Retinal function and loss of autofluorescence in Stargardt disease

Objective: To determine the relationship between reduced retinal autofluorescence and the dense scotomas in Stargardt disease. Method: Retrospective review of patients with Stargardt disease seen between 2000 and 2004 at a tertiary referral center. Patients: All 32 patients with Stargardt who underwent both autofluorescence (AF) imaging and scotoma mapping were included. Results: Forty-seven percent of eyes had dense scotomas corresponding to the areas of loss of AF (27% corresponding to the area of uniform loss of AF, and 20% corresponding to the area of uniform plus mottled loss of AF). Fifty-three percent of eyes did not have a good correlation of the dense scotoma with the area of loss of AF. Both eyes were judged to be in the same group in 75% of patients. Conclusions: Areas of reduced AF are measurable, but are not equivalent to the area of dense scotoma in 53% of patients. Measures of retinal sensitivity and scotomas are necessary for defining retinal functional involvement in Stargardt disease.