Pseudoinflammatory macular dystrophy.
A family with pseudoinflammatory macular dystrophy (PMD) is presented. This dominantly inherited macular dystrophy has its onset in the 3rd to 5th decades with the earliest manifestation being a macular subretinal neovascular network. Visual function tests (ERG, EOG, visual fields, retinal sensitivity) in the early and late stages indicates this is local or geographic disease. This dystrophy should be differentiated from other hereditary causes for subretinal neovascularization (angioid streaks, vitelliform dystrophy, dominant drusen of Bruch's membrane, optic nerve drusen and myopia). It is suggested that treatment be directed at early obliteration of the subretinal neovascularization with intense photocoagulation since the outcome in virtually all cases of untreated PMD is legal blindness.
D000328:Adult; D003117:Color Vision Defects / Q000150:complications; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D008028:Light Coagulation; D008268:Macular Degeneration / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D008297:Male; D010375:Pedigree; D010857:Pigment Epithelium of Eye / Q000473:pathology; D012162:Retinal Degeneration / Q000235:genetics*; D012163:Retinal Detachment / Q000473:pathology; D012166:Retinal Hemorrhage / Q000473:pathology; D012171:Retinal Vessels / Q000473:pathology; D012173:Retinitis / Q000150:complications