An unusual fundus phenotype of inner retinal sheen in X-linked retinoschisis.

摘要

X-linked retinoschisis (XLRS) is the leading cause of juvenile macular degeneration in men. The disorder is associated with mutations in the RSI gene.The usual clinical presentation is with reduced visual acuity, strabismus, or less commonly vitreous haemorrhage. The macula typically has a stellate appearance resulting from microcystic spaces radiating from the fovea and peripheral schisis is present in about 50% of cases.