Sheehan syndrome
Sheehan syndrome is postpartum pituitary infarction after massive obstetric hemorrhage, causing panhypopituitarism with failure of lactation and deficiencies of glucocorticoids, thyroid hormone, and sex hormones. First-line treatment is glucocorticoid replacement started before thyroid hormone, followed by levothyroxine and, when indicated, sex hormone and growth hormone replacement.
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What this article covers
- Overview
Definition: Sheehan syndrome is panhypopituitarism resulting from ischemic infarction of the pituitary gland in the setting of massive postpartum hemorrhage.
- Epidemiology
A recognized but infrequent cause of hypopituitarism: pituitary infarction, including Sheehan syndrome, is classified as an infrequent cause of primary pituitary disease among the broader causes of hypopituitarism.
- Risk factors
Massive postpartum hemorrhage: severe obstetric hemorrhage with hypovolemia is the essential precipitating event for pituitary infarction in Sheehan syndrome.
- Pathophysiology
Ischemic infarction mechanism: the enlarged, high-blood-flow pituitary gland of late pregnancy is supplied by a low-pressure portal venous system that is particularly vulnerable to hypoperfusion…
- Clinical features
Diagnostic core feature: the defining clinical pattern is failure of lactation (agalactia) in a woman with a history of massive postpartum hemorrhage, together with evidence of deficiency across multiple anterior pituitary hormone axes.
- Differential diagnosis
Lymphocytic hypophysitis: presumed autoimmune pituitary inflammation can also present postpartum with hypopituitarism or a sellar mass, but it is often associated with hyperprolactinemia from stalk…
- Diagnosis
Diagnostic basis: Sheehan syndrome is diagnosed clinically in a patient with a history of massive postpartum hemorrhage who develops agalactia and biochemical evidence of deficiency across two or…
- Management
First, start glucocorticoid replacement immediately once ACTH deficiency is suspected or confirmed: hydrocortisone 15-20 mg PO daily in two divided doses is the maintenance replacement for secondary…
- Complications
Adrenal crisis: unrecognized or inadequately treated ACTH deficiency can progress to life-threatening adrenal crisis during physiologic stress, infection, or surgery.
- Prognosis
Generally good with prompt hormone replacement: patients who receive appropriately sequenced and adequately dosed hormone replacement can expect a normal life expectancy and substantial symptomatic improvement.
- Prevention
Prevention and prompt treatment of postpartum hemorrhage: aggressive obstetric management of hemorrhage and hypovolemia during delivery reduces the risk of pituitary infarction.
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- Secondary adrenal insufficiency
Secondary adrenal insufficiency is ACTH deficiency from pituitary or hypothalamic disease, or most commonly from withdrawal of prolonged exogenous glucocorticoid therapy, causing isolated cortisol deficiency without hyperpigmentation or hyperkalemia because aldosterone secretion is preserved. Lifelong glucocorticoid replacement with hydrocortisone, with dose increases during illness, prevents adrenal crisis.
- Pituitary apoplexy
Pituitary apoplexy is sudden hemorrhage or infarction of a pituitary adenoma causing severe headache, visual loss, ophthalmoplegia, and acute adrenocorticotropic hormone (ACTH) deficiency, and is a medical emergency. First-line treatment is immediate IV hydrocortisone given empirically before confirmatory testing when adrenal insufficiency is suspected, with urgent pituitary MRI and surgical decompression reserved for progressive visual loss.
- Lymphocytic hypophysitis
Lymphocytic hypophysitis is a presumed autoimmune inflammation of the pituitary gland that typically presents in women during pregnancy or the postpartum period, with a sellar mass, hyperprolactinemia, or hypopituitarism. There is no specific therapy; management is hormonal replacement of any deficient axis as needed, with surgery reserved for mass effect that does not respond to observation.
- Hypothyroidism
Hypothyroidism is thyroid hormone deficiency, most commonly caused by autoimmune (Hashimoto) thyroid failure in iodine-sufficient countries, diagnosed by an elevated thyroid-stimulating hormone with a low or normal free thyroxine. First-line treatment is levothyroxine dosed at 1.6 mcg/kg lean body weight orally once daily, titrated using thyroid-stimulating hormone rechecked no sooner than 6 weeks after any dose change.