复发性椎管内蛛网膜囊肿脊髓黏连的再手术体会

目的总结手术治疗复发性椎管内蛛网膜囊肿脊髓黏连的体会和方法。方法在脊神经肌电监测下，对4例复发性椎管内蛛网膜囊肿脊髓黏连的病人（2例为再次手术，2例为第3次手术）采用显微手术分块切除囊肿，彻底松解黏连的脊髓和神经根，用人工硬脊膜修补缝合硬脊膜，防止术后再黏连。结果2例神经根型和1例脊髓型病人术后临床症状立即改善，另1例术前二便失禁的脊髓型病人术后无明显改善。随访6～10个月，均无复发。结论手术广泛切除复发性蛛网膜囊肿的囊壁可有效减压；在脊髓肌电神经监测下．彻底松解黏连严重的脊髓和神经根，有利于术后神经功能的恢复；采用人工硬脊膜修复缝合硬膜可预防术后再黏连的发生。     

儿童脊髓拴系综合征的显微外科治疗

目的 研究显微手术治疗儿童脊髓拴系综合征的疗效。方法 回顾性分析35例儿童脊髓拴系综合征，平均年龄2.2岁。均伴有脊柱裂，合并单纯脊髓脊膜膨出20例，脊髓脊膜膨出合并椎管内脂肪瘤13例，畸胎瘤1例，皮样囊肿1例。行显微手术松解黏连的脊髓和神经，切断牵拉的终丝。结果 会阴部感觉异常总有效率为80．0％，排尿功能障碍总有效率为77．1％．结合矫形手术可明显改善运动障碍。结论 显微外科手术松解脊髓圆锥和马尾神经及切断牵张的终丝．是治疗儿童脊髓拴系综合征的关键．结合矫形手术可提高病儿的生存质量。     

显微手术治疗脊髓室管膜瘤预后的影响因素分析

目的 探讨显微手术治疗脊髓室管膜瘤的疗效及影响预后的因素。方法 对手术治疗的80例脊髓室管膜瘤的临床资料进行回顾性分析,参照欧洲脊髓病评分（EMS）和McCormick分级评估手术前后脊髓功能;采用多因素Logistic回归分析检验预后影响因素。结果 肿瘤全切除51例,次全切除25例,大部分切除4例。术后平均随访（37.6±19.0）个月,复发5例,均为WHOⅡ级,复发时间为（58.4±43.1）个月。末次随访有49例脊髓功能较术前改善,19例无变化,12例加重。术后McCormick分级:Ⅰ级35例,Ⅱ级22例,Ⅲ级12例,Ⅳ级11例。多因素Logistic回归分析发现,术前存在大小便功能障碍是不良预后独立危险因素,而伴脊髓空洞是预后良好因素。末次随访EMS[（15.01±0.3）分]明显高于术前[（13.55±0.35）分;P<0.001]和术后3 d[（13.20±0.31）分;P<0.001]。结论 脊髓室管膜瘤显微手术治疗后脊髓神经功能在短期内以趋于稳定为主,但从长远来看,显微手术能有效治疗脊髓室管膜瘤。存在大小便功能障碍的室管膜瘤建议及早手术治疗;伴有脊髓空洞的脊髓室管膜瘤手术预后良好。     

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。     

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.     

脊髓空洞症的手术治疗及预后

作者报告22例脊髓空洞症的手术治疗结果,22例病人中,男性13例,女性9例;年龄7～67岁(平均43.5岁)。20例病人为自发性脊髓空洞症,另2例分别为外伤和脑膜炎所致。 所有病人有术前均有明显神经功能进行性恶化。病人常主诉局部或神经根痛,并显示脊髓长束损害造成的共济失调及痉挛性瘫,放射诊断采用脊髓造影(13例)、脊髓CT扫描(10例)和MR(术前12例、木后10例)。 手术一般切除一、二个椎板,应用手术显微镜,正中切开脊髓,置入Pudenz引流管数厘米于空洞内。本组病人7例行空洞蛛网膜下腔分流、8例行空洞胸膜腔分流、1例单纯行空洞切开。脊髓空洞内积聚的液体:16例清亮似脑脊液,3例黄变,3例无记     

