Hip instability in myelomeningocele: 158 patients followed for 15 years

In 158 patients with myelomeningocele, the prevalence of hip dislocation at birth and subluxation or dislocation of unoperated on hips at 2 and 15 years of age were assessed. In neonates examined during the first 10 weeks of life, 10 percent of the hips were dislocated. In children with a thoracic or L1-2 neurologic level, the cumulative incidence of dislocated hips increased from 8 to 26 percent and subluxations from 33 to 45 percent. In children with L3 and L4 levels, one fourth of the hips had dislocations and nearly half subluxations. At L5 and the sacral motor level, dislocations did not occur, but one fifth of the hips had subluxations. Children with L3 or L4 neurologic levels had bilateral hip dislocation at an earlier age than those with higher levels of paralysis. There appears to be no risk of developing hip dislocation at neurologic levels below L4, and spasticity promotes hip dislocation above L3.     

Hip instability in myelomeningocele. 158 patients followed for 15 years

In 158 patients with myelomeningocele, the prevalence of hip dislocation at birth and subluxation or dislocation of unoperated on hips at 2 and 15 years of age were assessed. In neonates examined during the first 10 weeks of life, 10 percent of the hips were dislocated. In children with a thoracic or L1-2 neurologic level, the cumulative incidence of dislocated hips increased from 8 to 26 percent and subluxations from 33 to 45 percent. In children with L3 and L4 levels, one fourth of the hips had dislocations and nearly half subluxations. At L5 and the sacral motor level, dislocations did not occur, but one fifth of the hips had subluxations. Children with L3 or L4 neurologic levels had bilateral hip dislocation at an earlier age than those with higher levels of paralysis. There appears to be no risk of developing hip dislocation at neurologic levels below L4, and spasticity promotes hip dislocation above L3.     

Obesity and myelomeningocele: anthropometric measures

Objective:

To evaluate the appropriate use of arm span measurements as a substitute for height/linear length to evaluate obesity in people with myelomeningocele by comparing calculated body mass indices (BMIs) with recently published BMI graphs by the Centers for Disease Control and Prevention (CDC) and National Center for Health Statistics standards (NCHS) published in 2000.

Study Design:

Retrospective analysis of collected data on patients seen in the University of Washington Birth Defects Clinic from July 1, 1965, through June 1, 2008. Observations included degree of paralysis, presence of scoliosis, height (linear length), weight, and arm span. We compared published CDC/NCHS BMIs with our data using both height and arm span in place of height/linear length. There were 14,701 measures collected during 4,968 visits from 709 patients. Mean values were calculated using age, gender, and lesion level as independent variables.

Results:

Comparison of BMI means of patients with myelomeningocele suggests that our observations using arm span and height are comparable with the CDC/NCHS BMI means using height for the 2 least paralyzed groups but not for those groups with paralysis from high-level lesions that are more likely to exhibit lower extremity deformities or scoliosis.

Conclusions:

Published CDC/NCHS graphs, with their percentiles, are appropriate for estimating normal growth by BMI for children born with myelomeningocele when arm span is substituted for length if severe body differences due to high-level paralysis are taken into consideration.     

Neuromuscular function and radioanatomical form of the myelomeningocele hip

The aim of this study was to describe variants in neuromuscular function and radioanatomical form of the myelomeningocele hip based on the level of spinal paralysis. For this purpose, 84 hip joints of 44 children with myelomeningocele age 1 to 10.75 years were classified into five spinal paralysis groups. Using anteroposterior pelvic radiographs, the position of the capital epiphysis, represented by the epiphyseal angle, and the position of the greater trochanter apophysis, represented by the apophyseal angle, were evaluated. The epiphyseal angle of the five paralysis groups showed three categories of average values. The thoracic/thoracolumbar group had an average of 0 degree, groups 2 (distal L1/L2) and 3 (distal L3/L4) had average values of 5 degrees to 6 degrees, and groups 4 (distal L5) and 5 (distal S1) had average values of 13 degrees to 14 degrees. The average values of the apophyseal angle showed a characteristic curve: the values dropped from group 1 (67 degrees) to groups 2 (45 degrees) and 3 (34 degrees) and then rose in groups 4 (44 degrees) and 5 (52 degrees). The authors concluded that the insufficiency of the hip abductors leads to a reduction of the epiphysis angle, and the relative insufficiency of the small glutei compared with the knee extensors leads to a reduction of the apophysis angle.     

