Open spina bifida: birth findings predict long-term outcome.

OBJECTIVES: To investigate if lifestyle in spina bifida at age 40±3 years, relates to neurological deficit in infancy or cerebrospinal fluid shunt history. DESIGN: Prospective cohort study with 100% ascertainment. SETTING: Community. PARTICIPANTS: 117 consecutive cases of open spina bifida whose backs were closed non-selectively at birth. In 2007, all 46 (39%) survivors and/or carers were surveyed by postal questionnaires and telephone interviews. RESULTS: Of the 38 children with absent sensation only below the knee (sensory level below L3), 23 (61%) survived of whom 14 (61%) were community walkers and only 5 (22%) needed daily care. But in 42 babies with absent sensation up to the umbilicus (sensory level above T11) only seven (17%) survived, none could walk and five (71%) needed daily care. Survivors with no shunt revisions were more likely to walk, live independently and drive a car. CONCLUSION: Mobility and the need for care at 40 can be predicted from the neurological deficit.

Cardiovascular disease risk factors and the relationships with physical activity, aerobic fitness, and body fat in adolescents and young adults with myelomeningocele.

OBJECTIVES: To describe cardiovascular disease (CVD) risk factors in adolescents and young adults with myelomeningocele (MMC) and to explore relationships with physical activity, aerobic fitness, and body fat. DESIGN: Cross-sectional study. SETTING: Outpatient clinic. PARTICIPANTS: Adolescents and young adults (N=31) with MMC (58% men) age 16 through 30 years; 13 were ambulatory and 18 were nonambulatory. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: We studied biologic and lifestyle-related CVD risk factors, including lipid and lipoprotein profiles, blood pressure, aerobic fitness (Vo(2)peak), body fat, daily physical activity, and smoking behavior. We considered subjects at increased CVD risk when 2 or more of the following risk factors clustered: systolic blood pressure, total serum cholesterol (TC), high-density lipoprotein cholesterol (HDL-C), and cigarette smoking. Relationships were studied using regression analyses. RESULTS: Levels of TC, low-density lipoprotein cholesterol, and triglycerides were elevated in 29%, 38%, and 3% of the participants, respectively. HDL-C was reduced in 19%. Hypertension was found in 20%, and 19% were current cigarette smokers. Based on the clustering of risk factors, 42% of the participants were at increased CVD risk: 15% of ambulatory participants and 61% of nonambulatory participants (P=.03). Adjusted for sex and ambulatory status, participants with higher aerobic fitness tended to be more likely to have no CVD risk (odds ratio=13.0; P=.07). CVD risk was not associated to physical activity and body fat. CONCLUSIONS: A large proportion of the study sample was at CVD risk, indicated by clustering of risk factors. Improving aerobic fitness in young adults with MMC may contribute in reducing CVD risk; this needs to be confirmed in future studies.

[Children with meningomyelocele become adults!]. / Barn med ryggmärgsbråck blir vuxna!

The survival for children with spina bifida has dramatically increased during the last 30 years. In Sweden today 40-50 adolescents with spina bifida are reaching adulthood each year the next 10 years. Children with spina bifida are from birth to adulthood followed by a multidisciplinary medical and paramedical team within the habilitation organisation. However, from the age of 18 this responsibility is discontinued, often with less readiness in adult medicine to meet the spina bifida adolescents and their special needs. Facing adolescence and adulthood both children and parents need a careful preparation from several points of view for the transition. It is also most important to prepare the adult medical disciplines about the special needs of this group. This process has to start early to reach successful management, including improvement in self-care.

Educational career and predictors of type of education in young adults with spina bifida.

