ONE CASE OF UNILUMBAR, PURE CAROLI-DISEAS E

We describe a new case of pure Caroli disease and review of the litera ture. Although often sporadic, this disease is usually considered an a utosomal recessive hereditary disease. It is often either associated w ith another congenital hepatorenal fibrocystic disease or with extrahe patic bile duct dilatation. The clinical examination and laboratory te sts are of not specific if congenital hepatic fibrosis or congenital c ysts of the coleduct is lacking. Certain echographic and scan images a re however very specific. Complications are related to intrahepatic st one formation and to superinfections. The long-term course appears to involve increased risk of cholangiocarcinoma. Treatment of the localiz ed 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.