Clinical profile and outcome of infants with biliary atresia in the era of liver transplantation.

Background: Biliary atresia (BA) is a progressive, fibro-inflammatory cholangiopathy of infants with Kasai portoenterostomy (KPE) as the first-line surgical treatment. Most children eventually develop end-stage liver disease requiring liver transplantation (LT). Some infants are treated with primary LT (PLT), but the majority undergo KPE with a subsequent salvage LT (KPSLT). This aim was to study the clinical profile and outcome of infants with BA in the era of LT. Methods: In this retrospective study from a tertiary care hospital of North India, all infants with BA who presented from January 2021 to October 2023 were included. The age at onset, disease severity based on Child-Pugh and Pediatric End-stage Liver Disease score, presence of cholangitis, decompensation, and complications were assessed. The age of undergoing KPE, need of primary or subsequent LT, and survival in patients of KPE with native liver (KPNL), KPSLT, and those with PLT were compared. Results: Of 30 patients with BA, 19 (63.3%) underwent KPE, while 10 (33.33%) underwent PLT. One patient died before undergoing any surgical treatment. Subsequent salvage LT was done in 9/19 (47.3%) of patients with KPE. The 1-year survival was 8/10 (80%) in KPNL group, 6/9 (66.7%) in KPSLT group, and 9/10 (90.0%) in PLT group. The overall survival of KPE group with native liver was 42.1% (8/19). The median age of presentation was higher in the PLT group compared with KPNL group (p < 0.001). Conclusion: Patients with BA with PLT have comparable outcome to those with KPSLT. Younger age of KPE correlates with better survival outcome.