The significance of right aortic arch in repair of esophageal atresia and tracheoesophageal fistula

Abstract

Of 130 infants with esophageal atresia who were operated on during the past 12 yr, 7 (5.4%) had a right aortic arch. In 1 infant, preoperative recognition of the right arch allowed successful primary repair of the esophagus through the left chest. In 6 infants, an unsuspected right arch was found at the time of initial right thoracotomy. In 2, repair from the right side was difficult but successful. In 4, however, the right arch markedly altered the clinical course, leading to the death of 1 infant, abandonment of esophageal repair and colon interposition in another, and bilateral thoracotomies for successful repair in 2 more infants. From this experience, we conclude that the presence of a right aortic arch is of considerable surgical significance in infants with esophageal atresia. We believe preoperative localization of the arch is important in every infant with esophageal atresia and tracheoesophageal fistula (TEF) not only because a right aortic arch poses a definite technical problem during right-sided repair, but also because a right aortic arch is frequently associated with congenital heart disease or vascular compression of the trachea and esophagus. The following recommendations arise from this review: (1) Infants with esophageal atresia should be screened preoperatively by noninvasive techniques for the presence of a right aortic arch. If these studies raise the possibility of a right aortic arch or there is any other evidence of cardiac disease, angiography should be performed. (2) Surgical repair of esophageal atresia is best performed from the side opposite the aortic arch. (3) If a previously unsuspected right aortic arch is found at the time of right thoracotomy for repair of esophageal atresia, the alternatives in management are a matter of surgical judgment. Our experience suggests that in many cases it is safest to close the right thoracotomy without further dissection of the esophageal pouches or with simple ligation of the TEF, and to perform the definitive esophageal repair through a subsequent left thoracotomy.