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. Author manuscript; available in PMC: 2020 Dec 15.
Published in final edited form as: Prog Retin Eye Res. 2020 Apr 9;79:100861. doi: 10.1016/j.preteyeres.2020.100861

Fig. 4.

Fig. 4.

Autofluorescence subtypes of the bull’s eye maculopathy (BEM) stage of ABCA4-associated retinopathy. Confined BEM lesions are generally the earliest manifestation of macular affection in ABCA4-associated retinopathy and are highly associated with the c.5882G>A, p.(Gly1961Glu) mutation. (A–C) Small, focal lesions are typically associated with a loss of the ellipsoid zone (EZ) band and subsequent cavitation of this space in the fovea (“optical gap”). (D–F) Uniformly round BEM lesions exhibit continuous autofluorescence borders and punctate debris within the atrophy region. (G–I) Elliptical BEM lesions also exhibit smooth, continuous autofluorescent borders; however, the region inside the lesion contains less debris and are marked by a central patch of autofluorescence (“bull’s eye”) indicating prior sparing of the fovea. Much less common are centrally mottled BEM lesions (J–L) which are distinct in that they lack a hyperautofluorescent perimeter and are almost exclusive to adolescent patients.