Spectrum of clinical findings in congenital retinoschisis. All examples are from boys with mutations in the RS1 gene. A, Fundus photograph shows the central radial yellow spokes consistent with foveal schisis. (The whitish patches in the outer macula represent light reflex from healthy internal limiting membrane and is a typical, normal finding in children.) B, Corresponding SD-OCT shows macular schisis. Compared with OCT findings in CME, those in macular schisis include more pronounced elongation of the inner retinal cavitations centrally and involvement beyond the parafoveal region (arrow). C, Montage photograph shows radial striae in the macula, “vitreous veils” traversing obliquely, and whitish spiculations in the superior and temporal periphery. D, Inferior periphery of the right eye shows a smooth, domed, convex elevation consistent with peripheral retinoschisis. E, Ultra-wide-field fundus photo graph shows extensive central and peripheral RPE atrophy consistent with advanced X-linked retinoschisis that is easily confused with rod–cone dystrophy. F, This 7-month-old boy, referred for nystagmus and strabismus, was found to have bilateral bullous schisis involving the superior macula (right eye is shown). G, Postoperative fundus photograph 2 months after vitrectomy, inner wall retinectomy, endolaser photocoagulation, and placement of silicone oil.