小儿脊髓纵裂及其合并畸形的治疗

[目的]探讨小儿脊髓纵裂及其合并畸形的治疗方法。[方法]回顾性分析近10a获随访经手术治疗的脊髓纵裂患儿48例，男29例，女19例；年龄7d-14岁。均因腰背部异常、双下肢和（或）二便功能障碍做全脊柱x线、MRI、CT等检查。手术先切断终丝松解栓系，再打开硬膜直视下切除纤维束或骨软骨嵴，合并先天性脊柱侧弯者一期或二期半脊椎后路切除矫形。[结果]48例患儿均有脊髓低位，伴发脊髓栓系综合征34例，脊柱侧弯及后凸26例，脊髓脊膜膨出14例，脊髓空洞15例，椎管内脂肪瘤12例。依照脊髓纵裂Pang分型：Ⅰ型27例，Ⅱ型18例，混合型3例。位于颈段1例，胸段6例，胸腰段1例，腰段38例，骶段2例。术后改善28例，恢复正常3例，无效3例。后路半脊椎切除术矫治先天性脊柱侧弯效果满意。[结论]脊髓纵裂极易合并脊髓栓系及先天性脊柱侧弯，患儿应常规行脊柱x线、MRI、CT等检查，以提高脊髓纵裂、脊髓栓系及其他脊柱畸形的诊断率。早期切断终丝松解栓系，直视下切除纤维性、骨或软骨性间隔是治疗脊髓纵裂的必要手段。同时一期或二期后路半脊椎切除是早期治疗先天性脊柱侧弯的有效方法。     

脊髓纵裂的手术治疗

脊髓纵裂常与脊柱侧弯或其它先天性畸形同时存在 ,在矫正脊柱侧弯之前应认真排除此病 ,否则会在哈氏棍撑开矫正畸形后并发截瘫或神经系统损害。本病的病理表现是自椎体后缘中央向背侧形成一骨性或纤维性纵隔 ,使脊髓和硬膜管分成左右两部 ,然后再合成一体 ,形成一用钉子将脊髓固定在脊柱上的表现。因骨组织与神经组织的发育增长快慢不同 ,故随之而来的是神经症状逐渐加重 ,最后导致截瘫。脊髓纵裂是一种少见的先天性脊柱脊髓畸形。文献报道脊髓纵裂在先天性脊柱侧凸中约占 5%。新疆脊柱外科研究所在 113例先天性脊柱侧凸患者中可疑有脊髓纵…     

脊髓

无骨折脱位型颈脊髓损伤44例临床分析；大鼠脊髓损伤后醛缩酶A和M型乳酸脱氢酶的表达变化；椎管内原始神经外胚层肿瘤；半椎板入路椎管内肿瘤显微切除术；小儿脊髓纵裂畸形合并脊髓栓系综合征的诊治     

脊柱侧弯合并脊髓纵裂的诊治体会

脊髓纵裂为神经管闭合不全畸形之一 ,其发生率在先天性脊柱畸形中约占 4%～ 9% [1～ 2 ]。自 1995年 3月以来共收治脊柱侧弯 3 2 4例 ,合并脊髓纵裂 2 3例 ,占 7.1% ,现将我们的诊治体会报告如下。临床资料一、一般资料　本组 2 3例 ,男 6例 ,女 17例 ,年龄 6～ 18岁 ,平均 10 .6岁。纵裂部位 :T2 1例、T3～ 1 2 7例、L1～ 313例、L4 2例。合并双下肢不对称、长短不等、肌萎缩的 19例 ,合并感觉、肌力、反射改变 11例 ,合并足部畸形 18例 ,合并尿失禁、尿潴溜 3例。二、X线及 CT检查　 2 3例中均有椎体及附件畸形 (半椎体、楔形椎、蝴蝶…     

