Caroli's Syndrome: A Rare Disorder

A forty eight year old male presented with recurrent pain in right hypochondrium and fever with chill's and rigors but without icterus and pruritus. His liver function tests were normal. Magnetic resonance cholangiopancreatography revealed focal cystic dilatation of intrahepatic biliary radicles affecting all the segments of liver. The liver biopsy showed dilated bile duct with accompanying hepatic fibrosis. He also had multiple cysts in both kidneys, so a diagnosis of Caroli's syndrome was made. Caroli's disease is a rare congenital condition characterized by non-obstructive saccular or fusiform dilatation of larger intrahepatic bile duct. When it is associated with renal cystic lesions and periportal fibrosis it is called Caroli's Syndrome. Magnetic resonance cholangio-pancreatography (MRCP) is a noninvasive technique that has shown good correlation with ERCPin diagnosing biliary tract abnormality.