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Pediatric-Like Brain Tumors in Adults. 成人小儿类脑肿瘤
Pub Date : 2024-01-01 DOI: 10.1007/978-3-031-53578-9_5
Sandra Fernandes Dias, Oliver Richards, Martin Elliot, Paul Chumas

Pediatric brain tumors are different to those found in adults in pathological type, anatomical site, molecular signature, and probable tumor drivers. Although these tumors usually occur in childhood, they also rarely present in adult patients, either as a de novo diagnosis or as a delayed recurrence of a pediatric tumor in the setting of a patient that has transitioned into adult services.Due to the rarity of pediatric-like tumors in adults, the literature on these tumor types in adults is often limited to small case series, and treatment decisions are often based on the management plans taken from pediatric studies. However, the biology of these tumors is often different from the same tumors found in children. Likewise, adult patients are often unable to tolerate the side effects of the aggressive treatments used in children-for which there is little or no evidence of efficacy in adults. In this chapter, we review the literature and summarize the clinical, pathological, molecular profile, and response to treatment for the following pediatric tumor types-medulloblastoma, ependymoma, craniopharyngioma, pilocytic astrocytoma, subependymal giant cell astrocytoma, germ cell tumors, choroid plexus tumors, midline glioma, and pleomorphic xanthoastrocytoma-with emphasis on the differences to the adult population.

小儿脑肿瘤在病理类型、解剖部位、分子特征和可能的肿瘤驱动因素方面都与成人不同。虽然这些肿瘤通常发生在儿童时期,但也很少出现在成年患者身上,要么是新诊断出的肿瘤,要么是儿童肿瘤在患者转入成人服务后延迟复发的肿瘤。由于儿童类肿瘤在成人中的罕见性,有关这些肿瘤类型在成人中的文献通常仅限于小规模的病例系列,治疗决策通常基于儿童研究中的管理方案。然而,这些肿瘤的生物学特性往往不同于在儿童中发现的相同肿瘤。同样,成人患者往往无法忍受儿童使用的积极治疗方法的副作用,而这些治疗方法在成人中的疗效证据很少或根本没有。在本章中,我们将回顾文献并总结以下儿科肿瘤类型的临床、病理、分子特征和治疗反应:成髓细胞瘤、上胚瘤、颅咽管瘤、朝粒细胞性星形细胞瘤、腮腺下巨细胞星形细胞瘤、生殖细胞瘤、脉络丛肿瘤、中线胶质瘤和多形性黄细胞瘤,并重点介绍其与成人的不同之处。
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引用次数: 0
Clipping of Anterior Circulation Aneurysms: Operative Instructions and Safety Rules for Young Cerebrovascular Surgeons. 夹闭前循环动脉瘤:年轻脑血管外科医生的操作指南和安全规则。
Pub Date : 2024-01-01 DOI: 10.1007/978-3-031-53578-9_7
Carmelo Lucio Sturiale, Alessandro Rapisarda, Alessio Albanese

Introduction: Due to the constant development of the technique, in the last 30 years, the endovascular treatment of the intracranial aneurysms (IAs) has gradually superseded the traditional surgery in the majority of centers. However, clipping still represents the best treatment for some anterior circulation IAs according to their angioarchitectural, topographical, and hemodynamic characteristics. Thus, the identification of residual indications for clipping and the maintenance of training programs in vascular neurosurgery appear nowadays more important than ever.

Materials and methods: We reviewed our last 10-year institutional experience of ruptured and unruptured IAs clipping. We appraised in detail all technical refinements we adopted during this time span and analyzed the difficulties we met in teaching the aneurysm clipping technique to residents and fellows. Then, we described the algorithm of safety rules we used to teach young neurosurgeons how to surgical approach anterior circulation IAs and develop a procedural memory, which may intervene in all emergency situations.

Results: We identified seven pragmatic technical key points for clipping of the most frequent anterior circulation IAs and constructed a didactic approach to teach young cerebrovascular surgeons. In general, they concern craniotomy; cisternostomy; obtaining proximal control; cranial nerve, perforator, and vein preservation; necessity of specific corticectomy; aneurysm neck dissection; and clipping.

