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Autosomal dominant neovascular inflammatory vitreoretinopathy
Journal article   Peer reviewed

Autosomal dominant neovascular inflammatory vitreoretinopathy

Steven R Bennett, James C Folk, Alan E Kimura, Stephen R Russell, Edwin M Stone and E Mike Raphtis
Ophthalmology (Rochester, Minn.), Vol.97(9), pp.1125-1136
09/1990
DOI: 10.1016/S0161-6420(90)32447-8
PMID: 2234842

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Abstract

Twenty-eight of 61 members of a six-generation family are affected by an autosomal dominant eye disease which has not been described previously. Affected patients are asymptomatic in early adulthood, but have vitreous cells and the selective loss of the b-wave on the electroretinogram. Later, peripheral retinal scarring and pigmentation, peripheral arteriolar closure, and neovascularization of the peripheral retina at the ora serrata or occasionally neovascularization of the optic disc develop. Cystoid macular edema, vitreous hemorrhage, tractional retinal detachment, and neovascular glaucoma can cause profound visual loss. Vitrectomy reduces traction on the retina and allows for retinal reattachment. The role of argon laser photocoagulation or cryopexy in reducing the neovascular complications remains uncertain.
Electroretinography Light Coagulation Retinal Diseases - genetics Humans Middle Aged Male Visual Acuity Genes, Dominant Pedigree Aged, 80 and over Adult Female Retinal Neovascularization - genetics Vitrectomy Aged Retinal Diseases - physiopathology Retinal Neovascularization - physiopathology Fundus Oculi Fluorescein Angiography

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