Chiari畸形合并脊髓空洞症的手术治疗

目的 探讨Chiari畸形合并脊髓空洞症的手术方法和疗效。方法 对16例经MRI证实的Chiari畸形合并脊髓空洞症患者行后颅窝减压术加空洞．蛛网膜下腔分流术并评定其疗效。结果 按Tator标准进行判定，优12例，良3例，差1例，有效率为93．75％，无手术死亡者。结论对有症状的合并Chiari畸形的脊髓空洞症患者早期手术有利于减轻症状和神经功能的恢复。     

Chiari Ⅰ畸形伴脊髓空洞的显微外科治疗

我院自1995年5月至2004年1月共收治Chiari Ⅰ畸形伴脊髓空洞症患者37例，全部病例均经MRI诊断，并在显微镜下行手术治疗，取得了较好的治疗效果．     

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。     

ChiariⅠ畸形合并脊髓空洞症脑积水的治疗

探讨ChiariⅠ畸形合并脊髓空洞症的机理及后颅凹减压术的作用与特点。全部手术治疗,明显好转7例,显效3例,加重1例,无死亡。强调后颅凹减压术在治疗ChiariⅠ畸形的重要性。     

86例Chiari畸形Ⅰ型合并脊髓空洞症患者手术疗效及预后分析

目的 研究Chiari畸形Ⅰ型（CM-Ⅰ）合并脊髓空洞症患者接受后颅窝减压合并小脑扁桃体切除术（PFDRT）的效果,探究影响患者预后的相关因素。方法 选择2016年1月—2022年2月郑州大学第一附属医院神经外科采用PFDRT治疗的成年CM-Ⅰ合并脊髓空洞症患者86例,分析患者手术前后的临床特征、影像学特征以及随访资料。使用芝加哥Chiari结局量表（CCOS）作为患者临床预后的评估指标,患者预后相关影响因素的分析则采用单因素及多因素Logistic回归。结果 该组临床治愈72例（83.72%）,脊髓空洞完全消失12例（13.95%）,脊髓空洞好转79例（91.86%）;术后发热14例（16.28%）,枕下积液5例（5.81%）。术后患者影像学指标较术前均有显著改变（P＜0.001）,大多数患者术后临床症状较术前改善明显（P＜0.05）。多因素Logistic回归分析均显示病程和小脑相关症状是患者临床治愈的危险因素,病程越长的患者预后越差,有小脑相关症状的患者预后较差。结论 PFDRT是治疗CM-Ⅰ合并脊髓空洞症患者的有效手段,长病程以及小脑相关症状均影响患者预后,对于有临床症状的CM-Ⅰ合并脊髓空洞症患者应该尽早治疗。     

后颅窝内减压治疗Chiari 畸形Ⅰ型并脊髓空洞中期疗效评价

目的 评价后颅窝内减压(后颅窝减压+后颅窝颅骨成形术)治疗Chiari 畸形Ⅰ型并脊髓空洞疾病的中期临床疗效.方法 采用后颅窝内减压术治疗40 例符合标准的Chiari 畸形Ⅰ型并脊髓空洞.观察手术时间、切口愈合情况,分析相关并发症.采用远期生活质量评估(KPS)和美国Lawton 和Brody1996 年制定的日常生...     

后颅窝内减压治疗Chiari 畸形Ⅰ型并脊髓空洞中期疗效评价

目的 评价后颅窝内减压(后颅窝减压+后颅窝颅骨成形术)治疗Chiari 畸形Ⅰ型并脊髓空洞疾病的中期临床疗效.方法 采用后颅窝内减压术治疗40 例符合标准的Chiari 畸形Ⅰ型并脊髓空洞.观察手术时间、切口愈合情况,分析相关并发症.采用远期生活质量评估(KPS)和美国Lawton 和Brody1996 年制定的日常生活能力量表评分(ADL)评价疗效,并且通过影像学检查对脊髓空洞的变化、脑脊液流速情况以及颅颈交界区稳定性情况进行评价.结果 随访KPS 分值由术前的(70 ±10)分提高到到末次随访时(80 ± 10)分(P ＜0.01),ADL 分值由术前的(20.6 ±2.2)分降至末次随访时(15.8 ± 0.5)分(P ＜ 0.01),最终疗效评价优良者占86%.颅颈交界区影像学观察脊髓空洞缩小及消失率为90.5% (19 /21).脑脊液流速较术前加快.结论 后颅窝内减压术治疗Chiari 畸形Ⅰ型并脊髓空洞有一定疗效.     