Conclusion: In the setting of an IA clipping, particularly when ruptured, the young cerebrovascular surgeon needs to respect an algorithm of safety rules, which are essential not only to avoid major complications, but they may intervene during the difficulties helping to manage potentially life-tethering conditions.

导言:由于血管内治疗技术的不断发展,在过去的 30 年中,大多数中心的颅内动脉瘤(IAs)血管内治疗已逐渐取代了传统手术。然而,对于一些前循环动脉瘤,根据其血管结构、地形和血流动力学特征,剪除仍是最佳治疗方法。因此,识别残余的剪切适应症和维持血管神经外科的培训计划如今显得比以往任何时候都更加重要:我们回顾了本院过去 10 年对破裂和未破裂的椎动脉内膜进行剪切的经验。我们详细评估了在此期间采用的所有技术改进,并分析了在向住院医师和研究员传授动脉瘤夹闭技术时遇到的困难。然后,我们介绍了我们用于向年轻神经外科医生传授前循环动脉瘤手术方法并培养程序记忆的安全规则算法,该算法可在所有紧急情况下进行干预:结果:我们确定了剪除最常见的前循环内膜的七个实用技术要点,并构建了一套教学方法,用于教授年轻的脑血管外科医生。总的来说,这些要点涉及开颅手术;蝶窦造口术;获得近端控制;颅神经、穿孔器和静脉保留;特定皮质切除术的必要性;动脉瘤颈部剥离;以及剪切:结论:在进行动脉瘤夹闭术(尤其是动脉瘤破裂时)时,年轻的脑血管外科医生需要遵守一套安全规则算法,这些规则不仅对避免重大并发症至关重要,而且还能在遇到困难时进行干预,帮助处理可能危及生命的情况。
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引用次数: 0
Values-Based Medicine Is an Ethical Concept for Implementing the Ethical Principles in Daily Practice. 以价值观为基础的医学是在日常实践中贯彻伦理原则的伦理概念。
Pub Date : 2024-01-01 DOI: 10.1007/978-3-031-53578-9_12
Faisal Almatrafi, Ahmed Ammar

Values-based medicine (VsBM) is an ethical concept, and bioethical framework has been developed to ensure that medical ethics and values are implemented, pervasive, and powerful parameters influencing decisions about health, clinical practice, teaching, medical industry, career development, malpractice, and research. Neurosurgeons tend to adopt ethics according to their own values and to what they see and learn from teachers. Neurosurgeons, in general, are aware about ethical codes and the patient's rights. However, the philosophy, concept, and principles of medical ethics are rarely included in the training programs or in training courses. The impact of implementing, observing the medical ethics and the patients' value and culture on the course, and outcome of patients' management should not underestimate. The main principles of medical ethics are autonomy, beneficence, nonmaleficence, justice, dignity, and honesty, which should be strictly observed in every step of medical practice, research, teaching, and publication. Evidence-based medicine has been popularized in the last 40-50 years in order to raise up the standard of medical practice. Medical ethics and values have been associated with the medical practice for thousands of years since patients felt a need for treatment. There is no conflict between evidence-based medicine and values-based medicine, as a medical practice should always be performed within a frame of ethics and respect for patients' values. Observing the principles of values-based medicine became very relevant as multicultural societies are dominant in some countries and hospitals in different corners of the world.

