Sheehan's syndrome

Overview

Sheehan's syndrome is a rare but serious condition characterized by hypopituitarism (underfunctioning of the pituitary gland) that occurs due to severe blood loss or extremely low blood pressure during or after childbirth. This hemorrhagic event can lead to infarction (death) of pituitary tissue, particularly affecting the anterior pituitary gland. Since the pituitary controls multiple hormones essential for bodily functions, Sheehan’s syndrome can lead to a wide range of hormonal deficiencies, impacting the thyroid, adrenal glands, reproductive system, and more. The onset of symptoms can be immediate or delayed, sometimes emerging years after the inciting event.

Causes

The primary cause of Sheehan’s syndrome is ischemic necrosis (tissue death from lack of blood flow) of the pituitary gland due to severe postpartum hemorrhage. During pregnancy, the pituitary gland naturally enlarges and becomes more vulnerable to hypoperfusion. Factors that can contribute to this include:

• Severe postpartum hemorrhage: Excessive blood loss during or after delivery is the most significant risk factor

• Hypotension during childbirth: Critically low blood pressure may impair blood flow to the pituitary

• Complicated deliveries: Especially those involving placental abruption or uterine rupture

• Delayed medical intervention: Inadequate management of obstetric emergencies increases the risk

Sheehan’s syndrome is more common in areas with limited access to maternal healthcare and blood transfusion services.

Symptoms

Symptoms of Sheehan’s syndrome vary based on the extent of pituitary damage and the specific hormones affected. They may develop acutely or insidiously over time. Common symptoms include:

Early/Postpartum Symptoms:

• Failure to lactate (due to prolactin deficiency)

• Amenorrhea or oligomenorrhea (absence or irregularity of menstruation)

• Fatigue and weakness

• Low blood pressure and hypoglycemia (due to cortisol deficiency)

Delayed or Chronic Symptoms:

• Cold intolerance

• Loss of pubic and axillary hair

• Weight gain or difficulty losing weight

• Depression or cognitive slowing

• Dry skin and constipation (due to hypothyroidism)

• Infertility or decreased libido

The severity of symptoms depends on which and how many pituitary hormones are affected, including ACTH, TSH, LH, FSH, prolactin, and growth hormone.

Diagnosis

Diagnosing Sheehan’s syndrome involves a detailed medical history, focusing on childbirth complications, and laboratory testing to assess pituitary function. Diagnostic steps include:

• Patient history: Key factors include severe postpartum hemorrhage, lactation failure, and menstrual irregularities

• Hormonal blood tests: To measure pituitary and target gland hormone levels, including:

  • Low cortisol and ACTH

  • Low TSH and free T4

  • Low LH, FSH, and estradiol

  • Low prolactin and growth hormone

• MRI of the brain: To visualize pituitary atrophy, empty sella, or other structural changes

Early diagnosis is often missed unless a high index of suspicion is maintained, especially in women with subtle or delayed symptoms following childbirth.

Treatment

Treatment of Sheehan’s syndrome centers on lifelong hormone replacement therapy to restore deficient hormones and manage symptoms. Main treatment approaches include:

Hormone Replacement Therapy:

• Glucocorticoids: Hydrocortisone or prednisone to replace cortisol (must be initiated before thyroid hormone)

• Thyroid hormone: Levothyroxine for central hypothyroidism

• Sex hormones: Estrogen and progesterone (for women) or testosterone (for men) to manage reproductive hormone deficiencies

• Growth hormone: Considered in selected patients for improving energy, metabolism, and quality of life

Supportive Measures:

• Stress-dose steroids during illness or surgery

• Monitoring and adjusting hormone doses regularly

• Patient education regarding emergency steroid use and identification

Prognosis

With appropriate and consistent hormone replacement therapy, the prognosis for individuals with Sheehan’s syndrome is generally good. Most women can manage the condition effectively and maintain a reasonable quality of life. However, untreated or inadequately treated Sheehan’s syndrome can lead to severe complications, including adrenal crisis, hypothyroid coma, and infertility.

Early diagnosis, patient compliance with treatment, and regular medical follow-up are essential for preventing complications and improving long-term outcomes. Awareness among healthcare providers and postpartum screening in high-risk women can help detect the condition earlier and reduce its impact.