Arnold ChiariⅠ畸形合并脊髓空洞的手术分析

目的 探讨Arnold Chiari Ⅰ畸形（Arnold Chiari Malformation type Ⅰ,ACM）合并脊髓空洞（Syringomyelia,SM）的外科治疗方法.方法 对2000～2006年36例ACM合并SM的患者采用后颅窝减压、成形术,并根据不同的临床特点予以空洞切开引流、下疝小脑扁桃体切除、颈枕交界区粘连分离以及脊髓中央管口松解术.结果 术后随访1年以上,症状明显缓解28例,稳定无变化7例,1例疼痛加重并出现对侧上肢麻木;MRI检查示2例脊髓空洞消失,26例空洞明显缩小,8例空洞无明显变化.结论 缓解颅颈交界区的压迫和疏通脑脊液循环是手术成功的根本.     

130例儿童ChiariⅠ畸形的手术经验

目的 介绍关于儿童ChiariⅠ型畸形的手术经验及长期随访结果。方法 对130例有临床症状的ChiariⅠ型畸形的儿科病人进行了后颅窝减压术。病人年龄2个月～20岁，平均11岁。住院时间2～7d，平均2．7d。随访3个月～15年，平均4．2年。结果 最常见的症状是头颈部疼痛(38％)及脊柱侧弯(18％)。与本组病人相关的诊断包括神经纤维瘤病Ⅰ型(5．5％)、脑积水(11％)、先天性生长激素缺乏症(5．5％)、颈椎分节不全(5％)和颅底陷入症(4％)。58％的病人有脊髓空洞症，17％的病人有脑干及第四脑室下部异位。术后83％的病人症状得到缓解，12％的病人头颈部疼痛及17％的脊柱侧弯未缓解。术后并发症发生率2.3％，包括急性脑积水及脑干腹侧受压(该病人症状严重，行经口齿状突切除术后症状缓解)。9例病人因症状无改善或脊髓空洞继续增大而再次手术。在术中发现10例病人(7．7％)的蛛网膜膈阻塞了第四脑室正中孔，其中9例合并脊髓空洞症。在行后颅窝减压术及硬脑膜修补术治疗后，几乎所有病人的脊髓空洞缩小或不再扩大。结论 无论是否合并脊髓空洞症，对仅有轻微症状的ChiariⅠ型畸形病人早期进行手术治疗有着较好的预后。有必要在中线部位进行后颅窝减压，并打开硬膜探查，开放第四脑室正中孔以保证脑脊液的流出。根据长期随访，切除C1后弓后未出现颈椎不稳。用颅骨膜进行硬脑膜修补，避免了异体移植带来的风险，经过长期随访无并发症发生。     

不同手术方式治疗Chiari Ⅰ畸形合并脊髓空洞的临床研究

目的通过同顾性比较枕大孔区减压硬膜成形术及枕大孔区减压环枕筋膜松解术对Chiari Ⅰ畸形合并脊髓空洞的治疗,明确两种不同术式治疗ChiariⅠ畸形合并脊髓空洞的疗效. 方法2002年1月至2004年4月对收治的62例ChiariⅠ畸形合并空洞患者行枕大孔区减压,其中46例患者剪开硬脑膜行硬膜成形术（硬膜成形组）,16例患者未剪开硬膜仅做环枕筋膜松解（筋膜松解组）.结果两组患者无一例死亡,硬膜成形组患者术后1年临床改善39例（84.78%）,脊髓空洞缩小30例,筋膜松解组临床改善9例（56.25%）,x^2=5.528,P=0.019,脊髓空洞缩小7例. 结论枕大孔区减压硬膜成形术是治疗ChiariⅠ畸形合并脊髓空洞症较为合理的术式,疗效优于枕大孔区减压环枕筋膜松解术.     

Factors contributing improvement of syringomyelia and surgical outcome in type І Chiari malformation

Objective  The aim of our study was to compare pre- and postoperative radiologic data of posterior fossa and the improvement of syringomyelia after posterior fossa decompression (PFD) with and without tonsillar management in Chiari type І malformation (CM-I). Material and methods  A retrospective analysis was made of all patients who underwent PFD between Oct 1991 and March 2007 for CM-I. Fifty-seven patients treated for CM-I at a single institution were included in the study. Patients were divided into two groups according to the procedures used during their PFD: PFD vs. PFD with tonsillar management. To determine whether the tonsillar management or changes of posterior fossa volume relate with surgical outcome, we measure posterior fossa size and syringomyelia pre- and postoperatively using magnetic resonance imaging. Results  Forty patients (70.2%) received PFD and 17 patients (29.8%) received PFD with tonsillar management. The length of syringomyelia affected improvement of syringomyelia (alteration rate, A-rate). Clinical symptoms, craniectomy size, syringomyelia type, and the surgeon’s specialty did not affect A-rate. Tonsillar management has no significant effect on improvement of syringomyelia. Four patients need repeated surgery due to recurrence. Conclusion  We have shown that tonsillar management do not lead to improve A-rate, and the radiologic changes of posterior fossa volume do not relate with radiologic improvement of syringomyelia. PFD without tonsillar management is sufficient to improve syringomyelia. The longer syrinx, the more A-rate improve in our study. However, a wider craniectomy is unrelated to A-rate. In cases of recurrent patients, we obtained good results with tonsillar management or syringosubarachnoid shunt. An erratum to this article can be found at     

