Severe hypercortisolism: a medical emergency requiring urgent intervention.

OBJECTIVES: To illustrate the importance of recognizing symptoms of severe hypercortisolism in the intensive care unit and key emergency measures to reduce this extreme hypercortisolism. DESIGN: Case report. SETTING: Intensive care unit in a university hospital. PATIENT: A 55-yr-old woman was admitted to the intensive care unit with multiorgan failure after perforation of the sigmoid. Recent-onset hypertension, spontaneous hypokalemia, and diabetes mellitus suggested severe Cushing's syndrome as the underlying disease. Markedly increased serum cortisol (5900 nmol/L) and adrenocorticotropic hormone (302 ng/L) levels were found, highly suggestive for ectopic adrenocorticotropic hormone secretion. Imaging studies failed to unequivocally establish a solitary source of ectopic adrenocorticotropic hormone secretion. The deteriorating condition of the patient urged rapid intervention. INTERVENTIONS: Etomidate was infused continuously to reduce endogenous adrenal cortisol secretion. Subsequently, a rescue bilateral adrenalectomy was undertaken. MEASUREMENTS AND RESULTS: Etomidate effectively reduced the cortisol level. Serial blood samples were obtained during the bilateral adrenalectomy. Plasma adrenocorticotropic hormone markedly decreased immediately after resection of the right adrenal gland. Histopathological examination revealed a tumor of the right adrenal gland identified as a pheochromocytoma and hyperplasia of the left adrenal gland, but no signs of malignancy. The patient recovered slowly. CONCLUSION: This case illustrates that severe hypercortisolism is a medical emergency and that specific and prompt combined medical and surgical intervention can be life-saving.