Outcome of Bilateral Adrenalectomy in Cushing's Syndrome: A Systematic Review

Background: The outcome of bilateral adrenalectomy (BADx) in patients with Cushing's syndrome (CS) is not well characterized. Methods: A literature search was performed with the search terms "bilateral adrenalectomy" or "total adrenalectomy" and "Cushing's" or "Cushing." Immediate and long-term outcomes after BADx in CS were analyzed using descriptive statistics (median [range]). Results: From 549 screened publications, 37 studies met inclusion criteria (1320 patients, 82% having Cushing's disease [CD], 13% having ectopic CS, and 5% having primary adrenal hyperplasia). Surgical morbidity and mortality of BADx(23 studies, 739 patients) were 18%(6-31) and 3%(0-15), respectively. In patients with CD, surgical mortality was below 1%. Although residual cortisol secretion due to accessory adrenal tissue or adrenal remnants was found in 3-34% (5 studies, 236 patients), less than 2% had a relapse of CS. Symptoms of hypercortisolism (eg, hypertension, obesity, or depression) improved in the majority of the patients after BADx(7 studies, 195 patients). The number of adrenal crises per 100 patient-years was 9.3 (6 studies, 203 patients). Nelson's syndrome occurred in 21% (0-47) of the patients (24 studies, 768 patients). Mortality (23 studies, 690 patients) was 17% (0-88) at a follow-up of 41 months(14-294). Remarkably, 46% of the patients died in the first year after surgery. The median ages at death were 62 years (CD) and 53 years (ectopic CS). Conclusion: BADx is relatively safe and provides adequate success. Excess mortality within the first year after surgery suggests that intensive clinical care for patients after BADx is warranted.