Rendu-Osler-Weber Syndrome: case report and literature review.
Hereditary 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.
Juares
Antônio José Cortez
AJ
Faculdade de Medicina de Marília, Brazil.
Dell'Aringa
Alfredo Rafael
AR
Nardi
José Carlos
JC
Kobari
Kazue
K
Gradim Moron Rodrigues
Vera Lúcia Muller
VL
Perches Filho
Renato Martins
RM
D000614:Aminocaproates / Q000627:therapeutic use*; D001803:Blood Transfusion; D004621:Embolization, Therapeutic; D004844:Epistaxis / Q000517:prevention & control*; D016558:Hemostasis, Endoscopic; D006801:Humans; D008297:Male; D008875:Middle Aged; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis / Q000628:therapy
D000614:Aminocaproates