A CASE OF CAROLI'S DISEASE ASSOCIATED WITH CHOLANGITIS, HEPATOLITHIASIS, AND POLYCYSTIC KIDNEY DISEASE : USEFULNESS OF THE MAGNETIC RESONANCE CHOLANGIOPANCREATOGRAPHY

Abstract

Caroli's disease is a rare congenital condition characterized by cystic dilatation of the intrahepatic bile ducts. A 74-year-old man with chronic renal failure complicated by polycystic kidney disease presented with jaundice and fever. Ultrasonography, non-contrast computed tomography, and magnetic resonance cholangiopancreatography (MRCP) were performed. Contrast medium was not administered because of the renal failure due to polycystic kidney disease. MRCP provided cholangiographic images of the biliary system. No hepatic fibrosis was observed on liver biopsied specimens. Based･ on the cystic dilatation of the intrahepatic bile ducts, stone formation, cholangitis, absence of hepatic cirrhosis, and association with cystic of the kidneys, a diagnosis Caroli's disease was made.