Background: Following bilateral adrenalectomy (BLA) serum cortisol is typically undetectable. Though in 12% endogenous cortisol production may recur in the form of adrenal remnant and rest tissue. We present a patient with Cushing’s disease (CD) managed with BLA and pituitary radiation with no glucocorticoid replacement for over 10 years, before presenting with an adrenal crisis.
Case Presentation: A 54 year-old male presented acutely unwell with fatigue and the sensation of felling hot. His blood pressure was 99/83mmHg, pulse 112/min and he had an altered level of consciousness. On physical examination he had bilateral laparotomy scars, and initially had hyponatremia. A diagnosis of adrenal crisis in the setting of sepsis was made and he was treated with intravenous fluids, intravenous hydrocortisone and antibiotics, with improvement in his clinical state within 24 hours.
He was diagnosed with CD at age 20 and at the time was managed with BLA and prophylactic pituitary radiation. Post-BLA he was replaced with hydrocortisone and fludrocortisone, however he self-ceased it more than a decade ago and was lost to follow-up. Despite not been on glucocorticoids for over 10 years, he reported been well, having reasonable energy levels and had not required any hospital admissions.
Incidentally during his admission, abdominal CT scan showed residual adrenal tissue on the right-side. His morning cortisol prior to hydrocortisone was 211 nmol/L, and the rest of his pituitary profile is enclosed in table 1. Given his adrenal crisis and BLA, he was discharged home on hydrocortisone 20mg in the morning and 10mg at midday, in addition with 50mcg of fludrocortisone.
Conclusion: Following BLA for Cushing's disease, endogenous cortisol production may recur. Therefore clinicians should regularly review the need and dosage of glucocorticoids, and if Cushingoid features reappear further evaluation should be pursued looking for clinically significant adrenal remnant and rest tissue.