Caroli's disease: study of six cases, including one with epithelial dysplasia

Six cases of Caroli's Disease are reviewed. Three of the patients had an associated form of the disease, one of which had a congenital hepatic fibrosis, another a cystic dilatation of extrahepatic bile duct and the last one suffered both the above mentioned associated anomalies. Three patients presented with the simple form which was initially described by Caroli. The distribution of the biliary lesions was a bilobar one in four cases and monolobar in two. One patient presented areas of biliary ectasia partially lined by dysplastic epithelium. Surgical treatment was used in four cases, a left hepatectomy being carried out in two of them and an internal biliary drainage by Roux-Y hepaticojejunostomy in the other two. This is a rare disease, frequently associated with congenital hepatic fibrosis, possibly pre-malignant and whose treatment of choice is hepatic resection.