Introduction: Hyperparathyroidism (HPT) in children is rare with limited outcome measures post surgery.
Methods: Retrospective case review of 26 (14M) children (<16 years) who underwent parathyroidectomies (PTx) between 1978 and 2011.
Results: Twenty-six children (14M, 12F) included six neonates with neonatal severe HPT (NSHPT) and 20 older children with HPT (13 sporadic, 7 familial).
All NSHPT neonates were symptomatic at presentation with raised serum calcium (3.038.10 mmol/l) and parathyroid hormone (PTH) (15.8360 pmol/l). 90% of older children were symptomatic with raised serum calcium (2.834.09 mmol/l) and PTH (9.462 pmol/l). Four neonates were tested for mutations of the CaSR gene. Two neonates were second degree relatives and both were identified to be homozygous for Q164X mutation. One neonate was found to have a compound heterozygous mutation with a R680C mutation (paternal) and C60F mutation (maternal). The remaining neonate was identified to have a C570 mutation.
Localisation studies in NSHPT (ultrasound (US), MIBI 2) showed no enlarged glands. In older children sensitivity and specificity of US (13) and MIBI (11) in distinguishing solitary from multi-gland disease was 100%. Accuracy of US and MIBI in predicting laterality of solitary enlarged glands was 100%, and for upper/lower localisation were 72 and 78% respectively.
Children with NSHPT underwent five curative total PTx and one non-curative subtotal PTx. In the older group, children with familial HPT underwent three total and four subtotal PTx (one reoperation in MEN1). Children with sporadic HPT underwent subtotal PTx (before 1980), bilateral neck explorations (19802002) and minimally invasive PTx (>2002). All were cured by first operation. Histology in sporadic HPT included 1 multigland hyperplasia and 12 solitary adenomas. None of the children had post operative complications.
Conclusion: Parathyroid surgery in children is safe and minimally invasive PTx is the operation of choice for children with sporadic HPT.
09 - 11 Nov 2011
British Society for Paediatric Endocrinology and Diabetes