Abstract
Although recognized as early as 1915, hypoplasia of the optic nerve is reported as rel atively rare. Review of the English litera ture reveals 16 unilateral and 12 bilateral cases. The largest series was comprised of six cases and was reported by Whinery and Blodi. This report also presented the first and only clinical histopathologic correlation. The essential features of optic nerve hypoplasia are marked visual impairment, re duced direct pupillary response to light, a small optic disk with normal appearing reti nal vasculature, and evidence that the defect has been present since birth. This entity has been reported in grossly deformed eyes, but the majority of cases show a paucity of other ocular findings. It may be seen in as sociation with other abnormalities, usually of the central nervous system.''
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