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.