Children with spina bifida (SB) often require special education. To date, little information is available about the educational career of these children. This study focuses on educational career and predictors of attending special education of young adults with SB, using a cross-sectional study including 178 young Dutch adults with SB aged from 16-25. The main outcome was attending regular versus special education. For searching predictive power we selected age, gender, type of SB, level of lesion, hydrocephalus (HC), number of surgical interventions, ambulation, continence and cognitive functioning. Chi-square tests and binary logistic regression were used in the data analysis. Participants with HC attended special primary education more often (59%) than participants without HC (17%). For those participants with HC, the necessity of special primary education was associated with below average intelligence (75% versus 35%), wheelchair dependence (82% versus 39%) and surgical interventions (74% versus 44%). Only half of the participants with HC followed regular secondary education, whereas for participants with SB without HC, the outcome in secondary education was similar to that of the general population (92%). Intelligence was the main predictor of attending special secondary education (odds 5.1:1), but HC (odds 4.3:1) and wheelchair dependence (odds 2.6:1) were also a significant. Other variables were not significant predictors of special secondary education.

Un caso de mielomeningocele, hipoplasia del sacro, amputación de miembro inferior por abscesos dérmicos en el que se detecta déficit de anticuerpos antipolisacáridos / A case of myelomeningocele, hypoplasia of the sacrum, amputation of lower limb due to dermal abscesses

El enfermo con mielomeningocele presenta entre otros síntomas, parálisis, tanto mayor cuanto más alto es el nivel, déficit sensitivos, deformidades en miembros inferiores, en parte producidas por el inicio de la carga, y trastornos urológicos. Describimos el caso de un paciente con mielomeningocele y múltiples infecciones dérmicas a lo largo de su crecimiento que conllevó la amputación del miembro inferior izquierdo. La frecuencia de abscesos y episodios de sepsis generalizadas, nos llevó a estudiar su sistema inmunológico, detectándose un déficit inmunológico de anticuerpos. Un tratamiento integral, tanto de los factores extrínsecos que podían producir los roces, mediante ayudas ortoprotésicas; de los factores locales mediante cirugía y de los factores intrínsecos mediante gammaglobulina, ha conseguido resolver los problemas que el paciente aquejaba. (AU)

Assessment of adherence with multiple informants in pre-adolescents with spina bifida: initial development of a multidimensional, multitask parent-report questionnaire.

Adherence to medical regimens was assessed in 67 pre-adolescents with spina bifida (8- and 9-year-olds; 37 boys, 30 girls), with mother, father, teacher, and health professional report. The Parent-Report of Medical Adherence in Spina Bifida Scale (PROMASB) was developed and includes multidimensional scales for the following tasks: catheterization, bowel care, skin care, medication, and ambulation. With few exceptions, the PROMASB has adequate psychometric properties. However, findings revealed modest to low correlations between respondents. Mothers and fathers reported significantly more noncompliance than teachers and health professionals. For the most part, all informants reported that most children were compliant across all tasks. Additional analyses based on qualitative data suggest that parents attribute compliance difficulties to motivational as well as attentional-memory factors.

[Future prospects for children with spina bifida aperta]. / Toekomstperspectieven voor kinderen met spina bifida aperta.

OBJECTIVE: To determine the prospects for independent functioning in society of children born with meningomyelocele who are receiving maximal treatment. DESIGN: Retrospective descriptive. SETTING: University Hospital Groningen, the Netherlands. METHODS: Data were collected in 1994 regarding personal care, mobility, education, living conditions and employment of 163 patients alive and born in 1966-1990. They were divided into three groups according to their age, corresponding to the primary school period (n = 47), secondary school period (n = 37) and subsequent education or employment period (n = 79). All data were related to the level of the neurological lesion. RESULTS: A higher level of the neurological deficit corresponded with a lower probability of functioning independently in society. This was mainly caused by the concomitant mental handicap that occurs frequently in children with a high neurological deficit. Nine out of 46 adult patients with a deficit higher than LIII were living independently. Eleven of these 46 patients were (or had been) capable of attending regular education, and 7 of these 11 patients had a regular job. CONCLUSION: The prospects of children born with a meningomyelocele of reaching independence are relatively poor, depending on the level of neurological deficit.