Department of Surgery, University of Tennessee Health Sciences Center, Chattanooga, TN, USA.
Department of Endocrinology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
World J Surg. 2020 May;44(5):1518-1525. doi: 10.1007/s00268-019-05362-8.
Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Data-driven guidelines for management in pediatric patients are limited.
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%).
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.
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.
儿童和青少年的原发性甲状旁腺功能亢进症（PHPT）并不常见。针对儿科患者的管理，目前的数据驱动型指南有限。
我们对在三个机构接受甲状旁腺切除术的所有年龄≤21 岁的 PHPT 患者（1997 年至 2017 年）进行了回顾性队列分析。分析了临床和人口统计学变量。主要手术结果为治愈（术后 6 个月以上血钙正常）；次要手术结果为手术成功（术中甲状旁腺激素降低≥50%）。
我们确定了 86 名年龄中位数为 17 岁（IQR：14、19）的患者；64%（n=55）为女性。术前血清钙平均值为 11.7mg/dL，中位数甲状旁腺激素（PTH）为 110pg/mL，中位数尿钙为 4.1mg/kg/24h。术前，91%（n=67）的患者 sestamibi 扫描定位，43%（n=19）的患者颈部超声定位。手术中最常见的病理为单个异位甲状旁腺腺瘤，占 71%（n=61）。观察到异位腺瘤（25%，n=22）的发生率较高，最常见于胸腺内（n=13），其次是气管食管沟（n=5）、颈动脉鞘（n=2）和甲状腺内（n=2）。在 56 名可获得术后>6 个月数据的患者中，56 名患者中的 55 名（98%）治愈。1 名因甲状旁腺腺瘤病就诊的患者需要后续再次手术。
在这项多机构的儿童和青少年 PHPT 系列研究中，大多数为散发性 PHPT，由单个腺瘤引起。我们观察到异位甲状旁腺腺瘤的发生率较高，最常见于胸腺内。鉴于儿童患者异位腺瘤的风险较高，儿童和青少年的甲状旁腺手术应由经验丰富的外科医生进行。
