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。     

脊髓中央管隔膜形成与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 127例Chiari畸形并脊髓空洞经MRI确诊,采用小脑扁桃体下疝切除,并脊髓中央管开口隔膜切开术. 结果术中发现脊髓中央管开口隔膜形成;术后随访89例,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.结论小脑扁桃体下疝,脊髓中央管开口隔膜形成是造成Chiari畸形并脊髓空洞的原因之一;小脑扁桃体下疝切除,脊髓中央管隔膜切开术是手术治疗Chiari畸形并脊髓空洞症的有效方法.     

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。     

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。     

Chiari畸形Ⅰ型合并睡眠呼吸暂停综合征的手术治疗分析

目的 探讨手术干预Chiari畸形对Chiari畸形Ⅰ型合并有睡眠呼吸呼吸暂停综合征（CSAS）病人睡眠的影响。方法 回顾性分析2012年7月至2016年5月收治的6例Chiari畸形Ⅰ型合并CSAS的临床资料,均行后正中入路软膜下下疝小脑扁桃体切除及枕大池重建术。术前、术后3个月行多导睡眠呼吸监测记录进入深睡眠至少4 h以上的呼吸暂停次数、每次暂停时间及每次暂停时的最低血氧饱和度。结果 术后3个月,呼吸暂停次数明显减少,暂停持续时间下降,血氧饱和度上升,临床表现明显改善。术后复查MRI示下疝的扁桃体切除,延颈髓背侧压迫解除,脑脊液循环通畅,部分合并脊髓空洞较术前缩小,甚至有部分脊髓空洞完全消失。结论 Chiari畸形Ⅰ型合并睡眠呼吸暂停综合征,通过颈后正中入路软膜下下疝小脑扁桃体切除及枕大池重建术,能够明显改善病人睡眠呼吸暂停情况。     

以脑脊液动力学分析为依据微创减压治疗Chiari畸形Ⅰ型

目的探讨基于脑脊液动力学分析的微创软脑膜下减压在Chiari畸形Ⅰ型手术治疗的应用。方法回顾性分析46例Chiari畸形Ⅰ型病人的临床资料,均采用小骨窗颅后窝减压,软脑膜下切除下疝小脑扁桃体,硬脑膜原位缝合。结果术后症状消失或改善45例,无变化1例,无症状加重、死亡病例。42例合并脊髓空洞病人中,复查MRI显示空洞直径缩小40例,无明显变化2例。术后并发症:发热10例,头痛16例,皮下积液2例。术后随访6～48个月,症状消失或改善45例,病情稳定1例,无神经功能恶化病例,有效率97.8%;42例合并脊髓空洞病人中,MRI显示空洞直径缩小或消失41例,无明显变化1例。结论基于脑脊液动力学分析的微创软脑膜下减压可有效解除枕大孔区压迫,重建脑脊液流体动力学循环是治疗Chiari畸形Ⅰ型的有效方法。     

小脑扁桃体下疝的MR分型

目的 研究小脑扁桃体下疝MR分型的诊断价值.方法 对45例Chiari畸形小脑扁桃体下疝病人进行M7检查,除常规矢状位外,增加轴位及冠状位扫描,小脑扁桃下疝深度6-13.6 mm,平均10.1 mm.结合MR轴位及冠状位,对小脑扁桃体下疝进行分型.结果 ①根据双侧小脑扁桃体下疝程度:单侧压迫型20例,双侧压迫型25例.②根据压迫部位:后方型31例,侧方型11例,侧前方型3例.45例均实施小脑扁桃体下疝显微切除术,术后感觉均较术前明显改善,无症状加重及死亡病例.随访40例,平均随访时间1.5年;颅颈交界区MR复查示小脑扁桃体下缘均位于枕骨大空连线以上,合并脊髓空洞症者均出现空洞不同程度缩小和消失.结论 MR是小脑扁桃体下疝的首选检查方法,对小脑扁桃体下疝分型有独特价值并可用于指导手术.     

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。     

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。     

International survey on the management of Chiari I malformation and syringomyelia

Introduction The availability of magnetic resonance imaging (MRI) has resulted in an increasing number of asymptomatic, minimally symptomatic, and doubtfully symptomatic patients being diagnosed with a Chiari I malformation with or without syringomyelia. In an attempt to clarify how neurosurgeons manage these clinical problems, an international survey on the Chiari I malformation and related syringomyelia was undertaken.Method A questionnaire on the expected natural course of the disease and on aspects of the surgical technique for a number of hypothetical cases relating to Chiari I malformation with and without syringomyelia was used to survey Pediatric Neurosurgeons worldwide.Results Of 246 questionnaires distributed, 76 (30.8%) were completed and returned. There was a consensus that no operation should be carried out in asymptomatic patients with a Chiari I malformation, unless there is associated syringomyelia. There was a consensus that decompression of the Chiari malformation should be performed in patients with scoliosis when syringomyelia is present, and the majority decompressed the Chiari malformation in scoliotic patients even in the absence of syringomyelia. Suboccipital decompression was the standard surgical procedure for Chiari I malformations. The majority of respondents favored routine dural opening at surgery and closure with a pericranial or synthetic patch graft. In the case of a persistent or progressive syrinx after suboccipital decompression, the majority recommended shunting of the syrinx to the subarachnoid space or to the pleural cavity.Conclusion There continues to be much variation in the management of the Chiari I malformation.     

