[A case of pure unilobar Caroli disease]

We describe a new case of pure Caroli disease and review of the literature. Although often sporadic, this disease is usually considered an autosomal recessive hereditary disease. It is often either associated with another congenital hepatorenal fibrocystic disease or with extrahepatic bile duct dilatation. The clinical examination and laboratory tests are of not specific if congenital hepatic fibrosis or congenital cysts of the coleduct is lacking. Certain echographic and scan images are however very specific. Complications are related to intrahepatic stone formation and to superinfections. The long-term course appears to involve increased risk of cholangiocarcinoma. Treatment of the localized form includes priority resection. In diffuse disease, treatment may be more medical with antibiotics and sometimes bile solvents. In case of failure, transplantation may be entertained.