脊髓栓系的尿动力学表现及临床意义

目的：探讨及评价脊髓栓系的尿动力学表现及临床意义。方法：对66例脊髓栓系患儿行尿动力学检查、MRI和排泄性膀胱尿道造影。结果：58例患儿发现有尿动力学异常改变，逼尿肌反射亢进35例，反射低下25例，逼尿肌括约肌不协调27例。54例膀胱顺应性，逼尿肌压增加，53例 残余尿增加，51例膀胱容量减少。8例尿动力学表现正常。脊髓圆锥位置正常5例，位于L3－L5间32例，L5以下29例。输尿管反流16例。结论：脊髓栓系可导致尿动力学发生不同的异常改变，脊髓圆锥位置与尿动力学表现的类型及上尿路损害无相关关系，上尿路损害与逼尿肌括约肌不协调、逼尿肌压和患儿的年龄密切相关。尿动力学检查是评价下尿路功能和治疗的客观指标。     

Split cord malformation as a cause of tethered cord syndrome in a 78-Year-old female

A 78-year-old woman presented for evaluation of back pain, urinary dysfunction, leg weakness and progressive equinovarus foot deformity. She reported that shortly after her birth in 1924, she underwent resection of a subcutaneous 'cyst' in the lower lumbar area. Seven years prior to evaluation at our institution, she had undergone bilateral total knee arthroplasty for osteoarthritis. After the procedure, she began to experience severe low back pain that radiated into her legs. Weakness of the foot inverters, urinary dysfunction and worsening bilateral equinovarus foot deformity developed in the years following the surgery. MRI revealed a split cord malformation with a tethered spinal cord. Because of the patient's age and poor medical condition, her symptoms were managed conservatively. This case demonstrates symptomatic deterioration in an elderly patient with a tethered spinal cord after many years of clinical stability.     

The tethered cord syndrome

The "tethered cord syndrome" as a complication of spinal dysraphism is probably more important than assumed earlier. An abnormally low position of the conus medullaris is caused by different anomalies: e.g. adhesions, lumbosacral lipoma, tight filum terminale. In some patients no skinny changes can be detected. A "tethered cord syndrome" should be considered, if neuromuscular skeletal changes as club-foot, scoliosis, muscular atrophy of disturbances of gait, sensibility or function of bladder and rectum are recognized. It is also a result of a inadequately operated meningomyelocele. For experienced examiners sonography is an interesting non-invasive diagnostic procedure during infancy. The diagnosis should be completed by spinal computerized tomography and myelography. Surgery should be performed prophylactically. The "tethered cord syndrome" is explained by case histories of the University Hospital of Children, Freiburg.     

Horizontal sacrum as an indicator of a tethered spinal cord

The horizontal sacrum has never been described as an indicator of a tethered spinal cord following myelomeningocele closure. We retrospectively analyzed 30 children with a myelomeningocele for this progressive change and its correlation with symptoms. At least two lateral radiographs over time were examined and the lumbosacral angle (LSA) was measured. We found that the majority of these children had an LSA that was greater than would be expected in patients with late and decreased ambulatory abilities. Also, we observed that the LSA was often inappropriately increased at the time many of these patients presented with symptoms indicative of a tethered spinal cord. We hypothesize that the LSA in this group of children is altered by a tethered spinal cord and that the horizontal nature of the sacrum may predate the clinically appreciable symptoms of a tethered spinal cord.     

The importance of early recognition of impeded cranial migration of the spinal cord]

Impeded cranial migration of the spinal cord is described in five cases which initially were not recognized. The aetiology, symptomatology and differential diagnosis are presented. The clinical manifestations are signaled by skin lesions typical for dysraphic lesions, signs of urinary and rectal incontinence, deformity or weakness of the feet, impaired gait, motor reflex and sensory changes, and occasionally trophic changes. The restriction in cranial migration of the spinal cord can only be proved by myelography. The importance of early neurosurgical intervention to prevent irreversible deterioration of the motor, sensory or urinary system is particularly emphasized. Improvement after surgery can be achieved in at least 30% of the patients who already developed neurological deficits.     

