Rendú Osler Weber Syndrome; case report

Abstract

Hereditary hemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber disease, is a dominant autosomal disease characterized by the presence of multiple telangiectasia in skin and mucus, associated with arteriovenous malformations (AVM) of various organs, including the lungs, gastrointestinal system and brain. HHT is presented most frequently as recurrent, spontaneous epistaxis. Patients may also present digestive, pulmonary and intracranial hemorrhage, as well as secondary anemia.This article reports the case of a female patient, 62 years old, with multiple episodes of epistaxis and vaginal bleeding, with diagnosis of complex HHT, which was managed with multiple embolizations, which improved symptoms and survival. In this kind of patient, it is possible, with timely diagnosis and treatment, to obtain a greater quality and expectation of life. Due to the fact that the severity and alterations in each patient are so variable, management should be individualized.

Keywords

• Rendu-Osler-Weber
• Hereditary hemorrhagic telangiectasia
• Arteriovenous fistula
• Arteriovenous malformation
• Epistaxis