Spinal cord abscess secondary to infected dorsal dermal sinus in an infant: uncommon presentation of a known entity.

Infection along the congenital dermal sinus tract is well known. However, congenital dorsal dermal sinus presenting with intramedullary abscess is quite rare. The sinus tract usually presents in the midline and acts as a portal of entry for infection that may manifest as meningitis, extradural or subdural abscess and may further involve the cord. Surgical drainage of pus and complete excision of the sinus tract is the standard treatment. Here we describe an infant with an infected congenital dorsal dermal sinus with atypical presentation as large paracentral abscess in the upper back. We further highlight the importance of recognising and treating these skin dimples even when clinically silent to avoid catastrophic complications.

Finite element investigation of the effect of a bifid arch on loading of the vertebral isthmus.

BACKGROUND: The biomechanical effect of a bifid arch as seen in spina bifida occulta and following a midline laminectomy is poorly understood. PURPOSE: To test the hypothesis that fatigue failure limits will be exceeded in the case of a bifid arch, but not in the intact case, when the segment is subjected to complex loading corresponding to normal sporting activities. STUDY DESIGN: Finite element analysis. METHODS: Finite element model of an intact L4-S1 human lumbar motion segment including ligaments was used. A section of the L5 vertebral arch and spinous process was removed to create the model with a midline defect. The models were loaded axially to 1 kN and then combined with axial rotation of 3°. Bilateral stresses, alternating stresses, and shear fatigue failure on both models were assessed and compared. RESULTS: Under 1 kN axial load, the von Mises stresses observed in midline defect case and in the intact case were very similar (differences <5 MPa) having a maximum at the ventral end of the isthmus that decreases monotonically to the dorsal end. However, under 1 kN axial load and rotation, the maximum von Mises stresses observed in the ipsilateral L5 isthmus in the midline defect case (31 MPa) was much higher than the intact case (24.2 MPa), indicating a lack of load sharing across the vertebral arch in the midline defect case. When assessing the equivalent alternating shear stress amplitude, this was found to be 22.6 MPa for the midline defect case and 13.6 MPa for the intact case. From this, it is estimated that shear fatigue failure will occur in less than 70,000 cycles, under repetitive axial load and rotation conditions in the midline defect case, whereas for the intact case, fatigue failure will occur only after more than 10 million cycles. CONCLUSIONS: A bifid arch predisposes the isthmus to early fatigue fracture by generating increased stresses across the inferior isthmus of the inferior articular process, specifically in combined axial rotation and anteroposterior shear.

Computerized analysis and modelling of patients with deformities of lower limbs.

The purpose of this paper was to model the human gait of typical subjects and patients with such deformities of lower limbs as: spastic diplegia cerebral palsy and spina bifida occulta. Model coefficients will lead to the development of a better computer system to support clinical decision-making in human gait in terms of assessment, diagnosis, and classification. Human gait was evaluated by using Motion Analysis System in the Syncrude Center for Motion and Balance in Edmonton. Kinetics data were used for the mathematical modelling based on regression function. The difference between the model coefficients of the patients with the deformities of lower limbs and typical subjects were analysed. There is shown that the model coefficients are different in each group. The modelling can help to define gait pathology and treatment for a large number of patients.

Prevalence of sacral spina bifida occulta and its relationship to age, sex, race, and the sacral table angle: an anatomic, osteologic study of three thousand one hundred specimens.

