X-Linked Retinoschisis

  • Mild to severe reduction in visual acuity
  • Splitting of retinal layers
    • cystoid-like changes in macula
    • peripheral schesis cavities
  • electronegative ERG
  • Typically presents in 1st decade and often relatively stable until 6th decade

Etiology

  • RS1 gene mutation
  • 1/15,000-1/30,000 males

Treatment

  • Topical or systemic carbonic anhydrase inhibitors
  • Treat hyperopia