This paper reviews the literature concerning optive nerve hypoplasia, including septo-optic dysplasia. It examines the cause and pathogenesis of optic nerve hypoplasia and analyzes the data from a total of 189 cases taken from three recent studies. General information, maternal history and subject characteristics are examined. Because of an apparent increase in the frequency with which this syndrome occurs, resulting in visual impairment and associated with midline defects, it is recommended that research which emphasizes behavioral and learning characteristics be conducted.
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