Fig. 1

Multimodal imaging findings at presentation. a Fundus photograph of the left eye at presentation shows foveal granularity surrounded by diffuse retinal discoloration with satellite slightly indistinct, multifocal deep yellow-white dots. There also were blurred disc margins with peripapillary whitening. b Fundus autofluorescence of the left eye at presentation shows multiple, coalescent, punctate hyperautofluorescent lesions associated with focal small hypoautofluorescent areas. c Early-phase fluorescein angiogram of the left eye at presentation shows patchy areas of choroidal hypofluorescence in a geographic configuration. d Late-phase fluorescein angiogram demonstrates punctate hyperfluorescence with a “wreath-like” configuration around the fovea and optic disc leakage. e Early-phase indocyanine green angiogram of the left eye at presentation shows well-demarcated geographic areas of choroidal hypofluorescence that extend beyond the clinical limits of the yellowish retinal lesions, with large choroidal vessels visible within these hypofluorescent areas. This is highly suggestive of choriocapillaris ischemia and allows excluding a masking effect. f Late-phase indocyanine green angiogram shows persistent hypofluorescence in the posterior pole, with associated peripapillary hypofluorescence and optic disc staining. g Swept-source OCT image of the left eye at presentation shows disruption of the ellipsoid zone, accumulations of a hyperreflective material over the RPE (arrows), and hyperreflective dots in the inner choroid (subfoveal choroidal thickness = 370 μm). h Swept-source OCT angiogram of the choriocapillaris reveals confluent extensive geographic areas of loss of signal that colocalize with the ICGA hypofluorescent areas with associated projection artifact from retinal vessels