Abstract
Purpose: :
Stargardt disease (SD) is a form of juvenile onset macular degeneration. It has been suggested that the presence of diffuse fundus flecks, particularly early in the course of disease, is an indicator of a more severe long–term course for visual acuity (VA) loss beyond 20/200 (Oh et al. 2004). We investigated the VA loss among patients with different stages of this disease.
Methods: :
The VA in 362 patients with SD was analyzed at their most recent visit. The best corrected VA, patient age, stage of SD, and appearance of the macula were recorded. The diagnosis and classification of SD was based on the presence of fundus flecks, macular appearance and, when available, angiographic evidence of a "dark choroid."
Results: :
One hundred and sixty–one patients were identified as having Stage 1 SD (mean age=33.1 years, range 8–76 years), of which 156 (96.9%) maintained 20/200 or better VA in at least one eye. Five (3.1%) had VA worse than 20/200. Two hundred and one were identified as having Stage 2 or Stage 2–3 SD (mean age=34.9 years, range=7–71 years), of which 154 (76.6%) maintained 20/200 VA or better. Forty–seven (23.4%) demonstrated VA worse than 20/200. Analysis of VA and stage for patients with Stage 1 and patients with Stage 2/2–3 showed that patients with Stage 1 were more likely to maintain 20/200 or better VA in at least one eye when compared, as a group, to patients with Stage 2/2–3 SD (x2 = 28.26, 1 df, p< 0.001). One hundred percent of Stage 1 patients and 95.5% of Stage 2/2–3 patients maintained 20/400 or better VA in at least one eye (x2 = 5.66, 1 df, p=0.017).
Conclusions: :
Patients with Stage 1 SD, and fundus flecks limited to the posterior pole, are more likely to maintain levels of VA 20/200 or better when compared to patients with Stage 2/2–3 disease who have diffuse fundus flecks. Nevertheless, over ¾ of the patients with Stage 2/2–3 SD still maintained VA of 20/200 or better. Additionally, only 4.5% of patients with Stage 2/2–3 had VA levels worse than 20/400. In our cohort of patients with SD and diffuse fundus flecks, the majority of patients did not lose visual acuity to a greater extent than those with localized flecks.
Keywords: retinal degenerations: hereditary • clinical (human) or epidemiologic studies: prevalence/incidence