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Abstract
IntroductionTo describe the use of combined preoperative imaging and intraoperative parathyroid hormone as a novel approach in the surgical management of a patient with tertiary hyperparathyroidism associated with X-linked hypophosphatemic rickets.Case presentationWe present the first documented description of combined preoperative imaging and intraoperative parathyroid hormone as well as a review of the literature surrounding the surgical management of tertiary hyperparathyroidism in the setting of X-linked hypophosphatemic rickets.A 23 year-old female with X-linked hypophosphatemic rickets and renal impairment presented with symptomatic hypercalcemia and tertiary hyperparathyroidism. She had failed medical management and presented for surgical evaluation. Technitium-99 m Sestamibi SPECT imaging and parathyroid ultrasound were used to localize the enlarged parathyroid glands preoperatively. Intraoperative findings correlated well with pre-operative imaging. She underwent successful subtotal parathyroidectomy for four-gland hyperplasia, using intraoperative parathyroid hormone guidance. Despite severe post-operative bone hunger, her serum calcium normalized and she experienced resolution of her preoperative symptoms.ConclusionX-linked hypophosphatemic rickets is an uncommon disorder of phosphate metabolism resulting in bone deformity. Patients are predisposed to the development of secondary hyperparathyroidism due to chronic vitamin D supplementation which may progress to tertiary hyperparathyroidism with autonomous parathyroid function. Preoperative evaluation with Technitium-99 m Sestamibi SPECT and ultrasound imaging, as well as the use of intraoperative parathyroid hormone are effective in guiding surgical resection. Subtotal parathyroidectomy with cryopreservation is indicated to produce operative cure and limit the risk of recurrence. Although these patients are susceptible to severe postoperative bone hunger, appropriate supplementation with intravenous and oral calcium can minimize hypocalcemic symptoms.
Highlights
Introduction: To describe the use of combined preoperative imaging and intraoperative parathyroid hormone as a novel approach in the surgical management of a patient with tertiary hyperparathyroidism associated with X-linked hypophosphatemic rickets
Case presentation: We present the first documented description of combined preoperative imaging and intraoperative parathyroid hormone as well as a review of the literature surrounding the surgical management of tertiary hyperparathyroidism in the setting of X-linked hypophosphatemic rickets
We documented a 90% drop in parathyroid hormone (PTH) after subtotal resection with normal calcium levels ten months post-operatively, indicating that she was surgically cured of her tertiary hyperparathyroidism, The mild elevation of her PTH was likely reflective of secondary hyperparathyroidism due to chronic renal insufficiency or vitamin D deficiency
Summary
X-linked hypophosphatemic rickets (XLHR) is a rare dominant hereditary bone disorder characterized by excessive renal phosphate excretion and impaired bone mineralization [1]. Case presentation A 23 year old Caucasian American female with a known diagnosis of XLHR presented with symptomatic hypercalcemia and tertiary HPT As a child, she had been treated with high dose phosphate and calcitriol supplementation which resulted in renal insufficiency and subsequent hypertension. The surgical pathology report (supplemental figure) describes all four parathyroid glands as “enlarged and hypercellular” As anticipated, she developed symptomatic postoperative hypocalcemia and was initially maintained on an intravenous calcium drip. At follow-up both six and ten months later she remained asymptomatic and her calcium and phosphate levels were normal (9.0 and 2.8 mg/dL, respectively), suggesting no recurrence of tertiary hyperparathyroidism She had a mild elevation of her PTH (136 mg/dL) in the setting of chronic renal insufficiency (creatinine 1.9)
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