tethered cord syndrome

脊髓栓系综合征
  • 文章类型: Journal Article
    背景:脊髓栓系综合征(TCS)是一种罕见的神经系统疾病,其特征是脊髓远端纵向拉伸。这种情况通常表现为腰骶部和下肢疼痛和虚弱,感觉障碍,和尿失禁。传统上,系绳释放一直是TCS的一线管理,但常见的并发症如脑脊液漏。因此,脊柱缩短术(SCS)椎体截骨术已成为一种潜在的替代方法。
    方法:这里,作者描述了1例48岁男性患者的首次单阶段俯卧侧位SCS椎体截骨术同时进行后方暴露,患者先前接受过多次直接分离手术.作者强调了案例介绍,手术技术,和术后过程。手术后,没有立即的手术并发症,患者注意到神经根疼痛和神经功能的临床改善。
    结论:本病例进一步支持SCS椎体截骨术作为TCS患者的有效治疗选择。它还证明了具有后部暴露的单阶段侧向入路作为脊柱缩短手术的微创选择的潜力。然而,有必要使用扩展队列和评估各种手术技术进行进一步研究.https://thejns.org/doi/10.3171/CASE24185.
    BACKGROUND: Tethered cord syndrome (TCS) is a rare neurological disorder characterized by longitudinal stretching on the distal end of the spinal cord. The condition commonly manifests in lumbosacral and lower-extremity pain and weakness, sensory disturbances, and incontinence. Traditionally, tethered cord release has been the first-line management for TCS, but retethering and complications such as cerebrospinal fluid leakage are commonly reported. As a result, spinal column shortening (SCS) vertebral osteotomy has emerged as a potential alternative.
    METHODS: Herein, the authors describe the first single-stage prone lateral SCS vertebral osteotomy with simultaneous posterior exposure in a 48-year-old male patient with multiple prior direct detethering procedures. The authors highlight the case presentation, operative technique, and postoperative course. Following surgery, there were no immediate surgical complications, and the patient noted clinical improvement in his radicular pain and neurological function.
    CONCLUSIONS: This case further supports SCS vertebral osteotomy as an effective treatment option for patients with TCS. It also demonstrates the potential for a single-stage lateral approach with posterior exposure as a minimally invasive option for spinal shortening procedures. However, further studies using expanded cohorts and assessing various surgical techniques are warranted. https://thejns.org/doi/10.3171/CASE24185.
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  • 文章类型: Journal Article
    背景:受脊髓栓系综合征(TCS)影响的儿童遇到多方面的挑战,包括教育,家庭和社会领域,强调对他们的主观情感幸福感和生活遭遇进行整体理解的重要性。尽管如此,医疗保健专业人员倾向于在整个治疗和康复过程中优先考虑患病个体的身体功能,经常忽略这些孩子的情感体验和要求,因为他们过渡到后阶段。
    目的:提高TCS患儿在重返家庭后的主观体验和认识,从医疗机构出院后的教育机构和更广泛的社会环境。
    方法:本研究在浙江省儿童医院进行。12名年龄在8-15岁的TCS儿童被纳入研究。研究设计采用了解释性定性方法,利用半结构化访谈作为主要的数据收集方法。数据分析采用反身性专题分析,促进对新兴主题和模式的全面探索。
    结果:从调查结果中确定了四个主要主题(和七个子主题):(1)成长的痛苦(一个可耻的秘密,理想与现实之间的距离);(2)家庭感情的不恰当表达(知道就是不理解,无法形容的内疚);(3)社会疏远(令人不快的区别,熟悉的陌生人)和(4)争取独立性和一致性。
    结论:受TCS影响的儿童在自我发育中表现出内在敏感性和挑战,家庭动态和社会互动。他们渴望实现一个以独立和自由为特征的未来,类似于他们典型的发展中的同龄人。这些发现可以帮助卫生专业人员,家庭和教育工作者对成为患有TCS的孩子有了更深入的了解,这些发现还可以作为一个干预平台,寻求促进这些儿童的自我表达,使他们能够将生活作为一个有意义和积极的过程来体验。
    这项研究在数据收集过程中得到了患有TCS的儿童及其监护人的支持,以及该部门的护士长。Coresearchers还为设计做出了贡献,数据收集,分析和写作。
    BACKGROUND: Children affected by tethered cord syndrome (TCS) encounter multifaceted challenges encompassing educational, familial and social spheres, underscoring the significance of a holistic comprehension of their subjective emotional well-being and life encounters. Nonetheless, healthcare professionals tend to prioritise the physical functionality of the afflicted individuals throughout the treatment and rehabilitation process, often neglecting the emotional experiences and requirements of these children as they transition into posthospitalization phases.
