Surgical treatment of hyperparathyroidism in patients with multiple endocrine neoplasia type 1

Hypothesis: Three-gland parathyroidectomy with transcervical thymectomy and cryopreservation is the preferred initial surgical approach for hyperparathyroidism (HPT) in patients with multiple endocrine neoplasia type 1. Design: Retrospective cohort study. Setting: Tertiary referral center. Patients: Thirty-seven patients with multiple endocrine neoplasia type 1 who underwent 1 or more surgical procedures for HPT from January 1, 1973, to April 30, 2004. Results: At initial parathyroid surgery, 16 (43%) of 37 patients had fewer than 3 parathyroid glands resected (group 1); 16 (43%), had at least 3 but fewer than 4 glands (group 2); and 5 (14%), 4 or more glands (group 3). Follow-up of at least 6 months after initial surgery was complete for 31 (84%) of 37 patients. Recurrent HPT developed in 20 (65%) of 31 at a median of 4 years. Reoperation for recurrent HPT was performed in 16 (52%) of 31, including 12 patients (75%) in group 1 and 4 (25%) in group 2. No patient in group 3 required reoperative cervical surgery. Permanent hypoparathyroidism occurred in 1 patient (3%), despite autograft of parathyroid tissue to the forearm. Conclusions: Recurrent HPT in patients with multiple endocrine neoplasia type 1 is frequent if fewer than 3 glands are removed at initial parathyroidectomy. Optimal surgical intervention must balance the risk of recurrent hypercalcemia with the morbidity of permanent hypoparathyroidism. Three-gland parathyroidectomy, transcervical thymectomy, and parathyroid cryopreservation constitute our preferred initial surgical procedure.