Neonatal Severe Hyperparathyroidism: Anaesthetic Considerations for Removal of Pea-size Glands in Children

Neonatal severe hyperparathyroidism (NSHPT) is an extremely rare disorder with uncontrolled severe hypercalcemia and its clinical manifestations. It is caused by a mutation in the <i>calcium-sensing receptor (CaSR)</i> gene, which modulates the negative feedback of parathormone. We present anaesthetic management of two children with NSHPT who were posted for total parathyroidectomy as a life saving procedure. Both patients presented with lethargy, failure to thrive, and hypotonia. Intraoperative anaesthetic challenges include susceptibility to bradycardia, prolonged QT interval, precipitation of hypercalcemic crisis in the form of renal failure, hyperkalemia and electrocardiography changes, unpredictable response to neuromuscular blockade, susceptibility to recurrent laryngeal nerve injury, refractory hypocalcemia, which may start developing within six hours after surgery. Anaesthetic goals include preoperative optimisation of serum calcium with subcutaneous. Calcitonin, intravenous pamidronate and tablet cinacalcet, which are calcimimetics, maintenance of hydration and readiness to deal with intraoperative hypercalcemic crises. Anaesthetic management of NSHPT posted for total parathyroidectomy is challenging. To the best of our knowledge, there is no anaesthetic literature published to this day and only four surgical cases have been reported. Genome sequencing in both patients showed a <i>CaSR</i> gene mutation that is homozygous for a suspected pathogenic variant. Management requires a preoperative multidisciplinary approach for severe hypercalcemia and postoperative refractory hypocalcemia. These patients need lifelong calcium and vitamin D supplementation.