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Peripheral Pigmented Retinal Lesions in Stargardt Disease
Peter Y Zhao1, Maria Fernanda Abalem1, Daniel Nadelman1
1W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan.
American Journal of Ophthalmology
|December 31, 2017
Summary
Peripheral pigmented retinal lesions were found in some Stargardt disease patients, resembling CHRPE. These lesions may indicate a more severe disease phenotype.
Area of Science:
- Ophthalmology
- Genetics
- Retinal Imaging
Background:
- Stargardt disease is a common inherited macular dystrophy.
- Peripheral retinal findings in Stargardt disease are not well-characterized.
Purpose of the Study:
- To determine the prevalence of peripheral pigmented retinal lesions in Stargardt disease patients.
- To describe the clinical features associated with these lesions.
Main Methods:
- Retrospective review of medical records from a single academic institution.
- Analysis of wide-field retinal imaging in genetically confirmed Stargardt disease patients.
- Description of demographics, clinical features, and pathogenic variants.
Main Results:
- 14 out of 62 Stargardt disease patients had peripheral pigmented retinal lesions.
- Lesions were flat, subretinal, well-defined, and located in the periphery.
- Patients with lesions showed a median visual acuity of 20/200 and median central scotoma of 20 degrees.
Conclusions:
- Peripheral pigmented retinal lesions, similar to CHRPE, are present in a subset of Stargardt disease patients.
- These lesions may be associated with a more severe Stargardt disease phenotype.
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