Carolis Disease

Caroli's Disease is a congenital disease of the intrahepatic biliary tree characterized by multiple, saccular dilations of the bile ducts. Two types occur: the simple type and the periportal fibrosis type. The simple type is associated with recurrent cholangitis, liver abscesses, abdominal pain, and fever. It is also associated with "medullary sponge kidney," cystic lesions of the kidney, 60-80% of the time. The periportal fibrosis type has the same type of intrahepatic biliary dilation but also is associated with congenital hepatic fibrosis, cirrhosis, and portal hypertension. Cho-langitis and kidney lesions are also commonly seen with this type. Caroli's disease is highly premalignant.

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