Primary hyperparathyroidism presenting as acute hypercalcemic crisis: a case report

The researchers said that patients with recurrent nephrolithiasis should be screened for hyperparathyroidism, a life-threatening entity. The aetiology includes malignancy, atypical adenoma, and hemorrhagic cystic degeneration.

Cystic parathyroid adenomas have high serum levels of PTH and calcium, low accuracy of preoperative localization tests, and risk of hypercalcemia crisis is higher. These patients may warrant more urgent surgical intervention.

The case report is published in the Journal Of Otolaryngology-Head & Neck Surgery.

One of the rare manifestations of primary hyperparathyroidism includes Hyperparathyroid crisis, or “parathyroid storm”.

The main characteristic features include sudden onset of symptomatic, severe hypercalcemia of > 3.5 mmol/L.

There need to be more case reports in the literature presenting Hemorrhage into a parathyroid adenoma as an inciting or associated event.

Gia Gill and colleagues presented a case report of Hemorrhage into a longstanding adenoma. The case presentation included the acute onset of profound hypercalcemia and its associated complications.

The summary of the case report includes the following:

• A 60-year-old male presented with symptoms of sudden onset confusion, weakness of muscle, and ataxia.
• Initial labs showed that the value of serum calcium, parathyroid hormone and creatinine was 4.79 mmol/L, 2043 ng/L, and 364 μmol/L, as previously demonstrated by labs.
• As per the patient’s medical history, he had a history of 4-year recurrent nephrolithiasis.
• There were no prior documented levels of calcium.
• The hypercalcemia was non-responsive to 5 days of aggressive medical management, including fluid resuscitation, denosumab and calcitonin. Later pamidronate and cinacalcet were also included.
• The patient continued to deteriorate.
• He required intubation and continuous renal replacement therapy.
• A 4.8 cm cystic right paratracheal mass was demonstrated by imaging.
• Technetium (Tc99m) Sestamibi scintigraphy was non-localizing.
• Urgent parathyroidectomy was done.
• 5 × 3.3 × 1.8 cm hemorrhagic, atypical hypercellular parathyroid was detected.
• Due to complications arising from anticoagulation therapy in managing deep vein thrombosis, the patient died.
• There was no recovery of renal function at the time of the patient's death.

To conclude,

The researchers gave insight into the aetiology of hyper parathyroid crisis. The report also conveys the difficulty encountered in achieving control of hypercalcemia with medical means.

The hypercalcemia of parathyroid storm is non-responsive to maximal medical therapy. The definitive management is the surgical intervention which should be considered early in these cases.

Further reading:

Gill, G., Agrawal, V. & Kerr, P. Primary hyperparathyroidism presenting as acute hypercalcemic crisis: a case report. J of Otolaryngol - Head & Neck Surg 52, 1 (2023). https://doi.org/10.1186/s40463-022-00600-x