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.     

小儿先天性脊柱侧弯合并椎管内神经畸形的临床研究

[目的]探讨小儿先天性脊柱侧弯合并椎管内神经畸形的发病情况和临床特征及其神经畸形与临床表现的相关性.[方法]回顾性研究122例先天性脊柱侧弯患儿,对患者的体格检查情况以及全脊柱CT、全脊髓MRI、双下肢肌电图等相关辅助检查结果进行整理分析.[结果]122例先天性脊柱侧弯有63例(51.6％)合并脊髓畸形,其中脊髓栓系(TCS) 69.8％(44/63);脊髓纵裂(SCM)占60.3％ (38/63),包括Ⅰ型脊髓纵裂占68.4％ (26/38),Ⅱ型纵裂占31.6％ (12/38);脊髓空洞27％ (17/63);脊膜膨出占20.6％ (13/63);椎管内囊肿占17.5％ (11/63);Chiari畸形4.8％ (3/63).[结论]小儿先天性脊柱侧弯合并椎管内神经畸形的发生率高,并且常表现为多种脊髓畸形同时存在.合并神经畸形的患儿常表现为腰背部中线处皮肤异常,足畸形和双下肢不对称,但对于不同神经畸形其与常见的临床表现具有不同的相关性.     

Intraspinal abnormalities and congenital spine deformities: a radiographic and MRI study

Forty-two patients with congenital spinal deformity were studied by magnetic resonance imaging (MRI). Sixteen patients had intraspinal abnormalities consisting of a tethered cord in 10, diastematomyelia in four, diplomyelia in three, syringomyelia in four, a low lying conus in three, and a teratoma of the sacrum in one patient. In seven patients, intraspinal anomalies were suspected based on routine spinal radiographs and physical findings. We recommend MRI in patients undergoing spinal stabilization procedures and in all patients with lumbosacral kyphosis and those with pain, neurologic findings, or cutaneous hairy patch.     

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.     

Intraspinal anomalies in myelomeningocele

This study reports the results of MRI analysis of the spinal cord in 83 patients with myelomeningocele. 63 patients had a "tethered cord" or a "suspected tethered cord". In 14 cases we found a "hydro-/syringomyelia". In only 2 cases there were no pathological findings. The influence especially of the "tethered cord" and the "hydro-/syringomyelia" on the development of deformities of the locomotor apparatus is discussed. Concerning this problem the experience reported in the literature is not sufficient enough to make final statements. However, in cases of deterioration of the neurological status in patients with myelomeningocele the influence of intraspinal anomalies should be considered giving rise to further diagnostic examinations.     

Occult spinal dysraphism in patients with anal agenesis

Recent reports have suggested an association between congenital anorectal anomalies and occult spinal dysraphism. Eighty-seven patients with anal agenesis have been treated at this institution over the last 14 years. Two of these patients had spinal cord anomalies recognized at birth (a myelomeningocele and a tethered spinal cord). Two additional patients presented with progressive neurologic deficits in early childhood and were each found to have a tethered spinal cord. To further assess the magnitude of this problem, we have used magnetic resonance imaging (MRI) of the spine to survey prospectively 23 infants with anal agenesis. Twenty-one former patients who were asymptomatic were recalled and also studied. Four of 44 patients (9%) were found to have significant occult spinal dysraphism; each child had undergone neurosurgical operation without morbidity. MRI found each child to have a tethered spinal cord, either as an isolated lesion (2) or in association with a syrinx (1) or lipomyelomeningocele (1). One of these patients had a neurologic deficit detected on careful preoperative evaluation. The other three, two of whom were less than 2 years old, had no detectable deficit. Neither the extent of the anorectal malformation, the absence of associated congenital anomalies, nor the demonstration of normal vertebral anatomy on plain radiographs of the spine precluded the presence of occult spinal dysraphism. Therefore, we recommend that all patients with anorectal anomalies undergo MRI imaging of their spines during initial evaluation to screen for occult spinal dysraphism. In addition, consideration should be given to recalling older patients for MRI evaluation.     