    OBJECTIVE: To advance the subjective experiences and perceptions of children with TCS upon reintegration into their families, educational institutions and wider societal contexts subsequent to their discharge from medical facilities.
    METHODS: The study was conducted at the Children\'s Hospital in Zhejiang. Twelve children aged 8-15 with TCS were included in the study. The research design used an interpretative qualitative approach, utilising semi-structured interviews as the primary data collection method. Data analysis was performed using reflexive thematic analysis, facilitating a comprehensive exploration of emerging themes and patterns.
    RESULTS: Four major themes (and seven subthemes) were identified from the findings: (1) growing pains (a shameful secret, distance between ideal and reality); (2) inappropriate expressions of familial affection (knowing is not understanding, unspeakable guilt); (3) social estrangement (uncomfortable distinctions, familiar stranger) and (4) striving for independence and consistency.
    CONCLUSIONS: Children affected by TCS exhibit internal sensitivity and challenges in self-development, family dynamics and social interactions. They aspire to attain a future characterised by independence and freedom, akin to that of their typically developing peers. These findings can help health professionals, families and educators gain a deeper understanding of what it takes to be a child with TCS, and the findings can also serve as a platform for interventions that seek to promote self-expression in these children so that they can experience life as a meaningful and positive process.
    UNASSIGNED: This study received support from children with TCS and their guardians during data collection, as well as from the head nurse of the unit. Coresearchers also contributed to design, data collection, analysis and writing.
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  • 文章类型: Journal Article
    Ehlers-Danlos综合征(EDS)代表一组遗传性结缔组织疾病,其中超移动亚型(hEDS)是最普遍的。hEDS表现为跨越肌肉骨骼的各种临床症状和相关的合并症,神经学,胃肠,心血管,和免疫系统。hEDS患者可能会出现脊髓神经并发症,包括由颅颈和/或宫颈不稳定/活动过度引起的宫颈延髓症状,以及脊髓栓系综合征(TCS)。TCS在自然界中通常是影像学隐匿性的,在标准成像上并不总是能检测到,并表现为下背部疼痛,平衡问题,下肢无力,感官损失,肠或膀胱功能障碍。由于韧带松弛引起的颈椎不稳定会导致头痛,眩晕,耳鸣,视力变化,晕厥,神经根病,疼痛,和吞咽困难.TCS和宫颈不稳定不仅具有共同的临床特征,而且在hEDS患者中也可以同时发生,在诊断和临床管理方面构成挑战。我们提出的文献和一个20岁的女性hEDS的案例研究的综述,他们接受了这些疾病的手术干预,强调诊断和管理这些复杂性的挑战,并强调量身定制的治疗策略对改善患者预后的重要性。
    The Ehlers-Danlos Syndromes (EDS) represent a group of hereditary connective tissue disorders, with the hypermobile subtype (hEDS) being the most prevalent. hEDS manifests with a diverse array of clinical symptoms and associated comorbidities spanning the musculoskeletal, neurological, gastrointestinal, cardiovascular, and immunological systems. hEDS patients may experience spinal neurological complications, including cervico-medullary symptoms arising from cranio-cervical and/or cervical instability/hypermobility, as well as tethered cord syndrome (TCS). TCS is often radiographically occult in nature, not always detectable on standard imaging and presents with lower back pain, balance issues, weakness in the lower extremities, sensory loss, and bowel or bladder dysfunction. Cervical instability due to ligament laxity can lead to headaches, vertigo, tinnitus, vision changes, syncope, radiculopathy, pain, and dysphagia. TCS and cervical instability not only share clinical features but can also co-occur in hEDS patients, posing challenges in diagnostics and clinical management. We present a review of the literature and a case study of a 20-year-old female with hEDS, who underwent surgical interventions for these conditions, highlighting the challenges in diagnosing and managing these complexities and underscoring the importance of tailored treatment strategies to improve patient outcomes.
