Liver Transplantation Consequential to Caroli's Syndrome: A Case Report

Abstract Caroli's disease is a rare condition that includes fibrocystic malformations of the bile duct. It consists of multifocal congenital dilatations of the intrahepatic bile ducts, which may be diffuse or limited, presenting in sack form that produces cystic structures which communicate wit...

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Bibliographic Details
Published in:	Transplantation proceedings Vol. 40; no. 9; pp. 3121 - 3122
Main Authors:	Tallón Aguilar, L, Sánchez Moreno, L, Barrera Pulido, L, Pareja Ciuró, F, Suárez Artacho, G, Álamo Matinez, J.M, Bernal Bellido, C, Garía González, I, Serrano Díaz-Canedo, J, Gómez Bravo, M.A, Bernardos Rodríguez, A
Format:	Journal Article Conference Proceeding
Language:	English
Published:	Amsterdam Elsevier Inc 01-11-2008 Elsevier
Subjects:	Adult Biological and medical sciences Caroli Disease - surgery Female Fundamental and applied biological sciences. Psychology Fundamental immunology Hepatectomy Humans Liver - pathology Liver Transplantation Liver, biliary tract, pancreas, portal circulation, spleen Medical sciences Surgery Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases Surgery of the digestive system Tissue, organ and graft immunology Treatment Outcome Case study Human Medicine Treatment Digestive system Surgery Liver Graft Homotransplantation Syndrome Liver transplantation
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Summary:	Abstract Caroli's disease is a rare condition that includes fibrocystic malformations of the bile duct. It consists of multifocal congenital dilatations of the intrahepatic bile ducts, which may be diffuse or limited, presenting in sack form that produces cystic structures which communicate with the biliary tree. Herein we have presented the case of a 44-year-old woman with recurrent cholangitis consequential to Caroli's syndrome. The distinctive feature of this case was that it was the first and only liver transplantation performed to date for this cause at our center among 700 procedures that had been performed over 19 years. The hepatectomy sample from the liver transplantation showed large cystic dilatations at the level of segments VII and VIII. The pathological study reported congenital dilatation of the intrahepatic bile ducts, associated with congenital hepatic fibrosis (Caroli's syndrome). Caroli's syndrome is a complex association of conditions which usually presents together with polycystic kidney lesions. Orthotopic liver transplantation is still the only therapeutic option for diffuse, uncontrollable cases or those with significant portal hypertension, as well as being the final option in the other cases in the event of a lack of response to other therapeutic options or as an alternative to them.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0041-1345 1873-2623
DOI:	10.1016/j.transproceed.2008.08.104