Liver & intrahepatic bile ducts
Developmental anomalies / cysts
Caroli disease
Author: Komal Arora, M.D.
Last author update: 1 April 2012
Last staff update: 24 December 2024 (update in progress)
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Caroli disease liver
Table of Contents
Definition / general | Clinical features | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Differential diagnosisCite this page: Arora K. Caroli disease. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/livercarolisdisease.html. Accessed December 26th, 2024.
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
Images hosted on other servers:
Dilated bile ducts
Microscopic (histologic) description
- Dilated ducts lined by cuboidal or columnar epithelium with fibrotic duct wall
Microscopic (histologic) images
Images hosted on other servers:
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
