Caroli disease

Case contributed by Murtadah Raad Kareem
Diagnosis almost certain


The patient presented with recurrent attacks of jaundice associated with right upper quadrant abdominal pain.

Patient Data

Age: 8 years
Gender: Male

There are multiple saccular cystic lesions connected directly to dilated intrahepatic bile ducts representing focal dilatation of intrahepatic bile ducts, there is no dilatation of the extrahepatic bile system (CBD).

An associated finding of bilateral duplicated renal collecting systems.

Case Discussion

Multifocal cystic dilatation of intrahepatic bile ducts with normal CBD is consistent with the diagnosis of Caroli disease.

An incidental finding of bilateral duplicated collecting system. No upper tract dilatation.

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