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多模态术中神经监测在靠近终丝圆锥的情况下切除终丝室管膜瘤的效用。

Utility of Multimodal Intraoperative Neuromonitoring for Excision of Filum Terminale Ependymoma in Close Proximity to Conus.

机构信息

Neurosurgical Oncology Services, Department of Surgical Oncology, Tata Memorial Centre, Mumbai, India.

Department of Health Sciences, Homi Bhabha National Institute, Mumbai, India.

出版信息

World Neurosurg. 2024 Sep;189:53-54. doi: 10.1016/j.wneu.2024.05.111. Epub 2024 May 23.

Abstract

Myxopapillary ependymomas (MPEs) are well-circumscribed tumors arising mainly from the caudal neuraxis, i.e., conus medullaris (CM) and filum terminale (FT), commonly seen in adults with median age at presentation of 39 years. Owing to its partially aggressive clinical behavior involving cerebrospinal fluid dissemination and local recurrence, MPE is classified as grade 2 in the fifth edition of the World Health Organization Classification of Tumors of the Central Nervous System. Gross total resection without capsular violation is key, with subtotal resection being directly associated with local recurrence.The FT has an intradural and extradural component. The intradural FT extends from the inferior tip of the CM to the coccyx. The intradural FT-CM junction is not demarcated, but rather a zone of transition, with neural tissue being incrementally replaced by fibrous tissue of filum, gradually converging to a pure non-neural FT. In intramedullary FT MPE in close proximity to the lower end of conus, achieving gross total resection presents a great challenge. Neuromonitoring is crucial to ensure preservation of vital CM functions. We present the case of a 33-year-old man with focal nocturnal back pain of 6 months' duration followed by bilateral lower limb deep boring pain. He had no neurological deficits. Preoperative magnetic resonance imaging revealed a T2 hyperintense, heterogeneous contrast-enhancing intradural extramedullary mass at L1. Video 1 highlights step-by-step en bloc excision of the FT MPE with technical nuances, including intraoperative neurophysiological monitoring. Triggered electromyography (EMG) was used to positively map the eloquent CM and identify the intradural FT-conus interface at the superior pole of the tumor, which was then carefully dissected under continuous bulbocavernosus reflex monitoring. Similarly, we confirmed non-neural intradural FT at the lower pole by negative mapping and resected the lesion en bloc with an adequate stump for clear margins. Free-run EMG monitored all the rootlets that adhered to or were in close proximity to the lesion, ensuring their integrity and an uneventful postoperative recovery. Figure 1 depicts the anatomical orientation of the lesion with surrounding neural structures. Histopathology confirmed MPE. En bloc resection with preservation of neurological function remains the mainstay of treatment for FT ependymoma. Understanding the transitional intradural FT-CM interface is essential, often precluding a clear filum stump superiorly while resecting MPE. Intraoperative neurophysiological monitoring is an indispensable adjunct to ensure safe en bloc resection. It is also theoretically possible to use tibial and pudendal sensory evoked potentials (SEPs) in this surgical procedure. However, the clinical utility of SEPs is limited in FT surgery compared with triggered EMG or transcranial motor evoked potentials because conventional SEPs from posterior tibial nerve of the lower extremity do not cover all the root levels at risk, and the change in SEPs cannot be immediately recognized (as SEPs are averaged responses, and there is always a time lag). We did not use pudendal SEPs in this study because SEPs may give information only on the sensory sacral pathway. Dermatomal SEPs may be helpful, but again, they provide only sensory information. Instead, we used triggered EMG for mapping the nerve roots and transcranial motor evoked potentials to monitor the motor tracts. Further, we used the bulbocavernosus reflex, an alternative and more precise technique to monitor both motor and sensory nervous pathways at the sacral root level. Moreover, SEPs are more difficult to monitor in very young children and are less relevant in guiding the surgical strategy. Thus, we used both mapping (triggered EMG) and monitoring (transcranial motor evoked potentials and bulbocavernosus reflex) techniques, which can preserve sensory and motor sacral roots in this surgical procedure.

摘要

粘液性乳头状室管膜瘤(MPE)是一种主要起源于尾部脊索的边界清楚的肿瘤,即脊髓圆锥(CM)和终丝(FT),常见于成年人,中位发病年龄为 39 岁。由于其具有部分侵袭性的临床行为,包括脑脊液播散和局部复发,MPE 在世界卫生组织第五版中枢神经系统肿瘤分类中被归类为 2 级。关键是要做到无包膜侵犯的全切除,次全切除与局部复发直接相关。FT 具有硬膜内和硬膜外两部分。硬膜内 FT 从 CM 的下尖端延伸到尾骨。硬膜内 FT-CM 交界处没有明确的分界,而是一个过渡区,神经组织逐渐被终丝的纤维组织取代,逐渐汇聚到纯非神经的 FT。在靠近圆锥末端的髓内 FT MPE 中,实现全切除是一项巨大的挑战。神经监测对于确保 CM 重要功能的保留至关重要。我们报告了一名 33 岁男性的病例,他有 6 个月的夜间局限性背痛,随后出现双侧下肢深部刺痛。他没有神经功能缺损。术前磁共振成像显示 T2 高信号,不均匀增强的硬膜内髓外肿块位于 L1。视频 1 突出了一步一步地整块切除 FT MPE 的技术要点,包括术中神经生理监测。触发肌电图(EMG)用于积极定位有功能的 CM,并识别肿瘤上极的硬膜内 FT-圆锥界面,然后在持续球海绵体反射监测下仔细解剖。同样,我们通过负向映射确认了下部的非神经硬膜内 FT,并整块切除了病变,留下足够的残端以获得清晰的边缘。自由运行 EMG 监测所有附着在病变上或靠近病变的神经根,以确保其完整性和术后无并发症的恢复。图 1 描绘了病变与周围神经结构的解剖方向。组织病理学证实为 MPE。保留神经功能的整块切除仍然是治疗 FT 室管膜瘤的主要方法。了解过渡性硬膜内 FT-CM 界面至关重要,这常常导致在切除 MPE 时,上方无法明确终丝残端。术中神经生理监测是确保安全整块切除的不可或缺的辅助手段。在这种手术中,理论上也可以使用胫后和阴部感觉诱发电位(SEP)。然而,与触发 EMG 或经颅运动诱发电位相比,SEP 在 FT 手术中的临床应用有限,因为来自下肢后胫神经的常规 SEP 并不能覆盖所有有风险的神经根水平,而且 SEP 的变化不能立即被识别(因为 SEPs 是平均反应,并且总是存在时间滞后)。我们在这项研究中没有使用阴部 SEP,因为 SEP 可能只提供关于感觉骶神经通路的信息。皮节 SEP 可能会有所帮助,但再次强调,它们仅提供感觉信息。相反,我们使用触发 EMG 进行神经根定位,使用经颅运动诱发电位监测运动束。此外,我们使用球海绵体反射,这是一种替代且更精确的技术,可以监测骶神经根水平的运动和感觉神经通路。此外,SEPs 在非常年幼的儿童中更难监测,并且在指导手术策略方面相关性较低。因此,我们使用了定位(触发 EMG)和监测(经颅运动诱发电位和球海绵体反射)技术,这些技术可以在这种手术中保留感觉和运动骶神经根。

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