[Upper abdominal pain with recurrent cholangitis and pyelonephritis]

A 38-year-old man presented with pain in the right upper abdomen combined with elevated C-reactive protein (5.37 mg/dl) and creatinine levels (2.0 mg/dl). The medical history revealed recurrent abdominal pain. Duplex sonography showed large intrahepatic cystic structures, partially combined with a ribbon-shaped flow signal inside, as well as multiple cysts in the kidneys. The following MRI identified these findings as dilatated bile ducts surrounding the portal vein branches. This is known as the central dot sign. Overall, this special constellation is indicative of Caroli's disease, a rare congenital cystic dilatation of the intrahepatic bile ducts. The imaging results substantiated the diagnosis, especially in combination with cystic renal disease and recurrent cholangitis. Depending on the grade of liver involvement the treatment is partial hepatectomy or, as in our case transplantation, which also eliminates the risk of associated cholangiocarcinoma.