脊髓纵裂合并脊髓栓系综合征的治疗

目的：分析双管型脊髓纵裂患者脊髓栓系综合征的手术治疗效果。方法：对34例行手术治疗的双管型脊髓纵裂TCS患者进行回顾性分析。结果：通过手术后观察和3个月～5年的随访发现,手术的总有效率可达91％,尤其是疼痛为主诉的患者恢复最为明显,术后恢复似乎与术前病程无关。结论：双管型脊髓纵裂合并TCS的患者一旦确诊,无手术禁忌症就应尽早手术。只要手术操作细致,大多患者症状可缓解,不会出现明显的神经并发症。     

脊髓纵裂分型治疗的疗效分析

目的：探讨脊髓纵裂分型治疗效果。方法：对1978年5月至2005年1月我院收治的有完整资料的121例单管型及双管型脊髓纵裂手术及非手术治疗的病例进行回顾性分析。结果：双管型脊髓纵裂通过手术后观察和6个月～15年的随访发现，手术的总有效率可达86％，尤其是疼痛为主诉的患者恢复最为明显；单管型脊髓纵裂手术前后无明显变化。结论：双管型脊髓纵裂脊髓损害重，且呈进行性加重，精细的手术治疗可以使大多数患者症状缓解，终止神经进行性损伤，确诊后应尽早手术；单管型脊髓纵裂脊髓损害轻，不需手术探查。     

成人脊髓纵裂

目的: 分析成人脊髓纵裂患者的手术治疗效果。方法: 对 13例行手术治疗的成人脊髓纵裂患者进行回顾性分析。结果: 通过手术后观察和 6个月 ~5年的随访发现, 手术的总有效率可达 87%, 尤其是疼痛为主诉的患者恢复最为明显, 术后恢复似乎与术前病程无关。结论: 双管型脊髓纵裂患者一旦确诊, 无手术禁忌证就应尽早手术。只要手术操作细致, 大多患者症状可缓解, 不会出现明显的神经并发症。     

脊髓纵裂手术治疗的疗效

[目的]探讨脊髓纵裂手术治疗效果.[方法]对1978年5月～2006年1月收治的有完整资料的121例单管型及双管型脊髓纵裂手术及非手术治疗的病例进行回顾性分析,并通过临床神经功能评分及胫后神经皮层体感诱发电位(PTNCSEP)P40波峰监测,评价其手术治疗的疗效.[结果]双管型脊髓纵裂通过手术后观察和6个月～15年(平均2年6个月)的随访发现,临床神经功能评分及PrNCSEP术后有明显改变,尤其是疼痛为主诉的患者恢复最为明显,手术的总有效率可达86%;单管型脊髓纵裂手术前后无明显变化.[结论]双管型脊髓纵裂脊髓损害重,且呈进行性加重,精细的手术治疗可以使大多数患者症状缓解,终止神经进行性损伤,确诊后应尽早手术;单管型脊髓纵裂脊髓损害轻,不需手术探查.     

成人脊髓纵裂的疗效分析

目的：分析成人脊髓纵裂患者的手术治疗效果。方法：对13例行手术治疗的成人脊髓纵裂患者进行回顾性分析。结果：通过手术后观察和6个月～5年的随访发现,手术的总有效率可达87％,尤其是疼痛为主诉的患者恢复最为明显,术后恢复似乎与术前病程无关。结论：双管型脊髓纵裂患者一旦确诊,无手术禁忌症就应尽早手术。只要手术操作细致,大多患者症状可缓解,不会出现明显的神经并发症。     

