脊髓中央管隔膜形成与Chiari畸形并脊髓空洞

目的探讨显微外科治疗Chiari畸形并脊髓空洞的手术方法.方法 127例Chiari畸形并脊髓空洞经MRI确诊,采用小脑扁桃体下疝切除,并脊髓中央管开口隔膜切开术. 结果术中发现脊髓中央管开口隔膜形成;术后随访89例,MRI检查示,所有患者下疝的小脑扁桃体均消失,并存脊髓空洞明显缩小和消失.结论小脑扁桃体下疝,脊髓中央管开口隔膜形成是造成Chiari畸形并脊髓空洞的原因之一;小脑扁桃体下疝切除,脊髓中央管隔膜切开术是手术治疗Chiari畸形并脊髓空洞症的有效方法.     

枕大池重建术治疗ChiariI畸形合并脊髓空洞

目的 探讨枕大池重建术治疗合并脊髓空洞的Chiari I 畸形的临床意义.方法 对48例合并脊髓空洞的Chiari I 畸形病人行枕下正中入路,咬开下项线至枕大孔后缘及寰椎后弓,"Y"形切开硬脑脊膜和蛛网膜.对下疝小脑扁桃体显微镜下弱电流电凝或软脑膜下切除,开放正中孔直至第四脑室底,打通两侧小脑延髓外侧池,切开脊髓中央管口假膜.扩大修补硬脑脊膜及蛛网膜,重建枕大池.结果 术中发现28例有脊髓中央管口假膜.随访3～24月, 感觉及肌力均有不同程度的恢复, MRI复查8例脊髓空洞消失,40例明显缩小.结论 后颅凹减压、开放四脑室正中孔与两侧小脑延髓外侧池相通、切开脊髓中央管口假膜,是手术治疗合并脊髓空洞的Chiari I畸形的关键.     

枕大池重建术治疗Chiari Ⅰ畸形合并脊髓空洞

目的探讨枕大池重建术治疗合并脊髓空洞的Chiari Ⅰ畸形的临床意义。方法对48例合并脊髓空洞的Chiari Ⅰ畸形病人行枕下正中入路,咬开下项线至枕大孔后缘及寰椎后弓,"Y"形切开硬脑脊膜和蛛网膜。对下疝小脑扁桃体显微镜下弱电流电凝或软脑膜下切除,开放正中孔直至第四脑室底,打通两侧小脑延髓外侧池,切开脊髓中央管口假膜。扩大修补硬脑脊膜及蛛网膜,重建枕大池。结果术中发现28例有脊髓中央管口假膜。随访3～24月,感觉及肌力均有不同程度的恢复,MRI复查8例脊髓空洞消失,40例明显缩小。结论后颅凹减压、开放四脑室正中孔与两侧小脑延髓外侧池相通、切开脊髓中央管口假膜,是手术治疗合并脊髓空洞的Chiari I畸形的关键。     

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。     

Chiari畸形合并脊髓空洞的外科治疗

目的 探查外科治疗chiari畸形合并脊髓空洞症的方法及效果.方法 对86例chiari畸形合并脊髓空洞,行后颅窝减压术21例,后颅窝减压脊髓空洞切开引流18例,后颅窝减压并小脑扁桃体切除47例,其中切除下疝小脑扁桃体,并脊髓中央管口假膜切开3例.结果 所有病例临床症状都有不同程度的缓解,72例病例术后脊髓空洞消失或明显缩小.结论 手术使延颈髓充分减压,可有效缓解症状,而切除下疝小脑扁桃体,并充分松解四脑室后正中出口处,更能有效缓解临床症状,减轻或消灭脊髓空洞.     

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。     

第四脑室输出道疏通术治疗Chiari畸形并脊髓空洞

目的探讨显微外科治疗Chiari畸形并脊髓空洞的手术方法。方法Chiari畸形Ⅰ型并脊髓空洞经MRI确诊。小范围后颅窝减压 C1后弓切除,纵形切开硬膜,显微镜下分离小脑扁桃体与脑干的粘连,切除小脑扁桃体下疝,探察脊髓中央管开口,发现有隔膜或活瓣形成并切开,探察第四脑室侧孔及中脑导水管,使第四脑室脑脊液循环通畅。结果第四脑室输出道疏通术共计97例,术中显微镜下发现脊髓中央管开口有隔膜或活瓣形成,手术切除脊髓中央管口隔膜或活瓣,并探察第四脑室正中孔及两侧外侧孔,恢复第四脑室脑脊液循环,原位缝合硬膜。术后随访48例,平均1.3年,MRI检查示所有患者下疝的小脑扁桃体均消失,并存脊髓空洞明显缩小和消失。结论第四脑室输出道疏通术能够改善Chiari畸形Ⅰ型并脊髓空洞患者的术后临床症状。     

Chiari畸形并脊髓空洞的治疗与小脑扁桃体下疝分型

目的探讨Chiari畸形并脊髓空洞手术中,下疝的小脑扁桃体下舌分型与切除的标准。方法术中显微镜下观察32例下疝的小脑扁桃体压迫延髓的部位及其关系,结合MRI,提出下疝的小脑扁桃体分型,并根据分型切除小脑扁桃体下舌,切开脊髓中央管开口隔膜或活瓣,恢复第四脑室内脑脊液循环通畅。结果小脑扁桃体分型：①根据压迫部位分为：后方型27例,侧方型4例,侧前方型1例;②根据小脑扁桃体下疝程度分为：单侧压迫型11例,双侧压迫型21例。术后随访28例,MRI检查示下疝的小脑扁桃体均消失,第四脑室内脑脊液循环通畅,脊髓空洞明显缩小和消失;术后病人临床症状大多改善。结论下疝的小脑扁桃体根据其分型并切除,可为手术治疗Chiari畸形并脊髓空洞提供标准及依据。     

