The Right-sided Aortic Arch in Children with Oesophageal Atresia and Tracheo-oesophageal Fistula
11 May 2011
24 June 2011
29 September 2011 (online)
Aim A right-sided aortic arch (RAA) occurs in around 5% of patients with oesophageal atresia and tracheo-oesophageal fistula (OA/TOF). This anatomical variation can complicate the operative management of these patients, as it is often not diagnosed preoperatively but only discovered at thoracotomy, and it remains unproven as to whether a right or left thoracotomy is the best operative approach. This retrospective study aimed to determine the prevalence of RAA in OA/TOF, review the accuracy of preoperative investigations, and investigate the best operative approach, by reviewing the literature and our own patient series.
Methods The case notes of all infants with OA/TOF over a 15 year period (1994–2008) were retrospectively analysed to identify those with a RAA. Birth weight, gestational age, associated anomalies, preoperative investigations, surgical management, postoperative complications and long-term prognosis were all extracted.
Main Results A total of 107 case notes of OA/TOF infants were reviewed, identifying 4 with a RAA. Preoperative echocardiography was performed in all of the 4 RAA infants, but RAA was only identified in one. All 4 infants were managed surgically via a right thoracotomy, regardless of the echocardiography result, with primary anastomosis achieved successfully in all. A laryngeal cleft repair was performed in 1 infant due to an interarytenoid cleft. Laparoscopic fundoplication was performed in 1 patient, because of severe gastro-oesophageal reflux. There were no postoperative anastomotic leaks, bleeding, or deaths in this group.
Conclusion In our study, the incidence of RAA in OA/TOF was 3.7%. Preoperative echocardiography identified the RAA in only 1 of 4 cases. However, echocardiography was helpful for diagnosing other cardiac anomalies, which might have potentially affected the management of these patients. Previous studies have cited the operative difficulties associated with RAA and OA/TOF. However, in this series of 4 infants, primary anastomosis was achieved via conventional right thoracotomy without complication, and with no effect on outcome or prognosis. Therefore, we conclude that, where possible, a conventional right-sided thoracotomy should be performed in OA/TOF patients with a RAA.
- 1 Canty TG, Boyle EM, Linden B , et al. Aortic arch anomalies associated with long gap esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 1997; 32: 1587-1591
- 2 Harrison M, Hanson B , et al. The significance of right aortic arch in repair of oesophageal atresia and tracheoesophageal fistula. J Pediatr Surg 1977; 12: 861-869
- 3 Harrison M, Weitzman J, De Lorimier L. Localization of the aortic arch prior to repair of esophageal atresia. J Pediatr Surg 1980; 15: 312
- 4 Berdon WE, Baker DH, Schullinger JN , et al. Plain film detection of right aortic arch in infants with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 1979; 14: 436-437
- 5 Bhimji S. Vascular rings (2008). Viewed at http://emedicine.medscape.com/article/426233-overview on 02/05/2011
- 6 Allen SR, Ignacio R, Falcone RA. The effect of a right-sided aortic arch on outcome in children with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg 2006; 41: 479-483
- 7 Ogunbiyi O, Makanjuola D. Right aortic arch: CT diagnosis. Afr J Med Med Sci 1994; 23: 23-27
- 8 Bowkett B, Beasley SW, Myers NA. The frequency, significance, and management of a right aortic arch in association with esophageal atresia. Pediatr Surg Int 1999; 15: 28-31
- 9 Babu R, Spitz PL, Drake DP. The management of oesophageal atresia in neonates with right-sided aortic arch. J Pediatr Surg 2000; 35: 56-58
- 10 Stringel G, Coln D, Guetrin L. Esophageal atresia and right aortic arch. Right or left thoracotomy?. Pediatr Surg Int 1990; 5: 103-105
- 11 Bicakci U, Tander B, Ariturk E , et al. The right-sided aortic arch in children with esophageal atresia and tracheo-esophageal fistula: a repair through the right thoracotomy. Pediatr Surg Int 2009; 25: 423-425