Congestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case report
Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome is a rare autosomal dominant vascular disorder characterized by epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations affecting various organs and systems. The liver is a commonly involved organ (74% of patients...
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| Format: | Article |
| Language: | English |
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Elsevier
2018-02-01
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| Series: | Radiology Case Reports |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1930043317303941 |
| _version_ | 1811194409634496512 |
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| author | Patrícia Leitão, PhD André Carvalho, MD Conceição Guerra, MD José Gonçalves, MD Isabel Ramos, PhD |
| author_facet | Patrícia Leitão, PhD André Carvalho, MD Conceição Guerra, MD José Gonçalves, MD Isabel Ramos, PhD |
| author_sort | Patrícia Leitão, PhD |
| collection | DOAJ |
| description | Hereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome is a rare autosomal dominant vascular disorder characterized by epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations affecting various organs and systems. The liver is a commonly involved organ (74% of patients with hereditary hemorrhagic telangiectasia), although symptomatic liver disease is quite infrequent. In symptomatic cases, clinical manifestations relate most commonly to the predominant type of vascular shunting present (arteriovenous, arterioportal, or portovenous). Clinically, liver disease can manifest as a high-output cardiac failure, portal hypertension, or biliary disease. Imaging plays an important role in diagnosis, characterization, and follow-up of liver involvement, with ultrasound, computed tomography, magnetic resonance imaging, and angiography being useful in this context. We present a case of congestive cirrhosis with florid liver findings in a patient with Osler-Weber-Rendu syndrome. Imaging findings that clinched the diagnosis are reviewed. A brief literature review is also provided. |
| first_indexed | 2024-04-12T00:25:31Z |
| format | Article |
| id | doaj.art-725cc9a1aa774380ba1e3b6f9eb085df |
| institution | Directory Open Access Journal |
| issn | 1930-0433 |
| language | English |
| last_indexed | 2024-04-12T00:25:31Z |
| publishDate | 2018-02-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Radiology Case Reports |
| spelling | doaj.art-725cc9a1aa774380ba1e3b6f9eb085df2022-12-22T03:55:32ZengElsevierRadiology Case Reports1930-04332018-02-01131515410.1016/j.radcr.2017.10.011Congestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case reportPatrícia Leitão, PhD0André Carvalho, MD1Conceição Guerra, MD2José Gonçalves, MD3Isabel Ramos, PhD4Radiology Department, Centro Hospitalar São João, Porto, PortugalRadiology Department, Centro Hospitalar São João, Porto, PortugalRadiology Department, Centro Hospitalar São João, Porto, PortugalRadiology Department, Centro Hospitalar São João, Porto, PortugalRadiology Department, Centro Hospitalar São João, Porto, PortugalHereditary hemorrhagic telangiectasia or Osler-Weber-Rendu syndrome is a rare autosomal dominant vascular disorder characterized by epistaxis, mucocutaneous telangiectasias, and arteriovenous malformations affecting various organs and systems. The liver is a commonly involved organ (74% of patients with hereditary hemorrhagic telangiectasia), although symptomatic liver disease is quite infrequent. In symptomatic cases, clinical manifestations relate most commonly to the predominant type of vascular shunting present (arteriovenous, arterioportal, or portovenous). Clinically, liver disease can manifest as a high-output cardiac failure, portal hypertension, or biliary disease. Imaging plays an important role in diagnosis, characterization, and follow-up of liver involvement, with ultrasound, computed tomography, magnetic resonance imaging, and angiography being useful in this context. We present a case of congestive cirrhosis with florid liver findings in a patient with Osler-Weber-Rendu syndrome. Imaging findings that clinched the diagnosis are reviewed. A brief literature review is also provided.http://www.sciencedirect.com/science/article/pii/S1930043317303941Osler-Weber-Rendu syndromeLiver cirrhosisVascular shunts |
| spellingShingle | Patrícia Leitão, PhD André Carvalho, MD Conceição Guerra, MD José Gonçalves, MD Isabel Ramos, PhD Congestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case report Radiology Case Reports Osler-Weber-Rendu syndrome Liver cirrhosis Vascular shunts |
| title | Congestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case report |
| title_full | Congestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case report |
| title_fullStr | Congestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case report |
| title_full_unstemmed | Congestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case report |
| title_short | Congestive cirrhosis in Osler-Weber-Rendu syndrome: A rare case report |
| title_sort | congestive cirrhosis in osler weber rendu syndrome a rare case report |
| topic | Osler-Weber-Rendu syndrome Liver cirrhosis Vascular shunts |
| url | http://www.sciencedirect.com/science/article/pii/S1930043317303941 |
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