Diagnosis of developmental pathology of the neuraxis by magnetic resonance imaging

We have used magnetic resonance imaging (MRI) to evaluate 22 patients with various clinical and/or radiographic findings potentially associated with intraspinal pathology whose evaluation would have necessitated myelography or computed tomography scanning in the past. Ten of these 22 children were diagnosed by MRI to have significant intraspinal lesions: diastematomyelia in four, syringomyelia in four, tethered spinal cord in three, intraspinal lipoma in two, and spinal cord tumor in one. MRI is noninvasive, requires no exposure to ionizing radiation, and provides multiplanar images and three-dimensional reconstruction potential, all with superior soft tissue imaging. In our patient population, it has proven to be a useful diagnostic adjunct.     

Intraspinal anomalies in scoliosis: An MRI analysis of 177 consecutive scoliosis patients

Background:

The association of intraspinal neural anomalies with scoliosis is known for more than six decades. However, there are no studies documenting the incidence of association of intraspinal anomalies in scoliotic patients in the Indian population. The guide lines to obtain an magnetic resonance imaging (MRI) scan to rule out neuro-axial abnormalities in presumed adolescent idiopathic scoliosis are also not clear. We conducted a prospective study (a) to document and analyze the incidence and types of intraspinal anomalies in different types of scoliosis in Indian patients. (b) to identify clinico-radiological ‘indicators’ that best predict the findings of neuro-axial abnormalities in patients with presumed adolescent idiopathic scoliosis, which will alert the physician to the possible presence of intraspinal anomalies and optimize the use of MRI in this sub group of patients.

Materials and Methods:

The data from 177 consecutive scoliotic patients aged less than 21 years were analyzed. Patients were categorized into three groups; Group A - congenital scoliosis (n=60), group B -presumed idiopathic scoliosis (n=94) and group C - scoliosis secondary to neurofibromatosis, neuromuscular and connective tissue disorders (n=23). The presence and type of anomaly in the MRI was correlated to patient symptoms, clinical signs and curve characteristics.

Results:

The incidence of intraspinal anomalies in congenital scoliosis was 35% (21/60), with tethered cord due to filum terminale being the commonest anomaly (10/21). Patients with multiple vertebral anomalies had the highest incidence (48%) of neural anomalies and isolated hemi vertebrae had none. In presumed ‘idiopathic’ scoliosis patients the incidence was higher (16%) than previously reported. Arnold Chiari-I malformation (AC-I) with syringomyelia was the most common neural anomaly (9/15) and the incidence was higher in the presence of neurological findings (100%), apical kyphosis (66.6%) and early onset scoliosis. Isolated lumbar curves had no anomalies. In group-C, incidence was 22% and most of the anomalies were in curves with connective tissue disorders.

Conclusion:

The high incidence of intraspinal anomalies in presumed idiopathic scoliosis in our study group emphasizes the need for detailed examination for subtle neurological signs that accompany neuro-axial anomalies. Preoperative MRI screening is recommended in patients with presumed ‘idiopathic’ scoliosis who present at young age, with neurological findings and in curves with apical thoracic kyphosis.     

先天性脊柱侧凸合并脊髓分裂症的诊治探讨

目的探讨先天性脊柱侧凸合并脊髓分裂症的诊断和手术治疗方法及效果。方法1999年5月至2004年6月共收治先天性脊柱侧凸患者353例,术前经脊髓造影和(或)CTM检查发现58例合并脊髓分裂症。按照脊髓分裂畸形(SCM)的分类方法分为双管型(SCMⅠ型)和单管型(SCMⅡ型)。对其中的56例患者进行了手术治疗,除1例合并蛛网膜囊肿的患者行椎管内探查手术外,其他患者在脊柱矫形前未切除分裂脊髓间的间隔(骨性、软骨性或纤维性)。分别对SCMⅠ型和SCMⅡ型患者的临床表现、影像学改变、手术治疗的方法和效果进行回顾性分析。结果SCMⅠ型11例,占19%,SCMⅡ型47例,占81%。17例术前合并神经系统的异常表现,其中SCMⅠ型2例、SCMⅡ型15例。接受手术的56例患者中,SCMⅠ型11例,术前Cobb角(60±25)°;SCMⅡ型45例,术前Cobb角(67±21)°。术后SCMI型Cobb角(29±13)°,矫形率(51±17)%;SCMⅡ型Cobb角(39±19)°,矫形率(41±24)%。以上两组之间的差异无统计学意义。术后所有患者未出现新的神经损伤症状或原有神经症状加重。26例(45%)患者获得了平均17.3个月(4～59个月)的随访,矫形丢失(6±10)°,SCMⅠ型的7例患者丢失(5±8)°,SCMⅡ型的19例患者丢失(6±11)°,两组之间的差异无统计学意义。术后随访时患者神经症状稳定。结论先天性脊柱侧凸常合并椎管内异常病变,手术治疗前应常规行椎管内的影像学检查(脊髓造影、CTM或MRI检查)。并不是所有合并SCM的先天性脊柱侧凸患者在脊柱矫形前都需要处理椎管内的病变。对于SCMⅠ型的先天性脊柱侧凸患者,如果术前无脊髓栓系的临床表现并且影像学显示骨性间隔的上下方存在分裂的脊髓,侧凸矫形前可不必处理椎管内的骨嵴。对于合并SCMⅡ型的先天性脊柱侧凸患者,如果术前患者无进行性加重的神经系统表现,位于单管内分裂的脊髓可以像正常脊髓一样耐受脊柱矫形。     

