Occupation Based Telerehabilitation Intervention for Adolescents with Myelomeningocele: A Pilot Study.

AIMS: This pilot study examined the impact of an occupation based intervention using a telerehabilitation format with adolescents with myelomeningocele (MMC). METHODS: We conducted a nonrandomized pilot study including four adolescents ages 14-18 with MMC. The intervention program included 10-12 sessions of the Cognitive Orientation to daily Occupational Performance remotely delivered via videoconferencing. Outcome measures included the Canadian Occupational Performance Measure (COPM), Wee-Functional Independence Measure;(Wee-FIM), and the Pediatric Quality of Life Inventory (PedsQL). Assessments were administered at baseline, post intervention and at three-month follow-up. RESULTS: Following intervention, participants rated their performance as having improved 2 points on the COPM for 8 out of 12 trained goals and on 2 out of 8 untrained goals. At 3-month follow-up improvement was reported on 9 out of 12 trained goals and 3 out of 8 untrained goals All participants made clinically significant improvements on the Wee-FIM total score following intervention and improvements were maintained at 3-month follow-up. The intervention effect on the PedsQL was inconclusive. Feedback interviews suggested that participants experience high satisfaction from the results and implementation of the intervention. CONCLUSIONS: Our results demonstrate potential efficacy of occupation based teleintervention for adolescents with MMC.

Daily Physical Activity in Children and Adolescents with Low Lumbar and Sacral Level Myelomeningocele.

PURPOSE: Children with low lumbar and sacral level myelomeningocele are expected to be community ambulators. However, they do not always meet the expected ambulation level by the time they reach adulthood. The purpose of this study is to investigate daily activity energy expenditure and physical activity levels of these patients in daily routine. MATERIALS AND METHODS: 20 patients and 20 healthy peers were included in this case-control study. Omnidirectional accelerometer monitor was used for assessing activity energy expenditure, and daily durations spent in sedentary, light activity, and moderate to vigorous physical activity levels (NCT04186338). RESULTS: Mean activity energy expenditure and moderate to vigorous physical activity duration were significantly lower in the patient group, while age was positively correlated with sedentary time and negatively correlated with light activity time. CONCLUSIONS: In patients with myelomeningocele, increasing daily activity energy expenditure and moderate to vigorous physical activity and, especially as age progresses, decreasing the sedentary behavior may assist in providing the expected ambulation level. These patients should be encouraged to partake in regular exercise programs.

Seeking tirelessly for better health and life conditions for the child with myelomeningocele / Buscando incansavelmente por melhores condições de saúde e vida para o filho com mielomeningocele / Buscando sin cesar mejores condiciones de salud y de vida para el niño con mielomeningocele

Objective: to understand the experience of families in the care of children with myelomeningocele. Method: a qualitative research study, which adopted Symbolic Interactionism and Grounded Theory as its theoretical and methodological framework, and the semi-structured interview as a data collection instrument. Twenty-eight participants from thirteen families living in a municipality in the inland of São Paulo took part in the study. Results: the family tirelessly seeks better health and life conditions for the child with myelomeningocele; aiming at the child's autonomy, it is mobilized to the necessary treatments, to learn the care, to adapt the routine, as well as to supply for all the child's needs so that it reaches potential development, looking for a less dependent future with inclusion and social ascension. Conclusion: family nursing shows potential support in the face of myelomeningocele, due to its ability to apprehend the family system, evaluate it and enable intervention proposals in the care process. In this study, rehabilitation nursing was also emphasized, since it goes together with the child and the family who experience myelomeningocele.

Objetivo: compreender a experiência de famílias no cuidado de crianças com mielomeningocele. Método: pesquisa qualitativa, que adotou como referencial teórico e metodológico o Interacionismo Simbólico e a Teoria Fundamentada nos Dados, e a entrevista semiestruturada como instrumento de coleta de dados. Vinte e oito participantes de treze famílias residentes em um município do interior paulista integraram o estudo. Resultados: a família busca incansavelmente por melhores condições de saúde e vida para o filho com mielomeningocele; objetivando a autonomia da criança, se mobiliza aos tratamentos necessários, a aprender os cuidados, a adaptar a rotina, assim como suprir por todas as necessidades da criança a fim de que esta alcance desenvolvimento potencial, prospectando um futuro menos dependente com inclusão e ascensão social. Conclusão: a enfermagem familiar mostra-se apoio potencial frente à condição da mielomeningocele, devido à sua capacidade de apreender o sistema familiar, avaliá-lo e possibilitar proposições de intervenção no processo de cuidado. Neste estudo enfatizou-se também a enfermagem de reabilitação, uma vez que esta caminha conjuntamente à criança e à família que experienciam a mielomeningocele.