合并后颅窝囊肿的脊髓空洞症

脊髓空洞症的发生机制目前仍未完全明了。作者报道两例脊髓空洞症病人,都合并有陷入颈椎管的后颅窝蛛网膜囊肿,行单纯的囊肿-腹腔分流术后囊肿和空洞均完全消失,作者对此类脊髓空洞症的发生机制提出讨论。     

脊髓空洞症的外科手术治疗

目的 探讨脊髓空洞症手术治疗适应证、手术方式及效果。方法 对52例MRI证实的脊髓空洞症患者的手术治疗情况行回顾性分析，其中28例行后颅窝减压术，10例行后颅窝减压术＋脊髓空洞蛛网膜下腔分流术，5例行脊髓空洞蛛网膜下腔分流，9例手术治疗原发病。结果 患者术后临床症状明显改善37例，无改善但稳定12例，恶化3例。随访27例患者，空洞消失或基本消失者19例。缩小者6例，无改变者2例。结论 后颅窝减压术。脊髓空洞切开分流术及后颅窝减压术＋脊髓空洞蛛网膜下腔分流术是治疗脊髓空洞症的有效方法。     

Arnoid-Chiari畸形合并脊髓空洞症发病机理的临床研究

目的　探讨 Ａｒｎｏｌｄ Ｃｈｉａｒｉ 畸形合并脊髓空洞症 （ Ａ Ｃ Ｍ／ Ｓ Ｍ） 的发病 机理, 寻求更好的手术方法。方法　对１４ 例 Ａ Ｃ Ｍ／ Ｓ Ｍ 的患者进行 Ｍ Ｒ Ｉ检查及术前奎科试验, 术中枕大池、空洞及脊髓蛛网膜下腔 （ Ｓ Ｓ Ａ Ｓ） 压力测量, 分析与空洞形成及大小相关的因素, 对单纯 Ａ Ｃ Ｍ 及 Ｈ Ｃ Ｉ（ 正中矢状面最宽处空洞与脊髓直径比值） ＜ ０５ 者行枕下颅骨切除＋ 硬膜扩大修补术 （ Ａ） ; Ａ Ｃ Ｍ／ Ｓ Ｍ 且 Ｈ Ｃ Ｉ≥０５者行 Ａ＋ 空洞蛛网膜下腔（ Ｓ Ｓ） 分流术（ Ｂ） 。结果　１４ 例患者均有不同程度的颈部位置性梗阻。空洞内液体与 Ｃ Ｓ Ｆ蛋白含量无显著差异（ Ｐ ＞ ００５） , 小脑扁桃体下疝的程度与空洞大小无显著线性关系（ Ｐｒ１ ＞ ０５ , Ｐｒ２ ＞ ０２） ; 枕大池与椎管蛛网膜下腔压力差与空洞大小呈显著正相关（ Ｐｒ３ ＜ ０１ , Ｐｒ４＜ ０００５） 。手术有效率为９３８ ％ 。结论　脑脊液动力学改变是脊髓空洞形成的关键, 当后颅窝与椎管内压力梯度达到一定值时, 脊髓空洞开始形成。治疗应根据 Ａ Ｃ Ｍ 是否伴有 Ｓ Ｍ 及 Ｓ Ｃ Ｉ值选择恰当的手术方法。     

Chiari畸形合并脊髓空洞的诊治体会

目的探讨Chiari畸形合并脊髓空洞的手术方法.方法通过对12例经磁共振扫描确诊为Chiari畸形合并脊髓空洞的病人,应用枕颈部彻底减压脊髓空洞内引流的治疗.结果 12例病人手术治疗后临床症状和体征均有不同程度的改善;经随访明显好转8例,好转4例,行磁共振复查8例空洞完成消失,4例较术前明显缩小,无病人死亡和手术并发症.结论枕颈部彻底减压加脊髓空洞内引流是治疗Chiari畸形合并脊髓空洞症的一种有效方法.     

