A case of simple type Caroli's disease confined to one segment of the liver / 대한내과학회지

ABSTRACT

Caroli's disease is a rare congenital anomaly of the intrahepatic biliary tree characterized by multiple, saccular and cystic dilatation of the bile ducts that are connected with the biliary drainage system. It is frequently associated with congenital hepatic fibrosis, resulting in portal hypertension. The complications are stone formation, recurrent cholangitis and occasionally cancer. Less than 20% of all cases reported describe monolobar or segmental invovement. In the pure form or simple type, the patient presents without hepatic fibrosis or portal hypertension. We report a case of simple type Caroli's disease confined to the one hepatic segment in a 22 year-old woman. She was admitted because of cystic mass, associated with right upper abdominal pain. Radiologic findings including abdominal ultrasonography, computerized tomography, magnetic resonance imaging demonstrated that the cystic lesions involving segment VIII of the liver. The lesion was treated with hepatic segmentectomy.