前屈-后伸位MRI对判断颅脊交界区畸形内固定指征的临床价值

目的分析前屈-后伸位MRI对诊断Arnold—Chiari畸形可能合并寰枢椎脱位的作用,以及指导治疗的临床意义。方法回顾分析40例Arnold—Chiari畸形患者的前屈-后伸位MRI影像学资料,测量寰齿间距,通过前屈位和后伸位颈椎椎管狭窄程度分级,判断颅脊交界区稳定性。单纯Arnold．Chiari畸形患者采用枕大孔减压和枕大池扩大成形术,存在寰枢椎脱位者兼行枕颈内固定融合术。结果经前屈．后伸位MRI检查,证实有12例患者存在颅颈失稳,于枕大孔减压的同时行枕颈内固定融合术。手术后第3天颈椎影像学检查,40例中10例脊髓空洞病灶明显缩小;12例兼行枕颈内固定融合术者颅颈复位满意,脊髓压迫解除;手术后3个月随访,脊髓空洞病灶明显缩小（17例）,颅脊交界区骨痂形成、骨融合效果良好、颅颈复位无丢失（12例）,日本矫形外科评分13．08±1．40,与手术前评分（11．08±1．61）比较,差异有统计学意义（t=5．928,P=0．000）。结论前屈．后伸位MRI对判断颅脊交界区稳定性、选择适宜的手术方式具有重要意义。     

颈枕融合术治疗复杂颅颈交界区畸形的疗效分析

目的 探讨颈枕融合术治疗复杂颅颈交界区畸形的疗效。方法 回顾性分析2012年2月至2018年2月武汉大学人民医院神经外科行枕颈融合术治疗的23例复杂颅颈交界区畸形的临床资料。3例行经口齿状突切除+后路枕颈固定融合术,20例行寰枢椎复位+后颅窝减压+后路枕颈固定融合术。结果 术后2周齿状突与钱氏线距离、日本骨科协会评分、延髓-脊髓角、寰齿间距较术前均明显改善（P<0.05）。23例术后随访半年至5年,均未出现关节松动,内固定及植骨均较为牢靠;复查头颈部MRI均示脊髓压迫明显减轻,寰枕关节复位良好,内固定固定良好。结论 颈枕融合术治疗复杂颅颈交界区畸形是一种安全且有效的方式。     

枕颈内固定与植骨融合治疗先天性复杂寰枕畸形

目的 探讨枕颈内固定并植骨融合治疗先天性复杂寰枕畸形的疗效.方法 回顾性分析21例复杂寰枕畸形患者,其中17例行后颅窝减压+枕颈植骨固定融合术,4例行经口咽寰枢椎间软组织及骨性组织切除减压,齿状突复位减压+后路后颅窝枕骨大孔减压术+枕颈植骨固定融合术;术后随访3个月至3年.结果 寰枢脱位并存在延颈髓腹、背侧受压者,选择经口咽寰枢椎间软组织及骨性组织切除减压齿状突复位减压+后路后颅窝枕骨大孔减压术+枕颈植骨固定融合术,疗效满意.硬脑膜扩大修补与否及小脑扁桃体切除术与否与最终远期疗效无关;伴有多节段脊髓空洞症者行脊髓空洞“T”管蛛网膜下腔引流.本组治愈14例,有效7例.结论 针对Chiari畸形解剖变异大的特点以及合并症形成的机制,选择不同的手术方案,提高手术治疗的远期疗效.     

Chiari畸形术后寰枕交界区稳定性的临床观察

目的 观察Chiari畸形术后寰枕交界区的稳定性。方法 2006~2010年对11例Chiari畸形进行小骨窗寰枕部减压术,术后对寰枕交界区稳定性进行6~10年的随诊观察。结果 11例中,10例（90.91%）寰枕交界区稳定;原有症状均有不同程度的改善,感觉障碍程度减轻,肢体活动有所增强,半年以后,均能从事正常的生产、生活。外伤性寰枢椎脱位齿状突后突合并扁桃体下疝的1例,1年后跌倒再受伤,症状加重,复查MRI见延髓脊髓交界区前方和后方压迫严重,矢状位上测量脑干-颈髓角为115°。术前合并有脊髓空洞症的7例中,术后6例明显缩小;合并脑积水的2例术后积水明显减轻。结论 ①单纯小脑扁桃体下疝畸形行小骨窗后颅窝骨性减压+寰枕筋膜松解术,没有行内固定,术后长期随访,寰枕交界区稳定。②小脑扁桃体下疝合并寰枢椎脱位行小骨窗后颅窝骨性减压+寰枕筋膜松解术,术后长期随访,寰枕交界区并不稳定,需要内固定。     