Type I congenital multiple intraspinal extradural cysts associated with distichiasis and lymphedema syndrome

OBJECTIVE: The hereditary syndrome of multiple congenital intraspinal cysts associated with distichiasis, lymphedema and other congenital deformities is extremely rare. Modern imaging techniques have promoted the non-invasive diagnosis of spinal pathology and paved the way for better surgical planning. We reviewed the clinical data, imaging studies and treatment outcomes of a 12-year-old boy with this syndrome. CLINICAL PRESENTATION: Progressive spastic paraparesis with signs of spinal cord compression leading to frequent falls. This was associated with bilateral double row of eyelashes and pretibial edema. The MRI of thoracic spine depicted two large elongated extradural lesions extending from D5-D10 with signal intensity compatible with cerebrospinal fluid leading to severe compression of the spinal cord dorsally. TREATMENT: Laminotomy and complete microsurgical excision of the cysts resulted in a fast and full clinical recovery of his neurological deficit. CONCLUSION: Type I congenital intraspinal cysts is a rare etiology of cord compression syndrome and may be associated with distichiasis, lymphedema and other congenital deformaties. It has several characteristics, which include the higher incidence in thoracic spine and younger age group, disproportional sever motor deficit as compared with sensory disturbances and the excellent clinical recovery following successful surgical treatment.     

Patterns of congenital bony spinal deformity and associated neural anomalies on X-ray and magnetic resonance imaging

Purpose

Congenital malformations of the bony vertebral column are often accompanied by spinal cord anomalies; these observations have been reinforced with the use of magnetic resonance imaging (MRI). We hypothesized that the incidence of cord anomalies will increase as the number and complexity of bony vertebral abnormalities increases.

Methods

All patients aged ≤13 years (n = 75) presenting to the pediatric spine clinic from 2003?2013 with congenital bony spinal deformity and both radiographs and MRI were analyzed retrospectively for bone and neural pathology. Chi-squared analysis was used to compare groups for categorical dependent variables. Independent t tests were used for continuous dependent variables. Significance was set at p < 0.05.

Results

Fifty-five percent of congenital spine deformity patients (n = 41) had associated spinal cord anomalies on MRI. Complex bony abnormalities had a higher incidence of cord anomalies than simple abnormalities (67, 37 %; p = 0.011). Mixed deformities of segmentation and formation had a higher incidence of cord anomalies (73 %) than failures of formation (50 %) or segmentation (45 %) alone (p = 0.065). Deformities in the sacrococcygeal area had the highest rate of spinal cord anomalies (13 of 15 patients, 87 %). In 35 cases (47 %), MRI revealed additional bony anomalies that were not seen on the radiographs.

Conclusions

As the number of bony malformations increased, we found a higher incidence of cord anomalies. Clinicians should have increased suspicion of spinal cord pathology in the presence of mixed failures of segmentation and formation.

