Caroli disease with white pyramid sign

Case contributed by Ahmed A. Khawwam


Abdominal pain

Patient Data

Age: 16 years
Gender: Female

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.

Annotated image

Eccentric portal vein branches at the periphery of the cysts.

Case Discussion

The association of multiple hepatic cystic lesions with portal vein branches suggests Caroli’s disease.

The white pyramid sign has a wide differential diagnosis. Medullary sponge kidney is one possible cause that has an association with Caroli’s disease however the charcteristic appearances are not shown here.

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