脊髓纵裂分型治疗的疗效分析

目的探讨脊髓纵裂分型治疗的疗效。方法对1978年5月至2006年11月收洽的有完整资料的121例单管型及双管型脊髓纵裂患者的病历资料进行回顾性分析，并通过临床神经功能评分及胫后神经皮层体感诱发电位（FTNCSEP）P40波峰监测，评价其手术及非手术治疗的疗效。结果所有患者均得到随访，随访时间6个月～15年，平均2年6个月。96例双管型患者中手术治疗86例，术后观察和随访发现24例痊愈、22例有明显改善、28例有一定改善、12例无明确效果，临床神经功能评分及PTNCSEP术后有明显改变，尤其是以疼痛为主诉的患者恢复最为明显，手术总有效率为86％；10例双管型患者未行手术治疗，随访发现临床症状无明显改善且有加重趋势。单管型患者25例，手术治疗16例，术前、术后临床神经功能评分及胛NCSEP无明显变化；未手术治疗的9例，随访时上述指标亦无明显变化。结论双管型脊髓纵裂患者脊髓损害重且呈进行性加重，精细的手术治疗可以使大多数患者症状得到缓解，终止神经进行性损伤，确诊后应尽早手术。单管型脊髓纵裂患者脊髓损害轻，无须手术探查。     

脊髓纵裂分型及临床意义

目的　研究脊髓纵裂临床病理分型及临床意义。方法　通过对 65例脊髓纵裂临床资料的回顾 ,根据影像学、病理解剖和临床表现 ,结合手术方法和效果 ,进行脊髓纵裂分型。结果　按硬脊膜形态及纵裂与脊髓关系分为双管型和单管型。双管型特征 :硬脊膜为两独立的次管 ,其内有各自分裂的半脊髓 ,两硬膜管间隙有骨性、软骨性或纤维性间隔。单管型硬膜管维持单管状 ,管腔扩大 ,其内有两个呈“镜影状”分裂的脊髓 ,少有间隔。结论　脊髓纵裂应按分型确定治疗方法。双管型应尽早手术治疗 ,如切除骨嵴 ,切断纤维性带状终丝 ,脊髓水囊肿内引流等。然后 ,进行先天性脊柱畸形矫正。单管型一般不主张脊髓探查 ,可考虑早期进行畸形脊柱的矫正治疗。     

脊柱截骨术治疗合并脊髓纵裂伴脊髓拴系综合征的先天性脊柱侧凸

目的:探讨脊柱截骨术治疗合并脊髓纵裂伴脊髓拴系综合征的先天性脊柱侧凸的安全性和有效性。方法:回顾性分析2007年6月~2013年6月在我院采用脊柱截骨手术治疗的23例合并脊髓纵裂伴脊髓拴系综合征的先天性脊柱侧凸患者。其中男6例,女17例;手术时年龄16.9±3.4岁(10~23岁)。腰骶部疼痛8例,其中5例仅表现为腰骶部疼痛,神经功能损害18例。术前冠状位Cobb角95.4°±25.2°(65°~156°)。Ⅰ型脊髓纵裂9例,Ⅱ型脊髓纵裂14例。脊髓圆锥位置均在L3水平以下。对合并Ⅰ型脊髓纵裂伴脊髓拴系的患者,采用骨性纵隔切除、一期脊柱截骨矫形;合并Ⅱ型脊髓纵裂伴脊髓拴系的患者单纯行一期脊柱截骨矫形(未处理纤维纵隔)。其中行全脊椎截骨13例,经椎弓根截骨10例。术前、术后3个月和末次随访时分别测量患者侧凸Cobb角,并按脊柱裂神经功能评分(SBNS)分级评估神经功能恢复情况。结果:手术时间571.1±136.5min(310~835min);术中失血量4888.3±2482.3ml(500~9600ml)。随访38.9±18.3个月(24~79个月)。术后冠状面Cobb角33.7°±15.9°(3°~73°),较术前明显改善(P0.05),矫正率平均为(62.3±14.1)%;末次随访时冠状面Cobb角37.4°±17.2°(5°~82°),矫正率平均为(58.1±14.7)%,较术后平均丢失4.2°±2.3°,与术后比较无明显矫形丢失(P0.05),但与术前相比有明显改善(P0.05)。末次随访时,18例患者神经损害症状获得不同程度改善,其中13例术前SBNS神经功能分级为Ⅱ级者术后恢复至Ⅰ级;2例Ⅲ级恢复至Ⅱ级;另外3例神经损害评分提高,SBNS分级维持不变,脊柱侧凸畸形及局部疼痛明显好转。围手术期出现并发症5例,其中2例术后出现单侧下肢肌力下降,1例术后2周下肢肌力恢复至4级,另1例于术后3个月恢复至术前水平,术后2年随访肌力基本恢复正常;术中发现胸膜破裂1例,术后脑脊液漏1例、泌尿系感染1例。所有病例术后无伤口感染、假关节形成、内固定松动/断裂及永久性神经损害并发症。结论:脊柱截骨术治疗合并脊髓纵裂伴脊髓拴系综合征的先天性脊柱侧凸患者安全有效,且对神经功能恢复有促进作用。     