先天性脊柱侧凸患者中肋骨畸形和椎管内畸形的特点

目的 探讨先天性脊柱侧凸患者的各类脊椎畸形、肋骨畸形与椎管内畸形的特点及发生情况.方法 回顾性分析2010年1月至2011年3月手术治疗的先天性脊柱侧凸患者118例,男性52例,女性66例;年龄3～50岁,平均(14±7)岁.统计椎管内畸形、肋骨畸形的总体发生比例及各自的好发部位,运用x2检验分析不同类型脊椎畸形、肋骨畸形与椎管内畸形的伴发情况.结果 118例先天性脊柱侧凸患者合并椎管内畸形57例(48.3％),多种畸形常并发存在,以脊髓纵裂(32.2％)和脊髓空洞症(21.2％)最为多见.合并肋骨畸形69例(58.5％),以凹侧为多见(68.1％,47/69).椎管内畸形的发生率在混合型脊柱侧凸(58.3％,35/60)和多脊椎畸形(54.8％,51/93)的患者中较高(x2= 14.05和27.50,P＜0.01).伴有肋骨畸形的先天性脊柱侧凸患者中有椎管内畸形的42例(60.9％),无椎管内畸形的27例(39.1％),不伴有肋骨畸形的先天性脊柱侧凸中有椎管内畸形的15例(30.6％),两组相比椎管内畸形的发生率差异有统计学意义(x2= 10.5,P＜0.01).结论 先天性脊柱侧凸伴发的椎管内畸形以脊髓纵裂和脊髓空洞为多见;肋骨畸形多发生于混合型脊柱侧凸及多脊椎畸形患者,以凹侧多见.脊椎畸形的类型及是否伴有肋骨畸形对椎管内畸形的诊断有重要的提示意义.椎管内畸形与脊椎畸形的好发部位相似,但各种椎管内畸形、脊椎畸形间缺少显著的、特定的对应关系.     

先天性脊柱侧弯术前MRI检查的应用价值

[目的]探讨MRI在先天性脊柱侧弯术前检查的应用价值。[方法]分析49例先天性脊柱侧弯患者术前MRI影像特征。[结果]其中椎体发育不良25例，椎管发育不良9例，椎板及棘突发育不良11例，脊柱裂5例，椎管内骨嵴4例，脊髓纵裂7例，Chiari氏畸形伴脊髓空洞3例，脊髓栓系综合征7例，椎管内肿瘤3例，脊膜膨出4例。背部皮毛窦1例，[结论]MRI技术的应用，大大提高了对先天性脊柱侧弯诊断的直观性和准确性，对手术治疗有极大的指导作用。     

先天性脊柱畸形伴椎管内异常外科治疗策略的再认识

正先天性脊柱畸形患者中椎管内异常的发生率为15%~43%,主要包括脊髓纵裂、脊髓拴系以及脊髓空洞三类[1~3]。如何处理先天性脊柱畸形半椎管内异常是脊柱外科医生面临的棘手问题。目前,已公认有明显神经损害或神经损害进行性加重是外科手术干预椎管内异常的明确指征,但对于无神经症状或者神经症状稳定的患者,其外科治疗策略仍存在争论。根据多年来对先天性脊柱畸形伴椎管内异常患者的     

脊髓纵裂的手术治疗

脊髓纵裂是一种少见的先天性脊柱脊髓畸形。文献报告脊髓纵裂[1～3]在先天性脊柱侧凸病例中约占5%～9%。本组自1980～1992年,手术治疗先天性脊柱侧凸143例,其中合并脊髓纵裂者6例,占4.2%。本组发病率较低的原因,可能与最初开始治疗脊柱侧凸时对脊髓纵裂并发症的认识不足有很大关系。从1985年以后,由于重视了对可疑脊髓纵裂的患者做周密细致的临床检查,X线该片,脊髓造影和CT扫描,使脊髓纵裂并发症的发现率明显提高。1资料与方法 本组6例中3例缺乏下肢神经症状,在垂直悬吊牵引下也无任何神经症状出现,但在正位X线平片上,特别是Stagnara位片…     

