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 |
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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.
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Key Words:
Septo-Optic dysplasia |
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