分期前后方减压治疗合并颅底陷入的Chiari畸形

目的 合并颅底陷入的Chiari畸形患者常有前方脑干压迫,多数经前路减压即可使扁桃体上移,空洞缩小,但少数患者前路减压后Chiari畸形和脊髓空洞并不减轻,本文旨在探讨采用分期前后方减压治疗该类患者.方法 9例患者经口咽入路齿状突磨除,观察1-2个月后发现Chiari畸形和脊髓空洞未减轻,遂行后方枕大孔减压,小脑扁桃体切除,硬膜扩大修补,枕颈或C1～2固定融合术.随访24-60个月(平均32个月).结果 本组无死亡或严重并发症患者,所有患者症状改善,8例Chiari畸形和脊髓空洞改善,1例无变化.结论 合并颅底陷入的Chiari畸形患者应首先行经口咽入路齿状突磨除术,多数患者后方仅需固定即可,但有些患者颅底陷入症状缓解,而Chiari畸形依旧,需后方减压加固定手术.     

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。     

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.     

改良后颅窝减压术对Ⅰ型Chiari畸形后颅窝形态学、颅颈交界区稳定性的影响

目的 探讨改良后颅窝减压术治疗Ⅰ型Chiari畸形的临床疗效及对后颅窝形态学参数、颅颈交界区稳定性的影响。方法 106例Ⅰ型Chiari畸形患者根据手术方法不同分为改良手术组(行改良后颅窝减压术，53例)、传统手术组(行传统后颅窝减压术，53例)，比较两组手术一般评价指标(手术时间、术中出血量、住院时间、手术费用)、手术治疗效果[Karnofsky功能状态(KPS)评分、术后脊髓空洞变化、芝加哥Chiari畸形结局量表(CCOS)评分]、后颅窝形态学参数(延髓颈髓角、小脑扁桃体下疝长度、枕大池容积)，血清GFAP、S100β、BDNF、NGF蛋白水平，颅颈交界区失稳率和并发症。结果 改良手术组术中出血量和住院时间较传统手术组减少，术后6个月KPS评分、CCOS评分改善率高于传统手术组，术后6个月小脑扁桃体下疝长度低于传统手术组，枕大池容积高于传统手术组(P<0.05)。改良手术组术前、术后6个月GFAP、S100β、BDNF、NGF、颅颈交界区失稳率、并发症发生率与传统手术组比较(均P>0.05)。结论 改良后颅窝减压术治疗Ⅰ型Chiari畸形下疝复位效果较好，可减少术中出...     

术中超声在小脑扁桃体下疝畸形颅后窝减压术的应用

目的总结术中超声在小脑扁桃体下疝畸形后颅窝减压术中应用及其效果分析。方法回顾性分析22例小脑扁桃体下疝畸形病例资料,术中应用超声辅助进行颅后窝减压术,包括单纯骨性减压术6例,骨性减压并硬膜成形术13例,枕大池重建术3例。结果在术中超声辅助监测下,22例病人均实现脊髓背侧脑脊液流动速率≥3 cm/s。22例病人术后第1天自觉症状得到改善。术后出现颅内感染2例,皮下积液1例,经治疗后病情得以控制并好转。随访3年,22例病人临床症状均明显改善。16例术前含有脊髓空洞的病人,脊髓空洞均减小,其中1例脊髓空洞消失。结论小脑扁桃体下疝畸形根据术中超声可采取单纯骨性减压术、骨性减压并硬膜成形术与枕大池重建术,从而以最小手术风险获得最大收益。     

基于枕大池重建理念手术治疗成人Chiari畸形Ⅰ型的临床疗效

成人Chiari畸形Ⅰ型是最常见的颅颈交界区畸形,通常伴有枕大池的消失以重建枕大池为目的行减压手术,延颈髓直接减压的同时恢复了颅颈交界区的正常脑脊液循环,从而使脊髓空洞好转,临床症状缓解。本研究中53例成人Chiari畸形I型患者术前均经临床及影像学评估,基于枕大池重建理念选择性应用后颅窝减压并硬膜成形术(PFDD)或PFDD+小脑扁桃体部分切除/热凝术,术后疗效确切,并发症较少。     

