Insights into autofluorescence patterns in Stargardt macular dystrophy using ultra-wide-field imaging

Abstract

Purpose: To characterize autofluorescence (AF) patterns occurring in Stargardt macular dystrophy (STGD1) using ultra-wide-field (UWF) imaging.

Methods: This paper is a cross-sectional observational study of 22 eyes of 11 patients (mean age 23.44 years) with Stargardt disease-fundus flavimaculatus who presented with decrease of vision at a tertiary eye care center. UWF short-wave AF images were obtained from all the patients using an Optos TX200 instrument. The main outcome measures were to assess patterns of AF changes seen on UWF AF imaging.

Results: All eyes showed a central area of hypoautofluorescence at the macula along with retinal flecks extending centrifugally as well as to the nasal side of the optic disc. Peripapillary sparing was seen in 100% of the eyes. Flecks were seen to be hypoautofluorescent in the center and hyperautofluorescent in the periphery in 77.8% eyes and were only hyperfluorescent in 27.2%. A background-increased fluorescence was visible in 100% of eyes, the outer boundary of which was marked by distribution of flecks in 81.9% eyes. A characteristic inferonasal vertical line was seen separating the nasal hypoautofluorescent area from the temporal hyperautofluorescent area in all the eyes.

Conclusions: UWF AF changes in STGD1 are not limited to the posterior pole and may extend more peripherally. UWF imaging is a useful tool for the assessment of patients with Stargardt macular dystrophy.

Keywords: Autofluorescence; Fundus flavimaculatus; Stargardt disease; Ultra-wide field.