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.     

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.     

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。     

Closed spinal dysraphism: a review on diagnosis and treatment in infancy.

This article reviews the clinical presentation, pathophysiology, diagnostic strategies, and therapeutic management of closed spinal dysraphism in infancy. Four groups of symptoms are distinguished: (1) cutaneous abnormalities, (2) lower motor neuron dysfunction due to congenital spinal and nerve root abnormalities, (3) upper motor neuron dysfunction due to tethering of the spinal cord, and (4) symptoms caused by associated malformations. Guidelines are proposed concerning timing and type of diagnostic investigations in infancy. This essentially encompasses a neurological assessment, including spinal ultrasonography and nuclear magnetic resonance imaging of the spine and the brain, and a urological assessment, including ultrasonography of kidneys and bladder, cystourethrography and a urodynamic study. As to the tethered cord syndrome it is concluded that first, already in infancy, a progressive neurological dysfunction can be detected; second, surgical untethering should be performed only upon appearance of upper motor neuron signs or upon progression of lower motor neuron signs. Despite its frequently asymptomatic course, the diagnosis of a congenital lumbosacral lipoma, and in the more general sense, of a closed spinal dysraphism, implies a periodic, multidisciplinary follow-up for life.     

显性脊柱裂术后脊髓再栓系的诊断和治疗

目的 囊性脊柱裂手术后,脊髓因纤维瘢痕粘连而造成脊髓栓系,使得神经损害症状加重或产生新的症状。本文就该病的诊断及治疗进行分析。方法 对１４例脊髓脊膜膨出或脂肪脊髓脊膜膨出修补术后病例资料进行分析。再手术时年龄２～１２岁,术后随访３～１２个月,对大小便失禁分别采用Ｋｅｌｌｙ评分法及Ｂｒｕｓｋｅｗｉｚｅ评分法。结果 第一次术后１０例原有的神经损害症状进一步加重,４例无神经损害症状者术后产生新的症状。Ｍ     

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.     

Cervical cord tethering mimicking focal muscular atrophy.

Spinal cord tethering rarely occurs in the cervical region. In adults, it usually results from previous operations. However, congenital origin is always diagnosed and treated early in the infant period. We report a 12-year-old boy with cervical spinal dysraphism which was erroneously diagnosed as focal muscular atrophy, a benign form of motor neuron disease. The patient was brought to our hospital because of rapid deterioration of symptoms. Careful evaluation disclosed a hairy dimple at the nuchal area, which led to the correct diagnosis. X-ray of the cervical spine showed spina bifida from C(4) to C(6) levels and fusion of the laminae of C(4) and C(5). Spine MRI studies disclosed that the cervical cord was tethered caudally and dorsally, and the ventral nerve roots were markedly stretched, especially over the left side. Surgical intervention was undertaken and the patient's muscle power improved after untethering. The purpose of this report is to acquaint the reader with a surgically treatable condition that may appear to be benign focal amyotrophy. Skin lesion at the nuchal area should be carefully looked for.     

先天性脊柱侧凸合并脊髓异常

调查先天性脊柱侧凸患儿脊髓异常的发生并探讨影像学诊断的地位。方法１０７例先天性脊柱侧凸病例中合并脊髓异常者３６中３３．６％。所有病例均摄Ｘ线平片,脊髓造影或ＣＴ脊髓造影２５例,ＭＲＩ检查２７例。     

The value of sacral skin lesions in predicting occult spinal dysraphism in children with voiding dysfunction and normal neurological examination

ObjectiveThe role of magnetic resonance imaging (MRI) in detecting occult spinal dysraphism (OSD) in children with voiding dysfunction and a normal neurological examination is still under debate. The aim of this study was to assess the correlation of sacral skin lesions with OSD detected on MRI, in a population of children with resistant lower urinary tract symptoms (LUTS).Patients and methodsA total of 114 children over 5 years of age with urinary tract infection (UTI) and/or LUTS and normal neurological examination were enrolled. All children underwent sacral neurological examination, urine analysis and cultures, renal/bladder ultrasound, voiding cystourethrogram and urodynamic examination. After a treatment period of 6 months, the patients were re-evaluated and spinal MRI was performed in 61 with ongoing LUTS or UTI.ResultsNineteen of 61 children (31%) had cutaneous stigmas. MRI detected spinal abnormality in 2/42 children with a normal sacral examination in comparison to 7/19 children with an abnormal sacral finding (Chi-squared test, P < 0.005). The sensitivity and specificity of an abnormal sacral finding in predicting MRI abnormality were 0.76 and 0.77, respectively. Urodynamic parameters did not predict an abnormal spinal MRI.ConclusionsAbnormal sacral findings, but not urodynamic studies, are strong predictors of OSD. A normal sacral examination does not rule out OSD.     

