.
Traguair. T. O. S., liii, 118, 1933.
Uhthoff. K. M. Aug., lxxv, 469, 1925.
Utgenannt. Z. f. orth. Chir., xli., 393, 1921.
Uyama. A. f. O., czzxvii, 438, 1937.
Velhagen. K. M. Aug., xliii (2), 440, 1905.
Viallefont. A. S. Méd. biol. Montpellier, xvi,
315, 1935.
Volhard. Zb. f. d. ges. O., v, 470, 1921; xxi,
129, 1929.
Wagener, Barker and Burke.
lxxx, 517, 1933.
Wagener and Gipner. Am. J. O., x, 650, 1927.
Wagenmann. A. f. O., x1 (3), 221, 1894;
xliv (2), 219, 1897.
Weiss. B. O. G. Heidel.., xxxviii, 205, 1912.
K. M. Aug., lxvi, 920, 1921.
Werner. T. O. S., xxxiii, 9, 1913;
1931.
Whiting. T. O. S., liv, 270, 1934.
Wolf and Davies. T. O. S., xlviii, 143, 1928.
Wood-White. O. Rev., i, 49, 1882.
Young. Brit. Med. J., ii, 878, 1932.
Zahn. K. M. Aug., xlviii (1), 338, 1910.
Zentmayer. O. Rec., xv, 613, 1906.
Am. J. Med.,
li, 654,
I I 2
2578 TEXT-BOOK OF OPHTHALMOLOGY
RETINAL ARTERIolar THROMBosis
A localized obliteration of a small vessel in the retina by thrombosis
is rarely mentioned in the literature. Localized areas of oedema associated
with vascular disease were noted by
Raehlmann (1902) and Adams (1920)
and were annotated by Friedenwald
(1930) (Fig. 2177). The subjects may
or may not show signs of arterio-
sclerosis; but in the affected region
of the fundus a minute arteriole shows
extreme sclerotic changes, finally be-
coming obliterated as it runs into a
white oedematous area. The affection
comes on suddenly with the appear-
ance of a haze before the eye which
persists as a scotoma; but it is in-
Fig. 2177.-RETINAL ARTERLola R. THROM- - - -
Bosis (Friedenwald, T. O. S.). teresting that despite their apparent
lack of general disease, the majority
of cases die within the next few years from cerebral haemorrhage or uraemia.
Adams. Brit. J. O., iv, 297, 1920. Raehlmann. Z. f. Aug., vii, 425, 1902.
Friedenwald. T. O. S., 1, 452, 1930.
CAPILLARY DISTURBANCEs
CENTRAL ANGIOSPASTIC RETINOPATHY
A peculiarly interesting group of cases occurring in young or middle-aged people,
particularly men, in which an oedema is limited exclusively to the macular region,
which is characterized by recurrent transient attacks of visual failure with complete
or almost complete restoration of function, seems best explained on an angiospastic
basis, the spasm affecting the especially vulnerable area of the central retina. It
may be termed CENTRAL ANGrospastic RETINopathy. There is ophthalmoscopical
evidence of oedema, which is frequently minimal in degree, during the attack with
slight atrophic changes afterwards, there are no evidences of inflammation, the patients
are not the subjects of arterial hypertension, nor are there any general evidences of
disease except frequently vaso-motor lability in the extremities. The oedema may be
sufficient to cause a swelling in the central area surrounded by a ring-shaped light
reflex, and dots varying in colour from white to yellow having a close resemblance
to Gunn’s dots, or pigmentary deposits may be left behind as a legacy.
It is possible that a large number of varying conditions can be correlated into such
an entity; in some the element of spasm seems to be lacking, and it is probable that
capillary damage by toxins produces the same clinical effect. Such conditions
will therefore be discussed together under the heading of central serous retinopathy."
OBSTRUCTION of THE RETINAL WEINs
VENOUS THROMBOSIS : ENDO-PHLEBITIS
Obstruction of the retinal veins was first described and established as a
clinical entity by Julius v. Michel (1878) who realized that many cases
* p. 2592.

DISEASES OF THE RETINA 2579
characterized by gross venous disturbances and profuse retinal haemorrhages
which had hitherto been described as haemorrhagic retinitis or retinal apopleaſy,
were due to this cause. Since that time the matter has been the subject of
almost continuous research, but nevertheless, even to-day, many points both
in the aetiology and in the intimate mechanism of the obstruction are still
unelucidated.
The general mechanism of the occlusion is somewhat complicated
depending usually upon events both in the arterial and the venous parts
of the circulation. It may be described in four stages.
1. The first predisposing factor is a slowing of the blood-flow, a necessary
prelude to the process of thrombosis which was first insisted upon by Leber
(1915). It is usually determined by events both in the arteries, so that the
entrance of blood is impeded, and in the veins so that its exit is obstructed.
Occasionally the entrance flow is diminished owing to a general loss of
vascular tone as occurs in highly toxic or marasmic states, but most usually
it is due to an anatomical constriction of the arteries, either part of general
systemic sclerotic disease or occurring as a local condition. Quite frequently
such a mechanical constriction of the central artery occurs at the lamina
cribrosa by the proliferation of the surrounding connective tissue (Coats,
1904–13 ; Harms, 1905; Scheerer, 1922–25), where, as we have already seen
in considering the question of arterial obstruction, the arteries are normally
constricted and are prone to pathological changes (Fig. 2169). The venous
return is also normally impeded at the lamina cribrosa ; but the important
consideration arises that the veins are so intimately bound up with the
arteries, both at this point and at the arterio-venous crossings in the fundus,
the two, indeed, frequently having a common intervening wall (Fig. 2294)*
(Koyanagi, 1928; Friedenwald, 1930), that any proliferating or sclerosing
pathological process affecting the latter is readily spread to the former
The same process, therefore, tends to slow up the blood-stream by impeding
the arterial flow and damming back the venous return.
A minor accessory cause is an anomalous emergence of the central vein from the
optic nerve. Harms (1905) found that in 10 out of 12 cases examined the vein left
the nerve 13 mm. behind the globe instead of the usual 7–15 mm. As a result of this
anomaly the blood-stream would presumably be further slowed up as it negotiated
two sharp right-angled bends instead of a relatively easy and gradual curve.
2. The second necessity is the formation of a roughened area on the
endothelium upon which deposits may collect. Opinions differ as to the actual
mechanism at this stage, and possibly various factors are contributory in
different cases. The roughening may be due to simple sclerotic changes ;
most commonly it is probably an expression of an endo- or meso-phlebitis
of systemic origin for which no particular toxin but probably a vast number,
including certainly the streptococcus and the treponema pallidum, must be
1 p. 2689.
2580 TEXT-BOOK OF OPHTHALMOLOGY
held responsible; while others believe that the essential process is a simple
endothelial proliferation (Verhoeff, 1906).
3. Upon this area cells and fibrin from the relatively stagnant blood-
stream are deposited so that a nodule or organized thrombus protrudes
into the lumen of the vein, itself damming back the blood-stream to a
greater extent, and gradually growing by accretion until the lumen is reduced
Fig. 21.78–Thrombosis of RETINAL WEIN.
Note exudative spots in area of haemorrhages and the segment of a macular
star indicating oedema. Below and to the right a silver-wire artery (Parsons).
to a mere slit and the circulation brought almost to a standstill (Coats,
1904–13) (Fig. 2180). Alternatively, in the view of others, notably of
Verhoeff (1906–07) who considered that thrombosis in the true sense is exceed-
ingly rare, the endo-phlebitis with proliferative activity of the endothelium
continues to build up a nodule which similarly gradually fills the lumen
(Harms, 1905; Scheerer, 1923; Ineze, 1928). Finally, in a number of cases
the pathology is represented by an acute phlebitis due to some infective
cause (Coats, 1904). In either case the terminal result is the same; the

DISEASES OF THE RETINA 2581
circulation is reduced to vanishing point, total occlusion suddenly occurs,
and the characteristic ophthalmoscopical picture appears in the retina.
The clinical picture thus produced is eminently dramatic and character-
istic, and is essentially the same whether the central vein is occluded at the
lamina and the entire fundus involved (Fig. 21.58, Plate XLVIII), or a branch
vein is occluded (TRIBUTARY THROMBosis) and a sector only is affected
(Fig. 2178). In the first case the veins become engorged, tortuous and enor-
mously dilated, doubling themselves up into great loops, at one spot buried
into the retinal tissues and at another standing out from them. At first there
Fig. 21.79.--THROMBosis of THE CENTRAL WEIN.
Thrombosis of central vein. Longitudinal section from the lamina (A). From
the point backwards the vein is occupied by homogeneous coagulum. At B this
becomes invaded with leucocytes and ends in a sharp point lying free in the centre of
the lumen, above which the vein is normal (x 120) (Coats, R. L. O. H. Rep.).
is some retinal oedema due to a driving out of fluid from the capillaries, but it
is never great in extent and is frequently difficult to observe ; its relatively
small degree is probably due to the approximate equality of the
intra-ocular and venous pressures. It may be sufficient, however, to
produce a star-shaped figure at the macular, and the oedematous fluid may
determine the formation of a macular hole (Williamson-Noble, 1922).
Almost immediately the whole retina becomes profusely bespattered with
haemorrhages, especially in the central region, probably usually capillary in
origin; they are of all sizes, irregular in shape and scattered throughout the
fundus, lying mainly in the nerve-fibre layer but also in the nuclear layers
where they do more damage by ploughing up the tissues (Figs. 2185, 2187).

Figs. 2180 to 2184-Thrombosis or THE CENTRAL vers.
- - - -
º * - º ".
----- _ _ _
º º º º
| - . -
-- A
- -
N N
- - - -
. - - - º
- Fig. 218.1–The same case as
| - Fig. 2180. Restoration of the lumen,
Fig. 21.80.-The lumen of the which is filled with coagulum, into
vein (above) encroached on by which a collateral from the sur-
organized tissue: the artery is rounding connective tissue plunges.
normal (x 120). From this point backwards the vein
was normal (x 120).
-
-
Fig. 21.84-A large lumen restored with an active circulation. The wall is very
Fig. 2182-Endarteritis of the artery Fig. 2183.-Restoration of a small lumen
(left) with eccentric thickening of the in the vein. The walls of which are very
intima. The vein is divided into loculi thick and infiltrated. Some distance behind
containing blood by trabeculae lined by the lamina (x 120).
endothelium and continuous with the
tissue of the wall (x 120).
thick and the vein has separated from the artery, part of which is shown to the left
(x 170) (Coats, R. L. O. H. Rep.).










DISEASES OF THE RETINA 2583
Occasionally a large vessel ruptures and a massive pre-retinal haemorrhage is
formed. Not infrequently after a little time, especially when they begin to
absorb, the haemorrhages become fringed with white, exudative-like areas;
these are probably mainly albuminous coagulates resulting from the oedema-
-
º
--- º *
- - -
- - *". --. is…º.
º: - **** º: º
---
TT 1- .
Fig. 2185.-THROMBosis IN A Fig. 2186.--THROMBosis IN A
RETINAL WEIN. RETINAL WEIN.
Showing abundant haemorrhage Showing results of complete
in retina. The internal nuclear occlusion. Note granules of blood-
layer bowed out (x 100) (Coats, pigment around the vein, and the
R. L. O. H. Rep.). good preservation of the outer
nuclear layer with atrophy of the
layers internal to it (x 170)
(Coats, R. L. O. H. Rep.).
Fig. 2187. VENous THROMBosis.
Showing profuse retinal haemorrhages in the superficial layers (Koyanagi).
tous process, but sometimes represent thickened and degenerated nerve fibres
(Leber, 1915). In the course of time the haemorrhages gradually disappear,
sometimes leaving no trace of their existence, although the exudates usually
persist much longer and occasionally are permanent. Eventually the fundus
may appear relatively normal, although pigmentary changes frequently
persist at the macula, a phenomenon accounted for pathologically by





2584 TEXT-BOOK OF OPHTHALMOLOGY
permanent degenerative and cystic changes in this region which, owing to its
delicacy, is unable to withstand the circulatory disturbance with impunity
(Spicer and Parsons, 1902; Williamson-Noble, 1922–23).
Tributary thrombosis is approximately as common as a complete
thrombosis; in his series of 62 cases Foster Moore (1924) encountered
31 examples of each. Almost invariably they arise at an arterio-venous
crossing where the vein is compressed. In Foster Moore's series 70%
occurred in the superior temporal vein, a proportion substantiated by
Koyanagi (1928).
In Koyanagi's (1928) series of 27 cases, 21 involved the superior temporal vein,
5 the inferior temporal, and 1 both the superior and inferior. This is to be correlated
with the fact that he found a vast preponderance of arterio-venous crossings on the
superior temporal region in normal eyes : in 124 eyes he counted 194 in this region,
80 in the inferior temporal quadrant, and only 79 on the entire nasal half. When a
tributary branch is involved in this way the territory supplied shows the same general
picture, although the changes are less severe, while the remainder of the fundus remains
normal (Fig. 2178). In this case, because of the extensive anastomoses of the deeper
capillaries, the haemorrhages tend to be localized superficially in the nerve-fibre layer
where they do less permanent damage.
The subsequent course of events is usually the same in all cases. The
circulation is restored, to some degree at any rate, comparatively rapidly,
at first probably by communications with adjoining veins through the
capillaries. At a relatively early stage, however, the clot becomes canalized
(Coats, 1904–13) (Figs. 218.1–84), a process which Verhoeff (1907) compared
to the development of a dissecting aneurysm. Meantime small collateral
vessels establish communication on either side of the obstruction, and at a
later stage larger vessels become visible ophthalmoscopically upon the disc
connecting the distal and proximal portions of the vein (Fig. 2159,
Plate XLVIII). These new vessels are always exceedingly tortuous and are
frequently formed in lavish exuberance, sometimes coiling upon themselves
and ending blindly apparently without serving any useful purpose (Batten,
1903; Foster Moore, 1924).
A most interesting feature of such cases is the behaviour of the intra-
ocular tension. At the time of the occurrence of thrombosis, when the
central vein is involved, the intra-ocular tension is usually low, even by as
much as 20 to 30% lower than in the unaffected eye (Foster Moore, 1922;
Wessely, 1935), a phenomenon probably associated with the diminution
of the blood-flow. In cases of tributary thrombosis there is little change.
Subsequently, however, it tends to rise, a phenomenon observed particularly
in those cases where an initial fall is absent. In this event the one complica-
tion which is to be feared after thrombosis is the incidence of a most recalci-
trant haemorrhagic secondary glaucoma, a distressing event which occurs in
so high a proportion of cases within three months of the illness that it cannot
be incidental. It is to be remembered, of course—a fact which is often
DISEASES OF THE RETINA 2585
forgotten—that thrombosis frequently occurs in pre-glaucomatous eyes; the
vascular accident may merely precipitate a pre-destined attack, and, indeed,
the glaucoma may be a factor in determining the venous obstruction. It
may be taken that glaucoma occurs in some 10 to 20% of cases of complete
thrombosis ; but its occurrence in tributary thrombosis is rare.
Leber (1915) found it in 12% of his cases ; Foster Moore (1922–24) in 27.8% ;
Uhthoff (1925) in 13% ; and Gradle (1937) in 8%. Coats (1913) found that the
average interval before the onset of raised tension was 100 days (18 out of 20 occurred
within 15 weeks, the shortest interval was 13 days and the longest 40 weeks); Foster
Moore (1922) found an average interval of 80 days, the shortest being 27.
The cause of the glaucoma has excited a considerable amount of specula-
tion and a large number of observers has studied the histological appearances
presented (Wagenmann, 1892; Weinbaum, 1892; Purtscher, 1896; Alt,
1897; Bankwitz, 1898; Würdemann, 1898; Spicer and Parsons, 1902;
Coats, 1904–13 ; Harms, 1905; Bartels, 1905; Verhoeff, 1907 ;
Tschirkowsky, 1908; Bauer, 1909; Inouye, 1910; Salzmann, 1933;
Samuels, 1935; Krause, 1936 ; and others). Some authors attributed the
rise of tension to physiological causes—a blockage of the drainage angle by
the albuminous nature of the intra-ocular fluids (Coats, 1904), a perivascular
sclerosis forming part of the general sclerotic condition (Fisher, 1925), a
turgescence of the vitreous owing to the absorption of products of haemor-
rhage (Wood, 1932) or of acid tissue-metabolites (Weinstein, 1939), and
so on. It would seem, however, that in addition to the factor of blockage
of the drainage channels at the nerve-head, a great deal of evidence is in
favour of its occurrence as a result of cyto-toxins generated in the damaged
retinal tissues, which at first produce an inflammation at the corneo-iridic
angle (Verhoeff, 1907), and then lead to the formation of extensive adhesions
(Inouye, 1910), and finally highly vascularized membranes over the entire
region of the anterior surface of the iris and the angle of the anterior chamber
(Samuels, 1935). It is the extreme vascularity of these membranes with
their rich complement of neo-capillaries which determines the haemorrhagic
nature of the glaucoma with its constantly recurring hyphaemata, and
also accounts for the frequently disastrous effect which a remedial decom-
pression operation produces. Unable to withstand operative trauma, these
capillaries burst en masse, piling a second haemorrhagic glaucoma upon the
first, making excision of the eye the only practicable treatment.
The symptoms of venous thrombosis at the time of the accident are
confined to visual loss. In a complete thrombosis the loss of sight comes on
rapidly, but not with the dramatic suddenness and completeness which
characterizes an arterial obstruction. If the field is mapped out at an
early stage—which is sometimes difficult—it usually shows considerable
concentric contraction with irregular defects, and almost constantly a central
or para-central scotoma. This, an evidence of the vulnerability of the
macula which is borne out pathologically, is essentially responsible for the
2586 TEXT-BOOK OF OPHTHALMOLOGY
great diminution of visual acuity. As time goes on, even although the
appearance of the retina improves, there is usually little visual amelioration,
but rather a slow diminution until eventually no useful vision may remain,
an unfortunate termination which may be worsened by the extreme pain-
fulness of a subsequent haemorrhagic glaucoma, necessitating excision of the
eye for its relief.
In tributary thrombosis the symptoms are less dramatic, and if the
macular area has escaped, useful central vision may be retained all the time.
When a main vessel is obstructed there is usually a corresponding sector
defect in the field of vision ; but when a more peripheral tributary is affected,
the defect may be small and may seem to show little correspondence with
the topography of the vascular lesion.
AEtiology and Incidence. As will be understood from our discussion of
the mechanism of venous obstruction, two distinct factors enter into the
aetiology of the condition—general vascular sclerosis and toxic factors.
The great majority of cases occur in arteriosclerotics over middle life, not
a few of whom are albuminurics with a high blood-pressure. Taking
the cases as a whole, Coats (1913) found the average age to be 60 with
extremes of 35 and 80, and Foster Moore (1924) an average of 60, 6 cases
only out of 59 being younger than 50. The sexes are equally represented.
In a large number of cases, particularly in the younger ones, a toaſic
element determines the thrombosis by causing an endo-phlebitis. Almost
every variety of toxic condition has been cited, from acute infections such
as septicaemia (Goh, 1897), endocarditis (Brav, 1911), influenza (Coats, 1904;
Jackson, 1920), pneumonia (Purtscher, 1912), meningitis (Metz-Klok, 1924),
or gonorrhoea (Valude, 1911), to chronic diseases such as syphilis. I have
seen one disastrous case after the too liberal removal of septic teeth, and a
bilateral case involving arterial thrombosis as well was reported by Bender
(1935) following a similar operation. There is no doubt that the strepto-
coccus is a common cause, and indeed, it has been recovered from blood-
culture (Greenwood, 1924; Tarun, 1933). Trauma has been cited as a
factor (Cozzoli, 1916) and poison-gas (Saupe, 1919).
Venous obstruction has also been noted in the amaurosis of quinine poisoning
(Parker, 1906)," and forms an element in the catastrophe cf thrombo-angiitis obliterans.
It is probable, also, that the diffusion of toxins posteriorly from the anterior segment
in irido-cyclitis “may occasionally be a causal factor (Williamson-Noble, 1922).
As would be expected in a disease which is usually determined by
systemic influences, venous obstruction is not uncommonly bilateral, either
occurring in the two eyes simultaneously or after an interval; thus Foster
Moore (1924) found 5 cases of bilateral obstruction in his series of 62. More-
over, even when complete obstruction does not occur in the fellow eye the
presence of some circulatory disturbance of the nature of endo-phlebitis is
1 p. 3031. 2 p. 2201.
DISEASES OF THE RETINA 2587
frequently shown by the presence of minute venous haemorrhages, especially
towards the periphery of the fundus.
The prognosis with regard to vision, as we have seen, is not good if the
macula is involved, and this obviously applies to all cases of complete
thrombosis. The prognosis with regard to life is not in question in cases of
acute and incidental phlebitis ; but in the large class of arteriosclerotics the
occurrence is of some vital significance. The prognosis, however, is by no
means so bad as that characteristic of catastrophies in the arterial circulation.
Thus Foster Moore (1924) traced 32 cases for 8 years and found that 15 (41.6%)
had suffered from a gross cerebral vascular lesion which proved fatal in 13 ; 16 were
known to be alive. It is probable that the cases occurring between 50 and 65 years of
age carry the worst prognosis (Straub, 1913): these are liable to die from apoplexy,
coronary disease, or nephritis.
Treatment. The treatment of venous thrombosis is unsatisfactory for
the damage is done initially and quickly. The generally accepted principle
is to prescribe iodides and perhaps mercury in the somewhat illusory hope
that they promote rapid absorption. With this is combined systemic
treatment to combat the general sclerotic and hypertensive condition if
vascular disease is present ; if no special medical treatment is indicated
these people should give up strenuous work and live philosophically in a
simple way. It is well, if no acute fibrile disease is present, to search for and
eliminate focal sepsis, especially of a streptococcal nature—a procedure,
however, which in such subjects can only be safely undertaken gradually
and with great care. Miotics are frequently employed for the first few
months to diminish the tendency to glaucoma—a justifiable procedure which
can do no harm.
Following the experience of Hessberg (1920) in the treatment of retinal haemor-
rhages, irradiation by small doses of X-rays was suggested by Löwenstein and
Reiser (1930): the effect is claimed to be due to the production of a vaso-dilatation,
and a stimulation of thrombolysis and of the formation of vascular anastomoses. Gradle
(1937) considered it had some indefinite value in preventing the development of
secondary glaucoma, and Brophy (1933) that it could lower the tension in such cases ;
but most authorities agree that it has no real value either in hastening absorption
or in preserving vision (Braun, 1937; Ascher, 1937; Gradle, 1937).
Alt. Am. J. O., xiv, 114, 1897. Cozzoli. A. d: Ott., xxiii, 373, 1916.
Ascher. K. M. Aug., xcviii, 401, 1937. Fisher. T. O. S., xlv. 288, 1925.
Bankwitz. A. f. O., xlv. (2), 384, 1898. Friedenwald. Path. of the Eye, Baltimore,
Bartels. Z. f. Aug., xiv., 103, 1905. 1930.
Batten. T. O. S., xxiii, 75, 1903. Goh. A. f. O., xliii, 147, 1897.
Bauer. A. f. Aug., lxiii, 13, 1909. Gradle. Am. J. O., xx, l l 25, 1937.
Bender. Am. J. O., xviii, 148, 1935. Greenwood. J. A. m. Med. As., lxxxii, 92,
Braun. K. M. Aug., xcviii, 401, 1937. 1924.
Brav. Ophthalmology, vii, 258, 1911. Harms. A. f. O., lxi, i, 1905.
Brophy. A. of O., ix, 683, 1933.
Coats. R. L. O. H. Rep., xvi, 62, 1904; 516,
1906.
T. O. S., xxiv, 161, 1904 ; xxxiii, 30, 1913.
A. f. O., lxxxvi, 341, 1913.
Hessberg. K. M. Aug., lxiv, 607, 1920.
Incze. K. M. Aug., lxxxi, 204, 1928.
Inouye. R. L. O. H. Rep., xviii, 24, 1910.
Jackson. Am. J. O., iii, 855, 1920.
Koyanagi. K. M. Aug., lxxx, 436, 1928.
2588
TEXT-BOOK OF OPHTHALMOLOGY
Rrause. A. f. O., cxxxv, 173, 1936.
Leber. G.-S. Hb., II, viiA, 355, 1915.
Löwenstein and Reiser. K. M. Aug., lxxxiv,
230, 1930.
Metz-Klok. Ned. Tij. v. Gen., lxviii (2), 2110,
1924.
v. Michel. A. f. O., xxiv. (2), 37, 1878.
Moore, Foster. T. O. S., xlii, 115, 1922.
Retinal Venous Thrombosis, Brit. J. O.,
Suppl., ii, 1924.
Parker. A. of O., xxxv, 420, 1906.
Purtscher. A. f. Aug., xxxiii, Erg., i, 1896.
K. M. Aug., l (2), 373, 1912.
Salzmann. Glaukom w. Netzhautzirkwlation,
Berlin, 1933.
Spicer and Parsons. T. O. S., xxii, 306, 1902.
Straub. T. O. S., xxxiii, 37, 1913.
Tarun. T. Am. O. S., xxxi, 246, 1933.
Tschirkowsky. K. M. Aug., xlvi (2), 272,
1908.
Uhthoff. B. O. G. Heidel., xlv., 63, 1925.
Valude. An. d’Oc., czlvi, 426, 1911.
Verhoeff. O. Rev., xxv, 353, 1906.
A. of O., xxxvi, i, 1907.
Wagenmann. A. f. O.,
1892.
Weinbaum. A. f. O., xxxviii (3), 191, 1892.
Weinstein. Brit. J. O., xxiii, 392, 1939.
Wessely. K. M. Aug., xcv, 398, 1935.
Williamson-Noble. Brit. J. O., vi, 67, 1922.
xxxviii (3), 213,
Samuels. A. of O., xiii, 404, 1935. T. O. S., xliii, 287, 1923.
Saupe. K. M. Aug., lxiii, 548, 1919. Wood. Brit. J. O., xvi, 423, 1932.
Scheerer. A. f. O., ex, 292, 1922; czii, 206, Würdemann. Beit. 2. Aug., xxix, 100, 1898.
1923; cxv, 370, 1925.
CEDEMA
CEdema of the retina is an extremely common condition, occurring to
some degree in most circulatory disturbances and all inflammatory condi-
tions. In most cases the escape of fluid from the circulation is through the
capillary walls, and since it is probable that these preserve their permea-
bility to a great extent so long as the endothelial cells are healthy, the
appearance of Oedema is usually an indication of capillary or tissue damage.
Simple considerations of pressure have little to do with the matter, for even
in the condition of hyperpiesia the capillary pressure is usually not high and
the oedema is probably the result of the action of toxins ; perhaps the initial
oedema due to venous obstruction is the nearest approach to a pressure-
oedema. The two essential factors, therefore, in the aetiology are :—
(1) An increased transference of fluid to the tissues when the capillary
permeability is increased, as in toxic and inflammatory conditions, a process
which, when accentuated, leads to the escape of albumin and fibrinogen
which coagulate in the form of exudates, and eventually to the escape of the
formed elements of the blood as actual haemorrhages; and
(2) An alteration of the molecular constitution of the retinal tissues
whereby large protein complexes are broken down to smaller entities, as
occurs in conditions of anoxaemia or toxaemia when the tissues are starved by
the loss of their nutrient blood-supply or surfeited with the accumulated
waste products of their deranged metabolism, in which case they imbibe
fluid freely not only from the retinal capillaries but from the chorio-capillaris
and probably from the vitreous as well.
This latter is much the more important factor, for the amount of
fluid which leaves the vessels is determined by the needs of the tissue rather
than the condition of the vessels. The first process is a reversible one ; the
second in its earlier stages is also reversible, but if the tissue-enzymes, acting
freely in the direction of catabolism in the acid media created by the
DISEASES OF THE RETINA 2589
anoxaemic state, continue to break down the tissue-proteins, irrevocable
damage may be done by autolysis until eventually a condition of complete
atrophy results, wherein the retina is represented only by its more resistant
supportive elements.
Histologically the oedematous process is usually first seen in the nerve-
fibre layer since here the majority of the vessels lie (Fig. 2188). The fibres,
normally surrounded by potential spaces only, become separated and the
whole tissue becomes boggy and thickened, a phenomenon most marked
where the layer is thickest—at the optic disc. Similarly, spaces appear in
Nº.
. º º
wº
Tº
Fig. 2188.-RETINAL (EDEMA.
From a case of uveitis in influenza. Note large cystic spaces in inner nuclear layer.
There is an endothelial membrane on the inner surface of the retina (Parsons.)
the ganglion-cell layer so that the cells lie in distended lacunae, and although
in the nuclear layers the elements are more closely linked together, there is
here also a tendency for them to separate in vertical columns following the
general direction of the fibres of Müller. In the internuclear layer the
collections of fluid become more obvious and large cysts are readily formed.
The development of these cystic spaces distorts and mutilates the surround-
ing tissues, and the rods and cones, readily absorbing fluid, become swollen
and homogeneous and finally disintegrate. When the process is confined
to the inner layers, the oedema may pass off without damage; but once the
internuclear layer is seriously involved, permanent damage results.
The aetiology of oedema is extremely varied. So far as circulatory
1 p. 2547.

2590 TEXT-BOOK OF OPHTHALMOLOGY
conditions are concerned, the most dramatic causes are the stasis of venous
obstruction " and the ischaemia of arterial obstruction *; in the latter
especially, as we have seen, the oedema, which is due to the avid imbibition
of fluid by the tissues, may produce a milky opacity of the retina within
a few minutes. A similar, although less intense oedema, is associated
with trauma. Such an oedema follows contusions to the globe and probably
depends, as in the brain, upon a sudden vaso-constriction followed by an
extreme vaso-dilatation, although a haemorrhagic effusion between the choroid
and sclera may also be a factor in its production (Berlin, 1873; Collins,
1917). It is always most pronounced at the macula where it is typified in
BERLIN’s OPACITY (1873) (COMMOTIO RETINAE), an oedematous condition
which tends to clear up after a few days. An electric shock may produce the
same result (Jourdan, 1929). Circulatory conditions associated with the
optic nerve have a similar effect, as is seen to some extent in optic neuritis
and in an extreme degree in papilloedema, a subject which will be discussed at
a later stage. Finally, trauma to the skull may produce a peculiar localized
type of oedema (the ANGIOPATHICA TRAUMATICA OF PURTSCHER, 1910),
occasionally forming a continuous patch round the disc (Ståhli, 1915), but
more usually producing small islands of opacity or more rarely a tongue-
shaped area running from the disc to the periphery in association with a
group of retinal vessels (Purtscher, 1910–13). The explanation usually
advanced is that the oedema is due to a sudden rush of cerebro-spinal fluid
into the eye from the optic nerve sheath, which follows the course of the
perivascular lymph spaces and becomes extravasated into the surrounding
retinal tissues (Purtscher, 1910; Vogt and Knüsel, 1921 ; Vogt, 1923;
Schneider, 1924; Chou, 1928). Others hypothecate the occurrence of small
rents in the retina and vessel walls (Best, 1922; Marchesani, 1925), while
Löwenstein (1936) considered the appearance due to fat emboli. The
question will be discussed further in the section dealing with injuries.
The oedema due to local toacins is associated with all retinal inflammations,
most choroidal ones, and, particularly affecting the posterior pole, with many
cases of irido-cyclitis.” In intra-ocular inflammations, particularly of the
anterior segment, the oedema is frequently limited to the macula, and here
the changes, although localized, may be extreme, leading to the production
of a central scotoma either by the development of cystic degeneration
(v. Hippel, 1906; Coats, 1907; Bane, 1907; Vogt, 1918; Tschenzow,
1923; and others), or even of a macular hole.* As we shall see, it is probable
that the Oedema accompanying hypertension, arteriosclerotic and renal
retinopathies and related states is probably essentially toxic in origin.
Most interest, however, attaches to those quite mysterious cases wherein
an Oedema, usually transitory in nature and limited to the posterior pole,
seems to depend on an extra-ocular infection, such as occurs in sinus infec-
tions or in association with acute dental sepsis or the extraction of a tooth
1 pp. 2578, 2656. * p. 2561. 8 p. 2201. 4 p. 2758.
DISEASES OF THE RETINA 2591
(Bailliart, 1938), in acute febrile diseases, in chronic anaemia or in cachectic
states. It is probable that some of the conditions described together for
convenience under the heading of central serous retinopathy 1 come
under this heading—in fact, in many cases the dividing line is impossible to
draw clinically. In any event it is evident that a transient oedema limited
to the macula and associated with visual disturbances of micropsia, meta-
morphopsia and even a central scotoma, may be associated with toxic states
(v. Graefe, 1866; Fuchs, 1916; Batten, 1921 ; Guist, 1925; Gissy, 1925;
Walsh and Sloan, 1936; Kiewe and Reh, 1936; and others); while the
same consideration probably refers to the central serous chorio-retinitis of
the Japanese.” Usually in these cases the oedema is mild and transitory;
at other times it is well-marked and associated with exudates and haemor-
rhages, and occasionally the formation of a macular star merits the name
neuro-retinitis pseudo-albuminurica stellata º given to it by Leber (1909).
Finally a retinal Oedema occurs of angioneurotic or neurotrophic origin, such as
that described by Castelli (1935) affecting the disc and peri-papillary region
in young people, particularly women, of a vaso-neurotic type. It is probable, also,
that some of the cases described under the heading of central angiospastic retinopathy 4
come under this category.
The clinical picture of retinal oedema in its milder aspects is frequently
difficult to detect. The retina looks thicker and more opaque than normally,
with a somewhat granular appearance, shimmering with irregular
reflexes. These phenomena are accentuated in red-free light, with which
the separation of the nerve fibres near the disc may be seen, and the finer
macular changes more readily recognized (Vogt, 1918). The symptoms
of this mild type may be absent or few, being limited to the appearance
of a translucent yellow veil before the object looked at, a reduction of
adaptation, and the development of a difficultly detectable scotoma
(Evans, 1933). In more advanced degrees the vessels are raised up and
become irregular and sinuous, their reflexes disappearing ; and finally a
diffuse milky translucency may set in as is seen most typically in obstruction
of the retinal artery (Fig. 2157, Plate XLVIII). The symptoms associated
with this type of Oedema may be marked, for visual acuity may fall to lack of
perception of light ; and if the oedema and the factors producing it continue
to operate until degeneration of the ganglion cells and necrosis of these fibres
supervene, vision may be permanently lost.
Bailliart. An. d’Oc., clxxv, 133, 1938. Chou. Brit. J. O., xii, 570, 1928.
Bane. O. Rec., xvi, 245, 1907. Coats. R. L. O. H. Rep., xvii, 69, 1907.
Batten. T. O. S., xli., 411, 1921. Collins. T. O. S., xxxvii, 112, 1917.
Berlin. K. M. Aug., xi, 42, 1873. Evans. Am. J. O., xvi, 417, 1933.
Best. K. M. Aug., lxviii, 725, 1922. Fuchs. Cb. pr. Aug., xl, 105, 1916.
Castelli. Am. dº Ott., lxiii, 561, 1935. Gissy. Z. f. Aug., lvii, 423, 1925.
1 p. 2592. * p. 2594. * p. 2595. * p. 2578.
T.O. —WOL. III. * K K
2592
TEXT-BOOK OF OPHTHALMOLOGY
v. Graefe. A. f. O., xii (2), 114, 1866.
Guist. Z. f. Aug., liv, 37; lv, 269, 1925.
v. Hippel. A. f. O., lxiv, 172, 1906.
Jourdan. An... d’Oc., clxvi, 725, 1929.
Fiewe and Reh. K. M. Aug., xcvi, 448,
1936.
Ruhnt. Z. f. Aug., iii, 105, 1900.
Leber. A. f. O., lxx, 200, 1909.
Löwenstein. K. M. Aug., xcvi, 62, 1936.
Marchesani. A. f. Aug., xcv, 238, 1925.
Nuel. A. d’O., xxviii, 737, 1908.
Purtscher. B. O. G. Heidel., xxxvi, 294, 1910.
A. f. O., lxxxii, 347, 1912.
Cb. pr. Aug., xxxvii, l, 1913. *
Schneider. K. M. Aug., lxxii, 640, 1924.
Ståhli. K. M. Aug., Iv, 300, 1915.
Tschenzow. Russ. O. J., ii, 398, 1923.
Vogt. K. M. Aug., lxi, 379, 1918.
Schw. med. W., liii, 336, 1923.
Vogt and Knüsel. K. M. Aug., lxvii, 513,
1921.
Walsh and Sloan. Am. J. O., xix, 195, 1936.
MACULAR CEDEMA
In the previous section we have seen that one of the sites of election for
retinal oedema is the macula. In states of circulatory oedema this is probably
due largely to the structure of the thick fibre-layer of Henle, which, with its
ability to swell, can absorb large quantities of fluid, a property also shared
by the retina around the disc where the nerve-fibre layer is thickest (Wolff,
1931); a second factor is the avascularity of the central area, for the absence
of capillaries will limit absorption (Iwanoff, 1869). Moreover, in toxic states
the delicate structure of the fovea allows it to succumb readily, especially as
the neural elements are readily exposed to toxins in the vitreous without the
protection of the normal layers (Fuchs, 1901–18). For these reasons, as we
have just seen, it is the favourite site in circulatory, traumatic or toxic
Oedemas. -
An extreme degree of macular oedema, especially when it is localized,
leads to the formation of cystic spaces in the retinal substance, which may
be visible as small flecks in the central area (CYSTIC MACULAR DEGENERATION ;
HONEYCOMB MACULA ; the VESICULAR MACULAR CEDEMA of Nuel). As
degenerative changes proceed these may rupture, and in so doing may
produce a depression in the central area if they are confined to the inner
layers of the retina, or a complete MACULAR HOLE if they involve its entire
thickness (the RETINITIS ATROPHICANs of Kuhnt). Such cysts and holes,
whether of traumatic, degenerative, or toxic origin, will be considered at
a later stage." Two special Oedematous conditions occur, however, which
deserve special mention—Central serous retinopathy and stellate retinopathy.
CENTRAL SEROUS RETINOPATHY
Under the heading of central angiospastic retinopathy we have
already noted a peculiar and characteristic oedema which is limited to the
macular region which is caused sometimes by spasm of the peri-foveal
capillaries and sometimes by toxic processes. It occurs particularly in
young adult males, and appears as an annular swelling of a darkish red
colour round the macula, usually less, but sometimes equal to the size of the
disc. The Oedema is essentially pre-retinal so that the area is raised above
the general retinal level and is surrounded by a ring-shaped light reflex;
1 p. 2755. * p. 2578.
DISEASES OF THE RETINA 2593
moreover, it frequently shows small exudative dots varying in colour
from white to yellow scattered over it. The condition is transient and
the prognosis is relatively good, but if it lasts any length of time, permanent
pigmentary and atrophic changes remain, so that although the final vision
may be 6/6, careful scotometry may reveal a small central scotoma which is
just not sufficient to become manifest in the usual tests for visual acuity, or
a slight distortion remains which may not be noticed unless elicited by
special tests.
A number of conditions almost certainly enter into the aetiology of this
condition, the most common of which is probably capillary damage by toxins.
A study of the literature shows that such a picture—or closely related
pictures—has excited a considerable amount of interest. Thus v. Graefe
Fig. 21.89–CHoRIo-RETINITIs CENTRALIs SERosa (Kitahara, K. M. Aug.).
(1866) and Fuchs (1916) described a “central recurrent retinitis,” suggesting
that the condition was caused by a circulatory disturbance due to syphilis.
Rayner Batten (1921) described a “fairly common "acute primary macular
disease of a transient nature and favourable prognosis which he considered
due to toxic causes. Kraupa (1923) annotated two cases as RETINITIs
CENTRALIS ANNULARIs, attributing the changes to retinalischaemia resulting
from circulatory disturbances. Guist (1925) and Gissy (1925) described a
somewhat similar condition under the name of PRE-RETINAL (EDEMA, and
assumed a tuberculous aetiology. Walsh and Sloan (1936) named a similar
condition IDIopathic FLAT DETACHMENT of THE MACULA and assumed that
it had a toxic aetiology, a conclusion also reached by Kiewe and Reh (1936).
Horniker (1927–37) collected together 43 cases in which he brought forward
ample evidence of vaso-motor instability and suggested the term CENTRAL
R. K. 2


2594 TEXT-BOOK OF OPHTHALMOLOGY
ANGIOSPASTIC RETINITIS, expressing the belief that the condition was much
more common than had been supposed, a view in which he was supported
by Zeeman (1931), Stern (1933) and Cattaneo (1937). Similarly, in France,
related observations were made by Bailliart (1934–38) under the term
CAPILLARITIS, observations confirmed in older people with hypertension by
Héry (1935). Finally, the whole subject was reviewed and the circulatory
instability in the aetiology of many cases established by Gifford and
Marquardt (1938), who, to stress the absence of inflammatory evidences
suggested the term CENTRAL ANGIOSPASTIC RETINOPATHY. My own
impression is that most of the cases are toxic, probably of an allergic nature,
and it may be of some significance that the majority of those which I have
seen have had a highly postive skin-reaction to tuberculo-protein, a reaction
which, of course, need not necessarily be specific.
It is possible that the condition of unknown aetiology called CHORIO-RETINITIS
CENTRALIS SEROSA and described as occurring commonly in Japan, has a similar toxic
or angiospastic basis. Noted first by Asayuma at the end of last century, full reports
have been published by Masuda (1914), Oguchi (1922), Abe (1929), Hasegawa (1932),
Ro (1933) and Kitahara (1933–36), the last of whom collected 100 cases. It affects
preferentially men of middle life, and is ushered in by micropsia, metamorphopsia.
and then the development of a central Scotoma. Ophthalmoscopically there is a round
or oval sharply defined area of retinal oedema in the macular region, surrounded by a
reflex ring, and scattered over with grey-white spots : frequently there is a flat macular
detachment (Fig. 2189). The prognosis, with or without treatment, is favourable,
the fluid being absorbed, the retina returning to place and the vision remaining un-
impaired.
The treatment of these conditions should include an elimination of all
toxic factors, a correction of metabolic disturbances, and a close overhaul of
the reactions of the peripheral circulation with a view to increasing its
stability and maintaining a dilatation of the smaller vessels. These
measures have already been enumerated in discussing the treatment of
obstruction of the retinal artery.”
Abe. K. M. Aug., lxxxiii, 55, 1929.
Bailliart. An. d’Oc., clxxi, 97, 1934 ; blxxv,
133, 1938.
Batten. T. O. S., xli., 411, 1921.
Cattaneo. An. di Ott., lxv, 721, 1937.
Fuchs. Z. f. Aug., vi, 181, 1901.
A. f. O., lxxix, 42, 1911; xcvii, 57, 1918.
Cb. pr. Aug., xl, 105, 1916.
Gifford and Marquardt. A. of O., xxi, 211,
- 1938.
Gissy. Z. f. Aug., lvii, 423, 1925.
v. Graefe. A. f. O., xii (2), 114, 1866.
Guist. Z. f. Aug., liv, 37, 1925; lv, 269, 1925.
Hasegawa.
1932.
Héry. Thése, Paris, 1935.
Acta O. S. Jap., xxvi, 322, 448,
|Horniker. An. di Ott., Iv, 578, 1927.
A. f. O., czziii, 286, 1930.
K. M. Aug., xcviii, 487, 1937.
Iwanoff. A. f. O., xv (2), 88, 1869.
Kiewe and Reh. K. M. Aug., xcvi, 448, 1936.
Kitahara. Acta O. S. Jap., xxxvii, 1122,
1844, 1927, 2094, 1933.
R. M. Aug., xcvii, 345, 1936.
Ro. Acta O. S. Jap., xxxvii, 977, 1933.
Kraupa. Z. f. Aug., i, 335, 1923.
Masuda. Tokio Igakkai Zasshi, xxviii, 267,
1914.
Oguchi. A. f. O., cx, 25, 1922.
Stern. Z. f. Aug., lxxix, 361, 1933.
Walsh and Sloan. Am. J. O., xix, 195, 1936.
Wolff. P. R. S. Med., xxiv, 1617, 1931.
Zeeman. K. M. Aug., lxxxvii, 548, 1931.
1 p. 2575.
DISEASES OF THE RETINA 2595
STELLATE RETINOPATHY
A further type of macular Oedema is characterized by the formation of
a star-figure (STELLATE RETINOPATHY). Here the oedematous retina is
rucked and folded in an arrangement determined by the radiating archi-
tecture of the nerve-fibres in this region, producing a star-shaped figure
in the rays of which brilliant white streaks and spots of exudate may be
deposited. The picture thus produced, especially when associated with
haemorrhages and an Oedematous haziness round the disc, may simulate
closely that of renal retinopathy (the pseudo-albuminuric stellate retinitis
of Leber, 1909)."
Such a condition is caused by any severe disturbance of the capillary
endothelium, whether traumatic, circulatory or toxic. It may follow ocular
(Magitot, 1930) or cerebral traumatism (Nitsch, 1924; Zanettin, 1932).
Circulatory causes include obstruction of a tributary artery or vein
(Parsons, 1907; Williamson-Noble, 1922) (Fig. 2178), retinal peri-phlebitis
(Schieck, 1929), papillitis (Brain, 1934) (Fig. 2557) or papilloedema (Paton
and Holmes, 1911; Colrat, 1930) (Fig. 2545). Toxic causes are more
frequent which act by damaging the capillary walls; these will be discussed
separately at a later stage.” Such a picture is, of course, merely incidental
and is without special significance ; and as a rule it clears up, provided the
retinal circulation can be restored to normal conditions within a reasonable
time.
Brain. T. O. S., liv, 221, 1934. Paton and Holmes. Brain, xxxiii, 389, 1911.
Colrat. A. d’O., xlvii, 773, 1930. Schieck. K. M. Aug., xlviii (2), 150, 1910.
Leber. A. f. O., lxx, 200, 1909. Zb. ges. O., xxi, 1, 1929.
Magitot. Bull. S. d’O. Paris, xlii, 39, 1930. Williamson-Noble. Brit. J. O., vi, 67, 1922.
Nitsch. Z. f. Aug., lii, 229, 1924. Zanettin. A. di Ott., lx, 411, 1932.
Parsons. T. O. S., xxvii, 121, 1907.
RETINAL HAEMORRHAGES
(a) INTER-RETINAL HAEMORRHAGES
In contra-distinction to their frequency in the brain, haemorrhages from
the arteries or arterioles of the retina are rare (Fig. 2193, Plate XLIX). It is
probably only in cases of very advanced local atheromatous degeneration
of the vessei waiis, or in acute cases of Septicaemia and pyaemia when
an embolus lodges within their lumen, that vessels of larger calibre than a
capillary give way. In the first case the haemorrhage may limit itself to a
seepage of blood between the adventitia and the perivascular membrane,
appearing as thin red lines just Outside the vessel, or alternatively the
bleeding may be profuse and plough up the tissues as occurs in thrombosis of
an artery (Pines, 1929) (Fig. 2176). In the second case, when an infective
1 p. 2644. * p. 2644.
2596 TEXT-BOOK OF OPHTHALMOLOGY
process eats through the vessel wall, a sudden and large infiltration of blood
may occur into the tissues or the vitreous.
Haemorrhages from the veins occur when the venous return is sufficiently
obstructed, but to be effective the obstruction must be considerable.
General conditions may be responsible such as coughing, vomiting, or
compression of the chest or neck. Compression of the head at birth is
probably a common cause, the bleeding occurring preferentially in the
macular region where it may be responsible for the development of one type
of congenital amblyopia. Obstruction of the venous exits from the eye is
seen in cases of papilloedema, sub-arachnoid haemorrhage, or thrombosis of
the cavernous sinus; while the obstruction may occur in the retina as when
venous thrombosis occurs. In the absence of obstructive factors, venous
haemorrhage may occur in cases of phlebitis.
It is essentially from the capillaries that haemorrhages of pathological
interest originate, and in their causation it is probable that mechanical
considerations play only a small and unimportant part. It has been claimed
that all retinal haemorrhages are associated with high blood-pressure (Lange
and Lange, 1928), but this view is certainly wrong. A healthy capillary
is an efficient and purposive mechanism capable of responding adequately
to a high pressure and a wide range of stimuli and yet preserves its essential
function of allowing the freest possible interchange between the blood and
the tissues, permitting the passage of diffusible substances in both directions
but retaining the colloid constituents and formed elements of the blood.
This, however, a damaged endothelium can no longer achieve ; and it would
seem that THE ESSENCE OF RETINAL HAEMORRHAGES IS PROBABLY CAPILLARY
DYS-FUNCTION RATHER THAN HIGH BLOOD-PRESSURE ITSELF. In the skin,
for example, where the pressure in the capillaries is normally low, these
vessels are capable of dealing actively with pressures up to 100 mm. Hg :
in the eye, therefore, where they are adapted to a much higher equilibrium
level, it is probable that haemorrhages are not caused essentially by an
increase in hydrostatic pressure, but, together with exudates, are the
result of injurious processes affecting their walls, or are an expression of
defective circulation through them, so that their endothelium is impaired.
This will allow, in one case, the formation of fibrinous exudates, and, in
another, the leakage of whole blood, in a manner analogous to the mechanism
we have seen in a previous chapter to be responsible for the formation of the
plasmoid aqueous. In contra-distinction to the mechanism of the usual
cerebral haemorrhage which occurs in the larger-sized arterioles, and is the
result of a high blood-pressure bursting a vessel weakened by atherosclerotic
changes so that it cannot withstand stress, or, at least, change of stress,
retinal haemorrhages are more of the nature of a capillary leakage, and
appear to have little immediate relation to the height of the pressure in
this part of the circulation, which in these cases is probably frequently
below the normal level.
DISEASES OF THE RETINA 2597
Sudden changes of blood-pressure, however, may be a factor of considerable
importance, and in this connection, when the vascular system is diseased, a fall in blood-
pressure may be more deleterious than a rise (Foster Moore, 1926), probably by deplet-
ing still further an already deficient capillary circulation and lowering the already.
inadequate standard of nourishment. A sudden lowering of the intra-ocular pressure,
as occurs on opening the eye operatively, or the sudden changes that occur in ocular
contusions 1 may produce haemorrhages from purely pressure effects, the walls of the
small vessels being unable to withstand the sudden mechanical stresses to which they
are exposed. Similar haemorrhages may follow indirect injuries, such as a parachute
leap (Colajanni, 1932), or sudden alterations in the intra-cranial pressure as after an
occipital puncture (Klar, 1933).
Pathological capillary haemorrhages may be divided into seven
classes :—
(1) Haemorrhages due to trauma.
(2) Haemorrhages due to obstruction, such as thrombosis, papilloedema,
sub-arachnoid haemorrhage, etc.
(3) Haemorrhages due to local inflammatory conditions in the retina.
(4) Haemorrhages due to toxic states, such as in acute febrile and
infective illnesses or cachectic states.
Of these a long list could be compiled—influenza (Rampoldi, 1890), malaria
(Galezowski, 1876; Harston, 1922; Bywater, 1922), dysentery (Harston, 1922),
variola (Méry, 1904), Weil's disease, glandular fever, and so on.
(5) Haemorrhages occurring in vascular retinopathies—as nephritis,
diabetes, arteriosclerosis, and hyperpiesia.
(6) Haemorrhages occurring in senile atherosclerosis.
(7) Haemorrhages occurring in diseases of the haematopoietic system, as
the anaemias, leukaemias, purpuras, haemophilia, and so on. . Q
The first three of these categories are self-explanatory ; in the fourth the
haemorrhages are due entirely to the action of toxins on the capillary
endothelium ; in the fifth class they are probably also mainly due to the
deleterious action of circulatory toxins and when arteriosclerosis com-
plicates the picture, an impairment of the functional integrity of the endo-
thelium due to deficient circulation, must be added as a contributory cause.
In the sixth class, this last becomes the most important factor ; while the
likeliest cause of the haemorrhages in the last class appears to be a defect in
the nutrition of the endothelium as a result of the depleted quality of blood,
or perhaps from defective oxygenation. None of them is to be construed
as a pressure-extravasation of blood, and the treatment of all of them is to be
directed to the primary metabolic or haematopoietic cause.
The appearance of retinal haemorrhages varies according to the site of
the extravasation of the blood. They are usually most numerous centrally
where the retina is thickest and where the vessels are largest. It is to be
remembered that the vessels do not penetrate more deeply than the external
1 Vol. IV.
2598 TEXT-BOOK OF OPHTHALMOLOGY
molecular layer, the outer layers being avascular; it follows, therefore, that
haemorrhages are confined to the inner (cerebral) layers unless the effusion
of blood is large enough to plough up all the tissues. In the nerve-fibre
layer the shape of the haemorrhage is modified by the arrangement of the
fibres and it appears striate and flame-shaped (Fig. 2193, Plate XLIX); if it
is very superficial it tends to spread out in a fan-shaped area. In the
deeper parts of the retina, on the other hand, the effusion is round and
irregularly shaped, being deeper in the third dimension than are the super-
ficial haemorrhages; these deep haemorrhages are slower of absorption, more
destructive to vision, and entail permanent damage more readily than
do the superficial hºmorrhages in the nerve-fibre layer. In appearance
most haemorrhages are of a homogeneously red colour; but if they are large
and dense they may appear of a slaty blue colour centrally. Occasionally,
---
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-
-
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- - -
- - -
- -- - -
| - - - - -- * --> º -
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-
*
- - |- º º º º
Fla. 21.90.-Rºmunal H ºnton Rhagº.
In venous thrombosis (Inouye, R. L. O. H. Rep.).
also, there are pale areas centrally, which perhaps represent coagula of
fibrin or masses of platelets (Foster Moore, 1916–26).
The fate of retinal haemorrhages is interesting, for it is probable that,
retaining all the time their original colour, they gradually become smaller
and tend simply to fade away. It is easy to calculate the precise time of the
incidence of the retinal haemorrhages which occur in the new-born, the
common occurrence of which both in man and in animals has been definitely
established by Jacobs (1924), Metzger (1925), Juler (1926) and Richman
(1936). The observations of the last of these show that the disappearance
of such a haemorrhage may take place in an intervalso short as four days.
On the other hand, when they are extensive, as, for example, after venous
thrombosis, Foster Moore (1926) has found them visible for months or years.
At the same time, unless the external limiting membrane is lacerated, no
matter how large or profuse they may be, they tend to pass away so com-
pletely that not even pigmentary changes result; those changes which are











DISEASES OF THE RETINA 2599
commonly seen to follow at the macula appear to be of degenerative origin
rather than a residue from the blood (Williamson-Noble, 1923). Similarly
with exudative patches, the larger the mass the longer it takes to become
absorbed ; a small patch may disappear completely in 14 days, while a more
massive one will show comparatively little change for several months. In
each case, of course, fresh haemorrhages and fresh patches are liable to occur,
but the individual extravasations clear up so completely that the part of
the fundus previously involved assumes a normal appearance.
The histological examination of retinal haemorrhages bears out their
irregular clinical behaviour (Fig. 2190). Some are rapidly absorbed and
leave no anatomical traces of their presence ; others, which remain a longer
time, may be associated with scarring, consisting of glial or connective
tissue proliferation. Occasionally small, densely white deposits are seen
around the scars, consisting of cholesterol and other lipoid disintegrative
products of nervous tissue, which again may disappear rapidly or remain for
many months. Occasionally also, particularly in the layer of Henle in the
macular region, cystic spaces of a round or ovoid shape are found filled
initially with serum and lipoid débris, which later may become converted
into hyalin-like material, appearing ophthalmoscopically as small white dots.
Bywater. T. O. S., xlii, 359, 1922. Metzger. Dewt. med. W., li, 1446, 1925.
Colajanni. An. di Ott., lix, 1017, 1932. Moore, Foster. T. O. S., xxxvi, 319, 1916.
Galezowski. Traité, Paris, 1876. Brit. J. O. Mongr. Supp., ii, 1924.
Harston. T. O. S., xlii, 233, 1922. Brit. Med. J., ii, 1097, 1926.
Jacobs. J. Am. Med. As., lxxxiii, 1641, 1924. Pines. Brit. J. O., xiii, 161, 1929.
Juler. T. O. S., xlvi, 42, 1926. Rampoldi. An. di Ott., xix, 70, 248, 1890.
Klar. XIV Internat. Cong., Madrid, i (3), Richman. P. R. S. Med., xxx, 277, 1936.
133, 1933. Stander. Surg. Gym. Obstet., liv, 129, 1932.
Lange and Lange. Kl. W., vii, 2286, 1928. Weekers and Rubin. A. d’O., i, 751, 1933. -
Méry. These, Paris, 1904. Williamson-Noble. T. O. S., xliii, 287, 1923
(b) PRE-RETINAL (SUB-HYALOID) HAEMORRHAGES
When a haemorrhage occurs on the inner surface of the retina and does
not penetrate the vitreous body, it produces a characteristic picture first
figured by Liebreicht (1863) in his Atlas, known as a PRE-RETINAL or SUB-
HYALOID-HAEMORRHAGE (Fig. 2191, Plate XLIX). Such haemorrhages are
found near the posterior pole ; they may be single, or more than one can
occur, and, as would be expected since the superficial retinal vessels are large,
they are usually fairly extensive. Originally they were taken to be arterial
in origin (Nettleship, 1884), but if this is so the arteries must be small ; most
usually they are derived from veins (Fisher, 1912). When the bleeding
occurs the effusion spreads out flat on the retinal surface as a dark red mass
usually of a circular shape or nearly so, but as a rule owing to the action of
gravity it tends eventually to sink and assume a hemispherical shape with a
straight upper margin, which, like a hyphaema, has been noted to alter its
shape with changes in the position of the head (Masselon, 1895). For some
2600 TEXT-BOOK OF OPHTHALMoLogy
time the outlines of the original form may be obvious and as a rule the upper
layers are lighter and less dense than the lower which absorb and disappear
last. On the other hand the effusion may assume irregular and quite
bizarre forms, and may completely embrace the disc (Dimmer, 1894). It is
interesting that at the macula the circular form may be retained, owing,
perhaps, to the closer attachment of the limiting membrane in this region,
the binding down of which may produce a fibrillar star-shaped appearance
delineating the arrangement of the nerve-fibres (Wells, 1890; Obermeier,
1901; Vogt, 1921). The mass of blood, if of any thickness, obliterates
any view of the retina or vessels and produces a complete scotoma, which,
if the macula is involved, abolishes central vision. .
If the haemorrhage is relatively small the blood becomes slowly absorbed,
frequently in a patchy manner from above downwards and vision is com-
pletely restored; it is rare for any remnants, such as white exudates or
glistening crystalline deposits to be left behind (Morton, 1889). On the
other hand, if it has been extensive and absorption has been slow, some degree
of nerve-fibre destruction with subsequent atrophy may ensue. Moreover,
it is to be remembered that such haemorrhages are usually a symptom of
vascular disease of some standing, so that recurrences are common and the
possibility of the advent of other complications should make the relatively
favourable prognosis of the haemorrhage itself more guarded.
The actual site of the haemorrhage has excited some controversy. The
older authors from their clinical observations concluded that it lay between
the retina and the vitreous (Lang, 1888; Morton, 1889; Haab, 1892) ; but
while some pathological studies have shown the blood to lie between the
nerve-fibre layer and the internal limiting membrane (Fisher, 1896;
Benedek, 1906–09 ; Harms, 1908–12, in 2 cases out of 9 ; Klauber, 1909;
Komoto, 1912), in others it has been situated between the limiting membrane
and the vitreous body (Harms, 1908–12, 7 cases out of 9 ; Fleischer, 1914).
The first case to be examined (Fisher, 1896) is probably the most instructive
since it showed blood in both situations and demonstrated what is probably
the common mechanism—the blood is poured out beneath the internal
limiting membrane which it finds little difficulty in stripping off and raising
up so that it forms a film immediately overlying the retina ; sometimes,
however, this membrane may be ruptured in which case the haemorrhage
usually forms a similar film between it and the vitreous; and occasionally
the blood bursts through the face of the vitreous and forms a diffuse opacifica-
tion in the gel (Hesse, 1910; Fisher, 1912).
The causes of pre-retinal haemorrhages are many and various, and
correspond essentially with the causes of inter-retinal haemorrhages. They
may be caused by trauma (Obermeier, 1901; Harms, 1912), even the mild
trauma of coughing (Power, 1888); but the most common factor in the
aetiology is arteriosclerosis. Toxic states, haematopoietic diseases and
menstrual disorders (Watson, 1881; Haab, 1892) are predisposing causes, as
PLATE XLIX
RETINAL HLEMoRRHAGES AND WAscular CHANGEs
Fig. 2191.-SUB-HYALoID HEMORRHAGE. Fig. 2192.-RETINITIs ProLIFERANs.
After repeated haemorrhages.
Fig. 2.194.-ANEurysms. Fig. 2.195. NEw VEssels IN WITREous.
|To face p. 2600.


DISEASES OF THE RETINA 260]
well as mechanical obstruction, as a venous thrombosis, papilloedema, or a
sub-arachnoid haemorrhage.
Benedek. A. f. O., lxiii, 418, 1906; lxx, 274, Lang. T. O. S., viii, 155, 1888.
1909. Liebreich. Atlas. Taf., viii, Ab. 2, 1863.
Dimmer. Beit. z. Aug., xv, 1, 1894. Masselon. La Clin. Opht., i, 19, 1895.
Fisher. R. L. O. H. Rep., xiv, 219, 1896. Morton. T. O. S., ix, 145, 1889.
P. R. S. Med., vi, 21, 1912. Nettleship. T. O. S., iv., 149, 1884.
Fleischer. K. M. Aug., lii, 769, 1914. Obermeier. K. M. Aug., xxxix (1), 293, 1901.
Płaab. Beit. z. Aug., v, 37, 1892. IPower. T. O. S., viii, 21, 1888. -
Harms. B. O. G. Heidel., xxxv, 294, 1908; Spicer. R. L. O. H. Rep., xiii, 352, 1892.
xxxviii, 383, 1912. Vogt. K. M. Aug., lxvii, 331, 1921.
Hesse. Z. f. Aug., xxiv, 327, 1910. Watson. T. O. S., i, 41, 1881.
Rlauber. A. f. O., lxx, 299, 1909. Wells. T. O. S., x, 156, 1890.
Komoto. K. M. Aug., 1 (1), 309, 1912.
(c) VITREOUS HAEMORRHAGES
Any inter-retinal or pre-retinal haemorrhage, if sufficiently profuse, may
burst through the internal limiting membrane and invade the vitreous body.
Here a small haemorrhage is occasionally localized and can be seen ophthal-
moscopically, but the surrounding vitreous always contains exudative
elements; eventually, however, it usually disperses and produces a uniform
vitreous haze. Large haemorrhages which fill the vitreous with blood can only
be suspected when the red reflex of the fundus is abolished in the presence of
a clear lens, although the red mass may be seen in the anterior parts of the
vitreous by oblique illumination or the slit-lamp. Such haemorrhages are un-
accompanied by pain and the Only symptoms are visual. A small haemorrhage
produces a sudden cloudiness of vision, and a massive effusion may produce
a sudden and complete black-out wherein perception of light only remains.
Their fate varies and the prognosis should always be guarded. Frequently
owing to the absence of fibroblasts in the vitreous and their scantiness in
the retina, they clear up uniformly, occasionally with remarkable rapidity ;
at other times an annoying vitreous haze persists indefinitely ; and on other
occasions a massive haemorrhage remains for many months or even per-
manently. Finally, particularly when some mild irritative factor is present
—tubercle, syphilis, nephritis, or chronic sepsis—fibroblastic proliferation
from the retinal blood-vessels is stimulated and organization occurs with the
development of retinitis proliferans."
The treatment of retinal haemorrhages can never be dramatic. Attention
should first of all be directed to the cause and the degenerative or toxic
element combated or eliminated, while an attempt may be made to increase
the coagulability of the blood by the administration of calcium. So far as
local treatment is concerned the primary essential is rest in the early stages
with the avoidance of all strains. It is questionable if local measures such
as sub-conjunctival injections undertaken with a view to stimulating
absorption are of any value.
1 p. 2604.
2602 TEXT-BOOK OF OPHTHALMOLOGY
RECURRENT INTRA-OCULAR HAEMORRELAGES IN YoUNG ADULTS : EALES’
DISEASE
(ANGIOPATHIA RETINALIS JUVENALIS)
It will be convenient to discuss here the vexed question of recurrent
haemorrhages into the retina and vitreous in young adults, but it must be
remembered that it is not a disease-entity but a clinical manifestation of
many diseased conditions. It was first adequately described by Henry Eales
(1880–82) under the title Primary retinal haemorrhage in young men ; he
concluded that it occurred in the absence of obvious constitutional disease
but was associated with constipation and epistaxis, the former being due to
a vaso-motor contraction and subsequent muscular inhibition of the
alimentary tract, and the latter (and the retinal haemorrhages) to a com-
pensatory capillary dilatation. For a considerable time the clinical entity
was usually referred to as haemorrhages of unknown aetiology, until the
researches of Axenfeld and Stock (1911) drew attention to the aetiological
significance of tuberculosis. Since then there has been a tendency, especially
among Continental writers, to ascribe all cases to this cause (tuberculous
retinal periphlebitis), a position, however, which is untenable. Many causes
are operative, among which the following are the most common.
1. Tuberculosis. This is probably the most common cause. The proof
of a tuberculous aetiology was afforded by Gilbert (1935) who found on
histological examination the tubercle bacillus in the neighbourhood of a
vein, and its probability has been suggested many times by the presence of
active tuberculosis elsewhere in the eye (Axenfeld and Stock, 1911; Fleischer,
1914; Mayerhofer, 1927; Lluesma, 1929; Goldstein and Wexler, 1930;
v. Hippel, 1935; Kokott, 1935), or in the body (Igersheimer, 1912; Davis,
1920; Löwenstein, 1931; Gilbert, 1935; and many others). The condition
has, moreover, been experimentally produced after the injection of tubercle
bacilli (Otori, 1915; Finnoff, 1924; Finnoff and Reynolds, 1933; Ohmart,
1933). In some cases the infection is most probably hamatogenous ; in
others a spread backwards from the ciliary body. This subject will be
discussed in detail under the heading of tuberculous peri-phlebitis."
2. Septic foci may undoubtedly be responsible for the condition, for a
considerable number of cases occur wherein the most exhaustive search for
tuberculosis locally or systemically has been negative, while the local
pathology has shown an inflammatory exudate of a nondescript type
(Ballantyne and Michaelson, 1937). These cases will be discussed under the
heading of retinal peri-vasculitis.”
3. Thrombo-angiitis obliterans (Buerger's disease) has been associated
with the condition, the evidence being mainly clinical, but in two instances
pathological (Birnbaum, Prinzmetal and Connor, 1934; Marchesani, 1935).
The clinical evidence is an association with numbness and coldness of the
1 p. 2668. 2 p. 2639.
DISEASES OF THE RETINA 2603
extremities, lack of a peripheral pulse, trophic disturbances and gangrene ;
and the histological picture has shown a narrowing and eventual obliteration
of the vascular lumen by the overgrowth of fibro-plastic tissue between the
elastica and the endothelium.
Other miscellaneous causes have been suggested—helminthiasis (Woods, 1914),
anaemia (Cohen, 1924), a delay in the blood-coagulation time (Wilmer, 1914), endo-
crine derangements and calcium deficiency (Zentmayer, 1920; Wilmer, 1921 ;
Jeandelize, Bretagne and Richard, 1922; Young, 1929); but the feature in most of
the cases is the normality of the general metabolism.
Whatever the aetiology, the clinical picture is much the same. It occurs
in young adults of an average age of 23 years, preponderatingly in males.
One eye is usually affected first and more extensively, and the fact that it
is the left eye (Paton, 1938) suggests a haematogenous origin, this eye being
in the more direct path. The haemorrhages appear suddenly with an obscura-
tion of vision ; they are usually peripheral and the macula is generally
spared ; they may remain intra-retinal and are sometimes associated with
exudates (Cunningham, 1912), but more usually they invade the vitreous,
obscuring all fundus details. Recurrences are the rule, their frequency
varying considerably, so that the vitreous may never be free from haze,
a certain amount of clearance being followed by a renewed extravasation of
blood. After a varying period the haemorrhages may become less frequent,
and for apparently no reason at all may cease altogether ; whereupon the
vitreous slowly clears—a slow and tedious process, so that useful vision may
be recovered after a long time. In other cases secondary glaucoma destroys
the sight rapidly ; and in still others retinitis proliferans " develops, which
may ultimately result in a detachment of the retina. The prognosis,
therefore, should always be guarded.
The treatment is always difficult, and apart from rest, should be directed
to the cause—when this can be determined. When it cannot, it is probably
safest to treat the condition as if it were tuberculous—that is, by tuberculin
and general treatment as already indicated ; * whatever the causal systemic
infection or dyscrasia, careful and controlled hygiene on these lines cannot
be amiss.
More heroic surgical measures, such as ligature of the internal carotid artery
(Axenfeld, 1905), the injection of haemolysin into the vitreous (Elschnig, 1905) or the
aspiration of the clouded vitreous and its replacement by physiological saline (Elschnig,
1912) are rarely indicated. -
Axenfeld. Münch. med. W., lii, 2538, 1905. Ballantyne and Michaelson. Brit. J. O., xxi,
B. O. G. Heidel., xlii, 298, 1920. 22, 1937.
K. M. Aug., lxxix, 66, 1927; lxxxv, 465, Birnbaum, Prinzmetal and Connor. A. Int.
1930. Med., liii, 410, 1934.
Axenfeld and Stock. K. M. Aug., xlix (1), Cohen. A. of O., liii, 362, 1924.
28, 1911. Cunningham. T. O. S., xxxii, 177, 1912.
1 p. 2604. * p. 2315.
2604
TEXT-BOOK OF OPHTHALMOLOGY
Davis. Am. J. O., iii, 657, 1920.
Eales. Birmingham Med. Rev., ix, 262, 1880.
O. Rev., i, 41, 1882.
Elschnig. A. f. Aug., li, 354, 1905.
A. f. O., lxxx, 514, 1912.
Finnoff. Am. J. O., vii, 81, 1924.
Finnoff and Reynolds. T. Am. Acad. Oto-
Lary.., xxxviii, 152, 1933.
Fleischer. K. M. Aug., lii, 769, 1914.
Gilbert. A. f. Aug., lxxi, 5, 1913.
K. M. Aug., xciv, 335, 1935.
Goldstein and Wexler. A. of O., iii, 552, 1930.
v. Hippel. A. f. O., czzxiv, 121, 1935.
Igersheimer. A. f. O., lxxxii, 215, 1912.
Kokott. K. M. Aug., xciv, 327, 1935.
Lluesma. Med. Ibera., ii, 537, 1929.
Löwenstein. Med. Kl., 879, 1931.
JK. M. Aug., xcv, 458, 1935.
Marchesani. A. f. Aug., ciz, 124, 1935.
Mayrhofer. Wien. kl. W., xl, 1199, 1927.
Ohmart. Am. J. O., xvi, 773, 1933.
Otori. A. f. Awg., lxxix, 44, 1915.
Paton. A. of O., xx, 276, 1938.
Wilmer. T. Am. O. S., xiii, 251, 1914; xix,
238, 1921.
Woods. T. Am. O. S., xiii, 254, 1914.
Young. T. Am. Acad. Oto.-Lary., xxxiv, 191,
1929.
Jeandelize, Bretagne and Richard. An. d’Oc., Zentnayer. Am. J. O., iii, 652, 1920.
clix, 655, 1922.
Sequeloe of Retinal Hoemorrhages
We shall now consider two conditions, proliferative and degenerative
in nature, which are essentially dependent on the prior occurrence of retinal
haemorrhages in persons in whom there is cardio-vascular or toxic disease ;
the first is a consequence of haemorrhages on the inner aspect of the retina
and in the vitreous—retinitis proliferans; the second of haemorrhages in
the outer layers of the retina and into the sub-retinal space—retinitis
haemorrhagica externa.
RETINITIS PROLIFERANS
Retinitis proliferans is a purely descriptive term applicable to a number
of different pathological conditions all of which have the common feature that,
following a haemorrhage into the vitreous body, fibrous tissue derived from
the mesoblastic elements associated with the retinal vessels forms prolifera-
tive masses in the vitreous cavity (Fig. 2196). The condition was first
figured in Jäger's Atlas (1869), it was named and its pathology established
by Manz (1876), and to-day its clinical and histological aspects are relatively
well understood.
Some writers (Marple, 1901 ; Klien, 1938) would give the condition a wider
connotation and divide cases of retinitis proliferans into two types: the first, which
forms the typical and classical picture, follows retinal haemorrhages into the vitreous
cavity, and is characterized by the formation, anywhere in the fundus but usually
at the disc, of dense opaque white membranes of fibrous tissue showing little vas-
cularization ; the second, which is dependent on circulatory impairment and degenera-
tive vascular disease and occurs always at the disc, is characterized essentially by the
profuse new formation of blood-vessels associated secondarily with a relatively small
amount of delicate fibrous tissue. The two processes may seem occasionally toºhnerge
the one into the other, but they are essentially different in that the first is purely
degenerative in nature and serves no useful function, while the second has usually a pur-
poseful aim, being an attempt, although often an abortive and misguided attempt, to
DISEASES OF THE RETINA 2605
establish a collateral circulation or to clear away foreign material. We shall therefore
describe the second type under the separate heading of new-vessel formation in the
vitreous.”
AEtiology. The development of retinitis proliferans depends essentially
on two factors—the presence of a haemorrhage on the inner aspect of the
retina and of a sufficient degree of irritation to stimulate a fibrous reaction. If
the vitreous hæmorrhage is not associated with irritative factors, it is
presumably absorbed without exciting any reaction owing to the absence
of fibroblasts in the vitreous and their paucity in the retina. If an extreme
Fig. 2196.-RETINITIs ProLIFERANs (Fleming).
degree of irritation is present, as in panophthalmitis, any attempt at fibrous
tissue reaction is prevented; but if a modest degree is present, retinitis
proliferans ensues. Such optimal conditions prevail in many pathological
states.
1. Trauma is a frequent cause, and is especially evident when the wound
involves the choroid as well as the retina since much more mesoblastic tissue
is available to supply proliferative elements (Parsons, 1903). The process
here has already been described and figured.” It is not at all uncommon,
however, for massive proliferation to occur when the retina alone is involved.
This type of proliferation indeed, especially as seen in war wounds and
1 p. 2618. * p. 2100.

2606 TEXT-BOOK OF OPHTHALMOLOGY
particularly if a foreign body is retained in the eye, can be extraordinarily
profuse.”
It is noteworthy that in new-born children traumatic hamorrhages (which are
common) tend to occur in the external plexiform layer of the retina where the vessels
terminate (Reese, 1936). In adults the haemorrhage, which usually arises from the
larger vessels of the internal layers, effuses into the vitreous where it may give rise to
a retinitis proliferans : but in infants it tends to remain in the outer layers and the
sub-retinal space producing a picture resembling Coats’ disease.”
2. Chronic infective conditions most usually provide the necessary degree
of irritation, and of these the most common are syphilis, wherein massive
haemorrhages are prone to occur (Liebreich, 1863; Masselon, 1883;
Galezowski, 1908; Raia, 1922; Fuchs, 1926; James, 1928; Klien, 1938),3
and tubercle which is characterized especially by the recurrent haemorrhages
associated with tuberculous peri-phlebitis (Fehr 1901; Harms, 1909;
Axenfeld, 1911 ; Fleischer, 1914; Wolf, 1921 ; and others).4
3. Chronic vascular disease which has a toxic basis is frequently present
—arteriosclerosis, nephritis (Wehrli, 1898), diabetes (Fisher, 1898; Raia,
1922), and so on.
Because of its preponderance in tuberculous and early syphilitic cases, most of the
patients are young and of the male sex : thus Guilbaud (1897) who collected 61 cases,
of which 46 were males and 15 females, found most of them between the ages of 20
and 40. A hereditary case involving three generations has been recorded in a haemo-
philic family (Vialet, 1895).
The pathology of retinitis proliferans has excited a considerable amount
of interest since the original paper of Manz (1876): Banholzer, 1892; Denig,
1895; Weeks, 1897; Flemming, 1898; Wehrli, 1898; Römer, 1901;
v. Hippel, 1906; Casali, 1909; Harms, 1909; Neame, 1923; Gallenga,
1925; Collins, 1929; Klien, 1938 (Fig. 2197). The changes are essentially
those which occur in the healing of a wound. Young fibrous tissue grows
over the surface of the retina and out into the vitreous. It is richly cellular
and not infrequently has a stratified appearance, but blood-vessels are
scarce except perhaps in the later stages. As a rule the proliferation is
associated with the disc, probably because here is situated the main mass of
retinal mesoblastic tissue ; and it would appear that a haemorrhage in the
periphery, especially if recurrent, may spread towards the disc and cause a
reaction and proliferation at this point by its toxic effect. In many cases,
however, new fibrous tissue lies along the surface of the retina especially
along the vessels, or is incorporated in its substance, or appears on its outer
surface where it blends with the proliferated pigmentary epithelium as
occurs in Coats’ disease.” In those cases an inflammatory reaction in the
choroid and the destruction of Bruch's membrane indicates that some of the
* See Section on Injuries. 2 p. 2610. 8 p. 2663.
4 p. 2670. * p. 2610.
DISEASES OF THE RETINA 2607
mesoblastic tissue may be derived from this source. It has been contended
that glial new-growth also enters into the proliferative process (Wehrli,
1898; Römer, 1901; and others); but the point is not proven.
Experimental attempts to reproduce the condition have been made on several
occasions by the injection of blood into the vitreous of animals, but in most cases the
- - - -
- --- . - - ---
- - --- --- ------ º . -
- ºº-ººººººººº.
Nº º º: -º-, --, --
º -
ºf - A
- º . . º |-
- - -- - -- --
- - - -
Fig. 21.97.-RETINITIs ProLIFERANs.
From a vitreous hæmorrhage associated with tuberculous periphlebitis. H. Free
haemorrhage in vitreous. F. Fibroblasts. C. Capillaries (Klien, A. of O.).
results have been complicated by the simultaneous production of a wound (Probsting,
1892; Ogawa, 1906; Birch-Hirschfeld and Inouye, 1909; Koyanagi, 1912; Oguchi,
1913; Schreiber, 1913).
The clinical picture presented by such cases is as a rule unmistakable (Figs.
2198–99 and 21.92, Plate XLIX). Usually some time after the haemorrhage,
but on occasion assoon as 70 days (Neame, 1923), veil-likewisps or membranes
may be seen, sometimes stretching across the retina or sometimes floating
forward into the vitreous, and usually starting at or near the optic disc.
They may assume any shape, either appearing as broad flat surfaces or as
compact bands. As they develop they become dense, opaque and white,
and they are usually avascular, blood-vessels, if present, being comparatively
small, few and late in their appearance. Occasionally the fibrosis
progresses until much of the vitreous is occupied by a fibrous mass so that
the eye is excised under the diagnosis of a pseudo-glioma (Neame, 1923).
T.O.-WOL. III. - L. L.



















2608 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2198. Massive. RETINAL HLEMORRHAGE INTo THE WITREous.
In a syphilitic case: “ neuro-retinitis papulosa" (James, Brit. J. O.).
Fig. 2199 –RETINITIs ProLIFERANs.
The same eye as Fig. 219.8, 44 years later (James, Brit. J. O.).


DISEASES OF THE RETINA 2609
The only symptom is the effect on vision, which is variable and depends
essentially on the position of the membranes. If they are peripheral and
avoid the macula, the condition may be unnoticed by the patient ; but if
they occupy the central area, fixation is lost. Occasionally partial absorp-
tion takes place with an improvement of vision (Hickman, 1902; Goldsmith,
1904), and exceptionally a rupture or splitting of the membrane may cause
a sudden and dramatic improvement ; but in the usual case the fibrotic
process is progressive and vision gradually and very slowly deteriorates.
The prognosis is therefore bad ; and a sudden calamitous ending may be
brought about by glaucoma, or much more commonly, by a detachment of
the retina caused by cicatricial contraction of the fibrous bands.
In the diagnosis distinction should be made between a true retinitis proliferans
and the organization of post-inflammatory fibrous tissue such as appears in cyclitic
membranes + or follows a chorio-retinitis and tends to result in phthisis bulbi. External
exudative retinitis (of Coats),” where organization forms externally to the retina, is a
closely related condition and, indeed, the two may occur together, membranes being
sometimes superficial and sometimes deep to the retinal vessels. In cases of new-
vessel formation in the vitreous * a distinction is sometimes difficult to draw when
much fibrous tissue is present ; indeed, ophthalmoscopically the two conditions may
merge almost imperceptibly into each other.
Treatment is unsatisfactory and it is probable that local measures are
useless : sub-conjunctival injections, styptics, fibrolysin and so on. The
only course which may tend to stop the progressive fibrosis is to attempt to
combat the causal toxaemia, but even in those cases where this can be done
relatively efficiently, the results are by no means encouraging.
A somewhat similar proliferative reaction may follow cases of severe choroiditis
when a pre-retinal fibrous membrane may be formed apart from the choroidal lesion
at the posterior pole (Knapp, 1937).”
Axenfeld. K. M. Aug., xlix (l), 28, 1911. James. Brit. J. O., xii, 313, 1928.
Banholzer. A. f. Aug., xxv, 186, 1892. Klien. A. of O., xx, 427, 1938.
Birch-Hirschfeld and Inouye. A. f. O., lxx, Knapp. A. of O., xviii, 558, 1937.
486, 1909. Koyanagi. K. M. Aug., 1 (2), 722, 1912.
Casali. Am... di Ott., xxxv, 191, 1906 ; xxxviii, Liebreich. Atlas, 1863.
897, 1909. Manz. A. f. O., xxii (3), 229, 1876; xxvi (2),
Collins. T. O. S., xlix, 166, 1929. 55, 1880.
Denig. A. f. Aug., xxx, 312, 1895. Marple. T. Am. O. S., ix, 254, 1901.
Fehr. Cb. pr. Aug., xxiv, 193, 1900; xxv, Masselon. Mémoires d’O., Paris, 11, 1883.
2, 39, 1901. Neame. T. O. S., xliii, 296, 1923.
Fisher. T. O. S., xviii, 150, 152, 1898. Ogawa. A. f. Aug., lv., 91, 1906.
Fleischer. K. M. Aug., lii, 769, 1914. Oguchi. A. f. O., lxxxiv, 446, 1913.
Flemming. T. O. S., xviii, 154, 1898. Parsons. R. L. O. H. Rep., xv, 215, 1903.
Fuchs. Z. f. Aug., lix, 213, 1926. Pröbsting. A. f. O., xxxviii (3), 114, 1892.
Galezowski. Rec. d’O., xxx, 313, 1908. Raia. Am. J. O., v, 946, 1922.
Gallenga. Bol. d’Oc., iv, 486, 1925. Reese. Am. J. O., xix, 576, 1936.
Goldsmith. T. O. S., xxiv, 117, 1904. Römer. A. f. O., lii (3), 514, 1901.
Guilbaud. Thése, Paris, 1897. Schreiber. B. O. G. Heidel.., xxxix, 348, 1913.
Harms. B. O. G. Heidel., xxxvi, 352, 1909 Vialet. Rec. d’O., xvii, 321, 1895.
Herford. Cb. pr. Aug., xxxi, 203, 1907. Weeks. T. A. m. O. S., xxxiii, 158, 1897.
Hickman. T. O. S., xxii, 166, 1902. Wehrli. A. f. Aug., xxxvii, 173, 1898.
v. Hippel. A. f. O., lxiv, 157, 1906. Wolf. A. f. Aug., lxxxix, 54, 1921.
Jäger. Atlas. Taf. xviii, 142, 1869.
1 p. 2170. * p. 2610. 8 p. 2618. 4 p. 2192.
L L 2
26.10 TEXT-BOOK OF OPHTHALMOLOGY
ExTERNAL HAEMORRHAGIC RETINITIs (CoATs)
In 1908 George Coats reported the pathological examination of 6 cases
and collected 48 in the literature showing certain characteristic features in
common—the occurrence, usually uni-ocularly, in young people particularly
of the male sex and in perfect health without significant personal or family
history, of large masses of white or yellow exudation in the fundus, lying
underneath the retinal vessels and frequently besprinkled with cholesterol-
Fig. 2200.-H.E.MoRRHAgio Exudative, RETINITIs : Coats' Disease.
A. Cavity containing débris and fibrin (F). B. The overlying retina thickened
and degenerated. G. A knot of dilated vessels. C. Organizing fibrous tissue invading
the mass. D. Sub-retinal exudate containing “ghost-cells.” E. Choroid, which
is normal (x 34) (Coats, R. L. O. H. Rep.).
like crystals and haemorrhages. The commencement of the disease is
usually unnoticed, and its course is slow ; sometimes vision is lost
from a detachment of the retina, from secondary cataract, or glaucoma,
and in other cases quiescence is attained without the development of these
untoward accidents. Coats assumed on the evidence of an early case that
the disease was the result of haemorrhage from the outermost vascularized
layer of the retina—the outer reticular ; that the outer layers were broken
through, causing a detachment of the retina; that in the sub-retinal space

DISEASES OF THE RETINA 261 I
the blood broke down with the formation of débris, fibrin and cholesterol:
that the irritation set up by this process in the surrounding tissue led to the
formation of granulation tissue and its encapsulation, associated with an
irritation of the choroid and a proliferation of the pigment epithelium
(Fig. 2200). At a later date (1912), in describing 4 additional cases, he still
maintained that the primary feature of the disease was haemorrhages in the
outer layers of the retina and thence into the sub-retinal space, but recog-
nizing the exudative and inflammatory nature of the condition, suggested
the term retinitis eacudativa. Three years later Leber (1915) suggested that
the principal factor was inflammatory and exudative and that the haemor-
rhages were incidental and secondary, and proposed the term sero-fibrinous
degenerative retinitis. Since then our knowledge has progressed considerably,
and to-day it is obvious that “ Coats’ disease ’’ is by no means a clinical
entity but embraces a considerable number of conditions (Feingold, 1924;
Heine, 1926; Wöfflin, 1926; Rebay, 1927; Zinsser, 1929; Junius, 1929–34;
v. Hippel, 1931 ; and others).
Such cases with massive exudation between the retina and choroid
may be classified as follows:–
1. ExTERNAL HAEMORRHAGIC RETINITIS (OF COATs), haemorrhagic cases
in which the element of haemorrhage seems undoubtedly to be primary and
the disease seems essentially vascular in origin (Coats, 1908; Crigler, 1920;
Demaria, 1921 ; Rados, 1921 ; Gourfein-Welt, 1922; Doesschate, 1927).
2. ExTERNAL ExUDATIVE RETINITIS (OF COATS) (SERO-FIBRINOUS
DEGENERATIVE RETINITIS OF LEBER) wherein haemorrhagic extravasations
are absent or minimal and the disease is toxic in origin and vascular in
nature (v. Hippel, 1913–31 ; Leber, 1915; Jervey, 1919; Hanssen, 1920;
Meller, 1921 ; Davis, 1921 ; Wöfflin, 1926; Marshall and Michaelson,
1933; Lamb, 1938).
3. ANGIOMATOSIS RETINAE (OF V. HIPPEL AND LINDAU); (MASSIVE
ExUDATION witH ARTERIO-VENOUS COMMUNICATION OF COATS); a congenital
disease of a neoplastic angiomatous nature which in its later stages has
exudative tendencies.
4. RETINAL DEGENERATION WITH MULTIPLE MILIARY ANEURYSMS (OF
LEBER)—a disease of unknown aetiology, perhaps toxic, perhaps degenerative,
and perhaps related to the angiomatous condition preceding."
5. Cases due to disturbances in the choroidal circulation. The majority
may be termed SENILE ExUDATIVE CHOROIDITIS (sometimes called the
SENILE FORM OF COATs’ DISEASE). Here the sub-retinal exudation is
derived from arteriosclerotic vessels in the choroid. The location of choice
is the posterior pole, and the condition has already been described as
disciform degeneration at the macula (of Kuhnt and Junius).” More rarely
it occurs in younger people owing to some obstruction in the choroidal
circulation such as thrombosis of the vortex veins (Mayou, 1915–28).
1 p. 2616. * p. 2116.
2612 TEXT-BOOK OF OPHTHALMOLOGY
6. Traumatic cases occurring in new-born infants, in whom a haemor-
rhage occurs preferentially from the outer plexiform layer and bursts
through to the sub-retinal space to form a typical picture of Coats’ disease
(Greeves, 1911 ; Reese, 1936). It will be recalled that in adults the site of
election of a traumatic haemorrhage is from the large vessels of the nerve-
fibre layer and the blood extravasates towards the vitreous.
Clinically the haemorrhagic variety is indistinguishable from the inflam-
matory, and pathologically the exudative type excites a similar tissue
reaction ; the detailed description of the condition will therefore be con-
sidered under the heading of exudative retinitis.” The other conditions are
described in their appropriate sections.
Coats. R. L. O. H. Rep., xvii, 440, 1908. Junius. Z. f. Aug., lxviii, 207, 1929.
A. f. O., lxxxi, 275, 1912.
R. M. Aug., lxxxvi, 577, 1931; xcii, 748,
Crigler. A. of O., xlix, 287, 1920. 1934.
Demaria. Rev. Asoc. Med. Argent., xxxiv, A. f. Aug., cvi, 475, 1932.
237, 1921. Lamb. Am. J. O., xxi, 618, 1938.
Davis. T. A. m. O. S., xix, 222, 1921. Leber. G.-S. Hb., II, vii A, 20, 1267, 1915.
Doesschate. K. M. Aug., lxxix, 505, 1927. Marshall and Michaelson. T. O. S., liii, 102,
Feingold. T. Am. O. S., xxii, 268, 1924. 1933.
Gourfein-Welt. Rev. gem. d’O., xxxvi, 149, Mayou. T. O. S., xxxv, 107, 1915; xlviii,
1922. 150, 1928.
Greeves. O. Rev., xxx, 349, 1911. Meller. Z. f. A ug., xlvii, 247, 1921.
Hanssen. K. M. Aug., lxv, 703, 1920. Rados. A. f. O., cv, 973, 1921.
Heine. Z. f. Aug., lx, 1, 1926. Rebay. A. de Oft. B. A., ii, 577, 1927.
v. Hippel. A. f. O., lxxxvi, 443, 1913; Reese. Am. J. O., xix, 576, 1936.
Wöfflin.
Zinsser.
cxxvii, 27, 1931.
A. f. O., cyvii, 33, 1926.
Jervey. Am. J. O., ii, 127, 1919.
A. f. O., csxi, 686, 1929.
B. Anomalies of the Blood-Wessels º
1. TORTUOSITY OF THE BLOOD-VESSELS
In addition to the congenital anomaly met with frequently in hypermetropic
eyes and, indeed, to a great extent in a large number of normal eyes (Gauss, 1930),
tortuosities and varicosities of the retinal arteries and veins are a relatively common
result of pathological conditions of prolonged stasis. A tortuosity of the smaller retinal
vessels is seen most commonly in arteriolar sclerosis (Thoma, 1889; Friedenwald,
1930; and others), a phenomenon frequently important and obvious in the peri-
macular region (de Schweinitz, 1906) * (Fig. 2301).
A greater degree of tortuosity, however, may be evident in the veins, where a
varicose condition appears after venous thrombosis, affecting especially the new
vessels formed to attempt to achieve a collateral circulation. This phenomenon,
first described by Axenfeld (1896) and Elschnig (1898), has already been noted *
(Fig. 2159, Plate XLVIII) and is well recognized (Batten, 1903; Doyne, 1904; Pollock,
1907; Fuchs, 1910; Foster Moore, 1924): the most complicated and apparently pur-
poseless convolutions may be found with elaborate glomerular-like tufts and capillary
nets. A similar phenomenon may follow retinal haemorrhages (Gunn, 1891; Friedenwald,
1896), or be found at the disc in chronic glaucoma (Hormuth, 1903). A general tor-
1 p. 2648. * For congenital anomalies see Vol. II, p. 1386 et. Sea.
8 p. 2691. * p. 2584.
DISEASES OF THE RETINA 2613
tuosity of the vessels may be associated with an intra-cranial cirsoid aneurysm (Wessely,
1933) and, of course, is extremely marked in the new-formation of exudative retinitis
or angiomatosis.
Congenital malformations of the veins may occur, wherein they become extremely
tortuous and dilated (Krug and Samuels, 1932). Thin angiomatous malformations
may be considered, with arterio-venous aneurysms 4 as a tumour of the blood-vessels,
but they must be sharply distinguished from the haemangioblastomas or true neoplasms
of the blood-vessels which will be discussed at a later stage.” The two types may be
clearly distinguished by the fact that the former do damage only by the mechanical
compression of the nerve-tissue between the vascular loops.
Axenfeld. Ber. kl. W., xxxiii, 925, 1896. Hormuth. K. M. Aug., xli., Beil., 255, 1903.
Batten. T. O. S., xxiii, 75, 1903. Krug and Samuels. A. of O., viii, 871, 1932.
Doyne. T. O. S., xxiv, 91, 1904. Moore, Foster. Brit. J. O., Supplement ii,
Elschnig. K. M. Aug., xxxvi, 55, 1898. 1924.
Friedenwald. Cb. pr. Aug., xx, 33, 1896. Pollock. T. O. S., xxvii, 128, 1907.
T. O. S., 1, 452, 1930. de Schweinitz. T. A. m. O. S., xi, 87, 1906.
Fuchs. Z. f. Aug., xxiii, 504, 1910. Thoma. A. f. O., xxxv (2), 1, 1889.
Gauss. A. f. O., czziii, 427, 1930. Wessely. K. M. Aug., xc, 95, 1933.
Gunn. Fest. f. Helmholtz, 6, 1891.
2. VARICOSITIES OF THE VEINS
The retinal veins may occasionally show definite varicosities and dilatations.
Such a phenomenon occurs near the disc in glaucoma (Liebreich, 1863; Morton, 1890),
in venous thrombosis and haemorrhages (Michaelsen, 1889; Friedenwald, 1896 ;
Doyne, 1904), and in advanced arteriosclerotic conditions (Gunn, 1898; Raehlmann,
1902) (Fig. 2297).
Doyne. T. O. S., xxiv, 91, 1904. Michaelsen. ('b. pr. A ug., xiii, 106, 1889.
Friedenwald. Cb. pr. Aug., xx, 33, 1896. Morton. T. O. S., x, 15 5, 1890.
Gunn. T. O. S., xviii, 356, 1898. Raehlmann. Z. f. A wy., vii, 425, 1902.
Liebreich. Atlas, Taf. xi, Ab. 1, 1863.
3. ANOMALOUS ANASTOMOSES
Pathological anastomoses in the retina are by no means uncommon,
both between the retinal vessels and between the vascular systems of the
retina and the choroid.
Inter-arterial anastomoses in the retina are relatively rare and are usually an
attempt to overcome an obstruction. A junction between a branch of the central
artery and a cilio-retinal artery after obliteration of the former by an embolus at the
disc was noted by Nettleship (1891) and Gonin (1905). When the obstruction is in a
branch on the fundus, one of the peripheral arterial branches may join up with the
obliterated one (Holden, 1893; Königshöfer, 1898; Barkan, 1903; and others)
(Figs. 2201, 2202), while in other cases multiple anastomoses may occur both on the
disc and in the periphery (Coats, 1913 ; Harms, 1914); Roenne, 1915; Candian,
1921 ; Rados and Candian, 1921) (Fig. 2203).
Vervous amastomoses in the retina are more common, occurring with considerable
facility and rapidity in conditions of obstruction to maintain the circulation (Liebreich,
1863 ; Hormuth, 1903 ; Batten, 1903 ; Pollock, 1907; Fuchs, 1910 ; Harms, 1912 ;
1 p. 2617. * p. 2844.
2614 TEXT-BOOK OF OPHTHALMOLOGY
Kraupa, 1915; Foster Moore, 1924) (Fig. 2204). A case reported by Coats (1905)
may be similar where an anastomotic loop joined up again with the main vessels as if
it had bridged over an obstruction. An arterio-venous communication in an eye
with poor vision in which the vessels were of normal size and had no resemblance
to an aneurysm was described by Gunn (1884). Similar anastomoses may be seen at
the disc in glaucoma (Bloch, 1906; Axenfeld, 1911; Kraupa, 1915).
Fros. 2201 to 2204.-NEw VEssels on RETINA.
Fig. 2201.-Branch em- Fig. 2202.-A later stage
bolism in retinal artery, of the same case.
showing new anastomic
channels compensating for
the obliterated artery
(Königshöfer).
Fig. 2203.-NEw VEssels Fig. 2204.-NEw WEssº Ls
on Disc AFTER ARTERIAL on Disc AFTER WENous
Obstruction. OBSTRUCTION.
After obstruction of the After venous thrombosis
central artery. of inferior vein; connec-
tions formed with branch of
superior vein which is
normal.
Peripheral anastomoses between the retinal and choroidal vessels not uncommonly
follow chorio-retinitis either in the periphery (Uhthoff, 1889: Lawson, 1910; Salus,
1917 : Ginzberg, 1918; Feingold, 1920; Gradle, 1920; Jennings, 1924) or at the
macula (Bickerton, 1906). Such a condition may be seen in the so-called
macular and atypical “colobomata” which are probably of inflammatory origin



DISEASES OF THE RETINA 2615
(Beaumont, 1891; Mann and Ross, 1929) (Fig. 1216, Plate XV). Axenfeld (1894)
reported a unique case of old choroiditis with retention of good vision wherein the
normal veins had shrunk to mere remnants and almost the entire venous system of
the retina drained through new-formed vessels into the choroidal system. -
4rterio-venous anastomoses occur occasionally in such conditions as angiomatosis,”
arterial obstruction (Kraupa, 1915), or after trauma (Harms, 1914).
A curious condition of the retinal vessels has been reported from Japan, associated
with other anomalies of the circulatory system, wherein numerous loop-like com-
munications with rounded or fusiform dilatations occur between the arterial and
Venous branches round the disc (Uchino, 1930 ; Kikkawa, 1935). The condition is
unassociated with inflammatory symptoms, but is accompanied by marked diminution
of vision and terminates in cataract.
Axenfeld. K. M. Aug., xxxii, 11, 1894. Jennings. Am. J. O., vii, 868, 1924.
Z. f. Aug., xxv, 362, 1911.
Barkan. A. f. Aug., xlviii, 284, 1903.
Batten. T. O. S., xxiii, 75, 1903.
Beaumont. T. O. S., xi, 221, 1891.
Bickerton. T. O. S., xxvi, 77, 1906.
Bloch. K. M. Aug., xliv (2), 413, 1906.
Candian. A. di Ott., xxviii, 89, 1921.
Coats. T. O. S., xxv, 309, 1905.
R. L. O. H. Rep., xix, 78, 1913.
Feingold. Am. J. O., iii, 499, 1920.
Fuchs. Z. f. Aug., xxiii, 504, 1910.
Ginzberg. K. M. Aug., lxi, 643, 1918.
Gonin. An. d’Oc., czzxiii, 167, 1905.
Gradle. Am. J. O., iii, 818, 1920.
Gunn. T. O. S., iv., 156, 1884.
Harms. K. M. Aug., l (2), 106, 1912.
A. f. O., lxxxvii, 334, 1914.
Rikkawa. Acta O. S. Jap., xxxix, 16, 1935.
Königshöfer. Med. Cor.-Bl. d. wurttemb.
arztl. Ver., Stuttgart, lviii, 237, 1898.
Rraupa. A. f. Aug., lxxviii, 182, 1915.
Lawson. T. O. S., xxx, 278, 1910.
Liebreich. Atlas, Taf. viii, Ab. 3, 1863.
Mann and Ross. Brit. J. O., xiii, 608, 1929.
Moore, Foster. Brit. J. O., Supplement ii,
1924.
Nettleship. Festschrift f. Helmholtz, 7, 1891.
Pollock. T. O. S., xxvii, 128, 1907.
Rados and Candian. K. M. Aug., lxvi, 797,
I921.
Roenne. K. M. Aug., liv, 522, 1915.
Salus. K. M. Aug., lviii, 257, 1917.
Uchino. Acta O. S. Jap., xxxiv, 246, 1930.
Uhthoff. A. f. Psy. w. Nervenkr., xxi (2), 18,
Holden. A. of O., xxii, 90, 1893. 1889.
Hormuth. K. M. Aug., xli., Beil., 255, 1903.
ANEURYSMS
In contra-distinction to the cerebral arteries, isolated aneurysms of the
retinal arteries are very rare (Fig. 21.94, Plate XLIX). It is interesting that
the first case to be noted was unique, the aneurysm occurring in the optic nerve
and causing pulsation (C. Graefe, 1808). The first ophthalmoscopically
recognized case was recorded by Sous (1865) at the disc, and later examples in
the larger retinal arteries were described by Raehlmann (1889–1902). Oeller
(1891) noted one in a macular artery; and Mannhardt (1875) described a case
associated with a rupture of the choroid which may have had a traumatic
Origin. Small miliary aneurysms presenting an uncomplicated Ophthalmo-
scopic picture are more common, but still rare. They may occur
in apparently normal and healthy young people (Pringle, 1917), but
are more typically associated with age and atheromatous vascular degenera-
tion (Reimar, 1890; Raehlmann, 1902). Post-mortem examination has
shown that they may be co-existent with similar aneurysms in the cerebral
vessels (Lionville, 1870; Litten, 1881), or they may be associated with
1 p. 2844.
2616 TEXT-BOOK OF OPHTHALMOLOGY
nephritis (Schmall, 1888; Raehlmann, 1902), syphilis (Goldzieher, 1889) or
diabetes (Mackenzie, 1877).
MILIARY ANEURYSMS WITH RETINAL DEGENERATION
The association of miliary aneurysms with marked and progressive
retinal changes of an exudative and degenerative nature occurring in young
people has long been remarked. Originally noted by English writers (Story
and Benson, 1883 : Story, 1886; Doyne, 1896), the condition was first
described as a clinical entity by Leber (1912). The aneurysms may be
Fig. 2205.-Multiple RETINAL ANEURysMs.
The aneurysms have the form of multiple dilatations of the vessels and are
progressive. Note massive retinal exudates and changes near macula (Fisher,
T. O. S.).
relatively few in number and localized to a small group of vessels (Fig. 2205);
in such a case Fisher (1903) noted the changes progressing rapidly for a time,
rows of dilatations appearing along the course of three neighbouring arteries
successively, accompanied by changes resembling circinate retinopathy,
until after some time the condition stabilized. Circinate changes were also
noted by de Schweinitz and Holloway (1910). Occasionally the changes are
limited to the macular region (Pergens, 1896). More usually, however, the
disease is widespread, affecting large areas, or the whole of the retina may be
plentifully besprinkled with minute saecular or sausage-like swellings
associated with massive sub-retinal exudation.

DISEASES OF THE RETINA 26.17
AEtiologically Krauss and Brückner (1907) associated the condition with
tubercle, and Leber (1912) with multiple emboli as from a latentendocarditis;
but both of these hypotheses are improbable. It seems likely that the
diffuse cases form a type of angiomatosis and are neoplastic in nature :
they will therefore be discussed at a later stage."
Coats. R. L. O. H. Rep., xvii, 440, 1908. Pergens. K. M. Aug., xxxiv, 170, 1896.
Doyne. T. O. S., xvi. 94, 1896. Pringle. Brit. J. O., i, 87, 1917.
Fisher. T. O. S., xxiii, 73, 1903. Raehlmann. K. M. Aug., xxvii, 203, 241,
Goldzieher. Cb. pr. Aug., xiii, 361, 1889. 1889.
Graefe, C. Angiektasie, Leipzig, 33, 1808. Z. f. Aug., vii, 425, 1902.
Krauss and Brückner. A. f. Aug., lvii, 157 Reimar. A. f. Aug., xxxviii, 209, 1890.
1907. Schmall. A. f. O., xxiv. (1), 37, 1888.
Leber. A. f. O., lxxxi (1), 1, 1912. de Schweinitz and Holloway. 0. Rec., xix,
Lionville. C. R. S. Biol., lxx, 498, 1870. 243, 1910.
Litten. Berlin, kl. W., xviii, 25, 1881. Sous. An. d’Oc., liii, 241, 1865.
Mackenzie. R. L. O. H. Rep., ix, 134, 1877. Story. T. O. S., vi, 336, 1886.
Mannhardt. K. M. Aug., xiii, 133, 1875. Story and Benson. T. O. S., iii, 108, 1883.
Oeller. A. f. Aug., xxii, 68, 1891.
ARTERIo-venous (RACEMosF). ANEURYSM
An ARTERIo-vKNous ANEURYsm (RACEMosº ANEURYsA1 or ANEURYSMAL VARIX)
is a great rarity, only a few cases having been reported (Magnus, 1874; Schleich,
Fig. 2206.-ANEURysMAL VARIx (Seidel).
1885; Seidel, 1899; Kreutz, 1903; Weve, 1923; Rentz, 1924; Ehlers, 1925 (Fig.
2206). It usually occurs in young people, the respective ages of the 7 cases being
29, 8, 18, 28, 52, 23 and 65 years. In each the general vascular system was normal,
except Kreutz’s case in which there was a racemose aneurysm of the carotid ; while
the aged cases had arteriosclerosis, chronic nephritis and hypertension (Weve) and
choroiditis (Ehlers). Trauma is mentioned only once (Magnus), while Rentz considered
p. 2844.

2618 TEXT-BOOK OF OPHTHALMOLOGY
his case congenital. As a rule the condition has not been noted to progress, but with
one exception (Seidel) the vision has been poor or absent. There is no effective
treatment.
Ehlers. Acta O., ii, 374, 1925. Schleich. Mitt. aus d. oph. Kl. in Tübingen,
Kreutz. Wien. med. W., liii, 1725, 1903. ii (2), 202, 1885.
Magnus. A. f. path. Amat., lx, 38, 1874. Seidel. A. f. Aug., xxxviii, 256, 1899.
Rentz. A. f. Aug., xcv, 84, 1924. Weve. A. f. Aug., xciii, 1, 1923.
NEw-VESSEL FORMATION
The formation of new vessels in the retina is not uncommon, occurring in
most inflammatory conditions, and as we have seen, in many cases where the
circulation has been impaired or obstructed, such as we have already noted
in cases of obliterative endarteritis, embolism 1 (Fig. 2203), or venous
thrombosis 2 (Fig. 2204). A similar phenomenon, especially apparent upon
the disc but sometimes extending peripherally, is seen commonly in glaucoma
(v. Graefe, 1854; Pagenstecher, 1871 ; and others) and optic neuritis
(Hutchinson, 1886; Roll, 1909; and others), both conditions again wherein
the circulation is obstructed. In some cases the vessels are small, so small,
indeed, as to be indistinguishable ophthalmoscopically, so that the disc on
superficial examination may appear to be pinker than normal, or a red
patch on the fundus may appear to be a haemorrhage until carefully analysed
(Harman, 1910), while in others large and usually convoluted vessels are
readily discernible, frequently linking up in an elaborate network of inter-
anastomoses (RETE MIRABILE). In all cases, however, even although the
new-formation has been exuberant so that elaborate and apparently meaning-
less ramifications spread themselves out in the most lavish manner, the process
is a purposeful one, and represents an attempt to supply a system of
collaterals which can take over the supply of an area the nourishment of
which has been impaired by vascular disease.
The growth of new vessels into the vitreous, a process which always
originates at or near the disc, is a rarer and more interesting problem, which
was first described by Coccius (1859). Here again, degenerative vascular
disease leading to circulatory impairment is the essential factor, and the
phenomenon is associated especially with syphilis (Nettleship, 1884;
Hutchinson, 1886; Flint and Harrington, 1934; Klien, 1938), tubercle,
particularly tuberculous peri-phlebitis (Theobald, 1887; Meller, 1932 :
Flint and Harrington, 1934; Tan, 1936), diabetes (Nettleship, 1888;
Lawson, 1899 ; Klien, 1938), and arteriosclerosis (Kraupa, 1925; Klien,
1938). Sometimes the vessels are large and coiled, apparently representing
aberrant attempts at re-establishing anastomoses, but the typical case is
associated with retinal haemorrhages into the vitreous. At first the new
vascular formation may therefore not be seen owing to the vitreous haze. A
1 p. 2569. * p. 2578.
DISEASES OF THE RETINA 2619
FIG. 2207.-NEw VEssBLs IN WITREous.
After a haemorrhage into the vitreous associated with arteriosclerosis. Brush-
like arrangement of new vessels surrounded by delicate connective tissue and ending
in coil-like convolutions in the vitreous (Klien, A. of O.).
Fig. 2208.-RETE MIRABILE IN THE WITREous.
Vascularization in the vitreous 6 years after retinal haemorrhages in a woman
aged 62. The new vessels (which appear flat in the drawing) form an arborescence
extending axially forwards to the back of the lens. Normal vision; but a retinal
detachment developed later (Giri, Brit. J. O.).


2620 TEXT-BOOK OF OPHTHALMOLOGY
single vessel may be present, coiling back upon itself; but more usually
there is an intricate arrangement of innumerable delicate vessels disposed in
a brush-like arrangement or forming a complicated anastomosing network
usually associated with the optic disc (Fig. 2.195, Plate XLIX). As a rule
the endings in the vitreous form coil-like convolutions returning again to
the disc, or alternatively they may end in aneurysmal-like dilatations
(Ferrer, 1932) (Fig. 2207), while sometimes they form an elaborate amas-
tomotic RETE MIRABILE (Fig. 2208). Such a mass of vessels is usually bound
Fig. 2209–RETINITIs ProLIFERANs.
Blood-vessel in vitreous splitting up into a brush-like arrangement of new
capillaries (B). V. A vein in the disc. H. Hyaline degeneration of connective tissue
(Klien, A. of 0.).
together by a delicate sheet of connective-tissue forming a gossamer-like veil,
which may eventually, however, become quite substantial. Histologically
the network is composed at first offibroblasts with a homogeneous transudate
between them, which may eventually become much thickened owing to
fibroblastic and neuroglial proliferation (Klien, 1938) (Figs 2209 to 2211).
It is interesting that unless this organization becomes very pronounced,
good vision may be retained (Giri, 1934). In most cases when the haze
of the vitreous hæmorrhage has completely cleared the new vessels regress,
sometimes remaining permanently smaller, sometimes shrinking to light
grey strands, and sometimes disappearing entirely (Charnley and Fox, 1881:

DISEASES OF THE RETINA 26.21
FIG. 2210.-RETINITIS PROLIFERANs.
R. A newly formed vein in the vitreous arising from a dilated and tortuous
vein (V) on the disc. N. Neuroglia (Klien, A. of O.).
FIG. 2211.-RETE MIRABILE.
Section through a mass projecting from the disc, composed of large thin-walled
vessels in a matrix partly fibro-cellular and partly amorphous. In a case of recurrent
retinal and vitreous hæmorrhages in a man of 30 years with peri-vasculitis retinae
(Ballantyne and Michaelson, Brit. J. O.).


2622 TEXT-BOOK OF OPHTHALMOLOGY
Flint and Harrington, 1934; Tan, 1936). It seems obvious that this
phenomenon, like neo-vascularization in the retina itself, is a purposive
formation reparatory in nature, the vessels acting as scavengers to clear up
the vitreous hæmorrhage and tending to disappear when this function is
completed.
New vessels grow into the vitreous cavity along preformed fibrous tissue tracts
in inflammatory membranes (Blok, 1904), and in retinitis proliferans.” Some authors
would, indeed, classify the type of neo-vascular formation which we are considering
with retinitis proliferans (Marple, 1906; Klien, 1938); but the two seem essentially
different—the former vascularization is exuberant, primary and purposive, while the
vascularization in retinitis proliferans is minimal, late in appearing and secondary in
nature.
Blok. Ned. Tij. v. Gen., ii, 723, 1904. Kraupa. K. M. Aug., lxxv, 711, 1925.
Charnley and Fox. R. L. O. H. Rep., x, 193, Lawson. T. O. S., xix, 288, 1899.
1881. Marple. T. Am. O. S., xi, 104, 1906.
Coccius. Ueber Glaukom, Leipzig, 47, 1859. Meller. Lectures on the Tuberculous AEtiology
Ferrer. Rev. Cub, de Oto-neuro-oft., i, 1, 1932. of Uveitis, Vienna, 1932.
Flint and Harrington. Brit. J. O., xviii, 27, Nettleship. T. O. S., iv., 150, 1884; viii, 159,
1934. 1888.
Giri. Brit. J. O., xviii, 24, 1934. Pagenstecher. K. M. Aug., ix, 425, 1871.
v. Graefe. A. f. O., i (1), 367, 1854. Roll. T. O. S., xxix, 222, 1909.
Harman. T. O. S., xxx, 80, 1910. Tan. A. of O., xvi, 1004, 1936.
Hutchinson. R. L. O. H. Rep., xi, 191, 1886. Theobald. T. Am. O. S., iv, 542, 1887.
Rlien. A. of O., xx, 427, 1938.
III. INFLAMMATION OF THE RETINA : RETINITIS
A. General Pathology
Inflammations of the retina are of relatively little significance, for in
the majority of cases they are secondary to inflammations in the uveal
tract. Moreover, inflammatory changes are little in evidence histologically,
being confined essentially to the blood-vessels and their associated mesoderm,
for the neural tissue of the retina itself has little or no inflammatory response.
If the inflammatory process is sufficiently acute the cellular elements suffer
necrosis and die, and apart from the mesodermal reaction, the only active
process is a compensatory proliferation of glial tissues.
The retinal changes during and subsequent to inflammation may be
described under three headings: inflammatory, proliferative, and degener-
ative.
Inflammatory Changes
The vascular response in inflammation of the retina does not differ
essentially from that occurring elsewhere in the body. At first the dilated
vessels are surrounded by a mantle of leucocytes, mainly polymorpho-
* p. 2604.
DISEASES OF THE RETINA 26.23
nuclears in the acute forms and lymphocytes in the more chronic; these first
pack the perivascular spaces and then wander afield, inwards into the
vitreous or outwards into the deeper layers of the retina (Fig. 2212). Even
at this early stage, owing to the toxic effect, the neural cells of the retina
may rapidly necrose.
The disorganization of the walls of the vessels is rapidly followed by
wdema, a phenomenon first seen in the nerve-fibre layer as a diffuse infiltra-
tion, since here the majority of the vessels lie, and eventually spreading to
the outer layers where the structures confine the fluid in more isolated areas
(Iwanoff, 1869) (Fig. 2188). The histology of this state has already been dis-
Fig. 2212.-Acute INFLAMMATIox of THE RETINA.
Wound of the retina caused by a foreign body 4 days after injury. A retinal
vessel is enclosed in a mass of leucocytes and necrotic tissue (x 300) (Parsons).
cussed," but in purely inflammatory states the oedema is rarely excessive and
cystic formation unusual. The fluid, however, is rich in protein constituents,
which owing to the presence of fibrinogen readily coagulate. In the nerve-
fibre layer these usually appear as ill-defined cloudy masses with soft edges
infiltrating the tissue-spaces diffusely (‘’ cotton wool” patches); but in
the outer layers, the exudates, like the oedematous fluid, tend to be sharply
confined in cyst-like spaces particularly in the outer fibrillar layer. On
cross-section these present a circular or oval shape, but in flat-section they
appear as an interlacing network (Fig. 2213), and ophthalmoscopically they
are usually seen as small white dots. Eventually such exudates tend to
undergo chemical degenerative changes forming homogeneous, hyaline-like
masses, interspersed with which may be a coagulated fibrinous network.
1 p. 2588.
T.O.-WOL. III. M. M.

2624 TEXT-BOOK OF OPHTHALMOLOGY
Sometimes these masses are absorbed by leucocytic phagocytosis; at other
times they become organized, being replaced by fibrous tissue derived from
the mesodermal elements of the vessels or they may undergo fatty or even
calcareous degeneration with the deposition of cholesterol crystals.
Associated with these cedematous and exudative changes, some degree
of haemorrhage almost invariably occurs. When in small quantity, this is
seen at first in the nerve-fibre layer drawn out into flame-shaped formations
by the arrangement of the fibres, and later in more rounded masses in the
nuclear layers; but if massive extravasations occur the retina may be torn
up mechanically, the resultant necrosis extending beyond the involved area,
Fig. 22.13–Exudates IN THE RETINA.
Flat section in a case of thrombosis of the central vein. The dark dots on the
left are the inner nuclear layer; those on the right the outer (x. 90) (Parsons).
º
or the blood may spread over the inner surface to form a pre-retinal haemor-
rhage, or burst through the limiting membrane and enter the vitreous cavity.
There is little tendency for such haemorrhages to clot, and if they are small
absorption occurs through leucocytic activity; but in the case of larger
haemorrhages, their encapsulation by cells from the vascular endothelium
and by the proliferation of glial elements results in their transformation into
fibrous tissue which may eventually undergo degeneration.
Proliferative Changes
The proliferative changes occurring in the retina as a sequel to inflamma-
tion are more interesting and distinctive. They are, of course, associated
with the milder and more chronic types of infection, for in acute and virulent

DISEASES OF THE RETINA 2625
conditions the tissue-response may be quite lacking. The ordinary process
of fibrosis in which the active cells are derived essentially from the vessel
walls, occurs as in other tissues, a feature of reparative activity we have
already discussed in describing the healing of wounds " . Sometimes,
however, the fibrosis becomes excessive and masses of fibrous tissue
are laid down in the retina, between it and the choroid, and upon its inner
surface, frequently extending profusely into the vitreous cavity. It is to be
remembered, however, that the sub-retinal fibrous masses are frequently
largely formed from the choroid which has shared in the inflammatory
Fig. 2214.-Folding of THE RETINA.
Folded retina in the neighbourhood of the disc from a shrunken globe. In a
child with necrosis of the cornea after membranous conjunctivitis ( x 11-5) (Parsons).
process; this by its contraction may distort the retina and heap it up into
folds which may form great tumour-like masses (Fig. 2214), and induce
profound secondary degenerative and cystic changes. The pre-retinal
fibrous tissue, which usually has its origin from the disc where the
vascular and mesodermal elements are concentrated, may also become
exuberant in its growth to form the picture we have already discussed as
RETINITIS PROLIFERANs,” and this by its subsequent contraction may do
equally widespread damage by detaching the retina and completely
destroying vision.
A more specific new-formation which may occur after chronic inflamma-
tions of a mild type is an endothelial new formation in the form of a membrane
* p. 2550. * p. 2192. * p. 2604.

2626 TEXT-BOOK OF OPHTH ALMIOLOGY
on the inner surface of the retina (Figs. 2188 and 2215), a phenomenon first
noted by Iwanoff (1865). Ophthalmoscopically such a membrane may be
seen as an extremely delicate veil, but it is usually a late appearance in blind
and shrunken eyes. Histologically it is made up of large flat cells with
oval nuclei and indefinite outlines, arranged sometimes in a single layer,
Fig. 2215.-ENDoth ELIAL MEMBRANE on RETINA.
From an eyeblind for 35 years. The endothelial cells are elongated into spindle
cells (x 70) (Parsons). Compare Fig. 2589.
sometimes in many strata, which arise from the adventitia of the vessel
walls (Theodore, 1879; Nordenson, 1887; Tepljaschin, 1894; Parsons,
1904).
The question of glial proliferation as a post-inflammatory phenomenon has excited
considerable controversy, largely because of the difficulty of distinguishing by histo-
logical stains between glial tissue and fibrous tissue of mesodermal origin: Pollack's
modification of the Weigert stain is the most conclusive. Moreover, in atrophic con-
ditions the neural elements readily disappear while the glial remain, so that the retina
is entirely represented by the latter; a picture is thus produced as if an overgrowth of
glia had occurred, whereas the increase may be relative and not absolute. The evidence
seems fairly conclusive that the greater part of the new-formed fibrous-like tissue found
in inflamed and degenerated retinae is of mesoblastic origin derived from the blood-
vessels (Parsons, 1903–05); but nevertheless a compensatory glial hypertrophy may
occur as well, and sometimes to a considerable degree.
Such a hyperplasic condition is termed GLIosis of THE RETINA, wherein
the glial cells are increased in number and their fibres thickened (Krückmann,
1905–17; v. Hippel 1918). In this way the retina may become thickened
to three or four times its normal dimensions, partly by fibrous tissue and
partly by glial tissue, with a fine layer of fibres arranged in festoons on its
inner surface (Figs 2216 and 2217). We have seen that a similar hypertrophy
occurs when complete necrosis of the neural elements is brought about by
obliterating the circulation (Fig. 2151) 1, and in cases of chorio-retinitis
when Bruch's membrane has been destroyed, the glial fibres may proliferate
into the choroid (Murakami, 1902). As a late end-result of long-continued
* p. 2548.

DISEASES OF THE RETINA 2627
Fig. 2216.-GLIosis RETINAE.
Pollack's stain for glial fibres (after Friedenwald).
Fig. 2217. –GLIosis of THE RETINA.
Individual glial cells. Pollack's stain (after Friedenwald).
inflammatory states, this proliferation may attain the dimensions of a
tumour of a benign, non-invasive nature, a condition which may be termed
MAssive GLIosis (v. Hippel, 1918; Friedenwald, 1926) (Fig. 2218).


2628 TEXT-BOOK OF OPHTHALMOLOGY
-
Fig. 2218.-MAssive GLIosis of THE RETINA.
In the region just behind the ora (x 55) (Parsons).
Degenerative Changes
The degenerative changes which occur subsequent to inflammations
are similar to those which characterize malnutritional states such as are
seen in vascular retinopathies, when the blood-supply is cut off, or when
the retina is detached. They involve the nerve-elements, the neuroglia,
the vessels, and the pigmentary epithelium (Figs. 2219 and 2220).
The nerve-elements are the first to suffer such changes, for degenerative
changes start from the beginning of the inflammatory process and readily
proceed to complete disappearance of the neural structures. The nerve-
fibres, even in the early stages of oedema, swell irregularly, frequently
showing varicose thickenings (Müller, 1858) (Fig. 2221), and eventually dis-
appear, so that the layer becomes thinned and shrunken or is replaced by
proliferated fibrous tissue. The ganglion cells are the least resistant in the
retina and degenerate first, frequently suffering irreparable damage in the
early cedematous stages. Lying in enlarged peri-cellular spaces filled with
fluid, the Nissl bodies break down so that the cells stain diffusely and
homogeneously, their contour becomes irregular, vacuoles containing fatty
material appear, and finally the cells shrivel up and disappear (Oeller, 1881;
Bach, 1895; Dolganoff, 1897; Birch-Hirschfeld, 1900–04; Birch-Hirsch-
feld and Inouye, 1905–09). The bipolar cells of the inner nuclear layer
are more resistant than the ganglion cells, but eventually they lose their
regular arrangement and swarm out to the adjacent layers, swell up and

DISEASES OF THE RETINA 26.29
Fig. 2219. Post-INFLAMMATORY DEGENERATION of THE RETINA.
Retinal oedema and degeneration with congestion of the choroid 3 weeks after
the lodgement of a steel foreign body in the vitreous (x 120) (Parsons).
FIG. 2220.-Post-INFLAMMATORY ATRoPHY OF THE RETINA.
From a blind eye affected 18 years. All the retinal layers are atrophied and the
choroid is congested (x 230) (Parsons). "


2630
TEXT-BOOK OF OPHTHALMOLOGY
become necrotic so that they take on their stains progressively feebly, and
finally shrink and disintegrate. The outer nuclear cells persist longest
1. –VARIcosº DEGENERATION or
NERVE FIBREs (Koyanagi).
down and others becoming hypertrop
Jaensch, 1930).
of all, and the rods and cones,
although they succumb rapidly to
cedema, are relatively resistant to
inflammatory processes; eventually,
however, they swell up, lose their
striations, and finally break down
into a granular detritus (Axenfeld,
1894; Krückmann, 1905; v. Hippel,
1911; Inouye, 1912).
The neuroglia, in addition to the
proliferative changes in gliosis, may
show pronounced fatty degeneration,
vacuoles like strings of pearls appear-
ing in the processes, while in the
cystic state the processes are pressed
apart, some strands being broken
hied and consolidated (Parsons, 1905;
The blood-vessels also become much altered in post-
Fig. 2222.-VAscular DEGENERATION.
Fibrosis of the connective tissue wall of a vein (x 120) (Coats).
inflammatory atrophies: endarteritis
may progress to complete obliteration
of the lumen and lead to widespread thromboses; hyaline degeneration may


DISEASES OF THE RETINA 2631
be marked (Fig. 2222) and the smaller vessels may be reduced to fibrous
cords.
The behaviour of the pigmentary epithelial cells in retinal inflammations
is exceptionally interesting, but although the changes in this layer are
very prominent from the ophthalmoscopic point of view, they play a purely
secondary rôle without much dependence upon the extent or severity of
the inflammatory process. In so far as they derive their nourishment from
the choroid they may survive when the retina has been destroyed, and their
reactions are most prominent when the peak of the inflammatory process
has been passed and the acute changes have begun to subside. The cells may
FIG. 2223.−BLADDER CELLs.
Formed by proliferation of the pigmentary epithelium. From a case of traumatic
irido-cyclitis (X 145) (Parsons).
react in two ways. In the more acute phases they may become loosened
from the membrane of Bruch and degenerate, the granules of pigment
being taken up by the leucocytes or lying free in the surrounding exudation.
The more typical reaction, however, occurs in the more chronic phases when
the cells alter their shape, proliferate and migrate (Krückmann, 1899). They
lose their hexagonal shape and most of their pigment and become larger and
more drawn out, assuming a spindle shape, and eventually they become
rounded, forming large blown-out BLADDER-CELLs which frequently contain
fatty droplets (the ghost-CELLs of Coats, 1908, or SPECTRAL CELLs of Leber
1915) (Fig. 2223). Much of the pigment is scattered and the cells become
desquamated and lie freely in the sub-retinal space. The cast-off cells are
replaced by proliferation, the new cells containing little pigment, and as the

2632 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2224.—BLADDER CELLs.
Massive proliferation in exudative retinitis (Marshall and Michaelson, T. O. S.).
Fig. 2225.-BLAppen CELLs INvADING THE RETINA,
In exudative retinitis. The cells from the sub-retinal space are densely infiltrat-
ing the retina in which the rods and cones have disappeared (Marshall and
Michaelson, T. O. S.).


DISEASES OF THE RETINA 2633
process proceeds in a progressive manner, massive collections of these cells
may accumulate in the sub-retinal space (Fig. 2224) (Donders, 1855;
Müller, 1856; Schweigger, 1859–63). These cells are endowed with actively
amoeboid movements and rapidly invade the retina itself, provided the
internal limiting membrane is not intact and the retinal tissues are
disintegrated (Wagenmann, 1890; Capauner, 1893; Krückmann, 1899)
(Fig. 2225). This process sometimes occurs to an extreme degree so that the
cells become arranged in regular vertical rows or irregular clumps. It is
probable that at this stage these cells act as macrophages ; and it is also
probable that at the later reparative stage they can metamorphose into
fibroblasts and may be responsible for much proliferative fibrous tissue
(Parsons, 1902; Leber, 1915; Llorca, 1932; Koyanagi, 1935; Daniel,
1937; Lamb, 1938; and others).
Authorities are not all agreed as to the nature of these bladder cells which may be
such a prominent feature especially in mild and chronic inflammations as exudative
retinitis, or degenerative conditions, such as circinate 1 or renal retinopathy. Coats
(1908–12) considered them to be swollen and disintegrated leucocytes; Meller (1922)
and Doesschate (1927) likened them to epithelioid cells; de Schweinitz and Shumway
(1901) assumed that they represented a dropsical degeneration of the rods and cones;
and Marshall and Michaelson (1933) concluded that they were histiocytes. The
histological evidence, however, seems to be definite that the cells of the pigmentary
epithelium can be metamorphosed to form bladder cells (Seefelder, 1929; Lamb,
1938; and others), although, of course, such an observation does not prove that this
is their only origin. We have already seen * that in conditions of under-nourishment
which lead to oxidative failure, the tissue-fat is unmasked from its invisible form and is
deposited in a visible form as droplets, which, acting as foreign bodies, excite first
the mobilization of phagocytic cells and eventually a process of fibrous proliferation.
Such a lipoid deposition is universal throughout the central nervous system in anoxaemic
states, and everywhere the same phagocytic reaction is stimulated so that histiocytic
cells which become loaded with fat appear in great numbers : it would seem that their
origin in the eye is mainly, at any rate, from the pigmentary epithelium.
Pigmentation is thus an extremely common and almost universal feature
of the later stages of retinal inflammation, either by pigment carried in from
the disintegrating epithelial cells by leucocytes, or by the active invasion
of the retina by the pigment cells themselves through their amoeboid move-
ments. Eventually, however, the invading epithelium and pigment-bearing
leucocytes atrophy, so that the pigment is free or is taken up by connective-
tissue cells. It has, however, a great tendency to become aggregated in the
adventitia of the vessels and the perivascular spaces, and occasionally
may even pass into the lumen (Fig. 2226).
The final stage of post-inflammatory atrophy is characterized by the
complete disappearance of the neural elements and their replacement by
fibrous tissue derived from the vascular elements and the glial supportive
tissue. The oedematous spaces in the nuclear layers, originally filled with
1 p. 2760. * Vol. II, p. 1436.
2634 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 22.26.-Migration of Pigment into THE RETINA.
From a case of chorio-retinitis. Note preferential accumulation around the
vessel (Parsons).
- -
- - -
-
-
- - - - - -
- - -
---- -º- __
- - —º.
Fig. 2227–Cystic Degeneration of THE RErina.
Section near disc. From a case of chronic irido-cyclitis (Parsons).



DISEASES OF THE RETINA 2635
oedematous and exudative fluid, may contain hyaline, fatty or granular
material, the disintegrated products of leucocytes, retinal and glial cells,
FIG. 2228.
HYALINE DEPosits IN RETINA (x 60) (Parsons).
but more frequently are empty, producing the picture of cystoid degeneration
(Fig. 2227). On the other hand, hyaline degeneration may be very much in
Fig. 2229.-FATTY AND CALCAREous DEGENERATION of RETINA.
The dark globules are fat stained with Sudan III; the linear spaces were occupied
by cholesterol crystals which have been dissolved out. In traumatic irido-cyclitis
( x 130) (Parsons).
evidence, masses of homogeneous material gathering in all the retinal layers,
especially the inner ones (Fig. 2228), or the old exudates may be impregnated
with deposits of calcium salts and cholesterol crystals (Fig. 2229) (Parsons,


2636 TEXT-BOOK OF OPHTHALMOLOGY
1902; Sugita, 1925). Ossification, however, never occurs primarily in the
retina, although in old shrunken globes it not uncommonly spreads to this
tissue from the choroid."
Axenfeld. A. f. O., xl (3), 1 ; (4), 103, 1894. Lamb. Am. J. O., xxi, 618, 1938.
Bach. A. f. O., xli (3), 62, 1895. Leber. G.-S. Hb., II, vii (A), 1267, 1915.
Birch-Hirschfeld. A. f. O., I, 166, 1900; lii, Llorca. A. de Oft., H.-A., xxxii, 349, 1932.
358, 1901; liii, 79 ; liv, 67, 1902; lyiii, Marshall and Michaelson. T. O. S., liii, 102,
469, 1904. 1933.
Birch-Hirschfeld and Inouye. A. f. O., lxi, Meller. Z. f. Aug., xlvii, 247, 1922.
499, 1905; lxx, 486, 1909. Müller. A. f. O., ii (2), 1, 1856; iv. (2), 41,
Capauner. B. O. G. Heidel., xxiii, 45, 1893. 1858.
Coats. R. L. O. H. Rep., xvii, 440, 1908. Murakami. A. f. O., liii, 439, 1902.
A. f. O., lxxxi, 275, 1912. Nordenson. Die Netzhautablösung, Wiesbaden,
Daniel. A. de Oft., B.A., xii, 384, 1937. I887.
Doesschate. K. M. Aug., lxxix, 505, 1927. Oeller. A. f. path. Anat., lxxxvi, 329, 1881.
Dolganoff. A. f. Aug., xxxiv, 196, 1897. Parsons. T. O. S., xxii, 255, 1902; xxiv, 149,
Donders. A. f. O., i (2), 106, 1855. 1904.
Friedenwald. Contrib. to Oph. Science, R. L. O. H. Rep., xv, 215, 1903.
Wiscon., 23, 1926. Pathology of the Eye, ii, 568, 1905.
v. Hippel. A. f. O., lxxix, 545, 1911; xcv, Schweigger. A. f. O., v. (1), 96, 1859; ix (1),
173, 1918. 192, 1863.
Inouye. A. f. O., lxxxi, 118, 1912. de Schweinitz and Shumway. Am. J. Med.
Iwanoff. A. f. O., xi (1), 136, 1865; xv (2), Sc., xxxvii, 283, 1901.
88, 1869. Seefelder. A. f. Aug., c.—ci, 334, 1929.
Jaensch. B. O. G. Heidel.., xlviii, 265, 1930. Sugita. A. f. O., czv, 260, 1925.
Royanagi. A. f. O., czkxiii, 173, 1935. Tepljaschin. A. f. Aug., xxviii, 354, 1894.
FCrückmann. A. f. O., xxxvii (3), 644; Theodore, Herzog Carl. A. f. O., xxv (3),
xlviii (1), 237, 1899; lx, 350, 452, 1905. 111, 1879.
Z. f. Aug., xxxvii. 1, 1917. Wagenmann. A. f. O., xxxvi (4), 1, 1890.
B. Non-specific Retinal Inflammations
Inflammations of the retina have in a general sense a twofold aetiology
primary infections, wherein the agent is carried first to the retina by the blood-
stream, and secondary infections, wherein the retina is infected secondarily
to an inflammation already established in the eye. These latter are the more
common ; usually the infection starts in the uveal tract and thence may
spread to the retina, most frequently from the choroid, but sometimes from
the anterior segment ; occasionally a retinitis is a sequel of a purulent
process in the vitreous which is usually of exogenous origin. These we shall
now proceed to consider.
I. ExOGENOUS PURULENT RETINITIS
Eacogenous purulent retinitis follows the introduction of infection into
the globe by a perforating wound or ulcer, and forms part of the picture of
ACUTE PANOPHTHALMITIS, the aetiology, pathology and clinical course of
which we have already fully discussed.” It is of interest to remember that
the retina is first and most acutely attacked at the ora and near the disc,
1 p. 2423. * p. 2221.
DISEASES OF THE RETINA 2637
the first region being infected by direct continuity with the ciliary body and
the second because of the concentration of toxins at the posterior pole of the
eye. It will be remembered also that the retina is totally destroyed, and
that although the acute polymorphonuclear infiltration may be replaced
by a sub-acute mononuclear inflammation so that a pseudoglioma or phthisis
bulbi results, the more usual termination is loss of the eye.
II. ENDOGENOUS PRIMARY RETINITIS
Endogenous infections of the retina may assume many diverse forms
depending on the degree of severity of the haematogenous infection. These
include a fulminating and destructive panophthalmitis, more localized
bacterial lesions, mitigated infections which remain as a peri-vasculitis, toxic
manifestations which may be general, as in exudative toxic retinitis, or
disseminated as in septic retinitis, and chronic affections the aetiology of
which is difficult to determine, as exudative retinitis.
(a) ACUTE METASTATIC RETINITIs
1. METASTATIC PANOPHTHALMITIS
The occurrence of an acute purulent retinitis due to the lodgement
of infective emboli in the retinal circulation was first recognized by Virchow
(1856), and its frequency was established by Herrnheiser (1892–4) and
Axenfeld (1894). These authors pointed out that a single large embolus
was a rarity, the more usual method of infection being a diffuse and wide-
spread dissemination of many foci in the smaller terminal vessels. The
ease of such an occurrence was experimentally demonstrated by Stock
(1903–7), Rados (1913), Seguini (1923), and others. It is probable, indeed,
that the retina is more frequently the site of such emboli than the uvea ;
but whichever tissue is attacked, the resultant picture of panophthalmitis
is the same and has already been studied."
Axenfeld. A. f. O., xl (3), 1 ; (4), 103, 1894. Stock. K. M. Aug., xli., Beil., 17, 1903.
Herrnheiser. K. M. Aug., xxx, 393, 1892; A. f. O., lxvi, 1, 1907.
xxxii, 137, 1894. Virchow. A. f. path. Amat., ix, 307; x, 170
Rados. A. f. O., lxxxvi, 213, 1913. 1856.
Seguini. An. di Ott., li, 301, 1923.
2. LOCALIZED ACUTE METASTATIC RETINITIS
Much more rarely the inflammatory process forms an abscess and
remains localized—until the eye is removed because of pain. A case of this
nature was reported by Spicer (1907) in a boy where a metastatic abscess
showing a colony of staphylococci in its centre formed a large tumour at the
1 p. 2221.
2638 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2230-ACUTE METASTATIC RETINITIs.
The condition was followed by secondary optic atrophy (Todd, Brit. J. O.),
-
Fig. 2231.-METAstatic ENDophthal Mitis.
Section through an abscess at the disc showing a colony of staphylococci
(Holmes Spicer, T. O. S.).


DISEASES OF THE RETINA 2639
disc encapsuled in fibrous tissue (Fig. 2231). Sometimes a still more
mitigated process occurs, when a patch of acute inflammation accompanied
by profuse haemorrhages and exudates appears, usually associated with con-
siderable and widespread oedema, especially of the macular region. A case
of this type, developing after a cold in the head, was described by Todd
(1926) (Fig. 2230). It is noteworthy that a favourite site is at the disc,
the site of election for emboli. In these cases vision may be gravely and
permanently impaired, and when the optic disc is involved atrophy may
€IASU162.
Spicer. T. O. S., xxvii, 230, 1907. Todd. Brit. J. O., x, 230, 1926.
(b) PERI-VASCULITIs
PERI-VASCULITIs of the retina is a relatively common condition due to
an inflammation of infective or toxic origin around the walls of the blood-
vessels. Many cases are due to the spread of infection from the ciliary body
to the retina ; these will be considered presently." Others are haematogenous
in origin and represent the metastatic distribution of some systemic infection,
preferentially in the veins (PERI-PHLEBITIs) and sometimes in both arteries
(PERI-ARTERITIs) and veins. Its most striking clinical feature is the
occurrence of haemorrhages in the retina or the recurrence of haemorrhages
into the vitreous (Eales’ Disease).”
AEtiology. Peri-vasculitis of the retina is not the clinical expression
of a single disease, but a clinical manifestation common to many infections.
Some of the more acute types are almost certainly due to a mild septicaemia,
such as the transient bacillaemias which occur in focal and particularly dental
sepsis (Fig. 2242, Plate L). A typical case, for example, due to metastatic
infection from a skin abscess was reported by Spicer (1907) and another in
which infected tonsils seemed to have aetiological significance by Knapp
(1935). A disastrous case associated with general infective symptoms after
removal of an infected tooth was reported by Bender (1935) which resulted in
complete blindness, while Friedenwald and Rones (1931) encountered such
lesions in a case of streptococcal septicaemia. Other cases presumably due
to focal infections have been reported by Lewis (1918), Finnoff (1921), Ellett
(1923), Radcliffe and Young (1923), Redding (1924), Godwin (1927), Swab
(1935), and others. The vast majority of the more chronic cases, especially
those occurring in young adults, are probably due to tubercle, a matter which
will be discussed in detail presently * (Fig. 2243, Plate L). Syphilis seems to
be an unimportant factor although a positive Wassermann reaction has been
noted (Santowsky, 1933). Finally, a large number of cases occur wherein
tubercle and sepsis have been excluded after the most exhaustive examination,
which cannot be placed in any obvious category (Ballantyne and Michaelson,
1937).
1 p. 2656. * p. 2602. 8 p. 2668.
T. O. —WOL. III. N N
Figs. 2232 to 2237.-PERI-PHLEBritis RETINE.
An infective case of unknown origin.
-
-
º º º º
-
- ".
º
-
Fig. 2234-Complete closure of lumen Fig. 2235–Final conversion of vein into
of vein without interruption of the a fibrous knot.
endothelium.
Fig. 2236–Endothelial proliferation of Fig. 2237.-Infiltration of
- artery
ven- (Ballantyne and Michaelson, Brº.
J. O.).










DISEASES OF THE RETINA 264. I
The pathology of the condition is fairly well understood, and although such eyes
are not frequently made available for histological investigation unless in their very
late stages, a considerable number of examinations have been reported (Fleischer, 1914;
Axenfeld, 1920; Eppenstein, 1920; Suganuma, 1927; Goldstein and Wexler, 1930;
Birnbaum, Prinzmetal and Connor, 1934; Gilbert, 1935; v. Hippel, 1935; Marchesani,
1936 ; Ballantyne and Michaelson, 1937). Of those the majority were tuberculous
(Fleischer, Goldstein and Wexler, Gilbert, v. Hippel), and two were typical of
thrombo-angiitis (Birnbaum, Marchesani), while others were non-specific.
The affection usually starts in the peripheral vessels, although quite
frequently in the larger branches, and the veins are more usually affected
than the arteries or capillaries. The primary condition is thus essentially
a peri-phlebitis, but occasionally the arteries, especially the superior temporal,
are associated with most haemorrhages (Derby, 1921 ; Kyrieleis, 1933;
Swab, 1935). In the more acute lesions the infiltration is of polymorpho-
nuclear cells (Friedenwald and Rones, 1931), in the more chronic or later
lesions it becomes mainly mononuclear, consisting of lymphocytes, sometimes
with epithelioid cells (Ballantyne and Michaelson, 1937). At first the
cellular infiltration is seen within the vessel wall, limiting itself to one
part in the early stages (Fig. 2232), but rapidly becoming annular (Fig. 2233);
until eventually it obliterates the lumen without impairing the endo-
thelium (Fig. 2234), the whole vessel being finally converted into fibrous
tissue (Fig. 2235). At other times there is a proliferation of the endothelium
(Fig. 2236); and at yet other times the exudative cells themselves invade
the lumen of the vessel directly, occluding it in this way (Fig. 2238). These
changes are usually seen in their most advanced stage in the peripheral
venules, but the process spreads in the direction of the blood-current back-
wards so that similar changes eventually occur in the central vein in the
optic nerve (Fig. 2239). The infiltration of the arterial walls is similar, at
the onset being well away from the endothelial lining and yet confined by
the perivascular space (Fig. 2237).
The clinical picture in the more acute septic cases is one of severe
haemorrhagic retinitis with much distension of the veins, with oedema and a
profusion of haemorrhages and sometimes exudates around the vessels
(Figs. 2242 and 2243 ; Plate L). ; frequently the exudative reaction
gives the appearance of “white-sheathing ” to the vessels, the blood-column
being enclosed between parallel white lines. As a rule these appearances
advance from the peripheral radicles and spread towards the disc (Ballantyne,
1909; Igersheimer, 1912 ; Davis, 1920; Kokott, 1935), but occasionally
the reverse progress is noted and the central veins are affected first (Davis,
1920; Ballantyne and Michaelson, 1937). The changes are usually most
marked where the vein bifurcates, but all over the involved area they tend
to progress rapidly : haemorrhages, both superficial and deep, increase and
are sometimes not associated with visible vessels, the veins become swollen
and beaded, broad areas of exudation appear occasionally burying the vessels,
the affected area or the whole retina may become oedematous, and eventually
N. N. 2
2642 TEXT-BOOK OF OPHTHALMOLOGY
the picture of venous thrombosis may result. At the end the affected vessels
may be represented by narrow branching white fibrotic lines, the origin of
which, whether arterial or venous, is often difficult to trace, while new
anastomotic vessels may appear on the retina in considerable profusion or
grow exuberantly into the vitreous. At any time, however, the fundus
may be obscured by a vitreous hæmorrhage. As end-results, a sudden
glaucoma may terminate the illness rapidly, or the gradual formation of a
rete mirabile in the vitreous," or of a retinitis proliferans, may slowly abolish
Figs. 2238 to 2239.-PERI-PHLEBITIs RETINAE.
Exudate invading and commencing to occlude a vein.
Fig. 2238. Transverse section. Fig. 2239.-Longitudinal section of the
central vein (Ballantyne and Michaelson,
Brit. J. O.).
vision. The picture in the more chronic cases is typified in that of tuber-
culous peri-phlebitis, under which heading it will be discussed,” while other
cases occur of an extremely mild nature, transient in duration and dis-
appearing without trace (Rohner, 1930).
As complications a uveitis may develop at a later stage, as well as infections of a
similar nature elsewhere in the body. Not the least interesting of these are symptoms
of a similar and usually diffuse vascular cerebral lesion, manifested by epileptic fits.
hemiplegia, and soon (Ballantyne, 1909; Ballantyne and Michaelson, 1937).
So far as treatment is concerned little can be done of any local value.
Attention should be paid to determining the causal infection and dealing
with it by general systemic and bacteriological measures.
p. 2618. * p. 2668.

DISEASES
2643
OF THE RETINA
Axenfeld. B. O. G. Heidel.., xlii, 298, 1920.
Ballantyne. Ophthalmoscope, vii, 662, 1909.
Ballantyne and Michaelson. Brit. J. O., xxi,
22, 1937.
Bender. Am. J. O., xviii, 148, 1935.
Birnbaum, Printzmetal and Connor.
Med., liii, 410, 1934.
Davis. T. Am. O. S., xviii, 55, 1920.
Derby. T. Am. O. S., xix, 253, 1921.
Ellett. Am. J. O. vi., 423, 1923.
Eppenstein. A. f. O., ciii, 154, 1920.
Finnoff. T. Am. O. S., xix, 238, 1921.
Fleischer. K. M. Aug., lii, 769, 1914.
Friedenwald and Rones. A. of O., v., 175,
1931.
Gilbert.
Godwin.
A. Int.
K. M. Aug., xciv, 335, 1935.
Am. J. O., x, 171, 1927.
Goldstein and Wexler. A. of O., iii. 552, 1930.
v. Hippel. A. f. O., exxxiv, 121, 1935.
Igersheimer. A. f. O., lxxxii, 215, 1912.
Knapp. K. M. Aug., xciv, 748, 1935.
Kokott. K. M. Aug., xeiv, 327, 1935.
Kyrieleis. A. f. Aug., cvii, 182, 1933.
Lewis. J. Am. Med. As., lxx, 1813, 1918.
Marchesani. A. f. Aug., cik, 124, 1936.
Radcliffe and Young. Am. J. O., vi, 496,
1923.
Redding. Atlantic Med. J., xxvii, 640, 1924.
Rohner. K. M. Aug., lxxxiv, 748, 1930.
Santowsky. A. de Oft. B. A., viii, 44, 1933.
Spicer. T. O. S., xxvii, 230, 1907.
Suganuma. A. f. O., exviii, 443, 1927.
Swab. A. of O., xiii, 620, 1935.
(c) PERI-ARTERITIs NoDos A
The nature of this rare disease has already been discussed," when it
was pointed out that it was characterized by the development of multiple
infective nodules of leucocytes, lymphocytes and plasma cells infiltrating
the entire thickness of the artery walls as the result of some generalized
(and usually fatal) infection. The occurrence of the typical lesions in the
retinal vessels is rare, but has been reported by Müller (1899), v. Herren-
schwand (1929), Böck (1929), and King (1935).
Fig. 2240.-PERI-ARTERITIs NoDos A.
Section of retina showing generalized inflammatory infiltration affecting par-
ticularly the arterial walls and peri-arterial zone (King, T. O. S.).
1 p. 2374.

2644 TEXT-BOOK OF OPHTHALMOLOGY
In Müller's case the inflammatory changes appeared earliest in the adventitia,
while later lesions showed involvement of the entire thickness of the arterial wall with
a fibrinous exudate into the media accompanied by necrosis. A certain amount of
endothelial proliferation was present as well as retinal oedema in the vicinity of the
lesion. In v. Herrenschwand's and Böck's cases the central artery was involved:
and in King's there was an intense general infiltration of the retina with lymphocytes
and plasma cells accentuated in the peri-arterial regions, while the normal structure of
the vessel walls was obliterated and endothelial proliferation was marked (Fig. 2240).
A unique case was reported by Goldstein and Wexler (1932) in a case of acute
disseminated lupus erythematosus wherein the arteries of the retina, alone among the
arteries of the body, were affected first by a plasma cell infiltration followed by
atrophy of the inner layers of the retina and the optic nerve.
Böck. Z. f. Aug., lxix., 225, 1929. King. T. O. S., lv. 246, 1935.
Goldstein and Wexler. A. of 0., viii, 852, Müller. Fest, d. Stadt. Krankh. Dresden, 458,
1932. 1899.
v. Herrenschwand. K. M. Aug., lxxxiii, 419,
1929.
(d) Toxic ExupATIVE RETINITIs
Pseudo-NEPHRITIC RETINITIs (or LEBER): STELLATE RETINITIs
We have already seen that a condition of pronounced retinal oedema, in
the absence of disease of the arteries or kidneys, and despite the diversity
Fig. 2241.-RETINITIs STELLATA.
Origin unknown (Goldsmith, T. O. S.).
p. 2591.

PLATE L
RETINITIs
Fig. 2242. –PERI-ARTERITIs of Fig. 2243.−TUBERCULouis PERI-PHLEBITIs.
STREPTococcAL ORIGIN.
Fig. 22.45.-Exudative RETINITIs Fig. 2246.-ExupATIVE RETINITIs
(of Coats). Early stage. (of Coats). Late stage.
[To face p. 2644


DISEASES OF THE RETINA 2645
of its aetiological factors, may produce a fairly uniform ophthalmoscopical
picture resembling renal retinopathy in the presence of exudates and
haemorrhages, a blurring of the optic disc, and the formation of a macular
star (Fig. 2241). As a descriptive term to embrace these cases Leber (1909)
proposed the name RETINITIS PSEUDO-NEPHRITICA STELLATA. It is to be
remembered, however, that the macular star is but an incidental, although
common distribution of the Oedema and exudates, and other similar cases
occur wherein peripheral exudative patches appear without the central
figure (Doyne, 1904) (Fig. 2244, Plate L). The macula, however, seems
especially liable to the accumulation of toxic oedema and exudates, perhaps
because its avascularity prevents the resorption of fluid, and because
Henle’s layer, like the nerve-fibre layer, owing to its loose fibrillar structure,
can readily swell and absorb larger quantities of fluid.
It is to be remembered, however, that the term does not connote a clinical entity.
We have seen, for example, that such a clinical picture may occur in conditions of
pure circulatory disturbance, such as ocular or cerebral traumatism, obstruction of a
retinal artery or vein, retinal peri-phlebitis or papilloedema.
A group of cases, however, occurs wherein the aetiology is undoubtedly
toxic, in which case the retinitis is due to damage to the capillary endothelium.
Thus it has been noted to occur in acute febrile illnesses, such as measles
(Stewens, 1930), influenza (Hartridge, 1893; Cross, 1893; Maklakoff, 1909;
Danco, 1921 ; Nadeldinger, 1927; Bakker, 1928), meningitis (Leber, 1909),
erysipelas (Vossius, 1880), focal infections such as infected teeth (Coppez,
1924), chronic infections, as tubercle (Kaminskij, 1929; Kleiber, 1930) or
syphilis (Schieck, 1910; Onfray and Margerin, 1923; Junius, 1930; Fewell,
1932) or parasitic infections such as taenia (Huber, 1932). In a considerable
number of other cases no aetiology can be found (Doyne, 1904; Goldsmith,
1908 ; Batten, 1909; Parsons, 1909 ; Pascheff, 1928; Zanettin, 1932).
Since the condition is merely a local symptom of a general disturbance,
treatment should be diverted to the latter ; and provided the causal factor
can be combated or eliminated the prognosis is generally good, for resolution
is the rule.
(e) RETINITIs CACHECTICORUM (PICK)
A condition somewhat related to the preceding, and characterized by
the appearance of haemorrhages, sometimes in abundant quantity, exudates,
and occasionally of a macular star, is seen in advanced anaemias, such as
chlorosis (Bitsch, 1879; Mackenzie, 1885; Werner, 1905; Augstein, 1919),
and in cachectic patients with extreme anaemia especially with carcinomatous
lesions—the RETINITIS CACHECTICORUM OF PICK (1901). Pathologically an
atrophy of the nerve-fibres and a profound lipoid degeneration of the retinal
substance has been observed (Nakaizumi, 1912), and it is probable that in
these cases the toxic factor is more important than the anaemic (Puscariu
and Nitzulescu, 1931).
2646
TEXT-BOOK OF OPHTHALMOLOGY
Augstein.
Bakker.
Batten. T. O. S., xxix., 35, 1909.
Bitsch. K. M. Aug., xvii, 144, 1879.
Copper. A. d’O., xli., 113, 1924.
Cross. T. O. S., xiii, 79, 1893.
Danco. K. M. Aug., lxvii. 87, 1921.
Doyne. T. O. S., xxiv, 123, 1904.
Fewell. A. of O. viii. 615, 1932.
Goldsmith. T. O. S., xxviii, 177, 1908.
Hartridge. T. O. S., xiii, 77, 1893.
Huber. K. M. Aug., lxxxviii, 507, 1932.
K. M. Aug., lxiii, 174, 1919.
Ned. Tij. v. Gen., lxviii. 556, 1928.
Nadeldinger. Bull. S. d’O. Paris, xxxix. 311,
1927.
Nakaizumi. K. M. Aug., 1 (1), 290, 1912.
Onfray and Margerin. A. d’O., x1, 635, 1923.
Parsons. T. O. S., xxvii. 121, 1907; xxix,
159, 1909.
Pascheff. B. O. G. Heidel. xlvii. 315, 1928.
Pick. K. M. Aug., xxxix (1), 177, 1901.
Puscariu and Nitzulescu. Brit. J. O., xv,
697, 1931.
Schieck. K. M. Aug., xlviii (2), 150, 1910.
Zb. ges. 0., xxi, l, 1929.
Junius. Z. f. Aug., lxx, 129, 1930. Stewens. K. M. Aug., lxxxiv, 714, 1930.
Kaminskij. Russ. O. J., ix. 742, 1929. Vossius. K. M. Aug., xviii, 410, 1880.
Kleiber. Z. f. Aug., lxx, 187, 1930. Werner. T. O. S., xxv, 93, 1905.
Leber. A. f. O., lxx, 200, 1909. Williamson-Noble. Brit. J. O. vi. 67, 1922.
Mackenzie. Brit. Med. J., i. 328, 1885. Zanettin. An, dº Ott., lx, 411, 1932.
Maklakoff. K. M. Aug., xlvii (1), 165, 1909.
(f) RETINITIs SEPTICA (of Roth)
RETINITIs septica is a mild and benign condition first described by
Roth (1872) occurring in patients with systemic infections, particularly
Fig. 2247.-RETINITIs SEPTICA or Roth (Doherty).
endocarditis (Doherty and Trubek, 1931), characterized by the appearance
of round or oval white spots in the retina usually near the disc, and frequently
associated with haemorrhages (Fig. 2247). It seems probable that the picture
is not caused by the action of infective emboli but is rather the result of an
endothelio-toxin derived from organisms of relatively low virulence.
Although Litten (1880) claimed that it occurred in 80% of septic

DISEASES OF THE RETINA 2647
infections, a figure which he reduced later (1902) to 30–40%, and Herrnheiser
(1894) in 32%, the ophthalmological picture is not common. The spots
may develop with great rapidity (within half an hour, Litten, 1877), and
while mainly near the disc they do not occur at the macula ; they are not,
however, necessarily associated with blood-vessels, but when they happen
to be near them they lie underneath. Typically a haemorrhage surrounds
the spot so that the former appears as if it had a white centre. The
remainder of the fundus and of the eye may show no other changes, and the
spots themselves are transient, but in cases of endocarditis they frequently
appear and disappear in successive crops.
Several pathological examinations have been made of this condition
(Kahler, 1880; Herrnheiser, 1894; Goh, 1897; Ischreyt, 1900; Michel,
1902 ; Grunert, 1902 ; Dellmann, 1919; Gilbert, 1920, Krückmann, 1920).
In many of the cases bacterial emboli have been found (staphylococci,
streptococci, pneumococci, etc.), but they appear to have been organisms
of little virulence which excited little or no reaction. In fact the absence
of tissue-reaction is everywhere striking—indefinite collections of leucocytes,
without any gross infiltration, some oedema and occasional exudates, and
scattered areas where cellular staining is faint and the endothelium of the
capillaries becomes indefinite and shows hyaline changes. The white spots
are due to varicose degeneration of the nerve-fibres, as originally postulated
by Roth (1872), which may become granular or hyaline.
In many respects the disease is analogous to the ophthalmia lenta of Gilbert
(1925) or the septic choroiditis of Friedenwald and Rones (1931).” It is probable that
it represents the effects of local toxic action on the capillaries, and that emboli of every
degree of virulence may occur from this attenuated form to the most virulent types, a
probability which is suggested by the simultaneous appearance of other infective
lesions in association with septic retinitis, such as a choroiditis in the same eye
(Schüssele, 1909) or a frank purulent metastatic panophthalmitis in the other
(Cantonnet, 1913). -
So far as the eye is concerned the prognosis is good, and the treatment
is to be directed entirely to the general causal condition.
Cantonnet. A. d’O., xxxiii, 425, 1913. Ischreyt. A. f. Aug., xli., 65, 1900.
Dellmann. K. M. Aug., lxiii, 661, 1919. Kahler. Prag. Z. f. Heilk., i, 111, 1880.
Doherty and Trubek. J. Am. Med. As., Krückmann. A. f. path. Amat., ceXxvii, 227,
xcvii, 308, 1931. 1920.
Friedenwald and Rones. A. of O., v., 175, Litten. B. O. G. Heidel., x, 140, 1877.
1931. Z. f. kl. Med., ii, 378, 1880.
Gilbert. A. f. Aug., lxxxvi, 29, 1920; xcvi, Med. W., Berlin, i, 158, 1902.
I 19, 1925. Michel. Diss., Tübingen, 1902.
Gimurto. Diss., Strassburg, 1893. Roth. A. f. path. Amat., lv., 197, 1872.
Goh. A. f. O., xliii (1), 147, 1897. Deut. Z. f. Chir., i, 471, 1872.
Grunert. B. O. G. Heidel., xxx, 338, 1902. Schüssele. K. M. Aug., xlvii (2), 50, 1909.
Herrnheiser. K. M. Aug., xxx, 393, 1892;
xxxii, 137, 1894.
1 p. 2139.
2648 TEXT-BOOK OF OPHTHALMOLOGY
(g) ExTERNAL ExUDATIVE RETINITIs (OF COATs)
(SERO-FIBRINOUS DEGENERATIVE CHORIO-RETINITIS OF LEBER)
We have already seen that the occurrence of large masses of white or yellow
exudation underneath the retina usually besprinkled with glistening cholesterol
crystals, appearing in young people, particularly males who are apparently healthy
and are without significant personal or family history, was originally described as a
clinical entity in a classical paper which will always serve as a model of what a clinico-
pathological study ought to be, by George Coats (1908) under the heading of eacternal
haºmorrhagic retinitis. He attributed the condition to the organization of haemorrhages
from the outer plexiform layer of the retina into the sub-retinal space; and although
later (1912) he suggested the alternative title eacudative retinitis, he still upheld the
same aetiology. Previously a number of cases had appeared in the literature of con-
ditions which might well be gathered into the same category, among the most typical
of which are those of Nettleship (1876), Brailey (1876), Snell (1886), Lawford (1888),
Collins (1889), Cramer and Schultze (1894), Guaita (1895), Weltert (1896), Gunn (1899),
Schieck (1900), Feilchenfeld (1901–07), Lawson (1903), Paton (1903), Jessop (1903),
Parsons (1904), Bickerton (1905), Hancock (1905), v. Geuns (1905), Doyne (1906),
v. Hippel (1906), Guzmann (1907) and Krauss and Brückner (1907). Shortly after
Coats's paper appeared v. Hippel (1913) suggested that in a case described by him,
vascular changes played a minor part, a view substantiated by Leber (1915), who
denied the haemorrhagic aetiology and considered the disease embolic or toxic in origin,
calling it sero-fibrinous degenerative chorio-retinitis. Subsequent work has established
that such a clinical entity, dependent on exudative processes of a mild inflammatory
nature, does exist (Jervey, 1919; Hanssen, 1920; Davis, 1921 ; Meller, 1922;
Wölfflin, 1926; Marshall and Michaelson, 1933; Lamb, 1938).
The aetiology of the inflammatory basis of the disease is unknown.
It is composite, and Coats (1908) himself suggested that no one cause was
probably responsible. Syphilis (Galezowski, 1911; Rados, 1921 ; v. Hippel,
1931) and tubercle (Axenfeld and Stock, 1911 ; Hanssen, 1920 ; Fleischer,
1925; v. Hippel, 1931) have been suggested as well as other infections and
febrile conditions (Holm, 1917; Wiegmann, 1925; Friedenwald, 1925),
or local metastatic emboli (Leber, 1915; v. Hippel, 1931), but in the majority
of cases no cause can be found either locally in the eye or in the general
health or history. The fact that in many cases there are anomalies of the
smaller blood-vessels suggests that many of them have a congenital basis,
either structural or functional, which allows exudations or haemorrhages to
occur more readily than usual (Junius, 1929–34), a supposition supported
by the very similar picture presented by angiomatosis in its late stages and
by the retinal degeneration associated with miliary aneurysms. -
However this may be, the lesion occurs, usually uni-ocularly, in
apparently healthy young people, especially males. Coats (1908) gave the
average age as 16 and the preponderance of males as 14 : 5. Leber (1915)
found that 80% of the cases were under 25. In 6 cases reported by Mayou
(1928) of 5 boys and 1 girl the oldest was 14 and the youngest 5. Marshall
1 p. 2610.
DISEASES OF THE RETINA 2649
Fig. 2249.-Exupative RETINITIs of CoATs (Harrison Butler, T. O. S.).

2650 TEXT-BOOK OF OPHTHALMOLOGY
and Michaelson (1933) reported 5 cases, all under 5 years, including one of
12 and one of 10 months; while Lamb (1938) described a case at 8 months.
The clinical picture presented by these cases is characteristic (Figs. 224.5
and 2248–49, Plate L). Irregular and prominently raised masses of white or
yellowish exudation are seen in the fundus, usually of a woolly or flocculent
consistency with soft edges, lying under the retinal vessels, which, however,
may be slightly obscured in places. Most frequently the exudative areas are
near the posterior pole, they may encircle the disc, less commonly the macula
is involved, but quite frequently they are peripheral or there may be a
combination of central and peripheral patches. The presence of glittering
spots suggestive of crystalline deposits is characteristic and haemorrhages
are frequently present. In many cases abnormalities of the vessels are
seen—loops, beading, spiral tortuosities, glomerular-like tufts, and anomalous
anastomoses (Coats, 1908; Olsho, 1929; and many others).
The clinical course is very slow and chronic. A retinal detachment
very frequently occurs which may become complete, while irido-cyclitis or
glaucoma may supervene. In other cases the exudative mass grows slowly
until it appears just behind the lens constituting a pseudo-glioma (Fig. 2246,
Plate L); while in yet other cases the process may progress so far and then
remain stationary indefinitely. The chronicity of the process, apart from
the fact that it has frequently been watched for many years, is seen in the
fact that in a number of cases the affected eye has been divergent,
suggesting a congenital basis.
The prognosis is therefore bad, and no effective treatment is known.
Pathological studies of these eyes have usually been made from excisions under-
taken under the diagnosis of a suspicious pseudo-glioma : the advent of glaucoma has
provided others. Most of them unfortunately have shown an advanced degree of the
condition (Brailey, 1876; Lawford, 1888; Collins, 1889; Cramer and Schultze,
1894; Guaita, 1895; de Schweinitz and Shumway, 1901 ; Parsons, 1904; Hancock,
1905; v. Hippel, 1906–31 ; Coats, 1908–12; Berg, 1919; Meller, 1922; Gourfein-
Welt, 1922; Sattler, 1925; Doesschate, 1927; Llorca, 1932; Koyanagi, 1935; Daniel,
1937; and others). The earliest cases have been described by Rados (1921), Marshall
and Michaelson (1933) and Lamb (1938).
The essential pathological feature in the majority of the earliest cases
is usually an infiltration of the retina, particularly in its outer layers, with
albuminous and oedematous fluid, leading to an exudate of the same fluid
under the retina, and resulting in a complete or partial detachment of this
tissue (Lamb, 1938) (Fig. 2250). In some cases the oedematous fluid is
replaced by hamorrhage (Coats, 1908). In other cases peri-vasculitis may
be seen, suggesting a mild inflammatory process (Fig. 2251); and in all
cases vascular sclerosis ultimately appears which is probably secondary to
the retinal degeneration (Fig. 2252). In most of the early cases the
pigment epithelium has been described as normal ; while the choroid is
initially unchanged. One particular feature, however, is the presence in
DISEASES OF THE RETINA 2651
Early stage. The retina is swollen and infiltrated with albuminous fluid, which
has collected in the sub-retinal space causing a detachment (Lamb, Am. J. O.).
Fig. 2251.-Infiltrative stage. Vascular Fig. 2252.-Advanced stage. Above is
disease of retina in neighbourhood of a greatly thickened vessel with
exudative mass showing leucocytic infil. eccentric lumen. Below (at A),
tration of vessel wall and peri-vascular smaller vessels completely obliterated
space. The rods and cones are absent and showing advanced hyaline
and the outer nuclear layer degenerated, degeneration (Coats, R. L. O. H.
and bladder cells are present in the sub- Rep.).
retinal exudation (x 120) (Coats,
R. L. O. H. Rep.).
FIGs. 2251 and 2252.-ExupATIVE RETINITIs of Coars: Vascular CHANGEs.






2652 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2253.-ExupATIVE RETINITIs of CoATs.
The rods and cones are absent and the outer reticular and nuclear layers are
characteristically infiltrated by bladder cells (Marshall and Michaelson T. O. S.).
Fig. 2254-ExupATIVE RETINITIs or Coats.
Massive infiltration of the sub-retinal space and outer retinal layers by bladder
cells (Marshall and Michaelson, T. O. S.).


DISEASES OF THE RETINA 26.53
the retina and sub-retinal space of large bladder-like cells, occasionally in
enormous quantities (Figs. 2253–54).
These cells, which form a prominent feature of the disease, although they are
met with in other conditions, such as albuminuric retinitis, have excited considerable
interest, and the various controversial views which have been put forward as to their
origin have already been discussed.* The majority of opinion, however, considers
that they represent a transformation of the hexagonal pigment cells (Llorca, 1932;
Koyanagi, 1935; Daniel, 1937; Lamb, 1938; and others).
In the later cases this exudative appearance is replaced by the develop-
ment of large amounts of connective tissue between the choroid and the
FIG. 2255.-Exu DATIVE RETINITIs of CoATs.
General view of retina. A. Cavity filled with débris and calcareous particles.
B. Retina. C. Fibrous tissue between retina and choroid stretching from the dise
to the ora (O). P. Pigmentary epithelium invading the fibrous tissue. E. Choroid
(Coats, R. L. O. H. Rep.).
retina and in the substance of the retina itself (Fig. 2255). The choroid is
involved only secondarily and not constantly. The pigmentary epithelium,
however, always eventually shows a considerable degree of proliferation, its
cells, as we have seen, wandering into the sub-retinal space and the retina
itself. These cells assume a preponderant rôle in the formation of the
1 p. 2633.

2654 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2256.-ExupATIVE RETINITIs of CoATs.
A. A mass of fibrous tissue between retina (B) and choroid (E)(Coats, R. L. O. H.
Rep.).
exudative mass, first as macrophages, and then by changing to
fibroblasts and laying down fibrous tissue. Eventually, however, gross
degenerative changes set in, both in the blood-vessels (Fig. 2252) and in the
retinal tissues (Fig. 2256); and cavities appear in the masses of cicatricial
tissue filled with all kinds of débris, the pigmented remains of haemorrhages,
Fig. 2257. ExupATIVE RETINInns of CoATs.
A focus of encapsulation loose in the sub-retina fluid. Newly formed connec-
tive tissue covers the periphery while the centre is occupied by a necrotic mass con-
taining cholesterol crystals (Lamb, Am. J. O.).


DISEASES OF THE RETINA 2655
hyaline connective tissue, giant cells, cholesterol crystals, and calcareous
particles (Fig. 2257). The retina, of course, in the affected area is extremely
degenerated and usually almost completely replaced by fibrous tissue, the
vessels are sclerosed, the neural elements destroyed and degenerated, and
the neuroglial tissues have proliferated to a varying degree.
The differential diagnosis is frequently difficult or impossible to make on clinical
grounds. From eacternal haemorrhagic retinitis (Coats) it is frequently impossible ;
but the vascular new-formations in angiomatosis and retinal degeneration with miliary
aneurysms usually indicate the nature of the case. Especially in the later stages a
diagnosis from an inflammatory pseudo-glioma due to uveitis may be difficult ; but
the most important point in the diagnosis is retino-blastoma. This will be considered
at a later stage." The age of the patient is sufficient to differentiate the condition
from senile eacudative retinitis (disciform degeneration of the macula).”
Axenfeld and Stock. K. M. Aug., xlix (1),
28, 1911.
Berg. A. f. O., xcviii, 21 1, 1919.
Bickerton. T. O. S., xxv, 102, 1905.
Brailey. R. L. O. H. Rep., viii, 548, 1876.
Coats. R. L. O. H. Rep., xvii, 440, 1908.
O. Rev., xxx, 289, 1911.
A. f. O., lxxxi, 275, 1912.
Collins. T. O. S., ix, 198, 1889 ; xxxi, 112,
1911.
Cramer and Schultze.
1894.
Daniel. A. de Oft., B. A., xii, 384, 1937.
Davis. T. A. m. O. S., xix, 222, 1921.
Doesschate. K. M. Aug., lxxix, 505, 1927.
Doyne. O. Rev., xxv, 154, 1906.
Feilchenfeld. Z. f. Aug., v, 115, 1901.
Cb. pr. Aug., xxxi, 267, 1907.
Fleischer. Z. f. Aug., lviii, 181, 1925.
Friedenwald. A. of O., liv, 111, 1925.
Galezowski. Am. d’Oc., czlvi, 431, 1911.
v. Geuns. Ned. Tij. v. Gen., xli., 179, 1905.
A. f. Aug., xxix, 288,
Gourfein-Welt. Rev. gen. d’O., xxxvi, 149,
1922.
Guaita. A m. di Ott., xxiv, 25, 1895.
Gunn. T. O. S., xix, 62, 1899.
Guzmann. Z. f. Aug., xvii, 40, 1907.
Hancock. R. L. O. H. Rep., xvi, 150, 1905.
Hanssen. K. M. Aug., lxv, 703, 1920.
v. Hippel. A. f. O., lxiv, 157, 1906; lxxxvi,
443, 1913 ; czXvii, 27, 1931.
Holm. K. M. Aug., lix, 319, 1917.
Jervey. Am. J. O., ii, 127, 1919.
Jessop. T. O. S., xxiii, 384, 1903.
Junius. Z. f. Aug., lxviii, 207, 1929.
K. M. Aug., lxxxvi, 577, 1931 ; xcii, 748,
1934.
A. f. Aug., cvi, 475, 1932.
Royanagi. A. f. O., czzxiii, 173, 1935.
Krauss and Brückner. A. f. Aug., lvii, 157,
1907.
Lamb. Am. J. O., xxi, 618, 1938.
Lawford. T. O. S., viii, 178, 1888 ; xxx, 157,
1910.
Lawson. T. O. S., xxiii, 64, 1903.
Leber. G.-S. Hb., II, vii (A), 1267, 1915.
Llorca. A. de Oft., H. -A., xxxii, 349, 1932.
Marshall and Michaelson. T. O. S., liii, 102,
1933.
Mayou. T. O. S., xlviii, 150, 1928.
Meller. Z. f. Aug., xlvii, 247, 1922.
Nettleship. R. L. O. H. Rep., viii, 515, 1876.
Olsho. Am. J. O., xii, 453, 1929.
Parsons. T. O. S., xxii, 255, 1902;
149, 1904.
xxiv,
Paton. T. O. S., xxiii, 63, 1903.
Rados. A. f. O., cv, 973, 1921.
Sattler. K. M. Aug., lxxiv, 222, 1925.
Schieck. B. O. G. Heidel.., xxviii, 88, 1900.
de Schweinitz and Shumway. Am. J. Med.
Sc., xxxvii, 283, 1901.
Snell. T. O. S., vi, 338, 1886.
Weltert. A. f. Aug., xxxii, 187, 1896.
Wiegmann. K. M. Aug., lxxiv, 519, 1925.
Wöfflin. A. f. O., czvii, 33, 1926.
III. Secondary Retinitis
SECONDARY RETINITIS, that is a retinitis arising as a complication of a
primary inflammation of other structures in the eye, is usually a sequel to an
inflammation of the uveal tract ; more rarely an inflammatory process
spreads from the optic nerve or its sheath and invades the retina. As one
would expect the most frequent origin is the choroid, but a very interesting
and important type of retinitis is associated with an irido-cyclitis.
1 p. 2825. * p. 2116.
T.O.-WOL. III. O O
2656 TEXT-BOOK OF OPHTHALMOLOGY
(a) CHORIO-RETINITIs
In our study of inflammations of the choroid we have seen that in most
cases the retina is involved also. In the slighter cases the retinal damage
may be merely nutritional and toxic affecting particularly the neuro-
epithelium by the direct diffusion of toxins ; but in the more severe cases
when Bruch's membrane is broken through, the inflammatory infiltration
invades the retinal tissues, and the two structures are finally welded together
inseparably in the cicatricial scar. The clinical and pathological features
of such conditions have already been fully discussed.* Alternatively
infected cells from a primary uveal focus may migrate, and, settling down on
distant parts of the retina, particularly near the veins, may set up secondary
areas of retinitis at these points (Verhoeff, 1929).
(b) RETINITIs SECONDARY TO TRIDO-CYCLITIS
We have already seen * that a reaction in the retina, particularly in
the posterior pole of the eye, is a commonplace in inflammations of the
anterior segment. The beginnings of this reaction are difficult to study
pathologically, since eyes with early irido-cyclitis are not frequently available
for histological section ; the main source of our knowledge has therefore
been in inflamed eyes which have been excised for trauma.
A considerable amount of knowledge has also been obtained from the study of the
spread of tuberculosis in the eye. We have already discussed in detail “the migration
of actual tubercles from the ciliary body backwards into the vitreous (the wander-
tubercle of v. Szily) (Verhoeff, 1910–29 ; Meller, 1921; Finnoff, 1924–31 ; v. Szily,
1931); while ample demonstration has been made by Straub (1912) and Ohmart
(1933) that after inoculation of the ciliary body a cellular exudate is thrown into the
vitreous, often containing actual tubercle-systems, which produces deposits on the
retina, an oedema of the retina, particularly at the posterior pole and at the papilla,
and a peri-vasculitis.
The simplest expression of retinal involvement is the deposition on its
surface of accumulations of leucocytes bound together by a small quantity
of fibrin (Fig. 2258). These correspond in every way to the keratic deposits
On Descemet's membrane, and may be quite unassociated with any reaction
in the retinal tissues. They may be microscopic in size, or just visible to
the naked eye as white pin-point spots when the inner surface is examined,
or they may reach enormous dimensions and occur in great numbers.
They cannot usually be seen ophthalmoscopically because of the accom-
panying vitreous haze ; but they have been noted histologically in cases of
trauma when post-traumatic inflammation has indicated an early excision
(Elliot, 1917; Lister, 1921 ; Samuels, 1936).
An actual inflammatory reaction in the retina may also occur, presumably
1 pp. 2190, 2205. * p. 2201. 8 p. 2301.
DISEASES OF THE RETINA 2657
the result either of the diffusion of toxins through the vitreous or of the
presence of the causal organisms in the cellular exudation. Such an
inflammation presents three characteristic features.
1. (Edema is frequently marked, especially at the posterior pole where
the nerve-head and the macula are particularly involved. QEdema and
exudation at the nerve-head may give rise to a marked papillitis, the
accentuation at this point being probably partly determined by the
concentration of toxins owing to the drainage of lymph posteriorly, and
partly by the thickness of the nerve-fibre layer, which owing to its looser
structure can imbibe much more oedematous fluid than the other more
closely knit layers of the retina. The macular oedema is of great importance,
Fig. 2258.-PUNCTATE DEPosits on THE RETINA.
In uveitis. The deposit lies above an artery the walls of which show no infiltra-
tion (Lister, T. O. S.).
for it is probably responsible for the marked loss of central vision which
accompanies some cases of irido-cyclitis, and leading, as it may do, to
permanent changes, it may be of considerable gravity. Cystic degeneration
is frequent in such cases, and the oedema may assume such proportions as to
cause a local exudative detachment in the central area (Figs. 2259–60). The
concentration of oedema at the macula is probably due in part to the
presence in this region of the fibre layer of Henle (Wolff, 1931) which like the
nerve-fibre layer adsorbs fluid greedily, and in part to the lack of facilities
for resorption owing to the absence of blood-vessels (Iwanoff, 1869). Its
ready vulnerability is easily understood in view of the delicacy of its struc-
ture and the proximity of its neural elements to the toxins in the vitreous,
unprotected as they are by the inner retinal layers (Casanovas, 1936, and
others).

0 0 -
2658 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2259.-SEcondary RETINITIs.
The macular region in irido-cyclitis. Showing cysts in the retina and a localized
detachment caused by exudative fluid between the rods and cones and the pigment
epithelium (Wolff).
2. A diffuse retinal infiltration is not unusual, mainly with lymphocytes
and plasma cells derived from the retinal blood-vessels, disposed for the
most part in the inner layers between the nerve-fibres and the ganglion cells,
and sometimes marshalled in rows underneath the internal limiting mem-
- - - - - - -
. . . * *
- - -
- - - - -
Fig. 2260.-SEcondary Rºrismis.
The macular region in irido-cyclitis. Showing enormous swelling of Henle’s fibre
layer and a detachment (Wolff).


DISEASES OF THE RETINA 2659
Fig. 2261.-SEcond ARY PERI-PHLEBITIs.
In uveitis showing round-celled infiltration of the walls of a vein (Lister, T.O.S.).
- -
- ---
7- - º
- -----
- - -
- º, -- ---
Fig. 2262.-SEcond ARY PERI-PHLEBITIs.
In uveitis. Showing extension of the peri-vascular infiltration to form a thrombus
in the lumen of the vein (Lister, T. O. S.).


2660 TEXT-BOOK OF OPHTHALMOLOGY
brane (A. Fuchs, 1924). In some cases this is most pronounced near
the Ora Serrata, but in most the posterior pole shows more severe
involvement. -
3. The typical infiltration, however, is around the veins, forming a
peri-phlebitis, which constitutes a distinguishing feature of such cases.
The pathological picture thus presented is similar to that of the peri-
vasculitis of haematogenous origin,” the veins at first showing a round-celled
infiltration of their walls (Fig. 2261), which eventually invades the lumen and
causes thrombosis (Fig. 2262) (Lister, 1921). It is probable that such a
process accounts for the lack of projection of light shown on clinical examina-
tion by some cases when pathological examination subsequently demon-
strates no retinal detachment. It is thought by some authorities (Zeeman,
1911–23) that such a peri-phlebitis is due to the direct spread of the infection
from the ciliary body backwards along the peri-venous lymphatics, but that
the mechanism is through the vitreous is proved in some cases at any rate
by the pathological demonstration of its occurrence in post-traumatic cases
with an iritis and without corresponding involvement of the ciliary body.
Such retinal complications, of course, add considerably to the gravity
of the prognosis of an inflammation of the anterior uvea ; and its treatment
is entirely secondary to that of the primary condition.
Casanovas. A. de Oft., H. -A., xxxvi, 239, Samuels. Am. J. O., xix, 493, 1936.
1936. Straub. T. O. S., xxxii, 60, 1912.
Elliot. Lancet, i, 325, 361, 1917. v. Szily. K. M. Aug., lxxxvii, 13, 1931.
Finnoff. A. of O., liii, 130, 1924. Verhoeff. T. Am. O. S., xii, 566, 1910.
Am. J. O., xiv., 1208, 1931. J. A. m. Med. As., lxiii, 13, 1914.
Fuchs, A. Atlas, Vienna, 1924. A. of O., xlii, 471, 1913; i, 63, 1929.
Iwanoff. A. f. O., xv (2), 88, 1869. Wolff. P. R. S. Med., xxiv, 1049, 1931.
Lister. T. O. S., xli., 275, 1921. Zeeman. A. f. O., lxxx, 259, 1911 ; czii, 153,
Meller. A. f. O., cv, 299, 1921. I923.
Ohmart. Am. J. O., xvi, 773, 1933.
C. Specific Retinal Inflammations
1. SYPHILIS
In a general sense it may be said that syphilitic affections of the retina
are usually associated with and secondary to involvement of the choroid,
forming part of the picture classically described by Förster (1874) as
disseminated syphilitic chorio-retinitis.” There are, however, a number of
cases where the retina is primarily and chiefly involved so that the term
syphilitic retinitis—or since changes at the optic nerve-head frequently enter
largely into the picture—SYPHILITIC NEURO-RETINITIS-is justified. Indeed,
pathological examination has shown that the retina can be markedly affected
while the choroid remains normal (Bach, 1894).
As with other types of retinitis, the inflammation is primarily and
1 p. 2639. 2 p. 2267.
DISEASES OF THE RETINA 2661
essentially associated with the blood-vessels, in and round the walls of which
—arteries, veins and capillaries—a typical round-celled syphilitic infiltration
accumulates, such as has been described elsewhere," the endarteritis being
occasionally so marked that the lumen is invisible and obliterated (Edmunds
and Brailey, 1880; Nettleship, 1886; Spicer, 1892; Uhthoff, 1893; Bach,
1894; Appel, 1894; Rochon-Duvigneaud, 1895–1906; Baas, 1898; Stein,
1903; v. Michel, 1907; Fuchs, 1921; Blatt, 1921; v. Hippel, 1926; and
many others). Although, however, the underlying pathology is the same,
characteristics of distribution determine several clinical types.
(a) DIFFUSE SYPHILITIC NEURO-RETINITIS
A DIFFUse syphilitic NEURO-RETINITIs appearing in the second stage
of the disease was first described by Jacobson (1859) and forms a character-
Fig. 2263-DIFFUse Syphilitic NEURO-RETINITIs.
Acute stage.
istic clinical picture. The entire retina becomes grey, cloudy and opaque,
the changes being localized particularly to the central area, where for a
considerable distance around the nerve-head the swellingisintense, obscuring
completely the margins of the disc (Fig. 2263). So localized may it be that
on occasions the descriptive term RETINITIs CIRCUM-PAPILLARIs (Iwanoff,
1868; Knapp, 1920) is applicable. At the same time the veins are engorged
1 p. 2275.

2662 TEXT-BOOK OF OPHTHALMOLOGY
and tortuous, densely grey exudative areas appearespecially at the macular
region, but haemorrhages are scanty, while details of the picture tend to be
obscured by a fine vitreous haze. As the condition subsides the vessels,
particularly the arteries, usually become much reduced in size showing
marked sheathing or being completely replaced in some regions by
white lines, the disc becomes atrophic, and, particularly round the periphery,
Fig. 2264.—SYPHILITIC NEURO-RETINITIs.
Late atrophic stage-secondary retinitis pigmentosa.
a migration of pigment into the retina assuming the well-known bone-
corpuscle arrangement produces a picture having many resemblances with
primary pigmentary degeneration (Magnus, 1872; Masselon, 1883
Hirschberg and Fehr, 1906) (secondary refinitis pigmentosa) (Fig. 2264).
Complications are not uncommon—a syphilitic irido-eyelitis in either eye or a
diffuse chorio-retinitis in the other (Rochon-Duvigneaud, 1906), and occasionally a
secondary glaucoma of the haemorrhagic type (Stoewer, 1924).

DISEASES OF THE RETINA 2663
Pathologically the lesion is a diffuse affection of the retinal vessels
resulting in oedematous and exudative changes which are confined essentially
to the inner retinal layers (Nettleship, 1886; Bach, 1894; Rochon-Duvig-
neaud, 1906; Igersheimer, 1913).
The symptoms sometimes appear with great suddenness and profound
loss of sight; the central vision is considerably lowered, and a ring scotoma
may occur, while night-blindness may be permanent. The disease is
resistant to treatment which should be carried out on the usual anti-
syphilitic lines.
(b) syPHILITIC PER1-vasculitis (sy PHILITIC RETINAL ANGIOPATHY.)
Occasionally the infection remains localized essentially to the vessels
without exciting a generalized cedema or infiltration of the retinal tissue.
Cases of this type are responsible for most of the examples of retinal arterial
Fig. 2265–SYPHILITIC CENTRAL CHoRIo-RETINITIs (Kitahara, K. M. Aug.).
obstruction or venous thrombosis which occur in syphilitic subjects. Intra-
retinal haemorrhages are common, and they readily become pre-retinal, the
haemorrhage into the vitreous usually resulting in a marked degree of
retinitis proliferans with a considerable tendency to new-vessel formation."
(Nettleship, 1886; Wagenmann, 1897–99; Lawford, 1898; v. Hippel, 1926;
and others). Such an affection of the retinal vessels may be localized to a
segment of the retina or may occur in association with a typical chorio-
retinitic lesion elsewhere; and a massive exudation at one spot may give
rise to a localized patch of inflammation in association with the affected
vessels.
p 2604.

2664 TEXT-BOOK OF OPHTHALMOLOGY
(c) Toxic ExUDATIVE SYPHILITIC RETINITIs
We have already seen * that a toxic exudative retinitis, frequently with the
formation of a macular star (retinitis stellata), may occur in secondary syphilis : its
appearance and behaviour differ in no characteristic respect from similar lesions
associated with other conditions (Schieck, 1910; Onfray and Margerin, 1923; Junius,
1930; Fewell, 1932).
(d) CENTRAL SEROUs syph ILITIC RETINITIS
A very rare affection occurring in the secondary stage of syphilis and limited to
the macular region, of a fleeting nature but with a tendency to recurrences, was noted
by v. Graefe (1866) (Fig. 2265). It is usually bilateral and is associated with a central
Scotoma ; it may only last a few days but recurs again in a varying period lasting a
few weeks to a few months. Soon after the development of the Scotoma fine greyish-
white spots usually appear in the central area, and permanent damage to vision may
remain (Alexander, 1876–95; Uhthoff, 1912; Fuchs, 1921 ; and others). It is
probably due to a disturbance of the peri-foveal capillaries with subsequent Oedema,
and represents a type of central angiopathy. *
CENTRAL SYPHILITIC CHORIO-RETINITIS has already been noted.”
(e) CIRCUMSCRIBED GUMMATOUs LESIONS
Circumscribed gummatous lesions in the retina are rare. Most cases represent a
secondary infiltration from the choroid, the ciliary body (Brixa, 1899; Morton and
Parsons, 1902; Parsons, 1903), or the region of the lamina cribrosa, the lesion in the
last case taking the form of a chorio-retinitis juxta-papillaris “(Verhoeff, 1916; Köhne,
1920; Stoewer, 1924).
(f) NEURITIS PAPULOSA
Although mainly associated with syphilis of the optic nerve, it is
convenient to note a syndrome associated with a characteristic triad of
neuritis, peri-phlebitis and chorio-retinitis described by A. Fuchs (1926) as
NEURITIS PAPULOSA.
The condition was first described as a clinical entity by Fuchs (1926) in a study
of 4 cases, but several similar cases had appeared in the previous literature (Liebreich,
1863–syphilitic retinitis ; Scheidemann, 1895—gummatous new-growth at the disc with
marked peri-vasculitis; Gutmann, 1907; Mylius, 1913; Igersheimer, 1918—eacudative
papillo- retinitis); and others have appeared since (James, 1928; Satanowsky, 1932;
Rlien, 1938).
The disease occurs within the first two years after infection, and may be
either uni- or bilateral. The primary focus is a syphilitic infiltration of the
papilla resulting in the formation of massive exudation which protrudes as a
grey, yellow or white mass into the vitreous, terminating by anchoring itself
in a patch of choroiditis in the fundus. There is always an associated
chorio-retinitis with atrophic and pigmented patches, numerous small white
or yellowish spots in the fundus grouped especially near the vessels, a
considerable amount of retinal oedema with frequently a macular star, and
almost invariably retinal and frequently large vitreous haemorrhages
1 p. 2645 2 p. 2593. . 8 p. 2269. 4 p. 2269.
DISEASES OF THE RETINA 2665
(Fig. 2198). Histological examination shows a round-celled infiltration
in the posterior segment associated particularly with the retinal vessels,
where the arteries are encircled by a cuff of lymphatic elements and their
lumen is obstructed by proliferating endothelium. The disease runs a very
long and chronic course, even despite treatment, and the exudative mass
becomes organized into a profuse retinitis proliferans in which a constant
feature is a band of connective tissue stretching from the disc to the atrophic
patch of chorio-retinitis in the periphery (Fig. 2266).
The vision is usually reduced to perception of light during the early
stages owing largely to the repeated and profuse vitreous hæmorrhages,
Fig. 2266.-SYPHILITIC NEURITIs PAPULosa (James, Brit. J. O.).
but after a long and tedious course a considerable amount of sight may
return, although a scotoma varying from an enlarged blind spot to a com-
plete central defect may result. Blindness, however, may ensue; but it is
noteworthy that, probably owing to its fixation in the peripheral patch, the
retinitis proliferans does not usually cause a retinal detachment.
(g) syPHILITIC ARTERIoscLERosis
Unlike the aorta and coronary arteries, the retinal vessels show little
predilection for syphilitic sclerosis; but if hypertension is present, sclerotic
changes are marked although they show nothing distinctive of luetic infec-
tion, except, perhaps, a more patchy distribution than is usually found in
these lesions (Bridgett, 1926; Friedenwald, 1930).

26.66 TEXT-BOOK OF OPHTHALMOLOGY
(h) The manifestations of CONGENITAL SYPHILIS have already been noted under
congenital syphilitic chorio-retinitis. -
The treatment of syphilitic infections of the retina is essentially directed
towards general anti-syphilitic measures," but it is to be noted that, like
central nervous lesions, they frequently do not show a rapid response ;
indeed, in many cases the more old-fashioned mercury and iodide seems
sometimes to be more efficacious than arsenic or bismuth preparations.
Alexander. Berlin. kl. W., xiii, 508, 523, 1876.
Syphil. u. Auge, Wiesbaden, 87, 1889.
Neue Erfahrungen w. luetische Augenerkr.,
Wiesbaden, 24, 1895.
Appel. Diss., Würzburg, 1894.
Baas. A. f. O., xcv (3), 641, 1898.
Bach. A. f. Aug., xxviii, 67, 1894.
Blatt. A. f. O., civ, 198, 1921.
Bridgett. Am. J. O., ix, 725, 1926.
Brixa. A. f. O., xlviii (1), 123, 1899.
Edmunds and Brailey. R. L. O. H. Rep., x,
132, 1880.
Fewell. A. of O., viii, 615, 1932.
Förster. A. f. O., xx (1), 33, 1874.
Friedenwald. T. O. S., 1, 452, 1930.
Fuchs, A. Z. f. Aug., lix, 213, 1926.
Puchs, E. A. f. O., cvii, 15, 1921.
v. Graefe. A. f. O., xii (2), 114, 1866.
Gutmann. Cb. pr. Aug., xxxi, 207, 1907.
v. Hippel. A. f. O., czvii, 221, 1926.
Birschberg and Fehr. Cb. pr. Aug., xxx,
289, 1906.
Igersheimer. A. f. O., lxxxiv, 48, 1913.
Syphilis u. Auge, Berlin, 1918.
Klien. A. of O., xx, 427, 1938.
Knapp. T. Am. Acad. Oto-Lary.., xxv, 132,
I920.
Röhne. K. M. Aug., lxv, 882, 1920.
Lawford. T. O. S., xviii, 146, 1898.
Liebreich. Atlas, x, 1863.
Magnus. Oph. Atlas, 76, 1872.
Masselon. Mémoires d’Oph., Paris, 1883.
v. Michel. Z. f. Aug., xviii, 295, 1907.
Morton and Parsons. T. O. S., xxii, 266, 1902.
Mylius. K. M. Aug., li (1), 648, 1913.
Nettleship. R. L. O. H. Rep., xi, 1, 1886.
Onfrey et Margerin. A. d’O., x1, 635, 1923.
Parsons. R. L. O. H. Rep., xv, 292, 1903.
Rochon-Duvigneaud. A. d’O., xv, 764, 1895.
xxvi, 175, 1906.
Satanowsky. A. de Oft., B. A., vii, 279, 1932.
Scheidemann. A. f. O., xli (1), 156, 1895.
Schieck. K. M. Aug., xlviii (2), 150, 1910.
Spicer, Holmes. T. O. S., xii, 116, 1892.
Stein. A. f. O., lvi, 463, 1903.
Stoewer. Z. f. Avg., lii, 76, 1924.
Uhthoff. A. f. O., xxxix (1), 83, 1893.
R. M. Aug., i (1), 475, 1912.
Iwanoff. K. M. Aug., vi, 424, 1868. Verhoeff. A. of O., xlv., 352, 1916.
Jacobson. Königsberg med. Jhrb., i, 283, 1859. Wagenmann. A. f. O., xliv, 219, 1897.
James. Brit. J. O., xii, 31, 1928. O. Kl., iii, 21, 1899.
Junius. Z. f. Aug., lxx, 129, 1930.
2. TUBERCULOSIS
Tuberculosis of the retina is always secondary in the sense that it is
haematogenously derived from a lesion elsewhere in the body ; but its lodg-
ment in the retina as the primary site in the eye is rare, and, indeed, if we
discount the relatively common condition of tuberculous peri-phlebitis, is
exceptional. Its spread to the retina secondarily from other ocular tissues
is, however, common, the usual sources being the choroid 4 (in which case
a chorio-retinitis is the rule), the ciliary body * and the optic nerve.”
This rarity of primary retinal localization is borne out by experimental inoculation.
Thus in his experiments wherein rabbits were injected intravenously with tubercle
bacilli, Stock (1903–07) produced irido-cyclitis and choroiditis regularly but never
retinitis, while Finnoff (1924), by intra-arterial injection, produced it twice in 46
animals. Even if the tubercle bacilli are injected into the eye the retina remains
astonishingly free from invasion, and when affected, the extension is from the choroid
in the vast majority of cases (Ohmart, 1933).
1 p. 2282. * p. 2284. 8 p. 2298. 4 p. 3037.
DISEASES OF THE RETINA 2667
(a) MILIARY TUBERCLE of THE RETINA
In generalized miliary tuberculosis the appearance of tubercles, although common
in the choroid, is exceptional in the retina itself. Ophthalmoscopically their location
may be difficult to establish, and very few histological examinations have been reported
(Bouchut, 1868–69; Litten, 1902).
(b) MAssive TUBERCLE of THE RETINA
A conglomerate tubercle starting in the retina and leaving the choroid
unaffected is very rare indeed ; the few lesions of this type have all occurred
in the macular region. A number of cases have been reported clinically
wherein the main diagnostic proof has been the therapeutic response to
tuberculin, but few have received pathological verification. Such a tumour
was reported by O'Sullivan and Story (1899) in a girl of 21 years, who
Fig. 2268.-TUBERCLE of THE RETINA.
Part of the retina of Fig. 2267 under high power showing giant cells and small-
celled infiltration (Hancock, R. L. O. H. Rep.).

26.68 TEXT-BOOK OF OPHTHALMOLOGY
showed ophthalmoscopically an intense papillitis and some white spots at
the macula which developed into a large tumour composed of typical
tuberculous systems; the pigment epithelium, choroid and optic nerve were
normal. Similar cases were described by Hancock (1905) in a man of 18
and Welter and Blum (1926) in a man of 36 (Figs. 2267–68).
(c) ExUDATIVE TUBERCULOUS RETINITIS
Cases of exudative retinitis, due presumably to tuberculo-toxin rather
than to the actual bacilli, are also rare. They are characterized by a
hyperaemia and venous dilatation in the retina and the deposition of
numerous whitish or yellow spots scattered over the fundus, sometimes
associated with haemorrhages (Knapp, 1913); a number of such cases have
been reported from Japan (Hayashi, 1918; Yamanaka, 1925; Suzuki,
1927; Shoji, 1930–31). To some extent they are comparable with Coats’
exudative retinitis.
Other cases have been reported of a more diffuse oedematous nature,
occasionally exhibiting a macular star (retinitis Stellata) (Kaminskij, 1929;
Rleiber, 1930).
(d) TUBERCULOUS PERI-PHLEBITIS
TUBERCULOUS PERI-PHLEBITIS as a clinical entity was first described
by Perls (1873), but owes its establishment to the work of Axenfeld and
Stock (1911). It is a relatively common disease occurring typically in
young adults, characterized by a chronic recurrent course, and making itself
evident clinically most typically by repeated attacks of haemorrhages into
the retina or the vitreous (EALES’ DISEASE * : ANGIOPATHIA RETINAE
JUVENILIs of CORDs, 1921). Tubercle, indeed, is almost certainly the
commonest (although not the only) cause of retinal peri-phlebitis, and its
aetiology has been definitely proved by the demonstration of tubercle bacilli
in the immediate neighbourhood of an affected vein (Gilbert, 1935).
AEtiologically it may be of two types: a direct spread of tuberculous
material from the ciliary body in cases of cyclitis, or the lodgment of
metastatic deposits in the retinal circulation. With regard to the first
route, a considerable number of cases have been reported wherein the
retinal condition has been preceded by, and was associated with, an active
tuberculosis in the anterior uvea (Axenfeld and Stock, 1911 ; Fleischer,
1914; Mayrhofer, 1927; Lluesma, 1929; Goldstein and Wexler, 1930;
Finnoff, 1931; v. Hippel, 1935; Kokott, 1935; and others). The patho-
logical spread of true tubercles composed of small round cells and epithelioid
cells which had developed in the inner vascular layer of the ciliary body and
had migrated into the posterior chamber and through the vitreous to the
retina, has already been fully described (Verhoeff. 1910–29 ; Meller, 1921 ;
Finnoff, 1924–31 ; v. Szily, 1931),” while its experimental demonstration
1 p. 2602. * p. 2299.
DISEASES OF THE RETINA 2669
has been provided by Straub (1912) and Ohmart (1933). The freedom,
however, of the anterior uvea from inflammation in the majority of cases
(Axenfeld, 1927), the widespread peripheral distribution of the lesions in
the terminal circulation of the retina in many of them, as well as the
demonstration of active tubercle elsewhere in the body (Axenfeld and
Stock, 1911; Igersheimer, 1912; Werner, 1913; Fleischer, 1914; Gilbert,
1914-35; Mayrhofer, 1927; Goldstein and Wexler, 1930; Löwenstein,
Fig. 2269.-TUBERCULous RETINAL PERI-PHLEBITIs (v. Hippel, A. f. O.).
1931; Flint, 1934; Kokott, 1935; Bonnet, Paufique and Sarragin, 1936),
or in the other eye (Clapp, 1930), and of a positive cutaneous reaction
to tuberculo-protein (Cords, 1911; Davis, 1920; Nicolato, 1927; Natale,
1932; and others)—all these points provide strong evidence of its
haematogenous origin, a possibility proved experimentally by Otori (1915),
Finnoff (1924) and Finnoff and Reynolds (1933). In these cases Axenfeld
(1920) believed that the bacilliemerged from the retinal capillaries and affected
the retinal veins directly by penetrating the peri-venous sheaths. In many
cases, on the other hand, it is difficult to be sure by which path the infection

2670 TEXT-BOOK OF OPHTHALMOLOGY
has arrived, such as those cases of miliary tuberculosis with lesions in the
anterior segment of the uvea and also in the retina (Perls, 1873; Goldstein
and Wexler, 1930).
The pathology has been investigated by several observers (Fleischer, 1914;
Axenfeld, 1920; Wolf, 1921 ; Meller, 1922; Goldstein and Wexler, 1930; Gilbert,
1935; v. Hippel, 1935); the general appearances are those of a peri-vascular infiltra-
tion of round cells, epithelioid cells, giant cells and lymphocytes which progress as
we have already discussed in dealing with peri-vasculitis generally (Fig. 2269).
Clinically the disease usually appears suddenly with a profusion of
retinal haemorrhages (Fig. 2243, Plate L), and frequently a vitreous
haemorrhage which obscures the picture. In severe cases the clinical picture
discussed under the heading of peri-vasculitis is duplicated, ranging in all
degrees of severity up to that typical of venous thrombosis. The process is
commonly of widespread and peripheral distribution, and the maculais usually
Fig. 2270-Tuberculous Choato-RETINºrts.
Following the course of the retinal vessels (Brown, Brit. J. O.).
spared, but it may start in the main veins (Davis, 1920; Gilbert, 1935) or
on the disc, producing the clinical picture of acute exudative optic neuritis
(Cords, 1921). In less severe cases the disturbance may be more localized,
confined to a few haemorrhages, with sometimes the appearance of a more
widespread exudative disturbance taking the form of a macular star (stellate
retinitis.)” It is frequently bilateral, one eye, preferentially the left, being
p. 2639. * p. 2644.

DISEASES OF THE RETINA 267.1
usually more advanced than the other, so that the combination arises of a
fundus-obscuring haemorrhage in one eye, and small petechial haemorrhages
with some white sheathing of the arteries in the other (Axenfeld and Stock,
1911; Igersheimer 1912; Suganuma, 1927; Kokott, 1935). At other times
the changes may be so small as to clear up within a few weeks (Axenfeld
and Stock, 1911), leaving, perhaps, definite areas of peri-phlebitis (Bonnet,
Fig. 2271.-TUBERCULouis ARTERITIs.
Occurring as a reaction to tuberculin (Muncaster and Allen, A. of O.).
Paufique and Sarragin, 1936). In all cases, however, the habit of the
disease, like most tuberculous manifestations, is to recur intermittently,
so that no sooner is one haemorrhage tending to clear up after a slow and
tedious course than another occurs, a sequence which may be repeated for
many years despite the most well-intentioned and thorough treatment. In
such cases the process may suddenly cease, apparently for no particular
reason, and even when recurrences have taken place over a period of years
and an advanced degree of retinitis proliferans has developed, good vision
may still be retained (Paton, 1938); in others, however, a secondary
T.O.-WOL. III. P. P.

2672 TEXT-BOOK OF OPHTHALMOLOGY
glaucoma supervenes (Werner, 1913; Safar, 1928), or there may be a
profuse formation of new vessels in the vitreous (Flint, 1934), or a proli-
ferating retinitis 1 destroys the vision by bringing on a retinal detachment
(Schall, 1922; Cervellati, 1927), no small catastrophe when the disease is
bilateral.
An anomalous picture described by Brown (1937) and called by him RETINO-
CHOROIDITIs RADIATA seems probably to result from tuberculous peri-phlebitis (Fig.
2270). His patient showed heavily pigmented chorio-retinal lesions in the atrophic
stage which radiated out from the disc, following closely the distribution of the retinal
veins. The most plausible explanation would appear to be that the peri-phlebitic
inflammation had developed in an unusual way and spread outwards involving the
choroid rather than, as it usually does, confining itself to the inner retinal layers and
tending to spread towards the vitreous.
(e) TUBERCULOUS PERI-ARTERITIS
A primary tuberculous peri-arteritis is a much rarer phenomenon than a peri-
phlebitis. A few cases have been described which appeared not to have been derived
from disease of a neighbouring vein (Derby, 1921; Kyrieleis, 1933); and one very
interesting case in which a bilateral peri-arteritis associated with a generalized
uveitis of some considerable severity occurred as a focal reaction to a tuberculin test
(Fig. 2271) (Muncaster and Allen, 1939).
Axenfeld. B. O. G. Heidel., xlii, 298, 1920. Kyrieleis. A. f. Aug., cvii, 182, 1933.
K. M. Aug., lxxix, 66, 1927. Litten. Deut. med. W., xxviii, 37, 1902.
Axenfeld and Stock. K. M. Aug., xlix (1), 28, Lluesma. Med. ibera, ii, 537, 1929.
1911. Löwenstein. Münch. Med. W., lxxviii, 261,
Bonnet, Paufique and Sarragin. A. d’O., liii, 1931.
849, 1936. Mayrhofer. Wien. kl. W., xl (2), 1199, 1927.
Bouchut. Gaz. de Hôp., de Paris, xli., 601, Meller. A. f. O., cv, 299, 1921.
1868; xlii, 2, 1869. Z. f. Aug., xlvii, 247, 1922.
Brown. Brit. J. O., xxi, 645, 1937. Muncaster and Allen. A. of O., xxi, 509, 1939.
Cervellati. Am... dº Ott., lv., 197, 1927. Natale. Rev. As. Med. Argent., xlvi, 1055,
Clapp. Am. J. O., xiii, 295, 1930. 1932.
Cords. Z. f. Aug., xxvi, 441, 1911. Nicolato. Boll. d’Oc., vi, 735, 1927.
A. f. O., cv, 916, 1921. Ohmart. Am. J. O., xvi, 773, 1933.
Davis. T. Am. O. S., xviii, 59, 1920. O'Sullivan and Story. T. Acad. Med. Ireland,
Derby. T. Am. O. S., xix, 238, 1921. xvii, 451, 1899.
Finnoff. T. Am. O. S., xix, 238, 1921. Otori. A. f. Aug., lxxix, 44, 1915.
A. of O., liii, 130, 1924. Paton. A. of O., xx, 276, 1938.
Am. J. O., vii, 81, 1924; xiv, 127, 1208, Perls. A. f. O., xix, (1), 221, 1873.
1931. Safar. A. f. O., czix, 624, 1928.
Finnoff and Reynolds. T. Am. Acad. Oto- Schall. A. f. O., eiz, 205, 1922.
Lary. xxxviii, 152, 1933. Shoji. K. M. Aug., lxxxv, 161, 1930.
Fleischer. K. M. Aug., lii, 769, 1914. A. d’O., xlviii, 241, 1931.
Flint. Brit. J. O., xviii, 27, 1934. Stock. K. M. Aug., xli (1), 81, 1903.
Frank. Z. f. Aug., lvii, 301, 1925. A. f. O., lxvi, 1, 1907.
Gilbert. A. f. Aug., lxxv, 1, 1913. Straub. T. O. S., xxxii, 60, 1912.
Münch. med. W., lxi, 306, 1914. Suganuma. A. f. O., czviii, 443, 1927.
R. M. Aug., xciv, 335, 1935. Suzuki. Nipon Gank., Zass. xxxi, 767, 1927.
Goldstein and Wexler. A. of O., iii, 552, 1930. v. Szily. K. M. Aug., lxxxvii, 13, 1931.
Hancock. R. L. O. H. Rep., xvi, 150, 1905. Velter and Blum. A. d’O., xliii, 141, 1926.
Hayashi. Gamka-Rimsho, xiii, 378, 1918. Verhoeff. T. Am. O. S., xiii, 366, 1910.
v. Hippel. A. f. O., czzxiv, 121, 1935. J. Am. Med. As., lxiii, 13, 1914.
Igersheimer. A. f. O., lxxxii (2), 215, 1912. A. of O., i, 63, 1929.
Kaminskij. Russ. O. J., ix, 742, 1929. Werner. T. O. S., xxxiii, 9, 1913.
Kleiber. Z. f. Aug., lxx, 187, 1930. Wolf. A. f. Aug., lxxxix, 54, 1921.
Knapp. T. Am. O. S., xiii, 486, 1913. Yamanaka. Nipon Gank. Zass... xxix, 1187,
Kokott. K. M. Aug., xciv, 327, 1935. 1925.
1 p. 2604.
DISEASES OF THE RETINA 2673
3. LEPROSY
The changes in the retina in leprosy are almost always secondary to those in the
uveal tract ; * Small nodules are found, especially in the anterior parts of the
fundus (ANTERIOR LEPROTIC RETINITIS, Trantas, 1899 ; Valettas, 1916), or occasionally
larger chorio-retinal lesions. In some of these cases the retinal tissues may undergo
extensive inflammatory changes, the lesions showing the presence of large numbers of
the bacilli (Philippson, 1893; Doutrelepont and Wolters, 1896; Franke and Delbanco,
1900; Prendergast, 1940).
Doutrelepont and Wolters. A. Derm. Syph., Philippson. Bett. 2. Aug., xi, 31, 1893.
xxxiv, 55, 1896. Prendergast. A. of O., xxiii, 112, 1940.
Franke and Delbanco. A. f. O., l (2), 380, Trantas. Bull. S. fr. d’O., xvii, 275, 1899.
1900. Valettas. K. M. Aug., lvi, 472, 1916.
4. ACTINOMYCOSIS
L. Müller (1903) described bilateral retinal lesions in actinomycosis, consisting of
small yellowish-white spots resembling miliary tubercles. Histologically they were
composed of mononuclear and epithelioid cells, but no organisms were found.
Müller. K. M. Aug., xli (1), 236, 1903.
5. ACUTE FEBRILE DISEASES
A large number, if not the majority, of acute infective diseases may have retinal
complications, usually of an exudative or haemorrhagic nature, resulting from injury
to the retinal capillaries from the infective agent or its toxins.
Thus in INFLUENZA retinal haemorrhages may occur (Fraenkel, 1920), or an
oadematous or exudative retinitis with a star-figure at the macula (retinitis stellata)
(Hartridge, 1893; Maklakoff, 1909; Knapp, 1916; Hessberg, 1920; Danco, 1921 ;
Nadeldinger, 1927; Bakker, 1928).
TYPHOID FEVER may be associated with retinal haemorrhages (Layson, 1914).
TYPHUs FEVER may be associated with more profound disturbances involving
much haemorrhage and endothelial proliferation so that extensive thrombosis may
result (Gutmann, 1916; Nauwerck, 1916; Jendralski, 1922; Braunstein, 1924).
MEASLEs may be associated with the picture of retinitis stellata (Stewens, 1930).
RECURRENT FEVER may be accompanied by retinal haemorrhages and an exudative
retinitis (Trantas, 1920).
MALARIA, as we have seen * is frequently accompanied by retinal haemorrhages
(Werner, 1911; Agricola, 1917; Connell, 1922; Harston, 1922; Kiep, 1922; Pereyra,
1922; and others): they may lead to a retinitis proliferans. These are doubtless due
to the presence of parasites, sometimes in enormous quantities, in the retinal veins
(Dudgeon, 1921), and they are sometimes followed by widespread thrombosis or a
diffuse retinitis which may be transient but may leave permanent defects
(Smith, 1904; Bywater, 1922; Marin Amat, 1923).
In SPIROCHAETOSIS ICTERO-HAEMORRHAGICA (Weil's disease) hamorrhages are not
unusual (Hertel, 1917; Moret, 1919), as well as chorio-retinitis (Schevensteen, 1917).
In PLAGUE an exudative retinitis leading to detachment may occur (Joudine ,
1915).
In GONORRHCEA, in addition to the relatively common occurrence of central
retinitis as a complication of irido-cyclitis, an infective thrombosis of the central vein
has been observed (Valude, 1911).
1 p. 2320. 2 p. 2597.
2674
TEXT-BOOK OF OPHTHALMOLOGY
Agricola. K. M. Aug., viii. 421, 1917.
Amat. A. de Oft., H.-4. Xxiii. 633, 1923.
Bakker. Ned. Tij. v. Gen., lxviii. 556, 1928.
Braunstein. A. f. O., exiii. 359, 1924.
Bywater. T. O. S., xiii. 359, 1922.
Connell. J. Trop. Med., xxv, 378, 1922.
Cross. T. O. S., xiii, 79, 1893.
Danco. K. M. Aug., lxvii. 87, 1921.
Dudgeon. T. O. S., xli. 236, 1921.
Fraenkel. D. med, W., xlvi, 673, 1920.
Gutmann. D. med. W., xlii, 1538, 1916.
Harston. T. O. S., xlii, 233, 1922.
Hartridge. T. O. S., xiii, 77, 1893.
Hertel. A. f. O., xciv, 28, 1917.
Hessberg. Munch. med. W., lxvii. 207, 1920.
Jendralski. K. M. Aug., lxviii. 832, 1922.
Kiep. T. O. S., xlii, 394, 1922.
Knapp. A. of 0., xlv. 247, 1916.
Layson. O. Rec. xxiii, 351, 1914.
Maklakoff. K. M. Aug., xlvii (1), 165, 1909.
Moret. La Clin. Opht., viii, 19, 1919.
Nadeldinger. Bull. S. d’O. Paris, xxxix, 311,
1927.
Nauwerck. Med. gesellsch, zu Chemnitz, 1916.
Pereyra. A. dº Ott., xxix, 49, 1922.
Schevensteen. An d'Oc., cliv, 728, 1917.
Smith. T. O. S., xxiv, 135, 1904.
Stewens, K. M. Aug., lxxxiv, 714, 1930.
Trantas. A. d’O., xxxvii, 193, 1920.
Valude. An d'Oc., exlvi. 426, 1911.
Werner. A. f. Schiffs- u. Tropen-Hyg., xv,
431, 1911.
Joudine. Westm. Oph., xxxii, 21, 1915.
6. DERMAto-Myosºtis
This rare disease of unknown toxic aetiology, first reported by Wagner (1863)
and named by Unverricht (1887), is characterized by a gradual onset with vague and
indefinite systemic prodromes, followed by oadema, dermatitis, and multiple muscle
Fig. 2272.-RETINITIs IN DERMAto-Myosºtis.
(Bruce, T. Am. O. S.)
inflammations. It may last a few weeks or several years, frequently with exacerbations
and remissions, but usually terminates fatally with broncho-pneumonia. On rare
occasions pareses of the external eye muscles have been noted (Oppenheim, 1923),
nystagmus (Wagner, 1863; Marinescu et alia, 1931), exophthalmos (Heyn, 1924), as
well as conjunctivitis and iritis (Oppenheim, 1923): 3 cases of retinitis have been
recorded by Bruce (1938) in one of which pathological examination was possible.

DISEASES OF THE RETINA 2675
The retinal picture (Fig. 2272) showed distended veins, but normal arteries and a
normal disc. Around the posterior pole, however, and involving the macula were
extensive superficial exudative deposits associated with round and flame-shaped
haemorrhages. The lesions increased rapidly in size and number and become confluent,
until after a few weeks they gradually resolved or the disease proved fatal.
Pathologically the retina showed extensive oedematous changes, particularly
in the inner layers where varicose nerve-fibres were obvious. In the outer layers,
especially the outer plexiform layer, were albuminous deposits and multiple
haemorrhages.
Bruce. T. Am. O. S., xxxvi, 282, 1938. Oppenheim. Lhb. d. Nervenkr., Berlin, VII,
Heyn. J. Am. Med. As., lxxxii, 1019, i, 840, 1923.
1924. Unverricht. Corresp.-Bl. allg. ārztl. Ver. v.
Marinescu et alia. An. de Méd., xxx, 145, Thiringen, xvi, 207, 1887.
1931. Wagner. A. f. Heilk., iv, 282, 1863.
IV. RETINOPATHIES ASSOCIATED WITH GENERAL DISEASES
It is the usual and generally accepted practice that the retinal manifestations of
general metabolic or cardio-vascular disease be classed under the heading retinitis.
The term is unfortunate, for although in some conditions there is undoubtedly an
inflammatory element present, the essential changes are degenerative in nature. It
seems more appropriate, therefore, instead of using terms which connote an inflam-
matory process, as arteriosclerotic, renal, diabetic retinitis, to use the more accurate
and less misleading term arteriosclerotic retinopathy, and so on.
1. Vascular Sclerosis and Retinopathies
A discussion of retinal angiopathies is one of unusual importance,
interest and difficulty. Of importance, because of the unique opportunities
offered in the eye of observing under high magnification the intimate changes
in the vascular tree down to its finest ramifications, and deducing therefrom,
with reservations, the general condition of the key-system of the body :
and the importance is augmented by the close relationship, which we shall
discuss presently, which exists between the retinal, the cerebral and the
renal vessels, for the eye can serve as a window through which the vessels of
the brain may be studied, and the retina, again with reservations, as an index
of the state of the parenchyma of the kidney. Of interest, because many of
the most fascinating and complicated problems in medicine are bound up in
the derangements of the cardio-vascular system, problems which are
undoubtedly increasing in number and importance with the accelerated
tempo of modern life. Of difficulty, because to-day, after a century of
research and study, we are still far from possessing the plan of the maze
into which the search for the ultimate causes of these extremely common
problems has led us. This is due to several reasons—the difficulty of
obtaining microscopical material in the early stages of disease and of corre-
2676 TEXT-BOOK OF OPHTHALMOLOGY
lating it accurately with ophthalmoscopic pictures, for most of our patho-
logical knowledge is limited to the ruins amongst which the most important
and significant changes have vanished ; the misfortune that histological
technique does not demonstrate many changes which are optical or chemical
rather than structural and organic ; and the fact that our knowledge is
patchy and made up of isolated pictures rather than correlated
and consecutive, a limitation due to the absence of evolutionary
observations on individual cases over a period of many years so that the
separate pictures which we see can be envisaged as successive steps in an
uninterrupted pathological sequence. Throughout the last century the
opinions of one generation have been upset and reversed by those of the
next, and to-day the views of modern authorities are frequently so much at
variance, although severally expressed with almost religious dogmatism, that
to compromise between them is almost impossible. Nevertheless a detailed
study of the ocular aspects of the problem is necessary, not only because of
its bearing on the eye and vision, but also because of its wider implications
in the medical assessment of the patient, in the ordering of his habits and
activities, and in prognosticating his fate.
The history of vascular diseases of the retina may be said to date from the time
when Bright (1836) first recognized the association of visual disturbances with renal
disease, but its study had to wait until, in 1851, v. Helmholtz presented the means to
undertake it in the ophthalmoscope. Almost immediately thereafter the interest of
the subject produced a host of clinical observations (v. Graefe, 1855; Liebreich, 1859;
Hulke, 1866; and others) and histological studies (Virchow, 1856; Müller, 1857–60;
Schweigger, 1860; Wedl, 1861; Iwanoff, 1865), so that Mauthner (1868), in the first
classical work on the subject, was able to describe with considerable accuracy a large
number of the typical phenomena. Thereafter the mile-stones in research were the
demonstration by Hirschberg (1882) of the occurrence of atheroma in the eye, and the
establishment of the fact by Raehlmann (1888–89) that ocular sclerosis was not a
local affection but part of a general disease, and his accurate description of its clinical
signs in the eye. Even at that time, however, knowledge had been fragmentary and
general interest lagged, until the appearance of the work of Marcus Gunn (1898),
wherein the entire subject of vascular sclerosis in the retina was studied with a degree
of accurate detail and penetrating care rarely equalled in any subject in medical
literature; his papers, which were of greatly enhanced importance since they coincided
with the introduction of the sphygmomanometer by Riva-Rocci in 1896, excited
universal interest, both amongst ophthalmologists and in the wider field of general
medicine, which has never slackened.
So complete and thorough were Gunn’s observations that few fundamental
advances in the field of clinical observation have been possible, but details of the
greatest interest and importance have been brought to light (Friedenwald, 1896–
1930 ; de Schweinitz, 1900–07 ; Foster Moore, 1916–24 ; Bailliart, 1923; Kahler and
Sallmann, 1925–33 ; Pines, 1927–29 ; Guist, 1931 : Salus, 1931–35 ; Horniker, 1936 ;
Ballantyne, 1937; and others). The main interest in the subject now lies in the
determination of cause and effect, in differentiating the types and assessing the
significance of the various manifestations of sclerosis and the part played by the all-
important factor of hypertension, and in unravelling the manifold and obscure aetiological
factors. The essential stimulus to this work depended upon the separation of the many
DISEASES OF THE RETINA 2677
syndromes which had hitherto been called nephritis and the recognition of the association
of some of them with general arterial disease by Löhlein (1907), Volhard (1921–31),
Schieck (1921–29), Fahr (1925), and others, and upon the elucidation of the effects of
hypertension upon the eye, both as a separate entity and in association with renal
disease, particularly by Wagener and his co-workers (1924–30), and Fishberg (1934).
Retinal Manifestations of Vascular Disease
The most important ophthalmoscopic evidences of sclerosis of the
retinal vessels can be conveniently summarized as follows:–
1. TRANSLUCENCY OF THE WESSEL WALLS
In the normal eye the translucency of the vessel walls is so nearly
perfect that they are transparent and invisible, but in many diseased states
this property disappears. In acute toxaemias, for example, it may disappear
within a few hours (Bailliart, 1923); and this sign, as was first noted by
Gunn (1898), forms a constant and early feature of vascular sclerosis and
hypertension. It is most easily assessed by the ability to see the veins
through the arteries where they are crossed by them, which, although not
possible in the days of the reflecting ophthalmoscope (Gunn, 1898), is normal
in the greater intensity of the illumination of the electric ophthalmoscope
(Fig. 2301).
2. THE WASCULAR REFLEX
The vascular reflex, which appears as a thin longitudinal bright streak
running along the convexity of the artery, is due to light reflected partly by
Fig. 2273.-RETINAL ARTERIoscLERosis.
Silver-wire arteries exudative spots and haemorrhages (Parsons).

2678 TEXT-BOOK OF OPHTHALMOLOGY
the blood-column (Dimmer, 1891) and partly by the vessel walls in which
the main reflecting element is the media (Wilmer and others, 1933): in
sclerotic conditions it is, of course, the latter element which alters. Since
the reflex is actually the optical image of the illuminated portion of the
patient's pupil formed by reflection from the vessel wall, it necessarily varies
with the type and method of illumination employed; moreover, since
Fig. 2274.-RETINAL ARTERIoscLERosis.
In renal retinopathy. A copper-wire artery showing hyaline degeneration of
the media (Friedenwald, T. O. S.).
alterations in its appearance are merely exaggerations of normal phenomena,
the interpretation of slight changes must be received with caution. It may
be said, however, that in sclerotic conditions the usual uninterrupted bright
streak may suffer three significant changes.
(a) CoPPER wire ARTERLEs. As was first adequately pointed out by
Gunn (1898), an early sign of sclerosis is an increase in the brightness of the
reflex of the retinal arteries so that instead of their normal rosy appearance
with a thin central bright line, they assume a burnished metallic appearance

DISEASES OF THE RETINA 2679
with a broader and softer reflex resembling copper wire (Fig. 2316, Plate LI).
The change in colour is most readily seen in the larger arteries of second and
third order, and is particularly evident in diffuse hyperplastic sclerosis
(Fig. 2301). Pathologically it is coincident with the commencement of
thickening and hyaline degeneration in the media (Coats, 1913; Friedenwald,
1930) (Fig. 2274).
º º
º
º
*** *-*.
º º ---
*: º fºr:
-
-: - --
--- º --- -
º * -
Fig. 2275. RETINAL ARTERIoscLERosis.
In renal retinopathy. A silver wire artery showing extreme hyaline degeneration
of the media involving obliteration of the lumen (Friedenwald, T. O. S.).
(b) SILVER wire. ARTERIES. In more advanced cases, as a result of the
still higher refracting power of the arterial wall, the entire vessel reflects light
homogeneously so that the whole thickness of the artery appears as a bright
white reflex like a wire of silver without any evidence of the red blood-
column (Fig. 2273). In his original description of the phenomenon Gunn
(1898) pointed out that it was usually associated with hypertension, and
suggested that it was due to hyaline degeneration of the vessel wall, a
suggestion verified anatomically by Coats (1913) and Friedenwald (1930)
(Fig. 2275). While this is undoubtedly so, there are others who think that












2680 TEXT-BOOK OF OPHTHALMOLOGY
-
this appearance could be produced by simple spasm without actual histo-
logical change (Gowers, 1904; Leber, 1915; Pines, 1929).
(c) IRREGULARITIES IN THE REFLEX, so that it shows dots or beading
of increased brightness, a sign first described by Foster Moore (1916), is a
very delicate sign and is always pathological. Its origin is disputed, but it
is probably due to various causes, being associated with localized histological
changes in the vessel walls, with irregularities in the lumen, or with areas of
localized spasm.
3. SHEATHING OF THE WEssels
This phenomenon, wherein white lines first appear running alongside
the walls of the vessels (PARALLEL sheathing) and wherein, in its most
Fig. 2276–RETINAL ARTERiosclerosis.
Perivascular changes at disc with sheathing of the arteries near the vascular
crossings: compression and deflection of the veins (Pines, Brit. J. O.).
extreme degrees, the entire vessel is converted into a white fibrous-looking
cord (PIPE stEM shºatHING), was again first adequately described by Marcus
Gunn (1898) (Fig. 2279). It is important to remember that such an appear-
ance frequently occurs in normal and youthful eyes on the disc and on the
retina in its immediate neighbourhood. In conditions of sclerosis the
ensheathing white lines appear first at the arterio-venous crossings where the
sheaths of the arteries and veins reinforce each other (Figs. 22.76 and 2290),
or alternatively when the same reinforcement is accomplished by an artery
and vein running in parallel (Fig. 2277); but in more advanced conditions

DISEASES OF THE RETINA 2681
Fig. 2277. RETINAL Fig. 2278.-RETINAL
ARTERIOsCLEROSIs. ARTERIOsCLEROSIs.
Marked local sheathing of the Extensive sheathing of the
inferior artery at the venous arteries (Pines, Brit. J. O.).
crossings: marked compression
of veins without appreciable de-
flection, and (above) complete
collapse of the vein (Pines, Brit.
J. O.),
Fig. 2279.-RETINAL ARTERIOsCLEROSIs.
Corkscrew veins: macular exudates: extensive sheathing silver wire
arteries.


2682 TEXT-BOOK OF OPHTHALMOLOGY
all the vessels may be uniformly ensheathed—a serious pathological feature
(Fig. 2278).
The cause of this appearance is a matter of dispute, and it seems probable,
as in many of these phenomena, that several factors may be concerned,
some of which are preponderant in various cases. In all cases, however,
the effect is due to a greater optical density of the vessel wall so that it
reflects sufficient light to be demonstrated by the ordinary ophthalmoscope,
at first at the sides where the whole lateral thickness of the vessel wall is
concerned (parallel sheathing), and eventually over the anterior surface
as well so that the entire artery becomes opaque (pipe-stem sheathing).
It seems probable that the increased optical density may be due on occasion
Fig. 22.80.-RETINAL ARTERIoscLERosis.
A retinal artery at a point where it showed parallel sheathing (Ballantyne,
Michaelson and Heggie, T. O. S.).
to non-organic, spastic phenomena, for it has been observed to disappear
(Foster Moore, 1925); but in most cases it denotes organic change. It is
obvious that it will be immaterial which part of the wall becomes more
optically dense, and there is evidence that the phenomenon may be due to
changes either in the perivascular adventitial tissue or in the sub-endothelial
tissue.
The phenomenon has generally been described as a fibrotic peri-vasculitis
of a reactive and compensatory nature strengthening the vessel against undue
strain. It will be remembered that the retinal vessels are surrounded by a
complicated system of neuroglial sheathing insulating the mesodermal
vascular elements from the neural ectoderm, and it seems probable that the
appearance of sheathing is sometimes caused by sclerosis of this perivascular

DISEASES OF THE RETINA 2683
tissue so that it is rendered visible. It seems necessary to suppose that the
congenital sheathing seen at and near the disc is due to a physiological
increase in this adventitial tissue, and it has been shown pathologically that
it may be considerably increased in sclerotic states (Friedenwald, 1930;
Sallmann, 1937–38) (Fig. 2295).
On the other hand, the histological examination of such an artery has
demonstrated conclusively that the same appearances may be due to an
atherosclerotic sub-endothelial degeneration without any perivascular fibrosis.
Ballantyne, Michaelson and Heggie (1938) have shown that the process can
be traced in three stages. First a thickening of the wall occurs due to a
cellular proliferation of the sub-endothelial tissue, which, since it is trans-
Fig. 2281.-RETINAL ARTERIoscLERosis.
A retinal artery at a point where it showed pipe-stem sheathing, showing com-
plete degeneration of the thickened intima (Ballantyne, Michaelson and Heggie,
T. O. S.).
parent, gives rise to the appearance of a narrowed blood-column only. In the
second stage, this new-formed tissue loses its nuclei and becomes amorphous,
finally disintegrating completely into fatty debris: this tissue being opaque,
it appears, if in small amount, as parallel sheathing (Fig. 2280), and if in
sufficiently great amount, as pipe-stem sheathing (Fig. 2281). It is probable
that in diffuse hyperplastic sclerosis a similar change in the media may
produce a similar optical effect.
4. ARTERIO-VENOUS CROSSINGS
We have already noted two phenomena most easily seen at the points
where the arteries and veins cross—the increased translucency of the artery

2684 TEXT-BOOK OF OPHTHALMOLOGY
as its walls become thickened, hiding the underlying vein from view, and the
appearance of perivascular fibrosis. Two other appearances are noteworthy :
constriction, apparent or real, of the veins, and their deflection.
(a) Construction, APPARENT or REAL, of THE WEINs was first
described by Gunn (1892), who pointed out that “when an artery, even a
small twig, passes over a retinal vein . . . the vein is indistinguishable
Fig. 2282.-RETINAL ARTERIoscLERosis.
Compression and displacement of the veins: exudative spots at the macula
(Pines, Brit. J. O.).
just at the spot where it is crossed, and is eventually distended some distance
peripherally from this point" (GUNN's sign) (Fig. 2282). Gunn (1898–1904)
interpreted the phenomenon as an actual compression of the vein, but it is
evident that several different sets of circumstances may be at work in
different cases.
(i) Concealment of the vein is the most simple appearance, when the
vessel appears sharply cut across by the artery, separated from it by a clear

DISEASES OF THE RETINA
2685
interval due presumably to the thickness of the translucent arterial wall
(Fig. 2283).
(ii) At other times, again without evidences of compression or deflection,
Figs. 2283–88. ARTERIo-venous CRossINGs.
Fig. 2283.-Simple con-
cealment of the vein.
Fig. 2284–Tapering of
vein at crossing.
Fig. 2285.-Depression
of the vein.
Fig. 2286.-Banking Fig. 2287.-Vein cros- Fig. 2288.- : Hump
of vein before crossing sing over artery showing bridge" crossing over
(Ballantyne, T. O. S.). constriction. artery without change of
calibre.
the vein may be pale or invisible not only at the actual point of crossing but
for some distance on either side, the ends frequently tapering to a point as
they approach the crossing (Fig.
2284). It is probable that in these
cases the process of sclerosis in
the arterial wall has spread to the
venous wall rendering it also
opaque.
(iii) Depression of the vein into
the retina is suggested in other
cases where the vein becomes
darkly shaded for some consider-
able distance on each side of the
artery, so that a layer of tissue lies
above it, an appearance which can
occur without any evidence of
compression or hindrance to the
blood-flow (Fig. 2285). The occur-
rence of such a phenomenon was
stressed by Doyne (1904) and Frost
Fig. 2289.-RETINAL ARTERIoscLERosis.
Arterio-venous crossing showing Salus' sign
(Koyanagi).
(1904), and later by Salus (1929) and Ballantyne (1937); and it has been
anatomically demonstrated by Koyanagi (1928) and Sallmann (1937). In




2686 TEXT-BOOK OF OPHTHALMOLOGY
young persons with normal eyes a vein occasionally describes a loop into
the outer nuclear layer of the retina (Sallmann, 1937); but in sclerosis,
Fig. 2290.-RETINAL ARTERLosolº Rosis.
Parallel sheathing at crossings: change of calibre of arteries (2nd branch on
dise of sup. ret: art.); deflection and banking of vein (2nd branch of sup.-nasal)
(Pines, Brit. J. O.).
especially in acute cases with hypertension, the same phenomenon may be
brought about pathologically (Fig. 2289). The occurrence, however, more
Fig. 2291,-RETINAL ARTERioscLERosis.
Aneurysmal dilatations of the veins (Pines, Brit. J. O.).
properly belongs to “deflections of the veins” and will be noted again
immediately.


DISEASES OF THE RETINA 2687
ſ
Fig. 2292.-RETINAL ARTERIoscLERosis.
Rt. sup: temp. vein crushed without deflection at first crossing, crushed and
deflected centrifugally at the second and third crossing; an arterial twig deflects it
centripetally. The small cilio-retinal artery shows changes in calibre (Pines, Brit. J. O.).
Fig. 2293.-RETINAL ARTERIosclerosis.
Compression and deflection of re. sup, temp. vein (Pines, Brit. J. O.).
T-0.-WOL. III. Q Q


2688 TEXT-BOOK OF OPHTHALMOLOGY
(iv) Compression of the veins, considered to be universal by Marcus
Gunn, is probably the most common phenomenon (Fig. 2286). To a very
slight degree this compression can be seen in the eyes of normal children
(Werner, 1913), so that, like many other of the evidences of sclerosis, this sign
is an accentuation of what may be a normal condition. It is important to
remember that much the same phenomenon can appear if the vein crosses
over the artery (Fig. 2287). Proximal to the crossing the vein is dilated and
swollen as if its circulation were to some extent impeded, a phenomenon
known as “banking,” and occasionally aneurysmal-like swellings appear
(Pines, 1929) (Figs. 2290–91), while in other cases an hour-glass constriction is
evident, a dilatation appearing both before and after the crossing (Fig. 2277).
The cause of these phenomena is so intimately associated with that of
deflection that the two will be considered together.
(b) Som E DEFLECTION OF THE VEINS OUT OF THEIR NORMAL COURSE
is a very common appearance. As we have just seen, in crossing under an
artery, the vein may dive down into the retinal tissues (Fig. 2285), and in
passing over it it may assume the appearance of an arch or a hump bridge
(Fig. 2288); but the most common deflections are lateral ones. Instead
of crossing under the artery obliquely, it does so at right angles taking,
apparently, the shortest possible route, a tendency which may be exaggerated
into the assumption of a sharp S-shaped or Z-shaped bend (Figs. 2292–93).
Alternatively, the vein is pushed centrifugally towards the periphery
(Figs. 2276, 2282 and 2297), and sometimes, a phenomenon first adequately
stressed by Pines (1929), it is pulled centripetally towards the disc
(Figs. 2292–93).
The phenomenon of combined concealment and deflection was elaborately
described by Salus (1929–39), who correlated the appearances particularly with
hypertension—“when an artery crosses a vein the latter forms before and behind the
crossing an arch, more or less large, and within the limits of this arch appears some-
what attenuated or even totally or partially invisible '' (SALUs’ SIGN).
It was generally assumed at first that the compression and deflection
of the vein was the simple result of pressure on its relatively soft
walls by the taut, hypertensive artery, and that this has some influence is
shown by the fact that a certain degree of arterio-venous compression can
be induced temporarily in the normal individual by the hypertension
artificially brought about by adrenalin (Bardsley, 1917), while it is noted
quite frequently in acute hypertensive states (acute glomerulo-nephritis, and
the toxaemia of pregnancy) long before there is time for organic sclerosis to
become manifest. This alone, however, cannot account for the extreme
degrees of the phenomenon met with in advanced sclerosis. These are the
result of organic changes in the vessel walls which normally are so intimately
associated that it is impossible to demarcate the two, a peculiar anatomical
relationship which has no analogy elsewhere in the body (Koyanagi, 1928;
Friedenwald, 1929; Sallmann, 1937–38) (Fig. 2294). The simple thickening
DISEASES OF THE RETINA
2689
FIG. 2294.-ARTERIO-VENous CRossING,
The artery is beneath the vein: showing fusion of the walls at this crossing.
(Friedenwald, T. O. S.).
of the wall of the artery will reduce the lumen of the vein; an athero-
sclerotic patch in the artery will penetrate into the vein causing a phlebo-
sclerosis (Coats, 1906), an actual displacement of the artery, due to thickening
and elongation, pushes the vein peripherally with it deflecting it centrifugally
Fig. 2295.-ARTERIo-venous CRossINo.
Showing thickened arterio-venous adventitia (Friedenwald, T. O. S.).


2690 TEXT-BOOK OF OPHTHALMIOLOGY
from its course, while a sclerotic contraction of the peri-vascularis pulls the
vein centripetally. The influence of perivascular sclerosis, which was first
stressed clinically by Pines (1929) and demonstrated anatomically by
Friedenwald (1930) (Fig. 2295), is probably very important: moreover, in
hypertensive subjects the adventitial sheath has been shown anatomically
to be particularly dense and constricting (Sallmann, 1937–38). The presence
in excess of this adventitial tissue is first demonstrated by the development
of sheathing at the crossings, and its subsequent contraction probably
accounts for the venous constriction when the vein passes over the artery, for,
enclosing the two vessels in its constricting embrace, the two will be pulled
together and the weaker of the two will be squeezed and deflected. The
natural tendency for the enlarged and tortuous artery will be to deflect the
vein centrifugally in the direction of the blood-stream this may be
neutralized by the opposite pull of the sclerotic peri-vascularis so that the
vein crosses at right angles, or the perivascular sclerosis may be the more
powerful influence and a centripetal deflection results.
5. IRREGULARITY OF THE LUMEN
In some cases, as was first shown by Raehlmann (1889), the lumen of
the arteries shows widenings and narrowings as judged by the breadth of the
Fig. 2296.-Remix at ARTERiosclerosis.
Sheathing, banking and deflection of veins; changes in calibre of artery
(sup, temp. art.) (Pines, Brit. J. O.). | -

DISEASES OF THE RETINA 2691
blood-column. This may be a fairly general phenomenon affecting both
arteries and veins, it may be localized to one vessel, or limited to small and
limited areas such as easily elude observation (Figs. 2296 and 2297); the
degree of constriction may also remain unchanged for years (Foster Moore,
1916). It has been demonstrated anatomically that these constrictions may
be due to the endarteritic proliferation of atherosclerosis, which is usually
-º º
º
Fig. 22.97.-RETINAL ARTERIoscLERosis.
Change of calibre of veins (sup, and inf. nasal), centrifugal deflection of inf.
temp. vein although crossing over artery (Pines, Brit. J. O.).
of patchy incidence (Raehlmann, 1889; Coats, 1904–13), a view which is
generally accepted; but on occasion it may possibly be associated with
localized sclerosis of the peri-vascularis (Pines, 1929) or to localized spastic
contraction (Mylius, 1928).
6. TORTUOSITY AND ENLARGEMENT OF THE WESSELS
A certain degree of tortuosity of the retinal arteries may be normal, a
phenomenon which may be greatly accentuated as a congenital anomaly; but
an increased degree of tortuosity which is always associated with an increase
in diameter, is frequent in sclerotic conditions, especially those of arteriolar
sclerosis. This was attributed by Thoma (1889) to weakening and stretching
of the vessel walls; but it is due rather to hypertrophic thickening. It is
obvious that such must be the case, for if the walls are increased in thickness

2692 TEXT-BOOK OF OPHTHALMOLOGY
they mustincrease in length also, since it is inconceivable that the new tissue
be laid down in one plane only. Owing to the physiological occurrence of
the phenomenon, however, its pathological assessment must be made with
reserve; but the extreme tortuosity of the smaller arterioles in the macular
region, as was pointed out by de Schweinitz (1906), is an extremely valuable
sign of the hyperplastic arteriolar sclerosis associated with raised arterial
tension (Fig. 2301). Tortuosity of the veins, especially of the smaller venules
(GUIst's sign, 1931), which is sometimes very marked and may be an early
and important sign of sclerosis, is probably a symptom of obstruction
(Figs. 2279, 2298).
7. GENERAL ATTENUATION OF THE RETINAL ARTERIES
At first a generalized narrowing of the retinal arteries so that they appear
much more attenuated than the veins, was described as a normal pheno-
menon of senility (Loring, 1886),
and although Gowers (1876) had
called attention to the phenomenon
in Bright's disease, its association
as a frequent sign in vascular
sclerosis was not established until
the work of Lurje (1893), Raehl-
mann (1889) and Gunn (1898):
its importance was emphasized by
Foster Moore (1916–17), Bardsley
(1917), Adams (1920), and Kahler
and Sallmann (1925–33). The
attenuation may be due to one of
two causes. Particularly in hyper-
tensive states it is caused by a
- tonic contraction of the walls
- º º ºº gº type : (Gowers, 1904; Volhard, 1929);
and in these the vessels may show
no histological changes. On the other hand, particularly in atherosclerotic
states, it may be due to organic thickening of the vessel walls, especially
of the sub-endothelial tissues which may reduce the blood-column to a
mere thread (Coats, 1913; Ballantyne, Michaelson and Heggie, 1938).
It is essentially a phenomenon associated with raised pressure, and it
may be taken as a general rule that if hypertension has persisted long the
arteries are small (Kahler and Sallmann, 1925–33). Friedenwald (1930)
considered that a reduction of calibre to one-half denoted a diastolic pressure
of 110, and Lobeck (1937) by actual measurement has shown that the
lumen of the arterioles may shrink to 26 microns in nephro-sclerotics from
the normal diameter of 134 to 88.
Fig. 2298-RETINAL ARTERLosclerosis.

DISEASES OF THE RETINA 2693
In sclerotic conditions associated with hypertension the course of the arteries
is straight, and the branches come off at acute angles as if the whole system had been
stretched (Lurje, 1893) (Fig. 2301). The cause of the phenomenon is disputed, and
histological evidence to explain the attenuation is lacking except that the thickened
and contracted walls make the blood-column thinner. Wagener (1924) suggested
that there is no actual diminution of the lumen and that the apparent reduction in
calibre of the blood-column was due to decreased transparency of the walls. Frieden-
wald (1930), on the other hand, basing his explanation on the proved fact of the
susceptibility of the central artery in the optic nerve to suffer advanced sclerotic
changes (Hertel, 1901; Coats, 1906–13), and on his observation of retraction of the
arterial anastomoses towards the disc when observed over a period of many years,
suggested that the appearances were due not merely to a decrease in calibre but also
to a decrease in length owing to sclerotic changes behind the disc.
THE PULSE. The frequent occurrence of a locomotor arterial pulse and a peri-
pherally progressive venous pulse was noted at an early period by Thoma (1889) and
Raehlmann (1889). The causation of these phenomena has already been discussed."
It may be especially marked at the arterio-venous crossings (Horniker, 1936).
Certain complications may be associated with vascular retinal sclerosis
the significance of which it will be well to summarize here.
(a) ANGrospas M. Spasm of the arteries, a phenomenon first adequately noted in
sclerosis by Wagenmann (1897), is of frequent occurrence. Apart from the fact that a
tonic spasm is responsible for the attenuation of the arteries in hypertensive cases,
Fig. 22.99.-RETINAL ANEURyswis IN ARTERIoscLERosis.
(Inf, temp. art.) (Ballantyne, T. O. S.).
* Vol. I, p. 398.

2694 TEXT-BOOK OF OPHTHALMOLOGY
at any rate in the initial stages, and may in large measure be responsible for the
pathological phenomena of retinopathies (Volhard, 1929), fleeting spasmodic contrac-
tions, sometimes obliterating the column of blood completely and giving rise to
symptoms of amaurosis fugax, are not uncommon. It will be remembered that such
a contraction occurs in other than arteriosclerotic states," but in these latter it is of
much graver prognostic significance.
(b) ANEURYsms. In direct contradistinction to their incidence in the cerebral
circulation, the formation of aneurysms in the retinal arteries is rare—when they do
occur they are usually of the miliary type as was first noted by Raehlmann (1889–1902).
They almost invariably appear in the smaller arteries, especially at the arterio-venous
crossings (Pines, 1929), or between localized points of constriction in the arteries (Leber,
1915; Bailliart, 1923). They are undoubtedly
due to the giving way of a diseased portion of
the arterial wall, and are essentially of the
nature of dissecting aneurysms rather than
actual dilatations of the lumen, and, although
they may be associated with haemorrhages
(Fernandez, 1920), the danger of rupture is
not great (Fig. 2299). For this reason they
may eventually disappear, their place being
taken by bright patches on the vessel wall
(Ballantyne, 1937).
Aneurysmal dilatations of the veins are
common at the arterio-venous crossings (Fig.
2291), but they may occur elsewhere,
particularly in small dilated collateral veins
or in terminal venous twigs (Fig. 2300).
(c) HAEMoRRHAges. In conditions of
sclerosis uncomplicated by retinopathy or
actual obstruction of the circulation, retinal
haemorrhages are relatively rare, and when
Fig. 2300–RETINAL they do occur they are usually small, occur-
ARTERLosol Enosis. ring by diapedesis rather than by rhexis and
Aneurysmal dilatation of vein, and a issuing from the smaller venules and capillaries
terminal umbrella-like compression and (Fig. 2299). They are usually a lated with
banking of vein (sup, temp.) (Pines, ------- y y associated wi
Brit. J. O.). hypertension (Lange and Lange, 1928), but
renal function may be adequate (Wagener,
1930). They are sometimes probably due to local atheromatous disease of the
vessel wall, but more usually are associated with capillary dysfunction resulting
from malnutrition owing to the poverty of the terminal circulation. Very varied
opinions have been expressed with regard to their prognostic importance. Gunn
(1898) considered a retinal haemorrhage the harbinger of a cerebral apoplexy; Pines
(1929) looked upon it as a serious warning, although the danger is not always immediate;
and Foster Moore (1925) took the intermediate view, which is probably most nearly
correct, that they are incidental to the arterial disease and not of any special significance,
although it must be remembered that sometimes an increase in their number precedes
the onset of serious symptoms.
(d) THROMBosts, particularly of the veins, but also of the arteries, occurs not
uncommonly, for apart from an infective phlebitis or arteritis, arterio- or phlebo-
sclerosis is the only other common aetiological factor in this calamitous accident.”
As we have already seen, the commonest cause is obstruction at an arterio-venous
p. 2566. * p. 2578.

DISEASES OF THE RETINA
2695
crossing. Geis (1911) concluded that only 40–50% of cases are associated with
Sclerosis and were of serious prognostic importance, but their significance can only
be assessed in association with the whole vascular picture.
(e) RETINOPATHY, the final complication, will be dealt with in the next section.
Adams.
1920.
P. R. S. Med., xvi, 16, 1922.
T. O. S., xlii, 21 1, 1922.
Brit. J. O., i, 161, 1917 ; iv., 297,
Bailliart. La Circulation retinienne, Paris,
1923.
Ballantyne. T. O. S., lvii, 301, 1937.
Ballantyne, Michaelson and Heggie. T. O. S.,
lviii, 255, 1938.
Bardsley. T. O. S., xxxiii, 46, 1913.
Brit. J. O., i. 239, 1917.
Bridgett. Am. J. O., ix, 725, 1926.
Bright. Guy’s Hosp. Rep., i, 356, 1836.
Coats. T. O. S., xxiv, 165, 1904 ; xxxiii, 37,
1913.
R. L. O. H. Rep., xvi, 62, 516, 1906.
Dimmer. Der Lichtrefleace d. Netzhaut, Wien,
1891.
Doyne. T. O. S., xxiv, 121, 1904.
Fahr. Hb. sp. path. Amat. Histol., vi (1), 156,
1925.
Fernandez. Am. J. O., iii, 641, 1920.
Fishberg. Hypertension and Nephritis, Phila.,
iii, 1934.
Friedenwald. J. Am. Med. As., xvi, 622,
1891 ; lixxxv, 428, 1925.
A. of O., xxv, 177, 1896.
Bull. Johns Hop. Hosp., xlv. 232, 1929.
T. A. m. O. S., xxvii, 188, 1929.
T. O. S., i, 452, 1930.
Frost. T. O. S., xxiv, 122, 1904.
Geis. K. M. Aug., xlix, 1, 1911.
Gowers. Brit. Med. J., ii, 743, 1876.
Manual of Med. Ophthalmoscopy, IV,
London, 1904.
v. Graefe. A. f. O., ii (1), 214, 1855.
Guist. Z. f. Aug., lxxiii, 232, 1931.
Gunn. T. O. S., xii, 124, 1892; xviii, 356,
1898 ; xxiv, 119, 1904.
Hertel. A. f. O., lii, 191, 1901.
Hirschberg. Cb. pr. Aug., vi, 327, 1882;
ix, 65, 1885.
Horniker. K. M. Aug., xcvii, 315, 1936.
Hulke. R. L. O. H. Rep., v, 16, 1866.
Iwanoff. K. M. Aug., iii, 328, 1865.
Kahler and Sallmann. Z. f. Aug., lvii, 368,
1925.
A. f. O., csxxi, 505, 1933.
Royanagi. K. M. A ug., lxxxi, 219, 1928.
Lange and Lange. Kl. W., vii, 2286, 1928.
Leber. G.-S. Hb., II, vii (a), 871, 1915.
Liebreich. A. f. O., v. (2), 265, 1859.
Lobeck. A. f. O., csxxvi, 434, 1937.
Löhlein. Arb. a. d. path. Inst., Leipzig, 1907.
Loring. T.-B. of Oph., N.Y., 1886.
Lurje. Diss., Dorpat., 1893.
Mauthner. Lehrb. d. Oph., Wien, 1868
Moore, Foster. T. O. S., xxxv, 159, 1915;
xxxvi, 319, 1916.
Quart. J. Med., x, 29, 1916.
Brit. J. O., Suppl., ii, 1924.
Medical Ophthalmology, London, II, 1925.
Müller, H. Verhl. d. phys.-med. G., Würzburg,
vii, 293, 1857.
Würzburg. med. Z., i, 45, 1860.
Mylius. Z. f. Aug., x, Beil., 1, 1928.
Pines. Brit. J. O., xi, 489, 1927 :
161, 226, 1929.
Raehlmann. A. f. Psy. Nervenkr., xx, 566,
1888.
K. M. Aug., xxvii, 203, 241, 496, 1889.
Fortsch. d. Med., vii, 928, 1889.
Z. f. kl. Med., xvi, 606, 1889.
Z. f. Aug., vii, 343, 425, 1902,
Sallmann. A. f. O., czyxvii,
cxxxviii, 380, 1938.
Salus. K. M. Aug., lxxxii, 47 1, 1929.
Med. Kl., ii, 1885, 1931 ; ii, 906, 1935.
A. of O., xxi, 505, 1939.
Scheerer. A. f. O., exii, 206, 1923; c Xv, 370,
1924.
Schieck. K. M. Aug., lxvi, 39, 1921.
Zb. f. ges. O., v., 465, 1921; xxi, i, 1929.
xiii, 97,
619, 1937;
Schweigger. A. f. O., vi (2), 294, 1860.
de Schweinitz. Maryland Med. J., xliii, 275,
1900.
O. Rec., xv, 383, 1906.
Internat. Clin., i, 177, 1907.
Thoma. A. f. O., xxxv (2), 1, 1889.
Virchow. A. f. path. Anat., x, 170, 1856.
Volhard. Zb. f. ges. O., v, 470, 1921 ; xxi,
129, 1929.
Hb. d. inneren Med., Berlin, vi, 1931.
Wagener. Am. J. O., vii, 272, 1924.
T. A. m. O. S., xxv, 349, 1927.
A. of O., iii, 335, 1930.
Wagenmann. A. f. O., xliv, 219, 1897.
Wedl. Atlas path. Amat. d. Auges, iv., 1861.
Werner. T. O. S., xxxiii, 9, 1913.
Wilmer et alia. A. of O., ix, 368, 1933.
We shall now proceed to discuss the various clinical syndromes which
occur in different types of vascular disease.
It must be emphasized, how-
ever, that it is rather the exception than the rule for them to be seen in a
simple uncomplicated form ; more usually one or more types are combined,
or the one merges into the other making differentiation difficult or impossible.
2696 TEXT-BOOK OF OPHTHALMOLOGY
The following clinical pictures must therefore be interpreted more as theore-
tical conceptions than as practical occurrences, and are described separately
merely as a matter of convenience. For systematic purposes, however, we
shall divide them into two main classes—vascular Sclerosis when gross changes
are limited to the vessels without extensive involvement of the retinal tissue,
and vascular retinopathies wherein the retina is disorganized to some degree.
Wascular Sclerosis
Conditions of vascular sclerosis assume myriad forms, but out of the
many clinical pictures there emerge two essential types of change, which,
of course, are more commonly found in combination than separately.
These are (1) the senile changes of diffuse atherosclerosis, and (2) changes
due to hypertension.
1. DIFFUSE ATHEROSCLEROSIS
DIFFUSE ATHEROSCLEROSIs (involutionary arteriosclerosis of Allbutt or
medial arteriosclerosis) a fibrous and hyaline degeneration of the muscularis,
is an almost universal age-change of a degenerative type and represents the
results of the wear and tear of life. It is more or less equally distributed all
over the body, and is quite independent of the blood-pressure ; indeed, in the
brain it may lead to dementia while the blood-pressure still remains normal.”
Its effect is evident in a slow diminution of the activities—mental and
physical—and although it attacks all the organs, in itself it rarely causes
serious tissue-damage. It is extraordinary, indeed, how well the parenchyma.
of organs survives in the presence of advanced sclerosis, for between the two,
the degree of sclerosis and the state of the parenchyma, there is little
parallelism. The latter is only invaded by an increase of interstitial tissue
and can carry on its function fairly well, although to a modified degree.
These senile changes, therefore, while attacking the retinal vessels diffusely, do
not cause a markedly pathological clinical picture so long as the blood-pressure
remains within normal limits : moreover, in comparison with other organs
they show themselves in the eye to a relatively slight extent and late.
Nevertheless they are of universal occurrence after the fifth or sixth decade.
Usually the first change to be detected is that the arteries show increased
translucency so that they become paler and their reflex streak duller, and
they eventually hide the veins as they cross them. At a later stage the
larger branches are full, tortuous, and begin to assume a copper-wire appear-
ance, while the most pronounced changes which occur in simple cases as the
arteries become harder are a moderate degree of arterio-venous constriction
with deflection of the veins and perhaps the development of white sheathing
(Figs. 2301, 2316, Plate LI). The grosser changes associated with sclerosis,
1 Vol. II, p. 1431.
DISEASES OF THE RETINA 2697
as well as haemorrhages or exudates, do not appear in the absence of
complications; indeed the essential damage to the retina in this condition
tends not to be primary, but rather to depend on sclerosis in the choroid,
a process which tends to involve a secondary degeneration in the central
region (senile macular degeneration)."
Fig. 2301. THE VEssBLs IN ARTERIoscLERosis AND HYPERTENsios.
A. Normal. B. Atherosclerosis without hypertension—beading and visibility
of arteries, moderate arterio-venous compression, and normal arterioles. C. Arterio-
sclerosis with hypertension—straight, generally constricted arteries branching at
acute angles, showing variations in calibre and marked arterio-venous constriction
with banking. D. Arteriolar sclerosis—full, tortuous copper-wire arteries, branching
at right angles, moderate arterio-venous constriction, while the arterioles show
irregular tortuosity, fine variations in calibre, visibility of the walls and silver-wire
changes (Friedenwald, T. O.S.).
Localized atheromatous changes in the intima are not common in the
retinal vessels, and even when atheroma occurs to a severe degree in the
aorta and coronary circulation, the retinal vessels may be free (Foster Moore,
1925). The condition manifests itself by endothelial and sub-endothelial
p. 2372.

26.98 TEXT-BOOK OF OPHTHALMOLOGY
proliferation as a response to circulating toxins of a bacterial or metabolic
nature and is notoriously patchy in its distribution (Fig. 2302). Its site
of election in the ocular circulation is the central retinal artery within
the optic nerve, particularly at the level of the lamina cribrosa (Siegrist,
1899: Hertel, 1901; Coats, 1906–13), and those lesions which occur in the
retina are to be regarded as outposts of this central process. Clinically the
ophthalmoscopic evidence produced is narrowing and irregularity of the
lumen of the arteries which is frequently most marked near the arterial
Fig. 2302, ATHERoma.
In atherosclerosis—a branch of the central retinal artery stained and mounted
flat (Friedenwald, T. O. S.).
divisions, but so long as it is not complicated by hypertension, clinical signs
are absent until obstruction of the artery occurs and thrombosis develops
with its characteristic oedema and haemorrhagic effusion."
2. HYPERTENSIVE SCLE Rosis
Essº NTIAL HYPERPIESIs is a persistent and usually progressive raising
of the blood-pressure, which represents the response of the vascular system
generally to some underlying disorder, hereditary, metabolic, toxaemic,
infective, endocrine or psychological, which probably causes primarily an
over-activity of the vaso-motor centre by nervous orchemical pressor agencies.
In the assessment of a high blood-pressure it is to be remembered that the systolic
pressure is much less significant than the diastolic. The former is an indication of the
maximum effort of the action of the heart and by its very height denotes a good
response; the danger it involves is the rupture of an artery, which in the eye is never
p. 2572.

DISEASES OF THE RETINA 2699
very great. A high diastolic pressure, which indicates the continued pressure and
therefore the permanent stress upon the vessel walls, is of much more serious significance,
and when it is raised, and more so when it keeps on rising, the prognosis is bad. When at
the same time the systolic pressure tends to fall, pointing to faltering in the heart’s
efforts, a fatal termination is imminent. So long as the systolic and diastolic pressures,
therefore, remain high but keep parallel the prognosis is more favourable : when they
approach one another the prognosis is serious.
The response of the vascular system to the aetiological factors deter-
mining hyperpiesia is initially a tonic contraction of the vessel walls—a
functional response which may persist, sometimes for years, without patho
logical changes when a state of high blood-pressure without sclerosis exists
If the aetiological factor is removed at this stage the blood-pressure
may come down to normal. If it persists sufficiently long, however, and
sometimes the interval need be very short, changes occur in the peripheral
arteries which may be grouped under the term DIFFUSE HYPERPLASTIC
ARTERIOSCLEROSIS.
This condition of essential or benign hypertension is relatively mild and the
changes it involves are essentially vascular ; moreover, it is not incompatible with a
long life. Death, when it does come is due to apoplexy, cardiac defeat or terminal
pneumonia. MALIGNANT HYPERTENSION, on the other hand, is an acute and relatively
uncontrollable toxic condition, but fortunately an uncommon one. It may arise
independently of or may follow from the former, and is more frequent among the young
than the benign form. Pathologically it is characterized by a diffuse and extreme
constriction of the arteries, resulting in intense Oedema and changes in the tissues which
are evident in the eye as a retinopathy"; clinically, by a short stormy course ending
in renal death with uraemia and convulsions.
Cases of benign hypertension may therefore be described in two stages
—the pre-sclerotic stage of hyperpiesia, and the Organic stage of diffuse
hyperplastic (arteriolar) sclerosis.
(a) SIMPLE HYPERPIESIA
A state of hypertension unaccompanied by vascular sclerosis betrays
itself by few retinal signs. Such a state may occur in the early stages of an
essential hyperpiesia and in other toxic conditions such as acute glomerulo-
nephritis, mild toxaemias of pregnancy, or hyperthyroidism (Bardsley, 1917;
Altnow, 1927; Friedenwald, 1930 ; and others). The fundus as a rule is
normal, showing as the earliest sign an absence of the light reflex on the veins
near the arterial crossings presumably owing to a flattening of their convexity
(Adams, 1920), and a slight degree of arterio-venous constriction. When the
blood pressure is raised, as is seen, for example, in acute fevers or toxaemias
(influenza, scarlet fever), the arteries may be engorged and their reflex streak
broadened (Bardsley, 1917); but after hyperpiesia has become developed
the arteries become small in size and pale in colour owing to hypertonic
contraction of their walls. At this stage, also, intermittent spasms in the
1 p. 2726.
2700 TEXT-BOOK OF OPHTHALMOLOGY
arterial wall may be observed (Volhard, 1929; Horine and Weiss, 1931),
which eventually assume a clonic form ; these may relax and the vessels
return to normal, but it is also possible that they may induce permanent
changes in the vessel wall. Engorged arteries are not associated with a
persistent hypertension. Only in the most severe case some oedema at the
disc and macula may occur.
An interesting case was reported by Cattaneo (1937) of the development of sudden
hypertension associated with constricted arteries and transient blindness occurring
in a young adult after mushroom poisoning. The vision improved after retro-bulbar
injections of acetyl-choline.
If the causal factors are removed these changes in the fundus may dis-
appear completely; if they are not, after a longer or shorter interval, diffuse
hyperplastic sclerosis may develop and complicate the picture.
(b) DIFFUSE HYPERPLASTIC ARTERIOsCLEROSIs: ARTERIOLAR SCLEROSIs
DIFFUSE HYPERPLASTIC ARTERIOSCLEROSIs, or since the changes are
seen most markedly in the intimate ramifications of the vascular tree,
ARTERIOLARSCLEROSIS, is therefore in large part a compensatory phenomenon,
being the mechanical response to increased strain. The essential change is
initially a proliferation and hypertrophy of the media, especially of its
muscular, sub-endothelial and elastic tissue, a process which is followed by
fibrosis both in the vessel wall and the peri-vascularis, and may end in
great narrowing or obliteration of the lumen.
Clinically this process is characterized in the larger arteries by marked
localized differences in calibre, irregular tortuosity, a well-developed copper-
wire reflex, arterio-venous constrictions, and gross sheathing. The most
typical changes, however, are seen in the smaller vessels which correspond
to arterioles, particularly those around the macula ; their visibility is
increased, and they become tenuous and tortuous to such a degree that the
term corkscrew is very applicable (de Schweinitz, 1906; O’Hare and Walker,
1924–28) (Figs. 2301, 2317, Plate LI). As time goes on these phenomena
increase in intensity, the entire arterial tree becoming more attenuated,
sheathing, beading, and irregular constrictions increasing and all the phenom-
ena of Salus' arch appearing at the arterio-venous crossings, until eventually,
if the condition cannot be controlled, haemorrhages and exudates appear, and
ultimately the typical picture of hypertensive or renal retinopathy develops.
It is important to remember that in this class of patient a considerable
number show exudative and haemorrhagic changes, such as used to be called
‘‘ albuminuric retinitis,” without the albumen appearing in the urine (present
in 21 and absent in 11 cases, Gresser, 1935).
These hyperplastic arteriosclerotic changes, however, are frequently not uncom-
plicated. In the presence of diffuse atherosclerosis they are more pronounced
and the existence of established sclerosis at once introduces an increased element of risk;
DISEASES OF THE RETINA 27.01
FIGs. 2303–08.-END ARTERITIs IN THE CENTRAL RETINAL VEssels.
(x 120) (Coats, T. O. S.).
Fig. 23.03.-Normal central vessels. Fig. 2304.-Endarteritis in central artery.
New formation of tissue on inner aspect of
elastic lamina which is thrown into folds.
The lumen is encroached upon but lined by
endothelium. There is an organizing
thrombus in the vein.
Fig. 2305-A more advanced condition Fig. 2306.-Degenerative stage. The
stained with Weigert’s elastic tissue stain. cells are swollen, fatty and breaking
The elastic lamina is enormously thickened down, but some elastic fibres survive
with much new formation of elastic fibres near the lumen.
on its inner aspect forming new membranes
within the old.
º º- + º-ºººº
- -------
ºº: -º-
"…" ºr-º- º
- --------------- --- -
…º.º.º.º. º
| * ...? -- -- $º.
-
*.*.*.*. -
ºr.
º
- --- -
------ * -
** ***
º
------
Sº
- - - -
º-ºº------.
Fig. 23.07.-An extreme example of endarteritis, Fig. 2308. Fibrosis of a retinal vessel.
showing the lumen reduced to very small dimen- The fibrous wall is greatly thickened, but
sions and much degeneration in the proliferated the endothelium is intact.
tissue. Infiltration of the walls of the vein.






2702 TEXT-BOOK OF OPHTHALMIOLOGY
but greater dangers ensue when a parallel impairment of renal function adds to the
toxemia. One of the most significant prognostic signs is the appearance of the
arterio-venous crossings: Brana and Radnai (1934), as a result of an intensive study
of 100 cases of hypertension, found that when the beginnings of a Salus' arch were
visible the mortality from hypertension was 2%, within 1 year, in cases when marked
variations occurred in the appearances at the crossings the mortality was 60%, and
when these phenomena were most striking, death within a year was invariable.
INVOLUTIONARY ARTERIOSCLEROSIS WITH HYPERTENSION
It is exceedingly difficult to draw any hard and fast line between the
picture of uncomplicated atherosclerosis and that complicated by hyper.
tension, for in most cases in age the blood-pressure gradually rises, and the
one picture merges into the other, the changes being rather of degree
than in kind. If, however, diffuse atherosclerosis is associated with any
degree of hypertension the clinical picture is at once more dramatic, and
signs of gross vascular sclerosis become evident. In these cases the
Figs. 2309-11-RETINAL ARTERIoscLERosis is a BRANch vessel.
-
Fig. 2309. - Eccentrie endo- Fig. 2310–The lumen is almost filled
thelial proliferation into the by endothelial proliferation (Coats).
lumen of an artery (Coats).
Fig. 2311-Fibrosis of connective tissue wall of vein: the intima is intact (Coats).


DISEASES OF THE RETINA 2703
essential and pathognomonic feature is that the arteries are typically
narrow and diffusely constricted, the larger vessels becoming small and the
smaller invisible (Fig. 2301); it may indeed be taken as almost axiomatic
that the most constant and characteristic sign of raised tension is a narrowing and
straightening of the arteries (Friedenwald, 1930; Kahler and Sallmann,
1925–33). As a rule the arteries are straight and frequently branch at
acute angles while their reflex becomes harder, changing to a silver-wire
appearance. In addition to the diffuse constriction, local variations in
Fig. 2312.-RETINAL ARTERIoscLERosis.
Almost complete obliteration of the lumen of the artery at the disc (Rones,
Am. J. O.).
calibre are the rule, so that the light streak becomes broken up and beaded,
and in markedly constricted vessels, may disappear entirely. At the same
time parallel sheathing becomes obvious and may progress to pipe-stem
sheathing while the phenomena at the arterio-venous crossing both of
compression and deflection are seen in an extreme degree. It is this type
of case which after a number of years ultimately tends to develop the
picture of arteriosclerotic retinopathy with the appearance of exudates and
haemorrhages.
The pathological anatomy of these changes has been studied by many observers,
particularly Raehlmann (1889–1902), Siegrist (1899), Hertel (1901), Harms (1905),
Coats (1906–13), Baumgårtner (1914), Cohen (1922), Bailliart (1923), Bridgett (1926),
T-0.-WOL. III. R. R.