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  • 文章类型: Case Reports
    幕下浅铁质沉着症,其特征是含铁血黄素沉积在脑干的脑膜下层,小脑,和脊髓,是一种罕见的进行性神经系统疾病.我们介绍了两例幕下浅表铁质沉着症。病例1涉及一名62岁女性,先前被诊断患有脊髓栓系综合征和胸椎脊髓病,谁,在11次脊柱手术之后,表现为脊髓病恶化,听力损失,和认知障碍。脑磁共振成像(MRI)显示广泛的浅表铁质沉着影响小脑和双侧小脑半球。病例2是一名27岁的男性,患有枪伤造成的创伤性T4脊髓损伤,被一个syrinx复杂化了,经历持续的下背部疼痛和下肢痉挛。MRI证实脊髓浅表铁质沉着症。本病例报告探讨了临床表现,影像学发现,管理策略,以及这些病例的预后。它还强调了幕下浅表铁质沉着症的各种临床表现和潜在病因。它强调了具有铁敏感序列的MRI在明确诊断中的关键作用。此外,管理层强调了多学科团队方法在为受影响的个人提供全面护理方面的重要性。
    Infratentorial superficial siderosis, characterized by hemosiderin deposition in the subpial layers of the brainstem, cerebellum, and spinal cord, is a rare progressive neurologic disorder. We present two cases of infratentorial superficial siderosis. Case 1 involves a 62-year-old female previously diagnosed with tethered cord syndrome and thoracic myelopathy, who, following 11 spinal surgeries, presented with worsening myelopathy, hearing loss, and cognitive impairment. Brain magnetic resonance imaging (MRI) revealed extensive superficial siderosis affecting the cerebellar vermis and bilateral cerebellar hemispheres. Case 2 is a 27-year-old male with a traumatic T4 spinal cord injury from a gunshot wound, complicated by a syrinx, experiencing persistent lower back pain and lower limb spasticity. MRI confirmed superficial siderosis in the spinal cord. This case report explores the clinical manifestations, imaging findings, management strategies, and prognosis of these cases. It also highlights the diverse clinical presentations and underlying etiologies of infratentorial superficial siderosis. It emphasizes the pivotal role of MRI with iron-sensitive sequences for definitive diagnosis. Furthermore, the management underscores the significance of a multidisciplinary team approach in providing comprehensive care for affected individuals.
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  • 文章类型: Journal Article
    目的:本研究旨在探讨临床特征的差异,诊断检查,治疗,和成人发作和儿童发作的脊髓栓系综合征(TCS)之间的病理结果。
    方法:作者搜索了PubMed,Embase,和CochraneLibrary数据库到2023年1月,用于TCS报告,提取临床特征信息,成像数据,治疗方式,预后,和病理研究结果。分析共纳入246篇文献中的6135例。这项审查是根据2020年PRISMA指南进行的,并在PROSPERO上注册。
    结果:成人最常见的临床表现是疼痛,泌尿症状,麻木;在儿童中,它们是泌尿症状,皮肤损伤,肠道症状,和非特异性运动缺陷。手术治疗是成人和儿童的主要方法,在成人中观察到更高的临床改善率。然而,成人的手术并发症发生率也高于儿童.TCS病理学研究尚未确定成人和儿童之间的差异,成人发病TCS的发病机制需要进一步研究。
    结论:成人起病和儿童起病的TCS在临床特征上表现出一定的差异,诊断检查,和治疗。然而,在目前的病理研究中,成人和儿童之间没有发现显着差异。系统审查登记号。:CRD42023479450(www.crd.约克。AC.英国/普劳迪略)。
    This study aimed to investigate the differences in clinical features, diagnostic examination, treatment, and pathological results between adult-onset and pediatric-onset tethered cord syndrome (TCS).