Investigation of daytime wetting: when is spinal cord imaging indicated?

BACKGROUND: Most children with daytime wetting have detrusor instability. A minority have neuropathic vesicourethral dysfunction. The commonest cause is spina bifida, which may be closed. Clinical features suggestive of closed spina bifida include cutaneous, neuro-orthopaedic or lumbosacral spine x ray abnormalities, impaired bladder sensation, and incomplete bladder emptying. MRI is the ideal method for detecting spinal cord abnormality. It has been suggested that MRI spine is an unnecessary investigation in children with daytime wetting in the absence of cutaneous, neuro-orthopaedic, or lumbosacral spine x ray abnormalities. Aim: To clarify indications for magnetic resonance imaging (MRI) of the spine in children with voiding dysfunction. METHODS: Retrospective study of children with voiding dysfunction referred from the Guy's Hospital neurourology clinic for MRI spine between April 1998 and April 2000. Clinical notes and results of investigations, including urodynamic studies and MRI spine were reviewed. RESULTS: There were 48 children (median age 9.1 years). Closed spina bifida was detected in five, of whom four had neuropathic vesicourethral dysfunction confirmed by urodynamic studies. Impaired bladder sensation and incomplete bladder emptying were more frequent in these children than in those with normal MRI spine. One child with spinal cord abnormality had no cutaneous, neuro-orthopaedic, or lumbosacral spine x ray abnormalities. CONCLUSION: Spinal cord imaging should be considered in children with daytime wetting when this is associated with impaired bladder sensation or poor bladder emptying, even in the absence of neuro-orthopaedic, cutaneous, or lumbosacral spine x ray abnormalities.     

1岁以下脊髓脊膜膨出患儿术前尿动力学结果分析

目的：以脊髓脊膜膨出的婴儿为研究对象,进行术前尿动力学检查,评估其膀胱功能。方法2010年2月至2014年3月,本院收治脊髓脊膜膨出患儿25例,年龄45d至1个月,平均4个月,术前应用加拿大莱博瑞（Laborie）尿动力检查系统,慢速灌注充盈膀胱进行膀胱测压。结果术前尿动力检查结果：尿动力表现完全正常13例（52％）;膀胱活动活跃6例（24％）;间断排尿4例（16％）;逼尿肌不稳定2例,其中1例膀胱顺应性3 mL/cmH2 O。所有患儿膀胱容量正常,（48．3±19.8）mL。残余尿均未见明显增加,平均（3．2±4．5）mL。因膀胱活动活跃、间断排尿多见于正常婴儿,本组23例被认为尿动力结果正常,占92％。所有患儿均经手术确定脊髓脊膜膨出及脊髓栓系诊断,其中脂肪脊髓脊膜膨出15例（占60％）。结论尿动力学检查提示术前脊髓脊膜膨出的婴儿,其膀胱功能多为正常,其神经源性膀胱的损害在1岁以内多未表现出来。     

Magnetic resonance imaging in the pre-operative assessment of closed spinal dysraphism in children

Magnetic resonance imaging (MRI) was used in the pre-operative assessment of closed spinal dysraphism in 61 paediatric patients. There were 25 cases of tethered cord, 6 of retethering at the site of myelomeningocele repair, 15 cases of diastematomyelia, 12 cases of lipomyelomeningocele, and 1 case each of dermal sinus, dermoid cyst and neurenteric cyst. MRI was found to have accurate correlation with surgical findings in all cases of tethered cord, and diastematomyelia. In cases of re-tethering at the site of previous myelomeningocele repair, while it was a significant advance on previous imaging techniques precise delineation of neural tissue and discrimination from post-operative fibrosis was frequently not possible. Imaging of dorsal lipomyelomeningoceles also correlated with surgical findings and provided accurate pre-operative information. However, the accuracy and pre-operative detail in cases of transitional and terminal lipomyelomeningoceles was disappointing.     

脊髓拴系综合征手术前后尿流动力学评价的临床意义

目的：评价脊髓拴系系松解术后临床疗效与尿流动力学改变的关系,方法：20例脊髓拴系综合征患儿行拴系松解术,手术前及术后6个月行尿流动力学检查,结果：11例症状改善,术后膀胱顺应性,膀胱容量,充盈末尿道闭合压增加,逼尿肌压降低,残余尿减少,6例无改善,3例加重,结论：拴系松解术是治疗脊髓拴系综合征的有效方法,手术前后尿充动力学检查是指导治疗和评价疗效的客观指标。     

Urodynamic findings in the tethered spinal cord: the effect of tethered cord division on lower urinary tract functions.

