A.B. Witte1, D. Lerner2, J. Schneider1, D. Lal1, C. Maloney1 1Medical College Of Wisconsin, Division Of Pediatric Surgery, Milwaukee, WI, USA 2Medical College of Wisconsin, Division Of Pediatric Gastroenterology, Medical College Of Wisconsin; Milwaukee, WI, WI, USA
Introduction: Esophageal atresia (EA), with or without tracheoesophageal fistula (TEF), can be associated with vascular anomalies of the aortic arch, including an aberrant right subclavian artery (ARSA) with a reported rate of 3.4-12%. The surgical approach to EA repair in patients with ARSA can be more challenging due to the proximity of the anomalous vasculature to the esophageal anastomosis. Additionally, ARSA is more common in long-gap EA, a patient population more likely to require numerous operative interventions before and after esophageal continuity is achieved. Previous case reports have described complications of arterio-esophageal fistula following esophageal repair in EA patients with ARSA. Yet, the incidence of these rare complications is unknown and the ideal management strategy of concomitant ARSA in EA patients remains unclear. We aim to better define the management, complications, and outcomes of ARSA in the setting of EA.
Methods: A single-institution retrospective chart review was conducted from 2012-2023, and included patients with diagnosis of ARSA and EA, with or without TEF. Data regarding patient management and complications were acquired. Descriptive statistics were performed.
Results: Of 128 patients with EA, 12 had an ARSA for an overall incidence of 9.4%. In this group, EA with distal TEF was observed in 8/12 (67%) patients, pure EA in 2/12 (17%), EA with proximal TEF in 1/12 (8.3%), and H-type in 1/12 (8.3%). ARSA was diagnosed on echocardiogram in 7/12 (58%) with 5/12 (42%) diagnosed on initial post-natal echocardiogram; the remainder were diagnosed on contrasted tomography angiography (CTA) in 3/12 (25%) and esophagram in 2/12 (8.3%). In 2 patients, ARSA was ligated at the time of initial EA repair without complication (mean follow-up 14 months). Eight (67%) patients with ARSA underwent esophageal dilation for stricture following esophago-esophagostomy. Three (25%) patients with ARSA had an esophageal stent placement for refractory and recurrent esophageal stricture: one patient developed an esophago-arterial fistula necessitating emergent ARSA ligation and reimplantation, one patient was found to have an occluded ARSA on subsequent imaging, and one had the stent prematurely removed due to concern for potential complications from ARSA. The majority of patients in our series (10/12, 83%) were diagnosed with ARSA within the last 5 years, potentially signifying improvement in echocardiography detection rates.
Conclusion: ARSA has the potential to complicate EA repair and may cause long-term complications in the setting of future esophageal procedures, including stent placement. Improved awareness of and screening for ARSA on initial echocardiogram allows for pre-operative planning; however, larger studies are necessary to determine the optimal management strategy for ARSA in infants with EA, particularly those with long gap at risk for future esophageal intervention.