Åsa Bartonek

Factors influencing ambulation in myelomeningocele: a cross-sectional study

A consecutive series of 53 children with myelomeningocele (mean age 7.6, range 3.2 to 11.4 years) was assessed in order to see if the children with motor paresis of the lower limbs achieved the expected level of ambulation, and if not, to identify possible causative factors. Methods used were clinical examination of orthopaedic and neurological status, information from medical reports, and documentation of orthoses use. Functional skills were documented and energy expenditure was examined. Thirty-one of 53 children had reached the expected ambulation considered possible according to their motor paresis, whereas 22 of the 53 performed worse than expected. Balance disturbances, occurrence of spasticity in knee and hip joints, and number of shunt revisions made differed significantly between the groups that achieved and did not achieve expected ambulation. Functional skills of mobility differed significantly between two muscle-function levels in children who had walking ability. Energy expenditure was higher in the non-achieving group than in the group who achieved expected ambulation in each of the muscle-function levels. Results show that children with similar muscle paresis exhibit different ambulatory function. This indicates the importance of a close analysis of other factors which may cause ambulation to deteriorate in order to predict future ambulation in children with myelomeningocele.

Centre of mass motion during gait in persons with myelomeningocele

The movement of the centre of mass in the vertical and lateral directions during gait in children with myelomeningocele was analyzed. The children were classified into five groups depending on the successive paresis of lower limb muscle groups and compared to a control group. In the groups with dorsi- and plantarflexor weakness, the excursions increased and an anterior trend in the centre of mass was observed. In the groups with additional abductor paresis, the lateral excursion was highest and the vertical excursion low due to increased transverse and frontal motion and reduced sagittal motion. With further paresis of the hip extensors, the centre of mass was more posteriorly positioned due to compensatory trunk extension. Improved understanding of individual childrens solutions to their muscle paresis can be obtained by visualizing the centre of mass relative to the pelvis. Centre of mass analyses in myelomeningocele offer an important complement to standard gait analysis.

Characteristic gait kinematics in persons with lumbosacral myelomeningocele

Thirty self-ambulatory children with mid-lumbar to low-sacral myelomeningocele who walked without aids and 21 control children were evaluated by three-dimensional gait analysis. Characteristic kinematic patterns and parameters in the trunk, pelvis, hip, knee and ankle were analyzed with respect to groups with successive weakness of the ankle plantarflexor, ankle dorsiflexor, hip abductor, hip extensor and knee flexor muscles. Extensive weakness of the plantarflexors resulted in kinematic alterations in the trunk, pelvis, hip and knee and in all three planes seen as knee flexion, anterior pelvic tilt and trunk and pelvic rotation. Additional extensive weakness of the dorsiflexors made little difference in the walking strategy. Large kinematic alterations in all planes were observed where there was a large extent of additional weakness of the hip abductor but strength remaining in the hip extensors. In this group, gait was characterized by large lateral sway of the trunk, rotation of the trunk and pelvis, pelvic hike and increased extension of the knees. In the group with total poresis hip extensors but yet some knee flexion, gait was similar to the previous group but there was less sagittal plane movement greates and posterior trunk tilt. Gait analysis provides an understanding of the compensatory strategies employed in these patients. Clinical management can be directed towards stabilizing the lower extremities and accommodating large upper body motion to preserve this method of self-ambulation even in children who have considerable hip extensor and abductor weakness.

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

PurposeModern 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.MethodsThirty-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.ResultsMuscle 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).ConclusionsThe 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.

Upper body movement during walking in children with lumbo–sacral myelomeningocele

Eight children with lumbo-sacral myelomeningocele (MMC) underwent three-dimensional movement analysis to determine whether or not differing levels of lower extremity strength affected the extent of shoulder, trunk and pelvis movement during independent walking when wearing orthoses. Fourteen control children were also investigated. The patterns of upper body movements in all MMC children were well defined and consistent, showing small standard deviations from the mean. In the frontal and transverse planes, segment displacements of the MMC children assigned into Group II (hip extensor and abductor muscle strength grade 0-2) were almost twice that of the MMC children in Group I (hip extensor and abductor muscle strength grade 3-4). All segment displacements in the frontal, transverse and sagittal planes for Group I and Group II children were significantly greater than those for the controls. In the frontal plane these differences were approximately 4-10 times greater, with the Group II children having the largest peak-to-peak displacements. These results indicate that the motion amplitudes of the upper body segments are related to the degree of muscle weakness of the lower limbs. No significant differences were found when comparing segment motions during walking with either the Ferrari type knee-ankle-foot or ankle-foot orthoses.

