Long-term outcome of pediatric liver transplantation for biliary atresia: A 10-year follow-up in a single center

The aim of this study was to review our experience in orthotopic liver transplantation (OLT) for biliary atresia (BA) in children and analyze the survival and prognostic factors, and long-term outcome. We reviewed 332 OLTs performed in 280 children between the years 1986 and 2000. Univariate and multivariate analysis were performed on patient and graft survivals according to recipients' and donors' characteristics as well as intraoperative data. The long-term outcome among the 80 children living at 10 years after OLT was studied according to growth, immunosuppressive therapy, and liver and renal functions. Liver graft status was eventually documented by liver biopsy. Status of rehabilitation was assessed by reviewing school performance and employment. Overall patient survival rates at 1, 5, and 10 years were 85, 82, and 82%, respectively, and the corresponding overall graft survival rates were 77, 73, and 71%. In the multivariate analysis, we identified 4 independent prognostic factors: polysplenia syndrome (P = .03), United Network for Organ Sharing (UNOS) status (P = .05), donor's age (P = .01), and perioperative surgical complications (P = .03). At 10 years after transplant, 80 children were alive and had normal growth rates. Liver histology was abnormal in 73% of these long-term survivors, mainly due to chronic rejection and centrilobular fibrosis. A total of 63 of the 80 children attended normal school and in 55 children (69%) school performance was not delayed. In conclusion, we discovered that a good long-term survival could be achieved after liver transplantation for BA, with a 82% survival rate at 10 years with normal scholastic studies in the majority of recipients.