一期后路手术治疗儿童先天性腰骶椎后凸合并脊髓畸形

[目的]探讨儿童先天性腰骶椎后凸合并脊髓畸形一期脊髓圆锥松解、后路顶椎切除椎弓根钉腰骨盆内固定治疗的可行性及临床疗效。[方法]手术步骤包括椎弓根钉固定腰椎、骶椎或髂骨翼,松解圆锥,后路切除顶椎,加压矫正后凸并自体骨加异体骨植骨融合。[结果]5例患儿后凸及神经症状逐渐加重。全部病例合并脊髓纵裂脊髓空洞脊髓低位栓系,固定1～3个腰椎节段。术前后凸Cobb角52°～91°,平均76°,术后Cobb角后凸15°～30°,平均22°,校正率63%～80%,平均71%。手术时间225～390分,平均300min,出血300～600ml,平均420ml。随访时间24～52个月,平均32个月。1例术后短暂神经症状加重,随访时5例神经症状均较术前好转,3例恢复明显。[结论]一期脊髓圆锥松解后路顶椎切除椎弓根钉腰骨盆内固定治疗先天性腰骶椎后凸合并脊髓畸形短期随访疗效好,手术可导致神经症状短暂加重。治疗越早效果越好,并发症越少。     

脊髓纵裂30例诊治分析

目的探讨脊髓纵裂的临床诊治。方法回顾性研究1995—2002年间我院收治的脊髓纵裂病例，分析其临床诊治过程。结果脊髓纵裂30例，女性22例(73．3％)，男性8例(26．7％)，临床表现主要有：脊柱畸形30例(100%)，背部皮肤病变13例(43．3％)，下肢神经缺陷12例(40．0％)。X线片发现椎体异常27例(90%)，脊髓造影、CT、CTM、MRI检查异常表现的比例分别为77．8％(14／18)、88．9％(8／9)、100％(16／16)、91．7％(11／12)。纵裂位于胸段15例，腰段4例，颈段及胸段l例，胸段及腰段9例，颈段、胸段及腰段1例；骨性纵裂6例。纤维性纵裂21例，混合性纵裂3例。治疗方法包括单纯骨嵴切除椎管减压1例(3．3％)。脊柱畸形矫形手术同时切除骨嵴4例(13．3％)，单纯行脊柱畸形矫形手术而未处理脊髓纵裂23例(76．7％)，未行任何手术2例。有神经缺陷者中有4例在术后获得改善。结论脊髓纵裂临床少见，以女性患者较多，往往以脊侧凸为首发表现，患者多合并有背部皮肤异常及下肢神经缺陷，CTM扫描常能明确诊断。纵裂多位于胸段及腰段，在脊柱畸形矫形手术时要注意对脊髓纵裂的影响。     

Evaluating congenital spine deformities for intraspinal anomalies with magnetic resonance imaging.

SUMMARY: The incidence of intraspinal abnormalities associated with congenital spinal anomalies as detected by magnetic resonance imaging (MRI) is becoming better defined. In this study, 41 nonrandomized children with congenital spinal deformities (excluding myelomeningocele) who underwent complete MR evaluation were reviewed. Of the 41 congenital spinal deformities, 37 demonstrated congenital scoliosis, with failure of formation in 19, failure of segmentation in 4, and mixed defects in 14. The remaining four deformities were cases of congenital kyphosis. Thirteen patients with congenital spine anomalies were noted to have intraspinal abnormalities identified by MRI: tethered cord in 12 patients, syringomyelia in 3 patients, and diastematomyelia in 5 patients. Of the 12 patients with tethered cord, 2 patients had neurologic deficits. Urorectal anomaly was one of the most common associated findings (15%). Considering an incidence of intraspinal anomalies of 31% and as clinical manifestations may not be initially detectable, MRI is recommended in patients with congenital spinal deformity as part of the initial evaluation even in the absence of clinical findings.     