    The authors searched the PubMed, Embase, and Cochrane Library databases through January 2023 for reports on TCS, extracting information on clinical features, imaging data, treatment modalities, prognosis, and pathological research results. A total of 6135 cases from 246 articles were included in the analysis. This review was conducted in accordance with the 2020 PRISMA guidelines and registered on PROSPERO.
    The most common adult clinical manifestations were pain, urinary symptoms, and numbness; in children, they were urinary symptoms, skin lesions, bowel symptoms, and unspecific motor deficits. Surgical treatment was the primary approach for both adults and children, with a higher clinical improvement rate observed in adults. However, adults also had a higher rate of surgical complications than children. TCS pathological studies have not yet identified the differences between adults and children, and the pathogenesis of adult-onset TCS requires further investigation.
    Adult-onset and pediatric-onset TCS exhibit certain differences in clinical characteristics, diagnostic examinations, and treatments. However, significant differences have not been found in current pathological studies between adults and children. Systematic review registration no.: CRD42023479450 (www.crd.york.ac.uk/prospero).
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  • 文章类型: Journal Article
    脊髓栓系综合征是一种脊髓被病理结构(如终末紧纤维)栓系的病症,有或没有连接的硬膜外成分的硬膜内脂肪瘤,硬膜内纤维粘连,脊髓纵裂,和脊髓膜膨出闭合后的神经胎盘粘连。它通常发生在童年和青春期,随着脊柱的长度增长,但它也可以在成年后发展。脊髓栓系综合征的症状是缓慢进展和变化的。如果医生缺乏对这种疾病的知识和理解,可能会提供不正确的诊断和不适当的治疗。本章旨在描述病理生理学,综合症,诊断成像,手术治疗,和脊髓栓系综合征的预后,以提高对这种情况的认识。
    Tethered cord syndrome is a condition in which the spinal cord is tethered by pathological structures such as a tight filum terminale, intradural lipomas with or without a connecting extradural component, intradural fibrous adhesions, diastematomyelia, and neural placode adhesions following closure of a myelomeningocele.It usually occurs in childhood and adolescence as the spine grows in length, but it can also develop in adulthood. Symptoms of tethered cord syndrome are slowly progressive and varied. Incorrect diagnosis and inappropriate treatment may be provided if the physician lacks knowledge and understanding of this disease.This chapter aims to describe the pathophysiology, syndromes, diagnostic imaging, surgical treatment, and prognosis of tethered cord syndrome to enhance the understanding of this condition.
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  • 文章类型: Journal Article
    脊髓栓系综合征(TCS)的手术治疗包括椎板切开术,用于终丝硬膜内溶解(LFT),目的是通过分割终丝来释放延髓圆锥上的多余张力。虽然LFT缓解了临床症状,它与风险和并发症有关,包括脑脊液(CSF)泄漏和感染,无论是肤浅的还是深的。LFT的一些风险和并发症与原发性硬脑膜闭合的效率和质量及其下游影响有关。我们试图评估非穿透性钛夹(TC)用于原发性硬脑膜闭合的实用性,特别关注手术持续时间。相关成本,以及一系列接受LFT的儿科患者的并发症情况,假设使用TC导致更有效的封堵,因此可能降低成本和潜在相关的麻醉长度和风险。
    4位外科医生,在2022年7月至2023年5月期间,28例儿科患者接受了LFT,随后使用AnastoClip®非穿透性钛夹或传统缝合技术进行了硬脑膜封堵.为了比较安全性,功效,以及两种硬脑膜闭合技术之间的成本效益,收集了相关数据,包括患者的人口统计学和CSF泄漏率,三个月随访时感染,再操作。记录从硬脑膜闭合开始到结束的手术持续时间和时间。
    共有28名儿科患者(平均年龄:5.9岁,43%是女性,范围:0.71-17年),TCS接受LFT。所有患者均接受了涉及腰椎硬膜内手术的手术。19例(67.9%)采用传统缝合,9例(32.1%)采用TC进行硬脑膜闭合。关于硬脑膜闭合的持续时间,使用传统缝合技术的平均闭合时间为1271秒(或21分钟和11秒),而TC的平均时间为265s(或4min和25s)。在三个月的随访中,在缝合队列中观察到1例脑脊液(CSF)渗漏或感染,需要再次手术.