2704 TEXT-BOOK OF OPHTHALMOLOGY
Verway (1927), Keith, Wagener and Kernohan (1928), Koyanagi (1928), Friedenwald
(1929), Kyrieleis (1930), Garsteiger (1937), Sallmann (1937–38), Ballantyne, Michaelson
and Heggie (1938) and others; nevertheless the subject is still incompletely under-
stood largely because in most of the eyes
examined the sclerosis has been compli-
cated by advanced and destructive tissue-
changes. It is interesting, however, that
pathological and clinical appearances do
not always coincide, for no pathological
changes can sometimes be found when
extensive disease has been noted clinically
(Agatston, 1926–28), and at other times
extensive pathological disease may exist
without ophthalmoscopic evidence (Coats,
1913).
In general the changes correspond
with those found elsewhere in the body
in similar circumstances. In the peri-
vascular adventitial tissue there may be
a considerable degree of fibrosis and
thickening. In the arterial wall itself in
the later stages of all types of changes
there is usually a proliferation of the
connective tissue and elastic elements
developing into a fibrous thickening of the
media and its eventual hyaline replacement
or fatty degeneration, tending to involve
narrowing of the lumen (Figs. 2303–13).
The most characteristic change is a
proliferation of the sub-endothelial tissue
of the intima. This is seen in nodular
form in atheromatous lesions, but in the
Hyaline degeneration of media in more common hyperplastic type of sclerosis
retina atteriole (Rones. Am. Joº. it involves the formation of new laminated
cellular tissue with reduplication of the
elastic structures which may encroach gravely upon the lumen frequently deviating
it eccentrically; eventually this tissue may undergo fatty degeneration and complete
disintegration.
Fig. 2313.-RETINAL ARTERLoscLERosis
The General Relationships of Vascular Sclerosis
Although as a whole there is little irregularity in the distribution of
sclerotic changes between the two eyes, the differences being of degree rather
than of kind, the variation of these changes throughout the body both in
degree and in incidence is frequently considerable; they do not occur
regularly as cause and effect, and in the later stages the most diverse conse-
quences may follow similar syndromes, this one showing predominantly
ocular disease, that one cerebral, another cardiac, and still another renal.
Thus Wagener (1924) reported that of 200 cases of hypertension 56-5% showed
clinical retinal arteriosclerosis, of which 30% showed no general arteriosclerosis:

DISEASES OF THE RETINA 2705
46.5% showed general disease of which 20.4% showed no retinal involvement ; 13.5%
had cerebral arteriosclerosis of which 25.9% had no retinal evidence. Statistics
given by various authors as to the occurrence of retinal sclerosis in general arterio-
Sclerosis varies, but most of them are high—Hirschberg (1885) 46%, Raehlmann
(1888) 50%, Friedenwald (1891) 82%, Rud and Möeller (1928) 4%, Wagener (1930)
80%. Similarly in 200 necropsies Bridgett (1926) found that sclerosis of the retinal
arteries was found in only 18% of the cases which showed gross changes in the aorta,
Coronary and cerebral arteries. A similarly close association between cardiac disease
with hypertension and retinal sclerosis was demonstrated by Altnow (1927) and
Yater and Wagener (1929).
It is indeed obvious that arteriosclerosis is a capricious disease, varying
much in the extent of its manifestations all over the body, but most are agreed
that arteriolar sclerosis is distributed regularly with great uniformity
throughout all the organs. Moreover, while retinal sclerosis can give no safe
indication of the degree of sclerosis elsewhere, its occurrence in the eye almost
certainly indicates its presence generally.
Retinal and Cerebral Arteriosclerosis
Ever since attention was drawn to the fact by Hirschberg (1882–85) that a number of
patients who showed retinal arteriosclerosis developed hemiplegia or died of apoplexy,
it has generally been considered that a close relationship existed between the condition
of the vessels in the eye and the brain (Raehlmann, 1888; Gunn, 1898; Marple, 1907;
Geis, 1911 ; Gordon Holmes, 1913; Adams, 1917–20 ; Davies, 1923 ; Uhthoff, 1927 ;
Wagener, 1930; Evans, 1933; and others). Foster Moore's (1916) material is typical :
in 44 cases of cerebral haemorrhage or thrombosis he found that 43% had a severe
and 27% a mild degree of retinal sclerosis, while 30% had no evidence of retinal
vascular disease. Pathologically the close relationship between sclerotic disease in the
two areas has also been confirmed (Lurje, 1893; Baumgårtner, 1914); there is, how-
ever, no question of uniformity in the relationship, for the retinal arteries are frequently
healthy when the cerebral vessels show advanced disease (Hertel, 1901 ; Coats, 1913).
It seems evident pathologically, however, that the association between the two so
far as arteriolar sclerosis is concerned is particularly close (Verwey, 1927; Volhard,
1929).
Although some writers (Rud and Möeller, 1928) deny any parallelism,
it must be admitted that the state of the arteries in the eye and the brain
shows on the whole a striking relationship, and that the best way to obtain
information about the cerebral vessels is by means of the ophthalmoscope.
The considerable difference in the distribution of atheroma must, however,
be remembered, and the fact that arteries of the calibre which usually are
associated with a cerebral haemorrhage are unrepresented in the eye. More-
over, the external support of the intra-ocular pressure and the danger of the
close association between arteries and veins at their crossings are phenomena
unrepresented in the brain.
Adams. Brit. J. O., i, 161, 1917 ; iv, 297, Altnow. A. Int. Med., xl, 757, 1927.
309, 1920. Bailliart. La circulation retinienne, Paris,
Agatston. Med. J. and Rec., csxiii, 799, 1926. 1923.
A. of O., lvii, 386, 1928. Ballantyne, Michaelson and Heggie. T. O. S..
lviii, 255, 1938.
R R 2
2706
TEXT-BOOK OF OPHTHALMOLOGY
Bardsley. Brit. J. O., i, 239, 1917.
Baumgårtner. A. f. Aug., lxxvii, 145, 1914.
Brana and Radnai. K.M. Aug., xciii, 455, 1934.
Bridgett.
Cattaneo.
Coats.
Am. J. O., ix, 725, 1926.
An. di Ott., lxv, 81, 1937.
R. L. O. H. Rep., xvi, 62, 516, 1906.
T. O. S., xxiv, 165, 1904; xxxiii, 37, 1913.
Cohen. Am. Med. As. Sect. O., 60, 1922.
Davies. P. R. S. Med., xvi, 26, 1923.
Evans. Brit. J. O., xvii, 257, 1933.
Friedenwald, H. J. Am. Med. As., xvi, 622,
1891.
T. O. S., l, 452, 1930.
Friedenwald, J. Bull. Johns Hopkins Hosp.,
xlv., 232, 1929.
Garrod. T. O. S., x1, 6, 1920.
Garsteiger. K. M. Aug., xcix, 604, 1937.
Geis. K. M. Aug., xlix, 1, 1911.
Gresser. Am. J. O., xix, 426, 1935.
Gunn. T. O. S., xviii, 356, 1898.
Harms. A. f. O., lxi, l, 1905.
Hertel. A. f. O., lii, 191, 1901.
Hirschberg. Cb. pr. Aug., vi, 327, 1882.
Hirschberg and Birnbacher. Cb. pr. Aug., ix,
65, 1885.
Holmes. T. O. S., xxxiii, 45, 1913.
Horine and Weiss. A. of O., vi, 535, 1931.
Rahler and Sallmann. Z. f. Aug., lvii, 368,
1925.
A. f. O., czzxi, 505, 1933.
Koyanagi. K. M. Aug., lxxxi, 219, 1928.
Kyrieleis. A. f. Awg., ciii, 161, 1930. -
Lobeck. A. f. O., cxxxvi, 434, 1937.
Lurje. Diss., Dorpat., 1893.
Marple. Med. Rec., N.Y., lxxi, 421, 1907.
Moore, Foster. Quart. J. Med., x, 29, 1916.
Medical Ophth., London, II, 1925.
O'Hare and Walker. A. Int. Med., xxxiii,
343, 1924.
J. A. m. Med. As., xc, 1436, 1928.
Raehlmann. A. f. Psy. Nervenkr., xx, 566,
1888.
Z. f. kl. Med., xvi, 606, 1889.
Z. f. Aug., vii, 425, 1902.
Rud and Möeller. Acta O., vi, 317, 1928.
Sallmann. A. f. O., czzxvii, 619, 1937;
cxxxviii, 380, 1938.
de Schweinitz. O. Rec., ii, 383, 1906.
Shaw. Hyperpiesis and Hyperpiesia, London,
2
$n
* * *
Siegrist. Z. f. Aug., ii, Beil., 36, 1899.
Uhthoff. K. M. Aug., lxxviii, Beil., 1, 1927.
Verway. K. M. Aug., lxxix, 148, 1927.
Volhard. Zb. f. ges. O., xxi, 129, 1929.
Wagener. Med. Clim. N. Amer., vii, 275,
1924.
Am. J. O., vii, 272, 1924.
A. of O., iii, 335, 1930.
Yater and Wagener.
clxxviii, 105, 1929.
Am. J. Med. S.,
Reith, Wagener and Kernohan. A. Imt. Med.,
xli, 141, 1928.
Wascular Retinopathies
When in addition to the factor of vascular sclerosis, hypertension and
toxaemia are added, more dramatic and generally distributed appearances
ensue in the retinal tissues themselves, characterized essentially by the
appearance of haemorrhages and exudates ; these may be classed as VASCULAR
RETINOPATHIES.
The term eacudates, used in this connection is an unfortunate word, for the white
or yellow areas are not usually exudative in nature, but degenerative and proliferative.
It has, however, acquired universal use, and since it is difficult to suggest an alternative
without circumlocution, I am retaining it.
Up to comparatively recently two entities were generally recognized,
Liebreich's (1859) term albuminuric retinitis being used as a generic descrip-
tion for all lesions of the fundus characterized by hamorrhages and exudates
occurring in patients with albumen in the urine, and v. Jaeger's (1856) term
diabetic retinitis for those accompanied by glycosuria. In a general
sense this position remained unchanged until Foster Moore (1916) differen-
tiated the picture produced by uncomplicated retinal arteriosclerosis as
arteriosclerotic retinitis from that characteristic of “ renal retinitis,” main-
taining that the two were not different stages of the same disease, as had
been assumed, but were different entities. At a later date Keith, Wagener
DISEASES OF THE RETINA 2707
and Kernohan (1928) differentiated between the picture produced by
malignant hypertension (malignant nephro-sclerosis), wherein toxaemic
hypertension is primarily at fault, from that occurring in glomerulo-
nephritis (secondary nephro-sclerosis) wherein renal disease is primarily at
fault. It would seem that these differentiations have been made with reason
and therefore we shall divide the traditional unitary conception of
albuminuric retinitis into three types: arteriosclerotic retinopathy, malig-
nant hypertensive retinopathy, and renal retinopathy. When glycosuria is
present, a fourth entity, diabetic retinopathy is prone to appear, which in
many cases, but not in all, may be considered as an extreme and more
acute form of the arteriosclerotic type occurring in diabetic persons. It is
noteworthy and important that, with the exception of some diabetic cases,
ALL OF THESE CONDITIONS ARE DEPENDENT UPON AND ONLY ASSOCIATED
witH THE CONDITION OF HYPERTENSION, without which they do not occur
even in the presence of arteriosclerosis or widespread renal destruction,
as when the kidney is practically destroyed by tubercle, sepsis or a neoplasm.
It is to be remembered, however, that a systematic differentiation of
this type has been questioned by some authorities who hold that any
differences are rather of degree than of type. Thus, as extreme views,
Batty Shaw (1922) contended that arteriosclerotic and renal retinopathies
were indistinguishable, the differences in the ophthalmoscopic picture in
individual cases being dependent merely on the concentration of the
action of toxins, while Garrod (1920) adopted a similar reasoning with regard
to diabetic retinopathy. It is true that the pathological basis of all these
conditions is similar, and in a large number of them to make a clinical
diagnosis from ophthalmoscopic appearances alone is sometimes quite
impossible and often both difficult and dangerous ; but it is only to be
expected that in a tissue so highly differentiated as the retina, degenerative
and toxic processés will produce pathological alterations of a closely related
type the nature of which is determined as much by the structure itself as by
the causal agent. Both from the topographical and the prognostic points
of view, however, there is undoubted justification for a differentiation of
types.
1. ARTERIOSCLEROTIC RETINOPATHY
When the conditions of vascular sclerosis and hypertension have
persisted for a number of years the normal evolution is for the ophthalmo-
scopic picture to evolve from that of vascular sclerosis into a generalized
retinal disturbance with the appearance of haemorrhages and exudates (the
arteriosclerotic retinitis of Foster Moore, 1916) (Figs. 2314 and 2318, Plate LI).
The retinal vessels show the characteristics of marked sclerosis :
irregularity of the lumen, copper-wire or silver-wire arteries, sheathing,
arterio-venous compression, and as a rule, marked tortuosity especially in
27.08 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2314.—ARTERIoscientoric RETINorATHY (Parsons).
the smaller vessels associated with diffuse hyperplastic sclerosis. The disc
shows no changes, except perhaps a slight haziness in its outlines or atrophic
changes in the later stages; the complete absence of oedema is the most
important point of differentiation from the more acute types characteristic
of hypertensive or renal disease.
The typical exudate consists of small bright spots mainly in the central
Fig. 2315.-ARTERIoscLERotic RETINopathy.
A. Nerve-fibre layer. B. Rod and cone layer. The circular masses in the
external nuclear layer are hyaline deeply stained by orcein, which form the exudative
dots seen ophthalmoscopically, Systolie B.P. 240 mm. Death from cerebral
thrombosis (Foster Moore, Medical Ophthalmology).


PLATE LI
VAscular Diseases oF THE RETINA
Fig. 2316.-ARTERIoscLERosis. Fig. 23.17. HYPERTENSION.
Without retinopathy. Note variations in Note attenuated tortuous arteries and
calibre of arteries, sheathing, and com- haemorrhages.
pression of veins (Williamson-Noble).
Fig. 2318.-ARTERIoscLERotic RETINop ATHY.
Fig. 2319.-DIABETIC RETINopathy. Fig. 2320.-DIABETIC RETINopathy.
Haemorrhagic type (Gray). Exudative type (Gray).
[To face p. 2708.



DISEASES OF THE RETINA 2709
area of the fundus; they are rarely of any great size, circular in shape,
sharp-edged, and without surrounding oedema. In contra-distinction to
the soft fluffy cotton-wool patches of the more acute retinopathies of malig-
nant hypertension or nephro-sclerosis they are described as hard. Patho-
logically they seem to be circular masses of hyaline material situated in the
outer molecular layer (Foster Moore, 1925) (Fig. 2315). They are usually
not very profuse and may be limited in number, but sometimes they powder
the central area liberally or are arranged in a circinate manner or in a macular
star. They appear stable and are slow to disappear, but when they do so
they are usually replaced by others, although occasionally they may dis-
appear entirely. The haemorrhages are usually small and scattered, super-
ficial or deep, and with or without relation to the larger vessels. As they
and the exudates are absorbed and are followed by others, the fundus picture
may change considerably from time to time.
We have seen that retinal arteriosclerosis is always bilateral, although
frequently unequally developed in the two eyes; these retinopathic changes
however, are not uncommonly unilateral and remain so for long periods
(in 45% of cases, Foster Moore, 1925). Their significance is merely that of
severe vascular sclerosis associated with hypertension, a condition in
which life may be prolonged, usually with somewhat restricted
habits, for an indefinite number of years, and death, when it does come,
tends to be cardiac or cerebral and is unassociated with failure of renal
function. Neither the presence of retinal exudates nor haemorrhages is of very
definite prognostic significance ; the most serious single feature is a marked
attenuation of the arteries themselves (Brana and Radnai, 1935; Gillan, 1939).
For the general treatment, see p. 2735.
Brana and Radnai. Wien. A. int. Med., Keith, Wagener and Kernohan. A. Int. Med.,
xxvii, 371, 1935. xli, 141, 1928.
Garrod. T. O. S., x1, 6, 1920. Liebreich. A. f. O., v, 265, 1859.
Gillan. Brit. Med. J., i, 609, 1939. Moore, Foster. Quart. J. Med., x, 29, 1916.
v. Jaeger. Beit. 2. Path. d. Auges, Wien, 33, Medical Ophthalmology, London, II, 1925.
1856. Shaw. Hyperpiesis and Hyperpiesia, London,
1922.
2. RENAL RETINOPATHY
At the commencement of this section it may be well to summarize our
knowledge of the various types of renal disease which affect the retina. It
used to be taken in a general way that “albuminuric retinitis '' was
associated with albumen in the urine, and, indeed, little headway in an
adequate pathology was possible until a reasonably scientific differentiation
of the many diseases known as nephritis had been made. Much work and
more theorizing has been extended upon this subject during the last 20 years,
but no one can claim that any adequacy has yet been attained. It is now,
however, generally accepted since the work of Volhard (1921–31) that the
pathogenesis of retinal changes depends essentially on hypertension and
27 10 TEXT-BOOK OF OPHTHALMOLOGY
arteriolar constriction, and consequently it is with those forms of renal
disease particularly in which these phenomena occur that retinopathies are
associated.
So far as ophthalmological interests are concerned the diseases known
as “nephritis' may be divided into two separate categories : toxic and
sclerotic. The commonest manifestation of the first form affects the
glomeruli (glomerulo-nephritis) and may be acute, sub-acute or chronic ;
it is only in the more chronic manifestations when vascular sclerosis also
enters into the pathology that ocular complications commonly arise. A
rarer toxic manifestation affects the tubules, and of the various types only
one is of ophthalmological interest : the toacaemia of pregnancy. In the
sclerotic types, the retina, as we have seen, shares in the vascular sclerosis,
but a true retinopathy only occurs when a toxic factor is introduced into
the picture also, as occurs particularly in malignant nephro-sclerosis. On
the whole, therefore, the production of retinopathy requires the combination
of two factors—toacaemia and hypertension with Sclerosis.
1. GLOMERULO-NEPHRITIS, primarily an inflammation of the glomeruli, may be
focal (as caused by infective emboli) or diffuse. The focal form, being unassociated
with hypertension, is not characterized by retinal changes ; but the diffuse form,
having this association, is liable to have ocular complications. The diffuse form
may follow some infection (streptococcal, Scarlet-fever, etc.). In its acute forms the
general toxaemia is occasionally characterized by general vascular constriction, raised
blood-pressure, and oedema, factors which rarely appear in the retina. In its chronic
forms which ultimately terminate in the clinical picture of the small white kidney
(secondary nephro-sclerosis or secondary contracted kidney), the spasmodically con-
stricted arteries undergo the compensatory changes characteristic of diffuse hyperplastic
arteriosclerosis which are complicated in the retina by the appearance of haemorrhages
and exudates, producing the typical picture in the eye of renal retinopathy. It is note-
worthy that the disease is occasionally very prolonged, as in a case reported by Wagener
and Keith (1938) which had been observed for 35 years and developed a temporary
retinitis for a period of 2 months when renal function was progressively decreasing.
2. The NEPHROSEs are characterized essentially by degenerative changes in the
renal tubules. The only type in this category which is associated with hypertension
is the acute nephrosis of pregnancy : this condition is therefore frequently associated
with retinal disease, and since the picture is characteristic, it will be considered
separately." Lipoid nephrosis and amyloid nephrosis are not associated with hyper-
tension and are therefore without ocular complications except on the rarest occasions
(Fishberg and Oppenheimer, 1930).
3. PRIMARY NEPHRO-scLEROSIS, the arteriosclerotic diseases of the kidney, from
the ophthalmological point of view may be divided into three categories:—
i. DIFFUSE (SENILE) ATHEROSCLEROSIS UNCOMPLICATED BY HYPERTEN-
SION OR RENAL INSUFFICIENCY provides no characteristic clinical picture.
In this condition the parenchyma of the kidney is relatively unharmed :
just as the visual acuity and the general functional integrity of the arterio-
sclerotic retina remain good,” so the arterio-sclerotic kidney maintains its
secretory activity, although to a modified degree and with the elimination
of albumen and casts, without endangering the well-being of the body or of
the eye.
1 p. 2723. * p. 2696.
DISEASES OF THE RETINA 27 | 1
ii. DIFFUSE HYPERPLASTIC ARTERIOSCLEROSIS WITH HYPERTENSION BUT WITHOUT
RENAL INSUFFICIENCY : BENIGN OR EssBNTIAL HYPERTENSION. In this
syndrome, wherein the blood-pressure may attain a considerable height and
remain high for years, but is not associated with dangerous vaso-
constriction, ocular changes do occur, but they are limited to those of
arteriosclerosis, the retinal arteries being usually affected along with the
cerebral ; haemorrhages may occur, but there are none of the clinical features
of a true renal retinopathy. If exudative features are present they are
usually associated with other complicating conditions (syphilis, diabetes,
etc.) (Gresser, 1935). The renal function follows parallel lines, the final
stage being the primary contracted kidney, and the patient, sometimes
after a considerable number of years of restricted activity, dies of apoplexy,
coronary thrombosis or cardiac failure. With the absence of angiospastic
features and as a descriptive term of the florid type of individual usually
affected, Volhard (1935) designated this condition red hypertension.
iii. MALIGNANT NEPHRO-SCLEROSIs: MALIGNANT HYPERTENSION. When the
toxaemia which is responsible for the hypertension attains such a degree that
it brings about a generalized contraction of the arterioles, the parenchyma.
of the kidneys, as of the other organs, is damaged and eventually destroyed.
In addition to the sclerotic changes in the arteries, there is added an arterio-
litis and frequently an arteriolo-necrosis (Fahr, 1925; Fishberg and Oppen-
heimer, 1930; Klemperer and Otani, 1931). The results throughout the
whole body are disastrous, and are somewhat similar to those met with in
terminal chronic glomerulo-nephritis, but are usually much more acute.
In the eye the picture of malignant hypertensive retinopathy is characterized
by marked Oedema, masses of cotton-wool patches and extremely con-
stricted vessels ; in the brain a similar state of arterial attenuation and
Oedema leads to a malignant cephalopathy ; and in the kidney complete
insufficiency results in uraemia. In describing this disease Volhard (1935)
employed the term pale or white hypertension. While, therefore, involu-
tionary arterio-sclerosis shows unimportant retinal changes, and essential
hyperpiesia marked sclerotic changes, malignant nephro-sclerosis presents the
characteristic changes of renal retinopathy in their most accentuated form.
In the first case the position may remain stationary for a large number of
years while the patient leads a relatively normal life ; the second condition,
depending on the degree to which the toxaemia can be eradicated or con-
trolled (frequently a problem of the utmost difficulty), may remain stationary
for an indefinite time if the activities are restricted, until eventually the
patient dies a cerebral or cardiac death—alternatively, if it is progressive,
it may pass into the third ; but the third, with the rarest exceptions, is
rapidly fatal and the victim dies of Tenal disease.
The cause of renal retinopathy has excited a great deal of discussion since Richard
Bright (1836) first described visual failure in renal disease. The original idea that
“ albuminuric retinitis '' was correlated in some way with albumen in the urine has
long been discarded. v. Graefe and Schweigger (1863), who were among the earliest
to give an adequate clinical description of the ocular condition, ascribed the retinal
lesions to a generalized disease of the vascular system (arterio-phlebo-sclerosis) which
affected the kidney as well—a view not far removed from modern conceptions of
hypertensive states. This theory received wide credence (v. Michel, 1899; and others),
but although such a process is present as a rule, Sclerotic processes can be shown to be
completely absent initially in acute cases, both on clinical and histological examination
(Treitel, 1876; Opin and Rochon-Duvigneaud, 1903–04; Schieck, 1907). An appeal
2712 TEXT-BOOK OF OPHTHALMOLOGY
was therefore made to chemistry. Some authors contended that the changes are
caused by an accumulation of nitrogenous waste products (azotemic retinitis) (Rochon-
Duvigneaud, 1904; Leber, 1909; Widal, Morax and Weill, 1910 ; Achard, 1924 ;
and others), while Dejean (1932) and Villard, Dejean and Cazals (1933) considered an
increase in toacic polypeptides in the blood aetiologically important. These factors,
however, although perhaps contributory, cannot be essential, since in the absence of
hypertension these substances can accumulate in lethal doses without alterations in
the fundus (Oppenheimer and Fishberg, 1924; Keith, Wagener and Kernohan, 1928;
Terrien and Renard, 1929). A second school associated them with hyper-cholesteroemia
(Chauffard, 1912; Gaudissart, 1922; Chabanier, 1924), a theory, however, which
cannot be sustained in view of the fact that a wealth of exudative-like material may
appear in the retina while the blood-fat is normal, and when it is highest, as in lipoid
nephrosis, the development of a retinopathy is the exception.”
It seems of unusual significance that the two most constant features in all renal
retinopathies are hypertension and attenuated arteries (Elwyn, 1934); and it is especially
noteworthy, as we shall see presently,” that extremely marked retinopathic changes
of the same type may occur in the presence of normally functioning kidneys in states
of malignant hypertension, while in forms of nephritis which run their course without
elevation of the blood-pressure (acute glomerulo-nephritis), normal fundi are the rule.
This school of thought has been expressed most forcibly by Volhard (1921–31) who
maintained that all the retinal changes are due solely to the increase in blood-pressure,
and that the local changes in particular organs, such as the kidneys and the eye,
are not in relation to cause and effect but are collateral effects of the general arterial spasm
and contraction, the parenchyma of the organs being affected through anaemia and
anoxaemia. Volhard (1929), therefore, would call renal retinopathy angiospastic
Yetinitis. Most pathologists, however, would not agree that all the changes are the
result of a simple ischaemia, and would prefer to assume the presence of two factors—
(1) hypertension with ischaemia and subsequent malnutrition together with (2) the effect of
Some toacir or toacins unknown. .
Experimental evidence has not been very valuable in clearing up the question.
Somewhat analogous retinal changes were produced by Ousemblo (1892) by poisoning
dogs and rats by phosphorus, the exudative effect being increased by Sugita (1924)
who employed a high cholesterol diet in addition. Orlandini (1904) used injections of
diphtheria anti-toxin, while Shiba (1908) injected iodine into the kidneys, but obtained
no constant and typical retinal changes except occlema and occasional haemorrhages;
a negative result was obtained also by La Rocca (1934) by the injection of uranium
acetate and specific vascular poisons, and by zur Nedden (1909) and Fusita (1912)
when both kidneys were removed. The nearest approach to simulation of the ocular
pathological conditions were the experiments of Goldblatt and his co-workers (1934–38)
which will be mentioned shortly, who obtained the changes characteristic of hyper-
tensive retinopathy by constricting the main renal arteries in dogs and monkeys.
By the injection of specific nephro-toxins (serums obtained after the repeated
systemic injection of an emulsion of kidney tissue) into the carotid artery, grey streaks
and patches can be produced in the retina as well as proliferative and degenerative
changes in the pigmentary epithelium (zur Nedden, 1909; Yonechi, 1936).
The Incidence of Renal Retinopathy
Although it is denied by some (West, 1916), it is generally agreed that
in the first attack of acute glomerulo-nephritis retinopathy does not occur
1 p. 2710. * p. 2728.
PLATE LII
Vascular RETINopATHIEs
Fig. 2321.-RENAL RETINopATHY, Fig. 23.22.-RENAL RETINopath Y.
Early stage. Late stage.
Fig. 2323.-RETINopATHY of PREGNANCY.
With exudative detachment.
Fig. 2324.—MALIGNANT HYPERTENsive Fig. 2325-MALIGNANT HYPERTENsive
RETINopathy. RETINopATHY.
Early stage. Late stage.
[To face p. 27.12.



DISEASES OF THE RETINA 2713
and that its presence is proof that the disease is of some standing (Herring-
ham, 1912; Bulson, 1912; Slocum, 1916 ; Foster Moore, 1925; Frieden-
wald, 1930; Gresser, 1935; and others). In the remitting sub-acute forms,
however, it does occur, and in the chronic forms it is common ; indeed,
Foster Moore (1925) considers that very few patients die of uraemia without
changes in the fundus.
Thus in 31 acute cases suffering from their first attack of nephritis Gresser (1935)
found that 12.8% had hypertension and 16.1% sclerotic vascular changes in the
retina, 22% had some degree of peri-papillary or retinal Oedema (which in 4 cases was
transient lasting 3–4 days and in 3 was agonal), while exudates appeared in 1 case
Only and that within 24 hours of a uraemic death.
On the other hand, in chronic cases, Gresser (1935) found that hypertensive
vascular changes usually with sclerosis were present in 74%, Sclerosis alone in 12%,
retinal haemorrhages in 56%, exudates in 52%, a star-figure in 10% and disc changes
in 42%. There was thus a definite renal retinopathy in 56% of cases. These figures
correspond with those of others (Miley, 1888, 52 cases in 166 nephritics; Elschnig,
1904, 43 in 199; Nettleship, 1903–06, 31 in 80 ; Herringham, 1912, 26 in 46 ; Foster
Moore, 1925, 34 in 102; Feigenbaum and Rachmilewitz, 1938, 30 in 45 ; and others).
In the latest stages, particularly in young people (small white kidney), the retinal
changes are almost constant in incidence and severe in degree (James, 1913).
TRENCH NEPHRITIS, a toxaemic condition of unknown aetiology which appeared
during the War of 1914–18, was characterized by the development of acute glomerulo-
nephritis. In the initial stages of the disease, when blood-pressure was high and
Oedema general, the fundus was usually normal apart from a slight peri-papillary
Oedema and a few hamorrhages which could be attributable to the general toxic state
(Keith and Thompson, 1918; Horniker, 1921). In the more severe cases, however,
in which resolution did not rapidly follow, more marked retinal changes tended to
appear after 6–10 weeks, and in quite a proportion a definite retinopathy characterized
by hamorrhages, exudates, and massive oedema sometimes developed (Foster Moore,
1915; Jessop, 1916; Hanssen and Knack, 1917; Keith and Thompson, 1918; and
others). In these cases the clinical and functional findings of chronic glomerulo-
nephritis developed and the prognosis was not good.
The age incidence of renal retinopathy lies mainly between 30 and
60 years, but although it does not occur in the aged, a retinopathy may
occur in the very young (5 years, Bull, 1886; 7, Herringham, 1912; 7 and 8,
Nettleship, 1906). Because nephritis occurs much more commonly in males,
the retinopathy is much more common among these than among females,
although in children under the age of 13 years the sex relationship is reversed
(Nettleship, 1906).
Clinical Picture
The clinical picture of a typical case of uncomplicated renal retinopathy
is made up of four characteristics; changes in the vessels, oedema, haemor-
rhages, and exudates. The vascular changes are constant, for the arteries,
particularly the secondary ones which are arteriolar in nature, always show
a distinct attenuation in calibre throughout their whole extent, sometimes to
2714 TEXT-BOOK OF OPHTHALMOLOGY
a degree as to become almost invisible. The other changes are less constant
and irregular in the order of their appearance, but are present in some degree
and at some time (Figs. 2321-22, Plate LII).
Some oedema is probably always present, perhaps in small degree, when
it is evident as a blurring of the margin of the disc and an irregular greyness
and striation of the retina in the peri-papillary region, or as a veiling of the
vessels (Pines, 1931), but at other times the entire retina may become
º,
- - -
º - |- A.
- -
- -
-
- º º º
º
-
º
-
- -
-
-- º
-
- -
-
-
Fig. 2326.-RENAL RETINopathy.
Death from chronic interstitial nephritis. A. Nerve-fibre layer. B. Ganglion
cells. C. Rods and cones. Note the large fat-containing bladder cells stained with
osmic acid (Foster Moore, Medical Ophthalmology).
diffusely oedematous and even suffer an exudative detachment. Haemor-
rhages practically always appear, usually small in size, striated and flame-
shaped since they lie superficially, and preponderatingly near the larger
vessels and close to the disc. They may, however, occur in any part of the
retina and sometimes they are deeply situated, being round or punctiform in
shape.
White exudative-like masses are almost constantly seen ; they are
diverse in appearance and in origin. In the more acute toxic cases the
“cotton-wool ’’ patches are the most characteristic–soft masses of irregular









DISEASES OF THE RETINA 2715
shape, greyish-white appearance with fluffy margins. They lie within
oedematous areas with a preference for the central region, particularly
near the disc ; they may lie underneath the vessels or may cover them
over and obscure them, and they are occasionally associated with
haemorrhages. At first they are small and few, but in some cases they may
increase enormously in size, becoming confluent and piling themselves up
in masses, sometimes extending as a solid sheet all round the disc. If the
patient survives they gradually become absorbed, and finally fade completely
.
-º-,
Fig. 23.27.-RENAL RETINopATHY.
Section near the macula : advanced stage. Henle’s layer (B) is entirely occupied
by bladder cells and the rods and cones (A) have degenerated (Foster Moore, Medical
Ophthalmology).
away leaving behind no trace. A second equally frequent feature is the
occurrence of sharply defined white spots. They may be round or irregularly
shaped, of pin-point size or about half-a-disc in diameter, but they always
lie deeply in the retina underneath the vessels. They may be widely and
irregularly scattered, but occur most frequently between the macula and
the disc. Most characteristically they are arranged in a radiating star-
figure round the macula, where their appearance is preceded by fine radiating
lines suggesting oedema. The rays of the star may be made up of discrete
dots, or these may become confluent, while in advanced cases a solid
pyramid of exudative material may be piled up (Figs. 2322, Plate LII;

2716 TEXT-BOOK OF OPHTHALMOLOGY
2328-29). When the star-figure is incomplete—as it frequently is—and is
represented by a quarter or a half, the fan-shaped segment is usually turned
towards the disc. In general the oedema and cotton-wool patches are
Fig. 2328–RENAL RETINopathy : STAR Figure Air Macula.
Note the exudative detachment (Koyanagi).
characteristic of an acute intoxication, while the more discrete patches and
the star-figure are associated with a more chronic process.
Variations in this typical picture constantly occur. In the milder
--- - - - -
-
º º -
- - - º
º - -
Fig. 2329,-RENAL RETINopathy : STAR Figure at Macula.
High magnification of Fig. 2328. Note partial disappearance of outer nuclear
layer (Koyanagi).
cases, no cedema may be detected clinically, and narrowed arteries and one
or two soft patches alone indicate the existence of disease. In chronic cases
all that may be seen are the attenuated arteries and one or two discrete
white dots. In other cases a papilloedema may simulate with the greatest


DISEASES OF THE RETINA 27.17
accuracy the clinical expression of a cerebral tumour, with perhaps some
tell-tale exudative spots and an attempt at a macular star (Fig. 2331). On
the other hand advanced arteriosclerosis may complicate the picture, the
vascular changes being more obvious than the retinal, a relationship most
markedly seen when a vessel becomes completely thrombosed, or a large
Fig. 2330–MACULA IN URAEMIA.
Showing cyst-formation and sub-retinal exudate (McDonald).
haemorrhage occurs. Finally, when oedema is marked an accumulation of
fluid underneath the retina may give rise to an exudative detachment. Such
detachments are usually large, globular, and bilateral, situated in the lower
part of the eye. In contra-distinction
to its frequency in pregnancy cases, this
is a relatively rare complication, although
it may occur more frequently than is
supposed as an agonal phenomenon
(Foster Moore, 1916); it is usually
associated with advanced disease and
generalized anasarca and is of bad prog-
nosis ; but if the patient survives
sufficiently long the detachment itself
resolves.
The sub-retinal fluid is usually considered
to be exudative in nature representing a leak
from impaired capillaries and thus corre-
sponding to the general cedema. Koyanagi
(1928–34), however, considered it an active
secretion of the cells of the pigmentary FIG. 23.31.-PAPILLCEDEMA IN
epithelium in the endeavour to eliminate URAEMIA (McDonald, T. O. S.).
toxic substances. His views were based
essentially on the fact that the fluid may have a greater content of albumen than
normal blood which would preclude its origin as a simple transudate.
The retinal changes tend on the whole to pursue a definite evolution.
Individual haemorrhages and exudative patches disappear, the haemorrhages
and cotton-wool patches sometimes after a relatively short course of some
weeks and the discrete hard patches of some months, to be succeeded by


2718 TEXT-BOOK OF OPHTHALMOLOGY
others, so that the general appearance of the fundus tends to change ; but
eventually, if the patient survives, the retinopathy passes its acme and
begins to subside. The veins, up to now engorged and succulently swollen,
become narrower, twist about in scraggy instead of bloated curves, and
become ensheathed with parallel white lines, the arteries become still smaller
and are similarly ensheathed, and the exudates give place to a fine white
powdering showing no regular distribution or star-shaped formation, and
finally pigmentary patches begin to appear. This pigmentation is some-
times irregular, representing a migration of the cells of the pigmentary
epithelium into the retina such as occurs in all low-grade pathological
processes, or it may be composed of small discrete black dots in the periphery
formed by localized areas of proliferation of the pigmentary epithelium into
wart-like growths (Nettleship, 1899; Moore, Evans and Neame, 1922).
As the latter authors described, these localized areas of proliferation of the pig-
mentary epithelium may assume considerable dimensions and invaginate the retinal
substance pushing the tissues upwards in front of them (Fig. 2340).
These regressive changes tend to occur after a time, despite the general
condition of the patient or the state of his kidneys, independently of whether
he is showing a temporary recovery or getting progressively worse. A most
interesting feature is that it would appear that once having occurred, the
retinopathy does not recur even although the patient lives for a number of
years and suffers several intermissions and finally dies in uraemia. Not only
does the atrophic retina not respond a second time in this way, but a retina
which has suffered antecedent disease of any kind is frequently immune ;
thus the presence of an old thrombosed vein or obstructed artery or of
advanced myopic atrophy in one eye has determined the most unusual
phenomenon of a unilateral renal retinopathy in the other (Nettleship, 1903;
Foster Moore, 1925; Kotsuka, 1932).
Pathology
Since the early investigations of Türck (1850) and Virchow (1855), a vast amount
of research has been done on the pathology of the condition. The most important
papers are those of Theodor (1887), Nordensen (1887), Weeks (1890), Nuel (1895),
Römer (1901), Litten (1903), Opin and Rochon-Duvigneaud (1903), Leber (1909),
Lauber and Adamūk (1909), Rachlis (1910), Ginsberg (1912), Rochon-Duvigneaud
(1912), Foster Moore (1925), Lo Cascio (1926), Verway (1927), Koyanagi (1928),
Wölfflin (1934), and others. The state of the vessels has been especially investigated
by Theodor (1887), Schieck (1907–29), Verway (1927), Kyrieleis (1930), and others :
in early cases they may be normal but in chronic cases they develop, frequently to an
extreme degree, the changes characteristic of Sclerosis with abundant hyaline and
fatty degeneration and obliterative endarteritis.
The pathology of the retinal changes shows a complete absence of
inflammatory evidences. They are usually more extensive than the
ophthalmoscopical appearance would indicate and depend on two funda-
FIGs. 2332–37.-RENAL RETINopATHY: RETINAL CHANGEs.
- - - -
-
- - -
- º
-- --- - -
-- - - --~~~~
- --
- --- - -
- | --- º º
º - * * º º º
º
- ---
- -- - - - - - -
Fig. 2332,-Varicose degeneration of º - - .. -
nerve fibres (Koyanagi). -
Fig. 2333.-Fibrinous exudate in inter-nuclear
layer with so-called ‘‘ basket" formations
(Koyanagi).
Fig. 2335. Accumulation of exudative
material into masses in the inter-nuclear
layer (Koyanagi).
* - ſº
. -
|- ----
º-º-º-º-º-º-º-º-º:
º
-- ~~~~ º
Fig. 2336.-Albuminous masses in inter- Fig. 2337.-Cystic spaces in inter-nuclear layer
nuclear layer (Koyanagi). with bladder cells undergoing fatty degenera-
tion (Koyanagi).
T.O.-WOL. III, s S.









2720 TEXT-BOOK OF OPHTHALMOLOGY
mental phenomena ; the exudation of plasma into the tissues, and degenera-
tion caused presumably partly by toxaemia and partly by undernourishment
and anoxaemia owing to the arterial constriction and the impaired capillary
circulation. In consequence of the arterial and arteriolar constriction the
capillaries dilate since their walls do not receive a sufficient amount of
oxygenated fluid ; their permeability therefore increases, first to the passage
of fluid to form an oedema, then fibrin-rich plasma, and finally of formed
elements which appear as haemorrhages (the peristatic condition of Ricker,
1927). It is this diffuse deposition of fibrin to form a dense network
throughout the retina which in the relatively loose tissues of the inner
layers accumulates in quantity to constitute the soft cotton-wool patches,
while the diffuse oedema rucks up the internal limiting membrane and
may even cause a sub-retinal effusion, which is frequently, but not
invariably, agonal. The haemorrhages are mainly capillary and are found
in all layers, but particularly the nerve-fibre layer and the inter-nuclear
layer (Figs. 2326–27).
The nerve fibres of the inner layer usually show profound changes,
including oedema and varicose swelling (Fig. 2332), while complete disintegra-
tion with the deposition of fatty granules may result. In the deeper layers
the exudative elements are more closely confined and usually become
aggregated in cyst-like spaces, particularly in the inter-nuclear plexiform
layer. Hyaline changes then occur associated with the deposition of lipoid
material in quantity both in the neural and glial tissues, a process we have
already seen to occur readily in the retina in all degenerative and mal-
nutritional states' (Figs. 2333–37); and as is usual in these circumstances, the
retina, particularly the inter-nuclear plexiform layer, becomes loaded with
phagocytic bladder cells derived from the pigmentary epithelium (Fig.2338).”
It is these hyaline and lipoid deposits arranged in groups along the radiating
fibres of Henle which form the typical macular star. In the deeper layers
there are thus zones showing amorphous deposits rich in fat and lipoids,
zones where the nerve-tissue is destroyed, and zones where all the structure
has disappeared and an empty cavity filled with amorphous coagula remains.
The white patches seen ophthalmoscopically may therefore have a varying
pathological constitution ; they may represent areas of Oedema, masses
of fibrin, absorbing haemorrhages, varicose nerve-fibres forming cystoid
bodies, fatty deposits, islands of necrosis or of hyaline accumulations. If the
patient survives the general degeneration reaches a high grade, and in the
late atrophic stages the normal tissue becomes shrunken and is largely
replaced by connective tissue.
The symptoms of renal retinopathy may be relatively few, and provided
the fovea is spared—as it frequently is even in the presence of a macular
star—the condition may only be discovered accidentally in a relatively late
stage. On the other hand the subjective phenomena may be distressing,
1 p. 2628. * p. 2631.
DISEASES OF THE RETINA 2721
the patient seeing imaginary objects in coloured patterns and coloured
lights. In the later stages, as would be expected, the visual disability may
be profound.
Prognosis. It has long been recognized that the prognosis of renal
**º-º-º-º-º:
Fig. 2338–Migration of pigmentary Fig. 2339–Proliferation of pigmentary
epithelium as bladder cells (Koyanagi). epithelium (Koyanagi).
Figs. 2338 39.-RENAL RETINopathy : CHANGEs IN THE Pigmentary EPITHELIUM.
Fig. 2340-RENAL RETINopathy.
A pigmentary mass in the retina (Foster Moore, Brit. J. O.).
retinopathy is bad, and it was pointed out by early observers that only a
small minority of patients lived longer than two years after its development
(Bull, 1886; Miley, 1888; Snell, 1888; Belt, 1895; Nettleship, 1903; and
S S >


2722
TEXT-BOOK OF OPHTHALMOLOGY
others); time has in no way modified this opinion.
Belt’s (1895) statistics
are typical ; of 419 patients 6% lived longer than 2 years and 65% died
within a year.
Exceptions, however, have occurred (6 years, Bull, 1886;
9 years, Haehnle, 1897; 10 years, Ormond, 1913; 7+ years after a retinitis
so severe as to cause a bilateral retinal detachment, Moore, Evans and
Neame, 1922).
Recovery has been reported even in children after the more
acute forms of nephritis (Lawson, 1907–08).
The treatment will be discussed subsequently."
Achard. Le système lacunaire, Paris, 1924.
Belt. J. Am. Med. As., xxv, 735, 1895.
Bright. Guy's Hosp. Rep., i, 356, 1836.
Bull. T. Am. O. S., iv., 184, 1886.
Bulson. J. Amer. Med. As., lix, 1032, 1912.
Chabanier et alia. Presse méd., xxxii, l 13,
1924.
Chauffard et alia. C. R. S. Biol., lxxiii, 283,
1912.
Dejean. A. d’O., xlix, 446, 1932.
Elschnig. Wien. med. W., liv, 494, 1904.
Elwyn. Nephritis, N.Y., 1926.
A. of O., xi, 300, 1934.
Fahr. Hb. sp. path. Anat. Hist., vi (1), 156,
1925.
Feigenbaum and Rachmilewitz. XV Internat.
Cong. Oph., Cairo, ii, 28, 1938.
Fishberg and Oppenheimer. A. Int. Med.,
xlvi, 901, 1930.
Friedenwald. T. O. S., i, 452, 1930.
Fusita. Nippon Gank. Zashi, 1912:
K. M. Aug., li (1), 746, 1912.
Gaudissart. Am. J. O., v, l 18, 1922.
Ginsberg. A. f. O., lxxxii, 1, 1912.
Goldblatt et alia. J. Eacp. Med., lix, 347,
1934; lxv, 233, 671 ; Irvi, 527, 1937;
lxxvi, 809, 1938.
An. Int. Med., xi, 69, 1937.
A. of O., xvii, 1040, 1937; xx, 812, 1938.
v. Graefe and Schweigger. An. d’Oc., xlix,
136, 1863.
Gresser. Am. J. O., xix, 426, 1935.
Haehnle. Diss., Tübingen, 1897.
Hanssen and Knack. K. M. Aug., lix, 263,
1917.
Herringham. Kidney Diseases, London, 1912.
Horniker. A. f. O., cv, 104, 1921.
James. O. Rev., xxxii, 164, 1913.
Jessop. T. O. S., xxxv, 1, 1916.
Keith and Thompson. Quart. J. Med., xi,
229, 1918.
Keith, Wagener and Kernohan. A. Int. Med.,
xli, 141, 1928.
Klemperer and Otani.
1931.
Kotsuka. Acta S. O. Jap., xxxvi, 54, 1932.
Koyanagi. K. M. Aug., lxxx, 436, 437, 1928.
A. f. O., cxxxii, 353, 1934.
Acta S. O. Japan, xxxviii, 78, 1934.
ISyrieleis. A. f. Aug., ciii, 161, 1930.
La Rocca. A. of O., xii, 509, 1934.
Ref.
A. of Path., xi, 60,
Lauber and Adamūk. A. f. O., lxxi, 429,
1909.
Lawson. T. O. S., xxvii, l 15, 1907; xxviii,
163, 1908.
Leber. A. f. O., lxx, 200, 1909.
Litten. Münch. med. W., i, 361, 1903.
Lo Cascio. Am. di Ott., liv, 3, 129, 1926.
v. Michel. Z. f. Aug., ii, 1, 1899.
Miley. T. O. S., viii, 132, 1888.
Moore, Evans and Neame. Brit. J. O., vi,
193, 1922.
Moore, Foster. T. O. S., xxxv, 159, 1915.
Lancet, ii, 1348, 1915.
R. L. O. H. Rep., xx, 262, 1916.
Medical Ophthalmology, London, II, 1925.
zur Nedden. A. f. Aug., lxiii, 217, 1909.
Nettleship. T. O. S., xix, 63, 1899.
R. L. O. H. Rep., xv, 320, 1903; xvi, l,
1906.
Nordenson. Die Netzhautablósung, 1887.
Nuel. A. d’O., xv, 593, 1895.
Opin and Rochon-Duvigneaud. J. de phys.
et path. gén., v, 1081, 1903.
A. d’O., xxiv, 155, 1904.
Oppenheimer and Fishberg. A. Imt. Med.,
xxxiv, 631, 1924.
Orlandini. Am. di Ott., xxxiii, 561, 1904.
Ormond. T. O. S., xxxiii, 90, 1913.
Ousemblo. Thesis, St. Petersburg, 1892.
Pines. Brit. J. O., xv, 75, 129, 1931.
Rachlis. K. M. Aug., xlviii (2), 322, 1910.
Ricker. Sklerose u. Hypertonie d. innervierten.
arterien, Berlin, 1927.
Rochon-Duvigneaud. An. d’Oc., czzxi, 485,
1904.
Bull. S. fr. d’O., xxix, 1, 1912.
Römer. A. f. O., lii, 514, 1901.
Schieck. B. O. G. Heidel., xxxiv, 77, 1907.
R. M. Aug., lxvi, 39, 1921.
Zb. ges. O., xxi, 1, 1921.
A. f. Aug., c-ci, 857, 1929.
Shiba. K. M. Aug., xlvi (1), 387, 1908.
Slocum. J. Amer. Med. As., lxvii, 5, 1916.
Snell. T. O. S., viii, 144, 1888.
Sugita. A. f. O., cxv, 260, 1924.
Terrien and Renard. A. d’O., xlvi, 594, 1929.
Theodor. Beit. 2. path. Amat. d. Auges bei
Nieremleiden, Wiesbaden, 1887.
Treitel. A. f. O., xxii (2), 204, 1876.
Türck. Z. d. Gesellsch. Wien. Alertze, 1850.
Verway. K. M. Aug., lxxix, 148, 1927.
1 p. 2735.
DISEASES OF THE RETINA 2723
Villard, Dejean and Cazals. A. Soc. med. Wagener and Keith. XV Internat. Cong. O.,
biol., Montpellier, xiii, 329, 1932; xiv, i, 1, 1938.
86, 1933. Weeks. A. f. Aug., xxi, 54, 1890.
Virchow. Deut. Kl., vii, 35, 1855. West. Lancet, i, 372, 1916.
Volhard. Zb. f. ges. O., v, 470, 1921 ; xxi, Widal, Morax and Weill. An. d’Oc., czliii,
129, 1929. 354, 1910.
Hb. d. inneren Med., Berlin, vi, 1931. Wölfflin. K. M. Aug., xciii, 446, 1934.
The Kidney in Health and Disease, Phila., Yonechi. A. f. O., exxxvi, 312, 1936.
1935.
3. TOXAEMIC RETINOPATHY OF PREGNANCY
The ToxAEMIC RETINOPATHY OF PREGNANCY is characterized by its acute
and sudden onset, the presence of marked exudative and haemorrhagic retinal
changes accompanied by an Oedema so massive that an extensive globular
detachment frequently results, its equally rapid restitution on the removal
of the cause, and its relatively good prognosis both as to vision and to life.
Although the cause of the toxaemia is unknown, whether it is associated with
placental products (Fisher, 1915) or the general tissue-metabolism (Zangemeister,
1926), the occurrence of such changes in association with an acute nephrosis has long
been known (v. Graefe, 1855), but nevertheless it is a relatively uncommon condition.
The older statistics suggested an incidence of about 1 in 3,000 pregnancies (Silex,
1895), and individual observers wrote as having seen relatively few cases (36 cases,
Culbertson, 1894; 35 cases, Silex, 1895; 22 cases, Nettleship, 1903). In subsequent
statistics these figures have been increased : thus Schiótz (1921) in 8,400 hospitalized
pregnancies found 680 intoxications with albuminuria, of which 35 had fundus changes
and 3 had retinal detachments. Among 134 eclamptics Lindgren (1921) met 14 cases
with eye changes. Of late years, however, owing to the recognition of the importance
of the pre-organic changes of angiospasm and hypertension, the number of cases coming
under this category has again increased (Stewens, 1930; Friedenwald, 1932; Wagener,
1933; Masters, 1933; Bruce, 1933; Vandegrift, 1933; du Toit, 1935; Hallum,
1936; Gibson, 1938; Schultz and O’Brien, 1938).
Thus Gibson (1938), in 39 cases of hypertensive toxaemia of pregnancy, found 5
with normal fundi, 23 with pre-organic spastic changes, and 11 with organic retinopathy.
Similarly Schultz and O’Brien (1938), in 47 patients, found the retina normal in 9
(19%), arteriolar spasm in 13, vascular sclerosis in 12, retinopathy in 12, while 3 had
small central retinal detachments.
The disease may occur at any age of the child-bearing period, either
during a first pregnancy or in multipara ; Nettleship (1903) found that the
majority of cases occurred after three of four previous confinements. It
rarely occurs before the 6th month of the pregnancy (3rd month, Brecht,
1872; Schultz and O’Brien, 1938, in a hydatidiform mole), and about 90%
of cases develop during the 9th. month. A large number have shown no
vascular or renal disease prior to the toxaemic manifestations, while in others
vascular sclerosis and nephro-sclerosis has been previously present, so that
an already established condition has been aggravated by child-bearing
(Nettleship, 1903; Stewens, 1930). In all cases, however, hypertension is
present, and it may be taken as a general rule that the retinal changes are
liable to occur when the systolic pressure rises above 150 and the diastolic
2724 TEXT-BOOK OF OPHTHALMOLOGY
above 100 mm. Hg, and are marked when these limits reach 200/130. In
general the retinal changes run parallel with the Severity of the hypertension and
therefore of the toxaemia; they do not depend on the degree of renal involve-
ment (for this is a collateral and not a causative change), and they may occur
in the presence of severe constitutional symptoms although the urine is
normal (Posey and Hirst, 1908; Semple, 1909–11).
The clinical course of the disease in the more acute cases may be divided
into three stages; the spastic stage of arterial irritation during which the
toxin excites angiospastic phenomena; the stage of sclerosis when organic
changes appear in the vessels; and the stage of retinopathy when oedema,
haemorrhages and the destruction of tissue occur. Complete recovery usually
follows the institution of adequate treatment during the first pre-organic
stage, but once organic changes have established themselves, the blood-
pressure tends to remain permanently high and there is some degree of
nephro-sclerosis. It may be taken that 60% of the cases fall into the first
category and 40% into the second. In the less acute cases between the
second and the third stage there may be a long latent phase lasting for
several years after the termination of the pregnancy which is entirely
symptomless except perhaps for a slight elevation of the blood-pressure.
During this time, however, vascular sclerosis becomes slowly and pro-
gressively more advanced, until, when it is so severe as to embarrass the
various vital organs so that their function becomes inadequate, the patient
passes to the terminal stage. The symptomatology of this stage depends
upon which system is predominantly affected by the diffuse vascular
disease ; the condition may simulate malignant hypertension, terminal
chronic nephro-sclerosis, or hypertensive encephalopathy, and may terminate
in either a cerebral, renal or cardiac death.
The first visible ocular sign of the toxaemia of pregnancy is usually an
attenuation of the retinal arterioles with a hardening of their reflex associated
with a rise in diastolic pressure, a fact which makes routine ophthalmoscopic
examination of such patients of primary importance. This attenuation
usually occurs first in the nasal periphery but gradually spreads towards the
disc and becomes generalized ; presumably it is an indication of a similar
widespread condition throughout the body. Once it has developed it usually
persists until the pregnancy is terminated. Quite frequently it is accen-
tuated by angiospasm, so that local constrictions appear, and at any part of
the fundus fleeting spasms may occur so that the column of blood may be
practically or quite indistinguishable, a phenomenon which has been photo-
graphed (Mylius, 1928). If this spastic condition is extreme and persistent
it may bring about obscurations of vision, and eventually give rise to
Oedematous patches in the retina in the neighbourhood of the spastic vessel.
It is frequently difficult to say when the functional tonic spasm passes
into Organic sclerosis, but eventually the latter change becomes obvious, to
be followed in more severe cases by the appearance of oedema, exudates
DISEASES OF THE RETINA 27:25
and haemorrhages which make up the clinical picture of renal or hyper-
tensive retinopathy. CEdema and exudation are sometimes very marked,
and while frequently resulting in the formation of a macular star or a flat
macular detachment (Schultz and O’Brien, 1938), may produce a large
globular retinal detachment (Fig. 2323, Plate LII).
A retinal detachment complicating the retinopathy of the toxaemia of
pregnancy is not very common, but since the first case was reported by
v. Graefe (1855) a considerable number have appeared in the literature.
Thus Helbron (1902) collected records of 21 cases, Schiótz (1921) 50 cases,
and Fry (1929) 57 cases. Individual authors have observed quite a number:
Foster Moore (1915) 5 cases, Clapp (1919) 6, and Schiótz (1919–21)7. The
detachment is usually bilateral (15 out of 27 cases, Fry, 1929), and Hill
(1924) reported bilateral detachments in two successive pregnancies. Fry's
Fig. 2341.-Tox AEMIC RETINopATHY or PREGNANCY.
A case of eclampsia. Retina detached by sub-retinal exudate (Koyanagi).
(1929) statistics showed that it occurs 1-2% of cases of late toxaemia and
10.4% of cases of eclampsia. The condition is always associated with a
generalized oedema, frequently marked on the face and lids, and, indeed,
is its localized expression in the eye, being dependent on the diseased condi-
tion of the vessels. It is always associated with marked retinopathic
changes, but it is probable that part of the exudative fluid is derived from
the choroid as well as the retina (Gourfein-Welt, 1904; Verderame, 1911)
(Fig. 2341). The prognosis for re-attachment is good, for this usually occurs
spontaneously, sometimes in a few days; but the prognosis with regard to
vision depends upon the residual changes left by the accompanying
retinopathy.
It may be said definitely that a woman who develops signs of retinal
changes during child-bearing is dangerously ill, the more so if established
sclerotic changes have antedated the pregnancy (Duggan and Chitnis, 1937).

2726 TEXT-BOOK OF OPHTHALMOLOGY
If the changes are confined to arterial attenuation and she is well within the
pre-organic stage of her toxaemia, she will probably respond to adequate
conservative treatment, and the pregnancy may justifiably be continued,
although under the closest supervision. If acute angiospastic phenomena,
however, become evident, a more grave view must be taken of the case, but
if vascular sclerosis and retinopathy develop the pregnancy should be
terminated ; its continuation involves the risk of permanent and perhaps
ultimately fatal damage to the vascular and visual systems of the mother
and the likelihood of a foetal fatality. Its termination, however, in the
majority of cases which are not complicated by an already established
chronic nephrosclerosis, is usually followed by happy results, in early cases
with a rapid resolution of the retinal changes and a liberal and frequently
complete restitution of visual acuity, and in the later cases with impaired but
useful vision. A certain degree of optic atrophy or permanent macular
changes remain as a lasting disability in more than 50% in this category,
and occasionally the visual disability is progressive (Culbertson, 1894;
Silex, 1895; and others). The prognosis with regard to life is also relatively
good ; in Nettleship's (1903) 22 cases only 1 died within 2 years and 1 was
alive 24 years later. The prognosis is therefore very much less grave than in
renal retinopathy ; nor, if it has once been present and has subsided, is the
likelihood of recurrences in future pregnancies very great, provided relief
has been obtained before permanent damage to the vascular system has
been done.
Brecht. A. f. O., xviii (2), 102, 1872.
Bruce. Am. Med. As. Sect. O., xxxix, 6, 1933.
Clapp. Am. J. O., ii, 473, 1919.
Culbertson. Am. J. O., xi, 133, 197, 1894.
Duggan and Chitnis. Brit. J. O., xxi, 585,
1937.
Fisher. P. R. S. Med., Sect. O., viii, 127, 1915.
Friedenwald. Path. of Albuminuric Retinitis,
Libman Anniv. Vols., N.Y., ii, 453, 1932.
Fry. A. of O., i, 609, 1929.
Gibson. Am. J. O., xxi, 22, 1938.
Gourfein-Welt. X Internat. Cong. O., 33, 1904.
v. Graefe. A. f. O., ii, 202, 1855.
|Hallum. J. Am. Med. As., cvi, 1649, 1936.
Helbron. Berlin. kl. W., xxxix, 68, 103, 1902.
Hill. A. of O., liii, 137, 1924.
Lindgren. A. f. O., cv, 286, 1921.
Masters. T. Am. O. S., xxxi, 416, 1933.
Moore, Foster. Lancet, ii, 1348, 1915.
T. O. S., xxxv, 159, 1915.
R. L. O. H. Rep., xx, 262, 1917.
Mylius. B. O. G. Heidel.., xlvii, 379, 1928.
Funktionelle Veránderungen am Gefässytem
de Netzhaut, Berlin, 1928.
Nettleship. R. L. O. H. Rep., xv, 320, 1903.
Bosey and Hirst. J. Am. Med. As., i, 865,
1908.
Schiótz. K. M. Aug., lxii, 234, 1919; lvii,
Beil., 1, 1921.
Schultz and O’Brien.
1938.
Semple. T. Am. O. S., xii, 817, 1909–11.
Silex. Berlin. kl. W., xxxii, 385, 1895.
Stewens. Z. f. Aug., lxxii, 293, 1930.
du Toit. S. African Med. J., ix, 559, 1935.
Am. J. O., xxi, 767,
Vandegrift. Am. Med. As. Sect. O., xxxix,
8, 1933.
Verderame. K. M. Aug., xlix (1), 452, 1911.
Wagener. J. Am. Med. As., ci, 1380, 1933.
Zangemeister. Die Lehre d. v. Eclampsie,
Leipzig, 1926.
4. MALIGNANT HYPERTENSIVE RETINOPATHY
A characteristic retinal picture has been described in cases of malignant
hypertension, not necessarily accompanied by nephro-sclerosis of any
detectable degree unless as a terminal event (Wagener, 1923–27 ; Kahler
DISEASES OF THE RETINA 27.27
and Sallmann, 1925; Keith, Wagener and Kernohan, 1928; Moschcowitz,
1929; Fishberg and Oppenheimer, 1930; Keith, Barker and Kernohan,
1931; Scott, Seecof and Hill, 1933; Moritz and Oedt, 1935; Cohen, 1937;
Wagener and Keith, 1938; and others). It is differentiated from the typical
renal retinopathy resulting from glomerulo-nephritis essentially by the
extreme papillary and peri-papillary oedema, the picture produced being
frequently almost indistinguishable from that of the papilloedema resulting
from a brain tumour; the swelling, indeed, may be as much as 6 dioptres
(Larsson, 1924) (Fig. 2343). It has been suggested that increased intra-
cranial pressure owing to cerebral oedema is responsible for this feature, but,
Fig. 2342. –HYPERTENsive RETINoPATHY,
A shallow exudative retinal detachment; haemorrhages between the internal
and external molecular layers. Degeneration of pigmentary epithelium ; and
choroidal degeneration with almost complete obliteration of the vessels (Tooke).
although a rise of the intra-cranial pressure sometimes to an enormous
extent may be an associated phenomenon (Cushing and Bordley, 1908;
Larsson, 1924; Merkulow, 1937), the fact that in other as dramatic cases
this pressure is within normal limits (Fishberg and Oppenheimer, 1930)
indicates that the two are collateral phenomena unrelated as cause and
effect. This papilloedema is of special prognostic and diagnostic import.
Although it is characteristic of hypertensive states, and particularly of the
malignant hypertension of young adults, a similar picture occurs as a terminal stage in
association with glomerulo-nephritis, and occasionally in the absence of renal involve-
ment, as in lead poisoning (Kahler and Sallmann, 1925; Nye, 1929), or in suprarenal
tumours when the renal function is normal (Oppenheimer and Fishberg, 1924; Winkel,
1928).

27:28 TEXT-BOOK OF OPHTHALMOLOGY
-
Sometimes the retinal changes appear acutely in a relatively normal
fundus in toxic states of recent inception, especially in young persons;
but usually they are associated with the narrow attenuated arteries
characteristic of hypertension of some duration, and the actual incidence
of the retinopathy may be preceded by evidences of angiospasm. The
clinical picture itself is characterized by marked arterial constriction,
hyperaemia and massive oedema of the dise, profuse superficial haemorrhages
and soft cotton-wool exudates. The exudates may be large and extremely
numerous, piling themselves up in heaps to form an extensive peri-papillary
ring or a pronounced macular star, or to cover the greater part of the central
area of the retina in great soft-contoured “woolly” masses which later on may
“harden” and become more sharply delineated (Figs. 2324–25, Plate LII).
-
- -
--- - - -
º sº º
- - - º
- -
---
º:
- º
- - -------
º
Fig. 23.43.-HYPERTENsive RETINopathy.
(Edema of disc with dilatation of central vein and extravasations of leucocytes
along vessel walls (Tooke).
Pathologically an actual arteriolo-necrosis has been observed with large areas
of necrosis appearing in the vessel walls (Cohen, 1940). The oedematous and
exudative changes may spread slowly from the disc to the macula and the
periphery and then recede leaving pigmentary proliferation. The whole
picture suggests the nature of the case—a condition of acute and perilous
toxaemia—andisassociated with symptoms of violent headache and vomiting;
ultimately it usually terminates in malignant nephro-sclerosis and at this
stage the fundus in the two conditions is usually identical.
The picture of malignant hypertensive retinitis is generally considered
to be a death-warrant, for after its development few patients live for two
years, and the majority much less. There are, however, exceptions: thus
Kahler and Sallmann (1925) observed 8 cases over long periods in which renal
failure did not develop, and partial or complete recession has been noted by













DISEASES OF THE RETINA 2729
them and by others (Schieck, 1928; Fishberg and Oppenheimer, 1930;
Floyd, 1931). These cases, however, are the exception.
Experimentally a state of hypertension can be induced in animals by section of
the nerves of Hering and Cryon to the carotid sinus (Weeks and Dautrebande, 1933)
Or more effectively by constriction of the main renal arteries (Goldblatt and others,
1934–38). In the latter case these animals show a benign phase characterized by a
slowly progressive vascular sclerosis with occasional small haemorrhages and Oedema,
and eventually a malignant phase with severe degenerative changes in the vessels with
massive papilloedema, oedema of the retina and its detachment by a massive sub-
retinal Sero-sanguineous exudation (Keyes and Goldblatt, 1937–38).
The general treatment of the condition will be considered subsequently.”
Cohen. A. of O., xvii, 994, 1937; xxiii, 1052, Moore (contal.). Quart. J. Med., x, 29, 1916.
1940.
Cushing and Bordley.
cxxxvi, 484, 1908.
Fishberg and Oppenheimer.
xlvi, 901, 1930.
Floyd. A. of O., vi, 433, 1931.
Goldblatt et alia. J. Eacp. Med., lix, 347,
1934 ; likv, 233, 671, 1937; lxxvi, 809,
1938.
A. Int. Med., x1, 69, 1937.
Kahler and Sallmann. Z. f. Aug., lvii, 386,
1925.
Keith, Barker and Kernohan.
Phys., xlvi, 66, 1931.
Keith, Wagener and Kernohan. A. Int. Med.,
xli, 141, 1928.
Reyes and Goldblatt. A. of O., xvii, 1040,
1937; xx, 812, 1938.
Larsson. Acta O., i, 193, 1924.
Merkulow. Acta O., xv, 406, 1937.
Moore, Foster. T. O. S., xxxv, 159, 1915;
xxxvi, 319, 1916.
Am. J. Med. Sc.,
A. Int. Med.,
T. Ass. A m.
5. DIABETIC
P. R. S. Med., xvi, 5, 1923.
Medical Ophthalmology, London II, 1925.
Moritz and Oedt. Am. J. Path..., xi, 885, 1935.
Moschcowitz. Am. J. Med. Sc., clzxviii, 244,
1929.
Nye. Med. J. Australia, ii, 145, 1929.
Oppenheimer and Fishberg. A. Int. Med.,
xxxiv, 631, 1924.
Schieck. Hb. sp. path. Amat. Histol., xi (1),
518, 1928.
Scott, Seecof and Hill.
xlviii, 283, 1933.
Volhard. Zb. f. ges. O., xxi, 129, 1929.
Wagener. Med. Clin. of N. Amer., vii, 275,
1923.
A. m. J. O., vii, 272, 1924.
T. Am. O. S., xxv, 349, 1927.
Wagener and Keith. XV Internat. Cong. O.,
Cairo, i, 1, 1938.
Weeks and Dautrebande.
1933.
Winkel.
T. Ass. A m. Phys.,
A. d’O., i, 786,
Dewt. A. f. kl. Med., clix, 1, 1928.
RETINOPATHY
The question as to whether a true diabetic retinopathy exists has
excited a great deal of controversy and the matter cannot be said yet to be
settled. The existence of specific changes in the fundi of diabetics was first
described by Jaeger (1856) and the various manifestations were fully
elaborated by Hirschberg (1890–91) who recognized three clinical types :—
1. An “inflammation * of the retina characterized by spots and
haemorrhages : central punctate retinitis.
2. Retinal haemorrhages.
3. Pigmentary degeneration.
Since the publication of his classical papers ophthalmological opinion
has been divided into two schools, one of which believes that these changes
are specific while the other denies it. The arguments of the first school may
be briefly stated thus (Lagrange, 1887; Hirschberg, 1891 : Nettleship,
1 p. 2735.
2730 TEXT-BOOK OF OPHTHALMOLOGY
1889–1904; Foster Moore, 1920; Friedenwald, 1925; Dabney, 1926–30;
Gifford, 1928; Folk and Soskin, 1935; and others).
1. A clinical picture exists which, while not pathognomonic for diabetes,
has certain features distinguishing it from arteriosclerotic or renal
retinopathies.
2. Although a high blood-pressure, arteriosclerosis and albumen in the
urine are usually associated with the condition, cases occur in which these
are minimal or absent (Leber, 1875; Mackenzie, 1879; Lawford, 1883;
Anderson, 1889; Hirschberg, 1891; Juler, 1892; Rockliffe, 1894; Nettle-
ship, 1903; Kako, 1903; Altnow, 1927; Lawrence, Madders and Millar,
1930; Gray, 1933; Wagener, Dry and Wilder, 1934; Folk and Suskin,
1935; Braun, 1936).
Some of the more recent and carefully controlled figures are quite high : thus
Gray (1933) found that 49 out of 66 cases of definite retinopathy were consistently
without albumen in the urine, and Folk and Soskin (1935) found that in 15 closely
studied cases with central punctate retinopathy, 9 showed no cardio-vascular com-
plications and 8 had a low blood-pressure.
3. Its frequent unilaterality, its occurrence in old people, its relatively
good vital prognosis and its occasional response to treatment differentiate it
from renal retinopathy.
On the other hand there are arguments for the opposite view.
1. The clinical picture is frequently not typical and transition forms
occur showing all gradations between what is generally accepted as character-
istic of arteriosclerotic retinopathy on the one hand and renal on the other.
2. There is no demonstrable relation between the severity of the
diabetes and the extent of the retinal changes, for they are not seen in young
subjects in whom the diabetes is frequently most accentuated, but only in
older patients usually with a relatively mild degree of the disease, and
frequently complicated by arteriosclerotic or renal changes.
3. It is argued that the clinical absence of albumen may be transitory
or may be associated with advanced pathological changes in the kidneys
(Garrod, 1920), and that the apparent absence of arteriosclerosis may be
histologically refuted (Cohen, 1929)—all these facts have led others to assume
that the condition is not a disease-entity but represents a type of arterio-
sclerotic retinopathy, modified perhaps by the diabetic diathesis (Hanssen
and Knack, 1917; Garrod, 1920; Wagener and Wilder, 1921 ; Frieden-
wald, 1930; Carreton and Camino, 1932; Gresser, 1933; Dirion, 1933).
Bessiere (1932), on the other hand, believes that its significance is that it is
a premonitory sign of commencing renal failure.
No doubt there is much truth on either side. Diabetic retinopathy
occurs most frequently in the subjects of vascular sclerosis or hyper-
tension, and even when not clinically apparent some vascular damage is
probably usually present ; moreover, a large number of diabetic patients
are the subjects of renal deficiency. Conversely many arteriosclerotics and
DISEASES OF THE RETINA 2731
hypertensives are potential diabetics (O’Hare, 1920), a tendency probably
due to vascular sclerosis in the pancreas. But the fact that these changes
may be minimal, or even in a minority altogether absent, makes it necessary
to assume that some factor of a toxic nature, presumably metabolic in origin,
enters into the question and determines the more frequent and ready
occurrence of retinopathy in diabetics with arteriosclerosis than in uncom-
plicated arteriosclerotics, just as gangrene of the limbs occurs more frequently
in the former than in the latter. Moreover, it would seem obvious that such
a factor could modify the clinical aspect of the case and give it an individuality
of its own. We could indeed go further and say that just as we have seen that
an infective toxic factor alone can produce a very similar clinical picture
(exudative retinitis 1), so there is no impossibility in the suggestion that a
metabolic toxic factor should not act similarly on the minute vessels of the
retina. In this connection it is interesting to recall that a derangement of
the lipoid balance of the blood can produce lesions in the retina similar to
diabetic retinopathy if that tissue is already diseased (Sugita, 1923; Jess,
1925). It is understandable that if this factor is little in evidence and if the
factor of arterial or renal disease is much in evidence, the clinical appearance
in the eye will not conform to type, but will tend to assume the charac-
teristics of one or other of these, and there is no reason why a pure
hypertensive or arteriosclerotic or renal retinopathy should not occur
in a diabetic. Even although the condition is usually associated with
arteriosclerosis, the fact that a parallel can be drawn with renal retinopathy,
which requires the combination of a circulatory disturbance and a toxic
factor for its development, makes it difficult to treat the one as a clinical
entity and the other not, for if diabetic retinopathy is to be called
arteriosclerotic retinopathy occurring in diabetics, so also should the other
be referred to as arteriosclerotic or hypertensive retinopathy occurring in
nephritics. -
It is interesting to speculate how closely diabetic retinopathy and other
nerve lesions may be related causally, for peripheral neuritis (ocular palsies,
etc.) and evidences of central disturbance (loss of the knee jerks, extensor
plantar response, central scotoma) occur also in this disease. These lesions
occur also quite irregularly whether the glycosuria is slight or abundant ;
and certain of them, such as loss of the knee jerks, are frequently associated
with the retinopathy. Some toxic element would appear to be distributed
by the blood which is capable of damaging sometimes one and sometimes
another area of the central nervous system.
Incidence. The published figures of the incidence of retinal lesions in
diabetics vary very widely, from 2 to 30%, a circumstance due in part to
the inclusion of children in some statistics which at once lowers the figure
considerably, and in part to the degree of vascular change recognized by
different authors as constituting a pathological entity.
4 p. 2644.
2732 TEXT-BOOK OF OPHTHALMOLOGY
Some of the figures in the literature are : Kako (1903), 29% ; Noorden (1912),
17% ; Grafe (1923), 13% ; Foster Moore (1925), 10% ; Spalding and Curtis (1927),
5% ; Joslin (1928), 5.2% ; Cammidge (1930), 4.8% ; Shepardson and Crawford
(1931), 23% ; Gresser (1933), 38% (including all vascular changes); Gray (1933),
13.3% ; Wagener, Dry and Wilder (1934), 12.2% (with 17.7% with haemorrhages);
Folk and Soskin (1935), 33% retinopathy of all types including 15% with central
punctate lesions; Goldfelder and Kopelovits (1937), 1.8%. There is no doubt that
pathological changes occur much more frequently in diabetics than in non-diabetics
of a corresponding age-group—33% as against 6% (Folk and Soskin, 1935).
Females are very much more prone to show these changes than males,
and retinopathy affects the older patients only, the commonest age-group
being 50–55 years and the usual extremes 40 to 70. Cases under 40 years
are exceptional.
Exceptional cases are—22 years (Jaeger, 1869 ; Adams, 1932), 26 (Bardsley,
1920), 35 (Nettleship, 1903), 37 (Foster Moore, 1925), 39 (Grafe, 1923), 75 (Adams,
1920).
There is no constant relationship between the duration and severity of
the diabetes and the occurrence or degree of the retinopathy (Russo, 1927).
It is rarely seen, however, unless the diabetes has been in existence for
3 years; Gresser (1933) found an average duration of 7 years, Grafe (1923)
of 10 years, and Lawrence, Madders and Millar (1930) found extremes of
2 and 20 years. The lesions are observed in mild cases as frequently as
severe ones; indeed, they occur not uncommonly in long-standing glycosuria
without general diabetic symptoms which is associated with difficulties
in the hepatic storage of carbohydrates rather than with a pancreatic lesion
(Hawthorne, 1916; MacLean, 1930). They occur whether or not the
diabetes is controlled and may appear when the disease has been adequately
controlled for years, whether or not insulin is used, and whether the carbo-
hydrate of the diet has been unlimited or restricted and replaced by fats.
Clinical Features. Retinopathic features in diabetics may be con-
sidered under three headings : the state of the vessels, the presence of
haemorrhages and of exudates.
All authorities are agreed that in the majority of cases the retinal
vessels show evidences usually of involutionary arteriosclerosis and some-
times of hypertension, and that the incidence and degree of those changes
are more marked in the diabetic than in the non-diabetic. In this respect
the retinal vessels conform with the state of those throughout the body. A
very frequent sign is loss of transparency (Pines, 1929; Gray, 1933), and
although some authors maintain that some degree of sclerosis or raised
blood-pressure is universal (Volhard, 1921 ; Gresser, 1933), a large number
of observers have found the vessels normal and the tension low in a con-
siderable proportion of retinopathic cases (Altnow, 1927; Lawrence,
Madders and Millar, 1930; Adams, 1932; Gray, 1933; Folk and Soskin,
1935; Koyanagi, 1935 ; Mylius, 1936; and others). A prominent feature
is the early engorgement and congestion of the veins.
DISEASES OF THE RETINA 2733
Haemorrhages are an extremely common event, occurring either alone
or in the presence of exudates. Two distinct types occur. Usually they
commence by being small and peripheral and they gradually increase in
numbers, approaching the macular area so that the entire fundus is peppered
with them (Fig. 2319, Plate LI). They are sometimes superficial and flame-
shaped but more usually are deep and round, and are quite frequently situated
away from the large vessels which may show no signs of sclerosis; they
Obviously represent an escape by diapedesis through permeable capillaries
rather than an extrusion of blood by mechanical pressure. When sclerosis
is present in addition, however, and particularly when a septic focus exists
elsewhere in the body, extensive haemorrhages may occur; these probably
result from an infective peri-vasculitis. Massive extravasations appear in
the retina, pre-retinal haemorrhages occur and recur, or the whole vitreous
may be flooded with blood, a catastrophe which is not infrequently followed
by retinitis proliferans and permanent loss of vision. It is especially
noteworthy that these massive haemorrhages usually occur in diabetics whose
condition is fully controlled, and that they are particularly associated with
sepsis and invariably with sclerosis.
It is significant also that there seems to be a consensus of opinion that the harmor-
rhagic diathesis is not aided by insulin treatment, but may even be increased thereby
(Dabney, 1926–30 ; Carter, 1930; Dirion, 1933; Goldfelder and Kopelovits, 1937).
The exudative spots which are characteristic of diabetic retinopathy
usually occur in the central area (central punctate retinitis) (Fig. 2320,
Plate LI). The peculiar features which differentiate them from other
retinopathies are that they are solid, soapy or waxy-looking, with well-
defined, sharply cut edges, and that they are distributed irregularly in the
central area frequently forming a rough circle round the macula ; a macular
star is rare except when renal complications are present, and when it does
occur it is usually partially formed and asymmetrical. The patches are
usually circumscribed but may attain considerable dimensions by accretion;
moreover, like the haemorrhages, they are inert and persistent, remaining
long without visible change. A retinal Oedema is rare, and when it does
occur it appears in slight degree and seems to be incidental to the process,
probably representing a change comparable to the oedema of the epithelium
of the iris ; the optic disc is usually unchanged.
The rarest appearance is that of irregular pigmentation, a phenomenon noted
by Hirschberg (1890–91) which has received little attention in the literature. It
may be peripheral, but it is sometimes particularly evident near the macula
(Butler, 1920).
The main features which differentiate the condition clinically from a
renal retinopathy are the solid, soapy, clear-cut patches in contra-distinction
to the soft woolly masses, their irregular central arrangement without a
1 p. 2383.
2734 TEXT-BOOK OF OPHTHALMOLOGY
macular star, their association with deep and round rather than flame-
shaped haemorrhages, the absence of oedema at the disc or in the retina so
that a retinal detachment never occurs, and the inertness of the whole
picture. The most important single feature is the absence of oedema. Such
applies to a typical case; but few cases are typical, and when sclerotic,
hypertensive or renal complications are added, any gradation may be
encountered from arteriosclerotic retinopathy on the one hand to renal on
the other.
Pathological investigation reveals little peculiar to the condition.
Sclerotic and endarteritic changes are common (Mackenzie, 1879; Strieff,
1898; Hertel, 1901); they may be extreme
(Humelsheim and Leber, 1901 ; Russo, 1927),
or so slight as not to be detectable clinically
(Cohen, 1929). Haemorrhages are typically
small and in the outer layers (Beauvieux
and Pesme, 1923), and the exudates are
albuminous rather than fibrinous and are
usually deeply placed in the plexiform layer
where cystic spaces abound (Foster Moore,
1925; Koyanagi, 1935) and are associated
with fatty deposits (Beauvieux and Pesme,
1923) (Fig. 2344).
The symptoms of diabetic retinopathy
depend almost entirely on the state of the
macula, and if it is unharmed, as it often is
for a very long time, the condition may be
discovered by accident when it has obviously
Fig. 2344–Diabetic Rºtisopathy been present for a considerable period and
Note massive exudative accumula is already in an advanced stage. When the
tions (Koyanagi). macula is involved, however, central vision
is lost, and when angiosclerotic or hypertensive complications are pronounced,
the catastrophe of recurrent and massive haemorrhages present an ever-
present danger of irremediable blindness. In uncomplicated cases its pro-
gress may be exceedingly slow, and, although it is by no means a universal
experience, improvement or even disappearance of the retinal lesions has
been recorded with the introduction and maintenance of efficient anti-
diabetic treatment (Bardsley, 1920; Chauffard, Grigart and Nida, 1925;
Folk and Soskin, 1935). The intensive administration of calcium has been
advocated (Cammidge, 1930), although its value is questionable (Lawrence,
Madders and Millar, 1930); but the elimination of sepsis, sometimes a
difficult problem in these cases, is of great importance. As already mentioned
in haemorrhagic cases insulin should only be given with the greatest care
and under the most expert supervision.
The prognosis of diabetic retinopathy with regard to life, while worse

DISEASES OF THE RETINA 2735
than that of a simple arteriosclerotic condition, is much more favourable
than in renal disease, for many cases have been followed for from 8 to 10 years.
In the presence of arteriosclerosis or hypertension, the outlook is by no
means good both with regard to vision and with regard to life, for death
from arterial disease, as by apoplexy, is common. A great deal depends,
also, on the absence of sepsis elsewhere, and—just as important—on the
co-operation afforded by the patient in the constant and unremitting treat-
ment of a disease which is always irksome and sometimes difficult. As a
general rule the prognosis with regard to vision is bad, for, despite treatment,
the changes tend to be progressive.
sidered immediately.
Adams. T. O. S., x1, 31, 1920.
Brit. J. O., xvi, 38, 1932.
Altnow. A. Int. Med., x1, 757, 1927.
Anderson. O. Rev., viii, 33, 1889.
Bardsley. T. O. S., x1, 43, 1920.
Beauvieux and Pesme. A. d’O., x1, 65, 1923.
Bessiere. A. d’O., xlix, 219, 1932.
Braun. A. f. O., czzxvi, 256, 1936.
Butler. T. O. S., xl, 45, 1920.
Cammidge. P. R. S. Med., xxiii, 292, 1930.
Carreton and Camino. A. de Oft. H.-A.,
xxxii, 185, 1932.
Carter. J. Michigan Med. S., xxix, 365, 1930.
Chauffard, Grigaut and Nida. C. R. S. Biol.,
xcii, 1356, 1925.
Cohen. A. of O., ii, 529, 1929;
1937.
Dabney. Kentucky Med. J., xxiv, 484, 1926;
xxviii, 209, 1930.
Dirion. A. of O., ix, 829, 1933.
Folk and Soskin. Am. J. O., xviii, 432, 1935.
Friedenwald. J. A. m. Med. As., lxxxv, 428,
1925.
T. O. S., i, 452, 1930.
Garrod. T. O. S., x1, 6, 1920.
Gifford. Med. Clin. N. Am..., xii, 423, 1928.
Goldfelder and Kopelovits. Sov. vest. Oft.,
ix, 798, 1937.
Grafe. K. M. Aug., lxix, 841, 1922.
Kl. W., ii, 1216, 1923.
Gray. Brit. J. O., xvii, 577, 1933.
xvii, 994,
Gresser. A m. J. O., xvi, 612, 1933.
Hanssen and Knack. K. M. Aug., lix, 263,
1917.
Hawthorne. T. O. S., xxxvi, 338, 1916 ; xl,
39, 1920. .
Hertel. A. f. O., lii, 191, 1901.
Hirschberg. Deut. med. W., xvi, 1181, 1236,
1890; xvii, 467, 1891.
Cb. pr. Aug., xv., 18, 68, 1891.
Humelsheim and Leber. A. f. O., lii, 336,
1901.
The Treatment of the
The general treatment will be con-
Jaeger. Beit. z. Path. d. Auges, Wien, 33,
1856
Oph. Hand-Atlas, Wien, 1869.
Jess. B. O. G. Heidel., xlv., 212, 1925.
Joslin. The Treatment of Diabetes Mellitus,
Phila., VIII, 1928.
Juler. T. O. S., xii, 137, 1892.
Kako. K. M. Aug., xli., (1) 253, 1903.
Koyanagi. K. M. Aug., xciv, 355, 1935.
Lagrange. A. d’O., vii, 65, 1887.
Lawford. T. O. S., iii, 163, 1883.
Lawrence, Madders and Millar.
J., ii, 559, 1930.
Leber. A. f. O., xxi (3), 226, 1875.
Mackenzie. R. L. O. H. Rep., ix, 134, 1879.
MacLean. T. O. S., l, 61, 1930.
Moore, Foster. T. O. S., x1, 15, 1920.
Medical Ophthalmology, London, II, 1925.
Mylius. B. O. G. Heidel., li, 150, 1936.
Nettleship. T. O. S., ix, 120, 1889.
R. L. O. H. Rep., xv, 338, 1903; xvi, 1 1,
1904.
Noorden. Die Zwckerkrankheit, 217, 1912.
O’Hare. A m. J. Med. Sc., clx, 366, 1920.
Onfray. An. d’Oc., clv, 533, 1918; clix, 599,
1922.
Pines. Brit. J. O., xiii, 97, 1929.
Rockliffe. T. O. S., xiv, 130, 1894.
Russo. Am. di Ott., lv, 222, 1927.
Shepardson and Crawford. Calif. and West.
Med., xxxv, l l 1, 1931.
Spalding and Curtis. Boston Med. Surg. J.,
cxcvii, 165, 1927.
Strieff. Diss., Zürich, 1898.
Sugita. Zb. ges. O., ix, 91, 1923.
Volhard. Verh. dewt. Ges. inn. Med., Wies-
baden, xxxiii, 422, 1921.
Wagener and Wilder. J. Am. Med. As.,
lxxvi, 515, 1921. |
Wagener, Dry and Wilder.
Med., coxi, 1131, 1934.
Brit. Med.
New Eng. J.
Vascular Retinopathies
The treatment of the vascular retinopathies does not really come within
the province of the ophthalmologist ;
physician and will not be entered into fully here.
T.O. —WOL. III.
it belongs essentially to the general
In general terms five
T T
2736 TEXT-BOOK OF OPHTHALMOLOGY
axioms should be insisted upon : (1) Dispel anxiety, (2) reduce speed,
(3) lessen intake, (4) promote elimination, and (5) exclude toxaemias.
The order of these indicates their relative importance. Apprehension,
anxiety and worry are potent aggravating factors ; mental and physical
vigour and restlessness are characteristic of these patients, who are always
greatly benefited by a liberal amount of rest and sleep. The diet should be
frugal, non-stimulating and non-alcoholic, of boiled rather than roasted
foods, and of vegetable rather than animal constituents. Elimination should
be encouraged through the kidneys by the drinking of an abundance of
bland fluids, by the skin, and above all, by the bowel, the latter not only with
a view to avoiding toxaemia, but also preventing the straining of constipation
—a dangerous practice ophthalmologically when the blood-vessels are
fragile. Finally, there is no doubt that chronic sepsis or intoxications
increase the incidence of retinal lesions, particularly in arteriosclerotic and
diabetic subjects, and may convert a slowly progressive disability into a
rapid tragedy. But in their elimination the greatest care should be exercised
lest by over-zealous treatment rashly undertaken the tragedy become
immediate, not only affecting vision but also life, for such patients are frail
and their defences poor. From the ophthalmological point of view it may
be true that a hypertensive sclerosis may be aggravated by a mouth full
of septic teeth, but their too sudden removal may easily convert an eye
which gives rise to anxiety only to the observer into one which, owing to a
thrombo-phlebitis, is quite useless to the patient——a change which reacts
greatly to the observer's discredit. There is no sphere in medicine where
theory requires to be tempered more by philosophy and understanding than
in this.
It is to be remembered, also, that the lowering of a high systolic blood-
pressure may not always be beneficial ; for in many of these cases the
capillary blood-pressure is abnormally low already and such interference
may lead to actual tissue-starvation, and an increase in the pathological
changes in the fundus. Iodides, so commonly prescribed, are safe, probably
because they have little effect. In cases of essential hypertension surgical
methods have achieved spectacular results in the hands of their advocates,
especially at the stage when angiospasm is predominant, but the time has
not yet arrived when the position can be evaluated.
The operative techniques have aimed chiefly at sectioning the splanchnic nerves
with a view to relieving constriction of the renal arteries, inhibiting the action of
the supra-renal glands, and providing a great vascular bed incapable of vaso-con-
striction to act as a passive reservoir and safety-valve. Hitherto, although some good
results have been achieved, both in the recession of retinal lesions and in an improve-
ment of visual acuity, no criterion of operability has been established, and these
procedures must still be left sub-judice.
With regard to local treatment to the eye there is indeed little that can
be done. Rest in bed is probably advisable if the ocular and general condi-
DISEASES OF THE RETINA 2737
tion suggests that a tragedy is imminent ; but on the whole it is inadvisable.
The use of the eyes in reading is permissible so long as it is relatively easy and
the light is good ; but when despite optical accessories it becomes difficult, it
is usually much more advisable for psychological reasons to prohibit it
rather than to allow the patient to be constantly annoyed and distressed by
his difficulties in attempting a task which becomes more and more trying
and ultimately impossible. The assurance that complete blindness will not
eventuate goes far to mitigate the sacrifice of the joys of reading.
2. Blood Diseases
1. THE ANAEMIAS
PERNICIOUS ANAEMIA
As in all severe anaemias, engorgement of the veins and retinal haemor-
rhages are the rule in pernicious anaemia, occurring in some 70% of cases
(Cabot, 1915), particularly when the red cell count falls to 25 or 30% of the
normal. They are usually small, superficial and flame-shaped with a pre-
dilection for the posterior pole, and they rarely affect vision unless the
macula happens to be involved (Bramwell, 1877; Carr, 1920; Rolleston,
1923; Ballantyne, 1923; and many others). Occasionally they are round
and deeply placed, and exceptionally large extravasations of blood may
occur (Mackenzie, 1882–84). Sometimes the haemorrhages show a pale
centre and they are not uncommonly associated with white woolly patches
(Fig. 2346, Plate LIII). These appearances, as well as the Opalescent hazi-
ness which may overspread the entire fundus as if it had been smeared with
milky water, may be due to oedema (Sargent, 1892; Holden, 1899).
Anatomical examination has shown the somewhat typical white centres of the
haemorrhages to be due to degenerating leucocytes (Manz, 1875; Bettmann, 1881 ;
Litten, 1881) or degenerated (varicose) nerve fibres (Uhthoff, 1880; Bondi, 1896).
In SUB-ACUTE COMBINED DEGENERATION, a disease in which a macrocytic anaemia
is combined with a degeneration of the spinal cord, retinal haemorrhages occur
somewhat rarely, but they may be very extensive (Collier, 1910; Taylor, 1913).
In APLASTIC ANAEMIA, a rapidly fatal toxic condition, there are few records of
fundus changes, but retinal haemorrhages do occur (Carslaw and Dunn, 1910; Weber,
1914).
CHLOROSIs, now an almost extinct disease, is singularly free from the complication
of haemorrhage. Papilloedema is the commonest ocular complication in the somewhat
sparse literature, and it may be associated with a macular star and rarely with haemor-
rhages (Foster Moore, 1925; Brazeau, 1925; Hamilton, 1936).
T T 2
2738 TEXT-BOOK OF OPHTHALMOLOGY
The SECONDARY ANAEMIAS are almost constantly associated with retinal changes
if they are of sufficient severity to reduce the red cell count to 50% (Mackenzie, 1884;
Foster Moore, 1925). These changes include a general pallor of the fundus and of the
vessels, frequently a distension of the veins, occasionally the presence of an opalescent
oedema-like haze over the fundus, and often the occurrence of multiple small
superficial haemorrhages frequently associated with soft woolly exudative patches. All
these appearances tend to clear up without symptoms or sequelae. Since such haemor-
rhages and exudates are relatively rare in post-traumatic haemorrhagic anaemia, it is
probable that some toxic factor is present which damages the capillary endothelium
and allows the escape of plasma and the formed elements of the blood.
The retinal sequelae of Post-HAEMORRHAGIC ANAEMIA have already been discussed.*
Very typical haemorrhagic appearances are seen in the retina induced by loss of blood
from intestinal parasites (Rampoldi, 1880; Fischer, 1892–96 ; Nieden, 1903; and
others).
Ballantyne. T. O. S., liii, 158, 1923. Holden. A. of O., xxviii, 125, 1899.
Bettmann. A. f. Aug., xi, 28, 1881. Litten. Berlin. kl. W., viii, 6, 1881.
Bondi. A. f. Aug., xxxiii, Erg., 83, 1896. Mackenzie. T. O. S., ii, 40, 1882; iv., 132,
Bramwell. Edin. Med. J., xxiii, 408, 1877. 1884.
Brazeau. Am. J. O., viii, 811, 1925. Manz. Zb. f. med. Wiss, xiii, 675, 1875.
Cabot. Osler’s System of Medicine, iv, 632, Moore, Foster. Med. Ophthal., London, II,
1915. 1925.
Carr. Am. J. Med. Sc., clx, 737, 1920. Nieden. Cb. pr. Aug., xxvii, 207, 1903.
Carslaw and Dunn. Glasgow Med. J., lxxiii, Rampoldi. Am. di Ott., ix, 121, 233, 1880.
331, 1910. Rolleston. T. O. S., liii, 110, 1923.
Collier. System of Med. (Allbutt dº Rolleston), Sargent. A. of O., xxi, 39, 1892.
vii, 799, 1910. Taylor. T. O. S., xxiii, 1, 1913.
Fischer. B. O. G. Heidel., xxii, 26, 1892. Uhthoff. K. M. Aug., xviii, 513, 1880.
Cb. pr. Aug., xx, 81, 1896. Weber. Proc. R. S. Med., vii (Sect. Med.),
Hamilton. Brit. J. O., xx, 18, 1936. 176, 1914.
2. ERYTHRAEMIA
ERYTHRAEMIA (POLYCYTHAEMIA), a disease first described by Vaquez
(1892) whose description was amplified by Osler (1903) (VAQUEZ' DISEASE or
the VAQUEz-OSLER DISEASE), is a clinical entity of unknown aetiology caused
by a hyperplasia of the erythroblastic elements of the bone marrow
(Weber, 1908). It occurs in adults, particularly males, and is characterized
by an enormously increased red cell count, an enlargement of the spleen and
liver and a florid cyanotic appearance. It is to be distinguished (poly-
cythaemia vera) from the closely related condition of secondary erythraemia
which is a compensatory reaction caused by congenital heart disease,
pulmonary stenosis, emphysema, and, to a less extent, is found temporarily
in persons reaching high altitudes. The general manifestations of the two
are similar, but the second occurs at all ages, is not associated with
an enlarged spleen, but is usually characterized by more extreme cyanosis,
while the great majority of cases show no ocular complication apart from the
cyanosis, visual disability due to endarteritis and thrombosis being
exceptional (Fig. 2155, Plate XLVIII).”
1 p. 2556. ? p. 2554.
PLATE LIII
HAEMopoietic Diseases
FIG. 2345.-LIPAEMIA.
234s-Leukemia (Early sta e.) Fig. 2349–Leukºna.
Fig. 23 ( y stag (Late stage of retinitis).
[To face p. 2738.

DISEASES OF THE RETINA 2739
Ocular complications occur with considerable frequency in polycythaemia vera ;
thus Lucas (1912) found visual disturbances in 13 out of 40 cases, Christian (1917)
in 8 out of 10, while in 5 cases Cohen (1937) found vascular changes in 3 and retinal
lesions in 2. The retinal appearances were called the fundus polycythemicus by Ascher
(1914), and papers of importance have been written, among others, by Hutchison and
Miller (1906), Engelbach and Brown (1906), Watson (1906), Jackson (1906–07), Parker
and Slocum (1908), Behr (1911), Dupuy-Dutemps and Lutembacher (1912), Cohen
(1918–37), Engelking (1920) and de Schweinitz and Woods (1925).
The retina may be normal but usually there is some engorgement and
tortuosity of the veins which appear purple, while the arteries remain
normal ; sometimes these phenomena become extreme and the veins become
enormously engorged and the whole fundus cyanotic. When the venous
stasis becomes extreme haemorrhages appear, sometimes in quantity,
together with Oedema of the disc, which may even simulate papilloedema
due to a cerebral tumour. A further stage is reached in the development
of thromboses, sometimes affecting the central vein (Cohen, 1918) and
occasionally extending to the cranial sinuses (Foster Moore, 1925). A
pathological examination recorded by Behr (1911) showed massive conges-
tion of the veins with oedema but a complete absence of inflammatory
evidences.
It is important that visual symptoms occur in the absence of retinal
changes and are frequently more profound than those changes would seem
to warrant. Photophobia, photopsiae, the appearance of black spots and
scotomata, hemianopia and a general mistiness of vision are not infrequent,
and indeed complete blindness (Hutchison and Miller, 1906; Lucas, 1912)
or very grave visual defects may be associated with a fundus which is normal
or shows few and unimportant changes (Osler, 1903; Friedenwald, 1919;
Foster Moore, 1925; and others). These phenomena may sometimes be
due to a retro-bulbar disturbance (Friedenwald, 1919) or to angiospasm
(Mylius, 1928), but are frequently associated with cerebral haemorrhages or
thromboses (Kerschner, 1921).
Treatment is on general lines by venesection, phenyl-hydrazine and
radiation.
Ascher. K. M. Aug., liii, 388, 1914. Kerschner. A. Neurol, and Psy., vi, 105, 1921.
Behr. K. M. Aug., xlix, 1, 672, 1911. Lucas. A. of Int. Med., x, 597, 1912.
Christian. Am. J. Med. Sc., Cliv, 547, 1917. Moore, Foster. Medical Ophthal., London, II,
Cohen. A. of O., xlvii, 2, 1918; xvii, 81 1, 1925.
1937. Mylius. B. O. G. Heidel., xlvii, 379, 1928.
Dupuy-Dutemps and Lutembacher. An. Osler. Am. J. Med. Sc., exxvi, 187, 1903.
d’Oc., cylviii, 81, 1912.
Engelbach and Brown. J. Am. Med. As.,
xlvii, 1265, 1906.
Engelking. K. M. Aug., lxiv, 645, 1920.
Friedenwald. Contrib. to Med. and Biol.
Researches, N.Y., i, 495, 1919.
Hutchison and Miller. Lancet, i, 744, 1906.
Jackson. T. A. m. O. S., xi, 363, 1906.
Ophthalmology, iv., 1, 1908.
Parker and Slocum. T. Am. O. S., xii, 505,
1909.
de Schweinitz and Woods. T. A. m. O. S.,
xxiii, 90, 1925.
Vaquez. Bull. med., vi, 849, 1892.
Watson. Liverpool Med. Chir. J., xxvi, 33,
1906.
Weber. Quart. J. Med., ii, 85, 1908.
2740 TEXT-BOOK OF OPHTHALMOLOGY
3. THE HAEMORRHAGIC DIATHESES
PURPURA HAEMORRHAGICA, a disease wherein the blood-platelets are
much reduced in number and which is characterized by spontaneous haemor-
rhages from the mucous surfaces, is sometimes, but not by any means
constantly, associated with retinal haemorrhages which are without prog-
nostic significance ; they are usually small, superficial and near the disc
(Ischreyt, 1921; Foster Moore, 1925; Kyrieleis, 1926). Rarely they are
extensive, infiltrating all the retinal layers, and giving rise to pre-retinal and
sub-retinal extravasations (Cohen, 1920), and even causing blindness (Pratt,
1915). In association with the haemorrhages there may be oedematous
changes, small exudative white spots and a macular star (Doyne, 1937).
In HAEMOPHILIA retinal haemorrhages do not occur except on the rarest
occasions (Hirschberg, 1879; Bramwell, 1886).
SCURVY (Barlow’s Disease) is rarely associated with retinal haemorrhages
although extravasations of blood in other regions are common (Löwenstein,
1917; Zlocisti, 1917). The common retinal disturbance is night blindness
associated with the metabolism of visual purple, a subject which has already
been discussed.*
Bramwell. Ed. Med. J., xxxii, 101, 1886. Löwenstein. K. M. Aug., lix, 583, 1917.
Cohen. A. of O., lii, 471, 1920. Moore, Foster. Medical Ophthal., London II,
Doyne. Proc. R. S. Med., xxx, 943, 1937. 1925.
Hirschberg. A. f. Aug., viii, 174, 1879. Pratt. System of Medicine (Osler and
Ischreyt. K. M. Aug., lxvi, 211, 1921. McCrae), iv, 698, 1915.
Kyrieleis. A. f. Aug., xcvii, 514, 1926. Zlocisti. K. M. Aug., lix, 572, 1917.
4. LEUKAEMIA
Changes in the fundus, sometimes referred to as leukaemic retinitis, are
common in all types of leukaemia, acute or chronic, lymphoid and myeloid.
They may be detected early and are almost constant in the later stages.
They were first described and figured by Liebreich (1863), and are now well
recognized as being pathognomonic in their more advanced stages.
Estimates of the incidence of retinal lesions vary : thus Foster Moore (1925) in
25 patients (20 myeloid and 5 lymphoid) found only 3 with normal fundi, while Gold-
stein and Wexler (1935) in a study of 11 cases found the retinal lesions minimal. In
the statistics of Johns Hopkins Hospital, Goldbach (1933) found retinal lesions in 10,
tortuous vessels in 4, and optic neuritis in 9 out of 45 cases of acute lymphatic leukaemia;
retinal lesions in 2, tortuous vessels in 9, and optic neuritis in 3 out of 42 cases of
chronic lymphatic leukaemia; retinal lesions in 5, tortuous vessels in 4, and optic
neuritis in I Out of 9 cases of acute myeloid leukaemia ; and retinal lesions in 25,
tortuous vessels in 25, and disturbances at the nerve-head in 22 out of 143 cases of
chronic myeloid leukaemia.
1 Vol I, p. 970 (982).
DISEASES OF THE RETINA 274.1
The clinical picture in its earliest stages shows an engorgement of the
retinal vessels, particularly the veins, which may be extreme in degree and
associated with marked tortuosity (Fig. 2348, Plate LIII). Eventually the
veins and arteries become yellowish, approaching each other in colour, and the
former become turgid and segmented into sausage-like formations, being
bitten deeply into by the arteries at the crossings. Eventually hamorrhages
appear, usually small and widely scattered, either superficial and flame-
shaped or deep and rounded. Typically they show a central white
area usually said to be due to leucocytic accumulation for which, however,
there is little histological evidence; and quite frequently exudative white
spots are scattered about, especially in the central region independently of
Fig. 2350.-Acute LEUKAEMIA.
Massive infiltration and haemorrhages in the retina and choroid (Parsons).
the haemorrhages. Occasionally the bleeding is extensive and it may be
pre-retinal (Stock, 1906).
In the fully developed condition of LEUKAEMIC RETINorATHY a
typical ophthalmoscopic picture is presented (Fig. 2349, Plate LIII). The
retina appears thickened and takes on a pale green or orange hue owing to
leucocytic infiltration of this tissue and choroid, the disc may become
oedematous, and sheathing the dilated and tortuous vessels are broad lines
of perivascular infiltration, sometimes as broad as the vessels themselves
and widest along the convexities of the curves of the veins. The haemor-
rhagic and exudative lesions may give rise to visual disturbances if they
occur in the central area, and in the most marked cases constrictions of the
visual fields and night-blindness may occur.
Pathological examinations of these cases have been recorded by several authors
(Deutschmann, 1878: Michel, 1878; Oeller, 1878; Edmunds, 1890; Puccioni

2742 TEXT-BOOK OF OPHTHALMOLOGY
1898 ; Murakami, 1901 ; Parsons, 1908 ; Verderame, 1910 ; Hudson, 1911 ; Kambe,
1914; Koyanagi, 1917; Kümmell, 1918; Cohen, 1918; Bab, 1922; Goldstein and
Wexler, 1935; Gibson, 1938; and others) (Fig. 2350). The tissues are widely infiltrated
with leucocytes which accumulate especially round the vessels distending the peri-
vascular spaces and giving them the sheathed appearance. Murakami (1901) found
that in places this process had proceeded to complete solution of the vessel
walls. Hypertrophy and degenerative changes occur in the nerve-fibre layer and
cystic spaces in the outer layers of the retina. The papilla is infiltrated and haemor-
rhages are found in all layers of the retina. Moreover, as we have seen,” similar
changes occur in the choroid, in which connection the question of the origin of the
infiltrating white cells has already been discussed.
Occasionally cases occur which run throughout their clinical course
without involvement of the retina, or without the development of ocular
signs, but an extensive retinal infiltration usually occurs before death. In
general there is a relation between the degree of retinal involvement and the
severity of the disease, the haemorrhages appearing and disappearing with
the exacerbations and remissions, but the correspondence is not sufficiently
close to have an absolute prognostic value. Goldbach (1933) found little
correspondence between the amount of retinal haemorrhage and the white
cell count, but the relation between the former and the degree of anaemia
seems to be closer (Meller, 1906; Gibson, 1938).
Treatment is that of the general disease ; but in an acute case in an infant
Alvis (1938) obtained temporary improvement by radiation which persisted
until the fatal termination of the illness.
In CHLOROMA retinal haemorrhages, exudative spots and papilloedema may occur
(Sidler-Huguenin, 1921 ; Rolleston, 1923; James, 1923), and occasionally a neoplastic
infiltration of the retina (Gallemaerts, 1914).
Alvis. Am. J. O., xxi, 31, 1938. Kümmell. A. f. O., xcv, 105, 1918.
Bab. Z. f. Aug., xlvii, 231, 1922. Liebreich. Atlas, 1863.
Cohen. A. of O., xlvii, 61, 1918. Meller. A. f. O., lxii, 130, 1906.
Deutschmann. K. M. Aug., xvi, 231, 1878. Michel. Deut. A. f. kl. Med., xxii, 439, 1878.
Edmunds. T. O. S., x, 157, 1890. Moore, Foster. Medical Ophthalmology,
Frank. Med. J. Australia, i, 364, 1935. London, II, 1925.
Gallemaerts. K. M. Aug., lii, 878, 1914. Murakami. K. M. Aug., xxxix, 136, 1901.
Gibson. A. of O., xx, 365, 1938. Oeller. A. f. O., xxiv. (3), 239, 1878.
Goldbach. A. of O., x, 808, 1933. Parsons. Path. of the Eye, iv, 1317, 1908.
Goldstein and Wexler. A. of O., xiii, 26, 1935. Puccioni. A. di Ott., xxvii, 519, 1898.
Hudson. R. L. O. H. Rep., xviii, 153, 1911. Rolleston. T. O. S., xliii, 110, 1923.
James. T. O. S., xliii, 153, 1923. Sidler-Huguenin. K. M. Aug., lxvii, 55,
Kambe. K. M. Aug., lii, 79, 1914. 1921.
Royanagi. K. M. Aug., liii, 152, 1914. Stock. K. M. Aug., xliv (1), 328, 1906.
Am. J. O., xxxiv, 230, 1917. Verderame. A. f. path. Anat., ce, 367, 1910.
5. LIPAEMIA
LIPAEMIA, a condition wherein fat in an emulsified state is present in the
blood to a degree which alters its appearance, occurs in a variety of condi-
tions, such as diabetes, alcoholism, starvation, asphyxia, phosphorus
1 p. 2376.
DISEASES OF THE RETINA 27.43
poisoning, pneumonia, peritonitis (Hale White, 1903), trench nephritis
(Foster Moore, 1915), and xanthomatosis (Wile, Curtis and Eckstein, 1929).
With one exception, however, that of a leukaemia which had undergone
radiational treatment (Wagener, 1922), diabetes is the only disease wherein the
condition becomes sufficiently marked to be seen ophthalmoscopically in the
retinal vessels as a LIPAEMIA RETINALLs, and in practically every case the
diabetes is associated with acidosis.
Lipoids are found in the blood in three forms—fat (tri-glycerides of the higher
fatty acids), phosphatids, and cholesterol ; in lipaemia the greatest increase occurs in
the fatty acid fraction, next is the cholesterol and next is the phospholipins. The
increase is essentially due to an inability to absorb or to utilize the fat ; but a simple
increase in fat content is not sufficient to produce the appearance of a lipaemia. It is
necessary for it to be emulsified and thus rendered visible (Wishart, 1922), so that the
anomalous position may arise of a lipaemia appearing when the blood contains 9.5%
lipoids and disappearing when it reaches a higher value (10.5%) (McCann, 1923).
The cause of the assumption of this physical state is unknown but it may have some
relation with acidosis. The level of blood lipoids necessary to produce the characteristic
appearance has been found to vary. The highest level recorded is the astonishing one
of 48% (Chase, 1927), and high values have been reported by Köllner (1912) of 26.25%,
by Jaffe and Schonfeld (1933) of 21.7%, by Reis (1903) of 18%, and by Ferber and
Appermann (1927) of 18% ; the lowest recorded levels at which the phenomenon
appeared in the retina are 3.5% (Wagener, 1922), 4% (Heine, 1906) and 4.5% (Wagener,
1922). In Chase's case (1931) the lipaemia disappeared between 1:42 and 1.13% ;
in McKee and Rabinowitch's (1931) at 1.13% ; and in Lepard’s (1933) it appeared when
the concentration rose to 4.25% and disappeared below 2.8%. On the average it
may be taken that lipaemia is liable to appear when the blood lipoids rise above 3.5%
and to disappear when they fall below 2–2.5%.
Lipaemia retinal's is not a common condition, and since its original
description by Heyl (1880), relatively few cases have appeared in the
literature. Parker and Culler (1930) summarized 38 cases, and added 2 of
their own, Jaffe and Schonfeld (1933) brought the number up to 41, Marble
and Smith (1936) to 48, and Allen and Howard (1936) added another. The
great majority of cases are young diabetics, and above 40 years of age it is
quite exceptional ; the average age is 25 years with extremes of 11 (Allen
and Howard, 1936) and 50 years (Gray and Root, 1923). The vast majority
occur in females (over 80% of cases); and all the cases are in or approaching
a state of coma.
The clinical picture is quite unmistakable and pathognomonic (Fig. 2345,
Plate LIII). The change in colour of the retinal vessels is seen first in the
periphery where they become hazy and slightly milky, and gradually the
appearance spreads up towards the disc, the arteries and veins in the average
case becoming flat and ribbon-like and assuming a uniform appearance as
if filled with milk, although in some cases a salmon-colour merging into cream
is retained on or near the disc. In other cases the appearance is yellowish,
but in all cases the light reflex becomes diffuse or is lost, and frequently
yellowish-white stripes accompany both arteries and veins towards the
2744 TEXT-BOOK OF OPHTHALMOLOGY
periphery as if there were a fatty infiltration of the peri-vascularis. At the
same time, the disc and ground of the fundus remain normal. In the
worst cases the milky colour deepens to become a dull ivory and the fundus
assumes a lighter hue due to similar changes in the choroidal vessels (Hale
White, 1903; Chase, 1927; Allen and Howard, 1936). As the condition
clears up the vessels near the disc become salmon-red and then normal, while
the peculiar appearance lingers longer at the periphery.
The only ocular complication in the literature is a very occasional small haemor-
rhage (Cohen, 1921 ; Allen and Howard, 1936). A xanthoma, which may be fleeting,
has occurred several times (Hardy, 1924; Ferber and Appermann, 1927 ;
Machlis, 1924).
Pathological examinations have shown consistently that the retinal tissues are
normal, but in the vessels the plasma is loaded with fat which permeates into the peri-
vascular spaces (Fraser, 1903; Reis, 1903; Heine, 1906–07; Moore, 1915; Hardy,
1924; Muskat, 1924).
Patients suffering from lipaemia retinalis are all, of course, extremely ill.
It is interesting that before the advent of insulin all the reported 18 cases,
with one exception (Hale White, 1903), died in coma ; since the advent of
insulin this proportion has been reversed and immediate deaths are
exceptional. The actual occurrence of lipaemia does not alter the prognosis
from that of other cases of impending diabetic coma ; these patients with
proper caution may expect to lead a relatively long life, but they usually
belong to the group of diabetics to whom indiscretions or inter-current
infections are disastrous.
Allen and Howard. Am. J. O., xix, 645, 1936.
Chase. Canad. Med. As. J., xvii, 197, 1927.
Marble and Smith. A. of O., xv, 86, 1936.
McCann. Bull. Johns Hopkins H., xxxiv,
J. Am. Med. As., xcvii, 171, 1931.
Cohen. A. of O., i, 247, 1921.
Ferber and Appermann. Med. J. and Rec.,
cxxv, 815, 1927.
|Fraser. Brit. Med. J., i, 1205, 1903.
Gray and Root. J. Am. Med. As., lxxx, 995,
1923.
Hardy. Am. J. O., vii, 39, 1924.
Heine. K. M. Aug., xliv. 451, 1906.
Deut. med. W., xxxiii, 85, 1907.
Heyl. T. Am. O. S., iii, 54, 1880.
Jaffe and Schonfeld. A. of O., ix, 531, 1933.
Röllner. Z. f. Aug., xxvii, 411, 1912.
Lepard. Am. J. O., xvi, 12, 1933.
Machlis. J. Am. Med. As., lxxxiii, 1428, 1924.
302, 1923.
McKee and Rabinowitch. Canad. Med. As.
J., xxv, 530, 1931.
Moore, Foster. Lancet, ii, 1348, 1915.
T. O. S., x1, 19, 1920.
Muskat. Am. J. O., vii, 288, 1924.
Parker and Culler. Am. J. O., xiii, 573, 1930.
Rabinowitch. Am. J. Med. Sc., clxxvi, 489,
1928.
Reis. A. f. O., Iv, 437, 1903.
Wagener. Am. J. O., v, 521, 1922.
White, Hale. Lancet, ii, 1007, 1903.
Wile, Curtis and Eckstein. A. of Derm. Syph.,
xix., 35, 1929.
Wishart. J. Metab. Res., ii, 199, 1922
HAEMOCHROMATOSIS
HAEMOCHROMATOSIS is a derangement of the hepatic and pancreatic metabolism
wherein excessive deposits of copper and iron are laid down in almost all the organs
and tissues. In consequence of these changes a clinical picture of diabetes mellitus
is produced accompanied by a slaty-blue discoloration of the skin (bronzed diabetes).
In the retina the picture has been described by Maddox (1933) (Fig. 2347, Plate LIII):
DISEASES OF THE RETINA 2745
a slaty-blue pigmentation is concentrated near the disc which tends to fade away
peripherally through which the retinal background appears of an ochre-colour.
Maddox. Brit. J. O., xvii, 393, 1933.
V. ATROPHIES AND DEGENERATIONS
A. Senile Changes
In the aged the retina shows less marked evidences of semility than most
tissues. As a whole it becomes less transparent owing largely to an increase
in the neuroglial elements, a process which is associated with a parallel
º
º
º
- º
* * * *
º -
º
Fig. 2351.-SENILE DEGENERATION of PIGMENTARY EPITHELIUM.
Irregularity of epithelium with clumping of the granules; arteriosclerosis of
the choroid (Rones, Am. J. O.).
tendency to atrophy of the neural elements. These changes are associated
with the presence of diffuse atherosclerosis," and when the vascular
changes are marked, atrophic changes may be widespread. In this event
they usually start at the periphery, and are most marked in the inner layers
which show a progressive atrophy of the nerve-fibre layer, the ganglion cells
1 p. 2696.

2746 TEXT-BOOK OF OPHTHALMOLOGY
and the inner nuclear layer, while the fibrous structure of the reticular layers
is more clearly displayed. When to this is added degeneration of the chorio-
capillaris, the outer layers are involved also, and the whole thickness of the
retina may undergo fibrous transformation and cystic degeneration, a matter
we shall discuss presently in a separate section. *
In this general atrophy two portions are particularly prone to suffer—
the periphery and the macula. The former is rarely of serious clinical
importance, but when the latter becomes involved, the vision may be
gravely affected. The more is this so when choroidal sclerosis denies this
avascular area its only source of nourishment, in which case the well-
known clinical picture of senile macular degeneration results."
The PIGMENTARY EPITHELIUM, like its homologue in the ciliary body
and the iris,” shows marked and characteristic changes, partly regressive and
partly hypertrophic in nature (Fig. 2351). This occurs early and with great
constancy, being seen in the majority of eyes over 50 years, particularly in
the neighbourhood of the ora. The cells lose their regular hexagonal shape,
and become irregularly pigmented. The pigment granules themselves
become spherical instead of rod-shaped, and are unevenly distributed, being
heaped up in some cells and almost disappearing from others. Further
regressive changes include the appearance of vacuolation and fatty degenera-
tion and eventually of nucleolysis, and a breaking up of the cell with a
scattering of the pigment granules. Alternatively, hyperplastic changes
may occur, the cells proliferating and increasing their pigmentary content,
some containing two nuclei and others being aggregated into piled-up
groups of ill-formed elements, while an increase in their metabolic activity is
seen in a thickening of the hyaline portion of Bruch's membrane and the
deposition of the same material in masses to form colloid bodies—a subject
which will be dealt with in detail presently.
B. Secondary Degenerations
SECONDARY DEGENERATIONS of the retina, such as follow inflammatory
conditions or occur in angiopathies, involving a disappearance of the nerve
elements, proliferation of the neuroglia, and the formation of fatty, hyaline
and even calcareous deposits, have already been fully discussed.” So also
have the general atrophic changes with diminution of the blood-vessels and
frequently some degree of optic atrophy which follow more diffuse conditions
such as syphilitic neuro-retinitis (Fig. 2662); it will be remembered that
in these the most striking feature is the migration of pigment into the retina
to constitute the picture of secondary pigmentary degeneration (retinitis
pigmentosa).
1 p. 2372. * pp. 2392, 2394. 8 p. 2628.
DLATE LIV
CoLLoID DEGENERATION
Fig. 2352.-DIFFU's E. Colloid Fig. 23.53.-MAssive Colloid
DEGENERATIox. DEGENERATION.
Fig. 2355.-RING-shaped Colloid Fig. 2356.-SENILE CENTRAL Colloid
DEGENERATION. DEGENERATIon.
[To face p. 2746.


DISEASES OF THE RETINA 2747
Two conditions, however, which are of peculiar interest must be
considered in detail—the formation of colloid bodies and cystic degeneration.
CoLLoID BoDIEs (DRUSEN)
The deposition of masses of hyaline material in association with the
cuticular layer of Bruch's membrane is a common and well-known event,
which was noted histologically by Wedl (1849) and was first elaborately
studied by Donders (1855) and Müller (1856). A degeneration of this type
appears in three sets of circumstances.
1. As a senile phenomenon, analogous to the similar Hassall-Henle bodies
deposited on the membrane of Descemet." This type is extremely common,
Fig. 2357.-Colloid BoDIEs IN PHTHIsis Belbi.
The lens is calcareous; the retina totally detached; the choroid studded with
colloid bodies. (x 4) (Parsons).
Müller (1856) finding them occasionally after 30, very frequently over 45,
and constantly over 60 years of age. -
Their occurrence in young people, when they are usually widely spread, may be
due to some obscure general or local metabolic disturbance or to the type of premature
degeneration called abiotrophy. They have been noted at the age of 12 (Alt, 1877),
15 (Verhoeff, 1937) and 22 (Davenport, 1929).
2. As a degenerative phenomenon in diseased states of the retina or the
choroid, either vascular, inflammatory or neoplastic. They are extremely
* Vol. II, p. 1993.

2748 TEXT-BOOK OF OPHTHALMOLOGY
common and numerous in phthisical eyes (Fig. 2357), and may be produced
experimentally by cutting the posterior ciliary arteries in animals (Schreiber,
1906).
3. As a transmissible primary degeneration in which case it constitutes
Fig. 2358.-Col. Lord Body.
Appearing as a simple sessile bulging of Bruch's membrane and covered by
a single layer of flattened epithelium. Haºmatoxylin and eosin (x 200) (Coats,
T. O. S.).
-
Fig. 23.59.-Colloid Body.
Covered by proliferated epithelium and containing nuclear inclusions. Note
extreme degeneration of choroid, from which vessels are almost absent, the chorio-
capillaris being degenerate (Coats, T. O. S.).
a separate clinical entity occurring in young adults (FAMILIAL colloid
DEGENERATION: Doy NE’s HoNEycomb choronditis). Such colloid
excrescences also occur as a primary degeneration in the condition of
retinitis punctata albescens of Mooren.”
The clinical appearance of colloid bodies is characteristic (Plate LIV).
p. 2786, * p. 2784.


DISEASES OF THE RETINA 27.49
The more simple type is seen in uncomplicated senile states, wherein the
excrescences appear as small bright sharply-defined circular points lying
underneath the retinal vessels, varying in colour from white to bright yellow,
somewhat elevated from the fundus and frequently showing slightly pig-
mented margins. In the earliest stages of their development they are small
spots slightly pinker than the rest of the fundus; as they enlarge they
become yellow, and eventually they appear almost white. Usually they
are discrete, but not infrequently they coalesce to form larger rounded
masses, and rarely they assume irregular shapes. They are found most
Fig. 2360.-Colloid BoDIEs.
Of laminated structure. Note the layer of pigmentary epithelium above, and
below the fine highly refracting zig-zag line of the outer elastic layer of Bruch's
membrane (x 200) (Coats).
numerously in the region of the ora serrata and at the posterior pole in the
disc-macular area, but at other times they are scattered uniformly and
frequently in great numbers over the entire fundus (Figs. 2352 and 2353, Plate
LIV).
The first adequate clinical report of the occurrence of these changes in the central
area was published by Jonathan Hutchinson (1875) under the title symmetrical central
choroido-retinal disease occurring in senile persons, but from the conspicuous part
ascribed by him to Waren Tay in the observations he recorded, the condition came to
be called Tay’s central guttate choroiditis. Correspondingly a universal distribution
of the excrescences over the fundus was called guttate choroiditis (Juler, 1893).
It must be remembered, however, that Hutchinson's original description embraced a
heterogeneous collection of different types including old choroiditis, renal retinopathy

2750 TEXT-BOOK OF OPHTHALMOLOGY
and traumatic changes as well as colloid degeneration, and consequently the literature
on “Tay’s choroiditis" is sometimes confused.
In pathological cases the colloid changes, as one would expect, affect
particularly the areas involved—the site of an old choroiditis, the part of the
retina overlying a tumour, and so on. While in these states the same round
discrete spots may occur, the tendency is for the depositions to be larger and
less regular with angular or sinuous margins and a flat or scaly surface. They
may also be raised up or lie in the substance of the retina itself, and on
occasions assume quite bizarre shapes.
The clinical symptoms produced by colloid bodies in uncomplicated cases
are negligible, for even if they occur in masses in the foveal region vision
may remain normal (Nettleship, 1903). Occasionally the central vision
does fall, but in these cases, although pressure upon the retinal elements
may have some deleterious influence, it is probable that the fall is rather due
to parallel degeneration and sclerotic changes in
the retina or in the central area of the choroid
underneath which are so frequently the con-
comitants of age.
Pathologically these colloid depositions have
received considerable study. They are always
associated with degenerative changes in the
pigmentary epithelium, the cells of which
become swollen and irregular, sometimes show-
º, º º * º ing proliferative and sometimes degenerative
(Parsons). tendencies. This, according to Müller (1856),
is followed in simple senile cases by a general
thickening of the cuticular portion of Bruch's membrane from which the
bodies take origin. The smaller elevations are covered with pigment
epithelium which may appear relatively normal (Fig. 2358); alternatively
the epithelium may proliferate (Fig. 2359) or over the larger ones the cells
may become flattened or fail altogether. At first the masses are finely
granular (Kerschbaumer, 1892), at a later stage they become homogeneous in
appearance, and as they become older and increase in size, a concentric
lamination is not uncommon (Alt, 1877) (Fig. 2360). Finally, calcium may
be deposited in them, the lime salts being deposited centrally or irregularly.
Occasionally, when the underlying membrane of Bruch is disintegrated, they
may be invaded by connective tissue from the choroid and true ossification
may occur (Fig. 2361).
As a rule in simple senile conditions the nodules are regularly shaped
and concentrically constructed, although the fusion of neighbouring masses
may produce irregular effects. They lie upon Bruch's membrane, sometimes
being attached thereto by a sessile stalk, but the elastic (choroidal) portion
of the membrane runs along unaffected by their presence. In pathological
conditions, however, the individual modules may assume the most compli-

DISEASES OF THE RETINA 2751
cated shapes, forming irregular plaques of all shapes and sizes, sometimes
buried in, and sometimes enclosing within them, masses of proliferated
pigmentary cells (Fig. 2362). Occasionally, indeed, hyaline depositions of
this type may be associated with epithelial cells which have migrated into
the retina and even reached its inner surface (Schreiber, 1906).
Theories of Origin. A very considerable controversy has taken place regarding
the aetiology of these colloid depositions, but to-day most authorities are agreed with
the theory originally put forward by Leber (1869) and elaborated by Coats (1905),
that they represent an aberrant secretory activity of the cells of the pigmentary epithelium.
The cuticular portion of Bruch's membrane is normally secreted by this epithelium,
Long scales of colloid substance clothed on each side by pigmentary epithelium
(Coats, T. O. S.).
Fig. 2362–ANoMALous Colloid Boones.
and in abnormal conditions the cells tend to secrete an abnormal amount of the same
substance.
This view, however, has by no means been universally accepted. Some writers
considered that the pigment epithelium was unconnected with their formation–
Rudnew (1871) claimed that they represent degenerated leucocytes, Pes (1904) that
they were hyalinized projections of the choroid pushed up by fibrous contraction,
Hofmann (1902) that they were thrombi from the chorio-capillaris, while Hanssen
(1917) held that the occasional presence of connective tissue elements and bone proved
a choroidal origin—but these theories may be disregarded. More doubt may be said
to exist as to whether they represent a degeneration of the pigmentary cells themselves,
that is, that they are formed by transformation rather than deposition (Donders,
1855; di Vicentiis, 1874; Schieck, 1903 ; Rones, 1937). The grounds advanced for this
hypothesis were essentially that sometimes a connection with the membrane of Bruch
could not be demonstrated, that this structure frequently could be seen unaffected
by the presence of the drusen, that they sometimes showed a different composition
from the euticular membrane, and that the highly degenerative cells were unlikely to
be responsible for increased secretory activity. Whether or not they are in continuity
with Bruch's membraneseems immaterial, and it is now recognized that the mesodermal
elastic portion of the membrane always runs on independently of them. Moreover,
T.D.—wol. IV. U U.

2752 TEXT-BOOK OF OPHTHALMOLOGY
the deposition of lime salts or lipoid material conforms to the usual degenerative
changes occurring in inert substances, while the proliferative activity of the epithelium
in degenerative conditions is well known. The general appearance, the regular build
of the senile type, and the frequent laminated structure all indicate that the hyaline
substance is probably an extra-cellular deposition, and it is probably Safest to accept
this theory until the demonstration of its intra-cellular deposition proves the opposite
view.
Alt. A. of O., vi, 304, 1877.
Coats. R. L. O. H. Rep., xvi, 164, 1905.
T. O. S., xxxi, 18, 1911.
Davenport. T. O. S., xlix, 110, 1929.
Donders. A. f. O., i (2), 106, 1855; iii (1),
Leber. A. f. O., xv (3), 1, 1869.
Müller. A. f. O., ii (2), 1, 1856.
Nettleship. R. L. O. H. Rep., xv., 189, 1903.
Pes. A. f. O., lix (3), 472, 1904.
Rones. A. of O., xviii, 388, 1937.
150, 1857.
Hanssen. K. M. Aug., lviii, 249, 1917.
Hofmann. A. f. Aug., xliv, 339, 1902.
Hutchinson. R. L. O. H. Rep., viii, 231, 1875.
Juler. T. O. S., xiii, 143, 1893.
Rerschbaumer. A. f. O., xxxviii (1), 127,
1892.
Rudnew. A. f. path. Amat., liii, 455, 1871.
Schieck. B. O. G. Heidel., xxxi, 320, 1903.
Schreiber. B. O. G. Heidel., xxxiii, 286, 1906.
Verhoeff. A. of O., xviii, 400, 1937.
di Vincentiis. Movimento med. chir. Estratto.,
31, 1874.
Wedl. Grundzüge d. path. Hist., Wien, 1849.
CYSTIC DEGENERATION OF THE RETINA
CYSTIC, or CYSTOID, DEGENERATION OF THE RETINA is properly a passive
degenerative process wherein gaps are formed within the tissue owing to the
disintegration of its neural elements. This process should thus be theoreti-
cally distinguished from the formation of true cysts in which fluid accumu-
lates progressively producing a separation of the tissue-structure. The
difference, however, is difficult to maintain in practice since the first process
tends to merge into the second.
In the typical degenerative type of change, as first described by Iwanoff
(1865), cystoid spaces usually arise where the supporting structure of the
retina is weakest, in the inner part of the outer nuclear layer and the inner
nuclear layer ; more rarely they occur in the ganglion-cell layer and the nerve-
fibre layer later as well (Kuhnt, 1881). At an early stage they frequently form
two parallel rows of small spaces in the two nuclear layers, but as they enlarge
they coalesce forming a single row, the individuals of which increase in size
so that eventually, as the true retinal layers gradually atrophy, Müller's
fibres become elongated by stretching, and finally the retina, which may
become two or three times its normal thickness, is represented merely by
pillars of drawn-out fibres, the ends of which are spread out on the two
limiting membranes. As the neuroglial skeleton is stretched apart, still
larger spaces are formed, while the denser partitions are pressed together
and consolidated to some extent by glial proliferation (Fig. 2363). Finally,
the outer and inner walls may break down so that a retinal hole is formed.”
The cysts arise by rarefaction of the nervous elements of the retina, and
are usually associated with degenerative changes in the capillaries (Iwanoff,
1 p. 2755.
DISEASES OF THE RETINA 27.53
1869; Berger, 1924) or neighbouring areas of choroiditis (Nettleship, 1872).
The contents of the cysts vary; the spaces usually appear empty, but
they may contain granular or degenerated material, and in the presence of
inflammatory processes, an albuminous exudate which forms histologically
a fibrous coagulum.
AEtiology. The causes of cystic degeneration are thus varied and
embrace practically the whole of ocular pathology. In general terms they
may be said to be due to the following factors:–
1. Senile degeneration, which in most cases is largely the result of vascular
Fig. 2363.-PERIPHERAL Cystic DEGENERATION of RETINA (Neame, T. O. S.).
sclerosis and deficient nutrition (Neame, 1920). As such it is most common
in the periphery.
2. Diseases of the Blood-vessels. In all cases of advanced vascular
disease cystic degeneration is common. It is invariably found in obstruction
of the central vein, in conditions of sclerosis, in the retinopathies (renal,
diabetic, circinate, cachectic, and so on), and in Coats and v. Hippel's
diseases.
3. As a degenerative change after traumatic or inflammatory disturbances
of the retina (Fig. 2422)."
4. In towic or infective conditions, such as irido-cyclitis or sub-acute
endophthalmitis when the most vulnerable region is at the macula.”
5. In ordematous conditions such as papilloedema, cystic degeneration
1 p. 2635. Fig. 2227. * Fig. 2259.

2754 TEXT-BOOK OF OPHTHALMOLOGY
is common but by no means constant, particularly in the circum-papillary
and macular regions.
6. In glaucoma cystic degeneration is common, particularly in the region
of the posterior pole.
7. In choroidal tumours, when advanced cystic degeneration is almost
invariably found in the overlying retina." To some extent this may be
a nutritional effect, but to a considerable extent it may be a toxic effect.
8. In detachment of the retina of any standing cystic degeneration is
universal (Fig. 2497), and the development of large individual cysts is
common.”
The great clinical interest of cystic degeneration, and particularly of
peripheral cystic degeneration, is the potential part which it plays in retinal
detachment. It will be seen 3 that it is probable that this event is frequently
preceded by a rupture of a cyst with the formation of a retinal hole, either by
a process of atrophy, or from stretching, or by a minor trauma in association
with a vitreous adhesion. It will be seen also that this sequence of events
may account to a large extent for the prevalence of retinal detachments in
senile and myopic eyes, and certainly pathological examinations of detached
retinae show this type of degeneration as one of their most common features *
(see Fig. 2480).
While cystic degeneration may occur anywhere in the retina, it shows
a predilection for the two places where this tissue is thinnest and least
efficiently vascularized—the periphery and the macula. Cystic degenera-
tion in the first of these sites is very common and has long been known ; in
the second, while its pathological identification is old, its recognition clinically
as a not uncommon occurrence is comparatively recent. A third relatively
common site is the peri-papillary region, particularly on the temporal side
of the disc.
PERIPHERAL CYSTIC (CYSTOID) DEGENERATION
This type of retinal degeneration occurs in a zone near the ora and is
a common phenomenon especially in senile and myopic eyes (Fig. 2364).
Cysts at the periphery of the retina were first described by Blessig (1855) who
considered them to be a normal occurrence ; Müller (1857) thought that they were a
post-mortem change ; and Iwanoff (1865–69), who first studied the condition minutely,
held that they were pathological phenomena due to retinal oedema (Iwanoff’s cystic
cedema), a view which finds supporters to-day (Casanovas, 1936). Merkel (1870), on
the other hand, considered them purely a senile manifestation, while subsequently
Nettleship (1872) regarded them as evidence of past inflammation of the choroid, calling
the condition “cystic disease of the retina.” Finally, Landsberg (1877) and Kuhnt
(1881) looked upon the condition as essentially a degenerative one affecting the nervous
elements of the retina, a view with which most later writers agree (Fuchs, 1921 ;
Hanssen, 1925; and others).
1 Fig. 2089. * p. 2810. 8 p. 2897. 4 p. 2875.
DISEASES OF THE RETINA 2755
Areas of cystoid degeneration usually lie too far in the periphery to be
seen ophthalmoscopically ; they rarely appear more than 7 mm. from the
ora serrata (Pressburger, 1929). The associated pigmented patches and
white atrophic areas, however, are frequently visible, and on careful examina-
tion the cysts can on occasion be recognized as isolated or confluent rounded
pores, separated by narrow partitions (Fig. 2367, Plate LV). Post-mortem,
when the retina has become opaque, they appear as dark areas in the grey
membrane (Ochi, 1927). They usually lie in irregular areas with zig-zag
outlines which do not run parallel to the ora, and the affected region is of
greatest breadth on the temporal side (Zeemann, 1912), that is, in the least
developed and most stretched zone of the retinal periphery.
Cystic degeneration occurs with great regularity to a well-marked
degree in the normal eyes of old people; it is the rule also in myopic
–º ---
_º-º-º: - -]
º- º -ºl ---
- º - -
---
Fig. 2364–PERIPHERAL Cystic DEGENERATIox of RETINA (Rones, Am. J. O.).
degeneration (Hanssen, 1925), in which condition it is not uncommon even
in comparatively early life (Ochi, 1927). Iwanoff (1865) found no instance
in 20 globes of children under 8, in 12% of patients between 20 and 40, and
in 50% of eyes between 50 and 80 years; but that it is by no means a purely
senile phenomenon is seen by the histological findings of Ochi (1927) who
found it not only in the eyes of myopes and the elderly, but three times in a
series of 15 children under 5 years of age whose death was due to various
causes. Salzmann (1912), indeed, considered that it frequently started in
early life, and it is possible that the first stages are commonly apparent
before the age of 20 years. It is to be remembered, also, that this region is
frequently attacked by mild infective processes, for here small areas of
chorioretinitis are a commonplace.
CYSTIC MACULAR DEGENERATION (HoNEY.comb MACULA): MACULAR HOLES
Like the periphery, the macular region is also prone to cystic degenera-
tion which shows the same pathological characteristics (Figs. 2365–66). At


27.56 TEXT-BOOK OF OPHTHALMOLOGY
first, cystic spaces are formed in the retinal substance which may be visible,
particularly with red-free light with which a typical honeycomb appearance is
Fig. 2365-SENILE Cystic DEGENERATIos Air Macula (Rones, Am. J. O.).
produced of small flecks in the central area (the vesicular macular aedema
of Nuel, 1908); eventually on rupturing these may produce a depression in
the central area if they are confined to the inner layers of the retina, or a
Fig. 2366–Cystic DEGENERATIox at Macula in Detachment
(Williamson-Noble, T. O. S.).
complete macular hole if the whole thickness of this tissue is involved (the
retinitis atrophicans of Kuhnt, 1900) (Figs. 2368–69, Plate LV).
The first cases of macular holes to be observed were isolated instances, the result
of oedema from contusions (Knapp, 1869 ; Noyes, 1871; Hoffmann, 1885; Hartridge


PLATE LV
SEcond ARY DEGENERATIows of THE RETINA
–
-
-
-
-
-
ºut- ------
Fig. 2368. - Cystic Fig. 2369.-Hole AT Fig. 2370. – Hole. At
DEGENERATIox AT MacULA (senile) (red- MACULA,
MACULA. free light).
Fig. 2371.-CIRCINATE RETINopathy.
[To face p. 2756.


DISEASES OF THE RETINA 2757
1889; Lawford, 1893); and our knowledge of their causation and pathology was
established by the publication almost simultaneously of 27 examples (Kuhnt, 1900;
Haab, 1900; Collins, 1900; Ogilvie, 1900). It was then recognized that retinal
vascular disease might be responsible (Kuhnt, 1900; Haab, 1900), while Fuchs (1901)
associated them with toxins derived from the anterior segment of the eye in irido-
cyclitis, a view upheld by de Schweinitz (1904), v. Hippel (1906), Coats (1907), Clapp
(1913), and others. Ogilvie (1900) assumed that the hole was formed mechanically
by contrecoup, a view upheld by Kipp (1908), but its occurrence in non-traumatic
conditions as well as its delayed appearance in traumatic cases subsequent to the
observation of Oedema show that this is not the universal mechanism ; that it may be
due to the effects of Oedema and was preceded by cystic formation was foreshadowed
by Fuchs (1901), substantiated by Reis (1906) and proved by Coats (1907). Cases
have indeed been reported wherein a hole has been observed to develop in a previously
healthy retina which was undergoing central degenerative changes (de Schweinitz,
1904; Crawford, 1933).
AEtiologically, therefore, macular cysts and holes may be divided into
two separate categories :—
1. Traumatic, the most common cause, especially after contusions to
the globe or the retention of foreign bodies. As we have seen, they have
long been known since their early description by Noyes (1871) (Haab, 1900;
Kuhnt, 1900 ; Fuchs, 1901 ; Pagenstecher, 1903; Dufour and Gonin,
1904; Reis, 1906; Purtscher, 1909; v. Szily, 1916 ; Lister, 1924; and
others). Salzmann (1919) found 8 in 2,400 war injuries, and Middleton
(1919) 23 in 100,000. They are usually due to a blow of considerable
severity on the anterior part of the eye, the macula being the point of contre-
coup, but they sometimes arise from laterally directed blows, the macula
suffering presumably because it is the thinnest point in the retina and is
unsupported by a capillary network. It is interesting that the diameter of
the usual hole (0-5 to 0-75 mm.) corresponds very closely with the avascular
area (0.4 to 0-5 mm.) (Leber, 1916). Sometimes the hole is seen very
shortly after the blow (Ogilvie, 1900; Quint, 1906; Reis, 1906; Noll,
1908), in which case it has been due to the direct effects of concussion. In
other cases there is an interval between the accident and the appearance of
the hole (Haab, 1900; Tweedmayer, 1907); in these cases a process of
cystic degeneration probably intervenes, the cyst rupturing later. It is
interesting that the formation of a macular hole or cyst has been seen in
eclipse blindness and after exposure to bright light (Harman and
MacDonald, 1922; Rauh, 1927; Würdemann, 1936).
2. Non-traumatic holes at the macula are less common ; in Haab and
Ogilvie's series they numbered 3 out of 27. It is known that they arise from
the rupture of a cyst, for the anatomical arrangement of the vessels in this
area makes the resorption of fluid difficult and the disintegration of degenera-
tive tissue easy. These non-traumatic cysts and holes may be due to two
general conditions —
(a) Degenerative, caused by
(i) senescence (Kuhnt, 1881–1900);
27.58 TEXT-BOOK OF OPHTHALMOLOGY
(ii) cardio-vascular disease (Haab, 1900; Küsel, 1906; Zentmayer,
1909; Moore, 1910), chorio-retinitis (Pagenstecher, 1875; Zeemann, 1912),
pigmentary degeneration of the retina (Nuel, 1896; Stock, 1908; Vogt,
1925), renal retinopathy (Reis, 1906), retinal detachment (Fuchs, 1918),
and other degenerative conditions.
(b) Toric, particularly from the effect of toxins in the vitreous body
derived from inflammatory processes in the anterior segment (Fuchs, 1901;
de Schweinitz, 1904; Zeemann, 1923; Vogt, 1925; Arnold, 1929; Samuels,
1930; Wolff, 1931; and others).
The clinical picture presented by macular cysts is sometimes difficult
to distinguish, and they are most easily recognized by red-free light which
accentuates the honeycomb appearance and
renders their inner wall visible (Vogt, 1925–34):
it is for this reason that, while the condition has
long been known histologically, it was not until
the observations of Vogt in 1925 that it became
recognized as a common clinical entity (Fig. 2368,
Plate LV). When a hole is formed there is a
circular dark red spot at the fovea, the appearance
of which suggests forcibly that the retina has
been “struck out with a punch" (Noyes, 1871) so
that one looks at the dark red finely granular
Fig. 2372–Macular Hour, surface of the choroid (Figs. 2370, Plate LV and
2372). The proof of the type of lesion is found
in the phenomenon of parallax, but sometimes by careful focusing of the
ophthalmoscope or by the use of the slit-ophthalmoscope, a reflex over the
"hole" may be obtained, indicating the persistence of the inner wall of a
cyst. The edges are usually clearly cut but are occasionally ragged, and it
may be surrounded by an indistinct halo or corona with radiating white
streaks indicating cedema of the surrounding retina. It is usually about
one-quarter or one-third the size of the disc, but can be as large as this
structure (Collins, 1900) or even larger (Foster Moore, 1910), and excep-
tionally it is situated somewhat eccentrically (Pagenstecher, 1903). Once
formed it remains permanently (42 years, Harman, 1901); but sometimes
a retinal detachment, which may remain localized and shallow, may develop
(Noyes, 1871; Haab, 1900; Ogilvie, 1900; Fuchs, 1901; and others).
The pathology of the condition is now fully understood. The early
investigations of Pagenstecher and Genth (1875), Haab, 1900), Fuchs (1901–
18), Murakami (1902); Spicer and Parsons (1902); and v. Hippel (1906),
were followed by the classical paper of Coats (1907), which subsequent
observations have confirmed (Zeemann, 1912–23; Alt, 1913; Williamson-
Noble, 1922; and others). The essential change is the presence of cystic
spaces full of oedematous fluid situated especially in the outer plexiform
layer, more particularly in Henle’s layer, in the foveal region (Fig. 2373),

DISEASES OF THE RETINA 27.59
together with a collection of fluid between the external limiting membrane
and the pigment layer, producing a shallow detachment. Occasionally some
retinal elements remain on the walls of such a cyst, a feature which, as we
have seen, can sometimes be seen ophthalmoscopically and which may
Fig. 2373. GEDEMATous Cystic SPACEs AT THE MACULA,
An early stage of hole-formation; slightly to one side of the macula seen in
Fig. 2374 : cysts in the inter-nuclear layer. In a high myope (x 32) (Coats).
explain the appearance of a hole without a complete central scotoma
(Collins, 1900); at other times the internal limiting membrane alone may
survive; but eventually degeneration may be complete and a central gap
results (Fig. 2374). At the edges of the gap the retinal layers may end
º a
- º
Fig. 2374.—MACULAR Holes.
The centre of the fovea of the retina seen in Fig. 2373 (x 32) (Coats).
cleanly and abruptly, but in some cases oedematous cystic spaces are found in
the surrounding area. There is no interruption of the inner layers of the
choroid which remain perfectly normal in the central area.
The formation of a macular hole is of course disastrous to central vision
and the disability remains as a permanent defect.


2760
TEXT-BOOK OF OPHTHALMOLOGY
Alt. Am. J. O., xxx, 97, 1913.
Arnold. A. f. O., cxxii, 299, 1929.
Berger. K. M. Aug., lxxii, 837, 1924.
Blessig. Diss., Dorpat., 1855.
Casanovas. A. de Oft. H.-A.,
1936.
Clapp. O. Rec., xxii, 79, 1913.
Coats. R. L. O. H. Rep., xvii, 69, 1907.
Collins. R. L. O. H. Rep., xiii, 65, 1890.
T. O. S., xx, 196, 1900.
Crawford. A. of O., x, 793, 1933.
Dufour and Gonin. Encyclopaedia fr. d’O.,
vi, 938, 1904.
Falchi. A. f. O., xli (4), 187, 1895.
Fuchs, E. Z. f. Aug., vi, 181, 1901.
A. f. O., lxxix, 42, 1911; xcvii, 57, 1918;
cv, 333, 1921.
Haab. Z. f. Aug., iii, 113, 1900.
xxxvi, 239,
Hanssen. K. M. Aug., lxxiv, 778 lxxv, 344,
1925.
Harman. T. O. S., xxi, 88, 1901.
Harman and MacDonald. Brit. Med. J., i, 637,
1922.
Hartridge. T. O. S., ix, 144, 1889.
v. Hippel. A. f. O., lxiv. (1), 172, 1906.
Hoffmann. K. M. Aug., xxiii, 40, 1885.
Iwanoff. A. f. O., xi (1), 135, 1865; xv (2),
88, 1869.
Ripp. T. Am. O. S., xi, 518, 1908.
Enapp. A. f. Aug., i, 22, 1869.
Kuhnt. K. M. Aug., xix, 1881.
Z. f. Aug., iii, 105, 1900.
Rüsel. K. M. Aug., xlvi (2), 464, 1906.
Landsberg. A. f. O., xxiii (1), 193, 1877.
Lawford. R. L. O. H. Rep., xi, 206, 1886.
T. O. S., xiii, 76, 1893.
Lawson. Path. S. Trans., xix, 362, 1869.
Leber. G.-S. Hb., II, viii.A., 1916.
Lister. Brit. J. O., viii, 1, 1924.
Merkel. U. d. Macula d. Menschem w. d. Ora
eines Wirbelthieres, Leipzig, 1870.
Middleton. Am. J. O., ii, 779, 1919.
Moore, Foster. T. O. S., xxx, 155, 1910.
Müller. Z. wiss. Zoo., viii, 1, 1857.
Murakami. A. f. O., liii (3), 439, 1902.
Neame. T. O. S., 161, 1920.
Nettleship. R. L. O. H. Rep., vii, 343, 1872.
Noll. A. f. Aug., lx, 254, 1908.
Noyes. T. Am. O. S., viii, 128, 1871.
Nuel. A. d’O., xvi, 164, 1896 ; xxviii, 737,
1908.
Ochi. Am. J. O., x, 161, 1927.
Ogilvie. T. O. S., xx, 198, 202, 1900.
Pagenstecher. Atlas path. Anat., Wiesbaden,
xxviii. 6, 1875.
A. f. O., lv. (1), 135, 1903.
Pagenstecher and Genth. Atlas d. path. Amat.,
Taf. xxv, 1875.
Pressburger. Z. f. Aug., lxviii, 331, 1929.
Purtscher. Z. f. Aug., xxii, 215, 1909.
Quint. K. M. Aug., xliv, (2), 134, 1906.
Rauh. Z. f. Aug., lxiii, 48, 1927.
Reis. Z. f. Aug., xv, 37, 1906.
Salzmann. Anat. w. Phys. d. Aug., Wien.,
84, 1912.
Wien. med. W., i, 281, 1919.
Samuels. A. of O., iv, 477, 1930.
de Schweinitz. T. Am. O. S., x, 228, 1904.
O. Rec., xiii, 285, 1904.
Sourdille. Bull. S. fr. d’O., xlv, 236, 1932.
Spicer and Parsons. T. O. S., xxii, 1902.
Stock. K. M. Aug., xlvi (1), 225, 1908.
v. Szily. B. O. G. Heidel., 1, 135, 1916.
Twietmeyer. Z. f. Aug., xviii, 447, 1907.
Vogt. Münch, med. W., lxxii, 1101, 1925.
K. M. Aug., lxxxiv, 305, 1930; xcii, 743,
1934.
Williamson-Noble. Brit. J. O., vi, 67, 1922.
T. O. S., xciii, 287, 1923.
Wolff. P. R. S. Med., xxiv, 1617, 1931.
Würdemann. Am. J. O., xix, 457, 1936.
Zeeman. A. f. O., lxxx, 259, 1911–12
cxii, 151, 1923.
Zentmayer. O. Rec., xviii, 198, 1909.
CIRCINATE DEGENERATION
A very typical picture, originally described by Jonathan Hutchinson
(1876) as “symmetrical central choroido-retinal disease occurring in Senile
persons,” and referred to by Goldzieher (1887) as “ Hutchinson’s changes,”
by de Wecker and Masselon (1891) as “degenerescence graiseuse ’’ and again
by de Wecker (1894) as “degenerescence blanche,” was studied clinically in
detail by Fuchs (1893), who gathered 12 cases met with in 70,000 patients
and named the condition RETINITIS
CIRCINATA. The condition occurs
typically, but not invariably, in old people, and is characterized by a girdle
of bright white spots in the deeper retinal layers around the central area and the
development of degenerative changes at the macula.
It runs a chronic course
and has a bad prognosis; and since it is entirely non-inflammatory and
DISEASES OF THE RETINA 2761
degenerative in nature, the term degeneration is more appropriate than
Fuchs's original name retinitis.
On the aetiology of the condition authorities are by no means in accord,
nor is it universally agreed to be a clinical entity ; but the views of the
majority follow the opinion originally propounded by de Wecker and
Masselon (1891) and substantiated by the histological investigations of
Ammann (1897) that the changes are primarily due to haemorrhages occurring
in the outer plexiform layer of the retina, that is, the outermost region to
which vascularization extends. The peculiar and characteristic arrange-
ment was explained by Nuel (1896) as being determined by the arrangement
of Henle’s fibres. In this view the condition is related to the external
haemorrhagic retinitis of Coats, in which, however, the haemorrhage breaks
into the sub-retinal space and organizes there (Fisher, 1910). There is no
doubt that a large number of cases are associated with haemorrhages,
many are preceded by them, and most are complicated by them in their
evolution. Goldzieher (1887–97), however, held that the haemorrhages were
secondary, while Fuchs (1893), Gunn (1898), and Leber (1916) considered
that the white spots were not the remains of degenerated blood but
represented rather coagulated albuminous exudate or the result of tissue
degeneration and proliferation, somewhat similar to that found in album-
inuric retinopathy. This, it has been seen, is essentially due to oedema ;
hence Siegrist's (1896) term for the disease : aedema retinae eacternum centrale.
It cannot be said to be proven that all the changes are purely haemorrhagic
in origin, a point which must await further elucidation when more
pathological evidence is available ; but that they are all essentially
degenerative is certain.
The cause of the haemorrhage or degeneration is still more obscure. A
large proportion of cases occurs in senile arteriosclerotics (Goldzieher,
1887–97; Nuel, 1896; and many others); thus Heinricy and Harms (1913),
reviewing 52 cases in the literature, found that 75% suffered from arterial
disease : 5 had cardio-vascular disease, 24 arteriosclerosis, 6 diabetes,
3 syphilis, 3 anaemia, 1 had leukaemia, and only 9 were free from obvious
general disease. Clausen (1922) ascribed the changes to embolism and
Junius (1932–34) to miliary aneurysms. It would seem, however, probable
that, especially in view of its occasional occurrence in young people, some
other factor, probably toxic, either of an infective or metabolic type, must
be hypothecated, which acts usually and most markedly in the presence of
vascular sclerosis, but sometimes in its absence (Scarlett, 1929; Edgerton,
1936 ; and others). Fisher (1910) found associated disturbances such as
anaemia, menstrual disorders and epistaxis in one of his youthful patients.
The close dependence of the disease on vascular conditions is suggested by
the appearances which frequently accompany it. Thus haemorrhages in the
other eye have been noted (Fisher, 1910), it has been seen with angiomatosis
(Junius, 1932–34), and exudative retinitis (Coats, 1908); and parallel
27.62 TEXT-BOOK OF OPHTHALMOLOGY
disturbances in the choroidal circulation have been indicated by the
associated occurrence of senile macular degeneration (Fisher, 1910;
Seefelder, 1929) or senile exudative choroiditis (Coppez and Danis, 1926;
Kahler and O'Brien, 1935) (Fig. 1787). It has also been seen to develop
some time after the occurrence of a choroidal haemorrhage (Sattler, 1930).
Indeed, in the opinion of some, the changes are primarily more choroidal than
retinal in origin, a view which can receive support both on clinical (Knapp,
Fig. 2375-Circinate DEGENERAtion of THE RETINA.
Showing degenerative changes at the macula (McMullen, T. O. S.).
1907; Hepburn, 1908–24: Evans, 1933) and pathological grounds
(Ammann, 1897; Seefelder, 1929).
Whatever the precise aetiology, the disease occurs preferentially in old
persons; in 80 cases in the literature Heinricy and Harms (1913) found the
average age to be 53 years. It does occur, however, in the young: 17 years
(de Wecker, 1894), 16 (Hoor, 1900), 12 (Peters, 1896), soon after birth and
considered to be congenital (de Wecker, 1894). Females are affected more
than males in the proportion of 3: 2. Most cases are bilateral, although the
degeneration may start in the second eye long after the first has been
established (Doyne and Stephenson, 1904); in 80 cases in the literature
Heinricy and Harms (1913) found 57 unilateral and 23 bilateral.
The clinical picture in a typical case is characteristic and comprises two
phenomena—changes in the macular region and a surrounding girdle of

DISEASES OF THE RETINA 2763
white spots (Figs. 2371, Plate LV and 2375). At the macula agrey or yellowish
discoloration is seen, occasionally cedematous changes suggesting a macular
detachment, and eventually changes resembling senile degeneration appear.
Around the central area, usually between the temporal vessels, there is a ring
of glistening, brilliantly white, sharply defined dots. The girdle may be
complete or not ; it may be made up of two arches of horizontal arcs open
-
---
º -
- ---- -
º - - - - -
-- - -- --- - -- -- --- - -
- - - - -- --
- - - - --- -*. --
----e. -- sº *sº - -
ºs - "º - *s - -
- - -
- º º ****
--- º 2*
-
Fig. 2376.-CIRCINATE DEGENERATION OF THE RETINA.
Section through girdle of exudate between dise and macula. S. Selera.
Ch. Choroid showing round-celled infiltration of vessels. B. Bruch's membrane.
R. Retina. P. Cells of pigmentary epithelium undergoing fatty degeneration
(Seefelder, A. f. Aug.).
at the temporal or nasal ends, or be open above and below, or be quite
irregular. The dots of which it is composed may coalesce to form
large irregular patches; but there is no pigmentation, no raising of the
retina, and the vessels pass over them undisturbed. Quite frequently the
picture is complicated by the appearance of a small or a large haemorrhage,
and as evolutions progress the entire central area becomes degenerated and
white, while after many years the exudative appearance may become
massive and extensive (Handmann, 1937). Occasionally an atypical
distribution occurs, such as ring-shaped exudates in the periphery or white

27.64 TEXT-BOOK OF OPHTHALMOLOGY
deposits on the nasal side of the disc, but these appearances are rare (Fisher
1910; Heinricy and Harms, 1913; Bachstez, 1913; Yano, 1921 ; Batten,
1922).
The pathology of the condition has unfortunately been little elucidated
since these eyes are rarely available for examination (Fig. 2376). Only
three cases have been reported. In the first of these, which was a glaucoma-
tous eye, Ammann (1897) found hyaline masses in the inter-nuclear layer
which showed all stages of transformation from disintegrating erythrocytes;
there were no signs of inflammation and the condition seemed obviously a
haemorrhagic degeneration. There were present numbers of large mono-
nuclear bladder-cells filled with fatty globules, resembling those present in
exudative retinitis and renal retinopathy.” A second eye which had a
melanotic tumour of the iris, was examined by Morax (1926); he found
masses of exudate similar to that characterizing renal retinopathy in the
internal plexiform layer and the internal nuclear layer as well as much
localized arteliosclerosis. Seefelder (1929) reported a third case in which
there was senile macular degeneration in the fellow eye. He concluded that
the girdle was composed of granular fatty cells consisting of large numbers
of swollen bladder-cells “acting as macrophages derived from the pigmentary
epithelial and glial cells. He considered, however, that these changes were
secondary to those at the macula, where there was advanced cystoid
degeneration with complete disappearance of the outer layer and the layer
of rods and cones. The pigmentary epithelium was also degenerated, and
between it and the choroid was deposited fibrous tissue. In the choroid
itself there was lymphocytic infiltration and arteriosclerotic changes, to
which, indeed, Seefelder considered the retinal changes largely secondary.
The symptoms are confined to a gradual and progressive decrease in
central vision. In the early stages disturbances of the colour fields may be
discovered (Galeazzi, 1932), but eventually a central scotoma develops,
sometimes associated with a ring scotoma (Vito, 1931). The central
scotoma is permanent, but the peripheral fields always remain unimpaired.
The prognosis is, therefore, bad, and although occasionally the exudative
deposits may diminish or sometimes even disappear (Fuchs, 1893; Krükow,
1900; de Schweinitz, 1903; Doyne, 1906; Fisher, 1910; Harman, 1912;
and others), these cases are exceptional, and, if the disease has advanced
to any extent, the central vision is irreparably lost.
Treatment is most unsatisfactory, and apart from correcting or com-
bating any cardio-vascular or metabolic disorder, little can be done.
Iodides have been given from time immemorial more in the spirit of faith
than from any demonstrable value ; and sometimes mercury. It is pro-
bably of more importance to seek for and eliminate foci of toxaemia, especially
dental or intestinal streptococcal infections. Sub-conjunctival injections of
irritating substances such as concentrated saline or systemic injections of
1 p. 2653. * p. 2720. * p. 2631.
DISEASES OF THE RETINA
2765
vaso-dilatators as acetyl choline (Redslob, 1934) have been advocated, but
their value is very questionable.
Ammann. A. f. Aug., xxxv, 123, 1897.
Baehstez. K. M. Aug., li (2), 232, 1913.
Batten. P. R. S. Med., xv, O. Sect., 27, 1922.
Clausen. K. M. Aug., lxix, 844, 1922.
Coats. R. L. O. H. Rep., xvii, 440, 1908.
Coppez and Danis. A. d’O., xliii, 461, 1926.
Doyne. Ophthalmoscope, iv, 384, 1906.
Doyne and Stephenson. T. O. S., xxiv, 91,
1904.
Edgerton. Am. J. O., xix, 463, 1936.
Evans. Brit. J. O., xvii, 257, 1933.
Fisher. T. O. S., xviii, 167, 1898.
R. L. O. H. Rep., xviii, 37, 1910.
Fuchs. A. f. O., xxxix (3), 229, 1893.
Galeazzi. Boll. d’Oc., xi, 870, 1932.
Goldzieher. Wien, med. W., 861, 1887.
Cb. pr. Aug., xiii, 364, 1889.
A. f. Aug., xxxiv, 112, 1897.
T. Am. O. S., xiv, 753, 1915.
Gunn. T. O. S., xviii, 171, 1898.
Handmann. K. M. Aug., xcviii, 618, 1937.
Hepburn. T. O. S., xxviii, 255, 1908; xxxii,
361, 1912.
Brit. J. O., viii, 401, 1924.
Hoor. Z. f. Aug., iii, 26, 1900.
Hutchinson. R. L. O. H. Rep., viii, 231, 1876
Junius. A. f. Aug., cvi, 475, 1932.
K. M. Aug., xcii, 748, 1934.
Kahler and O’Brien. A. of O., xiii, 937, 1935.
Knapp. K. M. Aug., xlv. (1), 171, 1907.
Krükow. Oph. Congress, Moscow, 1900.
Leber. G.-S. Hb., II, vii (A2), 1242, 1916.
Morax. Am. d’Oc., clxiii, 801, 1926.
Nuel. A. d’O., xvi, 473, 1896.
Peters. B. O. G. Heidel., xxv, 94, 1896.
Redslob. Bull. S. d’O. Paris, xlvi, l 19, 1934.
Sattler. K. M. Aug., xc, 190, 1930.
Scarlett. Am. J. O., xii, 633, 1929.
de Schweinitz. O. Rec., xii, 71, 1903.
Seefelder. A. f. Aug., c-ci, 334, 1929.
Siegrist. B. O. G. Heidel., xxv, 83, 1896.
Vito. A. di Ott., xxxviii, 180, 1931.
Harman. T. O. S., xxxii, 105, 1912. de Wecker. A. d’O., xiv., 1, 1894.
Heinricy and Harms. A. f. O., lxxxvi, 514, de Wecker and Masselon. Opht. Clin., Paris,
1913. 230, 1891.
Yano. Am. J. O., iv, 719, 1921.
C. Primary Degenerations
In a general sense the primary degenerations of the retina are associated with
disturbances of the pigmentary epithelium, and for them Leber (1916) used the term
TAPETO-RETINAL DEGENERATIONS, including thereunder the so-called retinitis pig-
mentosa with and without pigment, retinitis punctata albescens, chorio-retinal degenera-
tions and scleroses as gyrate atrophy, choroideremia, and central and peri-papillary
sclerosis, as well as congenital night-blindness without ophthalmoscopic evidences.
1. PRIMARY PIGMENTARY DEGENERATION OF THE RETINA
(RETINITIs PIGMENTOSA)
PRIMARY PIGMENTARY DEGENERATION OF THE RETINA is a disease of
unknown aetiology but with a definitely hereditary tendency, characterized
by a chronic course and a progressive habit, which, although rarely
manifest at birth, becomes apparent in childhood and frequently results in
blindness in middle or advanced life. Pathologically it is essentially a
degeneration of the retinal neuro-epithelium, particularly of rods, and
clinically its main features are an attenuation of the retinal vessels, optic
atrophy and, almost invariably, widespread pigmentary changes in the retina.
In so far as it is a purely degenerative condition without inflammatory involve-
ment, the usual term retinitis pigmentosa is not happily chosen. As we have
2766 TEXT-BOOK OF OPHTHALMOLOGY
seen, a very similar condition follows inflammatory diseases of the retina
and choroid, particularly syphilitic, and these are best termed secondary
pigmentary degenerations of the retina (Fig. 2264)."
Historical. The association of visual failure with the occurrence of pigmentary
flecks in the retina was known in pre-ophthalmoscopic days (Schön, 1828; v. Ammon,
1838), and almost as soon as the ophthalmoscope was introduced the clinical picture
was described by van Trigt (1853) in Holland. Arlt (1856) classed the condition as
congenital hemeralopia, and in the same year v. Graefe (1856) studied it fully, drawing
attention to the clinical features, the symptoms and the field defects. Since his time
the disease has been the subject of perennial interest partly on account of the
hereditary problems involved, partly on account of the difficulty experienced in eluci-
dating its pathology, and partly because of the perpetual enigma of its causation.
Heredity. Since Liebreich (1861) drew attention to the prevalence of
pigmentary degeneration of the retina in certain families and discovered
that the subjects of the disease were often the children of cousins, the
hereditary aspects of this condition have excited a very great deal of interest
and have stimulated an enormous literature. The most authoritative study
of the early literature and collection of data was provided by Nettleship
(1907–08) who found in 976 families in which the disease occurred evidence
of its hereditary transmission in about half the cases, without consanguinity
in 230 instances (23.5%), with consanguinity in 226 (23%), and with both
heredity and consanguinity in 32 (3.5%). A further elaborate study of
40 pedigrees was made by Usher (1913), and a survey of the literature by
Bell (1922). Indeed, so frequently is it hereditary that it can be accepted
as certain that it is so in many cases where proof is wanting and superficial
Questioning reveals no indications of its incidence in the immediate
precedents. -
The literature on the whole matter is somewhat confused. The earliest investi-
gators stressed consanguinity particularly (Liebreich, 1861 ; Derby, 1865; Bader,
1876; and others), at a later date heredity appeared to be the preponderating influence :
thus Webster (1878) found consanguinity in 13.6%, heredity in 31.8%, Ayres (1886)
consanguinity in 20% and heredity in 40%, Snell (1902–07) found no influence of
consanguinity, Nettleship (1907–08) found the two factors about equally potent,
while Scheurlen (1935) in an extensive investigation of 506 cases near Tübingen since
1877, in a region where inter-marriage is frequent, found that consanguinity did not
play a decisive rôle.
These differences of opinion are due to the diversity of the hereditary
pattern in which pigmentary degeneration may appear, for in different
pedigrees it may appear as a dominant, a recessive, or as a sex-linked trait
(Allan, 1937). The first is rare and appears in the larger and more heavily
affected families, the second is the most common, and the third is rarer.
As a dominant characteristic both sexes transmit and are affected equally
(Fig. 2377); in the second case the parents transmit recessive traits jointly,
1 p. 2662.
DISEASES OF THE RETINA 2767
consanguinity plays a potent rôle in determining its incidence, and the sexes
are equally affected (Fig. 2378); and as a sex-linked character the disease is
transmitted by females and appears in males only (Fig. 2379). This accounts
for the sex incidence of the disease wherein the proportion of males is higher
than that of females.
I o
|
II § {
— |
III * Q Q @
IV d º ! !
v ill
v. sº sº
FIG. 2377.-PRIMARY PIGMENTARY DEGENERATION OF RETINA.
Dominant heredity (Nettleship).
From the eugenic point of view the important fact emerges that no
reliable advice can be given as to the possibility of transmission to the next
generation until the nature of the pedigree has been worked out in each
individual case. When it appears as a
dominant characteristic each affected 1 Q Cſ
individual gets the disease directly from H
one parent and the chances are that it will II O Öſ
be passed on to half the children. Unless +
it is considered right to produce children |
who will eventually become blind, these III Q
people should have none. When it ſ T —
appears a simple recessive the prohibi- IV e º de
+ +-
tion of inter-marriages within the same
pedigree or into a family carrying the trait
becomes imperative, and if they do occur
they should be sterile. When it is .
inherited as a recessive sex-linked character half the sisters and all the
daughters of the affected man will tend to pass the disease to half their
sons; the responsibility therefore rests upon the women of the family.
FIG. 2378.-PRIMARY PIGMENTARY
DEGENERATION OF THE RETINA.
Recessive heredity (Nettleship).
In this connection it is well to remember that as a rule, and in England especially,
the families tainted with pigmentary degeneration are unusually prolific. Thus
Nettleship (1907–08) found amongst 195 affected childships an average of 9 children
to each marriage, the subjects of the disease itself having many children despite the
economic difficulties imposed upon them by their disability. In these circumstances
there is little likelihood of the disease dying out by a process of natural selection. On
T.O. — WOL. III. X X

2768 TEXT-BOOK OF OPHTHALMOLOGY
the other hand in Germany, Scheurlen (1935) found among 506 cases in a relatively
stable population that the affected numbers remained almost stationary over 50
years owing to the fact that a very large proportion of the diseased persons had no
descendants.
It is interesting that a condition precisely similar in its clinical characteristic
course and microscopical anatomy was cultivated in a strain of rats by Bourne, Camp-
bell and Tansley (1938) in which it was transmitted as a Mendelian recessive.
Associated Degeneracies. Other defects of a degenerative type are
frequently met in the genealogies: myopia, deafness, mutism, mental

(S) = Myopia.
€ = Epilepsy.
FIG. 2379.-PRIMARY PIGMENTARY DEGENERATION OF THE RETINA.
Sex-linked heredity (Usher).
inferiority or insanity, epilepsy and so on (Nettleship, 1907–08; Usher,
1913; and others). More widespread central nervous disease may occur,
such as disseminated sclerosis (Riechert, 1933), while a most interesting
feature is the occasional occurrence of widespread constitutional and
skeletal evidences of pituitary dys-function.
The association of pigmentary retinal degeneration, usually of an atypical
type, with obesity, polydactyly, hypogenitalism and mental retardation—the
whole clinical picture constituting the LAURENCE-Moon-BIEDL syNDROME–
was first noted in the classical paper of Laurence and Moon (1866) (Fig. 2380).
The condition is familial, the inheritance being of the recessive Mendelian
type (Cockayne, Krestin and Sorsby, 1935; Savin, 1935). The members of
the original family described finally developed paraplegia (Hutchinson, 1882–
1900; Nettleship, 1908) but this seems to have been a peculiar feature ;
DISEASES OF THE RETINA 2769
polydactyly, which was first noted by Höring (1865), Stör (1865) and de
Wecker (1868), in which supernumerary fingers and toes are more usual than
additional thumbs, is more common (Biedl and Raab, 1922–24; Cockayne,
Krestin and Sorsby, 1935; Casini, 1935); and obesity, hypogenitalism and
FIG. 2380.--THE LAURENCE-Moon-BIEDL SYNDRom E.
See Fig. 2396 (Savin, Brit. J. O.).
mental retardation are constant (Bardet, 1920; de Schweinitz, 1923;
Hansell, 1924; Velhagen, 1932; Avery and Sorsby, 1936).
An association with total colour-blindness or a high degree of colour-defect is
rare (Nettleship, 1909; Klainguti, 1923; Waardenburg, 1930; Pillat, 1930; Usher,
1935). Friedreich's ataxia (Kapuscinski, 1934), cerebellar ataxia (Biemond, 1934),
pigmentation of the skin (Casini, 1935) and other skeletal and organic anomalies have
been reported. The genetics of the condition have been fully discussed by Strieff
and Zeltner (1938) who have published all the available pedigrees in full, and the
literature has been fully summarized by Cockayne, Krestin and Sorsby (1935) and
Sorsby, Avery and Cockayne (1939).
Incidence. As we have seen, the determining factor in the incidence of
primary pigmentary degeneration is heredity, and owing to the proportion
of sex-linked cases, males are more frequently affected than females.
Nettleship (1907–08) and Wilmer (1924) give a proportion of 3:2;
Yokoyama (1933) of 63% and Usher (1935) of 58%. Little is known of
predisposing causes, although the initiation or an acceleration of symptoms
has been associated with severe illnesses as a massive loss of blood, the
acute exanthemata (Nettleship, 1907–08), malaria (Smith, 1904), Vincent's

X x 2.
2770 TEXT-BOOK OF OPHTHALMOLOGY
angina (Henderson, 1934), or the development of pregnancy (Henderson,
1934). There is no substantial evidence that syphilis or tubercle has any
particular effect in the aetiology.
The disease is almost invariably bilateral and usually both eyes are
equally affected. Exceptional cases of unilaterality, however, have appeared
in the literature (Pedraglia, 1865; de Wecker, 1868 ; Baumeister, 1873;
Derigs, 1882; Günsburg, 1890; Gonin, 1902; Nettleship, 1907–08; Hine,
1924; Rossi, 1926; Shoji, 1926; Beigelman, 1931; Aebli, 1938; Agatston,
1939). So rare is this occurrence, however, that some authorities suggest
that if sufficiently long observations were made, the second eye would
eventually become affected, or alternatively, that these cases are secondary
in nature. The observations of individual patients over considerable
periods, however, (6 years, Beigelman, 1931; 10 years, Shoji, 1926) when the
other eye was still normal, and the absence of other discoverable causes
indicate that these rarities do occur.
The clinical picture presented by a typical case of retinitis pigmentosa
is characteristic (Fig. 2381, Plate LVI). The two essential features are the
attenuation of the retinal vessels and the pigmentary changes, and of these the
first is constantly present while the second, although usually the most
prominent feature, may be very limited in degree or even completely absent.
The vessels may be extremely small, particularly the arteries which, especially
in the later stages of the disease, become thread-like ; while the disc becomes
a pale waxy colour and quite obviously atrophic. The pigment becomes
aggregated into characteristic jet-black accumulations of a spidery shape
resembling bone-corpuscles, and has obviously migrated into the retina
itself since it is seen particularly clustered around the retinal veins. As this
migration of pigment progresses the pigmentary epithelium becomes decolor-
ized so that the fundus is tessellated and the choroidal vessels come into
view. In the typical early case the spots are few and appear first in a
zonular girdle in the equatorial region, the retina in the extreme periphery
and in the central area remaining normal ; but as the disease advances
they increase in number and in size, the affected zone growing slowly
broader anteriorly and posteriorly until eventually almost the entire retina
may be thickly studded with pigmentary masses. Alternating widespread
disappearance of the pigmentary layer and tapeto-retinal atrophy may be
much in evidence (Fig. 2382, Plate LVI).
Other changes of a degenerative nature are frequently present in the eyes. Myopia
is frequent and sometimes it is of high degree. A posterior polar cataract of the com-
plicated type frequently develops eventually, and although its onset is usually late
and its progress extremely slow, it may cause a considerable degree of disability owing
to the smallness of the visual field : it is an evidence of malnutrition of the lens,
which is also possibly deleteriously affected by the accumulation of toxic materials
in the vitreous as a result of the gross impairment of the retinal circulation. The
occurrence of glaucoma, which, however, is not common, may be associated with the
same circumstances. A rare complication is the development of degenerative cysts at
PLATE LVI
PRIMARY DEGENERATIows of THE RETINA
Pig. 2381.--Typical, PRIMARY DEGENERA- Fig. 23.82.-PRIMARY PIGMENTARY
TIox of THE RETINA. DEGENERATION.
With much tapeto-retinal atrophy.
Fig. 2383.−RETINITIs Pigmentosa ALBEscENs.
Fig. 2384.—HEREDITARY MACULAR Fig. 23.85.-AMAurotic IDIocy.
DEGENERATION.
[To face p. 27.70.



DISEASES OF THE RETINA
FIG. 2390. FIG. 2391.
FIGS. 2386–91.-FIELDS OF PIGMENTARY DEGENERATION of RETINA.
Showing progressive stages of deterioration.

2772 TEXT-BOOK OF OPHTHALMOLOGY
the macula (Nuel, 1908; Stock, 1908), and an exceptional one the appearance of a
macular hole (Ginsberg, 1908 ; Perera, 1938); while drusen have been noted on the
disc 1 (Fig. 2635, Plate LXII).
Symptoms. Two characteristic symptoms are associated with pigmen-
tary degeneration : might-blindness and a progressive limitation of the visual
field which eventually becomes total. Defective vision in the dusk is usually
the first disability noticed, and it may be present for several years before
pigmentation of the retina becomes clinically visible. The symptom is
usually noted in early youth and as age advances the patients become
more and more incapacitated in the dusk. Only occasionally is there
no mention of any disability at night, and it is probable that this occurs in
those cases in which there remains a wide belt of healthy retina in the
periphery.
The characteristic feature of the visual field is the development of an
annular or ring-shaped scotoma corresponding to the degenerated zone of the
retina. This feature has been known since early ophthalmoscopic days
(v. Graefe, 1856–58), and, although it may escape detection unless carefully
sought, it is almost invariably present in some form or other (Baas, 1897;
Crzellitzer, 1900; Gonin, 1901–02 ; Hancock, 1906; Hepburn, 1908–38)
(Figs. 2386–91). The scotoma which may form a partial or complete ring or
may be very irregular, always occupies the equatorial region, and may
contain islets of functional areas in its midst. These may be confluent
so that a double ring scotoma results ; or conversely, small additional
scotomata may be located in the functioning areas. As the disease pro-
gresses it broadens both anteriorly and posteriorly, the field disappearing
Quadrant by quadrant, an area of the lower temporal periphery usually
lingering the longest, until a small portion only of the central region around
the fixation spot is left. This does not seem to diminish perceptibly under
ordinary illumination until the extreme periphery has entirely lost its
function, and it may remain intact for years. So long as any peripheral
field persists, therefore, the patient can be reassured that his central vision
is not yet in immediate danger. Even then central vision, if not normal,
may be quite good, but the patients grope about helplessly under the very
great disability of seeing only the one small area on which fixation is
directed (tube vision). Eventually, however, the central vision also tends to
disappear and all perception of light is lost.
The clinical course is slow, chronic, and progressive, but is very
frequently interrupted by remissions during which the visual acuity and the
fields improve, a happening which has too often been interpreted as being a
response to some particular line of treatment. Although the degenerative
tendency is transmitted and is undoubtedly present at birth, it is exceptional
for the disease to be evident at that time ; cases, however, have occurred
1 p. 3062.
DISEASES OF THE RETINA 2773
when children have been born blind or have gone blind in early infancy
(Hutchinson, 1866; Leber, 1869), and night-blindness is commonly found
as early as such a symptom can be elicited. In the great majority of cases
pigmentation is clinically evident between the ages of 3 and 8 years (Höring,
1864), at the end of which time it may be quite advanced. Usually serious
symptoms begin to be apparent in school life (6–12 years), and by 20 years of
age they may begin to be incapacitating. The age at which blindness
occurs varies; in a large number central vision fails between 40 and 45 years,
quite frequently it lasts until 50, but rarely beyond 60, and that only in
exceptionally chronic cases in which obvious symptoms commenced late in
life. These few rare senile cases in which the onset of symptoms may be
delayed until 50 years or later, probably do not represent a late onset but
rather exceedingly slow progress, for the development of the disease in an
adult whose eyes have been shown to have been normal has never been
indisputably established.
There is no bulk of evidence that the disease tends to be apparent at an earlier
age in subsequent generations. There are, however, exceptions as in a pedigree
studied by Hine (1928) wherein night-blindness came on at 50, 40 and 57 years in one
generation and at 29 and 35 in the next.
Atypical Clinical Manifestations
1. CENTRAL PIGMENTARY DEGENERATION, in which the pigmentary
disturbance, either as spider-like clumps or scattered black dots, takes the
Fig. 2392. ATYPICAL CENTRAL Pig MENTARY DEGENERATION of RETINA (Hine,
T. O. S.). -

2774 TEXT-BOOK OF OPHTHALMOLOGY
form of an island round the macula, is not at all common (Knapp, 1870;
Leber, 1871; Hirschberg, 1889; Nettleship, 1907–08; Hine, 1928)
(Fig. 1392). The epithelial disturbance is frequently associated with a
subjacent choroidal sclerosis, which may be sharply defined or merge
gradually into apparently normal tissue to be again succeeded by an
equatorial belt of typically pigmented retina. In these cases central vision
is much damaged and the patients may prefer a rather dull light to a bright
illumination.
Transition stages occur between the true central lesion and the classical equatorial
lesion wherein a pigmented zone occurs immediately around the macula leaving good
central vision (Kapuscinski, 1908; Wittmer, 1910).
2. DEGENERATIO SINE PIGMENTO. The clinical picture and symptoma-
tology of pigmentary degeneration may appear without any, or at any rate
with a minimal degree, of ophthalmoscopically visible pigment migration; but
it is probable that this is the initial stage of the ordinary disease in which
pigmentary appearances are sometimes delayed for many years after the
development of marked night-blindness (Nettleship, 1914); indeed, if seen
early enough, all cases would probably come under this category. Apart
from the symptoms, the condition is therefore diagnosed only by the appear-
ance of the disc and the retinal vessels and the somewhat equivocal appear-
ance of the pigmentary epithelium.
3. ACUTE PIGMENTARY DEGENERATION. A rapidly acute form of
pigmentary degeneration has been described by Stock (1936); his cases
occurred in 3 children of the same parents starting in the 6th year of life
and terminating in total blindness and dementia, and also in a man of 25
in whom the right eye became blind within 6 months and the left in one,
whereafter the patient developed epilepsy and dementia and died. The
disease may thus apparently affect a hitherto healthy adult. The clinical
picture differed from the ordinary type of the disease in that the entire retina
was involved simultaneously, while pathologically the nervous elements of the
retina were completely degenerated with an absence of glial proliferation.
The development of epilepsy and dementia and the fatal termination were
apparently due to a similar cerebral degenerative process.
Pathology. The essential feature of the pathology of primary pigmen-
tary degeneration of the retina is a progressive degeneration of the neuro-
epithelium, primarily of the rods, to be followed by a general atrophy of the
whole tissue associated with a glial overgrowth, a depigmentation of the retinal
epithelium with a migration of pigment into the retina, and an obliterative
sclerosis of the retinal vessels.
Few opportunities have been offered for histological examination so that several
points in the pathological anatomy are still disputed. The first case to be examined
anatomically was that of Maes (1861), and subsequent cases are those of Leber (1869),
DISEASES OF THE RETINA 2775
Landolt (1872), Poncet (1875), Bader (1876), Hirschberg (1879), Wagenmann (1891),
Deutschmann (1891), Bürstenbinder (1895), Gonin (1902–03), Stein (1903), Greeff
(1903), Lister (1903), Stock (1908–36), Ginsberg (1908), Greeves (1912), Suganuma
(1912), Friedenwald (1930), Verhoeff (1931), Koyanagi (1931), Ascher (1932), and
Asayama and Takagi (1937). Unfortunately most of those cases had been com-
plicated by other diseases or had arrived at a very advanced stage.
All authorities are agreed that the essential and first retinal change is
a degeneration and eventual disappearance of the rods and cones, primarily
the former ; then follows subsequently a degeneration of the external
nuclear layer and the inner layers. In advanced cases when the rods have
entirely disappeared, some cones may still remain especially in the region
between the disc and the macula; and occasionally the degeneration may
Fig. 2393. – PRIMARY PIGMENTARY DEGENERATION of RETINA.
Müller's fibres extend through retina and replace neuro-epithelium with a layer
of neuroglial fibres running parallel with retina. On the right the limiting membrane
is destroyed. The chorio-capillaris is normal (Verhoeff).
be patchy, areas of complete atrophy lying side by side with areas wherein
the structures are well preserved. The degenerated tissue, particularly the
neuro-epithelium, becomes replaced more or less completely by neuroglia
which appears to be derived essentially from Müller's fibres, the gliosis being
progressive, sometimes forming a thick fibrillated layer between the pigment
epithelium and the retina proper (Fig. 2393). Until a late period the
ganglion cells and the nerve-fibre layer may remain relatively unchanged,
even after the eye has been blind for many years. The retinal vessels,
arteries and veins alike, always show marked changes, sometimes atrophic
and at other times hyperplastic ; they often show great thickening of their
walls which may be due to an increase in the adventitia or of the intima
resulting in a progressive diminution of their calibre, while especially at the
periphery they may be converted into solid strands of hyaline connective
tissue. Sometimes the optic nerve is completely atrophic, and at other

2776 TEXT-BOOK OF OPHTHALMOLOGY
times, when the ganglion cells are relatively intact, little or no atrophy is
apparent, although there is usually some increase of neuroglia at the dise
which is probably an extension of the retinal gliosis and gives rise to the
waxy appearance in those cases wherein actual atrophy is limited in extent.
Fig. 2394, PRIMARY Pigment ARY DEGENERATIox of RETINA.
An advanced case. Two retinal vessels are seen occluded and covered with
pigment. Centrally a patch of pigmentary epithelium opposite which the choroidal
capillaries remain, and here the external limiting membrane is attached (x 200)
(Greeves).
Changes in the pigmentary epithelium have always been marked
particularly in the areas opposite the regions where degeneration of the
neuro-epithelium has occurred; these changes are both degenerative and
Fig. 2395. Primary Pigmentary Degeneration of RETINA.
A pigmented plaque (P) in the retina, close to a pigmented vessel (V) cut longi-
tudinally (Parsons).
proliferative. Over large areas the epithelium may be completely absent,
and here the rods and comes and the external limiting membrane have
disappeared and have been replaced by neuroglia; over other areas it may
be present but unpigmented, and elsewhere it may be deeply pigmented and


T)ISEASES OF THE RETINA 2777
several layers deep (Fig. 2394); in these areas the external limiting membrane
has been preserved. A striking feature is the migration of pigment in
Quantity into the retina wherever the limiting membrane has disappeared,
sometimes as free granules but more frequently intra-cellularly forming
large masses and clumps arranged particularly around the retinal veins
(Fig. 2395). Asayama and Takagi (1937) noted a widespread lipoid
degeneration of the pigmentary epithelium ; while the formation of colloid
bodies which may even become calcified, or of drusen at the disc is common.
Considerable variations have been noted in the state of the choroid.
Occasionally this structure shows extreme vascular sclerosis with a more
or less complete disappearance of the chorio-capillaris (Gonin, 1902–03;
Greeff, 1903; Koyanagi, 1931; and others); at other times a patchy
Sclerosis of the choroidal vessels corresponds accurately with a patchy
affection of the retina (Greeves, 1912) (Fig. 2394); but in the majority of cases
especially the more lately reported ones, the choroid, including the chorio-
capillaris, has been normal even in extremely advanced cases of the disease
(Fig. 2393) (Lister, 1903 ; Stock, 1908; Ginsberg, 1908; Suganuma,
1912; Friedenwald, 1930; Verhoeff, 1931 ; and others).
If the disease described by Bourne, Campbell and Tansley (1938) in rats is accepted
as a true pigmentary degeneration—as it almost certainly is—their findings provide
two interesting pathological points. The changes in advanced cases were identical
with those found in man, but the opportunity to obtain eyes in abundance at all
stages of development demonstrated conclusively that the rods degenerated first,
some considerable time prior to the cones, and that the first signs of degeneration are
in the nuclei and not in the outer limbs of these structures. They also demonstrated
that the pigment tended to migrate into the substance of the retina along neuroglial
tracks ; and they showed conclusively that vascular sclerosis both of the retina and
especially of the choroid was not present initially, but was of late appearance.
AEtiology. The aetiology of pigmentary degeneration has excited a
great deal of controversy. The earlier investigators considered it to be a
primary degeneration of the neuro-epithelium (Landolt, 1872 ; Bader, 1876),
but the finding of vascular sclerosis in the choroid in several pathological
examinations suggested to later observers that the retinal degeneration was
secondary to this condition. The ample demonstration, however, that the
choroid may remain normal even in the terminal stages of the disease caused
a swing back of opinion to the original view, and this is to-day the most
generally accepted conception. Other suggestions, however, have been put
forward : that the primary fault lies in the pigmentary epithelium, or that
the disease is part of an endocrine dyscrasia, or is the result of avitaminosis,
Or is toxic in origin, and so on. It may be said at once that we are ignorant
of the aetiology, but since our views thereon must determine a rational
treatment, we shall briefly consider and criticize the arguments put forward
by the sponsors of the various theories.
2778 TEXT-BOOK OF OPHTHALMOLOGY
I. Vascular Theories
(a) That the primary fault is a sclerosis of the choroid and a disappearance of the
chorio-capillaris was argued by Gonin (1902–03), Parsons (1905), and Nettleship
(1907–08), and is to-day maintained by many authorities (Hepburn, 1908–38; and
others). The theory is based on two arguments—the dependence of the vitality of
the neuro-epithelium on the choroidal circulation as demonstrated by the experiments
of Wagenmann (1890), and the occurrence of such an obliterative sclerosis in many
cases examined pathologically. It will be remembered that in his experiments
Wagenmann sectioned the posterior ciliary arteries (and nerves) in rabbits and produced
a destruction of the neuro-epithelium owing to the cutting off of its source of nourish-
ment. Subsequent repetition of this work, however, particularly by Nicholls (1938),
has shown that there is little or no similarity between the type of change following
destruction of the choroidal circulation and that of pigmentary degeneration. In the
first case the rods and cones disappear simultaneously, the process of degeneration
starting in the outer limbs, there is little gliosis or fibrosis, and the pigment migration
is limited to the deeper layers of the retina and has no relation to the vessels. In pig-
mentary degeneration, on the other hand, the rods disappear before the cones, the
process of degeneration starting (if the disease in rats of Bourne, Campbell and Tansley,
1938, be accepted as analogous) in the cell nuclei, there is massive gliosis and fibrosis,
and the pigment migrates to the inner layers of the retina and accumulates pre-
ferentially around the vessels. Although there is a superficial resemblance, therefore,
the differences are so fundamental as to point to a different mechanism in the two cases.
The evidence of the occurrence of choroidal sclerosis in certain cases or of a topo-
graphical relationship between areas of sclerosis and retinal atrophy is rendered
nugatory by the demonstration of its absence in others, including some which have
reached an extreme stage of development and have been blind for many years (20,

Verhoeff, 1931): unless it is agreed t the disease exist, one mesodermal
8LIl
idal sclerosis and the other ectodermal and primarily retinal
(Stock, 1908), it seems necessary to conclude that such sclerosis is incidental and not
causative, and indeed, its occurrence is not unexpected in eyes which have reached
an advanced degree of degeneration and in subjects who are generally old. It is
true that a pigmentary degeneration does occur as a consequence of choroidal sclerosis ;
but this should be considered secondary and not primary in nature. The final argu-
ment sometimes put forward that the zonular scotoma is due to early failure of the
least resistant part of the vascular system of the choroid at the meeting place of the
posterior and anterior ciliary circulations, and that the late persistence of central
vision depends on the greater thickness of the chorio-capillaris in this region is not
sufficiently convincing to outweigh the very conclusive evidence against the theory.
(b) That the essential cause is a sclerosis of the retinal vessels was suggested by
Aebli (1938) who pointed out that their attenuation was the most characteristic and
constantly occurring feature of the condition, more common indeed than the pig-
mentation. In this event the gradual occlusion of the peripheral vessels would be
followed by degeneration and a secondary invasion of the retina by pigment ; while a
parallel process in the ear and cerebral circulation could be held to explain the common
association of deafness and other central nervous symptoms. It must be remembered,
however, that in advanced and progressive obliterative conditions of vascular sclerosis,
there is no similarity in the pathological picture, nor do the end-results of complete
occlusion of the central artery or one of its branches have any significant points of
comparison.
2. The pigmentary epithelium has been associated primarily with the disease
since pigmentation is such a conspicuous feature of the clinical picture. There is no
1 p. 2102.
DISEASES OF THE RETINA 2779
doubt that pathologically the changes in the neuro-epithelium and the pigmentary
epithelium are directly related to each other. It has been shown experimentally by
Friedenwald and Chan (1932) that the entire retinal structure may become disorganized
by the presence in it of pigment or the migration through it of wandering cells laden
with pigment, for after the injection of pigment into the vitreous of animals they found
a destruction of the neuro-epithelium and finally of all the retinal layers and their
replacement by gliosis. Since, however, it is a commonplace to find no changes of
this nature in eyes in which the epithelium shows complete senile degeneration or even
partial calcification, it would seem more likely that the changes in the pigmented layer
and the neuro-epithelium are parallel results of a common aetiological factor rather
than related to each other as cause and effect.
3. That the newro-epithelium is primarily at fault seems therefore to be the con-
clusion to which one is forced. The reason for its degeneration, however, remains an
enigma, although several suggestions have been put forward.
(i) Abiotrophy. The most widely credited theory is that the degeneration is a
primary and inherent process of the nature of an abiotrophy (Collins, 1919–22), that is,
a premature senility and death of the cells of a specified tissue. The undoubted
hereditary tendency of the disease and its early onset are in favour of this assumption,
a conclusion strongly supported by the possibility of experimentally breeding animals
in which a rudimentary condition of the neuro-epithelium or its complete absence
(Keeler, 1927) or the typical picture of pigmentary degeneration (Bourne, Campbell
and Tansley, 1938) is handed down as a Mendelian recessive. Collins explained the
ring Scotoma on this theory by assuming that the equatorial parts of the fundus in
which the neuro-epithelium first attains its full development ontogenetically will be
the first to suffer a failure in its function, and he assumed that the collateral phenomena
of deafness and nervous disturbances were due to a similar abiotrophic process in the
ear and the central nervous system.
Against this theory with its bland assumption of a primary degeneration of a
completely unknown nature inherent in the tissues, little can be argued ; it may quite
probably be correct, but in so far as it is completely sterile in pointing the way to further
attempts at elucidation or treatment, it has never been universally and enthusiasti-
cally accepted. On the other hand, several suggestions have been put forward that
the degeneration is secondary ; but it may be said at once that none of them is wholly
satisfactory and all of them quite speculative.
(ii) The effect of light was considered by Leber (1916) to be effective in association
with a diminished resistance of the neuro-epithelium. He pointed out the ease with
which the rods became fatigued by light in congenital night-blindness and suggested
that if the condition were more marked the fatigue might become actual injury. The
Zonular scotoma he explained by the greater resistance of the macula, owing to the
better chorio-capillary circulation, and the survival of the periphery owing to the
absence of direct light. The hypothesis, however, seems unlikely.
(iii) Glandular Influences. In recent years there has been an increasing tendency
to associate pigmentary degeneration of the retina with endocrine dyscrasias,
particularly pituitary and diencephalic disturbances, an association typified in the
Laurence-Moon-Biedl syndrome. The aspect of pituitary hypo-function was especially
stressed by Pletneva (1930), Zondek and Koehler (1932), Zondek (1935) and Viallefont
(1936), while a wider polyglandular disturbance was suggested by Mecca (1936),
Farina (1936), Rinaldi (1937), Schupfer (1937), and others. Corroboratory evidence
of pituitary dysfunction was furnished by Dax (1938) who, in 8 mentally defective
patients found in the blood and the urine an abnormal substance derived from the
pituitary which will disperse the melanosomes of the frog, which apparently becomes
evident only when this gland is pathologically abnormal or is subjected to physiological
StreSS.
2780 TEXT-BOOK OF OPHTHALMOLOGY
The sea, hormones have also been associated with the disease, particularly by Lorenz
(1929) and Wibaut (1931–35), on the ground that the disease becomes accentuated
at puberty and is more frequent and of graver prognosis in males, the assumption being
that the female genital glands exercise a restraining influence over it. In the sex-
linked heredity carried through the female sex chromosome to the male offspring,
Wibaut hypothecated that in these males the sex hormone contributed by the mother
might be missing. A number of attempts have been made to introduce glandular
therapy based upon this hypothesis, which we shall discuss presently, with, however,
indeterminate results. Whatever influence any of the ductless glands have—if any—
upon this disease is, in the present state of knowledge, extremely nebulous. to say the
least of it.
(iv) Hepatic disease has frequently been associated with pigmentary degeneration
owing to the known association between diseases of the liver and a deficiency in the
regeneration of visual purple and night-blindness. We have already spoken of this
subject in dealing with choroidal diseases. It will be remembered that Dolganoff
(1897) ligated the bile ducts of dogs and produced degeneration of the pigment epi-
thelium and the retina, a result confirmed by Majima (1925) in the frog, although
Glüh (1928) obtained no change in the rate of degeneration of visual purple in the
rabbit. Similarly Sugita (1926) produced a rapid destruction of the outer retinal
layers and a lipoid infiltration of the pigment epithelium by injecting rats with bile
salts. A clinical association between cirrhotic and chronic inflammatory conditions
of the liver and degeneration of the pigmentary epithelium of the retina has been
reported by Baas (1894), Hori (1895), Purtscher (1900) and Koyanagi (1920); but
although it has been recorded that the subjects of pigmentary degeneration do suffer
from hepatic dysfunction (Takahashi, 1925), this association does not appear to be
by any means invariable.
(v) Vitamins. In a somewhat similar manner the well-known fact that vitamin
deficiency, particularly of vitamin A,” is frequently followed by night-blindness has
associated pigmentary degeneration with the deficiency diseases (Levine, 1933; and
others).
(vi) A toacic cause has been suggested by certain authorities (Leber, 1916; Verhoeff,
1931). It is certain that toxic influences can produce extremely similar conditions both
in their clinical appearances and in their symptoms, whether from the presence of toxins
in the blood, as in syphilitic neuro-retinitis, or in the vitreous, as in cases of leucoma
adherens, or a long-standing dislocation of the lens into the vitreous. To these clinical
occurrences a parallel is provided by experiments wherein pigmentary degeneration
has been produced by injecting toxic materials into the vitreous, for example, the
injection of papayotin by Capauner (1893), of atoxyl by Igersheimer and Itami (1909),
and of scharlach oil by Schreiber and Wengler (1910).
In the present state of our knowledge, therefore, we seem driven to the
conclusion that pigmentary degeneration of the retina is a degeneration
primarily of the retinal neuro-epithelium which almost certainly is affected
by an adherent hereditary weakness; whether extraneous influences are
effective in exploiting this weakness is unknown, but in the interests of
possible lines of therapy this possibility should by no means be ruled out and
should be further explored. Whether, again, two separate forms exist, as
suggested by Ascher (1932) and supported on genetic grounds by Wibaut
(1931), the One mesodermal or choroidal and the other ectodermal and
* Vol. I, p. 971 (983).
DISEASES OF THE RETINA 2781
retinal, the evidence available is insufficient whereon to base any legitimate
conclusion.
Treatment
From what has already been said it will be gathered that the treatment
of retinitis pigmentosa is most unsatisfactory, and, indeed, may be said not
to exist. As usually occurs, however, with every progressive disease of
unknown aetiology which is incurable, innumerable remedies have been
suggested, enthusiastically received, tried out, and then discredited, some-
times with no small psychological and economic loss to the sufferer. Most of
these depend upon the views held by their advocates on the aetiology of the
disease, and these will be briefly summarized.
Methods to induce vaso-dilatation are the most favoured. Their adop-
tion was suggested by the theory of the dependence of the condition upon
choroidal sclerosis and upon the demonstrable and invariable attenuation of
the retinal arteries ; but while their practice frequently leads to some
transient improvement, doubtless owing to the increased circulation and
metabolic activity excited in the eye, the ultimate results have been dis-
appointing. The vaso-dilatation has been induced by sub-conjunctival
$njections of saline and other substances (Zehnder, 1897; Verderame, 1906),
or by the administration of drugs such as daily inhalations of amyl nitrite
(Koyanagi, 1931; Biró, 1939) to be followed by nitro-glycerin (Rabinowitz,
1931), or by injections of acetyl choline (Corrado, 1933). The same effect
can be produced or reinforced by repeated paracenteses, the vaso-dilatatory
action of which is said to be reinforced by hypodermal or retro-bulbar injec-
tions of atropine (Vele, 1933; Folk, 1937), by miotics, or by permanently
decompressing operations as trephining (Mayou, 1916), or cyclodialysis
(Lauber, 1935–38; Sobanski, 1935–38). r
An even more heroic procedure is the attempt to produce a permanent
vaso-dilatation by section of the sympathetic supply to the eye, either by
section of the sympathetic in the neck (Abadie, 1901; Royle, 1930–34;
Meighen, 1931–35; Campbell, 1932; Caeiro, Malbran and Balza, 1933;
MacDonald and McKenzie, 1935 ; Walsh and Sloan, 1935 ; and others), or
stripping of the carotid artery (Magitot and Desvignes, 1934; Magitot,
1934; Löwenstein, 1935; Blobner, 1937) or both (Takáts and Gifford,
1935). Although some cases of improvement have been recorded many
more negative results have been reported, and in most cases the improve-
ment in the visual fields and acuity which have been noted have been
transitory. With the evidence available it is indisputable that the operation
which is not without hazard, is unjustified.
Those who advocate an endocrine origin of the disease have advocated
hormonal treatment, and successes have been claimed both for the administra-
tion of pituitary (Challous, 1921 ; Beck, 1929; Villefont, 1936), ovarian
(Wibaut, 1931–35; François, 1933; Guerrieri, 1935), or testicular extracts
2782 TEXT-BOOK OF OPHTHALMOLOGY
(Biró, 1939). Similarly, liver extracts have been tried (Carsten, 1928;
Biró, 1939), or mixed polylysates of several organs such as the liver,
pituitary, adrenal, retina, and so on (Manyukova, 1936). Vitamin A and
other vitamins in various forms have also been exhibited (Levine, 1933;
Casini, 1935), and physical methods of treatment such as galvanism (Dor,
1873; Gunn, 1881) or X-rays (Merkulow and Schick, 1928) have had their
advocates, as well as a host of other remedies. None of them, however, has
shown anything approaching lasting or consistent results, and it is probable
that the temporary improvement following many of them is due to an
increase in the metabolic activity in the eye rather than to any specific
action. In the assessment of all of them it is well to take into consideration
the natural fluctuations in the progress of the disease, as well as the
enthusiasm of the practitioner and the credulity or the desperate hopefulness
of the patient.
Abadie. Am... d’Oc., Czzv, 194, 1901.
Aebli. A. of O., xx, 680, 1938.
Agatston. Am. J. O., xxii, 420, 1939.
Allan. A. of O., xviii, 938, 1937.
Alt. A. f. Aug., vii, 376, 1878.
v. Ammon. Klim. Darstellung, i, 1838.
Arlt. Krankh. d. Auges, iii, 101, 1856.
Asayama and Takagi. K. M. Aug., xcviii,
162, 1937. -.
Ascher. B. O. G. Heidel.., xlix, 547, 1932.
A. f. Aug., cvi, 585, 1932.
Avery and Sorsby. Proc. R. S. Med., xxx,
388, 1936.
Ayres. Am. J. O., iii, 81, 1886.
Baas. A. f. O., xl (5), 212, 1894; xliii, 642,
1897.
|Bader. Guy’s Hosp. Rep., xxi, 229, 1876.
Bardet. Sur un Syndrome d’obesité congen.
avec polydactylie et Ret, pig., Paris, 1920.
Baumeister. A. f. O., xix, 261, 1873.
Beck. Endocrinology, xiii, 375, 1929.
Beigelman. A. of O., vi, 254, 1931.
Bell. Treasury of Human Inheritance, ii (1),
1922.
Biedl and Raab. Deut. med, W., xlviii, 1630,
1922.
Wien. A. f. inn. Med., vii, 443, 1924.
Biemond. Ned. Tij. v. Gen., lxxviii, 301,
1423, 1801, 1934.
Biró. Brit. J. O., xxiii, 332, 1939.
Blobner. K. M. Aug., xcviii, 289, 1937.
Bourne, Campbell and Tansley. T. O. S.,
lviii, 234, 1938. -
Bürstenbinder. A. f. O., xli (4), 175, 1895.
Caeiro, Malbran and Balza. Rev. As. Méd.
Argent., xlvii, 3403, 1933.
Campbell. Canad. Med. As. J., xxvi, 674,
I932. -
Capaurier. B. O. G. Heidel., xxiii, 45, 1893.
Carsten. Z. f. Aug., lxv, 189, 1928.
Casini. A. dº Ott., xlii, 409, 1935.
Challous. Am... d’Oc., clviii, 100, 1921.
Cockayne, Krestin and Sorsby. Quart. J.
Med., iv, 93, 1935.
Collins. T. O. S., xxxix, 165, 1919.
Intermat. Cong. Oph., Washington,
1922. -
Corrado. An... di Ott., lxi, 43, 1933.
Crzellitzer. A. f. Aug., x1, 279, 1900.
Dax. Brit. J. O., xxii, 345, 1938.
T. O. S., xlviii, 227, 1938.
Derby. Boston Med. Surg. J., lxxii, 149, 1865.
Derigs. Ueber Ret. Pig., Bonn, 1882.
Deutschmann. Beit. z. Aug., iii, 69, 1891.
Dolganoff. A. f. Aug., xxxiv, 196, 1897.
Dor. A. f. O., xix (2), 342, 1873.
A. d’O., iii, 481, 1883.
Farina. Rass. It. Ott., v, rö66, 1936.
Folk. Am. J. O., xx, 511, 1937.
François. Bull. S. fr. d’O., xlvi, 383, 1933.
Friedenwald. A. of O., iv, 767, 1930.
Friedenwald and Chan. A. of O., viii, 173,
I932.
Ginsberg. K. M. Aug., xlvi (2), 1, 1908.
Glüh. Z. f. Aug., lxiv, 69, 1928.
Gonin. Am... d’Oc., czzv, 101, 1901 ; cz.xviii,
90, 1902; cz Xix, 24, 1903.
v. Graefe. A. f. O., ii (2), 263, 282, 1856;
iv (2), 250, 1858.
Greeff. Orth’s Pathologie, 1903.
Greeves. R. L. O. H. Rep., xviii, 301, 1912.
Guerrieri. Atti S. O. Ital., 587, 1935.
Gunn. R. L. O. H. Rep., x, 161, 1881.
Günsburg. A. f. Aug., xxi, 184, 1890.
Bancock. R. L. O. H. Rep., xvi, 496, 1906.
|Hansell. T. O. S., xliv, 303, 1924.
Henderson. A. of O., xi, 763, 1934.
|Hepburn. R. L. O. H. Rep., xvii, 232, 1908.
T. O. S., xlix, 129, 1929 ; Iviii, 246, 1938.
|Hine. P. R. S. Med., xvii, 14, 1924.
T. O. S., xlviii, 160, 1928.
Hirschberg. A. f. Aug., viii, 202, 1879.
Cb. pr. Aug., 37, 1889.
Bori. A. f. Aug., xxxi, 393, 1895.
Höring. K. M. Aug., ii, 233, 1864; iii, 236,
1865.
103,
DISEASES OF
THE RETINA 2783
Hutchinson. R. L. O. H. Rep., v, 324, 1866;
vi, 39, 222, 272, 1867; vii, 43, 434, 1873;
viii, 34, 1874.
O. Rev., i, 28, 1882.
A. of Surg., i, 92, 1890;
xi, 118, 1900.
Igersheimer and Itami.
Pharm., lxi, 18, 1909.
Kapuscinski. Diss., Freiburg, 1908.
B. O. G. Münch., 1, 13, 28, 1934.
ix, 182, 1898;
A. f. eacp. Path.
Farsch. Z. f. Aug., xc, 157, 1936.
Reeler. J. Earp. Zoo., xlvi, 355, 1927.
Klainguti. Schw. med. W., liii, 910, 1923.
Knapp. T. Am. O. S., 120, 1870.
Royanagi. K. M. Aug., lxiv, 836, 1920.
A. f. O., czzvii, 1, 1931.
Landolt. A. f. O., xviii (1), 325, 1872.
Lauber. Z. f. Aug., lxxxvii, 65, 1935.
B. O. G. Heidel., li, 245, 1936.
Rl. Oz., xv, 230, 1937.
K. M. Aug., c, 108, 1938.
T. O. S., lviii, 661, 1938.
Laurence and Moon. O. Rev., ii, 32, 1866.
Leber. A. f. O., xv (3), 1, 1869; xvii (1),
314, 1871.
G.-S. Hb., II, vii (A2), 1076, 1916.
Levine. A. of O., ix, 453, 1933.
Liebreich. Deut. Kl., xiii, 53, 1861.
Lister. R. L. O. H. Rep., xv, 254, 1903.
Lorenz. Russ. O. J., ix, 548, 1929.
Löwenstein. A. f. O., exxxiii, 636, 1935.
MacDonald and McKenzie. A. of O., xiii, 362,
1935.
Maes. Jb. d. Utrechter Augenkl., 1861.
Magitot. Bull. Acad. de Sc., czi, 816, 1934.
Magitot and Desvignes. Am. d’Oc., clzxi, 897,
1934.
Majima. A. f. O., exv, 286, 1925.
Manyukova. Sov. vest. O., viii, 348, 1936.
Mayou. T. O. S., xxxvi, 350, 1916.
Mecca. An... dº Ott., lxiv, 172, 1936.
Meighen. T. O. S., li, 124, 1931; Iv, 93, 1935.
Merkulow and Schick. K. M. Aug., lxxxi,
844, 1928.
Nettleship. T. O. S., vii, 301, 1887; xxix,
p. cxxviii, 1909.
R. L. O. H. Rep., xv (3), 189, 1903; xvii,
1, 151, 333, 1907–08 ; xix, 123, 1914.
Nicholls. Brit. J. O., xxii, 672, 1938.
Nuel. A. d' O., xxviii, 737, 1908.
Parsons. Pathology of the Eye, London, ii,
602, 1905.
Pedraglia. K. M. Aug., iii, 114, 1865.
Perera. A. of O., xx, 471, 1938.
Pillat. Am. J. O., xiii, l, 1930.
Pletneva. Russ. A. Oft., vi, 471, 1930.
Poncet. Am. d’Oc., lxxiv, 234, 1875.
Purtscher. A. f. O., 1, 83, 1900.
Rabinowitz. Russ. O. J., xiv, 225, 1931.
Riechert. K. M. Aug., xci, 163, 1933.
Rinaldi. Rif. Med., liii, 371, 1937.
Rossi. Boll. d’Oc., v, 363, 1926.
T.O.-VOL. III.
Royle. Med. J. Australia, ii, 364, 1930; ii,
111, 1932.
T. Austral. Med. Cong., 205, 1934.
Savin. Brit. J. O., xix, 597, 1935.
Scheurlen. K. M. Aug., xciv, 761, 1935.
Schön. Hab. path. Anat., Hamburg, 202,
1828.
Schreiber and Wengler. A. f. O., lxxiv, 1,
1910.
Schupfer. Boll. d’Oc., xvi, 557, 1937.
de Schweinitz. T. O. S., xliii, 90, 1923.
Shoemaker. Retinitis Pigmentosa, Phila.,
1909.
Shoji. A. d’O., xliii, 402, 1926.
Smith. T. O. S., xxiv, 135, 1904.
Snell. T. O. S., xxii, 300, 1902; xxvii, 217,
1907.
Sobanski. Kl. Ocz., xiii, 239, 1935; xiv.
358, 1936,5 xvi, 44, 1938.
A. f. O., exxxv, 372, 383, 401, 1936.
Sorsby, Avery and Cockayne. Quart. J. Med.,
viii, 51, 1939.
Stein. A. f. O., lvi, 463, 1903.
Stock. K. M. Aug., xlvi, 225, 1908; xcvii,
577, 1936.
B. O. G. Heidel., li, 106, 1936.
Stör. K. M. Aug., iii, 23, 1865.
Strieff and Zeltner. A. d’O., ii, 289, 1938.
Suganuma. K. M. Aug., i (1), 178, 1912.
Sugita. A. f. O., czvi, 653, 1926.
Takahashi. A. f. O., czvi, 143, 1925.
Takáts and Gifford. A. of O., xiv, 441, 1935.
van Trigt. Ned. Lancet, ii, 492, 1853.
Usher. T. O. S., xxix, p. xciv, 1909; lv., 176,
1935.
R. L. O. H. Rep., xix, 130, 1913.
Vele. A. di Ott., xl, 203, 1933.
Velhagen. K. M. Aug., lxxxviii, 1, 1932.
Verderame. Z. f. Awg., xv, 415, 1906.
Verhoeff. A. of O., v, 392, 1931.
Viallefont. An. d’Oc., clxxiii, 33, 1936.
Villefont. An. d’Oc., clxxiii, 33, 1936.
Waardenburg. K. M. Aug., lxxxiv, 493,
I930.
Wagenmann. A. f. O., xxxvi (4), 1, 1890 ;
xxxvii (1), 230, 1891.
Walsh and Sloan. A. of O., xiv, 699, 1935.
Webster. T. Am. O. S., ii, 495, 1878.
de Wecker. Traité, II, ii, 337, 1868.
Wibaut. Ned. Tij. Gen., lxxii, 866, 1928;
lxxv, 4226, 1931 ; xxxiii, 139, 1935.
Deut. med. W., lvii, 1739, 1931.
R. M. Aug., lxxxvii, 298, 1931; lxxxviii,
299, 1931.
Wilmer. A. Newrol. and Psy., xii, 137, 1924.
Wittmer. A. f. Aug., lxviii, 81, 1911.
Yokoyama. Acta S. O. Jap., xxxvii, 1122,
1933.
Zehnder. Diss., Basel, 1897.
Zondek. Diseases of the Endocrine Glands,
London, 1935.
Zondek and Koehler.
2025, 1932.
Dewt. med. W., lviii,
2784 TEXT-BOOK OF OPHTHALMOLOGY
2. DEGENERATIO PUNCTATA ALBESCENS
While being closely related to pigmentary degeneration of the retina,
this condition has some fundamental differences. It is a familial disease
of a degenerative type commencing usually in early life, characterized by
the presence of innumerable discrete white dots scattered over the whole fundus
without pigmentary changes, and is usually non-progressive and stationary.
Since it was first described by Mooren (1882) under the title retinitis punctata
albescens, some 50 cases have appeared in the literature, the most interesting being those
described by Gayet (1883), Ulrich (1883), Landesberg (1883), Fuchs (1896), Griffith
(1897), Byers (1900), Spengler (1901), Wuestefeld (1901), Galezowski (1904), Pascheff
(1905), Takayasu (1906), Gradle (1907), v. Duyse (1907), Nettleship (1908–14), Lauber
(1910), Diem (1914), Cohen (1916), Agatston (1919), Healy (1921), Lievens (1921),
Scotti (1928), Appleman (1930), Satanowsky (1930), Marshall (1931), Milner (1932),
Atkinson (1932), Henderson (1934), Giannini (1934) and Garden (1938). The term
fundus albi-punctatus cum hemeralopia congenita was suggested by Lauber (1910).
The disease is usually congenital or starts in very early life, although
an anomalous case apparently started at 17 years of age after an acute
attack of Vincent’s angina (Henderson, 1934). It is familial, but although
it is common for several members of a family to be affected and con-
sanguineous marriages are frequent, it has never been seen in more than one
generation. It is interesting, however, that pigmentary degeneration or a
history of night-blindness in the same family is common.
The clinical picture is dominated by the powdering of the entire fundus
with discrete white dots, like dull white or off-white paint, and often so
small as to be unobserved by ophthalmoscopy by the indirect method
(Fig. 2383, Plate LVI). They are uniform in size and round, although
an occasional dumb-bell shape indicates the confluence of more than
one ; they are entirely free from pigment, and lie underneath the retinal
vessels, scattered all over the fundus centrally as well as peripherally,
leaving, however, the foveal region itself clear. The vessels are small, but
not markedly so ; the disc pale, but not grossly atrophic ; the entire
fundus depigmented and tessellated ; and the appearances are not pro-
gressive.
The symptomatology comprises night-blindness which is usually con-
genital, and sometimes depends more on a retardation of adaptation rather
than on a lowering of the light threshold, and a field defect noted in more
than half the cases usually taking the form of a peripheral contraction
frequently most marked in dim light, with the retention in many cases of
good central vision. The most striking feature of the condition which
distinguishes it from pigmentary degeneration is that it is stationary or at
the worst only infinitely slowly progressive. Six cases, indeed, have been
reported wherein the condition diminished or the dots disappeared (Gradle,
1907; Nettleship, 1914, 2 cases; Atkinson, 1932, 2 cases ; and Henderson,
DISEASES OF THE RETINA 2785
1934). Occasionally, however, pigmented deposits are found in association
with the white dots and these may be progressive, the albescens condition
becoming converted into a pigmentary degeneration (Fig. 2396). Occasionally
also, a central choroidal lesion may occur, such as a black pigmentary
disturbance (Derkac, 1924) or an area of choroidal sclerosis (Pillat, 1930).
Fig. 2396,-ATYPICAL ALBEscENT PUNCTATE DEGENERATION witH EARLY
PIGMENTARY DEGENERATION of RETINA.
Associated with the Laurence-Moon-Biedl syndrome (Savin, Brit. J. O.).
Since no pathological examination has been done the nature of the white
spots is problematical. The usual view is that they represent colloid bodies
perhaps in a partially calcified condition (Leber, 1916), and it is possible that
the difference in their appearance from that of the usual drusen is due to
their probable formation in intra-uterine life. It is frequently said also
that the condition is closely allied to pigmentary degeneration, occurring

Y Y 2
2786 TEXT-BOOK OF OPHTHALMOLOGY
perhaps in eyes poorly equipped with pigment so that the usual reaction
cannot become manifest. The latter hypothesis cannot be applicable since
the disease occurs in negroes (Cohen, 1916); while its stationary character
seems to rule out any relationship. Giannini (1934), having observed the
presence of Mizuo's phenomenon, considered the condition allied to Oguchi’s
disease.”
Agatston. A. of O., xlviii, 294, 1919. Landesberg. Cb. pr. Aug., vii, 261, 1883.
Appleman. A. of O., iii, 755, 1930. Lauber. K. M. Aug., xlviii (1), 133, 1910.
Atkinson. A. of O., viii, 409, 1932. Leber. G.-S. Hb., II, vii (A2), 1177, 1916.
Bell. Treasury of Human Inheritance, ii (1), Lievens. An. d’Oc., clviii, 829, 1921.
1922. Marshall. P. R. S. Med., xxv, 687, 1931.
Byers. Am. J. O., xvii, 11, 1900. Milner. Brit. J. O., xvi, 418, 1932.
Cohen. A. of O., xlv., 373, 1916. Mooren. Fünf Lustren oph. Wirksamkeit,
Derkac. Am... d’Oc., clxi, 33, 1924. Wiesbaden, viii, 216, 1882.
Diem. K. M. Aug., liii, 371, 1914. Nettleship. T. O. S., vii, 301, 1887.
v. Duyse. A. d’O., xxvii, 497, 1907. R. L. O. H. Rep., xvii, 377, 1908; xix, 123,
Fuchs. A. f. Aug., xxxii, 111, 1896. 1914.
Galezowski. Rec. d’O., xxvi, 714, 1904. Pascheff. O. Rev., xxiv, 65, 1905.
Garden. T. O. S., lviii, 453, 1938. Pillat. Am. J. O., xiii, l, 1930.
Gayet. A. d’O., iii, 385, 1883. Satanowsky. A. de Oft. B. A., v, 28, 1930.
Giannini. A. di Ott., lxii, 752, 1934. Scotti. Am... dº Ott., lvii, 45, 1928.
Gradle. J. Am. Med. As., xlix, 301, 1907. Spengler. Z. f. Awg., vi, 285, 1901.
Griffith. T. O. S., xvii, 51, 1897. Takayasu. A. f. O., lxiii, 281, 1906.
Healy. Brit. J. O., v., 18, 1921. Ulrich. K. M. Aug., xxi, 140, 1883.
Henderson. A. of O., xi, 763, 1934. Wuestefeld. Z. f. Awg., v. 110, 1901.
3. FAMILIAL COLLOID DEGENERATION
(HoNEYCOMB CHOROIDITIs OF DOYNE)
This disease, which appears to be a primary familial degeneration of the
tapeto-retinal type, is characterized by the appearance in massive quantity of
colloid deposits in a ring-shaped formation in the peri-macular area and
eventually by a complete degeneration of the central region. The dissociation
I c—HQ
[
II Óſ s * to &
º & Cº. ºf
H– H-
r & & Q • **
v 8 ; ; ; 5 TTº
Ó
| •+ Q
VI # -, -i-. Ó & 5
O Affected examined cases.
Q
Cſ

& Known to have bad sight.
FIG. 2397.-HEREDITARY COLLOID DEGENERATION OF DOYNE.
(1) Case of Fig. 2398. (2) Case of Fig. 2399 (Tree).
1 Vol. II, p. 1340.
DISEASES OF THE RETINA 2787
of a familial type of “guttate choroiditis" was due to the observations of
Doyne (1899–1910) on certain families in the neighbourhood of Oxford,
where the disease is still indigenous (Fig. 2397). Other cases have been
reported by Butler (1910), Mould (1910), Tree (1937). All these were found
in central England; and although a similar case has been reported by
Malbran and Androqué (1936) in South America, it was not familial.
The clinical appearance of an early case shows the presence of yellow
patches between the disc and the macula which tend to spread around the
Fig. 2398.-HEREDITARY Colloid Degeneration of Doyne.
Case (1) of Fig. 2397. Early changes (Tree, Brit. J. O.).
central area enclosing it as in a ring (Fig. 2398). Occasionally spots appear
on the nasal side of the disc, but the great mass occupies a horizontally
disposed oval area enclosed above and below by the upper and lower temporal
arteries. As a rule the spots are of uniform size and rounded, gradually
becoming whiter in colour, and are surrounded by shadow-rings suggesting
a honeycomb or mosaic appearance. As the disease slowly progresses the
individual spots which up to now might have been described as a “giant
Tay’s choroiditis” tend to merge together and to become more raised,
while degenerative changes and haemorrhages begin to appear in the central
area which is eventually occupied by an extensive atrophic zone wherein

2788 TEXT-BOOK OF OPHTHALMOLOGY
the choroidal vessels are visible (Fig. 2399). At this stage the vision,
which has hitherto remained good, becomes progressively and seriously
affected. The condition, which usually, but not invariably, affects females,
is always bilateral although one eye is usually more advanced than the
other. The pathological study of an eye by Collins (1913) showed large
plaque-like hyaline deposits similar in every way to typical drusen, without
Fig. 2399-HEREDITARy Colloid DEGENERATIox of Doyne.
Case (2) of Fig. 2397. Advanced changes with macular degeneration (Tree,
Brit. J. O.).
any changes in the retina or choroid which could not be attributed to pressure.
Presumably the disease is due to some hereditary degeneration of the
pigmentary epithelium.
Butler. T. O. S., xxx. 94, 1910 Malbran and Androqué. A. de Oft. B. A., xi,
Collins. Ophthalmoscope, ix. 537, 1913. 529, 1936.
Doyne. T. O. S. xix, 71, 1899; ºxx. 93, Mould. T. O. S., xxx, 189, 1910.
190, 1910. Tree. Brit. J. O. xxi. 65, 1937.
4. HEREDo-MACULAR DEGENERATION
Under this heading may be grouped a number of conditions of a heredi-
tary nature and with a familial incidence characterized by bilateral degenerative
changes at the macular area without degenerative changes in the central
nervous system. Such changes may appear at many ages, but they have

DISEASES OF THE RETINA 2789
a definite predilection for those periods of life in which physiological
stresses are marked : in the transition stage between intra-uterine and
extra-uterine life (the infantile type), at the second dentition (6–8 years : the
juvenile type), at puberty (12–15 years : the adolescent type), at the end of the
growing period (20 years : the adult type), at the beginning of senile regression
(45–50 years : the pre-senile type), and in senility (in advanced age : the
senile type). As a rule the familial character of the condition is maintained
in that all the affected members of a family are approximately alike with
regard to the time of the development of degenerative symptoms, the nature
of the lesion, and its rate of progress and general evolution.
The first recognition of the occurrence of familial degenerations at the macula was
due to Jonathan Hutchinson (1875), who in describing symmetrical chorio-retinal
diseases in senile persons (Tay’s central guttate choroiditis)" noted a familial pre-senile
type which occurred in three sisters aged 40, 48 and 57 years. The first group, however,
to be clearly described was the adolescent type which was noted to occur without
cerebral symptoms in two brothers at puberty by Rayner Batten (1897). Twenty-
eight years later they were both in good health apart from the presence of a central
scotoma (R. Batten, 1925). Some time later Best (1905) described a similar macular
degeneration which was congenital in incidence in 8 members of a family : BEST’s
DISEASE. Four years later Stargardt (1909–13) again drew attention to the familial
occurrence of the similar lesion at puberty unassociated with mental symptoms which
had first been noted by Rayner Batten (1897)—this type is usually known as
STARGARDT’s DISEASE. At a later date Behr (1920) noted the appearance of the adult
type about 20 years of age : BEHR’s DISEASE ; and Vossius (1921) and Weisel (1922)
drew attention to its appearance after the second dentition. Finally Behr (1920)
suggested that many cases of senile macular degeneration (of Haab) * have a similar
basis, and were primary degenerations of the tapeto-retinal type rather than secondary
degenerations depending on choroidal sclerosis.
Other conditions are sometimes considered closely related to this group in so far
as they constitute familial dystrophies of the tapeto-retinal type affecting the central
area. One of these is the familial colloid degeneration known as Doyne’s honeycomb
choroiditis.” There is some justification also for including familial day blindness in this
group (Sorsby, 1934); it has been associated with macular lesions (Nettleship, 1908;
Behr, 1920), and in the only histological examination on record the macular cones
were found to be affected (Larsen, 1921). There are other cases also as congenital
macular “ colobomata ?’ of familial occurrence sometimes associated with skeletal
anomalies (Sorsby, 1935) which may have a very similar, or even an identical basis ;
as well as those rare, ill-defined macular lesions which occur, sometimes in advanced
age, and may be associated with vague lesions in the central nervous system (Batten,
1922). The familial types of peri-papillary and central choroidal sclerosis, classed by
Leber (1916) as tapeto-retinal degenerations, are also probably nearly related.
So far as the macular lesion is concerned, a very similar clinical picture
is presented by a group of cases which presents associated symptoms of
central nervous involvement commencing at the age of the second dentition
or at puberty. Such a combination was first observed by F. E. Batten and
later by Mayou (1904) and Stephenson (1904). The condition was called
1 p. 2749. ? p. 2372. 8 p. 2786.
2790 TEXT-BOOK OF OPHTHALMOLOGY
familial maculo-cerebral degeneration by Oatman (1911), and, as we shall see
presently," represents a juvenile form of amaurotic family idiocy. In general
terms it may be said that the earlier the lesion appears, the more severe it is
and the more likely it is to be associated with mental symptoms; these,
with their usual termination in dementia, paralysis and death, do not occur
when the lesion becomes manifest after puberty (Beach, 1936).
* * * *
#Tº #
4 & & ; ; ; ; ; ;
* : * : ; ; ; ; , ;"

*
Čy &
FIG. 2400.-JUVENILE MACULAR DEGENERATION (Behr).
The influence of heredity in the determination of these macular and
cerebro-macular degenerations is seen in their strongly familial incidence
(Fig. 2400): a typical case, for example, is that of Lisch (1937) wherein
4 boys (aged 15, 16, 19, 20) out of a family of 6 were affected. Such an
o-d
+

& & & J & &
& ſº º Jº Jº
O Heredo-macular degeneration.
© Other affections (cochlear degeneration : amaurotic idiocy).
FIG. 2401.—HEREDITARY CEREBRO-MACULAR DEGENERATIONs (Alkio).
incidence is not, however, by any means invariable for isolated cases may
well appear without family history or consanguinity (Knapp, 1929; Schiff-
Wertheimer and Tillé, 1930; and others). Thomson (1932) reported a
family of 4 boys and 4 girls wherein all the males and only the males
were affected. As a rule the inheritance is therefore recessive; but occasional
instances have been recorded wherein an irregular dominance has been
1 p. 2801
DISEASES OF THE RETINA 27.91
apparent, as the family recorded by Gasteiger (1936) wherein 11 cases
occurred in 3 generations, the earliest at the age of 17, and in which all the
members of one generation were affected ; or that studied by Jung (1936)
which included 22 cases. In the same family other defects such as epilepsy,
mental retardation, primary pigmentary degeneration of the retina, cochlear
degeneration, or amaurotic idiocy may be noted (Fig. 2401).
The clinical appearance of the macula varies but only within fairly
narrow limits, and comprises essentially some pigmentary disturbance and
the appearance of whitish-grey foci resembling colloid bodies confined to the
central areas. At first the pigmentation is usually a fine punctate stippling
which can well be described as a pepper-and-salt macula. These appearances
may remain almost indefinitely, producing visual symptoms which appear
to be much out of proportion to the objective signs ; but more usually they
progress, large blobs of pigment appearing as well as greyish-white flecks
and foci which multiply until the central region is occupied by a dirty grey
area peppered over with pigment (Fig. 2384, Plate LVI). Exceptionally a
hole appears at the fovea itself, and there is usually some atrophy of the
optic nerve particularly on its temporal side.
Occasionally anomalous appearances are found—-rucks and folds, arranged perhaps
as a cart-wheel, a cone-like formation as if of glial hyperplasia (Rieger, 1929), or a
diffusely dull Smoky area comparable in appearance to beaten brass (Wright, 1935).
The symptoms may be slight or incapacitating. An early sign is a
central scotoma for colour, the appreciation of blue usually disappearing
before that of red, and fixation may become slightly eccentric. Occasionally
for a time there is a small ring scotoma with the preservation of central
vision, but eventually fixation becomes lost and a complete central scotoma
results. Although occasionally relatively good vision may be retained in
the presence of an advanced lesion, as a rule the visual loss appears to run
in advance of the clinically observable changes, and a lesion which appears
to be small and unimportant and may be easily missed, may be responsible
for a complete abolition of the central vision.
At times the disease shows evidence of cerebral involvement in the form
of mental backwardness (Nathan, 1938; and others), while cases occur in
which minor defects of the central nervous system can only be elicited after
minute search. These are probably due to a similar selective degeneration
in other systems of neurons, such as may occur in the juvenile form of
amaurotic idiocy (maculo-cerebral degeneration)," but are different from
the primary and diffuse lipoid degeneration characteristic of this disease.
Such a border-line case was recorded by Braun (1933) which showed a slight facial
weakness, left-handedness, and facility in mirror-writing. Bonnet and Colrat (1934)
described a similar family exemplifying the Stargardt type wherein both mother
(aged 43) and daughter (aged 23) revealed spasticity of the cerebellar type ; we shall
1 p. 2801.
2792 TEXT-BOOK OF OPHTHALMOLOGY
See that a selective neuronic degeneration of the granular layer of the cerebellum occurs
in maculo-cerebral degeneration (Greenfield and Holmes, 1925)."
Other complications are not common : a tendency to ocular haemorrhages (Mazzi,
1934, in a case of the adult type), microphthalmos (Borsellino, 1935), an anomaly of the
hair which had lanugo characteristics (Wagner, 1935), and so on.
About the pathology little is known ; presumably it corresponds to
that characterizing the outer retinal layers in juvenile family amaurotic
idiocy.”
Batten, F. E. T. O. S., xxiii, 386, 1903.
Batten, F. E., and Mayou. Proc. R. S. Med.,
Sect. O., viii, 70, 1915.
Batten, Rayner. T. O. S., xvii, 48, 1897;
xlii, 109, 1922; xliii, 550, 1923 : xlv.,
468, 1925.
Beach. A. of O., xv, 776, 1936.
Behr. K. M. Aug., lxv, 465, 1920.
Best. Z. f. Aug., xiii, 199, 1905.
Bonnet and Colrat. Bull. S. d’O. Paris, xlvi.
333, 1934.
Borsellino. Rass. It. Ott., iv., 181, 1935.
Braun. K. M. Aug., xci, 182, 1933.
Gasteiger. Med. Kl., ii, 1455, 1936.
Greenfield and Holmes. Brain, xlviii, 183,
1925.
Hutchinson. R. L. O. H. Rep., viii, 231, 1875.
Jung. B. O. G. Heidel., li, 1936.
Knapp. A. of O., i, 311, 1929.
Larsen. K. M. Aug., lxvii, 301, 1921.
No effective treatment exists.
Lisch. K. M. Aug., xcviii, 498, 1937.
Mayou. T. O. S., xxiv, 142, 1904.
Mazzi. A. di Ott., xli., 225, 1934.
Nathan. A m. J. O., xxi, 1029, 1938.
Nettleship. T. O. S., xxviii, 76, 1908.
Oatman. Am. J. Med. Sc., czlii, 221, 1911.
Rieger. Z. f. Aug., lxx, 22, 1929.
Savin. Proc. R. S. Med., xxx, 390, 1936.
Schiff-Wertheimer and Tillé. A m. d’Oc.,
clxvii, 1, 1930.
Sorsby. T. O. S., liv, 160, 1934.
Brit. J. O., xix, 65, 1935.
Stargardt. A. f. O., lxxi, 534, 1909.
Z. f. Aug., xxx, 95, 1913.
Stephenson. T. O. S., xxiv, 144, 1904.
Thomson. Brit. J. O., xvi, 681, 1932.
Vossius. A. f. O., cv, 1050, 1921.
Wagner. A. f. O., cxxxiv, 74, 1935.
Weisel. Diss., Giessen, 1922.
Wright. Brit. J. O., xix, 160, 1935.
Leber. G.-S., Hb., II, viii (A 2), 1204, 1916.
Familial Lipoid Degenerations
Although the matter cannot be said to be settled, in the present state
of our knowledge it seems legitimate to consider together three diseases
which appear to be due to a widespread lipoid degeneration : Tay-Sachs’
disease (infantile amaurotic family idiocy), Batten-Mayou’s or Spielmeyer-
Vogt's disease (juvenile amaurotic family idiocy; maculo-cerebral degenera-
tion); and Niemann-Pick's disease (lipoid spleno-hepatomegaly or lipoid
histiocytosis). Although the three conditions are not identical, the essential
pathological changes in all are the same—the appearance in great numbers
of vacuolated “foam ” cells undergoing lipoid degeneration. It would
seem probable that if this degeneration is limited to the central nervous
system, which includes the ganglion cells of the retina, the clinical picture
of Tay-Sachs' disease or Batten-Mayou’s disease results; if it spreads
beyond the central nervous system, or excluding the central nervous system
spreads throughout the rest of the body and inundates a number of organs,
the Niemann-Pick type results (Sachs, 1931).
The aetiology of such an inundation is quite obscure, for it is as yet
unknown whether it results from a disturbed activity of the reticulo-
1 p. 2796. * p. 2803.
DISEASES OF THE RETINA 2793
endothelial system in the metabolism of lipoids, or alternatively, a flooding
of the entire body with lipoids, mainly lecithin. A remarkable feature is
that in the infantile form both diseases occur almost exclusively in infant
children of the Jewish race, mainly of the female sex, and both of them have
a strong familial tendency. This suggested to the older writers a toxic
cause, probably dietary, acting either in utero or soon after birth, when it
seemed most probable that the vehicle must be the mother’s milk (Nettleship,
1908). Apart from its general unlikeliness, however, this theory seemed
ruled out of court by the fact of its occurrence in babies who were not reared
at the breasts of their mothers (Sachs, 1887–98), or were weaned after a very
short suckling period (Peterson, 1898).
Historically the first recognition of such a disease was due to the London ophthal-
mologist Waren Tay (1881–84), and although the ophthalmoscopic picture was
recognized by several writers (Knapp, 1885; Magnus, 1885; Goldzieher, 1885;
Wadsworth, 1887), its full clinical elucidation from the central nervous aspect was
essentially due to the studies of Sachs (1887–98); the condition as it was observed in
infants therefore became known as TAY-SACHs' DISEASE. Shortly thereafter, following
the clinical description of two cases (Kingdon, 1892–94). the pathology of the central
nervous changes were detailed by Kingdon and Russell (1897), when the peculiar
vacuolated degeneration of the cells was clearly established, and it was recognized that
the appearances observed were pathognomonic and quite different from those seen in
any other form of central nervous degeneration or idiocy. These observations have
been amply confirmed, the peculiar degeneration affecting not only cells of the central
nervous system, but also the cells of the spinal and sympathetic ganglia and the
ganglion cells of the retina (Poynton, Parsons and Holmes, 1906; Schaffer, 1906; Mott,
1907; Hancock and Coats, 1911 ; and others). Finally, the presence of lipoid material
of considerable quantity was demonstrated both in the cells of the brain (Sachs and
Strauss, 1910) and eventually in the ganglion cells of the retina (Grinker, 1927).
Some years after the infantile type of amaurotic family idiocy had been established
as a clinical entity, F. E. Batten (1903) observed ophthalmoscopically a symmetrical
pigmented macular lesion which he compared with an atypical form of retinitis
pigmentosa in two sisters, which became apparent at the ages of 5 and 7 years and was
associated with mental backwardness. In the following year similar cases were
described by Mayou (1904) and Stephenson (1904), and the pathology of one of Batten’s
original patients, who died with general central nervous degeneration, was described by
Batten and Mayou (1915). This familial disease characterized by macular degenera-
tion and cerebral symptoms coming on about the age of the second dentition or
puberty was therefore known in England as the BATTEN-MAYOU DISEASE, while in
America Oatman (1911) labelled it FAMILIAL MACULO-CEREBRAL DEGENERATION.
Meantime it had been demonstrated in neurological studies by Spielmeyer
(1905–06) and H. Vogt (1905) that a disease somewhat similar to that of Tay-Sachs,
characterized also by amaurosis, paralysis and idiocy, could occur during the end of
the first and the beginning of the second decade, the ocular complications of which were
worked out by Stock (1908); this juvenile type is therefore variously known in
Continental literature as the SPIELMEYER-VOGT or SPIELMEYER-STOCK disease. A
similar, although perhaps not identical, lipoid degeneration was demonstrated in the
central nervous system ; and in the eye, while the same changes affected the ganglion
cells, an additional primary degeneration affected the outer layers, producing a totally
different ophthalmoscopic picture of pigmentary degeneration.
Long after these conditions had become known the NIEMANN-PICK TYPE was
2794 TEXT-BOOK OF OPHTHALMOLOGY
differentiated, this time by a physician and not an ophthalmologist. Niemann (1914)
was the first to describe in a female infant a disease which resembled, but was not
identical with, Gaucher's disease, and 12 years later Pick (1926), analysing the literature
on this latter condition, separated from the cases formerly classed as Gaucher's disease
a considerable number which constituted a separate clinical and pathological entity
characterized by the presence of vast numbers of foam cells in most tissues of the body.
Because the spleen and liver were especially affected he proposed the term LIPOID SPLENO-
HEPATOMEGALY. Since, however, the great majority of foam cells were histiocytic in
origin and since they pervaded almost all the organs in addition to the liver and spleen,
and since lipoid deposits were also found in other cells—epithelial, endothelial, muscular,
ganglionic, glial, and Schwannian—Bloom (1925–28) proposed the term ESSENTIAL
LIPOID HISTIOCYTOSIS. Some time previously, Knox, Wahl and Schmeisser (1916) had
noted the peculiar and striking ophthalmoscopical appearance characteristic of
Tay-Sachs' disease in a case taken to be an example of Gaucher's disease. At a later
date, however, it became evident that the case was in reality one of the Niemann-Pick
group ; and the subsequent reports of four other cases showing a similar association
(Hamburger, 1927; Pick and Bielsehowsky, 1927; Bloom, 1928; Kramer, 1928)
seemed to indicate that the apparent co-existence of the two diseases was really due
to the fact that in those cases the amaurosis and retinal appearance were merely an
extension of the generalized lipoid disturbance to affect the cells of the central nervous
system as well as the other organs of the body. The matter seemed confirmed by the
pathological investigations of Pick and Bielschowsky (1927) who found that the histo-
logical changes in the brain in Niemann-Pick's disease were identical with those in
the Tay-Sachs type, but only more extensive, the lipoid material infiltrating the
connective tissue cells of the leptomeninges, the tela choroidea, and the walls of the
cerebral blood-vessels. Similarly in the eye, Goldstein and Wexler (1931) found the
same changes in the Niemann-Pick as in the Tay-Sachs type, except that instead of being
limited to the ganglion cells as in the former, here again they were much more widely
distributed, affecting also the nuclear layers of the retina, while a histiocytic infiltration
of foamy cells invaded the uveal tract, the sclera and the vascular adventitia.
AMAUROTIC FAMILY IDIOCY
Amaurotic family idiocy may therefore be defined as a familial disease of
early life due to a primary lipoid degeneration of the ganglion cells of the entire
central nervous system, in which the ganglion cells of the retina participate,
characterized by unique bilateral fundus appearances and invariably terminating
in blindness, paralysis, idiocy and death.
Several forms have been described depending on the age of onset.
The most common is the INFANTILE FORM which appears during the first
year of life, usually about the 5th month (TAY-SACHs' DISEASE); the LATE
INFANTILE FORM occurs during the second or third years, and the JUVENILE
FORM during the second half of the first decade or the first half of the second
(the BATTEN-MAYOU or SPIELMEYER-STOCK or SPIELMEYER-VOGT DISEASE).
The infantile form, like the Niemann-Pick disease, with extremely rare
exceptions (Magnus, 1885; Cockayne and Attlee, 1914; Cohen, 1923;
Condes and Horner, 1929; Paton, 1932; and others) is confined to members
of the Jewish race, but this is not true of the late infantile and juvenile forms.
In all its forms the disease is inevitably progressive and fatal, but the
DISEASES OF THE RETINA 2795
younger the patient the more rapid the downward course ; in the infantile
form death occurs usually in from 1 to 2 years, but in the juvenile form it
may be delayed for 5 or more years.
Although familial and therefore presumably due to an inherited predisposition,
there is usually no history of a similar disorder in previous generations. Exceptions,

|
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& * | | ||
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ſºlº
|| HT
ò º º & © & & )



FIG. 2402.—AMAUROTIC IDIOCY OF TAY-SACHs (Falkenheim-Rochliny).
however, occur (Goldfeder, 1927), one of which, a pedigree involving three generations
studied by Falkenheim (1901) and Rochliny (1924), is seen in Fig. 2402. The familial
character and clinical course is well ex-
emplified in the cases reported in the early C’— O
literature. Peterson’s family showing the +
infantile form is interesting in that it contains |
the great rarity of a long survival–5 children | | | |
(Russian Jews): 1st, normal, aged 7 ; 2nd, Ö O O O

blind at 6 months but alive and idiotic at 5%
years ; 3rd, normal, aged 4 ; 4th, died blind l 2 3 4
and idiotic at 10 months ; 5th, died blind FIG. 2403.−CEREBRO-RETINAL
and idiotic at 7 months. Koller (1896) also DEGENERATIONs. & ©
recorded a case which survived at least 4 years The parents were consanguinious,
e - e - and 2. Cerebellar heredo-ataxy and
by which time it had become a passionate optic atrophy. 3. Cerebellar diplegia
idiot. Sjögren (1931) found that the juvenile and optic atrophy. 4. Amaurotic idiocy
form is fairly common in Sweden where he (Higier).
considered that the ancestors were probably
heterozygotes, the disease following the course of a recessive monohybrid inheritance
with a distinct tendency to accumulation in certain defined areas in different parts of
the country. It is interesting that related degeneracies in the central nervous system
are frequently encountered in the same family (Higier, 1906) (Fig. 2403).
The clinical picture presented by all forms of the disease is similar. The
child is normal at birth, but eventually becomes apathetic and fails to raise
its head or sit up owing to weakness of the muscles of the neck and back.
As time goes on it takes less and less interest in its surroundings, progressive
2796 TEXT-BOOK OF OPHTHALMOLOGY
flaccid paralysis sets in, convulsions may occur, mental deterioration
increases, and finally it becomes blind and completely paralysed, failing to
respond to external stimuli, and dies in a state of marasmus. In the
juvenile type the general picture is similar except that convulsions are
commoner and spasticity and contractures may replace the flaccidity
characteristic of the infantile form, the child frequently dying in epileptiform
convulsions.
The pathology of the condition, as we have seen, has received a con-
siderable amount of attention (Kingdon and Russell, 1897; Vogt, 1905;
Fies. 24.04–05.-AMAuroric Family Idiocy : THE CEREBELLAR Cortex.
º-l. -
--- º º -----
º:
--- º
º
-
º --~~~~ - -
º
--
- º: º:
Fig. 2404–Stained by Bielsehowsky’s Fig. 2405-Stained by Scharlach R- and
silver method. (x 150) (Greenfield and haemalum. The lipoid swellings on the
Nevin, T. O. S.). dendrites of the Purkinje cells in Fig. 2404
are seen to be full of lipoid in Fig. 2405
(x 150) (Greenfield and Nevin, T. O. S.).
Poynton, Parsons and Holmes, 1906; Schaffer, 1906; Mott, 1907; Sachs
and Strauss, 1910; Bielschowsky, 1914–20; Torrance, 1922; Greenfield and
Holmes, 1925; Hurst, 1925; Hassin, 1926; Greenfield and Nevin, 1933;
and others). In the brain the characteristic change is found in the ganglion
cells, which first undergo a swelling of the hyaloplasm and subsequently a
granular degeneration with the deposition of lipoid material, while large
lipoid swellings are seen on the dendrites (Figs. 2404–06). The white
matter shows tract-degeneration with a profuse secondary neuroglial over-
growth, and there is a widespread accumulation of fat-granule cells. From
the histo-chemical point of view the lipoid is allied to the substances forming
normal myelin, and is probably closely related to the cerebrosides. In the












DISEASES OF THE RETINA
2
7
9
7

- -
- .. --
- -- - - - - -*
- - ---, - - - - - - - -
- - - º " wº. - - -- - - * º,
*** --- *, * * * *----- ** **, * ſº...º.
** -- " -- .*-------- - ----- - - ---
*** -ºº: º, ºrº
: ***, *, º ºsºvº º ºſº
ºf "...º.º.º.º.º. º.º.º.
*- -- - - : - - . - -- ": . - - º
***"... º. º.º.
ºf ºººººººº;
- **. º --. º ...t." **: -- º
. . *- ** * º - º **** º - - º
* > - -º-º-º: --_º - -
"º
- -- *. - º -- º º -
--- - --- --- - --- º º º -
- --- * - -º-º-º: º
- - - -- -- - -- - - -
-- .* - - " -, - -
- - - -" * -- * * - - - "... - , * -- **: º:
- - - --- - - - - - - - -
- * * - *. º - - º, … ...? ****** *
--- º - º - - - - º º: º *.
; : 'º' ... * * * * *
- - - - --- - -
-
Fig. 2406.-AMAURotic FAMILY I Dioc y : THE CEREBRAL Cortex.
Stained with Scharlach R. and haemalum. The fat granule cells appear as
black irregular bodies contrasting with the less intensely stained lipoid in the nerve-
cells (x. 50) Greenfield and Nevin, T. O. S.).
infantile form the cortex and the thalamus as well as the ganglion cells of
the spinal cord show severe changes; in the juvenile form the cerebellum is
particularly affected.
(a) THE INFANTILE FORM of AMAURotic FAMILY Idiocy (TAY-sachs’ Disease)
The fundus picture in the infantile form is quite characteristic and very
striking, being similar to that seen in sudden obstruction of the central
retinal artery although being due to an entirely different cause (Fig. 23.85,
Plate LVI). Around the macula there is a round, brilliantly white, some-
what elevated area fading off peripherally to the normal fundus, and in its
midst, situated at the fovea itself, is a small dark spot with somewhat fuzzy
margins varying in colour from brownish to cherry red and frequently lighter
in its centre. In the later stages optic atrophy develops with a chalky
2798 TEXT-BOOK OF OPHTHALMOLOGY
white disc and attenuated vessels, but the rest of the fundus remains
normal.
The first histological description of the changes in the retina was
Fig. 2407.-AMAurotic FAMILY IDiocy.
General view of the macular region, showing advanced changes, including folding
of retina, absence of the nerve-fibre layer and great swelling of the reticular layers
(Poynton, Parsons and Holmes, Brain).
published by Collins (1897) whose paper was followed by those of Holden
(1898), Shumway and Buchanan (1905), Poynton, Parsons and Holmes
(1906), Cohen (1907), Parsons (1908), Verhoeff (1909), Schuster (1909),
Fig. 2408.-AMAurotic FAMILY Idrocy.
Detail of macular region (Hagedoorn, A. of 0.).
Hancock and Coats (1911), Harbitz (1913), Bielschowsky (1920), Grinker
(1927). Heath (1933; and others. The pathological picture is similar
to that in the brain, the characteristic feature being a lipoid degeneration
of the ganglion cells and a consequent atrophy of the nerve fibres


DISEASES OF THE RETINA 2799
Fig. 2409.-AMAUROTIC FAMILY ID10cy.
The retina near the macular region. The ganglion cells (above) are swollen
and the nuclei eccentric ; the nerve-fibre layer is absent and the reticular layers
consist of spaced-out neuroglial elements (Poynton, Parsons and Holmes, Brain).
(Figs. 2407–10). The cells themselves are swollen and rounded, their
nuclei eccentric, and the cytoplasm vacuolated and foamy. In the nuclear
layers there is much less change although there may be thickening and a
spacing out of the individual elements by coagulated exudate. In these
layers, however, the cells themselves are normal. The peri-macular white
opacity is due to the thickening and degeneration of the ganglion cell layer
Fig. 2410.-AMAURotic FAMILY IDiocy.
The retina, showing staining for lipoid (Hagedoorn, A. of 0.).
T.O. — wol. III. 7. /


2800 TEXT-BOOK OF OPHTHALMOLOGY
which may be swollen to enormous dimensions (Hancock and Coats, 1911;
Heath, 1933); it is not the result of oedema. The red spot at the macula
is due to a contrasting attenuation of the fovea or sometimes its disappearance
with the formation of a hole (Parsons, 1908) so that the choroid is clearly
visible through.
(b) Essential Lipomp Histocytosis (NIEMANN-Pick's DISEASE)
This entity may be defined as a lipoid degeneration and infiltration of
most of the organs of the body, especially the liver and spleen, similar in
Fig. 24.11.-NIEMANN-Pick's Disease.
The cherry-red spot at the macula. Showing oedema of Müller's fibres in the
inter-nuclear layer (Bielschowsky’s stain) (Goldstein and Wexler, A. of 0.).
incidence to the preceding and equally incompatible with survival. Some-
times the cells of the brain and central nervous system are not affected, in
which case ophthalmological symptoms are not evident; sometimes they
* *
Fig. 24.12.-NIEMANN-Pick's Disease.
A group of ganglion cells at the macula showing various degrees of vacuolation
and necrosis. The more intact cells exhibit pyknotic nuclei (P) (Goldstein and
Wexler, A. of 0.).
are, and in this event the clinical and pathological picture of amaurotic
idiocy is accentuated (Figs. 2411–12). Clinically, the same grey-white area
with the central brownish-red spot appears, but the whole retina becomes


DISEASES OF THE RETINA 2801
mottled and the disc has a yellow, waxy, atrophic appearance, while the
associated symptoms of amaurosis and idiocy are identical. Pathologically
the lipoid degeneration is noted not only in the ganglion cells, but a foamy
vacuolation occurs in the cells of nuclear layers as well, giving rise to the
º
Fig. 24.13.-NIEMANN-Pick's Disease.
The inner nuclear layer of the retina, showing a group of vacuolated cells
(Goldstein and Wexler, A. of 0.).
mottled appearance of the fundus (Fig. 2413); in the optic nerve there is a
lipoid deposition in the glial cells, in the cells of the nerve-fibre sheaths and
in the cells of the septa, while an infiltration of foamy cells, probably
histiocytic in origin, permeates the choroid, the sclera, and is especially
prominent around the vessels of the sclera and the episclera (Fig. 2414)
º -->
* -
- º
FIG. 2414.—NIEMANN-Pick's Disease.
The choroid, showing cells containing pigment granules and vacuoles of lipoid
(Goldstein and Wexler).
(Goldstein and Wexler, 1931). These investigators found that even the
cells of an accessory episcleral ganglion exhibited similar changes. In
general, therefore, the pathology resembles that of the Tay-Sachs type with
an extension of the process to include practically all the tissues of the eye.
(c) THE JUVENILE ForM of AMAURotic FAMILY Idrocy; MACULo-
CEREBRAL DEGENERATION
(BATTEN-MAYou or SPIELMEYER-Vogt Disease)
The clinical picture of the JuvBNILE ForM of AMAURotic FAMILY
IDIocy (MACULo-CEREBRAL DEGENERATION) is quite different from that of
the infantile form in that the cherry-red spot at the macula is absent and
Z Z ~


2802 TEXT-BOOK OF OPHTHALMOLOGY
replaced by a finely mottled pigmentation of the pepper-and-salt type. It is
therefore not so pathognomonic, and resembles that seen in familial macular
degeneration without cerebral symptoms.” We have already seen that it
was first described by F. E. Batten (1903) in two sisters in whom the condition
Figs. 2415-17.-CEREBRo-MacULAR DEGENERATIox.
Showing three consecutive stages (Paton).
Fig. 2415.-CEREBR.o-macular Fig. 2416.-CEREBR.o-macular
DEGENERATIow. DEGENERATIow.
A small white spot at the macula with a Accentuated macular lesion, peri-vascular
normal disc. Eight months previously the disturbance, attenuated arteries, and pale
fundus was normal. dise.
Fig. 2417. cerebro-macular DEGENERATIow.
Final macular change, general retinal and optic atrophy.
was well developed; but the fullest clinical description is that of Holmes
and Paton (1925) who observed it in three members of a family in various
stages of its development.
At first the disc and the vessels are normal and the macula is occupied
by a tiny white area surrounded by a red areola, somewhat as if the fovea
* p. 2788.


DISEASES OF THE RETINA 2803
was unusually deep and bright (Fig. 2415). Later, however, the disc becomes
pale and the vessels smaller, while at the macula the white area increases,
the red areola is pronounced with a peppering of pigment, and in the
surrounding retina there are usually radiating branching white streaks
(Fig. 2416). In the later stages the disc is quite atrophic, the vessels thread-
like, the peripheral retina besprinkled with a dust-like peppering of pigment,
while in the macular region the atrophic area enlarges and becomes a highly
pigmented irregular patch without sharp outlines but shading off gradually
into an opaque peri-maeular area (Fig. 2417). At this stage the whole
retina becomes atrophied and the choroidal vessels are clearly visible.
Sometimes optic atrophy may be an early symptom, and if it is prominent
before the macular changes develop, a mistaken diagnosis may be made
(Pollock, 1917–32).
The pathological picture resembles a combination of the features of
amaurotic family idiocy and a primary pigmentary degeneration of the
neuro-epithelium, showing on the one hand a lipoid degeneration of the
ganglion cells and on the other a disappearance of the outer retinal layers
with their replacement by neuroglial proliferation and a migration of
pigment into the degenerated region. The more striking feature is the
changes in the outer layers, but the two degenerations appear to be inde-
pendent (Stock, 1908; Ichikawa, 1904; Wandless, 1909; Savini-Castano
and Savini, 1913; Bielschowsky, 1914–20 ; Batten and Mayou, 1915;
Rönne, 1916 ; Dide, Guiraud and Michel, 1920 ; Torrance, 1922 ; Holmes
and Paton, 1925). The degeneration of the neuro-epithelium appears without
doubt to be primary in nature, for the choroid has always been found to be
normal, and no vascular or toxic lesion has been discovered which might
account for it. Moreover, there is evidence of similar selective degenerations
in other systems of neurons, such as the granular layer of the cerebellum
(Greenfield and Holmes, 1925).
The picture varies in different cases. The inner layers of the retina are
always similarly affected, the ganglion cells undergoing the same primary
lipoid degeneration with consequent atrophy of the nerve-fibres as occurs
throughout the central nervous system. The outer layers of the retina,
however, are variously affected ; sometimes the changes here have been
relatively inconspicuous (Batten and Mayou, 1915; Dide, Guiraud and
Michel, 1920 ; Torrance, 1922); at other times they seem to be preponderant
(Stock, 1908; Bielschowsky, 1914–20 ; Rönne, 1916; Holmes and Paton,
1925), and in the more extreme cases a complete degeneration of all the
nervous elements of the retina results (Ichikawa, 1909; Wandless, 1909).
The degeneration is always most pronounced at the macula and becomes
less as the periphery is reached. The earliest recognizable change is a
breaking up of the rods and cones with a rarefaction and narrowing of the
outer nuclear layer, a process which probably affects first the cell-bodies of
these elements or involves simultaneously the whole neuron unit (Holmes and
2804 TEXT-BOOK OF OPHTHALMOLOGY
Paton, 1925) (Fig. 2418); the early loss of the outer segments of the rods
and cones reported in an anomalous case by Savini-Castano and Savini
(1913) was probably a post-mortem change. The next stage is represented
by the replacement of the degenerated outer neuron by proliferating
neuroglia, followed by an overgrowth of the pigmentary epithelium and the
Figs. 24.18–19.-CEREBR.o-MACULAR DEGENERATION: THE PERIPHERAL RETINA
-
º -
--
*.
Fº
- - -
Fig. 2418.-A less affected zone, Fig. 2419.-A severely affected
showing shrinkage of inter- zone. a. Ganglion cell layer.
nuclear layer (a), thinning of b. Inner reticular layer.
the outer nuclear layer (b),
and pigment migration among
the rods and cones (c) (Holmes
and Paton),
c. Inner nuclear layer, d. De-
generated and sclerosed tissue.
e. Pigmentary epithelium
(Holmes and Paton).
invasion of the retina by pigment granules. Eventually the retina becomes
much reduced in thickness, the only recognizable layers being the ganglion
cells and their fibres, the inner reticular layer, and the inner nuclear layer;
outside this is a mass of irregular tissue consisting essentially of glia and a
considerable amount of pigment, sometimes heaped up in oval masses
(Fig. 2419). Finally, the whole structure of the retina disappears in a uniform
degeneration of all the nervous elements.


DISEASES OF THE RETINA 2805
(d) LATE INFANTILE FORM OF AMAUROTIC FAMILY IDIOCY
The late infantile form of the disease, which occurs in the first and
second years of life and is not limited to Jews, has been extensively studied
by Bielschowsky (1920), Torrance (1922), Hassin (1926), Marinesco (1930),
Greenfield and Nevin (1933), and others. There is some doubt whether it
should be regarded as a clinical group possessing distinctive features, but it
seems more probable that such cases should be regarded as transitional
between the other two ; when the disease sets in early it resembles the
infantile form, and when it appears towards the end of the period the
character changes towards that of the juvenile form. The occurrence of a
cherry-red spot at the macula is rare (Wolfsohn, 1915; Condes and Horner,
1929; Greenfield and Nevin, 1933); more frequently the ophthalmoscopic
changes are pigmentary. Pathologically the first class shows only a lipoid
degeneration of the ganglion cells (Greenfield and Nevin, 1933) while
the other has in addition changes analogous to those found in juvenile cases
(Bielschowsky, 1920).
In all these conditions the prognosis is bad and no effective treatment
is known.
Batten and Mayou. P. R. S. Med., Sect. O., Holden. J. Nerv. and Mental Dis..., xxv, 550,
viii, 70, 1915. 1898.
Batten, F. E. T. O. S., xxiii, 386, 1903. Holmes, Parsons and Poynton. Brain, xxix,
Bielschowsky. Deut. Z. f. Nervenheilk., l, 7, 180, 1906.
1914.
J. f. Psy. u. Neurol.., xxvi, 123, 1920.
Bloom. A m. J. Path., i, 595, 1925.
J. Am. Med. As., xc, 2076, 1928.
A. of Path., vi, 827, 1928.
Cockayne and Attlee. P. R. S. Med., Sect. O.,
viii, 65, 1914.
Cohen. J. Am. Med. As., xlviii, 475, 1907.
A. of O., lii, 140, 1923.
Collins. Med. Chir. Trans., lxxx, 102, 1897.
Condes and Horner. Am. J. O., xii, 558, 1929.
Dide, Guiraud and Michel. L'Encephale, xv,
303, 1920.
Falkenheim. Jhb. f. Kinderheilk., liv, 123,
1901.
Goldfeder. K. M. Aug., lxxix, 176, 1927.
Goldstein and Wexler. A. of O., v, 704, 1931.
Goldzieher. Wien. med. W., xxxv, 336, 1885.
Greenfield and Holmes. Brain, xlviii, 183,
1925.
Greenfield and Nevin. T. O. S., liii, 170, 1933.
Grinker. A. of Path., iii, 768, 1927.
Hamburger, R. Jhb. f. Kinderh., cxvi., 41,
1927.
Hancock and Coats. Brain, xxxiii, 514, 1911.
Harbitz. A. f. Aug., lxxiii, 140, 1913.
Hassin. A. of Newrol. and Psy., xvi, 708,
I926.
Heath. A. of O., x, 342, 1933.
Higier. Deut. Z. f. Nervenhk., xxxi,
1906.
231,
Holmes and Paton. T. O. S., xlv., 447, 1925.
Hurst. Brain, xlviii, l, 1925.
Ichikawa. K. M. Aug., xlvii (1), 73, 1909.
Kingdon. T. O. S., xii, 126, 1892; xiv., 129,
1894.
Ringdon and Russell.
Trans., lxxx, 87, 1897.
Knapp. B. O. G. Heidel., xxiii, 217, 1885.
Rnox, Wahl and Schmeisser. Bull. Johns
Hopkins H., xxvii, 1, 1916.
Med. Chir. Soc.
Koller. T. Am. O. S., vii, 661, 1896.
Kramer. Med. Clim. N. A mer., xi, 905, 1928.
Magnus. K. M. Aug., xxiii, 42, 1885.
Marinesco. J. f. Psy. u. Neurol.., xli., 1, 1930.
Mayou. T. O. S., xxiv, 142, 1904.
Mohr. A. f. Aug., xli., 285, 1900.
Mott. A. of Neurol., iii, 218, 1907.
Nettleship. T. O. S., xxviii, 76, 1908.
Niemann. Jhb.f. Kinderh., lxxix, 1, 1914.
Oatman. Am. J. Med. Sci., czlii, 221, 1911.
Parsons. Path. of the Eye, London, iv, 1365,
1908.
Paton. T. O. S., lii, 226, 1932.
Peterson. J. Nervous and Mental Dis..., xxv,
529, 1898.
Pick. Erg. d. inn. Med. w. Kinderh., xxix,
519, 1926.
Pick and Bielschowsky. Kl. W., vi (2), 1631,
1927.
Pollock. T. O. S., xxxvii, 247, 1917; lii, 220,
1932.
2806
TEXT-BOOK OF OPHTHALMOLOGY
Poynton and Parsons. T. O. S., xxv, 312,
1905; xxvi, 77, 1906.
Poynton, Parsons and Holmes. Brain, xxix,
180, 1906.
Rochliny. Vracebnaje Delo, Nr. 8/9, 1924. :
Ref. Goldfeder, q.v.
Rönne. K. M. Aug., lvi, 497, 1916.
Sachs. J. Nervows and Mental Dis., xiv, 541,
1887.
N.Y. Med. J., liii, 697, 1896.
Deut. Med. W., xxiv, 33, 1898.
A. of O., v, 506, 1931.
Sachs and Strauss. J. Earp. Med., xii, 685,
1910.
Savini-Castano and Savini.
vii, 321, 1913.
Schaffer. A. f. Psy. u. Nervenkr., xlii, 127,
1906.
Z. f. Kinderh.,
Schuster. A. f. Aug., lxiv, 1, 1909.
Shumway and Buchanan. Amer. J. Med.
Sc., czzix, 35, 1905.
Sjögren. Hereditas, xiv, 197, 1931.
Spielmeyer. A. f Psy. w. Nervenkr., xl, 1038,
1905.
Nissl's List. Arbeiten, ii, 1906.
Stephenson. T. O. S., xxiv, 144, 1904.
Stock. K. M. Aug., xlvi (1), 225, 1908.
Tay. T. O. S., i, 55, 1881; iv., 158, 1884.
Torrance. Glasgow Med. J., xcvii, 193, 263,
341, 1922.
Verhoeff. A. of O., xxxviii, 107, 1909.
Vogt, H. Momat. f. Psych. w. Neurol.., xviii,
161, 1905.
Wadsworth. T. A m. O. S., iv, 572, 1887.
Wandless. N. Y. Med. J., lxxxix, 953, 1909.
Wolfsohn. A. Int. Med., xvi, 257, 1915.
VI. CYSTS OF THE RETINA
Taken in its widest sense as embracing cystoid degenerative conditions,
retinal cysts are extremely common, and, as we have seen," occur invariably
in senile eyes. Taken in the narrower sense, however, as representing an
active accumulation of fluid rather than a passive formation of gaps in a
rarified tissue, the condition is relatively rare. A clear differentiation
between the two is impossible, for in a condition which started as a purely
degenerative disappearance of neural elements or in a passive accumulation
of oedematous fluid, individual cysts of considerable size may be formed.
From the aetiological point of view retinal cysts may be divided thus:—
1. CongBNITAL CYSTs are frequently associated with microphthalmos
and colobomata ; these have already been discussed.” In otherwise well-
formed eyes cysts which are apparently congenital occur either in the
retina (de Schweinitz aſſº Wiener, 1919; Stieren, 1933) or associated
with the disc and presumably frequently with hyaloid remains (Koller, 1901 ;
McCulloch, 1930; Schwartz, 1936; and others). Probably most of the
cysts found floating freely in the vitreous are of this nature. It is interesting,
however, to note that it is possible that a developmental anomaly or weak-
ness resulting in the formation of a cyst near the Ora may be the cause by its
rupture of many cases of retinal dialysis leading to detachment, particularly
those occurring symmetrically in young hypermetropic eyes (Weve, 1935;
Ridley, 1935; Schmelzer, 1936; A. Fuchs, 1937; Korrzweig, 1940; and
others).
2. As a terminal culmination of CYSTIC DEGENERATION,” the formation
of cysts of considerable size is rare both in the periphery and at the macula,
whether formed as the result of purely degenerative, oedematous or toxic
1 p. 2752. * Vol. II, p. 1268. 8 p. 2752.
DISEASES OF THE RETINA 2807
processes (Fig. 2422). Such cysts occurring at the periphery may give rise
to a sharply localized globular detachment of the retina (Fig. 2424).
3. In ATRoPHIC AREAs of retinitis or after traumatism or irido-cyclitis,
cyst-formation, sometimes of considerable size, may occur (Veil and Guillau-
mat, 1938) (Fig. 2420).
4. TrAction cysts may be formed by the pull of shrinking fibrous
Fig. 2420.-RETINAL Cyst.
A bluish cyst in association with an area of chorio-retinal exudation, resembling
in some respects Coats' disease. There was a history of birth-injury (Hine, T. O. S.).
bands in the vitreous or of pre-retinal membranes (Neame, 1920; Fuchs,
1921).
5. PARAs.ITIc cysts will be dealt with separately."
Pathologically retinal cysts usually start in the inner nuclear layer, and
as they grow the layers of the retina are pushed aside. Sometimes, especially
in old areas of inflammation, the progress of the cyst is confined by the glial
elements which become elongated and hypertrophied. Such a cyst may
eventually occupy the entire thickness of the retina and is bounded by glial
* Chap. XLI.

2808 TEXT-BOOK OF OPHTHALMOLOGY
tissue and the internal and external limiting membranes (Kronfeld, 1935)
(Fig. 2422). At other times when the tissues are less consolidated a wide-
Fig. 2421.-RETINAL Cysts.
On the right a large cyst bisected; on the left a small cyst unopened (x 24)
(Neame, T. O. S.).
spread splitting of the retina occurs between the two nuclear layers and a
cyst of considerable size may be formed producing an ophthalmoscopically
Fig. 24:22–Cyst of the RETINA.
A cystic space occupying the entire thickness of the retina at the site of an
atrophic chorio-retinitic lesion. The extra-cellular pigment lies on the inner surface
of the retina (x 155) (Kronfeld, A. of 0.).
visible mass and a widespread retinal detachment (Lawford, 1887;
Collins, 1893; Neame, 1920; and others) (Figs. 2421. 24). It is noteworthy


DISEASES OF THE RETINA - 2809
that the bulging takes place outwards, the inner layers of the retina retaining
their normal position.
From the clinical point of view such cysts may be divided into two
classes: those which appear as a tumour-like mass, and those which are
associated with a widespread retinal detachment. The first type is rarer,
and such cysts are usually diagnosed as neoplasms, for which the eye has
been removed (Collins, 1893; de Schweinitz and Shumway, 1901; Velhagen,
1912; Deutschmann, 1914; Neame, 1920; Coulter, 1920; Parsons, 1920;
and others). It is not common for a clinical diagnosis to be made; in these
FIG. 2423.−RETINAL Cyst.
Shows the normal retina splitting at the posterior end of the large cyst seen in
Fig. 2421, separating the two nuclear layers (x 60) (Neame, T. O. S.).
cases there is usually a partial retinal detachment with a globular swelling of
cystic appearance at its edge usually overhanging the disc (Thompson,
1890; Derby, 1909) or situated in the periphery (Cridland, 1920; Butler,
1922). Apart from the localized globular appearance of a detachment in
which no hole can be seen, the diagnosis depends essentially on two tests.
First, the results of transillumination; but it must always be remembered,
that a lightly pigmented neoplasm may be translucent,” while a cyst may
have sufficient pigment in its walls to render it quite opaque (Neame, 1920).
The conclusive test is by tapping, when a clear straw-coloured albuminous
fluid is usually obtained; such a procedure is followed by a collapse of the
* p. 2514.

28.10 TEXT-BOOK OF OPHTHALMOLOGY
cyst but in most cases it rapidly re-forms (Cridland, 1920). It is, however,
a perfectly safe diagnostic measure to adopt, particularly prior to excision
in a doubtful case. It is probable that most cases of this nature are
regarded as simple detachments of the retina, and it is fortunate that
simple diathermic puncture as for a detached retina results in a cure in
the great majority (Goulden, 1935). Indeed, it is by no means unknown
for such cysts to rupture and disappear spontaneously (Deutschmann,
1914; Fuchs, 1937; Veil and Guillaumat, 1938).
Fig. 2424.-Cyst of RETINA at PERIPHERy.
The cyst (K) protrudes from the outer surface of the retina (r), which is detached
from the ora (o), and is torn at d. The vitreous (v) lies between the detached retina
and the ciliary body and iris (Gonin).
More commonly cysts, sometimes in considerable numbers and of
large size, are met with in detached retinae of long-standing, particularly in
cases following injury or inflammation which have lost all perception of
light (Collins, 1893; Falchi, 1895; Werner, 1907; Neame, 1920; and
others) (Fig. 2425). A plastic irido-cyclitis or a secondary glaucoma are
also common precedents (Veil and Guillaumat, 1938). They may occur
anywhere in the retina, and although they may be diagnosed from ophthal-
moscopic appearances (Gunn, 1890; and others), they are frequently found
accidentally on section. Quite frequently pseudo-cysts, indistinguishable
clinically from true cysts, are formed by the adhesions of folds of the retina
or of new-formed fibrous membranes on its inner surface (Parsons, 1905)
(Fig. 2426).
p. 2915.

DISEASES OF THE RETINA 28 ||
Fig. 2425.--RETINAL Cyst IN DETACHED RETINA.
Five years after an injury. Above and to the left is the degenerated ciliary
body; above and to the right a cataractous lens. The cyst, filled with coagulated
exudate, lies on the outer side of the detached retina (x 7) (Parsons).
Fig. 2426.-Pseudo-cyst IN DETACHED RETINA.
Phthisis bulbi after a perforating wound. The cysts are formed by adhesion
of folds of a detached retina. There is a large cyst below and to the right (x sy)
(Parsons).


28 12 TEXT-BOOK OF
OPHTHALMOLOGY
Butler. T. O. S., xlii, 304, 1922.
Collins. R. L. O. H. Rep., xiii, 41, 1893.
Coulter. T. O. S., x1, 172, 1920.
Cridland. T. O. S., x1, 172, 1920.
Derby. T. Am. O. S., xii, 827, 1909–11.
Deutschmann. Beit. z. Aug., ix, 591, 1914.
Falchi. A. f. O., xli (4), 187, 1895.
Fuchs, A. K. M. Aug., xcviii, 145, 1937.
Fuchs, E. A. f. O., lxxix, 42, 1911 ; xcvii,
57, 1918; ev, 333, 1921.
Goulden. T. O. S., lv., 407, 1935.
Gunn. T. O. S., x, 67, 1890.
Koller. T. Am. O. S., ix, 380, 1901.
Kornzweig. A. of O., xxiii, 491, 1940.
Kronfeld. A. of O., xiii, 779, 1935.
Lawford. R. L. O. H. Rep., xi, 208, 1887.
McCulloch. T. O. S., l, 619, 1930.
Neame. T. O. S., x1, 161, 1920.
Parsons. Path. of the Eye, London, ii, 623
1905.
T. O. S., x1, 171, 1920.
Ridley. Brit. J. O., xix, 101, 1935.
Schmelzer. K. M. Aug., xcvi, 19, 1936.
Schwartz. A. of O., xvi, 230, 1936.
de Schweinitz and Shumway. Am. J. Med.
Sc., cxxii, 736, 1901.
de Schweinitz and Wiener. J. Am. Med. As.,
lxxiii, 1187, 1919.
Stieren. T. Am. O. S., xxxi, l 16, 1933.
Thompson. T. O. S., x, 151, 1890.
Veil and Guillaumat. A. d’O., ii, 977, 1938.
Velhagen. K. M. Aug., l (2), 580, 1912.
Werner. O. Rev., xxvi, 248, 1907.
Weve. A. f. Aug., ciz, 49, 1935.
VII. TUMCURS OF THE RETINA
A. Primary Tumours
I, NEURO-EPIBLASTIC TUMC) URS
The earliest case on record of a primary neuro-epiblastic tumour of the
retina was published by Hayes (1767), while Wardrop (1809) gave the first
detailed clinical description. At this early period the disease was called by
the purely descriptive term of “fungus,” being referred to as fungus medullaris
in its earlier stages and fungus hoematodes when it attained such dimensions
as to become an ulcerating and bleeding mass. Robin (1854) published the
first accurate histological study, while the superficial resemblance to a round-
celled sarcoma led v. Graefe (1864) to regard it as a new-growth of the retina,
either carcinomatous or sarcomatous in type. Virchow (1864), in his
classical treatise on tumours, put forward the view that the growth arose
from pre-existing glial cells in the retina, and comparing it with tumours of
the brain, introduced the term glioma, describing the malignant transforma-
tion as glio-sarcomatous. Shortly thereafter two detailed and classical studies
by Hirschberg (1868–69) and Knapp (1868) served to define the natural
history and histology of these tumours and their metastases, and established
beyond question their non-sarcomatous nature.
Since that time the term “gliomata ‘’ has been almost universally
employed to designate all the primary epithelial tumours of the retina even
although subsequent work has made it quite clear that they are different in
type and habit from the gliomata of the brain. More recently several
attempts at a more scientific classification have been made, which we shall
notice presently ; but in so far as agreement has not by any means been
reached in the matter it will be more convenient if we first discuss the natural
history, clinical features and treatment of the group as a whole before we
individualize between the different histiogenic types.
DISEASES OF THE RETINA 2813
Incidence.
A very large literature makes it possible to assess the incidence of these
retinal tumours with considerable accuracy ; the two most important
sources are the series of 429 tumours studied by Wintersteiner (1897) and
the records of the Moorfields Hospital in London from 1871 to 1924 com-
prising 163 cases, the material from 1871 to 1890 being gathered by Lawford
and Collins (1890), from 1890 to 1896 by Marshall (1897), from 1896 to 1904
by Owen (1906), from 1904 to 1915 by Berrisford (1916), and from 1915 to
1924 by Davenport (1926). These neoplasms are relatively rare, forming
some 0.04% to 0.01% of all diseases of the eye (0.04%, Wintersteiner, 1897;
0.02%, Plaut, 1904; 0.01%, Berrisford, 1916) (1 in 2,000 patients,
Thompson, 1898; Sisson, 1906; 1 in 5,000, Krauss and Goldberg, 1905).
They are considerably rarer in negroes than in whites, but do occur in the
former (Cohen, 1930 ; Jaffé, 1934; and others).
Sea has little bearing : Wintersteiner (1897) in 429 cases gives 221 in
males and 208 in females. Reviewing 792 cases in the literature, Berrisford
(1916) found 398 in males and 361 in females.
A bilateral incidence occurs in some 20 to 30% of cases. In 405 of
Wintersteiner's (1897) cases, 308 were unilateral (143 right, 165 left) and 97
were bilateral (37 right first, 49 left first). Lawford and Collins (1890)
found 12 out of 35 cases bilateral ; Marshall (1897) 12 out of 32 ; Berrisford
(1916) 6 out of 39 ; and Davenport (1926) 5 out of 27. The growth in the
second eye is an independent focus, a conclusion proved by the survival of
patients free from any further symptoms who have had both eyes removed ;
such survival has been followed for long periods of time (5, 10 and 36 years,
Collins, 1895–1928). Again, the second eye may be involved and the nerve
found to be pathologically normal (Anderson, 1939). Both eyes may be
involved at birth (Helfreich, 1875; Snell, 1884; and others). Usually,
however, the neoplasm is noticed in the second eye some months after the
first, although its appearance may be delayed for some years (2}, Lawford ;
3 years, Collins, 1896). Maghy's (1919) case was quite exceptional wherein
the second eye commenced to fail l l years and was removed 18 years after
the first.
The age of incidence is limited almost exclusively to the early years of
life, a point first stressed by Hirschberg (1869). It is impossible to say
accurately when the neoplasm starts initially—possibly it is always
congenital, although the fact that involvement of the second eye has on
several occasions followed the first after a considerable interval during the
whole of which a most careful clinical watch has revealed no abnormality,
suggests that active growth must certainly be delayed in many cases until
after birth. It would seem, therefore, that only the developing retinal cells
are inclined to neoplastic activity, for when they reach the adult stage and
become highly differentiated they become so specialized that the more
2814 TEXT-BOOK OF OPHTHALMOLOGY
primitive reproductive potentiality is lost. This agrees with the failure of
the neural tissue of the retina to respond by proliferation in inflammatory
conditions."
In the literature two-thirds of the cases were noticed before the end of the 3rd
year (314 out of 429 cases, Wintersteiner, 1897 ; 102 out of 135 cases, Berrisford,
1916), the commonest age being 2 years : in Wintersteiner’s (1897) series 34 out of
429 were congenital, and more than one-third occurred within the 1st year of life.
Several reports, however, indicate its appearance in later years : in Wintersteiner’s
(1897) series 10 patients were over 9 and the oldest was 16 years; and in the
Moorfields series the oldest was 7 years (Davenport, 1926). In a case reported by
Maghy (1919) one eye was removed in the 2nd year, while the vision of the other
began to fail in the 13th and the eye was excised at the 20th. Makiuchi (1934) reported
a case which developed an amaurotic cat’s eye at 9 years, and a tumour at the limbus
at 11, the eye not being removed until the girl was aged 19. Wosnesenskaya (1933)
reported a case aged 20, the cells being epitheliomatous in type. Verhoeff’s (1929) case
of 48 years also probably arose from the ciliary epithelium. The 2 cases reported by
Gerard and Detroy (1926) and Gerard and Morel (1927) occurring at 66 and 35 years
respectively were possibly examples of massive gliosis “rather than of true neoplasms.
It is probable, therefore, that those neoplasms occurring after childhood are not the
usual retino-blastomata, and that those which are noticed after the first years of life
are exceptionally slowly growing rather than of late development.
The influence of heredity is important, for in a disease so very tragic in
its effects, eugenic prophylaxis is surely desirable, the need for which is
realized when it is remembered that Bell (1922) collected 36 pedigrees
wherein the condition occurred in two generations or in two or more siblings.
It is true that the majority of cases are sporadic, and in Davenport's (1926)
series of 27 cases collected from the Moorfields’ records from 1915 to 1924
there was no evidence of any hereditary or familial tendency, for in no single
instance was a second member of a family known to be affected. Examples
of familial incidence in the literature, however, are common. Two examples
may be cited : Wilson (1872) recorded a family of 8 children all of whom
were affected ; Newton (1902) saw a family of 16 of whom 10 were affected,
7 being bilateral, and of the 10 none lived beyond the age of 3 except 1 who
died of recurrences at 5. Benedict (1929) noticed the occurrence of a tumour
in similar situations in the homologous eyes of identical twins. Examples,
however, of direct inheritence are more rare (Owen, 1906; Hoffman, 1908;
Lukens, 1908; de Gouvea, 1910; Caspar, 1911 ; Dabney, 1915; Berrisford,
1916; Griffith, 1917–33; Traquair, 1919; Best, 1934; Hine, 1937).
Thus in Berrisford’s (1916) case the father had a unilateral tumour, a condition
inherited by his son who died of paralysis at 15 years. His daughter was healthy, but
of her 8 children 3 had bilateral tumours and 1 was unilaterally affected, all of these
dying at or before their 4th year. In this pedigree, therefore, a generation was skipped.
Griffith (1917) noted three examples—(a) a mother who was unilaterally affected had
6 children of whom 4 had bilateral tumours ; in each case both eyes were removed and
all died soon thereafter ; (b) a mother had her right eye removed for a tumour and of
1 p. 2622. * p. 2626.
DISEASES OF THE RETINA 2815
her 3 children 2 had a right neoplasm and I had bilateral neoplasms; all were well
13, 10 and 2 years after the operations. In Best’s (1934) case a unilateral sporadic
case in a family of 7 had 2 out of 3 children with bilateral disease. In a family reported
by Griffith (1933) and Hine (1937), a father who had unilateral disease had 5 children;
of these the first died prematurely and the other 4 were all affected, 3 bilaterally and
I unilaterally ; it is of great interest that while 2 of these died, and the unilaterally
affected eye was excised, 1 of the bilateral cases seemed to undergo a spontaneous cure.
Clinical Course
As in the case of malignant melanomata of the choroid, it is customary
to follow the scheme suggested by Knapp (1868) and consider the natural
history of these retinal tumours in four stages, that of (1) intra-ocular
growth, (2) secondary glaucoma, (3) extra-ocular extension, and
(4) metastases; but as we saw when considering the former tumours, it
FIG. 2427.-NEURO-EPITHELIoMA of RETINA.
The neoplasm arises in this case from the outer nuclear layer which fades into the
deep surface of the growth on the right (R. Scott).
is to be remembered that such a well-ordered evolution is the exception
rather than the rule. These retinal neoplasms usually arise from the
posterior part of the retina, even from the papilla, and especially from the
lower segment of the fundus. They probably arise from various layers,
not only in different cases but also in the same case, but the point of origin
is more usually the inner than the outer layers, particularly the inner nuclear
layer (Fig. 2427). Even in the early stages the tumours are usually multiple,
with one growth, presumably the primary growth, larger than the others, so
that the clinical appearance varies from that of an apparently single large
tumour or a nodule surrounded by a number of satellites, to a plentiful
sprinkling of the whole retina with miliary growths of various sizes. In all
probability most of these secondary masses are metastases seeded from
the parent neoplasm, but it is probable, especially in view of the common
bilaterality of the condition, that many of them represent independent foci.
The subsequent proliferation of the neoplasm varies in its relation to
T-0.-WOL. III. 3. A

2816 TEXT-BOOK OF OPHTHALMOLOGY
the retina, a relation which has a clinical but no pathological significance.
As a rule the main mass of the growth is directed outwards into the sub-
retinal space so that the retina becomes extensively detached (GLIoMA
Exophy TUM : Hirschberg, 1869) (Figs. 2428–29). Occasionally infiltration
occurs flatly along the plane of the retina (GLIoMA PLANUM) (Fig. 2427); and
sometimes irregular polypoid growths grow from the inner surface and
invade the vitreous cavity while the retina remains more or less in place
(GLIoMA ENDoPHYTUM) (Fig. 2430). In either event as the primary neoplasm
increases in size, it gradually overtakes and absorbs the neighbouring
secondary retinal deposits, and the cavity of the eye becomes filled with
new growth. The extension in the retina occurs partly by direct proliferation
Fig. 2428-NEuro-EpithºutoMA or RETINA.
A nodular growth projecting into the sub-retinal space with the external nuclear
layer stretched out on its surface (R. Scott).
along the newly-formed blood-vessels or the perivascular lymphatics, and
partly by the deposition of local metastases. The latter process is carried
out by clusters of cells which separate from the parent growth and float
freely in the sub-retinal space or the vitreous, where they proliferate and
whence they are sown over the retina. This phenomenon of the seeding of
satellites is almost confined to this type of tumour, for as we have seen,” it
is rare in malignant melanomata.
Intra-ocular extensions beyond the retina to the other ocular tissues
take place in the same two ways—by direct extension and by the seeding of
cellular clusters through the posterior and anterior chambers. Indeed, to
such an extent may this latter process occur that the aqueous may become
turbid, precipitates are deposited on the cornea and a hypopyon-like
sediment may appear in the anterior chamber (Walker, 1915; Behr, 1919;
* p. 2498.

