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.     

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.     

脑干血管畸形的显微手术治疗(附19例报告)

目的 探讨脑干血管畸形的显微外科手术治疗方法和预后.方法 显微手术切除脑干血管畸形19例.6例经枕下正中第四脑室底入路切除;9例经枕下乙状窦后入路切除;3例经小脑延髓裂入路切除;1例经枕下正中入路上抬蚓垂后切除.结果 19例病灶均镜下全切,其中海绵状血管瘤15例,动静脉畸形4例.13例症状改善,5例出现并发症,1例因下呼吸道感染死亡.随访中GOS 5分15例,GOS 4分2例,GOS 3分1例.MRI复查无病灶残留.结论 在恰当选择手术适应证、手术时机和手术入路的基础上,运用娴熟的显微外科技术,脑干血管畸形的手术治疗结果是令人满意的.

Abstract：

Objective To investigate the microsurgical treatment of brainstem vascular malformation and evaluate the surgical outcome. Method Brain stem vascular malformations in 19 cases were resected by microsurgical techniques. Six cases of cavernous malformations ( CM ) in the dorsal of pons and medulla underwent operations via through the base of the forth ventrical approach. Another nine cases of CM in lateral and ventral lateral pons were treated via suboccipito - retrosigmoid approach. Three cases of arteriovenous malformation(AVM) in cerebellomedullary fissure were treated via telovelar approach. One case of AVM in the dosal medulla were resected via transvermian approach. Results All the lesions were totally resected. Pathologic diagnosis were CM( 15 cases) and AVM(4 cases). The diameter of all the CM were less than 1 centimeter. One AVM was 1.5 centimeter in diameter, the other two lesions was 2. 0 centimeter, the last one was 2. 5 centimeter. The functional disorders were improved after operation in 13 cases. Complications appeared in five patients, which improved between one week and three months. One patient died of sever pneumonia. During two months to six years after operation, the brainstem vascular malformation didn't recrudesce and re - bleed. No one patient appeared new syndromes. Conclusions The microsurgical management of brainstem vascular malformation can effectively prevent re - bleeding. Selecting different surgical approach basing on the locations can reduce the neurofunction damage. In order to accelerate the recovery of damaged brainstem, early surgery should be choosen for the patients with surgical indications. Basing on the correct choice of surgical indications, using the extensive knowledge of microanatomy,new concept of minimal invasive surgery and skillful microsurgical techniques, the surgical results of brainstem vascular malformation are satisfactory.     

两次改良Foerster-Dandy手术治疗痉挛性斜颈的对比研究

目的 对比两次改良Foerster-Dandy手术治疗痉挛性斜颈的疗效及并发症.方法 回顾分析183例痉挛性斜颈患者,其中A组126例采用改良Foerster-Dandy手术,B组57例采用在硬性神经内镜辅助下二次改良的Foerster-Dandy手术.结果 全部患者平均随访33.4个月.两组患者术后均立即感觉痉挛状态明显缓解,差异无统计学意义(P>0.05).A组36例发生不同程度吞咽困难,随访期间恢复正常18例,明显好转11例,无明显变化而影响生活质量7例;B组8例发生不同程度吞咽困难,随访期间恢复正常5例,明显好转2例,无明显变化而影响生活质量1例(该例仅随访1个月),差异有统计学意义(P<0.05).A组颅内感染10例(7.9%),B组颅内感染2例(3.5%),出院前均治愈,差异有统计学意义(P<0.05).B组手术时间较A组手术时间显著减少,差异有统计学意义(P<0.05).B组术中平均失血量较A组明显减少,差异有统计学意义(P<0.05).结论 硬性神经内镜辅助下二次改良的Foerster-Dandy手术,术中不咬除枕骨鳞部及枕大孔,在不降低疗效及不增加神经系统并发症的前提下,可进一步减少手术创伤,缩短手术时间,减少术中失血量,增加寰枕部稳定性,明显降低并发症的发生率.

