Figure 4

Estimated photoreceptor survival and foveal B-scans for three STGD1 patients and their corresponding B-scan simulations. Patient P1 (female, 27 years old, best corrected visual acuity [BCVA] 20/100 OD) shows reduced foveal thickness with near normal parafoveal thickness (A), corresponding to severe cone loss and minimal rod loss (A′). Patient P2 (female, 58 years old, BCVA 20/20 OD) shows relative subfoveal preservation of the outer retinal layers with patches of parafoveal thinning (B), corresponding to slightly reduced cones and rods (B′). Patient P3 (female, 10 years old, BCVA 10/160 OD) shows widespread outer retinal loss (C), corresponding to nearly complete loss of cones, with some rods remaining (C′). The estimates of photoreceptor survival are computed with respect to the reference model. B-scans have been cropped to the extent of the ETDRS outer ring. Each patient is labeled in Fig. 3E. Rod and cone survival estimates can be fed back into the model to generate simulated B-scans (A′′, B′′, C′′).