Ischemic cholangitis: Lethal complication of Osler-Weber-Rendu disease

Osler-Weber-Rendu disease (OWRD), also known as hereditary haemorrhagic telangiectasia (HHT), is an autosomal dominant genetic disorder characterised by arteriovenous malformations in several organs. Ischemic cholangitis is a rare life-threatening complication of OWRD, with only a few documented cas...

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Bibliographic Details
Published in:Radiology case reports 2024-09, Vol.19 (9), p.3810-3813
Main Authors: Elmqaddem, Ouiam, Koulali, Hajar, Zazour, Abdelkrim, Nasiri, Meryem, Ismaili, Moulay Zahi, Kharrasse, Ghizlane
Format: Article
Language:English
Subjects:
Bevacizumab
Bilomas
Epistaxis
Ischemic cholangitis
Liver transplantation
Rendu Osler Weber disease
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Summary:Osler-Weber-Rendu disease (OWRD), also known as hereditary haemorrhagic telangiectasia (HHT), is an autosomal dominant genetic disorder characterised by arteriovenous malformations in several organs. Ischemic cholangitis is a rare life-threatening complication of OWRD, with only a few documented cases in the literature. A liver transplant is the main curative treatment. In this paper, we report a case of a 33-year-old woman with a history of recurrent epistaxis, admitted with abdominal pain and fever, physical examination found multiple cutaneous and mucosal telangiectasias and the biological workup showed cholestasis, abdominal imaging identified arterio-venous shunts and multiple cystic hepatic lesions, one of them seemed to communicate with an intrahepatic biliary duct, finally the diagnosis of ischemic cholangitis due to OWRD was retained and antibiotic treatment has been initiated. We review the various therapeutic options available to improve the management of this fatal complication.
ISSN:1930-0433
1930-0433
DOI:10.1016/j.radcr.2024.04.033