Journal of the Korean Ophthalmological Society 1991;32(3):327-330.
Published online March 1, 1991.
Septo-optic dysplasia.
Woo Jung Kim, Young Suk Yu, Bong Leen Chang
Department of Ophthalmology, College of Medicine Seoul National University, Seoul, Korea.
중격 시신경 형성 장애
김우중(Woo Jung Kim),장봉린(Bong Leen Chang),유영석(Young Suk Yu)
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).
Key Words: Septo-optic dysplasia;septum pellucidum;optic disc hypoplasia;hypothalamic ipituitary function


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