Abstract
Purpose:
To evaluate the different global patterns of fundus autofluorescence in stargardt’s macular dystrophy as seen with ultra wide-field fundus autofluorescence (UWFAF) imaging.
Methods:
Patients with SMD who underwent UWFAF were included in this retrospective imaging study. Each eye was evaluated for zone of hypoautofluorescence, pattern of hypoautofluorescence, the presence of hyperautofluorescent pisciform flecks, and the presence of isolated peripheral hypoautofluorescence.
Results:
Sixty-nine eyes of 35 patients were included. Nine (25%) patients were male. The average age of patients was 45 years (range, 10-72 years). Ten (14%) eyes had hypoautofluorescence located in the mid-peripheral zone. Six (9%) eyes had diffuse hypoautofluorescence extending anteriorly. Five (7%) eyes had an isolated nummular (coin shaped) pattern only. Thirty-two (46%) eyes had a confluent nummular pattern only. Thirty (43%) eyes had both individual and confluent nummular patterns. Forty-one (59%) of 69 eyes had pisciform flecks. Eighteen (26%) of 69 eyes from 35 patients had isolated islands of peripheral hypoautofluorescence.
Conclusions:
Although SMD has been thought of as predominantly a macular dystrophy, we found that 16 (23%) eyes had RPE atrophy extending to the mid-periphery and beyond as detected by hypoautofluorescence on UWFAF. Most eyes had individual nummular, confluent nummular, or both nummular patterns of autofluorescence.
Keywords: 550 imaging/image analysis: clinical •
585 macula/fovea •
696 retinal degenerations: hereditary