Figure 5.

Left: magnified section of AOSLO image for each patient around the fixation point (black dots). Patients 2 and 3 use a region of hyperreflective cones for fixation, whereas fixation is located between two highly reflective regions in patient 4. Right: horizontal OCT scans through fixation. Outer retinal structures are relatively preserved at the fovea, but the outer nuclear layer is thin, and the outer segments/RPE junction layer is hyperreflective and elongated in patients 2 to 4 (B–D). The outer segments in patient 1 (who had evidence of diffuse cone dysfunction and the best visual acuity and sensitivity) were the least elongated and hyperreflective (A, right). Scale bars, 0.67°.