RESUMO
Caroli's disease is characterized by uni- or bilobar cystic enlargement of the intrahepatic biliary tract. Clinical symptoms include recurrent cholangitis, right upper quadrant abdominal pain and jaundice. The diagnosis is usually established preoperatively based on abdominal ultrasound, computertomography and ERCP. Eight patients suffering from unilobar Caroli's disease underwent liver resection during a period of six years. The postoperative course was uneventful, and after a median follow-up of 39.6 months in one case only a temporary jaundice occurred. Liver resection is the method of choice for Caroli's syndrome when feasible. Apart from the curative intention liver resection represents also a prophylactic method since the risk of malignant transformation to cholangiocellular carcinoma in the cystic walls after draining procedures is considerably high. For diffuse spread of Caroli's disease in the liver orthotopic liver transplantation has emerged as therapeutic option.