枕骨大孔减压伴硬膜外层切开术治疗Chiari畸形合并脊髓空洞症的临床研究

目的探讨枕骨大孔减压伴硬膜外层切开术治疗Chiari畸形合并脊髓空洞症(ACM-SM)的手术疗效。方法按患者手术方式不同分为两组:A组30例,枕骨大孔减压组;B组38例,枕骨大孔减压伴硬膜外层切开组,回顾性分析两组患者预后情况。结果近期疗效:出院时,A组有效率70%(21/30),B组有效率92.1%(35/38),二者比较,B组患者近期疗效优于A组(P=0.02);远期疗效:A组67.9%(19/28)的患者症状有不同程度改善;B组93.9%(31/33)的患者症状有不同程度改善,两者比较,B组患者远期临床症状改善率优于A组(P<0.05);脊髓空洞:A组20例患者脊髓空洞大小较术前有不同程度缩小,6例空洞较前无明显变化,2例空洞较前略增大;B组患者随访提示31例患者脊髓空洞大小较术前有不同程度缩小,2例空洞较前无明显变化,无空洞增大病例,两者比较,B组患者在脊髓空洞改善方面优于A组(P<0.05)。结论外科手术是治疗ACM-SM的有效方法,应根据患者具体情况选择不同的术式;枕骨大孔减压伴硬膜外层切开术治疗ACM-SM患者术后长短期疗效均优于枕骨大孔减压术。     

Chiari畸形伴脊柱侧凸患者椎旁肌的失神经支配：后颅窝减压术后可获得改善吗？

 目的 通过研究椎旁肌细胞凋亡因子Bax、Bcl-2于后颅窝减压术（posterior fossa decompression,PFD）前后表达的差异,探讨PFD术对Chiari畸形/脊髓空洞伴脊柱侧凸患者椎旁肌失神经支配的改善效果。方法 前瞻性选取2011年7月至2012年7月,接受PFD及二期脊柱侧凸矫形手术的14例Chiari畸形伴脊髓空洞患者,于PFD及矫形术中分别切取颈胸段脊柱两侧椎旁肌中的部分竖脊肌组织。通过蛋白免疫印迹检测椎旁肌细胞Bax与Bcl-2的表达,测定灰度值并对其PFD前后表达差异进行定量分析。结果 14例患者接受PFD时年龄为（16.0±3.3）岁,主弯Cobb角为63.8°±18.3°,PFD与侧凸矫形术间隔（7.6±2.6）个月。Bax净灰度、阳性灰度积和阳性比于PFD术前分别为106.2±30.9、12.6×10⁴±9.4×10⁴和0.75±0.13,术后改善为73.9±38.5（P=0.021）、5.6×10⁴±6.1×10⁴（P=0.013）和0.28±0.15（P< 0.001）。Bcl-2净灰度、阳性灰度积和阳性比于PFD术前分别为84.4±35.8、10.6×10⁴±6.1×10⁴和0.40±0.17,术后改善为101.6±33.3（P=0.197）、21.3×10⁴±9.2×10⁴（P= 0.001）和0.85±0.19（P< 0.001）。结论 Chiari畸形/脊髓空洞伴脊柱侧凸患者椎旁肌的失神经支配可于PFD术后获得改善,提示此类患者的脊髓功能损害可能是一种可逆的病理过程。     

