'Simple' form of Caroli's disease

The case of a 71 year-old male patient with 'simple' form of Caroli's disease is reported. The 'simple' form is less common than the form associated with congenital fibrosis. In this case the intrahepatic bile duct dilatation was confined to the segments V and VI, and intrahepatic lithiasis and cholelithiasis were associated. Usually, Caroli's disease remains asymptomatic for the first 20 years of the patient's life, sometimes longer, and in few cases for the patient's whole life. 'Simple' asymptomatic Caroli's disease is generally unrecognized and in the case reported it was an intraoperating finding. Patient's age, the presence of a hepatitis C and the absence of malignant changes discouraged us from performing a right hepatectomy or a resection of segments V and VI. Therefore a Roux-en-Y intrahepatic bile duct jejunal anastomosis was done. Three years after the operation the patient is leading a normal life, and is symptom-free.