Spina bifida at the sacral level: more than minor gait disturbances

Objective: To investigate functional outcome in two groups of children with sacral level paralysis: myelomeningocele (MMC) versus lipomyelomeningocele (LMMC). Additionally both groups were compared with each other and when possible with reference values. Design: Cross-sectional study by means of ( 1) clinical assessment, and ( 2) disability measurement. Setting: Spina bifida outpatient clinic at a university hospital in the Netherlands. Subjects: Sample of 30 children with MMC and 14 with LMMC. Mean age (SD) 6.0 (4.9) and 8.4 ( 4.9) years respectively. Main measures: Muscle strength, ambulation level, motor performance (Bayley Scales of Infant Development (BSID) and Movement Assessment Battery for Children), and the Pediatric Evaluation of Disability Inventory (PEDI). Results: The majority of patients in both groups were normal ambulant, 14/21 (67%) in MMC and 9/14 (64%) in LMMC. Ambulation was strongly associated with muscle strength of hip abductors (odds ratio (OR): 13.5, 95% confidence interval (CI) 2.5 - 73.7), and ankle dorsal-flexor muscles ( OR: 110, 95% CI 8.9 - 135.9). No significant differences were found in lesion and ambulation level. Muscle strength and motor performance were significantly lower in the MMC group than in the LMMC group ( p <0.05). PEDI scores were comparable in both groups. Most problems were noted in mobility skills and caregiver assistance in self-care, especially regarding bladder and bowel management. Conclusions: Gross motor and functional problems were seen in both groups. The MMC group showed more muscle weakness and motor problems. However, in both groups caregiver assistance was needed for a prolonged period, especially regarding bladder and bowel management. These findings need special attention, particularly in children who attend regular schools

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

Determinants of functional independence and quality of life in children with spina bifida

Objective: To investigate determinants of functional independence and study which functional abilities were determinants for 'health-related quality of life' in children with myelomeningocele. Design: Cross-sectional study by means of clinical assessment, 'disability' measurement and questionnaires. Uni- and multivariate logistic regression models were used to investigate factors that were determinants for these outcomes. Results were expressed as odds ratios ( OR) and 95% confidence intervals ( 95% CI). Setting: Outpatient spina bifida clinic at a university hospital. Subjects: One hundred and twenty-two children with myelomeningocele. Mean age 7.9; range 1 - 18 years. Main measures: Functional independence as measured by the Pediatric Evaluation of Disability Inventory (PEDI), and quality of life as measured by the Spina Bifida Health Related Quality of Life Questionnaire. Results: Lesion level below L3 ( OR 0.4, 95% CI 0.1 - 1.0), mental status of IQ >= 80 ( OR 4.2, 95% CI 1.2 - 14.9), having no contractures in lower extremities ( OR 3.4, 95% CI 1.3 - 8.8), and having normal strength of knee extensor muscles ( OR 4.1, 95% CI 1.4 - 11.5) were most strongly associated with independence in self-care. Mental status ( OR 16.1, 95% CI 2.8 - 93.9), having no contractures in lower extremities ( OR 1.5, 95% CI 1.4 - 5.3), and normal strength in knee extensors ( OR 11.0, 95% CI 1.3 - 97.0) were the most important determinants for independence in mobility. Concerning functional abilities, being independent with regard to mobility was the most important determinant for 'health-related quality of life' ( OR 5.3, 95% CI 1.6 - 17.4). Conclusions: In children with myelomeningocele, good muscle strength, mental ability and being independent in mobility appeared to be much more important for daily life function and quality of life than other medical indicators of the disorder

Cerebral ischemia and white matter edema in experimental hydrocephalus. A combined in vivo MRI and MRS study

T2 and diffusion weighted MRI, as well as 31P and 1H MRS were performed in kaolin-induced hydrocephalic rats. Extracellular white matter edema was detected in the early stages of progressive hydrocephalus. Phosphocreatine (PCr)/inorganic phosphate (Pi) ratios in hydrocephalic animals were decreased compared to controls, and lactate was detected during the acute and chronic stages of hydrocephalus. These MR spectroscopic results are indicative of a compromised energy metabolism and suggest the occurrence of cerebral ischemia in experimental hydrocephalus