Complications after bilateral adrenalectomy for phaeochromocytoma in multiple endocrine neoplasia type 2--a plea to conserve adrenal function.

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 phaeochromocytoma in patients with MEN 2 syndrome, provided that they are carefully followed up.