Esophago-Vascular Fistulae in Children: Five Survivors, Literature Review, and Proposal for Management

Esophago-vascular fistulae in children are almost uniformly fatal with death occurring by exsanguination. We present a single centre series of five surviving patients, a proposal for management and literature review.

Patients were identified from surgical logbooks, surgeon recollection and discharge coding data. Demographics, symptoms, co-morbidities, radiology, management and follow up details were recorded.

Five patients (1M, 4F) were identified. Four were aorto-esophageal and one caroto-esophageal. Median age at initial presentation was 44 (8-177) months. Four patients had cross sectional imaging prior to surgery. Median time from presentation to combined entero-vascular surgery was 15 (0-419) days. Four patients required repair on cardio-pulmonary bypass with four undergoing staged surgical procedures. All required combined esophageal and cardio-vascular surgery. Length of PICU stay following combined surgery was 4 (2-60) days and overall hospital stay was 53 (15-84) days. Median follow up was 51 (17-61) months. Two patients had esophageal atresia and trachea-esophageal fistula managed as neonates. Three had no co-morbidities. Four had esophageal foreign bodies:1 esophageal stent, 2 button batteries, 1 chicken bone. One patient had a complication following colonic interposition. Four patients required an esophagostomy at the time of definitive surgery. All patients were alive and well at last follow up with one having successful reconnection surgery.

In this series, outcomes were favourable. Multidisciplinary discussion and surgery are mandatory. If hemorrhage is controlled at presentation, then survival to discharge is possible but the magnitude of surgical intervention is both significant and very high risk.

Level 3.