枕下减压扩大硬膜修补治疗小脑扁桃体下疝

目的 探讨小脑扁桃体下疝畸形的手术治疗方法。方法 回顾性总结2000年至2006年32例枕下减压扩大硬膜修补术治疗小脑扁桃体下疝畸形效果。结果 32例小脑扁桃体下疝患者,症状明显改善23例,缓解7例,2例因呼吸衰竭死亡。7例脑积水消失,脊髓空洞12例随访3月～3年,消失5例,缩小7例。结论 枕下减压扩大硬膜修补术是治疗小脑扁桃体下疝畸形的简便有效方法,但应严格掌握适应证。     

微骨窗减压篷式硬脑膜重建在Chiari畸形手术的应用

目的探讨微骨窗减压并硬脑膜篷式重建在Chiari畸形中治疗的临床效果。方法回顾性分析经手术治疗的18例Chiari畸形Ⅰ型病人的临床资料,枕骨大孔区颅骨开窗直径2 cm,寰椎后弓切除1.5 cm,硬脑膜切开并篷式重建。结果术后无明显并发症发生,术前症状明显缓解。术后MRI示:小脑扁桃体上移复位,脊髓空洞明显萎缩。随访6~12个月,按照Tator标准,症状明显改善17例,部分改善1例。结论微骨窗减压并硬脑膜篷式重建术可达到充分减压,减少手术创伤,同时有效避免手术并发症的目的,手术效果满意。     

自体斜方肌筋膜后颅窝重建术治疗Chiari畸形伴脊髓空洞症的临床应用

目的探讨自体筋膜后颅窝重建术治疗Chiari畸形伴脊髓空洞症的手术方法。方法采取颅颈区减压、硬膜下探查松解、自体筋膜后颅窝重建术,而不行空洞切开引流。结果34例随访3个月至7.5年,症状改善29例(85.3%),稳定4例(11.8%),加重1例(2.9%),无死亡。MRI复查示,脊髓空洞均缩小,枕大池重现,小脑扁桃体及延髓上升。结论颅颈区减压及自体筋膜后颅窝重建术治疗Chiari畸形伴脊髓空洞症是首选术式,可获得较好疗效。     

Chiari畸形合并脊髓空洞症的手术治疗分析

目的　探讨枕下减压、小脑扁桃体切除、枕大池成型手术方法对Chiari畸形合并脊髓空洞症的治疗效果。方法　对91例Chian畸形合并脊髓空洞症经枕下正中入路手术。以枕骨大孔为中心,在其上和两侧2cm咬除枕骨鳞部、咬除寰椎后弓,“Y”字型剪开硬膜,电灼并软膜下吸除小脑扁桃体,探查正中孔。取硬膜替代材料修补。结果　91例术后症状均有改善,术后复查MR除3例外均见脊髓空洞症缩小。结论　颅颈交界处充分减压并探查正中孔能缓解临床症状,提高病人脊髓空洞症及脑积水的治愈率,小骨窗减压有利于防止术后并发症。     

后颅窝重建手术治疗Chiari畸形合并脊髓空洞症

目的探讨后颅窝重建这种手术方法对Chiari畸形合并脊髓空洞症的治疗及其效果.方法枕下正中入路,以枕大孔为中心,在其上和两侧2 cm左右范围内去除枕骨鳞部,咬除环椎后弓,剪开硬膜,如需要打开蛛网膜,应尽量保持软脑膜完整.取自体肌筋膜或硬脑膜替代材料扩大修补.结果 30例病人,术后症状均有不同程度的改善,术后3个月复查MRI见脊髓空洞缩小.结论手术使颅颈交界区充分减压,改善了脑脊液循环状态,有效地缓解了临床症状,并对脊髓空洞有明显的治疗作用.扩大修补硬脑膜有利于减少术后并发症的发生.     

