The management of oesophageal atresia in neonates with right-sided aortic arch.

BACKGROUND/
PURPOSE: The management of oesophageal atresia (OA) in association with a right-sided aortic arch (RAA) is challenging. It is controversial whether right or left thoracotomy is the most appropriate surgical approach. The aim of this study was to determine the prevalence of RAA in patients with OA and the most appropriate surgical approach.
METHODS: The case records of all the neonates with OA treated over an 18-year period (1980 through 1997) were reviewed. Patients with RAA were analysed with particular reference to the preoperative investigations, operative approach and findings, and postoperative complications.
RESULTS: Of the 476 case notes reviewed, 12 (2.5%) had a RAA, and 5 of these infants had major cardiac anomalies. The diagnosis was not suspected on preoperative chest x-ray in any of these infants. In only 1 of the 5 neonates who had a preoperative echocardiogram was RAA suspected. A right thoracic approach was performed in the 11 infants in whom a RAA was not suspected. Immediate conversion to a left thoracotomy with primary anastomosis was carried out in 2 infants. In 2 further infants, fistula ligation alone was performed because of a long gap atresia. Anastomosis via the right thoracotomy was performed in 7 infants. There were 3 anastomotic leaks, 1 intractable stricture, and 1 recurrent fistula. Three patients (25%) had a double aortic arch, and, as a result, a left thoracotomy had to be abandoned in 2. There were 3 deaths, 2 as a result of major cardiac anomalies and 1 related to prematurity and sepsis.
CONCLUSIONS: RAA occurs in 2.5% of infants with OA. Preoperative chest x-ray and echocardiogram are unreliable as diagnostic modalities. Anastomosis via a right thoracotomy is associated with a 42% leak rate. The presence of a double aortic arch can make the oesophageal anastomosis via a left thoracotomy as difficult as via a right thoracotomy.