第四脑室输出道疏通术治疗Chiari畸形并脊髓空洞

目的探讨显微外科治疗Chiari畸形并脊髓空洞的手术方法。方法Chiari畸形Ⅰ型并脊髓空洞经MRI确诊。小范围后颅窝减压 C1后弓切除,纵形切开硬膜,显微镜下分离小脑扁桃体与脑干的粘连,切除小脑扁桃体下疝,探察脊髓中央管开口,发现有隔膜或活瓣形成并切开,探察第四脑室侧孔及中脑导水管,使第四脑室脑脊液循环通畅。结果第四脑室输出道疏通术共计97例,术中显微镜下发现脊髓中央管开口有隔膜或活瓣形成,手术切除脊髓中央管口隔膜或活瓣,并探察第四脑室正中孔及两侧外侧孔,恢复第四脑室脑脊液循环,原位缝合硬膜。术后随访48例,平均1.3年,MRI检查示所有患者下疝的小脑扁桃体均消失,并存脊髓空洞明显缩小和消失。结论第四脑室输出道疏通术能够改善Chiari畸形Ⅰ型并脊髓空洞患者的术后临床症状。     

小脑扁桃体切除并脊髓中央管口松解术治疗合并脊髓空洞的Chiari畸形

目的探索外科治疗合并脊髓空洞的Chiari畸形的新方法。方法采用切除下疝小脑扁桃体,并脊髓中央管口假膜切开术,原位硬膜缝合法。结果18例手术治疗,15岁以下儿童12例,术中发现均有脊髓中央管口(闩部)假膜;成人6例,3例有假膜形成。全部病例术后脊髓空洞消失或明显缩小。结论脊髓中央管口假膜是造成Chiari畸形脊髓空洞的原因之一,切除下疝小脑扁桃体并脊髓中央管口假膜切开术,是手术治疗合并脊髓空洞的Chiari畸形最根本的方法。     

Chiari畸形并脊髓空洞的治疗与小脑扁桃体下疝分型

目的探讨Chiari畸形并脊髓空洞手术中,下疝的小脑扁桃体下舌分型与切除的标准。方法术中显微镜下观察32例下疝的小脑扁桃体压迫延髓的部位及其关系,结合MRI,提出下疝的小脑扁桃体分型,并根据分型切除小脑扁桃体下舌,切开脊髓中央管开口隔膜或活瓣,恢复第四脑室内脑脊液循环通畅。结果小脑扁桃体分型：①根据压迫部位分为：后方型27例,侧方型4例,侧前方型1例;②根据小脑扁桃体下疝程度分为：单侧压迫型11例,双侧压迫型21例。术后随访28例,MRI检查示下疝的小脑扁桃体均消失,第四脑室内脑脊液循环通畅,脊髓空洞明显缩小和消失;术后病人临床症状大多改善。结论下疝的小脑扁桃体根据其分型并切除,可为手术治疗Chiari畸形并脊髓空洞提供标准及依据。     

小范围后颅窝减压治疗Chiari畸形合并脊髓空洞症（附97例报告）

目的评价以更小的创伤治疗Chiari畸形合并脊髓空洞症的临床疗效。方法小范围后颅窝骨性减压窗范围一般在3cm×3cm以内,不切除C1后弓,切除下疝的小脑扁桃体,松解脊髓中央管开口隔膜,疏通第四脑室脑脊液各输出道,使脑脊液循环通畅。结果37例术后12d内MRI示下疝的小脑扁桃体下缘上升到枕骨大孔水平以上。长期随访48例,29例患者症状逐渐改善;14例症状稳定未加重;5例较术前加重,但MRI示脊髓空洞缩小。结论小范围后颅窝减压术能够改善Chiari畸形合并脊髓空洞患者的临床症状,可作为外科治疗Chiari畸形合并脊髓空洞症的一种术式。     

小范围颅后窝减压治疗Chiari畸形并脊髓空洞

目的 评价小范围颅后窝减压术治疗Cbiari畸形并脊髓空洞的效果。方法回顾性分析97例Chiari畸形并脊髓空洞的手术经验。均行小范围颅后窝减压,骨窗约3cm×3cm,尽可能不切除C。后弓。直线切开硬脑膜并原位缝合,切除下疝小脑扁桃体,松解脊髓中央管开口并切开膈膜,疏通第四脑室脑脊液各输出道,使脑脊液循环通畅。结果术后完整随访45例,MRI复查示小脑扁桃体下缘均位于枕骨大孔水平线以上,脊髓空洞明显缩小或消失。结论小范围颅后窝减压术能改善Chiari畸形并脊髓空洞的术后症状。     