Abstract：

Objective To study the microsurgical effectiveness of modified Foerster-Dandy's operation for the treatment of spasmodic torticollis under endoscope-assisted.Method 183 cases of spasmodic torticollis patients were treated by microsurgical modified Foerster-Dandy's operation from July 2001 to June 2009, which was randomly classified into group A and group B.Group A(126 cases) were treated by firstly modified Foerster-Dandy's operation.Group B(57 cases) were treated by microsurgical modified Foerster-Dandy's operation under endoscope-assisting.The other surgical steps were same with firstly modified Foerster-Dandy's operation.Results All the patients were averagely followed up for 33.4 months after the surgery.The spasticity was relieved immediately after the operation in all the patients.In group A, dysphagia in different degree in 36 cases, in whom, dysphagia was disappeared in 18 cases,dysphagia was significantly relieved in 11 , and unchanged in 7 during the following-up period.In group B,dysphagia was in different degree in 8 cases, in whom, dysphagia was disappeared in 5 cases, dysphagia was significantly relieved in 2, and unchanged in 1 ( follow-up only one month) ( P < 0.05 ).The intracranial infection rates in A and B groups were 7.9% and 3.5% respectively( P <0.05).The mean operative time was also significantly shorter in Group B than in Group A( P <0.05).The mean intraoperative blood loss in Group B was less than in Group A( P <0.05).Conclusions Modified Foerster-Dandy's operation can be done under endoscope-assisted, without resection of occipital squama and foramen magnum.This modified operation is able to keep the efficacy and do not increase the nervous system complications.It further reduces the surgical injury and intraoperative blood loss, increases the stability of the atlanto-occipital, and significantly descreases the incidence of intracranial complications.     

吲哚菁绿术中荧光造影在脊髓硬脊膜动静脉瘘手术中的应用

目的 探引哚菁绿术中荧光造影术在脊髓硬脊膜动静脉瘘手术中的作用.方法 9例脊髓硬脊膜动静咏瘘患者,均经脊髓DSA确诊,行后正中全椎板切开,术中吲哚菁绿荧光造影明确供血动脉、引流静脉及瘘口,夹闭瘘口并选择性切除静脉血管畸形.结果 畸形血管位于颈段1例、胸段4例、胸腰段2例、腰段1例、胸腰骶段1例;介入栓塞不充分后转手术1例,介入栓塞微导管难以到位转手术8例;术后MRI显示髓周异常迂曲畸形血管消失,脊髓缺血水肿好转.术后症状消失2例,改善6例,无变化1例.结论 吲哚菁绿术中荧光造影能够明确供血动脉、引流静脉及瘘口情况,有效地提高了脊髓硬脊膜动静脉瘘手术的疗效.

Abstract：

Objective To evaluate the clinical significance of intraoperative indocyanine green (ICG)videoangiography in surgical management of spinal dural ateriovenots fistulae (dAVFs).Method In this retrospective analysis we examined nine cases of dAVFs, diagnosed by complete spinal angiography,in which laminoplasty were performed through posterior approach.An operating microscope - integrated light source containing infrared excitation light illuminated the operating field and was used to visualize an intravenous bolus of ICG.The locations of fistulae, feeding arteries and draininig veins were identified and compared before and after surgical obhteration by intraoperative ICG videoangiography.Results In the nine cases the dAVFs involved one cervical cord, four thoracic cord,two thoracic lumbar cord, one lumbar cord and one thoracic and lumbosacral cord.One of them used to take an unsuccessful endovascular embolization, while the rest of them were given the operation right after diagnosed by the spinal angiography.Microscope-based ICG videoangiography identified the fistulous point(s),feeding arteries and draining veins in all nine cases,as confirmed by postoperative MRI which showed complete obliteration of the dAVFs with improved spinal blood supply and reduced spinal cord edema.After the operation the clini cal symptoms were nearly disappered in two cases, improved in six cases, and present no obvious changes in one case.Conclusions Intraoerative ICG videoangiography provides real -time information about the precise location of spinal dAVFs,the feeding arteries and the draining veins, as well as additional feeding aiteries unrevealed by the preoperative spinal angiography and residue pathologic blood vessels during the operation, which efficiently improves the surgical outcomes and prognosis.     

枕大孔区肿瘤的分型及手术入路选择

目的 探讨枕大孔区肿瘤的分型及手术入路.方法 回顾性分析显微手术治疗的43例枕骨大孔区肿瘤,根据肿瘤位置分为:Ⅰ型(背侧)和Ⅱ型(腹侧),Ⅰ型又分为Ⅰ a(髓外)、Ⅰ b(髓内)、Ⅰ c(髓内外)三型,Ⅱ型又分为Ⅱa(髓外)和Ⅱb(髓内)两型.对于17例Ⅰ型患者选择后正中入路,26例Ⅱ型患者选择远外侧入路.结果 全切肿瘤35例(81%),无手术死亡,随访期内无肿瘤复发.临床症状改善32例;保持原有症状3例;加重8例,包括出现新的脑神经症状、肢体运动障碍和呼吸困难.结论 枕大孔区肿瘤的术前分型有助于手术入路的选择和判断手术效果,Ⅰ型多选择后正中入路,Ⅱ型选择远外侧入路.

