Vascular opacification and leakage in X-linked (juvenile) retinoschisis

A 22-year-old man had X-linked retinoschisis with extensive vascular sheathing in the posterior pole and dendritic opacified retinal vessels in the periphery. Detailed fluorescein angiographic studies were performed. In the far periphery areas of capillary nonperfusion were seen. Sluggish circulation was present in the majority of dendritic opacified vessels in the areas of schisis. Late staining of the disc and posterior arteries and veins was noted. Scattered intraretinal fluorescein leakage was present even in clinically nonschitic retina, which suggests that the vascular changes may precede the retinoschisis. The patient's half-brother also had X-linked retinoschisis, but the schisis and the vascular changes were much less prominent. Although the retinal vascular changes with X-linked retinoschisis are similar to those seen with the Favre-Goldmann syndrome, a differentiation of the two diseases can be made by family studies, the presence or absence of nyctalopia, examination of the macular areas, measurement of dark adaptation, and electroretinogram determinations.