以价值观为基础的医学(VsBM)是一个伦理概念,生物伦理框架的制定是为了确保医学伦理和价值观能够得到贯彻、普及和成为影响健康、临床实践、教学、医疗行业、职业发展、渎职和研究决策的有力参数。神经外科医生倾向于根据自己的价值观以及他们所看到的和从老师那里学到的东西来采纳伦理。一般来说,神经外科医生都了解伦理准则和病人的权利。然而,医学伦理的哲学、概念和原则很少被纳入培训计划或培训课程。执行和遵守医德规范以及病人的价值观和文化对病人管理的过程和结果的影响不容低估。医学伦理的主要原则是自主、受益、非渎职、公正、尊严和诚实,在医疗实践、研究、教学和出版的每一个环节中都应严格遵守。近四五十年来,循证医学得到了普及,以提高医疗实践水平。数千年来,自从病人感到需要治疗以来,医德和价值观就一直与医疗实践联系在一起。循证医学与价值观医学之间并无冲突,因为医疗实践始终应在道德框架内进行,并尊重患者的价值观。随着多元文化社会在一些国家和世界不同角落的医院中占据主导地位,遵守以价值观为基础的医学原则变得非常重要。
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引用次数: 0
Endoscopic Supraorbital Translaminar Approach. 内窥镜眶上横切法
Pub Date : 2024-01-01 DOI: 10.1007/978-3-031-61925-0_13
Mehdi Khaleghi, Kyle C Wu, Daniel M Prevedello

Surgical selection for third ventricle tumors demands meticulous planning, given the complex anatomic milieu. Traditional open microsurgical approaches may be limited in their access to certain tumors, prompting the exploration of alternative techniques. The endoscopic supraorbital translaminar approach (ESOTLA) has emerged as a promising alternative for managing these tumors. By combining a minimally invasive keyhole approach with endoscopic visualization, the ESOTLA provides enhanced illumination and a wider angle of view within the third ventricle. This unique advantage allows for improved access to retrochiasmatic tumors and reduces the need for frontal lobe and optic chiasm retraction required of microscopic techniques, decreasing the risk of neurocognitive and visual deficits. Complications related to the ESOTLA are rare and primarily pertain to cosmetic issues and potential compromise of the hypothalamus or optic apparatus, which can be minimized through careful subarachnoid dissection. This chapter offers a comprehensive description of the technical aspects of the ESOTLA, providing insights into its application, advantages, and potential limitations. Additionally, a case description highlights the successful surgical extirpation of an intraventricular papillary craniopharyngioma via the ESOTLA followed by targeted therapy. To better illustrate the stepwise dissection through this novel approach, a series of cadaveric and intraoperative photographs are included.

鉴于第三脑室肿瘤复杂的解剖环境,手术选择需要精心策划。传统的开放式显微外科手术方法可能在某些肿瘤的入路方面受到限制,这促使人们开始探索替代技术。内窥镜眶上转位方法(ESOTLA)已成为治疗此类肿瘤的一种很有前途的替代方法。通过将微创锁孔方法与内窥镜可视化相结合,ESOTLA 在第三脑室内提供了更强的照明和更宽的视角。这种独特的优势可以更好地探查脑后肿瘤,减少显微镜技术所需的额叶和视丘牵拉,降低神经认知和视觉障碍的风险。与 ESOTLA 相关的并发症非常罕见,主要涉及外观问题和下丘脑或视神经器的潜在损害,通过仔细的蛛网膜下腔剥离可将这些并发症降至最低。本章全面介绍了 ESOTLA 的技术方面,深入剖析了其应用、优势和潜在局限性。此外,本章还通过一个病例重点介绍了通过 ESOTLA 成功手术切除脑室内乳头状颅咽管瘤并随后进行靶向治疗的案例。为了更好地说明这种新方法的分步解剖过程,文中还附有一系列尸体和术中照片。
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引用次数: 0
Purely Endoscopic Evacuation of Intracranial Hematomas. 纯内窥镜颅内血肿清除术。
Pub Date : 2024-01-01 DOI: 10.1007/978-3-031-61925-0_9
Hisayuki Murai, Takuji Yamamoto, Toru Nagasaka

Cerebral hemorrhage is a frequent disease and one of the main causes of disabilities. Even in the case of cerebral hemorrhage, if there were a treatment that would improve the functional prognosis, the benefits would be immeasurable. Although there are limited reports with a high level of evidence in past studies, it has been found that surgery can be effective if a large amount of hematoma can be removed in a minimally invasive manner. Also, it has become clear that the control of bleeding becomes a problem when surgery is performed within 2 days after the onset of stroke and that the therapeutic time window might be longer. In Japan, since the introduction of the transparent sheath by Nishihara et al., endoscopic hematoma removal has been widely performed and has become the standard surgical procedure. The three basic equipment needed for this surgery are a rigid scope, a suction coagulator, and a transparent sheath. The key point of the surgery is to search for hematomas and bleeding points through a transparent sheath and coagulate the bleeding vessels. In this chapter, we will introduce surgical techniques using these devices, but it is important to carefully decide on surgical options by considering your own technique, the condition of each patient, and the devices available in the area.