Abstract：

Objective To explore the classification and surgical approach of magnum foramen tumor.Method A retrospective analysis was performed for 43 surgically treated patients with tumors involving the foramen magnum.According to the site of the tumor,the classification was divided to:Type Ⅰ ,located at dorsal, Ⅰ a extra - medullary, Ⅰ b intra - medullary, Ⅰ cintra - and extramedullary; Type Ⅱ,located at ventral, Ⅱ a extramedullary, Ⅱ b intramedullary.The midline approach was used in the Type Ⅰ (17cases), while the lateral or far- lateral approach for the Type Ⅱ (26 cases).Results Total excision was performed in 35(81% ).There were no mortality and no recurrence during the follow -up period.Thirtytwo patients had improvement in their neurological status, 3 cases unchanged.There were 8 cases aggravation,including newly cranial nerve deficits, hemiplegia, dyspnoea.Conclusions The preoperative classification was useful for the selection of surgical approach and evaluation of surgical outcome.The midline approach was apt to Type Ⅰ , while the lateral or far - lateral approach for the Type Ⅱ.     

神经内镜在面肌痉挛微血管减压术中的应用研究

目的 探讨神经内镜在显微血管减压术(MVD)治疗面肌痉挛(HFS)的应用价值.方法 济宁市第一人民医院神经外二科自2006年12月至2009年12月应用神经内窥镜辅助下MVD治疗HFS患者118例,回顾性分析患者的临床资料和疗效.结果 术中单纯经显微镜下发现责任血管的共106例(89.83%),其中有7例患者在神经内镜辅助下再次发现其它责任血管压迫;8例(6.78%)在显微镜下未见明显血管压迫,但经神经内镜发现有责任血管的存在.显微镜下减压完成后再用神经内镜观察,发现Teflon棉片未减压完全,需再次调整的17例(14.41%);术后102例(86.44%)痉挛立即消失,11例(9.32%)逐渐消失,3例(2.54%)部分缓解,2例(1.70%)无明显减轻,轻度复发.无脑脊液漏、偏瘫、死亡等严重并发症发生.结论 神经内窥镜辅助下的MVD能清晰显示神经血管的解剖关系,减少对脑干、各颅神经的牵拉和重要血管的损伤,对提高手术疗效、减少复发率和术后并发症有重要价值.

Abstract：

Objective To explore the value ofneuroendoscope in microvascular decompression for treatment of primary hemifacial spasm. Methods One hundred and eighteen patients with hemifacial spasm, admitted to our hospital from December 2006 to December 2009, underwent endoscope-assisted microvascular decompression via suboccipital retrosigmoid approach; their clinical data and treatment efficacy were retrospectively analyzed. Results The offending vessels were found in 106 patients (89.83%) under microscope, of which 7 (5.93%) were again found other offending vessels under neuroendoscope. Eight patients (6.78%) were noted as no significant vascular compression under microscope, but the offending vessels of these patients existed under endoscope. Seventeen patients performed decompression with Teflon cotton under microscope needed modification when they were checked under endoscope. The symptoms of 102 patients (86.44%) disappeared immediately after the operation; 11 (9.32%) disappeared gradually; 3 (2.54%) had partial remission and 2 (1.70%) had no significantly remission. No serious complications, including cerebrospinal leakage, paralysis and death, were noted. Conclusion Endoscope-assisted microvascular decompression, by improving the observation of the anatomic neurovascular relation, decreasing the damage to the brain stem, nerves and important vessels, can improve the surgical treatment, and reduce the recurrence rate and postoperative complications.     