小脑扁桃体下疝切除术治疗Chiari畸形并脊髓空洞

目的评价小脑扁桃体下疝切除术治疗Chiari畸形并脊髓空洞的临床效果。方法本组Chiari畸形合并脊髓空洞患者10例均采取手术治疗。手术时有效范围后颅窝骨性减压,铣刀铣开小范围后颅窝骨瓣(3cm×3cm),不切除C1后弓,直线侧切开硬脑膜,切除下疝的小脑扁桃体,松解脊髓中央管开口隔膜,疏通第四脑室脑脊液各输出道,使脑脊液循环通畅,原位缝合硬脑膜,颅骨锁固定骨瓣。结果手术无死亡及病残病例。10例术后(12d内)MR示小脑扁桃体下缘上升到枕骨大孔水平以上,后颅窝内容积扩大。术后长期随访9例,随访时间6个月～2年,2例患者症状逐渐改善,6例稳定未加重,1例较术前略加重,但MR示后颅窝内容积扩大,脊髓空洞缩小。结论小脑扁桃体下疝切除术治疗Chiari畸形并脊髓空洞患者,术后后颅窝内容积扩大,第四脑室内脑脊液循环通畅,临床症状多有改善。     

Chiari Ⅰ型畸形合并脊髓空洞症的显微外科治疗

目的 研究ChiariⅠ型畸形合并脊髓空洞症的显微手术治疗方法。方法 枕下正中入路，咬开枕骨大孔后缘及寰椎后弓，显微镜下切开硬脑脊膜，软脑膜下切除下疝的小脑扁桃体，重建第四脑室正中孔与两侧小脑延髓外侧池交通、开放脊髓中央管口，修补硬脑脊膜。结果 36例手术均顺利完成，术后恢复平稳、无感染，无死亡。病人肌力。温、痛感觉及肌张力均恢复良好。下疝的小脑扁桃体均消失。随访MRI示，6例空洞症消失，脊髓接近正常；30例脊髓空洞腔明显缩小。结论 采用显微手术切除下疝的小脑扁桃体，开放脊髓中央管口，恢复枕大池结构和功能，是治疗ChiariⅠ型畸形合并脊髓空洞症的有效方法。     

Chiari畸形合并脊髓空洞的外科治疗

目的 探查外科治疗chiari畸形合并脊髓空洞症的方法及效果.方法 对86例chiari畸形合并脊髓空洞,行后颅窝减压术21例,后颅窝减压脊髓空洞切开引流18例,后颅窝减压并小脑扁桃体切除47例,其中切除下疝小脑扁桃体,并脊髓中央管口假膜切开3例.结果 所有病例临床症状都有不同程度的缓解,72例病例术后脊髓空洞消失或明显缩小.结论 手术使延颈髓充分减压,可有效缓解症状,而切除下疝小脑扁桃体,并充分松解四脑室后正中出口处,更能有效缓解临床症状,减轻或消灭脊髓空洞.     

两种不同手术方法治疗合并脊髓空洞的ChiariⅠ畸形的效果比较

目的探讨合并脊髓空洞的ChiariⅠ畸形的手术治疗方法。方法 2007年1月至2009年12月,收治的ChiariⅠ畸形合并脊髓空洞的19例患者作为观察组,在显微镜下进行小脑扁桃体下疝切除+脊髓中央管口松解+枕下减压扩大硬脑膜修补术治疗。同时搜集2002年1月至2006年12月收治的ChiariⅠ畸形合并脊髓空洞的21例患者作为对照组,单纯采用枕下减压扩大硬脑膜修补术治疗,分析两组的临床效果。结果观察组症状缓解优良率为100.0%(19/19),对照组为52.4%(11/21),两组相较,差异显著(P<0.01)。结论应用显微手术进行小脑扁桃体下疝切除+脊髓中央管口松解+枕下减压扩大硬脑膜修补术治疗ChiariⅠ畸形合并脊髓空洞临床效果好,值得推广。     

