key: cord-339940-80757pwu authors: Zaidi, S. Javed; Schweig, Lorene; Patel, Dhaval; Javois, Alexander; Akhter, Javeed title: A novel approach to the diagnosis and treatment of hemoptysis in infants: A case series date: 2018-09-18 journal: Pediatr Pulmonol DOI: 10.1002/ppul.24160 sha: doc_id: 339940 cord_uid: 80757pwu INTRODUCTION: Hemoptysis in children is an uncommon presenting symptom but can be life‐threatening if massive. Cardiac catheterization and coil embolization of aorto‐pulmonary collateral vessels (APCs) is uncommon in pediatric hemoptysis patients without congenital heart disease. METHODS: We present a series of seven infants (≤12 months of age) with hemoptysis, all of whom underwent cardiac catheterization to look for and intervene upon APCs, if found. Only those patients who underwent both bronchoscopy as well as cardiac catheterization from January 1995 to January 2015 were included in this retrospective review. RESULTS: Seven patients met inclusion criteria, and three had a history of recurrent hemoptysis. The mean age was 3 months. Four had evidence of bleeding on bronchoscopy. All seven had respiratory distress which necessitated ICU admission; five required mechanical ventilation. Cardiac catheterization showed significant APCs (>2 mm) in six of the seven studied patients, all of which were coil embolized. One patient had no significant APCs and therefore, no embolization. All patients had complete resolution with no recurrences during the 10‐20‐year outpatient follow‐up period. Chest CT scans were not helpful in delineating the site or etiology of bleeding in any patient. CONCLUSIONS: APCs should be considered as a differential diagnosis for pulmonary hemorrhage in infants after more common causes have been ruled out. Hemoptysis, the coughing up of blood that arises from the lower respiratory tract, is a rare condition in infants and children, but it is potentially life-threatening and may cause significant morbidity. Overall mortality was found to be close to 13%. When massive and untreated, mortality rate may rise to 32%. 1 Unless the volume of blood is considerable, hemoptysis may be difficult to detect because younger children, especially infants, swallow their sputum. However, once identified, the source and underlying pathology must be investigated in a systematic manner. 2 Treatment is initiated as with other bleeds with stabilization of the patient and protection of the airway. Mild hemoptysis may be treated symptomatically while a diagnostic workup is underway. Massive hemoptysis in children needs immediate hospitalization and stabilization. The risk of asphyxiation though exsanguination remains a possibility, 3 underscoring the need for rapid diagnosis and intervention. The differentiation of bronchial arteries from aortopulmonary collateral (APC) vessels is an anatomic one. These terms are often erroneously used interchangeably. APCs arise from the systemic arteries and supply the pulmonary circulation. These vessels, in the setting of congenital heart disease (CHD), can cause a significant systemic to pulmonary shunt. 4 They typically arise from the descending aorta, subclavian artery or its branches, bronchial and intercostal arteries. They often supply blood to the terminal respiratory unit and unlike bronchial arteries do not necessarily travel in close proximity to the bronchial tree. 4 They have been known to be associated with lesions associated with decreased pulmonary blood flow (tetralogy of Fallot, pulmonary atresia) 5 but have also been seen in premature neonates with bronchopulmonary dysplasia. [6] [7] [8] [9] They are usually small and regress spontaneously in this latter group rarely needing intervention. 10, 11 APCs are synonymous with broncho-pulmonary arterial anastomoses and there are very few reports of coil embolization of these vessels for pediatric hemoptysis 2 aside from those associated with known CHD. Prominent APCs are more prevalent with cyanotic structural cardiac lesions. 4 Though massive hemoptysis is rare after palliated congenital cardiac lesions, there have been multiple reports of post-Fontan patients who present with massive hemoptysis, resolving after embolization of multiple aorto-pulmonary collateral vessels. [12] [13] [14] Bronchial arteries are a known cause of massive and recurrent hemoptysis in adults and are often intervened upon in this setting. According to Bruzzi et al, 15 bronchial arteries are responsible for massive hemoptysis requiring treatment in over 90% of cases. Bronchial artery embolization (BAE) is the most frequently performed endovascular procedure and promptly brings bleeding under control in 66-90% of patients. BAE is now considered to be most effective nonsurgical treatment of massive AND recurrent hemoptysis, either as first-line treatment or as an adjunct to elective surgery. 10 APCs may be a neglected source of bleeding in children except those with CHD. This case series focuses on infants, a small subset of hemoptysis patients, to underscore the importance of the presence of APCs in this cohort. All patients examined in this series were 12 months of age and younger and underwent cardiac catheterization after a work-up did not reveal the cause of bleeding. The aim of this study was to assess how many infants presenting with hemoptysis would have APCs and to assess the recurrence of hemoptysis after cardiac catheterization and coil embolization of these APCs, if found. After IRB approval was received, a retrospective review of the pediatric pulmonology and cardiology databases at our tertiary children's hospital was performed to identify patients with hemoptysis as their reason for hospitalization and who also underwent a bronchoscopy and subsequent cardiac catheterization between January 1995 and January 2015. All patients with a history of congenital heart disease (except foramen ovale) and/or older than 12 months of age were excluded. Data collection from the electronic medical record (EMR) included: demographics, symptoms, comorbid conditions, laboratory, and radiologic test results, as well as interventions and outcomes. Due to our institution's long-standing hemoptysis guidelines, all patients received the same standard diagnostic assessment that comprised the following testing: 1) routine and immunologic/ rheumatologic bloodwork (complete blood count, chem profile, anti- Our hospital's hemoptysis management also requires consultation with a pediatric cardiologist and cardiac catheterization (once hemoptysis and its location are confirmed and other common causes of hemoptysis are ruled out). Cardiac catheterization is performed to detect significant (>2 mm) APCs and occlude them, if found. Therefore, consistent with our standard of care, all patients described in this case series underwent a cardiac catheterization under general anesthesia to allow for better angiography with accurate digital subtraction; this has been noted to be less distressing for patients. 