Experience in the management of myelomeningocele in Puerto Rico

The medical records of 128 children with myelomeningocele who were treated at the Pediatric University Hospital, Puerto Rico Medical Center, from January, 1980, to July, 1985, were reviewed retrospectively. The medical and surgical management during the first hospitalization of these children was studied in detail for predefined parameters. The average age at the time of myelomeningocele repair was 6.6 days. Statistical analysis showed that repair of the myelomeningocele defect before 48 hours of age did not reduce the occurrence of ventriculitis. The incidence of ventriculitis secondary to the management of the myelomeningocele lesion was 12.5%. Complications after repair of the myelomeningocele (including skin flap necrosis, cerebrospinal fluid leaks, and wound infection) were present in 43.8% of the patients who developed ventriculitis and in 19.0% of those who did not. This observation is statistically significant (p = 0.03) and indicates that complications of healing after myelomeningocele repair represent the most significant risk factor for the development of ventriculitis.     

The use of vesicostomy as permanent urinary diversion in the child with myelomeningocele.

PURPOSE: Treatment of the child with myelomeningocele presents the urologist with many challenges. While vesicostomies are usually used for temporary bladder drainage until continent reconstruction is performed, we have a population of postpubertal patients with myelomeningocele in whom vesicostomies have been preserved. We review our experience with these older children. MATERIALS AND METHODS: A search of our database of 350 patients with myelodysplasia identified 23 with urinary diversion accomplished by permanent vesicostomy. Those who had undergone vesicostomy after age 11 years were designated as group 1. Those who received a vesicostomy early in life that was maintained post puberty were designated as group 2. Indications for vesicostomy, upper tract status before and after vesicostomy, recurrent infections and complications were noted. Upper tract status was determined by serial ultrasounds during routine followup. RESULTS: Group 1 was comprised of 5 patients with mean age at vesicostomy of 17 years and a mean followup interval of 5.2 years. Group 2 was comprised of 18 patients with mean age of vesicostomy at 2.6 years and mean followup interval of 13 years. Hydronephrosis resolved in all children from both groups. Vesicostomy revision was required in 1 patient secondary to prolapse. Other complications included mild prolapse that did not require revision, stenosis requiring revision 5 months after the initial procedure and an early bilateral ureterovesical obstruction that resolved. Of the patients 33% had recurrent upper tract calculi. CONCLUSIONS: While the goal of achieving urinary continence is well worth striving for in the child with myelomeningocele, it is not realistic for all patients. We believe that permanent cutaneous vesicostomy is an acceptable alternative that the reconstructive surgeon should consider. This reliable and simple technique ensures safe decompression of the upper urinary tracts, while avoiding the complications of urinary diversion using segments of bowel.     

Retrospective review of L3 myelomeningocele in three age groups: should posterolateral iliopsoas transfer still be indicated to stabilize the hip?

The data from 58 hips in 29 myelodysplastic children with L3 paralysis has been reviewed retrospectively. Posterolateral transfer of the iliopsoas (Sharrard technique) was performed on all hips to correct muscle imbalance between 1975 and 1992. The average length of follow-up was 21 years. Preoperatively, 58.6% of the hips were either subluxated or dislocated (these were surgically reduced at the same stage). Radiological assessment revealed that 91.4% of the hips were stable at preschool age and 84.5% in adolescence. Of the patients 86% were functional walkers at preschool age and 75.8% in adolescence. Downward transitions in seven patients were related to the presence of scoliosis, spasticity and obesity. All patients who were community walkers in adolescence maintained their function in adult life at the time of the review. We conclude that the procedure has value in obtaining hip stability and walking ability in carefully selected patients with myelomeningocele.     

