Figure 2. External and fundus photographs in patients with X-linked retinoschisis and identified mutations in the RS1 gene. A: External photograph of patient M,II-3 (4 years) showed total lens opacification, and Coats’ disease was suspected initially when he was young. B: Fundus photograph of patient M,II-3 (4 years) had a typical cartwheel-like appearance in the central macula and white spots in the posterior pole. C: Patient G,III-3 (17 years) had peripheral retinal splitting at the superior temporal area. D: Patient P,II-2 (31 years) had sheathed retinal vessels with retinal splitting and pigmentation. E: Patient B,II-1 (18 years) had mild vitreous hemorrhage. F: Patient F,I-2 (52 years) had macula atrophy. G: Patient H,II-1 (6 years) had subretinal fibrosis and peripheral retinal splitting. H: Patient C,II-1 (23 years) had peripheral splitting and pigmentation. I: Patient K,II-1 (22 years) had golden-yellow reflex at the temporal retina. J: Patient G,I-1 (72 years) had peripheral retinal pigmentation without splitting. He did not have myopia, so the peripapillary crescent may be related to age and may not be related to this disease. K: Patient P,II-2 (31 years) had retinal splitting across the macula and white dots at the inferior temporal retina.