    TC组的临床结果非常好,与以前的报道一致;我们的发现进一步表明,TC比传统缝合技术更有效地闭合硬脑膜。我们的研究结果表明,TC可能是安全的,有效的,在接受LFT手术的TCS患儿中,传统缝合的替代方法更有效地实现硬脑膜闭合。
    UNASSIGNED: Surgical treatment for tethered cord syndrome (TCS) involves a laminotomy for intradural lysis of filum terminale (LFT), with the goal of releasing excess tension on the conus medullaris by dividing the filum terminale. While LFT alleviates clinical symptoms, it is associated with risks and complications, including cerebrospinal fluid (CSF) leak and infection, either superficial or deep. Some risks and complications of LFT relate to efficiency and quality of primary dural closure and its downstream effects. We sought to assess the utility of nonpenetrating titanium clips (TC) for primary dural closure with a particular focus on operative duration, associated costs, and complication profiles in a series of pediatric patients undergoing LFT, hypothesizing that TC utilization leads to more efficient closure and therefore potentially lower costs and potentially associated anesthetic length and risks.
    UNASSIGNED: A 4-surgeon, single institution series of 28 pediatric patients underwent LFT with subsequent dural closure performed with either the AnastoClip® nonpenetrating titanium clips or traditional suture technique between July 2022 and May 2023. In order to compare the safety, efficacy, and cost-effectiveness between the two dural closure techniques, relevant data were collected including patient demographics and rates of CSF leak, infection at three-month follow-up, and reoperation. Operative durations and times from beginning to end of dural closure were recorded.
    UNASSIGNED: A total of 28 pediatric patients (mean age: 5.9 years, 43% female, range: 0.71-17 years) with TCS underwent LFT. All patients underwent procedures involving intradural surgery of the lumbar region. Dural closure was performed using traditional suturing in 19 patients (67.9%) and TC in 9 (32.1%). With respect to duration of dural closure, the average time to closure using traditional suturing techniques was 1271 s (or 21 min and 11 s), while the average time for TC was 265 s (or 4 min and 25 s). At three-month follow-up, one case of cerebrospinal fluid (CSF) leak or infection was observed in the suture cohort and required reoperation.
    UNASSIGNED: Clinical outcomes in the TC group were excellent, consistent with previous reports; our findings further suggest that TCs result in more efficient dural closure than traditional suturing techniques. Our findings suggest that TC may be a safe, efficacious, and more efficient alternative to traditional suture for achieving dural closure in pediatric patients with TCS undergoing LFT surgery.
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  • 文章类型: Journal Article
    目标:时间驱动的基于活动的成本核算(TDABC)是一种用于成本核算的方法,在卫生经济学中获得了牵引力,以确定价值优化计划。它衡量时间,将价值分配给花费在患者身上的时间增量,并整合每集护理中使用的材料和人力资源成本。在这项研究中,作者报告了首次使用TDABC评估儿科神经外科手术的费用.