Chiari畸形并脊髓空洞症的MRI分型及其治疗

对Ｃｈｉａｒｉ畸形并脊髓空洞症治疗方法及其效果的探讨。方法对１０３例Ｃｈｉａｒｉ畸形病例根据其ＭＲＩ征像分为Ａ、Ｂ、Ｃ三种类型。结果依据其类型采用不同的手术方式,并获得了较为满意的治疗效果。结论手术使延颈髓充分减压,可有效缓解临床症状,并对脊髓空洞症有明显的治疗作用。     

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。     

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。     

Endoscopic third ventriculostomy in the management of Chiari I malformation and syringomyelia associated with hydrocephalus

BACKGROUND: Chiari malformation with syringomyelia is conventionally managed with foramen magnum decompression and duroplasty. In presence of hydrocephalus, a ventriculoperitoneal shunt insertion has been the initial preferred mode of treatment. METHODS: Two patients of Chiari I malformation with syringomyelia who had associated hydrocephalus were initially treated with endoscopic third ventriculostomy (ETV). RESULTS: At follow up, both the patients symptomatically improved. Repeat MRI studies revealed collapse of the syrinx cavity and reduction of the Chiari malformation. CONCLUSIONS: Chiari malformation and syrinx associated with hydrocephalus may be effectively managed with endoscopic third ventriculostomy.     

与Chiari畸形或脊髓损伤无关的脊髓空洞症的外科治疗（英文）

PURPOSE Syringomyelia is a misleading disease since the problem always lies elsewhere.Arachnoiditis,because it is radiographically difficult to discern,is an especially insidious cause.To better guide selection from among surgical treatment options for syringomyelia,we reviewed our case series of patients without Chiari malformation or spinal injury.METHODS Excluding syringomyelia due to Chiari malformation,spinal cord injury,and tumors,32 patients(mean age 44 years)were operated on between 1995 and 2013and followed up for a mean of 53.8 months.Presumed causes at diagnosis,clinical and radiological findings,type of operation,clinical and radiological outcome were reviewed.RESULTS Duration of clinical history varied widely(range 6-164 months).Clinical assessment was based on the McC ormick classification(15 independent,17 dependent).Causes included birth trauma,pyogenic meningitis,tuberculous meningitis,postoperative scarring,dysraphism,and basilar impression.Treatment was local decompression with arachnoid lysis and shunts.Hindbrain-related syringomyelia was differentiated from non hindbrain-related syringomyelia.Hindbrain arachnoiditis was significantly associated with radiological findings at the foramen magnum(P=0.01)and craniocervical decompression(P0.03),with good clinical and radiological outcome at 6 months and later follow-up controls(P=0.02),whereas uneven results were observed in cases of non-hindbrain arachnoiditis.CONCLUSIONS To remove the cause of syringomyelia,surgical planning will rely on thorough clinical history and accurate imaging to determine the site of cerebrospinal fluid obstruction.Craniocervical decompression to dissect basal arachnoiditis in the posterior fossa can be recommended in hindbrain syringomyelia.Treatment of non-hindbrain arachnoiditis is more controversial,probably owing to uncertainties about the extent of adhesions.     

Revision of Chiari decompression for patients with recurrent syrinx

The management of adult patients with Chiari malformation associated with syrinx remains controversial. Although an abundance of literature exists for the pediatric population, there is an absence of guidelines for the adult population. It is unclear which of the different surgical approaches is appropriate in patients with Chiari I malformations and syringomyelia. A 36-year-old female patient had a posterior fossa decompression 3 years prior to recurrence. The patient developed recurrent symptoms with sensory loss and hyperesthesia in the right upper extremity. MRI revealed decreased cerebrospinal fluid flow at the craniocervical junction. The patient was taken to the operating room for revision of the posterior fossa decompression, lysis of adhesions and duraplasty. Re-exploration of a Chiari decompression, lysis of adhesions and revision duraplasty is an effective treatment option for recurrent syringomyelia.     

显微外科治疗Arnold-Chiari氏畸形Ⅰ型38例临床分析

目的探讨Chiari Ⅰ型畸形伴脊髓空洞显微外科治疗方式及效果。方法38例患者均采用枕大池成形术,其中29例采用长切口枕鳞部大骨窗,9例小切口小骨窗。结果术后有效者33例,采用不同手术方式的有效病例分别为长切口大骨窗25例、小切口小骨窗8例,统计学方面无明显差别。结论Chiari Ⅰ型畸形采用枕大池成形术可取得良好效果,小切口枕鳞部小骨窗可减小对患者的手术创伤,降低术后并发症,达到微创的目的。