Rendu-osler syndrome (rendu-osler- weber syndrome; hereditary hemorrhagic telangiectasis; babington disease; goldstein hematemesis; osler syndrome [2])

General: Etiology unknown; autosomal dominant in Jews; repeated epistaxis begins in childhood; gastrointestinal hemorrhages with melena and hematemesis manifest in middle and later life.

Ocular: Star-shaped angiomas of the palpebral conjunctiva; intermittent filamentary keratitis; small retinal angiomas and occasionally retinal hemorrhages; subconjunctival hemorrhages; small retinal arteriovenous malformations; bloody tears; conjunctival telangiectasias.

Clinical: Epistaxis; hematuria; melena; angiomata of the pharynx and oral and nasal mucosa; angiomas on lips, face, and upper extremities; cyanosis; polycythemia.

Brant AM, et al. Ocular manifestations III hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease). Am J Ophthalmol 1989; 107:642.

Ebert EM, Boger WP III, Albert DM. Phakomatoses. In: Albert DM, Jakobiec FA, eds. Principles and Practice of Ophthalmology, vol. V. Philadelphia: WB Saunders, 1994:3323.

Osler W. On a familial form of recurring epistaxis associated with multiple telangiectases of the skin and mucous membrane. Bull Johns Hopkins Hosp 1901; 12:333.

Rendu M. Epistaxis Repetees Chez un Sujet Porteur de Petits Angiomes Cutanes et Muqueux. Bull Mem Soc Med Hop 1896; 13:731.

Vase I, Vase P. Ocular lesions in hereditary haemorrhagic telangiectasia. Acta Ophthalmol 1979; 57:1084-l090.

Wolper J, Laibson PR. Hereditary hemorrhagic telangiectasis (Rendu-Osler-Weber disease) with filamentary keratitis. Arch Ophthalmol 1969; 81:272.