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。     

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.     

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。     

Chiari畸形Ⅰ型并脊髓空洞的显微外科治疗

目的总结采用显微外科技术治疗Chiari畸形Ⅰ型并脊髓空洞的手术技术改进。方法回顾性分析采用显微外科技术治疗Chiari畸形Ⅰ型并脊髓空洞436例的临床资料,10年间对手术步骤进行部分改进,手术方式逐渐由外减压到内减压。采用远期生活质量评估(KPS)和日常生活能力量表评分(ADL)评价疗效,并通过影像学检查对脊髓空洞的变化情况进行评价。结果术后感觉障碍明显改善371例;运动障碍改善312例,加重4例;无死亡病例。术后并发症:后枕部头痛30例,脑神经损伤2例,硬脑膜外囊肿37例,脑脊液漏40例,颅内感染51例,切口脂肪液化45例。170例经头颈部MRI检查显示:脊髓空洞均缩小。随访120例,时间0.5～6.5年,平均2年。末次随访KPS和ADL分值与术前相比,均显著升高(均P0.01);脊髓空洞缩小及消失率为83.4%。结论颅后窝减压术技术的改进对Chiari畸形Ⅰ型并脊髓空洞的治疗安全、有效。     

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。     

手术治疗ChiariⅠ型畸形的效果分析

目的比较单纯后颅窝减压术和后颅窝减压+硬膜成形+小脑扁桃体切除术治疗ChiariⅠ型畸形的临床效果。方法选择我科2008-08—2015-09收治的67例ChiariⅠ型畸形患者进行回顾性分析,其中行单纯后颅窝减压术治疗组27例,后颅窝减压+硬膜成形+小脑扁桃体切除术治疗组40例。结果术后随访12~48个月,单纯后颅窝减压组和后颅窝减压+硬膜成形+小脑扁桃体切除组在头痛及颈肩部不适的改善率分别为81.25%、76.00%(P0.05);肢体感觉障碍的好转率分别为33.33%、65.71%,差异有统计学意义(P0.05);脊髓空洞缩小率分别为33.33%、67.86%,差异有统计学意义(P0.05)。2组并发症发生率比较无显著差异(P0.05)。结论后颅窝减压+硬膜成形+小脑扁桃体切除组总体效果较显著,但在缓解头痛及颈肩部不适方面与单纯后颅窝减压术无明显差异,应根据临床特点选择个体化治疗方案。     

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.     

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:     

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。     

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。     

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     

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.     

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.     

Dural band pathology in syringomyelia with Chiari type I malformation

Surgical material taken from the ‘outer layer’ of thickened dura mater (dural band) at the craniovertebral junction of eight cases of syringomyelia with Chiari type I malformation was histologically examined in comparison with four autopsy cases as controls. The dural band was thickened and there were increased numbers of collagen fibers which showed fiber splitting, hyalinous nodule, calcification and/or ossification. These changes were not observed in the four control cases. Thus, it is suggested that the thickening of the dura mater may be a causative factor of syringomyelia with Chiari type I malformation. In addition, the histology of the thickened dura mater suggests the condition may be a consequence of birth injury in these patients.     

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。