OBJECTIVE: The management of esophageal atresia and tracheoesophageal fistula (EA/TEF) in the presence of a right-sided aortic arch (RAA) remains a significant surgical challenge. This systematic review aims to analyze the diagnostic trends, surgical approaches, and clinical outcomes for EA/TEF patients with an associated RAA.
METHODS: A comprehensive and systematic literature search was performed across major electronic databases, including PubMed, MEDLINE and Embase and systematic review was conducted in accordance with PRISMA guidelines, evaluating studies published between January 2000 and January 2026. The research protocol, detailing the predefined methodology, search strategy, and inclusion criteria, was prospectively registered on the PROSPERO (CRD420261339983) platform prior to the initiation of data extraction. Eligible studies included clinical trials, observational studies, and case series (n ≥ 5) reporting on the incidence, surgical strategies (thoracotomy side/thoracoscopy), and postoperative complications in neonates diagnosed with both EA/TEF and a RAA.
RESULTS: A total of nine studies were included in this systematic review. In eight studies consecutively evaluating all patients with EA/TEF (n = 1640), the prevalence of a RAA was determined to be 5.2% (86/1640). With the inclusion of one specific case series (n = 21) that exclusively reported on patients with concurrent EA and RAA, the clinical characteristics, surgical approaches, and survival outcomes of a total of 107 patients with RAA were analyzed in this review. Preoperative echocardiography (ECHO) correctly identified arch laterality in 10%-80% of cases across the included studies, with detection rates improving to over 70% in series from the last decade. A right-sided approach (thoracotomy or thoracoscopy) was the most common surgical strategy, used in 62.5%-100% of cases, while intraoperative conversion to the contralateral side was rare. Associated congenital heart disease was highly prevalent, ranging from 42% to 90%. Overall mortality ranged from 16.6% to 62.5% across studies, primarily driven by complex cardiac anomalies and severe tracheal impingement from vascular rings. Postoperative morbidity included anastomotic leaks (0%-42.8%), strictures (14.3%-53%), and chylothorax (0%-25%).
CONCLUSION: While right-sided repair is technically feasible for most surgeons due to anatomical familiarity, it might be associated with increased long-term morbidity. Preoperative identification of RAA and associated vascular rings is crucial for surgical planning. A personalized approach, considering a left thoracotomy or thoracoscopy in complex vascular cases, may be considered to optimize outcomes and reduce ischemic complications. Due to the rarity of concurrent EA/TEF and RAA, alongside the heterogeneity of available data, a formal meta-analysis could not be performed.
