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:
- Congenital hepatic fibrosis – bland portal fibrosis, hyperproliferation of interlobular bile ducts within the portal areas, with variable shapes and size of bile ducts, and preservation of normal lobular architecture.
- Cirrhosis
- Portal hypertension (HTN)
- Esophageal varices
- Renal cystic lesions also often occur
- 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.