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Caroli disease
MIM.600643
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.
Images
fem (40 yo), liver biopsy - dilated biliary ducts with plugs and fibrosis - Caroli Disease
Synopsis
hepatic malformative cysts
any part of intrahepatic biliary tree
the cysts communicate with the rest of biliary tree
- risk of bacterial ascending infections
Synopsis
segmental cystic dilatation of intrahepatic ducts
intrahepatic cholelithiasis
cholangitis
hepatic abscesses
Associations
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
- primary sclerosing cholangitis
Familial forms
in siblings (12468965, 1468609)
References
Yonem O, Bayraktar Y. Clinical characteristics of Caroli’s syndrome.World J Gastroenterol. 2007 Apr 7;13(13):1934-7. PMID: 17461493
