Application of Bioelectrical Impedance Analysis in Weight Management of Children with Spina Bifida.

BACKGROUND: Children with spina bifida (SB) face an elevated risk of obesity, which necessitates precise methods for assessing body composition to ensure effective weight management. Conventional measures like BMI are inadequate for this population because of variations in growth patterns and skeletal structure. Bioelectrical impedance analysis (BIA) is a method that offers a clearer picture of body composition, yet its use in children with SB remains underexplored. METHODS: Conducted on 57 children with SB and 28 healthy controls, with a median age of 11 years, this study evaluated anthropometrics, including BMI and BIA-derived metrics. The Hoffer's scale to assess physical activity was applied in SB children. RESULTS: Results showed that while 32% of SB patients were classified as overweight or obese based on BMI, 62% exhibited high body fat percentage via BIA. Fat-free mass, muscle and fat mass, and fat-to-muscle ratio (FMR) differed significantly compared to the reference group. Non-ambulators showed a higher median body fat mass percentage (25.9% vs. 17.8%, p = 0.01) and FMR (0.92 vs. 0.44, p = 0.003) in comparison to the community walkers. CONCLUSIONS: In SB children, BIA-measured fat mass is a better obesity indicator than BMI. Non-ambulatory, SB patients with obesity had the highest FMR values, indicating a higher risk for metabolic syndrome.

Body Composition and Energy Expenditure in Youth With Spina Bifida: Protocol for a Multisite, Cross-Sectional Study.

BACKGROUND: Obesity prevalence in youth with spina bifida is higher than in their typically developing peers. Obesity is associated with lifelong medical, psychological, and economic burdens. Successful prevention or treatment of obesity in individuals with spina bifida is compromised by (1) the lack of valid and reliable methods to identify body fat in a clinical setting and (2) limited data on energy expenditure that are necessary to provide daily caloric recommendations. OBJECTIVE: The objectives of this study will be to develop 2 algorithms for use in youth with spina bifida in a clinical setting, one to model body fat and one to predict total daily energy expenditure. In addition, physical activity and dietary intake will be described for the sample. METHODS: This multisite, prospective, national clinical study will enroll 232 youth with myelomeningocele aged 5 to 18 years (stratified by age and mobility). Participants will be enrolled for 1 week. Data obtained include 4 measures of body composition, up to 5 height measures, a ramped activity protocol, and a nutrition and physical activity screener. Participants will wear an accelerometer for the week. On the final study day, 2 samples of urine or saliva, which complete the doubly labeled water protocol, will be obtained. The analysis will include descriptive statistics, Bland-Altman plots, concordance correlation, and regression analysis. RESULTS: The study received extramural federal funding in July 2019. Data collection was initiated in March 2020. As of April 2024, a total of 143 (female participants: n=76, 53.1%; male participants: n=67, 46.9%) out of 232 participants have been enrolled. Data collection is expected to continue throughout 2024. A no-cost extension until November 2025 will be requested for data analysis and dissemination of findings. CONCLUSIONS: This study furthers previous pilot work that confirmed the acceptability and feasibility of obtaining alternate height, body composition, and energy expenditure measures. The findings from this study will enhance screening, prevention, and treatment of abnormal weight status by facilitating the accurate identification of youths' weight status category and recommendations of daily caloric needs for this population that is at higher risk of obesity. Furthermore, the findings have the potential to impact outcomes for youth diagnosed with disabilities other than spina bifida who experience similar challenges related to alterations in body composition or fat distribution or measurement challenges secondary to mobility issues or musculoskeletal problems. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/52779.

Bladder height to width ratio as a surrogate marker for non-physiological storage pressures in children with spinal dysraphism.