DISEASES OF THE RETINA 2817
Fig. 2429.-" GLIoMA ’’ Exophytum (x 3) (Parsons).
v. d. Hoeve, 1925; and others) (Fig. 2431). The choroid is usually affected
first after the retina, especially along the vessels near the disc ;
frequently also new-formed vessels near the ora pass from the retina to the
anterior area of the choroid and carry a direct extension in this way. The
youngest deposits are found in the outer layers among the larger vessels
Fig. 2430.-‘ GLIoMA ’’ ENDoPHYTUM (x 24) (Parsons).


28.18 TEXT-BOOK OF OPHTHALMOLOGY
where the cells form aggregations between the tissue-planes, always as a
diffuse thickening and never as circumscribed tumours. The metastases
are multiple and subsequently become confluent so that the whole tissue
from the posterior pole forward may become greatly thickened. Bruch's
membrane is not perforated at first, and althoughin the later stages this always
occurs, the infiltration is by extension inwards from the choroid (Parsons,
1905). The region of the anterior chamber may be invaded directly from the
choroid by continuity, the zonule being destroyed, or by metastatic deposits.
(Fig. 2432). Nodules may appear on the iris which may simulate tuberculo-
mata, sympathetic ophthalmitis or other inflammatory conditions (Jung,
Fig. 2431–RETINoblastoma - Deposits on IRIs.
Note flocculent hypopyon.
1891; Behr, 1919; Meisner, 1921; Sijpkens, 1922; Pascheff, 1924;
Knapp, 1924; and others). The occurrence of such deposits is usually
late, and they generally lie under the pigmentary epithelium. At other
times the iris may be embedded in new growth without suffering infiltration,
or it may be infiltrated directly from the choroid through the ciliary body
which may be completely replaced by tumour tissue. The sclera is attacked
by growth along the perforating vessels, but the cornea is usually not
involved until a later stage when invasion occurs most frequently through
the pectinate ligament, but sometimes through ruptures in Descemet's
membrane caused presumably by stretching. Thereafter multiple
ruptures in Bowman's membrane are common through which columns of
cells spread under the epithelium (Snell, 1933; and others).

DISEASES OF THE RETINA 28.19
While these neoplastic changes are progressing a general degeneration
occurs throughout the ocular tissues. The retina, which is extensively
detached in the exophytum type but remains wholly or mostly in place
in the planum or endophytum type, becomes atrophied to become a
thin fibrous membrane in which perhaps some evidences of the nuclear
layers remain. The whole of its neural structures eventually disappear and
the sub-retinal space becomes filled with a coagulable transudate. The
pigment epithelium varies between proliferative and degenerative changes,
the cells becoming vacuolated and depositing colloid masses on Bruch's
membrane. Optic atrophy is evident at an early stage, the fibres degenera-
ting as a result of the complete retinal atrophy, while the clinical appearance
of atrophy is later accentuated by the formation of a deep glaucomatous cup,
which, however, is invariably filled up in time by new growth. The vitreous,
which may contain avascular metastatic
clumps of cells, may remain clear for a
long time, but eventually breaks up,
sometimes with the formation of mem-
branes; these, if they stretch behind the
lens, may cause difficulties in clinical
diagnosis. The choroid also atrophies
and the supra-choroidal space becomes
obliterated before the tissue itself is
permeated with growth. A similar
extreme atrophy affects the ciliary body Fig. 2432-RETIso-BLASToxa, Extes:
- - - - sions to ANGLE of ANTERIOR
and the iris which becomes attenuated, ºnes.
vascularized, and shows an ectropion of The false angle is covered with
its pigment layer. This atrophy of the lº
iris, lacunae in which are clinically (Parsons).
apparent on dia-scleral transillumina-
tion, may be a striking feature even in the early stages while the
tumour is yet confined to the posterior part of the globe (Handmann,
1922). The lens also suffers, being pushed forwards and deformed, and
sometimes becoming absorbed so that the capsule only remains. Occasionally
its epithelium shows proliferative and degenerative changes and underneath
it a flat anterior polar cataract may form. As a rarity hyaline or calcareous
degeneration appears, but involvement of the lens in the growth is rare. The
sclera becomes stretched and thinned, a staphyloma occurring preferentially
in the ciliary region, while in the cornea the increasing pressure causes
multiple tears and rifts in Bowman's and Descemet's membranes, and
ulceration and eventual perforation with a proliferation of the growth
through the wound may be expected in cases which have been allowed to
progress. In exceptional cases the whole globe may become atrophic and
shrink, most usually, perhaps, after it has been perforated (Knapp, 1868;
Hirschberg and Happe, 1870; Wadsworth, 1873; Snell, 1884; Lukowics,

2820 TEXT-BOOK OF OPHTHALMOLOGY
1884; Brailey, 1884-85; Lawford and Collins, 1890; Collins, 1892–96 :
Marshall, 1897; Lagrange, 1901; Parsons, 1905; and others).
While such extension is going on, the disease has advanced to the
glaucomalous stage, which in those young eyes results in enlargement of the
globe, giving the appearance of buphthalmos and stretching of its coats, a
process usually accompanied by much pain. Megalocornea becomes
evident, and eventually the selera gives way usually in the intercalary zone
or between the insertions of the recti and the equator, an event heralded
by the immediate relief of pain owing to the diminution of tension. Once
the loose and vascular orbital or sub-conjunctival tissue is reached, however,
the neoplastic cells change their character to become spindle-shaped and
Fig. 2433–RETINo-BLAstonia: FungATING STAGE.
growth becomes enormously rapid, so that the orbit is filled, proptosis occurs
and a fungating ulcerating growth bulges forward between the stretched lids
forming an unusually sickening spectacle (Fig. 2433).
Occasionally the growth may reach a very large size, as in the case of the neuro-
epithelioma reported by Patwardhan (1930) wherein the tumour protruding from the
lids measured 9 by 10 inches and covered an area from the nose to the ear and from the
forehead to the angle of the jaw. The tumour attained these enormous dimensions
within 6 months after the receipt of an injury to the eye.
Extra-ocular extension occurs along two routes. It almost invariably
appears first along the optic nerve, and in the vast majority of cases these
tumours kill because of a direct extension of growth along the nerve into the
brain. The tumour cells usually make their way directly through the
lamina cribrosa, aided perhaps by the atrophy of the nerve fibres, and
reaching the nerve beyond, become spindle-shaped and proliferate rapidly.
The spread is by direct extension, a slowly progressive permeation occurring
comparable to the method of spread of breast tumours. Occasionally the
spread is discontinuous, a portion of apparently free nerve being succeeded
by a heavily infiltrated segment (Meighan and Michaelson, 1938); but the
general tendency is for a direct spread to occur continuously to the chiasma

DISEASES OF THE RETINA 2821
and into the brain. At other times, but more rarely, the cells break through
the papilla into the inter-vaginal space whence they proliferate intra-
cranially into the sub-arachnoid space (Rand, 1934 ; Cairns and Russel
1931 ; and others). Here the tumour may grow as one continuous sheet
throughout the basal cisterns, but more usually is seeded out to form multiple
nodules in any part of the leptomeninges whence the brain itself may be
involved. It would seem, however, that the dura forms a barrier to this
mode of spread, for the bones of the skull, although they may be eroded by
pressure, are not invaded by secondaries in the meninges or the brain even
although there may be very extensive intra-dural metastases.
The second method of direct extension is through the sclera into the
orbit along the perforating vessels—the anterior and posterior ciliary
vessels and the vortex veins—usually posteriorly ; rarely the sclera is
perforated directly or its inner layers are burst by the high tension and
thus attacked. Thence dissemination occurs more frequently by direct
extension after the manner of a carcinoma than by the blood-stream as is
the habit of malignant melanomata. The secondary growths, therefore,
spreading along the orbital lymphatics, tend to be in the vicinity of the
eye, in the preauricular and neighbouring glands (cervical, mediastinal) and
the bones of the skull and the face. It would appear that orbital invasion,
which, however, may be microscopic, is an essential preliminary to involve-
ment of the bones of the skull ; and here again the dura forms an efficient
barrier, for infiltration does not occur readily through it into the brain,
although this tissue may be affected by pressure necrosis (Hu, 1930). It
is interesting that when extensions to the brain and the bones of the skull
occur, the two sets of secondaries are not associated with each other (Knapp,
1924).
Metastatic dissemination, however, carried by the blood-stream is by no
means unknown, and it would appear that if the orbit is once involved, the
increased vascularity encourages this method of spread (Verhoeff, 1929) so
that metastases are formed in such distant sites as in the humerus, the
sternum (Irvine, 1935), or abdominal organs as the liver and ovary (Jaffé,
1934). Wintersteiner (1897) gave the following table which is in sharp
contrast to the dissemination of melanomata of the choroid."
Brain and meninges, 43 ; skull and bones of face, 40; lymphatic glands, 36 :
parotid (probably through lymphatic glands), 9; skeletal bones, 9 ; liver, 7 ; spinal
cord and meninges, 5 ; kidneys, 2 ; Ovary, 2 ; lungs, 1 ; spleen, 1.
The difference in habit of the recurrences is well brought out by the figures of
Lawford and Collins (1890): in 22 cases of recurrences of glioma, 17 were orbital, while
out of 79 cases of malignant choroidal melanomata only 7 had orbital growths. Daven-
port (1926) found that in every fatal case in which the globe was not obviously involved
externally, the optic nerve was infiltrated, while in no case which recovered was the
nerve involved.
1 p. 2500.
2822 TEXT-BOOK OF OPHTHALMOLOGY
Such is the usual tragic story of the evolution of these tumours. The
first intra-ocular stage before acute symptoms develop usually lasts for 6
months to a year; the second stage of raised tension varies within wide
limits, but an average may be taken as 7 months ; and Once the tumour has
perforated the eye and its cells are relieved from the restraint of the intra-
ocular tension and reach the rich pabulum of the highly vascularized tissues
of the orbit, growth runs on apace and the end comes rapidly. Exceptions,
however, occur particularly in older cases, in which events proceed less
rapidly.
The early stages may be very prolonged as in the case reported by Maghy (1919)
wherein, after one eye had been excised on account of a neoplasm at the age of 2,
symptoms were noted in the other at 13, but the eye was not removed until the age of
20. Even after perforation has occurred, events may progress slowly : Makiuchi
(1934) recorded the case of a girl in whom an amaurotic cat's eye was noted at 9 years,
a tumour at the limbus appeared at 11 years and the eye was removed at 19, the 3rd
stage lasting 7 years.
Spontaneous Retrogression. More exceptional still is the occurrence of
spontaneous retrogression. In the earlier literature there are several
reports of a temporary retrogression which was followed by a recommence-
ment of growth. Lagrange (1901) collected 21 such cases, which are typified
by that recorded by Grolmann (1887).
This was of a child whose first eye was removed at the age of 2; at the age of 6 a.
grey reflex filled the pupil of the other, which, after treatment with mercury, diminished
in size so that the mass could only be seen through a dilated pupil. Five weeks later,
however, nodules appeared in the vitreous, a hypopyon formed in the anterior chamber,
the eye filled with growth, the cornea perforated and extra-ocular extension occurred
rapidly, the child dying a month later.
Complete retrogression is a rarer phenomenon, and is probably associated
with complete necrosis, assisted perhaps by a contraction of the reactive
fibrosis excited by the neoplasm in the surrounding tissues. A few cases
have occurred wherein spontaneous retrogression of one tumour was
followed by the resumption of growth at another site altogether (Winter-
steiner, 1897; Deutschmann, 1912; Axenfeld, 1918); and at least eleven
well authenticated cases have appeared in the literature wherein spontaneous
cure has been recorded and the after-history has been followed for many
years (Knieper, 1911; de Kleijn, 1911; Lindenfeld, 1913 ; Purtscher, 1915;
Meller, 1915; Siegrist, 1920; v. Hippel, 1928; Sallmann, 1939; Stallard,
1936; Hine (2 cases) 1937). In most of the cases the diagnosis was con-
firmed by microscopical examination of a similar tumour in the other eye ;
in some of them the regression resulted in phthisis (Knieper, de Kleijn,
Lindenfeld), but in others a useful eye remained (Purtscher, Meller, Siegrist,
v. Hippel, Sallmann, Hine). The ophthalmoscopic appearance of such eyes
presents a characteristic appearance (Plate LVII), a patch of chorio-retinal
atrophy with a raised mass in its centre varying in colour from salmon-pink
DISEASES OF THE RETINA 28.23
to dark green, and usually showing crumbly chalk-white masses. The
histological examination of phthisical eyes has corroborated the complete
degeneration of the tumour with the deposition of calcium and the formation
of bone.
Thus in Knieper's (1911) case one eye was removed and the diagnosis of glioma
established; shortly thereafter the other became filled with tumour so that the globe
became buphthalmic. On the parents' refusal of a second enucleation the child was
sent home to die; but—surprisingly—was discovered 11 years later alive and well in
an institute for the blind ; the eye which was then completely phthisical was
enucleated and examined pathologically. In Stallard's (1936) case retrogression
occurred during a severe attack of
scarlet fever : the patient had 2
children, both of whom had bilateral
tumours; 1 of them was put in a
fever hospital in the hope that it
too would develop scarlet fever and
be cured ; it died. Hine's (1937)
cases were interesting in that the
hereditary element extended to
spontaneous cure. The father had
1 eye excised for a “glioma at
the age of 2 years and a healed
lesion in the other ; and of his
children 2 died of bilateral tumours,
1 had an excision of 1 eye at the
age of 3 months, and 1 (aged 10
` º healed lesions Fig. 2434.-RETINo-BLAstovia.
... - -
* ºn-
The tumour appearing behind the lens.
The clinical picture pre-
sented by these neoplasms is usually typical. As a general rule the
growth remains unnoticed until it is sufficiently far advanced to produce
a yellow reflex at the pupil, when the child is brought to the surgeon
showing the well-known condition of amaurotic cat's eye, originally
thus characterized by Beer (1817) (Fig. 2434). A tumour in the early
stage is usually seen only when it is specially looked for, as when one
eye has been affected and the second is kept under constant review. In this
event, in the case of a glioma endophytum, the first appearance is of a small
round yellowish-white nodule in the retina usually situated in the posterior
part of the fundus, which soon becomes surrounded by small satellites (Fig.
2435). Later polypoid masses grow into the vitreous and haemorrhages
may appear on its surface. In glioma exophytum, on the other hand, the
condition initially resembles a simple detachment of the retina, an appearance
which, however, when occurring in young children, should always be viewed
with the greatest suspicion. As the growth progresses difficulties in the
clinical picture may arise from the presence of vitreous opacities or dense
membranes stretching behind the lens (Cohen, 1930; and others); but in
the later stages the appearance of metastatic bodies in the vitreous, the


2824 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2435.-EARLY RETINo-BLAstonia IN PERIPHERy (Foster Moore, Brit. J. O.).
occurrence of a pseudo-hypopyon of tumour-cells, the early appearance of
atrophy and the later development of nodules in the iris, and the eventual
filling of the entire eye with neoplastic tissue presents a picture which is only
too obvious. The eventual stage of extra-ocular extension when an ulcera-
Fig. 2436.-Rºtuno-BLAstoma.
Extensions of the main peripheral tumour around the dise.


DISEASES OF THE RETINA
5
2
82
ting and bleeding fungoid mass protrudes beyond the lids is simulated by
nothing else in young children (Fig. 2433).
The differential diagnosis involves the differentiation between a neo-
plastic and a non-neoplastic mass behind the lens, and, when iridic nodules,
keratic deposits, and perhaps a hypopyon with a turbid aqueous and an
obscured vitreous are present, the distinction from a nodular inflammatory
process, such as tubercle. Considering the vital importance of the issues
involved, such cases should always be most exhaustively examined under an
anaesthetic after full dilatation of the pupil. Several conditions occurring
in children may simulate a neoplasm, and these are usually descriptively
grouped together under the term PSEUDO-GLIOMA. The most important of
these are :=
1. Congenital abnormalities, especially those due to a persistence of the fibro-
vascular sheath of the lens. 1
2. Inflammatory conditions, such as organized inflammatory tissue in the vitreous,
tuberculomata, gummata, exudative retinitis (of Coats), metastatic ophthalmitis, and
so on, while in the earlier stages an exudative choroiditis may cause anxiety.
3. A simple detached retina.
4. Other neoplastic conditions, such as angiomatosis, etc.
5. Cysticercus.
Of these the most common is the inflammatory group, and every care
should be taken to eliminate evidences of a past quiet cyclitis such as anterior
synechiae, a retraction of the root of the iris, and inflammatory keratic
precipitates, or to enquire into a history of past inflammation of the
eye, or of such diseases as may cause an endophthalmitis in children, as the
specific fevers, meningitis, syphilis, and so on. The tension may afford
some useful information, a lowered tension being in favour of a pseudo-
glioma and a raised tension suggesting a neoplasm. Radiography may
provide a clue. In a large proportion of cases small islets of calcification are
present in the tumour, and these in 75% of cases can be observed radio-
graphically (Pfeiffer, 1936); it is rare for calcareous degeneration to be
present in the eye of a child in other circumstances. Moreover, if infiltration
has occurred along the optic nerve a radiogram of the optic foramen may
reveal changes—an important point in any decision as to the method of
treatment, indicating as it does extension into the skull : if the new growth
is confined within the dural sheath the foramen may be enlarged, even to
two or three times the normal diameter, but if the sheath has been perforated,
erosion of the bone appears (van der Hoeve, 1925). In certain cases, however,
it may be quite impossible to make sure of the diagnosis, and in these, in view
of the fact that such eyes are permanently blind in any event, and since the
life of the patient may be at stake, it is always wisest to treat the condition
as if it were a neoplasm. Especially is this so when both eyes are involved, in
1 Vol. II, p. 1381.
2826 TEXT-BOOK OF OPHTHALMOLOGY
which case the more advanced should be enucleated and subjected to
histological examination.
Prognosis. The instances of spontaneous cure of these tumours are so
rare that in the assessment of any case they must be disregarded. The
prognosis, therefore, unless the disease can be completely eliminated, must
be taken as uniformly bad and a fatal termination must be expected. With
treatment the prognosis depends upon the completeness with which the
entire growth can be removed or destroyed. When it kills it does so almost
invariably by eactension up the optic nerve to the brain, so that in general terms
it may be said that the fate of the patient depends on the absence of such
infiltration of the nerve as cannot be adequately dealt with. Analysing a
series of 119 enucleated eyes with no clinically apparent extra-ocular
extension, Reese (1931) found the optic nerve invaded in 63 (52%), and of
these in 51 (81%) the invasion extended higher than point of operative
section. In 43% of clinically favourable cases, therefore, an infiltrative
extension of new growth remained. It is interesting that Leber's (1916)
figures for recovery after enucleation of the eye are exactly the same—57%.
It would appear, therefore, that in favourable cases without apparent extra-
ocular extension the favourable prognosis after excision is 57%. Up to the
present time the results after radiational treatment have been less good ;
and the outlook after extra-ocular extension is almost hopeless. It is to be
remembered, however, that the literature contains several reports of survival
after the removal of both eyes, or even after evisceration of the orbit had
become necessary, and it can probably be taken that if the patient lives for
3 years after removal of the eye he may be considered safe.
Treatment
The treatment of these neoplasms devolves on two methods—excision
or radiation. There is no doubt that at the present stage of experience
with the different forms of radiation, eaccision of the eye is the method of
choice and should always be adopted in unilateral cases, or in bilateral cases
when both eyes are already blind. In bilateral cases where one eye still
retains useful vision the question of radiation arises, and although the
prognosis therewith is less secure, it is certainly a legitimate method to
adopt. Although many who have been blind from infancy have lived long,
useful and happy lives, it is a horrible thing, not less for the surgeon than
for the parents, to remove both eyes from an infant ; and the decision,
with all its bearings and all its uncertainties, is probably best shared
between both parties.
The important points with regard to enucleation are that it should be
done at the earliest possible moment, and that as large a portion of the nerve
should be removed as possible. Since the extreme importance of extension
up the nerve in determining the prognosis has been realized, the results of
operative treatment have been improved, a point well brought out in the
DISEASES OF THE RETINA 2827
literature—thus Hirschberg (1869) obtained a survival rate of 5%, Winter-
steiner (1897) of 17%, Adam (1911) of 57%, and Leber (1916) of 57%. From
the considerations reviewed in the previous paragraph it would seem that
this figure expresses the optimum results from simple enucleation. After
the operation the cut end of the nerve should be immediately subjected to
histological examination, and if it is healthy the case may be left ; it must
be remembered, however, that a discontinuous spread may occur past an
apparently healthy segment (Meighan and Michaelson, 1938). If it is
infiltrated the choice lies between exenteration of the orbit with excision of
the nerve at the optic foramen, a resection of the nerve at the chiasma
through an intra-cranial approach—a serious operation which should be
undertaken after the lapse of a few days to allow healing of the orbit to
prevent the escape of cerebro-spinal fluid (Dott and Meighan, 1933; Rand,
1934)—or radiational treatment. It must be remembered also that in cases
which appear to be clinically free, some orbital extension may have occurred ;
it may be argued, therefore, especially if the choroid has been involved in the
growth, that an exenteration of the orbit is advisable as a routine, a some-
what drastic procedure which might well be replaced by radiational
treatment.
With regard to radiational treatment the early results were poor. It was
first tried by Axenfeld (1914) in the second eye of a bilateral case, the
neoplasm in which recessed and later recurred ; while similar cases, ulti-
mately ending fatally and involving considerable damage to the eye, were
recorded by Uhthoff (1919), Kusama (1919), Knapp (1920), Weeks (1920),
Janeway (1920), Chase (1920) and Poyales and Pajares (1921). Marx (1922)
found 37 cases in the literature so treated and in these, good results were
obtained in only 5, and even so it was by no means certain that the cure
was permanent. Since that time, however, improvements in technique have
altered the question considerably. Cures in some cases extending to 5 or
10 years have been reported from X-rays by Verhoeff (1921), di Marzio
(1926), Fleischer (1929), Knapp and Luedin (1929), Martin and Reese (1936),
and Scheyhing (1937), by the surface application of radium (Schönberg,
1919, free for 19 years ; Greeves, 1934), by the insertion of radium needles
into the orbit (McDowell and Marks, 1932), or of radon seeds into the tumour
itself (Moore, Stallard and Milner, 1931 : Stallard, 1933; Moore, 1933).
That radiation can be effective has been shown by the histological demon-
stration of widespread necrosis in the tumour after its application (Stallard,
1933; Fewell and Fry, 1935), and the resultant clinical appearance is that
of a quiet fibrosed scar involving both the retina and choroid (Fig. 2437).
The technique is by no means standardized and has varied in each case ;
the papers of Martin and Reese (1936) on X-rays and of Stallard (1933) on
radium should be especially consulted. Some of these cases, it is true,
healed up so readily as to indicate an unusual radio-sensitivity, while in
others the most intensive treatment produced no effect (Fewell and
2828 TEXT-BOOK OF OPHTHALMOLOGY
Fry, 1935). That such variations in radio-sensitivity exist is seen in a case
reported by Griffith (1933) wherein a tumour appeared to be cured and
calcified by radiational treatment, but a recurrence in the same eye proved
Fig. 2437.-RETINo-blastoma TREATED with Rados SEEDs.
Appearance of scar 3 years after treatment (Foster Moore).
completely unresponsive and despite protracted treatment, required excision.
The average case, however, requires a great deal of treatment, so much,
indeed, that considerable damage may be done to the eye. A radiational
cataract is a not uncommon sequel, but cases have survived its discission
happily (Knapp and Luedin, 1929; Scheyhing, 1937). In the present stage

T) ISEASES
2829
OF THE RETINA
of our knowledge, however, it cannot be pretended that this is the treatment
of election ; it is only applicable to cases in which excision seems unjustified.
Adam. Z. f. Aug., xxv, 330, 1911.
Anderson. Ophthalmologica (Z. f.
xcviii, 193, 1939.
Axenfeld. K. M. Aug., lii, 426, 1914.
B. O. G. Heidel., xli., 312, 1918.
Beer. Lehr. v. d. Augenkrankheiten, ii, 495,
1817.
Behr. K. M. Aug., lxiii, 277, 1919.
Bell. Treasury of Human Inheritance, ii, 1922.
Benedict. A. of O., ii, 545, 1929.
Berrisford. R. L. O. H. Rep., xx, 296, 1916.
Best. B. O. G. Heidel., 1, 9, 28, 1934.
Brailey. T. O. S., iv, 54, 1884; v, 61, 1885.
Cairns and Russel. Brain, liv, 377, 1931.
A ug.),
Caspar. Cb. pr. Aug., xxxv, 161, 1911.
Cattaneo. A. Ital. di Amat., xx, 371, 1923.
Chase. Am. J. O., iii, 806, 1920.
Cohen. A. of O., iv, 368, 1930.
Collins. R. L. O. H. Rep., xiii, 361, 1892;
xiv, 52, 1895.
T. O. S., xvi, 142, 1896 ; xxxvii, 246, 1917;
xlviii, 107, 1928.
Dabney. Am. J. Surg., xxix., 185, 1915.
Davenport. Brit. J. O., x, 474, 1926.
Deutschmann. Z. f. Aug., xxvii, 225, 1912.
Dott and Meighan. Am. J. O., xvi, 59, 1933.
Fewell and Fry. A. of O., xiv, 190, 1935.
Fleischer. K. M. Aug., lxxxiii, 332, 1929.
Flexner. Bull. Johns Hopkins H., ii, 115,
1891.
Gerard and Detroy. La Clin. Opht., xxx, 328,
1926.
Gerard and Morel. La Clim. Opht., xxxi, 374,
1927.
de Gouvea. Am... d’Oc., czliii, 32, 1910.
v. Graefe. A. f. O., x (1), 216, 1864; xii (2),
237, 1866; xiv. (2), 103, 1868.
Greeves. T. O. S., liv, 420, 1934.
Griffith. Brit. J. O., i, 529, 1917.
T. O. S., xxxvii, 242, 1917; liii, 238, 1933.
Grolmann. A. f. O., xxxiii (2), 47, 1887.
Handmann. K. M. Aug., lxix, 35, 1922.
Hayes. Medical Observations and Inquiries,
iii, 1767.
Helfreich. A. f. O., xxi (2), 236, 1875.
Hine. T. O. S., lvii, 173, 1937.
v. Hippel. K. M. Aug., lxxxi, 30, 1928.
Hirschberg. A. f. O., xiv. (2), 30, 1868;
xvi (1), 297, 1870.
Die Markschwamm d.
1869.
Hirschberg and Happe. A. f. O., xvi (1), 297,
1870.
v. d. Hoeve. T. O. S., xlv, 256, 1925.
Hoffmann. B. O. G. Heidel.., xxv, 15, 1908.
Hu. Am. J. Path., vi, 27, 1930.
Irvine. A. of O., xiii, 858, 1935.
Jaffé. A. of O., xii, 318, 1934.
Janeway. A. of O., xlix, 156, 1920.
Jung. A. f. O., xxxvii (4), 125, 1891.
de Kleijn. A. f. O., lxxx, 371, 1911.
Netzhaut, Berlin,
Knapp, A. A. of O., xlix, 575, 1920; liii,
470, 1924.
Knapp, H. Die intraokularen Geschwälste,
Rarlsruhe, 1868.
K. M. Aug., vi, 428, 1868.
Knapp, P. and Luedin. K. M. Aug., lxxxiii,
279, 1929.
Knieper. A. f. O., lxxviii, 310, 1911.
Krauss and Goldberg. Am. of O., xiv, 628,
1905.
Kusama.
Lagrange.
Lawford and Collins.
12, 1890.
Leber. G.-S. Hb., II, (A2), 1947, 1916.
Lindenfeld. A. f. O., lxxxvi, 141, 1913.
Lukens. J. Oph. Oto. Laryn., ii, l, 1908.
Lukowics. Diss., Halle, 1884.
Maghy. Brit. J. O., iii, 237, 1919.
Makiuchi. Acta S. O. Jap., xxxviii, 3, 1934.
Marshall. R. L. O. H. Rep., xiv, 456, 1897.
Martin and Reese. A. of O., xvi, 733, 1936.
Am. J. O., ii, 636, 1919.
Tumeurs de l’Oeil, Paris, i, 1901.
R. L. O. H. Rep., xiii,
Marx. Ned. Tij. v. Gen., lxvi (2), 683, 1922.
di Marzio. Boll. d’Oc., v, 567, 1926.
McDowell and Marks. Brit. J. O., xvi, 686,
1932.
Meighan and Michaelson. T. O. S., lviii, 208,
1938.
Meisner. K. M. Aug., lxvii, 388, 1921.
Meller. Am. J. O., xxxii, 193, 1915.
Moore. T. O. S., liii, 215, 1933.
Moore and Scott. P. R. S. Med., xxii, 951,
1929.
Moore, Stallard and Milner. Brit. J. O., xv,
673, 1931.
Newton. Lancet, ii, 1411, 1902.
Owen. R. L. O. H. Rep., xvi, 323, 1906.
Parsons. Path. of the Eye, London, ii, 626,
1905.
R. L. O. H. Rep., xvi, 141, 1906.
Pascheff. K. M. Aug., lxxiii, 126, 1924.
Patwardhan. Brit. J. O., xvi, 297, 1930.
Pfeiffer. A. of O., xv, 811, 1936.
Plaut. Diss., Giessen, 1904.
Poyales and Pajares. A. d’O., xxxviii, 122,
1921.
Purtscher. Cb. pr. Aug., xxxix, 193, 1915.
Rand. A. of O., xi, 982, 1934.
Reese. A. of O., v, 269, 1931.
Robin. Gaz. de Méd., Paris, ix, 419, 1854.
Sallmann. Fuchs' T.B. of Ophthal., XVI,
1939.
Scheyhing. K. M. Aug., xcviii, 756, 1937.
Schönberg. A. of O., xlviii, 485, 1919; Ivi,
221, 1927. -
Siegrist. K. M. Aug., lxv, 387, 1920.
Sijpkens. K. M. Aug., lxix., 27, 1922.
Sisson. O. Rec., xv, 156, 1906.
Snell, H. C. T. O. S., liii, 609, 1933; lvi, 269,
1936.
Snell, S. T. O. S., iv, 49, 1884.
2830
TEXT-BOOK OF OPHTHALMOLOGY
Stallard. T. O. S., liii, 224, 1933.
Brit. J. O., Monogr. Supplement vi, 1933.
P. R. S. Med., xxix., 963, 1936.
Susman. Brit. J. O., xxii, 722, 1938.
Taylor and Fleming. Brit. J. O., i, 92, 1917.
Thompson. J. Am. Med. As., xxxi, 628, 1898.
Traguair. Brit. J. O., iii, 21, 1919.
Uhthoff. K. M. Aug., lxii, 6, 1919.
Verhoeff. A. of O., i, 450, 1921 ; li, 120, 1922;
ii, 643, 1929.
Virchow. Die Krank. Geschwislte, ii (1), 151,
1864.
Wadsworth. T. Am. O. S., ix, l 1, 1873.
Walker. T. O. S., xxxv, 363, 1915.
Wardrop. Observations on Fungus Haema-
todes, Edin., 1809.
Weeks. A. of O., xlix, 241, 1920.
Wilson. Brit. Med. J., i, 381, 1872.
Wintersteiner. Das Newroepithelioma Retinae,
Wien, 1897.
Wosnesenskaya. Sov. vest. O., ii, 202, 1933.
The Nature of Neuro-epiblastic Tumours
The nature of the primary neuro-epiblastic tumours has long been a
matter of dispute. The appearance of the cells led the earliest writers to
assume that they represented a sarcomatous hyperplasia of the retina
(v. Graefe, 1864), a view disposed of by Virchow (1864), who, as we have seen,
assumed that they arose from the glial cells of the retina and were comparable
to the gliomata of the brain. Subsequent work, however, demonstrated the
vast difference in type and evolution between the two classes of tumour.
From time to time protests were therefore made against this unsatisfactory
and misleading comparison and against Virchow's term, glioma.
The first of these was by Flexner (1891) who, on discovery of the
presence of rosettes of epithelial cells, suggested the term neuro-epithelioma,
which, although adopted by Wintersteiner (1897) in his exhaustive mono-
graph, has never become popular. He considered the rosettes the “germ ‘’
of the growth, representing “rests '' of the outer layer of neuro-epithelium
(rods and cones) which had become “implanted ” in the sense of Cohnheim.”
Against this the arguments brought forward were that if the essential
neoplastic cells were rod-and-cone elements, it was curious that the earliest
proliferation was usually from the inner and not the outer nuclear layer, and
still more curious that rosettes should be absent from most tumours even
in their earlier stages.
The embryonic form of these tumours has long been recognized ;
Collins (1896) at an early date drew attention to their resemblance to the
undifferentiated retina of the 3rd or 4th month foetus. claiming that if this
were shown to a pathologist he would pronounce it a glioma ; just as a
sarcoma represents the foetal condition of the tissue from which it primarily
grows, so this neoplasm represents the foetal condition of the retina. Collins'
(1896) original illustrations stressing this point are reproduced in Figs. 2442
and 2443. Shortly thereafter Ginsberg (1899–1901) elaborated this idea ;
he considered the tumour cells embryonic elements which had not yet
developed but could potentially form spongioblasts (glial cells) and neuro-
blasts (nerve cells), the rosettes developing from undifferentiated cells in
the course of proliferation in a manner somewhat akin to the cylindrical
cells of the pars ciliaris retinae. At a considerably later date Fischer (1918),
* Vol. II, p. 1446.
PLATE LVII
RETINAL Tumours
Fig. 2438.-EARLY RETINo-BLAstovía AT Fig. 2439.-Right eye of father.
MACULA. -
Fig. 2440.-Right eye of son. Fig. 2441.-Left eye of son (Hine, T. O. S.).
Figs. 2438–41–Spontax rous Curr of A FAMILIAL RETINo-BLAstovía.
To face p. 2830.


DISEASES OF THE RETINA 2831
in view of the embryonic character of the growth, proposed the term neuro-
blastoma ; and Verhoeff (1922) retino-blastoma, which latter was adopted as
a general term by the American Ophthalmological Society in 1926. Although
perhaps not strictly accurate in depicting all the types which may be met with,
no better general term has been suggested. One thing is certain, that the
old term “glioma should be dropped and used only for those exceptionally
rare retinal neoplasms which are true astrocytomata, derived from the
supporting structure of the retina and not its nervous elements. Glial
tissue does occur in retino-blastomata, but it is not neoplastic in nature,
only forming a framework comparable in importance to the nutrient
***w net
Fig. 2442. –RETINo-BLASTOMA. Fig. 2443.−FoETAL RETINAL CELLs.
Neoplastic cells (Treacher Collins, Between months 3 and 4 (Treacher
Researches). Collins, Researches).
vascular supply of any tumour or the glial proliferation in the mesodermal
angiomata (Urra, 1923; Grinker, 1931–32).
While Verhoeff's term has been used somewhat sporadically in the
literature, a new element was introduced into the subject by the researches
of Bailey and Cushing (1926) who brought order out of chaos in the
classification of tumours of the brain. Since the retina is developed as a
budding-out of the brain and is formed from the same types of cells, it seemed
reasonable to assume that neoplasms occurring in the former would be
analogous to those occurring in the latter. Several attempts at correlation
have been made, and although a universally accepted system has by no
means been evolved as yet, there is no doubt that the analogy is correct in
principle.
Bailey and Cushing’s (1926) classification of brain tumours was a histogenic one
based on the development of the glial cells. It will be remembered that in the embryo
the medullary epithelium lining the neural tube first differentiates into three types of
cells—(1) the neuroblast, which ultimately develops into the neuron; (2) the primitive
spongioblast, which develops into the spongework of the brain, the majority becoming
glial cells (astrocytes) and a minority, ependymal cells; and (3) the medullo-blast,
T.O.-WOL. III. 3 B

2832 TEXT-BOOK OF OPHTHALMOLOGY
primitive and undifferentiated cells the subsequent fate of which is still uncertain,
which are thought to develop either into glial or neuronic cells. These various
cells are distinguishable by gold and silver impregnation methods and a tumour is
classified according to the predominant cell which it contains. Neuro-blastomata are
very rare. The spongio-blastoma is the commonest type of brain tumour and is highly
malignant ; almost as common is the more fully differentiated and comparatively
benign astrocytoma. The ependymoma is rare, arising from the ependyma of the
ventricles or the central canal, and containing rosettes. Medullo-blastomata are rare,
rapidly-growing and malignant, containing round cells arranged around blood vessels
to form pseudo-rosettes.
Since the primitive medullary epithelium of the neural tube forms not only the
primitive cerebral vesicle, but also the optic vesicle, the adult nervous elements of the
retina derive from the same cells, but attempts at classification with regard to the
retina have not been so successful as in the case of the brain. Grinker (1932) formulated
three types:—
1. Medullo-epitheliomata arising from the primitive cells of the medullary epi-
thelium, which are rare ; they contain retinoblastic and spongioblastic cells and show
neuro-epithelial rosettes.
2. Retino-blastomata, which are common, forming the great majority of retinal
“gliomata,” and are composed of retinoblastic cells. These cells are bipotential,
developing either into neuroblasts or spongioblasts (glial cells). Neuroblasts, however,
are not found in these tumours, the cells of which are spongioblastic in type.
3. Newro-epitheliomata, composed of primitive spongioblasts which may be
arranged in rosettes, and usually arise from the external nuclear layer, or in the pure
form from the ciliary processes.
Dejean (1936) also suggested three categories : (1) neuro-epitheliomata, derived
from the cells of the primitive epithelium of the ependymal cavity ; (2) newro-
Spongiomata, derived from the neuro-spongiomatous cells surrounding the canal ;
and (3) retino-cytomata, wherein the cells show signs of differentiation into retinal
Ilel II’OI)S.
Favaloro (1937) expressed doubts as to whether a true neuro-blastoma ever exists.
He differentiated six types of cell : (1) anaplastic glioblast (common), (2) astrocytic
spongioblast (less common), (3) ependymal spongioblast (very common and forming
rosettes), (4) syncytial glioblast (common), (5) fibrillar glioblast (rare), and (6) the
differentiated astrocyte (rare). As the usual tumour is always a mixture of types he
suggested a composite terminology depending on the histogenesis—as an ependymo-
spongio-fibrillo-glio-blastoma.
Susman (1938) put forward three main types—
(1) Neuro-epitheliomata, corresponding to the medullo-epitheliomata and neuro-
epitheliomata of Grinker. They are thus made up of columnar epithelium, either
primitive medullary or retinal epithelium and may show evidence of differentiation
into retinoblasts or ependymal cells. -
(2) Spongio-blastomata. These may be either (a) primitive or apolar (retino-
blastomata), with closely packed polyhedral cells ; or (b) polar and made up of large
fusiform cells resembling those of sarcomata. The first type, since the retinoblast is
identical with the medulloblast, corresponds with the medullo-blastomata of the
brain. -
(3) Neuro-blastomata or Neuro-cytomata, which are more fully ' , , -loped and
differentiated and tend to simulate adult retinal structures. -
Apart, therefore, from the comparatively differentiated neuro-cytomata,
all these epiblastic tumours are composed of primitive cells. Astrocytomata,
DISEASES OF THE RETINA 2833
i.e., true gliomata formed of developed glial cells, do occur, but they are
extremely rare (vide infra). Elschnig (1914) and Pieck (1936) reported the
occurrence of a neuro-cytoma (or neurinoma), and tumours have been
described as arising from the pigmented epithelium (Griffith, 1894; Deutsch-
mann, 1912), but these were probably degenerative or inflammatory
proliferations (Parsons, 1905; Reis, 1915).
In many ways it may seem that systems of classification on such a
basis are of theoretical rather than practical value and are essentially
artificial. In this argument there is indeed much truth, but it is probable
that the classification which will be ultimately evolved and adopted in the
future, when the genesis of these neoplasms is more fully understood, will
be on some such developmental histogenic basis relating the tumour cells
to stages in the natural history of the normal retinal elements, and will bear
a close analogy to that accepted in the central nervous system. It must be
remembered, however, that all these neoplasms have a highly mixed content
of cells ; a majority of elements must be taken to give it its character.
Moreover, it cannot be said that our knowledge is sufficiently far advanced
to allow a definitive classification, and the scheme adopted in these pages is
admittedly provisional and temporary.
It is also of interest to note the analogy between these retinal tumours and the
neuro-blastoma occurring in the adrenals or other parts of the sympathetic system ;
these neoplasms occur congenitally or in young patients, are rapidly growing and
spread by metastases, and are characterized by a very cellular structure showing
rosettes.
We shall discuss those primary neuro-epiblastic tumours under five
headings : (a) retino-blastomata, (b) neuro-epitheliomata, (c) medullo-
epitheliomata, (d) neurocytomata, and (e) astrocytomata or true gliomata.
Of these the first two are common and the last three very rare.
(a) RETINO-BLASTOMA
Retino-blastomata (RETINOMA of Cattaneo, 1923 ; RETINO-CYTOMA of
Mawas ; SPONGIO-BLASTOMA of Susman, 1938), which comprise the vast
majority of retinal tumours, are composed of primitive cells—retinoblasts—
which are analogous to the medulloblasts of the central nervous system.
These latter, arising from the medullary epithelium, are bipotential in that
they may develop along the neuroblastic series to form neurons or along the
spongioblastic series to form glia ; the spongioblastic type of cell is character-
istic of the retinal neoplasm. These tumours therefore correspond to the
medullo 'ºstomata of the brain—relatively rare but rapidly growing, soft
malignanu tumours occurring usually in association with the rool ºf the
fourth ventricle in young persons, which “infect ’’ the meninges with
metastases. In general terms the retino-blastomata are malignant tumours
arising in early youth preferentially from the posterior part of the retina, com-
3 B 2
2834 TEXT-BOOK OF OPHTHALMOLOGY
posed of small closely packed round or polygonal cells with large darkly staining
nuclei and scanty cytoplasm. A radial arrangement around blood-vessels to
form pseudo-rosettes is common, degenerative changes are frequent, and, as
would be expected owing to the embryonic nature of the cells, malignancy is high,
usually by local extension to the brain and orbit, and sometimes by distant
metastases. It is interesting that these are the only tumours of glial origin
known to metastasize to distant parts. Since they form the majority of
retinal “gliomata” the usual descriptions in the literature apply essentially
to this type of neoplasm.
Fig. 2444.—RETINo-BLAstoma.
The tumour is composed of small round darkly staining cells closely packed
together in which there are many areas of degeneration (Haematoxylin and eosin)
(x 100) (Grinker, A. of 0.).
In their general pathological features the retino-blastomata have a
characteristic appearance. They are grey, almost milky-white tumours,
soft like brain-matter, and usually interspersed with gelatinous areas of
degeneration, gritty patches of calcification, and pigmented haemorrhages.
Section shows a picture of closely packed small cells interspersed with poorly
staining areas of necrosis or fatty degeneration, the two being sometimes
sharply demarcated and sometimes separated by a transition zone (Figs.
2444-45). The deeply staining clusters are composed of cylinders of cells
arranged 10 to 20 deep along blood-vessels, an appearance usually described
as cellular mantles or pseudo-rosettes (Fig. 2446). This peculiar structure,
which was first described by Iwanoff (1869), is probably due to the dependence
of the cells for nourishment upon the blood-vessels which draw their cellular
mantle after them as they grow, while the necrotic areas are due to

DISEASES OF THE RETINA 2835
Fig. 2445, -RETINo-BLAstovia.
Typical structure; composed of branching processes of cells, each process with
a central vascular cone around which the well-nourished cells form a deeply staining
mantle. These are separated by necrotic areas (Scott).
FIG. 2446.-RETINo-BLASToxia.
A blood-vessel surround by a mantle of cells outside of which the cells are
necrotic (pseudo-rosette) (Parsons).


2836 TEXT-BOOK OF OPHTHALMOLOGY
insufficient nourishment to sustain the very high rate of metabolic activity.
The cells themselves are small, round or polygonal, and are frequently
provided with a tail-like process at one end giving them a carrot-like shape ;
there is thus a strong resemblance to embryonic retinal cells (Figs. 2442 and
2443). The nuclei are large and darkly staining with a definite chromatin
net-work, and the cytoplasm so scanty as to give the suggestion of free
nuclei. Mitotic figures are usually abundant ; and the inter-cellular
substance is sparse, while the vessels are of the nature of wide endothelial
tubes composed of a single layer of cells. Studies with silver impregnation
were made by Greeff (1896), Hertel (1897) and Urra (1923), the latter of whom
differentiated various stages in the histogenesis of retinoblastic cells ;
spongioblasts as well as adult astroblasts and astrocytes may be present, but
it is probable that the ganglion cells sometimes encountered are normal
retinal elements infiltrated by the tumour.
Degenerative changes are common and widespread, beginning in the
cells farthest removed from the vessels and sometimes attacking large areas.
The nuclei become pyknotic and shrink, their shape becomes irregular and
they fragment, eventually losing their ability to stain. Such areas of necrosis
may become infiltrated by large phagocytic cells rich in lipoids, mucin may
be demonstrated, fatty degeneration is common, and calcification surprisingly
frequent, indeed, almost invariable except in the earliest stages. These
degenerative changes occur early not only in the parent tumour, but also
in its metastases, intra-bulbar and extra-ocular.
In the metastatic deposits outside the retina, either intra-ocular in the
uvea and in the optic nerve, or in extra-ocular tissues, the cells change their
character : they become larger, angular or fusiform and with larger processes.
They thus resemble sarcomatous cells so closely that the older writers
considered that there was an actual transformation of a glioma into a sarcoma
in its metastatic growths (Virchow, 1864; Knapp, 1868 ; Pinto, 1886 ; and
others). These metastases also do not show the tubular structure charac-
teristic of the retinal growth which is determined by its blood-vessels.
Presumably the size of the cells and the architecture of the tumour are
altered because of the difference in the blood supply, derived in these
sites (choroid, optic nerve, and the extra-ocular tissues) not from relatively
few end-arteries as in the retina, but from a rich anastomosing supply
derived from the invaded tissue (Collins, 1928).
(b) NEURO-EPITHELIOMA
Neuro-epitheliomata form the second most common group of neuro-
epiblastic tumours of the retina : Wintersteiner (1897) found 11 out of
32 cases of “glioma,” but this proportion is unusually large. They corre-
spond to the neuro-epitheliomata of the brain—exceedingly rare tumours of
which Bucy and Muncie (1929) were only able to find six authentic instances.
DISEASES OF THE RETINA 2837
Fig. 2447.-NEURO-EPITHELIOMA OF RETINA.
The same tumour as Fig. 2427 showing the mass largely made up of rosette
formations (Scott).
They are composed of primitive spongioblasts, which appear as large
columnar cells arranged characteristically in true rosettes, a feature, it should
be remembered, shared also by the medullo-epitheliomata.
Fig. 2448.-NEURO-EPITHELIoMA of RETINA,
The margin of the growth of Fig. 2447 showing the development of the tumour
from the outer nuclear layer.
The neuro-epitheliomata arise in the external nuclear layer of the
retina (Figs. 2447–48) and are made up essentially of groups of large columnar
cells with round, sharply defined nuclei arranged in a radial manner like the spokes


Fig. 2449-NEURO-Epithelioma of RETINA.
Showing rosettes (Parsons).
Fig. 2450–Neuro-Epithelioma.
The intimate cellular structure, be, blood-vessel, o, oligodendroglia.
º, primitive spongioblast r, rosette. ab astroblast ac, astrocyte (Kanzler
impregnation) (Grinker, A. of 0.).


DISEASES OF THE RETINA 2839
of a wheel in a palisade form around a central cavity to form a RosBTTE. The
central cavity contains no blood-vessel, but at most colloid or hyaline
material (Fig. 2449). The broad end of the cell adjoins the cavity around
which the cell membranes form an internal limiting membrane ; the nuclei
lie at the end farthest from the central cavity, and from each end of the
cell runs a a cilium-like process, the inner one projecting through the
membrane into the cavity. Such rosettes are not always circular but are
sometimes horseshoe shaped, spiral or convoluted (Zeiss, 1926); they are
never very long and they are always open at one end. Sometimes the
neoplasm appears to be made up almost completely of rosettes, and at
other times they are scanty and irregularly distributed. Between them lie
masses of small round cells with large nuclei and scanty cytoplasm, presum-
ably retinoblasts, amongst which are blood-vessels and areas of necrosis ;
while at intervals are seen spongioblasts and more highly differentiated
astroblasts and astrocytes (Fig. 2450).
Rosettes were first discovered by Flexner (1891) who remarked on their
resemblance to the neuro-epithelium, the basal membrane representing the external
limiting membrane, the protoplasmic protrusions into the lumen the rods, and cones,
and the nuclei the outer granules. This comparison was adopted by Wintersteiner
(1897) who considered them, thus derived, the “germs ” of gliomata in general. There
is no reason to doubt the comparison, for the rods and cones are developed not from
neuroblastic cells, but as a sensory epithelium from a portion of the primitive retinal
epithelium corresponding to the primitive spongioblasts of the brain. It is interesting
that similar structures are sometimes seen in congenitally deformed eyes, or in
developing eyes in which a disturbance of the normal relationship between the
supporting elements and the nervous tissues of the retina has been brought about by
such agencies as radiation or trephining the eyes of newly born animals.” These
exuberant growths of the neuro-epithelium met with congenitally, however, have as
their origin a folding of the external limiting membrane, their lumen being probably
originally in continuity with that of the primary optic vesicle. It is only in tumour-
formation that rosettes are found around lumina not associated with the external
limiting membrane.
(c) MEDULLO-EPITHELIOMA
Medullo-epitheliomata arise from the medullary epithelium of the
primitive neural tube of the brain and the primitive retinal epithelium. In
the brain they are very rare indeed : Bailey and Cushing (1926) could find
only two examples, both of which arose from the area near the third ventricle
where the primitive roof and floor plate of the neural tube persist in adult
life. In the retina such tumours are similarly rare and they also arise from
that region wherein the medullary epithelium persists in almost undifferen-
tiated form—the ciliary epithelium. These tumours have already been
described in the section on tumours of the uveal tract,” when it was noted
that they were divided by Fuchs (1908) into two classes, the dyktyomata,
1 Vol. II, p. 1336. * p. 2449.
2840 TEXT-BOOK OF OPHTHALMOLOGY
resembling embryonic retina and sometimes of extreme complexity, and the
malignant epitheliomata retaining the more primitive structure of the
ciliary epithelium. They need not detain us further here. It will also be
remembered that the senile hyperplasias of the ciliary body have a some-
what similar formation.
(d) NEURocytoma
A few tumours have occurred which are characterised by the high
state of differentiation of their cells as neuroblasts or neurocytes, and by
-- -
-- -
º - - -
º º
º º º º --- º
- --- º
- º º -
- º nº sº - º
º - º º --- º-ºº:
- º º º
- - º * *
-
- -
tº
-ºº -
º
---
-
Fig. 2451.-NEurocytomia of RETINA.
The neoplastic process has formed recognizable retinal tissue (x 80) (Susman,
Brit. J. O.).
their attempts to form recognizable retinal structures (Dejean, 1936;
Susman, 1938) (Fig. 2451). As would be expected, they are not particularly
malignant.
(e) AstrocytomATA (TRUE GLIoMATA)
Astrocytomata, arising from true glial tissue, rank amongst the com-
monest of brain tumours (Bailey and Cushing, 1926), and since astrocytes
are plentiful in the inner layers of the retina similar to the astrocytic glial
elements of the central nervous system, it is reasonable to assume that a
similar glial neoplasm would occur in the eye. Apparently, however, they
p. 2447.














DISEASES OF THE RETINA 284.1
Fig. 2452.-Astrocytoma of RETINA (McLean, A. of 0.).
are extraordinarily rare (Dejean and Harant, 1932; Dejean, 1934; McLean,
1937). Like the corresponding tumour of the brain which causes damage by
pressure only, they appear in the second and third decades of life or later,
F---- º
-º-º-º-º-º-º:
º - - -
Fig. 2453.-Ast Rocytoma of RETINA.
The border of the tumour of Fig. 2452 showing the displacement of the normal
retinal elements by tumour cells (McLean, A. of O.).
and destroy vision only by the fact of their growth, and like them they are not
particularly locally invasive or malignant, neither recurring nor metastasizing
after removal.


2842 TEXT-BOOK OF OPHTHALMIOLOGY
McLean's (1937) case may be taken as typical, which occurred in a
woman of 23. It appeared as a sharply defined, smoothly outlined, highly
vascularized mass in the macular region, without satellites and without
causing disturbance to the rest of the ocular tissue (Fig. 2456, Plate
LVIII). Histological examination showed that the tumour was confined
to the retina, supplanting and displacing its elements; over it the limiting
membrane was intact, the rods and cones were preserved, and Bruch's
Sºº-
| -
- - -
- º
Fig. 24.54.—Astrocytoma or RETINA.
Cellular structure. Spindle cells with ovoid nuclei and long fibrillary processes.
(McLean).
membrane was undisturbed (Figs. 2452 and 2453). The tumour, which arose
from the inner retinal layers, was composed of long spindle cells with ovoid
nuclei and long fibrillar processes which differential staining showed to be
neuroglial and not connective tissue (Fig. 2454). Mitoses were few and
metaplasia not excessive.
The only other glial masses found in the retina are the result of long-standing
inflammatory or degenerative processes, in which event a tumour-like massive gliosis
may result as a rarity (v. Hippel, 1918; Friedenwald, 1927)."
Bailey and Cushing. A Classification of the Fischer. Zb. f. alg. Path, w, path. Anat,
Tumours of the Glioma Group, Phila., 1926.
Bucy and Muncie. Am. J. of Path., v., 157,
1929.
Cattaneo. An-dº Ott-, i. 759, 1923.
Collins. Researches, London, 1896.
T. O. S., xxxvii, 246, 1917: xlviii. 107,
1928.
Dejean. A. d’O., li, 257, 1934; iii, 81, 1936.
Dejean and Harant. A. S. Sc. méd, biol.,
Montpellier, xiii, 554, 1932.
Deutschmann. Z. f. Aug., xxvii, 225, 1912.
Elschnig, A. f. O., lxxxvii, 370, 1914.
Favaloro. An di Ott., lxv, 881, 1937.
xxix., 545, 1918.
Flexner. Bull. Johns Hopkins Hosp., ii, 115,
1891.
Friedenwald. Contrib. to Ophthalmic Science,
Wiscon. 23, 1927.
Fuchs. A. f. O., lxviii, 534, 1908.
Ginsberg. A. f. O., xlviii (1), 92, 1899.
Z. f. Aug., v. 208, 1901.
v. Graefe, A. f. O., x (1), 216, 1864; xii (2),
237, 1866; xiv. (2), 103, 1868.
Greeff. Deut. med. W., xxii, 327, 1896.
Griffith. T. O. S., xiv, 160, 1894.
p. 2626.

DISEASES OF THE RETINA 2843
Grinker. A. of O., v, 921, 1931. Parsons. Path. of the Eye, London, ii, 626,
Penfield’s Cytology & Cellular Path. of the 1905.
Nervous System, N.Y., iii, 1043, 1932. Pieck. A. f. O., czkxv, 451, 1936.
Hertel. K. M. Aug., xxxv, 323, 1897. Pinto, da Gama. Untersuch. ii. intraok.
v. Hippel. A. f. O., xcv, 173, 1918. Tumoren, Wiesbaden, 1886.
Hirschberg. A. f. O., xiv (2), 30, 1868. Reis. Z. f. Awg., xxxiii, 175, 1915.
Die Markschwamm d. Netzhaut, Berlin, Susman. Brit. J. O., xxii, 722, 1938.
1869. Urra. A. f. O., czii, 133, 1923.
Iwanoff. A. f. O., xv (2), 69, 1869. Verhoeff. A. of O., li, 120, 1922.
Knapp. K. M. Aug., vi, 428, 1868. Virchow. Die krankh. Geschwäslste, ii (l),
Die intraokularen Geschwielste, Karlsruhe, 151, 1864.
1868. Wintersteiner. Das Newroepithelioma Retinae,
McLean. A. of O., xviii, 255, 1937. Leipzig, 1897.
Zeiss. A. f. O., czvii, 273, 1926.
II. ASSOCIATED TU MOURS
Three syndromes, each characterized by their congenital origin, their
hereditary or familial incidence, and their widespread symptomatology, the
most marked feature of which is the appearance of multiple tumours and
cysts in various parts of the body, more particularly the central nervous
system and the retina, have been classed together by van der Hoeve (1923–
32) as the PHAKOMATOSEs (boºkós a birth-mark). They are ANGIOMATOSIS
associated usually with the names of von Hippel and Lindau, tuberous
sclerosis associated with the name of Bourneville, and neuro-fibromatosis
associated with the name of von Recklinghausen. At a later date van der
Hoeve and Mahoney (1937) added the Sturge-Weber syndrome to the triad.
In the nervous system these affections tend to attack different parts pre-
ferentially—Lindau's disease affects essentially the cerebellum, the medulla
and the spinal cord, Bourneville’s disease is usually limited to the cerebrum,
while von Recklinghausen’s disease affects the peripheral nerves and the
sympathetic system. The complexity of their clinical manifestations is seen
in Table LXV 1 modified from van der Hoeve (1932).
1. ANGIOMATOSIS.
(THE VON HIPPEL-LINDAU DISEASE)
Although the complete concept of angiomatosis of the retina as part of a general
systemic disease had to wait until the classical researches of Lindau (1926–27), the
local condition had long been known to ophthalmologists. The first case to be reported
was that of Panas and Remy (1879), who recorded the pathological examination of an
eye removed from a girl of 19, wherein the globe was filled by a lobulated cystic mass
with tufts of small vessels : Darier (1890) described the clinical condition of the other
eye which fell a victim to the same condition. A somewhat similar case was reported
by Fuchs (1882), who described an enormously dilated artery and vein associated with
a globular aneurysm and some exudate as an arterio-venous aneurysm of the retina.
Two years later, in the Argentine, Lagleyze (1884) described minutely the typical
appearance of dilated vessels in association with a peripheral aneurysm. Some
years later still, Wood (1892) brought a similar case to the Ophthalmological Society
of the United Kingdom complicated by a retinal detachment, and two years thereafter
1 p. 2844.
284.4
TEXT-BOOK OF OPHTHALMOLOGY
TABLE LXV.
VON HIPPEL-LINDAU.
Cerebellum, medulla,
spinal cord :
Angiomata. Cysts.
Proliferation of the
glia.
Syringomyelia.
Optic disc :
Angiomata.
Retina :
Angiomata.
Gliosis.
Cysts.
Degeneration.
Kidneys :
Cysts.
Hypernephromata.
Pancreas :
Cysts.
Tumours.
Ovary :
Cystadenomata.
Suprarenal glands :
Tumours.
Skin .
Angiomata.
Moles.
Bones :
Anomalies in the
vessels of the diploë
of the skull.
Arterio-venous
aneurysms.
BOURNEVILLE.
VON RECKLINGHAUSEN.
NERVOUS SYSTEMI.
Brain : Tumefaction in the
COrtex.
Proliferation of the glia.
Tumours in the ventricles.
Heterotopical spots in the
white substance.
Cysts.
Medwlla,
cord :
Sometimes tumefactions.
EYES.
Optic disc and retina :
Cysts. Tumours.
Neurofibrillomata.
Neurocytomata.
cerebellum, spinal
OTHER, ORGANS.
Heart : -
Rhabdomyomata.
Kidneys :
Cysts. Tumours.
Leiomyomata.
Angiomata.
Hypernephromata.
Fibromata.
Lipomata.
Uterus :
Fibromata.
Leiomyomata.
Intestine :
Lipomata.
Thyroid gland, mammae
Adenomata.
Skin :
Naevi. Moles.
Tumours.
Adenomata.
Fibromata.
Angiomata.
Lipomata.
Bones :
Defects in the skull ;
anomalies in the vessels
and of the diploë of the
skull.
CONGENITAL ANOMALIES.
Spina bifida, ectopia testis,
horseshoe-shaped kidney, etc.
Brain :
Heterotopical spots.
Cranial nerves, peripheral
nerves, sympathetic nerve :
Ganglioneuromata.
Neurofibromata.
Optic disc :
Degeneration
proliferation.
Cysts.
Retina :
Cysts. Tumours.
Neurofibrillomata.
Neurocytomata.
Angiomatous degenera-
tion.
Optic nerve: Tumours. Atrophy.
Iris : Tumours.
and glial
Thoraac
Intra-thoracic tumours.
Abdomen :
Intra-abdominal tumours.
Endocrine glands :
Various affections.
Skim ;
Naevi. Moles.
Tumours.
Bones :
Defects in the skull.
Affections of the spine.
Cysts. Tumours.
Spina bifida, syndactyly,
absence of kidney, etc.
DISEASES OF THE RETINA 284.5
Treacher Collins (1894) described the pathological examination of the eye of this patient,
together with that of a similar lesion occurring in the eye of his sister, as a capillary
angioma with cystic degeneration. In 1895, and again in 1903, v. Hippel, along with
others, presented cases at the Heidelberg Congress, and in the next year (1904) he
discussed 2 cases fully from the clinical point of view, and established it as a clinical
entity (von Hippel's disease): a pathological study followed some years later (v.
Hippel, 1911). The year following v. Hippel's clinical paper, Czermak (1905) reported
shortly the pathology of a case seen originally by Goldzieher (1899), but his intention
to publish it in full was forestalled by his death. Thereafter, a number of cases appeared,
but the next detailed study was that of Coats (1908), who reviewed the whole literature
and described the pathological examination of a case originally reported by Morton
(1908) (Fig. 2458, Plate LVIII). He included such cases as a sub-group of his “Forms
of Retinal Disease with Massive Exudation,” but agreed with Collins and Czermak that
Horizontal section looked at from below showing the haemangioma and cyst in
the right hemisphere (Sargent and Greenfield, Brit. J. Surg.).
FIG. 2455.-HAEMANGIoMAtous CEREBELLAR Cyst,
they should be classified separately as true vascular new-formations, a conclusion
upheld by von Hippel (1911). This condition has been recognized as such ever
since, being referred to in English literature as angioma of the retina (Pooley, 1910;
Moore, 1912) and frequently in German literature as v. Hippel's disease (Meller,
1913; Stern, 1913; Ginsberg and Spiro, 1914; Emanuel, 1915; Guzmann, 1915;
and others).
In the meantime Leber (1912) had gathered together a series of cases from the
literature characterized by the association of miliary aneurysms with marked and
progressive changes of an exudative and degenerative type occurring in young people."
Such cases, first noted by Story and Benson (1883), Story (1886) and Doyne (1896),
were classed by Leber as miliary aneurysms with retinal degeneration and were described
clinically by Oeller (1897), Schieck (1900), Krauss and Brückner (1907), Morton (1908),
Serr (1935), Dollfus (1936), and others, while they were studied pathologically by
Coats (1908), and Miyashita and Nisyake (1921). Leber (1912) considered the
multiple aneurysmal formations the result of multiple small emboli as from a
latent endocarditis, but pathological examination demonstrates their neoplastic and
1 p. 2616.

2846 TEXT-BOOK OF OPHTHALMOLOGY
angiomatous nature, the primary changes in the vessels being usually masked by the
exudative reaction (Junius, 1929–33).
Angiomatosis was thus considered to be a localized disease of the retina until
Lindau (1926), in a study of cerebellar cysts, noted the coincidence of an angiomatous
cyst in this region with an angiomatosis retinae, and discovered that the literature
contained several reports of such an association (Seidel, 1912; Berblinger, 1922;
Heine, 1923; and others) (Fig. 2455). Lindau (1927) then laid it down that the
angiomatous cysts of the cerebellum were merely isolated manifestations of a general
developmental disorder characterized by the occurrence of similar tumours elsewhere,
particularly in the retina, and frequently in the medulla and cord, and with polycystic
or angiomatous tumours in the pancreas, liver, kidneys, suprarenals, epididymis and
ovaries. This concept of Lindau's disease has been amply confirmed by numerous
monographs and case-reports which have since appeared, notably the papers of Dandy
(1928), Cushing and Bailey (1928), Sargent and Greenfield (1929), Hadfield (1929) and
Sladden (1930).
A typical case was reported by Brock, Dyke and Davison (1936) of a boy who
became blind in one eye in 1911 when aged 14, and in the other in 1918 with angiomatosis.
From 1911 to 1934 he exhibited widespread and varying gastro-intestinal, circulatory,
and central nervous symptoms. In 1928 a cystadenoma of the pancreas was removed,
and death in convulsions from involvement of the medulla occurred in 1934. Autopsy
showed innumerable cysts of the peritoneum, engorgement of the blood-vessels of the
abdominal viscera, hypernephroma of the kidney, cyst of the epididymis, haemangio-
blastomata of the retinae, brain-stem and spinal cord, and syringomyelia. At other
times the symptoms may be negligible as in the family reported by Prevec (1936),
wherein a mother and her three daughters showed merely dilated veins in the fundus,
and the diagnosis was only suggested by the development of symptoms of a spinal
tumour in the former.
It is certain that every case of Lindau's disease is not associated with retinal
complications, and, conversely, that some cases of angiomatosis retinae may be strictly
local in their incidence ; in other words, that v. Hippel’s disease is not necessarily
always a part of Lindau's. According to v. Hippel (1927), 20% of retinal cases have
intra-cranial complications. It must be remembered, however, that the cerebral or
systemic lesions may not give rise to symptoms, while the retinal lesion may be very
difficult to detect, and in many cases of cerebellar angiomata the periphery of the
retina—the part usually affected—has not been subjected to minute scrutiny. Thus
the retinal angioma may be microscopic in dimensions (Lindau, 1927), and may be
found post-mortem after the most careful clinical examination had revealed no
ophthalmoscopic anomaly (Seidel, 1932), or the condition may be represented
ophthalmoscopically merely by a dilated vein (such as was not appreciated by eight
observers, Cushing and Bailey, 1928), while anomalous appearance may lead to a
mistaken diagnosis. Thus a case reported by Thomas and Coats (1911) as a granuloma
of the retina was found on re-examination 20 years later, when the diagnosis of Lindau's
disease had been established at autopsy, to be an angioma with relatively slight
formation of blood-spaces (Sladden, 1930).
Incidence
Angiomatosis of the retina is a rare disease : reviewing the literature,
Usher (1935) was able to find only 119 cases wherein the fundus appearances
and the clinical history were in keeping with the condition. The lesion is
bilateral in 36% of the cases; in the unilateral cases the left eye has been
PLATE LVIII
*ETINAL Tuxtours
Fig. 2456.-Astrocytoxia (McLean).
Fig. 2458. ANGroMatosis (Morton).
To face p. 2846.



DISEASES OF THE RETINA 2847
slightly more frequently affected than the right. Males are more frequently
affected than females in the proportion 62 : 38. The age is most frequently
O – 3
# * :
H
@ é

i
é-
º
@
O Angiomatosis retinae.
Kº Angiomatosis retinae and cerebelli.
X, Angiomatosis cerebri and cerebelli.
FIG. 2459.-LINDAU’s DISEASE : RochAT’s HEREDITY.
the 3rd decade : of 107 cases in the literature the following are the age
periods—1–10 years, 9 ; 11–20, 32; 21–30, 44; 31–40, 14 ; 41–50, 6 ;
51-60, 2. The affection has been recorded in a prematurely born infant (v.
*
# * * * *
!

:
*
H
§
Ǻ Angiomatosis retinae and cerebral tumour.
Angiomatosis retinae and cerebellar tumour.
& Cerebral tumour.
X; Cerebellar tumour.
FIG. 2460.--LINDAU's DISEASE : MöLLER’s HEREDITY.
Hippel, 1934), and in a microphthalmic eye with a cyst in a man of 37 (Berg-
meister, 1921).
The influence of heredity is definite, but by no means universal, for a
small proportion only (about one-fifth) of the published cases show a
T.O.-WOL. III. 3 C
2848 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2462.-Multiple Angiomata (Collins, T. O. S.).

DISEASES OF THE RETINA 2849
hereditary or familial in-
cidence. It is of interest
that the earliest cases to
be recorded showed such a
character–Treacher Collins'
(1894) original cases occurred
in a brother and a sister,
and the son and daughter
of v. Hippel’s (1895) original (
case also had v. Hippel's
disease, the daughter dying
of a cerebellar cyst, syringo-
myelia, and a cystadenoma
of the kidney (Knodel, 1931),
while her eye showed a
retinal angioma (Seidel,
1932).
Interesting examples of its
hereditary transmission are
found in the cases of Griffith
and Ormond (1909), Seidel
(1912), Tresling (1920), Brandt
(1921), Polack and Frogé (1921),
Lindau (1927), Meller and
Marburg (1928), Cushing and
Bailey (1928), Möller (1929),
Mawas (1931), Hartmann and
Sourdille (1931), and Usher Fig. 246.3.−ANGIoMA RETINAE
(1935). Rochat’s (1927–31) (Foster Moore, T. O. S.).
pedigree which affected five
members of a family is illustrated in Fig. 2459, and Möller's (1929) which affected
six in Fig. 2460. Other examples occur in which the disease is hereditary, but
does not become manifest in the retina. Collier's (1931) family of four sisters is
illustrative-one had a capillary angioma of the cerebellum and a hypernephroma,
the second had a capillary angioma of the medulla and a hypernephroma, the
third died of Friedreich's disease, and the fourth had severe migrainous headaches,
while none of them had any retinal manifestations.
Clinical Picture
Clinically angiomatosis may appear with disconcerting polymorphism,
assuming many bizarre types and making its recognition frequently difficult.
The usual clinical course may be divided into four stages: (1) an early stage
of vascular dilatation and angiomatous formation ; (2) the appearance of
haemorrhages and exudates; (3) the stage of massive exudation and retinal
detachment, and (4) the final stage of glaucoma and destruction of the eye.

3 C 2
2850 TEXT-BOOK OF OPHTHALMOLOGY
As a rule the earliest sign is a fullness of the retinal veins in one segment,
which become dilated and tortuous; occasionally the ophthalmoscopic
picture develops no further, and it is only the co-existence of undoubted
signs of Lindau's syndrome in the central nervous system that indicates
Fig. 2464–ANgiomatosis RETINAE.
With extensive retinal detachment (Stallard, Brit. J. O.). -
the real significance of the dilatation. More usually the dilated vessels are
found to feed angiomatous formations which are usually, but not invariably,
found far out in the periphery especially on the temporal side (Figs. 2461-63).
Some of these may be quite small, light or dark red, or yellowish-grey areas
with an even surface, circular or oval in shape, and perhaps the size of the
optic disc. At a later stage the angioma becomes larger and raised up like
a toy balloon, and the typical stage of the disease is marked when two

DISEASES OF THE RETINA 285.1
enormously dilated vessels run out to feed a large cystic tumour, it being
difficult to distinguish which is artery and which is vein (Fig. 2463).
While these vascular changes are going on the retina becomes oedema-
tous and exudate changes begin to appear (Fig. 2457, Plate LVIII). At first
sub-retinal flecks appear, a macular star may form, a circinate arrangement
has been observed, but eventually the smaller patches become conglomerate
and large sub-retinal sheets are laid down. Partial retinal detachments may
occur simultaneously with these developments, isolated small portions in
the periphery, sector-shaped areas near the disc, or separate bulbous separa-
tions giving the appearance of multi-lobular cysts; but eventually the
Fig. 2465.-ANGIoMA RETINAE.
(Niccol and Foster Moore, Brit. J. O.).
detachment becomes complete and over it course enormous vessels, the veins
showing greater changes than the arteries, forming irregularly beaded
fusiform dilatations, great sausage-like swellings with quite irregular loops
and kinks and twists, and great turgid tortuosities (Fig. 2464). Eventually
a painful secondary glaucoma sets in, and the vision is permanently destroyed,
cataract, an atrophic irido-cyclitis and phthisis bulbi being a usual ending.
While this forms the typical clinical picture, many variations may
occur. A picture resembling papilloedema with great swelling and a macular
star may first be presented (Bedell, 1931), or the picture of circinate degenera-
tion may be simulated (Karasek, 1935). The angioma may take the form of
localized sharply defined raspberry-like tumours near the disc (Niccol and
Moore, 1934) (Fig. 2465), or a white exudative woolly mass near the disc
(Carr and Stallard, 1933), or occupy the posterior pole of the eye leaving
the periphery normal (Feig, 1938), or occur in the macular region (Gourfein-
Welt, 1920). A more frequent anomaly is the masking of the vascular

2852 TEXT-BOOK OF OPHTHALMOLOGY
condition by its exudative effects so that a condition resembling Coats’
disease results (Figs. 2466, 2458, Plate LVIII). As a rule, however, the
exudative mass is seen to be interspersed with saeculated or fusiform
vascular dilatations, and in this event the condition described by Leber
(1912) as miliary aneurysms with retinal degeneration results. Cystic
formation, retinal detachment, or secondary glaucoma may appear to occupy
the picture exclusively, obscuring the primary vascular lesion; while
occasionally a true papilloedema or a post-neuritic atrophy as a result of
the cerebellar tumour may introduce complications into the ophthalmoscopic
picture.
Fig. 2466-Angiomatosis RETINAs.
Multiple aneurysms and massive exudation. (Miyashita and Nisyake, Brit. J. O.).
In general the prognosis is bad, and without effective treatment the
condition tends to progress until the vision, and frequently the eye, is lost.
It must be remembered, however, that the disease probably frequently lies
latent indefinitely, and the fact that in the majority of cases it does not
become apparent until the 3rd decade and in a large number not until
late adult life, indicates that it quite frequently does so. Moreover, so far
as the general condition is concerned, recoveries can follow operative removal
of cystic tumours in the cerebellum and elsewhere (Cushing and Bailey, 1928;
Atkinson, 1932; and others).
Pathology
A large number of pathological studies have been made upon the condition–
Panas and Remy (1879), Collins (1894), Czermak (1905), Coats (1908), Griffith and
Ormond (1909), v. Hippel (1911–31), Meller (1913), Ginsberg and Spiro (1914), Guzmann

DISEASES OF THE RETINA 2853
(1915), Emanuel (1915), Gamper (1918), Miyashita and Nisyake (1921), Brandt
(1921), Berblinger (1922), Heine (1923), Lindau (1926), Paton, Williamson-Noble
and Greenfield (1930), Marchesani (1930), Mawas (1931), Barriere (1931), Oguchi
(1931), Carr and Stallard (1933), Junius (1933), Niccol and Moore (1934), Czukrasz
(1937), McDonald and Lippincott (1938), and others.
In advanced cases where the disease is diffuse the general picture may
resemble massive exudative retinitis, enormous masses of exudative and
Fig. 2467.-ANGIoMATosis RETINAE.
General view. A. Fibrous tissue mass on the outer aspect of retina (B).
D. Sub-retinal exudate. E. Choroid (Coats, R. L. O. H. Rep.).
haemorrhagic material in various stages of degeneration and fibrosis lying
between the choroid and the retina (Fig. 2467). The retina itself, when its
structure has not been destroyed, shows large convoluted vessels and
cystic spaces of obviously neoplastic nature (Fig. 2468). In the more
circumscribed cases as a rule the greater mass of the actual tumour is occupied
by a cellular matrix consisting chiefly of glial cells (Fig. 2469); these form a
dense interlacing network in which are less conspicuous clusters of endo-
thelial, fibroblastic and other cells, while scattered about, sometimes in large
masses, are degenerated blood-elements and sometimes sheaves of cholesterol
crystals. Enmeshed in this matrix are always innumerable capillaries and
large cystic dilatations sometimes filled with blood and sometimes empty,

2854
TEXT-BOOK OF OPHTHALMOLOGY
1G. 2468.-Angiomatosis RETINAE.
F.
periphery. The rods and
H. Rep.).
O
formation of vessels in the retinal
Dilatation and new
cones are lost and the retina degenerated (Coats, R. L.
|- -
ſae.
|-|×
№.
·
|-
ſae.
№.
}
|-
|-
|-
ſ.
##|-№
§3ºº.·
§§§：
Fig. 2469.-Angiomatosis RETINE.
g- -
e and
Donald alu
-work of connective tissu
smainly degenerated connective tissue (Me
y spaces in a ground
Showing large capillar
The area up and to the righti
Lippincott, A. of 0.).


DISEASES OF THE RETINA 2855
the largest being frequently devoid of lining cells, either as the result of
pressure or of degeneration.
The great preponderance of glial elements has led some observers to
consider the neoplasm ectodermal in origin, and primarily a gliosis followed
by a secondary vascularization (Meller, 1913; Czukrasz, 1937; and others):
the terms gliosis retinae diffusa teleangiectodes angio-gliomatosis (Ginsberg
and Spiro, 1914), gliosis retinae diffusa (Guzmann, 1915), or angiogliosis
retinae (Heine, 1923) have therefore been suggested. There seems no doubt,
however, that the original view of Collins (1894) is correct, that the neoplasm
is mesodermal arising primarily from the blood-vessels, even although the
subsequent reactive gliosis may almost mask its true derivation (Lindau,
1926–27 ; Grinker, 1931; McDonald and Lippincott, 1938; and others)
The fundamental feature is therefore the angiomatous growth, no matter
how important the subsequent developments, gliosis or exudative, may
appear to be. It seems undoubted, also, that the neoplasm depends on a
congenital anomaly, arising, according to Lindau (1926), in the third month
of foetal life. It is therefore a true haemangioblastoma.
The origin of the anomaly is unknown. Junius (1933) suggested that it depends
on a congenital absence of vessels in part of the retina, and is in reality a compensatory
mechanism to make good the deficiency, embryonic vessels which would normally
disappear being retained and eventually bursting out into tumour formation. Such
an angioblastomatous new-growth is to be sharply differentiated from a congenital
Fig. 2470.-H.EMANGIoMA RETINAE.
-- The same tumour as Fig. 2461 after treatment with radon seeds. Note the
obliteration of the large vessels of supply (Stallard, Brit. J. O.).

2856
TEXT-BOOK OF OPHTHALMOLOGY
vascular malformation, which may be capillary, venous or arterio-venous in type, in
which case damage is caused merely by the pressure of the enlargement of the vascular
channels (Cushing and Bailey, 1928).
Treatment.
Treatment is difficult, and, unless the condition is in its
early stages, impossible. In early cases, however, attempts at destruction
of the growth may be successful by applying radium (Holm, 1917; Traquair,
1932) or radon seeds over the sclera (Foster Moore, 1933; Stallard, 1933;
MacDonald, 1940), by roentgen therapy (Cordes and Hogan, 1940), by
electrolysis (Neame, 1936), or by diathermic puncture (Weve, 1939) (Fig.
2470). In favourable cases by any of these methods the end-result may be
good provided the case is an early one, but sufficient experience has not
yet accrued to allow of a dogmatic judgment on their relative values.
Atkinson. A. of O., vii, 510, 1932.
Barriere. A. d. Oft. H.-A., xxxi, 425, 1931.
Bedell. Am. J. O., xiv, 389, 1931.
Berblinger. A. f. O., cx, 395, 1922.
Bergmeister. A. f. O., cv, 1, 1921.
Brandt. A. f. O., cvi, 127, 1921 ; czviii, 348,
1927.
Brock, Dyke and Davison. A. of O., xv, 957,
1936.
Carr and Stallard. Brit. J. O., xvii, 525, 1933.
Coats. R. L. O. H. Rep., xvii, 440, 1908.
Collier. Brit. Med. J., ii, 144, 1931.
Collins. T. O. S., xiv., 141, 1894;
165, 1918; xlviii, 135, 1928.
Cordes and Hogan. A. of O., xxiii, 253, 1940.
Cushing and Bailey. A. of O., lvii, 447, 1928.
Tumours arising from the Blood-vessels of
the Brain, London, 1928.
Czermak. B. O. G. Heidel., xxxii, 184, 1905.
Czukrasz. Brit. J. O., xxi. 368, 1937.
Dandy. A. of Surg., xvii, 715, 1928.
Darier. A. d’O., x, 203, 1890.
Dollfus. Bull. S. d’O. Paris, xlviii, 308, 1936.
Doyne. T. O. S., xvi, 94, 1896.
Emanuel. A. f. O., xc, 344, 1915.
Feig. Brit. J. O., xxii, 295, 1938.
xxxviii,
Fuchs. A. f. Aug., xi, 440, 1882.
Gamper. K. M. Aug., lxi (2), 525, 1918.
Ginsberg and Spiro. A. f. O., lxxxviii, 44,
1914.
Goldzieher. Cb. pr. Aug., xxii, 65, 1899.
Gourfein-Welt. K. M. Aug., lxv (2), 105,
1920.
Griffith and Ormond. T. O. S., xxix., 279,
1909.
Grinker. A. of O., v, 920, 1931.
Guzmann. Z. f. Aug., xvii, 40, 1907.
A. f. O., lxxxix, 323, 1915.
Hadfield. Bristol Med.-Chir. J., xlvi, 21 1,
1929.
Hartmann and Sourdille. An. d’Oc., clxviii,
881, 1931.
Heine. Z. f. Aug., li, 1, 1923.
v. Hippel. B. O. G. Heidel., xxiv, 269, 1895;
xxxi, 199, 1903.
A. f. O., lix, 83, 1904; lxxix, 350, 1911 ;
xcv, 173, 1918 ; czviii, 348, 1927;
cxxvii, 27, 1931 ; czXxii, 256, 1934.
v. d. Hoeve. A. f. O., cv, 880, 1921 ; czi, l,
1923.
T. O. S., xliii, 534, 1923; lii, 380, 1932.
v. d. Hoeve and Mahoney. K. Acad. v.
Wetensch. te Versl., Amsterdam, xxxvi, l,
1937.
Holm. K. M. Aug., lix, 319, 1917.
Junius. Z. f. Awg., lxviii, 207, 1929.
K. M. Aug., xci, 747, 1933.
Karasek. K. M. Aug., xcv, 163, 1935.
Enodel. A. f. path. Anat., cclxxxi, 886, 1931.
Krauss and Brückner. A. f. Aug., lvii, 157,
1907.
Lagleyze. Rev. Argentina de Oft. pract. B. A.,
i, 2, 1884.
Leber. A. f. O., lxxxi (1), 1, 1912.
Lindau. Acta path. microbiol. Scand., Supp.,
1, 1926.
Acta O., iv., 193, 1927.
Marchesani. B. O. G. Heidel., xlviii, 341,
1930. .
Mawas. An. d’Oc., clxviii, 890, 1931.
Presse méd., xxxix, 985, 1931.
MacDonald. A. of O., xxiii, 564, 1940.
McDonald and Lippincott. A. of O., xx, 958,
1938.
Meller. A. f. O., lxxxv, 255, 1913.
Meller and Marburg. Z. f. Aug., lxvi, 1, 1928.
Miyashita and Nisyake. Brit. J. O., v, 448,
1921.
Möller. Acta O., vii, 244, 1929.
Moore. T. O. S., xxxii, 76, 1912.
P. R. S. Med., xxvi, 1036, 1933.
Morton. T. O. S., xxviii, 214, 1908.
Neame. P. R. S. Med., xxix., 961, 1936.
Niccol and Moore. Brit. J. O., xviii, 454, 1934.
Oeller. Atlas, 1897.
Oguchi. Acta S. O. Jap., xxxv, 987, 1931.
Panas and Remy. Amat. path. de l’Oeil, 88,
I879.
Paton, Williamson-Noble and Greenfield.
XIII Internat. Cong. O., Amsterdam, ii,
624, 1930.
DISEASES OF THE RETINA 2857
Polack and Frogé. Bull. S. d’O. Paris, xxxiii, Srinivasan. Brit. J. O., xviii, 221, 1934.
44, 1921. Stallard. Brit. J. O., Suppl., vi, 64, 1933.
Pooley. T. O. S., xxx, 238, 1910. Stern. Cb. pr. Aug., xxxvii, 298, 1913.
Prevec. K. M. Aug., xcvi, 200, 1936. Story. T. O. S., vi, 336, 1886.
Rochat. K. M. Aug., lxxviii, 601, 1927; Story and Benson. T. O. S., iii, 108, 1883.
lxxxvi, 23, 1931. Thomas and Coats. T. O. S., xxxi, 149, 1911.
Sargent and Greenfield. Brit. J. Surg., xvii, Traquair. T. O. S., lii, 311, 1932.
84, 1929. Tresling. K. M. Aug., lxiv, 306, 1920.
Schieck. B. O. G. Heidel., xxviii, 89, 1900. |Usher. T. O. S., lv., 183, 1935.
Seidel. B. O. G. Heidel., xxxviii, 335, 1912; Weve. T. O. S., lix, 43, 1939.
xlix, 535, 1932. Wood. T. O. S., xii, 143, 1892; xxv, 96,
Serr. A. f. O., czzxiii, 508, 1935. 1905; xxix, 115, 1909.
Sladden. Brit. J. O., xiv, 224, 1930.
2. TUBEROUS SCLEROSIS
(BOURNEVILLE's DISEASE)
Tuberous sclerosis, a syndrome established by Bourneville (1880) and
elaborately studied by H. Vogt (1908), is a rare congenital disease with many
systemic manifestations, particularly multiple potato-like (tuberous)
tumours in the cerebrum as well as in other organs, such as the heart,
kidneys, uterus, intestine, thyroid and skin ; it is usually fatal, the majority
of the sufferers dying before the age of 25 as epileptic idiots; sometimes
they may live to advanced age. The most striking clinical feature is the
presence of adenoma sebaceum on the skin, a hyperplasia frequently showing
&ei or yo sarconna.
ven tri e tº 4:
*

TTI-L-I-T-
! 6 6 a & orb º — abort –

FIG. 2471.-Bourn EVILLE's DISEASE (v. Bouwdijk Bastiaans).
pigmentation, and typically distributed in butterfly fashion on the face
(Pringle, 1890; Schuster, 1913). Considerable variations in the clinical
picture exist, but the three features, mental deficiency, epilepsy, and adenoma
sebaceum, are usually sufficient to establish the diagnosis.
It is a heredo-familial disease, and in the same family tree incomplete
forms of the syndrome and a strong psychopathic taint may be evident
(Critchley and Earl, 1932). In its heredity it probably behaves as a recessive
character. It has been noted in identical twins (Fabing, 1934) and has
occurred in three generations of one family (Kirpicnzik, 1910), and in 5 out
of 9 siblings (Bastiaans and Landsteiner, 1922) (Fig. 2471).
2858 TEXT-BOOK OF OPHTHALMOLOGY
The first to report a retinal tumour in this condition was probably von
Recklinghausen (1863), but little attention was paid to the matter until van
der Hoeve (1920) noted retinal neoplasms in the eyes of 7 patients
suffering from tuberous sclerosis. The appearance is fairly typical, so much
so, indeed, as to allow Nitsch (1927) to diagnose tuberous sclerosis with the
ophthalmoscope. The ocular condition is not common; thus Critchley
and Earl (1932) found a retinal neoplasm once only in their series of 29 cases.
Since van der Hoeve's papers (1920–32), however, some 26 cases have been
reported, a number which suggests that the lesion is commoner than the
meagre literature would indicate.
Fig. 2472.-TURERous Solºrosis
(van der Hoeve).
From the clinical point of view the tumours may be divided into two
classes: large nodular tumours extending into the vitreous, and oval or
circular grey areas, both of which may be found together in the same eye
(Fig. 2472). The large nodular tumours have a predilection for the region
of the optic disc (v.d. Hoeve, 1921; Nitsch, 1927; v. Herrenschwand,
1929; Bau-Prussakowa, 1933; Kuchenmeister, 1934; Rintelen, 1935;
and Regan, 1936), although they may occur also in the periphery (Horniker,
1932; A. Vogt, 1934; Messinger, 1936; Messinger and Clarke, 1937;
Block and Grove, 1938). As a rule the tumour is a glistening whitish-grey
mass, discrete and studded with nodules like a mulberry, and of the size of
the disc or larger. The modules may be compared to little buds with a thin

DISEASES OF THE RETINA 2859
stalk, and van der Hoeve (1921) found that they could break off and float
freely in the vitreous to form metastases by seeding on the retina ; occa-
sionally also cysts are present which may discharge or bleed intermittently.
The smaller flat tumours, which are the more common manifestation, are
frequently multiple ; they form white or grey areas on the retina, usually
with little measurable elevation, and have an average diameter of about
half that of the disc (Schob, 1925; Overbosch, 1926; Guillain and Lagrange,
1934; Gottlieb and Lavine, 1935; Storchheim and Taube, 1936; Hopwood,
1937; Kveim, 1937; Koch and Walsh, 1939). They are not in any close
relation with blood-vessels, appearing to be relatively avascular, and they
FIG. 2473.−TUBERous ScLERosis.
The cells of a retinal tumour showing variation in size and shape, vacuolation,
and a tendency to form a syncytium (Messinger and Clarke, A. of O.).
excite no tissue-reaction. On two occasions the ophthalmoscopic picture
has been complicated by choked discs due to long-standing raised intra
cranial pressure (Guillain and Lagrange, 1934; Koch and Walsh, 1939).
Pathological examinations have been reported by van der Hoeve (1923),
Schob (1925), Feriz (1930), Kuchenmeister (1935) and Messinger and
Clarke (1937), and all have similar appearances. Most usually the tumours
lie superficially extending not deeper than the nerve-fibre layer (v.d. Hoeve,
Schob) or the ganglion layer (Feriz); but they sometimes involve all the
thickness of the retina (Kuckenmeister, Messinger and Clarke). The mass
is made up essentially of fibres and cells among which may be incrustations
of calcareous deposits and concretions (v.d. Hoeve, Kuchenmeister,
Messinger and Clarke), while large spaces filled with blood and serum may
be prominent. van der Hoeve interpreted the fibres as derived from the
nerve-fibre layer, but subsequent workers have concluded that they are

2860 TEXT-BOOK OF OPHTHALMOLOGY
glial in nature. The cells are peculiar and characteristic (Fig. 2473); they
are large and polymorphic, sometimes round, sometimes angulated, some-
times spider-shaped, with a round vesicular nucleus, a prominent nucleolus,
and an indistinct cell-boundary suggesting a syncytium. They are probably
embryonic in type and glial in nature, but have obviously not differentiated
to any marked extent either into neuroglia or nerve cells. For this reason
Perraro and Doolittle (1936) suggested the term diffuse neuro-spongio-
blastosis for the disease. A definite classification is difficult, but the tumours
are probably astroblastic in origin, perhaps related to the rare astrocytomata,"
and of relatively low malignancy.
The ocular condition gives rise to no particular symptoms except a
decrease in vision, and is entirely subsidiary to the general one, for which
there is no effective treatment.
Bastiaans and Landsteiner.
lxvi (2), 248, 1922.
Bau-Prussakowa. Z. f. Neurol, w. Psy., cylv,
Ned. tij. v. Gen.,
275, 1933.
Block and Grove. A. of O., xix, 34, 1938.
Bourneville. A. de Newrol., i, 81, 1880.
da Costa, Mendes. Nederland. Dermatolo-
genverein. See Zb. Haut. u. Geschlechtskr.,
xxvii, 739, 1928.
Critchley and Earl. Brain, Iv, 311, 1932.
Fabing. Brain, lvii, 227, 1934.
Feriz. A. f. path. Amat., colxxviii, 690, 1930.
Ferraro and Doolittle. Psy. Quart., x, 365,
1936.
Gottlieb and Lavine. A. of Neurol, and Psy.,
xxxiii, 379, 1935.
Guillain and Lagrange. Bull. S. méd. Hôp.
de Paris, l, 1421, 1934.
v. Herrenschwand. K. M. Aug., lxxxiii, 732,
I929.
v. d. Hoeve. T. O. S., xi, 329, 1920;
534, 1923 ; lii, 380, 1932.
A. f. O., cv, 880, 1921 ; czi, 1, 1923.
xliii,
Hopwood. Ohio State Med. J., xxxiii, 277,
1937.
Horniker. B. O. G. Heidel., xlix, 357, 1932.
Boll. d’O., xi, 497, 1932
Kirpicznik. A. f. path. Amat.,
1910.
Koch and Walsh. A. of O., xxi., 465, 1939.
Ruchenmeister. Derm. W., xcix, 133, 1934.
Z. f. Aug., lxxxviii, 158, 1935.
Kveim. Acta dermat.-vener., xviii, 637, 1937.
Messinger. Am. J. O., xix, 516, 1936.
Messinger and Clarke. A. of O., xviii, 1, 1937.
Nitsch. Z. f. Aug., lxii, 73, 1927.
Overbosch. Ned. tij. v. Gen., ii, 2632, 1926.
Pringle. Brit. J. Derm., xx (2), 1, 1890.
v. Recklinghausen. Verhal. d. Geselsch. f.
Geburtsch., Berlin, xv, 75, 1863.
Regan. Am. J. O., xix, 516, 1936.
Rintelen. Z. f. Aug., lxxxviii, 15, 1935.
Schob. Z. f. Neurol. Psy., xcv, 731, 1925.
ccii, 358,
Schuster. Deut. Z. f. Nervenhk., i, 96, 1913.
Storchheim and Taube. Am. J. O., xix, 508,
1936.
Vogt, A. Z. f. Aug., lxxxiv, 18, 1934.
Vogt, H. Momat. f. Psy. Neurol.., xxiv, 186,
1908.
3. NEURO-FIBROMATOSIS
(VON RECKLINGHAUSEN's DISEASE)
Neuro-fibromatosis, a disease associated with the name of v. Reckling-
hausen, like the preceding, has a congenital basis and is characterized by
neoplastic anomalies of the nature of ganglio-neuromata and neuro-fibromata,
particularly in association with the peripheral and sympathetic nerves, and
pigmented neuro-ectodermal tumours of the skin, all of which are probably
derived from the cells of the sheaths of Schwann. Like the two preceding
neoplasms it may have a familial tendency (Fig. 2474). It is to be noted
1 p. 2840.
DISEASES OF THE RETINA
2861
*
6 73-y —d
+22 y
- = Recklinghausen.
& = Recklinghausen + iris tumours.
FIG. 2474. —v. RECKLINGHAUseN's Disease
(Waardenburg).
that some authorities consider that this disease is essentially the same as
tuberous sclerosis, but that in the first case the tumefactions are peripheral,
in the second central (Bielschowsky, 1924; Dickson, 1933). We have already
FIG. 2475.-v. RECRLINGHAUSEN's Disease.
Intra-ocular neuroma (Stallard, Brit. J. O.).

2862 TEXT-BOOK OF OPHTHALMOLOGY
noted the occurrence of pigmented tumours in the iris in this affection, 1
while neuro-fibromata of the optic nerve are well known 2: van der Hoeve
(1920-32) has drawn attention to the occurrence of multiple retinal tumours
very similar to those characteristic of tuberous sclerosis. The pathology of
such a tumour affecting the nerve-head and invading the retina laterally
-
Fig. 2476.-v. Rºokinghausen's Disease.
The tumour of Fig. 2475. Note the cystic spaces, clefts, bunches of neurocytes
and neurofibrils and areas of hyaline degeneration (stallard, Brit. J. o.).
between the nuclear layers was described by Stallard (1938); it was
surrounded by small yellow flecks of exudate (Fig. 2475). The growth was
a neuroma, containing many cystic spaces and clefts, and consisting of
bunches of neurocytes and neurofibrils with areas of degeneration (Fig. 2476).
Bielschowsky. J. f. Psy, w. Neurol.., xxx, 167, v, d. Hoeve. T. o. S., x1, 329, 1920; xliii,
1924. 534, 1923; lii, 380, 1932.
Dickson. Brit. Med. J., ii, 1016, 1933. A. f. O., ev, 880, 1921; exi. 1, 1923.
Stallard. Brit. J. O., xxii, 11, 1938.
1 p. 2471. * p. 3087.

DISEASES OF THE RETINA 2863
SARCOMA
Sarcomata have been reported as arising from the connective tissue associated
with the retinal blood-vessels by Schieck (1912) and Elschnig (1914); Berblinger (1922)
considered that the first of these cases was an angioma.
Berblinger. A. f. O., ex, 395, 1922. Schieck. A. f. O., lxxxi, 328, 1912.
Elschnig. A. f. O., lxxxvii, 370, 1914.
B. Secondary Tumours
A direct invasion of the retina by a malignant melanoma of the choroid
is, as we have seen," not unusual (Hirschberg and Happe, 1870; Williams and
Knapp, 1874; Knies, 1877; Treitel, 1883; and many others); metastatic
Fig. 2477.-METAstATIC CARCINoMA of RETINA.
A. The main mass of growth. c.—e. Implantation metastases (Smoleroff and
Agatston, A. of 0.).
tumours are, however, surprisingly rare. A METASTATIC CARCINoMA of the
retina is of exceptional rarity. Such a case was reported by Sattler (1926)
in a patient with carcinoma of the pancreas, and it is probable in this case
that although the whole of the retina was replaced by new-growth, the
original metastatic site was the optic disc. A second case described by
Smoleroff and Agatston (1934) illustrated the development of a metastasis
from a gastro-oesophageal carcinoma in the retina itself, without involving
any other portion of the globe (Fig. 2477). The origin of the growth, which
p. 2483.
T-o,-vol. 111. 3 D

2864 TEXT-BOOK OF OPHTHALMOLOGY
was a typical alveolar adeno-carcinoma, was in the nerve-fibre layer where it
sprang from the wall of a blood-vessel in which the embolus was presumably
held up. The tumour grew into the sub-retinal space but Bruch's membrane
was intact and the choroid un-infiltrated. The case reported by Arisawa
(1914) as a true carcinoma of the retina was probably a metastasis from an
abdominal tumour.
Two cases of METASTATIC SARCOMA affecting the papilla and the surround-
ing region of the retina have been reported (Schiess-Gemuseus and Roth,
1879; Heine, 1899).
Arisawa. K. M. Aug., lii, 386, 1914. Schiess-Gemuseus and Roth. A. f. O., xxv (2),
Heine. K. M. Aug., xxxvii, 326, 1899. 177, 1879.
Hirschberg and Happe. A. f. O., xvi, 302, Smoleroff and Agatston. A. of O., xii, 359,
1870. 1934.
Enies. A. f. Aug., vi, 158, 1877. Treitel. A. f. O., xxix (4), 179, 1883.
Sattler. Die basartigen Geschwiilste des Auges, Williams and Knapp. A. f. Aug., iv, 83, 1874.
Leipzig, 1926.
VIII. FOLDINGS AND DETACHMENTS OF THE RETINA
RETINAL GROOVES
Retinal grooves formed as the result of extreme deformation of the globe have
occasionally been noted, usually in association with an orbital tumour (Birch-
Hirschfeld, 1920; Löhlein, 1927; Thiel, 1932; Kugelberg, 1932). Kugelberg's
case was typical : it resulted from the deformation produced by a particularly severe
exophthalmic goitre and showed a series of light and dark lines alternating in a striate
arrangement occupying an area from the nasal side of the retina to the disc and beyond
the macula on the temporal side. The folding produced the entoptic appearance as
if objects were horizontally striated. It appeared that only the inner layers of the
retina were folded, and the phenomenon was interpreted as being an increase in
thickness to compensate for a decrease in extension.
Birch-Hirschfeld. G.-S. Hb., II., ix (1), 1920. Löhlein. K. M. Aug., lxxix, 769, 1927.
Rugelberg. K. M. Aug., lxxxix, 171, 1932. Thiel. Atlas, Berlin, 1932.
Detachment of the Retina
The term “detachment of the retina ’’ is a misnomer. In the first place the retina
as a whole is not involved, but a cleavage occurs between the two primitive retinal
layers, the pigment epithelium remaining in position attached to the membrane of
Bruch, while the inner (neural) layers become separated from it. Moreover, apart
from attachments at the nerve head and the Ora Serrata, the two layers are never
attached ; between the two the potential space of the optic vesicle is bridged across
only by the processes of the pigment cells.” The retina, therefore, is never, strictly
speaking, detached, but its inner layer becomes displaced from the outer, allowing
the potential space of the primary optic vesicle to become an actual one ; it would
* Vol. I, p. 82.
DISEASES OF THE RETINA
Fig. 2478.

2866 TEXT-BOOK OF OPHTHALMOLOGY
therefore be more accurate to speak of separation of the retina. To change a term,
however, so convenient which has gained the rights of possession by long usage,
would be mere pedantry ; and we will therefore also speak of the “sub-retinal space ’’
and “sub-retinal fluid,” where, more correctly, the term “inter-retinal ‘’ should be
applied.
The condition of detachment of the retina is not a pathological entity,
but is an anatomical accident which may be the result of many causes.
According as to whether these causes are obvious or obscure, it has been the
habit to classify detachments as SECONDARY or IDIOPATHIC (SPONTANEOUS).
Such a classification has the merit of differentiating between the two com-
pletely different view-points from which a detachment is considered from the
clinical aspect. In the first case the condition is an incident, an epiphe-
nomenon, a matter of secondary interest in a clinical picture dominated
by a neoplasm, gross inflammation, or some other pathological feature of
first-class importance ; in the second, the detachment itself is the essential
catastrophe and the causal lesion may be so slight as to escape notice even
when searched for. In the first case the clinical importance of the detach-
ment is frequently the fact that its presence may obscure the diagnosis ; in
the second, it becomes the sole focus of attention. Our knowledge of the
aetiology of retinal detachments is by no means perfect, but it seems probable
that the difference between the two classes is of degree rather than of kind,
that all detachments are secondary, the so-called idiopathic cases being
dependent on inflammatory or degenerative changes so slight as to be
recognized only with difficulty or sometimes not at all.
Historical. Historically the record of retinal detachment is not long. The
condition was described in animals' eyes by Maitre Jan (1722) and Morgagni (1740),
and was referred to by St. Yves (1722); but the first reliable histological observations
were due to Ware (1805), Wardrop (1818), and Panizza (1826). By these authors it
was described as hydrops subchoroidalis in contra-distinction to hydrops subscleroticalis–
a detachment of the choroid. Clinically the condition was described, but not
recognized, by Beer (1817) as amaurotic cat’s eye : but shortly afterwards the white
vascularized membrane was seen through the dilated pupil by Chelius (1839), Sichel
(1841), and Desmarres (1847). With the introduction of the ophthalmoscope, however,
its accurate clinical description became easy, and its frequency and importance among
ocular catastrophies became established (Coccius, 1853; van Trigt, 1853; Arlt, 1853;
von Graefe, 1854), while numerous detailed histological investigations rapidly
followed (H. Müller, 1858; Pagenstecher, 1862; Schweigger, 1863; and others).
The remainder of the story is occupied by the records of a long series of hypotheses to
account for the occurrence of the so-called idiopathic type of detachment and of as
long a series of attempts to bring about its reposition, all of which proved abortive
until comparatively recent years, when, after much patient research, Jules Gonin,
of Lausanne (1925–28), emphasized the importance of retinal tears in the aetiology of
the condition and the possibility of its cure by their closure. Gonin (1870–1935),
whose portrait appears in Fig. 2478, will always be remembered as being the first to
bring hope into the treatment of a lesion which hitherto had appeared virtually
hopeless ; but the service which he thus rendered to ophthalmology should not be
assessed alone in terms of the therapeutic revolution which he inaugurated, but also
in the light of the stimulus which his work gave to the intensive and productive
DISEASES OF THE RETINA 2867
research into the aetiology and pathology of the condition which has appeared during
the last decade and is still continuing.
AEtiology and Pathology
Predisposing Factors. As we have already suggested, the aetiology of
retinal detachment is a complex and, as yet, obscure problem. The literature
is enormous and confused, and a study of it shows that many who have
theorized on the subject have erred in their attempt to include all detach-
ments of the so-called idiopathic type under one particular heading. This is
certainly not the case, for the condition forms an incident in many patho-
logical processes. Before going on to the actual mechanisms producing
detachment, we shall first of all consider some predisposing factors in its
aetiology. -
1. Race. The marked preponderance among Jews is interesting ;
Gonin (1934) found that in his cases it occurred ten times more frequently
in this race than in others.
2. Heredity. This appears as an influence only very occasionally.
A number of the families recorded, however, were myopic and it seems
probable that this tendency rather than the detachment was inherited (Arlt,
1888; Salzmann, 1921; Schmelzer, 1929; Bane, 1930 ; Arruga, 1933).
Other records are similarly complicated by ocular anomalies which have
been hereditarily transmitted (e.g., Collins, 1892; Clarke, 1898; Pagen-
stecher, 1913; Heine, 1925; Komoto, 1926; Wagener and Gipner, 1925 :
Hemmes, 1931). The cases in which a seemingly uncomplicated detachment
runs in the same family are rare (Kennon, 1920; Schreiber, 1920 ; Vogt,
1924); Richner (1936) collected 18 pedigrees from the literature and 32
from the material at Zürich ; Zimmer (1937) gathered 34 cases from the
literature, while recent reports have been added by zur Nedden (1936),
Vogelsang (1937), and de Rötth (1939); Friedman (1937) saw a family
of 7, 5 of whom suffered from detachment. It would seem that the hereditary
element which is transmitted is the retinal degeneration which predisposes
to detachment.
3. Sea. The influence of sex is marked, males being affected in about a
two-thirds majority, a preponderance which probably depends largely, but
not exclusively, on the incidence of trauma. Nordenson (1887) found 7.3%
males, Poncet (1887) 62% ; Sattler (1905) 66% ; Helming (1915) 72%
Stallard (1930) 62% ; Arruga (1933) 60% : Shapland (1934) 61% ; and
Dunnington and Macnie (1937) 67%.
4. Bilaterality. A detachment of the retina frequently occurs in both
eyes; indeed, the tendency to bilateral occurrences is sufficient to merit
anxiety if a unilateral detachment occurs without a precedent chain of
events which definitely implicates the one eye. The statistics on this point
vary considerably according as they are compiled—for example, whether
they include lesions such as renal retinopathy or exclude trauma.
2868 TEXT-BOOK OF OPHTHALMOLOGY
According to Magnus (1883), 4.47% are bilateral ; Galezowski (1883), 9% and
(1895), 2.5% ; Gonin (1904), 11.6% ; Deutschmann (1910), 32% ; Elschnig (1914) 25% ;
Fodor (1933), 8% ; Arruga (1933), 7.7% of 682 patients ; Oradovskaya, Przhibylskaya
and Skorodinskaya (1934), 11% of 251 patients ; v. Manen and Weve (1936), 22% ;
Gifford (1936), 22.6% ; Bartels (1936), 9% of 186 patients; Jeandelize, Baudot and
Gault (1936), 9.2% of 123 patients; Dunnington and Macnie (1937), 17.8% of 314
patients; Maertens (1937), 20% of 229 patients; de Rötth (1939), 12.2% of 270
patients. It is interesting that in practically all these cases the greater proportion,
some 60–70%, occurred in men. The commonest age is between 30 and 45, and the
second eye is usually involved within 5 years of the first.
Apart from traumatic cases and the secondary exudative types (as in
nephritis) the above statistics show a sufficiently large proportion of bilateral
cases to excite interest. One of the most interesting features is that the
detachments in the two eyes are usually symmetrical in position and that the
same type of hole or dialysis exists in both. This suggests some congenital
weakness or a tendency to pre-senile degeneration which pre-disposes to
detachment ; as we have just seen, the occurrence of familial detachment
suggests that such a tendency can on occasion be hereditary.
5. Age. Detachment of the retina is very rarely congenital, and most
of the cases which have been published show that it has been due to a major
trauma at birth followed by hamorrhage (Hamma, 1910), gross intra-uterine
inflammation (Collins, 1892; Clarke, 1898; Rockliffe, 1898; Fleischer,
1907; Pesme, 1930; and others), or in association with congenital
deformities (Wagener and Gipner, 1925; Komoto, 1926). Again, in
infancy detachments are rare, but the incidence increases rapidly at
20 years, and much more rapidly again at 40, attaining a maximum
between 50 and 60, to fall rapidly again after 70 (Hofe, 1934) (Table LXVI)."
It is apparent, therefore, that in the decade when senile changes usually begin
to take place retinal detachments are most common.
TABLE LXVI.
Up to 40 Years Above 40 Years
Poncet (1887) . e tº e- 35% 65%
Löhlein (1931) . e e . 13 cases 40 cases
Years 1–10 1 1–20 21–30 31–40 41–50 51–60 61–70 Over 70
Stallard (1930) ... — 2 cases 20 16 20 27 13 l
S-y–’ S--"
Lindner (1931) º I 12 10 14 18 15
Arruga (1933) . e 12 24 73 120 155 174 103 21
6. The Influence of Myopia. The first to draw attention to the fact
that a retinal detachment was a frequent complication of myopia was von
* Vol. II, p. 1477.
DISEASES OF THE RETINA 2869
Graefe (1857), who found that this refractive condition was present in from
50% to 60% of cases. Summarizing the results in the literature between
1879 and 1914, Leber (1916) obtained a figure of 65% as representing the
occurrence of the higher grades of myopia. Other authors give similar
figures: Seible (1916) 51% ; Uhthoff (1922) 61% ; Arruga (1933) 58% ;
Shapland (1934) 62% ; Gonin (1934) 66% ; Dunnington and Macnie (1935)
66.6%. Helming (1915) found 33% and Schreiber (1920) 37% with myopia
as the only apparent cause. It may be taken, therefore, that about two-
thirds of the cases of detachment occur in myopes, and in more than half of these
cases no other obvious cause has been moted. Looked at from the opposite
point of view, a very considerable proportion of myopes develop detach-
ment ; Horstmann (1879) 3.5% ; Schleich (1882) 2.5% ; Arruga (1933) 5%.
Moreover, they tend to develop the condition at an earlier age than other
refractive denominations, the maximum frequency occurring between
36–40 years in myopes (Arruga, 1933) as against 50–60 in detachments
generally. Further, in the higher degrees of myopia, detachments are most
commonly found even although the higher degrees of myopia are much less
frequent than the lower.
Thus Horstmann (1879) found 39 cases in myopes of less than 8 D, and 43 in
those of more than 8 D ; Otto (1897) found it to occur in 5.9% of eyes with over 10 D
of myopia, and von Hippel (1899) in 6-7%. In a series of detachments, Nordenson
(1887) reports 26.3% with myopia under 6 D and 30.7% with myopia greater than
this ; while Gonin (1920) in a series of 80 cases found 16 below and 37 above this limit.
Shapland (1934) in a series of 425 cases found 62.3% myopes of which 20.2% were
under 4-5 D, 20.2% were between 5 and 10 D, and 21.9% were over 10 D. Arruga's
(1933) figure of 58% myopes was made up of 14% less than 4 D, 12% between 4 and
8 D, 22% between 8 and 16 D, and 10% between 16 and 32 D. Gonin’s (1934) figures
for the same divisions are 13%, 25%, 32% and 9%. It appears, therefore, that
detachments occur with considerable frequency in all grades of myopia, and that the higher
the myopia the greater the tendency to develop the condition.
7. The Influence of Eye-strain. Some authors consider that ocular
work enters into the aetiology of detachment (v. Csapody, 1932), but any
such influence must be extremely small and unimportant. When the
ciliary muscle contracts in the act of accommodation it is known that the
retina is slightly pulled forwards as far back as the macula," and it is con-
ceivable that this forward movement might precipitate a detachment already
imminent. It is interesting that Gonin (1934) has noted the occurrence of
detachments following the strengthening of the glasses of myopes, more
frequently than can be accounted for by coincidence.
8. Traumatism. A definite history of trauma in the aetiology of
detachment of the retina is an association which has long been recognized.
If perforating injuries are excluded, Leber (1916) considered that trauma
played a part in from 16 to 18% of detachments; Stallard (1930) found a
1 Vol. I, p. 565 (574).
2870 TEXT-BOOK OF OPHTHALMOLOGY
history of trauma in 16%, Shapland (1934) in 26%, Dunnington and Macnie
(1935) in 30%. It must be remembered that a history of this nature is not
necessarily reliable, for it is easy for a patient to discover some past injury,
and it is as easy, especially when it is remembered that the detachment may
become obvious only after the lapse of some time, to forget it.
The effects of trauma may be various, depending on the nature of the
injury ; some of these are gross, but more frequently they are not at all
obvious. The following types may be briefly noted.
A. Direct Injuries to the Eye, including (1) perforating wounds, with
or without subsequent infection ; (2) intra-ocular foreign bodies :
(3) contusions with or without scleral rupture.
The influence of the first two factors is obvious and they will be con-
sidered in detail subsequently. It may suffice to note that they may give
rise to two types of detachment ; a primary type, when the detachment
occurs at the time owing to the trauma itself, and a secondary type where it
occurs at a later stage, and is due to cicatrization and retraction of scar
tissue. So far as contusions are concerned, if they are of sufficient severity
they may well be the sole cause of detachment in a healthy eye. This may
occur either immediately or remotely. In the first case the mechanism of
the detachment may be a tearing of the retina from its attachments, either
at the ora or the optic disc, usually the former, by the sudden distension of
the globe. A localized dialysis of this type at the Ora is relatively common,
but very extensive tears may occur, extending half-way (Jeandelize, Baudot
and Delaveuve, 1931) or even completely round the circumference (Velhagen,
1900; Römer, 1901). An avulsion of the retina from the disc giving rise to
a total detachment of the retina is much more rare (Paul, 1905; Lister, 1924)
(Fig. 2500). Alternatively, damage may be done to the retina involving
the immediate or delayed formation of a hole, which subsequently leads
to a detachment. Such a hole usually occurs in one of three vulnerable
positions: (1) at the site of the injury, (2) at the macula, which is struc-
turally the weakest part of the retina, or (3) at the point opposite the site
of injury, i.e., the point of contrecoup. It is probable, however, as we
shall discuss more fully immediately, that contusions of a degree much
less than is required to cause such direct injury are responsible in a very
much larger number of cases for the incident of a detachment of the retina in
an eye already liable to this accident by the presence of some disease or
degenerative process. Such, for example, is the case of a detachment
following rubbing the eyes with the fingers to relieve a feeling of itchiness
(Gonin, 1934).
B. Indirect Injuries to the Eye, including (1) blows and contusions on
the head or, rarely, the body ; (2) sudden movements or shaking ; (3) the
increase of local blood pressure due to physical straining, such as, typically,
efforts to lift a heavy weight, a sudden increase in abdominal pressure, and
SO OFl.
DISEASES OF THE RETINA 287|
To a very large extent these indirect injuries, which are usually small,
act, not as the primary cause of detachment, but merely as the precipitating
agent in an eye already prome to this accident ; as we shall see later, the
sudden strain determines the formation of a tear in the retina after other
factors, such as degenerative or inflammatory processes, have produced a
region of weakness. In this sense, by acting as a trigger in starting a
chain of events which have already been predetermined by other and more
fundamental factors, minor and indirect traumata figure very constantly
in the aetiology of the condition. The practice of all ophthalmologists is
full of such incidents, as witness the occurrence of a detachment after
gymnastic exercises, a train journey, during the tail-spin of an airplane
(Neblett, 1924), abdominal straining (Ammann, 1919), the extraction of
teeth (McDavitt, 1920), and innumerable other such circumstances. On the
other hand, when the eye itself is healthy, a retinal detachment from
this cause alone is somewhat rare, a fact well seen in the comparative
rarity of the occurrence of this accident in the Great War (v. Szily, 1918;
Lagrange, 1918; Axenfeld, 1922). The choroid, indeed, is more easily
ruptured than the healthy retina, and the normal eye can stand the most
severe trauma involving even rupture of the sclera and the loss of vitreous
without the occurrence of a detachment.
One point noteworthy in connection with contusions as an aetiological
factor is the possibility of a considerable interval elapsing between the
receipt of an injury and the recognition of symptoms. The occurrence of a
delayed detachment may be due to several reasons, but probably the most
common is that the retina is torn at the time of the accident but does not
become detached, or so extensively detached as to attract notice, until some
time has elapsed. Alternatively, a localized oedema with a circumscribed
peripheral shallow detachment occurs at the time, which later becomes more
extensive. Many examples of this are to be found in the literature ; 4 weeks
(Ammann, 1904; Pfalz, 1904 ; Weill, 1906) ; 5 weeks (Oncken, 1905;
Cramer, 1905), and many others. Histories dating back several years must,
of course, be accepted with reserve (Brinton, 1922; and others), although
the sequence is obvious when cicatricial tissue and retracting scars have been
formed (Schwarz, 1907).
These considerations, of course, make the assessment of a claim for compensation
in many cases difficult. On the one hand, a patient is ready and wishful to conjure
up an accident to account for his symptoms ; and on the other hand, an accident of
some months priority which has passed unnoticed may be the real cause. Further, in
many eyes predisposed to detachment by myopic and senile changes, a strain or
considerable exertion sustained at work may prove to be the exciting factor which
precipitates the event ; and in this case partial compensation would seem the fairest
treatment. Again, years after the extraction of a foreign body, a perforating wound,
a haemorrhage, or the extraction of a traumatic cataract, a detachment may develop—
long after the claim has been settled and the eye assessed as of considerable use. It
is a complex and difficult subject requiring careful consideration (see Vogt, 1929;
Cords, 1930).
2872 TEXT-BOOK OF OPHTHALMOLOGY
The question of DETACHMENTS AFTER CATARACT EXTRACTION may be noted briefly
here. Detachments certainly occur more frequently in aphakic than in phakic eyes,
and may usefully be classified in three groups:
(a) After Eartra-capsular Eatraction. In these cases detachment occurs in about
2% after an average interval of 4 years—a figure which makes this a complication of
considerable importance in the operation, especially in myopes. After the primary
operation the main aetiological factor is loss of vitreous. Baurmann (1929) found a
detachment following 10% of extractions where this complication occurred, and
1.87% where it did not. The typical story in these cases is an incarceration of
vitreous in the wound, sometimes accompanied by capsular remains, a gradual con-
traction of this towards the scar in the process of cicatrization, with resultant traction
of the retina producing a detachment usually in the lower part of the globe.
After capsulotomy, detachments are more frequent than after the operation of
extraction itself. This may be due to actual tearing of the retina by too strenuous
manipulation at the time of the operation (Csapody, 1932), to vitreous prolapse into
the anterior chamber, or to the subsequent formation of thickened bands of vitreous
running backwards from the discission scar to the retina (Sinclair, 1916; Roberts,
1919; Meisner, 1925).
(b) After Intra-capsular Eatraction. Some authors have found that detachment
is more common after intra-capsular than after extra-capsular extraction (Manes,
1932), and others less. Arruga (1934) gives a comparable figure—2% ; Sinclair (1932)
in a series of 257 extractions of senile cataracts met with no instance ; and Knapp
(1936) reported 8 cases in 500 extractions. The incidence of the complication seems
to depend largely on loss of vitreous ; while it is more frequent on the whole than after
extra-capsular extractions, the influence of a subsequent discission is lacking.
(c) After Discission of Soft Cataract. After this operation retinal detachment is
a relatively common occurrence (10.7%, Shapland, 1934). Particularly is this so in
myopic cases, a fact which should contra-indicate this operation in the treatment of
myopia. The following figures are in the literature : 2 cases in 144 (v. Hippel, 1897),
1 in 8 (Hirschberg, 1897), 10 in 50 (Haedicke, 1898), 5 in 60 (Vossius, 1909), 6 in 85
(Otto, 1897), 23 in 253 (Hopner, 1909). (See also Grüner, 1928.)
9. Inflammatory Lesions. (a) Uveitis. A detachment of the retina has
always been recognized as a late sequel of a gross uveitis, as, for example,
sympathetic ophthalmitis (Milles, 1886; Asayama, 1902; Aykai, 1921 ;
Salzmann, 1921; Welhagen, 1926; Pascheff, 1928; and others). In these
cases the detachment is due either to the formation of exudative sub-
retinal fluid in bulk, to traction by cicatricial fibrous tissues, or to shrinkage
and atrophy of the globe, but it is only comparatively recently that a mild
uveitis has been remarked as occurring in frequent association with it.
Syphilis rarely figures in the aetiology (v. Hippel, 1913; Giesler, 1925),
slight lesions of a presumably tuberculous origin are considerably more
common (Dor, 1910; v. Hippel, 1913; Schall, 1922; Lauber, 1923;
Löwenstein, 1926; Heine, 1924–25; Velhagen, 1932; Arruga, 1929–34),
and minor lesions of equatorial and anterior choroiditis due to diverse forms
of inflammatory foci are more common still, while sub-acute inflammation
of the anterior ocular tissues also occurs. It will be seen subsequently that
this factor is probably of extreme importance in the aetiology. A small
patch of anterior choroiditis involves a weakening or even a complete
DISEASES OF THE RETINA 2873
solution of the retina frequently associated with adhesions to the choroid
and the vitreous body in its immediate neighbourhood ; in these conditions
a retinal hole easily results either spontaneously or on the receipt of a minor
trauma. In this connection the exudative detachments at the macula some-
times seen in severe inflammatory affections of the anterior segment of the
eye are of interest (Fig. 2259)."
(b) Other Inflammatory Lesions. An inflammatory lesion which pro-
duces changes in the choroidal circulation leading to the formation of a sub-
retinal exudate, whether it be of the nature of an inflammatory exudate
or determined hydrostatically as a passive oedema, may produce a detach-
ment of the retina. Any eacudative retinitis or choroiditis acts in this way
(see Leber, 1916). In renal retinopathy, for example, detachment occurs in
some 5% of cases (2 out of 43 cases, Schlessinger, 1884; 1 out of 35, Silex,
1895; 4 out of 54, Elschnig, 1904; Ochi, 1921 ; Foster Moore, 1925;
Hanssen, 1929).” In the toxic retinopathy of pregnancy it is relatively
common, frequently occurring bilaterally (v. Graefe, 1855; Hann and Knaggs,
1901; Helbron, 1902; Verderame, 1911; Schiótz, 1919; Hill, 1924; Fry,
1929; and others).” It is noteworthy that the prognosis is better when
associated with pregnancy ; in Foster Moore's series 60% of pregnant cases
recovered spontaneously, and only 15% of the remainder.
Scleritis has several times been noted as a causal factor (Purtscher, 1891 ;
Kamocki, 1892; Wagenmann, 1896); episcleritis (Pichler, 1925); tenonitis (Cramer,
1922); inflammation of the orbit (v. Graefe, 1863; Berlin, 1866; Becker, 1922); or
sinusitis (Barrenechea, 1922).
A massive choroidal exudation associated with general disease has been cited as
the aetiological factor in rare cases of detachment—in malaria (Terson, 1903), in rheu-
matism (Schreiber, 1920), and in gout (Greeff, 1929). In the last case, for example,
the explanation offered is that gouty deposits in the sclera excite an exudative
choroidal reaction. Similarly exposure to strong light 4 has been followed by retinal
oedema and a detachment (Harman and Macdonald, 1922; Rauh, 1927).
10. Hoemorrhagic Lesions. Most frequently hamorrhagic lesions produce detach-
ments as a late result by the contraction of organizing fibrous tissue : such a sequence
is frequently seen in recurrent vitreous haemorrhages. A choroidal haemorrhage,
however, bursting into the sub-retinal space may be responsible for the immediate
production of a detachment. This is a rare occurrence, and usually appears after
trauma (v. Hippel, 1901 ; Orlandei, 1922; Stanka, 1923; Gourfein-Welt, 1923;
Reichling, 1930–32); even slight trauma such as blowing the nose or whooping cough
may be sufficient (Steffan, 1873), and such an accident may occur spontaneously in
purpura (Segi, 1923; ten Doesschate, 1926). Such a detachment may be small, and
may resolve gradually as the blood absorbs, a process which may be followed by the
appearance and disappearance of successive pigment rings limiting the detached area
(Frenkel, 1920; Cassidy and Gifford, 1922). The clinical picture is a pretty one.
11. Embryonic remains, remnants of the hyaloid system running from
the ciliary region or lens to the disc, have seemed to be a causal factor in the
production of a detachment (Rollet and Rosnoblet, 1924; Lauber, 1923).
1 p. 2656. * p. 2717. * p. 2725. 4 p. 2757.
287.4 TEXT-BOOK OF OPHTHALMOLOGY
12. Parasites are rarely responsible for a retinal detachment. A
cysticercus is the most common example." A rare case is described by
Hess (1913) wherein the sub-retinal fluid in a case of irido-cyclitis with
detachment contained a larva of Hipoderma bovis 15 mm. in length.
Considering these various aetiological factors, it is seen that the great
majority of retinal detachments in which the aetiology is not immediately clear
as being due to gross trauma, a tumour, or to inflammatory or eacudative lesions,
are associated with the degenerative conditions of semility and myopia, or with
small infective lesions in the uvea, particularly of peripheral choroiditis, while
minor trauma is by no means an uncommon antecedent. Some statistics will
bear this out. Gonin (1934) gives the following figures in 200 cases of
idiopathic detachment :—
Myopia without senility (myopia over 4 D ; aged under 50) . 24%
Senility without myopia (myopia nil or under 4 D ; aged over 50) 16%
Myopia, with senility (myopia over 4 D ; age over 50) º . 45%
Neither myopia nor senility . & * º º g . 15%
The relative importance of trauma when myopia is not marked is
brought out by Shapland (1934) in his analysis of 425 cases :—
In myopes a history of trauma figured in 15.5%.
In emmetropes a history of trauma figured in 43.9%.
Histological Features
The pathological study of detachment of the retina is difficult since
fresh cases are rarely obtained unless they are associated with a gross
complicating lesion, such as a tumour or a major injury. If the detachment
is relatively uncomplicated, the eye usually remains in situ until such
conditions as uveitis or glaucoma supervene which may necessitate its
removal, and these, of course, render an unbiassed study of the changes
precedent to the detachment impossible ; as an aid to the intimate
pathology of the condition, therefore, these studies are of comparatively
little value. A few early cases, however, have been described : Nordenson
(1887), Gonin (1920), Kümmell (1925–28), Fuchs (1930), Redslob (1931),
Sourdille (1932), Bartels (1932), Kronfeld (1935), and Vogt (1937). Of these
the cases of Kümmell (1928) and Kronfeld (1935) are especially interesting
since the detachments in each were only seven days old at the time of the
examination.
Taken on the whole, these specimens have many points in common.
They may be divided roughly into two categories. Some cases are associated
with advanced vascular disease of the choroid and an albuminous sub-retinal
fluid of an eacudative type (Fuchs, 1930; Redslob, 1931 ; Bartels, 1932);
even after a considerable time has elapsed the retina may show little histo-
logical alteration, while the choroid is characterized by very marked
1 p. 3438.
DISEASES OF THE RETINA 2875
degenerative changes–hyaline degeneration of the chorio-capillaris,
sclerosis of the vessels, and marked pigmentary changes on occasion involving
complete disappearance of the pigment cells
(Verderame, 1911; Ochi, 1921 ; Hanssen,
1929, and others). It would seem obvious
that the detachment in these cases has been
secondary to the accumulation of a sub-
retinal oedema, the source of which is the
choroidal capillaries.
In the majority of cases, however, the
retina alone shows definite changes and the
sub-retinal fluid is of a serous type. The
most characteristic feature is the occurrence
of one or more holes, and associated with
these there are usually areas of cystoid de-
generation or advanced atrophy, situated as a
rule anterior to the equator (Figs, 2479–81).
The atrophic areas may be extremely thin
when the retina may be represented by a layer of glial tissue only, and
frequently advanced pigmentary changes in their neighbourhood indicate
their origin in previous foci of chorio-retinitis.
Another feature, described even in relatively early cases, is a prolifera-
tion of the ciliary epithelium, pigmented as well as non-pigmented, both
FIG. 2479.-RETINAL TEAR.
Seen from the outside (McDonald).
Fig. 2480.-THE EDGE of A RETINAL Hole.
Note extreme cystic degeneration (Lister, Brit. J. O.).


2876 TEXT-BOOK OF OPHTHALMOLOGY
toward the interior of the eye and into the ciliary body (Leber, 1882, 1916;
v. Hippel, 1900–08; Gonin, 1904–23). Sometimes this proliferation is
slight, even in old specimens, showing at most mushroom-like excrescences
(Kuhnt, 1881; Kerschbaumer, 1888); at other times a definite pre-retinal
membrane is found—a cellular membrane of varying thickness, lying on
the anterior surface of the retina and covering it like an endothelium
(Nordenson, 1887; Kümmell, 1925). In a case of three months duration
Gonin (1904) found such an epithelial layer covering the anterior surface of
the detached retina and the posterior surface of the retracted vitreous body;
and in cases of 28 years duration it lined the entire cavity of the globe
behind the retracted vitreous, converting it into a type of serous cavity.
Fig. 2481.-SPINDLE-shaped RENTs IN RETINA,
A cross-section through two spindle-shaped rents; note oedema of outer retinal
layers (x 44) (Kronfeld, A. of O.).
Even when the proliferative changes are small, cells or clumps of cells may
become detached and migrate into the vitreous forming floaters, a change
observed in the early stages (Kümmell (1929)).
It has been contested whether this epithelial proliferation was primary to the
detachment or not. Hanssen (1919) and Vogt (1924) suggested that it is a secondary
phenomenon, and Hanssen (1915) demonstrated that it does not occur invariably ;
but the fact that the presence of considerable proliferation was found in both the
earliest specimens so far examined (7 days, Kümmell, 1929; Kronfeld, 1933) seems to
indicate that this activity can precede the detachment.
Changes in the vitreous body have given rise to a considerable amount of
controversy, partly in attempts to make observations suit one or other
theory. In the most recent specimens there appears to be little or no
histologically discoverable difference between the vitreous in a detachment
and that in any other eye. Definite changes, however, have been described,
such as “fibrillary degeneration " of the vitreous by Leber (1882). He
presumed that these were due to the proliferated epithelial cells from the
ciliary body migrating into the vitreous when they become degenerated,
forming fibrous bands which shrank and disintegrated the solid vitreous gel.
In most cases, however, the vitreous shows some degree of degeneration and
is usually fluid; frequently it is shrunken, and retracted to lie close behind
the lens in the anterior part of its cavity (retraction of the vitreous body)

DISEASES OF THE RETINA 2877
(Fig. 2482). Indeed, Leber (1916) stated that in all cases of detachment
investigated by him, vitreous humour was not present in the posterior part
of the chamber, where it was replaced by serous fluid which sometimes filled
Fig. 2482-FUNNEL-shaped DETACHMENT of THE RETINA.
The retina (r) shows several cysts (k) on its external surface. The vitreous (v) is
retracted behind the lens and is only in contact with the retina in the anterior
periphery, where three horse-shoe tears (d) are present (Gonin).
the entire cavity except its most anterior part ; while Sallmann and Rieger
(1934) claimed to have demonstrated with a modified slit-lamp that anterior
retraction of the vitreous gel occurs in 70% of myopic and 55% of non-
Fig. 2483.-DETACHMENT of THE RETINA.
To show adherence of vitreous (c) to the retina. The vitreous contains
leucocytes carrying blood-pigment (Gonin).
myopic detachments. Adhesions between the surface of the vitreous body
and the retina in the region anterior to the equator have commonly been
observed (Fig. 2483), and torn pieces of the retina have been found on more
than one occasion adherent to the surface of the gel (Samuels, 1939).
Frequently these adhesions coincide with small areas of chorio-retinitis.


2878. TEXT-BOOK OF OPHTHALMOLOGY
The subsequent changes in a case of long standing are characterized
by appearances of atrophy to a varying degree (Samuels, 1939). The
choroid, which is usually originally congested, becomes thin and atrophic ;
the pigment epithelium shows irregularities and tends to proliferate into the
sub-retinal space ; the lamina vitrea becomes studded with drusen ; and
the retina itself undergoes atrophic changes, the most characteristic of which
is the development of widespread cystic degeneration," which may progress
to the formation of cysts of a very considerable size (Fig. 2425). These, as
originally described by Becker (1874) and Lawford (1887), bulge outwards,
originating characteristically in the outer nuclear layer, and leaving the inner
layers of the retina in their normal position. The rods and cones swell
and become degenerate, and eventually no trace of them is found (Speciale-
Cirincione, 1925), and finally all semblance of retinal structure is lost, the
retina undergoing glial transformation and the cyst-walls degenerating into a
nucleated fibrous sheet.
Retinal Holes and Tears
The Frequency of Retinal Holes. The presence of a hole in a detached
retina was noted shortly after ophthalmoscopic examination became possible
(Coccius, 1853; v. Graefe, 1863; Liebreich, 1863), and relatively soon
thereafter de Wecker (1870) drew attention to their striking frequency;
it is interesting that v. Graefe originally thought that the tear was part of
the healing process, until de Wecker advanced the opposite view. Subse-
quent writers repeatedly emphasized this observation ; thus Galezowski
(1883) found them in 20% of cases; Nordenson (1887) in 37%, Horstmann
(1891–98) in 45%. A new importance was attached to them by Leber (1916)
who suggested that in every case of “ spontaneous '' and sudden detachment
of the retina a tear was to be expected, and described them as occurring in
73% of recent cases. In one series of 23 cases of less than 3 months’
standing he found a tear in every one. The dramatic therapeutic results
obtained by Gonin on closing such holes focused attention upon them
anew, and with this stimulus more intensive search for their presence in
recent times has shown that they are much more frequent than even Leber
supposed. Thus Lindner (1931) found them present in 79% of cases,
Jeandelize and Baudot (1930) 12 cases in 14, Clausen (1930) 11 in 12,
Vogt (1930) 23 in 25, Veil and Dollfus (1931) 41 in 51, Weve (1932) 29 in 32,
Arruga (1932) in 90%, and again (1935) in 95% of fresh cases. Gonin in
his original papers (1904) discovered them in 60% of cases, and (1920) in
65%. In his later papers (1928) he obtained an incidence of 87% and
(1930) 85%, giving it as his opinion that they were almost invariably found in
spontaneous detachments if sufficient time and trouble were spent in looking for
them.
1 p. 2810.
PLATE LIx
DETACHMENT or THE RETINA
Fig. 2484.-RETINAL DETACHMENT witH Horse-shoe TEAR.
------ *A*L*
-º-º-
Fig. 2485. MultiPLE Fig. 2486.-Hole with Fig. 2487. - Hole: AT
Holºs IN DEGENERA- OPERCULUM. Focus of CHorto -
Tivº AREA. RETINITIs.
-------
- -º-º-º-
Fig. 2488.-SIMPLE TEAR. Fig. 2489.-Horse-shoe TEAR.
Fig. 2490.-ANTERIoR DIALYsis. - Fig. 2491.-RETINAL STRLE.
[To face p. 2878.






DISEASES OF THE RETINA 2879
Types of Retinal Holes and Tears. Retinal holes present very varying
appearances. In size they may vary from less than 0.1 mm. to enormous
rents measuring a dozen disc diameters and extending over large areas of the
fundus (Cole Marshall, 1939) (Fig. 2492). In shape they vary considerably,
a convenient classification being as follows:–
1. ARRow-HEAD, CRESCENT or HoRs E-shoe-shaped TEARs, the retinal
tissue in the concavity of which usually forms an operculum (Deckel or
lambeau). The convexity is always turned towards the optic nerve, and
Fig. 2492.-GIANT HOLE IN RETINA.
(Cole-Marshall, Brit. J. O.).
the concavity faces the ora. The operculum is usually somewhat retracted
into the vitreous and may occasionally be detached altogether. The
appearance of these rents suggests that they are formed by a tearing process
in a forward direction (Figs. 2484, 2489, Plate LIX).
2. Round Holes with a punched-out appearance, occurring either
singly or in groups which may honeycomb a degenerative fenestrated area
of the retina (Figs. 2485–87, Plate LIX).
3. DALYses at the ora serrata (exceptionally at the optic disc) (Fig.
2490, Plate LIX).
4. Irregular slits and rents (Fig. 2488, Plate LIX).
The relative frequency of these various types of holes, as seen in 210
T.n.—WOL. II. 5 E.

2880 TEXT-BOOK OF OPHTHALMOLOGY
consecutive cases at Moorfields' Hospital reported by Shapland (1932), and
as recorded by Gonin (1934) are as in Table LXVII.
TABLE LXVII.
Shapland Gonin.
(i.) Arrow-head tears & * * * 25% 68%
(ii.) Round holes G g g sº º 34% 22%
(iii.) Dialyses . º e & * º 31% 10%
(iv.) Slits and Irregular g © * tº 10% -
The distribution of the commoner types of holes is of interest. At the
macula round punched-out holes occur ; in the periphery their topographical
FIGs. 2493–95.--THE DISTRIBUTION OF RETINAL Holes (Shapland).
T.
FIG. 2493.−ARRow-HEAD RENTs. FIG. 2494.—ROUND HOLEs. FIG. 2495.-ANTERIOR DIALYSES.
distribution is as in Table LXVIII (Shapland, 1932) (see Figs. 2493–95),
statistics which agree closely with those of others (Gonin, 1920–28 ; Arruga
1932).
TABLE LXVIII
Arrow-head Round Dialysis
Supero-temporally . sº 64% 52.2% 8.8%
Infero-temporally g * e 2% 30.4% 77.9%
Infero-nasally . w tº gº 6% 15.9% 7.4%
Supero-nasally . & * º 28% 1.5% 5.9%

DISEASES OF THE RETINA 2881
The round hole, therefore (in addition to its occurrence at the macula),
occurs chiefly in the temporal half of the globe, its site of election being the
supero-temporal quadrant, but it has no very preponderant site of predilec-
tion ; the dialysis occurs almost exclusively in the lower part of the globe ;
while the arrow-head tear has its main incidence in the upper half of the
globe, in all cases the site of election being on the temporal side. It is
important also that round holes, while most numerous anterior to the
equator, may occur anywhere, even at the macula, while arrow-head rents
have a decided predilection for a relatively narrow zone near the periphery;
thus in 100 such tears Gonin (1934) found 3 at 1 disc diameter from the ora,
9 at 2, 19 at 3, 25 at 4, 23 at 5, 12 at 6, 5 at 7, and only 4 at a less distance
from the posterior pole than the periphery.
The incidence in varying refractive conditions of these different types of
holes is of considerable interest. The figures in Table LXIX is taken from
Shapland's statistics (1932) of the same 200 cases seen at Moorfields’
Hospital.
TABLE LXIX
& f s Emmetropes and
High Myopes | Low Myopes Hypermetropes
(1) Arrow-head tears . & e 38.2% 19.6% 15.1%
(2) Round holes * * º 43.4% 43.2% 17.8%
(3) Dialyses º tº e * 9.2% 23.5% 58.9%
(4) Slit and irregular . * & 9.2% 13.7% 8.2%
It is seen that while the slit-like and irregular rents show a fairly equal
distribution throughout the three groups, the round hole, on the other hand,
is found mainly in myopia, and is distributed approximately equally between
the low and high degrees of error. The arrow-head rent also shows its main
incidence in myopic eyes, but it has a decided preference for the higher
degrees. The peripheral dialysis, on the other hand, occurs chiefly in the
emmetropic and hypermetropic eye and is relatively rare in high myopia.
Multiplicity of Holes. In the majority of detachments one hole is
present, but this is by no means invariable : Gonin (1928) has counted as
many as 20 in 1 quadrant. In 53 cases of his, 39 had 1 hole, 8 had 2, 4 had 3,
and 2 more than 4. In 55 cases of Lindner's (1931) 31 had 1, 13 had 2, 2
had 3, and 9 had more than 3 holes.
Macular Holes
Macular holes, which may be either traumatic or degenerative in nature,
have already been fully discussed " (Figs. 2368–70, Plate LV.). They are
1 p. 2755.
3 F. 2
2882 TEXT-BOOK OF OPHTHALMOLOGY
always round with a punched-out appearance and may be single or multiple,
and differ from those found in the periphery in that they are by no means
invariably associated with retinal detachments; thus in his series of 23
cases Middleton (1919) found only one detachment. Some authors, indeed,
consider the association very rare (Haab, 1900; Lister, 1924); but a large
number of reports of detachments in the presence of macular holes are to
be found in the literature. It is of importance, however, that while the
detachment associated with a peripheral hole is usually large and extensive, that
associated with a macular hole is typically limited and non-progressive.
Peripheral Holes and Tears
1. Round or Oval Holes. These form the majority of retinal holes, and
may occur singly or in clumps: thus Vogt (1929) described chains of 6 or 8,
and Gonin (1934) an archipelago of 13 in one quadrant. They are round or
oval in shape, usually with a raised sharp edge, giving them a crateriform
appearance at the bottom of which the red trellis-work of the choroid is
Fig. 2496.-DETAchMENT or RETINA.
Adherence of retina to choroid (Lister, Brit. J. O.).
visible. Occasionally they are bridged across by a vessel apparently
unaltered (Nordenson, 1887; Elschnig, 1892; Dunn, 1896; Doyne, 1902;
Lister, 1924; Arruga, 1934; and others). Around them are frequently
noted pigmentary changes and evidence of old degeneration or inflammation,
while histological examination shows that they can develop from conditions
of retinal atrophy and cystic degeneration (Nordenson, 1887; Hanssen,
1919–25; Lister, 1924; Gonin, 1934). Elschnig (1914)recorded a case where

DISEASES OF THE RETINA 2883
first a patch of chorio-retinitis was seen, and at a later examination a
corresponding hole was found; while Arruga (1931) studied the gradual
evolution of a hole in a case of tuberculous
retino-choroiditis (Fig. 2487, Plate LIX).
Similarly, Zeeman and Oltmanns (1935)
observed the development of a large hole
by the confluence of smaller ones after
the formation of oedema subsequent to
trauma. Sometimes the degenerated
piece of retina remains attached to
the choroid (Collins, 1900) (Fig. 2496);
while at other times it becomes adherent
to and is withdrawn into the vitreous
(Fig. 2497). The evidence, therefore,
points to the fact that the greater number
of such holes is due to the actual destruction
of retinal tissue by peripheral retino-choroidal
inflammation or degeneration. A single
round hole points to a localized focus of
inflammation or cystic degeneration and a
fenestrated atrophic retinal area owes its
origin to a more widespread lesion.
Fig. 2497.-RETINAL TEAR.
The detached fragment is adherent
to the vitreous (Sourdille).
2. Arrow-head and Horse-shoe Shaped Rents. As has been noted this
Fig. 2498.-RETINAL TEAR.
The tear (d) is partly spanned by an operculum (l) to which the vitreous is
adherent Gonin).


2884 TEXT-BOOK OF OPHTHALMOLOGY
type of tear is found in many different forms with a general semi-lunar
configuration, the concavity being directed away from the disc, and the
retina in the concavity (the operculum) frequently being drawn in towards
the vitreous, or sometimes being pulled in and detached altogether (Fig. 2486).
The appearance indicates that this piece of tissue can be firmly attached to
and drawn inwards by the vitreous body (v. Rötth, 1933); that this is the
case has been demonstrated histologically (Lister, 1924) (Fig. 2497), and
clinically the operculum has frequently been seen to move about with
movements of the eyes. It may be more extensive than the actual tear, in
which case it is also thinner, the superficial layers only of the retina being
torn off (Fig. 2498). From the clinical and anatomical appearances the
conclusion seems inevitable that these holes result from the traction of an
adherent vitreous on a weakened area of the retina. It will be noted that
in 92% of cases this type of tear occurs in the upper half of the globe where
the mobility of the vitreous will be most effective by its shaking movements,
and that it has a site of predilection in a relatively narrow zone near the
periphery where the retina has little support from the nerve fibre layer and
is therefore easily torn, and where the base of the vitreous body is particu-
larly adherent to it.” This type of tear in the retina is therefore more
accurately described as a rent rather than a hole.
3. Dialyses. Anterior dialyses represent a tearing away of the retina
at its relatively thin attachment at the ora serrata (Fig. 2490, Plate LIX).
Since their position so far forward makes detection difficult unless specially
looked for, their relatively frequent occurrence escaped attention until
comparatively recent times. They vary much in size and may be single or
multiple, running round the periphery of the fundus festoon-fashion.
Occasionally they are of an enormous size extending half-way round the
circumference (Jeandelize, Baudot and Delaveuve, 1931 ; Goldenburg,
1933), and if they affect the upper half of the circumference the unusual
picture may be presented of the upper half of the retina falling down upon
the lower so that the entire outer surface of the detached retina is seen
through the pupil below and the bare choroid above (REFLECTION OF THE
RETINA) (Constentin, 1904; Lawson, 1917; Ballantyne, 1931). It will be
noted later that this condition gives rise to an interesting type of diplopia.”
The most extreme degree possible has been reported, in which after an injury which
left no corneal scar, the entire retina was separated at the Ora and lay as a shrunken
mass resembling a tumour on the optic disc (Velhagen, 1900) (Fig. 2499); while Römer
(1901) reported a case of traumatic rupture of the globe with a complete anterior
dialysis where the retina was represented by a cord of tissue running towards the
scleral wound, while the pigment epithelium was exposed lying intact on the choroid.
Sometimes in localized dialyses the retina is drawn considerably inwards at the
dialysis as if it were retraced by a shrinking vitreous: thus in a case of Scheffels’ (1891)
the external surface of the retina was exposed to the observer's view through the pupil.
* Vol. I, p. 111. p. 2901.
DISEASES OF THE RETINA 2.885
As a rule dialyses occur in emmetropic young adults and are situated
generally in the infero-temporal quadrant, that is, in the least developed
and most stretched zone of the retinal periphery those most inferiorly
situated are frequently associated with a detachment which tends to be
relatively non-progressive. It is important that these sometimes account
for the delayed development of a detachment some time after trauma
(Cramer, 1905.)
The great majority of peripheral dialyses and practically all cases of
grossly irregular rents are associated with well-defined trauma, whether it
be a perforating wound (Collins, 1917), a slight contusion (Scheffels, 1891;
Knapp, 1931), or an indirect injury such as a fall
(Müller, 1931; Haitz, 1931); we have seen,
moreover, that these occur mainly in young
emmetropes. It would seem, therefore, that this
appearance is usually the result of gross trauma
in a relatively healthy eye. When no other weak
spot exists, and if an injury is sufficiently severe,
the retina gives way at the point where it is
thinnest and most prone to degeneration, where it
is unsupported by vessels, where the vitreous
framework has its fullest development and its
firmest anchorage, and where the mode of appo-
sition of the two layers of the optic vesicle Fig. 2499– Co M P L E t E
suddenly alters, the loose relationship between ſºº DIALysis of
the retina and the pigment epithelium giving The rolled-up retina re-
place abruptly to the firm fusion of the two layers º "º".
of ciliary epithelium. Genth).
It is not to be imagined, of course, that
all these cases are traumatic ; that even unrecognized small traumata
cannot be arraigned is evidenced by the not infrequent occurrence of
symmetrical “spontaneous” bilateral dialyses involving bilateral detach-
ments, which have been described as a clinical entity occurring in
young people, especially males (Shapland, 1932). It is probable that
many of these may be due to a developmental anomaly, perhaps with
cystic formation (Weve, 1935; Ridley, 1935; Schmelzer, 1936; Fuchs,
1937; Kornzweig, 1940; and others). Velhagen (1932) has suggested
that many of these cases may be due to small recurrent haemorrhages,
which in young adults are probably usually tuberculous in origin. Finally,
a type of dialysis is not an infrequent accompaniment of advanced cyclitis "
in which shrinking cyclitic exudates after organization may retract the iris,
draw the ciliary processes inwards, and detach the retina, tearing it away
close to its anterior limit (Fig. 1811). A similar phenomenon is seen as
the result of the retraction of scar tissue after a perforating injury "
Vol. I, p. 332. * p. 2172. 3 Vol. IV.

2886 TEXT-BOOK OF OPHTHALMOLOGY
(Lawson, 1917; Poos, 1928; Martin, 1929; and others); while a secondary
dialysis has been reported on several occasions as the result of tension on
the retina from a previous operation of ignipuncture for detachment
(Shapland, 1931; Anderson, 1932).
A unique case was described by Bartels (1933) wherein the retina was split into
two leaves along the internuclear layer, the internal layer being detached from the
ora and appearing as a tear, while the external layer formed a fine membrane flooring
this and itself showed very fine tears.
A posterior dialysis, wherein the retina is torn away at the optic dise is
an extremely rare result of
gross trauma (Fig. 2500) (Paul,
1905; Lister, 1924).
The Healing of Retinal
Holes. The retina shares the
tendency of all tissues to spon-
taneous healing only to a very
small degree, but it may be that
in exceptional cases retinal holes
do heal (Deutschmann, 1929).
Samuels (1939) considered that
the phenomenon was probably
due to glial proliferation, per-
haps incited by the irritation
caused by a haemorrhage. Such
healing has been seen clinically
(Brons, 1924; Vogt, 1929) and
Fig. 2500–CoMPLETE Posterior Dialysis of experimentally (Dejean, 1934;
RETINA : Total Detachment.
(Lister, Brit. J. O.). Hagedoorn, 1934). and has
been held to explain the fact,
noted by several observers, that holes are more rarely found in old detach-
ments than in recent ones (Leber, 1916; Vogt, 1930; Sourdille, 1931;
Gonin, 1934; and others). The general tendency, however, is probably
in the opposite sense; the retina has a greater specific gravity than the
vitreous gel, and its movement in the vitreous together, in many cases,
with a continuance of the traction which initiated the tear, tends rather to
perpetuate it when once formed and to increase its size.
The Ocular Tension in Detachment
The tension of the eye is not characteristic in detachment of the retina,
but as a rule if the detachment is uncomplicated, it remains normal for some
time and then falls below normal (Leber, 1916).
Thus Nordenson (1887) in 62 recent cases found the tension always normal, and
in 58 old cases always sub-normal, with 4 exceptions in which glaucoma developed. In

DISEASES OF THE RETINA 2887
Horstmann’s (1891) series (106 cases) 60 showed a normal tension and 46 a sub-normal;
in 53 cases Lauber (1908) found hypotension in 48 ; in 345 cases Deutschmann (1910)
found the tension lowered in 13.3% only ; in 16 recent cases Kümmell (1920) found a
lowered tension each time ; in a subsequent 52 cases he found the tension in the
affected eye less than the other in 45, equal in 5, and raised in 2; and in a series of
247 cases Thomas (1925) found raised tension only once. In recent detachments up
to 7 days Kleiner (1933) found hypotony in 27%, in detachments of from 3 to 8 weeks
duration in 54%, and in detachments of more than a year's standing in 66.6%.
It is probably the case that in the presence of a hole the tension tends to fall,
but such a fall is usually slight. The phenomenon may well be due to the greater
ease with which fluid leaves the eye on the rupture of the continuity of the membrane
surrounding its cavity.” Thus Arruga (1934) has shown in the rabbit that if Indian
ink is injected into the vitreous gel, whereas normally it finds an exit mainly by the
anterior drainage channels at the angle of the iris,” this route is retained in the case of
a detachment without a tear, but if a tear is present in the retina, the major part of the
injected matter passes into the sub-retinal space. It appears, therefore, that in these
cases the normal outflow of intra-ocular fluid is considerably disorganized, the influence
of the hole being similar to that of the operation of cyclodialysis.
An acute hypotony may occur, often with the early development of
symptoms of irido-cyclitic irritation, the eye becoming softened, smaller
and injected. Such a sudden fall was first described by Schnabel (1876),
who thought it was due to the bursting of a myopic staphyloma, and Leber
(1916) established it as a clinical entity. This may on some occasions be
due to the sudden exit of vitreous fluid through a hole (Beigelman, 1929),
but an alternative cause is the development of a uveitis. It is possible
that this may be due to irritant substances which are liberated associated
with the detachment, and the same cause may eventually produce a secon-
dary glaucoma of the inflammatory type, a reversal of circumstances which
has been recorded as resulting in a diminution of the detachment (Sedan,
1928).
The association of normal or sub-normal tension with detachment is
not, however, invariable. When the detachment is complicated by a
tumour the tension is usually raised, a circumstance which may also occur
in the presence of haemorrhages (Bergmeister, 1919), sub-retinal exudate
(Raeder, 1925), or a plastic irido-cyclitis (Morax, 1920; Maggiore, 1923).
The Sub-Retinal Fluid
Considered grossly as it is seen in pathological specimens the sub-
retinal fluid in recent cases of detachment varies according as to whether or
not a hole is present ; in general terms, if a hole is present it is relatively
clear and non-coagulable resembling vitreous fluid, but with a higher
content of albumen and a variable chloride content ; if no hole is present
it is highly coagulable and appears as a firm jelly-like mass, grey and
albuminous, or yellow or even brown, depending on the presence of
inflammatory or haemorrhagic elements (Nordenson, 1887; Ginsberg
p. 3356. * Vol. I, p. 454 (458).
2888 TEXT-BOOK OF OPHTHALMOLOGY
and Simon, 1898; Lister, 1924; Gonin, 1934 ; and others). Where
preceding inflammatory changes have been marked the albumen content
has been high (Lauber, 1908; Kümmell, 1913; Baurmann, 1929). In the
first case the obvious deduction is that vitreous fluid has percolated through
the retinal hole ; in the second case the sub-retinal fluid is an exudation
from the choroid. In the first case as the age of the detachment increases
the albumen content usually increases; it is as if the sub-retinal fluid when
a hole is present is at first practically unchanged vitreous fluid, which after-
wards becomes altered owing to its irritative reaction causing a vaso-
dilatation in the choroid (Arruga, 1933); while eventually in the case of
very old detachments the albumen may diminish pari passu with the develop-
ment of choroidal atrophy. At this stage, while before it showed turbidity,
the sub-retinal fluid becomes optically empty, and the image of an
ophthalmoscopic source of light can be readily seen on the pigment epithelium
(Friedenwald, 1927–29). In very old standing detachments it becomes
laden with cellular elements of disintegration, epithelial débris, and even
crystals of cholesterol (Gonin, 1934). The irritative action of a fluid of this
nature on the uveal tract can be demonstrated experimentally, for on the
injection of sub-retinal fluid into an eye, an iritis results (Fuchs, 1920), and
it is probably responsible for the mild uveitis which frequently accompanies
the condition.
Several authors have examined this fluid chemically, and, as is to be expected,
their results vary within very wide limits. The albumen content has been estimated
by Raehlmann (1893) at 9%, Löwenstein (1911–26), Birch-Hirschfeld (1912) at 2.6%,
Magitot and Lemoir (1932) at from 0-135 to 60% in an old case, Baurmann (1929) at
from 1.2% to 11.2%, Jasinski (1933) at from 0.7% to 5.8%, and Arruga (1933) at
from 0.2% to 8.1%, and in a case of 2 years’ standing at 0.09%. It is thus always
higher than that of the vitreous fluid (Magitot, 1934). The sugar content has been
found to show no great difference from that of the vitreous body, but not to resemble
it closely (Magitot and Lemoir, 1932; Arruga, 1934; Magitot, 1934) and the chlorides
may be more or less than in the blood (Magitot, 1934). Ferments, such as amylases,
are also present (Weve and Fischer, 1936). The density is low in recent cases, rising
as the age of the detachment progresses, being always, however, greater than that of
the vitreous gel (Arruga, 1933; Visser-Heerema, 1936). The osmotic pressure, the
electrical resistance, and the viscosity have been estimated by Jasinski (1933).
The fact that the sub-retinal fluid contains more albumen, an unequal sugar
content, and a different chloride content has led Magitot (1934) to deny a vitreous
origin ; Koyanagi (1928–34) suggested that in certain cases, particularly exudative
detachments, it was secreted by the pigmentary epithelium.
Eacperimental Detachment
Although a considerable amount of experimental work has been done
since the first attempts by Chodin in 1875, it cannot yet be said that
permanent detachments can be produced in animals’ eyes which simulate
all types met with clinically. The reason is probably that detachments
essentially occur in diseased eyes—a clinical fact to which we have already
DISEASES OF THE RETINA 2889
drawn attention. Such as they are, however, these experimental attempts
are not without their clinical interest and value.
Such experiments are most easily studied in groups. An eacudative type of
detachment is relatively easily produced in rabbits’ eyes. Any traumatic or noxious
influence which induces an exudative choroiditis will produce a detachment of the
retina, the result depending not so much on the nature of the stimulus as on the
intensity of its effects and the pathological changes it sets up. Such a condition has
been produced by sub-conjunctival injections (phloridzin, Best, 1906; zinc chloride,
Castresana, 1912); episcleral injections (iodine, Weekers, 1925); by injections
between the sclera and the choroid (cantharides, Boucheron, 1884; Simi, 1884); by
a stream of hot air (Spinelli, 1933); or more commonly by cauterization of the sclera
(the steam cautery, Wessely, 1904; the galvanocautery, Weekers, 1925; Tillé, 1932;
diathermy, Dollfus and Veil, 1932). With a small degree of trauma an oedema
of the choroid and retina results ; more intense trauma involves destruction of the
choroid and retina, perhaps with the production of retinal tears and the out-pouring
of an albuminous sub-retinal exudate. In the slighter cases the detachment is
transitory ; in the more severe cases eventual re-attachment is the rule, even in a
high percentage of cases where a retinal tear is produced, unless gross damage with
haemorrhage and marked vitreous changes are present. A similar effect may result
from the stasis and Oedema produced by ligating the optic nerve (Weiss and Evans,
1934).
A second type of detachment can be produced by the injection of chemical substances
into the globe or of foreign bodies; this type also has its clinical counterpart. Such
experiments have been done on rabbits by Raehlmann (1876) and Sugita (1937) using
salt, by Bach (1895) using salt and sublimate, by Schreiber and Wengler (1909) using
electrolytic iron, and by Leber (1882) using metallic particles. -
A third group of experiments involves the withdrawal of vitreous, either through
scleral incisions (Chodin, 1875), or through a cannula (Birch-Hirschfeld and Inouye,
1909; Hagedoorn, 1930; Lobeck, 1932; Arruga, 1933; Dejean, 1934). The results
of these experiments are fairly consistent ; a detachment is relatively easily produced
in this manner ; if no hole is formed re-attachment is rapid, and if a hole is formed
the tendency to spontaneous re-attachment still persists in a number of cases.
In a final group of experiments the retina has been detached mechanically by the
insertion of a spatula underneath it, but again re-insertion frequently occurred
regardless of the presence of a retinal hole (Castresana, 1912; Mayer, 1931 ; Arruga,
1933). A further step of considerable importance was taken by Castroviejo (1934),
who withdrew some of the vitreous gel into a syringe, detached the retina with a
spatula leaving a retinal hole, and re-injected the vitreous into the sub-retinal space.
In previous experiments the sub-retinal fluid was exudative and absorbable, but in
these last up to the time of reporting (4 months) no tendency towards spontaneous
cure was apparent. These last correspond more closely than any others to the non-
exudative type of detachment wherein a hole is present and vitreous fluid lies in the
sub-retinal space.
It is interesting that in experimental detachments which persist, pathological
changes occur similar to those described clinically ; these have been extensively
studied and figured by Arruga (1933). The retina undergoes changes of a cystic and
degenerative type and eventually atrophies, while the choroid also shows atrophic
changes.
AEtiological Theories
Having now collected the aetiological and pathological facts relative to
detachment of the retina, we are in a position to consider the theories put
2890 TEXT-BOOK OF OPHTHALMOLOGY
forward to account for its occurrence. For the last 70 years ophthalmologists
have expended their ingenuity in devising new theories to explain this
accident, and the diversity of these which have been propounded and
accepted merely serves to indicate the difficulty and complexity of the
problem. Custom has divided these theories into four categories :
Distension, Exudation, Traction and Hypotony.
I. THE THEORY OF DISTENSION. That stretching of the owter coats of the eye is
the cause of detachment of the retina.
That stretching of the sclera and choroid was the cause of retinal detachment
was first suggested by v. Graefe (1857) because of the high incidence of detachments
in myopes; he believed that the retina was not so extensible as the two outer coats,
and that in the elongation of the eye in axial myopia, it became separated, assuming
the chord of the arc which it cannot describe. Schweigger (1889) elaborated the
hypothesis maintaining that vitreous fluid leaked into the sub-retinal space through
holes in the retina, Axenfeld (1903) accepted the theory and applied it to conditions of
buphthalmos, and more recent writers have applied it to particular cases (Hanssen,
1919; Fuchs, 1937). It is to be remembered that as a sphere enlarges the surface
increases proportionately to the square of the radius and the volume proportionately
to the third power of the radius; a relatively small increase in the axis, therefore,
involves a considerable distension of the sclera (which can occur partly by stretching
and partly by thinning), of the choroid (which occurs by thinning and atrophy), and
also of the retina, the response of which is limited owing to its thinness and delicacy,
which, therefore, failing to keep pace with the others (it is said) tends to slip away
from them. In addition, the normal support of the vitreous is lacking (Iwanoff, 1869),
for the volume occupied by it is increased in a higher proportion, and, since the gel
itself cannot be augmented," its place is taken partly by an accumulation of intra-ocular
fluid, a process which tends to instability.
That distension per se, however, is the cause of detachments is very unlikely.
As Leber (1916) pointed out, that the retina is as extensible as the choroid, perhaps
more so, is seen in the fact that it usually remains intact when a contusion produces
a choroidal tear, and in the extreme rarity of detachment in posterior staphylomata.
Moreover, the obvious deduction that in extreme myopia detachment should be the
rule does not hold good. Further, while most detachments are anteriorly situated,
the myopic eye suffers its greatest elongation behind the equator. Finally, both
in myopia and in buphthalmos detachments usually occur long after the period of
active elongation has past. Indeed, histological evidence points to the fact that in
advanced degrees of myopic atrophy detachment tends to be prevented by the frequent
presence of adhesions between the degenerated retina and the atrophic choroid (Gilbert,
1920; Fuchs, 1920; Dor, 1921 ; Lauber, 1923). It seems clear that there is no such
thing as a specific “myopic detachment ’’; myopia acts only as a predisposing factor,
and, as we shall see, the predisposition lies in the associated myopic degeneration and not
in the distension. It is probable that the only occasion when distension acts as the
main aetiological factor is in cases of violent and sudden distension due to trauma.
II. THE THEORY OF ExUDATION. That the retina is pushed up by an eacudate from
wnderneath.
This was the early pre-ophthalmoscopic view taken of the cause of detachment—
that it was a “sub-choroidal hydrops '' (Wardrop, 1818; Sichel, 1841; Desmarres,
1847), and as a considered theory it is associated with the names of Arlt (1853) and
* Vol. I, p. 462 (467).
DISEASES OF THE RETINA 2891
v. Graefe (1854), who considered the primary lesion in non-myopic “spontaneous ”
detachments to be either a serous choroiditis or a choroidal haemorrhage. There is no
question that a large group of detachments is due to exudation from diseased or
damaged choroidal capillaries (albuminuric retinopathy, choroidal tumour, etc.).
Frank (1920) reported a very interesting case wherein he watched a detachment appear
and disappear in angiomatosis, a phenomenon certainly due to a choroidal vascular
disturbance which was temporarily compensated. That such a mechanism, however,
can account for the average case of “spontaneous ” serous detachment is negatived
by the absence of an exudative sub-retinal fluid, and of any sign of choroidal change
such as would explain a massive and rapid transudation.
III. THE THEORY OF TRACTION. That the retina is pulled inwards, and usually
torn in the process.
That many cases of retinal detachment are due to traction is obvious from
histological preparations of eyes with contracting fibrous bands in the vitreous chamber,
whether these be derived from the reaction to foreign bodies, or gross inflammatory
or haemorrhagic disturbances. That the same process in a more subtle form is the
activating agent in most cases of detachment is a view which has obtained very wide
acceptance. Two factors have been suggested—the retraction of the vitreous itself,
and the contraction of the inflammatory membranes of a pre-retinitis.
(a) The theory of vitreous retraction was originally put forward by Müller (1858);
reasoning from the analogy of the gross findings in cases of intra-ocular inflammation
and haemorrhage, he concluded that the anterior retraction of the vitreous body seen
histologically would act in the same way. The idea was more fully elaborated by
Leber (1882), who concluded, from experimental work wherein he introduced metallic
particles into the gel, that detachments with large retinal holes were formed by
retraction of the vitreous body. In clinical cases the common occurrence and
appearance of retinal tears led him to suggest that their formation was the essential
mechanism, that they were formed by shrinkage of a vitreous body undergoing
‘‘fibrillary degeneration,” and that the detachment was consummated by free fluid
passing through the tear and raising up the retina. Unlike some others who followed
him, he postulated no well-defined cords or membranes, but structures themselves so
delicate as to escape observation, the existence of which was deducible only through
their consequences. He demonstrated histologically a fine fibrillary change in the
vitreous which adhered to the retina, in which finding he was supported by Nordenson
(1887), Deutschmann (1895–99), Addario (1904), and Gonin (1904). Apart from these
authors, the theory attracted little support until it was again warmly advocated by
Gonin (1923), who, however, transferred the primary lesion to the retina itself; the
primary lesion was a pre-equatorial chorio-retinitis or atrophy, adhesions between
the retina and the vitreous resulted, and retraction of the vitreous caused a retinal tear,
the adherent atrophic region readily giving way. -
(b) At a later date Leber (1908) modified his views. The vitreous itself he
considered to play a passive rôle ; the active agent was the proliferation of new tissue
from the ciliary epithelium which is sometimes very evident histologically,” and
spreads out into the vitreous body and over the retina, forming membranes and fibrous
bands which adhered to the retina and underwent contraction and shrinkage, causing
tears and detachment. These were assumed to be the result of slight inflammatory
changes in the pars plana of the ciliary body. The whole picture he called pre-retinitis,
and he claimed that it produced a condition of “primary retinal contraction ‘’—a
wrinkling and tearing of the retina due to a hyalitis—in contra-distinction to “secondary
contraction '' due to the formation of obvious connective tissue strands in the vitreous
p. 2876.
2892 TEXT-BOOK OF OPHTHALMOLOGY
in cases of advanced cyclitis and phthisis bulbi. It has been contended that these
appearances are secondary to the detachment and therefore cannot be causal (Hanssen,
1919; Vogt, 1924), but their presence in very early specimens proves that they may
be precedent (Kümmell, 1929)."
The theory of retraction has not been without its opponents, and many objections
have been raised against it. The impossibility of seeing ophthalmoscopically any
vitreous change in the region of most holes, and the difficulty of imagining that the
vitreous body, by its own nature, was capable of forming bands have made many
writers, both early and late, sceptical (Schweigger, 1889; Raehlmann, 1893; Greeff,
1905; Lauber, 1908; Vogt, 1923–37; Koby, 1925; Wessely, 1930, and others).
Similarly, Hanssen (1915), in the histological examination of both eyes of a patient
with “spontaneous '' detachments, failed to find any evidence either of vitreous bands
or cellular membranes or ciliary hyperplasia. At the same time it has been argued
that in recent detachments structures so delicate may be readily hidden by opacities
(Fuchs, 1920); while, on the other hand, fine vitreous bands such as might have caused
a detachment have been observed clinically with the slit-lamp by Comberg (1924),
and, in one case, running to the summit of a detachment after a needling operation
(Meisner, 1925). Finally, that very considerable traction need not necessarily cause
a detachment was maintained by Fuchs (1921), who described a series of eyes which
showed little or no tendency to detachment, although contracting retinal scars and
exudates and cyclitic membranes had distorted the retina considerably.
IV. THE THEORY OF HYPOTONY. That lowering of the tension in the vitreous cavity
produces a detachment by withdrawal of the support which normally keeps the retina in
position.
That a retinal detachment could be due to a sudden hypotony was proposed
initially by Stellwag (1861), and since his time many theories have been suggested
modifying in various respects the mechanism of the fall in tension. It will be recalled
that the easiest way to produce a detachment experimentally is by withdrawal of
vitreous, and that hypotony is a frequent accompaniment of detachment. That
this hypotony was the primary factor was maintained by Lauber (1908) and Kümmell
(1921): they concluded that it was due to a mild uveitis which also caused a vitreous
degeneration, both of these producing a compensatory ea vacuo capillary dilatation in
and transudation from the choroid, a sequence which accounted for the detachment.
The basis of the sequence of events, therefore, is a slow affection of the uvea ; the
subsequent shrinkage of the vitreous body lowers the pressure in the chamber of the
eye which tends to suck the retina away from the choroid, as well as to draw in the iris-
lens diaphragm and increase the depth of the anterior chamber ; the negative pressure
also dilates the already damaged uveal vessels and causes a transudation from them
which, acting in the opposite sense, also aids in causing the detachment by forcing the
retina up from underneath. Fuchs (1923), with somewhat similar reasoning, considered
a myopic detachment secondary to low tension and a detached vitreous body, and
Redslob (1932) suggested that an acidosis, by altering the reaction of the vitreous gel,”
might cause it to shrink and so initiate the same train of events. The hydrostatic
theory was put into another form by Baurmann (1929), whose views have been endorsed
by Deutschmann (1932) and Stein (1932): on the state of the capillaries of the “pre-
retina ’’ (the ciliary body) depends the tension in the chamber of the eye, and on the
state of the capillaries of the “retro-retina ’’ (the choroid) depends the tension in the
sub-retinal space. Normally the tension in the capillaries of the pre-retina is greater
and the sub-retinal space remains a potential cleft, but if this difference was reversed,
a transudation would occur into the sub-retinal space upsetting the osmotic-hydrostatic
1 p. 2876. * Vol. I, p. 464 (470).
DISEASES OF THE RETINA 2893
equilibrium, and a detachment be produced, a result which could be attained either by a
hypotony in the chamber of the eye, or a rise of the pressure in the choroidal capillaries.
The first condition could be produced by a uveitis; the second by a choroiditis or a
degeneration of a considerable area of the chorio-capillaris so that the remaining
capillaries became necessarily over-engorged.
Most are agreed that low tension alone is insufficient to produce a detachment
unless it occurs suddenly and dramatically, as it occurs experimentally on withdrawal
of vitreous, and as it may occur, for example, after gross vitreous loss in a cataract
extraction (Baurmann, 1929): even then detachment of the choroid is the more
common.” For a retinal detachment to occur, the transudation of fluid must be
facilitated by some vascular affection in the uveal tract. Thus Wessely (1922) found
experimentally that while a controlled loss of vitreous alone did not produce a detach-
ment, it would do so if preceded by a lesion such as a scald of the sclera which produced
a choroidal vaso-dilatation, and most writers agree on clinical grounds that some
capillary upset must be associated with the hypotony, a mild uveitis usually being
blamed (Vail, 1920; Weekers, 1925; de Decker, 1929; Beigelman, 1929; and
others).
On the basis of the fact that a concentrated salt solution injected into the vitreous
body produces a detachment experimentally, Raehlmann (1893) suggested the some-
what far-fetched idea that abnormal diffusion from an altered vitreous would excite a
choroidal exudation, thus causing a detachment, and that, once excited, the proteins
in the sub-retinal fluid would attract osmotically more vitreous fluid into the sub-
retinal space, thus extending the detachment, until the pressure was equalized by the
retina giving way in the formation of a tear.
Sourdille (1923–31) put forward the original theory—based on little evidence—
that detachments are due primarily to the pigment epithelium, the pigmentary pro-
cesses of which fail to interlink between the rods and cones. The commonest cause of
this is a peripheral choroiditis ; and choroidal transudation or exudation and retinal
holes are secondary phenomena. The same factor, the loss of the physiological union
provided by the pigment epithelium has been stressed by Marquez (1933), Rollet
(1934), and Giannini (1934). On the other hand, Magitot, (1934) suggested a primary
retinal dyserasia of the nature of syringomyelia or syringobulbia as the primary lesion.
The Significance of Retinal Holes
The last two groups of theories raise acutely the question of the significance of
retinal holes; in the theory of vitreous retraction, the tearing of the retina, which
allows the fluid vitreous to run behind this membrane, is the pivotal incident ; in the
theory of hypotony, hole formation is secondary and incidental. That holes can
develop in an already detached retina is true ; such an incident has been actually
observed by Elschnig (1914) and Deutschmann (1933). On the other hand, the
histological investigations of Kümmell (1929) showed that in a recent detachment the
retinal hole was of precedent date. Although the majority of observers maintain
that the causal factor in many detachments is the formation of a hole or tear, a view
which seems substantiated by the undoubted therapeutic effect of a closure of the hole,
unanimity on the question is by no means complete (Pascheff, 1935; Rubbrecht,
1935, and others). All are agreed, however, that in the presence of a hole spontaneous
re-attachment is rare (Lister, 1924), and that retinae which do attach themselves
spontaneously show no tear (Birch-Hirschfeld, 1912). Holes have been observed to
heal, whereupon the detachment has gone back (Brons, 1924; Vogt, 1928). On the
1 p. 2538.
2894 TEXT-BOOK OF OPHTHALMOLOGY
other hand, it has been shown, experimentally and clinically, that holes may heal
and the detachment persist (Deutschmann, 1929), while Dejean (1934) and
Hagedoorn (1934) found in rabbits that the formation of a retinal hole did not
necessarily lead to a detachment, and that in the process of the production of an
experimental detachment the fragility of the retina made holes develop readily—they
were an incident, not a cause. It has been suggested that they are formed at the time
of the detachment by rupture owing to the difference in hydrostatic pressure on either
side of the retina—the hypotony on the vitreous side and the pressure of the choroidal
exudation on the other side (Kümmel, 1921 ; Weekers, 1925; Baurmann, 1929);
or by osmotic forces causing an increase of pressure in the sub-retinal fluid, since the
high osmotic pressure of this protein-rich fluid will attract vitreous fluid into itself
(Raehlmann, 1893); or alternatively, in the process of detachment by mechanical
tearing when the retina was locally tacked down by adhesions to the choroid (Elschnig,
1914); or that at a later stage they may develop as the result of the action of autolytic
ferments (Elschnig, 1914) and maceration of the retinal tissue (Löwenstein, 1924).
Finally, the advocates of the conception that holes are of secondary formation
point to the clinical occurrence of cases of detachments without holes, and of holes without
detachments. Undoubtedly these exist. In the first case, when the detachment is
serous and non-exudative, it may always be argued in the opposite sense that the
clinical examination has been incomplete, that a hole existed and was not visible
ophthalmoscopically. In the second case, holes without detachments have been
repeatedly noted (Sachs, 1930; Trantas, 1930–34; Gonin, 1930; Jeandelize and
Baudot, 1932 ; Anderson, 1932; Pavia and Dusseldorf, 1933; Auruga, 1934; Vogt,
1934); in the case of macular holes a detachment is relatively rare ; while the hole
formed by scleral puncture in a normal retina heals up and does not lead to detachment.
In certain cases the retina around a hole may be tacked down by adhesions, or the
appearance of a hole may be given when the limiting membrane is still intact, but
from many considerations it would seem to be the case that the hole itself is not sufficient to
cause the detachment : other factors—disease, degeneration, or severe trauma—must
operate as well.
With all the relevant information collected, we are now in a position to
proceed to consider in general terms the aetiology of retinal detachment in
the light of our present knowledge. The problem can be stated as follows:
Normally, as we have seen, apart from its anterior and posterior attachments
at the ora and the optic nerve, the neural layer of the retina merely lies upon
the pigment epithelium, the potential space of the primary optic vesicle
intervening between the two. In the ordinary course of events, the
retina stays in position partly because of capillary attraction between
the two layers, and largely because of the influence of the intra-ocular
pressure distributed uniformly through the vitreous body pressing it from
within on to the epithelium. So long as the sub-retinal space remains a
potential one and the intra-ocular pressure remains normal, and so long as
the retina itself remains intact, no detachment can take place. Detachments,
however, occur in the following conditions :—
A. THE RETINA Is RAISED UP FROM WITHOUT BY ABNORMAL CONTENTS
OF THE SUB-RETINAL SPACE.
1. Intra-ocular tumours or cysts (choroidal malignant melanomata,
“glioma ‘’ exophytum, sub-retinal cysticercus).
DISEASES OF THE RETINA 2.895
It is to be noted that in these cases the detachment is usually
much larger than the actual bulk of the neoplasm warrants,
Owing to the accumulation of an exudative sub-retinal fluid.
2. A collection of exudative fluid gathers in the sub-retinal space, due to
Some disturbance in the choroidal circulation, either congestive
or inflammatory. &
This may be due to (a) pathological causes outside the eye,
such as an orbital tumour or abscess, sinusitis, scleritis, tenonitis ;
OI’
(b) within the eye, as retinitis or retinopathy (infective,
metastatic, exudative, renal, etc.), papilloedema, or any exudative
choroiditis or choroidal haemorrhage ; or alternatively,
(c) to some mechanical factor such as trauma which disturbs
the normal balance between the capillary circulation in the
choroid and the intra-ocular pressure.
B. THE RETINA IS PULLED UP FROM WITHIN. This occurs in a gross
degree as a consequence of fibrous tissue formation in the vitreous chamber,
and may be a consequence of :—
1. A perforating wound, especially involving the ciliary body, or the
reaction to a foreign body retained in the eye.
2. Organising intra-ocular haemorrhages.
3. Intra-ocular inflammation or suppuration followed by cicatricial
fibrous tissue formation in the vitreous cavity.
There is a large amount of evidence that in a much less obvious degree traction
upon the retina by an adherent vitreous may be responsible for the formation of
certain retinal tears. This aetiological factor therefore enters into the causation of the
next group.
C. A soluTION OCCURS IN THE CONTINUITY OF THE RETINA allowing
fluid from a degenerated vitreous body to pass underneath it and float it
up, due to either
(a) the formation of a hole at a small focus of inflammation or
degeneration ; or g
(b) the formation of a tear by an adherent vitreous body pulling on a
degenerated area of the retina ; or
(c) a rupture due to a direct or indirect injury. In the latter case the
retina may be torn by the sudden distension of the globe caused
by a severe contusion. -
D. THE RETINA Is THRowN INTO LOOSE FOLDs by a marked diminution
of the volume of the globe, occurring either as a sudden calamity as in a
perforating injury, or in a slow process of progressive phthisis bulbi.
A unique case was described histologically by Takamatsu (1933) wherein a
detachment appeared to be due to the traction of fibrous tissue (of tuberculous origin)
on the outside of the retina, between it and the pigment epithelium.
T.O. — WOL. III. 3 F
2896 TEXT-BOOK OF OPHTHALMOLOGY
The mechanism of some of these is obvious, particularly of those
detachments due to the pushing inwards of the retina by a tumour, the
pulling inwards of the retina by organizing fibrous tissue, the tearing of the
retina by sudden traumatic distension, and the wrinkling up of the retina in
a shrunken globe. The remainder of the types of detachment can be classed
into two groups: (a) Eacudative detachments which are not typically
associated with a retinal hole, and (b) serous detachments in which a hole is
characteristically present.
Eacudative Detachments. In certain types of detachment this aetiology
is clear—the type which is associated with a passive congestion of the
choroid (tumours, extra-ocular inflammations) and that caused by
transudation from the vessels such as is typified in renal retinopathy.
In these cases the fluid in the sub-retinal space is very highly albuminous and
readily coagulates; and it seems fairly certain that the sequence of events
involves a stasis in the choroid, a raising of the capillary pressure in the
chorio-capillaris, a dilatation of the small vessels, an increased permeability
of their walls, and the exudation therefrom of a protein-rich transudate.
This entails a disturbance of the pressure equilibrium on either side of the
retina. When the capillary dilatation and increased permeability involve
the ciliary body and iris—as occurs, for example, in irido-cyclitis—we get
an albuminous aqueous and a tendency to secondary glaucoma because of
the increase in colloid osmotic pressure. When the choroidal capillaries
are thus affected and an exudation of a fluid rich in colloids dialyses into the
potential space of the primary optic vesicle, the dis-equilibrium between the
chamber of the eye and the sub-retinal space must entail a rise of the hydro-
static pressure behind the retina, and hence a tendency for its detachment.
The pressure behind the retina is now greater than the pressure in the chamber
of the eye by an amount depending on the colloids in the sub-retinal fluid.
To speak figuratively, in this localized region we have one of the conditions
determining a secondary glaucoma. Once a colloid-rich fluid starts to
collect here, being of a high osmotic pressure and surrounded by membranes
impermeable to colloids (for the retina acts as a semi-permeable membrane,
allowing the free passage of water only : Key, 1932), it will attract water
from the chambers of the eye to itself by osmotic forces to dilute it. Con-
sequently, not only does the fluid remain, but it tends to augment in bulk.
The hydrostatic pressure behind the retina therefore tends continually to
increase, and the detachment tends to be perpetuated and to grow.
Such a process need not be accompanied by an increase of ocular tension, since
the extra volume occupied by the sub-retinal fluid can be compensated by an increased
drainage of intra-ocular fluid, a process which would be aided by the fact that in these
cases the vitreous gel is almost invariably of fluid consistency.
It is a moot question how far this mechanism can explain cases other than those
obvious ones which we have mentioned. It would seem arguable that any condition
which gives rise to an increased capillary pressure and an increased capillary
DISEASES OF THE RETINA 2897
permeability in the choroid could give rise to a similar train of events. A patch of
choroiditis would act in this way; and it must be admitted that the more carefully
cases of detachment are examined, the more frequently areas of equatorial or anterior
choroiditis are found, or, at any rate, patches of choroidal degeneration. Trauma
would act in the same way provided the vitreous body was of somewhat fluid con-
sistency, which is usually the case. Any slight pressure on the eye—on the receipt
of trauma, a slight exertion, or even on bending—raises the intra-ocular pressure,
and consequently some fluid is forced out from the chambers of the eye. As soon as
the extraneous pressure is removed, the pressure in the chambers of the eye becomes
less than normal, since some of its fluid content has been lost. In this temporary
hypotony, the capillaries, losing their normal pressure-support, become dilated, their
permeability is increased, and a colloid-rich fluid dialyses from them to replace the
intra-ocular fluid which was lost. In the chambers of the eye a plasmoid aqueous 1
is formed ; and if the vitreous gel has lost its normal turgescence, so that it no longer
supports the retina from within, the fluid which dialyses from the chorio-capillaris
will collect underneath the retina more easily than anywhere else, causing a retinal
detachment. Any condition, in fact, wherein the tension of the eye becomes sub-
normal (especially suddenly subnormal), is theoretically conducive to a detachment,
for in these circumstances capillary dilatation and increased permeability tend to
occur. If to these is added a transient increase in blood-pressure, which would increase
the transudation of the fluid, the tendency to detachment is increased ; if the capillaries
are diseased or degenerate, so that transudation is easy, the likelihood is greater ;
and if, finally, the vitreous gel is also degenerate and offers no support from within,
the likelihood becomes greater still.
The great fault—and an essential one—in the argument is the fact that in the
vast majority of cases a retinal hole is present through which fluid can pass, so that
the semi-permeable membrane between the chamber of the eye and the sub-retinal
space no longer exists. It is, of course, possible to argue that the retinal tear is formed
incidentally at the time of detachment, for example, by the tearing of a degenerated
or adherent area, and that its presence, by allowing the free ingress of vitreous fluid
behind the retina, may be of importance chiefly in that it is the main factor in per-
petuating the detachment. The dramatic effects of closure of the hole, it may be
said, need not thus be taken to prove any aetiological significance. The sum of evidence,
however, points to the conclusion that retinal tears do play a determining part in the
causation of many detachments, and consequently, while we will admit that a type of
detachment occurs due solely to a disturbance of the pressure equilibrium on either
side of the retina, we will proceed to a consideration of a second and more important,
because a more common type—that which is characterized by a retinal hole and a
relatively non-albuminous fluid in the retro-retinal space—the serous detachment—
in many cases of which the factor of exudation may indeed enter, but in which the
determining cause seems to be the retinal hole or tear.
Serous Detachments. We have already seen that it seems very
probable that in most cases the actual determination of a hole depends
primarily upon an area of degeneration in the retina, either the result of
inflammatory activity or cystoid degeneration, and it has been pointed out
that foci of inflammation or degeneration are almost the rule in detachments.
These inflammatory, myopic, or senile degenerative changes in the choroid
and retina tend to find their counterpart in the vitreous body, where
the same causal factors produce a separation of the colloid basis from the
1 Vol. I, p. 436 (440).
2898 TEXT-BOOK OF OPHTHALMOLOGY
gel into fibrillar formations. Even in the healthy vitreous gel definite lines
of force exist (Duke-Elder, 1930); in the gel which is breaking down or in
one which has become largely fluid, these become much more marked (Duke-
Elder and Robertson, 1934). We have seen that the colloid basis of the gel
is derived essentially from the retinal ectoderm ; it, the internal limiting
membrane, and Müller's fibres have all a common origin "; these tissues
always tend to retain a close association, and in pathological sections the in-
ternal limiting membrane frequently adheres to the vitreous body rather
than to the retina itself. Even in the normal eye if the vitreous is removed
it usually tears away with it pigmented portions of the ciliary epithelium
and the anterior part of the retina, so strong is the adhesion between the
two. Around a focus of chorio-retinitis it would seem obvious that
adhesions of these elements of the vitreous would be pronounced, and that
such is the case has been histologically demonstrated ; while in the periphery,
where cystoid degeneration is most marked, the “base of the vitreous ”
constantly adheres to the retina with considerable tenacity. In all pro-
bability this explains the importance of the factor of small unnoticed traumata
or physical strains in the aetiology of detachment of the retina. Around the
areas of inflammation the vitreous framework becomes soldered, near the
ora it is already fixed ; as the eye moves, it exerts traction on the weakened
areas of the retina, and the more fluid and degenerated the vitreous gel has
become, the more abrupt will be the forces exerted (Duke-Elder and
Robertson, 1934), and the more likely will the retina be torn, since, when
a movement is initiated or stopped, the shock of the momentum of the mass
of gel will be felt at one or two points only, whereas when the gel is solid, the
stress is uniformly distributed. While the essential element in the forma-
tion of a circular punched-out hole seems to be a destructive inflammatory
focus or a cyst, it is probable that in the formation of horseshoe-shaped or
arrow-head rents it is not the accompanying retinal lesion but rather this
factor of tearing by vitreous adhesions which determine the shape. In
certain cases, it is true, the contraction of organizing fibrous tissue in the
vitreous obviously pulls the retina away, but apart from that, the action of
well-defined vitreous bands possibly plays a small part ; and an equally
small part is probably played by the invasion of the anterior vitreous by
inflammatory elements from the ciliary body as postulated in the pre-
retinitis of Leber. The vitreous changes are usually degenerative, and of a
much more subtle nature.
It would seem, therefore, that in this class of detachment, apart from
gross trauma, the essential precursors of the condition are either inflam-
matory or degenerative processes in the retina and the vitreous body.
These processes in the retina determine the formation of a hole or of a
weakened area. To this area are anchored strands of the colloid basis of
the vitreous gel, which, on some sudden movement, minor trauma, or
1 Vol. I, p. 342.
DISEASES OF THE RETINA 2899
physical strain, tear a rent in the weakened area of the retina by their
traction upon it. Once such a hole is formed, a detachment does not
necessarily follow, particularly if the vitreous gel has remained solid; but
if the latter is fluid the tendency will be, either at the time the retinal tear
is formed or on some subsequent occasion, for some of the vitreous fluid to
get behind the retina and detach it ; and so long as free passage through the
hole is allowed, the tendency will be for further movement or vitreous
traction to increase and perpetuate the detachment. The essential condi-
tions, therefore, are a degenerated retinal area, vitreous liquefaction,
adhesions between the vitreous and this retinal area, and a movement of the
vitreous sufficient to exert a strain such as will cause a tear in the retina.
If the retina is sound it probably tears with difficulty, but if the retina is
diseased the causal factors need be very slight. Such detachments may be
called “spontaneous ” in the sense that the eye is unable to withstand the
ordinary strains and stresses of everyday life.
In summary, we may therefore divide “spontaneous ” retinal detach-
ments into two main classes :—
(a) AN ExUDATIVE TYPE, essentially due to a vascular disturbance in the
choroid, either caused by inflammation or engorgement. The essential disturb-
ance is in the choroidal circulation ; the immediate cause is an upset of the
pressure equilibrium in the eye. Such detachments do not necessarily
exhibit a hole in the retina, and the treatment, as we shall see, should be
along medical lines. *
(b) A SEROUS TYPE, essentially due to local inflammatory conditions of
chorio-retinitis, degenerative changes (usually those of myopia or senility), or
traumata. The essential lesion is in the retina itself; the immediate cause of
such a detachment is the formation of a retinal hole or the tearing of a weakened
retinal area by a vitreous adhesion, and the treatment, as we shall see, should
be closure of this hole by surgical interference, the evacuation of the sub-
retinal fluid, and the production of adhesions between the retina and the
choroid underneath.
The most important consideration in the whole matter is the fact that
the detachment itself is to be taken as a terminal incident only—dramatic
though it be. THE ESSENTIAL FACTOR IN THE AETIOLOGY Is THE PRECEDING
INFLAMMATION OR, DEGENERATION WEHICH RENDERED POSSIBLE IT'S OCCUR-
RENCE, AND IT IS THE PROCESS WHICH GAVE RISE TO THIS WHICH IS THE TRUE
CAUSE OF THE CONDITION AND SHOULD RECEIVE PRIMARY CONSIDERATION IN
TREATMENT. -
Clinical Symptoms
When a detachment is secondary to gross disease of the eye and forms an incident
in a clinical picture dominated by other things, typical symptoms are not evident.
We shall here consider the symptomatology of those cases wherein the detachment
itself is the essential clinical feature.
2900 TEXT-BOOK OF OPHTHALMOLOGY
A detachment of the retina may appear quite suddenly and without
warning, but very frequently two prodromal symptoms precede its occurrence.
(a) The appearance of muscae volitantes. The complaint of “seeing
spots” in the eyes is so common as not to merit special attention, particu-
larly in the classes most prone to detachment—the myopic and the senile ;
but the sudden appearance of muscae in quantity, or their sudden increase
should merit attention, as suggesting the onset of a rapid degenerative change
in the vitreous gel or the development of a focus of peripheral choroiditis
with their potentialities for detachment.
(b) The sensation of flashes of light (photopsiae) is of greater significance,
indicating, as it does, slight movements of the retina involving a mechanical
stimulation of the percipient elements: their importance is increased if
they are constantly projected from a single region of the retina. The
patient complains of seeing showers of sparks or luminous arcs, usually
white or blue, often of considerable intensity, and frequently causing
considerable alarm. Sometimes they come in transient attacks, sometimes
they are constantly present, and sometimes they are apparent only on
moving the eyes in a particular direction ; a detachment may follow
their appearance in a few moments, or after the interval of some days,
although the sequence is by no means necessary or invariable. Gonin (1934)
reported the case of a high myope who, for 10 years, experienced such
sensations whenever he looked to the right, who suffered no detachment.
This observer suggested that the photopsiae were due to traction by vitreous
adhesions upon the retina, an explanation which has much to recommend
it. At the moment of detachment photopsiae are sometimes markedly evident.
Once a detachment has developed the onset of symptoms may become
apparent in one of three ways : if the detachment is large or central, vision
may be deranged or blotted out with the suddenness of an unexpected
calamity; visual disturbances may gradually increase as a peripheral
detachment spreads to the central area ; or, if the detachment is localized
and shallow and some distance from the macula it may give rise to no
symptoms at all. In Stallard's series (1930) the onset was sudden in 52%,
gradual in 42%. When symptoms are entirely lacking, particularly if the
lesion is limited to the lower retina, the detachment may escape the notice
of the patient until it is discovered on routine clinical examination. It is
not associated with pain; the only symptom is visual impairment or failure.
When the impairment is obvious the patient complains of a veil or cloud
suddenly appearing in front of the eye. If the detachment is peripheral,
central vision remains initially unimpaired : in one case it remained 6/5 after
14 months in the presence of a bilateral detachment (Lawson, 1924). An
area of the visual field, however, corresponding to the area of the detachment
is sharply cut off, only part of an object, for example, being seen : in this
event, perimetry will reveal an absolute scotoma. If the detachment is
shallow, it is true some vision may remain in the detached area : in these
DISEASES OF THE RETINA - 2901
circumstances the scotoma is relative, there is an increase in the light mini-
mum and a defect of colour vision of the tritanopic type * is evident.
If the detachment involves the macula, central vision is lost ; and as a
peripheral detachment spreads towards the macula and this area becomes
progressively involved, the vision is increasingly disturbed : objects appear
distorted, metamorphopsia and micropsia become evident, and eventually
as the detachment increases the world assumes a shimmery, vague, and
unsteady aspect as if it were viewed through water, an appearance which
causes no little annoyance and sometimes vertigo. So long as the detach-
ment remains shallow this uncertain type of vision may remain, even for
periods of many months; but as the rods and cones separate further from
their source of nourishment in the chorio-capillaris, all vision is gradually
lost, and eventually even perception of light disappears.
If a large balloon detachment is present, uni-ocular diplopia may occur ; and in
those rare cases of inversion of the retina where, in an extensive dialysis, the detached
retina is folded over the intact retina, an inverted diplopia results, since the image due
to stimulation of the inverted layer is projected as if it were still lying on the choroid.
Objects on the right are thus projected to the left, movements of the two images take
place in opposite senses, and a wheel rotating in one direction appears to rotate in
the opposite (Baquis, 1896; Constentin, 1904).
After a detachment has developed, and even in long-standing cases when the
patient is blind, movements of the retina may give rise to persistent photopsiae, which
are sometimes of a very brilliant nature, and may entail a considerable amount of
distress—so much, indeed, as to induce the patient to request removal of the eye.
Clinical Signs
Externally the eye appears normal ; the anterior chamber may be deep,
but is not necessarily so, and the tension may be either normal or diminished.”
By the plane mirror, the red reflex is altered to appear grey over the detached
area, and if the retina is displaced far anteriorly, details of the fundus may
be seen. On ophthalmoscopy in the typical case, the retina is seen to be
thrown into folds which oscillate with movements of the eye ; these may be
small or may assume the proportions of a large balloon, sometimes obscuring
the disc. At other times the whole area appears crinkled, with innumerable
tiny waves, an appearance perhaps associated with a relatively solid
vitreous (Fig. 2501). At first, and particularly if the detachmentis shallow, the
retina may remain transparent so that the red reflex of the choroid is retained,
but usually (although not invariably) as time progresses, or if the folds are
well marked, light is reflected from the retina, which assumes a characteristic
greyish tint, until finally the crests of the folds appear brightly white and
the depressions greyish-green.
The appearance of the vessels is important. Lying away from the red
ground of the fundus, they intercept the light coming to the observer's eye,
1 Failure to recognize blue; see Vol. I, p. 976 (988). * p. 2886.
2002 TEXT-BOOK OF OPHTHALMOLOGY
and therefore, like vitreous opacities, they appear black and lose their
central reflex: they seem small, and, as they follow the undulations of the
retina, they assume the form of dark
tortuous cords, oscillating with the move-
ments of the detached retina. Sometimes
in a shallow detachment their shadows
can be seen on the pigment epithelium
and parallax can be elicited between the
vessel itself and its shadow (Aust, 1932).
Associated with the detachment
several clinical features deserve note.
White exudative spots are not at all
uncommon, and minute retinal haemor-
rhages are almost the rule. A hole or a
tear, as we have seen, is present in the
Fig. 2501.-Detached Rºnina. great majority of cases, the clinical
The wavy appearance denoting a characteristics and distribution of which
relatively solid vitreous gel (Lindner, we have already discussed in detail. The
(A. f. O.). frequent occurrence of patches of i-
quent occurre D S 01 peri
pheral choroiditis, retino-choroidal degeneration near the ora, and pigmen-
tary disturbances in the pre-equatorial region, bears witness to the
important aetiological influence of degeneration and inflammatory changes.
The Finding of a Retinal Hole or Tear
Since important points in the differential diagnosis of the type of
detachment and the crucial consideration of operative treatment depends
on the finding of a retinal holeorrent, to establishits presence is of paramount
importance. Frequently it is no easy thing to do. It involves the careful
and repeated examination of every part of the fundus under full mydriasis,
and, since most holes (about 80%) are near the ora, particular attention
should be paid to the periphery. Further, since more than one hole frequently
exists, the finding of one does not justify the cessation of the search. Small
holes in the main part of the fundus are best seen by direct ophthalmoscopy,
but owing to the prismatic effect of the condensing lens, the indirect method
allows of easier and fuller access in the extreme periphery. If a hole cannot
be seen at a first examination, it may well be hidden by some retinal fold,
in which event it may become evident after the patient has been kept
recumbent for some days when the configuration of the detachment may
have changed, or after the sub-retinal fluid has been withdrawn with the
same end in view. It is important that it need not necessarily be found at the
site of detachment at the time of examination, for, particularly if the hole
is in the upper part of the retina, the sub-retinal fluid may have
gravitated to the lower part of the globe, producing an inferior detachment
and leaving the hole in an area of flat retina marking the position of the

DISEASES OF THE RETINA 2903
causal lesion. Some help in its location may be obtained from the patient's
history, for he may indicate the initial position of a loss of field or the
appreciation of a black spot or a premonitory flash of light in a definite
direction (Hamilton, 1930). The proximity of a focus of old choroiditis is
frequently a useful guide ; and the use of red-free light is helpful, since a
greater contrast is obtained when the free edges of the opaque retina are
accentuated against the dark choroid.
To facilitate finding a hole, Knobloch (1933) suggested injecting fluorescein into
the sub-retinal space, and then watching it diffuse through the hole into the vitreous
chamber. A hole is to be
differentiated from a very thin
area of retina or a small haemorrhage.
The distinguishing points are that a
hole is of a brighter red than a
haemorrhage, while, in contrast with
both, its edge is sharper and fre-
quently curled or white, and, lying
in front of the choroid, it is possible
to elicit parallax between its edges
and the choroid underneath.
The vitreous gel in almost every
case is fluid and constantly shows
some degeneration and opacities."
With the slit-lamp, particularly in
myopic detachments, the anterior
region is usually seen to be broken
up into fibres which are freely mov-
able and show white and brown
pigmentary dots, all signs of a regular
Scaffolding having disappeared (Vogt,
1921 ; Koby, 1925; Caramazza, FIG. 2502.-DETACHMENT OF THE RETINA.
1933). Occasionally the entire vit- Isopters for 20, 5, and 1/330 white and 2012,000
reous is of a greyish-yellow colour, red (...) and blue (-,-,-). Note the interlºing
presumably due to serous infiltration. of the colour fields and the relative central defect
- g blue. W = 6/12 (Traguair, Clinical Perimetry).
Perimetry in Detachment. The for blue |12 (Traq ty)
field defect in detachment is usually
relative at first and gradually becomes absolute as the age of the detachment increases,
and in serous detachments it is bounded by a transitional zone of varying width unless
a sharp edge is mechanically produced by an overhanging fold of ballooned retina ; in
cases complicated by a tumour the edge of the defect is usually sharp. Since, as we
have seen, there is an increase in the light minimum and an acquired tritanopia, the
full extent of the field-defect is best brought out by perimetry in reduced illumination,
and the relative scotoma is best delineated by a blue test-object, since it is more
intense for this colour. It follows that, especially in the central area, the red field
overlaps the blue, thus producing a true interlacing of the colour fields (Fig. 2502).
Clinical Progress
Once established, a retinal detachment may re-attach itself spon-
taneously, remain practically stationary, remain localized but migrate to
1 p. 3262.

2904 TEXT-BOOK OF OPHTHALMOLOGY
another and more dependent part of the eye, or tend progressively to
spread.
Spontaneous re-attachment is not very uncommon in exudative detach-
ments if conditions allow for the absorption of the exudate—in the detach-
ment associated with the toxaemia of pregnancy, for example, the prognosis
is fairly good –but in serous detachments, particularly if a hole is present,
this happy event is rare. Usually when it does occur, it does so during
complete rest, but instances have been reported occurring after violent
Fig. 2503.-Dºmachºn RETINA.
An inferior doubly-ballooned detachment formed by gravitationa collection of
sub-retinal fluid, showing a median furrow (Shapland, T. O. S.)
movements (a fall, vomiting, etc.) (Mooren, 1882; Remak, 1907). The re-
attachment may be dramatic, as witness a case of complete detachment
of 3 months duration which completely re-attached itself after 48 hours'
rest (de Schweinitz, 1899), or a case which re-attached after a detachment
had persisted for between 10 and 12 years (Cruise, 1923). Usually, however,
such a re-attachment is temporary, the retina tending to come away again,
a sequence which may happen quite a number of times (Hirschberg,
1874–91; Stilling, 1883; Schweigger, 1883; Fränkel, 1895; Horstmann,
p. 2725.

DISEASES OF THE RETINA 2905
1898; Highett, 1924; and others). A committee appointed by the Ophthal-
mological Society of the United Kingdom (1916) reviewed 85 cases of
cured detachment, in 28% of which recovery was apparently spontaneous:
among these 85 cases, 3 had tears, and of these 2 seemed to have re-attached
spontaneously : it is to be remembered that retinal holes have been observed
to heal." Out of 351 cases of re-attachment Uhthoff (1922) found that 31%
were spontaneous. It is probable that in the vast majority of these the
primary lesion was a choroidal effusion ; a retina which re-attaches spon-
taneously has no hole, and if a hole is present the chances of re-attachment
are negligible.
To remain stationary is a more common clinical event, which is seen
most frequently in cases wherein the retina is partially tacked down by
choroiditis, and sometimes in cases of an anterior dialysis, particularly when
the lower part of the retina is involved : in these cases some degree of
pigmentary disturbance is seen round the edge of the detached area. Such
a partial detachment may remain unchanged for a long period—even for
17 years (Neame, 1928).
The migration of detachments is interesting—quite a common occurrence
if the lesion starts in the upper segment of the globe. Frequently in these
cases, the sub-retinal fluid, which is heavier than the vitreous, seeps down-
wards to the lowest part of the eye, and it may well be that the detachment
in the upper region re-apposes itself to be replaced by a detachment below
where it may remain stationary for a very long time. In these circumstances
it frequently takes the form of a double balloon, the two bellies being
separated by a deep cleft running backwards in the mid-line (Fig. 2503). This
formation of a deep medial sulcus is characteristic of a detachment which has
migrated from above and should direct the search for the retinal tear, not
in the neighbourhood of the detachment itself, but to the upper half of the
retina.
To progress to complete separation is undoubtedly the commonest course
taken by a retinal detachment. Sometimes few further changes take place,
the eye giving rise to no further trouble, and looking normal externally for
an indefinite time. Usually, however, a long-standing detachment is
eventually associated with a slow degeneration of most of the tissues of the
globe. As time goes on the retina loses its grey colour and becomes trans-
parent again, and when its separation becomes complete, it retains its
attachment only at the ora and the disc, taking the general shape of a
convolvulus flower, the greater part being crowded up behind the lens,
while the remainder runs as a cord towards the disc. The vitreous body
gets more and more clouded, and not uncommonly a faint ciliary flush
indicates the onset of a mild uveitis, while a secondary cataract appears
which progresses rapidly to a pearly-white maturity. As time goes on,
degenerative changes go on apace, the tension falls, and general atrophy
1 p. 2886.
2906 TEXT-BOOK OF OPHTHALMOLOGY
and shrinkage result, or inflammatory changes dominate the picture with the
development of plastic cyclitis with the formation of synechise, leading
ultimately to the onset of a secondary glaucoma which necessitates
removal of the eye because of pain (Fig. 1883).
The Appearance of the Fundus after Re-attachment
If re-attachment has not been too long delayed the retina may appear
ophthalmoscopically normal, but usually the partial disappearance and
migration of pigment gives it a spotted and irregularly pigmented character.
If the detachment has been of some standing, the most characteristic
appearances are white lines, arranged roughly concentrically, which indicate
the temporary limits of a receding area of detachment—stria, RETINA,
Fig. 2504.—STRLE RETINAE.
After a detachment (x 122) (Hagedoorn, A. of 0.).
(v. Jäger, 1869; Stilling, 1883) (Fig. 2491, Plate LIX). These lines may be
quite numerous and they may branch; they lie deep to the retinal vessels
which run over them without undulation. Rönne (1910) found their histo-
logical basis to be fibrous tissue between the retina and choroid. It has been
suggested that they are due to the organization of sub-retinal haemorrhages
(Holden, 1895), but most are agreed that they are characteristicofare-apposed
detachment and represent remnants of coagulated fibrin which produce
adhesions between the retina and the choroid (Leber, 1877; Uhthoff, 1894;
Klainguti, 1923; Slocum, 1926) (Fig. 2504). As the exudative sub-retinal
fluid is absorbed, new boundaries of coagulum are left, which may be
* This appearance is usually called "retinitis striata"—an obviously bad name. v. Jäger
called the lines “retinal cords": Stilling introduced the term “striae retine,” designating as
“retinitis striata" any inflammatory change which assumed a striate arrangement.

DISEASES OF THE RETINA 2907
compared to the lines of débris which mark the limits of the receding tide
upon the sand of the sea-shore.
Diagnosis
Although in well-defined cases the diagnosis is obvious, it may present
great difficulty if the detachment is localized and shallow, particularly if it
is in the periphery. The essential points to be noted are the alteration in the
red reflex, the necessity to employ a stronger convex lens to focus one
retinal area compared with another, the small black tortuous reflexless
vessels, and the defect in the visual field most marked in feeble illumination
and to a blue test-object. If the media are opaque, either from a vitreous
haemorrhage or a cataract, a certain diagnosis is much more difficult ; all
that can be done is to point out the possibility of detachment if the projection
of light is deficient, particularly if the tension is sub-normal.
The diagnosis between a simple detachment and a retinal cyst is not
easy. The majority of the latter are situated peripherally, and suspicion may
be aroused by the smooth globular shape of the detachment, its slow progress,
and the absence of a hole. Fortunately the differential diagnosis is of little
more than academic importance, for with diathermic puncture the cyst
collapses and the ultimate prognosis is relatively good."
Once the diagnosis is established, the essential point is the decision as to
whether the detachment is simple or complicated by a tumour. It is
sometimes not an easy matter to establish this with certainty, and since
on the differential diagnosis depend decisions of the highest importance, the
distinguishing points merit the closest attention.
(a) A detachment due to a tumour appears w8wally more solid than a serous
detachment and tends to be less tremulous. Especially is this so in the region of the
neoplasm, for at this point the retina tends to remain in contact with the most prominent
part of the tumour regardless of the extent of any extension of the detachment due
to exudation (Lindahl, 1920).
(b) At this point also the retina appears sometimes degenerated and pigmented,
showing a yellow, brown, or dark coloration. If the detachment is anteriorly situated,
the pigment spots may be seen by the slit-lamp (Meesmann, 1920).
(c) It is claimed by some that in the presence of a malignant melanoma the
sub-retinal fluid is usually of a yellow colour giving the detachment a yellowish tinge
(Rönne, 1928).
(d) The statement that the presence of a retinal hole excludes a neoplasm (Lister,
1924) has never been contradicted.
(e) Newly formed vessels running over the surface of a detached retina should
give rise to a suspicion of the presence of a new growth ; these, it is to be noted, are to
be distinguished from the newly formed vessels which tend to grow into the
vitreous body at the site of a patch of degeneration or inflammation.” Similar
new vessels may even on occasion be seen in the corresponding sector of the iris.
Small haemorrhages and minute new vessels are best distinguished in red-free light.
(f) A careful watch over the progress of the detachment may give information
of value. A serous detachment more readily shifts its position, or may even go back
1 p. 2810. * p. 2618.
2908 TEXT-BOOK OF OPHTHALMOLOGY
after rest, while a malignant one tends slowly and progressively to increase ; it does
not regress. Stereoscopic photography may be useful in recording these changes.
(g) A gradually and regularly increasing field defect is suggestive of neoplasm ;
marked variation or a stationary Scotoma indicates a serous detachment. The field
defect, moreover, due to a tumour is characterized by a sharp definition; it lacks the
sloping edge of a serous detachment, and the extension found in dim illumination is
less generally present. Finally, the presence of a very dense Scotoma within the
relative Scotoma is suggestive.
(h) Trans-illumination may provide information of the utmost value, and in cases
of doubt should be exhaustively employed in all its forms. In difficult cases an
alternating examination by direct Ophthalmoscopy and trans-Scleral trans-illumination
is especially useful, and has on occasion been the only means by which a diagnosis has
been made 1 (Lindahl, 1920; Trantas, 1926; Holth, 1926; Rönne, 1928; Klein,
1929, and others). Any highly pigmented tumour obstructs the light, but it is to be
remembered that a cyst containing blood pigment, a retro-choroidal haemorrhage
or a choroidal exudate may cast a shadow, while a tumour free from pigment may
be translucent.”
(i) The tension sometimes gives an indication. As a rule (but not constantly)
the tension of an eye with a neoplasm is not seriously lowered, especially if the neoplasm
is anteriorly situated. It is to be remembered, however, that some diminution is
frequent, and also that the tension may be raised in a simple detachment.”
(j) When doubt still exists and suspicion is so strong that observation over some
time seems unjustified, a Scleral puncture may be performed to draw off some sub-retinal
fluid. The fundus may then be examined to see if the detachment grows less, and
the sub-retinal fluid can be examined for neoplastic cells (Meisner, 1923). Some
authorities consider this justifiable, others unjustifiable, and others useless. It is
only very rarely justifiable, when all other methods have failed and when one is
prepared to enucleate the eye immediately, since rapid extension of the neoplasm may
occur under the conjunctiva at the site of puncture.
Treatment
Historically the treatment of retinal detachments can be divided into a
pre- and post-Gonin epoch. Up to the time when Gonin (1925–28) advocated
an operative technique for the closure of retinal tears and holes, the
ophthalmologist could offer little or no hope for successful treatment.
As is usual in these circumstances, a large number of methods had been
proposed, each of which has had its vogue, and all of which had given rise
to alternating phases of encouragement and disillusionment. Of all these
methods, two survive—rest and operative closure of the retinal hole with
the formation of chorio-retinal adhesions; the remainder, being practically
useless, we shall discuss only shortly.
Prophylaxis. Although a detachment of the retina usually presents
itself as a fait accompli to the ophthalmologist, the presence of the prodromal
symptoms of photopsiae (especially when referred from one localized region)
and a sudden increase of muscae volitantes in an eye which may be considered
predisposed to detachment (showing myopic or senile degeneration or
1 Vol. II, p. 1153. * p. 2514. * p. 2886.
DISEASES OF THE RETINA 2909
multiple foci of peripheral choroiditis) or after a trauma, should suggest
the application of prophylactic measures in the shape of complete rest in
bed under observation, perhaps in very suspicious cases with bandaging of
the eyes for some days, or at any rate the wearing of opaque spectacles with
a central small aperture.” Moreover, it is probably legitimate to close a
hole operatively if it is found on routine examination, particularly if it is in
the periphery where the frame-work of the vitreous is normally adherent,
even if at the time it is not associated with a detachment.
A. Medical Methods of Treatment
1. Rest. Since it was originally advocated by Stellwag (1861) and
Donders (1866), all are agreed that rest is essential in the treatment of
detachment of the retina, whether or not it is combined with operative
measures; and by rest is meant immobility as complete as can be obtained—
admittedly no easy matter, especially when prolonged over a period of weeks.
In cases of exudative detachments without a retinal hole, it offers a fair
prospect of success especially if it is combined with measures to promote the
absorption of the exudative sub-retinal fluid. When a hole exists, however,
the policy of despair so often advocated before a reasonably successful
operative technique was available, of keeping the patient absolutely immobile
for six weeks or longer, is almost invariably without result. As we shall note
later, however, absolute rest as an adjuvant to operation is essential.
When pushed to extremes, however, even in these cases, complete rest may be
curative—as witness a case reported by Vogt (1928) of a myope, who after rest,
bandaging and saline injections for 15 months showed a detachment with a tear
unchanged in condition, but in whom, after a further year’s immobility, the hole
gradually healed, the retina went back, and the vision after 5 years was 6/4. Such
heroic measures, however, can Surely be seldom applicable, ophthalmologists capable
of applying them are few, and patients able to undergo them are rare indeed.
(a) The question of decubitus is important. Complete immobility on
the back with the head between sand-bags used to be the favourite position
adopted. It is to be remembered, however, that the sub-retinal fluid tends to
gravitate to the most dependent part of the eye, and such a position may
encourage the detachment to spread to the macular area. If it is possible,
an attitude should be chosen so that the detachment is in the most dependent
position : if it is below, the Fowler's position should be adopted; if at the
side, the patient should lie on that side; and if above, the most satisfactory
position (which is usually impossible to maintain for long) is to lie supine
with the foot of the bed raised.
(b) Immobility of the eyes is more important than immobility of the
body, and to attain this, bandaging of both eyes is essential. When a hole
is present the most damaging movement is rotary, for thereby the vitreous has
1 vide infra.
2010 TEXT-BOOK OF OPHTHALMOLOGY
the greatest tearing effect (Lindner, 1931–32–34). To prevent this type of
movement Lindner therefore suggested that opaque spectacles with a small
central aperture should be worn prior
to operation to prevent a spread of
the detachment, and subsequently
in the stage of convalescence when
the period of strict immobility
attained by bandaging has passed
(Fig. 2505).
Weekers (1933) ensured immobility
of the eye by injecting 1 : 500 mercury
Fig. 2505.-Stºxopæic Spectacles. oxycyanide into the extra-ocular muscles
and into the superior formix, a procedure
which brings about a paresis of these muscles and ptosis. Arruga (1934–35) sutured
the inferior rectus to the lower lid, or alternatively, made a retro-bulbar injection of
the patient’s blood serum.
(c) Intra-ocular immobility should be maintained by keeping the eye
constantly atropinized. The importance of this is evident when it is
remembered that a contraction of the ciliary muscle pulls the choroid
forwards and disturbs the retina even as far back as the macula.
2. Compression-bandaging. Samelsohn (1875) was the first to advocate a definite
routine in the hitherto vague treatment of detachment, insisting on the primary
importance of rest and compression-bandaging for many weeks. His suggestions
have been followed by many clinicians with varying degrees of thoroughness, even
to the extent of overcoming the mechanical difficulty of applying even pressure to
the globe by using a plaster mould made to fit the eye and orbital ridges of each
individual (Mendoza, 1920). The motives advanced for the use of compression have
been many : that it prevents an ectasia of the globe (Donders, 1866); that it pushes
the retina back against the counter-pressure of the elastic sclera (Samelsohn, 1875),
an action which Fehr (1913) attempted to render more effective by a preliminary
seleral puncture; that it mechanically inhibits hyperaemia (Stellwag, 1861); that it
tends to lessen transudation and increases absorption (v. Graefe, 1863; Grossmann,
1883), and so on. It is true that occasionally good results seem to have followed the
procedure (e.g., Wessely, 1905); but most of its advocates (even the promoter of
the method–Samelsohn, 1887) have given it up, some of them, it is to be noted,
because of the development of keratitis and cyclitis (Leber, 1916). It is probable
that its only value is in the maintenance of immobility.
3. Diet. To diminish the tendency to exudation and promote the absorption
of fluid, it is frequently advocated that a special diet with restricted fluids should be
given, such as one containing a high proportion of fat to carbohydrates; this tends
to lessen the retention of water by the tissues. Marx (1922) advocated a salt-free
diet, reporting 10 cures; in this he was followed by Noiszewski (1923), Löwenstein
(1924), Jablonski (1929), Schieck (1930), and others. Perhaps the most important
dietetic question is that it should be such as will avoid any gastric disturbance or
constipation with its consequent discomfort and straining—always a difficulty in
immobilized patients.
4. Drugs. The drugs most frequently advocated in the treatment of detachment

DISEASES OF THE RETINA 291 l
are those favouring diaphoresis, such as sub-cutaneous injections of eserine—combined
perhaps with hot-air baths and purging—the idea being to favour the absorption of
the sub-retinal fluid (Donders, 1866; and after him very many others). As adjuvants
there have been added sodium salicylate (Schweigger, 1883; Ulrich, 1889;
Deutschmann, 1904, and others), iodides (Stellwag, 1861; Kerry, 1928, and others),
mercury, usually as inunctions (de Wecker, 1899; and others), quinine (Belarminow,
1903), thyroid (Lamb and Ziegler, 1921), calcium chloride since it diminishes
capillary exudation (Stargardt, 1922), and so on. All of these are probably alike in
being useless.
5. Other remedies of equal valuelessness have been suggested from time to time ;
venesection or the application of leeches (Hamburger, 1928; Aschner, 1929; Jablonski,
1929), massage (Gradenigo, 1894), and so on.
B. Surgical Treatment
A chaotic literature is full of innumerable methods which have been
proposed for the operative treatment of “idiopathic ’ detachment of the
retina—their number speaking eloquently for their valuelessness. They
may be classified into certain groups —
The evacuation of the sub-retinal fluid.
The formation of adhesions between the retina and the choroid.
The division of vitreous strands.
The reduction of the capacity of the globe.
The raising of the intra-ocular pressure.
The increasing of the bulk of the vitreous.
The closure of the retinal aperture and the simultaneous formation
of chorio-retinal adhesions.
Of all these the last is the only procedure of real value.
1. The evacuation of the sub-retinal fluid may be attempted by establishing either
a temporary or a permanent drainage.
(a) Temporary drainage was first attempted by James Ware (1805), who employed
Scleral puncture with a knife, the first operation devised for detachments; later,
v. Graefe (1863) extended the method by puncturing the retina also so that the sub-
retinal fluid could drain into the vitreous cavity. The thermo-cautery as a method
of puncture was introduced by Martin (1881), de Wecker (1882), and Abadie (1882),
and was popularized by Dor (1895–1907). As extended to multiple punctures
combined with attempts to form adhesions it has had a considerable following.
(b) The provision of permanent drainage has also had its advocates since trephining
was employed by de Wecker (1872) and Argyll Robertson (1876). The introduction
of Elliot's operation for glaucoma stimulated a renewed interest in the method
and many reports followed (Parker, 1915; Thomson, 1916–20 ; Ohm, 1917;
Chipman, 1921 ; Damel, 1921 ; Lambert, 1923; Hessberg, 1924; MacCallan, 1926;
and many others). Permanent successes, however, were scant, and in any event the
trephine hole soon closed up with fibrous tissue and drainage ceased. Previously
Galezowski (1890) transfixed the conjunctiva, sclera and choroid with gold wire, but
infection invariably led to loss of the eye. Wiener (1924) made two trephine holes
1 mm. apart, and threaded a strand of horse-hair with the aid of a curved needle into
one hole and out of the other. Galezowski (1902) suggested iridectomy : Meller (1923)
and Sloan (1926) advocated chorio-dialysis—opening the sclera, separating it from the
choroid, and then perforating the choroid. Grönholm (1921) reported some success
T.O.-WOL. III. 3 G
2912 TEXT-BOOK OF OPHTHALMOLOGY
with Holth's pre-equatorial sclerectomy—the removal of a large disc of sclera so that the
supra-choroidal space is put into communication with the space under Tenon's capsule.
(c) Sub-conjunctival injections have been advocated as a means of withdrawing
the sub-retinal fluid by osmotic attraction—a futile hope, since in the first place a
protein-rich exudative fluid could not pass through the coats of the eye, and in the
second, osmotic dilution of the injected fluid will take place much more readily from
the lax orbital tissues than from within the eye. Any very questionable effect sub-
conjunctival injections may have, apart from the rest with which they are combined,
must be due to the induction of a choroidal hyperaemia favouring absorption and the
formation of chorio-retinal adhesions. They were suggested for this purpose by
Grossmann (1883) and were first popularized by Mellinger (1896), who used hypertonic
saline—and innumerable authors have followed his lead using concentrations varying
from 1% to 30% ; while others have injected under Tenon's capsule (Dor, 1896;
Foster Moore, 1921). The sodium chloride was combined with mercury oxycyanide
by Ramsay (1906) and dionine by Tristaino (1922) to make the reaction more intense,
and with novocaine by Terrien (1922) to make it less painful. Other substances have
been used—corrosive sublimate (Zossenheim, 1895; Maraval, 1901), cyanide of
mercury (Fromaget, 1896; Darier, 1898; Lamb, 1921), sodium sulphate (de Wecker,
1899), sodium citrate (Savage, 1912), calcium chloride in sea water (Dor, 1910), guaiacol
(Darier, 1920), magnesium sulphate (McNabb, 1929), to mention a few.
2. The formation of chorio-retinal adhesions without special reference to the
closure of a retinal hole, as it was originally practised, was equally unsuccessful.
Every possible modification of “retinopexy '' has been attempted : puncture
with the cautery (Martin, 1881 ; de Wecker, 1882; Deutschmann, 1899),
superficial cauterization (Gaupillat, 1888); the application of the cautery directly
to the choroid after dissection of a scleral flap (Stargardt, 1922); electrolysis (Schoeler,
1893; Abadie, 1893; de Grandmont, 1894; Terson, 1895; Verhoeff, 1917; Sourdille,
1923–29 ; Gallois, 1926); the injection of irritant substances underneath the retina,
such as tincture of iodine (Schoeler, 1889), sublimate (Guende, 1890), blood-plasma
(Strampelli, 1933; Guist, 1933), caustic potash (Guist, 1933), or Lugol's solution
(Szymanski, 1933); even the heroic attempt to suture the retina to the choroid
(Galezowski, 1890; Rubbrecht, 1933).
3. The Division of Vitreous Strands. Believing that the essential mechanism
in the causation of detachment was elevation of the retina by vitreous strands,
Deutschmann (1895) attempted its cure by cutting them. Apart from an occasional
brilliant result in special cases, the operation has met with no success and
has been abandoned (Deutschmann, 1930).
4. The reduction of the capacity of the globe. Failing in their attempts to bring
the retina to the choroid, operators turned their endeavours to bringing the choroid
into proximity with the retina by excising bands of the Sclera near the equator (Müller,
1903). Török (1920) collected reports of 50 such operations, and he himself performed
it 21 times with no permanent success. In a series of 18, Koch (1927) had one success,
and it has recently been revived as a subsidiary to other methods by Guist
(1933), Hildesheimer (1933), and particularly by Lindner (1933). Vogt (1933) suggested
the formation of a fold in the sclera by suture. Although the operation seems a drastic
one, the excision of such equatorial bands of Sclera involves little permanent functional
damage (Ramach, 1935).
5. The Raising of the Intra-ocular Pressure. Several operative procedures have
been proposed with the purpose of raising the intra-ocular pressure in the hope that
the retina would in this way be re-apposed. The most popular has been that of
colmatage, introduced by Lagrange (1912), wherein, underneath a conjunctival flap
dissected up from the limbus, a triple row of cauterizations were made. The resultant
rise of tension may be due partly to cicatrization in the region of Schlemm's canal
DISEASES OF THE RETINA - 29.13
(Lagrange), or to the formation of a protein-rich plasmoid aqueous (van Heuven,
1926). Reports of success by this procedure were published by Birch-Hirschfeld
(1912), Pesme (1921), Löwenstein (1923), Delorme (1923), van Heuven (1926),
Csapody (1928), Fodor (1929), v. Rötth (1929), among others. An operation of
much the same type was practised by Bettremieux (1928), wherein under a con-
junctival flap in the same region the superficial tissues were dissected out. In
addition to producing a rise of tension, Bettremieux held that the new anastomoses
thus formed increased the nutrition of the eye.
An endeavour to raise the tension of the eye has also been made by the injection
into the anterior chamber of substances which would find exit with difficulty—ox vitreous,
gelatine or glycerine (Carbone, 1925).
6. Increasing the Bulk of the Vitreous. In order to push the retina directly back
upon the choroid, injections into the vitreous cavity of various types have been tried—
rabbit’s vitreous (Deutschmann, 1895), cerebro-spinal fluid, the sub-retinal fluid
(Wood, 1920), paraffin and gelatine (Procksch, 1926), protein bodies (Nakashima, 1926),
and air (Rohmer, 1912; Jeandelize and Baudot, 1926 ; Szymanski, 1933).
7 The closure of the retinal aperture and the simultaneous formation of
chorio-retinal adhesioms.
We have seen that in the small class of detachments which are not
characterized by a retinal hole, reposition of the retina may be hoped for
by prolonged rest, combined, perhaps, with eliminative treatment. In the
large class in which the retina is torn so that free passage is allowed for the
vitreous fluid to the sub-retinal space, conservative measures are usually
useless and every one of the innumerable attempts at reposition so far
described are equally disappointing. It was left to Gonin (1925) to point out
and apply the obvious corollary that if the hole were closed the detachment
ought to be amenable to cure. It is true that Schoeler (1889) injected iodine
at the site of the tear, and that ignipuncture in this region was performed
by Deutschmann (1899) and desultorily by Galezowski (1902); but there is
no question that it is due to Gonin's researches and persistent advocacy
that the treatment of these cases has been revolutionized. At the time of
its introduction the operation created an outcry, for to insert a red-hot
cautery into the eye to heal a hole in a tissue so delicate as the retina was
derided as “therapeutic nihilism,” and “using cannon to shoot sparrows ‘’;
but as a dramatic and revolutionizing operative discovery it can take its
place beside the introduction of iridectomy for glaucoma by v. Graefe.
Gonin first practised the operation in 1916, and made early reports in 1919
and 1923, in 1925 he described 5 cases all cured, in 1928, 26, and in 1931 he
reported on a series of 300 cases. As a result of the stimulus of his work
many modifications of operative procedure have been employed all with the
same end in view, and these we shall shortly review ; the technical details
will be discussed in the section on operative surgery.
They may be classified as follows:–
(a) Obliterating ignipuncture.
(i) By the Paquelin cautery (Gonin, 1925).
(ii) By the galvano-cautery (Vogt, 1929).
3 G 2
2.914 TEXT-BOOK OF OPHTHALMOLOGY
(b) Chemical cauterization of the choroid.
(i) Through multiple trephine openings in the region of the hole
(Guist, 1931).
(ii) Through trephine openings delimiting the hole (Lindner, 1931).
(iii) After undermining the choroid (Lindner, 1933).
(c) Diathermy.
(i) Superficial cauterization (Weve, 1930; Larssen, 1930–32).
(ii) Closure of the hole with micro-punctures (Weve, 1932).
(iii) Multiple delimiting micro-punctures (Safar, 1932).
(d) Electrolysis employed to obliterate the hole.
(i) Bipolar electrolysis (v. Szily and Machemer, 1934).
(ii) Anodal (Imre, 1932; v. Szily and Machemer, 1934).
(iii) Cathodal (Vogt, 1934).
(a) The original operation of ignipuncture practised either with the
Paquelin (Gonin, 1925) or galvano-cautery (Vogt, 1929), which depended
upon the exact localization of the hole on the sclera and transfixing it after
the sub-retinal fluid had been evacuated,
is now replaced by more efficient and
less traumatic methods. Technically
the operation was difficult owing to the
exactitude of the localization required,”
for if the hole was missed, it had to be
repeated at a later date; moreover,
the trauma involved was considerable,
for, although the hole was secluded and
the retina and choroid were soldered
together, considerable atrophy and
puckering frequently resulted, which
ºne ºn sº ºn tº " occasion produced secondary tears.
- cºsmºs. (b) Chemical Cauterization of the
Peripheral area 6 weeks after operation. Choroid. These difficulties led Guist
º ºº potassium hydr- (1931) to evolve a different operation
wherein a more widespread and less
drastic effect would be produced. Adopting, in a sense, the technique of
Schoeler (1889), he cauterized the choroid by touching it with a caustic
potash stick in several places after it had been exposed through multiple
trephine openings through the sclera round the region of the hole, evacuating
the sub-retinal fluid to allow the retina to fall upon the cauterized choroid
(Fig. 2506). While such an operation allows multiple holes to be treated at
one sitting, causes less damage to the retina, does not lead to the formation
of traction strands, and does not require an exact but only an approximate
localization of the retinal tear, it suffers from the disadvantages that it is
tedious and lengthy, and involves the risk of perforating the globe
* For methods of localization, see Vol. II, p. 1180.

DISEASES OF THE RETINA 2915
while trephining at the earlier stages, an accident which means its
postponement.
The method was modified to some extent by Lindner (1930) who threw a barrage
of trephine holes round extensive retinal rents and thus delimited the affected
area. Other modifications in the cauterizing agent have been employed : sodium
hydroxide (Arruga, 1932), abrin and jequiritol (Rubbrecht, 1932), tincture of
iodine and carbon dioxide snow (Deutschmann, 1933; Bietti, 1933–34 ; Gilbert, 1934),
the patient’s blood serum (Guist, 1933), carbolic acid (Foster Moore: see Shapland,
1934). Hildesheimer (1933) excised portions of sclera by diathermy and then applied
the caustic.
The undermining method. Cauterization of the choroid by the injection of a 6%
caustic potash solution underneath it after undermining it by a spatula thrust through
a scleral incision was practised successfully by Lindner (1933), a method useful in
detachments near the posterior pole to
which access for trephining is impossible.
It is a courageous operation which
causes much less permanent damage
than might be thought, for good vision
may result even if the choroid under
the macula has been touched by the
caustic (Fig. 2507).
(c) Diathermy. Cauterization
of the scleral surface over the region
of the hole in the retina to excite
an exudative choroidal reaction,
with subsequent evacuation of the
sub-retinal fluid, was described and
practised successfully by Weve
(1930) and Larssen (1930–32)
(surface diathermy): the former
evacuated the fluid through a tre— Fig. 2507–ScARRING Produced by Caustics.
phine hole, and the latter by The macular area 4 weeks after central
diathermic puncture. Weve (1932) ºssium hydroxide. V =
later evolved a technique of
diathermic puncture somewhat resembling that of Gonin, wherein the hole in
the retina was sealed off by micro-puncture by a diathermy needle, the
operation being performed under visual control. Eventually he combined
the two techniques, employing both surface and puncture applications by
unipolar electrodes (Weve, 1934). -
Safar (1932), in his technique of simultaneous multiple punctures, surrounded the
retinal tear with a barrage of electrodes, while other modifications have been intro-
duced by Walker (1934–35) and practised also by Peter (1934), Post (1934) and others.
Other minor alterations in the form of the electrodes have been suggested by
Gresser (1934) and Schoenberg (1935). Coppez (1932–34) and Meesmann (1934)
employed a pyrometric electrode which incorporated a thermo-couple for puncture to
control the temperature of the tissue at the moment of coagulation; by this means
they claimed they could produce a constant degree of choroiditis; Coppez advised

2916 TEXT-BOOK OF OPHTHALMOLOGY
as a suitable temperature 80°C., and Meesmann 75° C. Lacarrère (1933) used an
electro-diaphake, claiming to get more constant results; but it must be admitted
that the factor of resistance varies so much between different eyes that the only safe
measurement is not instrumental but the actual ophthalmoscopic observation of the
coagulative effect.
(d) Electrolysis. While diathermy acts merely by the heat which is
generated in the tissues, the galvanic current acts chemically through
the action of the acids and bases liberated at the poles. We have already
noted that Schoeler (1893) and Abadie (1893) originally employed
electrolysis in the treatment of detachments without success, largely
because their technique took no consideration of the retinal hole.
Recently, however, with this object in view, the method has been employed
with considerable success. In bipolar electrolysis both electrodes are used,
and when they are kept close together the reaction can be conveniently
limited : the method has been studied experimentally and employed
clinically by v. Szily and Machemer (1934), Machemer (1934) and Hudelo
(1935). The anode has also been employed as the active electrode (Imre,
1932 ; v. Szily and Machemer 1934), while the use of the cathode was advo-
cated by Vogt (1934). This forms a very delicate and non-traumatic method
of sealing the hole, and its accuracy can be ensured by following the procedure
with the ophthalmoscope by observing the bubbles of hydrogen arising from
the site. Probably of all methods it invokes the least damage. Cauteriza-
tion produces local temperatures of between 500° C. and 1,000°C., diathermy
may cause serious burns although the temperature in the globe is never
dangerously high (Bietti, 1933), chemical cauterization may be uncon-
trollable, whereas catholysis deals only with very small quantities of energy.
The scars produced are delicate, secondary holes, secondary cataract, and
late haemorrhages are thus avoided. Moreover, the light needle is easily
inserted and several punctures can safely be made sealing up large or
numerous holes without serious loss of vitreous or damage to the eye, the
whole procedure being under ophthalmoscopic control.
Other Methods. As we have seen, any procedure which will produce an adhesive
choroiditis near the region of the retinal hole is sufficient for the purpose. Heat applied
by the thermophone has been employed by Langdon (1935). Freezing has a similar
effect, for which carbon dioacide snow or the “cryo-cautery ‘’ of Bietti (1934) may be
employed.
The pathological changes following these operative procedures are of
importance. Unfortunately opportunities of obtaining human eyes recently
operated upon have been few, but sufficient material has been studied to
provide an impression of considerable accuracy (Amsler, 1930; Terry, 1932;
Stallard, 1932; Kurz, 1933; Safar, 1933; Levkoeva, 1934; Coppez, 1934;
Fischer, 1934). Moreover, these studies have been amplified by animal
experiments wherein animal eyes have been subjected to ignipuncture,
chemical cauterization, diathermy, and electrolysis (Herzfeld, 1930; Luntz,
DISEASES OF THE RETINA 29.17
1931; Arruga, 1932; Caramazza, 1933; v. Rötth, 1933; Cordero, 1934;
Campos, 1934; Bucalossi, 1934; v. Szily and Machemer, 1934; and others).
It is found that after the first two methods of treatment, very considerable
tissue-destruction takes place, which is more marked in the case of igni-
puncture; when diathermy is used, however, this is much less extensive, unless
the reaction had been unduly vigorous. In the study of the sections of all
cases the striking feature is the slightness of inflammatory changes, a finding
which is well substantiated by clinical post-operative appearances. The
histological picture is one of localized uveitis (Fig. 2508). The sclera shows a
zone of hyaline degenera-
tion around the puncture,
but does not undergo any
considerable tissue-damage
or necrosis. The choroid
beneath the treated site
shows aggregations of
lymphocytes, plasma cells,
and a few giant cells, while
clumps of inflammatory
cells burst through Bruch's
membrane and the retinal
pigment epithelium, which,
combined with aggrega-
tions of fibroblasts, assist
in forming adhesions be- Fig. 2508.-ScLERAL DIATHERMic Coagulation.
tween the retina and Diathermy at 175°C., the eye being enucleated at the
choroid These buds of * day. The centre of the coagulated area (Coppez,
- - T. O. S.).
granulation tissue hermiat-
ing through Bruch's membrane, act as grappling irons securing the detached
retina to the choroid, a process which is eventually consolidated by chorio-
retinal adhesions of fibrous tissue. The retina itself shows degeneration of
the rods and cones over the area of coagulation, and its disorganized structure
becomes infiltrated with fibroblasts and plasma cells, while clumps of large
mononuclear leucocytes, polymorphs, lymphocytes, and strands of fibrin are
present on the surface and in the structure of the vitreous body. This latter
is usually drawn forwards and adheres to the retina at and near the site of
the application. It is to be remembered, of course, that when this process
is induced near the ora serrata the immediate reaction produces a cyclitis,
while an excessive amount of fibrous tissue formation round the circumlental
space may not be altogether without future complications.
Unfortunately at the moment most of these procedures are too recent
to allow any final verdict upon them ; they are still in the experimental stage.
As the position stands it is probable that the ideal operation has not yet been

29.18 TEXT-BOOK OF OPHTHALMOLOGY
devised. The impetus to new work which the initial success of Gonin's
procedure provided has not yet been expended, and further developments
will without doubt arise. The essentials of a good operation are that it
should be easy of performance, that it should be widely applicable, that the
choroidal reaction be controllable and the retinal damage as little as possible,
that the hole be closed and the diseased retinal area shut off, that the sub-
retinal fluid be drained for some days, and that it be repeatāble after a short
interval.
It is probable in the present position of the evolution of the subject that
where a small hole in a relatively healthy retina is involved, or in cases of
macular holes, electrolysis with its little trauma and dainty scarring is the
method of choice. When large or multiple holes occur, or when considerable
areas of the retina show atrophic or inflammatory changes, the combination
of surface and puncture diathermy as elaborated by Weve (1934-39) with
such magnificent success is most efficient. The features of this technique
are the preliminary accurate localization of the retinal holes, their localization
at the time of operation by trans-illumination and ophthalmoscopy (Weve,
1934), the constant ophthalmoscopic control during the entire operation,
the confinement of the surface diathermy (using a unipolar electrode) to the
required area, the withdrawal of the sub-retinal fluid through a diathermic
puncture by suction, and the verification after the operation not only that
the hole has been closed but that the retina is lying in apposition to the
choroid, a consummation which can be achieved, if necessary, by the injection
of air into the vitreous (Arruga, 1933 ; Rosengren, 1938).
Prognosis -
Although it is true that with the new operative procedures introduced
in modern times, the outlook in cases of detachment of the retina has changed
from one of almost complete hopelessness and impotence, still the prognosis
is always grave : the condition is one of the major catastrophies of
ophthalmology. When the detachment is complicated by ocular disease of
a gross nature—tumour, cicatricial formation, phthisis bulbi, advančed
retinal disease, very severe trauma, and so on—the fact of the detachment
takes a secondary place in the general prognosis. When no very gross ocular
disease is present and the detachment becomes important rather than
incidental, the prognosis varies as to whether it is exudative in nature
without a retinal tear or serous with a retinal tear. In the first case, when
medical treatment is directed towards an absorption of the exudate, the
prognosis is frequently good if the causal lesion can be cured ; if it cannot,
the prognosis is bad. Thus a detachment following on orbital phlegmon
will probably clear up on evacuation of the abscess, one due to renal
conditions will probably disappear if these cases be relieved as may occur
in the toxaemia of pregnancy, but the detachment will probably not
resolve in the presence of advanced arterial disease when the renal condition
DISEASES OF THE RETINA 29 19
is terminal. In the second case, where a hole is present, the entire prognosis
depends upon the success of operative measures.
In the literature before 1925 quite a number of cures of retinal detach-
ments had been reported, either with rest or with the active methods of
treatment already described which were in vogue before operative closure of
the retinal hole was practised : some of these were spontaneous cures, most of
them were exudative detachments, and in practically none was a hole
present. Statistics vary according as to the type of detachment included.
The Committee appointed by the Ophthalmological Society of the United
Kingdom (1916) collected 85 cures, of which only 3 had a retinal hole.
Vail (1912), in America, circularized 460 oculists ; 250 of the 281 who
replied had never treated a detachment successfully ; 31 oculists had in
their lives cured 41 detachments ; the prospect of cure in the series is
therefore less than 1 in 1000. On the continent of Europe, Leber (1916)
found that 8.5% were cured anatomically, but only 3.6% regained
moderately useful vision ; Seible (1916) reported 6.5% cures in 186 eyes,
Darier (1920) 16% in 103 cases, Schreiber (1920) 7.5% in 186 eyes, Uhthoff
(1922) 9%. Gathering statistics of the world literature, Ovio (1933) found
that in all types of cases an average of about 22% of improvements and
cures have been noted after medical treatment.
The prognosis after operative treatment is very much better in suitable
cases. A large number of statistics have recently been published of which
the following is a selection taken from different countries.
Ignipuncture (Gonin's method and modifications). Bruckner (1929), 4.2%
recoveries or very great improvement in 12 cases ; Vogt (1930), 42% in 26 cases;
Meller (1930), 12% in 50 cases; Lindner (1930), 39% in 33 cases; Amsler (1929), 41%
in 29 cases ; Bielschowsky (1931), 11% in 18 cases; Stein (1931), 24% in 41 cases;
Wessely (1931), 17% in 18 cases; Clausen (1932), 25% in 30 cases; Gonin (1932), 53%
in 221 cases; Denti (1933), 50% in 12 cases; Rieger (1933), 29% in 90 cases; v. Hippel
(1934), 55% in l l cases; Gradle and Meyer (1936), 42% in 19 cases. The Moorfields
statistics are: Shapland (1931), 40% in 100 cases; Doggart and Shapland (1931), 32%
in 75 cases; Shapland (1934), 30% in 221 cases.
Chemical cauterization (Guist-Lindner method and modifications). Lindner (1931)
68% in 19 cases; Guist (1932), 55% in 109 cases and (1933) 85% ; McKeown (1933),
39% in 51 cases; Terrien, Veil and Dollfus (1933), 30% in 20 cases; Rieger (1933),
47% in 81 cases; Dunnington and Macnie (1935), 47% in 92 cases. The Moorfields
statistics are Black (1932), 40% in 39 cases; King (1933), 21% in 42 cases; Shapland
(1934), 26% in 79 cases.
Diathermy . Surface diathermy (Larssen method and modifications). Larssen
(1932), 50% in 40 cases; (1934), 52% in 98 cases; Weve during 1930–1 (Visser-Heerema
and Weve, 1935), 48% in 23 cases. The Moorfields statistics are : King (1933), 58%
in 31 cases ; Shapland (1934), 43.5% in 131 cases.’
Multiple Puncture (Safar and Modifications). Safar (1933), 60% in 40 cases and
(1934), 70% in 80 cases; Jeandelize and Baudot (1933), 83% in 12 cases; Marshall
(1933), 60% in 39 cases; Knapp (1933), 66% in 12 cases; Schoenberg (1934), 60% in
23 cases; Walker (1935), 81% in 21 cases.
Surface and Puncture (Weve Method and Modifications). Saint-Martin (1933), 62%
2920 TEXT-BOOK OF OPHTHALMOLOGY
in 13 cases; Meesmann (1934), 52% in 50 cases; v. Hippel (1934), 53% in 19 cases;
Dunnington and Macnie (1935), 38% in 18 cases; Gradle and Meyer (1936), 65% in
24 cases. Weve's (1939) evolutionary statistics in 750 cases are remarkable and unique:
1930–1, 48% ; 1932, 63% ; 1933, 72% ; 1934, 75% ; 1935, 77% ; 1936, 75% ;
1937, 80% ; 1938, 81% of all cases and 90% of fresh cases.
In sum, it may be said that approacimately 50% of all cases of detachment
can be cured, and it is interesting that in some cases re-attachment may not
occur until some weeks after the operation when the patient is moving about
again (Rieger, 1933; Stallard, 1935). So far as favourable cases are con-
cerned approximately 75% should be cured, and in the hands of an expert
this figure may rise to 80% or even higher. There is, however, a residuum
of cases which seems completely to fail to respond, no matter what treatment
is adopted and no matter how whole-heartedly the surgeon and the patient
co-operate in its application. -
In any assessment, however, two considerations are important—the
age of the detachment and the state of the eye.
With regard to the time factor, after a preliminary day or two of rest
to allow the retina to settle down as much as may be and to permit several
repeated and meticulous fundus examinations, the sooner the operation is
done the better. As the duration of the detachment increases the prognosis
gets worse.
Gonin (1931), for example, gives the following percentage recovery : less than
3 weeks’ duration, 55% ; up to 3 months’, 48% ; up to 1 year, 40%. For the same
operation Shapland (1934) gives 40% within 6 weeks; 28%, 6 weeks to 3 months;
22%, 3 to 6 months; and 10% over 6 months. Weve (1932) writes similarly : 85%
under 2 months and 33% over 2 months. At the same time this is not invariable,
and extreme exceptions have occurred ; a bilateral case of my own with several holes
was reposed after 5 years’ duration, the visual result, however, being poor, and Vogt.
(1934) reported one of 73 years' standing.
So far as the eye is concerned, the healthier the eye the better the
prognosis. Young emmetropes are the most suitable subjects, the chances
of cure receding as age or myopia with their accompanying degenerative
changes increases: over 50 years of age the presence of degenerative changes
is universal, and the prognosis becomes progressively worse. Apart from
disease of the retina, the number, size and accessibility of the retinal holes are
a further matter of importance ; and finally—and not least—the ability of
the patient to endure a trying and prolonged convalescence, with the
possibility at its end of having to submit to a second operation. Given a
reasonable and healthy patient with a relatively healthy eye and a recent
detachment showing few and readily accessible holes, the chances of opera-
tive reposition are considerably higher than 75%. The prognosis, however,
should always be guarded ; the patient must go in for the operation in the
spirit that he is taking a chance for which he, for his part, is prepared to do
a lot to make a winning chance.
DISEASES OF THE RETINA 2921
The functional results after a successful operation are good if the detach-
ment is relatively recent, for the degree of improvement depends more on
the duration of the disease than on any other single factor. The field for
white returns almost immediately, leaving perhaps a small scotoma at the
site of operation ; the field for red returns next, and blue last, tritanopia
sometimes lasting for some time, so that the normal relationship of the
colour fields may take some years to re-establish itself (Sallmann and
Sveinsson, 1934). The character and extent of the detachment seem to have
little bearing on the recovery of the field, but if the age of the lesion
is reckoned in years, recovery may be disappointing or nil. A late operation,
however, may be justified in a partial detachment, in an attempt to maintain
the status quo, and to prevent the calamity of the gradual development of
complete blindness. The central vision usually returns to little short of
normal in recent cases, provided the macular area has not been involved.
In macular detachments, however, the central vision is usually seriously
impaired, especially if it has persisted for any length of time, a disability
perhaps due to the development of cystic degeneration (Reese, 1937). A fair
degree of recovery may result if the duration has been less than two months
(Kronfeld, 1933; Stallard, 1933), although it sometimes takes several
months to do so ; more usually, however, it remains about the level of
6/36. When a hole has existed at the macula and has been successfully
treated, the functional result, as has already been noted, is sometimes
Surprisingly good.
When the eye is grossly diseased, and an extensive detachment with
enormous rents are present, the prospects of successful treatment are, of
course, remote, and the inevitable must be submitted to. In these patients
the relative peace of complete blindness may be distressingly disturbed by a
persistent and annoying photopsiae, to relieve which, section of the optic
nerve or its injection with alcohol may sometimes provide a less distasteful
alternative than excision of the eye.
Abadie. Gaz. hebd., 49, 1882. YIV Inter. Cong. O., ii (1), 5, 1933.
Am... d’Oc., cii, 203, 1889; cz, 36, 1893. R. M. Aug., xciii, 52, 1934.
Addario. Inter. Cong. O., Lucerne, p. 141, A. of O., xiii, 523, 1935: xviii, 501, 1937.
1904. Asayama. A. f. O., liv, 444, 1902.
Allan. Am. J. O., xix, 1000, 1936. Aschner. Münch. med. W., lxxvi, 2135, 1929.
Ammann. Z. f. Aug., xi, 406, 1904. Aust. A. f. O., cxxix, 17, 1932.
K. M. Aug., lxiii, 80, 1919. Axenfeld. K. M. Aug., xl, Beil., 5, 1903.
Amsler. Am. d’Oc., clxvi, 871, 1929; clºvii, Hb. d. Aerz. Erfahrungen in Weltkriege, v,
115, 1930. 1922.
K. M. Aug., lxxxiv, 113, 1930; lxxxvi, 1, Aykai. K. M. Aug., lxvi, 951, 1921.
1931. Bach. A. f. O., xli (3), 62, 1895.
Anderson. Detachment of the Retina, London, Ballantyne. T. O. S., li, 113, 1931.
1931. - Bane. Am. J. O., xiii, 1004, 1930.
Brit. J. O., xvi, 641, 705, 1932. Baquis. An. di Ott., xxv, 241, 1896.
Arlt. Krankheiten d. Auges, ii, 158, 1853. Barrenechea. Rev. Cub. Oft., iv, 62, 1922.
Am. J. O., v, 355, 1888. Bartels. B. O. G. Leipzig, xlix, 477, 1932.
Arruga. A. de Oft. H.-A., xxix, 581, 1929; R. M. Aug., xci, 437, 1933; xcvi, l, 687,
xxxii, 461, 614, 1932. 1936.
Bull. S. fr. d’O., xliv, 461, 1931. Baurmann. K. M. Aug., lxxx, 682, 1928.
X VII Ass. S. Oft. H. -A., xvii, 1932. A. f. O., cxii, 415, 1929.
2922
TEXT-BOOK OF OPHTHALMOLOGY
Becker.
1874
K. M. Aug., lxviii, 240, 1922.
Beer. Lehre v. d. Augenkrankheiten, ii, 495,
1817.
Beigelman. A. f. O., i, 463, 1929.
Belarminow. Russ. Wraksch., ii, Nr. 23, 1903.
Bergmeister. Z. f. Aug., xlii, 254, 1919.
Berlin. . K. M. Aug., iv, 77, 1866.
Best. B. O. G. Heidel.., xxxiii, 186, 1906.
Bettremieux. An. d’Oc., clxv, 914, 1928.
A. d’O., xlv., 773, 1928.
Bielschowsky. K. M. Aug., lxxxvi, 402, 1931.
Bietti. XIV Inter. Cong. O., ii (2), 205, 1933.
Boll. d’Oc., xii, 1427, 1933; xiii, 576, 1934.
Birch-Hirschfeld. A. f. O., lxxix, 210, 1912.
Birch-Hirschfeld and Inouye. A. f. O., lxx,
486, 1909.
Black. T. O. S., lii, 486, 1932.
Boucheron. Bull. S. fr. d’O., ii, 59, 1884.
Bousseau. Le Décollement de la Rétine, Paris,
1932.
Brinton. Am. J. O., v, 806, 1922.
Brons. Z. f. A ug., liv, 117, 1924.
Bruckner. K. M. Aug., lxxxiii, 670, 1929.
Bucalossi. An. di Ott., lxii, 1039, 1934.
Buchanan. A. of O., xvii, 947, 1937.
Campos. An. di Ott., lxii, 360, 1934.
Caramazza. Boll. d’Oc., xii, 1357, 1933.
Ras. It. Ott., ii, 1123, 1933.
Carbone. Atti Cong. Oft. It.,
R. M. Aug., lxxv, 828, 1925.
Cassidy and Gifford. Am. J. O., v, 434,
1922.
Castresana. A. de Oft. H.-A., xii, 289, 1912.
Castroviejo. XIV Inter. Cong. O., ii (2), 96,
1933.
Am. J. O., xvii, 1112, 1934.
Chelius. Hb. d. Augenheilk., ii, 366, 1839.
Chipman. Canad. Med. J., x, 1007, 1921.
Chodin. Zb. f. d. med. Wiss., xiii, 68, 1875.
Clarke. T. O. S., xviii, 136, 1898.
Clausen. Z. f. Aug., lxxi, 119, 1930.
B. O. G. Heidel., xlviii, 63, 1930.
R. M. Aug., lxxxviii, 811, 1932.
Coccius. U. d. Anwendung d. Augenspiegels,
125, 1853.
Collins. R. L. O. H. Rep., xiii, 381, 1892.
T. O. S., xvi, 81, 1896; xx, 196, 1900;
xxxvii, 236, 1917.
Comberg. K. M. Aug., lxxii, 692, 1924.
Committee O. Soc. U. K. T. O. S., xxxvi, 352,
I916.
Constentin. Inter. Cong. O., Lucerne, 181,
1904.
Coppez. Bull. S. fr. d’O., xlv., 281, 1932.
A. d’O., I, 593, 598, 1933.
Bull. S. Belg. d’O., lxix., 119, 1934.
A. Int. de Méd. Earp., ix, 177, 1934.
A. of O., xiii, 1, 1935.
Cordero. A. di Ott., xli., 65, 1934.
Cords. K. M. Aug., lxxxiv, 222, 1930.
Cramer. Z. f. Aug., xiii, 31, 1905.
K. M. Aug., lxviii, 240, 1922.
Cruise. Am. J. O., vi, 140, 1923.
Atlas d. path. Topographie d. Auges,
1925 ; Ref.
Csapody. K. M. Aug., lxxx, 641, 1928;
lxxxviii, 783, 1932.
Damel. Sem. Med., xxviii, 478, 1921.
Darier. Cong. Inter. de Méd., xii, 241, 1898.
La Clin. Opht., xxiv, 10, 1920.
de Decker. A. f. Aug., c.—ci, 223, 1929.
Dejean. XIV Inter. Cong. O., Madrid, ii (2),
118, 1934.
Delorme. A. d’O., xl, 166, 1923.
Denti. Boll. d’Oc., xii, 42, 1933.
Desmarres. Traité d. malad. d. yewa:, 1847.
Deutschmann. Beit. z. Aug., ii, 850, 1895;
xx, 1, 1895; iv, 658, 1899; xl, 1, 1899;
vi, 739, 1904.
B. O. G. Heidel., xxxi, 258, 1904.
A. f. O., lxxiv, 206, 1910; czXii, 359, 1929.
Münch. med. W., lxxvii, 102, 1930.
K. M. Aug., lxxxviii, 441, 1932; xci,
450, 1933.
ten Doesschate. Ned. Tij. v. Gen., lxx, 211,
1926.
Doggart and Shapland. Brit. J. O., xv, 257,
1931.
. Dollfus, Veil and Desirgnes. A. d’O., lii, 307,
1935.
Dollfus and Veil. Bull. S. d’O. Paris, xliv,
235, 1932.
Donders. Anomalies d. Refraktion w. Akkom-
modation d. Auges, Wien, 1866.
Bull. S. fr. d’O., xiii, 181, 1895; xxiv,
342, 1907.
Rev. gen. d’O., xxv, 447, 1921.
Dor, H. Bull. S. fr. d’O., xiv, 396, 1896.
Dor, L. An. d’Oc., czzvii, 383, 1902; czXxvii,
440, 1907.
Bull. S. fr. d’O., xxvii, 224, 1910.
Doyne. O. Rev., xxi, 108, 1902.
Duke-Elder. The Nature of the
Body, London, 1930.
Duke-Elder and Robertson. Proc. R. S. (B),
cxii. 215, 1933.
Brit. J. O., xviii, 433, 1934.
Dunn. A. f. O., xxv, 1, 1896.
Dunnington and Macnie. A. of O., xiii, 191,
1935; xviii, 532, 1937.
Elschnig. K. M. Aug., xxx, 416, 1892;
xlii (2), 529, 1904.
A. f. Aug., lxxvii, 6, 252, 1914.
Fehr. A. f. O., lxxxv, 336, 1913.
Fischer. Z. f. Aug., lxxxiii, 95, 1934.
Fleischer. G. D. Naturf. Aerz., ii (2), 288,
I907.
K. M. Aug., lxxiv, 196, 1925.
Z. f. Aug., lxviii, 375, 1926.
Fodor. K. M. Aug., lxxxiii, 360, 1929.
Orvosi hetel., lxxvii, 1111, 1933.
Fränkel. K. M. Aug., xxxiii, 410, 1895.
Frank. K. M. Aug., lxiv, 143, 1920.
Frenkel. Rev. gen. d’O., xxxiv, 1, 1920.
Friedenwald. Bull. Johns Hop. H., xl, 201,
1927.
A. of O., i, 575, 1929.
Friedman. A. of O., xvii, 382, 1937.
Fromaget. An d’O., exv, 267, 1896.
Fry. A. of O., i, 609, 1929.
Dor.
|Vitreous
DESEASES OF
THE RETINA 2923
Fuchs, E. A. f. O., ci, 265, 1920; civ, 230,
1921.
T.-B. of Oph., VII Ed., 1923.
R. M. Aug., lxxxiv, 39, 1930.
Fuchs, A. K. M. Aug., xcviii, 145, 1937.
Galezowski. Rec. d’O., 669, 694, 1883; 1,
1890; 385, 1895.
Cong. Chir., Paris, xv, 417, 1902.
Gallois. A. d’O., xliii, 532, 1926.
Gaupillat. A. d’O., viii, 271, 1888.
Giannini. A. dº Ott., lxii, 1024, 1934.
Giesler. K. M. Aug., lxxiv, 776, 1925.
Gifford. A. of O., xvi, 405, 1936.
Gilbert. A. f. Aug., lxxxvi, 282, 1920.
R. M. Aug., xciii, 689, 1934.
Ginsberg and Simon. Cb. pr. O., xxii, 161,
1898.
Goldenburg. A. of O., ix, 256, 1933.
Gonin. An. d’Oc., czzxii, 30, 1904; clvi, 281,
1919; clviii, 175, 1921; clziv, 817, 1927;
clxvii, 361, 1930; clzviii, 689, 1931.
Rap. S. fr. d’O., 1920.
K. M. Aug., lxxi, 232, 1923; lxxxiii, 667,
1928.
Bull. S. fr. d’O., xxxviii, 614, 1925;
275, 1928; xliii, 324, 1930.
A. d’O., xlv., 554, 1928; xlviii, 487, 1931 ;
xlix, 289, 1932; 1, 336, 1933; li, 426,
1934.
T. O. S., l, 531, 1930.
A. of O., iv, 621, 1930.
Am. J. O., xvii, 74, 1934.
Le décollement de la Rétine, Lausanne, 1934.
Gourfein-Welt. K. M. Aug., lxx, 565, 1923.
xli,
Gradenigo. Scritti ottal., Padova, 1894.
Gradle and Meyer. Am. J. O., xix, 873,
1936.
v. Graefe. A. f. O., i (1), 362, 1854; ii (1),
222, 1855; ii (1), 277, 1857; ix (2), 88,
III, 1863.
R. M. Aug., i, 49, 1863.
de Grandmont. A. d’O., xiii, 337, 1894.
Greeff. A. f. Aug., liii, 119, 1905; c.—ci, 116,
1929.
Gresser. Am. J. O., xvii, 340, 1934.
Grönholm. A. f. O., cv, 899, 1921.
Grossmann. A. d’O., iii, 122, 1883.
Grüner. Ges. deut. Naturf. Aerz., Sept., 1928.
Guende. Bull. S. fr. d’O., viii, 137, 1890.
Guist. Z. f. Aug., lxxiii, 389 ; lxxiv, 232,
1931.
K. M. Aug., lxxxviii, 810, 1932;
1933.
A. de Oft. B.-A., viii, 429, 1933.
Haab. Z. f. Aug., iii, 113, 1900.
Haedicke. Diss., Berlin, 1898.
Hagedoorn. Wiederländ. Aug. G., Amsterdam,
1930.
Am. J. O., xvii, 400, 1934.
xc, 771,
Haitz. K. M. Aug., lxxxvi, 105, 1931.
Hamburger. Münch. med. W., lxxv, 2208,
1928,
Hamilton. Brit. J, O., xiv, 455, 1930.
Hamma. A. f. Aug., lxvii, 183, 1910.
Hann and Knaggs. Lancet, i, 1391, 1901.
Hanssen. K. M. Aug., lxiii, 295, 1919;
lxxiv, 778, 1925; lxxv, 344, 1925; lxxxii,
40, 1929.
Harman. T. O. S., xxi, 88, 1901.
Harman and Macdonald. B. Med. J., 637,
1922.
Heine. K. M. Aug., lxxii, 305, 1924.
Z. f. Aug., lvi, 155, 1925.
Helbron. Ber. k. W., xxxix, 69, 103, 1902.
Helming. Diss., Giessen, 1915.
Hemmes. Ned. Tij. v. Gen., lxxv, 4738, 1931.
Herzfeld. A. of O., iv, 298, 1930.
Hess. A. f. Aug., lxxiv, 227, 1913.
Hessberg. Z. f. Aug., liii, 118, 1924.
v. Heuven. K. M. Aug., lxxvi, 340, 1926.
Highet. Brit. J. O., viii, 226, 1924.
Hildesheimer. XIV Inter. Cong. O., ii (2), 52,
1933.
Hill. A. of O., liii, 137, 1924.
v. Hippel. D. med. W., xxiii, 395, 1897.
A. f. O., xlix (2), 387, 1899; li, 132, 1900;
467, 1901; lzviii, 38, 1908.
B. O. G. Heidel.., xxxix, 385, 1913.
K. M. Aug., xcii, 145, 1934.
Hirschberg. Klim. Beob. a. d. Auges, Wien,
59, 1874.
Cb. pr. Aug., xv, 168, 1891; xxi, 65, 1897.
Hofe. K. M. Aug., xciii, 745, 1934.
Holden. A. f. Aug., xxxi, 287, 1895.
Holth. K. M. Aug., lxxvi, 510, 1926.
Hopner. A. f. O., lxx, 1, 1909.
Horstmann. K. M. Aug., xvii, 487, 1879.
B. O. G. Heidel., xxi, 140, 1891.
A. f. Avg., xxxvi, 166, 1898.
Hudelo. Bull. S. d’O. Paris, xlvii, 642, 1935.
Igersheimer. K. M. Aug., lxxxiii, 669, 1929.
Imre. K. M. Aug., lxxxix, 545, 1932.
Orv. Hetil., 245, 1932.
Iwanoff. A. f. O., xv (2), 1, 1869.
Jablonski. Münch. med. W., ii, 1762, 1929.
v. Jäger. Ophth. Handatlas, 121, 1869.
Jan, Maitre. Traité des maladies de l’oeil,
Troyes, 241, 331, 1722.
Jasinski. XIV Inter. Cong. O., ii (2), 89, 1933.
Jeandelize and Baudot. A. d’O., xliii, 413,
1926 ; xlvii, 764, 1930; 1, 793, 1933.
Bull. S. fr. d’O., xlv, 245, 1932.
Jeandelize, Baudot and Delaveuve. Bull.
S. O. Paris, xliii, 202, 1931.
Jeandelize, Baudot and Gault. Bull. S. fr.
d’O., xlvii, 338, 1934 ; xlix, 269, 1936.
Kamocki. Cb. pr. Aug., xvi, 15, 1892.
Kennon. Virg. Med. Monthly, xlvii, 175,
1920.
Kerry. Canad. Med. J., xviii, 62, 1928.
Kerschbaumer. A. f. O., xxxiv. (4), 16, 1888.
Key. Sven. Läk.-săllok. Hall., lviii, 165, 201,
1932.
King. Brit. J. O., xvii, 287, 1933.
Klainguti. Z. f. Awg., l, 71, 1923.
Klein. K. M. Aug., lxxxiii, 489, 1929.
A. of O., xiii, 250, 1935.
Kleiner. A. f. O., cxxix, 485, 1933.
Knapp. A. f. Aug. Ohrenh., 1, 22, 1869.
A. of O., x, 733, 1933; xvi, 770, 1936.
2924
TEXT-BOOK OF OPHTHALMOLOGY
Knapp, P. K. M. Aug., lxxxvii, 399, 1931.
Rnobloch. Cesk. Oft., i, 20, 1933.
Koby. Slit-lamp Microscopy of the Living
Eye, London, 1925.
Koch. K. M. Aug., lxxix, 138, 1927.
Komoto. Am. J. O., ix, 414, 1926.
Rornzweig. A. of O., xxiii, 491, 1940.
IGoyanagi. K. M. Aug., lxxx, 436, 1928.
A. f. O., cxxxii, 353, 1934.
Rronfeld. A. of O., x, 646, 1933; xiii, 779,
1935.
Euhnt. B. O. G. Heidel., xiii, 38, 1881.
Rümmell. A. f. O., lxxxiv, 317, 1913.
B. O. G. Heidel.., xlii, 231, 1920.
K. M. Aug., lxvii, 180, 1921; Ixxiii, 776,
1924.
A. f. Aug., xcv, 214, 1925; c.—ci, 314, 1929.
XC Versam. Ges. dewt. Naturf. w. Aerzte,
Hamburg, 1928; Ref. Zb. ges. O., xx,
633, 1929. -
Kurz. Cesk. Oft., i, 66, 1933.
Lacarrère. XIV Inter. Cong. O., ii (2), 92,
1933.
Lagrange. Rév. gen. d’O., xxxi, 379, 1912.
Atlas d’Ophtalmoscopie de guerre, Paris,
1918.
Lamb. Am. J. O., iv, 668, 1921.
Lamb and Ziegler. Am. J. O., iv, 668, 1921.
Lambert. A. of O., lii, 267, 1923.
Langdon. Am. J. O., xviii, 550, 1935.
Larssen. Acta O., viii, 172, 1930; x, 173,
1932.
A. of O., vii, 661, 1932.
B. O. G. Heidel., 1, 110, 1934.
Lauber. Z. f. Aug., xx, 118, 208, 1908.
K. M. Aug., lxx, 246, 1923.
Lawford. R. L. O. H. Rep., xi, 208, 1887.
Lawson. T. O. S., xxxvii, 231, 1917; xliv,
96, 1924. -
Leber. G.-S. Hb., I, v, 693, 1877; II,
vii A. (2), 1374, 1916.
Inter. Med. Cong., London, iii, 75, 1882.
B. O. G. Heidel., xiv., 18, 1882; xxxv, 120,
1908.
Levkoeva. Sovet. vest. O., iv, 567, 1934.
A. f. O., czzxiii, 467, 1935.
Liebreich. Atlas d. Ophthalmoscopie, 17, 1863.
Lindahl. K. M. Aug., lxv, 11, 1920.
Lindner. B. O. G. Heidel., xlvii, 53, 1930;
xlix, 83, 476, 1932.
A. f.O., czzvii, 117, 177, 1931; cz.xviii, 654;
cxxix, 138, 1932; czXxiii, 476, 1935.
Z. f. Aug., lxxvii, 125 ; lxxviii, 303 ;
lxxix, 191, 408, 1932; lxxxi, 111, 186,
277, 1933.
K. M. Aug., xc, 289, 757, 1933.
A. of O., xi, 148, 1934.
Wien. k. W., i, 240, 1935.
Lister. Brit. J. O., viii, 1, 1924.
B. Med. J., ii, 1127, 1927.
Lobeck. A. f. O., czzviii, 513, 1932.
Löhlein. Zb. g. O., xxiv, 289, 1931.
Löwenstein. A. f. Aug., lxx, 26, 1911.
K. M. Aug., lxxi, 769, 1923; lxxiii, 784,
1924. +
A. f. O., czvii, 130, 1926.
Luntz. Z. f. Aug., lxxiii, 380, 388, 1931.
MacCallan. P.R. S. Med., Sect. O., xix, 39,
1926.
Machemer. K. M. Aug., xciii, 489, 1934.
Maertens. Diss., Koln, 1937.
Maggiore. An. di Ott., li, 1, 1923; lxiv, 361,
1936.
Magitot. A. of O., xi, 159, 1934.
Magitot and Lemoir. Bull. S. fr. d’O., xlv.,
225, 1932.
Magnus. Die Blindheit, 1883.
Mamoli. An. d’Oc., clxxiv, 309, 1937.
v. Manen and Weve. Bull. S. fr. d’O., xcix,
281, 1936.
Manes. A. d. Oft. B.-A., vii, 742, 1932.
Maraval. La Clin. Opht., vii, 260, 1901.
Marquez. A. de Oft. H.-A., xxxiii, 672, 1933.
Marshall. P. R. S. Med., xxvi, 755, 1933.
Brit. J. O., xxiii, 369, 1939.
Martin. T. Internat. Cong. Med., iii, 110,
1881.
Am. J. O., xii, 652, 1929.
Marx. A. f. O., cviii, 237, 1922.
Mayer. T. Am. Acad. O. and Otolaryn., 89,
1931.
A. of O., vii, 499, 1932.
McDavitt. South. Med. J., xiii, 377, 1920.
McKeown. A. of O., ix, 64, 1933.
McNabb. T. O. S., xlix, 463, 1929.
Meesmann. K. M. Aug., lxv, 922, 1920.
Atlas, Berlin, 1927.
B. O. G. Heidel., 1, 121, 130, 1934.
Meisner. K. M. Aug., lxx, 722,
lxxiv, 178, 1925.
Meller. Oph. Surg. (Sweet), p. 311, 1923.
2. f. Awg., lxx, 207, 1930.
Mellinger. Jb. d. Aug., Basel,
1896.
Mendoza.
Middleton.
1923;
xxxii, 82,
Rev. Cub. de O., ii, 143, 1920.
Am. J. O., ii, 779, 1919.
Milles. R. L. O. H. Rep., xi, 48, 1886.
Moore, Foster,. Lancet, coi, 174, 1921.
Medical Ophthalmology, London, 1925.
Mooren. Funf Lustren. O. Wirk., viii, 224,
1882.
Morax. A. d’O., xxxvii, 374, 1920.
Morgagni. Epist. Anat., xviii, 1740.
Müller, H. A. f. O., iv. (1), 363, 1858.
Müller, Hans. Z. f. Aug., lxxiii, 163, 1931.
Müller, L. K. M. Aug., xli (1), 459, 1903.
Nakashima. A. f. O., czvi, 403, 1926.
Neame. P. R. S. Med., xxxii, 25, 1928.
Neblett. Am. J. O., vii, 564, 1924.
z. Nedden. K. M. Aug., xcvii, 236, 1936.
Noiszewski. Clin. Oczema, iii, 1, 1923; Ref.
K. M. Aug., lxxi, 271, 1923.
Nordenson. Die Netzhautablösung, Wies-
baden, 1887.
Nuel. A. d’O., xvi, 593, 1896.
Ochi. Nip. Gank. Zas., July, 1921. Ref:
- R. M. Aug., lxix, 160, 1922.
Ohm. D. med. W., xliii, 748, 1917.
Oncken. Z. f. Aug., xiv, 165, 1905.
Oradovskaya, Przhibylskaya and Skorod-
inskaya. Sov. vest. O., iv, 388, 1934.
Orlandei. An. di Ott., l, 445, 1922.
IDISEASES OF
THE RETINA 2925
Otto. A. f. O., xliii (3), 543, 1897.
Ovio. XIV Inter. Cong. O., ii (1), 1, 1933.
Pagenstecher, A. Klin. Beobachtungen, ii, 74,
1862.
Pagenstecher, H. A. f. O., lxxxvi, 457, 1913.
Panizza. Sul fungo midoll. dell'occhio, Pavia,
1826. -
Parker. J. Am. Med. As., Sect. O., 163, 1915.
Pascheff. B. O. G. Heidel.., xlvii, 315, 1928.
A. d’O., lii, 717, 1935.
Paul. K. M. Aug., xliii (1), 185, 1905.
Pavia and Dusseldorf. Rev. oto.-mewr.-Oc.,
viii, 356, 1933.
Pesme. A. d’O., xxxviii, 591, 1921.
Bull. Soc. fr. d’O., xliii, 301, 1930.
Peter. A. of O., xi, 262, 1934.
Pfalz. Z. f. Aug., xii, 386, 1904.
Pichler. Wien. med. W., lxxv, 2544, 1925.
Pischel. Am. J. O., xix, 795, 1936 ; xxii,
130, 1939.
Poncet. Bull. S. fr. d’O., v, 67, 1887.
Poos. Am. J. O., xi, 299, 1928.
Post. Am. J. O., xvii, 1122, 1934.
Procksch. Z. f. Aug., lviii, 444, 1926.
Purtscher. Cb. pr. Awg., xv, 334, 1891.
Raeder. K. M. Aug., lxxiv, 424, 1925.
Raehlmann. A. f. O., xxii (4), 233, 1876.
A. f. Aug., xxvii, l, 1893.
Ramach. A. f. O., czkxiii, 321, 1935.
Ramsay. T. O. S., xxvi, 79, 1906.
Rauh. Z. f. Awg., lxiii, 48, 1927.
Redslob. Bull. S. d’O. Paris, xliii, 211, 1931.
Le Corps Vitré; Rap. S. fr. d’O., 1932.
Reese. Am. J. O., xx, 591, 1937.
Reichling. A. f. Aug., lxxxiv, 118, 1930;
cv, 468, 1932.
Remak. Cb. pr. Aug., xxxi, 262, 1907.
Richner. A. f. O., czz.xv, 49, 1936.
Ridley. Brit. J. O., xix, 101, 1935.
Rieger. A. f. O., czkxi, 410, 1933.
Roberts. T. O. S., xxxix, 371, 1919.
Robertson, Argyll. R. L. O. H. Rep., viii,
404, 1876.
Rockliffe. T. O. S., xviii, 139, 1898.
Rohmer. A. d’O., xxxii, 257, 1912.
Rollet. A. d’O., li, 431, 1934.
Rollet and Rosnoblet. A. d’O., xli., 48, 1924.
Römer. K. M. Aug., xxxix, 306, 1901.
Rönne. A. f. O., lxxv, 284, 1910.
B. O. G. Heidel., xlvii, 241, 1928.
Rosengren. Acta O., xvi, 3, 1938.
de Rötth. A. of O., xxii, 809, 1939.
v. Rötth. K. M. Aug., lxxxiii, 359, 1929;
xci, 682, 1933.
Rubbrecht. Bull. S. fr. d’O., xlv, 257, 1932.
A. d’O., l, 608, 1933; lii, 786, 1935.
Rumach. A. f. O., czkxvi, 78, 1936.
Sachs. Z. f. Aug., lxxi, 96, 1930.
Safar. B. O. G. Heidel.., xxxix, 119, 1932.
Z. f. Aug., lxxvii, 270 ; Ixxviii, 92, 1932;
lxxxiii, 189, 234, 1934.
Behandlung d. Netzhautabhebung m.
multipler diathermischer Stichelung, Berlin,
1933.
A. of O., xi, 933, 1934.
Saint-Martin. Bull. S. fr. d’O., lxvi, 323,
1933.
Sallmann and Rieger. A. f. O., czzxiii, 75,
1934.
Sallmann and Sveinsson. A. f. O., czkx, 1,
1934.
Salzmann. Wien. med. W., xxiv, 1082, 1921.
Samelsohn. Z. f. med. Wiss., xlix, 833, 1875.
Cb. pr. Aug., xi, 351, 1887.
Samuels. A. of O., xxi, 273, 1939.
Sattler. D. med. W., xxxi, 15, 56, 1905.
Savage. O. Rec., xxi, 344, 1912.
Schall. K. M. Aug., lxviii, 236, 1922.
Scheffels. A. of O., xx, 403, 1891.
Schieck. Kurzes Hb. d. O., Berlin, v, 455,
1930. -
Schiótz. K. M. Aug., lxii, 234, 1919.
Schleich. Mit. O. Kl. Tübingen, iii, 1, 1882.
Schlessinger. Beit. 2. Pathologie d. Sehnerven
w. d. Netzhaut, Berlin, 51, 1884.
Schmelzer. A. f. Aug., c-ci, 268, 1929.
K. M. Aug., xcvi, 19, 1936.
Schnabel. A. f. Aug., v, 67, 1876.
Schoeler. K. M. Aug., xxxi, 215,
xxxii, 382, 1894 ; xxxiv, 18, 1896.
Münch. med. W., 117, 1889.
Schoenberg. A. of O., xii, 709, 1934; xiii,
252, 1935.
Schreiber. A. f. O., ciii, 75, 1920.
Z. f. Aug., xlviii, 171, 1922.
Schreiber and Wengler. A. f. O., lxxvi, 99,
1909.
Schwarz. Z. f. Aug., xvii, 54, 1907.
Schweigger. A. f. O., ix (1), 199, 1863.
A. f. Aug., xii, 52, 1883.
B. O. G. Heidel.., xx, 132, 1889.
de Schweinitz. O. Rev., xviii, 150, 1899.
T. Am. O. S., x, 228, 1904.
1893 ;
Sedan. A m. d’Oc., clxv, 582, 1928.
Segi. K. M. Aug., lxxi, 53, 1923.
Seible. K. M. Aug., lvi, 587, 1916.
Shapland. T. O. S., li, 152, 1931; lii, 170,
I932; liii, 127, 1933 ; liv, 176, 1934.
Brit. J. O., xviii, 1, 1934.
Sichel. Am. d’Oc., v, 243, 1841.
Silex. Münch. med. W., i, 106, 1895.
Simi. Bal. d’Oc., vii, 39, 1884.
Sinclair. T. O. S., xxxvi, 373, 1916;
p. lvii, 1932.
Sloan. South. Med. J., xix, 228, 1926.
Slocum. Contrib. to oph. Science, Wisconsin,
210, 1926.
Sourdille. Bull. S. d’O. Paris, xli., 684, 1929;
xliv, 349, 1932.
Conf. S. d’O. Catalogue, Barcelona, 1931.
A. d’O., xl, 419, 1923.
lii,
Speciale-Cirincione. Am. di Ott., liii, 641,
1925.
Spinelli. A. di Ott., xl, 404, 1933.
Stallard. Brit. J. O., xiv., 1, 1930; xvii, 294,
1933; xix, 31, 1935 ; xxi, 35, 1937.
Stanka. K. M. Aug., lxx, 707, 1923.
Stargardt. B. O. G. Heidel.., xliii, 248, 1922.
Steffan. Jb. ii. Verwalt. Med., Frankfurt,
xvii, 18, 1873.
2926
TEXT-BOOK OF OPHTHALMOLOGY
Stein. K. M. Aug., lxxxvi, 402, 1931.
A. f. Aug., cv, 290, 1932.
Stellwag. Lehrb. pr. Aug., Wien, 1861.
Stilling. A. f. Aug., xii, 332, 1883.
Strampelli. Boll. d’Oc., xii, 629, 1933.
St. Yves. Nouveau traité des maladies des
Ayewa, Paris, 331, 1722.
Sugita. A. f. O., czzxvii, 447, 1937.
v. Szily. Atlas d. Kriegsaugenh., 407, 1918.
v. Szily and Machemer. K. M. Aug., xc, 806,
1933; xci, 248, 1933; xcii, 44; xciii,
721, 1934; xcvi, 36, 1936.
Szymanski. XIV Inter. Cong. O., ii (2), 51,
1933.
Takamatsu. Acta S. O. Jap., xxxvii, 14, 1933.
Terrien. Médicine, iii, 251, 1922.
Terrien, Veil and Dollfus. A. d’O., xlix, 353,
1932; 1, 331, 1933.
Terry. A. of O., viii, 182, 1932.
Terson. An. d’Oc., cziv, 22, 1895.
Bull. Soc. fr. d’O., xx, 209, 1903.
Thomas. Z. f. Aug., liv, 333, 1925.
Thomson. An. of O., xxv, 20, 1916.
A. of O., xlix, 563, 1920.
Tillé. Bull. S. d’O. Paris, xliv, 648, 1932.
Török. A. of O., xlix, 506, 1920.
Trantas. A. d’O., xliii, 149, 1926.
XIII Inter. Cong. O., i, 257, 1930.
K. M. Aug., xciii, 521, 1934.
v. Trigt. D. Augenspiegel w. S. Anwendung,
1853.
Tristaino. Gior. d’O., iii, 109, 1922.
|Uhthoff. Ber. kl. W., xxxi, 723, 1894.
D. med. W., xxviii, 115, 1922.
Ulrich. K. M. Aug., xxvii, 337, 1889.
Vail. O. Rec., xxi, 553, 1912.
A. of O., xlix, 553, 1920.
Veil and Dollfus. Bull. S. fr. d’O., xliv, 485,
1931.
Velhagen. A. f. O., xlix, 599, 1900.
K. M. Aug., lxxvii, 644, 1926;
317, 1932.
Verderame. K. M. Aug., xlix (1), 452, 1911.
Verhoeff. O. Rec., xxvi, 10, 1917.
Visser-Heerema. A. f. Aug., ciz, 543, 1936.
Visser-Heerema and Weve. K. M. Aug.,
xciv, 721, 1935.
Vogelsang. K. M. Aug., xcviii, 252, 1937.
Vogt. Atlas, Berlin, 1921. -
Z. f. Aug., xlix, 67, 1923; lxxxiii, 321, 322,
324; lxxxiv, 19; lxxxv, 2, 1934.
K. M. Aug., lxxii, 212, 335, 1924; xcii,
577; lxxxii, 691, 1929; lxxxiv, 305,
1930; xciii, 109, 1934; xcvi, 10, 15, 1936;
xcviii, 735, 1937.
lxxxviii,
Schw. med. W., lix, 331, 1929.
XIV Inter. Cong. O., ii (1), 1, 1933.
A. f. O., czzxiii, 26, 1934. -
Brit. J. O., xviii, 650, 1934.
A. of O., xii, 842, 1934.
Operative Therapie w. Path. der Netzhaut-
ablösung, Stuttgart, 1936.
Vossius. Berlin. kl. W., xlvi (1), 1197, 1909.
Wagener and Gipner. Am. J. O., viii, 694,
I925.
Am. J. O., xvii, 1, 1934; xviii, 246, 1935;
xix, 392, 558, 1936.
A. of O., xiii, 1056, 1935.
Wagenmann. B. O. G. Heidel., xxv, 230,
1896.
Walker. Am. J. O., xvii, 1, 1934; xviii, 246,
1935; xix, 392, 558, 1936.
A. of O., xiii, 1056, 1935.
Wardrop. Essays on the morbid anat. of the
Human Eye, ii, 64, 1818.
Ware. Chirurg. Observations relat. to the Eye,
London, II, i, 168, 1805.
de Wecker. Traité d. maladies d. fond des
gewa, Paris, 153, 1870.
An. d’Oc., lxviii, 137, 1872; lxxxvii, 39,
1882.
Bull. S. fr. d’O., xvii, 332, 1899.
Am... d’Oc., czzviii, 81, 1902.
Weekers. A. d’O., xlii, 321, 1925.
XIV Inter. Cong. O., ii (2), 14, 1933.
Weill. Z. f. Aug., xv., 140, 1906.
Weiss and Evans. Am. J. O., xvii, 627, 1934.
Wessely. O. kl., vii-viii, 265, 1903–4.
K. M. Aug., xliii (1), 654, 1905;
275, 1922; lxxxvi, 247, 1931.
D. med. W., i, 901, 950, 1930.
Weve. Ned. tij. v. Gen., lxxiv, 2354, 4733,
1930.
Behandlung d. Netzhautablósung m. Dia-
thermie, Berlin, 1932.
R. M. Aug., lxxxix, 822, 1932.
An. d’Oc., clxxi, 1, 1934.
A. f. Aug., ciz, 49, 534, 1935–36.
A. of O., xvi, 173, 1936.
T. O. S., lix, 43, 1939.
Weve and Fischer. A. f. Aug., cz, 198, 1936.
Wiener. A. of O., liii, 368, 1924.
Wood. Brit. J. O., iv., 413, 1920.
Zeeman and Oltmanns. A. of O., xiii, 971,
lxviii,
1935.
Zentmayer. A. of O., xvii, 946, 1937.
Zimmer. Diss., Lausanne, 1937.
Zossenheim. Beit. z. Aug., ii, 429, 1895
CHAPTER XXXVII
DISEASES OF THE OPTIC NERVE
ONE of the difficulties in writing this book has been to decide at which point to
take note of GEORGE EDMUND DE SCHwBINITZ (1858–1938) (Fig. 2509); it is quite
obvious that the great representative American ophthalmologist must be included,
and there is hardly a chapter in ophthalmology which he has not enriched by his
shrewd and careful clinical observations and which his photograph could not legiti-
mately grace. In view of the fact, however, that his earliest important work
was on the toxic amblyopias and one of his last outstanding contributions to Our
knowledge—his Bowman lecture in 1923—dealt with the optic nerve, I have chosen
this chapter. Its choice, moreover, emphasizes the fact that he was more than an
ophthalmologist : he was a great physician who will always be remembered in medical
history for his influence on the progress of knowledge, and among those who knew
him for his erudite and highly cultured intellect and the unusual charm of his per-
Sonality.
I. GENERAL CONSIDERATIONS !
We have already seen that the optic nerve—which is actually not a
“nerve * in the true sense of the word—the fibres of which arise in the
ganglion cells of the retina and terminate mainly in the lateral geniculate
body, is ontogenetically, morphologically and functionally a tract of the
central nervous system. Its fibres have no sheath of Schwann, it lacks the
highly specialized reticular connective tissue framework which characterizes
the cerebro-spinal nerves, and it possesses the supporting tissues character-
istic of the white matter of the brain and spinal cord ; moreover, its intra-
orbital portion is clothed by the coverings of the brain—the dura, the arach-
noid and pia, the spaces between which are in open communication with the
corresponding spaces of the brain. It follows, therefore, that diseases of the
optic nerve are essentially diseases of the central nervous system, and that,
while the anterior portion of the nerve is frequently affected in intra-ocular
diseases, whether circulatory, inflammatory, degenerative or neoplastic in
nature, it is also prone to be affected by meningeal disease. It is important
that, protected as it is from most harmful external influences, its involvement
in pathological processes usually indicates some disease of general systemic
or central nervous origin ; its pathology is therefore more usually of general
than of local import. Most of these diseases are serious ; and consequently
an affection of the optic nerve is frequently a matter of concern not only for
the function of the eye but for the general health or even the life of the
patient.
1 Vol. I, p. 243.
T.O. —WCL. III. 2027 3 H
29.28 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 25.09.-George Edmund de Schweinitz.
(1858–1938).

