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A case of septo-optic dysplasia.

Journal of the Korean Pediatric Society 1991;34(12):1707-1711.
Published online December 31, 1991.
A case of septo-optic dysplasia.
Su Kyung Kang, Sung Hee Kim, Myoung Jae Chey, Hak Soo Lee
Department of Pediatrics, Gil General Hospital, Incheon, Korea
Septo-Optic Dysplasia 1 례
강수경, 김성희, 최명재, 이학수
중앙길병원 소아과
Received: 15 June 1991   • Accepted: 29 August 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.
Key Words: Septo-Optic dysplasia


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