STUDY DESIGN: An anatomic, osteologic study of spina bifida occulta (SBO). OBJECTIVE: To determine the prevalence and patterns of SBO in a large population and examine its relationship to age, sex, and race; then to evaluate SBOs relationship to the sacral table angle (STA) when compared with an age-matched control group. SUMMARY OF BACKGROUND DATA: SBO has a reported prevalence of 1.2% to 50% and has been implicated in various pathologic problems. SBO is often associated with spondylolysis or spondylolithesis. The STA has been implicated as an etiologic or predictive factor in the presence of pars defects. METHODS: Three thousand one hundred osteologic specimens were evaluated for the presence of SBO. SBO was graded on a scale from 0 to III. Information on the age, sex, race, and STA of each specimen was recorded and measured, respectively. Prevalence and patterns of SBO were enumerated. The STAs of an age-matched control group of 355 specimens were examined. The SBO group and control groups were compared in regards to STA, controlling for age, sex, and race. RESULTS: Overall, 355 specimens displayed SBO, for an overall prevalence of 12.4%. Of the SBO specimens, 68.7% were white, 88.2% were men, 53% were grade I, 37% II, and 10% III. All 3 grades of SBO were more common in men than women (88.2% vs. 11.8%) and more prevalent in whites than blacks (68.7% vs. 31.3%) (P = 0.01). SBO decreased in prevalence with increasing age. The average STA in SBO specimens was 95.9 degrees . This differed from an age-matched control group, 92.1 degrees (P < 0.0001). Every 1 degrees increase in STA resulted in a 6% increased likelihood of SBO. In SBO specimens, the STA decreased with increasing age, contrary to age-matched controls. CONCLUSION: SBO has an overall prevalence of 12.4% in a large, diverse population. SBO is more common in men and whites and decreases in prevalence with increasing age. The STA is greater in SBO when compared with controls and an increased STA predicts SBO. In SBO, the STA decreases with increasing age.

Contribution of the multifidus muscle for control of upright posture in subjects with spina bifida occulta.

CONTEXT: The multifidus muscle plays a role in controlling lumbosacral position and postural sway. One of its attachment sites is the exact site of spina bifida occulta (SBO). OBJECTIVE: To investigate the role of the muscle for postural control in SBO. DESIGN: Cross-sectional cohort. PARTICIPANTS: Eighty subjects with SBO (38 in minor; 42 in major) and controls. INTERVENTIONS: N/A. MAIN OUTCOME MEASURES: Subjects stood upright on a platform at 0 degrees and on an inclined surface (10 degrees and 20 degrees) with feet in plantarflexion/dorsiflexion, together with eyes open and closed. The platform system measured posture to obtain sway area and sway mean for statistics. RESULTS: Upon sway area/mean, the group differences of major/minor SBO vs. control were all significant. Sway means of minor SBO were lower than those of major SBO at corresponding inclined degrees. CONCLUSIONS: Subjects with SBO demonstrated increased sway as compared to controls.

Outcome of untethering for symptomatic spina bifida occulta with lumbosacral spinal cord tethering in 31 patients: analysis of preoperative prognostic factors.

BACKGROUND CONTEXT: The most important goal for treating symptomatic lumbosacral spinal cord tethering is early untethering. PURPOSE: To investigate preoperative symptoms that may have affected the outcome. STUDY DESIGN: Patients with or without improvement and with or without favorable outcome after untethering were compared retrospectively by chart and image review. PATIENT SAMPLE: Thirty-one patients (age between 2 days to 25 years) with spina bifida occulta and symptomatic cord tethering were analyzed. Presenting symptoms (neurological deficits, urological dysfunction, and lower limb deformities) were assessed before and after untethering. OUTCOME MEASURES: Favorable outcome was defined as complete relief of symptoms or mild symptoms whereby patients are able to look after their own personal care without assistance. Unfavorable outcome was defined as moderate or severe disability whereby patients are unable to attend to their own bodily needs without assistance, are bedridden, or require constant nursing attention. METHODS: Differences in patient characteristics and presenting symptoms were compared between those with and without clinical improvement and favorable outcome. Multivariate logistic regression was used to identify prognostic factors affecting the outcome. RESULTS: The average age at surgery was 7.2 years, with a male-to-female ratio of 1.2. The average follow-up time was 4 years. At least one of the following symptoms was present in all patients: neurological deficits (83.9%), urological dysfunction (77.4%), or limb deformities (38.7%). After untethering, all patients had either symptoms stabilized (14 patients, 45.2%) or improved (17 patients, 54.8%), and 14 patients (45.2%) achieved total resolving of symptoms. Logistic regression confirmed that younger age (< or =2 years, odds ratio [OR] 22.0, p=.026), lipomas of filum terminale (OR 25.6, p=.042), and a poor anal tone (OR 10.4, p=.061) were positive prognostic factors for the improvement in symptoms. The functional outcome was determined by the age at surgery (OR 0.9 per year since 1 year old, p=.04) and the presence of limb deformities (OR 0.06, p=.017). CONCLUSIONS: In conclusion, our study suggests that untethering should be performed immediately once the patient shows evidence of symptomatic lumbosacral cord tethering, irrespective of age. Untethering can interrupt progression of symptoms, but sphincter dysfunction and muscle weakness are more likely to improve or resolve. Benefits can be seen in all patients, but young children (before 2 years old) have a higher chance to gain favorable outcome. Retethering is a main concern during follow-up, particularly for the more complicated lipomyelomeningoceles. Investigations using electrophysiologic and urodynamic studies are helpful for early detection of subtle symptomatic cord tethering or retethering.

