Pediatric Hyperparathyroidism Treatment & Management

Updated: Apr 17, 2017
  • Author: Edna E Mancilla, MD; Chief Editor: Sasigarn A Bowden, MD  more...
  • Print
Treatment

Medical Care

FHH is mild, usually asymptomatic, and therefore does not require specific therapy. Treatment for NSHPT has been surgical but the development of new drugs has allowed for successful medical management in several cases. These therapies are considered experimental and include calcimimetics (cinacalcet) and bisphosphonates (pamidronate) which have been used as monotherapy or as combination therapy. [21, 22] Cinacalcet, an allosteric modulator of the CASR, has not been approved in children and requires close supervision. Hypocalcemia has been the principal side effect described when used for NSHPT, and a trial was suspended because of one death in an older child which may or may not be related to the medication. [22, 23] Bisphosphonates are similarly not approved in children but have been used more extensively in Osteogenesis Imperfecta and Pediatric Osteoporosis.  Treatment is surgical if medical therapy fails, with total parathyroidectomy being the preferred approach to avoid recurrence. [24]

Patients with calcium levels at 12-14 mg/dL should be admitted to the hospital. If asymptomatic, saline hydration may suffice. The treatment of acute severe hypercalcemia (serum calcium level >14 mg/d, or 12-14 mg/dl but symptomatic) includes hydration with isotonic sodium chloride solution to restore extracellular fluid volume that may be depleted secondary to vomiting and to induce calciuresis. Loop diuretics (eg, furosemide) should be avoided as they may worsen dehydration. Additional forms of medical treatment that can be used to decrease bone resorption include calcitonin and bisphosphonates such as pamidronate or zoledronate. These rapid acting therapies may be followed by cinacalcet while awaiting surgery.

 

 

Next:

Surgical Care

Primary hyperparathyroidism due to a solitary adenoma is cured by resection of the adenoma. Total or subtotal parathyroidectomy with autotransplantation of a parathyroid gland is curative for multiglandular disease. As in adults, a minimally invasive approach with the use of intraoperative PTH (iPTH) measurements is the gold standard for surgical resection of solitary adenomas in children. [8, 1, 7, 25]  Rapid PTH assays have been used more routinely in adults; Nussbaum first described the use of  iPTH using sensitive immunoradiometric assay (IRMA) and demonstrated that a decrease of PTH levels to less than 40% of baseline values 15 minutes after parathyroidectomy confirmed cure. [26] According to the Miami criteria, cure is confirmed by a drop of  ≥ 50% of  the highest pre incision or pre excision PTH level when PTH is measured 10 min post complete resection. [27] The use of iPTH has significantly improved surgical outcome in cases with negative imaging studies. [8] Surgical outcome depends on the experience of the surgeon.

Postoperative complications include transient hypocalcemia until parathyroids regain their sensitivity to circulating calcium. This requires treatment with calcitriol and calcium supplements until parathyroid function recovers. If hypocalcemia is mild calcium supplements may suffice.  Hungry bone syndrome, a prolonged period of hypocalcemia, can occur postoperatively in those cases of primary hyperparathyroidism that demonstrated significant bone demineralization. Bones reaccumulate calcium at the expense of circulating levels and calcium supplements are needed for a longer time period. Finally, as in thyroid surgery, a risk of damage to the recurrent laryngeal nerve resulting in permanent hoarseness of the voice may be observed.

Previous
Next:

Consultations

The primary care provider should consult a pediatric endocrinologist.  Consultation with a surgeon may be obtained after consultation with the pediatric endocrinologist.  A urologist would need to be involved in the case of renal calculi. Genetic counseling for the patient and family members should be offered if the diagnosis of a genetic multiple endocrine neoplasia syndrome is made.

Previous
Next:

Diet

No strict dietary requirements are necessary for management of primary hyperparathyroidism. Vitamin D deficiency should be corrected to differentiate primary from secondary hyperparathyroidism.

 

Previous
Next:

Complications

Surgical complications include recurrent laryngeal nerve injury, bleeding and infection. Complications of primary hyperparathyroidism include consequences of hypercalcemia, such as nephrolithiasis, dehydration, and cardiac arrhythmias, and complications due to end organ involvement such as renal failure and fractures.

Previous