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Caroli’s disease is a rare congenital condition characterized by sacular or fusiform dilatation of the intrahepatic bile ducts and should be considered in the differential diagnosis of chronic cholestasis of unknown cause. Most patients present before 30 years of age and its estimated incidence is 1 in 1.000,000 population. Imaging studies are important for the diagnosis of Caroli’s disease and its complications (cholangitis, liver cirrhosis and cholangiocarcinoma). Ursodeoxycholic acid (UDCA) is used to treat and prevent episodes of hepatolithiasis and cholangitis. Orthotopic liver transplantation is the best therapeutic option for diffuse forms of Caroli’s disease.

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2010-05-31   |   769 visitas   |   Evalua este artículo 0 valoraciones

Vol. 9 Núm.2. Abril-Junio 2010 Pags. 192-193 Ann Hepatol 2010; 9(2)