Epidemiology


Etiology

  • Sheehan syndrome: postpartum necrosis of the pituitary gland. Usually occurs following postpartum hemorrhage, but can also occur even without clinical evidence of hemorrhage.
    • During pregnancy, hypertrophy of prolactin-producing regions increases the size of the pituitary gland, making it very sensitive to ischemia.
    • Blood loss during delivery/postpartum hemorrhage → hypovolemia → vasospasm of hypophyseal vessels → ischemia of the pituitary gland → empty sella turcica on imaging

Clinical features

  • ACTH deficiency (Secondary adrenal insufficiency):
    • Fatigue, weight loss, hypoglycemia, orthostatic hypotension.
    • No hyperpigmentation (unlike primary adrenal insufficiency, POMC/MSH is not elevated).
    • No hyperkalemia or salt-wasting (aldosterone synthesis is regulated by RAAS, not ACTH). c
    • Cortisol normally exerts negative feedback on ADH secretion. A deficiency in cortisol removes this inhibition, leading to increased ADH secretion (dilutional hyponatremia) and hypovolemic/euvolemic hyponatremia. c
  • TSH deficiency (Secondary hypothyroidism):
    • Fatigue, cold intolerance, constipation, dry skin, bradycardia, proximal muscle weakness.
  • LH/FSH deficiency (Hypogonadotropic hypogonadism):
    • Females: Amenorrhea, infertility, vaginal atrophy, loss of libido.
    • Males: Erectile dysfunction, testicular atrophy, loss of libido, decreased muscle mass.
  • GH deficiency:
    • Adults: Increased abdominal adiposity, decreased bone mineral density, vague fatigue.
    • Children: Short stature, severe growth deceleration.
  • PRL deficiency:
    • Inability to lactate postpartum (highly specific for Sheehan syndrome).
  • Pituitary Apoplexy presentation:
    • Sudden-onset, severe “thunderclap” headache, meningismus, fever.
    • Bitemporal hemianopsia (optic chiasm compression), ophthalmoplegia (CN III, IV, VI palsy).
    • Cardiovascular collapse due to acute, life-threatening ACTH deficiency.

Diagnostics


Treatment

  • Treat the underlying cause: For example, surgical resection of a pituitary adenoma.
  • Hormone Replacement Therapy: This is the cornerstone of treatment and is typically lifelong.
    • Glucocorticoids (hydrocortisone, prednisone): Replace for ACTH deficiency. This must be done FIRST before thyroid hormone replacement to prevent precipitating a life-threatening adrenal crisis.
    • Levothyroxine: Replace for TSH deficiency.
    • Sex Hormones: Testosterone for men; estrogen and progesterone for women.
    • Growth Hormone (somatropin): Used for children with GH deficiency and may be used in adults to improve body composition and quality of life.
    • Desmopressin (DDAVP): Used if there’s concomitant posterior pituitary damage leading to diabetes insipidus.