16 This entailed right and left heart catheterization, right pulmonary artery angiography, left pulmonary artery angiography and ascending and descending aortography using biplane angiography to reduce radiation dosage in all cases. If significant APCs were found, coil embolization was performed with 3 × 3 cm stainless steel or platinum coils. Of the 16 patients initially identified in the combined databases, nine patients were excluded due to age and/or congenital heart defect. The remaining seven patients who met inclusion criteria were studied. The mean age was 3 months (range 1-12 months). Four patients (47%) were female. A summary of the number and percentage of infants with hemoptysis-related characteristics can be found in Table 1 . Although the quantity of blood loss was not consistently documented in the EMR, the volume was qualified to be significant based The respiratory viral panel was positive for Coronavirus in one patient. On culture of bronchial washings, none of the patients showed specific infectious agents. All remaining cultures were negative for bacterial, fungal, and mycobacterial organisms. One patient's anti-nuclear antibodies were positive. The hemoglobin range of the entire study population was between 9.5 and 13.1 g/dL. No patients required blood transfusions. Chest radiography had positive findings in all seven patients, and five of them demonstrated either bilateral hilar or bibasilar interstitial streaky opacification. In the remaining two patients, the chest x-ray was not diagnostic for a specific site or etiology, however it did accurately reveal which lung was affected as confirmed by subsequent bronchoscopy. In one patient with right pulmonary opacification, right bronchus bleeding was found. In the other, bilateral pulmonary opacification was consistent with visualization of bleeding from both lungs on bronchoscopy. Cardiac catheterization was performed in all seven patients after their workup failed to reveal specific hemoptysis etiology. General diagnostic information including hemodynamic and saturation measurements were performed and within the normal range for all patients. There was no evidence of pulmonary hypertension in any of the cohort patients. Significant APCs (defined as vessels >2 mm in diameter at take-off from the descending aorta) were found in six of the seven patients, and coil embolization using 3 mm coils was performed in all six of these patients (see Figures 1 and 2) . In one patient, no significant APCs were seen; therefore, no embolizations were performed during cardiac catheterization. Long-term hemoptysis recurrence post-embolization 0 (0%) A chest x-ray or, when indicated, a CT scan may provide additional information but were not helpful in our study with all patients getting a chest CT but none of the scans being diagnostic for the site or etiology of bleeding. A 2012 study from Egypt, 18 using multi-detector chest CT scans identified the cause in 84% of cases, and the site and vascular origin in 76% of patients presenting with hemoptysis (mean age 50 years). However, this study was in the adult population and different from our cohort. Its applicability to the pediatric population is unknown. This modality offers the advantage of being non-invasive, but the disadvantage is the inability to perform interventions, unlike cardiac catheterization. Both modalities lend themselves to radiation exposure. However, with recent advances in technology, operators in both fields strive to minimize this exposure. Bronchoscopy is an essential tool to help aid the localization of bleeding and possible etiology. In addition, bronchoscopy can be used for therapeutic interventions 19, 20 mostly in older children, which include CO 2 laser bronchoscopy, Nd-YAG laser bronchoscopy, endoscopic balloon occlusion of a lobe or main bronchus, topical airway vasoconstrictors, endoscopic tumor excision, and lobectomy. 4, 13 As expectorated blood often originates from the bronchial arteries, selective bronchial arteriography, and bronchial artery embolization (BAE) has been found to be the safest and most effective therapy for hemoptysis in adults. 21 It is now considered the procedure of choice for the management of massive and recurrent hemoptysis, either as a first-line therapy or as an adjunct to elective surgery. 17, [22] [23] [24] Non-bronchial systemic artery embolization has also been reported in the treatment of hemoptysis in adults. 17 Inadvertent embolization in spinal branches of the bronchial arteries is the | 1507 most serious complication of a BAE. 25 Surgery is the most definitive form of therapy for these patients if endoscopy or embolization fails repeatedly. Bronchial artery embolization has also been reported in children with hemoptysis though this practice is not common. 2, 16, 26 The known complications from bronchial artery embolization in adults include chest pain, dysphagia, and very rarely transverse myelitis due to spinal cord ischemia. 27 Mal et al 21 The retrospective nature of our study has well known limitations and may preclude assessment of subtle differences between our patients. Our sample size was typical for a single center but not large enough to provide conclusive results that can be applied to the general population. We had an inclusion bias due to our institutional policy wherein all infants who presented with hemoptysis in whom a cause could not be determined on standardized testing underwent cardiac catheterization. One infant in our study who presented with hemoptysis did not have APCs and had no recurrence of hemoptysis on follow up. The natural history of hemoptysis in infants is not well studied and it is not possible to conclusively associate our intervention with no recurrences. A larger multicenter study may be necessary to overcome both the sample size and selection bias. Aorto-pulmonary collateral vessels should be considered as a differential diagnosis for pulmonary hemorrhage in an infant after other more common causes of hemoptysis have been ruled out. In addition to early consultation with pediatric pulmonology, a consultation with pediatric cardiology should be considered. Many of these events may possibly be related to these vessels. A bronchoscopy with differential lavage of right and left lungs would confirm and help localize the site of bleeding allowing for selective embolization of these collateral vessels when present during cardiac catheterization. Other imaging modalities may be helpful but cardiac catheterization would have the advantage of being able to perform interventions if needed. 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