The location of the en face OCT shown in (D) is outlined on the fundus photograph (A). The EZ disruption in this patient is most clearly visualized with B-scan OCT, as the en face image revealed a poorly discriminated hyper-reflectivity at the center of the macula (D). Dashed lines on D indicate locations of the horizontal and vertical B-scans (B,C), while the square represents the area shown in (E–G). The confocal AOSLO image revealed a severely disrupted mosaic, where the vast majority of photoreceptors are non-waveguiding (F). The split-detector image revealed a contiguous photoreceptor mosaic composed of cones at the center, and then rods and cones in the immediate surrounding parafovea (G). The area of EZ hyper-reflectivity in (E) corresponds spatially to the area of densest cone packing (G). Although the EZ reflectivity appears normal in the parafovea (E), the confocal image revealed that the majority of cones are abnormal and non-waveguiding (F). B–D scalebars = 200 μm. E–G scalebars = 100 μm.