Abstract
The clinical and neuroradiologic features in 22 patients with septooptic dysplasia are reviewed. The most consistent abnormality seen in 15 patients was absence of the septum pellucidum and flattening of the roofs and inferior pointing of the floors of the anterior horns of the lateral ventricles. Hypoplasia of the anterior optic pathways was seen in 10 patients and a primitive optic ventricle was seen in two others. All patients had clinical optic disc hypoplasia; 12 patients had pituitary hormonal deficiencies. In most instances the diagnosis of septooptic dysplasia can be established by physical examination but neuroradiologic study is required to document associated structural abnormalities.
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