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Measurement of Carotenoids in Perifovea using the Macular Pigment Reflectometer
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Macular hyperpigmentary changes in ABCA4-Stargardt disease
Maria Fernanda Abalem1,2, Amro A Omari1, Dana Schlegel1
11Department of Ophthalmology and Visual Sciences, W. K. Kellogg Eye Center, University of Michigan, 1000 Wall Street, Ann Arbor, MI 48150 USA.
International Journal of Retina and Vitreous
|April 16, 2019
Summary
Macular hyperpigmentary changes in Stargardt disease (STGD) indicate a severe phenotype. These lesions grow and spread over time, suggesting they may signal advanced STGD.
Area of Science:
- Ophthalmology
- Genetics
- Retinal Diseases
Background:
- Stargardt disease (STGD) and age-related macular degeneration (AMD) share similarities.
- Macular hyperpigmentary changes are linked to poorer prognosis in AMD.
Purpose of the Study:
- To characterize macular hyperpigmentary changes in STGD patients.
- To associate these changes with the severity of the STGD phenotype.
Main Methods:
- Retrospective cross-sectional study of 141 STGD patients.
- Evaluation of hyperpigmentary changes using fundus photography and OCT.
- Phenotype severity assessed by ffERG, FAF, and visual acuity.
Main Results:
- 21.7% of patients exhibited macular hyperpigmentary changes in four patterns.
- Follow-up showed lesion enlargement and RPE atrophy progression in 11/17 patients.
- ffERG and FAF revealed varying degrees of retinal dysfunction and disease spread.
Conclusions:
- Macular hyperpigmented lesions in STGD are associated with a severe phenotype.
- These lesions increase in size and migrate across retinal layers over time.
- Macular hyperpigmentation may serve as a marker for advanced STGD.
Keywords:
Age related macular degenerationGeneticsOptical coherence tomographyPrognosisStargardt diseaseMore Related Videos
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