小脑扁桃体下疝切除术治疗Chiari畸形并脊髓空洞

目的评价小脑扁桃体下疝切除术治疗Chiari畸形并脊髓空洞的临床效果。方法本组Chiari畸形合并脊髓空洞患者10例均采取手术治疗。手术时有效范围后颅窝骨性减压,铣刀铣开小范围后颅窝骨瓣(3cm×3cm),不切除C1后弓,直线侧切开硬脑膜,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,原位缝合硬脑膜,颅骨锁固定骨瓣。结果手术无死亡及病残病例。10例术后(12d内)MR示小脑扁桃体下缘上升到枕骨大孔水平以上,后颅窝内容积扩大。术后长期随访9例,随访时间6个月～2年,2例患者症状逐渐改善,6例稳定未加重,1例较术前略加重,但MR示后颅窝内容积扩大,脊髓空洞缩小。结论小脑扁桃体下疝切除术治疗Chiari畸形并脊髓空洞患者,术后后颅窝内容积扩大,第四脑室内脑脊液循环通畅,临床症状多有改善。     

枕大池重建术治疗ChiariⅠ型畸形合并脊髓空洞症

目的 介绍枕大池重建术治疗合并脊髓空洞症的chiariⅠ型畸形的显微手术方法.方法 枕下正中入路,咬开下项线至枕大孔后缘及寰椎后弓,手术显微镜下"Y"形切开硬脑脊膜和蛛网膜,软脑膜下吸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,修补硬脑脊膜及蛛网膜,恢复枕大池正常结构.结果 36例手术治疗,术中发现30例有脊髓中央管口活瓣样假膜.随访3月～5年,感觉及肌力均有不同程度的恢复,MRI复查6例脊髓空洞症消失,30例明显缩小.结论 采用显微手术方法吸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,恢复枕大池正常结构和功能,是治疗chiarⅠ型畸形合并脊髓空洞症的关键.     

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.     

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。     

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。     

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。     

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.     

Surgical management of syringomyelia: a five year experience in the era of magnetic resonance imaging.

We summarize our experience with 59 consecutive surgically managed cases of syringomyelia (SM) over a 5 year period. All cases had magnetic resonance imaging (MRI) preoperatively and postoperatively. Twenty-eight patients presented with SM and the adult Chiari (Chiari I) malformation (SM-ACM), 6 patients had post traumatic syrinxes, 14 patients had syrinxes associated with an intramedullary neoplasm, 3 patients had syringomyelia associated with spinal arachnoiditis and 8 patients had idiopathic syringomyelia. Holocord syrinxes were more often associated with SM-ACM, while focal syrinxes were associated with posttraumatic, spinal arachnoiditis and neoplastic conditions. In all cases with neoplasms the MRI revealed parenchymal intramedullary signal abnormalities in addition to the syrinx cavity. Posterior fossa decompression with obex plugging (the Gardner operation) was the procedure of choice for SM-ACM and for idiopathic holocord syringomyelia. Exploration and drainage of the syrinx with or without shunting was carried out mainly for focal syrinxes associated with trauma and neoplasm. Patients with SM-ACM responded well to posterior fossa decompression with satisfactory results in 24/28 patients. Idiopathic SM is probably a forme fruste of SM-ACM and when treated with the Gardner procedure showed good results in all 8 patients. Posttraumatic and neoplastic SM had the least predictable results with surgery. The disappearance of the syrinx on postoperative MRI correlated well with a good surgical outcome.     

Treatment of Syringomyelia due to Chiari Type I Malformation with Syringo-Subarachnoid-Peritoneal Shunt

Chiari type I malformation is a tonsillar herniation more than 3 mm from the level of foramen magnum, with or without concurrent syringomyelia. Different surgical treatments have been developed for syringomyelia secondary to Chiari''s malformations: craniovertebral decompression with or without plugging of the obex, syringo-subarachnoid, syringo-peritoneal, and theco-peritoneal shunt placement. Shunt placement procedures are useful for neurologically symptomatic large-sized syrinx. In this paper, authors define the first successful treatment of a patient with syringomyelia due to Chiari type I malformation using a pre-defined new technique of syringo-subarachnoid-peritoneal shunt with T-tube system.     

International survey on the management of Chiari I malformation and syringomyelia

Introduction The availability of magnetic resonance imaging (MRI) has resulted in an increasing number of asymptomatic, minimally symptomatic, and doubtfully symptomatic patients being diagnosed with a Chiari I malformation with or without syringomyelia. In an attempt to clarify how neurosurgeons manage these clinical problems, an international survey on the Chiari I malformation and related syringomyelia was undertaken.Method A questionnaire on the expected natural course of the disease and on aspects of the surgical technique for a number of hypothetical cases relating to Chiari I malformation with and without syringomyelia was used to survey Pediatric Neurosurgeons worldwide.Results Of 246 questionnaires distributed, 76 (30.8%) were completed and returned. There was a consensus that no operation should be carried out in asymptomatic patients with a Chiari I malformation, unless there is associated syringomyelia. There was a consensus that decompression of the Chiari malformation should be performed in patients with scoliosis when syringomyelia is present, and the majority decompressed the Chiari malformation in scoliotic patients even in the absence of syringomyelia. Suboccipital decompression was the standard surgical procedure for Chiari I malformations. The majority of respondents favored routine dural opening at surgery and closure with a pericranial or synthetic patch graft. In the case of a persistent or progressive syrinx after suboccipital decompression, the majority recommended shunting of the syrinx to the subarachnoid space or to the pleural cavity.Conclusion There continues to be much variation in the management of the Chiari I malformation.