Excellent Maternal and Fetal Outcomes Associated With Early Detection and Treatment of Cushing’s Syndrome During Pregnancy

Abstract

Background: Despite its relative high prevalence in women of reproductive age, pregnancy is uncommon in Cushing’s syndrome (CS) as hypercortisolism typically results in anovulation and infertility. Early diagnosis and treatment are essential to reduce maternal and fetal complications.Clinical Case: A 31-year-old woman with Type 2 diabetes mellitus and hypertension was referred for significantly elevated midnight salivary and urine cortisol levels consistent with CS. Six months after initial laboratory findings, she presented to our endocrinology clinic eight weeks pregnant with di/di twins. She reported worsening blood pressure and glucose control, mood fluctuations, and easy bruising for the past several months. Menses were regular prior to conception. Physical exam was notable for round facies, mild dorsal hump, and supraclavicular fullness. Lab tests at eight and thirteen weeks gestation respectively showed: elevated 24-hr urine free cortisol 368.5 and 597.5 ug/24hr (< 45); elevated midnight salivary cortisol 0.88 mcg/dL (<0.09) at eight weeks; ACTH <1.1 and 8.1 pg/mL (7.2 - 63.3); and elevated morning cortisol 34.2 and 36 ug/dL (6.2 - 19.4). Her hemoglobin A1C was 8.1% on metformin and Levemir. Pituitary MRI was normal. MRI of the abdomen without contrast demonstrated a 2.5 x 3.5 cm solid homogeneous mass in the right adrenal gland. The patient underwent successful right adrenalectomy at seventeen weeks gestation and was discharged on glucocorticoid replacement. Glucose control was labile due to non-compliance but otherwise the remainder of her pregnancy was uncomplicated. The patient delivered healthy twins at thirty-five weeks. At six months postpartum she is doing well on low dose hydrocortisone replacement. Most recent hemoglobin A1C was 5.7% without diabetes medication.Conclusion: There is no consensus on the management of CS during pregnancy due to the rarity of this condition. CS in pregnancy is associated with high maternal and fetal morbidity and mortality, thus early diagnosis and prompt treatment is essential. Our case demonstrates the importance of discussing contraception with Cushing’s patients of reproductive age. It also adds to prior studies and showed that when treated early in pregnancy, either surgically or medically, maternal and fetal risks such as uncontrolled diabetes and fetal loss are significantly reduced. The ideal management of these patients is an area of ongoing investigation.