Osler-Weber-Rendu Syndrome with Severe Hepatic Manifestations: A Rare Clinical Case

  • Maria Inês Silva Internal Medicine Department, Hospital Prof. Dr. Fernando Fonseca EPE, Amadora, Portugal
  • Clara Matos Internal Medicine Department, Hospital Prof. Dr. Fernando Fonseca EPE, Amadora, Portugal
  • Fabio Correia Internal Medicine Department, Hospital Prof. Dr. Fernando Fonseca EPE, Amadora, Portugal
  • Sofia Carola Internal Medicine Department, Hospital Prof. Dr. Fernando Fonseca EPE, Amadora, Portugal
  • Maria João Gomes Internal Medicine Department, Hospital Prof. Dr. Fernando Fonseca EPE, Amadora, Portugal
  • Teresa Branco Internal Medicine Department, Hospital Prof. Dr. Fernando Fonseca EPE, Amadora, Portugal

Keywords

Rendu-Osler-Weber syndrome, arteriovenous malformations, epistaxis, congestive heart failure, chronic hepatic disease

Abstract

Rendu-Osler-Weber syndrome is a rare inherited syndrome with autosomal dominant transmission characterized by systemic arteriovenous malformations (AVMs) with multi-organ involvement. Its incidence is 1–2/100,000 and it is predominant in females (the male/female ratio varies from 1:2 to 1:4.5).Clinical manifestations and complications are related to recurrent bleeding and, in some cases, the development of end-organ failure. Management is mostly supportive care and it is essential to promote control of the disease as much as possible and screen eventual complications.

We describe the case of a 67-year-old male patient with Rendu-Osler-Weber syndrome admitted to the emergency department with decompensated heart failure due to acute anaemia because of severe epistaxis. During hospitalization, the patient progressed to acute-on-chronic liver failure with hepatic encephalopathy and an abdominal computed tomography scan showed multiple hepatic AVMs considered to be the cause of the chronic liver disease.

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Published: 2020-08-03

Issue: LATEST ONLINE (view)

Section: Articles

How to cite:
1.
Silva MI, Matos C, Correia F, Carola S, Gomes MJ, Branco T. Osler-Weber-Rendu Syndrome with Severe Hepatic Manifestations: A Rare Clinical Case. EJCRIM [Internet]. 2020Aug.3 [cited 2020Sep.24];20. Available from: https://ejcrim.com/index.php/EJCRIM/article/view/1831

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