INTRODUCTION: Spinal dysraphism is the most frequent cause of neurogenic bladder. Urodynamic study (UDS) is an important component of the follow-up of a child with neurogenic bladder. However, it suffers from a lack of widespread availability and is further hampered by technical difficulties and difficulty in its interpretation in children. A neurogenic bladder often appears vertically elongated; only limited and sparse literature is available regarding objectively defining the bladder shape and the urodynamic parameters in the cohort. OBJECTIVES: This study aimed to investigate the usefulness of the bladder's height-to-width ratio (HWR) on cystogram as a screening tool for identifying "non-physiological" bladder pressures in children with spinal dysraphism. A prospective study was undertaken to evaluate children operated for spinal dysraphism. Cystogram, ultrasonography and UDS evaluation were performed. HWR was calculated by the ratio of the maximum height to the maximum bladder width at maximum cystometric capacity (MCC), where MCC was calculated using standard Koff's formula, given by (age in years + 2) *30 ml in children more than one year and weight *7 ml for infants. The children were categorised into groups based on maximum detrusor pressure (MDP) into two groups (MDP ≥ 30 cmH2O and MDP < 30 cmH2O). A receiver-operative characteristic curve was constructed to analyse the sensitivity and specificity of HWR in predicting the MDP. RESULTS: A total of 53 children, operated for spinal dysraphism, met the study criteria during the study period, from March 2021 to September 2022. The median age of children was 4 years (IQR-3-5.5 years). The HWR ratio was compared between the two groups and was significantly higher for the non-physiological pressure bladders than for physiological pressure bladders (mean of 1.55 vs 1.26, p = 0.001). On evaluating the sensitivity and specificity of HWR for discerning children with non-physiological bladder pressures were 87.5% and 48.28%, respectively. The area under the curve (AUC) was 0.781, with a cut-off value of 1.3. DISCUSSION: We attempted to evaluate the HWR based on bladder shape objectively. We demonstrated a moderate correlation between the bladder shape and the bladder pressures. An HWR of 1.3 or higher could be significant for identifying a non-physiological bladder storage pressure. CONCLUSION: The height to width ratio of the bladder on cystogram is a useful tool as a surrogate marker for non-physiological storage pressures in bladders of children with spinal dysraphism.

A 31-year follow-up of those with occult spinal dysraphism, who underwent spinal cord untethering: Lower urinary tract function, skin stigmata, fertility, and work participation.

OBJECTIVES: This study evaluated patients with occult spinal dysraphism who underwent spinal cord untethering. METHODS: Twenty-four patients who visited us between 1983 and 2000 were followed-up for a mean duration of 31 years. We studied their lower urinary tract function, skin stigmata, fertility, and work participation. RESULTS: Questionnaires sent in 2022 revealed that 5 patients had normal voiding (Group A) and 19 patients had abnormal voiding (Group B). Groups A and B underwent spinal cord untethering at a mean age of 5.7 and 13.0 years, respectively, showing a significant statistical difference (p = 0.036). After spinal cord untethering, the number of patients with detrusor normoactivity increased from 0 to 5, i.e., 3 of 6 with detrusor overactivity (50%), 1 of 2 not examined, and 1 of 5 not known. Patients with detrusor underactivity also increased from 11 to 19. Severity of incontinence in the International Consultation on Incontinence Questionnaire-Short Form resulted in a mean value of 2.4 in Group A, which was significantly superior to the mean value of 9.1 in Group B (p = 0.004). Fourteen patients (58.3%) were married and had 21 healthy children. A majority of patients have had full-time jobs. A variety of skin stigmata were present in the lumbosacral region, and changes in vesico-urethral configurations were observed during a video-urodynamic study. CONCLUSIONS: Our study identified that the early timing of spinal cord untethering performed in neonates or infants and detrusor overactivity prior to untethering surgery are important factors in achieving normal bladder function.

Pittsburgh Impairment Testing Tool for Spina Bifida Can Predict Ambulation and Transfer Ability in Adults With Spina Bifida.

OBJECTIVE: The aim of this study was to evaluate the predictive accuracy of the Pittsburgh Impairment Testing Tool. It was hypothesized that the Pittsburgh Impairment Testing Tool would have a good overall accuracy (≥80%) for predicting both ambulation and transfer ability and that overall accuracy of the Pittsburgh Impairment Testing Tool would be higher than that of other scales. DESIGN: A retrospective chart review was used to classify 409 adults with spina bifida according to seven neurological scales. A Naïve Bayes classifier was used to obtain accuracy estimates for predicting both ambulation and transfer ability as a function of each scale. RESULTS: The Pittsburgh Impairment Testing Tool was the only scale demonstrating >80% overall accuracy for predicting both ambulation and transfer ability. While several scales demonstrated ≥80% overall accuracy in predicting transfer ability, none were useful in predicting inability to transfer. Inability to transfer was difficult for all tools to predict. CONCLUSIONS: The Pittsburgh Impairment Testing Tool demonstrated good overall accuracy for predicting both ambulation and transfer ability. Sensory and anatomic levels were less useful than motor level in predicting functional ability.

Fetal cardiovascular changes during open and fetoscopic in-utero spina bifida closure.