大鼠胚胎神经干细胞移植治疗类脊髓栓系综合征的实验研究

目的将胎鼠的神经干细胞移植到脊髓栓系综合征大鼠的病变脊髓中，观察治疗效果。方法从孕17d大鼠胚胎脊髓中分离、培养神经干细胞并诱导分化，通过免疫组化技术研究证实其特性。制作大鼠脊髓栓系模型，3d后将未分化的神经干细胞注人病变脊髓内，2个月后观察大鼠的运动功能，处死大鼠，研究移植后的干细胞在体内存活、迁移及分化情况。结果实验中分离、培养的神经干细胞能够被诱导分化成神经元、星形胶质细胞和少突胶质细胞。脊髓栓系大鼠移植干细胞后运动功能改善，神经元数目增多，显著好于未移植组。免疫组化方法证实移植后的干细胞在体内可以存活、迁移、分化成神经细胞。结论大鼠胚胎神经干细胞体内、体外均具有多向分化潜能，移植神经干细胞是治疗脊髓栓系神经变性的一种有效途径。     

Clinical outcome in children undergoing tethered cord release utilizing intraoperative neurophysiological monitoring

Release of tethered spinal cord by sectioning of the filum terminale carries a risk of injuring neighboring motor and sensory nerve roots involved in bowel and bladder control. Therefore, intraoperative neurophysiological monitoring techniques have been developed to prevent neurological complications postoperatively. We performed a retrospective chart review of 63 patients who had undergone tethered cord release. We excluded adult patients, those lost to follow-up and patients with either a myelomeningocele and/or lipoma. This limited our study to 25 pediatric patients, aged 4 months to 12 years, who underwent tethered cord release for either a thickened filum terminale and/or a low-lying conus. For intraoperative monitoring, we utilized electrical stimulation of the filum terminale, lumbosacral nerve roots and electromyography recordings. Ventral nerve roots were identified and their electrical thresholds obtained. The mean was 0.32 V, the mode 0.1 V and the range 0.05-1.0 V. These values were compared to electrical thresholds obtained by stimulation of the filum terminale. The mean was 26.1 V, the mode 20.0 V and the range 8-100 V. In over 70% of patients, muscle activation via the filum required 100 times the voltage needed to activate a motor root. This motor root to filum threshold of 1:100 was useful in identifying the filum. Clinical outcome showed no significant worsening with respect to bowel and bladder control or pain and motor indices. Significant bowel and bladder improvement was seen in 4 out of 25 patients, motor improvement in 9 out of 25 patients and improvement of pain in 4 out of 25 patients. Three patients developed postoperative urinary tract infections, but no cerebrospinal fluid leaks or pseudomeningoceles were encountered. These results suggest that patients with a thickened filum or low-lying conus can safely undergo tethered cord release. Intraoperative neurophysiological monitoring provides a helpful adjunct to distinguish nerve roots from the filum. A ratio, rather than an absolute number, is beneficial in distinguishing motor roots from the filum and eliminates variability due to patients' individual differences in electrical thresholds.     

Cutaneomeningospinal angiomatosis (Cobb syndrome) with tethered cord.

A newborn presented with a skin-covered lumbar mass with a subcutaneous hemangioma and on a magnetic resonance image (MRI) revealed a tethered spinal cord with a local mass. The mass had signal characteristics compatible with a lipoma. An initial diagnosis of a lipomeningocele with tethered cord was made, and the patient underwent surgical exploration and subtotal resection of the mass. A follow-up MRI revealed that the cord was still tethered, but an additional mass was present. The initial mass with signal characteristics of lipomatous tissue was accompanied by a low-signal mass in the lumbosacral canal, ventral to the cord, and bilateral enlargement of the foramina at the lumbosacral level. Because of a concern for an intraspinal tumor, a second operative intervention was performed. Multiple biopsies of the mass inside the spinal cord, the nerve roots and at the level of the foramina revealed angiomas that had similar pathology in all the specimens. A partial resection of the masses and a release of the tethered cord was performed by sectioning the thickened filum terminale. The diagnosis of Cobb's syndrome was made. The unique association of a tethered cord and the Cobb syndrome is reported here.     

Toward the prediction of neurological injury from tethered spinal cord: investigation of cord motion with magnetic resonance

Experience with spinal dysraphism has taught that spinal cord tethering will eventually produce neurological deficit. The primary diagnostic and monitoring tool, magnetic resonance (MR) scanning, almost always produces images consistent with cord tethering, even in asymptomatic or improved, previously operated patients. This presents a problem in selecting appropriate candidates for prophylactic intervention. Ideally, a method of monitoring should predict functional deterioration. Real-time ultrasonography, electrophysiological tests, and phase-motion MR studies could potentially be adapted for this purpose. The authors have evaluated the latter technique in both symptomatic and asymptomatic dysraphic patients with tethering. Studies of cervical cord motion show a clear separation between results in patients with cord expansion from hydromyelia and those with pure tethering. Symptomatic patients with progressive deficit or pain have limited cervical cord motion which improves after surgical untethering. MR studies of cord motion have definite promise in the prediction of deficit from spinal cord tethering and should be further evaluated in comparison with ultrasound and electrophysiologic methods.     

