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:     

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。     

复杂寰枕部畸形1例报道

1病例患者,女,24岁,因左下肢肌肉萎缩,肌力下降3年余入院。自2007年来出现进行性左下肢肌肉萎缩,肌力下降,偶伴头昏,无头痛、呕吐,不伴大小便失禁。颈椎正侧位,斜位,张口位X线片示:颈椎可见向左侧弯,生理曲度存在,各椎体及附件未见明显骨质增生,椎间隙正常,前纵     

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。     

保留寰椎后弓对Ⅰ型Chiari畸形手术效果的影响

目的研究保留寰椎后弓对小脑扁桃体下疝手术临床疗效的影响。方法回顾性分析2017-01—2019-05徐州医科大学附属医院收治的63例Ⅰ型Chiari畸形患者,其中35例行保留寰椎后弓的小脑扁桃体下疝手术,28例行寰椎后弓切除的小脑扁桃体下疝手术,比较寰椎后弓保留组和切除组患者手术时间、术中出血量、术后住院时间、术后并发症、术后12个月颈椎主动活动度等指标。术后随访1 a,使用KPS和CCOS评分评价两种术式对患者预后的影响。结果 2组住院时间、术中出血量、术后并发症、术后KPS和CCOS评分等方面差异无统计学意义(P0.05),而寰椎后弓保留组手术时间明显缩短,差异有统计学意义(P0.05);寰椎后弓切除组颈椎左右旋转角度较保留组明显减小,差异有统计学意义(P0.01)。结论保留寰椎后弓的小脑扁桃体下疝手术是一种安全有效的术式,保留寰椎后弓不仅缩短了手术时间,而且有效防止术后颈椎活动度的减小。     

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畸形脊髓空洞症发病机制的力学探讨

早在1564年Etienne就提出脊髓病理性空洞的改变，而脊髓空洞症（syringomyelia，SY）的名称则是于1873年由Oliver D’Angers提出的；1891年Arnold chiari通过尸检发现先天性枕大孔区畸形和后脑畸形中，多有脊髓空洞发现，故常称为：Arnold chiari畸形脊髓空洞症（ACM—SY）：本病好发于20—30岁左右的中青年。根据大宗SY自然病史的研究表明，该病为一种慢性进展性疾病，它的年发生率为8．4／10万人口。其病因学研究仍是目前争论的焦点，但其形成原因主要为枕大孔区畸形和小脑扁桃体下疝，即Arnold chiari畸形（Arnold chiari’s malformation，ACM）。此外，尚有髓内肿瘤、外伤和炎症等。随着MRI的问世，对SY的认识和外科干预起到了积极的推动作用，深入研究SY的发病机制对其治疗、疗效评价和动物模型的制作等有重要意义。     

Chiari畸形手术方法的改良

目的探讨小脑扁桃体下疝畸形(Chiari畸形)的手术方法，提高治疗效果，减少并发症方法本院自1993年12月至2003年12月对收治的86例Chiari畸形及部分合并脊髓空洞症患者实施了改良的枕大孔区小骨窗骨性减压、局部硬膜外层剥脱术。术后82例得到随访，平均随访5．5年(1～9年)，其中50例MRI复查头颈部？结果患者症状明显改善59例，部分改善10例，无变化13例，有效率占随访者84．15％，无手术并发症。结论改良的手术方式，在枕颈交界区骨性减压后，保留枕大孔区内层硬膜结构的情况下，可改善局部蛛网膜下腔脑脊液循环，缓解小脑扁桃体对延颈髓的压迫，效果较显著，同时减少了并发症。     