Chiari畸形合并脊髓空洞症的显微手术治疗

目的探讨Chiari畸形合并脊髓空洞症的治疗方法及效果。方法 2008~2013年收治Chiari畸形合并脊髓空洞症患者32例,20例采用后颅窝减压寰枕筋膜松解术,12例空洞严重者采用后颅窝减压寰枕筋膜松解术＋空洞-蛛网膜下腔分流术。结果术后随访0.5~3年,28例患者症状改善,4例无变化;25例复查MRI示下疝的小脑扁桃体回复,23例脊髓空洞较术前明显缩小。结论后颅窝减压寰枕筋膜松解术治疗Chiari畸形合并脊髓空洞效果明显,并发症少。     

大脑中动脉镜像动脉瘤

目的 探讨大脑中动脉(MCA)镜像动脉瘤的临床特征、影像学改变及显微外科手术治疗的疗效.方法 回顾分析显微外科手术治疗的9例18个MCA镜像动脉瘤患者的临床资料.采取早期与择期、一期与分期相结合的方法处理MCA镜像动脉瘤,原则是先处理破裂动脉瘤,再处理未破裂动脉瘤.结果 动脉瘤直接夹闭17个,未处理1个.依据GOS判断:优良6例,轻残2例,重残1例.结论 将MCA镜像动脉瘤分为Ⅰ型(完全对称型)和Ⅱ型(不完全对称型)有助于判断出血的责任动脉瘤.显微外科手术治疗MCA镜像动脉瘤效果显著.

Abstract：

Objective To study the clinical characteristics, neuroradiological manifestations of mirror - image aneurysm of middle cerebral artery (MCA) and effects of microsurgical management.Method The clinical data of 9 cases with 18 mirror - image aneurysms of MCA which underwent microsurgical operation were analysed retrospectively. The mirror - image aneurysms of MCA were treated through the method of combining early - stage with select - stage operation and one - stage with two - stage operation. The operating principle was the ruptured aneurysm performed treated first and the unruptureed aneurysm performed treated second. Results Seventeen aneurysms were clipped directly and one aneurysm did not treat. Surgical outcome were good in 6 cases, light disability in 2 cases and severe disability in 1 case. Conclusions It can help to determine the responsibility aneurysm of ruptured aneurysms if the mirror - image aneurysm of MCA had been divided into Ⅰ model ( perfectly symmetry model ) and Ⅱ model ( in perfectly symmetry model). The surgical outcome were predominance if the mirror -image aneurysm of MCA performed microsurgical operation.     

Chiari畸形I型手术治疗的临床观察

目的 探讨小切口、微骨窗寰枕减压术+寰枕筋膜松解术治疗Chiari畸形I型的临床疗效，并观察术后寰枕交界区稳定性。方法 分析2012年1月—2017年12月该院伴有头晕的Chiari畸形I型行小切口、微骨窗寰枕减压术+寰枕筋膜松解术治疗31例，依据芝加哥Chiari畸形预后量表（CCOS）评估疗效，术后对寰枕交界区稳定性进行3~6年的随诊观察。结果 所有病例均顺利完成手术，无手术死亡病例，无严重神经功能障碍等并发症。依据CCOS在术后1、3、6及12个月的重复评估，评估得出总平均分13.56分，预后良好24例（77.4%），改善不明显5例（16.1%），1例预后较差（3.2%）。随访观察3~6年，头晕缓解有效率为93.5%，30例寰枕交界区稳定，1例伴寰枢椎脱位病例1年后症状加重，需行内固定。结论 小切口、微骨窗寰枕减压术+寰枕筋膜松解术治疗Chiari畸形I型，手术安全、有效,且手术简单、并发症少，对伴有的头晕症状缓解率高。经长期随访寰枕交界区稳定。     

Chiari I畸形合并脑积水的手术治疗(附33例报告)

目的　研究Chiari I畸形合并脑积水的手术方式和手术方法,并评价手术效果。方法　回顾性分析33例Chiari I畸形合并脑积水患者,全部病例均采用后颅凹减压术并在显微镜、脑室镜等辅助下切除下疝的小脑扁桃体并松解去除脑室系统内外粘连,使用神经补片减张缝合硬膜。术前术后分别作MR和脑室系统径线的测量比较并随访复查。结果　全部病例MR显示颈延髓压迫解除,枕大池形态恢复,2 3例症状明显改善,2 5例脑室明显缩小,6例脑室系统变化不显著,2例脑积水仍有发展。结论　Chiari I畸形合并脑积水以梗阻因素为主,术中除作好骨性结构的充分减压外,还应松解粘连、解除梗阻并重建枕大池,争取在手术中一次性解决颈延髓压迫和脑积水两个问题。     

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。     

Intraoperative aneurysmal subarachnoid hemorrhage after rupture of a previously undiagnosed intracranial aneurysm during Chiari decompression

