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

Agnollitto, Paulo Moraes; Façanha Barreto, André Rodrigues; Pinsetta Barbieri, Raul Fernando; Junior, Jorge Elias; Muglia, Valdair Francisco

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.

RADIOTHERAPY; NOSEBLEED; HEREDITARY hemorrhagic telangiectasia; VASCULAR diseases; INTRACEREBRAL hematoma; MOLECULAR biology

Radiologia Brasileira, 2013, Vol 46, Issue 3, p168

0100-3984

Academic Journal

10.1590/S0100-39842013000300011