Ambulation in patients with myelomeningocele : A 12-year follow-up

Factors determining change in ambulatory status were studied over a 12-year observation time in 60 ambulating patients with myelomeningocele. There were 26 female and 34 male subjects with a median age of 22 years (range, 12-54). We used the method of Lindseth to define the neurologic level of the lesion and classified walking ability according to the criteria of Hoffer. The prevalence of spasticity and spine and lower-limb deformities was assessed. Orthopedic and neurosurgical interventions and other medical events were registered, as well as occurrence of pressure sores, musculoskeletal pain, and use of orthoses. There were 19 patients with downward transitions in ambulatory level during the follow-up time. Factors explaining deterioration in these 19 patients included deterioration of the neurologic level of lesion, spasticity, knee and hip flexion contractures, low-back pain, lack of motivation, as well as those of major medical events like stroke, recurrent septicemia, lower limb edema, and invasive surgical interventions.

Comparison of different systems to classify the neurological level of lesion in patients with myelomeningocele

In 73 patients with myelomeningocele (mean 17.2 years, range 5 to 40 years) the classification of level of lesion was studied according to six commonly used classification systems and to the ambulation groups of Hoffer. The distribution of the patients into classes of thoracic‐level lesions was the same for four classification systems in 10 patients and for two classification systems into categories of level L3 in 14 patients. For the other patients discrepancies occurred between systems for lesions of level L3 and downward. None of the patients was consistently categorized in the functional ambulation groups of Hoffer using all classification systems. The results show that it is not possible to compare neurological lesion levels classified according to the different systems described in this study and consequently that the distribution into the functional ambulation groups of Hoffer varies. To enhance communication and facilitate comparing the results of treatment we suggest using some basic criteria for patient documentation.

Does botulinum toxin A improve the walking pattern in children with idiopathic toe-walking?

BackgroundNumerous recommendations have been made for treating idiopathic toe-walking (ITW), but the treatment results have been questioned. The purpose of this study was to investigate whether botulinum toxin A (BTX) improves the walking pattern in ITW as examined with 3-D gait analysis.Participants and methodsA consecutive series of 15 children (aged 5–13 years) were enrolled in the study. The children underwent a 3-D gait analysis prior to treatment with a total of 6 units/kg bodyweight Botox® in the calf muscles and an exercise program. The gait analysis was repeated 3 weeks and 3, 6, and 12 months after treatment. A classification of toe-walking severity was made before treatment and after 12 months. The parents rated the perceived amount of toe-walking prior to treatment and 6 and 12 months after treatment.ResultsEleven children completed the 12-month follow-up. The gait analysis results displayed a significant improvement, indicating decreased plantarflexion angle at initial contact and during swing phase and increased dorsiflexion angle during midstance at all post-treatment testing instances. According to the parents’ perception of toe-walking, 3/11 children followed for 12 months had ceased toe-walking completely, 4/11 decreased toe-walking, and 4/11 continued toe-walking. After 6–12 months, the toe-walking severity classification improved in 9 of the 14 children for whom data could be assessed.ConclusionsA single injection of BTX in combination with an exercise program can improve the walking pattern in children with ITW seen at gait analysis, but the obvious goal of ceasing toe-walking is only occasionally reached.

EQ‐5D‐Y as a health‐related quality of life measure in children and adolescents with functional disability in Sweden: testing feasibility and validity

The EQ‐5D‐Y is a newly developed generic instrument measuring health‐related quality of life in children and adolescents.

Motor development toward ambulation in preschool children with myelomeningocele--a prospective study.

Purpose: To describe motor development toward ambulation in children with myelomeningocele. Methods: Forty-three children were followed prospectively from 6 months to 6 years of age. Results: Walking function had been achieved at the 1-year follow-up in 2 of 38 children, at the 1.5-year follow-up in 7 of 39, at the 2-year follow-up in 14 of 36, at the 3-year follow-up in 21 of 28, at the 4-year follow-up in 28 of 36, and at the 6-year follow-up in 30 of 38. At the 6-year follow-up, spasticity was present in 22 of 38 children, 42 of 43 used orthoses, and 9 children had not achieved ambulation expected with respect to muscle function. Conclusions: In children with myelomeningocele, walking starts in some during the first year of life and is seen increasingly more frequently until 6 years of age. Motor development before ambulation varies among children with similar muscle function. An increased incidence of spasticity is found among those not having achieved ambulation with respect to muscle function.