Hypothalamic-pituitary function in children with optic nerve hypoplasia
G. Costin and A. L. Murphree
We studied the clinical characteristics and hypothalamic-pituitary function
in 23 patients with optic nerve hypoplasia (ONH), 6 months to 19 years old.
All patients had decreased visual acuity and small optic discs; the septum
pellucidum was absent in five of 19. Nine of 11 patients had minor EEG
abnormalities, and two had microcephaly. The height, weight, and growth
rate were normal in all patients 6 months to 3 1/2 years old.
Hypopituitarism was found in 15 patients. Fasting and stimulated prolactin
levels and the area under the prolactin curve after thyrotropin releasing
hormone were significantly greater than in controls and in patients with
idiopathic hypopituitarism. These results associate ONH with a high
incidence of hypopituitarism, hyperprolactinemia, and neurologic
abnormalities. The normal growth in the absence of measurable growth
hormone suggests that hyperprolactinemia may stimulate growth temporarily
and that a normal height in childhood or the presence of the septum
pellucidum do not exclude the possibility of hypopituitarism.