脑出血是一种常见病,也是导致残疾的主要原因之一。即使是脑出血,如果有一种治疗方法可以改善功能预后,其收益也是不可估量的。虽然以往研究中证据水平较高的报告有限,但研究发现,如果能以微创方式清除大量血肿,手术治疗是有效的。此外,在脑卒中发生后 2 天内进行手术,出血控制会成为问题,治疗时间窗口可能会更长。在日本,自 Nishihara 等人引进透明鞘后,内窥镜血肿清除术已广泛开展,并成为标准手术方法。这种手术所需的三个基本设备是硬镜、抽吸凝固器和透明鞘。手术的要点是通过透明鞘寻找血肿和出血点,并凝固出血血管。在本章中,我们将介绍使用这些设备的手术技术,但重要的是,要根据自己的技术、每位患者的病情以及该地区现有的设备,谨慎决定手术方案。
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引用次数: 0
The Anterior Interhemispheric Transcallosal Approach to the Ventricles: How We Do It. 脑室的前半球间经胼胝体入路:我们是如何做到的
Pub Date : 2024-01-01 DOI: 10.1007/978-3-031-42398-7_7
Lydia J Bernhardt, Alan R Cohen

Intraventricular tumors of the lateral and third ventricles are relatively rare, accounting for 1-2% of all primary brain tumors in most large series [1-4]. They can be uniquely challenging to approach due to their deep location, propensity to become large before they are discovered, and association with hydrocephalus [5, 6]. The surgeon's goal is to develop a route to these deep lesions that will cause the least morbidity, provide adequate working space, and achieve a complete resection. This must be performed with minimal manipulation of the neural structures encircling the ventricles, avoiding functional cortical areas, and acquiring early control of feeding vessels [7, 8].

侧脑室和第三脑室的室管膜瘤相对罕见,在大多数大型系列研究中占所有原发性脑肿瘤的 1-2%[1-4]。由于位置较深、在被发现之前肿瘤就已经变大以及与脑积水有关,这些肿瘤的治疗具有独特的挑战性[5, 6]。外科医生的目标是找到一条通往这些深部病变的路径,以减少发病率、提供足够的工作空间并实现完全切除。在进行手术时,必须尽量减少对环绕脑室的神经结构的操作,避开皮质功能区,并尽早控制进血管[7, 8]。
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引用次数: 0
Jugular Foramen Paragangliomas. 颈静脉孔副神经节瘤
Pub Date : 2024-01-01 DOI: 10.1007/978-3-031-42398-7_10
Guilherme H W Ceccato, Luis A B Borba

Paragangliomas are the most common tumors at jugular foramen and pose a great surgical challenge. Careful clinical history and physical examination must be performed to adequately evaluate neurological deficits and its chronologic evolution, also to delineate an overview of the patient performance status. Complete imaging evaluation including MRI and CT scans should be performed, and angiography is a must to depict tumor blood supply and sigmoid sinus/internal jugular vein patency. Screening for multifocal paragangliomas is advisable, with a whole-body imaging. Laboratory investigation of endocrine function of the tumor is necessary, and adrenergic tumors may be associated with synchronous lesions. Preoperative prepare with alpha-blockage is advisable in norepinephrine/epinephrine-secreting tumors; however, it is not advisable in exclusively dopamine-secreting neoplasms. Best surgical candidates are young otherwise healthy patients with smaller lesions; however, treatment should be individualized each case. Variations of infratemporal fossa approach are employed depending on extensions of the mass. Regarding facial nerve management, we avoid to expose or reroute it if there is preoperative function preservation and prefer to work around facial canal in way of a fallopian bridge technique. If there is preoperative facial nerve compromise, the mastoid segment of the nerve is exposed, and it may be grafted if invaded or just decompressed. A key point is to preserve the anteromedial wall of internal jugular vein if there is preoperative preservation of lower cranial nerves. Careful multilayer closure is essential to avoid at most cerebrospinal fluid leakage. Residual tumors may be reoperated if growing and presenting mass effect or be candidate for adjuvant stereotactic radiosurgery.

