• Antônio José Cortez Juares, Alfredo Rafael Dell'Aringa, José Carlos Nardi, Kazue Kobari, Vera Lúcia Muller Gradim Moron Rodrigues, and Renato Martins Perches Filho.
• Faculdade de Medicina de Marília, Brazil.
• Braz J Otorhinolaryngol. 2008 May 1; 74 (3): 452-7.

AbstractHereditary Hemorrhagic Telangiectasia or Rendu-Osler-Weber Disease is a rare fibrovascular dysplasia that makes vascular walls vulnerable to trauma and rupture, causing skin and mucosa bleeding. It is of dominant autosomal inheritance, characterized by recurrent epistaxis and telangiectasia on the face, hands and oral cavity; visceral arteriovenous malformations and positive family history. Epistaxis is often the first and foremost manifestation. It's associated to arteriovenous malformations in several organs. There are possible hematologic, neurologic, pulmonary, dermatologic and gastrointestinal complications. Treatment is supportive and helps prevent complications. This study is a case report of a patient with this syndrome who came to the ENT Outpatient Ward of the Faculdade de Medicina de Marília; and we have done a bibliographic review of the disease's etiopathogenesis, clinical manifestations and clinical-surgical treatment options.

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