Caroli disease with white pyramid sign
Updates to Case Attributes
This case representsThe association of multiple hepatic cystic lesions with portal vein branches suggests Caroli’s disease.
The white pyramid sign has a well recognized association between Caroli disease, medullarywide differential diagnosis. Medullary sponge kidney and medullary nephrocalcinosisis one possible cause that has an association with Caroli’s disease however the charcteristic appearances are not shown here.
-<p>This case represents a well recognized association between Caroli disease, medullary sponge kidney and medullary nephrocalcinosis.</p>- +<p>The association of multiple hepatic cystic lesions with portal vein branches suggests Caroli’s disease. </p><p>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.</p>
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Updates to Study Attributes
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 (branches of hepatic artery) giving(portal vein branches). When central, this is called the typical appearance of central dot sign.
Normal CBD.
The constellation of findings is highly suggestive of Caroli’s disease or Caroli’s syndrome with medullary sponge kidney.
Updates to Study Attributes
Central dot sign, Caroli syndromeEccentric portal vein branches at the periphery of the cysts.