DISEASES OF THE OPTIC NERVE 2929
THE HEALING OF Wounds
In common with other white tracts of the central nervous system, the
fibres of the optic nerve, when cut, degenerate without evidence of sustained
regenerative activity. Not only does the degeneration proceed in a central
direction as would be expected in view of the origin of the majority of the
fibres in the ganglion cells of the retina, but it also occurs peripherally, the
ganglion cells themselves degenerating after section of the nerve (Birch
Hirschfeld, 1900); the process of peripheral degeneration does not therefore
involve the efferent fibres only, but the afferent ones also, and results in an
º
*ºº.
* * #. -
º:
º º º: -
-
º
-
-
- --
FIG. 2510.-ActiTE NEC Rosis IN THE Optic NERVE AFTER INJURY.
Twenty-four hours after a basal fracture crossing the optic canal. Polymorpho-
nuclear leucocytes predominate (Cone and MacMillan, Penfield's Cytol, and Cellular
Path. of the Nervous System).
atrophic appearance at the disc sometimes within 14 days and usually within
3 months after the injury. To some extent this retrograde degeneration
may be the result of vascular disturbances caused by the section, but the
fact that it is more marked the nearer the injury is to the bulb, suggests
that the ganglion cells of the retina are of such delicacy as to be unable to
withstand the shock of the trauma. If the nerve has been severed
anatomically, the cut ends become bound together by the proliferation of
fibrous and neuroglial tissue which invades indiscriminately the spaces
between the fibres and the spaces between the sheaths (Wagenmann, 1890;
v. Michel, 1901; Reis, 1908; Stöwer, 1910; v. Hippel, 1918; Bachstez,

3 H 2
29.30 TEXT-BOOK OF OPHTHALMOLOGY
1920; and others). If the solution of continuity has been functional, such
as is caused by pressure from an enlarged pituitary or a diseased artery, the
breaking up of the medullary sheaths and the destruction of the neuroglial
cells is accompanied by less fibrous tissue proliferation (Bernheimer, 1891;
Sachs, 1893; and others). In actual practice, of course, these events are
usually complicated by hamorrhagic disturbances, bleeding taking place
from the central vessels, from the vessels of the sheaths, or from the cranial
cavity with the subsequent formation of
a fibrous connective tissue scar ; those
clinical and pathological details will be
considered in the section of injuries to
the eye. From the point of view of the
present context the important fact is that
healing of the nervous tissue does not
occur, and that the result of any solution
of the continuity of the fibres is complete
and permanent blindness in the affected
area of the visual field.
The intimate histology of these
changes is of importance, expressing, as
it does, the fundamental reaction of the
tissues of the nerve to all pathological
processes-traumatic, inflammatory and
degenerative. In general terms the im-
mediate result at the site of injury is
an acute necrosis exactly similar to the
picture presented by the white matter of
the brain after trauma or infarction; the
axons are reduced to detritus, the medul-
lary sheaths fragment, the neuroglial
Fig. 2511. Regeneratios or Fºres elements degenerate, and the area becomes
- soºn Nºve. invaded by polymorphonuclear leucocytes
sº." º "º (Fig. 25.10). These are presumably at-
balls. B. Collateral ball. C. Bifurca tracted chemotactically by the products of
ºnº" * * * tissue-disintegration but in non-infective
conditions they are transient, and with
the subsidence of the acute reaction, are replaced by microglial phagocytes.
Finally, when the débris is thus cleared away, the stage is occupied by
astrocytes which eventually form a neuroglial scar.
CHANGES IN THE NERVE FIBREs. While this massive reaction is taking
place in the immediate neighbourhood of the injury, Wallerian degeneration
occurs both proximally and distally. The finer changes have been studied
by Tello (1907), Ortin and Arcaute (1913) and Cajal (1928). At the site

DISEASES OF THE OPTIC NERVE 2931
of the lesion bulbous retraction buds which take on a silver stain, form on
the axis cylinders, but for some days little change is seen in the fibres beyond
the traumatized area. After eight days, however, the nerve fibres lose
their neuro-fibrils and take on a granular appearance, showing irregular
varicosities in their course ; after 15 days fragmentation occurs ; and
eventually they gradually disappear, while the medullary sheaths show
fatty droplets, become broken up, and are eventually phagocyted.
Regeneration. Unless the ganglion cells have been completely destroyed
by retrograde degeneration, some degree of regeneration is attempted, which,
however, always proves abortive (Stroebe, 1895; Tello, 1907; Ortin and
Arcaute, 1913; and others). The regeneration occurs at both ends of the
cut nerve, but is always more intense at the ocular end since the centrifugal
fibres are fewer in number. At a varying period (7 to 14 days) after the
section, growth may become evident, the proliferating fibres terminating
in club-shaped extremities which occasionally bifurcate (Fig. 2511).
Their growth, however, is aimless, the fibres forming devious tangles, and
although they may pass through the proliferating neuroglial tissue and even
penetrate the mesodermal scar at the site of the injury, they never establish
connection through it, but always become strangled, and finally degenerate
and disappear. This failure in regeneration, which is characteristic of all
the white matter of the central nervous system, is considered by Cajal
(1928) to be due not to any deficiency in the parent ganglion cells, but to
the lack of a neuroglial framework such as could provide the necessary
stimulation and guidance which is supplied to the peripheral nerves by the
sheath of Schwann and the highly specialized connective tissue framework
which surrounds each fibre of these nerves.
THE INTERSTITIAL TIssu E. The interstitial tissue of the optic nerve,
the intimate study of which has only recently become available through the
methods of staining by metallic impregnation introduced by Del Rio-Hortega
(1917–21), like that of the central nervous system, can be divided into two
distinct types (Penfield, 1928) : mesodermal microglia and ectodermal
neuroglia. Their pathological reactions have been most intimately studied
by Cone of Montreal (1928), from whose researches the following description
is largely taken. In general terms it may be said that the function of the
two types is entirely different : the microglia of mesodermal origin acts as
phagocytes to remove the detritus left by the degenerated neural elements,
while the neuroglia of ectodermal origin has a reparative function, replacing
the neural elements by a neurogiiai scar.
Microglial cells were demonstrated first in the optic nerve by Lopez
Enriquez (1926) and Marchesani (1926); they are the only type of interstitial
cell either in the brain or in the optic nerve which acts as a phagocyte (Cone,
1928). Normally, irregularly oval cells with many long and slender pro-
cesses lying quiescent and irregularly distributed among the neuroglia and
2932 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 25.12.-Microglia iN Optic NERVE.
The cells are irregularly intercalated between the other constituents of the nerve.
(Del Rio-Hortega's silver stain) (Cone and MacMillan).
- -
- - - - -
- * - - - -
Fig. 2513.-AMoEboid Microglia. CELL.
Most of the expansions have been withdrawn. (Del Rio-Hortega's silver stain)
(Cone and MacMillan).






DISEASES OF THE OPTIC NERVE 2.933
nerve fibres, they spring to activity on the incidence of trauma, or, for that
matter, on the occurrence of any degenerative process in the nerve fibres or
neuroglia following inflammatory, oedematous, or degenerative conditions
(Figs. 25.12 and 2513). Their small nuclei enlarge and become elongated and
hook-shaped, the complicated cytoplasmic processes are withdrawn, the cells
become oval or rod-shaped, and as phagocytosis proceeds the cell-bodies
become round and enormously swollen with accumulated débris so that the
now pyknotic nucleus is pushed to the side (Fig. 2514). These changes take
place rapidly at the site of the injury, masses of turgid cells accumulating
and replacing the leucocytes within 48 hours, while after some days as
degeneration proceeds, cells -
filled with fat are seen
everywhere in the nerve
(Fig. 2515).
The changes in the
ectodermal neuroglia are
more degenerative innature.
These cells may be divided
into fibrous astrocytes and
oligodendrocytes.
The fibrous astrocytes
are large cells with many
processes containing fibrillae
and ending in foot-plates,
running generally at right
angles to the course of the
nerve fibres, and insinuating
themselves between them
(Fig. 2517). These cells Fig. 25.14.-Microglia PHAgocytes.
with their foot-plates form Typical compound granular corpuscles. The end-
- - - result of the phagocytic change of microglia. (Del Rio-
a barrier insulating the Hortega's silver stain: in the cat) (Cone and MacMillan).
nervous tissue from the
mesodermal tissue. They form the marginal glia beneath the pia (the
peripheral glial mantle of Greeff, 1899, and Kiribuchi, 1899), and they abut
on the connective tissue septa ; in the scleral portion of the lamina
cribrosa they are most numerous near the centre of the nerve-bundles
and their cytoplasmic projections run out to end in foot-plates on the
connective tissue rings surrounding the nerve-bundles; in the choroidal
portion they form a sieve through which the nerve-fibre bundles pass;
and they constitute the border-tissue between the nerve fibres and the
choroid and the central tissue meniscus of Kuhnt at the centre of the
papilla (Jacoby, 1905).
These cells react to injury in two ways—at the point of direct trauma
(and also in acute diseases of the central nervous system) changes occur

2934 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2515–Microglial, CELLs of Optic Fig. 2516.-CELL-Forms is Optic
NERVE. NERVE.
Different forms assumed as they become A. Typical oligodendrocyte.
active phagocytes (Lopez Enriquez). B. Small oligodendroglia. C. Oligo-
dendroglia in interfascicular rows.
D. Fibrous astrocyte. E. Microglia.
F. Connective tissue septa (Lopez
Enriquez).
Fig. 2517. Fibrous Astrocyte.
The cell projections run at right angles to the nerve fibres. (Cajal gold stain) (Cone
and MacMillan).


DISEASES OF THE OPTIC NERVE 2935
Fig. 2518. HYPERTRoPHIED FIBROUs Ast Roc YTE.
A Nissl plump cell, occurring in conditions of gliosis. (Cajal gold stain) (Cone
and MacMillan).
Fig. 2519.-GLIosis IN THE OPTIC NERVE.
Gliosis showing no definite pattern consisting of a tangle of cells and fibres.
Occurring in papilloedema in the intermediate tissue of Kuhnt (Cone and MacMillan).


2936 TEXT-BOOK OF OPHTHALMOLOGY
which are typically degenerative (Cone, 1928)—the cytoplasmic processes
become varicose, the intra-cellular fibrils do not stain, the cell-body swells
(the AMOE.boid glial CELLs of Alzheimer, 1910) and the nucleus becomes
pyknotic. Finally the cell-bodies break up and the processes fragment
(CLASMATodºx DRosis of Cajal, 1913).
Outside the region of acute
--- change, progressive alterations of
gliosis occur. The nucleus of the
º - astrocyte enlarges, the intra-cellular
fibrils disappear, the cytoplasm
becomes voluminous and homo-
geneous, and the cytoplasmic
process becomes thickened. This
hypertrophied form persists so long
as phagocytes are present, and
with their large cell-bodies and
varicose projections, correspond to
the gliosis described by Greeff (1896)
in sub-acutedegenerative conditions
(the Nissl, PLUMP CELLs) (Fig.
2518). At this stage also they
increase enormously in numbers
by amitotic division, while smaller
forms appear, perhaps derived
from oligodendrocytes. When the
phagocytes have cleared the débris,
the nucleus returns to a resting
stage and the cell-body shrinks, the
nerve fibres being then replaced by
astrocytes and their processes.
When the degeneration of the
nerve fibres has been rapid, these
Fig. 2520-Column AR GLIosis IN THE Optic -
NERVE. form a dense tangle of neuroglial
Glial replacement following the general elements (Fig. 2519); but when
course of the nerve fibres and reduplicating the
normal architecture (Cone and MacMillan). the process of degeneration has
been slow the replacement by
astrocytes is orderly, the cell processes lie in parallel columns simulating
the columnar gliosis of the spinal cord as it occurs in tabes (Spielmeyer,
1906) (Fig. 2520).
Oligodendroglial cells, originally demonstrated in the optic nerve by
Enriquez (1926) and Marchesani (1926), are small cells with round or oval
nuclei, granular cytoplasm, and delicate branching processes provided with
varicose thickenings (Fig. 2521). They are grouped in rows between the
nerve fibres usually near the centre of the bundles to which their processes

DISEASES OF THE OPTIC NERVE 2937
run parallel, and they differ from astrocytes in the absence of foot-plates
and in having no relation to connective tissue septa. Pathologically, they
show a marked and rapid alteration to injury, an acute degenerative
change, involving nuclear pyknosis, swelling of the cell-body, and
fragmentation of the processes (Penfield and Cone, 1926; Cone, 1928)
(Fig. 2522). When these changes are slight, they may be reversible and
the cell returns to normal; but when they are severe, the cell dies rapidly.
The more chronic changes have been studied by Cone (1928) in the stumps
Fig. 2521.-Normal, OLIGoDENDROGLIA IN OPTIC Fig. 2522. Acute Swelling of OL1-
NERVE. GoDENDRoglia on INJURY.
Longitudinal section; cytoplasmic projections The nucleus is impregnated ; fluid
run along the nerve fibres. (Del Rio-Hortega's pushes out the cell-membrane; and the
silver stain) (Cone and MacMillan). cytoplasmic projections have fragmented.
(Del Rio-Hortega's silver stain) (Cone
and MacMillan).
of nerves after enucleation of the globe; he noted a preliminary
hypertrophy, but at the stage when the phagocytes were disappearing, no
more oligodendrocytes were to be found ; it is possible that they had
undergone a transition to cells of the astrocyte type, as has been shown to
occur in tumour-formation. It is possible, indeed, as Cone (1928) suggested,
that these cells are related closely to the medullated fibres, playing some
part in the metabolism of myelin, and are thus analogous to the Schwannian
cells of peripheral nerves; they have not been described apart from medul-
lated fibres, and it may be that when these fibres disappear the character of
this type of cell changes.
Alzheimer. Histol. u. Histopath. Arb. ii. die Cone. A. of Neurol, and Psy-, *.x, 34.
Grosshirm, iii, 401, 1910. 1928.
Bachstez. K. M. Aug., lxv, 827, 1920. Enriquez. Bol. Real Soc. espan. de Hist.
Bernheimer. A. f. O., xxxvii (2), 37, 1891. Nat., xxvi, 294, 301, 1926.
Birch-Hirschfeld. A. f. O., i (1), 166, 1900. Bol. Soc. espan. de Biol. xii, 79, 1926.
Cajal. Trab. del Lab.de Invest. Biol. xi, 219, Greeff. A. f. Aug., xxix, 324, 1894; xxxiii,
1913. 214, 1896.
Degeneration and Regeneration in the IX Internat. Cong. Utrecht, 287, 1899.
Nervous System, Oxon., 1928. v. Hippel. A. f. O., xevi, 134, 1918.

2938
TEXT-BOOK OF OPHTHALMOLOGY
Jacoby. K. M. Aug., xliii, 129, 1905.
Riribuchi. A. f. Aug., xxxix, 76, 1899.
Marchesani. A. f. O., czvii, 575, 1926.
v. Michel. Z. f. Aug., vi. 1, 1901.
Ortin and Arcaute. Trab. del Lab. de Invest.
Biol., xi, 239, 1913.
Penfield. Cowdry's Special Cytology, N.Y.,
ii, 1032, 1928.
Penfield and Cone.
xvi, 131, 1926.
J. f. Psy. wrºd Neurol.., xxxiv, 204, 1926.
A. of Neurol. and Psy.,
Rio-Hortega. Trab. del Lab. Invest. Biol.,
xiv, 1, 1916; xv, 367, 1917.
Bol. S. espan. de Biol., viii, 229, 1918;
ix, 68, 1919; x, 121 ; xiv, 5, 1928.
Sachs. A. f. Aug., xxvii, 154, 237, 1893.
Spielmeyer. K. M. Aug., xliv (1), 97, 1906.
Stöwer. K. M. Aug., xlviii (1), 426, 1910.
Stroebe. Cb. f. all. Path. u. path. A mat., vi,
849, 1895.
Tello. Trab. del Lab. de Invest. Biol., v, 237,
1907
Tita. An di ott., lxvi. 51, 1938.
Wagenmann. A. f. O., xxxvi (iv), 126, 1890.
Reis. A. f. O., lxvii, 360, 1908.
II. DISTURBANCES OF THE CIRCULATION
Most of the disturbances of the circulation of the optic nerve have
already been discussed in the section on Diseases of the Retina, for the
nerve, fed by the same vessels, shares intimately in the vascular pathology
of this tissue. Thus a hyperaemia of the retina is accompanied by a hyperaemia
of the disc, in which region, indeed, the condition is usually recognized
clinically. An anaemia of the retina is accompanied by a similar condition
of the nerve, and the death of the ganglion cells, consequent on a local
anaemia by occlusion of the retinal artery or a general anaemia such as
follows a massive loss of blood, is followed by the development of Optic
atrophy. Embolism and thrombosis of the central vessels, as we have seen,
is more usually an affection of the optic nerve than the retina in the strictest
sense of the terms, for the most usual place for the artery or the vein to be
occluded is at the optic disc where the lumen of the vessels is constricted
by the tissues of the lamina cribrosa." Two conditions, however, remain to
be considered ; haemorrhages into the intervaginal space and the Oedematous
condition which becomes apparent clinically as papilloedema.
HAEMORRHAGE INTO THE OPTIC NERVE SHEATHS
Anatomically a haemorrhage may occur either into the sub-dural or the
sub-arachnoid space or both, but since the former is a potential space only
and the latter is in free communication with the wide sub-arachnoid spaces
of the central nervous system, it will be understood that the sub-arachnoid
type is the commoner and usually of more serious import.
(a) SUB-DURAL HAEMORRHAGES
Sub-dural haemorrhages (Fig. 2523) are most commonly traumatic
in origin, for non-traumatic or spontaneous haemorrhages are usually
sub-arachnoid in type. The first to call attention to their occurrence
was Priestley Smith (1884) who, in a traumatic meningeal haemorrhage,
found the sub-dural space of the optic nerve distended with blood and the
1 p. 2570.
DISEASES OF THE OPTIC NERVE 2939
sub-arachnoid filled with clear cerebro-spinal fluid, a finding duplicated
almost simultaneously by Silcock (1884). Liebrecht (1906–12) showed that
in traumatic cases such a haemorrhage could occur in three ways: (1) by a
spread from the sub-dural space
of the brain, (2) by the blood
travelling along the perivascular
spaces of the ophthalmic artery
and thence breaking into the
sub-dural space, and (3) in frac-
tures of the bony wall of the orbit
near the optic foramen when the
dural vessels are torn. In the
last case, if the arachnoid is un-
injured, there is no extension of
the haemorrhage into the sub-
arachnoid space. Apart from
these traumatic cases—which are
the most common–sub-dural FIG. 2523.-SUB-DURAL HAEMoRRHAGE.
haemorrhages may occur spon- Haemorrhage in dura and sub-dural space. Note
taneously in blood - diseases the collapsed vessels in the sub-dural space (x 20)
* (MacDonald).
(scurvy, Freud, 1884; secondary
anaemia in carcinoma, Uhthoff, 1901; and others), or in massive cerebral
haemorrhage due to vascular disease when the brain substance has been
extensively lacerated (MacDonald, 1931).
It is probable that small haemorrhages in the sheaths of the optic nerve may
produce indefinite symptoms which are difficult to diagnose with any certainty (Cross,
1913). Such cases result from injury, quite frequently very slight in degree, which
may or may not involve a fracture of the optic canal, and may be followed by irregular
field defects—concentric contractions, sector defects, or rarely a central scotoma or
even complete blindness. The nature of these defects shows that the lesion is in the
nerve, and their steep edges suggest that they are probably not due to the pressure of
blood but rather to a destruction of the nerve fibres resulting from a rupture of small
vessels passing from the sheaths into the nerve: such defects are of course permanent
(Traquair, 1931). If the haemorrhage has been extensive, however, the symptoms
resemble those of a sub-arachnoid haemorrhage. -
(b) SUB-ARACHNOID HAEMORRHAGES
Sub-arachnoid haemorrhages can also be divided aetiologically into two
main groups—traumatic and spontaneous–and it is important to remember
that in both the cause is never primarily orbital, for the blood is derived
from the general sub-arachnoid space (Figs. 2524 and 2525).
In traumatic cases, which are due to the rupture of meningeal vessels
and are the more common, the usual cause is a basal fracture. In non-
traumatic cases the blood is invariably of intra-cranial origin and may be
due to :-
1. The rupture of a basal aneurysm.

2940 TEXT-BOOK OF OPHTHALMOLOGY
2. A spontaneous cerebral haemorrhage, from which the blood usually
reaches the sub-arachnoid space by bursting through the brain substance.
3. Meningeal haemorrhages in blood diseases, diabetes, renal disease,
and so on.
Of these the most interesting are the haemorrhages resulting from
the bursting of a sacculated aneurysm of one or other of the basal arteries.
Most of such aneurysms are congenital in origin and are unassociated with
arterial hypertension or general arterial disease, the actual accident of
Fig. 2524–Sub-ARAcHNord HAEMoRRHAGE (Goulden).
rupture being usually precipitated by a temporary rise of blood-pressure
(Wichern, 1912; Turnbull, 1915–18; Fearnsides, 1916; Symonds, 1924; and
others): such aneurysms are usually situated in an artery of the first order,
less commonly in a cortical branch, the favourite site being the anterior half
of the circle of Willis. Acquired aneurysms are usually due to the lodgement
of infective emboli, most frequently of endocarditic origin; atheroma is a
much less frequent cause, and syphilis plays a small part in the aetiology.
Quite frequently the actual bursting of a basal aneurysm is preceded by
premonitory symptoms of considerable duration which point to the occur-
rence of intermittent small leaks of blood–paroxysmal headaches of a very

DISEASES OF THE OPTIC NERVE 2941
severe type coming on suddenly, usually after a slight exertion such as
stooping, and frequently associated with tinnitus. These may be subject
to periodic exacerbations until eventually a catastrophe occurs indistinguish-
able in its effects from a major meningeal haemorrhage from any cause:
Such an event may be followed by coma and death with all the signs of
cerebral and medullary compression: in such cases the tear in the wall of
the aneurysm is large. At other times, however, when the leak is less
profuse, the sequence of events is less dramatic. An initial period of coma
may be followed by a period of meningeal irritation with delirium, restless-
ness and vomiting, accompanied by severe headache, rigidity of the neck
Fig. 2525.-Sub-ARAcHNoid H.A.MoRRHAGE (Goulden).
with Kernig's sign, bilateral evidences of pyramidal disturbance, paresis of
cranial nerves, particularly the IIIrd and VIth, and the ocular symptoms
about to be described. Recovery from such an attack is not unusual,
consciousness returning, the palsies disappearing, and the reflexes becoming
normal after some 2 or 3 weeks; but recurrent seizures are common, one of
which eventually will likely prove fatal. In all these cases the point which
clinches the diagnosis is the finding of blood in the cerebro-spinal fluid.
Ocular signs associated with a meningeal haemorrhage may be entirely
lacking, but in a large number of cases, whether the haemorrhage be traumatic
or spontaneous, its extension into the sheaths of the optic nerve leads to the
development of a fairly characteristic clinical picture the main features of
which are papilloedema and retinal haemorrhages, to which are frequently
added ocular palsies (in 70% of cases, Uhthoff, 1901) usually involving the

2.942 TEXT-BOOK OF OPHTHALMOLOGY
IIIrd and VIth nerves, and proptosis (Fig. 2528, Plate LX). Since the
haemorrhage involves the general sub-arachnoid space, almost invariably
both optic sheaths are involved and these symptoms are bilateral.
Papilloedema is the most constant symptom, and although occasionally
severe (Rollet, 1908), it is usually slight in amount: only rarely is it
completely absent (Priestley Smith, 1884; Elschnig, 1895–99). It is
bilateral, and may become apparent with great rapidity: Uhthoff (1901)
has observed its appearance within half an hour and its full development
within 5 hours of the occurrence of the haemorrhage. On the other hand,
if the haemorrhage does not immediately spread diffusely into the sheaths
but becomes encysted, the oedema may not appear for some months (Paton,
Fig. 2526.-Sub-ARAchnold AND Sub-Hyaloid H.A.MoRRHAGEs.
Note the congested central vessels and the absence of communication between the
two haemorrhages at the disc margin (x 10) (MacDonald).
1924). In the first case, when the papilloedema occurs rapidly, it is probably
due to obstruction of the venous outflow from the eye, a matter which will
be considered more fully in the next section. It is obvious that, in addition
to the increased pressure about the nerve caused by the blood, the angulation,
stretching and compression of the vessels as they traverse the distended
inter-vaginal space between their points of fixation in the pia and in the
dura may have an important bearing on its development (Fig. 2527)
(Dupuy-Dutemps, 1914; Riddoch and Goulden, 1925; MacDonald, 1931;
and others). It is significant that Riddoch and Goulden (1925) found that
the oedema of the nerve ended abruptly at the point at which the central
vein left, and that behind this point the nerve was healthy.
Retinal haemorrhages are associated with the majority of cases (Fig.
2528, Plate LX). Sometimes they may be the parallel effects of the arterial
disease or blood disease which caused the cerebral haemorrhage; but in the

DISEASES OF THE OPTIC NERVE 2943
majority of the cases, whether traumatic or due to the bursting of a basal
aneurysm in an otherwise healthy person, the retinal vessels are normal.
In such cases the haemorrhages are frequently small and usually near the
disc; sometimes, however, they are sub-retinal in position (Symonds, 1924),
but more frequently they are pre-retinal, in which case they may be of large
size and may burst into the vitreous and obscure all details of the fundus
for some considerable time (Paton, 1924; Riddoch and Goulden, 1925).
Some controversy exists as to the causation of these haemorrhages. In traumatic
cases Liebrecht (1912) concluded from their clinical appearance and development as
well as from the fact that the retinal vessels were sometimes of normal calibre, that
they were the result of the direct leakage of blood through the lamina cribrosa and
along the perivascular spaces into the optic nerve-
head and the pre- and retro-retinal tissues. This
view received some support from a pathological
examination reported by Hale White (1895) wherein
blood from a burst aneurysm of the internal carotid - -
had caused a profuse sub-dural and sub-arachnoid sº e-
haemorrhage which distended the optic nerve sheaths - -
whence it had torn its way under the retina to form subdural
a prominent dark swelling. The improbability of space
blood being able to follow this path, with all subarachno
ordinary pressure conditions which can be produced space-
pathologically, will be fully discussed when we con-
sider the pathogenesis of papilloedema ; and in all
recent pathological examinations of cases of sub-
arachnoid haemorrhage there has been no haemorrhage
into the nerve substance and no continuity between
the pools of haemorrhage in the nerve sheaths and in
the eye (Riddoch and Goulden, 1925; MacDonald,
1931) (Fig. 2526). It seems probable, therefore, that Fig. 2527-D1AGRAM to show
the mechanism of these haemorrhages is usually, at º sº*::::
any rate, the same as that of the haemorrhages HAEMORRHAGE BY BACK-PRES-
occurring in papilloedema-an obstruction of the sure (MacDonald).
central vein as it passes through the meningeal
spaces of the nerve, and a leakage from the intra-ocular veins by back-pressure
(Fig. 2527). Similarly the proptosis, which may develop quite suddenly, is probably
due to venous congestion in the orbit.
Subhyaloid Haemºrrhage
Re-inal vessels
| Cºtle nerve
Ratina
– ºra
Dura
Arachnoid
Central
-------
DLA-, + 1
It is obvious that in a condition so serious when consciousness is either
clouded or abolished, these ocular symptoms are not always noted and
visual failure is only complained of when recovery is advanced. Sometimes,
however, when massive vitreous hæmorrhages occur the visual loss may be
marked, and if the nerve is seriously damaged it may be complete (Favory,
1931; Manes, 1931; and others). The occurrence, however, of papilloedema
and retinal haemorrhages, frequently associated with a paresis of the extra-
ocular muscles and proptosis, in a person suffering from the effects of cranial
trauma or the subject of a sudden seizure pointing to cerebral pressure or
meningeal irritation, especially, in the latter case, after intermittent pro-
T-0.-WOL. III. * I





2944 TEXT-BOOK OF OPHTHALMOLOGY
dromal attacks of paroxysmal headaches, is sufficiently characteristic to
point to the nature of the affection, the diagnosis of which can be confirmed
by lumbar puncture and the demonstration of blood. The treatment, of
course, is entirely that of the general condition.
Cross. T. O. S., xxxiii, 43, 1913. Rollet. Rev. gem. d’O., xxvii, 49, 1908.
Dupuy-Dutemps. An. d’Oc., cli, 161, 1914. Silcock. T. O. S., iv, 274, 1884.
Elschnig. A. f. O., xli (2), 179, 1895; xlviii, Smith, Priestley. T. O. S., iv, 271, 1884.
461, 1899. Symonds. Quart. J. Med., xviii, 93, 1924.
Favory. A. d’O., xlviii, 81, 1931. Traguair. Clinical Perimetry, London, II,
Fearnsides. Brain, xxxix, 224, 1916. 175, 1931.
Freud. Wien. med. W., xxxiv, 244, 1884. Turnbull. Quart. J. Med., viii, 201, 1915.
Goulden. T. O. S., xlix, 333, 1929. Brain, xii, 50, 1918.
Liebrecht. A. f. Aug., lv., 36, 1906. Uhthoff. B. O. G. Heidel., xxix, 143, 1901.
A. f. O., lxxxiii, 525, 1912. White, W. H. T. Clin. Soc., London, xxviii,
MacDonald. T. Am. O. S., xxix, 418, 1931. 5, 1895.
Manes. A. de Oft. B. A., vi, 575, 1931. Wichern. Deut. Z. f. Nervenhk., xliv, 220,
Paton. T. O. S., xliv, 110, 1924. 1912.
Riddoch and Goulden. Brit. J. O., ix, 209,
1925.
PAPILLOEDEMA
Although disturbances at the optic nerve-head had long been known
to be associated with conditions of raised intra-cerebral pressure since
Türk (1853) first drew attention to the occurrence of retinal haemorrhages
and v. Graefe (1860) first described the swelling of the disc in cases of tumour
of the brain, the conception of papilloedema as a separate clinical entity is
of relatively recent date. Originally all conditions characterized clinically
by a swelling of the nerve-head were called optic neuritis, and if the swelling
were great it was called “ choked disc '' (Stauungspapille); but the latter
was regarded only as an exaggerated degree of the former. It was not
until 1908 that Parsons introduced the term papilloedema to apply to cases
showing more than 2 dioptres of swelling associated with raised intra-
cranial pressure and retained the term papillitis for more moderate degrees
of optic neuritis of varied aetiology. It was, however, recognized even in
the early days of ophthalmology (Hughlings Jackson, 1863) that two types
of “neuritis '' could exist—one involving no necessary loss of function while
displaying marked clinical signs, and the other which could in certain cases
proceed to optic atrophy without other ophthalmoscopic change. The position
was clarified by the classical paper of Paton and Holmes (1911) who
differentiated PAPILLOEDEMA as a passive oadema due to raised intra-cranial
pressure without primary inflammatory changes and often without disturbance
of function, and OPTIC NEURITIs as a swelling of the disc associated with
inflammation and loss of function. The two conditions have a different
aetiology, a different pathology, different symptoms and different sequelae,
but to a much less extent a different appearance, and in the early stages a
clinical differentiation between the two may be extremely difficult.
With a certain degree of logic, cases of papilloedema may be sub-divided
PLATE LX
CIRCULATORY Disturbascºs AT THE Disc
Fig. 2528.-Suis-ABAchnoid H.E.MoRRHAGE. Fig. 2529.-EARLY PAPILL ELEMA.
Fig. 2530.-Optic ATRoPHY AFTER Fig. 2531.-ExtraEME PAPiLLCEDEMA.
PAPILLEDEMA.
Note concentric foldings around disc
margin (Paton),
To face p. 2944.


DISEASES OF THE OPTIC NERVE 2945
into two classes: a more common type which is due to increased intra-
cranial pressure occurring in a healthy eye, and in which the oedema is
circumscribed in the immediate region of the optic disc (plerocephalic aedema);
and a rarer group in which the retina also shares in the oedema and is
therefore more diffuse, as may occur, for example, in thrombosis of the
central vein or renal retinopathy.
AEtiology
In a general sense it may be said that papilloedema results when the
normal pressure relationship of the circulation on either side of the lamina
cribrosa is disturbed. This may be caused by (a) ocular, (b) orbital, (c) intra-
cranial, or (d) general systemic events, and of these, intra-cranial factors are
much the commonest.
(a) Ocular Causes. A sudden lowering of the intra-ocular pressure is
not infrequently followed by papilloedema, although it is not usually marked
in amount. It can be produced experimentally by trephining the eye in
rabbits (Gilbert, 1910) or in dogs and monkeys (Kyrieleis, 1929), and has
been verified histologically to be a true oedema without inflammatory
evidences. Moreover, if the intra-cranial pressure is raised in dogs and
monkeys and One eye is trephined, the experimental papilloedema occurs
first in the hypotonic eye (Parker, 1916). Clinically it is liable to occur in
any condition where a hypotension develops, such as perforation of the
globe, particularly when a fistula develops (Elschnig, 1902; Stock, 1903;
Fuchs, 1904–28 ; Kampherstein, 1904; Gilbert, 1910 ; Behr, 1912;
Inouye, 1912; Schieck, 1926; Scardapane, 1930): in these cases it usually
disappears if the fistula is closed. A similar result has followed a cautery
puncture operation after the technique of Gonin for a detached retina
(Knapp, 1931). It has also followed a trephine operation which resulted
in profound fall of tension (Smith, 1939), or the sudden reduction of tension
in glaucoma by miotics (Carle, 1931) or vibratory massage (Eppenstein,
1930). Similarly, the prolonged hypotony following a concussion injury to
the globe may be marked by papilloedema (Collins, 1901–16–17 ; Polanyi,
1929). A similar finding in phthisis bulbi after irido-cyclitis (van der Borg,
1908), must, however, raise the question of inflammatory complications.
(b) Orbital Causes. Pressure on the optic nerve within the orbit so
that the circulation is impeded is liable to bring about a papilloedema. Such
a condition has been repeatedly brought about experimentally in animals
by ligating or clamping the nerve (Sobanski, 1937; Kyrieleis, 1936; Campos,
1937; Behr, 1937): the more proximal the ligature the less dramatic the
effect, which is greatly increased if it is applied distal to the entrance of
the central vessels, and becomes immediately massive if the vein and not
the artery is included (Sobanski, 1937). The literature contains several
reports wherein these conditions have been simulated clinically by orbital
tumours of all types—sarcoma (Rumjanzewa, 1930), fibroma (van der Hoeve,
3 I 2
2946 TEXT-BOOK OF OPHTHALMOLOGY
1925), myeloma (Tahano, 1935), aneurysm of the ophthalmic artery (Pfingst,
1936), echinococcus cysts (Teulières, 1927), Mikulicz's disease (Heine, 1925),
or a tumour of the nerve itself (v. Heuss, 1932). An interesting case of
sarcoma of the nerve sheath was described by Graf and Mikulinska (1928)
which had caused a pressure-atrophy of half of the nerve fibres : the
corresponding half of the disc was atrophic and the other oedematous and
swollen 3 dioptres. -
Cases of orbital abscess (Kljacko, 1929) or sinusitis (Belgeri and Arana, 1932;
Smith, 1939) may also be associated with a swelling of the disc, and although this
may be due to a pressure effect either from the phlegmon or from inflammatory
thickening of the tissues, it is difficult to eliminate with certainty the element of
infection : but the height of the swelling (4 dioptres has been reported), the type of
field with enlarged blind spot and concentric contraction, the retention of good vision,
and the reported sudden return of the disc to normal after evacuation of the pus,
suggests a mechanical origin.
An oedematous swelling of the disc sufficient sometimes to suggest the diagnosis
of tumour, may accompany an acute inflammation of the optic nerve, as in disseminated
sclerosis (Paton, 1914), particularly when the distal end of the nerve is affected ; but
it is better to regard these cases as examples of optic neuritis with an extreme degree
of Oedema.
(c) Intra-cranial Causes. A rise in intra-cranial pressure is by far the
commonest cause of papilloedema and accounts for the great majority of
cases; the rise may be due either to an increase in the cerebral contents,
as by a neoplasm, inflammatory growths, or blood-clots, or by an increase
in the volume of cerebro-spinal fluid as in meningitis, or to a general oedema
of the brain in systemic diseases as anaemia or nephritis, or, finally, to a lack
of normal cranial capacity, as in oxycephaly.
Uhthoff’s (1914) statistics showing the aetiology of bilateral papilloedema are of
interest :—
Brain tumour, 71 per cent.
Tuberculosis, 3.6 per cent. ..) 74.6 per cent.
Cerebral syphilis tº & e g º & . 12 2 3
Oxycephaly . tº & & & º & . 2-2 , ,
Brain abscess . º * e º & & e 2-2 , ,
Hydrocephalus and serous meningitis . wº & . 2-2 , ,
Cysticercus $ º :- e º º * & l' 1 , ,
Meningitis * tº e * > * se g * l. 1 , ,
Nephritis § º $ e g * * º 1. 1 , ,
Anaemia . sº gº * e tº & 4 × e 0-9 , ,
Sinus thrombosis e e g & tº g º 0-6 , ,
Lead poisoning. o * g e g sº ... 0-6 ,,
Traumatic hamatoma * º $º g $ * 0-3 ,,
Bony cicatrix of skull * , º e * º ... 0-3 , ,
Retro-bulbar septic processes . gº * º . 0-3 ,,
Pseudo-neuritis with cerebral symptoms . g ... 0-3 , ,
Diagnosis uncertain . ſº * tº t † . 2.2
DISEASES OF THE OPTIC NERVE 2.947
Brain tumours, it is seen, are much the commonest cause, and some 80%
of them are associated with papilloedema (Gowers, 1904; Paton, 1909). In
Paton's (1909) series of 252 cases, every case of temporo-sphenoidal, occipital
and cerebellar tumour was associated with papilloedema, while 87% of
frontal tumours, 86% of parietal tumours, 75% of mid-brain tumours, 68%
of sub-cortical tumours, and 57% of tumours of the pons and medulla were
thus associated. In cerebellar tumours the papilloedema develops particu-
larly rapidly; in cerebral tumours it is usually later in appearance and
slower in evolution. It would seem that the potency of tumours of the
posterior fossa to produce an acute papilloedema in this way, characterized
by an enormous swelling, filiform arteries, engorged veins and plentiful
Fig. 2532.-PAPILLCEDEMA IN CEREBRAL TUMoUR.
Note dilatation of central vein (Paton).
haemorrhages, is their habit of developing an internal hydrocephalus
(Fig. 2532).
The question of the value of an estimation of the degree of papilloedema
in the localization of a tumour cannot be gone into fully here, but will be
considered again at a later stage. It will suffice to say at present that while
the older authors considered that the point was of considerable diagnostic
importance, the most comprehensive investigations show that in the great
majority of cases the degree of swelling is equal in both eyes, and that in
the remainder it is almost as frequently greater and appears earlier on the
contra-lateral side as on the homo-lateral (Paton, 1908–36; Uhthoff,
1914): the time of appearance and the degree of papilloedema are therefore
not to be relied upon as diagnostic criteria.
The other conditions giving rise to increased pressure are much less
common. Brain abscess is associated with papilloedema in a relatively

2948 TEXT-BOOK OF OPHTHALMOLOGY
small proportion of cases—some 25% to 30% (Kampherstein, 1905); and
of these, abscesses in the temporal lobe are commonest. In this condition,
according to Uhthoff (1914), homo-laterality occurs in 80% of the cases, a
consideration of some diagnostic importance. Solitarity tubercle, usually
occurring in the cerebellum or pons, was found by Kampherstein (1905) in
4% of his cases of papilloedema, ; but syphilis as represented by a basal
gummatous meningitis or gummatous tumours is more common (Uhthoff,
1914). An aneurysm is an exceptional cause, but sub-dural bleeding after
head injuries is probably more common than is usually supposed owing to
the absence of routine ophthalmoscopic examination (Liebrecht, 1912;
Cairns, 1929): Furlow (1936) found a mild papilloedema in 68% of such
cases, and King (1937) in 44% of 224 reported cases.
Serous meningitis, if of any severity, frequently produces papilloedema,
but it is usually small in degree, although accompanied by prominent visual
symptoms. In this type the prognosis is good, the Oedema sometimes
disappearing after lumbar puncture (Terrien, 1935). Acute syphilitic
meningitis, a rare disease occurring in the early secondary stage or in
hereditarily infected infants, and occasionally appearing as a neuro-recidive
reaction following insufficient treatment, is not uncommonly associated
with papilloedema (Glass and Garvey, 1928). Drake (1933) found 8 cases of
papillitis and 16 of papilloedema out of 50 cases, the swelling varying from
2 to 5 dioptres, 14 being bilateral and 2 unilateral. Here again the response
to treatment is good. Tuberculous meningitis is a rare cause, papillitis being
the commoner complication ; but an infective traumatic meningitis has been
recorded on several occasions (Wallis, 1917 : Tanasescu and Lazarescu,
1931 ; and others).
Cerebral haemorrhage can give rise to a papilloedema, which, as we have
seen,” is invariable and considerable in extent if the blood enters the optic
nerve sheaths in quantity. In thrombosis of the cavernous sinus, papilloedema
is relatively rare.
Cysticercus is a rare cause of papilloedema : Jacoby (1903), who collected 24 cases,
found that in the majority the parasite was in the fourth ventricle.
(d) Certain general diseases are associated with papilloedema, some of
which are characterized by a definite degree of cerebral oedema and increased
intra-cranial pressure, but in others the association is not at all clear. They
include the anaemias, particularly chlorosis (Hawthorne, 1908; Meller,
1913; Augstein, 1919; Ohashi, 1932), leukaemia (Grunert, 1901; Bondi,
1901 ; Hegner, 1912; Kümmell, 1918; Jaensch, 1929; Hill, 1937; and
others), and sub-acute combined degeneration (Rolleston, 1923), nephritis
and hypertensive states, wherein the swelling of the disc may be extreme
(Figs. 2533 and 2534).” Papilloedema has also been noted in emphysema
(Cameron, 1933) when venous engorgement may enter into its causation; and
1 p. 2942. 2 p. 2727.
DISEASES OF THE OPTIC NERVE 29.49
in association with increased intra-cranial pressure in pregnancy (Schaeffer,
1932). Finally it is possible that it may occur in toxic states associated with
Fig. 2533.-PAPILLCEDEMA IN URAEMIA (MacDonald).
a general retinal oedema which is usually manifested as a stellate retinitis."
Thus the literature contains reports of cases wherein an oedematous nerve-
head subsided rapidly after the elimination of septic foci, such as tonsils
Fig. 2534.-PAPILLCEDEMA IN MALIGNANT
HyPERTENsion (MacDonald).
(Wilson and Drakes, 1933); but the mechanism of such cases is not clear
and their true aetiology unknown.
Unilaterality. In the great majority of cases papilloedema is bilateral.
Cases of ocular or orbital origin are, of course, usually unilateral; but
p. 2644.


2950 TEXT-BOOK OF OPHTHALMOLOGY
cases of unilateral change as well as of unequal change due to increased
intracranial pressure do occur. This may be due to several causes:-
1. A lower intra-ocular pressure on one side than the other.
2. An excess of fibrous tissue on one disc (Paton, 1936), or the presence
of an already existing optic atrophy (Brückner, 1930; Marchesani, 1931-35),
for papilloedema only occurs in a vital nerve.
3. A blockage of the inter-vaginal space on one side from the general
Fig. 2535-Established PAPILLEDEMA.
Ten days after commencement in a case of cerebral tumour. The main mass of
fibres of the lamina cribrosa in normal position (a, aj, but the anterior ones (b. b)
curved forwards and separated (Paton).
sub-arachnoid cavity. This may be due to inflammatory adhesions, as in
syphilitic meningitis, or to the pressure of a tumour near the region of the
optic canalon one side. In this latter event there may be a pressure-atrophy
on the side of the tumour and a papilloedema on the other, a combination
which, although noted by Gowers (1904), Paton (1909), Paton and Holmes
(1911), and others, is usually known as the Foster Kennedy (1911) syndrome.
Apart from these three factors unilateral papilloedema may still occur, although
rarely, in generalized intra-cranial hypertension; and in the present state of our
knowledge of the pathogenesis of the process an explanation is difficult. Two

DISEASES OF THE OPTIC NERVE 2.951
anatomical features may be of some importance. We shall see that obstruction of
the central vein of the retina as it passes through the optic nerve sheaths most probably
enters into the causation of the oedema, and since the distance the vein may run through
the inter-vaginal space may vary greatly (Fry, 1930), one vein may be more exposed
to pressure than the other. Further, it is possible that pressure on one transverse
sinus or a variation in the development and anastomoses of the sinuses might tend
to raise the venous pressure on one ophthalmic vein and thus facilitate the development
of oedema on one side (Swift, 1930; Gibbs, 1932).
Pathology
Pathologically papilloedema is a simple oedema of the optic nerve head
with an oedematous swelling of the nerve fibres and an infiltration of all the
Fig. 2536.-PAPILLCEDEMA.
Part of Fig. 2532 to show the distortion in the course of the peripheral nerve
fibres which take a lateral bulge, describing a double curve from the retina to bend
abruptly round Bruch's membrane to enter the lamina (Paton).
tissues with fluid (Fig. 2535). The most extensive series of cases to be
studied histologically are those of Elschnig (1895) (26 eyes), Kampherstein
(1904) (51 eyes), Schieck (1910) (6 eyes), Paton and Holmes (1911) (60 eyes),
Wilbrand and Saenger (1909) (54 eyes), Behr (1920) (45 eyes), Fry (1931)
(40 eyes), and Samuels (1938) (50 eyes). The area in which the oedema is
marked and intense is usually fairly limited, extending from the retina a
little beyond the margin of the optic disc to end usually fairly abruptly
in the trunk of the nerve at the point where the retinal vessels leave the nerve.
All the tissue-elements, both nerve-fibres and interstitial tissues are perco-
lated and swollen, the fluid appearing inter-fascicularly and intra-fascicularly,
producing a considerable amount of swelling. The lamina cribrosa, particu-
larly its less dense choroidal part, is bowed forwards with a great anterior
convexity (a feature which distinguishes the condition from a neuritis), the

2
9
5
2
TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2537-Papillºn EMA.
Longitudinal section of optic nerve near the disc. a. Nerve-fibre bundles.
b. Edema in sub-pial zone. c. Pia. d. Sub-arachnoid space (Paton).
physiological cup is smaller or quite obliterated, and the tissues of the disc
project into the cavity of the eye, displacing the retina laterally and throwing
it up into small folds (Fig. 2532). The axial nerve fibres become raised to
fill the central depression and the peripheral ones, instead of forming a
regular are between the retina and the nerve, form a double S-shaped curve,
bending laterally and curving acutely backwards before they pass around
the termination of Bruch's membrane to meet the nerve (Fig. 2536). The
outer retinal layers, however, are very
little affected, the only change being a
little oedema close to the disc margin.
The vessels, however, both veins and
capillaries, are invariably distended, while
haemorrhages upon and around the disc
are frequent : when it leaves the nerve,
however, and enters the vaginal space the
central vein is usually compressed in
plerocephalic cases (Figs, 2543–44). In
these the sub-arachnoid space of the nerve
is greatly distended so that it ends
anteriorly at the sclera in a large bulging
cul-de-sac. In the nerve there is invari-
ably much sub-pial cedema distal to the
point of entry of the vessels, a process
which usually extends along the septa;
while the perivascular lymph-sheath of the
central vessels may be markedly dilated.
Fig. 2538.-PAPILLºpºnia. The nerve fibres themselves show
Large varicosities of the peripheral important alterations, oedematous changes
nerve fibres of optic dise next to the appearing first in the periphery of the
retina in an early case (Cone and -
MacMillan). nerve trunk and spreading subsequently


DISEASES OF THE OPTIC NERVE 295.3
Fig. 2539.-PAPILLOEDEMA : Swell ING or THE NERVE FIBREs.
Early swelling of the nerve fibres, the neuro-fibres being separated at the points
of swelling by imbibed fluid. (Gros-Bielschowsky's stain) (Cone and MacMillan).
Fig. 2540.-PAPILLEDEMA : VARI- Fig. 25.41.-CYToID BoDIEs.
cosities IN NERVE FIBREs. -
In some instances the nerve fibre can be seen
Neuro-fibrils can be followed from entering and leaving the bodies (Cone and
the undilated part of the fibre into the MacMillan).
varicosities, where they break into fine
granules. (Gros-Bielsehowsky's stain)
(Cone & MacMillan).


2954 TEXT-BOOK OF OPHTHALMOLOGY
into the axial parts, the pathological process thus following the changes
in the visual fields (Fig. 2537). At an early stage the non-medullated
fibres upon the disc, particularly those near the margin, swell up and show
varicosities wherein the fine neuro-fibrils are seen clearly separated by
the oedematous fluid (Figs. 2538–39). As the swelling progresses these
varicosities multiply and enlarge until the papilla is filled with them
(Fig. 2540). After a variable time degenerative changes begin to develop:
the neuro-fibrils in the varicosities disappear and fine granules fill the fusiform
enlargements. Eventually the granular structure becomes homogeneous,
and the enlargements appear to lose connection with the fibres from which
- - .
- --- -
- --------
--------
- - - º: º
Fig. 2542–AdvancED ATRoPax Apter Papillºoemia.
Showing marked glial sclerosis (Paton).
they have developed: at this stage they are called cytold BoDIEs (Fig. 2541),
and as degeneration proceeds these, too, suffer lipoid degeneration and
disappear. Finally a centripetal Wallerian degeneration develops in the
nerve fibres, and an axonal chromatolysis in their retinal ganglion cells of
origin, while the usual reaction of the neuroglial tissue completes the picture
of degeneration –a phagocytosis of the degenerated neural elements by
the microglia, and their replacement by a marked proliferation of the
fibrous astrocytes (Figs. 2519 and 2542).
During all this process the evidences of inflammatory changes are
negligible although they were stressed by the earlier writers (Elschnig,
* p. 29.30.




DISEASES OF THE OPTIC NERVE 2955
1895). At the most there is occasionally a scanty perivascular infiltration
with lymphocytes, but the inflammatory cells are so few and incidental
that it is only legitimate to conclude that they represent a secondary reaction
to the destructive and degenerative changes occurring in the nerve. This
is so even when papilloedema occurs in septic meningitis involving the optic
nerve sheaths, in which case there is no particular evidence of inflammation
in the tissues of the swollen disc (Paton and Holmes, 1911) : it is obvious,
therefore, that the process is not an inflammatory condition but a passive
Oedema.
Pathogenesis
Ever since v. Graefe (1860) first theorized on the subject, the mechanism
of the formation of papilloedema has excited an immense amount of research
and controversy, but to-day the question is still unsettled. At the present
time the consensus of opinion inclines to a mechanical explanation of the
phenomenon as being due to a circulatory obstruction in the region of the
optic nerve, although different opinions have been advanced as to the precise
mechanism of the obstruction. The facts of its occurrence with a low intra-
ocular pressure or a high intra-cranial pressure, and its dramatic relief by an
adjustment of the pressure to a normal level admit of no other conclusion.
It will be well, however, first of all, to dispose of several of the more unlikely
theories.
1. An inflammatory theory was first formulated by Gowers (1879) and supported
by Leber (1881), who considered that an Oedematous inflammation was set up by
toxic material associated with the intra-cranial disease. Although supported by
Deutschmann (1887) on experimental grounds, and by Edmunds and Lawford (1884),
Elschnig (1895), and others on histological grounds, we have already seen that later
pathological study has clearly demonstrated that the essential factor is a passive
Oedema.
2. Vaso-motor theories were put forward by several of the older authors. Hughlings
Jackson (1863) and after him Loring (1882), ascribed the condition to an upset
of the nerves controlling the circulation and nutrition of the optic nerve, Benedict (1868),
considered a derangement of the sympathetic system responsible, and Adamkiewicz
(1895), classed papilloedema as a neuro-paralytic disturbance. A theory of
somewhat similar type was formulated by Kornder (1919) based on experimental
work on dogs in which he blocked the aqueduct of Sylvius by the intra-cranial injection
of paraffin : he considered papilloedema due to the effects of a venous stasis which was
part of a generalized increase in systematic venous pressure due to an accentuated
inhibitory action of the vagus resulting from the central stimulation of the raised
intra-cranial pressure.
The more mechanical theories of the mechanism of papilloedema are
based on experimental work which is to some extent conflicting. They may
be divided into four groups—those attributing the causal mechanism
essentially to venous obstruction in the region of the optic nerve ; those
assuming an obstruction of a normal flow of tissue fluid centripetally from
the eye along the optic nerve ; those postulating a forcing of cerebro-spinal
2956 TEXT-BOOK OF OPHTHALMOLOGY
fluid into the tissue of the nerve; and those considering the swelling of the
nerve-head to be part and parcel of a general oedematous condition of the
brain. The first three of these groups, all of which assume local pressure
conditions as the causal agent, have as their starting point the demonstration
Fig. 2543.-PAPILLoºpewa,
Cross section showing central vessels in nerve before exit of vein; the vein is
fully dilated (Paton).
Fig. 2544.—Papillºpºxia.
The vein in cross section in sub-arachnoid space after leaving nerve; it is com-
pressed (Paton).
by Schwalbe (1870) that the inter-vaginal space of the nerve is in free
anatomical communication with the sub-arachnoid space of the brain and
that a fluid-pressure could readily be transmitted from one to the other.
3. Theories of Venous Engorgement. The original theory advanced by
v. Graefe (1860) comes under this category: he suggested that the tumour
of the brain pressed upon the cavernous sinus, thus producing congestion of


DISEASES OF THE OPTIC NERVE 2957
the ophthalmic veins. On the demonstration by Sesemann (1869) of the
free inter-communication between the Ophthalmic and facial veins, however,
he retracted his opinion ; the theory has not been revived until Swift (1930)
suggested that rudimentary or ill-developed anastomoses of the transverse
sinuses—which show numerous and profound variations (Woodhall, 1936)—
might exert a determining influence in its production. Most authors,
however, place the obstruction locally at the nerve-head itself. This
view was put forward initially by Manz (1865), who concluded from
experimental work that the compression occurred at the lamina cribrosa.
Deyl (1899), however, being struck with the distension of the inter-
vaginal space, attributed the obstruction to the acute bending and com-
pression involved as the vein entered the dura (Fig. 2527), a view
supported by Knapp (1909). Dupuy-Dutemps (1900), however, considered
that the compression occurred in the inter-vaginal space, a suggestion con-
firmed by Merz (1900) by experiments on rabbits. The same position was
taken up by Paton and Holmes (1911), who concluded from their elaborate
pathological studies, particularly from the fact that the oedematous changes
were essentially limited to the distal part of the nerve wherein the central
vessels lay, that the compression of the central vein occurred in its intra-
vaginal course where it was reduced to slit-like dimensions as it became
subjected to the increased sheath pressure (Figs. 2543–44). As a result of
the venous engorgement thus produced, fluid transuded into the eye in
Quantity while the increased sheath pressure prevented its drainage, all of
which factors contributed to the production of papilloedema. This view of
venous compression in the inter-vaginal space was concurred with patho-
logically by Fry (1930–31), who advocated as a secondary cause a forward
propulsion of the cerebro-spinal fluid along the perivascular lymphatic spaces.
4. Theories depending on the blockage of the tissue-fluid drainage from the eye
along the optic nerve were introduced by Rochon-Duvigneaud (1895), advanced
as a part explanation by Paton and Holmes (1911), and have been more fully
elaborated by Saenger (1911), Behr (1911–37), Levinsohn (1912–29),
Liebrecht (1922), Berens, Smith and Cornwall (1928), and others. According
to this view, which has been most ably advocated by Behr (1911–37), there
is normally a stream of tissue-fluid from the disc centripetally along the
nerve, interference with which by pressure leads to stasis and oedema.
There is no doubt that such a theory has many attractive features, but that
such a flow of fluid is responsible for the phenomenon has never been
adequately demonstrated.
The experimental evidence upon which the conception of a flow of tissue-fluid
from the eye centrally along the Optic nerve is somewhat contradictory. Nine human
eyes have been experimented upon with a view to demonstrate such a current, two
by Nuel and Benoit (1900), one by Weekers (1922), one by Schneider (1924) and five
by Berens and Posner (1933). The first three authors used indian ink injected into the
vitreous ; in no case was the ink found in the Optic nerve. Berens and Posner used
2958 TEXT-BOOK OF OPHTHALMOLOGY
as an injection-fluid the more physiological potassium ferrocyanide solution, and
found that it gained access to the perivascular regions of the nerve in the one eye
which was normal, but not in the other four which were pathological. In animals
the results vary : all are agreed that a posterior drainage occurs in rabbits (Ulrich,
1884; Gifford, 1886; Nuel and Benoit, 1900; Weekers, 1923; Berens and Posner,
1933), but in dogs and pigs negative results have been obtained (Weekers, 1923;
Berens and Posner, 1933). It will be remembered that in excised animals’ eyes Priestley
Smith (1888) and Niesnamoff (1896) concluded from manometric experiments that a
small portion, about one-fiftieth, of the intra-ocular fluid left the globe through the
optic disc. From these results it is difficult to reach a definite conclusion, and the
difficulties are further increased by the demonstration by Behr (1914) and Krückmann
(1917) that the glial-ectodermal system of spaces within the substance of the nerve and
retina is not in communication with the mesodermal perivascular spaces. Although
Behr claimed to have demonstrated a centripetal current within the glial spaces of
the nerve, he concluded that the fluid there was tissue-fluid of the nerve-tissue itself,
and was quite unrelated to the intra-ocular fluid and its drainage.
5. A theory explaining the phenomena of papilloedema on the supposi-
tion that cerebrospinal fluid was forced under pressure into the optic nerve was
first put forward by Schmidt-Rimpler (1888), and has been most fully
elaborated by Schieck (1910–24). He postulated that the cerebro-spinal
fluid was forced along the perivascular sheaths of the axial bundle to the
disc, and supported his contention (1937) by experimental evidence wherein
dye was injected under pressure into the sub-arachnoid space of a freshly
excised human eye and took such a course. His views were supported on
experimental evidence by Fry (1931) and Jeffers, Griffiths, Fry and Fewell
(1937); but it must be noted that most workers who have attempted to
demonstrate such a flow of fluid have had negative results (Schwalbe, 1870;
Leber, 1877; Wolff and Davies, 1931).
6. That the spreading forward of an oedematous process in the brain was
responsible for the condition was suggested by Parinaud (1879), Ulrich
(1887), Sourdille (1901) and Kampherstein (1904). It is well known that
the brain-tissue in the neighbourhood of a tumour becomes oedematous ;
and it was shown by Cannon (1901) that under all conditions of malnutrition
the brain tended to take up water Osmotically, presumably owing to the
retention of metabolic products, and hence to swell to a degree sufficient to
exert a pressure strong enough to exclude the blood from the cerebral vessels.
Simultaneously with the brain, the optic disc becomes oedematous and
papilloedema in this view is thus merely a local eacpression of a general cerebral
o:dema. Such a theory has been especially advocated by Marchesani (1930–34)
as an explanation not only for the papilloedema of cerebral tumours, but also
of hypertension and uraemic states, his advocacy being based on experiments
such as the injection of one carotid with distilled water and the other with
10% saline, a procedure which is followed by a papilloedema in the first
case and an excavation of the disc in the second.
Van Heuven and Fischer (1935–37) have shown that the brain has a
water-binding capacity comparable to that of a true gel, a property in which
DISEASES OF THE OPTIC NERVE 2959
the optic nerve shares : moreover, it appears that the intra-bulbar portion
of the nerve swells most markedly, and that it swells in an axial direction
towards the globe. The retina, on the contrary, swells to very much less
extent, a fact which would explain the limitation of the process to the
neighbourhood of the nerve-head. Papilloedema may therefore be regarded
as swelling in the colloid-chemical sense, constituting part of the general
reaction of the central white matter.
That a simple increase in intra-cranial pressure alone is not sufficient
to account for the occurrence of papilloedema must probably be admitted.
Some experimenters claim to have produced swelling of the nerve-head by
the simple injection of fluid under pressure into the sub-arachnoid spaces
(Manz, 1870; Merz, 1900; Cushing and Bordley, 1909), but most have failed
to produce anything more than venous engorgement even although the
pressure was maintained at a great height for long periods (v. Schulten,
1885; Adamkiewicz, 1895–1905; Kampherstein, 1905; Wolff and Davies,
1931 : Lindberg, 1934; van Heuven, 1938). That a venous blockage
with consequent stasis and Oedema plays some part, perhaps a crucial
part, in its development seems likely ; and it seems obvious that the
point of pressure is in the inter-vaginal space. The factor of venous com-
pression is suggested pathologically by the limitation of gross changes to
the distal end of the nerve up to the point of exit of the vessels, and experi-
mentally by the negative results obtained by ligating the optic nerve in
animals in its proximal half, in contrast to the positive results when the
ligature is distal to the exit of the blood-vessels (Kyrieleis, 1936; Campos,
1937), and the rapid and extreme swelling obtained when the vein and not
the artery is included in the ligature so that an unimpeded arterial circulation
increases the congestion (Sobanski, 1934–37). It has been demonstrated,
and it is an obviously logical proposition, that in general terms there is a
fairly close relationship between the venous pressure at the disc and the
intra-cranial pressure ; the former keeps some 2 to 4 mm. Hg above the
latter in order to allow the circulation to be maintained, rising with it step
by step until the cerebro-spinal pressure reaches the intra-ocular arterial
pressure at which point the circulation ceases (Baurmann, 1935). It was
found by Sobanski (1937) that if the normal relationship between the
pressure in the central vein and artery (usually 1 : 3) was seriously disturbed
so that the two approximated, papilloedema was prone to develop : if the
arterial pressure rose with the intra-cranial pressure (that is, with the venous
pressure), no oedema followed, but if it did not and the relation between the
venous and arterial pressure approximated 1 : 1-5, papilloedema invariably
resulted.
Because it is able to maintain the circulation adequately, a high arterial pressure in
the central artery thus tends to prevent the development of papilloedema, whilst a low
pressure facilitates it. For this reason Lindberg (1935) found the optimal intra-cranial
T.O. —WOI. III. 3 K
2960 TEXT-BOOK OF OPHTHALMOLOGY
pressure for the production of papilloedema to be 40 mm. Hg : at this point the
difference between the pressure of the arteries and veins is minimal and stagnation
is encouraged ; above this point the blood-pressure tends to become raised by a
general vaso-motor reaction to overcome the cerebral anaemia, and a greater difference
becomes established.
That simple venous congestion, however, cannot be the whole story is
certain, since venous congestion of an extreme degree need not necessarily
cause a true papilloedema, as has been shown experimentally (Cushing and
Bordley, 1909), and is seen clinically in cases of complete venous obstruction;"
moreover its effects should apply equally over the whole retina in which the
circulation is terminal. Whether the additional factor is an obstruction of a
flow of tissue-fluid centripetally along the nerve, or a pushing of fluid into
the nerve-head, is questionable, but possible. The more likely explanation is a
colloid-chemical one, the water-binding properties of the tissues being altered
in the upset of metabolism caused by the venous stasis and the derangement
in the normal traffic of the tissue-fluids. The absence of papilloedema in an
atrophic nerve is significant from this point of view. What the factors
causing this hypothetical change may be, however, are at present quite
unknown.
Clinical Picture
In the early stages of papilloedema the clinical signs may be significant :
they can best be appreciated stereoscopically by the binocular Gullstrand
ophthalmoscope. Most authorities consider the first evidence to be an
increased redness of the disc, with a slight haziness and blurring of its
margin, always appearing first at the upper and lower margins, usually the
upper nasal quadrant, spreading thence round the nasal margin, and appear-
ing last at the temporal margin (Paton, 1905; Paton and Holmes, 1911 ;
Best, 1916; Behr, 1916–20). This distribution depends on the density of
the nerve fibres, for it will be remembered that with the papillo-macular
bundle occupying the greater part of the Outer aspect of the disc, the upper
and lower quadrants are the most heavily crowded with nerve fibres (Fig.
2867). An indistinctness and blurring is often seen at a very early stage
also in the centre of the disc where the physiological cup becomes filled in,
the tissues become veiled, and grey streaks appear along the central vessels :
these delicate central changes are presumably due to a distension of the
perivascular lymphatics with fluid, and is claimed by Schieck (1911–37),
Meesmann (1921), and Koeppe (1922) to be in evidence at an even earlier
stage than the swelling of the disc margin. These appearances are usually
associated with a slight but perceptible turgescence of the veins. The
general blurring is not only due to the swelling of the tissues of the nerve-
head but also to the dispersal of light by the accumulated fluid, and the
increase of redness is due to injection of the smaller vessels, a phenomenon
1 p. 2578.
DISEASES OF THE OPTIC NERVE 2961
which gradually develops into a characteristic juicy appearance in the
well-developed stage. Over the margin and for some little distance along
the retina, a greyish-white striation is frequently obvious, due to separation
of the nerve fibres by Oedema. A third early sign is the absence of a venous
pulse at the disc, and the impossibility of eliciting it by pressing upon the
globe until the intra-ocular pressure is thus raised to a higher level than the
intra-cranial pressure (Baurmann, 1925–35).
Usually after a week or ten days, the blurring of the edges has passed
round the disc leaving only the outer margin still visible, and the central
cup has filled up ; at this stage there is usually some measurable swelling,
usually less than 2 dioptres, sometimes showing a distinct difference between
the nasal and the temporal sides. During the course of another week in
the average case of tumour, the disc has assimilated itself to the colour of
the surrounding retina so that its position is indicated only by the confluence
of the larger vessels, small linear haemorrhages frequently appear, and
its surface may become speckled with white dots while white exuda-
tive patches are seen on its margin (Fig. 2529, Plate LX). Sometimes slowly,
sometimes rapidly the swelling increases, until eventually a great forward
protrusion of the papilla develops averaging 5 to 7 dioptres, but sometimes
8 or 9 dioptres or more, its edges overlaying the surrounding retina above
which it bulges mushroom-like so that parallax can be elicited, and the
vessels are not only bent acutely and lose their reflex as they dip down on
to the retina, but may be buried by the swollen tissues and appear to be
completely interrupted in their course (Fig. 2531, Plate LX). At this stage,
while the arteries remain small, the veins are always enormously distended
and engorged with dark blood, and the tissues of the disc itself lose their
transparency and become increasingly opaque. Occasionally around it fine
concentric lines are seen, marking the folds into which the retina is thrown
as it is displaced laterally by the swollen tissues (Fig. 2530, Plate LX).
The occurrence of haemorrhages is very irregular ; they may be the
first sign to appear, and they may be altogether absent when the swelling
has reached an extreme degree. Usually, however, they are obviously
present, most frequently as superficial flame-shaped haemorrhages in the
nerve fibre layer, but sometimes as rounded masses in the outer molecular
layer at the level of capillary distribution. On the disc itself the soft-edged
white patches are produced by the swelling and degeneration of the nerve-
fibres marking the formation of cytoid bodies ; and over the retina the oedema
may become evident, being always most marked where it is confined by the
configuration of the macular region. Between this point and the disc fluid
vesicles collect under the internal limiting membrane, and, reflecting the
light brilliantly, appear as a radiate arrangement of white spots, smaller than
the exudative accumulations of the macular star of renal retinopathy, and
forming usually a macular fan between the fovea and the disc instead of a
complete star-shaped figure (v. Graefe, 1866; Schmidt and Wegner, 1869)
3 K 2
2962 TEXT-BOOK OF OPHTHALMOLOGY
(Fig. 2543). Paton (1909) found such a configuration 22 times in 146 cases,
and it may disappear temporarily in the relief obtained by lumbar puncture
(Colrat, 1930).
If the papilloedema subsides, little or no permanent changes may result;
on occasions it disappears completely after remissions and relapses, but if
it has persisted for long the appearances of secondary atrophy generally
result (Fig. 2530, Plate LX). These are due to the destruction of nerve
fibres and their replacement by neuroglia. A decided subsidence of the
vascularity of the disc becomes evident, usually with a sinking of its
prominence, an increasing pallor of its surface, and an increasing con-
traction of the arteries and a thickening of their perivascular sheaths,
Fig. 2545.-PAPILLEDEMA showing FAN-shaped MacULAR Figure
(Paton).
until eventually the final picture of secondary atrophy becomes established."
Symptoms. The symptomatology of papilloedema may be very vague
and remain slight even when the swelling has been long established, some-
times for months or years, and has reached an extreme degree; in this it
differs from papillitis, for a marked oedema with normal visual acuity is by
no means uncommon. Frequently, however, premonitory transient attacks
of blurred vision or even blindness occur, which, although they pass off
rapidly, usually give rise to a considerable amount of apprehension. Their
cause is unknown, and several explanations have been advanced ; that
they are cortical in origin, a type of epileptic amaurosis (Jackson, 1873), that
they are due to sudden pressure from the third ventricle through the infundi-
bulum upon the chiasma (Leber, 1877), or to a temporary spasm of the retinal
arteries as Harms (1906) observed, or to sudden compression of the nerve
at the optic canal (Behr, 1930). The fact, however, that these premonitory
p. 3067.

DISEASES OF THE OPTIC! NERVE 2963
attacks are commonest with cerebellar tumours suggests that pressure on the
chiasma by an internal hydrocephalus may be responsible for the
phenomenon (Paton, 1909; Holmes, 1937).
Although good central vision and normal pupillary responses are fre-
quently found with a marked degree of swelling and even in the presence of
a macular star, it is more usual for visual symptoms to become increasingly
evdent; but in the oedematous phase these are usually slight, for they
do not become evident until atrophy is advanced. Perimetrically the
earliest sign is a concentric enlargement of the blind spot (Knapp, 1870),
the onset of which is usually gradual, the area corresponding roughly with
the degree of oedema , it is due to the increase in size of the disc and the
lateral displacement of the retina. The intensity of the scotoma diminishes
wº- *
Žº
fº
Q 42 °2
Sºº
af
Yºr
º
wº sº
\ºs IIII
ºftºſ)
NºHºº'ſ]
& Hº
Q}^{+\º
29
& SEº
Hºt O
Soº O4; 2 33%
FIG. 2546.-PAPILLCEDEMA IN BRAIN FIG. 2547.-ADVANCED POST-
TU MOUR. PAPILLOEDEMA ATROPHY.
In the early stage of oedema. 10, 1/330.
5/330 ; 25/2,000; 6/2,000.
towards the periphery so that it shows sloping edges, and it may extend for
several degrees around the normal blind spot and reach out towards the
fixation spot (Fig. 2546). If the oedema involves the macular area a relative
central scotoma develops in which blue blindness is predominant ; this
produces a diminution of central vision, and ultimately in severe cases the
two may meet, producing a relative centro-caecal defect.
While this central depression of vision is the only symptom of the
oedema as such, when atrophy sets in, a concentric contraction of the peripheral
field becomes apparent. As atrophy progresses the contraction increases
apace, usually more rapidly on the nasal side than on the temporal so as
sometimes to simulate a bi-nasal hemianopia, and eventually in the later
stages a small field more or less central in position and embracing the blind
spot remains (Fig. 2547). Finally, this too disappears, and permanent
blindness with a widely dilated immobile pupil results.
























2964 TEXT-BOOK OF OPHTHALMOLOGY
While in a general sense the enlargement of the blind spot and the depression of
central vision are due to the Oedema, and the progressive peripheral contraction to
the subsequent atrophy, complications may occur owing to localized interference
with the visual paths. Thus accidental special interference with a section of the
nerve at the disc margin, particularly in the upper nasal region, may produce a nerve-
bundle defect, such as a Bjerrum Scotoma or a nasal step as occurs in glaucoma
(v. Szily, 1913; Rönne, 1913; Löhlein, 1914). An absolute central scotoma is a
rarity and should suggest an orbital optic nerve lesion or an affection of the nasal
sinuses, but other varied defects (bitemporal or quadrantic hemianopia) may result
from localized pressure, as by a tumour, higher up the visual pathway. The general
symptomatology, of course, is frequently obscured by the more clamant signs of
intra-cranial pressure—headaches, vomiting and stupor.
The diagnosis which involves a differentiation between papilloedema, pseudo-
neuritis and papillitis will be considered when dealing with papillitis.”
Prognosis and Treatment. As a rule, unless the causal disease is
treatable, as in syphilis, or unless a decompression can be done, the course of
papilloedema is chronic and the prognosis bad. To this general rule there
are some exceptions. In the first place it is to be remembered that even in
cases which have reached an intense stage, the vision may remain unimpaired
for long periods, even 6 or 7 years (Gowers, 1879; Holmes, 1937; and
others). In other rarer cases the condition may subside completely without
visual impairment, as may occur in tuberculomata, gummata, degenerated
gliomata, a transient internal hydrocephalus, or a serous meningitis asso-
ciated with an infective process within the skull (Gowers, 1879; Krimsky,
1936; Holmes, 1937; and others). These cases of transient papilloedema
which recover, sometimes ingenuously referred to as “pseudo-tumours,”
undoubtedly exist and should give pause to any hasty decision for an
immediate cranial operation as soon as papilloedema is diagnosed, for
the effects of such an operation must always be considerable. In the
great majority of cases, however, if the patient is to be saved from blindness,
the cause of the condition must be removed, or if that prove impossible,
relief must be obtained by a decompression operation on the skull. The
indications for such a step are a marked degree of swelling (above 5 dioptres),
a great engorgement of the veins, the presence of extensive haemorrhages
and the early appearance of exudative spots, and above all, a progressive
deterioration. When these changes come on rapidly the indication for
surgical intervention is more urgent; and when the earliest signs of atrophy
appear it becomes imperative. Before the stage of consecutive atrophy the
prognosis for vision is good ; when atrophy has commenced it may progress
despite decompression, and the result of the operation is doubtful ; if it is
advanced, operation may accelerate it and become dangerous and may, indeed,
be better left undone so far as vision is concerned. It is therefore of extreme
importance to observe such cases carefully and repeatedly and to note the
1 p. 2980.
DISEASES OF THE OPTIC NERVE 2965
first indications of atrophic change. Ophthalmoscopically the first danger
signal is usually a diminution of the calibre of the arteries indicating the
commencement of constriction by glial tissue ; an increasing greyness and
density of the swollen disc and a subsidence of the swelling come later. The
most readily detected feature, however, is probably the commencement of
peripheral contraction of the visual fields and these should be repeatedly
examined, using small visual angles under constant conditions in every case
wherein surgical relief is being deliberately postponed.
When a decompression operation is done, improvement is usually rapid
and dramatic ; occasionally, however, in infective and meningeal conditions
the subsidence may be delayed or the swelling may even be temporarily
increased (Meyer, 1930). Immediately the dura (not the skull) is opened
the appearance of the vessels on the disc may change, a small artery hitherto
occluded may fill and an over-filled vein may be less congested. In the
following two weeks the swelling subsides markedly, the outer edge clearing
first and the other margins appearing again in the inverse order of their
disappearance, a process which goes on in steady progression until at the end
of two months the swelling has disappeared and the tissues of the disc are
gradually resuming their normal proportions. For some time, however,
in cases which have been relieved before atrophy has set in, the disc remains
muddy and solid-looking, and the central cup remains filled although the
vessels are of normal proportions. In the course of some months, however,
the disc may regain its normal clarity. The physiological cup re-appears,
the edge becomes sharp, and no evidences of the disturbance may remain
except perhaps a derangement of the pigment around, which looks as if it had
been carried away from the margin of the disc and left stranded like wrack
on the sea-shore. The macular changes also tend to clear away completely,
except perhaps for the persistence of occasional dusky dots. On the other
hand, if atrophy has occurred, the disc generally has a filled-in appearance,
the colour is grey or papery white and opaque, and the arteries and veins
become thread-like and are partially concealed by white perivascular tissue.
Occasionally after the operation there is a rapid deterioration of sight,
sometimes accompanied by an increase in congestion and haemorrhages;
but this is transient and is probably due to the upset of sudden changes of
pressure ; sometimes, however, and fortunately very rarely, such a relapse
is unexpectedly and distressingly progressive and permanent. Much more
commonly, if the impairment of vision before operation has been slight, it
remains good and may even improve considerably ; but it is to be remem-
bered that if it has been seriously reduced, any appreciable recovery is
extremely rare and is not to be anticipated.
An interesting suggestion applicable to cases which demand decompression
and in whom decompression of the skull seems unwarranted, was made by Gomez-
Marquez (1935)—to drain the sub-arachnoid space locally by an approach exposing
the optic nerve after a lateral canthotomy and a division of the external rectus muscle.
2966
TEXT-BOOK OF OPHTHALMOLOGY
Adamkiewicz. Z. f. kl. Med., xxviii, 28, 1895.
New rol. Zb., xxiv, 206, 1905.
Augstein. K. M. Aug., lxiii, 174, 1919.
Baurmann. B. O. G. Heidel.., xlv., 53, 1925.
A. f. O., crxxiv, 192, 1935.
Behr. Neurol. Zb., xxx, 66, 1911.
R. M. Aug., l, 56, 1912; lvii, 465, 1916.
B. O. G. Heidel., xxxviii, 14, 1912.
A. f. O., lxxxix, 1, 265, 1914–15 ; ci, 165,
1920; czXxiv, 249, 1937; cxxxvii, l,
1937.
Kl. W., ii, 1818, 1928.
Z. f. Aug., lxxi, 275, 1930.
Belgeri and Arana. Bol. inform. oft., v, 237,
1932.
Benedict. Allg. wien. med. Zeit., xiii, 21, 1868.
Berens and Posner. Am. J. O., xvi, 19, 1933.
Berens, Smith and Cornwall. A. of Newrol.
Psy., xx, 1151, 1928.
Best. B. O. G. Heidel.., xl, 95, 1916.
Bondi. Prag. med. W., xxvi, 311, 1901.
v. d. Borg. K. M. Aug., xlvi (1), 359, 1908.
Brückner. B. O. G. Heidel.., xlviii, 359, 1930.
Cairns. T. O. S., xlix, 314, 1929.
Cameron. Brit. J. O., xvii, 167, 1933.
Campos. A. di Ott., xliv, 211, 1937.
Cannon. Am. J. Phys., vi, 91, 1901.
Carle. Forhandl. oftal. Selsk., 31, 1931.
Collins. T. O. S., xxi, 100, 1901 ; xxxvi,
204, 1916 : xxxvii, 281, 1917.
Colrat. A. d’O., xlvii, 773, 1930.
Cone and MacMillan. The Optic Nerve. In
Penfield’s Cytology and Cellular Path. of
the Nervous System, N.Y., 1932.
Cushing and Bordley. Bull. Johns Hopkins
Hosp., xx, 95, 1909.
Deutschmann. Ueber Newritis optica, Jena,
1887.
Deyl. O. Kl., ii, 27, 1898.
Wien... kl. Rundschaw, xiii, 165, 1899.
Doyne. T. O. S., xxv, 161, 1905.
Drake. A. of O., ix, 234, 1933.
Dupuy-Dutemps. These, Paris, 1900.
Edmunds and Lawford. T. O. S., iv., 172,
1884.
Elschnig. A. f. O., xli (2), 179, 1895.
|Wien. kl. Rundschau, xvi., 41, 1902.
Eppenstein. K. M. Aug., lxxxv, 672, 1930.
Fry. A. of O., iv., 180, 1930; vi. 921, 1931.
Am. J. O., xiv, 874, 1931.
A. f. O., lviii, 391, 1904; cxx, 694,
1928.
Furlow. A. of Surg., xxxii, 688, 1936.
Gibbs. A. of Newrol. Psy., xxvii, 828, 1932;
xxxv, 292, 1936.
Gifford. A. f. Aug., xvi, 421, 1886.
Gilbert. A. f. O., lxxvii, 199, 1910.
Glass and Garvey. Am. J. O., xi, 377, 1928.
Gomez-Marquez. Bull. S. d’O. Paris, xlvii,
814, 1935.
Gowers. Medical Ophthalmoscopy, 1879.
The Ophthalmoscope in Medicine, 1904.
v. Graefe. A. f. O., vii (2), 58, 1860; xii (2),
120, 1866.
Graf and Mikulinska. A. d’O., xlv., 288, 1928.
Grunert. Cb. pr. Aug., xxv, 225, 1901.
Harms. B. O. G. Heidel., xxxiii, 253, 1906.
Hawthorne. Lancet, ii, 857, 1908.
Hegner. K. M. Aug., l, 119, 1912.
Heine. B. O. G. Heidel.., xlv., 132, 1925.
v. Heuss. K. M. Aug., lxxxviii, 240, 1932.
v. Heuven. T. O. S., lviii (2), 549, 1938.
v. Heuven and Fischer. Brit. J. O., xix, 390,
1935 ; xx, 204, 1936; xxi, 352, 1937.
Am. J. O., xx, 1127, 1937.
Bul. S. fr. d’O., xxxviii, 700,
Hill.
v. d. Hoeve.
1925.
Holmes. T. O. S., lvii, 3, 1937.
Inouye. A. f. O., lxxxi, 238, 1912.
Jackson, Hughlings. R. L. O. H. Rep., iv
10, 389, 1863; vii, 573, 1873.
Jacoby. K. M. Aug., xli (2), 223, 1903.
Jaensch. A. f. O., czkii, 618, 1929.
Jeffers, Griffith, Fry and Fewell. Am. J. O.,
xx, 457, 881, 1937.
Rampherstein. K. M. Aug., xlii (1), 501,
1904; xliii (1), 449, 588, 728, 1905.
Kennedy. Am. J. Med. Sc., cxlii, 355, 1911.
King. Am. J. O., xx. 149, 1937.
FClauber. K. M. Aug., lx, 504, 1918.
Kljacko. Russ. A. Oft., v, 503, 1929.
Knapp. T. Am. O. S., i, 118, 1870.
Mitt. a. d. Aug. d. Carol. med.-chir. Inst.,
Stockholm, x, 1, 1909.
K. M. Aug., lxxxvii, 255, 1931.
y
Koeppe. A. f. O., xcix, 121, 1919; ciz, 454,
1922.
Rornder. A. Int. Med., xxiii, 197, 1919.
Krimsky. A. of O., xv, 36, 1936.
Krückmann. Z. f. A ug., xxxvii, 1, 1917.
Rümmell. A. f. O., xcv, 105, 1918.
Kyrieleis. A. f. O., czzi, 560, 1929; cz.xxv,
100, 1936.
Leber. G.-S. Hb., I, v (7), 778, 1877.
Intermat. Cong. Med. London, iii, 53, 1881.
Levinsohn. A. f. O., lxxxi, 15, 1912; czXiii,
34, 1929.
Liebrecht. B. O. G. Heidel.., xxx, 172, 333,
1902.
A. f. O., lxxxiii, 525, 1912.
A. f. Aug., xci, 84, 1922.
Lindberg. Finska Läk. Sällsk., lxxvi, 694,
1934.
A. f. O., cxxxiii, 191, 1935.
Löhlein. A. f. Aug., lxxvi, 165, 1914.
Loring. New York Med. J., xxxv, 561, 1882.
Manz. K. M. Aug., iii, 281, 1865.
A. f. O., xvi (1), 265, 1870.
Marchesani. Zb. ges. O., xxiv, 191, 1930.
A. f. Psy. Neurol.., xev, 447, 1931.
A. f. Aug., cvii, 238, 1933.
B. O. G. Heidel., 1, 354, 1934.
K. M. Aug., xciv, 98, 1935.
Meesmann. K. M. Aug., lxvi, 417, 1921.
Meller. Cb. pr. Aug., xxxvii, 271, 1913.
Merz. A. f. Aug., xli., 325, 1900.
Meyer. K. M. Aug., lxxxiv, 671, 1930.
Niesnamoff. A. f. O., xlii (4), l, 1896.
Nuel and Benoit. A. d’O., xx, 161, 1900.
Ohashi. Acta S. O. Jap., xxxvi, l l 1, 1932.
DISEASES OF THE OPTIC! NERVE
2967
4 n. d’Oc., lxxxii, 5, 1879.
J. A. m. Med. As., lxvii. 1053, 1916.
Path. of the Eye, London, iv, 1349,
Parinaud.
Parker.
Parsons.
1908.
Paton. T. O. S., xxv, 129, 1905; xxxiv, 252,
1914 : xxxviii, 170, 1918 ; xlii, 104,
1922 ; li, 364, 1931.
Brain, xxxii, 65, 1909.
A. of O., xv., 1, 1936.
Paton and Holmes. Brain, xxxiii, 389, 1911.
T. O. S., xxxi, 117, 1911.
Pfingst. A. of O., xvi, 829, 1936.
Polanyi. Orvosi hetil., ii, 1318, 1929.
Rochon-Duvigneaud. A. d’O., xv, 401, 1895.
Rolleston. T. O. S., xliii, l 10, 1923.
Rönne. A. f. Aug., lxxiv, 180, 1913.
Rumjanzewa. A. f. Aug., lxxii, 391, 1930.
Saenger.
Samuels. Am. J. O., xxi, 1242, 1938.
Scardapane. Saggi di Oft., v, 408, 1930.
Schaeffer. A. d’O., xlix, 325, 1932.
Schieck. K. M. Aug., xlviii (2), 150, 1910.
B. O. G. Heidel.., xxxvi, 179, 1910.
Die Genese d. Stauungspapille, Wiesbaden,
I9 I ().
A. f. O., lxxviii, 1, 1911 ; cziii, 157, 1924;
cxxxvii, 203 ; czXxviii, 48, 1937.
|Verhandl. d. phys.-med. Ges., li, 121, 1926.
Schmelzer. K. M. Aug., xci, 479, 1933.
Schmidt-Rimpler. A. f. O., xv (2), 193, 1869.
A. f. Aug., xviii, 152, 1888.
Schmidt and Wegner. A. f. O., xv (3), 253,
1869.
Deut. Z. f. Nervenhk., xli., 99, 1911.
Schneihdr. K. M. Aug., lxxiii, 210, 1924.
v. Sculeten. A. j. kl. Chir., xxxii, 453, 735,
1885.
Schwalbe. A. f. mik. Amat., vi, l, 1870.
de Schweinitz. T. O. S., xliii, 12, 1923.
Serr. A. f. O., czz.xv, 431, 1936.
Sesemann. A. f. Amat. Phys., 154, 1869.
Smith. A. of O., xxi, 356, 1939.
Smith, Priestley. O. Rev., vii, 193, 1888.
Sobanski. A. f. O., czzxvii, 84, 1937.
Sourdille. A. d’O., xxi, 378, 441, 1901.
Stock. K. M. Aug., xli (1), 81, 1903.
Swift. A. of O., iii, 47, 1930.
v. Szily. K. M. Aug., li (1), 196, 1913.
Tahano. Acta S. O. Jap., xxxix, 80, 1935.
Tanasescu and Lazarescu. K. M. Aug.,
lxxxvii, 108, 1931.
Terrien. A. d’O., lii, 465, 1935.
Teulieres. Bull. S. fr. d’O., xl, 506, 1927.
Türk. Z. d. Ges. d. Wien. Aerzte, ix, 1853.
Uhthoff. T. O. S., xxxiv, p. xlvii, 1914.
Ulrich. A. f. O., xxx (4), 235, 1884.
A. f. Aug., xvii, 30, 1887.
Wallis. Brit. J. O., i, 492, 1917.
Weekers. A. d’O., xxxix., 279, 1922; x1, 269,
652, 1923.
Wilbrand and Saenger. Neurol. d. Auges,
Wiesbaden, iv, 751, 1909.
Wilson and Drakes. J. A. m. Med. As., c,
1233, 1933.
Wolff and Davies. Brit. J. O., xv, 609, 1931.
T. O. S., li, 170, 1931.
Woodhall. A. of Surg., xxxiii, 297, 1936.
III. INFLAMMATION OF THE OPTIC NERVE
Inflammations of the optic nerve may be divided into two classes,
depending on whether the inflammatory process affects primarily the
sheaths and is really a meningitis (PERINEURITIS or PERIPHERAL INTERSTITIAL
OPTIC NEURITIs) or the
(Figs. 2548–50).
substance of the nerve
The former is a rare, the latter a common condition.
(OPTIC NEURITIs)
<d
A. Perineuritis
PERINEURITIS, an inflammatory condition primarily of the sheaths of
the optic nerve, is relatively rare, and although it may on occasions be an
isolated phenomenon, it is usually due to an extension of an inflam-
matory process from the meninges or from the orbital tissues, the most
common aetiological factors being the pyogenic bacteria, tuberculosis and
syphilis. Michel (1878) originally divided it into two main types as it
affected either the dura (PACHYMENINGITIS) or the pia-arachnoid (LEPTO-
MENINGITIs); but since the dura is rarely seriously involved owing to its
2968 TEXT-BOOK OF OPHTHALMOLOGY
dense tissues and lowly organization, the inflammatory processes are essen-
tially leptomeningeal. It is more useful, therefore, to consider two types
only—exudative and purulent perineuritis.
Fig. 2549.-Optic PERINEurrºris.
Fig. 2550.-OPTIC NEURITIs
(Edmunds and Lawford).
1. EXUDATIVE PERINEURITIs
As in all similar meningeal inflammations, the essential pathological
change in an exudative perineuritis is an infiltration, particularly of the
arachnoid, with polymorphonuclear and mononuclear leucocytes, the
former being most abundant in the acute stages (Michel, 1878; Elschnig,
1895) (Fig. 2551). The inflammatory process spreads along the sheaths
of the optic nerve in the same way and at the same time as on the surface
of the brain and spinal cord. In the dura, which is least affected, the inner


DISEASES OF THE OPTIC NERVE
2969
FIG. 2551.-OPTIC PERINEURITIs.
In acute epidemic cerebro-spinal meningitis.
Polymorphonuclear leucocytes
infiltrate the leptomeninges and abound in the sub-arachnoid space (Cone and
MacMillan).
layers show the most dense infiltration which is essentially perivascular
in distribution ; endothelial
form, may be prominent. The
arachnoid reacts most actively; it is
densely infiltrated and may occa-
sionally in acute cases be rarefied
or destroyed, but more usually its
loose trabecular network is trans-
formed into a highly cellular dense
membrane showing marked en-
dothelial proliferation and frequently
fusing with the dura on the one side
and the pia on the other. The latter
shows a similar leucocytic infiltration
which passes along the septa into
the nerve (Fig. 2552); these haema-
togenous elements, however, do
not usually infiltrate the nerve
parenchyma directly, a proof of the
efficacy of the glial barrier separating
the mesodermal elements from the
ectodermal.
proliferation, sometimes assuming a nodular
--------- -
º
º
º º
º º ---
º
º º
**º-º-º:
- º
tºº.º.º.
º- º
º
-
-º º
Fºº-ºº-ºº:
º
Fig. 2552.-Optic PERINEURITIs.
The same case as Fig. 2551, showing the
leucocytic infiltration from the pia along the
septal system deep into the nerve, but not
invading the nerve tissue (Cone and
MacMillan).












297 () TEXT-BOOK OF OPHTHALMIOLOGY
Frequently, especially in the more acute cases, there is a considerable
inflammatory exudate, the sheaths being separated by coagulable fluid rich
in albumen ; but, particularly in the chronic cases, proliferative changes are
most evident. The endothelium on the inner surface of the dura may be
markedly affected in this way, so that an enormously thick stratum of
laminae of endothelial cells visible to the naked eye, interspersed with whorled
groups resembling psammomata, binds this membrane to the arachnoid (the
PACHYMENINGITIS CHRONICA of Michel, 1878). The more normal termina-
tion, however, is a proliferation and organization of fibrous tissue, so that
the membranes and the spaces between them are extensively pervaded, the
latter being frequently partially obliterated. Such a process, as would be
expected, interferes so seriously with the circulation and nutrition of the
nerve that a secondary interstitial neuritis develops and, unless in the
milder cases, atrophy of some degree invariably follows.
2. PURULENT PERINEURITIS
In purulent inflammations, which are usually of streptococcal or
pneumococcal origin, the whole process is more acute, the cellular infiltration
more massive, and destructive changes tend to be more marked than
proliferative activities. Although the outer surface of the dura may be
relatively normal, apart from a perivascular infiltration, the inner surface
may be so eroded that its differentiation is difficult ; the arachnoid is
embedded in pus, and is frequently un-
recognizable ; while the dense infiltration
of the pia is usually associated with
haemorrhages and invariably with an intense
infiltration of the nerve-trunk, in which
appear areas of necrosis, small abscesses,
and even thrombosis of the central vessels
(Michel, 1881 : Axenfeld, 1897 : Lieto-
Vollaro, 1903). Such cases, of course,
being part of a suppurative cerebro-spinal
meningitis, are usually rapidly fatal.
The clinical symptoms presented by
Fig. 2553–Perseverts. acute suppurative perineuritis are entirely
ãº. * * * secondary to those of the general disease ;
but in the milder exudative cases, although
diagnosis is usually difficult, ocular symptoms frequently give some
indication of the events occurring in the nerve sheaths. Papilloedema
may be present, but as a rule the most important feature is the change
in the visual fields—a peripheral contraction with retention of central
vision—which indicates the inward spread of the inflammatory processes
in the pia along the connective tissue septa (Fig. 2553). Sometimes

DISEASES OF THE OPTIC NERVE 297 I
an irregular spread may produce sector-like defects in the field ; but
as a rule the peripheral defect spreads regularly and gradually, until
eventually only a small central area may remain in which the visual acuity
is low. A considerable degree of recovery may occur, or alternatively,
central vision may also disappear, and complete blindness associated with
Optic atrophy result, the atrophic appearance developing some time after
visual failure has been complete. -
Apart from general considerations, there is no distinction in the ocular
signs, perimetric or otherwise, between the different aetiological infections.
Most usually these are apparent, common causes being a spread of the basal
meningitis following head injuries (Edmunds and Lawford, 1883–87, and
others), or of infective processes within the skull, epidemic cerebro-spinal
meningitis (Terrien and Bondier, 1909–10), and occasionally the spread of
disease from the posterior ethmoid and sphenoid sinuses *; in the absence
of other obvious factors, syphilis should always be suspected. The treat-
ment is directed entirely to the primary condition ; and the prognosis,
which depends on the cause and the degree of atrophy present, is on the
whole unfavourable, although a relatively good response may be obtained in
syphilitic cases if treatment is instituted at a sufficiently early stage.
Axenfeld. Mom. f. Psy. Neur., ii, 413, 1897. Michel. Deut. A. f. kl. Med., xxii, 439, 1878.
Edmunds and Lawford. T. O. S., iii, 138, Sitz. d. Physik.-med. Ges., Würzburg, 1881.
1883; v., 184, 1885; vii, 208, 1887. Terrien and Bondier. A. d’O., xxix, 301,
Elschnig. A. f. O., xli (2), 179, 1895. 1909; xxx, 214, 1910.
Lieto-Vollaro. K. M. Aug., xli, Beil., 237,
1903.
B. Optic Neuritis
From the pathological point of view an inflammation of the optic nerve
may be purulent and acutely destructive in character, Or, assuming a less
dramatic type, it may be characterized by proliferative changes in the inter-
stitial tissues, which are followed by degenerative changes in the nerve-fibres.
1. PURULENT OPTIC NEURITIS
Purulent inflammations of the optic nerve with the formation of
abscesses in its substance are very rare, and are always incidental exten-
sions of other primary processes (Fig. 2554). Their aetiology is four-fold :-
1. An extension of a purulent perineuritis, the meningeal inflammation
invading the nerve along the pial extensions.”
2. An extension of a purulent endophthalmitis or panophthalmitis, the
inflammation entering the disc by direct extension * (Reese, 1927;
Wolff, 1931 ; and others).
3. From a purulent process in the orbit (Reis, 1904). It is interesting
1 p. 2985. * p. 2970. * p. 2164.
297.2 TEXT-BOOK OF OPHTHALMOLOGY
that in such cases of orbital phlegmon a patch of acute necrosis has
occasionally been noted in the nerve (Oeller, 1901; Cheney, 1923).
4. Metastatic abscesses are the most common cause, but these also are
rare. v. Michel (1877–1902) recorded 2 cases, one of endocarditis, and
the other with pyaemia and metastatic irido-cyclitis. Axenfeld (1894)
reported multiple abscesses in the nerves of two children with meningitis, one
pneumococcal and the other streptococcal. A somewhat similar syndrome
was described by Nakaizumi (1910) in a case of meningitis which had also a
Fig. 2554–PURuLENT OPTio Nºuritis.
A secondary papillitis, in panophthalmitis following a penetrating injury with
steel. The nerve-head is infiltrated with polymorphonuclear leucocytes and the
vessels engorged (x 50) (Wolff).
purulent choroiditis. A staphylococcal case was described by Spicer (1907)
where a metastatic abscess involved the temporal part of the dise and the
retina, the origin of the infection being a furuncle of the skin (Fig. 2231);
and a streptococcal infection with multiple abscesses in a case of pyaemia
following a wound of the finger was reported by Kyrieleis (1930).
In such cases the severity of the causal illness or the resultant pyaemia
usually proves fatal; or alternatively the eye has had to be excised owing
to pain and the intra-ocular spread of the suppurative process.
Axenfeld. A. f. O., x1 (3), 1, 1894. Oeller. Fest. d. Prinzregenten Luitpold,
Cheney. A. of O., lii, 252, 1923. Erlangen, iii, 117, 1901.
Kyrieleis. K. M. Aug., lxxxv, 194, 1930. Reese. A. of O., lvi, 265, 1927.
v. Michel. A. f. O., xxiii (2), 213, 1877. Reis. A. f. O., lix, 155, 1904.
z-f. Aug., vii. 1, 1902. Spicer. T. O. S., xxvii, 230, 1907.
Nakaizumi. K. M. Aug., xlviii (2), 17, 1910. Wolff, P.R.S. Med., xxiv, 1617, 1931.

DISEASES OF THE OPTIC NERVE 2973
2. INTERSTITIAL OPTIC NEURITIS
An interstitial inflammatory process may attack the optic nerve in any
part of its course; but since the process is clinically visible only if the optic
nerve-head is affected, and since the presence of inflammation in the trunk
of the nerve can only be inferred indirectly from other symptoms, it is
customary to divide such cases into two classes: PAPILLITIs (or intra-ocular
neuritis) and RETRo-BULBAR NEURITIS. In view of the fact that the general
pathology is the same whatever the localization, this will be considered first as
a whole. It will be remembered that many retinal inflammations involve
Fig. 2555.-PAPILLITIs.
General infiltration of the tissues with leucocytes and lymphocytes (Cone and
MacMillan).
also the optic nerve, while conversely, inflammations of the nerve-head
may spread to the retina ; these may be termed NEURO-RETINITIs. It is
to be remembered also that although retro-bulbar inflammations usually
affect the sub-chiasmal nerve-trunk, the chiasma itself and the optic tracts
are not immune from similar lesions, which can be differentiated clinically
only by the type of defect in the visual fields.
General Pathology
The histo-pathology of inflammations of the optic nerve follows closely
on the lines already discussed in the section on the healing of wounds–
proliferative changes in the interstitial tissues followed by degenerative
changes in the neural tissues. Both are invariably present, in one type one
predominates, in another the other, and both are followed by a reparative

2974 TEXT-BOOK OF OPHTHALMOLOGY
gliosis. Among the most instructive papers in the literature are those of
Edmunds and Lawford (1887), Elschnig (1892), Uhthoff (1893), Dalén (1899),
Bielschowsky (1901), Schieck (1909), Clowes and Taylor (1912), Rönne (1912),
Wilbrand and Saenger (1913), Ulrich (1913), Abelsdorff (1918), and
Igersheimer (1918–19) (Figs. 2255–56).
The inflammatory changes occur primarily around the blood-vessels,
and are ushered in by oadema of all the tissues and a dense infiltration of the
septa with haematogenous cells–leucocytes, lymphocytes and plasma cells.
Such changes affecting the distal end of the nerve are evident clinically as a
papillitis. In this way the connective tissue septa of the nerve become
Fig. 2556.-Acute Axial RETRo-BULBAR NEURITIs
(Behr, A. f. O.)
thickened as new vessels and proliferating granulation tissue are formed ;
these with their bulk, in addition to the oedematous changes, compress the
nerve fibres, a process which becomes intensified as fibrosis and shrinkage
develop. Sometimes the whole thickness of the nerve is implicated, but
the lesion has a habit of being insular in distribution so that some bundles
of fibres become more involved than others. As they become increasingly
compressed and strangled, even if they are not themselves attacked by the
toxin, the medullary sheaths break up into fatty droplets, a process which
is seen at an early stage when the noxa is of a demyelinating type. This is
accompanied by degeneration of the nerve fibres both centrally and in a
retrograde direction. At this stage the leucocytes tend to be replaced by
microglia, which, acting as phagocytes, appear as enormously dilated

DISEASES OF THE OPTIC NERVE 2975
bladder cells loaded with fat and crowd the inflamed area (Fig. 2514). The
final stage is reached when, with the disappearance of the débris, the glial
astrocytes proliferate, and, assuming large cell-bodies and voluminous
cytoplasmic processes, replace the degenerated neural tissue and further
intensify the process of cicatricial contraction. In acute conditions this
replacement occurs in a dense tangle ; but when the degeneration has
been slow, the replacement is correspondingly orderly so that the fibrillary
projections of the cells tend to be parallel taking the place of the
degenerated fibres (columnar gliosis), a process which may occur both in
the nerve-trunk and on the nerve-head (Spielmeyer, 1906) (Figs. 2519–20).
Clinical Picture
Clinically optic neuritis manifests itself essentially by loss of vision—
typically a loss of central vision—irrespective of the ophthalmoscopic
appearances of the optic disc, which may be normal (in retro-bulbar neuritis),
Oedematous (the neural oedema caused by an inflammatory process in the
nerve near the disc), or inflamed (in papillitis). This cardinal symptom of
loss of vision may be profound and precedes any objective appearances of a
pathological nature. It may be preceded by some headache and nausea and
is usually associated with three subsidiary symptoms: (1) some pain in
and behind the eye, especially on movement, together with tenderness of the
globe on pressure, (2) a peculiar papillary reaction common to all forms of
conduction interference wherein, although both the direct and consensual
reactions are present, the contraction is not maintained under bright
illumination so that the pupil slowly dilates again while the light is still
kept upon the eye, and (3) a lowering of dark adaptation. According to
Percival (1926), the light difference is impaired but not the light minimum ;
but Wilmer (1930) found both affected.
These symptoms vary to a considerable extent depending on the site
or the severity of the lesion. Thus, while the loss of vision is usually central,
owing to the greater liability of the relatively delicate papillo-macular
bundle to succumb to noxious agents, the scotoma may be relative for
colours or absolute, or it may be paracentral, caeco-central, sectorial or
annular ; some peripheral contraction is the rule ; or blindness may be
complete. The symptoms as a general rule come on suddenly ; in the
majority of cases they are transient, lasting some 1 to 4 weeks; and recovery
of vision may be very complete. This tendency to recovery is indeed
one of the characteristics of optic neuritis, for, while the conducting
powers of the nerve-fibres are readily diminished, the axis cylinders are very
resistant, so that when the disease resolves, conduction rapidly becomes
re-established. In every case, however, wherein the inflammation of the
nerve has been sufficiently severe to cause destruction of the nerve-fibres,
some degree of degeneration follows, which in the milder cases involves a
pallor of the disc limited to its temporal aspect corresponding to the papillo-
T.O. —WOL. III. 3 L
2976 TEXT-BOOK OF OPHTHALMOLOGY
macular bundle, but in the more severe cases results in the appearance of a
complete secondary (or consecutive) atrophy with correspondingly perma-
ment visual defects. From the patient's point of view the residual vision
may be quite satisfactory, but careful scotometry usually shows a central
relative scotoma wherein colour perception is impaired, or small scotomatous
areas wherein many nerve fibres have died.
We shall now proceed to examine the various clinical types which may
be encountered.
(a) PAPILLITIs
When the inflammation affects the nerve-head the clinical picture of
papillitis is produced (Figs. 2558–59, Plate LXI). The disc becomes
Fig. 2557 –PAPILLITIs.
Acute optic neuritis showing oedematous star at macula (Brain, T. O. S.).
hyperaemic so that its differentiation from the rest of the retina becomes
difficult, its edge becomes blurred and indistinct, the veins become dilated,
although the arteries are less affected, and haemorrhages and exudates may
appear in the tissue of the papilla, although not usually in great quantity.
The haziness of the picture is frequently accentuated by the development of
a cloud of opacities in the posterior vitreous; and almost invariably some

PLATE LXI
Optic NEURIt is
Fig. 2558-Toxic PAPi Litis. Fig. 2559.-Syphulitic PAPILLITIs.
-
Fig. 2561.-RETRo-Bulbar NEURITIs. Fig. 2562–Post-Neurºtic ATRoPHY
(INTRA-cer EBRAL SYPHILIs).
[To face p. 2976.


DISEASES OF THE OPTIC NERVE 2977
swelling of the nerve-head is evident. This is usually relatively small—less
than 2 dioptres—but occasionally, especially if the lesion is situated just
behind the lamina, the Oedema, in this case a neural Oedema of an inflam-
matory nature, may be extreme producing an elevation of 6 or more
dioptres (Paton, 1914); these cases are, however, rare, and as will be
discussed presently, present difficulties in diagnosis from plerocephalic
Oedema. Occasionally, also, the Oedema and exudate spread from the disc
upon the retina producing a neuro-retinitis; in this event the region of
the macula is particularly susceptible, where a star-figure may develop
(Fig. 2557). At the same time, it is to be remembered that the maximally
developed clinical picture of a greatly swollen disc with exudates and
haemorrhages, an Oedematous macula, and a hazy vitreous, is exceptional ;
as a rule the ophthalmoscopical appearances are less than the dramatic
failure of visual acuity would seem to warrant.
As we have seen the symptoms usually come on suddenly, the loss of
sight preceding the ophthalmoscopic changes. As a rule these changes
are of short duration, the recovery of vision being synchronous with their
subsidence. Both may be remarkably complete, and after the most marked
swelling vision may gradually improve to normal or nearly so, while no
trace of pathological change may be evident after the lapse of some weeks.
Most commonly, however, some degree of POST-NEURITIC ATROPHY
(Fig. 2562, Plate LXI) is evident, distinguished from the other types of optic
atrophy by its indefinite greyish appearance due to the presence of a variable
amount of fibrous tissue upon the disc ; this obscures the lamina cribrosa,
fills up the physiological cup, extends over the disc margin, making it
indefinite, and thickens the vessel walls, appearing as white perivascular
sheaths, throttling and constricting the vessels, particularly the arteries,
as it does so. This fibrosis represents a reactionary organization in the
inflammatory exudation, and provides a permanent picture from which
a diagnosis of a previous papillitis can be made with confidence.
The differential diagnosis of papillitis is important and involves three conditions.
(1) Marked refractive errors, particularly of astigmatism, which may produce the
appearance of blurring of the disc margin. If, however, the margin can be seen
clearly by varying the lens in the ophthalmoscope, neuritis does not exist.
(2) Pseudo-mewritis (Fig. 2560, Plate LXI), a congenital condition which has
already been discussed.” In it the nerve fibres are heaped upon the disc to form an
elevation which is further accentuated by an excess of glial tissue thereon ; but
there is never an enlarged blind-spot. There is an absence of venous engorgement,
haemorrhages or exudates, and—most important—the condition is non-progressive,
a fact which requires observation of the case over a considerable period for its
establishment, controlled preferably by fundus photography.
(3) Papilloedema frequently presents a difficult diagnostic problem. In typical
cases the ophthalmoscopic appearances and the symptoms are quite different, but in
early cases doubts frequently arise, and in those cases of papillitis complicated by
1 Vol. II, p. 1342.
2978 TEXT-BOOK OF OPHTHALMOLOGY
massive oedema the difficulties may be extreme. The important differential points
8.Te :
(i) The degree of swelling in papillitis is rarely above 2 dioptres : in papilloedema
it is frequently higher.
(ii) Wenous engorgement and hamorrhages are usually less marked in papillitis.
(iii) The functional symptoms are usually much more marked in papillitis. The
loss of sight is sudden, intensive and profound, and precedes ophthalmoscopic
changes. In papilloedema, the functional loss is less than objective appear-
ances seem to warrant, and apart from temporary obscurations in the
initial stages, is late in being appreciated and gradual in onset.
(iv) The scotoma is typically central and pronounced for colours in papillitis.
A concentric regular and gradual contraction with co-ordination of colour
vision is more typical of papilloedema.
(v) The swelling in papillitis is usually temporary and recovery is common in
2 or 3 weeks : in papilloedema the changes tend to be progressive until relief
is obtained.
(vi) Recovery of vision may be complete in papillitis even after the development
of complete blindness. In papilloedema visual deterioration progresses until
the condition is relieved.
It is interesting that Selinger (1934) found an increased protein content (as judged
by the trichloracetic acid test) in the aqueous removed by paracentesis in papillitis but
not in papilloedema.
(b) RETRO-BULBAR NEURITIS
1. ACUTE RETRO-BULBAR NEURITIS. If the inflammation occurs in the
trunk of the optic nerve the same dramatic and profound functional symptoms
occur with, however, no ophthalmoscopic changes. It is true that if the
lesion is near the nerve-head the resulting oedema may be apparent on the
disc, producing on occasion a swelling of several dioptres (Paton, 1914;
Klar, 1932), and it may be associated with some slight dilatation of the
retinal veins; but in the majority of cases the fundus appears normal, and
the condition may truly be defined as a disease wherein neither the surgeon
nor the patient sees anything.
In the most severe case when the whole nerve is involved, complete
blindness may develop with great rapidity associated with a dilated or fixed
pupil (TOTAL TRANSVERSE NEURITIs); but in the majority of cases the
inflammation takes the form of a localized plaque so that the block in the
nerve is partial and affects particularly the macular fibres (AXIAL RETRO-
BULBAR NEURITIS). The lesion develops rapidly and then resolves slowly,
leaving the axis cylinders for the most part capable of conduction. The
characteristic clinical feature is therefore the sudden onset of visual failure
in one eye followed by gradual recovery. Associated with the visual failure
are a sluggishness of the pupil with a failure to maintain contraction on
prolonged stimulation, and pain on Ocular movements with tenderness on
pressure on the globe. The patient complains of a fogginess of vision
coming on suddenly as if a cloud were hanging over the centre of the field of
DISEASES OF THE OPTIC NERVE 2979
vision, and the visual acuity is usually better in the dusk than in bright light.
The symptoms usually persist from 2 to 4 weeks, the rate of recovery varying
considerably, sometimes being gradual at first and then clearing up more
rapidly, but occasionally a considerable defect may persist for some months.
As a general rule, however, the prognosis is good, although some temporal
pallor of the disc and a slight persistent depression of central vision are
COIOIſlOI).
In the typical case the scotoma is central in position, although not
necessarily peri-central (Fig. 2563); the fixation point may well be near its
margin, and a centro-caecal type is common. It may be small in extent,
occupying an approximately circular area in the field some 10 to 15 degrees
in diameter ; occasionally it may be so large that only a comparatively
narrow zone of normal field remains near
the periphery. Its intensity also varies ;
it may be relative for colours only, but
much more usually is absolute and com-
plete. As a rare event sector defects
occur, or an arcuate disposition may
indicate the special involvement of par-
ticular bundles of nerve fibres, while not
uncommonly if the scotoma is large it
may break through at one point and
reach the periphery (Fig. 2563). Fre-
quently also the peripheral field shows
some contraction. These changes rapidly
reach their maximum, and as recovery Fig. 2568. Acure Axial Renao.
occurs the defects shrink, the appreciation BULBAR NEURITIS.
of colours lagging after that of white. A typical field showing a central
Scotoma with absolute central nucleus
and contracted periphery showing
breaking through. 10, 5, 1/330
white.
2. CHRONIC RETRO-BULBAR NEURITIS
The chronic form of the disease is
comparatively rare, and is more typical of the toxic amblyopias than of
inflammatory lesions; moreover, it is more frequently bilateral. It is of
slow onset, may persist for many months, and as a rule has a more serious
prognosis in that marked visual deterioration is more commonly permanent.
The field defects, however, are substantially the same.
3. CHIASMAL NEURITIS
Although originally described by Gowers and Marcus Gunn (1904) and
studied in detail by Rönne (1912–28) and Traquair (1917–25), neuritic
affections in the chiasma or tracts are rarely recognized. The condition
runs the same course as when the optic nerve itself is affected ; the onset
is sudden, sometimes accompanied by headache and sickness and occa-

2.980 TEXT-BOOK OF OPHTHALMOLOGY
sionally by visual hallucinations. The visual defect is necessarily bilateral,
although it is frequently more marked in one eye than the other ; and as
it is often caused by disseminated foci which arise and subside in the sub-
geniculate pathway, the defects in the fields may be irregular and appear to
wander from one part of the field to another, one area recovering vision
while another loses its function. Moreover, the chiasmal disease is frequently
complicated by a retro-bulbar lesion, which, indeed, may extend to the disc
so that a papillitis may be ophthalmoscopically apparent. In this event a
stage may be reached when one disc shows atrophy and the other a papillitis
(Brain, 1934).
In a chiasmal lesion the typical field shows a bitemporal type of defect
(Fig. 2564), but other variations may occur. A small central lesion
_º wº-iss
22 ––SX\,.
*-
7/5: . e .9 N
yº. ///º
%Yº ſ/º Nº.
% hº º If §3. N
O % ŻZA Y-r/ o, O NATI-ÉS WN
/ºs ASRs.1% O NWSNNNNN 3
* ', % // /. 2 / . § 3, - \\\\ \\ XN y §§ o
*% º &º
º
=º
FIG. 2564.—ACUTE CHIASMAL NEURITIS.
5, 1/330 white.
may produce a limited altitudinal defect, while a localized patch at the
junction of one nerve with the chiasma may give rise to a hemianopic
scotoma in the ipsolateral eye while the field of the other eye is normal : the
junction Scotoma of Traquair (Fig. 2765). It may be also that a lesion in
one nerve at this point may produce a defect in the upper temporal quadrant
of the other eye owing to an involvement of the “knee '' of crossed fibres of
the other nerve. -
Differential Diagnosis. The differential diagnosis between the different
forms of optic neuritis, whether of the inflammatory or toxic group, will be
considered presently : the remaining conditions wherein visual failure due
to a central scotoma may arise are (1) pressure upon the nerve sufficient to
cause interference with conduction, as by a neoplasm or a chiasmal arach-
noiditis, and (2) a vascular lesion. With regard to the first group of cases,





















DISEASES OF THE OPTIC NERVE 298]
a slow onset and a gradual increase in severity of the symptoms with no
tendency to recover form a sharp contrast to the usual inflammatory con-
dition with its rapid onset, fluctuating nature, and strong tendency to
recovery : a careful study of the fields and evidences of a source of pressure
are usually sufficient to suggest the diagnosis. A vascular origin of a central
Scotoma should be considered in elderly persons in whom arterial disease is
present : in these cases the defect appears suddenly and is unilateral, but is
either stationary or progressive, while sector defects
are more common than a central scotoma.
The prognosis in general in optic neuritis is
good as regards recovery of sight, although, as we
shall see presently, this is not invariably the case.
As a general rule the prognosis in papillitis is worse
than in retro-bulbar neuritis, perhaps because the
danger of acute strangulation is greater. Uni-
lateral cases have a better prognosis than bilateral,
although in the former case the danger of sub-
sequent complications in the central nervous 2 * * * *
& g & |FIG. 2565.-C H I A S M A L
system should always be kept in mind. Chronic NEURITIS : JUNCTION
conditions and slowly progressive symptoms SCOTOMA. 20, 2/1,000.
usually involve greater visual defects than acute
cases, and the prognosis is worse the longer the onset of improvement is
delayed.
Treatment
The essential treatment of optic neuritis should be directed against the
cause of the condition ; but, particularly if the disease is acute, certain
general measures are of value. In such acute cases rest in bed is advisable,
and every endeavour should be made to reduce the local Oedema before
damage to the nerve fibres by strangulation occurs. Provided the aetio-
logical factor does not contra-indicate it, this end is best attained by the
administration of salicylates, a free opening of the bowels, the induction of
sweating by hot-air baths, pilocarpine injections, and so on. Sometimes
protein shock, as by intravenous injection of typhoid vaccine, appears to
give remarkably good results and seems to abort the attack (Benedict, 1933;
and others). Similar good results have been claimed by inducing a general
vaso-dilatation, as by the intravenous injection of 10% sodium nitrite
(Aubaret and Sedan 1928; Duggan, 1936) or the sub-cutaneous or intra-
muscular injection of atropine or acetyl-choline (Saint-Martin, 1931;
Duggan, 1937).
Operative measures have frequently been advocated, especially if the
acute condition shows no signs of resolving after a few days. To a large
extent these have concerned themselves with opening up the sphenoid and

2982 TEXT-BOOK OF OPHTHALMOLOGY
posterior ethmoid sinuses, a drastic step originally undertaken on the theory
that infection from these sinuses was aetiologically responsible for a large
number of cases of this condition. We shall see presently 4 that while nasal
disease can undoubtedly cause an optic neuritis, such an aetiology is probably
rare ; indeed, it is possible that the good, and in many cases the dramatic
results which have been commonly reported following an intra-nasal opera-
tion are due, not to an elimination of the causal infection, but to the local
relief of tissue-tension, the subsequent hyperaemia, and perhaps the effect of
the absorption of blood which acts as a foreign protein. These questions
will be discussed subsequently when dealing with rhinogenous neuritis & ;
but it is significant that equally good results have been claimed from produc-
ing a nasal anaemia by the local application of adrenaline or a hyperaemia by
plugging the nose with iodine.
An alternative operative measure is a direct decompression of the optic
canal through a sub-periosteal approach along the upper and inner wall of
the orbit (Worms, 1930): the region of the posterior ethmoidal cells is
reached in this way, and, these being opened, the inner wall of the optic canal
is resected, a longitudinal incision being made in the nerve-sheath if
necessary.
An assessment of the value of these operative procedures is difficult
largely because of the marked tendency of the disease to recover rapidly and
spontaneously ; indeed, in the majority of acute cases improvement occurs
while the patient is still undergoing the usual investigations to establish
the diagnosis. It is possible, however, that in the absence of a well-defined
aetiology which can be tackled, and with the persistence of complete amaurosis
over several days, a decompression, either through a nasal or orbital approach
may be justifiable especially if radiological examination shows that the optic
canal is narrow—for example, smaller than 5.35 mm. in diameter (de Kleijn
and Stenvers, 1917; White, 1928). It can be argued that in such cases
strangulation of the nerve at the canal is a definite possibility which should
be obviated; but it must be admitted that the cases wherein the question
seriously arises are few.
In the more chronic types of the disease, apart from the elimination of
the causal agent, main reliance should be placed upon improving the general
health and resistance, and the institution of stimulatory and eliminative
measures. These measures will be discussed more fully when dealing with
the question of tobacco amblyopia,” but two methods of therapy are deserv-
ing of special attention because of their occasional good results—the induction
of vaso-dilatation by the repeated injection of such drugs as amyl nitrite
(Pfimlin, 1930; Duggan, 1932–35; Shannon and McAndrews, 1934; Cordes
and Harrington, 1935; and others), or acetyl choline (Bonnefon, 1931;
Orr, 1936; Duggan, 1937), and the administration of abundance of vitamin
B (Shastid, 1929; Carrol, 1935–37; Paternostro, 1938).
1 p. 2985. * p. 2985. 8 p. 3017.
DISEASES OF THE OPTIC NERVE
2983
Abelsdorff. Z. f. kl. Med., lxxxv, 435, 1918.
Aubaret and Sedan. La Clin. Opht., xvii, 255,
1928.
Benedict. Proc.
Clinic, 1933.
A. of O., ix, 893, 1933.
Bielschowsky. Myelitis w. Sehnerventzündung,
Berlin, 1901.
Bonnefon. Pract. med, franc., xii, 65, 1931.
Brain. T. O. S., liv, 221, 1934.
Carroll. A. of O., xiv, 112, 421, 1935; xvi,
919, 1936; xviii, 948, 1937.
Clowes and Taylor. Ophthalmology, viii, 584,
1912
Staff Meetings, Mayo
Cordes and Harrington.
1935.
Dalén. A. f. O., xlviii, 672, 1899.
Duggan. A. of O., viii, 304, 1932; xiii, 1059,
1935; xvi, 380, 1936; xvii, 579, 1937.
Edmunds and Lawford. T. O. S., vii, 208,
1887.
Elschnig. A. f. Aug., xxvi, 56, 1892.
A. of O., xiii, 435,
Orr. Brit. Med. J., ii, 69, 1936.
Paternostro. An. dº Ott., lxvi, 891, 1938.
Paton. T. O. S., xxxiv, 252, 1914; xxxviii,
170, 1918.
Percival. T. O. S., xcvi, 392, 1926.
Pfimlin. K. M. Aug., lxxxv, 787, 1930.
Rönne. A. f. O., lxxxiii, 505, 1912.
R. M. Aug., l (2), 446, 1912; ly, 68, 1915.
Acta O., vi, 332, 1928.
Saint-Martin. An. d’Oc., clxviii, 102, 1931.
Schieck. A. f. O., lxxi, 466, 1909.
Selinger. Am. J. O., xvii, 1130, 1934.
Shannon and McAndrews. A. of O., xi, 757,
1934.
Shastid. Am. J. O., xii, 903, 1929.
Spielmeyer. K. M. Aug., xliv (1), 97, 1906.
Traguair. Brit. J. O., i, 216, 281, 357, 1917;
ix, 433, 1925.
T. O. S., xliii, 480, 1923; 1, 351, 1930.
Uhthoff. A. f. O., xxxix (1), 1 ; (3), 126,
1893.
Ulrich. Z. f. Aug., xxix, 195, 1913.
Gowers and Gunn. Medical Ophthalmology, White. An. Oto. Rhin. Lary.., xxxvii, 128,
London, 1904. 1928.
Igersheimer. A. f. O., xcvi, 1, 1918; xcviii, Wilbrand and Saenger. Neurologie d. Auges,
67, 1919; ci, 79, 1920. v, 1913.
Johnson. A. of O., xxi, 602, 1939. Wilmer. A. of O., iv, 817, 1930.
Klar. K. M. Aug., lxxxix, 645, 1932. Worms. Bull. S. fr. d’O., xliii, 417, 1930.
de Kleijn and Stenvers. A. f. O., xciii, 216,
1917.
AEtiology
To establish the aetiology of many cases of optic neuritis is frequently
difficult and may be impossible, largely because the affection of the nerve
is so often the symptomatic evidence of a generalized disease which may
be obscure or may not manifest itself until after the lapse of years. In
these circumstances it is not strange that the literature is full of contradic-
tions, and we find in one publication that practically every case is due to
disseminated sclerosis (Adie, 1930), in another that nasal disease is the
preponderant factor (van der Hoeve, 1922–25), or again that focal sepsis
accounts for the majority of cases (White, 1928; Wilmer, 1930; and others).
One very misleading factor, which we have already noted, is the tendency in
the majority of cases to spontaneous recovery, a circumstance which has
frequently been interpreted as having an aetiological significance : if a
retro-bulbar neuritis improves after the removal of a tooth or the opening
of a sinus, the association loses its significance when it is found that the next
case behaves similarly if nothing is done. There seems little doubt that the
great majority of cases, particularly of the usual type of acute unilateral
retro-bulbar neuritis is due to a virus infection of the central nervous
system, particularly that of disseminated sclerosis ; and it seems probable
that many cases the aetiology of which is unknown and in which no further
general symptoms develop, are the expression of an unknown virus or viruses
with neurotrophic tendencies but of mono-symptomatic incidence.
2984 TEXT-BOOK OF OPHTHALMOLOGY
The aetiology of 225 cases of retro-bulbar neuritis studied by Benedict (1933) at
the Mayo Clinic was determined as follows:— -
Multiple sclerosis e te º e * e e . 155
Pernicious anaemia . * * º * ſº * & 14
Diabetes . * º º * * , & * : º . 14
Alcohol and tobacco . g g & * tº e tº 28
Syphilis . e º e º sº tº $º º * > 2
Congenital amblyopia
Familial causes
Sinus disease ſº º
Post-partum haemorrhage .
Plumbism te
Indeterminate causes
i
As a provisional classification we propose to divide the aetiological
factors of optic neuritis into the following categories, which we shall proceed
to consider in detail.
I. Local inflammatory conditions.
1. Uveitis and retinitis.
2. Sympathetic ophthalmitis.
3. Meningitis.
4. Nasal sinus disease and Orbital infections.
II. General inflammatory conditions.
1. Central nervous (? virus) infections.
(a) Disseminated sclerosis.
(b) Acute disseminated encephalo-myelitis.
(c) Neuro-myelitis optica (of Devic).
(d) Encephalitis periaxialis diffusa (of Schilder).
(e) Herpes zoster.
(f) As a rarity in encephalitis lethargica, poliomyelitis, rabies
inoculation, etc.
2. Syphilis.
3. Tuberculosis.
III. Leber’s disease (of unknown aetiology).
IV. Endogenous toxins.
1. Acute infective diseases:
Influenza, malaria, measles, mumps, pneumonia, etc.
2. Septic foci: -
Teeth, tonsils, sinuses, etc.
3. Metabolic dyscrasias:
Diabetes, anaemia, pregnancy, lactation, avitaminosis.
V. Intoxications by exogenous toxins:
Tobacco, ethyl alcohol, methyl alcohol, lead, arsenic, thallium,
apiol, quinine, ergot, optochin, filix mas, etc.
DISEASES OF THE OPTIC NERVE 2985
I. Optic Neuritis secondary to Local Infections
INTRA-OCULAR INFECTIONS
We have already seen when discussing the subjects of uveitis and
retinitis that it is no uncommon thing for an intra-ocular infection to affect
the optic nerve, producing in the first place a papillitis. Not only is this
process due to direct spread from a juxta-papillary choroiditis, but it is seen
very typically in inflammatory conditions of the anterior uvea, when the
direct diffusion of toxic material from a severe irido-cyclitis through the
vitreous to the posterior pole of the eye, or a periphlebitic extension along
the retinal veins excites an inflammatory response in the optic nerve.
Sometimes this is seen as a definite papillitis; at other times a retro-bulbar
affection must be inferred from the development in the course of an irido-
cyclitis of a relative or absolute central scotoma (Meller, 1921 ; Kleinasser,
1922; Clegg, 1922–24; Zeeman, 1923; Fry, 1938). It will be remembered
that such a papillitis was induced experimentally (Straub, 1912) by the
inoculation of the ciliary body with tubercle bacilli (Fig. 1835).
No condition is of more significance than sympathetic ophthalmitis,
in certain cases of which a sympathetic papillo-retinitis may be a
prominent symptom, and indeed may be the first to become apparent in the
sympathizing eye (Gilbert, 1910; Law, 1929). In this connection it will be
remembered that a definite school of thought exists which attributes this
disease to the spread of an infection, presumably virus in nature, from one
eye to the other along the optic nerves and chiasma (Leber, 1881–1904;
Deutschmann, 1882–1927; Fuchs, 1925).
INTRA-CRANIAL INFECTIONS. Intra-cranial infections are a rare
cause of optic neuritis; more commonly their effect becomes evident as a
peri-neuritis.” The most common causes are an abscess of the middle
ear, infective processes following a fracture of the skull, and epidemic
cerebro-spinal meningitis. The question of syphilis and tuberculosis will
be considered at a later stage.
INTRA-ORBITAL INFECTIONs may spread directly to the optic nerve
causing an inflammatory infiltration (Horner, 1863), or areas of necrosis
(Oeller, 1901; Bartels, 1907), or even abscess formation. Those cases,
however, which have as origin the orbital periosteum or tissues, or which
spread from the face, as erysipelas (Hutchinson, 1871), are rare ; more
frequently the infection is rhinogenous.
THE NASAT, SINUSES
A very large literature abounds with confusion and contradictions on
the vexed question of the existence or the prevalence of a rhinogenous optic
neuritis. In the early part of this century, largely stimulated by the con-
vincing anatomical studies of Onodi (1904–08) and Loeb (1909) in America,
1 p. 2967.
2986 - TEXT-BOOK OF OPHTHALMOLOGY
a great body of opinion contended that the majority of cases of optic and
retro-bulbar neuritis were secondary to infections of the sinuses. These
workers showed that while the optic nerve was usually closely related to the
sphenoid and posterior ethmoid cells, an almost infinite variety of relations
might exist from their separation by a dense bony wall to one of paper-
thinness; occasionally the nerve may traverse the sinuses unprotected by
any bony shield whatever, while in all cases there exists an intimate inter-
connection with blood-vessels and lymphatics (Fig. 2566). With this as
a starting point, and citing as confirmation the fact that a large number of
such cases cleared up dramatically after opening the sinuses operatively, a
number of writers placed sinusitis in the forefront of the aetiology (v. d.
Fig. 2566–RETRo-Bulbaº NEURiºtis rºom SINU's Diseasº.
Retro-bulbar neuritis from an empyema of right sphenoidal sinus, from which the
optic nerve, about which puscells had collected, is separated only by a verythin plate
of bone (MacDonald).
Hoeve, 1922–25), others ascribed to it a considerable proportion of cases
(14% Herzog, 1920–28; 4.5%, Meller and Hirsch, 1926; 11%, Wilmer,
1930; 4 in 13, Gifford, 1931; 26 in 120, Poos, 1930), while, on the other
hand, others insisted that any influence nasal inflammation might have
was rare and incidental (1 in 280 cases, Beck, 1923–24; 1 in 35, Weill,
1923–29: 1-5%, Scheerer, 1929; v. Hippel, 1932; 1 in 225, Benedict,
1933; 1 in 225, Lillie, 1934), or was non-existent (Cushing, 1920).
Several suggestions have been put forward as to the rationale of infection –
(a) A direct spread of the infective process from the sinuses to the nerve, a process
occurring more rapidly when the anatomical relationship between the two was intimate,
but capable of occurring by erosion of the bone. That such an extension does occur
has been proved by anatomical demonstration on several occasions (v. d. Hoeve
1922–25; Pickworth, 1928; ten Doesschate, 1928; Redslob, 1930; Worms, 1936)
(Fig. 2566).
(b) Pressure from a distended sphenoid sinus may affect the nerve, exciting
infiltration and oedema sufficient to strangulate it at the optic foramen (Crane, 1927;
Letchworth, 1932).

DISEASES OF THE OPTIC NERVE 2987
(c) A transference of infection indirectly through venous and lymphatic channels
to the orbital periosteum and thence to the dural sheath of the nerve (Gradle, 1915;
Gradle and Meyer, 1929).
(d) Thrombosis of the emissary veins between the sinuses and the orbit (ten
Doesschate, 1928).
(e) A haematogenous transference of infection, the infected sinus acting as any
other focus of infection (White, 1928).
(f) A localized allergic reaction involving the tissues of both sinus and
orbit (Stark, 1921).
An evaluation of the part played by sinus disease in the causation of
optic neuritis is difficult. There are no clinical criteria differentiating a
nasal aetiology from any other, for the neuritis is a typical retro-bulbar
lesion. Van der Hoeve (1910) suggested that an early symptom of import-
ance was a symmetrical enlargement of
the blind-spot with the retention for a s_*- :
time of central vision, but this is by no ſº ºf Sº *
means diagnostic (de Kleijn, 1910–12 ; ~
Gjessing, 1912). The scotoma is usually
either central or centro-caecal, and is Ö. º,
typically progressive with fluctuations, thus s º: wº
behaving differently from the ordinary Vº 3
scotoma of acute retro-bulbar neuritis Q §§2. & §
(Traguair, 1924) (Fig. 2567): annular (v. § SST
d. Hoeve, 1922; Elles, 1938) or bilateral
crescentic scotomata (de Schweinitz, 1915)
are rare. Such cases as that reported by
Lemoine (1938) of a tract lesion which FIG. 2567.-RETRO-BULBAR NEU-
resolved after evacuating an infected arris row sists Disease.
sphenoid cell which ran into the posterior ..º.º. Scotoma and
clinoid process are exceptional, although
heteronymous hemianopic chiasmal effects, due presumably to pressure,
have been reported (Grönholm, 1910).
In any discussion two points must be borne in mind. First, that in simus
disease generally, optic nerve symptoms are a rarity. It is true that some
authors claim that in a considerable proportion of sinus cases, minute exami-
nation reveals defects in the visual fields (Herzog, 1920–28 ; Bordley, 1921 ;
v. d. Hoeve, 1922–25); but in general terms it is the universal experience
that visual symptoms are not a common complication of sinus infections.
Nor have experimental attempts on animals shown that even a widespread
sinus infection can reach the optic nerve (Pichler, 1928–29 ; Wolfkovitch,
1933). Second, it is rare for cases of optic neuritis to be associated with
serious simus disease. Against this it is argued that the usual cause is a
chronic hyperplastic catarrh with a quiet or latent sinusitis, such as gives
rise to few clinical and no radiological signs (Herzog, 1920–28 ; Beck, 1924;
















2988 TEXT-BOOK OF OPHTHALMOLOGY
Oliver and Crowe, 1927), or merely a congestive condition (Charlin, 1936),
acting perhaps by the creation of a local negative pressure attracting toxins
to the locality—a vacuum sinusitis (White, 1922). None of this can be said
to be proven; and the good results which follow operative measures may be
completely deceptive and without any serious diagnostic value, for it is to be
remembered that most of these cases recover spontaneously whether operated
on or not, so that if every case were operated upon the cures would be about
90% ; moreover, many of the cases thus considered “cured ” have
developed disseminated sclerosis at a later date. With almost equal reason
it can be argued that if recovery is really associated with the operation,
the effect may be due not necessarily to the relief of infection, but perhaps
to vaso-motor reflexes, the immediate effect of haemorrhage and drainage
of tissue -fluid (Escat and Frenkel, 1928), and the subsequent local
hyperaemia and absorption of blood acting as a foreign protein (Benedict,
1933). Dramatic cures have followed operations in which the material
removed has shown no histological evidences of inflammation whatever
(Beck, 1923–24), and it is significant that results as good have been claimed
by the decongestion produced by the local application of ephidrine (v.
Herrenschwand, 1925; Herzog, 1928; White, 1928; Mylius, 1928), or by
the hyperaemia produced by plugging the nose with iodine (Benedict,
1933).
However that may be, it is necessary to admit the possibility of the
direct extension of obvious purulent spheno-ethmoidal disease to the optic
nerve ; but this is probably a rare event. So also is a haematogenous spread
possible, but almost certainly rare. Whether mild or “latent ’’ inflamma-
tions have a similar effect is unlikely : it is certainly not proven, but on the
other hand is not impossible. The present state of our knowledge is such
that no pragmatic opinion based on scientific evidence can be offered, but
individual opinions and theories must still prevail. The sinuses of every
case of retro-bulbar neuritis should be investigated, but the evidence points
to the conclusion that intra-nasal surgery should not be resorted to lightly
except in progressive cases, in the presence of obvious sinus disease and in
the absence of other aetiological factors.
Adie. T. O. S., i, 262, 1930.
Deutschmann. A. f. O., xxviii (2), 291, 1882;
Bartels. A. f. Aug., lvi, 267, 1907.
xxx (3), 77, 331 ; (4), 315, 1884; cxix
Beck. Monat. f. Ohr., lvii, 893, 1923.
Z. f. Aug., liii, 295, 1924.
Behr. Münch. med. W., lxxviii, 1379, 1931.
Benedict. Proc. Staff Meetings, Mayo Clinic,
1933.
A. of O., ix, 893, 1933.
Bordley. A. of O., l, 137, 1921.
Charlin. A. de Oft. H.-A., xxxvi, 77, 1936.
Clegg. Brit. J. O., vi, 118, 1922.
T. O. S., xliv, 86, 1924.
Crane. An. Ot. Rhin. Lary.., xxxvi, 201,
1927.
Cushing, J. Am. Med. As., lxxv, 236, 1920.
347, 1927.
ten Doesschate.
1928.
Elles. Am. J. O., xxi, 1365, 1938.
Escat and Frenkel. A. d’O., xlv., 353, 1928.
Fry. A. of O., xix, 833, 1938.
Fuchs. Z. f. Aug., lvi, 275, 1925.
Gifford. A. of O., v, 276, 1931.
Gilbert. A. f. O., lxxvii, 199, 1910.
Gjessing. A. f. O., lxxx, 153, 1912.
Gradle. An. of O., xxiv, 637, 1915.
Gradle and Meyer. Am. J. O., xii, 802, 1929.
Grönholm. Z. f. Aug., xxiv, 311, 1910.
K. M. Aug., lxxx, 831,
IDISEASES OF THE OPTIC NERVE
2989
v. Herrenschwand. Z. f. Aug., lvii, 78, 1925.
Herzog. A. f. Lary. Rhin.., xxxiii, 604, 1920.
Wien. Jel. W., xxxvii, 972, 1924.
A. f. Aug., xcix, 292, 1928.
v. Hippel. A. f. O., cxxviii, 23, 1932.
v. d. Hoeve. A. f. Aug., lxvii, 101, 1910.
Z. f. Aug., xliii, 223, 1920.
A. of O., li, 210, 1922.
Am. J. O., viii, 101, 1925.
A. f. O., czv, 355, 1925.
Horner. K. M. Aug., i, 71, 1863.
Hutchinson. R. L. O. H. Rep., vii, 35, 1871.
de Kleijn. A. f. O., lxxv, 513, 1910; lxxxii,
150, 1912.
Eleinsasser. Z. f. Aug., xlviii, 61, 1922.
Law. Brit. J. O., xiii, 364, 1929.
Leber. A. f. O., xxvii (1), 325,
lviii (2), 324, 1904.
Lemoine. A. of O., xx, 966, 1938.
Letchworth. Clin. J., lxi, 137, 1932.
Lillie. Am. J. O., xvii, 110, 1934.
1881 ;
Loeb. An. Ot. Ithin. Lary.., xviii, 243, 1909.
Meller.
Meller and Hirsch.
A. f. O., cv, 299, 1921.
Ueber d. rhinogene
Onodi. Z. f. Aug., xii, 23, 1904.
An. Ot. Rhin. Lary.., xvii, 1, 1908.
Pichler. Monat. f. Ohr., lxii, 1159, 1928;
lxiii, 120, 1929. •
Pickworth. J. Lary. Otol., xliii, 186, 1928.
Poos. Am. J. O., xiii, 605, 1930.
Redslob. Rev. olo.-newro. opht., i, 707, 1923;
vii, 405, 1929.
An... d’Oc., clxvii, 692, 1930.
Reiss. A. f. O., lix, 155, 1904.
Scheerer. K. M. Aug., lxxxiii, 164, 1929.
de Schweinitz. O. Rec., xxiv, 163, 1915.
Stark. J. Am. Med. A8., lxxvii, 678, 1921.
Straub. T. O. S., xxxii, 60, 1912.
Thies. A. f. O., cxxii, 75, 1929.
Traguair. J. Laryng. and Otol., xxxix, 384,
I 924.
Weill. An... d’Oc., clx, 793, 1923.
A. of O., i, 307, 1929.
White. Laryngoscope, xxxii, 382, 415, 1922;
xxxiv, 135, 255, 1924.
Boston, Med. J., oxcv, 1195, 1926.
An. Oto. Rhin. Lary.., xxxv, 1163, 1926;
xxxvii, 128, 1928.
Newritis retrobulbare, Berlin, 1926. Wilmer. A. of O., iv, 817, 1930.
Mylius. Z. f. Aug., lxiv, 22, 1928. Wolfkovitch. Sov. vest. oft., ii, 405, 1933.
Oeller. Fest. d. Universität Erlangen, 1901. Worms. A. d’O., liii, 207, 1936.
Oliver and Crowe. A. of Otolaryn., vi, 503, Zeeman. A. f. O., cxii, 152, 1923.
1927.
II. Central Nervous Infections
Four central nervous diseases figure prominently in the aetiology of
optic neuritis : (1) disseminated sclerosis, (2) acute disseminated encephalo-
myelitis, (3) neuro-myelitis optica (of Devic), (4) diffuse peri-axial encephalitis
(of Schilder). It is by no means certain that they represent different disease-
entities, for apparent clinical distinctions may depend on the time-element
or other subsidiary factors rather than on fundamental differences in the
aetiological agent or the pathological process. All of them are of unknown
aetiology, but they are certainly of an infective or toxic origin, the
most likely hypothesis being that they are caused by neurotropic
viruses. Pathologically there is little to distinguish between them ;
they are all characterized by the development of acute inflammatory
lesions involving perivascular invasion by inflammatory cells ; an
extensive demyelinization follows, the destroyed myelin being removed by
the phagocytic microglia ; this is accompanied by degeneration of the axis
cylinders, and finally there is a proliferative gliosis. All of them may attack
the optic nerves, chiasma and tracts, and since the lesions appear where the
nerve-fibres are myelinated, the characteristic lesion in the optic nerve is
retro-bulbar in type : when the disease is extensive, or when it occurs far
forward in the nerve the clinical picture of papillitis may appear. In dis-
seminated sclerosis the lesions tend to be unilateral, circumscribed and insular,
so that the classical type of axial retro-bulbar neuritis tends to be produced :
2990 TEXT-BOOK OF OPHTHALMOLOGY
the immediate prognosis for vision is good but there is a decided tendency
to relapses. In neuro-myelitis optica and acute encephalomyelitis the
involvement is usually bilateral and extensive, but there is little or no
tendency to recurrence. In diffuse peri-axial encephalitis the involvement
is frequently more extensive still, and the disease is always progressive and
fatal. In all of them there is no specific treatment known, and the optic
neuritis must be treated on general lines.
(a) DISSEMINATED (MULTIPLE) SCLEROSIS
There is no doubt that multiple sclerosis is the commonest cause of
optic neuritis (Figs. 2568–2570). The association was first noted by
Charcot (1893) who called attention to the frequent occurrence of “func-
tional ‘’ eye troubles without visible disease of the nerve which were of great
value in diagnosis as a first sign of this disease. Thereafter its frequency
was generally recognized by such writers as Uhthoff (1889) and Marcus Gunn
(1897) who attributed 50–60% of their cases of retro-bulbar neuritis to this
cause. After the beginning of the present century there was, particularly
in America, a Swing towards seeking the Origin of many of such cases in nasal
or focal sepsis, but again more recently the pendulum has swung back and
an even higher proportion of cases has been attributed to this disease
(134 out of 203 cases, Scheerer, 1929; 155 out of 225, Benedict, 1933; and
others). The extreme position was taken up by Adie (1930) that “the only
known common cause and with rare exceptions the only cause ’’ of acute
retro-bulbar neuritis is disseminated sclerosis. Lillie (1934) found in 500
proved cases of disseminated sclerosis seen in his clinic, that in 15% visual
disturbances were the primary episode in the disease, and occurred as a
second or third episode in from 35 to 40%.
The fact that the virus of disseminated sclerosis seems to have a
particular affinity for the optic nerve, so that an optic neuritis is frequently
the first symptom of the disease, makes its diagnosis frequently difficult.
It follows that no other evidences of central nervous involvement may be
present, or at most isolated and relatively insignificant signs, such as absence
of the abdominal reflexes. It is undoubtedly the fact, however, that if these
cases are watched for 10 or 20 years or sometimes even longer (Adie, 1930),
generalized central nervous symptoms develop, the average interval being
6 or 7 years. Thus Fleischer (1908) found that in a series of 30 cases of acute
retro-bulbar disease, 21 showed evidences later of disseminated sclerosis,
while of 12 who had acute papillitis 5 subsequently developed the same
general disease. It is obvious, therefore, that in every case of unilateral
optic neuritis, particularly of retro-bulbar neuritis, the possibility of disseminated
sclerosis should be considered even if no other signs of damage to the central
nervous system are demonstrable.
The typical case of optic neuritis associated with disseminated sclerosis
DISEASES OF THE OPTIC NERVE 2991
occurs unilaterally in a young woman under 35 with the acute onset of a
complete central scotoma without ophthalmoscopic change: it is therefore
of the arial retro-bulbar type. A simultaneous bilateral incidence, a slow
evolution and a less serious fall of vision should suggest an alternative
FIG. 2568.-ACUTE DissEMINATED FIG. 2569.-ACUTE DissEMINATED
SCLERosis. SCLE Rosis.
Discretely outlined patches of demyelina- Discretely outlined patches in
tion in optic nerve (Paton). the chiasma (Paton).
Fig. 25.70—RETRo-BULBAR NEURITIs IN Disse MINATED ScLERosis.
Section through optic canal showing an axial lesion (Behr, A. f. O.).
diagnosis. Nor is absolute amaurosis common, for the discernment at least
of hand movements or the perception of light is usually retained (in every case
in 600, Sarbo, 1932). Tract and chiasmallesions may, however, occur, and
if the inflammation is near the distal part of the nerve, a papillitis may be
evident (5 in 100, Uhthoff, 1889; 2 in 38, Bruns and Stölting, 1900)
T.O.-WOL. III. 3 M


2992 TEXT-BOOK OF OPHTHALMOLOGY
(Fig. 2571). The papillitis is usually slight in degree—a venous engorgement
with blurring of the disc margins, perhaps some haemorrhages and a dioptre
or two of swelling; but occasionally it may be severe, and may show a
swelling of many dioptresso that a trueplerocephalic papilloedema is simulated
(Paton, 1914–36).
The most typical field defect is a dense and absolute unilateral central
scotoma varying in shape and size (Fig. 2572): sometimes it is associated with
a peripheral depression. More rarely multiple small relative scotomata
occur, central, para-central, or peripheral in position, which are indistinct
and difficult to map out and occur bilaterally (Klingmann, 1910; Paton,
1924; Hensen, 1924). When the lesion affects the chiasma or tracts,
hemianopic or quadrantic scotomata appear. In them all a characteristic
Fig. 2571. -Optic NEURiºns IN Acute Fig. 2572. - Dissºux ATED
Dissºux ATED Solerosis (Paton). Solºtosis.
3, 1/330 white.
feature is the variable, mobile and fluctuating course of the defects, which
have the habit of varying in shape, extent and intensity, and readily
leave one part of the field to appear in another or recur after they have
disappeared. In the typical case recovery is rapid: the central scotoma
itself is usually fleeting, rarely lasting longer than 3 months (Hensen, 1924),
but some field defect and some degree of atrophy usually remain, at the least
a temporal pallor and at the most a paper-white disc. Thus out of 134 cases
studied by Scheerer (1929) 5 of whom were practically blind from 6 to 14 days,
all regained full vision save 2, one of whom had 5/12 and the other remained
permanently blind. Subsequent attacks, however, both in the same and
the other eye, may unfortunately occur.
(b) Acute Dissºux ATED ENCEPHALO-MYELITIs
This heading almost certainly embraces a heterogeneous collection of
diseases, among which two large groups can be recognized: spontaneous

DISEASES OF THE OPTIC NERVE 2993
infections and those following some other primary infection, as vaccination,
anti-rabic inoculation and the specific fevers—measles, varicella, mumps,
scarlet fever, whooping-cough (Berliner, 1935; and others). Whether in
some cases of the latter type the responsible organism assumes neurotropic
properties and is itself responsible for the nervous lesions, or whether the
infection has sensitized the nervous system to become susceptible to the
Original infection, or activates another virus, is unknown. The disease
manifests itself first by symptoms of a general infection with headaches,
drowsiness, vomiting and convulsions, a phase which is followed by evidences
of destructive lesions of the central nervous system—sensory disturbances,
loss of reflexes, and pareses. In the 60% of patients who survive, an
immunity seems to be established so that recurrences are unusual.
Despite the widespread and irregular distribution of the areas of
demyelination, a constant feature is involvement of two areas, the sub-
ependymal zone adjacent to the walls of the lateral ventricles and the optic
nerves, chiasma and tracts (Globus, 1932): the lesion is bilateral although
not necessarily simultaneously so, papillitis is common, and some degree of
subsequent atrophy invariable : in addition to the usual central scotoma,
hemianopic defects are common. In mild or abortive cases, indeed, the
involvement of the optic nerves may be the chief feature of the disease
(Klar, 1932).
(c) NEURO-MYELITIs OPTICA (OF DEVIC)
In this disease both optic nerves and the chiasma show marked infiltra-
tion and demyelination, changes which are also found in the cord, a process
which may be diffuse but is usually limited to a few segments in the lower
cervical and upper dorsal region (Figs. 2573–2580); it differs from dis-
seminated sclerosis in that its course is self-limited and relapses do not occur.
This connection between a bilateral optic neuritis and a myelitis was first
noted by Clifford Allbutt (1870) and Erb (1879) and was described as a
clinical entity by Devic (1894), but the disease is rare ; Goulden (1914) was
able to find only 52 cases in the literature, a number brought up to 70 by
Beck (1927), and since then about 50 cases have been reported.
In some 80% of the cases the optic neuritis appears before the myelitis;
and one eye is usually first affected, to be followed after an interval varying
from hours to weeks by involvement of the other. Thereafter the myelitis
appears, usually within 5 or 6 days, but it may be delayed for 2 months.
The neuritis is acute and profound, almost complete blindness with a widely
dilated immobile pupil being the rule, but in favourable cases, after an
interval of a few days or weeks, vision begins to return, and the ultimate
recovery, although frequently with restricted fields, may be astonishing
even in the presence of completely white discs (Paton, 1936 ; Fetterman and
Chamberlain, 1940). Sometimes the process is entirely retro-bulbar so
3 M 2
Figs. 2573-77-NEuro-Myºlitis Optica.
Fros. 2573–74–Longitudinal section of optic nerve showing diffuse demyelinating process.
Fig. 2573.-Right nerve. Fig. 2574. —Left nerve.
Figs. 2575–76.-Transverse section of the optic nerves.
Fig. 2575.-A limited area of Fig. 2576–Diffuse demyelination
demyelination. (same case as Figs. 2573–74).
Fig. 2577–Superior portion of chiasma; it and the tracts are severely demyelinated with cavity
formation, but the commissure of Meynert is intact. Weigert-Pal. (Leslie Paton.)



DISEASES OF THE OPTIC NERVE 2995
that no changes are seen ophthalmoscopically; but more usually a papillitis
is evident, the appearance varying from hyperaemia and muzziness of the
margins to a distinct amount of swelling with exudates and haemorrhages.
Pathologically the lesions in the nerve are usually most marked anterior to
the chiasma, but they may extend proximally to the tracts: there is intense
perivascular infiltration and massive demyelination over very extensive
FIGs. 2578–80.-NEURO-MYELITIs OPTICA : TRANsverse SECTIONs of THE Corp.
Fig. 2578–Sixth thoracic seg, Fig. 2579.-Seventh thor-
.." li º d wºad acic segment showing less
emyelination and cavitation,
with a dilated canal. marked changes.
Fig. 2580.-First sacral segment, with changes only in the right lateral column
(Leslie Paton).
areas (Achard and Guinon, 1889; Katz, 1896; Bielschowski, 1901; Beck,
1927; Milian and others, 1931; Fralick and De Jong, 1937).
The myelitis tends to have an insidious onset and has the usual evidences
of paresis, loss of reflexes and trophic disturbances; it also may largely
resolve, although a perfect recovery is rare, but in some 50% of cases it
proves fatal by ascending to involve the vital medullary centres. The many
measures of treatment which have been tried have met with little or no
success (Fralick and De Jong, 1937).


2996 TEXT-BOOK OF OPHTHALMOLOGY
Figs, 2581–84.-ENCEPHALITIs PER1-Axialis DIFFUSA.
Figs. 2581–82.-Degeneration in occipital lobes.
Fig. 2581.-General view Fig. 2582–Higher magnification
(Leslie Paton). (Leslie Paton).
sº
- ºf - - -
Fig. 2584–The optic nerve : longitudinal section (Leslie Paton).


DISEASES OF THE OPTIC NERVE 2.997
(d) ENCEPHALITIS PER1-Axialis DIFFUSA (of ScHILDER)
Encephalitis peri-axialis diffusa, a disease first described by Schilder
(1912) which occurs chiefly in infancy and childhood, is characterized by a
massive demyelination in the sub-cortical regions of the cerebral and cere-
bellar hemispheres, the occipital and temporal lobes being particularly
affected, a process followed by a dense
replacement gliosis (Figs. 2581–82). It
differs from the other demyelinating
types in the absence of an acute meso-
dermal reaction, a circumstance which
led Globus and Strauss (1928) to classify
it as a degenerative encephalopathy
rather than an inflammation. The -------------- - -
disease is rare; but Berliner (1935) Fig. 2585–Acute Optic Neurºtis is
was able to collect 31 cases from the º PERI-Axialis DIFFUsA
literature.
Clinically it is characterized by signs of general cerebral involvement—
apathy, blindness, deafness, spastic paralysis—which run a chronic course
and reach a fatal conclusion after an interval varying from weeks to some
years. In 60% of the cases blindness has resulted, coming on usually as a
late symptom, and in practically all of these cases complete bilateral optic
atrophy developed before death. Acute papillitis is rare (Schilder, 1912:
Shelden, 1929); and histological examination may fail to reveal a single
myelin sheath in the optic nerve (Figs. 2583–84).
(e) HERPEs zost ER
Optic neuritis occurs as a rare complication of herpes zoster ophthal-
micus: when it does occur, however, it may be severe, involving the total
abolition of the perception of light and being followed by atrophy. The
reports, however, in the literature are few (Jonathan Hutchinson, 1866;
Bowman, 1869 ; Daguenet, 1877; Gould, 1888; Wangler, 1889; Sulzer,
1898; Veasey, 1919; Paton, 1923–26; Murzin, 1937).
It is of interest here to note the occasional occurrence of OPTIC NEURITIs witH
PolyNEURITIs of THE CRANIAL NERVES : the condition is probably usually toxic in
origin, or may be associated with the ingestion of a poison, such as apiol (Shumway,
1905–35; Brain, 1934). It also has been noted in puerperal polyneuritis (Schanz,
1896). An axial neuritis has been noted as one of the central nervous complications
of botulism in association with involvement of the cranial nerves (Barbel, 1932). It is
interesting also that a severe neuritis without necessarily other central symptoms but
involving abolition of perception of light, may follow an anti-rabic inoculation (Cormack
and Anderson, 1934; Hasabe, 1934).

2998
TEXT-BOOK OF OPHTHALMOLOGY
Achard and Guinon.
1889.
A. de Méd. earp., 696,
Adie. T. O. S., I, 262, 1930.
Allbutt. Lancet, i, 76, 1870.
Barbel. Russ. A. O., viii, 591, 1932.
Beck. Brain, i, 687, 1927.
Benedict. A. of O., ix, 893, 1933.
Berliner. A. of O., xiii, 83, 1935.
Bielschowski. Myelitis w. Sehnerventzündung,
Berlin, 1901.
Bowman. R. L. O. H. Rep., vi, 1, 1869.
Brain. T. O. S., liv, 221, 1934.
Bruns and Stölting. Momat. f. Psy. Neurol.,
vii, 184, 1900.
Z. f. Aug., iii, 1, 1900.
Buzzard. Lancet, ii, 835, 1918.
Charcot. Wiestn. O. Ref. A. d’O., xiii, 397,
1893.
Cormack and Anderson. Brit. J.O., xviii, 167,
1934.
Daguenet. Rec. d’O., iv, 117, 1877.
Devic. Congr. Med. Internat., Lyons, 1894.
Erb. A. f. Psy., x, 146, 1879.
Fetterman and Chamberlain, A. of O., xxii,
577, 1940.
Fleischer. K. M. Aug., xlvi (1), 113, 1908.
Fralick and De Jong. Am. J. O., xx, 1119,
1937.
Globus. A. of Neurol. Psy., xxviii, 810, 1932.
Globus and Strauss. A. of Neurol. Psy., xx,
1190, 1928.
Gould. Polyclinic, vi, 109, 1888.
Goulden. T. O. S., xxxiv, 229, 1914.
Gunn. T. O. S., xvii, 207, 1897.
Hall. T. O. S., xli., 499, 1921.
Hensen. K. M. Aug., lxxii, 75, 1924.
Hogue. Am. J. O., iv, 592, 1921.
Holden. A. of O., l, 101, 1921.
Hutchinson. R. L. O. H. Rep., v, 191, 331,
1866.
Katz. A. f. O., xlii (1), 202, 1896.
Klar. K. M. Aug., lxxxix, 645, 1932.
Klingmann. J. Nervous Mental Dis., xxxvii,
734, 1910.
Lenz. K. M. Aug., lxvi, 928, 1921.
Lillie. Am. J. O., xvii, 110, 1934.
Milian et al. Rev. neurol., ii, 257, 1931.
Much and Hüppi. K. M. Aug., xciii, 333,
1934. -
Murzin. Sov. vest. O., xi, 758, 1937.
Paton. T. O. S., xxxiv, 252, 1914 :
137, 1924.
P. R. S. Med. (Sect. O), xvi, 27, 1923.
Brit. J. O., x, 305, 1926.
A. of O., xv, 1, 1936.
Salvati. A. dº Ott., xxxviii, 310, 1931.
Sarbo. K. M. Aug., lxxxviii, 762, 1932.
Schanz. Deut. med. W., xxii, 443, 1896.
Scheerer. K. M. Aug., lxxxiii, 164, 1929.
Schilder. Z. ges. Neurol. Psy., x, 1, 1912.
Shelden et alia. A. of Neurol. Psy., xxi, 1270,
1929.
Shumway. J. Am. Med. As., xliv, 463, 1905.
A. of O., xiii, 8, 1935.
Sulzer. An. d’Oc., czix, 401, 1898.
Swab. A. of O., xix, 926, 1938.
Symonds. Lancet, ii, 1245, 1920.
Uhthoff. A. f. Psy., xxi, 55, 1889.
Veasey. T. Am. O. S., xvii, 297, 1919.
Waardenburg. Am. J. O., iv, 580, 1921.
xliv,
Hasabe. Acta S. O. Japan, xxxviii,
1934.
102, Wangler. Diss., Zürich, 1889.
III. LEBER'S DISEASE
Leber's disease is a relatively rare condition of unknown aetiology but
with a definite hereditary tendency, affecting particularly males about the
20th year of life, which, without associated constitutional disturbances, is
characterized by a rapid onset of visual failure affecting both eyes in different
degrees and not simultaneously, showing in its early stages fleeting evidences
of optic neuritis, followed by a secondary atrophy which involves a serious
permanent impairment of central vision with retention of the peripheral
fields.
While early cases of this condition were described by v. Graefe (1858) and others,
it was left to Leber (1871), then a young clinician of 31 years of age, to establish the
existence of this hereditary type of visual defect as a clinical entity by collecting
55 cases in 16 families. Since his classical paper the literature on the subject has been
enormous, and has been gathered together by Klopfer (1898) (214 cases in 48 families),
Hormuth (1900) (284 cases in 65 families), Nettleship (1909) (360 cases), and Julia
Bell (1931) (1,182 cases in 240 families). Of these last, 17 pedigrees involving 164
affected members occurred in Japanese, and the remainder were of European races.
DISEASES OF THE OPTIC NERVE 2999
Heredity. Although sporadic cases do occur it is undoubted that in
the main Leber's disease is hereditary ; but the laws governing its trans-
mission are by no means clear. To a large extent it is recessive and sex-
linked, but not on strictly Mendelian principles (Fig. 2586). It occurs
generally in males and is usually transmitted through females who themselves
are unaffected (thus Bell, 1931, found that of 573 males 95%, and of 88 females
84% were affected through the mother); but under special conditions the
father may transmit the disease and an affected mother may transmit it to
her son (Lawson, 1907). The number of cases occurring in a particular
family are as a rule small (Blegvad and Rönne, 1920). Moreover, cases
occur wherein the condition suddenly appears in a family hitherto quite
healthy in abnormal circumstances of marriage. Thus Weekers and Hubin
(1933) traced a family back to the 14th century which showed no evidence of
visual disability, and then when three brothers married three sisters the
Q
! - !, T,
ſº . . . . . . […]
ſº º, lº º { º
ſ!! ºlli ! ºn

FIG. 2586.-LEBER's OPTIC NEURITIS.
Recessive heredity (Waardenburg).
disease suddenly appeared in all three families: this suggests the union of un-
known predisposing factors present in two separate families. Consanguinity
is not common in the genealogies, but has been reported from time to time.
Thus Russell (1931) reported a family wherein, although the three previous
generations were healthy, a consanguineous marriage resulted in the appear-
ance of three cases among the children : it is evident that consanguinity
can only increase the incidence of a familial disease if both parents carry
the disease-factor. Such an occurrence also suggests the presence of a
second determining factor, not sex-linked ; or alternatively can be explained
by a reduction in the general vigour of the stock by consanguinity so that a
dormant trait became manifest. It would appear, however, that if the
sisters of affected males were to refrain from breeding the disease would be
practically exterminated.
An anomalous type of hereditary atrophy has quite a different mode of inheritance.
This type tends to be congenital, or at any rate appears in early childhood, affects both
sexes equally, and has a dominant transmission, appearing in Successive generations,
3000 TEXT-BOOK OF OPHTHALMOLOGY
and may be transmitted sometimes both through the male or female members of the
pedigree (Rampoldi, 1883 : 7 members of 4 generations), sometimes through affected
fathers only (Griscom, 1921 : 14 members of 3 generations), and sometimes through
affected mothers only (Thompson and Cashell, 1935: 16 members of 6 generations)
(Fig. 2587). It is probable that such cases cannot be accepted as Leber's disease.
Sea. Leber (1871) originally found that females were affected much
more rarely than males, in the proportions of 1:9 ; Hormuth (1900) found
88% males ; but Bell (1931) found the curious anomaly that while in peoples
of European race this high percentage of males obtained (84.8%), among
the Japanese the proportion between the two sexes were more equal
(59.1% males).
Age. Leber’s (1871) dictum that the disease manifests itself essentially
soon after puberty, between the ages of 18 and 23, has been found to remain
O
+
-H. H-
© Öſ & e e
+ + +
| | | –
* : * 6’ Af eſ
| | […T. F- | | ſ=–
e is sº sº sº of e º 6’ 3’ & 5 &
+ •+ +- -4-
º H Ar ºf H
o Q 2 ° O O O O

FIG. 2587.-HEREDITARY OPTIC ATROPHY.
Dominant heredity (Thompson and Cashell).
substantially true. In the comprehensive statistics gathered by Bell (1931),
the average age of Onset among Europeans is 23 years for males and 25 for
females; while among Japanese it is slightly earlier and with a reversal of
the sexes (21 years in males and 20 in females). Cases do, however, occur
well outside these limits. At one extreme Sedgwick (1862) observed a man
who became blind, aged 60, whose four sons lost their vision aged 56, 46,
46 and 42. Other cases in advanced age have been reported by Fuchs
(1879), 57 years, Norris (1882), 50 years, and others, while instances occurring
in the 4th and 5th decades are relatively common. On the other hand cases
may occur before puberty; indeed, although it is denied by some (Drexel,
1922), there is considerable reason for including those cases of congenital
optic atrophy which occur in the pedigrees of Leber's disease as belonging
to the same group (Nettleship, 1909; Usher, 1927; Reidl, 1935), although
DISEASES OF THE OPTIC NERVE 300 I
it is questionable whether those pedigrees of congenital atrophy with a
dominant transmission can be legitimately considered in the same category.
Clinical Course. In the typical case, which occurs in a young and
otherwise healthy male of about 20 years of age, the disease is ushered in by
a rapid loss of sight in one eye which fails quickly for some days or weeks and
then more slowly so that the maximum disability is reached in two months ;
it is rare for symptoms to progress after six months. Thereafter some
slight improvement usually occurs very gradually, but in a general sense
the defect can be considered stationary so that a serious permanent impair-
ment of vision is the rule. The other eye is seldom involved simultaneously
or to the same degree ; it usually shows symptoms within a few weeks and
it is rare for its onset to be delayed for six months. Occasionally the loss
of vision is quite sudden (Bedell, 1934), and sometimes it may be preceded by
local pain. Ophthalmoscopically in the
early stages a mild degree of papillitis is *——o.
usually observed, involving an obscuration º Af
of the disc margin without the development 2-
Of hemorrhages or exudates or any degree %*s *
of swelling ; and after the acute stage ſ ſº
has passed the appearances of secondary . ||| {{#iº º
atrophy develop, limited in the milder cases \ |\º #[]
to a temporal paleness, and in the more 3.
serious appearing as a complete blue-grey
pallor (Fig. 2631, Plate LXII).
The characteristic scotoma is a central
one with a dense absolute nucleus at the Oz &
fixation point and a sector-shaped de- Fig. 2588–LEBER's Optic ATRoPHY.
pression breaking through in One segment 20, 5, 1/330, white.
to the periphery usually upwards and inwards, thus indicating a sub-
chiasmal defect in the nerve trunk (Rönne, 1910) (Fig. 2588). This
central scotoma occurs in the vast majority of cases (75%, Groenouw, 1904;
88%, Bell, 1931), and in some 35% of cases is associated with peripheral
contraction. It is, however, not invariably present, a peripheral contraction
alone being sometimes evident (Leber, 1871; Alexander, 1874 ; Knapp,
1904; Batten, 1909; Clemesha, 1910; Worton, 1913 ; Hirsch, 1923;
Usher, 1927). If improvement does occur, central vision may recover first
so that a ring scotoma may develop in the later stages (Hancock, 1907).
A slight degree of improvement of vision is not uncommon, which is
usually greater in young subjects, but the usual recuperative propensities
of retro-bulbar lesions are little in evidence. Thus in 211 cases Bell (1931)
found that 86 (29%) showed considerable betterment, of which 15 showed
a marked improvement and 12 apparently complete recovery. Thus Nettle-
ship (1903) found that a patient whose vision fell to 6/60 eventually read the
smallest type with ease ; Hancock (1907) in a family of 12 affected members



















3002 TEXT-BOOK OF OPHTHALMOLOGY
observed almost complete recovery in 6: Batten (1909) recorded a case
whose vision reached 6/6, Kawakami (1926) another in whom symptoms
completely disappeared, while marked degrees of amelioration have been
recorded by others (Usher, 1927; Holloway, 1933; and others). On the
other hand, complete blindness is exceptional so that the prognosis, although
bad for central vision, is not absolutely unfavourable. The prognosis as
to life seems to be unaffected by the presence of the disease. No treatment
is known to be of any value.
Complications in Leber's disease are rare. Leber (1871) considered that it is
essentially found in families with a neuropathic taint among whose members headaches,
Fig. 2589–LEBER's Optic ATRophy.
Transverse section 3 mm. behind globe. On the nasal side (left) the nerve tissue
is normal; on the temporal (right), atrophic (x 27) (Rehsteiner, A. f. O.).
vertigo, tremors and such indefinite symptoms occur. This has been remarked in the
literature, as witness the occurrence of epilepsy (Hancock, 1907), insanity (Story, 1885),
cramp and pains in the limbs (Taylor, 1892; Batten, 1909; Russell, 1931). Nystagmus
has also been noted (Nettleship, 1897; Behr, 1909; Waardenburg, 1924; Pines and
Tron, 1925: Usher, 1927); but evidences of organic central nervous disease are so
exceptional that they may be regarded as incidental (Habershon, 1887; Taylor and
Holmes, 1913).
Pathology. Despite the fact that this disease has been known for
three-quarters of a century and well over a thousand cases are on record,
only one pathological report is available (Rehsteiner, 1930) (Fig. 2589). In
this case the optic nerve was markedly atrophic in the position corresponding
to the papillo-macular bundle where few or no medullary sheaths remained

DISEASES OF THE OPTIC NERVE 3003
and glial overgrowth was very evident. The only intra-ocular change was
atrophy of the ganglion cells and the nerve fibre layer of the retina.
The aetiology of Leber's disease is quite conjectural. Two views have
been put forward neither of which can be substantiated. By the majority
of writers it is considered to be a neuritis followed by atrophy belonging to the
towic-inflammatory group ; but what the causal agent may be is still a
mystery. The suggestion was put forward by Fisher (1916) that it was due
to an inherited temporary disease of the pituitary gland becoming evident
after puberty. His argument was based partly on the similarity of the clinical
signs and symptoms to those associated with pituitary enlargement, and
partly on the finding of X-ray evidence of enlargement or other abnormalities
of the pituitary fossa (Brunner, 1912; Fisher, 1916–17 ; Pollock, 1917;
Zentmayer, 1918; Borroughs, 1924; and others); but these findings have
not been universally endorsed. Thus in 60 X-ray examinations reported
in the literature and analysed by Bell (1931), the sella turcica was enlarged
only in 14, and in a typical case examined operatively with a view to exploring
this region, Kuhn (1931) found no abnormalities. A general disturbance
of the ductless glands has also been suggested (v. Hippel, 1915).
The second school of thought considers the disease to belong to the
class of abiotrophies—an inherent lack of vital energy in the tissues to
maintain their function (Gowers, 1902; Collins, 1922; and others). This
conception has much to be said for it and is notoriously difficult to disprove,
although the general character of the field-defect and its behaviour, as well
as the occasional but quite authentic fact of its recovery, points rather to a
toxic-inflammatory lesion than to a primary and therefore presumably final
atrophy.
Alexander. K. M. Aug., xii, 62, 1874. v. Hippel. A. f. O., xc, 198, 1915.
Batten. T. O. S., xxix, 144, 1909. Hirsch. K. M. Aug., lxx (1), 710, 1923.
Bedell. Am. J. O., xvii, 195, 1934. Bolloway. A. of O., ix, 789, 1933.
Behr. K. M. Aug., xlvii (2), 138, 1909. Hormuth. Diss., Heidel., 1900.
Bell. Treasury of Human Inheritance, ii (4), Kawakami. A. f. O., czvi, 568, 1926.
1931. Rlopfer. Diss., Tübingen, 1898.
Blegvad and Rönne. K. M. Aug., lxv, 206, Knapp. A. of O., xxxiii, 383, 1904.
1920. Kuhn. A. of O., v, 408, 1931.
Borroughs. T. O. S., xliv, 399, 1924. Lawson. T. O. S., xxvii, 169, 1907.
Brunner. T. A. m. O. S., xiii, 162, 1912. Leber. A. f. O., xvii (2), 249, 1871.
Clemesha. Am. J. O., xxvii, 139, 1910. Nettleship. T. O. S., xvii, 277, 1897; xxiii,
Collins. Internat. Cong., Washington, 103, 109, 1903; xxix, p. cv, 1909.
1922. Norris. T. Am. O. S., iii, 355, 1882.
Drexel. A. f. Awg., xcii, 49, 1922. Pines and Tron. Z. ges. Newr. Psy., xcv, 762,
Fisher. T. O. S., xxxvi, 298, 1916 ; xxxvii, 1925.
251, 1917. Pollock. T. O. S., xxvii, 247, 1917.
Fuchs. K. M. Aug., xvii, 332, 1879. Rampoldi. Am. di Ott., xii, 1, 269, 1883.
Gowers. Lancet, i, 1003, 1902. Rehsteiner. Schw. med. W., lx, 122, 1930.
v. Graefe. A. f. O., iv (2), 266, 1858. A. f. O., cxxv, 14, 1930.
R. M. Aug., iii, 222, 1865. Reidl. K. M. Aug., xcv, 332, 1935.
Griscom. Am. J. O., iv, 347, 1921. Rönne. K. M. Aug., xlviii (1), 331, 1910.
Groenouw. G.-S. Hb., II, xi (1), 453, 1904. Russell. T. O. S., li, 187, 1931.
Habershon. O. Rev., vi. 330, 1887. Sedgwick. Med. Times and Gaz., i, 309,
Hancock. R. L. O. H. Rep., xvii, 167, 1907. 1862.
3004 TEXT-BOOK OF OPHTHALMOLOGY
Story. T. Acad. Med. Ireland, iii, 23, 1885. Usher. Brit. J. O., xi, 417, 1927.
Taylor. T. O. S., xii, 146, 1892. Waardenburg. K. M. Aug., lxxiii (2), 619,
Taylor and Holmes. T. O. S., xxxiii, 95, 116, 1924. -
1913. Weekers and Hubin. A. d’O., l, 241, 1933.
Thompson and Cashell. P. R. S. Med., Worton. Lancet, ii, 1112, 1913.
xxviii, 1415, 1935. Zentnayer. A. of O., xlvii, 627, 1918.
IV. Optic Newritis from Endogenous Toacims
1. ACUTE INFECTIVE DISEASEs are sometimes associated with optic
neuritis, either a papillitis or a retro-bulbar lesion. To a certain extent such
an event suggests the association of acute disseminated encephalo-myelitis
with these diseases, and although the lesion in the optic nerve may appear
as an isolated phenomenon without other central nervous symptoms or
sequelae, it may be argued that the rationale is the same, the Ocular involve-
ment being a mono-symptomatic evidence of central nervous involvement.
On the other hand the absence of complications, the relative mildness of the
disease and the good prognosis suggest a more simple toxic cause.
In any event an optic neuritis has been reported as an incidental
complication in a number of acute infective conditions. Analysing 253 cases
in the earlier literature, Uhthoff (1900) gathered the following statistics:
Influenza ge * e . 72 cases. | Beri-beri e e e . 5 cases.
Syphilis gº g e . 61 , , Erysipelas 3 ,
Rheumatism . te º . 36 , , Scarlet fever . 3 ,
Malaria . * & g . 17 , , Tuberculosis . * . 3 ,
Typhoid fever & tº . 17 ,, Typhus exanthematus . . 3 ,
Measles . e & e . 9 , , Gonorrhoea * e 2 ,
Diphtheria 6 ,, Recurrent typhus 2 ,
Polyneuritis . * tº . 7 , Acute rheumatism . I case.
Variola. 6 , ,
2. SEPTIC FOCI have always been accused of causing an optic neuritis,
and, although the scientific establishment of the relation of cause and effect
is impossible, it is probable that they may be responsible through their
toxic effects. Usually the visual defect appears as a central scotoma, but
in the more chronic cases it has been suggested that a peripheral contraction
with general lowering of the visual acuity may occur (Davies, 1930). It
may be that many of the so-called “ rheumatic ’’ cases associated with
rheumatic manifestations elsewhere (Groenouw, 1900), or coming on after
exposure to cold (Buchanan, 1923), belong to this category. It may be
argued, and with much reason, that there is little evidence that such toxins
show a sufficiently strong neurotropic tendency to produce such an effect ;
and while in the present state of our knowledge it is unjustifiable to give
any dogmatic ruling, it seems quite illegitimate to speculate that a very
large proportion of cases of optic neuritis (44%, Wilmer, 1930; 63%, White,
1928) is due to this cause.
DISEASES OF THE OPTIC NERVE 3005
However this may be, typical cases of optic neuritis in which the writers have
considered a septic aetiology justifiable are commonly reported. Some typical papers
are as follows: teeth—Jessop, 1907; Saint-Martin, 1921 ; Ackland, 1923; Lang,
1923; White, 1928; sinuses, acting as a septic focus—Pihl, 1905; White, 1928;
Wilmer, 1930; intestine, Allport, 1912.
3. ENDOGENOUs TOXINS seem quite definitely to be associated with the
development of optic neuritis. The most common are as follows:–
Diabetes. Although a rare complication in diabetes, retro-bulbar
neuritis is by no means unknown ; thus Leber (1896) saw 14 cases in 50
diabetics, and Foster Moore (1920) 6 in 61. It attacks people all ages and is
frequently bilateral, but the great majority of victims belong to the male
sex (Blegvad and Rönne, 1920). Coming on suddenly with the develop-
ment of a scotoma, it may clear up rapidly or may persist indefinitely result-
ing in some degree of permanent atrophy. Pathological examination has
revealed nothing distinctive in the type of
atrophy (Nettleship and Edmunds, 1881 ;
Lawford and Edmunds, 1883 : Frazer and
Bruce, 1895; Schmidt-Rimpler, 1896; v.
Grosz, 1898 ; Rönne, 1913 ; Thomsen,
1921). The scotoma, which may be relative
Or absolute, is usually central, and may be
centro-caecal, but it is sometimes peri-
central ; it is well-defined with steep edges
and may vary considerably in shape
(Fig. 2590). Although the scotoma lacks
the constant and typical characteristics of
tobacco amblyopia, in many of the cases its
origin from diabetic toxins has been uncon- Fig. 2590-RETRO-Bulbar NEURITIs
tº e tº e IN DIABETEs.
vincingly demonstrated ; and, since it may 5, 1 '330.
be argued that diabetics are more prone * ,
to toxic effects generally than normal subjects, it has not always been clear
that the condition is not due to poisons such as alcohol and tobacco. Thus
Kako (1903) could only exclude alcohol and tobacco in 2 out of 16 of his
cases ; but on the other hand, so many instances have occurred wherein
other poisons can be excluded that the existence of the condition as a
diabetic complication must be admitted (Reber, 1910, and others). It may
have some connection with diabetic acidosis thus raising some theoretical
parallelism between alcohol and acetone (Gallus, 1922; Francis and Koenig,
1926); while the factor of arteriosclerosis is obvious in certain cases
(Hummelsheim and Leber, 1901 ; Abelsdorff, 1924).
The Amcemias. The occasional occurrence of optic neuritis with the anaemias
has been noted (Benedict, 1933); of particular interest is its appearance in pernicious
anaemia with evidence of central nervous involvement (sub-acute combined degenera-
tion of the cord) (Hine, 1935).

3006 TEXT-BOOK OF OPHTHALMOLOGY
Malignant Disease. In the toxic states of advanced malignant disease,
symptoms of retro-bulbar neuritis may occur, with a central scotoma due
to atrophy of the papillo-macular bundle. This is probably another instance
of the preferential vulnerability of these fibres to toxic processes (Nuel, 1896;
Lohmann, 1902; Birch-Hirschfeld, 1907; de Kleyn and Gerlach, 1914).
Pregnancy and Lactation. Child-bearing has long been associated with
a papillitis or a retro-bulbar neuritis ; the complication, however, is rare
both during pregnancy (Reich, 1882; Bull, 1892; Knapp, 1893 : Knaggs,
1896; Kipp, 1906; Weigelin, 1908; Langenbeck, 1914; and others) or
in the puerperium (Reuling, 1877; Pflüger, 1878); a similar complication
may also occur during lactation (Nettleship, 1893; Derby, 1905; Villard,
1912; Lillie, 1934). The prognosis of the commoner type occurring in
pregnancy is not always good, for bilateral blindness has resulted (Weigelin,
1908); if, therefore, the condition does not clear up rapidly, an artificial
termination of the pregnancy should be considered. In lactation the
condition appears typically about the 7th week in the strong and healthy
as well as in the less robust. It is frequently bilateral, a papillitis is
not unusual, the loss of sight is usually rapid, and recovery, while some-
times quick, may be slow ; it is, however, usually greatly accelerated by
weaning. The cause of such a neuritis is obscure. It has been suggested
that pregnancy is an incidental factor in the aetiology, precipitating, for
example, a neuritis due to disseminated sclerosis, but its reported occurrence
in three consecutive pregnancies seems to indicate a closer relationship
(Kagan, 1934). On the other hand, it may be associated with an upset in
metabolism or be toxic in origin, while there is no doubt that some cases are
pressure effects associated with enlargement of the pituitary (Hagedoorn,
1937).
It is noteworthy that an optic neuritis has been found in association with puerperal
polyneuritis (Schanz, 1896) and with a toxaemia of pregnancy (Schiótz, 1921).
The association of optic neuritis with puberty, menstruation and the climacteric is
much less clear ; but isolated cases have been reported wherein symptoms of an
acute axial neuritis may conceivably be associated with a toxic condition due to
pituitary disturbance rather than to simple pressure effects (Stein, 1922).
Extensive skin burns may involve sufficient toxic absorption to give rise to an
axial retro-bulbar neuritis (Thies, 1925). -
Severe haemorrhage, particularly of non-traumatic origin, may occa-
sionally give rise to a bilateral optic neuritis of bad prognosis, which may
be followed by necrosis of the ganglion cells of the retina, optic atrophy, and
blindness. The pathology and clinical features of the condition have already
been discussed when it was pointed out that in all probability the ocular
effect is essentially toxic, due to absorption of blood protein in massive
Quantities.
1 p. 2556.
DISEASES OF THE OPTIC NERVE 3007
Avitaminosis
There is a very considerable amount of evidence that avitaminosis,
notably lack of vitamin B, may be responsible for the development of an
optic neuritis usually of the axial type but sometimes appearing as an
exudative papillitis. Extreme degrees of this type of deficiency appear as
pellagra, which is typically characterized
by dermatitis, stomatitis, diarrhoea, poly-
neuritis, and psychic disturbances, par-
ticularly of the depressive type. These
are associated with degenerative changes
in the central nervous system, and, as an
Occasional event, with an optic neuritis
which may progress to atrophy (Fig. 2591).
Such cases have been observed in Italy
(Bietti, 1901), in Russia (Krylov, 1932), in
Japan (Simizu, 1934) and in America
(Whaley, 1909; Calhoun, 1918; Levine,
1934; Fine and Lachman, 1937). There
is evidence, also, that the condition of
alcoholic polyneuritis is closely allied to
pellagra, the deficiency in diet being partly
due to inadequate intake of solid food and partly to impaired digestion by
an alimentary canal grossly impaired by the continual ingestion of large
Quantities of alcohol over long periods; in this condition also an axial Optic
neuritis may occur (Cronin, 1933; Carroll, 1936). This raises the question
of a possible influence of avitaminosis in the common type of tobacco-alcohol
amblyopia.' A similar type of amblyopia has been noted in beri-beri (Elliot,
1920).
FIG. 2591.-RETRO-BULBAR NEURITIS
IN PELLAGRA.
5, 1/330, white.
In Japan an axial neuritis has been noted occurring in epidemics in association
with a superficial keratitis, apparently caused by a deficiency in vitamin A (Oguchi,
1930).
Abelsdorff. A. f. Aug., xcv, 143, 1924.
Ackland. T. O. S., xliii, 417, 1923.
Davies. T. O. S., l, 393, 1930.
Derby. A. of O., xxxiv, 9, 1905.
Allport. O. Rec., xxi, 670, 1912. Elliot. Tropical Ophthalmology, London,
Benedict. A. of O., ix, 893, 1933. 1920.
Bietti. K. M. Aug., xxxix (1), 337, 1901. Fine and Lachman. Am. J. O., xx, 708, 1937.
Birch-Hirschfeld. A. f. O., lxv, 440, 1907.
Blegvad and Rönne. K. M. Aug., lxv, 206,
1920.
Buchanan. Brit. J. O., vii, 170, 1923.
Bull. Am. d’Oc., cviii, 286, 1892.
Burstein. Sov. vest. Oft., v, 476, 1934.
Calhoun. Am. J. O., i, 834, 1918.
Carroll. A. of O., xvi, 919, 1936.
Cronin. J. Nerv. Mental Dis., lxxviii, 35,
1933.
Francis and Koenig. J.
lxxxvii, 1373, 1926.
Frazer and Bruce. Brit. Med. J., i, 1149,
1895.
Gallus. Z. f. Aug., xlviii, 89, 1922.
v. Grosz. Orvosi Hetilap. Szemeszet, 2, 1898.
Groenouw. K. M. Aug., xxxviii, 186, 209,
1900.
Hagedoorn. Am. J. O., xx, 690, 1937.
Harry. Brit. J. O., vi, 216, 1922.
A m. Med. As.,
1 p. 3011.
T.O. —WOL. III.

3008
TEXT-BOOK OF OPHTHALMOLOGY
Hine. P. R. S. Med., xxix, 386, 1935.
Hummelsheim and Leber. A. f. O., lii, 336,
1901.
Jaeger. K. M. Aug., lxxxvi, 812, 1931.
Jessop. T. O. S., xxvii, 170, 1907.
Kagan. Sov. vest. Oft., v, 503, 1934.
Kako. K. M. Aug., xli (i), 253, 1903.
Ripp. O. Rec., xv, 320, 1906.
de Kleyn and Gerlach. A. f. O., lxxxvii, 157,
1914.
Knaggs. T. O. S., xvi, 330, 1896.
Knapp. Brit. Med. J., ii, 731, 1893.
Erylov. Sov. vest. Oft., i, 388, 1932.
Lang. T. O. S., xliii, 426, 1923.
Langenbeck. A. f. O., lxxxvii, 226, 1914.
Lawford and Edmunds. T. O. S., iii, 160,
1883. -
Leber. B. O. G. Heidel., xxv, 104, 1896.
Levine. A. of O., xii, 902, 1934.
Lillie. Am. J. O., xvii, 110, 1934.
Lohmann. Cb. pr. Aug., xxvi, 17, 1902.
Marburg. Z. f. Aug., xliv, 125, 1920.
Moore, Foster. T. O. S., x1, 15, 1920.
Medical Ophthal., II, 210, 1925.
Nettleship. R. L. O. H. Rep., xiii, 97, 1893.
Oguchi. Acta S. O. Jap., xxxiv, 125, 1930.
Pflüger. A. f. O., xxiv. (2), 169, 1878.
Pihl. K. M. Aug., xliii (2), 50, 1905.
Reber. O. Rec., xix, 121, 1910.
Reich. K. M. Aug., xx, 349, 1882.
Reuling. N. Y. Med. J., xxvi, 393, 1877.
Rönne. A. f. O., lxxxv, 489, 1913.
Sabatzky. K. M. Aug., lxxxii, 351, 1929.
Saint-Martin. La Clin. Opht., xxv, 325,
1921.
Schanz. Deut. med. W., xxii, 443, 1896.
Schiótz. K. M. Aug., lxvii. Beil., 1, 1921.
Schmidt-Rimpler. B. O. G. Heidel., xxv, 99,
1896.
Simizu. Acta S. O. Jap., xxxviii, 106, 1934.
Stein. A. f. Aug., xci, 256, 1922.
Thies. K. M. Aug., lxxv, 384, 1925.
Thomsen. Investigations on Degeneration of
the Optic Nerve, Aarhus, 1921.
Uhthoff. B. O. G. Heidel.., xxviii, 30, 1900.
Vancea. Cluj. med., x, 462, 1929.
Villard. An. d’Oc., czlvii, 321, 1912.
Weigelin. A. f. Aug., lxi, 1, 1908.
Whaley. T. Nat. Conf. Pellagra, Columbia
S.C., i, 279, 1909.
Nettleship and Edmunds. T. O. S., i, 124, White. Am. Ott. Rhin. Lary., xxxvii, 128,
1881. 1928.
Nuel. A. d’O., xvi., 479, 1896. Wilmer. A. of O., iv, 817, 1930.
OPTIC NEURITIS IN CALVES
It is of interest here to note a peculiar condition occurring in calves, usually bulls
of Guernsey breed, which has received a considerable amount of attention (Nettleship
and Hudson, 1913; MacCallan and Mann, 1926; Crocker, 1919 ; Summers, 1927;
de Schweinitz, 1932; de Schweinitz and de Long, 1934). The animals may be born
blind, but visual failure usually becomes apparent towards the end of the first year
of life ; apart from the development of complete blindness with a widely dilated pupil,
other symptoms are lacking. Ophthalmoscopically a high degree of papillitis or
papilloedema is seen, sometimes associated with patches of choroiditis and retinal
haemorrhage and always resulting in optic atrophy. Pathologically both the papillae
and the brain are found to be occlematous ; there is a complete degeneration of the
retinal ganglion cells and their fibres with a neuroglial overgrowth, sometimes asso-
ciated with inflammatory foci. The aetiology is unknown. The disease has been
attributed to some toxin which has a special affinity for the ganglion cells; while on
the other hand the suggestion has been made that it might be a type of familial de-
generation due to in-breeding, comparable in some degree with Leber's disease.
Crocker. Cornell Vet., ix, 171, 1919.
MacCallan and Mann. T. O. S., xlvi, 229,
1926.
Nettleship and Hudson.
xix, 12, 1913.
de Schweinitz. A. of O., vii, l, 1932.
de Schweinitz and de Long. A. of O., xi, 194,
1934.
Summers.
R. L. O. H. Rep., T. O. S., xlvii, 221, 1927.
C. Toxic Amblyopia
The term toxic amblyopia is generally used to designate those conditions
wherein visual loss results from the absorption of eaſternal poisons. A similar
condition, as we have seen, may result from the effects of endogenously
DISEASES OF THE OPTIC NERVE 3009
elaborated toxins, as is exemplified in the retro-bulbar neuritis which occurs
in diabetes or pregnancy, or may even be said to occur from the effects of
bacterial toxins. The pathological dividing line between the two is thus
more artificial than real, but is maintained for its obvious clinical con-
venience. Again, it must be remembered that all these exogenous poisons
do not act in the same way ; some undoubtedly act primarily upon the
ganglion cells of the retina and should therefore be considered among
diseases of the retina, but, again, for reasons of convenience, we shall
consider them all together.
As a group, these poisons have certain features in common. They attack
the sub-chiasmal portion of the visual pathway only, the ganglion cells or
the nerve fibres, so that hemianopic or quadrantic defects do not occur in
the visual fields : nerve-bundle defects are also absent. Moreover, their
effects are always bilateral, and although in some cases recovery does not
ensue (wood-alcohol, organic compounds of arsenic), on the whole the visual
defect tends not to be permanent. From the clinical point of view they may
be divided into two groups. The more common group has a selective
affinity for the papillo-macular bundle and causes a central or centro-caecal
scotoma—tobacco, methyl, alcohol, lead, carbon disulphide, inorganic
arsenic compounds, optochin, etc.; with many members of this group a
peripheral neuritis also may occur. In the second group the visual defect
takes the form of a peripheral contraction advancing to a general depression,
while peripheral neuritis is rare—quinine, salicylic acid, filix mas, toxic
organic compounds of arsenic. Why different substances act in different
ways is unknown, a circumstance rendered more intriguing by the opposite
mode of action of close relatives such as quinine and optochin or the organic
and inorganic arsenical preparations. Further, how most of these substances
act is still obscure, and as we shall see, it is still debated whether all of them
are essentially neurotoxic or some of them act primarily upon the blood-
vessels and cause a secondary neuronic degeneration owing to vaso-con-
striction. The subject, indeed, is one about which little fundamental is
known.
A. Poisons Producing a Central Visual Defect
1. TOBACCO
TOBACCO AMBLYOPIA—or as the condition is sometimes referred to,
TOBACCO-ALCOHOL AMBLYOPIA, because of the usual adjuvant effect of the
latter poison—is the most common type of toxic amblyopia met with in
England. It constitutes a distinct clinical entity characterized essentially
by the bilateral impairment of central vision with the development of a centro-
cacal scotoma initially for red and green with an intact peripheral field without
3 N 2
3010 TEXT-BOOK OF OPHTHALMOLOGY
ophthalmoscopic changes; it is relatively common, accounting for 0.5 to 1.0%
of all ophthalmic patients attending European clinics.
The condition was originally described by Beer (1817), whose observation, how-
ever, was overlooked ; interest in the condition was resuscitated by Mackenzie (1854),
whose conclusions were confirmed by Hutchinson (1863–76), Wordsworth (1863),
v. Graefe (1865), Sichel (1865), and others, and today the literature on the subject
is enormous. The earliest authoritative collection of cases was the symposium staged
by the Ophthalmological Society of the United Kingdom in 1887 (Nettleship, Eales,
Hutchinson, Gunn, Morton, Frost, Griffith, Berry and Shears). Čle Schweinitz (1896)
in his treatise collected references to nearly 200 articles ; Uhthoff (1901) gathered
327 cases; Usher and Elderton (1927) reported a detailed study of 1,100 cases, and
Traguair (1930) of 1,525 cases.
AEtiology. Although the use of tobacco is frequently associated with
alcohol so that the two poisons probably act in conjunction, there is no
doubt that amblyopia can result from tobacco alone uncomplicated by
alcoholism (Shears, 1887; Connor, 1890; and others). Thus Uhthoff (1901)
in 327 cases of toxic amblyopia found 41 of pure tobacco, the remaining 286
being associated with both tobacco and alcohol, while Sattler (1923) in 56
cases of intoxication amblyopia found in 16 alcohol to be the cause, in 22
tobacco, and in 18 both. Traquair (1930) found little correlation between
alcohol and amblyopia, and Usher and Elderton (1927) reported that 112
out of their 1,100 cases were total abstainers, and that two-thirds of the
total were extremely abstemious drinkers. The tobacco is usually smoked
but similar symptoms may follow chewing or snuff-taking, or even in
those who work in tobacco factories although they have never smoked
(de Schweinitz, 1896). The amount of tobacco necessary to cause toxic
symptoms varies considerably with the individual, for many are susceptible
while others seem to be quite immune, smoking enormous quantities with
impunity; but in most cases of amblyopia the consumption over a period
of many years has been considerable. Nettleship (1887) found that the
shortest time for the development of symptoms after the commencement
of smoking was one year. Affected after middle age the patients have usually
been heavy smokers since early youth : in their large series both Usher and
lderton (1927) and Traquair (1930) found an average weekly consumption
of 3 oz. with extremes of 4 oz. and 9 oz. It is thus obvious that cases of
idiosyncrasy exist, a fact substantiated elsewhere than in the eye by the
development of a positive skin reaction to nicotine in a proportion of people
(Sulzberger, 1934), or the occurrence of abnormal vaso-pressor reactions in
susceptible persons after a few puffs of a cigarette (Cusick and Herrell, 1939).
There is some evidence, indeed, that this idiosyncrasy may be hereditary
(Nettleship, 1887). The fact, moreover, that recovery or improvement in
vision may occur after the development of symptoms despite the con-
tinuance of consumption, points to the possibility of the establishment of
tolerance. Apart from this individual variation, the incidence of amblyopia
DISEASES OF THE OPTIC NERVE 30 II
also varies with the kind of tobacco employed. The most frequent method of
Smoking is pipe-smoking, especially of strong tobacco , cigars and cigarettes,
especially the latter, are rarely the cause, and in those countries of the Near
East where smoking is extensive and almost universal among women and
men, and averages some 60 to 100 cigarettes a day, but is confined to
cigarettes of mild and pure tobacco, amblyopia is unknown (van Milligen,
1888; Meyerhof, 1921). What precisely the noxa is which causes the
symptoms is unknown ; it has never been conclusively shown to be nicotine,
and other poisonous substances more highly volatile may well be responsible,
such as pyridine and its derivatives, picoline, lutidine and collidine.
While some poison associated with tobacco is the true cause of tobacco
amblyopia, frequently combined with alcohol, there is no doubt that in
a large majority of cases the disease is determined by a depression in the
patient’s general health. The onset of symptoms is almost invariably preceded
by a state of physical depression or under-nutrition, a general toxaemia, a
period of mental worry or over-work, or, less commonly, an acute illness.
Its usual onset at a period in life when arteriosclerotic degeneration begins
to be evident is also significant (Zentmayer, 1925; Evans, 1939, and
others). The most common story, therefore, is that for many years the
patient had been an excessive smoker and a considerable drinker without
ill-effects, but eventually, as a result of over-work, worry or illness, the
poisons to which he had hitherto been immune became effective and his
vision began to fail. .
That the general nutrition of the patient has an important bearing on the onset
of symptoms was recognized by Mackenzie (1854), and stressed by more recent writers
(Holth, 1927; Traquair, 1930–5; Letchworth, 1932; and many others), but in
recent years it has been suggested that one of the most potent determining causes is
a specific nutritional deficiency, usually of a sub-clinical degree, associated particularly
with lack of vitamin B, a suggestion correlating tobacco amblyopia with the retro-
bulbar neuritis occurring in pellagra and states of avitaminosis and in the poly-
neuritis of chronic alcoholism (Shattuck, 1928; Minot, 1929; Keefer, 1931 ; Carroll,
1935–7).
It is interesting that a form of blindness occurs in horses in Australia due to eating
the Australian tobacco plant (Nicotiana suaveoleus) (Husemann, 1894; Barrett, 1897) :
the deer in Virginia, however, eat tobacco leaves with impunity, having apparently
developed a tolerance to the drug (de Schweinitz, 1896).
The incidence of the disease, as would be expected from its aetiology, is
much higher in men than in women : women, however, are not immune
(27 out of 1,100 cases, Usher and Elderton, 1927; 7 out of 1,525 cases,
Traguair, 1930). The average age is about 55 years : it is rare below 30
and above 70, but Traquair (1930) found extremes of 23 and 84.
Clinical Picture. The clinical picture is very characteristic. The
patient, usually a man of a little over 50, complains that for some weeks his
sight has gradually and without apparent reason failed, so that he now
finds difficulty in reading or doing fine work : he sees a mist before his eyes
3012 TEXT-BOOK OF OPHTHALMOLOGY
hanging in front of everything he looks at, which is less obvious in the dusk
than in bright day-light. His colour values, particularly for red and green,
may have changed, and he may, for example, insist in all seriousness that his
wife, despite her best endeavours, has lost her complexion and looks like a
corpse. His breath usually smells strongly of stale tobacco, there may be a
slight tremor of the hands, and questioning will usually reveal a recent
illness, a period of worry and anxiety, or a vague admission of deterioration
in the general well-being and activity. Objective examination reveals little—
a normal fundus, with perhaps some slight pallor of the temporal side of the
disc, while in advanced cases, according to Vogt (1921), the normal fibre-
streaking of the papillo-macular bundle as seen by red-free light is replaced
by an irregular mottling. Subjective examination elicits a diminution of
visual acuity and a characteristic scotoma. Although the two eyes are
usually affected unequally, the visual involvement is invariably bilateral at
the stage when patients present themselves for examination : for even in
those cases where the central vision is still normal, some slight defect in the
better eye can be elicited by careful scotometry. The case reported by
Hutchinson (1887) wherein, apart from a colour defect, the onset of visual
symptoms in the second eye was delayed for 6 months after marked
deterioration in the first, is exceptional ; but at all times perfect symmetry
of the defect in both eyes is rare. It is not common for the central vision
to fall below 6/60, but it is never reduced so that hand movements cannot be
perceived, and only in the most severe cases of long standing does any con-
siderable degree of atrophy result (Nettleship, 1887; de Schweinitz, 1896).
IBlindness, however, never occurs.
The scotoma has definite and characteristic features, showing variations
only within very narrow limits (v. Graefe, 1865; Leber, 1869 ; Förster,
1871; Bjerrum, 1890; Sattler, 1923; Traquair, 1930) (Figs. 2592–93). It
involves vision for white and colours with a considerable disproportion,
the defect for red being especially greater than that for white, and it is
essentially centro-caecal in position with diffuse margins not easily defined.
It commences to the nasal side of the blind-spot, and as the disease develops
it gradually extends towards the fixation point, which it eventually engulfs,
thus impairing central vision. In the milder cases all that may be observed
is a relative deficiency for colours in the area between the blind-spot and
the fixation spot, but as the disease progresses the appreciation of white
becomes defective. The macular fibres are not the most severely involved,
for within the affected area are one or more nuclei of intense defect, situated
on or near the horizontal meridian. Typically these are two in number, one
near the blind-spot and one near the fixation spot. As development
proceeds these nuclei fuse into a central core, the macula becomes more
deeply involved and central vision fails. While the failure for white objects
remains typically localized to a relatively small area near the centre of the
field, that for colour may become much more widespread, indicating a
DISEASES OF THE OPTIC NERVE 3013
conduction defect of moderate intensity. This deficiency is especially
marked for red, perception for which is lost first in the upper and outer
quadrant, then in the lower and outer quadrant so that a somewhat irregular
temporal hemianopia may ensue, and finally all perception for this colour
may be lost. When improvement occurs the colour fields return in the
reverse order, the scotoma becomes smaller and may disappear, although
in the more severe cases some permanent traces may remain as a projection
On the nasal side of the blind-spot. When there has been an extensive dense
scotoma for white with severe impairment of the fields for colours, although
7 5. 9 O
e9
º
--is:
ſº
//º
ſitiº
º
& ÇTºxº
&
& , ºf
* †
FIG. 2592.--TOBACCO AMBLYOPIA : FIG. 2593.−TOBACCO AMBLYOPIA :
EARLY STAGE. LATE STAGE.
Centro-caecal scotoma for 1/2000 Centro-cacal scotoma involving the
white not involving the fixation point; fixation point and with a dense
the dotted line is the field for red nucleus : 10, 2/ 2000, The dotted line
5/2000 which still survives at the ls the field for red 5| 2000 which occu-
fixation spot. pies a small area in the nasal peri-
phery only.
the colour fields enlarge and the defect for white may shrink considerably,
recovery is always very prolonged and usually imperfect.
Weekers (1932) suggested as a diagnostic sign the production of a positive scotoma,
which he claimed to be characteristic of this type of amblyopia. The patient has his
eyes covered by the handſ for some time while they remain open, and then, the hand
being rapidly withdrawn, he fixes a sheet of white paper intently on which a dark
central patch is seen.
Course and Prognosis. If, despite the advance of symptoms, indulgence
in tobacco is continued, the visual failure may increase until eventually some
degree of permanent atrophy may develop : no patient, however, becomes
blind, although the visual defect may remain stationary for many years
(Lawford, 1883). At other times deterioration ceases, or a tolerance may be
acquired so that some improvement occurs. When, however, the patient
ceases tobacco or in many cases cuts it down, the vision invariably improves.
In the milder cases this may take some 6 weeks or so to become manifest,











3014 TEXT-BOOK OF OPHTHALMOLOGY
and in the more severe cases any signs of amelioration may be delayed for
3 months, while not uncommonly an increase of symptoms occurs during
the first weeks of abstention, suggesting a possible cumulative effect, an
occurrence which may give rise to unjustifiable apprehension both to the patient
and the physician as to the correctness of the diagnosis. On the other hand,
if the onset of improvement is delayed for longer than 3 months, it may be
assumed that smoking is being continued or that the diagnosis is wrong.
As a general rule improvement is extremely slow, so that several years may
elapse before the final state is reached : thus in 170 cases Griffith (1887)
found an average time of 17 months, with extremes of 3 and 42 months;
but on the whole the prognosis is good, for serious incapacity is rare and
limited almost entirely to the aged or the infirm.
Thus Brauchli (1889) found in 32 cases in which vision had fallen to the counting
of fingers at 1 to 2 metres, that in 4 recovery occurred to the normal standard, in 5
from 2/3 to 1/3, in 2 from 1/10 to 1/20, and only in 9 cases did it remain below 1/20.
In his cases Griffith (1887) found 42% ultimately completely recovered, 37% partially
recovered, 17% stationary over periods varying from 6 to 48 months, while 4% got
WOI’Se.
It is curious that if a cure is once attained and the habit subsequently
resumed, recurrences of amblyopia are rare, even although smoking is
persisted in ; thus Griffith (1887) saw no recurrence in 170 cases, and Shears
(1887) 1 in 52. It is possible in these circumstances that the fear of blindness
has inspired habits of greater moderation, but it seems probable that a loss
of the idiosyncrasy and the acquirement of some degree of tolerance is the
rule. It seems necessary to admit in common sense, however, that a person
to whom a pleasant but unnecessary poison has done so much damage,
should not lightly resume indulgence in it.
Differential Diagnosis. A bilateral centro-caecal scotoma of a diffuse
nature with ill-defined margins and one or more dense nuclei near the
horizontal meridian, with a greater defect for red than for white, occurring
in an adult, provides a picture so constant as to justify the diagnosis of
tobacco amblyopia even in females who hesitate to admit addiction to the
drug. Alternative diagnoses are confined to other forms of amblyopia with
bilateral symmetrical centro-caecal scotomata. In retro-bulbar neuritis the
defect for blue is practically co-extensive with that for red, whereas in
tobacco poisoning it is small and confined to the middle of the scotomatous
area. Those conditions causing slowly increasing chiasmal pressure, such as
a pituitary tumour, sometimes give rise to difficulties, but here a temporal
hemianopia for white is usually more definite than the depression of central
vision, and the defects are always more sharply demarcated.
Pathology. Although a number of pathological examinations have been
done in cases of tobacco-alcohol amblyopia the pathology of the condition
is still obscure (Samelsohn, 1882; Sachs, 1893; de Schweinitz, 1897;
DISEASES OF THE OPTIC NERVE 30.15
Nuel, 1900; Sourdille, 1900–01; Uhthoff, 1901; Schieck, 1902; Birch-
Hirschfeld, 1902; Dalén, 1906; Rönne, 1910; Behr, 1935; and others).
The essential changes are in the papillo-macular bundle (Figs. 2594–97).
In the retina there is degeneration of the ganglion cells and atrophy of the
nerve fibres, changes which are not limited exclusively to the macular
region (Nuel, 1900; Birch-Hirschfeld, 1902; and others). In the nerve the
Figs, 2594–97. THE OPTIC NERVE IN Tobacco AMBLyopia.
The optic nerve, showing isolated degeneration of the papillo-macular bundle
(Behr, A. f. O.).
-
Fig. 2594–Immediately behind the Fig. 2595.-In the middle of the orbit.
bulb.
Fig. 2596–At the optic canal. Fig. 2597.-The intra-cranial portion.
degeneration can sometimes be traced from the globe up through the
chiasma to the primary visual centres, the papillo-macular bundle being
demarcated along the entire way, but sometimes the changes are patchy,
being more marked near the globe or in the region of the optic foramen
(Samelsohn, 1882). Atrophy of the nerve fibres is prominent with glial
proliferation, and the most recent examinations point to the view that the
interstitial proliferative changes, which are minimal in amount, are not
inflammatory in nature but rather compensatory. In only a few cases have


3016 TEXT-BOOK OF OPHTHALMOLOGY
marked vascular changes been noted such as endovasculitis (Sourdille,
1900–01) or obliteration of the capillaries (Schieck, 1909). -
The pathogenesis of the condition has given rise to some controversy,
and several theories have been put forward based on the histological findings.
1. A primary inflammatory condition of interstitial neuritis in the nerve
trunk with a consecutive atrophy (Uhthoff, 1887–1901; de Schweinitz,
1897; Nuel, 1900; and most of the older authors). As has been stated,
however, recent cases (such as that of Dalén, 1906, which was examined a
few hours after death and in which symptoms had only lasted nine weeks),
and those subjected to modern histological methods (Birch-Hirschfeld,
1902; Rönne, 1910; Behr, 1935) show an absence of inflammatory changes.
2. A primary vascular condition : a spasmodic constriction of the small
vessels by nicotine causing a secondary degeneration of the nerve-fibres
(Parsons, 1901; de Rosa, 1927; Raverdino, 1928; Farnarier, 1928;
Scalinci, 1932). Parsons (1901) suggested that the macular region was
especially susceptible since its vascular supply was already sparse ; while
Sachs (1888–93) and Schieck (1902–09) laid stress on thrombotic or infective
changes in the veins. Experimentally it has been shown in animals that
large doses of nicotine cause an enormous rise of blood-pressure owing to
increased vascular tone which leads to a passive dilatation of the intra-
ocular vessels (Parsons, 1903; Duke-Elder, 1931); but in man, especially
in susceptible persons, there is evidence not only of a general vaso-constric-
tion, but of a local retinal constriction also. The transient veiling of the
central visual field and slight dizziness after the first few puffs of a cigarette
in such persons was ascribed by Farnarier (1928) to such a spasm, and its
effect on the circulation of the skin and extremities was demonstrated by
the observation of blanching and a lowering in temperature by Maddock and
Coller (1933), Wright (1933), and Barker (1933). A similar spasm of the
retinal arteries has been observed by Raverdino (1928) and Cusick and
Herrell (1939), who found that in persons susceptible to nicotine, small doses,
as in smoking cigarettes, resulted in a measurable reduction in the calibre
of the retinal arterioles accompanying the general mild vaso-pressor reaction.
It may be argued that such a spasm, if continually repeated, may lead to
permanent changes which eventually become clinically obvious as amblyopia,
while the marked therapeutic effect claimed for vaso-dilating drugs, which
we shall note presently, also adds an argument to the case. A relative
scotoma of this type and position, however, is quite unlike any other lesion
caused by vaso-constriction, while the similarity of the symptoms, pathology,
and course of tobacco amblyopia with that caused by alcohol, which is a
vaso-dilatator, and other poisons which have no vaso-constrictor effects,
seems to suggest a common pathogenesis.
3. Schanz (1920) advanced the theory that nicotine sensitized the
retina to light which thus damaged the ganglion cells: for this view there
is little evidence.
DISEASES OF THE OPTIC NERVE 3017
4. A primary neuronic poisoning. That the condition is due to a primary
poisoning of the neural elements—either of the nerve-fibres with a secondary
or simultaneous degeneration of the ganglion cells, or of the ganglion cells
with a secondary degeneration of the nerve-fibres—is suggested by the
available pathological studies, a suggestion supported by the proved selective
neuronic affinity of other poisons of similar action (Birch-Hirschfeld, 1902;
Dalén, 1906; Rönne, 1910; Traquair, 1930; and others).
How the facts of susceptibility and tolerance can be explained, or the sudden
breakdown of tolerance after many years of safe indulgence, is unknown. The onset
of damage may result from the development of a lack of resistance to the poison, or
alternatively a breakdown in the metabolic process which normally deals with the
toxic agent. What this process may be is quite unknown, but it is interesting that
Dixon and Lee (1912) found that the liver had the faculty of mitigating the vaso-
pressor effects of nicotine so that animals could to some extent be immunized
against it, while de Schweinitz and Edsall (1903) remarked on the constant presence
of urobilinuria in their patients.
Treatment. The essential treatment of tobacco amblyopia should
involve the cessation of the consumption of tobacco, to which in the usual
case should be added strict moderation in alcohol. If this is done con-
scientiously improvement invariably occurs, and in most cases an eventual
practical cure results ; subsequently tobacco in reasonable amount can be
resumed, a course, however, which is probably in most cases unwise.
Recovery, as we have noted, can occur without abstention, but it is usually
tedious and rarely complete (Morton, 1887; Griffith, 1887; and others);
thus Shears (1887) reported 57 cases, most of which recovered completely
although only 2 gave up smoking. The continuance of drinking does not
delay recovery to the same extent as continuance of smoking (Nettleship,
1887); indeed, some observers maintain that moderate drinking may be
beneficial (Gunn, 1887; Usher and Elderton, 1927), an observation which
points to the relative unimportance of alcohol, but is not universally
admitted.
In addition to abstention every endeavour should be made to relieve
or mitigate the important adjuvant factors : a careful review of the general
health and habits of the patient should be undertaken, focal sepsis should
be eliminated, arteriosclerosis should be combated, an adequate, regular
and vitamin-rich diet instituted, and worry and anxiety eliminated as far
as this is possible. General stimulatory and eliminative measures have been
widely advocated and are probably of some value, although any marked
shortening of the convalescence period when compared with the results of
simple abstention with reasonable care of the general health can rarely be
satisfactorily demonstrated—strychnine, potassium iodide, potassium
citrate, the drinking of very large quantities of alkaline waters, sweat-baths,
and so on. Römer (1922) suggested intravenous injection of 2 to 5 c.c.
lecithin emulsion, and Trombetta (1900) the administration of caffein.
30 18 TEXT-BOOK OF OPHTHALMoLOGY
Two specific measures have been advocated in recent years, which have
been claimed by their sponsors to cure the condition speedily without
abstinence from tobacco and alcohol, claims which may be correct in a
number of cases but are not apparently of universal application. The
first is the use of vaso-dilatory drugs, a method of therapy instituted by
Pfimlin (1930) using sodium nitrite, and employed by Duggan (1932–36),
Lazlo (1932), Shannon and McAndrews (1934), Cordes and Harrington
(1935) and others, while Bonnefon (1931), Orr (1936) and Duggan (1937)
reported favourably on intra-muscular injections of acetyl choline.
Pfimlin used nitroscleran (sodium nitrite as a 10% solution in sodium chloride 0.6%,
di-sodium phosphate 0.36% and di-potassium phosphate 0.2%) given twice weekly
intra-venously in doses of 20 to 40 mg. of the nitrite. Duggan (1932–35), who has used
the method most extensively as daily intra-venous injections of the nitrite, found in
the majority of his cases a rapid improvement despite the continuance of Smoking in
the relatively short period of under a month.
The second therapeutic measure which has been suggested is the
administration of vitamin B preparations, either as brewer's yeast (Carroll,
1937), thiamin chloride (Johnson, 1939), or crystalline vitamin B,
(Paternostro, 1938), a course which is probably most applicable to those
cases wherein chronic alcoholism has impaired digestion.
Barker. Proc. Staff Meetings Mayo Clinic,
viii, 284, 1933.
Barrett. Intercol. Med. J. Australia, ii, 172,
1897.
Beer. Lehr. v. d. Augenkrankheiten, ii, 1817.
Behr, A. f. O., cxxxiv, 227, 1935.
Berry. T. O. S., vii, 91, 1887.
Birch-Hirschfeld. A. f. O., liii, 79, 1902;
liv, 68, 1902.
Bjerrum. X Internat. Cong., Berlin, 66, 1890.
Bonnefon. Pract. med. franç., xii, 65, 1931.
Brauchli. Diss., Zürich, 1889.
Carroll. A. of O., xiv, 112, 421, 1935; xvi,
919, 1936; xviii, 948, 1937.
Connor. J. Am. Med. As., xiv, 217, 1890.
Cordes and Harrington. A. of O., xiii, 435,
1935.
Cusick and Herrell. A. of O., xxi, 111, 1939.
Dalén. Mitt. a. d. Augenkl. d. Carol. med.-
chºir. Inst., Stockholm, H.8, 1906.
Dixon and Lee. Qwart. J. Eacp. Phys., v, 373,
1912.
Duggan. A. of O., viii, 304, 1932; xiii, 1059,
1935; xvi, 380, 1936; xvii, 579, 1937.
Duke-Elder. P. R. S. (B), ciz, 19, 1931.
J. Phys., lxxi, 1, 1931.
Eales. T. O. S., vii, 54, 1887.
Evans, T. O. S., lix, 241, 1939.
Farnarier. Rev. d’Oto.-neuro-oft., vi,
1928.
Förster.
647,
K. M. Aug., ix, 337, 1871.
Frost. T. O. S., vii, 78, 1887.
v. Graefe. K. M. Aug., iii, 129, 1865.
Griffith. T. O. S., vii, 81, 1887.
Johnson.
Gunn. T. O. S., vii, 67, 1887.
Holth. Acta O., v, 195, 1927.
Husemann. Deut. med. W., xx. 819, 1894.
Hutchinson. R. L. O. H. Rep., iv, 235, 1863;
vii, 169, 1871; viii, 456, 1876.
Hutchinson, J. (Jr.). T. O. S., vii, 61, 1887.
A. of O., xxi, 602, 1939.
Keefer. New Eng. Med. J., cov, 1086, 1931.
Lawford. T. O. S., iii, 163, 1883.
Lazlo. K. M. Aug., lxxxix, 554, 1932.
Leber. A. f. O., xv (3), 26, 1869.
Letchworth. Clin. J., lxi, 137, 1932.
Mackenzie. Treatise, 4th ed., 1854.
Maddock and Coller. Am. of Surg., xcviii, 70,
1933.
Meyerhof. K. M. Aug., lxvi (1), 107, 1921.
van Milligen. T. O. S., viii, 240, 1888.
Minot. Am. Int. Med., iii, 216, 1929.
Morton. T. O. S., vii, 71, 1887.
Nettleship. T. O. S., vii, 36, 38, 1887.
Nuel. Z. f. Aug., iv, 250, 1900.
Orr. Brit. Med. J., ii, 69, 1936.
Parsons. O. Rev., xx, 189, 1901.
The Intra-ocular Circulation, London, 1903.
Paternostro. An. di Ott., lxvi, 891, 1938.
Pfimlin. K. M. Aug., lxxxv, 787, 1930.
Revº. Rev. d’Oto.-neuro-oft., vi, 653,
1928.
Römer. K. M. Aug., lxviii, 385, 1922.
Rönne. A. f. O., lxxvii, 1, 1910.
de Rosa. Sull' ambliopia tossica da tabacco,
Napoli, 1927.
Sachs. A. f. Aug., xviii, 21, 1888; xxvii, 154,
1893.
DISEASES OF THE OPTIC NERVE 3019
Samelsohn. A. f. O., xxviii (1), 1, 1882. Sichel. An. d’Oc., liii, 122, 1865.
Sattler. Z. f. Aug., li, 111, 1923. - Sourdille. La Clin. Opht., vi, 280, 1900.
K. M. Aug., lxx, 318, 433, 1923. A. of O., xxx, 191, 1901.
Scalinci. Boll. d’Oc., ix, 752, 1932. Sulzberger. J. Am. Med. As., cii, 11, 1934.
Schanz. Z. f. Aug., xliii, 73, 1920. Traguair. T. O. S., l, 351, 1930.
Schieck. A. f. O., liv, 458, 1902; lxxi, 466, Edin. Med. J., xlii, 153, 1935.
1909. Trombetta. Clin. moderna, vi, 377, 1900.
de Schweinitz. The Toxic Amblyopias, Phila., Uhthoff. A. f. O., xxxii (4), 95; xxxiii (1),
1896. 257, 1887.
T. Am. O. S., viii, 186, 1897; xi, 337, 1907. G.-S. Hb., II, xi (2), 25, 1901.
O. Rec., vi, 118, 1897. Usher and Elderton. An. Eugenics, ii, 245,
de Schweinitz and Edsall. T. A m. O. S., x, 1927.
41, 1903. Vogt. K. M. Aug., lxvi, 718, 1921.
Shannon and McAndrews. A. of O., xi, 757, Weekers. A. d’O., xlix, 485, 1932.
1934. Wordsworth. Lancet, ii, 95, 172, 1863.
Shattuck. Am. J. Trop. Med., viii, 539, 1928. Wright. J. Am. Med. As., ci, 439, 1933.
Shears. T. O. S., vii, 92, 1887. Zentmayer. Am. J. O., viii, 365, 1925.
2. ETHYL ALCOHOL
We have just seen in the preceding section that there is evidence
to show that in the majority of cases of toxic amblyopia, the two agents,
tobacco and alcohol, are jointly responsible. In Great Britain there
seems to be no doubt that the major cause is tobacco. Thus among
the earlier writers, Nettleship (1887), Eales (1887), Gunn (1887) and Berry
(1887) met with no case in their experience wherein amblyopia could be
ascribed to alcohol and smoking could be excluded. In 1922 the Committee
appointed by the Ministry of Health could find no authentic instance of a
purely alcoholic amblyopia, while among the later writers Usher and
Elderton (1927) and Traquair (1930–35), reviewing between them 2,625
cases, considered alcohol as incidental and relatively unimportant. There
is no doubt, however, that cases occur in heavy drinkers who are also non-
smokers (the amblyopia potatorum) (Hutchinson, 1887; Griffith, 1887;
Connor, 1890; de Schweinitz, 1896). On the Continent of Europe the
condition is more common (44% of cases of amblyopia, Uhthoff, 1886; 27%
Sattler, 1923).
The condition is always associated with a long history of alcoholism,
and frequently with peripheral neuritis. Here again, also, as in tobacco
amblyopia, a recent deterioration of the physical and mental health enters
largely into the aetiology, a matter in which mal-nutrition, owing to long-
standing alcoholic gastro-enteritis, usually plays a prominent part. So
much, indeed, has this aspect of the matter impressed some writers that
they consider the condition as a deficiency disease related to alcoholic
pellagra, and due essentially to lack of absorption vitamin B (Keefer, 1931 ;
Carroll, 1935–37; and others).
Little experimental work of value has been done on the question, largely owing
to difficulties in inducing a chronic poisoning in animals. de Schweinitz (1896) kept
a monkey constantly drunk for six months but found neither ophthalmoscopic nor
histological evidence of evil effects in the eyes. Rymowitsch (1896) and Friedenwald
3020 TEXT-BOOK OF OPHTHALMOLOGY
(1901), however, after chronically poisoning rabbits for one year, observed degenera-
tion of the ganglion cells, without connective tissue overgrowth or cellular infiltration,
or degeneration of the nerve-fibres as revealed by Marchi’s method of staining.
The clinical picture, symptoms and course of the disease are exactly
similar to the amblyopia of tobacco: there is the same central scotoma for
red and green in the early stages, the same centro-caecal scotoma for white
in the later, but the duration of the dis-
ability is usually longer and the prognosis
worse than in tobacco cases, partly because
some degree of optic atrophy with a temporal
pallor of the disc is more common, and
partly because the subjects are usually more
debilitated and less readily persuaded to
maintain abstinence. The pathology is also
similar, being characterized by a primary
neuronic degeneration affecting particularly
Fig. 25us. THE Orrio NERVE the papillºmacular neurons, and involving
"ºn... "... the ganglion cells and the nerve fibres
AMBLyopia. (Nettleship and Edmunds, 1881; Samelsohn,
showing papillºmºula de 1882; Edmunds and Lawford, 1887; Uhthoff,
generation (Juba, K. M. Aug.). - -
1901; Birch-Hirschfeld, 1902; Rönne, 1910;
Juba, 1935) (Fig. 2598). Atrophy is rarely complete, normal fibres being
present even in the most affected areas, while the connective-tissue frame-
work is considerably thickened and proliferated.
Morax (1935) recorded a case wherein a woman was given an intravenous injection
of 200 e.e. of 33% alcohol for puerperal septicæmia, who developed bilateral absolute
central scotomata with optic atrophy.
Treatment should be conducted on similar lines to that outlined in
dealing with tobacco, and should involve a cutting down or abstinence from
alcohol, a similar restriction being placed on smoking if it also is indulged in
a complete overhaul of the general health, and, above all, the institution
of a healthy and regular diet with an abundance of vitamin B, in the form
of yeast or otherwise (Carroll, 1935-37; Johnson, 1939). As in tobacco
amblyopia, Bonnefon (1931) reported good results from the injection of
vasodilatory drugs (intra-muscular injections of 0-1 gm acetylcholine).
Berry. T. O. S. vii. 91, 1887. Friedenwald. 0. Rec., ix, 428, 1901.
Birch-Hirschfeld. A. f. o. iii, 79, 1902; Griffith. T. O. S., vii. 81, 1887.
liv, 68, 1902. Gunn. T. O. S. vii. 67, 1887.
Bonnefon. Pract, med, franc., xii. 55, 1931. Hutchinson. T. O. S., vii. 61, 1887.
Carroll. A. of 0., xiv. 421, 1935; xviii. 948, Johnson. A. of 0., xxi. 602, 1939.
1937. Juba. K. M. Aug., xev, 148, 1935.
Connor. J. 4 m. Med. As wiv. 217, 1890. Keefer. New England Med. J., cov. 1086,
Eales. T. O. S., vii. 54, 1887. 1931.
Edmunds and Lawford. T. o. s. vii. 76, Morax. An d'Oe., clºxi, 30l., 1935.
1887. Nettleship. T. O. S. vii. 36, 1887.

DISEASES OF THE OPTIC NERVE 3021
Nettleship and Edmunds. T. O. S., i, 124, Traquair. T. O. S., l, 351, 1930.
1881. Edin. Med. J., xlii, 153, 1935.
Rönne. A. f. O., lxxvii, 1, 1910. Uhthoff. A. f. O., xxxii (4), 95, 1886; xxxiii
Rymowitsch. Diss., Petersburg, 1896. (1), 257, 1887.
Samelsohn. A. f. O., xxviii (1), 1, 1882. G.-S. Hb., II, xi (2), 3, 1901,
Sattler. K. M. Aug., lxx, 318, 433, 1923. Usher and Elderton. An. Eugenics, ii, 245,
de Schweinitz. The Toxic Amblyopias, 1927.
Phila., 1896.
3. METHYL ALCOHOL
Whereas ethyl alcohol produces a chronic amblyopia after prolonged
ingestion, methyl alcohol produces typically an acute poisoning, charac-
terized usually by complete amaurosis and frequently resulting in optic
atrophy and permanent blindness. That the action is similar in type to
that of the tobacco-ethyl alcohol group of poisons, however, is seen in the
central scotoma which appears in the stage of retrogression in those cases
in which some degree of recovery ensues
(Fig. 2599).
Methyl, or wood alcohol, may be
ingested in one of three ways—through
the stomach, the lungs, or the skin. Most
commonly it is drunk in cheap adulterated
or fortified beverages. In this event a
very small quantity may cause immense
damage, especially when taken on an
empty stomach (Hirschberg, 1912), for a
teaspoonful may produce permanent
blindness and 1 oz. may be fatal (Ziegler,
1921). It may also be absorbed by the
inhalation of fumes in several commercial Fig. 2599–METHyd Alcohol.
processes, for the alcohol is widely used as AMBLYOPIA.
a solvent for shellacs and varnishes, as ...Yº...”.....”.” *
g poisoning : 3/330, white.
well as in the manufacture of china-
cement, rubber, and in other processes. This method of poisoning is
usually more chronic and insidious but is amply authenticated as occurring
in persons working in closed ill-ventilated spaces (de Schweinitz, 1896–1911 ;
Wood and Buller, 1904; Gruening, 1911; Tyson, 1912; Ziegler, 1921 ;
and others). Cutaneous absorption is rare, but cases of blindness have
been recorded, as following the prolonged daily use of liniments, bay rum
and toilet waters (Wood and Buller, 1904; Ziegler, 1921).
Wood alcohol poisoning was common in the United States as the result
of drinking illicit liquor. In the days before the Great War of 1914 it was a
frequent cause of blindness (Gruening, 1911): thus Casey Wood and Buller
(1904) were able to collect 153 cases of blindness and 122 of death. After
the War in the epoch of prohibition the incidence rose, and at that time

3022 TEXT-BOOK OF OPHTHALMOLOGY
Jackson (1920) considered that it caused more blindness than all other toxic
causes put together : Hubbard (1920) reported 52 cases in New York in
1918. In England it is relatively rare, but on the Continent it is more
common. In one Berlin institution for tramps 130 cases were admitted in
1911; and in the first 5 months of the War in 1914, Goldflam (1920)
collected 110 cases in Warsaw. Similarly in Denmark, Blegvad and Rönne
(1920) found that an amblyopia hitherto unknown in that country became
common during the War owing to the use of substitute liquor because of the
expensiveness of potable spirit.
Some of the instances of wood-alcohol poisoning have been indeed tragic, such as
the housewife seen by Ziegler (1921) who, after a drink of adulterated ginger-ale,
went completely blind, or the man who went similarly blind after a single drink of
fortified cider. A soldier was reported by Wilmer (1911) who went permanently blind
after drinking 4 oz. of wood-alcohol in mistake for whisky. Whole parties of people
have been affected in this way, some dying, others becoming permanently blind, and
others escaping with much impaired vision. Thus Mendel (1912) reported such a
tragic party from which 130 people were taken to hospital, of whom 51 died and many
became blind, developing complete optic atrophy.
The general symptoms of acute poisoning by wood-alcohol are headache,
dizziness, nausea, vomiting, abdominal pain, cardiac weakness, marked
prostration, delirium, convulsions, stupor, and finally death. In cases where
recovery occurs, blindness is usually noticed on the second or third day,
when the stupor wears off. In the worst cases the blindness may be early,
sudden, complete, and permanent. In less severe cases there may be marked
recovery at the end of 4 or 5 weeks, which itself may be lasting or may be
followed by gradual failure and ultimate blindness. In cases of more chronic
or insidious absorption, or where the poison is drunk in repeated small
quantities or absorbed by inhalation, headache and obscure nervous and
gastro-intestinal symptoms may appear associated with a gradual deteriora-
tion of vision and the development of a dense central or paracentral scotoma.
Objectively there may be papillitis with considerable swelling and dilatation
of the veins; alternatively a chalky white pallor of the disc may develop
rapidly ; or in the more insidious cases the symptoms may be those of
retro-bulbar neuritis followed by the appearance of atrophy. The atrophy
following this type of poisoning is usually profound, and the discs are
typically excavated, resembling a glaucomatous cup (Gruening, 1910;
Morrison, 1922; Shannon, 1932). The pupils are usually dilated and may
be inactive, and paresis of the extra-ocular muscles with diplopia and
ptosis may occur, while a certain degree of hypotony may exist.
A considerable amount of experimental work has been done on methyl
alcohol poisoning in animals (Holden, 1899; Birch-Hirschfeld, 1902; Hunt,
1902; Igersheimer and Verzar, 1913; Tyson and Schoenberg, 1914; and
others), but pathological examinations in human subjects have been rela-
tively few (Eleonskaja, 1925; MacDonald, 1929). The general pathological
DISEASES OF THE OPTIC NERVE 3023
picture is one of profound and widespread degeneration of the ganglion
cells of the retina (Figs. 2600–01), with degeneration of the nerve fibres
in the optic nerve, which in severe and chronic cases may extend up to the
external geniculate body (Eleonskaja, 1925). Some controversy has arisen
as to whether the changes in the retina or the nerve are primary (Uhthoff,
1886), but the view of Holden (1899), Birch-Hirschfeld (1902), and Tyson
and Schoenberg (1914) that the injury to the ganglion cells is primary and
that the degeneration in the nerve is an ascending secondary phenomenon
seems substantiated by the findings of MacDonald in the earliest human
-- º -
-
-
-- -
-
- - º - -
º -
- _ - -
- - - º-
- Lº º:
| - -
- º
Fig. 2600.--THE RETINA IN Acute Fig. 2601.--THE RETINA IN Acure
METHYL Alcohol. Poison INg. METHYL Alcohol, Poison INg.
Autopsy, 6 hours after death, showing The same case as 2600 showing swollen
marked changes about the vessel and ganglion cells and globules under the int.
degenerative débris under the internal limit. limiting membrane. (Haematoxylin and
ing membrane. (Mallory's phospho-tungstic eosin) (x 400) (MacDonald).
acid haematoxylin) (x 100) (MacDonald).
case examined pathologically (who died on the third day and was examined
six hours after death), wherein the degeneration in the retina was marked
and no pathological change could be observed in the optic nerve.
The rationale of the poisoning is still obscure. It is known that whereas
ethyl alcohol is rapidly and completely oxidized in the body, methyl alcohol
is much less readily dealt with, remaining many days, so that a cumulative
effect is readily possible (Völtz, 1912). Tyson and Sohoenberg (1914) found
that there was a general acidosis due to this imperfect oxidation of the
methyl alcohol, resulting in the formation of formic acid in the blood-stream,
with an increase in the acidity of the aqueous humour. It has been shown
also by Goldschmidt (1922) and Oguchi (1932) that as a result of this the
T.O.-W.O.L. 111. 3 o

3024 TEXT-BOOK OF OPHTHALMOLOGY
Oxidative processes in the retina are much impaired and its respiratory
activity lowered, so that the metabolism may be reduced by 40% to 50%.
The former investigator also concluded that the damage was greater when
the retina was exposed to light, a view put forward also by Schanz (1920),
and Schieck (1922), but not universally accepted (Schwarzkopf, 1922).
Treatment. In severe cases of poisoning the rapidity of the toxic action
makes treatment of little avail. Gastric lavage should be carried out
immediately and at recurrent intervals during the first few days, since there
is evidence that the alcohol in the system is continuously returned to the
stomach ; more, indeed, may be recovered in this way in the second and
third day after ingestion than during the first (Ziegler, 1921). Eliminative
treatment by diaphoresis, hot-packs, pilocarpine, hot drinks and Saline purga-
tives is also indicated to wash as much poison out of the system as possible.
Acidosis should be overcome by the early and massive administration of
alkalis, sodium bicarbonate being given by mouth or intravenously (500 c.c.
of 5% solution : Ziegler, 1921). Ziegler (1921) also recommended negative
galvanism with a high voltage (60 volts) and a low average (1 milliampere)
to maintain the function in the optic nerve ; while good results have been
claimed from repeated lumbar punctures (Pincus, 1920; Zethelius and Wersen,
1920). In view of the possible intensification of the retinal damage by light,
Schieck (1922) stressed the importance of keeping the patient in the dark.
Birch-Hirschfeld. A. f. O., lii, 358, 1901; Oguchi. Acta S. O. Jap., xxxvi, 155, 1932.
liv, 68, 1902. Pincus. K. M. Aug., lxv, 695, 1920.
Z. f. Aug., xxxv, 1, 1916. Schanz. Z. f. Aug., xliii, 73, 1920.
Blegvad and Rönne. K. M. Aug., lxv, 206, Schieck. A. f. O., liv, 458, 1902.
1920.
Eleonskaja. Russ. O. J., iv, 40, 1925.
Goldflam. K. M. Aug., lxiv, 684, 1920.
Goldschmidt. B. O. G. Heidel.., xliii, 129,
1922.
Gruening. A. of O., xxxix, 333, 1910.
T. Am. O. S., xii, 522, 1911.
Hirschberg. Berl. kl. W., xlix (1), 247, 1912.
Holden. A. of O., xxviii, 125, 1899.
Hubbard. N. Y. Med. J., czi, 16, 1920.
Hunt. Bull. Johns Hopkins Hosp., xiii, 213,
1902.
Igersheimer and Verzar. A. f. Aug., lxxv, 27.
1913.
Jackson. Am. J. O., iii, 150, 1920.
MacDonald. XIII Intermat. Cong., Amster-
dam, ii, 440, 1929.
Mendel. Cb. pr. Aug., 43, 1912.
Z. f. Aug., xlviii, 187, 1922.
Schwarzkopf. Z. f. Aug., xlviii, 317, 1922.
de Schweinitz. The Toaric Amblyopias,
Phila., 1896.
T. Am. O. S., xii, 523, 1911.
Shannon. A. of O., vii, 813, 1932.
Tyson. T. Am. O. S., xiii, 146, 1912.
Tyson and Schoenberg. J. Am. Med. As.,
lxiii, 915, 1914.
Uhthoff. A. f. O., xxxii (4), 95,
xxxiii (1), 257, 1887.
K. M. Aug., liv, 48, 1915.
Völtz. Med. Kl., viii, 697, 1912.
Wilmer. T. Am. O. S., xii, 523, 1911.
Wood (Casey) and Buller. J. Am. Med. As.,
xliii, 972, 1058, 1117, 1213, 1289, 1904.
Zethelius and Wersen. K. M. Aug., lxv, 51,
1920.
1886
Morrison. Am. J. O., v, 645, 1922. Ziegler. Brit. J. O., v, 365, 411, 1921.
4. CARBON DISULPHIDE
Carbon disulphide, as used in Vulcanizing rubber, was not an uncommon
cause of poisoning until its toxic properties on inhalation became generally
recognized. The first to draw adequate attention to the matter was Delpech
(1856) in Paris, and a Committee appointed by the Ophthalmological
Society (Frost, Gunn and Nettleship) in 1885 gathered 24 cases from the
DISEASES OF THE OPTIO' NERVE 3025
literature. In modern times, however, since ventilation in the factories
wherein it is used has been adequately controlled, poisoning in this way has
disappeared.
The general symptoms are always striking. A frontal or temporal
headache may precede any other evidences of absorption by months or years.
More definite symptoms of poisoning are ushered in by a period of exaltation
of spirits when temporary disturbances of vision and hearing are common,
manifestations which are followed by a stage of depression and general
weakness or collapse when visual symp-
toms as well as affections of hearing, taste,
and smell, are more common. The typical
visual symptom is a bilateral central
scotoma, which, as in other conduction
effects, is earliest and greatest for red and
green, particularly the former (Fuchs,
1885), to which colour there may be
complete blindness (Fig. 2602). The
central scotoma, which differs from that
due to tobacco in being peri-central in
position, may be small or very extensive,
and may be associated with a contraction
and general depression of the field : ". -º-ºpertº
occasionally the contraction of the field The field for red (Fuchs).
occurs alone (Little, 1887). There is no
constant ophthalmoscopical picture; sometimes the fundus is normal, but
slight papillitis or atrophy has usually been described.
Experimental observations in animals have shown degeneration of the
cells of the central nervous system, but no changes in the ganglion cells of the
retina (Koester, 1899; Birch-Hirschfeld, 1900).
The symptoms progress as long as the worker is exposed to the fumes
of carbon disulphide, but on his being removed from them, the prognosis
is usually good, the vision slowly improving in all but some 20% of the more
severe cases (de Schweinitz, 1896): re-exposure to the fumes, however,
inevitably brings about a relapse. Blindness is very rare, although a
considerable amblyopia with partial optic atrophy may persist.
Birch-Hirschfeld. A. f. O., 1, 166, 1900. Gunn. T. O. S., v., 165, 1885.
Delpech. Memoire sur les accidents que Koester. A. f. Psy... xxxii. 569, 1899.
developpe dans les ouvriers en ('aoutchouc, Little. T. O. S., vii, 73, 1887.
Paris, 1856. Nettleship. T. O. S., v., 149, 157, 1885.
Frost. T. O. S., v., 165, 1885. de Schweinitz, The Toric Amblyopias,
Fuchs. T. O. S., v. 152, 1885. Phila., 1896.
5. IODOFORM : IODINE
Iodoform, if absorbed in large amounts, may give rise to complete
amaurosis (Valude, 1893), or more usually to an amblyopia characterized

3 O 2
3026 TEXT-BOOK OF OPHTHALMOLOGY
by a loss of central vision with peripheral defects and without ophthalmo-
scopic changes. The drug may be administered by mouth (Hutchinson,
1886; Priestley Smith, 1893, 1,000 grains in 41 days), or as an ointment
for tuberculous abscesses or a dressing for carcinoma (Valude, 1893;
Critchett, 1898; Mohr, 1902). The scotoma is similar to that for carbon
disulphide ; but with stoppage of the drug the symptoms clear up in a period
varying from some days to some months.
It is interesting that iodine in the form of PREGLE’s SOLUTION, when injected
intravenously in septic conditions, may bring about a sudden amaurosis which is
followed by the development of a central scotoma as well as a peripheral contraction
of the visual field. Ophthalmoscopically there is a yellowish-brown colouring of the
fundus with a general oedema and narrowing of the vessels, which is followed after
Some time by a degenerative picture resembling primary pigmentary degeneration, an
appearance which can be produced in rabbits (Scheerer, 1926; v. Bünau, 1929 ;
Riehm, 1929). Pathologically an acute necrosis of the neuro-epithelium and the
pigmentary epithelium has been found in rabbits (Riehm, 1929). As treatment
Roggenkämper (1927) found an intra-venous injection of 10% lecithin emulsion
effective.
v. Bünau. K. M. Aug., lxxxiii, 345, 1929. Roggenkämper. K. M. Aug., lxxix, 827,
Critchett. T. O. S., xviii, 383, 1898. I927.
Hutchinson. N. Y. Med. J., xliii, 16, 1886. Scheerer. K. M. Aug., lxxvi, 524, 1926.
Mohr. A. f. Aug., xlv., 183, 1902. Smith, Priestley. O. Rev., xii, 101, 1893.
Riehm. A. f. Aug., c-ci, 872, 1929. Valude. An. d’Oc., cir, 378, 1893.
6. LEAD
It has been known for many years that lead may produce symptoms
of general poisoning and visual defects if it is introduced into the body either
by the mouth, through the skin, or inhaled as dust. Most cases arise through
industrial processes, but owing to greater efficiency in protective measures,
cases of poisoning are steadily becoming less frequent : thus in England
599 cases of lead poisoning, including 20 with optic neuritis, were reported
between 1905 and 1909, and only 279 with 6 cases of optic neuritis between
1915 and 1919 (Legge, 1922). Other less readily recognizable sources,
however, are common : thus an epidemic affecting 62 children was reported
by Gibson (1908) due to lead carbonate in the paint with which house railings
were painted, and I have seen a typical case in a boy whose hobby was the
making of lead soldiers. As a rule the visual symptoms of poisoning only
develop after exposure over long periods of time, but they may arise within
12 days of exposure (de Schweinitz, 1896), and complete immunity may be
maintained for many years. On the whole, ocular sysmptoms are rare,
and almost invariably they do not occur alone but are associated with other
general signs of plumbism, a blue line round the gums, headaches, colic,
and evidences of peripheral neuritis such as wrist drop. The diagnosis may
be clinched by the finding of lead in the urine.
DISEASES OF THE OPTIC NERVE 3027
The Ocular manifestations of lead poisoning are various, depending on
changes in the central nervous system, changes in the neural elements of the
Optic nerve and retina, changes in the blood-vessels, and the indirect effects
of a lead nephritis.
(a) A sudden bilateral amaurosis of central origin and without changes
in the fundus may occur : it may be complete and is usually transient, perfect
recovery occurring in a few days (Loewe, 1906; Galezowski, 1906).
Homonymous hemianopia may also occur, but this is rarer (Westphal, 1888;
Hertel, 1890; Bihler, 1900; and others); while an increase of intra-cranial
pressure due to a saturnine encephalitis may give rise to a papilloedema
which may be followed by progressive atrophy leading to depression and
contraction of the field terminating sometimes in blindness.
(b) A towic optic neuritis, usually of gradual onset, but sometimes sudden
and simultaneously bilateral (Lillie, 1934), may appear, to be followed by
some degree of atrophy, which, in some 10% of cases (Uhthoff, 1887), is
complete resulting in permanent blindness. The lesion may be retro-bulbar
in type without obvious fundus change analogous to that associated with
other toxic agents, the disc may be hyperaemic and ill-defined, or a definite
papillitis with haemorrhages and exudates may be evident (Hutchinson,
1871). In this type, which is the most common ocular expression of the
poisoning, the typical scotoma is bilateral, pericentral, and roughly circular,
and the peripheral field is relatively unaffected. Of 34 cases examined by
Stood (1884), 17 had had optic neuritis or atrophy, and of 47 gathered by
de Schweinitz (1896) in which ophthalmoscopic examinations had been
made, 34 were thus affected.
(c) Various types of retinitis and vascular changes may occur. An
extreme degree of spasm of the retinal arteries was noted by Elschnig (1898),
and all types of arteritis and peri-arteritis with retinal haemorrhages have
been described, some of which are primarily retinal and some secondary to
nephritic changes.
Other ocular complications include paresis of the external muscles, particularly
of the external rectus (Snell, 1904; and others), or even a complete external ophthalmo-
plegia ; nystagmus may also occur. Pupillary paresis may also appear (27 in 310 cases,
Neiding and Feldmann, 1925; 21 in 320, Blatt, 1931), or accommodative failure
(2 cases in 320, Blatt, 1931).
Anatomical examination shows that the lesion in the optic nerve is of
the inflammatory rather than the purely degenerative type with interstitial
proliferation and thickening of the sheaths (Brailey, 1876; Pflüger, 1883;
Parisotti and Melotti, 1885; and others). Vascular changes are also
common among which sclerosis, obliterative endarteritis and hyaline
degeneration are common. It is difficult to determine how much of the total
effect is primarily a neuronic poisoning and how much is secondary to vascular
mischief, but probably both factors are usually operative.
3028 TEXT-BOOK OF OPHTHALMOLOGY
Treatment should consist in the immediate elimination of lead from the
sufferer's environment, and an attempt to remove the poison from the
system as thoroughly and rapidly as possible. Potassium iodide and sulphur
baths are widely recommended but the lead may probably be more efficiently
precipitated by the administration of calcium lactate and parathormone.
Bihler. A. f. Aug., xl, 274, 1900. Loewe. A. of O., xxxv, 164, 1906.
Blatt. K. M. Aug., lxxxvi, 482, 1931. Neiding and Feldmann. Deut. Z. f. Nervenkr.,
Brailey. R. L. O. H. Rep., viii, 549, 1876. lxxxiv, 297, 1925.
Elschnig. Wien. med. W., xlviii, 1305, 1411, Oeller. A. f. path. Anat., lxxxvi, 329, 1881.
1898. Parisotti and Melotti. Rec. d’O., 520, 1885.
Galezowski. Rec. d’O., xxvii, 592, 1905. Pflüger. Ber. de Berner Augenklinik, 1883.
A. gén. de méd., i, 1613, 1906. de Schweinitz. The Toacic Amblyopias,
Gibson. Brit. Med. J., ii, 1488, 1908. Phila., 1896.
Hertel. Charité Ann., xv, 220, 1890. Snell. T. O. S., xxiv, 184, 1904.
Hutchinson. R. L. O. H. Rep., vii, 6, 1871. Stood. A. f. O., xxx, 215, 1884.
Legge. T. O. S., xlii, 2, 1922. Uhthoff. A. f. O., xxxiii (1), 257, 1887.
Lillie. Am. J. O., xvii, 110, 1934. Westphal. A. f. Psy., xix, 620, 1888.
7. THALLIUM
Thallium forms a rarer source of poisoning, and is usually acquired after
the long use of cosmetic facial creams which contain thallium acetate owing
to its depilatory properties (Teleky, 1928; Girot and Braun, 1929; Lillie
and Parker, 1932; Mahoney, 1932; Stine, 1932; Rudolphy, 1935; and
Others). Such a chronic type of poisoning is usually ushered in by gastro-
intestinal symptoms, muscular weakness, and sometimes polyneuritis and
alopoecia. There is an absolute bilateral central scotoma, the development
of which may be followed by permanent grey atrophy. Cataract may occur
as a complication.
A case of acute poisoning was reported by Kaps (1927) which occurred after
eating rat poison containing thallium acetate : symptoms of an acute retro-bulbar
neuritis were followed by alopoecia, peripheral neuritis, dementia and death. A partial
Optic atrophy was produced experimentally in animals by Buschke, Löwenstein and
Joel (1928).
Buschke, Löwenstein and Joel. Kl. W., vii, Lillie and Parker. J. Am. Med. As., xcviii,
1515, 1928. 1347, 1932.
Girot and Braun. Rev. neurol.., xxxvi (1), Mahoney. J. Am. Med. As., xcviii, 618, 1932.
244, 1929. Rudolphy. A. of O., xiii, 1108, 1935.
Raps. Wien. kl. W., x1, 967, 1927. Stine. Am. J. O., xv. 949, 1932.
Teleky. Wien. med. W., i, 506, 1928.
8. INORGANIC ARSENIC
The inorganic forms of arsenic rarely cause amblyopic symptoms, and
cases in the literature are few. Thus in a mass-poisoning due to drinking
arsenic-contaminated beer in Manchester which affected 1,000 people, no
single case of amblyopia or disturbance of the retina or optic nerve occurred
(Reynolds, 1900). Cases, however, have occurred which have showed a
varying degree of neuro-retinitis and are characterized by the development
DISEASES OF THE OPTIC NERVE 30.29
of bilateral central scotomata (de Haas, 1919), while Igersheimer (1909)
produced degenerative changes in the ganglion cells and inner nuclear layer
as well as in the nerve and chiasma in experiments upon animals.
de Haas. A. f. O., xcix, 16, 1919. Reynolds. Brit. Med. J., ii, 1492, 1900.
Igersheimer. A. f. O., lxxi, 379, 1909.
Z. phys. Chem., lix, 256, 1909.
THY ROID given in excessive doses over long periods has been associated with a
reduction of visual acuity with the production of a central Scotoma, an association
rendered suggestive by the finding of degeneration in the ganglion cells and optic
nerves in experimental dogs by Birch-Hirschfeld and Inouye (1905). -
A temporary diminution of vision associated with a centro-caecal scotoma has
been noted by Sattler (1923) to follow the administration of BARBITURATEs as ADALIN
(bromo-diethyl-acetyl-urea) (8–10 gm.) and BROMURAL (monobrom-isovalarianyl-
urea) (10–20 gm.).
APIOL, a drug used as an abortifacient and emmenagogue, has been noted to
produce a polyneuritis with an optic neutritis involving a bilateral central scotoma
(Juhasz-Schäffer, 1932; Brain, 1934). Recovery appears to be the rule.
SULPHANILAMIDE has been reported as producing an optic neuritis (Bucy, 1937).
Birch-Hirschfeld and Inouye. A. f. O., lxi, Bucy. J. Am. Med. As., cix, 1007, 1937.
499, 1905. Juhasz-Schäffer. Kl. W., xi, 1232, 1932.
Brain. T. O. S., liv, 221, 1934. Sattler. Z. f. Aug., li, 113, 1923.
B. Poisons producing a peripheral Contraction of the Field
1. ORGANIC ARSENIC PREPARATIONS
In contrast to the inorganic preparations of arsenic which rarely give
rise to visual defects and produce a central scotoma when this does occur,
the organic preparations manifest a high toxicity for the visual elements,
producing on occasion grave visual disabilities with a peripheral contraction
of the fields, widespread degeneration of the retina and optic nerve, and
involving no tendency to recovery so that the prognosis is thoroughly bad.
Of these preparations the tri-valent compounds are relatively non-toxic but
the penta-valent are markedly so. In an extensive investigation on rabbits
Young and Loevenhart (1924) found that the toxicity of these compounds
for the optic tracts was greatest when an amino group or a substituted amino
group occupied the para-position in the molecule, and the evidence favours
the same rule being applicable to man.
Among the first compounds which demonstrated this disastrous visual
effect were atoacyl and Soamin which were given for trypanosomiasis and
syphilis in considerable quantities and rapidly produced a large number of
cases of permanent blindness (Fehr, 1907; Coppez, 1909; Clarke, 1910 ;
Dawnay, 1910; Wray, 1910; and others). For this reason the use of these
drugs has been abandoned. Since then several other preparations have
produced similar results although in less numbers, even the relatively
non-toxic acetylarsam (Tissot-Daquette, 1934) or tryparsamide (Lazar, 1934 ;
303() TEXT-BOOK OF OPHTHALMOLOGY
Veil, 1935; and others). It would appear that this latter substance is
relatively safe if given carefully in suitable subjects and if a sharp watch is kept
upon the peripheral fields at the stage when the course has reached the tenth
injection (Fine and Barkan, 1937). Even the comparatively safe tri-valent
compounds may produce blindness : thus Butler (1932) treated one twin
with congenital syphilis with sulphostab with the result that optic atrophy
developed, while the other twin similarly affected was untreated but retained
good vision. It would appear that the subjects for arsenical treatment
should be carefully chosen : old age should give rise to caution, for the great
majority of the recorded cases of poisoning have occurred in persons over
40 years of age ; advanced disease of the arteries, kidneys or liver should
be viewed as contra-indications, and the presence of any disease of the
optic nerve should call for the most anxious care in the use of these drugs.
While arsenical preparations may cause
considerable damage to a normal optic
nerve if carelessly administered to an
unsuitable subject (and in exceptional
cases if carefully given to a suitable
one), they may easily produce disaster to
a diseased nerve.
As a rule the symptoms come on after
some weeks or months of injection treat-
ment, and frequently the visual symptoms
precede all signs of general intoxication—
languor, headache, dizziness, colic, vomit-
ing, labyrinthine disturbances, incontinence
4' •) { { - $ •r + º-º: - * * † 4 +
Fig. 2003–ORGANIC ARSENIC and ataxv.
(ATONYI.) Poison ING. *...”
5 l () ()(), white.
In the most dramatic cases a bilateral,
sudden and complete amaurosis may
occur from which no recovery results (Tissot-Daquette, 1934); but
more usually the clinical picture is that of a rapidly developing extreme
concentric contraction of the visual fields with a central depression, which
is followed by pallor of the disc developing into atrophy with no subsequent
tendency to recovery (Fig. 2603). It is interesting that the pupillary
reactions may be well retained. The deterioration may progress steadily
until, after a period of from 1 to 2 years, all perception of light fails (Veil,
1935). Treatment is usually unavailing, although Veil (1935) reported
improvement after the immediate injection of acetyl-choline.
Experimental researches on animals have been carried out by Wendel-
stadt (1908), Igersheimer (1908–11), Igersheimer and Itami (1909),
Igersheimer and Rothmann (1909) and Birch-Hirschfeld and Köster (1910).
Gross degenerative changes were found both in the retina, particularly
in the ganglion cells, and in the nerve, and it would appear from the fact
that the earliest changes were found in the latter and that they are usually

DISEASES OF THE OPTIC NERVE 3031
most marked in its proximal parts, that the poison attacks both the retina
and the nerve. Similar changes have been noted histologically in human
subjects (Nonne, 1908; Birch-Hirschfeld and Köster, 1910; Sattler,
1912). The pathological picture is essentially that of a non-inflammatory
degeneration.
A different manifestation is sometimes seen, particularly in association
with the salvarsan group of compounds, characterized by dermatitis,
haematuria, kerato-conjunctivitis sometimes necrotic in intensity,” iritis,
retinal haemorrhages, and occasionally papillitis (Morpurgo, 1914; Hegner,
1917; Horvath, 1922; Terrien, 1929, and others).
Beck. Cb. pr. Aug., xxxiii, 129, 1909.
Igersheimer and Itami. A. f. eacp. Path., lxi,
Birch-Hirschfeld and Inouye. A. f. O., 18, 1909.
lxxix, 81, 1911. Igersheimer and Rothmann. Z. f. phys.
Birch-Hirschfeld and Köster. A. f. O., lxxvi, Chem., lix, 256, 1909.
403, 1910. Lazar. A. of O., xi, 240, 1934.
Butler. Brit. J. O., xvi, 356, 1932. Morpurgo. Münch. med. W., lxi, 657, 1914.
Clarke. T. O. S., xxx, 240, 1910. Nonne. Deut. med. W., xxxiv, 1291, 1908.
Coppez. La Clin. Opht., xv, 273, 1909. Sattler. A. f. O., lxxxi, 546, 1912.
A. de Oft. H. -A., ix, 409, 1909, Terrien. A. d’O., xlvi, 513, 1929.
Dawnay. T. O. S., xxx, 247, 1910.
Fehr. Deut. med. W., xxiii, 2032, 1907.
Fine and Barkan. Am. J. O., xx, 45, 1937.
Hegner. K. M. Aug., lix, 624, 1917.
Horvath. Szemeszet, lvi, 4, 1922.
Igersheimer. B. O. G. Heidel., xxxv, 242,
1908 ; xxxvii, 65, 1911.
Tissot-Daquette. A. d’O., li, 17, 1934.
Veil. Bull. S. d’O. Paris, xlvii, 71, 1935.
Wendelstadt. Berlin. kl. W., xlv., 2263,
1908.
Wray. T. O. S., xxx, 240, 1910.
Young and Loevenhart. J. Pharm. and Earp.
Therap., xxiii, 107, 1924.
A. f. O., lxxi, 379, 1909.
2. QUININE AND ITS RELATIONS
Quinine not uncommonly produces toxic visual symptoms, always
bilateral and usually of an acute nature and sudden onset, which in the
worst cases involves a complete amaurosis, but typically takes the form of
a contraction of the visual fields associated with a marked constriction of
the retinal arteries and some degree of optic atrophy ; the tendency is
towards recovery, although in all but the mildest cases some permanent
defect remains.
Quinine is usually taken as an anti-febrile remedy, particularly against
malaria, but its use as an abortifacient has on several occasions produced
amblyopia. The quantity necessary to produce visual symptoms varies
greatly for there is a definite idiosyncrasy to the drug. In susceptible
persons I gr., taken, for example, as a preventative against cold, may
produce a transient obscuration of vision : cases of visual symptoms follow-
ing relatively small doses are common–0. 13 gm. (Elliot, 1919), 1 gr. (Duggan
and Nanavati, 1929), 1.25 gr. (Schwabe, 1901). Foster Moore (1925) recorded
the case of a man who developed a permanent optic atrophy with greatly
restricted fields after drinking two bottles of a proprietary orange and
quinine wine, and another, a sea captain, who swallowed some quinine
1 Vol. II, p. 1711.
3032 TEXT-BOOK OF OPHTHALMOLOGY
to abort a cold and lost all perception of light for 48 hours. On the other
hand, some people can take almost unlimited quantities with impunity.
Experimental clinical observations on volunteer healthy human subjects
undertaken by Barabaschew (1891) have shown that definite symptoms with
diminished acuity and peripheral constriction of the fields with contraction of
the vessels and pallor of the disc, and sometimes a transient complete amaurosis,
can be produced in the average individual by 40 to 60 gr. of quinine
hydrochloride. de Schweinitz (1896) gave the toxic dose for dogs at 1 to
4 gr. per lb., and Holden (1898) at 0.07 gr. per kilo. body weight.
The development of toxic symptoms is ushered in by tinnitus and
deafness, phenomena which are followed within a few hours by visual
failure. This may be complete and absolute with total loss of perception
of light, the pupils being widely dilated, the retinal arteries contracted
and tenuous, and the fundi pale. Occasionally in the earliest stages the
retina may become oedematous (Smith,
1919), and the picture of complete obstruc-
tion of the central artery with development
of a cherry-red spot at the macula may be
simulated. As a rule the onset is sudden,
the vision being rapidly and completely
obscured as if a shutter were drawn down
over the eyes, but occasionally it may be
gradual and take several days for its full
development. In less severe cases the
central acuity is diminished but the periphery
becomes constricted. In all cases the
tendency is for recovery : in the milder
degrees of poisoning the vision may recover
in a few hours, but in the more severe
cases amaurosis may persist for several days or even weeks, and in this
event some degree of atrophy of the nerve and some permanent loss of
vision is invariable (Fig. 2633, Plate LXII). Permanent blindness,
however, has not been recorded. When recovery occurs the fields show a
varying degree of peripheral contraction, particularly for blue (Fig. 2604);
central vision is affected least and recovers first, but some peripheral defect
remains permanently except in the mildest cases.
A considerable amount of experimental work has been done upon dogs
and rabbits with a view to determining the histological changes after poisoning
with quinine (de Schweinitz, 1891–1906; Holden, 1898; Birch-Hirschfeld,
1900 ; Druault, 1900 : Altland, 1904 ; Vermes, 1905; Behse, 1909 :
Giannini, 1934). Changes have been observed within 10 hours after adminis-
tration of the drug—a chromatolysis and degeneration of the ganglion
cells, degeneration in the nerve fibres, and, in the later stages, thickening
of the walls of the blood-vessels, with thromboses and obliteration of their
FIG. 2604.—QUININE POIs ON ING.
50, 5, 1000, white.