Longitudinal assessment of oxygen cost and velocity in children with myelomeningocele: comparison of the hip-knee-ankle-foot orthosis and the reciprocating gait orthosis.

Oxygen consumption and cost and velocity were evaluated over time in 23 children with myelomeningocele to determine whether differences exist when children walk with hip-knee-ankle-foot orthoses (HKAFOs) versus reciprocating gait orthoses (RGOs). Children using HKAFOs had similar oxygen cost as children using RGOs while achieving a faster velocity. Children walking with HKAFOs into adolescence had a faster velocity and lower oxygen cost than children who discontinued use of their HKAFOs. No significant differences in velocity or oxygen cost were found between children who continued to walk with RGOs and those who discontinued use of their RGOs. Upright ambulation may progress from ambulation with an RGO, when the child's upper extremity strength to mass ratio is low, to an HKAFO when upper extremity strength improves and velocity or keeping up with peers is of concern. Wheelchair mobility should be offered when speed and an energy-efficient method of community mobility are desired.     

Long-term follow-up of surgery for equinovarus foot deformity in children with cerebral palsy

The factors associated with failed operative intervention in the treatment of equinovarus foot deformity in children with cerebral palsy (CP) were evaluated after long-term follow-up. One hundred eight children with CP who had surgery on the posterior tibialis tendon (split tendon transfer, intramuscular lengthening, or Z-lengthening) on 140 feet were reviewed at a mean age of 16.8 years with 7.3 years of follow-up. The surgery was considered a failure when a 10 degrees or greater varus or valgus hindfoot deformity was present or if an additional operative intervention was required or planned. Involvement of CP, age at operation, and preoperative status of ambulation were significant factors in the outcome of the surgery. Hemiplegic patients demonstrated the best results, regardless of age or surgical procedure. Seventy-five percent of diplegic and quadriplegic patients who were younger than 8 years or who were not capable of community ambulation failed operative intervention, and surgery on the posterior tibialis tendon is not recommended in this group of patients.     

Neurological classification in myelomeningocele as a spine deformity predictor

In myelomeningocele, spinal deformities are responsible for major disability. Our aim was to check the predictive power for future spine deformity of a neurological classification applied at 5 years of age. We classified patients into four groups according to their neurological examination made at the age of 5 years. Groups were defined as follows: group I, L5 or below (meaning that all the patients in this group have a paralysis that at least leaves the L5 segment intact); group II, L3-L4; group III, L1-L2; group IV, T12 and above (meaning that all the patients in this group have a paralysis that reaches T11 or above). One hundred and sixty-three patients were included. The results showed that group I is a predictor for the absence of spinal deformity. Group III or IV is a predictor for spinal deformity. Group IV is a predictor of kyphosis. It was previously known that the higher the neurological level, the higher the rate of spinal deformity at maturity, but no work had given physicians a guideline to assess the spinal prognosis in myelomeningocele patients. Our work showed, on the basis of this classification made at the age of 5 years, that future spinal disorders may be expected in some patients, while no spinal deformity may be expected in some others. Thus, an appropriate therapeutic strategy and follow-up can be planned.     

Ambulation in patients with myelomeningocele: a multivariate statistical analysis

Factors determining ambulation in 163 patients with myelomeningocele were studied by a multivariate statistical method. Neurological dysfunction unrelated to the plaque was analyzed by magnetic resonance imaging. There were no ambulators at the thoracic or L1-L2 level. At the L3 level, 54% ambulated, and at the L4 level, 67% ambulated. Eighty percent were ambulators at L5 and all at the sacral level. Below L1-L2, one-half of the nonambulators had neurological deficiencies caused by syringohydromyelia or Chiari malformations preventing ambulation. Severe scoliosis was closely, age moderately, and hip flexion contracture slightly related to the inability of the other nonambulators to walk, while pelvic obliquity, hip dislocation, or knee flexion contracture was not.     