后颅窝减压术治疗Chiari畸形Ⅰ型合并脑积水疗效的Meta分析

目的 评价后颅窝减压术（PFD）治疗合并脑积水的Chiari畸形Ⅰ型的有效性。方法 计算机检索EBSCO、PubMed、EmBase、OVID等英文数据库以及中国知网、维普、万方等中文数据库,检索从建库到2015年5月31日发表的文献。采用STATA 13.0软件进行Meta分析。结果 共纳入3篇文献,86例,PFD治疗83例;PFD术后好转率为82.3%（95% CI 74.2%~90.4%）;合并脑积水的Chiari畸形Ⅰ型中,女性占62.9%（95% CI 52.6%~73.1%）。结论 合并脑积水的Chiari畸形Ⅰ型中,女性占大多数;PFD治疗合并脑积水的Chiari 畸形Ⅰ型具有较高的有效率。     

History,anatomic forms,and pathogenesis of Chiari I malformations

Introduction Chiari I malformations constitute a group of entities of congenital or acquired etiology that have in common descent of the cerebellar tonsils into the cervical spinal canal. In recent years, since the advent of magnetic resonance imaging, an increasing number of asymptomatic, doubtfully symptomatic, and minimally symptomatic patients with Chiari I malformations have been diagnosed. This has resulted in controversy about the multiple therapeutic strategies indicated for these problems.Object With the intention of updating the knowledge that we currently have on the Chiari I malformation and the related syringomyelia we review the literature and discuss the historical background, pathogenesis, anatomic forms, clinical presentation, and diagnostic procedures of these abnormalities.     

Posterior fossa morphometry in symptomatic pediatric and adult Chiari I malformation

The linear dimensions and volume of the posterior fossa, and the length of the supra-occiput and the clivus in children with Chiari I malformations (CMI) were studied. A statistical relationship between patient demographics, radiological features, posterior fossa and occipital bone morphometry in the study and control groups was investigated. The results of 21 pediatric patients was compared to those of a matched control group. The posterior fossa volume (PFV) of an adult CMI group was also studied. Linear measurements were used to calculate the length of the occipital bone, spherical PFV and intracranial volume (ICV) using pre-operative MRI and CT scans. A PFV to ICV ratio was obtained to standardize the comparison. The volumetric measurement in the pediatric study group was also compared to the adult CMI group.The antero-posterior dimension, width, and volume of the posterior fossa and the PFV to ICV ratio in pediatric CMI patients was significantly lower than in the control group (p < 0.05). There was no statistical difference in the length of the clivus and the supra-occiput between the two pediatric groups. The difference in the PFV to ICV ratio between pediatric and adult CMI patients was not statistically significant. Thus, the authors provide a simple, yet accurate, and reproducible method of comparison of posterior fossa volume in patients with CMI in different age groups, based on linear measurements. There is no significant difference between the length of the occipital bone at the base of the skull in pediatric CMI patients and the normal pediatric population. Development of the clivus due to late fusion of the sphenoid and occipital synchondroses in the second decade could result in manifestation of the disease in adulthood in CMI patients who were asymptomatic as children.     

有限小骨窗后颅凹减压显微术式治疗Chiari Ⅰ型畸形

目的 探讨采用有限小骨窗后颅凹减压显微术式治疗Chiari I型畸形的疗效. 方法 成都军区总医院神经外科自2004年至2008年采用有限小骨窗后颅凹减压显微术式治疗Chiari Ⅰ型畸形患者29例.标准手术程序包括有限小骨窗枕颈减压,硬膜扩大成形、硬膜下探查术、硬膜扩大修补等.按照Tator标准评价患者手术治疗的预后情况. 结果 本组患者手术效果评价优23例(79.3%),良6例(20.7%).远期随访患者15例,患者脊髓空洞进一步缩窄9例,复发1例.结论有限小骨窗后颅凹减压显微术治疗Chiari Ⅰ型畸形创伤小,疗效显著.     

