Long-Term Outcomes of Biliary Atresia in Korea: A Nationwide Multicenter Cohort Study (2012-2021).

PURPOSE: Biliary atresia (BA) is the leading reason for pediatric liver transplantation (LT). This nationwide multicenter study assessed long-term outcomes following Kasai portoenterostomy (KPE) and identified prognostic factors influencing native liver survival (NLS) and overall survival (OS) in a contemporary Korean cohort. METHODS: We conducted a retrospective analysis of 426 infants diagnosed with BA who underwent KPE between 2012 and 2021 at 15 tertiary centers within the Korean Association of Pediatric Surgeons registry. Clearance of jaundice (CoJ) was defined as a serum total bilirubin level of ≤ 2.0 mg/dL. High-volume centers were identified as those performing ≥ 30 KPEs during the study period. We utilized Kaplan-Meier and multivariable Cox regression analyses. RESULTS: The median age at KPE was 61 days (interquartile range, 42-74). Type III BA represented 62.7% of cases, with CoJ achieved in 54.0%. The ten-year rates for NLS and OS were 51.5% and 92.4%, respectively. Multivariable analysis indicated that CoJ was the strongest predictor of NLS (hazard ratio [HR], 0.12; 95% confidence interval [CI], 0.09-0.18; P < 0.001). Type III anatomy (HR, 1.95; 95% CI, 1.26-3.04; P = 0.003) and biliary atresia splenic malformation (BASM; HR, 4.07; P = 0.011) were linked to poorer NLS. For OS, undergoing KPE at age ≤ 90 days was independently associated with improved survival (HR, 0.26; 95% CI, 0.10-0.67; P = 0.005), while the presence of associated malformations (HR, 3.63; P = 0.008) and failure to achieve CoJ (HR, 0.20 for CoJ achieved; P = 0.004) were negative predictors. CONCLUSIONS: The OS rates were comparable to contemporary international studies, while NLS remained limited. Early KPE and effective bile drainage are crucial for transplant-free survival, underscoring the need for early detection strategies and the evaluation of care centralization in Korea. LEVEL OF EVIDENCE: Treatment Study, Level III.