DISEASES OF THE OPTIC NERVE 3033
lumen. Oguchi (1932) found that the metabolism of the retina was con-
siderably reduced by an amount varying from 20% to 30%. Pathological
examinations of human eyes have been few (Fortunati, 1905 ; Giannini,
1934), but have shown the same changes.
The pathogenesis of the condition is not yet agreed upon. In all
probability the quinine acts directly on the nerve elements, but there can be
no doubt also that the element of vaso-constriction is of extreme importance :
some authors, indeed, claim that this is the only effective factor. This is
substantiated by the experiments of Giannini (1934), who found in dogs
that while constricted arteries and pallor of the disc accompanied by
blindness could be produced relatively easily by quinine, associated histo-
logically with degeneration of the ganglion cells and obliteration of the
arteries, no observable effects, either clinically or pathologically, followed
the administration of quinine in combination with acetyl-choline. By
sectioning the sympathetic on one side, in which case blindness became
apparent only on the other, Vermes (1905) concluded that the vaso-
constrictor action of the drug was not directly upon the vessel walls but
was mediated nervously. The fact, however, that the amaurosis may
come on before the vascular constriction is evident, and the finding experi-
mentally in early cases that extensive degeneration of the nerve elements
precedes vascular changes would indicate that in all probability these play
a secondary rôle.
The treatment of quinine amblyopia should be directed to producing
vaso-dilatation. Visual improvement has been noted after the adminis-
tration of amyl nitrite (Buller, 1881; Gainsborough, 1922), or injections of
atropine (Lacat, 1925; Abadie, 1929). Elliot (1919) recommended para-
centesis of the anterior chamber or corneo-scleral trephining to maintain the
vaso-dilatation, and Alt (1927) noted improvement of the visual fields after
lumbar puncture.
Drugs related to quinine have been noted to give rise to somewhat similar
symptoms—QUINIDINE, CHINOLIN and others. Of these, however, the most interesting
is optochin.
OPTOCHIN (ETHYL-HYDRO-CUPREIN), a drug introduced by Morgenroth and Levy
(1911) in the belief that it was specific for the pneumococcus, has on several occasions
given rise to amblyopic symptoms (Fraenkel, 1912; Parkinson, 1913; Schreiber,
1916; Pincus, 1916; Jedwabrik, 1916; Oepen, 1917; Moore and Chesney, 1918;
and others). According to Abelsdorff (1919–23) the toxic dose is 0.75 gm. The
symptoms are the same as those associated with quinine : in the severest cases
complete amaurosis develops, there is the same peripheral constriction of the field,
although a central scotoma may also occur, and the same degenerative changes both
in the ganglion cells of the retina and the nerve have been demonstrated pathologically
(Abelsdorff, 1919; Uhthoff, 1916–17; Velhagen, 1925; Giannini, 1934) and produced
experimentally in animals (Schwarzkopf, 1922).
Somewhat similar effects, although less marked, have been noted with EUCUPRIN
(iso-amyl-hydro-cuprein) (Franke, 1920 : Franke and Hegler, 1920). -
3034
TEXT-BOOK OF OPHTHALMOLOGY
Abadie. Bull. Soc. d’O. Paris, xli., 100, 1929.
Abelsdorff. K. M. Aug., lxii, 31, 1919.
Deut. med. W., xlix, 792, 1923.
Alt. K. M. Aug., lxxviii, 556, 1927.
Altland. K. M. Aug., xlii, 1, 1904.
Barabaschew. A. f. Aug., xxiii, 91, 1891.
Behse. A. f. O., lxx, 239, 1909.
Birch-Hirschfeld. A. f. O., l, 166, 1900.
Buller. T. Am. O. S., iii, 262, 1881.
Druault. Travail du Lab. d’Ophtal., Paris,
1900.
Duggan and Nanavati. XIII Intermat. Cong.
Oph., Amsterdam, ii, 483, 1929.
Moore, Foster. Medical
London, II, 1925.
Moore and Chesney. A. of Int. Med., xxi,
663, 1918.
Morgenroth and Levy. Berlin. kl. W., xlviii
(2), 1560, 1979, 1911.
Oepen. Diss., Bonn, 1917.
Oguchi. Acta S. O. Jap., xxxvi, 155, 1932.
Parkinson. Z. f. Chemotherap., ii, 1, 1913.
Ophthalmology,
Pincus. Münch. med. W., lxiii, 1027, 1916.
Schreiber. A. f. O., xci, 305, 1916.
Schwabe. A. f. Aug., xlii, 47, 1901.
Schwarzkopf. Z. f. Aug., xlviii, 77, 1922.
Elliot. Brit. J. O., iii, 8, 1919. de Schweinitz. T. Am. O. S., vi, 23, 1891 ;
Fraenkel. Berlin. kl. W., xlix (1), 664, 1912. xi, 338, 1906.
Franke. B. O. G. Heidel.., xlii, 177, 1920. O. Rev., x, 49, 1891.
Franke and Hegler. Med. Kl., xvi, 628, 1920. The Toxic Amblyopias, Phila., 1896.
IFortunati. Riv. Ital. di Ott., i, 5, 1905. O. Rec., vii, 392, 1898; viii, 610, 1899.
Gainsborough. Brit. J. O., vi, 259, 1922. Smith. T. O. S., xxxix, 310, 1919.
Giannini. Am... dº Ott., lxii, 1069, 1934. Uhthoff. K. M. Aug., lvii, 14, 1916; lviii, 1,
Holden. T. Am. O. S., viii, 405, 1898. 1917.
Jedwabrik. Diss., Königsberg, 1916. Velhagen. K. M. Aug., lxxv, 122, 1925.
Lacat. La Clim. Opht., xiv, 373, 1925. Vermes. Z. f. Aug., xiv, 337, 1905.
3. ASPIDIUM (FILIX MAs)
Filix mas, which is used as an anthelmintic and is a violent gastro-
intestinal poison, may cause amblyopic symptoms similar to those arising
in quinine poisoning. The toxic dose varies very considerably with different
individuals, 4 gm. having produced marked visual effects while amounts
up to 45 gm. may be taken with impunity (Sidler-Huguenin, 1898): Stülp
(1905) met 4 cases in 22,000 treatments for worms. The symptoms come
on 1 to 12 days after the administration of the drug ; amaurosis may be
complete and may be permanent, or temporary and followed by a constric-
tion of the peripheral fields. A peculiar feature is that the symptoms are
not uncommonly unilateral (17 out of 47 cases, Stülp, 1905), but the prog-
nosis is worse than that of quinine poisoning. In acutely severe cases the
entire fundus may be occlematous and the vessels constricted, but in many
cases the fundi have been reported as normal, although optic atrophy
frequently follows. Experimental investigations on animals have demon-
strated chromatolysis and degeneration of the ganglion cells and the cells
of the inner nuclear layer as well as degenerative changes in the optic
nerve (Katayama and Okamoto, 1894; Masius and Mahaim, 1898; de
Schweinitz, 1896; Birch-Hirschfeld, 1900).
de Schweinitz. The Toasic Amblyopias,
Birch-Hirschfeld. A. f. O., l, 166, 1900.
Katayama and Okamoto.
gerichtl, Med., viii, Supp. 148, 1894.
Masius and Mahaim. Bull. Acad. Roy. de Méd.
de Belge, xii, 325, 1898.
Viertelsjhr. f.
Phila., 1896.
Sidler-Huguenin. Corresp.-Bl. f. Schw. Aerzte,
xxviii. 513, 553, 1898.
Stülp. A. f. Aug., li, 190, 1905.
4. SALICYLATES
SALICYLATES, given in large doses, may produce amaurotic effects resembling
quinine poisoning, although frequently the trouble may be central in origin.
The
DISEASES OF THE OPTIC NERVE 3035
Ocular symptoms come on with deafness and tinnitus, the pupils are dilated, and while
the fundus is sometimes normal, the arteries may show marked constriction (de
Schweinitz, 1896; Snell, 1901 ; and others). Recovery, however, is usually rapid.
Similar effects may follow aspirin, antipyrin, antifebrin and the related compounds
(Hotz, 1906).
Hotz. A. of O., xxxv, 160, 1906. Snell. T. O. S., xxi, 306, 1901.
de Schweinitz. The Toeic Amblyopias,
Phila, 1896.
5. ERGOT
ERGOT in massive doses, in which case it may be consumed as a fungus con-
taminating rye cereals or be taken as an abortifacient, may produce the symptoms
popularly known as “ St. Anthony’s fire,” characterized by pains in the limbs and
numbness followed by gangrene, or by convulsions (as well as uterine contractions).
The individual sensitivity varies (Kolosoff, 1912), but since its first observation by
Meier (1862), visual symptoms have been frequently noted (in some 25% of cases,
v. Bechterew, 1892), of which cataract is a frequent cause." The ophthalmoscopic
picture is characterized by retinal vaso-constriction and oedema. The associated
amblyopia, however, which involves a peripheral contraction of the fields, sometimes
with a central Scotoma, is transient and optic atrophy does not occur (Kortneff,
1892; Orlow, 1905 ; Kolosoff, 1912; Kaunitz, 1932; Kravitz, 1935). Experimentally
Peters (1902) found degeneration in the vessel walls and in the ganglion cells of the
retina.
v. Bechterew. Neurol. Cb., xi, 769, 1892. R ravitz. A. of O., xiii, 201, 1935.
Kaunitz. An. of Surg., xxv, II 35, 1932. Meier. A. f. O., viii (2), 120, 1862.
Kolosoff. Russ. Vrach., xi, 55, 120, 198, 1912. Orlow. Neurol. vestnik., ix, 304, 1905.
Kortneff. Vestn. O., ix, l 14, 1892. Peters. B. O. G. Heidel.., xxx, 20, 1902.
6. ANILIN AND ITS RELATIONS
ANILIN (AMIDOBENZOL) and NITROBENZOL, both of which are used in the dyeing
industry, and their relatives DINITROBENZOL and TRINITROTOLUOL, which are used in
the manufacture of explosives, may all produce symptoms of poisoning which may
include ocular effects. The drugs may be absorbed by the inhalation of fumes or fine
dust, or by absorption through the skin. Both a central scotoma and peripheral
constriction occur in the visual fields, together with constricted arteries, tortuous
veins, a haziness of the disc, occasional haemorrhages, and sometimes a dark violet
colouration of the fundus (Litten, 1881 ; Nieden, 1888; White, 1889; Snell, 1894;
Friedländer, 1900 : Berger, 1904 : Mellinghof, 1906; Legge, 1922; and others). On
removal of the workers from contact with the chemicals the prognosis is good, all the
symptoms clearing up.
Berger. A. f. Aug., l, 299, 1904. Mellinghof. K. M. Aug., xliv (2), 34, 1906.
Friedländer. Neurol. Cb., xix, 155, 294, 1900. Nieden. Cb. pr. Aug., xii, 193, 1888.
Legge. T. O. S., xliii, 2. 1922. Snell. Brit. Med. J., i, 449, 1894.
Litten. Berlin. kl. W., xviii, 23, 1881. White. Practitioner, xliii, 14, 1889.
3.036 TEXT-BOOK OF OPHTHALMOLOGY
D. Specific Infections
1. Tuberculosis
On the whole, tuberculosis of the optic nerve is rare. The infection
may reach the nerve or its sheaths in one of two ways—either by a process
of direct spread from a tuberculous lesion in the eye, the orbit, or the brain,
or by metastatic deposition from the blood-stream.
A. Tuberculosis by DIRECT SPREAD From NEIGHBouring STRUCTURES
(1) A spread of a tuberculous process from an intra-ocular lesion may
occur by direct extension from the uveal tract or retina, particularly in the
Fig. 2605. Military Tubercle of Choroid INvADING Optic NERVE.
A. Intense neuritis of optic nerve. B. Round-celled exudation. C. Caseating
tubercle in choroid. D. Retinal cedema. E. Sub-retinal exudate (Cargill and
Mayou, T. O. S.).
former, when the lesion is juxta-papillary in site." In this event the tissues
of the disc may be invaded directly by granulation tissue and an enormous
oedema and swelling may result (Fig. 2605). There is a general inflammatory
infiltration of the nerve with giant cells and occasionally well-defined
tubercle systems, followed by a considerable proliferative reaction of the
neuroglia and connective tissue. The terminal result, if disorganization
* p. 2306.

DISEASES OF THE OPTIC NERVE 3037
does not ensue, is a secondary atrophy of the nerve-fibres (Jung, 1891 ;
Emanuel, 1902; Friedenwald, 1902; Cargill and Mayou, 1906; Knapp,
1913; van der Hoeve, 1914, and others). A similar process may also
occur by indirect spread from the more anterior parts of the eye, especially
in irido-cyclitis, the infection reaching the nerve-head either by migration
through the vitreous body or by a periphlebitic extension around the
perivascular lymphatics up the retinal veins to the central vessels, a process
which has been demonstrated experimentally (Straub, 1912) and studied
clinically (Meller, 1922; and others). The pathology of these cases has
already been sufficiently discussed in a previous chapter." It is to be noted
that gross signs of disease may be evident in the orbital portion of the
nerve behind the lamina and that the sheaths may eventually be affected
(Bergmeister, 1925–27 ; Mauksch, 1924).
(2) A spread of a tuberculous process from an orbital lesion is more rare,
the sheaths being first attacked, and degeneration of the nerve-fibres
being eventually brought about partly by actual penetration and partly
as the result of reactive connective tissue overgrowth (Rochon-Duvigneaud
and Onfray, 1906; Birch-Hirschfeld, 1910).
(3) A spread of a tuberculous process from the meninges is not un-
common in tuberculous basal meningitis. The condition was produced
experimentally by Deutschmann (1881), who injected pus from a tuber-
culous knee-joint into the cranial cavity of rabbits, and produced a clinical
picture of papillitis and choroidal tubercles; necropsy showed a wide-
spread tuberculous meningitis, a peri-neuritis wherein the dural and pial
sheaths were studded with tubercles, and a spreading peripheral neuritis.
Clinical cases wherein an analogous process has occured have been recorded
by Chiari (1877), Sattler (1878), Sisaric (1921), Igersheimer (1924), and
others : there is a general inflammatory reaction of the sheaths and the
nerve itself may be converted into a thick mass of granulation tissue with
areas of caseation. A much rarer occurrence is the formation of an isolated
tuberculoma in the chiasma (Hjort, 1867) or in the intra-cranial portion of
the nerve (Cruveilhier, 1862) in cerebral and meningeal tuberculosis.
B. METASTATIC TUBERCULOSIS
Metastatic tuberculosis may either be part of a general miliary dissemina-
tion, or appear in the optic nerve as a solitary lesion ; in addition, there
are a number of reported cases showing symptoms of retro-bulbar neuritis
in which the presumptive diagnosis is tuberculosis.
(i) MILIARY TUBERCLE
That the optic nerve, and more particularly its sheaths, may parti-
cipate in the general dissemination of miliary tuberculosis has been
1 p. 2668.
3038 TEXT-BOOK OF OPHTHALMOLOGY
known since the early observations of Kabsch (1882) and v. Michel (1903):
it usually occurs in association with a simultaneous involvement of the
meninges and the uveal tract. The dissemination may be widespread
with the characters of a purulent tuberculous peri-neuritis, the vaginal space
being filled with inflammatory tissue and the pial septa at the periphery
of the nerve being studded with miliary tubercles. Similar tubercles appear-
ing to lie in the nerve substance are found to be associated with the small
vessels, while the central vessels may be heavily diseased, the central
vein being on occasion filled with
giant-cell systems and the tissues
around being infiltrated with
lymphocytes and epithelioid cells
(Fig. 2606) (Goldstein and Wexler,
1930).
(ii) solitary MASSIVE TUBERCLE
Solitary and massive con-
glomerate tuberculous lesions of
the optic nerve are rare, and are
met with in children and young
adults. The majority of cases
occur in the region of the papilla,
whereupon, through a considerable
vitreous haze, a swelling of whitish
colour and varying size projects
into the vitreous cavity. There
are usually large haemorrhages
associated with it and the reaction
- in the surrounding retina is con-
Fig. 2606.-MILIARY TUBERCLE or THE - - - - - - - - -
Optic NERVE. siderable; a plastic irido-cyclitis is a
Tuberculous infiltration around the cen- usual accompaniment, presumably
tral vessels. Two sections of the vein below of toxic origin. Eventually a. pic-
contain giant cells; the artery above con- -
tains blood (Goldstein and Wexler, A. of 0.). ture is presented of a complete
detachment of the retina, a grey
reflex from the fundus resembling a pseudo-glioma, or a severe irido-
cyclitis with an impenetrable vitreous. The process is accompanied by an
abolition of vision and the development of severe pain. If the disease
occurs further up the nerve, however, the only ocular evidences may be a
swelling and oedema of the disc with retinal haemorrhages; in such cases it
is obvious that a clinical diagnosis cannot be made with certainty. The
diagnosis in all cases is extremely difficult, and, although it may be sug-
gested by the clinical appearance, the presence of active tuberculosis else-
where, or the therapeutic response to tuberculin, it can never be accepted as
unquestionable unless the process is so intense as to make enucleation

DISEASES OF THE OPTIC NERVE 3039
necessary or to cause a fatal termination and thus allow histological
examination.
Several pathological examinations have been recorded of such lesions
(Weiss, 1877; Brailey, 1883; O'Sullivan and Story, 1899; Spalding, 1903;
Knapp, 1903; Coats, 1906; Verderame, 1908; Komoto, 1912; Jacobs,
1912; MacRae, 1928) (Fig. 2607). There is an intense inflammatory
infiltration with lymphocytes, plasma or epithelioid cells, and occasional
Fig. 2607.-SolITARY TUBERCLE of THE NERVE-HEAD.
The intra- and extra-ocular portions are connected by a narrow neck through the
scleral canal where the lamina has disappeared. The intra-ocular portion is
necrotic and the retina is detached. The sheaths of the nerve are little infiltrated
(Coats, R. L. O. H. Rep.).
giant cells sometimes with tubercle bacilli, which is usually most marked
near the central vessels. The vein particularly is frequently largely
destroyed by infiltration of its walls with small round cells, while in the area
of intense inflammation the nerve-fibres disappear. For a considerable
distance around, there is infiltration of the connective tissue septa between
the bundles of fibres and a proliferation of fibroblasts. This intense patho-
logical destruction has usually been limited to the region of the nerve-head
at the time when pathological examination has been made, but it may
extend right up to the apex of the orbit, the whole nerve being enormously
T.O.-WOL. III. 3 P.

3040 TEXT-BOOK OF OPHTHALMOLOGY
swollen and its fibres completely destroyed (MacRae, 1928). As a rule the
choroid or retina is only involved for a short distance round the disc, evidently
as a secondary invasion for the infiltration ceases somewhat abruptly, but
here tolerably well-formed tubercle systems are more usually found. There is
usually some small-celled infiltration of the sheaths of the nerve, and excep-
tionally this direction of spread may be so pronounced as to give rise to fatal
tuberculous basal meningitis (Szabo, 1931).
In general terms the course of the disease is progressive and the
prognosis bad, so far as the eye is concerned. Cases have been reported
where an obvious lesion on the papilla has cleared up under tuberculin with
more or less damage to the sight (Mayou, 1914; Hata, 1935; and others),
but as a rule the disease is progressive until excision of a blind and painful
eye becomes advisable. If this is done in time, the prognosis with regard
to life is frequently good.
Many cases of optic neuritis, retro-bulbar in type, have been reported in the
literature as being presumably tuberculous in origin. In most of these the diagnosis
has been based on the response to tuberculin (Kiep, 1936; and others), a somewhat
dangerous argument. In others corroboratory evidence has been brought forward,
such as a rarefaction (presumably a tuberculous Osteitis) of the sphenoid bone (Laschi,
1931); but in them all a definite clinical diagnosis is impossible.
The treatment of the secondary form of tuberculosis follows that of
the original lesion ; the treatment of the miliary form is purely sympto-
matic ; while in the conglomerate type of tubercle, the usual treatment
for tuberculosis with tuberculin may be tried, but in the event of its failure
to keep the process in check, resort must be had to excision of the eye
with the nerve.
Bergmeister. Z. f. Aug., liii, 175, 1925. Knapp. A. of O., xxxii, 22, 1903.
Die tuberkulösen Erkramkungen, d. Augen,
Berlin, 1927.
Birch-Hirschfeld. Z. f. Aug., xxiv, 193, 1910.
Brailey. T. O. S., iii, 129, 1883.
Cargill and Mayou. T. O. S., xxvi, 101, 1906.
Chiari. Wien. med. Jb., iv, 559, 1877.
Coats. R. L. O. H. Rep., xvi, 381, 1906.
Cruveilhier. Traité d’Amat. path. générale,
Paris, iv, 793, 1862.
Deutschmann. A. f. O., xxvii (1), 224, 1881.
Emanuel. K. M. Aug., xl (2), 210, 1902.
Friedenwald. T. Am. O. S., ix, 577, 1902.
Goldstein and Wexler. A. of O., iii, 553, 1930,
Hata. Acta Jap. O. S., xxxix., 18, 1935.
Hjort. K. M. Aug., v, 166, 1867.
v. d. Hoeve. K. M. Aug., liii, 487, 1914.
Igersheimer. A. f. O., cxiv, 267, 1924.
Jacobs. K. M. Aug., l (2), 37, 1912.
Jung. A. f. O., xxxvii (4), 125, 1891.
Kabsch. Diss., Würzburg, 1882.
Riep. T. O. S., lvi, 298, 1936.
A. f. Aug., lxxv, 259, 1913.
Komoto. K. M. Aug., 1 (1), 82, 1912.
Lamb. Am. J. O., xx, 390, 1937.
Laschi. An... di Ott., xxxviii, 17, 1931.
MacRae. T. O. S., xlviii, 441, 1928.
Mauksch. Z. f. Aug., liv, 49, 1924.
Mayou. T. O. S., xxxiv, 180, 1914.
Meller. Z. f. Aug., xlvii, 247, 1922.
v. Michel. Münch. med. W., 1, 7, 1903.
O’Sullivan and Story. T. R. Acad. Med.
Ireland, xvii, 451, 1899.
Rochon-Duvigneaud and Onfray. A. d’O.,
xxvi, 129, 1906.
Sattler. A. f. O., xxiv. (3), 127, 1878.
Sisaric. Wien. med. W., lxxi, 445, 1921.
Spalding. T. Am. O. S., x, 141, 1903.
Straub. T. O. S., xxxii, 60, 1912.
Szabo. K. M. Aug., lxxxvii, 805, 1931.
Verderame. K. M. Aug., xlvi (1), 401, 1908.
Weiss. A. f. O., xxiii (4), 141, 1877.
DISEASES OF THE OPTIC NERVE 304 I
2. SYPHILIS
(i) syPHILITIC OPTIC NEURITIs
Optic neuritis or papilloedema occurs not infrequently in the earlier
stages of a syphilitic infection. Sometimes the ophthalmoscopic changes
are unaccompanied by any disturbance of function or signs of cerebral
involvement ; at other times there is a transitory derangement of vision
and signs of mild cerebral irritation such as violent headaches; relatively
rarely the clinical picture of acute syphilitic meningitis and meningo-
encephalitis develops with widespread central nervous symptoms. All types
are presumably due to an involvement of the optic nerve in a central
syphilitic process of greater or less severity.
These evidences of neuro-syphilis tend to occur early in the infection,
usually within the first year, and may be of four types: (a) most commonly
a mild meningeal infection occurs during the secondary stage, either before,
contemporaneously with, or soon after the secondary exanthem, some 3 to
9 months after the primary infection (5% of syphilitic cases, Uhthoff, 1893;
Wilson and Gray, 1917; Neumann, 1918; Carr, 1929); (b) much less
commonly in infants with congenital syphilis (Sharkey and Lawford, 1884;
Hutenil, 1913; Lavergne, 1916; Mazzeo, 1926; Ström-Olsen, 1930);
(c) rarely in latent periods during the tertiary stage of acquired syphilis
(up to 20 years, Drake, 1933), and (d) more commonly, as a neuro-recidive
reaction after inadequate treatment. This last manifestation in which the
optic neuritis is frequently associated with paresis of the oculo-motor,
abducens, trochlear, facial and auditory nerves, became relatively common
after the introduction of arsenicals as a method of therapy, and a large
crop of cases were reported in the early days of arsenic when a single large
injection was considered sufficient treatment (Fehr, 1912; Werncke, 1913;
Terrien and Prélat, 1914; Lawford, 1916; Pfister, 1926 ; and others).
Thus Finger (1911) reported 45 recidive reactions in 500 cases treated with
arsenic, and 5 in 2,000 treated with mercury. Presumably the reaction is
due to the activation of latent groups of organisms in the central nervous
system (Artz and Kerl, 1914), and since treatment by arsenic has been more
thoroughly carried out, the incidence of this type of accident has greatly
declined.
The meningeal syphilitic infection, as we have seen, may be so slight
and localized as to produce no clinical symptoms, but the occurrence of
headaches is commoner. In the more acute cases these may be very
violent, and at times, particularly in children, the disease is ushered in
abruptly with delirium, vomiting, convulsions and coma. A papillitis is a
common symptom, and papilloedema, varying from 2 to 5 dioptres denoting
a disturbance in the orbital portion of the nerve, still more common ; it is
usually bilateral but unilateral cases also occur (Uhthoff, 1893; Drake,
1933). Pupillary disturbances are the rule : inequality, irregularity and
3 P 2
3042 TEXT-BOOK OF OPHTHALMOLOGY
sluggishness or absence of the light and accommodation-convergence
reflexes. Especially when meningo-emcephalitic symptoms are present,
Fig. 2608. Active Syphilitic OPTIo
NEURITs.
Concentric contraction with a
nerve-bundle defect, running from
the upper temporal border of the
blind spot, 3/330, white.
involvement of various cranial nerves
occur, particularly the 3rd, 4th, 6th, 7th
and 8th, and here the affection is fre-
quently bilateral; the resultant diplopia,
facial paresis, or deafness, however, may be
very distressing.
In the milder cases the vision may
be unimpaired, but in the more severe
forms the loss of vision may be considerable
or complete. The visual fields show no
characteristic change although a con-
centric contraction for white and colour is
the most characteristic involvement; an
enlargement of the blind spot may occur,
a central, a centro-caecal, or a para-central
scotoma, or a nerve-bundle defect (Sloan
and Woods, 1938) (Fig. 2608).
Pathologically the characteristic change
is a diffuse small-cell infiltration of the pia-arachnoid in which lymphocytes
predominate, although in the early stages of acute cases, polymorphonuclear
cells may temporarily be the preponderating element. The infiltration is
Fig. 2609.-Syphilitic PER1-NEURiºnis AND INTERstitial Nºuritis.
At the healed stage showing disseminated spots of interstitial neuritis. The sheaths
and septa are immensely thickened and the nerve fibres atrophie (Behr, A. f. O.).


DISEASES OF THE OPTIC NERVE 3043
essentially perivascular, producing an intimal proliferation without
degenerative changes, and it may follow the blood-vessels into the brain-
tissue to form encephalitic areas. This meningeal infiltration may involve
the cerebral and cerebellar convexities, but is usually especially evident in
the region of the chiasma, while the cranial nerves, particularly the optic
and the auditory, are frequently markedly involved, the infiltration
affecting not only the sheaths but also the nerve-tissue itself as in a
peripheral interstitial neuritis (Uhthoff, 1893–94). The end-result is usually
a regression and disappearance of the inflammatory elements corresponding
to a clinical cure, but chronic changes may result if the condition is not
fatal. These consist of extreme interstitial proliferation, so that the nerve-
elements are destroyed and are replaced by granulation tissue and glial
proliferation which eventually develop into masses of dense neuroglial and
fibrous tissue (Fig. 2609).
The diagnosis is based on the history of infection, on the evidences of
syphilis elsewhere, and above all on the serological reactions for syphilis.
The blood Wassermann is positive in 85% of cases and the cerebro-spinal
fluid reaction is almost invariably so ; the colloidal gold curve is inconstant
and may be of the luetic or paretic type. The cerebro-spinal fluid may
vary from being clear to turbid, but the protein content is increased, and it
may contain cells, largely or entirely lymphocytic in type. In a number of
cases the treponema has been found in the fluid.
In quite a proportion of cases the affection is transitory and leaves
no sequelae, and in the majority, if the diagnosis is made early and
adequate treatment instituted, the prognosis is good. Vision may com-
pletely return, and the field usually shows a tendency to almost complete
recovery with no residuum other than an enlarged blind spot or unimportant
peripheral defects for white and colours. Recurrences are rare, but have
been recorded (Kiep, 1924). In cases where the inflammation has been
severe, however, and secondary atrophy has developed, the changes in the
field are permanent. In the worst cases bilateral blindness may result.
Treatment should be early and intense ; it should be that of cerebral
rather than ocular syphilis, and should be carried out vigorously so that if
the ideal of destroying all the organisms cannot be attained, the patient’s
resistance to the infection may be so raised that it can be maintained in a
state of latency. It is probably best to commence treatment in most cases
with an intensive course of mercury (by inunction or intra-muscular injection)
or bismuth (by intra-muscular injection) combined with iodides (up to 60
or 90 grains a day if it can be tolerated). After two weeks arsenic may be
started, at first in small doses, watching carefully for any untoward reaction,
and maintained in weekly doses for 6–8 weeks. As we have seen, the
trivalent preparations are safer than the pentavalent ; although trypars-
amide has a popular reputation, neo-arsphenamine is probably safer; but in
all cases extreme care must be taken that the patient is not susceptible, or
3044 TEXT-BOOK OF OPHTHALMOLOGY
does not develop a susceptibility after about the tenth dose. Such courses,
combining mercury or bismuth with arsenic and iodide, should be given with
intervals of 2 months' rest during which small doses of iodide only are taken,
until repeated examination of the blood and cerebro-spinal fluid shows that
a negative Wassermann is attained and maintained over a number of years.
When the intra-cranial pressure is raised and papilloedema becomes intense,
repeated lumbar punctures may give relief.
(ii) GUMMATA
Gummata of the optic nerve are rare. In his exhaustive study of the
subject, Uhthoff (1893–1903) found the intra-cranial part and the chiasma
Fig. 2610.-Gummatous PER1-Neuritis.
The intra-eranial portion of the nerve. The pial sheath is enormously thickened
with a massive small-celled infiltration which infiltrates the nerve along the peri-
vascular tissues (Behr, A. f. O.).
affected most commonly, either being directly involved or being attacked by
the extension of a gummatous meningitis which is relatively common in the
chiasmal region. In this latter event the nerve may be simply surrounded,
or an interstitial neuritis may be set up, or its substance may be heavily
infiltrated with granulation tissue which destroys the nerve-fibres and is
eventually replaced by a dense mass of neuroglial and fibrous tissue (Fig. 2610).
The intra-canalicular or the intra-orbital portions of the nerve may be
similarly attacked, resulting in the clinical appearance of a descending
neuritis or a descending atrophy.
Gummatous formations on the optic disc are also rare but give rise to a

DISEASES OF THE OPTIC NERVE 3045
more characteristic picture (Juler, 1897 ; Wagner, 1903; Stock, 1905;
Parsons, 1905; Verhoeff, 1910; Matsukawa, 1913; and others). Clinically,
the gumma may appear as an intra-ocular tumour, the papilla being enor-
mously swollen and the surrounding retina much thickened and detached,
while the vitreous is hazy with Opacities and a plastic irido-cyclitis is the
rule. Microscopical examination shows the nerve enormously thickened
with an intense small-cell infiltration usually interspersed with areas of
caseation and necrosis, the process causing a thrombosis and obliteration
of the central vein and complete destruction of the nerve fibres.
The condition of neuritis papulosa described by A. Fuchs (1926–32), Opin (1934)
and others, wherein a syphilitic new formation on the disc is associated with other
areas of chorio-retinal disease, has already been discussed.*
The treatment is the same as for syphilitic interstitial neuritis.
(iii) PARA-syPHILITIC (PRIMARY) OPTIC ATROPHY
The atrophy of the optic nerve associated with the late manifestations of
neuro-syphilis—dementia paralytica, the tabetic form of dementia paralytica
and, more particularly, tabes—and occurring apparently sometimes as an
isolated phenomenon, has been traditionally called PRIMARY OPTIC ATROPHY,
since it was conceived to be due to a primary degeneration of the ganglion
cells of the retina owing to the action of syphilitic toxins ; nowadays it is
generally conceded to be a secondary atrophy following inflammatory
mischief in the nerve, but although the term “primary '' is thus patho-
logically speaking a misnomer, its retention has considerable justification in
the clinical sense in so far as the atrophy is not preceded by any
ophthalmoscopically visible changes.
The occurrence of optic atrophy in para-syphilis is fairly common and
accounts for some 1% of eye diseases (Uhthoff, 1903). It is most common
in tabes in which disease it affects some 6.5% of cases (Gowers, 1904); in
juvenile tabes the percentage is much higher (50%, Wilbrand and Saenger,
1913), and it is found in some 8 to 10% of general paralytics (Uhthoff, 1903).
Moreover, a number of cases exist where the atrophy of the optic nerve
seems an independent para-syphilitic manifestation quite apart from other
evidences of central nervous disease (Wilson, 1912; McIntosh and Fildes,
1913; Head and Fearnsides, 1914). In all cases it usually appears some
10–15 years after the primary infection, between the ages of 30 and 50, and
in congenital cases the juvenile forms become evident as a rule about the
10th year of life.
The clinical course of this manifestation of neuro-syphilis is fairly
characteristic. Failure of vision and the development of atrophy may be the
first sign of tabes and not infrequently leads the patient to come for advice;
1 p. 2664.
3046 TEXT-BOOK OF OPHTHALMOLOGY
indeed, this visual symptom may precede the onset of other signs of disease
by as much as 20 (Gowers, 1883) or 28 years (Mott, 1903) and usually comes
on in the presence of a paucity of other neurological signs. These may
include Argyll Robertson pupils, absence of the knee-jerks, the presence of
anaesthesias (particularly of the 5th nerve), inco-ordination, and so on. On
the other hand, the atrophy may come on late when ataxy is well developed.
Berger (1889) saw 29 cases in the pre-ataxic stage against 12 in the ataxic
and only 3 in the paralytic, while Galezowski (1904) noted 55 in the pre-
ataxic stage against 8 in the stage of ataxy. It is generally agreed that
when optic atrophy comes on early, the tendency for its progression is strong
while the development of ataxy is slow, and conversely, when it appears
late it progresses slowly, while the spinal symptoms run their usual course.
Thus Förster (1900) found the average pre-ataxic period to be 4 years in
the absence of optic atrophy, and 9% years in its presence. It is to be
remembered, however, that these patients in whom the development of
ataxic symptoms is delayed, show areas of degeneration in the posterior
columns, according to Mott (1903), in every case. It seems probable,
therefore, that an involvement of the two localities is to some extent mutually
exclusive, although the view of Slinger and Horsley (1906) and Maloney
(1913) must be remembered, that the delay in the development of ataxic
symptoms is apparent rather than real, and is due to their mitigation by an
abnormal development of the muscular and arthrodial senses stimulated
by the blindness with a consequent increase in co-ordination.
The atrophy appears first unilaterally but the second eye always becomes
affected, usually showing the same type of field defect, but lagging behind
the other in its development. Sometimes it progresses rapidly, but usually
slowly, until eventually after some months or years, it becomes complete
and blindness is absolute with a fixed and dilated pupil. It is noteworthy
that the appearance of the degree of atrophy is a very uncertain guide as to
the amount of vision remaining, for relatively good sight is not incompatible
with an apparently completely atrophic disc. The disc becomes grey or
dead white, sometimes with a greenish or bluish tint, the stippling of the
lamina cribrosa is seen at its base, the edge is clear cut and very sharply
defined, and the surrounding retina looks normal (Fig. 2632, Plate LII).
Owing to the disappearance of the nerve elements there may be a slight
amount of atrophic cupping, which is distinguished from a glaucomatous cup
by its shallowness and saucer-like edges. The vessels are relatively normal,
showing little constriction or sheathing, a circumstance which, in the absence
of proliferating elements which destroy the clarity of its surface and the
clear definition of the edge, distinguishes the condition from a post-neuritic
atrophy.
The clinical symptoms of para-syphilitic atrophy are notoriously
variable, the variability appearing in the relationship between the visual
acuity, the field defects and the ophthalmoscopic appearances, as well as in
DISEASES OF THE OPTIC NERVE 3047
the rate of the development of the disease. The most important symptoms
8,I’é –
(a) A loss of dark adaptation, which according to Behr (1915), Rutgers
(1924) and Gasteiger (1927), is a most important early sign and may precede
any observable changes in the fundus, or any discoverable alteration in the
visual acuity, fields, or colour sense.
(b) A contraction of the colour fields, so that a stage of acquired colour-
blindness may occur. As is usual in lesions of the conducting pathways the
defects for red almost invariably precede those for blue.
(c) The appearance of subjective light phenomena as photopsiae, and
the development of coloured vision are rarer symptoms, everything appearing
as if seen through a brilliant curtain of green (Dodd, 1899–1900) or red
(Uhthoff, 1903), or shimmering with gold or silver (Uhthoff, 1903).
(d) The development of defects in the visual fields, most typically a
general and progressive loss of sensitivity with a peripheral contraction, but
frequently with sharply delineated isolated scotomata. These defects are
gradually progressive until central vision is engulfed ; perception of light
may remain for a considerable time in the temporal field but eventually, with
few exceptions, blindness is complete.
The usual course of development of these symptoms is that of a pro-
gressive and steady deterioration without remissions, blindness resulting in
an average time of 2–3 years; as extremes Uhthoff (1903) found 2 months
and 12 years. The natural history is divided by Behr (1915) into four
stages —
1st. A disturbance of dark adaptation with normal acuity, visual
fields, colour sense and ophthalmoscopic appearances.
2nd. A disturbance of adaptation with the appearance of atrophy,
other functions being normal.
3rd. A disturbance of adaptation and visual fields for white and
colours, a diminution of visual acuity and advanced atrophy.
4th. Complete atrophy and amaurosis.
This orderly sequence is not, however, always maintained. Although atrophic
changes usually precede marked defects in the fields, there is evidence that the opposite
sequence may occur. Thus Igersheimer (1918) and Sloan and Woods (1938) reported
a group of cases in which field defects typical of tabetic atrophy occurred in the
presence of clinically normal discs; some of these developed a frank atrophy later,
but others retained a normal fundus and an undiminished central acuity for periods
up to five years. It appears, therefore, that in a minority of neuro-syphilitics perimetric
changes may ante-date ophthalmoscopic changes or visual symptoms in the develop-
ment of para-syphilitic atrophy.
The field changes in para-syphilitic atrophy are extremely inconstant and
varied, showing no specific characteristics. In any given case the tendency
for the defects in the two eyes to be symmetrical is by no means constant,
and is almost invariably unequally developed in degree. Uhthoff (1903),
3048 TEXT-BOOK OF OPHTHALMOLOGY
who summarized the early work on perimetry in this disease, divided the
field defects into two large classes —
I. The entire field suffers simultaneously with a general reduction of
form and colour sense and of peripheral and central acuity. Perception
of red and green fade first, blue and yellow later, and white last. In this
event a full field may be obtained with a large white object while the visual
FIG. 261 l—l 4.—PRIMARY OPTIC ATROPHY.
FIG. 261 I - Vision 6/60. 10/330 FIG. 26.12.--Vision 6 9. 10330, white.
white.
FIG. 261.3.−Vision : counts fingers. FIG. 2614.—Centro-caecal scotoma.
20/330, white.
acuity becomes very low. Such a development is the more common and
probably corresponds to an involvement of the entire cross-section of the
nerve (Fig. 2611).
2. Areas of visual defect are sharply delineated from areas in which
normal function is retained ; in this event central acuity may remain good
for a considerable time. Such a defect denotes a patchy distribution of the
disease. In other instances there may be sectorial loss with imperfect


DISEASES OF THE OPTIC NERVE 30.49
Figs. 2615–17. –PRIMARY OPTIC ATROPHY.
Left. Right.
FIG. 2615. Vision : L. 6 1S. R. 636, colours lost bitemporal quadrantic
defect. 10,330, white.
Right.
Fig. 2616. Bitemporal hemianopie defect.
Right.
Fig. 2617. Horizontal hemianopic defect.



3050 TEXT-BOOK OF OPHTHALMOLOGY
functioning of the remainder of the field when both tendencies come into
play. These various defects have been closely studied by a number of
observers over large series of patients (Uhthoff, 1903; Rönne, 1909–12;
Fuchs, 1911; Langenbeck, 1912; Stargardt, 1913; Igersheimer, 1918;
Paton, 1922; Arlt, 1922; John, 1929; Goldberg, 1935; Rutherford,
1935; Sloan and Woods, 1938). The most common forms which are met
with are these :—
(a) Concentric contraction of the peripheral field with late loss of
central vision (Figs. 2612–2613).
(b) A central or centro-caecal scotoma with normal or defective peri-
pheral fields denoting especial involvement of the papillo-macular
bundle (Fig. 2614). Different authors give very varying estimates
of the frequency of such a scotoma. Paton (1922) never met one ;
Uhthoff (1903) considered it rare (2%); Fuchs (1911) found 30
cases; Arlt (1922) 3 out of 53; John (1929) 8 out of 83 ; Sloan and
Woods (1938) 30 out of 56 cases.
(c) Quadrantic and hemianopic defects have been frequently observed,
often homonymous in distribution (Figs. 2615, 2616 and 2617).
Quadrantic defects are relatively common (Rönne, 1912; Paton,
1922); bitemporal hemianopia was recorded by Zimmermann
(1905), Wilbrand and Saenger (1913); binasal hemianopia by
several observers (Lang and Beevor, 1894; Rönne, 1912; Heed
and Price, 1914; Lutz, 1928–30; Drake, 1934); a homonymous
left-sided hemianopia was seen by Nonne (1921); and a purely
horizontal hemianopic loss by Uhthoff (1903). It is considered by
some authors that such defects are indicative of disease in the
chiasma or tracts (Fuchs, 1911); others judge them to be merely
an expression of symmetrical defects affecting the nerve (Uhthoff,
1903), a view upheld by Rönne (1909–12) and Langenbeck (1912),
who found that careful testing usually revealed that the
hemianopia was not of a true type with a sharp defect for all sizes
of objects; while others have considered them as evidence of
some complicating cerebral or basal lesion usually of a vascular
nature (Stargardt, 1913; Sloan and Woods, 1938).
Pathology. Despite a very great deal of work, the pathology of the
optic atrophy is still in dispute, as is also that of the general para-syphilitic
processes in the central nervous system. This might seem strange in view
of the common occurrence of neuro-syphilis, but is explained by the great
difficulty of obtaining early cases for histological examination, and the
probability that in the later stages the lesion is complicated by the presence
of other of the many diverse lesions which syphilis can produce in the
central nervous system. According to the most popular view held to-day,
the Optic atrophy in tabes, dementia paralytica, and tabo-paresis is
essentially of the same type and is due to a primary peripheral and interstitial
DISEASES OF THE OPTIC NERVE 3051
neuritis arising as an extremely chronic exudative process essentially from
the pia, due to the local production of toxins in the presence of the treponema,
with a secondary degeneration of the nerve-fibres and their parent ganglion
cells, the process becoming apparent first in the periphery of the nerve and
most frequently in the intra-cranial portion distal to the chiasma (Fig. 26.18).
It is well to remember, however, that this view of the pathology is not
universally accepted: nor is agreement reached on the pathogenesis of the
parent diseases. This applies both to the cause of the essential changes,
their site, and their essential nature. In dementia paralytica the pia is
Fig. 2618–TABETIC ATRoPHY (Behr, A. f. O.).
in a state of obvious inflammation, but in tabes degenerative changes
appeared to be predominant to the earlier workers. This para-syphilitic
manifestation was therefore thought to be due to the action of a meta-luetic
toxin produced by the treponema in other parts of the body which had an
elective action on certain nerve-tracts or cells. In this connection Orr and
Rows (1903–04) showed that when celloidin capsules containing bacteria
were placed in contact with afferent nerves, the toxins passed along the
peri-neural lymphatics to the cord where they produced a degeneration of
the posterior columns indistinguishable from that occurring in tabes. The
discovery of the treponema in the brain of paretics by Noguchi (1913),

3052 TEXT. BOOK OF OPHTHALMOLOGY
however, led to the suggestion that the treponema would also be found in
tabes, an expectation realized by Richter (1921) who discovered the organism
in granulation tissue in the sub-arachnoid recess surrounding the “radicular
nerves” where, as demonstrated by Nageotte (1894–1906), the sub-arachnoid
space forms a pocket running towards the spinal ganglia along the posterior
roots. Similarly Igersheimer (1921) found this organism in close relation
to the optic nerve in cases of paresis and tabo-paresis, and Biffis (1937)
found it in the nerve itself; and although it has not yet been demonstrated
in cases of tabes, it is generally considered that the changes in both are
probably due to the direct effect of the organism. The remarkable lack of
correlation between the number of organisms and the extent of the lesions
may best be explained on the principle of allergy : the tissues are rendered
susceptible in the generalized secondary stage of the infection, so that the
awakening into activity of the few organisms which have escaped the general
destruction (either by anti-bodies or drugs) which takes place towards
the end of the secondary stage, produces a disproportionately massive
effect.
With regard to the nature of the lesion in dementia paralytica there is
little controversy ; the pia is in a state of chronic inflammation and the
brain-tissue shows an intense perivascular infiltration of lymphocytes and
plasma cells with a proliferation of the endothelium of the capillaries,
changes in the mesoblastic tissues which are associated with a reaction of
the ectoblastic tissues comprising a degeneration of the ganglion cells and a
compensatory proliferation of the neuroglial elements. The virus seems to
be in sufficient concentration to act on both interstitial and parenchymatous
elements. In tabes, however, the earlier authors thought that the changes
were purely degenerative, the meta-luetic toxin having a selective action on
the posterior columns of the cord (Flechsig, 1890), the ganglion cells of the
posterior root ganglia (Stroebe, 1894), or the nerve-fibres of the posterior
roots (Orr and Rows, 1903–04). According to this view the toxin was
purely parenchymatous in its effects and the interstitial changes were
secondary and compensatory. The observation, however, of definite
vascular proliferation and small-celled infiltration which might be marked
when the degeneration was yet trivial, led to the view that there must be
a co-ordinate but independent action of the virus both on the neural elements
and the supporting tissue (McIntosh and Fildes, 1913; Head and Fearnsides,
1914). In view of the fact, however, that there is never degeneration
without granulation tissue and that the latter may be well marked before
the former appeared, Richter (1921) put forward the hypothesis that the
nervous degeneration was purely secondary and was due partly to a disturb-
ance of nutrition owing to the presence of the granulation tissue and partly to
mechanical constriction. In tabes Richter concluded that there were no
cells of haematogenous origin but an overgrowth of fibroblastic cells ; in
general paralysis and tabo-paresis an abundance of haematogenous cells,
DISEASES OF THE OPTIC NERVE 3053
both lymphocytes and plasma cells, invade the tissues and the perivascular
lymph spaces.
So also in the optic nerve, a corresponding number of hypotheses have
been put forward.
1. The original view of a primary neuronic degeneration with secondary
fibrosis has now been generally abandoned. Corresponding to the theories
put forward with regard to events in the cord, two hypotheses were advanced.
The earlier authors thought that the primary degeneration was in the
ganglion cells of the retina and that the atrophy of the nerve-fibres was
consecutive (Gowers, 1883; Popow, 1893; Wagenmann, 1894; Moxter,
1896; Gliksmann, 1900; and others). It is true that extensive degenerative
changes occur in the ganglion cells and the nerve-fibre layer, but it soon
became evident that this hypothesis was untenable, partly because the
retinal changes were quite non-pathognomonic, but indicated rather a
descending atrophy similar to those found after a pressure-atrophy or
section of the nerve, and partly because they were absent in the earlier
stages of the disease, and even in the presence of complete atrophy of the
nerve were usually less than the changes in the nerve, and when present
were always strictly confined to regions corresponding to atrophic parts
of the nerves. Accordingly it was propounded that the primary degeneration
occurred in the nerve-fibres, and that the changes in the ganglion cells were
secondary, a view widely accepted for many years by reason of its advocacy
by Uhthoff (1903) and Wilbrand and Saenger (1913).
2. That a primary peripheral and interstitial neuritis followed by a secondary
degeneration of the nerve-fibres was the essential pathology was first widely
advocated by Léri (1904) and Marie and Léri (1905), and became established
by the elaborate investigations of Stargardt (1911–13), Spielmeyer (1914–25),
Palich-Szanto (1917), Richter (1921), Fujiwara (1925), Igersheimer (1921–25),
Behr (1926) and Ingvar (1927). Elschnig (1899) considered that the nerve-
fibres were strangulated at the disc, and Schlagenhaufer (1897) at the optic
foramen, but the great majority of writers favour Léri’s (1904) original
conclusion that the process starts as an interstitial neuritis, a syphilitic
cirrhosis of vascular origin and a syphilitic meningitis. Both Stargardt
(1911–13) and Richter (1921) concluded that the earliest changes were
exudative in nature, in which lymphocytes and plasma cells predominate.
This is mainly perivascular in the pial sheath and extends into the nerve
along the septa associated with this sheath, while the subsequent degenera-
tion of the myelin sheaths and axis cylinders seems to progress contempo-
raneously, to be followed by an astrocytic glial proliferation. On the other
hand, Behr (1926–36) considered that chronic sclerotic changes in the
lymphatics and glial system were the immediate cause of the atrophy of the
nerve-fibres. Which of the two hypotheses is correct—the meningeal or the
glial—is still controversial.
3. That there is a parallel parenchymatous and interstitial degeneration,
3054 TEXT-BOOK OF OPHTHALMOLOGY
running contemporaneously but independently, was advocated by Paton
(1922), largely from the clinical point of view. In this way he explained
the variability of the fields, the rapid diffuse loss of function denoting a
parenchymatous defect, and the circumscribed losses denoting interstitial
lesions outside which the parenchyma functioned normally. It will be
remembered that both in paresis and tabes the changes in the optic nerve
are very similar and seem to be essentially those of an exudative process
of haematogenous origin derived essentially from the pia : in this respect in
both cases they resemble the changes occurring in general paresis rather
than the reaction in the spinal cord in tabes, and in the former both paren-
chyma and interstitial tissue are attacked by the virus. In general paralysis
the exudative process spreads from the brain to the Optic nerve ; in tabes
it is an isolated phenomenon. The peculiar reaction of this nerve may, as
Richter (1921) suggests, be due to the presence in it of a supporting
structure of ectodermal origin while that of other nerves is mesodermal.
In the posterior roots the pia is unrepresented and the granulation tissue
described by Nageotte (1894–1906) is of mesodermal origin and spreads
readily in a mesodermal framework; in the optic nerve the primary changes
are in the pia and, as in dementia paralytica, the changes are essentially
exudative and vascular in origin. It must be admitted, however, that the
relation between the proliferative and degenerative processes is as yet
unknown. -
However this may be, there is no regularity in the localization and
spread of the degenerative process, and all sorts of variations are possible
and occur. As one would expect, they are seen primarily and most markedly
in the periphery of the nerve and usually in its intra-cranial portion just
distal to the chiasma itself (Stargardt, 1913) or in the orbital portion
(Igersheimer, 1918). From the periphery the atrophic process proceeds
both in a longitudinal and an axial direction, producing a uniform and
sometimes a patchy degeneration (Igersheimer, 1926), but in practically all
cases a slowly progressive one.
The prognosis of para-syphilitic optic atrophy is bad, for despite the
fact that the progress of the disease in isolated cases may become arrested,
the usual outcome is blindness within a period of 2–3 years; despite the
most efficient treatment any persistence of vision beyond 5–8 years is
exceptional. Even in the rare cases wherein progress becomes stayed,
any actual atrophy which has occurred is, of course, permanent. It follows
that a most unfortunate circumstance is the insidiousness of its onset, for,
since the early concomitant signs and symptoms of neuro-syphilis are so
slight that they usually escape the notice of both the patient and the
physician, the former does not report until his vision is failing, at which time
atrophy is usually far advanced. The prognosis of the general disease is also
bad. Before the introduction of malarial treatment, dementia paralytica
was almost invariably fatal within 3 years : this method of treatment has
DISEASES OF THE OPTIC NERVE 3055
considerably improved the general outlook, but the visual prognosis has not
been equally improved. Tabes, however, is extremely variable in its rate
of progress, and although some cases show a rapidly progressive course, it
is not uncommon for patients to linger on for many years : even in those
cases, however, the optic atrophy, when progressive, invariably ends in
blindness.
Treatment. The treatment of this type of Optic atrophy is an unusually
unsatisfactory and melancholy record of failure. In all cases, no matter
what treatment is tried, atrophic fibres cannot be regenerated ; no treatment
is therefore of value unless it is given early before extensive atrophy has
become established. The best treatment, indeed, is prophylazis, which
should include the intensive and prolonged treatment of every patient with
syphilis at an early stage, the early diagnosis and treatment of neuro-syphilis
which can only be attained by the periodic routine examination of syphilitic
patients by an expert acquainted with its somewhat obscure early signs,
and, so far as the eye is concerned, the periodic perimetric examination of
patients with a history of infection of some years’ standing. So notoriously,
indeed, does the disease progress despite treatment that some authorities
decry its value entirely (Behr, 1936). Such an attitude seems, however,
unjustified, for, although a worsening of the disease may in some cases
follow treatment, the evidence is that in the majority of patients its progress
can at any rate be delayed, and in a few exceptional cases the vision
can be temporarily improved or held stationary for a considerable number
of years.
Thus Lehrfeld and Gross (1937) examining the records of 552 cases, found that
in those who received no treatment, blindness ensued in less than three years in 74%,
and within five years in 100%. Among those who had general anti-syphilitic treatment,
24% became blind in less than three years and 100% within eight years. It is an
interesting commentary on the more specialized methods of treatment that among
those who received fever and sub-dural therapy, 28% were blind within three and 100%
were blind within eight years.
A general course of vigorous anti-syphilitic treatment should therefore
be undertaken in all cases, and it is probably safest to place greatest reliance
upon iodides and mercury or bismuth. Arsenic is frequently given in addi-
tion but its advisability is very debatable : the trivalent compounds are
safer than the pentavalent, and although some authorities claim that
tryparsamide gives the best results and does no harm (Mayer, 1934–38),
others consider it dangerous and neo-arsphenamine safer (Gifford, 1932).
We know, however, that arsenic is not without its risks," and since any
evidence of its special value is extremely unconvincing, it is probably
better omitted, and if attempted, should be immediately discontinued
1 p. 3028.
T.O. —W () I... l II. 3 Q
3056 TEXT-BOOK OF OPHTHALMOLOGY
on the first sign of any general sensitivity or deterioration in the visual
fields.
It is only natural that in a disease which tends to progress grimly to
inevitable blindness, many methods of therapy should have been tried.
In view of the inaccessibility of the central nervous system to drugs given
orally or intravenously, Behr (1916), Dercum (1920), and Reid (1929)
attempted to increase the concentration of the drug by intermittently
draining the cerebro-spinal fluid during arsenical treatment, but the results
have been disappointing (Stokes and Osbourne, 1921 ; and others). A
more direct approach was the sub-dural injection of drugs such as Salvar-
sanized serum, arsphenaminized serum, mercuric bichloride, or of air. They
were given at first by spinal injections, originally by Sicard (1913) who
employed mercury, and Swift and Ellis (1913) who used the patients’ own
serum some minutes after he had received an intra-venous injection of
salvarsan. With the Swift-Ellis method some good results were reported,
but this technique has been largely dropped in favour of intra-ventricular
or preferably intra-cisternal injections. For this Schoenberg (1916) used
salvarsanized serum, and Gifford (1923–32), Keegan (1923), Suker and
Jacobson (1933) and others, mercuric bichloride. Since the resulting
meningeal irritation probably increases the permeability of the meninges
to drugs already present in the blood (Sicard, 1913 ; Mehrstens and
MacArthur, 1919), intravenous arsenic is usually given at the same time ;
but the reaction of headache and vomiting is considerable, and the results
by no means uniformly good. Sub-dural injections of air were employed
by Fazakas and Thurzo (1927), Fazakas (1929), Löwenstein (1929), and
others.
Another method of approach has been fever therapy by intra-venous
typhoid, intra-muscular oil of sulphur (Weinberg, 1932 ; Busacca, 1933),
or intra-venous sulfosin (Larsen, 1939), artificial pyrexia induced by the
hypertherm of Kettering (Culler and Simpson, 1936), electro-pyrexia by
short waves (Schiff-Wertheimer, 1932), and malaria (see Hambresin, 1937).
The introduction of infection by benign tertian malaria is probably the most
efficient of these methods, and since its introduction by Wagner von Jauregg
(1918) in the treatment of general paralysis, a proportion of cases of this
hitherto intractable disease has become distinctly amenable to treatment.
The malaria is effective by reason of the pyrexia which it induces, partly
owing to increased permeability of the vessels to drugs and anti-bodies,
partly to a general stimulation of the reticulo-endothelial system, and
possibly because the anti-bodies to the parasite may be effective in some
degree against the treponema. In any event, after such treatment in
paralytic dementia, the treponemata disappear from the brain and the
exudate diminishes, while in acute forms of tabes some amelioration may
result. So far as optic atrophy is concerned, however, while some authors
have reported improvement in the fields or retardation of visual deterioration
DISEASES OF THE OPTIC NERVE 3057
(Hessberg, 1930; Clark, 1930–36; Fleischer, 1933; Weskamp, 1935;
Heinsius, 1935; and others), the results have frequently proved very
disappointing, and sometimes apparently disastrous (Behr, 1926 : Iger-
sheimer, 1926; Gasteiger, 1934; and others).
A third method of approach is to increase the nutrition of the nerve
by increasing the efficacy of the circulation. The first to advocate the import-
ance of vaso-motor insufficiency as a factor in determining the progress of
the optic atrophy was Abadie (1924), who employed atropine in a retro-
bulbar injection as a vaso-dilatator. This method, or atropine as a systemic
injection, has received a considerable trial, sometimes with good temporary
results (Gapeeff, 1930 ; Springovitsch. 1933–36; Kriloff, Levin and
Tropishko, 1934; Cordes, 1937 : Folk, 1937), and sometimes with no
effect (Fejer, 1936). Retro-bulbar injections of acetyl-choline have also
been tried (Springovitsch, 1933–36). An increased circulation and vaso-
dilatation in the nerve has also been stimulated by decompression operations
on the globe, as by repeated paracenteses or sclerectomy (Deutschmann,
1924; Friede, 1924; Gilbert, 1924; Vele, 1933; Folk, 1937). A cervical
sympathectomy was advised by Magitot (1934) to bring about a vaso-dilatation,
but others have found the method useless and unjustifiable (Löwenstein,
1935; Blobner, 1937). The whole conception of improving the local
circulation has been most enthusiastically advocated and practised by
Lauber, (1935–38) and Sobanski (1935–38), who found that as a rule the
blood-pressure in tabetics was low and that the diastolic pressure in the
retinal artery relative to the intra-ocular pressure was not of a level sufficient
to maintain the local tissue nutrition. To redress the balance these workers
recommended systemic measures to increase the blood-pressure (tonics,
caffein, strychnine, ephedrine, mountain air, etc.) and local measures to
reduce the intra-ocular pressure (miotics, cyclodialysis). Their methods have
received extensive trial, Arruga (1936), Miklos (1937), and Rintelen (1937)
reporting good results, and Langhammerova (1937), Ascher (1937), Albrich
and Kukan (1938), Müller and Brüning and Sohr (1938) reporting failures.
It would seem necessary to admit that a local improvement of the circulation
must be a good thing in an attempt to preserve the vitality of the nerve,
but all the evidence points to the conclusion that the beneficial effects are
meagre.
On the whole the treatment of syphilitic optic atrophy is a disappointing
affair, and despite all the ingenuity which has been expended upon it, the
problem has proved insurmountable.
Abadie. Am. d’Oc., clx, 385, 1923. Behr. K. M. Aug., lv., 449, 1915; lvi, l,
La Clin. O., xiii, 247, 1924. 1916.
Albrich and Kurkan. K. M. A ug., c, č45, Münch. med. W., lxxiii, 311, 365, 1926.
1938. Zb. ges. O., xvii, 423, 1926.
Arlt. Z. f. Aerztl. Fortbild... xix, 367, 1922. Der Augenbefund . . . bei Tabes dorsalis,
Arruga. K. M. Aug., xcvii. 308, 1936. Lues cerebrospinalis, usu., Berlin, 1936.
Artz and Kerl. Wien. kl. W., xxvii. 787, 1914. Berger. Rev. gen. d’O., viii, 193, 1889.
Ascher. K. M. Aug., xcviii, 398, 1937. A. f. Aug., xix, 305, 391, 1889.
3 Q 2
30.58 TEXT-BOOK OF
OPHTHALMOLOGY
Biffis. Am. di Ott., lxv, 161, 1937.
Blobner. K. M. Aug., xcviii, 289, 1937.
Busacca. K. M. Aug., xc, 352, 1933.
Carr. Am. J. Syph., xiii, 360, 1929.
Clark. Am. J. O., xiii, 946, 1930.
T. Am. O. S., xxxii, 452, 1934.
A. of O., xv, 250, 1936.
Cordes. Am. J. O., xx, 53, 1937.
Culler and Simpson. A. of O., xv, 624, 1936.
Dercum. A. of Newrol. and Psy., iii, 230, 1920.
Deutschmann. Z. f. Aug., liii, l, 1924.
Dodd. T. O. S., xix, 281, 1899 ; xx, 264,
1900.
Drake. A. of O., ix, 234, 1933; xii, 583, 1934.
Elschnig. Wien. kl. W., xii, 257, 1899.
Fazakas. K. M. Aug., lxxxiii, 297, 1929.
Fazakas and Thurzo. K. M. Aug., lxxviii,
644, 1927.
Fehr. Med. Kl., viii, 942, 1912.
Fejer. A. f. Aug., cz, 76, 1936.
Finger. Med. Kl., vii, 1750, 1911.
Flechsig. Neurol. Zb., ix, 33, 72, 1890.
Fleischer. K. M. Aug., xc, 335, 1933.
Folk. Am...'.J. O., xx, 511, 1937.
Förster. Am. J. O., 257, 1900.
Friede. Z. f. Aug., lii, 99, 1924.
Fuchs, A. Z. f. Aug., lix, 213, 1926.
A. de Oft., B.A., vii, 279, 1932.
Fuchs, E. A. of O., x1, 469, 1911.
Fujiwara. A. f. O., czv, 562, 1925.
Galezowski. These, Paris, 1904.
Gapeeff. K. M. Aug., lxxxv, 203, 1930.
Gasteiger. K. M. Aug., lxxviii, 827, 1927.
A. f. Aug., lviii, 471, 1934.
Gifford. Brit. J. O., vii, 506, 1923.
Handbook of Ocular Therapeutics, London,
I932.
Gilbert. Z. f. Aug., liii, 344, 1924.
Glass and Garvey. Am. J. O., xi, 377, 1928.
Gliksmann. Diss., Freiburg, 1900.
Goldberg. Sov. vest. O., vi, 508, 1935.
Gowers. T. O. S., iii, 193, 1883.
Medical Ophthalmoscopy, London, 181, 1904.
Hambresin. An. d’Oc., clxxiv, 721, 1937.
Head and Fearnsides. Brain, xxxvii, 1, 1914.
Heed and Price. J. A. m. Med. As., lxii, 771,
1914.
Heinsius. Z. f. Aug., lxxxvii, 298, 1935.
Hessberg. K. M. Aug., lxxxiv, 261, 1930.
Hutenil. Rev. gen. de clin., xxvii, 437, 1913.
Igersheimer. Syphilis u. Auge, Berlin, 1918.
Deut. med. W., xlvii, 738, 1921 ; lii, 943,
1926.
B. O. G. Heidel.., xliv, 99, 1924; xlv., 5, 1925.
Ingvar. Acta Med. Scand., lxv, 645, 1927.
v. Jauregg. Psy. Newrol. W., xx, 132, 1918.
John. Z. f. Aug., lxix., 283, 1929.
Juler. A. d’O., xvii, 542, 1897.
Keegan. Brit. J. O., vii, 522, 1923.
Kiep. T. O. S., xliv, 379, 1924.
Kriloff, Levin and Tropishko. Sov. vest. O.,
v, 230, 1934.
Lang and Beevor. T. O. S., xiv, 246, 1894.
Langenbeck. K. M. Aug., l (2), 148, 1912.
Langhammerova. Cechosl. Oft., iii, 101, 1937.
Larsen. Brit. J. O., xxiii, 585, 1939.
Lauber. Wien. kl. W., xlviii, 1079, 1935.
Z. f. Aug., lxxxvii, 65, 1935.
A. of O., xvi, 555, 1936.
T. O. S., lviii, 661, 1938.
K. M. Aug., c, 108, 1938.
Lavergne. A. de Méd. d. emf., xix, 579, 1916.
Lawford. T. O. S., xxxvi, 10, 1916.
Lehrfeld and Gross. A. of O., xviii, 666, 1937.
Léri. Nouvelle Iconogr. de la Salpêtrière, xvii,
304, 1904.
Löwenstein. K. M. Aug., lxxxiii, 548, 1929;
xciv, 705, 1935.
A. f. O., czkxiii, 636, 1935.
Lutz. A. f. O., czix, 423, 1928; cxxv, 103,
1930.
Magitot. Bull. S. d’O. Paris, xlvi, 358, 1934.
An. d’Oc., clxxi, 897, 1934.
Maloney. J. of Nervous and Mental Dis., x1,
572, 1913.
Marie and Léri. Rev. Neurol.., xii, 199, 1904 ;
xiii, 246, 1905.
Matsukawa. K. M. Aug., li (2), 665, 1913.
Mayer. Ill. Med. J., lxv, 258, 1934.
A. of O., xix, 307, 1938.
Mazzeo. Pediatria, xxxiv, 299, 1926.
McIntosh and Fildes. Brain, xxxvi, l, 1913.
Mehrstens and MacArthur. A. of Newrol. and
Psy., ii, 369, 1919.
Miklos. A. f. O., czzxviii, 219, 1937.
Mott. Arch. of Neurol., ii, 30, 1903.
Moxter. Z. f. kl. Med., xxix, 334, 1896.
Müller, Brüning and Sohr. K. M. Aug., c,
112, 1938.
Nageotte. Bull. S. Biol., i, 713, 1894.
A. de Newrol.., xxx, 273, 1895.
C. R. S. de Biol., 738, 1899; 354, 1900;
1443, 1082, 1902.
Pathogenie du Tabes dorsal, Paris, 1903.
Nouvelle Iconogr. de la Salpêtrière, xvii, 17,
1904 ; xix, 217, 1906.
Neumann. Zb. f. inn. Med., xxxix, 609,
1918.
Noguchi. J. Am. Med. As., lxi, 85, 1913.
Nonne. Syphilis u. Nervensystem, Berlin,
1921.
Opin. A. d’O., li, 193, 1934.
Orr and Rows. Brain, xxxvi, 271, 1903 ;
xxxvii, 460, 1904.
Palich-Szanto. A. f. Aug., lxxxii, 48, 1917.
Parsons. Pathology of the Eye, ii, 683. 1905.
Paton. Brit. J. O., vi, 289, 1922.
Pfister. Z. f. ges. Neurol. Psy., ciii, 455, 1926.
Popow. Deut. Z. f. Nervenh., iv, 270, 1893.
Reid. Lancet, i, 916, 1929.
Richter. Z. ges. Neurol. Psy., lxvii, l, 1921.
Rintelen. Schw. med. W., ii, 791, 1937.
Rönne. A. f. O., lxxii, 481, 1909.
K. M. Aug., xlix, 154, 1911; 1, 452, 1912.
Rutgers. K. M. Aug., lxxii, 8, 1924.
Rutherford. T. A m. O. S., xxxiii, 81, 1935.
Schiff-Wertheimer. Sp. Rapport d S. d’O.,
Paris, xliv, 1932.
Schlagenhaufer. Jb. f. Psy. Neurol.., xvi, l,
1897.
DISEASES OF THE OPTIC NERVE
3059
Schoenberg. J. Am. Med. As., lxvi. 2054,
1916.
Sharkey and Lawford. T. O. S., iv. 232, 1884.
Sicard. J. Méd. Franç., 207, 1913.
Slinger and Horsley. Brain, xxix. 26, 1906.
Sloan and Woods. A. of O., xx, 201, 1938.
Sobanski. Kl. Ocz., xiii, 239, 1935; Xiv, 358,
1936; xv. 34, 670, 1937; xvi. 44, 1938.
A. f. O., exxxv, 372, 383, 401, 1936.
K. M. Aug., xevii. 1, 1936.
Spielmeyer. K. M. Aug., xliv (1), 96, 1906.
Z. ges. Neurol. Psy., xxv, 562, 1914 :
xovii, 287, 1925.
Springovitsch. K. M. Aug., xe, 343, 1933;
xcvi, 342, 1936.
A. of 0., xi, 1074, 1933.
Lancet, ii, 78, 1930.
Ill. Med. J., lxiii, 21.
Ström-Olsen.
Suker and Jacobson.
1933.
Swift and Ellis.
2054, 1913.
A. Int, Med., xii, 331, 1913.
Terrien and Prélat, A. d’O., xxxiv, 23, 1914.
Uhthoff. A. f. O., xxxix (1), 1 ; (3), 126,
1893 : x1 (1), 1894.
G.-S. Hb., II, xi (2b), 1039, 1903.
Vele. A. di Ott., x1, 203, 1933.
Verhoeff. K. M. Aug., xlviii (2), 315, 1910.
Wagenmann. A. f. O., x1, 256, 1894.
Wagner. K. M. Aug., xli (2), 1, 1903.
Weinberg. Deut. med. W., i, 173, 1932.
Werneke. Z. f. Aug., xxix. 434, 1913.
Münch, med. W., lx, 1977,
Stargardt. B. O. G. Heidel. xxxvii. 139, Weskamp. An d'Oe., clºxi, 449, 1935.
1911; xxxviii, 214, 1912. Wilbrand and Saenger. Neurologie d. Auges,
A. f. Psy, li, 711, 1913. v. 585, 1913.
Stock. K. M. Aug., xliii (1), 640, 1905. Wilson. Brain, xxxv, 173, 1912.
Stokes and Osbourne. J. Am. Med. As., Wilson and Gray, Brit. Med. J., ii, 419, 1917.
lxxvi, 708, 1921. Zimmermann. Z. f. Aug., xiv. 362, 1905.
Stroebe. Cb, allg. Path., v, 853, 1894.
IV. DEGENERATIONS
A. Degenerative Depositions
1. ARACHNOID CELL NESTS
The formation of ARACHNoid cºll, NESTs by a proliferation of the mesodermal
cells lining the outer surface of the arachnoid to form oval clusters which eventually
become permeated with lime salts was first noted by Meyer (1859) (Fig. 2619). These
Fig. 2619.-ARACHINoid CELL NEST.
In arachnoid of human optic nerve (Cone and MacMillan).
areas of thickening of the arachnoid membrane may be said to be a normal physio-
logical phenomenon (Essick, 1920); in cats, for example, they are never found in the
young, occasionally in adults, and constantly in very old animals in which they
regularly become impregnated with lime salts (Cushing and Weed, 1915; Weed, 1920):
occasionally they proliferate to form a fibroblastomatous tumour."
p. 3093.

3060 TEXT-BOOK OF OPHTHALMOLOGY
Cushing and Weed. Johns Hopkins Hosp. Meyer. A. f. kl, Med., xvii, 209, 1859.
Bull.., xxvi, 297, 1915. Weed. Johns Hopkins Hosp, Bull.., xxx, 347,
Essick. Carnegie Instit. Wash. Publ. 1920.
Nos. 22, 379, 1920.
2. corpora AMYLACEA
Corpora amylacea, or amyloidea, may also be said to form a normal constituent
of the central nervous system : they are constant in the optic nerves of the cat and
Fig. 2620–Corpus AMy Laceum.
In arachnoid sheath of optic nerve, infiltrated with calcium salts (x 200)
(Parsons).
Fig. 2621. Corpus Amylaceum is Rºrisa.
Lying in the nerve-fibre layer, in a patient aged 64, whose eye was excised for
septic irido-cyclitis following a wound tº 260) (Coats, T. O. S.).


DISEASES OF THE OPTIC NERVE 3061
dog, and are common in old people, especially in atrophic nerves. Here they are
usually found in the intra-cranial portion of the nerve and the chiasma, lying some-
times within the bundles of nerve fibres, sometimes in the septa between the bundles
and sometimes in the sheaths (Fig. 2620); they are also found in the disc and more
rarely in the nerve-fibre layer of the retina (Fig. 2621) (Leber, 1873–79; Sigert, 1892;
Pusey, 1904: Best, 1905; Coats, 1912; Fuchs, 1920; and others). Fuchs (1920)
found them in an atrophic nerve stump two years after enucleation of the eye.
They are round or oval, highly refractile, homogeneous bodies, 6–25u in diameter,
which do not give the characteristic amyloid staining reactions, rarely showing any
traces of lamination, but are provided with a definite capsule: in their later stages they
may exceptionally become impregnated with calcium salts. Leber (1873), who originally
contended that they were derived from degenerated nerve-fibres, considered later
(1879) that they originated as an amyloid degeneration of neurolgial cells, a view
supported by Coats (1912); while Sigert (1892) suggested that they represented a
combination of free myelin with the surrounding tissue-fluid.
Best. K. M. Aug., xliii (1), 240, 1905. Leber. A. f. O., xix (1), 191, 1873; xxv (1),
Coats. T. O. S., xxxii. 119, 1912. 257, 1879.
Fuchs. A. f. O., ciii, 304, 1920. Pusey. K. M. Aug., xlii (2), 561, 1904.
Sigert. A. f. path. Anat, exxix, 583, 1892.
3. CORPORA ARENACEA
Corpora arenacea are usually found in the dura or in tumours of the meninges,
forming the “sand” in psammomata. They occur with considerable frequency in the
sheaths of the optic nerve lying usually on the inner aspect of the dura or the outer
- - - - - -
- - - -
- - - - - -
- - - - --- - - -º- -
Fig. 2622–Corpora ARENAcEA IN Optic NERVE-sheath.
(x 120) (Coats, T. O. S.).
aspect of the arachnoid (Fig. 2622). They vary considerably in size, being usually
larger than corpora amylacea, and grow by the deposition of successive layers. They are
definitely laminated in a wavy manner from their centre outwards, and they have no
capsule; practically invariably they become calcified (Sigert, 1892; Coats, 1912).
Coats. T. O. S., xxxii, 119, 1912. Sigert. A. f. path. Anat... cxxix., 583, 1892.

3.062 TEXT-BOOK OF OPHTHALMOLOGY
4. HyALINE Bodies (DRUSEN)
Hyaline bodies upon the optic disc (Drusenbildung, granular formations)
are rare, but present a typical and striking ophthalmoscopical picture. The
dise may be swollen, sometimes enormously so, up to 14 dioptres, and may
suggest the appearance of optic neuritis; at other times discrete, white
Fig. 2623–DRusºn AT THE OPTIc Disc.
A man aged 51; vision normal; no symptoms. The granular mass projects
forwards 2D (R. R. James, T. O. S.).
Fig. 2624–Coxcºrtion is The Papilla (Coats, T. O. S.).


DISEASES OF THE OPTIC NERVE 3063
and shiny beady-looking concretions lie upon its surface, either scattered
about or piled up into large masses of grape-like clusters, sometimes
greater than the diameter of the disc itself, and projecting exuberantly
into the vitreous (Fig. 2623) (Viner, 1918; Plummer, 1935). They may be
situated in the centre of the disc, sometimes they lie at the periphery, and
occasionally they are scattered all over it. They are usually bilateral, and
may occasionally be associated with similar formations at the macula or
elsewhere on the retina (de Schweinitz, 1894; Heyl, 1895; Walker, 1915;
and others). They occur at all
ages, the youngest reported case -
being 8 years old, but are more
common in the aged and in the
presence of retinal disease (Fig. 2635,
Plate LXII). They may remain
for years with little change, and if
they are situated superficially may
give rise to few or no symptoms;
if, however, they lie deeply in the
sclero-choroidal canal they may
produce a pressure-atrophy of
the contiguous nerve fibres with
resultant changes in the visual
fields (Juler, 1914 : Soriano and
Picoli, 1935; and others) (Fig. 2624).
These hyaline bodies were first
found microscopically by Heinrich
Müller (1858) and Iwanoff (1868), while
Liebreich (1868) first observed them
ophthalmoscopically. Thereafter a
number of cases appeared in the
literature (Nieden, 1878; Jany, 1879;
Lawson, 1883; Story, 1883; Stord,
1883; and others), and the condition,
although rare, became generally recog- (Tones, Am. J. O.).
nized : Parsons (1903) collected 42 cases
from the literature, Hoëg (1909) 78 cases observed clinically and 21 studied histo-
logically, while the later literature has been reviewed by Wetzel (1911), Lauber (1921),
Tobler (1922) and Jensen (1935). Since the original pathological investigations of
Müller and Iwanoff a considerable number of histological examinations have been
made, the most important of which are those of Oeller (1879), Hirschberg and Cirincione
(1891), Gurwitsch (1891), de Schweinitz (1892), Sachsalber (1898), Morton and Parsons
(1903), Demaria (1904), Wetzel (1911), Coats (1912), Lauber (1921), Tobler (1922),
and Goldstein and Givner (1933).
FIG. 26.25-Col. LoID Body IN OPTIC NERVE
Pathologically these bodies are made up of concentric laminations
with no cellular structure or capsule, and frequently showing masses

3064 TEXT-BOOK OF OPHTHALMOLOGY
of calcification (Fig. 2625). The great majority of observers agree that
their basis is a hyalin-like material : Tobler (1922) found them insoluble
in water, alcohol, ether, acetic acid, and potassium and sodium hydroxide,
and concluded that they represented a split produgt of albumen, related to
hyalin, which has a tendency to take up calcium. They are associated with
no surrounding inflammatory reaction, but while a great number of the
eyes in which they occur are healthy, others have been pathological.
Retinitis pigmentosa is the most commonly associated condition, and they
have also been noted in cases of post-neuritic atrophy, renal retinopathy,
chorio-retinitis, glaucoma, or old injury. It is not clear, however, how
close the association is with any of these conditions. It is interesting that
in the literature there are ten instances of a hereditary tendency (Leimgruber,
1936).
The pathogenesis of these depositions is not yet fully elucidated.
Müller (1858) regarded them as outgrowths of Bruch's membrane, a view
which in time became modified to the theory that, like Bruch's membrane,
they were derived from pigmentary epithelium, in this case displaced into
the disc, and corresponded therefore with the colloid bodies of the choroid 4
(Lauber, 1921 ; Löhlein, 1931). A. Fuchs (1927), on the other hand, con-
sidered them to be of neural origin, a view supported by their frequent
close association with neuroglia (Goldstein and Givner, 1933). Parsons
(1905) concluded that while some of them might be of cellular origin, others
originated from exudates laid down in the nerve-head, probably from a
previous inflammatory process which may have left no evidence of its
existence. All that can be said is that they seem to represent the deposition
of a hyalin-like material because of some local metabolic disturbance of
unknown and probably varied aetiology.
Coats. T. O. S., xxxii, 119, 1912. Müller. A. f. O., iv (2), 12, 1858.
Demaria. K. M. Aug., xlii (1), 339, 1904. Nieden. Cb. pr. Aug., ii, 6, 1878.
Fuchs, A. Atlas d. Histopath., Vienna, 1927. A. f. Aug., xx, 72, 1889.
Goldstein and Givner. A. of O., x, 76, 1933. Z. f. Aug., iii, 361, 1900.
Gurwitsch. Cb. pr. Aug., xv, 225, 1891. Oeller. A. f. Awg., viii, 449, 1879.
Heyl. T. Am. O. S., ix, 355, 1895. Parsons. Path. of the Eye, London, ii, 661,
Hirschberg and Cirincione. Cb. pr. Aug., xv, 1905.
166, 198, 1891. Plummer. Brit. J. O., xix, 215, 1935.
Hoég. A. f. Ohrenh., lxix, 355, 1909. Sachsalber. Beit. z. Aug., iii, 1, 1898.
Iwanoff. K. M. Aug., vi, 425, 1868. de Schweinitz. T. Am. O. S., vi, 349, 1892;
Jany. Cb. pr. Aug., iii, 167, 1879. viii, 212, 1894.
Jensen. A. of O., xiv, 269, 1935. Soriano and Picoli. A. de Oft. B. A., x, 685,
Juler. T. O. S., xxxiv, 177, 1914. 1935.
Lauber. Cb. pr. Aug., xxxi, 144, 1907. Stord. K. M. Aug., xxi, 506, 1883.
A. f. O., cv, 567, 1921. Story. T. O. S., iii, 102, 1883.
Lawson. T. O. S., iii, 117, 1883. Tobler. Z. f. Aug., xlvii, 215, 1922.
Leimgruber. A. f. O., cxxxvi, 364, 1936. Viner. Brit. J. O., ii, 426, 1918.
Liebreich. K. M. Aug., vi, 426, 1868. Walker. T. O. S., xxxv, 366, 1915.
Löhlein. K. M. Aug., lxxxvi, 433, 1931. Wetzel. Ueber Drusenbildung, Rostock,
Morton and Parsons. T. O. S., xxiii, 135, 1911.
1903.
DISEASES OF THE OPTIC NERVE 3065
AMYLOID DEGENERATION
A unique case was described by Coats (1915) of very extensive amyloid disease
affecting primarily the conjunctival and sub-conjunctival tissues 1 and subsequently
spreading to the orbital tissue and involving also the dural sheath of the optic nerve
FIG. 26.26.-Colloid AND AMYLoID DEGENERATION.
Transverse section of the optic nerve, which is atrophic. The trabeculae of the
nerve, the pia, arachnoid and inter-vaginal space are unaffected, but the dura is
enormously thickened (Coats, T. O. S.).
(Fig. 2626). This structure was enormously thickened with amyloid material, and
although the pia-arachnoid and the inter-vaginal space were not invaded, the nerve
itself became atrophic.
Coats. T. O. S., xxxv, 257, 1915.
B. Wascular Degenerations.
Senile changes occur in the blood-vessels supplying the optic nerve as
in other tissues, comprising essentially the interposition of a sub-endothelial
layer between the intima and the elastic lamina ; this appears first as a
sparsely nucleated, almost homogeneous stratum, which later becomes heavily
thickened with elastic tissue (Hertel, 1901) (Figs. 2627–28). Upon this the
changes characteristic of atherosclerosis may be superimposed, either patchy
or diffuse in its incidence (Coats, 1904). We have seen that the common
occurrence of endarteritis obliterans in the central vessels just as they traverse
* Vol. II, p. 1748.

3066 TEXT-BOOK OF OPHTHALMOLOGY
the lamina cribrosa is responsible for the common incidence of arterial and
venous obstruction at this point, but a widespread obliterative process in
the small vessels supplying the nerve may be responsible for the development
of an arteriosclerotic optic atrophy which may progress to blindness.
When the central artery itself is occluded, extensive atrophy occurs
(Abelsdorff, 1924); while in other cases, occlusion of the small feeding
vessels from the sheaths produces a patchy degeneration (Henschen, 1911:
Fuchs, 1920; Igersheimer, 1925). In addition to this atrophy due to
sclerosis of the small nutrient vessels, it is to be remembered that a common
cause of optic atrophy in arteriosclerotic subjects is pressure upon the nerve
by a calcareous internal carotid artery in the region of the chiasma or by the
Fig. 2627-Vascular Sciºtosis. Fig. 26.28. Vascular Sotºrosis.
Two vessels on the papilla. The The central artery, showing a
artery (right) shows much elastic very thick crenated membrane
tissue, a proliferated intima and within which is a felt-work of new-
eccentric narrowing of the lumen. formed elastic fibres (x. 90)
The vein has a verythick wall (x 120) (Coats).
(Coats).
ophthalmic artery in the optic foramen.” It is interesting that the larger
vessels, the internal carotid, the ophthalmic, and the central retinal arteries,
may show advanced sclerotic changes without involvement of the small
vessels of the nerve or its sheaths, while the reverse picture is frequently
seen (Stief, 1929).
Depending on whether the resulting degeneration is diffuse, patchy or
peripheral in distribution, as well as on the extent of the damage done to
the nerve-fibres, the visual loss may take the form of a general depression
of the whole field without characteristic features, a symmetrical peripheral
loss, or, alternatively, sector defects or a central scotoma may appear
(Fuchs, 1920) (Figs. 2629–30). The diagnosis is frequently difficult, but
is usually possible from a consideration of the field defects, the ophthalmo-
p. 2570. * Vol. IV.

IDISEASES OF THE OPTIC NERVE 3067
scopic appearance and other signs of arteriosclerosis occurring in an elderly
patient.
Since obliteration of the blood-supply leads to death of the nerve-
fibres, the prognosis is not good, for any visual loss which may have occurred
tends to be permanent. The most that can be done is to arrest the progress
Left. Right.
FIGS. 2629–30.-ARTERIOSC LEROTIC ATROPHY.
The left field show's peripheral contraction with a centrocaecal scotoma : the
right is retained only in the extreme lower temporal periphery (10330).
of the degeneration so far as that is possible by a carefully controlled cardio-
vascular regime combined with the administration of iodine. Favourable
results have been reported from vaso-dilatatory treatment, as by the retro-
bulbar or intra-venous injection of atropine (Abadie, 1924; Springovitsch,
1933–36; Cordes, 1937 ; and others),' but any dramatic improvement is
out of the question, although the element of spasm, which in some cases
may be considerable, may be alleviated.
Abadie. La ("lin. Opht., xiii, 247, 1924. Henschen. A. f. O., lxxviii, 212, 1911.
Abelsdorff. Z. f. A ug., lii, 273, 1924. Hertel. A. f. O., lii, 191, 1901.
Coats. R. L. O. H. Rep., xvi. 62, 262, 516, Igersheimer. B. O. G. Heidel.., xlv. 5, 1925.
19{}+. Springovitsch. K. M. A ug., xc, 343, 1933 :
Cordes. 4 m. J. O., xx, 53. 1937. XC. vi. 342, 1936.
Fuchs. A. f. O., ciii, 304, 1920. Stief. Z. f. A uſ/., lxx, 41, 1929.
C. Optic Atrophy
The many various conditions which result in optic atrophy form a very composite
group, most of which have already been discussed, while the remainder will be considered
in the chapters dealing with glaucoma and the diseases of the central nervous system.
It is convenient, however, to summarize these conditions here. A perfect classification.
however, completely inclusive and exclusive in its scope, is quite beyond the limits of
our present knowledge.
1 p. 3057.

3068 TEXT-BOOK OF OPHTHALMOLOGY
It is customary to divide the optic atrophies into two types—primary and
secondary or consecutive; of the former the prototype is tabetic atrophy,
showing ophthalmoscopically a simple degeneration of the nerve-fibres
without any complicating processes, while the latter is characterized by
evidences on the disc of an antecedent neuritis or papilloedema. Such a
system of classification is most misleading, for an inflammatory process at
the disc will produce a secondary atrophy while an identical lesion a little
distance up the nerve will produce an apparently primary atrophy. From
a systemic point of view such a classification should be abandoned ; it is
only tolerable when it is remembered—as it rarely is in the literature—that
it is a purely descriptive term applied to the clinical appearance of the
optic nerve-head. So far as these ophthalmoscopic appearances go, six
descriptive categories are more appropriate :-
1. Consecutive atrophy following chorio-retinal disease, typical in the
late stages of pigmentary degeneration of the retina or diffuse chorio-
retinitis, and characterized by a yellow waxy colour of the disc and extreme
attenuation of the arteries (Fig. 2635, Plate LXII).
2. Post-neuritic atrophy, following a papillitis and distinguished by
the presence of post-inflammatory tissue on the disc (Fig. 2562,
Plate LXI). -
3. Temporal atrophy, following retro-bulbar neuritis or compression and
distinguished by a temporal pallor of the disc (Fig. 2634, Plate LXII).
4. Glaucomatous atrophy, distinguished by the presence of cupping
(Fig. 2859).
5. Vascular atrophy, distinguished by extreme attenuation of the
vessels, as occurs in occlusion of the central artery or after quinine poisoning
(Fig. 2633, Plate LXII).
6. Simple or primary atrophy, wherein the entire disc is greyish white
and clear-cut, the vessels are normal, and there are no ophthalmoscopic
evidences of disease except the appearance of the atrophy itself: this is
typified in the atrophy of tabes or that following such poisons as atoxyl
methyl alcohol, etc. (Fig. 2632. Plate LXII).
From the systematic point of view, an aetiological classification such as
the following may be suggested :—
1. Consecutive atrophy, secondary to retinal disease and destruction of
the ganglion cells.
(a) Post-inflammatory—as after a diffuse chorio-retinitis."
(b) Degenerative.
(i) In primary pigmentary degeneration.”
(ii) Part of a systemic degeneration as amaurotic family
idiocy, cerebro-macular degeneration,” etc.
(iii) In myopia.”
1 p. 2633. * p. 2765.
8 p. 2792. 4 Vol. IV.
PLATE LXII
OPTIC ATRoPhy
Fig. 2631.-LEBER's Disease. Fig. 26.32.-PRIMARY SYPHILITIC OPTIC
ATRoPHY.
Fig. 2633.-ATRoPHY IN QUININE AMBLYopia (9 Months
AFTER TARING 60 GRAINs).
Fig. 2634.-CoMPREssion ATRoPHY. Fig. 2635–ATRoPHY witH DRUsex.
(Pituitary tumour). (In primary pigmentary degeneration).
[To face p. 3068.



DISEASES OF THE OPTIC NERVE 3069
2. Circulatory atrophy.
(a) Occlusion of the central artery."
(b) Post-haemorrhagic.”
(c) Arteriosclerotic, producing ischaemic degeneration.”
3. Pressure and traction atrophy.
(a) Glaucomatous.*
(b) Post-papilloedema,” due to swelling and pressure at the
disc.
(c) Arterial, a sclerosed and frequently calcified artery pressing
upon the nerve, either the internal carotid in the region of
the chiasma, or the ophthalmic at the optic foramen.
Occasionally a normal artery displaced by a pituitary
tumour exerts sufficient traction to cause atrophy.
(d) Aneurysms of the internal carotid.
(e) Bony pressure at the optic foramen, as in Osteitis deformans,
oxycephaly, leontiasis ossea, etc.
(f) Tumours, either of the optic nerve sheaths, in the orbit, or in
the cranium, particularly those of the pituitary, the frontal
and temporo-sphenoidal lobes, or basal meningiomata.
(g) Inflammatory adhesions, as in a basal arachnoiditis.
(h) A swelling of the nerve itself so that it is strangulated at
the optic foramen, as in the neuro-fibromatous degenerated
nerve of v. Recklinghausen’s disease.
4. Post-inflammatory atrophy.
(a) Local inflammation, from an optic neuritis, from an intra-
ocular inflammatory process, or from a peri-neuritis derived
from the orbital tissues, the nasal sinuses, or the meninges.
(b) Metastatic infections, as in a septicaemia, tubercle or syphilis.
(c) As part of a central nervous disease, such as disseminated
sclerosis, neuro-myelitis optica, disseminated encephalo-
myelitis, herpes zoster, encephalitis lethargica, tabes,
dementia paralytica, etc.
5. Toa'ic atrophy.
(a) Endogenous toxins," as in diabetes, anaemia, malignant
tumours : hyperthyroidism has produced atrophy
experimentally in dogs.
(b) Exogenous poisoning,” as tobacco, alcohol, arsenic, lead, etc.
6. Traumatic atrophy—by mechanical injury as in avulsion of the optic
nerve, or by lightning, electricity, etc. Also secondary to a fracture of the
skull in the region of the optic canal. -
7. Atrophy of unknown aetiology—Leber's disease.”
1 p. 2561. * p. 2556. * p. 3065.
* p. 3351. * p. 2954. * p. 3004.
7 p. 3008. 8 p. 2998.
3.07.0
TEXT-BOOK OF OPHTHALMOLOGY
From the pathological point of view optic atrophies may be divided
into three types, depending on the nature of the replacement gliosis.
1. As a general rule when the degeneration of the nerve-fibres has been
Fig. 2636,-CAvºn Nous Optic ATRoPHY showing Schwab EL's Cavºr Ns
(after Thiel).
rapid, their replacement by fibrous astrocytes takes place immediately but
follows no special pattern, the fibres of these cells forming a dense tangle
running in all directions (Fig. 2519).
2. When, however, the degeneration of the axons has been slow, the
astrocytes replace them more accurately : their processes, which normally
- º |-
- - - º --> -
- -
--- º -> *
- - - º * -- -
- - º º:
--
- - -
--- - . ". - ---
* > ** - - - - -
--- - - ---
Fig. 2637.-CAvºn Nous Optic Atrophy
showing SoHNABEL's Caverns
(x 200) (after Thiel).
run at right angles to the nerve-
fibres, tend to take the actual
course of the nerve-fibres and to
run longitudinally, thus producing
the columnar gliosis described by
Spielmeyer (1906) in tabes (Fig.
2520).
3. Cavernous (or lacunar)
atrophy, wherein in the earlier
stages there is atrophy of the
nerve-fibres without a proliferative
replacement of neuroglia, so that
empty spaces are seen histologically in the nerve between the connective
tissue septa (Figs. 2636–37).
This condition differs from other types of
atrophy in that gliosis does not proceed parallel with and compensate for
the atrophy.
particularly with chronic glaucoma.
It was first described by Schnabel (1892), who associated it
It occurs, however, also in conditions

DISEASES OF THE OPTIC NERVE 307 I
wherein there is no rise in the ocular tension, particularly in myopia (Axenfeld,
1905 ; Ogawa, 1912) and in orbital tumours (KOyanagi, 1930), in all of
which conditions the nerve-fibres are liable to stretching and even mechanical
rupture.
Cupping of the Disc and Optic Atrophy : Pseudo-glaucoma
Owing to the disappearance of the bulk of the nerve-fibres, optic atrophy
is frequently associated with a considerable amount of cupping of the disc,
more especially—although probably not exclusively—in cases wherein a
physiological cup existed previously (Elschnig, 1908). Such a cupped disc
is characterized by a white floor, in which the lamina cribrosa is
visible over the extent of the previous physiological cup, it is total in the
sense that it corresponds in extent to the area of the entire disc, but it is
rarely deep, and is usually saucer-shaped and without excavated borders.
Occasionally, however, it is deep with overhanging edges simulating in
every way a glaucomatous cup, constituting a problematical type of case
showing a glaucomatous excavation of the nerve-head but without raised
intra-ocular tension. The difficulties of the diagnosis and pathogenesis are
rendered more acute by the fact that such a condition may be progressive
and may show field defects (nasal step, Bjerrum scotoma, etc.) closely
simulating a true chronic glaucoma.
Such cases were first recognized by v. Graefe (1857), who spoke of a
primary malady of the optic nerve under the title of “amaurosis with
eaccavation,” and they have excited a considerable amount of controversy
(Schnabel, 1892, 1908; Elschnig, 1895–1924; Pickard, 1921–31 ; Hofe,
1929; Reid, 1937; Nielsen, 1937; and many others). By some they are
considered to be cases of true glaucoma in which increased tension has
previously occurred and has subsided, or in which a rise of tension occurs at
intermittent intervals and has eluded observation, an assumption, of course,
which cannot be proved (glaucoma without hypertension). By others an
unusually poorly developed cribriform plate is considered to give way before
a normal intra-ocular pressure, so that at the nerve-head a state of relative
glaucoma exists (Fuchs, 1911–16). To others, again, the condition is one
of atrophy unassociated, or only associated casually, with glaucoma. It
was assumed by Schnabel (1892) that such cases were examples of primary
cavermous optic atrophy, an assumption which, however, still awaits general
pathological confirmation. Elschnig (1924) considered the excavation due
to the histiolytic action of the intra-ocular fluid as it passes through the optic
nerve, a phenomenon which he assumed to occur as a rule in glaucoma, but
sometimes in its absence, a view, however, which is by no means generally
recognized (Kapuscinski, 1930 ; and others)." Finally, it has been regarded
by Gradle (1917) as a sequel to an intra-ocular neuritis of toacic origin. In
this connection it will be remembered that in some cases of toxic amblyopia,
1 p. 3354.
T.O. —WOL. III. 3 R
3072 TEXT-BOOK OF OPHTHALMOLOGY
particularly in methyl alcohol poisoning, the optic atrophy is frequently
associated with a deep excavation so similar to that seen in glaucoma as to
have led to a decompression operation." -
However that may be, it seems clear that cases do exist in which optic
atrophy accompanied by deep cupping of the glaucomatous type is
unassociated with any demonstrable rise of tension : but although the
clinical appearance of the nerve-head is identical and the fields for white are
similar, according to Pickard (1925) they are distinguished from cases of
true glaucoma in that the colour-fields are of the atrophic type, being
disproportionately smaller than the field for white. In glaucoma, on
the other hand, it will be seen” that the defects in the fields for white and
colours are similar, since all conduction is equally destroyed by pressure.
Pickard (1938) also found in colour-perimetry that the photo-chromatic
interval for red (that is, the peripheral area in which a red object appears
colourless) was generally smaller than 5° in glaucoma and larger in atrophic
cases, as if the pressure had obliterated the liminal sub-chromatic sensation.
The prognosis is also different, for while in glaucoma the almost invariable
tendency is for the disease to progress until eventual blindness results, in
this type of case—in the absence of glaucoma—the tendency is for it to
progress so far and then to stabilize. It is true that cases do occur in which
all vision is eventually lost, but these are rare, and complete stabilization
has been observed over long periods of time (19 years, Pickard, 1931). The
name so frequently used to designate such cases—glaucoma without
hypertension—is a terminological contradiction ; cavernous optic atrophy
should not be employed, since it has a definite pathological connotation
which has not been shown to be applicable ; it would seem safest in the
meantime to employ the clinically useful term pseudo-glaucoma.
The cause of such a condition is disputable, but the aetiological factor
is almost certainly a local one operative in the optic nerve itself, a view
supported by the occasional occurrence of an atrophy of this type confined
to one quadrant of the disc (Stewart, 1936). Presumably the immediate
cause is optic atrophy associated with a recession of the lamina cribrosa by
cicatricial contraction from behind, with consequent traction on the
nerve-fibres and the development of nerve-bundle-defects in the field.
It is significant that the great majority of cases occur in the late adult
life of arteriosclerotics. It may be that in most cases the optic
atrophy is arteriosclerotic in origin, and that the recession of the lamina is
due to its weakening owing to malnutrition as a result of the sclerotic
process (Reid, 1937), thus producing an ischaemic degeneration : it will be
remembered that the vascular supply of this tissue is derived from the circle
of Zinn and that it does not share in the vascularization of the retina or the
nerve. It is also of great significance that in many of the cases there is
clinical or radiographical evidence of advanced sclerosis of the cerebral vessels
1 p. 3021. * p. 3380.
DISEASES OF THE OPTIC NERVE 3073
(Thiel, 1930; Knapp, 1932; Kurz, 1936; Ohm, 1936; Nielsen, 1937; and
others). It has been known since the observations of Türck (1852), Knapp
(1875), Otto (1893), and others, that arteriosclerosis of the cerebral vessels
may cause field defects of the nerve-bundle type either by pressure on the
chiasma, the tracts, or the nerve, or by causing trophic changes from
nutritional disturbances, and it is probable that such an occurrence is much
more frequent than is generally recognized. Presumably such atheromatous
changes are accompanied by advanced arteriosclerotic degeneration in the
vessels supplying the nerve itself (Knapp, 1940). These questions, however,
will be dealt with more fully in the chapter on glaucoma.
Axenfeld. B. O. G. Heidel.., xxxii, 303, 1905. Neilsen. Acta O., xv., 151, 1937.
Elschnig. B. O. G. Heidel.., xxiv, 149, 1895. Ogawa. A. f. Aug., lxxii, 10, 1912.
A. f. Aug., xxxiii, Erg., 187, 1896. Ohm. A. f. O., czzxv, 537, 1936.
K. M. Aug., xl (2), 81, 1902. Otto. Untersuch. ii. Sehnervenkr. be? Arterio-
A. f. O., lxviii, 126, 1908. Sklerose, Berlin, 132, 1893.
Z. f. Aug., lii, 287, 1924. Pickard. P. R. S. Med., xiv, 31, 1921;
Fuchs. Z. f. Aug., xxv, 108, 1911. xxiii, 57, 1930.
A. f. O., xci, 435, 1916. Brit. J. O., vii, 81, 1923; ix, 385, 1925;
Gradle. A. of O., xlvi, 117, 1917. xv, 323, 1931 ; xxii, 391, 1938.
v. Graefe. A. f. O., iii (2), 456, 1857. Reid. Brit. J. O., xxi, 361, 1937.
Hofe. A. f. Aug., c-ci, 414, 1929. Schnabel. A. f. Aug., xxiv, 273, 1892.
Kapuscinski. A. d’O., xlvii, 779, 1930. Z. f. Aug., xiv., 1, 1905; xix, 558, 1908.
Knapp, A. A. of O., viii, 637, 1932. Spielmeyer. K. M. Aug., xliv, 97, 1906.
Knapp, H. A. f. Aug. iv, 209, 1875. Thiel. B. O. G. Heidel.., xlviii, 355, 1930.
Koyanagi. A. f. O., cxxiii, 537, 1930. Türck. Sitz. d. k. Akad. d. Wiss. Math-mat
Kurz. A. f. Aug., ciz, 108, 1936. Cl., Wien, ix, 299, 1852.
W. TUMIOURS OF THE OPTIC NERVE AND ITS SHEATHS
Clinical Symptoms and Course
From the clinical point of view tumours of the optic nerve may be
divided into three classes—those situated intra-ocularly on the papilla,
which are very rare ; those of the orbital portion of the nerve, which,
although more common, are also rare ; and tumours of the intra-cranial
nerve and chiasma.
(a) Intra-ocular Tumours
Tumours of the papilla are intra-ocular growths and to a large extent
share the symptomatology of these neoplasms. In their early stages they
give rise to no subjective symptoms and may be found accidentally on
routine examination ; as they grow larger they cause a diminution of vision
either by obstructing the macula or by causing a disturbance or detachment
of the retina : at this stage the vision may fail with dramatic rapidity
(Schieck, 1912). Eventually, if allowed to progress, the natural termination
is the development of glaucoma (Sidler-Huguenin, 1920; Oguchi, 1933;
Halbertsma, 1934). Such tumours are, of course, readily visible ophthalmo-
3 R 2
3074 TEXT-BOOK OF OPHTHALMOLOGY
scopically. The primary tumours occurring at this site may be classified
into three types:—
1. Pigmented tumours, which may be simple or malignant
melanomata."
2. Tumours belonging to the group of phakomatoses—angiomatosis of
Lindau, tuberous sclerosis of Bourneville, and fibromatosis of v.
Recklinghausen : these have already been discussed in association with
tumours of the retina.
3. Nerve Tumours. These have been described as endotheliomata
(Sidler-Huguenin, 1920), peritheliomata (Schieck, 1912), psammomata
(Argand and Couadau, 1936), gliomata (Gibson, 1921) or neurocytomata
(Halbertsma, 1934) : probably most of them were gliomatous in nature.
Secondary tumours spreading from adjacent tissue are more common,
especially malignant melanomata from the choroid, neuroblastomata from the
retina, and, much more rarely, neoplasms extending forwards from the optic
nerve behind the lamina and its sheaths—gliomata and endotheliomata.
Metastatic tumours are exceptional.”
The differential diagnosis lies between hyaline bodies (drusen),” hyaloid
cysts associated with hyaloid remnants,” and inflammatory masses such
as those due to syphilis or tuberculosis. If such tumours are progressive
the only treatment is enucleation of the globe together with as much of the
nerve as is practicable.
(b) Intra-orbital Tumours
Tumours affecting the optic nerve or its sheaths in its orbital course
were first described by Wishart (1833) and Middlemore (1838); they were
clearly differentiated from tumours of the orbital tissues by Goldzieher
(1873), and their clinical characteristics were fully established by v. Graefe
(1864). They are not very common, occurring in the Moorfields’ records in
2 in 388,000 cases (Collins and Marshall, 1900): thus Byers (1901) collected
102 cases from the literature of primary intra-dural tumours, and Parsons
(1903) 18 cases of the extra-dural type, a total which Lawford (1908) brought
up to 21. Hudson (1912) discussed 182 cases, and in the succeeding litera-
ture Mathewson (1930) found an additional 61 cases. About 80% of these
neoplasms are gliomata forming tumours of the nerve itself; the remaining
20% are associated with the sheaths, the majority (some 17%) being
endotheliomata while the residual 3% are fibromata.
Such tumours have the symptomatology of orbital tumours; v. Graefe
(1864) originally standardized their three classical symptoms—proptosis, a
disturbance of the motility of the globe, and visual deterioration.
Proptosis is usually an early symptom, and, in contradistinction to
orbital tumours, the displacement of the globe is usually directly forwards
1 p. 2474. * pp. 3100–1.
8 p. 3062. 4 p. 1378.
DISEASES OF THE OPTIC NERVE 3075
or approximately so; quite frequently, however, especially in the later
stages, there is some deviation which is usually downwards and outwards
(Figs. 2368–40). The displacement almost invariably progresses slowly, but
eventually reaches such dimensions as to produce chemosis, keratitis,
necrosis of the cornea, perforation of the globe and panophthalmitis. The
motility of the globe is less impaired than might be expected from the degree
of proptosis, a circumstance which applies particularly to tumours of the
nerve itself with which relatively good movement may persist until the growth
has practically filled the orbit by its own mass and made mobility mechanic-
ally impossible; in the case of tumours of the sheaths, however, when the
FIG. 2638. TUMoUR of THE OPTIC NERVE.
18 mm. of proptosis with upward displacement; glioma of optic nerve (Neame).
FIGs. 2539 and 2640.-TUMoUR of THE OPTIC NERVE.
Proptosis with downward displacement; glioma of optic nerve (Westkamp).
orbital muscles and nerves become infiltrated, ocular movements may
become considerably restricted.
The visual acuity, as would be expected, usually deteriorates early and
rapidly, especially in the case of nerve tumours, a fact which frequently
prevents the occurrence of diplopia as a result of the displacement of the
globe, a symptom so common in the case of orbital tumours: occasionally,
however, the vision is impaired to a much less extent than would be con-
sidered possible, and has remained normal or practically so even when the
tumour is far advanced (Axenfeld and Busch, 1899).
As a rule the neoplasm grows laterally into the orbit, and it may even-
tually be palpated if the finger is pushed deeply between the globe and the


3076 TEXT-BOOK OF OPHTHALMOLOGY
orbital margin. The usual tendency is for it to spread backwards towards
the brain rather than towards the globe; it may, however, cause a hyper-
metropic refraction by pressure upon the posterior pole of the eye (Collins
and Marshall, 1900; 11:0 D. Braunschweig, 1893). More commonly the
effects of obstruction of the circulation are seen-papilloedema and eventually
a secondary atrophy are relatively common, while retinal haemorrhages,
venous thrombosis, a star-figure at the macula, or white exudative flecks
may appear. On the other hand, a simple atrophy due to pressure on the
nerve-stem may occur, but is rare, and in some cases, particularly in those
wherein the nerve immediately behind the globe is unaffected, the fundus
may be ophthalmoscopically normal. In most cases, indeed, the degree of
Figs. 2641–42–GLIoMA or The Optic NERVE.
Radiograms to show the enlarged optic foramen on the affected side (A) in
comparison with the normal side (B) (Rand, Irvine and Reeves).
visual defect is out of all proportion to the ophthalmoscopic signs. The
spread of the neoplasm centrally may cause an erosion or an enlargement of
the optic foramen, an appearance which can be recorded radiographically
(v. d. Hoeve, 1925; Knapp, 1926) (Figs. 2641–42); such an examination is of
the greatest prognostic importance since it indicates intracranial extension.
The evolution of these neoplasms is, however, usually slow, in most cases the
proptosis having been noted for one or several years before steps were con-
sidered necessary to deal with it. This period is usually passed without
pain, although a trigeminal neuralgia occasionally occurs; but eventually
acute trouble arises from keratitise lagophthalmo, or symptoms of cerebral
involvement initiate the terminal phase. Lymphatic or other metastases are
rare. Almost universal characteristics of all types are slow growth, local
malignancy, and absence of metastases.

DISEASES OF THE OPTIC NERVE 3.077
The differential diagnosis from orbital tumours will be more fully deve-
loped in the section dealing with the latter ; the main points, however,
which suggest a neoplasm of the nerve itself are the proptosis, axial, non-
pulsatile, irreducible, and slowly progressive, the small limitation of move-
ment, the early visual failure, and the frequent presence of papilloedema or
atrophy ; if erosion of the optic canal is radiographically evident the
diagnosis is beyond doubt. The characteristic symptoms and prognosis of
the various types of tumour will be noted when these are discussed in detail.
(c) Intra-cranial Tumours
The symptomatology and clinical course of tumours of the intra-cranial
portion of the optic nerve, the chiasma, and the tracts, belong essentially
to the surgery of the central nervous system. Such tumours are gliomatous
in nature, but are rare ; among 826 histologically examined intra-cranial
tumours, Martin and Cushing (1923) found 345 gliomata of which only 7 arose
from the chiasma. The most frequent findings in such cases are atrophy
of the optic nerve and slowly progressive loss of vision in one eye if the pre-
chiasmal part of the nerve is affected ; if the chiasma is involved these
signs may be bilateral and the visual fields show bitemporal or homony-
mous hemianopia, or total blindness in One eye and hemianopia in the other.
Papilloedema is a rare phenomenon and only occurs exceptionally unless the
tumour has reached a size such as will cause a rise in intra-cranial pressure
(Dandy, 1922; Aitchison, 1936). X-rays can frequently be of considerable
diagnostic help, since they may show deformity of the sella turcica, erosion
of the anterior and posterior clinoid processes, erosion of the sphenoid ridge,
and, most significant, enlargement of the optic foramen.
A backward extension is the more usual direction of spread, and as the
base of the brain is involved, vegetative symptoms result from implication
of the diencephalic centres, and pressure symptoms from obstruction of the
narrow third ventricle. The former include disturbances in fluid or fat
metabolism or in the heat regulation ; the pressure symptoms are much
more severe, producing, if intermittent, attacks of stupor and decerebrate
posture, and if continuous, a progressive and ultimately fatal hydrocephalus.
A forward extension is rarer, but it is interesting that a tumour of intra-
cranial origin may first become evident as an orbital tumour, the true origin
being determined only after operation or pathological examination (Seefelder,
1931).
The diagnosis of the intra-cranial gliomata of the optic nerve and
chiasma is extremely difficult ; as a rule, indeed, a clinical diagnosis is
impossible. Martin and Cushing (1923), for example, were able to suspect
the condition with any degree of certainty in only one out of their 7 cases.
If ocular symptoms alone are present and cerebral evidences of infiltration
or pressure are absent, the greatest diagnostic difficulty arises with a retro-
bulbar neuritis (Lundberg, 1935) or a basal (chiasmal) meningitis. If radio-
3.078 TEXT-BOOK OF OPHTHALMOLOGY
graphic evidences of infiltration are present or cerebral symptoms exist, a
surgical exploration is usually justified, for although the chiasmal gliomata
are not amenable to surgical treatment, the usual lesions which give rise to
diagnostic difficulties can frequently be operatively removed : pituitary
adenomata, meningiomata and craniopharyngiomata. A most significant
feature is enlargement of the optic canal, but since a centripetal spread is the
commoner, the absence of this sign is of little value.
Aitchison. T. O. S., lvi, 292, 1936.
Argand and Couadau. A. d’O., liii, 869, 1936.
Hudson. R. L. O. H. Rep., xviii, 317, 1912.
Knapp. Contrib. to Ophthalmic Science,
Axenfeld and Busch. A. f. Aug., xxxix., 1,
1899.
Braunschweig. A. f. O., xxxix (4), 1, 1893.
Byers. Studies from the R. Victoria Hosp.,
Montreal, i, 1, 1901.
Collins and Marshall. T. O. S., xx, 156, 1900.
Dandy. Am. J. O., v, 169, 1922.
Gibson. Brit. J. O., v, 67, 1921.
Ginsberg. K. M. Aug., lxxx, 357, 1928.
Goldzieher. A. f. O., xix (3), 119, 1873.
v. Graefe. A. f. O., x (1), 193, 1864; xii (2),
100, 1866.
Halbertsma. Ned. Tij. v. Gen., ii, 3742, 1932.
v. d. Hoeve. A. f. O., czv, 355, 1925.
Am. J. O., viii, 101, 1925.
Wiscon., 1926.
Lawford. T. O. S., xxviii, 42, 1908.
Lundberg. Ueber d. primären Tumoren d.
Sehnerven u. d. Sehnervenkrenzung,
Stockholm, 1935.
Martin and Cushing. A. of O., lii, 209, 1923.
Mathewson. Am. J. O., xiii, 881, 1930.
Middlemore. London Med. Gaz., xxii, 897,
1838.
Oguchi. A. f. O., czzx, 427, 1933.
Parsons. T. O. S., xxiii, 116, 1903.
Schieck. A. f. O., lxxxi, 328, 1912.
Seefelder. Wien. kl. W., xliv, 838, 1931.
Sidler-Huguenin. A. f. O., ci, 113, 1920.
Wishart. Edin. Med. Surg. Jil., x1, 274, 1833.
A. Primary Tumours
Since the first full pathological examination of a tumour of the optic
nerve was published by v. Graefe (1866), much discussion has arisen over
their nature ; they were divided anatomically by Leber (1877) into two
classes, intra-dural, or essential tumours of the nerve itself, originating from
the nerve elements or from the connective tissue of its septa or the pia-
arachnoid, and eactra-dural tumours springing from the dural sheath and
attacking the nerve secondarily. The nature of the neoplasms, however,
remained controversial and in the earlier literature an amazing confusion of
terminology is encountered : glioma, gliosarcoma, fibroma, fibrosarcoma,
myxoma, myxosarcoma, Sarcoma, neuroma, endothelioma, fibromatosis,
and so on. They were variously considered mesoblastic or epiblastic, benign
or malignant, a hypertrophy or a neoplasm, the pathological diagnosis being
continually changed from time to time, and different views being expressed
as to their significance and prognosis. This chaotic state of opinion was
largely due to the great diversity of the microscopical appearances with the
ordinary type of staining, but at the end of the last century and the beginning
of the present one, the weight of pathological opinion inclined to the view
that they were mesodermal in origin (Sattler, 1892; Emanuel, 1902; Byers,
1901; Parsons, 1905; and others). In an exhaustive and classical paper,
however, in which he reviewed the whole literature of 182 cases (neglecting
DISEASES OF THE OPTIC NERVE 3079
33 which could not be classified for lack of data), Hudson (1912) divided
them into three main classes : gliomatous, endotheliomatous and fibro-
matous, the first associated with the nerve and the latter two with its
sheaths ; subsequent work with specific neuroglial stains has fully confirmed
this broad thesis. Hudson held that all the tumours developing in the nerve-
stem were essentially of the same nature, differing only by showing varying
degrees of development and a varying preponderance of different tissues—in
Some a majority of more embryonic cells suggested a gliomatous nature,
while in others more fully developed cells or much fibrous tissue-reaction
suggested a diagnosis of fibroma. The position was finally clarified in a
classical paper by Verhoeff (1922), who, using modern neuroglial staining,
showed that all such tumours were of the same gliomatous type, their
differences being quantitative only, depending on the presence of various
glial elements in different proportions and arrangements. It is to be noted
that they are true gliomas, related to those occurring in the brain and with
no relation to common malignant tumours of the retina popularly called a
“glioma.”
We therefore classify primary tumours of the optic nerve and its sheaths
into three groups —
1. Essential (ectodermal) tumours of the nerve : GLIOMATA.
2. MENINGIOMATA : mesodermal tumours of the sheaths —
(a) Endotheliomata.
(b) Fibromata.
3. NEURO-ECTODERMAL TUMoURs.
(a) Neurinomata.
(b) Malignant melanomata.
Incidence. As we have seen, these tumours are rare ; in all medical
literature less than 350 cases have been reported. Of these the gliomata
have been much the most common. Of the cases reported on pathologically,
Hudson (1912) found 118 gliomata, 24 endotheliomata and 7 fibromata, and
Mathewson (1930), reviewing 61 cases in the intervening literature, found
52 gliomata, 9 endotheliomata and I fibroma. The proportion is therefore
roughly 20 : 4: 1.
With regard to age, most occur preferentially in the early years of life.
In gliomata Hudson (1912) found 75% in the first decade among which
many occurred before the age of 5, and 88% before the age of 20 ; Mayou
(1905) reported a case at 17 months and Brailey (1876) at 62 years. Endo-
theliomata, on the other hand, occur later, more than half of the cases being
Over the age of 30, the average in Hudson's series being 35 years ; they may,
however, occur in advanced age (79 years, Neame, 1923). Fibromata also
occur in the young, some 75% appearing in the first decade. The female Sea is
the more prone, Hudson's series showing a proportion of females to males of
70 to 43 in gliomata, 20 to 7 in endotheliomata, and 5 to 1 in fibromata.
3080 TEXT-BOOK OF OPHTHALMOLOGY
ESSENTIAL TU MOUR'S OF THE NERVE
GLIoMA (spongioblastomA)
The vast majority of gliomata arise from the intra-orbital portion of the
optic nerve; records of an origin from the disc are few (Gibson, 1921), and
chiasma (Martin and Cushing, 1923) or purely intra-cranial neural tumours
(Seefelder, 1931 : Aitchison, 1936) are rare. In a very large number of
cases, however, in which the clinical symptoms pointed to an intra-orbital
tumour only, intra-cranial extension has been found, and it is difficult to say
whether the origin was in the cranial cavity or the orbit: probably multiple
foci of origin do not occur, and the most common nidus is in the region of the
Fig. 2643–GLIoMA or Optic NERVE (Griffith, To. S.).
optic foramen from which extension occurs in both directions, a circum-
stance which makes an enlargement of the optic foramen an important
diagnostic point. It is possible also that a simultaneous origin throughout
the course of the nerve may occur.
The growth and development of a glioma is fairly characteristic. It is
almost invariably slow, and although temporary periods of rapid growth
have been reported, the usual history is of steady and gradual progression
over a period measured in years. It advances by direct extension along the
intra-neural portion of the nerve and does not penetrate the sheaths so that
as a rule a diffuse and uniform hypertrophic swelling is produced (Fig. 2643).
A solitary spindle-shaped or spherical enlargement may occur, or several
nodules may be separated by thick tubular tissue. The extension is usually
mainly in a centripetal direction, but occasionally the disc and from it the
retina have been invaded, giving the ophthalmoscopic appearance of a solid

DISEASES OF THE OPTIC NERVE 3081
swelling or a cyst-like formation (Fig. 2644). More usually extension is
towards the brain, a fact which constitutes the serious danger of the disease.
Eleven cases of invasion of the optic disc by gliomata of the nerve have been
reported : v. Graefe (1864), Barraquer (1902), Pereyra (1914), Verhoeff (1921), Sattler
FIG. 2644.-GLIoMA of OPTIC NERVE.
Glial proliferation in nerve-head with marked hyperplasia of mesothelial cells of
arachnoid at anterior end of optic nerve canal (F. A. Davis).
(1925), Weigelin (1931), Foerster and Gagel (1931), Wilson and Farmer (1940), and
Davis (1940). Martin and Cushing (1923) noted a swelling on the disc resembling
papilloedema in the case of a chiasmal tumour which was due to a gliomatous
infiltration.
Pathology
As we have seen, owing to inadequate glial staining, the early pathological studies
were largely without value. A large number of later papers, however, are of great
importance–Fischer (1908), Löhlein (1910), Hudson (1912), Fleischer and Scheerer
(1920), Verhoeff (1922–32), Neame (1923), Kiel (1923), Hidano (1925), Musial (1930),
Grinker (1930), Foerster and Gagel (1931), Gluck (1932), de Long (1934), Mehney
(1936), Kiehle (1936), Rand, Irvine and Reeves (1939), Wilson and Farmer (1940),
Davis (1940), and others.
Macroscopically the tumour is a smooth or nodular elastic mass enclosed
within the dura, which, although it may be thinned out and contain neo-
plastic material on its inner surface, is not penetrated (Figs. 2645–50). When
cut the mass is recognizable as a diffusely thickened optic nerve, or the nerve is

3082 TEXT-BOOK OF OPHTHALMOLOGY
seen compressed peripherally. Cysts filled with a mucinous substance are
almost constantly found, and may indeed be so conspicuous as to constitute
Fig. 2645.-GLIonia or OPTIC NERVE.
Early stage of sheath invasion. The sheath tumour is almost entirely glial there
being only a thin layer of arachnoid cells beneath the dura (F. A. Davis).
Fig. 2646.-GLIoMA or Optic NERVE.
Late stage of sheath invasion. The sheath tumour is made up entirely of glia
(F. A. Davis).
practically the entire tumour (Adamiick, 1894). This mucinous substance
gave rise to the old term of myxoma so frequently applied to such tumours,
but their contents do not show the staining reactions for mucin (Fleischer


DISEASES OF THE OPTIC NERVE 3083
Fig. 2647. GLIoMA of OPTIC NERVE.
The growth invading sheath; same section as Fig. 2645 (x 80). The fibrous
septa of nerve almost entirely destroyed. The sheath tumour is glial in nature
(F. A. Davis).
Fig. 2648.-GLIoMA of OPTIC NERVE.
Transverse section showing bands of connective tissue representing the normal
fascicular structure of the nerve, the intervals between being occupied by typical
gliomatous tumour (Rand, Irvine and Reeves).


3084 TEXT-BOOK OF OPHTHALMOLOGY
and Scheerer, 1920; Sattler, 1925); Löhlein (1910) considered that the
cavities resulted from areas of degeneration secondary to hyaline changes in
Fig. 2649–GLuoma or Optic Nºrvº.
Early stage of glioma of nerve. Marked increase of glial cells (chiefly astrocytes
and oligodendrocytes) with condensation of glial fibres adjacent to fibrous septa
(H. and E. stain, x 200) (F. A. Davis).
Fig. 2650–GLIoMA or Optic Nºve.
Late stage of glioma of nerve from the same specimen as Fig. 2649 removed from
the stump 4 years after the first specimen. Note marked increase of progress of
glioma (H. and E. stain, x 200) (F. A. Davis).
the vessels, and Verhoeff (1932) looked upon them as a fusion of reticular
vacuoles filled with serous fluid. As a rule the tumour is not particularly
vascular, although the septa may contain many dilated thin-walled vessels


DISEASES OF THE OPTIC NERVE 3085
showing hyaline degeneration and secondary hamorrhages, and occasionally
these may be so numerous as to suggest as angiomatous growth.
The infiltration of the neoplastic tissue varies considerably. Essentially it
permeates the inter-septal spaces, and occasionally the septa are thinned and
stretched round the broadbands of tumour cells which eventually invade them.
More usually, however, the septa become enormously thickened, showing an
exuberant growth sometimes larger than and extending far beyond the
tumour itself and compressing the non-tumourous portion of the nerve ; it
is made up of collagenous fibres arranged in irregular masses into which glial
fibres grow in all directions. The significance of this hyperplasia is not
clear, but it is probably reactive in nature and secondary to the gliomatous
---
Fig. 2651.-GLIoMA of OPTIC NERVE.
The nerve-fibres have completely disappeared. Note holes where nerve-fibres
have atrophied and the dense glial felt-like masses bordering the fibrous septa (H. and
E. stain, x 550) (F. A. Davis).
process in the nerve; it is particularly obvious when a glioma occurs in
v. Recklinghausen's disease." Verhoeff (1932) concluded that the growth does
not advance so much by invading the adjacent structure as by stimulating
the pre-existing neuroglia in the neighbourhood to proliferation ; it is
possible that there is a similar stimulus given to the tissues of the sheaths.
Meantime the nerve-fibres are spread apart, stretched and compressed, so
that as a rule they become demyelinized at an early stage, but occasionally
bundles of axis cylinders may remain intact allowing a persistence of vision.
All authorities now agree that the essential neoplastic cell is glial in
nature, and is a uni- or bi-polar spongioblast in type. The cells are long
and spindle-shaped, with oval nuclei, and processes, sometimes straight,
1 p. 3087.

3086 TEXT-BOOK OF OPHTHALMOLOGY
sometimes corkscrew-like, arising from each end. Verhoeff (1922) described
three main types all of which may undergo transition the one to the other—
a finely reticulated type similar to the normal neuroglia of the nerve, an
Fig. 2652.-GLIoMA or Optic NERVE.
The same specimen as Fig. 2650. Differential silver staining to show arrangement
of glial fibres (processes of neoplastic astrocytes) (Mallory's phospho-tungstic
acid, x 200) (F. A. Davis).
Fig. 2653-Advanced Stage of Glioma or Optic NERVE.
The same specimen as Fig. 2654 with differential silver staining. A giant
neoplastic astrocyte with a less number of oligodendrocytes (F. A. Davis).
exaggerated coarsely reticulated type, and a sickle-shaped cell made up of
many coarse neuroglial fibres. Sometimes mitotic figures are seen (Grinker,
1930), and at other times these are absent (Verhoeff, 1932), and degenerative
changes, such as vacuolation of the cytoplasm, are common (Figs. 2651–53).


DISEASES OF THE OPTIC NERVE 3087
Occasionally rounded or elongated hyaline masses, sometimes continuous
with the process of a neuroglial cell have been described ; Verhoeff (1922), who
called them cytoid bodies, considered them atypical, giant neuroglial fibres, and Martin
and Cushing (1923) thought that they represented masses of coalesced neuroglial
fibres. They appear to have no particular significance.
Favaloro (1928) found multi-nucleated cells without processes which he
considered to be oligodendroglia, and he and Gluck (1932) found astrocytes, while
Goldstein and Wexler (1932) described cells resembling ganglion cells of a neuroblastic
type in a case associated with v. Recklinghausen's disease; but, with these exceptions,
the fundamental cell comprising the mass of the tumour has so far always been found
to be a primitive spongioblast showing no differentiation into the more mature glial
- - -
*** º
- - --
º º º -
-
-
- - - - ºs- - º:
º - --- * -- ~~~~ - º -
--- * ------- - *-
Fig. 2654.—AdvancED STAGE of GLIoMA of Optic NERVE.
The glial cells (astrocytes and oligodendrocytes) resemble sarcoma cells
(longitudinal section). (See Fig. 2653.) (F. A. Davis.)
cells, and identical with the characteristic cells of this type of tumour when found in
the brain. Since the optic nerve is really a tract of the central nervous system and
contains all adult forms of neuroglia, it is theoretically possible to expect the great
variety of gliomata which are found in the brain with their different characteristics
and different degrees of malignancy. It may be that other types will eventually
be found, but it is to be remembered that the cerebral gliomata have a definite
topographical relationship ; thus, in addition to its predilection for the optic
nerve and chiasma, the polar spongioblastoma is found particularly in the region
of the third ventricle, the medulloblastoma predominates in the cerebellar region,
while the glioblastoma multiforme, pre-eminently the glioma of adult life, is
characteristic of the cerebral hemispheres.
Glioma and Neuro-fibromatosis. The association of a glioma of the optic nerve
with the diffuse neuro-fibromatosis of v. Recklinghausen was first noted by v. Michel
(1873), since which time several reports have come to hand (Emanuel, 1902; Martin
and Cushing, 1923; Grinker, 1930; Goldstein and Wexler, 1932; Fleischer, 1934).
T.O.-WOL. III. 3 S

3088 TEXT-BOOK OF OPHTHALMOLOGY
Emanuel (1902) considered the enormous hyperplasia of the connective tissue
of the nerve-sheath so frequently found with gliomata as a neuro-fibroma analogous to
the peripheral neuro-fibromata characteristic of this disease (elephantiasis neuro-
matodes). Such a relationship is not unexpected in a disease wherein systemic mani-
festations are frequently found, a most instructive example being the case reported by
Shapland and Greenfield (1935) which showed meningeal psammomata, a similar
tumour on the optic nerve, neuro-fibromata associated with the cranial and spinal
nerves, gliomata in the cord and frontal lobe, and a gliosis, perhaps of a reactive
nature, involving one disc and the surrounding retina. Davis (1939) also reported a
case of plexiform neuro-fibromatosis of the orbit and globe, wherein the nerves of the
orbit, sclera, choroid, ciliary body and iris were involved, associated with a glioma of
the optic nerve in the orbit and gliomatosis
of the intra-cranial portions of both nerves,
the chiasma, thalamus, medulla and pons,
and the temporal lobe of the brain (Fig.
2655).
The clinical symptoms of gliomata
of the optic nerve have already been
fully described both in their intra-
orbital 1 and their intra-cranial *
occurrence. They differ from menin-
giomata in their usual onset at an
earlier age, in the fact that visual
disturbances usually precede the prop-
tosis sometimes by many years, and
that limitation of movement is less
marked and later in onset.
The prognosis depends largely on
the site and the stage at which re-
Fia. 2655. Neuro resowatosis or moval is undertaken. Although these
Optic NERVE AND Ciliary GANGLIow. tumours are of slow growth–some-
In X. Recklinghausen's disease (F. A. times so extremely so as to suggest a
Davis). -
benign nature—and although they do
not give rise to metastases, they eventually prove fatal from cerebral extension
if left. If, however, the tumour can be completely removed the prognosis
is good. If partial removal only is attained, and in a purely orbital operation
this frequently results, local recurrences in the orbit do not occur, but after
a period of time which is frequently measured in years, fatal intra-cranial
complications set in. Even in the presence of intra-cranial recurrences, a
patient may live for 5 or more years (Werner, 1903), and death from
recurrences have been reported as long as 9 (Barraquer, 1902), 10 (Byers,
1901) or 26 years (Pagenstecher, 1902) after an original operation. In
chiasmal tumours the prognosis is always bad and any attempt at their
removal proves fatal.
1 p. 3074. * p. 3077.

DISEASES OF THE OPTIC NERVE
3089
The treatment of gliomata will be considered with that of optic nerve
tumours in general.
Adamūck. A. f. Aug., xxviii, 129, 1894.
Aitchison. T. O. S., lvi, 292, 1936.
Barraquer. A. de Oft. H.-A., ii, 132, 1902.
Brailey. R. L. O. H. Rep., ix, 231, 1876.
Byers. Studies from R. Victoria Hosp.,
Montreal, i, 1, 1901.
Davis. A. of O., xxii, 761, 1939.
A. of O., xxiii, 735, 957, 1940.
Emanuel. A. f. O., liii (1), 129, 1902.
Favaloro. An... di Ott., lvi, 619, 1928.
Fischer. A. f. Aug., lix, 181, 1908.
Fleischer. B. O. G. Heidel., l, 185, 1934.
IFleischer and Scheerer. A. f. O., ciii, 46, 1920.
Foerster and Gagel. Z. ges. Newrol. Psy.,
cxxxvi, 335, 1935.
Gibson. Brit. J. O., v, 67, 1921.
Gluck. Brit. J. O., xvi, 406, 1932.
Goldstein and Wexler. A. of O., vii, 259,
Martin and Cushing. A. of O., lii, 209, 1923.
Mathewson. Am. J. O., xiii, 880, 1930.
Mayou. R. L. O. H. Rep., xvi, 155, 1905.
Mehney. A. of O., xvi, 95, 1936.
v. Michel. A. f. O., xix (3), 145, 1873.
Musial. Z. f. Aug., lxxii, 189, 1930.
Neame. Brit. J. O., vii, 209, 1923.
Pagenstecher. A. f. O., liv, 300, 1902.
Parsons. Path. of the Eye, ii, 693, 1905.
Pereyra, Am. di Ott., xliii, 402, 1914.
Rand, Irvine and Reeves. A. of O., xxi, 799,
1939.
Sattler. Beit. z. Chir., Festschr. Th. Billroth,
Stuttgart, 314, 1892.
Die bosártigen Geschwilsted. Auges, Leipzig,
1925.
Seefelder. Wien. kl. W., xliv, 838, 1931.
Shapland and Greenfield. T. O. S., lv, 257,
1932. 1935.
v. Graefe. A. f. O., x (1), 193, 1864; xii (2), Verhoeff. Am. Med. As., T. Sect. O., 146,
100, 1866. 1921.
Grinker. A. of O., iv, 497, 1930. A. of O., li, 120, 129, 1922.
Hidano. Z. f. Aug., lvii, 31, 1925. Penfield's Cytology and Cellular Path. of the
Hudson. R. L. O. H. Rep., xviii, 317, 1912. C.N.S., N.Y., iii (21), 1029, 1932.
Kiehle. A. of O., xv, 686, 1936. Weigelin. K. M. Aug., lxxxvii, 527, 1931.
Riel. A. f. O., czii, 64, 1923. Werner. T. O. S., xxiii, 82, 1903.
Leber. G.-S. Hb., I, v, 910, 1877. Wilson and Farmer. A. of O., xxiii, 605,
Löhlein. A. f. O., lxxiii (2), 335, 1910. 1940.
de Long. Am. J. O., xvii, 797, 1934.
MENING IOMATA
1. ENDOTHELIOMATA
As we have seen, endotheliomata of the optic nerve-sheaths are rare :
up to 1930 only 33 cases had been reported (Mathewson, 1930) and since
then the cases in the literature have been few. They become apparent
clinically as orbital tumours, but since at the time of removal intra-cranial
extension had frequently taken place, the precise point of origin has usually
remained obscure. Probably the usual origin is in the orbit; they may
arise in the optic canal and extend forwards into the orbit and backwards
into the middle cranial fossa (Cohen and MacNeal, 1921); while anterior
basal meningiomata of an endotheliomatous nature are not very uncommon
(Cushing, 1922; Henderson, 1938; and others). The tumour arises either
from the endothelial cells lining the sub-dural space or from the cells covering
the arachnoid, and while in many cases it does not extend beyond the dura,
it may eventually do so and practically fill the entire orbit; occasionally
the growth is almost completely extra-dural. Invasion of the neighbouring
structures, indeed, is of fairly frequent occurrence, and extension may
occur not only to the orbital tissues but into the nasal fossae (Hudson, 1912).
3 S 2
3090 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2656.-ENDoth ELIoMA or Optic NERVE SHEATHs.
a. Endothelioma in arachnoid sheath, b. Compressed optic nerve. c. Sub-
retinal fluid beneath detached retina (Stallard, Brit. J. O.).
Fig. 2557-Exuoruelioma or Optic Nºve.
Same case as Fig. 2658 (Coston, A. of 0.).


DISEASES OF THE OPTIC NERVE 3091
Extension also occurs along the nerve in both directions; in contra-
distinction to glioma, infiltration of the sclera in the vicinity of the disc
and the posterior pole of the eye is relatively common although involvement
º
|- * -
--
Fig. 26.58.-ENDoTHELIoMA of OPTIC NERVE.
The tumour cells at the edge of the disc. From the case of Fig. 2657 (Coston,
A. of O.),
of the choroid is exceptional (Cushing and Eisenhardt, 1938) and invasion
of the disc rare (Jacobson, 1864; Goldzieher, 1873; Baeumler, 1886;
Fig. 2659.-ENDoTHELIoMA of OPTIC NERVE SHEATH.
Showing growth from dural sheath with which the (pale) septa are continuous
(Brailey, T. O. S.).
Neame, 1923; Schwarz, 1936; and Coston, 1936) (Figs. 2657–58); intra-
cranial extension is a terminal event. On the other hand, infiltration does
not occur through the pia which remains intact, so that the nerve itself is


3092 TEXT-BOOK OF OPHTHALMOLOGY
only damaged by compression, the axis cylinders retaining their function
for a long time (Fig. 2659).
Pathological examinations yield a fairly uniform picture (Griffith,
1911; Hudson, 1912; Cohen and MacNeal, 1921; Neame, 1923; Twelmeyer
1923; Gilchrist, 1924; Neame and Wolff, 1925; Mathewson, 1930;
Verhoeff, 1932; Stallard, 1935; Coston, 1936; Schwarz, 1936; and
others). The cells are typically endothelial, but since these are merely
differentiated connective tissue cells the term meningeal fibroblastoma is
preferred by some writers. They are large with vesicular nuclei and pale
staining protoplasm, arranged as syncytial masses in irregular lobules or
Fig. 26.60–ENDoth Eltonia or Optic Nºrvº. Sheaths.
Showing psammoma bodies, clefts, collagen fibrils and connective tissue trabeculae
(x 275) (Stallard, Brit. J. O.).
ill-defined columns, with a tendency to an arrangement in whorls (Fig. 2660).
Between these masses are fibrils of collagenous material, sometimes
forming dense fibrous trabeculae, while psammoma bodies with concentric
lamination may be seen.
The symptoms of an endothelioma of the optic nerve have already been
discussed : it is differentiated from a glioma by its occurrence at a more
advanced age, by the fact that the exophthalmos usually precedes the
visual failure, by the earlier and more apparent limitation of movement, by
the evidence of circulatory disturbances in the nerve-sheath (oedema of
the lids and conjunctiva, venous stasis and haemorrhages in the retina),
and by the occasional presence of pain owing to intra-orbital infiltration.
- p. 3073.

DISEASES OF THE OPTIC NERVE 3093
The prognosis is parallel with that of optic gliomata. The progress of
the growth is usually exceedingly slow, being measured in years, and in
spite of the infiltrative tendency, local recurrences are rare even in the
event of incomplete removal. Recurrences, however, can appear and a
fatal termination ensue even despite an exenteration of the orbit (Stevenson,
1927). On the other hand, even after a partial orbital excision, the patient
may remain well for a long period (5 years, Schwarz, 1936). The treatment
will be dealt with subsequently."
Baeumler. K. M. Aug., xxiv, 5, 1886. Jacobson, A. f. O., x (2), 55, 1864.
Cohen and MacNeal. A. of 0., I, 128, 1921. Mathewson. Am. J. O., xiii. 880, 1930.
Coston. A. of O., xv, 696, 1936. Neame. Brit. J. O. vii, 209, 1923.
Cushing. Brain, xiv, 282, 1922. Neame and Wolff. Brit. J. O., ix. 609, 1925.
Cushing and Eisenhardt. Meningiomas, Schwarz. A. f. O. cxxxv, 247, 1936.
Springfield, Ill., 287, 1938. Stallard. Brit. J. O., xix, 576, 1935.
Gilchrist. T. O. S. Kliv, 196, 1924. Stevenson. T. O. S., xlvii, 417, 1927.
Goldzieher. A. f. O., xix (3), 139, 1873. Twelmeyer. K. M. Aug., lxx, 360, 1923.
Griffith. T. O. S., xxxi, 132, 1911. Verhoeff. Penfield's Cytology and Cellular
Henderson. Brit. J. Surg., xxvi, 124, 1938. Path. of the Nervous System, N.Y. iii,
Hudson. R. L. O. H. Rep., xviii, 317, 1912. 1029, 1932. -
2. FIBROMATA
Fibromata of the optic nerve-sheaths are extremely rare, and,
according to Hudson (1912), are characterized by an enormous and diffuse
fibrous hyperplasia and a benign tendency, affecting the optic nerve from
Fig. 2661.-FIBRoma of OPTIC NERVE-
sHEATH (Gilchrist, T. O. S). Fig. 2662.-FIBRoma of OPTIC NERVE-
SHEATH (Parsons, T. O. S.).
pressure symptoms only. Parsons (1903) concluded that such neoplasms
1 p. 3097.

3.09.4 TEXT-BOOK OF OPHTHALMOLOGY
should be regarded as secondary in nature, being caused by some type of
irritation, but sufficient material has not been available to allow of reliable
conclusions. As a rule the dural sheath alone is involved, but the mass of
the tumour may fill the whole orbit (Figs. 2661–62), moreover it blends
with the superficial layers of the sclera, obliterates Tenon's capsule and
fuses with the muscle-sheaths. The structure of the tumour is also vastly
Fig. 2663.-MENINGEAL FIBRoblastonia or Optic Shearh.
Arising from the arachnoid cells of the optic sheath. D. The dura. Beneath
it the sub-arachnoid space is distended with nests of tumour cells (T) and psammoma
bodies (Pb). P. Pia. (Cone and MacMillan).
different from that of the endotheliomata. Instead of being essentially
cellular it is essentially fibrous, with only islands of cell-infiltration in the
periphery consisting mainly of small round cells and plasma cells, while
the vessels show endothelial proliferation and perivascular infiltration.
Hudson. R. L. O. H. Rep., xviii, 317, 1912. Parsons. T. O. S., xxiii, 116, 1903.
BASAL MENINGIOMATA
Anterior basal meningiomata in association with the intra-cranial portion of the
nerve also occur, a subject, however, which is essentially outside the scope of the
present section. These are endotheliomatous in nature (Cushing, 1922), and may
occupy various sites.

DISEASES OF THE OPTIC! NERVE 3095
(a) Those associated with the posterior part of the olfactory groove, which are
characterized by a long history of monocular blindness with optic atrophy due to
compression of the nerve before symptoms of increased intra-cranial pressure supervene
(Cushing, 1927; Elsberg, 1935 ; Olivecrona and Urban, 1935; David and Askenasy,
1937).
(b) Those associated with the tuberculum selloe which show a progressive bitemporal
hemianopia and produce total blindness without X-ray evidences or signs of increased
intra-cranial pressure (Holmes and Sargent, 1927).
(c) Those occurring in association with the sphenoid ridge which produce an
isolated failure of vision in one eye : increased intra-cranial pressure and localizing
signs are late in appearing, but X-ray evidence of erosion and hyperostosis is usually
unmistakable (Cushing and Eisenhardt, 1929; Elsberg and Dyke, 1934; Alpers and
Groff, 1934).
Most of these tumours, unless they have attained a large size, are amenable to
surgical removal or diathermic destruction, although partial removal of a frontal lobe
may be necessary (Henderson, 1938).
Alpers and Groff. A. of Neurol. Psy.., xxxi, Elsberg. Laryngoscope, xlv., 7 12, 1:35.
7 13, 1934. Elsberg and Dyke. A. of O., xii, 644, 1934.
Cushing. Brain, xlv. 282, 1922. Henderson. Brit. J. Surg., xxvi, 124, 1938.
Lancet, i, 1329, 1927. Holmes and Sargent. Brain, l, 518, 1927.
Cushing and Eisenhardt. A. of O., i, 168, Olivecrona and Urban. Beit. z. clim. Chir.,
1929. clxi, 224, 1935.
David and Askenasy. Rev. Neurol., lxviii,
489, 1937.
NEURO-ECTODERMAL TU MOURS
I. NEURINOMATA
We have already seen that neuro-fibromatous hyperplasias occur in association
with the optic nerve, sometimes in company with a gliosis of the nerve tissue,” and
sometimes associated with psammomatous tumours in the sheaths in v. Reckling-
hausen’s disease (Shapland and Greenfield, 1935). Discrete neurinomata have also
been reported as a great rarity arising from the dural sheaths of the nerve (Gilchrist,
1924; Wohlwill, 1931 ; Jessel, 1933; Müller, 1934).
Gilchrist. T. O. S., xciv, 108, 1924. Shapland and Greenfield. T. O. S., Iv, 257,
Jessel. Z. f. Aug., lxxxii, 31, 1933. 1935.
Müller. K. M. Aug., xcii, 592, 1934. Wohlwill. Kurzes Hb. d. Oph., vi, 66, 1931.
2. NAFVI AND MALIGNANT MELANOMATA
SIMPLE MELANOMATA forming pigmentary plaques occur not infrequently on the
optic disc (Coats, 1907 : Oguchi, 1933) * presumably arising from uveal elements
incorporated in its tissues. MALIGNANT MELANOMATA, however, are a rarity, but have
been described by Vasquez-Barrière (1911), Orloff (1915), Speciale-Cirincione (1925),
Oguchi (1933), Levine (1935) and Bucalossi (1935). The tumour may grow forwards
1 p. 3087. * Vol. II, p. 1397.
3.096 TEXT-BOOK OF OPHTHALMOLOGY
and project into the vitreous, but more usually permeates backwards through the
lamina cribrosa which, like the membrane of Bruch, acts as a bottle-neck, whereafter
the neoplastic tissue spreads out in the
tissues of the nerve (Fig. 2664). Here
it infiltrates first the supporting tissue
of the septa, compressing the nerve
fibres which undergo atrophy and are
gradually replaced by melanotic cells
(Fig. 2665): there is little reaction or
glial proliferation. As a rule the dura is
not invaded, since it would appear to
offer greater resistance than the nerve
tissue. Any pigmented mass upon the
disc should be closely observed, and on
its showing any signs of growth an early
enucleation should be performed with
as free an excision of the nerve as is
practicable.
Fig. 2664.-MALign ANT MELANoMA or A reddish vascular tumour arising
OPTic Disc (Levine, A. of 0.). from the dise which became anaemic on
pressure on the globe was described by
Purtscher (1936); no pathological examination was made, but it may have been a
vascularized naevus.
Fig. 2665.-MALIGNANT MELANoMA or Optic Disc.
Cross-section of nerve of same case as Fig. 2664 (Levine).
Bucalossi. An-dº Ott., lxiii. 676, 1935. Purtscher. Z. f. Aug., lxxxviii, 217, 1936.
Coats. T. O. S., xvii, 225, 1907. Speciale-Cirincione An, di Ott, liii. 850, 1925.
Levine. A. of 0., xiv. 229, 1935. Vasquez-Barrière. K. M. Aug., xlix, 43, 73,
Oguchi. A. f. O. cxxx, 427, 1933. 1911.
Orloff. K. M. Aug., iv, 313, 1915.


DISEASES OF THE OPTIC NERVE 3097
The Treatment of Primary Tumours of the Optic Nerve and its Sheaths
The treatment of election of primary tumours of the optic nerve and its
sheaths is as early excision as is practicable. For this, several methods are
available. If all the signs point to an intra-orbital growth without intra-
cranial extension, and if the optic foramen is not radiologically enlarged,
an orbital operation is indicated. If it is deemed necessary to sacrifice the
eye, a simple excision of the globe together with the optic nerve up to the
apex of the orbit is sufficient, or, in the case of an extra-dural endothelioma,
an exenteration of the orbit. The eye, however, can frequently be saved, in
which case it is sometimes possible to section the nerve near the apex of the
orbit and behind the globe through an anterior incision which is probably
most conveniently placed at the upper or outer bony margin of the orbit
(Knapp, 1874 ; Lagrange, 1892; Collins, 1912 ; Benedict, 1934). A more
satisfactory approach, however, is by a Krönlein operation in which the
bony lateral wall of the orbit is displaced in a flap and the whole of the
cavity thrown open. This operation, which was originally devised by
Krönlein in 1886 for the removal of a dermoid cyst, was first employed for
the removal of a tumour of the optic nerve by Braunschweig (1893), and
rapidly became established in surgical practice (see Werner, 1903, who
collected 21 cases from the literature): it provides a good approach for
complete removal of the tumour, at the same time leaving the eye intact.
In suitable cases a good cosmetic result may well be obtained (Knapp, 1926;
Westkamp, 1935; and many others); but at other times an unsightly ptosis
or a strabismus results, while degenerative changes in the cornea owing to
the development of anaesthesia may cause much trouble (Neame, 1923; and
others). Again, owing to extension of the tumour, this technique is
frequently inadequate, for although orbital recurrences do not follow, central
infiltration, while it may be slow and delayed for many years, may ultimately
prove fatal. Moreover, there is a certain risk of meningitis, which occurred
in 10% of the cases collected by Hudson (1912): it is true that some of these
belonged to the pre-aseptic era, but nevertheless the risk exists when intra-
cranial extension has occurred.
When intra-cranial extension is suspected to have occurred, or when
an orbital operation has been performed and examination of the cut end
of the nerve shows that removal has been incomplete, an intra-cranial
operation should be considered by the trans-frontal route (Dandy, 1922;
Martin and Cushing, 1923; Stammers, 1931; and others). If an orbital
operation has already been performed and has been found to be inadequate,
the wound should be allowed to heal before removal is completed by the
intra-cranial operation in order to avoid meningitis and a leak of cerebro-
spinal fluid. If it is obvious that intra-cranial extension has occurred,
the procedure of choice is undoubtedly a trans-frontal operation with
unroofing of the orbit, which allows adequate approach to the entire length
of the tumour and sectioning of the nerve proximally and distally to it.
3098 TEXT-BOOK OF OPHTHALMOLOGY
In experienced hands such an operation carries a low mortality, adequate
dural closure prevents leakage of the cerebro-spinal fluid and the risk of
meningitis is negligible. If, however, it becomes apparent that enucleation
of the globe is also necessary, this should be delayed until the wound has
healed, for meningitis develops if craniotomy and enucleation are performed
at the same time. It is to be remembered that if the chiasma is involved
surgical intervention is contra-indicated, for in no case has any beneficial
result been obtained, the patient usually dying within 48 hours in hyper-
pyrexia, probably as a result of a disturbance of the heat-regulating centre
behind this structure (Martin and Cushing, 1923; Grinker, 1930; Mehney,
1936; and others).
Apart from operative treatment the only other possible method is by
high voltage roentgen therapy; but this has proved of little significant
value.
Benedict. Surg. Gym. Obst., lviii, 383, 1934. Knapp, H. K. M. Aug., xii, 439, 1874.
Braunschweig. A. f. O., xxxix (4), 1, 1893. Lagrange. An. d’Oc., cvii, 378, 1892.
Collins. T. O. S., xxxii, 396, 1912. Martin and Cushing. A. of O., lii, 209, 1923.
Dandy. Am. J. O., v, 169, 1922. Mehney. A. of O., xvi, 95, 1936.
Grinker. A. of O., iv, 497, 1930. Neame. Brit. J. O., vii, 209, 1923.
Hudson. R. L. O. H. Rep., xviii, 317, 1912. Stammers. T. O. S., li, 555, 1931.
Knapp, A. Contrib. to Oph. Science, Wiscon., Werner. T. O. S., xxiii, 82, 1903.
1926. Westkamp. A. f. O., xiii, 630, 1935.
B. Secondary Tumours
Secondary tumours of the optic nerve include those which affect this
tissue by direct spread from adjacent structures, and those which appear
as metastatic deposits. The first class includes malignant melanomata of
the choroid, in which such a method of spread is relatively rare,” and
retinoblastomata, in which extension up the nerve is common and exuberant,
forming the common fatal termination in these cases.”
METASTATIC TU MOU RS
Metastatic tumours in the optic nerve and its sheaths are very rare
indeed, and only a few cases are to be found in the literature.
1. CARCINOMA
Metastatic carcinoma is the most common tumour of this type. It may
be deposited in the substance of the nerve itself when the nerve-fibres and
ultimately the septa are replaced by neoplastic tissue of the type of the parent
tumour (Figs. 2666–67). Such an occurrence has been noted in a bronchial
1 p. 2498. * p. 2820.
DISEASES OF THE OPTIC NERVE 3.099
Fig. 2666.-METAstATIC CARCINoMA or OPTIC NERVE.
(McDannald and Payne, A. of 0.)
Fig. 2667.-METAstATIC CARCINoMA of OPTIC NERVE
(McDannald and Payne).


3100 TEXT-BOOK OF OPHTHALMOLOGY
carcinoma (Elschnig, 1891) or in carcinoma of the breast (Holden, 1902;
McDannald and Payne, 1934). An original focus in the disc is an exception
(Davis, 1932, in a carcinoma of the breast). Alternatively, and more
commonly, the deposit occurs in the sheaths of the nerve in which case the
nerve is first compressed and then infiltrated (Fig. 2668). This has been
reported in carcinoma of the kidney (Elschnig, 1891), the female genitals
(v. Haselberg, 1914), the ovary (Krohn, 1872), the lung (Ginsberg, 1921),
the breast (Behr, 1922), and the stomach (Goldstein and Wexler, 1931).
Sometimes this meningeal invasion is associated with and follows from a
widespread meningeal involvement including the chiasmal region, a syndrome
noted several times with carcinoma of the stomach (Saxer, 1902; Marchand,
1907; Cords, 1922; Terry and Dunphy, 1933); and at other times an
Fig. 2668–METAstatic CARCINoMA or Optic NERVE-sheath
(Goldstein and Wexler, A. of 0.).
orbital metastasis envelops and finally infiltrates the nerve (Schoeller,
1883; Uhthoff, 1891). A unique case was recorded by Cohen and MacNeal
(1921) in which, 16 months after the removalofascirrhus cancer of the breast,
an endotheliomatous tumour was found in the sheath of an optic nerve
containing nests of carcinomatous metastases.
The symptoms of such cases are those of a tumour of the optic nerve,
with, however, as a rule a very considerable degree of pain, and early and
complete amaurosis. Ophthalmoscopically pallor or oadema of the dise
is usual. On the other hand, especially when the sheaths only are involved,
there may be a complete absence of subjective and objective signs, but this
is exceptional (Goldstein and Wexler, 1931). Occurring as they do in
patients who must of necessity die within a short time, such tumours should
be left alone unless severe pain necessitates their removal, which in this
case should be done in the simplest possible way.

DISEASES OF THE OPTIC NERVE 3101
2. SARCOMATA
A few cases of metastases have been reported as part of the general
sarcomatosis following a primary mediastinal growth. Heine (1899)
described one where the metastatic growth was confined to the disc ;
Ballantyne (1906) examined one in which a tumour of the nerve-stem
spread through the lamina and became apparent intra-ocularly ; and
v. Meigs and de Schweinitz (1894) one wherein the intra-cranial part of the
nerve was affected.
A MALIGNANT MELANOMA of the skin has given rise to a metastatic
pigmented tumour of the disc (Schiess-Gemuseus and Roth, 1879).
Ballantyne. T. O. S., xxvi, 111, 1906.
McDannald and Payne. A. of O., xii, 86,
Behr. K. M. Aug., lxix., 788, 1922.
Cohen and MacNeal. A. of O., l, 128, 1921.
Cords. B. O. G. Heidel., xliii, 293, 1922.
Davis. A. of O., viii, 226, 1932.
Elschnig. A. f. Aug., xxii, 149, 1891.
Ginsberg. K. M. Aug., lxvii, 232, 1921.
Goldstein and Wexler. A. of O., vi, 414, 1931.
v. Haselberg. Berlin. kl. W., li, 1237, 1914.
Heine. K. M. Aug., xxxvii, 326, 1899.
Holden. A. of O., xxxi, 427, 1902.
Rrohn. K. M. Aug., x, 103, 1872.
Marchand. Münch. med. W., xiii, 636, 1907.
1934.
v. Meigs and de Schweinitz.
Sc., cviii, 193, 1894.
Saxer. Verhandl. d. deut. path. Ges., Karlsbad,
Am. J. Med.
v, 161, 1902.
Schiess-Gemuseus and Roth. A. f. O., xxv (2),
I77, 1879.
Schoeller. Cb. pr. Aug., vii, 236, 1883.
Terry and Dunphy. A. of O., x, 611, 1933.
Uhthoff. Internat. Beit. 2. wiss. Med., ii, 423,
1891.
CHAPTER, XXXVIII
DISEASES OF THE LENS
I. GENERAL CONSIDERATIONS
SINCE the lens is devoid of blood-vessels and lies, an isolated colony
of cells in a capsule, suspended in the intra-ocular fluid, its pathology is
very much more simple than that of most other tissues. Its avascularity
makes the typical reaction to pathological injury impossible ; inflammatory
processes do not therefore occur, and the result of such traumata is merely
passive and degenerative. The only cells in the lens, indeed, capable of
regenerative activity or of an active response to irritation are those of the
epithelial layer beneath the anterior and equatorial capsule. Since, however,
the maintenance of optical transparency—the primary necessity of the
function of the lens—depends upon the smooth working of a complicated
metabolism which is precluded from active response to any insults offered
to it, a whole host of conditions—traumatic, toxic or metabolic, local or
systemic—are liable to occur wherein its chemistry is upset, and degenera-
tion and consequent opacification result. Thus although limited in their
variety, diseases of the lens are not rare ; and apart from changes which
may occur in the capsule, they are essentially limited to the optical dis-
abilities of a failure to maintain its transparency (cataract) and a failure to
maintain its position, both of which, of course, have serious functional
results.
It is to be remembered that although primary inflammation of the lens
does not occur, if the capsule is broken through in the presence of an intra-
ocular inflammation, pus, phagocytic cells and vascular elements may invade
its substance through the capsular opening causing a secondary inflamma-
tion. The pathology of such a development has already been dealt with 1:
the lytic properties of the pus cells dissolve the lens fibres, granulation
tissue and fibroblasts following in their wake convert the lens into an
organized vascularized mass of connective tissue, and eventually a firm
and distorted cicatrix is formed which may finally become ossified. It is
important, however, that in these changes the tissues of the lens retain
their passive rôle.
THE HEALING OF WOUNDS
As a general rule wounds in the lens of man do not heal ; if the capsule
is perforated, either by accident or design as in the operation of discission,
1 p. 2177.
3102
I)ISEASES OF THE LENS 31 03
the rent remains open, the lens fibres coming into contact with the aqueous
imbibe fluid freely, swell up and become opaque, sometimes within a few
hours, a process which we shall consider more fully when dealing with
cataract. Occasionally with small punctures, as often happens in a dis-
cission Operation, the wound becomes plugged and a stationary partial
cataract results. More usually if the laceration has been large, the capsule
gapes, the swollen lens material protrudes through the wound, and soon
becomes disintegrated by lytic ferments ; this allows more lens fibres to
come into contact with the aqueous until eventually the entire lens becomes
Opaque. In young persons the end-result may be a complete absorption of
the lens, apart from the capsule, but in older subjects the sclerosed state of
the central fibres forbids this happy termination.
In animals (rabbits, etc), however, the reaction is different, for the
Opacity tends to be more localized : the formation of a complete cataract
is exceptional and if the anterior or equatorial capsule is involved, a successful
attempt at healing may be made by proliferation of the sub-capsular epithe-
lium. The question was first adequately studied by Schlösser (1887) and
Schirmer (1889), whose findings have been confirmed by later workers
(Sanna, 1931 ; and others). The epithelium around the wound, as we have
seen to occur in wounds of the cornea, becomes pushed towards the
edges, the cells cease to be regularly cubical, become flattened, undergo
mitosis and begin to proliferate. These cells have the appearance and
staining reactions of connective tissue fibroblasts and at times have been
mistaken for such ; but, availing himself of their known capacity for
proliferation in tissue culture (Kirby, 1927; Kirby, Estey and Tabor,
1929), Bakker (1936) proved their ectodermal origin by observing their
formation from the sub-capsular cells in vitro. The phenomenon is therefore
a typical example of metaplasia. Finally, normal cubical cells grow along
the anterior and posterior surfaces of the cicatricial mass which slowly
secrete a hyaline membrane similar to the normal capsule.
It will be remembered that in man this process is identical to that which
causes an anterior polar cataract 4; a similar process also follows toxic or
mechanical injury in adult life (Lamb, 1937). Proliferative changes in the
anterior epithelium also occur after tituration of the lens (Schirmer, 1888);
a similar epithelial new-formation is seen lining the posterior surface of
capsular hyaline bodies (Becker, 1883), extending to cover the posterior
capsule in a degenerated and shrinking cataractous lens in which case the
large vesicular cells of Wedl may develop (Becker, 1883; Collins, 1898), and
in association with the after-cataract left behind following an extra-capsular
extraction,” where the large globular cells of Elschnig (1911) may appear.
A slighter degree of proliferation may also occur in the intact lens after
contusions (Vogt, 1921) or in the dislocated lens (Cowan and Fry, 1937).
1 Vol. II, p. 1359. * p. 3232.
T.O. —WOL. III. 3 T
3104 TEXT-BOOK OF OPHTHALMOLOGY
Bakker. A. f. 0., exxxv, 581; exxxvi, 166, Kirby, Estey and Tabor. A. of O., i. 358, 1929
333, 1936. Lamb. A. of 0., xvii. 877, 1937.
Becker. Zur Anat, d, gesunden w. kranken Sanna. A. di Ott., lix, 543, 1931.
Lºnse, Wiesbaden, 1883. Schirmer. A. f. O., xxxiv, 220, 1888;
Collins. T. O. S., xviii, 124, 1898. xxxv (3), 147, 1889.
Cowan and Fry, A. of 0., xviii, 13, 1937. Schlösser. Earper. Studie über traumatische
Elschnig, K. M. Aug., xlix, 444, 1911. Kataract, München, 1887.
Kirby. A. of O. lvi. 450, 1927. Vogt. Atlas, Berlin, 1921.
II. THE CAPSULE AND SUB-CAPSULAR EPITHELIUM
Capsular ExFoliation
Although it usually appears as a homogeneous structureless membrane, it has
long been known that the lens capsule is composite. An outer layer was first demon-
strated by Berger (1882) after maceration with potassium permanganate; this he called
the zoNULAR LAMELLA, a structure which Retzius (1894) termed the PER1-capsular
Fig. 2669-THE Lºss Capsule.
After discission. The separation of the zonular lamella from the capsule
proper is well seen, each of the two parts being divided into separate layers.
Some of the cuboidal epithelial cells are attached to the cortical remains (Tooke,
Brit. J. O.).
MEMBRANE. It was soon seen pathologically that in addition to this outer layer, the
capsule was made up of severallamellae between which leucocytes from a peri-lenticular
exudate might penetrate or into which the capsule might split after trauma (Fig. 2669).
This layering may be demonstrated histologically in the normal capsule by staining
with aniline blue (Beauvieux, 1922) or by the use of the silver impregnation method of
Bielschowsky (Busacca. 1929). Evento-day, however, there is not complete agreement
as to the exact anatomical arrangement, some authors (Vogt, 1925–32; Elsehnig,

DISEASES OF THE LENS 3105
1929) believing that the zonular lamella (that is, the layer to which the zonular fibres
are attached) extends completely over the anterior surface, while others (Busacca,
1929) claim that there are three distinct structures, (1) a zonular lamella confined to
the lateral portions of the lens, (2) a fine peri-capsular membrane surrounding the
entire lens, and (3) a capsule proper which itself is composed of several layers. In
the first view the common exfoliative process affects primarily the zonular lamella;
in the second, a degenerative detachment of the zonular lamella results in a spontaneous
luxation of the lens, while the exfoliation about to be described affects first the peri-
capsular membrane and ultimately the capsule itself.
Whichever view is correct, the phenomenon remained a pathological curiosity
without apparent clinical importance, until Meesmann (1922) described a traumatic
rupture of the zonular lamella with dislocation of the lens, wherein, without a tear in
the capsule proper, the entire lamella could be seen as a fine crinkled membrane
FIG. 2670.--THE LEN's IN IRIDo-cyclitis.
To the left the corneal section. There is a marked aqueous flare. The arrow points
to a completely detached zonular lamella (drawn thicker than natural). Fluid clefts
in the anterior parts of the lens which is sub-luxated backwards (Harrison Butler,
T. O. S.).
floating in the anterior chamber at the pupillary margin. This finding was followed
by the observation by Elschnig (1922–23) and Kubik (1923) of a spontaneous rupture
and exfoliation of the lamella in glass-blowers. Soon after this, gross exfoliation
became generally recognized, while the finer type associated with senility became known
and excited even more interest. Some time previously in Scandinavian literature a
report had been made of a flaky appearance on the iris by Lindberg (1917) and on the
surface of the lens by Malling (1923), but their observations excited no interest;
eventually, however, the subject was brought into prominence by Vogt (1925–32),
who originally (1921) had mistaken the condition for remnants of the pupillary mem-
brane, but now assessed it at its true value and, as Lindberg (1917) and Malling
(1923) had done, stressed its possible clinical importance in the aetiology of chronic
glaucoma.
Exfoliation of the superficial layers of the capsule occurs in four
conditions.

3 T 2
3106 TEXT-BOOK OF OPHTHALMOLOGY
1. Traumatic detachment (Meesmann, 1922; Butler, 1938; and others)1
(see Fig. 2771).
2. Toa'ic—occurring in atrophic eyes (Elschnig, 1923–29: Butler,
1938), after prolonged irido-cyclitis (Rohrschneider, 1936; Butler, 1938)
(Fig. 2670), or after the lodgement of a metallic foreign body in the lens
(Vogt, 1932). *
3. By the action of heat, when a relatively coarse, thick, single lamella
peels off the anterior surface of the lens, which may wave about in the
anterior chamber or curl up upon itself like a roll of parchment (Elschnig,
1922–23; Kubik, 1923 ; Goulden, 1925; Weill and Levy, 1926; Rotter,
1926; Cords, 1926; Stein, 1926; and many others).”
4. Senile eafoliation, wherein a fine desoluamation occurs in successive
layers, and disintegrates to form a powdery deposit on the iris and in the
angle of the anterior chamber.
SENILE EXFOLIATION OF THE LENS CAPSULE
Despite its late clinical recognition, senile exfoliation of the lens
capsule is not particularly uncommon. In its slighter degrees, however, it
is difficult to see and since the changes may be confined to the periphery of
the lens, a mydriatic may be necessary for its recognition, facts which
account for the varied estimates of its incidence. It occurs in old age,
usually between 60 and 80, the youngest recorded cases being 56 (Alling,
1927), 55 (Rehsteiner, 1929), 55, (Busacca, 1929), and 45 years of age
(Trantas, 1929).
Handmann (1926) saw 3 cases in 7 years, and Kirby (1930) 7 in a like period. At
Zürich, Rehsteiner gave an incidence of 4 in 238 cases over 60 years (1.7%), Baumgart
(1933) 46 in 611 cases over 50 (7.5%), Hörven (1937) 19 in 237 cases over 50 (8%), and
Trantas (1929) 42 in 237 cases over 55, most of which were examined without mydriatics;
the last observer believed that the phenomenon would be found in 25% of persons
over 55 years of age.
It occurs along with other senile changes in the eye, particularly with cataract:
thus Lindberg (1917) found exfoliation in 28 out of 142 cases of cataract (30%), Busacca
(1929) in 26%, and Hörven (1937) in 10 out of 55 cataractous patients. Its association
with glaucoma will be noted later.
The clinical picture presented by all cases is very similar. It comprises
essentially three features:
(a) An exfoliation of the anterior lens capsule in flakes distributed as
a homogeneous disc in the pupillary region and as a granular girdle round
the periphery with a clear band between the two affected areas.
(b) The appearance of flakes on the zonular fibres.
(c) The deposition of flakes on the iris, the cornea and in the angle of
the anterior chamber.
The central disc on the anterior surface of the lens corresponds in extent
1 Vol. IV. 2 Vol. IV.
DISEASES OF THE LENS 3107
to the minimum dilatation of the pupil (Fig. 2671). It is usually of a
uniform homogeneous structure and may be difficult to see, appearing as a
faint milky fleck without a sharp boundary. At other times it is quite
Fig. 2671.-CAPsular ExFoliation (Hörven, Brit. J. O.),
conspicuous, with a clearly defined edge which is often curled up so that a
ring of tags marks the position of the maximal pupillary contraction. Out-
side this, lying behind the iris usually in the outer third of the distance
Figs. 2672–73.-SENILE CAPsular ExFoliation.
from the anterior pole to the equator, is a much more conspicuous girdle of
a coarse granular appearance like hoar-frost, looking as if the lens had been
sprinkled over with a white powder (Fig. 2672). This peripheral band


3.108 TEXT-BOOK OF OPHTHALMOLOGY
varies in breadth, and although it is separated from the central disc by
an area wherein the capsule appears clinically normal, the axial border may
be denticulate, and bridges may extend across the clear girdle to reach the
central disc (Fig. 2673). At the peripheral border the granular incrustation
may gradually fade away merging with normal capsule, or the boundary
may be distinct and sharp, in which case it is always festooned with radial
projections striking out towards the zonular fibres. No exfoliation at the
equatorial border has been observed. Although it is as yet unknown where the
process of exfoliation starts, whether first at the periphery or as a general
process over the entire surface, the limitation of the central disc at the
pupillary margin, the fact that it may be made to decrease in size if the
Fig. 26.74—The Zonule in Capsular Expoitation
(Hörven, Brit. J. O.).
pupil is kept contracted by miotics, and the presence of exfoliated fragments
appearing on the iris and elsewhere in the eye, all indicate that probably
the process affects the entire anterior capsule and that the movements
of the iris rub off the incrustations immediately under it so that the
peculiar configuration results of a central disc and a peripheral band separated
by an apparently unaffected zone.
That the zonular fibres become powdered with white crust-like particles
was noted incidentally by Trantas (1929) and Vogt (1931), but that this
forms an integral part of the clinical picture was first pointed out by Hörven
(1936), who found this appearance invariably present when the zonule was
exposed to view by an iridectomy for glaucoma or in enucleated eyes
(Fig. 2674). It is unknown whether it represents a primary degeneration
of the zonule, or whether the phenomenon is of secondary origin, the flakes
being deposited from the capsule.

DISEASES OF THE LENS 3109
Such a deposit is a very constant feature, however, on the iris, on
the pupillary margin of which a powdery accumulation of white flakes is
frequently seen (Fig. 2675). They occur only in the presence of lenticular
changes and seem obviously to be derived from the capsule, having either
desquamated spontaneously or been rubbed off by the movements of the
iris. It is interesting that Wollenberg (1926) found exfoliation in one eye
where the pupil was active but none in its fellow wherein the pupil was
fixed. The flakes are constantly changing in quantity, appearing here and
disappearing there; and the fact that they are seen immediately after an
iridectomy in dense masses along the edge of the coloboma suggests that they
may cover most of the posterior surface of the iris. Such flakes are also
Fig. 2675.-ExFoliation of THE CAPsule.
Powdery deposit on the margin of the iris (Garrow, Brit. J. O.).
seen on remnants of the pupillary membrane, appearing like rime on a
telegraph wire; they float in the anterior chamber; they become attached
to the posterior surface of the cornea; and they accumulate in the angle
of the anterior chamber where they may be seen gonioscopically (Barkan,
1938) or in pathological preparations (Sobhy Bey, 1932).
Pathological examinations of such eyes have shown that the appearance
on the capsule is due to the desquamation of its superficial layers as a
purely degenerative change without any inflammatory evidences (Busacca,
1927–29; Rehsteiner, 1929; Vogt, 1930; Sobhy Bey, 1932) (Figs, 2676–77).

31.10 TEXT-BOOK OF OPHTHALMOLOGY
Sometimes the capsule remains of normal thickness and the superficial
lamella detaches itself, and at other times it first becomes thickened and
puffy, the thickened area eventually becoming rubbed away so that it
becomes smooth again but thinner than normal. Handmann (1926)
advanced the theory that the flakes on the iris represented a degenerative
Fig. 2676–Capsular ExFoliation.
Peeling of the superficial layers of the capsule (Garrow and Michaelson).
change of the iris epithelium which produced a hyaline material, but patho-
logical investigations have made their capsular origin clear.
The process is of insidious origin, and of long duration, taking many
years for its full development. It is without symptomatology and the only
Fig. 2677-Capsular Expoitatios.
Fine strands peeling from the surface of the capsule (Garrow and Michaelson).
complication which may arise—and it is a serious one—is the development
of glaucoma.
The relation between capsular exfoliation and glaucoma was recognized
by the discoverers of the condition. Lindberg (1917) found these changes
in 30 out of 60 cases of chronic primary glaucoma, and Malling (1938) in
33 out of 81 glaucomatous eyes. In his first paper on the subject, Vogt.


DISEASES OF THE LENS 31 II
(1925) found that of the 12 persons affected 9 suffered from advanced
“ primary * glaucoma, and, concluding that the glaucoma was in reality
secondary due to the blockage of the angle of the anterior chamber by
exfoliated material, he termed it glaucoma capsulare. That chronic glaucoma
is a common occurrence in patients with exfoliation is well established :
it may, indeed, be said to occur in some 70%.
The following statistics appear in the literature : 9 cases of glaucoma Out of 12
cases of exfoliation (Vogt, 1925), 34 out of 45 (Vogt, 1931), 27 out of 30 (Busacca,
1927), 14 out of 42 (Trantas, 1929), 50 out of 78 cases in the literature (Rehsteiner,
1929), 90 out of 156 cases in the literature (Grzedzielski, 1931), 29 out of 44 (Sobhy
Bey, 1932), 29 out of 46 (Baumgart, 1933).
Similarly a large proportion of cases of chronic glaucoma in elderly
persons are found to have exfoliation, estimates of which vary from 7%
to 93%.
Sixty cases of chronic glaucoma included 30 with exfoliation (Lindberg, 1917),
81 eyes included 33 (Malling, 1923), 38 included 16 (Busacca, 1929), 59 included 29
(Baumgart, 1933), 150 operated cases included 128, and 43 cases of glaucoma under
treatment included 40 with exfoliation (Hörven, 1937), 51 included 8 (Garrow, 1938).
Although some authorities deny any close relationship (Malling, 1938;
Butler, 1938), it must be admitted that the difference in the incidence of
exfoliation in glaucomatous subjects and in normal subjects seems too great
to be incidental and points to a relationship of cause and effect, a subject
which will be discussed again in dealing with this disease.
Alling. A. of O., lvi, l, 1927. Lindberg. Klin. undersökningar Över depig-
Barkan. T. O. S., lviii, 588, 1938. menteringen av pupillarramden och genom-
Baumgart. Boll. d’Oc., xii, 560, 1933. lysbarheten an iris. Helsingfors, 1917.
Beauvieux. A. d’O., xxxix, 491, 1922. Malling. Acta O., i, 79, 1923; xvi., 43,
Berger. A. f. O., xxviii (2), 28, 1882. 1938.
Busacca. A. f. O., csix, 135, 1927. B. O. G. Heidel., lim 369, 1936.
K. M. Aug., lxxxiii, 737, 1929. Meesmann. A. f. Aug., Xci, 261, 1922.
Butler. T. O. S., lviii, 575, 1938. Rehsteiner. K. M. Aug., lxxxii, 21, 1929.
Cords. Z. f. Aug., lx, 251, 1926. Retzius. Biol. Untersuch., vi, 67, 1894.
Elschnig. K. M. Aug., lxix, 732, 1922;
lxx, 325, 1923.
A. f. Aug., c-ci, 760, 1929.
Garrow. Brit. J. O., xxii, 214, 1938.
Goulden. T. O. S., xlv., 718, 1925.
Grzedzielski. A. f. O., csxvi, 409, 1931.
Handmann. K. M. Aug., lxxvi, 482, 1926.
Holloway and Cowan. Am. J. O., xiv., 189,
1931.
FIörven. Acta O., xiv, 231, 1936.
Brit. J. O., xxi, 625, 1937.
Kirby. A. of O., iv., 93, 1930.
Kubik. K. M. Aug., lxx, 327, 1923.
COLLOID BODIES.
Rohrschneider. K. M. Aug., xcvi, 31, 1936.
Rotter. K. M. Aug., lxxvi, 71, 1926.
Sobhy Bey. Brit. J. O., xvi, 65, 1932.
Stein. K. M. Aug., lxxvi, 75, 1926.
Tooke. Brit. J. O., xvii, 466, 1933.
Trantas. A. d’O., xlvi., 482, 1929.
Vogt. Atlas, Berlin, 1921.
K. M. Aug., lxxv, 1, 1925; lxxxiv, 1, 1930;
lxxxvi, 736, 1931 ; likxxix, 581, 587,
1932.
Z. f. Aug., lviii, 379, 1926; lxvi, 105, 1928.
Weill and Levy. An. d’Oc., clxiii, 748, 1926.
Wollenberg. K. M. Aug., lxxvii, 128, 1926.
Hyaline bodies (Drusen) may be formed on the
inner surface of the capsule in conditions of senility or in a proliferative
phase of epithelial activity (Becker, 1883).
Like the colloid bodies of
3112 TEXT-BOOK OF OPHTHALMOLOGY
Descemet's or Bruch's membranes, they probably represent deposits of
capsular material by the epithelial cells."
Becker, Zur Anat, d. gesunden w. kranken Linse, Wiesbaden, 1883.
SUB-CAPsul AR EPITHELLAL OPACITIES (SUB-CAPsulAR CATARACT)
The subject of congenital sub-capsular opacities has already been dealt with fully in
discussing congenital abnormalities * : the same phenomenon, however, also occurs in
adult life. Pathologically they are due to a proliferation of the cells of the anterior
epithelium of the lens, which results in a non-progressive opacification immediately
underneath the capsule, a process which is liable to occur whenever these cells become
stimulated to abnormal growth by mechanical or toxic stimuli. As we have seen,
they are found, often as multiple dots, in association with remnants of the pupillary
membrane or posterior synechiae, or they may become very obvious as an anterior
polar cataract which may be transformed into a pyramidal cataract,” a lesion formed
typically when the lens comes into contact with an inflamed cornea, especially after
perforation of an ulcer.
Fig. 2678-ANTERior Capsula R. CATARAct.
Meridional section through one side of the cataract showing proliferation of sub-
capsular epithelium and, to the left, new-formed connective tissue-like cells (Lamb,
A. of 0.).
Such opacities, however, may occur in later life when they may appear
in two conditions: (1) as the result of contusions and perforations, or
(2) as a “planar opacity "in complicated and hypermature cataracts wherein
a great deal of epithelial proliferation may take place (Fig. 2678). In those
cases the capsule itself is unaffected except that it may be wrinkled and its
curve modified so that over the affected area it loses its normal shagreen
(Vogt's sign, 1922); the essential change is limited to a proliferation of the
sub-capsular epithelium, with which, however, is sometimes associated a
degeneration of the subjacent lens fibres.
Such folds in the lens capsule may appear without proliferation of the sub-capsular
epithelium, in which case they are usually conditioned by a shrinking of the lens
substance (Bedell, 1927; Harms, 1927; Vogt, 1931; Bücklers, 1932–35).
* Vol. II, p. 1993. * Vol. II, p. 1358. * Vol. II, p. 1359.
* p. 3227.

DISEASES OF THE LENS 31.13
The histology of sub-capsular cataract in congenital cases has already
been described those occurring in post-natal life present a similar picture
(H. Müller, 1856; Knies, 1880; Becker, 1883; Wagenmann, 1889; Schirmer,
1889; Gepner, 1890; Treacher Collins, 1892–1908; Krüger, 1903; Peters,
1921; Beauvieux and Germain, 1922; Maucione, 1925; Bücklers, 1935; Lamb,
1937). The proliferating sub-capsular cells beneath the intact capsule lose
their cubical shape and become polygonal, and as they multiply they raise
up the capsule, sometimes making a distinct conical projection above the
level of the lens. Eventually they elongate, throw out long processes, and
Fig. 2679.-ANTERIoR CAPsulAR CATARAct.
Showing ingrowth of cubical epithelium behind the cataract (x 120) (Parsons).
develop into spindle-shaped cells resembling in every way fibrous connective
tissue (Fig. 2678). The phenomenon is a typical example of metaplasia.
After some time degenerative changes tend to occur. The whole tissue
may become condensed and converted into a laminated and almost structure-
less hyaline mass, around which the epithelial cells may proliferate, insinuat-
ing themselves between it and the lens (Fig. 2679). In so doing they may
secrete a new cuticular membrane, so that the appearance may be as if the
capsule had split into two layers at the margin of the opacity, one running
over it and one running underneath it. Alternatively depositions of fat or
lipoid material may occur, including a plentiful array of flat or needle-shaped
crystals of cholesterol of varying brilliant colours: the DystroPHIA
EPITHELIALIs LENTIs ADIPosa of v. Szily (1933) (Handmann, 1933; Sagher,
* Vol. II, p. 1358.

3114 TEXT-BOOK OF OPHTHALMOLOGY
1934). Calcareous degeneration is also relatively common, a process which
may give an appearance like stalactites (H. Müller, 1856; Krüger, 1903).
In cases of perforated ulcer when the anterior capsule has adhered to the
cornea, adhesions may form between the two. When the anterior chamber
reforms, the connective tissue which binds the two structures together
elongates, but the traction may distort the lens, drawing it out anteriorly into
a pyramid (PYRAMIDAL CATARACT) (Fig. 2680). In such a case the corneal
endothelium may grow over the lens secreting a hyaline membrane corre-
sponding to Descemet's membrane, so that the lens eventually becomes
covered by two cuticular membranes, one a new hyaline membrane covered
by endothelium, and the other the true capsule lined by cubical epithelium
(Fig. 2681) (Haring, 1897; de Vries, 1902: Parsons, 1905).
Fig. 2680,-Pyraminal CATARAct.
From a child, aged 8, after a perforating
hypopyon ulcer following measles. A small
anterior capsular cataract is to the left of the
summit (x 6) (Parsons).
Fig. 2681.-PyRAMinal CATARAct.
The same case as Fig. 2680, showing double
hyaline membrane on surface. The outer
membrane is covered by endothelium derived
from the cornea and is a euticular deposit
corresponding to Descemet's membrane.
The inner membrane is the lens capsule
(x 90) (Parsons).
The treatment of such cataracts depends essentially on the condition of the
lens itself. If the lens is clear the cataract is usually small, in which case
the vision may not be greatly impeded; if necessary it may be helped by
dilatation of the pupil or an optical iridectomy. If, however, the lens also is
opaque the treatment of the sub-capsular cataract becomes secondary to
that of the main lenticular opacity.
Beauvieux and Germain, A. d’O., xxxix,
285, 1922.
Becker Zur. Anat, d, gesunden w. kranken
Linse, Wiesbaden, 1883.
Bedell. J. Am. Med. As lºviii. 548, 1927.
Bücklers. K. M. Aug., lxxxix, 832, 1932;
xciv. 289, 1935.
Collins. T. O. S., xii, 89, 1892; xviii, 124,
1898.
Ophthalmoscope, vi, 577, 663, 1908.
Gepner. A. f. O., xxxvi (4), 255, 1890.
Handmann. K. M. Aug., xei, 488, 1933.
Haring. A. f. O., xliii (1), 25, 1897.
Harms. K. M. Aug., lxxviii. 57, 1927.

DISEASES OF THE LENS 3 115
Knies. K. M. Aug., xviii, 181, 1880. Sagher. K. M. Aug., xciii, 355, 1934.
Krüger. Z. f. Aug., ix, 35, 1903. Schirmer. A. f. O., xxxv, 220, 1889.
Lamb. A. of O., xvii, 877, 1937. v. Szily. K. M. Aug., xc, 607, 1933.
Maucione. A. di Ott., xxxi, 145, 1925. Vogt. A. f. O., cviii, 182, 1922.
Müller, H. A. f. O., ii, 1, 1856; iii, 55, 1857. Atlas, Berlin, 1931.
Parsons. Path. of the Eye, London, ii, 407, de Vries. A. f. O., liv, 500, 1902.
1905. Wagenmann. A. f. O., xxxv, 172, 1889.
Peters. A. f. O., cv, 154, 1921.
III. CATARACT
Historical. The history of cataract is long and extends backwards at least 3,000
years and probably further. The earliest authentic record which has been traced is in a
treatise on surgery written some thousands of years before Christ by the great surgeon
of ancient India, Sušruta " : as a pupil of Dhanwantari, the Father of Indian Medicine,
he was the first to practise and teach the principles of surgery. Long before the
Hippocratic era he taught the foundations of surgery based on anatomical dissections,
he practised aseptic surgery (advising that the operating room be fumigated with
sweet vapours and that the surgeon keep his hair and beard short, his nails and
hands clean and wear a sweet smelling dress) and apparently used some kind of inhala-
tion anaesthetic. In the wealth of his teaching on general surgery, he dealt systemati-
cally and elaborately with the anatomy, physiology, and pathology of the eye, and
he described in accurate and beautiful detail different varieties of cataract, giving an
admirable account of the technique of its treatment by couching and the post-operative
Care which he successfully practised. It would seem obvious that this extremely
advanced account was preceded by previous knowledge and experience in the rich
civilization of early Hindustan.
Although, however, Sušruta described cataract as an opacity of the lens due to a
derangement of the intra-ocular fluid, the learning of the Far East was forgotten, and
the subsequent history of the subject is strangely full of fantasies and prejudices.
The name itself is comparatively recent : like the older Latin name suffusio, it arose
Out of mediaeval Latin translations from the Arabic expressing the early view of its
pathology—the humour that flowed down into the eye. Where and when this patho-
logical concept was conceived is a mystery, for the Hippocratic writings made no
distinction between the blindness due to cataract and glaucoma. In any event the
first classical teachings are found in the writings of Celsus (25 B.C.—A.D. 50), who pro-
pounded a complete system of pathology and treatment derived presumably from the
Alexandrine School. In this view, a suffusio was a corrupt inspissated humour which
collected in the empty space between the pupil and the lens (a structure which was
considered to be situated in the centre of the globe): this obstructed the visual spirits,
but sight could be restored by removing the ill-humour either by displacing it by a
needle to another part of the eye or by breaking it up. The later Greek writers main-
tained and developed this view—glaucoma was a drying up of the lens itself and was
incurable, but cataract a humour in front of the lens which could be removed—a
pathology which was advocated by Galen (131–210), and was even retained in spite
of the demonstration by Kepler (1571–1630) that the lens was not the essential organ
of vision.
* Susruta Samhita, Vol. VII, Chapter 17, verses 57–73 ; dated B.C. 1000 (edited by Yadava
Sharma, Bombay, 1931). See Dutt, A. of O., xx, 1, 1938 : Bidyadhar, A. of O., xxii, 550,
1939.
31.16 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 26s2–Jacques Daviet-
1695–1752.

DISEASES OF THE LENS 31.17
Eventually, however, dissentient opinions began to appear, among the first of
which was that of the Frenchman Quarré (1643), who, as the ancient Hindus had done
thousands of years before, taught that cataract was an opacification of the lens itself.
But in spite of the actual anatomical demonstration of the opaque lens by Rolfinck in
1656, and the observation by Maitre-Jan at the end of the seventeenth century that
the material displaced in couching formed, not a thin membrane as had been supposed,
but a thick rounded body, it was not until the rediscovery of the fact by Brisseau in
1705 that the lens itself was displaced, that serious doubt was thrown upon the pre-
vailing humoral pathology. It is interesting that on Brisseau's discovery, his teacher,
Duverney, strongly advised him against publication lest he jeopardize his future by
arousing prejudice; but nevertheless, holding with excellent spirit that “those
who opposed his view had more at stake,” he raised a controversy in the Académie
Royale des Sciences of Paris," and eventually, with the support of Maitre-Jan and
later of Mery, carried the day, but not without an acrimonious fight which cost him
his position in the French Academy.
Hardly had this storm died down when a second arose on the publication by
Daviel in 1748 of his account of an extraction of the lens. His photograph appears
in Fig. 2682. Up to this time the treatment of cataract was practically confined to
depression of the lens. It is true that occasional mention had been made in the early
literature of radical methods of removing the lens, both in the Arabic writings and
in those of Galen ; the latter, for example, mentions extraction, and Ammar (eleventh
century), an Arabian, devised an operation of suction with a glass tube. It is true
also that St. Yves in 1722 extracted in toto a lens dislocated into the anterior chamber,
and that Petit removed piecemeal the fragments of a broken-up lens. But it was not
until a similar unplanned emergency was forced upon Daviel that he conceived a
planned extraction, and although it was taken up enthusiastically for a short time,
the majority of influential support returned to the older procedure of couching, so
that it was not until the lapse of almost 100 years that it became generally accepted as
the procedure of choice.
General AEtiology
.Etiologically cataract may be considered as a loss of transparency of the
lens developing as a result of altered physico-chemical processes within its
tissue ; but since our knowledge of the normal metabolism is yet far from
complete, no satisfactory solution of the more complex problem of its derange-
ments can be offered. Fundamentally the loss of transparency is due to a
disturbance of the intimate structure of the lens, the fibres of which form a
colloid system in which a large amount of water is bound. The disturbance
may be of two kinds : a swelling opacity, a reversible change producing a
diffractive effect in which the essential feature is a variation in the size of
the complex protein micellae ; or a coagulation opacity, an irreversible
chemical change producing a refractive effect in which the essential change
is a derangement and eventually a destruction of the micellar structure.
The survival of the lens is possible only when this structure is preserved.
Unlike the process of precipitation (a purely physical change brought about,
* MICHAEL BRISSEAU, médecin major des Hôpitaux du Roy et pensionnaire de la ville de
Tournay : Traité de la cataracte et du glaucoma. A Paris chez Laurent d'Houry 1709 avec
Approbation et Privilège du Roy.
3.118 TEXT-BOOK OF OPHTHALMOLOGY
for example, by the action of concentrated salts whereby aggregates of
chemically unchanged particles are thrown out of colloid solution) coagulation
?s an irreversible chemical change. It occurs in two stages—denaturation,
whereby the protein is altered chemically to an unstable, readily coagulable
form although it still retains its transparency, and agglutination, a physical
change whereby coagula are formed by the flocculation of the denatured
particles. The first change is probably essentially one of hydrolysis, while
the second process of agglutination is brought about most commonly by
alterations in the concentrations of salts and hydrogen ions. The two
processes usually occur simultaneously in experimental conditions, but may
be separated by an indefinite time-interval during which the denatured
protein, although showing definite chemical and physical changes involving
a decrease in stability, may remain unagglutinated, leaving the medium of
which it forms part transparent until such time as suitable conditions induce
flocculation and consequent opacity.
THE CHEMICAL PATHOLOGY OF THE LENs
A considerable amount of information has now accumulated as to the
chemical changes which occur in cataract, and although these observations
have as yet produced no definite clues to the aetiology of the condition and
must be considered largely as isolated findings, they will doubtless fit
eventually into a general scheme.
In comparing the difference between the normal and pathological lens,
we may group the various constituents into three classes : proteins, lipoids,
and water-soluble extractives.
(a) PROTEINs
From the point of view of the pathogenesis of cataract the proteins are
the most important constituent of the lens. As we have seen they are very
abundant, forming some 35% by weight of the whole tissue ; they comprise
(Mörner, 1894; Woods and Burky, 1928; Krause, 1932–34):-
. Cº-crystallin, a soluble pseudo-globulin prominent in the Outer cortex.
. 8-crystallin, a soluble globulin prominent in the inner cortex.
. y-crystallin, an albumin.
Albuminoid, an insoluble eu-globulin prominent in the nucleus.
. Traces of nucleo-protein.
. Traces of muco-protein.
:
In any individual mature fibre the albuminoid probably forms the inert
peripheral Zone, while the active central region contains the three crystallins.
The nucleo-protein is in the nucleus, and the muco-protein is presumably
extra-cellular and binds the fibres together, at the same time allowing them
to override one another in the movements of accommodation.
* Vol. I, p. 475 (484).
DISEASES OF THE LENS 3119
As age advances the total protein content increases (Jess, 1920; Shoji,
1923), the synthesis of growth being presumably accomplished by the absorp-
tion of amino-acids (lysine, cystine, histidine, tryptophane, etc.) from the
blood through the aqueous. Correspondingly, the content of water, the
medium wherein substances react, gets progressively less (Jess, 1913). As
age advances and in cataract, while the B-crystallin remains practically
constant in amount, o.-crystallin gets less and the insoluble albuminoid
increases in amount, the one presumably being converted into the other
(Krause, 1933). This decrease in soluble and increase in insoluble protein
with age is very considerable : thus in the young calf the ratio between
soluble and insoluble protein is 80 : 20 and in the old ox 40 : 60 (Jess, 1913–
20 ; Bürger and Schlomka, 1927). It follows that over the whole lens, as
the fibres of the cortex become gradually transposed in development to
become the fibres of the nucleus, the proportion of insoluble protein in the
central areas steadily rises, and, while the cortex of the young contains
practically no albuminoid, the nucleus of the aged contains little else
(Krause, 1933–38); the ageing of the whole lens is therefore a summation
of the same process which occurs in each individual fibre. It is thus
obvious that as the lens grows old (or becomes cataractous) active soluble
proteins are gradually converted into inactive substances, a process which
tends towards metabolic stability and inertia.
Proteolysis. Normally the rate of formation of new proteins in the lens
is greater than their breakdown, but under pathological conditions the reverse
may occur and autolysis set in. It has been known for long that after the
death of the lenticular fibres in cataract, the amount of amino-acids in the
lens and in the aqueous increases, suggesting hydrolysis of the proteins
and diffusion of the soluble end-products through the capsule (Clapp,
1911 ; Burdon-Cooper, 1914; Goldschmidt, 1914; Labbé and Lavagna,
1925; and others). The intimate mechanism of the reaction was estab-
lished by Krause (1933), who found the process to be determined by the
development of an acid reaction, in which circumstances two proteolytic
enzymes became active. The first (8-protease), which became active
between pH 7 and 4 with a maximum efficiency at pH 5, hydrolyses
the 8-crystallin and albumin into simpler proteins, and the second
(o-protease), which became active between pH 8 and 3, breaks up
these primary cleavage products into small fragments (peptones,
peptides and amino-acids). The second is inactive in the normal lens
because no primary cleavage products are available ; the first because of the
normal alkaline reaction. Apparently these enzymes have no effect upon
o-crystalline and albuminoid; but the former becomes insoluble at a pH
more acid than 6, while the latter is already insoluble and inert. Acidity of
the lens, therefore, however produced, throws all the protein constituents out
of action, some of them being coagulated and others broken down into amino-
acids which diffuse away. In young lens fibres, since the amount of albumi-
T.O.—WOL. III. 3 U.
3.120 TEXT-BOOK OF OPHTHALMOLOGY
noid is negligible, practically all the proteins are thus broken up. Since
complete hydrolysis increases the Osmotic pressure some 400 times, the
initial effect is an enormous imbibition of water with a consequent swelling,
which may be followed as diffusion proceeds by an almost complete dis-
appearance of the lenticular substance. In older fibres the presence of the
insoluble protein makes disappearance impossible, although the lens as a
whole shrinks owing to the loss of the 8-crystallin and albumin, the shrinkage
being necessarily most apparent in the Superficial layers. If, as occurs in
hypermature cataract, the capsule is impermeable, the soluble moiety cannot
escape, the lens becomes a sac holding a protein-rich fluid with a solid
albuminoid nucleus floating in it. If, on the other hand, the capsule is
ruptured, the proteases in the aqueous as well as phagocytic cells reach the
fibres, and these extra-lenticular enzymes, although they play no part in the
primary cataractous changes, help to complete the break-down of the
proteins (Rodigina, 1932).
It is important to note that the cleavage products of this process of hydrolysis
are toxic when injected into the aqueous humour and are capable of producing an
iritis (Rötth, 1929; Krause, 1934).
The following amino-acids have been isolated in the proteolysis of lenticular
protein (Jess, 1920)—glycocoll, alanine, valine, leucine, aspartic acid, arginine,
glutamic acid, tyrosine, protine, lysine, histidine, phenylalanine, serine, tryptophane,
cysteine-cystine.
(b) LIPOIDs
The lipoid content of the lens increases as age advances and in cataract.
Zehender, Matthiessen and Jacobsen (1879) found 2.1% cholesterolin dried normal
lenses of patients under 60 years of age, 2.4% in those over 60, and 2% in cataractous
lenses; Cahn (1881) found that a normal percentage of 0-62 cholesterol rose to 4-55
and 6-22 in cataract ; Jess (1922) found in the ox that the ether extract rose from
0.14% to 0.63% of the total weight of the tissue in oxen with age ; Bürger and Schlomka
(1927) found an increase of cholesterol with age in the bovine lens from 1.34% to
6.10% and Goldschmidt (1922) an increase of total lipoids in the human from 2.2%
to 8.35%, a greater increase, indeed, than he found in cataractous lenses. This
increase has been repeatedly confirmed, as by Adams (1929), Magnasco (1929),
Neuschüler (1930), Parhon and Werner (1931), Cahane (1931), and Salit (1931–37),
the latter of whom found in human cataractous lenses 0.21% cholesterol, 0.48–1.88%
lecithin and 1.53–4.0% saponifiable fat. On the whole, therefore, it would seem that
there is a significant increase of cholesterol both in aged and cataractous lenses (Salit
and O’Brien, 1935).
Many of the estimations on the lipoid substances, however, are of doubtful value
and their correlation is difficult from lack of standardization in the methods of estima-
tion and the terminology employed. This applies, for example, to myelin and the
doubly refracting lipoids. The term myelin is a vague one chemically, but includes
substances arising from phospho-lipoids and cerebrosides, while calcium Soaps, phos-
phates, carbonates and cholesterol esters are doubly refractive. Myelin substances
appear not to be present in the normal lens, but arising from the unmasking of phospho-
lipoids and cerebrosides, appear in cataract—in traumatic and complicated cataract
(Mettenheimer, 1857–58), in autolysis of the lens (Hoffmann, 1913), in senile and
lamellar cataract (Kranz, 1927), and in coronary cataract (Metzger, 1931).
DISEASES OF THE LENS 3121
While it seems thus beyond dispute that the total lipoids increase in age
and in cataract so greatly, indeed, that occasionally in the former (Purtscher,
1938) and quite frequently in the latter they may appear in visible crystalline
deposits (v. Graefe, 1854; Tweedy, 1873; Lang, 1895; Wessely, 1922;
Bunge, 1936; Kranz, 1927; and others)—their function in metabolism is
quite unknown. Of the several members of this group Salit (1935–37) found
that the phospho-lipoids decrease, the cholesterol remains practically
constant, and the unclassified fat increases as the cataract approaches
maturity. It is, of course, certain that the essential lipoids (phospho-lipoids,
cholesterol) are necessary for the life of the cell, and these substances presum-
ably have some biological significance of which we are as yet ignorant. It
may be that the increase in fat is a secondary deposition comparable to the
common process of fatty deposition occurring in senile and degenerating
tissues generally, depending essentially on a lessening of oxidative activity.
(c) EXTRACTIVES : INORGANIC MATERIALS
CALCIUM. Of the inorganic materials in the lens the change in the
calcium content in cataract is of the greatest significance. The normal lens
is poor in calcium, and the cataractous lens extremely rich : about this there
is unanimity among all observers. The first to draw attention to this
phenomenon was Burge (1909), who found that while the calcium content
of the normal lens was about 0.1% and that of the cataractous lens was
about 15%, i.e., an increase of 150 times. Subsequent observers have not
found so great a rise in concentration, but that a rise of considerable dimen-
sions, although of extremely variable degree, does occur in all types of
cataract, whether senile or produced experimentally, as by naphthalene or
parathyroidectomy, is universally admitted (Burdon-Cooper, 1928–33;
Adams, 1929; Salit, 1930–33; Kirby, 1931; Evans and Kern, 1931;
Mackay, Stewart and Robertson, 1932; Updegraff, 1932; Campbell, 1933;
Grabar and Nordmann, 1933; Lo Cascio, 1937; Rinaldi, 1937; and many
others). Moreover, the deposition increases progressively as the cataract
approaches maturity (Mackay, Stewart and Robertson, 1932), crystalline
deposits of calcium phosphate and carbonate may be evident (Wessely,
1922; Kranz, 1927; Boente, 1931), until eventually almost complete
calcification may occur within an intact capsule and the lens becomes
opaque to X-rays. It is to be noted that deposits of calcium carbonate or
phosphate may occur in the clear lenses of old people (Kranz, 1927; Boente,
1931).
The question which immediately arises is the significance of this
increase in calcium. In view of the fact that the increase does not occur in
incipient cataract but becomes progressively marked in the later stages,
since its deposition is irregular and fortuitous rather than based on any
systemic plan and shows a wide variability between different individual
lenses, and since in cataractous subjects, while, as we shall see presently,
3 U 2
3122 TEXT-BOOK OF OPHTHALMOLOGY
the calcium metabolism is within normal limits with a tendency to hypo-
calcaemia rather than hypercalcaemia, it seems probable that the accumulation
is secondary and not primary, incidental to the cataractous process rather than
a causal factor. Moreover, Kirby (1931) has shown that while an increase
of calcium has little toxic effect on the growth of lenticular fibres in vitro,
a diminution of the concentration in the nutrient fluid has a marked effect.
In cataractous lenses the metal is found deposited in a non-diffusible form,
which is probably a reaction secondary to the slow death of the tissues, a
concentration by adsorption to the large molecules of the lipoids and
proteins, and a deposition owing to the final change of the hydrogen ion
concentration to the alkaline side, in which circumstance, corresponding
to a low tension of carbon dioxide, ionized calcium becomes precipitated
in the form of phosphate. In this sense the deposition corresponds to that
in an atheromatous artery, a degenerated cornea, or a tuberculous
focus in the lung ; it is a passive and inert accumulation in a damaged
Structure.
Although, however, almost certainly not the cause of cataract, it does not follow
that the accumulation of calcium may not have some effect on its development. As
will be discussed later, it may have an effect (1) by decreasing the permeability of the
capsule, (2) by sensitizing the proteins to ultra-violet light, and (3) by direct injury to
the lenticular proteins.
POTASSIUM. While the quantity of calcium increases, that of potassium,
decreases markedly in cataract. Burge (1909) found the percentage in the
ash to fall from 38% to 40% in normal lenses to 9.8% in cataractous lenses;
Burdon Cooper (1928) found a similarly large drop ; and Mackay, Stewart
and Robertson (1932) found the percentage in dry lens to change from the
normal 0.635 to 0.309 in immature senile cataract, and 0.092 in mature
cataract, the percentage in the ash being 21-8, 8.45 and 2.4 respectively. The
normal lens is thus extremely rich in potassium, and the potassium/calcium
ratio is high (380 : 1, Burge, 1909), but in senile cataract it is reduced to
2 : 3 or even less. -
In the normal lens only a small quantity of the immense potassium stock
exists in diffusible form (0.6%, Burge, 1909); the remainder is presumably
combined with complex protein ions. The element, being a cell-food and a
substance necessary to life, is retained by the cells. On the death of the cells,
however, it becomes liberated and diffuses away concurrently with other
vital constituents of the lens.
SODIUM. While sodium is present in minute quantities in the normal
lens, it increases in cataract (Burge, 1909; Evans and Kern, 1931; Mackay,
Stewart and Robertson, 1932; Lebensohn, 1936): Mackay and his co-
workers reported an increase in the dried lens from 0.38% in the normal to
0-62. Corresponding to the decrease in potassium, this increase in sodium
is probably a diffusion phenomenon, for the aqueous and plasma normally
DISEASES OF THE LENS 3.123
contain sodium in excess of potassium. In this respect the tendency is
therefore for the lens to take on a constitution similar to that of the plasma.
In a general sense, indeed, the potassium/sodium ratio may be taken as an
index of tissue-vitality, and while in the normal cortex and nucleus it is 1.65
and 1.34 respectively, in cataract it falls to 0.41 and 0.32 (Lebensohn, 1936).
MAGNESIUM. In the normal lens magnesium is present in traces ; in cataract it
is increased from 1.2% to 8.0% in the ash (Burge, 1909; Burdon-Cooper, 1928;
Evans and Kern, 1931). The importance of magnesium lies in the fact that it probably
acts as a co-enzyme in the exchange of phosphate in glycolysis.
In addition, a large number of other metals—lithium, strontium, zinc, lead,
silver, iron, silicon, etc.—are present in traces in the normal lens; in cataract they
appear to be increased, but the meagre information at our disposal of their disposition
and function does not allow the drawing of any conclusions from their presence
(Burdon-Cooper, 1928).
CHLORIDEs. Chlorides form the predominant ion in the lens, and are increased
in cataract ; thus Mackay, Stewart and Robertson (1932) found 0.097% in the dry
human lens, 0.272% in immature cataract, and 0.325% in mature senile cataract.
The increase thus corresponds with that of sodium, and probably represents a diffusion
phenomenon. The function of the chloride ions lies essentially in the maintenance of
the acid-base equilibrium and the distribution of water.
SULPHATEs. There is evidence that the sulphate content of the lens increases in
cataract (Mackay, Stewart and Robertson, 1932), but how much of this change is due
to the sulphur in lenticular protein and how much to inorganic sulphate is unknown.
PHOSPHATEs. The amount of total phosphate in the lens decreases in cataract,
sometimes as much as 50% of its normal value (Adams, 1929; Evans and Kern, 1931 ;
Mackay, Stewart and Robertson, 1932; Updegraff, 1932). Much of the phosphorus
is organically combined, and while Müller (1936) found this moiety decreased in age
and in cataract, he found that the inorganic phosphates, like the calcium, increased
in the latter.
(d) ORGANIC SUBSTANCES
SUGAR. Both the normal and pathological lens contains glucose in the same form
as in the blood (Kronfeld and Bothman, 1928); O’Brien and Salit (1931) found it
slightly increased in age, and Lottrup-Andersen (1927) in diabetic cataract. The
metabolic importance of glucose lies in the source of energy derived from its break-down
to lactic acid, but there is no evidence of significant change in cataract.
UREA. As in all other tissues, urea is present in the normal lens, but is less in
age and cataract (Schmerl, 1932; Grümer-Schmoll, 1934); as a normal product of
protein catabolism, the diminution is presumably due to the lessening of normal
activity.
(e) water AND SOLID CONTENT
The lens has less water than any other tissue, but like all other tissues
its water content decreases with age : from birth to 4 years the water
content of the cow's whole lens decreases from 68 to 64%, and while the
cortex of the adult cow contains 70% of water, the older nuclear fibres have
only 50% (Buglia, 1925; Lebensohn, 1933). In senile sclerosis (and in
cataracta nigra) this tendency for an increase of solids in relation to water
3124 TEXT-BOOK OF OPHTHALMOLOGY
is intensified (Gifford and Puntenny, 1933); but in cortical cataract the
opposite process is seen.
The question of the water content of the normal and cataractous lens
was first investigated by Deutschmann (1879), whose conclusion that the
amount of water in cataract was increased has been confirmed by several
subsequent observers (Kubik, 1930; Cahane, 1931; Lebensohn, 1933;
Salit, 1938). Their results are in substantial agreement : Salit’s average
figures, while showing an unimportant and irregular increase in water
content with age, are—in incipient cataract 139.7 mg. water or 66%, in
intumescent cataract 168.9 mg. or 74.5%, and in mature cataract 133.5 mg.
or 73.2%. On the other hand, there is a marked loss of solids, the correspond-
ing figures being 69-6, 58.0 and 48.4 mg., that is, a loss of 20% and 44%.
These changes entail a considerable alteration in weight, an initial increase
owing to the addition of water and an ultimate decrease owing to loss of
solids; for while the average weight of the lens with incipient cataract is
0-2057 gm., this increases to 0-2269 gm. during intumescence, and falls to
0- 1819 gm. at maturity, and further still on hypermaturity (Clausnitzer,
1911; Adams, 1929; Kubik, 1930; Mackay, Stewart and Robertson, 1932;
Salit, 1933–38; and others). -
Much less work has been done on the more important question
of the physical state of this water. Its quantity is determined by
the balance of osmotic, imbibitional and tension forces, and part of
it is “free * in the sense that it is not in intimate physico-chemical
combination, while part is “bound * in that it is so closely combined
with the colloids of the tissue as not to be capable of acting as water of
solution. In the lens the proportion of bound water is high, a matter of
considerable importance in maintaining its transparency (Fischer, 1930–33).
By examining the relative vapour pressure at different stages of desiccation,
Lebensohn (1933) concluded that the difference in water content with age
affected almost entirely the free water. It would seem that in the develop-
ment of cataract the initial increase in water content is essentially an osmotic
effect owing to the high pressure generated by the fragmentation of large
protein molecules into the smaller osmotically active derivatives of autolysis ;
this water remains free, accumulating beneath the capsule, gathering in
vacuoles and in fissures in the sutures. Such an accumulation impairs the
transparency of the tissue, a tendency increased by the lessened imbibitional
power of the lenticular proteins, so that while the free water increases, the
bound water becomes less. Finally, in the stage of maturity when the
products of proteolysis diffuse away though the capsule, thus reducing the
Osmotic pressure, the amount of free water, and therefore of total retained
water, becomes considerably less. The cataractous process, therefore, is
not so much the result of an abnormal deposition of solids in the lens as an
accumulation of free water (and fat) between shrunken fibres, the whole
simulating to some extent the structure of a sponge.
DISEASES OF THE LENS 3125
JREACTION
The pH of the normal lens is approximately that of blood, that is, about
7.4 (Scalinci, 1929; Sauermann, 1933; Nordmann, 1935; Salit, 1939), but
the nucleus is slightly more alkaline than the cortex. Salit (1939) found a
slight tendency to acidity with age (pH 7.56 decreasing to 7:43 in cattle).
Estimates in cataractous lenses have been few, but the tendency appears
to be towards acidity with the development of cataract and eventually towards
alkalinity as the state of maturity is reached. Thus Labbé and Lavagna
(1925), who found the normal pH to be 7.5 to 7-6 in the senile lens,
obtained values for immature and mature cataract of pH 6-6 and for
hypermature cataract 7-8; and Buglia (1925) also obtained a lower
value in cataract. Sauermann (1933) also found the average pH of
human cataracts to be 6.2 while that of the bovine lens was 7-4. Again,
in naphthalene cataract in rabbits, Reiss and Nordmann (1938) found the
pH to fall to 7-0 in the earlier stages and to rise to 7-8 at maturity. The
importance of the early acid shift lies in its relation to proteolysis, and the
interest of the ultimate alkaline shift in its relation to the deposition of
calcium.
Little accurate work has been done on the ISO-ELECTRIC POINT of lenti-
cular protein, although its interest is considerable as indicating the point of
maximal instability of the proteins. Woods and Burky (1928) and Woods
(1930) concluded by the colorimetric method that the iso-electric point of
o-crystallin was pH 4-8, of 8-crystallin pH 6. By the quinhydrone method,
Tsuji (1932) found the iso-electric point of 8-crystallin to be greater than
pH 5. Of the other proteins nothing yet is known. Taking the lens as a
whole, however, the point of maximal precipitation of protein and minimal
absorption of water provide a considerable amount of information. This
has been variously determined as lying between pH 4 and 5—by de Haan
(1922) as pH 5, by Gulotta (1926) as pH 4, by Scalinci (1929) as pH 3.5 for the
cortex and 4.5 for the nucleus, by Gonçalves (1930) as pH 4-6 for the whole
lens, by O’Brien and Salit (1931) as 5.16 (cortex 5.10, nucleus 5:44), and by
Maestro (1937) as pH 5.
In summary, therefore, it may be said that the essential chemical changes
occurring in cataract involve a hydration followed by a dehydration, an
acidification followed by an alkalosis, a replacement of soluble by insoluble
protein, an increase in calcium and lipoids, and an averaging of the concentra-
tion of the Salts to correspond with that of the blood.
Adams. Bioch. J., xxiii, 902, 1929. Burdon-Cooper. O. Rev., xxxiii, 129, 1914.
Boente. A. f. Aug., cii, 260, 1931. T. O. S., xlviii, 340, 1928; liii, 401, 1933.
Bourne and Campbell. Brit. J. O., xx, 684, Burge. A. of O., xxxviii, 435, 1909.
:* 1936. Bürger and Schlomka. Z. eacp. Med., lviii
Buglia. A. di Ott., xxxii, 193, 1925. 710, 1927.
Bunge. A. f. Aug., cik, 503, 1936. Cahane. C. R. S. Biol., cviii, 992, 1931.
3126
TEXT-BOOK OF OPHTHALMOLOGY
Cahn. Z. f. phy. Chem., v, 213, 1881.
Campbell. T. O. S., liii, 391, 1933.
Clapp. J. Am. Med. As., lvi, 806, 1911.
Am. J. O., vii, 131, 1924.
Clausnitzer. Cb. pr. Aug., xxxv, 262, 1911.
Deutschmann. A. f. O., xxv (2), 213;
(4) 427, 1879.
A. f. d. g. Phys., xx, 420, 1879.
Evans and Kern. Am. J. O., xiv., 1029, 1931.
Fischer. A. f. Aug., ciii, 1, 1930; cviii, 80,
517, 1933.
Gifford, S. R., and Puntenny. Am. J. O.,
xvi, 1050, 1933.
Goldschmidt. Münch. med. W., lxi (1), 657,
1914.
A. f. O., lxxxviii, 405, 1914.
Bioch. Zeit., czzvii, 210, 1922.
Gonçalves. A. phys. Biol., viii, 5, 1930.
Grabar and Nordmann. C. R. S. Biol., czii,
1534, 1933.
v. Graefe. A. f. O., i, 323, 1854.
Grümer-Schmoll. B. O. G. Heidel., 1, 161,
1934.
Gulotta. A. dº Sc. biol., viii, 48, 1926.
de Haan. A. néer. de phys., vii, 245, 1922.
Hoffmann. Münch. med. W., lx (1), 741,
1913.
Jess. A. f. Aug., lxxi, 259, 1912.
Z. f. Biol., lxi, 93, 1913.
Z. f. phy. Chem., cz, 266, 1920; cxii, 160,
1922.
A. f. O., cv, 428, 1921; ciz, 463, 1922.
Eirby. A. of O., v, 754, 856, 868, 1931.
Kranz. A. f. O., cxviii, 571, 1927.
Krause. A. of O., viii, 166, 1932; ix, 617;
x, 631, 788, 1933; xiii, 71, 187, 1935;
xv, 522, 1936; xvii, 468, 1937.
Am. J. O., xvii, 502, 1934; xxi, 1343, 1938.
Eronfeld and Bothman. Z. f. Aug., lxv, 41,
1928.
Kubik. A. f. Aug., cii, 657, 1930.
Labbé and Lavagna. C. R. S. Biol., clxxx,
1186, 1925.
Lang. T. O. S., xv, 114, 1895.
Lebensohn. Am. J. O., xvi, 1062, 1933.
A. of O., xv, 217, 1936.
Lo Cascio. Am. dº Ott., lxv, 801, 1937.
Lottrup-Andersen. Acta O., v, 226, 1927.
Mackay, Stewart and Robertson. Brit. J. O.,
xiv, 193, 1932.
Maestro. Am... di Ott., lxv, 453, 1937.
Magnasco. Saggi di Oft., iv, 358, 1929.
Mettenheimer. Förd. d. wiss. Heilk., No. 24,
1857; No. 31, 1858.
Metzger. K. M. Aug., lxxxvii, 850, 1931.
Mörner. Z. f. phys. Chem..., xviii, 60, 1894.
Müller. A. f. Aug., ciz, 497, 1936.
Neuschüler. Lett. Oft., vii, 237, 1930.
Nordmann. A. d’O., lii, 78, 170, 1935.
O’Brien and Salit. Am. J. O., xiv, 582, 1931;
xvi, 866, 1933.
Parhon and Werner.
989, 1931.
Purtscher. A. f. O., cxxxix, 358, 1938.
Reiss and Nordmann. C. R. S. Biol., czkviii,
111, 1938.
Rinaldi. Am. d: Ott., lxv, 667, 1937.
Rodigina. Sov. vest. O., i, 121, 1932.
Rötth. A. f. O., czzii, 34, 1929.
Salit. Am. J. O., xiii, 1072, 1930; xiv, 523,
1931; xx, 157, 1937; xxi, 763, 1938;
xxii, 413, 1939.
A. of O., v, 354, 623, 1931; ix, 571, 1933;
xvi, 271, 1936; xviii, 403, 1937.
Brit. J. O., xix, 663, 1935.
Salit and O’Brien. A. of O., xiii, 227, 1935.
Sauermann. Am. J. O., xvi, 985, 1933.
Scalinci. Boll. S. biol. sper., iv, 359, 1929.
Schmerl. K. M. Aug., xi, 953, 1932.
Shoji. An. d’Oc., clx, 356, 1923; clziv, 344,
1927.
Smith, Priestley. T. O. S., iii, 79, 1883.
Tsuji. J. Biochem., xv, 33, 1932.
Tweedy. Lancet, ii, 519, 1873.
Updegraff. P. S. Eacp. Biol. Med., xxix, 964,
1932.
Wessely. A. f. Aug., xci, 158, 1922.
Woods. A. of O., iv, 96, 1930.
Woods and Burky. A. of O., lvii, 464, 1928.
Zehender, Matthiessen and Jacobsen. K. M.
Aug., xv, 311, 1877; xvii, 307, 1879.
C. R. S. Biol., cviii,
THE CHEMICAL PATHOLOGY OF THE BLOOD
A considerable amount of enquiry has been made as to whether
chemical changes in the blood have an influence on the development of
cataract, and it may be said at once that, on the whole, these investigations
have had negative results. There is no evidence that a gross derangement of
blood chemistry is aetiologically associated with semile cataract.
The fact that cataract is characterized by an accumulation of calcium in the lens
led to the hypothesis that an aetiological factor might be a hypercalcaemia. Such a
condition has on occasion been reported (Adams, 1929). On the other hand, the
occurrence of cataract after parathyroidectomy or in association with tetany has
suggested the opposite hypothesis, and a subnormal serum calcium has been recorded
DISEASES OF THE LENS 3127
in senile cataractous patients by Levina and Cheifetz (1928). While a calcium
deficiency is undoubtedly associated with the development of cataract in infantile
tetany or after parathyroidectomy, and although a diminution of calcium may induce
electro-chemical changes and a consequent upset of the labile colloidal solution of
the lens protein, it must be remembered that parathyroid dysfunction is a systemic
disease involving other factors than this. Moreover, a hypocalcaemia from other causes
does not give rise to cataract, while the great mass of evidence tends to show that in
the average case of senile cataract the calcium content of the serum is neither notably
in excess nor diminished, and the study of the endogenous calcium metabolism shows
that the patients are in perfect balance (Tron, 1926; Pellathy and Pellathy, 1927;
Poljuchova, 1928; Kirby, 1931 ; Burdon-Cooper, 1933; and others). There seems
therefore to be no evidence that variations in the serum calcium can stand in a primary
aetiological relation to the development of cataract.
Similarly the magnesium content of the serum has been found to be normal
(Givner and Gannon, 1938), as also the chloride content (Cucchia, 1928), and the
sulphur metabolism (Bourne and Campbell, 1936).
A mild hypercholesteraemia has been reported by several observers to exist in
cataractous patients (O’Brien and Meyers, 1928; Filippi-Gabarde, 1931; Salit, 1931;
Cantonnet, 1933), while others have found the cholesterol normal (Galeazzi, 1934).
It is difficult to assess the significance of these observations, but any increase is almost
certainly incidental, associated perhaps with Senile changes, or, as was suggested by
Michail and Vancea (1927) who produced hypercholesteraemia in rabbits by feeding
them with naphthalene, it may be part of a defence reaction of the organism against
a general toxic poisoning, which itself may well be associated with the development
of cataract. -
The frequent occurrence of cataract in diabetics has led to the sugg stion that a
hyperglycaemia may be a causal factor, and several reports have appeared indicating
a raised blood sugar content or a low glucose tolerance in a proportion of non-diabetic
cataractous patients (Langdon, 1922–25; Baldwin and Barthel, 1924; Giannantoni,
1927–28 ; Sipov, 1930; O’Brien, 1931). On the other hand, Mehlhose (1929), Levina
and Cheifetz (1928), Andersen (1929), Kirby and Wiener (1933), and others have found
no significant change. It is to be remembered that a lowering of the efficiency of the
carbohydrate mechanism is a common feature of age, and hence is often present
in association with senile cataract, not in the relation of cause and effect, but as
expressions of the same Senile deterioration.
A number of observers have reported an occasional slight rise of the total nitrogen
in the blood, an increase of the urea, the creatine and creatinine, with a diminution
of the urea in the urine, and a slight diminution of the alkaline reserve in the blood
with an uncompensated alkalosis (Lo Cascio, 1926; Giannantoni, 1928; O'Brien and
Meyers, 1928; Levina and Cheifetz, 1928). These changes, however, are slight and
irregular, and, as would be expected in the type of patient suffering from senile cataract,
are evidences of impaired renal function which are incidental rather than causal.
Adams. Bioch. J., xxiii, 902, 1929. Giannantoni. Am. di Ott., lv., 375, 1927;
Andersen. Acta O., vii, 339, 1929. lvi, 916, 1928.
Baldwin and Barthel. J. Am. Med. As., Givner and Gannon. A. of O., xix, 941, 1938.
lxxxiii, 994, 1924. Rirby. A. of O., v, 868, 1931.
Bourne and Campbell. Brit. J. O., xx, 684, Kirby and Wiener. A. of O., x, 25, 1933.
1936. Langdon. T. Am. O. S., xx, 340, 1922.
Burdon-Cooper. T. O. S., liii, 401, 1933. T. O. S., xlv., 204, 1925.
Cantonnet. A. di Ott., x1, 451, 1933. Levina and Cheifetz. A. oft. Russ., v, 71, 1928.
Cucchia. Lett. Oft., v, 84, 1928. Lo Cascio. An. Fac. di Med. Chir., xxix, 32,
Filippi-Gabarde. VII Congr. S. Ital. d’O., 1926.
1931 : Ref. Zb. ges. O., xxviii, 646, 1933. Atti di congr. d’Oft. Ital., 40, 1926.
Galeazzi. Rass. It. d’Ott., iii, 199, 1934. Mehlhose. K. M. Aug., lxxxiii, 97, 1929.
3128 TEXT-BOOK OF OPHTHALMOLOGY
Michaël and Vancea. C. R. S. Biol., xcvii, Poljuchova. Russ. O. J., vii, 196, 1928.
1569, 1927. Salit. A. of O., v, 354, 1931.
O’Brien. T. Am. Med. As., Sect. O, 132, Sipov. Russ. O. J., xii, 321, 1930.
1931. Tron. A. f. Awg., xcvii, 356, 1926.
O’Brien and Meyers. A. Int. Med., xlii, 376, Vele. Rass. It. d’O., ii, 88, 1933.
1928. "- Villani. An. di Ott., lxv, 306, 1937.
Pellathy and Pellathy. K. M. Aug., lxxix,
198, 1927.
THE NORMAL AND PATHOLOGICAL METABOLISM OF THE LENS
Since the first volume of this text-book was written so considerable an
amount of information has been brought to light regarding the metabolism
of the lens that it is necessary to re-summarize the position. Even yet,
however, our knowledge is most fragmentary, and although some leading
facts are known, the intimate mechanism of the essential processes is only
barely realized, and it is quite impossible to fit whole subjects into the
general scheme. While, therefore, some main indications may be given, it
is impossible to describe adequately the biochemical foundations of cataract.
Until comparatively recently it was considered that the metabolism
of the lens was minimal ; but such is not the case. Its general dependence
on metabolic activity for the maintenance of its transparency has already
been indicated,” when it was pointed out that if the normal lens was kept in
saline without any supply of food-stuffs or arrangements for the elimination
of waste products, it slowly turns opaque, a change which also follows an upset
of intake and output such as occurs after ligating the vortex veins or the
posterior ciliary arteries, or in a state of congestive glaucoma, or by rendering
the capsule impermeable either experimentally or by the deposition upon it
of exudative inflammatory material. That energy-producing reactions
such as Oxygen-consumption and glycolysis are essential for the continuance
of the integrity of cells which do no external work is not unique, for the mere
maintenance of the cell in the living state demands the continual performance
of work, which, although invisible, is nevertheless indispensable.
The most important process appears to be the carbohydrate metabolism,
for the greater part of the available energy is derived from the break-down
of glucose. That the lens uses up glucose in considerable quantity was
proved by Kronfeld (1927), Kronfeld and Bothman (1928) and Cohen and
Killian (1929); Kronfeld (1933), indeed, showed that the whole rabbit lens
consumed 0.3 mg. oxygen and changed 2.4 mg. glucose to lactic acid each
day. This glycolytic activity was demonstrated indirectly by Wittgenstein
and Gaedertz (1926), who showed that the lactic acid content of the aqueous
is higher than that of the blood, by Fischer (1931), who showed that the
normal concentration of lactic acid in the aqueous humour could fall from
28 mg. "% to 14.5 in the aphakic eye, and by Kronfeld (1933), who found
a low aqueous glucose in congestive glaucoma when the lens was present,
* Vol. I, p. 488 (497).
DISEASES OF THE LENS 3129
and a normal concentration in aphakia. It would appear that the greatest
activity occurs in the sub-capsular epithelium (Michail and Vancea, 1932),
while the nucleus shows the least.
There are, of course, many other substances which participate in the metabolism—
uric acid, allantoin, citric acid, and so on—but about their activities little or nothing
is known.
The carbohydrate metabolism appears to occur in two stages. The first
is an anaerobic process of glycolysis effected by enzymes whereby glucose is
broken down to lactic acid; the second an aerobic process of inner respiration
whereby lactic acid is degraded to carbon dioxide and water.
The first process of glycolysis is an energy-liberating process requiring
no oxygen, and it is not surprising, therefore, that it appears to be more
active in the nucleus than the cortex (Michail and Vancea, 1932). The
reaction, taking place in the absence of oxygen, is activated by enzymes, two
of the most important of which are lacto-flavin (vitamin B2) and aneurin
(vitamin B1).
The process evidently occurs in cyclic equilibria by the removal of phosphate
from creatine phosphate to form adenosine triphosphate, and its replacement by the
breaking down of hexose phosphate, which is thus split up and rearranged molecularly
to form lactic acid (Krause, 1937–38). In the lens, particularly the cortex, there is a
considerable amount of creatine (Krause, 1939); and, as we shall see presently,
lacto-flavin is active in anoxybiotic fermentation involving phosphoric acid.
Catabolism appears to take place through the stages—fructose—pyruvic acid—lactic
acid, the last transposition being made effective through the medium of vitamin B1.
In the absence of this substance pyruvic acid cannot be converted into lactic acid,
and hence there is an accumulation of the former in cataractous lenses.
A further and smaller part of the energy of the lens is derived from the
oxidation of part of this lactic acid into the ultimate products carbon dioxide
and water. In contrast to the first mechanism this process requires oxygen
in a more activated form than in solution, and for this purpose apparently
the lens is provided with catalases and oxidases (Lo Cascio, 1922; Alajmo,
1924; Ahlgren, 1927) and with complex autoxidation systems.
That the lens has a definite and measurable oacygen consumption has been
amply shown 1 both by the actual measurement of the oxygen up-take and
by its ability to decolourize methylene blue (Ahlgren, 1923–27; Mashimo,
1923; Goldschmidt, 1924; Adams, 1925; Alajmo, 1924; Schmerl, 1927–29;
Fronfeld and Bothman, 1928; Friedenwald and Pierce, 1937; Field,
Tainter, Martin and Belding, 1937). The oxidative activity can also be
measured by estimating the electrical potential by platinum electrodes, an
increase of the potential denoting a diminution of the reduction power
(Nordmann and Reiss, 1933; Müller, 1933). The whole lens of the rabbit
consumes something like 4 cm. of oxygen per hour. It is of incidental
importance that the mechanism whereby it obtains this Oxygen is not under-
* Vol. I, p. 489 (498).
3130 TEXT-BOOK OF OPHTHALMOLOGY
stood, for it uses some ten times the amount dissolved in the aqueous, a
circumstance which stimulated the suggestion by Friedenwald (1933) that
the ciliary epithelium perhaps secreted it. However this may be, the
oxidative activity of the cortex is greater than that of the nucleus, and that
of the whole lens is increased in anaerobic conditions or on exposure to light
(Meesmann, 1930), while it is decreased on exposure to infra-red or ultra-
violet light or in age, and abolished by dialysation or in cataract. It is
interesting that Meesmann (1930) and Fischer (1931), by comparing the
oxygen and carbon dioxide in the carotid artery and the vortex veins, found
that the respiratory quotient was unity, and therefore concluded that only
carbohydrate was oxidized.
In so far as it is not supplied with blood vessels but forms an isolated
system suspended in the aqueous humour, the lens cannot obtain and utilize
oxygen as other vascularized tissues. It therefore avails itself of intra-
cellular autoacidation systems which depend on substances which act as
hydrogen acceptors"; these form a supplementary Oxidative mechanism in
most tissues, but in the lens they assume the primary rôle. There is
evidence that more than one such mechanism is active —
1. Proteins, the reducing properties of which are due to the sulphydril
group in cysteine.
2. Glutathione, also a sulphydril compound, which forms a reversible
oxidation-reduction system with the proteins.
3. Vitamin A which appears to act as an oxygen catalyser.
4. Vitamin B1 (aneurin), which is probably necessary for the reduction
of pyruvic acid liberated in the catabolism of carbohydrates.
5. Vitamin B, (lacto-flavin), acting in the anaerobic glycolysis by forming
a reversible oxidation-reduction system.
6. Vitamin C (cevitamic acid : ascorbic acid), forming an irreversible
oxidation-reduction system, probably in association with glutathione, thus
freeing energy for the metabolic needs of the lens.
Among these, three important Oxidation systems are now known :
(1) The sulphydrils, (2) ascorbic acid, and (3) lacto-flavin.
Sulphydrils : Cysteine and Glutathiome. The discovery by Arnold
(1910) of the development of a strong purple colouration in the lens with
sodium nitro-prusside formed the first proof of the presence of sulphydril
compounds in the lens, and was attributed by him to the presence of cysteine,
a view proved to be chemically accurate at a later date by Jess (1922). The
interest in this substance and its relation to the metabolic activity of the
lens was heightened by the observation of Reis (1912), which was confirmed
by Jess (1912–13), that the reaction was absent in the cataractous lens. This
led Goldschmidt (1917) to enquire as to the possibility of the presence of an
oxidation-reduction system in the lens depending on the balanced SS-SH
system,” an enquiry stimulated by the discovery by Hopkins (1921) of the
Vol. I, p. 479 (489). * Vol. I, p. 479 (489).
DISEASES OF THE LENS 3131
frequent occurrence in the body of a diffusible compound of cysteine with
glutamic acid (glutathione) which took an active part in such a system.
Soon thereafter it was determined that while some of the sulphydril complex
in the lens was bound up with protein, a considerable proportion was
diffusible (Abderhalden and Wertheimer, 1922), and that this labile moiety
reacted reversibly in an oxidation-reduction interchange with an insoluble
thermo-stable residue (Goldschmidt, 1924), which was identified as being
8-crystallin (Adams, 1925). Although inferred to be glutathione since it
gave the nitro-prusside reaction, which, however, merely depends on the
reducing properties of the sulphydril complex, the fact that the diffusible
constituent was indeed this substance was only proved at a considerably
later date by Bellows and Rosner (1936) who isolated it in the pure form.
It would appear that this substance maintains a continuous and economical
process of autoxidation whereby, starting as the reduced form (SH), it is
oxidized (or dehydrogenated) by free oxygen to the oxidized form (SS)
giving up its hydrogen to form water, to be again immediately reduced by
8-crystallin ; this latter substance, which has all the properties of a thermo-
stable residue, can be alternately oxidized and reduced, indefinitely retaining
its power to react with glutathione.
Several significant facts have been brought to light with regard to these
sulphydril compounds.
1. Glutathione occurs in the lens in very high concentration, varying
considerably among species: Cordero (1933) found it present in a concen-
tration varying from 71.5 mg. per 100 c.c. lens tissue in the horse to 261.96mg.
in the rabbit, that of man being 74.38 mg. In the blood, in which it is
wholly contained in the corpuscles, it is in much lower concentration, average
figures for man being 30 mg. of the reduced form and 3 to 11 mg. of the
oxidized form per 100 c.c. (Woodward and Fry, 1932). It is not present in
the normal aqueous. It would appear that at birth the glutathione content
of the lens approximates that of the blood, an understandable occurrence
since the embryonic lens derives its nourishment from the blood, but that
immediately after birth its concentration rapidly increases since a substitute
mechansim is necessitated (Rosner, Farmer and Bellows, 1938).
2. The glutathione content decreases with age and gradually disappears
with the development of cataract, while its activity ceases in conditions of
acidity (Reis, 1912; Goldschmidt, 1917; Jess, 1921; Shoji, 1927; Cohen,
Rillian and Kamner, 1928 ; Tassman and Karr, 1929; Weinstein, 1931;
Gifford, 1932; Cordero, 1933 ; Rosner, Farmer and Bellows, 1938 ; and
others). It would appear, as has been demonstrated by Tsuji (1932) in
experimentally produced cataract, that the freely diffusible cysteine
(glutathione) is first changed to its oxidized form and is then lost, presumably
by diffusion, while the bound cysteine (8-crystallin) is decreased in quantity.
3. By adding glutathione to the lens system, the oxygen consumption
is increased, and on removing it by dialysation the consumption of oxygen
3132 TEXT-BOOK OF OPHTHALMOLOGY
by the lens ceases; in this event the addition of a minute amount of gluta-
thione restores the oxygen up-take to normal (Adams, 1925). With the
loss of glutathione, therefore, the vital power of oxidation fails.
The question obviously arises whether the disappearance of glutathione
is secondary to the cataractous process in the lens, or precedes it and conse-
quently may be responsible for it. There is little evidence that the loss of
glutathione is precedent, and therefore potentially causal, except that pro-
duced by Rosner, Farmer and Bellows (1938), who found that after feeding
with galactose, the appearance of cataract followed the loss of glutathione, and
that, on the return of glutathione to the lenses of rats placed on a normal
diet after a period of galactose diet, the lens recovered from incipient cataract.
On the other hand, it was established by Campbell (1936) that there was
no significant variation in the glutathione content of the blood with age or
diet or in cataractous patients, and by Bourne and Campbell (1936) that the
entire sulphur metabolism in such patients was normal. It would appear,
therefore, in the light of our present knowledge, that, although the absence
of this active factor in the oxidative activity of the lens in cataract is estab-
lished, its rôle as a determinant factor must remain Sub judice.
Vitamin C (Cevitamic or Ascorbic Acid). Cevitamic acid, a diffusible
crystalloid, is a strong reducing agent giving off hydrogen easily by convert-
ing itself into an oxidized form, and is probably of fundamental importance
in tissue-respiration by acting as a hydrogen transport agent : it can exist
in three forms, a reduced form, a reversibly oxidized form and an irreversibly
oxidized form. Its presence in the lens and aqueous was first established
by H. K. Müller (1932), and has subsequently been amply confirmed, in which
tissue indeed it assumes proportions higher than in any other organ of the
body, varying in different species from 2 mg. per 100 gm. of tissue in the rat
to 104 mg. in the fish. The value in man is about 30 mg. or from 12 to 20
times the amount in blood serum.
Müller's findings have been substantiated by a number of writers : Müller
(1933–37), Harris (1933), Birch and Dann (1933–34), v. Euler and Martins (1933),
v. Euler and Malmberg (1934), Müller and Buschke (1934), Fischer (1934), Müller,
Buschke, Gurewitsch and Brühl (1934), Bietti (1935), Weinstein (1935), Bellows (1936),
and many others. The estimations have mainly been done by titration with 2, 6,
dichloro-phenol-indophenol, which is reduced by cevitamic acid. Conflicting reports
have appeared as to whether this method is specific for cevitamic acid so that its results
might be affected by other reducing substances, a fear which was stressed by Birch and
Dann (1934), v. Euler and Malmberg (1935), Demole and Müller (1935), and Bietti
(1935), who found that lens extracts did not provide the calculated amount of protection
biologically in vitamin C deficiency. It might be that other toxic substances in the
lens could interfere with such a quantitative correlation, but spectrographic methods
of estimation also give lower results than the titration method (Johnson, 1936). On
the other hand, Bellows and Rosner (1936) found that after contact with the enzyme,
cevitamic acid oxidase, any reduction of the dye ceases, a fact which suggests, since
the enzyme is specific, that the decolourization is due to vitamin C alone. Whatever
the accuracy of these quantitative methods, however, the presence of cevitamic acid
DISEASES OF THE LENS 3.133
is undoubted, and of the total reduction in the lens, as measured by the iodometric
method, it would seem that some 30% is due to the activity of vitamin C.
From these investigations it has been determined that -
1. Cevitamic acid is present in a relatively high proportion in the lens
and in the aqueous humour, and in the fully developed lens it is more
abundant in the cortex than in the inert nucleus (Gurewitsch, 1934; Glick
and Biskind, 1936).
2. Its concentration in the lens falls considerably with age (Müller,
1933–34 ; Gurewitsch, 1934; and others), and very considerably in cataract,
in which condition it may disappear entirely (Müller, 1933–37; Nordmann
and van Wien, 1934; Nakamura, 1935; Bietti, 1935; Bellows, 1936; and
many others).
3. Its presence in the aqueous, which is normally in much higher
concentration than in the blood or the cerebro-spinal fluid, is increased by
augmenting the intake of vitamin C in the diet (v. Euler and Malmberg,
1935; Bietti, 1935), and decreased in cataract, in aphakia, or in the plasmoid
aqueous obtained after paracentesis (Müller, 1933–34 ; Müller, Buschke,
Gurewitsch and Brühl, 1934; Bietti, 1935 ; Monyukova and Fradkin,
1934–35; and others), as well as in animals fed on a diet deficient in
vitamin C (Bietti and Cartini, 1934; v. Euler and Malmberg, 1935). In the
aqueous of aphakic or cataractous eyes the small quantity of cevitamic acid
is present, not in the reduced form as occurs normally, but only in the
reversibly oxidized form (Goldmann and Buschke, 1935; Bellows, 1936).
The occurrence of such very large quantities of cevitamic acid in the
normal aqueous naturally excites speculation, for its presence in a higher
concentration than in the blood or the cerebro-spinal fluid rules out a simple
filtration process. A secretion or selective filtration from the blood could be
postulated, but its absence in aphakia and cataract could not be thus
explained and seems to indicate a dependence on the presence of an actively
functioning lens. An alternative explanation was advanced by Müller and
Buschke (1934) and Fischer (1934) that cevitamic acid is manufactured in
the lens from dextrose whence it diffuses through the capsule into the
aqueous, while Müller (1936) brought forward evidence to show that
phospho-glyceric acid might be a precursor and that in such a reaction
glutathione was implicated according to the equation
dextrose + oxidized glutathione —- cevitamic acid + reduced glutathione.
On the other hand, Bietti (1935) found that the concentration of the oxidized
form of cevitamic acid is in all circumstances equal to that in the blood,
suggesting that it dialysed into the eye, where, in the presence of the lens, it
became converted by dehydrogenation into the reduced form, thus playing a
part in the oxidation-reduction system of the lens, and incidentally accumu-
lating in the aqueous. In this view the lens has a preservative action on the
3134 TEXT-BOOK OF OPHTHALMOLOGY
vitamin in the aqueous, an effect which is lost on the removal or the death
of this tissue (Müller, 1935; Bellows, 1936).
The absence of cevitamic acid in cataract and aphakia raises the
question of the relation of this substance to the formation of cataract.
Either the loss of vitamin C is secondary to changes in the lens, probably
of the nature of a disturbance of glycolysis, or its diminution or deficiency
precedes and may determine the formation of cataract. A number of
authorities incline towards the first view (Nordmann and van Wien, 1934;
Fischer, 1934; Bietti, 1935). Others again support the second view, which,
of course, opens up the question of dietary therapeutic possibilities
(Weinstein, 1935; Bellows, 1936; and others). It must be remembered
that scurvy, even in its severe forms, either clinically or experimentally, is
not usually complicated by cataract, and in animals a scorbutic diet may be
pushed to extremes, so that the lens and the aqueous are practically depleted,
and yet the lens remains transparent (Bietti and Cartini, 1934). Monyukova
and Fradkin (1934–35), it is true, obtained occasional lens changes in young
guinea-pigs when their mothers were fed on a scorbutogenous diet, and
consistent changes when a paracentesis was performed so that the supplies
of vitamin C disappeared from the aqueous : they therefore concluded that
the cataract depended on the loss of the vitamin, and suggested that the
clinical occurrence of senile cataract depended upon a disturbance of the
aqueous-blood barrier in a general background of vitamin insufficiency, a
view concurred in by others (Müller, 1937; and others). It is possible that
a state of vitamin C sub-nutrition is common in cases of senile cataract,
a suggestion supported by estimations of the vitamin C content of the blood
in cataractous patients by Bellows (1936), but denied by Hawley and
Pearson (1938). The claim, which will be considered later,” that the
administration of vitamin C delays the onset of the cataract induced by
feeding with naphthalene tends to substantiate this view, but in the light of
our present knowledge the question as to whether the diminution of
vitamin C precedes the formation of cataract or is the result of it cannot be
satisfactorily answered.
Vitamin Be (or G) (lacto-flavin). It seems probable that vitamin Ba
(lacto-flavin), the heat-stable vitamin of the B complex which is essential to
growth and nutrition at all ages, is present in the normal lens. It was
shown by Thiel (1926) that the lens fluoresces, a property which is gradually
lost as cataract develops until eventually it disappears entirely. Koch and
Fischer (1933) demonstrated that this property was not affected by changes
in the water content or by the dissolution of the lenticular proteins, and
Fischer (1934) finally associated it with the presence of lacto-flavin, one of
the factors comprising vitamin B2. It should be noted that both Euler and
Adler (1934) and György (1935) failed to confirm his results. It has been
shown, however, that when a diet deficient in this vitamin is administered
1 p. 3151.
DISEASES OF THE LENS 3135
to young rats, cataract of varying morphological types, cortical, nuclear,
or diffuse, develops and passes throughout all stages of development of
incipiency, intumescence and maturity if the diet is persisted in ; if, however,
yeast or lacto-flavin is added to the diet, cataract does not develop or, if it
has already started, its progress is stopped (Day, Langston and O’Brien,
1931; O’Brien, 1932; Langston, Day and Cosgrove, 1933; Yudkin,
1933–38; Day, Darby and Langston, 1937). It would appear that the
development of the cataract in these circumstances is a local process, since
it occurs while the animals are yet healthy and their general nutrition is
good.
It will be seen at a later stage 1 that other workers have not obtained
uniformity in their results, but a case can be made out that lacto-flavin
may act as a cataract-preventive factor. Its interest in the metabolic
activity of the lens may lie in the fact that, as we have seen, it acts with
phosphoric acid as a dehydrogenase in anaerobic glycolysis by maintaining
a process of anoxybiotic fermentation. Molecular oxygen takes two
hydrogen atoms from it which are converted into H2O, and thence into
H2O + O, and in this transfer the place of oxygen can be taken by any
other hydrogen-acceptor. It is interesting, also, that lacto-flavin is highly
photo-sensitive and is readily broken down by the action of light to inactive
products, such as lumichrome and lumi-flavin, both of which are found in
cataractous lenses (Fischer, 1934). It is also of importance that this
disintegration is prevented by Oxygen, so that an ideal economy is maintained
by which respiration, by protecting against photolysis, preserves its own
functioning.
Metabolically, therefore, the most important features in the develop-
ment of cataract are a diminished metabolism, a decrease in permeability, and
a loss of the substances active in oacidation—cysteine, glutathione, and ascorbic
acid : whether the relation is causal or consequential, a cataractous lens is an
asphyxiated lens.
Abderhalden and Wertheimer. A. ges. Phys., Bourne and Campbell. Brit. J. O., xx, 684,
cxcvii, 131, 1922. 1936.
Adams. P. R. S. (B), xcviii, 244, 1925. Campbell. Brit. J. O., xx, 33, 1936.
Brit. J. O., ix, 281, 1925 ; xiv, 49, 1930.
Ahlgren. Skand. A. f. Phys., xliv, 196, 1923;
xlvii, Supp. 1925.
Acta O., v., 1, 1927.
Alajmo. A. d. Ott., xxxi, 127, 1924.
Arnold. Z. phys. Chem., lxx, 300, 1910.
Bellows. A. of O., xv, 78; xvi, 58, 762, 1936.
Bellows and Rosner. A. of O., xvi, 248, 1001,
1936.
Bietti. Boll. d’Oc., xiv. 3, 1935.
Bietti and Carteni. Boll. S. It. Biol. Sper.,
ix, 283, 983, 1066, 1245, 1934.
Birch and Dann. Nature, czzxi, 469, 1933.
Biochem. J., xxviii, 638, 1934.
Cohen and Killian. XIII Intermat. Cong.,
Holland, i, 166, 1929.
Cohen, Killian and Kamner.
Biol., xxv, 677, 1928.
Cordero. Rass. It. d’O., ii. 69, 1933.
Day, Darby and Langston. J. Nutrition,
xiii, 389, 1937.
Day, Langston and O’Brien.
1005, 1931.
Demole and Müller.
1935.
v. Euler and Adler.
coxxiii, 105, 1934.
P. S. Earp.
A m. J. O., xiv,
Biochem. Z., colxxxi, 80,
Z. f. physiol. Chem.,
1 p. 3161.
T. () — WOL. I I I.
3136
TEXT-BOOK OF OPHTHALMOLOGY
v. Euler and Malmberg. Z. physiol. Chem.,
ccxxx, 224, 1934.
A. f. Aug., ciz, 225, 1935.
v. Euler and Martins. Z. physiol. Chem.,
coxxii, 65, 1933.
Field, Tainter, Martin and Belding.
J. O., xx, 779, 1937.
Fischer. Erg. d. Physiol., xxxi, 540, 1931.
A. f. Aug., cvii, 295, 1933; cviii, 527, 544,
1934.
Klin. W., xiii, 596, 1934.
Friedenwald. T. Am. O. S., xxxi, 143, 1933.
Friedenwald and Pierce. A. of O., xvii, 477,
1937.
Gifford, H. A. of O., vii, 763, 1932.
Glick and Biskind. A. of O., xvi, 990, 1936.
Goldmann and Buschke. Kl. W., i, 239 ;
ii, 1326, 1935.
A. f. Aug., ciz, 205, 314, 1935.
Goldschmidt. A. f. O., lxxxviii, 405, 1914;
xciii, 447, 1917; cxiii, 160, 1924.
Gurewitsch. A. f. Aug., cviii, 572, 1934.
György. Biochem. J., xxix, 741, 1935.
Harris. Nature, czz.xii, 27, 1933.
Hawley and Pearson. A. of O., xix, 959, 1938.
Hopkins. Bioch. J., xv, 286, 1921; xix, 787,
1925.
Jess. A. f. Aug., lxxi, 259, 1912; lxxii, 156,
1912.
A m.
tº
dº
Z. f. Biol., lxi, 93, 1913.
A. f. O., cv, 428, 1921 ; ciz, 463, 1922.
Johnson. Biochem. J., xxx, 1430, 1936.
Koch and Fischer. A. f. Aug., cvii, 440, 1933.
Krause. A. of O., xvii, 468, 1937; xxi, 1027,
I939.
Am. J. O., xxi, 1343, 1938.
Kronfeld. B. O. G. Heidel., xlvi, 230, 1927.
Am. J. O., xvi, 881, 1933.
Kronfeld and Bothman. Z. f. Awg., lxv, 41,
1928.
Langston, Day and Cosgrove. A. of O., X,
508, 1933.
Lo Cascio. A m. di Ott., 1, 219, 1922; li, 653,
1923.
Mashimo. Kl. W., ii, 1809, 1923.
Meesmann. B. O. G. Heidel., xlviii, 99, 1930.
Michail and Vancea. C. R. S. Biol., ciz, 1011,
1932.
Monyukova and Fradkin.
97, 1934 ; vi, 29, 1935.
A. f. O., cxxxiii, 328, 1935.
Müller, H. K. B. O. G. Heidel., xlix, 168,
1932.
A. f. Aug., cviii, 41, 51, 64, 1933; cik, 304,
434, 497, 1935–36; cz, 321, 1937.
Nature, crxxii, 280, 1933.
Müller and Buschke. Schw. med. W., xv, 585,
I934.
A. f. Aug., cviii, 368, 597, 1934.
Müller, Buschke, Gurewitsch and Brühl.
Kl. W., xiii, 20, 1934.
Nakamura. A. f. O., czkxiv, 197, 1935.
Nordmann and Reiss. C. R. S. Biol., cKiv, 12,
1933; czvi, 223, 1934.
Nordmann and van Wien.
Paris, xlvi, 136, 1934.
O’Brien. A. of O., viii, 880, 1932.
Reis. A. f. Aug., lxxii, 156, 1912.
A. f. O., lxxx, 588, 1912.
Sov. vestm. O., v,
Bull. S. d’O.
Rosner, Farmer and Bellows. A. of O., xx,
4.17, 1938.
Schmerl. A. f. O., czix, 117, 1927 ; cxxii,
488, 1929.
Kl. W., xi, 952, 1932.
Shoji. Am. d’Oc., clz, 356, 1923; cliv, 344,
1927.
Tassman and Karr. A. of O., ii, 431, 1929.
Thiel. Z. f. Aug., lviii, 86, 1926.
Tsuji. J. Biochem..., xv, 33, 1932. -
Weinstein. K. M. Aug., lxxxvii, 393, 1931.
A. f. Aug., ciz, 221, 1935.
Wittgenstein and Gaedertz.
clxxvi, 1, 1926.
Woodward and Fry. J. Biol. Chem..., xcvii.
465, 1932.
Yudkin. J. A. m. Med. As., ci, 921, 1933.
A. of O., xix, 366, 1938.
Am. J.O., xxi, 871, 1938.
Biochem. Z.,
THE PERMEABILITY OF THE CAPSULE
Since, owing to its avascularity, the entire metabolic activity of the
lens depends on biological interchange through the capsule, abnormalities
in the permeability of this membrane have from time to time been considered
as being aetiologically concerned with the formation of cataract. On the
one hand, Löwenstein (1925–34), and Löwenstein and Haurowitz (1929),
basing their conclusions on the fact that a rupture of the capsule led to the
development of traumatic cataract, postulated that lenticular opacities
were due to an increased permeability of the capsule in old age, a view
supported by some authors through their observations on cataract produced
by radiation and toxic substances such as naphthalene, the supposition
being that essential substances such as glutathione diffused away (Hess,
DISEASES OF THE LENS 3 137
1911 ; Schmerl and Thiel, 1929; Scotti, 1930–31 ; and others). On the
other hand, by actual experimental measurements, Friedenwald (1930)
found that the permeability of the capsule tended to decrease with age and in
early cataract ; he therefore sponsored the opposite hypothesis and produced
experimental evidence to show that if the permeability were slightly
decreased by the precipitation on the capsule of inert non-toxic substances
such as eosin and gentian violet, a cataract readily developed, presumably
by the prevention of the diffusion inwards of nutrient material and outwards
of waste products. He also found that contact with cataractous cortex
increased the permeability, a fact which he used to explain the diffusion of
products from a hypermature cataract.
The experimental evidence produced by several workers (Hess, 1911;
Friedenwald, 1930 ; Gifford, Lebensohn and Puntenny, 1932) makes it
certain that the lens capsule acts in vivo and in vitro as an inert, non-selective
semi-permeable membrane, freely permeable to water, electrolytes and
colloids with small molecules, but impermeable to large complex molecules:
it is also permeable in vivo to alcohol from the blood (Nicloux and Redslob,
1931) or iodide from the conjunctival sac (Daniels, 1931). The degree of
permeability seems to be slightly decreased with age, a fact which corresponds
with its anatomical increase in thickness (Bürger and Schlomka, 1927). It
may be that this decrease in permeability may be an adjuvant factor in the
development of senile sclerosis and cataract by decreasing the metabolic
exchange and lowering the vitality of the cells, a process comparable with the
development of an opacity when the capsule becomes plastered with cyclitic
exudates ; but although this decrease of permeability may be influential,
the available evidence seems to indicate that no constant and significant
change such as could exercise any primary and determinant effect can be
claimed in cataract, either of the senile type or that produced by toxic
substances such as naphthalene (Gifford, Lebensohn and Puntenny, 1932),
dinitrophenol (Borley and Tainter, 1937), or galactose (Bellows and Rosner,
1938). Destructive changes can be initiated in the interior of the lens by
such agents as infra-red rays, osmotic changes or toxins without observable
changes in the capsule, which indeed appears to be one of the most resistant
parts of the lens.
It is evident that the permeability of the isolated capsule is different
from that of the capsule lined in situ by the epithelial cells : this is indicated
by the relative amounts of calcium, potassium and sodium in the normal
living lens, and by the selective permeability of the whole lens (to potassium
and not to sodium or lithium in the frog, Manca and Ovio, 1904). One
peculiar thing is the relative impermeability in normal circumstances to
water, for we have no knowledge why water does not penetrate while
complex ions find their way into the lens from the aqueous in a few seconds.
We know that in life the lens system is permeable to highly dispersed
electro-positively charged ions, carbohydrates, alcohols, iodine, potassium
3 X 2
3138 TEXT-BOOK OF OPHTHALMOLOGY
ions, and oxygen, and only slightly so to water ; we know also that in the
healthy state hydrogen ions, carbon dioxide, lactic, pyruvic and ascorbic
acids diffuse from the lens to the aqueous. If the capsule is damaged
potassium ions, lacto-flavin and glutathione diffuse to the aqueous, while
sodium ions diffuse into the lens ; the mineral content of the normal lens,
which, with its excess of potassium, magnesium, phosphates and sulphates
and its paucity of sodium, calcium and chloride, is quite different from that
of the surrounding fluids and the blood, becomes more like these in the
damaged condition so that the composition of the two approximate. We
do not know what proportion of these substances is normally present in
the ionized state and how much is bound to proteins in an undissociated
state ; but we do know that the maintenance of the integrity of the
capsular system and of the oxidative activity of the lens is essential for
the preservation of the normal relations, and in the cataractous lens wherein
no oxidative energy is available these relations are lost. It is evident that
this complex selective permeability depends on the expenditure of energy
which is available only in the living respiring lens. -
Bellows and Rosner. A. of O., xx, 80, 1938. Löwenstein and Haurowitz. A. f. O., c2(xii,
Borley and Tainter. A. of O., xviii, 908, 1937. 654, 1929.
Bürger and Schlomka. Z. Earp. Med., lviii, Manca and Ovio. Osmotischer Druck u.
710, 1927. Ionenlehre, Wiesbaden, 1904.
Daniels. Z. f. Aug., lxxv, 129, 1931. Nicloux and Redslob. C. R. S. Biol., cvii,
Friedenwald. A. of O., iii, 182; iv, 350, 1930. 997, 1931.
Gifford, Lebensohn and Puntenny. A. of O., Schmerl and Thiel. A. f. O., czkii, 482, 1929.
viii, 414, 1932. Scotti. An. di Ott., Iviii, 963, 1930 ; lix, 19,
Hess. G.-S. Hb. III, ix, 1911. 1931.
Löwenstein. K. M. Aug., lxxiv, 786, 1925.
A. f. O., cxvi., 438, 1926; cz.xxii, 224, 1934.
ExPERIMENTAL CATARACT
The highly speculative status of the aetiology of cataract lends special
interest to the experimental production of cataract. This may be accom-
plished in several ways, but unfortunately none of these has yet offered a
definite clue to the mechanism involved. Since, however, the problem will
eventually be solved, and almost certainly through the experimental
approach, these studies will certainly be of more than academic interest.
The various experimental methods of producing cataract may be summarized
thus :—
1. Mechanical injury.
Concussion, contusion, massage, traumatic.
2. Physico-chemical causes.
(a) Osmotic influences.
(b) Cold and heat.
(c) Acidity.
3. Radiational cataract.
Electric, thermal, ultra-violet, radium and roentgen rays.
DISEASES OF THE LENS 3139
. Decrease of the permeability of the capsule.
. Interference with nutrient supplies.
. Conditions of anoxaemia, as experimental asphyxia.
. Deficiency cataracts.
Lack of specific proteins and vitamins : tryptophane, cystein, vitamin Bg.
8. Toxic cataract. -
Naphthalene, lactose and galactose, thallium.
9. Parathyroidectomy and tetany.
:
1. CATARACT DUE TO MECHANICAL INJURY, whether involving con-
cussion, contusion, or induced by massage or rupture of the lens capsule,
will be dealt with under the section of injury to the eyes. Various aetio-
logical factors are operative. In concussion injuries the proteins are coagu-
lated by the energy imparted through mechanical shock and agitation :
these aggregates may be dispersed again with the return of translucency, or
necrosis of the cells may follow. With a rupture of the capsule different
conditions arise owing to entrance of the aqueous, the loss of essential
diffusible substances, and rapid proteolysis.
2. CATARACT DUE TO PHYSICO-CHEMICAL CAUSEs
(a) OSMOTIC CATARACT
It has long been known that an osmotic hydration or dehydration of
the lens could bring about an opacity, which in its lesser degrees, so long as
the physical state only of the water in the lens is involved, is reversible ;
if, however, coagulation of the proteins occurs, the opacity is irreversible.
Thus if the isolated lens is kept in physiological saline at body temperature,
its transparency is maintained for a long time, but in anisotonic solutions
opacification appears especially in the superficial regions of the cortex,
while the lens gains or loses in weight according as to whether it is immersed
in hypo- or hypertonic solutions (Kunde, 1857; Deutschmann, 1877 ;
Heubel, 1879; Bergami, 1927). In the extreme case of immersion in
distilled water the swelling may be so great that the nucleus may take up
84% of its own weight of water (Jaeger, 1861). Similarly in vivo, temporary
lens opacities can be induced in animals by the injection of concentrated
solutions by rectum, intra-peritoneally, sub-conjunctivally, or into the
eye itself (Kunde, 1857; Köhnhorn, 1858; Richardson, 1860 ; Manca and
Ovio, 1897–98; Salffner, 1904; Panico, 1929; Collevati, 1930). As would
be expected, an injection of concentrated saline into the lens itself has the
same effect (in rabbits, Selenkowsky, 1925).
For this the frog is the best osmometer. Thus if 0-2 gm. NaCl is injected sub-
cutaneously, the lens becomes opaque, and if the animal is then immersed in water
the lens regains its transparency (Kunde, 1857). Again, frogs placed in an atmosphere
of calcium chloride develop cataract in one day (Köhnhorn, 1858). With mammalia
the production of cataract is more difficult, but it has been accomplished in cats and
rabbits by hypertonic injections (Richardson, 1860; Heubel, 1879).
3140 TEXT-BOOK OF OPHTHALMOLOGY
That these processes are purely physical and not chemical is shown by
the fact that practically any crystalloid in comparably effective concentra-
tion produces the same effect. The changes, moreover, depend not directly
on the molecular concentration of the blood but of the aqueous immediately
in contact with the lens (Heubel, 1879). Ultra-microscopic examinations
have shown that the initial reversible opacity is due essentially to the state
of the water bound to the protein (Cattaneo, 1927; Fischer, 1934); when
these are merely salted out or their state of turgescence altered, recovery
can take place, but this cannot occur if their structure has been destroyed and
they have become coagulated.
It is interesting that the opacity produced by the abstraction of water may
immediately clear up on massage of the cornea or by the effect of a sharp blow on the
head (Ewald, 1898).
The clinical importance of the osmotic opacities lies in their relation to the
development of diabetic cataract."
(b) COLD CATARACT
If an isolated lens is frozen, it turns completely opaque, and on thawing
clears up from the periphery (v. Michel, 1882), the opacity being a reversible
physical change probably due to an increased refractive index of the frozen
fibres, an effect seen most markedly in the nucleus. Similarly if an animal
is frozen, a cataract develops which clears up on thawing, although the lens
may become hazy again when the animal begins to move (Kunde, 1857;
Grünhagen, 1875; Ritter, 1876; v. Michel, 1882). Here again the reaction
occurs most easily in the frog, but can be produced in young mammals
(Henle, 1866–78; Ritter, 1876–77; Hess, 1911), particularly if they are
rendered prone to lens changes by deprivation of vitamins (v. Szily and
Eckstein, 1923; Goldschmidt, 1927; Yoshimoto, 1928). The clinical
importance of this type of opacification lies in its relation to the treatment
of retinal detachments by the cryocautery.
(c) HEAT CATARACT
The lens becomes opaque on warming above 65° C. (v. Michel, 1882; Daddi,
1898), an effect due to coagulation of the proteins which is irreversible.
(d) CATARACT OF ACIDIFICATION
If the pH of the lens is reduced to between 4-5 and 5, it turns opaque owing to
precipitation of the proteins (de Haan, 1922; and others).”
Bergami. Atti Accad. Naz. da Lincei, Roma, Fischer. A. f. Aug., cviii, 80, 519, 1934.
vi, 117, 1927. Goldschmidt. Kl. W., i, 635, 1927.
Cattaneo. Atti. Accad. Naz. da Lincei, Roma, Grünhagen. Berl. kl. W., xii, 21, 1875.
v, 711, 1927. de Haan. A. meerl. de physiol., vii, 245,
Collevati. Sag. di Oft., v, 85, 1930. 1922.
Daddi. Am. di Ott., xxviii, 375, 1898. Heubel. A. ges. Physiol., xx, 114, 1879.
Deutschmann. A. f. O., xxiii, 127, 1877. Henle. Hb. d. syst. Anatomie, i, 1866.
Ewald. A. ges. Physiol., lxxii, l, 1898. Zur Amat. d. Krystallinse, Göttingen, 1878.
1 p. 3204. * Vol. I, p. 477 (486).
DISEASES OF THE LENS 3.141
Hess. G.-S. Hb., III, ix (2), 249, 1911. Richardson. Med. Times and Gaz., i, 319,
Jaeger. Ueber d. Einstellungen d. dioptrischen 412, 1860.
Apparates, Wien, 1861. Ritter. A. f. O., xxii, 255, 1876; xxiii, 44,
Köhnhorn. Diss., Gryphiae, 1858. 1877.
Runde. Z. Zool., viii., 466, 1857. Salffner. A. f. O., lix, 520, 1904.
Manca and Ovio. A. di Ott., v, l l 2, 141, Selenkowsky. Russ. O. J., iii, 613, 1925.
1897; vi., 69, 1898. v. Szily and Eckstein. K. M. Aug., lxxi, 545,
v. Michel. Ueber natürliche u. küntsliche 1923.
Linsentribung, Würzburg, 1882. Yoshimoto. A. f. Aug., xcix, 169, 1928.
Panico. An. di Ott., lvii, 613, 1929.
3. RADIATIONAL CATARACT
We have already seen that cataract can be produced by radiant
energy of any form. The long wave-lengths of electrical oscillations can
produce a cataract as in the discharge from a Leyden jar (Hess, 1888;
Riribuchi, 1900), as also can an electric shock (Brixa, 1900 ; Desbrières and
Bargy, 1905; Franklin and Cordes, 1925; Richter, 1925; Holloway, 1930;
and others). Infra-red rays readily produce a posterior polar cataract
experimentally (Vogt, 1912–31 ; Reichen, 1914; Ginella, 1924; Müller,
1924 ; Kranz, 1925; Duke-Elder, 1926; Bücklers, 1926 ; and others),
and the common occurrence of occupational cataract in workers such as glass-
blowers or in metal workers who are exposed to intense heat will be noted
subsequently. Visible rays, obtained by concentrating sunlight, have been
shown to have a similar effect (Werneck, 1834; Czerny, 1867; Deutsch-
mann, 1882; Herzog, 1903). Ultra-violet light does not cause changes
of so definite a nature, and although cataract formation has been claimed
after exposure by Widmark (1889), Herzog (1903), Chalupecky (1913)
and Schanz (1915–18), this has been denied by others (Ogneff, 1896;
Chotzen and Kuzintzky, 1918; Jess and Koschella, 1923; Trümpy, 1925).
These rays, however, do produce histological evidence of proliferation and
degeneration in the sub-capsular epithelium, as well as degenerative changes
in the lens fibres (Hess, 1907; Martin, 1912; Verhoeff and Bell, 1916;
Duke-Elder, 1929); moreover, as we shall see, chemical and metabolic
changes are produced in the lens which may be interpreted as conducive
to the formation of cataract. Finally, radium and X-rays have been found
to produce cataract experimentally (Birch-Hirschfeld, 1904–09; Alphonse,
1909; Peter, 1930; v. Szily, 1938), while the clinical occurrence of
cataract after therapeutic exposure to radium and roentgen rays is now
well known (Paton, 1909; Wilkinson, 1920) (see Injuries to the Eye).
It being thus admitted that any region of the radiant spectrum can
affect the transparency of the lens deleteriously, the question has naturally
arisen as to whether such an action can figure in the aetiology of cataract.
Radiation can only be effective in so far as it is absorbed, and while, as
we have seen,” the lens absorbs a very large proportion of infra-red rays
(between 14,000 and 11,000 A.U.), a sufficient proportion of the longer
ultra-violet rays (between 4,000 and 2,930 A.U.) penetrate the cornea and
1 Vol. I, p. 817 (827). * Vol. I, p. 812 (822).
3.142 TEXT-BOOK OF OPHTHALMOLOGY
are also absorbed by the lens to make it possible that daylight might
conceivably be an effective agent, partly from its thermal and partly from
its abiotic action.
In addition, the question of photo-sensitizers might come into play in certain
cases. It will be remembered that these so modify a substance with which they may
be associated that it reacts photo-chemically to long wave-lengths which would
ordinarily have an inappreciable effect. Thus in an animal injected with ha-mato-
porphyrin, visible rays produce the same effects as ultra-violet, and while it remains
normal so long as it remains in the dark, it dies in a few hours if placed in sunlight.
Howell (1924) has reported the development of cataract in animals so sensitized and
exposed to daylight. The possibility must therefore be considered in certain
pathological cases of visible light acting on sensitized lenticular protein.
While development of cataract after exposure to the long infra-red or
short radium emanations and X-rays is generally recognized, the possibility
of associating senile cataract with the absorption of radiant energy from the
sun, particularly the long ultra-violet rays, has been advanced since the
common and early occurrence of cataract has been noted in tropical countries
such as India (Hirschberg, 1898; Snell, 1907), or the Arctic regions
where the proportion of ultra-violet in the sunlight is high (Daland, 1917).
Such a suggestion has been supported by observations that workers in
fields who are exposed to light show a higher incidence of cataract than
town dwellers (Hirschberg, 1898; Gross, 1907), that uni-ocular cataract
tends to occur industrially on the side of constant brilliant illumination
(Ascher, 1917), and that normally senile cataract commences in the lower
quadrant of the lens where the incident light falls more directly (Greene,
1908 ; Handmann, 1909 ; Barth, 1914).
From the clinical point of view radiant energy could have two effects
on the lens. It could, by irritating or traumatizing the ciliary body, so
alter the nutrient fluid to the lens that its metabolism was disturbed and
what could virtually be called complicated cataract resulted (Parsons, 1913;
Hartridge and Hill, 1915; v. d. Hoeve, 1918–26 ; Goldmann, 1930–33),
or it could have a direct effect upon the lens itself. From the experimental
point of view two facts are of importance with regard to the latter hypo-
thesis : the first is that after radiation with thermal or abiotic rays the
glutathiome disappears from the lens (Adams, 1925; Shoji, 1931); the second
is that while ultra-violet light does not coagulate the lenticular proteins
it does so in the presence of calcium salts (Burge, 1914–18; Adams, 1929;
Hinricks, 1931).
It may be, indeed, that this mechanism may be of importance in the opacification
and ultimate calcification of the lens. Burge, Wickwire and Schamp (1937) have shown
that all radiant energy, like trauma of any kind on all tissues, turns the lens electro-
negative. They conclude that in these circumstances the lenticular phosphate
becomes ionized, liberating negative phosphate ions which combine with positive
calcium ions to form a precipitate of insoluble calcium phosphate.
DISEASES OF THE LENS 3143
We have already seen that the formation of cataract is essentially
due to a change in the lenticular proteins from the colloid to the particulate
condition by a process of coagulation occurring in two stages—dematuration,
whereby the protein is altered chemically to a readily coagulable form, and
agglutimation, a physical change whereby coagula are formed by flocculation
of the denatured particles. The first change may be brought about by any
form of radiant energy, thermal radiation (Chick and Martin, 1910–12),
visible light (Young, 1922), ultra-violet light (Dreyer and Haussen, 1907;
Bovie, 1913; Clark, 1922–35) or radium (Dreyer and Haussen, 1907;
Fernan and Pauli, 1915). The intimate mechanism of this process of
denaturation, whether thermal or photo-chemical, is probably not the
same, but in all cases, whether it occurs rapidly or slowly, it is a chemical
process of the monomolecular order and may be separated from the second
by an indefinite time-interval. The influx of radiant energy may therefore
induce denaturation, and induce it over a long period, and the denatured
protein, although showing definite physical and chemical changes involving
a decrease in stability, may remain unagglutinated, leaving the medium of
which it forms part transparent, until such time as suitable conditions
induce flocculation and consequent opacity.
It is highly improbable that, in the average case of senile cataract, the
solar energy induces direct coagulation of the lenticular protein ; but
from the foregoing it is evident that a case can be made out that radiant
energy may possibly not be without influence in a more subtle way. One
may conceive of a possible slow denaturation of the lenticular protein over
a long period owing to the continual absorption of radiant energy, thus
putting the colloid system into a more labile state so that it can more easily
be coagulated by other factors, associated perhaps with changes in reaction
or salt-content or nutritional disturbances, which acting alone in more
favourable circumstances would have little or no effect.
Again, the effect of light on the activity of the oxidation-reduction
systems on which the vitality of the lens must be remembered. We have
already noted that after exposure to light a loss of glutathione occurs.
We have seen also that lacto-flavin is highly photo-sensitive and is broken
down by light to inactive products. It must be remembered also that this
reaction is inhibited by oxygen, so that the continuance of respiration pre-
vents photolysis. By the action of light, also, the reaction SH –-SS involved
in the glutathione system is accelerated, and in the presence of flavins ascorbic
acid is oxidized ; the effectivity of all the respiratory systems in the lens
is therefore affected and maintained by the action of light. Whether,
therefore, the maintenance of transparency is due to respiration, or the
continuance of respiration depends upon the transparency is not at all clear,
but in the present state of our knowledge the former possibility must be
kept in mind.
3144
TEXT-BOOK OF OPHTHALMOLOGY
Adams. P. R. S. (B), xcviii, 244, 1925.
Biochem. J., xxiii, 902, 1929.
Alphonse. A. f. Aug., lxiv, 277, 1909.
Ascher. Med. Kl., xiii (i), 527, 1917.
Barth. Z. f. Aug., xxxii, 143, 1914.
Birch-Hirschfeld. A. f. O., lviii, 469, 1904;
lix, 229, 1904; lxxi, 573, 1909.
R. M. Aug., xlvi (2), 129, 1908.
Bovie. Science, xxxvii, 24, 373, 1913.
Brixa. K. M. Aug., xxxviii, 759, 1900.
Bücklers. A. f. O., czvii, 1, 1926.
Burge. Am. J. Phys., xxxvi, 21, 1914.
A. of O., xlvii, 12, 1918.
Burge, Wickwire and Schamp. A. of O., xvii,
234, 1937.
Chalupecky. Wien. med. W., lxiv, 1515, 1913.
Chick and Martin. J. Phys., xl, 404, 1910;
xliii, 1, 1911; xlv., 61, 261, 1912.
Chotzen and Kuznitzky. K. M. Aug., lx, 198,
1918.
Clark. Amer. J. Physiol., lxi, 72,
cxiii, 538, 1935.
Czerny. Acad. d. Wissen., lvi, 2, 1867.
Daland. O. Rec., xxvi, 116, 1917.
Desbrières and Bargy. O. Rev., xxiv, 217,
1905.
Deutschmann. A. f. O., xxvii, 241, 1882.
Dreyer and Haussen. C. R. Ac. d. Sc., cylv,
234, 1907.
Duke-Elder. Lancet, i, 1188, 1926.
Brit. J. O., xiii, 1, 1929.
Fernan and Pauli. Bioch. Z., lxx, 426, 1915.
Franklin and Cordes. J. Am. Med. AS.,
lxxxv, 245, 1925.
Ginella. A. f. O., cziv, 483, 1924.
Goldmann. A. f. O., cxxv, 313,
cxxx, 93, 1933.
Greene. J. Am. Med. As., li, 400, 1908.
Gross. A. f. Aug., lvii, 107, 1907.
Handmann. K. M. Aug., xlvii (2), 692, 1909.
Hartridge and Hill. P. R. S. (B), lxxxix, 58,
1915.
1922;
1930 ;
Herzog. B. O. G. Heidel., xxxi, 223, 1903.
Hess. Internat. O. Congr., 1888.
A. f. Aug., lvii, 185, 1907.
Hinricks. P. S. Eacp. Biol. Med., xxvii, 335
1931.
Hirschberg. Cb. pr. Aug., xxii, 113, 1898.
v. d. Hoeve. A. f. O., xcviii, l, 39, 49, 1918.
K. M. Aug., lxviii, 492, 1922.
A. f. O., czvi, 245, 1926.
Holloway. Am. J. O., xiii, 595, 1930.
Howell. In Clark’s Lighting in Relation to
Public Health, 164, 1924.
Jess and Koschella. A. f. O., czi, 370, 1923.
Kiribuchi. A. f. O., l, 1, 1900.
Kranz. K. M. Aug., lxxiv, 56, 1925.
Martin. P. R. S. (B), lxxxv, 319, 1912.
Müller. A. f. O., exiv, 503, 1924.
Ogneff. A. g. Phys., lxiii, 209, 1896.
Parsons. Internat. Cong. Med., London,
ix (1), 193, 1913.
Paton. T. O. S., xxix, 37, 1909.
Peter. A. f. O., cxxv, 428, 1930.
Reichen. Z. f. Aug., xxxi, 20, 1914.
Richter. J. A. m. Med. As., lxxxv, 315, 1925.
Schanz. A. ges. Phys., clzi, 384, 1915;
clxiv, 445, 1916; clzix, 82, 1917; clxx,
646, 1918.
Shoji. A. d’O., xlviii, 28, 1931.
Snell. Brit. Med. J., i, 8, 1907.
v. Szily. T. O. S., lviii, 595, 1938.
Trümpy. A. f. O., cxv, 495, 1925.
Verhoeff and Bell. P. A m. Acad. Arts. Sc., li,
630, 1916.
Vogt. A. f. O., lxxxi, 155 ; likxxiii, 99, 1912.
Z. f. Aug., x1, 123, 1918.
Schw. med. W., lv., 425, 1925.
K. M. Aug., lxxxvi, 289, 1931.
Werneck. Ammon's Z. f. O., iv., 14, 1834.
Widmark. Skand. A. f. Phys., i, 264, 1889;
iii, 14, 1892; iv. 281, 1893.
Wilkinson. Am. J. O., iii, 435, 1920.
Young. P. R. S. (B), xciii, 235, 1922.
4. A DECREASE OF THE PERMEABILITY OF THE CAPSULE has been shown
by Friedenwald (1930), by the precipitation upon it of non-toxic dyes, to
result in the rapid formation of lenticular opacities."
Such a procedure
stops the normal traffic of nutrient and metabolic material, and probably
produces a condition of acidosis, and is comparable to the effects of the
deposition of inflammatory exudates in the complicated cataract of cyclitis
when the lens becomes plastered with plastic exudates.
5. AN INTERFERENCE WITH NUTRIENT SUPPLIES, as by the ligation of
the posterior ciliary arteries, has been shown to produce cataract (Wagen-
mann, 1890): a similar reaction follows extirpation of the iris and ciliary
body in rabbits (Deutschmann, 1880), and, more slowly and less dram-
atically, after ligation of the vortex veins (Koster Gzn, 1895; van Geuns,
1899).
The effects probably resemble those determined by a decreased
1 p. 3137.
DISEASES OF THE LENS 31.45
permeability of the capsule and have their clinical counterparts in condi-
tions of uveal vascular stasis or congestive glaucoma—decreased anabolism,
increased catabolism, increased acidity, and necrosis.
6. CATARACT IN ASPHYXIA. In the course of extensive studies on
reversible asphyxia in rats, Amantea (1934), and Biozzi (1935) noted the
development of bilateral cataract affecting the anterior cortex. The latter
Observer studied particularly the phenomenon in an animal which had been
incompletely asphyxiated every day for a month, the most interesting
features being the rapid development and the high degree of reversibility
of the changes in the lens. The other notable ocular change was a vaso-
dilatation so marked that it led to a massive increase in the protein content
of the aqueous such that it assumed exudative proportions. He concluded
that the cataract was the result of the uncompensated acidosis which
accompanies asphyxia.
Amantea. Rend. Accad. Lincea, Roma, vi (A), v. Geuns. A. f. O., xlviii (2), 249, 1899.
20, 1934. Koster Gzn. A. f. O., xli (2), 30, 1895.
Biozzi. A. f. O., cxxxiii, 429, 1935. Pflimlin. K. M. Aug., xcii, 54, 1934.
Deutschmann. A. f. O., xxvi (3), 135, 1880. Wagenmann. A. f. O., xxxvi (4), 1, 1890.
Friedenwald. A. of O., iii, 182, 1930.
7. DEFICIENCY CATARACTS
Cataract due to nutritional disturbances has been brought about
experimentally by depriving animals either of essential amino-acids or of
vitamins, the former being necessary for the elaboration of lenticular proteins
and the latter for the continuance of metabolism.
With regard to amino-acids, the occurrence of cataract has been reported
in rats on diets deficient in tryptophame (Curtis, Hauge and Kraybill,
1932); while the larvae of the tiger salamander fed on purified milk and
casein showed a similar change (Patch, 1934). In the latter case, that the
effect was due to lack of cystine was suggested by the fact that the develop-
ment of lens changes was prevented when this substance was added to the
diet.
A lack of vitamin A was claimed by v. Szily and Eckstein (1923) to lead to the
formation of cataract, but the observations of Jess (1925) indicated that this was a
secondary effect of concomitant severe changes in the cornea and anterior segment."
The effects of a lack of vitamin B2 are much more highly substantiated.
We have already seen that it was found by Day, Langston and O’Brien
(1931) that rats fed on a diet deficient in the vitamin B, complex developed
cataract in almost 100% of cases, a complication which was prevented by
adding the vitamin to the diet. This observation has been confirmed by
other associated workers (Langston, Day and Cosgrove, 1933; Langston
and Day, 1933–34 ; Yudkin, 1933–38; Day, Darby and Langston, 1937).
1 Vol. II, p. 1754.
3.146 TEXT-BOOK OF OPHTHALMOLOGY
Apparently the deficiency must be complete, since to be effective the diet
must start on the weaning of the animals (O’Brien, 1932; Yudkin, 1933),
presumably to avoid the accumulation of previous reserves in the body.
Other workers, however, have not obtained by any means the same
consistency in their results. Sen, Das and Guha (1935) obtained cataract
in only 5 out of 41 rats, Bourne and Pyke (1935) in 15 out of 98 rats, Jansen
(1935) and György (1935) saw no lens changes in their animals, and Yudkin
(1933) obtained negative results in dogs. Moreover, in man pellagra is not
associated with cataract. It seems necessary to conclude, therefore, that
while cataract formation may occur on such a diet, it does not do so regularly,
a circumstance which suggests that the essential factor involved is uniden-
tified and therefore uncontrolled. The vitamin B2 complex, of course, is a
composite entity, and its importance lies in the possibility of lacto-flavin,
one of its constituents, taking an active part in the oxidative metabolism
of the lens."
Bourne and Pyke. Biochem. J., xxix., 1805, Langston, Day and Cosgrove. A. of O., x,
1935. 508, 1933.
Curtis, Hauge and Kraybill. J. Nutrition, v, O'Brien. A. of O., viii, 880, 1932.
503, 1932. Patch. Science, lxxxix, 57, 1934.
Day, Darby and Langston. J. Nutrition, xiii, Sen, Das and Guha. Science and Culture, i,
389, 1937. 59, 1935.
Day, Langston and O’Brien. Am. J. O., xiv., v. Szily and Eckstein. K. M. Aug., lxxi, 580,
- 1005, 1931. 1923.
György. Biochem. J., xxix, 741, 760, 1935. Yudkin. J. A. m. Med. As., ci, 921, 1933.
Jansen. Acta brev. meerl. Physiol., v, 165, 1935.
A. of O., xix, 366, 1938.
Jess. K. M. Aug., lxxiv, 49, 1925. A m. J. O., xxi., 871, 1938.
Langston and Day. South. Med. J., xxvi,
128, 1933.
J. Nutrition, vii, 97, 1934.
8. TOxIC CATARACT
(a) NAPHTHALENE CATARACT
Since the early experimental work of Bouchard and Charrin (1886),
Hess (1887), Panas (1887), Kolinsky (1889), Magnus (1890), Faravelli (1893),
Manca and Ovio (1896–98) and others, the possibility of producing cataract
in animals after feeding with naphthalene has been well known. The
optimum dose should not exceed 1 gm. per kilo. body weight daily. During
the dietary in the majority of cases the animals have remained relatively
healthy, but signs of a general toxaemia, particularly of a gastro-intestinal
nature, are common, and in some cases these have been so severe as to cause
death before the development of cataract (Klingmann, 1897; Kolinsky,
1889; Igersheimer and Ruben, 1910; Michail and Vancea, 1927; Panico,
1928; and others).
The cataract does not by any means develop invariably, but when it
does, it usually appears in from 2 to 3 weeks after the commencement of
4 p. 3134.
DISEASES OF THE LENS 3147
the diet. The lenticular changes are remarkably consistent and bilateral;
an initial increase in weight and volume of the lens due to the imbibition of
fluid (Salffner, 1904; Panico, 1928) is followed within 24 hours by the
appearance of fluid vacuoles, which are rapidly succeeded by the develop-
ment of a true opacity of the lenticular fibres beginning at the periphery,
spreading through the cortex, and finally involving the nucleus. Within
5 days from its onset the cataract is usually complete, both lenses being
milky-white and eventually developing a grey or brown pigmentation
(Fig. 2683) (Panas, 1887; Michail and Vancea, 1927). The histological
changes resemble those of senile cataract very closely, and include a sub-
Fig. 268.3.−NAPHTHALENE CATARACT.
In the rabbit (Adams, Brit. J. O.).
capsular accumulation of fluid, the separation of the fibres by homogeneous
material, the appearance of vacuoles in the fibres, and eventually a pro-
liferation of the epithelium and complete destruction of the fibres (Fig. 2684)
(Hess, 1887; Klingmann, 1897; Salffner, 1904; Busacca, 1927; Panico,
1928; Adams, 1930). Unless the naphthalene is stopped before the stage
at which actual opacities first appear, a cessation of the dose does not
prevent the full development of cataract.
Other Ocular Lesions. As we have seen, the development of cataract is
not constant, for sometimes the eyes remain normal and at other times
other ocular changes appear with or without concomitant changes in the

3148 TEXT-BOOK OF OPHTHALMOLOGY
lens. The most common of these are in the retina, where one of two appear-
ances may be seen. (1) At an early stage (3rd to 5th day) the ophthalmo-
Fig. 2684. Naphthal ENE CATARAct.
Showing complete destruction of the fibres (v. Szily, T. O. S.).
Fig. 2685. Naphthalº Poisoning in the Rabbit.
Exudative retinal changes showing the final stage of retinal atrophy and
detachment (Adams).
scopical appearance of erudative changes becomes evident at first as small
deposits in the periphery, which later develop into large confluent masses in
the central area (Panas, 1887; Dor, 1887; and many others) (Fig. 2685). As


DISEASES OF THE LENS 3149
Fig. 2686–NAPHTHALENE Poison INg.
Exudative changes in the retina of the rabbit (Adams, Brit. J. O.).
- - º
- - -
- - º- -
- - -- -
- º
A. B.
Fig. 2687.-NAPHTHALENE Poison INg.
Crystalline deposits in the retina of the rabbit: two large crystals of calcium
oxalate (?) are seen in the ganglion-cell layer opposite A and B.









