Acute pituitary disease in pregnancy: how to handle hypophysitis and Sheehan's syndrome.

Abstract

Pregnancy-related hypophysitis (PR-Hy) that typically occurs during late pregnancy and the early postpartum period is considered an autoimmune disorder. Knowledge and awareness of characteristic clinical symptoms and specific endocrine and neuroradiological features mostly enables a correct diagnosis and guarantees best possible management. In the presence of mild clinical symptoms, a "wait-and-watch" approach is recommended. Transsphenoidal surgery or glucocorticoid therapy is indicated if progressive clinical signs or severe visual symptoms are observed. Reported cases indicate that transsphenoidal surgery performed during pregnancy can reverse visual symptoms and has a minimal risk to the health of the fetus and mother. Glucocorticoid therapy has a high initial response rate, but recurrence can occur after discontinuation. In late-pregnancy hypophysitis, the options of delaying therapy until the child is born or, if symptoms are severe, of inducing delivery prior to therapy have to be considered. Sheehan's syndrome (SS) is characterized by irreversible, partial or complete hypopituitarism due to pituitary infarction. The predominating causative event is massive peripartum hemorrhage (PPH). A considerable diagnostic delay is mostly observed. PPH and the typical postpartum findings of agalactia and amenorrhea should alert for the diagnosis of SS and prompt endocrine evaluation. Timely hormone replacement therapy is paramount to reduce morbidity and mortality and improve quality of life of afflicted women. Adrenal failure is a major endocrinological risk both of hypophysitis and of Sheehan syndrome. Early recognition and treatment is paramount to avoid life-threatening adrenal crisis.