What are the symptoms of Sheehan syndrome?

Symptoms of Sheehan Syndrome

Sheehan syndrome presents with a spectrum of symptoms resulting from pituitary hormone deficiencies following postpartum hemorrhage, with failure to lactate and amenorrhea being the most common initial manifestations.

Pathophysiology and Risk Factors

Sheehan syndrome occurs due to ischemic necrosis of the pituitary gland following severe postpartum hemorrhage (PPH). Several factors contribute to its development:

• Enlarged pituitary gland during pregnancy (more vulnerable to ischemia)
• Small sella turcica size
• Vasospasms caused by PPH
• Thrombosis associated with pregnancy or coagulation disorders
• Disseminated intravascular coagulation
• Possible autoimmune mechanisms 1, 2, 3

Clinical Presentation

Acute Symptoms (Immediate Postpartum Period)

• Severe headache (may be the first symptom) 4
• Visual disturbances
• Signs of hemodynamic instability related to PPH
• Failure to lactate (agalactia) due to prolactin deficiency 2, 3

Subacute and Chronic Symptoms

1. Reproductive System:

   • Failure to resume menstruation (amenorrhea) 2, 3
   • Decreased axillary and pubic hair
   • Loss of libido
   • Vaginal dryness

2. General Symptoms:

   • Fatigue and weakness
   • Cold intolerance
   • Dry skin
   • Weight changes (usually weight loss)
   • Mood changes and depression 4

3. Endocrine Deficiencies (in order of frequency):

   • Growth hormone and prolactin deficiency (most common) 2, 5
   • Gonadotropin deficiency (FSH/LH)
   • Thyroid-stimulating hormone (TSH) deficiency
   • Adrenocorticotropic hormone (ACTH) deficiency (can lead to adrenal crisis) 1

4. Other Clinical Manifestations:

   • Anemia or pancytopenia
   • Osteoporosis
   • Impaired cognitive function
   • Reduced quality of life 2, 5

Timing of Symptom Onset

The presentation of Sheehan syndrome can vary in timing:

• Acute onset: Rare but can develop immediately after delivery with severe hypopituitarism 1
• Delayed onset: More commonly, symptoms develop gradually over months to years after delivery 3

Severity Spectrum

The clinical manifestations can range from:

• Mild disease with single pituitary hormone deficiency
• Partial hypopituitarism with multiple hormone deficiencies
• Complete panhypopituitarism in severe cases 2

Diagnostic Clues

The most important diagnostic clues include:

• History of severe postpartum hemorrhage (though rarely can occur without massive bleeding) 3, 4
• Failure to lactate after delivery
• Amenorrhea or oligomenorrhea
• Non-specific symptoms like fatigue, weakness, and cold intolerance

Complications

If left untreated, Sheehan syndrome can lead to:

• Adrenal crisis (life-threatening)
• Severe hypothyroidism
• Osteoporosis with increased fracture risk
• Anemia and other hematological abnormalities
• Impaired quality of life 2, 5

Important Considerations

• Symptoms may be subtle and progress slowly, leading to delayed diagnosis
• The condition is more common in developing countries but can occur anywhere
• A high index of suspicion is needed in women with a history of PPH who present with failure to lactate, amenorrhea, and other symptoms of hypopituitarism 6, 3

Early recognition of these symptoms is crucial for prompt diagnosis and appropriate hormone replacement therapy to prevent long-term complications and improve quality of life.