Caroli's Disease and Caroli's Syndrome: CT Findings

Horia Marin, Jorge Chain, Rafik Sakri, Phillippe Nusbaum,Catherine Turnani

Abstract

Caroli's disease is a congenital disease characterized by a non-obstructive, multifocal segmental dilatation of intrahepatic bile ducts. It may appear isolated or associated to congenital hepatic fibrosis and various renal anomalies. We present two patients: one with Caroli's disease (a 80 year old female) and the other with Caroli's syndrome (a 45 year old male), illustrated with suggestive CT images. The CT findings included, for the first patient, diffuse cystic dilatation of intrahepatic biliary tree with the presence of enhanced portal radicals (central dot sign). The second patient presented saccular dilatation of biliary ducts of the 7th hepatic segment with intraluminal protrusion representing enhanced portal radicals, dysmorphic liver, signs of portal hypertension, nephrolithiasis on medullary sponge kidney. Enhanced computed tomography gives not only a strong orientation for the diagnosis of Caroli's disease (segmental dilatation of intrahepatic bile ducts with central dot sign) but also may show many associated anomalies (liver dysmorphy in congenital hepatic fibrosis, renal anomalies) proving to be a powerful diagnostic tool.

Key words

Computed tomography - Caroli's disease - Caroli's syndrome