ArticleRetina (Philadelphia, Pa.)2016
MAPPING THE DENSE SCOTOMA AND ITS ENLARGEMENT IN STARGARDT DISEASE.
Article in Retina (Philadelphia, Pa.), 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 18 citations in OpenAlex.
- The Limitations on Vergence Responses to Naturalistic Images Resulting From Simulated Central Scotomas and Defocused Peripheral Retina.Ophthalmic & physiological optics : the journal of the British College of Ophthalmic Opticians (Optometrists) · 2026Article
- Characterization of the Subclinical Perilesional Zone in the Macula of Early-Stage ABCA4 Disease.Translational vision science & technology · 2025Article
- Superotemporal predisposition to traumatic subretinal fibrosis in Stargardt disease: A case report.American journal of ophthalmology case reports · 2025Article
- Clinical spectrum, genetic complexity and therapeutic approaches for retinal disease caused by ABCA4 mutations.Progress in retinal and eye research · 2020Review
- Analysis of retinal sublayer thicknesses and rates of change in ABCA4-associated Stargardt disease.Scientific reports · 2020Observational
- Abnormal Visual Function Outside the Area of Atrophy Defined by Short-Wavelength Fundus Autofluorescence in Stargardt Disease.Investigative ophthalmology & visual science · 2020Article
- Correlation between Choriocapillaris Density and Retinal Sensitivity in Stargardt Disease.Journal of clinical medicine · 2019Article
- Macular hyperpigmentary changes inInternational journal of retina and vitreous · 2019Article
- Comparison of Green Versus Blue Fundus Autofluorescence inTranslational vision science & technology · 2018Article
- Peripheral Visual Fields in ABCA4 Stargardt Disease and Correlation With Disease Extent on Ultra-widefield Fundus Autofluorescence.American journal of ophthalmology · 2017Article
- The Importance of Outcome Measure Research in Stargardt Disease.JAMA ophthalmology · 2017Article
- Juvenile Macular Degenerations.Seminars in pediatric neurology · 2017Review
Corrections and comments
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Authors and funding
4 authors at 3 institutions in 2 countries.
Funding
Abstract
purposeTo describe the enlargement of the dense scotoma over time in Stargardt disease and to highlight methodologic issues in tracking enlargement.
methodsRetrospective study of patients with full mapping of the border of the dense scotoma using the MP-1 for at least two visits.
results14 eyes of 7 patients met this criterion. Patients had median of 3 visits (range 2-5), with median total follow-up of 4.5 years (range 1.5-8). Mean baseline visual acuity was 20/56 (range 20/25-20/200), mean baseline dense scotoma area was 2.23 mm (range 0.41-5.48), and mean dense scotoma enlargement rate was 1.36 mm/year (range 0.22-2.91). The younger patients tended to have more rapid loss of visual acuity, which tended to plateau when the visual acuity was 20/100 or worse. The patients who developed Stargardt before age 20 years, and the single patient who developed Stargardt disease after age 40 years, had more rapid enlargement rates, with preservation of central vision in the oldest patient. The ability to precisely define the dense scotoma area was dependent on the density location of the points tested; this led to significant variability in the assessment of the scotoma enlargement rate in three of the seven patients. The dense scotoma was not described adequately by the extent of the homogeneous dark area on fundus autofluorescence imaging.
conclusionMicroperimetry is necessary for mapping the scotoma in patients with Stargardt disease, because current imaging is not adequate. Standardized grid testing, plus a standardized procedure for refining the border of the dense scotoma, should allow more precise testing and longitudinal assessment of enlargement rates.
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