Surgical approach to the patient with familial hyperparathyroidism.

Familial hyperparathyroidism encompasses the diagnoses of multiple endocrine neoplasia (MEN) type 1, MEN type 2A, and familial isolated primary hyperparathyroidism. All patients should undergo bilateral neck exploration and identification of all four or more parathyroid glands to evaluate for gross abnormalities. MEN-1 patients should have subtotal parathyroidectomy and cervical thymectomy because this operation achieves an appropriate balance between optimizing the potential for cure yet minimizing the risk of permanent hypocalcemia. However, MEN-2A patients may best be treated by selective resection of abnormal parathyroid glands, although some experts recommend a total parathyroidectomy and autotransplantation in the forearm. Familial isolated hyperparathyroidism is a rare disorder, and authors have described success in treatment with subtotal parathyroidectomy or limited adenoma resections. Some patients with familial isolated hyperparathyroidism also have jaw tumors, and members of these families are more likely to have parathyroid carcinoma. Concurrent cryopreservation of parathyroid tissue for all of these disorders is recommended if there is any concern for possible permanent hypoparathyroidism.