脊髓末端积水合并脊髓栓系综合征10例临床研究

目的总结脊髓末端积水合并脊髓栓系综合征的临床表现、影像学特点、诊断与治疗经验。方法与结果 10例脊髓末端积水合并脊髓栓系综合征患者(1例为全脊髓积水、8例为骶管内脊膜囊肿、1例合并骶部皮毛窦),临床表现为不同程度双下肢无力,尤以肢体远端显著,5例伴尿道和肛门括约肌功能障碍;5例存在与脊髓空洞平面相关的感觉障碍平面,尤以浅感觉减退为主。术前MRI显示脊髓低位、脊髓末端无圆锥结构、脊髓和终丝内异常信号。于手术显微镜下切断终丝、引流髓内积水、切除骶管内脊膜囊肿、松解脊髓栓系。手术成功率达100%,平均手术时间2.15 h、术中出血量220 ml。无一例发生手术相关并发症。术后视觉模拟评分降低,下肢肌力、尿道和肛门括约肌功能改善。术后平均随访6.10年,Mc Cormick神经功能分级均达到Ⅰ级。末次随访时复查MRI显示,脊髓圆锥结构恢复,脊髓末端上升,髓内积水消失,脊柱生理曲度未发生变化。结论脊髓末端积水合并脊髓栓系综合征临床罕见,主要表现为慢性脊髓功能障碍;MRI特征性表现为脊髓低位,圆锥结构消失,终丝和髓内呈长T1、长T2信号,囊壁无强化。于手术显微镜下切断终丝、引流髓内积水、松解脊髓栓系,效果满意。     

21例儿童脊髓栓系综合征的诊断和治疗

目的:讨论儿童脊髓栓系综合征的诊断及治疗。方法:回顾性分析21例脊髓栓系综合征的临床资料、诊断依据、X线平片及MRI。手术方法均采用终丝切断术。结果:外观上腰背部隐性脊柱裂特有的皮肤改变16例,X线平片18例有隐性脊柱裂,MRI显示终丝脂肪变性9例,纤维变性12例,圆锥低位13例,正常位置8例,均予手术治疗。除1例无变化外,其余患者症状均有改善或恢复正常。结论:脊髓栓系综合征的诊断靠临床表现,脊柱平片及MRI,终丝有否病变是诊断的标准,而圆锥低位不是判断有无脊髓栓系综合征及是否需要手术的唯一关键指标。对于终丝变性,无论有无神经损害症状,均应尽早手术,手术方法终丝切断。     

硬膜内、外腔联合松解术治疗脊髓栓系综合征的疗效

目的 探讨硬膜内、外腔联合松解术治疗脊髓栓系综合征的临床疗效。方法 回顾性分析1998年1月至2016年6月手术治疗的286例脊髓栓系综合征患儿的临床资料,采用常规手术治疗77例（常规组）,采用硬膜内、外腔联合松解术治疗209例（联合松解组）。结果 常规组术后并发症发生率（10.4%,8/77）与联合松解组（6.2%,13/209）无统计学差异（P>0.05）。常规组术后6个月Hoffman分级较术前无明显差异（P>0.05）。联合松解组术后6个月Hoffman分级较术前明显改善（P<0.05）,而且明显优于常规组（P<0.05）。术后6个月,常规组显效7例,改善6例,稳定43例,加重21例;联合松解组显效30例,改善32例,稳定132例,加重15例;联合松解组疗效明显优于常规组（P<0.05）。结论 对于脊髓栓系综合征,在硬膜内腔脊髓栓系松解的基础上,探查硬膜外腔,松解硬膜外腔粘连栓系、离断外终丝,可进一步提高手术效果     

终池成形术治疗脊髓栓系综合征的疗效观察

目的 探讨显微神经外科技术并终池成形治疗脊髓栓系综合征的方法和疗效。方法 对42例脊髓栓系综合征患者采用显微手术彻底松解栓系的脊髓．并根据术中情况做终池成形。结果 术后第一天23例患者即有症状的缓解，住院期间34例患者症状得到明显的改善，在随访期内未有术后粘连引起再栓系。结论 采用显微手术彻底松解栓系的脊髓，并根据术中情况做终池成形可明显提高脊髓栓系综合征的手术效果，并可有效的预防术后粘连引起再栓系。     

儿童脊髓栓系综合征的显微外科治疗

目的探讨儿童脊髓栓系综合征的临床特点、治疗方法和预后。方法回顾性分析21例脊髓栓系病儿的临床资料,均在显微镜下行脊髓栓系松解手术。结果 21例病儿成功行脊髓栓系松解术。MRI提示脂肪瘤大部分切除。术后随访3个月~3年,症状均有改善。结论儿童脊髓栓系早期手术治疗能取得较好的预后,待病儿出现明显并发症后再手术的效果较差。     