后路复位内固定并颅后窝小骨窗减压治疗合并颅底凹陷、寰枢椎脱位和脊髓空洞症的ChiariⅠ型畸形

研究背景ChiariⅠ型畸形为颅颈交界区软组织畸形,可合并其他复杂骨性畸形如寰枢椎脱位、颅底凹陷、扁平颅底及寰枕融合等.对这些复杂畸形,目前尚无成熟的治疗方法.本文探讨颅后窝小骨窗减压并Ⅰ期经后路复位内固定术治疗合并颅底凹陷、寰枢椎脱位和脊髓空洞症的ChiariⅠ型畸形的临床疗效.方法 回顾分析2004 年7 月-2011 年9 月治疗的寰枢椎脱位和颅底凹陷患者临床资料,分别采用日本骨科协会(JOA)17 分评分系统和MRI 影像学数据评价颈脊髓功能和脊髓空洞改善程度.结果 根据纳入标准,共筛选14 例符合入组条件的患者,男性4 例,女性10 例;平均年龄为(31.86 ±11.36)岁.术前JOA 评分平均为13.07 ± 1.59,术后增加至15.57 ± 1.02,二者比较差异具有统计学意义(t = 9.946,P = 0.000);术前脊髓空洞大小平均为(7.05 ± 1.98)mm,术后缩小至(2.21 ± 1.91)mm,手术前后比较差异亦有统计学意义(t = 7.271,P = 0.000).手术后无一例发生并发症或死亡.结论 经后路复位内固定联合颅后窝小骨窗减压术治疗合并脊髓空洞症、寰枢椎脱位及颅底凹陷的ChiariⅠ型畸形能够显著改善患者预后、缩小脊髓空洞.     

经口咽前路寰枢椎复位钢板置入内固定治疗颅颈交界部畸形的MRI评价

背景：颅颈交界部畸形经口咽前路寰枢椎复位钢板置入内固定治疗过程中,X射线、CT对于颅颈交界部病变的检查只能显示其骨性结构,无法显示脊髓情况。 目的：分析颅颈交界部畸形经口咽前路寰枢椎复位钢板内固定前后的磁共振图像,探讨MRI成像对其内固定术式的选择及效果判断的价值。 方法：选择2009-06/2010-04收治的颅颈交界部畸形患者25例,男9例,女16例,术前均行X射线、CT、MRI检查,采用经口咽前路寰枢椎复位钢板置入内固定治疗方式,置入后6个月内行MRI复查。 结果与结论：所有患者均顺利完成内固定,未出现脊髓血管损伤等严重并发症。25例患者置入后MRI成像显示内固定在位,寰枢椎复位,脊髓受压情况改善,11例随访患者中,原上颈椎局部疼痛、活动受限等情况有9例明显改善或消失。提示MRI能够多方位清晰显示颅颈交界部畸形情况,对脊髓及颅内病变的显示更直接,可为颅颈交界部畸形经口咽前路寰枢椎复位钢板置入内固定手术方式的选择、脊髓受压情况及手术效果的判断、术后恢复提供客观依据。     

颅颈交界后路内固定技术进展

颅颈交界区的后路内固定术通常指寰枢椎内固定术和枕颈内固定术,主要应用于先天畸形、外伤、炎症以及肿瘤破坏等各种原因导致的颅颈交界区失稳。寰枢椎脱位是颅颈交界区失稳的关键病理变化,因此各种内固定技术主要针对寰枢椎加以固定和植骨,即寰枢椎内固定术。有时寰椎或枢椎不     

合并寰枢椎脱位的复杂颅颈交界区畸形后路减压复位内固定术疗效初步观察

目的 总结合并寰枢椎脱位的复杂颅颈交界区畸形经后路减压复位内固定术的临床经验.方法 回顾分析18 例合并寰枢椎脱位的复杂颅颈交界区畸形患者(先天性寰枢椎脱位15 例、经口腔入路齿状突磨除术后症状加重致枕颈失稳1 例、外伤所致2 例)的临床资料.施行经后路减压复位钉棒内固定术,术中行体感诱发电位及肌电图监测,根据日本骨科协会(JOA)17 分评分系统和影像学改善程度评价手术疗效.结果 术后影像学检查显示,18 例中16 例钉棒内固定系统和寰枢椎复位良好,1 例复位不良;骨性融合良好16 例,欠佳1 例.术后临床表现均不同程度好转,1 例突发呼吸骤停死亡.术后平均随访6.62 个月(3 ~ 28 个月),JOA 平均评分为11.62 ± 3.23,与手术前评分(7.51 ± 3.82)相比,差异具有统计学意义(t = - 5.476,P = 0.004).结论 经后路减压、复位、钉棒内固定术治疗合并寰枢椎脱位的颅颈交界区畸形临床疗效良好,能够减少患者痛苦、避免再次手术,值得临床推广应用.     