The presyrinx state due to adhesive arachnoiditis: a case report]

We report a 66-year-old woman with a presyrinx state due to adhesive arachnoiditis. She had a history of subarachnoid hemorrhage one year ago. She became aware of gait disturbance and abnormal sensation at the chest 6 months ago. Neurological examination revealed weakness of the both lower extremities and sensory disturbance below the T6 level. MR images of the thoracic spine revealed swelling of the thoracic cord at the T3-4 level, with an intramedullary hyperintensity on T2 weighted images (WI) and hypointensity on T1-WI. The intramedullary hypointensity on T1-WI was less than the CSF intensity. In addition, the thoracic cord lower than T5 level was compressed by an arachnoid cyst in the spinal canal. The preoperative diagnosis was the presyrinx state due to adhesive arachnoiditis. An operation for detachment of arachnoidal adhesion and restoration of CSF flow pathway was performed. After one month from the operation, neurological findings were improved. Postoperative MR images revealed improvement of swelling of the thoracic cord and intramedullary abnormal intensity. The arachnoid cyst in the spinal canal was decreased in the size. The presyrinx state is recently recognized condition before the formation of syringomyelia and that is known to resolve with proper treatment. We wish to emphasize we could prevent a progression to syringomyelia by a proper treatment for the presyrinx state.     

Diagnosis of syringomyelia and its classification on the basis of symptoms, radiological appearance, and causative disorders

Although it is easier to accurately diagnose syringomyelia with the advent of magnetic resonance (MR) imaging, syringomyelia still poses challenges to clinicians because of its complex symptomatology, uncertain pathogenesis and multiple treatment options. Here, we propose criteria for classification of syringomyelia not related to those associated with spinal intramedullary tumors. The classification aims to distinguish between the presyrinx state and syringomyelia, between asymptomatic and symptomatic syringomyelia and to clarify the associated disorders such as Chiari malformations, spinal arachnoiditis and spinal cord trauma. Diagnostic criteria for Chiari I and II malformations with MR imaging were also defined. Several hypotheses proposed to explain the pathogenesis of syringomyelia associated with Chiari I malformation were reviewed. A questionnaire survey on syringomyelia based on the proposed criteria conducted between November 2009 and April 2010 in Japan revealed that 160 (22.6%) of the 708 patients were asymptomatic. Chiari I malformation was the most frequent causative disorder (48.3%), followed by spinal arachnoiditis (15.8%). The proposed criteria for classification of syringomyelia will facilitate a nationwide survey of syringomyelia in Japan. Such a survey will inform us of its prevalence and prognosis more precisely, and enable us to build a reliable database that may help determine the optimal treatment for the disease in the future.     

Surgical management of syringomyelia associated with spinal adhesive arachnoiditis.

The authors describe a new surgical technique to minimise the postoperative recurrence of adhesion after microlysis of adhesion to treat syringomyelia associated with spinal adhesive arachnoiditis. A 47 year old male presented with numbness of the lower extremities and urinary disturbance and was demonstrated to have a case of syringomyelia from C1 to T2 which was thought to be secondary to adhesive spinal arachnoiditis related to a history of tuberculous meningitis. Following meticulous microlysis of the adhesions, maximal expansion of a blocked subarachnoid space was performed by expansive duraplasty with a Gore-Tex surgical membrane, expansive laminoplasty and multiple tenting sutures of the Gore-Tex graft. Postoperatively, the syringomyelia had be en completely obliterated and improvement of the symptoms had been also achieved. The technique described may contribute to improvement of the surgical outcome following arachnoid dissection by maintaining continuity of the reconstructed subarachnoid space.     

