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

Background/Purpose:The management of oesophageal atresia (OA) in associatio n 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-ye ar period (1980 through 1997) were reviewed. Patients with RAA were analyse d with particular reference to the preoperative investigations, operative a pproach and findings, and postoperative complications. Results: Of the 476 case notes reviewed, 12 (2.5%) had a RAA, and 5 of thes e infants had major cardiac anomalies. The diagnosis was not suspected on p reoperative chest x-ray in any of these infants. In only 1 of the 5 neonate s 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 car ried out in 2 infants. In 2 further infants, fistula ligation alone was per formed 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 aorti c arch, and, as a result, a left thoracotomy had to be abandoned in 2. Ther e 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-ra y and echocardiogram are unreliable as diagnostic modalities. Anastomosis v ia 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 thorac otomy as difficult as via a right thoracotomy. Copyright (C) 2000 by W.B. S aunders Company.