Long-term outcome of bilateral adrenalectomy in patients with Cushing's syndrome

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.

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.

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.

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.