Portuguese Version

Year:  2000  Vol. 66   Ed. 2 - (13º)

Relato de Casos

Pages: 172 to 180

Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Disease): Otolaryngologic Diagnostic.

Author(s): Eloisa N. Maudonnet*,
Cláudio C. Gomes*,
Eulália Sakano***.

Keywords: hereditary hemorrhagic telangiectasia, epistaxis

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease) is a systemic autosomal dominant fibrovascular dysplasia, characterized by classical trio: telangiectasias, recurrent epistaxis and family history. Arteriovenous malformations, aneurisms and telangiectasias can occur over all the body vessels system which will show, then, endothelial cells degeneration, defective endothelial junctions and weakness of the connective tissue. Its prognostic is essencialy benign, but hepatic, gastrointestinal, pulmonary, cardiac and neurological complications may happen. Epistaxis is the most common bleeding (80% of the affected) and generally it is the first hemorrhagic event o€ the disease. The diagnosis includes history, physical examination, hematologic tests, endoscopy, angiography, chest X ray and fluoroscopy. Theraphy is supportive, offering iron supplement and blood transfusions, but hormonal and surgical treatment have obtained substancial results in bleeding control. Its diagnostic, referring to the organs involved, is important for the prevention and adequate treatment of the complications, as well as for genetic orientation. We present 9 cases and considerations about their clinical symptoms, making a revision of the current physiopathology, clinical manifestations, clinical and surgical treatment.






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