ChiariⅠ型畸形合并脊髓空洞症的外科治疗探讨

目的探讨颈枕区减压和后颅窝重建术治疗ChiariⅠ型(chiarimalformationtypeⅠ,CMⅠ)畸形合并脊髓空洞症(syringomyelia,SM)的疗效。方法对19982004年收治的69例患者,采取颈枕区减压和后颅窝重建术治疗。结果术后58例获得随访,症状改善47例(81%),稳定7例(12%),恶化4例(7%),无手术死亡;MRI复查脊髓空洞有不同程度缩小,部分枕大池解剖恢复。结论对CMⅠ合并SM患者,行颈枕区减压和后颅窝重建术,可以阻止病情发展,手术效果肯定,但要长期随访。     

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外科治疗

目的对Chiari畸形合并脊髓空洞症的手术治疗方法进行探讨,评价其治疗效果。方法对30例Chiari畸形病例(合并脊髓空洞症26例)根据其MRI影像特点,选择不同手术方式进行治疗:18例无或合并轻度脊髓空洞症者行后颅窝减压术,12例合并重度脊髓空洞症行后颅窝减压并脊髓空洞切开分流术,其中4例小脑扁桃体下疝超过C2水平行后颅窝减压并小脑扁桃体切除术。结果患者术后临床症状有不同程度改善。26例获随访1～4年,其中21例空洞明显缩小或消失,5例无明显变化;17例临床症状改善。结论对Chiari畸形合并脊髓空洞症患者依据其影像学特点采取不同的手术方式,疗效满意,手术能使颅颈交界处充分减压,改善临床症状,或控制病情进展。     

颅后窝重建术治疗Chiari I畸形合并脊髓空洞症

目的探讨后颅窝重建术(后颅窝减压+硬脑膜成形+小脑扁桃体切除+蛛网膜粘连分解)治疗Chiari I畸形合并脊髓空洞症的方法及疗效。方法 2002年11月至2008年1月对收治的46例Chiari I畸形合并脊髓空洞症患者行后颅窝重建术。结果后颅窝重建术症状改善率84.8%,脊髓空洞长度明显减小,KPS评分显著改善。结论后颅窝重建术是治疗Chiari I畸形合并脊髓空洞症较为合理的术式。     

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。     

自体骨瓣后颅窝扩大成形术治疗Chiari畸形合并脊髓空洞症

目的 报告利用自体骨瓣进行后颅窝扩大成形术治疗Chiari畸形合并脊髓空洞症的手术方法，并对临床效果进行讨论。方法 对8例Chiari畸形合并脊髓空洞症病人取自体骨瓣实施后颅窝扩大成形术。结果 随访6～18个月，平均13个月，病人术后临床症状、体征均改善，复查MRI示扩大成形的后颅窝容积稳定，成形良好，枕大池重建，脊髓空洞消失或不同程度缩小，后颅窝容积有效扩大10．13～19．45ml，平均14．54ml。结论 利用自体骨瓣行后颅窝扩大成形术可有效治疗Chiari畸形及其合并的脊髓空洞症，临床效果满意。     

Arnold-Chiari畸形Ⅰ型并脊髓空洞症的显微外科治疗

目的 探讨Arnold-Chiari畸形Ⅰ型并脊髓空洞症的手术方式及术后疗效. 方法 泸州医学院附属医院神经外科自1997年1月至2010年6月采用不同手术方式治疗Arnold-Chiari畸形Ⅰ型并脊髓空洞症患者185例,回顾性分析其临床资料,总结手术方式及疗效. 结果 出院时患者症状消失或改善156例,无变化29例,无症状恶化和死亡病例.随访147例患者,症状消失或改善110例,症状无变化26例,症状恶化11例;95例行MRI复查,其中枕大池解剖成形者82例,脊髓空洞明显缩小79例. 结论 后颅窝减压+硬膜成形术、小脑扁桃体切除+枕大池成形术是治疗Arnold-Chiari畸形Ⅰ型并脊髓空洞症有效、合理的手术方式.     

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。     

Chiari畸形合并脊髓空洞的手术治疗

目的探讨Chiari畸形合并脊髓空洞的外科治疗方法及效果。方法对36例经MRI证实了的Chiari畸形合并脊髓空洞患者采用后颅窝减压术、小脑扁桃体切除术、枕大池成形术、脊髓空洞穿刺术、经口齿状突磨除术等综合手术治疗。结果36例Chiari畸形合并脊髓空洞者中，术后症状明显改善者29例，轻度改善者4例，无变化者3例。结论针对不同的Chiari畸形合并脊髓空洞患者，采用不同的手术方式，可获得良好的治疗效果。     

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。     

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 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。     

Surgical experience of syringomyelia with reference to the findings of magnetic resonance imaging.

We present our surgical experience of 20 patients with syringomyelia, who were divided into two groups based on the findings of magnetic resonance (MR) imaging: a "non-visible cisterna magna" group, in which MR imaging did not reveal cerebrospinal fluid (CSF) in the cisterna magna, and a "visible cisterna magna" group. Patients with non-visible cisterna magna were associated with Chiari malformation (14 patients) or tight cisterna magna (4 patients) and underwent craniocervical decompression. Intradural exploration was performed when CSF movement in the cisterna magna or CSF outflow from the fourth ventricle appeared to be insufficient. It is important to confirm CSF outflow from the foramen of Magendie. Patients with visible cisterna magna were associated with tuberculous meningitis (2 patients) and underwent shunting procedures. Postoperatively, improvement in symptoms and a reduction in syrinx size were demonstrated in all patients except one. Two patients experienced recurrence of symptoms and syrinx dilatation.     

