Autosomal Dominant Vitreoretinochoroidopathy

Stuart J. Kaufman, Morton F. Goldberg, David H. Orth, Gerald A. Fishman, Howard Tessler, Katsuyoshi Mizuno

Research output: Contribution to journalArticlepeer-review

Abstract

Autosomal dominant vitreoretinochoroidopathy is a newly described fundus dystrophy characterized by abnormal chorioretinal hypopigmentation and hyperpigmentation, usually lying between the vortex veins and the ora serrata for 360° In this zone, there are a discrete posterior boundary, preretinal punctate white opacities, retinal arteriolar narrowing and occlusion, and, in some cases, choroidal atrophy. Most affected family members have diffuse retinal vascular incompetence, cystoid macular edema, and presenile cataracts. The vitreous is characterized by fibrillar condensation and a moderate number of cells. Electroretinograms are normal in younger affected individuals and are only moderately abnormal in older ones. Preretinal neovascularization, present in the posterior pole, is progressive in the proband. There are no identifiable systemic or skeletal abnormalities, high myopia, optically empty vitreous, lattice degeneration, areas of white-without-pressure, retinal breaks, or retinal detachment; thus, previously described vitreoretinopathies can be excluded from diagnostic consideration. Progression of this disease seems to be extremely slow in most family members.

Original languageEnglish (US)
Pages (from-to)272-278
Number of pages7
JournalArchives of ophthalmology
Volume100
Issue number2
DOIs
StatePublished - Feb 1982
Externally publishedYes

ASJC Scopus subject areas

  • Ophthalmology

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