后颅窝重建术治疗Chiari畸形合并脊髓空洞

目的探讨后颅窝重建手术治疗Chiari畸形合并脊髓空洞的方法及效果。方法27例Chiari畸形合并脊髓空洞患者经MRI确诊后,应用后颅窝重建显微外科技术治疗,并随访3月以上。结果27例患者术后症状均有不同程度的改善,复查头颅MRI见脊髓空洞缩小。结论该手术可使颅颈交界区充分减压,改善脑脊液循环,有效缓解临床症状,并对脊髓空洞症有明显的治疗作用。扩大修补硬脑膜有利于减少术后并发症的发生。     

Chiari畸形Ⅰ型合并脊髓空洞症患者后颅窝扩大重建术后脑脊液漏原因分析

目的 探讨后颅窝手术较常见并发症脑脊液漏的原因.方法 通过回顾性的方法对21例脑脊液切口漏患者的临床资料进行分析总结.结果 本组21例Chiari畸形患者术后发生脑脊液漏,最早发生于术后第2天,最晚发生于术后第14天,平均8 d,均在拔引流管当日或后;拆线前15例,拆线后6例;其中切口漏15例均在近枕骨粗隆部位,引流口漏6例.临床表现为敷料渗湿,肉眼见切口或针眼流出无色或血性液体.结论 硬脑膜内外存在压力差是脑脊液漏的根本原因;术中硬膜、枕下肌肉及皮下组织缝合不严密及局部遗留死腔是脑脊液切口漏形成的主要原因;拔引流管后引流口未能清创缝合或清创缝合不彻底是脑脊液引流口漏的主要原因;最易发生脑脊液漏的部位是靠近枕骨粗隆处(本组占71%).     

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。     

枕大池重建术治疗Chiari畸形合并脊髓空洞

目的　介绍一种小脑扁桃体切除 枕大池成形术治疗Chiari畸形合并脊髓空洞症的手术方法。方法　枕下正中入路 ,咬开枕大孔后缘上至下项线 ,两侧至枕大孔的 4、 8点钟位置 ,约2 5cm× 4 0cm。咬除寰椎后弓 ,“T”形切开硬脑脊膜和蛛网膜。软膜下切除两侧小脑扁桃体 ,开放正中孔直至四脑室底并且打通两侧小脑延髓外侧池。取肌筋膜行硬脑脊膜连同蛛网膜的扩大修补 ,形成新的枕大池。结果　 12例中 ,男 5例 ,女 7例 ,年龄 18～ 5 1岁。MRI示脊髓空洞腔 <5节段 2例 ,5～ 8节段 8例 ,>8节段 2例。术后 3个月至 2年随访 9例 ,MRI复查见空洞腔均明显缩小 ,其中 3例空洞消失。感觉的恢复优于肌力的恢复。结论　此手术方法效果满意。其要点是 :(1)小脑扁桃体在软膜下切除并行软膜缝合。 (2 )四脑室正中孔开放并与两侧小脑延髓外侧池相通。 (3)枕大孔区蛛网膜下腔扩大修补缝合。无须行后颅窝减压术     

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。     

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。     

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.     

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畸形合并脊髓空洞症的手术治疗

目的 探讨Chiari畸形合并脊髓空洞症的手术方法和疗效。方法 对16例经MRI证实的Chiari畸形合并脊髓空洞症患者行后颅窝减压术加空洞．蛛网膜下腔分流术并评定其疗效。结果 按Tator标准进行判定,优12例,良3例,差1例,有效率为93．75％,无手术死亡者。结论对有症状的合并Chiari畸形的脊髓空洞症患者早期手术有利于减轻症状和神经功能的恢复。     

Chiari畸形合并脊髓空洞症的外科治疗

Chiari畸形又称小脑扁桃体下疝畸形.John Cleland于1883年首次描述了脑干、小脑移位变形和延髓空洞,Chiari分别于1891年、1895年将颅-椎移行区神经轴下移的程度进行了分类,1894年德国病理学家Arnold又作了更为详尽的描述,因此,1907年Schwalb和Gredig将此命名为ArnoldChiari畸形,而Sarnat和Williams则称其为Chiari畸形或描述表达为小脑扁桃体下疝畸形[1].     

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...     

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。     

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。     

Segmental hyperhidrosis in syringomyelia with Chiari malformation

Segmental hyperhidrosis was seen in three cases of syringomyelia with Chiari malformation confirmed by magnetic resonance imaging. Using the iodine starch reaction, all three cases showed hyperhidrosis in the areas roughly corresponding to those of sensory disturbances. Two possible modes of pathogenesis are discussed: stimulation of preganglionic neurons and interference to the inhibitory tract.     

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.     

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.     

Evidence of familial syringomyelia in discordant association with Chiari type I malformation

We report a sister and two half brothers who presented with magnetic resonance imaging (MRI)-proven syringomyelia and associated Chiari type I malformation in two cases. The individuals have the same mother but two different fathers. The mother shows no clinical signs of syringomyelia. The two fathers died through unknown causes. In a third healthy son of the mother by a relationship with a third father syringomyelia was excluded by MRI. We believe that an autosomal-dominant predisposition is the primary factor in the appearance of syringomyelia in these cases.     

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.     

Surgical results of posterior fossa decompression for patients with Chiari I malformation

Introduction An increasing number of children with Chiari I malformations are coming to the attention of neurosurgeons today, although a consensus on the surgical approach to these lesions has yet to be found.Methods We present a retrospective analysis of posterior fossa decompression (PFD) performed at our institution on 96 patients from 1989 to 2001. Statistical analyses based on clinical and radiographic presentation and the types of surgical procedures used formed the basis for our review.Results Most of the patients with hydromyelia underwent duraplasty procedures with or without tonsillar manipulation. In contrast, most patients without hydromyelia underwent bony decompression with dural scoring and intraoperative ultrasound. PFD with bony decompression and dural scoring showed a 72% success rate, compared with 68% for duraplasty. Dural opening was not more likely to improve or arrest hydromyelia. The group subjected to duraplasty, however, had a significantly higher complication rate. Patients under the age of 8 fared better than their older counterparts.Conclusions Overall, we favor a tailored posterior fossa craniectomy with dural scoring as the initial surgical procedure in children with Chiari I malformation with or without a syrinx. This less invasive approach minimizes complications associated with dural opening and offers comparable success rates.