Caroli's disease classification: Difference between revisions
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==Classification== | ==Classification== | ||
* Two subtypes of [[Caroli's disease]] have been described: | * Two subtypes of [[Caroli's disease]] have been described: | ||
:* Simple type | :* '''Simple type''' | ||
::* As classically described, without [[cirrhosis]] or [[portal hypertension]] | ::* As classically described, without [[cirrhosis]] or [[portal hypertension]] | ||
::* 60-80% associated with medullary sponge [[kidney]] | ::* 60-80% associated with medullary sponge [[kidney]] | ||
:* [[Periportal]] [[fibrosis]] type | ''':* [[Periportal]] [[fibrosis]] type''' | ||
::* Also associated with: | ::* 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. | :::* [[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. | ||
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* The [[cysts]] are nonobstructive saccular or fusiform dilatations of the [[intrahepatic]] ducts. | * The [[cysts]] are nonobstructive saccular or fusiform dilatations of the [[intrahepatic]] ducts. | ||
* The disease may be unilobed or bilobed. Unilobed disease is most commonly localized to the left lobe of the [[liver]]. | * The disease may be unilobed or bilobed. Unilobed 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. | * 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]]. | * 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. | * The incidence of [[malignant]] transformation in intrahepatic cysts is ~7% in Caroli’s disease. | ||
==References== | ==References== | ||
Revision as of 20:03, 11 March 2022
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Rinky Agnes Botleroo, M.B.B.S.
Classification
- Two subtypes of Caroli's disease have been described:
- Simple type
- As classically described, without cirrhosis or portal hypertension
- 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 hypertension.
- The cysts are nonobstructive saccular or fusiform dilatations of the intrahepatic ducts.
- The disease may be unilobed or bilobed. Unilobed 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.