The pathogenesis of syringomyelia associated with lesions at the foramen magnum: a critical review of existing theories and proposal of a new hypothesis

Syringomyelia is frequently accompanied by an extramedullary lesion at the foramen magnum, particularly a Chiari I malformation. Although syringomyelia associated with foramen magnum obstruction has characteristic clinical, radiological, and neuropathological features, its pathogenesis remains unclear. Currently prevalent hydrodynamical theories assert that obstruction of the subarachnoid space at the foramen magnum interferes with flow of cerebrospinal fluid (CSF) between the spinal and the intracranial subarachnoid compartments. As a result, spinal CSF is driven into the spinal cord through the perivascular spaces to form a syrinx. These theories are implausible biophysically because none postulates a pump adequate to drive fluid through these spaces. None of the theories can explain why syrinx pressure is higher than CSF pressure; why extensive gliosis, edema, and vascular wall thickening regularly occur; and why the composition of syrinx fluid is not identical with that of CSF.

A new theory of pathogenesis is proposed to address these difficulties. In the presence of subarachnoid obstruction at the foramen magnum, a variety of activities, such as assuming the erect posture, coughing or straining, and pulsatile fluctuations of CSF pressure during the cardiac cycle, produce transiently higher CSF pressure above the block than below it. There are corresponding changes in transmural venous and capillary pressure favoring dilation of vessels below the block and collapse of vessels above the block. The spatially uneven change of vessel caliber produces mechanical stress on the spinal cord, particularly caudal to the block. The mechanical stress, coupled with venous and capillary dilation, partially disrupt the blood–spinal cord barrier, allowing ultrafiltration of crystalloids and accumulation of a protein-poor fluid.

The proposed theory is consistent with the neuropathological findings in syringomyelia and with the pressure and composition of syrinx fluid. It also accounts for the prolonged course of syringomyelia and its aggravation by cough, strain, and assumption of an erect posture. It contributes to understanding the low incidence and the morphology of syringobulbia. It explains the poorly understood presentation of foramen magnum meningiomas with symptoms of a mid- to low-cervical myelopathy. The theory also affords an understanding of the late recurrence of symptoms in children with hydromyelia who are treated with a ventricular shunt.     

An efficacy analysis of posterior fossa decompression techniques in the treatment of Chiari malformation with associated syringomyelia

A retrospective study of the efficacy of posterior fossa decompression (PFD) was carried out in 132 patients with Chiari malformation (CM) with associated syringomyelia (SM). Of these 132 patients, 69 received extended PFD (large craniotomy group), and the other 63 patients received only local PFD (small craniotomy group). At the short-term postoperative evaluation (1–4 weeks) the extended PFD appeared to be more effective than the local PFD (p < 0.05). However, there was no significant difference in long-term analysis (6 months–11 years) (p > 0.05). In the large craniotomy group, there was no difference between the short-term and long-term efficacy (p > 0.05). However, in the small craniotomy group, long-term efficacy clearly improved (p < 0.05). Furthermore, patients who had undergone local PFD exhibited more obvious radiological improvement of SM (p < 0.05) and fewer postoperative complications compared to patients undergoing extended PFD (p < 0.05). Therefore, local PFD is preferable for the surgical treatment of CM with associated SM.     

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…     

枕骨大孔减压联合枕颈融合术治疗寰枕畸形的临床疗效

目的 探讨枕骨大孔减压联合枕颈融合术治疗寰枕畸形的临床疗效.方法 选择我院2011-03-2013-06收治的80例寰枕畸形患者作为研究对象,其中观察组40例采用枕骨大孔减压联合枕颈融合术,对照组40例采用单纯的枕骨大孔减压术进行治疗,比较2组患者的总有效率、平均住院时间、携带颈托的时间以及术后半年内的复发率.结果 手术后观察组患者的总有效率为92.5%,明显高于对照组患者,且组间差异具有统计学意义（χ2=4.50,P＜0.05）,观察组的平均住院时间、携带颈托时间分别为（9.5±2.1）d、（66.2±13.1） d,均明显短于对照组,组间差异具有统计学意义（P＜0.05）,且术后半年的随访结果 显示,观察组复发率2.5%,明显低于对照组,组间差异具有统计学意义（χ2=3.91,P＜0.05）.结论 枕骨大孔减压联合枕颈融合术治疗寰枕畸形可有效改善患者的临床疗效,大大提高了患者的生活质量,值得临床应用.     

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].