Endocrine Abstracts

Cushing’s syndrome in pregnancy is rare and is associated with increased fetal and maternal morbidity. It has previously been described in the presence of ectopic LH receptor expression, and has been associated with gestational diabetes and preeclampsia but to our knowledge has never been associated with any other endocrine pathology.

We here report a 34-year-old woman, who presented with hypertension, weight gain, fluid retention and easy bruising at 11 weeks gestation. On assessment she had marked proximal myopathy, thin skin with pale striae, hypertension: 170/110 mmhg and proteinuria.

Pregnancy associated Cushing’s Syndrome was confirmed biochemically. Two 24 h urinary cortisol were 11 344 and 9552 nmol/24 h on successive days. Midnight cortisol was elevated at 755 nmol/l with undetectable ACTH level, and low dose dexamethasone suppression test showed no suppression: 853 to 811 nmol/l. Ultrasound scan revealed a viable 12 week foetus and non-contrast MRI scan confirmed the suspicion of a unilateral adrenal adenoma. She underwent uneventful laparoscopic adrenalectomy at 13 weeks, then received hydrocortisone replacement throughout the rest of the pregnancy which was subsequently withdrawn, and delivered a healthy male fetus at 39 weeks.

Two years later the patient has represented with symptoms suggestive of Diabetes Insipidus. Water deprivation testing has confirmed this diagnosis, with normal repeat urinary free cortisols and low dose dexamethasone testing. MRI pituitary is apparently normal, with no evidence of recurrence on the adrenal CT scan. Auto-immune screen, ferritin, hCG, α-fetoprotein, ESR, and angiotensin converting enzyme as well as anterior pituitary profile are all normal.

The patient has responded well to intranasal desmopressin. However, no unifying diagnosis has yet been confirmed and we believe this to be the first case presenting with this combination of diagnoses.