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Management of hereditary hemorrhagic telangiectasia

Claire Shovlin, PhD, FRCP
Section Editor
Lawrence LK Leung, MD
Deputy Editor
Jennifer S Tirnauer, MD


Hereditary hemorrhagic telangiectasia (HHT; also called Osler-Weber-Rendu syndrome) is an autosomal dominant vascular disorder associated with a variety of clinical manifestations including mucocutaneous telangiectasia, epistaxis, gastrointestinal bleeding, and iron deficiency anemia. In addition, arteriovenous malformations (AVMs) commonly occur in the pulmonary, hepatic, and cerebral circulations, demanding knowledge of the risks and benefits of screening and treatment of patients with these complications.

The management of HHT will be reviewed here. The pathophysiology, epidemiology, and diagnosis of HHT are discussed in detail separately.

Additional discussions of pulmonary AVMs, which affect over half of individuals with HHT, are also discussed separately. (See "Pulmonary arteriovenous malformations: Epidemiology, etiology, and pathology in adults" and "Pulmonary arteriovenous malformations: Clinical features and diagnostic evaluation in adults" and "Therapeutic approach to adult patients with pulmonary arteriovenous malformations".)


Major management issues in individuals with HHT span the full range of clinical manifestations (table 1), and include the following [1-11]:

Patient education.

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Literature review current through: Oct 2017. | This topic last updated: Sep 29, 2017.
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