Congenital bile duct dilatation (Caroli's disease--Grumbach's disease)

Congenital bile duct dilatation is an autosomal recessive inherited disease. Pathologically tortuous dilated, dysplastic intrahepatic bile ducts were found. It may be associated with hepatic fibrosis (Grumbach's disease), or without (Caroli's disease). A common additional finding is an infantile polycystic kidney. Three cases are reported and the radiological and sonographic findings are discussed.