Long-term outcomes in pediatric patients who underwent living donor liver transplantation for biliary atresia

Background: There is no consensus about long-term outcomes in patients with biliary atresia. We retrospectively reviewed the long-term outcomes in pediatric patients who underwent living donor liver transplantation for biliary atresia. Methods: Between May 2001 and December 2020, 221 (73%) of 302 pediatric patients who underwent living donor liver transplantation had biliary atresia. The median age at living donor liver transplantation was 1.2 (range 0.2-16.5) years, and follow-up was 10.3 +/- 5.5 years. Results: The 10-year graft survival rates in patients with and without biliary atresia were 94% and 89%, respectively (P = .019). The 10-year graft survival was significantly poorer in patients >= 12 years of age (84%) versus those <12 years of age at living donor liver transplantation (0-2 years: 95%; 2-12 years: 96%) (P = .016). The causes of graft failure in patients with biliary atresia included late-onset refractory rejection (n = 6), bowel perforation (n = 2), and acute encephalitis (n = 2), as well as cerebral hemorrhage, hepatic vein thrombosis, and sepsis (n = 1 for all). All 7 patients with graft failure due to refractory rejection and hepatic vein thrombosis underwent repeated liver transplantation and are alive in 2021. The rates of post-transplant portal vein complications and early-onset acute cellular rejection in patients with biliary atresia were higher than in those without biliary atresia (P = .042 and P = .022, respectively). In 2021, of 60 adolescents with biliary atresia, 14 (23%) reported medication nonadherence. The rate of liver dysfunction due to late-onset acute cellular rejection and graft failure due to late-onset refractory rejection in patients with nonadherence was higher than in patients with satisfactory adherence (P = .009). Conclusion: The long-term prognosis after living donor liver transplantation in pediatric patients with biliary atresia is quite good. However, long-term support to enhance medication adherence is required in adolescents with biliary atresia. (C) 2021 Elsevier Inc. All rights reserved.