神经分离松解术治疗脊髓栓系综合征的疗效评价

目的：对脊髓栓系综合征手术治疗的效果进行探讨。方法：通过54例手术，针对不同的病理因素，解除栓系，分离粘连，梳理神经。结果：术后临床症状改善者33例，无变化18例，加重3例，26例进行了体感诱发电位（SEP）的观察，术后均有明显好转。3例术后MRI显著脊髓末端位置无明显变化。结论：脊髓栓系综合征提倡手术，手术后恢复是一个长期的慢性过程；大小便功能恢复的测定。下肢及足畸形肌电的改善都是非常复杂的。     

脊髓栓系综合征电生理检测特点及临床意义

利用电生理检测技术,评价脊髓栓系综合征患儿神经功能改变。方法对ＴＣＳ患儿组及对照组行胫后神经体感诱发电位、胫后神经及腓总神经运动神经传导速度检测。结果ＴＣＳ患儿组ＰＴＮ－ＳＥＰ检测出现皮层电位潜伏期延长,波幅下降甚至消失,部分马尾电位波幅消失;ＭＣＶ轻、中度减慢、动作电位波幅下降,甚至出现传导阻滞,病变随病情而加重。     

Bowel function after surgery for anorectal malformations in patients with tethered spinal cord

Tethered spinal cord (TC) is an anomaly frequently recognized in association with anorectal malformations (ARM). However, the influence of TC on bowel function in children with ARM remains unknown. Furthermore, there are few studies that have assessed anorectal function in children with ARM and TC. The aim of this study was to evaluate anorectal function in ARM patients with TC using clinical assessment and anorectal manometry. Among 258 patients with ARM, this retrospective investigation included 35 patients who underwent spinal magnetic resonance imaging (MRI) after surgery for ARM. The patients were divided into two groups based on the presence or absence of TC, and bowel function was assessed by Kelly’s clinical score and anorectal manometry. Tethered cord was found in nine of the 35 patients (26%) with ARM. Of the ARM patients, TC was noted in four of 11 (36%) with high type anomalies, one of 8 (13%) with intermediate type anomalies, two of 14 (14%) with low type anomalies, and two of two patients (100%) with cloacal anomalies. Kelly’s clinical score did not significantly differ between the two groups. However, two of the nine patients with TC had poor bowel function (Kelly’s score; 2–0 points). On the contrary, patients without TC did not have poor bowel function. Anorectal manometry did not show a significant difference between patients with and without TC. However, the two patients with TC who had poor bowel function by Kelly’s score had low anal resting pressure, which was essential for achieving fecal continence. In conclusion, the present study showed that tethered cord was more frequently found in patients with more severe anorectal anomalies. Patients with TC were more likely to have poor bowel function, but this did not reach statistical significance. Presented at the 14th International Paediatric Colorectal Club, York, UK, 14–16 July 2007.     

超声吸引装置在脂肪瘤型脊髓拴系综合征中的应用研究

目的探讨超声吸引装置(Cavitron ultrasonic surgical aspirator,CUSA)在脂肪瘤型脊髓拴系综合征中的应用价值。方法收集2016年5月至2018年11月于湖南省儿童医院神经外科收治的62例脂肪瘤型脊髓拴系综合征患儿作为研究对象,按是否采用CUSA技术分为常规组(n=47)和CUSA组(n=15),对比两组患儿手术时间、术后并发症及预后情况。结果 CUSA组手术时间为(120±43)min,常规组为(220±40)min,差异有统计学意义(t=8.281,P<0.05)。CUSA组并发症发生率为0 (0/15),常规组并发症发生率为12.7%(6/47),差异无统计学意义(X^2=2.12,P=0.321)。常规组中症状加重4例,分别为大小便功能障碍和双下肢畸形,改善10例,稳定33例。CUSA组无一例症状加重;需间歇导尿1例,3个月随访时排尿功能恢复;改善4例;稳定11例。结论通过采用显微外科技术,术中使用CUSA切除脂肪瘤组织等综合措施,可缩短手术时间,减少脂肪瘤型脊髓拴系患儿术后并发症。     

