Summary
The clinical and radiological findings in four children with septo-optic dysplasia are reported. All four had growth retardation associated with growth hormone deficiency, as well as varying degrees of ophthalmologic dysfunction. The CT scan findings spanned a spectrum from normal to the expected abnormalities involving the ventricular system and orbits. Only two of the four had an absent septum pellucidum. A third patient had normal CT scans except for optic nerve hypoplasia, while the fourth had entirely normal CT scans of both the brain and orbits. Because the anatomic defects of septo-optic dysplasia may be subtle, an apparently normal CT scan does not invalidate the clinical diagnosis of septo-optic dysplasia.
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Smith DW (1982) Recognizable patterns of human malformation. Saunders, Philadelphia, pp 466–467
Hale BR, Rice P (1974) Septo-optic dysplasia: clinical and embryological aspects. Develop Med Child Neurol 16:812–820
Zaias B, Becker D (1978) Septo-optic dysplasia developmental or acquired abnormality? A case report. Trans Am Neurol Assoc 103:273–277
Patel H, Tze WJ, Crichton JU, McCormickAQ, Robinson GC, Dolman CL (1975) Optic nerve hypoplasia with hypopituitarism. Am J Dis Child 129:175–180
Huseman CA, Kelch RP, Hopwood NJ, Zipf WB (1978) Sexual precocity in association with septo-optic dysplasia and hypothalamic hypopituitarism. J Pediatr 92:748–753
Krause-Brucker W, Gardner DW (1980) Optic nerve hypoplasia associated with absent septum pellucidum and hypopituitarism. Am J Ophthalmol 89:113–120
Mannelfe C, Rochiccioli P (1979) CT of septo-optic dysplasia. AJR 133:1157–1160
Hamill PVV, (1978) NCHS growth curves for children Vital Health Stat, Series 11:165
Kaplan SA (ed) (1982) Clinical pediatric and adolescent endocrinology. Saunders, Philadelphia, pp 32–33
Elster AB, McAnarney ER (1979) Maternal age re-septo-optic dysplasia. J Pediatr 94:162–163
Purdy F, Friend JCM (1979) Maternal factors re-septo-optic dysplasia. J Pediatr 95:661
Costin G, Roe TF Thomas DW, Sinatra FR, Neustein HB (1983) Neonatal hepatitis-cholestasis (NHC) with optic nerve hypoplasia (ONH). Clin Res 31: 106A (abstract)
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Wilson, D.M., Enzmann, D.R., Hintz, R.L. et al. Computed tomographic findings in septo-optic dysplasia: Discordance between clinical and radiological findings. Neuroradiology 26, 279–283 (1984). https://doi.org/10.1007/BF00339771
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DOI: https://doi.org/10.1007/BF00339771