副神经节瘤是颈静脉孔处最常见的肿瘤,给外科手术带来了巨大挑战。必须进行仔细的临床病史和体格检查,以充分评估神经功能缺损及其时间演变情况,并对患者的表现状态进行概述。应进行全面的影像学评估,包括核磁共振成像和 CT 扫描,还必须进行血管造影,以描述肿瘤供血和乙状窦/颈内静脉通畅情况。建议通过全身成像筛查多灶性副神经节瘤。有必要对肿瘤的内分泌功能进行实验室检查,肾上腺素能肿瘤可能与同步病变有关。对于分泌去甲肾上腺素/肾上腺素的肿瘤,术前最好使用α-受体阻滞剂;但对于只分泌多巴胺的肿瘤,则不宜使用α-受体阻滞剂。最佳手术人选是病变较小但身体健康的年轻患者,但治疗应因人而异。根据肿块的扩展情况,可采用不同的颞下窝入路。关于面神经的处理,如果术前保留了面神经的功能,我们会避免暴露面神经或改变其走向,而更倾向于采用输卵管桥技术在面神经管周围进行手术。如果术前面神经受损,则需暴露乳突段面神经,如果面神经受侵犯,可进行移植,或仅进行减压。如果术前保留了下颅神经,关键是要保留颈内静脉前内侧壁。仔细的多层闭合对避免脑脊液漏至关重要。残余肿瘤如果不断生长并出现肿块效应,可再次手术,或进行立体定向放射外科辅助治疗。
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引用次数: 0
Transorbital Route to Intracranial Space. 颅内空间的跨轨道路线。
Pub Date : 2024-01-01 DOI: 10.1007/978-3-031-61925-0_14
Alberto Di Somma, Marta Codes, Giulia Guizzardi, Alejandra Mosteiro, Roberto Tafuto, Abel Ferres, Jessica Matas, Alberto Prats-Galino, Joaquim Enseñat, Luigi Maria Cavallo

The endoscopic superior eyelid transorbital approach has emerged as a notable and increasingly utilized surgical technique in recent years. This chapter presents an overview of the approach, tracing its historical development and highlighting its growing acceptance within the skull base community.Beginning with an introduction and historical perspective, the chapter outlines the evolution of the transorbital approach, shedding light on its origins and the factors driving its adoption. Subsequently, a comprehensive exploration of the anatomic bone pillars and intracranial spaces accessible via this approach is provided. Hence, five bone pillars of the transorbital approach were identified, namely the lesser sphenoid wing, the anterior clinoid, the sagittal crest, the middle cranial fossa, and the petrous apex. A detailed correlation of those bone targets with respective intracranial areas has been reported.Furthermore, the chapter delves into the practical application of the technique through a case example, offering insights into its clinical utility, indications, and limitations.

近年来,内窥镜经眶上睑入路已成为一种引人注目且应用日益广泛的外科技术。本章从导言和历史视角入手,概述了经眶入路的演变过程,阐明了其起源和推动其采用的因素。本章从简介和历史角度入手,概述了经眶入路的演变过程,阐明了其起源和推动其采用的因素,随后全面探讨了经眶入路可进入的解剖骨柱和颅内空间。因此,确定了经眶入路的五个骨支柱,即小蝶骨翼、前蝶骨、矢状嵴、中颅窝和鞍顶。此外,本章还通过一个病例深入探讨了该技术的实际应用,对其临床实用性、适应症和局限性提出了见解。
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引用次数: 0
Enhanced Recovery After Surgery (ERAS) Spine Pathways and the Role of Perioperative Checklists. 术后恢复强化(ERAS)脊柱路径和围手术期检查单的作用。
Pub Date : 2024-01-01 DOI: 10.1007/978-3-031-42398-7_5
Scott C Robertson

