Abstract
Hereditary LEREDITARY hemorrhagic telangiectasia manifests itself by the formation of localized arteriovenous capillary connections that are tiny arteriovenous fistulas. The first comprehensive description was given by Rendu 1 in 1896. Osler 2 in 1901 and Weber 3 in 1907 added to knowledge of the entity, which brought about the designation "Rendu-Osler-Weber syndrome." In 1909, Hanes 4 used the term "hereditary hemorrhagic telangiectasia," which is the preferred designation. Hodgson and colleagues 5 studied 231 members of a large family of which 91 descendants had the disease entity. Of the descendants studied, 40% had telangiectasia and 6% had pulmonary arteriovenous fistulas. In 50% of the cases of pulmonary arteriovenous fistula, telangiectasia elsewhere in the body is encountered. 6 Approximately 5% to 6% of patients with Rendu-Osler-Weber syndrome have pulmonary arteriovenous fistula. 7 Pulmonary arteriovenous fistulas may occur in persons with or without hereditary hemorrhagic telangiectasia, but the close relationship makes it imperative that patients with this disorder be investigated
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