Intrahepatic cystic bile duct dilatations and stone formation: a new case of Caroli's disease

Abstract

Caroli's disease is a rare familial anomaly with circumscribed saccular dilatations of enlarged intrahepatic bile ducts coated by regular or pseudopapillomatous epithelium. The disease may be combined with additional malformations of the extrahepatic biliary tract as well as with medullary sponge kidney. Infection and stone formation are frequent complications. Surgery is indicated if the disease is limited to one love which should be resected or if biliary obstruction in diffuse disease necessitates biliodigestive anastomosis. Antibiotics and CDCA-mediation are essential for symptomatic therapy. We analysed the findings in 40 cases including one own observation.