Egebæk Christine Holm, Lilja-Fischer Jacob, Rejnmark Lars, Rolighed Lars
Department of Otorhinolaryngology, Aarhus University Hospital, Aarhus N, Denmark.
Department of Otorhinolaryngology, Aarhus University Hospital, Aarhus N, Denmark.
Int J Surg Case Rep. 2025 Jan;126:110764. doi: 10.1016/j.ijscr.2024.110764. Epub 2024 Dec 24.
Primary hyperparathyroidism (PHPT) is a frequent complication to multiple endocrine neoplasia type 1 (MEN1), presenting challenges due to increased risk of multi-gland disease and recurrence post parathyroidectomy (PTX). This case report examines the management of PHPT in a MEN1 patient, emphasizing possible benefits from intraoperative parathyroid autofluorescence imaging (AF).
A 21-year-old woman with MEN1 presented with mild hyperparathyroidism symptoms in 2014. Initial unilateral PTX in 2015 normalized plasma parathyroid hormone (PTH) and ionized calcium (Ca) but was followed by biochemical recurrence in 2018. In 2022 a subtotal PTX was performed using AF to reduce the risk of permanent hypoparathyroidism. This technique distinguished pathological from non-pathological parathyroid tissue. Most of two glands were removed, leaving a small rim of non-pathologic tissue, achieving long-term normalization of plasma PTH and Ca. Despite prior pregnancy complications suspectedly related to hypercalcemia, she gave birth to a healthy child after the second PTX, who tested negative for MEN1.
This case highlights the complexity of managing MEN1-associated PHPT, demonstrating the importance of advanced surgical techniques like AF to minimize complications and enhance outcomes. Surgical and medical management of MEN1 is crucial given the high recurrence-rate. According to the literature, hypercalcemia in patients with PHPT may affect pregnancy outcomes.
This case-report emphasize the need for a tailored approach in MEN1-related PHPT, combining innovative surgical methods to optimize outcomes. Further research on the impact of MEN1 on pregnancy outcomes and the long-term efficacy of AF are essential for improving management strategies.
原发性甲状旁腺功能亢进症(PHPT)是1型多发性内分泌腺瘤病(MEN1)常见的并发症,由于多腺体疾病风险增加以及甲状旁腺切除术后(PTX)复发,带来了诸多挑战。本病例报告探讨了一名MEN1患者的PHPT管理,强调术中甲状旁腺自体荧光成像(AF)可能带来的益处。
一名21岁的MEN1女性患者于2014年出现轻度甲状旁腺功能亢进症状。2015年首次进行单侧PTX后,血浆甲状旁腺激素(PTH)和离子钙(Ca)恢复正常,但在2018年出现生化复发。2022年,为降低永久性甲状旁腺功能减退的风险,采用AF进行了次全PTX。该技术区分了病理性和非病理性甲状旁腺组织。切除了大部分两个腺体,留下一小圈非病理性组织,使血浆PTH和Ca长期恢复正常。尽管此前怀疑妊娠并发症与高钙血症有关,但她在第二次PTX后生下了一个健康的孩子,该孩子MEN1检测呈阴性。
本病例突出了MEN1相关PHPT管理的复杂性,证明了AF等先进手术技术对于减少并发症和改善预后的重要性。鉴于高复发率,MEN1的手术和药物管理至关重要。根据文献,PHPT患者的高钙血症可能影响妊娠结局。
本病例报告强调了对MEN1相关PHPT采取个性化方法的必要性,结合创新手术方法以优化预后。进一步研究MEN1对妊娠结局的影响以及AF的长期疗效对于改进管理策略至关重要。