小儿脊髓栓系综合征

目的 探讨小儿脊髓栓系综合征（ＴＣＳ）的病理及手术治疗效果。方法 本组ＴＣＳ患儿２１例,检查确诊后,手术切除脂肪瘤或切除瘢痕、松解粘连、切断终丝,使ＴＣＳ得以松解。结果 ＴＣＳ在大体病理上有脂肪瘤型及瘢痕型两大类,部分病例有神经根缺失。１９例得到随诊,单纯双下肢软瘫的１例治愈,１７例尿失禁患儿中,３例治愈,６例明显好转,８例无变化。结论 ＴＣＳ患儿应尽早治疗,疗效确切。对脊髓脊膜膨出患儿应在ＴＣＳ     

小儿脊髓拴系综合征的诊断与治疗

为探讨小儿脊髓拴系综合征（ＴＣＳ）的发病机理、临床表现、诊断和治疗，对１９８６年６月～１９９４年６月手术治疗的４６例患儿进行分析。临床凡有排尿、排便障碍，下肢感觉、运动障碍，足畸形等症，体检发现隐性脊柱裂、显性脊柱裂（脊髓脊膜膨出）或曾行脊膜膨出手术的患儿均可疑为该病。磁共振（ＭＲＩ）和Ｍｅｔｒｉｚａｍｌｕｅ脊髓造影ＣＴ检查可确定诊断。本组术后随访１～８年，手术有效率５２％。该病一经确诊即应尽早行脊髓拴系松解术，以缓解症状，减少神经系统病残率。     

Predictors of urinary continence following tethered cord release in children with occult spinal dysraphism

ObjectiveChildren with occult spinal dysraphism represent a wide spectrum of patients. Previous studies assessing urologic outcomes have in part been deficient due to the inability to appropriately categorize these patients and gather long-term follow-up data. In this study, a uniform set of patients that had occult spinal dysraphism with magnetic resonance imaging findings of a fatty filum terminale (FF) and/or low-lying cord (LLC) was identified. Utilizing long-term follow-up data, predictors for achieving urinary continence following tethered cord release (TCR) were determined.MethodsA retrospective chart review of pediatric patients with a diagnosis of tethered cord who underwent TCR from 1995 to 2005 was performed. Analysis was limited to patients who had primary TCR by one of two neurosurgeons within our multidisciplinary spina bifida clinic, who had greater than 1-year follow-up, and who were old enough to have continence status assessed (age > 6 years unless definitively toilet trained earlier). Patients with other associated forms of spinal dysraphism (lipomyelomeningeocele, spinal lipomas, sacral agenesis), anorectal malformations, and genitourinary anomalies were excluded. Pre- and post-TCR urodynamics, radiographic studies, functional orthopedic status, and urologic outcomes were assessed. Urodynamic results were categorized by three blinded urologists into one of three urodynamic patterns: (1) normal, (2) indeterminate, and (3) high risk.ResultsA total of 147 patients with FF and/or LLC that underwent TCR were reviewed. 51 patients were excluded because of another associated spinal dysraphism (15/51 patients) or an anorectal/genitourinary anomaly (36/51 patients). Fifty-nine of the remaining 96 patients had adequate long-term follow-up data to be included in the study. 20 patients were asymptomatic at the time of TCR while 39 presented with orthopedic and/or urologic symptoms. The average age at surgery was 59.3 months (range 2–277 months) with an average follow-up of 7.0 years (range 1–16 years). At latest follow-up, 47 (80%) patients were continent while 12 (20%) were either incontinent or utilizing clean intermittent catheterization (CIC). Statistical analysis revealed that age of untethering, type of cutaneous lesion, level of conus, presence of hydronephrosis, and high-grade vesicoureteral reflux (VUR) were not independent predictors of continence. In patients with a cutaneous lesion who were asymptomatic, 19/20 obtained continence post-TCR (*p = 0.036). In patients who were old enough to assess continence pre-TCR, 14/25 patients were continent pre-TCR and 11/25 were incontinent. Of the 14 who were continent pre-TCR, all remained continent post-TCR (*p = 0.002). Of the 11 who were incontinent pre-TCR, five (45%) eventually became continent post-TCR. Assessment of urodynamic data revealed that neither pre- nor post-TCR urodynamics predicted continence status.ConclusionIsolated cutaneous lesions and preoperative continence status are positive predictors for post-TCR continence. While pre- and post-TCR urodynamics do not predict continence status, their utility in preoperative work-up, monitoring for retethering, and long-term urologic follow-up requires further examination.     