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。     

脊髓拴系综合征的诊断和治疗体会

目的探讨脊髓拴系综合征的诊断和治疗。方法采取扩大椎板切除、黏连松解、肿瘤切除、终丝切断和适当地塞米松蛛网膜下腔注入的方法治疗22例脊髓拴系综合征病人。结果病人术后感觉、运动、大小便功能均有不同程度改善，儿童和年轻病人术后效果较好。结论MRI检查是诊断脊髓拴系的重要方法和手段，诊断明确后应尽早手术。并发症的预防是防止复发的关键。     

复杂型脊髓拴系综合征患者的诊治策略

目的 研究复杂型脊髓拴系综合征患者的临床症状、影像学表现、治疗策略等.方法 回顾性分析89例复杂型脊髓拴系综合征患者的临床资料,通过分析造成脊髓拴系的原因,采取不同的手术策略,达到完全松解脊髓拴系的目的,从而探索复杂型脊髓拴系综合征的个体化诊治策略.结果 复杂型脊髓拴系综合征中造成脊髓拴系的主要原因包括终丝病理性增粗及...     

显微松解术治疗26例儿童脊髓栓系综合征疗效分析

目的观察显微松解术对小儿脊髓栓系综合征的治疗效果。方法选择26例脊髓栓系综合征行显微松解术患儿为研究对象,回顾分析其治疗效果。结果 26例患儿中,18例自感症状明显减轻;术前存在大、小便异常症状的21例患儿,术后13例症状显著改善。术前存在下肢运动及感觉功能障碍症状的13例患儿,术后6例下肢感觉功能增强,4例运动障碍明显改善。结论采用显微松解手术治疗脊髓栓系综合征,可显著改善患者的大小便异常、下肢功能障碍等症状,有较高的临床使用价值。     

应用游离骶囊术治疗新生儿巨大型骶尾部脊髓脊膜膨出的疗效观察

脊髓脊膜膨出是由于胚胎期神经轴中胚叶发育缺陷、神经管闭合不全形成脊柱裂所致。它常合并脊髓圆锥下移，终丝粗大、短缩，尤以骶尾部巨大型脊髓脊膜膨出发生率高。所以此类患儿出生时多已合并脊髓栓系的症状。如果在一期修补术的同时，采取游离骶囊的方法，切断外终丝，松解骶管内的粘连，则会在早期制止脊髓栓系综合征的发生，我们自2002年至今对9例新生儿采取了此术式，现报告如下。     

脊髓栓系综合征的MRI表现及临床意义

目的探讨脊髓栓系综合征的MRI特征,对其进行分型,评价其诊断意义。方法对50例脊髓栓系综合征MRI特征回顾性分析。结果脊髓圆锥的位置均位于第三腰椎平面及以下部位。根据脊髓牵张的原因将其MRI表现分为以下7种类型:脂肪瘤型13例;脊髓纵裂型6例;脊髓脊膜膨出型11例;脊膜膨出型3例;单纯终丝增粗型8例;肿瘤型1例;混合型8例。50例中33例出现中央管扩张或脊髓空洞症,脊柱裂42例。结论 MRI是评价脊髓栓系综合征的非常有价值的方法,MRI分型对于判断预后及指导手术治疗具有重要意义。     

脊髓栓系伴神经原性膀胱的治疗策略

目的探讨脊髓栓系综合征伴有神经原性膀胱的跨学科协作治疗。方法本组脊髓栓系综合征患者24例,年龄4~47岁,平均35.1岁,男10例,女14例。手术前后行尿流动力学测定,神经外科手术分离粘连、松解脊髓,修补缺损及硬脊膜重建。排尿障碍改善不佳者行尿道支架置入及骶神经调控器植入术。结果术前24例患者均诊断为神经原性膀胱,包括逼尿肌无力9例,扩约肌-逼尿肌共济失衡15例。病理诊断包括脂肪瘤8例,表皮样囊肿5例,先天性脊髓脊膜膨出11例。随访8~36月,19例患者术后尿流动力学检查有显著改善;3例放置尿道支架,2例行骶神经调控后改善。所有患者均无神经系统症状加重。结论伴有神经原性膀胱的脊髓栓系综合征应早期行栓系松解术,排尿障碍改善不明显者放置尿道支架或行骶神经调控术。早期诊断及成立医疗协作小组是积极有效的,治疗的最终目的是阻止神经病变加重及预防可能发生的上尿路系统损害。     

The specificity and sensitivity of transcranial ultrasound in the differential diagnosis of Parkinson's disease: a prospective blinded study

