Cushing Disease Treated Successfully With Metyrapone During Pregnancy

Area of Science:

• Endocrinology
• Obstetrics
• Reproductive Medicine

Background:

• Cushing disease (CD) in pregnancy presents significant maternal and fetal risks.
• Management options for CD during gestation are limited, especially when surgery is contraindicated or unlikely to be curative.

Observation:

• A 34-year-old woman with persistent ACTH-dependent CD post-surgery was treated with metyrapone throughout pregnancy.
• Treatment aimed to maintain urinary free cortisol (UFC) below 150 μg/24 h, accounting for gestational increases.
• The patient achieved diet-controlled gestational diabetes and well-controlled hypertension.

Findings:

• Metyrapone effectively controlled hypercortisolism in pregnancy, enabling a healthy term delivery.
• Neither mother nor infant experienced adrenal insufficiency post-birth.
• No fetal adverse events were noted during metyrapone treatment.

Implications:

• Metyrapone is a viable therapeutic option for pregnant patients with Cushing disease when surgery is high-risk or cure is unlikely.
• Close maternal surveillance for hypertension, hypokalemia, and adrenal insufficiency is crucial during metyrapone therapy.
• Long-term fetal effects of in-utero metyrapone exposure remain unknown, necessitating continued research.