Maculopathy of sex-linked juvenile retinoschisis

Observations in 14 eyes in 7 patients with sex-linked juvenile retinoschisis over a 4 to 8 year period show that the maculopathy tends to progress through a series of stages (Figs. 14, 15, 16) which are associated with increasing impairment in visual acuity. Electrical changes confirm the pathological state of the macula but suggest a pan-retinal disease. Desite the clinical appearance, the extramacular retina seems to be significantly affected. The basic pathological condition may be in the Muller cells. We have been unable to distinguish carriers from normal individuals.