OBJECTIVE: Fetoscopic closure of spina bifida using heated and humidified carbon dioxide gas (hhCO2) has been associated with lower maternal morbidity compared with open closure. Fetal cardiovascular changes during these surgical interventions are poorly defined. Our objective was to compare fetal bradycardia (defined as fetal heart rate (FHR) < 110 bpm for 10 min) and changes in umbilical artery (UA) Doppler parameters during open vs fetoscopic closure. METHODS: This was a prospective cohort study of 22 open and 46 fetoscopic consecutive in-utero closures conducted between 2019 and 2023. Both cohorts had similar preoperative counseling and clinical management. FHR and UA Doppler velocimetry were obtained systematically during preoperative assessment, every 5 min during the intraoperative period, and during the postoperative assessment. FHR, UA pulsatility index (PI) and UA end-diastolic flow (EDF) were segmented into hourly periods during surgery, and the lowest values were averaged for analysis. Umbilical vein maximum velocity was measured in the fetoscopic cohort. At each timepoint at which FHR was recorded, maternal heart rate and systolic and diastolic blood pressure were measured. RESULTS: Fetal bradycardia occurred in 4/22 (18.2%) cases of open closure and 21/46 (45.7%) cases of fetoscopic closure (P = 0.03). FHR decreased gradually in both cohorts after administration of general anesthesia and decreased further during surgery. FHR was significantly lower during hour 2 of surgery in the fetoscopic-repair cohort compared with the open-repair cohort. The change in FHR from baseline in the final stage of fetal surgery was significantly more pronounced in the fetoscopic-repair cohort compared with the open-repair cohort (mean, -32.4 (95% CI, -35.7 to -29.1) bpm vs -23.5 (95% CI, -28.1 to -18.8) bpm; P = 0.002). Abnormal UA-EDF (defined as absent or reversed EDF) occurred in 3/22 (13.6%) cases in the open-repair cohort and 23/46 (50.0%) cases in the fetoscopic-repair cohort (P = 0.004). There were no differences in UA-EDF or UA-PI between closure techniques at the individual stages of assessment. CONCLUSIONS: We observed a decrease in FHR and abnormalities in UA Doppler parameters during both open and fetoscopic spina bifida closure. Fetal bradycardia was more prominent during fetoscopic closure following hhCO2 insufflation, but FHR recovered after cessation of hhCO2. Changes in FHR and UA Doppler parameters during in-utero spina bifida closure were transient, no cases required emergency delivery and no fetoscopic closure was converted to open closure. These observations should inform algorithms for the perioperative management of fetal bradycardia associated with in-utero spina bifida closure. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Association between ambulatory skills and diffusion tensor imaging of corpus callosal white matter in infants with spina bifida.

OBJECTIVES: To assess brain white matter using diffusion tensor imaging (DTI) at 1 year of age in infants diagnosed with open neural tube defect (ONTD) and explore the association of DTI parameters with ambulatory skills at 30 months of age. METHODS: Magnetic resonance imaging (MRI) was performed at an average of 12 months of age and included an echo planar axial DTI sequence with diffusion gradients along 20 non-collinear directions. TORTOISE software was used to correct DTI raw data for motion artifacts, and DtiStudio, DiffeoMap and RoiEditor were used for further postprocessing. DTI data were analyzed in terms of fractional anisotropy (FA), trace, radial diffusivity and axial diffusivity. These parameters reflect the integrity and maturation of white-matter motor pathways. At 30 months of age, ambulation status was evaluated by a developmental pediatrician, and infants were classified as ambulatory if they were able to walk independently with or without orthoses or as non-ambulatory if they could not. Linear mixed-effects method was used to examine the association between study outcomes and study group. Possible confounders were sought, and analyses were adjusted for age at MRI scan and ventricular size by including them in the regression model as covariates. RESULTS: Twenty patients with ONTD were included in this study, including three cases that underwent postnatal repair and 17 cases that underwent prenatal repair. There were five ambulatory and 15 non-ambulatory infants evaluated at a mean age of 31.5 ± 5.7 months. MRI was performed at 50.3 (2-132.4) weeks postpartum. When DTI analysis results were compared between ambulatory and non-ambulatory infants, significant differences were observed in the corpus callosum (CC). Compared with non-ambulatory infants, ambulatory infants had increased FA in the splenium (0.62 (0.48-0.75) vs 0.41 (0.34-0.49); P = 0.01, adjusted P = 0.02), genu (0.64 (0.47-0.80) vs 0.47 (0.35-0.61); P = 0.03, adjusted P = 0.004) and body (0.55 (0.45-0.65) vs 0.40 (0.35-0.46), P = 0.01, adjusted P = 0.01). Reduced trace was observed in the CC of ambulatory children at the level of the splenium (0.0027 (0.0018-0.0037) vs 0.0039 (0.0034-0.0044) mm2 /s; P = 0.04, adjusted P = 0.03) and genu (0.0029 (0.0020-0.0038) vs 0.0039 (0.0033-0.0045) mm2 /s; P = 0.04, adjusted P = 0.01). In addition, radial diffusivity was reduced in the CC of the ambulatory children at the level of the splenium (0.00057 (0.00025-0.00089) vs 0.0010 (0.00084-0.00120) mm2 /s; P = 0.02, adjusted P = 0.02) and the genu (0.00058 (0.00028-0.00088) vs 0.0010 (0.00085-0.00118) mm2 /s; P = 0.02, adjusted P = 0.02). There were no differences in axial diffusivity between ambulatory and non-ambulatory children. CONCLUSION: This study demonstrates a significant association between white matter integrity of connecting fibers of the corpus callosum, as assessed by DTI, and ambulatory skills at 30 months of age in infants with ONTD. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Early childhood neurodevelopmental outcome after open prenatal spina bifida aperta repair.

