Ischemic cholangitis during Osler-Weber-Rendu disease: a case report
Abstract Background Osler-Weber-Rendu disease (OWRD) is a rare autosomal dominant genetic disease that causes skin and mucosal telangiectasias and visceral arteriovenous malformations. These shunts can lead to high-output heart failure, portal hypertension, biliary or mesenteric ischemia. We report...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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SpringerOpen
2022-01-01
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| Series: | Egyptian Liver Journal |
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| Online Access: | https://doi.org/10.1186/s43066-021-00163-y |
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| author | Oussama Kharmach Mohamed Borahma Nawal Lagdali Imane Benelbarhdadi Fatima-Zohra Ajana |
| author_facet | Oussama Kharmach Mohamed Borahma Nawal Lagdali Imane Benelbarhdadi Fatima-Zohra Ajana |
| author_sort | Oussama Kharmach |
| collection | DOAJ |
| description | Abstract Background Osler-Weber-Rendu disease (OWRD) is a rare autosomal dominant genetic disease that causes skin and mucosal telangiectasias and visceral arteriovenous malformations. These shunts can lead to high-output heart failure, portal hypertension, biliary or mesenteric ischemia. We report the case of a patient with OWRD complicated by ischemic cholangitis and biloma. Case presentation A 50-year-old female patient with personal history of spontaneous epistaxis and gingival bleeding, family history of Osler-Weber-Rendu disease (OWRD) in mother, and epistaxis in siblings. She consulted for intermittent and minimal biliary colic. Physical exam found mucocutaneous telangiectasia. Abdominal Doppler ultrasound/CT angiography showed arteriovenous shunts in the liver between hepatic artery and hepatic vein, hepatic artery and portal vein. Other sites of arteriovenous malformations were found in the nostril and jejunum. The diagnosis of OWRD was made. The evolution after 1 year was marked by the appearance of a slight anicteric cholestasis. MRCP found intrahepatic bilomas with associated ischemic cholangitis of segment VII. We decided to continue monitoring only. Conclusion The management of OWRD is based on screening for visceral arteriovenous malformations and on symptomatic measures that are often disappointing. Ischemic cholangitis is a rare complication, the diagnosis is based on imaging, liver transplantation is the only therapeutic alternative in this cases. |
| first_indexed | 2024-12-23T11:46:04Z |
| format | Article |
| id | doaj.art-346db8fe453b49eca3569d40abc131bf |
| institution | Directory Open Access Journal |
| issn | 2090-6226 |
| language | English |
| last_indexed | 2024-12-23T11:46:04Z |
| publishDate | 2022-01-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | Egyptian Liver Journal |
| spelling | doaj.art-346db8fe453b49eca3569d40abc131bf2022-12-21T17:48:21ZengSpringerOpenEgyptian Liver Journal2090-62262022-01-011211610.1186/s43066-021-00163-yIschemic cholangitis during Osler-Weber-Rendu disease: a case reportOussama Kharmach0Mohamed Borahma1Nawal Lagdali2Imane Benelbarhdadi3Fatima-Zohra Ajana4Department C of Hepato-gastroenterology, Ibn Sina University Hospital, Mohammed V UniversityDepartment C of Hepato-gastroenterology, Ibn Sina University Hospital, Mohammed V UniversityDepartment C of Hepato-gastroenterology, Ibn Sina University Hospital, Mohammed V UniversityDepartment C of Hepato-gastroenterology, Ibn Sina University Hospital, Mohammed V UniversityDepartment C of Hepato-gastroenterology, Ibn Sina University Hospital, Mohammed V UniversityAbstract Background Osler-Weber-Rendu disease (OWRD) is a rare autosomal dominant genetic disease that causes skin and mucosal telangiectasias and visceral arteriovenous malformations. These shunts can lead to high-output heart failure, portal hypertension, biliary or mesenteric ischemia. We report the case of a patient with OWRD complicated by ischemic cholangitis and biloma. Case presentation A 50-year-old female patient with personal history of spontaneous epistaxis and gingival bleeding, family history of Osler-Weber-Rendu disease (OWRD) in mother, and epistaxis in siblings. She consulted for intermittent and minimal biliary colic. Physical exam found mucocutaneous telangiectasia. Abdominal Doppler ultrasound/CT angiography showed arteriovenous shunts in the liver between hepatic artery and hepatic vein, hepatic artery and portal vein. Other sites of arteriovenous malformations were found in the nostril and jejunum. The diagnosis of OWRD was made. The evolution after 1 year was marked by the appearance of a slight anicteric cholestasis. MRCP found intrahepatic bilomas with associated ischemic cholangitis of segment VII. We decided to continue monitoring only. Conclusion The management of OWRD is based on screening for visceral arteriovenous malformations and on symptomatic measures that are often disappointing. Ischemic cholangitis is a rare complication, the diagnosis is based on imaging, liver transplantation is the only therapeutic alternative in this cases.https://doi.org/10.1186/s43066-021-00163-yOsler-Weber-Rendu diseaseBiliary colicHepatic arteriovenous malformationsIschemic cholangitisCase report |
| spellingShingle | Oussama Kharmach Mohamed Borahma Nawal Lagdali Imane Benelbarhdadi Fatima-Zohra Ajana Ischemic cholangitis during Osler-Weber-Rendu disease: a case report Egyptian Liver Journal Osler-Weber-Rendu disease Biliary colic Hepatic arteriovenous malformations Ischemic cholangitis Case report |
| title | Ischemic cholangitis during Osler-Weber-Rendu disease: a case report |
| title_full | Ischemic cholangitis during Osler-Weber-Rendu disease: a case report |
| title_fullStr | Ischemic cholangitis during Osler-Weber-Rendu disease: a case report |
| title_full_unstemmed | Ischemic cholangitis during Osler-Weber-Rendu disease: a case report |
| title_short | Ischemic cholangitis during Osler-Weber-Rendu disease: a case report |
| title_sort | ischemic cholangitis during osler weber rendu disease a case report |
| topic | Osler-Weber-Rendu disease Biliary colic Hepatic arteriovenous malformations Ischemic cholangitis Case report |
| url | https://doi.org/10.1186/s43066-021-00163-y |
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