Long-term results of total adrenalectomy for Cushing's disease

The objective of this study was to present the long-term results of total a drenalectomy for Gushing's disease. Forty-four patients undergoing total ad renalectomy for Gushing's disease between 1953 and 1989 at Leiden Universit y Medical Center, The Netherlands, were studied retrospectively. Remission was achieved in 42 patients (95%), with a mean duration of 19 years. Adrena l remnants were observed in 12 patients (27%), and were without clinical co nsequence in the majority of patients, but caused early recurrent disease i n 2 patients. Nine patients (20%) experienced Addisonian crises up to 30 ye ars following treatment. Nelson's syndrome developed in 10 patients (23%) 7 -24 years following total adrenalectomy. Prior pituitary irradiation was a protective factor against Nelson's syndrome as it delayed its onset (p = 0. 025). On the other hand, subnormal dose or noncontinuous glucocorticoid rep lacement therapy was associated with increased risk of development of Nelso n's syndrome (p = 0.047). The incidence of Nelson's syndrome increased with prolonged follow-up, and female patients seemed to be at increased risk. Q uality-of-life assessment showed less favorable scores on mental health and health perception scales, for which no explanation can be found except the long-lasting metabolic effects of Gushing's disease, even when successfull y treated. In conclusion, total adrenalectomy remains the final treatment f or Gushing's disease. The presence of adrenal remnants which can cause recu rrent disease and the development of Nelson's syndrome during prolonged fol low-up enhance the need for continued regular follow-up, Pituitary irradiat ion prior syndrome.