Abstract

Background. Familial isolated primary hyperparathyroidism (FIHPT) is characterized by earlier onset, higher incidence of multiglandular disease, and higher recurrence rate when compared with sporadic primary hyperparathyroidism. Excision of 3.5 or 4 glands with autotransplantation has been recommended; however, these approaches lead to permanent hypoparathyroidism in 13% to 41% of patients. It is reported that many patients with FIHPT return to normocalcemia after single-gland excision. The use of preoperative localization and intraoperative parathyroid hormone assay permits limited resection of only hypersecreting glands. We report the outcome of this operative approach. Methods. Fifteen consecutive patients with FIHPT underwent limited parathyroidectomy with resection guided by intact parathyroid hormone secretion in 2 university centers. Patients were followed up postoperatively for serum calcium and intact parathyroid hormone levels. Results. With an operative success of 93%, 14 patients had only single-gland excision and 80% had unilateral neck exploration. All initial patients had their hypercalcemia corrected. In 4 reoperations, permanent hypoparathyroidism occurred in 2 patients. One recurrence was observed in 40 (8-122) months of follow-up. Conclusion. Limited parathyroidectomy allows successful single-gland excision in many patients with FIHPT, thus decreasing the risk of hypoparathyroidism. In these patients, a low incidence of hypoparathyroidism may be preferable to the possibility of late recurrence. (Surgery 2002;132:1050-5.)

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