CAROLIS-DISEASE - A SURGICAL DILEMMA

We report the case of a 31-year-old male patient with a diffuse form o f Caroli's disease presenting as recurring bouts of biliary pancreatit is. Following sphincterotomy, the patient remained asymptomatic for 5 years. He then developed acute cholangitis and, at laparotomy, all sup erficial liver cysts were fenestrated in order to remove intrahepatic bile duct calculi. A right hepatectomy, removing the most severely aff ected liver parenchyma was not considered feasible because of the smal l size of the left lobe and the existence of an associated congenital hepatic fibrosis. In diffuse forms of Caroli's disease biliary drainag e techniques have often proved ineffective in preventing recurring bou ts of cholangitis. Moreover, liver resection is seldom feasible becaus e of associated congenital hepatic fibrosis. In this setting, liver tr ansplantation may represent the only effective and durable form of tre atment.