Foveal sparing in patients with Japanese Stargardt’s disease and good visual acuity

Abstract

Patients with Stargardt's disease usually have a poor visual prognosis. However, in our clinical practice we have observed some patients with dark-red foveal pigmentation (sparing) who had good best corrected visual acuity (BCVA) despite the presence of a late-stage disease. The purpose of this study was to investigate the BCVA outcomes in Japanese patients with Stargardt's disease with foveal sparing. Eighteen consecutive patients (36 eyes) with Stargardt's disease underwent ophthalmoscopy, fluorescein angiography, fundus autofluorescence imaging, and near-infrared fundus autofluorescence (NIA) imaging. The patients were divided into two groups based on the presence or absence of foveal sparing. The presence of foveal sparing was determined based on ophthalmoscopy and NIA imaging results. The association between foveal sparing and BCVA was assessed statistically by Students' t test. Of the 36 eyes, ten (27.8 %) had dark-red foveal sparing. The mean BCVA of the group with sparing was 0.16 ± 0.31, expressed in logarithm of the minimum angle of resolution (logMAR) units, and that of the group without sparing was 1.03 ± 0.39 logMAR units, which is a significant difference (p = 0.0000002507). A subgroup of Japanese patients with late-stage Stargardt's disease and dark-red foveal sparing maintained a relatively good BCVA. The pigmentation was clearly observed using NIA and proved useful for assessing the BCVA prognosis.