X-linked congenital retinoschisis

Graefes Arch Clin Exp Ophthalmol. 1990;228(5):432-7. doi: 10.1007/BF00927256.


The natural history and electrophysiological findings of 52 patients with X-linked congenital retinoschisis with a follow-up of up to 26 years are described. The mean visual acuity was reduced to 0.24 +/- 0.2 and remained unchanged in most patients during this time. If visual loss occurred, it usually happened in the first decennium. The complications were retinal detachments in 11% and vitreous hemorrhages in 4% of the eyes. In general, the vitreous hemorrhages resolved spontaneously. Retinal detachments were treated successfully with conventional buckling procedures. Redetachments occurred in about 40%. Prophylactic laser coagulation was of no use because it was complicated by detachment in 43% of our series. The electro-oculogram was usually normal. In addition to the known electrorentinographic findings of normal a-wave and reduced b-wave amplitudes, we found prolonged b-wave latencies and implicit times, as well as a reduced 30 Hz flicker response.

MeSH terms

  • Adolescent
  • Adult
  • Color Perception Tests
  • Electrooculography
  • Electroretinography
  • Female
  • Follow-Up Studies
  • Genetic Linkage*
  • Humans
  • Male
  • Middle Aged
  • Refractive Errors / etiology
  • Retinal Detachment / etiology
  • Retinal Diseases / complications
  • Retinal Diseases / congenital
  • Retinal Diseases / genetics*
  • Retinal Diseases / physiopathology
  • Vision, Binocular
  • Visual Acuity
  • Visual Fields
  • Vitreous Hemorrhage / etiology
  • X Chromosome*