Occult intraspinal anomalies in congenital scoliosis

Thirty consecutive patients with congenital spinal deformity underwent magnetic resonance imaging (MRI) to determine the incidence of occult intraspinal anomaly. These congenital spinal deformities included 29 cases of congenital scoliosis and one case of congenital kyphosis. Physical examination findings and plain radiographs were reviewed in an attempt to correlate these findings with subsequent intraspinal pathology. Nine patients had intraspinal anomalies identified on MRI consisting of five with tethered cord, four with syringomyelia, three with lipoma, and one with diastematomyelia. One patient required surgery for diastematomyelia; another underwent release of his tethered cord. Only one patient, with diastematomyelia associated with a syrinx and bifocal tethering, had his anomaly suggested by physical examination and plain radiographs. Two other patients had findings on plain radiographs previously associated with high prevalence of occult intraspinal anomalies; one patient with congenital kyphosis had a tethered cord, and one patient with a unilateral hemivertebrae associated with a contralateral bar had a tethered cord. Two of nine patients with occult intraspinal anomalies required surgery for their anomaly. In patients with a congenital spinal deformity, we found nine (30%) of 30 to have an associated anomaly within the spinal canal. Only three of these nine had plain radiographs and physical examination findings suggestive of their subsequent MRI findings. Given the poor correlation between findings on physical examination, plain radiographs, and subsequent occult intraspinal anomalies on MRI, we believe that MRI is helpful in evaluating patients with congenital spinal anomalies.     

Segmental vessel ligation in patients undergoing surgery for anterior spinal deformity

Segmental vessel ligation during anterior spinal surgery has been associated with paraplegia. However, the incidence and risk factors for this devastating complication are debated. We reviewed 346 consecutive paediatric and adolescent patients ranging in age from three to 18 years who underwent surgery for anterior spinal deformity through a thoracic or thoracoabdominal approach, during which 2651 segmental vessels were ligated. There were 173 patients with idiopathic scoliosis, 80 with congenital scoliosis or kyphosis, 43 with neuromuscular and 31 with syndromic scoliosis, 12 with a scoliosis associated with intraspinal abnormalities, and seven with a kyphosis. There was only one neurological complication, which occurred in a patient with a 127 degrees congenital thoracic scoliosis due to a unilateral unsegmented bar with contralateral hemivertebrae at the same level associated with a thoracic diastematomyelia and tethered cord. This patient was operated upon early in the series, when intra-operative spinal cord monitoring was not available. Intra-operative spinal cord monitoring with the use of somatosensory evoked potentials alone or with motor evoked potentials was performed in 331 patients. This showed no evidence of signal change after ligation of the segmental vessels. In our experience, unilateral segmental vessel ligation carries no risk of neurological damage to the spinal cord unless performed in patients with complex congenital spinal deformities occurring primarily in the thoracic spine and associated with intraspinal anomalies at the same level, where the vascular supply to the cord may be abnormal.     

先天性脊柱侧凸患者中的脊髓畸形和脊椎畸形

目的:探讨先天性脊柱侧凸患者的各类脊髓畸形与脊椎畸形之间的关系。方法:对我院2003年～2005年收治的211例已确诊的先天性脊柱侧凸患者的临床资料进行回顾性分析,统计先天性脊柱侧凸患者脊髓畸形、脊椎畸形的发生率及其好发部位,观察两者间的关系。结果:211例先天性脊柱侧凸患者中脊髓畸形44例(20.85%),其中脊髓空洞症25例次(11.85%),脊髓纵裂30例次(14.22%),马尾终丝栓系28例次(13.27%),低位圆锥12例次(5.69%),其他类型少见,多种畸形常并发存在;脊髓畸形好发于脊柱胸段、胸腰段、腰段、腰骶段。44例伴有脊髓畸形的先天性脊柱侧凸病例中不良分节34例(77.27%),孤立性半椎体1例(2.27%),非孤立性半椎体21例(47.73%),脊柱裂30例(68.18%),混合型41例(93.18%);脊椎畸形好发于脊柱胸段、腰段、腰骶段。结论:先天性脊椎畸形常合并脊髓畸形,总体上脊髓畸形与脊椎畸形的好发部位相似,但各种脊髓畸形、脊椎畸形间缺少显著的、特定的对应关系。     

