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Liver transplantation consequential to Caroli's syndrome: a case report.
Transplant Proc. 2008 Nov; 40(9):3121-2.TP

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.

Authors+Show Affiliations

Liver Transplant and Hepatobiliopancreatic Surgery Unit, General and Gastrointestinal Surgery Department, Hospitales Universitarios Virgen del Rocío, Seville, Spain. Itallona@hotmail.comNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

19010212

Citation

Tallón Aguilar, L, et al. "Liver Transplantation Consequential to Caroli's Syndrome: a Case Report." Transplantation Proceedings, vol. 40, no. 9, 2008, pp. 3121-2.
Tallón Aguilar L, Sánchez Moreno L, Barrera Pulido L, et al. Liver transplantation consequential to Caroli's syndrome: a case report. Transplant Proc. 2008;40(9):3121-2.
Tallón Aguilar, L., Sánchez Moreno, L., Barrera Pulido, L., Pareja Ciuró, F., Suárez Artacho, G., Alamo Matinez, J. M., Bernal Bellido, C., Garía González, I., Serrano Díaz-Canedo, J., Gómez Bravo, M. A., & Bernardos Rodríguez, A. (2008). Liver transplantation consequential to Caroli's syndrome: a case report. Transplantation Proceedings, 40(9), 3121-2. https://doi.org/10.1016/j.transproceed.2008.08.104
Tallón Aguilar L, et al. Liver Transplantation Consequential to Caroli's Syndrome: a Case Report. Transplant Proc. 2008;40(9):3121-2. PubMed PMID: 19010212.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Liver transplantation consequential to Caroli's syndrome: a case report. AU - Tallón Aguilar,L, AU - Sánchez Moreno,L, AU - Barrera Pulido,L, AU - Pareja Ciuró,F, AU - Suárez Artacho,G, AU - Alamo Matinez,J M, AU - Bernal Bellido,C, AU - Garía González,I, AU - Serrano Díaz-Canedo,J, AU - Gómez Bravo,M A, AU - Bernardos Rodríguez,A, PY - 2008/11/18/pubmed PY - 2009/6/24/medline PY - 2008/11/18/entrez SP - 3121 EP - 2 JF - Transplantation proceedings JO - Transplant Proc VL - 40 IS - 9 N2 - 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. SN - 0041-1345 UR - https://www.unboundmedicine.com/medline/citation/19010212/Liver_transplantation_consequential_to_Caroli's_syndrome:_a_case_report_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0041-1345(08)01182-2 DB - PRIME DP - Unbound Medicine ER -