3150 TEXT-BOOK OF OPHTHALMOLOGY
a rule these changes are found on histological examination to be oedematous in
nature (Adams, 1930), but an albuminous exudate has been described
between the layers of the retina, between the rod and cones and the
pigment layer, and between this and the choroid (Panas, 1887; Igersheimer
and Ruben, 1910; Takamura, 1911 ; and others) (Fig. 2686). This is
followed by atrophy, vacuolation, and more or less complete degeneration
of the retina and choroid. (2) The second appearance, which comes later
(2 to 3 weeks) and is much less common, is that of crystalline deposits, at
first in the vitreous resembling synchisis scintillans and then in the retina
(Panas, 1887; Kolinsky, 1890; and others). The crystals may be in
enormous numbers and in heaped-up masses so that the retina appears
“as a clear starry heaven,” while histologically they have been seen chiefly
in the ganglion-cell layer (Fig. 2687). They are doubly refractive and may
be either needles, plates or prisms, arranged in spheroidal or plate-like groups
and are most probably calcium oxalate (Adams, 1930). It is interesting
that while cataract frequently follows the development of the exudative
retinal change, it is rare in the animals which develop crystalline deposits,
as if these were evidence of an established resistance, or of compensation
to the effects of naphthalene.
The earlier observers (Hess, 1887; Klingmann, 1897; Sala, 1903; Lindberg,
1922), noting haemorrhages, vacuoles and hyperaemia in the ciliary body, assumed that
a disturbance of the nutrition through interference with ciliary function might be a
causal factor in the development of the Ocular lesion. It would appear, however,
that these changes are too slight and inconstant to warrant this conclusion (Salffner,
1904; Igersheimer and Ruben, 1910; Takamura, 1911; Panico, 1928; Adams,
1930).
At first it was hoped that a study of naphthalene cataract would shed
light on the pathogenesis of senile cataract, but unfortunately these hopes have
not been realized, largely because we still remain ignorant of why the changes
develop. Presumably the effect of naphthalene is either the production of a
chemical toxin or a disturbance of the normal metabolism. It can be said
definitely that a study of the effects of naphthalene (Bouchard and Charrin,
1886; Penzoldt, 1886; Igersheimer and Ruben, 1910) or of its derivatives
(Kolinsky, 1889; Salffner, 1904; v. d. Hoeve, 1907; Takamura, 1911 ;
Jess, 1928; Adams, 1930) have failed to produce evidence of any toxic
ocular effect. Similar inconclusive results have followed metabolic studies.
Although there may be a slight hyperglycaemia (Michail and Vancea,
1926–27; Komura, 1928) the sugar metabolism is within normal limits
(Adams, 1930); there is a slight hypercholesteraemia (Michail and Vancea,
1926; Adams, 1930); and the figures for calcium show no great deviation
from the normal (Michail and Vancea, 1927; Cade and Barral, 1928;
Adams, 1930; Bourne and Campbell, 1933). Bourne (1933) found that
the introduction of cabbage into the diet of the experimental animals
prevented the onset of cataract, an effect which was coincident with the
DISEASES OF THE LENS 315]
maintenance of a high blood-calcium by such a diet ; this fact, combined
with the circumstance that the deposition of calcium oxalate crystals in
the retina, which was also found to be associated with the maintenance of
a high blood-calcium, protected the animals from the development of
cataract, may be of some significance. The occurrence of cataract with a
low blood-calcium after parathyroidectomy or in tetany may be a comple-
mentary link in a chain of evidence which is not yet clear, and at the moment
is certainly most inconclusive. The most important suggestion as to the
mechanism of the development of the cataract follows from the experimental
work of Gifford (1932) and Tsuji (1932), who found that the glutathione
content of the lens was diminished after the administration of naphthalene.
This observation seemed to be rendered significant by the discovery by
Nakashima (1934) of a rise in the neutral sulphur fraction of the urine and
by the subsequent observations of Bourne and Young (1934), Stekol
(1935–37), White (1936) and Bourne (1937). They found that naphthalene
became conjugated with cysteine and was excreted as a derivative of mer-
capturic acid, so that, in detoxicating itself, the animal used up its available
cysteine, thus depleting its stock in the lens and lowering the efficiency or
even abolishing the activity of its oxidative mechanism. As a corollary,
Bellows (1936) suggested that the preventive effectivity of a diet rich in
vitamin C (cabbage, etc.) might be due to a replacement of glutathione by
this substance : it is interesting that a loss of vitamin C is a late phenomenon
in naphthalene cataract (Müller, Buschke, Gurewitsch and Brühl, 1934;
v. Euler and Malmberg, 1934). The point, however, is by no means proved.
Apparently the glutathione may be only slightly reduced when the lenticular
opacities occur (Gifford, 1932) and may still be present when the stage of
total opacity is reached (Jess, 1913), while other substances such as bromo-
benzene, which are also excreted as mercapturic and might therefore be
expected also to cause the withdrawal of cysteine, do not cause cataract
(Bourne, 1937).
Adams. Brit. J. O., xiv, 49, 545, 1930. Goldmann. K. M. Aug., lxxxiii, 433, 1929.
Bellows. A. of O., xvi, 762, 1936. Hess. B. O. G. Heidel.., xix, 54, 1887.
Bouchard and Charrin. C. R. S. Biol., viii., v. d. Hoeve. A. f. Aug., lvi, 259, 1907.
614, 1886. ... Igersheimer and Ruben. A. f. O., lxxiv, 467,
Bourne. Brit. J. O., xvii, 210, 1933. 1910.
Phys. Rev., xvii, 1, 1937. J Z jol... lxi. 93. 1913
Boºd Campbell. Brit. J. O., xvii, 220, º f. ãº, iº 913.
* > e ~~~~~ : . . Rlingmann. A. ges. Phys., czlix, 12, 1897.
Boºd Young. Biochem. J., xxviii, 803, Kolinsky. 4. j. Q., xxxv (2), 29, 1889.
Busacca. Atti Acad. Naz, da Lince, Roma, vi, .4.” ”,” º 'º",
175, 1927. Komura. A. f. O., cxx, 766, 1928.
Cade and Barral. C. R. S. Biol., xcix, 520, Lindberg. K. M. 419., lxviii. 527, 1922.
1928. Magnus. A. f. O., xxxvi (4), 150, 1890.
Dor. Rev. gen. d’O., vi, 1, 1887. Manca and Ovio. A. di Ott., iv., 167, 1896;
v. Euler and Malmberg. Z. physiol. Chem., vi, 3, 69, 1898.
ccxxx, 225, 1934. Michaîl and Vancea. C. R. S. Biol., xciv,
Faravelli. An. di Ott., xxii, 8, 1893. 291, 1926; xcvi, 63, 65, 1456; xcvii,
Gifford, H. A. of O., vii, 763, 1932. 1097, 1569, 1927.
T.O.-VOL. III. 3 Y.
3.15.2 TEXT-BOOK OF OPHTHALMOLOGY
Müller, Buschke, Gurewitsch and Brühl. Sala. K. M. Aug. xli., 1, 1903.
Kl. W., xiii, 20, 1934. Salffner. A. f. O., lix, 520, 1904.
Nakashima. J. Biochem., Tokyo., xix, 281, Stekol. J. Biol. Chem., ex, 463, 1935;
1934. exiii, 475, 1936; exvii, 147, 1937.
Panas. An d'Oc., xevii. 246, 313, 1887. Takamura. A. f. Aug., lxx, 335, 1911.
Panico. An dº Ott. Ivi, 799, 1928. Tsuji. J. Biochem. Tokyo, xv, 33, 1932.
Penzoldt. A. Earp. Path. Pharm., xxi, 34, White. J. Bºol. Chem., exii, 503, 1936.
1886.
(b) LACTosº AND GALACTosº. CATARACT
Since the production of cataract was first observed by Mitchell and
Dodge (1935) and Yudkin and Arnold (1935) after feeding rats with a diet
rich in lactose, its occurrence has been amply confirmed both with lactose
and galactose (Dodge, 1935; Jansen, 1935; Mitchell, 1936; Day, 1936;
Bellows, 1936–38; Bellows and Rosner, 1937; Yudkin, 1938; Borley
and Tainter, 1938; Jess, 1938; Mitchell and Cook, 1938; Susaki, 1938;
Cashell and Kon, 1939; and others) (Fig. 2688). There is a considerable
variability in the effects among strains of rats (Mitchell, 1936), but cataract
formation becomes apparent with considerable regularity if the animals are
Fig. 2688-Expºrtiºnal Lacrosº Cataract.
A. Normal: B. On lactose diet (Mitchell and Dodge, J. Nutrition).
fed on an adequate diet in which the sole carbohydrate is lactose or galactose,
provided that this substance occupies from 50 to 70% of the whole.
Galactose injected intra-peritoneally has no effect (Yudkin, 1938). Lenticular
changes come on more rapidly with galactose than lactose; with the former
peripheral opacities usually appear about the 3rd day, a complete superficial
opacity is apparent by the 9th to 11th day and the cataract has become
mature by the 30th day. The earliest changes are vacuoles in the periphery
and in the anterior cortex, while a posterior polar opacity and a nuclear
opacity may precede complete maturation. As a general rule the actually

DISEASES OF THE LENS 3153
growing fibres are first affected, the cortex in adult animals, and the whole
lens in the young.
The cause of the development of the cataract is still obscure. The
sugar content of the blood is increased (Mitchell, Merriam and Cook, 1937),
as is also that of the aqueous, although it would appear that the concentra-
tion of simple sugars in the lens does not alter (Susaki, 1938). A possible
osmotic effect may here be suggested. Mitchell and Cook (1937) found that
a low protein diet accelerated cataract formation, and Bellows and Rosner
(1937) that an abundant intake of cysteine had a retarding influence, a
conclusion, however, denied by Mitchell and Cook (1937) and Yudkin
(1938). On the whole, sufficient metabolic studies have not yet been done
to show whether an upset of the general metabolism is responsible. Bellows
(1936) and Bellows and Rosner (1937) found an early absence of glutathione
which disappeared from the lens before the Onset of opacities, suggesting
a derangement of the Oxidation-reduction mechanism as a causal factor.
Bellows (1938) also found an increased permeability of the lens capsule, a
fact established by experiments in vitro by Kirby, Wiener and Estey (1932):
the suggestion has therefore been made that this may exert an influence
either by allowing a disturbance of the ionic equilibrium in the lens (Mitchell,
1935), or an escape of glutathione (Bellows, 1938).
Bellows. A. of O., xvi, 762, 1936; xx, 80, Mitchell. P. S. Eacp. Biol. Med., xxxii, 971,
1938
& 1935.
Bellows and Rosner. Am. J. O., xx, 1109, J. Nutrition, xii, 447, 1936.
1937. Mitchell and Cook. P. S. Eacp. Biol. Med.,
Borley and Tainter. Am. J. O., xxi, 1091, xxxvi, 806, 1937.
1938. A. of O., xix, 22, 1938.
Cashell and Kon. T. O. S., lix, 199, 1939. Mitchell and Dodge. J. Nutrition, ix, 37, 1935.
Day. J. Nutrition, xii, 395, 1936. Mitchell, Merriam and Cook. J. Nutrition,
Dodge. A. of O., xiv, 922, 1935. xiii, 501, 1937.
Jansen. Acta brev. néerl. Phys., v, 165, 1935. Susaki. A. f. O., czzxviii, 351, 365, 1938.
Jess. K. M. Aug., ci, 761, 1938. Yudkin. Am. J. O., xxi, 871, 1938.
Kirby, Wiener and Estey. T. Am. Acad. O., Yudkin and Arnold. A. of O., xiv, 960, 1935.
Oto-Lary.., xxxvii, 142, 1932.
(c) THALLIUM CATARACT
The discovery that on a diet containing thallium, cataract can be
produced in rats is due to the work of Buschke (1922). In his early work
(1901) he had found that alopoecia resulted from this type of poisoning, but
in more extensive experiments he and his associates succeeded in producing
a symptom-complex involving a widespread degeneration of epithelial
structures, cataract, atrophy of the testicles and adrenal glands, nephritis,
a stunting of growth, and optic atrophy, all of which he attributed to an
influence on the endocrine system (Buschke and Peiser, 1922; Ginsberg and
Buschke, 1923; v. Mellin, 1923 ; Buschke, Löwenstein and Joel, 1928;
Donski, 1932). In one series of 100 animals, Buschke noted 11 which
developed cataract, of which 2 were unilateral, but there was evidence of an
3 Y 2
3154 TEXT-BOOK OF OPHTHALMOLOGY
unexplained individual and hereditary susceptibility. The lenticular
changes start as sub-capsular opacities some 2 to 4 weeks after the com-
mencement of the administration of the metal, and as the experiment
proceeds a complete cataract gradually develops. If, however, the feeding
is stopped, the other symptoms disappear and the lenticular changes remain
stationary. - n
Buschke. Verh. deut. dermat. Ges., Breslau, Buschke and Peiser. D. med. W., xlviii, 1466,
1901. 1922.
© º & Donski. A. f. O., cxxviii, 294, 1932.
Z. f. Aug., xlviii, 302, 1922. Ginsberg and Buschke. K. M. Aug., lxxi,
Buschke, Löwenstein and Joel. Klin. W., 385, 1923.
vii, 1515, 1928. v. Mellin. Diss., Giessen, 1923.
Toxic cataracts can be produced in man by other substances, such as
DINITROPHENOL, but it has not been found possible yet to produce these
experimentally, and about their aetiology nothing is known : their clinical
occurrence will be discussed in the appropriate section. -
9. CATARACT IN APARATHYROIDEA AND TETANY
The occurrence of a cataract of a peculiar type characterized by discrete
sub-capsular opacities in association with tetany, whether post-operative or
spontaneous, occurs so commonly that it could not fail to excite interest.
It was first noted that cataract could occur in relatively young persons who
were, or had been sufferers from muscular cramps by Logetschnikow (1872)
and Schmidt-Rimpler (1883); and a little later attention was drawn more
vividly to the subject on the recognition by Landesberg (1888) of a rapidly
developing cataract in a patient who suffered from epileptiform convulsions
after thyroidectomy. As clinical examples of this began to multiply rapidly,
it was realized that the occurrence of tetany with cataract was due to
accidental parathyroidectomy. The clinical features and treatment of
these cataracts will be discussed subsequently 1 ; in the meantime it is
sufficient to note their common occurrence. The sequence of cause and
effect was eventually proved by experiments on animals by Erdheim (1906),
and following him a considerable number of workers have succeeded in
demonstrating lens changes comparable to those occurring clinically by pro-
ducing tetany in dogs, rats and rabbits after parathyroidectomy (Pfeiffer and
Mayer, 1907; Adler and Thaler, 1908; Edmunds, 1915; Luckhardt and
Blumenstock, 1923; Hiroishi, 1924; Dragstedt, Sudan and Phillips, 1924;
Siegrist, 1927–28; Goldmann, 1929; v. Pellathy, 1929; Borsellino, 1934;
Campos, 1937; Lo Cascio, 1937; and others) (Fig. 2689). Histological
examination of such cataracts shows signs of degeneration of the sub-
capsular epithelium, and necrosis of the fibres in the underlying region,
with the formation of vacuoles and the aggregation of droplets of myelin and
1 p. 3210.
DISEASES OF THE LENS 3155
cellular detritus, while the remainder of the eye is normal (Hiroishi, 1924;
Siegrist, 1928; Goldmann, 1929; v. Pellathy, 1929).
The cause of the production of the lenticular opacities is unknown, and
several theories have been advanced in explanation. That they are closely
associated with the occurrence of tetany seems obvious, for clinically they
do not occur in its absence, and among young people patients with rickets
who have had cataract have undoubtedly also had tetany. That the same
rule holds experimentally was shown most convincingly by Goldmann (1929),
who was able to keep his parathyroidectomized dogs alive for 12 months,
during which time he allowed periods of tetany to come
and go by withholding and administering calcium ; he
found that during these periods sub-capsular opacities
developed, but when tetany was kept away, clear fibres
were laid down so that he was able to establish alternate
layers (in one case, six) of affected and clear zones in the
cortex. Again, the tetany is always associated with a
decreased serum calcium and increased phosphorus, and if
the calcium level is maintained no cataract develops.
Not only does this occur clinically, but in his para-
thyroidectomized dogs Rauh (1937) found that if the
blood calcium were kept at a normal level by the adminis-
tration of irradiated ergosterol, no cataract resulted.
Fig. 2689.-Axial
Moreover, without interfering with the parathyroids, SECTION or LEN's
Bahr (1936) found in feeding animals on a rachitogenic º º
diet, that if the calcium-phosphorus ratio was 4:1 in the Mised Dog.
diet no cataract developed, but if it were 1: 1, in (Slit-lamp view
which case a sudden fall of serum calcium results, (after Siegrist.)
cataract immediately appeared.
The early explanation that the cataract was caused mechanically by a
tetanic spasm of the ciliary muscle (Schmidt-Rimpler, 1883; Wettendorfer,
1897; Peters, 1900), seems unlikely 1 ; apart from the fact that
artificially produced convulsions cause no lens changes in dogs (Eiseman and
Luckhardt, 1927), cataractis unknown in other convulsive diseases as epilepsy
or after the administration of miotics over many years. It would seem more
probable that both the cataract and the convulsions are parallel results of the
same basic cause, as also most probably are the associated dystrophies of
other epithelial structures such as the teeth, the hair, the nails and the skin.
As an explanation, two alternative views have been put forward—that the
lenticular changes are due to chemical action upon the lens owing to the
deficiency of calcium, and that they are due to the action of a toxin. With
regard to the first Greenwald (1924), who found no evidence for toxic action,
believed that an actual deposit of calcium occurred in the absence of the
parathyroid hormone, but this has been denied by Evans and Kern (1931),
1 p. 3159.

3.156 TEXT-BOOK OF OPHTHALMOLOGY
who found the mineral content of these lenses to correspond with those of
senile cataract. Lo Cascio (1937) found a decrease in the ascorbic acid, the
glutathione, and the glycolytic power of the lens, associated with an increase
of calcium, an alkalosis, and a shift of the iso-electric point to the alkaline
side. Campos (1937), however, found that the decrease of oxygen consump-
tion was negligible but that the anaerobic glycolysis was definitely decreased.
But whether these evidences of lowered metabolism are cause or effect we
do not know. It is conceivable also to postulate with Kirby (1931) that the
alteration in calcium concentration may produce electro-chemical changes
with disturbances in the equilibrium of the inorganic constituents of the lens
which might alter the lability of the colloidal system. A possible alkalosis
must also be considered. It must be admitted, however, that these ideas
are merely conjectural.
The alternative theory depends on the action of a toxin affecting the
sub-capsular epithelium and the fibres thereunder in isolated areas corre-
sponding to the development of the discrete opacities (Pineles, 1906;
Goldmann, 1929; and others). What the toxin may be is not clear.
Jeremy (1919) postulated a toxaemia, uncontrolled by adequate parathyroid
secretion, and Weinstein (1933) that such a toxaemia was due to substances
such as tyramine, histamine, and guanidine liberated from muscle at the
time of spasms. Again, these conclusions are conjectural.
Whatever the aetiology may be, there is a considerable body of opinion
that many types of pre-senile cataract may be associated with tetany. The
infantile type of tetany, for example, may be responsible for lamellar
cataract, clear lens fibres being laid down after the metabolic disturbance has
passed. We have already noted that since the time of Logetschnikow (1872),
Schmidt-Rimpler (1883) and Wettendorfer (1897), juvenile cataract has been
associated with a history of convulsions, and a considerable number of
authorities also suggest that in many cases of cataract occurring in young
adults, symptoms of latent tetany may be elicited, associated with a low
blood-calcium and widespread epithelial disturbances (Peters, 1901–05;
Hesse and Phelps, 1913; Romanowa, 1934; Meesmann, 1936; Mecca,
1937; and others). Fischer and Triebenstein (1914), for example, con-
cluded that 88.2% of cases of pre-senile cataract was associated with latent
tetany, a figure which Greppin (1922) put at 50%. It is obvious that while
this position is easy to exaggerate, these arguments are not to be put lightly
aside.
Adler and Thaler. Z. f. Geburt. u. Gym., lxii, Eiseman and Luckhardt. P. S. Eacp. Biol.
194, 1908. Med., xxv, 6, 1927.
Bahr. Hygiea, xlviii, 797, 1936. Erdheim. Mitt. a. d. Grenzgeb. d. Med. u.
B. O. G. Heidel., li, 369, 1936. Chºir., Jena, xvi, 632, 1906.
Borsellino. A. dº Ott., xli., 56, 1934. Evans and Kern. Am. J. O., xiv., 1029, 1931.
Campos. An. di Ott., lxv, 481, 1937. Fischer and Triebenstein. K. M. Aug., xxvii,
Dragstedt, Sudan and Phillips. Am. J. 441, 1914.
Physiol., lxix, 477, 1924. Goldmann. A. f. O., czzii, 146, 1929.
Edmunds. P. R. S. Med. (Sect. O), ix, 53, Greenwald. J. Biol. Chem., lxi, 33, 1924.
1915. Greppin. Schw. med. W., lii, 1260, 1922.
DISEASES OF THE LENS
3157
Hesse and Phelps. Z. f. Aug., xxix, 238, 1913.
Hiroishi. A. f. O., cziii, 381, 1924.
Jeremy. Brit. J. O., iii, 315, 1919.
Kirby. A. of O., v, 868, 1931.
Landesberg. Cb. pr. Aug., xii, 39, 1888.
Lo Cascio. XV Internat. Cong. O., Cairo, iii,
401, 1937.
Logetschnikow. K. M. Aug., x, 351, 1872.
Luckhardt and Blumenstock. Am. J.
Physiol., lxii, 409, 1923.
Mecca. An. di Ott., lxv, 609, 1937.
Meesmann. B. O. G. Heidel., li, 363, 1936.
v. Pellathy. K. M. Aug., lxxxiii, 438, 1929.
Pfeiffer and Mayer. Mitt. a. d. Grenzgeb. d.
Med. u. Chir., Jena, xviii, 377, 1907.
Pineles. Cb. pr. Awg., xxx, 235, 1906.
Rauh. B. O. G. Heidel., li, 375, 1936.
Internat. Cong., Cairo, iv, 36, 1937.
Romanowa. A. f. O., czkxiii, 142, 1934.
Schmidt-Rimpler. K. M. Aug., xxi, 181,
1883.
Siegrist. B. O. G. Heidel., xlvi, 217, 1927.
Der Graue Alterstar., Berlin, 1928.
Weinstein. Brit. J. O., xvii, 236, 1933.
Wettendorfer. Wien. med. W., xlvii, 469,
528, 1656, 1897.
Peters. Z. f. Aug., iv, 337, 1900; v, 89,
1901.
K. M. Aug., xliii (1), 621, 1905.
AETIOLOGICAL THEORIES
A very large number of theories have been put forward to explain the
aetiology of cataract. Most of them suffer from the defect of generality and
in the attempt to embrace all types of cataract in one category. There is
no reason to suppose that there is only one cause, or one principal cause
operative in all cases, and doubtless there are many factors capable of
initiating the pathological changes. It may be said at once that in no case
is the mechanism understood, but it is necessary to review the various view-
points put forward. -
These theories may be divided into five classes :-
1. Biological (a) an expression of senility.
(b) genetic theories.
2. Immunological.
3. Functional, due to excessive accommodation.
4. Local disturbances (a) of nutrient supply ;
(b) of the chemistry of the lens.
5. General metabolic disturbances :
(a) General toxaemia.
(b) Conditions of deficiency.
(c) Endocrine disturbances.
1. BIOLOGICAL THEORIES
(a) Semility
In man cataract is primarily associated with senility, and there is no
doubt that in the senile cases this factor plays a preponderant rôle ; in con-
ditions of senile sclerosis of the lens it is possibly the sole factor. In this
respect the lens merely shows the characteristics of all tissues which grow
until maturity is reached and then descend into senescence. This evolution is
seen strikingly in the epithelial tissues, among which senile cataract may be
compared to the whitening of the hair, the brittleness of the nails and the
wrinkling of the skin. The changes in the sclerosed lens correspond to those
3158 TEXT-BOOK OF OPHTHALMOLOGY
of ageing tissues generally—a gradual dehydration with a loss of the water-
binding capacity, a diminished metabolism, an accumulation of waste
material with the deposition of sterol and calcium deposits, a decrease in
permeability, and a re-arrangement of the mineral skeleton of the tissue.
These changes, of course, are affected by other conditions—the results of
the stresses and strains of life, the cumulative effect of low-grade toxins
acting over a long time, endocrine failure, and the little understood but
immensely important effects of constitution and heredity.
(b) Genetic Theories
That the senile degenerative phenomena of cataract are genetically
determined has been most strongly advocated by Vogt (1928), in whose
view the lens fibres are vitally differentiated germ-plasmatically and
inherited from generation to generation. In the absence of nuclei in the
mature lens fibres, this influence must be a structural change imposed upon
one or more of the protoplasmic components, imparting lasting effects which
render the lens fibres vulnerable to degenerative influences. In this
hypothesis the primary change is thus a genetically induced alteration
in the structural configuration of the protein molecule so that its resistance
is weakened, and it succumbs more readily to environmental changes such as
may be induced by radiant energy, hormonal, nutritional, or toxic influences.
In the light of our present ignorance of these problems, such a theory is
impossible to prove or disprove, but the undoubted hereditary element in
some cases of senile cataract, and the somewhat vague findings in experi-
mental animals that some strains are more susceptible to noxious influences
than others, suggest the possible influence of some genetic groundwork.
It is arguable, however, whether these hereditary factors act directly upon
the lens itself or upon the basic malady which leads to opacification of the
lens.
2. IMMUNOLOGICAL FACTORS
A number of theories have been put forward on the general basis that
cataract is an immunological phenomenon, but in the light of our present
knowledge, although some of the suggestions are stimulating, none of them
can bear scientific criticism.
These theories are all based on the antigenic properties of lenticular
protein, and date from the claim of Uhlenhuth (1903) that the lens protein is
organ-specific and not species-specific, that is, that this protein is immuno-
logically foreign. Although some workers have reported negative results
(Braun, 1931–32), this postulate is generally agreed upon (Römer, 1905–14;
v. Szily and Arisawa, 1912; Hektoen and Schulhof, 1924; Woods and
Burky, 1927; and many others). It would appear from the later work of
Woods and Burky (1928–33) that of the three crystallins, cº-crystallin is truly
organ-specific, while 8- and y-crystallin are only partially so, the latter being
DISEASES OF THE LENS 3159
immunologically inert unless combined with one or both of the other fractions,
in which event it has a tendency to inhibit the action of the first. It is by
no means so certain, however, that the lenticular protein can act as an
antigen ; as we have seen", there is much conflicting evidence on this point,
and it would appear that oc-crystallin alone has this property (Burky, Woods
and Woodhall, 1930). .
With the exception of two experiments, however, (Guyer and Smith,
1918–24; Woods and Burky, 1933) both of which are open to criticism,
all attempts to produce cataract in experimental animals by immunological
procedures such as sensitization, intoxication, or immunization have been with-
out results. Nevertheless on the basis of one clinical finding, Römer (1906)
put forward the theory that lens protein acted as an antigen in homologous
individuals, producing specific antibodies acting as cytolysins for lens tissue.
He presumed that these cytolysins diffused into the clear lens through the
capsule, attacked the proteins there and produced a cataract. At a later
date Davis (1923–25), and after him several others, attempted the treatment
of cataract by the injection of lenticular protein 4 ; these were assumed to
stimulate the formation of antibodies which, diffusing into the lens, would act
selectively on the opaque lens fibres and cure the cataract. Unfortunately,
however, although it is known that after rupture of the capsule and the
escape of lenticular protein, a certain proportion of individuals develop a
hypersensitivity to these proteins,” there is no foundation in fact for the
supposition that the lenticular protein may exert any systemic antigenic
activity through the unruptured capsule, a conception rendered most
improbable by the impermeability of the capsule to cytolysins and other
immunological substances. It is evident, therefore, that any theory as to
the aetiology of cataract based on the immunological properties of the
lenticular protein must be viewed with as much scepticism as its cure by
these means. -
3. FUNCTIONAL THEORIES
For many years the view has been tentatively put forward that strains
of accommodation might have a deleterious effect on the lens fibres and thus
predispose to opacification. Thus, observing the development of cataract in
convulsive ergot poisoning, Meier (1862) concluded that it was due to the
tonic and clonic spasm of the ciliary muscle, while quite a number of
authorities viewed similarly the cataract associated with tetany and
aparathyroidea (Schmidt-Rimpler, 1883; Wettendorfer, 1897; Peters,
1900). Senile cataract in this view is dependent upon the extra accommo-
dative effort required with a sclerosed lens in presbyopia and the accumu-
lative effect of partial ciliary contraction to overcome astigmatism (Schön,
1889); and Burdon Cooper (1922), in support of such a suggestion, found
* Vol. I, p. 476 (485), * p. 31.79. 8 p. 2133.
3160 TEXT-BOOK OF OPHTHALMOLOGY
opacities to develop preferentially in cases of hypermetropic astigmatism.
Up to quite recent times the theory that accommodation and uncorrected
errors of refraction have a determining influence on the formation of cataract
has received sporadic support, but the failure of the development of cataract
in convulsive diseases other than tetany (such as epilepsy), or in the prolonged
ciliary spasm induced by long-continued miotics, or in experimentally pro-
duced convulsions of a type more severe than those occurring after para-
thyroidectomy (as by the intra-venous injection of sandalwood oil, Eiseman
and Luckhardt, 1927) takes away much of the point of this reasoning.
That there is an internal deformation of the lens on accommodation is
undoubted, an occurrence which can be verified by the movement of discrete
lenticular opacities observed clinically with the slit-lamp on contraction of the
ciliary muscle, movements which are abolished by atropine (Stanka, 1922;
Vogt, 1924; Gala, 1925; and others). The effect may depend on passive
strains imposed by the ciliary muscle, while in the opinion of others, an
active contraction of the lens fibres themselves in the act of accommodation
cannot be ignored. It will be remembered that a muscular function of these
fibres was postulated originally by Descartes in 1637 and Thomas Young in
1801 (the musculus cristallinus), a suggestion, however, which, although
revived by Krauss (1926), has received little or no corroboration apart from
the researches of Gillescu (1937–38) who found under the action of histamine
and acetyl choline injected through a glass capillary into the lens, an increase
of refraction up to 12 D when the ciliary muscle had been put out of action
by atropine, which he ascribed to active contractile powers of the fibres.
Sufficient research, however, has not been done upon this subject to allow of
drawing any dogmatic conclusions.
4. LOCAL DISTURBANCES
(a) Disturbances of the local nutrient supply were among the first causes
to be suggested as being of aetiological importance in cataract. In v. Michel’s
(1881) view this was due to interference with the blood supply owing to
sclerosis in the carotids and their supplying branches; in Peters’ (1904–05)
view the fault lay in senile or pathological changes in the ciliary epithelium
which, in the case of senile or pathological cataract, so altered the intra-
ocular fluid that degenerative changes appeared in the lens. In this way
cataracts produced by such diverse influences as heat or light or toxic
substances could, as we have already seen, 1 be considered explicable by
injury to the ciliary body. None of these theories, however, can claim
adequate experimental confirmation to serve as a general basis for a compre-
hensive aetiology, although the complicated cataract associated with intra-
ocular degenerations and inflammations is almost certainly due to the local
effects of toxins in the intra-ocular fluid and the consequent impairment or
derangement of the nutrition of the lens.
1 p. 3142.
DISEASES OF THE LENS 316]
(b) Local disturbances in the lens itself provide a much more attractive
basis wherein to build a theory of the pathogenesis of cataract, and the
number of propositions put forward have been legion. Becker's (1883)
original theory had a physical basis, depending on the occurrence of
irregular sclerosis involving a shrinkage of the nucleus, the appearance of
fissures between the cortical fibres and a consequent general disturbance
of their nutrition. More recent theories have been chemical in nature.
One group attributes degenerative changes to a lowering and abasement of
metabolism due to such changes as have been demonstrated in the
cataractous lens—a gradual alteration in water content, an alteration from
soluble to insoluble protein, an accumulation of calcium and lipoids, and
so on. Another seeks their cause in a failure in the oxidative mechanism—
a loss of glutathione, or a deficiency in the vitamins C or B2. As has
already been fully discussed, however, the problem as to whether these
changes are precedent and causative or subsequent and consequential is not
solved.
It will be of value, however, to summarize the initial local physico-
chemical effects which probably occur in certain forms of cataract without
dogmatizing on their causal value.
Concussion cataract—coagulation of protein through mechanical agitation.
Radiational cataract—denaturation of protein, acceleration or retardation of
enzyme action, displacement of chemical equilibria, and changes in the
electrical charges of particulate matter.
Naphthalene cataract—removal of cystine.
Cataract due to deficiency of vitamin B2—lack of lacto-flavin.
Myotonic cataract—interference with creatine and hence with glycolysis.
Cataract due to lack of capsular permeability by inflammatory membranes,
etc.—increased acidity, proteolysis.
A third group of theories, as we have seen, concerns the permeability
of the capsule or rather the capsulo-epithelial membrane. An increased
permeability has been postulated (Löwenstein, 1925–34); but more probably
any such effect is due to a decreased permeability, either as an age
phenomenon or owing to the deposition round the lens of inflammatory
membranes (Friedenwald, 1930), a circumstance which produces decreased
anabolism, increased catabolism, an accumulation of waste products,
increased acidity, proteolysis and necrosis.
5. GENERAL METABOLIC DISTURBANCES
That general metabolic disturbances have an influence in the determina-
tion of cataract has always seemed obvious both on account of the bilaterality
of the condition and because of its tendency to develop in conditions of ill-
health. Senile cataract in this view is the result of senescent metabolic
changes, and the occurrence of cataract in pathological conditions, such as
3.162 TEXT-BOOK OF OPHTHALMOLOGY
acute illnesses, diabetes, or tetany, is the direct result of these processes act-
ing upon the lens through their effects on the general metabolism. Thus at
various times evidence of renal insufficiency (Deutschmann, 1879–83;
Burdon-Cooper, 1922) a lowering of the efficiency of the carbohydrate
metabolism (Langdon, 1922–25 ; O'Brien, 1931), or a hypercholesteraemia
(Michail and Vancea, 1927; O’Brien and Meyers, 1928; Salit, 1931) has
been associated with the development of senile cataract, but on the whole,
as we have seen,” such changes are more probably a concomitant expression
of senility without any causal relation to the development of cataract, with
which no gross derangement of the blood chemistry is constantly associated.
One of the most frequently quoted derangements is that of the calcium
metabolism. The presence of large calcium deposits in the cataractous lens
led at a very early date to the view that upset of the calcium metabolism
might be of aetiological importance in the development of cataract, a
suggestion rendered the more weighty by the occurrence of lenticular
changes in conditions of tetany and by the experimental demonstration of
the effect of calcium salts on the permeability of the capsule and on the
stability of the lenticular proteins by sensitizing them to the action of
light.” We have seen, however, that a hypocalcaemia from causes other
than a parathyroid disturbance does not give gise to cataract, nor is there
any conclusive evidence that in tetany the decrease in serum calcium is
itself a causal factor. Moreover, in senile and other types of cataract, both
experimental and clinical, the calcium content of the serum is usually normal
while the endogenous calcium metabolism shows perfect balance.” It is
true that little work has been done upon the relative importance of ionized
and non-ionized calcium in the serum, but the available evidence is not
sufficient to ascribe any fundamental aetiological importance to a metabolic
disturbance of this type.
There are, however, some specific features which deserve special
mention.
(a) General Toacaemia. The experimental production of cataract by
various poisons such as naphthalene, lactose, thallium and dinitrophenol,
and the possible rôle of toxins in the cataract of aparathyroidea, lend some
credence to the view that the metabolic effect of a general toxaemia may
result in the development of cataract. In this event the rapidly developing
cataract of such acute illness as scarlet fever, meningitis, and perhaps
diabetes, has its counterpart in the slow advent of senile cataract which is
considered as the result of the cumulative effect of toxic substance in the
blood owing to faulty metabolism (Römer, 1914). In this connection a host
of authors have called attention to the part possibly played by focal sepsis,
dental, tonsillar, intestinal, and so on. How far and how intimately these
factors are associated with the development of cataract is impossible to say :
but it seems obvious that, whatever the actual aetiology of the pathological
1 p. 3127. * p. 3142. 8 p. 3126.
DISEASES OF THE LENS 3.163
changes in the lens may be, the presence of any toxic influence should
accelerate these degenerative changes, while its elimination may possibly
tend to retard their development.
(b) Vitamin Deficiency. The demonstration of the active part played
by vitamin C, and presumably also by vitamins B1 and B2 in the oxidative
metabolism of the lens, their disappearance in cataract, and the experimental
production of cataract in animals by a diet deficient in the last, have
suggested that these dietary constituents may have a possible aetiological
importance (Bellows, 1936; Yudkin, 1938; and others). It is true that
cataract generally develops at an age when vitamin deficiency could be
expected, but the evidence is not sufficient to attribute to a vitaminosis
a primary aetiological rôle.
(c) The Endocrine Glands. The number of clinical conditions wherein a
disturbance of the endocrine glands is so obviously associated with the
development of cataract is so great and the association so striking as to
provide some justification for the suggestion of a general relationship
between the two. The more obvious types of endocrine cataract present a
relatively characteristic picture of discrete sub-capsular opacities, and if
the theory is carried to its extreme limits these may be taken to comprise
a relatively comprehensive list (Possek, 1907; Schiótz, 1913; Grönholm,
1927; Elschnig, 1932; Löwenstein, 1934; and many others):—
Diabetes—associated with hypo-function of the pancreas.
Tetany—associated with hypo-function of the parathyroid.
Myotonia—associated with hyper-function of the thyroid (?).
Mongolian idiocy—associated with hypo-function of the thyroid.
Acromegaly—associated with hyper-function of the anterior lobe of the pituitary.
Diabetes insipidus—associated with hyper-function of the pars intermedia of the
pituitary(?).
Dermatoses (sclerodermia, hyperkeratosis, etc.)—associated with thyroid and
pluri-glandular disturbances.
These views have been pushed considerably further. Thus congenital
and juvenile cataracts, particularly of the lamellar and coronary types, have
been associated with latent tetany and therefore with a parathyroid defi-
ciency, and a similar aetiology has been brought forward to account for senile
cataract (Hesse and Phelps, 1913; Fischer and Triebenstein, 1914; Greppin,
1922; and others). A pituitary insufficiency has also been proposed as a
cause of senile cataract, particularly by Blatt, Bratianu, Jovin and Milcou
(1937) who concluded from metabolic analyses that such subjects had an
absence of the luteinizing factor and a deficiency of the gonadotropic
principle. The gonads, also, have received attention, and a failure, particu-
larly of the interstitial cells, has been assumed to be of determining import-
ance not only in causing juvenile (coronary) cataract, but of senile cataract
also (Siegrist, 1928; Fischer-Galati, 1929). While the development of
discrete lenticular opacities of a definite pathognomonic type is certainly
3164 TEXT-BOOK OF OPHTHALMOLOGY
associated with specific endocrine disturbances, these further associations
are very vague and extremely poorly substantiated ; moreover, even in the
admitted cases, what the mechanism of the association may be is quite
unknown.
In summary it must be said that neither the aetiology nor the mechanism
of cataractous formation is known. All the many theories which have been
proposed have some foundation in fact in that many factors have a deter-
mining influence, but in most cases it is a moot question whether the
influence is upon the lens itself or upon the underlying malady which leads
to its opacification. Probably the causes of cataract are many and various,
and it may be that they affect the transparency of the lens in many different
ways; on the other hand the rapid advance in recent years in our know-
ledge of the intimate mechanism of its metabolism may at any time provide
one single clue which may serve as a common denominator to bring order
into a region which at present is admittedly chaotic.
Becker. Zur. Amat. d. gesunder w. kranken
Lºnse, Wiesbaden, 1883.
Bellows. A. of O., xv, 78; xvi, 58, 762, 1936.
Blatt, Bratianu, Jovin and Milcou. XV
Internat. Cong., Egypt, iv., 109, 1937.
Braun. A. f. Aug., cv, 122, 1931 ; cvi, 99,
1932.
Burdon-Cooper.
1922.
Burky, Woods and Woodhall. T. Am. O. S.,
xxix, 168, 1930.
A. of O., ix, 446, 1933.
Davis. Am. J. O., vi, 295, 1923.
A. of O., liv, 172, 1925.
Deutschmann. A. f. O., xxv (2), 213; (4),
427, 1879; xxvii (1), 315, 1881; xxix (3),
191, 1883.
Eiseman and Luckhardt.
Med., xxv, 6, 1927.
Elschnig. Med. Kl., xxviii, 217, 1932.
Fischer-Galati. Clinique, xxiv, 119, 1929.
JFischer and Triebenstein. K. M. Aug., xxvii,
441, 1914.
Friedenwald. A. of O., iii, 182; iv, 350, 1930.
Gala. A. d’O., xlii, 17, 1925.
Gillescu. K. M. Aug., xcviii, 817, 1937.
A. f. O., czzxviii, 598, 1938.
Greppin. Schw. med. W., lii, 1260, 1922.
Grönholm. Acta O., v, 166, 1927.
Guyer and Smith. J. Eacp. Zoo., xxvi, 65,
Brit. J. O., vi, 385, 433,
P. S. Earp. Biol.
1918; xxxi, 171, 1920; xxxviii, 449,
1924.
Hektoen and Schulhof. J. Infect. Dis., xxxiv,
433, 1924.
Hesse and Phelps. Z. f. Aug., xxix, 238, 1913.
Jackson. Am. J. O., xxi, 264, 1938.
Krauss. Kl. W., v., 1777, 1926.
Langdon. T. Am. O. S., xx, 340, 1922.
T. O. S., xlv., 204, 1925. -
Löwenstein. K. M. Aug., lxxiv, 786, 1925.
A. f. O., cxvi., 438, 1926; cxxxii, 224, 1934.
Meier. A. f. O., viii, 120, 1862.
Michail and Vancea. C. R. S. Biol., xcvii,
1569, 1927.
v. Michel. Festschrift f. Horner, Wiesbaden,
1881.
O’Brien. T. Am. Med. As., Sect. O., 132,
1931.
O’Brien and Meyers. A. Int. Med., xlii, 376,
1928.
Peters. Z. f. Aug., iv, 337, 1900.
K. M. Aug., xlii (2), 37, 1904;
621, 1905.
Possek. K. M. Aug., xlv, Beil., 1, 1907.
Römer. A. f. O., lx, 175, 239, 1905.
A. f. Aug., lvi, Erg. 150, 1906; lxxvi, 120,
139, 1914.
Salit. A. of O., v, 354, 1931.
Schiótz, Carl. Norsk. Mag. f. Lägevidenskab.,
lxxiv, 1201, 1913.
Schmidt-Rimpler. K. M. Aug., xxi, 181,
1883. . -
Schön. A. f. Aug., xix, 77, 1889.
Siegrist. Die Grave Alterstar, Berlin, 1928.
Stanka. K. M. Aug., lxix, 731, 1922.
v. Szily and Arisawa. B. O. G. Heidel.,
xxxviii, 253, 1912.
Uhlenhuth. Festschr. 60 Geburtstag. v. R.
Koch, Jena, 1903.
Vogt. K. M. Aug., lxxii, 412, 1924.
Atlas, Berlin, 1928.
xliii (1),
Wettendorfer. Wien. med. W., xlvii, 470,
1656, 1897.
Woods and Burky. J. Am. Med. As., lxxxix,
102, 1927.
A. of O., lvii, 464, 1928; vi, 548, 1931.
Am. J. O., xvi, 951, 1933.
Yudkin. Am. J. O., xxi, 871, 1938.
DISEASES OF THE LENS 31.65
General Pathology
No matter what the aetiology or type of cataract may be, the general
pathological changes are essentially the same and relatively simple—
changes of degeneration of the lens fibres, either of sclerosis or necrosis,
associated with some aberrant activity of the epithelium. For most of our
knowledge on the subject we are indebted to the classical treatises of the
older writers, particularly Becker (1883) and Collins (1896).
The Lens Fibres
The lens fibres react in two ways. They may become sclerosed and die
slowly, a process occurring normally in the aged nucleus, and in conditions
of senile sclerosis affecting practically the entire tissue. The change is
analogous to the keratinization of the epithelium of the skin, but in the lens
the cells cannot be exfoliated and are retained. Although it causes a
change in refraction, such a process may not impair the transparency of
the fibres seriously, but in extreme instances, largely because it occurs
irregularly, involving the development of multiple irregular refracting
surfaces, the transparency of the whole lens may be gravely affected. The
process is slow and very gradual, leaving no sharp demarcation between the
living and dead cells which become fused together into an almost structure-
less homogeneous mass of rubber-like or even cartilaginous consistency.
Alternatively the lens fibres may suffer a more rapid death involving
the autolysis and necrosis characteristic of true cataract formation. In
certain circumstances mild reversible changes occur. A state of Oedema.
may interfere with the optical transparency and resolve again, as may
occur, for example, in early diabetic cataract, or alterations in the colloid
system may occur after a contusion, whereafter peptization of the proteins
results in a return of transparency. Moreover, if the metabolism is decreased
locally to a mild degree, cholesterol esters or calcium in the form of phos-
phates or carbonates or even proteins may be precipitated by a process
similar to that termed infiltration in other tissues. It is interesting that
such a precipitation may occasionally occur in a clear lens either of car-
bonates (Kranz, 1927; Boente, 1931), cholesterol (Purtscher, 1938), or
of trysoine (Braun, 1927).
If, however, an injury of any intensity is in question, coagulation of the
proteins and necrosis of the tissue result. Chemically the initial sequence
of events depends on acidification, probably largely by the accumulation
of lactic and phosphoric acids, in which circumstances, as we have seen,
proteolytic enzymes are activated and some of the proteins (8-crystallin
and albumen) are broken down into smaller fragments, while others
(o-crystallin and albuminoid) are thrown out of the colloid system in an
insoluble form.” The first reaction increases the osmotic pressure within
1 p. 31.18.
3166 TEXT-BOOK OF OPHTHALMOLOGY
the lens so that water in quantity is drawn into it from the aqueous: the
second releases water normally bound in the colloidal system of the fibres
Fig. 2690.-CATARAcrous LENs.
Showing general disintegration and necrosis of the fibres with the formation of
Morgagnian globules (x. 55) (Parsons).
Fig. 2691--Crystals is a Catar acrous LEN's
(Burdon Cooper, Brit. J. O.).
and extrudes it into the peri-fibrillar spaces to add to the accumulation
already there, while at the same time it produces a cloudy swelling of the
fibres themselves. The pathological picture thus presented is therefore


DISEASES OF THE LENS 3.167
one of gaps and fissures of all forms and sizes filled with fluid between the
fibres, which themselves are granular, cloudy and swollen, and distorted into
grotesque forms (Fig. 2690). As the necrotic changes proceed the nuclei, if they
are present, disappear, vacuoles appear which coalesce to form large spaces,
and eventually the fibres break down into round globules (Morgagnian
globules) frequently lying in rows, staining faintly with eosin and not with
haematoxylin. As time goes on, masses of albuminous coagula, homogeneous
or granular, are found, staining deeply with haematoxylin, and in the later
stages the fissures and spaces increase in size becoming filled with an almost
infinite variety of the waste products of the original fibres-clear fluid,
Morgagnian globules, albuminous coagula of insoluble protein, fatty droplets
FIG. 2692.-CHolestEROL AND TYRosis E Fig. 2693. TYRosin E CRYSTALS IN LENs.
CRYSTALs IN DIABETIC CATARACT, (Burdon Cooper, Brit. J. O.).
(Burdon Cooper, Brit. J. O.).
of myelin and lipoids, the detritus of partially disintegrated fragments of
fibres, and crystalline deposits of various types, some clear and some richly
multi-coloured (Fig. 27.28, Plate LXIII)—cholesterol (v. Graefe, 1854;
Tweedy, 1873; Lang, 1895; Krautschneider, 1897; Leber, 1906; Gross,
1906; Hoffmann, 1913; Vogt, 1919; and others), calcium phosphate and
carbonate (Wessely, 1922; Glüh, 1923; Busacca, 1925; Kranz, 1927:
Boente, 1931), and the relatively insoluble amino-acids, leucine and tyrosine
(Becker, 1877; Baas, 1897; Coats, 1912; Burdon-Cooper, 1922) (Figs. 2691
and 2692–93). Subsequent degenerative changes may take the form of exten-
sive fatty depositions which have been noted in senile cataract (Toufesco, 1906),
in coronary cataract (Metzger, 1931) and in the cataract complicating
glaucoma (Jess, 1933). A more common change, however, is an impregna-
tion with calcium salts. Such a cataracta calcarea is a common occurrence
T.O.-WOL. III. - 3 Z.

31.68 TEXT-BOOK OF OPHTHALMOLOGY
particularly in the later stages of complicated cataract (Becker, 1883;
Kahler, 1911; Ribas and Menacho, 1915; and others).
When the lens capsule has been ruptured or disintegrated, changes of a more
advanced nature may occur, including the entrance of fully formed granulation and
fibrous tissue. Rarer events are a widespread fatty impregnation (xANTHoMATosis
LENTIs: v. Szily, 1923; Sala, 1935), and the formation of true bone showing sometimes
Haversian systems (CATARACTA ossºa. Gluge, 1843; Ayres, 1882; Dunn and Holden,
1898; Aubineau, 1904; Roure, 1905; Pitsch, 1926; Betsch, 1927; Michail, 1934).
Some of these cases are associated with ossification in the choroid, and the bone in
the lens has been detected radiographically (Kaufmann, 1932).
The ultimate fate of the lens substance depends largely upon its age.
In the young lens if the injury is severe and general, it frequently happens
that owing to the preponderance of soluble protein and the paucity of
insoluble protein, the process of proteolysis may be so complete that the
lens may become entirely absorbed leaving a condition of aphakia with
the capsule alone remaining. If the injury is localized, a portion of the
Fig. 2694–Zonullan (LAMELLAR) CATARAct (v. Szily, T. O. S.).
lens substance may be destroyed; the débris may be almost entirely absorbed
leaving a faceted, flattened lens; or alternatively, a localized area of
opacity may remain which is enclosed and buried by the formation of new
clear fibres resulting in a lamellar or a punctate cataract (Fig. 2694). If,
however, the lens is of adult age, a considerable residue remains which
resists enzymic action. In this event, while the nucleus may remain rela-
tively unchanged, showing perhaps large fissures and areas of regional
degeneration, the cortex becomes converted into a pultaceous mass. At
this stage, owing to the diffusion away of the products of proteolysis, there
is a loss of water, and the lens becomes inspissated, wrinkled and shrunken,
flat and yellowish, and frequently glistening with scattered cretaceous
deposits and bright crystalline accumulations. Such a cataract is termed
hypermature. If, on the other hand, owing to thickening of the capsule
and proliferation of the epithelial cells, permeability is abolished, the water
is retained within the capsule, and the cortex, instead of becoming pul-
taceous, turns fluid. The fluid is of a milky colour and is richly albuminous,
holding in suspension globules of myelin and coagulated protein, granular

DISEASES OF THE LENS 3.169
º
Fig. 2695–MoRGAGNIAN CATARAct (v. Szily, T. O. S.).
detritus and remnants of individual fibres with frayed ends and corroded
edges. Meanwhile the nucleus, which is gradually converted into a homo-
geneous shrunken mass, sinks to the bottom of the capsule and is dislocated
Fig. 2696–Mortgagxian CATARAct.
The capsule contains albuminous fluid at the bottom of which lies the darkly
staining nucleus lying with its long axis antero-posteriorly (> 10) (Parsons).


3 * *
3170 TEXT-BOOK OF OPHTHALMOLOGY
*
Fig. 2697. --MoRaag NIAN CATARAct.
Showing Morgagnian globules (x 100) (Parsons).
Fig. 2698 –Capsular Opacities is Mona Aastas Cataract.
tº 72) (Herbert, T. O. S.).


DISEASES OF THE LENS 317 I
either into the posterior or anterior chamber (Morgagnian cataract) (Figs.
2695-97). Morgagnian cataracts may undergo several further interesting
degenerative changes (Herbert, 1915 ; Gabrielidès, 1924; and others).
In the most advanced cases the nucleus also entirely disappears leaving
only milky fluid enclosed in a thickened capsule dotted with small white
Opacities. These spots represent spaces enclosed between layers of the
capsule filled with fluid and granular débris which may eventually undergo
calcareous degeneration (Fig. 2698).
While at one time a Morgagnian cataract was a commonplace, so much so, indeed,
that Morgagni (1764) looked upon it as the normal form of cataract, since the adoption
of widespread operative treatment it has become rare. Thus, although it occurs more
frequently than the figures indicate, Taylor (1911) recalled that no case had been
brought before the Ophthalmological Society of the United Kingdom for 30 years,
and Chance (1912) found a similar absence in the American Ophthalmological Society
for 22 years.
Although it is the rule in young persons that a cataract is completely
absorbed if the capsule is opened, partly through the action of ferments
and partly through phagocytosis (Rodigina, 1932), spontaneous absorption
Occasionally occurs although the capsule is apparently unharmed (Ferrer,
1928; Vancea, 1932; and others). Even in old people a cataract may as
a rarity be completely absorbed through an intact capsule, either spon-
taneously (v. Reuss, 1900; Ballantyne, 1926; Holloway and Cowan, 1932),
or after an iridectomy (Paterson, 1930 ; Butler, 1932). It may happen,
also, in a Morgagnian cataract that the nucleus, floating in a relatively clear
liquid, may sink to the bottom of the capsule, allowing a considerable
amount of vision through the upper part of the pupil (Butler, 1925; Aliquo-
Mazzei, 1930). It is possible that some of the cases reported as of
spontaneous absorption may have been caused by a rupture in the thin
capsular wall, an occurrence which has been noted on several occasions
(v. Szily, 1884; Rollet and Genet, 1913; Gonzales, 1919; Kaufman,
1933; Knapp, 1937).
The Epithelium
The only cells of the lens which show any signs of regenerative activity
are those of the sub-capsular epithelium. As age advances some degenera-
tive evidences may become apparent—a flattening and vacuolation of the
cytoplasm and a loss of nuclei. We have seen,” however, that in response
to injury these cells proliferate, sometimes forming masses several cells
thick (Schirmer, 1888–89), and in cataract the same tendency occurs so
that under the anterior capsule several layers may appear. Treacher
Collins (1890) suggested that the stimulus was a lessened tissue-tension
owing to shrinkage of the cataractous fibres, but a more probable stimulating
1 J. BAPT. MoRGAGNI (1682–1771) Professor of Anatomy in Padua : Adversaria Anatomica
Omnia Lugduni Batavorum, 1723. Epistolae Anatomicoe, xviii and xix, p. 356-377, 1764.
2
p. 3103.
3172 TEXT-BOOK OF OPHTHALMOLOGY
influence is the abnormal chemical products of the pathological lens. When
the normal whorl-like arrangement of the equatorial fibres is destroyed,
the proliferative process proceeds apace, and cells of irregular shapes and
sizes spread underneath the posterior capsule so that the entire lens may
become surrounded by epithelium (Fig. 2758). Sometimes individual cells
become large, swollen and spherical, with a faintly staining nucleus which
never shows mitosis and may eventually disappear. These vesicular cells
(the Bläschenzellen of Wedl) were considered by Becker (1883) to be due to
“hydropic degeneration,” but more probably they represent abortive
attempts to realize the inherent capacity of such cells to form lens fibres
(Parsons, 1905).
Aliquo-Mazzei. Boll. d’Oc., ix, 170, 1930.
Aubineau. An. d’Oc., czz.xii, 100, 1904.
Ayres. A. f. Awg., xi, 323, 1882.
Baas. A. f. O., xlv, 657, 1897.
Ballantyne. Brit. J. O., x, 202, 1926.
Becker. G-S. Hb., I, v, 186, 1877.
Zur. Amat. d. gesunden u. kranken Linse,
Wiesbaden, 1883. -
Betsch. K. M. Aug., lxxix, 48, 1927.
Boente. A. f. Aug., cii, 260, 1931.
Braun. A. f. O., czviii, 701, 1927.
Burdon-Cooper. Brit. J. O., vi, 385, 433,
1922.
Busacca. Rev. g6m. d’O., xxxix, 439, 1925.
Butler. T. O. S., xlv, 657, 1925.
Brit. J. O., xvi, 35, 1932.
Chance. J. Am. Med. As., lix, 1013, 1912.
Coats. T. O. S., xxxii, 153, 1912.
Collins. T. O. S., x, 145, 1890.
Researches, London, 1896.
Dunn and Holden. A. of O., xxvii, 499, 1898.
Ferrer. Am. J. O., xi, 886, 1928.
Gabrielidès. Am. d’Oc., clzi, 561, 1924.
Gluge. An. d’Oc., x, 266, 1843.
Glüh. Diss., Rostock, 1923.
Gonzales. Am. J. O., ii, 742, 1919.
v. Graefe. A. f. O., i, 323, 1854.
Gross. A. f. Aug., lvii, 107, 1906.
Herbert. T. O. S., xxxv, 349, 1915.
Hoffmann. Münch. med. W., lx, 741, 1913.
Holloway and Cowan. A. of O., vii, 332, 1932.
Jess. K. M. Aug., xci, 830, 1933.
Kahler. Diss., Marburg, 1911.
Kaufman. A. of O., ix, 56, 1933.
Raufmann. Röntgenpraac., iv, 437, 1932.
Enapp, H. C. Am. J. O., xx, 820, 1937.
Rrautschneider. Beit. z. Aug., xxvi, 34, 1897.
FCranz. A. f. O., exviii, 571, 1927.
Lang. T. O. S., xv, 117, 1895.
Leber. A. f. O., lxii, 85, 1906.
Metzger. K. M. Aug., lxxxvii, 850, 1931.
Michail. A. f. O., czkxi, 390, 1934.
Parsons. Path. of the Eye, London, ii, 392,
1905. .
Paterson. T. O. S., l, 617, 1930.
Pitsch. K. M. Aug., lxxvii, 636, 1926.
Purtscher. A. f. O., czzxix, 358, 1938.
v. Reuss. Cb. pr. Aug., xxiv, 33, 1900.
Ribas and Menacho. A. de Oft. H.-A., xv, 257,
1915.
Rodigina. Sov. vestm. O., i, 121, 1932.
Rollet and Genet. Rev. gem. d’O., xxxii, l,
1913.
Roure. Rev. gen. d’O., xxiv, 49, 1905.
Sala. Boll. d’Oc., xiv, 266, 1935.
Schirmer. A. f. O., xxxiv. (1), 220, 1888 ;
xxxv (1), 131, 1889.
v. Szily. Cb. pr. Aug., viii, 17, 1884.
K. M. Aug. lxxi, 30, 1923.
Taylor. T. O. S., xxxi, 146, 1911.
Toufesco. Am. d’Oc., cxxxv, 265; cxxxvi,
1, 1906.
Tweedy. Lancet, ii, 519, 1873.
Vancea. A. d’O., xlix, 78, 1932.
Vogt. K. M. Aug., lxiii, 232, 1919.
Wessely. A. f. Aug., xci, 158, 1922.
General Symptomatology
THE SUBJECTIVE SYMPTOMATOLOGY of cataract is comprised shortly in
disturbances, then diminution, and finally failure of vision. To a large
extent the disability depends on the situation of the opacity. Thus, because
it is situated at the nodal point of the dioptric system, a relatively small
opacity at the posterior pole may be incapacitating, while peripheral
opacities which leave the axial region free may cause little inconvenience.
Similarly a nuclear sclerosis interferes with vision much more decisively
than a cortical opacity, which indeed may be compatible with almost normal
DISEASES OF THE LENS 3.17.3
acuity if clear spaces exist between the opaque areas. Moreover, a patient
with an axial opacity sees best in dull light when the pupil is dilated, while
in peripheral cataract a bright light improves the vision because of the
contraction of the iris.
In general, however, the subjective symptoms are four-fold. First,
the appearance of black spots which move only with movements of the eyes
and are especially prominent when looking at a bright background.
It will be remembered that under suitable conditions, as by looking through a
stenopaeic hole in a black card held at the anterior focus of the eye, lenticular opacities
can be clearly seen and their pattern drawn and their progress followed by the patient
***
LEFT RIGHT
Fig. 2699.-ENTopTIC APPEARANCE of SENILE CATARACT.
The inverted entoptic image of the lens opacities seen by looking at a window
through a pin-hole opening. The piece cut offin the right eye is the interception of the
upper lid (East, Brit. J. O.).
himself (Fig. 2699) (Darier, 1895; East, 1922; Priestley Smith, 1924). More accurate
optical conditions, but relatively little more practical results, are achieved by the use
of an entoptoscope * (Barrett, 1918; Scheerer, 1924–25).
Second, uni-ocular polyopia is frequently an early symptom, whereby
the images of objects are distorted or reduplicated; the phenomenon is
due to irregular refraction within the lens, a development which also
produces stars and beams and coloured diffraction halos around lights.
The differential diagnosis of such halos from those due to glaucoma has
already been considered.” Third, the development of lenticular
myopia, due essentially to nuclear sclerosis: this may be relatively
slight, and in so far as the presbyopic person may be able to read
without his glasses, may be interpreted by the patient as an actual
strengthening of vision (second sight), but when it attains marked dimensions
(up to −20 D and more) may be extremely incapacitating. Finally an
impairment of vision eventually appears. Reversible oedematous and
refractive changes may cause considerable variations in the visual acuity
1 Vol. I, p. 809. (819). * Vol. II, p. 1231. * Vol. II, p. 1232.

3174 TEXT-BOOK OF OPHTHALMOLOGY
from time to time, but eventually as the opacification progresses, the vision
decreases until finally quantitative perception of light only may be left.
An incidental feature which is not often remarked is the change in colour values.
The sclerosing lens absorbs the shorter wave-lengths of the spectrum, first the violet
and blue, and eventually sometimes up to the yellow, so that the resultant effect is
as if looking through a colour-filter with the blue and violet excluded. It has been
said that this disability accounted for the warmth of the colours in the works painted
by Turner in his latter years. Conversely, after an extraction of cataract, the sudden
influx of short-waved light in the presence of an established adaptation to the yellow
and longer wave-lengths, frequently results in a fleeting blue vision (cyanopsia).”
THE OBJECTIVE SIGN of cataract is the presence of opacities in the lens.
By direct illumination these appear grey or white, but in judging the condi-
tion of the lens it must be remembered that, owing to internal reflection
and refraction and absorption of the shorter wave-lengths, the senile lens
may appear grey and relatively opaque when in reality it is highly
transparent and interferes little with visual acuity. While an advanced
cataract is obvious, a more accurate assessment of early opacities is made
by transmitted light when actual opacities, obstructing the light reflected
from the fundus, appear black in the pupillary reflex.
THE OBJECTIVE CLINICAL ExAMINATION is therefore most satisfactorily
started by observing the fundus reflex with the ophthalmoscope or retino-
scope, at first at a distance of about ; metre, and then with a + 20 D lens
at close focus. Such an examination should always be done under full
mydriasis since the earlier opacities are frequently peripheral. Not only
by this method are the opacities recognized as such, but their position in
the lens can be assessed by the phenomenon of parallax.”
Examination with the slit-lamp, however, provides information of
even more value, allowing as it does a detailed microscopic view of the lens
by direct or transmitted light and by indirect lateral illumination by which
fine changes and vacuoles can be detected, as well as permitting a study
of the specular reflection from the surfaces.” By its means not only can an
accurate knowledge of the type and form of any opacity be gained but more
subtle effects, such as the presence of sub-capsular iridescence and the
relief of the adult nucleus, can be appreciated : moreover, pathological
changes can be accurately localized topographically in the cortex, the
adult, infantile or foetal nucleus.
In the clinical examination certain objective and subjective tests are
of importance in assessing the operability of the case and the prognosis.
Of the objective questions the most important are :
1. The state of the nucleus : whether it shows evidence of senile sclerosis and how
far the sclerotic process extends towards the periphery, questions which affect the
ease of delivery of the lens in extraction.
1 Vol. I, p. 1000. (1012). * Vol. II, p. 1163. * Vol. II, p. 1144.
DISEASES OF THE LENS 3.175
- ©
FIG. 2700.
FIG. 27.01.
FIGs. 2700–01.--THE CLINICAL DETERMINATION of MATURITY.
In immature cataract a shadow of the iris appears by oblique illumination
(Fig. 2700), which is absent in mature cataract (Fig. 2701).
2. The state of the cortex : whether it is still clear (in which case the cataract is
clinically immature), whether complete opacification extends right up to the capsule
(mature cataract), or whether it is liquefied (Morgagnian cataract). To estimate the
depth of clear cortical substance, a useful rough clinical test depends upon the observa-
tion of the depth of the shadow cast by the iris on oblique illumination; if clear
substance still remains, the iris throws a shadow of itself upon the grey opacity in the
Fig. 2702.-MoRo AGNIAN CATARACT.
Note the iridescent shagreen on the capsule. The nucleus lies below, free in the
liquid cortex (Harrison Butler, T. O.S.).


3.17.6 TEXT-BOOK OF OPHTHALMOLOGY
pupil, but if the cortex is completely opaque so that the pupillary margin lies almost
in contact with the opacity, no shadow appears (Figs. 2700 and 2701). A much
more accurate measure can, however, be obtained by direct observation of the optical
section of the lens with the slit-lamp. If the cortex is liquefied the dense grey nucleus
may be seen by direct illumination occupying the lower area of the pupil, but here
again a more accurate assessment can be made by the slit-lamp. with which the nucleus
can be seen in optical section floating freely in the liquid cortex (Fig. 2702).
3. The presence or absence of any signs of inflammation should be carefully
determined to eliminate a possible complicated aetiology—corneal bedeving or
endothelial deposits, cells and débris in the aqueous, or iridic synechiae and patches of
atrophy. With the relatively immobile pupil of old persons, the freedom of the iris
cannot sometimes be adequately established without dilatation with a mydriatic.
4. Every cataract, no matter how dense, transmits light freely, so that a pupillary
reflex should be obtained on transillumination : its absence indicates growth or
organized material or blood in the vitreous. .
THE FUNCTIONAL TESTs for the demonstration of a healthy functioning
retina and optic nerve are of importance ; if the lens is opaque the fundus
cannot be seen and such knowledge is essential if the extreme disappoint-
ment is to be avoided of undertaking an operation which may lead to no
useful result owing to the existence of other disease. For this reason the
careful and complete examination of the fundus under full mydriasis of
every case of incipient cataract should be scrupulously undertaken for the
purpose of future reference. If, however, the lens is already opaque, the
following tests are of value :—
1. The pupil should always react promptly and normally.
2. A point of light should be immediately and accurately located in every part of
the visual field. Such a test should be undertaken in a dark room, when a small
point of light should be easily seen at 6 metres’ distance, and a light should be accurately
localized by pointing with the finger when held in all quadrants of the visual field.
3. Macular function can be assessed by holding in front of the eye a black disc
perforated with two or three pinholes, behind which a light is shining ; ability to count
the holes and recognize their pattern indicates a healthy macula (Young, 1917–23).
4. With an intelligent patient considerable information may be obtained by the
method of auto-ophthalmoscopy described by Eber (1922) and Friedman (1931);
it will be remembered 1 that by rubbing the lower lid with the electric bulb of an
ophthalmoscope pressed against the globe, an inverted image of the fundus is seen
by the patient wherein macular or peripheral lesions or field defects appear as black
patches in an illuminated ground.
THE COMPLICATIONS OF CATARACT are few, the most important being a
secondary rise of tension. Such a secondary glaucoma occurs either at the
intumescent or the hypermature stage. Not infrequently in the intumescent
stage the rapid swelling of the lens presses upon the ciliary body and sets
up neuro-vascular reflexes leading to stasis and congestion and eventually
produces an irritative irido-cyclitis and finally obliterates mechanically
an already restricted drainage angle ; this complication was first clearly
recognized by v. Graefe (1869) and has been studied histologically by
" Vol. II, p. 1233.
DISEASES OF THE LENS 3177
Ischreyt (1909), Salus (1910), Morax (1922), and Gonzales (1922). A secon-
dary glaucoma also occurs in hypermature and Morgagnian cataracts. In
the latter the occurrence of secondary glaucoma associated with an absorp-
tion of the cataract is an interesting phenomenon of which there are a
considerable number of reports in the literature (4 cases of glaucoma in
34 collected cases of absorption, v. Reuss, 1900; H. Gifford, 1900–27;
Verrey, 1916; Verhoeff and Lemoine, 1922; and others). Occasionally
in such cataracts the rise in intra-ocular pressure follows a spontaneous
rupture of the capsule, which had become thin and frail (v. Szily, 1884;
Rollet and Genêt, 1913; Gonzales, 1919; Kaufman, 1933; H. C. Knapp,
1937): in this event the tension is probably due to stasis and congestion
induced by the setting up of neuro-vascular reflexes aided by blockage of
the drainage channels by the débris of the lens. A further influence may
be the toxic properties of this material (Gifford, 1918–27), to which a
superimposed allergic reaction may be added if sensitivity to lenticular
protein exists.” Finally, A. Knapp (1927) suggested that the mechanical
effect of the loose nucleus might irritate the ciliary body. The rapid resolu-
tion of the tension which frequently follows evacuation of the anterior
chamber suggests a mechanical and reflex explanation.
A further complication of hypermature cataracts is sub-lua'ation or
dislocation of the lens 4 owing to atrophy of the zonular fibres : if the dis-
location is backwards no reactive catastrophe may result, at any rate for
a considerable time ; but if the luxation is forwards and the lens is arrested
in the pupil (v. Szily, 1884), or becomes dislocated into the anterior chamber
(Natanson, 1891; Mitvalsky, 1892; Harms, 1905; and others), a severe
º
A
irritative and exudative reaction is invariably followed by secondary
glaucoma.
Barrett. T. O. S., xxxviii, 349, 1918. Mitvalsky. Cb. pr. Aug., xvi, 289, 1892.
Darier. Am... d’Oc., cziv, 198, 1895. Morax. An... d’Oc., clix, 185, 1922.
East. Brit. J. O., vi, 365, 1922. Natanson. K. M. Aug., xxix, 423, 1891.
Eber. A m. J. O., v, 973, 1922. v. Reuss. Cb. pr. A ug., xxiv, 33, 1900.
Friedman. A. of O., v, 636, 1931. Rollet and Genét. Rev. gen. d’O., xxii, l, 1913.
Gifford, H. Am. J. O., xvii, 290, 1900; i, Salus. K. M. Aug., xlviii (2), 167, 1910.
83, 1918. Scheerer. K. M. Aug., lxxiii, 67, 1924 ;
A. of O., lvi, 457, 1927. lxxiv, 688, 1925.
Gonzales. Am. J. O., ii, 742, 1919. Smith, Priestley. Brit. J. O., viii, 145, 1924.
Rev. Cub. de Oft., iv., 100, 1922. v. Szily. Cb. pr. Aug., viii, 17, 1884.
v. Graefe. A. f. O., xv (3), 153, 1869. Verhoeff and Lemoine. A m. J. O., v. 700,
Harms. K. M. Aug., xliii, 147, 1905. 1922.
Ischreyt. A. f. Aug., lxii, 272, 1909. Verrey. Am. J. O., xxxiii, 230, 1916.
Kaufman. A. of O., ix, 56, 1933. Young. Brit. J. O., i, 362, 1917; vii, 167.
Knapp, A. A. of O., lvi, 124, 1927. 1923.
Knapp, H. C. Am. J. O., xx, 820, 1937.
General Treatment
With a name associated in the popular mind with the catastrophe of
blindness or alternatively with the ordeal of an operation, with a symptom-
atology characterized by a slowly progressive and apparently inevitable
- 1 p. 2133. * p. 3241.
31.78 TEXT-BOOK OF OPHTHALMOLOGY
diminution of vision at a time when physical disabilities make the patient
depend necessarily more and more upon reading for his comfort and
happiness, and having a course which no palliative treatment can appreciably
alter, cataract presents a therapeutic problem with all the characteristics
necessary to stimulate the exploitation of more or less illegitimate “cures.”
To a large extent the reputation which temporarily attaches to such cures
is due to the fact that many cataracts independently of any treatment
progress exceedingly slowly, and most of them in their evolution show
periods of visual betterment owing to refractive changes in the lens : the
delay and the improvement are readily attributed to the nostrum. The
point of view of the patient in submitting to such exploitation is under-
standable : the healer may derive comfort from the hope that he is allaying
the anxieties of his patient and saving him from more flagrant exploitation
by others; but psychological motive apart, from the time of Celsus (“when
recent a cataract is often removed by medicine '’) and Galen (“ a cataract
at the beginning can be dispersed, but not later ’’), the history of the thera-
peutics of cataract presents a unique display of what can most charitably
be called extreme credulity in unsupported theories or wishful interpreta-
tion of clinical results. It is true that in certain forms of acute cataract,
if the exciting conditions are controlled, the process may be reversed so
long as irreversible changes have not taken place in the lens; but it is also
true that once an organic opacity is formed it is immutable. The coagulation
of protein is an irreversible chemical change. It seems not unreasonable
to hope that in the future, when our knowledge of these changes and the
factors causing them is more fully developed, it may be possible to prevent
the incidence of cataract or delay its occurrence ; but at the present
time, while it is certainly legitimate and incumbent upon the physician to
attack or eliminate any exciting cause or predisposition that is known or
suspected, this is as far as honesty can go.
The methods which have been advised for the treatment of cataract
are legion. They may be considered under two headings—treatment to
cause absorption of the opacities, and treatment designed to improve
nutritional and metabolic disturbances or deficiencies which may play some
determining part in the opacification of the lens. There remain the provision
of optical expedients to aid the patient when the diminution of vision
becomes embarrassing, and finally the removal of the opaque lens by
operative measures.
The first category can be rapidly dismissed, for local treatment designed
to cause delay in the formation of the opacities or their absorption when
formed has little basis in reason and as little justification in results. It is
to be remembered, however, that when the independence and isolation of
the lens is recalled, it is by no means impossible that future progress in
knowledge may indicate local measures which may so maintain or stimulate
its metabolism that opacification may be prevented.
DISEASES OF THE LENS 317.9
Among drugs employed the most common, and one of the oldest remedies proposed,
has been iodine, which, with a view to aiding absorption, has been given in every
conceivable form, mainly as iodide—by mouth, as drops, in an eye-bath, as a salve,
in sub-conjunctival injections, and by iontophoresis (Pagenstecher, 1897; Badal,
1902; Verdereau, 1904; v. Pflugk, 1906; Pick, 1909; Dor, 1911; Meyer-Steineg,
1913–24 ; Walter, 1914 ; and many others). It is true that iodine does enter the
lens from the conjunctival sac (Daniels, 1931), although with difficulty and in small
quantity (Löhlein, 1910), but no basis exists for the extravagant claims put forward
with regard to its therapeutic efficacy. Calcium is frequently advised with the iodide
either by mouth as a salve (Hildersheider, 1929; Burdon-Cooper, 1933), and, owing to
its diminution in the cataractous lens, potassium either by mouth as the iodide or
locally by iontophoresis (Burdon-Cooper, 1933). The maintenance of a high serum
calcium level by the administration of the metal or of irradiated ergosterol (Rauh, 1936)
may prevent the development of cataract in experimental tetany," but this applies
to one particular type of cataract only. Homoeopathic remedies, such as infinitesimal
doses of naphthalene or secale, have of course had their advocates (Tischner, 1914;
and others).
The production of a local hypercemia has been claimed to be of value, this having
a supposedly beneficial effect on the nutrition of the lens. Various methods have been
enthusiastically practised, the instillation of dionine (Pollock, 1923; Greenwood, 1924),
or boracic and glycerine (Weeks, 1930), sub-conjunctival injections of cyanide of
mercury (Smith, 1912–28 ; Dean, 1924–27), injections of lactogenin (a milk prepara-
tion) (Harkness, 1925), and so on.
Physical methods of treatment have also received a trial : radium, even although
it causes cataract (Cohen and Levin, 1920 ; Levin, 1920 ; Franklin and Cordes, 1920),
pneumo-massage and electrotherapy (Harris, 1924).
Immunological therapeutics have had their place. Römer (1907–10) was the
pioneer in this line of thought : he claimed that specific toxins for lens substance
(phakolysins) are formed in the body owing to faulty metabolism against which
immunity could be acquired by immunization. Lentocalin, a preparation of lenticular
protein, was employed for this purpose. Salus (1926) used a sub-conjunctival injection
extract for systemic immunization (Selinger, 1935) or fish lens extract (Shropshire,
(1937). Davis (1932) has employed lens protein in the hope that it will produce specific
lysins which will absorb the lenticular opacities; but any treatment based on the use
of lens antigen has yielded no results which can be substantiated (Ellis, 1928; Biffis
and Quaglio, 1933; Selinger, 1935 ; and others).
A further attempt at therapy has depended upon the maintenance of the flagging
lenticular metabolism by supplying the active factors lacking in cataract. Thus
feeding with sulphydril compounds (glucothiose) has been advocated by Kögel (1931),
and an abundant intake of vitamins by Burdon-Cooper (1933), Yudkin (1938), and
others. -
Finally, the prevention and retardation of the opacities has been advocated by
those who have seen in the activity of the ciliary muscle an aetiological factor in
cataract by the constant employment of glasses to ease accommodative strain (Schön,
1887–89; and others).
The second line of approach—the rectification of the metabolic failures
which are responsible for cataract—has more to recommend it. In those
cases where in the aetiology is clear and simple, the results may be brilliant
and the progress of opacification may cease. Thus typical diabetic cataract
1 p. 3155.
3.180 TEXT-BOOK OF OPHTHALMOLOGY
may be arrested by adequate control of the diabetes, and those types of
cataract due to specific endocrine failure may remain stationary with an
ample supply of the deficient hormone. This applies, for example, to the
cataract of aparathyroidea, of Mongolian idiocy, and so on ; but when these
principles have been strained and applied to more debatable problems, the
results have been more than disappointing. For example, senile cataract
has been ascribed, as we have seen, to a chronic condition of latent tetany
and treatment by parathyroid and calcium advocated (Treu, 1908; and
many others): the premises are wrong and the treatment inefficacious
(Kirby, 1931). Again, senile cataract has been attributed to a pluriglandular
failure, particularly of the gonads, and hence treatment has been advocated
by ovarian extracts (Gallus, 1923) or a mixture of thyroid, parathyroid, and
gonad extracts (euphakin: Siegrist, 1928) together with vasotomy: here
again the results have been less than inconclusive. -
Whatever the aetiology of senile cataract may be, there is no doubt
that its development is associated with the onset of senile changes generally
throughout the body, and its presence should serve as an indication for
reviewing the general life and habits of the patient. Herein lies the import-
ance of examining the eyes of all over middle age under a mydriatic, for the
state of the lens is the one sign of senescence which can be diagnosed in its
earliest microscopic stages with absolute certainty. A complete systemic
examination should exclude foci of infection or any constitutional disease
(diabetic, gastro-intestinal, nephritic, arteriosclerotic, and so on); apart
from the fact that an opportunity is afforded of establishing the presence of
such insidious affections and bringing their importance to the notice of the
patient, it is not unreasonable to suppose that their efficient treatment may
at least tend to delay the progress of the changes in the lens by improving
the general health and resistance. Not only should the health be considered,
but also the diet and the general habits. The diet should include an abund-
ance of fluid and a sufficiency of vitamins (green vegetables, carrots, lemon
juice, yeast, etc.). Habits in work, rest and recreation should also be
reviewed and the stresses of living should be so adjusted that they become
compatible so far as possible both with physical limitations and with the full
realization of life.
The third problem in the treatment of cataract is to mitigate as far as
possible the optical disadvantages under which the patient may be labouring.
Repeated and careful tests of the refraction every six months or so are
essential, as a progressive myopia and changes both in the strength and axis
of cylinders are the rule owing to refractive changes occurring in the lens.
Dazzling by bright light can be relieved by tinted glasses, and when the
maximum acuity is required at the same time, a tinting of the periphery of
the lens frequently gives considerable relief. Amber-tinted glasses
which cut the readily diffused short rays are perhaps the most generally
useful. Moreover, for close work efficient arrangement of the illumination
DISEASES OF THE LENS 3.18.1
is of the utmost value : it should be brilliant if the pupillary area is free,
duller and placed to the side and behind if the opacities are central. The
contrast of using a sheet of dark paper so cut that it isolates a few lines of
print at a time may also be of considerable assistance. Telescopic glasses
will allow some people to read who would otherwise be unable to do so ; and
a magnifying glass held in the hand or supported on the page will give
similar aid to those on whom it seems desirable to postpone an operation.
In cases of axial cataract, dilatation of the pupil by atropine over any
length of time for visual purposes should be avoided in elderly persons unless
a tonometric and perimetric record can be regularly undertaken to exclude
an insidious glaucoma : after middle age where there is any doubt on this
Question an optical iridectomy is a safer procedure.
At the same time it is desirable to sustain by every means the patient’s
morale and dispel his anxieties. The term cataract has an unfortunate
connotation, and while it should never be used by the ophthalmologist in
the many cases of stationary opacities, and rarely in the case of progressive
opacities until they begin to become embarrassing to vision ; when it must
be spoken of, stress should be laid on the fact that the condition is curable,
that blindness will not ensue, that the ordeal of an operation is much less
in realization than in anticipation, and that the operative results are good.
Operative Treatment. The indications for operation vary in each
particular case. In general terms, the safest time is certainly when the
cataract is mature. In practice, however, in bilateral cases once a patient
reaches the stage when he can no longer get about comfortably, when he
cannot read, write and carry on his usual occupation, one eye should be
operated upon : he should never be allowed, without some very special
reason, to drop his usual activities and sink into listlessness. The immaturity
of a cataract should present no real difficulty to the skilful operator. The
most advanced eye should therefore have been operated upon and become
useful before the better eye has become too blind to be serviceable. In
senile cases if the other eye still has useful vision it is usually wisest to
operate when the cataract reaches maturity unless, either from age or other
disease, there is reason to believe that the good eye will outlast the patient ;
in this case the dangers of hypermaturity are avoided, some field vision is
obtained on the blind side, and the disadvantages of an operation at a still
more advanced age are avoided. In such cases, also, provided the patient
has stood the first Operation well, and unless he is frail and inactive, it is
usually advisable to operate on the second eye at a later date, both to
obtain the advantages of binocular vision and to insure against the effects of
injury or disease of the first eye, a relatively common occurrence in the aged
which would necessitate an operation in less advantageous circumstances.
In the case of young persons or those with a normal lens in the other eye,
the advisability of operation is rendered more questionable by the possible
annoyance of subsequent diplopia which may cause considerable embarrass-
3.182 TEXT-BOOK OF OPHTHALMOLOGY
ment by reason of the large blurred image produced even when the operated
eye is uncorrected by glasses. This consideration should therefore always
be discussed, and the impossibility of attaining binocular vision should be
stressed unless through the wearing of a contact glass. It is probably best
in most cases to leave such a unilateral cataract alone unless the patient’s
calling demands a wide visual field or unless the affected lens shows signs
of progressing to a stage of hypermaturity. The value of a cosmetic
operation is hardly a medical one and should be decided by the patient.
With these considerations in mind, provided the patient is in reasonably
good health, and after sources of auto-intoxication or focal sepsis have been
eliminated, and when it has been established that the lacrimal passages are
healthy and the conjunctival sacs contain no pathogenic Organisms, if a
detailed examination of the eye reveals no other signs of active disease
(keratic precipitates, etc.) and indicates that the visual function of the
retina is good," operation can be embarked upon. There are few absolute
contra-indications. Severe focal sepsis, particularly dental infection which
cannot be eradicated, should give pause. Diabetes, as the condition which
gives rise to the largest crop of post-operative complications, particularly
those of haemorrhage and infection, is no absolute contra-indication, but
should receive adequate treatment ; while high blood-pressure cannot be
ignored. When the blood-pressure is extremely high it is well to reduce it a
little time before, and perhaps to precede operation by a venesection to avoid
the calamity of an expulsive haemorrhage.” The physical ability of the patient
to remain reasonably quiet should also receive consideration, while the
mental capacity to withstand the anxiety of the ordeal is also of importance.
The various operative techniques with their advantages and dis-
advantages will be discussed in the section of operative surgery. Briefly,
in the young a complete absorption of a cataract follows discission. In
adolescents this has frequently to be followed by a curette evacuation ;
and in adults an extraction of the lens is required. In the last case the most
popular methods are either an intra-capsular extraction by one of the various
techniques employed, or an extra-capsular extraction followed usually by a
discission of the secondary membrane. With the intra-capsular operation the
Question of maturity does not arise, but if the cataract is immature and
the extra-capsular method is employed, a preliminary discission of the
lens may be indicated. The method most suitable is a matter depending
upon the nature of each individual case and the experience and skill of the
surgeon. It is certainly the case that an intra-capsular extraction usually
gives the most dramatic results if technical complications do not arise, but
it is to be borne in mind that it demands skill in the selection of cases * and
1 p. 3176. * p. 21 13.
* The advisability of performing a preliminary iridectomy in certain cases should be con-
sidered, but although it may facilitate the subsequent extraction, the indications for subjecting
the patient to two operations instead of one are relatively few. It may, as we have seen,
prolong useful vision if the cataract is central ; but the operation rarely hastens maturity.
DISEASES OF THE LENS 3 183
a long apprenticeship and a delicacy of technique not readily attained, SO
that the spectacular results obtained in the hands of its various exponents
are not always equalled by those of more average dexterity and less experi-
ence who follow in their footsteps. There is no doubt that the method of
most general application is the older procedure of extra-capsular extraction,
even although it has to be prefaced in certain cases by a preliminary discission;
the merits of the later operations lie not so much in standardizing a new
technique as in putting forward fresh points of view in the light of which each
individual may orientate and model the technique which suits him best.
However that may be, in uncomplicated cases, with good surgery and a
reasonably tractable patient, the results are excellent, and apart from
the loss of accommodation and the limitation of the field, the intelligent
patient is little inconvenienced. -
The optical condition of aphakia has already been described," and its correction
by lenses will be discussed in the subsequent volume.
Complications, of course, present difficulties. Thus with nuclear
sclerosis in the aged and frail, it may be advisable merely to be content
with the increased vision following an iridectomy. If, because of local
infective disease, severe chest trouble or great physical or mental debility,
any operation by the open methods is considered inadvisable, the advantages
of couching, which should give good vision for a period varying from 3 to 7
years, should not be forgotten. Finally, mechanical complications such as
a tremulous or dislocated lens, iridic adhesions, inflammatory membranes
or the presence of glaucoma will be considered in the section on surgery.
Angelucci. A. di Ott., xxxiii, l, 1926. Hildersheider. XIII Internat. Cong., Amster-
Badal. Bull. S. fr. d’O., xix, 422, 1902. dam, i, 216, 1929.
Biffis and Quaglio. Am. di Ott., lxi, 642, 1933. Jackson. Am. J. O., vii, 775, 1924.
Burdon-Cooper. T. O. S., liii, 401, 1933. Kirby. A. of O., v, 754, 1931.
Cohen and Levin. Rev. Cub. de Oft., ii, 457, Kögel. A. f. O., cxxvi, 502, 1931.
1920. Levin. Am. J. Roentg., vii, 107, 1920.
Daniels. Z. f. Aug., lxxv. 129, 1931. Löhlein. A. f. Aug., lxv, 417, 1910.
Davis. T. Am. O. S., xxii, l l 2, 1924. Meyer-Steineg. W. f. Ther. u. Hyg. d.
A m. Med., xxvii, l, 1932. Auges, xvi, 377, 1913; xvii, 221, 229,
Dean. T. Am. Acad. Oph. Oto-Lary.., xxix, 1914 : xix, 153, 1916.
131, 1924 ; xxxii, 231, 1927. Deut. med. W., l, l l 1, 1924.
Dor. La Clim. Oph., xvii, 11, 1911. Pagenstecher. K. M. Aug., xxxv, 109, 1897.
Ellis. A. of O., lvii, 46, 1928. v. Pflugk. K. M. Aug., xliv (2), 400, 1906.
Franklin and Cordes. Am. J. O., iii, 643, 1920. Pick. Z. f. Aug., xxi, 302, 1909.
Gallus. A. f. Aug., xcii, 34, 1923. Pollock. Glasgow Med. J., xcix, 32, 1923.
Gilbert. Z. f. Aug., liii, 343, 1924. Rauh. B. O. G. Heidel., li, 375, 1936.
Greenwood. T. Am. Acad. Oph. Oto-Lary. Römer. A. f. Aug., lvi, Erg., 150, 1907.
xxix, 125, 1924. B. O. G. Heidel., xxxv, 195, 1908; xxxvi,
Harkness. Am. J. O., viii, 132, 1925. 97, 1910.
Harris. A m. J. Electrother..., xlii, 226, 1924. Deut. med. W., i. 284, 553 ; ii, 1423, 1909.
In complicated cases it may be used as a test of the reaction to the eye to operative interference.
thus indicating the advisability of embarking upon further major procedures ; and in an old
irido-cyclitis it may be of value in breaking down extensive adhesions. Its greatest indication.
however, is in the presence of increased intra-ocular tension, in which case a considerably greater
margin of safety is provided than if the combined operation were performed at one sitting
(see ‘‘ Operative Surgery ''). -
1 Vol. I, pp. 738 (748), 774 (784).
T. (). —WOL. III.
31.84 TEXT-BOOK OF OPHTHALMOLOGY
Salus. Med. Kl., xxii, 1787, 1926. Tischner. W. f. Ther. u. Hyg. d. Auges, xvii,
Schön. A. f. Aug., xvii, 1, 1887; xix, 77, 301, 1914.
1889, Treu. A. f. Aug., lvii, 56, 1908.
B. O. G. Heidel., xv., 170, 1889. Uhthoff. D. med. W., 1, 899, 946, 978,
Selinger. A. of O., xiv, 244, 1935. 1924.
Shropshire. A. of O., xvii, 505, 508, 1937. Verdereau. La Clin. Oph., x, 358, 1904.
Siegrist. Der Graue Alterstar, Berlin, 1928. Walter. W. f. Ther. w. Hyg. d. Auges, xvii,
Smith. La Clin. Oph., iv, 234, 1912. 306, 313, 1914.
T. O. S., xlviii, 89, 1928. Weeks. J. Am. Med. As., xciv, 403, 1930.
Taylor. Lancet, cevi, 700, 1924. Yudkin. Am. J. O., xxi, 871, 1938.
Clinical Types of Cataract
A. Evolutionary Lenticular Opacities
Under this heading we propose to group a number of clinical conditions
which differ in aetiology, pathology, symptomatology and clinical course
from pathological cataracts properly so-called. They are all developmental
in nature, made up of minute, limited, discrete opacities, which, although
visible on oblique illumination, are frequently so small as to disappear in
transmitted light, rarely so gross as to cause visual symptoms, and to a
large extent are stationary and non-progressive in type, and call for no
treatment. Only exceptionally is vision impaired and the opacities multiply
as life proceeds. These conditions cannot be called physiological, nor can
they be classed with progressive cataracts of a pathological nature of which
they do not even form a premonitory symptom.
In so far as these opacities are circumscribed, discrete and show little
or no tendency to develop, they resemble the congenital types of lens
opacity which we have already considered in a previous section.” Such
opacities, indeed, may appear at all stages of growth, and it will be
remembered that the lens continues to develop until adult life. The date of
their appearance determines their site : they are represented in pre-natal
development by punctate nuclear cataract, floriform cataract, sutural
cataract, and so on. While similar in their clinical appearance and pathology,
the developmental opacities appearing in post-natal life differ from those
of ante-natal life only in their site ; they represent the result of the same
type of process, a failure in development or degeneration of lens fibres,
occurring, however, after birth and therefore, instead of affecting the central
area, appearing in the infantile or adult nucleus or in the cortex. Their
persistence is due to the fact that the lens is what may be designated as a
“ permanent organ ” (v. Szily, 1938) because it consists of imperishable
elements, so that any disturbances in the course of development, no matter
how trivial, may give rise to permanent changes.
PUNCTIFORM OPACITIES (BLUE-DOT CATARACT)
Small punctiform opacities scattered irregularly in the adult nucleus
and cortex are so common as almost to be considered physiological (Fig. 2704).
* Vol. II, p. 1364.
DISEASES OF THE LENS 3.185
Usually they are so minute and fine as to be invisible with the ophthalmoscope
and not to interfere in anyway with the view of the fundus; by oblique illumina-
tion with the loupe or the slit-lamp, however, they appear as fine bluish-white
spots, presenting, when numerous, an exceedingly beautiful picture.
Clinically they never interfere with vision, neither do they show any
tendency to development. They correspond to the cataracta centralis
pulverulenta of the central area or punctate sutural opacities - which are of
pre-natal development.
The bright blue colour, which suggested the name CATARACTA CAERULEA or
BLUE-Dot CATARACT, is a physical phenomenon depending on the dispersion of light.
In any opalescent medium minute particles disperse the light irregularly inversely as
the fourth power of the wave-length, so that the major part of the dispersed light is
made up of the short blue and violet waves. The cataract is blue, therefore, for the
same reason as, owing to dispersion by the atmosphere, the sky is blue.
Other more prominent opacities occur after birth in the later stages of
development. It will be remembered that the FLORIFoRM CATARACT *
described by Koby (1922) with its peculiar annular elements may occur
in the adult nucleus and in the cortex; similarly the speaR-CATARACT *
described by Vogt (1922) may affect the outer layers of the lens. Two
further types which affect the outer layers must be considered—DILACERATED
CATARACT and coron ARY CATARACT, the former occurring in the superficial
layers of the infantile and the deeper layers of the adult nucleus, and the
latter not appearing until puberty and affecting the superficial layers of the
adult nucleus and the deeper layers of the cortex.
DILACERATED CATARACT
This type of opacity, described originally by Vogt (1922), shows a
predilection for the axial parts of the lens, lying
in the superficial zone of the infantile and the
deep zone of the adult nucleus; it is therefore
congenital or develops soon after birth. The
opacities have a peculiar appearance like pieces
of teased-out moss filled with holes and having
combed-out edges (Fig. 2703). They are very
fine and thin and are confined within one layer
so that they show blue or green reflexes by direct
illumination. They rarely occur alone and are Fig. 27.03.-DidaceRATED
- - --- OPACITY.
usually associated with punctate opacities and
with coronary cataract, but they show no tendency to evolve.
* Vol. II, p. 1369. * Vol. II, p. 1371.
* Vol. II, p. 1639. * Vol. II, p. 1371.

4 a 2
3.18.6 TEXT-BOOK OF OPHTHALMOLOGY
CoRo NARY CATARACT
This type of developmental punctate cataract appears usually just
after puberty. The older writers considered it a rare form, but after the
Fig. 27.04.-Coronary Cataract.
(Foster and Benson, T. O. S.).
advent of the slit-lamp its common occurrence was noted by Vogt (1917–18)
who found it present in 25% of persons; from the crown-like arrangement
Fig. 2705-Advanced Coronary and
Puxtºn ATE Opacities.
of the opacities he termed the condition
coBox ARY CATARACT (Figs. 2704–05). His
observations have been amply confirmed
(Weissenbach, 1917; Krenger, 1918;
Horlacher, 1918; Gjessing, 1920; and
others). The opacities are arranged as
a regular corona in a zone in the outer
layers of the adult nucleus and the inner
layers of the cortex, the axial region and
the extreme periphery remaining free:
quite frequently, therefore, they are
behind the iris and do not become appar-
ent until the pupil is dilated. They
are of all shapes, the most common
being a club-shape with the head of the


DISEASES OF THE LENS
31.87
club pointing centrally, but punctiform, ring-shaped and disciform types
are common, while they vary in colour from white to grey, yellow, brown,
red, or blue : the latter appearance is due to the same
optical effect as we have discussed in blue-dot
cataract. They may be associated with punctate or
nummular opacities scattered throughout the axial
region of the lens, for they differ from other develop-
mental punctate opacities only in the lateness of their
appearance in life and not in their nature. Like
these they are non-progressive, or so slowly pro-
gressive as never to lead to complete opacification
of the lens; in elderly people the regular process
of senile cortical cataract must be superimposed upon
them if a complete opacity is to develop.
The anatomical basis of punctate cataract of the
pre-senile type and coronary cataract is round, ellip-
tical or fusiform lacunae between the fibres in the
deeper layers of the cortex (Hess, 1893) (Fig. 2707).
These spaces are filled with homogeneous or finely
Fig. 2706.-Corton ARY
CATARACT.
(Foster and Benson,
T. O. S.).
granular coagula which stain deeply with haematoxylin, while the fibres
themselves and the entire nucleus remain normal.
Fig. 2707.-PUNCTATE LENTICULAR OPACITIEs (Parsons).
In considering the aetiology of these juvenile and pre-senile cataracts two factors
must be remembered—their possible relation to tetany and to the endocrine glands.
We have seen 1 that tetany, both in its latent and in its manifest forms, is frequently
p. 3154.


3.188 TEXT-BOOK OF OPHTHALMOLOGY
associated with cataract, and a considerable body of opinion holds that in its latent
form it is common. The mechanism of the injury to the lens, whether chemical or
toxic, is a matter of controversy," but arguments can be put forward that some cases of
zonular cataract are due to a temporary condition of infantile tetany, and many cases
of sub-capsular opacities to a juvenile tetany. Some writers, indeed, (Peters, 1901–05;
Hesse and Phelps, 1913; Fischer and Triebenstein, 1914; Greppin, 1922; Romanowa,
1934; and others) ascribe between 50% and 80% of pre-senile cataracts to this cause.
Others again invoke an endocrine origin, Fischer-Galati (1930), for example, ascribes
a dominant influence to the seasual glands. He prefers to call coronary cataract
“ puberty cataract,” considering it due to a deficiency of the interstitial cells associated
with some degree of impotence and lack of sexual desire ; the superposition of senile
cataract in old age he associates with complete inactivity of these cells ; and the appro-
priate treatment, stimulation of the cells by unilateral vaso-ligation. Nothing approach-
ing conclusive evidence, however, has been advanced in support of either of these
hypotheses.
Fischer-Galati. Clinique, xxv, 119, 1930. Krenger. K. M. Aug., lx, 229, 1918.
Fischer and Triebenstein. K. M. Aug., Peters. Z. f. Aug., iv, 337, 1900; v, 89, 1901.
xxvii, 441, 1914. K. M. Aug., xliii (1), 621, 1905.
Gjessing. K. M. Aug., lxv, 233, 1920. Romanowa. A. f. O., czzxiii, 142, 1934.
Greppin. Schw. med. W., lii, 1260, 1922. v. Szily. T. O. S., lviii, 595, 1938.
Hess. A. f. O., xxxix (1), 183, 1893. Vogt. K. M. Aug., lviii, 579, 1917.
Hesse and Phelps. Z. f. Aug., xxix, 238, 1913. Z. f. Aug., x1, 123, 1918.
Horlacher. Z. f. Aug., x1, 33, 1918. A. f. O., cvii, 196, 1922.
Roby. A. d’O., xl, 492, 1922. Weissenbach. K. M. Aug., lix, 527, 1917.
B. Senile Changes
Senile changes of some degree in the lens, as in all other tissues, are of
universal occurrence and must be considered physiological, their intensity
and the date of their onset being determined partly by hereditary and
constitutional factors and partly by the stresses imposed by environmental
conditions. We have already seen that the growth of the lens proceeds
regularly and .asymptotically until maturity is reached, whereafter senes-
cence sets in when catabolic processes exceed anabolic activity until, when
it becomes sufficiently advanced, it ends in death. In the lens these
phenomena in general run parallel with evidences of senescence in the other
organs of the body (Steinhaus, 1932; Berstein, 1934), and are strictly
comparable to the similar cycle seen particularly well in all epithelial tissues,
such as the skin or the hair ; but they are readily noted in the lens because
of its transparency, and they become rapidly cumulative since this tissue
cannot exfoliate but must retain its débris.
These changes may be divided into three categories : physiological
changes of senescence which are universal in age, pathological senile
changes which form the precursors of cataract formation, and the various
types of senile cataract.
1 p. 3156.
DISEASES OF THE LENS 31.89
PHYSIological CHANGEs of SENESCENCE
The principal physiological changes of senescence apparent in the ageing
lens are increased sclerosis, particularly of the nuclear part, and the formation
of relief on the internal surfaces of optical discontinuity.
PHYsiological sºn ILE scLERosis. The gradual sclerosis of the nuclear
area of the lens may be considered physiological so long as the tissue remains
transparent. The oldest fibres, which become gradually pushed into the
central region by the development of the younger cortex, slowly become
more condensed and inert, a process which goes on gradually and con-
Fig. 2708. RELIEF of ADULT NUCLEU’s IN THE SENILE LENs,
tinuously throughout life. Anatomically, these retrogressive changes are
associated with loss of the nuclei of the fibres and their compression into a
homogeneous rubbery mass; chemically, they are accompanied by a loss of
water, a replacement of soluble by insoluble protein, and a deposition of
inert materials; physiologically, permeability is diminished, oxidative
activity is lost, and metabolism fails. Clinically, the increase in the internal
dispersion of light from the hardened and compressed elements leads to an
increased optical density so that the lens becomes opalescent and takes on a
grey or yellowish colouration, for the short waves are largely dispersed and
only the longer ones are transmitted. Functionally, although the visual
acuity may be little impaired, the increased consistency diminishes accom-
modative adaptability and results in presbyopia; while the increased
density of the central region leads to the development of lenticular myopia,

3190. TEXT-BOOK OF OPHTHALMOLOGY
a tendency, however, usually compensated by the increased flattening of
the lens as a whole with age."
THE ForMATION of NUCLEAR RELLEF. The peculiar modelling of the
surfaces of optical discontinuity of the nuclei of the lens is clearly visible
only with the slit-lamp. It may become distinguishable soon after 20 years
of age, but is very common after 50 (Vogt, 1919; Vogt and Lussi, 1919).
The anterior surface of the adult nucleus shows the phenomenon most
clearly, particularly in the axial region where the sutures form prominent
branching projections throwing shadows on oblique illumination and marking
out the design of the lens fibres, while rounded protuberances, presumably
vacuoles, are frequently present (Fig. 27.08). It is to be noted that this
optical effect is not derived from a single internal optical surface but some-
what diffusely from a zone of varying thickness (Goldmann, 1937).
Occasionally a similar phenomenon is seen on the posterior face of the
adult nucleus, but here, of course, the relief is reversed; and as a rarity
a more simple and less embossed pattern is seen on the anterior surface of
the foetal nucleus.
Neither of these senile phenomena impair the function of vision ; nor
are they necessarily a prelude to the development of cataract.
PATHological SENILE CHANGEs
A group of senile changes must now be considered which cannot be
called physiological; they may, however, remain without progression
indefinitely, although the eventual frequent super-
imposition of cataract upon them justifies their being
looked upon as forerunners of this condition. They
are all indicative of hydration of the cortex and are the
expression of the accumulation of fluid which is probably
largely derived from without, but may partly be
abstracted from colloid combination in the fibres.” This
fluid may collect in globules and vacuoles, or forcing
itself between the structures of the lens, may make great
clefts in a radial direction or force apart the sutures, or
separating the radial lamellae, it may cause innumerable
lines of cleavage between them.
VACUole, FoRMATION. Isolated vacuoles filled with
º fluid occurring either under the capsule or in the interior
* Lººs. of the lens, particularly on the surface of the adult
sub-capsular and nucleus, are not infrequent, and carry no grave prog-
º ** nostic significance (Fig. 2709). They occasionally occur
in young people and may remain stationary for years;
in age they tend to multiply; and in early cortical cataract they may
appear in enormous numbers under the capsule forming a veritable carpet.
* Vol. I, p. 126. * p. 31.17.

DISEASES OF THE LENS 3.191
THE ForMATION OF CLEAR CLEFTs. The formation of clear radial clefts
(Wasserspalten fissured cataract) is of graver prognostic significance
for the development of cataract, a fact which was noted by the earlier
Fig. 27.10. Fig. 2711.
FIGs. 2710–11.-CLEAR FLUID CLEFTs.
Fig. 27.10 in focal light : Fig. 2711 by the plane mirror.
observers (Becker, 1883; Magnus, 1890). Such spaces rarely appear before
the age of 50, but begin to get relatively common thereafter (Vogt, 1921;
Pfeiffer, 1921). They occur most frequently in the cortex, usually just
underneath the capsule, but occasionally they penetrate into the adult
Fig. 2712.-CLEAR FLUID CLEFTs. Fig. 27.13.−CLEAR Fig. 2714.-FLUID
FLUID CLEFTs. CLE Fºrs w IT H
G R A N tº L. A. R.
In optical section
with the slit-lamp.
DEBRIs.
nucleus; Pfeiffer (1921) found that they usually occurred in the anterior
cortex (53%), quite frequently in the anterior and posterior cortex (42%)
and only rarely in the latter alone (5%). They may occur anywhere in the
lens, but as would be expected, they have a predilection for the sutures
which appear to gape (dehiscence of the sutures), a phenomenon readily seen in
the isolated lens on maceration (Vogt, 1914; van der Heydt, 1930) (Fig.2712).
Although they are quite transparent in transmitted light, being filled with


3.192 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2715,-LAMELLAR SEPARAtion.
Seen by diffuse oblique illumination.
a homogeneous fluid, in focal illumination they appear dark or black because
of the lack of optical density (Figs. 2710–11), and in the optical beam of the
slit-lamp they appear as irregularly angulated spaces (Fig. 27.13). As
cataractous changes develop, however, they become filled with débris and
droplets of myelin, so that they appear opaque (Fig. 2714).
LAMELLAR separation. This is a senile phenomenon which begins to
Fig. 2716– LAMELLAR SEPARATIox. Fig. 2717–LAMELLAR SEPARAtion.
Surface view of anterior cortex with Optical section (Harrison Butler).
slit-lamp.


DISEASES OF THE LENS 31.93
appear usually in the anterior cortex after the age of 50 (in 7% of lenses)
and is found in 50% of people above 80 years (Schild, 1921); it is intimately
associated with early cortical cataract, evidences of which are usually
present. It represents a separation of the radial lamellae in a series of fine
parallel lines, usually occupying a whole sector of the lens, running upwards
and outwards from the infero-nasal region, but sometimes forming two or
more systems in separate bands cutting each other at right angles (Vogt,
1914–19) (Figs. 2715–17).
SENILE CATARACT
Senile cataract is very common, and may indeed be said to be the rule
in advanced age. Thus Horlacher (1918) found that 65% of all persons
between 51 and 60, and Barth (1914) that 96% of those above 60 years of
age have some lenticular opacities visible with the slit-lamp ; but it is to
be remembered that the majority of these are extremely slowly pro-
gressive, and the proportion of cases in which the process becomes so
advanced before death as to cause visual disability is comparatively small.
Two estimates of the incidence of lens changes in the various age-groups,
One of them taken from Scandinavia and the other from America, are as
follows:–
70 + - 92. ()
Age | Andersen (1925) Gradle (1926)
4 1–50 38-29, h 34.1%
51–60 65. 1 66-2
61–7() 85. () 68-4
90.9
|
There can be no doubt that the early and rapid development of cataract
has a hereditary tendency, a feature common to all the phenomena of senes-
cence, but we are ignorant as yet whether the cataract itself or the influences
which determine it are genetically conditioned. In a previous volume we
have already seen the extraordinary hereditary influence in many types of
congenital lenticular opacities ; juvenile and pre-senile opacities have the
same tendency (Nettleship, 1909–12 ; Rowan and Wilson, 1921 ;
Halbertsma, 1928). This influence may not be so potent in senile cataract,
but it was sufficiently apparent to attract the attentions of so early an
observer as Belivier (1818), who noted its preferential incidence in a family
over a period of 100 years. In the subsequent literature there are numerous
reports of families showing a high incidence over several generations
(Bastard, 1850; Dyer, 1850; Green, 1890; Nettleship, 1905–12; Casey Wood,
1906; Parker, 1916; Vogt, 1918; Garfunkel, 1926; Moretti, 1931; Bücklers,
1936; Biro, 1936; and many others). Thus Nettleship collected 122 families
3.194 TEXT-BOOK OF OPHTHALMOLOGY
of which 375 persons comprising 145 males (38%) and 230 females (62%)
had advanced cataract. The heredity shows a direct, continuous and dominant
transmission, either sex transmitting but the female sex showing the highest
incidence. It is interesting that many genealogies show, although with
exceptions, a tendency for the cataract to appear at approximately the same
age in one generation, and to develop at an earlier age in succeeding genera-
tions. This phenomenon of anticipation has been remarked by several
observers, as if a cataract which in the first generation had a senile develop-
ment, appeared in subsequent generations in earlier adult life, and eventually
9 *
Age |
Of
Com- | | | | | | | | | | | |
º: Óðöööösöööö's see
IOleIll). + + +
| | | | | |
40-50 ~6 * & & CŞ • CŞ.


|
&
20-30 yrs. & |
O
+

8 & 83 & 8 & & 6 & 8
+ + + + + +
TT
O O O
+ + +
O
i
6-9 yrs. & (3) & O
-H
FIG. 2718.-HEREDITY OF CATARACT.
Showing anticipation (Green).
had a juvenile and ultimately a congenital incidence (Becker, 1883;
Appenzeller, 1884; Jeaffreson, 1886; Green, 1890; Norrie, 1896; Nettle-
ship, 1909; Andrassy, 1921 ; Vinsonhaler and Cosgrove, 1936) (Fig. 2718).
The clinical forms in which senile cataract may appear are numerous,
and although arguments may be put forward against their differentiation
into separate categories, and despite the fact that different forms may
appear simultaneously in the two eyes and that frequently more than one
form is seen together in the same lens, it is convenient for purposes of
description to study them separately. They may be divided into two large
classes, nuclear and cortical : some 20%–25% are nuclear and the majority
are cortical in type. Nuclear cataracts vary only in the degree of sclerosis,
being either uniform over the whole central zone, or affecting the foetal
DISEASES OF THE LENS 3.19.5
nuclei when a double-focus lens results, or becoming extreme in degree and
extent, in which case the rare condition of brown or black cataract results.
Cortical cataract may be divided into three main types which differ in their
site and evolution-cuneiform, in the peripheral cortex; punctate peri-
nuclear; and cupuliform in the posterior cortex.
The relative incidence of these types has been variously assessed. Knapp (1916)
estimated nuclear cataracts at 1-5% of the total, cuneiform at 33%, peripheral equa-
torial opacities at 50%, and posterior cortical at 6.3%. Kirby (1932) found 29%
nuclear cataracts, 52% in the peripheral cortex and 3.5% in the posterior cortex,
with a mixture of nuclear and cortical in 9%, nuclear and posterior cortical in 4%, and
peripheral and posterior cortical in 2.5%. Foster and Benson (1934) found 22%
nuclear, 37% cuneiform, 17% punctate, 6% cupuliform, and 18% mixed in which
category the nuclear cataract predominated. They also found that the cupuliform
type occurred earliest with a maximum incidence between 50 and 60 years, cuneiform
and punctate between 60 and 70, and nuclear between 70 and 75. Over most parts
of the world the average age for operation is 65 years (Gradle, 1930).
1. NUCLEAR CATARACT
(HARD CATARACT)
A nuclear cataract, as we have seen, is merely the intensification of the
physiological change of sclerosis which has progressed to such a degree that
|
Fig. 2719.-NucLEAR CATARAct.
Seen by oblique illumination (Foster and Benson, T. O. S.).

31.96 TEXT-BOOK OF OPHTHALMOLOGY
vision becomes affected (Figs. 2719–20), a process which usually begins about
the age of 50 and reaches a maximum incidence between 70 and 75 (Foster
and Benson, 1934). There is a diffuse
increase of the optical density of the
nuclei beginning in the deeper layers of
the foetal nucleus and spreading gradually
in a peripheral direction to include the whole
adult nucleus : the Y-sutures and the
nuclear relief are particularly obvious. The
change is a diffuse one, for any circum-
scribed, sharply defined opacities in this
central area must be of congenital origin.
With transmitted light the senile yellow or
orange colouration of the lens is partic-
ularly marked owing to the preferential
dispersion of the shorter waves of light.
Such a sclerotic process is a gradual one
showing no sudden changes and is usually
extremely slowly progressive, and is not
associated with the appearance of vacuoles,
Slit-lamp section (Foster and clefts, lamellar separation or any evidences
Benson, T. O. S.). of hydration of the lens. It is true that
cortical changes are often superimposed
upon the nuclear sclerosis, in which case the latter process tends to hold
the former in check and delays the development of the soft cataract.
- -
*
-
º
Fig. 2720. Nuclean Cataract.
-
-
Fig. 2721–H and CATAR Act.
Showing induration of the whole lens (v. Szily, T. O. S.).
Pathologically the lens shows exactly the opposite changes to those
which characterize cortical cataract (Fig. 2721): there is a dense uniform
induration of the affected area which assumes a rubber-like consistency, the


















DISEASES OF THE LENS 31.97
whole mass of hardened and compressed fibres showing a regular concentric
lamination with only sporadic inter-fibrillar spaces and displaying a resist-
ance to all histological stains (Becker, 1883; v. Szily, 1938; and others).
On the whole, therefore, the lens retains its anatomical integrity, suffering
a change in certain physiological and physical attributes, particularly its
refractive index and density. Chemically it differs completely from cortical
cataract, since it shows a diminution of its water content and an increase of
its solids (Gifford and Puntenny, 1933), together with a large increase of
potassium compared with calcium (11 : 1, Burdon-Cooper, 1933). Clinically
the fundus may be seen although it is generally considerably distorted ;
and the main visual symptoms are a slow and gradual diminution of visual
acuity especially in bright light, a distortion of objects, and a progressive
myopia.
Two special types deserve separate notice ; in the first the sclerosis is
limited to the foetal nucleus, and in the second it progresses to involve
practically the entire lens and is associated with marked colouration.
LENS WITH DOU. BLE FOCUS
This condition, although noted by L. Müller (1894), was first described
by Demicheri (1895) under the name of FALSE LENTICONUs, and it has been
variously called FALSE CATARACT (Halben, 1903), CENTRAL HYPER-REFRIN-
GENCE (Koby, 1930) or LENs witH DOUBLE FOCUs (v. Szily, 1903). Patho-
logically the process is a simple sclerosis, but it is limited to the foetal
nucleus : the central interval of the embryonic nucleus is obliterated and
the entire foetal nucleus becomes homogeneous with the Y-sutures alone
distinguishable. Its limits are well marked and are separated from the
adult nucleus by a dark space (Vogt, 1923); but eventually this interval
disappears and finally the whole adult nucleus becomes homogeneously
sclerosed.
The most arresting clinical feature of this type of cataract is the
development of a double refraction, the axial area being highly myopic
while the peripheral part remains hypermetropic. The difference between
the two may be very considerable, differences in refraction of 10 or
12 dioptres being frequently noted, while one of 27 dioptres has been
recorded (Donders, 1888 ; Guttmann, 1898; Hess, 1911; Kistler, 1928;
Gaiser, 1928; and others). Demicheri at first thought that the disparity
between the two regions was due to cortical changes involving a peripheral
hypermetropia, but the slit-lamp has demonstrated without doubt that the
cause is the increased optical density of the nucleus, an effect enhanced by
the fact that the curvature of the foetal nucleus is considerably greater than
that of the lens. Occasionally, also, the refractive effect of the optical
surface thus created may be intensified by the development of a true internal
lenticonus (Moeller, 1927). .
31.98 TEXT-BOOK OF OPHTHALMOLOGY
BLACK CATARACT (CATARACTA BFUNESCENS OR NIGRA)
As the process of senile sclerosis proceeds it gradually spreads towards
the periphery of the lens, and although it does not actually reach the capsule,
so that some cortical substance always remains, it may eventually occupy
practically the entire tissue. In this event the colour of the lens gradually
changes from the yellow appearance of nuclear sclerosis, to amber, reddish
brown, and eventually it becomes almost black (Figs. 2722–24, Plate LXIII).
This occurrence which was called CATARACTA NIGRA by Wenzel (1788) and
CATARACTA BRUNESCENs by Becker (1883), is relatively rare (23 out of 6,206
cataracts, Mahieu, 1922). The clinical appearance is peculiar, for although
the pupil appears black on oblique focal illumination, the fundus reflex is
absent, a circumstance which makes the condition resemble the effect of
a massive haemorrhage into the vitreous body.
The origin of the colour in black cataract has excited a considerable amount of
controversy, and several explanations have been offered.
(1) That it is a purely physical phenomenon dependent upon an increased refractive
indear, the yellow colouration of the senile lens gradually passing through red-brown
to black by gradations of the same process (Hess, 1905 ; Rollet and Bussy, 1921 ;
Busacca, 1925; Magnasco, 1929; Carmi, 1930; Vogt, 1931).
(2) That it was due to the presence of blood-pigment was an early view (v. Graefe,
1854); but that this hypothesis is wrong has been shown by the negative results
obtained in the examination of numbers of lenses both by chemical tests (Hess, 1905;
Speciale-Cirincione, 1906; Rollet and Bussy, 1921 ; Busacca, 1925; Magnasco, 1929;
Carmi, 1930; Neuschüler, 1930; and others), or by spectroscopic analyses (Elschnig
and Zeynek, 1913; Herzfeld, 1922; Bücklers, 1930; Mawas, 1930; and others).
(3) The presence of bile pigments has been suggested, but spectroscopic analyses
for these have been negative (Herzfeld, 1922).
(4) The possibility of lipoid substances has been considered, but pigmentation
from them has been discounted (Busacca, 1925).
(5) That it is due to the presence of melanin or a melanin-like substance has long
been a favourite hypothesis (McHardy, 1882). Alt (1900) concluded that the melanin
must have entered the lens from the pigmentary epithelium, but the impenetrability
of the capsule to large-sized molecules rules this origin out of court. There remains the
possibility that it is formed within the lens from an unpigmented precursor. That this
might be tyrosine, which turns black in the presence of an oxydase, was suggested by
Gatti (1904) and supported by Michail and Vancea (1926–27), and that the process was
enzymic in nature, and due to tyrosinase, was suggested by Burdon-Cooper (1922)
who found that the cortex of ordinary senile cataract turned brown when treated with
a weak acid and exposed to light, a reaction which previous boiling of the lens inhibited :
it is known that tyrosine, leucine and cystine, all of which may be found in the lens,
form a melanin-like compound under the action of ultra-violet light (Krause, 1933).
A number of observers, however, have failed to find tyrosine in black cataract
(Neuschüler, 1930; Gifford and Puntenny, 1933); while Sauermann (1933), being
unable to identify tyrosinase in the lens, suggested an origin by the oxidation of free
tryptophane. Gifford and Puntenny (1933) found evidence of the presence of a ferment
in the lens capable of oxidizing dopa (the unpigmented precursor of melanin)," to melanin
* Vol. I, p. 374.
PLATE LXIII
(ATAR act
Fig. 2722-SENILE Nucle:AR ScLERosis:
RED CATARACT witH Corox ARY AND
SENILE PERiº Hºral ("HANGEs.
Fig. 2724.-SENILE Nuclear Fig. 2725-CUPuli-
ScLERosis : RED CATARACT. For M. CATARACT.
Fig. 2726.-EARLY DIABETIC Fig. 2727-CoMell- Fig. 272S.--Crystals
CATARACT. cATED CATARACT. IN LENs.
Punctate opacities and fluid Polychromatic lustre
droplets in cortex. at posterior pole.
To fuee p. 319s,




DISEASES OF THE LENS 31.99
and suggested that such a ferment acted on substances developed from the lenticular
proteins. A further suggestion was made by Ropshaw (1933), who found that a black
colour was produced by the interaction of cysteine and protamine, the first of which
could be derived from glutathione and the second from the disintegration of the
nucleoproteins in the central fibres; this theory received support from the experi-
mental work of Bellows (1935). Puscariu and Nitzulescu (1936), on the other hand,
found that the pigment they obtained did not show all the reactions of melanin but
corresponded to lipofuscin, a related pigment which accumulates in age in the cells of
heart muscle, the adrenal glands and the spinal ganglia, and multiplies considerably in
pathological conditions.
It must be admitted, since many authors have obtained a coloured
pigment from such lenses (Speciale-Cirincione, 1906; Elschnig and Zeynek,
1913; Sauermann, 1933; Gifford and Puntenny, 1933; Bellows, 1935;
Puscariu and Nitzulescu, 1936), that the cause of the colouration cannot be
physical. A pigment must be formed from the products of the slow proteo-
lysis of the lenticular proteins ; whether the pigment is melanin or lipofuscin,
or one of the related but little understood substances, is not yet clear. It is
arguable, of course, that the same substance in smaller concentration may
to some extent at any rate be responsible for the yellow colour of the senile
lens.
The treatment of the sclerotic type of cataract has popularly had a
reputation of danger, since some authors consider operation liable to be
followed by irido-cyclitis or infection owing to a peculiar obstinacy of the
cortex to absorb. The general experience, however, does not bear this out,
and if the precaution is taken to make a large incision in order that the
ample nucleus may be delivered easily, the operative prognosis should be
good.
2. CORTICAL CATARACT
(SoFT CATARACT)
In contrast to the sclerosis of nuclear cataract, the phenomena character-
istic of cortical cataract are those of hydration and intumescence. The
opacities are typically preceded by the pre-cataractous signs of hydration
which we have already studied : the formation of vacuoles, separation of the
lamellae by fluid, and water-splitting of the sutures to form clear clefts.
These processes are eventually associated with the development of an ill-
defined haze in the cortex, which in its initial stages may be a physical
oedema and therefore reversible, but eventually proceeds to denaturation and
coagulation of the proteins with the consequent formation of true opacities.
According to the nature and position of these opacities such cataracts may
be divided into several types.
T.O.-WOL. III. 4 B
3200 TEXT-BOOK OF OPHTHALMOLOGY
CUNEIFORM CATARACT
This is the most typical form of senile cataract in which the opacities
develop in the peripheral parts of the deep cortex, particularly in the lower
Fig. 2729.-SENILE Custºn-
Foºt CATARAct.
Seen by transmitted light.
segment, and run axially both anterior and
posterior to the nucleus forming radial spokes
aimed at the centre of the pupil. These, in
contrast to water-clefts, appear grey by oblique
illumination and black and opaque on trans-
illumination (Figs. 2729-31). Sometimes
these opaque spokes may be numerous, small
and discrete, and at other times they become
fused, losing their individuality to form great
plaques occupying a large sector of the
periphery. When they occur alone without
other changes and are sharply defined, their
progress may be exceedingly slow and they
may remain stationary for years, or one or two
elements may break into the pupillary region
or strike right across the lens. At other times, when they are associated
with much hydration of the cortex they may show a stormy progression;
in this event the appearance of clear clefts and lamellar separation is
followed by a cloudy swelling of the whole cortex which becomes swollen
Fig. 27.30–Cuxºtroºm Catan act.
Seen by oblique illumination (Foster and Benson, T. O. S.).


DISEASES OF THE LENS 320.1
- -
Fig. 2731.-CUNEIForM CATARAct. Fig. 2732–HYPERMATURE CATARACT.
Slit-lamp section (Foster and Benson, T. O. S.).
in a state of intumescence, a phase which is eventually followed by the
autolysis of the fibres and loss of fluid characteristic of the stage of hyper-
maturity (Fig. 2732).
Pathologically, in the stage of intumescence the most prominent feature
FIG. 2733.-SoFT CATARAct.
Intumescent stage (v. Szily, T. O. S.).
is the disintegration and splintering of the swollen lens fibres, and the
accumulation of their detritus as spherical and granular agglomerations in
the gaps and fissures between them (Fig. 2733). Beneath the lens capsule
there is sometimes a remarkable folding of the fibres, but ultimately the


4 B 2
3202 TEXT-BOOK OF OPHTHALMOLOGY
majority become fragmented to form globules of myelin and granular
detritus frequently running in algae-like formations. The nucleus may be
preserved for some time, but eventually it also tends to become fissured, and
Fig. 27.34-HyPERMATURE CATARACT.
Liquefied cortex and displacement of the fissured nucleus (v. Szily, T. O. S.).
as the cortex gradually becomes dehydrated and shrinks to form a pultaceous
mass, the cataract passes into the stage of hypermaturity (Fig. 2734) and
eventually may reach the Morgagnian condition.
PERI-NUCLEAR PUNCTATE CATARACT
This type of opacity which goes under several names (DUst-LIKE
CATARACT IN conce NTRIC LAYERs, etc.) appears in elderly people usually in
association with coronary cataract. Its onset is recognized by a thickening
and intensification of the appearance of the anterior and posterior bands of
Fig. 2735.-PERINuclean AND Cortical CATARAct.
Inter-lamellar fissures filled with finely granular detritus (v. Szily, T. O. S.).


DISEASES OF THE LENS 3203
the adult nucleus, and eventually multiple small opaque dots usually
interspersed with larger plaques are seen in the deeper layers, forming
aggregations of small foci, concentric lines and cloudy patches (the PERI-
PHERAL CONCENTRIC OPACITIES of Vogt, 1918). Pathologically these early
opacities are seen to represent more or less narrow fissures following generally
the course of the lens fibres filled with finely granulated detritus (Fig. 2735);
but although such an appearance may remain relatively unchanged for
some considerable time, it usually becomes complicated by the addition of
other types of cortical cataract and complete opacification eventually results.
CUPULIFORM CATARACT
This type of opacity—the POSTERIOR SAUCER-SHAPED CATARACT of
Vogt (1919)—is confined to the posterior layers of the cortex, where it lies
directly under the capsule. In the focal illumination of the slit-lamp it
appears distinctly yellow, probably because of the dispersion of the shorter
rays of light, and is made up of a cloud of minute opacities, frequently
crystalline in appearance, between which are numerous rounded vacuoles
(Figs. 2723, 25, Plate LXIII). This kind of cataract progresses very slowly of
itself, but it is usually complicated by other types: nuclear sclerosis is the
most common association, while other types of cortical opacities accelerate
the general opacification of the lens. Such an opacity, being near the nodal
point of the dioptric system, obstructs vision early and may interfere to
a surprising degree with the visual acuity at a stage when it may be so
slight as to be readily overlooked with the ophthalmoscope. Herein lies the
clinical importance of its recognition and the value of examining the posterior
region of the lens under mydriasis with the slit-lamp in cases of failing vision
in elderly persons. A second important clinical matter is the differential
diagnosis from complicated cataract, sometimes a matter of some difficulty
which will be discussed when dealing with the latter. Fortunately, however,
despite the apparent immaturity, the lens is usually easily extracted even by
the extra-capsular method, for relatively little cortex is left behind.
Alt. A m. J. O., xvii, 15, 1900. Burdon-Cooper. Brit. J. O., vi, 385, 483,
Andersen. Acta O., ii, 250, 1925. 1922.
Andrassy. K. M. Aug., lxvi, 568, 1921. T. O. S., liii, 401, 1933.
Appenzeller. Mitt. d. oph. Kl. Tiibingen, ii, Busacca. Rev. gen. d’O., xxxix, 439, 1925.
120, 1884. Carmi. Boll. d’Oc., xxiii, 426, 1930.
Barth. Diss., Basle, 1914. Demicheri. Am. d’Oc., cziii, 93, 1895.
Bastard. Thése de Montpellier, 1850.
Becker. Zur Anat. d. gesunden w. kranken
Linse, Wiesbaden, 1883.
Belivier. In Guille : Nowvelles recherches sur
la cataracte et la goutte Sereine, Paris, 1818.
Bellows. A. of O., xiv, 99, 1935.
Berstein. Growth and Decay, N.Y., ii, 209,
1934.
Biro. A. d’O., liii, 685, 1936.
Bücklers. B. O. G. Heidel., xlviii, 234, 1930.
K. M. Aug., xcvi, 119, 1936.
Donders. Anomalien d. Refraktion w. Akkom.
d. Auges, Wien, 1888.
Dyer. Prov. Med. and Surg. J., 383, 1846; 91,
1850.
Elschnig and Zeynek. Z. f. Aug., xxix, 408,
1913.
Foster. Brit. J. O., xvii, 408, 1933.
Foster and Benson. T. O. S., liv, 127, 1934.
Gaiser. A. f. O., exxi, 145, 1928.
Garfunkel. A. Jul. klaus-Stift., ii, 71, 1926.
Gatti. Am. di Ott., xxxiii, 610, 1904.
3204
TEXT-BOOK OF OPHTHALMOLOGY
Gifford and Puntenny. Am. J. O., xvi, 1051,
1933.
Goldmann. A. f. Aug., cx, 405, 1937.
Gradle. A. of O., iv, 589, 1930.
Contrib. to Oph. Science, 255, 1926.
v. Graefe. A. f. O., i (1), 333, 1854.
Green. T. Am. O. S., v, 724, 1890.
Guttmann. Cb. pr. Aug., 493, 1898.
Halben. A. f. O., lvii, 277, 1903.
Halbertsma. K. M. Aug., lxxx, 108, 794,
1928.
Herzfeld. A. f. O., cviii, 218, 1922.
Hess. G-S. Hb., II, vi (2), 1905; iii, 1911.
v. d. Heydt. A. of O., iv, 188, 1930.
Horlacher. Z. f. Aug., x1, 33, 1918.
Jeaffreson. Lancet, i, 387, 434, 479, 1886.
Rirby. A. of O., viii, 97, 1932.
Kistler. K. M. Aug., lxxx, 181, 1928.
Rnapp, A. A. of O., xlv, 600, 1916.
Roby. Slit-lamp Microscopy of the Living
Eye, London, 1930.
Rrause. A. of O., ix, 617, 1933.
Magnasco. Saggi di Oft., iv, 63, 1929.
Magnus. A. f. O., xxxvi (4), 150, 1890.
Mahieu. A. d’O., xxxix, 510, 1922.
Mawas. Bull. S. fr. d’O., xliii, 3, 1930.
McHardy. T. O. S., ii, 10, 1882.
Michail and Vancea. C. R. S. Biol., xciv, 291,
1926; xevi, 65, 1927.
Moeller. Acta O., v, 258, 1927.
Moretti. A. di Ott., xxxviii, 288, 1931.
Müller. K. M. Aug., xxxii, 178, 1894.
Nettleship. R. L. O. H. Rep., xvi, 179, 1905.
T. O. S., xxix, p. lxxviii, 207, 1909; xxxii,
337, 1912.
Neuschüler. Bull. Acad. med. Roma, lvi, 134,
1930.
Lett. oft., vii, 237, 1930.
Norrie. Ugeskr. Laeg., iii, 937, 1896.
Parker. J. Michigan Med. S., xv, 188, 1916.
Pfeiffer. A. f. O., cvii, 71, 1921.
Puscariu and Nitzulescu. Brit. J. O., xx,
531, 1936.
Rollet and Bussy. A. d’O., xxxviii, 65, 1921.
Ropshaw. Am. J. Physiol., ciii, 535, 1933.
Rowan and Wilson. Brit. J. O., v, 64, 1921.
Sauermann. Am. J. O., xvi, 985, 1933.
Schild. A. f. O., cvii, 49, 1921.
Speciale-Cirincione. A. f. Avg., lvi, 68, 1906.
Steinhaus. A. f. Aug., cv, 731, 1932.
v. Szily. K. M. Aug., xli (2), 44, 1903.
T. O. S., lviii, 595, 1938.
Vinsonhaler and Cosgrove. A. of O., xv, 222,
1936.
Vogt. A. f. O., lxxxviii, 329, 1914; cvii, 196,
1921.
Z. f. Aug., xl, 123, 1918; 1, 145, 1923.
K. M. Aug., lxi, 89, 1919; lxii, 396, 582,
593, 1919.
Schw. med. W., li, 265, 1921.
Atlas, Berlin, ii, 1931.
Vogt and Lussi. A. f. O., c, 157, 1919.
Wenzel. Abhand. u. d. Star, Nürenberg, 1788.
Wood, Casey. Ophthalmoscopy, xv, 142, 1906.
C. Pathological Cataract
1. DLABETIC CATARACT
That sufferers from diabetes mellitus are particularly prone to the
development of cataract has been recognized for many years; this was
noted, for example, by as early a writer as John Rollo (1798), while by the
time of Berndt (1834), Benedikt (1842) and Himley (1843), its recognition
was common. In this class of person two types of cataract occur :—
1. Ordinary changes of the senile type differing in no way from those
usually met with in aged subjects, except that in diabetics they occur more
frequently and at an earlier age than in non-diabetic subjects and tend to
progress more rapidly to maturity.
2. A true diabetic cataract characterized by widespread and pathog-
nomonic sub-capsular changes, occurring bilaterally, which tends to run a
relatively acute course, and occurs preferentially in young persons.
The first type of cataract is common ; the second rare.
The common occurrence of cataract in diabetes is confirmed by numerous
statistics; typical are those of Kirby (1933), who found in diabetics of all
ages that 36% had clear lenses and 64% had opacities of some kind—a very
DISEASES OF THE LENS 3205
much higher proportion than that found in the normal population. Among
these he found 70% to be of the senile cortical type, 21% nuclear, 7%
posterior cortical, and 2% sub-capsular. The severity of the diabetes makes
little difference in the incidence, for cataract tends to be more frequent in
the moderate cases than in the severe; but the duration of the disease is
important, for Kirby (1933) found that of the diabetics of 1 year's standing
22% had cataract, of 2 years' standing 62%, 3 years' 44%, 4 years' 63%,
5 years' 40%, and over 5 years’ 70%. Even in young diabetics (3 to
33 years) O'Brien, Molsberry and Allen (1934) found that 16% of 126 patients
showed marked lens opacities.
It is to be noted, however, that these findings are not universally agreed upon,
for authorities such as Andersen (1929) and Waite and Beetham (1935), although
finding punctate opacities and crystalline deposits more common in diabetics, concluded
on the whole that these patients were no more prone to cataract than the general popula-
tion.
Typical, DLABETIC CATARAct (the snow-FLAKE CATARACT of O'Brien,
Molsberry and Allen, 1934) tends to occur in patients who, while they have
severe diabetes, are yet relatively well and in whom the water-balance is
Fig. 2736.-DLABETIC CATARACT.
Showing dense band of sub-cortical opacities in optical section (Goulden,
T. O. S.).
well maintained. It may appear at a very early age in cases of infantile
diabetes, the earliest case having been reported at 11 months (Major and
Curran, 1925). In adolescents it becomes more common (7 years, Schnyder,
1923; 10 years, Strouse and Gradle, 1924; 12 years, Joslin, 1923; 15 years,
Sherrill, 1922; 16 years, Kirby, 1933; and others); it is met with
not very infrequently in adults, but in the aged the typical sub-capsular
opacities become rare. Characteristically the cataract matures rapidly

3206 TEXT-BOOK OF OPHTHALMOLOGY
especially in the young, sometimes within a few hours (Litten, 1893;
Scheffels, 1898), usually within some days (4 days, Scheffels, 1898; Kirby,
Fig. 2737. CATARA or
1N DIABETEs.
The superficial fibres
of the lens: surface view
(Goulden, T. O. S.).
1933; 11 days, Neuberger, 1893), and seldom taking
more than a few weeks. In adults, however, its
course is less stormy, for progression of the opacities
may proceed leisurely presenting periods of arrest
and even retrogression with variations in the intensity
of the glycosuria.
Clinically the cataract, which invariably occurs
bilaterally, has a typical appearance consisting
essentially of sub-capsular opacities, particularly in
the posterior cortex. This distribution of the early
changes was known to the older writers both from
clinical (Förster, 1876; Neuberger, 1893; Klein,
1901) and pathological (Deutschmann, 1877–87)
investigations, while in more recent times the slit-
lamp has confirmed its more intimate details (Schnyder, 1923; Goulden,
1928; Weilland Nordmann, 1930; O'Brien, Molsberry and Allen, 1934; and
Fig. 2738.-Mature, DLABET to CATARAct
(Kirwan, Brit. J. O.).
others). Immediately under the capsule there appears a veritable carpet of
vacuoles interspersed with fine punctate and flaky white opacities, and in
the cortex underneath extensive hydration of the tissues is usually revealed
by the presence of water clefts and separation of the sutures (Figs.
2736-7, 2726, Plate LXIII). Thereafter more diffuse cloudy opacities
appear, usually grey and transparent in front and forming white solid


DISEASES OF THE LENS 3207
plaques behind, until the stage of general intumescent cataract is reached
and the opacification becomes complete and uniform (Fig. 2738). It is to
be noted that in the early stages, when the changes are limited essentially
to Oedema without denaturation of the proteins, adequate control of the
diabetes may result in a disappearance of the opacities (Nettleship, 1885;
Alt, 1906 ; and others). This, of course, is exceptional, but in the average
case, Operative treatment does not entail a bad prognosis provided the
diabetes can be kept under control.
Pathological examinations have repeatedly been done on diabetic cataract (His,
1854; Knapp, 1863; Becker, 1883; Deutschmann, 1887; Kamochi, 1887; Görlitz,
1894; Kako, 1903; Groenouw, 1907; Hess, 1911 ; and others). The general histo-
logical appearances resemble those of other types of lenticular opacity, but the peculiar
features are the localization of the opacities to the sub-capsular region and the occa-
Sional massive Oedema of the tissue (Hydrops capsulae lentis). In chemical analyses
Carey and Hunt (1935) found that diabetic cataractous lenses had the same composition
as those of Senile cataract except that the phosphorus content was much lower.
While it is undoubtedly the case that the operative treatment of
cataract in diabetics provides anxieties more than in non-diabetic patients,
the presence of diabetes in itself is no contra-indication to dealing with the
cataract in the ordinary way. The two complications most to be dreaded
are the occurrence of haemorrhage and the subsequent development of
infection, to avoid which every care should be taken. In these subjects
therefore, hyperpiesia should be given particular attention, and a very rigid
exclusion of focal sepsis should be undertaken ; but with these precautions,
Operation should lead to good results provided the diabetes has been
adequately controlled for some time beforehand. For some months prior
to Operation, therefore, the patient should be under the unremitting care of
the physician, and for some days before the operation sugar should be
eliminated, or as nearly so as may seem advisable, and the dietary conditions
and insulin dosage standardized in the hospital where the operation is to be
performed. It is to be remembered, however, that it is inadvisable to push
insulin in large doses immediately before the operation since there is
evidence that this increases the liability to haemorrhage (Benedict, 1925).
It is to be remembered that the proper use of insulin has changed the prognosis
of such operations completely. Thus before its introduction, Gifford (1911) found that
43% of young diabetics after extraction lost their eyes from post-operative infection,
while Wheeler (1916) over a series of 2,123 extractions met haemorrhages in 5% of
ordinary cases and in 28.94% of diabetics.
AEtiology. The reason for the common occurrence of cataract in
diabetics has excited much interest, and several theories have been advanced
in explanation.
1. The oldest suggestion that the lenticular opacities were due to the
presence of sugar in the aqueous and the lens (Kunde, 1857; Stoeber, 1862;
3208 TEXT-BOOK OF OPHTHALMOLOGY
and others) had necessarily to be discarded when it was demonstrated that
to be effective a concentration of 5% was required, which is far above that
compatible with life ; moreover, actual estimations showed that this was
never approached in the aqueous, while the cataractous lens itself might be
sugar-free (Deutschmann, 1877–87).
2. Deleterious toacic products of metabolism circulating in the blood
have been considered a causal factor (Parsons, 1905).
3. The oedematous and degenerated State of the ciliary epithelium in the
diabetic eye" has been said to interfere with the nutrition of the lens (Peters,
1893).
4. A photo-chemical eacplanation was advanced by Schanz (1916), who
pointed out that sugar and acetone sensitized protein to the denaturing
action of light.
5. Endocrine failure, affecting primarily the internal secretion of the
pancreas, has been suggested as the primary cause (Schiótz, 1913;
Löwenstein, 1926–34 ; and others).
6. A decrease in the permeability of the capsule in vitro in the presence of
glucose was demonstrated by Bellows (1938), who suggested that this might
predispose to the development of cataract. It is interesting that Kirby,
Estey and Wiener (1933) found that while sugar did not interfere with the
growth of the lens epithelium in vitro, ketone bodies (acetone and oxybutyric
acid) were toxic in concentrations such as are met with in moderately severe
diabetes.
7. The hydration of the lens by osmotic influences owing to an upset of
the equilibrium of the tissue-fluids may exert an influence (Duke-Elder,
1925–26). With a rising blood-sugar, provided the water-balance of the
body is reasonably maintained, the effective osmotic pressure of the tissue-
fluids tends to fall owing to a loss of salts: these to some extent are replaced
by glucose, while the heavy loss of salt in the increased excretion of urine
is not adequately replaced although the bulk of fluid is made up by drinking
water. The lowered osmotic pressure of the aqueous thus determines an
inflow of water into the lens, causing first its deformation with the production
of myopia,” and eventually the oedema and hydration which results in
Cataract.
8. Acidosis associated with diabetes may play some considerable part
in the determination of the development of cataract, for it will be remem-
bered that with a lowering of the pH, proteolytic ferments become active
which may to some extent be directly responsible for opacification ; more-
over, it may also act indirectly by increasing the osmotic pressure within
the lens owing to the breakdown of large osmotically inactive molecules
to smaller complexes, thus determining an osmotic inflow of fluid into the
lens (Krause, 1934).
1 p. 2383. 2 Vol. IV.
DISEASES
OF THE LENS 3209
CATARACT IN CHOLERA AND HUNGER
The cataract which sometimes suddenly develops in the collapse stage of CHOLERA
may have a somewhat similar osmotic basis to that which possibly enters into the
aetiology of diabetic cataract.
In this case the loss from the bowel may lead to an
Osmotic dilution of the body-fluids, including the aqueous, and a consequent inflow of
water into the lens.
A similar explanation might be effective in the cataract produced by prolonged
STARVATION when water only is taken.”
Alt. Am. J. O., xxiii, 294, 1906.
Andersen. Acta O., vii, 339, 1929.
Becker. Z. Amat. d. gesunden u. kramkh.
Linse, Wiesbaden, 1883.
Bellows. A. of O., xx, 80, 1938.
Benedict. Ohio State Med. J., xxi, 648, 1925.
Benedikt. Abhandl. a.d. Gebiet. d. Augenh.,
Breslau, i, 38, 1842.
Berndt. Kl. Mitteilungen, Greifswald, 1834.
Carey and Hunt. New England J. Med.,
coxii, 463, 1935.
Deutschmann. A. f. O., xxiii (3), 112, 1877;
xxv (2), 213, 1879; xxxiii (2), 229, 1887.
Duke-Elder. Brit. J. O., ix, 167, 1925.
Lancet, i, 1188, 1250, 1926.
Förster. G-S. Hb., I, vii, 220, 1876.
Gifford. O. Rec., xx, 243, 1911.
Görlitz. Diss., Freiburg, 1894.
Goulden. T. O. S., xlviii, 97, 1928.
Groenouw. Alg. Abh. Augenh., vii, 14, 1907.
Hess. G-S. Hb., III, ix (2), 126, 1911.
Himley. Die Krankh. w. Missbildungen d.
mensch. Auges, 1843.
His. A. f. path. Amat., 561, 1854.
Joslin. Treatment of Diabetes Mellitus, III,
1923.
Kako. K. M. Aug., xli., 253, 257, 1903.
Kamochi. A. f. Aug., xvii, 247, 1887.
Rirby. A. of O., ix, 966, 1933.
Rirby, Estey and Wiener. A. of O., x, 37,
1933.
Rlein. Wien. Jel. W., 1097, 1901.
Knapp. K. M. Aug., i, 168, 1863.
Krause. Am. J. O., xvii, 507, 1934.
Kunde. A. f. O., iii, 275, 1857.
Leber. A. f. O., xxi, 208, 1875.
Litten. Münch. med. W., x1, 880, 1893.
Löwenstein. A. f. O., czvi, 438, 1926;
cxxxii, 224, 1934.
Major and Curran. J. Am. Med. As., lxxxiv,
674, 1925.
Nettleship. T. O. S., v., 107, 1885.
Neuberger. Cb. pr. Aug., xvii, 165, 1893.
O’Brien, Molsberry and Allen. J. Am. Med.
As., ciii, 892, 1934.
Parsons. Path. of the Eye, London, ii, 425,
905.
Peters. A. f. O., xxxix, 221, 1893.
Rollo. Cases of Diabetes Mellitus, 1798.
Schanz. A. f. O., xci, 238, 1916.
Scheffels. O. Kl., ii, 124, 1898.
Schiótz, Carl. Nors. Mag. f. Lägevidenskab.,
lxxiv, 1201, 1913.
Schnyder. K. M. Aug., lxx, 45, 1923.
Sherrill. J. Metab. Res., i, 667, 1922.
Stoeber. Am... d’Oc., xlviii, 192, 1862.
Strouse and Gradle. J. Am. Med. As., lxxxii,
546, 1924.
Waite and Beetham. New England J. Med.,
coxii, 367, 429, 1935.
Weill and Nordmann. Bull. S. fr. d’O., xliii,
17, 1930.
Wheeler. T. A. m. O. S., xiv, 742, 1916.
2. ENDOCRINE CATARACT
Lenticular opacities of a peculiar type, characterized by discrete
punctate opacities in the cortex, are commonly associated with disturbances
of the ductless glands. Whether or not this is the true explanation in all
cases, they are conveniently considered together because of their morpho-
logical similarity. In their pure form—for they are frequently complicated
by other changes—these discrete opacities are characterized anatomically
by rounded, oval foci with deeply staining contents, sometimes homogeneous,
sometimes flocculated and sometimes granular (v. Szily, 1938) (Fig. 2739).
Their most striking clinical characteristics are their early onset, their
bilateral incidence and their zonular distribution.
1 p. 3139.
3210 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2739.-E.N.Doc RINE CATARACT.
Oval homogeneous or flocculated foci with dispersed honeycombed granular
deposits (v. Szily, T. O. S.).
CATARACT IN APARATHY ROIDEA AND TETANY
Tetany may be of two types, post-operative or idiopathic. Post-operative tetany
(tetania strumipriva) occurs as a rule 2 to 3 days after an operation for removal of the
thyroid gland in which the parathyroids have been removed also in mistake. Idiopathic
tetany occurs in young persons, particularly in infants (infantile tetany); it may be
associated with spontaneous hypo-parathyroidism, it may complicate rickets, appear
in endemic form, or occur without apparent cause. Clinically either type may appear
in two forms, latent and manifest. In latent tetany the only clinical evidences are
certain trophic changes in the epithelial structures (falling out of the hair, brittleness
of the nails, wrinkling of the skin and dental decay) and a hyper-excitability of the
peripheral nervous system (Erb'ssign, that is, increased response to galvanic stimulation:
Chvostek's sign, facial twitchings produced by tapping the facial nerve in the parotid
region; Trousseau's sign, muscular contractions of the forearm and hand on constric-
tion of the arm). When the condition becomes manifest cramps and spasms appear in
the hands and arms, and later in the feet and legs, until they become generalized
convulsions and may result in death. A constant occurrence is a reduction of the
serum calcium both in its absolute and ionized forms, an increase of phosphorus
and a relative increase of potassium. In latent tetany the serum calcium usually
varies from 6 to 10 mg. per 100 c.c.; if it falls below 5 mg. convulsions occur. The
administration of calcium and the hormone of the parathyroid almost invariably
controls the symptoms.
It was first noted that cataract could occur in relatively young persons
who were or had been sufferers from muscular cramps by Logetschnikow
(1872) and Schmidt-Rimpler (1883); and a little later attention was drawn

DISEASES OF THE LENS 3.211
more vividly to the subject by the recognition by Landsberg (1888) of a
rapidly developing cataract in a patient who suffered from epileptiform
convulsions after thyroidectomy. Thereafter a large number of observations
fully established this clinical sequence (Hoffmann, 1897; Schiller, 1899;
Westphal, 1901; Possek, 1907; Königstein, 1907; Purtscher, 1909;
Vogt, 1911; Jeremy, 1919; Matthews, 1920; Jaeger, 1920; Eiselberg,
1921; Pamperl, 1921; van Lint, 1922; Sainton and Péron, 1922; Sidler-
Huguenin, 1922; Greppin, 1922; Knüsel, 1924; Heine, 1925; Aub,
1926; Hunter, 1928; Jacques,
1928; Goulden, 1928; Cole, 1930;
Salvesen, 1930; Marcove, 1931;
O’Brien, 1932; Ellet, 1934; and
others). It must be remembered,
however, that the occurrence of
cataract is by no means invariable;
thus Boothby, Haines and Pemberton
(1931) reported a series of 88 cases of
post-operative tetany without a single
instance of cataract. We have al-
ready noted the ease with which
cataract can be produced in animals
after removal of the parathyroids."
The clinical appearance of opaci-
ties in the lens sometimes becomes
- - Fig. 2740–CATARACT IN APARATHY Roidea.
evident a few months after parathyroi- In a woman of 42, 20 years after a
dectomy (2 months, Goulden, 1928; thyroidectomy; , Nº signs ºf myxoedema,
- but carpo-pedal, laryngeal, and oculo-
3 to 4 months, Jeremy , - 1919; 5 motor spasms with positive Chvostek's,
months, Matthews, 1920), but may be Erb's and Trousseau's signs. There are
white powdery and red, blue and green
delayed for some years (7 Yººts, crystalline deposits in the cortex (Goulden).
Knüsel, 1924; 11 years, Eiselberg,
1921; 20 years, Heine, 1925). The lens changes may take on many forms, but
the typical appearance is that of numerous, small, discrete opacities in the
cortex lying in a layer separated from the capsule by a clear zone of
disjunction (Figs. 2740–41); usually these are small white and punctate,
sometimes they are aggregated into larger flakes, and not infrequently they
are interspersed with angular iridescent crystals of a blue, green or red colour.
A feature stressed by some writers is a lessened antero-posterior diameter
of the lens (Kast, 1926), and sometimes there is a more or less homogeneous
opacity of the lamellar type surrounding the nucleus (O'Brien, 1932). The
three constituents, punctate spots, flakes and iridescent crystals, may vary
in their relative proportions, and one or other may be predominantly present,
and usually they are most prominent near the posterior pole. The condition
may remain stationary indefinitely with little disturbance of vision, or
1 p. 3154.

3.212 TEXT-BOOK OF OPHTHALMOLOGY
alternatively the lesions may multiply, opacities of the senile type may
supervene, or the whole lens may become intumescent and uniformly
opaque in a comparatively short time. The pathological examination of
such a fully developed opacity described by Bartels (1906) showed the outer
cortex riddled with fissures, vacuoles and punctate opacities, beneath which
was a homogeneous layer of deep cortical opacity, while the nuclear fibres
had begun to degenerate and disintegrate.
The diagnosis is made upon the history of thyroidectomy, the evidences
of tetany or of latent tetany as
evinced by hyper-excitability of the
nervous system, the low serum
calcium and high serum phosphorus
content, and the characteristic
morphological appearance of the
lenticular opacities occurring bilater-
ally. We have seen that in experi-
mental cataract after removal of the
parathyroids in animals the develop-
ment of opacification is controlled by
maintaining a high blood calcium or
by the administration of radiated
ergosterol (Rauh, 1936–38). On the
whole, however, it has been found
clinically that treatment of the
Fig. 2741.-CATARACT IN TETANY
STEATorºnhº A.
In a case of coeliac dwarfism. There are
accompanying tetany has little effect
on the development of the cataract
cortical opacities and powdery and crystalline which tends to progress even
deposits in the nucleus. Vision normal - -
(Goulden). although the general clinical symp-
toms of tetany are controlled
(Jacques, 1928; Cole, 1930; O'Brien, 1932; and others). There is no
indication, however, that the operative removal of the lens itself is in any
way different from that of other types of cataract, although the occa-
sional reported occurrence of severe post-operative haemorrhage (Kast,
1926; O'Brien, 1932) is an indication for the administration of calcium
and parathyroid hormone before, during, and after the extraction.
The cause of the condition has already been discussed in the section on experi-
mental cataract, and the various aetiological views put forward have been detailed-
that the opacities are due to excessive muscular action, to a local chemical upset owing
to a disturbance of the ionic equilibrium by the calcium deficiency, or to the liberation
of a toxin either owing to a deficiency of parathyroid secretion or to a substance such
as histamine, guanidine or tyramine liberated during tetanic spasms. It will be
remembered that all these views are still conjectural.
In discussing the aetiology of cataract we have seen that many believe
that a number of congenital and juvenile opacities have a similar aetiology;

DISEASES
3213
OF THE LENS
this may well be, but at the same time the normal calcium metabolism found
in the majority of cases of senile cataract must rule out a latent tetany or
a parathyroid influence in this latter condition.
Aub. Boston Med. Surg. J., czciv, 844, 1926.
Logetschnikow. K. M. Aug., x, 351, 1872.
Bartels. K. M. Aug., xliv (I), 374, 1906. Marcove. Am. J. O., xiv, 887, 1931.
Boothby, Haines and Pemberton. Am. J. Matthews. T. O. S., x1, 440, 1920.
Med. Sc., clzxxi, 81, 1931. O’Brien. A. of O., vii, 71, 1932.
Cole. Lancet, i. 13, 1930. Pamperl. Deut. Z. f. Chir., clxi, 258, 1921.
Eiselberg. A. f. kl. Châr., cxviii, 387, 1921. Possek. K. M. Aug., xliv, Beil., 1, 1907.
Ellet. A. of O., xi, 58, 1934. Purtscher. Cb. pr. Aug., xxxiii, 97, 1909.
Goulden. T. O. S., xlviii, 97, 1928. Rauh. B. O. G. Heidel., li, 375, 1936.
Greppin. Schw. med. W., lii, 1260, 1922. Y V Internat. Congress O., Cairo, iv, 36,
Heine. Z. f. Aug., Iv, 1, 1925. 1938.
Hoffmann. Z. f. Nervenh., ix, 278, 1897. Sainton and Péron. Rev. neurol.., xxix, 442,
Hunter. P. R. S. Med., xxi, 1409, 1928. 1922.
Jacques. Am. J. Med. Sc., clz.xv, 185, 1928. Salvesen. Acta Med. Scand., lxxiii, 511;
Jaeger. Zb. f. Chir., xlvii, 565, 1920. lxxiv, 13, 1930. -
Jeremy. Brit. J. O., iii, 315, 1919. Schiller. Beit. kl. Chir., xxiv, 535, 1899.
Kast. Z. f. Aug., lix, 357, 1926. Schmidt-Rimpler. K. M. Aug., xxi, 181, I883.
Knüsel. A. f. O., cziv, 636, 1924. Sidler-Huguenin. A. f. O., cvii, 1, 1922.
Königstein. K. M. Aug., xlv., 268, 1907. v. Szily. T. O. S., lviii, 595, 1938.
Landsberg. Cb. pr. Aug., xii, 39, 1888. Vogt. Kor.-Bl. f. Schw. Aertze, xli., 695, 1911.
van Lint. Ann. et Bull. S. Roy de Sc. méd de Weinstein. Brit. J. O., xvii, 236, 1933.
Bru.relles, lxxvi-lxxvii, 36, 1922. Westphal. Berl. kl. W., xxxviii, 849, 1901.
CATARACT IN MYOTONIC DYSTROPHY
MYOTONIA DYSTROPHICA, a disease first described as myotomia congenita by Leyden
in 1874, was fully established as a clinical entity by Batten and Gibb (1909) and
Steinert (1909): it is characterized by myotonia (that is an excessive contractility and
a lessened relaxation) which is at times widespread and may affect the muscles generally,
but is most characteristically present in the hand-grips which show an inability to
relax, weakness of the facial and masticatory muscles, atrophy particularly of the
sterno-mastoids, the vasti of the thighs, the dorsiflexors of the feet and the flexors of
the hands, together with baldness, atrophy of the testes or premature menopause, and
cataract. There is a typical myotonic facies, with an unlined, expressionless face, a
drooping jaw, a slow unrelaxing Smile accompanied by a slow thick voice. Mental
changes are usually present, the patients being slow, unsociable and suspicious ; and
they are sterile. These symptoms usually appear between the ages of 20 and 30 and
show a gradual progression, most of the patients dying of secondary disease at a
relatively young age, usually before 45 years.
The first to draw attention to the occurrence of cataract in myotonia
was Greenfield (1911), who pointed out that pre-senile cataracts seen in
those patients were not a fortuitous but an essential part of the clinical
picture, and occurred not only in the victims of the general disease but
also in various members of the same family (Fig. 2742). The phenomenon
of anticipation is seen as in many other heredo-familial diseases, for an
uncomplicated cataract frequently occurs in several successive generations
of a family at progressively earlier ages, and then suddenly the “ dystrophic
generation ” appears with the fully developed disease with myotonia,
muscular wasting and other dystrophic signs (Greenfield, 1911;
3214 TEXT-BOOK OF OPHTHALMOLOGY
I. d & 9
N \
d * @
\
º
º
§ 3.66% 63&º 38 gºdd'd
II.s senile cataracts extracted 1902, ast-sº-ob-75-
ºzºa is cataracts - weak arºs, probably ºystonia- -* * early cat-, ob-sudden-
Iv-1 sudden death aet-25-
-4 symmetrical cataracts, L-extraction ast-39, typical myotonia-
-5 short-stout-º sub-pituitary, catam-ast-15-21-mid-6 years-no pregnancy; chor--retis-
-14 epileptic. -is ob-tuberol---23 mental hosp-13 years-
-25- ºb-in mental home-º ºyotonia and eater-at-
-ze unexplained cataract in only eye, aet.ze, extraction, probably myotonia-
II.
º
d
(2)º
ZZZ
*
:
-
******
s
- 2- --
*
V. 3 dº
Fig. 27.42. –Pedigree Is Myotoxia ATRophica
(Souter, T. O. S.).
Fig. 2743. Fig. 2744.
Figs, 2743–44. Cataract is Dystrophia Myotoxica.
Fig. 2743, optical section. Fig. 2744, surface view of anterior cortex (Caughey,
T. O. S.).

DISEASES OF THE LENS 3215
Curschmann, 1912; Kinnier Wilson, 1918; Fleischer, 1921 ; Frey, 1924;
Adie, 1924; Caughey, 1933; Souter, 1933; and others). Greenfield's
(1911) family is a typical case, wherein the members of the father's genera-
tion developed cataract at 60 and 69 years, while in the dystrophic
generation it occurred at 22, 28, 30 and 30 years. As a rule the fully
developed disease is confined to one generation of which some members
may remain healthy, some may have premature cataract, others may
have some muscular disease in an incomplete form, and yet others
present the full syndrome of
muscular atrophy with myotonia,
and extra-muscular symptoms in-
cluding cataract. In quite a number
of reports, however, examples of the
disease have occurred in more than
one generation (Hoffmann, 1912;
Kyrieleis, 1925; Heine, 1925;
Scheffels, 1925; Henke and Seeger,
1927; Berg, 1927; Gifford, Bennett
and Fairchild, 1929; Souter, 1933;
and others).
The characteristic type of
cataract originally noted by Green-
field (1911) and confirmed by
Ormond (1911), Hirschfeld (1911), Fig. 2745.-CATARAct IN Myoto.NiA
Hoffmann (1912), Löhlein (1914) DYSTRoPHICA.
and Fleischer (1918), has now nº.º.º.
become well recognized through
many detailed slit-lamp studies (Hauptmann, 1918; Vogt, 1921–24;
Lüssi, 1922; Scheffels, 1925; Foix and Lagrange, 1925; Kyrieleis, 1925;
Goulden, 1928; Gifford, Bennett and Fairchild, 1929; Caughey, 1933;
and others) (Figs. 2743–45). Pathological examinations have been
described by Vogt (1921) and Gil and Querol (1931). The pathognomonic
feature of the cataract is the bilateral appearance of a carpet of dust-like
and punctate opacities intermingled with larger angulated flakes giving an
iridescent display of scintillating colours, mainly red and green ; these
opacities are sharply localized in a thin zone of cortex just underneath the
anterior and posterior capsules. With this is frequently but not invariably
associated a stellate grouping of opacities at the posterior pole : it is probable
that this is a later development in some cases owing to progressive opacifica-
tion of the lens fibres along the suture lines. The sharp localization of the
initial lesions to one cortical layer is presumably due to the involvement of
the single stratum of the newest fibres of the lens at the time when the disease
has progressed to a certain point: the subsequent stellate appearances at
the posterior pole indicate a more diffuse and widespread involvement of
T-0--vol. III. 4. C

3216 TEXT-BOOK OF OPHTHALMOLOGY
the fibres. The condition may remain stationary in this stage for several
years with little alteration in vision (Bencini, 1929), or alternatively is
complicated by the development of water-fissures and a gradual opacification
of the entire lens after the manner of a senile cataract (Fig. 2746). Apart
from the occasional occurrence of optic atrophy, no other ocular complication
has been seen, and the results of numerous operations for extraction of the
cataract have been good.
The cause of the disease is unknown ; undoubtedly it has a congenital
basis. Numerous histological descriptions of the atrophic muscles have
Fig. 2746.-CATARAct IN Myotoxia ATRophica.
Advanced stage (Souter, T. O. S.).
been given, but no proved lesion has been found in the central nervous
system. On the other hand, a few observations of the atrophic changes in
the endocrine glands have been described, and the similarity of the changes
in the lens with the cataract occurring in aparathyroidea and other
dyscrasias of the ductless glands certainly points to their aetiological
influence (Curschmann, 1912; v. Szily, 1921–35; Heine, 1925; Kyrieleis,
1925). Although Krause (1938) has suggested that the lenticular opacities
may be associated with a disturbance of the metabolism of creatine, which,
as we have seen, is an important element in the metabolism of the lens, the
factor which links together the widely diverse changes of function and
structure seen in this disease are not, however, by any means clear. Terrien,
Sainton and Veil (1929), on the other hand, suggested that the lens changes
usually seen in the disease are merely those of premature senescence.

DISEASES OF THE LENS 3217
A rare case, aged 27, was reported by Monyukova (1935) in which the neurological
diagnosis was a combination of para-myotonia and myotonic dystrophy : endocrine
dysfunction manifested itself in hyperaesthesia, baldness, sexual impotence, debility,
and dental atrophy. Ten years previously one eye had been needled and the other
showed a typical lamellar cataract.
Adie. Brit. J. O., viii, 497, 1924.
Batten and Gibb. Brain, xxxii, 187, 1909.
Bencini. Boll. d’Oc., viii, 575, 1929.
Berg. Deut. Z. f. Nervenh., xcviii, 29, 1927.
Caughey. T. O. S., liii, 60, 1933.
Curschmann. Dewt. Z. f. Nervenh., xlv., 161,
1912.
Fleischer. A. f. O., xcvi, 91, 1918.
R. M. Aug., lxvii, 306, 1921.
Foix and Lagrange. Am. d’Oc., clxii, 637,
1925.
Frey. A. f. Rassembiol., xvii, 1, 1924.
Gifford, Bennett and Fairchild. A. of O., i,
335, 1929.
Gil and Querol. Rev. Esp. med., vi, 962, 1931.
Goulden. T. O. S., xlviii, 97, 1928.
Greenfield. Rev. Newrol. Psych., ix, 169, 1911.
Hauptmann. K. M. Aug., lx, 576, 1918.
Heine. Z. f. Aug., lv., 1, 1925.
Henke and Seeger. Biol. Zb., xlvii, 727, 1927.
Hoffmann. A. f. O., lxxxi, 512, 1912.
Krause. Am. J. O., xxi, 1353, 1938.
Ryrieleis. K. M. Aug., lxxiv, 404, 1925.
Z. f. Aug., liv, 185, 1925.
Löhlein. K. M. Aug., lii, 453, 1914.
Lüssi. Schw. med. W., lii, 796, 1922.
Monyukova. Sov. vest. O., vi, 669, 1935.
Ormond. T. O. S., xxxi, 214, 1911.
Scheffels. K. M. Aug., lxxiv, 512, 1925.
Souter. T. O. S., liii, 73, 1933.
Steinert. Deut. Z. f. Nervenh., xxxvii, 58;
xxxix, 168, 1909.
v. Szily. Zb. ges. O., v, 97, 1921 ; xxxii, 97 ;
xxxiii, 161 ; xxxiv, 193, 1935.
Terrien, Sainton and Veil. A. d’O., xlvi, 193,
1929.
Vogt. Schw. med. W., i, 669, 1921.
R. M. Aug., lxvii, 330, 1921 ;
1922; lxxii, 421, 1924.
A. f. O., eviii, 212, 1922.
lxix, 120,
Hirschfeld. Z. f. Neurol., v, 682, 1911. Wilson, Kinnier. T. O. S., xxxviii, 183, 1918.
FAMILIAL HYPERTEROPHIC MUSCULAR DYSTROPHY
FAMILIAL HYPERTROPHIC MUSCULAR DYSTROPHY (MYOTONIA CONGENITA or
THOMSEN’s DISEASE), is characterized only by the presence of myotonia without signs
of wasting or atrophy. As a rarity lens changes similar to those seen in myotonic
dystrophy are seen. Heine (1925) reported 2 cases of Thomsen's disease in a pedigree
of myotonic dystrophy, both of which showed typical sub-capsular opacities; while
Grönholm (1927), in a patient with Thomsen's disease, found one eye with a mature
cataract and the other with a posterior cortical opacity combined with white punctate
spots under the anterior capsule.
Grönholm. Acta O., v, 166, 1927. Heine.
Z. f. Aug., lv., 1, 1925.
MONGOLIAN IDIOCY
Mongolian idiots show characteristic lenticular opacities of the same
type but with a somewhat different distribution than those seen in myotonia
atrophica. Thus of 28 cases of such imbeciles, Pearce, Rankin and Ormond
(1910) found that 19 had lens changes of some form, 18 of which were of
the characteristic type : the average age of the 20 males included in this
series was 16% years and of the 8 females, 13 years. The occurrence of such
cataracts has been confirmed by Ormond (1912), van der Scheer (1919),
Jeremy (1921), Koby (1924), Weil and Nordmann (1926–30), Goulden
(1928), and others (Fig. 2747). The changes in the lens are not seen in
very young Mongols and are therefore acquired and not congenital.
A multitude of minute opacities are seen arranged in a zone running
4 ( 2
3.218 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2747–Forms of CATARAct round in Mongolias IMBECILEs (Ormond,
T. O. S.).
concentrically round the nucleus: some are powdery and punctate, some
annular and some flaky, while others have a crystalline appearance; the
majority are white, while others glitter with a red or green light (Fig. 2748).
Posteriorly they may be arranged in a stellar figure, and they are always more
numerous axially than peripherally. The foetal nucleus itself is clear, and the
-
º
.
|
.
º
-
.
º
º
º
-
Fig. 2748–CATARAct Is Mongolian Idrocy (Koby).
Optical section. -


DISEASES OF THE LENS 32.19
affected zone is separated from the capsule by a clear interval. The condition
thus resembles closely that seen in myotonia although the distribution of
the opacities points to a much earlier development.
Goulden. T. O. S., xlviii, 97, 1928. Pearce, Rankin and Ormond. Brit. Med. J.,
Jeremy. P. R. S. Med. (Dis. of Children), 11, i, 186, 1910.
1921. v. d. Scheer. K. M. Aug., lxxii, 155, 1919.
Koby. Rev. gen. d’O., xxxviii, 365, 1924. Weil and Nordmann. An d'Oc., clziii, 40,
Ormond. T. O. S., xxxii, 69, 1912. 1926.
Bull. S. fr. d’O., xliii, 17, 1930.
CRETINISM
Similar lenticular changes may develop in cretins, consisting essentially
of small flaky opacities interspersed with iridescent crystalline deposits in
Fig. 2749.-CATARAcT IN CRETINIsM (Goulden, T. O. S.).
the superficial cortex (Jeremy, 1921) (Fig. 27.49). Goulden (1928) reported
two cases, one of 19 and the other of 53 years, neither of whom had ever
taken thyroid therapeutically. In both the changes were only discernible
with the slit-lamp and in neither did they interfere with vision.
Goulden. T. O. S., xlviii, 97, 1928. Jeremy. P. R. S. Med. (Dis, of Children), 11.

1921.
3220 TEXT-BOOK OF OPHTHALMOLOGY
DERMATOGENOUS CATARACT
Certain types of skin disease have been associated with cataract since the
time of Rothmund (1868): clinically such cataracts occur in young persons,
are of bilateral incidence and are composed of discrete opacities, and since the
diseases in which they occur are frequently ascribed to endocrine insuffi.
ciency, particularly of the thyroid and the gonads, it is usual to ascribe the
Fig. 2750–CATARAct in Fig. 2751.--ENDoorise
SEVERE Eczema. CATARAct.
In a child (after Lowen- In a 35-year-old patient, an
stein). old case of meningitis with
polyg landular dyscrasias
(Löwenstein A.F.O.).
lens changes to an endocrine origin. That this is the case is not proven;
but they are conveniently considered together as cataracta dermatogenes
or cataracta syndermatotica (Kugelberg, 1934) (Figs 2750–51).
Nºunoprºtatºris formed the earliest and is the commonest association of a skin
disease with cataract (Rothmund, 1868; Werner, 1904; Androgsky, 1914; Vogt,
1922; Siegrist, 1928; Metzger, 1932; Franceschetti, 1934; Löwenstein, 1934:
Kugelberg, 1934; and others). The cataract is typically circumscribed and shield-
shaped, limited by concave borders and situated in the anterior cortex. It frequently
shows a metallic lustre, and from it fine linear opacities may radiate peripherally.
Sotºropºrºſia forms a second recognized association (Werner, 1904; Stekker,
1920; Vossius, 1920; Ehrmann, 1922: Monjukowa, 1923; Barbot, 1925: Sézary,
Favory and Mamon, 1930; and others). The usual opacity takes a fine fibrillar form
in a thin sub-capsular zone in the anterior cortex with a star-shaped opacity in the
Posterior cortex. Associated with the lenticular changes are pigmentary anomalies
of the skin, alopoecia, and dystrophies of the teeth and nails.
Poikilopºnyta ATRoPHICANs vasculawe (Rothmund's disease), a rare familial
disease characterized by an atrophie, tightly stretched skin showing patches of de-
pigmentation and hyper-pigmentation and the occurrence of telangietases, may be
associated with cataract (Rothmund, 1868 ; Siegrist, 1928; Lutz, 1932: Schnyder,
1935). Thus in Schnyder's family, 2, aged 4, and 12, in a generation of 4 were
affected, the cataract consisting of a star-shaped figure at the posterior pole combined
with a clouding of the entire cortex.

DISEASES OF THE LENS 322]
CHRONIC ECZEMA has also been associated with cataract (Vogt, 1922; Levy,
1931 : Franceschetti, 1934); as also DARIER’s DISEASE (v. Szily, 1921–35; Gjessing,
1922).
Androgsky. K. M. Aug., lii, 824, 1914. Monjukowa. Russ. O. J., ii, 174, 1923.
Barbot. These de Paris, 1925. Rothmund. A. f. O., xiv., 159, 1868.
Ehrmann. A. f. Dermat., csxxviii, 346, 1922. Schnyder. Sch. med. W., lxv, 719, 1935.
Franceschetti. B. O. G. Heidel., 1, 345, 1934. Sézary, Favory and Mamon. Bull. S. Méd.
Gjessing. Tijd. morsk. leg., 414, 1922. Hóp., Paris, lxvi, 358, 1930.
Kugelberg. K. M. Aug., xcii, 484, 1934. Siegrist. Der Graue Alterstar, Berlin, 1928.
Levy. K. M. Aug., lxxxvi, 681, 1931. Stekker. Diss., Rostock, 1920.
Löwenstein. K. M. Aug., lxxii, 653, 1924; v. Szily. Zb. ges. O., v, 97, 1921 : xxxii, 97;
lxxvi, 539, 1926. xxxiii, 16 l ; xxxiv, 193, 1935.
A. f. O., czkxii, 224, 1934. Vogt. A. f. O., ciz, 195, 1922.
Lutz. Kurzes Hb. Oph., vii, 291, 1932. Vossius. Z. f. Aug., xliii, 640, 1920.
Metzger. K. M. Aug., lxxxix, 821, 1932. Werner. Diss., Kiel, 1904.
3. TOxIC CATARACT
We have already seen that a toxic cataract can be readily produced in
animals by the administration of naphthalene, thallium, lactose and
galactose. A similar aetiology applies to some types of cataract seen
clinically, particularly those produced by dinitrophenol, paradichloro-
benzene and ergot, and probably also to the lenticular opacities occurring
in certain acute toxic illnesses.
(i) DINITROPHENOL CATARACT
Since the researches of Cazeneuve and Lepine (1885) and Gibbs and
Reichert (1891) it has been known that drugs of the type of dinitronaphthol
and dinitrophenol increased the metabolic activity of animals to such a
degree that they produced heat so fast that they sometimes died of
the resulting fever. This pharmacological activity excited renewed
interest by the occurrence of dinitrophenol poisoning among French munition
workers in the First World War, with the result that its use was suggested
in America with the object of reducing weight rapidly without dietary
restrictions. Its wide-spread and indiscriminate use, however, soon resulted
in the appearance of toxic symptoms, one of which was the development of
cataract. The first cases to be reported were those of Boardman (1935)
and Horner, Jones and Boardman (1935), and soon thereafter upwards of a
hundred records appeared (Cogan and Cogan, 1935; Kniskern, 1935;
Lazar, 1935 ; Allen and Benson, 1935 ; Schutes, 1935 ; Mann, 1936 :
Spaeth, 1936; Veasey, 1936 : Whalman, 1936; Hessing, 1937). There
must be a definite individual idiosyncrasy, since of the hundreds of thousands
of people who have used the drug only an insignificant number have
developed cataract ; moreover, the total dose taken in those cases in which
lenticular changes have ensued has varied within the widest limits. Hessing
(1937) concluded that not only was there an individual susceptibility but
that this was familial. It is interesting that the great majority of these
3222 TEXT-BOOK OF OPHTHALMOLOGY
cases came from America, but some have been reported from Europe
(Onfray and Gilbert-Dreyfus, 1937, from France ; Helminen, 1937, from
Finland): indeed, the geographical distribution suggested to Dally (1936)
that the occurrence of cataract was probably due not to dinitrophenol but
to some impurity, such as dinitronaphthol, which had crept into recent
American manufacture. In this event the cataract might be considered
comparable with naphthalene cataract. In this connection it is to be noted
that a similar cataract has followed the use of reducing drugs which do not
contain dinitrophenol (Barkan and Bettman, 1938).
The changes in the lens may come on rapidly or be delayed for some
months (6 to 7) after cessation of the drug. They commence with the
appearance of bilateral, grey, dust-like Opacities just beneath the anterior
capsule, giving a stippled, polychromatic, striate or downy effect, while
the posterior cortical region shows a peculiar saucer-shaped granular
metallic lustre which has been likened to a cloth of gold (Horner, 1936).
At this stage the vision may remain unaffected, but development usually
proceeds rapidly, the opacities invading the cortex and finally the nucleus,
so that the whole lens becomes silky-grey and eventually pearl-like and
completely opaque, sometimes showing segmentation with dark wedge-like
intervening spaces. In the initial stages the lens frequently swells, making
the anterior chamber shallow and bringing about a secondary glaucoma ;
but operative treatment for the cataract usually has excellent results.
The cause of the cataract is quite unknown. Experimentally it has
been found that dinitrophenol administered orally finds its way into the
lens (Horner, 1936), but it has no precipitating effect in vitro upon the
lenticular proteins (Cogan and Cogan, 1935). There are no observable
changes in the metabolism of the lens after its administration, except that
it stimulates oxidative processes (Field, Tainter, Marten and Belding,
1937), nor in the permeability of the capsule in vivo and in vitro (Borley
and Tainter, 1937). Indeed it would seem to be impossible to reproduce
cataract in animals in any circumstances even although those which are
ordinarily susceptible to cataract, such as rats (Tainter, 1936), guinea-pigs
(Sohr, 1938), or rabbits (Helminen, 1937), are dosed with the drug up to
fatal concentrations continuously from weaning until death. This failure
persists even although every other encouragement to the formation of
cataract is afforded, such as by feeding with a vitamin-deficient diet (Borley
and Tainter, 1938); nor does the administration of the drug accelerate or
influence in any way the development of cataract by other experimental
means, such as by feeding with lactose (Borley and Tainter, 1938). It
would appear, therefore, that if dinitrophenol is responsible for the develop-
ment of cataract it does so by an interference with metabolism peculiar to
man ; and while tentative suggestions may be made, such as that of Onfray
and Gilbert-Dreyfus (1937), that a toxicosis involving hepatic insufficiency
causes first a dehydration and then a sudden rehydration of the lens with
DISEASES
OF THE LENS 3.223
a massive imbibition of fluid and consequent opacification, or that of Krause
(1938) that it interferes with glycolysis by deranging the creatine metabolism,
such suggestions must at the present time remain merely speculative.
Allen and Benson. J. Am. Med. AS., cv, 795,
1935.
Barkan and Bettman. Am. J. O., xxi, 165,
1938.
Boardman. J. A. m. Med. As., cv, 108, 1935.
Borley and Tainter. A. of O., xviii, 908,
1937 ; xx, 30, 1938.
Am. J. O., xxi, 1091, 1938.
Cazeneuve and Lepine. C. R. Ac. Sc., ci,
1167, 1885.
Cogan and Cogan. J. Am. Med. As., cv, 793,
1935.
Dally. A. S. Méd. biol. Montpellier, xvii, 109,
1936.
Field, Tainter, Marten and Belding. Am.
J. O., xx, 779, 1937.
Helminen. Acta O., xv, 490, 1937.
Pſessing. A. of O., xvii, 513, 1937.
Horner. A. of O., xvi, 447, 1936.
Horner, Jones and Boardman. J. Am. Med.
As., cv, 109, 1935.
Rniskern. J. Am. Med. As., cv, 794, 1935.
Krause. Am. J. O., xxi, 1343, 1938.
Lazar. J. Am. Med. As., cv, 794, 1935.
Mann. A. of O., xv, 116, 1936.
Onfray and Gilbert-Dreyfus.
d’O., l, 114, 1937.
Schutes. Am. J. O., xviii, 752, 1935.
Sohr. A. f. O., czzxviii, 332, 1938.
Spaeth. Am. J. O., xix, 320, 1936.
Tainter. J. Pharm. Eacp. Therap., lxiii, 51,
1936.
Bull. S. fr.
Gibbs and Reichert. Am. Chem. J., xiii, 289,
1891.
Veasey. Am. J. O., xix, 332, 1936.
Whalman. Am. J. O., xix, 885, 1936.
(ii) PARADICHLOROBENZENE CATARACT
PARADICHLOROBENZENE, a white crystalline powder formed as a by-product in
the manufacture of trinitrophenol, is used commercially as a moth-repellant. Up to
quite recently it had been considered innocuous to man, although a powerful insecticide.
Berliner (1939) has recently found that inhalation of the vapour may have some toxic
effects on human beings, characterized by hepatitis with jaundice, great loss of weight,
and the formation of cataract. As is frequently the case in dinitrophenol cataract,
the changes in the lens appear as a delayed reaction several months after the patient
is removed from the fumes of the drug. The toxic effects can be elicited in animals
in which a necrosis of the liver may develop, but Berliner was unable to produce an
experimental cataract except in one rabbit which was fed with the drug orally.
Berliner. A. of O., xxii, 1023, 1939.
(iii) ERGOT CATARACT
The occurrence of cataract in ergot poisoning was first noted by Meier
(1861) in an epidemic caused by the eating of rye cereals contaminated by
fungus : 23 cases of cataract developed in 283 affected people. Since then
several other reports have followed, largely from Russian sources (Tepliaschin,
1889 : v. Bechterew, 1892 ; Kortneff, 1892 ; Kanzel, 1906 ; Karnicki,
1906; Germann, 1906; Kolosoff, 1912; Rodigina, 1929; and others).
The cause of the cataract (cataracta raphanica), which develops within a few
months, is obscure. Meier (1861) concluded the lenticular changes were due
to tonic and clonic spasms of the ciliary muscle, comparable to the cataract
associated with tetany. Orlow (1905) considered that it was the result
of a direct poisoning of the capsular epithelium, and that it thus fell into
line aetiologically with the associated neuro-refinitis, which he concluded
was also a direct toxic effect. Kortneff (1892) and Kaunitz (1932), on the
3224 TEXT-BOOK OF OPHTHALMOLOGY
Other hand, suggested a vaso-motor constriction and nutritional defect ;
while Peters (1902) found degeneration in the vessel walls.
Stellate lenticular opacities occurring in a case of RAYNAUD’s DISEASE
were described by Cattaneo (1931), who attributed their development to the
effects of arterial spasm and considered it comparable to ergot cataract.
v. Bechterew. Neurol. Cb., xi, 769, 1892. FColosoff. Russk. Vrach., x, 1803, 1842, 1911;
Cattaneo. Am. di Ott., xxxviii, 684, 1931. xi, 55, 120, 198, 1912; xii. 182, 211, 1913.
Germann. Vestn. O., 102, 1906 : Ref. Z. f. Kortneff. Vestn. O., ix, 114, 1892.
Aug., xvii, 94, 1907. Meier. A. f. O., viii (2), 120, 1861.
Kanzel. Oph. Congr. St. Petersburg, 1906 : Orlow. Neurol. Vestn., ix, 304, 1905.
Ref. Z. f. Aug., xvii, 93, 1907. Peters. B. O. G. Heidel.., xxx, 20, 1902.
Karnicki. Prezegl. Lekarski, lxv, 1906. Rodigina. Russ. A. O., v, 473, 1929.
Kaunitz. A. of Surg., xxv, 1135, 1932. Tepliaschin. Med. Oborzenje, xxxi, 525, 1889.
(iv) CATARACTA CACHECTICA
Bilateral cataract, usually of uniform distribution in the lens cortex and
maturing rapidly, is occasionally seen in acute toxic illnesses. The most
typical are infective fevers—diphtheria, malaria, typhus, cholera, small-pox,
scarlet-fever, typhoid fever ; but cataract may occur in similarly severe
toxic states, such as extreme cachexia, nephritis, pernicious anaemia, or
after a massive loss of blood. The prognosis of these cataracts is secondary
to that of the exciting illness, for they are usually operable if the patient
I'êCOVerS.
It is interesting that Farina (1927) produced a cataract in rats after prolonged
starvation, the animals being allowed only water to drink.
Farina. Lett. Oft., iv, 579, 1927.
4. COMPLICATED CATARACT
CATARACTA CoMPLICATA was a concept introduced by Becker (1876)
to embrace the lenticular changes which frequently appear in various intra-
ocular diseases as “retinal detachment, intra-ocular tumours, cysticercus,
absolute glaucoma, cyclitis, irido-cyclitis, and the unknown processes which
result in buphthalmos and other ectatic processes in the eyes '': his concep-
tion still holds although its basis has been broadened. The characteristic
clinical features of such cataracts with their typical rosette-formations in
the anterior and posterior cortex were detailed with great exactitude by
Fuchs (1910), but it is to Vogt's (1919) studies with the slit-lamp that our
knowledge of their exact morphology is essentially due.
AEtiologically in the majority of cases a complicated cataract is presum-
ably due to a derangement of the metabolism of the lens by the diffusion
into it of toxins from the intra-ocular fluid ; and owing to the thinness of the
posterior capsule and its lack of a supporting epithelial barrier, the earliest
DISEASES OF THE LENS 3225
clinical changes are seen in the region of the posterior pole (Figs. 2752–53).
At this point faint flaky opacities appear immediately underneath the
capsule, which even before they themselves can be distinguished by the slit-
Fig. 2752. PostERIok CorticAL Fig. 27.53.−PostERior ConticAL
AND CAPsulAR CATARACT. CATARACT.
Seen with the loupe: early stage Seen with the loupe: advanced
(Harrison Butler). stage (Harrison Butler).
lamp, give rise to a polychromatic lustre, whereby the normal whitish sheen
of the zone of specular reflection (Fig. 1052, Plate VIII') is replaced with
an iridescence in which are grouped brilliant colours of red and blue
(Fig. 2727, Plate LXIII). As the
opacities in this region progress, they
usually take the form of a rosette
(Fig. 2754), and when they become
sufficiently dense they assume a tufted
or breadcrumb-like appearance: at this
stage in the thicker central region the
polychromatic lustre is lost and remains
only in the attenuated peripheral
parts of the rosette. As opacifica-
tion proceeds, it usually progresses in
two directions by sudden leaps; the
opacities spread axially towards the
centre of the lens, and also in a
peripheral direction in concentric layers
parallel to the posterior surface. As ºº showing typical
they develop they become larger,
denser and more yellow, and are always preceded by a whitish cloudy haze.
Frequently a new centre appears behind the posterior band of the adult
1 Vol. II.
Fig. 2754.—CoMPLICATED CATARACT.


3226 TEXT-BOOK OF OPHTHALMOLOGY
nucleus which acts as a second centre of opacification, and spreads in
a manner similar to the first and eventually the same changes, preceded
by the typical polychromatic lustre and developing into the formation of a
rosette, commence at the anterior pole. Thereafter
opacification of the entire lens slowly evolves.
The diagnosis of a complicated cataract is of
importance, since the question of its operability opens
up considerations wider than the state of the lens
itself; but fortunately its recognition is rendered
easy by the two characteristics which distinguish it
from all other types—the presence of a polychromatic
lustre in the early stages, and the fact that the opacities
are never clearly demarcated from the surrounding tissue
but are submerged in a cloudy haze. It is of great
ºº: ººººººººººº importance also that the slit-lamp reveals the charac-
CATARACT (TRAUMATIC). - - - - - - - - -
Optical section of pos- teristic lustre before any opacities are visible with
terior hole. the ophthalmoscope, so that a certain diagnosis can
be made from the earliest stages.
The two most important points in differential diagnosis are a cupuliform senile
cataract and a traumatic cataract. With regard to the former, the opacities are
dome-shaped and not arranged in a rosette, the thickness is uniform throughout and
Fig. 2756.-CoMPLICATED CATARAct
Shrunken lens with plicated capsule after irido-cyclitis (Harrison Butler, T. o. S.).
not increased in the axial region, there is no polychromatic lustre, and there is no
enveloping cloudy haze. Traumatic cataract, instead of assuming a rosette-form,
follows the design of the lens fibres with a feathery pattern, and here, too, the patho-
* Vol. IV.


DISEASES OF THE LENS 3227
gnomonic lustre is absent. It must be remembered, of course, that in old people a
complicated cataract is usually associated with pre-existing senile opacities in the
lens which may to some extent obscure the characteristic picture.
Pathologically also the lens presents a characteristic picture
(Wagenmann, 1891; Treacher Collins, 1896; Fuchs, 1906–17 : Burkart,
1909; Schlippe, 1910; v. Hippel, 1918; Schall, 1921; v. Szily, 1938; and
others). In the earlier stages the changes are typical of those of cataract
generally although usually limited initially to the posterior cortex (Fig. 2757).
In most cases, however, the whole lens becomes opaque, but shows as a rule
Fig. 2757-PostERIoR CorticAL CATARAct (x 60) (Parsons).
more profound degenerative changes than a simple senile cataract, and in the
later stages liquefaction, cholesterol deposition and calcification are prominent
while the capsule becomes thickened and the whole lens becomes shrunken,
distorted and tremulous (Fig. 2756). The most characteristic feature,
however, is the thickening of the capsule associated with a proliferation of
the epithelium which almost invariably multiplies to cover the entire posterior
surface so that a continuous layer lines the capsule without interruption, a
process which is most evident when the lens has become embedded in a mass
of organized cyclitic tissue. The new epithelium on the posterior surface
continues to grow, suffering vesicular enlargement of the individual cells
(Wedl's vesicle cells)"; these cells, separating and penetrating into the
cortex, form a characteristic feature of such cataracts (Fig. 2758). In those
cases, however, in which the lens is surrounded by granulation tissue, or
* pp. 3103, 3172.

3228 TEXT-BOOK OF OPHTHALMOLOGY
becomes embedded in pus, as in a panophthalmitis, this proliferative activity
does not appear, and the capsule itself may become completely absorbed.
In this event the barrier to the entrance of inflammatory tissue into the
Fig. 2758.-CoMPLICATED CATARAct.
Advanced stage. Flattened anterior capsular cataract. Wandering of epithelial
cells to posterior surface. Wedl’s vesicle cells and calcification (v. Szily, T. O. S.).
lens is broken, and vascularized fibrous tissue may form and ossification
ultimately develop."
While a complicated cataract may occur in innumerable intra-ocular
conditions, several types may be differentiated.
(i) CATARACT Associated witH IRIDo-CYCLITIs
The cataract which complicates irido-cyclitis is very typical, particularly
that seen in sub-acute inflammations and sympathetic ophthalmitis. It
comes on slowly, usually after the uveal disease has existed for some years,
and is frequently preceded by the organization of posterior synechiae and a
plastering over of the lens by cyclitic exudates. This exudative material
may make observation of the lens difficult or impossible, and its extent
can sometimes only be gauged after an iridectomy has been performed.
The evolution of the cataract is usually slow, and if the uveal inflammation
is brought under control, its progress may stop; but with a persistence of
the cyclitis, opacification of the entire lens proceeds until it finally assumes
a densely white, mother-of-pearl appearance, showing crystalline and
p. 2177.

DISEASES OF THE LENS 3229
calcareous deposits and usually covered with new blood-vessels from the iris
(Figs. 2759, 1855, Plate XXXIV).
The treatment is frequently difficult and depends on the activity of the
uveal inflammation. A considerable amount of vision can sometimes be
attained by an iridectomy at the stage when the lenticular opacities are
mainly central in position, but an extraction operation should seldom be
undertaken while active cyclitis is present or fresh keratic precipitates are
in evidence, lest a recrudescence of the inflammation supervene and vision
be ultimately lost from phthisis bulbi. The prognosis may, however, be
good in carefully selected cases, provided the eye has remained quiet for some
Fig. 2759.-CoMPLICATED CATARACT : IN IRIDo-cyclitis.
time and the tension is good, and provided it has survived a preliminary
operation of iridectomy with impunity. Not only is a preliminary iridectomy
of value as an indication of how the eye may be expected to stand up to
operative procedures, but by breaking down adhesions it prepares the way
for the subsequent extraction. Incidentally, because of these adhesions, an
intra-capsular extraction is contra-indicated in these cases.
(ii) HETERoch Rovic CATARACT, which is associated with an exceedingly
slow and benign inflammation of the uveal tract, has already been discussed,
when the good prognosis of operative treatment has been pointed out."
The localized sub-capsular opacities associated with adhesions of the pupillary
membrane have already been described, when it was pointed out that many such
cases are of inflammatory origin. The opacities associated with posterior synechiae
are of the same nature: they are usually situated eccentrically near the worst foci of
inflammation of the iris; the epithelial proliferation is more active, although the
deposition of hyaline material to form new membranes is less evident.
(iii) CATARAct compIICATING SEVERE corn EAL INFLAMMATION, particu-
larly a hypopyon ulcer, is of much the same type. The localized forms of
1. ------->
p. 2362.

3230 TEXT-BOOK OF OPHTHALMOLOGY
anterior polar cataract and pyramidal cataract which result from a perfora-
tion of the cornea in infancy have already been described an inflammatory
anterior capsular cataract occurring in adult life from the same cause has
the same general histological picture.”
(iv) CHORomo AL CATARACT is the term sometimes applied to the com-
plicated cataract following chronic inflammatory or degenerative processes
in the posterior segment of the eye. Owing to the absence of pupillary
Fig. 2760.-CoMPLICATED CATARAct. Fig. 27.61.-CoM-
- - - - - P. L. I. C. A. T. E. D.
Secondary to chorio-retinitis. CATARACT.
Secondary to
chorio - retinitis :
optical section of
posterior pole.
exudates, this comprises the type of complicated cataract most readily
studied clinically (Figs. 2760–61). The opacification starts at the posterior
pole with a typical polychromatic lustre and proceeds in a rosette forma-
tion, running the usual course of a complicated cataract, and eventually,
often after a very slow evolution, reaches the stage of maturity when the
lens appeals shrunken with a thickened and wrinkled capsule showing
cretaceous deposits, and may eventually become tremulous and dislocated.
Such a cataract may follow any chronic inflammation in the posterior
segment, particularly tuberculous choroiditis. Probably, however, the most
frequent causes are two degenerative conditions—high myopia and primary
pigmentary degeneration of the retina. In high myopia lenticular changes
p. 1359. * p. 3112.

DISEASES OF THE LENS 323]
usually appear between 50 and 60 years of age and resemble to a large extent
the nuclear cataract seen in senile persons: the cataract is of very insidious
Onset and slow evolution, and it is frequently problematical to decide how
far the loss of visual acuity is due to lenticular and how far to macular
changes. In primary pigmentary degeneration of the retina, on the other
hand, the changes are essentially at the posterior pole and have all the
characteristics of a typical complicated cataract. Here again progression is
usually very slow, and, largely owing to the small visual field accompanying
the later stages of this condition, the functional results of operation may be
disappointing.
(v) CATARACT COMPLICATING A DETACHED RETINA has also been noted."
A diffuse opacification of the lens appears which matures rapidly and
usually suffers calcareous degeneration. Not infrequently the lens ultimately
shrinks, becomes tremulous, and may eventually become dislocated.
Extraction is rarely indicated and should be attempted only in two excep-
tional circumstances. The first is the case of a bilateral detachment when
the retention of some of the faculty of projecting light is retained indicating
a partial detachment, in which case a successful operation may result in the
enjoyment of some vision instead of complete blindness. The second is
when the patient desires an operation for cosmetic reasons.
(vi) CATARACT COMPLICATING ABSOLUTE GLAUCOMA is the rule in the
terminal stages of this condition.” The cataract is nuclear in type rather
than cortical, and the lens presents a dull green appearance behind the
widely dilated pupil. It is a degenerative phenomenon occurring in an eye
already blind, and any operative treatment is contra-indicated.
(vii) CATARACT couracºsa AN INTRA-OCULAR TUMoUR is relatively
so common that it is a wise precaution to transilluminate every eye showing
unilateral cataract. The lenticular changes are presumably due to the
absorption of toxic products, but in those cases of ciliary tumour which abut
against the lens itself, the mechanical influence of actual pressure is probably
super-added. Such a tumour may indent the equator of the lens considerably,
deforming it and eventually sub-luxating it, and in these the opacification
starts in, and may remain localized for some considerable time to the area
in contact with the tumour. As a rarity the pressure of the tumour may
rupture the capsule and produce a traumatic cataract (Becker, 1883;
Lange, 1890 ; Mitvalsky, 1894; Treacher Collins, 1896; Groenouw, 1899 ;
Symens, 1901; Nordmann, 1930; Merrill, 1933; and others). Any
treatment of such cataracts, of course, does not enter into the question.
(viii) In HEPATO-LENTICULAR DEGENERATION (PSEUDO-SCLEROSIS), in association
with the characteristic Kayser-Fleischer ring in the cornea,” a sun-flower cataract some-
1 p. 2905. * p. 3367. * Vol. II, p. 2040.
T.O. —WOL. III. 4 D
3232 TEXT-BOOK OF OPHTHALMOLOGY
times occurs, characterized by an anterior sub-capsular opacity with radiating lines
of a grey-blue or grey-green colour (Vogt, 1929; Thiel, 1934). The typical changes
will be described when dealing with copper cataract."
Becker. G-S. Hb., I, v, 227, 1876. Merrill. K. M. Aug., xci, 598, 1933.
2ur Amat. d. gesunden u. kranken Linse, Mitvalsky. A. f. Aug., xxviii, 152, 1894.
Wiesbaden, 1883. Nordmann. Bull. S. d’O. Paris, xlii, 157, 1930.
Burkart. Diss., Freiburg, 1909. Schall. K. M. Aug., lxvii, 584, 1921.
Collins, Treacher. Researches, London, 1896. Schlippe. A. f. Aug., lxvii, 97, 1910.
Fuchs. Z. f. Aug., xv, 191, 1906. Symens. K. M. Aug., xxxix (2), 863, 1901.
Lhb. d. Aug., Wien, 1910. v. Szily. T. O. S., lviii, 595, 1938.
A. f. O., xciii, 381, 1917. Thiel. K. M. Aug., xciii, 12, 1934.
Groenouw. A. f. O., xlvii, 282, 398, 1899. Vogt. K. M. Aug., lxii, 582, 593, 1919
v. Hippel. A. f. Aug., xcv, 257, 1918. lxxxii, 433 ; 1:xxiii, 417, 1929.
Lange. A. f. O., xxxvi, 247, 1890. Wagenmann. A. f. O., xxxvii, 21, 1891.
D Secondary Cataract
(AFTER-CATARACT)
SECONDARY CATARACT (a term sometimes used to connote a complicated
cataract secondary to intra-ocular disease) is best applied to the remnants
of the lens left behind after operative removal of the lens by the extra-
capsular method or after its destruction by traumatism. These remnants
may comprise the following elements :—
1. Capsular remains, particularly of the posterior capsule, which may
be very diaphanous and thin. Associated with the capsule is the sub-capsular
epithelium which almost always shows some proliferation and may occasion-
ally give rise to the large globular bladder-cells of Elschnig (1911).
2. Capsulo-lenticular remains in which a mass of lens fibres are associated
with the capsule. These may be arranged irregularly, or may be amassed
in the periphery, where they form a ring enclosed between the anterior and
posterior capsules leaving the pupillary area relatively free (the ring of
Soemmering, 1828).
3. Pigmentary, haemorrhagic or inflammatory elements may form
organized masses in addition to the capsular and lenticular remains.
In their slighter degrees capsular remains may be quite invisible to the
ophthalmoscope and may not interfere in any way with vision, but they are
always distinguishable with the slit-lamp (Fig. 2762). The pupil may
remain perfectly active and regular, and there is a fine veil-like curtain
separated by a clear space from the iris in front and again from the anterior
face of the vitreous behind. This always exhibits numerous crinkles and
folds and usually a polychromatic lustre. In the more marked degrees there
may be a considerable amount of remains of the cortical fibres of the lens,
arranged usually in bundles of a snowy colour with a frayed appearance,
1 Vol. IV.
DISEASES OF THE LENS 3233
and such remnants, sometimes enclosed between folds of the anterior and
posterior capsules, may form bands of considerable thickness and strength
(Fig. 2763).
Pathologically the anterior capsule is seen to become retracted and
folded upon itself, the edges of the wound in the anterior capsule becoming
adherent to the posterior capsule so that a ring of folded capsule containing
a varying quantity of lens substance tends to form and persists behind the
iris (the RING or cushion of sorºmmerING, 1828) (Fig. 2764). The central
part occupying the pupillary area is usually occupied by a thinner membrane
consisting essentially of the posterior capsule to which may be added fibrous
FIG. 2762.-SEcond ARY CATARACT. FIG. 2763.-SEcoRDARY CATARACT.
The posterior capsule after a The two capsules after extra-capsular
vertical discission. The vitreous is removal of the lens (Harrison Butler,
seen behind. T. O. S.).
tissue laid down by fibroblasts from the iris. As a rule there is a consider-
able amount of lens fibres enclosed in the folded capsule behind the iris, and
the ring-shape is particularly evident if the centre of the lens has been
penetrated and has become absorbed either by a discission operation or by
an accident; it results, therefore, in its most typical form from a needling
operation for congenital cataract or from Fukala’s operation in high myopia.
Pathological specimens sometimes show lens fibres relatively intact, but
more usually they are grossly degenerated, breaking down into Morgagnian
globules and amorphous débris, and showing areas of calcareous degeneration
(Fig. 2765).
So long as such a secondary cataract remains in situ, no symptoms
arise and vision is maintained through the axial aperture, but particularly
in myopic eyes, in which the zonule is stretched and degenerated and
the vitreous is fluid, it may break loose. This interesting complication may
occur, usually with but sometimes without a history of trauma, to any

4 D 2
3234 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2764–Ring of Soemmering.
In its normal position after extra-capsular cataract extraction (Tooke, Brit. J. O.).
after-cataract, but particularly when it assumes the form of a ring of
Soemmering, so that it is dislocated either into the vitreous (Wessely,
1910; Poos, 1931) or through the pupil into the anterior chamber, where
it may lie like a tiny white pessary (Schneider, 1927; Jess, 1930; Tooke,
1933; Jacoby and Wolpaw, 1935). A dislocation anteriorly is usually
Fig. 2765-Secondary Cataract.
Cortical remains consisting of lens fibres, some of which are intact, but many
of which form tumescent spaces filled with broken down amorphous débris. Note
the separation of the zonular lamella (Tooke, Brit. J. O.).


DISEASES OF THE LENS 3235
followed by a sharp inflammatory reaction with raised tension, but successful
removal of the ring by forceps after corneal section may be followed by an
excellent result (Jess, 1931; Tooke, 1933), although if much degeneration
FIG. 2766.-SEcox DARY CATARACT.
Showing proliferation of cuboidal epithelial cells (Tooke, Brit. J. O.).
has occurred, it may behave on manipulation much as a Morgagnian cataract
and melt away in milky fluid. A dislocation posteriorly, on the other
hand, may be followed by no symptoms or impairment of vision (Poos, 1931).


3236 TEXT-BOOK OF OPHTHALMOLOGY
Associated with these passive remnants there is considerable prolifera-
tive activity, sufficient, indeed, to suggest to the older writers the conception
of a “regeneration of the lens” (Milliot, 1872; Textor, 1872; Baas, 1899).
This phenomenon occurs preferentially, but by no means exclusively, in
younger patients, and concerns especially the sub-capsular epithelium which
in time may line the entire posterior capsule (Becker, 1883; Wagenmann,
1889–91; and others), and, turning round the torn margins, covers both
sides of the anterior capsule (Cowan and Fry, 1937). This activity may be
associated with the deposition of hyaline capsular material forming irregular
Fig. 2768–SEcondary CATARAct.
Cystic proliferation of epithelium.
masses and membrane-like strata (Wagenmann, 1889; Hocquard, 1902);
while in other localities the cells may be several layers thick, in which case
each layer may be separated by loose interlacing bands of hyaline tissue
similar to that of the capsule (Fig. 1766). Frequently the individual cells
become swollen and vacuolated, and eventually may attain considerable
dimensions (Hirschberg, 1901; Elschnig, 1911): Cowan and McDonald
(1939) noted their presence in some 25% of cases. Such cells, which are
usually termed the GLoBULAR or BLADDER CELLs of Elsch NIG, are
reminiscent of the vesicular cells of Wedl, and probably represent aberrant
attempts of the epithelium to form new fibres. They are rounded or oval
and perfectly transparent, like toy balloons or soap bubbles (ELschinig's
PEARLs) (Fig. 1767); they may be single or arranged in clumps like

DISEASES OF THE LENS 3237
bunches of grapes or masses of frog's spawn, and may eventually occlude
the entire pupillary aperture. Single cells may attain a considerable size
(2 mm., Cowan and Fry, 1937). The picture they produce may vary from
time to time ; sometimes they appear a few days after operation, and
continue to form and vanish Over a period of years. Pathological examina-
tion shows them to be large vesicle-like structures enclosed by an extremely
attenuated wall. At other times much more solid opaque cysts of capsular
material are formed which may eventually burst spontaneously (Fig. 1768).
Pigmentary remains in the form of a fine, brown, powdery deposit from
the iris on the membrane formed by a secondary cataract is a commonplace,
even although the extraction has been uncomplicated and has been followed
by little or no post-operative reaction : it is due to a dispersion of the
pigment following a traumatic disintegration of the pigment epithelium
lining the posterior surface of the iris and ciliary body. An extremely
interesting phenomenon, however, is the occasional active and abundant
proliferation of the pigmented cells which may become intermingled with
the lenticular remains sometimes forming large pigmented plaques, an occur-
rence sometimes particularly evident in diabetic cataract (Brückner, 1919;
Fuchs, 1920; Sgrosso, 1932; Mans, 1933).
A more serious occurrence is the incorporation of organizing material
from post-operative haemorrhage or cyclitic exudates into the secondary
cataract. In the latter event especially, if the cyclitic process remains
active, increasing amounts of plastic exudate and cellular infiltration occur,
to be followed by fibroblastic invasion and cicatrization, so that a dense and
completely opaque mass occupies the entire pupillary area. In such cases,
of course, the functional value of the eye may well be lost owing to the
general disintegration of the globe in phthisis bulbi.
The prevention of an after-cataract is of the greatest importance, an ideal
which, of course, is most readily attained by an intra-capsular extraction.
It is to be remembered, however, that even when the lens is removed
completely in its capsule, some degree of condensation and clouding of the
anterior face of the vitreous eventually occurs." If an extra-capsular operation
has been decided upon, the amount of lenticular remains is minimized if
the operation is postponed until the cataract is mature, a consideration,
however, which cannot over-ride economic necessities or the unhappiness
of a long period of virtual blindness. During the operation a free incision
of the anterior capsule or the removal of a portion of it by forceps lessens
the tendency to secondary complications, but more important is the assiduous
removal of soft cortical material by lavage. The avoidance of post-operative
haemorrhage or inflammation, and the rigorous exclusion of foci of autogenous
infection are of the greatest importance, as well as prolonged post-operative
atropinization ; short wave diathermy is also frequently of considerable
value in promoting rapid absorption of cortical remains.
1 p. 3276.
3238 TEXT-BOOK OF OPHTHALMOLOGY
The treatment of a secondary cataract is entirely surgical—the division
of the membrane or of bands by a knife, a knife-needle or scissors, or their
complete excision.” In all cases it is important that manipulations should be
as delicate as possible, both to avoid a retinal detachment and subsequent
post-operative inflammatory reaction with the reformation of plastic
exudates. The division of an elastic or leathery membrane may be a difficult
and tricky proceeding, and it is better to open the eye and excise the mem-
brane, or, if the pupil is drawn peripherally, to perform an iridotomy, than
to use unjustifiable force. The time of intervention is also of importance. A
discission should not be done until all post-operative reaction or inflamma-
tion has completely disappeared, the eye shows no congestion and the
aqueous is clear from excess protein, cells and débris. These considerations
become more important in the case of complicated cataracts, for a premature
attempt to create a gap in the pupil in such cases will almost certainly be
followed by its re-closure by plastic exudate so that the last condition is
worse than the first. The repeated formation of bladder-cells may constitute
an annoying therapeutic problem necessitating several operations ; in these
cases the growth may be inhibited by a short exposure to X-rays.
Baas. Münch. med. W., ii, 1609, 1899. Mans. K. M. Aug., xc, 650, 1933.
Becker. Zur Amat. d. gesunden u. kranken Milliot. J. Amat. Phys., viii, 1, 1872.
Linse, Wiebaden, 1883. Poos. K. M. Aug., lxxxvi, 449, 1931.
Brückner. K. M. Aug., lxii, 461, 1919. Schneider. Am. J. O., x, 273, 1927.
Cowan and Fry. A. of O., xviii, 12, 1937. Sgrosso. Boll. d’Oc., xi, 737, 1932.
Cowan and McDonald. A. of O., xxii, 1074,
1939.
Elschnig. K. M. Aug., xlix, 444, 1911.
Fuchs. K. M. Aug., lxiv, 1, 1920.
Gonin. Beit. 2. path. Amat., xix, 497, 1896.
Hirschberg. Einführung in d. Augenh.,
Leipzig, 159, 1901.
Hocquard. A. d’O., xxii, 435, 1902.
Jacoby and Wolpaw. A. of O., xiii, 634, 1935.
Jess. Kurzes Hb. d.O., Berlin, v, 248, 1930.
K. M. Aug., lxxxvi, 98, 1931.
Soemmering. Beobachtungen u. d. organischen
Weränderungen im Auge mach Staropera-
tionen, Frankfurt, 1828.
Textor. Diss., Würzburg, 1872.
Tooke. Brit. J. O., xvii, 466, 1933.
Vannas. Acta O., iv, 262, 1927.
Wagenmann. A. f. O., xxxv, 172,
xxxvii, 21, 1891.
Wessely, A. f. Aug., lxvi, 277, 1910.
1889 :
IV. DISPLACEMENTS OF THE LENS
Normally the lens remains suspended by the fibres of the suspensory
ligament so that its axis corresponds with the visual line, an alignment
constantly maintained by the tautness of this ligament in all circumstances.
We have already seen that congenital anomalies in the zonule lead to
ectopia of the lens; acquired abnormalities also occur either through
trauma or disease, whereby the suspensory apparatus is weakened so that
the lens becomes tremulous or sub-luxated, or is ruptured so that a complete
dislocation occurs.
1 Vol. IV.
DISEASES OF THE LENS 323.9
Atiology. Displacements of the lens may be classified aetiologically as
follows:–
(a) congenital’;
(b) traumatic * :
(c) consecutive or spontaneous, arising from intra-ocular disease:
(i) from mechanical stretching of the zonule;
(ii) from inflammatory disintegration of the zonule:
(iii) from degenerative conditions of the zonule.
Displacements owing to mechanical stretching of the zonule are seen
most commonly in conditions such as buphthalmos (v. Hippel, 1897),
staphylomata or ectasias of the globe (Panas, 1894), or in high myopia
(Wagenmann, 1889; Halben, 1897; Ringelhan and Elschnig, 1931). A
similar accident may also follow the sudden strain resulting from the
Fig. 2769.-Spont'ANEous SUB-LuxATION of LENs.
Showing two areas of vitreous prolapse (Bedell, T. O. S.).
perforation of a large central corneal ulcer, when, indeed, the lens may be
completely extruded from the eye. Inflammatory adhesions may also
pull the lens out of position, while intra-ocular tumours may push it out of
place. Inflammatory destruction of the zonule occurs in panophthalmitis
when the fibrillar structure may be digested by leucocytes and liquefied ;
moreover, the same process occurs less dramatically in chronic inflammations
when the zonule is invaded by cyclitic granulation tissue (v. Michel, 1906).
* Vol. IV, p. 1349. * See Injuries: Vol. IV.

3240 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2770. Sub-Luxation of LENs.
There is no trace of a zonule; vestigial remains of the pupillary membrane are
evident (Harrison Butler).
More usually, however, the cause is a degenerative or atrophic condition
of the zonular fibres, a circumstance which, in view of the common origin
of the two structures, is usually accompanied by degeneration and liquefac-
tion of the vitreous gel. This occurs particularly in high myopia, old
Fig. 2771-DETAcHMENT or Zonullan LAMELLA.
After a blow : the zonular lamella is torn from the sub-luxated lens and folded
back and into it are inserted the zonular fibres (Harrison Butler, T. O. S.).


DISEASES OF THE LENS 3241
choroiditis or cyclitis, or in detachments of the retina, or merely from
gradually increasing senile degenerative changes producing fragility in the
suspensory apparatus. Finally, in hypermature cataracts the degenerative
changes spread to the Zonule, and the fact that dislocation of such
lenses frequently occurs spontaneously, or with great readiness in the
manipulations during their removal, constitutes an argument of considerable
weight against leaving a hypermature cataract to reach the shrunken stage.
Once the degeneration of the zonule has occurred the actual dislocation
of the lens may occur spontaneously by its own weight, or by some trauma,
frequently quite insignificant in degree. On dislocation the Zonule may be
preserved attached to the lens, in which case the fibres undergo a degener-
tion which renders them progressively more opaque (Fig. 2769). A detach-
ment at the ciliary insertion, however, is rare ; more usually the detachment
occurs at the zonular lamella and the edge of the lens shows no fringe of
fibres (Fig. 2770). In this event the whole suspensory apparatus is drawn
behind the iris, although occasionally a portion of the suspensory ligament,
having lost its ciliary attachment, is seen floating about associated with
a fine pellucid membrane (the zonular lamella) (Fig. 2771) (Meesmann,
1922; Maggiore, 1924; Jess, 1926; Stein, 1926; Busacca, 1927).
Displacements of the lens may be classified topographically as in the
following scheme —
A. SUB-LUXATION, the lens remaining in the pupillary aperture.
B. LUxATION or DISLOCATION, when the lens is completely displaced from the
pupil, in which case it may appear :
(a) in the anterior chamber ;
(b) in the vitreous, when it may either
(i) float about—lens matans, or
(ii) become anchored—lens fia’ata ;
(c) in the sub-retinal space, passing through a retinal tear ;
(d) it may wander from one locality to the other ;
(e) it may be extruded out of the globe, either partially (phakocele or lenticele),
or completely, either through a perforating corneal ulcer, or through a
traumatic rupture of the sclera, in which case it may rest sub-conjunctivally
or under Tenon’s capsule.
SUB-LUxATION or PARTIAL DISLOCATION of the lens gives rise to visual
symptoms varying in degree depending on the position occupied. If the
lens remains in the primary axis, the sole result may be the development of a
lenticular myopia, since the curvature increases owing to relaxation of the
suspensory apparatus. Alternatively, however, it may be tilted on a vertical,
horizontal or oblique axis, resulting in a marked astigmatism, which is
always difficult and usually impossible to correct adequately with glasses.
More usually, however, the lens becomes laterally displaced, in which event the
images are completely distorted by the myopic astigmatism of the equatorial
3242 TEXT-BOOK OF OPHTHALMOLOGY
region. If the displacement is sufficient to become apparent in the pupil so
that the lens occupies part of the pupillary aperture while the other part is
aphakic, such a sub-luxation is accompanied by monocular diplopia, an
indistinct image being seen through the aphakic part, and a distorted image
through the phakic part. The condition may be rendered still more difficult
optically if the lens moves with alterations in the position of the head, for
then varying confusion images are produced.
The objective signs accompanying such a sub-luxation are usually
obvious. The anterior chamber is deep, and if some rotation of the
lens has occurred the depth may vary in different sectors. Owing to the
lack of its usual support any movement of the eyes gives a readily marked
tremulousness to the iris (iridodonesis). In focal illumination the lens
appears grey, particularly in the equatorial region, owing to the increased
internal reflection of light, and by ophthalmoscopic examination its edge,
if it occupies the pupillary aperture, appears as a black crescent owing to
the total reflection of the light coming from the fundus (Fig. 1070, Plate IX)".
With the slit-lamp the zonular fibres may be seen, sometimes stretched and
sometimes torn, and in the aphakic area the anterior face of the vitreous may
herniate forwards. Ophthalmoscopically a double image of the fundus may
be seen, one through the phakic and the other through the aphakic area of
the pupil.
Such a sub-luxated lens may remain in the pupillary aperture
indefinitely, giving rise to no symptoms other than the optical disability.
Occasionally, although relatively rarely, it eventually turns opaque, but
more usually it gives rise to an irritative irido-cyclitis, or a secondary
glaucoma.” A further complication is its complete dislocation.
The treatment depends on the state of the eye and the vision. If the
eye is quiet and the vision of the other eye normal, the condition is usually
best left alone. If the vision of the other eye is poor, an attempt at optical
correction should be made, utilizing either the phakic or the aphakic area
and neglecting the other. It frequently happens that owing to the irregu-
larity of the astigmatism in the phakic area, the latter affords the best
results, and if the available aperture is small, these may be improved by the
dilatation of the pupil with weak atropine in young persons or by an
iridectomy in the aged. Discission of such lenses in young people is usually
difficult, but may be accomplished with two needles ; in those cases,
however, absorption of the lens is frequently very slow and unsatisfactory,
but the happy result may sometimes ensue that the needled lens turns
opaque and shrinks, thus eliminating one of the confusion images as well as
enlarging the aphakic area and allowing better visual results. In older
people, if painful symptoms ensue, the best operation is usually an intra-
capsular extraction preceded by a wide preliminary iridectomy, the lens
being supported from behind with a scoop during its delivery : the operation,
1 Vol. II. * p. 3307.
DISEASES OF THE LENS 3243
however, is not without its hazards and is usually associated with loss of
vitreous.
DISLOCATION OF THE LENS is a more serious matter than its sub-
luxation. In this case the vision corresponds to that of an aphakic eye, and
the absence of the lens from the pupillary aperture is recognized by the
deep anterior chamber, the tremulousness of the iris, the absence of
lenticular reflexes on focal illumination, and the direct observation of the
anterior face of the vitreous by the slit-lamp.
If the lens is dislocated into the anterior chamber it can be recognized
initially as a clear body in the deepened chamber appearing more convex
and smaller than ordinarily. If the lens is clear it looks like a drop of oil,
and if it is opaque, it appears as a white disc. In all cases complications
tend to arise, the most prominent of which are a severe irritative irido-
cyclitis, a corneal dystrophy resulting in an opacity probably from a
disturbance to the endothelium, and an acute and intractable secondary
glaucoma (Burk, 1912; Wagenhäuser, 1913; and others)." In these cases it
is interesting that miotics frequently raise the tension still further while
mydriatics promptly reduce it (“ glaucoma inversum ”). The only effective
treatment is surgical removal of the lens, a difficult procedure ; and, failing
that, enucleation of the eye.
A posterior dislocation is usually tolerated better than an anterior
displacement, but eventually the vision is usually lost. Clinically the same
appearance of an aphakic pupil and a tremulous iris with a deep anterior
chamber presents itself, and on ophthalmoscopic examination the lens may
be seen, either as a clear globule with a dark margin or as an opaque
cataractous body, lying usually in the lower part of the vitreous or resting
upon the ciliary body. At first it is usually movable, but eventually
organized membranes tend to anchor it. Provided the capsule has not been
ruptured the eye may tolerate the lens in this position without apparent
ill-effects for some years, but, as in the operation of couching,” degenerative
changes éventually destroy vision (Werncke, 1903; Sievert, 1903); more-
over, if the capsule is ruptured so that lens matter escapes, or if mechanical
irritation of the ciliary body becomes evident, a severe irido-cyclitis with
raised tension usually results (Elliot, 1919), and occasionally sympathetic
ophthalmitis (Dor and Paufique, 1932). The only available treatment
which may give relief, apart from enucleation of the eye, is removal of the
lens. Occasionally it can be coaxed into the anterior chamber by laying
the patient on his face, where it may be imprisoned by contracting the pupil
with eserine (WANDERING LENs). Its removal from this position is easier,
but the manipulation is not usually successful. Removal from the vitreous
is an operation of considerable hazard and is always associated with much
loss of vitreous : perhaps the easiest method is, under complete akinesia,
1 p. 3308. * p. 3183.
3244 TEXT-BOOK OF OPHTHALMOLOGY
to transfix the lens with a diathermy needle, induce coagulation until the
needle is firmly embedded in the lens and then to extract it."
A unique case was reported by Fralick (1937), in which a congenitally
sub-luxated lens in a patient's only eye became dislocated and eventually
travelled through a large retinal tear to come to rest in the sub-retinal space.
After the patient had lain face downwards for 24 hours, the lens appeared
in the anterior chamber, whence it was extracted, but unfortunately a
subsequent operation for re-attachment of the retina proved unsuccessful.
Dislocation into the sub-conjunctival space or under Tenon's capsule through a
rupture of the limbus occurs only after trauma “: but a partial or complete extrusion
of the lens may occur after the perforation of a large central corneal ulcer.
TREMULOUSNESS OF THE LENs on movements of the eye is a rarity, but
has been observed in a mature cataract (d’Ombrain, 1936): the phenomenon
is probably due to a degenerated suspensory ligament and the presence of a
fluid vitreous.
Burk. A. f. O., lxxxiii, 114, 1912. v. Michel. Gedenkschrift f. v. Leuthold, ii, 617,
Busacca. Zb. ges. O., xviii, 433, 1927. 1906.
Dor and Paufique. Bull. S. O. Paris, xliv, d'Ombrain. Brit. J. O., xx, 22, 1936.
434, 1932. Panas. Traité, Paris, 1894.
Elliot. Brit. J. O., iii, 49, 1919. Ringelhan and Elschnig. A. f. Aug., civ, 325,
Fralick. Am. J. O., xx, 795, 1937. 1931.
Balben. Diss., Jena, 1897. Sievert. Diss., Freiburg, 1903.
v. Hippel. A. f. O., xliv (3), 539, 1897. Stein. K. M. Aug., lxxvi, 75, 1926.
Jess. K. M. Aug., lxxvi, 465, 1926. Wagenhäuser. K. M. Aug., li (1), 619, 1913.
Maggiore. An. di Ott., lii, 817, 1924. Wagenmann. A. f. O., xxxv (1), 172, 1889.
Mees.Inann. A. f. Aug., xci, 261, 1922. Werncke. K. M. Aug., xli., Beil., 283, 1903.
* See Operative Surgery: Vol. IV. - * See Injuries to the Eye: Vol. IV.
CHAPTER XXXIX
DISEASES OF THE WITFEOUS BODY
I. GENERAL CONSIDERATIONS
IN the section on Anatomy we have seen + that in the normal state the
vitreous body is a clear homogeneous gel formed as a cell-product and can
in no sense be considered as an integrated tissue. We have also seen that
physiologically it is completely inert “; for the lack of metabolism is
demonstrated by the absence of any gaseous exchange both in vitro and in vivo
(Fischer, 1929–32). It follows that in its pathological reactions it is entirely
passive, so that the old terms such as “hyalitis '' (Schmidt-Rimpler, 1878 ;
Straub, 1896–1913) connoting pathological activity should be avoided.
Moreover, when lost it is not renewed but is replaced by intra-ocular fluid
(Ogawa, 1906 ; and others). Although its reactions are therefore merely
degenerative, involving changes such as liquefaction, opacification, shrinkage,
and so on, the very delicate balance of its colloidal structure is so readily
disturbed by a great variety of changes, such as an alteration in the reaction,
the carbon dioxide tension, the salt content, and the infiltration of toxins,
that it shares in a large number of diseases of the inner eye.
It is to be remembered that although it is inert and takes no active part in inflam-
matory reactions, the vitreous body forms an excellent culture-medium so that any
infective process reaching it tends to run on apace. This lack of resistance to organismal
growth has been stressed by many investigators, who have found that bacteria readily
multiply in it and develop a severe inflammatory reaction in the surrounding tissues
which readily proceeds to abscess formation, a generalization which applies even to
organisms of so low a degree of pathogenicity that elsewhere in the eye they would
merely remain encapsuled (B. subtilis, sarcinae, etc.) (Pagenstecher, 1869; Gifford,
1886; Deyl, 1893; Perles, 1895; Cronstedt, 1923; Gray, 1933; and many others).
The pathology of these cases has already been discussed in dealing with inflammations
of the uveal tract : the complete passiveness of the vitreous body in the process is
the feature of present interest.
In recent years the use of the slit-lamp has greatly increased our oppor-
tunities for studying these degenerative changes, for by ophthalmoscopic
examination the vitreous appears quite transparent and the only visible
alteration which can be observed is the presence of opacities sufficiently
dense to obstruct transmitted light. With the slit-lamp, however, to which
the anterior segment of the vitreous is readily accessible and with which the
entire mass can be seen with the aid of a contact glass, the arrangement of
the micellae which make up its protein basis endows it with a complicated
1 Vol. I, p. 111. * Vol. I, p. 467 (473).
3.245
3246 TEXT-BOOK OF OPHTHALMOLOGY
optical structure of pseudo-fibres and pseudo-membranes of an extremely
polymorphous nature, which appear to hang like delicate gossamer curtains
of moiré silk full of folds and pleats moving slightly and characteristically
in a pendulum-like motion with movements of the eye. We have already
discussed the physical basis of this optical effect and have seen that while
a fibrillar structure was accepted by the older anatomists (a deduction from
the artefacts of histological coagulation) and a membranous lamellar consti-
tution has been claimed by some more recent workers (Friedenwald and
Stiehler, 1935), the probability is that these complex appearances merely
represent the optical effect in the beam of light produced by a varying
arrangement of the countless colloidal micellae which make up the gel. These
fibrillary particles which form the basis of the vitreous consist of a minute
protein residue which adsorbs an enormous quantity of water so that they
swell up to form an elastic gel possessed of a definite consistency. In places
where these fibrillae are arranged indiscriminately the vitreous appears
optically empty; but if they are arranged in parallel they reflect the light
sufficiently to give the appearance of a fibrillary membrane, a summation
effect seen most clearly in places where they are particularly condensed,
such as the interfaces within the vitreous body, on the outer surface, where
indeed the condensation layer possesses sufficient tensile strength to justify
the clinical term hyaloid “ membrane,” and, pre-eminently, in that very
condensed portion which forms the “fibres’’ of the zonule (Duke-Elder,
1930). Although it must be pointed out that this view of the intimate
structure of the vitreous body has not yet been adequately proved and is
not universally accepted, a study of the changes in these optical phenomena,
no matter what physical interpretation be given to them, provides informa-
tion of considerable clinical value, not only regarding pathological processes
occurring in the eye itself but also in many cases regarding the general state
of bodily health.
Cronstedt. Svenska lak. Hand., xlix (3), 157, Gray. Brit. J. O., xvii, 15, 1933.
1923. Ogama. A. f. Aug., lv., 91, 1906.
Deyl. Böhmische Akad. d. Wiss., 1893. Pagenstecher. A. f. Aug., i (2), 1, 1869.
Duke-Elder. The Nature of the Vitreous Perles. A. path. Anat., czl, 210, 1895.
Body, London, 1930. Schmidt-Rimpler. B. O. G. Heidel.., xi, 100,
Fischer. A. f. Aug., cii, 146, 1929; cvi, 463, 1878.
1932. Straub. B. O. G. Heidel.., xxv, 108, 1896.
Friedenwald and Stiehler. A. of O., xiv, 789, Z. f. Avg., ii, Erg., 1, 1899.
1935. T. O. S., xxxii, 60, 1912.
Gifford. A. of O., xv, 180, 1886. A. f. O., lxxxvi, 1, 1913.
II. DEGENERATIONS OF THE WITREOUS BODY
FLUIDITY OF THE WITREous (SYNCHISIs)
A liquefaction of the vitreous body (SYNCHISIS, oùv, together: Xèa, to pour)
is the most common feature of degeneration, occurring in senile and myopic
DISEASES OF THE WITREOUS BODY 3247
conditions and after contusions as well as in most degenerative and inflam-
matory states of the eye. It is due to a conversion of the colloid gel into a
sol and is usually associated with the development of ophthalmoscopically
visible opacities formed by the colloidal micellae which aggregate together
in dust-like particles, strands, or membranes. In a sense it is probably a
dehydration rather than a hydration in so far as it represents the loss by the
micellae of their water of adsorption which becomes free intra-ocular fluid
leaving the protein residue shrunken and agglutinated. Such a break-down
may be due to purely physico-chemical factors but may also be conditioned
by enzymic digestion of the protein basis of the gel.
In simple cases the only clinical symptom is the appearance of floating
spots before the eyes, which instead of having a localized excursion as occurs
when the elastic structure of the gel is maintained, become freer and untram-
Fig. 2772.--THE Norm AL WITREous. Fig. 2773-Liquºr Action of
Seen by the slit-lamp. The THE WITREous.
posterior surface of the lens is to the Seen by the slit-lamp. A senile
left, behind which is a relatively case. The gel is fluid and shows a
optically empty space occupied by homogeneous fibrillar degeneration.
primary vitreous. Posteriorly dense
membranous-like masses of gel are
seen.
melled, moving right across the field of vision. Ophthalmoscopically the
actual liquefaction produces no visible alteration, and the vitreous may
appear normal but for the fact that the opacities, if they are visible, are
possessed of abnormally free and unrestrained movement. With the slit-
lamp, however, the change in consistency is always obvious. In uncompli-
cated cases an absence of the usual curtain-like membranes is evident, and
the whole body of the vitreous tends to become homogeneous, the primary
and secondary elements being mixed so that no relatively clear retro-lental
space (occupied normally by the primary vitreous) exists, for the mass of
vitreous comes forward immediately behind the lens. This mass, instead of
showing membranous formations and folds undulating with a restricted
excursion in an optically clear gel, tends to be interspersed with opacities of
relatively uniform distribution, floating about with much greater freedom
(Figs. 2772–73).
At other times a partial liquefaction occurs, cavities appearing usually
in the central area, or the posterior region turning fluid. In the first case
T.O.-WOL. III. 4 E

3248 TEXT-BOOK OF OPHTHALMOLOGY
the patient complains of a speck or “fly ’’ which appears suddenly in front
of his eye and floats about with a limited excursion. In the second case,
which is a relatively common occurrence owing to the delicacy of vitreous
frame-work posteriorly, the whole of the protein basis of the gel tends to be
drawn anteriorly where it becomes condensed into membranous formations
of considerable density. It will be remembered that it is fixation of these
to the retina and the traction which they exert as they wave about with
movements of the eye which are believed by many authors to be causal
factors in determining the tears in this tissue which lead to its detachment,
particularly in myopic eyes where the vitreous is fluid."
Fluidity of the vitreous calls for no treatment ; its clinical significance
lies in the demonstration of degenerative changes in the eye, and in the
possibility of serious complications occurring in intra-ocular operations, for
in these eyes the zonule also is usually weak, and a dislocation of the lens and
prolapse of the vitreous may easily occur.
VITREOUS OPACITIES
AEtiologically vitreous opacities may be divided into three classes —
1. Congenital remnants of the hyaloid vascular system.”
2. Endogenous opacities:
(a) protein coagula of the colloid basis of the gel ;
(b) crystalline deposits (i) asteroid bodies,
(ii) synchisis scintillans.
3. Exogenous opacities :
(a) protein coagula—the plasmoid vitreous ;
(b) exudative cells ;
(c) blood ;
(d) tissue cells : epithelial, histiocytic, glial ;
(e) tumour cells ;
(f) pigment : melanotic and haematogenous.
ENDOGENOUS OPACITIES
(a) PROTEIN COAGULA
The endogenous opacities of protein origin may be extremely small and
dust-like—so small indeed as to be almost if not quite invisible to the ophthal-
moscope, and then only with a plain mirror and a high plus lens, while at
other times they take the form of quite considerable fibrous-like strands and
membranes (Figs. 2773 and 2786). The fibres are arranged in irregular
bundles like tangled skeins of wool which move about with optically empty
spaces between them. The smaller particles probably represent the dis-
integration of the micellae of the gel into granular coagula, and the fibros
their agglutination into strands, for whereas in the normal condition their
state of turgescence demanded their massing together before they became
1 Vol. IV. * Vol. II, p. 1379
DISEASES OF THE WITREOUS BODY 3249
optically visible, their higher refractive index in the coagulated form allows
them to become more readily visible separately. The membranes, on the
other hand, are typically associated with a general liquefaction of the gel
posteriorly in which case the colloid basis is drawn forwards in lamellae which
fuse with the dense basis of the vitreous opposite its ciliary attachment to
form thick wavy membranous sheets which wave about behind the lens.
Sometimes the coagulated masses after being tossed about in the fluid
vitreous for some time assume a spherical shape ; and many of them even-
tually attract cells which become adherent to them. At other times the
surface condensation of the gel which forms a limiting membrane in
detachments of the vitreous, may form opacities of a very definite nature."
(b) CRYSTALLINE DEPOSITs
Crystalline deposits in the vitreous are relatively uncommon : Dor (1906) found
them 32 times in 82,732 patients and Westpfahl (1915) 40 times in 65,000. They were
known, however, even in pre-ophthalmoscopic days, for in pronounced cases the brilliant
corpuscles were seen by focal light floating about in the pupil (Spintheropia, Spin-
theromma, Sichel, 1846–51 ; scintillatis pupillae, Backer, 1851). Originally little
discrimination was made between the types of crystalline deposits, and the earlier
writers referred to them all as synchisis Scintillans (Desmarres, 1845–50; Sichel, 1846;
Robert, 1847; Hirschberg, 1876; Poncet, 1876; Adams, 1881; and others), until
Benson (1894) differentiated white spherical bodies as asteroid hyalitis, distinguishing
them from the golden crystals typical of synchisis scintillans. The former, which are
the commoner, occur in a substantially normal vitreous and appear to be deposits of
calcium soaps ; the latter occur in a fluid vitreous and are probably cholesterol crystals.
(i) ASTEROID BODIES : SCINTILLATIO ALBESCENS OR NIVEA
Although noted by such writers as Webster (1883), Cross (1886),
D’Oench (1889) and Walk (1889), the occurrence of spherical or disc-shaped
white bodies was first differentiated from synchisis scintillans by Benson
(1894) as ASTEROID HYALITIS (BENSON’s DISEASE); the name is an apt one
to describe the appearance of “ hundreds and thousands of small spheres of a
light cream colour . . . like the stars in a clear night,” although in the
absence of inflammatory changes in the vitreous, the term “ hyalitis '' is
better dropped. Argyll Robertson (1894) described them as SNOw-BALL
OPACITIES, a name adopted by Holloway (1917), while Wiegmann (1918)
suggested the term SCINTILLATIO ALBESCENS or NIVEA.
The condition is not common ; thus Holloway (1917–22) saw 9 cases,
Feingold (1922) 8, Westpfahl (1915) recorded 40 cases from the literature,
and Rutherford (1933), in reporting 18 cases seen in the Iowa Hospital in
7 years, collected records of 56 from the literature. It is essentially a senile
phenomenon, for the literature shows that the age of the affected patients
averages 60 years, and varies from 30 to 84; it is commonest in the 7th and
8th decades of life. So far as sex is concerned, it appears to be three times
1 p. 3270
4 E 2
3250 TEXT-BOOK OF OPHTHALMOLOGY
as common in males as in females; and it is much more frequently unilateral
than bilateral; indeed, only some 25% of the cases affect both eyes.
The asteroid bodies are small discrete bodies, disc-shaped or spherical,
sometimes marshalled in strands and columns, sometimes in bunches, but
more usually showing no orderly
arrangement (Fig. 2774). With the
ophthalmoscope they are seen by
reflected light as creamy or white and
shiny, looking like snowballs or stars
in the night sky (Fig. 2775); by
transmitted light they are dark (Fig.
2776); and in the intense focal beam
of the slit-lamp they usually appear
dead-white and round and gleam
brilliantly (Erggelet, 1914; Vogt, 1921;
and many others subsequently) (Fig.
2777). They may be found scattered
throughout the whole vitreous or
FIG. 2774–AstERond Hyaliſm accumulated in any part of it, and
ºi.…..","...º." they have been noted lying on a
detached retina (D'Oench, 1889); some-
times there are only two or three of them to be seen, and at other times
they appear to be innumerable. The vitreous itself, while usually
showing some degenerative changes, retains its solidity, so that when
Figs. 2775–76–AstERoid Hyaliſtis.
Snow-ball opacities seen by reflected light in Fig. 2775, by transmitted light in
Fig. 2776 (Holloway and Fry).
the eye is moved the bodies move with a wave-like excursion and return to
their original location; they do not show an unrestrained excursion nor
do they settle to the bottom of the vitreous chamber. They have been
noted in the anterior chamber in cases of vitreous prolapse with dislocation


DISEASES OF THE WITREOUS BODY 3251
of the lens where they lie bound up in the jelly-like mass of vitreous
(Gallenga, 1931). Sometimes the crystals are associated with cellular
elements which Janson (1925) considered derived from the retinal
epithelium.
Chemical and histological examinations have been undertaken by several
workers (Verhoeff, 1921; Bachstez, 1921–24; Holloway and Fry, 1929;
Fig. 2777.-AstERold BoDIEs.
Slit-lamp appearance.
Clapp, 1929; Hanssen, 1931; and Gallenga, 1931), all of whom agree that
these bodies are most probably calcium soaps, palmitate, stearate and
marganate being mentioned ; sodium and calcium phosphates and chlorides
have been described, as well as fatty or lipoid substances in various
grades of stability. Tests for cholesterol, tyrosine and albumen have been
negative.
The cause of the deposition is unknown. Associated ocular or general disease
seems on the whole to be co-incidental, for although arteriosclerosis (Black, 1909;
Holloway, 1917–22; Verhoeff, 1921), nephritis (Stark, 1917), diabetes (Vogt, 1921),
and other general diseases have been noted from time to time, and local conditions
have coincided such as old choroiditis (Pollack, 1896; Holloway, 1917; Bachstez,
1924; Matthewson, 1927), cyclitis (Stark, 1924; Clapp, 1929), retinal haemorrhages
(Wiegmann, 1909; Holloway, 1917; Bailey, 1926) or thrombosis (Tenner, 1922), as
frequently the patients have had no other discoverable lesion or disease. With quite
insufficient evidence Stark (1917) associated them with syphilis and Bailey (1926)
with tubercle. The deposition may be due to changes in the composition of the blood,
although the frequent unilaterality of the condition would indicate that the process
must be aided by local causes in the vitreous; it may resemble the deposition of
gall-stones. We have already seen that calcium deposits (probably of the oxalate) are
common in naphthalene poisoning in rabbits,” an animal in which similar depositions
can beformed by rendering the vitreous alkaline (Jess, 1922).
There is no evidence that the presence of such bodies has given rise to
symptoms or has been responsible for a reduction of vision ; nor that they
have been modified in any way by treatment
* p. 3150.

3.252 TEXT-BOOK OF OPHTHALMOLOGY
(ii) synchisis scINTILLANs
SYNCHISIs scINTILLANs is probably a rarer occurrence than asteroid
bodies; moreover, it occurs in younger patients frequently under 35 years
of age, and is more usually bilateral. In contrast to the white spheres of
the asteroid bodies, these crystalline deposits have a flat, angular, crystalline
appearance. Occurring in a degenerated and fluid vitreous, they may lie
hidden at the bottom of the vitreous chamber while the eye remains still,
and then when the globe is moved, they leap up into sight, flash about with
Fig. 2778–SYNoHists Sonſtillans.
The shower of particles in the vitreous just after it has been disturbed.
such rapidity that exact observation of their shape is difficult, and finally
settle down like a shower of glittering golden sovereigns or silvery timsel-like
particles to the bottom of the vitreous chamber where they remain until
they are disturbed again. Both with the ophthalmoscope and the slit-lamp
they present a beautiful and quite characteristic picture as they flash by
with a gleam as of burnished gold (Fig. 2778).
It is interesting that their movements have been recorded by stereo-photography
by Lijo-Pavia (1934).
Sometimes the fluid vitreous fills the anterior chamber, in which case they may

DISEASES OF THE WITREOUS BODY 3253
form a pseudo-hypopyon gleaming with metallic reflexes (Koby, 1932), or the entire
anterior chamber may be filled with the crystals (Hughes, 1937).
Microscopical and chemical examinations have shown that the crystals
of this type are usually composed of cholesterol although needles of
tyrosine and margarin have been noted (Stout, 1847; Poncet, 1876; Berlin,
1883; Sgrosso, 1892; Westpfahl, 1915; Valentine, 1920; Koby, 1932;
Hughes, 1937; and others).
It is to be remembered that in contrast to asteroid bodies which occur
in relatively healthy but aged eyes, this condition is secondary in that it
occurs in degenerative conditions of the eye of long standing, following
traumatic, haemorrhagic, or inflammatory lesions; but of itself it gives rise
to no symptoms and demands no treatment.
ExoGENous OPACITIES
Exogenous opacities are of various kinds.
(a) PROTEIN forming a diffuse dust-cloud or aggregating into clumps is
a common accompaniment of all conditions in which the capillaries of the
ciliary body are dilated. Such a plasmoid vitreous corresponds in every way
Fig. 2779.-CELLs IN THE WITREous FROM RETINAL Wound.
Organizing tissue of long spindle-shaped cells in the vitreous 15 days after injury
in a monkey (> 300) (Parsons).
with a plasmoid aqueous and is associated particularly with cyclitis, chorio-
retinitis, choroidal tumours, or contusions. A powdery exudative opacity
of this type is frequently difficult to analyse ophthalmoscopically, for the
fundus merely appears hazy with its details obscured, and the disc may

3254 TEXT-BOOK OF OPHTHALMOLOGY
appear indistinct and muzzy as if an optic neuritis were present. Sub-
jectively a cloudiness and lowering of the visual acuity is usually remarked.
(b) Exudative cells, particularly lymphocytes, leucocytes and
plasmoid cells, accompany inflammatory conditions of the ciliary body and
retina. We have already seen that in cases of retinitis it is a commonplace
for great quantities of such cells to breakthrough the internal limiting mem-
brane of the retina and become aggregated on the surface of the vitreous in
heaps or in large sheets, sometimes appearing ophthalmoscopically as if
-
-
- -
-
--- -- . - -
- - - - - - - - -
º - º-- ------- º -- **-*. ***- - ---
- - -
-
Fig. 2780–INFECTIVE WITREous Opacities.
A post-traumatic infection. The vitreousgelis shrunken and contains leucocytes
scattered throughout it. There are several large cavities in its substance, the walls
of which are crowded with leucocytes seen in the upper part of the figure (Straub,
T. O. S.).
they were exudative masses in the retina itself (Fig. 1799). Thence the gel
may become diffusely infiltrated or the cells may remain largely localized to
cavities of liquefaction, to the secondary vitreous, or confined by the
lamellar structure of the stroma (Fig. 2789). Pathological specimens show
that these cells accumulate preferentially on the walls of cavities of
liquefaction or cling to the membranous septa of histological specimens
(Dhai, 1911; Straub, 1912; Brückner, 1919; Fuchs, 1920; Rados, 1920;
Kafka, 1923; Samuels, 1930) (Figs. 2780-81). Such an infiltration may
be present to any degree, varying from a general bespattering of the vitreous
* p. 2623.

DISEASES OF THE WITREOUS BODY 3255
so that the retina is seen hazily, to the conversion of the vitreous cavity into
an abscess as occurs in panophthalmitis. Such cells may remain unchanged
for an indefinite time; alternatively degenerative changes ensue when they
become crenated, star-shaped, and vacuoled (physaliphores), or suffer fatty
changes; and finally, when inflammatory exudation has been intense,
organization and fibrosis develop, with the formation of cyclitic membranes,"
or a pseudo-glioma.” In this event giant cells, fatty globules and pig-
mentary deposits may appear and vascularization, calcification, cartilage
Fig. 2781–OUTER SURFACE of VITREous CoATED witH Round CELLs (Samuels,
T. O. S.).
formation and eventually ossification may develop, and the contraction of
the mass may lead to retinal detachment and shrinkage of the globe.
(c) BLooD, escaping as a rule from a retinal haemorrhage, may surround
the surface of the vitreous gel, forming a sub-hyaloid haemorrhage posteriorly,
or a layer of blood immediately behind the lens; in either case one peculiar
feature is the length of time during which the blood remains bright red, and
the perfect state of preservation maintained by the corpuscles many months
afterwards (Samuels, 1930). At other times the haemorrhage may be
confined within Cloquet's canal, or is localized within pockets in the body
1 p. 2187. * p. 2825.

3.256 TEXT-BOOK OF OPHTHALMOLOGY
of the vitreous; while again, thered cells may be diffusely scattered through-
out the gel, and in such quantity that it may be quite impermeable to light.
Such haemorrhages may be traumatic or inflammatory in origin or due to
disease of the retinal vessels. They may be slowly or completely absorbed
by the solution of the fibrin and the removal of the cells and débris by
leucocytes, or in rare cases they may organize to form a retinitis proliferans.”
(d) EPITHELIAL CELLs from the ciliary body may be found in the
vitreous, sometimes intact but more usually broken up, the walls being
ruptured and the small black pigment granules set free. Occasionally
macrophages engulf these granules, and it may be difficult histologically to
draw a distinction between them and a pigmented epithelial cell.
RETICULAR CELLs (histiocytes) enormously swollen and loaded with
fat may be found in clumps in the vitreous in lipoidosis bulbi (Heath, 1933)
(Fig. 2782).
SPHERICAL CELLULAR ELEMENTS are
occasionally seen, sometimes floating freely
and sometimes attached to the retina or
nerve-head by a pedicle; they probably
represent proliferations of glial cells, while
others containing pigment granules may have
been ultimately derived from the pigment
epithelium (Samuels, 1930).
CELLs of HoRTEGA-mesodermal phago-
cytic cells from the retina—have been described
ria, ºnse. Lºomposs Burer in the vitreous by López Enriques (1935).
Clumps of fatty and pig- (e) TUMoUR CELLs are rare visitors to the
º cells in the vitreous vitreous, but they may occur and may even set
- up metastatic deposits. Such a happening is,
as we have seen, exceptional in melanomatous and epitheliomatous
neoplasms, but much more common in the case of retinoblastomata, being
indeed the rule in “glioma endophytum.”
Other organized cellular elements are occasionally found, such as wandering
tubercles” or post-inflammatory tissue adhering to a detached vitreous."
(f) Provºst GRANULES
MELANotic Pigmºnt GRANULEs, both retinal and uveal, are found,
sometimes in large quantities in conditions of semility, in glaucoma, after
trauma and inflammations, in retinal detachments, and with melanotic
tumours. The pigmentation may be slight, comprised of a few minute and
scarcely visible individual deposits, or may be very abundant and arranged
in large groups; the granules, which are usually of a brownish colour,
p. 2595. * p. 2604.
p. 2302. * p. 3266.

DISEASES OF THE WITREOUS BODY 3257
frequently attach themselves to the supporting fibrils, and may produce
beautiful effects as they reflect the incident light of the slit-lamp at various
angles ; moreover, they may be deposited extensively on the posterior
capsule of the lens.
BLOOD PIGMENT may also persist for a very considerable time after
haemorrhages, sometimes in small amounts, and sometimes in sufficient
quantity to change the colour of the gel and obscure the architecture of its
framework.
These various cellular elements are quite indistinguishable separately
by the ophthalmoscope, appearing merely as dust-like opacities when not
sufficiently numerous to obliterate the reflex of the fundus. If they are present
in great quantities, the whitish-green reflection of a purulent exudate or
the dark red glow of a haemorrhage may be evident by focal illumination.
With the slit-lamp red cells in the anterior vitreous are evident ; while
exudative cells appear as white brilliant points without special form, diffusing
or diffracting the light rather than reflecting it, and tending to cling to the
pseudo-fibres of the gel.
The treatment of vitreous opacities is difficult and usually disappointing.
No matter what treatment is adopted, cellular elements, if not too abundant,
tend to disappear, and it is probable that fine dust and strands derived
from the stroma may also disappear in time. Grosser opacities, however,
and connective tissue strands and membranes as well as crystalline deposits
always persist. To aid the disappearance of such elements which can be
dissolved, various expedients are usually suggested, but since they tend to
disappear of themselves spontaneously, the efficacy of any treatment is
difficult to assess. It is, however, obvious that any underlying disease of
the eye should be adequately treated, and any general condition which
determines the local degeneration, whether infective, toxic, or sclerotic,
should receive adequate attention. The most important treatment is
aetiological—the prevention of the development of further trouble by
eliminating the cause so far as is possible.
The local expedients generally employed include heat, radiotherapy,
sub-conjunctival injections, dionine, and other measures designed to quicken
metabolic interchange and promote absorption ; while as to general treat-
ment, rest with the administration of mercury and iodine, if there is no
general contra-indication for them, even although their therapeutic value is
based rather on hope than on proved results, can at least do the local condi-
tion no harm. Operative measures have also been advocated, such as
trephining with a view to accelerating tissue-interchange (Henton and Henton,
1937; and others). When the opacities are sufficiently massive to abolish
vision, and all reasonable hope of spontaneous absorption has been given
up, the more heroic procedure of vitreous replacement may be attempted.
Replacement by the vitreous of another animal always leads to shrinkage
3258 TEXT-BOOK OF OPHTHALMOLOGY
of the eye (Dor, 1912; and others), but the withdrawal of a small quantity
of opacified vitreous (zur Nedden, 1920–22) and its replacement by physio-
logical salt solution (Elschnig, 1911; Löwenstein and Samuels, 1911),
serum (Sattler, 1909), or cerebro-spinal fluid (Hegner, 1928) may occasionally
lead to good results. The withdrawal should always be in stages, less than
one-third of the volume of the vitreous being removed each time lest a retinal
detachment ensue.
None of these methods of treatment, however, can be
said to be particularly satisfactory or encouraging.
Adams. T. O. S., i, 135, 1881.
Bachstez. Wien. med. W., lxxi, 1044, 1921.
Z. f. Aug., liv, 36, 1924.
Backer. Med. Z., Basel, Nr. 5, 1851.
Bailey. Am. J. O., ix, 700, 1926.
Benson. T. O. S., xiv., 101, 1894.
Berlin. Z. f. vgl. Aug., ii, 110, 1883.
Black. O. Rec., xviii, 201, 1909.
Brückner. A. f. O., c, 179, 1919.
Clapp. A. of O., ii, 635, 1929.
Cross. T. O. S., vi, 376, 1886.
Desmarres. An. d’Oc., xiv, 220, 1845; xxiv,
495, 1850.
Dhai. Ophthalmology, vii, 708, 1911.
D’Oench. N. Y. Med. Rec., xxxv, 268, 1889.
Dor. L’Ophtal. Prov., v., 101, 1906.
La Clin. Opht., xviii, 119, 1912.
Elschnig. B. O. G. Heidel., xxxvii, 11, 1911.
A. f. O., lxxx, 514, 1911.
Enríques, López. Bull. S. fr. d’O., xlviii, 298,
1935.
A. de Oft., H.-A., xxxv, 197, 1935.
Erggelet. K. M. Aug., liii, 449, 1914.
Eeingold. Am. J. O., v, 840, 1922.
Fuchs. A. f. O., ciii, 228, 1920.
Gallenga. A. di Ott., xxxviii, 398, 1931.
Hanssen. Z. f. Aug., lxxvi, 77, 1931.
Heath. A. of O., x, 342, 1933.
Hegner. B. O. G. Heidel., xlvii, 391, 1928.
Henton and Henton. A. of O., xix, 103, 1937.
Hirschberg. Deut. Z. f. prakt. Med., iii, 39,
1876.
Holloway. T. Am. O. S., xv, 153, 1917.
A. of O., xlvii, 50, 1918.
Am. J. O., v., 100, 1922.
Holloway and Fry. A. of O., ii, 521, 1929.
Hughes. A. of O., xviii, 477, 1937. -
Janson. K. M. Aug., lxxv, 681, 1925.
Jess. K. M. Aug., lxviii, 790, 1922.
Kafka. Zb. ges. O., x, 65, 1923.
Koby. Biomicroscopie du corps vitré, Paris,
p. 100, 1932.
Lijo-Pavia. Bull. S. d’O. Paris, xlvi, 35, 1934.
Löwenstein and Samuels. A. f. O., lxxx, 500,
1911.
Matthewson. Am. J. O., x, 53, 1927.
zur Nedden. A. f. O., ci, 145, 1920.
K. M. Aug., lxiv, 593, 846, 1920; lxvi. 59,
1921 ; lzix, 514, 1922.
Pollack. Beit. 2. Aug., xxiv, 83, 1896.
Poncet. An... d’Oc., lxxv, 235, 1876.
Rados. A. f. O., ciii, 331, 1920.
Robert. Am... d’Oc., xviii, 79, 1847.
Robertson, Argyll. T. O. S., xiv, 102, 1894.
Rutherford. A. of O., ix, 106, 1933.
Samuels. A. of O., iv, 838, 1930.
Sattler. A. f. Aug., lxiv, 390, 1909.
Sgrosso. Rev. gen. d’O., xi, 461, 1892.
Sichel. An. d’Oc., xv, 167, 1846; xxiv, 49,
1850; xxv, 9; xxvi, 3, 1851.
Stark. A. of O., xlvi, 38, 1917.
T. Am. O. S., xxii, 315, 1924.
Stout. Gaz. Méd., ii, 72, 1847.
Straub. T. O. S., xxxii, 60, 1912.
Tenner. A. of O., li, 507, 1922.
Valentine. Z. physiol. Chem., cv, 33, 1920.
Valk. N. Y. Med. Rec., xxxv, 569, 1889.
Verhoeff. Am. J. O., iv., 155, 1921.
Vogt. Atlas, Berlin, 1921.
Webster. A. of O., xii, 179, 1883.
Westpfahl. A. f. Aug., lxxviii, 1, 1915.
Wiegmann. W. f. Therap. u. Hyg. d. Auges,
xii, 353, 1909.
K. M. Aug., lxi, 82, 1918.
SPECIFIC IDEGENERATIONS
1. SENILE DEGENERATION
In old age some degree of vitreous degeneration is universal, the essential
change being a destruction of the normal framework of the gel with the
development of liquefaction and the appearance of endogenous opacities.
Z’Brun (1921) found such changes in over 50% of adult subjects.
Most old
people complain of seeing these floating spots entoptically, but their presence
DISEASES OF THE WITREOUS BODY 3259
is unattended by inflammation or visual disability, and they show little or
no tendency to progress. The liquefaction usually starts by the formation of
cavities filled with fluid in the anterior central part of the vitreous (Fig. 2783).
The cavities grow in size and multiply in numbers and are so common as to be
considered physiological (Samuels, 1930; Baurmann, 1935). Their develop-
ment is probably the cause of the common complaint of old persons that they
suddenly see a “fly" in the visual field which remains relatively stationary
in position, an entoptic appearance caused by the coagulum of the gel in
Fig. 2783.-SENILE DEGENERATION of THE WITREous.
The vitreous body contains cavities in which coagulation opacities (b) are seen,
while the more condensed parts of the gel (a) show a fine fibrillary structure (Samuels,
T. O. S.).
the liquefied area. It is possible that the suddenness of this appearance
may be due to the local dissolution of the gel by a minimal trauma or a sharp
rotation of the eye so that a part of its colloidal frame-work gives way. The
second most common site of liquefaction is at the posterior pole where the
framework is thinnest. In this region a detachment of the vitreous gives
gives rise to the SENILE ANNULAR opacity (of Vogt, 1925)—a small annular
opacity situated near the disc which probably represents the result of a
detachment of the gel from the nerve-head, a subject which will be dis-
cussed presently.”
1 p. 3269.

3260 TEXT-BOOK OF OPHTHALMOLOGY
With the ophthalmoscope the lesser degrees of degeneration may be
invisible, but with the slit-lamp (which is profitably employed with an
overloaded lamp to increase the intensity of the beam : Streiff, 1924) a
filamentous structure is usually very obvious, the fibres being long and wavy
and running in all directions (Fig. 2773).
Sometimes they are aggregated into
clumps which may be visible ophthalmo-
scopically while their relatively free mobility
betrays the liquefaction of the gel (Koeppe,
1918; Vogt, 1924; Comberg, 1924)
(Fig. 2784); at other times minute white
irregularities are seen on the strands as if
they had been sprinkled with sugar—the
- sENILE PEPPERING of Koby (1932). In
Irregular fibrillar appearance -
ophthalmoscopically as opacities, are present.”
Seen with the slit-lamp.
Fig. 2784–THE SENILE WITREous.
The diagnosis of senile changes in
the vitreous from inflammatory opacities can only be accomplished by
the slit-lamp, whereby their fibrillary constitution can be observed and the
absence of inflammatory cells verified. No treatment is of any value, nor is
any required.
2. MYOPIC DEGENERATION
The highly myopic eye suffers a degeneration of the vitreous very similar
to the senescent eye, for in both cases the changes are due to disintegration
of the gel and the opacities are endogenous. Liquefaction is the rule, a
process seen most extensively at the
posterior pole, and the tendency is for the
colloid basis of the gel, condensed into
membranous formations, to accumulate
anteriorly (Fig. 2786). Immediately behind
the lens the slit-lamp therefore usually
reveals numerous and irregular filaments
showing nodes and thickenings running
typically in a vertical direction, behind -
which more or less well-developed and Fig. 2785–The Vitreous IN
extensive diaphanous membranes are Myopia.
almost constantly seen. sometimes showing a.º. º, º
an irregular fibrillation (micro-fibrillary
degeneration) (Fig. 2785) (Koeppe, 1918; Streiff, 1924; Comberg, 1924;
Vogt, 1924; Koby, 1932; and others). Pathologically such a vitreous
body, while showing punctate and fibrillar coagula and a few incidental
cellular elements, reveals nothing characteristie (v. Graefe, 1854; Greeff,
* p. 3249.


DISEASES OF THE WITREOUS BODY 3.261
1902–06; Terrien, 1906; Cattaneo, 1931; and others); the lesions are not
histological, but physico-chemical.
The subjective symptoms of such a condition may cause the patient
much annoyance, and the appearance of spots and streamers in front of the
eyes may not only become a nuisance but also give rise to considerable
anxiety, while in the more severe forms the vision may be considerably
- - * º
-
-
-
º -
- - -
--~
Fig. 2786.--THE WITREous IN HIGH Myopia.
Complete liquefaction of posterior half (Samuels, T. O. S.).
affected. Nor is the anxiety completely without foundation for it may be
that while a solid or completely fluid vitreous is relatively safe, the mem-
branes and strands of a semi-fluid vitreous, if they are attached to the
peripheral part of the retina at a patch of chorio-retinal degeneration or
old inflammation, may conceivably exert sufficient traction to tear and
detach this tissue as they wave about with the movements of the eye (jactatio
corporis vitrei) (Baurmann, 1935)."
* p. 2898.