Prevalence of primary tethered cord syndrome associated with occult spinal dysraphism in primary school children in Turkey

The prevalence and associated factors of primary tethered cord syndrome (PTCS) in primary school children were investigated. A cross-sectional study was performed in four demographically different primary schools in Turkey. Demographic, familial and physical data were collected from 5,499 children based on enuresis as a predominant symptom and dermatologic and orthopedic signs as clues of occult spinal dysraphism. Statistical analysis and input of the data were carried out with the SPSS package program 10.00, and logistic regression analysis was used to identify discriminating factors between enuretic children with or without neurologic signs. Of 5,499 analyzed children, 422 (7.7%) had enuresis nocturna, and 19.9% of 422 children had also daytime incontinence. Sixteen of these 422 enuretic children (3.8%) had several dermatologic signs. Five of them had spina bifida on plain radiographies, and 4 of them had cord tethering on lumbar MRI. Fifteen of 422 enuretic children (3.7%) had gait disturbances and orthopedic anomalies without cutaneous manifestations. Six of 15 children had spina bifida on plain graphies and 2 of them had tethered cord syndrome on MRI. The general prevalence of PTCS was found to be 0.1% of 5,499 analyzed children and 1.4% of enuretic children. A good outcome after untethering was found in 83.0% in this series. Practitioners should be aware of these clues of occult spinal dysraphism and resort to further radiologic and neurosurgical assessment. Early surgical intervention may halt the progression of the neurologic deficits and stabilize or reverse symptoms.     

小儿脊髓纵裂诊疗分析

目的探讨小儿脊髓纵裂的临床特点及治疗方法。方法回顾性分析2001年1月至2011年5月作者收治的9例脊髓纵裂患儿临床资料,年龄2个月至6岁,平均年龄17个月。出现症状时间平均7个月。临床表现有背侧中线皮肤病损7例,脊柱畸形4例,下肢功能障碍及畸形4例,排尿障碍6例,排便障碍5例,下肢疼痛1例。6例术前经CT或MRI检查确诊,3例为术中发现。Pang分型：Ⅰ型6例,Ⅱ型3例。所有病例均合并终丝栓系,合并其它脊髓畸形6例,包括脊髓脊膜膨出2例,脊髓脂肪瘤3例,皮样囊肿1例。所有病例均行手术治疗,切除脊髓分隔,同时解除合并的其它脊髓栓系畸形。结果9例患儿中,5例痊愈,所有神经症状消失,3例显著改善,1例好转,无症状加重及无效病例。结论小儿脊髓纵裂多合并其它脊髓畸形,临床表现不典型。早期诊断、早期治疗是提高疗效的关键。     

Tethered cord associated with anorectal malformation

Ten children with a tethered cord and also an anorectal malformation are reported in this document. The anorectal malformations comprised 5 vesicointestinal fissures, 2 cloacal exstrophies, 2 rectovesical fistulas and 1 rectobulbar fistula. All of the patients underwent colostomy in advance of surgery for untethering of the spinal cord. Although their neurologic deficits had previously been considered static, they were subjected to radiographic examination of the caudal spine and found to have a tethered cord. These 10 children were among 55 children with a tethered cord surgically treated at the Division of Neurosurgery of the Osaka Medical Center and Research Institute for Maternal and Child Health during the last 11 years. Data were obtained for these 10 children (6 boys and 4 girls, mean age 1.7 years) who underwent surgical untethering. Several hypotheses are offered to explain this association. Anorectal malformations are related to underlying spinal cord anomalies, which may be amenable to neurosurgical correction. Eight of our patients had no skin stigma of the lumbosacral region, in contrast to an ordinary tethered cord. Spinal cord imaging is necessary to closely scrutinize these children.     

Combination of myxopapillary ependymoma and fatty filum in a child with tethered cord syndrome. Case report

The authors present a case of a child with a tethered spinal cord associated with a myxopapillary ependymoma. This 16-month-old boy presented to the authors' institution with developmental delays in standing and walking. Magnetic resonance (MR) imaging demonstrated a fatty terminal filum and tethered cord. The child underwent surgical exploration of the spine with resection of the fatty filum tissue and release of the cord. Histological analysis of the fatty filum suggested the presence of a coexisting myxopapillary ependymoma. The child made a good recovery with no evidence of tumor recurrence after 4-years of follow-up with serial MR imaging. This unusual combination has not previously been reported in children, and to the authors' knowledge there is only one reported case in an adult. The likelihood of a common pathophysiological process in these conditions is also discussed.