两种手术方式对Chiari畸形Ⅰ型合并脊髓空洞的系统评价及meta分析

目的系统评价后颅窝减压(posterior fossa decompression,PFD)和后颅窝减压加硬膜成形术(posterior fossa decompression plus duraplasty,PFDD)在Chiari畸形Ⅰ型合并脊髓空洞患者中的有效性及安全性。方法检索Embase、Cochrane、Pubmed、Ovid、Medline、Science Direct、谷歌学术、万方、知网等数据库自建库到2019年的文献,筛选文中对PFD与PFDD进行了比较的随机对照研究或非随机对照研究,统计术后临床症状和影像学改善情况、并发症等指标,运用Revman(5. 3版本)进行数据分析。结果最终纳入10篇文献,共3 188例,PFDD组1 383例,PFD组1 805例。在脊髓空洞改善率(OR:5. 53; 95%CI:2. 86,10. 69)、症状缓解率(OR:2. 53; 95%CI:1. 30,4. 91)、并发症发生率(OR:3. 46; 95%CI:1. 40,8. 59)、脑脊液漏发生率(OR:9. 36; 95%CI:2. 63,33. 34)、假性硬脑膜膨出率(OR:1. 89; 95%CI:1. 28,2. 79)方面PFDD组高于PFD组(P 0. 05)。在切口感染发生率(OR:1. 44; 95%CI:0. 57,3. 59)、皮下积液发生率(OR:1. 71; 95%CI:0. 50,5. 80)方面两种术式无差异(P 0. 05)。结论针对Chiari畸形Ⅰ型合并脊髓空洞的患者,PFDD的有效性优于PFD。     

两种手术方式对Chiari畸形Ⅰ型合并脊髓空洞的系统评价及meta分析

目的 系统评价后颅窝减压（posterior fossa decompression,PFD）和后颅窝减压加硬膜成形术（posterior fossa decompression plus duraplasty,PFDD）在Chiari畸形Ⅰ型合并脊髓空洞患者中的有效性及安全性。方法 检索Embase、Cochrane、Pubmed、Ovid、Medline、ScienceDirect、谷歌学术、万方、知网等数据库自建库到2019年的文献,筛选文中对PFD与PFDD进行了比较的随机对照研究或非随机对照研究,统计术后临床症状和影像学改善情况、并发症等指标,运用Revman（5.3版本）进行数据分析。结果 最终纳入10篇文献,共3 188例,PFDD组1 383例,PFD组1 805例。在脊髓空洞改善率（OR：5.53;95%CI：2.86,10.69）、症状缓解率（OR：2.53;95%CI：1.30,4.91）、并发症发生率（OR：3.46;95%CI：1.40,8.59）、脑脊液漏发生率（OR：9.36;95%CI：2.63,33.34）、假性硬脑膜膨出率（OR：1.89;95%CI：1.28,2.79）方面PFDD组高于PFD组（P<0.05）。在切口感染发生率（OR：1.44;95%CI：0.57,3.59）、皮下积液发生率（OR：1.71;95%CI：0.50,5.80）方面两种术式无差异（P>0.05）。结论 针对Chiari畸形Ⅰ型合并脊髓空洞的患者,PFDD的有效性优于PFD。     

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.     

Suboccipital craniotomy in the surgical treatment of Chiari I malformation

Object  The object of this study was to present craniotomy for Chiari type I patients. Materials and methods  Six patients with Chiari type I underwent suboccipital craniotomy. All patients showed clinical improvement, and none had any complications. Two patients had syringomyelia; it disappeared in entirety. We describe the procedure for posterior fossa decompression. Results  Three-dimensional volumetric analysis using Vitrea workstation for postoperative posterior fossa volumes was calculated and was seen to have been increased on an average, from pre-operative (168 cc) to postoperative volume (192 cc). Conclusion  We thus conclude that suboccipital craniotomy results in resolution of the Chiari symptoms yet achieves effective expansion of posterior fossa.     

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.     

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.     

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.     

两种不同术式治疗Chiari畸形合并脊髓空洞症的疗效分析

目的 通过回顾性比较后颅窝成形术及枕大池成形术对Chiari畸形合并脊髓空洞症的治疗,明确两种不同术式治疗Chiari畸形合并脊髓空洞的疗效.方法 对收治的85例Chiari畸形合并空洞患者,其中39例患者行后颅窝成形术(后颅窝减压+硬脑膜成形);46例患者行枕大池成形术(后颅窝减压+硬脑膜成形+小脑扁桃体切除+蛛网膜粘连分解).结果 两组远期疗效相比有统计学意义,症状改善率分别为64%和90%,恶化19%和3%;术后脊髓空洞长度改变,两组比较有统计学意义.结论 枕大池成形术是治疗Chiari畸形合并脊髓空洞症较为合理的术式,疗效优于后颅窝成形术.