Chiari畸形Ⅰ型患者小脑扁桃体下疝程度及脊髓空洞形态与后颅窝容积的相关性

目的:分析Chiari畸形Ⅰ型(Chiari malformation typeⅠ,CMⅠ)患者小脑扁桃体下疝程度及脊髓空洞形态与后颅窝线性容积的关系,探讨影响小脑扁桃体下疝程度的后颅窝解剖学因素。方法:2003年6月~2011年6月在我科接受治疗并符合入选标准的CMⅠ患者共59例,男34例,女25例,年龄16~20岁,平均17.9岁,Risser征5级,均有完整MRI资料(包括头枕部及全脊髓矢状面扫描图像);均无颅内占位性病变、颅骨破坏、后颅窝手术史或获得性Chiari畸形。均伴有不同形态的脊髓空洞,55例(93.2%)伴有不同程度的脊柱侧凸畸形。在MRI T1加权像正中矢状位扫描层面上评估患者的小脑扁桃体下疝程度和脊髓空洞类型;测量后颅窝斜坡长度、枕骨大孔前后径、枕骨鳞部长度、后颅窝矢状径、后颅窝高径和斜坡倾斜角。将CMⅠ患者按照小脑扁桃体下疝严重程度分为三度:Ⅰ度,小脑扁桃体下缘超过枕骨大孔水平5mm但没有到达C1后弓上缘;Ⅱ度,小脑扁桃体下缘尾向移位超过C1后弓上缘但未超过C1后弓下缘;Ⅲ度,小脑扁桃体下缘尾向移位超过C1后弓下缘。依据脊髓空洞类型分为膨胀型、念珠型、细长型和局限型四组。比较不同组间后颅窝线性容积的差异,并对相关指标进行相关性分析。结果:Ⅰ度扁桃体下疝CMⅠ患者的后颅窝斜坡长度明显大于Ⅱ、Ⅲ度扁桃体下疝CMⅠ患者(P<0.05),Ⅲ度扁桃体下疝患者的斜坡倾斜角较Ⅰ、Ⅱ度患者明显减小(P<0.05),其余指标三组间无显著性差异;CMⅠ患者的斜坡倾斜角与小脑扁桃体下疝程度之间存在显著性负相关关系(r=-0.626,P=0.005)。膨胀型脊髓空洞患者的斜坡倾斜角显著小于其他类型的脊髓空洞患者(P<0.05),其余指标各类型之间无显著性差异。结论:后颅窝斜坡短平可能是促使CMⅠ患者小脑扁桃体下疝加重的重要因素之一,同时对CMⅠ患者的脊髓空洞的形成和发展也存在影响。     

小脑扁桃体下疝切除术治疗Chiari畸形伴脊髓空洞症

Chiari畸形并脊髓空洞症是神经外科常见疾病之一,近年来,随着MRI的广泛应用,该病的诊断、治疗均有较多进步,但对其确切的发病机理仍然不清楚,手术方式较多,疗效各异.我们采用后路减压,显微外科手术软膜下切除小脑扁桃体、正中孔开放+硬膜扩大修补治疗Chiari畸形合并脊髓空洞症17 例,报告如下.     

颅颈区减压及后颅窝重建术治疗Chiari畸形合并脊髓空洞症

目的 探讨颅颈区减压及后颅窝扩大重建术治疗小脑扁桃体下疝畸形（ｃｈｉａｒｉ ｍａｌｆｏｍａｔｉｏｎ,ＣＭ）－脊髓空洞（ＳＭ）复合征的疗效。方法 对１９９４年至１９９９年收治的３７例ＣＭ－ＳＭ复合征患者,采取颅颈区减压、显微硬膜下探查松解、后颅窝扩大重建术,而不行空洞切开引流。结果 ３７例随访６个月至４．５年,症状改善３１例（８３．８％）;不变５例（１３．５％）,加重１例（２．７％）,无死亡;ＭＢＩ     

Chiari畸形并脊髓空洞症的MRI研究

脊髓空洞症与许多先天畸形、肿瘤、外伤、蛛网膜炎等因素有关〔１、２〕,其临床分类比较混乱。国内有关Ｃｈｉａｒｉ畸形伴脊髓空洞症的ＭＲＩ研究较少,作者通过回顾性分析８６例该类患者的ＭＲＩ检查结果,探讨其形态信号特征及二者的关系。１资料和方法１１一般资料...     