PURPOSE: The aim of this study is to evaluate the effect of division of the tethered spinal cord urodynamically in spinal dysraphic cases. METHODS: Between 1995-1997, 20 cases (11 males, 9 females) aged from 5 months to 13 years with TSC were investigated. 13 cases (65%) were classed as belonging to the myelomeningocele group and 5 cases (35%) to the spina bifida occulta group. We used a computerized urodynamic system to evaluate the functions of the lower urinary tracts pre- and postoperatively. The definitive diagnosis of cord tethering was made using magnetic resonance imaging (MRI) in 19 cases (95%) and spinal ultrasound in 1 case (5%). Division of filum terminale and laminectomy were carried out in all cases by the Neurosurgery Department, and 2 cases with retethering were operated on twice. RESULTS: All of these cases were assessed urodynamically in the preoperative and postoperative period. Significant improvements were noted in detrusor functions (35%); electromyography recordings (45%); high leak point pressures (55%) and anal and urinary continence (70%). CONCLUSION: Lower urinary tract dysfunctions secondary to tethered cord syndrome are very common in spinal dysraphic cases and significant improvements can be achieved with a judiciously timed division of the spinal tethered cord.     

Impact of spinal dysraphism on urinary and faecal prognosis in 25 cases of cloacal malformation

ObjectiveUrinary and faecal continence are key challenges goal of cloacal malformation management. Most well-known prognostic factors are the length of common channel (CC) and the presence of a sacral defect, but the impact of associated spinal dysraphism is less well documented. The aim of this study was to investigate the impact of different types of dysraphism on urinary and faecal continence in this patient population.Materials and methodsFrom 1991 to 2011, charts and office notes of 25 patients with cloacal malformation were retrospectively reviewed. At last clinic visit, urinary and faecal continence status according to Krickenbeck criteria were correlated with the length of CC, the presence of a sacral defect (sacral ratio), and the presence of different types of spinal cord dysraphism using magnetic resonance imaging (MRI) and Fisher's exact test.ResultsMean follow-up was 8 years (4 months–21 years). The sacral ratio was abnormal (below 0.74) in 18 cases out of 25 (72%). MRI review showed normal spinal cord in eight out of 23 cases (Group 1), spinal cord anomaly in 15 out of 23 cases (65%) including nine cases of tethered cord complex (Group 2) and six cases of a short spinal cord (Group 3). While statistical analysis showed a difference regarding urinary prognosis between the groups (p = 0.005), no significant difference was found regarding faecal prognosis. None of the six patients with short spinal cord were continent for both urinary and faecal prognosis.ConclusionsThis is the first study, which highlights the impact of different types of spinal dysraphism on functional outcome in patients with cloaca. Short spinal cord seemed to carry the worst prognosis. A prospective study with a larger series is mandatory to confirm these preliminary results.     

Tethered cord as a cause of scoliosis in children with a myelomeningocele

Scoliosis interferes significantly with the functional ability of most children with a myelomeningocele. While it is recognized that tethered cord at the repair site causes neurological deterioration, it has been controversial whether tethered cord causes scoliosis. The spinal cord was untethered in 30 children with progressive loss of function and scoliosis. Of 6 children with curves greater than 50 degrees only 1 improved. Of the other 24 children their curves were stable or improved at 1 year follow-up. At late follow-up, 2-7 years, 63% were stable or improved while 38% began to progress. Tethered cord causes scoliosis and stability or improvement can be anticipated following untethering. Close long-term follow-up is essential to identify those individuals with retethering of their cord.     

Spinal dysraphism associated with the cutaneous lumbosacral infantile hemangioma: a neuroradiological review

Background

Spinal dysraphism is suspected in patients with midline abnormalities, especially in those with lumbosacral cutaneous markings. A recent prospective study demonstrated that isolated cutaneous infantile hemangiomas (IH) of the lumbosacral region have one of the highest risks (relative risk of 438) of associated spinal dysraphism.

Objective

The specific types of dysraphism and radiological findings associated with cutaneous IH of the lumbosacral region have not been described in detail, to the best of our knowledge. The aim of this multicenter study is to retrospectively classify types of spinal anomalies associated with the cutaneous lumbosacral IH.

Materials and methods

The radiological images of 20 cases of lumbosacral infantile hemangioma associated with spinal dysraphism were reviewed.

Results

Tethered cord was found in 60% of the 20 cases, spinal lipoma was present in 50% and 45% had intraspinal hemangiomas. Sinus tract was found in 40% of the children.

