X-linked congenital retinoschisis.
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.
Kellner
U
U
Zentrum für Augenheilkunde, Universität Essen, Federal Republic of Germany.
Brümmer
S
S
Foerster
M H
MH
Wessing
A
A
D000293:Adolescent; D000328:Adult; D003119:Color Perception Tests; D004585:Electrooculography; D004596:Electroretinography; D005260:Female; D005500:Follow-Up Studies; D008040:Genetic Linkage; D006801:Humans; D008297:Male; D008875:Middle Aged; D012030:Refractive Errors / Q000209:etiology; D012163:Retinal Detachment / Q000209:etiology; D012164:Retinal Diseases / Q000150:complications / Q000151:congenital / Q000235:genetics* / Q000503:physiopathology; D015348:Vision, Binocular; D014792:Visual Acuity; D014794:Visual Fields; D014823:Vitreous Hemorrhage / Q000209:etiology; D014960:X Chromosome