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Pseudoinflammatory macular dystrophy
Abstract:
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.
Insights
Pseudoinflammatory macular dystrophy (PMD) is a dominantly inherited condition. Early photocoagulation of subretinal neovascularization is recommended to prevent legal blindness.
Area of Science:
- Ophthalmology
- Medical Genetics
Background:
- Presents a family with pseudoinflammatory macular dystrophy (PMD), a rare inherited retinal disorder.
- Characterizes PMD as a dominantly inherited condition with onset in the 3rd to 5th decades of life.
Observation:
- The earliest sign of PMD is the development of a macular subretinal neovascular network.
- Visual function tests (ERG, EOG, visual fields, retinal sensitivity) indicate a localized or geographic disease pattern in both early and late stages.
Findings:
- PMD requires differentiation from other hereditary conditions causing subretinal neovascularization, such as angioid streaks, vitelliform dystrophy, dominant drusen, optic nerve drusen, and myopia.
- Untreated PMD invariably leads to legal blindness.
Implications:
- Early detection and intervention are crucial for managing PMD.
- Intense photocoagulation targeting subretinal neovascularization is suggested as a potential treatment to preserve visual function and prevent blindness.