Spina bifida occulta. Foot deformities, enuresis and vertebral cleft: clinical picture and neurophysiological assessment.

AIM: The aim of the study was to investigate the relationship between the clinical evidence of foot deformities in spina bifida occulta and the associated neurophysio-logical damage. METHODS: The authors studied 47 patients with foot deformities (37 flat foot, 10 pes cavus) and vertebral cleft, variably associated with enuresis, midline cutaneous lesions, and further orthopaedic deformities. An electrophysiological evaluation was performed in an attempt to investigate the peripheral nervous system in greater detail, including conventional motor and sensory nerve conduction, F-wave recording and electromyogram (EMG) testing. RESULTS: The peroneal nerve F wave latency was longer in patients with pes cavus than in those with flat foot (P<0.04). Conversely, the posterior tibial nerve F-wave latency was longer in patients with flat foot than in those with pes cavus (P<0.02). Needle EMG showed large amplitude motor unit potentials during voluntary recruitment in all patients, suggesting a neurogenic origin of these EMG changes. Neurophysiological study makes it possible to distinguish between myogenic and lower motor neuron involvement. The existence of some degree of spinal cord dysraphism may be pathophysiologically associated with foot deformities. CONCLUSION: Children with foot deformities and clinical evidence of occult spinal dysraphism should have a neuro-physiological assessment in order to obtain an early diagnosis and avoid ineffective foot surgery.

GDNF plasma levels in spina bifida: correlation with severity of spinal damage and motor function.

Glial-derived neurotrophic factor (GDNF) is one of several powerful survival factors for spinal motoneurons that play a key role in sprouting, synaptic plasticity, and reorganization after spinal cord damage. The aim of this study was to investigate the expression of GDNF in plasma of children with spina bifida (SB) and to determine its correlation with both the severity of spinal cord damage and the motor function of these patients. To measure the GDNF expression, we collected plasma samples from 152 children with SB and in 149 matched controls. Endogenous GDNF levels were quantified using a two-site immuno-enzymatic assay. The statistical analysis was performed using the Mann-Whitney two-tailed two-sample test. In children with SB the mean levels of GDNF (131.2 +/- 69.6 pg/mL) were significantly higher (p < 0.001) with respect to the mean levels of the control group (102.7 +/- 6.8 pg/mL). Moreover, in open SB, the GDNF levels (139.2 +/- 81.1 pg/mL) were significantly higher (p < 0.05) with respect to closed SB (117.2 +/- 41.3 pg/mL). In terms of the motor function of patients, we found that in children with poorer motor function, the GDNF levels (134.5 +/- 67.4 pg/mL) were higher, but not statistically significant (p < 0.1), than in patients with better motor outcome (122.3 +/- 72.2 pg/mL). Our study demonstrates GDNF over-expression in children with SB. This upregulation is significantly associated with the severity of spinal cord damage in SB patients and appears to correlate with poor motor function of children, representing an important biochemical marker of the severity of spine injury.