脊椎裂后遗足踝畸形的临床分析

目的 分析脊椎裂后遗足踝畸形的发病情况、临床特点、畸形类别与矫形治疗状况.方法 对我科1990年1月至2009年7月接受手术治疗脊椎裂后遗足踝部畸形患者进行回顾性研究,对资料进行总结分析.结果 手术治疗脊椎裂后遗足踝部畸形共107例患者.其中男性44例,女性63例;手术年龄1.3～52.0岁,平均17.7岁,其中18岁以上者50.5%(54/107).隐性脊椎裂50.5%(54/107),显性(囊性)脊椎裂49.5%(53/107).1例为胸椎裂(T3～8),余106例为腰骶椎裂.单侧足受累49例(左侧22例,右侧27例),双侧足受累58例,共165足.畸形类型:内翻足76足,外翻足23足,夏科连枷足15足,其他类足畸形51足.合并其他受累部位:膝部屈曲或膝反屈畸形4例,髋部畸形17例(包括髋内收、屈曲或髋关节脱位,骨盆倾斜、双下肢不等长等),合并大小便功能障碍30例.本组54例隐性脊椎裂致足踝畸形患者中,29例既往在多个医院未能确诊发病原因,误诊或漏诊的比率高达53.7%(29/54).107例患者中,既往接受过足踝畸形矫形手术者仅26例.18岁以上既往未能实施合理矫形外科治疗遗留严重足踝畸形者50.5%(54/107).结论 脊椎裂继发畸形主要在足踝部.由于缺乏对此病的整体认识与矫形治疗经验,延误了对此类疾病的早期诊断、早期干预的治疗时机,遗留了很多未经矫形外科治疗的严重足踝畸形.     

椎管内硬膜下肿瘤的手术治疗

[目的]探讨颈椎管内硬膜下肿瘤的手术治疗方法及疗效。[方法]回顾性分析经手术治疗的21例颈椎管内硬膜下肿瘤患者的I临床资料及治疗效果，所有病人均行颈椎后路椎板切除椎管减压进行肿瘤切除，部分病人并行后路钉棒系统内固定。[结果]15例完全切除，4例次全切除，2例部分切除；症状消失13例，症状减轻7例，症状恶化1例，无死亡病例发生。[结论]术前根据影像学资料仔细评价肿瘤大小及肿瘤与脊髓的关系是决定手术成功的关键，术中仔细操作避免损伤脊髓，并注意保护好椎动脉及防止椎管内静脉丛大出血，是将肿瘤完全切除的前提。     

脊髓神经源性足部畸形的分类和治疗

[目的]研究脊髓神经源性足部畸形的发病机理、分类和手术治疗方案.[方法]1988年10月～2006年6月,回顾性分析脊髓病变、脊髓和脊神经因被牵拉或压迫引起的足部畸形167例258足,根据脊髓损伤的性质和发病机理,将足部畸形分为上运动神经元损伤型和下运动神经元损伤型两大类,两类足部畸形采用不同的治疗方案.上运动神经元损伤型足部畸形,手术方案以选择性脊神经后根切断术或周围神经缩窄术为主;下运动神经元损伤型足部畸形,手术方案以软组织松解、肌腱转位术和截骨术为主,其中僵硬性足部畸形使用Ilizarov外固定器缓慢矫正.[结果]得到至少5年随访的147例228足进行总结分析,上运动神经元损伤型足部畸形42足,下运动神经元损伤型足部畸形186足.采用Laaveg-Ponseti足功能评分系统:优94足,良84足,可32足,差18足;优良率78.1％.第1次术后复发36足,复发率15.8％.第2次术后复发8足.[结论]根据脊髓神经源性足部畸形的分类,采用不同的手术治疗方案,可提高治疗效果,减少术后畸形复发.     

