A case report of acute Sheehan syndrome with a review of 29 existing reports from 1990 to 2024: is it still considered a rare disease?

一例急性席汉氏综合征病例报告,并回顾了 1990 年至 2024 年的 29 篇现有报告:它仍然被认为是一种罕见病吗?

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Abstract

BACKGROUND: Sheehan's syndrome (SS) can occur as a consequence of massive postpartum hemorrhage (PPH). Although traditionally considered rare, acute SS may be underestimated, especially in resource-limited settings, where even minor obstetric complications other than the commonly recognized uterine atony could lead to significant clinical consequences. CASE REPORT: A 32-year-old pregnant woman (gravida 1, para 0) at 37 weeks and 6 days of gestation developed massive PPH complicated by hemorrhagic shock, cardiac arrest, and an emergent hysterectomy, with an estimated blood loss of 20 L. Despite initial stabilization, the patient experienced acute delirium and hyperpyrexia on postpartum day 9. Urgent brain magnetic resonance imaging (MRI) and hormonal studies revealed acute necrosis of the anterior pituitary gland accompanied by multiple endocrine abnormalities. Empirical dexamethasone therapy, initially administered due to clinical suspicion of bacterial meningitis before hormonal study results became available, fortunately resulted in rapid clinical improvement. Although the exact mechanism remains unclear, steroid therapy was successfully tapered and discontinued during hospitalization, allowing the patient to be discharged without ongoing hormonal replacement therapy. Follow-up care revealed continued recovery, with partial empty sella observed on MRI 31months postpartum as a sequela of SS. CONCLUSION: Acute SS may be underrecognized clinically, suggesting a higher incidence than previously reported. Immediate hormonal replacement might be crucial when acute SS is clinically suspected, given the delays in confirmatory testing. Careful monitoring of postpartum patients exhibiting an unusual clinical course should be implemented, as it may facilitate the early detection of potentially life-threatening complications such as adrenal crisis or acute endocrine dysfunction.

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