Endocrine Abstracts

Background: Cushing’s syndrome during pregnancy is exceedingly rare and associated with significant maternal and fetal morbidity. Diagnosis is often delayed, as physiological adaptations in pregnancy can obscure the clinical and biochemical features of hypercortisolism. ACTH-independent Cushing’s syndrome, most commonly due to an adrenal adenoma, is particularly uncommon in pregnancy.

Case: A 34-year-old woman, gravida 3 para 0, with a spontaneous monochorionic diamniotic twin pregnancy, presented at 20 weeks’ gestation with an atraumatic right femoral neck fracture. She exhibited classic Cushingoid features, persistent hypertension, insulin-dependent diabetes, and recurrent hypokalaemia. Biochemical testing confirmed marked hypercortisolism with suppressed ACTH, and MRI identified a 4×3 cm left adrenal adenoma, consistent with ACTH-independent Cushing’s syndrome. Due to maternal metabolic instability and twin gestation, adrenalectomy was deferred, and metyrapone therapy was initiated for medical control of hypercortisolism. Despite treatment, serial ultrasound scans demonstrated severe fetal growth restriction and reversed end-diastolic flow in one twin, necessitating emergency caesarean section at 27 + 4 weeks’ gestation. Two liveborn female infants (610 g and 430 g) required neonatal intensive care for prematurity and transient adrenal suppression, successfully managed with hydrocortisone replacement. Postpartum, the mother’s blood pressure and glycaemic control normalised. Definitive laparoscopic adrenalectomy performed three months later confirmed a benign cortisol-secreting adenoma.

Conclusion: This case illustrates an exceptionally rare presentation of ACTH-independent Cushing’s syndrome in twin pregnancy, initially manifesting as an atraumatic fracture. Early recognition and coordinated multidisciplinary management were essential in achieving favourable maternal and neonatal outcomes despite significant metabolic and obstetric challenges.