后路复位内固定并颅后窝小骨窗减压治疗合并颅底凹陷、寰枢椎脱位和脊髓空洞症的ChiariI型畸形

研究背景ChiariI型畸形为颅颈交界区软组织畸形,可合并其他复杂骨性畸形如寰枢椎脱位、颅底凹陷、扁平颅底及寰枕融合等。对这些复杂畸形,目前尚无成熟的治疗方法。本文探讨颅后窝小骨窗减压并I期经后路复位内固定术治疗合并颅底凹陷、寰枢椎脱位和脊髓空洞症的ChiariI型畸形的临床疗效。方法回顾分析2004年7月-2011年9月治疗的寰枢椎脱位和颅底凹陷患者临床资料,分别采用日本骨科协会（JOA）17分评分系统和MRI影像学数据评价颈脊髓功能和脊髓空洞改善程度。结果根据纳入标准,共筛选14例符合入组条件的患者,男性4例,女性10例;平均年龄为（31．86±11．36）岁。术前JOA评分平均为13．07±1．59,术后增加至15．57±1．02,二者比较差异具有统计学意义（t=9．946,P=0．000）;术前脊髓空洞大小平均为（7．05±1．98）mm,术后缩小至（2．21±1．91）mm,手术前后比较差异亦有统计学意义（t=7．271,P=0．000）。手术后无一例发生并发症或死亡。结论经后路复位内固定联合颅后窝小骨窗减压术治疗合并脊髓空洞症、寰枢椎脱位及颅底凹陷的ChiariI型畸形能够显著改善患者预后、缩小脊髓空洞。     

颅颈交界区畸形

颅颈交界区(CVJ)畸形的发生发展既有先天胚胎发育因素,又有后天病理生理学及生物力学改变的影响。在许多情况下,多种畸形同时存在,错综复杂,如颅底凹陷(BI)、扁平颅底(platybasia)、寰枕融合(AOA)、寰枢椎脱位(AAD,失稳)等,有时还可同时合并小脑扁桃体下疝、脊髓空洞症(SM)等,给疾病的诊断、鉴别诊断带来困难,误诊误治现象时有发生。     

Surgical Treatment of Craniovertebral Junction Instability : Clinical Outcomes and Effectiveness in Personal Experience

Objective

Craniovertebral junction (CVJ) consists of the occipital bone that surrounds the foramen magnum, the atlas and the axis vertebrae. The mortality and morbidity is high for irreducible CVJ lesion with cervico-medullary compression. In a clinical retrospective study, the authors reviewed clinical and radiographic results of occipitocervical fusion using a various methods in 32 patients with CVJ instability.

Methods

Thirty-two CVJ lesions (18 male and 14 female) were treated in our department for 12 years. Instability resulted from trauma (14 cases), rheumatoid arthritis (8 cases), assimilation of atlas (4 cases), tumor (2 cases), basilar invagination (2 cases) and miscellaneous (2 cases). Thirty-two patients were internally fixed with 7 anterior and posterior decompression with occipitocervical fusion, 15 posterior decompression and occipitocervical fusion with wire-rod, 5 C1-2 transarticular screw fixation, and 5 C1 lateral mass-C2 transpedicular screw. Outcome (mean follow-up period, 38 months) was based on clinical and radiographic review. The clinical outcome was assessed by Japanese Orthopedic Association (JOA) score.

Results

Nine neurologically intact patients remained same after surgery. Among 23 patients with cervical myelopathy, clinical improvement was noted in 18 cases (78.3%). One patient died 2 months after the surgery because of pneumonia and sepsis. Fusion was achieved in 27 patients (93%) at last follow-up. No patient developed evidence of new, recurrent, or progressive instability.

Conclusion

The authors conclude that early occipitocervical fusion to be recommended in case of reducible CVJ lesion and the appropriate decompression and occipitocervical fusion are recommended in case of irreducible craniovertebral junction lesion.     