BACKGROUND: Increased echogenicity of the substantia nigra (SN), as determined by transcranial sonography (TCS), is characteristic of idiopathic Parkinson's disease (iPD). The results of initial retrospective studies indicate that this ultrasound sign is specific for iPD and can help to differentiate it from atypical parkinsonian syndromes (aPS); however, these early studies were done in patients with later disease stages and known clinical diagnosis. We aimed to determine the diagnostic value of TCS in the early stages of parkinsonian syndromes, when the clinical symptoms often do not enable a definite diagnosis to be made. METHODS: 60 patients who presented with the first, but still unclear, clinical symptoms of parkinsonism had TCS in this prospective blinded study. Investigators were blinded to the results of the clinical investigations, the ultrasound findings, and the diagnosis at time of investigation. The patients were followed-up every 3 months for 1 year to assess and re-evaluate the clinical symptoms. The patients in whom a clinical diagnosis could not be made with certainty were investigated with raclopride PET or dopamine transporter single-photon emission computed tomography (SPECT), or both. FINDINGS: A clinical diagnosis of parkinsonism could not be established at baseline in 38 patients. At 12 months, 39 patients were clinically categorised as having iPD. Compared with endpoint diagnosis, the sensitivity of TCS at baseline was 90%7% and the specificity was 82.4%; the positive predictive value of TCS for iPD was 92.9% and the classification accuracy was 88.3%. INTERPRETATION: TCS is an easy to implement, non-invasive, and inexpensive technique that could help in the early differential diagnosis of parkinsonian syndromes. The routine use of TCS in the clinic could enable disease-specific therapy to be started earlier. FUNDING: Michael J Fox Foundation for Parkinson's Research.     

What is the true tethered cord syndrome?

Introduction The tethered cord syndrome (TCS) is a stretch-induced functional disorder of the spinal cord with its caudal part anchored by an inelastic structure. Discussion This article clarifies the reversible lesions that occur in the cord segments above any of the inelastic abnormalities. These lesions are found mostly in the lumbosacral cord, occasionally in the cervical cord and closely correlate with clinical findings. Imaging studies alone do not allow accurate diagnosis of the TCS. The authors emphasize the importance of adhering to the physiological terms “tethered cord syndrome” and “tethered spinal cord” to avoid controversies derived from terms that are not based on the pathophysiology of TCS.     

31例脊髓拴系综合征患者的远红外热像特征分析

目的分析脊髓拴系综合征患者的远红外热像特征。方法我院诊断出的脊髓拴系综合征患者31例,暴露检查部位,应用ATIR-M301B非制冷焦平面远红外热像仪,采取不同部位远红外热图,测量远红外热像图中同部位两侧的温度差,并进行统计学分析。结果脊髓拴系综合征患者检测部位的远红外热像显示,双下肢远红外热像图呈左右不对称分布,主要呈缺血性改变。肌肉萎缩的下肢呈低温改变,腰骶部脂肪沉积的呈低温改变;足部溃疡处呈高温改变;既往曾行手术的瘢痕处呈高温改变。红外热像检查结果与患者主诉和主要异常体征检查基本一致。结论远红外热像检查能较好的反映出脊髓拴系综合征患者身体相应部位的异常温度变化,为临床诊治该类疾病提供功能学影像依据,是对CT和MRI及超声等组织形态学影像技术的重要补充。     

Surgical Management and Outcome of Tethered Cord Syndrome in School-Aged Children, Adolescents, and Young Adults

Objective

The adolescent presentation of tethered cord syndrome (TCS) is well-recognized, but continues to pose significant diagnostic and management controversies. The authors conducted a retrospective study of clinical outcomes after surgical intervention in 24 school-aged children, adolescents, and young adults with TCS.

Methods

All 83 patients with a lipomyelomeningocele (LMMC) underwent untethering surgery for caudal cord tethering between 1987 and 2007. The clinical charts and follow-up data were reviewed. Of these patients, 24 school-aged children, adolescents, and young adults with TCS were studied with respect to the clinical, radiologic, pathologic features, and surgical outcomes.

Results

Untethering procedures were performed in 24 patients (age range, 7-25 years) for TCS of various origins (lipoma, lipomyelomeningocele, and tight filum terminale). Specific circumstances involving additional tugging of the already tight conus, and direct trauma to the back precipitated the onset of symptom in 50% of the patients. Diffuse and non-dermatomal leg pain, often referred to the anorectal region, was the most common presenting symptom. Progressive sensorimotor deficits in the lower extremities, as well as bladder and bowel dysfunction, were also common findings, but progressive foot and spinal deformities were noted less frequently. The most common tethered lesions were intradural lipomas, thickened filum and fibrous band adhesions into the placode sac. The surgical outcome was gratifying in relation to pain and motor weakness, but disappointing with respect to resolution of bowel and bladder dysfunction. Of the 24 patients with TCS, pre-operative deficits improved after surgery in 14 (58.3%), remained stable in 8 (33.4%), and worsened in 2 (8.3%).