     

Tethered cord in a patient with multiple vertebral segmentation defects: a case report

Short trunk dwarfism with multiple vertebral segmentation defects (MVSD) represents a heterogeneous group of disorders characterized by the presence of multiple vertebral and rib abnormalities. A two and one-half year-old female with the spondylothoracic dysostosisform of MVSD is presented. In addition to skeletal anomalies, a lumbar hemangioma, bilateral foot deformities, distal leg atrophy and weakness, and areflexia at the ankles were present. An underlying neuropathic process was suspected. Results of urodynamic studies were suggestive of a neurogenic bladder. Magnetic resonance imaging of the spine demonstrated a tethered spinal cord. Although various brain and spinal cord anomalies have been described in MVSD, this is the first reported case, to our knowledge, of a tethered spinal cord in a patient with MVSD. We recommend that the management of patients with MVSD include comprehensive neurological evaluation and monitoring with appropriate electrodiagnostic, urodynamic, and neuroimaging studies.     

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。     

Use of MRI in evaluation of anorectal anomalies

Congenital anorectal anomalies present with a wide spectrum of anatomical deformity. The level of atresia, the extent of the development of the pelvic musculature, and the presence of associated anomalies all greatly influence treatment options. Further complexity may be introduced by previous attempts at correction. Magnetic resonance imaging (MRI) recently has been suggested as an effective tool in evaluation of such patients for surgical repair. Thirteen patients undergoing evaluation for either primary or secondary operations for imperforate anus were studied with MRI. Age range was newborn to 26 years. These patients had anorectal deformities in various stages of treatment--from newly diagnosed imperforate anus in the newborn to patients who had undergone previous surgical repair of their deformity with poor results. In newborns, MRI was very useful in assessing the level of atresia and determining whether perineal repair was advisable. In older patients with high atresias, MRI was useful in planning operative strategy and predicting the outcome by providing information about the pelvic musculature. This information was directly related to the size of the patient, ie, the larger, and therefore the older the patient, the more detail MRI provided about the pelvic musculature. Especially in secondary cases, this information proved to be helpful in determining the cause of the initial failure, ie, lack of adequate pelvic musculature or poor placement of the distal colon in relation to the striated muscle complex at the initial operation. MRI demonstrated previous unsuspected lesions such as tethered cord, sacral deformity or urinary tract abnormalities.(ABSTRACT TRUNCATED AT 250 WORDS)     

Intraspinale Missbildungen – Tethered Cord Syndrom

The importance of intraspinal malformations associated with "tethered cord" (attachment of the spinal cord) has increased in recent years, because of better imaging methods using nuclear magnet resonance (MRI). Orthopedic malformations such as club feet, equinus deformity, lordosis, hip dislocation, kyphosis, and differences of leg lengths, which up till now have been mostly treated by orthopedic surgeons, are usually first examined for congenital anomalies. According to the results of this examination, a neurosurgical operation for untethering is performed. The aim of our study is to define spinal malformations more exactly and to elucidate their importance for orthopedics. In addition, indications for operating, operative aims, diagnosis, therapy and follow-up are discussed. Examples of our results are shown, and the significance for interdisciplinary cooperation is emphasized.     

Evaluation of children and young adults with tethered spinal cord syndrome. Utility of spinal and scalp recorded somatosensory evoked potentials

The diagnosis of tethered spinal cord syndrome should be considered in young patients with progressive orthopedic deformities, lower extremity weakness, urinary and fecal incontinence, low back pain, or combinations of these symptoms. Myelographic, computed tomographic, and urodynamic studies are useful for establishing a diagnosis, but contribute little to the evaluation of lower extremity sensory function or to the assessment of electrophysiologic impairment of the spinal cord itself. To determine the diagnostic usefulness of the somatosensory evoked potential after posterior tibial nerve stimulation (posterior tibial nerve somatosensory evoked potential) in tethered spinal cord syndrome, 22 consecutive patients with symptoms of tethered spinal cord syndrome (aged 18 months to 22 years) underwent recording of posterior tibial nerve somatosensory evoked potential; results were correlated with clinical, myelographic, and operative findings. In patients with clinical symptoms but no myelographically demonstrable lesions, posterior tibial nerve somatosensory evoked potentials were within normal limits, suggesting normal physiologic function. In patients with myelographically and operatively confirmed tethering dysraphic lesions, posterior tibial nerve somatosensory evoked potential was predictive of the level and laterality of the lesion. Similarly, ranking the severity of neurological impairment and extent of dysraphism at operation, as well as the extent of abnormality of posterior tibial nerve somatosensory evoked potential, revealed a significant (r = 0.81, p less than 0.001) correlation between clinical severity and posterior tibial nerve somatosensory evoked potential abnormalities. Postoperatively, in 8 patients, posterior tibial nerve somatosensory evoked potential also reflected improved function in relation to the level and type of dysraphic lesion present. These findings indicate that posterior tibial nerve somatosensory evoked potential is a sensitive indicator of neurophysiologic status in patients with tethered spinal cord, and is useful for determining the level of the conus medullaris, degree of spinal cord displacement, and severity of neurological impairment associated with this congenital disturbance of neuraxis formation. Recording of posterior tibial nerve somatosensory evoked potential is noninvasive and offers a more sensitive diagnostic tool than the clinical testing of sensation for detection of the development of neurologic deficits in patients with tethered cord syndrome.     