    方法:由多功能团队开发临床路径。每个护理团队成员的时间调查,包括外科医生,医疗助理(MA),和病人服务代表(PSRs),在儿科神经外科诊所进行了为期10周的前瞻性研究。连续患者遭遇Chiari畸形(CM),脑积水,或脊髓栓系综合征(TCS)包括在内。相遇被归类为新的或已建立的。相对年度人员成本,使用PSR的工资作为参考(即,1.0-单位成本),使用调整后的部门财务数据为所有成员计算。每个人员的相对容量成本率(分钟-1),表示每分钟的人均成本,然后得出,并计算了每次就诊的相对费用。
    结果:共110次就诊(24次新,86个已建立)被捕获,包括40%CM,41%脑积水,和19%的TCS遭遇。外科医生的相对容量成本率最高(118.4×10-6),比MA或PSR高10倍以上(分别为10.65×10-6和9.259×10-6)。在几乎所有就诊中,与其他护理团队相比,外科医生在患者身上的时间也更多(p≤0.002);因此,总就诊费用主要由外科医生费用驱动(p<0.0001).总的来说,外科医生费用占总就诊费用的绝大部分(92%-93%),不管访问是新的还是既定的。就诊费用因诊断而异。平均而言,新访视时间长于既定访视时间(p<0.001).这种差异主要是由新的CM访问(44.3±13.7分钟)驱动的,显着长于已建立的CM访问(29.8±9.2分钟;p=0.001)。
    结论:TDABC可能通过突出每个护理提供模块中的可变性和高成本的实例来揭示最大化价值的机会。儿科神经外科的医师领导者可能能够使用这些信息来分配成本并简化价值护理途径。
    OBJECTIVE: Time-driven activity-based costing (TDABC) is a method used in cost accounting that has gained traction in health economics to identify value optimization initiatives. It measures time, assigns value to time increments spent on a patient, and integrates the cost of material and human resources utilized in each episode of care. In this study, the authors report the first use of TDABC to evaluate costs in a pediatric neurosurgical practice.
    METHODS: A clinical pathway was developed with a multifunction team. A time survey among each care team member, including surgeons, medical assistants (MAs), and patient service representatives (PSRs), was carried out prospectively over a 10-week period at a pediatric neurosurgery clinic. Consecutive patient encounters for Chiari malformation (CM), hydrocephalus, or tethered cord syndrome (TCS) were included. Encounters were categorized as new or established. Relative annual personnel costs, using the salary of a PSR as a reference (i.e., 1.0-unit cost), were calculated for all members using departmental financial data after adjustments. The relative capacity cost rates (minute-1) for each personnel, a representation of per capita cost per minute, were then derived, and the relative costs per visit were calculated.
    RESULTS: A total of 110 visits (24 new, 86 established) were captured, including 40% CM, 41% hydrocephalus, and 19% TCS encounters. Surgeons had the highest relative capacity cost rate (118.4 × 10-6), more than 10-fold higher than that of an MA or PSR (10.65 × 10-6 and 9.259 × 10-6, respectively). Surgeons also logged more time with patients compared with the rest of the care team in nearly all visits (p ≤ 0.002); consequently, the total visit costs were primarily driven by the surgeon cost (p < 0.0001). Overall, surgeon cost constituted the vast majority of the total visit cost (92%-93%), regardless of whether the visits were new or established. Visit costs did not differ by diagnosis. On average, new visits took longer than established visits (p < 0.001). This difference was largely driven by new CM visits (44.3 ± 13.7 minutes), which were significantly longer than established CM visits (29.8 ± 9.2 minutes; p = 0.001).
    CONCLUSIONS: TDABC may reveal opportunities to maximize value by highlighting instances of variability and high cost in each module of care delivery. Physician leaders in pediatric neurosurgery may be able to use this information to allocate costs and streamline value care pathways.
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  • 文章类型: Journal Article
    背景:与脊髓栓系综合征相关的脊柱侧凸是最有挑战性的脊柱畸形之一。已经开发了多种手术方法,包括传统的分阶段和伴随程序,脊柱缩短截骨术,和单独的脊柱切除术。
    方法:一名10岁女性,患有先天性脊柱侧后凸,曲线进展恶化,束缚脊髓,还有遗尿症的病史.脊柱侧凸已发展为26°冠状曲线和55°胸腰椎后凸。术前脊柱磁共振成像显示L3-4水平之间的系绳和L1处的大的后凸畸形。病人接受了椎板切除术,术中运动信号丢失。在L4椎板切除术之前,在L1进行了计划的半椎体切除术。解开丝状终端的束缚,从T11到L2的后路脊柱融合术。手术后,患者经历了短暂的下肢无力,在接下来的2个月中,她的神经功能从基线改善。最终,这项手术的目的是阻止运动功能的进行性下降,这是成功实现的。
    结论:关于这种复杂疾病的治疗还有很多需要学习的地方,尤其是在合并脊柱侧弯的情况下。这种情况可以举例说明在尝试管理这种临床综合征时存在的复杂治疗范例,还有更多的知识需要学习。
    BACKGROUND: Scoliosis associated with tethered cord syndrome is one of the most challenging spinal deformities to manage. Multiple surgical approaches have been developed, including traditional staged and concomitant procedures, spine-shortening osteotomies, and individual vertebral column resections.
