Endocrine Abstracts

A 34-year-old primi gravida, 11 weeks into pregnancy, presented with hypertension (blood pressure 170/110 mmHg) proteinuria and hypokalaemia. She gave a 6-week history of tiredness, weight gain, and easy bruising. On examination, there was marked facial puffiness, non pitting oedema, thin skin with multiple bruises, marked proximal myopathy and no pigmented striae. Pregnancy associated Cushing’s was suspected.

Investigations: Hypokalaemia (K⁺ 2.9 mmol/l) and alkalosis, otherwise normal renal function. Urinalysis confirmed proteinuria. Midnight cortisol 755 nmol/l, corresponding ACTH undetectable. USG showed a 12 week normal viable foetus. Urinary cortisol was 9552 and 11 344 nmol/24 h on successive days. Low and high dexamethasone suppression tests showed no suppression of cortisol levels. Adrenal androgen profile was unremarkable apart from a mildly raised androstenedione level. Random aldosterone level and random plasma rennin activity were mildly suppressed. Serum and urinary metanephrines were normal. A non contrast MRI showed a 4 cm left sided adrenal mass.

Blood pressure was controlled with methyl dopa, and potassium corrected orally. She underwent laparoscopic adrenal surgery at 12 weeks gestation. Histology was in keeping with an adrenocortical tumour. Immediately post operatively, hydrocortisone replacement therapy was instituted. Within 24 h, off all treatment, her blood pressure and hypokalaemia had normalised. Proteinuria, as well as clinical features of cortisol excess disappeared within 6 weeks. To date, the pregnancy is progressing normally. Full re-assessment of HPA axis, withdrawal of hydrocortisone and repeat imaging have all been deferred until after delivery. However, ACTH is now detectable and pre dose cortisol is suggestive of early recovery of the contra lateral gland.

The diagnosis of pregnancy associated Cushing’s syndrome is difficult to make, due to significant overlap of physical and biochemical findings. Less than 150 cases have been reported to date. Untreated this condition carries extremely high maternal and fetal morbidity and mortality, hence prompt recognition and treatment are crucial.