Endocrine Abstracts

Introduction: Sporadic primary hyperparathyroidism (sPHPT) is rare in children. There are few large cohorts characterising presentation, investigations and outcomes. Surgical practice is also changing; minimally invasive parathyroidectomy (MIP) and intra-operative parathyroid hormone (IOPTH) monitoring are being performed more frequently.

Method: Retrospective casenote review of children with sPHPT who had parathyroidectomy between 1977-2022 at a single tertiary endocrine centre.

Results: A total of 30 children had parathyroidectomy for sPHPT, aged 7 to 17 years; 17 (56%) female. Patients presented with (data available,n = 26): abdominal symptoms (14/26, 54%); bone pain, deformity or fracture (7/26, 27%); mood change or lethargy (5/26, 19%); renal stones or haematuria (5/26, 19%); polydipsia and polyuria (3/26, 12%). Incidental hypercalcaemia was reported in 3/26 (12%). Presenting corrected calcium was 3.12 +/- 0.47 mmol/l (mean +/- SD). Pre-surgical treatment included (data available,n = 19): hyperhydration (9/19, 47%); bisphosphonates (5/19, 26%); furosemide (2/19, 11%); cinacalcet (3/19, 16%); calcitonin (1/19, 5%). Data were available for parathyroid ultrasounds in 23/30 patients, for Technetium 99m Sestamibi scans in 18/30, and for both in 17/30. Ultrasound identified abnormal parathyroid in 21/23 (91%); Sestamibi was abnormal in 15/17 (89%); both were abnormal in 13/17 (76%). Of those with discordant imaging, 2/4 had abnormal ultrasound and normal Sestamibi, and 2/4 had abnormal Sestamibi and normal ultrasound. 21/30 (70%) children had MIP, including all operations in the last 15 years (bar one). IOPTH monitoring was used since 2016. One parathyroid gland was removed for 27/30 (90%). Histopathology showed solitary adenoma in 27/30 (3/30 were indeterminate or missing data). Post-operative treatment for hypocalcaemia was oral in 9/30 (30%) patients, intravenous in 3/30 (9%), and not needed in 18/30 (61%). No recorded use of alfacalcidol or calcitriol. There was no recurrence or surgical complications.

Conclusion: We have characterised a large cohort of sPHPT in children. Most patients (88%) present symptomatic of hypercalcaemia. Ultrasound was 91% sensitive for identifying an abnormality. We suggest ultrasound for first line imaging, using Sestimibi when this is reported normal. Performing MIP with IOPTH, we report no surgical complications and no recurrence, and believe it should be considered the operation of choice for children with sPHPT.