外伤后颈髓空洞症九例临床分析

目的回顾分析外伤后颈髓空洞症的症状、体征及影像学改变,探讨显微外科手术对颈髓空洞症的治疗效果。方法9例患者于颈椎损伤后7个月至6年经CT和MRI检查确诊为颈髓空洞;采取显微外科手术治疗;8例患者进行8个月至5年的随访以评判治疗效果。结果外伤后颈髓空洞症最常见症状包括疼痛,感觉缺失和肌力减退等。术后7例症状不同程度改善,2例无明显变化。MRI示1例空洞无明显变化,8例空洞不同程度缩小及消失。结论外伤后颈髓空洞可加重原有的神经损害,MRI是诊断脊髓空洞的金标准。神经功能障碍逐渐加重的脊髓空洞症患者宜接受显微外科手术治疗,可根据病变特点选择椎板切除减压术、空洞切开分流术治疗等外科术式,手术效果较好。     

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。     

Spinal syringomyelia following subarachnoid hemorrhage

Subarachnoid blood has been reported as a cause of chronic spinal arachnoiditis. Although syringomyelia has been thought to be caused by spinal arachnoiditis, reports of syringomyelia following aneurysmal subarachnoid hemorrhage (SAH) are very rare. We describe two patients with syringomyelia associated with chronic spinal arachnoiditis following SAH. From January 2001 to December 2010, 198 patients with aneurysmal SAH were treated at Kinki University School of Medicine. Two of the 198 patients had syringomyelia following aneurysmal SAH; thus the rate of syringomyelia associated with aneurysmal SAH was 1.0%. Patient 1 was a 54-year-old woman who presented with back pain, back numbness and gait disturbance 20 months after SAH. Her MRI revealed syringomyelia of the spinal cord from C2 to T10. She underwent shunting of the syrinx to the subarachnoid space. Patient 2 was a 49-year-old man, who was admitted to the hospital with headache, diplopia, hoarseness, dysphagia and ataxia five months after SAH. MRI revealed syringomyelia from the medulla oblongata to C6, and an enlargement of the lateral and fourth ventricles. After foramen magnum decompression and C1 laminectomy, a fourth ventricle-subarachnoid shunt was placed by insertion of a catheter. Spinal arachnoiditis and spinal syringomyelia are rare but important chronic complications after SAH.     

Syringoperitoneal shunt for treatment of cord cavitation.

A series of twenty-nine patients with cord cavitation were treated by syringoperitoneal shunt. Twenty-two of them showed improvement after operation, five were unchanged and two worsened. Operation was performed in patients with post-traumatic cystic myelopathy, idiopathic syringomyelia, intramedullary tumours associated with cysts, and patients who had developed cystic myelopathy in association with spinal arachnoiditis. Pain improved in twenty patients, sensory symptoms and signs in eleven and weakness in ten.     

与Chiari畸形或脊髓损伤无关的脊髓空洞症的外科治疗（英文）

PURPOSE Syringomyelia is a misleading disease since the problem always lies elsewhere.Arachnoiditis,because it is radiographically difficult to discern,is an especially insidious cause.To better guide selection from among surgical treatment options for syringomyelia,we reviewed our case series of patients without Chiari malformation or spinal injury.METHODS Excluding syringomyelia due to Chiari malformation,spinal cord injury,and tumors,32 patients(mean age 44 years)were operated on between 1995 and 2013and followed up for a mean of 53.8 months.Presumed causes at diagnosis,clinical and radiological findings,type of operation,clinical and radiological outcome were reviewed.RESULTS Duration of clinical history varied widely(range 6-164 months).Clinical assessment was based on the McC ormick classification(15 independent,17 dependent).Causes included birth trauma,pyogenic meningitis,tuberculous meningitis,postoperative scarring,dysraphism,and basilar impression.Treatment was local decompression with arachnoid lysis and shunts.Hindbrain-related syringomyelia was differentiated from non hindbrain-related syringomyelia.Hindbrain arachnoiditis was significantly associated with radiological findings at the foramen magnum(P=0.01)and craniocervical decompression(P0.03),with good clinical and radiological outcome at 6 months and later follow-up controls(P=0.02),whereas uneven results were observed in cases of non-hindbrain arachnoiditis.CONCLUSIONS To remove the cause of syringomyelia,surgical planning will rely on thorough clinical history and accurate imaging to determine the site of cerebrospinal fluid obstruction.Craniocervical decompression to dissect basal arachnoiditis in the posterior fossa can be recommended in hindbrain syringomyelia.Treatment of non-hindbrain arachnoiditis is more controversial,probably owing to uncertainties about the extent of adhesions.     

