[Caroli's disease]

Caroli's disease (CS) is a rare malformation of the bile duct with multiple segmental communicating dilatations of intrahepatic bile ducts affecting the whole of or parts of the liver. The disease is presumably of autosomal recessive hereditary character. Caroli described two types: the rare isolated variety (type 1), is characterized by recurring episodes of cholangitis. The more frequently occurring type 2 is associated with congenital hepatic fibrosis, and consequently there are also symptoms of portal hypertension. Both types may make their first appearance at a very early age. A case story is presented. In the light of the development of carcinoma in 7% of the patients, the authors recommend that patients who cannot be operated radically should have regular, clinical follow-ups including ultrasonography and biopsies if necessary.