Caroli's disease classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Classification

  • Two subtypes of Caroli's disease have been described:
  • Simple type
  • As classically described, without cirrhosis or portal HTN
  • 60-80% associated with medullary sponge kidney
  • Periportal fibrosis type
  • Also associated with:
  • Hepatic function is preserved in most, but many patients have recurrent cholangitis, liver abscesses and portal HTN.
  • The cysts are nonobstructive saccular or fusiform dilatations of the intrahepatic ducts.
  • The disease may be unilobar or bilobar. Unilobar disease is most commonly localized to the left lobe of the liver.
  • Some reviews have reported that pure Caroli’s disease is not that common, and more patients actually have Type IVA disease, with both intra- and extra-hepatic cysts.
  • Intrahepatic biliary cystic disease should be differentiated from polycystic liver disease, an autosomal dominant disorder characterized by cystic collections of serous fluid not in communication with the biliary tree.
  • The incidence of malignant transformation in intrahepatic cysts is ~7% in Caroli’s disease.

References