Hypothalamic-Pituitary Functions in Patients with Transsphenoidal Encephalocele and Midfacial Anomalies*

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Abstract
Hypothalamic-pituitary functions were studied in 2 patients with transsphenoidal pituitary herniation associated with midfacial anomalies. Both had a defect in the floor of the sella turcica. The male patient had short stature and a low plasma growth hormone level with no response to arginine infusion and insulin-induced hypoglycemia. Both patients were euthyroid and showed normal plasma thyrotropin responses to TRH [thyroliberin]. Serum PRL [prolactin] levels were borderline high in both, and 1 patient had a delayed, but excessive, PRL response to TRH. Neither patient had evidence of adrenal insufficiency, hypogonadotropic-hypogonadism, diabetes insipidus or a visual field defect. One patient had mild to moderate mental retardation. The parents of the patients were consanguineous. The anatomy of the sella turcica and hypothalamic-pituitary functions must be evaluated in patients with midfacial anomalies.

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