Infants with esophageal atresia and right aortic arch: Characteristics and outcomes from the Midwest Pediatric Surgery Consortium

J Pediatr Surg. 2019 Apr;54(4):688-692. doi: 10.1016/j.jpedsurg.2018.08.002. Epub 2018 Aug 21.


Purpose: Right sided aortic arch (RAA) is a rare anatomic finding in infants with esophageal atresia with or without tracheoesophageal fistula (EA/TEF). In the presence of RAA, significant controversy exists regarding optimal side for thoracotomy in repair of the EA/TEF. The purpose of this study was to characterize the incidence, demographics, surgical approach, and outcomes of patients with RAA and EA/TEF.

Methods: A multi-institutional, IRB approved, retrospective cohort study of infants with EA/TEF treated at 11 children's hospitals in the United States over a 5-year period (2009 to 2014) was performed. All patients had a minimum of one-year follow-up.

Results: In a cohort of 396 infants with esophageal atresia, 20 (5%) had RAA, with 18 having EA with a distal TEF and 2 with pure EA. Compared to infants with left sided arch (LAA), RAA infants had a lower median birth weight, (1.96 kg (IQR 1.54-2.65) vs. 2.57 kg (2.00-3.03), p = 0.01), earlier gestational age (34.5 weeks (IQR 32-37) vs. 37 weeks (35-39), p = 0.01), and a higher incidence of congenital heart disease (90% vs. 32%, p < 0.0001). The most common cardiac lesions in the RAA group were ventricular septal defect (7), tetralogy of Fallot (7) and vascular ring (5). Seventeen infants with RAA underwent successful EA repair, 12 (71%) via right thoracotomy and 5 (29%) through left thoracotomy. Anastomotic strictures trended toward a difference in RAA patients undergoing right thoracotomy for primary repair of their EA/TEF compared to left thoracotomy (50% vs. 0%, p = 0.1). Side of thoracotomy in RAA patients undergoing EA/TEF repair was not significantly associated with mortality, anastomotic leak, recurrent laryngeal nerve injury, recurrent fistula, or esophageal dehiscence (all p > 0.29).

Conclusion: RAA in infants with EA/TEF is rare with an incidence of 5%. Compared to infants with EA/TEF and LAA, infants with EA/TEF and RAA are more severely ill with lower birth weight and higher rates of prematurity and complex congenital heart disease. In neonates with RAA, surgical repair of the EA/TEF is technically feasible via thoracotomy from either chest. A higher incidence of anastomotic strictures may occur with a right-sided approach.

Level of evidence: Level III.

Keywords: Anastomotic stricture; Complications; Esophageal atresia; Right aortic arch; Thoracotomy.

Publication types

  • Multicenter Study

MeSH terms

  • Aorta, Thoracic / abnormalities
  • Aorta, Thoracic / surgery
  • Cohort Studies
  • Esophageal Atresia / complications
  • Esophageal Atresia / epidemiology
  • Esophageal Atresia / surgery*
  • Esophagus / surgery
  • Female
  • Humans
  • Incidence
  • Infant
  • Infant, Newborn
  • Male
  • Postoperative Complications / epidemiology
  • Retrospective Studies
  • Thoracotomy / adverse effects
  • Thoracotomy / methods*
  • Tracheoesophageal Fistula / complications
  • Tracheoesophageal Fistula / epidemiology
  • Tracheoesophageal Fistula / surgery*
  • United States / epidemiology
  • Vascular Ring / complications
  • Vascular Ring / epidemiology
  • Vascular Ring / surgery*

Supplementary concepts

  • Esophageal atresia with or without tracheoesophageal fistula