Long-Term Outcomes of Biliary Atresia in Korea: A Nationwide Multicenter Cohort Study (2012-2021)
In brief
About half of Korean infants kept their native liver 10 years after surgery
In this 426-infant cohort, 51.5% were alive with their native liver 10 years after Kasai surgery, while overall survival was 92.4%; jaundice cleared in 54%. Jaundice clearance was strongly linked to native-liver survival, and surgery by 90 days was linked to better overall survival, but this retrospective study cannot establish cause and effect.
- Journal
- Journal of pediatric surgery (Q1)
- Published
- 24 September 2026
- Study design
- Retrospective cohort
- Evidence level
- Level 3, Low (CEBM 3b)
- Authors
- Kyong Ihn, Ki Hoon Kim, Soo-Hong Kim, Hae-Young Kim, Hyun-Young Kim, Younghyun Na, et al.
- PMID
- 42785667
- DOI
- 10.1016/j.jpedsurg.2026.163501
Why clinicians should know about it
- Picked for Pediatric Surgery (paper of the day, 25 September 2026): Nationwide cohort of biliary atresia outcomes after Kasai procedure
Abstract
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.
Abstract as published, via PubMed.
For healthcare professionals. The summary is generated by AI from the published abstract, and the evidence level is assigned automatically from the study design on the Oxford CEBM hierarchy. Neither is medical advice. Read the full paper before changing practice.