Fundus autofluorescence in Stargardt macular dystrophy-fundus flavimaculatus

Abstract

Purpose: To quantify autofluorescence (AF) levels in patients with Stargardt macular dystrophy-fundus flavimaculatus (STGD-FFM), and to identify patterns of AF.

Design: Observational, comparative study.

Methods: Prospective study.

Settings: Patients were recruited at Moorfields Eye Hospital.

Study population: Forty-three STGD-FFM patients aged 20 to 40 years and 35 age-matched normal volunteers. The right eye was chosen arbitrarily for measures of AF.

Intervention: The AF images were obtained using a confocal scanning laser ophthalmoscope. Levels of AF across the macula were measured. The distribution of AF was also evaluated. In 36 patients (84%) pattern electroretinogram (PERG) and full-field ERG were obtained and results were evaluated with respect to levels of AF.

Main outcome measures: Values of AF, AF distribution, PERG, and ERG.

Results: Normal or high AF at the center of the macula with high AF temporally or nasally or both was detected in 17 patients (39%). In nine (21%), low AF at the center of the macula with normal or low AF temporally or nasally or both was found. Levels of AF were normal throughout the macula in six patients (14%). In 11 (26%), high, normal, and low levels of AF were found. All patients tested with low AF at the center of the macula and normal or low AF temporally or nasally or both had peripheral cone/rod dysfunction. None of the patients tested that had normal or high AF at the fovea and high AF temporally or nasally, or normal AF throughout the macula, had peripheral cone/rod dysfunction.

Conclusion: AF is not universally high in STGD-FFM. Some patients have normal or low AF. Autofluorescence patterns appear to relate to functional abnormalities.