Septo-optic dysplasia

ABSTRACT

Septo-optic dysplasia, first described by de Morsier in 1956, is an uncommon congenital midline structural abnormality of the brain, including agenesis of septum pellucidum, primitive optic ventricle and dysplasia of the optic chiasm, optic nerves, and infundibulum. Agenesis of septum pellucidum, initially thought to be an integral part of this syndrome, is inconstant and not an essential part of the disorder. Also, it reveals a variable spectrum of associated abnormalities in disturbed hypothalamic-pituitary function. We recently experienced 3 patients of septo-optic dysplasia who showed bilateral optic disc hypoplasia clinically and midline structural abnormalities in computerized tomography(CT) or magnetic resonance imaging(MRI).