Thursday, September 20, 2012

Caroli's Disease and Syndrome

Caroli's Disease
  • autosomal recessive abnormality in ductal plate development
  • communicating cavernous biliary ectasia 
  • US will show:
    • dilated intrahepatic bile ducts that are saccular or fusiform
    • outpouchings may contain sludge/calculi due to biliary stasis 
    • central dot sign - color Doppler shows flow in the portal triads surrounded by the dilated ducts
  • ERCP/MRCP will show dilated ducts that communicate with the central biliary tree
Caroli's syndrome is Caroli's disease + congenital hepatic fibrosis.

Levy AD, Rohrmann CA, Murakata LA, et al. Caroli's disease: radiologic spectrum with pathologic correlation. AJR Am J Roentgenol 2002;179:1053-7.

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