Cushing Disease Treated Successfully With Metyrapone During Pregnancy

BACKGROUND: Cushing disease (CD) during pregnancy is a rare but serious disease that adversely impacts maternal and fetal outcomes. As the sole use of metyrapone in the management of CD has been rarely reported, we describe our experience of using it to treat a pregnant patient with CD. CASE REPORT: A 34-year-old woman with hypertension was diagnosed with adrenocorticotropic hormone–dependent CD on the basis of a urinary free cortisol (UFC) level of 290 μg/24 h (reference range, 6-42 μg/dL) and an abnormal dexamethasone suppression test (cortisol level, 12.4 μg/dL) before becoming pregnant. She conceived naturally 12 weeks after transsphenoidal surgery and was subsequently found to have persistent disease with a UFC level of 768 μg/dL. Surgery was deemed high-risk given the proximity of the tumor to the right carotid artery and the high likelihood of residual disease. Instead, she was managed with metyrapone throughout her pregnancy and titrated to a goal UFC level of <150 μg/24 h due to the known physiologic increase in the cortisol level during gestation. The patient had diet-controlled gestational diabetes and well-controlled hypertension. She gave birth to a healthy baby boy at 37 weeks of gestation, without adrenal insufficiency in the baby or her. DISCUSSION: This case highlights the successful use of metyrapone throughout pregnancy to manage CD in patients in whom surgery is considered high-risk or in those with a low likelihood of cure. Although metyrapone is effective, close surveillance is required for worsening hypertension, hypokalemia, and potential adrenal insufficiency. Although no fetal adverse events have been reported, this medication crosses the placenta, and the long-term effects are unknown. CONCLUSION: We describe a case of CD during pregnancy that was successfully treated with metyrapone.