Hypothalamic-hypophyseal dysgenesis as a neuroimaging correlate of pituitary hormone deficiency in anophthalmia

Am J Ophthalmol. 1996 Nov;122(5):747-8. doi: 10.1016/s0002-9394(14)70505-7.

Abstract

Purpose: To document the association of neurohypophyseal dysgenesis with hypopituitarism in a child with primary bilateral anophthalmia.

Methods: An infant with bilateral anophthalmia underwent magnetic resonance imaging and endocrinologic evaluation.

Results: Magnetic resonance imaging showed dysgenesis confined to the hypothalamus and hypophyseal stalk. Endocrinologic testing showed low serum cortisol and pituitary gonadotropin levels.

Conclusion: Magnetic resonance imaging can help predict which children with anophthalmia will have endocrinologic deficiencies.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple / blood
  • Abnormalities, Multiple / genetics*
  • Anophthalmos / blood
  • Anophthalmos / genetics*
  • Gonadotropins, Pituitary / blood
  • Gonadotropins, Pituitary / deficiency
  • Humans
  • Hydrocortisone / blood
  • Hydrocortisone / deficiency
  • Hypopituitarism / blood
  • Hypopituitarism / diagnosis*
  • Hypopituitarism / genetics
  • Hypothalamo-Hypophyseal System / abnormalities*
  • Hypothalamo-Hypophyseal System / pathology*
  • Infant, Newborn
  • Magnetic Resonance Imaging
  • Male
  • Pituitary Hormones / blood
  • Pituitary Hormones / deficiency*

Substances

  • Gonadotropins, Pituitary
  • Pituitary Hormones
  • Hydrocortisone