Abstract
A 12-year-old boy with growth hormone deficiency and partial diabetes insipidus resulting from transsphenoidal meningoencephalocele and with eye abnormalities is described. Fifteen other patients with transsphenoidal meningoencepalocele have been reported. Hypothalamic-pituitary dysfunctions were diagnosed by endocrinological studies in seven cases. It is important to recognize transsphenoidal meningoencephalocele as a cause of hypopituitarism, since some cases may have gone unrecognized. The association of hypothalamic-pituitary dysfunction and a midline craniocerebral anomaly has been reported in patients with cleft lip and/or palate, septo-optic dysplasia, the holoprosencephalies, and Kallmann syndrome. However, there was no evidence of transsphenoidal meningoencephalocele in these disorders and this may be a different form of midline craniocerebral and midfacial anomaly.
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References
Avanzini G, Grivelli G (1970) A case of sphenopharyngeal encephalocele. Acta Neurochirurgia 22:205–212
Danoff D, Sebru J, French LA (1965) Encephalocele extending into the sphenoid sinus. Report of a case. J Neurosurg 24:684–686
Ellyin F, Khatir AH, Singh SP (1980) Hypothalamic-pituitary functions in patients with transsphenoidal encephalocele and midfacial anomalies. J Clin Endocrinol Metab 51:854–856
Francés M, Knorr D, Martinez R, Neuhäuser G (1966) Hypophysärer Zwergwuchs bei Lippen-Kiefer-Spalte. Helv Paediatr Acta 21:315–322
Hinz RL, Menking M, Sotos JF (1968) Familial holoprosencephaly with endocrine dysgenesis. J Pediatr 72:81–87
Ingraham FD, Swan H (1943) Spina bifida and cranium bifidum. 1. A survey of five hundred and forty six cases. N Engl J Med 228:559–563
Laron Z, Sarel R (1970) Penis and testicular size in patients with growth hormone insufficiency. Acta Endocrinol 64: 625–633
Laron Z, Taube E, Kaplan I (1969) Pituitary growth hormone insufficiency associated with cleft lip and palate. Helv Paediatr Acta 24:576–581
Larsen JL, Bassøe HH (1979) Transsphenoidal meningocele with hypothalamic insufficiency. Neuroradiology 18:205–209
Lewin ML (1976) Spheno-pharyngeal meningocele and cleft palate. Case report with 12-year follow-up. Cleft Palate J 13:61–73
Lieblich JM, Rosen SW, Guyda H, Reardan J, Schaaf M (1978) The syndrome of basal encephalocele and hypothalamic-pituitary dysfunction. Ann Intern Med 89:910–916
Lovrenčić MK, Oberiter V, Banovac ZR, Schmutzer L, Petek M (1979) Pituitary function in a patient with septo-optic dysplasia and pituitary dwarfism (Kaplan-Grumbach-Hoyt syndrome). Eur J Pediatr 129:47–53
Pollock JA, Newton TH, Hoyt WF (1968) Transsphenoidal and transethmoidal encephalocele. Radiology 90:442–453
Roitman A, Laron Z (1978) Hypothalamo-pituitary hormone insufficiency associated with cleft lip and palate. Arch Dis Child 53:952–955
Rosen SW (1965) The syndrome of hypogonadism, anosmia and midline cranial anomalies. Abstract of the 47th meeting, The Endocrine Society New York, p 123
Rudman D, Davis T, Priest JH, Patterson JH, Kutner MH, Heymsfield SB, Bethel RA (1978) Prevalence of growth hormone deficiency in children with cleft lip or palate. J Pediatr 93:378–382
Shirataki K, Sakamoto K, Ohara Y (1976) Diagnosis and treatment of transsphenoidal encephalocele-Report of a case with review of literature. Brain Nerve 28:281–291 (Jpn)
Streletz LJ, Schatz NJ (1973) Transsphenoidal encephalocele associated with colobomas of the optic discs and hypopituitary dwarfism. In: Smith JI (ed) Neuro-Ophthalmology Symposium. St Louis, C. V. Mosby Company, pp 78–86
Van Nouhuys JM, Bruyn GW (1964) Nasopharyngeal transsphenoidal encephalocele, craterlike hole in the optic disc and agenesis of the corpus callosum, pneumoencephalographic visualisation in a case. Psychiat Neurol Neurochir 67:234–258
Walsh FB, Hoyt WF (1969) Clinical neuroophthalmology, vol 1. Williams and Wilkins, Baltimore, pp 715–719
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Nishi, Y., Muraki, K., Sakoda, K. et al. Hypopituitarism associated with transsphenoidal meningoencephalocele. Eur J Pediatr 139, 81–84 (1982). https://doi.org/10.1007/BF00442087
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DOI: https://doi.org/10.1007/BF00442087