Conclusion

A range of spinal anomalies is associated with cutaneous lumbosacral infantile hemangiomas and MRI can be used to characterize these abnormalities.     

Outcome of meningomyelocele/lipomeningomyelocele in children of northern India

OBJECTIVE: To evaluate the clinical profile, associated anomalies and surgical outcome of children with meningomyelocele (MMC)/lipomeningomyelocele. METHODS: Out of a total of 181 cases of spinal dysraphism treated at our Institute between 1996 and 2004, 102 (56.35%) cases of MMC/lipomeningomyelocele were analyzed retrospectively and prospectively. The clinical profile and radiological findings of these children were recorded. Craniospinal MRI was the essential investigation and was done as a management protocol at our Institute for these children. Eighty-two out of 102 (80.3%) cases had pure MMC/lipomeningomyelocele and 20/102 (19.6%) had associated split cord malformation (SCM; complex spina bifida). All these children underwent surgery for their primary and associated malformations as indicated. They were clinically assessed over a mean follow-up period of 3.6 years ranging from 1.5 months to 8 years. No urodynamic or evoked potential studies were done to assess the sphincteric outcome following surgery. RESULTS: Forty-six (45.1%) of children with MMC had other associated tethering lesions, including the presence of SCM. Craniospinal axis screening remained an important tool to understand the associated tethering lesions and malformations. Only 58.8% of children had hydrocephalus; thus the incidence was much lower compared with reports from the western literature (80-96%). 63.3% of children with overt hydrocephalus required shunt surgery prior to the definitive surgery; however, 23.3% of cases required a shunt after the MMC has been closed. Improvement in clinical profile following microneurosurgery was observed in 42.8% of cases with motor involvement, 46.8% of cases with sensory dysfunction and 39.5% of cases with sphincteric involvement. Motor improvement was seen in 43.6% of cases of pure MMC/lipomeningomyelocele as compared to only 40.0% of cases of complex spina bifida. Sensory improvement was also better in pure MMC/lipomeningomyelocele group being seen in 48.0% of cases as compared to only 42.9% of cases of complex spina bifida. CONCLUSION: Presence of SCM with MMC is referred to as complex spina bifida and is seen in a significant proportion (19.6%) of all cases of MMC. Thorough assessment of the craniospinal imaging needs to be done to look for the presence of multiple tethering lesions which could be present in the same case. Not all children with spinal dysraphism with hydrocephalus required shunt surgery or CSF diversion but a constant and vigilant follow up could avoid it in 13.3% of cases. Improvement in motor and sensory dysfunction was better in the pure MMC/lipomeningomyelocele group than in the complex spina bifida group. Improvement in sphincteric dysfunction, although seen in significant cases, was less compared with improvement in motor and sensory dysfunction. This probably represents a lack of definitive objective criteria for urodynamic improvement and a lack of proper rehabilitation. Electromyographic studies and uroflowmetry are required to asses the true sphincteric outcome following surgery.     

脊髓拴系松解术后脊柱裂患者的肛管直肠功能研究

目的：与临床对于脊柱裂致膀胱功能障碍的大量研究报道相比,关于脊柱裂患者的肠道功能障碍研究资料非常有限。该文拟研究脊柱裂患者行脊髓拴系松解术后的肛管直肠功能。方法：采用多通道肛管直肠测压技术对因排尿功能障碍而来就诊的21例脊柱裂患者进行肛管直肠功能检测,入选患者皆已于至少2年前行脊髓拴系松解术。结果：脊柱裂患者的最大肛管静息压低于对照组,但二者没有统计学差异(P=0.372)。在嘱脊柱裂患者行最大限度收缩肛门动作时,绝大多数患者肛管压力没有任何升高。在行模拟排便动作时,19例(90.5%)患者表现为盆底功能紊乱型肛管压力变化。直肠肛管抑制反射在所有受检者均存在,诱发该反射所需最小直肠气囊容量在脊柱裂患者组和对照组间差异无显著性(P=0.725);诱发持续性直肠肛管抑制反射所需直肠气囊容量在脊柱裂患者组显著性高于对照组(P<0.001)。直肠感觉阈值在脊柱裂患者显著高于对照组(P<0.0001)。结论：大多数脊柱裂患者不能自主收缩肛门外括约肌,排便时表现为盆底功能紊乱型直肠肛管压力曲线,同时直肠感觉功能也受到严重损害。直肠肛管抑制反射在所有脊柱裂患者均存在,该反射可能受到中枢神经系统的调控。     

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。