青少年伴/不伴Arnold-Chiari畸形的脊髓空洞的影像学特征比较

目的探讨继发于Arnold-Chiari畸形的脊髓空洞(Arnold-Chiari malformation-associated syringomyelia,ACMS)与特发性脊髓空洞(idiopathic syringomyelia,IS)之间影像学特征的差异及其临床意义。方法对2006年6月~2013年6月在本院接受治疗并符合入选标准的132例(男70例,女62例)ACMS患儿及46例(男24例,女22例)IS患儿进行回顾性分析。在全脊髓MRI图像上评估2组患儿空洞的形态学特征,包括空洞与脊髓最大比值、空洞长度、空洞位置及形态。结果 ACMS组空洞与脊髓最大比值为0.61±0.18,空洞长度为9.01±4.88(2~20)个节段,均显著大于IS组(0.43±0.14,4.09±2.07),差异有统计学意义(P0.05)。ACMS组中82.57%空洞累及颈胸段,而IS组中50.00%空洞仅累及颈段。ACMS组中43.94%为膨胀型,而IS组中78.26%为局限型,差异有统计学意义(P0.05)。结论与IS相比,ACMS的空洞与脊髓最大比值更大,累及节段更长。     

Arnold-Chiari畸形合并脊髓空洞症的显微外科治疗

目的介绍Arnold-Chiari畸形合并脊髓空洞症(ACM-SM)显微外科治疗的术式及要点.方法总结我院1997年1月～2000年12月25例ACM-SM显微外科治疗的经验.结果25例ACM-SM术后临床症状改善23例(92%).术后平均随访1.5年,获得随访19例,复查MRI小脑扁桃体下疝全部消失,均有人工枕大池形成,脊髓空洞消失13例,空洞明显缩小6例.结论在颅后窝减压基础上,显微镜下松解小脑扁桃体与颈髓的粘连并切除,重建枕大池对治疗ACM-SM有明显疗效.     

单纯后颅窝减压术治疗Chiari Ⅰ型畸形合并脊髓空洞症的效果研究

目的探讨单纯后颅窝小骨窗骨性减压术治疗ChiariⅠ型畸形合并脊髓空洞症的临床疗效。方法对我院神经外科2008年1月至2014年4月收治的27例ChiariⅠ型畸形合并脊髓空洞症患者的临床资料进行回顾性分析,并依据Tator评价标准对所有病例的恢复情况进行评估。结果术后全部患者的感觉及运动症状均有改善,出院时依据Tator评价标准评估疗效,优17例,良10例;术后随访24例患者,随访时间0.5~6.0年,患者症状均有缓解,且MRI检查提示脊髓空洞较术前缩小或塌陷,无小脑移位等并发症出现,末次随访时依据Tator评价标准评估疗效,优15例,良8例,差1例。结论单纯后颅窝小骨窗骨性减压术并发症少,远期效果理想,是治疗ChiariⅠ型畸形的一种有效方法。     

Chiari畸形伴脊髓空洞症的脑脊液动力学研究

在Ｃｈｉａｒｉ畸形中，除小脑病扁桃体直接压迫延，颈髓所致的病理生理改变外，一直为人关注的是ＣＳＦ的动力学变化在合并脊髓空洞症时的病理生理，本文报道７例Ｃｈｉａｒｉ畸形Ｉ型体脊髓空洞症患者，经ＭＲ证实，并由ＣＴ明确有无脑室扩大，全部病例行颈部的过伸，过屈，正常位置下的Ｑｕｅｃｋｅｎｓｔｅｄｔ试验（奎氏试验），并根据试验结果分别脑室一腹腔分流术，后颅窝Ｃ１椎板减压术和硬膜减张，术后随访上述测试，均有Ｃ     

Surgical technique for cranio-cervical decompression in syringomyelia associated with Chiari type I malformation

Summary Our purpose is to present our results with the surgical treatment of syringomyelia associated with Chiari type I malformation. Between October 1989 and October 1995, twenty-eight patients underwent a sub-occipital craniotomy and a C₁ laminectomy. After dura mater opening the cerebellar tonsils were mobilised. Neither catheter, nor plugging of the obex, nor tonsillar tissue removal was performed. The dura mater was enlarged by means of a wide graft to create a newcisterna magna of adequate size. Postoperative MRI scans showed an ascent of the cerebellum of 4.3±4.8 mm (measured by thefastigium to basal line), as well as of the brainstem (mean migration of the mesencephalon-pons junction of 4.3±3.3 mm). The tonsils emigrated cranially 6.5±4.8 mm. While preoperative mean syringo-cord ratio was 66.3%±13.3, post-operatively was 12.1%±12.7 (p<0.0001). A complete collapse of the syrinx was observed in 39% of the patients. Long-term improvements were obtained in 73% of the cases and 27% were unchanged. No patient got worse. We conclude that in the treatment of syringomyelia associated with Chiari I malformation an artificial cisterma magna of sufficient size must be created. This is achieved by means of an extensive sub-occipital craniotomy and C₁ laminectomy, followed by dural opening. Small bone removal with limited enlargement of theposterior fossa often results in failures of treatment and recurrences. Tonsillar removal is not necessary to obtain a good reconstruction of the cisterma magna.     

