What are the key points of Sheehan syndrome (postpartum pituitary necrosis)?

From the Guidelines

Sheehan syndrome is a rare but potentially life-threatening condition that requires prompt recognition and treatment to prevent long-term morbidity and mortality, and its key points include etiology, clinical manifestations, diagnosis, and treatment, with a focus on hormone replacement therapy and regular monitoring to improve quality of life. The etiology of Sheehan syndrome stems from ischemic damage to the pituitary gland during childbirth complications, particularly when significant blood loss occurs, as seen in placenta accreta spectrum patients who are at risk of ongoing abdominopelvic bleeding, fluid overload, and other postoperative complications 1. Clinical manifestations vary widely, from subtle symptoms to severe hypopituitarism, including failure to lactate, amenorrhea, fatigue, hypotension, hypoglycemia, and inability to respond to stress. Diagnosis requires a history of postpartum hemorrhage combined with laboratory evidence of pituitary hormone deficiencies, particularly low levels of prolactin, growth hormone, ACTH, TSH, FSH, and LH.

Key points in the management of Sheehan syndrome include:

• Lifelong hormone replacement therapy tailored to the specific hormonal deficiencies
• Glucocorticoids (hydrocortisone 15-25 mg daily in divided doses) to replace cortisol
• Thyroid hormone (levothyroxine 1.6 mcg/kg/day) to replace thyroid hormone
• Estrogen-progesterone replacement for premenopausal women to replace sex hormones
• Growth hormone replacement if indicated
• Regular monitoring of hormone levels and clinical response, with dose adjustments as needed
• Patient education about stress dose adjustments for glucocorticoids during illness
• Medical alert identification to alert healthcare providers of the patient's condition. Early recognition and treatment are crucial to prevent life-threatening complications, such as adrenal crisis, and improve quality of life, as highlighted in the management of postpartum complications, including placenta accreta spectrum 1.

From the Research

Definition and Causes of Sheehan Syndrome

• Sheehan syndrome is a rare cause of hypopituitarism characterized by pituitary gland necrosis after postpartum hemorrhage 2, 3, 4, 5.
• The initial insult is caused by massive postpartum haemorrhage (PPH), leading to impaired blood supply to the pituitary gland, which has become enlarged during pregnancy 4.
• Small sella turcica size, vasospasms (caused by PPH) and/or thrombosis (associated with pregnancy or coagulation disorders) are predisposing factors; autoimmunity might be involved in the progressive worsening of pituitary functions 4.

Symptoms and Presentation

• Symptoms are caused by a decrease or absence of one or more of the pituitary hormones, and vary, among others, from failure to lactate and nonspecific symptoms (such as fatigue) to severe adrenal crisis 4.
• The secretion of growth hormone and prolactin is most commonly affected, followed by follicle-stimulating hormone and luteinizing hormone; severe necrosis of the pituitary gland also affects the secretion of thyroid-stimulating hormone and adrenocorticotropic hormone 4.
• Symptoms usually become evident years after delivery, but can, in rare cases, develop acutely 2, 4.
• Presenting signs varied, including adrenal insufficiency, diabetes insipidus, hypothyroidism, and panhypopituitarism 2.

Diagnosis and Treatment

• Diagnosis is based on clinical manifestations combined with a history of severe PPH; hormone levels and/or stimulation tests can confirm clinical suspicion 4, 5.
• Low basal or stimulated pituitary hormones along with classic clinical setting clinches the diagnosis 5.
• Hormone replacement therapy is the only available management option so far 4, 5.
• Replacement of deficient hormones is the mainstay of treatment, however emphasis on better obstetric care to common masses would help to eradicate the disease in future 5.

Incidence and Importance

• The incidence of Sheehan syndrome depends, to a large extent, on the occurrence and management of PPH 4.
• Sheehan syndrome is an important cause of hypopituitarism in developing countries, but has become rare in developed countries 4.
• It is a frequent cause of hypopituitarism in developing countries that occurs secondary to postpartum haemorrhage (PPH) 6.
• Strict vigilance, timely diagnosis, and appropriate management are essential to avoid diagnostic delay and to improve the patient outcome 6.