Caroli's disease: a surgical dilemma

We report the case of a 31-year-old male patient with a diffuse form of Caroli's disease presenting as recurring bouts of biliary pancreatitis. Following sphincterotomy, the patient remained asymptomatic for 5 years. He then developed acute cholangitis and, at laparotomy, all superficial liver cysts were fenestrated in order to remove intrahepatic bile duct calculi. A right hepatectomy, removing the most severely affected liver parenchyma was not considered feasible because of the small size of the left lobe and the existence of an associated congenital hepatic fibrosis. In diffuse forms of Caroli's disease biliary drainage techniques have often proved ineffective in preventing recurring bouts of cholangitis. Moreover, liver resection is seldom feasible because of associated congenital hepatic fibrosis. In this setting, liver transplantation may represent the only effective and durable form of treatment.