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.     

彩色多谱勒超声评价ChiariⅠ畸形环枕减压硬膜成形前后脑脊液动力学变化

目的 通过彩色多谱勒超声对Chiari Ⅰ畸形(CMI)患者术中环枕骨性减压硬膜剪开前及硬膜成形术后枕大孔区脑脊液动力学进行研究,评价环枕骨性减压硬膜成形术对枕大孔区脑脊液循环恢复的影响.方法 对北京大学第三医院神经外科自2008年12月至2009年6月收治20例CMI患者(合并脊髓空洞17例)采用后正中入路枕大孔区减压术,取Neuropatch修剪成形修补硬膜.术后依据Tator评分、MRI评价患者疗效及脊髓空洞消减情况.在硬膜切开前及硬膜成形术后10～12 d对患者应用彩色多谱勒超声经枕部骨性减压窗分别于小脑扁桃体下缘水平脊髓腹、背侧蛛网膜下腔测量脑脊液的头向及尾向流动的流速.结果 术后15例患者Tator评分为优,余5例患者为良,无术后恶化者,术后改善率为75%.术后MRI示17例脊髓空洞患者中14例脊髓空洞有明显缩小(改善率82.4%),所有患者枕大池均重现.患者术后小脑扁桃体下缘水平脊髓背侧蛛网膜下腔均可见脑脊液的头向及尾向流动,与心动周期对应,并有呼吸节律,尾向流速为(8.92±5.19)cm/s,头向流速为(6.85±2.44)cm/s;术中环枕骨性减压后硬膜成形前11例可见脑脊液流动(55%),尾向流速为(5.68±3.25)cm/s,头向流速为(4.40±2.93)cm/s.结论 环枕骨性减压硬膜成形术是治疗CMI的有效方法,可有效恢复枕大孔区脑脊液的流动.彩色多谱勒超声检查是CMI患者术中及术后测量枕大孔区脑脊液流动的有效实用方法,可为术中决策提供客观依据.

Abstract：

Objective To evaluate the recovery of circulation of cerebral spinal fluid (CSF)around foramen magnum before and after duraplasty in patients with Chiari Ⅰ malformation (CMI) treated by foramen magnum decompression by using colored Doppler ultrasound (CDU). Methods Twenty patients suffered from CMI, including 17 combined with syringomyelia, admitted to our hospital from December 2008 to June 2009, were performed foramen magnum decompression via posterior midline approach; Neuropatch was used to repair the dura mater. Tarlov's grading scale and MRI was employed to evaluate the efficacy and the improvement of syringomyelia. All patients underwent CDU before and 10-20 d after the duraplasty to evaluate the circulation of CSF in arachnoid space below tonsil of cerebellar (TC). Results Fifteen patients (75%) got improvement and 5 without change after the surgery. Postoperative MRI indicated that 82.4% patients (14/17) combined with syringomyelia enjoyed good results. Cisterna magna reappeared after the surgery. CDU showed that bidirectional flow of CSF was recovered around foramen magnum in all patients with the Caudal-directed flow rate of CSF in dorsal arachnoid space below TC 8.92 ±5.19 cm/s and cephalad-directed flow rate of 6.85 ±2.44 cm./s; as compared with that after the surgery, the bidirectional flow could be found in only 11 patients before duraplasty (55%). Conclusion Foramen magnum decompression with duraplasty can restore the circulation of CSF around foramen magnum effectively in patients with CMI, and CDU, providing accurate data of circulation of CSF during or after the surgery, is a useful tool to evaluate the flow of CSF in patients with CMI.     

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.     

