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。     

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...     

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.     

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.     

后颅窝减压并植骨融合内固定术治疗复杂Chiari畸形

目的 探讨后颅窝减压并植骨融合内固定术治疗复杂Chiari畸形的效果。方法 2010年2月至2014年11月收治复杂Chiari 畸形17例,均采用后颅窝减压并植骨融合内固定术治疗,术中均行颅骨牵引+后路窝小骨窗减压植骨融合并内固定。结果 复位成功5例,未打开硬脊膜;复位不成功12例,行枕大池重建。术后出现颅内感染1例、切口感染伴脑脊液漏1例。术后5 d因呼吸衰竭死亡1例,其余16例术后随访3个月~3.5年,采用Tator等方法评价术后效果,16例均有效,有效率为94.1%（16/17）。结论 对复杂Chiari畸形,术前需明确诊断畸形的种类和性质,术中麻醉下牵引辅助治疗很有必要,后颅窝小骨窗减压并植骨融合并内固定是治疗复杂Chiari畸形的一种有效方法。     

小脑扁桃体下疝畸形合并脑室扩张的外科治疗

目的 探讨小脑扁桃体下疝畸形合并脑室扩张的手术方法及其效果。方法 回顾性分析20例小脑扁桃体下疝畸形合并脑室扩张病人的临床资料,全部病例均采用后正中入路小骨窗枕下减压加自体筋膜减张缝合术。结果 全部病例术后MRI显示延颈髓压迫解除、枕大池形态恢复;术后20例病人均获得随访,平均随访24个月,2例症状完全消失,12例症状明显改善,5例症状无明显改变,1例症状加重;20例病人脑室大小均未见明显改变,无脑室扩张加重病例。结论 脑室扩张是小脑扁桃体下疝畸形先天畸形的一部分,而不是由于枕大孔区梗阻造成的梗阻性脑积水;小脑扁桃体下疝畸形合并脑室扩张患者颅内压多正常,在行枕下减压术前一般不需要行脑室外引流术,后正中入路小骨窗枕下减压术可取得良好效果,术后未发现有脑室进行性扩大或颅内压增高的现象。     

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。     

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.     

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。     

小范围后颅窝减压治疗Chiari畸形合并脊髓空洞症（附97例报告）

目的评价以更小的创伤治疗Chiari畸形合并脊髓空洞症的临床疗效。方法小范围后颅窝骨性减压窗范围一般在3cm×3cm以内,不切除C1后弓,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。结果37例术后12d内MRI示下疝的小脑扁桃体下缘上升到枕骨大孔水平以上。长期随访48例,29例患者症状逐渐改善;14例症状稳定未加重;5例较术前加重,但MRI示脊髓空洞缩小。结论小范围后颅窝减压术能够改善Chiari畸形合并脊髓空洞患者的临床症状,可作为外科治疗Chiari畸形合并脊髓空洞症的一种术式。     

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畸形颅后窝减压及重建显微手术的临床研究

目的探讨Chiari畸形颅后窝减压及重建显微手术方法。方法回顾性分析70例Chiari畸形行显微手术的病例资料。均行颅后窝减压,皮质下切除下疝小脑扁桃体,人工硬膜无张力缝合,扩大骨瓣回置。结果术后症状消失或改善68例（97．1％）,无变化2例。59例随访1个月～3．5年,平均1年,53例症状消失或改善,4例病情稳定,2例神经功能恶化,有效率96．6％。54例合并脊髓空洞,空洞明显缩小或消失52例（96-3％）,无变化1例,空洞扩大l例。结论颅后窝减压及重建显微手术重点是解除枕大孔区压迫、重建脑脊液流体力学,是治疗Chi撕畸形有效方法。     

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。     

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.     

后颅窝扩大重建术治疗Chiari畸形合并脊髓空洞症

目的探讨后颅窝扩大重建术对Chiari畸形合并脊髓空洞症脊髓功能恢复的影响。方法选择56例Chiari畸形合并脊髓空洞症病人,显微镜下行后颅窝扩大重建术,定期随访,并与本科以往行后颅窝减压术的62例病人做疗效对比分析。结果后颅窝扩大重建术组的上肢、下肢、感觉障碍的有效率分别为75.7%、71.4%、70.0%,而后颅窝减压术组分别为58.5%、60.0%、65.9%。结论后颅窝扩大重建术是治疗Chiari畸形合并脊髓空洞症的有效方法,可作为外科治疗Chiari畸形合并脊髓空洞症的首选术式。     

