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.     

分期前后方减压治疗合并颅底陷入的Chiari畸形

目的 合并颅底陷入的Chiari畸形患者常有前方脑干压迫,多数经前路减压即可使扁桃体上移,空洞缩小,但少数患者前路减压后Chiari畸形和脊髓空洞并不减轻,本文旨在探讨采用分期前后方减压治疗该类患者.方法 9例患者经口咽入路齿状突磨除,观察1-2个月后发现Chiari畸形和脊髓空洞未减轻,遂行后方枕大孔减压,小脑扁桃体切除,硬膜扩大修补,枕颈或C1～2固定融合术.随访24-60个月(平均32个月).结果 本组无死亡或严重并发症患者,所有患者症状改善,8例Chiari畸形和脊髓空洞改善,1例无变化.结论 合并颅底陷入的Chiari畸形患者应首先行经口咽入路齿状突磨除术,多数患者后方仅需固定即可,但有些患者颅底陷入症状缓解,而Chiari畸形依旧,需后方减压加固定手术.     

Chiari畸形合并脊髓空洞症的外科治疗

目的对Chiari畸形合并脊髓空洞症的手术治疗方法进行探讨,评价其治疗效果。方法对30例Chiari畸形病例(合并脊髓空洞症26例)根据其MRI影像特点,选择不同手术方式进行治疗:18例无或合并轻度脊髓空洞症者行后颅窝减压术,12例合并重度脊髓空洞症行后颅窝减压并脊髓空洞切开分流术,其中4例小脑扁桃体下疝超过C2水平行后颅窝减压并小脑扁桃体切除术。结果患者术后临床症状有不同程度改善。26例获随访1～4年,其中21例空洞明显缩小或消失,5例无明显变化;17例临床症状改善。结论对Chiari畸形合并脊髓空洞症患者依据其影像学特点采取不同的手术方式,疗效满意,手术能使颅颈交界处充分减压,改善临床症状,或控制病情进展。     

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.     

ChiarⅠ型畸形的MRI表现及其手术治疗

目的探讨ChiariⅠ型畸形的MRI分型及治疗方法.方法 23例Chiari Ⅰ型畸形患者,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例行后颅窝减压并小脑扁桃体切除术,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.     

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。     

Chiari畸形合并脊髓空洞的手术治疗

目的探讨Chiari畸形合并脊髓空洞的外科治疗方法及效果。方法对36例经MRI证实了的Chiari畸形合并脊髓空洞患者采用后颅窝减压术、小脑扁桃体切除术、枕大池成形术、脊髓空洞穿刺术、经口齿状突磨除术等综合手术治疗。结果36例Chiari畸形合并脊髓空洞者中，术后症状明显改善者29例，轻度改善者4例，无变化者3例。结论针对不同的Chiari畸形合并脊髓空洞患者，采用不同的手术方式，可获得良好的治疗效果。     

Chiari畸形研究进展

Chiari畸形是一种病理改变复杂的先天发育异常性疾病,以小脑扁桃体下疝至枕大孔平面以下为特征,常伴有脊髓积水.家族性Chiari畸形的研究提示其致病原因可能与某些基因的突变相关;后颅窝形态学研究证实其发病机制可能为后颅窝骨性发育不良,导致后颅窝拥挤所致;治疗方法多样,后颅窝减压术是目前治疗Chiari畸形最主要的术式.对于合并颅底凹陷的复杂Chiari畸形,可行经口齿状突切除、后外侧入路枕大孔减压加齿状突切除术及后颅窝减压加枕颈植骨融合术等.     

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。     

The complex Chiari: issues and management strategies

“Complex Chiari” malformations may be defined as cerebellar tonsil herniation combined with one or more of the following radiographic findings: brainstem herniation through the foramen magnum (Chiari 1.5 malformation), medullary kink, retroflexed odontoid, abnormal clival-cervical angle, occipitalization of the atlas, basilar invagination, syringomyelia or scoliosis. Patients with “complex Chiari” malformation are different from those with typical Chiari 1 malformation in that their management strategy has a higher chance of including other operative interventions aside from a typical suboccipital decompression. These other operative procedures include odontoid resection and craniocervical fusion. This paper outlines specific scenarios where these other operative procedures must be considered and carried out.     

Chiari Ⅰ畸形的外科治疗

目的探讨ChiariⅠ畸形的外科治疗方法。方法采用后颅窝减压,扩大重建术治疗6例；后颅窝减压,扩大成形术加脊髓空洞分流术治疗10例。结果所有患者症状和体征均明显改善,无死亡和其他并发症。结论后颅窝减压,扩大成形术和(或)脊髓空洞分流术是治疗ChiariⅠ畸形的有效方法。     

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。     

Posterior fossa decompression for Chiari I deformity,including resection of the cerebellar tonsils

This is an analysis of 19 consecutive cases of symptomatic patients with Chiari I deformities, undertaken to evaluate the long-term effect of posterior fossa decompression and duraplasty, assessed by postoperative imaging. Sixteen of the patients had syringomyelia and three had foramen magnum syndromes without a syrinx. Eighteen patients underwent posterior fossa craniectomy, subpial resection of the cerebellar tonsils, and duraplasty. Four patients were 16 years of age or younger. One of the children with syringomyelia had a posterior fossa decompression without resection of the tonsils. In the 15 patients with syringomyelia whose surgery included resection of the tonsils, the syrinx was reduced or resolved in 14. The patient whose syrinx did not change was a child with a lumbosacral lipoma. Three patients had syndromes of the foramen magnum without a syrinx, and of these only a patient with prior chemical and bacterial meningitis caused by a lumboureteral shunt failed to improve dramatically. When our patients are combined with 40 in the literature treated by decompression and duraplasty, 51 of 55 patients had reduction or resolution of the syrinx. Although it does not clearly affect the result, resection of the tonsils can be done safely.     

