Complications after bilateral adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia Type 2 - A plea to conserve adrenal function

Abstract

Objective: To evaluate the complications of the adrenocortical supplementation that is needed after bilateral adrenalectomy for phaeochromocytoma in patients with multiple endocrine neoplasia (MEN) type 2 syndrome. Design: Retrospective study. Setting: University hospital, The Netherlands. Materials: 28 patients with MEN 2 who underwent total adrenalectomy for phaeochromocytoma between 1972 and 1996. Main outcome measures: Perioperative morbidity and mortality, histopathological findings, complications of adrenocortical supplementation therapy. Results: 22 patients had bilateral phaeochromocytomas on histopathological examination (79%) and 6 patients had initially unilateral phaeochromocytomas. There was no operative mortality or substantial morbidity except for one splenic injury that necessitated splenectomy. During a mean follow-up period of 14 years (range 1-26) nine patients (32%) had a total of 19 Addisonian crises that necessitated admission to hospital. One patient died of an unrecognised Addisonian crisis. Conclusion: Complications of adrenocortical supplementation therapy are considerable, but they can be reduced when unilateral adrenalectomy is done for a unilateral phaeochromoytoma in patients with MEN 2 syndrome, provided that they are carefully followed up.