Pituitary and hypothalamic dysfunction in a patient with a basal encephalocele.

A 31 year old Chinese man was found to have a large basal encephalocele extending into the nasopharynx associated with a cleft palate and chiasmal damage producing visual field defects. He was found to have marked hypothalamic-pituitary dysfunction including deficiencies of growth hormone, thyrotropin, adrenocorticotropic hormone and gonadotropins, diabetes insipidus, adipsia with essential hypernatremia and poikilothermia. This man illustrates the importance of careful assessment of patients with craniofacial anomalies and outlines a simple investigational protocol.