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Published in: World Journal of Surgery 5/2020

01-05-2020 | Ultrasound | Original Scientific Report

Single Gland, Ectopic Location: Adenomas are Common Causes of Primary Hyperparathyroidism in Children and Adolescents

Authors: Robert D. Rampp, Edna E. Mancilla, N. Scott Adzick, Michael A. Levine, Rachel R. Kelz, Douglas L. Fraker, Pallavi Iyer, Brenessa M. Lindeman, Vicente A. Mejia, Herbert Chen, Heather Wachtel

Published in: World Journal of Surgery | Issue 5/2020

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Abstract

Background

Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Data-driven guidelines for management in pediatric patients are limited.

Methods

We performed a retrospective cohort analysis of all patients (1997–2017) with PHPT ≤ 21 years of age who underwent parathyroidectomy at three institutions. Clinical and demographic variables were analyzed. Primary operative outcome was cure (normocalcemia > 6 months after surgery); secondary outcome was operative success (intraoperative parathyroid hormone decrease of ≥ 50%).

Results

We identified 86 patients with a median age of 17 years (IQR: 14, 19); 64% (n = 55) were female. The mean preoperative serum calcium was 11.7 mg/dL, median parathyroid hormone (PTH) was 110 pg/mL, and median urine calcium was 4.1 mg/kg/24 h. Preoperatively, sestamibi scan localized in 41/71 patients (58%); neck ultrasound localized in 19/44 (43%). The most common pathology at surgery was a single ectopic parathyroid adenoma in 71% (n = 61). A high incidence of ectopic adenomas (25%, n = 22) was observed, most commonly intrathymic (n = 13), followed by tracheoesophageal groove (n = 5), carotid sheath (n = 2), and intrathyroidal (n = 2). Of 56 patients with retrievable data > 6 months postoperatively, cure was achieved in 55 of 56 patients (98%). One patient who presented to us with parathyromatosis require subsequent reoperation.

Conclusion

In this multi-institutional series of PHPT in children and adolescents, the majority were sporadic PHPT and were due to a single adenoma. We observed a high incidence of ectopic parathyroid adenomas, most commonly intrathymic. Given the high risk for ectopic adenoma in pediatric patients, parathyroid surgery in children and adolescents should be performed by experienced surgeons.
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Metadata
Title
Single Gland, Ectopic Location: Adenomas are Common Causes of Primary Hyperparathyroidism in Children and Adolescents
Authors
Robert D. Rampp
Edna E. Mancilla
N. Scott Adzick
Michael A. Levine
Rachel R. Kelz
Douglas L. Fraker
Pallavi Iyer
Brenessa M. Lindeman
Vicente A. Mejia
Herbert Chen
Heather Wachtel
Publication date
01-05-2020
Publisher
Springer International Publishing
Published in
World Journal of Surgery / Issue 5/2020
Print ISSN: 0364-2313
Electronic ISSN: 1432-2323
DOI
https://doi.org/10.1007/s00268-019-05362-8

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