Chiari畸形合并脊髓空洞的诊治体会

目的探讨Chiari畸形合并脊髓空洞的手术方法.方法通过对12例经磁共振扫描确诊为Chiari畸形合并脊髓空洞的病人,应用枕颈部彻底减压脊髓空洞内引流的治疗.结果 12例病人手术治疗后临床症状和体征均有不同程度的改善;经随访明显好转8例,好转4例,行磁共振复查8例空洞完成消失,4例较术前明显缩小,无病人死亡和手术并发症.结论枕颈部彻底减压加脊髓空洞内引流是治疗Chiari畸形合并脊髓空洞症的一种有效方法.     

22例Chiari畸形Ⅰ型合并脊髓空洞症的疗效分析

目的探讨后颅窝减压术合并枕大池重建术治疗Chiari畸形Ⅰ型合并脊髓空洞症的手术疗效。方法回顾性分析22例Chiari畸形Ⅰ型合并脊髓空洞症患者的临床资料。结果手术后1周内症状消失或改善的19例;随访术后6个月~2年,症状消失或改善的14例;其中脊髓空洞症减小或消失10例。结论后颅窝减压合并枕大池重建术是临床治疗Chiari畸形Ⅰ型合并脊髓空洞症安全有效的手术方法。     

Chiari畸形合并脊髓空洞的诊治体会

目的 探讨Chiari畸形合并脊髓空洞的手术方法。方法 通过对12例经磁共振扫描确诊为Chiafi畸形合并脊髓空洞的病人，应用枕颈部彻底减压脊髓空洞内引流的治疗。结果 12例病人手术治疗后临床症状和体征均有不同程度的改善；经随访明显好转8例，好转4例，行磁共振复查8例空洞完成消失，4例较术前明显缩小，无病人死亡和手术并发症。结论 枕颈部彻底减压加脊髓空洞内引流是治疗Chiari畸形合并脊髓空洞症的一种有效方法。     

枕大池扩大重建加小脑扁桃体切除术治疗Chiari畸形合并脊髓积水

目的探讨治疗Chiari畸形合并脊髓积水的手术方法.方法 19例Chiari畸形合并脊髓积水患者,脊髓空洞腔＜5节段3段,5～8节段12例,＞8节段4例,伴延髓空洞3例,手术扩大枕大池并切除小脑扁桃体.结果 19例术后患者症状明显好转;MR复查,空洞腔明显缩小.结论该术式治疗Chiari畸形合并脊髓积水安全、效果好.     

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。     

Chiari畸形合并脊髓空洞症的临床分类和手术方式探讨

目的 探讨Chiari畸形合并脊髓空洞症的临床分类和手术方式。方法 对54例Chairi畸形合并脊髓空洞症患，根据脊髓空洞大小，分别采用单纯后颅窝减压术和后颅窝减压术加空洞分流术治疗。结果 两种术式均使Chiari患症状和空洞缩小。结论 脊髓空洞大小对Chiari畸形合并脊髓空洞症患的手术有指导意义。     

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。     

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。     

后颅窝重建术治疗Chiari畸形合并脊髓空洞

目的探讨后颅窝重建手术治疗Chiari畸形合并脊髓空洞的方法及效果。方法27例Chiari畸形合并脊髓空洞患者经MRI确诊后,应用后颅窝重建显微外科技术治疗,并随访3月以上。结果27例患者术后症状均有不同程度的改善,复查头颅MRI见脊髓空洞缩小。结论该手术可使颅颈交界区充分减压,改善脑脊液循环,有效缓解临床症状,并对脊髓空洞症有明显的治疗作用。扩大修补硬脑膜有利于减少术后并发症的发生。     

ChiariΙ型畸形手术方式的选择

目的探讨Chiari Ι型畸形合并脊髓空洞症、颈椎病等的手术治疗方法.方法通过对174例经MRI证实的Arnold-Chiari畸形患者进行回顾性研究,分析不同的合并症与手术方式之间的关系.结果 174例中全部有小脑扁桃体下疝及脊髓空洞,合并颅底凹陷51例,脊柱侧弯19例,环枕融合20例,颈椎管狭窄22例.手术总有效率94.8%.结论 Chiari Ι型畸形系颅颈交界区畸形,常合并不同的脊柱、脊髓畸形.手术时应详细了解病变情况,采取不同的手术方式.     

Scoliosis in a child with Chiari I malformation and the absence of syringomyelia: case report and a review of the literature

Background Scoliosis is a common finding in children with Chiari I malformation and syringomyelia. The development of scoliosis associated with a Chiari malformation typically has been ascribed to the presence of syringomyelia. Decompression of the hindbrain hernia will often alleviate the syringomyelia and, in many cases, stabilize or reverse the scoliosis.Case report We report a child with Chiari I malformation and scoliosis who presented with Valsalva-induced headache/neck pain. MRI revealed no syringomyelia or hydrocephalus. Radiographs revealed that her scoliotic curvature was approximately 13° and was a single levoscoliotic curve. This patient underwent a posterior fossa decompression with duraplasty for her Valsalva-induced pain. Postoperatively, she had resolution of her pain and there has been no progression of her scoliosis at 3 years follow-up.Prognosis Intriguingly, and scattered throughout the medical literature, many have noted cases of scoliosis in patients with only a Chiari I malformation and no syringomyelia. Moreover, experimental studies have induced scoliosis in animals after compression of the dorsal columns. After a review of the medical literature regarding a potential cause and effect of herniated hindbrain-induced scoliosis in the absence of syringomyelia, this association although rare, does seem plausible.     

