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Y Rotenstreich, GA Fishman; Visual Acuity Loss in 270 Patients with Stargardt Disease . Invest. Ophthalmol. Vis. Sci. 2002;43(13):1164.
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Purpose: To assess visual acuity impairment in Stargardt disease. Methods: Cross-sectional data on best-corrected visual acuity in 270 Stargardt patients were analyzed for their most recent visit. Included were 143 female and 127 male patients. Of these, 150 were Caucasians, 81 African American, 20 Hispanic, and 19 with various other racial and ethnic backgrounds. Their mean age was 34 years (range: 8-78 years; median: 33 years). Fundus photographs and electroretinographic findings were reviewed and patients were categorized into four clinical phenotypes. Results: Sixty-three patients (23.3%) were 20 years old or less, 118 (43.7%) ranged from 21-40 years, 72 (26.6%) from 41-60 years, and 12 (4.4%) were 61 years or older. Sixty-six of the 270 patients (24.1%) had 20/40 or better acuity in at least one eye, 59 (21.9%) 20/50-20/100, 134 (49.6%) 20/200-20/400 and 12 (4.4%) were worse than 20/400 in each eye. An ANOVA analysis did not detect any statistical significant age difference between the above four visual acuity groups (p≷0.05). Sixteen percent of the patients with 20/40 or better were 20 years or less, 25% were 21-40 years and 26% were 41-60 years old (linear correlation r2=0.001 p=0.81). Fifty-two percent of the patients with 20/200-20/400 were 20 years or less, 48% were 21-40, and 57% were 41-60 (linear correlation r2=0.03 p<0.05). No statistical effect of age on LogMAR equivalent acuity was detected, when using the first three age groups in the context of ANOVA (p=0.58) or when using continuous age with regression (p=0.52). Fundus findings demonstrated foveal sparing in 71 (26.3%) of the patients. Of these, 49 (69%) had visual acuity of 20/40 or better, 14 (20%) had 20/50-20/100, and only 8 (11%) had visual acuity of 20/200 or less. Of the 199 patients without foveal sparing, 132 (66.3%) had visual acuity of 20/200 or less and only 15 (7.5%) had acuity of 20/40 or better. Of those with 20/40 or better 75.5% had foveal sparing. Conclusion: In a large cohort of Stargardt patients, almost a quarter had vision of 20/40 or better in at least one eye while less than 5% had acuity of worse than 20/400. The presence of foveal sparing was associated with a higher prevalence of patients with visual acuity of 20/40 or better.
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