Objetivo: comprender la experiencia de las familias en el cuidado de niños con mielomeningocele. Método: investigación cualitativa, que adoptó el Interaccionismo Simbólico y la Teoría Fundamentada como marco teórico y metodológico, y la entrevista semiestructurada como instrumento de recolección de datos. Participaron del estudio 28 participantes de trece familias residentes en una ciudad del interior de São Paulo. Resultados: la familia busca sin cesar mejores condiciones de vida y salud para el niño con mielomeningocele; con el objetivo de estimular la autonomía del niño, se realizan los tratamientos necesarios, se aprenden los cuidados, se adecua la rutina y se satisfacen todas las necesidades del niño para que alcance su desarrollo potencial, proyectando un futuro menos dependiente con inclusión y ascensión social. Conclusión: la enfermería familiar muestra un potencial apoyo frente a la condición de mielomeningocele, debido a su capacidad para aprehender el sistema familiar, evaluarlo y posibilitar propuestas de intervención en el proceso de atención. En este estudio también se enfatizó la enfermería de rehabilitación, ya que va de la mano del niño y la familia que padecen mielomeningocele.

Relationship Between Motor Level and Wheelchair Transfer Ability in Spina Bifida: A Study From the National Spina Bifida Patient Registry.

OBJECTIVE: To identify the specific features that contribute to the variability in baseline wheelchair transfer and the changes in transfer ability (gain or loss) over time for a large cohort of patients with spina bifida (SB) in the National Spina Bifida Patient Registry. DESIGN: Longitudinal cohort study. SETTING: A total of 35 United States outpatient SB clinic sites. PARTICIPANTS: Individuals (N=1687) with SB ages 5-73 (median, 13.33) years who were therapeutic ambulators or nonambulators. INTERVENTION: Not applicable. MAIN OUTCOME MEASURE: Ability to transfer from a wheelchair to another level surface. RESULTS: Bayesian Network Analysis was used to reduce the initial variable set to the following predictors: SB subphenotype, motor level, age, insurance, sex, race, ethnicity, surgical procedures, and number of visits. We used a multinomial logistic model with Wald Chi-square analysis of effects to examine the relationships between transfer ability and predictors. A total of 295 of 1687 eligible patients (17.56%) with myelomeningocele (MMC) and 6 of 58 eligible patients (10.32%) with non-MMC experienced changes in transfer ability during the period of the study. For those with MMC and non-MMC, the highest number of individuals exhibiting changes in motor level had changes from thoracic to high-lumbar, high-lumbar to thoracic, high-lumbar to midlumbar, and midlumbar to high-lumbar lesion levels. Results of the Bayesian Network Analysis revealed that motor level was the predominant factor associated with baseline transfer ability followed by age. The combination of SB sub phenotype, motor level, age, insurance status, number and type of surgical procedures, and time point accurately classified the loss, gain, or no change in transfer ability 82.7% of the time. CONCLUSIONS: Motor level was the predominant factor associated with baseline transfer ability, and the change in transfer ability was directly related to a corresponding change in motor level that might be explained by changes in muscle strength of the iliopsoas and quadriceps.

Early Treadmill Practice in Infants Born With Myelomeningocele: A Pilot Study.

PURPOSE: To determine the feasibility of an early treadmill training program for infants with myelomeningocele (MMC) and to measure changes in overt infant motor development and control, including mechanisms underlying the overt changes. METHODS: Ten infants with MMC were initially enrolled: 8 infants completed 12 consecutive months of training, and 2 completed 6 months of training. Training consisted primarily of home-based, parent-administered treadmill stepping practice 5 days per week, 10 minutes per day starting within 6 months postbirth. We measured motor milestones, treadmill steps, spinal-level reflexes, and body composition. RESULTS: Infants showed earlier acquisition of gross motor skills than previously reported. The number of alternating steps performed increased, indicating more complex neuromotor control and strength. Integrity of monosynaptic pathways and body composition were improved after controlling for chronological age. CONCLUSIONS: This study demonstrates the feasibility of using early, home-based treadmill training for infants with MMC starting within 6 months postbirth.

