Impact of VACTERL association and chromosomal anomalies on outcomes after esophageal atresia repair: insights from the EUPSA Registry

Abstract

Objective: although VACTERL association is a recognized entity in patients with esophageal atresia (EA), its impact on surgical outcomes remains unclear. This study aimed to evaluate the influence of VACTERL association and chromosomal anomalies (VACTERL-CA) on the surgical outcomes of EA patients, offering novel insights into risk stratification.

Methods: all patients enrolled in the European Pediatric Surgeons' Association (EUPSA) Esophageal Atresia Registry (EAR) between July 2014 and December 2017 were included. Patients were classified into two groups: those with VACTERL-CA and those without these anomalies (non-VACTERL). Groups were compared for demographics, associated malformations, surgical approach, complications, and outcomes.

Results: among 372 patients, 22% (n = 82) were classified as VACTERL-CA. This group had significantly lower gestational age (35.9 weeks vs. 37.1 weeks, p = 0.004), birth weight (2,312 g vs. 2,663 g, p < 0.001), and APGAR scores at 5 and 10 minutes (p = 0.005). Surgical strategies, including rates of primary anastomosis (88% in both groups), did not differ. Anastomotic leak and stricture rates were similar; however, recurrent fistula was more common in VACTERL-CA (4.9% vs. 1.0%, p = 0.023). Overall mortality was higher in VACTERL-CA (14.6% vs. 5.2%, p = 0.003), largely due to associated anomalies such as cardiac or neurologic conditions, whereas EA-related mortality was more frequent in non-VACTERL (1% vs. 0%). Sepsis was also more frequent in VACTERL-CA (10.9% vs. 4.5%, p = 0.033). In multivariate analysis, low birth weight (adjusted odds ratios [aOR]: 0.95 per 100 g, p = 0.010) and cardiac malformations (aOR: 2.33, p = 0.002) were independently associated with VACTERL-CA.

Conclusion: EA patients with VACTERL-CA represent a high-risk subgroup characterized by prematurity, major cardiac defects, and increased sepsis risk. These findings highlight the need for early cardiac screening, standardized infection-prevention bundles, and tailored multidisciplinary care to improve survival and reduce preventable complications.

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Identifiers

ORCID for Nigel J. Hall: orcid.org/0000-0001-8570-9374

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