The authors present a study of a cohort of patients with genetically proven ABCA4 gene mutation related Stargardt disease. They aimed to quantify the effect of lesion location and topography on disease progression using fundus autofluorescence imaging. One hundred and ninety-three eyes of 97 patients met initial inclusion criteria, and 71 eyes of 36 patents had repeat visits and imaging. Mean duration of follow-up was 30.6 months. The patients had wide field fundus autofluorescence imaging using Optos California cameras, and the resulting images were projected onto a curved surface of 24mm diameter to represent the configuration of a normal eye and allow lesion area to be accurately measured. Lesions were classified in a semi-automated manner with the aid of two independent graders into areas of definitely and questionably decreased autofluorescence. These were then used to show the average distribution of lesions, and also to calculate rate of progression of lesion size. The authors show that while the incidence of Stargardt lesions increases with proximity to the fovea, the growth rate of the lesions increase with more peripheral location. They suggest that these findings are of importance to the future design of clinical trials of treatments for Stargardt disease, in particular when considering outcome measures for future studies.
Progression of macular atrophy in Stargardt disease
Reviewed by Ian Reekie
Local progression kinetics of macular atrophy in recessive Stargardt disease.
CONTRIBUTOR
Ian Reekie
Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK.
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