Surgical management of severe neonatal hyperparathyroidism: one center's experience.
BACKGROUND
Neonatal severe hyperparathyroidism (NSHPT) is a rare disease that presents early with severe hypercalcemia. We reviewed our experience with NSHPT management at a tertiary-care institution.


METHODS
A retrospective chart review was conducted for patients managed for NSHPT over the last 10 years. Demographic, clinical, and follow-up data were collected, and descriptive data were generated.


RESULTS
Five patients were managed for NSHPT, 3 females and 2 males, with a mean age of 18 days at presentation. The mean preoperative parathyroid hormone and calcium levels were 3607 ng/L and 3.84 mmol/L, respectively. Preoperative parathyroid gland localization tests were all negative. All patients had a total parathyroidectomy and autotransplantation at a mean age of 65 days, with a mean follow-up of 5.5 years. One patient had normal parathyroid hormone and normal calcium levels 9.5 years after surgery without medication. One patient had normal levels without medication for 2 years then needed calcium and vitamin D supplements thereafter (8.5 years postoperatively). Three patients are still on calcium and vitamin D supplementation 5.5 years, 3.5 years, and 8 months, respectively, after surgery.


CONCLUSIONS
Neonatal severe hyperparathyroidism is managed effectively with total parathyroidectomy. Preoperative localization studies are not helpful, and autotransplantation has a modest effect in our experience.
Al-Shanafey
Saud
S
King Faisal specialist Hospital and Research Center, P O Box 3354, MBC-40, Riyadh 11211, Kingdom of Saudi Arabia. saud132@hotmail.com
Al-Hosaini
Rana
R
Al-Ashwal
Abdullah
A
Al-Rabeeah
Abdullah
A
D005260:Female; D006801:Humans; D006934:Hypercalcemia / Q000209:etiology / Q000628:therapy; D006961:Hyperparathyroidism / Q000150:complications / Q000601:surgery*; D007231:Infant, Newborn; D008297:Male; D010280:Parathyroid Glands / Q000637:transplantation*; D016105:Parathyroidectomy; D012189:Retrospective Studies; D014182:Transplantation, Autologous; D016896:Treatment Outcome