Arnod-Chiari畸形合并颅底成角畸形的外科治疗

目的探讨Arnod-Chiari畸形合并无寰枢椎脱位的颅底成角畸形的治疗方法。方法30例Arnod-Chiari畸形患者合并颅底成角畸形但无完全寰枢椎脱位,MR显示脑干与颈脊髓腹侧明显受压,测量其颅底角小于120°,行枕下减压手术治疗同时纠正颅底角角度后内固定。结果30例患者术后半年复查MR均有人工枕大池的形成,脊髓空洞均缩小,脑干与颈脊髓之间夹角较术前明显增大,植骨已融合,颅底角大于130°。术后患者症状均得到明显改善。结论对Arnod-Chiari畸形合并颅底成角畸形患者进行枕下减压手术同时纠正颅底角角度并固定,可以解决脑干与颈脊髓腹侧受压问题,能达到缓解临床症状或阻止临床症状加重的目的。     

Surgical results of arachnoid-preserving posterior fossa decompression for Chiari I malformation with associated syringomyelia

We analyzed the outcome of posterior fossa decompression accompanied by widening of the cisterna magna, without disturbing the arachnoid, in patients with Chiari I malformation (CMI) associated with syringomyelia. Twenty-five adult patients with CMI and syringomyelia, who underwent surgery between October 2000 and December 2008, were enrolled in this study. All patients underwent foramen magnum decompression with C1 decompression, with or without C2 decompression. Three surgeons performed a dura opening with duraplasty in 20 patients, and another surgeon excised the outer layer of the dura without duraplasty in five patients. Clinical and radiological assessments were performed preoperatively and during the follow-up period. After surgery, 20 (80%) patients achieved a significant improvement in their clinical symptoms. However, four patients (16%) achieved only a stable state, and one patient's symptoms worsened. Radiological analysis showed that 17 patients (68%) had a favorable result; that is, a total collapse, or a marked reduction, of the syrinx. Seven patients (28%) were stable in terms of syrinx size. However, the syrinx enlarged in one patient who had undergone excision of the outer dura. Twenty-four patients achieved a widened cisterna magna with ascent of the cerebellar tonsils into the posterior fossa and acquisition of a more rounded shape. Postoperative complications included a transient headache and vomiting in three patients and transient motor weakness in one patient. Two patients developed a superficial wound infection. This study shows that arachnoid-preserving posterior fossa decompression is a safe and effective treatment for patients with CMI with associated syringomyelia.     

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.     

Convergence nystagmus associated with Arnold-Chiari malformation

A case of convergence nystagmus associated with an Arnold-Chiari type I malformation is presented. The nystagmus appeared in the absence of fixation, was provoked during Valsalva's maneuver and neck flexion and extension, and attenuated on deep inspiration. Sagittal magnetic resonance images showed that the diameter of the cerebral aqueduct increased with the neck in full flexion and in full extension. Surgical foramen magnum decompression considerably reduced the nystagmus and abolished the postural variation of aqueduct diameter. It is postulated that this nystagmus was due to a combination of mechanical distortion and abnormal transmission of cerebrospinal fluid pressure to the aqueductal region.     

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。     

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。     

成人非创伤性慢性寰枕脱位的诊断与手术治疗(附二例报告)

目的 报告2例成人非创伤性慢性寰枕脱位病例,以提高其诊治水平.方法 1例为58岁女性,进行性四肢无力10年;t例为18岁男性,进行性四肢无力半年.头颅CT和MRI示寰枕后脱位,斜坡下部压迫延髓,伴小脑扁桃体下疝和脊髓空洞.手术包括:经口咽人路切除斜坡下部、颅骨牵引及二期经后路颅颈固定融合.结果 术后四肢肌力改善,延髓减压充分,小脑扁桃体复位,脊髓空洞缩小.结论 三维重建CT和MRI是诊断非创伤性寰枕脱位的好方法,前路减压联合后路颅颈固定融合对治疗非创伤性慢性寰枕脱位安全有效.     

Management of congenital atlanto-axial dislocation: some lessons learnt

Congenital atlantoaxial dislocation (AAD) has a high incidence in India. In these patients, even a minor trauma may precipitate severe morbidity. The management of mobile AAD consists of posterior stabilization. In fixed AAD, the offending compressive element is present anterior to the cervicomedullary junction, which should be generously removed by the transoral approach. In many of these patients, the assimilated posterior arch of atlas or the incurving posterior margin of the foramen magnum or associated Chiari I malformation also adds to the compromise of the canal diameter at the foramen magnum. Thus, in cases of fixed AAD, the hour glass appearance of the spinal canal at the craniovertebral junction should be converted into a funnel shaped appearance which is close to normal. Since these procedures require a generous removal of the osteoligamentous structures, posterior fusion should be done in all cases after decompressive surgery. In this review, the observations that emerged during the management of congenital AAD are presented with a special focus on the assessment scales modified to the Indian settings, hypermobile AAD, rotary C1-2 dislocation, and AAD associated with Chiari I malformation and syringomyelia.