Functional Mobility Improved After Intensive Progressive Resistance Exercise in an Adolescent With Spina Bifida.

PURPOSE: To describe the use and effectiveness of a novel intensive progressive resistance exercise (PRE) approach to address the functional goals of a 14-year-old adolescent with a myelomeningocele. SUMMARY OF KEY POINTS: The child had lower extremity weakness, knee and hip flexion contractures, impaired somatosensation, and cardiopulmonary deconditioning, affecting gait mechanics and functional ambulation. An 8-week intensive PRE-based intervention was designed to improve walking in the home by targeting both power-generating and stabilizing lower extremity musculature. Secondary intervention focused on cardiopulmonary endurance training. CONCLUSIONS: The child demonstrated improvements in gait speed, walking endurance, and functional lower extremity strength. Knee contracture was moderately responsive to sustained stretching and positioning. WHAT THIS CASE ADDS TO EVIDENCE-BASED PRACTICE: This intensive PRE training approach had been effective for improving function among youth with cerebral palsy, and to our knowledge it had not yet been applied to youth with other neurological conditions.

Independence in self-care activities in children with myelomeningocele: exploring factors based on the International Classification of Function model.

PURPOSE: To investigate the relationship between disease factors, body functions and structures, activities, personal and environmental factors with independence in self-care activities in children with myelomeningocele (MMC), utilizing the International Classification of Function model. METHOD: 113 children with MMC, aged 3-18 years were assessed during an annual MMC-clinic visit. Data on the personal and environmental factors, disease factors, hand function, mobility, visual perception, cognition, and communication were assessed in relation to the Pediatric Evaluation of Disability Inventory (PEDI) self-care domain. RESULTS: The mean PEDI caregiver assistance score was 22.1 (ranged between <10 and 62) well below the norms. The most significant determinant of dependence in self-care activities was the presence of hydrocephalus, a child with hydrocephalus scored a mean of 9.2 points less on the PEDI caregiver scale (p = 0.02). By using a linear regression, the most significant factors associated with the PEDI scores among children with hydrocephalus were the age of the child, function of the non-dominant hand, ambulation, and cognitive function. CONCLUSION: In children with MMC the presence of hydrocephalus appears to be more significantly associated with functional independence than the neurological level. The International Classification of Function model provides an in-depth multidisciplinary model that facilitates in investigating independence in self-care activities in children with MMC. It is important to take personal factors and various functional impairments into account when working with these children to promote independence. Implications for Rehabilitation When working with children with myelomeningocele on independence in daily living activities, it is necessary that health professionals demonstrate a holistic view of the child and his/her disability. Health professionals should take into account information on all factors of the International Classification of Function, in order to help the children, achieve maximal independence. It is important for health professionals to consider presence or lack of hydrocephalus when working with children with myelomeningocele to achieve functional gains. Improving upper extremity function and cognitive ability in children with myelomeningocele may help promote independence in activities of daily living. It is necessary for health professionals to continue to work with children with myelomeningocele on maximizing independence as they get older, for it is possible for them to achieve independence at a later age. While this study did not explore participation, this is an essential part of the International Classification of Function and is generally related to function. Participation allows a child to be involved in life situations, contributes to life satisfaction and prepares children for critical adult roles. It is therefore important to assess and monitor this measure. Further studies should examine associations between independence in self-care activities and participation.

Urologic self-management through intermittent self-catheterization among individuals with spina bifida: A journey to self-efficacy and autonomy.

PURPOSE: To describe the age of independence in intermittent self-catheterization (ISC) in a diverse patient population and identify factors associated with ISC in individuals with spina bifida. METHODS: Two hundred patients with myelomeningocele or lipomyelomeningocele, who were ⩾ 3 years of age and utilized catheterization for bladder management were included. Data regarding diagnosis, functional level of lesion, race, ethnicity, presence of shunt, method of catheterization, self-management skills, fine motor skills, and cognitive abilities were collected. RESULTS: Fifty-five percent of individuals were able to perform ISC with a mean age of 9.45 years (SD = 2.97) and 22.7% used a surgically created channel. Higher level of lesion and female gender were associated with a lower rate of ISC. Intellectual disability was present in 15% of the individuals able to perform ISC and in 40% of those not able to perform ISC (p= 0.0005). Existent self-efficacy regarding activities of daily living (i.e. dressing, bathing, skin care) were associated with ISC (p< 0.0001). CONCLUSIONS: The average age of ISC emerged as a target for culturally-appropriate educational interventions to stimulate greater early independence. Future research on factors that may foster an 'independent spirit' early in childhood leading to self-management are warranted.

