What is the treatment for Sheehan syndrome?

Treatment of Sheehan Syndrome

The treatment for Sheehan syndrome consists of hormone replacement therapy targeting all deficient pituitary hormones, with glucocorticoid replacement being the most urgent priority to prevent life-threatening adrenal crisis.

Understanding Sheehan Syndrome

Sheehan syndrome is characterized by hypopituitarism resulting from ischemic necrosis of the pituitary gland following severe postpartum hemorrhage. The enlarged pituitary gland during pregnancy is particularly vulnerable to ischemic damage during hemorrhagic shock 1.

Key Clinical Features

• History of severe postpartum hemorrhage
• Failure to lactate after delivery
• Amenorrhea or oligomenorrhea
• Fatigue, weakness, and cold intolerance
• Hypotension and hypoglycemia (in severe cases)
• Empty sella on MRI

Treatment Algorithm

1. Immediate Management

• Glucocorticoid replacement (highest priority):
  • Hydrocortisone 15-20 mg in the morning and 5-10 mg in the afternoon 1
  • For adrenal crisis: IV hydrocortisone 100 mg bolus followed by 50-100 mg every 6-8 hours 2

2. Subsequent Hormone Replacement (in order of clinical importance)

• Thyroid hormone replacement:

  • Levothyroxine (L-T4) starting at 25-50 μg/day, titrated based on free T4 levels 3, 4
  • Note: Always start thyroid replacement AFTER glucocorticoids to prevent precipitating adrenal crisis

• Sex hormone replacement (if hypogonadism present):

  • Females: Estrogen with progesterone for menstruating women
  • Males: Testosterone replacement

• Growth hormone replacement:

  • Consider in patients with confirmed GH deficiency
  • Particularly beneficial for improving quality of life, body composition, and bone mineral density 4

3. Management of Complications

• Sodium and water disturbances:

  • Monitor and correct hyponatremia, which is common in Sheehan syndrome 5
  • Fluid restriction for dilutional hyponatremia
  • Salt supplementation for hypovolemic hyponatremia

• Cardiovascular complications:

  • Screen for and manage pericardial effusion or cardiac tamponade 3
  • Adequate hormone replacement often resolves cardiac manifestations

Monitoring and Follow-up

• Regular assessment of hormone replacement adequacy:

  • Cortisol levels (or clinical assessment of symptoms)
  • Thyroid function tests (TSH, free T4)
  • Sex hormones
  • Electrolytes, particularly sodium levels

• Annual evaluation of bone mineral density

• Regular assessment of quality of life and cognitive function

Special Considerations

• Acute illness or surgery: Increase glucocorticoid dose (typically 2-3 times maintenance dose) 2
• Pregnancy: Requires close monitoring and adjustment of hormone replacement
• Osteoporosis prevention: Consider calcium and vitamin D supplementation

Pitfalls to Avoid

1. Initiating thyroid replacement before glucocorticoids, which can precipitate adrenal crisis
2. Failing to recognize atypical presentations (cardiac manifestations, electrolyte disturbances)
3. Inadequate stress dosing of glucocorticoids during illness or surgery
4. Missing the diagnosis in patients with subtle symptoms years after delivery

Early diagnosis and appropriate hormone replacement therapy are essential to prevent life-threatening complications and improve quality of life in patients with Sheehan syndrome.