[Amelogenesis imperfecta and hypothalamo-hypophyseal insufficiency]

Eleven patients with hypopituitarism, medio-facial hypoplasia and amelogenesis imperfecta were examined. The hypothalamo-pituitary dysfunction affected somatotrophic, gonadotrophic, corticotrophic and thyrotrophic function. The facial anomalies included medio-facial hypoplasia with hypertelorism and amelogenesis imperfecta. The latter--hypoplastic in nature--was noted in all anterior teeth and affected both dentitions. The enamel defect was confirmed on microscopic examination of an impacted tooth. The spectrum of malformation may also present as amelogenesis imperfecta in association with somatotropin deficiency. A genetic mechanism may possibly account for the clinical elements.