AIM: To investigate neurodevelopmental outcome of children with open prenatal spina bifida aperta (SBA) repair. METHOD: Prenatal SBA repair was performed in 130 fetuses at the Zurich Center between 2010 and 2019. Seventy-seven children underwent 1 year assessment with the Griffiths Mental Developmental Scales (Griffiths) and 65 with the Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) at 2 years. Anatomical and functional level and ambulation status were assessed. Descriptive statistics and multiple linear regression analyses for risk factors were performed. RESULTS: The Bayley-III cognition composite score in children with prenatal SBA repair was within normal limits but lower compared to population norms (mean=95.15, SD=14.683 vs norm=100, SD=15, p=0.01). Fine motor development (mean=9.58, SD=2.744, p=0.227) was typical while gross motor development was lower than the norm (mean=3.02, SD=2.758 vs norm=10, SD=3, p<0.001). Griffiths developmental quotient subscales correlated significantly with corresponding Bayley-III scores (all p<0.001, r=0.519-0.594). At 2 years, 50.8% could walk. INTERPRETATION: Children with non-trial open prenatal SBA repair show favourable cognitive outcome in the low-average range at 1 and 2 years of age. While gross motor function remained delayed, fine motor function was age appropriate. The correlation between Griffiths and Bayley-III allows a prediction about neurodevelopmental outcome at the age of 1 year. What this paper adds Children with non-trial open prenatal spina bifida repair show favourable cognitive outcome. Gross motor function remains impaired, while fine motor function is age appropriate. At 2 years of age, 50.8% of children were walking. Neurodevelopmental testing correlated between 1 (Griffiths Mental Developmental Scales) and 2 (Bayley Scales of Infant and Toddler Development, Third Edition) years.

Cardiac Autonomic Modulation of Heart Rate Recovery in Children with Spina Bifida.

We aimed to analyse cardiac autonomic control by assessing the post-exercise heart rate recovery (HRR) and physical fitness in children and adolescents with spina bifida (SB), compared to participants with typical development. A total of 124 participants, 42 with spina bifida (SB group) and 82 typical developmental controls (CO group) performed the arm cranking exercise test with a gas analysis system. HRR was determined at the first (HRR_1) and second (HRR_2) minute at recovery phase. Data are shown as [mean difference (95% CI)]. The SB group showed reduced HR reserve [14.5 (7.1-22.0) bmp, P<0.01], slower HRR_1 [12.4 (7.4-17.5) bpm, P<0.01] and HRR_2 [16.3 (10.6-21.9) bpm; P<0.01], lower VO2peak [VO2peak relative: 7.3 (4.2-10.3) mL·min-1·kg-1, P<0.01; VO2peak absolute: 0.42 (0.30-0.54) L·min-1, P<0.01], and lower O2 pulse [2.5 (1.8-3.2) mL·bpm, P<0.01] and ventilatory responses [13.5 (8.8-18.1) L·min-1, P<0.01] than the CO group. VE/VO2 was not different between groups [-2.82 (-5.77- -0.12); P=0.06], but the VE/VCO2 [-2.59 (-4.40-0.78); P<0.01] and the values of the anaerobic threshold corrected by body mass [-3.2 (-5.8- -0.6) mL·min-1·kg-1, P=0.01] were higher in the SB group than in the CO group. We concluded that children and adolescents with SB have reduced physical fitness and a slower HRR response after maximal effort.

Sexual Function and Dysfunction in Individuals with Spina Bifida: A Systematic Review.

