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World J Gastroenterol. Apr 7, 2007; 13(13): 1930-1933
Published online Apr 7, 2007. doi: 10.3748/wjg.v13.i13.1930
Clinical characteristics of Caroli’s disease
Ozlem Yonem, Yusuf Bayraktar
Ozlem Yonem, Yusuf Bayraktar, Hacettepe University Faculty of Medicine, Department of Gastroenterology, Sihhiye 06100, Ankara, Turkey
Author contributions: All authors contributed equally to the work.
Correspondence to: Yusuf Bayraktar, MD, Hacettepe University Faculty of Medicine, Department of Gastroenterology, Sihhiye 06100, Ankara, Turkey. bayrak@hacettepe.edu.tr
Telephone: +90-312-4439428 Fax: +90-312-4429429
Received: October 11, 2006
Revised: December 3, 2006
Accepted: December 20, 2006
Published online: April 7, 2007
Abstract

Caroli’s disease is a rare congenital condition chara-cterized by non-obstructive saccular or fusiform dilatation of larger intrahepatic bile ducts. Cholangitis, liver cirrhosis, and cholangiocarcinoma are its potential complications. The diagnosis of Caroli’s disease depends on demonstrating that the cystic lesions are in continuity with the biliary tree which can be showed by ultrasonography, computerized tomography, endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography or magnetic resonance cholangiopancreatography. Treatment of Caroli’s disease relies on the location of the biliary abnormalities. While localized forms confined to one lobe can be treated with surgery, liver transplantation is the only effective modality for diffuse forms. Although a rare disorder; Caroli’s disease should always be considered in the differential diagnosis of chronic cholestasis of unknown cause.

Keywords: Caroli’s disease, Liver transplantation, Endoscopic retrograde cholangiopancreatography