The frequency, significance, and management of a right aortic arch in association with esophageal atresia

An unrecognised right aortic arch (RAA) found at thoracotomy may complicate the repair of oesophageal atresia (OA) and tracheo-oesophageal fistula (TO F). This paper analyses the patient characteristics. peri-operative managem ent, and outcome of 16 infants with a RAA, and proposes management guidelin es. Between 1948 and 1996, 709 patients with OA/TOF were admitted to the Ro yal Children's Hospital. of whom 13 had a RAA. Three additional cases from two other paediatric surgical units were included. All 16 case records were reviewed retrospectively. The overall incidence of RAA in OA was 1.8%. Nei ther a chest radiograph in 16, nor antenatal ultrasonography in 7 detected a RAA. Post-natal echocardiography (ECHG) detected a RAA in only 1 of 7 inf ants examined: that patient underwent repair of the OA through a left (L) t horacotomy. The other 15 infants underwent initial right (R) thoracotomy. S ix of these had a complete repair fi om the R side and 5 had division of th e fistula only; 2 of these 5 had initial division of the fistula. and the O A was repaired through a repeat R thoracotomy 4 and 7 weeks later. In the r emaining 4 infants where the fistula could not be located at the initial R thoracotomy. complete repair proved possible through the L chest. Three of these infants underwent an immediate L thoracotomy: the 4th had a delayed L thoracotomy week later. There were 6 deaths: these occurred early in the s tudy and were related to severe prematurity, congenital heart disease (CHD) , and post-operative respiratory complications. CHD was identified in 11 of 16 infants (71%). Routine pre-operative ECHG is unreliable in determining the laterality of the aortic arch. Should a RAA be encountered during a R t horacotomy for OA, it is often possible to divide the fistula and repair th e OA from that side, but where repair looks potentially difficult it is wis e to proceed to an immediate L thoracotomy.