成人脊髓纵裂的诊断与治疗

目的：通过对20例成人型脊髓纵裂的分析，提供成人型脊髓纵裂的诊断与治疗方法。方法：回顾性研究成人型脊髓纵裂的病史、临床表现、影像学检查、手术疗效。结果：成人型脊髓纵裂是由于脊髓的过屈牵拉，导致间隔撞击裂口下端脊髓，引起发病，常有损伤病史。其主要症状为疼痛，无矫形外科综合征，其症状较儿童型轻，手术可显著改善症状。结论：成人型脊髓纵裂的症状及手术疗效与儿童型脊髓纵裂有明显区别，早诊断早治疗有良好疗效。     

23例单管型脊髓纵裂的回顾性临床分析

目的 分析单管型脊髓纵裂的诊断与治疗方法。 方法 对23例单管型脊髓纵裂患者的神经功能进行临床评分，并检查其胫后神经皮层体感诱发电位（PINCSEP），按临床表现及是否需要手术治疗分组，探讨手术适应证及疗效。结果 7例无临床症状患者随访前后的临床评分及PTNCSEP P40潜伏期无变化；11例非进行性神经功能缺陷患者，无论是否手术，随访前后临床评分及PTNCSEP P40潜伏期亦无变化；5例进行性神经功能缺陷患者，手术后的临床评分及PTNCSEP P40潜伏期明显改善，术中均发现间隔或纤维带等异常结构。结论 对单管型脊髓纵裂应按是否存在进行性神经功能缺陷分类并治疗，对进行性神经功能缺陷的患者应及时手术；无症状或非进行性神经功能缺陷患者不宜手术，应长期观察，定期随访。     

成人脊髓栓系综合征的诊治

目的研究成人脊髓栓系综合征（ＴＣＳ）的临床特征以指导诊断和治疗。方法报告１８例成人ＴＣＳ的诊治情况。患者均经ＭＲＩ证实并行手术松解;根据手术所见和影像学分析其致病机理及手术要点。结果栓系原因分别为椎管内肿瘤（６例）、脊髓脊膜膨出（６例）、终丝增粗（３例）、脊髓双裂畸形（２例）及术后粘连（１例）。经８个月至５年随访,其中１４例获得满意疗效。结论成人ＴＣＳ在临床表现及预后等方面有别于幼儿ＴＣＳ;术中应避免损伤圆锥和马尾神经;病程过长及再栓系是疗效不佳的主要原因     

Occult intraspinal anomalies in congenital scoliosis

Thirty consecutive patients with congenital spinal deformity underwent magnetic resonance imaging (MRI) to determine the incidence of occult intraspinal anomaly. These congenital spinal deformities included 29 cases of congenital scoliosis and one case of congenital kyphosis. Physical examination findings and plain radiographs were reviewed in an attempt to correlate these findings with subsequent intraspinal pathology. Nine patients had intraspinal anomalies identified on MRI consisting of five with tethered cord, four with syringomyelia, three with lipoma, and one with diastematomyelia. One patient required surgery for diastematomyelia; another underwent release of his tethered cord. Only one patient, with diastematomyelia associated with a syrinx and bifocal tethering, had his anomaly suggested by physical examination and plain radiographs. Two other patients had findings on plain radiographs previously associated with high prevalence of occult intraspinal anomalies; one patient with congenital kyphosis had a tethered cord, and one patient with a unilateral hemivertebrae associated with a contralateral bar had a tethered cord. Two of nine patients with occult intraspinal anomalies required surgery for their anomaly. In patients with a congenital spinal deformity, we found nine (30%) of 30 to have an associated anomaly within the spinal canal. Only three of these nine had plain radiographs and physical examination findings suggestive of their subsequent MRI findings. Given the poor correlation between findings on physical examination, plain radiographs, and subsequent occult intraspinal anomalies on MRI, we believe that MRI is helpful in evaluating patients with congenital spinal anomalies.     

