Rendu-Osler-Weber syndrome: what radiologists should know. Literature review and three cases report


Autoria(s): Agnollitto,Paulo Moraes; Barreto,André Rodrigues Façanha; Barbieri,Raul Fernando Pinsetta; Junior,Jorge Elias; Muglia,Valdair Francisco
Data(s)

01/06/2013

Resumo

Rendu-Osler-Weber syndrome or hereditary hemorrhagic telangiectasia is an autosomal dominant vascular disease involving multiple systems whose main pathological change is the presence of abnormal arteriovenous communications. Most common symptoms include skin and mucosal telangiectasias, epistaxis, gastrointestinal, pulmonary and intracerebral bleeding. The key imaging finding is the presence of visceral arteriovenous malformations. The diagnosis is based on clinical criteria and can be confirmed by molecular biology techniques. Treatment includes measures for management of epistaxis, as well as surgical excision, radiotherapy and embolization of arteriovenous malformations, with emphasis on endovascular treatment. The present pictorial essay includes a report of three typical cases of this entity and a literature review.

Formato

text/html

Identificador

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-39842013000300168

Idioma(s)

en

Publicador

Colégio Brasileiro de Radiologia e Diagnóstico por Imagem

Fonte

Radiologia Brasileira v.46 n.3 2013

Palavras-Chave #Rendu-Osler-Weber #Hereditary hemorrhagic telangiectasia #Arteriovenous fistula #Epistaxis
Tipo

journal article