Age-related differences in executive function among children with spina bifida/hydrocephalus based on parent behavior ratings.

Previous research has suggested that adolescents with myelomeningocele and shunted hydrocephalus (MMH) have difficulties with aspects of executive functioning and, in turn, with functional independence. There is little research, however, examining patterns of executive functioning across adolescence in this population. The goal of this cross-sectional study was to examine parent ratings of executive function in children with MMH and in typically developing peers across late childhood and adolescence. Parents of 36 individuals with MMH and 35 typically developing peers, ages 10 to 18 years, completed the Behavior Rating Inventory of Executive Function (BRIEF). The BRIEF is organized into eight scales and two primary indices-Metacognition (MCI) and Behavioral Regulation (BRI). As a whole, the children with MMH had significantly higher BRIEF T-scores, as well as a higher prevalence of clinically significant T-scores across subscales, particularly those representing cognitive control. Effects of group, age, and age-by-group interactions on the mean raw scores of the MCI and BRI were examined using regression analyses. There were significant group effects (p <. 05) for both the BRI and MCI, with the controls having significantly lower mean ratings than the MMH group. There was also a significant contribution of age-by-group interaction on the BRI (p <. 05). Although mean raw scores on the BRI for the MMH group remained stable across ages, mean raw scores in the control group decreased as age increased. Thus, healthy children have age-related improvements in executive control behaviors across adolescence, particularly behavioral control, while children with MMH demonstrate no age-related improvements in parent reported executive behaviors across adolescence. Therefore, children with MMH may continue to require targeted interventions and modifications to address executive dysfunction into young adulthood in order to promote functional independence.

Clinical urological outcomes following primary tethered cord release in children younger than 3 years.

PURPOSE: Current practice at our institution is to recommend tethered cord release at diagnosis to prevent the onset or worsening of symptoms. Tethered cord release is frequently performed in children younger than 3 years who often have no urological manifestations. To our knowledge there are currently no long-term data on urological outcomes in this age group. MATERIALS AND METHODS: We completed a retrospective review of 475 cases of tethered cord release performed at a single institution between 1995 and 2002. Of these surgeries 173 were performed in children younger than 3 years. Clinical outcomes, and preoperative and postoperative urodynamic and radiographic studies were evaluated. RESULTS: A total of 79 patients met study criteria. Average age at surgery was 9.6 months and average followup was 5.2 years (range 6 months to 11.2 years). At followup 49 patients (62.1%) had no urological complaints and 30 (38%) had urological problems. A total of 20 children (25.3%) had minor problems (constipation, delayed toilet training or other) and 10 (12.7%) had major problems (need for clean intermittent catheterization, febrile urinary tract infection or reflux). Of 66 patients 30 (45.5%) had abnormal preoperative urodynamics. One of 31 patients (3.2%) had hydronephrosis on preoperative ultrasound. Statistical analysis revealed that abnormal preoperative urodynamics and ultrasound were not predictive of major urological problems. Lipomatous dysraphism and preoperative musculoskeletal symptoms positively correlated with major urological problems (p = 0.0076 and 0.0484, respectively). CONCLUSIONS: The majority of children did not experience urological problems following tethered cord release. Only a small set of children had major urological problems. Children with lipomatous dysraphism and musculoskeletal symptoms were more likely to experience poor urological outcomes.

Postural sway at ground and bevel levels in subjects with spina bifida occulta.