    METHODS: A 10-year-old female presented with congenital kyphoscoliosis with worsening curve progression, tethered spinal cord, and a history of enuresis. The scoliosis had progressed to a 26° coronal curve and 55° thoracolumbar kyphosis. Preoperative magnetic resonance imaging of the spine revealed a tethered cord between the levels of L3-4 and a large kyphotic deformity at L1. The patient underwent laminectomy, during which intraoperative motor signals were lost. A planned hemivertebrectomy at L1 was performed prior to an L4 laminectomy, untethering of the filum terminale, and posterior spinal fusion from T11 to L2. After surgery, the patient experienced transient lower-extremity weakness, with her neurological function improving from baseline over the next 2 months. Ultimately, the goal of this surgery was to halt the progressive decline in motor function, which was successfully achieved.
    CONCLUSIONS: Much remains to be learned about the treatment of this complicated disease, especially in the setting of concomitant scoliosis. This case serves to exemplify the complex treatment paradigms that exist when attempting to manage this clinical syndrome and that more remains to be learned.
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  • 文章类型: Journal Article
    目的:本研究旨在通过计算延髓圆锥终点(N)以下椎体骨化中心的数量来确定延髓圆锥的位置,并评估其在筛查闭合性脊髓发育不良和脊髓栓系综合征中的作用。
    方法:本研究共纳入了900名正常胎儿和146名患有闭合性脊髓发育不良或脊髓栓系综合征的胎儿。沿脊柱纵向平面计算并比较N值。利用接收器工作特性曲线,并对N的截断值进行了分析。
    结果:在856例正常胎儿和146例异常胎儿中成功进行了N计数。在正常组,观察到N随胎龄增加。具体来说,在17-20周胎儿亚组中,131例中117例,N≥6。这一数字在21-24周增加到213人中的211人,在25-41周增加到512人中的512人,分别。在17-41周范围内,N≥7的病例占856例胎儿中的715例。在异常组中,在163个胎儿中,N小于7/152,显示两组之间的统计学差异。截断值为6.5,特异性和敏感性分别为93.3%和83.5%。
    结论:发现N的计数是评估延髓圆锥位置的一种直接有效的方法。
    This study aimed to ascertain the conus medullaris position by counting the number of ossification centers in the vertebral bodies below the conus medullaris endpoint (N) and assess its utility in screening for closed spinal dysraphism and tethered cord syndrome.
    A total of 900 normal fetuses and 146 fetuses with closed spinal dysraphism or tethered cord syndrome were included in this study. The N values were tallied and compared along the spinal longitudinal plane. The receiver operating characteristic curve was utilized, and the cut-off value of N was analyzed.
    The counting of N was successfully performed in 856 normal and 146 abnormal fetuses. In the normal group, an increase in N with gestational age was observed. Specifically, in the subgroup of 17-20 wk fetuses, N was ≥6 in 117 out of 131 cases. This figure increased to 211 out of 213 in 21-24 wk and 512 out of 512 in 25-41 wk, respectively. Cases with N ≥7 accounted for 715 out of 856 fetuses in the 17-41 wk range. In the abnormal group, N was less than 7 in 152 out of 163 fetuses, showing statistical differences between the two groups. With a cut-off value of 6.5, specificity and sensitivity reached 93.3% and 83.5%.
    The counting of N was found to be a straightforward and efficient method for evaluating the position of the conus medullaris.
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