Ischemic cholangitis: Lethal complication of Osler-Weber-Rendu disease
| Autor: | Ouiam Elmqaddem, Hajar Koulali, Abdelkrim Zazour, Meryem Nasiri, Moulay Zahi Ismaili, Ghizlane Kharrasse |
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| Jazyk: | angličtina |
| Rok vydání: | 2024 |
| Předmět: | |
| Zdroj: | Radiology Case Reports, Vol 19, Iss 9, Pp 3810-3813 (2024) |
| Druh dokumentu: | article |
| ISSN: | 1930-0433 34487999 |
| DOI: | 10.1016/j.radcr.2024.04.033 |
| Popis: | 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. |
| Databáze: | Directory of Open Access Journals |
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