Health self-management, transition readiness and adaptive behavior in persons with cerebral palsy or myelomeningocele.

PURPOSE/OBJECTIVE: This study was conducted to examine the associations between generic and condition-specific health self-management and levels of adaptive behavior in 2 groups of transition-age youth with congenital neurodevelopmental conditions. METHOD: The sample included 43 adolescents/young adults diagnosed with cerebral palsy (CP) and 36 with spina bifida/myelomeningocele (SBM), ages 13-29, mean age 18.96 (4.77), 51.9% female. Health self-management was assessed with the Transition Readiness Assessment Questionnaire (TRAQ) completed by the child, and the Kennedy Krieger Independence Scales-Spina Bifida (KKIS-SB) completed by the parent/guardian. The Adaptive Behavior Assessment System (ABAS-II) completed by the parent/guardian was used to assess levels of adaptive behavior. RESULTS: There were significant group differences in condition-specific health self-management, including lower KKIS-SB Initiation of Routines and KKIS-SB Prospective Memory scores in the group with SBM. Those differences were no longer significant when scoring was modified to account for item applicability. Group differences in generic health self-management and adaptive behavior were not significant. There were significant differences in the correlations between health self-management instruments and ABAS-II composite scores. CONCLUSIONS: For youth with congenital neurodevelopmental conditions who are in the transition to adulthood, there are important condition-specific self-management needs that are not captured by measuring generic transition readiness or adaptive behavior. Findings highlight the need for clinicians to assess health self-management needs from multiple perspectives, utilizing generic and condition-specific measures that can inform targeted interventions and supports for optimal independence. (PsycINFO Database Record

Mastery motivation and executive functions as predictors of adaptive behavior in adolescents and young adults with cerebral palsy or myelomeningocele.

PURPOSE/OBJECTIVE: To examine mastery motivation and executive functions or behaviors as predictors of adaptive behavior in adolescents and young adults with congenital neurodevelopmental conditions. METHOD: Participants were 2 groups of adolescents and young adults, ages 13-29, including 43 with cerebral palsy and 36 with myelomeningocele living with a parent or caregiver. Participants completed measures of mastery motivation, executive functions or behaviors, and a measure of adaptive behavior. RESULTS: Group differences in mastery motivation, executive functions and executive behaviors, and adaptive behavior profiles were not significant. Mastery motivation, executive functions, and executive behaviors explained a significant portion of variance in adaptive behavior. CONCLUSIONS: Findings highlight the importance of assessing and addressing motivational and executive needs in developing interventions to promote independence. Findings also suggest the need for more comprehensive assessment of adaptive behaviors that include the ability to self-direct others in the completion of tasks necessary for successful daily functioning. (PsycINFO Database Record

Conventional physical therapy and physical therapy based on reflex stimulation showed similar results in children with myelomeningocele.

We aimed to investigate whether infants with myelomeningocele would improve their motor ability and functional independence after ten sessions of physical therapy and compare the outcomes of conventional physical therapy (CPT) to a physical therapy program based on reflex stimulation (RPT). Twelve children were allocated to CPT (n = 6, age 18.3 months) or RPT (n = 6, age 18.2 months). The RPT involved proprioceptive neuromuscular facilitation. Children were assessed with the Gross Motor Function Measure and the Pediatric Evaluation of Disability Inventory before and after treatment. Mann-Whitney tests compared the improvement on the two scales of CPT versus RPT and the Wilcoxon test compared CPT to RPT (before vs. after treatment). Possible correlations between the two scales were tested with Spearman correlation coefficients. Both groups showed improvement on self-care and mobility domains of both scales. There were no differences between the groups, before, or after intervention. The CPT and RPT showed similar results after ten weeks of treatment.

