Operative Planning in Esophageal Atresia With Right Arch-Based Vascular Rings: A Referral-Center Experience

Esophageal atresia (EA) with complete vascular ring (VR) presents complex airway, esophageal, and vascular challenges. We reviewed our referral-center experience to characterize morbidity, patterns of reoperation, and operative planning considerations.

We reviewed children with EA and VR managed between 2017-2025. Patients were categorized as primary or referred (prior management elsewhere). Data included anatomy, imaging, operative approach, and outcomes.

Twenty-four patients were identified (83% type C EA). VR subtypes included right aortic arch with aberrant left subclavian artery (RAA+ALSCA 71%), double aortic arch (17%), and right aortic arch with left ligamentum (13%). Echocardiography was concordant with operative findings in 70%, discordant in 10%, and missed a VR in 20%. Twenty patients (84%) were referred; 75% required reoperation. Referred patients had prior or active leak (42%), refractory stricture (60%), recurrent fistula (28%), dysphagia (90%), and vocal fold movement impairment (53%). Reoperation addressed airway pathology (e.g. tracheomalacia) or vascular compression (n=10), EA-related complications (n=8), and incomplete EA or VR repair (n=4). We identified and describe a new entity: "trapped esophagus" (n=3) characterized by extrinsic vascular compression of an EA anastomosis positioned within an unrepaired VR (RAA+ALSCA) without intrinsic stricture, leading to dysphagia and requiring reoperation. Four patients underwent multidisciplinary primary EA+VR concurrent repair at our institution, without complications.

EA+VR can be associated with high morbidity and need for reoperation. Patients with EA + right arch on echocardiography could benefit from pre-operative cross-sectional imaging and multidisciplinary planning to consider primary EA+VR concurrent repair, taking into consideration reasons for reoperation.

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