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Caroli disease


Monday 8 November 2004

congenital polycystic dilatation of intrahepatic bile ducts. Ent. Nom. Caroli, 1958.

Definition: Caroli’s syndrome is characterized by multiple segmental cystic or saccular dilatations of intrahepatic bile ducts associated with congenital hepatic fibrosis (ductal plate malformation).

Caroli disease is a member ot fibropolycystic diseases of the hepatobiliary system characterized by segmental cystic dilatation of intrahepatic ducts.


- fem (40 yo), liver biopsy - dilated biliary ducts with plugs and fibrosis - Caroli Disease


- hepatic malformative cysts
- any part of intrahepatic biliary tree
- the cysts communicate with the rest of biliary tree

  • risk of bacterial ascending infections


- segmental cystic dilatation of intrahepatic ducts
- intrahepatic cholelithiasis
- cholangitis
- hepatic abscesses


- ductal plate malformations (congenital hepatic fibrosis )
-  renal cysts

  • renal tubular ectasia
  • medullary sponge kidney
  • adult-type polycystic kidney disease (ADPKD )

Differential diagnosis

- acquired cholangiectases - acquired cholangiectasis

- hepatic fibrocystic diseases

Familial forms

- in siblings (12468965, 1468609)


- Yonem O, Bayraktar Y. Clinical characteristics of Caroli’s syndrome.World J Gastroenterol. 2007 Apr 7;13(13):1934-7. PMID: 17461493