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

Abstract

An unrecognised right aortic arch (RAA) found at thoracotomy may complicate the repair of oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF). This paper analyses the patient characteristics, peri-operative management, and outcome of 16 infants with a RAA, and proposes management guidelines. Between 1948 and 1996, 709 patients with OA/TOF were admitted to the Royal 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%. Neither 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 infants examined; that patient underwent repair of the OA through a left (L) thoracotomy. The other 15 infants underwent initial right (R) thoracotomy. Six of these had a complete repair from the R side and 5 had division of the fistula only; 2 of these 5 had initial division of the fistula, and the OA was repaired through a repeat R thoracotomy 4 and 7 weeks later. In the remaining 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 1 week later. There were 6 deaths: these occurred early in the study 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 thoracotomy for OA, it is often possible to divide the fistula and repair the OA from that side, but where repair looks potentially difficult it is wise to proceed to an immediate L thoracotomy.

Tripartite esophagus

“…Babu [3] found RAA in 12 of 476 patients, 11 unsuspected, 7 repaired on the right, 2 converted immediately to left, and 2 had fistula ligation only. Bowkett [4] has similar results-15 RAA in 709 patients, all undiagnosed, 6 repaired, 4 converted, and 5 having fistula ligation only. The overall advice would seem to be to use the approach which makes the operator most confident, realizing that both have disadvantages.…”

Impact of preoperative diagnosis of congenital heart disease on the treatment of esophageal atresia

“…MRI [28][29][30] or aortography [28,31] can accurately delineate the position of the aortic arch but they are not available everywhere around the clock and it is not realistic to pretend that that their routine use in newborns with EA is required. On the contrary, ultrasonography is the best non-invasive method of diagnosis of RAA [14] as attested by the high rate of preoperative diagnosis in our series (50%) in comparison with other series (8-15%) [24][25][26]. Nevertheless, since like other authors [24,25] we used a right approach in all operated cases without any major technical problem related to the RAA, we do not believe that this diagnosis is determinant prior to EA repair.…”

“…On the contrary, ultrasonography is the best non-invasive method of diagnosis of RAA [14] as attested by the high rate of preoperative diagnosis in our series (50%) in comparison with other series (8-15%) [24][25][26]. Nevertheless, since like other authors [24,25] we used a right approach in all operated cases without any major technical problem related to the RAA, we do not believe that this diagnosis is determinant prior to EA repair. The same can be applied to our two cases of dextrocardia or ARSA.…”

“…Six children (3%) had RAA, a proportion similar to that reported in other series (1.8-5%) [24][25][26]. Although it has been claimed that the finding of tracheal displacement at a certain level in plain X-ray of the thorax suffices the diagnosis of RAA [26,27] other investigators disagree [28] and our experience goes in the same direction because no baby in our series was diagnosed on this basis.…”

Surgical management of esophageal atresia and tracheo-esophageal fistula associated with right-sided aortic arc

“…Bowkett ve ark. sağ torakotomi ile onarımın denenmesini, ancak yapılamadığı durumlarda sol torakotomiye geçilmesini önermişler (4) . Babu ve ark.…”