Table of Contents    
Case Report
 
Liver pathology findings in infant with Caroli's syndrome
Blagica Dukova1, Boro Ilievski2, Snezana Duganovska3, Vladimir Chadikovski4 Aco Kostovski5
1Junior assistant, Pathologist, Institute of pathology, Medical faculty, Skopje, Republic of Macedonia.
2Pathologist, Institute of pathology, Medical faculty, Skopje, Republic of Macedonia.
3Professor, Pathologist, Institute of pathology, Medical faculty, Skopje, Republic of Macedonia.
4Pediatric surgeon, University clinic of pediatric surgery, Skopje, Republic of Macedonia.
5Professor, Pediatrician, Medical Director of University Children's Hospital, Chief of Department of Pediatric Gastroenterology, University Children's Hospital, Skopje, Republic of Macedonia.

Article ID: 100004IJHPDBD2012
doi:10.5348/ijhpd-2011-4-CR-2

Address correspondence to:
Blagica Dukova
Shumadinska 5/18
1000 Skopje
Republic of Macedonia
Europe
Phone: +38970678209
Fax: + 389 2 3226 948
Email: blagicadr@yahoo.com

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How to cite this article:
Dukova B, Ilievski B, Duganovska S, Chadikovski V, Kostovski A. Liver pathology findings in infant with caroli's syndrome. International Journal of Hepatobiliary and Pancreatic Diseases 2012;2:4-8.


Abstract
Introduction: Caroli's syndrome (CS) is a rare congenital disorder characterized by intrahepatic bile duct dilatation and congenital hepatic fibrosis. The clinical features of this condition include signs of portal hypertension, cholangitis and lithiasis. Liver transplantation is the ultimate treatment in most patients with liver failure.
Case Report: A three month old infant treated with the diagnosis of biliary atresia, after two liver biopsies presented with distended abdomen, hepatosplenomegaly and signs of portal hypertension. Liver transplantation was preformed after four months. We found ectatic hilar bile ducts and intrahepatic bile duct dilatation. The pathologic finding of congenital hepatic fibrosis and proliferated dilated bile ducts suggested the diagnosis of Caroli's syndrome.
Conclusion: Caroli's disease and Caroli's syndrome may represent single disorder distinguished by congenital hepatic fibrosis. Fibrosis itself leads to portal hypertension appearing late in patients with Caroli's disease while it's dynamic and progressive in CS. Elevated white blood cell count is due to recurrent cholangitis, cholestasis and hepatolithiasis. Caroli's disease can be associated with extrahepatic bile duct dilatation, but the exact incidence is not known. CS often is associated with kidney lesions and cardiac disease. Liver transplantation should be preformed early. Symptoms are presented early in life due to congenital and progressive hepatic fibrosis. Caroli's syndrome must be considered in differential diagnosis in neonates with jaundice, ascites and hepatosplenomegaly. The first child with liver transplantation in Republic of Macedonia was diagnosed as Caroli's syndrome.

Key Words: Caroli's syndrome, Caroli's disease, Congenital hepatic fibrosis, Intrahepatic bile duct dilatation

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Author Contributions:
Blagica Dukova - Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Final approval of the version to be published
Boro Ilievski - Acquisition of data, Critical revision of the article, Final approval of the version to be published
Snezana Duganovska - Acquisition of data, Critical revision of the article, Final approval of the version to be published
Vladimir Chadikovski - Acquisition of data, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published
Aco Kostovski - Acquisition of data, Analysis and interpretation of data, Critical revision of the article, Final approval of the version to be published
Guarantor of submission:
The corresponding author is the guarantor of Submission.
Source of support:
None
Conflict of interest:
The authors declare no conflict of interest.
Copyright:
© Blagica Dukova et al. 2012; This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any means provided the original authors and original publisher are properly credited. (Please see Copyright Policy for more information.)