枕大池重建术治疗ChiariI畸形合并脊髓空洞

目的 探讨枕大池重建术治疗合并脊髓空洞的Chiari I 畸形的临床意义.方法 对48例合并脊髓空洞的Chiari I 畸形病人行枕下正中入路,咬开下项线至枕大孔后缘及寰椎后弓,"Y"形切开硬脑脊膜和蛛网膜.对下疝小脑扁桃体显微镜下弱电流电凝或软脑膜下切除,开放正中孔直至第四脑室底,打通两侧小脑延髓外侧池,切开脊髓中央管口假膜.扩大修补硬脑脊膜及蛛网膜,重建枕大池.结果 术中发现28例有脊髓中央管口假膜.随访3～24月, 感觉及肌力均有不同程度的恢复, MRI复查8例脊髓空洞消失,40例明显缩小.结论 后颅凹减压、开放四脑室正中孔与两侧小脑延髓外侧池相通、切开脊髓中央管口假膜,是手术治疗合并脊髓空洞的Chiari I畸形的关键.     

枕颈减压、脊髓空洞穿刺抽液治疗Chiari畸形合并脊髓空洞症

目的：探讨一种治疗Chiari畸形合并脊髓空洞症的新手术方法。方法：61例经MRI诊断为Chiari畸形合并脊髓空洞症患,均采用枕颈减压加脊髓空洞空刺抽液术治疗。结果：全部病例术后临床症状,体征均有不同程度改善;随访48例（术后1－5年）,明显转好45例,好转3例,其中8例术后2－4年经MRI复查,脊髓空洞消失2例,空洞明显缩小5例,轻微缩小1例,手术无并发症,无死亡,结论：枕颈减压加脊髓空洞空刺抽液术治疗Chiari畸形合并脊髓空洞症是一种有效的方法。     

Chiari畸形伴脊髓空洞症的微创手术治疗

目的探讨Chiari畸形并脊髓空洞症的微创外科手术治疗方法。方法回顾性分析74例患者微创手术治疗情况。微创手术治疗74例,均行后颅窝小骨窗减压,下疝小脑扁桃体切除,松解蛛网膜下腔的粘连,开放正中孔至第四脑室及小脑延髓侧池,改善蛛网膜下腔脑脊液循环,原位缝合硬脑膜。结果术后临床症状消失和改善者68例,无变化6例。随访56例,脊髓空洞明显缩小。结论微创手术是治疗Chiari畸形并脊髓空洞症的有效方法。恢复蛛网膜下腔的脑脊液循环是治疗的关键。     

ChiariⅠ型畸形的MRI表现及其手术治疗

目的 探讨Chiari Ⅰ型畸形的MRI分型及治疗方法。方法 23例Chiari Ⅰ型畸形患E,21例有颈神经根症状,16例表现为中央管周围损害症状,另有8例分别存在小脑及颅神经损害症状。MR检查显示,单纯小脑扁桃体下疝(B型)7例(30.43％),小脑扁桃体下疝合并脊髓空洞症(A型)16例(69.57％)。根据MRI所显示病变部位,采用不同的手术方式,其中11例行后颅窝减压术,7例行后颅窝减压井小脑扁桃体切除术,5例行后颅窝减压并脊髓空洞切开引流术。结果 手术后18例患者(78.26％)症状改善,5例(21.74％)与手术前比较无明显变化。对16例伴脊髓空洞症患者中的13例进行随访,其中3例空洞消失,7例空洞显著缩小,3例空洞较手术前缩小50％。结论 手术治疗可使延髓、颈髓充分减压,有效缓解临床症状,并对脊髓空洞症具有良好的治疗效果。     