Adult onset of tethered spinal cord syndrome due to fibrous diastematomyelia: case report

Adult onset of the symptoms of tethered spinal cord is a rare entity that is occasionally associated with diastematomyelia. Only one case of fibrous diastematomyelia in an adult has been reported. The fibrous nature of this disease may present a diagnostic difficulty. A 32-year-old man with the adult onset of impairment of sacral functions with lumbar fibrous diastematomyelia is reported. Surgical release of the spinal cord was followed by improvement of the patient's function.     

Evaluating congenital spine deformities for intraspinal anomalies with magnetic resonance imaging.

SUMMARY: The incidence of intraspinal abnormalities associated with congenital spinal anomalies as detected by magnetic resonance imaging (MRI) is becoming better defined. In this study, 41 nonrandomized children with congenital spinal deformities (excluding myelomeningocele) who underwent complete MR evaluation were reviewed. Of the 41 congenital spinal deformities, 37 demonstrated congenital scoliosis, with failure of formation in 19, failure of segmentation in 4, and mixed defects in 14. The remaining four deformities were cases of congenital kyphosis. Thirteen patients with congenital spine anomalies were noted to have intraspinal abnormalities identified by MRI: tethered cord in 12 patients, syringomyelia in 3 patients, and diastematomyelia in 5 patients. Of the 12 patients with tethered cord, 2 patients had neurologic deficits. Urorectal anomaly was one of the most common associated findings (15%). Considering an incidence of intraspinal anomalies of 31% and as clinical manifestations may not be initially detectable, MRI is recommended in patients with congenital spinal deformity as part of the initial evaluation even in the absence of clinical findings.     

Diastematomyelia presenting as progressive weakness in an adult after spinal fusion for adolescent idiopathic scoliosis.

BACKGROUND CONTEXT: Diastematomyelia is uncommon and rarely presents in adulthood. This report draws attention to the fact that patients who underwent spinal fusion for deformity before the widespread use of computed tomography (CT) and magnetic resonance imaging (MRI) may have unrecognized spinal cord abnormalities. This should be considered if revision surgery is contemplated. PURPOSE: This case report focuses on the late presentation of lower-extremity weakness in a 44-year-old woman with a split cord malformation (diplomyelia), diastematomyelia and tethered cord syndrome. STUDY DESIGN/SETTING: METHODS: The patient underwent instrumented posterior spinal fusion with a Harrington rod as a child for progressive thoracolumbar scoliosis. As an adult, she developed paraparesis after a traumatic event.The patient underwent decompressive laminectomy, subtotal resection of the old fusion mass and resection of the osseous septum. Postoperatively, an anterior spinal fluid leak in the lower thoracic region required repeated fascial grafting, resection of a pseudomeningocele and reverse left latissimus dorsi flap transfer. The leak was controlled, and the patient had near complete resolution of her paraparesis 1 year after her surgery. RESULTS: The case described herein is unusual in that patients with diplomyelia and diastematomyelia rarely are symptomatic in adulthood. However, trauma may precipitate the onset of neurologic symptoms. This patient underwent spinal surgeries to address deformity, pain and progressive lower-extremity weakness. Preoperative CT and MRI studies showed a split cord malformation and diastematomyelia at L1-L2 with spinal stenosis and tethering of both hemicords. CONCLUSIONS: Progressive weakness without any previous neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may develop in the adult with unrecognized diastemotomyelia. This case demonstrates that a thorough preoperative workup of patients with complex spinal deformities is imperative.     

