Bilateral Juvenile Retinoschisis in four Brothers of a Family

ABSTRACT

Juvenile retinoschisis is a vitreoretinal dystrophy with X-linked recessive mode of transmission that shows microcystic degeneration of the macula associated with splitting of the sensory retina, predominantly within the nerve fiber layer. We experienced X-linked juvenile retinoschisis, in four borthers within a family in which were the onset of visual disturbance between second decades and third decades, and all showed maculopathy, RPE atrophy, vitreous veils extended to vitreous, partial optic atrophy, specific electroretinographic findings.