Abstract
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.
Keywords
right-sided aortic arch - oesophageal atresia - tracheo-oesophageal fistula
