Hereditary hemorrhagic telangiectasia: On the subject of a clinical case

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Mario Alvarez T.
Mackarena Otárola M.
Tamara Novoa O.
Daniel Moyano M.
Luis Cabezas C.

Keywords

Hereditary hemorrhagic telangiectasia, Rendu Osler Weber Syndrome Arterio-Venous Malformation

Abstract

Epistaxis is a common symptom in the otorhinolaryngology consultation. Among the possible causes are hereditary hemorrhagic telangiectasia (Rendu Osler Weber syn- drome), which corresponds to an autosomal dominant disorder characterized by nasal and gastrointestinal bleeding associated with systemic arteriovenous malformations. Its most frequent manifestation is epistaxis, presenting more frequently in people over 40 years of age, without gender preference. We present the case of a male patient of 46 years old who consults for recurrence and severe epistaxis. During hospitalization, conventional treatment of epistaxis, retroactive diagnosis of Rendu Osler Weber syndrome and multidisciplinary management of pathology are performed. We review the literature and discuss the management of patients with this disease.

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