Conventional physical therapy and physical therapy based on reflex stimulation showed similar results in children with myelomeningocele / Fisioterapia convencional e fisioterapia com estimulação reflexa apresentaram resultados semelhantes em crianças com mielomeningocele

ABSTRACT We aimed to investigate whether infants with myelomeningocele would improve their motor ability and functional independence after ten sessions of physical therapy and compare the outcomes of conventional physical therapy (CPT) to a physical therapy program based on reflex stimulation (RPT). Twelve children were allocated to CPT (n = 6, age 18.3 months) or RPT (n = 6, age 18.2 months). The RPT involved proprioceptive neuromuscular facilitation. Children were assessed with the Gross Motor Function Measure and the Pediatric Evaluation of Disability Inventory before and after treatment. Mann-Whitney tests compared the improvement on the two scales of CPT versus RPT and the Wilcoxon test compared CPT to RPT (before vs. after treatment). Possible correlations between the two scales were tested with Spearman correlation coefficients. Both groups showed improvement on self-care and mobility domains of both scales. There were no differences between the groups, before, or after intervention. The CPT and RPT showed similar results after ten weeks of treatment.

RESUMO O estudo investigou se crianças com mielomeningocele melhorariam sua habilidade motora/funcional após dez sessões de fisioterapia e comparou o quadro motor de um grupo submetido à fisioterapia convencional (FC) com outro tratado com fisioterapia com estimulação reflexa (RF). Doze crianças foram alocadas em FC (n=6, 18,3 meses de idade) ou FR (n=6, 18,2 meses de idade). FR envolveu facilitação neuromuscular proprioceptiva. As crianças foram avaliadas com a Medida de Função Motora Grossa (GMFM) e o Inventário de Avaliação Pediátrica de Incapacidade (PEDI) antes e depois do tratamento. Testes de Mann-Whitney compararam a melhora na GMFM e PEDI (FC versus FR) e testes de Wilcoxon compararam FC e FR (antes versus depois). Possíveis correlações entre GMFM e PEDI foram testadas por coeficientes de Spearman. Ambos os grupos melhoraram na GMFM e PEDI (domínios autocuidado e mobilidade). Não houve diferença entre os grupos antes e após a intervenção. FC e FR apresentaram efeitos semelhantes depois de dez semanas de tratamento.

Rehabilitation of children and adolescents with myelomeningocele: the daily life of mothers-caregiversRehabilitation of children and adolescents with myelomeningocele: the daily life of mothers-caregivers. / Reabilitação de crianças e adolescentes com mielomeningocele: o cotidiano de mães cuidadoras.

OBJECTIVE: To analyze the daily routine of mothers-caregivers of children and adolescents with myelomeningocele at home. METHOD: Descriptive, exploratory, qualitative research. Data were collected with 16 mothers of children and adolescents with myelomeningocele by means of interviews and observation from May to August 2015, in municipalities of Minas Gerais, Brazil. The data were submitted to qualitative analysis. RESULTS: The daily care routine of the mothers at home revealed two empirical categories: a) the impact of the birth on naturalization of care, and (b) changes in the routine of the mothers and women. The daily lives of these mothers is marked by caring that restricts their possibilities and activities to within the walls of their homes, although the mothers desire the independence of their children. CONCLUSION: The emotional, economic, and social transformations, and the difficulties of these mothers to ensure the continuity of care highlight the importance of inclusive public policies, and the importance of health workers in the rehabilitation of children and adolescents with myelomeningocele and their families.

A combined surveillance program and quality register improves management of childhood disability.

PURPOSE: To describe a concept for prevention of secondary conditions in individuals with chronic neuromuscular disabilities by using two Swedish developed follow-up-programmes for cerebral palsy (CP; CPUP) and myelomeningocele (MMC; MMCUP) respectively as examples. METHOD: This paper describes and outlines the rationale, development and implementation of CPUP and MMCUP. RESULTS: Both programmes are multidisciplinary longitudinal follow-up programmes that simultaneously serve as national registries. The programmes are population-based and set in Swedish habilitation clinics. Most children (95%) born 2000 or later with CP are enrolled in CPUP and the recruitment of adults is underway. CPUP has also been implemented in Norway, Denmark, Iceland, Scotland and parts of Australia. In MMCUP, almost all children with MMC born 2007 or later participate and individuals of all ages are now invited. The registries provide epidemiological profiles associated with CP and MMC and platforms for population-based research and quality of care improvement. CONCLUSIONS: Through multidisciplinary follow-up and early detection of emerging complications individuals with CP or MMC can receive less complex and more effective interventions than if treatment is implemented at a later stage. Possibilities and challenges to design, implement and continuously run multidisciplinary secondary prevention follow-up programmes and quality registries for individuals with CP or MMC are described and discussed. Implications for rehabilitation Individuals with disabilities such as cerebral palsy or myelomeningocele are at risk of developing secondary conditions. Multidisciplinary population-based longitudinal follow-up programmes seem effective in preventing certain types of secondary conditions.