Chiari畸形的外科治疗及相关问题探讨

目的 探讨Chiari 畸形的外科手术治疗和相关临床问题.方法 回顾分析35 例Chiari 畸形(26 例合并脊髓空洞)的外科治疗经验.全部病例均行后颅窝减压,C1 后弓减压,枕大池扩大成形,小脑扁桃体下疝切除术或电灼术.结果 全部病例随访6 个月～ 4 年,获得不同程度的临床症状改善,动态复查影像均获得良好的效果.结论 Chiari 畸形的外科治疗中根据临床症状和影像特点,个性化的选择行后颅窝大或小的骨窗减压,枕大池扩大成形和小脑扁桃体下疝切除手术治疗,是处理该种畸形获得临床疗效的好方法.     

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型手术治疗的临床观察

目的 探讨小切口、微骨窗寰枕减压术+寰枕筋膜松解术治疗Chiari畸形I型的临床疗效,并观察术后寰枕交界区稳定性。方法 分析2012年1月—2017年12月该院伴有头晕的Chiari畸形I型行小切口、微骨窗寰枕减压术+寰枕筋膜松解术治疗31例,依据芝加哥Chiari畸形预后量表（CCOS）评估疗效,术后对寰枕交界区稳定性进行3~6年的随诊观察。结果 所有病例均顺利完成手术,无手术死亡病例,无严重神经功能障碍等并发症。依据CCOS在术后1、3、6及12个月的重复评估,评估得出总平均分13.56分,预后良好24例（77.4%）,改善不明显5例（16.1%）,1例预后较差（3.2%）。随访观察3~6年,头晕缓解有效率为93.5%,30例寰枕交界区稳定,1例伴寰枢椎脱位病例1年后症状加重,需行内固定。结论 小切口、微骨窗寰枕减压术+寰枕筋膜松解术治疗Chiari畸形I型,手术安全、有效,且手术简单、并发症少,对伴有的头晕症状缓解率高。经长期随访寰枕交界区稳定。     

Posterior fossa dimension and volume estimates in pediatric patients with Chiari I malformations

Introduction Chiari I malformations (CMI) involve pathological hindbrain abnormalities reported to be correlated with a hypoplastic posterior fossa. CMI was traditionally characterized by the downward herniation of the cerebellar tonsils with a descent of 5 mm or more below the foramen magnum. The fullness of the cisterna magna and CSF flow at the level of the cervicomedullary junction have been shown to be more useful in selecting symptomatic patients for surgical decompression. The present study calculates posterior fossa dimension and volume estimates in pediatric patients using magnetic resonance imaging. The combination of neuroradiological and clinical findings is used to re-examine the criteria used for diagnosis and treatment of pediatric CMI patients. Materials and methods A retrospective chart review was conducted on patients who were admitted to the Division of Neurosurgery of the Children’s hospital of Eastern Ontario between 1990 and 2007. Clinical and radiological assessments were performed on all patients. Posterior fossa volumes (PFV) and intracranial volumes (ICV) were measured from sagittal head magnetic resonance imaging scans using the Cavalieri method. Results Sixty-one CMI patients were identified. There were 32 male and 29 female patients with a mean age of 10 years (range: 8 weeks–18 years). Thirty-four (55%) of these patients were symptomatic with scoliosis (38%), suboccipital headaches (29%), and motor/sensory deficits (26%) being the most prominent symptoms. The mean PFV/ICV ratio for all the CMI patients (0.110) was found to be statistically smaller than that of the control patients (0.127, p = 0.022). Mean PFV/ICV ratios for asymptomatic and symptomatic CMI patients were found to be similar for children aged 0–9 years (p = 0.783) but different for children aged 10–18 years (p = 0.018). Discussion Mean PFV values were found to be smaller in pediatric CMI patients than control patients; this complements earlier studies in adults and supports the present theory concerning the pathophysiological mechanism of CMI. Subtle morphometric differences among asymptomatic and symptomatic patients aged 0–9 years stress the importance of monitoring asymptomatic patients for the onset of symptoms in their adult years. Symptom development in CMI is likely multifactorial and is much more extensive than the degree of cerebellar tonsillar herniation.     

Chiari Ⅰ畸形的外科治疗

目的探讨ChiariⅠ畸形的外科治疗方法。方法采用后颅窝减压,扩大重建术治疗6例；后颅窝减压,扩大成形术加脊髓空洞分流术治疗10例。结果所有患者症状和体征均明显改善,无死亡和其他并发症。结论后颅窝减压,扩大成形术和(或)脊髓空洞分流术是治疗ChiariⅠ畸形的有效方法。