Conclusion

The pathologic lesions of tethered cord syndrome in school-aged children, adolescents, and young adults, are mostly intradural lipomas and tight filum. It is suggested that the degree of cord traction results in neurologic dysfunction in late life due to abnormal tension, aggravated by trauma or repeated tugging of the conus during exercise. Early diagnosis and adequate surgical release might be the keys to the successful outcome in school-aged children, adolescents, and young adults with TCS.     

痉挛型脑瘫合并脊髓栓系综合征Ⅰ期显微外科手术治疗的长期随访

目的探讨Ⅰ期显微外科手术治疗痉挛型脑瘫合并脊髓栓系综合征(TCS)的疗效。方法回顾性分析50例痉挛型脑瘫合并TCS病人的临床资料,均行Ⅰ期显微外科手术治疗,即选择性腰骶段脊神经后根部分切断术(SPR)、硬脊膜囊尾端松解术及内外终丝切断术。结果所有病人随访36~130个月,平均65个月。下肢痉挛缓解率为100%,步态功能改善率为92%,小便功能改善率为77.4%,大便功能改善率为73.7%,生活质量提高率为100%。术后并发症:下肢感觉障碍18侧,肌无力15侧,长期腰痛5例,随访期间均好转;大小便功能障碍加重2例,随访期间稍改善。结论显微外科Ⅰ期手术治疗痉挛型脑瘫合并TCS,可同时缓解下肢痉挛状态和改善大小便功能,是安全、有效的术式。     

Tethered cord syndrome in children - impact of surgical treatment on functional neurological and urological outcome

BACKGROUND AND PURPOSE: Tethered cord syndrome (TCS) consists of solid adhesion of the distal spinal cord to adjacent structures, resulting in repetitive spinal cord traction during truncal movements. The condition is usually a sequel of lumbosacral dysraphism, spinal cord injury or surgical procedure. Clinical signs of TCS include sphincter disturbances, lumbosacral pain, sensorimotor deficits and orthopaedic deformity. The aim of this study was to assess long-term results of surgical treatment of TCS in children and to define any prognostic factors associated with long- -term outcome. MATERIAL AND METHODS: Between 1980 and 2005, we treated 59 children with TCS. The group included 22 boys and 37 girls (mean age at surgery: 7.7 years). In total, 75 surgical procedures were performed. Mean follow-up time was 4.4 years. RESULTS: Meaningful clinical improvement was obtained in 19 children (32.2%) and was more pronounced in lower extremities than in sphincters. Degree of untethering was the only factor significantly influencing outcome. Neither age at surgery nor severity of spinal cord traction had any prognostic value. Significant correlations were found between severity of pre-existing myelopathy and development of neurogenic bladder, independently of treatment instituted. There was no permanent morbidity and perioperative mortality was nil. CONCLUSIONS: Late results of surgical treatment of TCS in children are generally unsatisfactory - worthwhile improvement was obtained in about 1/3 of patients only. Improvement was more pronounced in lower extremities than in sphincters. The sole factor contributing to good functional outcome was complete untethering of the cord. The decision whether to operate on a child with TCS must be based on coherent clinical, radiological and urological premises; surgery should be performed before irreversible deficits appear.     

脊髓栓系综合征手术前后尿动力学研究

目的 研究脊髓栓系综合征患者手术前后尿动力学改变.方法 选择21例脊髓栓系综合征患者,手术前后的尿动力学检查指标包括膀胱初感觉容量、膀胱顺应性、最大逼尿肌压力、最大尿流率、残余尿量、逼尿肌反射.结果 21例患者中,术前尿动力学检查正常的2例;异常的19例,其中顺应性正常膀胱13例,低顺应性膀胱4例,高顺应性膀胱2例;逼尿肌反射正常3例,无逼尿肌反射8例,逼尿肌亢进5例,逼尿肌减退3例;术后2周内尿动力学复查结果显示膀胱顺应性、逼尿肌反射、膀胱初感觉容量与术前相比,差异无统计学意义;术后残余尿量较术前明显减少,差异有统计学意义(P＜0.05).结论 脊髓栓系综合征患者常有明显的膀胱功能障碍;手术可以改善患者的部分排尿功能;尿动力学检查对评估脊髓栓系综合征患者手术前后的膀胱功能有重要意义.