Subclavian artery-esophageal fistula is a rare but potentially fatal vascular anomaly. Inherent to Downs syndrome, trisomy 21 presents with a variety of rare cardiac and vascular anomalies. Subclavian-esophageal fistulae are rare and often fatal complications of a right-sided aortic arch. We herein describe a rare case of a 31-year-old man with trisomy 21 who developed massive upper gastrointestinal hemorrhage secondary to an aberrant left subclavian artery-esophageal fistula requiring vascular surgical intervention. Although the etiology remains uncertain, we suspect that our patient's subclavian-artery esophageal fistula developed as a result of a combination of nasogastric tube compression and endotracheal/tracheostomy compression in the setting of aberrant left subclavian artery and a right-sided aortic arch. A broad differential and high index of suspicion is required in diagnosing a patient with trisomy 21 with known congenital cardiac and aortic arch anomaly presenting with acute upper gastrointestinal hemorrhage.
Clements et al. (Tue,) studied this question.