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Endoscopic therapy for gastro-oesophageal varices of Caroli’s syndrome: a case report

Caroli’s disease (CD) is a very rare congenital disorder that is characterized by non-obstructive, segmental and cystic dilatation of intrahepatic ducts. Most patients with CD are asymptomatic, but some patients may suffer from hepatic fibrosis, liver cirrhosis or/and portal hypertension. In complex...

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Autores principales: Wang, Song, Xiao, Mei, Hua, Liqun, Jia, Yong, Chen, Si, Zhang, Kaiguang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7607268/
https://www.ncbi.nlm.nih.gov/pubmed/31594447
http://dx.doi.org/10.1177/0300060519877993
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author Wang, Song
Xiao, Mei
Hua, Liqun
Jia, Yong
Chen, Si
Zhang, Kaiguang
author_facet Wang, Song
Xiao, Mei
Hua, Liqun
Jia, Yong
Chen, Si
Zhang, Kaiguang
author_sort Wang, Song
collection PubMed
description Caroli’s disease (CD) is a very rare congenital disorder that is characterized by non-obstructive, segmental and cystic dilatation of intrahepatic ducts. Most patients with CD are asymptomatic, but some patients may suffer from hepatic fibrosis, liver cirrhosis or/and portal hypertension. In complex CD, cystic dilatations of the intrahepatic bile ducts can be present with congenital hepatic fibrosis, liver cirrhosis, portal hypertension, oesophageal varices and autosomal recessive polycystic kidney disease; a condition known as Caroli’s syndrome. This report describes the case of a 28-year-old woman that had gastro-oesophageal varices that were caused by hepatic fibrosis and portal hypertension as part of Caroli’s syndrome. The patient underwent successful treatment with endoscopic injection sclerotherapy with lauromacrogol and endoscopic variceal obturation using tissue adhesive. There were no immediate complications and the patient remained free of complications at 1-year follow-up. There are no current reports in the published literature describing Caroli’s syndrome induced by gastro-oesophageal varices that were treated by a combination of endoscopic injection sclerotherapy and endoscopic variceal obturation. Endoscopic therapy was an effective technique for the treatment of gastro-oesophageal varices in a patient with Caroli’s syndrome awaiting a liver transplant.
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spelling pubmed-76072682020-11-13 Endoscopic therapy for gastro-oesophageal varices of Caroli’s syndrome: a case report Wang, Song Xiao, Mei Hua, Liqun Jia, Yong Chen, Si Zhang, Kaiguang J Int Med Res Special Issue: Rare Diseases: Advances in Diagnosis, Prevention, Treatment and Management Caroli’s disease (CD) is a very rare congenital disorder that is characterized by non-obstructive, segmental and cystic dilatation of intrahepatic ducts. Most patients with CD are asymptomatic, but some patients may suffer from hepatic fibrosis, liver cirrhosis or/and portal hypertension. In complex CD, cystic dilatations of the intrahepatic bile ducts can be present with congenital hepatic fibrosis, liver cirrhosis, portal hypertension, oesophageal varices and autosomal recessive polycystic kidney disease; a condition known as Caroli’s syndrome. This report describes the case of a 28-year-old woman that had gastro-oesophageal varices that were caused by hepatic fibrosis and portal hypertension as part of Caroli’s syndrome. The patient underwent successful treatment with endoscopic injection sclerotherapy with lauromacrogol and endoscopic variceal obturation using tissue adhesive. There were no immediate complications and the patient remained free of complications at 1-year follow-up. There are no current reports in the published literature describing Caroli’s syndrome induced by gastro-oesophageal varices that were treated by a combination of endoscopic injection sclerotherapy and endoscopic variceal obturation. Endoscopic therapy was an effective technique for the treatment of gastro-oesophageal varices in a patient with Caroli’s syndrome awaiting a liver transplant. SAGE Publications 2019-10-08 /pmc/articles/PMC7607268/ /pubmed/31594447 http://dx.doi.org/10.1177/0300060519877993 Text en © The Author(s) 2019 https://creativecommons.org/licenses/by-nc/4.0/ Creative Commons Non Commercial CC BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Special Issue: Rare Diseases: Advances in Diagnosis, Prevention, Treatment and Management
Wang, Song
Xiao, Mei
Hua, Liqun
Jia, Yong
Chen, Si
Zhang, Kaiguang
Endoscopic therapy for gastro-oesophageal varices of Caroli’s syndrome: a case report
title Endoscopic therapy for gastro-oesophageal varices of Caroli’s syndrome: a case report
title_full Endoscopic therapy for gastro-oesophageal varices of Caroli’s syndrome: a case report
title_fullStr Endoscopic therapy for gastro-oesophageal varices of Caroli’s syndrome: a case report
title_full_unstemmed Endoscopic therapy for gastro-oesophageal varices of Caroli’s syndrome: a case report
title_short Endoscopic therapy for gastro-oesophageal varices of Caroli’s syndrome: a case report
title_sort endoscopic therapy for gastro-oesophageal varices of caroli’s syndrome: a case report
topic Special Issue: Rare Diseases: Advances in Diagnosis, Prevention, Treatment and Management
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7607268/
https://www.ncbi.nlm.nih.gov/pubmed/31594447
http://dx.doi.org/10.1177/0300060519877993
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