To assess whether the postural function is impaired by comparing the performances in upright standing at ground and bevel levels in adult subjects with spina bifida occulta (SBO). Eighty subjects with SBO (38 with minor type and 42 with major type) and 35 healthy control subjects participated in the study. All participants performed ten tests while standing upright on a platform at ground level (0 degrees, baseline) and on a beveled surface (with their feet in dorsiflexion and plantarflexion at 10 degrees and 20 degrees). Tests were done with their eyes open and closed. The postural sway was examined using a force platform (CATSYS, Danish) that records sway intensity and velocity. Sway intensity and sway velocity were universally associated with group, degree of bevel, open- or closed-eyes condition, and dorsiflexion or plantarflexion after adjusting for age and gender. With respect to sway intensity, the differences of minor or major SBO group were significantly decreased at different bevel degrees when compared with control groups, whereas the differences between minor and major SBO were significant differences at 10 degrees and 20 degrees. With respect to sway velocity, the differences of major SBO group were significantly decreased at different bevel degrees when compared with minor SBO and control groups, whereas the difference in minor SBO was only significant at 0 degrees when compared with control. Group differences (minor SBO vs. control, major SBO vs. control) showed a significant decrease in sway velocity when comparing at 10 degrees than at 0 degrees and at 20 degrees than at 0 degrees. In all subjects with SBO, the sway intensity/velocity values obtained with open eyes and with plantarflexion had lower values, when compared with values obtained with closed eyes and with dorsiflexion. This study supports the hypothesis that SBO impairs control of postural sway in both the resting upright and stressful postures. Our results imply that the larger the bone defect at the lumbosacral midline, the more the group differences in different stressful conditions. Both velocity and intensity were able to reflect the function of the postural sway from our results. This is the first report to add the bevel degree and foot position, as well as visual input as being the part of the study in investigating the postural sway.

Outcome in patients who underwent tethered cord release for occult spinal dysraphism.

PURPOSE: Tethered cord syndrome encompasses a group of clinical symptoms caused by abnormal spinal cord fixation. We evaluated a select cohort of patients with primary tethered cord syndrome in regard to urodynamic and clinical outcome after cord release. MATERIALS AND METHODS: We retrospectively reviewed the records of patients with the diagnosis of tethered cord from May 2001 to October 2004. Patients were assessed preoperatively by standard urodynamic studies, which was repeated a mean of 6.4 months after tethered cord release. Clinical and urodynamic outcomes were analyzed. RESULTS: Ten male and 14 female patients 1 month to 12 years old (median age 6 years) were evaluable. Preoperatively 14 of the 24 patients with a median age of 8.1 years were toilet trained and 7 (50%) had diurnal incontinence. Constipation was noted in 10 of 24 patients (42%) and urinary tract infections developed in 6 (25%). Postoperatively only 1 patient (7%) experienced diurnal incontinence (p = 0.04). Constipation was observed in 6 patients (25%) and urinary tract infections developed in 1 (4.2%) (p = 0.29 and 0.07, respectively). Ten of the 21 children (48%) with abnormal urodynamics had normalized studies postoperatively. Ten of the 17 patients with neurogenic detrusor overactivity achieved normalization and 7 remained unchanged. Two of 3 patients with normal preoperative urodynamics had neurogenic detrusor overactivity and 1 had poor bladder compliance. Four patients with low bladder capacity and/or low compliance preoperatively did not improve. CONCLUSIONS: Our results suggest that tethered cord release is beneficial in terms of clinical and urodynamic outcomes. Patients with abnormal urodynamics had 48% improvement after tethered cord release. Neurogenic detrusor overactivity seems to respond better with 59% improvement in urodynamics. The level of the conus on magnetic resonance imaging did not seem to be predictive of urodynamic outcome. Patients with a normal bladder may show urodynamic deterioration postoperatively, which raises cause for concern.

Biomechanical comparison of lumbar spine with or without spina bifida occulta. A finite element analysis.

STUDY DESIGN: Biomechanical study using finite element model (FEM) of lumbar spine. OBJECTIVES: Very high coincidence of spina bifida occulta (SBO) has been reported more than in 60% of lumbar spondylolysis. The altered biomechanics due to SBO is one considerable factor for this coincidence. Thus, in this study, the biomechanical changes in the lumbar spine due to the presence of SBO were evaluated. SETTING: United States of America (USA). METHODS: An experimentally validated three-dimensional nonlinear FEM of the intact ligamentous L3-S1 segment was used and modified to simulate two kinds of SBO at L5. One model had SBO with no change in the length of the spinous process and the other had a small dysplastic spinous process. Von Mises stresses at pars interarticularis were analyzed in the six degrees of lumbar motion with 400 N axial compression, which simulates the standing position. The range of motion at L4/5 and L5/S1 were also calculated. RESULTS: It was observed that the stresses in all the models were similar, and there was no change in the highest stress value when compared to the intact model. The range of motion was also similar in all the models. The lumbar kinematics of SBO was thus shown to be similar to the intact model. CONCLUSION: SBO does not alter lumbar biomechanics with respect to stress and range of motion. The high coincidence of spondylolysis in spines with SBO may not be due to the mechanical factors.