特发性左侧胸椎侧凸

目的：探讨特发性左侧胸椎侧凸的临床特点及其可能病因。方法：对77例特发性左侧胸椎侧凸作回顾性分析,其中23例行肌电图检查。47例行全脊柱MRI检查。结果：物理检查12例有异常神经学发现,肌电图检查23例均提示神经原性损害,MRI检查有9例证实神经异常。结论：特发性左侧腰椎侧凸与脊髓异常有关,对于此类畸形应常规行MRI检查,以免遗漏脊髓异常。     

Routine MRI evaluation of low imperforate anus reveals unexpected high incidence of tethered spinal cord

Background/Purpose: Previous clinical practice has included evaluation for the presence of tethered cord in those children who have imperforate anus with a high lesion. To define the incidence in children with low lesions, the authors reviewed their experience with a protocol employing routine magnetic resonance imaging (MRI), regardless of the level of the lesion, to determine the presence of a tethered cord in all children with imperforate anus. Methods: A retrospective review of children with imperforate anus was conducted over the last 13 years at our institution. Lesions were categorized as high versus low based on the supralevator or infralevator position of the fistula. Results: Sixty-three patients completed evaluation for a tethered cord. Twenty-two (34.9%) of these 63 patients had a tethered cord: 11 of 41 (26.8%) patients with high lesions and 11 of 22 (50.0%) of those with low lesions. Of those children with a low lesion, 83% of the boys had a tethered cord, whereas 38% of the girls had a tethered cord. Forty-five percent of the patients with low lesions and a tethered cord did not have any other lumbosacral anomalies. All 22 children with a tethered cord underwent surgical release. Conclusions: The incidence of tethered cord in children with low lesions of imperforate anus is not lower than those with high lesions. The authors advocate early evaluation of all children with imperforate anus for a tethered cord. J Pediatr Surg 37:966-969.     

脂肪瘤型脊髓栓系综合征的显微手术治疗

目的探讨脂肪瘤型脊髓栓系综合征的显微手术治疗. 方法 32例脂肪瘤型脊髓栓系综合征在显微镜下切除脂肪瘤、松解脊髓、切除硬膜内外的牵拉成分. 结果术后脑脊液皮下漏3例,经脱水、俯卧位等对症治疗后痊愈.术后随访3～12个月,平均6个月,治愈5例,好转21例,无变化6例. 结论显微手术治疗脂肪瘤型脊髓栓系综合征术后并发症少,神经损害情况改善较好.     

Correlation between magnetic resonance imaging and surgical findings in the tethered spinal cord

Between October 1982 and August 1987, 20 patients underwent magnetic resonance imaging (MRI) and subsequent surgical release of a tethered spinal cord. The tethering was caused by a thick filum terminale in 6 patients. On MRI scans, the conus medullaris was at L4 in 2 patients, at L2 in 3 patients, and the filum terminale appeared thick in 1 patient. The spinal cord was tethered to an intradural lipoma correctly demonstrated by MRI in 6 patients. Increased epidural fat was misdiagnosed as an intradural lipoma in one patient and a lipomatous stalk was not identified in 2 other patients. Scar tissue resulting from repair of a meningocele had tethered the cord in the remaining 8 patients. On MRI scans, the conus medullaris was located between L3 and S3; in 5 of the patients, scar tissue was apparent on the MRI scan. This correlative study supports the use of MRI as the initial, and possibly the only, imaging modality when a tethered spinal cord is suspected. Improved or more recent MRI techniques will help demonstrate these anomalies better.