Enhanced recovery after surgery (ERAS) proposes a multimodal, evidence-based approach to perioperative care. ERAS pathways have been shown to help reduce complications, hospital length of stay (LOS), 30-day readmission rates, pain scores, and ultimately surgical costs, while improving patient satisfaction scores and outcomes in multiple surgical subspecialties [1-6]. Numerous specialties have implemented ERAS programs across the globe, providing a foundation for spine surgeons to begin the process themselves. Over the last few years, a significant number of papers have been addressing ERAS pathways for spinal surgery [7-19]. The majority have addressed the lumbar spine [9, 20-26]. The number of cervical ERAS pathways has been limited [27-29]. Many spine programs have begun the implementation of ERAS pathways, incorporating principles and interventions to various spine surgical procedures. Although differences in implementation across programs exist, there are a few common elements that promote a successful enhanced recovery approach [11, 16, 23, 25, 30-33]. All spinal ERAS pathways have three major elements, which are preoperative, perioperative, and postoperative phases. Within these phases some common elements include preoperative and intraoperative surgical checklists. Intraoperative checklist in addition to the "surgical time out" has been integrated into the workflow of most hospitals doing surgeries and have become a standard of care. The surgical checklist is designed to help reduce surgical errors and prevent wrong site/patient surgeries. Several surgical checklists have been developed throughout the years. Despite these safety protocols wrong site/level and other surgical errors continue to occur. Many cases of wrong level spine surgery (WLSS) still occur even when intraoperative imaging is performed [34, 35]. One survey reported that about 50% of spine surgeons have performed at least one WLSS during their career [36, 37]. Another survey reported that 36% of spine surgeons had performed at least one WLSS that was not recognized intraoperatively [38]. On a similar account, about 30% of spine surgery fellows have experienced wrong-site surgery [39]. From raw incidence rates, WLSS may seem rare, but these surveys show that the experience of WLSS is rather common among spine surgeons. WLSS is not yet a "never event." This may be due to poor quality of the intraoperative images, hindering subsequent level identification [34, 35, 38, 40]. Errors in interpretation of the imaging may also occur, including inconsistency in numbering vertebrae, inconsistency in landmark usage for level counting, and problems with numbering vertebrae due to lumbosacral transitional vertebrae (LSTV) and other anatomical variants [34, 38, 41-43]. This chapter will describe a framework for the development and implementation of ERAS pathway for patients undergoing spine surgery. In addition, we will propose preoperative imaging guidelines and a comprehensive spi

加强术后恢复(ERAS)提出了一种以证据为基础的多模式围手术期护理方法。ERAS 途径已被证明有助于减少并发症、住院时间(LOS)、30 天再入院率、疼痛评分,并最终降低手术成本,同时提高多个外科亚专科的患者满意度评分和疗效[1-6]。全球已有多个专科实施了ERAS计划,为脊柱外科医生自己开始这一过程奠定了基础。在过去几年中,大量论文都在探讨脊柱手术的ERAS途径[7-19]。其中大部分都是针对腰椎的[9, 20-26]。颈椎ERAS路径的数量有限[27-29]。许多脊柱项目已开始实施ERAS路径,将原则和干预措施纳入各种脊柱手术过程。虽然不同项目在实施过程中存在差异,但有一些共同点可以促进成功的强化康复方法[11, 16, 23, 25, 30-33]。所有脊柱 ERAS 途径都有三大要素,即术前、围术期和术后阶段。在这些阶段中,一些共同的要素包括术前和术中手术检查表。除 "手术超时 "外,术中清单已被纳入大多数医院的手术工作流程,并成为护理标准。手术清单旨在帮助减少手术失误,防止错误部位/病人手术。多年来,已开发出多种手术核对表。尽管制定了这些安全规程,但错误的手术部位/层次和其他手术错误仍时有发生。即使进行了术中成像,仍有许多错误水平脊柱手术(WLSS)病例发生[34, 35]。一项调查报告显示,约 50%的脊柱外科医生在其职业生涯中至少实施过一次 WLSS [36,37]。另一项调查报告显示,36% 的脊柱外科医生至少实施过一次术中未被发现的 WLSS [38]。与此类似,约有 30% 的脊柱外科实习医生经历过错位手术 [39]。从原始发生率来看,WLSS 似乎很少见,但这些调查显示,WLSS 在脊柱外科医生中相当普遍。WLSS尚未成为 "从未发生的事件"。这可能是由于术中图像质量不佳,妨碍了随后的水平鉴定[34, 35, 38, 40]。影像解读错误也可能发生,包括椎体编号不一致、用于水平计数的地标使用不一致,以及腰骶部过渡椎体(LSTV)和其他解剖变异导致的椎体编号问题[34,38,41-43]。本章将介绍为脊柱手术患者制定和实施 ERAS 路径的框架。此外,我们还将提出术前成像指南和综合脊柱手术清单,以纳入围手术期阶段,帮助减少进一步的手术失误和 WLSS。
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引用次数: 0
Multimodal Approach for the Treatment of Complex Hypothalamic Hamartomas. 治疗复杂下丘脑 Hamartomas 的多模式方法。
Pub Date : 2024-01-01 DOI: 10.1007/978-3-031-53578-9_4
José Hinojosa, Santiago Candela-Cantó, Victoria Becerra, Jordi Muchart, Marta Gómez-Chiari, Jordi Rumia, Javier Aparicio