Chiari畸形0型后颅窝减压手术治疗8例分析及文献复习

目的 结合文献复习探讨Chiari 0型患者经有限后颅窝减压术的术后疗效.方法 收集2013年1月-2015年1月在山西省人民医院神经外科符合诊断标准确诊为Chiari0型患者8例,患者均采用有限后颅窝减压术给予治疗,采用Tator标准评价,分为明显改善、部分改善、无明显变化,以评估术后疗效.结果 8例Chiari0型患者行后颅窝有限减压手术后,患者临床症状明显改善,术后1周MRI回报脊髓空洞均较术前明显缩小,术后3个月、1年门诊随访复查MRI示脊髓空洞较术后1周无明显变化,症状未见恶化.结论 有限后颅窝减压术对Chiari 0型伴脊髓空洞症的患者可能是合理、有效的术式之一.     

Spontaneous resolution of a Chiari I malformation associated syringomyelia in one child

Summary A child with complete spontaneous resolution of a Chiari I malformation associated Syringomyelia without surgical intervention is presented. The child was followed clinically by serial magnetic resonance imaging (MRI) and remains neurologically stable after 8-years of follow-up. To our knowledge, only 6 pediatric cases with spontaneous resolution of a spinal cord syrinx documented by MRI without surgical intervention have been reported. This case is of interest in the light of the postulated theories to explain spontaneous resolution of syringomyelia.     

Chiari Type 1 malformation: CSF flow dynamics and morphology in the posterior fossa and craniocervical junction and correlation of these findings with syrinx formation

BackgroundChiari type 1 malformation (CMI) is a disorder in which cerebellar tonsils descend below the foramen magnum. Although syringomyelia associated with CMI thought to be caused by hypoplastic posterior fossa and stenosis at the craniocervical junction; it has characteristic neurological and radiological features and the exact mechanism of syringomyelia remains unknown.PurposeThe purposes of this study were to gain insight into morphological changes in posterior fossa and to find whether there is a difference in aqueductal stroke volume (ASV) between CMI with syrinx and without syrinx which may be an underlying mechanism of syrinx development.Materials and methodsWe consecutively evaluated 85 patients with Chiari malformation between January 2017 and December 2019 who had undergone phase-contrast MRI examination for CSF flow and between 18–60-years-old. We divided patients into two groups as subjects with syrinx (n = 19) and without syrinx (n = 66). After evaluating morphological changes, peak and average velocity (cm/s), forward and reverse flow volume (μl), net forward flow volume (μl), ASV (aqueductal stroke volume) (μl), aqueductus Sylvi (AS) area (mm²), and prepontine cistern diameter to AS diameter ratio (PPC/AS) were calculated. Distribution of variables from two groups was evaluated by using Shapiro–Wilk normality test. Independent t test was used for groups comparison.ResultsThe forward and reverse volumes were statistically significantly higher in patients with syrinx (P = 0.021, P = 0.005 respectively). ASV was significantly increased in patients with syringomyelia (P = 0.014). The PPC/AS was significantly lower in patients with syrinx compared to those without (P < 0.001). AS area was significantly larger in those with syrinx. (P = 0.022). The diameter of foramen magnum was significantly lower in patients with syrinx than those without (P < 0.0001). The diameter of the herniated tonsilla at the foramen magnum level was found to be significantly lower in those with syrinx (P = 0.011).ConclusionForamen magnum diameter, ASV, diameter of herniated tonsil, and PPC/AS ratio are important factors in syrinx development.