Growth and pubertal development in children with congenital and acquired hypothalamic-pituitary abnormalities

Abstract

Hypothalamic-pituitary disorders, either congenital or acquired, frequently affect the growth and pubertal development of children. Two distinct groups were investigated. The first group consisted of 70 patients with congenital optic nerve hypoplasia (ONH): 16 (73%) of the 22 patients had growth hormone deficiency; among the 21 patients who were old enough and had adequate data for pubertal evaluation, 2 (10%) had delayed or absent puberty and 4 (19%) had precocious puberty. In the second group, 14 children had undergone head irradiation and/or surgery for brain/orbital tumors. Of these, 21% had precocious puberty, 36% had delayed puberty, and 10 (90%) of 11 patients tested had growth hormone deficiency. The pubertal growth spurt can conceal hGH deficiency. Linear growth continues at a rate near normal for childhood but low for puberty. Careful monitoring of growth and early initiation of growth hormone replacement are essential to achieve a normal adult height in adolescents with hypothalamic-pituitary disease.