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。     

小脑扁桃体下疝并脊髓空洞症的诊治进展

小脑扁桃体下疝畸形也称Chiari畸形(Chiarimalformation,CM),是一种以小脑扁桃体向下疝入枕骨大孔为特征的先天性畸形,脊髓空洞症(syringomyelia,SM)为其常见的合并症。本文就CM并SM的诊治进展作一综述。一、CM脊髓空洞的发病机理1.CM的发病机理:Chiari畸形的发病学说很多,但均不能满意地解释一些问题。现较流行的学说为CM胚胎中胚层枕骨部体节发育不良,导致枕骨发育滞后,而小脑脑干发育正常出现后颅窝过度拥挤现象,从而疝出到椎管内[1]。研究表明病人后颅窝容积明显小于正常人是该病的特征性表现[2,3]。Badie等[4]测量病人和正常人…     

从小脑扁桃体下疝看Chiari畸形

Chiari畸形（Chiari malformation,CM）,即小脑扁桃体下疝畸形,迄今已经有100多年的历史。虽然第一个提出该病的John Cleland比Hans Chiafi和JuliusAmold早了10年,但真正对该病进行了系统研究的是奥地利人HansChiari,     

Chiari畸形合并脊髓空洞症的MRI研究

Chiari畸形(CM)中有20％～70％常与脊髓空洞症(SM)合并发生,单纯CM引起的症状多为后组颅神经症状,常不能引起病人注意,多以脊髓损害导致的运动异常及感觉异常而就诊。我院自1989年至1996年共收治脊髓空洞患者1080例,对术后半年有MRI复查资料的162例患者术前MRI资料进行回顾性分析,现报告如下:     

小脑延髓池成形术治疗Chiari畸形并脊髓空洞症

目的观察合并脊髓空洞症的Chiari畸形的临床和影像学特征.探讨小脑延髓池成形术对其治疗效果.方法对15例Chiari畸形并脊髓空洞症病人,采用后正中入路,枕颈减压,小脑延髓池成形术治疗.结果术后经6～58个月临床和影像学随访.15例中,空洞消失、症状消失者4例(痊愈);空洞消失或缩小、症状明显减轻者8例(显效);空洞缩小、症状改善者3例(有效).结论小脑延髓池成形术能使小脑扁桃体还纳,畅通颅内和脊髓蛛网膜下腔CSF循环,脊髓空洞逐渐缩小,有助于改善临床症状.     

后颅窝内容物内减压治疗Chiari畸形并脊髓空洞症

自2006年1月到2007年11月,作者对10例Chiari畸形并脊髓空洞,行小脑扁桃体下疝及脊髓中央管隔膜切除,使后颅窝内容积扩大,恢复脑脊液循环通畅,后颅窝骨瓣复位,称之为后颅窝内容物内减压。报告如下。     

脊髓空洞症与小脑扁桃体下疝19例报告

目的　探讨脊髓空洞症与小脑扁桃体下疝的关系。方法　选择 19例脊髓空洞症合并小脑扁桃体下疝的患者 ,结合临床与MRI资料进行分析。结果　脊髓空洞症好发于颈髓段 ,且高颈段多见。结论　小脑扁桃体下疝与脊髓空洞症关系密切 ,小脑扁桃体下疝是脊髓空洞症的一个重要成因。     

Chiari畸形合并脊髓空洞症的显微手术治疗

目的探讨Chiari畸形合并脊髓空洞症的治疗方法及效果。方法 2008~2013年收治Chiari畸形合并脊髓空洞症患者32例,20例采用后颅窝减压寰枕筋膜松解术,12例空洞严重者采用后颅窝减压寰枕筋膜松解术＋空洞-蛛网膜下腔分流术。结果术后随访0.5~3年,28例患者症状改善,4例无变化;25例复查MRI示下疝的小脑扁桃体回复,23例脊髓空洞较术前明显缩小。结论后颅窝减压寰枕筋膜松解术治疗Chiari畸形合并脊髓空洞效果明显,并发症少。     

Chiari畸形并脊髓空洞症的外科治疗

Ｃｈｉａｒｉ畸形并脊髓空洞症的外科治疗黄延林张懋植王磊北京天坛医院神经外科于１９９２年１２月～１９９６年５月共收治７２例Ｃｈｉａｒｉ畸形并脊髓空洞症的病人，以不同的方式进行了手术，其中４４例得到随访，现分析如下。临床资料１．一般资料：本组男性４４例，...     