Scoliosis associated with syringomyelia presenting in children

The clinical presentations and radiological features of scoliosis accompanying syringomyelia were analyzed in 14 cases of syringomyelia associated with a Chiari malformation in children. Scoliosis was the initial symptom in 11 out of 16 patients (64%) with syringomyelia and present in 14 (88%) at the initial examination. The scoliosis associated with syringomyelia was characterized by a higher incidence of a single curve (6 cases, 43%) and convexity to the left (7 cases, 50%) than seen in idiopathic scoliosis. The syrinx was shifted to the convex side of scoliosis on the axial section at the middle or lower thoracie level in patients with a single curve, and at the cervical or upper thoracic level in patients with a double curve. The authors think that the scoliosis develops in children as a result of damage done to the anterior horn, which innervates the muscles of the trunk, by an asymmetrically expanded syrinx.     

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°.     

Consensus conference on Chiari: a malformation or an anomaly? Scoliosis and others orthopaedic deformities related to Chiari 1 malformation

In this article, we analysed the orthopaedic malformation in patients affected by Chiari I malformations as well as conservative or surgical treatment. The most common deformity in these patients is scoliosis. Different studies suggest a causal relation between syringomyelia and spinal deformities that differ by the type of deformities: asymptomatic scoliosis is characterized by a higher incidence of a single curve and convexity to the left, while symptomatic scoliosis is characterized by a double thoracolumbar curve. The conservative treatment with brace in these patients is not effective and scoliosis is typically evolutive. The evidence of the international data is that in patients without myelomeningocele or congenital scoliosis, but with Arnold Chiari I malformation and syringomyelia, suboccipital craniectomy gives the best chance for syrinx reduction and scoliosis improvement, particulary in children younger than 10 years and below a Cobb angle of 30°. The orthopaedic treatment in late decompression or in progressive curve is spine arthrodesis.     

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.     

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。     

Isolated Horner's syndrome and syringomyelia

Although syringomyelia has been associated with Horner's syndrome, it is typically associated with other neurological findings such as upper limb weakness or numbness. A patient is described who had an isolated Horner's syndrome as the only manifestation of syringomyelia. A 76 year old woman was discovered to have right upper lid ptosis and right pupillary miosis. Neurological examination was unremarkable, and pharmacological testing was consistent with localisation of the lesion to a first or second order sympathetic neuron. Neuroimaging disclosed a Chiari I malformation with a syrinx extending to the C2 to C4 level. An isolated Horner's syndrome may be the presenting manifestation of syringomyelia.     

Consensus conference on Chiari: a malformation or an anomaly? Scoliosis and others orthopaedic deformities related to Chiari 1 malformation

In this article, we analysed the orthopaedic malformation in patients affected by Chiari I malformations as well as conservative or surgical treatment. The most common deformity in these patients is scoliosis. Different studies suggest a causal relation between syringomyelia and spinal deformities that differ by the type of deformities: asymptomatic scoliosis is characterized by a higher incidence of a single curve and convexity to the left, while symptomatic scoliosis is characterized by a double thoracolumbar curve. The conservative treatment with brace in these patients is not effective and scoliosis is typically evolutive. The evidence of the international data is that in patients without myelomeningocele or congenital scoliosis, but with Arnold Chiari I malformation and syringomyelia, suboccipital craniectomy gives the best chance for syrinx reduction and scoliosis improvement, particulary in children younger than 10 years and below a Cobb angle of 30°. The orthopaedic treatment in late decompression or in progressive curve is spine arthrodesis.     

Syringomyelia in children with primary scoliosis

The clinical notes of 35 children presenting with scoliosis were reviewed; all 35 had been investigated with MRI. Seven were found to have syringomyelia, and six of these had Chiari malformation. Correction of the syrinx resulted in improvement or stabilisation of the spinal curvature. We recommend that all cases presenting with primary scoliosis should have MRI and should be treated if a syrinx is found. Received: 22 July 1998 Accepted: 31 August 1998     

Familial Chiari type I malformation with syringomyelia in two siblings: case report and review of the literature

Objective Familial cases of Chiari malformation with syringomyelia are rare. The majority of the reported series and case reports detail sporadic cases. The authors report two siblings who presented with Chiari type I malformation and syringomyelia (CMI+S).Clinical presentation We report two sisters who each presented with scoliosis on routine school physicals. Their clinical examination was unremarkable; however, imaging studies demonstrated a Chiari malformation with syringomyelia. Both underwent cervicomedullary decompression, and follow-up imaging studies revealed resolution of the syringomyelia.Conclusion A review of the literature reveals fewer than ten previous reports of familial CMI+S in the past 30 years. Although rare, the existence of familial cases of CMI+S suggests a genetic component to the pathogenesis of this condition in at least a proportion of patients. Neurosurgeons should be aware of the familial aggregation of CMI+S.