Endocrine Abstracts

JOINT2294

Introduction: Primary Hyperparathyroidism (PHPT) in children is very rare and arrival of better imaging, minimally invasive parathyroidectomy (MIP) and intra-operative parathyroid hormone (IOPTH) monitoring is changing current surgical practice.

Method: Retrospective case notes review of children with PHPT who had parathyroidectomy between 1977 and 2022 at a single tertiary endocrine centre.

Results:: A total of 49 children (0.2–17 years, 24 boys) had parathyroidectomy for PHPT; 30 (7–17 years) for sporadic (sPHPT), 10 (6–16 years) familial (fPHPT, 6MEN1, 2 MEN2a, 2JT-HPT)) and 9 (0.2–3 years) Neonatal Severe Hyperparathyroidism (NSHPT). Children with sPHPT presented most commonly with abdominal symptoms (54%), incidental hypercalcaemia was reported in 12%. 44% of children with fPHPT had abdominal symptoms; 33% hypercalcaemia on screening. All children with NSHPT presented with poor feeding, behavioural change and/or developmental delay, except one with incidental hypercalcaemia. Preoperative corrected calcium was 3.12±0.47 mmol/l (mean±S.D.) in sPHPT, 2.97±0.31 mmol/l (P=0.4) in fPHPT and 4.6±1.6 mmol/l (P<0.05) in NSHPT. Pre-surgical treatment was most commonly hyperhydration and bisphosphonates; cinacalcet was used in 3 sPHPT and 4 NSHPT; calcitonin in 1 sPHPT and 2 NSHPT. In children with sPHPT ultrasound identified abnormal parathyroid in 91%; Sestamibi was abnormal in 89%; both abnormal in 76%. Of those with discordant imaging, 2/4 had abnormal ultrasound and normal Sestamibi, and 2/4 had abnormal Sestamibi and normal ultrasound. In children with fPHPT 86% of ultrasound images were abnormal, with Sestamibi in agreement except for 1 case showing no uptake. When imaging was performed in NSHPT (66%), no abnormal parathyroids were identified. Seventy percent of sPHPT children (all but one in the last 15 years) had MIP and 90% removal of 1 parathyroid). IOPTH monitoring was used in all operations since 2016. 20% of children with fPHPT had MIP and others had neck exploration with removal of 1(20%) – 4(20%) parathyroids. Eight children with NSHPT had 4 and one 3½ parathyroidectomies. Recurrence rate in children with fPHPT was 40% and 0% in sPHPT. There were no surgical complications.

Conclusion: Most children with PHPT are symptomatic and have high levels of calcium meeting criteria for immediate surgery. Ultrasound correctly identifies abnormal parathyroids and should be first line imaging in children with sPHPT and fPHPT, with Sestamibi recommended only when US negative. Imaging parathyroids is not helpful in NSHPT. Parathyroidectomy in children is safe in experienced hands and MIP with IOPTH should be the operation of choice for sPHPT.