脊髓空洞症与小脑扁桃体下疝19例报告

目的 探讨脊髓空沿症与小脑扁桃体下疝的关系。方法 选择19例脊髓空洞症合并小脑病扁桃体下疝的患者，结合临床与MRI资料进行分析。结果 脊髓空沿症好发于颈髓段，且高颈段多见。结论 小脑扁桃体下疝与脊髓空沿症关系密切，小脑扁桃体上疝是脊髓空沿症的一个重要成因。     

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。     

Treatment of Syringomyelia due to Chiari Type I Malformation with Syringo-Subarachnoid-Peritoneal Shunt

Chiari type I malformation is a tonsillar herniation more than 3 mm from the level of foramen magnum, with or without concurrent syringomyelia. Different surgical treatments have been developed for syringomyelia secondary to Chiari''s malformations: craniovertebral decompression with or without plugging of the obex, syringo-subarachnoid, syringo-peritoneal, and theco-peritoneal shunt placement. Shunt placement procedures are useful for neurologically symptomatic large-sized syrinx. In this paper, authors define the first successful treatment of a patient with syringomyelia due to Chiari type I malformation using a pre-defined new technique of syringo-subarachnoid-peritoneal shunt with T-tube system.     

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畸形合并脊髓空洞症的外科治疗

Chiari畸形又称小脑扁桃体下疝畸形.John Cleland于1883年首次描述了脑干、小脑移位变形和延髓空洞,Chiari分别于1891年、1895年将颅-椎移行区神经轴下移的程度进行了分类,1894年德国病理学家Arnold又作了更为详尽的描述,因此,1907年Schwalb和Gredig将此命名为ArnoldChiari畸形,而Sarnat和Williams则称其为Chiari畸形或描述表达为小脑扁桃体下疝畸形[1].     

Stereological and Morphometric Analysis of MRI Chiari Malformation Type-1

Objective

In this study, we aimed to investigate the underlying ethiological factors in chiari malformation (CM) type-I (CMI) via performing volumetric and morphometric length-angle measurements.

Methods

A total of 66 individuals [33 patients (20-65 years) with CMI and 33 control subjects] were included in this study. In sagittal MR images, tonsillar herniation length and concurrent anomalies were evaluated. Supratentorial, infratentorial, and total intracranial volumes were measured using Cavalieri method. Various cranial distances and angles were used to evaluate the platybasia and posterior cranial fossa (PCF) development.

Results

Tonsillar herniation length was measured 9.09±3.39 mm below foramen magnum in CM group. Tonsillar herniation/concurrent syringomyelia, concavity/defect of clivus, herniation of bulbus and fourth ventricle, basilar invagination and craniovertebral junction abnormality rates were 30.3, 27, 18, 2, 3, and 3 percent, respectively. Absence of cisterna magna was encountered in 87.9% of the patients. Total, IT and ST volumes and distance between Chamberlain line and tip of dens axis, Klaus index, clivus length, distance between internal occipital protuberance and opisthion were significantly decreased in patient group. Also in patient group, it was found that Welcher basal angle/Boogard angle increased and tentorial slope angle decreased.

Conclusion

Mean cranial volume and length-angle measurement values significantly decreased and there was a congenital abnormality association in nearly 81.5 percent of the CM cases. As a result, it was concluded that CM ethiology can be attributed to multifactorial causes. Moreover, congenital defects can also give rise to this condition.     

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 malformation presenting with hearing loss: surgical treatment and literature review

OBJECTS: Chiari I malformations can present with a number of clinical signs and symptoms. METHODS: We present a case of an 11-year-old girl that presented with significant sensorineural hearing loss as her only Chiari-related symptom. The patient had four audiograms that all demonstrated progressive bilateral hearing loss between 10 and 30 dB. On magnetic resonance scan, the patient was found to have a Chiari I malformation. The patient had 9 mm of tonsillar herniation but no syrinx or hydrocephalus was present. On exam, the patient did not exhibit any other symptoms of her Chiari malformation or cranial nerve abnormalities other than sensorineural hearing loss. The patient underwent a suboccipital craniotomy, C1 laminectomy, and duraplasty. The patient noted a subjective improvement in hearing and an audiogram performed at 3 months postoperatively demonstrated normal hearing bilaterally. CONCLUSIONS: Sensorineural hearing loss may be caused by Chiari I malformations. This symptom may improve following decompression.     

Chiari畸形的外科治疗及相关问题探讨

目的 探讨Chiari 畸形的外科手术治疗和相关临床问题.方法 回顾分析35 例Chiari 畸形(26 例合并脊髓空洞)的外科治疗经验.全部病例均行后颅窝减压,C1 后弓减压,枕大池扩大成形,小脑扁桃体下疝切除术或电灼术.结果 全部病例随访6 个月～ 4 年,获得不同程度的临床症状改善,动态复查影像均获得良好的效果.结论 Chiari 畸形的外科治疗中根据临床症状和影像特点,个性化的选择行后颅窝大或小的骨窗减压,枕大池扩大成形和小脑扁桃体下疝切除手术治疗,是处理该种畸形获得临床疗效的好方法.     

Chiari畸形外科治疗回顾与进展

Chiari畸形的外科治疗始于1932年,70余年来随着医学技术的不断进步,其手术方式不断改进。然而,由于发病机制至今尚未明确,致使不能形成一种临床公认的最佳外科手术方法,手术过程中不同层次的操作步骤或具体方法仍存有争议。本文拟就Chiari畸形的概念、发病机制、诊断与神经外科手术治疗方法进行回顾,并对目前的热点问题和新技术应用进行概述。