Abdimajid Mohamed, Valeria E Ruiz-Santana, Alex Huang, Rosa S Kim, Daniel Castellanos, Lianne Cole, Brian P Fallon, Somala Mohammed, Farokh R Demehri, Christopher Baird, Benjamin Zendejas
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引用次数: 0
Abstract
Purpose: 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.
Methods: We reviewed children with EA and VR managed between 2017 and 2025. Patients were categorized as primary or referred (prior management elsewhere). Data included anatomy, imaging, operative approach, and outcomes.
Results: 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.
Conclusion: 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.
期刊介绍：
The journal presents original contributions as well as a complete international abstracts section and other special departments to provide the most current source of information and references in pediatric surgery. The journal is based on the need to improve the surgical care of infants and children, not only through advances in physiology, pathology and surgical techniques, but also by attention to the unique emotional and physical needs of the young patient.
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