Dr Madeline Spooner
Royal Brisbane and Women’s Hospital, Brisbane


Title: Cushing’s Syndrome In Pregnancy: A Diagnostic And Management Conundrums

Authors: Spooner M, Wolski P, D’Emden M, Barrett H, and Britten F.

Affiliation: Royal Brisbane and Women’s Hospital, Brisbane

Abstract Text: 
Cushing’s syndrome (CS) during pregnancy is a rare condition associated with high maternal and fetal morbidity. The rare presentation has impaired the formulation of established guidelines for diagnosis, management, and surveillance. We report a case of adrenocorticotrophic (ACTH) independent CS diagnosed in pregnancy.

A 27 year old previously well primigravid woman, was referred at 24 weeks gestation with gestational diabetes mellitus. She reported a preceding 12-week history of rapid weight gain and fatigue, and on examination was noted to be plethoric with prominent supraclavicular and dorsocervical fat pads, diffuse violaceous striae, hirsutism, acne and hypertension with a blood pressure of 165/90 mmHg. 

Clinical findings suggestive of CS prompted investigations to demonstrate hypercortisolism. Elevated urinary free cortisol (UFC) measurements and loss of normal diurnal rhythm were confirmative, and the repeated finding of an undetectable ACTH indicated ACTH-independent CS. Magnetic resonance imaging of the abdomen revealed a left adrenal adenoma measuring 2.9 x 2.6 x 2.4cm.

Medical management with 250mg BD of metyrapone was commenced after multi-specialist consultation deemed surgical intervention to be too high risk. The dose of metyrapone was titrated to the UFC aiming for 200-300nmol/24h, measured twice weekly. Reassuring serial growth scans reveal a large for gestational age fetus. She is currently 30 weeks gestation and remains in a stable condition.

This case exemplifies the major challenges in diagnosis and management of CS and its’ consequences in pregnancy. We will review the case progress and surrounding literature.

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