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Updated: Aug 22, 2026
An Ivor Lewis Esophagectomy Designed to Minimize Anastomotic Complications and Optimize Conduit Function
Published on: April 17, 2020
Operative planning in esophageal atresia with right arch-based vascular rings: A referral-center experience
Abdimajid Mohamed1, Valeria E Ruiz-Santana2, Alex Huang1
1Division of Pediatric Surgery, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Insights
Esophageal atresia with vascular ring is complex, often requiring reoperation due to complications. Early multidisciplinary planning and advanced imaging are crucial for better outcomes in these challenging pediatric cases.
Area of Science:
- Pediatric surgery
- Congenital anomalies
- Thoracic surgery
Background:
- Esophageal atresia (EA) with complete vascular ring (VR) presents significant challenges in airway, esophageal, and vascular management.
- Management is complex, often requiring specialized centers and multidisciplinary approaches.
Purpose of the Study:
- To review outcomes and reoperation patterns in patients with EA and VR.
- To identify considerations for operative planning in complex EA and VR cases.
Main Methods:
- Retrospective review of pediatric patients with EA and VR managed between 2017-2025.
- Categorization into primary and referred cases, with detailed analysis of anatomy, imaging, surgical approach, and outcomes.
Main Results:
- Twenty-four patients were analyzed, predominantly with type C EA and right aortic arch variants.
- Eighty-four percent of patients were referred, with 75% requiring reoperation for complications like leaks, strictures, fistulas, dysphagia, and vocal fold impairment.
- A novel entity, "trapped esophagus," was identified, requiring reoperation due to vascular compression of the EA anastomosis.
Conclusions:
- EA with VR is associated with high morbidity and reoperation rates.
- Preoperative cross-sectional imaging and multidisciplinary planning are recommended for patients with EA and right aortic arch.
- Concurrent primary repair of EA and VR should be considered in select cases to improve outcomes.
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.
Level Of Evidence:
IV.
