Osler-Weber-Rendu Syndrome as a Rare Cause of Liver Cirrhosis

It is essential to comprehend the clinical manifestations of hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome. This autosomal dominant vascular disorder presents with distinct symptoms, including mucocutaneous telangiectasia, epistaxis, gastrointestinal bleeding, and iron deficiency anemia. Furthermore, arteriovenous malformations (AVMs) commonly occur in the pulmonary, hepatic, and cerebral circulations. Hepatic involvement may be uncommon, depending on the subtype of HHT. HHT is a rare cause of liver cirrhosis. We present the case of a 59-year-old female patient referred to a hepatology consultation for the etiological study of liver disease that progressed to liver cirrhosis. From the study carried out, the presence of hepatic arteriovenous shunt was detected, which raised the suspicion of HHT, with the genetic study confirming the diagnosis. In this case, we intend to demonstrate the diagnostic difficulty of this entity, which led to a long time between the initial manifestation and the diagnostic conclusion. We would also like to highlight the importance of imaging and genetic studies in determining the etiology of the disease.

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Publication Details

Journal
Cureus
Published
2024-09-21
DOI
https://doi.org/10.7759/cureus.69869
Primary Topic
Vascular Anomalies and Treatments
Type
article
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article

Osler-Weber-Rendu Syndrome as a Rare Cause of Liver Cirrhosis

José Presa Ramos, Sofia Garcês Soares, Maria João Silva, Célia Tuna et al.
Cureus
Vascular Anomalies and Treatments
article

Osler-Weber-Rendu Syndrome as a Rare Cause of Liver Cirrhosis

José Presa Ramos, Sofia Garcês Soares, Maria João Silva, Célia Tuna, Teresa Frazão, Inês Pinho
article en

Abstract

It is essential to comprehend the clinical manifestations of hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome. This autosomal dominant vascular disorder presents with distinct symptoms, including mucocutaneous telangiectasia, epistaxis, gastrointestinal bleeding, and iron deficiency anemia. Furthermore, arteriovenous malformations (AVMs) commonly occur in the pulmonary, hepatic, and cerebral circulations. Hepatic involvement may be uncommon, depending on the subtype of HHT. HHT is a rare cause of liver cirrhosis. We present the case of a 59-year-old female patient referred to a hepatology consultation for the etiological study of liver disease that progressed to liver cirrhosis. From the study carried out, the presence of hepatic arteriovenous shunt was detected, which raised the suspicion of HHT, with the genetic study confirming the diagnosis. In this case, we intend to demonstrate the diagnostic difficulty of this entity, which led to a long time between the initial manifestation and the diagnostic conclusion. We would also like to highlight the importance of imaging and genetic studies in determining the etiology of the disease.

CureusVol. 16(9)
Hospital Center of Cova da Beira (PT), Hospital da Senhora da Oliveira Guimarães (PT), Unidade Local de Saúde de Trás-os-Montes e Alto Douro (PT), Centro Hospitalar do Tâmega e Sousa (PT)
Good health and well-being
Openalex Percentile: Top 74%
Vascular Anomalies and Treatments
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