Background:
Cushing syndrome in pregnancy is associated with significant maternal and fetal morbidity. Physiological hypercortisolism in pregnancy may obscure the diagnosis, making timely recognition and management challenging.
Case Report:
A 31-year-old G3P2 woman presented at 21+2 weeks gestation with progressive exertional dyspnoea, worsening peripheral oedema, and 8-kg weight gain over one week. Her medical history included chronic hypertension, type 2 diabetes mellitus, obesity (BMI 37kg/m²), congenital pulmonary valve stenosis with previous valvotomy, a known right adrenal lesion and prior L1 vertebral compression fracture. Severe obstructive sleep apnoea was diagnosed during pregnancy.
Investigations demonstrated elevated NT-proBNP (247ng/L) and severe pulmonary regurgitation on transthoracic echocardiogram. Despite four antihypertensive agents, hypertension remained difficult to control, with no signs of placental dysfunction. Persistent fluid overload, easy bruising and prominent abdominal striae raised suspicion for hypercortisolism. Biochemical testing showed markedly elevated 24-hour urinary free cortisol (1650nmol/24 h; reference <270), elevated serum cortisol (672nmol/L), suppressed ACTH (<0.3pmol/L), and elevated midnight salivary cortisol, consistent with ACTH-independent Cushing syndrome. Adrenal MRI demonstrated interval enlargement of a lipid-poor right adrenal adenoma.
Following multidisciplinary discussion, metyrapone was commenced as bridging therapy and definitive treatment with right adrenalectomy was performed at 24+2 weeks gestation to mitigate maternal and fetal risks. Postoperatively, insulin requirements decreased by >50%, antihypertensive therapy by 50%, and fluid overload improved (92kg to 86kg). The remainder of the pregnancy was uncomplicated, with elective caesarean delivery at 37 weeks.
Conclusion:
Multidisciplinary management and timely surgical intervention of Cushing syndrome in pregnancy can result in favourable maternal and pregnancy outcomes.