Background: Pregnancy is rare in women with Cushing’s Disease (CD) due to infertility. There are 55 documented cases in the literature of active CD during pregnancy and it is associated with adverse maternal and neonatal outcomes.
Case: DMF is a 44-year-old woman was referred for Type 2 Diabetes Mellitus planning pregnancy with comorbid obesity (BMI40), metabolic syndrome and PCOS. She was G2P1M1. Examination was consistent with CD. A 1 mg Dexamethasone suppression test (DST) was 163 nmol/L, 24 hour urinary free cortisol was 139 nmol/day. DMF was advised not to fall pregnant.
In 2023 a 4mg IV DST was suggestive of CD. MRI pituitary was normal. Despite endocrine advice to the contrary DMF underwent embryo transfer interstate with donor egg and donor sperm.
At 22 weeks gestation an early morning plasma cortisol level was 1,046 nmol/L and ACTH was 10 pmol/mL and DMF was started on cabergoline which was escalated to 1 mg twice a week without significant biochemical benefit. At 25 weeks gestation a non-contrast high-resolution MRI pituitary identified a 4 mm pituitary lesion. DMF underwent endoscopic endonasal resection of this lesion at 26 weeks; histology was consistent with a pituitary adenoma. Surgical recovery was complicated by acute hypoglycaemia and hypotension; cabergoline was ceased. Cortisol on the third post operative day was 204 nmol/L. Morning cortisol at 30 weeks gestation was 729 nmol/L and ACTH was 8 pmol/L. Insulin requirements fell following surgery despite normal evolving foetal morphology and placental function. Emergency LUSCS was performed at 35+5 due to increasingly severe hypertension. The baby weighed 2.87 kg (50th percentile) and required NICU for hypoxia. DMF remains on oral hydrocortisone replacement postpartum.
Conclusion: We present a case of a 44-year-old female with CD who fell pregnant. She failed medical therapy and underwent surgery in the second trimester with favourable response.