Scoliosis in children with Chiari I-related syringomyelia

Objective To study the relationship between scoliosis and Chiari I malformation, with reference to the possible role of cranio-vertebral decompression in preventing the need for scoliosis correction.Material and methods Out of a total of 36 patients with symptomatic Chiari I, who underwent primary cranio-vertebral decompression by a single paediatric neurosurgeon with an interest in Chiari malformation between 1998 and 2003, 13 had clinically detected scoliosis. Of these, ten had no other structural spine abnormality, which could influence the natural history of scoliosis and were included in this study.Results In all but one patient, syringomyelia improved significantly after cranio-vertebral decompression. Of the ten patients, eight had levoscoliosis (left convexity), all single curves, and two had curves to the right (both double curves). Six patients did not require corrective scoliosis surgery after cranio-vertebral decompression. The mean Cobb angle for those not requiring scoliosis correction was 29° in contrast to a mean of 76° for those requiring correction [p=0.001, one-way analysis of variance (ANOVA)]. The mean age of patients requiring corrective surgery was 158 months against 125 months for those not requiring correction (p=0.084, one-way ANOVA). These findings were confirmed by multivariate analysis, which also confirmed that symptom duration, syrinx length and site were not significant in predicting the need for corrective surgery following cranio-vertebral decompression.Conclusions Cranio-vertebral decompression for Chiari I may prevent the need for corrective scoliosis surgery when performed before the age of ten and below a Cobb angle of 30°.     

Morphometric analysis of the craniocervical juncture in children with Chiari I malformation and concomitant syringobulbia

Introduction  Although very uncommon, Chiari I malformation (CIM) with syringomyelia may be associated with concomitant syringobulbia. We hypothesized that the anatomy of the craniocervical region may be different in CIM patients with syringomyelia who develop syringobulbia in conjunction with their syringomyelia compared to other patients with CIM with and without syringomyelia. The present study was conducted in order to prove or disprove such a theory. Materials and methods  A group of 189 children with operated CIM were reviewed for the presence of syringobulbia, and this cohort then underwent morphometric analyses of their craniocervical juncture. These measurements were then compared to both our prior patient findings and historic controls. Results  The current study did not identify any morphometrical peculiarities for patients with CIM and syringobulbia compared to other CIM patients with and without isolated syringomyelia. Conclusions  Based on our study, the mechanism behind such cerebrospinal fluid distention into the brain stem remains elusive with no single morphometrical difference in patients with CIM and syringobulbia compared to other patients with CIM. Perhaps, future testing aimed at identifying pressure gradients across the foramen magnum in patients with and without syringobulbia and concomitant CIM may be useful.     

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…     

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].     

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。     

Chiari畸形的临床诊断和显微手术治疗体会

目的 探讨Chiari畸形的诊断及显微外科处理方法.方法 回顾性分析50例Chiari畸形的临床资料,其中合并脊髓空洞30例,齿状突型颅底陷入3例.结果 50例术后平均随访35个月,按Tator疗效标准:优36例(72%);良12例(24%);差2例(4%).有效率96%.空洞缩小21例,消失7例.结论 有限的后颅窝减压及枕大池重建是治疗Chiari畸形的有效方法;合并齿状突型颅底陷入应先行口咽入路齿状突磨除术或许为较好的选择.

Abstract：

Objective To study the diagnosis and the microsurgical treatment for Chiari malformation(CM).Methods The clinical data of 50 cases of Chiari malformation were analyzed retrospectively,30 cases were combinated with syringomyelia,3 with cranial basal invagination referred to dens of axis.Results 50 cases were followed up for an average 35 months.According to Tator's standard,the curative effect was excellent in 36,good in 12 and bad in 2 patients.The effective rate was 96%.The cavities in spinal cords reduced in 21 cases,disappeared in 7.Conclusion Limited posterior fossa decompression with reconstruction of cistern magna is a effective method for treatment of CM;CM with cranial basilar invagination referred to dens of axis treated firefly by anterior decompression of odontoidectomy via oral approach may be an optimal selection.     

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.     

Winged scapula as the presenting symptom of Chiari I malformation and syringomyelia

Case report We report a 19-year-old girl with a 4-month history of an inability to fully elevate her upper extremity past the horizontal position. Physical examination revealed a winged scapula. MRI was demonstrative of a Chiari I malformation with a small cervical syrinx eccentrically placed to the same side as the dysfunctional extremity. Discussion We believe this to be the first report of dysfunction of the long thoracic nerve via a hindbrain hernia-induced syrinx with a resultant winged scapula. We would hypothesize that enough anterior horn motor neurons and their axons destined for the long thoracic nerve were injured by the syringomyelia to result in isolated deinnervation of the serratus anterior muscle. Conclusion The clinician may wish to include syringomyelia in the differential diagnosis of a winged scapula.     

