ISSN:
1432-1076
Keywords:
Hypopituitarism
;
Transsphenoidal meningoencephalocele
;
Midline craniocerebral and midfacial anomaly
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
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.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00442087
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