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Abstract:
Four generations of a family with congenital retinoschisis are described. Six male members in the second generation suffer from this disease. The fundus changes in these patients were advanced with marked chorioretinal atrophy. Schisis of the retina was only seen in one of them. Three boys in the fourth generation exhibited retinoschisis with typical macula changes. One had detachment in one eye. ERG was in most cases subnormal or extinguished. Fluorescein angiography showed leakage of dye in 2 patients and one of these had vessel malformation.