Hypothalamic hamartomas (HHs) are rare congenital lesions formed by heterotopic neuronal and glial cells attached to the mammillary bodies, tuber cinereum, and hypothalamus.They often present with an intractable epilepsy typically characterized by gelastic seizures but commonly associated with other types of refractory seizures. The clinical course is progressive in most of the cases, starting with gelastic seizures in infancy and deteriorating into complex seizure disorders that result in catastrophic epilepsy associated with cognitive decline and behavioral disturbances.Hamartomas are known to be intrinsically epileptogenic and the site of origin for the gelastic seizures. As antiepileptic drugs are typically ineffective in controlling HH-related epilepsy, different surgical options have been proposed as a treatment to achieve seizure control. Resection or complete disconnection of the hamartoma from the mammillothalamic tract has proved to achieve a long-lasting control of the epileptic syndrome.Usually, symptoms and their severity are typically related to the size, localization, and type of attachment. Precocious puberty appears mostly in the pedunculated type, while epileptic syndrome and behavioral decline are frequently related to the sessile type. For this reason, different classifications of HHs have been developed based on their size, extension, and type of attachment to the hypothalamus.The bigger and more complex hypothalamic hamartomas typically present with severe refractory epilepsy, behavioral disturbances, and progressive cognitive decline posing a formidable challenge for the control of these symptoms.We present here our experience with the multimodal treatment for complex hypothalamic hamartomas. After an in-depth review of the literature, we systematize our approach for the different types of hypothalamic hamartomas.

下丘脑火腿状瘤(HHs)是一种罕见的先天性病变,由附着在乳腺体、小结节和下丘脑上的异位神经元和胶质细胞形成。大多数病例的临床病程呈进行性发展,从婴儿期的凝胶样发作开始,逐渐恶化为复杂的发作性疾病,导致伴有认知能力下降和行为障碍的灾难性癫痫。已知 Hamartomas 本身具有致痫性,是凝胶样发作的起源部位。由于抗癫痫药物通常无法有效控制 HH 相关性癫痫,因此人们提出了不同的手术治疗方案,以达到控制癫痫发作的目的。事实证明,切除火腿状瘤或将其与乳突丘脑束完全断开可长期控制癫痫综合征。性早熟多见于有蒂型,而癫痫综合征和行为衰退则常与无梗型有关。更大、更复杂的下丘脑仓鼠瘤通常伴有严重的难治性癫痫、行为障碍和进行性认知功能下降,这给控制这些症状带来了巨大挑战。我们在此介绍我们对复杂下丘脑仓鼠瘤进行多模式治疗的经验。在深入查阅文献后,我们系统地介绍了针对不同类型下丘脑仓瘤的治疗方法。
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引用次数: 0
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Advances and technical standards in neurosurgery
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