Effect of Orthotic Gait Training with Isocentric Reciprocating Gait Orthosis on Walking in Children with Myelomeningocele.

Background: Mechanical orthoses are used to assist in standing and walking after neurological injury in children with myelomeningocele (MMC). Objectives: To evaluate the influence of orthotic gait training with an isocentric reciprocating gait orthosis (IRGO) on the kinematics and temporal-spatial parameters of walking in children with MMC. Methods: Five children with MMC were fitted with an IRGO. They walked at their own comfortable cadence using the orthosis. The hip joint angle, spatial temporal parameters, and compensatory motions were measured and analyzed. Results: Significant increases in walking speed and step length were demonstrated following orthotic gait training during walking with the IRGO. The sagittal plane hip range of motion was also significantly increased; however, the vertical and horizontal compensatory motions were significantly decreased. Conclusion: This study evaluated the influence of gait training with an IRGO on the kinematics and temporal spatial parameters in MMC children. The findings showed that orthotic gait training improved hip joint range of motion, increased walking speed and step length, and decreased lateral and vertical compensatory motions during level-ground walking trials.

Walking activity during daily living in children with myelomeningocele.

PURPOSE: To quantify the walking activity of children with myelomeningocele during daily living. METHOD: Walking activity was measured using a StepWatch activity monitor over one week in 47 children with myelomeningocele (27 males; 9 years 11 months SD 2 years 7 months; 18 sacral, 9 low lumbar, 20 mid-high lumbar) and seven children with typical development (5 males; 11 years 1 month SD 1 year 11 months) in a prospective, cross-sectional study. Average total steps per day, number of steps and minutes spent at low, medium and high intensity stepping were evaluated. Groups were compared using t-tests and chi-squared tests with Bonferroni post-hoc adjustment. RESULTS: Children with sacral and low lumbar myelomeningocele exhibited no significant differences in demographic characteristics or walking performance compared to typically developing children. Children with mid-high lumbar myelomeningocele exhibited higher BMI percentile than the control group (p = 0.04) and took fewer total steps per day than all other groups (p ≤ 0.04). Children with mid-high lumbar myelomeningocele also spent significantly less time taking steps at all intensity levels, particularly medium-intensity, than the sacral and low lumbar groups (p ≤ 0.04). CONCLUSIONS: Children with sacral and low lumbar myelomeningocele had walking performance similar to typically developing children despite a common need for braces and assistive devices. Children with mid-high lumbar myelomeningocele were less active, which may lead to heightened risk for secondary health conditions in addition to those associated with myelomeningocele. Implications for Rehabilitation Obesity, muscle weakening and disuse osteoporosis are issues for those with myelomeningocele, all of which are affected by walking activity. Understanding walking activity and intensity in children and adolescents with myelomeningocele may aid in developing focused rehabilitation interventions and strategies. Real world walking activity as an objective and quantified measure has the potential to help guide therapists and surgeons to more effective treatments.

Health-related quality of life in children with myelomeningocele: a systematic review of the literature.