椎旁切口双入路切除胸腰段Eden Ⅱ型和Ⅲ型椎管内外沟通性肿瘤的临床疗效

目的初步探讨一期椎旁切口双入路切除胸腰段Eden Ⅱ型和Ⅲ型椎管内外沟通性肿瘤的临床疗效。方法回顾性分析2013年8月至2019年4月中国人民解放军东部战区总医院神经外科收治的12例胸腰段椎管内外沟通性肿瘤患者的临床资料,其中Eden分型Ⅱ型5例,Ⅲ型7例;术前McCormick分级Ⅰ级8例,Ⅱ级1例,Ⅲ级2例,Ⅳ级1例。所有患者均行一期椎旁切口双入路肿瘤切除术,3例行椎弓根螺钉内固定术。门诊随访患者的症状改善情况、有无轴性症状、McCormick分级以评估疗效,复查脊柱MRI,观察多裂肌有无萎缩以及脂肪浸润程度(由轻至重分为Ⅰ~Ⅲ度)。结果12例患者的肿瘤均全切除,术中横突部分医源性破坏3例。病理学结果显示,神经鞘瘤10例,原始神经外胚层肿瘤、黑色素细胞瘤各1例。术后1例患者出现泌尿系感染,经抗感染治疗后明显,缓解。术后McCormick分级Ⅰ级8例,Ⅱ级1例,Ⅲ级2例,Ⅳ级1例。12例患者的随访时间为(32.1±21.7)个月(6~62个月)。至末次随访,6例症状改善,5例症状稳定,1例(原始神经外胚层肿瘤)症状加重;Ⅱ例MrCormirk分级Ⅳ级改善为Ⅲ级,1例Ⅲ级改善为Ⅱ级,1例Ⅲ级加重为Ⅳ级,8例Ⅰ级和1例Ⅱ级患者的分级无变化;仅1例出现轴性疼痛症状;脊柱MRI显示,1例患者肿瘤复发,所有患者内固定均在位,无松动,骨质愈合好,脊柱稳定性良好,10例患者肿瘤侧的多裂肌萎缩,12例患者的多裂肌均有Ⅰ~Ⅱ度的脂肪浸润。结论初步观察发现,一期椎旁切口双入路切除胸腰段Eden Ⅱ型和Ⅲ型椎管内外沟通性肿瘤,可以达到肿瘤全切除,临床疗效较好。     

Syringomyelia and tethered cord in children

Introduction

The presence of syringomyelia varies in patients with different forms of dysraphism; from 21 % to 67 %. Only around 60 % of patients with syringomyelia is likely to experience symptoms related to it.

Pathophysiology

Many theories have been outlined for the creation of syringomyelia. The one most applicable to tethered cord dictates that tensile radial stress may create a syrinx in a previously normal cord tissue and transiently lower pressure may draw in interstitial fluid, causing the syrinx to enlarge if fluid exit is inhibited. In addition, arachnoiditis increases flow resistance in the spinal subarachnoid space, altering temporal CSF pulse pressure dynamics, which promotes entry of CSF in to the spinal cord.

Clinical presentation

There is a significant overlap between the symptoms that are due to tethered cord and syringomyelia, both in newly presenting patients with coexisting syringomyelia, and in previously treated patients who during follow-up present recurrent symptoms and a new syringomyelia cavity.

Treatment

The treatment of patients with tethered cord and syringomyelia is directed towards untethering the cord from its most caudal region upwards and restoring spinal anatomy with reestablishment of unobstructed CSF flow in the subarachnoid space. Only if complete untethering has been ensured and syringomyelia deteriorates, then surgical treatment can be directed against the syrinx. In patients with spinal dysraphism and coexisting hydrocephalus, radiological presentation of new syringomyelia or deterioration of previously known syringomyelia may signify shunt obstruction “until proven otherwise”.

Conclusion

In most occasions, satisfactory cord untethering addresses the development of syringomyelia.