Abstract
Familial occurrence of congenital biliary dilatation (CBD) has been reported, but that in adult cases is rarely reported. Although CBD is sometimes associated with bile duct cancer, coexistence of cancer, dysplasia and hyperplasia of the bile duct has never been documented. We experienced a case of mother and daughter with CBD who were diagnosed at adult age. A 70-year-old woman presented with jaundice and underwent extrahepatic bile duct resection for Todani Type IV CBD and anomalous pancreatobiliary junction. Pathological examination revealed cancer, dysplasia and hyperplasia in the dilated bile duct. Her daughter had undergone surgery for Todani Type I CBD without biliary neoplasia at the age of 37 years. In 32 reported cases of CBD from 16 families, only two cases from one family were diagnosed at adult age and none had biliary tract cancer. Because biliary tract cancer associated with CBD has poor prognosis, CBD should be treated before cancer develops. Screening for CBD is recommended to relatives with family history of CBD.
