All issues > Volume 34(12); 1991

Original Article

J Korean Pediatr Soc. 1991;34(12):1707-1711. Published online December 31, 1991.

A case of septo-optic dysplasia.

Su Kyung Kang¹, Sung Hee Kim¹, Myoung Jae Chey¹, Hak Soo Lee¹

¹Department of Pediatrics, Gil General Hospital, Incheon, Korea

Received: June 15, 1991;  Accepted: August 29, 1991.

Abstract

Septo-optic dysplasia, which was first described by de Morsier in 1956, is a rare developmental anomaly of anterior midline structures of brain, combining agenesis of the septum pellucidum and hypoplasia of the optic nerves, chiasm, and infundibulum. It is considered a form of holoprosence- phaly. It is a common cause of hypopituitarism in children, and the pituitary hormone most commonly affected is growth hormone, but diabetes insipidus, sexual precocity, hypothyroidism and adrenocor- ticotropic hormone deficiency also occur. We have experienced a case of septo-optic dysplasia in 45-day old male who had generalized tonic-clonic seizure, hypoplasia of the optic nerves in fundoscopic finding and agenesis of the septum pellucidum in ultrasonogram and CT scan of brain.

Keywords :Septo-Optic dysplasia