3262 TEXT-BOOK OF OPHTHALMOLOGY
3. RETINAL DETACHMENT
Although the vitreous may be relatively normal in the case of recent
detachment, in the majority of cases, particularly in myopic eyes, and
eventually in all cases, definite evidences of degeneration are seen; these
are present invariably and in
increasing degree as the detach-
ment advances in age. In addition
to the usual evidences of lique-
faction with the formation of
endogenous opacities, Koby (1932)
described as an almost constant
phenomenon the development of
tube-like anastomosing strands
looking as if they represented a
rolled-up membrane (Fig. 27.87);
Fig. 2787.-THE WITREous is RETINAL in addition punctate opacities are
DETAcantºnT. always evident, some of them
white and round and many of them pigmented and stellate, probably
representing depositions of pigment from the retinal epithelium on the
pseudo-membranes.
4. INFLAMMATORY CONDITIONs
In any intra-ocular inflammation of some standing and severity the
vitreous shows changes due to the appearance of exogenous opacities; in
more severe cases when liquefaction of the gel occurs, endogenous opacities
Fig. 2788.--THE WITREous IN IRudo-cyclinis.
Slit-lamp appearance showing punctate and membranous opacities.
are added to these. It used to be said that syphilis—particularly syphilitic
retino-choroiditis–was the most common cause of such opacities, but,
while they are numerous and pronounced in this disease, it by no means
holds a monopoly.
In the early stages of an irido-cyclitis, chorio-retinitis, or a papillitis


FIG. 2789.-IRIDo-cycliſtis.
Post-traumatic infective condition showing the exudate in the anterior chamber
and the shrinkage of the vitreous which contains a cavity to the right, the wall of
which is lined by leucocytes (Straub, T. O. S.).
FIG. 2790.-THE WITREous IN ExogFNous INFECTION.
The posterior limiting layer and Cloquet's canal are outlined in exudative
cells (Samuels, T. O. S.).
T.O.-WOL. III. 4 F


3264 TEXT-BOOK OF OPHTHALMOLOGY
the entire vitreous may assume an opalescent effect due to its being flooded
with a protein-rich fluid from the dilated capillaries, while it is permeated
by minute opacities appearing in the beam of the slit-lamp as small,
brilliantly white dots, rounded or stellate, lying free in the vitreous or
encrusting its fibrillar structure (Fig. 2788). Histological examination shows
them to be leucocytes, mainly polymorphonuclear cells in the earlier stages of
the disease and lymphocytes and plasma cells in the later (Fig. 2789). As time
goes on they become yellowish and brownish, and if the inflammation dies
down they may slowly disappear. During this time the structure of the
vitreous may be relatively normal, but if the inflammation is prolonged the
Fig. 2791–THE WITREous IN CycLITIs (Bedell, T. O. S.).
gel eventually breaks down and liquefies throwing down filamentous and
membranous opacities, until eventually the combination of the two types
may produce an intense opacification. Occasionally the exudative opacities
are confined almost exclusively to Cloquet’s canal (Butler, 1928; Koby,
1932) (Fig. 2790).
In advanced cases the combination of the two types of opacity may
produce a very dense and muddy haze with disastrous effects upon vision.
With the slit-lamp the picture may be complicated (Fig. 2791); an abund-
ance of minute white specks, spangles and threads are mingled with large
agglutinations like tangled skeins of wool, broad sinuous bands, wavy
membranes, and extremely dense floating clouds, the whole oscillating
slightly and swaying across the pupil with movements of the eyes.

DISEASES OF THE VITREOUS BODY 3265
The observation of the early opacities may sometimes be of great clinical
importance. They may, for example, form a very early symptom of sympathetic
ophthalmitis, in which disease, in conjunction with corneal precipitates and similar
cells circulating in the aqueous, a multitude of minute opacities in the anterior vitreous
and deposited on the posterior capsule of the lens, may form a diagnostic sign of
great value (Vogt, 1921); these vitreous opacities may be the first to appear, and,
indeed, cases have been reported in which they have formed the only evidence of
sympathetic disturbance (Molotese, 1924; Alajmo, 1929). Heterochromic cyclitis is
peculiar in that although the exudative opacities may be numerous and particularly
dense over many years, the structure of the gel may remain intact indefinitely.
A third clinical appearance of very considerable interest is the sudden occurrence of
opacities of the exogenous type, sometimes in considerable quantity, without apparent
cause. Such a happening is of common and general occurrence, but is seen most
typically in women in late middle age. It is presumably due to peripheral foci of
anterior choroiditis, so minute as to escape notice or so far forward as to escape
ophthalmoscopic detection, of which the disturbance of the vitreous forms the
only clinical evidence. The importance of the syndrome is that it may well form the
prelude to a series of similar attacks each involving the disability of further vitreous
opacities, or to more serious and widespread uveal inflammation of a recalcitrant and
chronic nature, unless the aetiology is discovered and combated—in many cases a
task of no small difficulty.
5. HAEMORRHAGES
The question of haemorrhages, particularly recurrent haemorrhages,
into the vitreous has already been discussed. In localized pre-retinal
haemorrhages the vitreous may show no changes evident clinically with the
slit-lamp. In large haemorrhages, however, the whole vitreous may be
involved, at first with small whitish opacities probably representing the
deposition of protein, and eventually with red cells. In the periphery of
the vitreous these red cells may take up a lamellar arrangement (Lindner,
1934–36), and the canal of Cloquet may be preferentially distended with
them (Hoffmann, 1926; Barkan, 1928). The deposition of blood-cells on
the posterior surface of the lens is also the rule, and there they may remain
unchanged for a very long time, sometimes for years; the blood may be
scattered over the posterior capsule indiscriminately at first, but in time
there is a tendency for it to settle down into a crescentic band near the
periphery at the line where the vitreous normally comes into contact with
the capsule (Egger's line). Any large haemorrhage leaves the vitreous fluid,
and even when absorption has proceeded so far as to allow the return of
normal vision, some opacities, usually coloured with blood pigment, always
remain permanently. We have seen that in such cases organization may
occur and bands of fibrous tissue form to constitute a retinitis proliferans,”
while, as a rarity, new vessels may proliferate into the vitreous presumably
in the attempt to aid absorption.”
1 p. 2604. * p. 2618.
3266 TEXT-BOOK OF OPHTHALMOLOGY
6. TUMoURS
Intra-ocular tumours affecting the posterior segment of the eye always
cause some disorganization of the vitreous, somewhat resembling senile
degeneration. In addition, however, in melanotic tumours a fine yellowish
dust of pigment may be seen; while in retinoblastomata actual neoplastic
nodules may be visible with the slit-lamp (Scheel, 1925).
Alajmo, Boll. d’Oc. viii, 400, 1929. Koeppe. A. f. O., xevi, 232, 1918.
Barkan. A. of 0., lvii, 502, 1928. - Lindner. B. O. G. Heidel. 1, 86, 1934.
Baurmann. K. M. Aug., xev, 259, 1935. A. f. O., exxxv, 332, 1936.
A. f. O. cxxxiv, 201, 1935. Molotese. Boll. d’Oc., iii. 1029, 1924.
Butler. T. O. S., xlviii, 409, 1928. Samuels. A. of 0., iv, 838, 1930.
Cattaneo, Boll, d'Oc., x, 265, 1931. Scheel. K. M. Aug., lxxv, 670, 1925.
Comberg. K. M. Aug., lxxii. 692, 1924. Streiff. K. M. Aug., lxxiii. 703, 1924.
v. Graefe, A. f. O., i(1), 390, 1854. Terrien. A. d’O., xxvi, 737, 1906.
Greeff. Path. Anat, d. Auges, Berlin, 1902-6. Vogt. Atlas, Berlin, 1921.
Hoffmann. K. M. Aug., lxxvii, 641, 1926. K. M. Aug., lxxii, 212, 1924; 1xxv, 463,
Koby. Biomicroscopie du corps vitré, 1925.
Paris, 1932. Z"Brun. A. f. O., evii. 61, 1921.
III. DETACHMENTS AND DISPLACEMENTS
DETACHMENT OF THE WITREous Body
From the earliest days of ophthalmic pathology it has been known
that a detachment of the vitreous owing to the shrinkage of inflammatory
Fig. 2792.-Detachment Fig. 27.93.-DETAchment
or the Virardous. or THE WITREous.
Antero-lateral and infundi- Globular detachment of the
bular detachments of the vitreous. There is a trau-
vitreous in an eye injured 5 matic dislocation of the lens
weeks previously (Parsons). and vitreous passes into the
anterior chamber (Parsons).
products which have been poured into it from the uveal tract or the retina,
occurs commonly as an end-result of gross intra-ocular disease (Müller,

DISEASES OF THE WITREOUS BODY 3.267
1856; Iwanoff, 1869; de Wecker, 1876; and many others). Such a
detachment may be of four types:
(1) A relatively small cul-de-sac detachment occurring at any point in
the circumference (Fig. 2792).
(2) An infundibular detachment (Milles, 1886) wherein the vitreous
assumes a tent-like shape, the apex being at the disc and the base round
the ora (Fig. 2792). At other times the apex is situated at the site of a
patch of chorio-retinitis or of a perforating wound.
(3) A globular detachment (Milles, 1886) wherein the attachment at the
optic disc gives way and the entire mass of vitreous gets bunched up as a
hemi-spherical mass behind the lens (Fig. 2793).
(4) An anterior detachment is very rare, but this portion of the vitreous
may be stripped from the lens and
zonule and fall back considerably :
such a condition may be associated
with a posterior detachment as well,
in which case the vitreous assumes the
form of a broad band at the level of
the ora serrata. A tearing away of the
base of the vitreous from its attach-
ment at this point is a still greater
rarity, although contraction by or-
ganization may be so excessive that
one-half of the circumference may
give way so that the entire vitreous
may be represented in cross-section by
an Indian club attached to the ora -
and the flat part of the ciliary body Fig. 2794–Detachment of THE
VITREous.
(Samuels, 1930). - - Organizing connective tissue at apex of
Such a detached vitreous is always a detached vitreous (x 55) (Parsons).
degenerated and shrunken, and is -
pulled forwards by shrinking exudates and organizing fibrous tissue. Its
outer surface is frequently covered by an endothelial membrane similar in
appearance and origin to the membranes which in similar circumstances
cover the retina it may secrete a hyaline layer or be associated with a
considerable amount of fibrous tissue. Occasionally where there are points
of organization, this adventitious tissue may be of considerable thickness,
and if it tears away with the detached vitreous a dense round vitreous
opacity results (Parsons, 1905; Samuels, 1930) (Fig.2794). The space between
the vitreous and the retina is usually filled with fluid containing albumin;
and the intra-ocular tension is low. In these cases, of course, the detachment
of the vitreous is merely a pathological incident in a lesion the seriousness
of which lies elsewhere.
p. 2626.

3268 TEXT-BOOK OF OPHTHALMOLOGY
A type of detachment occurring with less obvious cause in a vitreous
not grossly degenerated has given rise to more controversy. Most of the older
pathologists, following Müller (1856) and Iwanoff (1869), considered that a
detachment of this nature complicated such conditions as high myopia and
staphylomata and that it was essentially a detachment e vacuo (Iwanoff,
1869); others again considered the pathological picture to be due to an
artefact of fixation (Greeff, 1902; Elschnig, 1904). More recent histological
research, however, both using ordinary fixatives (Samuels, 1930) and the
pyridine method which involves no shrinkage (Sallmann, 1936), have
demonstrated that it does occur. It is true that an artificial posterior
detachment due to post-mortem fixation does occur, but the ante-mortem
variety can be differentiated histologically by the presence of albumin in
Fig. 2795. – Postºnio R. FIG. 2796. – SUPERIOR
DETACHMENT OF THE DETACHMENT OE THE
VITREous (after Reiger). VITREous (after Reiger).
the fluid between the vitreous and the retina and the occasional presence
of cells.
Clinically a similar controversy arose referring particularly to myopic
eyes. Early writers such as Brière (1875) and Galezowski (1877) described
a grey circular opacity seen in front of the disc in myopic eyes as a detach-
ment of the vitreous, a view strongly advocated by Weiss (1885–97), who
described the appearance of a reflex marking the posterior limit of the
vitreous some distance in front of the retina when the posterior pole of the
eye had elongated in myopia. A similar view was adopted by Dimmer
(1882) and Dor (1898), while Masuda (1913) explained a faint opacity in
this region in an emmetropic eye as probably a congenital malformation.
Thereafter, however, more modern methods of optical examination by the
arc lamp, the stereoscopic ophthalmoscope and eventually the slit-lamp
aided by a contact glass opened up the vitreous to more minute inspec-
tion, and it has been definitely established that detachments of this type
are relatively common, occurring not only in myopes but also in emmetropes
and hypermetropes in degenerative and inflammatory conditions. The

DISEASES OF THE WITREOUS BODY 3269
pioneer in these observations was Kraupa (1914–25) and his findings have
been amply confirmed by many others, particularly Lister (1922), Pillat
(1922–36), Comberg (1924), Vogt (1924–35), Isakowitz (1926–32), Amsler
(1930), Koby (1932), Anderson (1933), Rötth (1933–34), Sallmann and
Rieger (1934), Fodor (1935), Baenziger (1935), Sallmann (1935–36), Bassin
(1936), Cavka (1937), Churgina (1937), Kleefeld (1937), and others.
Such detachments may be of several kinds. A posterior detachment is
FIG. 2797.-DETACHMENT OF THE WITREous.
A. * superior detachment in an aphakic eye seen by the slit-lamp (Pillat, K. M.
*g.).
the commonest manifestation, occurring most usually in myopia, semility,
and chronic degenerative and inflammatory conditions, wherein the reflex of
the posterior surface of the vitreous, sometimes condensed into an observable
film, may be seen just anterior to the disc or
well forward behind the lens (Figs. 2795, 98).
Alternatively a superior detachment may occur,
when, presumably largely by the action of
gravity, the gel falls away from the upper
part of the globe (Fig. 2796): occasionally
such a detachment may be very extensive,
extending from the base of the vitreous at
the ora (where the retinal attachment is
usually firm) to below the disc, so that the
whole vitreous falls to the anterior and
- - - - - - - Fig. 2798.-Post ERIOR DETACH-
inferior portions of its original cavity MENT of THE WITREous (after
(Fig. 2797). An unusually extensive detach- *
ment of this type was observed by Pillat (1936) after an intra-capsular
extraction when the whole of the upper part of the vitreous gave way, its
anterior surface cutting across the aphakic pupil and its posterior surface
showing a ring-shaped tear above the disc. Finally, and more rarely, an
anterior detachment may occur, due usually either to trauma or to the
presence and presumably the contraction of pseudo-membranes in a
degenerating vitreous after irido-cyclitis (Comberg, 1924; Koby, 1932;
Fodor, 1935; Vogt, 1935; Bassin, 1936; Cavka, 1937).
Such detachments are frequently associated with an appearance sugges-


3270 TEXT-BOOK OF OPHTHALMOLOGY
tive of a thickening and opacification of the limiting layer of the vitreous,
which may be blurred, indefinite and shaggy, or clearly defined and
even thrown into delicate folds. Quite frequently also holes appear in this
layer (HYALoID Hours)—sometimes a single hole, and sometimes an area
Fig. 27.99 —VITRE- Fig. 2800. Witneous
Lºuis Detach- Detachment with Cox-
- E. N. T. w T. H. DENsation Opacity (Reiger,
Opacity. 4. f. O.).
Fig. 2801. Witneous Detach- Fig. 2802.-Vitreous DETAch-
MENT with Hole (Reiger, MENT witH. TRELLis-work
A. f. O.). - VITREous Opacity.
showing a mosaic of them—and not uncommonly a membranous opacity is
seen floating freely in the vitreous representing a torn area of this limiting
layer (Figs. 2799-2802). Thus in posterior detachments the vitreous sepa-
rates at the margin of the disc tearing away from the wide funnel-shaped
expansion of Cloquet's canal. Such a ring-shaped aperture is common in
myopia and semility and is best seen ophthalmoscopically by the are lamp
and the plane mirror or by the slit-lamp used by Koeppe's method with
a contact glass." The ring hovers at a variable distance in front of the
* Vol. II, p. 1147.


DISEASES OF THE WITREOUS BODY 327]
Optic disc, its edges forming an opacity, which when seen from the side may
appear oval and distorted, and with the passage of time may alter in
density, shape and position. Vogt (1924), who called the appearance the .
senile ring, considered it as characteristic of age as circum-papillary atrophy
and lens opacities. Similarly in optic neuritis a ring-shaped opacity of this
type may be formed (Cavka, 1937), as well as in association with localized
foci of chorio-retinal disease or degeneration situated at the macula or
eccentrically (Purtscher, 1919; Kraupa, 1914–25; Lister, 1922; Goldstein,
1923; Bedell, 1925; Isakowitz, 1926; Anderson, 1933; and others). It
would seem that as a sequel to any degenerative or inflammatory disease,
the vitreous becomes degenerated and shrinks and tends to become
detached ; the thickened limiting layer may become adherent at the site
of such a lesion so that, as the shrinkage proceeds, it becomes torn at this
point. Through such a tear the degenerated and fluid vitreous pours to fill
the space between the vitreous and the retina, and the membranous limiting
layer becomes thickened and contracted forming opacities which float about
in the liquefied gel.
The cause of such detachments has given rise to controversy. Iwanoff’s
(1869) theory, applied to myopic eyes in which the condition was first seen,
was expressed in the term detachment e vacuo-that the stretching of the
globe left a space between the vitreous and the posterior pole of the eye.
That such a factor has some considerable importance in the myopic eye is
undoubted, but it cannot explain the occurrence of the same phenomenon
in emmetropic eyes or in peripheral parts of any eye. In these a shrinkage
of the vitreous must be postulated, essentially of a degenerative nature.
Indeed, Lindner (1936) contended that such a contraction could be brought
about merely by increasing the protein content of the vitreous by capillary
dilatation such as follows the establishment of a fistula in the eye. It is
significant that all the conditions in which such detachments occur are
associated with the common factor of retino-choroidal changes of degenera-
tion or inflammation, whether they be of senility, myopia, uveitis, chorio-
retinitis, primary pigmentation of the retina, and so on (Rieger, 1937).
It would seem probable, therefore, that the main cause of these detachments
is identical with that determining the gross detachments in more highly
pathological eyes—a shrinkage of the vitreous itself.
The frequency of the occurrence of such detachments is considerable : thus
Churgina (1937) found a posterior detachment 9 times in 32 myopic eyes. In a more
extensive investigation Rieger (1936) investigated 321 eyes with simple myopia in
persons between 10 and 80 years of age : increasing with age and the degree of myopia,
vitreous detachment was found present in 188 and doubtful in 24 eyes. In 35 eyes,
of which the fellow had a detached retina, a vitreous detachment was marked in 28
and doubtful in 4. He also found a senile detachment of the vitreous in 68 eyes, in
7 out of 14 individuals with primary pigmentary degeneration of the retina, and in
29 out of 46 eyes with uveitis of various forms.
3272 TEXT-BOOK OF OPHTHALMOLOGY
It is, of course, possible for a detachment to occur by the pushing forward of the
vitreous by fluid collecting between it and the retina—as by a chorio-retinal exudate
or a sub-hyaloid haemorrhage.
The only clinical symptom associated with such detachments is the
occurrence of photopsiae at the time of their development. Sometimes the
detachment is accompanied by the development of a haze of vitreous
opacities; and occasionally the ring-like formation of a posterior detachment
or more irregular opacities are seen entoptically.
The main clinical interest, however, of such conditions is their relation
to retinal detachments. It is probable that the senile and myopic vitreous
detachments have no constant or immediate relation with a “ spontaneous”
detachment of the retina; and it is possible that the ring-shaped tear, which
completely releases the posterior vitreous from the retina, may act as a
substitute for a retinal detachment. The process which causes vitreous
detachment with the formation of “hyaloid holes' associated with scattered
foci of choroiditis or the peripheral degenerative lesions of senile and myopic
eyes, however, is probably, as we have seen,” of considerable aetiological
importance in the development of retinal detachments, for if the limiting
layer of the vitreous is adherent to the retina, a hole may be torn in the
latter tissue, the torn portion sometimes appearing as an operculum, which
floats as an opacity in the vitreous some distance away ; the formation of
such a hole in the retina may well be the immediate cause of a detachment
of this tissue.”
Amsler. Bull. S. fr. d’O., xliii, 315, 1930.
Anderson. Brit. J. O., xvii, 460, 1933.
Baenziger. A. f. O., czzxiv, 23, 1935.
Bassin. K. M. Aug., xcvii, 599, 1936.
Bedell. T. O. S., xlv., 646, 1925.
Brière. An. d’Oc., lxxiv, 138, 1875.
Cavka. A. f. O., cxxxvii, 472, 1937.
Churgina. Sov. vestm. O., xi, 13, 1937.
Comberg. K. M. Aug., lxxii, 695, 1924.
Dimmer. K. M. Aug., xx, 259, 1882.
Dor. B. O. G. Heidel.., xxvii, 321, 1898.
Elschnig. K. M. Aug., xlii (2), 529, 1904.
Fodor. K. M. Aug., xciv, 651, 1935.
Galezowski. Gaz. Méd., Paris, vi, 167, 1877.
Goldstein. A. of O., lii, 271, 1923.
Greeff. Anleitung d. mic. Untersuch. d. Auges,
Berlin, I902.
Isakowitz. K. M. Aug., lxxvii, 121, 1926;
lxxxviii, 369, 1932.
Iwanoff. A. f. O., xv (2), 31, 1869.
Rleefeld. Bull. S. Belge d’O., lxxiv, 21, 116,
1937.
Koby. Microscopie du corps vitré, Paris, 1932.
Kraupa. Z. f. Awg., xxxi, 149, 1914;
lxxxviii, 224, 1936.
R. M. Aug., lxx, 716, 1923;
1924; lxxv, 708, 1925.
Lindner. Z. f. Aug., lxxxvi, 70, 1935.
A. f. O., czzxv, 332, 1936.
Lister. Am. J. O., v, 488, 1922.
1 p. 2897.
lxxii, 476,
Masuda. K. M. Aug., li, 452, 1913.
Milles. R. L. O. H. Rep., xi, 26, 1886.
Müller. Sitz. phys.-med. Ges., Würzburg, 1856.
Parsons. T. O. S., xxv, 99, 1905.
Pillat. K. M. Aug., lxix, 429, 1922;
396, 1936; xcvii, 60, 1936.
2. f. Aug., lvii, 347, 1925; Iviii, 443, 1926;
lxi, 105, 1927; lxxxiv, 260, 1934;
lxxxviii, 34 ; likxxix, 244, 1936.
Purtscher. Z. f. Aug., xcii, 256, 1919.
Rieger. A. f. O., cxxxi, 410, 1934; cxxxvi,
119, 1937.
Z. f. Aug., lxxxix, 247, 1936.
K. M. Aug., xevi, 396, 695, 1936.
Rötth. K. M. Aug., xci, 682, 1933; xciii, 47,
1934.
Sallmann. Z. f. Aug., xxxvii, 1, 1935.
A. f. O., czz.xv, 593, 1936.
Sallmann and Rieger. A. f. O., czzxiii, 75,
1934.
Samuels. A. of O., iv, 838, 1930.
Vogt. K. M. Aug., lxxii, 212, 1924; 1xxv,
463, 1925; xcv, 94, 1935.
A. f. O., czzxiv, 1, 1935.
Z. f. Aug., lxxxviii, 1, 1935.
de Wecker. G-S. Hb., I, iv, 713, 1876.
Weiss. A. f. O., xxxi, 239, 1885.
Ueber d. Vorkommen v. Scharf begrenzten
Ektasien im Augengrunde bei hochgradiger
Myopie, Wiesbaden, 1897.
2 p. 2893.
xcvi,
DISEASES OF THE WITREOUS BODY 3273
PRol APSE of THE WITREous Body (VITREous HERNIA)
An ExTERNAL PROLAPSE of the vitreous body from the vitreous chamber
may occur in a perforating wound when the gel may bulge through the rent
in the sclera : this condition will be discussed in the section on Injuries to
the Eye.” An INTERNAL PRODAPsF, or HERNIA, wherein the gelcomes forward
into the anterior chamber, occurs commonly in three conditions:–
1. Following an intra-capsular cataract extraction.
2. Following the discission of the lens or an after-cataract (Fig. 2803).
3. In cases of dislocation or sub-luxation of the lens (Figs. 2769 and 2803).
It may be said always to occur in some degree on extraction of the lens
when the posterior capsule is opened or removed, and is always a post-
Fig. 2803.-SEcond ARY CATARACT AND WITREous PRollapse.
After discission of a congenital cataract (Bedell, T. O. S.).
traumatic or post-operative condition. It is therefore a common occurrence,
a fact well brought out by the statistics of the Giessen clinic as recorded by
Liebmann (1908), Bercovici (1915), Buschmann (1917), Kaulen (1917),
Tomforde (1919) and Schmitgen (1919).
The prolapse may assume two forms: a general bulging and a sacculated
form. The former is much the more common (Fig. 2803). As a rule after
both an intra-capsular extraction or a discission operation a very considerable
hernia into the anterior chamber occurs, which after some days may recede
so that eventually the anterior face of the vitreous returns behind the
pupil: there it may stay or the hernia may reappear later and persist. In
the same way in the case of a capsulotomy the retro-lentral space, which
was abolished immediately after the operation, tends to be reformed so
1 Vol. IV.

74 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2804 –VITREous PRollapse.
After dislocation of the lens and an iridectomy for complicated glaucoma. The
vitreous face, powdered with blood-cells, almost touched the cornea (Bedell, T. O. S.).
Fig. 2805–HAEMoºrhage into a VITREous Pouch.
A biloculate pouch of vitreous prolapsing into the anterior chamber containing
blood after a cataract extraction (Harrison Butler, Brit. J. O.).


DISEASES OF THE WITREOUS BODY 3275
that there is a clear space between the capsular remains and the anterior
surface of the vitreous. Sallmann (1936) associated the original large pro-
trusion after extraction and its subsequent recession with the development
and disappearance of apost-operative choroidal detachment." Gentle massage
of the globe will always push out a prolapse further, and a sudden trauma,
a physical exertion, or even a maximal dilatation of the pupil is sufficient
to increase it, or induce a prolapse when formerly none existed. A sacculated
Fig. 2806.-IN corportATIox of THE WITREous IN A CATARAct Wound.
Vertical lines of tension are seen in the vitreous running towards a detached
retina (Shapland, T. O. S.).
prolapse, on the other hand, is rare (Fig. 2805) and only a few cases are
reported in the literature (Siegfried, 1896; Haab, 1900; Hesse, 1919;
Cowan, 1932; Butler, 1933; Paula-Santos, 1936). In this type one or
more distinct sac-like pouches are formed which hang down in the anterior
chamber and frequently contain haemorrhage. It is as if the anterior limiting
layer of the vitreous had ruptured, but the indiscriminate protrusion forward
of the gel had been prevented by the rapid formation of a weaker surface
condensation of sufficient tensile strength to retain the gel within it.
The extent and density of the prolapse may vary very considerably.
It may be so delicate as to escape observation unless specially looked for.
1 p. 2541.

3.276 TEXT-BOOK OF OPHTHALMOLOGY
It may seem to have a single membranous covering, or it may be grossly
laminated, composed as it were of a series of pseudo-membranes of some
thickness, each showing a glassy contour and each bestrewn by fine pig-
mentary remnants. The surface may be quite smooth and covered with
what appears to be a “hyaloid membrane" of new-formation (Cowan,
1932) behind which a zone of optically empty vitreous intervenes in front of
the more dense structure of the mass of the gel. The apparently distinct
surface membrane is probably a mechanical effect of the tension and stress
Fig. 2807.-PRollapse of VITREous INTo A Wound.
After a cataract extraction in which a prolapse of vitreous occurred. There is a
mass of inflammatory tissue sub-conjunctivally and extending along the wound and
continuing posteriorly into the adherent vitreous (Collins, T. O. S.).
created by the hernia on the micellar structure of the gel causing a re-
arrangement, a re-orientation and a condensation of its fibrillar elements.
Occasionally this surface condensation has a central hole through which the
semi-fluid primary vitreous pours into the anterior chamber (Samuels,
1930; Bassin, 1936). At other times the anterior surface is frayed and
broken showing teased-out cotton-like fibres which may wave freely in the
anterior chamber or become adherent to the capsule or the iris. Again,
vitreous débris may appear on the posterior surface of the cornea as a
frayed white dust, or a dense band of vitreous may run like a synechia to the
back of the cornea or the limbal scar where it remains adherent (Butler,
1927; Giovanni, 1933; and others) (Figs. 2806–07). Finally the entire
anterior chamber may be filled with a formless gelatinous mass. In all cases

DISEASES OF THE WITREOUS BODY 3.277
there tend to be some white cellular elements and a considerable amount
of pigment both of uveal and haemorrhagic origin in the hermiated gel.
A hernia of the vitreous gives rise to no symptoms of itself, and it is
often surprising how much vision may be obtained when the pseudo-
membranes appear of considerable density. The most frequent complication
is the occurrence of a secondary glaucoma, an occurrence not very common
considering the frequency of prolapses.
Such a rise of tension occurring particularly after a capsulotomy may be
due to a number of causes. The cases which occur early associated with
inflammatory evidences are probably due to irido-cyclitis set up or aggra-
vated by traction on the ciliary processes as was hypothecated by v. Graefe
(1869). Cases of a more chronic nature occurring later are probably directly
Fig. 2808.-VITREous ProLAPsF AFTER Dislocation of THE LENs.
After a couching operation. Note the marked condition of iris bombé with the
prolapse of vitreous, and the secondary formation of peripheral synechiae (Hudson,
R. L. O. H. Rep.).
due to the vitreous. Some of them are associated with a deep anterior
chamber and a flat iris; these are probably cases of obstruction at the
angle of the anterior chamber by the gel so that the normal drainage of
the intra-ocular fluid is interfered with (Cheney, 1909): signs of inflammation
are absent, the vitreous is usually abnormally fluid, and the condition is
usually relieved by eserine and heat in quite a dramatic manner. A second
type of case is associated with a bombé condition of the iris and is not
relieved by eserine (Bowman, 1865; Knapp, 1894). This type is probably
due to an obstruction of the pupil owing to the hernia through it, a consequent
over-distention of the posterior chamber with the formation of an iris
bombé, and subsequent adhesions of the iris to the cornea obliterating the
drainage angle (Hudson, 1911) (Fig. 2808). As treatment Bowman (1869)
advocated atropine to relieve strangulation at the pupil, failing this an iris
puncture was practised by Bowman (1869) or an iridectomy by Knapp (1894),
but probably the most generally successful operation is a cyclodialysis.

3278 TEXT-BOOK OF OPHTHALMOLOGY
Bassin. K. M. Aug., xcvii, 599, 1936. Haab. Z. f. Aug., iii, 117, 1900.
Bedell. T. O. S., xlv., 646, 1925. Hesse. Z. f. Aug., xlii, 191, 1919.
Bercovici. Diss., Giessen, 1915. Hudson. R. L. O. H. Rep., xviii, 203, 1911,
Bowman. R. L. O. H. Rep., iv, 332, 1865. Kaulen. Diss., Giessen, 1917.
Buschmann. Diss., Giessen, 1917. Knapp, H. A. f. Aug., xxx, 1, 1894.
Butler. Illustrated Guide to the Slit-lamp, Liebmann. Diss., Giessen, 1908.
p. 121, Oxford, 1927. Paula-Santos. A. d’O., liii, 876, 1936.
Brit. J. O., xvii, 343, 1933. Sallmann. A. f. O., cxxxv, 602, 1936.
Cheney. Ophthalmoscopy, v, 27, 1909. Samuels. A. of O., iv, 838, 1930.
Cowan. Am. J. O., xv, 125, 1932. Schmitgen. Diss., Giessen, 1919.
Giovanni. Am... di Ott., lxi, 904, 1933. Siegfried. Beit. z. Aug., xx, 1, 1896.
v. Graefe. A. f. O., xv (3), 108, 1869. Tomforde. Diss., Giessen, 1919.
IV. CYSTS IN THE WITREOUS
Cysts in the vitreous are very rare, so rare, indeed, as to be curiosities
in ophthalmic literature ; it is possible, however, that the usual absence of
symptoms and the difficulty of seeing them since they are transparent may
allow cases to be missed in routine examination. Usually they are trans-
parent, or almost so, with a spherical or bumpy outline and a scarcely visible
wall, sprinkled over with pigment and sometimes wrinkled so as to give the
appearance of grey hyaline areas, sometimes floating freely in the vitreous
and sometimes anchored in one region by one or more delicate strands.
Their aetiology in the complete lack of pathological material is speculative ;
probably the majority are congenital while others seem to be associated
with inflammatory and degenerative conditions. The following causes
suggest themselves —
1. Congenital cysts associated with (i) remains of the hyaloid system and other
mesodermal elements in the vitreous (Koller, 1901, and others), or with the
tissues of Bergmeister’s papilla (Cassady, 1939);
(ii) cysts of the foetal cleft.
. Cysts of the ciliary body.
. Cysts formed from inflammatory exudative material or haemorrhage.
. A product of retinal proliferation or degeneration.
. Parasitic cysts.
:
Congenital cysts have already been described 1 and parasitic cysts will
be discussed presently.” For the rest only vague guesses can be made.
In discussing the first case to be described, that of Tansley (1899), Alt
suggested that it represented a ciliary adenoma which had undergone cystic
degeneration and had broken loose. Troncoso (1903), in describing a similar
case, suggested that it arose from a degenerated ciliary process filled with
aqueous. Again, the occurrence of cysts in association with tuberculous
retinitis, irido-cyclitis with much exudate, or following a pre-retinal haemor-
rhage suggests a possible aetiology in inflammatory exudative material
or an old haemorrhage (Brewerton, 1913; Tertsch, 1937); while
* Vol. II, p. 1392. * p. 3438.
DISEASES OF THE WITREOUS BODY 3279
the very occasional appearance of bilateral cysts in high myopia (Vennin,
1910), and primary pigmentary degeneration of the retina (Litinsky, 1931 ;
Perera, 1936), although not excluding a congenital origin, indicates a
possible connection with retinal degeneration.
Brewerton. T. O. S., xxxiii, 93, 1913. Tansley. T. Am. O. S., viii, 507, 1899.
Cassady. A. of O., xxi, 45, 1939. Tertsch. Z. f. Aug., xciii, 121, 1937.
Koller. T. A. m. O. S., ix, 380, 1901. Troncoso. An. d’Oc., czzx, 341, 1903.
Litinsky. K. M. Aug., lxxxvii, 205, 1931. Vennin. Lyon Méd., czix, 974, 1910.
Perera. A. of O., xvi, 1015, 1936.
T.O.-WOL. III 4 G
CHAPTER XL
ANOMALIES OF THE INTRA-OCULAR PRESSURE
GLAUCOMA
THE term GLAUCOMA does not connote a disease-entity, but embraces a
composite congeries of pathological conditions which have the common feature
that their clinical manifestations are to a greater or less eatent dominated by an
increase in the intra-ocular pressure and its consequences. The degree of
raised pressure which assumes a pathological significance is impossible to
define since it varies within wide limits from one eye to another : it may
be taken as that pressure which the tissues of the particular eye in question are
wnable to withstand without damage to their structure or impairment of their
function. Despite the enormous amount of work and speculation which has
been expended upon the subject, the rationale of the increase in pressure is
still far from being satisfactorily elucidated. It is true that in the more
obvious cases the pathology is plain ; but in the more numerous less obvious
cases the subtle initial derangements, which are probably physico-chemical
in nature, have so far eluded investigation, and that probably for two
reasons. To some extent this is because the raised intra-ocular pressure, the
most prominent feature in the clinical picture, has generally been seized
upon, and instead of being considered as a symptom, has been regarded as
if it were the disease-process itself. To speak of the pathogenesis of glaucoma
is therefore not justified unless on the understanding that the concept
embraces the aetiology of many diseases all of which are characterized by a
common symptom. Moreover, the insidiousness of the origin of many types
of hypertension makes real aetiological research unusually difficult, and those
eyes which become available for biochemical or pathological study are
usually in the terminal stage when the initial defect has long been obscured
in the ruins of advanced disease.
Historical
Although the word glaucoma appeared in the writings of Hippocrates, the concep-
tion of a disease associated with a raised intra-ocular pressure is of a much later date.
In the Hippocratic Aphorisms the term y\avkajorets (greenish or bluish) was used to
describe blindness coming on in advancing years associated with a greenish or bluish
appearance of the pupil—“If the pupil become sea-coloured sight is destroyed and
blindness of the other eye often follows.” The term was used without any specific
pathological connotation and represented no morbid entity, but probably included
absolute glaucoma, ; chronic glaucoma as we know it to-day was quite unrecognized
and was classified as amblyopia, amaurosis, or gutta serena. Originally, indeed, the
3280
ANOMALIES OF THE INTRA-OOULAR PRESSURE 3281
Fig. 2809.-PRIESTLEY SMITH.
1845–1933.
(From a photograph of the portrait by Harold Speed, Brit. J. O.).

4 G
3282 TEXT-BOOK OF OPHTHALMOLOGY
condition was undifferentiated from cataract, and it was only later recognized by
Galen and other writers of the early Christian centuries that the morbid conditions
situated behind the pupil which gave rise to blindness could be differentiated into two
groups, “suffusions" (Öröxvows) or cataracts which were amenable to operative
treatment, and the glaucomata which were not. Even then the most hazy ideas were
prevalent about the pathology of the condition. Maitre-Jan (1650–1730) contended
that both diseases resided in the lens, a conception disproved by Brisseau (1709) who
located cataract in this tissue and disproved any lenticular abnormality in glaucoma
by the anatomical examination of the eyes of Bourdelot, the blind physician of Louis
XIV; he suggested that the seat of the disease was the vitreous, a view supported by
Joseph Beer (1792), Middlemore (1835) and other authorities. Others attributed the
blindness to disease of the optic nerve (Wenzel, 1808; Wardrop, 1828; Tyrrell, 1840)
or the choroid (Mackenzie, 1830 ; Lawrence, 1833; v. Ammon, 1838).
The first clear recognition of a separate morbid entity corresponding to what is
now known as glaucoma seems to occur in the writings of the Arabian Sams-ad-din
(?–1348), who described among the ophthalmias a “migraine of the eye ’’ or “head-
ache of the pupil,” an illness associated wih pain in the eye, hemicrania and dullness
of the humours, and followed by dilatation of the pupil and cataract : if it became
chronic, tenseness of the eye and blindness supervened. The first clear recognition of
absolute glaucoma in European writings is due to Richard Banister (1622), an itinerant
English oculist and author of the first formal book on ophthalmology in English ; he
clearly differentiated between curable cataract and gutta serena wherein “the humour
settled in the hollow nerves, be growne to any solid or hard substance, it is not possible
to be cured,” and gave a tetrad of symptoms—tension (“if one feele the eye by rubbing
upon the Eie-lids, that the Eye be growne more solid and hard than it naturally
should be '’), as well as long duration, no perception of light and no dilatation of the
pupil. This very excellent description seems, however, to have passed unnoticed
although the same four points were quoted by Sir William Read (1706), the oculist to
Queen Anne, who held that glaucoma “proceeds from the viciated crystalline humour,”
and, not without boasting, extolled the good results obtained by performing paracen-
tesis on a patient, Jeremiah Puttiford, in 1705. Although the fact of hardness of the
eye was also noted by J. Z. Platner (1738) in Germany, the symptom-complex of acute
glaucoma was not adequately recognized until the detailed clinical description of Beer
(1792). He, however, although giving an accurate clinical description of neglected
acute glaucoma, omitted the cardinal feature of a raised tension and attributed the
condition to an iritis of gouty origin : for such a condition Sir William Lawrence
introduced the term ACUTE GLAUCOMA in 1829. Finally, the essential feature of
raised tension was fully established by William Mackenzie (1830), who in his
classical text-book ascribed the raised tension to an increase in the watery contents of
the globe owing to a serous choroiditis, and employed puncture of the vitreous for its
relief. To this great Scottish clinician, whose photograph forms the frontispiece of
this volume, must be given credit for the final establishment of the diagnosis between
cataract and glaucomatous amaurosis, the propounding of a rational pathology of
the latter condition, and a reasoned suggestion for its relief. -
The next epoch in the history of glaucoma, followed the introduction of the
ophthalmoscope, when clinical observations on the glaucomatous cup began to accumu-
late (Jacobson, 1853; Jaeger, 1854; v. Graefe, 1854–57; Weber, 1855; and others),
observations which were confirmed by the pathological researches of Heinrich Müller
(1856). It is interesting that both Jaeger (1854) and v. Graefe (1854) originally
described the appearance as a swelling of the disc, a mistake corrected by Weber (1855)
and by v. Graefe himself in the same year. When this had been established, the fact
that hypertension was the essential cause of acute glaucoma was universally admitted
and Mackenzie’s contention resuscitated. The final important clinical observation
ANOMALIES OF THE INTRA-00ULAR PRESSURE 3283
Fig. 2810,-FELIX LAGRANGE.
1855–1927.

3284 TEXT-BOOK OF OPHTHALMOLOGY
was contributed by the unifying conception of Donders (1862), who recognized in the
last of v. Graefe’s (1857) four sub-divisions—acute, absolute, and secondary glaucoma,
and amaurosis with excavation of the disc—the condition of simple chronic glaucoma
wherein an incapacitating increased tension occurred without any inflammatory
Symptoms.
Thereafter concern was made with the aetiology of the disease. A great variety
of suggestions followed that of Beer’s (1792) gouty iritis, and Mackenzie’s (1830)
serous choroiditis—irritation of the secretory nerves of the eye (Donders, 1862; de
Wecker, 1863), thickening of the sclera (Cusco, 1862; Coccius, 1863; Adamūck,
1867; v. Graefe, 1869), a hydrops of the vitreous (Stilling, 1868), and so on—all of
which stimulated an immense amount of research on the nature of the intra-ocular
fluid, a subject which is by no means clarified yet. The first clue based on proved
pathological facts was announced by Max Knies (1876) and Adolph Weber (1876),
working independently, who discovered the frequency of obstruction of the angle of
the anterior chamber in glaucoma, a clue which was taken up and elaborated by Priestley
Smith (1879) of Birmingham. The classical researches of this author, who devoted
much of his lifetime to the subject and stressed faulty drainage rather than over-
production of fluid, sought the immediate cause of glaucoma in abnormalities at the
angle of the anterior chamber. Although his conceptions account Satisfactorily for
many features of this disease, his relatively simple mechanical hypothesis is by no
means exhaustive ; but since scientific work upon the subject has been based essen-
tially upon his labours, it is only fitting that his portrait should form the prelude to
this chapter (Fig. 2809). 3.
The treatment of glaucoma, as we have just noted, may be said to have begun
with the advice of Mackenzie (1830) to relieve the tension of the eye by a paracentesis
or a sclerotomy. At best, however, this was a temporary measure, and until the
ophthalmoscopic era, the conception of raised intra-ocular pressure as the dominating
cause of the disease had not caught the general imagination. Until the time of v.
Graefe, therefore, glaucoma was generally recognized to be incurable and the establish-
ment of the diagnosis was tantamount to a sentence of blindness. v. Graefe (1857),
however, observing a recession of staphylomata after iridectomy, presumably
owing to a lowering of tension, applied the operation to acute glaucoma, and thereby,
Somewhat by chance, inaugurated a revolution in ophthalmic surgery. In any history
of the disease the introduction of this operation must serve as a landmark : v. Graefe’s
photograph has already introduced the second volume of this text-book (Fig. 1023).
The second innovation of first magnitude was the discovery of the efficacy of miotics
by Laqueur (1876) of Strasburg, a discovery which curiously aroused the fiercest
opposition. The applicability of the operation of iridectomy excited much controversy,
its theoretical justification was extremely vague, and its results by no means uniform,
and it is to the credit of v. Graefe’s pupil and ardent admirer in France, de Wecker
(1867–1901), who looked upon the condition essentially as a disturbance between the
Secretion and excretion of intra-ocular fluid, that attempts were made to realize the
end which v. Graefe only dimly appreciated, and re-establish permanent drainage
through a filtering scar. It is true that he failed to attain this end satisfactorily, but
his work stimulated research on this all-important subject ; from it there resulted the
introduction of an operation for the production of a fistula by iris-inclusion by Herbert
(1903) of Bombay, a principle popularized in the iridencleisis of Holth (1907) of Oslo;
the development of cyclodialysis by Heine (1905) of Breslau ; and its stimulus culmi-
nated in the brilliant achievement of sclerectomy by Felix Lagrange (1905) of Bordeaux.
This Operation and the other filtration procedure based upon it, the corneo-scleral
trephining of Elliot (1909), at once attracted universal attention, and did more than
anything else to revolutionize the treatment of chronic glaucoma. The photograph
of the great French surgeon is found in Fig. 2810.
ANOMALIES OF THE INTRA-OCULAR PRESSURE
3285
Adamūck. An. d’Oc., lviii, 5, 1867.
v. Ammon. Klin. Darstellungen, Berlin, 1838.
Banister. A Treatise on 113 Diseases of the
Eyes and Eyelids, 1622. -
Beer. Die Lehre v.d. Augenkrankheiten, Wien,
1792.
Brisseau. Traité de la cataracte et du glaucome,
Paris, 1709.
Coccius. A. f. O., ix (1), 1, 1863.
Cusco. Am... d’Oc., xoviii, 291, 1862.
Donders. A. f. O., viii (2), 160, 1862.
Elliot. Ophthalmoscope, vii, 804, 1909.
v. Graefe. A. f. O., i (1), 371, 1854; (2), 299;
ii (1), 248, 1855; iii (2), 456, 1857;
iv (2), 127, 1858; vi (2), 150, 1860;
viii (2), 242, 1862; ix (2), 110 ; (3), 126,
1863; xv (3), 118, 1869.
Heine. K. M. Aug., xliii (2), 164, 1905.
Herbert. O. Rev., xxii, 234, 1903.
T. O. S., xxiii, 324, 1903.
Holth. Am. d’Oc., czzxvii, 345, 1907.
Jacobson. Diss., Königsberg, 1853.
Jaeger. Star w. Star-operationen, Wien, 1854.
Knies. A. f. O., xxii (3), 163, 1876.
Lagrange. Soc. méd. de Bordeaua, Jan. 8th,
1905.
Cb. med. Wiss., 752, 1121, 1876.
Lawrence. Treatise, London, 1833.
Mackenzie. Treatise on Diseases of the Eye,
London, 1830.
Middlemore. Treatise on the Diseases of the
Eye, London, 1835.
Laqueur.
Müller. Gesam. u. hinter. Schriftem, i, 340,
1856.
Platner. De motu ligamenti ciliaris in oculo,
Leipzig, 1738.
Read. A Treatise of the Eyes, 1706.
Smith, Priestley. Glaucoma, London, 1879,
1891.
Stilling. A. f. O., xiv. (3), 259, 1868.
Tyrrell. A Practical Work on Diseases of the
Eye, London, 1840.
Wardrop. Morbid Anat. of the Human Eye,
London, 1828.
Weber. A. f. O., ii (1), 140, 1855; xxiii (1), 1,
1876.
de Wecker. Traité theoretique et pratique des
maladies des Yeuac, Paris, 1863–7.
Am... d’Oc., cxi, 321, 1894; cziv, 95, 1895;
cxvi, 249, 1896; cxxi, 321, 1899 ;
cxxiv, 337, 1900; cz.xv, 421, 1901.
Wenzel. Manuel de l’Oculiste, Paris, 1808.
A. d’O., xxvi, 481, 1906.
AEtiologically, pathologically and clinically the term glaucoma includes
so many disease-entities that it is impossible to consider them as different
manifestations of the same condition. In the present state of our ignorance,
however, it is useful to admit a classification into two groups :-SECONDARY
GLAUCOMA, wherein the symptom of raised pressure is due to some obvious
ocular lesion which is known, and PRIMARY GLAUCOMA, wherein the raised
pressure is due to some inobvious cause at present unknown. It is true
that all cases of glaucoma are secondary to some condition or other, so that
such a classification is unreal and unscientific in so far as its boundaries are
drawn not by natural phenomena but by the state of human knowledge;
but at the present stage it forms the most useful basis for description and
clinical study. Since it is easier to pass from the known to the unknown, and
since the facts discovered regarding the former throw a considerable light
upon the obscurities of the latter, we shall deal with the problems of
secondary glaucoma in the first place.
Secondary Glaucoma
SECONDARY GLAUCOMA may therefore be defined as a loosely-knit and
wnrelated clinical group of cases the only common denominator among which is
the fact that some recognized pathological lesion is complicated by an increase
in the intra-ocular pressure with attendant symptoms. Such an occurrence is
not at all rare, and statistics as to its incidence vary very widely with the
care with which the previous disease is diagnosed : average statistics vary
between 20 and 40% of all cases of raised tension (Carvill, 1932; Lehrfeld
3286 TEXT-BOOK OF OPHTHALMOLOGY
and Reber, 1937), but many authors claim that if more care were taken to
recognize cases of low-grade sub-threshold types of intra-ocular inflammation,
the proportion would be much higher. Indeed, it has been said that if
glaucoma is not post-inflammatory or due to lenticular changes, it is secon-
dary to vascular disease (Redslob, 1935). The increase in tension and the
symptoms to which it may give rise may be violent and acute or insidious
and chronic, but in all cases the complication should be looked upon as a
serious one, for the treatment frequently involves problems the solution of
which requires much clinical judgement, and in a large proportion of cases,
despite the greatest care and the largest experience, the end-results are bad.
The aetiological conditions are many and various. It may be said in
general terms that in the great majority of cases there is A DISTURBANCE
of THE INTRA-oCULAR CIRCULATION TO WHICH IS FREQUENTLY ADDED AN
obsTRUCTION TO THE CIRCULATION OF THE INTRA-OCULAR FLUIDS, sometimes
at the pupil and more frequently at the drainage angle. The classical
conception, propounded particularly by Priestley Smith (1891), was that the
second factor was the essential one and in most cases the only one ; but
that this is true is very questionable. It is, indeed, much more probable
that SECONDARY GLAUCOMA. HAS ESSENTIALLY A VASCULAR BASIS, and that
BMBARRASSMENT OF THE DRAINAGE CHANNELS, WHICH IS USUALLY BUT
NOT INVARIABLY PRESENT, IS AN ADJUVANT BUT BY NO MEANS A
NEGLIGIBLE FACTOR IN THE AETIOLOGY.. The vascular disturbance is the
development of a state of congestion and stasis and subsequent tissue-
oedema brought about either by actual obstruction of the veins draining
the uveal tract, or by a dilatation and increased permeability of the
capillary-venous system induced by vaso-motor reflexes, an effect spread
throughout the uveal tract through the mediation of axon reflexes, and
frequently instituted by the liberation in situ of histamine-like substances
(Duke-Elder, 1931). The production of such a vascular “flare '' throughout
the uveal tract not only results in the development of a high capillary pressure
and the formation of a colloid-rich plasmoid aqueous, but disorientates
entirely the normal dialysation of the intra-ocular fluid. If the drainage
channels are freely open, a pathological rise of tension may to a large extent
be compensated, but when, as frequently occurs in such cases, they are
impeded and their safety-valve mechanism rendered ineffective by patho-
logical alterations, a secondary glaucoma develops much more readily.
When the drainage channels are completely obliterated, as occurs in
congenital deformities or essential atrophy of the iris, a pathological rise of
tension tends to occur gradually and insidiously, for even in the absence of
an acute vaso-motor upset, the circulation is unable to accommodate
physiological variations of pressure.
In so far as the conditions with which a secondary glaucoma is associated
have all been discussed in detail in this book, it will be sufficient to recapitu-
late them briefly here ; and in the following paragraphs attention will be
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3287
focused on the aetiology and pathology of the causal factors with a note on
the specific therapeutic measures advisable for each particular condition,
while a fuller discussion of the pathology and symptomatology of the
hypertensive state per se will be reserved for the section on primary glaucoma.
1. GLAUCOMA SECONDARY TO INTRA-OCULAR INFLAMMATIONS
We have already discussed at length the fact that a raised tension is not
an uncommon, and too frequently a formidable complication of intra-ocular
inflammations of all types, most typically of an irido-cyclitis, less commonly
of choroiditis or retinitis, and frequently of a keratitis and a scleritis.
Although all aspects of the mechanism of this complication are by no means
thoroughly understood, it is convenient to divide such cases into two
categories, the one in which the raised tension occurs as a complication of
the inflammatory process itself (INFLAMMATORY GLAUCOMA), and the other
wherein the complication results from changes of a lasting character
produced by the inflammation (POST-INFLAMMATORY GLAUCOMA).
(a) INFLAMMATORY GLAUCOMA
From the clinical point of view inflammatory glaucoma may be divided
into two types depending on whether the tension complicates an acute or
sub-acute inflammatory process : in the first the symptoms of inflammation
dominate the picture in which the raised tension is obviously secondary,
while in the second the glaucomatous symptoms may occur with evidences
of inflammation so slight that their inflammatory origin frequently escapes
notice.
(i) Acute Inflammations
Acute irido-cyclitis provides the most typical picture in this group of
cases. Here the tension usually rises a few days after the onset of the
illness when the picture of acute irido-cyclitis, frequently characterized by a
liberal outpouring of keratic precipitates and a deep anterior chamber, is
suddenly intensified by increased congestion and the development of a
steamy cornea, while the pain becomes more unbearable and the vision much
more blurred. In its aetiology such a rise of tension, which may follow the
administration of a mydriatic, is presumably comparable with the acute
congestion which characterizes any inflamed organ. In the vascular stasis
which ensues, the uveal capillaries are intensely dilated, the permeability of
their walls increased, and the aqueous becomes converted into a colloid-rich
Oedematous fluid in which circumstances the whole of its balanced inter-
change is thrown out of gear. There is no doubt that the complete upset of
the osmotic and hydrostatic forces dominating the fluid-traffic in the eye
due to the vascular stasis and Oedema accompanying the inflammation are
sufficient in themselves to cause an acute rise in tension, corresponding to
3288 TEXT-BOOK OF OPHTHALMOLOGY
the state of a violently inflamed, swollen and Oedematous gland or joint, but
in addition, the condition may be, and frequently is, rendered more acute
by the fact that the drainage channels, which in the Ordinary course of
events can deal with a considerable surplus of fluid, may become clogged
with the proteins and cellular elements which in these circumstances usually
abound in the aqueous and accumulate in the spaces of Fontana and the angle
of the anterior chamber. Pathologically, therefore, although a blockage of
the excretory channels can frequently be demonstrated, they may be patently
open and the anterior chamber may be deep. The liberation of histamine-
like substances in the eye on injury and the spread of their vascular effects
over the uveal tract by axon-reflexes causing acute vaso-dilatation, stasis,
and massive and general oedema throughout the ocular tissues is sufficient
to account for the development of these hypertensive phenomena without
the added complication of obstructed excretion. It is noteworthy, for
example, that the anterior chamber may be filled with an almost solid
gelatinous exudate in an acute plastic (gonococcal) irido-cyclitis without a
rise of tension sufficient to cause symptoms. It is probable, therefore, that
although an embarrassment of the excretory channels will certainly be a
predisposing factor of no inconsiderable importance, particularly in bringing
on an acute exacerbation, the primary fault lies in the static vascular pheno-
mena of inflammation, a circumstance of great importance in the treatment
of such cases.
Severe keratitis also figures in the aetiology of inflammatory glaucoma,
whether it be an interstitial keratitis 1 or corneal ulcers, more particularly a
hypopyon ulcer * of which disease a raised tension forms a serious complica-
tion : herpes simplex or zoster may also be associated with a severe and
recalcitrant glaucoma.” In scleritis, also, a secondary rise of tension is a
frequent and sometimes very persistent complication.” In all these condi-
tions, of course, the uveal tract is also involved in the inflammatory process,
so that the mechanism of the glaucoma is similar, with the probable
complication that vascular stasis is increased by perivascular infiltration
around the anterior ciliary and vortex veins.
(ii) Chronic Inflammations
The chronic inflammations of the weal tract form a more difficult problem
in their clinical recognition and frequently demand more judgement in their
treatment. The clinical picture may resemble an acute or a chronic glaucoma.
In the first case there may be a history of transient obscurations of vision
with the appearance of halos appearing and disappearing over a period of
some years, usually occurring in a young person, which culminates in an
acute hypertensive attack characterized by congestion of the eye, dilatation
of the pupil, a steamy cornea, a shallow anterior chamber, neuralgic pain
1 Vol. II, p. 1976. * Vol. II, p. 1925.
* Vol. II, p. 1905. * Vol. II, p. 2056.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3289
and rapid failure of vision. The picture, indeed, resembles a straight-
forward primary acute glaucoma, but careful examination with the slit-lamp,
which should be done meticulously in all such cases and may be difficult
owing to the corneal haze, reveals the presence of one or two minute keratic
precipitates which betray the real nature of the condition. In other cases
the typical picture of chronic glaucoma develops, usually with great
insidiousness, the discs becoming cupped and scotomata gradually extending;
and again it is only by careful clinical examination that the presence of fine
vitreous opacities and inconspicuous keratic precipitates reveals the existence
of a quiet anterior uveitis (serous cyclitis),” despite the existence of a mobile
pupil.
Opinions vary very considerably as to the frequency of the incidence
of this type of glaucoma, and there is no doubt that since the slit-lamp has
become generally employed, its recognition has been very much more
frequent. Some authorities, indeed, go so far as to say that the majority
of cases formerly called “primary '' come under this category (Risley, 1914;
Malling, 1923; Larsen, 1924; Redslob, 1935). Malling (1923), for example,
found precipitates in 62 out of 71 cases which would ordinarily have been
accepted as simple glaucoma and concluded that the majority of cases of so-
called primary glaucoma are in reality secondary to irido-cyclitis. This view
is probably extreme ; but that such cases are not uncommon and are frequently
misinterpreted is undoubted, and it is likely that into this category should
come many eyes which at first do well after a filtering operation undertaken
for primary glaucoma, but later show an increasing tension and ultimately do
badly, or those which subsequently to operation show progressive evidences
of degeneration in the iris, lens and vitreous, due rather to a chronic toxaemic
than to a glaucomatous process (Wilder, 1930; Weingott, 1936; and
others).
The aetiology of these cases is disputed. It is probable that most of them
are inflammatory in type although sub-threshold in degree, depending
particularly on tubercle, syphilis, and those mild and chronic foci of infection
to which the uveal tract is so susceptible but are so difficult to inculpate and
more difficult to eliminate ; but it must be remembered that the presence
of keratic precipitates may not necessarily indicate an inflammation due to
toxic or organismal causes, but may possibly result from a simple vascular
stasis. A comparison with neurogenic heterochromic cyclitis due to a lesion
of the sympathetic nerve suggests itself here, or an analogy with angio-
neurotic oedema (Kraupa, 1935; Redslob, 1935). The mechanism of the
rise of tension is again probably vascular in origin, depending primarily, as
in the case of the acute inflammatory types, on stasis and Oedema. But here
again, it is undoubted that a very important factor in many cases is probably
an embarrassment of the filtration angle by the invasion of the spaces of
Fontana at first by exudative cells, and then by granulation tissue which
1 p. 2236.
3290 TEXT-BOOK OF OPHTHALMOLOGY
may consolidate, so that this type of case merges into the class of post-
inflammatory glaucomas. Some, indeed, would claim that this is the
essential factor rather than the inflammation itself; and it is arguable that
in many such cases we are dealing with eyes predisposed to glaucoma in
which the excretory channels have lost their normal and considerable
elasticity in dealing with variations in the flow of the intra-ocular fluid, and
have retained only a meagre margin of safety owing to anatomical changes
at the filtration angle. The clinical conduct of such cases, however, and their
tardy and unpredictable response to treatment, indicate the conclusion which
we shall reach in discussing primary glaucoma—that they are not altogether
explicable on a thesis of mechanical blockage, but depend on a more funda-
mental mechanism, in this case the vascular stasis and Oedema determined
by chronic inflammatory changes.
The treatment of inflammatory glaucoma always presents difficult and
anxious problems some of which have already been touched upon." It goes
without saying, of course, that the usual treatment of the inflammatory
condition should be undertaken with the utmost thoroughness—a search
for and an elimination or neutralization of the cause, general treatment with
salicylates, sulphanilamides, or otherwise as indicated, together with local
treatment with heat, leeches (frequently of great value), diathermy or
short-waves, and so on.
With regard to the treatment of the actual complication of the raised
tension the choice lies between medical and surgical measures, and it may be
said at once that in general the more apparent the inflammatory evidences the
less the indications for surgery, a ruling which applies even although the
conditions look alarmingly acute. Of the two alternatives which present
themselves of lowering the tension with miotics or relieving the inflammation
by mydriatics, the latter should always be chosen, since the tension is
secondary to the inflammation, and the first course, by increasing irritability,
usually results in the establishment of a vicious circle and the aggravation
of the symptoms. In these acute inflammatory cases epinephrine and its
derivatives are dangerous. In such cases, therefore, despite the raised
tension, atropine is indicated, particularly if the anterior chamber seems
unimpeded : in this event atropine should be pushed or combined with
adrenaline and cocaine as a sub-conjunctival injection to attain synergic
mydriasis. In these circumstances eserine usually makes the inflammatory
condition worse, aggravates the congestion and predisposes to the formation
of synechiae; but with the establishment of efficient mydriasis, the acute
inflammatory symptoms usually become controlled and the tension falls in
proportion. This behaviour thus falls into line with the probable aetiology,
for we have seen that the tension rises more because of vascular congestion
*
1 p. 2218.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3291
and stasis than because of the embarrassment of filtration. In addition,
the hypotensive effect can frequently be augmented very effectively by the
use of hypertonic intravenous injections of sodium chloride (Duke-Elder,
1926), sorbitol (Bellows, Puntenney and Cohen, 1938) or acacia (Gifford,
1940). As a rule these measures are effective in bringing the tension to
normal, but if this happy result does not ensue, resort may be had to a
paracentesis which may be opened repeatedly on subsequent days. An
iridectomy is to be avoided on an acutely inflamed iris, a ruling which indeed
applies to all surgical procedures apart from a corneal paracentesis.
In sub-acute and chronic inflammations the problem is sometimes more
difficult and may call for more judgement. Some authorities would advise
atropine in all such cases also (Larsen, 1924); others prefer miotics (Malling,
1923; Hagen, 1925); while others vary their allegiance (Greeves, 1928;
Lawson, 1928; Wilder, 1930; and others). It is impossible to lay down
hard and fast laws in a condition which varies with almost every eye
affected and in the same eye from day to day, but as a general rule mydriatics
can safely be tried if the anterior chamber is deep, caution being exercised
if necessary by commencing their action first with homatropine, the effeet
of which is more easily controlled, or laevoglaucosan which has less tendency
than atropine to raise the tension. If the tension remains uncontrolled,
eserine may be employed for a few days and then mydriatics again tried, a
paracentesis may be performed as a temporary measure, or surgery of a more
permanent nature may be resorted to. If the anterior chamber is shallow
and the eye appears one pre-disposed to glaucoma, and particularly if the
inflammatory evidences are insignificant, the last alternative should be
adopted more readily provided atropinization proves unsuccessful.
The choice of operative measures of a more permanent nature than
paracentesis must depend to a considerable extent on the particular case and
the individual preference of the surgeon. The least traumatic and safest
procedure is an anterior flap sclerotomy, but it tends to become blocked
with inflammatory products and their organization, a criticism, indeed,
which may be applied to all fistulizing operations: this operation can,
however, be very easily repeated. An iridencleisis or iridotasis is unsuitable
since an inflamed or degenerated iris does not lend itself well to sub-
conjunctival incarceration. A cyclodialysis is contra-indicated since it
irritates still further an inflamed ciliary body and in congested cases the lacera-
tion of the insertion of the ciliary muscle may lead to haemorrhage. An
iridectomy is perhaps logical in chronic cases, but the end-results are
frequently not good. Perhaps a sclerectomy with a large (2 mm.) trephine
offers the best prospect of success; but frequently it too becomes eventually
occluded. There is no question, indeed, that the best hope of ultimate
success in the control of the tension lies in adequate control of the inflam-
matory process, from the elimination of the exciting cause, and thorough
and sustained treatment on medical lines.
3.292 TEXT-BOOK OF OPHTHALMOLOGY
(b) Post-INFLAMMATory glaucoma
A secondary rise of tension is by no means unusual as a result of the
pathological changes which may follow inflammation; these all act by
obstructing the circulation of the intra-ocular fluid either in the region of
the pupil or of the angle of the anterior chamber.
(i) In the region of the pupil adhesions between the iris and the capsule
of the lens by forming annular posterior synechiae may produce a seclusion
of the pupil. Such an occurrence stops the circulation of the intra-ocular
fluid, which is banked up under pressure behind the iris producing an iris
bombé and eventually secondary glaucoma. A more serious blockage may
Frº. 2811–Post-Iwº animatony Glauconia.
Peripheral anterior synechia in secondary glaucoma following irido-eyelitis
(x 55) (Parsons).
occur if total posterior synechiae bind the entire posterior surface of the iris
to the lens so that the circum-lental space is blocked with cyclitic exudate,
but in such cases the more common termination is hypotony and phthisis
of the globe owing to widespread destruction of the ciliary body by the
inflammatory process.
(ii) In the angle of the anterior chamber obstruction may be produced
by two mechanisms. The inflammatory swelling of the tissues at the root
of the iris and the anterior part of the ciliary body, aided frequently by the
forward bellying of the former tissue, makes this region of the uvea approach
the cornea so closely that the narrowed angle becomes bridged across by
strands of new-formed tissue which gradually consolidate to form peripheral
anterior synechiae which may eventually completely block the filtration angle

ANOMALIES OF THE INTRA-00ULAR PRESSURE 3293
(Fig. 2811). Again, obstruction to the drainage of aqueous may occur owing
to post-inflammatory changes in the tissues of the angle itself. Here the
spaces of Fontana are at first clogged with fibrin and inflammatory cells and
then by granulation tissue which may eventually consolidate into fibrous
tissue, so that ultimately, as pathological investigation has demonstrated
(Parsons, 1904; Greeves, 1928; and others), the fibres of the pectinate
ligament may become indistinguishable as separate entities but are
converted into a solid mass, sometimes associated with the formation of new
hyaline membranes lined by endothelium and with proliferation of the
endothelium forming the wall of the canal of Schlemm. In all these cases the
aetiology of the glaucoma is primarily mechanical, the tension rising because of
obstruction of the circulation, blockage of the channels of exit, and abolition
of the safety-valve mechanism of the canal of Schlemm which is essential
to cushion the vicissitudes of tension which constantly occur.
It is interesting that in cases of glaucoma secondary to inflammation, pathological
changes of this nature may be seen not only in the pupillary region and the angle of
the anterior chamber, but also at the optic nerve-head. Here the glaucomatous cup
Fig. 28.12.-SEcond ARY GLAucovia.
Showing cupping and optic atrophy in a man of 75 with glaucoma following a
hypopyon ulcer. Note the thickening and degeneration of the retina and the
organizing exudate filling the cup. The vaginal space is widely dilated (> 10)
(Parsons).
may be partially or completely filled with organizing exudate which may consolidate
to form loose connective tissue supplied by new vessels (Figs. 2812-13) and even
bone (Movetti, 1928). It may happen, indeed, that the subsequent contraction of

3.294 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2813–Secondary Glaucoma.
Cupping and atrophy. There are fibrous tissue and new vessels on the surface
of the disc and the cup is filled with loose connective tissue. From a case of annular
scleritis (x 28) (Parsons).
-
º
- -
-
º
-
-
º
-
- - - -
SQ --
Fig. 2814–RETINA IN GLAucoma Cup.
The retina dragged into the cup and folded, undergoing cystic denegeration;
in an inflammatory glaucoma (x 55) (Parsons).



ANOMALIES OF THE INTRA-OCULAR PRESSURE 3295
this tissue pulls upon the retina and drags it as a folded and degenerated mass into
the concavity of the excavation (Campherstein, 1903 : Parsons, 1904) (Fig. 2814).
The treatment of such a mechanical condition must be essentially
surgical—the opening up of the channels of circulation. The best treatment,
of course, is prophylactic and lies in the prevention of the formation of
organized adhesions by the establishment of opportune mydriasis from the
earliest stages of the inflammation, but once they have become consolidated
sufficiently to cause hypertension, it is rare for even the strongest available
mydriatics to be effective in breaking them down. In the case of an iris
bombé in a quiet eye, or in an inflamed eye when immediate relief is desired
with the minimum of operative interference, it may be sufficient to perform
a transfia:ion of the iris. This may be done by the kerato-iridic paracentesis
of Terson (1922), who introduced a knife into the anterior chamber opposite
the point of greatest elevation of the iris, which tissue is merely incised allowing
the post-iridic fluid to escape as the knife is withdrawn. Another method of
transfixion is Fuchs’ four-point iridotomy, wherein the knife is passed through
the cornea in the horizontal meridian, to pierce the iris, pass beneath it and
emerge from it near the pupillary aperture, and then to transfix the iris
again in a similar manner on the opposite side of the pupil before it finally
re-emerges through the cornea at the opposite point in the limbus : in this
way the iris is transfixed four times. If such an operation is successful, the
protrusion of the iris should collapse immediately, the tension should fall
and symptoms recede. Unfortunately, however, such relief may be only
temporary, since the apertures in the iris may soon become occluded again
by exudate if the inflammation is still active, or may fail altogether if, as
frequently happens, a cause of the raised tension remains in the presence of
peripheral anterior synechiae. In these circumstances, and when total
posterior synechiae are present, a basal iridectomy must be performed. In
cases of difficulty Curram’s (1920–31) peripheral iridotomy may be con-
sidered : therein a short, narrow knife-needle is introduced subconjunctivally
and transfixes the iris before it emerges and thus cuts a peripheral hole
in the iris underneath the scleral shelf. A very useful alternative is an
iridectomy ab eacterno (Elschnig, 1928), wherein, under a conjunctival flap,
a scleral incision 1 mm. behind and parallel to the limbus is scratched in
layers with a keratone until the root of the iris is reached. Sometimes,
however, in the presence of complete posterior synechiae and an atrophic
iris, an attempted iridectomy results in a splitting of the iris so that its
posterior ectodermal layer is left adhering to the capsule of the lens; indeed,
any operation upon a fragile and atrophic iris may be most unsatisfactory,
and when its tissues tear so that a satisfactory basal excision is impossible,
provided congestion is absent, a greater chance of success may lie in abandon-
ing the idea of an iridectomy and performing an iris-inclusion operation
instead. In those chronic cases wherein the iris itself remains free and the
T.O. —WOL. III. 4 H
3296 TEXT-BOOK OF OPHTHALMOLOGY
blockage lies in the angle of the anterior chamber alone, resort should be
had to a filtering operation such as corneo-scleral trephining.
Bellows, Puntenny and Cohen. A. of O., xx, Larsen. Acta O., i, 345, 1924.
1036, 1938. A. f. O., cv, 144, 1924.
Carvill. T. A. m. O. S., xxx, 71, 1932. Lawson. T. O. S., xcviii, 60, 1928.
Curran. A. of O., xlix, 131, 1920. Lehrfeld and Reber. A. of O., xviii, 712, 1937.
T. O. S., li, 520, 1931. Malling. Acta O., i, 97, 1923.
Duke-Elder. Brit. J. O., x, 1, 30, 1926. Moretti. Z. f. Aug., lxvi, 239, 1928.
P. R. S. (B), ciz, 19, 1931. Parsons. T. O. S., xxiv, 9.9, 1904.
Elschnig. K. M. Aug., lxxx, 382, 1928. Path. of the Eye, London, i, 304, 1904.
Gifford. A. of O., xxiii, 301, 1940. Redslob. Am. d’Oc., clxxii, l, 1935.
Greeves. T. O. S., xlviii, 45, 1928. Risley. Am. of O., xxiii, 437, 1914.
Hagen. A. f. O., czv, 251, 1925. Smith, Priestley. Glaucoma, London, 1891.
Rampherstein. K. M. Aug., xli (1), 25, Terson. An. d’Oc., clix, 441, 1922.
1903. Weingott. A. d’O., liii, 672, 1936.
Rraupa. A. f. Aug., cik, 416, 1935. Wilder. Am. J. O., xiii, 681, 1930.
2. GLAUCOMA SECONDARY TO TRAUMA
A rise of tension following trauma to the eye may occur shortly after
contusions whether they be uncomplicated or involve gross intra-ocular
lesions, or may develop some considerable time after a perforating injury,
either traumatic or operative, healing of which is complicated by the
incarceration or ingrowth of tissue. These may be differentiated as traumatic
and post-traumatic glaucoma.
(a) TRAUMATIC GLAUCOMA
The behaviour of the tension of the eye after a simple contusion is very
variable. In animal experiments there is usually a short initial period
(lasting some 30 minutes) of hypertony followed by a longer period of
hypotony (lasting some 3–7 days), a reaction which may be evident also in
the fellow eye even although it had not been injured (Magitot, 1920; Leplat,
1922–25; Schmidt and de Decker, 1930; Larsson, 1930). In man it is usually
impossible to take tonometer readings so constantly, but the evidence shows
that in general terms a somewhat similar reaction takes place. Considerable
irregularities of behaviour, however, occur, a period of low tension following
a period of high tension, or occasionally an attack of relatively acute
glaucoma supervening. The most constant feature is the easistence of a
period of instability of tension, which occurs even in young people with normal
eyes after a contusion which has caused no visible injury to the inner eye.
In a typical case the tension of the two eyes varies within considerable
limits for a number of days, periods of mild and transient hypertony alterna-
ting with periods of hypotony, and then it gradually returns to normal.
As a rule, however, this instability passes away without serious effects;
sometimes a considerable or even alarming hypotony results *; and
very occasionally a secondary glaucoma supervenes.
1 p. 3419.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3297
The incidence of this type of glaucoma is rare, for although its occurrence
was described at an early date (Priestley Smith, 1881), only about 100 cases
have been reported in the literature. They have been noted in every type
of person from 6 (Villard, 1905) to 75 (Agnello, 1931) years of age ; but they
all have this in common that the contusion is considerable, the eye having
been struck with considerable force as by a fist or a cricket ball. In the
majority of cases the hypertension comes on immediately or very shortly
after the contusion, usually within the first week ; only very rarely
is its onset delayed beyond this time. An analysis of 75 cases in the
literature in which this interval is recorded shows that in 32 the glaucoma
came on immediately, in 21 in from 3 to 5 days, in 6 in from 5 to 7 days, in
| DAYS 2 3 4. 5 6 7 8
FIG. 2815.-TRAUMATIC GLAUCOMA.
After a contusion. The continuous line represents the tension of the affected
eye ; the dotted line that of the sound eye. Treatment by eserine.
6 in the 2nd week, in 2 in the 3rd week, and in the remaining 7 at a longer
interval up to 7 weeks. In about 80% of cases, therefore, the tension rises
within the 1st week after the accident. The rise of tension is accompanied
by obvious symptoms, pain, an immobile pupil, and traumatic myopia,
associated frequently with nausea and vomiting ; it may gradually die down
spontaneously or after medical treatment, or persist until surgical relief is
obtained (Fig. 2815).
The cases which develop glaucoma, in contrast to those which
are characterized only by a mild degree of variable tension, are usually
complicated by intra-ocular lesions of some severity, although instances do
occur of an acute attack of hypertension without visible injury to the inner
eye. Intra-ocular haemorrhages are the most common lesion, occurring
either into the anterior chamber, confined to the retina and choroid, or

4 H 2
3.298 TEXT-BOOK OF OPHTHALMOLOGY
filling the vitreous cavity. Tears of the iris also occur, a dislocation of the
ciliary body, a rupture of the choroid or retina particularly at the ora,
Fig. 2816.-Traumatic GLAucoma.
- A ciliary artery showing partially disorganized wall. The surrounding selera
is infiltrated and contains both uveal and hematogenous pigment (Tillema, 4 of 90.
Fig. 2817. Thaumatic GLAU coma.
A vortex vein showing erythrocytes in the lumen and pigment granules round
the wall (Tillema, A. of 0.).
Fig. 2818. Traumatic GLAucoma.
A ciliary nerve. On the right are well-stained myelin sheaths; but in the large
bundle on the left these are missing (Tillema, A. of 0.).
or a hole at the macula. A sub-luxation or dislocation of the lens is a
frequent complication which tends to carry with it a bad prognosis.
Pathological examinations of such eyes have been few: Garnye, 1891;
Genet, 1921 ; Morax, 1921; Tillema, 1937.



ANOMALIES OF THE INTRA-OCULAR PRESSURE 3299
Garnye's case showed generalized Oedema of the iris, ciliary body and choroid,
with tearing of the ciliary muscle and thrombosis of the ciliary veins : in the sclera
the perivascular and perineural channels were infiltrated, but the angle of the anterior
chamber was unaffected and open, and the lens was in normal position. Morax’s case
showed general haemorrhagic Oedema and necrosis of the uveal tract and retina, while
the lens had suffered an anterior sub-luxation. In Genét's case there was a haemorrhage
in the anterior chamber, a detached retina with a large tear into which the lens had
become dislocated. Tillema reported two cases. In the first, the sclera showed two
ruptures of its inner layers—a typical one originating in Schlemm’s canal and an
atypical one near the equator. The Scleral vessels had been disorganized in places
and the nerves showed isolated patches of degeneration, while there was much necrosis
in the uveal tract. In the second case, there were, in addition, a rupture of the anterior
capsule of the lens, a prolapse of vitreous into the anterior chamber and a rupture of
the central vein at the optic disc. In both there were endothelial changes in the
vortex veins, and—a change probably of considerable significance—isolated patches
of degeneration in the ciliary nerves (Figs. 2816–18).
The aetiology of traumatic glaucoma has given rise to a considerable
amount of speculation. Lagrange (1922) concluded that such an entity did
not exist, but considered that hypertension only occurred in traumatized eyes
of persons who were already predisposed to glaucoma, a claim, however, which
is untenable when it is recalled that a traumatic glaucoma may occur in young
persons, and seems very unlikely in view of the regularity withºwhich
an instability of the tension occurs after contusions both in humans and
animal eyes. Granting the occurrence of the condition, a large number of
hypotheses have been put forward to explain its rationale—oedema of the
uvea due to blockage of the perivascular lymphatics with loose cells (Garnye,
1891); the increased protein content of the aqueous, caused by vaso-
dilatation owing to vascular paralysis, blocking the filtration angle (Sala, 1904;
Peters, 1904); sympathetic irritation causing hypersecretion (Fromaget,
1913); thrombosis of the ciliary vessels (Garnye, 1891); tearing of the
ciliary muscle (Stoewer, 1904–07); the presence of intra-ocular haemorrhages
(Morax, 1921); or the development of an orbital haematoma obstructing the
venous outflow from the eyes (Magitot, 1917–18). Finally, a dislocation of
the lens has repeatedly been blamed for the advent of tension (Fromaget,
1913; and many others). It is true that one or more of these complications
may be present, in which case the tendency to hypertension will undoubtedly
be aggravated ; many of them, indeed, may have an influence sufficient to
"convert a relatively innocuous instability of tension into an actual glaucoma.
But the facts that none of them is a constant feature and that hypotony
may occur with all of them, preclude them from being considered as
determining causes.
Thus an intra-ocular haemorrhage may obstruct the filtration channels and
accentuate a rise in tension ; but a traumatic glaucoma may occur in its absence,
and it is frequently associated with a normal or lowered tension. Similarly a sub-
luxated lens, although common, is by no means an invariable occurrence, nor does it

3300 TEXT-BOOK OF OPHTHALMOLOGY
necessarily and inevitably lead to a raised tension. Thus Hegner (1915) found only
18 cases of glaucoma in 48 traumatic dislocations ; a sub-luxation of the lens may be
accompanied by hypotony (Tillema, 1937); and even a dislocation into the anterior
chamber, an event usually accompanied by a rise in tension," need not necessarily be so
(Hegner, 1915).
It would seem reasonable to assume that the mechanism which pro-
duces the instability of tension in the ordinary case is the primary cause of
the rise of tension in the exceptional case, aided in all probability by those
traumatic lesions which we have just noted acting as exciting and intensifying
factors in the aetiology. This primary mechanism is undoubtedly an upset
of the local nervous control of the circulation, any disturbance of which is
generalized over the entire uveal tract by aa.on refleaves (Duke-Elder, 1931).”
Such a disturbance produces a vascular instability with dilatation of the
capillaries, engorgement of the circulation, stasis, and Oedema, a mechanism,
indeed, comparable to that which occurs in inflammatory glaucoma. We
have seen that in some cases neural damage may be so gross as to be seen
histologically (Tillema, 1937); and the behaviour both of the injured eye
and of its fellow in clinical or experimental traumata can only be explained
on this basis (Magitot, 1917–23; Favaloro, 1924; and others). It also
explains the lessening of the reaction on deep anaesthesia of the experimental
animal (Leplat, 1922–25). We may take it, therefore, that the variations
in pressure after a contusion are caused by local neuro-vascular reactions,
and that traumatic glaucoma is based on these reactions. Simple hypertony
or hypotony is the physiological response to the trauma, glaucoma the
pathological effect. In the former a healthy neuro-vascular system
re-adjusts itself; in the latter it cannot, partly because it itself is damaged,
and partly because the hypertensive reaction is accentuated by other patho-
logical lesions tending to accentuate it and make it permanent.
The prognosis of a traumatic glaucoma depends very largely on the
seriousness of the associated lesions. In uncomplicated cases the tension
usually returns to normal in the course of a few days; but when serious
intra-ocular lesions are present the outlook is more doubtful. Particularly
is this the case when a dislocation of the lens is present or a haemorrhage into
the vitreous has occurred. The majority of cases, however, terminate
favourably.
Treatment. In cases of simple hypertension without evidences of acute
glaucoma no special treatment is required, for with rest and protective
bandaging the tension returns to normal. In more serious cases characterized
by a rise of tension sufficient to produce symptoms, there has been some
controversy whether to use mydriatics or miotics. It is significant, however,
that those cases wherein atropine was given while the tension was high have
usually retained only reduced vision (Jacobson, 1884; Scheffels, 1890; Peters,
1904; Brand, 1905; Cordes and Horner, 1932; and others), while those
1 p. 3238. * Vol. I, p. 416.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3301
wherein eserine was substituted when the tension rose have frequently done
better (Myers, 1900; Sala, 1904; Cantonnet, 1912; Marx and Quartero,
1930; and others). A most useful drug is adrenalin (11,000) given as an
instillation, or better as a sub-conjunctival injection (Magitot, 1923). If
this is not effective a paracentesis may lower the pressure permanently
(Peters, 1904; Sala, 1904), a measure aided by the precedent administration
of adrenalin. In the more acute cases when drugs fail to control the condi-
tion, surgical treatment is frequently exceedingly difficult and disappointing ;
on the principle of its general value in acute glaucoma, a basal iridectomy
is probably the most logical procedure, and if it fails any other anti-glaucoma-
tous measure may be adopted (sclerotomy, sclerectomy, etc.); but it is not
unusual, especially in grossly injured eyes, for excision to become necessary
for the relief of pain.
(b) POST-TRAUMATIC GLAUCOMA
Post-traumatic glaucoma is usually due to a perforating injury in which
some of the intra-ocular tissues become incarcerated in the wound, the most
common example being a corneal perforation with anterior synechiae : a
similar result may follow a
perforated corneal ulcer. The
pathological picture may vary
from a simple incarceration of
the iris to the formation of an
anterior staphyloma, wherein the
cornea, the iris and the lens are
fused into one mass of cicatricial * *
tissue (Fig. 2819). In such cases at...","...",...,"...".
the rise of tension is undoubtedly obliteration of the drainage angle (Priestley
partially due to obliteration of *"
the filtration angle, which indeed may be entirely abolished, but a factor
which must also be considered is a disturbance of neuro-vascular reflexes
by the irritation of the iris and ciliary body partly by mechanical traction
and partly by inflammatory reaction (Fuchs, 1908). The treatment of such
a condition has already been discussed and is entirely surgical—a freeing
of the tied-up iris, an extensive iridectomy, or, at the least, an isolation of
the adherent part of the iris by an iridectomy on either side of it. Operations
undertaken purely for the relief of tension are rarely effective, and if the
glaucoma remains uncontrolled, enucleation is usually necessary. It fre-
quently happens, however, even if the tension is apparently controlled,
that such cases tend slowly and gradually to suffer a decline in vision.
POST-OPERATIVE GLAUCOMA forms a peculiar and important type of post-
traumatic hypertension ; it occurs sometimes after an iridectomy, but usually
after one of the various operations for cataract. Thus of 27 cases studied
1 Vol. II, p. 1825. * Vol. II, p. 1829.
FIG. 2819.-SECON DARY G LAU COMA.

3302 TEXT-BOOK OF OPHTHALMOLOGY
by Lehrfeld and Reber (1937), 3 followed iridectomy, I capsulotomy, I
discission, and 22 cataract extraction. There is a consensus of opinion
that in these cases the rise of tension is determined by mechanical obstruc-
tion at the filtration angle ; this is undoubtedly partially true, but the
factor of circulatory instability due to the excitation of neuro-vascular reflexes
caused by traction on the iris and ciliary body by incarcerated tissues aided
by inflammatory irritation must also be borne in mind. Not only does this
produce a vascular stasis and congestion, but it throws out of gear the
mechanism of formation and elimination of the intra-ocular fluid and
changes its composition.
After Iridectomy. A secondary glaucoma after iridectomy is not
particularly common, and when it does occur, apart from accidental wound-
ing of the lens, it is associated with conditions which embarrass the filtration
angle (Collins, 1888), a factor obviously most potent in eyes already affected
with glaucoma for the relief of which the operation had been undertaken.
Several circumstances may account for this—the base of the iris may not
have been resected so that a portion is left blocking up the filtration area, an
obstruction often rendered additionally effective by the stump becoming
entangled in the wound ; a prolapse or adhesion of the anterior part of the
ciliary processes may occur into the wound ; or the lens may become
anchored to the wound by exudate, particularly if re-formation of the
anterior chamber has been delayed.
After Cataract Eatraction. The occurrence of post-operative glaucoma
after the extraction or discission of a cataract is a complication recognized
since the time of Bowman (1865) and v. Graefe (1869), the very considerable
literature on which has been discussed at length by Natanson (1889), Dalén
(1901), Chance (1910), and Fox (1936). In eatra-capsular eactractions its
incidence has been said to vary from 0-6 to 3.0% : 9 in 1,405 cases (0.64%)
(Collins, 1905), about 2% of 529 cases (Da Gama Pinto, 1897), 6 out of 200
cases (A. Knapp, 1928), 14 out of 1,182 extractions (1.2%) (Fox, 1936). In
the majority of cases the tension rises during the first few days or weeks
after operation, in which period inflammatory reaction and the cicatricial
pulling on the iris and ciliary body undoubtedly excite neuro-vascular
reflexes which render circulatory conditions unstable. Although, however,
the glaucoma usually appears within the first month, hypertension may come
on several years after the extraction, when obstructive causes can be demon-
strated. The suspicion arises that in these delayed instances the glaucoma
may be primary and independent in nature, and not entirely dependent upon
but only precipitated by the results of the precedent extraction.
The cause of such a secondary glaucoma varies, but depends in the
main upon the following events :-
1. Incarceration of the iris in the wound is perhaps the commonest factor, a
circumstance which has been verified by many pathological examinations (Stölting,
1887; Collins, 1888–1914; Dalén, 1901; Fox, 1936; and others).
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3303
Fig. 28.20.-IN CARCERATIox of THE LEN's CAPsule IN CoRNEAL Wound.
After a cataract extraction. The external part of the wound has united, but
the internal part gapes and into it is incarcerated the lens capsule (Collins).
Fig. 2821.-INGRowth of SURFACE EPITHELIUM.
After a cataract extraction. The ingrowth lined the anterior chamber and caused
secondary glaucoma necessitating excision (Collins, T. O. S.).
2. Entanglement of the capsule of the lens in the wound is almost as common,
and is a prolific cause of raised tension (Natanson, 1889: Collins, 1888–1914 : A.
Knapp, 1910–28; Stölting, 1912; Gros, 1925; and others) (Fig. 2820).


3304 TEXT-BOOK OF OPHTHALMOLOGY
3. A prolapse of vitreous into the wound and a blocking of the angle of the
anterior chamber thereby (H. Knapp, 1894; Risley, 1910; Weil, 1920; Stieren, 1921 ;
Urbanek, 1924) (Fig. 2807).
4. A post-operative irido-cyclitis may determine the onset of an inflammatory
glaucoma, to which may be added the mechanical effect of a plastering of the iris upon
the capsule (Priestley Smith, 1891). An inflammatory condition of an irritative
nature may also be induced by slow cicatrizing traction on the ciliary body by capsular
or iridic remnants adherent in the wound (Dupuy-Dutemps, 1904).
5. An ingrowth of epithelium through the wound into the anterior chamber, the
whole of which may eventually be lined by the proliferating cells, occasionally produces
a most recalcitrant type of glaucoma (Meller, 1901; Elschnig, 1903; Collins, 1914;
Salus, 1927; Corrado, 1931; Levine, 1933; Vail, 1936; and others) + (Fig. 2821).
6. Massive quantities of soft lens matter in the anterior chamber may clog the
filtration angle and also excite a congestive inflammatory reaction (Fox, 1936).
7. Intra-ocular haemorrhage may act as a predisposing cause.
After a capsulotomy a secondary rise of tension is again not an infre-
Quent event, and is, indeed, about twice as frequent as after the operation
of extraction. Fortunately the condition is frequently controlled by miotics.
In some cases the hypertension arises primarily from the operation itself,
and in others is probably due to the original extraction, but is precipitated
by the second operation upon the after-cataract. This type of glaucoma
has already been discussed, when it was pointed out that three aetiological
factors might enter into the question :=
1. An irido-cyclitis aggravated by traction on the ciliary processes (v. Graefe,
1869). This is best obviated by early discission while the capsule is still soft, the use
of a sharp knife for cutting rather than a needle for tearing, and confining the manipula-
tions to the most delicate portions of the membrane and avoiding tough bands.
2. A blocking of the angle of the anterior chamber by prolapsed vitreous (Cheney,
I909).
3. Obstruction of the pupil by prolapsed vitreous producing a condition of iris
bombé with the formation of secondary anterior peripheral synechiae (Hudson, 1911),
(Fig. 2822).
Intra-capsular eactraction is undoubtedly less liable to lead to secondary
glaucoma than the extra-capsular operation and many surgeons with a
large practical experience have met with no case (Fox, 1936; Davis, 1938;
Gifford, 1940), but nevertheless this unfortunate complication may occur.
Thus Sinclair (1932) experienced 1 in 257 extractions, and A. Knapp
(1926–33) encountered 5 instances in 200 extractions, and again (1936)
4 in 500. It must be remembered that although the two great dangers of
an incarceration of the capsule in the wound and a second capsulotomy
operation are avoided, such complications as incarceration of the iris, loss
of vitreous, haemorrhage, and post-operative irido-cyclitis are liable to occur.
The discission of a soft cataract is frequently followed by a secondary
rise in tension, largely from the mechanical reason of a rapid swelling of the
1 Vol. II, p. 1817.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3305
lens with mechanical obliteration of the filtration angle, to the effects of
which may be added that of an irritative irido-cyclitis. As a rule such a
Secondary glaucoma is readily controlled by evacuation of the swollen lens.
The prognosis in such cases of post-operative glaucoma is on the whole
poor : sometimes the tension is very difficult to control, and even when this
is successfully accomplished, the ultimate visual results are often disappoint-
ing. The best treatment is prophylactic—the elimination of any factors
predisposing to post-operative inflammation, the performance of a simple
extraction with at most a small peripheral iridectomy, a clearing in so far
as is possible of the anterior chamber, the avoidance of unnecessary operative
trauma, and, above all, the most scrupulously careful toilet of the wound.
Once glaucoma has developed, medical measures may be tried. Since in
many cases the rise of tension is primarily mechanical, it may fall dramatically
On the cessation of atropine and the exhibition of eserine or other miotics: 1
Gifford (1932) recommended the intra-muscular injection of ergotamine
tartrate. Heat, leeches, and other local measures should also be tried and
may be successful, particularly in inflammatory cases. If the tension remains
uncontrolled, however, surgery should not be too long delayed if vision is
to be retained, for the prognosis depends largely on the promptness of the
relief of tension before structural anomalies become consolidated. The
choice of the most suitable surgical technique presents innumerable problems,
to which the literature provides innumerable answers, none of which can
claim to be a sovereign remedy and all of which too frequently fail. It is
occasionally possible to cut synechiae of the lens capsule with Lang's knives
(Dupuy-Dutemps, 1904; Collins, 1914), but more usually compensatory
surgical relief must be sought. If the lens was extracted without an
iridectomy, this may be done, for which H. Knapp (1892) employed a hook.
If an iridectomy has been done, an anterior sclerectomy may be tried, or a
corneo-scleral trephining : in the latter event the site of the iridectomy
should be avoided, since the trephine opening readily blocks with a vitreous
plug or becomes occluded in scar-tissue, so that it is better to locate the
trephine to the side of one or other pillar or at “ 6 o'clock.” In those cases
which are mechanical rather than inflammatory in type, cyclodialysis, which
is unaffected by the condition of aphakia and does not entail the presentation
of vitreous, is probably the most widely useful operation. It is especially
indicated in post-operative cases, when the operation should be done over a
large quadrant including the site of the previous operation, so that any
adherent pillars of the iris or tags of capsule are separated from the scar by
the spatula. Cases of epithelial ingrowth are quite untreatable unless
radiation with X-rays relieves the tension by preferential destruction of the
actively proliferating cells (Vail, 1936).
1 p. 3392.
3306 TEXT-BOOK OF OPHTHALMOLOGY
TRAU MATIC GLAUCOMA COMPLICATED BY A RETAINED FOREIGN BOIDY
The presence of a minute foreign body of a non-irritating type is some-
times responsible for the development of a delayed chronic glaucoma. As a
rule the rise of tension comes on insidiously with cupping of the disc,
frequently showing exacerbations, and it does not appear until several
months or even years have elapsed after the injury. For this reason
the nature of the glaucoma is often missed unless a sufficiently minute
clinical examination reveals evidence of a perforating wound and the foreign
body is then discovered radiologically. Since foreign bodies of all types,
either metallic or non-metallic, may cause such a reaction, even although
inert in nature, it would seem probable that the hypertension depends
upon neuro-vascular reactions excited by their presence ; the aetiology thus
corresponds to that of the glaucoma following dislocation of the lens. The
ideal treatment is to extract the foreign body, whereupon the tension
may return to normal ; occasionally, however, it remains high, in which
case, and also when the foreign body cannot be extracted, a fistulizing
operation (such as a trephining) may be tried. If this does not control the
condition and if signs of irritability are present, resort must be had to
enucleation (Morax, 1917–21 ; Greeves, 1937; and others).
Agnello. Lett. oft., viii, 520, 1931. Knapp, A. T. Am. O. S., xii, 472, 1910.
Bowman. R. L. O. H. Rep., iv, 365, 1865.
Brand. Cb. pr. Aug., xxix, 275, 1905.
Cantonnet. A. d’O., xxxii, 690, 1912.
Chance. Ophthalmology, vi, 565, 1910.
Cheney. Ophthalmoscopy, v, 27, 1909.
Collins. R. L. O. H. Rep., xii, 19, 1888;
xiii, 166, 1891 ; xvi, 247, 1905.
Researches, London, 1896.
T. O. S., xxxiv, 18, 1914.
Cordes and Horner. Am. J. O., xv, 942,
1932.
Corrado. Am... dº Ott., lix, 706, 1931.
Dalén. Mitt. a. d. Augenkl. Carol. med.-chir.
Instit., Stockholm, iii, 75, 1901.
Davis. A. of O., xix, 867, 1938.
Duke-Elder. P. R. S. (B), ciz, 19, 1931.
Dupuy-Dutemps. Am. d’Oc., exxxii, 93,
1904.
Elschnig. K. M. Aug., xli (1), 247, 1903.
Favaloro. Am. di Ott., lii, 547, 1924.
Fox. A. of O., xvi, 585, 1936.
Fromaget. An. d’Oc., cxlix, 1, 1913.
Fuchs. A. f. O., lxix., 254, 1908.
Garnye. Vrach, xii, 636, 1891.
Genét. Médicine, ii, 263, 1921.
Gifford. Handbook of Ocular Therapeutics,
Phila., 1932.
A. of O., xxiii, 301, 1940.
v. Graefe. A. f. O., xv (3), 108, 1869.
Greeves. Brit. Med. J., ii, 1107, 1937.
Gros. La Clin. Opht., xxix, 691, 1925.
Hegner. Beit. z. Aug., ix, 707, 1915.
Budson. R. L. O. H. Rep., xviii, 203, 1911.
Jacobson. A. f. O., xxx (4), 157. 1884.
A. of O., lv, 257, 1926; x, 6, 1933; xvi,
770, 1936.
J. A. m. Med. As., xci, 1794, 1928.
Knapp, H. A. of O., xxi, 293, 1892.
A. f. Aug., xxx, 1, 1894.
Lagrange. Glaucome et Hypotomie, Paris,
1922.
Larsson. Acta O., viii, 261, 1930.
Lehrfeld and Reber. A. of O., xviii, 716, 1937.
Leplat. C. R. S. Biol., lxxxvii, 982, 1922.
Am... d’Oc., clxi, 87, 1924; clzii, 81, 1925.
Levine. Am. J. O., xvi, 796, 1933.
Magitot. Am. d’Oc., cliv, 667, 1917; clv,
1, 66, 1918; clvii, 680, 1920; clx, l,
81, 1923.
Marx and Quartero. K. M. Aug., lxxxv, 758,
1930. -
Meller. A. f. O., lii (3), 436, 1901.
Morax. An. d’Oc., cliv, 11, 1917.
Glaucome et les glaucomateua, Paris, 1921.
Myers. A. of O., xxix, 150, 1900.
Natanson. Diss., Dorpat, 1889.
Peters. K. M. Aug., xlii (2), 545, 1904.
Pinto, Da Gama. Am. d’Oc., czvii, 22, 1897.
Risley. Ophthalmology, vi, 572, 1910.
Sala. K. M. Aug., xlii (1), 316, 1904.
Salus. K. M. Aug., lxxviii, 368, 1927.
Scheffels. A. f. Aug., xxii, 308, 1890.
Schmidt and de Decker. A. f. Aug., cii, 700,
1930.
Sinclair. T. O. S., lii, p. lvii, 1932.
Smith, Priestley. O. Rev., i, 273, 1881 ; ii,
257, 1883.
Glaucoma, London, 1879, 1891.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3307
Stieren. Am. J. O., iv, 424, 1921. Urbanek. Z. f. Aug., liv, 164, 1924.
Stoewer. K. M. Aug., xlii (1), 143, 1904; Vail. A. of O., xv, 270, 1936.
xlv. (1), 347, 1907. Villard. Am. d’Oc., czzxiii, 241, 1905;
Stölting. A. f. O., xxxiii (2), 177, 1887; cxxxvi, 458, 1906.
lxxxi, 518, 1912. Weil. A. d’O., xxx, 716, 1920.
Tillema. A. of O., xvii, 586, 1937.
3. GLAUCOMA SECONDARY TO CHANGES IN THE LEN's
(a) DEFORMITIES OF THE LENS
MICROPHAKIA (or SPHEROPHAKIA), a congenital and bilateral condition
wherein the lens is small and spherical," may be complicated by glaucoma, a
circumstance remarked by Bowman (1865) and studied by several subsequent
writers (Wessely, 1910; Fleischer, 1916; Urbanek, 1930; Gnad, 1931;
Shapira, 1934). In this case the hypertension is probably due to an obstruc-
tion of the flow of the intra-ocular fluid through the pupillary aperture,
which becomes blocked by the rounded bulging anterior surface of the lens,
which may, indeed, approach quite closely to the cornea. It is obvious,
therefore, that if the pupil is contracted the pupillary aperture will be more
firmly blocked, while under full mydriasis the entire circumference of the
lens becomes visible and a wide communication exists between the posterior
and anterior chambers. It follows that in this type of glaucoma a miotic
raises the tension and a mydriatic lowers it, an anomaly which has prompted
the name INVERSE GLAUCOMA (Urbanek, 1930).
Thus in Gnad’s (1931) case the tension was 36 mm. (Schiótz) with pilocarpine and
fell to 25 mm. with homatropine ; in Shapira’s (1934) without drugs and a 3 mm.
pupil the tension was 36 mm., with pilocarpine and a 1–2 mm. pupil an uncompensated
glaucoma developed with a tension of 51 mm., which fell with a pupillary dilatation
of 6 mm. under homatropine to 25–21 mm., and on full atropine mydriasis to 15 mm. Hg.
When the symptoms come on in early life a staphylomatous condition
may result, but more usually the onset is delayed until youth, when acute
attacks of hypertension are liable to occur. These can usually be controlled
with mydriatics, but the best permanent treatment has been found to be an
intra-capsular extraction of the lens, a procedure which usually gives good
results and incidentally relieves the very high lenticular myopia. A compli-
cation which may occur is luxation of the lens owing to the congenitally
weak zonule ; this accident may bring on an acute glaucomatous attack,
which again may be controlled by atropine prior to removal of the lens
(Bowman, 1865; Jacobs, 1937).
(b) DISPLACEMENTS OF THE LENs
A SUB-LUXATION OF THE LENs, whether it be congenital, traumatic,
or secondary to intra-ocular disease, is frequently, but by no means invariably,
followed by the development of secondary glaucoma. Hypertension is not
1 Vol. II, p. 1348.
3308 TEXT-BOOK OF OPHTHALMOLOGY
very commonly associated with a congenital sub-luxation, although the
occurrence has been known since the time of Bowman (1865), who recorded
a case which resulted in absolute glaucoma. In traumatic cases this compli-
eation is more common: thus Hegner (1915) recorded glaucoma in 18 out
of 22 traumatic sub-luxations collected from Jena and is out of 48 collected
from Breslau. In sub-luxations secondary to intra-ocular disease a rise of
tension is also common, but here the condition is usually over-shadowed
by more serious events.
A complete dislocation of THE LENs is more frequently followed by
the complication of raised tension. In Posterior dislocations into the
vitreous we have already seen that the end-result is usually a gradual loss
of vision owing to degenerative and glaucomatous processes, which, however,
Fig. 2822–Secondary Glaucoma Arres Dislocation or THE LENs.
Nºte the prolapse of vitreous into the anterior chamber and the peripheral
synechise abolishing completely the filtration angle (Hudson, R. L. O. H. Rep.).
may require a considerable number of years for their fruition (Burk, 1912;
Wagenhäuser, 1913). In anterior dislocations an immediate secondary
glaucoma is the usual result, whether it be spontaneous or traumatic.
thus in 15 cases of dislocation into the anterior chamber (7 traumatic and
7 spontaneous) Hegner (1915) found glaucoma in 14.
Ætiologically these cases have generally been accepted as being due
to mechanical obstruction of the filtration angle, either by pressure of the
lens on the ciliary body, by peripheral anterior synechiae formed as a result
of the irritation, or by the presence of the lens itself in the anterior chamber
(Priestley Smith, 1891; Hudson 1911) (Fig. 2822). This, however, cannot
be the whole explanation, for a sub-luxation of the lens may be associated
with hypotony (Tillema, 1937), and even a dislocation into the anterior
chamber may occur without complication (Hegner, 1915). There seems no
doubt that the underlying cause is a derangement and stasis of the circula-
tion excited by neuro-vascular reflexes set up by the mechanical irritation

ANOMALIES OF THE INTRA-OCULAR PRESSURE 3309
of the ciliary body and iris, the effect of which, of course, is augmented and
converted into an acute attack of hypertension, partly by obstruction of the
filtration angle and sometimes, when the lens occupies the pupillary region,
at the pupil. The effect of the latter is seen in the fact that, as in sphero-
phakia, pilocarpine tends to raise the tension and precipitate glaucoma,
while atropine, by opening up communication between the anterior and
posterior chambers, lowers it (INVERSE GLAUCOMA). It is also seen in those
cases wherein glaucoma occurs when the patient lies prone and subsides
when he lies upon his back (Beccaria, 1893; Sabata, 1931).
The treatment of such cases is not at all easy and the glaucoma frequently
takes an unfavourable course. It is to be remembered that in sub-luxations
miotics are to be avoided ; indeed, eserine may precipitate a glaucomatous
attack in an eye never before affected (Minor, 1881), while atropine may
relieve a crisis. A change in position or massage of the globe may also
relieve the symptoms (Bailliart, 1922; Saito, 1934). There is no doubt
that the only effective treatment is removal of the lens at the earliest
moment, by forceps if that is possible, or alternatively by a scoop, or by
transfixing and withdrawing it by a diathermy needle.
(c) INTUMESCENCE OF THE LENs
A rapid swelling of the lens quite frequently excites a secondary rise in
tension, a sequence first clearly realized by v. Graefe (1869). This may
occur in two conditions : (a) with a rapidly developing intumescent cataract
of the senile type (Ischreyt, 1909; Salus, 1910; Wright, 1910; Morax, 1922;
Gonzáles, 1922), and (b) in traumatic cataract, caused either by a perforating
injury or an operation of discission. It is generally accepted that the
sequence is one of pressure forwards of the anterior portion of the ciliary
body and the root of the iris and consequent mechanical obstruction of the
filtration angle (Priestley Smith, 1879; Ischreyt, 1909), a factor which, when
the capsule is ruptured, may be further intensified by the spaces of Fontana
becoming clogged with soft lens matter ; but here again mechanical pressure
upon the ciliary body probably excites neuro-vascular reflexes which
precipitate the reaction, while in those cases where the capsule is opened, the
irritative irido-cyclitis produced by the soft lens matter has a similar effect.
Drugs are usually of little value in this condition, and treatment should
consist of iridectomy followed by removal of the lens by extraction in the
case of intumescent cataract, or curette evacuation in traumatic cataract.
(d) IN MORGAGNIAN CATARACT
A Morgagnian cataract is frequently associated with the development
of a secondary glaucoma, which may be acute and stormy in its incidence,
or may develop slowly and insidiously so that all perception of light has been
lost before any damage to the eye apart from the cataract has been noted.
Occasionally this is caused by various degrees of dislocation of the shrunken
3310 TEXT-BOOK OF OPHTHALMOLOGY
hypermature cataract, a phenomenon almost always followed by severe
irritation and a serious secondary glaucoma (v. Szily, 1884; Natanson,
1891; Mitvalsky, 1892; Harms, 1905; and others). A similar effect may
possibly result from irritation of the ciliary body by the loose nucleus lying
within the capsule resting upon the ciliary process (A. Knapp, 1927),
a factor which would determine a reflex vascular disturbance throughout
the eye. A more interesting phenomenon, however, is the glaucoma which
accompanies spontaneous absorption of the cataract " (v. Reuss, 1900;
H. Gifford, 1900–27; Verrey, 1916; Verhoeff and Lemoine, 1922). It will
be remembered that this event is sometimes associated with a spontaneous
rupture of the capsule which has become thin and frail (v. Szily, 1884;
Rollet and Genêt, 1913; Gonzáles, 1919; Kaufman, 1933; H. C. Knapp,
1937). Such a rise in tension is probably due to neuro-vascular reflexes set
up by the irritative lenticular matter (Gifford, 1918–27), aided by a blockage
of the drainage channels by lenticular débris. Relief is usually obtained
by surgical removal of the lens and any lens matter in the anterior chamber.
(e) sRNILE EXFOLIATION OF THE LENS CAPSULE (GLAUCOMA CAPSULARE)
We have already discussed at length * the phenomenon of senile exfolia-
tion of the lens capsule and how it has been associated with the occurrence
of chronic glaucoma. It is true that glaucoma occurs in about 70% of patients
with exfoliation, and although some authors deny any association between
the two conditions (Malling, 1938; Butler, 1938), others consider that the
changes in the lens capsule are the essential cause of the rise in tension (Vogt,
1925–31 ; Busacca, 1927–29 ; Trantas, 1929; Rehsteiner, 1929 ; Sobhy
Bey, 1932; Hörven, 1936–37 ; Garrow, 1938; Irvine, 1940). The close
relationship between the two conditions and the high incidence of exfoliation
in glaucomatous subjects seem to indicate more than an incidental associa-
tion, and it is probable that a clogging of the filtration angle with débris
from the capsule acts as a predisposing cause in determining the development
of a glaucoma which might otherwise remain clinically latent were the
efficiency of the drainage angle unimpaired.
Bailliart. Bull. S. d’O. Paris, xxxv, 98, 1922. v. Graefe. A. f. O., xv (2), 153, 1869.
Beccaria. Am. di Ott., xxii, 115, 1893. Harms. K. M. Aug., xliii, 147, 1905.
Bowman. R. L. O. H. Rep., v., 1, 1865. Hegner. Beit. z. Aug., ix, 707, 1915.
Burk. A. f. O., lxxxiii, 114, 1912. Hörven. Acta O., xiv, 231, 1936.
Busacca. A. f. O., czix, 135, 1927. Brit. J. O., xxi., 625, 1937.
R. M. Aug., lxxxiii, 737, 1929. Hudson. R. L. O. H. Rep., xviii, 203, 1911.
Butler. T. O. S., lviii, 575, 1938. Ischreyt. A. f. Aug., lxii, 272, 1909.
Fleischer. A. f. Aug., lxxx, 248, 1916. Irvine. A. of O., xxiii, 135, 1940.
Garrow. Brit. J. O., xxii, 214, 1938. Jacobs. Am. J. O., xx, 1042, 1937.
Gifford, H. Am. J. O., xvii, 290, 1900; i, 83, Kaufman. A. of O., ix, 56, 1933.
1918. Knapp, A. A. of O., lvi, 124, 1927.
A. of O., lvi, 457, 1927. Rnapp, H. C. Am. J. O., xx, 820, 1937.
Gnad. K. M. Aug., lxxxvii, 33, 1931. Malling. Acta O., xvi, 43, 1938.
Gonzáles. Am. J. O., ii, 742, 1919. Minor. N. Y. Med. J., xxxiii, 194, 1881.
Rev. Cub. de Oft., iv., 100, 1922. Mitvalsky. Cb. pr. Aug., xvi, 289, 1892.
1 p. 3171. * p. 3106.
ANOMALIES OF THE INTRA-OCULAR PRESSURE
33ll
Morax. An d'Oc., clix, 185, 1922.
Natanson. K. M. Aug., xxix, 423, 1891.
Rehsteiner. K. M. Aug., lxxxii, 21, 1929.
v. Reuss. Ch. pr. Aug., xxiv, 33, 1900.
Rollet and Genét. Rev. gen. d’O., xxii, 1, 1913.
Sabata. Oft. Shorn., vi, 202, 1931.
Saito. Chuo. Ganka Iho., xxvi, 1934: Ref.
Zb. ges. O., xxxi, 642, 1934.
Salus. K. M. Aug., xlviii (2), 167, 1910.
Shapira. Am. J. O., xvii, 726, 1934.
Smith, Priestley. Glaucoma, London, 1879,
1891.
Sobhy Bey. Brit. J. O., xvi, 65, 1932.
Ch. pr. Aug., viii, 17, 1884.
Tillema. A. of 0., xvii, 586, 1937.
Trantas. A. d’O., xlvi. 482, 1929.
Urbanek. Z. f. Aug., lxxvii, 171, 1930.
Verhoeff and Lemoine. Am. J. O., v, 700,
1922.
Verrey. Am. J. O., xxxiii. 230, 1916.
Vogt. K. M. Aug., lxxv, 1, 1925;
736, 1931.
Wagenhäuser. Diss., Tubingen: Ref., K. M.
Aug., li (2), 619, 1913.
Wessely. A. f. Aug., lxv, 295, 1910.
Wright. O. Rec., xix, 303, 1910.
v. Szily.
lxxxvi,
4. GLAUCoMA SECONDARY to VASCULAR ANoMALIEs of THE RETINA
(a) IN VENOUs THROMBOSIs: HAEMORRHAGIC GLAUCoMA
The frequent occurrence of secondary glaucoma after thrombotic
obstruction of the central vein of the retina (but not of a tributary vein)
Fig. 28.23.-SEcoSDARY GLAucoma IN CENTRAL WEIN THRow Bosts.
View of the filtration angle, which is open. Note the fibrinous coagulum in the
anterior chamber and the dense infiltration of the tissues of the angle.
There is
also much peri-corneal congestion and infiltration (Inouye, R. L. O. H. Rep.).
has already been fully discussed." At the time of the thrombosis the tension
in the affected eye may be normal or lower than that of its fellow (Foster
- p. 2584.
T.O.-WOL. III.
4 I

3312 TEXT-BOOK OF OPHTHALMOLOGY
Moore, 1922; Wessely, 1935), but in some 10% to 25% of cases of complete
thrombosis, hypertension begins to become evident as a rule within 3 months
after the start of the illness. The resultant HAEMORRHAGIC GLAUCOMA is
usually severe and extremely painful : the anterior chamber is typically
of normal depth and frequently contains some blood, the pupil is small
and the iris shows new vessels on its surface, the vitreous is usually clouded
with red cells precluding any view of the fundus, all perception of light is
rapidly lost, and the associated pain is usually unbearable and intractable
and shows little tendency to diminish. Isolated instances have occurred
wherein the tension has been controlled by eserine (Mayou, 1918), but in
general, any attempt at operative interference merely makes matters more
acutely worse by inducing profuse and recurrent haemorrhages; one haemor-
rhagic glaucoma is thus superimposed on another, the resulting state being
appropriately termed MALIGNANT GLAUCOMA for which the only practical
method of treatment is enucleation or retro-bulbar alcohol injection. On
occasion, relief has been claimed from roentgen rays (Hess, 1920; Brunetti,
1923; Hensen and Schäfer, 1924; Weinstein, 1939) or radium (Deutsch, 1928).
The aetiology of these cases has given rise to much speculation. Patho-
logical examinations have shown that an intense inflammatory reaction
occurs in the anterior segment of the globe so that the iris and spaces of
Fontana are packed with inflammatory exudates and cells (Fig. 2823),
while later, highly vascularized membranes cover the iris and line the angle
of the anterior chamber (Verhoeff, 1907; Inouye, 1910; Coats, 1913;
Samuels, 1935; and others): it is the rupture of the neo-capillaries in these
membranes that is responsible for the constantly recurrent hyphaemata and
the disastrous haemorrhages which follow operative interference. This block-
age of the filtration angle is undoubtedly a determining cause, to which other
suggestions have been added—the effect of a highly albuminous aqueous
(Coats, 1904), a turgescence of the vitreous owing to haemorrhagic products
(Wood, 1932) or of acid tissue-metabolites (Weinstein, 1939), or a general
perivascular sclerosis (Fisher, 1925). There is no doubt that a chronic
state of stasis and congestion brought about by degenerative and toxic
products has much to do with the matter.
(b) IN CYANOSIS RETINAE
Severe cases of cyanosis of the retina may terminate disastrously in an uncon-
trollable secondary glaucoma (Goldzieher, 1904; Baquis, 1908; Ginsberg, 1928).
In these eyes marked visual deterioration occurs, and they are characterized patho-
logically by extensive endarteritis, multiple arterial thrombotic occlusions in the
arteries and veins, and secondary hamorrhages ; the pathology is thus similar to that
of haemorrhagic glaucoma.
(c) IN VASCULAR RETINOPATHIES
In retinopathies and degenerative conditions of the retina, particularly renal
and atherosclerotic retinopathy, a secondary glaucoma may occasionally develop ;
1 p. 2585.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3313
pathological examinations of such cases have been described by Pagenstecher (1871)
Hutchinson (1878), Schnabel (1879), Edmunds and Brailey (1880), Weeks (1889),
Harms (1905), Tschirkowsky (1908), Cords (1921), and others. The same complication
may occur also in exudative retinitis (Coats, 1912). In these cases the glaucoma may
resemble the haemorrhagic type associated with venous thrombosis. Wagenmann
(1892) considered the rise of tension due to the effect of irritative products of
degeneration causing inflammatory changes in the anterior segment and embarrassing
filtration, while the influence of the highly colloid aqueous was stressed by Stölting
(1897) and Vollaro (1898).
(d) IN PAPILLITIS
Glaucoma has also been noted after papillitis ; here again, it is probably
associated with obstruction of the retinal veins (Cords, 1921).
(e) A note may be inserted here regarding the extremely rare occurrence of
glaucoma after intensive radium therapy which has resulted in a general telangiectatic
condition of the skin and conjunctiva. Such a case was reported by Gifford (1940)
after prolonged treatment for a frontal osteosarcoma. The condition did not respond
to miotics and every operation was followed by intra-ocular haemorrhages with a
return of tension, which, however, finally fell, although the visual result was poor.
Baquis. A. f. O., lxviii (2), 177, 1908. Inouye. R. L. O. H. Rep., xviii, 24, 1910.
Brunetti. L’Actinoterapia, iii, 70, 1923. Mayou. Brit. J. O., ii, 521, 1918.
Coats. R. L. O. H. Rep., xvi, 62, 1904; 516, Moore, Foster. T. O. S., xlii, 115, 1922.
1906. Pagenstecher. A. f. O., xvii, 98, 1871.
T. O. S., xxiv, 161, 1904. Römer. A. f. O., xlii, 514, 1896.
A. f. O., lxxxi, 275, 1912; lxxxvi, 341, Samuels. A. of O., xiii, 404, 1935.
1913. Schnabel. A. of O., vii, 12, 1879.
Cords. A. f. O., cv, 916, 1921. Stölting. A. f. O., xliii, 306, 1897.
Deutsch. Z. f. Aug., lxiv, 156, 1928.
Edmunds and Brailey. R. L. O. H. Rep., x,
132, 1880.
Fisher. T. O. S., xlv., 288, 1925.
Gifford. A. of O., xxiii, 301, 1940.
Ginsberg. K. M. Aug., lxxxi, 591, 1928.
Goldzieher. Cb. pr. Aug., xxviii, 257, 1904.
Harms. K. M. Aug., xlv., 143, 1905.
Hensen and Schäfer. A. f. O., cziv, 123, 1924.
Tschirkowsky. K. M. Aug., xlvi (2), 272,
1908.
Verhoeff. A. of O., xxxvi, 1, 1907.
Vollaro. Lav. de clin. ocul. Napoli, iii (3), 302,
1898.
Wagenmann. A. f. O., xxxviii (3), 213, 1892.
Weeks. A. f. Awg., xxi, 54, 1889.
Wehrli. A. f. Aug., xxxvii, 173, 1898.
Weinstein. Brit. J. O., xxiii, 392, 1939.
Hess. A. f. Aug., lxxxvi, 89, 1920. Wessely. K. M. Aug., xcv, 398, 1935.
Hutchinson. Med. Times dº Gazette, i, 104, Wood. Brit. J. O., xvi, 423, 1932.
1878.
5. GLAUCOMA SECONDARY TO INTRA-OCULAR TUMoURS
It is well known that although intra-ocular neoplasms may frequently
be associated with a secondary glaucoma, the sequence is by no means
inevitable, and that the development of glaucoma depends little upon the
size of the tumour or the degree of exudation as evinced, for example, by
the presence of a retinal detachment : even with very large tumours the
tension may remain normal or low (Heymans-May, 1921). It was suggested
by Priestley Smith (1879) that anatomical displacement forwards of the
lens and iris diaphragm accounted for the rise of tension, a view supported
by Fuchs (1917) and Nakayama (1927), but it has now been established
that the factors responsible are not those of size and volume, but rather
4 I 2
3314 TEXT-BOOK OF OPHTHALMOLOGY
those of site. In the early stages of the development of a tumour the
tension is usually normal or sub-normal (Dunnington, 1938), and the onset
of raised tension usually varies in the anterior and posterior segments of the
globe (Koliopoulos, 1929). Those which are so situated that the angle of
the anterior chamber is infiltrated at an early stage or which obstruct the
venous return from the eye by encroaching upon the ciliary body or vortex
veins, bring about a relatively early rise of tension (Birnbacher, 1905;
Parsons, 1908). This applies particularly to malignant tumours, in which
case obstruction may be produced by neoplastic extensions and by accumu-
lations of neoplastic cells and pigmentary deposits as well as by mechanical
means. Accompanying such vascular obstruction, secondary changes
Fig. 2824.-GLAucovia Secondary to INTRA-ocular TUMoUR : PERIvascular
INFILTRATION (Evans, Brit. J. O.).
develop among which the most obvious is the presence of perivascular
infiltration by round cells which Evans (1939) claimed to be pathognomonic
of this type of glaucoma (Fig. 2824). The fact that obstruction of the
venous return is of such primary aetiological importance explains the
occurrence of transitory attacks of raised tension in the case of steadily
growing tumours. This type of glaucoma is therefore primarily congestive
and is due to venous stasis more than to any other single factor. In the
later stages, when irritative products of necrosis are liberated and inflam-
matory symptoms appear, the element of vascular stasis is intensified by
inflammatory congestion (Kerschbaumer, 1900; Luedde, 1906; Reis, 1911;
and others). In all such cases, of course, the only adequate treatment is
excision of the globe, a method of treatment usually advisable to adopt in
all cases of blind painful eyes with raised tension in view of the likelihood
of their containing a neoplasm.

ANOMALIES OF THE INTRA-OCULAR PRESSURE 3315
Birnbacher. Fest. Julius Hirschberg, 89, 1905. Luedde. A. f. O., lxiii, 468, 1906.
Dunnington. A. of O., xx, 359, 1938. Nakayama. A. f. O., czviii, 311, 1927.
Evans. Brit. J. O., xxiii, 745, 1939. Parsons. Pathology of the Eye, London, iii,
Fuchs. A. f. O., xciv, 43, 1917. 1086, 1908.
Heymans-May. A. d’O., xxxviii, 479, 1921. Reis. A. f. O., lxxx, 69, 1911.
Rerschbaumer. Das Sarcom d. Auges, Smith, Priestley. Glaucoma, London, 1879.
Wiesbaden, 1900.
Koliopoulos. Am. d’Oc., clxvi, 206, 1929.
6. GLAUCOMA SECONDARY TO DETACHMENT OF THE RETINA
A secondary glaucoma occurring in a simple detachment of the retina
is rare, but it occurs in a small proportion of cases in which the detachment is
of some duration. Thus Nordenson (1887) found no rise of tension in 62 recent
cases but in 4 of 58 old cases ; Kümmell (1921) found a raised tension in
2 out of 52 cases, and Thomas (1925) in 1 out of 247. While the complication
thus occurs typically in detachments of some standing, particularly in old
persons, exceptions do occur : thus Morax (1921) noted it in a youth one
month after the occurrence of the detachment, and Magitot and Hallard
(1931) in a myope of 28 years. Such a rise of tension is usually associated
with degenerative or inflammatory lesions accompanying the detachment,
such as recurrent haemorrhages (Bergmeister, 1919 ; Stanka, 1923), sub-
retinal exudates (Raeder, 1925), a low-grade uveitis (Fuchs, 1920;
Halbertsma, 1924; Wilder, 1931), or a plastic irido-cyclitis of an irritative
type (Morax, 1920; Maggiore, 1923); they are thus primarily vascular in
origin although some exceptions exist wherein the glaucoma is mild and is
controlled by a decompression operation (Wilder, 1931). Unfortunately
such a secondary glaucoma is frequently more than usually painful, a
circumstance which makes enucleation of the eye the only practicable
treatment since such an eye is visually useless and does not respond to
decompression operations, while the difficulty of eliminating with certainty
the presence of a neoplasm is great.
Bergmeister. Z. f. Aug., xlii, 254, 1919. Morax. A. d’O., xxxvii, 374, 1920.
Fuchs. A. f. O., ci, 265, 1920. Glaucome et les Glaucomateur, Paris, 1921.
Halbertsma. K. M. Aug., lxxiii, 252, Nordenson. Die Netzhautablösung, Wies-
1924. baden, 1887.
Kümmell. K. M. Aug., xvii, 180, 1921. Raeder. K. M. Aug., lxxiv, 424, 1925.
Maggiore. Am. di Ott., li, 1, 1923. Stanka. K. M. Aug., lxx, 707, 1923.
Magitot and Hallard. Bull. S. d’O. Paris, xliii, Thomas. Z. f. Aug., liv, 333, 1925.
98, 1931. Wilder. A. of O., v, 55, 1931.
7. GLAUCOMA SECONDARY TO INTRA-OCULAR HAEMORRHAGE
Intra-ocular haemorrhage of an arterial mature, if at all profuse, is always
followed by an extremely acute rise of tension wherein the intra-ocular
pressure is suddenly raised to approximate the level of the blood-pressure
in the arteries. We have already seen that the most typical instance of this
distressing accident is in the case of an expulsive haemorrhage after an
33 16 TEXT-BOOK OF OPHTHALMOLOGY
intra-ocular operation, that the haemorrhage is usually sub-choroidal from
one of the posterior ciliary arteries, and that the only treatment usually
effective in relieving the excruciating pain is excision of the globe.” CAPILLARY
AND VENOUS HAEMORRHAGEs are not usually followed by glaucoma unless
they are recurrent, in which case, although it is frequently said to be caused
by a mechanical obstruction of the filtration channels by blood cells, the rise
of tension has probably a vascular basis depending upon the irritative
effects of the continued absorption of blood-products.
8. GLAUCOMA SECONDARY TO ATROPHIC AND SCLEROTIC ConDITIONS
(a) ESSENTIAL ATROPHY OF THE IRIs
When discussing essential atrophy of the iris,” we pointed out that
usually, but not invariably, the condition eventually resulted in absolute
glaucoma and blindness. It is probable that the deformity is caused by
traction and shrinkage of new-formed tissue at the angle of the anterior
chamber which eventually becomes completely obliterated, the root of the
iris being fused with the cornea (Bentzen and Leber, 1895; Wood, 1910;
Feingold, 1918; Licsko, 1923 ; Rochat and Mulder, 1924; Ellett, 1928).
To this the rise in tension is probably due, as well as to the elimination of
the dialysing surface of the iris. As a rule the glaucoma progresses to an
absolute condition despite any treatment, although occasionally the tension
has been controlled by fistulizing operations (sclerectomy, Waite, 1928;
cyclodialysis, Grosz, 1936).
(b) In Sclerotic and atrophic conditions of the posterior segment, a secondary
glaucoma is more unusual. It has been observed in PRIMARY SCLEROSIS OF THE CHOROID
when a branch of a vortex vein has been involved (Thiel, 1931). It has also been noted
in PIGMENTARY DEGENERATION OF THE RETINA, a disease in which it will be remembered
choroidal sclerosis is frequently an important feature (Galezowski, 1862; Schnabel,
1878; v. Hippel, 1901; Schmidthäuser, 1904).
Bentzen and Leber. A. f. O., xli (3), 229, 1895. Rochat and Mulder. Brit. J. O., viii, 362,
Ellett. T. Am. O. S., xxvi, 306, 1928. 1924.
Feingold. Am. J. O., i, 1, 1918. Schmidthäuser. Diss., Tübingen, 1904.
Galezowski. Am. d’Oc., xlviii, 269, 1862. Schnabel. A. of O., vii, 12, 1878.
Grosz. A. f. Aug., cz, 111, 1936. Thiel. Kurzes Hb. d. O., Berlin, iv, 820, 1931.
v. Hippel. A. f. O., lii, 498, 1901. Waite. Am. J. O., xi, 187, 1928.
Licsko. K. M. Aug., lxxi, 456, 1923. Wood. Ophthalmoscope, viii, 858, 1910.
9. GLAUCOMA SEconDARY TO VENOUS OBSTRUCTION IN THE ORBIT
Any condition which causes an obstruction of the venous drainage from the eye
is liable to set up an attack of secondary glaucoma. This may arise from a TENONITIS
(Seefelder, 1924; Larsson, 1926) or an ORBITAL HAEMATOMA (Magitot, 1918; Magitot
and Tillé, 1932), INFLAMMATION (Guist, 1925), ABSCEss (Magitot, 1918), TUMoUR, or a
PULSATING ExopBTHALMos determined by arterio-venous aneurysm (Elschnig, 1916;
Morax, 1921 ; Poulard and Bailliart, 1921 ; Hudelo, 1928). In these cases the
1 p. 21.13. * p. 2404.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3317
glaucoma depends upon passive congestion, and if the obstruction is sufficiently .
complete the tension of the eye may thereby ultimately be raised to approximate that
of the arterial pressure (95 mm. Schiótz, Magitot and Tillé after a retro-bulbar injection
for anaesthesia). In other cases an endophlebitis or thrombosis in the retinal and
choroidal vessels adds to the circulatory disturbance, while further embarrassment
may be caused by a chronic round-celled infiltration, an event which occurs not only
in inflammatory conditions but also in the absence of inflammation, as, for example,
in association with an arterio-venous aneurysm (Kraupa, 1911 ; Salus, 1918; Gazepis,
1922; Jaensch, 1924–25).
Elschnig. A. f. O., xcii, 101, 1916. Magitot and Tillé. Bull. S. d’O. Paris, xliv,
Gazepis. A. f. O., cz, 375, 1922. 170, 1932.
Guist. Z. f. Aug., lv, 308, 1925. Morax. Glaucome et les Glaucomateu.r, Paris,
Hudelo. Thése, Paris, 1928. 1921.
Jaensch. K. M. Aug., lxxiii, 251, 1924; Poulard and Bailliart. Bull. S. d’O. Paris,
lxxiv, 23 l ; 13 xv, 369, 1925. xxiii. 21, 1921.
Kraupa. K. M. Aug., xlix (2), 191, 1911. Salus. K. M. Aug., lx, 254, 1918.
Larsson. Acta O., iii, 207, 1926. Seefelder. Wien. med. W., lxxiv, 2053, 1924.
Magitot. An. d’Oc., clv, 1, 1918.
10. GLAUCOMA SECONDARY TO EPIDEMIC DROPSY
Epidemic dropsy is a disease commonly seen in India which affects rice
eaters in proportion as this cereal constitutes an important element of their
diet; it is characterized by general Oedema, hypertrophy and dilatation of
the heart, and, rarely, peripheral neuritis. It is a toxic condition caused
by eating highly polished, par-boiled rice which has been stored in damp
heat ; the toxin, which resembles histamine in its physiological reactions
(Chopra and De, 1937), is probably partially manufactured by the diseased
rice and partially elaborated after ingestion in the alimentary canal (Acton
and Chopra, 1925–27). The most interesting pathological feature is a genera-
lized teliangiectatic dilatation of the capillaries generally, without inflamma-
tory reaction or great tendency to haemorrhages, a phenomenon seen through-
out the body and in the blood-vessels of the entire uveal tract even in
cases which have shown no clinical signs and symptoms of glaucoma before
death.
Glaucoma was first noted in association with epidemic dropsy by
Maynard (1909) who reported a series of 100 cases in an epidemic in 1908–09 ;
Mukerjee (1927) observed another series of 253 cases in Bengal, and
Kirwan (1934) 325 cases between 1929 and 1933. The glaucoma may
occur in youth, from 8 years of age upwards, and is most common between
the ages of 20 and 35. It is bilateral, chronic and non-congestive in
type, for the eye remains white even when the tension is extremely high ;
it is rarely accompanied by pain, and the two essential symptoms are halos
due to corneal oedema and gradually fading vision. The anterior chamber
is deep and the pupil usually normal, while cupping and atrophy of the
disc are late in appearance. The visual fields show the defects typical of
chronic primary glaucoma, but the most outstanding feature is the height
33.18 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2825.-Glaucoma in EPIDEMio Dropsy.
The ciliary body, showing vascular dilatation and cedema of the tissues without
abnormality of the epithelium (Kirwan, Brit. J. O.).
Fig. 2826–Glaucoma in Epidemic Dropsy.
showing enormous vaso-dilatation in the choroid (Kirwan, Brit. J. O.).


ANOMALIES OF THE INTRA-OOULAR PRESSURE 3319
of the tension, which is rarely below 50 mm. Schiótz and may readily reach
100.
Pathologically an enormous dilatation of the small vessels of the uveal
tract is a constant feature (Figs. 2825–26); but there is no evidence of any
inflammatory process and the filtration angle is widely open and shows no
abnormality (Fig. 2827) (Kirwan, 1936). The aqueous humour shows a
marked increase of albumen, a raised osmotic pressure due to its presence
(Kirwan and Mukerjee, 1938), and contains a histamine-like substance in
considerable quantity.
It would seem, therefore, that in its aetiology this type of glaucoma
Fig. 2827. GLAU covia IN EPIDEMIC DRoPsy.
The filtration angle showing complete absence of abnormality (Kirwan, Brit.
J. O.).
corresponds in every way to the generalized oedema of the tissues throughout
the body. It has a vascular basis without any evidence of retention of
fluid at the drainage exits. The essential feature is a capillary dilatation and
increased permeability throughout the uveal tract due to the action of the
histamine-like substance which is responsible for the general condition;
this vascular disturbance raises the dialysing pressure-equilibrium to a
higher level and at the same time, by raising the osmotic pressure of the
aqueous, makes the intra-ocular pressure approximate still more closely to
the blood pressure.
In treatment the most urgent method of approach is to treat the general
condition by eliminating rice completely from the diet, and washing out

3320 TEXT-BOOK OF OPHTHALMOLOGY
the alimentary tract by purgation and the liberal administration of fluid.
If the visual fields are relatively unimpaired and the optic nerves are
healthy, local treatment to the eye may be delayed until an amelioration in
the general condition reduces the ocular tension, but in the event of this not
occurring rapidly the tension must be relieved. It is interesting that medical
treatment of any kind seems to be valueless in this type of glaucoma–neither
miotics, adrenalin nor hypertonic intra-venous injections. Of the surgical
measures a trephining operation acts with immediate and great efficiency in
controlling the tension, whereafter subsequent deterioration, either in the
vision or the fields, does not occur.
Acton and Chopra. Indian Med. Gaz., lx, l, Kirwan and Mukerjee. Brit. J. O., xxii, 329,
1925; likii, 359, 1927. 1938.
Chopra and De Indian J. Med. Res., xxv, Maynard. Indian Med. Gaz., xliv, 373, 1909.
101, 1937. Mukerjee. Proc. Far Eastern Cong., vii. 272,
Kirwan. A. of 0., xii. 1, 1934. 1927.
Brit. J. O., xx, 321, 1936.
11. GLAucoma Second ARY TO CongºNITAL ANoMALIEs : BUPHTHALMos
(a) UN.com PLICATED BUPHTHALMos
The clinical occurrence of buphthalmos has already been discussed,"
when it was pointed out that pathological studies had made it clear that
structural abnormalities in the region of the angle of the anterior chamber
Fig. 2828. BUPHTHALMos.
Illustrating the deep anterior chamber and the anomalous angle (Lister).
* Vol. II, p. 1292.

ANOMALIES OF THE INTRA-00ULAR PRESSURE 3321
offered an obstruction to the drainage-mechanism of the intra-ocular fluids
so that the pressure of the eye was raised, and since the coats of the eye at
this stage were plastic, the whole globe became enlarged so that the typical
Fig. 2829.-ANoMALouis FILTRATION ANGLE IN BUPHTHALMos (Parsons).
picture of buphthalmos resulted (Fig. 2828). The usual deformation is an
anomaly of the mesoderm at the corneo-scleral junction resulting in a per-
Fig. 2830.-GLAU coma IN ANIRIDIA.
The angle is blocked by a rudimentary iris. The ciliary body has become
detached from the sclera and is pointing downwards as a result of manipulation
of the specimen (Collins).
sistence of the pre-natal or pre-human condition wherein the trabeculae are
not opened up (Horner, 1880; Collins, 1896–99; Reis, 1905–11; Seefelder,
1906–20; Parsons, 1908) (Fig. 2829). Filtration may be further embarrassed
by an absence of the canal of Schlemm and its venous radicles (Cross, 1896;


3322 TEXT-BOOK OF OPHTHALMOLOGY
Gross, 1897; Reis, 1905), or an incomplete separation or persistent
adherence of the iris and the cornea (Collins, 1899; Mayou, 1910; Böhm,
1913–14; Schläflºe, 1914; Meisner, 1923; Lagrange, 1925; Theobald and
Clapp, 1923). In other cases a pre-natal inflammation may have produced
the same defect, leading secondarily to obliteration of the drainage
channels (Raab, 1876; Grahamer, 1884; Gallenga, 1885; Byers, 1931 ;
and others).
In ANIRIDIA a secondary glaucoma develops in a considerable proportion
of cases (Brailey, 1890; de Schweinitz, 1891; Collins, 1893; Holloway,
1913; Augstein, 1914; Böhm, 1915; Meller, 1917; Hudson, 1921 ;
Kubik, 1924). In these cases also the pectinate ligament is found to be
faulty, and the stump of the iris is often adherent to the sclera round a
considerable part of the circumference (Fig. 2830). Although part of the
angle is therefore open, sufficient is closed to render filtration inefficient to
cope successfully with intercurrent factors which may embarrass filtration
or lead to complete obliteration.
(b) COMPLICATED BY WIDESPREAD VASCULAR OR NIEURAL ANOMALIES
(1) ANGIOMATA. An association between glaucoma and angiomata,
particularly a capillary (“port-wine ‘’) navus of the face (Naevus flammeus)
was first observed by Schirmer (1860), and since then some 80 cases have
appeared in the literature. The majority of these have been summarized
by Krause (1929), Ballantyne (1930) and O’Brien and Porter (1933). The
association is not invariable, but is sufficiently common to be remarkable and
to suggest a relationship of cause and effect. It occurs when the facial
naevus involves the lids and usually some of the ocular structures, and in
the vast majority of cases is unilateral : bilateral cases are rare and occur
usually when the naevus is widespread, affecting also the pharynx and
nasal mucosa and sometimes the limbs and trunk (Beltman, 1904; Safar,
1923; Knapp, 1928).
The glaucoma, like the angiomatous condition, is usually congenital
and is buphthalmic in type in about 70% of the cases; but, on the other
hand, it may take on the form of a simple chronic glaucoma without
buphthalmic deformation of the globe (Beltman, 1904; Duschnitz, 1923;
Safar, 1923; Bär, 1925), and the onset of tension may be delayed until late
in life (Salus, 1923; Zuereva, 1927; Yamanaka, 1927; Pincus, 1939). It
is chronic and non-inflammatory in its clinical features, associated with a
characteristically deeply cupped and atrophic disc, inducing typical field
defects and frequently progressing to complete blindness. This result,
however, is not by any means invariable, and the visual acuity and fields
may remain normal for an indefinite period even although the tension is
raised (Vögele, 1928), while in other cases the condition appears to become
arrested (O'Brien and Porter, 1933). The tension, which is usually mode-
rately high, may, however, remain within normal limits, and since this may
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3323
occur in the presence of deep cupping of the disc and diminution of vision
(Löwenstein, 1923; Bär, 1925; McRae, 1929 ; O'Brien and Porter, 1933;
Evans and Evans, 1939), the possibility arises that in these cases the optic
atrophy progresses in the absence of hypertension or after its arrest. Other
changes which may occur in the eye include a dilatation of the vessels of
the conjunctiva, sclera and iris, heterochromia, hyperplasia of the iris, and
very frequently a choroidal angioma and tortuous and dilated retinal
vessels.
Pathological examinations have been reported in 15 eyes (Milles, 1884;
Lawford, 1885; Snell, 1886; Wagenmann, 1900; Stoewer, 1908;
Quackenboss and Verhoeff, 1908; Love, 1914; Safar, 1923; Knapp, 1928;
Clausen, 1928; de Haas, 1928; Weber, 1929; Jahnke, 1931 ; Dunphy,
1935; Evans and Evans, 1939). In all cases the intra-ocular capillaries
have been dilated ; in 12 there has been an angioma of the choroid, and in
2 an angioma of the iris ; in 5 the angle of the anterior chamber was closed
by dense tissue or anterior synechiae, but in 2 this region showed no abnor-
malities, while in I the optic atrophy (without tension) was of the cavernous
type. It is noteworthy that an open filtration angle has also been noted
gonioscopically (Tyson, 1932).
An interesting feature of those cases is a co-existent angiomatous condition
of the meninges, an association suggested by Sturge (1879) and Horrocks (1883), and
confirmed post-mortem by Cushing (1906) and Cushing and Bailey (1928). Evidence
of intra-cranial angiomata or tortuous or calcified diplôic vessels has also been brought
forward on many occasions by X-ray examination (Brushfield and Wyatt, 1927;
Rötth, 1928; Aynsley, 1929; Weber, 1929; Hudelo, 1929; Jahnke, 1931 ; Tyson,
1932; O’Brien and Porter, 1933), or by the occurrence of a spastic hemiplegia
or epileptiform convulsions (Horrocks, 1883; Aynsley, 1929; Rötth, 1928; Weber,
1929). This association indicates an affection of the distribution of the Vth nerve,
and can be correlated with hypertrophies of the deeper structures of the face and eye,
including the facial bones (Nakamura, 1922; Kiranoff, 1925; and others).
Occasionally the distribution of the angiomatous condition is more widespread to
include, in addition to the territory of the trigeminal, that of the spinal nerves and
sacral plexus (Vita, 1927), or the arms and legs (McRae, 1929), as well as the meninges
and liver (Krause, 1929).
The cause of the glaucoma is disputed. It is undoubtedly true
that many cases are associated with blockage of the filtration angle, but
since this is not universally present, it cannot represent the whole story.
The venous and capillary dilatation, however, and the frequent presence
of uveal angiomata suggest an alternative hypothesis in a venous stasis
and a disorganization of the fluid-traffic of the eye, a circumstance suggested
by the findings that the entrance of fluorescein is more rapid into the affected
eye than its fellow (Tyson, 1932), that the aqueous (Mehney, 1937) and the
secondary aqueous formed after paracentesis has a higher protein content
(Imai, 1932), and that the tension of the affected eye in contrast to its fellow
3324 TEXT-BOOK OF OPHTHALMOLOGY
is unaffected osmotically by an intravenous injection of dextrose (Dunphy,
1935). As in so many other glaucomatous conditions it is probable that
both causes are effective, the one aiding and abetting the other.
(2) NEUROFIBROMATOSIS. Diffuse neurofibromatosis of the uveal tract
is frequently, although not invariably, associated with glaucoma, and as a
rule, since the condition is congenital, the eye is buphthalmic (Sachsalber,
1897; Snell and Collins, 1903 ; Verhoeff, 1903 ; Collins and Batten, 1905;
Sutherland and Mayou, 1907; Weinstein, 1909; Murakami, 1913; Wiener,
1925; Knight, 1925; Davis, 1939; and others). Occasionally, however,
glaucomatous symptoms do not come on until adult life (Meeker, 1936).
Histologically the uveal tract is found to be densely infiltrated with the
fibrous-like cells of Schwann,” the angle of the anterior chamber being
involved, while the iris may be ill-developed and adherent to the cornea
(Wheeler, 1937) (Fig. 2054). Such a condition undoubtedly predisposes to
secondary glaucoma, an event which may also be partly determined by the
pathological involvement of the nerves which control the local activity of
the circulation.
Treatment. The treatment of these cases of congenital glaucoma is full
of disappointments and frequently quite ineffectual. Some cases, it is
true, become arrested ; a few respond to treatment ; but absolute glaucoma
and ultimate blindness is the usual end-result. For purposes of treatment
these cases may be divided into 3 types, depending on the stage of evolution
of the disease : early cases without corneal opacities or tears in Descemet's
membrane or glaucomatous excavation of the disc ; established cases where
deformation of the eye is apparent and the raised tension has started to
cause structural damage ; and advanced cases wherein the eye is grossly
deformed and its function has been lost. In the first group conservative
treatment with miotics is indicated ; decompression operations should be
undertaken in the second ; while in the third removal of the eye is the usual
ultimate resort. In the second group an iridectomy is usually quite ineffec-
tive for the anatomical defect at the filtration angle is too gross; if the
tension is to be lowered a fistulizing operation is necessary. The choice
among the many possible procedures is difficult, and different authors claim
successes with different techniques. Repeated flap sclerotomies around the
limbus, a sclerecto-iridectomy, trephining, and repeated cyclodialysis have
each achieved their measure of successes and failures. In recalcitrant
cases a good plan is to combine trephining (using a large 2 mm.
trephine) with a cyclodialysis, a combination which can be repeated more
than once if necessary. It frequently happens, however, that even if the
tension is controlled, the visual result is poor, while at other times collapse
1 p. 2466.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3325
of the eye or a progressive degeneration of the cornea puts an end to initial
hopes. Moreover, accidents are prone to occur at the time of the operation,
such as dislocation of the lens, detachment of the retina, and particularly
in angiomatous cases, disastrous haemorrhages. Finally, it should be remem-
bered that these children occasionally die under an anaesthetic, a tendency
attributed by Magitot (1912) and Hardesty (1934) to hyperplasia of the
thymus. It may be said that a good functional result is exceptional, a
moderate result is the most that can be hoped for and that usually only
after several operations, while a too common end-result is blindness and
enucleation.
Augstein. K. M. Aug., liii, 405, 1914. Mayou. T. O. S., xxx, 120, 1910.
Aynsley. Brit. J. O., xiii, 612, 1929. Meeker. A. of O., xvi, 152, 1936.
Ballantyne. Brit. J. O., xiv, 481, 1930. Mehney. A. of O., xvii, 1018, 1937.
Bär. Z. f. Aug., lvii, 628, 1925. Meisner. A. f. O., czii, 433, 1923.
Beltman. A. f. O., lix, 502, 1904. Meller. A. f. O., xcii, 34, 1917.
Böhm. K. M. Aug., lii, 841, 1913; liii, 75, Milles. T. O. S., iv, 168, 1884.
1914 : Iv, 544, 1915. Murakami. K. M. Aug., li (1), 514, 1913.
Brailey. T. O. S., x, 139, 1890. Nakamura. K. M. Aug., lxix, 312, 1922.
Brushfield and Wyatt. Brit. J. Child. Dis., O’Brien and Porter. A. of O., ix, 715, 1933.
xxiv, 98, 209, 1927. Parsons. Path. of the Eye, London, iii, llll,
Byers. A. of O., vi, 578, 1931. 1908.
Clausen. K. M. Aug., lxxxi, 393, 1928. Pincus. A. of O., xxi, 741, 1939.
Collins. T. O. S., xiii, 128, 1893. Quackenboss and Verhoeff. T. Am. O. S., xi,
Researches, London, 1896.
IX Internat. Congr. Utrecht, 88, 1899.
Collins and Batten. T. O. S., xxv, 248, 1905.
Cross. T. O. S., xvi. 340, 1896.
Cushing. J. Am. Med. As., xlvii, 178, 1906.
Cushing and Bailey. Tumours arising from
the Blood-vessels of the Brain, Springfield,
Ill., 1928.
Davis. A. of O., xxii, 761, 1939.
Dunphy. Am. J. O., xviii, 709, 1935.
Duschnitz. K. M. Aug., lxx, 404, 1923.
Evans and Evans. Brit. J. O., xxiii, 95, 1939.
Gallenga. An. di Ott., xiv., 149, 1885.
Grahamer. A. f. O., xxx (3), 265, 1884.
Gross. These de Paris, 1897.
de Haas. Ned. tij. v. Gen., ii, 4326, 1928.
Hardesty. Am. J. O., xvii, 689, 1934.
Bolloway. O. Rec., xx, 540, 1913.
Horner. Kramkh. d. Auges, Tübingen, 1880.
Horrocks. T. O. S., iii, 106, 1883.
Hudelo. A m. d’Oc., clxvi, 889, 1929.
Hudson. T. O. S., xli., 274, 1921.
Imai. Acta S. O. Japan, xxxvi, l 14, 1932.
Jahnke. Z. f. Aug., lxxiv, 165, 1931.
Kiranoff. K. M. Aug., lxxiv, 502, 1925.
Enapp, A. A. of O., lvii, 219, 1928.
Rnight. Am. J. O., viii, 791, 1925.
Rrause. Z. f. Aug., lxviii, 244, 1929.
Rubik. K. M. Aug., lxxii, 686, 1924.
Lagrange. Bull. S. fr. d’O., xxxviii, 3, 1925.
Lawford. T. O. S., v., 136, 1885.
Love. A. of O., xliii, 607, 1914.
Löwenstein. K. M. Aug., lxx, 540, 1923.
McRae. Brit. J. O., xiii, 63, 1929.
Magitot. An. d’Oc., exlvii, 241, 1912.
510, 1908.
Raab. K. M. Aug., xiv, 22, 1876.
Reis. A. f. O., lx, 1, 1905.
B. O. G. Heidel., xxxvii, 348, 1911.
Rötth. K. M. Aug., lxxx, 405, 1928.
Sachsalber. Beit. z. Aug., xxvii, l, 1897.
Safar. Z. f. Aug., li, 301, 1923.
Salus. K. M. Aug., lxxi, 305, 1923.
Schirmer. A. f. O., vii (1), 119, 1860.
Schläflze. A. f. O., lxxxvi, 106, 1914.
de Schweinitz. T. Am. O. S., vi, 59, 1891.
Seefelder. A. f. O., lxiii, 205, 481, 1906;
ciii, 1, 1920.
Snell. Brit. Med. J., ii, 68, 1886.
Snell and Collins. T. O. S., xxiii, 157, 1903.
Stoewer. K. M. Aug., xlvi (2), 323, 1908.
Sturge. T. Clin. S., xii, 162, 1879.
Sutherland and Mayou. T. O. S., xxvii, 179,
1907.
Theobald and Clapp. T. Am. O. S., xxi, 43,
1923.
Tyson. A. of O., viii, 365, 1932.
Verhoeff. T. O. S., xxiii, 176, 1903.
Vita. Atti S. It. Oft., 1925 : Ref. Zb. ges. O.,
xvii, 555, 1927.
Vögele. K. M. Aug.,
lxxxi, 393, 1928.
Wagenmann. A. f. O., li, 532, 1900.
Weber. P. R. S. Med., xxii, 431, 1929.
Weinstein. K. M. Aug., xlii (2), 577, 1909.
Wheeler. Am. J. O., xx, 368, 1937.
Wiener. A. of O., liv, 481, 1925.
Yamanaka. K. M. Aug., lxxviii, 372, 1927.
Zuereva. Trudy II. Moskow Univ., i, 159,
1927 : Ref. Zb. ges. O., xx, 847, 1929.
lxxiv, 775, 1925 ;
3326 TEXT-BOOK OF OPHTHALMOLOGY
12. ExPERIMENTAL GLAUCOMA
The experimental work which has been done to elucidate the cause of
glaucoma has led to little pragmatic result. We have already seen in the
first volume of this text-book 1 that the intra-ocular pressure may be raised
by several methods—by raising the general or local blood-pressure, by
inducing venous engorgement, by bringing about capillary stasis, by exciting
the local neuro-vascular axon reflexes, by varying the osmotic equilibrium
between the blood and the intra-ocular fluid by lowering the former or
raising the latter, or by increasing the volume-contents of the globe, either
of the aqueous by osmotic means or of the vitreous by changing the reaction.
A condition of established glaucoma, however, is difficult to produce in
experimental animals; it has only been accomplished by variations of
two methods—the production of a permanent condition of vascular stasis
and congestion, and a blockage of the circulation of the intra-ocular fluids.
The resultant glaucoma, of course, is of the secondary type. The usual
experimental animal has been the rabbit in which a condition somewhat
akin to human buphthalmos can be produced provided a considerable
rise of pressure has been maintained for a sufficient time.
(a) THE PRODUCTION OF CIRCULATORY STASIS
By ligating the vortea: veins as they issue from the eye, intra-ocular
pressures up to 80 or 90 mm. Hg can be readily produced (Adamūk, 1867;
Leber, 1873; Weber, 1877; Schöler, 1879; v. Schultén, 1884; Ulrich,
1889 ; Koster, 1895; v. Geuns, 1899 ; and others). The resultant rise in
tension is associated with an extreme vascular dilatation and the formation
of a colloid-rich plasmoid aqueous, but in the course of a few weeks the
pressure returns to normal, probably owing to the opening up of anastomoses
through the anterior ciliary vessels. It is noteworthy that a similar glaucoma
can be produced accidentally by obliterating a vortex vein by diathermy in
operating upon a detached retina. -
After ligating the anterior ciliary veins a similar acute glaucoma results
(Bartels, 1905). In this case, however, the rise of tension is more permanent
and may last several months, pointing to the fact that the venous drainage
of the ciliary body and iris is of more importance than that of the choroid.
It is noteworthy that in all these cases microscopical examination demon-
strates that the angle of the anterior chamber remains open and that no
inflammatory changes of a secondary nature occur in the spaces of Fontana.
A similar result is seen clinically in the secondary glaucoma following
extensive lime burns of the sclera (Zade, 1909).
* Vol. I, p. 506 et seq. (515 et seq.).
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3327
(b) OBSTRUCTION OF THE CIRCULATION OF INTRA-OCULAR FLUID
The most usual method by which this has been achieved is by obstructing
the angle of the anterior chamber. This has been done in three ways:
(i) By the injection of non-diffusible oils and colloids into the eye which
embarrass the filtration channels mechanically, and if they are irritating
in nature, intensify the obstruction by exciting an inflammatory
reaction (Weber, 1877; Heisrath, 1879; Bentzen, 1895; Geering, 1896;
Bajardi, 1896; Troncoso, 1901–05; Schreiber and Wengler, 1909; Seidel,
1921 ; and others).
(ii) By eacciting an inflammatory reaction in the filtration angle with the
production ultimately of synechiae by punctures and scratches at the limbus
(Bentzen, 1893), cauterization (Schöler, 1879; Bentzen, 1895; Kümmell,
1912; Lagrange, 1922), or irritating sub-conjunctival injections (Coburn,
1923).
(iii) By eacciting a proliferative reaction of the endothelium at the
filtration angle by the action of electrolytic products of iron, either by
introducing a steel needle as a positive pole into the anterior chamber, or
introducing therein the prepared electrolytic products (Erdmann, 1907;
Schreiber and Wengler, 1909; Parisotti, 1911; Miyaki, 1923).
An eacperimental obstruction at the pupil was established by Rombolotti (1903)
by the introduction of a celluloid disc ; glaucoma Supervened, but also a severe
complicating irido-cyclitis.
Adamūk. Zb. med. Wiss., v., 433, 1867. Miyaki. Jap. O. Cong. Kioto, 1922 : Ref. Zb.
Bajardi. Atti R. Acad. med. Torino, 1896. ges. O., ix, 87, 1923.
Bartels. Z. f. Aug., xiv., 103, 258, 458, 1905. Parisotti. Bull. S. fr. d’O., xxviii, 340, 1911.
Bentzen. A. f. O., xli (4), 42, 1895. Rombolotti. A. f. Aug., xlvi, 297, 1903.
Coburn. An. A mer. Neurol. Ass., 387, 1923. Schöler. A. f. O., xxv (4), 63, 1879.
Erdmann. A. f. O., lxvi, 325, 391, 1907. Schreiber and Wengler. A. f. O., lxxvi, 99,
Geering. Diss., Basel, 1896.
v. Geums. A. f. O., xlvii, 249, 1899.
PHeisrath. Zb. med. Wiss., 769, 1879.
1909.
v. Schultén. A. f. O., xxx (3), 1, 1884.
Seidel. A. f. O., civ, 357, 1921.
Roster. A. f. O., xli (2), 113 ; (4), 274, 1895. Troncoso. An. d’Oc., csxvi, 401, 1901 ;
Kümmell. A. f. Aug., lxxii, 261, 1912. cxxxiii, 5, 250, 1905.
Lagrange. Du Glaucome et de l’Hypotonie, Ulrich. A. f. Aug., xx, 270, 1889.
Paris, 1922. Weber. A. f. O., xxiii. 1, 1877.
Leber. A. f. O., xix (2), 87, 1873. Zade. A. f. O., lxxii, 507, 1909.
Primary Glaucoma
By primary glaucoma, we mean a glaucoma the cause of which is not
yet known ; it is a conception which will doubtless disappear in the future.
In contrast to secondary glaucoma, which is due to some obvious ocular
lesion, the primary type seems probably to be the local expression of
general systemic pathological or degenerative processes.
T.O.-WOL. III. 4 K
3328 TEXT-BOOK OF OPHTHALMOLOGY
Incidence
Glaucoma is a common disease in all countries of the world, statistics
showing that it represents from 1 to 2% of all ophthalmic cases. This to a
large extent is independent of race as is shown from the following figures
giving its proportionate occurrence among diseases of the eye :—
Europe: France (de Wecker, 1900) e tº 1.17%
Germany (Schmidt-Rimpler, 1908). 1.27%
(Peters, 1930) tº 1.48%
Austria (Peters, 1930) 1.26%
Denmark (Peters, 1930). 2.4%
Sweden (Nelander, 1933) 1.1%
Greenland (Börresen, 1926) . 0.64%
Greece (Bistis, 1925) 1.28%
Italy (Bossalino, 1907) . 2-73%
(Maggi, 1907) ſº e 0.95%
Portugal (da Gama Pinto, 1906) 1.1%
Russia (Lange, 1889) . 1.14%
(Krükow, 1889) . 1.8%
(Wagner, 1897) . 1.6%
Belgium (Coppez, 1900) 1.02%
Poland (Markow, 1897). & º tº 1.25%
America : United States (Lehrfeld and Reber, 1937) 0.78%
Africa : Egypt (MacCallan, 1922) & º * 1.75%
Tunis (Cuénod and Nataf, 1928) 2.0%
Asia : Siberia (Cykulenko, 1930) 1.4%
(Batarkukov, 1929) 2.5%
Turkestan (Pokrowsky, 1924). 2.1%
India (Wright, 1929–31) 1.6–2.6%
China (Pillat, 1933) * º © 1.11%
Indo-China (Bargy, 1929) † º 2.0%
East Indies (Drake-Brockman, 1885) 4.75%
Turkey (de Wecker, 1900) 1.93%
Japan (Kagoshima, 1915) 0.02%
It is generally stated that the percentage among Jews is high (Clausen, 1925);
but this is questionable, for it is possible that partly from racial nervousness and partly
from relatively greater wealth, they tend to seek surgical advice early and are apt to
multiply confirmatory opinions from several surgeons (Brav, 1931 ; Carvill, 1932;
Lehrfeld and Reber, 1937). Some peoples show a very high incidence ; thus Pilman
(1927) reported 15.7% in a region of Turkestan where the population is a mixture of
Turkish, Persian and Mongolian elements.
Sea. The disease is fairly evenly distributed between the sexes,
although statistics show a definite preponderance of females (some 54%).
Thus, analysing 2,785 cases from the literature, (Schmidt-Rimpler (1908) found
1,240 males and 1,545 females. Priestley Smith’s (1879) series of 1,000 cases contained
56.9% females, Haag (1915) found 61%, Carvill (1932), 57%, Weinstein (1934), 60.7%,
Lehrfeld and Reber (1937), 51.5%. The incidence thus varies in different series from
a slight preponderance of females over males to a proportion of 6:5 (Priestley Smith,
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3329
1879), 5:4 (Schmidt-Rimpler, 1908), 3:2 (Rohner, 1927; Lugue, 1929), 2 : 1
(Nelander, 1933), or even 3 : 1 (Pillat, 1933). In Trantas’ (1934) series males slightly
out-numbered the females. In the most exhaustive study of the literature by Bell
(1932) comprising 8,569 cases, 46% were males and 54% females.
The two clinical types of glaucoma also vary between the sexes, for the
35
30 H
25 H
20
T I I I —I |
|0–19 20-29 30-39 40-49 50-59 60-69 70-79 80-89
•—e English cases (1000) (Priestley Smith).
UH- — — — — — -D American cases (1415) (Lehrfeld and Reber).
O— - – - – - —o European cases: German, Russian and Italian (2021)
(Schmidt-Rimpler).
X- - - - - - - . . . . . . . . . . . .× Hereditary cases: all nationalities (196) (Bell).
FIG. 2831.-AGE OF ONSET OF PRIMARY GLAUCOMA.
acute congestive incompensated type is more common in women, while the
chronic non-congestive compensated type is more common in men.
Thus Priestley Smith (1879) found in the congestive type 346 females and 178
males; in the non-congestive type 223 females and 253 males. Similarly Carvill
(1932) found 54% of the non-congestive cases were men. The majority of reports

4 K 2
3330 TEXT-BOOK OF OPHTHALMOLOGY
show a similar distribution, although Nelander (1933) found twice as many females
in both types, and Lehrfeld and Reber (1937) found a preponderance of females in the
congestive type, while the chronic simple type was approximately equally distributed
between the sexes. It is probable that the greater tendency for females to develop
the congestive type of the disease is due to vaSo-motor instability after the
menopause. .
Age. Glaucoma is a disease of late middle life ; in the first and second
decades it is rare ; thereafter it increases slowly until the age of 40 when the
incidence becomes much greater. It is most common in the sixth and
seventh decades, but above 70 years it again becomes rare (Fig. 2831).
The average age is about 60, and the non-congestive type occurs preferen-
tially a little later than the congestive type.
Thus Priestley Smith (1879) found 29.3% of cases in the sixth and 29% in the
seventh decade ; Schmidt-Rimpler (1908) 32% in the sixth ; Haag (1915) 31.8% in
the seventh ; Carvill (1932) 39.6% in the seventh ; ; Pillat (1933) 34% in the sixth.
The occurrence of the disease in young people is referred to as JUVENILE
GLAUCOMA, but different writers put varying limits to this category, some
making the upper limit 20 years, others 35, and others 40. The condition is
not common, but many reports are to be found in the literature since v.
Graefe’s (1862) observation in a girl of 10 years. Thus in his series of
1,032 cases, Haag (1915) found that 4 occurred in the first, 16 in the second,
26 in the third decade, and Lehrfeld and Reber (1937) found 20 cases between
the ages of 18 and 32 out of 1876. There is no marked sex difference in its
incidence; but one significant fact is that a large number of the cases are
myopic, this type of refraction being either as common as hypermetropia
(Löhlein, 1913; Keerl, 1920; Carvill, 1932) or more so (Haag, 1915; Lehrfeld
and Reber, 1937). Some authors have found that a large proportion
have other congenital deformities (Löhlein, 1913; Haag, 1915), but this is
by no means invariable (Lehrfeld and Reber, 1937). Heredity is also claimed
to exert an influence on its occurrence (in 15% Löhlein, 1913), but in the
majority of cases this cannot be demonstrated. As a rule, but not invariably
in such cases, the prognosis is not good, blindness frequently coming on
rapidly despite treatment.
Seasonal Incidence. Although it has been claimed that glaucoma is
more common in hot countries than cold, there is general agreement that it
occurs more frequently in the winter months than the summer, a circum-
stance which applies particularly to acute glaucoma (Steindorff, 1902;
Bauer, 1903; Büttner, 1921 ; Rohner, 1927; Pillat, 1933 ; Weinstein,
1934; Lehrfeld and Reber, 1937). In India it is most frequent in June and
July when solar radiation is at a minimum (Maynard, 1908). This pecu-
liarly has been associated with pupillary dilatation owing to diminished
light, changes in barometric pressure, and the frequency of infections of
the upper respiratory tract.
Bilaterality. In the majority of cases glaucoma becomes eventually
ANOMALIES OF THE INTRA-OCUTLAR PRESSURE 3331
bilateral. Statistics on this question are misleading since many cases which
are unilateral at the time of examination become bilateral at a later date.
Thus Bossalino (1907) found 107 unilateral cases out of 275, and Schmidt-Rimpler
(1908) found 120 out of 379. Lehrfeld and Reber’s (1937) statistics which were more
fully analysed are of more value : out of 392 congestive cases 9 showed early, and 99
advanced unilateral glaucoma, 28 had early, 168 advanced, and 48 absolute bilateral
glaucoma ; of the whole, 42% were seen first when the condition was unilateral and
58% when it was bilateral. In 1,023 non-congestive simple cases, 78 were seen first
with early, 199 with moderately advanced, and 219 with late unilateral disease ; 88
with early, 167 with moderately advanced, and 297 with advanced or absolute bilateral
disease ; that is, 50.7% showed unilateral and 49.3% bilateral involvement on their
first appearance at hospital.
Heredity. Although by no means common, familial glaucoma is a
definite entity. Its occurrence was first noted by Benedict (1842), and the
TTI-I-I-T-L TLTTTL-l
Ö Ó C) d Ó dº Ó CŞ CŞ & O C3 c5
+ + + + + +
T-L-T-L-L-L-l
O is de ó i ö ()
d + + +
Óð k
+ +
FIG. 2832.-HEREDITY OF GLAUCoMA (Lawford).
6ss

|
º, a, , º, ø, &
6 & & © e., o 0 & 3 Ö do o o
+ H. H.8 + + + + + +
FIG. 2833.−HEREDITY OF GLAUCOMA.
Showing anticipation. The figures indicate the ages at which clinical signs
were first noted (Zorab). *
literature has been summarized by Lawford (1907–13), Schmidt-Rimpler
(1908), Groenouw (1920), Clausen (1925) and Bell (1932), the last of whom
collected 295 cases; subsequent families of interest are those of Zorab (1932),
Upthomas (1934) and Briggs (1939). In most cases the records are scanty
and badly documented, but from their study several points of great interest
emerge (Figs. 2832 and 2833). The heredity is dominant and continuous and
is transmitted by both seates, and while either parent is equally potent as a
3332 TEXT-BOOK OF OPHTHALMOLOGY
transmitter, the parent doing so is usually affected. The descent shows
anticipation, as was first noted by v. Graefe (1869); the members of a sib-
ship tend to become liable at approximately the same age, the age being
determined by the age of onset in the parent, an average being that the mean
age of onset in the offspring is some 10 years earlier than that in the parent
(Fig. 2833). For this reason the age of onset in hereditary cases is on the
average some 20 years earlier than in the general series of cases of glaucoma.
Thus Bell (1932) found that 52% of 196 hereditary cases occurred before the
age of 30 years, whilst only 3.6% of 3,021 general cases came under this
category ; as a general rule the hereditary cases have appeared in otherwise
healthy stock with no demonstrable association with any other disease or
congenital defect. -
The mechanism by which the hereditary influence acts is quite unknown.
It has been suggested that the transmission of a small eye and a large lens
may have some influence in the matter, or that congenital malformations
at the filtration angle may be handed down ; but the complete lack of
association between hereditary primary glaucoma and buphthalmos seems
to negative this explanation. The fact remains that the literature contains
no evidence of the source of glaucoma in hereditary cases, but there is no
doubt that the affected members of a family so tainted should abstain from
parentage ; indeed, there is a grave risk to the offspring of any member of
an affected sibship belonging to a stock known to carry the disease in
hereditary form.
Bargy. Bull. S. fr. d’O., xlii, 345, 400, 1929.
Batarkukov. Russ. A. O., vi, 278, 1929.
Bauer. Diss., Tübingen, 1903.
Bell. Treasury of Human Inheritance, ii (v),
Lange. Westm. O., vi, 1889.
Lawford. R. L. O. H. Rep., xvii, 57, 1907;
xix, 42, 1913.
Lehrfeld and Reber. A. of O., xviii, 712, 1937.
448, 1932. Löhlein. A. f. O., lxxxv, 393, 1913.
Benedict. Abl. aus d. Gebiete d. Aug., Lugue. A. de Oft. H.-A., xxx, 317, 1929.
Breslau, 1842. MacCallan. K. M. Aug., lxix., 539, 1922.
Bistis. A. d’O., xlii, 422, 1925. Maggi. Cl. Ocul., viii,. 2872, 1907.
Börresen. Ugeskrift. f. Laeger, lxxxviii, 194, Markow. Westm. O., xviii, 729, 1897.
1926.
Bossalino. Il glaucoma primaria, Pisa, 1907.
Brav. Am. J. O., xiv, 48, 1931.
Briggs. Brit. J. O., xxiii, 649, 1939.
Büttner. A. f. Aug., lxxxviii, 204, 1921.
Carvill. T. Am. O. S., xxx, 71, 1932.
Clausen. Zb. ges. O., xiii, l, 1925.
Coppez : see de Wecker. An. d’Oc., czziv,
45, 1900.
Cuénod and Nataf. Bull. S. fr. d’O., xli., 330,
1928. -
Cykulenko. Russ. O. J., xii, 17, 1930.
Drake-Brockman. O. Rev., iv., 189, 1885.
v. Graefe. A. f. O., viii (2), 242, 1862;
xv. (3), 108, 1869.
Groenouw. G-S. Hb., III, Erbliche Augen-
leiden w. Glaukom, 746, 1920.
Haag. K. M. Aug., liv, 133, 1915.
Kagoshima. Nip. Gank. Zas., 1915 :
Ophthalmology, xii, 791, 1916.
Keerl. Diss., Leipzig, 1920.
Krükow. Westm. O., vi, 327, 1889.
Ref.
Maynard. Brit. Med. J., ii, 744, 1908.
Nelander. Acta O., xi, 370, 1933.
Peters. G-S. Hb., III, Das Glaukom, 1930.
Pillat. A. f. O., exxxix, 299, 1933.
Pilman. Russ. O. J., vi, 1160, 1927.
Pinto, da Gama. Encyc. fr. d’O., xv, 96, 1906.
Pokrowsky. Verhl. Aerzte-kongr. Turkestan,
16, 1924.
Rohner. Schw. med. W., lvii, 780, 1927.
Schmidt-Rimpler. G-S. Hb., II, vi, 1, 1908.
Smith, Priestley. Glaucoma, London, 1879,
1891.
Steindorff. D. med. W., xxviii, 929, 1902.
Trantas. Bull. S. fr. d’O., xlvii, 277, 1934.
Upthomas. Fasciculus Cestriensis, 1934.
Wagner. Intern. Cong. Moskow, 179, 1897.
de Wecker. An. d’Oc., exxiv, 45, 1900.
Weinstein. K. M. Aug., xciii, 794, 1934.
Wright. Administrative Reps., Madras, 1929-
31.
Zorab. T. O. S., lii, 446, 1932.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3333
Predisposing Factors
1. The Refraction. It has long been recognized that the commonest
refractive error met with in primary glaucoma is hypermetropia, so much so,
indeed, that a causal relationship has been inferred, the assumption being
that the hypermetropic eye is usually smaller than the myopic and therefore
suffers blockage of its relatively narrow filtration angle more easily (Priestley
Smith, 1879). It is not to be thought, however, that the myopic eye is
immune, for quite a large percentage of cases occur in myopes, and the
disease may occur even when the myopic error is high ; moreover, the
combined percentage of myopes and emmetropes is higher than that of
hypermetropes. Further, there is general agreement in the literature that
the preponderance of hypermetropia is greater in the congestive type than
in simple chronic glaucoma, ; and it is of clinical importance that the chronic
simple glaucoma occurring in myopes is frequently insidious and is often
missed until a late stage, partly because the tension frequently remains
relatively low, and partly because the appearance of the disc is misinter-
preted as being due to the atrophy accompanying myopic changes.
Priestley Smith’s (1879) statistics are fairly typical : in 1,000 cases he found 45%
hypermetropic, 41% emmetropic and 14% myopic. Other figures are : Gilbert (1912)
in simple glaucoma 38% hypermetropic, and in congestive glaucoma 77% ; Carvill
(1932) found 64% hypermetropic and 29% myopic ; Weinstein (1934) 6.2% hyper-
metropic and 14% myopic ; Rohner (1927) reported 67 cases of hypermetropia against
9 cases of myopia and 130 of emmetropia; and Lehrfeld and Reber (1937) 24%
hypermetropes against 14% myopes in congestive glaucoma, and 4.1% hypermetropes
against 14% myopes in simple glaucoma. The most decisive statistics on this point
are those of Gala (1930), who found the incidence of myopia in primary glaucoma to
be as low as 1.6%. On the other hand, others believe that the myope is just as liable
to glaucoma as the emmetrope or the hypermetrope (Löhlein, 1913), while many have
recorded impressive percentages of its high incidence in myopia—Lange (1896) 43%
myopes in glaucoma simplex ; Goldschmidt (1923) 34 myopes and 4 hypermetropes
in 60 cases of glaucoma simplex ; Knapp (1925) reported a series of 25 cases of simple
glaucoma in myopes, and so on. Quite a number of cases, also, have been reported
in which the degree of myopia was high (Ischreyt, 1910; Gilbert, 1912; Rolandi, 1913;
and many others).
2. Mydriasis. Anything which brings about a dilatation of the pupil
may precipitate a glaucomatous attack in a predisposed eye. A mydriasis
may be brought about by the eacclusion of light from the eye (Grönholm
1910), and as we shall see presently, the rise of ocular tension on dark
adaptation in an eye predisposed to glaucoma forms the basis of a useful
clinical method of diagnosis. This has been attributed to crowding of the
angle of the anterior chamber with the retracted iris, thus embarrassing the
drainage of the intra-ocular fluid (Seidel, 1920–27 ; Serr, 1929), but the fact
that the typical reaction occurs after the administration of pilocarpine or
atropine or after an iridectomy or in aniridia (Brückner, 1922; Puscariu,
3334 TEXT-BOOK OF OPHTHALMOLOGY
Cherchez and Nitzulescu, 1925; Feigenbaum, 1928–29) indicates that the
dilatory action of light on the intra-ocular capillaries must be considered.
Mydriatic drugs are a more common cause of an acute rise in tension in
eyes previously free from clinical signs of glaucoma (MYDRIATIC GLAUCOMA).
Of these atropine is much the most common, and following the initial
caution by v. Graefe (1858), there are a large number of reports in the
literature of an intractable acute glaucoma being caused thereby in pre-
disposed eyes or in eyes already affected by chronic glaucoma (see Schmidt-
Rimpler, 1908; Peters, 1930; Abraham, 1933). A similar result has
followed the subcutaneous injection of atropine. (Myashita, 1914). More
rarely cases have been reported after homatropine (Hodges, 1885; Rogers,
1895; Shears, 1900; Gifford, 1900–16; Pyle, 1903; Friedman, 1908;
Stevenson, 1913; Levitt, 1917; Levinsohn, 1918; Desai, 1919), scopol-
amine (Walter, 1899), euphthalmine (Ring, 1903; Breuil, 1909),
cocaine (Manz, 1885; Javal, 1886; Hinshelwood, 1900; Snell, 1901;
Sergiewski, 1903; Hamilton, 1917; Ganguly, 1923; and others) and even
holocaine (Plastinin, 1914; Gjessing, 1914; Hughes, 1917). The action of
these drugs may be three-fold: the mydriasis tends to embarrass the filtration
angle, the paresis of the ciliary muscle, by abolishing the pull upon the
scleral spur, also allows the filtration angle to become more readily occluded,
and they cause a vasodilatation with consequent congestion and stasis. Of
the three factors the last should not be forgotten, for it is not counteracted
by a miotic ; moreover, its importance is seen in the production of a
mydriatic glaucoma after the instillation of atropine into an eye with aniridia
(Thiel, 1922).
Unfortunately these cases may be severe and an acute attack of con-
gestive glaucoma may be the result ; those following atropine may be
disastrous, since in the absence of drugs which can neutralize the effect, an
operation must frequently be undertaken on a highly congested eye. Care
should therefore be taken not to administer mydriatics as a routine, particu-
larly in patients over 40, until the absence of an early glaucoma has been
excluded ; and in all cases eserine should be instilled subsequently and seen
to be effective before the patient leaves. It is to be remembered, however,
that even this precaution has not always prevented the disaster, since the
action of the mydriatic may outlast the effect of the miotic.
3. The Properties of the Blood. A great deal of work has been done on
the physico-chemical properties of the blood in glaucoma with a view to
determining aetiological factors, but it must be said at once that an analysis
of the literature yields extremely meagre and incomplete results, frequently
confusing and contradictory, from which no definite conclusions can be
drawn.
1 Vol. I, p. 519 (528).
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3335
With regard to the cellular constitution of the blood reports are few. Passow (1930)
reported an increase in erythrocytes, and Massoud (1937) of mononuclears, findings
which are probably without much significance.
With regard to the chemistry of the blood, it would appear that there is no
abnormality in the proteins, sugar, sodium and phosphorus (Cohen, Killian and
Halpern, 1932); Ascher (1922), however, found the chlorides slightly diminished, but
Passow (1930) and Gionnantoni (1932) found them increased. There have been
several investigations into the cholesterol content, and although the reports are not
unanimous, the conclusion is legitimate that a hypercholesteraemia is not usually
present (Salvati, 1929 ; Peterson and Levinson, 1930 ; Musselevich, 1930 ; Passow,
1930; Trovati, 1935). More interest has attached to the calcium and potassium,
partly because a lowering of the potassium-calcium quotient inclines towards alkalinity,
while a decrease of calcium increases the excitability of the sympathetic nervous
system and the permeability of the capillaries. It is to be remembered that it is the
ionic diffusible calcium that is concerned in this activity, the determination of which
is still outside the realm of practical laboratory procedure. Nevertheless, a diminution
of the calcium and an increase of the potassium have been reported by Biffis (1933–34),
Trematore (1934), Trovati (1935), and others, more indeterminate results were recorded
by Rossi (1932), Cohen, Killian and Halpern (1932), and Tron and Odnasheva (1937),
while the opposite relation was found by Fradkin, Krasnoff and Chifetz (1930).
Although, therefore, the majority of investigators have found a low quotient, the
variations are by no means constant and at most not great enough to alter seriously
the alkaline reserve or raise the sympathetic tonus.
With reference to the physical properties of the blood most attention has been
devoted to the reaction. Some authors have claimed to have found an increased
alkalinity (Baurmann, 1925; Meesmann, 1925; Mawas and Vincent, 1926; Redslob,
1928; Kubik, 1928; Trovati, 1935; and others), while others have got indeterminate
results (Seidel, 1927; Jasinski, 1927; Gala and Melka, 1928; Schmerl, 1928; Wegner
and Endres, 1928; and others). The osmotic pressure was claimed to be slightly
below normal in glaucoma by Hertel and Citron (1921) and Pletnewa (1923); but in
all these cases sufficiently consistent material is lacking to lead to any pragmatic
conclusion or to justify the opinion that the blood of the glaucomatous differs from
the normal to any significant degree.
4. The Capillary System. There is a considerable amount of evidence
that one of the peculiarities commonly occurring in glaucomatous subjects is
a generalized disturbance of the capillary system. Scheerer (1924), indeed,
came to the conclusion that every case of glaucoma showed some patho-
logical change in structure or in function of the capillaries of the skin where
there was constriction of the arterial part and dilatation of the venous part
of the capillary loops, tending especially towards stasis, dilatation and
increased permeability. Scheerer (1924) also found a lack of regular pulsa-
tion in the peri-macular capillaries when they were viewed entoptoscopically.
This view has been supported by capillaroscopic and oscillometric observa-
tions by several observers who have noted other general signs of vaso-neurosis
(Pletnewa, 1926; Horniker, 1928; Schmidt, 1929; Wegner, 1930; Petersen
and Levinson, 1930; Goldenburg, 1931; Saint-Martin and Mériel, 1932;
Ferrari, 1932; Meizaros and Toth, 1933; Tewbin and Wilensky, 1933);
while Dieter (1928), using a subjective method of measurement, found the
capillary pressure in the eye always raised in glaucoma.
3336 TEXT-BOOK OF OPHTHALMOLOGY
This tendency to derangement of the capillary endothelium is seen in the positive
results got by the “drinking water test " of Marx (1926–28)—a quantity of distilled
water (50 gm. or over) is drunk on an empty stomach, after which determinations of the
haemoglobin, and the amount and specific gravity of the urine are said to give a typical
curve, deviations from which are taken to indicate changes in the capillary permeability
(Schmidt, 1928–31 ; Wegner, 1930): at the same time the intra-ocular pressure rises.
The same tendency is also seen in the increaesd albumen content of the aqueous
Sometimes found in glaucomatous patients (Troncoso, 1902) and in the increased ease
with which fluorescein enters the eye after systemic injection, a peculiarity which,
as we shall see, has been used as a diagnostic test for early glaucoma (Thiel, 1922).
5. Systemic Diseases. The influence of systemic diseases in determining
the onset of primary glaucoma is very difficult to evaluate. It is probable
that acute febrile diseases tend to precipitate an acute congestive attack, and
it is only to be expected that the vaso-motor instability accompanying such
illnesses would readily form a predisposing cause. A long list could be
quoted—acute sepsis, erysipelas, influenza, etc.—but their influence is
essentially incidental. In the more chronic types of the disease, focal sepsis
has been frequently associated with the aetiology (Kerry, 1925; Geiger and
Roth, 1928; Levy, 1930; Rossi, 1932; and many others); any part which
such factors play in the aetiology of glaucoma is questionable, although their
association with the chronic types of secondary glaucoma is more close.”
With regard to the vascular and metabolic diseases the position is somewhat
different, and many authorities maintain, and with much reason, that
primary glaucoma is essentially a local symptom of a systemic disease of
this type. On the other hand, it must be remembered that although
statistics can be quoted showing a high percentage of syphilis, arterio-
sclerosis, hypertension, nephritis, diabetes, and so on, in glaucomatous
subjects, the figures given are rarely higher than would be expected in any
sample group of the population of the same age. It is undoubtedly the case,
however, that glaucoma does tend to occur in subjects of cardio-vascular
disease, and Lagrange (1922) is certainly correct in his contention that a
glaucomatous eye is “a sick eye in a sick body.”
A high blood-pressure has from time to time been considered to be
aetiologically associated with glaucoma, largely because of the close physio-
logical relationship between it and the intra-ocular pressure ; while this
relationship undoubtedly holds for physiological variations, however, it is
by no means true clinically, for a high systemic pressure is frequently
associated with a normal or a low capillary pressure (and on this latter the
pressure of the eye depends) for the reason that the block offered by spastic
or sclerosed arterioles frequently results in a deficient capillary circulation.
In non-glaucomatous cases, therefore, it is found that a raised systemic
pressure is not associated with a high intra-ocular pressure (Foster Moore,
1915). In glaucomatous subjects reports vary. The first investigators to
examine the problem (Terson and Campos, 1898) found, as most of their
1 p. 3288.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3337
successors have done, that many cases are not associated with a high blood-
pressure, and concluded that there was no causal relationship between the
two. Their immediate followers differed somewhat, Bajardi (1900), Joseph
(1904), Frenkel and Garipuy (1906), Kümmell (1911)and Gilbert (1912) finding
the blood-pressure high in cases of glaucoma, while Krämer (1910), Craggs
and Taylor (1913) and MacRae (1915) found little difference from controls.
Subsequent statistical evidence has shown that although a high-blood-pressure
and a high intra-ocular pressure are frequently associated and are to a certain
eatent inter-related, the relation between them is accidental rather than essential
(Elschnig, 1917; Wessely, 1918; Jackson, 1918; Viguri, 1923; Kahler
and Sallmann, 1923; Vele, 1933; Weinstein, 1935; and many others).
It may be taken, therefore, that the systemic blood-pressure is not a primary
Or even an important factor in the aetiology of glaucoma. It must be
remembered, however, that while in the normal individual the regulating
mechanism is very efficient and the blood-pressure is kept at a remarkably
constant level, in the high-pressure subject this mechanism has ceased to be
adequate, and the pressure, in addition to being maintained at an abnormally
high level, is subject to sudden and extensive variations excited by influences
so slight as to have little recognizable effect in the normal person. To this
extent, therefore, a hyperpietic diathesis will predispose to an acute crisis
in the eye.
Moreover, it is certainly true that glaucoma is very frequently associated
with arteriosclerosis, most usually of a widespread nature. Charlin (1923),
indeed, from an extensive clinical study, concluded that 90% of such
patients showed well-marked degenerative and diseased conditions in the
vascular system, while Calhoun (1929) put the incidence at 95%. Such
changes are a constant index of general deterioration in the tissues, and we
shall see in the section on pathology that arteriosclerotic changes are the
rule in glaucomatous eyes. In the same way nephritis is commonly met with
(37%, Calhoun, 1929); and syphilis has frequently been associated in the
aetiology. This association was first suggested by Pflüger (1885), and has
been supported by many observers (Elschnig, 1913; Hirschberg, 1919;
Charli, 1923; Carlotti, 1923; Arnoux, 1924; Lugue, 1929; Calhoun,
1929; Mazal, 1931). So much, indeed, have some clinicians been impressed
by the relationship that the term “primary syphilitic glaucoma ‘’ has been
used ; but others have reported on its rarity (6 in 534 cases, Giesler, 1925;
Beckh, 1935). It is probable that primary glaucoma is in no special way
related to syphilis, and that any influence the latter may have is indirect
Only in so far as the infection tends to bring about vascular disease.
But it is unquestionably the case that a large group, particularly of cases of
chronic glaucoma, are the victims of vascular sclerosis and degeneration.
6. The Neuro-vegetative System. Ever since Adamiik’s experiments
indicating a preliminary rise in the intra-ocular pressure on stimulation of
3338 TEXT-BOOK OF OPHTHALMOLOGY
the sympathetic nerve 1 and Jonnesco's advocacy of the treatment of
glaucoma by sympathectomy, which will be noted presently, a hyper-
excitability of the sympathetic has been stressed as a characteristic of
glaucomatous patients. Particular emphasis was thrown upon this
characteristic by Felix Lagrange (1922), who went so far as to say that “at
the base and at the beginning of every glaucoma there is an intervention
of the sympathetic nerve ’’; this view has been advocated by many writers
(Pichler, 1917; Abadie, 1923; Andrade, 1924; Lagrange, 1925; Scalinci,
1926; Bistis, 1930; Smith and Barkan, 1930; and many others). Evi-
dences of sympathetic upset are seen in general vaso-motor instability, the
tendency for the development of oedema of the angio-neurotic type (Barkan,
1919; Bailliart, 1930), the ready dilatation of the pupil with adrenalin
(A. Knapp, 1921 ; Mazzei, 1925; Thiel, 1925; Vannas, 1927), the frequent
response of the hypertensive state to the vaso-stimulatory action of adrenalin,
and the association of attacks of glaucoma with the menses and psychic
disturbances. Some writers consider the diathesis a diminution of sym-
pathetic tone (Hamburger, 1924–25), others an increase (Thiel, 1926); but
there is no doubt that patients suffering from glaucoma are as a rule unstable
in their neuro-vegetative reactions, although this instability does not corre-
spond uniformly to a vagotonia. It is rather an absence of the normal
balance between the sympathetic and para-sympathetic systems, varying
at one time to a preponderance of the one and at another time of the other.
The influence of the menses may be considered in this connection, for there is no
doubt that acute attacks of hypertension tend preferentially to occur during the
menstrual epoch (Marx, 1923; Salvati, 1923; Lagrange, 1925; Costi, 1930;
Weinstein, 1935), while it has already been noted that the frequency of glaucoma in
women is probably to be explained by the vaso-motor instability at the menopause.
"Psychic disturbances have also been frequently observed as determining the onset
of an attack of glaucoma—nerve-shock and strain, anxiety, overwork, sleeplessness,
nervous exhaustion, while the ordeal of an operation on one eye may precipitate an
attack in the other (Sonder, 1906; Maiden, 1917; Scalinci, 1924; Besso, 1924;
Krasso, 1930; and many others). Indeed the emotional and excitable temperament
of glaucomatous subjects has been recognized for over a century ; at the
beginning of the last century it was remarked on by Demours (1818), and was well known
in the middle of the century to such observers as v. Graefe (1853–55), Donders (1862)
and de Wecker (1863).
7. The Endocrine Glands. Closely related to an imbalance of the neuro-
vegetative system in its effects on vaso-motor control is a lack of stability
in the endocrine system, and a host of authors have called attention to this
factor in glaucomatous subjects (Hertel, 1918; Imre, 1921–24; Paltracca,
1921 ; Lagrange, F., 1922; v. Csapody, 1923; Müller, 1924; Scalinci,
1924; Lagrange, H., 1925; Brana, 1925; Lamb, 1926; Salvati, 1928;
Passow, 1930; Mossa, 1934; and many others). Passow (1930) claimed to
have demonstrated an excess of iodine in the blood in 83% of glaucomatous
1 Vol. I, p. 522 (531).
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3339
cases indicating a thyrotoxicosis, while he, Wessely (1918), and Tron and
Odnasheva (1937) found an increase of a vaso-constricting substance
(epinephraemia) indicating over-action of the adrenals; Dollfus and Séguy
(1936) also found in the urine an abnormal quantity of folliculin. The pre-
cise influence of the hormones in regulating the balance between the
sympathetic and para-sympathetic systems in thus controlling the tone of
the blood-vessels and through them the constitution of the tissue-fluids is a
matter which yet requires much elucidation ; but it seems undoubted that
the influence of the thyroid, pituitary and supra-renals in determining the
incidence of glaucoma must be considered, while that of the ovaries is not
by any means negligible.
In summary, therefore, while it must be admitted that many of these
observations are scanty and somewhat perfunctory, it may be said in general
terms that primary glaucoma tends to occur in persons over middle age who
have some vascular disability, either of Sclerosis or of dysfunction : the first
group is essentially of a degenerative type ; the second is one of which medical
science has yet scanty knowledge, but it is characterized essentially by vaso-
motor and endothelial instability and bound up with imbalance in the neuro-
wegetative and endocrine systems.
Abadie. La Clin. Opht., xii, 303, 1923.
Abraham. A. of O., x, 757, 1933.
Andrade. An. d’Oc., clxi, 771, 1924.
Arnoux. La Clin. Opht., xiii, 440, 1924.
Ascher. A. f. O., cvii, 247, 1922.
Bailliart. Bull. S. d’O. Paris, xlii, 786, 1930.
Bajardi. Giorn. del. Acad. med. Torino, czv,
307, 1900.
Barkan. A m. J. O., ii, 800, 1919.
Baurmann. A. f. O., czvi, 96, 1925.
Beckh. Am. J. O., xviii, 1129, 1935.
Besso. Boll. d’Oc., iii, 683, 1924.
Biffis. Am... di Ott., lxi, 109, 284, 1933.
K. M. Aug., xcii, 138, 1934.
Bistis. A. d’O., xvii, 96, 1930.
Brana. Z. f. Aug., lvi, 68, 1925.
Breuil. La Clin. Opht., xv, 146, 1909.
Brückner. B. O. G. Heidel.., xliii, 97, 1922.
Calhoun. Am. J. O., xii, 265, 1929.
Carlotti. La Clin. Opht., xii, 428, 1923.
Carvill. T. Am. O. S., xxx, 71, 1932.
Charlin. Am. d’Oc., clviii, 861, 1921.
K. M. Aug., lxx, 123, 1923.
Cohen, Killian and Halpern. A. of O., viii,
39, 1932.
Costi. A. di Oft. H.-A., xxx, 521, 1930.
Craggs and Taylor. Ophthalmoscope, xi, 350,
- 1913.
v. Csapody. K. M. Aug., lxx, 111, 1923.
Demours. Traité des maladies des yeur,
Paris, 1818.
Desai. Brit. J. O., iii, 251, 1919.
Dieter. A. f. Aug., xcix, 678, 1928.
Dollfus and Séguy. Bull. S. d’O. Paris,
xlviii, 229, 1936.
Donders. A. f. O., viii (2), 160, 1862.
Elschnig. Prag. med. W., xxxviii, 377, 1913.
A. f. O., xcii, 101, 237, 1917.
Feigenbaum. K. M. Aug., lxxx, 577, 1928.
ACIII Internat. Cong. O., Amsterdam, ii, 491,
I929.
Ferrari. A. di Ott., xxxix, 147, 1932.
Fradkin, Krasnoff and Chifetz. Russ. A. O.,
vii, 786, 1930.
Frenkel and Garipuy. A. d’O., xxvi, .. 645,
1906.
IFriedman. O. Rec., xvii, 92, 1908.
Gala. Oft. Sbornik, v, 119, 1930.
Gala and Melka.
1928.
Ganguly. Indian Med. Gaz., lviii, 379, 1923.
Geiger and Roth. Ill. Med. J., liii, 110,
1928.
Giannantoni. Let. Oft., ix, 319, 1932.
Giesler. K. M. Aug., lxxiv, 776, 1925.
Gifford. O. Rec., ix, 328, 1900.
J. Am. Med. As., lxvii, l 12, 1916.
Gilbert. A. f. O., lxxxii, 389, 1912.
Gjessing. K. M. Aug., liii, 379, 1914.
Goldenburg. Am. J. O., xiv, 944, 1931.
Goldschmidt. Diss., Halle, 1923.
v. Graefe. A. f. O., i (2), 299, 1853; ii (1),
248, 1855; iv. (2), 127, 1858.
Grönholm. A. f. Aug., lxvi, 346; lxvii, 136,
1910.
Hamburger. Med. Kl., xx, 274, 1924.
D. med. W., li, 186, 1925.
FIamilton. Liverpool Med. Chir. J., xxxvi,
156, 1917.
Hertel. B. O. G. Heidel.., xli, 57, 1918.
Cas. lek. Cesk., lxvii, 172,
3340
TEXT-BOOK OF OPHTHALMOLOGY
Hertel and Citron. A. f. O., civ, 149, 1921.
Hinshelwood. O. Rev., xix, 305, 1900.
Hirschberg. Cb. pr. Aug., xliii, 129, 1919.
Hodges. A. of O., xiv, 42, 1885.
Horniker. A. f. O., czix, 488; czXi, 347,
1928. -
Hughes. Am. J. O., xxxiv, 140, 1917.
Imre. A. f. Aug., lxxxviii, 155, 1921.
K. M. Aug., lxxi, 777, 1923; lxxiii, 206,
1924.
A. of O., liii, 205, 1924.
Ischreyt. A. f. O., lxxiii, 566, 1910.
Jackson. Am. J. O., i, 373, 1918.
Jasinski. Kl. Ocz., v, 97, 110, 1927.
Javal. Progr. Med., xvii, 355, 1886.
Joseph. Thèse de Paris : Ref. A. d’O. xxiv,
708, 1904.
Rahler and Sallmann. Wien. kl. W., xxxvi,
883, 1923.
Kerry. T. O. S., xlv., 355, 1925.
Rnapp. T. Am. O. S., xxiii, 61, 1925.
Enapp, A. A. of O., l, 556, 1921.
FCrämer. A. f. O., lxxiii, 349, 1910.
Rrasso. Z. f. Aug., lxx, 355, 1930.
Rubik. A. f. Aug., xcviii, 483, 1928.
Rümmell. A. f. O., lxxix., 183, 1911.
Lagrange, F. Du Glaucome et de l’Hypotonie,
Paris, 1922.
Lagrange, H. Brit. J. O., ix, 398, 1925.
Lamb. T. Am. O. S., xxiv, 105, 1926.
Lange. Abh. v. Vossius, i (7), 36, 1896.
Lehrfeld and Reber. A. of O., xvii, 712, 1937.
Levinsohn. K. M. Aug., lxi, 174, 1918.
Levitt. N. Y. Med. J., cvi, 362, 1917.
Levy. Am. J. O., xiii, 991, 1930.
Löhlein. A. f. O., lxxxv, 393, 1913.
Lugue. A. de Oft. H.-A., xxx, 545, 1929.
MacRae. Ophthalmoscope, xiii, 304, 1915.
Maiden. O. Rec., xxvi, 174, 1917.
Manz. B. O. G. Heidel.., xvii, 118, 1885.
Marx, E. An. d’Oc., clx, 873, 1923.
Marx, H. Kl. W., iv (2), 2339, 1925; v (1),
92, 1926; vi (2), 1750, 1927.
Deut. A. f. kl. Med., clii, 354; cliii, 358,
1926; clviii, 149, 1928.
Massoud. Brit. J. O., xxi, 559, 1937.
Mawas and Vincent. Proc. Intern. Phys.
Cong. Stockholm, 1926.
Mazal. Brat. lek. listy., xii, 82, 1931.
Mazzei. Bol. d’Oc., iv, 588, 1925.
Meesmann. A. f. Aug., xcvii, l, 1925.
Meizaros and Toth. K. M. Aug., xc, 67, 1933.
Moore, Foster. R. L. O. H. Rep., xx, 115,
1915.
Mossa. Ras. It. d’O., iii, 28, 1934.
Müller. Wien. kl. W., xxxvii, 1270, 1924.
Musselevich. Russ. A. O., vii, 529, 1930.
Myashita. K. M. Aug., lii, 561, 1914.
Paltracca. A. di Ott., xxviii, 129, 1921.
Passow. A. f. Aug., ciii, 111, 1930.
Peters. G-S. Hb., III, Das Glaucom., 151,
1930. -
Peterson and Levinson. A. of Path., ix, 282,
1930.
Pflüger. B. O. G. Heidel., xxiii, 91, 1885.
Pichler. A. f. Aug., lxxxii, 194, 1917.
Plastinin. K. M. Aug., lii, 896, 1914.
Pletnewa. Russ. O. J., ii, 7, 1923.
Fest. P. Averbach., 1926.
Puscariu, Cherchez and Nitzulescu.
Biol., xcii, 1085, 1925.
Pyle. T. Am. Med. As., x1, 1725, 1903.
Redslob. An. d’Oc., clxv, 641, 1928.
Ring. T. Am. O. S., xxxix., 189, 1903.
Rogers. O. Rec., v, 421, 1895.
Rohner. Schw. med. W., lvii, 780, 1927.
Rolandi. A. dº Ott., xli., 741, 1913.
Tossi. Am... dº Ott., xxxix, l, 51, 1932.
Saint-Martin and Mériel. A. d’O., xlix, 705,
1932.
Salvati. Am. d’Oc., clx, 568, 1923.
Giorn. di Ocul., ix, 54, 1928.
A. di. Ott., xxxix, 1, 39, 1932.
Scalinci. Giorn. di Ocul., v, 33, 1924.
Am... dº Ott., liv, 235, 1926.
C. R. S.
Scheerer. K. M. Aug., lxxiii, 29, 67; lxxiv,
688, 1924.
Schmerl. A. f. Aug., xcviii, 565, 1928.
Schmidt. A. f. Aug., xcviii, 569, 1928;
c—ci, 190, 1929; civ, 102, 1931.
Schmidt-Rimpler. G-S Hb., II, vi, 134, 1908.
Seidel. A. f. O., cii, 415, 1920; civ, 162,
1921 ; czix, 15, 1927.
R. M. Aug., lxxviii, 77, 1927.
Sergiewski. K. M. Aug., xli (1), 554, 1903.
Serr. A. f. O., czzi, 3, 1929.
Shears. T. O. S., xx, 254, 1900.
Smith and Barkan. Am. J. O., xiii, 1076,
1930.
Smith, Priestley. Glaucoma, London, 1879,
1891.
Snell. O. Rev., xx, 31, 1901.
Sonder. A. d’O., xxvi, 567, 1906.
Stevenson. Ophthalmoscope, xi, 73, 1913.
Terson and Campos. A. d’O., xviii, 209,
1898.
Tewbin and Wilensky. Z. f. Aug., lxxx, 141,
1933. -
Thiel. K. M. Aug., lxviii, 244, 1922; lxx,
766, 1923 ; lzxvii, 753, 1926.
A. f. Aug., xcvi, 34, 331, 1925.
Trematore. Let. Oft., xi, 161, 1934.
Tron and Odnasheva. Sov. vest. O., xi, 3,
1937.
Troncoso. An. di Ott., iv, 97, 135, 167, 1902.
Trovati. An. di Ott., lxiii, 641, 1935.
Vannas. Acta O., iv, 339, 1927.
Vele. An. dº Ott., lxi, 511, 1933.
Viallefont et Lafont. Bull. S. fr. d’O., xlviii,
455, 1935.
Viguri. An. S. Mea. Oft., iv, 114, 1923.
Walter. An. d’Oc., cxxi, 306, 1899.
de Wecker. Traité, Paris, 1863.
Wegner. A. f. Aug., ciii, 511, 1930.
Wegner and Endres. Z. f. Aug., lxiv, 43,
1928.
Weinstein. K. M. Aug., xciii, 794, 1934.
A. of O., xiii, 181, 1935.
Wessely. A. f. Aug., lxxxiii, 99, 1918.
B. O. G. Heidel., xli, 57, 1918.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3341
Pathology
As has already been mentioned, the study of the pathology of glaucoma
is rendered difficult by the fact that almost all the histological material is
represented by eyes which have been excised in the absolute stage of the
disease, long after the presence of a high tension has produced extensive
and widespread degenerative changes. Some few specimens have been
obtained, however, in relatively early stages, particularly in patients who
have died from other causes. These will be reviewed first, and then a
résumé will be given of the changes which result from the continued action
of an increased intra-ocular pressure from any cause. It is to be expected
that, as primary glaucoma is not a single disease-entity, eyes with hyper-
tension do not by any means present a consistent histological picture, so
that any description cannot have a universal application ; but they have
many features in common which are more or less characteristic.
In chronic simple glaucoma it is obviously only by chance that an eye in
the early stages is obtained for histological examination, and the three most
relevant cases which had not been subjected to high or prolonged tension
provide little satisfactory information (Levinsohn, 1908; Rönne, 1913;
Hanssen, 1918). All of them showed evidences of circulatory stasis and
vascular lesions, either of peri- or endo-phlebitis of the episcleral or vortex
veins, with areas of leucocytic infiltration : in one the angle of the anterior
chamber was free, in the other two it was to some extent infiltrated with
cells and pigment.
Similarly in acute congestive glaucoma only a few eyes have reached
histological investigation at an early stage (Birnbacher, 1890; Elschnig,
1896; Friedenwald, 1930); to these may be added two cases in which excision
was performed soon after an acute attack had superimposed itself upon a
chronic simple glaucoma (Hussels, 1912; Schieck, 1934). All of them are
singularly and significantly alike in their pathology, the essential feature
of which was in each case a generalized oedema, involving not only the
vascularized tissues, but also the cornea, the retina, and the optic nerve.
The Oedema seemed to be due to extreme vaso-dilatation and congestion,
associated usually with a perivascular cellular infiltration and with a sero-
fibrinous or haemorrhagic extravasation. This was general over the uveal
tract, but was centred particularly in the ciliary body which had been swollen
and approximated the cornea to narrow or obliterate the filtration angle.
A finding common to four of the cases was an obliteration of one or more of
the vortex veins by endo- or peri-phlebitis or thrombosis.
The Pathological Results of Increased Pressure
In the cornea the epithelium almost invariably becomes Oedematous in
conditions of high tension, with the result that this tissue becomes opaque
(Fig. 2834). The process was studied histologically first by v. Graefe (1853),
3342 TEXT-BOOK OF OPHTHALMOLOGY
Leber (1878) and Fuchs (1881–1902). It is commonly held that fluid is
forced into the substantia propria under pressure from the periphery and
traverses Bowman's membrane by way of the nerve canals (Sgrosso and
Fig. 2834. Conneau (Enemia.
A glaucomatous eye. On the left the middle cells are elongated and vacuolated:
centrally a large vesicle raises up the epithelium. The lamellae are separated by
fluid, and Bowman's membrane has disappeared except towards the extreme right
which is near the centre of the cornea (x 60) (Parsons).
Antonelli, 1890; Newolina, 1908), although Redslob (1936) maintained that
it enters the cornea through lesions in the endothelium and that Bowman's
membrane offers little resistance to its transit. The oedema appears first
Fig. 2835. PANNUs DEGENERATIvus.
From a case of glaucoma. There are vessels and granulation tissue between the
degenerated epithelium and the intact Bowman's membrane. In the granulation
tissue lie large islands of epithelium (Parsons).
as droplets between the basal epithelial cells, between which they are
sometimes seen arranged in rows like beads in a rosary; these may coalesce
into vesicles, lifting a length of cells from Bowman's membrane, while the
fluid filters between the polygonal cells, forcing them apart, until eventually


ANOMALIES OF THE INTRA-OCULAR PRESSUERE 3343
in the severer forms large bullae are formed and entire layers of cells may be
exfoliated. At the same time degenerative changes in the cells—turgescence,
shrinkage, necrosis and vacuolation—are manifold. It is possible that the
process may be accelerated by abolition of the trophic influence of the
corneal nerves owing to pressure (Birnbacher and Czermak, 1885–86), a
phenomenon which explains the corneal anaesthesia. In Bowman’s mem-
brane the canaliculi of their perforating branches become wider, and next
the epithelium hyaline-like bodies may be seen in association with them,
which probably represent degenerative products (Wolff and Lyle, 1937;
Talbot, 1938; Salzmann, 1938) : the significance of these changes,
however, is unknown. The corneal parenchyma itself becomes vacuolated
and the interstitial cells degenerate (Courtis, 1933); while the endothelium
becomes rarefied and eventually proliferates in places, especially near the
angle. On its inner surface there are frequently found cells and pigment
from the degenerated iris.
In long-standing cases degenerative changes of a more permanent
character appear which are termed glaucomatous (or degenerative) pannus
(Baas, 1900; Bietti, 1908; Gilbert, 1909; Fuchs, 1916; and others)
(Fig. 2835). The corneal surface becomes dull and uneven and is frequently
broken by the bursting of vesicles. Histologically the essential change is
the appearance of connective-tissue, at first between Bowman’s membrane
and the degenerated epithelium ; this invades the epithelium to form bullae
and encloses islands of epithelial cells in its meshes, and also penetrates
inwards, eroding Bowman's membrane and infiltrating the substantia
propria. The terminal result may be the formation of an opaque homo-
geneous mass showing large areas of hyaline and amyloid degeneration.
The uveal tract shows changes either of congestion or degeneration, the
former preponderating in the acute and the latter in the chronic forms of
glaucoma. The acute stages of hypertension are marked by extreme
venous stasis throughout the entire uveal tract, so that the tissues are
swollen by oedema and congested with fibrin, leucocytes, and red cells. In
the iris this process may lead to the formation of bullae on the anterior surface
(Redslob, 1935) and eventually to extensive necrosis ; the ciliary body
becomes enormously swollen, and the choroid heavily engorged. This
effect is seen most markedly in the ciliary processes, which become
greatly thickened, the spaces between them are obliterated and they are
pushed far forwards beyond their normal limit so that they become tightly
wedged between the iris in front and the lens behind, narrowing the anterior
chamber and thrusting the root of the iris forward, frequently to come into
contact with the posterior surface of the cornea (Priestley Smith, 1879;
Friedenwald, 1930 ; and many others).
In the later stages of an acute attack and in chronic cases this picture is
replaced by degenerative changes. The iris becomes thin and atrophic,
T.O.-WOL. III. 4 L
3344 TEXT-BOOK OF OPHTHALMOLOGY
vascular sclerosis is usually extremely marked and the retraction of the tissue
results in a marked ectropion of the pigment layer' (Fig. 2836). The
chromatophores become rounded and gradually disappear, and the epithelial
pigmentary cells disintegrate, liberating their pigment which is scattered
over the surface of the iris, powdering the corneal endothelium and collecting
in the angle of the anterior chamber. The muscular tissue disappears, the
stroma becomes rarefied, and eventually advanced degeneration and atrophy
result in the appearance of dehiscences and the formation of a tissue formed
of little more than fibroblasts. Similarly the ciliary processes retract and
shrink, the muscle atrophying and the whole ciliary body becoming flattened
(Fig. 2837). The choroid also atrophies and becomes thin until it may be
represented by little more than a pigmented line.
One feature of the whole uveal tract which may be of considerable
- - º
-º-º:
-
Fig. 2836–THE IRIs IN CHRoxic Fig. 2837. THE CILLARY Body is
Glaucovia. Chronic GLAucoma.
Showing general sclerosis with ectropion Showing marked atrophy (Castroviejo).
of the pigment layer and migration of
pigment towards the anterior surface
(Castroviejo).
importance is the frequency of a perivascular infiltration which is almost
universal in the later stages of congestive glaucoma (Fig. 2838); it is
probably a result of circulatory stasis (Latham, 1938). Disturbances of a
more widespread nature have frequently been seen at the venous exits, some-
times involving the anterior ciliary veins (Bartels, 1905; Heerfordt, 1913),
but more especially the vortex veins. In the case of the latter, advanced
changes are common, particularly in congestive, but also in simple glaucoma,
involving periºphlebitis, endo-phlebitis and sometimes thrombosis, so that
the venous exits are frequently narrowed and sometimes obliterated (Birn-
bacher and Czermak, 1885–86; Birnbacher, 1890; Elschnig, 1896; Heer-
fordt, 1911–12; Hussels, 1912; Hanssen, 1918; and others).
The retina shows degenerative changes as soon as hypertension becomes
established. The first evidences are seen in the ganglion cells, which become
p. 2402.

ANOMALIES OF THE INTRA-OCULAR PRESSURE 3345
degenerated and vacuolated, a change followed by the appearance of cystic
degeneration,' first at the macula, plentifully at the ora serrata and
º
Fig. 2838–PERIvascular INFILTRATIos Is GLAucoma (Evans, Brit. J. O.).
eventually indiscriminately (Schreiber, 1906; Hanssen, 1918; Melanowski,
1923; Verhoeff, 1925; Castroviejo, 1931). The rods and cones become
Fig. 2839.-ABsolute GLAucovia.
Showing atrophy and the migration of pigment into the retina (Hepburn, T. O. S.).
matted together and lie flattened almost parallel to the surface (Bernstein,
1900; v. Hippel, 1901), while the vessels show advanced sclerosis and
1 p. 2754.



4 L 2
3346 TEXT-BOOK OF OPHTHALMOLOGY
hyaline degeneration (Gilbert, 1915–19;
Hanssen, 1918; and others).
The pigment epithelium remains unchanged for a considerable time, but
Fig. 28.40.-Normal FILTRATION ANGLE.
Fig. 2841.-PERIPHERAL ANTERior SYNECHLA-
In glaucoma of 4 months' standing (x 25) (Parsons).
eventually also undergoes
degenerative changes, the
pigment migrating into the
retina (Fig. 2839); defects
appear, among which the
most obvious is around the
optic disc forming the cir-
cum-papillary glaucomatous
halo, which to a certain
extent thus resembles the
circum-papillary atrophy of
senile eyes (Elschnig, 1928).
The angle of the anterior
chamber in glaucomatous
eyes has excited a great
deal of interest since the
importance of its occlusion
in the aetiology of glaucoma
was suggested by Knies
(1876) and Weber (1877), and
a vast amount of investiga-
tion has been expended upon
it (Brailey, 1880; Priestley
Smith, 1882–91; Birnbacher
and Czermak, 1885–86; and
many others). In a great
many cases, particularly in
congestive conditions, the
base of the iris is adherent to
the posterior surface of the
cornea, so that the angle is
closed and the aqueous is
prevented from reaching the
canal of Schlemm. The
generally accepted view of
the pathology of the process
is that during a congestive
attack the base of the iris is
pushed forwards against the cornea, first narrowing and then obliterating
the angle, and eventually, as the result of a plastic exudative process, the


ANOMALIES OF THE INTRA-00ULAR PRESSURE 3347
two become adherent (Fig. 2841). In this way peripheral synechiae are
formed, at first at isolated spots, and ultimately in some cases in an ever-
widening belt until the entire periphery may be involved. As time goes on
and the pressure rises, the two may become firmly compressed together
and the area of adhesion extends (Fig. 2842), and finally, as atrophy and
shrinkage set in, the adhesion is stretched and may even be torn away
from the cornea.
This process occurs most constantly and rapidly in acute congestive
glaucoma, but is rarer in the chronic simple type, particularly in cases
which have never experienced sub-acute attacks in their evolution ; in these
- - --
º ºf " -
- º |
- * -
- º - - º º - -
º º º
Fig. 2842.-ExTENSIVE PERIPHERAL ANTERIoR SYNECHIA.
The iris is almost completely atrophied (x 55) (Parsons).
the peripheral synechiae occur usually at a late stage, are more commonly
partial so that much of the angle remains free, or are absent altogether.
This absence has been verified anatomically (Priestley Smith, 1879), and
has been seen clinically either by the aid of a contact glass or the gonioscope.
These gonioscopic findings are of the greatest significance. Salzmann (1914–15),
who made the first observations, found the angle free in 4 cases of secondary traumatic
glaucoma and partially closed in 3 cases of congestive and 1 case of simple glaucoma.
Troncoso (1925–35) reported on a series of 87 cases: among 26 eyes with acute con-
gestive glaucoma the angle was open in 26%, closed in 26% and partially closed in 46%;
among 8 eyes with chronic congestive glaucoma the angle was open in 25%, closed in
50% and partially closed in 25% ; in 34 cases with chronic simple glaucoma the angle
was open in 58-8%, closed in 5-8%, and partially closed in 38.2%; in 6 eyes with simple
glaucoma with sub-acute exacerbations the angle was partly closed in 50% and closed
in 50% ; while in cases of secondary glaucoma the angle was open in 62%, closed in

3348 TEXT-BOOK OF OPHTHALMOLOGY
23%, and partially closed in 15%. Similarly Thorburn (1927) in 6 cases of congestive
glaucoma found the angle open in 3, and of 9 eyes with simple glaucoma, 6 had an open
angle. Werner (1932) found 39 out of 49 eyes with simple glaucoma with an open
angle. On the other hand, Barkan, Boyle and Maisler (1936) found sclerosis of the
trabeculæ and its blockage with pigment common, and concluded that, while
narrowing of the angle may be an early phenomenon, its blockage by adhesions
occurs only at a late stage.
It would seem therefore than an angle closed by peripheral synechiae is
common after an attack of congestive glaucoma, which, if it is severe, may
result in complete and permanent closure, but that in chronic glaucoma the
Fig. 2843. Pigment in THE FILTRATION ANGLE.
In irido-cyclitis with raised tension following a blow. The angle is infiltrated
with leucocytes and pigment cells (x. 55) (Parsons).
angle is usually open in the early stages and frequently remains so for a con-
siderable time thereafter. The generally accepted opinion of its almost
invariable closure depends on the histological reports of eyes removed in
the latest stages of the disease, when it is indeed common.
In all cases of long-standing, degenerative changes in the trabeculae of the angle
of the anterior chamber are the rule. They include a selerosis and thickening of the
peetinate ligament (Henderson, 1910; Verhoeff, 1915) and a deposition of pigment,
sometimes in considerable quantity in its meshes (Levinsohn, 1908–22; Schieck,
1918; and others) (Fig. 2843). It will be noted presently that, as in the case of peri-
pheral synechiae, some authorities have attributed an aetiological significance to these
changes.
The sclera has frequently been said to be excessively rigid in glaucoma,
so much so, indeed, that an aetiological importance has been attached to

ANOMALIES OF THE INTRA-00ULAR PRESSURE 3349
this factor; it is true that rigidity increases with age, but it would appear
that glaucomatous rigidity is a consequence rather than a cause of the
increased pressure (Friedenwald, 1937). We have already seen that in the
Fig. 2844–INTERCALARY STAPHYLoMA IN ABsolute GLAucoma (Parsons).
Fig. 2845. AN EYE witH ABsolute GLAucoma.
Horizontal meridianal section showing equatorial bulging (Parsons).
eyes of infants the sclera readily and uniformly stretches under the influence
of hypertension so that a buphthalmic globe is produced ; in the fully
developed eye, however, an ectasia is a late result, is only partial, and is


3350 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2846.-Normal. Optic NER ve.
(Weigert’s stain : Parsons.)
Fig. 2847. EARLY GLAucoºtarous ATRoPHY.
(Weigert’s stain : Parsons.)


ANOMALIES OF THE INTRA-OOULAR PRESSURE 3351
probably largely due to ischaemic degeneration. Such staphylomata may
appear in the later stages of absolute glaucoma, at first small and localized,
and eventually, under the influence of continued pressure, becoming confluent
and even annular. Two main types occur, ciliary staphylomata and
intercalary staphylomata" (Birnbacher and Czermak, 1885–86). In the
former the sclera covering the ciliary region becomes stretched, and the
thinned-out ciliary body lines the inner surface of the bulge ; in the latter
the sclera anterior to the ciliary body suffers distension, and since peripheral
synechiae are usually well marked at this advanced stage of the disease, the
adherent root of the iris lines the staphyloma from the anterior edge of
which the iris appears to come (Fig. 2844). Equatorial staphylomata are
Fig. 2848-ADvancED GLAU contatous CUPPING (Parsons).
rare, and usually develop in the neighbourhood of a vortex vein (Fig. 2845).
All of them show histologically an extremely attenuated sclera, which forms
a thin membrane, on the inner surface of which the stretched and atrophied
uvea is recognizable only as a pigmented line. A spontaneous rupture is a
pathological curiosity (Stölting, 1888; Knape, 1910; Meller, 1918).
The optic nerve shows pathological changes so constantly in all forms
of chronic glaucoma that the cupped appearance of the disc is one of the
primary diagnostic features of the disease. The changes were first described
by H. Müller (1856–58) and comprise two essential features—an atrophy
of the nerve-fibres and an ectasia of the disc. The atrophy of the nerve-
fibres commences at an early stage, and is most marked at first at the
* p. 2046.

3352 TEXT-BOOK OF OPHTHALMOLOGY
temporal side (Figs. 2847 and 2848), but ultimately involves the entire
nerve. The ectasia starts with the formation of a concavity by the
lamina cribrosa, which gradually travels back beyond the level of the
sclera, so that the scleral ring thus forms the lateral edge of the cup and
eventually constitutes a prominent overhanging lip jutting out over the
excavated nerve-head. As a general rule the cup is empty and is filled with
vitreous; but sometimes its surface is covered with proliferated neuroglial
tissue or connective tissue, which in exceptional cases may even fill it and
project into the vitreous cavity (Römer, 1901 ; Behr, 1914).
The cause of the formation of the optic cup is by no means clear.
Originally it was generally accepted that the ectasia was a mechanical
pressure-effect by which a hernia was produced at the weakest spot in the
ocular coats, while the nerve atrophy was also regarded as a secondary result
of pressure, partly owing to degeneration in the ganglion cells and partly
owing to a combination of the effects of pressure and stretching as they pass
over the rim and sides of the cup at the nerve-head (Müller, 1856;
Birnbacher and Czermak, 1885–86 ; Stock, 1908–27 : Fuchs, 1916 :
Kapuscinsky, 1930 ; and others). That this mechanical influence
undoubtedly enters into the question is seen in the fact that an excavation
can be produced in the dead eye by raising the intra-ocular pressure (Laker,
1886; Birnbacher and Czermak, 1886), or, conversely, by suddenly
decreasing the intra-cranial pressure (v. Szymansky and Wladyczko, 1925).
It is suggested also by the diminution in the depth of the cup, which has
been noted after the relief of hypertension by operation (v. Graefe, 1869), and
even by miotics (Lange, 1912). Fuchs (1911–16) explained the differences
in the appearance and depth of various cups, particularly those in the
formation of which a raised pressure has been slight, by variations in the
anatomical configuration of the supportive tissue of the nerve-head ; the
lamina itself may be far forward or far back, while in some eyes the supportive
tissue is made up essentially of strong transversely running laminae, and in
others of frailer bundles of glial tissue running longitudinally. From
histological studies he concluded that the sequence of events was first a
disappearance of the delicate anterior glial fibres and then of the deeper
fibres incorporated in the lamina ; this is followed by the bending backward
of the connective tissue lamellae, which at first became sclerosed and then
thinner and atrophic and eventually broken up, sometimes with the forma-
tion of spaces, under the influence of continued pressure. While the
supporting framework of the intra-ocular portion of the nerve is undergoing
these changes, pressure-atrophy begins to be apparent in the nerve-fibres.
He suggested that this may occur extensively at an early stage when only
the anterior glial fibres have disappeared and the connective tissue lamina
is still in a normal position, producing the clinical appearance of an
increased physiological cup with atrophy. On the other hand, when the
lamina is weak it may yield while the nerve-fibres are still intact, producing
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3353
a deep cup with little field defect, and in extreme cases it may yield before
a normal intra-ocular pressure.
Schnabel (1892–1908), however, put forward the opposite view, claiming
that the primary process is a neuritic atrophy, the degeneration of the nerve
fibres leading to the formation of small empty spaces which finally coalesced
(CAVERNous DEGENERATION) (Fig. 2849): he claimed that these lacunae
appear at a very early period before any excavation of the disc is visible.
Fig. 2849.-CAvernous ATRoºhy IN GLAucoma (Evans, Brit. J. O.).
As a compensatory process, a proliferation of interstitial connective tissue
occurs which becomes vascularized and then contracts, so that the lamina
is not pushed back by the intra-ocular pressure but pulled back by the
shrinking connective tissue of the atrophic nerve. He pointed out that the
cavernous degeneration is very apparent in the region of the lamina, where
by the coalescence of clefts the lamina itself is exposed, and eventually a
single large cavern results in this situation—the glaucomatous excavation.
His views were to a large extent confirmed by many observers (Elschnig,
1895–1928; Hümelsheim and Leber, 1901; Schnaudigel, 1904; Evans,
1939; and others), but it is to be remembered that this cavernous atrophy
is by no means specific for glaucoma.”
1 p. 3070. * p. 3070.

33.54 TEXT-BOOK OF OPHTHALMOLOGY
Lagrange and Beauvieux (1925) on the other hand, considered the
changes in the nerve secondary to vascular changes which were themselves
secondary to pressure. They found that the cup terminated in a deep
conical projection penetrating backwards alongside the vessels, a process
which caused vascular obliferation and sclerosis which resulted in neuro-
retinal atrophy. It would seem, however, that although the blood-vessels
of the nerve always show marked sclerosis in cases of advanced glaucoma,
the blood-supply of the lamina and the anterior third of the optic nerve is
derived from branches of the posterior ciliary arteries without help from the
central artery of the retina (Behr, 1937). It may well be that the factors
of capillary stasis and congestion allow the accumulation of waste-products,
while sclerosis of the nutrient arteries brings on an ischaemic degeneration,
either process resulting in a weakening of the tissues and the formation of a
staphyloma just as occurs in the anterior segment of the sclera in terminal
conditions of long-standing pressure. In addition to the ischaemic factor,
Elschnig (1924) suggested a possible histiolytic influence of a toxic intra-
ocular fluid being forced into the nerve-head. Such a conception brings the
glaucomatous excavation into line with the similar appearance of cupping
associated with typical field-defects which we have already discussed as
occurring in arteriosclerotic conditions (Knapp, 1928; Hofe, 1929; Kurz,
1935; Reid, 1937; and others), and explains the occurrence of optic atrophy
with cupping without the incidence of increased tension (Pickard, 1925–31 ;
Nielsen, 1937) and its progress in glaucomatous cases after the relief of
tension. Whether these cases should be considered glaucomatous at all is,
of course, a most difficult question, and it would seem most suitable that
v. Graefe’s (1857) original suggestion “amaurosis with eaccavation of the
disc " or the term pseudo-glaucoma is more suitable.”
Although with our present knowledge an absolute decision is impossible,
probably both factors, mechanical pressure and ischaemic atrophy, enter
into the question, the relative importance of either varying much from case
to case : the question merely seems to stress the impossibility of considering
glaucoma as a single disease-entity conditioned by a uniform pathology.
Baas. K. M. Aug., xxxviii, 417, 1900.
Barkan, Boyle and Maisler. Am. J. O., xix,
209, 1936.
Bartels. Z. f. Aug., xiv, 458, 1905.
Behr. K. M. Aug., lii, 790, 1914.
A. f. O., lxxxix, 265, 1915; czXxiii, 227,
1937. -
Bernstein. A. f. O., li (1), 186, 1900.
Bietti. K. M. Aug., xlvi (1), 337, 1908.
Birnbacher. Fest. d. K. K. Univ. Gratz, iii,
1890.
Birnbacher and Czermak. A. f. O., xxxii (2),
1, 1885; (4), 1, 1886.
Brailey. R. L. O. H. Rep., x, 10, 1880.
Castroviejo. A. of O., v., 189, 1931.
Courtis. A. de Oft. B. A., viii, 399, 1933.
Elschnig. B. O. G. Heidel., xxiv, 149,
1895.
A. f. Aug., xxxiii, Erg., 187, 1896.
K. M. Aug., x1 (2), 81, 1902; lxxviii, 93,
1927.
A. f. O., lxxiii, 126, 1908; cxx, 94, 1928.
2. f. Aug., lii, 287, 1924.
Evans. Bråt. J. O., xxiii, 745, 1939.
Friedenwald. A. of O. iii, 560, 1930.
Am. J. O., xx, 985, 1937.
Fuchs. A. f. O., xxvii (3), 66, 1881 ;
197, 1916 : xci, 435, 1916.
T. O. S., xxii, 15, 1902.
Z. f. Aug., xxv, 108, 1911.
xcii,
1 p. 3071.
ANOMALIES OF THE INTRA-OCULAR PRESSURE
3355
Gilbert. A. f. O., lxix., 1, 1909; lxxxii, 389,
1912; xc, 76, 1915.
4. f. Aug., lxxxv, 74, 1919.
v. Graefe. A. f. O., ii (1), 206, 1853; iii (2),
456, 1857; xv (3), 118, 1869.
Hanssen. K. M. Aug., lxi, 509, 1918.
Heerfordt. A. f. O., lxxviii, 413, 1911 ;
lxxxiii, 149, 1912.
B. O. G. Heidel., xxxix, 322, 1913.
Henderson. Glaucoma, London, 1910.
v. Hippel. A. f. O., lii (3), 498,
lxxiv, 101, 1910.
1901;
Hofe. A. f. Aug., c-ci, 414, 1929.
Hümelsheim and Leber. A. f. O., lii (2),
336, 1901.
Hussels. Z. f. A ug., xxvii, 213, 354, 1912.
Kapuscinsky. A. d’O., xlvii, 779, 193().
Knape. Finska läk.-săllsk Handl., lii, 547,
1910.
Knapp, A. A. of O., lvii, 219, 1928.
Knies. A. f. O., xxii (3), 163, 1876; xxiii (2),
62, 1877.
Eurz. A. f. Aug., ciz, 108, 1935.
R. M. Aug., xciv, 703, 1935.
Lagrange and Beauvieux. A. d’O., xlii, 129,
1925.
Laker. K. M. Aug., xxiv, 187, 1886.
Lange. K. M. Aug., l (2), 540, 1912.
Latham. Med. J., Australia, 293, 1938.
Leber. A. f. O., xxiv. (1), 257, 1878.
Levinsohn. A. f. Aug., lxii, 131, 1908.
Z. f. Aug., x1, 344, 1918.
R. M. Aug., lxi, 174, 1918; 1.xviii, 471,
Newolina. K. M. Aug., xcvi (2), 360, 1908.
Nielsen. Acta O., xv., 151, 1937.
Pickard. Brit. J. O., ix, 385, 1925; xv. 323,
1931.
Redslob. An. d’Oc., clxxii, 1, 1935.
Bull. S. fr. d’O., xlix, 145, 1936.
Reid. Brit. J. O., xxi, 361, 1937.
Römer. A. f. O., lii (3), 514, 1901.
Rönne. K. M. Aug., li (2), 505, 1913.
Salzmann. Z. f. Awg., xxxi, l, 1914; xxxiv,
26, 160, 1915.
A. f. O., czzxix, 413, 1938.
Schieck. K. M. Aug., lxi, 332, 1918.
B. O. G. Heidel, l, 69, 81, 1934.
Schnabel. A. f. Aug., xxiv, 273, 1892.
Z. f. A ug., xiv., 1, 1905; xix, 558, 1908.
Schnandigel. A. f. O., lix (2), 344, 1904.
Schreiber. A. f. O., lxiv, 237, 1906.
Sgrosso and Antonelli. A. di Ott., xix, 166,
1890.
Smith, Priestley. R. L. O. H. Rep., x, 25,
1882.
Glaucoma, London, 1879, 1891.
Stock. K. M. Aug., xlvi (1), 342,
lxxviii, Beil., 61, 1927.
Stölting. A. f. O., xxxiv. (2), 135, 1888.
v. Szymanski and Wladyczko. Kl. Oz., iii,
145, 1925.
Talbot. Brit. J. O., xxii, 210, 1938.
Thorburn. Diss., Stockholm, 1927.
Troncoso. Am. J. O., viii, 433, 1925; xviii,
103, 1935.
T. O. S., liii, 366, 1933.
1908
1922. A. of O., xiv, 557, 1935.
Mathewson. Am. J. O., xv, 139, 1932. Verhoeff. A. of O., xliv, 129, 1915; liv, 20
Melanowski. Kl. Ocz., i, 24, 1923. 1925. y
Meller. K. M. Aug., lx, 458, 1918. Weber. A. f. O., xxiii (1), 1, 1877.
Müller, H. Gesam. Schriftem, Leipzig, i, 340, Werner. Acta O., x, 427, 1932.
1856. Wolff and Lyle. P. R. S. Med., xxx, 391
A. f. O., iv (2), 1, 1858. 1937. 5
Aºtiology
In so far as we are using the term primary glaucoma as the description
of a symptom common to several morbid conditions of unknown
pathology, it is understandable that any summary of its aetiology must
still remain vague. One thing, however, is certain—that conditions
of “ primary ‘’’ ocular hypertension are not all due to the same cause,
and the many attempts in the literature to ascribe to the condition a single
aetiology have met with no success. In our discussion of secondary glaucoma
we have seen that the two factors which run through its many manifestations
are (1) a vascular disturbance and (2) some obstruction to the circulation and
drainage of the intra-ocular fluid, and that of the two the first is the primary
factor and the second is adjuvant. It would seem most probably the case
that the same two factors dominate the development of primary glaucoma.
The most fundamental change is a vascular instability or inefficiency, which
when it involves an acute stasis may determine the onset of a congestive
3356 TEXT-BOOK OF OPHTHALMOLOGY
glaucoma, and when less dramatic changes occur, may determine the
evolution of a simple glaucoma. Such a vascular crisis or prolonged
inefficiency may be compensated in a healthy eye ; but if the circulation
of the aqueous is already impaired by sclerotic or other pathological
changes, a rise of tension will become evident more easily : a contributory
factor of the greatest importance, therefore, is any embarrassment to the
circulation of the intra-ocular fluid.
It cannot be pretended, however, that the whole of the aetiology of glaucoma
lies in these two conceptions ; there is certainly a more complex and
subtle mechanism involved about which we are almost completely ignorant.
This opens up the large and vague question of the maintenance of physiological
values at a constant level (Elwyn, 1938). This is a conception of vital signi-
ficance about which physiologists have as yet little knowledge, but it applies
to all organs and functions, the activities of which are controlled and main-
tained by a regulatory mechanism in which the main factors appear to be
chemical, neurogenic and hormonal. In this view, glaucoma holds analogies
with other diseases wherein adequate regulation fails, such as hypertension and
diabetes. Nor is the whole story of the local pressure-equilibrium of the eye
encompassed in the facts of capillary permeability and circulation of fluid,
for between the capillary wall and the chambers of the eye the fluid has to
traverse further membranes which form a tissue-aqueous barrier. It will be
remembered that multi-layered membranes line most of the interior of the
eye–Bowman's membrane and the corneal endothelium with its endothelial
prolongation across the angle of the anterior chamber, and Bruch's membrane
and the retinal epithelium with their prolongations over the ciliary region
and the posterior surface of the iris—the entire inner surface of the globe,
in fact, except the anterior surface of the iris, and it may be significant that
this structure is an early sufferer in the atrophy and sclerosis of glaucoma
(Duke-Elder, 1938). It is known that the passage of water and salts through
such multi-layered membranes involves obedience to complex laws. The
fundamental work of Wertheimer (1923–25) on the permeability of mem-
branes has shown that their activity is influenced by differences in the
surface electrical charges and in the hydrophilic swelling of the proteins on
either side of the membrane, so that some degree of uni-directional
permeability and the capacity for maintaining concentration-gradients
exists. F. P. Fischer (1930) has shown the existence of such factors in the
cornea and Friedenwald and Stiehler (1939) in the ciliary region ; but about
their intimate mechanism and their derangements we know nothing. It is
possible—indeed probable—that these two factors—the complex systemic
control of the normal physiological values, and subtle local changes in the
mechanism of ocular permeability—have much to do with the aetiology of
glaucoma. The point must again be stressed that primary glaucoma is not
a local disease of the eyes, but seems more probably the ocular complication
of some constitutional disturbance still unknown ; nor is the rise of tension
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3357
the primary pathological change, for, although it may be the dominant
feature, it is merely a symptom of some deeper and more subtle defect.
The glaucomatous eye is not merely a hypertonic but a diseased organ,
sharing its illness with the rest of the body : neither do the clinical
symptoms, the pain, the pathological changes, or the functional disabilities,
run parallel with the tension.
THE VASCULAR BASIS
(a) The Capillaries. It would seem that almost every case of glaucoma
is associated with a disturbance of the capillaries, involving stasis. In the more
chronic non-congestive cases the disturbance is mild, either one of sclerosis
or of vaso-neurosis. The first, as we have just seen, is almost universal in
chronic glaucoma. The evidence for the influence of the second factor in
other cases has already been discussed when summarizing the large literature
dealing with the functional instability of the capillary circulation throughout
the body and in the eye of such subjects, their lack of neuro-vegetative tone,
their endocrine imbalance, and their psychotic tendencies. Different
authors lay stress upon various factors; to some the primary fault is an
endocrine imbalance (Imre, 1921–24), to others a diminution of sympathetic
tone (Hamburger, 1924–25), to others an increase (Thiel, 1926), and to others
a defective regulation (Dieter, 1928). In no case has our knowledge advanced
sufficiently to give a reasoned opinion. In all cases the important feature is
a failure in the integrity of the endothelial function which ultimately dominates
all questions of the maintenance of the fluid-balance; and, as we have noted,
it is difficult to say how closely this function, with its chemical, electrolytic,
osmotic, and oxidative control, runs parallel with the effectivity of the
complex membrane forming the tissue-aqueous barrier.
The more acute congestive attacks in many cases are probably con-
ditioned by the same mechanism, and in these the functional derangements
are to be especially stressed ; and once capillary function has become
disorganized, the spread of vascular disturbances throughout the entire uveal
fract through the medium of aaºon reflexes probably plays a dominating rôle in
the production of acute glaucoma. It has been shown experimentally
that a localized lesion, probably through the production of histamine-like
substances, results in the development of a generalized vascular crisis
involving a dilatation of the small vessels throughout the eye, a phenomenon
which, although parallel to the histamine flare of the skin, is seen in no other
internal organ of the body (Duke-Elder, 1931). Such a reaction, producing
an extreme capillary dilatation, a high capillary pressure, an intense degree
of stasis, and resulting in the production of a protein-rich plasmoid aqueous,
could account for most types of secondary glaucoma and the congestive
attacks and acute strangulation of primary glaucoma ; moreover, a
histamine-like substance has been isolated from the aqueous in acute
glaucoma (Friedenwald, 1930) and in the high tension of dropsical glaucoma
3358 TEXT-BOOK OF OPHTHALMOLOGY
(Kirwan, 1936), while the pathological picture of an experimental glaucoma
produced in this way duplicates that seen in the clinical condition (Frieden-
wald, 1930). Once this state has developed, the circulatory stasis will
determine the formation and allow the accumulation of additional waste-
products and histamine-like substances which will accentuate and perpetuate
the crisis, establishing a vicious circle, the outcome of which is complete
strangulation.
(b) The Veins. In addition to those factors associated with the
capillaries, a venous-capillary stasis may be brought about by some local
obstruction to the venous circulation such as the perivascular infiltrations
so frequently seen in glaucomatous eyes. This view of the importance of an
obstructed venous circulation was first promulgated by Stellwag v. Carion
(1874) and has received much support, either as affecting the choroidal
vessels, which almost invariably show sclerotic changes even in the early
stages of the disease (Magitot and Bailliart, 1921–25), the anterior ciliary
veins (Bartels, 1905), or the vortices (Birnbacher and Czermak, 1885–86).
As we have already seen, changes are frequently found in association with
the latter, involving peri-phlebitis, extensive endothelial thickening, and
thrombosis, so that the lumen is frequently narrowed and sometimes
obliterated. In the causation of such an obstruction Sondermann (1934)
would add the factor of increased sclerosis of the sclera, while Schieck
(1934) found histological evidence that even when the vein itself was
structurally unimpaired, it could be mechanically compressed almost to
obliteration in the oblique scleral canal. Similarly Salzmann (1914–15)
described how the central vein of the retina may be narrowed by prolifera-
tive changes so that it became practically occluded. It is obvious that
obstructions of this type, if chronic, could produce a condition of sustained
capillary stasis, and if acute, a state of maximal congestion resulting in the
development of a glaucoma similar to that associated with uveal tumours or
orbital lesions.
CONTRIBUTORY FACTORS
(a) Obstruction to the Eait of the Intra-ocular Fluid. Ever since the
discovery by Leber (1873) of the preferential channel of exit of the intra-
ocular fluid at the angle of the anterior chamber, and the demonstration of
the closure of this angle in glaucoma either by inflammatory processes
(Knies, 1876) or by a swelling of the ciliary body (Weber, 1877), this factor has
always assumed pride of place in any discussion of the aetiology of glaucoma,
being firmly established in medical thought by the work of Priestley Smith
(1879). In a previous volume we have already detailed the evidence
proving the existence of a current through the pupil finding an exit
from the eye preferentially by this route : the facts that the capillary
pressure in the ciliary region is particularly high (Duke-Elder, 1926) and the
permeability of the capillaries here peculiarly great (Friedenwald and
ANOVIALIES OF THE INTRA-OCULAR PRESSURE 3359
Stiehler, 1939), and that the canal of Schlemm, with its endothelial wall
through which fluid-traffic can take place with the same ease as in the case
of the capillaries, is situated far down the venous pressure-gradient, necessitate
such a current without other postulates. It will be remembered also that
the canal of Schlemm with its safety-valve mechanism can to a large extent
neutralize abnormal rises of pressure by increasing its efficiency ; indeed,
Friedenwald and Pierce (1932) concluded that it readily accommodated 5 to
10 times the normal outflow if the pressure of the eye were raised by 3 to
10 mm. Hg. It is obvious, therefore, that by its cushioning effect, an efficient
drainage system at this point can largely neutralize rises of intra-ocular pres-
sure and thus prevent the development of glaucoma; it is equally obvious that
if it is embarrassed or inefficient, small rises of pressure will tend to become
cumulative and acute rises disastrous, so that a clinically apparent glaucoma,
in the first case chronic, in the second acute, will readily develop ; and it
will be understood that in an established glaucoma, a re-opening of this
channel or the formation of a substitute by operation will relieve the tension
and, so long as it is efficient, keep in check—but not cure—the glaucoma.
Various theories have been put forward to explain the embarrassment to
filtration :-
1. A mechanical narrowing of the angle followed by the development of
peripheral synechiae between the root of the iris and the cornea, eventually
shutting it off completely, constitutes the classical theory. In chronic
glaucoma Bowman (1862) first suggested that the decisive factor in this
process was a pushing forwards of the ciliary body and iris by a relatively
large lens in the typically small glaucomatous eye, a suggestion elaborated
by Priestley Smith (1879) in much detail by statistical evidence of the gradual
increase in size of the lens in age and the smallness of the eye in glaucomatous
subjects. In acute glaucoma, as we have seen, the formation of anterior
synechiae by the congested and swollen ciliary body is a much more obvious
fact (Knies, 1876; and many others). It will be remembered, however,
that, as we have already pointed out, in the early stages of glaucoma these
synechiae are by no means invariably found ; indeed, while their presence
will necessarily accentuate any glaucomatous process, they are essentially
characteristic of the later stages of the disease, and are more probably a
result of the initial disturbance than its cause.
2. A primary sclerosis and thickening of the pectinate ligament due to
age (Henderson, 1910; Verhoeff, 1915).
3. The deposition of pigment in the meshes of the pectinate ligament—a
very frequent occurrence in glaucomatous eyes (Levinsohn, 1908–22 ;
Koeppe, 1916–20 ; Schieck, 1918; Gradle, 1921 ; Barkan, Boyle and
Maisler, 1936). It is to be remembered, however, that pigmentary deposits
of this nature are a constant occurrence in senile eyes 1 and are found in
plentiful masses in such conditions as diabetes, pigmentary degeneration
1 p. 241S.
T. O. — WOL. III. 4 M
3360 TEXT-BOOK OF OPHTHALMOLOGY
of the retina and retinal detachments, without affecting the tension (Trantas,
1935); indeed, it would seem probable that they are the result of the
sclerosis and atrophy of the iris associated with glaucoma rather than its
Cà, UISé.
4. The deposition of flakes from the lens capsule in the trabeculae of the
pectinate ligament has been held by some authors to be a common cause of
primary glaucoma ; this we have discussed under the heading of Secondary
Glaucoma.
5. Abnormalities in the endothelium of Schlemm's canal rendering it
relatively impermeable (Seidel, 1927).
6. A defective inward pull of the ciliary muscle largely determined by
anatomical peculiarities so that the suction action of the scleral spur upon
the canal of Schlemm cannot be adequately effected (Herbert, 1923–29 ;
Fortin, 1929).
7. Friedenwald (1936) suggested that the resorption of aqueous might be
hindered owing to failure of osmotic pull from Schlemm's canal due to a
sclerosis of the afferent vessels preventing the entrance therein of plasma.
Many of these anatomical changes have been considered the primary
causes of glaucoma in the past (Priestley Smith, 1879; Seidel, 1918–24 ;
and others), but the evidence is that most of them are collateral or consecu-
tive processes; there can be no doubt, however, that their presence will
predispose to the incidence of glaucoma in eyes already likely to be affected
so that they constitute exciting causes, or that they will aggravate and main-
tain the disease once it has already developed, thus acting as contributory
Cà, UISéS.
(b) A loss of elasticity of the sclera due to the sclerosis of age has been
cited as an important factor in the determination of chronic glaucoma
(Kuschel, 1908). The elasticity of this structure is, however, so small that
it cannot exert any great influence except in so far as its sclerosis or
turgescence may embarrass the filtration channels at the angle of the
anterior chamber or may compress the vortex veins as they travel through
it (Stransky, 1912–13; Bourgeois, 1919; Kadlicky, 1922; Sondermann,
1929–34).
OTHER THEORIES
Hypersecretion. v. Graefe (1856) first assumed that glaucoma was due to the
hypersecretion of intra-ocular fluid owing to a serous choroiditis, a view modified by
Donders (1862) who held that the cause of the excessive secretion was nervous irritation
of the choroid. Following his teaching a number of observers have attributed glaucoma
to an over-Secretion by ciliary “glands '' under the stimulus of trigeminal (Mooren,
1882; v. Hippel and Grünhagen, 1868–70) or sympathetic irritation (Abadie, 1897–
1923; and others), or owing to general influences (Hill and Flack, 1912); but for this,
or even the existence of a secretory process as an essential factor in the fluid-traffic of
the eye, there is no satisfying evidence (Duke-Elder, Quilliam and Davson, 1940).
Swelling of the Vitreous Body. The compression of the vascular tissues of the
ANOMALIES OF THE INTRA-OCULAR PRESSURE 336]
eye and the pushing forwards of the iris and lens in glaucomatous eyes have suggested
to many investigators that swelling of the vitreous body may be a determining factor
in the aetiology of glaucoma. The first work done on this subject was by M. H. Fischer
(1908–09) and Thomas and Fischer (1910), who, on immersing excised eyes in acids,
generated a pressure sufficient to burst the sclera ; this Fischer explained as an
Cedematous swelling of the vitreous determined, as Oedema frequently is in other
tissues, by a disturbance of the acid-base equilibrium. The subsequent observations
of Knape (1909), v. Fürth and Hanke (1913), Ruben (1914), Nakamura (1926), Heesch
(1926) and F. P. Fischer (1930), however, have demonstrated that the high pressures
generated were due to a swelling of all the other ocular tissues, mainly a turgescence of
the sclera itself, thereby causing a great diminution of the contents of the globe. More-
over, the concentrations of acid used by Fischer were far in excess of physiological
variations; and in clinical conditions of acidity, as in diabetic coma, the tension of
the eye tends to be low. Finally, it is found experimentally that both the isolated
proteins of the vitreous (Duke-Elder, 1930), and the vitreous gel in vitro (Meesmann,
1924; Redslob, 1928; Lobeck, 1929; Duke-Elder, 1930) show a decrease of volume
in an acid reaction and an increase in an alkaline reaction, a result which has been
corroborated in the perfused experimental animal (Duke-Elder, 1931).
Interest in a possible turgescence of the vitreous owing to increased alkalinity
was stimulated by these experiments, more particularly when an increased alkalinity
of the blood of glaucomatous patients was reported by Baurmann (1924) and Meesmann
(1925) and of the vitreous itself by Gala (1925). Subsequent studies, however, on the
reaction of the blood and the calcium-potassium quotient of such patients have, as
we have seen,” produced negative or varying results, while Schmelzer (1927) and
Wegner and Endres (1928), after raising the pH of the blood by over-ventilation of the
lungs and by the exhibition of large quantities of sodium bicarbonate, failed to produce
any alteration in the pressure of the eye. The conclusion must therefore be accepted
that excessive alkalinity has no aetiological significance in this disease. It is true
that the vitreous gel is capable of turgescence, and it has been shown that swelling
does occur to a small extent in alkaline reactions (Duke-Elder, 1926–30 ; Duke-Elder,
Davson and Robertson, 1934; Duke-Elder and Davson, 1935); but the fact that the
maximum swelling pressures which can be generated both in the normal and glauco-
matous vitreous are of the same order and exceedingly small—-1 to 2 mm. of saline
(Duke-Elder, Davson and Benham, 1936)—rules out of court any influence of the
vitreous in the aetiology of pathological rises of tension. •
Other suggestions as to the possible mechanism of a swelling of the vitreous have
been made—an alteration of the permeability of the hyaloid membrane, an increase
in its protein content (Raeder, 1924; Nordensen, 1924–26), or a retention of mucin
(Verhoeff, 1925).
Osmotic Theories. The general osmotic concentration of the body was assumed
to be at fault in glaucoma by Cantonnet (1904), who concluded that the raised ten-
sion was due to an osmotically determined Oºdema. Somewhat similar views were
suggested by Hertel (1913); but we have already seen * that the blood of glaucomatouš
patients shows no constant osmotic deviation. Osmotic changes in the intra-ocular
fluid have also been associated with rises of tension, particularly a rise in the protein
content, which, by raising the osmotic pressure of the aqueous to approach that of the
blood, allows the intra-ocular pressure to approximate to the capillary pressure. This
reaction undoubtedly occurs (Duke-Elder, 1927), and to this consideration may be added
an embarrassment of filtration owing to the colloids in the plasmoid aqueous formed in
cases of capillary dilatation. It may, indeed, play a considerable part in the deter-
mination of the rise of tension in secondary and congestive glaucoma, but its action
1 p. 3334. * p. 3335.
4 AI 2
3362 TEXT-BOOK OF OPHTHALMOLOGY
is secondary to the vascular changes ; and although it has been claimed that the
aqueous in simple glaucoma contains a high concentration of protein (Troncoso,
1901–10), this is not a uniform finding (Dieter, 1925; Magitot, 1931; Hodgson, 1938).
Toacaemia. That glaucoma is the expression of a general toxaemia has been
suggested by several authors (Bjerrum, 1912; Orr, 1914; Sattler, 1916; Kerry,
1925; Davis, 1928; and others). A toxaemia may well produce a vasomotor
instability, but there is no evidence that it acts primarily on the pressure of the eye.
In summary, therefore, it may be said that our position as to the
aetiology of primary glaucoma, as I conceive it, is that at least four elements
may figure in it, two of which are problematical and largely hypothetical,
and two of which are certain.
1. A disturbance of the central mechanism regulating the complex:
factors maintaining the tonus of the eye at a relatively constant value. About
this, as about the similar control of all physiological functions, we know
little or nothing, nor can hope to until medicine has progressed much
further.
2. A disturbance of the membrane-system forming the tissue-aqueous
barrier. By a disturbance involving an abnormal uni-directional
permeability, an excessive formation or a lessened elimination of intra-ocular
fluid would result. Although the existence of such a membrane is proved, we
know next to nothing about its normal activities and nothing about its devia-
tions from the normal, but these problems, although difficult, should become
rapidly amenable to physico-chemical research.
3. A disturbance of the venous-capillary circulation, that is, essentially,
of endothelial function (the blood-tissue barrier). Such a disturbance may be
either of a sclerotic or vaso-neurotic character determined by degenerative,
neuro-vegetative or endocrine factors about which our knowledge is still
most indefinite and chaotic.
4. A disturbance of the drainage-system of the intra-ocular fluids. This
is often a contributory factor only, and, being local and relatively gross in
nature, is the influence about which our knowledge is most complete.
It is probable that in some cases all these factors may act in consort,
that in others they may react in varying combinations, or that in yet others
one may exert a dominating influence. It is obvious, therefore, that primary
glaucoma cannot be considered a single disease, but is rather a composite
congeries of clinical entities which we cannot yet disentangle. No one can
complain, however, of the variety, the scope, the interest, or the difficulty
of the problems awaiting future research.
_Abadie. A. d’O., xvii, 375, 1897. Birnbacher and Czermak. A. f. O., xxxii (2),
La Clin. Opht., xii, 303, 1923. 1, 1885; xxxii (4), 1, 1886.
Barkan. A. of O., xv, 101, 1936. Bjerrum. K. M. Aug., l (1), 42, 1912.
Barkan, Boyle and Maisler. Am. J. O., xix, Bourgeois. K. M. Aug., lxiii, 768, 1919.
209, 1936. Bowman. Brit. Med. J., i, 377, 1862.
Bartels. Z. f. Aug., xiv., 103, 258, 458, 1905. Cantonnet. A. d’O., xxiv, 193, 1904.
Baurmann. A. f. O., cziv, 276, 1924. v. Carion, Stellwag. A. f. O., ii (2), 207, 1874.
Biffis. Am. di Ott., lxi, 109, 1933. Davis. Am. J. O., xi, 335, 1928.
ANOMALIES OF THE INTRA-OCULAR PRESSURE
33.63
Dieter. A. f. Aug., xcvi, 8,
xcix, 678, 1928.
Donders. A. f. O., viii (2), 160, 1862.
Duke-Elder. Brit. J. O., x, 513, 1926.
J. Physiol., lxi, 409, 1926; lxiv, 78, 1927;
lxxi, 268, 1931.
The Nature of the Vitreous Body, Brit. J. O.,
Supp., 1930.
P. R. S. (B)., ciz, 19, 1931.
T. O. S., liii, 281, 1933 : Iviii, 99, 1938.
Duke-Elder and Davson. Biochem. J., xxiv,
1121, 1935.
Brit. J. O., xix, 133, 1935.
179, 1925;
Duke-Elder, Davson and Benham. Brit.
J. O., xx, 520, 1936.
Duke-Elder, Davson and Robertson.
Biochem. J., xviii, 433, 1934.
Duke-Elder, Quilliam and Davson. Brit.
J. O., xxiv, 1940.
Elwyn. A. of O., xix, 986, 1938.
Fischer, F. P. A. f. Aug., c-ci, 146, 480,
1930.
Fischer, M. H. A. ges. Physiol., czziv, 69 ;
cxxv, 99, 396, 1908; crxvii, 1, 46,
1909.
Fortin. Semana Med., ii, 209, 1929.
Friedenwald. Am. J. O., iii, 560, 574, 1930 ;
xvi, 64, 1936.
Friedenwald and Pierce. A. of O., viii, 9,
1932.
Friedenwald and Stiehler. A. of O., xx, 761,
1939.
v. Fürth and Hanke. Z. f. Aug., xxix, 252,
1913. -
Gala. Brit. J. O., ix, 516, 1925.
Cas. lek. cesk., lxiv, 409, 1925.
Gradle. Am. J. O., iv, 428, 672, 1921.
v. Graefe. A. f. O., ii (1), 248, 1856.
Hamburger. Med. kl., xx, 274, 1924.
D. med. W., li, 186, 1925.
Heesch. A. f. Aug., xcvii, 546, 1926.
Henderson, T. Glaucoma, London, 1910.
Herbert. Brit. J. O., vii, 469, 1923 ; xiii,
289, 337, 1929.
T. O. S., xlv, 333, 1925; xlix, 312,
1929.
Hertel. K. M. Aug., li (2), 351, 1913.
Hill and Flack.
1912.
v. Hippel and Grünhagen. A. f. O., xiv. (3),
219, 1868 ; xv (1), 265, 1869; xvi, 27,
P. R. S. (B), lxxxv, 439,
I S7 ().
Hodgson. T. O. S., lviii, 87, 1938.
Imre. A. f. Aug., lxxxviii, 155, 1921.
K. M. Aug., lxxi, 777, 1923; lxxiii, 206,
1924.
Kadlicky. Bratis lav. lek. listy., i, 313,
1922.
Kerry. T. O. S., xlv., 355, 1925.
Kirwan. Brit. J. O., xx, 321, 1936.
FCnape. Skand, A. f. I’hysiol., xxiii, 162,
1909.
Knies. A. f. O., xxii. (3), 163, 1876; xxiii (2),
62, 1877.
Roeppe. B. O. G. Heidel., xl, 478. 1916;
xcii, 87, 1920.
Z. f. Aug., xc, 138, 1918.
A. f. O., ci, 238, 1920.
Ruschel. Z. f. Aug., xix, 45, 97, 193, 426 ;
xx, 423, 1908.
Leber. A. f. O., xix (2), 87, 1873.
Levinsohn. A. f. Aug., lxii, 131, 1908.
Z. f. Aug., xl, 344, 1918.
R. M. Aug., lxi, 174, 1918;
1922.
Lobeck. A. f. O., czkii, 668, 1929.
Magitot. Am. d’Oc., clxvi, 356, 439, 565, 1929.
A. of O., vi, 647, 1931.
Magitot and Bailliart. An. d’Oc., clviii, 81,
1921.
Am. J. O., viii, 761, 1925.
lxviii, 471,
Meesmann. A. f. Aug., xciv, 116, 1924;
xcvii, 1, 1925.
Mooren. Ueber d. Verbreitung d. sym.
Störungen, Wiesbaden, 1882.
Nakamura. A. f. Aug., xcvi, 131, 1926.
Nordenson. Uppsala. Lák. Förh., xxix, l,
1924 ; xxxi, 289, 1926.
Acta O., i, 311, 1924.
Orr. O. Rev., xxxiii, 33, 1914.
Raeder. T'id. f. mors. laeg., xiv, 19, 1924.
Redslob. Am. d’Oc., clxv, 641, 1928 ; clzvi,
1, 1929.
Ruben. A. f. O., lxxxvi, 258, 1914.
Salzmann. Z. f. Aug., xxxi, l, 1914 ; xxxiv,
26, 1915.
Sattler. K. M. Aug., lvi, 580, 1916.
Schieck. K. M. Aug., lxi, 332, 1918.
B. O. G. Heidel., l, 69, 81, 1934.
Schmelzer. A. f. O., czviii, 1, 195, 1927.
Seidel. A. f. O., xcv, 39, 1918; ci, 392; cii,
189, 372, 415, 1920 ; civ, 158, 284, 409 ;
evi, 187, 1921 ; cvii, 92, 101, 1922;
cxi, 157, 167, 388 ; cziii, 188 ; cziv,
157, 163, 388, 1924.
Abderhalden’s Hb. d. biol. Arbeitsmethoden,
v (6), 1019, 1927.
Smith, Priestley. Glaucoma, London, 1879,
1891.
Sondermann. A. f. Awg., cii, l l 1, 1929.
K. M. Aug., xcii, 313, 1934.
Stransky. Anomalien d. Skleralspannung,
Wien., i (1), 1912.
|Wien. kl. W., 1323, 1913.
Thiel. K. M. Aug., lxxvii, 753, 1926.
Thomas and Fischer. Am. of O., xix, 40,
1910.
Trantas. Bull. S. Hellénique d'O., iv, 1935.
Troncoso. An. d’Oc., czzvi, 401, 1901 :
cxxxiii, 5 ; czXxiv, 250, 1905.
A. d’O., xxx, 91, 151, 1910.
Verhoeff. A. of O., xliv, 129, 1915; liv, 20,
1925.
Weber. A. f. O., xxiii (1), 1, 1877.
Wegner and Endres. Z. f. Aug., lxiv, 43,
1928.
Wertheimer. A. ges. Physiol... cxcix, 383,
1923 ; c.cviii, 669; cox, 527, 1925.
3364 TEXT-BOOK OF OPHTHALMOLOGY
Clinical Picture
Although from the aetiological point of view it is unjustifiable, it is
convenient from the clinical point of view to divide cases of glaucoma into
two main groups each of which presents a completely different clinical
picture so that they appear to be separate and unrelated diseases—SIMPLE
and CONGESTIVE. It must be emphasized that the differentiation is not
one of tension, for an eye may retain the clinical appearance of simple
glaucoma and yet have a much higher tension than an obvious case of
acute congestive glaucoma : in the first case the tension may be 90 mm.
Schiótz, in the second 40. The differentiation rests upon the adaptability
of the circulation to accommodate itself to the tension, for if capillary
stasis and permeability become so great that the eye becomes Oedematous
or if the venous return becomes embarrassed so that the circulation is
seriously interfered with, a congestive phase develops. The terminology of
“inflammatory ‘’’ as applied to the congestive manifestations seems inappro-
priate and should be reserved for glaucoma secondary to inflammation. An
alternative and again satisfactory choice of words to describe the two states
are the terms introduced by Elschnig (1928)—COMPENSATED and INCOM-
PENSATED. Heerfordt (1911) suggested the terms lymphostatic and haemostatic
glaucoma, but these suggest aetiological concepts with which all would not
agree. It is not to be thought, however, that these terms necessarily imply
definite or separate clinical entities, for any glaucomatous eye may at different
times pass from a simple, non-congestive, compensated phase to a sub-acute
or acute, congestive, incompensated phase and back again, and either type
tends in the end to progress to a condition of ABSOLUTE GLAUCOMA and
finally to undergo degenerative changes (GLAUCOMA DEGENERATIVUM).
SIMPLE GLAUCOMA (NON-CONGESTIVE OR COMPENSATED)
GLAUCOMA SIMPLEX, by far the commoner variety, varies very widely
in its clinical manifestations in different patients, but as a rule it develops
slowly, quietly and insidiously, and may, indeed, have arrived at a far
advanced stage in both eyes before anything amiss is noted by the patient
either in the appearance of the eye, in subjective symptoms of pain and
discomfort, or in apparent functional efficiency. Signs of congestion are
completely absent and the eye is white, apart, perhaps, from an enlargement
of the anterior ciliary vessels at their points of perforation of the sclera.
The anterior chamber is frequently shallow, but not invariably so ; the
pupil is frequently somewhat dilated and sluggish although it may retain
its normal size and reactions for a long time ; and the iris is frequently
atrophic, often in a patchy manner, showing eventually some degree of
ectropion of the pigment layer and a considerable amount of pigmentary
atrophy (Fig. 2852). Sooner or later the disc tends to become cupped and
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3365
atrophic, while the tension is usually found to be above normal. Meantime,
although central vision may remain good until a late stage, a shrinkage of
the peripheral field develops or para-central scotomata become annoying.
Apart, however, from the occasional appearance of coloured halos around
lights and a vague feeling of fullness or headache, the patient may be little
inconvenienced. As the disease progresses, the evidences of general atrophy
increase : the sclera assumes a delicate, bluish-white, porcelain-like appear-
ance, the atrophy of the iris and the dilatation of the pupil become more
evident, the choroid shows evidences of depigmentation, particularly around
the disc, where a non-pigmented ring, the peri-papillary glaucomatous halo,
forms, and excavation and atrophy of the nerve-head increase. The
shrinkage of the visual field may now cause considerable embarrassment
and the patient walks hesitatingly, turning his head repeatedly from side to
side, fumbling for objects and stumbling over obstacles in his path.
A gradual progression of all these symptoms occurs, sometimes rapidly,
sometimes exceedingly slowly over a period of many years, until eventually,
and usually not without the intervention of headache and pain, the eye
becomes intensely hard, all vision is lost and a state of absolute glaucoma
is reached. At other times intermittent attacks of congestion occur, indi-
cated by the occurrence of headache, the appearance of halos and transient
obscurations of vision, and characterized by a fine corneal oedema, a ciliary
flush, and an increased dilatation of the pupil. These may come and go,
the eye returning again after each to a condition of simple glaucoma with
every disability accentuated after each attack; or at any time compensa-
tion may completely break down, and a painful sub-acute attack or a frankly
acute congestive phase may intervene.
CONGESTIVE OR INCOMPENSATED GLAUCOMA
An attack of congestive glaucoma may develop in a variety of ways:
as we have seen, it may become superimposed on a simple glaucoma , it
may appear without warning and with great suddenness in an eye apparently
healthy in every way ; but more usually it is preceded from time to time by
mild prodromal sub-acute attacks, the significance of which may have not
been realized, and the occurrence of which may only be elicited after careful
questioning. The evolution of the disease may also vary considerably. The
typical sequence involves five phases: (1) an initial phase of sub-acute
prodromal attacks, which culminate in (2) an acute attack, followed by
(3) a chronic congestive phase which terminates in (4) absolute glaucoma,
the eye eventually undergoing (5) degenerative changes. These phases
differ only in degree, the first consisting of mild replicas of the second, and
the third a persistence of the same state showing increased violence and
indefinite prolongation, to be followed by degenerative changes due to the
long-continued raised tension and hyperaemia. The first and second stages
3366 TEXT-BOOK OF OPHTHALMOLOGY
may occur alone and resolve, although rarely, and a chronic phase may
develop gradually with no acute introduction. Although the disease is
usually bilateral, an acute attack on both eyes simultaneously is uncommon.
The PRODROMAL ATTACKs, which were first described by Demours (1818)
with surprising accuracy, may occur intermittently for months or years
before the final crisis arrives, and are viewed by some patients with interested
amusement and by others with lively terror. They are infrequently induced
by conditions which upset the vasomotor balance, and are particularly
associated with worry, sleeplessness, fatigue or constipation, and occur
commonly in the morning. The vision becomes temporarily misty,
sometimes for a few minutes, sometimes for a few hours, rainbow rings
appear around lights or entoptic flashes are seen, while complaint is usually
made of a localized headache. Examination of the eye reveals a peri-corneal
flush and dilatation of the ciliary vessels, a somewhat steamy cornea, usually
a shallow anterior chamber and dilated pupil, a raised tension, but quite
frequently a normal disc. Each attack indicates a period of vascular stasis
and congestion and a failure of the eye to deal with the resulting oedema,
and as time goes on and the vaso-neurosis becomes further developed and
the filtration channels more inefficient, the recurring crises become more
frequent, last longer, and are more severe. Occasionally, it is true, if the
vascular element subsides and the angle of the anterior chamber remains open,
nothing further may develop for an indefinite time, but in the great majority
of cases, sooner or later compensation entirely breaks down and the condition
passes almost imperceptibly into the phase of a chronic congestive glaucoma
or an acute crisis may develop with dramatic intensity.
An ACUTE GLAUCOMATOUS ATTACK may be one of the most dreadful
occurrences in medicine. It usually, but not invariably, starts suddenly
and violently in one eye during the early hours of the morning when the
diurnal variation of the tension is at its highest level. The two dominant
groups of symptoms are, first, an excruciating pain in the eye together with
profuse lacrimation and an intense trigeminal neuralgia radiating over the
head and jaws and sometimes beyond, frequently associated with nausea
and vomiting, and second, a rapid fall in vision which may involve complete
loss of perception of light within a few hours. The constitutional disturbance
may be so great that the patient is prostrated, with an irregular, intermittent
pulse, pallid face and cold extremities, or flushed and fevered with a raised
temperature. The eye is intensely red and congested, the conjunctiva perhaps
chemotic and the lids Oedematous ; the globe is extremely tender and the
patient shrinks from the slightest touch, but it can be felt to be stony hard.
The cornea is dull, steamy and insensitive, the pupil widely dilated and
vertically Oval in shape, the anterior chamber shallow and clouded, and the
lens may have a peculiar greenish appearance. Ophthalmoscopic examina-
tion is usually impossible through the Oedematous cornea, but if the optic
disc can be seen it is oedematous and hyperaemic.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3367
Such an acute attack may evolve in one of three ways. (1) Sometimes,
after a period varying from some hours to some days, the acute phase may
resolve, but although to casual examination the eye may appear normal,
it is permanently damaged. (2) More frequently, resolution may be partial and
a condition of CHRONIC CONGESTIVE GLAUCOMA results. The tension remains
above normal, usually considerably so, there is a permanent circum-corneal
congestion with dilatation of the ciliary vessels, the cornea is hypo-aesthetic,
the anterior chambershallow with its angle partially or completely obliterated,
the pupil moderately or widely dilated, the iris sluggish and congested or
grey and atrophic and sometimes richly vascularized, while ophthalmoscopic
examination reveals a cupped disc with a surrounding halo of atrophy, a
tessellated fundus, congested veins and pulsating arteries. Meantime the
visual acuity is markedly diminished, the fields constricted and the blind-
spot enlarged. In this way the eye gradually deteriorates in structure and
function, its downward course being assisted by periodic acute crises, until
it passes on to complete blindness and absolute glaucoma. (3) More rarely
resolution may not occur but the attack ends in a permanently hard eye and
involves swift and complete blindness—GLAUCOMA FULMINANS. Such an
outcome is rare, however, in a first attack, which usually undergoes
partial resolution.
ABSOLUTE GLAU COMA
The condition of absolute glaucoma from every point of view is quite
without hope. The sclera is porcelain-white and on it the anterior ciliary
vessels, dilated and varicose, show up vividly by contrast in an irregular
anastomotic circle ; the cornea is quite insensitive ; the anterior chamber is
very shallow and its angle has become entirely occluded ; the pupil is widely
dilated and immobile ; the iris, bounded by a dark rim of pigmented
epithelium, has receded almost behind the scleral rim and has become depig-
mented and assumed the slate-grey appearance of advanced atrophy :
the fundus has become markedly tessellated and the dead-white disc shows
a deep excavation with a prominent overhanging edge ; and vision is per-
manently and completely lost. Unfortunately pain and headache are
usually constant and are liable to suffer severe exacerbations with the
recurrence of congestive attacks.
Finally DEGENERATIVE CHANGES complete the picture. Hyaline-like
opacities appear on the cornea, running in band-shape across it or spreading
irregularly over its surface ; chronic oedema breaks down in bullae which
periodically rupture; or opacification may be completed by the develop-
ment of a degenerative pannus.* The lens becomes opaque and the iris
extremely atrophic. Eventually, advancing degeneration in the sclera may
result in its yielding before the sustained pressure with the formation of
* p. 3342.
33.68 TEXT-BOOK OF OPHTHALMOLOGY
equatorial, ciliary or intercalary staphylomata. It is the exception for
relief from the continued pain to be obtained by the bursting of the globe
owing to mild trauma; more usually, if the eye is allowed to remain, further
intra-ocular degenerative changes–haemorrhages, irido-cyclitis, or pan-
ophthalmitis–complicate the sufferings of the patient, or alternatively the
cornea may disintegrate in a degenerative or hypopyon ulcer, in which
event perforation may result. In most cases if the eye survives
sufficiently long, the tension falls and it may even become soft owing partly
to stretching but mainly to general degenerative changes; in other
cases a persistent uveitis results in phthisis bulbi, but here again relief
from pain is not always obtained owing to the shrinkage of the eye. The
picture is indeed a tragic one, the pathos of which is intensified by the fact
that glaucoma is usually a bilateral disease.
Symptomatology
(a) External Earamination
The conjunctiva shows little change in simple glaucoma except that it
becomes more friable, and its circulation, while showing an abnormal
dilatation of the small vessels (Rollin,
1934), is poor in the limbal plexus.
Continued hypertension induces a visible
- varicosity of the vessels, sometimes with
- large ampulliform dilatations at their
- points of emergence associated with
cyst-like formations, where deposits of
uveal pigment may also be found (Thiel,
- 1929). Finally the constant engorge-
ment leads to permanent dilatation and
- the formation of new anastomoses, the
end-result in absolute glaucoma being
º y the formation of a prominent, tortuous
- - and irregular circle of venous anas-
tomoses—the Medusa head (Heerfordt,
1911; Köllner, 1922) (Fig. 2850). At
this stage the conjunctiva has become
atrophied and extremely brittle, and the sclera, owing to the progress
of sclerotic and degenerative changes, assumes a delicate bluish-white
appearance like porcelain. The further degeneration and consequent
stretching of this tissue to form staphylomata has already been discussed
(Fig. 2850).
- - -
-
Fig. 2850–Absolute GLAucoma.
Medusa head and ciliary staphylomata.
It is to be noted that a strikingly similar dilatation of the ciliary vessels may
occur as a rarity as a congenital anomaly (A. Fuchs, 1928) or may be inflammatory in
origin (Leber, 1880). These rare cases may be mistaken for glaucoma.


ANOMALIES OF THE INTRA-00ULAR PRESSURE 3369
Mild congestive attacks, while increasing the venous engorgement,
usually involve the development of a ciliary flush ; while in the more acute
phases the general congestion and redness may be intense and confluent
and provoke a massive chemotic oedema.
In the cornea two evidences of raised pressure are commonly seen—
haziness and anaesthesia—the pathology of which has already been
described " : clinically a steaminess and irregularity of the surface appears
which looks like ground glass, with sometimes the occurrence of large
bullae (Fig. 2851). The oedema may be fleeting or permanent, but it is not
in all respects a function of the tension; it may appear when the tension is
relatively low or persist after it has fallen, and it may be absent when the
tension has reached a very great height. Magitot (1936) found that it
disappeared after a retrobulbar injection of alcohol, and it would appear
that an abolition of the action of the corneal nerves exerts some influence
upon its development. It is probable that
this oedema is largely responsible for the
entoptic appearance of halos and the transient
periods of misty vision in the prodromal attacks
of acute glaucoma. It may, also, by pressure
on the perforating branches of the corneal
nerves as they traverse Bowman's membrane,
produce the anaesthesia of this tissue.
In the iris in all cases of glaucoma some
degree of atrophic change is evident ; its
structure loses its delicate architecture, the - --- --- -
crypts and the collarette disappear and º
eventually large patches of advanced atrophy tion, in an old case of absolute
become apparent. This has already been dis- “"“”
cussed”; as also has the shrinkage which results in an ectropion of the uveal
layer, and the almost constant pigmentary disturbance owing to which
transparent lacunae may be seen in the iris on transillumination, and its
anterior surface as well as the angle of the anterior chamber may become
plentifully besprinkled with pigmentary deposits (Bergler, 1925) (Fig. 2852).
In congestive glaucoma, particularly of the chronic type, enlarged veins are
commonly found running over the iris, an indication of the general congestion
which is also apparent on the sclera and the fundus (Fig. 2853).
The anterior chamber in simple glaucoma varies considerably in depth :
as a general rule in the young it is normal or deep, and in the old shallow ;
in emmetropes it is normal, in myopes deep, and in hypermetropes shallow.
Thus among 67 relatively young cases Haag (1915) found 55% of normal
depth, 18% deep, and 26% shallow, while similar findings were reported by
Löhlein (1913) and Rosengren (1929). The actual depth does not vary
closely with the tension (Raeder, 1923). When the chamber is shallow the
p. 3342. " - p. 2402.