Efficacy of clinical-grade human placental mesenchymal stromal cells in fetal ovine myelomeningocele repair

BackgroundWhile fetal repair of myelomeningocele (MMC) revolutionized management, many children are still unable to walk independently. Preclinical studies demonstrated that research-grade placental mesenchymal stromal cells (PMSCs) prevent paralysis in fetal ovine MMC, however this had not been replicated with clinical-grade cells that could be used in an upcoming human clinical trial. We tested clinical-grade PMSCs seeded on an extracellular matrix (PMSC-ECM) in the gold standard fetal ovine model of MMC.MethodsThirty-five ovine fetuses underwent MMC defect creation at a median of 76 days gestational age, and defect repair at 101 days gestational age with application of clinical-grade PMSC-ECM (3 × 10⁵ cells/cm², n = 12 fetuses), research-grade PMSC-ECM (3 × 10⁵ cells/cm², three cell lines with n = 6 (Group 1), n = 6 (Group 2), and n = 3 (Group 3) fetuses, respectively) or ECM without PMSCs (n = 8 fetuses). Three normal lambs underwent no surgical interventions. The primary outcome was motor function measured by the Sheep Locomotor Rating scale (SLR, range 0: complete paralysis to 15: normal ambulation) at 24 h of life. Correlation of lumbar spine large neuron density with SLR was evaluated.ResultsClinical-grade PMSC-ECM lambs had significantly better motor function than ECM-only lambs (SLR 14.5 vs. 6.5, p = 0.04) and were similar to normal lambs (14.5 vs. 15, p = 0.2) and research-grade PMSC-ECM lambs (Group 1: 14.5 vs. 15, p = 0.63; Group 2: 14.5 vs. 14.5, p = 0.86; Group 3: 14.5 vs. 15, p = 0.50). Lumbar spine large neuron density was strongly correlated with motor function (r = 0.753, p<0.001).ConclusionsClinical-grade placental mesenchymal stromal cells seeded on an extracellular matrix rescued ambulation in a fetal ovine myelomeningocele model. Lumbar spine large neuron density correlated with motor function, suggesting a neuroprotective effect of the PMSC-ECM in prevention of paralysis. A first-in-human clinical trial of PMSCs in human fetal myelomeningocele repair is underway.     

Spinal fusion in children with spina bifida: influence on ambulation level and functional abilities

The aim of this study was to determine the influence of spinal fusion on ambulation and functional abilities in children with spina bifida for whom early mobilization was stimulated. Ten children (three males and seven females) with myelomeningocele were prospectively followed. Their mean age at operation was 9.3 years (standard deviation (SD): 2.4). Spinal curvature was measured according to Cobb. Pelvic obliquity and trunk decompensation were measured as well. The ambulation level was scored according to Hoffer, and functional abilities, as well as the amount of caregiver assistance, were documented using the Pediatric Evaluation of Disability Inventory. All patients were assessed before surgery and three times after surgery, with a total follow-up duration of 18 months after surgery. After spinal fusion, magnitude of primary curvature decreased significantly (p=0.002). Pelvic obliquity and trunk decompensation did not change. In spite of less immobilization as compared with other reported experiences, ambulation became difficult in three out of four patients who had been able to ambulate prior to surgery. Functional abilities and amount of caregiver assistance concerning self-care (especially regarding dressing upper and lower body, and self-catheterization) and mobility (especially regarding transfers) showed a nonsignificant trend to deterioration within the first 6 months after surgery, but recovered afterwards. From pre-surgery to 18 months after surgery, functional skills on self-care showed borderline improvement (p=0.07), whereas mobility did not (p=0.2). Mean scores on caregiver assistance improved significantly on self-care (p=0.03), and borderline on mobility (p=0.06), meaning that less caregiver assistance was needed compared with pre-surgery. The complication rate was high (80%). In conclusion, within the first 6 months after spinal fusion, more caregiver assistance is needed in self-care and mobility. It takes about 12 months to recover to pre-surgery level, while small improvement is seen afterwards. After spinal fusion, ambulation often becomes difficult, especially in exercise walkers. These findings are important for health-care professionals, in order to inform and prepare the patients and their parents properly for a planned spinal fusion.     