Pathogenetic role of myelitis for syringomyelia

BACKGROUND: CSF-flow obstruction is regarded as a mandatory factor for the development of syringomyelia. However, there are conditions in which syringomyelia is not associated with evident persistent CSF-flow obstruction, as in the case of inflammatory spinal cord lesions. In these instances we hypothesize that the accumulation of vasogenic edema may play a role in the development of the syrinx. Recently proposed theories underline, even in the event of CSF-flow obstructions, a major role for the accumulation and final coalescence of interstitial spinal fluid, rather than CSF penetration through the spinal cord. AIM: To clarify the relationship between syrinx development and spinal cord inflammation, through the analysis of the role of intrinsic medullary factors versus CSF-flow block. METHODS: A prospective case series including patients with transient syringomyelia associated with different examples of non-infectious myelitis: sarcoidosis, post-infectious transverse myelitis, Devic's disease and multiple sclerosis. Cavitations resulting from cystic myelomalacia were excluded. CSF-flow block was assessed by structural MRI. RESULTS: Syringes associated with myelitis shared some common features: they developed during the acute phase of myelitis and disappeared after steroids, were all non-communicating cavitations involving the central canal, and occurred in the same spinal segment affected by myelitis. CSF-flow obstruction was detected in one patient (Chiari I malformation), while in the other three patients we could not detect anatomical predispositions. CONCLUSION: Only one patient had structural abnormalities, though without evidence of a pathogenetic role in itself: however, CSF space obstruction and reduced CSF compliance could have accelerated the development of syringomyelia triggered by intramedullary inflammation. The clinical and radiological features in this patient are consistent with the label "presyringomyelia". The absence of any anatomical predisposition in the other patients suggests a major pathophysiological role for intrinsic medullary mechanisms, including blood-spinal cord barrier breakdown, impairment of extracellular fluid drainage, and leakage of subarachnoidal CSF into the nervous tissue.     

Cervico-thoracic neurenteric cyst: clinicoradiological correlation with embryogenesis

Two cases of cervico-dorsal neurenteric cysts are presented, one located dorsal and the other ventral to the spinal cord, with associated Klippel-Feil anomaly, meningocele, spinal dysraphism and possibly spinal cord malformation. One patient was operated on as a neonate for a cervico-thoracic meningocele and presented during adolescence with spastic quadriparesis. Imaging revealed spina bifida, a dorsally located neurenteric cyst and a tendency towards split cord malformation. The other child presented with cervicothoracic kyphoscoliosis and paraparesis. Imaging demonstrated persistence of a cervico-thoracic remnant of the primitive neurenteric canal through the upper thoracic spine.     

Intraoperative measurement of spinal cord blood flow in syringomyelia

The role of spinal cord ischemia in the pathophysiology of syringomyelia remains undetermined. Previous reports in the literature suggest that shunting of syringes can improve spinal cord blood flow. In order to determine the effects of syrinx decompression on spinal cord blood flow in patients with syringomyelia, we prospectively measured regional spinal cord blood flow (RSCBF) intraoperatively pre and post shunting in patients with symptomatic syringomyelia using laser doppler flowmetry. Six patients with MRI documented syringomyelia were studied (three with Arnold Chiari I malformation and associated syrinx and three with post-traumatic syringomyelia). Surgery was performed on all patients with either a syringopleural or syringoperitoneal shunt. Laser doppler blood flow and somatosensory evoked potentials were monitored prior to myelotomy and after shunt insertion. Results indicate that there was a significant increase in RSCBF after decompression of the syrinx. This study supports the hypothesis that spinal cord ischemia is important in the pathophysiology of syringomyelia and confirms previous reports in the literature regarding RSCBF in syringomyelia.     

Hypothesis on the pathophysiology of syringomyelia based on simulation of cerebrospinal fluid dynamics

OBJECTIVES: Despite many hypotheses, the pathophysiology of syringomyelia is still not well understood. In this report, the authors propose a hypothesis based on analysis of cerebrospinal fluid dynamics in the spine. METHODS: An electric circuit model of the CSF dynamics of the spine was constructed based on a technique of computational fluid mechanics. With this model, the authors calculated how a pulsatile CSF wave coming from the cranial side is propagated along the spinal cord. RESULTS: Reducing the temporary fluid storage capacity of the cisterna magna dramatically increased the pressure wave propagated along the central canal. The peak of this pressure wave resided in the mid-portion of the spinal cord. CONCLUSIONS: The following hypotheses are proposed. The cisterna magna functions as a shock absorber against the pulsatile CSF waves coming from the cranial side. The loss of shock absorbing capacity of the cisterna magna and subsequent increase of central canal wall pressure leads to syrinx formation in patients with Chiari I malformation.