Myelomeningocele (MMC) is the most complex congenital birth defect compatible with life. To provide the best health care for children with MMC, clinicians and researchers have to understand health and functional status of their patients as well as factors influencing their quality of life (QOL). The objective is to review studies that assess health-related quality of life (HRQOL) and determinants of HRQOL in children with MMC. The data sources include MEDLINE, along with reference lists of relevant reviews and included articles. Study eligibility criteria are quantitative studies that assess HRQOL using validated HRQOL instrument. Participants include children aged 0-18 years with diagnosis of MMC or spina bifida. Risk of bias was assessed using the Hayden system for non-randomized studies. Narrative synthesis and tables of results of HRQOL studies were conducted. Children with MMC have decreased HRQOL compared with normative population. Physical HRQOL is the most exposed aspect of QOL, while discrepancies in psychosocial domains vary by study. Many determinants of QOL have been found, and it is still not clear which determinant can be described as the strongest predictor. Psychosocial factors are the most consistent determinants of HRQOL.Heterogeneous study sample size, study designs, determinant and outcomes measures limited synthesis of findings. The HRQOL in children with MMC is complex and mediated by a number of associated medical problems, together with a variety of psychosocial and environmental factors. Future research is needed (a) on the relation between complex medical problems, functional independence, parent's and clinician's expectation and HRQOL in children and (b) to assess the differences in HRQOL by social environment and healthcare system.

Bone Mineral Content in Infants With Myelomeningocele, With and Without Treadmill Stepping Practice.

PURPOSE: To study development of bone mineral content (BMC) in infants with myelomeningocele (MMC) who did or did not receive upright supported stepping practice (USSP) and in infants with typical development (TD). METHODS: We tested 36 infants across the ages of 1 to 18 months. Dual-energy x-ray absorptiometry was used to collect raw BMC data for the whole body, legs, and arms, which were normalized using anthropometrics. RESULTS: Whole-body BMC generally increased with age; values for infants with MMC were slightly lower than those for infants with TD. Bone mineral content for legs and arms was lower for infants with MMC than for infants with TD. Between MMC groups, the group receiving USSP showed increases in BMC with age. CONCLUSION: Bone mineralization in infants with MMC begins to lag compared with infants with TD across infancy, and daily USSP can improve BMC as early as the first year of life.

Benefits from using an ankle-foot orthosis in children with myelomeningocele / Benefícios do uso da órtese tornozelo-pé nas crianças com mielomeningocele

Myelomeningocele (MMC) or spina bifida is a defect of the neural tube in which the spinal cord, its envelopes (meninges), and vertebral arches develop abnormally in the beginning of gestation, and owing to this failure of closure there appear a series of congenital defects and associated comorbidies, impairing in several aspects the functioning of the life of children with MMC. The congenital clubfoot has been found the most common orthopaedic anomaly in patients with MMC. The ankle-foot orthosis (AFO) is an orthopaedic device commonly used by these children to minimize the sequelae caused by this anomaly. OBJECTIVE: Identify the functional benefits brought about by the use of the AFO to children with MMC, as reported by their guardians. METHOD: Descriptive, transversal study. Convenience sample consisting of 25 guardians of children with MMC who were using/had used an AFO. RESULTS: Eighty percent of the guardians have reported at least one benefit brought about by the use of the AFO, among them: improvement in foot position (68%), foot growth (40%), improvement in foot balance (32%), and balance sitting position (15%). CONCLUSION: The use of the AFO by children with MMC can provide several benefits reported by their guardians.

A mielomeningocele (MMC) ou espinha bífida é um defeito do tubo neura no qual a medula espinal, seus envoltórios (meninges) e os arcos vertebrais desenvolvem-se anormalmente no início da gestação e, como consequências, temos uma série de defeitos congênitos e comorbidades associadas prejudicando a funcionalidade em diversos aspectos da vida das crianças com MMC. O pé torto congênito foi apontado como a anomalia ortopédica mais comum nos pacientes com MMC e a órtese tornozelo-pé (OTP) é um aparelho ortopédico que pode ser usado nessas crianças para amenizar as sequelas geradas por essa anomalia. OBJETIVO: Identificar os benefícios funcionais trazidos pelo uso da OTP em crianças com MMC relata-dos pelos seus responsáveis. MÉTODO: Estudo descritivo, transversal. Amostra de conveniência composta por responsáveis de 25 crianças com MMC que usavam/usaram a OTP que responderam a uma entrevista estruturada que contemplava as variáveis estudadas. RESULTADOS: Oitenta por cento dos responsáveis relataram pelo menos um benefício causado pelo uso da OTP, dentre eles: melhorou a postura do pé (68%), o pé cresceu (40%), melhorou o equilíbrio em pé (32%), melhorou o equilíbrio sentado (15%). CONCLUSÃO: O uso da OTP em crianças com MMC pode proporcionar benefícios identificados pelos responsáveis.