The role of liver transplantation in patients with Caroli's disease

Caroli's disease, characterized by segmental or diffuse dilation of the int rahepatic biliary ducts, Is a rare disease which is difficult to treat. The course of the disorder is characterized by recurrent episodes of cholangit is and hospital stays, with a consequent loss of quality-of-life and produc tive capacity, often ending in death due to uncontrolled infection. Endosco pic drainage of the bile duct, percutaneously or surgically, is palliative, and presents bad results in the follow-up of these patients. Orthotopic li ver transplantation appears to be an effective curative option for the trea tment of patients with Caroli's disease associated to complications. The au thors present the course of two cases of this disease, associated with cong enital fibrosis of the liver worsened by repeated episodes of cholangitis, submitted to orthotopic liver transplantation.