Chiari畸形的临床诊断和显微手术治疗体会

目的 探讨Chiari畸形的诊断及显微外科处理方法.方法 回顾性分析50例Chiari畸形的临床资料,其中合并脊髓空洞30例,齿状突型颅底陷入3例.结果 50例术后平均随访35个月,按Tator疗效标准:优36例(72%);良12例(24%);差2例(4%).有效率96%.空洞缩小21例,消失7例.结论 有限的后颅窝减压及枕大池重建是治疗Chiari畸形的有效方法;合并齿状突型颅底陷入应先行口咽入路齿状突磨除术或许为较好的选择.

Abstract：

Objective To study the diagnosis and the microsurgical treatment for Chiari malformation(CM).Methods The clinical data of 50 cases of Chiari malformation were analyzed retrospectively,30 cases were combinated with syringomyelia,3 with cranial basal invagination referred to dens of axis.Results 50 cases were followed up for an average 35 months.According to Tator's standard,the curative effect was excellent in 36,good in 12 and bad in 2 patients.The effective rate was 96%.The cavities in spinal cords reduced in 21 cases,disappeared in 7.Conclusion Limited posterior fossa decompression with reconstruction of cistern magna is a effective method for treatment of CM;CM with cranial basilar invagination referred to dens of axis treated firefly by anterior decompression of odontoidectomy via oral approach may be an optimal selection.     

Spontaneous resolution of syringomyelia in an adult patient with tight cisterna magna

Spontaneous resolution of syringomyelia in adult patients with Chiari malformation is exceptionally rare, with only 10 cases having been reported. A 21-year-old man working as a carpenter presented with a 1-year history of paresthesias in his right arm. A magnetic resonance imaging scan disclosed a cervicothoracic syrinx associated with tight tonsillar impaction of the cisterna magna without herniation. The patient left the carpentry job and underwent close monitoring with serial clinical and neuroradiological controls. The patient’s symptoms gradually disappeared and magnetic resonance imaging studies revealed progressive shrinkage of the syrinx despite persistence of crowding of posterior fossa structures at the level of the foramen magnum. This case suggests that spontaneous resolution of syringomyelia can occasionally be triggered by the cessation of daily physical strain in patients with tight cisterna magna. Health care professionals should be aware that strenuous physical activities could affect the natural history of syringomyelia.     

微创枕大池重建术辅助术中B超评价ChiariI型畸形合并脊髓空洞症临床疗效

目的探讨微创枕大池重建术治疗ChiariI型畸形术中辅助B超判断枕大池重建效果的可行性。方法选择2008年1—12月诊断明确的ChiariI型畸形合并脊髓空洞症患者共93例,施行微创枕大池重建术。术中保留寰椎后弓,并于切开硬膜前通过B超实时观察小脑扁桃体下疝形态、大小、“活塞”样往复运动状态,以及枕大池形态和大小;并于切除部分小脑扁桃体后对枕大池重建效果进行判断。分别于术后6和30个月时通过Tator法进行临床疗效评价。结果所有患者均顺利完成手术,术后并发症包括小脑出血（1例）、小脑梗死（1例）、脑积水（1例）、皮下积液（2例）,均经对症治疗痊愈出院。术后6个月随访93例患者,临床症状好转36例、稳定55例、加重2例,MRI显示脊髓空洞缩小或完全消失90例、无变化3例;术后30个月随访80例患者,原症状稳定者中12例好转、1例加重,MRI显示脊髓空洞无进一步变化。结论微创枕大池重建术治疗ChiariI型畸形损伤小、术后恢复迅速、效果稳定、并发症少、安全性高。术中通过B超判断枕大池重建效果简单易行,数据可靠。     

Revision of Chiari decompression for patients with recurrent syrinx

The management of adult patients with Chiari malformation associated with syrinx remains controversial. Although an abundance of literature exists for the pediatric population, there is an absence of guidelines for the adult population. It is unclear which of the different surgical approaches is appropriate in patients with Chiari I malformations and syringomyelia. A 36-year-old female patient had a posterior fossa decompression 3 years prior to recurrence. The patient developed recurrent symptoms with sensory loss and hyperesthesia in the right upper extremity. MRI revealed decreased cerebrospinal fluid flow at the craniocervical junction. The patient was taken to the operating room for revision of the posterior fossa decompression, lysis of adhesions and duraplasty. Re-exploration of a Chiari decompression, lysis of adhesions and revision duraplasty is an effective treatment option for recurrent syringomyelia.     

Syringomyelia in twin brothers discordant for Chiari I malformation: case report

Familial syringomyelia outside of trauma, tumor, or infection has been reported. Cases are presented that highlight the possible connection between familial syringomyelia and the Chiari 0 malformation. We report on 11-year-old twin brothers both with syringomyelia. Magnetic resonance imaging further revealed that one brother had Chiari I malformation and the other had Chiari 0 malformation. Both underwent posterior fossa decompression with radiologic improvement of their syringes. These case reports lend credence to earlier reports of improvement in syringomyelia following posterior fossa decompression in the absence of Chiari I malformation, the so-called Chiari 0 malformation. In addition, these case reports should influence the manner in which familial syringomyelia without tonsillar ectopia is defined and addressed, that is, posterior fossa decompression versus shunting of the syrinx, thereby addressing the potential cause of the syringomyelia and not only the enlarged cavity itself.