IMPACT OF VACTERL ASSOCIATION AND CHROMOSOMAL ANOMALIES ON OUTCOMES AFTER ESOPHAGEAL ATRESIA REPAIR: INSIGHTS FROM THE EUPSA REGISTRY.

Abstract

Aim: 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 association and/or chromosomal anomalies (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 vs. 37.1 weeks, p=0.004), birth weight (2312 g vs. 2663 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 (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.