Aortoesophageal fistula and double aortic arch: two important points in management.

Two children with double aortic arch and aortoesophageal fistula (AEF) are reported to warn of this lethal complication of double aortic arch and to stress important points in the diagnosis and management. A review of the records of 30 children with double aortic arch disclosed two patients who had AEF. The first patient had respiratory distress and repair of a vascular ring (double aortic arch) at 5 weeks of age. At 9 weeks of age, because of difficulty with tracheal extubation, aortopexy was performed. Ten days later, profuse upper gastrointestinal bleeding required control by a Sengstaken-Blakemore (SB) tube. Thoracotomy and repair AEF was accomplished successfully under cardiopulmonary bypass. The second patient had hepatomegaly and Pseudomonas sepsis. Endotracheal and nasogastric intubation was necessary, and subsequently the double aortic arch was demonstrated by magnetic resonance imaging (MRI). On the 48th day of hospitalization, life-threatening upper gastrointestinal hemorrhage required insertion of an SB tube. Cardiopulmonary bypass allowed successful repair of the AEF. Both children are alive, after 3 and 2 years (respectively). These patients demonstrate that AEF must be diagnosed clinically (no imaging technique is effective); its history and physical presentation are typical. The SB tube is effective for controlling the hemorrhage until cardiopulmonary bypass can be performed to allow repair.