Segmental vessel ligation in patients undergoing surgery for anterior spinal deformity

Segmental vessel ligation during anterior spinal surgery has been associated with paraplegia. However, the incidence and risk factors for this devastating complication are debated. We reviewed 346 consecutive paediatric and adolescent patients ranging in age from three to 18 years who underwent surgery for anterior spinal deformity through a thoracic or thoracoabdominal approach, during which 2651 segmental vessels were ligated. There were 173 patients with idiopathic scoliosis, 80 with congenital scoliosis or kyphosis, 43 with neuromuscular and 31 with syndromic scoliosis, 12 with a scoliosis associated with intraspinal abnormalities, and seven with a kyphosis. There was only one neurological complication, which occurred in a patient with a 127 degrees congenital thoracic scoliosis due to a unilateral unsegmented bar with contralateral hemivertebrae at the same level associated with a thoracic diastematomyelia and tethered cord. This patient was operated upon early in the series, when intra-operative spinal cord monitoring was not available. Intra-operative spinal cord monitoring with the use of somatosensory evoked potentials alone or with motor evoked potentials was performed in 331 patients. This showed no evidence of signal change after ligation of the segmental vessels. In our experience, unilateral segmental vessel ligation carries no risk of neurological damage to the spinal cord unless performed in patients with complex congenital spinal deformities occurring primarily in the thoracic spine and associated with intraspinal anomalies at the same level, where the vascular supply to the cord may be abnormal.     

成人脊髓栓系综合征的诊治

目的研究成人脊髓栓系综合征（ＴＣＳ）的临床诊断和治疗。方法回顾性报告１８例成人脊髓栓系综合征的临床资料。所有患者均经ＭＲＩ证实并行手术松解;根据手术所见和影像学分析其致病机理及手术要点。结果脊髓或圆锥的栓系原因各不相同,包括椎管内肿瘤（６例）、脊髓脊膜膨出（６例）、终丝增粗（３例）、脊髓双裂畸形（２例）及术后粘连（１例）。经８个月～５年随访,其中１４例获得满意疗效。结论成人ＴＣＳ在临床表现及预后等方面有别于幼儿ＴＣＳ。术中应避免损伤圆锥和马尾神经。病程过长及再栓系是疗效不佳的主要原因。     

先天性椎管内畸形分类及手术处理

目的回顾性分析一组椎管内畸形病例的临床资料,探讨此类疾病的诊治策略和治疗效果。方法 2002年3月至2009年3月收治椎管内畸形患者80例,男36例,女44例,年龄1个月至71岁。脊柱裂70例,脊髓拴系综合征55例。脊髓纵裂11例,椎管内脂肪瘤19例。椎管内皮样囊肿5例。脊膜膨出14例,脊髓脊膜膨出20例,脂肪性脊髓脊膜膨出12例。合并有2种或2种以上畸形78例,仅2例为单纯椎管内畸形。所有病例分别按照脊膜膨出、椎管内脂肪瘤、脊髓纵裂的特殊方法进行手术。术前、术后随访均拍摄MRI作为研究资料。采用综合感觉、运动和排尿功能评估及手术操作是否达到目的作为疗效评估手段。结果结合MRI对80例患者进行病理性分析,大部分为脊柱裂合并的畸形。80%脊膜膨出和脊髓拴系病例获得功能改善。而椎管内脂肪瘤、二分脊髓近半数的病理未见明显效果。结论脊柱裂伴发椎管内畸形应早期发现,正确详尽诊断,综合评估,尽早医学介入,严格掌握手术适应证。     

Uterus-like mass of müllerian origin in the lumbosacral region causing cord tethering. Report of two cases

The presence of müllerian-origin tissue in the lumbosacral region is extremely uncommon. The authors report two cases of müllerian-origin tissue in that region. In the first case a 33-year-old woman harbored a conus medullaris mass lesion. Spinal dysraphism, tethered cord syndrome (TCS), and diastematomyelia were also present. In the second case a 24-year-old woman presented with low-back pain and a conus medullaris lesion, which was a cause of the TCS. Pathological examination in both cases revealed a uterus-like structure with evidence of fresh and old hemorrhage. The rarity of this lesion and its association with diastematomyelia requires documentation.