Endocrine Abstracts

A 59 year old woman was referred for further management of Cushings syndrome confirmed in another hospital. Presenting features included proximal myopathy, hypertension and hypokalaemia. Corticotrophin releasing hormone testing suggested an ectopic ACTH source.
Within 48 hours of admission for petrosal sinus sampling, the patient acutely deteriorated with type 1 respiratory failure requiring ventilatory support. A chest radiograph and computed tomography scan revealed a right basal pneumonia whilst bronchoscopy excluded a bronchial neoplasm. Anaemia requiring repeated transfusion necessitated gastroscopy that identified both oesophagitis and an actively bleeding duodenal ulcer. Despite various supportive treatments, gastrointestinal blood loss persisted and the patients condition became critical. The hypercortisoleamic state was felt to be contributing significantly to the gastrointestinal ulceration, bleeding, septicaemia and thrombocytopaenia. Urgent parenteral treatment was required to reduce circulating cortisol and stabilise the patient's condition. Intravenous etomidate infusion 1.2 millilitres per hour was commenced with hydrocortisone replacement which rendered the patient eucortisolaemic within 24 hours. In the subsequent 2 weeks, the GI bleeding and chest and systemic sepsis were controlled; the patient was extubated. Intravenous etomidate was substituted with oral metyrapone and ketoconazole to allow the patient to regain her mobility and general reserve.
Elective bilateral adrenalectomy was performed. Histology has revealed an incidental left sided phaeochromocytoma in addition to bilateral adrenocortical hyperplasia. Immunohistochemistry for ACTH on the phaeochromocytoma was negative. Postoperative MIBG scanning has so far remained negative and ACTH levels elevated.
In summary, this case highlights potentially life-threatening sequelae of Cushing's syndrome. Rapid intervention by intravenous Etomidate to correct hypercortisolaemia in seriously ill patients can be life saving in such a critical setting.