Logo American Journal of Case Reports

Call: 1.631.629.4328
Mon-Fri 10 am - 2 pm EST

Contact Us

Logo American Journal of Case Reports Logo American Journal of Case Reports Logo American Journal of Case Reports

01 October 2003

Caroli’s disease: case report and review of the literature

Przemysław Besser, Viola Bacia, Włodzimierz Mazur, Zbigniew Gonciarz

Case Rep Clin Pract Rev 2003; 4(3):176-181 :: ID: 450616

Abstract

Background: Caroli’s disease is a malformation characterized by nonobstructive saccular or fusiform dilatations of the intrahepatic bile ducts affecting the whole or parts of the liver and is associated with stone formation and recurrent bacterial cholangitis. The presence of only about 200 cases of Caroli’s disease up today may reflects less the rarity
of the condition than the difficulty of differentiating it from other developmental abnormalities of the liver.
Case report: The case of Caroli’s disease in a young boy with bilobular dilatation of the intrahepatic bile ducts and with portal hypertension is reported. In this case portal hypertension and liver failure typically developed as a result of the congenital hepatic fibrosis and bile duct dilatation and it was less prominent. The diagnostic evaluation
and treatment possibilities of this disease is discussed on the basis of case and review of the literature.
Conclusions: The diagnosis of Caroli’s disease is difficult especially, that it remains usually asymptomatic even whole life. The clinical manifestations have usually been nonspecific; the nature of the clinical presentation depends on whether intrahepatic biliary ductal ectasia or hepatic fibrosis is the dominant feature. If the fibrosis is predominant then the clinical presentations relate to portal hypertension, and hematemesis from varices can be first significant symptom, how was it in our case.

Keywords: Caroli’s disease, congenital hepatic cirrhosis, cysts of biliary tract

Add Comment 0 Comments

42

In Press

12 Feb 2024 : Case report  USA

Erdheim-Chester Disease Occult on Radiographs and CT but Visible on MRI and PET

Am J Case Rep In Press; DOI: 10.12659/AJCR.941169  

0:00

12 Feb 2024 : Case report  USA

Surgical Treatment of Spontaneous Superficial Temporal Artery Arteriovenous Malformation: A Case Report

Am J Case Rep In Press; DOI: 10.12659/AJCR.942839  

13 Feb 2024 : Case report  Bahrain

Warfarin Woes: A Rare Case of Hemoperitoneum with Intramural Small Bowel Hematoma

Am J Case Rep In Press; DOI: 10.12659/AJCR.943519  

13 Feb 2024 : Case report  Portugal

Hemorrhagic Presentation in Primary Central Nervous System Lymphoma: A Case Study

Am J Case Rep In Press; DOI: 10.12659/AJCR.942951  

Most Viewed Current Articles

07 Mar 2024 : Case report  USA

Neurocysticercosis Presenting as Migraine in the United States

DOI :10.12659/AJCR.943133

Am J Case Rep 2024; 25:e943133

0:00

10 Jan 2022 : Case report  Germany

A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...

DOI :10.12659/AJCR.935263

Am J Case Rep 2022; 23:e935263

19 Jul 2022 : Case report  Saudi Arabia

Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19

DOI :10.12659/AJCR.936128

Am J Case Rep 2022; 23:e936128

23 Feb 2022 : Case report  USA

Penile Necrosis Associated with Local Intravenous Injection of Cocaine

DOI :10.12659/AJCR.935250

Am J Case Rep 2022; 23:e935250

Your Privacy

We use cookies to ensure the functionality of our website, to personalize content and advertising, to provide social media features, and to analyze our traffic. If you allow us to do so, we also inform our social media, advertising and analysis partners about your use of our website, You can decise for yourself which categories you you want to deny or allow. Please note that based on your settings not all functionalities of the site are available. View our privacy policy.

American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923