Pott's paraplegia in patients with severely deformed dorsal or dorsolumbar spines: treatment and prognosis

STUDY DESIGN: Pott's paraplegic patients with severe spinal deformity were reviewed retrospectively after being treated with chemotherapy and/or decompressive surgery. OBJECTIVES: To determine the most appropriate treatment protocol and to predict the prognosis for Pott's paraplegics with severe spinal deformity. SETTING: Catholic University of Korea Medical Center and Moon-Kim's Institute of Orthopedic Research, Seoul, Korea from 1971 to 1996. METHOD: In this study, there were 33 patients (eight children and 25 adults), ranging from 13 to 56 years of age. They developed spinal tuberculosis at the age of 9 years (range, 2-29 years), and remained neurologically symptom free from an average of 16 years (range, 4-27 years). Four adults who responded well to treatment initially suffered relapses of paraplegia. Only six patients had previously received a full course of triple chemotherapy. Seven (two children, five adults) had healed disease, and 26 (six children, 20 adults) had active disease. Eleven cases had frequent drainage from the sinuses. Kyphoscoliosis was found in 11 patients: four children and seven adults. The remaining patients had kyphosis only. Among the 26 patients with active tuberculosis, 10 had triple chemotherapy itself and the rest had additional decompression surgery (10 anterior and six posterior). All seven patients with healed tuberculosis were subjected only to surgery (two anterior and five posterior). RESULTS: In seven patients with healed tuberculosis, surgery did not improve neurologically except in one child patient. In four patients, the severity of paralysis remained unchanged and two patients, deteriorated neurologically after surgery. In six children with active tuberculosis, there were remarkable neurological recoveries by either conservative treatment or surgical decompression. Seven adults with active tuberculosis recovered slowly, improving by one or two Frankel grades (three Frankel C, three D, one E). In 11 out of 13 surgically treated adults with active disease, paralysis that had persisted for less than 3 months gradually improved by one or two Frankel grades. One Frankel A and one Frankel B paraplegic patients who had paralysis that had lasted through 6 months did not recover after surgery. CONCLUSION: The neurological recovery of Pott's paraplegics with severe spinal deformity resulted in three different outcomes: (1) severe deformity is different from moderate and mild deformities; (2) patients with healed tuberculosis had poorer prognosis than patients with active tuberculosis; (3) children had better prognosis than adults. Furthermore, patients with paralysis persisting over 6 months did not recover neurologically after surgery.     

Results in the treatment of paralytic calcaneus-valgus feet with the Westin technique

Between 1988 and 2003, 23 patients with paralytic calcaneus-valgus feet were submitted to the Westin procedure and 17 patients (25 feet) were re-evaluated. Nine patients were male and eight were female. The mean age at the surgical procedure was 8±5 years. The aetiology of paralysis was sequelae of poliomyelitis in 6 patients (8 feet) and of myelomeningocele in 11 patients (17 feet). The mean follow-up period was 6±6 years. The results were analysed clinically and radiographically considering the decrease of the retropulsion, the patient’s satisfaction, and the increase of the lateral tibiocalcaneal angle. Results were considered satisfactory when the patients showed a decrease of the retropulsion during gait, improvement of the gait pattern, and an increase of the tibiocalcaneal angle. As an overall result, 16 patients (94.2%) were satisfied and 1 patient (two feet) dissatisfied with the outcome. The increase of the tibiocalcaneal angle was significant for the myelomeningocele patients (P=0.001), but not for poliomyelitis (P=0.053). No statistical relation between the follow-up period and the increase of the tibiocalcaneal angle was found (r=0.04). The authors concluded that the Westin procedure is a good technique for the treatment of paralytic calcaneus valgus feet with myelomeningocele.     

