Congenital X-linked Retinoschisis and Familial Exudative Vitreoretinopathy: Pathogenesis, Diagnosis, and Treatment

Congenital X-linked retinoschisis (CXLRS) is characterized by symmetric bilateral macular involvement, usually beginning in the first decade of life and affecting males. In fundus examination, a wheel pattern developing due to schisis areas observed macula. Fundus examination optical coherence tomography (OCT) are essential for diagnosis early stages disease exhibit characteristic findings. Peripheral occurs approximately 50% cases, inferior temporal quadrant. Visual acuity deteriorates second decades life, but then remains relatively stable until fifth or sixth decade. There no proven treatment CXLRS so far, gene therapy studies ongoing. Familial exudative vitreoretinopathy (FEVR) rare inherited vitreoretinal disorder abnormal incomplete vascularization peripheral retina. The frequently as autosomal dominant less recessive recessive. FEVR manifested which may be asymmetrical. Its distinctive features include avascular retina ischemia, retinal neovascularization, subretinal exudation. Epiretinal membrane tractional detachment also develop some patients. Laser photocoagulation effective patients with ischemia neovascularization. this review, pathogenesis, clinical features, methods will discussed.