Sheehan Syndrome

Content of This Page

1- Introduction

2- Causes

3- Symptoms

4- Investigations & Lab Results

5- Prognosis

6- Treatment

Introduction

Sheehan Syndrome is a rare endocrine disorder that occurs in women, typically as a result of severe blood loss or extremely low blood pressure during or after childbirth. This postpartum complication leads to ischemic necrosis (tissue death) of the pituitary gland, which causes hypopituitarism—a deficiency in one or more of the hormones normally produced by the anterior pituitary.

The condition arises because the pituitary gland enlarges during pregnancy and becomes more vulnerable to reduced blood flow. If a woman experiences massive postpartum hemorrhage or shock, the reduced perfusion can damage the gland. As a result, hormone production declines, potentially affecting growth hormone (GH), prolactin, adrenocorticotropic hormone (ACTH), thyroid-stimulating hormone (TSH), luteinizing hormone (LH), and follicle-stimulating hormone (FSH).

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Causes

  • Severe postpartum hemorrhage (excessive bleeding during or after childbirth)

  • Resulting hypotension (low blood pressure) and shock causing decreased blood flow to the pituitary gland

  • Ischemic necrosis (tissue death) of the enlarged anterior pituitary due to insufficient blood supply

  • Pituitary enlargement during pregnancy increases vulnerability to ischemic injury

  • Less common causes:

    • Severe hypotension from other causes (e.g., infection, trauma)

    • Preexisting pituitary abnormalities

    • Coagulation disorders worsening bleeding

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Symptoms

  • Failure to lactate (inability to produce breast milk) soon after delivery

  • Amenorrhea (absence of menstruation) or irregular periods

  • Fatigue and weakness

  • Loss of pubic and axillary hair

  • Cold intolerance (due to hypothyroidism)

  • Hypotension (low blood pressure), sometimes causing dizziness or fainting

  • Weight loss

  • Depression or poor concentration

  • Hypoglycemia (low blood sugar) episodes

  • Signs of adrenal insufficiency such as nausea, vomiting, and abdominal pain

  • Delayed or absent recovery after childbirth complications

Investigations & Lab Results

  • Serum cortisol ↓ (low, due to decreased ACTH)
  • ACTH ↓ (low or inappropriately normal)
  • TSH (thyroid-stimulating hormone) ↓ or → (low or normal despite low thyroid hormones)
  • Free T4 (thyroxine) ↓ (low, indicating central hypothyroidism)
  • LH and FSH ↓ (low, causing hypogonadism)
  • Prolactin ↓ (low, leading to failure of lactation)
  • Growth hormone (GH) ↓ (low)
  • Blood glucose ↓ (hypoglycemia possible due to cortisol deficiency)
  • Serum sodium → or ↓ (may be low if adrenal insufficiency causes hyponatremia)
  • MRI of pituitary gland → or shows empty sella or atrophic pituitary (indicating pituitary necrosis)

Prognosis

  • Sheehan Syndrome is a chronic condition because the damage to the pituitary gland is usually permanent. However, with timely diagnosis and appropriate lifelong hormone replacement therapy, the prognosis is generally good, allowing affected women to lead relatively normal lives.

     

    Key Prognostic Points:

    • Lifelong hormone replacement (glucocorticoids, thyroid hormones, sex hormones) is essential to manage symptoms and prevent life-threatening complications like adrenal crisis.

    • Early treatment improves quality of life and reduces risks related to hormone deficiencies.

    • Without treatment, patients risk severe complications such as adrenal insufficiency, hypothyroidism, infertility, and increased mortality.

    • Some pituitary functions rarely recover spontaneously; most patients require lifelong follow-up and therapy.

    • Fertility can sometimes be restored with appropriate hormone therapy and assisted reproductive techniques

Treatment

  1. Glucocorticoid Replacement

    • Typically hydrocortisone or prednisone to replace cortisol (due to low ACTH).

    • Essential before starting thyroid hormone therapy to avoid adrenal crisis.

  2. Thyroid Hormone Replacement

    • Levothyroxine to treat central hypothyroidism caused by low TSH.

  3. Sex Hormone Replacement

    • Estrogen and progesterone in women to induce and maintain secondary sexual characteristics and regulate menstrual cycles.

    • Can improve bone health and quality of life.

  4. Growth Hormone Replacement (optional)

    • Sometimes given if GH deficiency causes symptoms like fatigue and poor quality of life.

  5. Management of Electrolyte Imbalances and Hypoglycemia

    • Supportive care as needed, especially during acute illness or stress.

  6. Patient Education and Regular Monitoring

    • Lifelong follow-up to adjust hormone doses.

    • Monitoring for complications like osteoporosis.

  7. Fertility Treatment

    • Possible with appropriate hormonal therapy and assisted reproductive techniques.

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