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。     

神经内镜下寰枕部减压手术治疗Chiari畸形

目的 探讨神经内镜下寰枕减压手术治疗Chiari畸形的手术方法和临床疗效.方法 对23例经MRI确诊为Chiari畸形但不伴有寰枢锥脱位和齿突凹陷的患者在神经内镜下进行了寰枕减压手术,并进行疗效观察.结果 23例术后随访0.5-3.0年,原有症状均有不同程度改善,感觉障碍程度减轻,肢体肌力有所增强.无手术后并发症.术前合并脊髓空洞症的16例患者中,术后7例空洞消失,8例范围缩小,1例范围无明显变化.结论 神经内镜下寰枕减压手术治疗Chiari畸形方法可行,手术安全、有效、创伤小、疗效满意.

Abstract：

Objective To investigate the methods and effectiveness to treat Chiari malformation with the surgical decompression of the foramen magnum under endoscope. Method 23 cases with Chiari malformation.(without atlanto- axialdislocation and basilar invagination) diagnosed by magnetic resonance imaging( MRI)were operated with the surgical decompression of the foramen magnum under the endoscope. Results 23 cases were followed- up 0. 5 -3. 0, years after operation, all the symptoms and signs were remarkably relieved after the operation. No complications was found. In 16 cases with spinal cavities,7 cases spinal cavities disappeared and scases deflated,l case no evident change. Conclusions The endoscope -assisted decompression of the foramen magnum is a safe and effective surgical method to treat Chairi malformation.     

Diagnosis of Chiari I malformation and related syringomyelia: radiological and neurophysiological studies

Introduction The diagnosis of Chiari I malformation relies mainly on the anatomical demonstration of the abnormal position of the cerebellar tonsils below the foramen magnum, and of the associated bony anomalies of the posterior cranial fossa and craniocervical junction, as well as of the eventually-associated spinal cord cavitations. Consequently, the neuroradiological work-up plays a fundamental role in the definition of the malformation and in the follow-up of operated patients.Review The authors review the pertinent literature on the neuroradiology of the Chiari I malformation, with special regard to MRI, with the aim of providing the reader with an updated instrument for its diagnosis.     

Clinical features of Chiari I malformations

Background With the advent of MRI, the diagnosis of Chiari I malformation is being made in younger patients, often with minimal or no neurologic symptoms.Clinical features The symptoms and signs of a Chiari I malformation may be related to the compression of neural and/or dural structures at the cranio-cervical junction or may be related to the associated syringomyelia. Scoliosis is a particularly important and common finding in those children with syringomyelia.     

Chiari 1.5 Malformation : An Advanced Form of Chiari I Malformation

The Chiari 1.5 malformation is defined as a tonsillar herniation within a Chiari I malformation with additional caudal descent of the brainstem through the foramen magnum. We describe a patient with Chiari I malformation who evolved to Chiari 1.5 malformation during longitudinal follow-up. A 15-year-old girl presented with neck pain during exercise for two years. She had been diagnosed with Chiari I malformation with mild hydrocephalus after minor cervical trauma at the age of six years. At that time, she was asymptomatic. After she complained of aggravated neck pain, neuroimaging (nine years after first imaging) revealed caudal descent of the brainstem and syringomyelia in addition to progression of tonsillar herniation. Posterior fossa decompressive surgery resulted in complete resolution of neck pain. Based on neuroimaging and operative findings, she was diagnosed as Chiari 1.5 malformation. Neuroimaging performed seven months after surgery showed an increased anterior-posterior diameter of the medulla oblongata and markedly decreased syringomyelia. This case demonstrates progressive developmental process of the Chiari 1.5 malformation as an advanced form of the Chiari I malformation.     

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。     

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.     

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。     

脊柱侧凸为首发症状的Chiari畸形临床研究

目的 研究以脊柱侧凸为首发症状的Chiari畸形的临床特征和治疗策略.方法 收集南京鼓楼陕院住院治疗的Chiari畸形患者60例,35例以脊柱侧凸为首发症状;25例末合并脊柱侧凸作为对照组,病例对照研究.结果 35例Chiari畸形合并脊柱侧凸患者,年龄3～33岁(平均13.75岁),97%合并脊髓空洞,不具有典型的Chiari畸形的临床症状和体征,以腹壁反射异常多见.两组之间手术方式和手术并发症无统计学意义.结论 Chiari畸形以脊柱侧凸为首发症状,多合并脊髓空洞,发病年龄轻,其临床症状和神经损害体征不明显,治疗策略上主张早期进行枕颈部枕骨大孔区减压和硬脑膜重建成形手术.     

Segmental hyperhidrosis in syringomyelia with Chiari malformation

Segmental hyperhidrosis was seen in three cases of syringomyelia with Chiari malformation confirmed by magnetic resonance imaging. Using the iodine starch reaction, all three cases showed hyperhidrosis in the areas roughly corresponding to those of sensory disturbances. Two possible modes of pathogenesis are discussed: stimulation of preganglionic neurons and interference to the inhibitory tract.     

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。