Linda S Kim1, Gerald A Fishman. 1. Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois, USA.
Abstract
PURPOSE: To investigate a previously reported observation that the presence of diffuse, as compared with localized, fundus flecks is an indicator of a more severe course for visual acuity (VA) loss beyond 20/200 in patients with Stargardt's disease. DESIGN: Retrospective clinic-based cross-sectional study. PARTICIPANTS: Four hundred five patients with Stargardt's disease. METHODS: Visual acuity in 405 patients with Stargardt's disease was analyzed at their initial and most recent visits. The stage of Stargardt's disease and appearance of the macula were recorded. The diagnosis and stage of Stargardt's disease were based on the extent and appearance of fundus flecks. MAIN OUTCOME MEASURES: Best-corrected VA, stage of Stargardt's disease, and appearance of the macula at the initial and most recent visits were used in the cross-sectional analysis. RESULTS: At their initial visit, 199 patients were identified as having stage 1 Stargardt's disease, of whom 97.5% maintained 20/200 or better VA in at least one eye. One hundred eighty-five were identified as having stage 2 or stage 2-3 Stargardt's disease combined, of whom 83.2% maintained 20/200 VA or better. Patients with stage 1 were more likely to maintain 20/200 or better VA in at least one eye when compared with patients with stage 2/stage 2-3 Stargardt's disease (chi2(1) = 21.25, P<0.001). Ninety-nine percent of stage 1 patients and 94.1% of stage 2/stage 2-3 patients maintained 20/400 or better VA in at least one eye (chi2(1) = 5.72, P = 0.017). CONCLUSIONS: Patients with stage 1 Stargardt's disease exhibiting fundus flecks limited to the posterior pole are more likely to maintain a level of VA at 20/200 or better when compared with patients with stage 2/stage 2-3 disease who have diffuse fundus flecks. Nevertheless, over 80% of the patients with stage 2/stage 2-3 Stargardt's disease still maintained VA of 20/200 or better. Only 5.9% of patients with stage 2/stage 2-3 had VA levels worse than 20/400. In our cohort of patients with Stargardt's disease and diffuse fundus flecks, the majority of patients did not lose VA to a greater extent than those with localized flecks.
PURPOSE: To investigate a previously reported observation that the presence of diffuse, as compared with localized, fundus flecks is an indicator of a more severe course for visual acuity (VA) loss beyond 20/200 in patients with Stargardt's disease. DESIGN: Retrospective clinic-based cross-sectional study. PARTICIPANTS: Four hundred five patients with Stargardt's disease. METHODS: Visual acuity in 405 patients with Stargardt's disease was analyzed at their initial and most recent visits. The stage of Stargardt's disease and appearance of the macula were recorded. The diagnosis and stage of Stargardt's disease were based on the extent and appearance of fundus flecks. MAIN OUTCOME MEASURES: Best-corrected VA, stage of Stargardt's disease, and appearance of the macula at the initial and most recent visits were used in the cross-sectional analysis. RESULTS: At their initial visit, 199 patients were identified as having stage 1 Stargardt's disease, of whom 97.5% maintained 20/200 or better VA in at least one eye. One hundred eighty-five were identified as having stage 2 or stage 2-3 Stargardt's disease combined, of whom 83.2% maintained 20/200 VA or better. Patients with stage 1 were more likely to maintain 20/200 or better VA in at least one eye when compared with patients with stage 2/stage 2-3 Stargardt's disease (chi2(1) = 21.25, P<0.001). Ninety-nine percent of stage 1 patients and 94.1% of stage 2/stage 2-3 patients maintained 20/400 or better VA in at least one eye (chi2(1) = 5.72, P = 0.017). CONCLUSIONS:Patients with stage 1 Stargardt's disease exhibiting fundus flecks limited to the posterior pole are more likely to maintain a level of VA at 20/200 or better when compared with patients with stage 2/stage 2-3 disease who have diffuse fundus flecks. Nevertheless, over 80% of the patients with stage 2/stage 2-3 Stargardt's disease still maintained VA of 20/200 or better. Only 5.9% of patients with stage 2/stage 2-3 had VA levels worse than 20/400. In our cohort of patients with Stargardt's disease and diffuse fundus flecks, the majority of patients did not lose VA to a greater extent than those with localized flecks.
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