Clinical significance of congenital lumbosacral malformations in young male population with prolonged low back pain.

STUDY DESIGN: A prospective clinical investigation. OBJECTIVE: To determine the clinical significance of congenital lumbosacral malformations in young male patients with subacute or chronic low back pain and to investigate a possible worsening of the clinical picture with the concurrence of transitional vertebra (TV) or spina bifida occulta (SBO). SUMMARY OF BACKGROUND DATA: Although the causative roles of congenital malformations in low back pain and lumbar disc herniation have been investigated, there is no study that compares the incidence of congenital malformations in prolonged low back pain between radicular and nonradicular groups; nor is there a study that compares the pain intensity between the groups with and without congenital malformations. METHODS: Lumbosacral plain radiographs of 881 young male patients with low back pain lasting for >4 weeks were evaluated. For all patients, we recorded the clinical signs and their pain intensities based on a 10-cm visual analog scale (VAS). RESULTS: Congenital lumbosacral malformations were determined in 88 of 881 patients. Of these, 48 were TV, 38 were SBO, and 2 were a combined lesion of TV and SBO. The difference in positive clinical sign and VAS was statistically significant (P < 0.05) between the groups with and without congenital lumbosacral malformations. In contrast to SBO, there was a statistically significant difference of TV incidence between the sign positive and the sign negative groups (P < 0.05). The difference in VAS values was statistically significant for both TV and SBO (P < 0.001). CONCLUSIONS: The results of this study show that SBO and TV increase the severity of the clinical picture whether or not they have a causal relationship. Additionally, transitional vertebrae seem to increase nerve-root symptoms whereas spina bifida occulta does not.

Aging with a disability.

UNLABELLED: This focused review highlights important issues in the care of persons who are aging with a disability. It is part of the study guide on geriatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article specifically focuses on significant medical and rehabilitation issues pertinent to persons with cerebral palsy, spina bifida, postpoliomyelitis syndrome, and selected other neurologic and neuromuscular diseases. In addition to normal physiologic aging, people with these conditions often experience secondary complications and accelerated impairments because of aging itself. These complications are described, and monitoring strategies and treatment are recommended. OVERALL ARTICLE OBJECTIVE: To summarize issues in the care of persons aging with a disability.

Multidisciplinary evaluation of occult spinal dysraphism in 47 children.

OBJECTIVE: To present the clinical (urologic, orthopedic and neurologic) and urodynamic findings of 47 children suffering from occult spinal dysraphism, together with the long-term follow-up results obtained with various treatment modalities. MATERIAL AND METHODS: Between 1997 and 2000 a total of 47 children (27 girls, 20 boys; male:female ratio 1.3) referred to the Urology and/or Pediatrics Departments with symptoms and signs of closed spina bifida were enrolled in the study program. All patients underwent routine assessment of the urinary tract, including detailed anamnesis, physical examination and radiologic evaluation (X-ray, renal bladder ultrasonography and sacral MRI). In addition to video-urodynamic evaluation of the lower urinary tract, all patients were also evaluated by the Orthopedic Department with respect to possible lower extremity deformities. RESULTS: The age range of the children was 2 months to 16 years (mean 6.9 years). At first referral, 23 children were found to have normal urinary and fecal continence after toilet training; among the other presenting symptoms and signs, 34% of patients demonstrated recurrent urinary tract infections and 38.2% had abnormal findings on urinary tract investigations. Evaluation of urodynamic parameters before and after conservative treatment demonstrated an increase in age-related bladder capacity in 34 patients and detrussor instability had been cured in 23/30 patients (p < 0.05). Overall, bladder capacity was found to be normal in 40 children following conservative management (p < 0.05). The conservative approach proved to be effective in 40 children (85.1%), and intravesical instillation therapy with oxybutynine hydrochloride was successful in one of the remaining seven children (14.2%). Bladder augmentation was performed in six children (12.7%) in whom conservative measures were ineffective. CONCLUSIONS: In the light of our findings and the literature data it is obvious that a multidisciplinary approach together with early urologic evaluation to determine the extent of neurologic involvement of the lower urinary tract is essential to ensure a successful treatment outcome and to prevent the occurrence of serious functional and structural complications. Clinical, radiologic and video-urodynamic assessments should be performed to define the neuro-urologic pathophysiology and to provide management guidelines and a baseline for future comparison.

