Successful Medical Management of Cushing Disease With Metyrapone Throughout Pregnancy: A Report of 2 Cases

Cushing syndrome (CS) in pregnancy is a rare disorder with challenging diagnostic and therapeutic considerations. For patients with medically managed CS planning pregnancy, bilateral adrenalectomy is advised before conception. To our knowledge, no case of Cushing disease (CD) medically treated throughout pregnancy with cortisol synthesis inhibition has been described. We present 2 cases of CD in pregnancy managed with metyrapone, a non-Food and Drug Administration-approved drug for pregnancy. The first case involves a 31-year-old woman with recurrent CD who conceived twins via in vitro fertilization while on metyrapone. She had an uneventful pregnancy, delivering healthy twins at 37 weeks. Postpartum, she developed hypertension, which resolved spontaneously, and remained on metyrapone, with stable cortisol levels and normal development of her twins. The second case describes a 29-year-old woman with recurrent CD since adolescence, status post 2 transsphenoidal resections and gamma knife radiosurgery, who conceived naturally on metyrapone. After an uncomplicated pregnancy, she developed postpartum preeclampsia requiring temporary antihypertensive therapy. Both cases showed favorable maternal and fetal outcomes, highlighting metyrapone as a therapeutic option for managing CD during pregnancy.