Figure 3.

Fundus photographs and optical coherence tomography images of patients IV/1 and III/2 suffering in X-linked juvenile retinoschisis. In patient IV/1, the fundi show radially oriented intraretinal foveomacular cysts in a spoke-wheel configuration, with the absence of foveal reflex (IV/1 A, B). A golden-yellow reflex called Mizou-Nakamura phenomenon is seen on the posterior pole of both eyes of patient IV/1 marked by white arrowheads (A, B). The OCT images of him (IV/1 C, D) reveal retinoschisis in the inner nuclear layer (marked by blue arrow), in the photoreceptor layer (marked by yellow arrow) and some cysts in the outer plexiform layer (marked by white arrows) in both eyes, and one cyst in the ganglion cell layer (marked by red arrow) in the right eye. His OCT scans and foveal thickness maps show significant diffuse thickening of the right fovea (IV/1 C, C1-2), and because of the huge central cyst the significant pronounced thickening of the left fovea (IV/1 D, D1-2) compared with the controls. The eccentric fixation is clearly identifiable on his left FT map (IV/1 D1). In patient III/2, the fundi show spoke-wheel configurations in the foveas with the absence of foveal reflex (III/2 A, B). The OCT images reveal retinoschisis in the inner nuclear layer (pronounced in the left eye), small cysts in the ganglion cell layer of both eyes (III/2 C, D) and one cyst in the outer plexiform layer of left eye (III/2 D). His OCT scans and foveal thickness maps show significant diffuse thickening of the right fovea (III/2 C, C1-2), and because of the huge central cyst the significant pronounced thickening of the left fovea (III/2 D, D1-2). The eccentric fixation is clearly identifiable on his left FT map (IIII/2 D1).