Chiari畸形并脊髓空洞症的MRI分型及其治疗

对Ｃｈｉａｒｉ畸形并脊髓空洞症治疗方法及其效果的探讨。方法对１０３例Ｃｈｉａｒｉ畸形病例根据其ＭＲＩ征像分为Ａ、Ｂ、Ｃ三种类型。结果依据其类型采用不同的手术方式,并获得了较为满意的治疗效果。结论手术使延颈髓充分减压,可有效缓解临床症状,并对脊髓空洞症有明显的治疗作用。     

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。     

脊柱侧凸为首发症状的Chiari畸形临床研究

目的 研究以脊柱侧凸为首发症状的Chiari畸形的临床特征和治疗策略.方法 收集南京鼓楼陕院住院治疗的Chiari畸形患者60例,35例以脊柱侧凸为首发症状;25例末合并脊柱侧凸作为对照组,病例对照研究.结果 35例Chiari畸形合并脊柱侧凸患者,年龄3～33岁(平均13.75岁),97%合并脊髓空洞,不具有典型的Chiari畸形的临床症状和体征,以腹壁反射异常多见.两组之间手术方式和手术并发症无统计学意义.结论 Chiari畸形以脊柱侧凸为首发症状,多合并脊髓空洞,发病年龄轻,其临床症状和神经损害体征不明显,治疗策略上主张早期进行枕颈部枕骨大孔区减压和硬脑膜重建成形手术.     

神经内镜下寰枕部减压手术治疗Chiari畸形

目的 探讨神经内镜下寰枕减压手术治疗Chiari畸形的手术方法和临床疗效.方法 对23例经MRI确诊为Chiari畸形但不伴有寰枢锥脱位和齿突凹陷的患者在神经内镜下进行了寰枕减压手术,并进行疗效观察.结果 23例术后随访0.5-3.0年,原有症状均有不同程度改善,感觉障碍程度减轻,肢体肌力有所增强.无手术后并发症.术前合并脊髓空洞症的16例患者中,术后7例空洞消失,8例范围缩小,1例范围无明显变化.结论 神经内镜下寰枕减压手术治疗Chiari畸形方法可行,手术安全、有效、创伤小、疗效满意.

Abstract：

Objective To investigate the methods and effectiveness to treat Chiari malformation with the surgical decompression of the foramen magnum under endoscope. Method 23 cases with Chiari malformation.(without atlanto- axialdislocation and basilar invagination) diagnosed by magnetic resonance imaging( MRI)were operated with the surgical decompression of the foramen magnum under the endoscope. Results 23 cases were followed- up 0. 5 -3. 0, years after operation, all the symptoms and signs were remarkably relieved after the operation. No complications was found. In 16 cases with spinal cavities,7 cases spinal cavities disappeared and scases deflated,l case no evident change. Conclusions The endoscope -assisted decompression of the foramen magnum is a safe and effective surgical method to treat Chairi malformation.     

Surgical decompression without dural opening for symptomatic Chiari type II malformation in young infants

Objective

Prompt surgical decompression for symptomatic Chiari type II malformation in neonates and young infants has been reported to be effective. However, the optimal operative method has been arguable. The authors present their experiences with surgical decompression without dural opening.

Methods

We retrospectively analyzed surgical results of patients with symptomatic Chiari type II malformation. The charts were reviewed for demographic data, clinical presentation, surgical procedures, and clinical outcomes.

Results

Ten consecutive patients with symptomatic Chiari type II malformation were surgically treated from August 2004 to December 2012. The mean age was 3.5 months. The median interval between onset of symptoms and surgery was 19.5 days. Three patients were treated by upper cervical bone decompression with dural opening and duraplasty. Seven patients were treated by upper cervical bone decompression with delamination of the outer layer of the dura. There were no perioperative complications. The mean follow-up period was 55 months (range 3–102 months). All 10 patients remain alive. In patients with dural opening, one patient showed complete recovery, and two patients had residual deficits and required a tracheostomy, one with ventilator support and the other with home oxygen therapy. In patients without dural opening, six patients (85 %) showed complete or nearly complete recovery, and one patient required tracheostomy and home oxygen therapy.

Conclusion

Upper cervical bone decompression with delamination of the outer layer of the dura can be a safe and effective treatment for symptomatic Chiari type II malformation in neonates and young infants.