Associations between orthopaedic findings, ambulation and health-related quality of life in children with myelomeningocele

Purpose Modern principles for treatment of patients with myelomeningocele include early closure of the neural tube defect, neurosurgical treatment of hydrocephalus and treatment aimed at minimizing contractures and joint dislocations. The aim is to achieve a better survival rate and a better quality of life (QOL). Better ambulatory function is thought to improve the management of activities of daily living. This study focused on evaluating which factors might affect ambulation, function and health-related QOL in children with myelomeningocele. Methods Thirty-eight patients with neurological deficit from myelomeningocele were examined in an unbiased follow-up. This included a physical examination using validated methods for ambulatory function and neuromuscular status, chart reviews and evaluation of radiographs in terms of hip dislocation and spine deformity. The Pediatric Evaluation of Disability Inventory (PEDI) was used to measure mobility, self-care and social function, and the Child Health Questionnaire (CHQ-PF50) was used to measure QOL. Results Muscle function class, quadriceps strength, spasticity in hip and/or knee joint muscles and hip flexion contracture as well as the ambulatory level all affected functional mobility as well as self-care/PEDI. Patients with hip dislocation, spinal deformity or those who were mentally retarded also had significantly worse functional mobility. Besides being affected by the severity of the neurological lesion, self-care/PEDI was significantly impaired by hip flexion contracture and absence of functional ambulation. General health-related QOL was significantly lower in this patient group than for US norms. Nonambulatory and mentally retarded patients had a significantly lower physical function of their QOL (CHQ). Conclusions The severity of the disease, i.e. reduced muscle strength and occurrence of spasticity around hip/knee, affected ambulation, functional mobility and self-care. Acquired deformities (hip dislocation and spine deformity) affected functional ambulation only. Patients with reduced functional mobility and self-care experienced lower physical QOL. Children with myelomeningocele had significantly reduced QOL compared to healthy individuals.     

The paralysis associated with myelomeningocele: clinical and experimental data implicating a preventable spinal cord injury

Paralysis seen in children with myelomeningocele has been attributed to congenital myelodysplasia. We suspected that paralysis may be due in part to a spinal cord injury caused by exposure of the neural tube to the amniotic fluid. This hypothesis was tested using a fetal rat model of surgically created dysraphism. Each pup from the experimental group of rats in which the spinal cord was intentionally exposed to the amniotic fluid was born with severe deformity and weakness of the hind limbs and tail. Control fetal rats, subjected to the same procedure without directly exposing the spinal cord to the intrauterine environment, were normal at birth. Histological studies of the exposed spinal cord revealed extensive erosion and necrosis, findings similar to those described in children with myelomeningocele. We therefore propose a "two-hit" hypothesis to explain the paralysis seen in children with myelomeningocele: congenital myelodysplasia complicated by an intrauterine spinal cord injury. Intrauterine protection of the exposed spinal cord might prevent some or all of the paralysis. The possible implications of these findings for the future treatment of myelomeningocele are discussed.     

Outcome following hindbrain decompression of symptomatic Chiari malformations in children previously treated with myelomeningocele closure and shunts.

