Subtotal parathyroidectomy as an adequate treatment for primary hyperparathyroidism in multiple endocrine neoplasia type 1

HYPOTHESIS: The most appropriate surgical approach for hyperparathyroidism (HPT) in multiple endocrine neoplasia type 1 remains controversial. It has been advocated that reoperations for recurrent disease are easier to perform after total parathyroidectomy (TP) with autotransplantation than after subtotal parathyroidectomy (SP). In view of our large experience in patients with secondary HPT for whom TP with autotransplantation did not simplify reoperations, SP remains our preferred treatment for patients with HPT and multiple endocrine neoplasia type 1. DESIGN: Retrospective cohort study. SETTING: Tertiary referral medical center. PATIENTS: A total of 29 consecutive patients (22 women, 7 men; mean age, 42.2 years) with multiple endocrine neoplasia type 1 who underwent definitive cervical exploration for HPT. MAIN OUTCOME MEASURES: Temporary and permanent hypocalcemia, pattern of parathyroid disease, and sites and timing of recurrent HPT. Definitive primary surgery included SP in 21 patients, TP with autotransplantation in 4 patients, and less-than-subtotal parathyroidectomy in 4 selected patients. RESULTS: The mean follow-up was 88.5 months (range, 8-285 months). Four patients died during follow-up; 2 of these deaths were related to multiple endocrine neoplasia. No patients had persistent HPT. Temporary hypocalcemia occurred in 12 SP cases (57%), 4 TP with autotransplantation cases (100%), and 0 less-than-subtotal parathyroidectomy cases. Permanent hypocalcemia requiring long-term treatment occurred in 2 SP cases (10%), 1 TP with autotransplantation case (25%), and 0 less-than-subtotal parathyroidectomy cases. Four patients developed recurrent disease, including 1 with SP, 2 with TP with autotransplantation, and 1 with less-than-subtotal parathyroidectomy at 57 months, 197 and 180 months, and 164 months, respectively, representing 14% of all of the patients and 43% of patients with more than 10 years of follow-up. CONCLUSIONS: Recurrent HPT occurs many years after definitive primary surgery (median, 14.3 years). Surgical treatment should therefore aim to minimize the risk of permanent hypocalcemia and facilitate future surgery. When correctly performed, SP fulfills these objectives.