Biliary Fibrosis and Portal Hypertension as a Rare Cause of Cholangiocarcinoma

  • Ammar Hassan
  • Simcha WeissmanEmail author
  • Mark Pinkhasov
  • Michael Sciarra
Case Report


Caroli’s syndrome (type 2) is a rare, complex form of Caroli’s disease (type 1) with an estimated incidence of 1/1,000,000. The syndrome manifests as saccular dilatations of the intra-hepatic biliary ducts and is associated with fibrosis and portal hypertension, distinguishing it from the disease form [1, 2]. Importantly, it predisposes patients to regionalized malignancy. Generally this presents before the age of 40. Intriguing however in our case was the late presentation of the disease. The aim of this case report is to educate clinicians as to an obscure presentation of this rare disease in an attempt to help prevent cirrhosis as well as the subsequent early detection of cholangiocarcinoma that is precipitated by this syndrome.

Case Report

A 78-year-old Asian female with a history of hypertension and cholelithiasis presented to our hospital with abdominal pain, jaundice, and fever. Magnetic resonance cholangiopancreatography (MRCP) revealed saccular intra-hepatic...


Author Contributions

SW, AH, and MP acquired and selected figures and drafted the manuscript; MS edited and critically revised the manuscript. MP and MS provided supervision and critical revision of the manuscript. SW and AH are the article guarantors.

This case has been presented at the ACG conference in San Antonio, Texas, on October 28, 2019.

Compliance with Ethical Standards

Conflicts of Interest

The authors declare that they have no conflict(s) of interest.

Informed consent was obtained for publication of the case details.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Gastroenterology and HepatologyHackensack University-Palisades Medical CenterNorth BergenUSA
  2. 2.Department of MedicineHackensack University-Palisades Medical CenterNorth BergenUSA

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