Posterior cord syndrome: Demographics and rehabilitation outcomes

Context/Objectives: To describe demographics, clinical characteristics, and functional outcomes of patients with incomplete spinal cord injuries and posterior cord syndrome (PCS).Design: Five-year retrospective case series.Setting: Spinal cord injury (SCI) rehabilitation unit at a Level 1 tertiary university medical center.Participants: 9 patients with incomplete cord injuries diagnosed with PCS admitted to rehabilitation within the past 5 years.Outcome measures: Functional Independence Measure (FIM) motor scores, length of stay (LOS), discharge disposition.Results: Incidence of PCS was 2% with an average age of 62.0 years. The most common etiology for PCS was spinal cord compression from localized tumors (78%). Seven (78%) patients had paraparesis. All patients had an American Spinal Injury Association impairment scale (AIS) classification of AIS D. SCI-related complications most commonly included: neuropathic pain (78%), spasticity (44%), and neurogenic bladder (78%). Average LOS on the rehabilitation unit was 28 days. Average admission and discharge FIM motor scores were significantly improved (P = 0.001) from 41 to 65, respectively. Two-thirds (67%) of patients were able to walk at least 150 feet with a rolling walker prior to discharge. Most (78%) patients were discharged to home. Continence improved from admission to discharge from 22% vs 56% (bladder) and 67% vs 78% (bowel).Conclusions: We can conclude that PCS most often results in paraparesis due to tumor compression. Typical SCI-related medical complications are encountered. These patients often experience significant functional improvements during SCI rehabilitation with the majority also having bladder and bowel continence allowing them to return home at discharge.     

外固定器治疗脊髓栓系综合征僵硬性足部畸形

[目的]观察外固定器治疗脊髓栓系综合征僵硬性足部畸形的临床效果,探讨脊髓神经源性足部畸形外科矫正和功能重建的新技术.[方法] 1988年10月～2006年6月,回顾性分析脊髓栓系综合征僵硬性足部畸形61例97足;年龄6～42岁,根据足部畸形的特征,设计不同构型的外固定器.上运动神经元损伤型足部畸形主要实施选择性胫神经缩窄手术和距下关节外融合手术或距下关节融合术,同时安装外固定器.下运动神经元损伤型足部畸形主要实施软组织松解、肌腱转位术或截骨手术,同时安装外固定器.[结果]得到至少3年随访的54例、86足进行分析,马蹄内翻足39足,马蹄足21足,马蹄外翻足16足,跟行足6足,高弓足4足.其中上运动神经元损伤型足部畸形19足,下运动神经元损伤型67足.佩戴外固定器时间8～12周,平均11周.采用Laaveg - Ponseti足功能评分系统:优38足,良29足,可13足,差6足.优良率77.9％.第1次术后复发3足,复发率3.5％.钉道感染18足,清理炎性分泌物或更换钉道位置后治愈.足底溃疡3足,清创和短期避免负重后治愈.[结论]外固定器治疗脊髓栓系综合征僵硬性足部畸形符合生物学重建理论,可提高疗效,减少复发.     

急性脊髓损伤外科干预时机对神经功能恢复影响的系统评价

目的 :系统评价不同手术时机对急性脊髓损伤患者神经功能恢复的影响。方法 :计算机检索Pub Med、EMBASE、Cochrane Library、ISI Web of knowledge、中国生物医学文献数据库、中文科技期刊全文数据库(VIP)、中国期刊全文数据库(CNKI)及万方数据库中不同手术时机干预治疗急性脊髓损伤的随机对照试验(RCT),检索时间均从建库至2017年2月。结果:纳入4个RCT,共156例急性脊髓损伤的患者。在不完全性脊髓损伤患者当中早期手术可以改善患者ASIA运动功能评分[MD=3.29,95%CI(-7.90,14.49),P=0.56],Frankel评分总体改善率[OR=7.65,95%CI(2.69,21.74),P=0.000 1];在完全性脊髓损伤患者当中早期手术与晚期手术对患者Frankel评分总体改善率影响不明显[OR=4.88,95%CI(0.74,32.09),P=0.10];早期手术与晚期手术对住院时间[MD=-3.4,95%CI(-8.12,1.32),P=0.16],死亡率[OR=1.07,95%CI(0.21,5.56),P=0.93],褥疮发生率[OR=1.07,95%CI(0.17,6.69),P=0.94]差异无统计学意义。结论 :早期手术在一定程度上能促进患者脊髓功能恢复,也并没有带来更多的并发症,未来仍需要进行高质量的随机对照研究来验证这个结论。