Evolving hypopituitarism in children with central nervous system lesions

At the time children with suspected hypopituitarism are seen with a subnormal growth rate, it is expected that testing will demonstrate growth hormone (hGH) deficiency. Seven patients with organic CNS lesions - three with histiocytosis X, one with septo-optic dysplasia, one with neonatal meningitis, one with an anterior encephalocele and meningitis, and one with neurofibromatosis who had normal growth hormone concentrations (> 7 ng/mL) despite a subnormal growth rate - were studied. Subsequent retesting 0.5 to 4.6 years later demonstrated the development of growth hormone deficiency. Four of the patients had evidence of other pituitary hormone deficiencies at the time of initial testing whereas two subsequently developed other deficiencies. The initial subnormal growth rate in these children may be secondary to their organic CNS lesion or an evolving hypopituitarism, perhaps with deficient somatomedin generation. These studies point out the need for continued observation and retesting in such patients.