Efficient short-term control of hypercortisolaemia by low-dose etomidate in severe paediatric Cushing's disease

Background: Paediatric Cushing's disease ( CD) is rare, but is associated with considerable morbidity and requires effective treatment. Control of hypercortisolaemia is recommended prior to definitive therapy by transsphenoidal pituitary surgery with selective adenomectomy. We describe a 6.2-year-old male with severe hypercortisolaemia and life-threatening complications of Cushing's disease. Control of cortisol with metyrapone and ketoconazole was ineffective, and due to his deteriorating condition, the decision was taken to proceed to bilateral adrenalectomy. Methods: Low-dose IV infusion of etomidate, with dose titration according to serum cortisol levels, was administered. Results: Etomidate infusion (3.0 mg/h i. v.) decreased serum cortisol from 1,250 to 250 nmol/l within 24 h. Combined etomidate and hydrocortisone therapy was maintained to provide stable serum cortisol levels within the desired range for 12 days prior to successful bilateral adrenalectomy. Conclusion: In our experience, etomidate was effective and safe for short-term control of severe hypercortisolaemia in a severely ill child. Copyright (C) 2005 S. Karger AG, Basel.