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OBJECTIVE. The purpose of our study was to describe the spectrum of radiologic and pathologic features of Caroli's disease.CONCLUSION. Caroli's disease and its complications have overlapping radiologic appearances that reflect the underlying pathology of fibrosis, ductal dilatation, cholangitis, stone formation, and malignancy.

In 1958 Jacques Caroli described communicating cavernous ectasia of the biliary tree as an uncommon cause of chronic, often life-threatening hepatobiliary disease. The disease now most often referred to as Caroli's disease is a rare condition characterized by nonobstructive saccular or fusiform dilatation of the intrahepatic bile ducts. In the so-called 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 dilatation usually is less prominent; portal hypertension and eventual liver failure typically develop as a result of the hepatic fibrosis. Caroli's disease usually is manifested in childhood and is thought to be congenital and probably inherited. Associated conditions include renal cystic disease, choledochal cysts, and cholangiocarcinoma. This pictorial essay illustrates the broad spectrum of imaging findings in Caroli's disease.

Intrahepatic calculi in non-Asian patients were studied by sonography and computed tomography (CT). Three patients were studied by CT cholangiography also. In two cases, the calculi were consecutive to Caroli disease, and in two others, the biliary stones were formed proximal to a stenosis of a previous surgical anastomosis. Five patients spontaneously developed intrahepatic calculi. All sonograms were abnormal. Image specificity was good, even when bile ducts were not dilated, if appropriate technique allowed identification of a double-arc-shadow pattern. Sonography strongly suggested the diagnosis in eight patients and was nonspecific in only one. On CT, calculi had various densities, and they were not visible in two patients. CT cholangiography was not particularly helpful. Finally, CT added little more information when performed after sonography. Both examinations strongly underestimate the number of stones, and direct cholangiography remains indicated if surgery is planned.

Caroli’s disease is characterized by segmental saccular dilatation of the intrahepatic bile ducts. Bile stasis in the dilated segments predisposes to stone formation and cholangitis, giving the disease a serious prognosis.The clinical features, laboratory findings and differential diagnosis of this disease are discussed.A new case of Caroli’s disease is reported.