Liver & intrahepatic bile ducts

Developmental anomalies / cysts

Caroli disease

Last author update: 1 April 2012
Last staff update: 15 July 2022

Copyright: 2002-2023,, Inc.

PubMed Search: Caroli disease liver

Komal Arora, M.D.
Page views in 2022: 2,617
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Cite this page: Arora K. Caroli disease. website. Accessed June 2nd, 2023.
Definition / general
  • Also called communicating cavernous biliary ectasia
  • Autosomal recessive disorder, mildly associated with autosomal dominant and autosomal recessive polycystic kidney disease (Wikipedia: Caroli Disease [Accessed 25 October 2017])
  • Due to arrest of remodeling of ductal plate of larger intrahepatic bile ducts
  • Usually presents during childhood and early adulthood (Curr Gastroenterol Rep 2007;9:151)
  • Larger ducts of intrahepatic biliary tree are segmentally dilated and may contain inspissated bile; when progressive, leads to intrahepatic stones, recurrent cholangitis, portal hypertension, cholangiocarcinoma and liver failure
  • Usually associated with congenital hepatic fibrosis; 7 - 14% develop dysplasia and cholangiocarcinoma
  • Often gallstones, ulcer, hyperplasia
  • Poor prognosis, with death due to sepsis or liver failure
  • Caroli syndrome: Caroli disease plus congenital hepatic fibrosis
Clinical features
  • Jaundice, right upper abdominal pain, fever; due to associated complications of hepatolithiasis or bacterial cholangitis
Gross description
  • 1 - 4 cm cysts separated by normal bile ducts
Gross images

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Dilated bile ducts

Microscopic (histologic) description
  • Dilated ducts lined by cuboidal or columnar epithelium with fibrotic duct wall
Microscopic (histologic) images

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Dilated portal bile ducts (trichrome stain), with inspissated bile, with congenital hepatic fibrosis

Differential diagnosis
  • Primary sclerosing cholangitis:
    • Autoimmune disorder associated with inflammatory bowel disease; not inherited; fibrosing cholangitis with onion skinning around affected ducts
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