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Pattern dystrophy of the pigment epithelium
American Journal of Ophthalmology
|July 1, 1977
Summary
This study describes a family with inherited retinal pigment epithelium changes, presenting as granular or reticular patterns. These findings suggest a new inclusive term for pigmentary disorders affecting the retina.
Area of Science:
- Ophthalmology
- Medical Genetics
Background:
- Pigmentary changes in the retinal pigment epithelium can lead to visual disturbances.
- Several distinct types of pigment epithelial dystrophies have been identified.
Observation:
- A family spanning three generations exhibited progressive alterations in the retinal pigment epithelium.
- Younger individuals presented with subfoveal granular pigment, while older members displayed a reticular pigment pattern.
Findings:
- Most affected individuals had subnormal electro-oculogram results.
- Visual fields, color vision, dark adaptation, and electroretinogram remained normal across affected family members.
- The observed pigmentary changes resemble those in known reticular and butterfly-shaped dystrophies.
Implications:
- The study proposes a unifying clinical term, "pattern dystrophies of the pigment epithelium," due to overlapping clinical presentations.
- This inclusive terminology aims to improve classification until distinct pathophysiological differences are elucidated.