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Liver and intrahepatic bile ducts-nontumor

Developmental anomalies/cysts

Caroli's disease

Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 9 April 2012, last major update April 2012-IN PROGRESS
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.


● Also called communicating cavernous biliary ectasia
● Autosomal recessive disorder, mildly associated with autosomal dominant and autosomal recessive polycystic kidney disease (Wikipedia)
● 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
Caroliís syndrome: Caroliís 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

Dilated bile ducts

Micro description

● Dilated ducts lined by cuboidal or columnar epithelium with fibrotic duct wall

Micro images

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

End of Liver and intrahepatic bile ducts-nontumor > Developmental anomalies/cysts > Caroli's disease

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