3370 TEXT-BOOK OF OPHTHALMOLOGY
quantity of aqueous which can be abstracted from the eye is less (Magitot
and d’Autrevaux, 1925; Magitot, 1931), but its composition is normal
Fig. 2852, GLAucomatous ATRophy of THE IRIs witH PIGMENTARY
DEGENERATION.
(Magitot, 1931; Hodgson, 1938). In acute congestive cases, on the other
hand, the chamber is always shallow and with the slit-lamp the aqueous is
usually found to be cloudy with an excess of protein. In the angle of the
Fig. 2853.-ENLARGED IRIs VºssELs IN CHRonic GLAucoma.
anterior chamber the question of the formation of anterior peripheral
synechiae has already been discussed, when it was pointed out that gonio-
scopic examinations have made it clear that while they are common as a


ANOMALIES OF THE INTRA-OCULAR PRESSURE 3371
relatively early event in acute glaucoma as a result of the congestion, they
are of late formation in the average case of simple glaucoma (Figs. 2854–58);
Fig. 2854.--THE Normal ANGLE SEEN Gonioscopic ALLY.
C.B.I., ciliary border of iris. C.B., dark line indicating ciliary body. Sch. Zo.,
zone of Schlemm's canal. Scl., selera. Cor., cornea (after Troncoso).
Fig. 2855.-EARLY SIMPLE FIG. 2856.-Acute GLAucoma.
GLAU coma. Iris atrophic and discoloured.
The angle in simple glaucoma, Annular peripheral synechiae; no
showing commencing peripheral ciliary body visible and seleral zone
synechiae. The border of the iris is narrow (after Troncoso).
sinuous and covers the ciliary body,
but the zone of Schlemm's canal is
still seen in the depressions (after
Troncoso).
FIG. 2857.-SUB-ACUTE GLAU conta. Fig. 285S.-CHRosic GLAucovia.
Partial deflection of a peripheral The effect of eserine in detaching a
synechia by eserine, the ciliary body peripheral synechia apart from a single
being uncovered towards the left (after tag (after Troncoso).
Troncoso).
it is interesting that in congestive conditions posterior synechiae of a similar
nature binding the iris to the lens are not uncommon.
The pupil undergoes important changes in simple glaucoma. At first
it may appear normal but close examination reveals a disappearance of the
normal oscillatory reaction to light, a sluggishness to contract in the light



3372 TEXT-BOOK OF OPHTHALMOLOGY
and a readiness to dilate in the dark. These tendencies increase until
eventually it becomes inactive and dilated, a condition explicable partly
by atrophy of the musculature of the iris and partly by a pressure-paresis
of the ciliary nerves. Its maximal dilatation and its typical vertically
oval shape in acute or prolonged rises of tension are not quite understood.
The phenomenon appears some 3 to 4 hours after the commencement of the
attack, and the dilatation and rigidity persist until the tension falls spon-
taneously or after medical measures; if surgical relief of the tension is obtained
the pupil may remain dilated throughout life. A paresis of the nerves
probably enters into the question, but the phenomenon does not occur
with experimental hypertension, nor does it vary clinically parallel to the
tension. In a case of tabetic miosis with an Argyll Robertson pupil
without synechiae, Terson (1925) found no enlargement in the crises of acute
glaucoma. The phenomenon may depend to some extent on sympathetic
irritability, and it may be influenced not essentially by the tension itself
but by a disturbance of the local nervous apparatus by the oedema of the
ciliary body and the root of the iris (Magitot, 1939).
In the lens the most interesting feature in chronic glaucoma is the
frequent presence of capsular exfoliation, a senile phenomenon which we
have already discussed as perhaps having some aetiological significance in
the determination of hypertension by embarrassing the filtration channels."
It will be remembered that chronic glaucoma is said to occur in some 70%
of the subjects of exfoliation, and a considerable proportion, estimates of
which vary from 7% to 93%, of cases of chronic glaucoma in elderly persons
are found to have some degree of exfoliation. In acute glaucoma the smoky
greenish hue of the lens is an optical effect dependent upon a dilatation of
the pupil and a lack of transparency of the cornea, the aqueous and the lens
itself. In conditions of long-continued pressure the development of some
lens opacity is the rule, starting at first as a general haze, especially pos-
teriorly, and eventually progressing to form a complicated cataract.
The development of cataract in glaucoma, even although it is of the ordinary senile
type, may cause some anxiety. The advent of hypertension undoubtedly accelerates
the development of cataract (which is normally present in some degree in most persons
over middle life), and in the intumescent change the Swelling of the lens may interfere
further with the already inefficient drainage angle, thus increasing the tension still
further. A vicious circle is thus set up which is only broken by an operation for the
glaucoma, which again will almost certainly make the cataract worse.
(b) Ophthalmoscopic Eacamination
In acute congestive glaucoma, examination of the fundus is usually
impossible owing to haziness of the media, but if it can be made out, the disc
is usually seen to be oedematous and hyperaemic. In chronic glaucoma,
whether simple or congestive, several changes of considerable importance
1 p. 3106.
ANOMALIES OF THE INTRA-OOULAR PRESSURE 3373
occur. In the general fundus after the existence of prolonged pressure there
is always some degree of atrophy of the choroid and the pigmentary
epithelium, so that eventually the larger choroidal vessels are exposed and
a tessellated appearance results. This atrophy is frequently accentuated
in two situations—around the disc where a peri-papillary halo marks a ring
of extreme atrophy which allows the sclera to shine through , and at the
points of exit of vortex veins where a whitish or yellow areola appears.
Apart from these general changes two phenomena require special mention
—the pallor and cupping of the disc and the condition of the retinal vessels.
The optic disc presents one of the most important diagnostic features
in glaucoma of some standing. It undergoes two marked changes—atrophy
and cupping. We have already discussed the pathology of these changes
and have concluded that their causation must still be a matter of dispute.
Fig. 2859.-GLAU covt.A.Tous CUPPING. Fig. 2860.-PHYsiological CUP.
From the clinical point of view, however, the degree of pallor, indicating
the extent of atrophy of the nerve fibres, is of great importance, particularly
as a prognostic indication. The presence of optic atrophy is the most
constant clinical sign of established glaucoma, but the factor of mechanical
pressure does not apparently cause it alone: it does not vary in degree
with the height of the pressure (Magitot, 1929); it may be absent in the
presence of long-continued high pressure (Orr, 1914; Neuhäuser, 1923;
Elschnig, 1924; Nippe, 1927; Dvorzec, 1928; Merigot and Treiquy, 1938;
and others); and we have already seen that atrophy with excavation may be
extremely marked in the apparent absence of hypertension." In addition,
therefore, to the factor of pressure acting both upon the ganglion cells and
the nerve fibres, that of ischaemia due to vascular sclerosis is probably of
great, and sometimes of supreme importance.
The cupping of the disc is also, as we have discussed,” the result of a
p. 3071. p. 3351.

3374 TEXT-BOOK OF OPHTHALMOLOGY
combination of the same two factors of mechanical pressure and ischaemic
degeneration acting in various cases to varying degrees. From the clinical
point of view the commencement of cupping is betrayed by the presence of
a kink in the vessels as they cross the edge of the disc, particularly on the
temporal side. In its fully developed form the excavation appears as a
deep cup involving the whole of the area of the disc with deep straight sides
and usually an overhanging rim, while the bottom is pale and may show
the markings of the lamina or be covered with glial or connective tissue
(Fig. 2859). The course of the blood-vessels is important : as a rule they
are dragged over in a bundle towards the nasal side and climb over the
sharp edge with an acute bend. When the cup is deep the rim obscures the
sides so that the vessels disappear as they climb from the floor to the rim,

| N 7

* S
FIG. 2861. FIG. 2862.
FIGS. 2861–62.--TO ILLUSTRATE THE DISPLACEMENT OF VEssELS IN A GLAUCOMA CUP.
The sheet of paper with diagonal lines seen in Fig. 2861 is folded at right angles
first along the broken line and then along the dotted line. The resulting appearance
(Fig. 2862) shows how the apparent displacement of the diagonal lines corresponds
to that of the vessels.
to reappear again as they bend sharply over the edge. In doing so they
appear to suffer an apparent discontinuity in their direction, a phenomenon
best explained pictorially as in Figs. 2861–2862. -
The actual fact of cupping may be easily verified by the method of parallactic
displacement using indirect ophthalmoscopy"; and the depth of the excavation may
be measured by the direct method, by focusing a vessel on the floor of the excavation
and then on the rim. A difference of 5 dioptres is sometimes found between the two
levels, indicating a depth of 1.6 mm. The extent of this will be realized when it is
remembered that the diameter of the disc is 1.5 mm. ; in such cases the floor of the
cup extends beyond the outer surface of the sclera, and in excising such an eye it is
not impossible to cut through the cup and open the globe instead of cutting the optic
1) GI’Ve.
The retinal vessels frequently show the phenomenon of pulsation at the
disc. Venous pulsation is common in normal eyes and may be absent in
conditions of high tension, but it is of importance that an arterial pulse is
usually more readily evoked by pressure upon the globe than in the normal
1 Vol. II, p. 1179.
ANOMALIES OF THE INTRA-00ULAR PRESSURE 3375
eye and may, indeed, bespontaneous. This phenomenon of arterial pulsation
in glaucoma was first noted by v. Graefe (1855) and its study was continued
by Jacobi (1876), Ballantyne (1913), Krämer (1920) and others. It must be
remembered that a spontaneous arterial pulse is not diagnostic of glaucoma,
as is sometimes stated, for it may be conditioned by many factors and it
has long been known to occur in normal eyes (Donders, 1855; Becker, 1872;
Ballantyne, 1913; and others); but it is of significance in that it may
indicate that the intra-ocular pressure has approached or exceeded the
diastolic arterial pressure. In this event the expansile pulse becomes
complete at the disc, the arteries emptying at diastole and filling at systole,
and the blood-column flashing out of sight across the disc to appear
again as suddenly. Measurements by the ophthalmodynamometer *
have shown that the blood-pressure in the vessels is generally raised, that
in the arteries by a small amount, but that in
the veins considerably, a phenomenon which still
remains after the tension has been relieved by
treatment (Bailliart and Magitot, 1925); it is
interesting that by entoptic observation of the
peri-foveal circulation, Dieter (1925) found a rise
in the capillary pressure, while Seidel (1924)
found a similar rise in pressure in the anterior
ciliary vessels. Ophthalmoscopically the arteries
usually show little change in calibre, although
they may be slightly constricted, but it is the
rule for the veins to be engorged and congested. * *º. "
So much so, indeed, may this venous stasis be Showing dilated tortuous
apparent that even in non-congestive glaucoma, Yºssels (after , operation)
- - - - (Henderson and James, Brit.
particularly in the region of the disc, large jo.
venous loops and tortuosities or new anastomotic
channels may appear, a phenomenon comparable to the venous changes on
the sclera and in the iris (Fig. 2863).
The Ocular Tension
Both in simple and acute glaucoma the tension may vary within wide
limits, and it must be again emphasized that the actual height of the tension
has little to do with the type of glaucoma or its pathological effects, either
upon the structure or function of the eye : one eye may withstand amazingly
high pressures for a long time without apparent damage, another may
undergo an acute crisis with a tension of 30 mm. Schiótz, which may almost
be considered within the upper limits of the normal range; and a third will
progress to blindness with intermittent periods of raised tension so un-
obtrusive that they have to be specially investigated. In any particular
* Vol. I, p. 398. * Vol. I, p. 404.
T-0---0-111. + N.

3376 TEXT-BOOK OF OPHTHALMOLOGY
case that tension is pathological which the tissues of the eye in question cannot
withstand without damage.
In the investigation of the tension the tonometer is indispensable, and in low
tension and early cases repeated tonometric measurements become invaluable.
Whatever its absolute accuracy may be, as an indicator of relative measurements
between the two eyes of one individual and between the same eye at different times,
the tonometer is more dependable than most clinical instruments.
Not only is the height of the intra-ocular pressure of importance;
its instability is of great significance. It may be said that, particularly when
it is not materially raised, the tension of a glaucomatous eye responds in an
eacaggerated way to stimuli which a Sound eye can neutralize to a large extent.
Thus in the healthy eye the tension should not differ materially from that
of its fellow and should not vary over the limits of a few millimetres Schiótz
at different times of the day, in different illuminations, on the adoption of
the horizontal position, or on an
exhibition of miotics, whereas the
glaucomatous eye may show a
marked instability in all these cir-
cumstances.
The Diurnal Variation. We
have already discussed the normal
diurnal variation of the intra-
Ocular pressure,” in which, as was
FIG. 2864.—THE DIURNAL VARIATION. first pointed out by Maslenikow
& Chronic primary glaucoma, the continuous (1904), the pressure is highest in
line ; the sound eye, the dotted line. e tº
the morning before getting out
of bed and lowest late in the evening ; each variation occurs in two
steps, a sharp fall at first, followed by a gradual decline throughout
the day, and a slow rise at night, tending to become accentuated towards
morning. The variation may be due in some measure to the redistribution
of blood during decubitus and changes in its Osmotic value owing to feeding
and exercise, and to changes between light and darkness, but the essential
influence appears to be the lack of muscular activity at night and its
effectiveness on waking. This acts both by massage of the globe by the
extra-ocular muscles and by the opening up of the canal of Schlemm by the
ciliary muscle. To a large extent, therefore, it is a measure of the efficacy
of the drainage channels. In the normal eye the total variation should not
be much greater than 3 mm. Schiótz, but in the glaucomatous eye this
difference may be greatly exceeded : at one time of the day the tension
may be normal and at another 20 or more mm. Schiótz higher (Fig. 2864).
These variations have been investigated by Pisarello (1915), Köllner (1916–18),
Thiel (1922–5), Hagen (1925), Raeder (1925), Löhlein (1926), Seidel (1927),
Andrezen (1928), Lauber (1928), Sallmann and Deutsch (1930) and others,
1 Vol. I, p. 502 (511).
A fº A hº
|

ANOMALIES OF THE INTRA-OCULAR PRESSURE 3377
and important results have emerged from their findings. The most favour-
able time to elicit the presence of raised tension is from 5–7 o’clock in the
morning before the patient has stirred : in glaucoma simplex the tension
in the afternoon and evening is frequently sub-normal. In the
diagnosis of this type of glaucoma, the extent of the variation rather than
the absolute tension may be of importance, for in an early case a glauco-
matous curve may lie wholly below the normal level (Hagen, 1925). It
follows that to study the pressure thoroughly in cases of difficulty the
patient should be made to lead a regular life and the tension measured at
the same hours, one of which should be the time
of maximum tension, for three successive days.

Occasionally, for reasons which are not yet clear, a
reversal of the curve takes place, the tension being
highest in the evening (Hagen, 1925; Raeder, 1925;
:
:
:
Dominguez, 1929). # 90
A variation of the ocular tension in the dark ź
is also of significance in glaucoma. It was first = 40
shown by Grönholm (1910) that the tension of the 5
normal eye tends to rise somewhat after a stay :
in the dark and that a glaucomatous eye shows 3 30
a considerably higher rise (Fig. 2865). These obser- #
vations were confirmed by Seidel (1920–28) and 2O| L-J.--T---
applied as a clinical test ; his observations have s sº
been extended by Serr (1928), Feigenbaum
iO
Time in hours.
(1928), Sallmann (1929) and Poos (1934).
Grönholm (1910) considered the reaction due to gº
g g e e e tº 8 FIG. 2865.--THE REACTION
pupillary dilatation resulting in an abolition of the OF THE TENSION IN
drainage capacity of the iris; Seidel (1920) thought PARKSEss.
that the dilatation allowed the angle of the anterior º hiſ ºn i.e. Primary
& © & glaucoma the continuous
chamber to be blocked by the iris; but since he line; the sound eye the
showed that the variation had no relation to the **
size of the pupil, was unaltered in aniridia and after an iridectomy, and
occurred although modified in degree after atropine and pilocarpine,
Feigenbaum (1931) concluded that it was due to the direct action of light
upon the ocular capillaries rendered more labile in the glaucomatous state :
he showed, moreover, that the reaction occurred bilaterally in unilateral
glaucoma, even although the affected eye was blind so long as it was
not degenerated, and that this bilaterality was abolished after an optico-
ciliary neurectomy, from which he inferred that it was mediated by a vaso-
motor axon reflex. In the normal eye the change occurs after dark adap-
tation has lasted 40 minutes, the rise is small (about 2 mm. Schiótz), and
the tension falls rapidly on re-exposure to light. A rise of over 6 mm.
Schiótz is suspicious of primary glaucoma, and variations of 30 mm. or more
may occur (Feigenbaum, 1928). -
4 N 2
3378 TEXT-BOOK OF OPHTHALMOLOGY
Thus Seidel (1920) observed a rise of from 20 to 80 mm. after half an hour in the
dark with a return to normal within half an hour in the light, and Poos (1934) found
a rise of over 15 mm. in 70% of his cases of primary glaucoma which was usually
lowered by the previous exhibition of eserine. It is interesting that in secondary
glaucoma the pressure may remain unchanged or fall (Hagen, 1925; Raeder, 1925;
Feigenbaum, 1928; Magitot, 1933).
The induction of vascular congestion also produces an abnormally high rise of
pressure in the vascularly unstable glaucomatous eye. This may be seen, for example,
after raising the general blood-pressure by caffein (Thiel, 1925; Wegner, 1925;
Löhlein, 1926; Lauber, 1928), by
inducing decubitus (Thibert, 1922 ;
Bailliart and Laval, 1924; Thiel,
1925), by lowering the head or by
compressing the jugular veins (Thiel,
1924; Wegner, 1925).
Finally, massage of the globe should
lower the tension considerably in the
healthy eye, whereafter it returns to
100 normal ; in the glaucomatous eye,
presumably owing to the difficulty in
the exit of fluid, the fall is less, or the
tension remains unchanged, while
Variation in the tension in an eye with
hronic primary gla (conti line) thereafter it may show a reactive rise
Chr0111C l r glaucoma COIlú1I] U1OUIS line * º
and a sound eye (dotted line). (Gelder, 1911 ; Knapp, 1912 ; Wegner,
1925; Dieter, 1928) (Fig. 2866).
Time in minutes.
FIG. 2866.--THE EFFECTS of MASSAGE.
It is to be noted that in all these variations the other eye may react
similarly, although to a less degree, even if it is healthy : the facts that this
reaction is abolished by a retro-bulbar injection of alcohol or an optico-
ciliary neurectomy, and does not occur on stimulation of the good eye after
enucleation of the affected eye, show that it is reflex in origin (Leplat, 1924;
Sédan, 1927; Magitot, 1927–33; Birch-Hirschfeld, 1929; Feigenbaum,
1931 ; Weekers and Fauchamps, 1936).
Subjective Symptoms
As we have noted, subjective symptoms are usually absent in simple
glaucoma until a late stage when a raised tension may give rise to a feeling
of fullness in the eye and frontal headache, which in the more advanced
degrees give place to pain. In congestive glaucoma, however, pain is more
in evidence and in acute glaucoma it becomes one of the most excruciating
pains known. To the distressing and sickening local pain associated with an
organ enclosed in an unyielding capsule under hypertension, there is added an
acute neuralgia, confined not alone to the frontal distribution of the orbital
nerve, but radiating over the area of supply of the entire trigeminal to the
jaws and sometimes beyond. The lacrimation, which is sometimes profuse,
may be a reflex nervous phenomenon, while the associated nausea and
vomiting is due to a similar spread of excitation from the bulbo-spinal root
of the trigeminal to the nucleus of the vagus.

ANOMALIES OF THE INTRA-OCULAR PRESSURE 3379
Wisual Symptoms
The visual acuity in simple glaucoma may remain unimpaired for a
very long time—long after the visual field has suffered severely. Occa-
sionally, however, refractive changes occur in the direction of myopia, due
probably to advancement of the lens. More commonly, the opposite
change of an increase in hypermetropia is apparent, which may be due
to a latent error becoming manifest owing to weakness of the ciliary muscle
induced partly by pressure on the nerves or partly by the atrophic action of
pressure on the muscle fibres themselves and general sclerosis. This is most
markedly seen in a weakness of accommodative power, and an early and
Tapidly increasing presbyopia is a common and significant sign of commencing
glaucoma. In the more advanced stages of the disease the vision fails
owing to retinal ischaemia and degeneration and atrophy of the optic nerve.
In the exacerbations of simple glaucoma and in sub-acute and acute
congestive glaucoma, definite attacks of indistinctness of vision appear, the
patient experiencing a feeling as of looking through a haze or mist ; they
are most typically seen in the prodromal phases of the congestive types.
The phenomenon is due to corneal oedema, and the same anatomical change
may produce the entoptic appearance of coloured halos round lights owing
to diffraction by droplets of fluid in the corneal epithelium. These attacks
tend to come on in the early morning when the tension is highest, and they
may be induced by any factor which excites vasomotor instability, such
as a sudden change of atmospheric temperature, a hot bath, or a temporary
phase of excitement. As a rule, in the early stages of the disease they are
fleeting, vision rapidly regaining its normal standard ; at a later stage they
may easily be dissipated by a miotic ; but in states of high tension they
may be lasting and distressing. In acute phases the vision fades out
suddenly and completely, due presumably to a pressure-block of nerve
impulses; if the pressure is rapidly reduced vision may return almost as
rapidly as it went and within 48 hours may be almost normal; but if the
tension remains high for a sufficient time to allow degenerative changes to
take place, vision is permanently lost.
A glaucomatows halo is best seen when looking at a bright light from a distance
in the dark : two coloured rings are always seen, an inner blue-violet and an Outer
yellow-red, the red tinge being on the outside, while a green ring may separate these.
At times they are so faint that they have to be specially elicited, and at other times
their prominence causes much worry and confusion to the patient. Their physical
causation, the methods of testing for them, and their differentiation from halos due
to physiological and other pathological conditions have already been fully discussed.*
It may be useful to demonstrate to the patient what the appearance is, and, as Fraun-
höfer first showed in 1850, this can be done by asking him to look at a light through
a film of lycopodium powder pressed between two glass plates, a device which may
be retained permanently in an aluminium trial lens frame (Foster, 1937). In diagnosis
it will be remembered that the pathological halos are distinguished from the physio-
1 Vol. I, p. 810 (820).
3380 TEXT-BOOK OF OPHTHALMOLOGY
logical (due to the lens) by the fact that they are not partially eclipsed and broken up
but only suffer a diminution of intensity if a straight edge (Druault, 1898–1923) or a
stenopaeic slit (Emsley and Fincham, 1923) is passed across the pupil. Halos due to
corneal oedema can further be differentiated from other pathological halos by a measur-
ment of their diameter. The former have an angular diameter varying with the size
of the oedematous droplets, from 7°–12° (Sheard, 1919; Koeppe, 1920; Elliot,
1921–23; Druault, 1923; Priestley Smith, 1924)—that is, the linear diameter of the outer
red ring measured on a wall, when the light is 10 ft. from the patient, is 15 to 25 in.
Halos due to conjunctival mucus or lacrimal secretion are larger (14°), those due to
lenticular diffraction are smaller (6° to 7°) and those due to the corneal endothelium
smaller still (4°). It is to be noted, however, that a halo of the glaucomatous type is
not pathognomonic of glaucoma, for it occurs in any condition of corneal oedema
whether the pressure is raised or not (Duke-Elder, 1927).
The Visual Fields
In acute congestive glaucoma the condition of the vision is such that
a study of the visual fields is impossible, apart from rough tests to hand
movements or light perception ; in chronic congestive glaucoma, the fields
may or may not be typically glaucomatous ; but in simple glaucoma,
especially in its earlier stages, perimetry is a most valuable method of
investigation not only to establish the presence of the disease but also to
measure its progress, estimate its prognosis, and assess the value of treatment.
The main features of the glaucomatous field are :—
1. A general depression with peripheral contraction which is most
pronounced on the nasal side and is frequently associated with a nasal step
and at a later stage with wide sector defects : the defects are at first relative
but eventually become absolute.
2. The formation of arcuate scotomata around the fixation point, with
retention of central vision until a late stage.
3. Colour vision is not impaired to a disproportionate degree.
The characteristic changes are nerve-fibre bundle defects, which in this
disease attain a very high state of development (Figs. 2867–68). The absence
of a preferential loss of the colour field in areas which are partially affected
indicates that the functional loss is due not to the partial impairment of all
the fibres but to the complete impairment of a small proportion of the fibres
in a particular bundle. The evidence would seem to indicate that these defects
are largely brought about by the anatomical conditions at the disc, the
two massive bundles which enter the disc on the outer side of its upper and
lower poles being first affected at which points the fibres are most numerous ;
thereafter successive fibres to the nasal side are involved and the macular
fibres last. The phenomena cannot, however, be explained as pressure-
atrophy of the nerve fibres alone, but suggest that the vascular factor is
also of importance ; its influence in determining these scotomata is seen in
their independence of the tension of the eye and their occasional progress
after its relief, while their dependence on the fluid-traffic of the eye is seen
in the varying changes observed by Samojloff (1922–25) and corroborated by
ANOMALIES OF THE INTRA-OCULAR PRESSURE 338]
Wegner (1925) which occur when the tension is similarly altered by agents
which have different vasomotor effects, such as adrenalin, pilocarpine and
hypotonic saline.
The history of the study of the fields in glaucoma is a long and interesting one
which started with the studies of v. Graefe (1855–69). After his initial researches
investigators confined themselves to the perimeter, and therefore, apart from the
description of a Scotoma in the shape of a comet by Landsberg (1869), they were
aware only of the gross sector defects. This state of affairs existed until Bjerrum
(1889) introduced the campimeter and founded quantitative perimetry. With this
more efficient technique he rediscovered the comet defect which goes by his name
(BJERRUM's SIGN), an observation which attracted world-wide attention to the value
NASAL TEN-1PORAL
FIGS. 2867–68.-NERVE-FIBRE BUNDLE SCOTOMATA.
The course of the nerve-fibres in the retina is seen in Fig. 2867. The fibre .
involved in lesions A, B and C produce the correspondingly marked nerve-fibre bundle
scotomata in Fig. 2868.
of perimetry and was elaborated particularly by Meisling (1900) in France, Frieden-
wald (1902) in America, and Sinclair (1905) in Scotland. Shortly afterwards Rönne
(1909) described the ultimate fate of Bjerrum’s comet scotoma to end in the horizontal
raphe when it was deliminated sharply in RöNNE’s NASAL STEP. Further elaboration
led Seidel (1914) to claim the earliest appearance of this peculiar Scotoma as a prolonga-
tion upwards or downwards of the blind-spot (SEIDEL’s SICKLE SCOTOMA), a finding
amplified by Elliot (1922) who described its feathery edges. For some considerable
time thereafter it was the generally accepted opinion that the field-defect of glaucoma
always began as an extension of the blind-spot, but, working with still smaller visual
angles, Traquair (1927) and Peter (1927) discovered that the first scotomatous defect
appeared as a small detached area above or below the blind-spot which later became
merged with it. Such a scotoma is difficult to find since it is away from the blind-spot
and isolated, relative in nature and frequently fleeting, and occurs in eyes not yet
usually suspected of being pathological. More recently these defects have been further
amplified by the technique of angioscotometry by Evans (1926–38).
The initial field-defect as determined by very small visual angles is a
depression of the 1/2000 isopter (Traquair, 1939), which is usually especially




3382 TEXT-BOOK OF OPHTHALMOLOGY
FIGS. 2869–74.—GLAUCOMATOUS FIELDs.
<2
4%º
Ǻjº
#& #2
FIG. 2870.-EARLY NASAL STEP :
(5/330,
FIG. 2869. –BARING OF THE
BLIND-SPOT (5/330, 1/2000). BARING OF THE DISC
1/2000).
9 * C & 2
FIG. 287 l.-EARLY COMET SCOTOMA FIC: . 2872.--SEIDEL's SCOTOMA
witH NUCLEUs (5/330, 1/2000). (5, 2/330).
O º
UV tº
\\º
º
&\sº
& Oz 2
FIG. 2873.--—SECTOR DEFECT AND FIG. 2874.—DouBLE ARCUATE
ARCUATE SCOTOMA (5, 2/330). (RING) ScotoMA (5/330).


























ANOMALIES OF THE INTRA-OCULAR PRESSURE 3383
noticeable on the outer side of the blind-spot ; the isopter passes to its nasal
instead of its temporal side so that the blind-spot is, as it were, laid bare
usually on its upper aspect (Fig. 2869). This occurs while the peripheral
field is normal, and as constriction of this isopter proceeds, a small nasal
step may appear. Thereafter this nasal step usually develops into a partial
arcuate Scotoma advancing towards the blind-spot but not in contact
FIGS. 2875–78.—GLAU comATOUs FIELDs.
FIG. 2875. — DOUBLE ARCUATE FIG. 2876.--—RETENTION OF FIXATION
SCOTOMA AND QUADRANTIC AND TEMPORAL PERIPHERY
DEFECT (5/330). (5/330).
FIG. 2877.--CENTRAL DEFECT witH- FIG. 2878. –Loss OF Low ER
OUT PERIPHERAL CHANGE (5/330). FIELD (5/2000).
with it (Fig. 2870). In it there ultimately appears a small blind area
forming an absolute scotoma, at first not connected with the blind-spot
(Fig. 2871), but eventually merging into it to form a Seidel’s sickle Scotoma
(Fig. 2872). These early scotomata may be inconstant, demonstrable on
some days and not on others, and they may disappear after the use of
miotics and vary with the state of the eye, sometimes advancing and some-
times receding (Samojloff, 1924–25; Evans, 1930 ; Sloan, 1931 ; and


3384 TEXT-BOOK OF OPHTHALMOLOGY
others); moreover, they can only be elicited by the most careful quantitative
methods, and can sometimes be demonstrated in reduced illumination while
the field is normal in a good light (Haffmans, 1861 ; Marlow, 1932; Roll,
1938).
The more fully formed defect eventually forms Bjerrum’s comet scotoma
and arches from the blind-spot round the fixation area to end on the
horizontal meridian where it forms the sharp step of Rönne (Fig. 2873);
sometimes both upper and lower sides are affected, in which case the fixation
area may be almost (Fig. 2874) or completely isolated by a ring Scotoma
(Fig. 2875). Thereafter large sector defects may develop, the peripheral
field failing more rapidly than the central, and the nasal field disappearing
in advance of the temporal ; central vision usually is long preserved, but
eventually it also succumbs, usually to be survived by a patch in the temporal
periphery (Fig. 2876). Occasionally the central part of the field fails early
while the periphery remains intact, a small arcuate scotoma forming in
the centro-caecal area (Fig. 2877), or the whole central field or its upper or
lower half is impaired or lost at a comparatively early stage of the disease
(Fig. 2878). Exceptionally small spike-like scotomata appear in the peri-
pheral field (Smith, 1925).
It is interesting that as a rule these changes, both peripheral and
central, tend to be remarkably symmetrical in the two halves of the same
field. No definite sequence is followed and all types of variatiqn occur,
producing an infinite variety of different appearances, in all of which,
however, the same basal tendencies can be found. It is to be remembered,
however, especially in comparing the condition on different occasions,
such as in an assessment of the progress of the disease or of the effectivity
of treatment, that every care should be taken to maintain constancy in
the conditions, such as the degree of illumination, the size of the pupil, the
time of day and its relation to meals and stimulants, and the state of fatigue,
excitement or anxiety.
The Light Sense
It has long been recognized that the light sense suffers severely in
chronic glaucoma, and patients remark constantly of their difficulties in
adapting themselves on entering a dim illumination after a bright light.
Delorme (1912) and Beauvieux and Delorme (1913) originally thought that
the light difference was first impaired, but that a fall in the perception of
the light minimum was a late happening and indicative of optic atrophy.
Henry (1920), however, concluded that at the earliest stages of simple
glaucoma there was a rapid reduction of the light minimum but only little
change in the light difference, the reverse of the case of optic atrophy. The
further observations of Ammann (1921), Tschenzow (1922) and Möller (1925)
confirmed these findings to some extent, laying stress upon the influence of
lowered visual acuity and field-defects; and the question was finally
ANOMALIES OF THE INTRA-OCULAR PRESSURE 33.85
clarified by the extended researches of George Derby of Boston and his
co-workers (Waite, Derby and Kirk, 1925; Derby, Chandler and O’Brien,
1929; Derby, Chandler and Sloan, 1929). Using elaborate controls of the
physical and physiological variables, they established that at low intensities
of illumination the changes of the light difference were insignificant either
in early or advanced simple glaucoma, but that the light minimum was
considerably raised and that the rate of dark adaption was retarded
(Fig. 2879). In established glaucoma these tendencies may be marked, but
these workers confirmed Henry's (1920) view that they were demonstrable
N -
" -
º
-
Tº
D
GE
-
-
ls. º
º -
º o * * sº * - *
Fig. 2879-THE LIGHT SENse IN GLAucoma.
Comparative curves of the light-minimum. The ordinates represent micro-
millilamberts as units of intensity at the screen (Waite, Derby and Kirk).
in the earliest stages of the disease: a delay in the development of dark-
adaptation and a dulling of sensitivity to dim light therefore form a syndrome
of considerable value in the diagnosis of the disease in its prodromal stages.
At a later date Zanettin (1931) concluded that a reduction in the light sense
of the peripheral retina was a more constant feature of the disease, occurring
even when the function of the central region is normal, and affecting most
markedly the temporal area, and thereafter, in order, the superior, inferior
and nasal. It is to be noted, however, that these changes in sensitivity
are not universal in glaucoma and that great individual variations occur
with no constant relation to the height or duration of the tension; they
may have a vascular basis (Feigenbaum, 1928), but their origin is unknown.
The condition of the light sense cannot therefore be taken as absolutely


3386
TEXT-BOOK OF OPHTHALMOLOGY
diagnostic, nor does it form a trustworthy basis for prognosis (Casten and
Shaad, 1933; Feldman, 1939).
The colour sense is unaffected preferentially in glaucoma, and the colour fields
are not disproportionately constricted. Wessely (1927) has observed a tendency to
failure in the perception of red in certain patients; the classical example of this
defect was Javal, who suffered markedly from red-blindness long before he lost his
sight completely from this disease.
Ammann. K. M. Aug., lxvii, 564, 1921.
Andrezen. Russ. O. J., vii, 303, 1928.
Bailliart and Laval. Bull. S. d’O. Paris,
xxxvi, 364, 1924.
Bailliart and Magitot. An. d’O., clxii, 736,
1925.
Ballantyne. Ophthalmoscope, xi, 271, 338,
460, 1913.
Beauvieux and Delorme. A. d’O., xxxiii, 93,
1913.
Becker. A. f. O., xviii, 206, 1872.
Bergler. A. f. Aug., xcv, 35, 1925.
Birch-Hirschfeld. Z. f. Aug., lxx, 1, 1929.
Bjerrum. Skand. O. Mag., ii, 141, 1889; v,
32, 1892.
Casten and Shaad. A. of O., ix, 52, 1933.
Cordes. A. of O., xvii, 896, 1937.
Delorme. Thése, Toulouse, 1912.
Demours. Précis des maladies des Yewa, 1818.
Derby, Chandler and O’Brien. A. of O., i,
693, 1929.
Derby, Chandler and Sloan.
xxvii, 110, 1929.
Dieter. A. f. Aug., xcvi, 179, 1925; xcix,
678, 1928.
Dominguez. A. de oft. H.-A., xxx, 491, 1929.
Donders. A. f. O., i (2), 75, 1855.
Druault. A. d’O., xviii, 312, 1898; xl, 458,
536, 1923.
Duke-Elder. Brit. J. O., xi, 342, 1927.
Dvorzec. Russ. A. O., iv, 510, 1928.
IElliot. Brit. J. O., v, 500, 1921.
Treatise on Glaucoma, London, 1922.
Am. J. O., vi, l, 1923.
Elschnig. Z. f. Aug., lii, 287, 1924.
A. f. O., czz, 94, 1928.
Emsley and Fincham. Am. J. Phys. Opt.,
iv, 247, 1923.
Evans, J. N. Am. J. O., ix, 118, 489, 1926;
xii, 194, 1929; xiv, 772, 1931 ; xvi, 417,
1933.
A. of O., iii, 153, 1930.
Clinical Scotometry, New Haven, 1938.
Feigenbaum. K. M. Aug., lxxx, 577, 1928.
A. of O., v, 261, 1931.
Feldman. A. of O., xxii, 595, 1939.
Foster. T. O. S., lvii, 364, 1937.
Friedenwald, H. An. of O., xi, 157, 1902.
Wuchs, A. Brit. J. O., xii, 65, 1928.
Gelder. K. M. Aug., xlix (2), 592, 1911.
v. Graefe. A. f. O., i (2), 299, 1855; ii (2),
291, 1856; xv (3), 108, 1869.
Grönholm. A. f. Aug., lxvi, 346; lxvii, 136,
1910.
T. Am. O. S.,
Haag. K. M. Aug., liv, 133, 1915.
Haffnnans. A. f. O., viii (2), 125, 1861.
Hagen. Acta O., ii, 199, 1925.
Heerfordt. A. f. O., lxxviii, 413, 1911.
Henry. Brit. Med. J., ii, 111, 1920.
Hodgson. T. O. S., lviii, 87, 1938.
Jacobi. A. f. O., xxii, 111, 1876.
Knapp, P. K. M. Aug., 1 (1), 691, 1912.
Roeppe. K. M. Aug., lxv, 556, 1920.
Röllner. A. f. Aug., lxxxi, 120, 1916;
lxxxiii, 135, 1918 ; likxxvi, 114, 1920;
xci, 181, 1922.
Krämer, A. f. O., ciii, 14, 1920.
Landsberg. A. f. O., xc (1), 204, 1869.
Lauber. Lij. Vjes., l, 140, 1928.
Leber. A. f. O., xxvi (2), 169, 1880.
Leplat. An. d’Oc., clxi, 87, 1924.
Löhlein. A. f. O., lxxxv, 393, 1913.
K. M. Aug., lxxvii, Beil., 1, 1926.
Magitot. An. d’Oc., clxiv, 482, 1927; clxv,
481, 1928; clzvi, 454, 1929 ; clzviii,
785, 1931 ; clxx, 465, 1933; clzxiii, 785,
1936.
A. of O., vi, 647, 1931.
Traité d’Ophtal., vi, 226, 1939.
Magitot and d’Autrevaux. Bull.
Paris, xxxvii, 111, 1925.
Marlow. A. of O., vii, 211, 1932.
Maslenikow. Westm. O., xxi, No. 5, 1904.
Meisling. An. d’Oc., czziv, 417, 1900.
Merigot and Treiquy. Bull. S. d’O. Paris,
l, 10, 1938.
Möller. Acta O., iii, 170, 196, 1925.
Morax. Glaucome et les Glaucomateua, Paris,
1921.
Neuhäuser. A. f. Aug., xcii, 235, 1923.
Nippe. Ohio State Med. J., xxiii, 221, 1927.
Ohm. A. f. O., czz.xv, 537, 1936.
Orr. O. Rev., xxxiii, 33, 1914.
Peter. A. of O., lvi, 337, 1927.
Pisarello. An. di Ott., xliv, 544, 1915.
Poos. B. O. G. Heidel., 1, 73, 81, 1934.
Raeder. A. f. O., czii, 29, 1923.
R. M. Aug., lxxiv, 424, 1925.
Redslob. Bull. S. fr. d’O. xlix, 145, 1936.
Roll. K. M. Aug., c, 600, 1938.
Rollin. A. d’O., li, 107, 1934.
Rönne. K. M. Aug., xlvii (1), 12, 1909.
A. f. O., lxxi, 52, 1909.
Rosengren. Hosp. tid., i, 26, 1929.
Sallmann. XIII Internaf. Conq., Amster-
dam, ii, 482, 1929.
Sallmann and Deutsch. A. f. O., cxxiv, 624,
1930.
S. d’O.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3387
Samojloff. K.M. Aug., lxix, 59, 1922; lxxiv, Thiel. K. M. Aug., lxviii, 244, 1922; lxx,
652, 1925. 766, 1923.
An. d’Oc., clxi, 523, 1924. A. f. O., cxiii, 329, 347, 1924.
Z. f. Aug., lviii, 214, 282, 1925. A. f. Aug., xcvi, 331, 1925.
Sédan, Bull. S. d’O. Paris, xxxix, 350, 1927. B. O. G. Heidel.., xlvii, 466, 1929.
Seidel. A. f. O., lxxxviii, 102, 1914; cii, Traquair. Introduction to Clinical Perimetry,
415, 1920; cxix, 15, 1928 ; czx, 417, London, 1927.
1930. A. of O., xxii, 949, 1939.
B. O. G. Heidel.., xliv, 79, 1924 : xlvi, 398, Tschenzow. Russ. O. J., i, 235, 1922.
1927. Waite, Derby and Kirk. T. O. S., xlv, 301,
Serr. A. f. O., czki, 3, 1928. 1925. -
Sheard. Am. J. O., ii, 185, 1919. A. of O., lv, 575, 1926.
Sinclair. T. O. S., xxv, 384, 1905. Weekers and Fauchamps. A. d’O., liii, 513,
Sloan. A. of O., v, 601, 1931. 1936.
Smith, D. P. Brit. J. O., ix, 233, 1925. Wegner. Z. f. Aug., lv, 381, 1925; lvi, 48,
Smith, Priestley. Brit. J. O., viii, 145. 1924. 1925.
Terson, Bull. S. fr. d’O., xxxviii, 664, 1925. Wessely. K. M. Aug., lxxix, 811, 1927.
Thibert. Bull. S. Belge d’O., xlv., 36, 1922. Zanettin. A m. di Ott., lix, 847, 1931.
Diagnosis
In congestive incompensated glaucoma the only disease which can give
rise to difficulty in diagnosis, particularly in acute cases, is iritis : the
differentiation between the two has already been discussed.” Apart from
this there may arise doubts as to whether the glaucoma is primary or
secondary, particularly to an intra-ocular tumour—a question which is
frequently insoluble if the inner eye cannot be examined until decompression
has been effective.
Occasionally the radiating headache and violent nausea and vomiting divert
attention to the abdomen, the patient being in too unhappy a condition to observe
that he has lost the sight of an eye ; in this way the diagnosis has been missed altogether
by the unwary.
Simple compensated glaucoma when well-developed and showing the
classical signs of raised tension, dilated sluggish pupil, cupped disc, and
restricted field, presents no diagnostic difficulties. The one clinical sign on
which doubt may arise is the cupping of the disc, for slight or atypical
cases occur in which differentiation may be difficult or impossible on this
sign alone. In any case, however, it is dangerous to make a diagnosis
solely on this feature, but the evidence which it supplies should be correlated
with that from every available source in forming a judgment ; and this is
sometimes only possible after repeated observations maintained over some
time. The following conditions may be mistaken for a glaucomatous cup —
1. A Physiological Cup. Here the depression is rarely deep and usually does not
involve the entire area of the disc ; the portion which is excavated is white in colour
but other areas appear pink. The cup does not show an overhanging edge and the
vessels can usually be traced from their first appearance without interruption until
they reach the fundus; and although the nasal vessels may show a distinct bend they
show no abnormal pulsation (Fig. 2860), The glaucomatous cup, on the other hand,
although it may start on one side, eventually involves the entire circumference and
the whole area of the disc, so that its sides are steep and not shelving. Difficult cases,
1 p. 2203.
3388 TEXT-BOOK OF OPHTHALMOLOGY
of course, arise, particularly when a physiological cup passes into a pathological one,
in which case differentiation may be an impossibility.
2. An atrophic cup is usually very shallow without the steeply overhanging edge,
and the vessels are normal : a differentiation is usually possible by the visual fields,
and particularly the colour fields which are disproportionately affected in atrophic
and not in glaucomatous states. Moreover, in glaucoma the light minimum is affected
and the light difference not, while in atrophy the reverse is usually the case.
3. A colobomatous cup is usually larger than the area of a normal disc and generally
gives the impression of an irregular funnel in emerging from which the vessels rarely
show regular kinking (Fig. 1162, Plate XI). In most of these congenital conditions
there are some congenital deformities elsewhere in the eye or its fellow, and the
vessels on the floor of the coloboma are frequently veiled with neuroglial tissue.
Moreover, if a peri-papillary halo is present, it is irregular and usually heavily
pigmented in a colobomatous eye (Stood, 1884; Zade, 1906; Rönne, 1921 ; A.
Fuchs, 1924–28). It is to be noted also that an obliquely inserted nerve-head may
produce an apparent bending of the vessels at the margin of the disc (A. Fuchs, 1924).
4. The conditions of pseudo-glaucoma (amaurosis with excavation : cavernous
atrophy) and atrophy due to Sclerosis of the cerebral arteries frequently present many
difficulties. As we have seen, in the former condition some help may be obtained
from the fact that the field for red is disproportionately constricted and the photo-
chromatic interval is greater than 5°. In most cases, however, reliance must be
placed on the constant absence of abnormal tension, and this can only be safely assumed
after the demonstration of a normal diurnal tension curve over a period of some days
and the ability of the eye to withstand more than one provocative tension test. These
questions will be discussed immediately.
The diagnosis of early simple glaucoma, on the other hand, is a problem
sometimes of the utmost difficulty, but always of the greatest importance.
Suspicious symptoms in border-line cases are rapidly increasing presbyopia,
headaches or fleeting obscurations of vision, and a feeling of fullness in the
eye which may appear on waking in the morning, after a stay in the dark
(as in a cinema), or after slight vasomotor upsets (as a warm bath, or the
taking of coffee or stimulants), and the occasional presence of halos. Three
early clinical signs are of importance : (1) the baring of the macula and
the appearance of small scotomata in the arcuate belt when the field is
explored by the 1/2000 test-object, or a wing-like enlargement of the blind-
spot to form Seidel’s scotoma if larger test-objects are used ; (2) the lowering
of the light sense which may be rapidly and satisfactorily gauged by
comparing the patient's rate of adaptation with the examiner's on the
minimal readings of a foot-candle-meter after both have sat 5 minutes in
the dark room ; (3) the appearance of a fine pigmentary powdering on the
iris. It is to be noted that cupping of the disc is a sign of well-established
glaucoma. -
The question of tension must now be considered and it cannot be
stated too strongly that in the diagnosis of early simple glaucoma the finding
of a normal—or even sub-normal tension—on one or even more occasions is no
criterion that glaucoma does not exist. An investigation of the diurnal variation
in tension should be undertaken tonometrically over a period of three days
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3389
while the patient is maintained in a regular mode of life, measurements
being taken two or three times a day, the first before the patient stirs in the
morning. Even although the tension never rises above the “normal ‘’
limits, a variation of 10 mm. Schiótz indicates latent glaucoma. If this
test is negative, or if it cannot be undertaken, certain provocative (or loading)
tests are available by which an abnormal rise of tension may be excited.
The object of the diurnal and provocative tests is to elicit any instability
in the ocular tension, for a normal eye has the capacity to maintain its
pressure in equilibrium despite considerable provocation, an adaptability
which the glaucomatous eye has lost.
Several such provocative tests have been advocated, based on the
abnormal reactions we have already considered.” Since, however, none of
them is universally applicable or absolutely reliable, it is always advisable
to apply more than one (Thiel, 1924–25; Gradle, 1931; Stein, 1933;
Gouterman, 1934; Ohm, 1936 ; and others).
1. The dark-room test of Seidel (1914–28). The patient, after having his tension
taken, is put in a dark room for 40 to 60 minutes, at the end of which time the tension
is taken again in dim light ; he then looks at a brightly illuminated wall or at a clear
sky. The tension rapidly falls to its former level. In the normal eye no great variation
of tension occurs ; a variation of over 6 mm. Schiótz is pathological. -
2. The decubitus test of Köllner (1916–20) and Thibert (1922). If the patient is
made to lie down, particularly with the head low, a rise of 6 mm. Schiótz indicates
glaucoma.
This test is best combined with the dark-room test to accentuate the combined
effect (Poos, 1934). In unilateral cases the more rapid dilatation of the pupil in the
dark in comparison with its fellow is significant (Gradle's test, 1936).
3. Blood-pressure Tests. The caffein test (Wegner, 1925–30; Löhlein, 1926). In
its simplest form the patient is given one or two cups of strong black coffee to drink :
in the normal eye there is no variation ; in the glaucomatous eye in from 20 to
40 minutes a rise of tension (which may be 15–25 mm. Schiótz) follows the rise in
blood-pressure. A similar reaction follows the intra-venous injection of 0-2 gm. caffein.
An inhalation of amyl nitrite has a similar effect.
4. The Venous Congestion Test. By lowering the head (Wegner, 1930) or con-
stricting the neck with a bandage so that venous congestion is produced but speaking
and swallowing is not affected, a rise of tension tends to occur in glaucomatous eyes
(Thiel, 1925). The tension is taken before the test commences, and then after con-
gestion has been maintained for an hour : an increase of more than 6 mm. Schiótz is
pathological. It is to be remembered that in the arteriosclerotic this test is not without
its dangers.
5. The drinking test of Marx (1925–28) applied to the eye by Schmidt (1928). The
patient drinks one quart of water before breakfast as rapidly as possible ; in patients
with the vasomotor instability typical of glaucoma the tension, taken every 15 minutes,
should rise from 6 to 15 mm. Schiótz in about half an hour, and at the same time the
blood haemoglobin and the quantity and specific gravity of the urine show an atypical
variation.
6. The Reading Test. The tension is measured ; the patient reads small print
concentratedly for 45 minutes, and in a glaucomatous eye the tension may rise some
10–15 mm. Schiótz (Gradle, 1931).
1 p. 3376.
3390 TEXT-BOOK OF OPHTHALMOLOGY
7. The adrenalin test of Knapp (1921). One drop of 1 : 1000 solution of adrenalin
hydrochloride repeated at minute intervals for 5 minutes dilates the pupil indicating
sympathetic excitability. It should not do so normally.
8. The massage test of Knapp (1912). In the normal eye 1 minute's deep massage
of the globe, or the resting of a weight (250 gm.) upon the closed lid for 10 minutes,
should lower the tension to one-third or one-half of its normal value ; in 60 to
70 minutes it should return to normal. In the glaucomatous eye the tension falls less
or not at all : it returns to its normal level more rapidly (30 minutes), rises above this
level, and only regains it in 13 hours (Dieter, 1928). As this indicates deficient drainage
it is an indication for operative interference (Gradle, 1931).
9. The paracentesis test of Kronfeld (1930–33). This, like the preceding, measures
reactive hypertonia. The rise of tension after paracentesis should be to a height of
some 25–35 mm. Schiótz : in glaucomatous eyes it may be 40 to 60 mm. or more
(Magitot, 1931; Lin Ching K’uel, 1936).
10. The Mydriatic Test. Mydriasis should not raise the tension of a normal eye ;
it may do so to a considerable extent in the glaucomatous. The most suitable mydriatic
is euphthalmin, which acts promptly and is easily controlled if hypertension develops.
11. The fluorescein test of Thiel (1922–24). Sodium fluorescein is given the patient
by mouth (as in coffee), whereafter in iritis and glaucoma a fluorescence may be
visible in the pupil with the slit-lamp. The test indicates an increased capillary
permeability.
It is to be remembered that these tests, while fairly conclusive if positive,
mean nothing if negative, for they do not occur universally, nor is there a
uniform response with the same patient at different times; their value lies
in the evidence they bear in conjunction with other factors. While eliciting
them, hospitalization of the patient is advisable, if for nothing else than
to stabilize him during the initial investigation of the diurnal variation.
This test should be done first, and if necessary may be followed by the
dark room and decubitus test, the caffein test, the massage test, and perhaps
the drinking test, each at considerable intervals. If these are all negative
or inconclusive, the mydriatic test may be tried ; and if this also is negative
that patient may be pronounced non-glaucomatous.
Cordes. A. of O., xvii, 896, 1937.
Dieter. A. f. Aug., xcix, 678, 1928.
Fuchs, A. Am. J. O., vii, 425, 1924.
Brit. J. O., xii, 65, 1928.
Gouterman. A. of O., xii, 407, 1934.
Gradle. Am. J. O., xiv, 936, 1931.
Berens' The Eye and its Diseases, Phila.,
699, 1936.
Rnapp, A. T. Am. O. S., xix, 69, 1921.
A. of O., l, 556, 1921.
Rnapp, P. K. M. Aug., 1 (1), 691, 1912.
Köllner. A. f. Aug., lxxxi, 120, 1916;
lxxxiii, 135, 1918; lxxxvi, 114, 1920.
FCronfeld. Z. f. Aug., lxxi, 58, 1930.
A. of O., v, 674, 1931; ix, 801, 1933.
Lin Ching K’uel. Chinese Med. J., l, 1323,
1936.
Löhlein. K. M. Aug., lxxvii, 909, 1926.
Magitot. An. d’Oc., clxviii, 801, 1931.
Marx. Kl. W., iv (2), 2339, 1925; v (1), 92,
1926; vi (2), 1750, 1927.
Marx (contal.). Deut. f. A. kl. Med., clii, 354;
cliii, 358, 1926; clviii, 149, 1928.
Ohm. A. f. O., cxxxv, 537, 1936.
Poos. B. O. G. Heidel., 1, 73, 81, 1934.
Rönne. A. f. O., ev, 465, 1921.
Schmidt. Med. Kl., xxiv, 859, 1928.
Seidel. A. f. O., lxxxviii, 102, 1914; czix,
15, 1928.
Serr. A. f. O., czzi, 3, 1928.
Stein. Med. Kl., xxix, 1235, 1933.
Stood. K. M. Aug., xxii, 285, 1884.
Thibert. Bull. S. Belge d’O., xlv., 36, 1922.
Thiel. K. M. Aug., lxviii, 244, 1922; lxx,
766, 1923.
A. f. O., cziii, 329, 347, 1924.
A. f. Aug., xcvi, 34, 331, 1925.
Wegner. Z. f. Aug., lv., 381; lvi, 48, 1925.
A. f. Aug., cii, 1, 1929; ciii, 303, 511, 1930.
Zb. ges. O., xxiv, 1, 1930.
Zade. K. M. Aug., xlv. (2), 435, 1906.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3391
Treatment
A. General Principles of Treatment
Since any rational system of treatment should be directed primarily
against the cause of the disease and secondarily against the manifestations
of its symptoms, the treatment of primary glaucoma must still be considered
very unsatisfactory. It is true we have progressed far beyond the state of
affairs obtaining before the introduction of iridectomy by v. Graefe (1857),
when a diagnosis of glaucoma was tantamount to a sentence of blindness ;
but we still have a long journey to travel. In summarizing the aetiology of
primary glaucoma we concluded that of the four potential factors probably
entering into the question, two—the central controlling mechanism and the
tissue-aqueous barrier—were quite unknown and wholly outside the realm
of therapeutics as medicine stands to-day. The third—the vascular factor
—whether it involve sclerosis or vaso-neurosis, can only be attacked by
flank movements in vague and indefinite ways, since we are up against
problems of great complexity the intimate mechanism of which is little
understood, in the first case the fundamental degenerations resulting from
the wear and tear of living, in the second, the vagaries of the endothelial,
the neuro-vegetative, and the endocrine systems. Some of these are
susceptible to amelioration, few of them to relief, and none of them to cure.
The remaining factor—that of deficient drainage—can alone be tackled
seriously, and to this, perforce, the treatment of primary glaucoma is still
essentially confined ; but in so far as it usually constitutes only an adjuvant
factor in the aetiology, the disease, with its fundamental background still
undisturbed, often has a tendency to progress despite our efforts.
In general terms, therefore, it may be said that the available treatment
of primary glaucoma devolves into an attempt to correct the more funda-
mental causes in so far as they can be traced by regulating the patient’s
hygiene and habits and counteracting any constitutional diathesis, while
at the same time maintaining the tension of the eye within normal limits
by medicinal methods. If these fail and the tension remains high or shows
a dangerous instability, or if a deterioration of function persists, operative
measures must be undertaken for its relief. On the different methods of
treatment there is a confusing diversity of views, and it would seem most
economical in space to summarize the more important of these first, and
then to give a logical and workable scheme for the various clinical types.
B. Medical Methods of Reducing Tension
I. Pharmacological Methods
It is customary to divide the various drugs employed in the treatment
of glaucoma into two classes : (1) miotics (pilocarpine, etc.), the primary
1 p. 3362.
T.O.-WOL. III. 4 O
3392 TEXT-BOOK OF OPHTHALMOLOGY
action of which was considered to be upon the musculature of the iris ; and
(2) those having a vascular basis (adrenalin, etc.). It is probable, however,
that this division is to some extent artificial, for while the first class un-
doubtedly act to a considerable extent by the mechanical action of freeing
the angle of the anterior chamber, it is certain they all have a similar
fundamental vascular action which, as in the case of adrenalin, may be
effective even with a coincident mydriasis. In general terms this reaction
is a mild dilatation of the capillary bed resulting in an acceleration of the
circulation thereby relieving stasis, flushing away waste-products, altering
the conditions of the fluid-traffic of the eye, and promoting the absorption
of accumulated intra-ocular fluid, thus combating a state of static oedema.
(1) THE MIOTICs
|Miotic drugs, therefore, which behave as supplementers of the para-
sympathetic (autonomic) system, act in two ways: (1) by combating
vascular stasis and oedema ; (2) by inducing miosis.
The existence and efficacy of the vascular factor in the action of miotics
is demonstrated clinically by the facts that the hypotensive reaction of
these drugs does not vary with the degree of miosis produced, that it may
be evident in cases where the angle of the anterior chamber has been
demonstrated gonioscopically to be open, and that its effectivity is still
apparent, although not to the same degree, when miosis cannot be effective
owing to complete anatomical obliteration of the angle, or when it is
impossible, such as in cases of aniridia or when the pupil is completely
tied down by posterior synechiae (Köllner, 1920; Thiel, 1924–28 ; Magitot,
1929; and others). This vaso-dilatatory reaction of pilocarpine and eserine
has been amply demonstrated in experimental animals (Duke-Elder, 1931),
and has been verified clinically by the observation of capillary dilatation
in the conjunctiva (Michail and Vancea, 1926), by the altered rate of the
peri-foveal circulation as seen entoptically (Hess, 1920), and by the increased
vascular pulse registered tonometrically (Kadlicky, 1922); moreover, an
indication of the increased circulatory flow in the eye is suggested by the
experiments of Gala (1927), who found that sodium iodide, which normally
is present in the aqueous for some time after its injection into the general
circulation, is not found after the instillation of pilocarpine or eserine.
Owing to this vaso-dilatation these miotic drugs tend to produce a slight
rise in pressure in the normal eye (Duke-Elder, 1931); but in the circulatory
stasis of glaucoma the reaction is reversed and the state of nutrition
improved.
The miotic action varies with the drug employed. We have already
seen that the para-sympathetic (autonomic) nerves do not act upon the
muscle-fibres directly, but become effective through the liberation in situ
of acetyl-choline, which in turn, acting on the muscle-fibres, stimulates them
1 Vol. I, p. 530 (539).
ANOMALIES OF THE INTRA-00ULAR PRESSURE 3393
to contraction: normally, in order to maintain function within physiological
limits, the acetyl-choline, immediately it has done its work, is destroyed by
Fig. 2880.-Norm AL ANGLE. ON RAPID Fixation AFTER DEATH.
C.A. Anterior chamber. S. Canal of Schlemm (Fortin).
Fig. 2881-THE ANGLE on RAPID Fixation UNDER INFLUENCE of Eserise.
The pull of the ciliary muscle has opened out Schlemm's canal (Fortin).
a ferment, acetyl-choline esterase: these reactions have been demonstrated
in the iris and ciliary body (Englehart, 1931). Of the commoner miotic
drugs, pilocarpine and histamine stimulate the muscle-fibres directly;


4 O 2
3394 TEXT-BOOK OF OPHTHALMOLOGY
eserine and prostigmin act by inhibiting the esterase and allowing the
normally formed acetyl-choline to react uninterruptedly; the choline
derivatives act by supplementing the natural supplies of acetyl-choline.
The miosis thus produced becomes effective in several ways:–
(a) The contraction of the iris increases mechanically the efficiency of
the drainage angle by opening it out (Weber, 1877) (Figs. 2880–81).
(b) The contraction of the ciliary muscle opens out the tissues of
the angle and the canal of Schlemm by its pull upon the scleral spur
(Figs. 2880–81).
(c) The absorptive surface of the iris is increased (Hamburger, 1925).
A. (d) Since the long posterior ciliary
--tº-
- - - -
arteries run through the belly of the ciliary
muscle, the fibres of which surround them
sphincter-like, and since the origin of the
-
-
_
muscle is distributed over the external
surface of the choroid, its contraction, by
tending to occlude the arteries, will tend to
º keep down a high blood-pressure in the
anterior segment of the eye, and, by
opening up the choroidal veins, hasten
the venous return (Küsel, 1906; Fortin,
1929) (Fig. 2882). The miosis itself thus
aids in overcoming stasis.
Seidel (1922), it will be remembered, con-
sidered that these drugs acted essentially by
modifying the secretion of the intra-ocular fluid.
(a) PILOCARPINE, which was first intro-
duced into ophthalmic practice by Weber
(1877), and acts by stimulating end-
organs supplied by the autonomic nerves,
is the most generally useful of the miotics:
that it acts on the muscle directly is seen in the persistence of its effectivity
after complete degeneration of the nerves and nerve-endings has occurred
(Schoenberg, 1938). With the exception of acute and absolute cases it
tends to lower the tension of almost any glaucomatous eye, to some degree
although not necessarily sufficiently (Löhlein, 1926), and in addition lessens
the amplitude of variations in the tension (Thiel, 1928; Odinzow and Nejman,
1929).
Fig. 2882.-SECTIon THRough
the CILLARY MuscLE.
A. A long posterior ciliary artery
surrounded by fibres of the muscle
(Fortin).
It may usually be given over long periods with little intolerance or irritation,
although an allergic conjunctival reaction may eventually occur. It is usually given
as drops, best as pilocarpine nitrate (or chloride) in 0-5% or 1-0% solution, which
produces a miosis lasting about 6 hours: the action may be prolonged by preseribing
it as oily drops in parolein, or as an ointment in a vaseline base.


ANOMALIES OF THE INTRA-OCULAR PRESSURE 33.95
(b) HISTAMINE. Histamine, a much more powerful miotic and vaso-
dilator than pilocarpine, was first used by Dieter (1925), who found a fall
in the ocular tension associated with hyperaemia after the sub-conjunctival
injection of 0-5 to 1.0 mg. A related synthetic substance, amino-glavcosam
(beta-imidazolethylamine), was introduced by Hamburger (1926) which
could be given as drops ; while v. Hofe (1928) advocated a combination of
histamine and thyramine (tenosin). These drugs were claimed to induce
miosis in acute glaucoma, ; but perhaps because of the severe vascular
reaction, the hypotensive effect is not uniformly successful nor is their use
free from pain and complications (Gapejeff, 1928; Duke-Elder and Law,
1929; Moscardi, 1929; Ellett and Rychener, 1929; Castresana, 1929;
Post, 1934). They cannot replace pilocarpine but may produce a miosis
in acute glaucoma when pilocarpine fails.
(c) EserINE (PHYSOSTIGMINE), the first miotic to be introduced, by
Laqueur (1876), acts by inhibiting the destruction of the acetyl-choline
formed by stimulation of the third nerve : the musculature therefore becomes
hyper-excitable, liable to go into spasm at the slightest stimulus, thus
causing headache and discomfort. It produces a more powerful and longer
miosis than pilocarpine, lasting some 12 hours, while the irritable state may
persist for some days.
It is usually prescribed as drops of the salicylate in 0.2–0.5% solution, but it
tends to deteriorate, particularly if exposed to light, warmth and air. Its consistent
use frequently leads to a chronic irritable allergic conjunctivitis, which, while it may
be postponed or mitigated by the use of fresh sterile solutions only, and by washing
out the conjunctival sac with saline half an hour after its use, often precludes its
employment over long periods of time and necessitates alternations with other miotics.
It is essentially a drug for periodic or emergency use.
(d) PROSTIGMIN, a drug which, like eserine, inhibits the destruction
of acetyl-choline, has been employed as drops in 4 to 5% solution by Clarke
(1939) both in acute and simple glaucoma. It is claimed to be better than
eserine in that it does not deteriorate, causes less unpleasant symptoms, and
has a stronger and more prolonged action.
(e) CHOLINE DERIVATIVEs. The choline derivatives supplement the
contractile action upon the muscle fibres of the acetyl-choline normally
formed on stimulation of the para-sympathetic nerves : they therefore act
only in conditions wherein eserine is effective. In addition to having a
very powerful miotic action, acetyl-choline has an extremely powerful
vaso-dilator effect, which produces a fall in the general blood-pressure :
all its activities are neutralized by atropine. Since, however, the action is
violent and very short-lived, derivatives have been employed ophthalmo-
logically since Passow’s (1929) original suggestion ; none of them, however,
is of long standing and their use has not yet been standardized.
(i) Mecholyl (hypotam) (acetyl-beta-methylcholine) is a very powerful miotic and
produces an immediate ocular hyperaemia; it has been found very effective in simple
3396 TEXT-BOOK OF OPHTHALMOLOGY
glaucoma when employed as drops in 5% solution (Villaret, Besançon and Gallois, 1981;
Onfray, Aberloos and Suys, 1934). Clarke (1939) found it useful in acute glaucoma,
given as a 20% retro-bulbar injection, to avoid further congestion and sub-conjunctival
haemorrhage in an already congested eye ; and Gradle (1940) found it most effective
used as drops in a 20% solution in combination with a 5% solution of prostigmin.
He found that this combination reduced the tension to normal in difficult cases of
secondary glaucoma wherein other miotics and ephidrine had failed.
(ii) Doryl (carbaminoylcholine), which was introduced by Velhagen (1933), is a
potent miotic, contracting the isolated sphincter in hormonic doses (1 in 4,000,000,000).
It is used as drops in 0.75% solution and is particularly effective in producing miosis
and lowering the tension in simple glaucoma (Velhagen, 1934 ; Miloro, 1935; de
Sanctis, 1937). The vascular reaction produced renders its use inadvisable in con-
gestive glaucoma.
(iii) Iricoline (carbaminoyl-methylcholine), used as drops in 1–2% solution, is
claimed as a still more active product (Magitot, 1939).
Synergic Miosis. The possibility arises of using these drugs in com-
bination so that the effect of one will augment that of the other synergically.
Thus pilocarpine and eserine supplement each other, the first acting on the
muscle fibres, and the second increasing the effectivity of the normal
stimulatory mechanism : this latter effect can be further augmented by
adding a choline derivative. Leaving out the histamine group with their
violent circulatory reaction, the most powerful miotic combination is
therefore pilocarpine to stimulate the muscle, doryl, mecholyl or iricoline
to supplement the normal product of nervous activity, and eserine or
prostigmin to preserve the effectivity of the choline normally formed or
artificially introduced ; to these might be added an injection of ergotamine
tartrate to paralyse the sympathetic and weaken the opposing action of the
dilatator.
(2) SYMPATHETICO-TONIC DRUGS : ADRENALIN
The action of adrenalin has already been studied,” when it was pointed
out that in the normal eye it causes a dilatation of the pupil and a variation
in the tension consisting of an initial lowering Owing to vaso-constriction
followed by a rise owing to the effects of an increased general blood-pressure :
subsequently after about an hour there is a reactive capillary dilatation and
an increased flow of blood, which may produce a rise in pressure. In many
glaucomatous eyes the same initial reaction is seen, but in the subsequent
reactive hyperaemia the pathologically raised tension frequently falls, the
fall beginning in about an hour and reaching its maximum in between
12 to 24 hours : this stimulus to the circulation is therefore exactly com-
parable to that immediately following the exhibition of miotics (Kadlicky,
1924; Thiel, 1924; Bailliart, 1927; Ungerer, 1929). The beneficent
reactive hyperamia, which abolishes capillary stasis and oedema, tends
to occur in simple glaucoma ; but when the capillaries are already atonic
1 Vol. I, p. 512 (521).
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3397
and dilated, they enter upon a refractory phase and do not respond by an
initial constriction, but may even dilate further (Duke-Elder, 1931). It
follows that instead of the induction of a beneficent hyperaemia, stasis and
oedema are increased, a circumstance which, when the effect of mydriasis
is added, makes the drug unsafe to use in congestive chronic glaucoma and
dangerous in acute glaucoma. Not a few instances have been reported
wherein catastrophe has resulted from its use in such cases (Gifford, 1928;
Duke-Elder and Law, 1929 ; Horner and Bettman, 1936 ; Howell, 1936 ;
Wiener and Alvis, 1937). Even in chronic primary glaucoma such accidents
have been reported as a rarity, requiring paracentesis and even iridectomy
for their control (Howell, 1936). Most, however, appear to be associated
with the mydriasis and are readily controlled by eserine ; this drug in no
way lessens the effect of adrenalin on the tension, and it is wise to instil
| 2 3 4. 5 6 7 8 9 |O
S DAY NIGHT 2 2: 2
S. — — —
º ESERINE – 5 # #
C
JC ! LL LL! Lll
< CI CC OC
: º º º
= 50
º
.9
É
CD
F 20
|O
FIG. 2883.−THE EFFECT OF ADRENALIN ON CHRONIC PRIMARY GLAUCOMA.
it at half-hour intervals for the first two hours of the adrenalin treatment
in order to lessen pupillary dilatation.
The action of the adrenalin group of drugs is therefore somewhat
unreliable and should be confined to simple primary glaucoma, when they
should be given with eserine and the effect on the tension observed for some
hours before dismissing the patient ; their use in chronic congestive glaucoma
is questionable, and in acute glaucoma dangerous. In simple glaucoma their
indications are that they are sometimes effective when miotics fail, so that
if operation is contra-indicated they may tide over a crisis. Again, if a
patient leading a miotic life is going down-hill, periodical short courses of
adrenalin seem to stimulate the circulation sufficiently to allow the ordinary
miotics to keep the disease in check (Fig. 2883). Finally, the mydriatic
effect may be useful to improve vision when the lens is sclerosed centrally
or to facilitate Ophthalmoscopic examination when the pupil is small.
They are of great value in secondary glaucoma as mydriatics to break down
posterior synechiae.
(a) Adrenalin. The natural adrenalin was first employed ophthalmologically
by Darier (1900), whose interest had been stimulated by the physiological experiments

3398 TEXT-BOOK OF OPHTHALMOLOGY
of Wessely." His observations were followed by the early trials of MacCallan (1903)
and others, but the first extended clinical and experimental researches were due to
Erdmann (1913–14), which were followed by the reports of Köllner (1918–20) and
Knapp (1921). Thereafter the papers of Hamburger (1923–24), advocating the
method with great vigour, stimulated more widespread attention (Gradle, 1924;
Heinmann, 1924; Kafka, 1924; Safar, 1924; Thiel, 1924–25; and many others).
Adrenalin solution (1 in 1,000) may be employed in several ways—
. As drops (Knapp, 1921 ; 1 in 100 sol. Barkan and Maisler, 1937).
. As a sub-conjunctival injection (Hamburger, 1923–24).
. Soaking a pledget of cotton-wool inserted in the fornix (Gradle, 1925).
. As an ointment (Thiel, 1926).
. As a retro-bulbar injection with novocaine (Fromaget, 1921; Bailliart and
Bollack, 1921 ; Aubaret, 1923; Liebermann, 1923; Bonnefon, 1924 ;
Friedenwald, 1932).
(b) Glaucosan. Following the widespread use of adrenalin, however, reports
accumulated of disturbing general effects, such as palpitation, dizziness or collapse in
hypertensive patients or in those suffering from sympathetico-tonia or thyrotoxicosis
(Mans, 1924; Rentz, 1924; Ring, 1925). Hamburger (1925) therefore introduced a
synthetic drug—a combination of the relatively inactive dextro-epinephrine with a
product formed in its synthesis, and called it glaucosan : this he subsequently (1926)
made stronger so that it could be used as drops by substituting the more active lavo-
isomer in loºvo-glaucosan.
(c) Epinephrine bitartrate in drops as a 2% solution (or as an ointment) is now
most generally employed, as being cheapest, most readily obtainable and equally
effective (Green, 1931; Post, 1934; Howell, 1934–36; Cordes and Harrington, 1935;
Horner and Bettman, 1936 ; Howell, 1936 ; Wiener and Alvis, 1937; and others).
An extract of supra-renal cortex (cortin) was claimed by Josephson (1935) to be
singularly effective in glaucoma by inducing a diminution of capillary permeability, a
claim, however, which has not been substantiated (Woods, 1935; Cordes, 1937).
:
(3) syMPATHETICO-PARALYTIC DRUGS : ERGOT
With a view to reducing sympathetic activity, and believing that
glaucoma was essentially due to over-activity of the sympathetic nervous
system, Thiel (1924) suggested its treatment by the ergot group of drugs :
for this purpose he employed ergotamine tartrate (gynergen). It may be given
by mouth in 1 mg. tablets three times a day for some days, by sub-cutaneous
injection, or by sub-conjunctival injection. It has been reported as being
of some value in restraining the progress of simple glaucoma (Heim, 1927;
Hanssen, 1928; Poos and Santori, 1929; Werner, 1931; Gapeeff, 1932).
(4) CALCIUM has been recommended in the treatment of glaucoma, since it is
thought to reduce sympathetic vasomotor instability and diminish capillary per-
meability. It has been given for simple glaucoma, usually as the chloride by mouth
in large doses (15 gm.) for some months (Weekers, 1912–23; Gouterman, 1929),
or sub-conjunctivally (Chiari and Januschke, 1911; Kleiber, 1922). Abadie (1929)
favoured the combination of epinephrine, ergot and calcium chloride by mouth,
together with pilocarpine drops. The reports of calcium treatment, however, have
not been convincing.
1 Vol. I, p. 512 (521).
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3399
A calcium-magnesium compound, injected intravenously, was said to reduce the
tension by Gallois and Viard (1934).
Pancreatic eactracts and insulin have been credited with lowering the intra-ocular
tension (Accardi, 1925; de Jongh and Wolff, 1925; Kadlicky, 1926); pituitrim by
Samojloff (1927) and Taratin (1928); and a host of other remedies.
II. Physical Methods of Reducing the Tension
(1) Osmotic Treatment. It has already been pointed out 1 that the
intra-ocular pressure may be lowered by producing a hypertonic state of the
blood. The effect is due to the abstraction of fluid from the eye owing to
the general osmotic depletion of tissue-fluid. This effect is temporary, the
lowering of the tension appearing after an hour or so and persisting some
24 hours (Fig. 2884). The method is therefore only of emergency value as,
for example, a pre-operative measure, when its effect may be invaluable,
:
t
C)
C
acº
50
40
3O
2O
|O
2 4 6 8 10, 12 14- )
Time in hours.
FIG. 2884.—THE EFFECT OF HYPERTONIC SALINE INTRAVENOUSLY IN PRIMARY
GLAUCOMA.
or as a means of tiding over a crisis by lowering the tension in an acutely
affected eye on which operation is not advisable (as in secondary glaucoma)
and reducing it to a condition wherein it will respond to miotics, in which
case its effect may be dramatic. It should not, however, be employed in
the hyperpietic, or in those cardio-renal cases with retention of chlorides
who are already waterlogged.
Such an attempt was first made by Cantonnet (1904), who advised
sodium chloride by mouth ; Thomas (1915) gave sodium carbonate and
chloride by mouth and rectum. Intra-venous injections of salt were
employed by Hertel (1913–15), Pletnewa (1923), Weekers (1923), Duke-
Elder (1926), Lambert and Wolff (1929), Palmieri (1936) and others.
Glucose was employed intra-venously and rectally by Sansum (1917), sucrose
by Dyar and Matthew (1937), and, owing to its relative indiffusibility and
consequent prolonged action, sorbitol by Bellows, Puntenney and Cohen
1 Vol. I. p. 507 (516).







3400 TEXT-BOOK OF OPHTHALMOLOGY
(1938). Solutions of gum acacia were found effective by Gradle (1940).
After the injection, of course, if its maximal value is to be obtained, the
patient should be forbidden to drink water.
Sub-conjunctival injections of concentrated solutions, such as sodium citrate,
are of little value.
(2) Massage has always been employed as an aid to lowering the tension
in glaucoma since the demonstration of its effectivity by Pagenstecher (1878).
It is to be remembered, however, that its value lies only in the extent to
which it forces intra-ocular fluid out of the eye, and if the drainage channels
are ineffective the immediate lowering of tension may be small and the
subsequent reactive rise inopportune." For this reason it is an excellent
post-operative measure when a filtering scar should be kept open.
The best massage is digital, which the patient soon learns to do by himself (Pagen-
stecher, 1878), mechanical contrivances, such as vibratory (Maklakoff, 1893) or pneu-
matic (Domec, 1906) or electric (Hallett, 1927), are unnecessary.
(3) Long- or Short-wave Diathermy is sometimes of considerable value in
lowering high tension, and although the effect is temporary, may be effective
in tiding over a crisis or relieving the tension preparatory to operation
in acute congestive cases (Maddox, 1933; Law, 1933; Martin, 1935; Lloyd,
1938–39; Phillips, 1939; and others).
(4) The constant galvanic current has been suggested in acute cases of hypertension,
either employed on the eye to which the negative pole is applied, or to the cervical
sympathetic (Allard, 1900; Panas, 1902). It is probably valueless.
(5) Röntgen rays have been used in cases of absolute glaucoma. They may be of
value in relieving pain, but the tension remains unchanged (Lloyd, 1927; Kreibig,
1936).
C. Surgical Methods of Relieving the Tension
Any operation devised for the relief of glaucoma should ideally be such
as to preserve the function of the eye, maintain its tension within normal
limits, and retain the integrity of the globe. The number of operations
advocated from time to time is evidence that this ideal has never been
attained. This state of affairs will remain until our ideas regarding the
aetiology of glaucoma are clarified, for the operative technique should ideally
vary with the type of disease present in each particular case and the stage
which it has reached. All of them are imperfect, in that all of them are
liable to affect the visual function and allow a return of hypertension,
while most of them leave the integrity of the eye considerably impaired.
In this section we shall merely outline the various operative procedures, indicating
their applicability, their advantages and disadvantages: the details of technique will
be dealt with in the section on Operative Surgery.”
1 p. 3378. 2 Vol. IV.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3401
l. IRIDECTOMY
The classical basal iridectomy of v. Graefe (1857–69), the introduction of
which revolutionized the treatment of the disease, must always take pride
of place in any discussion on the operative treatment of glaucoma. In it
a considerable sector of the iris is removed up to the root. The rationale of
the reduction of the tension is still a matter of controversy, and many
theories have been advanced since v. Graefe’s original suggestion that
removal of part of the iris diminished the secretion of the aqueous. The
most universally accepted theory, suggested by Weber (1877) and illustrated
anatomically by Collins (1894), ascribes its main effect to the mechanical
opening up of a closed angle of the anterior chamber, and it is generally
held that the operation is successful only in so far as it does this and the
iris is removed so completely that the angle cannot be blocked again.
Gonioscopic studies, however, show that this cannot be the entire explanation,
for the base of the iris is left behind in many cases; if it is, the result may
be good, and if it is totally removed, the result may be bad (Troncoso,
1925–34 ; Werner, 1931; Sonales, 1937). Other suggestions have been
put forward : that a fresh absorbing surface is presented to the aqueous
(Ulrich, 1884) which, since a wound in the iris does not cicatrise (Fuchs
1896; Henderson, 1907; McBurney, 1914), remains permanently ; that a
filtering scar is formed accidentally with or without the incorporation of
uveal tissue (de Wecker, 1867); or that vasomotor reflexes are cut
and abolished (Abadie, 1910). Until we know what causes glaucoma it is
difficult to be dogmatic on the matter.
There is essentially one indication only for iridectomy in primary glaucoma—in
acute cases which do not respond rapidly to medical treatment. Statistics vary
greatly as to its effectivity in this, but those compiled by Schmidt-Rimpler (1908)
from the literature representing the results of many surgeons at a time when no
alternatives were available, are interesting : the successes in acute glaucoma were
80%, in chronic congestive glaucoma 53%, and in glaucoma simplex 33%. Most
statistics, however, give lower figures than this, and a good average for successes in
acute glaucoma is 60% (Wilmer, 1927; Ploman and Granström, 1932; and many others).
v. Graefe’s classical technique has survived to the present day almost without
change. Bowman (1862) recommended cutting the two pillars of the iridectomy
only and tearing the iris away at its root in order to ensure its complete removal. To
avoid the difficulties of entering a narrow anterior chamber and the dangers of damage
to the lens and of suddenly opening into the eye, Elschnig (1928) suggested a scratch
incision with a keratone (incisio ab externo) under a conjunctival flap. Butler
(1932–36) confined himself to a peripheral basal iridectomy undertaken through a
trephine opening, the disc being left on a hinge ; while in chronic glaucoma with a
closed angle and a narrow anterior chamber, Barkan (1939) practised multiple peripheral
iridectomies through small limbal punctures.
2. Operations to establish Eartra-ocular Drainage
(a) SCLEROTOMY. Historically these operations derive from the
suggestion of Mackenzie (1830) of performing a corneal paracentesis or a
3402 TEXT-BOOK OF OPHTHALMOLOGY
posterior sclerotomy. While being useful in secondary inflammatory
glaucoma, the former has no place in primary glaucoma ; but posterior
Sclerotomy is of value in cases of grave hypertension in acute primary
glaucoma, both to relieve tension temporarily as an emergency measure, and
as a pre-operative measure to encourage the formation of an anterior chamber
when its shallowness makes operation technically difficult (Priestley Smith,
1894). de Wecker (1867), however, advocated sclerotomy with a view to
establishing a permanent filtering scar, and to this end made a puncture and
counter-puncture in the sclera 1 mm. behind the limbus, cutting up a little
way as if making a cataract section before the knife was withdrawn.
Repeated operations, however, were necessitated by the invariable closure of
the wound, and even although the operation was combined with iridectomy
(1901) and the eye was treated with massage (Dianoux, 1905) it was
perforce abandoned.
Several modifications were proposed from time to time in order to
maintain drainage, the most satisfactory of which is the small flap sclerotomy
of Herbert (1907–13), which can be combined with an iridectomy. In this
operation a small incision is made into the anterior chamber parallel to the
limbus, at either end of which two perpendicular cuts are made towards the
corneal margin, thus leaving a rectangular trap-door of sclera with its base
attached to the cornea. Cruise (1921), adding slight modifications, insisted
on the value of post-operative massage.
In a second device Herbert (1913) isolated a wedge of sclera at the limbus so that,
attached to the conjunctiva only, it shrivelled up—the wedge-resection of Herbert.
As a general rule more permanent drainage operations than Sclerotomy are
desired, but the sclerotomy of Herbert has definite indications in simple glaucoma in
that it reduces a high tension gradually, a point of particular value in advanced
cases; moreover it involves little trauma, no permanent disability and can be repeated
as often as desired. It is therefore suitable for frail subjects.
(b) SCLERECTOMY, whereby a piece of the sclera is excised, provides
drainage of a much more permanent nature. The idea was first put into
practice by Argyll Robertson (1876), who trephined a disc of sclera behind
the ciliary body in the hope of relieving the pain of absolute glaucoma.
The credit for the introduction of an operation of this type, however, to
fulfil de Wecker's aspiration to establish a filtering scar of a permanent
nature, belongs to Felix Lagrange (1905).
(i) Lagrange's sclerecto-iridectomy (1905–07) consists in the cutting out
of a small corneo-scleral-conjunctival flap upwards, snipping away a
piece of the corneal lip of the wound with scissors, performing an
iridectomy and replacing the flap so that the aqueous drains freely into
the sub-conjunctival tissues. In order to make a cleaner aperture Holth
(1909) performed the sclerectomy on the corneal lip of the wound with
punch forceps.
(ii) Trephining. (a) Posterior Trephining. Fergus (1909) revived
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3403
Argyll Robertson's idea by opening into the sub-choroidal space with a
trephine under a conjunctival flap, so that the opening lies on the ciliary
muscle, and improved upon it by introducing a spatula through it into
the anterior chamber, thus combining the sclerectomy with a dialysis.
Such an operation is frequently effective in recalcitrant cases (Sallmann,
1935; Ivanov, 1937; Lauber, 1939). Young (1924) advised a double
sclerectomy in this region, removing a portion of the muscle in so doing.
(b) Corneo-scleral Trephining. Trephining has become popular
through the corneo-scleral trephining of Elliot (1909–32). This operation
consists in cutting a trephine hole at the corneo-scleral margin into the
anterior chamber under a conjunctival flap, and then performing an
iridectomy at the trephine hole ; the principle is thus the same as in the
operation of Lagrange. It has been widely used without serious
modification.
Although, like most other operations, both these types of Sclerectomy are most effec-
tive in chronic glaucoma in the lower ranges of hypertension, they have a wide applica-
bility. This applies particularly to trephining which may be used in the sub-acute
or chronic stages of congestive glaucoma, and in all stages of simple glaucoma. More-
over, given equal technique, Lagrange's operation is more difficult technically, involves
more trauma to the eye, and probably tends more readily to the Subsequent production
of cataract. Elliot's operation also involves very considerable trauma to the eye,
and may be followed by delay in the re-formation of the anterior chamber with its
ultimate sequel of cataract, persistent hypotony, a recurrence of tension due either
to dense scarring with endothelial proliferation in the aperture (Collins, 1914) or its
blockage by an anterior dislocation of the ciliary body (Troncoso and Reese, 1935),
or the occurrence of the tragedy of late infection through the thin conjunctival bleb.
This unfortunate sequel, which usually appears some months to some years after
operation, has already been discussed," and has been variously estimated. Reviewing
the literature, Scardapane (1926) found an average incidence of 2.27% of late infections
after Elliot's trephining, in 44% of which the eye was retained and in only 4% did
useful vision result. The reports, however, vary widely, different surgeons reporting
percentages of from 0 to 13-6 (Erola, 1934, in an analysis of 5,616 trephines). Reviewing
536 operations performed at Moorfields, Davenport (1926–27) found 14 cases of late
infection of which 6 retained good vision and 2 required evisceration. Fewer late
infections are reported after Lagrange's sclerectomy (1.54%, Scardapane, 1926).
Whatever the actual danger, this complication has been the chief cause of a waning in
enthusiasm for the operation of trephining in recent years. An average figure for
failures (that is, recurrence of tension or persistent loss of vision) may be taken at 25%
(Wilmer, 1927; Knapp, 1933).
(c) IRIS-INCLUSION OPERATIONS. The first to suggest the intentional
inclusion of the iris in the wound to maintain filtration was George Critchett
(1858) who brought the iris into a corneal incision in his operation of iridodesis.
Such an unprotected inclusion, of course, was liable to infection. Some of
the earlier surgeons, believing that the effectivity of an iridectomy lay in the
incarceration of iris tissue, deliberately left it in the wound (Coccius, 1859–63;
Bader, 1881 ; Parinaud, 1901); but the first to advocate a specially designed
1 p. 2129.
3404 TEXT-BOOK OF OPHTHALMOLOGY
iris-inclusion as a filtration operation was Herbert (1903–34). Three years
later the simple and practical operation of iridencleisis was designed by
Holth (1906). In this operation the anterior chamber is opened sub-
conjunctivally, the iris is drawn up into the wound, an iridectomy is done to
cut the sphincter, and the iris is left under the conjunctival flap. In the
operation of iridotasis Borthen (1909) made the sub-conjunctival prolapse
without an iridectomy. In both, an essential post-operative measure is the
administration of miotics and massage for some months.
The advantages of iridencleisis are that it is very easy to perform, it disturbs the
eye only to a slight extent and involves little trauma, the anterior chamber re-forms
Fig. 2885-The Filmºrang Scan Antºn TREPHINING.
The ciliary body is entangled in its posterior aspect, the trephining having been
done too far back, the fistulous passage is in free communication with a widely
opened sub-conjunctival filtering space; both the passage and space are filled with
very loose connective tissue poor in cells and enclosing a number of open spaces
which are not lined by endothelium (Elliot).
rapidly and there is no sudden and acute fall in tension. Subsequent cataract for-
mation is rare, and late infections are statistically fewer than after sclerectomy.
Some of its advocates use it in every type of case, acute and chronic (Holth, 1927-33;
Gjessing, 1931–39), but this is probably over-sanguine.
For a long time after its introduction the operation did not achieve widespread
popularity, largely because of the fear of sympathetic ophthalmitis and late infection.
Both of these undoubtedly occur; thus the loss of both eyes through sympathetic
disease has occurred, and Wilmer (1927) reported 0-38% of late infections, a figure
close to that of 0-3% found by Erola (1934)-that is, considerably lower than trephin-
ing. As the long end-results of large series began to accrue, however, confidence has
become greater and to-day it is undoubtedly and deservedly very popular. Thus
some authors report a very high proportion of successes (83%, Gjessing, 1923; 87%,

ANOMALIES OF THE INTRA-OOULAR PRESSURE 3405
Wilmer, 1927; 90%, Butler, 1932), while others speak highly of it (Pillat, 1928;
Blaickner, 1930; Holst, 1931–34; Butler, 1932–36; Goar and Schultz, 1939; Weekers
and Bonhomme, 1940). Similarly, although not so widely used, iridotasis has its
advocates (Harrower, 1918; Wilder, 1923; Bell, 1930).
In very recalcitrant cases, particularly of the chronic congestive type, Greenwood
(1933) suggested an iridencleisis combined with a Lagrange sclerectomy, a combination
which in his hands produced very satisfactory results.
The Filtering Scar. In all these operations the object is to create a
filtering scar which will remain a permanently open channel for drainage
- -
- - º
- º º --
-- º º º
- | º
º
- º -
- º
-
-
º
-
Fig. 2886.--THE FILTERING SCAR AFTER IRIDENCLEIsis.
The walls of the fistula are lined by uveal epithelium which has prevented the
walls of the scleral wound from closing (Gjessing, T. O. S.).
from the anterior chamber to the sub-conjunctival space. Such scars have
received a considerable amount of pathological study, both those resulting
from trephining (Collins, 1914; Verhoeff, 1915; Greeves, 1915; Elliot, 1918;
Holth, 1922) (Fig. 2885), and from iridencleisis (Berner, 1913; Holth,
1922–33) (Fig. 2886). The perfect trephine scar is filled with delicate con-
nective tissue derived from the sub-conjunctival tissue, full of empty
spaces inter-communicating and traversing its extent, and free from
endothelium (Verhoeff, 1915). Closure may follow from dense scar-tissue
derived from a raggedly cut wall in the sclera (Holth, 1922), or from the
growth of endothelium into the area (Collins, 1914), or from prolapse of the
ciliary processes (Greeves, 1915). The most permanent and efficient factor in

















3406 TEXT-BOOK OF OPHTHALMOLOGY
keeping a patent channel would appear to be the migration of the pigmentary
cells of the iris, which being ectodermal, are not engulfed in mesodermal
overgrowth (Holth, 1933) (Fig. 2886); for this reason it is to be expected
that the filtering scar of an iris-inclusion operation has more chances of
survival than that of a sclerectomy.
3. Operations to Form Paths for Intra-ocular Drainage
(a) CYCLODIALYSIs is an operation conceived by Heine (1905) of Breslau,
which was based on a suggestion of Axenfeld that the good results of iridec-
tomy were due to the frequently associated choroidal detachment. It
depends on making a communication between the supra-choroidal space
and the anterior chamber by breaking down the attachments of the pectinate
ligament by a spatula introduced through the sclera behind the ciliary
body. Its action is claimed to depend on two factors: (a) the establish-
ment of drainage for the aqueous posteriorly through the choroid (Heine,
1905), and (b) the production of atrophy of the ciliary body following
damage to its blood-supply (Krauss, 1907; Salus, 1920). The influence of
the former is suggested by the fact that forward re-attachments of the iris
can be seen gonioscopically in cases where the operation has failed (Barkan,
Boyle and Maisler, 1936), while histological examinations have confirmed
the presence of the latter to some small degree (Weekers, 1907; v. Grosz,
1924; Elschnig, 1932; Kronfeld, 1936). -
The advantages of cyclodialysis are that it is easy to perform, it has few com-
plications, it involves no cosmetic defect and no weakening of the globe, it can be
repeated several times, and it can be followed by any other operation. Individual
experiences vary from 50% to 90% successes, but it has been employed in very varying
conditions.
Cyclodialysis is not effective for cases with an acutely raised tension ; “the more
urgent the indications for operation the less beneficial are the results of cyclodialysis ‘’
(Heine, 1905). It is particularly applicable to chronic simple cases in which the
tension is not high. If used in a high-tension eye it may provoke a further reactive
rise sometimes owing to hamorrhage, perhaps Owing to the ciliary trauma (Yoshida,
1923), while the pressure of the ciliary region against the sclera prevents it from being
effective by the formation of new adhesions between the iris and the angle of the anterior
chamber. In chronic congestive cases the immediate result may be good (87%, de
Grosz, 1932), but the end-result after some years is poor in more than half of them.
Apart from low tension cases of compensated glaucoma, its greatest value lies as an
adjunct to other operations, particularly an iridectomy ; moreover, it is an operation
of great value in aphakic eyes.
To avoid the failure of a cyclodialysis, Mauksch (1924) suggested tucking the
iris into the sub-choroidal space, thus in a sense combining an iridotasis with a cyclo-
dialysis (Raeder, 1928; Suker, 1931). A further combination of considerable value in
desperate cases is to combine a cyclodialysis with an iridectomy, thus opening up two
channels of exit for the aqueous (Czermak, 1906; Török, 1923; Jervey, 1927; Wootton,
1932; Wheeler, 1936; Lauber, 1939). Troncoso (1940) suggested the original idea of
implanting a strip of magnesium in the wound between the ciliary body and the sclera ;
the metal is absorbed in about 20 days, giving off free bubbles of hydrogen, leaving a
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3407
lacunar scar between the anterior chamber and the supra-choroidal space, while its
temporary presence and the bubbles of gas prevent an early re-attachment of the
ciliary body to the sclera.
(b) TRABECULOTOMY. Reasoning that glaucoma was caused by a failure
of the aqueous to reach Schlemm's canal, the Italian surgeon de Vincentiis
(1891) conceived the rational idea of opening the canal by a knife introduced
into the anterior chamber ; the attempt was unsuccessful because he could
not see where he was going. This feat has recently been achieved by the
ingenuity of Otto Barkan (1936–38) of San Francisco, who, in his operation
of trabeculotomy (or goniotomy), with the aid of a contact glass and intense
transillumination, has devised a technique by which the canal can be cut
open from within. This operation, which re-establishes the normal outlet
of the aqueous is applicable only to those cases of simple glaucoma in which
the anterior chamber is relatively deep and the angle open; its remote end-
results will be awaited with interest.
Other operations may be noted in passing.
Hancock (1860), believing that the action of the ciliary muscle was responsible for
glaucoma, severed the ciliary body through a scleral incision (intra-ocular ciliary
myotomy), an operation also practised by Solomon (1861). Abadie (1910) advised
a somewhat similar ciliarotomy, cutting the ciliary body meridionally in order to
section the ciliary nerve plexus. With a view to stopping nerve reflexes he also
advised a total iridotomy. A small basal iridotomy in order to facilitate drainage
was suggested by Curran (1920) and practised also by Gifford (1921–24); it was found
ineffective by Elschnig (1923). Verhoeff (1924) recommended a cyclectomy, a small
portion of the anterior part of the ciliary body being excised through a scleral incision
after a button-hole iridectomy.
Seton operations have been tried, drainage being attempted from the anterior
chamber or the supra-choroidal space by threads (Mayou, 1912–13; Zorab, 1912–13;
Gradle, 1927; Wolfe and Blaess, 1936), horse-hair (Row, 1934) or gold drains (Weekers,
1922; Stephanson, 1925). This may occasionally be legitimate, but only in absolute
glaucoma when enucleation is impossible.
Resection of the cervical sympathetic, suggested by Abadie (1897) and introduced
by Jonnesco (1899) of Bucharest, was practised to a considerable extent for some time
thereafter (Abadie, 1899–1901 ; Ziehe and Axenfeld, 1901, 74 cases in Germany;
Rohmer, 1902, 68 cases in France ; Wilder, 1904, 114 cases in America): the results,
however, were irregular and disappointing (Elschnig, 1912).
D. Systematic Treatment
This section is particularly difficult to write because, more than any other condition,
glaucoma cannot be legislated for en masse. This applies most particularly to the type
of operation suitable for individual cases, for each surgeon tends to have a favourite
operation which he uses preferentially often without valid reason except that he has
had successes with it and failures with others or that he has become more familiar with
it. Thus in the recent past, Lagrange's operation was peculiarly favoured in France,
Elliot's in England and America, Heine's in Central Europe and Holth's in Scandinavia.
This, of course, is a perfectly valid reason, for the best operation is frequently that which
is done best, with all the precautions and attention to minutiae which can only come
T.O.-WOL. III. 4 P
3408 TEXT-BOOK OF OPHTHALMOLOGY
from experience ; but the sole employment of any one operation, while resulting in
many successes, will entail more failures than is necessary. It is obvious, therefore,
that many will not agree with the following summary, and I would hasten to rid it.
of any pretence to accepted authority.
General and Hygienic Treatment
Every case of primary glaucoma should be treated, not as a sick eye
alone, but as a sick individual; and although little is known of the systemic
origins of the disease, some attempt should be made to combat them. A
detailed general medical examination should first be undertaken, when it
will be found that a large number of the patients fall into one of two groups—
the sclerotic or the angiopathic. The particular diathesis should be treated
accordingly. Cardio-vascular-renal disease should be explored, hyper-
tension adjusted, syphilis eliminated or treated, constipation avoided and a
regular hygiene for the bowel instituted, and the toxic effect of focal sepsis
rigidly eliminated in so far as that is possible. In sclerotic patients iodides,
small doses of thyroid, and sometimes a little mercury may be of value. In
the angiopathic type of patient endocrine imbalance should be particularly
investigated as well as any disturbance of the neuro-vegetative system.
With regard to the general hygiene of life, dangerous living should be
avoided. In so far as it is possible, extremes of emotion, anxiety or fatigue
should be eliminated, sudden changes of temperature (going out of doors or
in ; hot or cold baths) mitigated, congestion of the head (prolonged stooping,
sleeping on a low pillow) guarded against, and excesses in food and drink
(alcohol, strong coffee) avoided ; a salt-free diet may be considered, and
constipation eliminated. With regard to the eyes themselves, their use need
not be restricted although fatigue should be guarded against, refractive
errors corrected, and in order to encourage miosis, darkness (including dark
spectacles) avoided rather than bright lights.
The local treatment to the eye is best considered separately for each
clinical picture. It is to be remembered that in every unilateral case the
other eye should be held suspect, and every operation on One eye should be
preceded by eserine in the other to eliminate the effect of axon reflexes
between the two.
This last point is a matter of great importance. Any intra-ocular operation—
apart altogether from glaucoma—is accompanied by a reflex hypo- or hypertensive
reaction in the other (Towbin, Protopopof and Urnishewskaja, 1934), and in an eye
predisposed to glaucoma (as the fellow eye of a glaucomatous one frequently is even
although it appears normal) this reflex reaction may be, and frequently has been,
disastrous.
1. Simple Glaucoma
(a) Early Stage of Low Tension and Maintained Function. If the patient
is in the very early stages when the tension is still within normal limits and
function is relatively little impaired, medical treatment is alone indicated.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 34.09
In those cases, and in cases wherein suspicion as to the diagnosis arises,
hospitalization is of the greatest value when thorough examinations of the
fields, the adaptation, and, more particularly, the vagaries of the tension, can
be studied with the patient under standardized conditions. Not only should
the initial treatment be instituted in this way for each particular case, for
the strength of miotic and the frequency of the dosage required to maintain
miosis and control the tension is an individual matter, but (comparable to the
precaution generally taken with diabetics) such an investigation should be
undertaken at periodic intervals to re-assess the progress of the case and
the adequacy of treatment. So long as such a patient retains a relatively
low tension which shows no great diurnal variation or unusual response to
the more simple provocative tests, and so long as his fields do not show a
progressive decline, medical treatment can be continued provided the
patient will submit to observation. It is to be emphasized that the state of
the central vision provides no adequate index to the progress of the disease.
The local medical treatment may be summed up in the phrase—the
miotic life. In this pilocarpine is the sheet-anchor, for if eserine is required
to control the tension, operation is usually advisable. Pilocarpine nitrate
should be given in the smallest doses which produce adequate miosis
(starting from 0-2 and increasing to 2.0% or over) and at intervals which
allow it to control the highest levels in the tension as brought out in its
diurnal variations. Thus the usual morning rise may be combated by a drop
at night and another on waking in the morning ; a rise after the fatigue of
the day’s work may call for an extra drop in the afternoon; while an
additional drop before going out in the dark or to a cinema is a wise precaution.
If it is suspected that continuous miosis may be leading to pupillary
adhesions, an occasional dilatation with adrenalin may be advisable.
It has frequently been said that immediately glaucoma is diagnosed
operation should be undertaken at once ; but the immediate hazards of an
operation and the ultimate dangers to which it may expose the eye cannot
legitimately be taken so lightly. On the other hand, many cases can be
controlled for a number of years by medical measures without an increase
in tension or a decrease in function (Lawson, 1913; Posey, 1914–20 ;
Young, 1924–33 ; and many others). Fisher (1921), for example, studied a
case which showed cupping and Scotomata but no progressive loss of
function for 40 years under miotics. There is no question, however, that
if any deterioration in the level or stability of the tension or of the fields occurs,
operation should be undertaken at once, for it is to be remembered that delay
in operating has been responsible for more ultimate failures in the end-results
of glaucoma than anything else.
(b) The Stage of Established or Unstable Tension or of Deteriorating
Function. A patient in this group may be a transfer from the first, or be
seen for the first time ; in the first case operation is advisable, but in the
second an attempt lasting about a month (except in urgent cases) can be
4 P 2
3410 TEXT-BOOK OF OPHTHALMOLOGY
made to stabilize him, for if the tension can be reduced and maintained
within normal limits and functional deterioration stopped, medical treat-
ment may be safely continued provided adequate control is possible. It is to
be remembered that in deciding on operation a limit of tension is difficult
to name, for a pathologically high tension for any particular eye is that
degree of tension which does damage—a very variable quantity. In the
lesser degrees, instability as judged from diurnal and provocative variations
is more important than height ; a bad response to the massage test, suggesting
bad drainage, is an indication for operation ; and in the functional tests
variations in the 1/2,000 field and in angioscotoma are the most delicate
index. Even if the tension is not high and function deteriorates, operation
should be considered, for, as we have seen in the discussion on optic atrophy,
there is little doubt that a lowered intra-ocular pressure aids the nutrition
of an atrophic nerve.
If deterioration indicates that an operation should be done, several
choices are available.
If the tension is relatively low (under 40) and its variations and the
evidence of the fields suggest that the case is not rapidly progressive but is
just not holding its own under miotics, a cyclodialysis is a good choice : it
usually acts well in such cases, it has few complications, it leaves the
integrity of the eye unimpaired, it can be repeated readily, and it leaves
the field free for any other operative procedure. If it reduces the tension,
miotics must be continued or the effects of the operation will probably not
be permanent. If a return of tension appears after the lapse of 6 months
or more, the operation may be repeated with greater chances of success;
but if the tension recurs within a few weeks, a fistulizing operation is
indicated.
If the tension is above 40 Schiótz a filtering operation should probably
be done. Since late results of Barkan's trabeculotomy are not yet available,
the choice lies between a trephining or an iridencleisis ; both of these cause a
permanent impairment of the integrity of the globe ; iridencleisis involves
less trauma and on statistics has safer end-results ; but probably the higher
the tension, within limits, the more the indication for trephining. It may
also be considered that, owing to the less resistance of the filtration scar of
the trephine to infection and other influences, the longer the expectation of
life of the patient, the greater the indication for iridencleisis.
(c) The Stage of High Tension or Advanced Functional Loss. If the
tension is very high a determined attempt to lower it should be made before
operating, the patient being put to bed and purged, synergic miosis
alternated with adrenalin and massage, and osmotic treatment with saline
or sorbitol instituted. Finally, before operation a retro-bulbar injection of
novocaine and adrenalin is frequently of great hypotensive value. By all
these methods the tension can frequently be brought down temporarily to
bring the case into the second category.
ANOMALIES OF THE INTRA-OCULAR PRESSURE 34.11
If the tension still remains high, particularly if the anterior chamber is
shallow, or, alternatively, if the functional loss is eatreme so that only a small
portion of the central field is left, the trauma and the sudden and complete
loss of tension involved in trephining may be inadvisable, in the first case
owing to non-formation of the anterior chamber and subsequent sclerosis
of the lens, in the second because of a sudden and complete obliteration of
the central field—a rare but disastrous accident. In this event it is probably
best to lower the tension gradually by a Herbert's flap-door sclerotomy with
subsequent massage, to be followed by an iridencleisis at a later date. In
frail and aged patients, the former alone may well suffice. In those sclerotic
cases where the field is minimal and the tension is not particularly high,
repeated injections of acetyl-choline may improve the circulation and render
operation safer.
If despite these operative measures the tension still remains high, a
second operation should be undertaken before too much permanent damage
is done. A trephining can be repeated, and may be combined with a cyclo-
dialysis when it is performed ; or a separate cyclodialysis can be made ;
one or more trephines made over the ciliary muscle combined with cyclo-
dialysis are frequently effective ; an iridencleisis combined with a Lagrange
sclerectomy may be tried ; while a combination of an iridectomy with a
cyclodialysis may be successful.
If for any reason of age, illness or psychology, operation is not under-
taken, the progress of the disease should be delayed so far as that is possible
by alternating the various miotic drugs, employing them synergically,
instituting periods of adrenalin treatment, and paying constant attention
to the general regime.
The treatment of absolute glaucoma will be considered later.
2. Congestive (Incompensated) Glaucoma
(a) In the prodromal stage of this type of glaucoma, general medical and
hygienic treatment is of the greatest importance and miotics essential, but
if under investigation the tension shows evidences of marked instability,
operation should not long be delayed lest, despite prophylactic measures,
the catastrophe of acute glaucoma develop. Probably the most popular
procedure to adopt is a fistulizing operation—a trephining or an iridencleisis,
but unless the tension is raised, an iridectomy, which need only be peripheral,
and leaves the function and integrity of the eye unimpaired, will usually
prevent the onset of an acute attack in the future. In a quiet eye the
operation itself is without danger. *
(b) In acute glaucoma treatment must be instant and urgent. The
ideal is to reduce the tension by medical methods so as to convert the acute
into a chronic congestive phase before operating. If, however, this cannot
be accomplished within 24 to 48 hours after the beginning of the attack,
operation should not be delayed beyond this interval; the function of the
3412 TEXT-BOOK OF OPHTHALMOLOGY
eye will probably be greatly and permanently impaired, and peripheral
adhesions will almost certainly have formed in the angle of the anterior
chamber so that the effectivity of most operations undertaken thereafter
is vitiated. There is no doubt that the results of operation vary inversely
with the length of time it is delayed.
Hypotensive medical treatment should include rest in bed with the head
raised, sufficient morphia to allay pain and anxiety, synergic miosis
administered every 2 hours, purges and general sweating, and—of great
value—leeches to the temple. Osmotic treatment intravenously is some-
times very effective, and diathermy also may be of considerable value. A
retro-bulbar injection of 0-2 to 0.3 c.c. 1: 1,000 adrenalin with novocaine
may be particularly useful, while Magitot (1937) claims that with a retro-
bulbar injection of 1 c.c. of 40 to 60% alcohol after novocaine (which may
involve a transient muscular paresis) pain is abolished dramatically and,
presumably by cutting off vascular reflexes, the tension is usually lowered
sufficiently within an hour to allow operation with safety.
A fall of tension after medical treatment is always heralded by a
lessening of the pupillary dilatation. This, indeed, forms a good index of
the pressure in the eye, as it indicates a lessening of the oedema and forward
thrust of the ciliary body which is essentially responsible for the strangula-
tion, and the return of the function of the intra-ocular nerves. At first the
pupil contracts slowly, and then as the tension approaches normal, a sudden
miosis develops. Simultaneously the chamber deepens, the tension may fall
to sub-normal, and vision returns; within 6 to 8 hours thereafter the con-
gestion and other signs of the attack may have gone. In this event miotics
may be continued if supervision can be adequate ; but if this cannot be
efficiently done, or if tests show that the tension is unstable, a prophylactic
peripheral iridectomy should be performed. If the glaucoma still remains
in an acute or relatively acute phase, the operation of choice, particularly
if the attack is a first one, is a basal iridectomy. If the anterior chamber is
very shallow and the tension high, a preliminary posterior sclerotomy to allow
the escape of vitreous is of great value. It is not always easy to perform
for the vitreous may not present and sometimes even cannot be sucked away
by a syringe, nor is it always accomplished without risk ; but the risks
are less than those of suddenly entering a very hard eye through a
barely existent anterior chamber by section. If it is not done, or if the
anterior chamber is still shallow, an external incision after Elschnig's
method should be made under a conjunctival flap. If the iridectomy is
not effective, it may be followed by a cyclodialysis in the same region,
or even by one or more posterior trephine sclerectomies combined with
cyclodialysis.
During an attack of acute glaucoma unusual care should always be given
to the other eye in which miosis must be maintained ; it may, indeed, be
advisable, if any suspicion arises regarding its condition, to perform a
ANOMALIES OF THE INTRA-OCULAR PRESSURE 34.13
prophylactic peripheral iridectomy upon it to avoid its sharing the fate of
its fellow.
(c) In the chronic congestive phase attempts should be made to lower
the tension as much as possible on the lines already laid down : in this type,
however, longer time can be taken over medical treatment than in the
acute stage. Pilocarpine is rarely efficacious and eserine or prostigmin, in
combination with a choline derivative, is usually to be relied upon ; but
eventually operation must be resorted to, a step which, depending on the
response of the eye, should not be delayed many weeks. The operation
chosen must vary not only with the degree of tension but also with the
intensity of congestion. On the choice it is impossible to legislate, for it is
frequently difficult ; on the whole, if tension and congestion are not extreme,
a iridencleisis may be done, which, if it seems necessary, may be effectively
combined with a Lagrange sclerectomy; if they are marked, a trephining is
probably more effective ; and if they are very marked, it may be safer and
wiser to do an iridectomy, which, as it alone is frequently unsuccessful, can
be followed by a cyclodialysis in the same quadrant. If the condition is
not controlled thereby, further combinations should be tried as already
indicated. .
3. Absolute Glaucoma
In absolute glaucoma the treatment of election is to enucleate the eye
as soon as pain becomes distressing ; in the degenerative stage no other
treatment should be considered. Apart from the difficulty of controlling
pain, it is important to remember that a considerable proportion of such
eyes contain an unsuspected malignant melanoma : thus Neame and
Khan (1925) found that 10% of 402 eyes enucleated at Moorfields Hospital
over an 11-year period under the diagnosis of absolute glaucoma contained
an unsuspected tumour of this kind. If, however, enucleation is contra-
indicated, an attempt should be made to relieve the pain, and for this many
expedients have been proposed.
Of the available non-surgical methods, X-rays may be given a trial. Of the
surgical methods available for relieving the tension, none offers the prospect
of invariable success. A trephining operation is probably most effective,
and, owing to the advanced pathological changes frequently present in the
angle of the chamber, more efficient results may be obtained by trephining
over the ciliary muscle : more than one opening may be made and a seton
inserted. Gradle (1935) produced relief by plunging a diathermy needle
into the middle of the vitreous, which presumably produced its coagulation
and shrinkage. Failing these expedients, recourse may be made to rendering
the eye insensitive. Of the various methods which have been tried, destruc-
tion of the ciliary ganglion is the easiest. Rohmer (1902) practised its
extirpation in a Krönlein operation, but a retro-bulbar injection of 1 c.c. of
90% alcohol after the method of Grüter (1918) causes less trauma, even
3414 TEXT-BOOK OF OPHTHALMOLOGY
although it may produce a temporary proptosis and paresis of the extra-ocular
muscles (Jaensch, 1925; Fejer, 1932). Anaesthetization of the Gasserian
ganglion was practised by Alexander (1927–30) by the method used for
trigeminal neuralgia ; but it must be remembered that all these measures
tend to hasten the incidence of degenerative changes.
Prognosis
To give a prognosis of a composite entity such as primary glaucoma is
impossible. On the whole, however, it may be said that, although exceptions
do occur, without treatment the disease is progressive and results in absolute
bilateral blindness. With adequate treatment the majority of simple compen-
sated glaucomas can be stabilized or their progress delayed so that useful
vision remains until the death of the patient : fortunately most patients are
old, but a juvenile glaucoma is an unpleasant problem. Quite a number,
however, particularly those of the sclerotic type, gradually and imperceptibly
go down-hill even although the tension is adequately controlled : the
deterioration is part, although it may be the most conspicuous part, of a
general pathological process which is beyond medical control. Again in this,
as in all other types of glaucoma, a malignant form occurs, which progresses,
and frequently progresses rapidly, despite all treatment. In the average
case the end-results depend essentially on two factors—the stage at which
the patient comes under treatment, and the sustained adequacy of the
treatment : the disease is not cured, but arrested ; it is always there so far
as our present methods of treatment are concerned, and constant observation
and eternal vigilance must be maintained in a cat-and-mouse manner so that
any exacerbation may be met by a change in the medical treatment or a new
surgical attack. On the whole many more bad results arise from postponing
an operation than from performing one. In general terms it may be said in
simple glaucoma that with reasonably adequate treatment some 70% of
cases do well in so far as they become stabilized, 20% deteriorate so slowly
that they retain a useful amount of sight to the end, while 10% do badly.
In acute congestive glaucoma approximately the same figures can be obtained
if the attack is overcome rapidly by medical treatment and a prophylactic
operation is done on a quiet eye ; but if the condition has lasted longer
than 48 hours or if resort has been necessarily made to surgery of a more
desperate type, the figures are considerably worse and a disappointing
end-result in 30% is to be expected. In chronic congestive glaucoma,
allowing for the great clinical variations between different cases, the figures
lie between the former two. The result is that a very large proportion of
blindness is due to this disease—some 9% of total blindness (Marshall and
Smith, 1932), while in the higher decades of life the incidence is considerably
greater. Thus Magnus (1913) considered that glaucoma caused from
one-quarter to one-third of all blindness occurring after 45 years of age,
ANOMALIES OF THE INTRA-OCULAR PRESSURE
34 15
accounting for 11.61% of all blindness between 30 and 45 years, in which
category it ranked third among diseases, 27% of all blindness between
45 and 60 years, when it heads the list of causal diseases, and reached the
figure of 58.11% between 60 and 75 years.
Abadie. A. d’O., xvii, 375, 1897; xix, 94,
1899 ; xxx, 262, 1910. .
An. d’Oc., czzv, 194, 1901.
Presse Méd., xxxvii, 220, 1929.
Accardi. Boll. d’Oc., iv, 289, 1925.
Alexander. Med. Kl., xxiii, 1103, 1927.
K. M. Aug., lxxxiv, 65, 1930.
Allard. O. Rev., xix, 127, 1900.
Alt. Am. J. O., i, 184, 1918.
La Cl. Opht., xxiii, 312, 1919.
Aubaret. La Cl. Opht., xxvii, 276, 1923.
Bader. Internat. Med. Cong., London, iii, 98,
1881.
Bailliart. La Cl. Opht., xvi, 253, 1927.
Bailliart and Bollack. An. d’Oc., clviii, 641,
1921.
Barkan. Am. J. O., xix, 951, 1936 ; xx, 1237,
1937; xxi, 1099, 1938.
A. of O., xix, 217, 1938; xxi, 331, 1939.
Barkan, Boyle and Maisler. Am. J. O.,
xvii, 21, 1936.
Barkan and Maisler.
1937.
Bell. A. of O., iii, 194, 1930.
Bellows, Puntenny and Cohen. A. of O., xx,
1036, 1938.
Am. J. O., xx, 504,
Berner. B. O. G. Heidel.., xxxix, 359, 1913.
Blaickner. Z. f. Aug., lxxii, 265, 1930.
Bonnefon. C. R. Soc. Biol., xci, 1214, 1924.
Borthen. A. f. Aug., lxv, 42, 1909.
Bowman. Brit. Med. J., ii, 377, 1862.
Brückner. Schw. med. W., lxvi, 1264, 1936.
Butler. Brit. J. O., xvi, 741, 1932.
T. O. S., lvi, 194, 1936.
Cantonnet. A. d’O., xxiv, 1, 1904.
Castresana. Siglo med., lxxxiii, 116, 1929.
Chiari and Januschke. A. Exp. Path. w.
Pharm., lv., 120, 1911.
Clarke. Am. J. O., xxii, 249, 1939.
Coccius. Ueber Glaukom., Leipzig, 1859.
A. f. O., ix (1), 1, 1863.
Collins. Hunterian Lectures,
Lancet, i, 1329, 1463, 1894.
Ophthalmoscope, xii, 589, 1914.
1894 : Ref.
Cordes. A. of O., xvii, 896, 1937.
Cordes and Harrington. Am. J. O., xviii,
451, 1935.
Critchett, G. R. L. O. H. Rep., i, 57, 1858.
Cruise. T. O. S., xli, 248, 1921.
Curran. T. Sec. O. Am. Med. As., p. 75,
1920.
A. of O., xlix, 131, 1920.
Czermak. Prag. med. W., xxxi, 313, 1906.
Darier. La Clin. Opht., vi, 141, 1900.
Davenport. T. O. S., xlvii, 283, 1927.
Brit. J. O., x, 474, 1926.
Denig. A. f. O., czzv, 156, 1930.
T)ianoux. An. d’Oc., czzxiii, 81, 1905.
Dieter. A. f. Aug., xcvi, 179, 1925.
Domec. La Clin. Opht., xii, 303, 1906.
Duke-Elder. Brit. J. O., x, 1, 30, 1926.
P. R. S., (B), cvii, 332, 1930.
J. Physiol., lxxi, 1, 1931.
Duke-Elder and Law. Brit. Med. J., i, 590,
1929.
Duverger. Thérapeut. chir. oph., Paris, 1926.
Dyar and Matthew. A. of O., xviii, 57, 1937.
Ellett and Rychener. Am. J. O., xii, 368,
1929.
Elliot. Ophthalmoscope, vii, 804, 807, 1909;
viii, 482, 779, 1910.
T. O. S., xxxviii, 227, 1918.
Treatise on Glaucoma, London, 1922.
Am. J. O., xiv, 999, 1931.
A. of O., viii, 797, 1932.
Elschnig. K. M. Aug., I (1), 538,
lxx, 667, 1923; lxxx, 382, 1928.
B. O. G. Heidel., xlix, 277, 353, 1932.
Englehart. A. ges. Physiol., ccxxvii, 220,
1931.
Enroth. Acta O., v, 112, 1927.
Erdmann. K. M. Aug., lii, 520, 1913; lxxiv,
413, 1925.
Z. f. Awg., xxxii, 216, 1914.
Erola. Acta O., xii, 137, 1934.
Fejer. Am. J. O., xv, 135, 1932.
Fergus. Brit. Med. J., ii, 983, 1909.
Ophthalmoscope, viii, 74, 1910.
O. Rev., xxxiv, 202, 1915.
Fisher. T. O. S., xli., 264, 1921.
Fortin. A. d. Oft., B. A., iv, 359, 454, 1929.
Friedenwald. Am. J. O., xv, 189, 1932.
Fromaget. An. d’Oc., clviii, 424, 1921.
Fuchs, E. B. O. G. Heidel., xxv, 179, 1896.
Gala. Congr. Czechoslovak. Oph., Prague,
May, 1927: Ref. K. M. Aug., lxxix, 123,
1927,
Gallois and Viard. Bull. S. d’O. Paris, xlvi,
325, 1934.
Gapeeff. Russ. A. O., viii, 563, 1932.
Gapejeff. K. M. Avg., lxxxi, 626, 1928.
Gifford. Am. J. O., iv, 889, 1921; vii, 346,
1924; xi, 628. 1928.
A. of O., xxiii, 301, 1940.
Gjessing. T. O. S., xliii, 616, 1923; lix, 311,
1939.
Acta O., v., 149, 1927.
A. of O., vi, 489, 1931.
Goar and Schultz. A. of O., xxii, 1035, 1939.
Gouterman. A. of O., ii, 670, 1929.
Gradle. Am. J. O., vii, 851, 1924; xiv, 936,
1931; xviii, 730, 1935.
J. Am. Med. As., lxxxiv,
lxxxix, 2025, 1927.
Ill. Med. J., liii, 126, 1928.
A. f. O., xxiii, 301, 1940. &
1912;
675, 1925
3416
TEXT-BOOK OF OPHTHALMOLOGY
v. Graefe. A. f. O., iii (2), 456, 1857; iv. (2),
127, 1858; vi (2), 150, 1860; viii (2),
242, 1862; xiv. (2), 147, 1868; xv (3),
118, 1869.
Green. A. of O., v, 350, 1931.
Green and Green.
xxi, 328, 1939.
Greenwood. A. of O., x, 472, 1933.
Greeves. T. O. S., xxxv, 445, 1915.
de Grosz. B. O. G. Heidel., xliv 151, 163,
1924. -
A. of O., v, 327, 1931.
A. d’O., xlix, 625, 1932.
A. of O., vi, 752, 1931;
Grüter. B. O. G. Heidel., xli., 85, 1918.
Hagen. Brit. J. O., vi, 360, 1922.
Hallett. J. Oph. Oto. Lary.., xxxi, 219,
I927.
Hamburger. Med. Kl., xix, 1215, 1923; xx,
267, 1924; xxi, 1495, 1925.
K. M. Aug., lxxii, 47, 1924; lxxvi, 849,
1926.
D. med. W., li, 1397, 1925.
Z. f. Aug., lx, 109, 1926.
A. of O., lv., 533, 1926.
Brit. J. O., xiv., 172, 1930; xix, 455, 1935.
Hancock. R. L. O. H. Rep., iii, 13, 1860.
Hanssen. Nordwest dewt. Augenörz. Verein.,
Hanover, April, 1928 : Ref. K. M. Aug.,
lxxx, 690, 1928.
Harrower. A. of O., xlvii, 37, 1918.
Heim. K. M. Aug., lxxix, 345, 1927.
Heine. B. O. G. Heidel., xxxii, 3, 1905.
Heinmann. Med. Kl., xx, 713, 1924.
Henderson, T. O. Rev., xxvi, 191, 1907.
Herbert. T. O. S., xxiii, 324, 1903; xxxix,
220, 1919; xli., 239, 1921.
Ophthalmoscope, v, 292, 1907; ix, 76, 1911 ;
xi, 398, 1913.
Brit. J. O., iv, 216, 550, 1920; v, 183, 417,
1921 ; xiv, 433, 1930; xviii, 142, 1934.
Hertel. K. M. Aug., li, 351, 1913.
A. f. O., xe, 309, 1915.
Hess. A. f. Aug., lxxxvi, 89, 1920.
v. Hofe. A. f. Aug., xcviii, 201, 1928.
Holst. K. M. Aug., lxxxvii, 602, 1931.
Acta O., xii, 348, 1934.
Bolth. B. O. G. Heidel., xxxiii, 123, 1906;
xxxix, 355, 1913.
An. d’Oc., czzxvii, 345, 1907; czlii, 1,
1909. ;
Ophthalmoscope, ix, 87, 1911 ; xii, 347,
1914
Brit. J. O., v, 544, 1921 ; vi., 10, 1922.
R. M. Aug., lxxix, 620, 1927.
A. of O., iv, 803, 1930; vi, 151, 1931; ix,
913, 1933.
T. O. S., liii, 326, 1933.
Horner and Bettman. Am. J. O., xix, 311,
1936.
Howard. Am. J. O., xviii, 461, 1935.
Howell. A. of O., xii, 833, 1934;
1018, 1936.
Ivanov. Viest. oft., xi, 519, 1937.
Jaensch. K. M. Aug., lxxiii, 250, 1924.
Z. f. Aug., lviii, l, 1925.
Jervey. T. Am. O. S., xxv, 160, 1927.
xvi,
de Jongh and Wolff. Ned. tij. v. Gen., lxix,
2665, 1925.
Jonnesco. Wien. kl. W., xii, 483, 1899.
Josephson. Eye, Ear, Nose, Throat Monthly,
xiii, 453, 1935.
Kadlicky. Brat. lek. Listy., i, 313, 1922.
Cas. lek. Cesk., lxiii, 1035, 1924; lxv, 1285,
I926.
Kafka. Z. f. Aug., liv, 235, 1924.
Kayser. K. M. Aug., lxxii, 796, 1924.
Rleiber. A. f. Aug., lxxxi, 288, 1922.
Knapp, A. A. of O., l, 556, 1921; x, 298,
1933.
N. Y. State Med. J., xxxiii, 1431, 1933.
Köllner. Münch. med. W., lxv, 229, 1918.
Z. f. Aug., xlii, 381 ; xliii, 381, 1920.
Krauss. Z. f. Aug., xvii, 318, 1907.
Kreibig. Wien. kl. W., xlix, 843, 1936.
FCronfeld. A. of O., xv, 411, 1936.
Rüsel. K. M. Aug., xliv (2), 80, 236, 1906.
Lagrange, F. Soc. med. de Bordeawa, Jan. 8th,
1905.
A. d’O., xxvi, 481, 1906; xxvii, 429, 1907.
An. d’Oc., czzxvii, 89, 1907.
T. O. S., xlvii, 312, 1927.
Lambert and Wolff. A. of O., ii, 198, 1929.
Laqueur. Cb. med. Wiss., xxiv, 421, 1876.
Lauber. T. O. S., lix, 267, 1939.
Law. T. O. S., liii, 474, 1933.
Lawson. T. O. S., xxxiii, 194, 1913.
Liebermann. K. M. Aug., lxxi, 781, 1923.
Lloyd. A. of O., lvi, 445, 1927. -
T. O. S., lviii, 774, 1938 ; lix, 181, 1939.
Löhlein. K. M. Aug., lxxvi, Beil., i, 1926.
MacCallan. T. O. S., xxiii, 374, 1903.
Mackenzie. Treatise on Diseases of the Eye,
London, 1830.
Maddox. Brit. J. O., xvii, 161, 1933.
Magitot. Bull. S. d’O. Paris, xlvii, 641, 1935;
Jan., 1939.
Am... d’Oc., clxvi, 356, 439, 1929;
361, 1937.
Magnus. Amer. Encyclo. of Oph., ii, 1126,
1913.
Maklakoff. A. d’O., xiii, 530, 1893.
Mans. K. M. Aug., lxxiii, 674, 1924.
Marshall and Smith. T. O. S., lii, 282, 1932.
Martin. Brit. J. O., xix, 48, 1935.
Mauksch. Z. f. Aug., lii, 167, 1924.
Mayou. Ophthalmoscope, x, 254, 1912; xi,
258, 1913.
McBurney. A. of O., xliii, 12, 1914.
Michail and Vancea. An. d’Oc., clxiii, 561,
1926.
Miloro. An. d. Ott., lxiii, 780, 1935.
Moscardi. Saggi. Oft., iv., 122, 1929.
Neame and Khan. Brit. J. O., ix, 618, 1925.
Odinzow and Nejman. Trudy Sezda Vrac., li,
51, 1929.
Onfray, Abeloos and Suys. Bull. S. fr. d’O.,
xlvii, 297, 1934.
Pagenstecher. Cb. pr. Avg., 281, 1878.
Palmieri. An... dº Ott., lxiv, 217, 1936.
Panas. A. d’O., xxii, 69, 1902.
Parinaud. An. d’Oc., cxxv, 438, 1901.
Passow. K. M. Aug., lxxxiii, 339, 1929.
clxxiv,
ANOMALIES OF THE INTRA-OCULAR PRESSURE
34.17
Phillips. T. O. S., lix, 193, 1939.
Pillat. Z. f. Aug., Supp. ix, 1928.
Pletnewa. Russ. O. J., ii, 7, 1923.
Ploman and Granström. Acta O., x, 54, 1932.
Poos and Santori. A. f. O., czzi, 443, 1929.
Posey. J. Am. Med. As., lxiii, 219, 1914.
A. of O., xlix, 293, 1920.
Post. A. of O., xi, 187, 1934.
Am. J. O., xx, 170, 1937.
Raeder. Acta O., vi, 390, 1928.
Rentz. K. M. Aug., lxxiii, 356, 1924.
Ring. Am. J. O., viii, 553, 1925.
Robertson, Argyll. R. L. O. H. Rep., viii,
404, 1876.
Rohmer. Am. d’Oc., exxvii, 328 ; cz.xviii, l,
1902.
Row. A. of O., xii, 325, 1934.
Safar. K. M. Aug., lxxiii, 510, 1924.
Sallmann. Z. f. Aug., lxxxvi, 111, 1935.
Salus. K. M. Aug., lxiv, 433, 1920.
Samojloff. K. M. Aug., lxxviii, Beil., 55,
1927.
de Sanctis. An. di Ott., lxv, 25, 1937.
Sansum. J. Amer. Med. As., lxviii, 1885,
1917.
Scardapane. Boll. d’Oc., iv, 866, 1926.
Schmidt-Rimpler. G-S. Hb., II, vii, 186,
1908.
Schoenberg. Brit. J. O., xxii, 417, 1938.
Seidel. A. f. O., cviii, 285, 1922.
Smith, Priestley. VII Internat. Cong. Edin.,
33, 1894.
Solomon. Med. Times and Gaz., i, 55, 83, 141,
221, 327, 1861.
Solowieff. Sov. vest O., v, 21, 1934.
Sonales. Am. J. O., xx, 731, 1937.
Stack. T. O. S., xli., 265, 1921.
Stephanson. Am. J. O., viii, 681, 1925.
Suker. Am. J. O., xiv, 732, 1931.
Taratin. Russ. O. J., viii, 716, 1928.
Thiel. B. O. G. Heidel.., xliv, 118, 1924.
A. f. O., cziii, 319, 1924.
A. f. Awg., xcvi, 34, 1925.
R. M. Aug., lxxii, 534, 1924; lxxvii, 753,
1926.
Z. f. Aug., lx, 114, 1926; lxv, 373, 1928.
Thomas. Ophthalmology, xiii, 582, 1915.
Török. A. of O., lii, 574, 1923. .
Towbin. Protopopof and Urnishewskaja.
A. f. O., czkxi, 554, 1934.
Tristaino. A. di Ott., xx, 589, 1912.
Troncoso. A. of O., xiv, 557, 1925; xxiii, 270,
1940.
XIV Internat. Cong., Madrid, iv, 409, 1934.
Troncoso and Reese. Am. J. O., xviii, 103,
1935.
Ulrich. A. f. O., xxx (4), 235, 1884.
Ungerer. An. d’Oc., clxvi, 769, 1929.
Velhagen. K. M. Aug., xc, 512, 1933; xcii,
472, 1934.
A. f. Aug., cvii, 319, 1933.
Verhoeff. Ophthalmoscope, xiii, 352, 1915.
A. of O., xliv, 129, 1915; liii, 228, 1924.
Villaret, Besançon and Gallois. Bull. S. fr.
d’O., xliv, 532, 1931.
de Vincentiis. Am. di Ott., xx, 92, 1891.
Weber. A. f. O., xxiii (1), 1, 1877.
de Wecker. Traité d. maladies des yeux,
Paris, ii, 571, 1867.
Am... d’Oc., czi, 321, 1894 ; cziv, 95, 1895;
cxvi, 249, 1896; cxxi, 321, 1899; cxxiv,
337, 1900; cz.xv, 421, 1901.
Weekers. K. M. Aug., xlv. (2), 230, 1907.
La Clin. Opht., xviii, 282, 1912.
A. d’O., xxxvii, 413, 1920; xxxix., 279,
1922; xl, 513, 1923.
Acta O., viii, 253, 1930.
Weekers and Bonhomme.
xcix, 180, 1940.
Ophthalmologica,
Werner. Kinska Lak. Sal. Hld., lxxiii, 196,
1931.
Acta O., ix, 112, 275, 286, 1931 ; x, 426,
1932.
Wheeler. A. of O., xvi, 569, 1936.
Wiener and Alvis. Am. J. O., xx, 497, 1937.
Wilder. Am. J. O., xiii, 17, 1904.
J. Am. Med. As., lxxxi, 2095, 1923.
Wille. Brit. J. O., xx, 229, 1936.
Wilmer. T. O. S., xlvii, 230, 1927.
Wolfe and Blaess. Am. J. O., xix, 400, 1936.
Woods. A. of O., xiv, 936, 1935.
Wootton. T. A m. O. S., xxx, 64, 1932.
Wright and Nayar. Brit. J. O., ii, 456, 1929.
Yoshida. Am. J. O., vi, 356, 1923.
Young. T. O. S., xliv, 338, 1924; xlvi, 181,
1926; liii, 348, 1933.
Ziehe and Axenfeld. Samml. Abh. d. Geb. d.
Aug., ix, 1, 1901.
Zorab. Ophthalmoscope, x, 258, 1912; xi,
211, 1913.
HYPOTENSION : OPHTHALMOMALACIA
The subject of ocular hypotension has excited much less interest and
speculation than that of hypertension, partly because its effects are less
dramatic and partly because it is usually a consequential and incidental
event in a clinical picture dominated by other more impelling factors; but
nevertheless it is of considerable theoretical and practical importance. Just as
glaucoma is the condition produced by a raised tension, so ophthalmomalacia
may be used to indicate the state characterized by a lowered tension.
3418 TEXT-BOOK OF OPHTHALMOLOGY
Historically the first writer to devote particular attention to states of markedly
low tension was v. Graefe (1866), who termed the condition essential phthisis. Then
followed the work of Nagel (1867), Swanzy (1870), Landesberg (1871), and others,
the general opinion being that whereas glaucoma represented an increased secretion
of intra-ocular fluid, ophthalmomalacia was the result of diminution of secretion
which could be caused by vascular or nervous disorders. Among the most compre-
hensive studies in the pathology of the condition are those of Treacher Collins
(1916–18); while from the physiological and aetiological points of view the work of
Magitot (1917–33), of Paris, is outstanding.
Aºtiology
The causes of a fall in the intra-ocular pressure to a level considerably
below normal are various and little understood. The most important of
them, however, may be summarized as follows:
1. CONGENITAL. Just as congenital hypertension may occur, so it would appear
that an eye may show a consistently sub-normal tension from birth. The phenomenon
is rare and is associated with other congenital anomalies. Thus Knapová (1929)
reported a case of an enophthalmic hypermetropic eye, the tension of which never rose
above 8 mm. Schiótz, and Mazal (1934) a family wherein the three siblings showed
hypotension with irideremia or colobomata.
2. MYOPIA
A connection between hypotony and high axial myopia is well known,
but the association is not by any means invariable ; indeed, as we have
already seen, myopia is not rarely associated with glaucoma. It is probably
the case, however, that about 1/3 of myopic eyes over —8 or – 10 D
have a sub-normal tension (Lagrange, 1922), a tendency well brought out by
Urio (1933) who found over a series of 35 markedly anisometropic cases with
unilateral myopia, that in 80% the eye with high myopia had a tension lower
than its fellow. This subject will be discussed more fully when dealing with
refractive errors; but it would seem that the sub-normal tension is associated
particularly with chorio-retinal degeneration and a fluid vitreous full of fine
opacities. " .
3. RETINAL DETACHMENT
We have already seen that although a retinal detachment is usually
popularly associated with a low tension, this is by no means the rule ;
while statistics vary considerably, some authors finding hypotension
almost invariably (Lauber, 1908; Kümmell, 1920), some in equal proportions
with a normal tension (Horstmann, 1891), and others rarely (Deutschmann,
1910), it is probable that in the majority of cases the tension is normal at
first, or may even on occasion be raised, but tends to fall to a subnormal level
as the age of the detachment increases (Nordenson, 1887; Leber, 1916;
Fleiner, 1933). The phenomenon is probably due to a disturbance of the
normal circulation of the intra-ocular fluid which has the opportunity of
ANOMALIES OF THE INTRA-OCULAR PRESSURE 34.19
draining out through a retinal hole into the sub-retinal space. Such an easy
exit is taken preferentially by material such as indian ink injected into the
vitreous in animals instead of the normal but longer route anteriorly through
the angle of the anterior chamber.” The disorganization of the normal
drainage mechanism is seen in a lessening of the influence of the general
blood-pressure upon the intra-ocular pressure, the loss of the diurnal pressure
variation, and in the failure of the ophthalmotonic response after paracentesis
(Magitot and Hallard, 1931).
The fall in tension in retinal detachments is usually not very great ; but
occasionally an acute phase of hypotony occurs suddenly when the tension
of the eye falls so that the globe can hardly be palpated through the closed
lids, a phenomenon first noted by Schnabel (1876) and fully authenticated
by Leber (1916). In investigating this manifestation Beigelman (1929)
concluded that it was due to the passage of fluid from a liquefied vitreous in
quantity through the retinal hole to the sub-retinal space and its absorption
into the choroidal circulation; but an alternative explanation is that the
fall is due to an irritative cyclitis caused by the detachment.
4. TRAUMA
Traumatism is one of the commonest causes of ocular hypotension. It
is, of course, understandable that in perforating wounds wherein a loss of
aqueous or vitreous occurs, a profound hypotony follows immediately ; but
the same result may be seen after a minimal perforating wound, as by a
minute foreign body which has not allowed the escape of any of the contents
of the globe, a clinical phenomenon which has been verified experimentally
in animals (Weekers, 1932). In addition, a similar phase of hypotony may
follow a contusion to the globe, either a direct injury or an indirect contusion
which has primarily affected the facial bones. Prolonged pressure on the
globe by bandaging or massage has the same effect, as well as orbital pressure
as by a haematoma.
The most characteristic type of trauma is that of a direct contusion
(Fig. 2887). Magitot (1920) has shown experimentally that the fall of pressure
is preceded by a rise ; but in clinical conditions either a fall or a rise of
pressure may occur. If hypotony develops, its duration may vary consider-
ably, the tension returning to normal in the majority of cases within a few
days, but occasionally lasting so long that it appeared probably permanent
(Leplat, 1890; Collins and Hinnell, 1901; Collins, 1916–17 ; Magitot,
1917–33; Weekers, 1932; and many others). Moreover, it frequently
behaves irregularly, the pressure rising one day and falling the next, perhaps
to remain sub-normal for a considerable time, occasionally to become
temporarily very low, and all the time being unaffected by atropine. Some-
times the vision remains good despite the change of tension, and at other
times it deteriorates markedly, frequently with no particularly obvious
* Vol. I, p. 454 (458).
3420 TEXT-BOOK OF OPHTHALMOLOGY
lesion to account for the functional failure. Not the least interesting and
important of the features of the condition is the fact that the fellow eye,
although quite unaffected by the contusion, may share the hypotony reflexly
(Magitot, 1933).
The almost universally accepted aetiology advanced for such a traumatic
hypotony by the older writers was an arrest of the secretion of the intra-
ocular fluid owing to paralysis of the fibres of the sympathetic nerve (Leplat,
1890; Nettleship, 1901 ; and others). Such a theory, however, has no
proven foundation. Alternatively, a disturbance of the vascular circulation
has been suggested such as would cause a diminution in the formation of

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FIG. 2887.-TRAUMATIC HYPOTONY.
After a contusion. The dotted line the sound eye, the continuous line the injured
eye.
intra-ocular fluid. The severance of the posterior ciliary arteries with the
retention of normal intra-ocular tension in the operation of optico-ciliary
neurotomy suggested to Collins (1916) that these could have little to do
with the matter, and he brought forward evidence that a tearing of the
anterior ciliary arteries in a traumatic cyclodialysis was the causal
factor. More likely, however, is the effect of traumatism influencing the
minute vessels directly (Schmidt and Decker, 1930; Poos, 1931). Another
hypothesis depends upon the development of increased facilities for the
drainage of intra-ocular fluid, a hypothesis supported by the experiments
of Weekers (1932) who found an acceleration of the passage of fluorescein
through the aqueous humour. Such an increase in the elimination of fluid
is undoubtedly the cause of hypotony after prolonged pressure upon the
globe (Wessely, 1905), and a contusion may act similarly on occasion by
increasing the capacity of the existing drainage channels or by opening new
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3421
ones, as by an incomplete internal scleral rupture in the region of the canal
of Schlemm (Fuchs, 1911), or by a rupture of the pectinate ligament (Collins,
1892–1916). Into such a category would come the hypotony which may well
follow the operative production of a filtering scar undertaken for the relief
of glaucoma. While it is true, however, that these factors may exert some
influence, perhaps in some cases a preponderating influence, on the produc-
tion of hypotony in contusions and other traumatic conditions, it is probable
that the most potent cause is a disturbance of the circulation through the
local nervous azon-reflea mechanism which governs the condition of the
small vessels and is regulated by axon reflexes through the ganglionic nerve
system in the choroid, a factor which accounts not only for the upset
in the tension of the contused eye but also explains the similar reflex
phenomena in its fellow.
5. INFECTIONS AND INFLAMMATIONS
Any prolonged inflammation of the inner eye is liable to be followed by
a condition of lowered tension, a result most frequently seen when the
ciliary body is seriously involved, in which case the eyeball shrinks and may
become completely disorganized. It will be remembered that such a condi-
tion of ophthalmomalacia is particularly common in cases of keratitis," with
which, of course, some degree of uveitis is almost always combined. It can
also be brought about experimentally ; for, as shown by Weekers (1932),
scleral cauterization over the ciliary region in animals leads to a short-lived
phase of hypertension followed by a prolonged hypotonic phase.
6. GENERAL ILLNESSES
It is to be noted that a lowering of the intra-ocular tension may be
associated with severe general illnesses, particularly those involving toxaemic
states. Such an ocular hypotony is by no means always dependent upon
a lowering of the blood-pressure. Moreover, in poisoning such as by quinine
or the barbiturates, the ocular tension falls in spite of a rise in the blood-
pressure (Magitot and Offret, 1936).
DLABETIC COMA. The occurrence of acute hypotony in diabetic coma
was first noted by Krause (1904) and Heine (1906), and has since received
a considerable amount of attention (Imre, 1924–25; Elschnig, 1929;
Patek, 1929; Poos, 1932; and others). It is a phenomenon, however,
which has become much more rare since the more modern and efficient
treatment of diabetes has become universal. Abnormally low pressures
in this condition are relatively common, but with replenishment of the
body-fluids these are usually rapidly made good (Waite and Beetham,
1935); occasionally, however, in the most severe cases, especially in those who
do not survive, the loss of tension may be extremely profound, the cornea
at times collapsing as if a negative pressure existed. Such an ophthalmo-
1 Vol. II, p. 1875.
3422 TEXT-BOOK OF OPHTHALMOLOGY
malacia occurs in no other type of coma, and may serve as a diagnostic sign
of diabetes ; and it is also quite independent of the height of the blood-
pressure and the blood-sugar. . *
The cause of the hypotony is not clear. Hertel (1913) assumed that it
was due to changes in the molecular concentration of the blood ; a second
hypothesis attributes it to a condition of acidosis (Ehrmann and Esser,
1911), a factor which can lower the intra-ocular pressure considerably by
bringing about a diminution of the volume of the vitreous body (Duke-Elder,
1931): it is significant, however, that hypotony does not occur in acidosis
without coma, it attains its maximum with the development of coma and
disappears with it. It is interesting that Rönne (1913) and Kochmann
and Römer (1914) produced a prolonged hypotony in rabbits by the injection
of a small quantity of serum from comatose diabetics. The aetiology is
still obscure and it is possible that it is brought about by a number of factors
including the loss of tissue-fluid, acidosis, a fall of blood-pressure, and toxic
action upon the capillaries and their nervous regulating mechanism. It
is also of interest that de Jongh and Wolff (1924) produced a comparable
hypotony associated with convulsions in hypoglycaemia induced by insulin.
PRIMARY ANAEMIA. The primary anaemias are also associated with a
mild degree of ocular hypotony, particularly agranulocytosis and pernicious
anaemia (Suker, 1934). The decrease in tension is never so profound as in
diabetes, nor is it associated with ocular complications, but it may be very
prolonged, for the tension of the eye does not regain its normal level until
the blood-picture has become relatively normal. Any variation in the
blood-picture is likely to be accompanied by a variation in the tension of
the eye, the relationship being closer to the haemoglobin and red-cell count
than to the colour index and white-count.
7. NERVOUS INFLUENCES
We have already discussed the influence on the intra-ocular pressure
of the two nerves supplying the inner eye * : it will be sufficient here merely
to recapitulate the subject.
The TRIGEMINAL NERVE, apart from its share in mediating the local
axon reflexes within the eye, takes no part in the regulation of the intra-
ocular pressure, and the earlier observers who concluded that its stimulation
produced a rise in tension (v. Hippel and Grünhagen, 1868–69; v. Schultén,
1884; Bellarminow, 1886), were led astray by coincident stimulation of
the associated sympathetic fibres or by a spread of stimulation to the
neighbouring parts of the brain which caused a reflex rise in blood-pressure
(Henderson and Starling, 1904). Similarly, stimulation of the ciliary ganglion
is without influence on the tension of the eye, and its resection leaves it
unimpaired (Collins, 1916); the fall observed by Saitoh (1933) after this
operation has been unconfirmed. It is to be remembered, however, that if
* Vol. I, p. 521 (530).
ANOMALIES OF THE INTRA-OCULAR PRESSURE 3423
the nerve is diseased, as in herpes zoster, an acute hypotony may result
(Juler, 1928; Sédan, 1933; and others), an effect probably due to the
disturbance of local axon reflexes.
The syMPATHETIC has similarly little influence upon the production of
hypotony, and any action it has is due to the effect of vaso-constrictor
nerves on the vessels. Thus its stimulation causes a fall in intra-ocular
pressure due to vaso-constriction (Parsons, 1903; Henderson and Starling,
1904; Wessely, 1908; and others), but section of the cervical sympathetic
or extirpation of the superior cervical ganglion has given equivocal
results." If, however, the influence of the sympathetic is lost, there is
evidence that any factor tending to alter the intra-ocular pressure acts more
unrestrainedly and with greater effect (Leplat, 1923; Adler, Landis and
Jackson, 1924).
It is interesting that in cerebral diplegia the intra-ocular pressure is said to be
lower on the paralysed side, even although the blood-pressure is higher (Kahler and
Sallmann, 1923).
8. THE ENDOCRINE GLANDS
In discussing the aetiology of glaucoma we have already seen that
many authors contribute a preponderating influence to the ductless glands.
In ophthalmomalacia any problematical connection is much less close,
although some association is seen in the hypotony which sometimes accom-
panies pregnancy which Imre (1921) associated with hyperpituitarism, or
that which follows ovariectomy. A hypotony also has been noted
occasionally in hyperthyroidic states (Imre, 1921 ; Terrien, 1922), but
this is by no means the rule. There seems to be little experimental or
clinical justification for Lagrange's (1922) postulation of the frequent
occurrence of an “essential ophthalmomalacia '' in a vagotonic type of
individual who shows a general debility and lassitude, whose “cilio-spinal
sympathetic centre does not function, whose endocrine system functions
irregularly, and in whom “ the gland of the aqueous humour is deficient,”
in contrast to the sympathetico-tonic individual who is predisposed to
glaucoma with his nervous, emotional temperament, his vigorous outlook
and his tendency to congestion.
In résumé, therefore, it would seem that hypotony of the globe, apart
from incidental traumatic causes, is usually due to some disorganization of
the uvea, either by gross pathological lesions, whether inflammatory or degenera-
tive, or by more subtle interference with the circulatory system. Although the
intimate mechanism of the second type of lesion is not yet clear, the most
probable hypothesis would appear to be a disorganization of the local neuro-
genic control of the small vessels by the nervous reflexes in the eye itself, a factor
which may act either by decreasing the formation of intra-ocular fluid or
1 Vol. I, p. 522 (531).
T.O. —WOL. I J. 4 Q
3424 TEXT-BOOK OF OPHTHALMOLOGY
by increasing its elimination by an upset of the tone and permeability of the
arteriolar or venous components of the capillary bed. To this influence
must probably be added an alteration in the effectivity of the physiological
membrane forming the tissue-aqueous barrier : this, as we have already
discussed," is a factor as yet almost completely unknown, but it is easy to
understand that it would readily be disorganized in any uveal disease.
It is probable, therefore, that all types of ophthalmomalacia are secondary,
and that the classical differentiation of hypotony into two types—a
secondary type due essentially to a lesion of the ciliary body and a primary
type (the “essential phthisis” of Lagrange, 1922) due to a nervous disorder
which is capable of altering the secretion of the aqueous humour—is
misleading, the difference between them being one not of kind but of
obviousness.
Pathology
The pathological changes which accompany hypotony, while lacking
the dramatic nature of those associated with hypertony, nevertheless merit
some notice. The immediate effects of an acute fall intension are essentially
Fig. 2888-Hypotony.
GEdema of the ciliary body and iris with the formation of a coagulum of protein-
rich plasmoid aqueous in the anterior chamber and between the ciliary body and the
sclera causing a choroidal detachment (Collins, T. O. S.).
shown in the circulation and the composition of the intra-ocular fluid which
escapes from dilated and freely permeable capillaries. The universal vaso-
dilatation which occurs throughout the uveal tract is essentially a pressure-
effect, for the walls of the small vessels, deprived of the outer support of the
p. 3356.

ANOMALIES OF THE INTRA-00ULAR PRESSURE 34.25
normal intra-ocular pressure, give way before the blood-pressure, an effect
probably aided and intensified by a failure of the adjusting reflex nervous
mechanism. A plasmoid fluid therefore escapes from them in all directions,
into the tissues of the iris, the ciliary body and choroid causing an oedema
throughout (Fig. 2888). This becomes most evident in the richly vascularized
regions, such as the ciliary body, where large vesicles of oedematous fluid
may form under the ciliary epithelium (the vesicles of Greeff, 1894; Bauer,
1896; Henderson and Lane-Claypon, 1907; Rados, 1922) (Fig. 2889), or
in the iris, on the posterior surface of which similar collections of fluid may
become evident under the epithelium (Carlini, 1910; Carrère, 1923;
Fig. 2889.--THE CILLARY Body IN Hypotony.
In the rabbit after the experimental establishment of a fistula. Showing oedema
}} * of the epithelium (Henderson and Lane-Claypon, R. L. O. H.
Samojloff, 1925–27). Escaping out from the tissues, this protein-rich fluid
reaches all the available spaces in the eye, filling the anterior and posterior
chambers with a plasmoid aqueous, percolating the vitreous gel, distending
the supra-choroidal space and thus causing a detachment of the choroid
and sometimes of the ciliary body from the sclera," and occasionally
accumulating in mass between the retina and the pigmentary epithelium
and causing a detachment of the former tissue *. A final oedematous
manifestation is the development of papilloedema from uncompensated
intra-cranial pressure, a phenomenon which may be fleeting but may last so
long as to appear to be permanent; this complication has already been
discussed and the relevant literature detailed.”
p. 2538. * p. 2896. * p. 2945.

4 Q 2
3.426 TEXT-BOOK OF OPHTHALMOLOGY
In the higher degrees of ophthalmomalacia, particularly when the
condition is of long standing, the pathological picture is dominated by a
relaxation of the tissues usually kept taut. The cornea becomes wrinkled,
Fig. 2890.-Folds iN Bowman's MEMBRAN.E.
A common histological feature of shrunken
globes. The depressions are filled in with
epithelium which maintains the surface level
(x-55) (Parsons).
and the two elastic laminae show
folds, those in Bowman's mem-
brane producing superficial double
contour lines and those in
Descemet's membrane giving rise
to the clinical picture of striate
* keratitis.” Pathologically the
folds in Bowman's membrane
were first noted by Schirmer
(1896) in a shrunken globe in
which the cornea was much
diminished in size, and they have
repeatedly been described in con-
ditions of ophthalmomalacia from
any cause (Caspar, 1903–16 :
Weiche, 1905; Haab, 1916; Spicer and Greeves, 1916; Collins, 1917;
Fuchs, 1918; Reis, 1921): the membrane itself shows pronounced waves,
a configuration in which the anterior lamellae of the substantia propia
Fig. 2891.-HyPotoxy.
Note rucking of Descemet's membrane and the inner corneal lamellae; cedema
of the iris, and the coagulum in the anterior chamber (Collins, T. O. S.).
participates, but the epithelium fills in the troughs of the waves anteriorly
so that the surface of the cornea is smooth and regular (Fig. 2890). The
folds in Descemet's membrane were first noted in phthisical eyes by


ANOMALIES OF THE INTRA-00ULAR PRESSURE 3427
v. Graefe (1866) and were studied experimentally by Hess (1892–96), Schirmer
(1896), and Vogt (1919), and pathologically by Becker (1875), Fuchs (1902),
Parsons (1904) and others (Fig. 2891). They represent a purely mechanical
deformation in which the deeper layers of the substantia propria also
take part.
The sclera, too, becomes thickened and puckered, large furrows being
found behind the insertions of the recti muscles, while a wrinkling and
spacing-out of the fibres throughout, particularly in the deeper layers,
result in a thickening of the whole membrane (Fig. 2892). A similar
wrinkling of the elastic lamina of the choroid occurs, a process associated
FIG. 2892. HYPOToNY.
Experimental hypotony in the rabbit. The selera and choroid showing
coagulum of protein-rich fluid in the spaces between the two (Collins, T. O. S.).
as a rule with a heaping up of the pigmentary epithelium on the inner
surface where the folds are deep (Collins, 1917). Finally, the loss of
tension in the coats of the eye leads to a relaxation of the zonule and
a forward displacement of the lens with resulting myopia.
The clinical symptoms of ophthalmomalacia are, particularly in contrast
with those of hypertony, by no means striking: in the absence of the active
contraction of cyclitic membranes of inflammatory origin, pain does not
occur, and in the presence of very low tension an eye may retain its function
apparently unimpaired for years, a phenomenon frequently seen in decom-
pression operations in which excessive filtration occurs. On the other hand,
grave visual disabilities may appear and persist. These may be acute, as in
cases wherein pronounced papilloedema is present (Collins, 1916), or a

3428 TEXT-BOOK OF OPHTHALMOLOGY
persistent detachment of the choroid. More frequently, however, long-
distance nutritional effects may eventually appear, such as a diffuse opacity
of the lens or the development of a quiet atrophic or irritative condition of
the iris with occlusion of the pupil by the deposition of pigment (Juler,
1939). In most cases, however, the picture is dominated by the effects of
the initial lesion which has produced the hypotonic state.
Treatment by drugs such as atropine is usually inefficacious, for in the
presence of patent excretory channels the tension does not rise. The most
efficient method of raising the tension in such cases is by obliterating to
some degree the filtration channels, such as can be done by cauterization
around a segment of the limbus (colmatage) with a view to creating scar-
tissue (Kümmell, 1912; Lagrange, 1922). It is questionable, however, if
such an operation is frequently legitimate. As will be discussed in the section
on operative surgery, the hypotony due to a fistula or an over-active filtration
operation is usually best treated by covering the site with a conjunctival flap.
Adler, Landis and Jackson. A. of O., liii, 239,
1924.
Bauer. A. f. O., xlii (3), 193, 1896.
Becker. Atlas path. Topog. d. Auges, 1875.
Beigelman. A. of O., i, 463, 1929.
Bellarminow. A. ges. Phys., xxxix, 449, 1886.
Carlini. A. f. O., lxxvii, 96, 1910.
Carrère. C. R. S. Biol., lxxxviii, 475, 1923.
Caspar. K. M. Aug., xli (2), 289, 1903;
lvii, 385, 1916.
Collins. T. O. S., xii, 180, 1892; xxxvi, 204,
1916; xxxvii, 281, 1917; xxxviii, 217,
1918.
Ophthalmoscope, xiv, 348, 1916.
Collins and Hinnell. T. O. S., xxi, 100, 1901.
Deutschmann. A. f. O., lxxiv, 206, 1910.
Duke-Elder. J. Phys., lxxi, 268, 1931.
Ehrmann and Esser. Z. kl. Med., lxxii, 496,
1911.
Elschnig. Karlsbad. dirtzl. Vortr., x, 158, 1929.
Fuchs. T. O. S., xxii, 15, 1902.
A. f. O., lxxix, 53, 1911; xcvi, 323, 1918.
v. Graefe. A. f. O., xii (2), 256, 1866.
Greeff. A. f. Awg., xxviii, 178, 1894.
Beit. 2. Aug., x, 1, 1916.
Haab. Beit. 2. A ug., x, 1, 1916.
Heine. K. M. Aug., xliv (2), 451, 1906.
Henderson and Lane-Claypon. R. L. O. H.
Rep., xvii, 97, 1907.
Henderson and Starling. J. Phys., xxxi, 305,
1904.
Hertel. K. M. Aug., li (2), 351, 1913.
Münch. med. W., lx (1), 1191, 1913.
Hess. A. f. O., xxxviii (4), 1, 1892.
A. f. Aug., xxxiii, 204, 1896.
v. Hippel and Grünhagen. A. f. O., xiv. (3),
219, 1868; xv (1), 265, 1869.
Horstmann. B. O. G. Heidel.., xxi, 140, 1891.
Imre. A. f. Aug., lxxxviii, 155, 1921.
A. of. O., liii, 205, 1924.
B. O. G. Heidel.., xlv., 41, 1925.
de Jongh and Wolff. Ned. tij. w. Gen., lxviii,
2703, 1924.
Juler. T. O. S., xlviii, 179, 1928 ; lix, 253,
1939.
kahler and Sallmann. Wien. kl. W., xxxvi,
883, 1923.
Rleiner. A. f. O., czzix, 485, 1933.
Knapová. Cas. lek. cesk., i, 348, 1929.
Kochmann and Römer. A. f. O., lxxxviii,
528, 1914.
Krause. XXI Cong. Inn. Med., xviii, 439,
1904.
Kümmell. A. f. Aug., lxxii, 261, 1912.
B. O. G. Heidel.., xlii, 231, 1920.
Lagrange. Du Glaucome et de l’Hypotonie,
Paris, 1922.
Landesberg. A. f. O., xvii (1), 292, 1871.
Lauber. Z. f. Aug., xx, 118, 208, 1908.
Leber. G-S. Hb., II, vii (a), 1422, 1916.
Leplat. An. d’Oc., ciii, 209, 1890; clx, 348,
1923.
Magitot. An. d’Oc., cliv, 660, 683, 1917;
clv, 1, 66, 1918; clvii, 680, 1920; clzx,
465, 480, 1933.
Magitot and Hallard. Bull. S. O. Paris, xliii,
100, 1931.
Magitot and Offret. Bull. S. d’O. Paris,
xlviii, 163, 1936.
Mazal. 1931 see Z. f. Aug., xxxii, 218, 1934.
Nagel. A. f. O., xiii (1), 407, 1867.
Nettleship. T. O. S., xxi, 120, 1901.
Nordenson. Die Netzhautablósung, Wies-
baden, 1887.
Parsons. The Ocular Circulation, London,
1903.
Path. of the Eye, London, i, 181, 1904.
Patek. J. Am. Med. As., xcii, 438, 1929.
Poos. K. M. Aug., lxxxvi, 535, 1931; lxxxix,
145, 1932.
Rados. A. f. O., ciz, 342, 1922.
Reis. A. f. O., ev, 617, 1921.
ANOMALIES
3.429
OF THE INTRA-OCULAR PRESSURE
Rönne. A. f. O., lxxxv, 489, 1913.
Saitoh. Acta S. O. Japan, xxxvii, 729, 1933.
Samojloff. K. M. Aug., lxxv, 382, 1925.
A. f. O., czviii, 391, 1927.
Schirmer. A. f. O., xlii (3), 1, 1896.
Schmidt and Decker. A. f. Aug., cii, 700,
1930.
Schnabel. A. f. Aug., v, 67, 1876.
v. Schultén. A. f. O., xxx (3), 1 ; (4), 61,
1884.
Sédan. Rev. d’Otol., xi, 601, 1933.
Spicer and Greeves. Ophthalmoscope, xiv,
116, 1916.
Suker. Am. J. O., xvii, 125, 1934.
Swanzy. An. d’Oc., lxiv, 212, 1870.
Terrien. A. d’O., xxxix,, 716, 1922.
Urio. A. f. O., czzxi, 377, 1933.
Vogt. A. f. O., xcix, 296, 1919.
Waite and Beetham. New England J. of
Med., coxii, 367, 429, 1935.
Weekers. A. d’O., xli., 641, 1924; xlviii, 321,
593, 1931; xlix, 24, 1932.
Weiche. Diss., Greifswald, 1905.
Wessely. K. M. Aug., xliii (1), 654, 1905.
A. f. Aug., lx, l, 1908.
CHAPTER XLI
INTRA-OCULAR PARASITES
INTRA-OCULAR parasites occur so rarely that they are legitimately
considered as ophthalmological curiosities. Moreover, very few types occur
in the eye, comprising representatives of two groups—worms and the
larvae of flies. Of the worms the group of Nemathelminthes (round worms)
is represented by several species of Filaria (thread worms), while the group
of Platyhelminthes is represented by the cysticercus and the echinococcus.
A. Nemathelminthes
FILARIA
The FILARIA or THREAD-WORMS have already been noted among diseases
of the conjunctiva." It will be remembered that they are unsegmented
thread-like worms which in the larval form circulate in the blood-stream
and in the adult form are parasitic in the lymphatics. Their evolution
depends upon an intermediate host, either an insect, usually a mosquito,
or a crustacean, and the initial human infestation, which is by the cutaneous
route, follows a bite from this host. Intra-ocular infection occurs apparently
on rare occasions by the worm boring its way through the coats of the
eye, but much more usually is probably due to the lodgment of the
micro-filariae in the intra-ocular vessels.
The geographical distribution of filariasis is therefore limited to the
habitat of the intermediate host. It is a malady of warm climates, generally
from 40° north to 30° south, and in temperate climates the disease is
unknown apart from individual cases returning from tropical or sub-tropical
regions; it is interesting, however, that the infection may lie dormant for
many years and may not become apparent until long after the traveller's
return. Various types of nematode have given rise to intra-ocular infection
and unfortunately most of the records in the literature suffer from lack of
precision in their zoological classification. In general terms, however, the
commoner visitants are :
1. The Filaria loa, found in tropical West Africa, particularly the
Congo, where the intermediate host is the blood-sucking mangrove fly
(Chrysops dimidiata or Chrysops silaceas).
2. The Filaria bancrofti, which has a wide distribution in the tropics,
1 Vol. II, p. 1665.
3430
INTRA-OCULAR PARASITES 3431
particularly India, China and the South Seas: it is generally agreed that in
India its carrier is the Culer fatigams.
3. The Filaria medimensis (Dracunculus medimensis) or guinea-worm,
which is distributed preferentially around the shores of the Red Sea, and
occurs in India and some parts of tropical Africa whence it was transported
by Negro slaves to Central and South America. The intermediate host is a
small crustacean (Cyclops quadricornis) which re-infests man through
drinking water.
4. The Onchocerca. This is a large group of Filaridae of which only
One species is known to infect man—the Onchocerca volvulus, which forms an
almost universal infection among the natives of Western Africa, the Congo,
Uganda, Senegal, the Cameroons, Kenya and the Sudan. It is also found
very extensively in Central America and in the adjacent portions of the
North and South American continents : for some time the American and
African species were considered separate entities, the former being called
Onchocerca caecutiens (the blinding filaria), but since there is no morphological
or biological differentiation between the two, it is probable that it was
imported into America in the course of the slave trade, and to-day the
separate terminology has been discarded. The vector is a fly of the genus
Simulium (buffalo gnats, black flies, etc.) which breeds in running water.
It is interesting that of the several other types of Onchocerca which infest
animals (horses, cattle, etc.), none is associated with ocular symptoms.
It is probable, however, that these types are by no means exclusive, and sporadic
cases have been reported in regions where filariasis is unknown, as in a case described by
Gabriélidès (1938) in a Greek who had never left his country. The suggestion has been
made to retain von Nordmann’s (1832) original term for all filarial ocular infections—
Filaria oculi human? ——but it has no zoological meaning.
With most of these types filarial infection of the eye is exceedingly
rare, and is confined to the chance entry into the eye of a parasite. There
are islands in the Pacific, for example, where from 90% to 100% of the
inhabitants carry F. bancrofti without any evidence of intra-ocular complica-
tion, and Europeans have been known to have been infested with filariasis for
periods upwards of 40 years without ocular symptoms (Manson-Bahr,
1939). At occasional intervals, however, an adult filaria has been seen in
the eye forming a group of cases of peculiar interest. In the case of Oncho-
cerciasis the matter is different, for in this condition infection with micro-
filaria is so common as almost to be the rule and is associated with a dis-
tressing amount of blindness in the endemic areas of Africa and America.
With regard to the chance invasion of the eye by adult worms the
earliest record of such a case is that of Mercier (1771), who recorded
the presence of a thread-worm in the anterior chamber of a negress from
San Domingo, and since that time reports have come to hand sporadically
of the lodgment of such parasites not only in the anterior chamber, but also
3.432 TEXT-BOOK OF OPHTHALMOLOGY
the lens, the vitreous, and the sub-retinal space, while instances have
occurred where the worm has bored its way right through the eye to the
accompaniment of much pain.
Thus in a case reported by Laignier-Terrasse (1932), a large worm several times
bored its way through the anterior chamber without apparently exciting much reaction,
its journeys, which occupied from one-quarter to three-quarters of an hour, being
observed in a mirror by the patient, a Frenchman of 32 years who had lived at Gabon.
Two filaria were eventually extracted surgically from the sub-conjunctival tissue and
the lid. Dufougeré (1910) also recorded a European living in the Congo who for years
had harboured a worm 30 mm. in length, which habitually wandered from one eye to
the other, and from the globe to the lid and the sub-conjunctival tissues. The worm
proved too mobile to be caught.
In the Anterior Chamber. The presence of a filaria in the anterior chamber
may at first have surprisingly little effect, although eventually a reaction is to
be expected. The worm is usually quite visible to the naked eye, for when it is
alive it swims about with great vigour,
flipping across the pupil as a bright
fluorescent reflex, or rapidly lashing and
winding itself round into a loose tangle
of coils: as it moves it may be seen
entoptically as a fleeting shadow by the
patient. The parasite may be at any
stage of development from a micro-filaria
Mühlens and Mylius, 1932) to quite a
large worm (32 × 2 mm., Gabriëlidès,
(1938), but on the whole the aqueous as a
medium seems to inhibit development.
Atropine may inhibit the movements and
kill the worm (Lopez, 1891; Schnaudigel,
Fig. *º º 1922), but pilocarpine apparently irritates
you). it and makes it burrow deeper into the
tissues (Gabriëlidès, 1938). After the worm has died, an eye which has
tolerated the living filaria well for a long period may suddenly become
inflamed (Coppez, 1894), or alternatively, the parasite may become calcified,
exciting no obvious reaction (Mayou, 1931) (Fig. 2983). The majority of
cases have, however, shown a considerable inflammatory reaction, the
irido-cyclitis being usually associated with some degree of keratitis, a
cloudy aqueous and vitreous opacities, and occasionally with a most severe
reaction and raised tension. Apart from these inflammatory evidences,
however, symptoms are absent unless the worms burrow into the ciliary
body, in which case the pain may be excruciating (Gabriëlidès, 1938).
The earlier observations of thread-worms in the anterior chamber
were in many cases doubtful and in most cases the species has not been
recognized (Mercier, 1771; Macnamara, 1864; Barkan, 1876; Bacheler,

INTRA-OCULAR PARASITES 3433
1880; Lopez, 1891 ; Drake-Brockman, 1894): that reported by Barkan
(1876), of an Australian in San Francisco, was an immobile white thread
on the iris removed by an iridectomy which Knapp considered to be
a Filaria medimensis. The following are observations of a later date and
among those only in a few cases (Wright, 1934; Fernando, 1934; Tawara,
1936) has an adequate zoological identification been made, the species in
all cases being F. bancrofti.
Coppez (1894) and Lacompte (1894), in a negress from the Congo, aged 2 ; the
worm remained moving about actively in the anterior chamber for a long period
without producing any inflammatory reaction, but the day after it died, iritis and
keratitis developed. It was then removed and found to be an incompletely developed
filaria 15-2 mm. in length.
Gauthier (1895), in a 5-year-old child from the Congo ; a filaria, 20 × 1 mm.,
flipped about in the anterior chamber so rapidly that it seemed like a reflex in the
pupil ; as it did so it temporarily obscured the vision.
Terrien and Prélat (1914) recorded the operative removal of a worm 20 mm. long
from the anterior chamber of a 6-year-old Congo negro : it had excited a nodular
reaction in the iris.
Spamer (1922) recorded a case in the Siberian War of a filaria which was first seen
in the anterior chamber ; subsequently it went into the vitreous cavity and eventually
disappeared without inducing inflammatory reaction.
Mayou (1931) described what appeared to be a partly calcified dead nematode
in the eye of a soldier wounded at Gallipoli (Fig. 3893).
Nayar and Pillai (1932), in an Indian of 25 years, first saw an adult worm (probably
F. bancrofti) wriggling about briskly in the vitreous with one end buried in a clot of
blood on the retina near the macula. The only inconvenience the visitor caused was
the entoptic shadow cast during its movements. Nineteen days later he saw flashes
of red and green light and the shadow disappeared. The worm, 2.5 cm. long, was
then found in the anterior chamber, whence it was removed through a keratome
incision, the parasite escaping with the gush of aqueous. Subsequently vision was
normal.
Wright (1934), in a Hindu of 25 years from the Madras area, saw an
actively moving parasite about 1 inch long lashing about in the anterior chamber ;
there was irido-cyclitis, a cloudy aqueous and vitreous, optic neuritis and retinal
haemorrhages. The parasite escaped through a keratome incision, and the vision
returned to normal. The worm was subsequently captured with great difficulty and
proved to be an adult F. bancrofti.
Fernando (1934) reported a similar case in Ceylon : the worm, about an inch long,
escaped through a keratome incision and was identified as F. bancrofti.
Tawara (1936) described a case in a Japanese of 34 years. There was an actively
motile worm, 10 × 0.5 mm., in the anterior chamber, and the reaction included corneal
Oedema, a cloudy aqueous, and an exudative iridocyclitis with synechiae. After
extraction the parasite proved to be a partially developed F. bancrofti.
McMullen (1937), in an Indian student with a severe iridocyclitis, saw a worm
moving about actively in the anterior chamber. The iritis died down on ordinary
treatment with the worm still apparently in the eye ; the subsequent history is not
recorded. Micro-filario bancrofti were found in the blood.
Gabriëlidés (1938) reported a case which ended tragically in a Greek of 20 years.
A filaria, 32 × 1.5 mm., coiled up in the anterior chamber had excited a violent irido-
cyclitis associated with secondary glaucoma and complete blindness. The
3434 TEXT-BOOK OF OPHTHALMOLOGY
administration of pilocarpine made the worm burrow into the ciliary body, thereby
causing pain so acute that the eye was excised.
Jones, Jordan and Sullivan (1938) saw an adult nematode which emerged from a
pocket in the iris; the free part, 7 to 9 mm. long, was in constant rapid coiling
movement. The worm was removed but escaped capture; it was probably a P. bancrofti.
In the Lens. The finding of filaria in the lens is so rare as to be question-
able. In a lens sent to him by v. Graefe, Nordmann (1832) found two dead
thread-worms of 2 mm. length, while Gescheidt (1833) found three varying
in length from 1-5 to 4-5 mm. in a lens sent to him by v. Ammon. Schöler
(1875) also demonstrated to the Medical Society of Berlin a living and
actively motile parasite 12 to 15 mm. long which Virchow pronounced a
Fig. 2894–FILARIA AT MACULA (Barrada).
filaria. It is significant that since these early records no others have appeared
in the literature despite the greater clinical facilities for observation with
the slit-lamp ; and although suspicious appearances have been reported
(Salzer, 1924–25), it seems probable that curious and discrete opacities,
such as, for example, those of coralliform cararact, may have been inter-
preted as parasitic. Were it not for the fact that von Nordmann and
Gescheidt were very eminent helminthologists, it would be natural to
question the occurrence of filaria in the lens altogether.
In the Posterior Segment. The early reports of clinical observations of
thread-worms in the vitreous are open to a considerable amount of doubt,
for some of these at any rate are not free from suspicion that they were
filiform opacities or hyaloid remnants (Quardi, 1858; Fano, 1868; Schöler,
1875; Fernandez, 1882; Eversbusch, 1891; Malgat, 1893). Two cases

INTRA-OCULAR PARASITES 3435
which have already been noted are, however, unequivocal—that of Spamer
(1922), wherein a filaria left the anterior chamber for the vitreous chamber
and then disappeared, and that of Nayar and Pillai (1932), wherein a worm
issued from the sub-retinal space into the vitreous and then travelled forward
into the anterior chamber, whence it was extracted. Two further cases are
authentic : that of Kuhnt (1892) in which a worm in the sub-retinal space
protruded into the vitreous, whence it was extracted operatively, and of
Barrada (1934) in which a worm in a similar position simply disappeared.
Ruhnt’s (1892) case occurred in a man of 31 years. In an otherwise normal eye
a pin-head white swelling was situated near the macula, which within 2 months had
increased in size and from which a worm protruded into the vitreous. Five months
later, seeing movements in the swelling, Kuhnt, concluding he was dealing with a
worm in the sub-retinal space making its way into the vitreous, made a scleral incision,
through which the parasite was carried with a stream of escaping vitreous ; it proved
to be a nematode worm.
Barrada’s (1934) case concerned a 15 year old Egyptian. Near the macula
was a red circular hole surrounded by a raised darkish zone radiating from which
were innumerable reflexes of folds of the internal limiting membrane. Protruding
from the hole was a nematode, resembling in its markings the Onchocerca worm, in
constant movement lashing the surrounding retina (Fig. 2894). The vitreous was
clear, vision was 3/60, and symptoms were absent except that the patient saw a long
black string waving about in front of the eye. Some time later the filaria disappeared,
leaving the central hole and an opaque film over the surrounding area.
It may be noted that Leber (1914) attributed the common occurrence of retinal
haemorrhages and exudative spots in the South Sea Islanders to vascular obstruction
by the larvae of Filaria bancrofti.
In intra-ocular filariasis a coincident eosinophilia is the rule, while microfilaria
may be found in the blood, but in quite a number of the cases these general symptoms
have been absent. No drugs are of any value at all in dealing with the infection ;
the ideal treatment is surgical removal if the parasite is at all accessible. In the
anterior chamber it may be washed out through a keratome incision ; if it is adherent
to the iris an iridectomy may be required ; but in the posterior segment its surgical
removal would appear to be fraught with serious difficulties. As we have seen, the
parasite may disappear or may migrate to a more accessible spot.
It is interesting that, although rare in man, intra-ocular filarious
parasites are more common in animals; they are well known in horses and
oxen to those practising veterinary surgery in India, Burma, Ceylon and
China (Lingard, 1906 ; Pridmore and Coats, 1915; and others), and have
been seen in dogs (Rossi, 1895). There are usually signs of irritation and
inflammation, iritis, turbidity of the aqueous and keratitis. If the parasite
is removed at an early stage, the prognosis is good ; but otherwise the outlook
is bad. The worm can be seen swimming about in the anterior chamber
with rapid lashing movements, and its extraction is usually easy. The
animal is thrown, the eye cocainized, a keratome incision made without the
loss of aqueous, and when the worm comes near its vicinity its lower lip is
depressed sharply by a curette so that the worm is expelled in the gush of
3436 TEXT-BOOK OF OPHTHALMOLOGY
aqueous (Elliot, 1909). Filariae have also been noted in the lenses of frogs
and fish (Nordmann, 1832; Salzer, 1907; Greeff, 1907).
ONCHOCERCLASIS
The Onchocerca volvulus, which, as we have seen, is a habitant of Central
and Western Africa and the central regions of America, is responsible for
widespread eye-disease, but its association with blindness has only recently
been recognized. From 40% to 100% of the native population of the
endemic areas are infected with the worm and the inhabitants of whole
villages are blind ; it is estimated, indeed, that in some provinces of the
Belgian Congo 10% of the entire population is blind from this cause. Both
in America and Africa this applies particularly to villages 2,000 to 3,000 feet
above sea-level where life is rendered almost unbearable, natives being
sometimes forced to evacuate their villages owing to the prevalence of the
small black lymph-sucking flies which form the intermediate host and vector
of the worm. The infestation is primarily associated with fibrous nodules
in the subcutaneous tissues (in Africa usually below the shoulders; in
Central America on the head). These nodules contain the adult male and
female worms, the latter of whom give birth to countless micro-filariae,
which pervade the skin in large numbers and may enter the eye.
The association of blindness with onchocerciasis was first recognized
by the natives of Guatemala (Calderon, 1917; Luna, 1918–19), a belief
which was discredited for some considerable time. In Africa, however,
the association was insisted upon by Hissette (1932), who found the micro-
filaria in the eye, while similar observations were made in Mexico (Mühlens
and Mylius, 1932). The question was finally cleared up by an expedition
into Guatemala and Mexico from the Department of Tropical Medicine of
Harvard University in 1930 and 1931, and a similar expedition to the
Belgian Congo in 1932, when vast numbers of the micro-filariae were demon-
strated in the conjunctiva, cornea, ciliary body and iris and choroid, and
sometimes in the aqueous and vitreous of affected patients (Strong, Sand-
ground, Bequaert and Ochoa, 1934): the presence of the adult worm in
the eye is exceptional (Wilson, 1933). In addition to the native population,
European residents in the affected districts may fall victims to the disease,
and a few cases have been recorded in England, in whom ocular symptoms
commenced soon after their return home from a sojourn in parts of Africa,
such as the Belgian Congo (Adams, 1938) or Kenya (Levy, 1939; McMullen,
1939).
Ocular symptoms commence gradually with photophobia, lacrima-
tion and diminution of vision, but usually without acute pain. There
is considerable ciliary and conjunctival injection, a marked deep punctate
keratitis, and cyclitis. The corneal infiltrations are at various levels
between Bowman's and Descemet's membranes, and appear as small
foci of a grey colour in the periphery, which coalesce to form larger plaques
INTRA-OCULAR PARASITES 3437
in the central area : there is considerable vascularization from the limbus
and keratic precipitates. An exudative cyclitis may develop, as well as a
severe chorio-retinitis, which leads to rapid optic atrophy and blindness.
Occasionally the anterior or the posterior segment of the eye may be affected
alone ; and in the former case micro-filariae may be seen in the cornea with
the slit-lamp and also swimming about actively in the aqueous, in which
event the movements of the parasites may be seen entoptically by the patient.
The treatment of ocular onchocerciasis is difficult. A very marked
decrease in the local symptoms is usually brought about by the ordinary
measures adopted for intra-ocular inflammation—atropine, heat, and so on.
Injections of stibosan have a marked lethal effect on the micro-filariae, a
claim which has been put forward for antimony and tartar emetic, but
since these drugs have no effect upon the adult worm their effect is evanes-
cent. The only known method of dealing with the latter is the methodical
excision of the sub-cutaneous tumours which contain them as they appear.
Desensitization treatment has also been advocated by injections of filarial
antigen (Murgatroyd, 1939); but no adequate method of radical extirpation
of the infection is known.
AsCARIs LUMBRICOIDES, although One of the most common of the nematodes, is
found in the eye only exceptionally. The larvae were found in quantity in the retina
and uvea by Suyemori (1925) after the experimental inoculation of guinea-pigs, and
Calhoun (1937) reported a unique case in a boy of 8 years. The larva caused a severe
irido-cyclitis with secondary glaucoma and a dislocation of the lens, and, although
an attempt to remove it was unsuccessful, the parasite eventually died and disintegrated
and was absorbed, whereupon the eye became quiescent.
Adams. Lancet, i, 545, 1938. |Hissette. An. S. Belge méd. trop., xii, 433,
Bacheler. Med. Rec., N.Y., xvii, 244, 1880. 1932.
Barkan. A. of O., v., 15, 1876. Jones, Jordan and Sullivan. A. of O., xx,
Barrada. Bull. O. S. Egypt, xxvii, 63, 1934. 1006, 1938.
Calderon. Enfermedad Nueva en Guatemala, Kuhnt. A. f. Aug., xxiv, 205, 1892.
1917. Lacompte. An. S. med. de Gand, Oct., 1894.
Calhoun. A. of O., xviii, 963, 1937. Laignier-Terrasse. Nemathelminthes et Plat-
Coppez. A. d’O., xiv, 557, 1894. helminthes de l’Appareil Oculaire humain,
T)rake-Brockman. O. Rev., xiii, 331, 1894. Paris, 1932.
Dufougeré. Bull. S. Path. exot., iii, 309, 1910. Leber. A. f. O., lxxxvii, 541, 1914.
Elliot. T. O. S., xxix, 23, 1909. Levy. P. R. S. Med., xxxii, 1620, 1939.
B. O. G. Heidel., xxi, 249, Lingard. J. Trop. Vet. Sc., i, 175, 1906.
Lopez. Rev. de Ciensas Medicas de Habana,
vi, 269, 1891.
Eversbusch.
1891.
Münch. med. W., xxxviii, 532, 1891.
Fano. Union med., Paris, iv, 389, 1868. Luna. Am. J. O., i, 122, 805, 1918 ; ii, 793, ,
Fernandez. Crom. med. Quir. Habana, v, 436, 1919.
1880 ; vii, 116, 1882. Macnamara. Indian Annals of Med. Sc., xvii,
Fernando. J. Trop. Med. and Hyg., xviii, 405, 1864.
17, 1934. Malgat. Rec. d’O., xv, 280, 1893.
Gabriëlidès. Am. d’Oc., clºxv, 581, 1938. Manson-Bahr. P. R. S. Med., xxxii, 1621,
Gauthier. Am... de l’Instit. Chir. de Bruacelles, 1939.
1895. Mayou. P. R. S. Med., xxv, 474, 1931.
Am... d’Oc., oxiv., 152, 1895. McMullen. P. R. S. Med., xxxi, 128, 1937.
Gescheidt.
1833.
Greeff. B. O. G. Heidel., xxxii, 77, 1905.
A. f. Aug., lvi, 330, 1907.
Z. f. O., iii, 405, 407, 420, 435,
T. O. S., lix, 587, 1939.
Mercier, 177 l ; see Parsons :
Eye, London, i, 321, 1904.
Path. of the
3438 TEXT-BOOK OF OPHTHALMOLOGY
Mühlens and Mylius. Z. f. Aug., lxxviii, 113, Schnaudigel. K. M. Aug., lxviii, 248, 1922.
1932. Schöler. Berlin. kl. W., xii, 682, 1875; xiii, 8,
Murgatroyd. P. R. S. Med., xxxii, 1623, 1876.
1939. - Spamer. K. M. Aug., lxviii, 248, 1922.
Nayar and Pillai. Brit. J. O., xvi, 549, 1932. Strong, Sandground, Bequaert and Ochoa.
Nordmann. Mikro. Beit. 2. Naturges. d. Harvard Inst. Trop. Biol. Med., No. 6,
Wirbellosen Tiere, Berlin, 1832. 1934.
Pridmore and Coats. T. O. S., xxxv, 387, Suyenori. Taiwan Igakkai Zasshū, i, 239,
1915, 1925.
Quardi. Congrès d'O., Bruxelles, p. 153, 1858. Tawara. Acta S. O. Jap., xl, 518, 1936.
Rossi. L’Allivatore, ceiv, 1072, 1895. Terrien and Prélat. A. d’O., xxxiv, 294, 1914.
Salzer. A. f. Aug., lviii, 19, 1907. Wilson. Rep. Giza Hosp. Oph. Lab., viii, 85,
B. O. G. Heidel.., xliv, 278, 1924. 1933.
A. f. O., cxv, 515, 1925. Wright. Brit. J. O., xviii, 646, 1934.
B. Platyhelminthes
1. CYSTICERCUS CELLULOSAE
We have already discussed 1 how the CYSTICERCUS CELLULOSAE, the
larva of the taenia solium, or large tape-worm inhabiting the intestine, is
carried to man by eating raw infected pork. Such an infection is of world-
wide distribution, but is exceedingly rare in England and North America.
In the middle of last century, however, it was common in Central Europe,
particularly North Germany. Thus v. Graefe (1866), who was the first to
see a cysticercus ophthalmoscopically and the first to extract one by opera-
tion, out of 80,000 ophthalmic patients, saw 90 cases of cysticercus between
1853 and 1866, most of which were intra-ocular, and Hirschberg (1904), also
in Berlin, saw 70 cases among 60,000 patients between 1869 and 1885, but
only 3 cases among 78,000 patients between 1886 and 1894, and none among
65,000 patients between 1895 and 1902. The fall was without doubt due
to more adequate meat inspection which was enforced in Berlin in 1883.
Up to the end of the last century Ballaban (1900) collected 324 cases from
the literature. In Central Europe infection became less, but has again become
more common since the First Great War ; thus in Rumania before the War
only one case was reported (Manolescu, 1885), that of a sub-conjunctival
cysticercus, while since the War 12 cases have appeared (Michail, 1935): a
similar increase has occurred over all Europe. In certain countries the disease
has always been sporadic and endemic : of these Russia forms the best
example. In the literature of this country some 150 cases were reported
up to 1928, of which more than 100 occurred after 1900 (Lotin, 1928); in
the Ural district Loginov (1933) saw 14 cases in 4 years, while epidemics
occur sporadically : thus Balabonina (1925) saw 20 intra-ocular cases
within 6 months at Lousow. Other Eastern countries are similarly affected :
as at Turin where Grignolo (1926) saw 30 cases between 1888 and 1915,
or Greece, where Cosmettatos and Anargyros (1924) saw 80 cases between
1900 and 1923; while in South America it is also common (62 cases among
70,000 patients, Burnier and Sales, 1935). Cases are also seen in the Far
1 Vol. II, p. 1673.
INTRA-OCULAR PARASITES 3439
East (Tso, 1937). Its frequency depends entirely on the habits and hygiene
of the people concerned (Zehender, 1887).
Intra-ocular infection by the cysticercus arises through the blood-
stream : the eggs of the tape-worm are deposited in the stomach, where
their envelope is dissolved allowing the cysticerci to escape into the circula-
tion, whence they are distributed throughout the body. Upwards of a
hundred can be thus disseminated, but it is a curious thing that bilateral
intra-ocular infection is exceedingly rare ; Hirschberg (1892) recorded one
case, Zieminski (1899) a second, and Würdemann (1903) a probable one.
The occurrence of more than one parasite in the same eye, although again
exceedingly rare, has been noted on more than one occasion (Becker, 1867;
Dufour, 1888; Schöbl, 1893; Alfred Graefe, 1893). The coincidence of
ocular and other infection, as of the brain or skin, is, however, more common
(v. Graefe, 1868; Hirschberg, 1892; and others).
In the eye itself the location varies, much the most common site being
sub-retinal, in which case the parasite enters through the posterior ciliary
arteries. Any part of the eye, however, may serve as a nidus or as a resting-
place. Thus in his 90 cases v. Graefe (1866) found 80 in the retina and
vitreous, 3 in the anterior chamber, l in the lens, 5 sub-conjunctival and
1 orbital ; Vosgien (1911), in reviewing 372 cases in the literature up to
1911, and Laignier-Terrasse (1932), reviewing the literature from 1911 to
1932, found the following distribution :
Vosgien Laignier-Terrasse
Sub-retinal e e e tº | 20 | 6
In the vitreous . & tº is 42 } ()
In the anterior chamber * & 20 |
Sub-conjunctival {º} * * 84 7
Scleral ſº * & * º - |
Michail (1935) in 11 cases found 7 sub-retinal, 2 in the vitreous, 1 in the
anterior chamber and 1 in the supra-choroidal space. In the last case the
scolex was lodged in the supra-choroidal space, the vesicle was in the
vitreous and the neck, surrounded by cicatricial tissue, traversed the retina
and choroid.
In the eye the life of the cysticercus is at least 2 years—probably
3 or 4—and while in the early stages when the parasite is minute, symptoms
may be absent, these always develop as it increases in size until the eye is
ultimately destroyed and vision lost. Parasites up to 15 mm. in diameter
have been reported (Hirschberg, 1876; Leber, 1886). It appears as a cystic
swelling, globular and translucent, with its wall freely scattered over with
T.O.-WOL. III. 4 R
3440 TEXT-BOOK OF OPHTHALMOLOGY
fine calcareous granules. Occasionally it performs leech-like movements,
while a heaving movement followed by a slower subsidence is common
(Wood, 1901). The head may be retracted as if into the cyst (Fig. 2895),
or may stand out upon the neck capped by its ring of hooklets, and with
its slow and regular movements, may recall the motion of an elephant's
trunk (Fig. 2896). As time goes on it acts as a foreign body and sets up a
chronic inflammatory reaction with leucocytosis and the proliferation of
connective tissue, occasionally resulting in actual suppuration, and the
circumscribed formation of bone is not unusual (v. Graefe, 1854–66;
Hirschberg, 1871–1904; Leber, 1886; Schroeder and Westphalen, 1889;
riffith, 1897; Berardinis, 1899; Wagenmann, 1891; Pincus, 1894; Stock,
Fig. 2895.-INTRA-ocular Fig. 2896.-INTRA-ocular
Cystic Enous. Cystic ERous.
In the vitreous : with retracted Head and neck extended
head (Griffiths, T. O. S.). (Griffiths, T. O. S.).
1918; Kress, 1924; and many others). The suppuration is probably due
to chemical rather than bacterial action, a fact which accounts for its
localization and the formation of an encapsuled abscess, a process, however,
which eventually leads to complete disorganization of the eye. The reaction
proceeds even after the death of the animal.
The experimental implantation of the cysticercus into rabbits eyes has been
investigated by Demaria (1919) and Yamaga (1938). Minute vesicles (0-1-0-2 mm.).
after exciting an irido-cyclitis, may be destroyed, but larger embryos (10 mm.) result
in an uncontrollable reaction leading to the production of corneal staphylomata and
ectasias resulting in destruction of the eye.
IN THE ANTERIOR CHAMBER. The presence of a cysticercus in the
anterior chamber is a great rarity. The first recorded cases, in the pre-
ophthalmoscopic era, were recorded here (Sömmering, 1830; Neumann,
1831; Mackenzie, 1839–51; and others). Up to the beginning of the
present century some 20 cases are to be found in the literature, among

INTRA-OOULAR PARASITES 3441
which the more interesting are those of v. Graefe (1854–66), Hirschler
(1858), Windsor (1862), Teale (1866), Krüger (1867), Hirschberg (1872),
Reynolds (1874), Kries (1878), Treitel (1885) and Prager (1892); but since
then reports have been fewer (Rembe, 1907;
Marquez and Pittaluga, 1915 ; Barsan, 1928 :
Guliakov, 1936). As a rule the parasite is attached
to the iris (Fig. 2897), only occasionally to the
anterior capsule of the lens (Marquez and Pittaluga,
1915; Guliakov, 1936). In this position it is very
well seen, altering its shape and protruding its
head, movements which may produce visual dis-
turbances. Eventually, however, its presence gives
rise to symptoms, and if the parasite is left a severe
plastic iritis results, although its early removal may
- - - Fig. 2897. – Cystic ERCU's
allow good vision to be retained. on THE IRIs (Teale,
IN THE LENs. A cysticercus in the lens is R. L. O. H. Rep.).
exceptional. We have already seen that some of
the parasites in the anterior chamber may be adherent to the anterior
lens capsule (Marquez and Pittaluga, 1915 ; Guliakov, 1936); and v.
Graefe (1866), after extracting a cataract which was complicated by violent
iritis, found in it a cysticercus 6 mm. in diameter.
IN THE POSTERIOR SEGMENT of THE EYE. This is much the most
common locality for the presence of a cysticercus (Fig. 2898). As a rule
the parasite is found in the sub-retinal space probably indicating an entrance
through the posterior ciliary arteries. In this position the movements of
the worm give rise to an extensive detachment of the retina, the parasite
not only showing contractions in itself but migrating from one part of the
fundus to the other. Thus within 4 months Michail (1935) observed a worm
travel from the supero-temporal region to below the macula, and then to
the temporal side and eventually the infero-nasal side of the disc. Moreover,
quite frequently it has a tendency to try to get to the vitreous. On occasion
its progress through the retina has been observed, a process accompanied
by a considerable reaction and violent pain, and usually obscured by the
formation of a cloud of vitreous opacities or haemorrhages (Cirincione, 1907).
As we have seen, the animal may be caught on the way as in the case recorded
by Michail (1935) wherein the vesicle was in the vitreous and the head
remained in the supra-choroidal space, the neck being partially strangled
in scar tissue in the choroid and retina. If it is situated in the retina less
harm is done and the parasite gets into the vitreous more easily and without
exciting pain or opacification of the media: in this event it is well seen
with the ophthalmoscope. In the vitreous it is tolerated much more easily
than when situated in or between the membranes of the eye, but eventually
an exudative uveitis develops. The most common situation in the vitreous
is pre-retinal: thus Lewitzky (1935) in reporting 12 cases found 10 pre-

4 p. 2
3442 TEXT-BOOK OF OPHTHALMOLOGY
retinal and 2 sub-retinal. Here it may be fixed in position; but it may lie
in the centre of the vitreous cavity, its active movements being easily visible,
or it may come up close behind the lens. An exceptional occurrence is its
migration forwards through the pupil into the anterior chamber (Michall,
1935).
The diagnosis of an intra-ocular cysticercus when it is alive and well
seen is unmistakable. The globular translucent swelling formed by
the head, the heaving movements of the cyst itself and the undulating
movements of the head resemble nothing else. Sometimes the movements
are accentuated by light (Cirincione, 1907), and they may be elicited by
-
Fig. 2898-INTRA-ocular Cystic ERcus (D. J. Wood).
passing a galvanic or faradic current through the patient's head (de
Vincentiis, 1899). In the sub-retinal space diagnosis may be more difficult,
but the presence of a movable swelling which changes its position from time
to time is diagnostic (Pavia and Durando, 1933; and others). In the later
stages when a severe exudative uveitis has developed and disorganization
of the globe has commenced, diagnosis may be impossible, and syphilis,
tubercle or an intra-ocular tumour may be simulated.
The local symptoms may be confirmed by systemic diagnosis, the two
important points in which are the discovery of worms in the intestinal
tract and the presence of a positive complement fixation test. It must be
remembered, however, that neither of these is constant. The intestinal
parasites may not be evident or may have been evacuated before the
ocular symptoms appeared, and in quite a proportion of intra-ocular cases
the complement fixation test is negative (30%, Sales, 1934).

INTRA-OCULAR PARASITES 3443
Prognosis. Provided the parasite is in an accessible position and is
removed early, the prognosis may be good ; but if it is left, even although it
may die, disorganization and shrinkage of the eye is to be expected : blind-
ness may indeed follow its successful operative extraction (Lech, 1931).
Treatment
Medical methods of treatment have been suggested in dealing with
cysticercus infection—the administration of turpentine, filix mas, arsenic,
iodides, and so on—but from the Ocular point of view they are quite
useless. Neither are attempts to kill the parasite in situ effective, as by
electrolysis (Dor, 1882), or radium (Balbuena, 1928), or the injection of
sublimate solution (Bardelli, 1922), since even after its death disorganization
of the eye may ensue. The only satisfactory treatment is the surgical
removal of the parasite at the earliest possible time, before it has had the
opportunity to affect the eye gravely. This is most easily accomplished in
the anterior chamber, in which case the worm can be removed by itself, or, if
it is adherent to the iris, with an iridectomy. Guliakov (1936), in his case
wherein the parasite was adherent to the lens, caused a traumatic cataract
in its removal which eventually became completely absorbed. In the
posterior segment extraction is more difficult, but is frequently possible.
If the parasite is small and floating freely in the vitreous this may be
impracticable, and it may be necessary to enucleate the eye. The best
prognosis, of course, attaches to those cases where the parasite is under the
retina, for in this position it can be accurately localized and can be extracted
without loss of vitreous.
The localization of the cyst previous to operation is important ; this
is done in the same way as in the localization of a retinal hole, and is usually
most easily performed by the method of Gonin " (Verdaguer, 1934; Bruck,
1935; and others). Opposite this site an incision is carefully made through
the sclera, layer by layer, and the most happy result is that the parasite
drops out of the eye when the lips of the wound are pulled apart with the
loss only of sub-retinal fluid. If the retina is also injured, some vitreous
may be lost. The first operation to be performed was by v. Graefe who
made a large iridectomy and then extracted the worm by forceps through
a scleral wound about two lines behind the corneo-scleral margin. If the
cysticercus is situated far posteriorly, instruments may be introduced into
the eye from an anterior incision (Treitel, 1890), a Barraquer’s sucker may
be employed (Elschnig, 1925), or, more satisfactorily, adequate access may
be obtained by a Krönlein operation (Bruck, 1935).
With such technique a considerable amount of success may be achieved ;
failures are to be expected in 30 to 40% of cases.
Thus Alfred Graefe (1885) reported 45 operations : in 30 of these the parasite
was extracted, and in 15 the worm could not be removed, sometimes because of lack
of localization owing to vitreous opacities, sometimes because it was moving about
1 Vol. II, p. 1180.
3444 TEXT-BOOK OF OPHTHALMOLOGY
freely in the vitreous. Of the 30 successful operations A. Graefe was able to follow
up 24; in almost all of them the inflammatory symptoms subsided and several of
them retained useful vision. Of the 15 unsuccessful cases, all the eyes had eventually
to be enucleated. Wagner (1895) analysed the results of operation in 60 cases of
cysticercus in the vitreous ; among these in 11 the extraction failed and the eye had
to be enucleated, in 5 extraction was successful, but the eye had eventually to be
enucleated, in 44 (73%) the extraction was successful and the eye was retained ; of
this last group, in 21 blindness ensued, in 4 vision remained the same, and in 19
(31.5%) it increased. Out of 44 sub-retinal cases, in 14 the extraction failed and the
eye was lost, while in 30 (68%) the extraction was successful : 1 of these was lost
trace of, 1 resulted in phthisis, 10 remained blind with a cosmetically valuable eye,
in 9 the previous vision remained and in 9 it increased ; that is, vision remained or
increased in 40.86%. More modern writers, although with more limited experience,
get approximately the same results. Thus, using a Krönlein approach, Bruck (1935)
reported 3 successful extractions with the retention of some vision, and Mattos (1936)
recorded 10 successful extractions out of 11 cases. The visual results, however, as
would be expected, are frequently poor, usually as a result of an extensive retinal
detachment.
As would be expected, a cysticercus in the brain can affect the eye indirectly,
acting as a cerebral tumour and causing papilloedema and optic atrophy (Saba, 1935).
Hirschler. A. f. O., iv (2), 113, 1858.
Rraemer. G-S. Hb., II, x (18), 87, 1899.
Balabonina. Russ. O. J., iv, 900, 1925.
Balbuena. A. de Oft. H.-A., xxviii, 1, 1928.
Ballaban. Wien. med. W., 1, 2018, 2070, 1900. Kress. Am. J. O., vii, 182, 1924.
Bardelli. Boll. d’Oc., i, 19, 1922. Kries. A. f. O., xxiv. (1), 148, 1878.
Barsan. A. d’O., xlv., 694, 1928. Krüger. K. M. Aug., v, 59, 1867.
Becker. Wien. med. W., xvii, 889, 1867. Laignier-Terrasse. Nemathelminthes et
Berardinis. An... dº Ott., xxviii, 349, 1899. Platyhelminthes de l’Appareil Oculaire
Bruck. A. of O., xiii, 1042, 1935.
Burnier and Sales. Arquiros do Inst. Penido
Burnier, iv, 131, 1935.
Cirincione. A. f. Aug., lvii, 263, 1907.
Cosmettatos and Anargyros. A. d’O., xli,
558, 1924.
Demaria. Acta y trab. L. congr. med., Buenos
Ayres, 1919: Ref. K. M. Aug., lxiv, 133,
1920.
Dor. A. d’O., xxviii, 567, 1882.
Dufour. Rev. med. de la Suisse rom., viii,
453, 1888.
Elschnig. K. M. Aug., lxxv, 130, 1925.
Graefe, Alf. A. f. O., xxiv. (1), 209; (3), 267,
1878; xxxi (4), 33, 1885.
Cb. pr. Aug., xvi, 362, 1892.
K. M. Aug., xxxvi, 261, 1893.
v. Graefe. A. f. O., i (1), 453, 1854; ii (1),
259, 1855; iii (2), 308, 1857; iv (2), 171,
1860; ix (2), 79, 1863; xii (2), 174,
1866; xiv. (3), 143, 1868.
Griffith. T. O. S., xvii, 220, 1897.
Grignolo. Boll. d’Oc., v, 378, 1926.
Guliakov. Sov. vestm. O., ix, 512, 1936.
Hirschberg. A. f. Aug., ii, 227, 1871.
A. f. path. Amat., liv, 276, 1872.
Berlin. kl. W., ix, 485, 1872.
A. f. O., xxii (3), 146, 1876.
Beit. 2. Aug., iii, 71, 1878.
Cb. pr. Aug., iii, 173, 1879; ix, 417, 1885;
x, 265, 1886; xvi, 300, 1892; xvii, 135,
1893; xxviii, 241, 1904.
hºwmain, Paris, 1932.
Leber. A. f. O., xxxii (1), 281, 1886.
Lech. K. M. Aug., lxxxvii, 105, 1931.
Lewitsky. Z. f. Aug., lxxxvi, 300, 1935.
Loginov. Sov. vestm. O., ii, 5, 1933.
Lotin. Russ. O. J., viii, 186, 1928.
Mackenzie. Treatise on Diseases of the Eye,
III, 910, 1839.
Med.-Chir. Trans., xxxii, 41, 1849.
An... d’Oc., xxv, 60, 1851.
Manolescu. Fragmente medicale, 147, 1885.
Marquez and Pittaluga. A. de Oft. H.-A.,
xv. 349, 1915.
Mattos. Rev. Oto. Neuro-Oft., xi, 235, 1936.
Michail. An. d’Oc., clxxii, 385, 1935.
Neumann. Rust’s Mag., xxxiii, 529, 1831.
Pavia and Durando.
viii, 394, 1933.
Rev. Oto. Neur. Oft.,
Pergens. K. M. Aug., xxxiv, 434, 1896.
Pincus. A. f. O., x1 (4), 231, 1894.
Prager. Wien. kl. W., v, 209, 1892.
Rembe. O. Rec., xvi, 20, 1907.
Reynolds. American Practitioner, 336, 1874.
Saba. An. di Ott., lxiii, 13, 1935.
Sales. Arquiros do Inst. Penido Burnier, iii,
183, 1934.
Schöbl. Cb. pr. Aug., xvii, 101, 1893.
Schroeder and Westphalen. A. f. O., xxxv (2)
97, 1889.
Sömmering. Oken’s Isis, viii, 717, 1830.
Stock. K. M. Aug., lx, 791, 1918.
Teale. R. L. O. H. Rep., v, 318, 320, 1866.
INTRA-00ULAR PARASITES 3445
Treitel. A. f. Aug., xv, 258, 1885; xxi, 270, Windsor. R. L. O. H. Rep., iii. 322, 1862.
1890. Wood. T. O. S., xxi. 89, 1901.
Tso. Chinese Med. J.. li. 545, 1937. Würdemann. An of O., xii. 581, 1903.
Verdaguer. A. de Oft. H.-A., xxiv, 622, 1934. Yamaga. K. M. Aug., c, 414, 1938.
de Vincentiis. An. di Ott., xxviii, 191, 1899. Zehender. T. O. S. vii. 1, 1887.
Vosgien. These de Paris, 1911. Zieminski. Przeglad lekarski No. 2: Ref. Jb.
Wagenmann. A. f. O., xxxvii. (3), 125, 1891. ges. O., xxx, 331, 1899.
Wagner. Diss., Greifswald, 1895.
2. ECHINococcus
The larvae of the TAENIA ECHINococcus, a group of which with the
surrounding tissue-reaction constitutes a HYDATID CYST," is an extremely
rare occurrence within the eye, although
more common in the orbit. Cases
published by Gescheidt (1833) and Wood
(1906) are open to doubt, but fully
authenticated cases have been described
pathologically by Griffith (1897), Werner
(1903) and Demaria (1916), while a
clinical case was observed ophthalmos-
copically by Scholtz (1906) (Fig. 2899).
The possibility of such an infection has
been demonstrated experimentally by
Demaria (1916) who injected material from
hydatid cysts into the eyes of animals,
and by Dévé (1921) who injected the
scolices into the carotid artery of the
rabbit and produced intra-ocular cysts.
Fig. 2899.-ECHINococcurs IN
VITREous. (After Scholtz).
Gescheidt's (1833) case occurred in a youth of 24 who had ophthalmitis as a
child and died of phthisis. One eye showed a yellow opacity behind the lens which
proved post-mortem to be a cyst between the choroid and the retina. It contained
numerous oval bodies which on microscopical examination showed small suckers but
no hooklets.
Griffith's (1897) patient was a girl of 33 years who showed a glistening white,
opaque, non-vascular opacity in contact with the back of a clear lens; glaucoma
developed after the administration of atropine and the eye was enucleated with the
diagnosis of a tumour. Pathologically the entire vitreous cavity was lined by a dense
continuous membrane adherent all round to the lens, ciliary body and retina, which
proved to be a simple hydatid ecto-cyst, without, however, any trace of endo-cyst or
scolices. The retina, choroid and other parts of the eye were histologically normal.
Werner's (1903) case concerned a farm labourer of 28 whose eye presented an
appearance of a greyish-white mottled opacity immediately behind the lens: the
eye was staphylomatous and glaucomatous, and was removed with a diagnosis of a
tumour. Pathologically a hydatid cyst was found between the retina and the choroid:
it thus filled the posterior segment of the eye, occupying the sub-retinal space, the
lower half of the retina being doubled over the upper and being stretched and thinned
* Vol. II, p. 1672.

3446 TEXT-BOOK OF OPHTHALMOLOGY
Fig. 2900–INTRA-ocular Hyparin Cyst.
Cyst adherent to choroid. a. Parenchyma of cyst, b. Hyaline laminated
ectocyst. c. Layer of connective tissue and exudation. J. Choroid. e. Sclerotie.
(Werner, T. O. S.)
Fig. 2901,–INTRA-ocular Hypatin Cyst. Fig. 2002–INTRA-ocular Hypatin Cyst.
Hyaline laminated ectocyst, curved in- Ruptured brood capsule, showing scolices,
wards. The outer surface is greatly wrinkled. the majority with rostellum retracted. One
(Werner, T. O. S.). can be seen above with rostellum and hooks
evaginated, and the suckers on each side
(Werner, T. O. S.).


INTRA-00ULAR PARASITES 3.447
over the cyst. The cyst was surrounded by inflammatory and fibrous tissue, its wall
consisting of two layers, an outer hyaline laminated ecto-cyst and an inner delicate
parenchymatous endo-cyst (Fig. 2900). Arising from the latter were numerous brood
capsules each containing numbers of scolices, some of them retracted and others
extended showing the neck-like constriction and the head armed with hooklets and
suckers (Fig. 2902).
Wood’s (1906) case concerned a man of 52 from the Transvaal whose eye became
blind and painful and was excised. In the sub-retinal space were three cysts, one of
which was found to be a curling laminated membrane resembling a hydatid ecto-cyst.
Demaria's (1916) case occurred in a 35-year-old man, the eye being excised for
intractable glaucoma : a large hydatid with ecto-cyst, endocyst and secondary vesicles
filled the eye completely. -
Scholtz's (1906) case did not come to pathological examination since it occurred
in an only eye and was therefore not verified : a mass was seen ophthalmoscopically
to obtrude from a detached retina into a clouded vitreous in the upper quadrant of
the eye, and on it were some twenty small globular vesicles (Fig. 2899). Eventually
the mass was completely hidden by progressive vitreous opacities.
Demaria. A. de Oft. H. A., xvi. 597, 1916. Scholtz. A. f. Aug., liv, 170, 1906.
Dévé. A m. d’Oc., clviii, 721, 1921. Werner. T. O. S., xxiii, 193, 1903.
Gescheidt. v. Ammon's Z. f. O., iii, 437, 1833. Wood. T. O. S., xxvi, 152, 1906.
Griffith. T. O. S., xvii. 220, 1897.
C. Arthropoda
OPHTHALMoMYIASIS INTERNA
While an invasion of the conjunctival sac with the larvae of flies
(maggots) is not very uncommon (external ophthalmomyiasis),' invasion of
the inner eye is much rarer. The types of parasite
found in the eye are restricted : the most common
visitants are Diptera larvae of the (Estridae family,
particularly the Hypoderma bovis (hornet fly) which
infects cattle (Fig. 2903). A second parasite met with
occasionally is the Wohlphartia magnifica or flesh-fly,
which also infects barns and cow-sheds. Both have
similar effects upon the eye, for the symptoms, prognosis
and treatment depend rather upon the location of the
larva within the globe than upon its identity.
There seems to be little doubt that the larvae enter
the eye by direct penetration of its coats, the larva being
deposited on the skin near the eye from the patient's
hands and penetrating rapidly without being noticed.
Their burrowing habits are well known ; Brauer, for
example, placed an OEstrus larva on his arm to examine
it and it had vanished under the skin before he had time
to take up his magnifying glass. Such a direct portal of * º
entry has been demonstrated several times; thus Behr witH LARVA (B)
(1920), Zeeman (1926) and Archangelsky and Braunstein (Ennema, A. of 0.).
* Vol. II, p. 1676.

3448 TEXT-BOOK OF OPHTHALMOLOGY
(1931) demonstrated its dia-scleral course histologically, while occasionally
its path into the eye is marked by a corneal scar (Weisz, 1937): in
De Boe’s (1933) case the larva presented through the lamina cribrosa and
moved into the vitreous. The theory of Hess (1913) that ocular infection
is through the blood-stream has never been justified. It is probably for
this reason that the disease occurs almost entirely in young children in whom
the sclera is soft and readily penetrated ; Maggiore's (1922) case was aged
16, while cases in adults are very rare—De Boe (1933) aged 48, and
Anderson (1934–35) aged 46. It is interesting that both of these adult cases
were reported from America.
As would be expected, the occurrence of parasitic larvae in the human
eye is a rarity : it is a disease essentially of hot countries, and is usually
determined by close association of the population with infested animals
and by living conditions of filth and squalor. Most of the cases have been
reported from Russia, Germany, Austria : one comes from Sweden (Ståhl-
berg, 1901), one from England (Thomas and Parsons, 1909), two from Italy
(Maggiore, 1922; Bietti, 1923), two from the Netherlands (Zeeman, 1926;
Ennema, 1934), and two from America (De Boe, 1933; Anderson, 1934–35).
Cases also occur in northern Africa, India and South America (Linke, 1931).
In OPHTHALMOMYIASIS ANTERIOR, which comprises the great majority
of cases, the larva has entered the anterior chamber (Krautner, 1900 ;
Ståhlberg, 1901; Ewetzky and v. Kennel, 1904; Thomas and Parsons,
1909; Maggiore, 1917–22 ; Bietti, 1923; Kuriks, 1923; Hartmann, 1927;
Balod, 1934; Borsello, 1936; Spanyol, 1936; Weisz, 1937; Dérer, 1937).
In all cases a violent exudative irido-cyclitis has been set up with much
pain and reaction ; but in most cases, if the larva has been extracted, the
eye has quietened down with the retention of some vision, but sometimes,
even after a successful extraction, phthisis bulbi and blindness have resulted.
The extraction has sometimes been accomplished by forceps, but an iridec-
tomy may be required if the parasite is adherent to the iris.
Cases of OPHTHALMOMYIASIS POSTERIOR, wherein the larva has entered
the posterior chamber, are somewhat rarer. If the larva remains sub-retinal
in position, a violent exudative uveitis is set up together with a detachment
of the retina, so that the eye is usually excised under the diagnosis of tubercle
or new-growth (Hess, 1913; v. Schmidt zu Wellenberg, 1917; Behr, 1920 ;
Zeeman, 1926; Archangelsky and Braunstein, 1931 ; Ennema, 1934)
(Fig. 2904). There is usually an abundance of eosinophil cells and much
perivasculitis over the whole area with dense lymphoid infiltration and
much exudate. In the vitreous the reaction is considerably less : in one
case the parasite was removed but the eye remained blind (Purtscher,
1925), in a second the larva became inactive (De Boe, 1933), and in a third
it shrivelled up without doing much damage to the Ocular tissues (Anderson,
1934).
In all these cases, if the larva is seen, the diagnosis is relatively easy ;
INTRA-OCULAR PARASITES 3449
if it is not, it is impossible. If the case is seen early and the parasite is in
a position where it can be easily removed, the prognosis may be good :
otherwise it is well-nigh hopeless.
Fig. 2904.—OPHTHALMoMYLAsis PostERIok.
Due to a sub-retinal larva, showing changes in the anterior as well as the posterior
segment. A large cyst in the anterior chamber due to separation of the epithelial
layers of the iris. The retina completely detached from the disc to the ora. In a
boy of 3 (Ennema, A. of O.).
It will be remembered that in cases of destructive myiasis when maggots are
deposited in large numbers in the conjunctival sac of an emaciated and neglected
patient, they may eat into the tissues and attack the eye. Indeed, within 24 hours
the eye may be destroyed and the whole orbit converted into a crawling pit from
the depths of which the caudal ends of the maggots protrude; the bone may even
be eaten away and nasal and cranial cavities attacked, an event which, of course,
rapidly leads to coma and death. In one such case Reis (1914) found 240 maggots
in one eye, while other cases of this hideous nature have been described by Wahba
(1915), Goldschmidt (1919), Wright (1927) and Pitchoolevski (1933). The treatment
of such cases should be profuse washing with chloroform water or 5% carbolic solution
to narcotize and kill the parasites, combined with potassium permanganate to combat
the disgusting smell, together with all possible stimulating and restorative measures.
Anderson. T. Am. Acad. Oph. Otol., 218, De Boe, A. of O., x, 824, 1933.
1934. Dérer. K. M. Aug., xcviii. 339, 1937.
Am. J. O., xviii. 699, 1935. Ennema. A. of 0., xii, 180, 1934.
Archangelsky and Braunstein. K. M. Aug., Ewetzky and v. Kennel. Z. f. Aug., xii, 337,
lxxxvii. 340, 1931. 1904.
Balod. K. M. Aug., xciii. 657, 1934. Goldschmidt. Wien. kl. W., xxxii. 1159,
Barczinski. Z. f. Aug., lxviii, 353, 1929. 1919.
Behr. K. M. Aug., lxiv, 161, 1920. Hartmann. K. M. Aug., lxxviii, 233, 1927.
Bietti. An, dº Ott... li. 207, 1923. Hess. A. f. Aug., lxxiv, 227, 1913.
Borsello. Ras, It. d’Ott., v. 604, 1936. Krautner. Z. f. Aug., iv, 209, 1900.

3450
TEXT-BOOK OF OPHTHALMOLOGY
Eesti. Arst., ii, 339, 1923.
A. f. O., czzvi, 644, 1931.
An. di Ott., x1, 107, 1917; 1, 67,
Kuriks.
Linke.
Maggiore.
1922.
Pitchoolevski. Sov. vestm. O., iii, 367, 1933.
Purtscher. Z. f. Aug., lvii, 601, 1925.
Reis. Ophthalmology, x, 152, 1914.
v. Schmidt zu Wellenberg. Cb. pr. Aug., xli,
1, 1917.
Spanyol. K. M. Aug., xcvi, 494, 1936.
Ståhlberg. Hygiea, 269, 1901.
Thomas and Parsons. T. O. S., xxix, 14,
1909.
Wahba. Bull. O. S. Egypt, viii, 84, 1915.
Weisz. Ceskoslov. Oft., iii, 337, 1937.
Wright. Am. J. O., x, 411, 1927.
Zeeman. Ned. tid. v. Gen., ii, 1192, 1926.
INDEX
A
Abscess, cerebral, and papilloedema, 2947
in iris, 21.65
Accommodation and cataract, 31.59
Acne rosacea and iritis, 2154
Actinomycosis of retina, 2673
of uveal tract, 2367
Acute disseminated encephalo-myelitis
and optic neuritis, 2992
pigmentary degeneration of retina, 2774
Adaptation in glaucoma, 3384
Adeno-carcinoma of choroid, 2536
Adenoma of ciliary body, 2448
Adrenalin in glaucoma, 3396
After-cataract, 3232
Air embolism, 2108
Albuminuric choroiditis, 2387
retinitis. See Renal retinopathy.
Alcohol amblyopia, ethyl, 3019
methyl, 3021
Allergy and sympathetic ophthalmitis,
2334
and syphilitic uveitis, 2262
and tuberculous uveitis, 2289
and uveitis, 2140
Alopoecia and uveitis, 2364
Amaurosis with excavation, 3071, 3354
Amaurotic cat’s eye, inflammatory, 2229
neoplastic, 28.23
tuberculous, 2308
family idiocy, 2794
infantile form, 2797
juvenile form, 2801
late infantile form, 2805
Amblyopia, alcohol, 3019, 3021
anilin, 3035
arsenic, inorganic, 3028
organic, 3029
aspidium, 3034
carbon disulphide, 3024
ergot, 3035
ethyl alcohol, 3019
filix mas, 3034
iodine, 3025
Amblyopia, iodoform, 3025
lead, 3026
optochin, 3033
quinine, 3031
salicylate, 3034
thallium, 3028
tobacco, 3009
toxic, 3009
Amyloid degeneration of optic nerve, 3065
Anaemia and hypotony, 3422
and optic neuritis, 3005
of retina, 2555
of uvea, 2105
Anaemia, aplastic, retinal complications
of, 2737
Anaemia, pernicious, retinal complications
of, 2737
Anaemia, secondary, retinal complications
of, 2556, 2737
Aneurysmal varix of retina, 26.17
Aneurysms, arterio-venous of retina, 26.17
in arteriosclerosis, 2694
miliary, 2616
racemose, 26.17
retinal, 26.15
Angio-gliosis retinae, 2855
Angioid streaks, 2413
Angioma of choroid, 2458
of ciliary body, 2458
of iris, 2456
of retina, 2843
of uveal tract and glaucoma, 3322
Angiomatosis of retina, 2843
Angiospasm, retinal, 2566
in arteriosclerosis, 2693
Anilin amblyopia, 3035
Aniridia and glaucoma, 3322
Annular synechiae, 21.68
Aparathyroidea and cataract, 3154, 3210
Aplastic anaemia, retinal complications of
2737
Aqueous flare in irido-cyclitis, 21.98
Arachnoid cell nests in optic nerve, 3059
Arsenic amblyopia, inorganic, 3028
organic, 3029
3451
34.52
INDEX
Arsenic in uveitis, 2214
Arteriolar sclerosis in retina, 2700
Arteriosclerosis, cerebral, 2705
in optic nerve, 3065
in retina, 2677, 2696
syphilitic, 2665
See also Atherosclerosis.
Arteriosclerotic retinopathy, 2707
Arterio-venous aneurysm of retina, 26.17
crossings in retina, 2683
Ascaris lumbricoides, 3437
Aspergillosis of uvea, 2367
Aspidium amblyopia, 3034
Asteroid bodies, 3249
Astrocytoma, 2840
Atheroma in retina, 2697
Atherosclerosis, in cataract, 3160
in glaucoma, 3337
in optic nerve, 3065
in retina, 2675, 2696
in uveal tract, 2369
Atrophic irido-dialysis, 2.185
uveitis, 2132, 2283
clinical picture, 2253
Atrophy, cavernous, of optic nerve, 3071,
3353
central areolar choroidal, 2412
of choroid, 2398
central areolar, 2412
essential, 2407
glaucomatous, 3344
gyrate, 2407
post-inflammatory, 2401
of ciliary body, 2398
post-inflammatory, 2401
circum-papillary choroidal, 2397
glaucomatous, of uvea, 2402, 3343
of optic nerve, 3351 *
gyrate, 2407
of iris, 2398
glaucomatous, 2402, 3343
ischaemic, 2402
neurogenic, 2403
post-inflammatory, 2398
primary, 2404
progressive, 2404
syphilitic, 2280
traumatic, 2402
ischaemic, in optic nerve, 3069
in retina, 2555
in uvea, 2107, 2402
Leber’s optic, 2998
Atrophy, neurogenic of uvea, 2403
of optic nerve, 3067
arteriosclerotic, 3065
cavernous, 3071, 3353
circulatory, 3069 g
consecutive, 3068
glaucomatous, 3351
Leber’s, 2998
post-inflammatory, 3068
post-papilloedema, 2954
pressure, 3069
primary, 3045
syphilitic, 3045
toxic, 3069
traumatic, 3069
post-inflammatory, in optic nerve, 2068,
2633
in retina, 2628
in uvea, 2398
primary, of iris, 2402
progressive of iris, 2402
traumatic, of optic nerve, 3069
of uvea, 2402
of retina, 2745
Of uvea, 2398
Atropine. See Mydriatics.
Attenuated tubercle of Leber, 2296
Auto-haemotherapy, 2216
Auto-intoxication and uveitis, 2156
Avitaminosis and cataract, 3.162
and optic neuritis, 3007
B
Banking of retinal veins, 2688
Darlow's disease, retinal complications of,
2740
Basal meningioma, 3094
Batten-Mayou disease, 2801
Behr’s disease, 2789
Benign epithelioma of ciliary body, 2448
Best’s disease, 2789
Bjerrum's sign, 3381
Bladder cells, of pigmentary epithelium,
263]
of Elschnig, 3236
Blastomycosis of uvea, 2367
Blood chemistry in cataract, 3126
in glaucoma, 3334
diseases and retinal lesions, 2737
and uveal lesions, 2376
INDEX
3453
Blood pigment in iris, 24.18
in vitreous, 3257
vessels, diseases of. See Arteriosclerosis,
Atherosclerosis, etc.
Blue-dot cataract, 3184
Boeck's sarcoid and uveitis, 23.58
Bourneville's disease in retina, 2857
Brain abscess and papilloedema, 2947
tumours and papilloedema, 2947
Buerger's disease and intra-ocular
haemorrhages, 2602
retinal complications of, 2575
Buphthalmos and angiomata, 2460
and glaucoma, 3320
(!
Cachexia and cataract, 3224
Capillaritis of macula, 2594
Capillary system and glaucoma, 3335
Capsular exfoliation, 3104
senile, 3 106
and glaucoma, 3310
("apsule of lens, permeability of, 3136
Carbon disulphide amblyopia, 3024
('arcinoma of optic nerve, 3098
orbital, and intra-ocular extension. 2521
metastatic of retina, 2863
secondary in uveal tract, 2522
('ataract, 3 l l 5
and acidification, 3140
aetiological theories, 31.57
aetiology of, 31 17
after-, 3232
in aparathyroidea, 3154, 3210
and asphyxia, 31.45
black, 3 198
blood chemistry in, 3 l 26
blue-dot, 3184
brunescens, 3198
cachectic, 3224
Calcarea, 31.67
and calcium, 3121
choroidal, 3230
cold, 3140
complicated, 3224
complications of, 3176
coronary, 3186
cortical, 3199
in c. retinism, 32 19
Cataract cuneiform, 3200
cupuliform, 3203
and deficiencies, 31.45
dermatogenous, 3220
diabetic, 3204
dilacerated, 3.185
dinitrophenol, 3221
dislocation of lens in, 3177
dust-like, 3202
endocrine, 3209
ergot, 3223
experimental, 31.38
extraction and expulsive haemorrhage,
2 I I 3
false, 31.97
in familial hypertrophic
dystrophy, 32.17
galactose, 3152
and glaucoma, 3176, 3309
glaucoma in, 3176
har(l, 319.5
heat, 3140
heterochromic, 3229
history of, 3115
hypermature, 31 68, 317.5
immature, 31.75
and injury, 3139
lactose, 31 52
lamellar, 31.68
in Mongolian idiocy, 32.17
Morgagnian, 3.17 1, 31.75
in myotonic dystrophy, 2313
naphthalene, 3.146
nigra, 3198
nuclear, 3195
Ossea, 31.68
Osmotic, 3139
paradichlorobenzene, 3223
pathology of, 3105
peri-nuclear, 3202
posterior saucer-shaped, 3203
punctiform, 3184
pyramidal, 3114
radiational, 3141
secondary, 3232
senile, 31.93
snow-flake, 3205
soft, 31.99
sub-capsular, 3112
symptoms of, 3172
in tetany, 3154, 3210
thallium, 3153
muscular
3.454
INDEX
Cataract, toxic, 31.46, 3221
treatment Of, 3177
Cavernous optic atrophy, 307 1, 3353
Cellular mantle, 2834
Central angiospastic retinitis, 2593
retinopathy, 2578
choroiditis, 2249
guttate choroiditis, 2749
pigmentary degeneration of retina, 2773
punctate retinitis, 2729, 2733
serous retinitis, syphilitic, 2664
retinopathy, 2592
Cerebral arteriosclerosis and retinal
disease, 2705
tumours and papilloedema, 2947
Cerebro-retinal degenerations, 2792
Cerebro-spinal meningitis and
2 I 5 ||
Chiasmal neuritis, 2979
Chicken-pox and uveal disease, 2150
Chloroma in uvea, 2379
Chlorosis, retinal complications of 2737
Cholera and Cataract, 3209
and uveal disease, 2 150
Cholesterol deposits in uvea, 2422
in vitreous, 3252
Choked disc, 2944. See Papilloedema.
Choline derivations in glaucoma, 33.95
Chorio-retinitis, 2656
See also Choroiditis and Retinitis.
adhesive, 2 192
centralis serosa, 2367
proliferating traumatic. 210 l
symptoms of, 2205
syphilitic, 2266
treatment of, 2206
Chorion-epithelioma of choroid, 2535
Choroid, anaemia of, 2105
adeno-carcinoma of 2536
angioma of, 2458
atrophy of, central areolar, 2412
essential, 2407
glaucomatous, 3344
gyrate, 2407
post-inflammatory, 2401
secondary, 2401
carcinoma, Secondary, of, 2522
chorion-epithelioma of, 2535
circulatory disturbances in, 2101
cirrhosis of, 2388
dermoid of, 2520
detachment of, 2538
liveit is,
Choroid, detachment of, post-operative,
254 |
embolism in, 2107
endothelioma, secondary of, 2522
fatty degeneration of, 2422
fibroma of, 21.94
gliosis in, 2453
haemorrhages in, 2109
hyperaemia of, 2105
infarct of, 2107
inflammations of, 2126
See also ('horoiditis.
ischaemia of 2102
melanoma, secondary, of, 2522
benign, 2473
malignant, in . 2481
secondary. 2534
myeloma of 2461
naevus of, 2473
neuro-blastoma. Secondary. Of, 2522
Ossification in, 2423
in Raynaud’s disease, 2105
sarcoma. See Melanoma. malignant.
metastatic. 2534
secondary, 2522
sclerosis of, 2409
and glaucoma, 3316
senile changes in, 2936
thrombosis of , 2 108
tuberculoma of 2306
vascular sclerosis of , 2368
wounds of, 21 ()()
Choroidal cataract, 3230
('horoidit is, acute diffuse serous, 2366
aetiology of, 2126
albuminuric, 2387
allergic, 2.140
anterior, 224 l
areolar, 224 l
central, 2241, 2249
areolar, 2241
guttate, 2749
circumpapillary, 2248
circumscribed exudative, 2241
purulent, 2229
deep, 2242
diffuse, 2238
purulent, 2229
disseminated, 2239
metastatic. 2230
exudative, 2237
Förster’s, 224 l
INDEX
3455
Choroiditis, haemorrhages in, 2111
honeycomb of Doyne, 2786
hyperplastica, 2.192
Jensen’s, 2248
juxta-papillary, 2248
leprosa praecox, 2322
leprotic, 2321
macular, 2249
metastatic, 2.136, 2229
nodular, 2191
pathology of, 2158
purulent, 2228
senile macular exudative, 2115
septic, 2139
superficial, 2242
suppurative, 2228
sympathetic, 2324
symptomatology, 2203
syphilitic, 2266
Tay’s, 2749
treatment of, 2206
in trypanosomiasis, 2323
tuberculous, 2203
Chromatophoroma, 2478
Ciliary arteries, effects of section of, 2102
body, adenoma of, 2448
angioma of, 2458
atrophy of, 2401
carcinoma, secondary of, 2527
diktyoma of, 2449
displacement of, 2538
endothelioma of, 2461
epithelial hyperplasia of, 2447
malignant, 2450
epithelioma of, 2449
benign, 2448
malignant, 2450
fatty degeneration in, 2421
hypernephroma of, 2533
melanoma, malignant of, 24.86
naevus of, 2473
neuroblastoma of, 2453
sarcoma. See Melanoma, malignant.
secondary, 2522
senile changes in, 2393
tuberculoma of, 2304
wounds of, 2099
epithelium, in retinal detachment, 2875
staphyloma in glaucoma, 3351
Circinate degeneration, 2760
Circulatory disturbances in optic nerve,
2938
T.O.-WOL. III.
Circulatory disturbances in retina, 2551
in uveal tract, 2101
Circum-papillary choroidal
senile, 2397
glaucomatous halo, 3346
Cirrhosis of choroid, 2388
Cleft-cysts, 2441
Coats’ disease, 2610, 2648
Colloid bodies on Bruch's membrane,
2747
on lens capsule, 3111
degeneration, familial, 2786
of optic nerve, 3065
Contusion, ocular, and haemorrhage, 21.13
and retinal detachment, 2870
and sympathetic ophthalmitis, 2329
Copper wire arteries, 2678
Cornea, complications in uveitis, 2164
ulcers of, and sympathetic ophthal-
mitis, 2328
atrophy,
Coronary cataract, 3186
Corpora amylacea, 3060
arenacea, 3061
Cotton-wool patches in retina, 2714
Cretinism and cataract, 32.19
Cuneiform cataract, 3200
Cupping of disc, atrophic, 3071, 3353
arteriosclerotic, 3069
glaucomatous, 3351
Cupuliform cataract, 3203
Cyanosis of retina, 2552
Cyclitic membrane, 2170
pathology of, 2170
Cyclitis, aetiology of, 2126
allergic, 2.140
heterochromic, 2360
metastatic, 2.136
pathology of, 2158
plastic, 2.187
serous, 2187
symptomatology of, 2200
treatment of, 2206
tuberculous, 2298
Cyclodialysis, 3405
Cystic degeneration of retina, 2752
Cysticercus cellulosae in eye, 3438
Cystoid degeneration of retina, 2752
Cysts, hydatio, intra-ocular, 3441
of iris, 2431 -
of retina, 2806
in vitreous, 3278
Cytoid bodies, 2954, 3087
3456
INDEX
D
Dalén-Fuchs nodules, 2345
Dalén's spots, 2337
Degeneratio punctata albescens, 2784
sine pigmento, 2774
Degenerations, amyloid, of optic nerve,
3065
calcareous, choroid, 2426
in uvea, 2423 -
circinate, 2760
colloid in optic nerve, 3065
in retina, 2747
familial, 2786
familial colloid in retina, 2786
lipoid, 2792
macular, 2788
fatty, in choroid, 2422
in ciliary body, 2421
in iris, 2421
in uveal tract, 2421
glaucomatous, 3341
heredo-macular, 2788
hyaline of optic nerve, 3062
of pupillary margin, 2390
of uvea, 2423
lipoid, 2792
macular, arteriosclerotic, 2372
cystic, 2755
familial, 2788
senile, 2372
maculo-cerebral, 2801
of optic nerve, 3059
amyloid, 3065
vascular, 3065
See also Atrophy.
of retina, colloid, 2747
cystic, 2752
cystoid, 2752
macular, 2755
peripheral, 2754
familial colloid, 2786
pigmentary, 2765
acute, 2774
primary, 2765
secondary, 2745
primary, 2765
senile, 2745
senile, in choroid, 2396
in ciliary body, 2393
in iris, 2389
in macula, 2372
Degenerations, senile,
epithelium, 2746
in retina, 2745
in vitreous, 3258
tapeto-retinal, 2765
of uveal tract, 2389
of vitreous, 3246
myopic, 3258
post-inflammatory, 3262
senile, 3258
Degenerative pannus, 3342
Dermato-myositis of retina, 2674
Dermoid of choroid, 2520
Detachment of choroid, 2538
delayed, 2542
operative, 2541
post-inflammatory, 2538
post-operative, 2541
serous, 2538
of iris, 2537
of macula, idiopathic, 2593
inflammatory, 2657
of pigmentary layer of iris, 2538
of retina, 2864
in choroiditis, 2172
cysts in, 28.10
delayed, 2871
experimental, 2888
exudative, 2896
post-inflammatory, 2538, 2872
post-operative, 2872
remote, 2483
in renal retinopathy, 2717
in retinopathy of pregnancy, 2725
serous, 2897
and tension of eye, 2886, 3315
in tumours, 2483, 2513
traumatic, 2870
in uveitis, 2172
of vitreous, 3266 &
Devic's disease and optic neuritis, 2993
Diabetes insipidus and irido-cyclitis, 2386
* mellitus and cataract, 3204
and hypotomy, 3421
* and iris, 2383
* and iritis, 2156
and lipaemia, 2742
and optic neuritis, 3005
* and retinopathy, 2729
and uveal changes, 3283
Diffuse hyperplastic arteriosclerosis of
retina, 2699, 2700
in pigmentary
INDEX
3457
Diktyoma, 2449
Dilacerated cataract, 3.185
Dinitrobenzol amblyopia, 3035
cataract, 3221
Disciform degeneration of macula, 2115
Disseminated choroiditis, 2239
suppurative, 2230
syphilitic, 2267
tubercular, 2303
sclerosis and optic neuritis, 2990
Dropsy, epidemic, and glaucoma, 3317
Drusen, 2747, 3062
Dysacousia and uveitis, 2364
Dysentery and uveal disease, 2151
Dystrophia epithelialis lentis adiposa,
3113
E
Eales’ disease, 2602
Echinococcus, intra-ocular, 3445
Eclampsia, retinal detachment in, 2725,
2873
retinopathy in, 2723
Ectropion of pigment layer of iris after
iritis, 2185
atrophic, 2400
glaucomatous, 2403, 3344
Eczema and cataract, 3221
Elschnig's pearls, 3236
spots, 2387
Embolism, air, in uvea, 2108
in retina, 2568
in uveal tract, 2105
Encephalitis peri-axialis diffusa and optic
neuritis, 2997
Endarteritis, retinal, 2572
IEndocrine cataract, 3209
glands and glaucoma, 3338
Endo-phlebitis, retinal, 2578
Endophthalmitis, exudative, 2251
phaco-anaphylactica, 2133
Endotheliogenous connective tissue, 2201
Endothelioma of optic nerve, 3089
spread to choroid, 2522
of uveal tract, 2461
Epidemic dropsy and glaucoma, 3317
Epithelial hyperplasia of ciliary body,
2447
Epithelioma, benign, of ciliary body, 2448
of ciliary body, 2449
Epithelioma, of intra-ocular
extension, 2520
malignant, of ciliary body, 2450
secondary in uvea, 2520
Ergot amblyopia, 3035
cataract, 3223
in glaucoma, 3398
Erythema nodosum, and irido-cyclitis,
2227
Erythraemia,
2738
uveal complications of, 2380
Eserine in glaucoma, 3395
Essential hyperiesia and retina, 2698
lipoid histiocytosis, 2800
Exanthemata and uveitis, 2149
Exsanguination and retinal complica-
tions, 2556
Exudative choroiditis, 2373
irido-cyclitis, 2231
iritis, 2231
retinitis, 2648
tuberculous, 2668
limbus,
retinal complications of,
F
Familial colloid degeneration of retina,
2786
hypertrophic muscular dystrophy and
cataract, 32.17
lipoid degeneration, 2792
macular degeneration, 2788
Fatty degeneration in choroid, 2422
in ciliary body, 2421
in iris, 2421
in uveal tract, 2421
Febrile diseases and glaucoma, 2422, 3336
and retinitis, 2673
and uveitis, 2149
Fever therapy in uveitis, 2216
Fibrillary degeneration of vitreous, 2876
Eibris uvea-parotidea sub-chronica, 2355
“Fibroma ‘’ of choroid, 2.194
Fibroma of optic nerve, 3093
Field of vision in alcohol amblyopia, 3021
in arsenic amblyopia, 3030
in carbon disulphide poisoning, 3025
in chiasmal neuritis, 2980
in choroiditis, deep, 2246
superficial, 2241
syphilitic, 2269
4 S 2
3458
INDEX
Field of vision in disseminated sclerosis,
2992
in encephalitis peri-axialis, 2997
in glaucoma, 3380
in Leber's disease, 3001
in Optic atrophy, arteriosclerotic,
3067 -
primary, 3047
neuritis, 2979, 3005, 3007
perineuritis, 2970
in papilloedema, 2963
in pigmentary degeneration of retina,
2772 -
in quinine amblyopia, 3032
in retinal detachment, 2703
embolism, 2565
thrombosis, 2585
in retro-bulbar neuritis, 2979
in sinus disease, 2987
in tobacco amblyopia, 3012
Filaria, 3430
. in anterior chamber, 3432
in lens, 3434
in posterior segment, 3434
Filix mas amblyopia, 3034
Filtering scar, 3405
Flat sarcoma of uvea, 2490.
Floccules of Busacca, pathology, 2181
clinical appearance, 2198
of iris, tuberculous, 2299
Focal sepsis and cataract, 3.162
and glaucoma, 3362
and optic neuritis, 3004
and retinitis, 2637
and uveitis, 2140
Foreign bodies, intra-ocular, and glau-
coma, 3306
Förster’s areolar choroiditis, 2241
syphilitic uveitis, 2268
Fungus infections of uvea, 2367
G
Galactose cataract, 3152
Gas gangrene panophthalmitis, 2224
Genito-urinary tract and uveal disease, . .
2146
Ghost cells, of Coats, 2631
Glaucoma, 3280
absolute, 3367
acute, 3365
Glaucoma, adaptation in, 3384
in angiomata, 3322
in aniridia, 3322
in buphthalmos, 3320
capsular, 3310
and capsular exfoliation, 3110
and cataract, 3176, 3231
in cataract, intumescent, 3309
Morgagnian, 3309
compensated, 3364
congestive, 3365
degenerative changes in, 3367
in dislocation of lens, 3308
in epidemic dropsy, 3317
experimental, 3326
fatty degeneration in, 2422, 3336
fields of vision in, 3380
in haemorrhages, intra-ocular, 3315
haemorrhagic, 2584, 3311
incompensated, 3365
inflammatory, 3287
inverse, 3243, 3307
light sense in, 3384
in luxation of lens, 3243, 3308
malignant, 3312
in microphakia, 3307
miotics in, 3392
mydriatic, 3334
in neurofibromatosis, 3324
in papillitis, 3313
pigmentary disturbances in, 2419
post-inflammatory, 3292
post-operative, 3301
post-traumatic, 3301
primary, 3327
aetiology, 3355
clinical picture, 3364
diagnosis, 3387
incidence, 3328
pathology, 3341
symptoms, 3368
treatment, 3391
in retinal detachment, 2886, 3315
secondary, 3285
simple, 3364.
in sub-luxation of lens, 3242, 3307
tension in, 3375
tests for, 3389
traumatic, 3296
in tumours, intra-ocular, 2473, 2511,
2820, 3313
in venous thrombosis, 2584, 3311
INDEX
3459
Glioma, 2840
endophytum, 2816
exophytum, 2816
of optic nerve, 3080
planum, 2816
of retina, 2840
See also Retinoblastoma,
epithelioma, etc.
Gliosis in choroid, 2453
of retina, 2626
Glomerulo-nephritis, and retinal disease,
2710
Gold treatment in uveitis, 2214
Gonorrhoeal irido-cyclitis, 2257
retinitis, 2673
Gout and uveal disease, 2155
and uveitis, 2381
Grönblad-Strandberg syndrome, 2417
Grooves of retina, 2864
Gumma of choroid, 2272
of ciliary body, 2272
of iris, 2272
of optic nerve, 3044
of retina, 2664
Guist’s sign, 2692
Gunn’s sign, 2684
Gyrate atrophy of choroid, 2407
Neuro-
H
Haab's macular degeneration, 2372
FIaemangioblastoma retinae, 2855
Haemangioma of iris, 2456
of choroid, 2458
of ciliary body, 2458
IIaemochromatosis, retinal complications
of, 2744
Baemophilia and iridic haemorrhage, 2109
retinal complications of, 2740
Haemorrhages in choroid, 2109
after contusion, 21.13
expulsive, 21.13
inter-retinal, 2595
intra-ocular and glaucoma, 3315
and uveitis, 2252
vitreous changes in, 3265
in iris, 2109
and iritis, 2132
in optic nerve sheaths, 2938
sub-arachnoid, 2939
sub-dural, 2938
Haemorrhages, post-operative, 21.13
pre-retinal, 2599
retinal, 2595
in arteriosclerosis, 2694
in influenza, 2673
in malaria, 2673
in recurrent fever, 2673
in spirochaetosis ictero-haemorrhagica,
2673
in sub-arachnoid haemorrhage, 2942
in typhoid fever, 2673
in typhus fever, 2673
in Weil's disease, 2673
sub-arachnoid, 2939
sub-choroidal, 2112
sub-dural, 2938
sub-hyaloid, 2599
in uveal tract, 2108
vitreous, 2601
recurrent, 2602
Haemorrhagic retinitis, external, 2610
Pſalo, peri-papillary, senile, 2397
glaucomatous, 3346
Halos, entoptic, in glaucoma, 3379
|Harada’s disease, 2366
Heerfordt's disease, 2355
Heredo-macular degeneration, 2788
Herpes iridis, 2255
zoster and optic neuritis, 2997
Herpetic iritis, 2254
Heterochromia, neurogenic, 2362
FHeterochromic cataract, 3229
cyclitis, 2360
v. Hippel’s disease, 2843
Histamine in glaucoma, 3391
Holes, hyaloid, 3270
at macula, 2755
of retina in detachment, 2878
Honeycomb choroiditis of Doyne, 2786
macula, 2756
Hutchinson’s changes, 2760
Hyaline bodies in optic nerve, 3062
degeneration of iris, 2390
of optic nerve, 3062
of pupillary margin, 2390
of uvea, 2423
Hyaloid holes, 3270
Hydatid cyst, intra-ocular, 3445
Hyperaemia of choroid, 2105
of iris, 2104
inflammatory, 2197
of optic disc, 2938
3460
INDEX
Hyperaemia of retina, 2552
Hypernephroma of uvea, 2533
Hyperplastic choroiditis, 2.192
Hypertension and glaucoma, 3336
retinopathy in, 2726
Hypertensive sclerosis in retina, 2698
Hyphaema, 2109
and iritis, 2197
Hypoderma bovis, intra-ocular, 3447
Hypopyon, 2160, 2198
in leukaemia, 2377
recidivans, 2227
recurrent, 2227
Hypotony, 34.17
anaemic, 3442
congenital, 3418
diabetic, 3421
endocrine, 3423
inflammatory, 3421
in myopia, 3418
neurogenic, 3422
pathology of, 3424
in retinal detachment, 3418
symptoms of, 3427
traumatic, 3419
treatment of, 3428
in tumours, intra-ocular, 2512
I
Implantation cysts of iris, 2432
Infarct of choroid, 2107
Infective disease and optic neuritis, 3004
and retinitis, 2673
and uveitis, 2149
Influenza and retinitis, 2673
and uveitis, 2151
Inoculation chancre, 2332
Intestinal tract and uveal disease, 2146
Involutionary *arteriosclerosis in retina,
2702
Iodine amblyopia, 3025
Iodoform amblyopia, 3025
Iridectomy and expulsive haemorrhage,
2113
in glaucoma, 3401
in irido-cyclitis, 2217
Iridencleisis, late infections after, 2129
Irido-cyclitis. See Iritis and Cyclitis.
acute, 2234
and cataract, 3228
Irido-cyclitis. causing retinitis, 2656
chronic, 2236
and glaucoma, 3287
gonorrhoeal, 2257
herpetic, 2254
leprotic, 2320
pseudo-tuberculosa, 2130, 2368
recidivans purulenta, 2227
recurrent with hypopyon, 2227
septica, 2227
suppurative, 2225
syphilitic, 2263
recurrent, 2264
in trypanosomiasis, 2323
tuberculous, 2298
Irido-dialysis, atrophic, 2.185
Iridodonesis, 3242
Irido-sclerosis, 2371.
Iris, abscess of, 21.65
angioma of, 2456
atrophy of, essential, 2404
glaucomatous, 2402, 3343
ischaemic, 2402
neurogenic, 2403
post-inflammatory, 2398
primary, 2404
progressive, 2404
senile, 2389
syphilitic, 2280
traumatic, 2402
carcinoma, secondary of, 2527
cysts of, 2432
degenerations of, 2389
detachments of, 2537
displacements of, 2537
essential atrophy of, 2404
and glaucoma, 3316
fatty degeneration in, 2421
gumma of, 2272
haemorrhages in, 2109
hyperaemia of, 2104
hypernephroma of, 2533
inflammations of, 2126
See also Iritis.
leprosy of, 2320
melanoma, malignant of, 2488
secondary, 2534
myoma, 24.54
naevus of, 2471
necrosis in, 2429
neurogenic atrophy of, 2403
papule of, 2270
INDEX 3461
Iris, in polycythaemia, 2105
roseola of, 2262
rubeosis of, 2384
sarcoma. See Melanoma malignant.
metastatic, 2534
secondary, 2522
senile changes in, 2389
syphilomata of, 2270
tuberculoma of, 2304
tuberculosis of, 2284
tumours of, 2446
wounds of, 2098
Iris-inclusion operations, 3403
Iritis, acute, 2232
in acne rosacea, 2154
aetiology of, 2126
allergic, 2.140
diabetic, 2156
gonorrhoeal, 2258
gouty, 2155, 2380
haemorrhagic, 2197
herpetic, 2254
hyphaema in, 2197
leprotic, 2320
metastatic, 2.136
nodular, 2182
obturans of Schieck, 2304
partial, 2131
pathology of, 2158
post-operative, 2129
recurrent, 2233
rheumatic, 2155
in rosacea, 2154
serous, 2236
simple, 2232
in skin disease, 2153
sub-acute, 2233
superficial, 22.45
symptomatology of, 2197
syphilitic, 2263
in trypanosomiasis, 2323
treatment of, 2206
tuberculous, 2284
urica, 2155
and uveal pigment, 2134
Ischaemia, of retina, 2556
of uvea, 2102
J
Jactatio corporis vitrei, 3261
Jarisch-Herxheimer reaction, 2264 °
Jensen’s disease, 2248
Juvenile disciform degeneration of
macula, 2124
exudative macular choroiditis, 2124
FC
Reratic precipitates, pathology, 2179
Reratitis and cataract, 3229
Ridney disease and retinopathy, 2710
and uveal tract, 2386
Roeppe's nodules, clinical appearances,
21.98
pathology, 2180
in tuberculosis irido-cyclitis, 2298
L
Lactation and optic neuritis, 3006
Lactose cataract, 3152
Lamellar separation, 3.192
Late infections, 2129
Laurence-Moon-Biedl syndrome, 2768
Lead amblyopia, 3026
poisoning and uveal arteriosclerosis,
2371 *
Leber's optic atrophy, 2998
neuritis, 2998
Leiomyoma of iris, 24.54
Lens, chemical pathology of, 3118
clefts in, 3.191
diseases of, 3102
dislocation of, 3243
and glaucoma, 3308
displacements of, 3238
double focus, 3197
fixata, 324I
metabolism of, 3128
natans, 3241
senile changes in, 3189
sub-luxation of, 3241
and glaucoma, 3307
tremulousness of, 3244
uveitis, complications in, 2164, 2177
vacuoles in, 3190
wounds of, 3102
xanthomatosis of, 31.68
Lenticonus, false, 3197
Lenticular opacities, evolutionary, 3184
Lepromata in iris, 2321
3462
INDEX
Leprosy of retina, 2673
in uveal tract, 2320
Leukaemia, retinal complications of, 2740
uveal complications of, 2376
Leukiridia, 2419
Light sense in glaucoma, 3384
Lindau's disease, 2843
Lipa-mia, 2742
retinalis, 2743
Lipiodosis, vitreous in, 3256
Lipoid degeneration, 2792
Lupoid, benign miliary, 2358
Lymphoma of uveal tract, 2461
Lympho-sarcoma of uveal tract, 2461
* M
Mackenzie, William, 2097
Macropsia in choroiditis, 2204
Macula, cherry spot at, in amaurotic
idiocy, 2797
in ischaemia, 2561
choroiditis at, 2249
exudative, 2115
degeneration of, cystic, 2755
disciform, 2115
hereditary, 2788
lipoid, 2792
senile, 2372
detachment of, idiopathic, 2593
inflammatory, 2657
See Retina, detachment of.
haemorrhages at, 2110
holes at, 2755
in detached retina, 2881
honeycomb, 2756
in irido-cyclitis, complications at, 2201,
2657
juvenile exudative choroiditis at, 2124
oºdema of, 2592
in irido-cyclitis, 2201, 2657
secondary, 2657
vesicular, 2756
senile degeneration of, 2372
exudative choroiditis at, 2115
Maculo-cerebral degeneration, 2801
Malaria and retinal haemorrhages, 2673
uveal complications of, 2151
Malignant hypertension and retina, 2699
retinopathy in, 2727
melanoma. See Melanoma, malignant.
Measles and retinitis, 2673
and uveal inflammations, 2149
Medullo-epithelioma, 2839
Medusa head in glaucoma, 3368
Melanoma, cellular nature of, 2461
malignant, of choroid, circumscribed,
2481
diffuse, 2492
See also of uvea.
of ciliary body, circumscribed, 2486
diffuse, 2494
See also of uvea.
of iris, circumscribed, 2488
diffuse, 2491
See also of uvea.
of optic nerve, 3095
metastatic, 2535, 3101
of retina, secondary, 2863
of uvea, 2477
circumscribed, 2481
clinical course, 2480
degeneration of, 2508
diagnosis of, 2513
diffuse, 2490
and glaucoma, 2511
haemorrhages in, 2507
heredity of, 2479
histology of, 2503
incidence of, 2478
infiltrating, 2490
metastases of, 2498
necrosis of, 2508
prognosis of, 2514
spread of, 2495
and sympathetic Ophthalmitis,
2329
symptoms of, 2512
treatment of, 2516
simple, of choroid, 2473
of ciliary body, 2473
of iris, 2471
of optic nerve, 3095
of pigmentary épithelium, 2446
See also Naevus.
Melanoses, 2477
Meningiomata of optic nerve, 3089
Metamorphopsia in choroiditis, 2204
Metastatic retinitis, 2637
tumours of retina, 2863
v. Michel’s flecks, 2298
Microphakia and glaucoma, 3307
Micropsia in choroiditis, 2204
INDEX
3463
Miliary aneurysms of retina, 2616
tubercle of choroid, 2294
of iris, 2293
of optic nerve, 3037
of retina, 2667
Miotics in glaucoma, 3392
Mongolian idiocy and cataract, 3217
Monilia in uvea, 2367
Morbilli and retinitis, 2673
and uveal disease, 2149
Morgagnian cataract, 3171
and glaucoma, 3309
globules, 3167
Mumps and uveitis, 2152
Mycoses of uveal tract, 2367
Mydriatics in iritis, 2208
in glaucoma, 3333
Myeloma of choroid, 2461
Myoma of iris, 24.54
Myopia, choroidal haemorrhages in, 2111
and hypotension, 3418
vitreous changes in, 3260
Myotonia congenita and cataract, 32.17
Myotonic dystrophy and cataract, 32.13
N
Naevus of choroid, 2473
of ciliary body, 2473
flammeus and angioma of choroid, 2460
of iris, 2471 -
of optic nerve, 3095
nature of, 2461
Naphthalene cataract, 3.146
Nasal sinuses and optic neuritis, 2985
and uveal disease, 2145
Necrosis in uveal tract, 2429
Nemathelminthes, 3430
Neoplasms. See Tumours.
Nephritis. See also Renal disease.
and retinopathies, 2710
trench and retinopathies, 2713
and uveitis, 2157
Nephro-sclerosis and retinal disease, 2710
Nephroses and retinal disease, 2710
Nerve, optic. See Optic nerve.
Neurinoma, ciliary, 2469
of optic nerve, 3095
Neuritis of optic nerve, 2971
See Optic neuritis.
• Neuritis papulosa, 2664
retinae, 2246
retro-bulbar, 2978
Neuroblastoma, 2831
of ciliary body, 2453
of iris, 2453
Neurocytoma, 2840
Neurodermatitis and cataract, 3220
Neuro-epithelioma, 2836
Neuro-fibrilitis, 2246
Neurofibroma of uveal tract, 2466
discrete, 2469
Neuro-fibromatosis of optic nerve, 3087
of retina, 2860
of uvea, 2466
and glaucoma, 3324
Neuroma, ciliary, 2471
Neuro-myelitis optica and optic neuritis,
2993
Neuro-retinitis, diffuse, 2661
pseudo-albuminurica stellata, 2591
syphilitic, 2660
New vessels in retina, 2618
in vitreous, 2618
Niemann-Pick's disease, 2800
Nitrobenzol amblyopia, 3035
Nodular choroiditis, 2191
iritis, 2182
Nodules of iris, tuberculous, 2298
of Koeppe, pathology of, 2180
clinical appearance of, 2198
O
Occlusio pupillae, pathology of, 2169
CEdema of macula, 2592
pre-retinal, 2593
of retina, 2588
retinae externum centrale, 2761
Onchocerciasis, 3436
Opacities in cataract, 3115
in lens, 3184
epithelial, 3112
sub-capsular, 3112
in vitreous, 3248
Ophthalmia hepatica, 2388
lenta, 2139, 2227
migratoria, 2333
nodosa, 2130, 2368
Ophthalmitis, sympathetic, 2324
3464
INDEX
Ophthalmomalacia, 34.17
essential, 3423
Ophthalmomyiasis, anterior, 3448
interna, 3447
posterior, 3448
Optic atrophy, 3067
arteriosclerotic, 3065
cavernous, 3071
circulatory, 3069
consecutive, 3068
glaucomatous, 3351
Leber’s, 2998
post-inflammatory, 3068
pressure, 3069
primary, 3045
in sarcoid, 2359
syphilitic, 3045
toxic, 3069
traumatic, 3069
nerve, arteriosclerosis of, 3065
atrophy of, 3067
circulatory disturbances in, 2938
degenerations of, 3059
diseases of, 2927
drusen in, 3062
gummata of, 3044
hyaline bodies in, 3062
inflammation of, 2967
neuritis of, 2971
perineuritis of, 2967
sheaths, haemorrhages in, 2938
syphilis of, 3041
tuberculosis of, 3036
tumours of, 3073
uveitis, complications in, 2164, 2189,
220.1
vascular disease of, 3065
wounds of, 2929
neuritis, 2971
in acute disseminated encephalo-
myelitis, 2992
aetiology, 2983
in calves, 3008
chiasmal, 2979
in disseminated Sclerosis, 2990
in encephalitis peri-axialis diffusa,
2997
and herpes zoster, 2987
interstitial, 2973
and lead, 3026
Leber's, 2998
and nasal sinuses, 2985
Optic neuritis in neuro-myelitis optica,
2993 º
with polyneuritis of cranial nerves,
2997
purulent, 2971
retro-bulbar, 2978
acute, 2978
axial, 2978
chronic, 2979
total transverse, 2978
syphilitic, 3041
tuberculous, 3036
perineuritis, 2967
Optochin amblyopia, 3033
Oral sepsis. See Focal sepsis.
Ossification in uveal tract, 2423
Osteitis deformans and arteriosclerosis,
2371
and angioid streaks, 2417
choroidal haemorrhages, 2417
P
Paget’s disease. See Osteitis deformans.
Panophthalmitis, 2221
due to gas gangrene, 2224
metastatic, 2637
pathology of, 21.59
Papillitis, 2976
and glaucoma, 3313
in tuberculous irido-cyclitis, 2301
Papilloedema, 2944
aetiology of, 2941
in chlorosis, 2757
choroidal haemorrhages in, 2111
clinical picture of, 2960
in malignant hypertension, 2727
pathenogenesis of, 2955
pathology of, 2951
prognosis of, 2964
in renal retinopathy, 2717
in sarcoid, 2359
in sub-arachnoid haemorrhages, 2942
symptoms of, 2962
treatment of, 2964
Papules of iris, 2270
Paracentesis in uveitis, 2216
Paradichlorobenzene cataract, 3223
Parasites, intra-ocular, 3430
Para-tuberculosis in sarcoid, 2359
in uveo-parotitis, 2357
INDEX
3465
Parotitis (epidemic) and uveitis, 2152
Partial iritis, 2131
Pearl tumours of iris, 2433
Pepper-and-salt fundus, 2273
Peri-arteritis nodosa, 2643
in uvea, 2374
of retina, 2639
syphilitic, 2663
tuberculous, 2672
Perineural fibroblastoma, 2469
Perineuritis, exudative, 2968
of optic nerve, 2967
purulent, 2970
Peri-papillary halo, glaucomatous, 3346
senile, 2397
Peripheral cystoid degeneration of retina,
2754
Peri-phlebitis of retina, 2639
secondary, 2660
syphilitic, 2663
tuberculous, 2668
Peristaltic condition, 2720
Peri-vasculitis, retinal, 2639
secondary, 2660
sympathetic, 2346
syphilitic, 2663
tuberculous, 2668, 2672
Pernicious anaemia, ocular
tions, 2737
Pertussis and uveitis, 21.51
Phakomatoses, 2843
Photopsiae in choroiditis, 2204
Pick’s disease, 2645
Pigment disturbances in uvea, 2419
Pigmentary degeneration of retina,
primary, 2765
and glaucoma, 3316
secondary, 2239, 2662, 2766
epithelium, senile changes in, 2746
Pilocarpine in glaucoma, 3394
Pipe-stem sheathing of retinal vessels,
2680
Plague and retinitis, 2673
Platyhelminthes, 3438
Plerocephalic oedema, 2945
Plexiform neuroma of uvea, 2466
Poikilodermia atrophicans vasculare and
cataract, 3220
Poliosis and uveitis, 2364
Polycythaemia, iris complications, 2105
retinal complications, 2738
and retinal cyanosis, 2553
complica-
Polyneuritis and optic neuritis, 2997
Pregnancy and optic neuritis, 3006
retinopathy in, 2723
Pre-retinal occlema, 2593
Pressure, intra-ocular.
Hypotony.
Proliferating traumatic chorio-retinitis,
2101
Prostigmin in glaucoma, 3391
Protein shock therapy in uveitis, 2214
Pseudo-glaucoma, 3071, 3354
Pseudo-glioma, 2825
inflammatory, 21.70
pathology of, 2170
Pseudo-neuritis, 2977
Pseudo-rosette, 2834
Pseudo-tuberculous irido-cyclitis, 2130,
2368
Pseudo-xanthoma elasticum, 2417
Pulsating exophthalmos and glaucoma,
3316 -
Pupil in iritis, 2199
occlusion of, pathology, 21.69
seclusion of, pathology, 21.68
Pupillary margin, hyaline degeneration of,
2390
Purpura haemorrhagica, retinal complica-
tions of, 2740
and iridic hamorrhages, 2109
Pyramidal cataract, 3112
See Glaucoma,
Q
Quinine amblyopia, 3031
R
Racemose aneurysm of retina, 26.17
Radiation in retinal tumours, 2827
in uveal tumours, 2517
in uveitis, 2212
in venous thrombosis, 2587
Raynaud’s disease, choroidal changes,
2105
Recidive irido-cyclitis, 2264
v. Recklinghausen’s disease of optic
nerve, 3087
of retina, 2860
uveal manifestations of, 34.66
Recurrent fever and retinitis, 2673
3466
INDEX
Reflex of retinal vessels, 2677
Relapsing fever and uveal disease, 2151
Renal disease and uveal arteriosclerosis,
2371
and choroidal disease, 2386
and retinal disease, 2709
retinopathy, 2712
Rete mirabile, 2620
Retina, actinomycosis of, 2673
anaemia of, 2555
angiospasm in, 2566
arteriolar sclerosis of, 2700
arteriosclerosis of, 2696
atheroma in, 2697
atrophy of, 2745
circulatory anomalies of, 2551
cyanosis of, 2552
in cyclitis, 2182, 2201
cystic degeneration of, 2752
cysts of, 2806
degenerations of, 2745
post-inflammatory, 2628
dermato-myositis of, 2679
detachment of, 2864
aetiology, 2889
and cataract, 3231
in choroiditis, 2172
cysts in, 28.10
delayed, 2871
diagnosis, 2907
experimental, 2888
exudative, 2896
and glaucoma, 3315
histology of, 2874
holes in, 2878
hypotension in, 3418
and iritis, 2132
post-inflammatory, 2538
post-operation, 2872
predisposing factors, 2867
prognosis of, 2918
remote, 2483
in renal retinopathy, 27.17
in retinopathy of pregnancy, 2725
in tumours, 2513
serous, 2897
sub-retinal fluid in, 2887
symptoms of, 2899
tears in, 2878
tension in, 2886, 3315
traumatic, 2870
treatment of, 2908
Retina, detachment of, in tumours, 2483,
2513
and uveitis, 2172
uveitis following, 2132, 2252
vitreous changes in, 3262
diffuse hyperplastic arteriosclerosis in,
2700
diseases of, 2544
embolism in, 2568
gliosis of, 2626
haemorrhages in, 2595
See also Haemorrhages.
hyperaemia of, 2552
hypertensive sclerosis in, 2698
inflammation of, 2622
involuntary arteriosclerosis of, 2702
ischaemia of, 2556
leprosy of, 2673
neuritis of, 2246
Oedema of, 2588
peri-arteritis of, 2639
peri-phlebitis of, 2639
secondary, 2660
peri-vasculitis of, 2639
syphilitic, 2663
pigmentary degeneration of, primary,
secondary, 2239
punctate deposits on, 2656
senile changes in, 2745
syphilis of, 2660
thrombosis, arteriolar, 2578
venous, 2578
tumours of, 2812
vascular diseases of, 2675
vessels of, in arteriosclerosis, 2677
wounds of, 2549
Retinal arteries, obstruction of, 2561
embolism of, 2568
endarteritis of, 2572
spasm of, 2566
thrombosis of, 2572
haemorrhages, 2595
See also Haemorrhages.
striae, 2906 *
veins, anastomoses, anomalous, 2613
endo-phlebitis of, 2578
obstruction of, 2578
thrombosis of, 2578
varicosities in, 2613
vessels, anastomoses, anomalous, 2613
aneurysms of, 2615
INDEX
34.67
Retinal vessels, anomalies of, 2612
tortuosity of, 2612
varicosities in, 2613
See also Retinopathy, 2622
Retinitis,
atrophicans, 2756
cachecticorum, 2645
central angiospastic, 2593
punctate, 2729, 2733
centralis annularis, 2593
circinate, 2760
circum-papillaris, 2661
external exudative, 2646
haemorrhagic, 2610
exudative, 2648
in gonorrhoea, 2673
gummatous, 2664
honeycomb of Doyne, 2786
in influenza, 2673
leprotic, 2673
in malaria, 2673
in measles, 2673
metastatic, diffuse, 2637
localized, 2637
pigmentosa, 2765
See Retina, pigmentary degeneration
of.
in plague, 2673
primary, 2637
proliferans, 2604
pseudo-nephritic, 2644
punctata albescens, 2784
purulent, 2636
in recurrent fever, 2673
of Roth, 2646
secondary, 2655
senile exudative macular, 2116
septica, 2646
sero-fibrinous degenerative (of Leber),
2648
in spirochaetosis ictero-haemorrhagica,
2673
stellata, 2644
syphilitic, 2660
central serous, 2644
toxic exudative, 2664
toxic exudative, 2644
tuberculous, 2666
exudative, 2668
Retino-blastoma, 2833
Retino-choroiditis, 2246
radiata, 2672
Retino-cytoma, 2833
Retinoma, 2833
Retinopathy, arteriosclerotic, 2707
central angiospastic, 2578, 2594
serous, 2592
circinate, 2760
diabetic, 2729
hypertensive, 2726
leukaemic, 2741
malignant hypertensive, 2726
of pregnancy, 2723
renal, 2709
stellate, 2553, 2591, 2595
toxaemic of pregnancy, 2723
treatment of, 2735
vascular, 2706
Retro-bulbar neuritis, 2978 .
Rheumatism and uveitis, 2156
Ring sarcoma of uvea, 2490
synechiae, 2168
Rönne's step, 3381
Rosacea iritis, 2154
Roseola of iris, 2262
Rosette, 2837
Rothmund’s disease and cataract, 3220
Roth’s disease, 2646
Rubeosis of iris, 2385
S
Salicylate amblyopia, 3034
Salicylates in uveitis, 22.13
Salus' sign, 2688
Sarcoid of Boeck, and iritis, 2358
Sarcoma, metastatic of retina, 2864
of optic nerve, 3101
of uvea, secondary, 2522
metastatic, 2534
of uveal tract. See Melanoma,
malignant.
Scarlatina and uveal disease, 2150
Scarlet fever and uveal disease, 2150
Schilder’s disease and optic neuritis, 2997
Scintillatio albescens (nivea), 3249
Sclerectomy in glaucoma, 3402
late infections after, 2129
Sclerodermia and cataract, 3220
Sclero-uveitis, 2131
Scotoma, Bjerrum’s comet, 3381
Förster’s ring, 2269
junction, 2980
3468
INDEX *
Scotoma, nerve bundle, 3381
Rönne's step, 3384
sector, 2246
Seidel’s sickle, 3381
See also Fields of vision.
Scurvy, and iridic haemorrhage, 2109
retinal complications of, 2740
Seclusio papillae, pathology of, 2168
Sector-shaped scotoma, 2246
Seidel’s sickle scotoma, 3381
Senile capsular exfoliation, 3106
changes in choroid, 2396
in ciliary body, 2393
in iris, 2389
in pigmentary epithelium, 2746
in retina, 2745
circum-papillary choroidal atrophy,
2397
elastosis and angioid streaks, 24.17
hyperplasia of ciliary epithelium,
2394
macular degeneration, 2372
miosis, 2390
peri-papillary halo, 2397
rigidity of pupil, 2390
Separation of retina. See Retina, detach-
ment of, 2864
Septic choroiditis, 2139
Sero-fibrinous exudative chorio-retinitis
of Leber, 2648 -
Sheathing of retinal vessels, 2680
Siderosis, 2420
Siegrist's spots, 2387
Silver-wire arteries, 2679
Skin disease and uveitis, 2153
Small-pox and uveal disease, 2150
Snow-ball opacities in vitreous, 3249
Spectral cells of Leber, 2631
Spherophakia and glaucoma, 3307
Spielmeyer-Vogt disease, 2801
Spirochaetosis ictero-haemorrhagica and
retinitis, 2673
and uveal disease, 2151
Spongioblastoma of optic nerve, 3080
of retina, 2833
Staphyloma, in glaucoma, 3351
Stargardt’s disease, 2789
Stellate retinopathy, 2553, 2595
Striae retinae, 2906
Sub-acute combined degeneration, retinal
complications, 2737
Sub-arachnoid haemorrhages, 2939
Sub-capsular cataract, 3112
opacities, 3112
Sub-dural haemorrhages, 2938
Sub-hyaloid haemorrhages, 2599
Sulcus circum-marginalis, 2391
Sulphanilamides in uveitis, 22.13
Sympathectomy in pigmentary degenera-
tion of retina, 2781
Sympathetic irritation, 2324
ophthalmitis, 2324
precocious, 2336
perivasculitis, 2346
Synchisis, 3246
scintillans, 3252
Synechia, anterior peripheral, pathology
of, 2169
annular, 2168 -
posterior, pathology of, 2168
ring, 2.168
total, 21.68
Syphilis of optic nerve, 3041
of retina, 2660
of uveal tract, 2261.
T
Tabes, atrophy of iris in, 2403
of optic nerve in, 3045
Tapeto-retinal degenerations, 2765
Tay-Sachs’ disease, 2797
Tay’s choroiditis, 2749
Teeth and uveal disease, 2144
Tenonitis and glaucoma, 3316
Tension of eye, anomalies of, 3280
in irido-cyclitis, 2202
in retinal detachments, 2881
in uveitis, 2218
Tetany and cataract, 3154, 3210
Thallium amblyopia, 3028
cataract, 3153
Thomsen's disease and cataract, 32.17
Thrombo-angiitis obliterans, retinal
complications, 2575
vitreous haemorrhages in, 2602
Thrombosis, arteriolar in retina, 2578
in choroid, 2105
venous and glaucoma, 3311
in retina, 2578
of vortex veins, 2108
Tobacco amblyopia, 3009
Tonsils and uveal disease, 2145
INDEX
3469
Toxaemic retinopathy of pregnancy, 2723
Toxic amblyopia, 3008
cataract, 3221
exudative retinitis, 2644
syphilitic retinitis, 2664
Trabeculotomy, 3407
Trauma, pigmentary disturbances in, 2419
and retinal detachment, 2870
and sympathetic ophthalmitis, 2327
Traumatic glaucoma, 3296
holes at macula, 2757
hypotony, 3419
proliferating chorio-retinitis, 2101
uveitis, 2135, 2253
Trench nephritis and retinopathy, 2713
Trephining, 3402
late infections after, 2129
Trinitrotoluol amblyopia, 3035
Trypanosomiasis and irido-cyclitis, 2323
Tuberculin, in uveitis, 2316
Tuberculoma of choroid, 2306
of ciliary body, 2304
of iris, 2304 -
of optic nerve, 3038
of retina, 2667
Tuberculosis of optic nerve, 3036
of retina, 2666
and sympathetic disease, 2331
tests for, 2312
of uveal tract, 2284
uveo-parotid, 2857
Tumours, cerebral, and papilloedema, 2947
intra-ocular and cataract, 3231
and glaucoma, 3313
and uveitis, 2132, 2252
and vitreous degeneration, 3266
of iris, 2446
of optic nerve, 3073
orbital and glaucoma, 3316
of retina, 2812
of uvea, metastatic, 2522
primary, 2446
secondary, 2520
Typhoid fever and uveal disease, 2150
Typhus fever and retinitis, 2673
U
Ulcers, corneal, and irido-cyclitis, 2131
and sympathetic ophthlamitis, 2328
Uratic iritis, 2380
Uvea, detachments of, 2537
diseases of, 2097
displacements of, 2537
inflammations of, 2126.
See Uveitis, Iritis, Cyclitis, Choroi-
ditis.
vascular diseases of, 2369
Uveal pigment and iritis, 2134
Uveitis, 2126
aetiology of, 2126
allergic, 2.140
with alopoecia, vitiligo, poliosis and
dysacousia, 2364
atrophic, 2132, 2253
exudative, 2251
with fungus infections, 2367
gouty, 2380
metastatic, 2.136
pathology of, 2158.
serous, 224.5
symptomatology of, 2197
traumatic, 2135, 2253, 2303
treatment of, 2206
vitreous changes in, 3262
Uveo-parotid fever, 2358
tuberculosis, 2357
Uveo-parotitis, 2355
V
Vaccines in uveitis, 2207
Vaccinia and uveal disease, 2150
Vaquez's disease, uveal complications,
2380
Varicella and uveal disease, 2150
Variola and uveal disease, 2150
Vascular disease of optic nerve, 3065
of retina, 2675
of uvea, 2369
sclerosis. See also
Atherosclerosis.
primary in choroid, 2369
Vesicular macular oadema, 2756
Virus infections and uveitis, 2139
Vitamins and cataract, 3132, 3145
Vitiligo, 2419
iridis, 2255
and uveitis, 2364
Vitreous body, blood in, 2601, 3255
blood-vessels in, 2618
Arteriosclerosis,
3470
INDEX
Vitreous body, cells in, 3254
cysts in, 3278 -
degenerations of, 3246
detachments of, 3266
diseases of, 3245
fibrous tissue in, 2604
fluidity of, 3246
in haemorrhage, 3265
haemorrhages into, 2601
hernia of, 3273
infections of, 2128
in inflammation, 3262
in lipiodosis, 3256
micro-fibrillary degeneration of, 3260
in myopia, 3260
opacities in, 3248
annular, 3259
pigment in, 3216
prolapse of, 3273
in retinal detachment, 2876, 3262
senile changes in, 3258
in tumours, 3266
Vogt's sign, 3112 . .
Vortex veins, thrombosis of, 2108
W
Wander-tubercle of v. Szily, 2302
Weil's disease and retinitis, 2671
Weil's disease and uveitis, 2151
Whooping cough and uveitis, 2151
Wood alcohol amblyopia, 3021
Wounds, healing of,
in choroid, 2100
in ciliary body, 2099
in iris, 2098
in lens, 3102
in optic nerve, 2929
in retina, 2549
in uveal tract, 2098
of orbit and retinal complications,
2558
and sympathetic ophthalmitis, 2327
X
Xanthomatosis lentis, 3168
Z
Zonular cataract, 31.68
lamella, 3104 .
Zonule of Zinn, degeneration of, 3239
and dislocation of lens, 3239
and tremulousness of lens, 3244
HENRY KIMPION, 263 High Holborn, LoNDON, w.c.1.
UNIVERSITY OF MICHIGAN
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