Abstract
Abstract: :
Purpose: To examine and compare structural changes in macular dystrophies with optical coherence tomography (OCT), and to assess visual acuity impairment in Stargardt disease (SD). Methods: A total of 18 individuals with Stargardt disease were included in the study and examined clinically and with optical coherence tomography (OCT) scan, and results compared with visual field exam. Results: The patients were classified into four arbitrary age groups according to their age, and the level of their visual acuity was determined. The patients ranged between 8 and 48 years old. Ten patients were 20 years or less, 7 ranged from 21 to 40 years, and 2 from 41 to 60 years. Including all subjects, 3 (17%) had 20/40 or better acuity in at least one eye, 3 (17%) 20/50 to 20/100, and 6 (34%) 20/200 to 20/400, whereas 6 (34%) had worse than 20/400. Assessing the impairment of visual acuity within the age groups, 7 patients among 9 who were 20 years or less had visual acuity worse than 20/200 compared with 4 among 7 within the 21 to 40 age group. Fundus findings demonstrated maculopathy in 15 patients, and 12 of them presented visual acuity worse than 20/200. Fourteen patients presented flecks, and 11 presented flecks associated with maculopathy. OCT scans of the patients showed sub–pigmentepithelial deposits, diffuse retinal atrophy and loss of photoreceptors. Conclusions: Our results indicate that most of the patients with Stargardt disease had vision of 20/200 or worse during early ages of life. Maculopathy and flecks are common findings, and the presence of maculopathy was associated with a higher prevalence of 20/200 or worse visual acuity. OCT has the efficacy to remark the main structural retinal abnormalities in patients suffering from SD.
Keywords: retinal degenerations: hereditary • degenerations/dystrophies • visual acuity