Survival in a 2-year-old boy with hemorrhage secondary to an aortoesophageal fistula

Aortoesophageal fistula (AOF) in children with no history of cardiac disease or trauma is extremely rare, and survival is even rarer. We present a case of a 2-year-old boy who presented with massive hematemesis secondary to a primary aortoesophageal fistula and was found at endoscopy to have a submucosal hematoma. He then suffered a cardiac arrest with further catastrophic bleeding only controlled by a Sengstaken-Blakemore tube. Subsequent cardiopulmonary bypass was instituted and allowed direct repair of the fistula. He was discharged home 4 weeks later. The etiology of the fistula remains unknown.