Between 1975 and 1989, 25 children treated with myelomeningocele closure and shunting for hydrocephalus at the Children's Hospital of Pittsburgh developed progressive lower brain-stem dysfunction from their Chiari malformation. Retrospective univariate and multivariate analyses of these cases were undertaken to assess the relationship between preoperative clinical factors and postoperative outcome. Since earlier reports have suggested that neonates with symptomatic Chiari malformations show a less favorable response than older children to craniocervical decompression, particular attention was directed at examining the effect of age on preoperative symptoms and postoperative outcome. Patients were subdivided by age into two groups, namely: 13 patients who became symptomatic before 2 months of age (neonatal group) and 12 older infants and children who developed initial symptoms between 6 months and 10 years of age. Once symptoms developed, patients in both groups deteriorated progressively until brain-stem decompression was performed. The mode of presentation and the rate and extent of neurological deterioration differed substantially in the two groups. Whereas the neonates typically showed rapid neurological deterioration and often manifested profound brain-stem dysfunction within a period of several days, the older patients experienced a more insidious symptom progression and rarely demonstrated the severe degree of impairment seen in the neonates. All patients underwent suboccipital craniectomy, cervical laminectomy, and dural decompression. A shunt from the fourth ventricle and/or syrinx to the subarachnoid space was placed in those with significant syringomyelia. Following surgery, 17 patients had complete or nearly complete resolution of all signs of brain-stem compression, three had mild to moderate residual deficits, and five showed no improvement. Outcome correlated closely with the preoperative neurological status. In particular, the presence of bilateral vocal cord paralysis was associated with a poor response to surgery (p < 0.001 on both univariate and multivariate analyses). Of the six patients (all neonates) who progressed to complete bilateral vocal cord paralysis before surgery, only one improved. In contrast, all patients with less profound but nonetheless severe deficits recovered function postoperatively. Although the neonates as a group had a poorer outcome than did the older patients (p = 0.02 on univariate analysis), this in large part reflected their more severe preoperative impairments; neonates who still had some preservation of vocal cord function before surgery subsequently did as well as the older patients. Accordingly, age did not prove to be an independent prognostic factor on multivariate analysis. Taken together, these results indicate that, in most patients with symptomatic Chiari II malformations (including neonates), neurological deficits are potentially reversible if hindbrain decompression is performed expeditiously.     

Urodynamic findings in children with myelomeningocele after untethering of the spinal cord

PURPOSE: The effect on bladder function of tethered spinal cord release in individuals with myelomeningocele differs in published studies, and the benefit has been the subject of debate. We studied the urodynamic findings in a consecutively treated population. MATERIALS AND METHODS: Of 120 children born with myelomeningocele between 1986 and 1999, 20 underwent surgery for the tethered cord syndrome at a median age of 8 years (range 2 to 13). Time points for cystometry used for comparison were before symptoms at a median age of 20 months (range 9 to 106), a median of 5 months preoperatively (1 to 33) and at 12 months postoperatively (6 to 19). Severe bladder dysfunction was defined as basal pressure more than 40 and/or overactivity greater than 60 cm H2O, moderate dysfunction as basal pressure 20 to 40 and/or overactivity 20 to 60 cm H2O, and mild dysfunction as basal pressure less than 20 cm H2O. RESULTS: Of the 20 patients who underwent untethering 7 had improvement to a milder dysfunctional level, while 1 deteriorated postoperatively. Six patients experienced deterioration in bladder dysfunction level before untethering, and all returned to a milder level afterward. A total of 10 patients had unchanged bladder dysfunction before symptoms and preoperatively. Of these patients 9 (90%) continued unchanged after untethering, while 1 deteriorated. CONCLUSIONS: After untethering secondary to myelomeningocele 35% of the patients experienced improved bladder function and 5% deteriorated. All of the patients who deteriorated before untethering improved afterward, and 90% of those who were stable preoperatively continued to be stable postoperatively. Therefore, regular evaluation of bladder function in children with myelomeningocele should be performed.     

Intrauterine structure of foot muscles in talipes equinovarus due to high-level myelomeningocele: a light microscopic study in fetal cadavers

The purpose of this study was to investigate the light microscopic structure of extrinsic foot muscles in talipes equinovarus (TEV) deformity that developed during intrauterine life due to high-level myelomeningocele. Ten feet of five fetal cadavers ranging in age from 18 to 20 weeks were dissected. Five feet had typical TEV deformity and the other five feet did not have any deformity (control group). Under light microscopic examination quantitative measurement of both muscle fiber sizes and fibrosis in the muscle tissue were performed to investigate the denervation muscle atrophy. Mean muscle fiber size of the TEV group was found to be significantly lower than that of the control group in all foot muscles except the gastrocnemius muscle. The proportion of fibrosis due to denervation atrophy was significantly higher in the TEV group than in the control group in all muscles. This situation was most evident in the peroneus longus muscle. It was concluded that muscular imbalance due to significant muscular atrophy might be the cause of TEV deformity that developed during intrauterine life due to high-level myelomeningocele.