Ghali Michael G Z, Srinivasan Visish M, Hanna Ehab, DeMonte Franco
Department of Neurobiology and Anatomy, Drexel University College of Medicine, Philadelphia, Pennsylvania, USA.
Department of Neurosurgery, Baylor College of Medicine, Houston, Texas, USA.
World Neurosurg. 2017 May;101:559-567. doi: 10.1016/j.wneu.2017.02.073. Epub 2017 Feb 27.
Carotid body paragangliomas are rare, usually benign, tumors arising from glomus cells of the carotid body. Bilateral involvement is present in ∼5% of sporadic cases and up to one third of familial cases. In most patients undergoing bilateral resection of carotid body tumors, a condition known as baroreflex failure syndrome (BFS) develops after resection of the second tumor characterized by headache, anxiety, emotional lability, orthostatic lightheadedness, hypertension, and tachycardia. This condition is believed to result from damage to the carotid baroreceptor apparatus. Patients without overt cardiovascular abnormalities may have subclinical baroreceptor dysfunction evident only on specific testing, measuring heart rate and sympathetic nerve responses to baroloading (e.g., phenylephrine) and barounloading (e.g., Valsalva maneuver). Given the high incidence of BFS in patients undergoing bilateral resection of carotid body tumors, it is suggested that operation is limited to unilateral resection of the dominant/symptomatic lesion and nonsurgical intervention (i.e., embolization, radiotherapy) on the contralateral side. Alternatively, refinement of surgical technique to prevent injury to elements of the baroreceptor apparatus may prevent this complication of bilateral tumor resection.
We present a case of a 16-year-old girl with bilateral jugular vagale and carotid body tumors who developed hypertension after surgical resection of her left jugular vagale tumor and worsening of hypertension concurrent with progression, requiring intensity-modulated radiation therapy and a resection for significant progression of her left jugular vagale tumor. Additional case studies and series of bilateral carotid body tumors and BFS were identified through a comprehensive literature search in the PubMed database.
Our case shows the generalizability of BFS to patients with tumors involving the vagal baroafferent fibers.
颈动脉体副神经节瘤是一种罕见的、通常为良性的肿瘤，起源于颈动脉体的球细胞。约5%的散发性病例及高达三分之一的家族性病例存在双侧受累情况。在大多数接受双侧颈动脉体肿瘤切除术的患者中，在切除第二个肿瘤后会出现一种称为压力反射衰竭综合征（BFS）的病症，其特征为头痛、焦虑、情绪不稳定、体位性头晕、高血压和心动过速。这种病症被认为是由颈动脉压力感受器装置受损所致。没有明显心血管异常的患者可能存在仅在特定测试中才明显的亚临床压力感受器功能障碍，这些测试测量心率以及交感神经对压力负荷（如去氧肾上腺素）和压力卸载（如瓦尔萨尔瓦动作）的反应。鉴于双侧颈动脉体肿瘤切除术患者中BFS的高发生率，建议手术仅限于对优势/有症状病变进行单侧切除，并对侧进行非手术干预（即栓塞、放疗）。或者，改进手术技术以防止损伤压力感受器装置的元件可能会预防双侧肿瘤切除的这种并发症。
我们报告一例16岁女孩，患有双侧颈静脉迷走神经和颈动脉体肿瘤，在手术切除左侧颈静脉迷走神经肿瘤后出现高血压，且随着病情进展高血压恶化，需要调强放射治疗以及因左侧颈静脉迷走神经肿瘤显著进展而进行切除。通过在PubMed数据库中进行全面的文献检索，确定了其他双侧颈动脉体肿瘤及BFS的病例研究和系列报道。
我们的病例表明BFS可推广至涉及迷走神经压力传入纤维的肿瘤患者。
