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AbstractFR.18.02 Functional correlates of annular fundus autofluorescence abnormalities in different types of retinal dystrophy Robson A. G. Fundus autofluorescence (AF) imaging allows visualization of the distribution of lipofuscin at the level of the retinal pigment epithelium. In patients with retinal disease, abnormal high density areas of autofluorescence may represent accumulation of lipofuscin resulting from disrupted metabolism at the level of the RPE. Absence of autofluorescence may reflect photoreceptor cell death with or without RPE atrophy. An annular parafoveal distribution of increased autofluorescence has been described in different retinal disorders including rod-cone (RCD) and cone-rod dystrophy (CRD). Some of these patients may manifest a parafoveal ring of abnormal increased autofluorescence. Functional correlates of these abnormalities have been investigated using pattern ERGs, multifocal ERGs, full-field ERGs and psychophysical measures of rod and cone sensitivity. In subjects with RCD and normal visual acuity, AF rings can vary in size and encircle areas of preserved AF and normal photopic function. In some patients AF rings show progressive changes with time that correlate spatially with visual field constriction. AF rings in subjects with CRD correspond with annular areas of sensitivity loss in younger subjects and encircle areas of central atrophy and severe dysfunction in older subjects. Ring size in CRD correlates with age and shows evidence of expansion in some individuals. This presentation will describe some of these studies and the significance of recent work aimed at further establishing the functional significance of fundus autofluorescence abnormalities in different types of retinal dystrophy. |
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