A 55-year-old woman with MEN1 syndrome has primary hyperparathyroidism (serum calcium 11.8 mg/dL, PTH 145 pg/mL). Sestamibi scan is negative. The most appropriate surgical strategy differs from sporadic primary hyperparathyroidism because:

• A Minimally invasive parathyroidectomy based on sestamibi alone is standard
• B Medical management with cinacalcet is always preferred over surgery in MEN1
• C Unilateral exploration based on intraoperative PTH drop is adequate
• D Bilateral exploration with subtotal parathyroidectomy (3.5 gland removal) or total parathyroidectomy with autotransplantation is preferred ✓

Explanation

In MEN1-associated primary hyperparathyroidism, multigland disease is the rule (asymmetric 4-gland hyperplasia), so focused minimally invasive parathyroidectomy based on sestamibi is inadequate. Standard surgical approach is bilateral cervical exploration with subtotal parathyroidectomy (leaving 40-60 mg of the most normal-appearing gland) or total parathyroidectomy with forearm autotransplantation. Concurrent thymectomy is performed to remove supernumerary glands and reduce risk of thymic carcinoid. Recurrence rates are higher than sporadic PHPT, necessitating marking the remnant with a clip for future re-exploration.

Reference: Bailey & Love's Short Practice of Surgery, 27th ed.

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Written and medically reviewed by the StethoPrep medical team.