Background: Bilateral adrenalectomy provides an effective surgical option for patients with Cushing's syndrome. Knowledge of the long-term outcome of the patient who has undergone adrenalectomy is an important factor in determining management strategy in this situation.
Methods: Fifty consecutive patients undergoing bilateral adrenalectomy (1980 to 1991) were studied. Twenty-five had Cushing's disease, 18 ectopic adrenocorticotrophic hormone production, and seven primary adrenal hyperplasia. Median age was 43 years (range, 14 to 82 years); male-to-female ratio was 13:37. At follow-up 17 patients had died; the remaining 33 were interviewed at a median of 62 months after operation.
Results: Operative mortality was 4%. Late mortality included two patients with Cushing's disease who died of progressive pituitary disease and eight patients with ectopic adrenocorticotrophic hormone production who died of metastatic disease. Five-year survival was: 100% for patient with adrenal hyperplasia, 86% for patients with Cushing's disease, and 39% for patients with ectopic adrenocorticotrophic hormone production. Of 33 survivors followed for a median of 62 months, three (9%) were hospitalized with acute steroid deficiency. The principal complaint among survivors was chronic fatigue (60%). Only 10 (30%) patients had no symptoms. No steroid-related complications resulted after 16 surgical procedures subsequently performed. Of 20 survivors of Cushing's disease, three had Nelson's syndrome.
Conclusions: Bilateral adrenalectomy for Cushing's syndrome is associated with occasional morbidity and mortality consequent to adrenal insufficiency. These patients suffered a high incidence of chronic physical complaints that may reflect the residual sequelae of chronic Cushing's syndrome. An active program of long-term rehabilitation is essential.