A type I Chiari malformation occurs when caudal displacement of the cerebellar tonsils below the level of the foramen magnum obstructs the normal flow of cerebrospinal fluid (CSF) between the cranial and spinal spaces, a condition that often needs surgical decompression to restore normal CSF circulation. Abrupt changes in CSF flow dynamics after Chiari decompression can affect the intracranial CSF dynamics to the extent that a previously undiagnosed intracranial aneurysm remote from the site can rupture. The authors describe the development of an intraoperative aneurysmal subarachnoid hemorrhage that occurred as a result of spontaneous rupture of a previously undiagnosed right distal posterior inferior cerebellar artery in a 57-year-old woman with type I Chiari malformation who was undergoing surgical decompression. The mechanism of the aneurysmal rupture appears to be related to the changes of CSF flow dynamics during surgical decompression. Normally, pressure equilibrium between the two sides of the aneurysmal wall prevents its rupture, but factors that significantly affect this equilibrium, such as systemic hypertension, can cause the aneurysm to rupture. To our knowledge, the concept of spontaneous intraoperative rupture of intracranial aneurysm remote from the site of surgery has been described twice previously but under different scenarios. This scenario, to our knowledge, has not been previously described.     

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。     

保留枕骨大孔的颅后窝减压术治疗Ⅰ型小脑扁桃体下疝畸形的疗效

目的 探讨探讨保留枕骨大孔的颅后窝减压术治疗Ⅰ型小脑扁桃体下疝畸形 （ CM-Ⅰ）的疗效。方法 回顾性分析2015年1月至2016 年5月收治的21例CM-Ⅰ的临床资料。采用枕骨小骨窗减压（骨窗大小约2.5 cm×2.5 cm）及枕大池成形术，并保留枕骨大孔后缘约0.5 cm骨质。术前、术后1周、术后1年采用KPS评分、神经症状改善及小脑扁桃下疝程度评估疗效。结果 术后1周KPS评分[（66.52±9.8）分]较术前[（52.9±9.1）]明显增高（P<0.05）。术后1年KPS评分[（84.7±7.2）分]较术后1周明显增高（P<0.05）。术后1年神经症状改善率（90.48%，19/21）较术后1周（71.43%，15/21）明显提高（P<0.05）。术后1年小脑扁桃体下疝程度[超过枕骨大孔（2.9±1.8）mm]较术前[（4.1±1.4）mm]明显缩小（P<0.05）。结论 保留枕骨大孔的颅后窝减压+枕大池成形术是治疗 CM-Ⅰ安全、有效的方法。     

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.     

前屈-后伸位MRI对判断颅脊交界区畸形内固定指征的临床价值

目的分析前屈-后伸位MRI对诊断Arnold-Chiari畸形可能合并寰枢椎脱位的作用,以及指导治疗的临床意义。方法回顾分析40例Arnold-Chiari畸形患者的前屈-后伸位MRI影像学资料,测量寰齿间距,通过前屈位和后伸位颈椎椎管狭窄程度分级,判断颅脊交界区稳定性。单纯Arnold-Chiari畸形患者采用枕大孔减压和枕大池扩大成形术,存在寰枢椎脱位者兼行枕颈内固定融合术。结果经前屈-后伸位MRI检查,证实有12例患者存在颅颈失稳,于枕大孔减压的同时行枕颈内固定融合术。手术后第3天颈椎影像学检查,40例中10例脊髓空洞病灶明显缩小;12例兼行枕颈内固定融合术者颅颈复位满意,脊髓压迫解除;手术后3个月随访,脊髓空洞病灶明显缩小(17例),颅脊交界区骨痂形成、骨融合效果良好、颅颈复位无丢失(12例),日本矫形外科评分13.08±1.40,与手术前评分(11.08±1.61)比较,差异有统计学意义(t=5.928,P=0.000)。结论前屈-后伸位MRI对判断颅脊交界区稳定性、选择适宜的手术方式具有重要意义。     

Recurrent syncope and Chiari malformation

Downward herniation of the cerebellar tonsils through the foramen magnum (Chiari I malformation) is usually revealed by head and neck pain, often associated with brain-stem or spinal cord disturbances. Syncopes are rarely reported and may be difficult to link to their cause when they occur alone. We report two cases with brief and repetitive syncopes revealing a Chiari I malformation. These manifestations may be attributed to transient compression of brain stem or vascular structures at the craniocervical junction, triggered by intracranial pressure increase, as they disappear after posterior fossa decompression.     

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.     

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。