J Korean Child Neurol Soc.  1998 May;5(2):393-397.

A Case of Septo-Optic Dysplasia

Abstract

Septo-optic dysplasia, as first described by de Morsier, is a rare developmental anomaly of absent or hypoplastic septum pellucidum, a primitive optic vesicle, and dysplasia of the optic nerve, chiasm, and optic tracts. It is a common cause of hypopituitarism in children and has variable hypothalamic-pituitary dysfunction. We experienced a case of septo-optic dysplasia in 5 month old female baby who presented with developmental delay and seizure. A magnetic resonance imaging of the brain showed absence of the septum pellucidum and the fundoscopic examination disclosed the absence of left optic disc and atrophy of right optic disc.


MeSH Terms

Atrophy
Brain
Child
Female
Humans
Hypopituitarism
Infant
Magnetic Resonance Imaging
Optic Nerve
Seizures
Septo-Optic Dysplasia*
Septum Pellucidum
Visual Pathways
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