Fig. 2

Comprehensive advanced multimodal imaging evaluation. Legend Color fundus, autoflurescence, OCT B-scan images in RE (A-C) and LE (A1-C1), Angio OCT and enface in right eye (D) and in left eye (D1) (A-A1). The color fundus (Confocal fundus scanner, Eidon, Centervue, Padova, Italy) images show bone spicule pigmentations and retinal atrophy in all peripheral retina and at posterior pole. Through the atrophy of the retinal pigment epithelium (RPE) transpire the large vessels of the choroid also clear at the posterior pole. In addition, the optic nerve appears waxy and the arterial vessels are threadlike and of poor visualization. (B-B1) Autofluorescence (FAF) (Scanning laser Ophthalmoscope Mirante, Nidek, Milano, Italy) images highlight the areas of rounded atrophy at posterior pole, in peripapillary region and in middle periphery. The focal area of hyperautofluorescence at the foveal level shows central sparing by partial preservation of RPE and outer retina. The perifoveael hyperautofluorescent halo represents the area of increasing atrophy delineating future enlargement of atrophy in macula area. (C-C1) OCT B-Scan (Optovue Solix Visionix, Inc. Freemont, CA, USA) shows diffuse atrophy of the inner and outer retina with disappearance of the ellipsoid zone and external limiting membrane. Severe backscattering denotes diffuse atrophy of the RPE. Shadow cone effect in subfoveal region indicates the presence of preservation of the outer retina with associated underlying junction/EPR complex. (D-D1) Angio OCT (Optovue Solix Visionix, Inc. Freemont, CA, USA) shows choroidal transparency by choriocapillary atrophy of the RPE. Enface images show the delimited area of RPE atrophy (indicated by a yellow dash line) from the area where the RPE is still preserved