Fatal aortoesophageal fistula in two cases of tight vascular ring

Abstract

Vascular rings are rare vascular congenital anomalies causing oesophageal and tracheal compression. An aortoesophageal fistula is a devastating, in part iatrogenic, complication of vascular rings. It is seen with increasing frequency, and can be misleading, since differential diagnosis with other causes of haematemesis and melaena is often difficult, especially in infants. We report two infants with aortoesophageal fistulas secondary to double aortic arches forming a vascular ring. In both, the diagnosis was missed, and massive haemorrhage led to death. In both cases, the fissuration on the oesophageal and aortic sides of the fistula had sharp edges, highly suggestive of an iatrogenic laceration caused by manipulation of nasogastric tubes. The key for the diagnosis of vascular rings is, therefore, clinical suspicion and awareness of this condition. Prompt identification in infants with stridor, wheezing, or respiratory distress can prevent prolonged intubation, thus avoiding the formation of an aortoesophageal fistula and hopefully preventing a fatal outcome.