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.