[Occult spinal dysraphism]. / Okkult spinal dysrafisme.

BACKGROUND: Spinal dysraphism is a group of congenital anomalies of the spine characterized by a midline defect affecting the nervous tissue and its bony and soft tissue coverings. Closed spinal dysraphism, spina bifida occulta, refers to skin-covered lesions. However, there are a number of cutaneous stigmata or other physical signs that serve as indicators of an underlying dysraphic malformation of the lower spinal canal. MATERIAL AND METHODS: Based upon relevant literature, we present a review of occult spina bifida. RESULTS AND INTERPRETATION: The cutaneous stigmata that may indicate an underlying dysraphism are particularly hairy patches, subcutaneous lipomas, capillary hemangiomas, dorsal dermal sinuses and sacral cutaneous pits. The clinical examination of a child with lumbosacral cutaneous stigmata should focus on neurological signs and symptoms, urological disturbances and orthopaedic deformities in the lower limbs. Spinal MRI is the imaging modality of choice in children presenting with features consistent with occult spinal dysraphism. Children with occult spinal dysraphism should be referred to a neurosurgical unit.

Malaysian children with spina bifida: relationship between functional outcome and level of lesion.

PURPOSE: To determine the relationship between spinal lesion level and functional outcome in children with spina bifida. METHODS: Prospective observational study of 66 children who attended the Spina Bifida Clinic from 1994-1997. Data were obtained from serial physical examination and parent interview to determine mobility status, neurosensory deficits, continence and school placement. RESULTS: Eighteen (27.3%) had high-level (thoracic and thoracolumbar), 27 (40.9%) intermediate (low lumbar) and 21 (31.8%) low-level (sacral) lesions. Children with high-level lesions experienced more mobility problems (independent ambulation, balance and use of appliances), than those with intermediate or low-level lesions (p<0.001). 58.8% of children with low-level lesions used diapers, compared with those with high (90%) or intermediate (100%) level lesions (p = 0.005), but there were no significant differences in the incidence of soiling or urinary incontinence among all three groups. There were no significant differences among the three groups in terms of school placement, skin breakdown, epilepsy or visual defect. CONCLUSION: Although there is good correlation between the level of the lesion and mobility in children with spina bifida, other measures of functional outcome like continence and school placement are more difficult to predict. These data are important for realistic counselling of families with newborns with spina and planning long term rehabilitation resources.

Limited dorsal myeloschisis associated with multiple vertebral segmentation disorder.

A 3-year-old girl was admitted to our department with spina bifida occulta. At birth, thoracic dysplasia with severe respiratory dysfunction and a soft pedunculated mass connecting with an intradural mass were noted. The patient did not start to walk and partial removal of the intradural mass was performed via a laminectomy of the fused vertebrae. There was no boundary between the spinal cord and the mass and the histological diagnosis of this mass was connective tissue. The anomalies in this case were considered to be multiple vertebral segmentation disorder (MVSD) and limited dorsal myeloschisis. The coincidence of these anomalies might suggest the causal genesis of MVSD.

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.