Gouveia Sofia, Rodrigues Dírcea, Barros Luísa, Ribeiro Cristina, Albuquerque Anabela, Costa Gracinda, Carvalheiro Manuela
Endocrinology, Diabetes and Metabolism Department, Coimbra's University Hospital, Coimbra, Portugal.
Arq Bras Endocrinol Metabol. 2012 Aug;56(6):393-403. doi: 10.1590/s0004-27302012000600009.
Primary hyperparathyroidism (PHPT) is a common endocrine disorder that mainly affects middle-aged women. Patients are usually asymptomatic. The disease might be ascribable to hyperplasia, carcinoma, and single or multiple adenomas. PHPT may be sporadic or familial, the latter comprising multiple endocrine neoplasia type 1 or 2A, familial benign hypocalciuria hypercalcemia, and hyperparathyroidism-jaw tumor syndrome. The most common causes for persistent PHPT are multiglandular disease, and missed abnormal ectopic or orthotopic parathyroid glands. Imaging localization studies should precede a new surgical intervention. Ectopic parathyroid glands are rarely located at the aortopulmonary window. For diagnosis confirmation, (99m)Tc-sestamibi SPECT/CT seems to be an advantageous test. Another possibility is to perform (99m)Tc-sestamibi followed by thoracic CT or MRI. Parathyroidectomy may be performed by means of median sternotomy, thoracotomy, or video-assisted thoracoscopy. We describe a case of persistent primary hyperparathyroidism due to the presence of an ectopic parathyroid gland found at the aortopulmonary window. As the investigation necessary to clarify the etiology of recurrent nephrolithiasis proceeded, the diagnosis of PHPT was determined. The patient underwent subtotal parathyroidectomy; nevertheless, PHPT persisted. Genetic syndromes that could account for this condition were excluded. Imaging studies available at that time were not able to locate abnormal glands; moreover, the patient refused to undergo surgical exploration. Later, the patient underwent (99m)Tc-sestamibi SPECT/CT, which revealed a parathyroid gland at the aortopulmonary window.
原发性甲状旁腺功能亢进症（PHPT）是一种常见的内分泌疾病，主要影响中年女性。患者通常无症状。该病可能归因于增生、癌以及单发或多发腺瘤。PHPT 可能是散发性的或家族性的，后者包括 1 型或 2A 型多发性内分泌腺瘤病、家族性良性低钙血症性高钙血症以及甲状旁腺功能亢进 - 颌骨肿瘤综合征。持续性 PHPT 最常见的原因是多腺体疾病，以及遗漏异常的异位或原位甲状旁腺。在进行新的手术干预之前，应先进行影像学定位研究。异位甲状旁腺很少位于主动脉肺窗。为了确诊，（99m）锝 - 甲氧基异丁基异腈单光子发射计算机断层扫描/计算机断层扫描（99mTc - sestamibi SPECT/CT）似乎是一种有利的检查。另一种可能性是先进行（99m）锝 - 甲氧基异丁基异腈检查，然后进行胸部 CT 或磁共振成像（MRI）。甲状旁腺切除术可通过正中胸骨切开术、开胸术或电视辅助胸腔镜手术进行。我们描述了一例因在主动脉肺窗发现异位甲状旁腺而导致持续性原发性甲状旁腺功能亢进症的病例。随着对复发性肾结石病因进行必要的调查，确定了 PHPT 的诊断。患者接受了甲状旁腺次全切除术；然而，PHPT 仍然存在。排除了可能导致这种情况的遗传综合征。当时可用的影像学检查未能定位异常腺体；此外，患者拒绝接受手术探查。后来，患者接受了（99m）锝 - 甲氧基异丁基异腈 SPECT/CT 检查，结果显示在主动脉肺窗有一个甲状旁腺。
