01 October 2003
Caroli’s disease: case report and review of the literature
Przemysław Besser, Viola Bacia, Włodzimierz Mazur, Zbigniew GonciarzCase 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
42
In Press
12 Feb 2024 : Case report
Erdheim-Chester Disease Occult on Radiographs and CT but Visible on MRI and PETAm J Case Rep In Press; DOI: 10.12659/AJCR.941169
12 Feb 2024 : Case report
Surgical Treatment of Spontaneous Superficial Temporal Artery Arteriovenous Malformation: A Case ReportAm J Case Rep In Press; DOI: 10.12659/AJCR.942839
13 Feb 2024 : Case report
Warfarin Woes: A Rare Case of Hemoperitoneum with Intramural Small Bowel HematomaAm J Case Rep In Press; DOI: 10.12659/AJCR.943519
13 Feb 2024 : Case report
Hemorrhagic Presentation in Primary Central Nervous System Lymphoma: A Case StudyAm J Case Rep In Press; DOI: 10.12659/AJCR.942951
Most Viewed Current Articles
07 Mar 2024 : Case report
Neurocysticercosis Presenting as Migraine in the United StatesDOI :10.12659/AJCR.943133
Am J Case Rep 2024; 25:e943133
10 Jan 2022 : Case report
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
Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19DOI :10.12659/AJCR.936128
Am J Case Rep 2022; 23:e936128
23 Feb 2022 : Case report
Penile Necrosis Associated with Local Intravenous Injection of CocaineDOI :10.12659/AJCR.935250
Am J Case Rep 2022; 23:e935250