Caroli disease
MIM.600643
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congenital polycystic dilatation of intrahepatic bile ducts. Ent. Nom. Caroli, 1958.
Caroli disease is a member ot fibropolycystic diseases of the hepatobiliary system characterized by segmental cystic dilatation of intrahepatic ducts.
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
- primary sclerosing cholangitis
heaptic fibrocystic diseases