Liver Transplantation for Congenital Biliary Dilatation

Abstract

Background : Congenital biliary dilatation is a rare disease. Although the possibility of refractory cholangitis and/or the frequency of malignant tumours legitimize hepatobiliary surgery, repeated cholangitis and biliary obstruction result in secondary liver cirrhosis even after polysurgery. There are no definitive guidelines of liver transplantation for congenital biliary dilatation patients. Patients : A total of 1,101 liver transplantation recipients were enrolled in this study. Eleven congenital biliary dilatation patients including 5 Caroli's disease patients were retrospectively analyzed in detail. Results : Nine of eleven patients underwent initial surgeries before liver transplantation, while two Caroli's disease patients received liver transplantation as initial surgery with good outcomes. All patients had intractable symptoms caused by liver cirrhosis, and growth reduction was considerable in patients aged <20 years. Histopathological analysis of the native liver revealed hepatic fibrosis (≥F2). One patient with ABO incompatibility finally died. Though one Caroli's disease patient accompanied with intrahepatic carcinoma, this patient survives 11.8 years after liver transplantation without any recurrences. Conclusions : The congenital biliary dilatation patients with refractory symptoms and complications secondary to liver failure are appropriate candidates for liver transplantation. We suggested that liver transplantation become an effective therapeutic option for congenital biliary dilatation patients, under the thoughtful considerations of many supportive factors, such as clinical course, growth reduction, image findings and histopathological analysis.