IMAGING FINDINGS IN CAROLIS-DISEASE

In 1958 Jacques Caroli described communicating cavernous ectasia of th e biliary tree as an uncommon cause of chronic, often life-threatening hepatobiliary disease. The disease now most often referred to as Caro li's disease is a rare condition characterized by nonobstructive saccu lar or fusiform dilatation of the intrahepatic bile ducts, In the so-c alled pure form, dilatation is classically segmental and saccular and is associated with stone formation and recurrent bacterial cholangitis . In the form associated with congenital hepatic fibrosis, bile duct d ilatation usually is less prominent; portal hypertension and eventual liver failure typically develop as a result of the hepatic fibrosis. C aroli's disease usually is manifested in childhood and is thought to b e congenital and probably inherited. Associated conditions include ren al cystic disease, choledochal cysts, and cholangiocarcinoma. This pic torial essay illustrates the broad spectrum of imaging findings in Car oli's disease.