The outcome of subtotal parathyroidectomy for the treatment of hyperparathyroidism in multiple endocrine neoplasia type 1.

BACKGROUND The efficacy of subtotal parathyroidectomy for the treatment of hyperparathyroidism in multiple endocrine neoplasia type 1 (MEN 1) is unclear. The long-term outcome and optimal timing of operation remain controversial. OBJECTIVE To determine the long-term outcome of parathyroidectomy for primary hyperparathyroidism in the presence of MEN 1. DESIGN Case series and retrospective analysis. SETTING Tertiary referral center. PATIENTS Patients with MEN 1 from 2 families. INTERVENTIONS Subtotal parathyroidectomy, ie, resection of 3 1/2 parathyroid glands from each patient. MAIN OUTCOME MEASURES Recurrence of hyperparathyroidism. RESULTS Thirty-seven patients underwent subtotal parathyroidectomy. Overall, persistent postoperative hypoparathyroidism developed in 24%, normocalcemia was maintained in 46%, and hyperparathyroidism recurred in 30%. However, after adjustment for the duration of follow-up (by using the Kaplan-Meier method), the cumulative recurrence rates for hyperparathyroidism were 15% at 2 years, 23% at 4 years, 55% at 8 years, and 67% after 8 years. Early recurrence of hyperparathyroidism (within 5 years of operation) was less likely to develop in patients in whom ionized calcium levels of 1.00 mmol/L (4.00 mg/dL) or less were achieved during the perioperative period than in patients in whom this degree of hypocalcemia failed to develop (P=.01). CONCLUSIONS While relatively long periods of disease remission are possible after subtotal parathyroidectomy, our results indicate that recurrent hyperparathyroidism eventually develops in most patients with MEN 1.