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.     

空洞分流对ChiariⅠ畸形伴脊髓空洞症近远期疗效的影响

目的比较chiariⅠ畸形伴脊髓宅洞症空洞分流与否对近远期疗效的影响，探讨脊髓空洞分流的必要性和临床意义，为临床选择手术方法提供依据．方法回顾性分析21例ChiariⅠ畸形伴脊髓空洞症手术治疗病例，观察8例经后颅窝减压 颈1、2或3椎板切开 硬脑膜修补(A组)与13例同时行脊髓空洞-蛛网膜下腔分流病人(B组)术后早期、随访半年以上临床症状与体征的变化，并采用Kamofsksyr行为能力评分法对两组病例远期疗效进行评定．同时比较术后空洞变化与临床症状的关系。结果A组术后早期症状改善5例(62．5％)。稳定3例，无症状恶化者，随访4例．按照Kamofsky，评分标准，临床症状改善3例，有效率75％(3／4)-B绀术后早期症状改善6例．稳定5例．恶化1例，有效率46.3％(6／13)；随访8例，临床症状改善7例，有效率87％(7/8)。随访MRI结果显示，B绀空洞缩小程度明显优于A组，多数空洞缩小者，症状好转，少数病例症状无变化结论后颅窝减压加颈1、2或3椎板切开及硬脑膜修补足治疗ChiariⅠ畸形伴脊髓空洞症的有效方法.加行空洞分流能有效缩小空洞体积，多数患者远期疗效优于未分流者，但部分病人存在无效或症状恶化可能。     

颅颈连接处的畸形

目的探讨颅颈连接处各种各样畸形的发生和发展。方法对38 例骨畸形伴小脑扁挑体下疝、脊髓空洞症的病人行手术治疗,其中20例行后颅窝减压硬膜扩大成形术,17例行单纯后颅窝减压及硬膜外囊带切除术,其中1例行单纯枕大孔减压后加枕骨C2椎体棘突固定术,1例未手术;6例行脊髓空洞穿刺术,2例行分流术。结果术后大部分病人得到恢复或改善。3例合并夏克关节的病人术后均有所改善,有椎体束损伤、肢体瘫痪、肌肉萎缩的病人短期内恢复欠佳。结论颅颈连接处骨畸形常并发神经系统以及附近软组织的发育异常。只要手术选择恰当,术后效果均比较满意。     

Chiari I malformation with syrinx

The Chiari I congenital malformation is characterized by caudal displacement of the cerebellar tonsils through the cervical canal. Although this malformation is often asymptomatic, coexisting syringomyelia can result in neurologic symptoms. We report a case of progressive ataxia with brainstem dysfunction in an adolescent female manifesting a severe Chiari I malformation with syrinx. Chiari decompression 4 years after initial presentation led to rapid improvement in most of her long-term symptoms. This case demonstrates the importance of consideration of Chiari I with syringomyelia in the differential diagnosis of progressive ataxia and brainstem symptoms.     

Management of craniocervical junction dislocation

The discovery of a craniocervical junction malformation requires management in three steps: (1) The patterns must be recognized using tomographic measurements (Chamberlain's line, Wackenheim's line). Dynamic flexion-extension studies are necessary to assess stability or instability. Stable patterns range from platybasia to basilar invagination, with gradual deformation, and are frequently associated with Chiari malformation. Unstable patterns characterized by odontoid instability are the equivalent of an odontoid fracture. The origin is malformative (hypoplasia, aplasia of the dens, os odontoidum), but the last may be difficult to distinguish from an old odontoid fracture. They are found in many syndromes (Down, Morquio, etc.). Unstable atlantoaxial patterns with atlas assimilation are hardly reducible; they evolve toward progressive instability. (2) The neurological consequences must be defined from the clinical features of the spinal cord and the cranial nerves. Both static and dynamic MRI scans must be performed; in this way identification of the neural abnormalities (hydromyelia, Chiari, etc.) and of the osseous compression is possible. (3) The most appropriate operative procedure must be selected: stable platybasia with a nervous compression by Chiari is cured only by posterior decompression; odontoid instability is cured by reduction and posterior fixation, using hooks and autologous bone grafts on the posterior arches of C-1 and C-2. Sometimes a transarticular screw fixation of C1-2 is necessary if there is a defect on the C-1 posterior arch. Craniocervical dislocations with assimilation of the atlas require posterior occipito-vertebral bony fixation with grafts and external halo immobilization or internal fixation with hooks or screws, with anterior transoral decompression in a second step.