CT
Non-contrast study shows dense renal medulla with multiple hepatic cystic lesions which appear to be related to biliary tree.
Post-contrast study shows the cystic lesion related to portal triad with eccentric or central enhancing vessels (portal vein branches). When central, this is called the central dot sign.
Normal CBD.
The constellation of findings is highly suggestive of Caroli’s disease or Caroli’s syndrome.
White pyramid sign.