First Author: F.Batıoğlu TURKEY
Co Author(s): P. Bingol Kızıltunc S. Demirel E. Ozmert 0 0 0 0 0 0 0 0 0
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To describe the clinical findings and disease progression of Stargardt – Fundus flavimaculatus disease with short-wave fundus autofluorescence (SW-AF) and near-infrared autofluorescence (NIR-AF) imaging and to evaluate their correlation with visual acuity.
Retrospective case series
The records of 40 eyes from 20 patients with Stargardt – Fundus flavimaculatus disease were evaluated retrospectively. The visual acuity (Snellen), fundus examination, SW-AF and NIR-AF findings were noted. Patients were divided into 3 groups according to their fundus autofluorescence (FAF) findings.
Of the patients, 10 were female and 10 were male. The mean age was 37 years. At initial examination there were 6 eyes with stage 1, 12 eyes with stage 2 and 22 eyes with stage 3. Patients with stage 1 showed increased autofluorescence with peripapillary sparing. At stage 2, there were flecks located in extrafoveal region to the periphery. Active flecks were hyperautofluorescent and inactive flecks were hypoautofluorescent. At stage 3 flecks were accompanied by macular atrophy. Although the hypoautofluorescent atrophic areas were sharply demarcated in SW-AF, the margins were irregular and atrophic areas were larger in NIR-AF. Also central involvement was more distinctive in NIR-AF. At 32 months follow-up, atrophy progression was seen in 4 eyes. The number of flecks were increased in 6 eyes and the hyperautofluorescent flecks became hypoautofluorescencent in 2 eyes. The mean visual acuity was 0.3 and only eyes with large central atrophy had worse visual acuity. There was no correlation between stages of the disease and the visual acuity.
The different phenotypic features determined by FAF imaging in Stargardt – Fundus flavimaculatus disease can give information about the progression and prognosis of the disease. These findings should be supported with genotype studies in large case series.