AORTOESOPHAGEAL FISTULA AND DOUBLE AORTIC-ARCH - 2 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 arc h and to stress important points in the diagnosis and management. A re view of the records of 30 children with double aortic arch disclosed t wo patients who had AEF. The first patient had respiratory distress an d repair of a vascular ring (double aortic arch) at 5 weeks of age. At 9 weeks of age, because of difficulty with tracheal extubation, aorto pexy was performed. Ten days later, profuse upper gastrointestinal ble eding required control by a Sengstaken-Blakemore (SB) tube. Thoracotom y and repair of AEF was accomplished successfully under cardiopulmonar y bypass. The second patient had hepatomegaly and Pseudomonas sepsis. Endotracheal and nasogastric intubation was necessary, and subsequentl y the double aortic arch was demonstrated by magnetic resonance imagin g (MRI). On the 48th day of hospitalization, life-threatening upper ga strointestinal hemorrhage required insertion of an SB tube. Cardiopulm onary bypass allowed successful repair of the AEF. Both children are a live, after 3 and 2 years (respectively). These patients demonstrate t hat AEF must be diagnosed clinically (no imaging technique is effectiv e); 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. (C) 1996 by W.B. Saunders Company