OBJECTIVE: To conduct a systematic review of self-reported experiences of sexual function and dysfunction in individuals with spina bifida (SB). MATERIALS AND METHODS: Medline, Embase, and Web of Science were systematically searched. Studies included contained self-reported data from SB patients on one or more of the following sexual function domains: Genital sensitivity, orgasm, erectile function, ejaculation, lubrication, and/or dyspareunia. Two authors independently assessed eligibility, extracted data, and cross-checked results, with disagreements resolved by consensus. Studies included contained self-reported data from SB patients on one or more of the following sexual function domains: Genital sensitivity, orgasm, erectile function, ejaculation, lubrication, and/or dyspareunia. RESULTS: Systematic search yielded 23 studies representing 1441 patients (816 males, 625 females). Eight utilized questionnaires validated in non-SB adults; the remainder used semi-structured interviews and non-validated instruments. Eleven assessed dysfunctions in both sexes, 10 in males, and 2 in females. Erectile function and orgasm were the most commonly assessed outcomes in males and females respectively. 12%-88% of males experienced erectile dysfunction; a majority (51%-90%) reported normal ejaculatory function. Many females were unable to experience orgasm (28%-63%). CONCLUSION: Males with SB report significant erectile and ejaculatory dysfunction. Both sexes report impaired orgasms and genital sensitivity. SB-specific instruments assessing sexual dysfunction are needed in order to improve multidisciplinary care for this population.

Let's Talk About Sex: Special Considerations in Reproductive Care and Sexual Education in Young Women With Spina Bifida.

Medical advances in the last several decades have allowed an increasing number of children with spina bifida to reach adolescence and adulthood. As they reach puberty, girls with spina bifida face unique social and health challenges to their sexuality, such as orthopedic restrictions, continence, and pelvic floor disorders. Recent research efforts have focused on better understanding female sexual desires, dysfunctions, and activity and the role of the physician in educating this vulnerable population. This article aims to summarize current literature on sexual function, knowledge gaps, and the importance of tailored sexual education from providers in women with spina bifida.

Gait Function in Adults Aged 50 Years and Older With Spina Bifida.

OBJECTIVE: To study gait function among individuals with spina bifida (SB) aged 50 years or older. DESIGN: A cross-sectional study conducted in 2017. SETTING: Home-dwelling participants from all regions in Norway. PARTICIPANTS: Individuals between the ages of 51 and 76 years (N=26; 16 women) categorized as independent walkers (n=9), walkers with aids (n=10) and nonwalkers (n=7). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Questionnaire, pain assessment, anthropometry, Falls Efficacy Scale International (FES-I), objective gait analysis, 6-minute walk test (6MWT), and timed Up and Go (TUG). RESULTS: Walking speed correlated with SB severity (ρ=-.59; P=.008). Individuals who walked slower than 0.81 m/s had a higher body mass index (BMI) than those who walked faster (P=.008). Independent walkers walked slower than healthy age-matched walkers (P=.046); spatiotemporal variables showed that this was owing to shorter steps rather than cadence. The mean TUG was 10.6±2.6 seconds in independent walkers and 20.2±6.5 in walkers with aids (P<.01). The mean 6MWT was 504±126 meters in independent walkers and 316±88 in walkers with aids (P<.01). The mean pain intensity (numeric rating scale) was 4.9±2.2 in independent walkers and 4.2±1.6 in walkers with aids, but the difference was not statistically significant. FES-I was significantly lower among independent walkers (mean, 23.6±3.9) than walkers with aids (mean, 31.4±10.0) (P=.042). CONCLUSIONS: Participants commonly experienced an early onset deterioration in gait function, and walking speed was influenced by SB severity and BMI. This highlights the importance of early monitoring and weight management during follow-up for SB.

Variability in Kidney Function Estimates in Emerging Adults With Spina Bifida: Implications for Transitioning From Pediatric to Adult Care.

OBJECTIVE: To examine the variability of estimated glomerular filtration rate (eGFR) in emerging adults with spina bifida (SB) by comparing multiple equations across the transitional age period, hypothesizing that creatinine (Cr)-based equations show greater variability than cystatin-C (CysC)- or combination-based equations. METHODS: A retrospective cohort study was performed from 2012 to 2017 at a multidisciplinary SB clinic. Emerging adults were defined as patients ages 18-28 years old. Four pediatric, 3 adult, and 3 averaged eGFR equations were considered. Cross-sectional variability in eGFR data was assessed using coefficients of variation, chronic kidney disease (CKD) stage classification, and pairwise percent relative difference in eGFR between analogous pediatric and adult equations based on included lab values. Longitudinal changes in eGFR over time were compared across equations using a covariance pattern model accounting for repeated measures. RESULTS: Seventy-five emerging adults with SB (median age 21.8 years; 55% female; 83% with myelomeningocele) were included in cross-sectional analyses. Adult equations gave higher median eGFRs by 22%-27% and generally milder CKD stage classification than analogous pediatric equations. In longitudinal analyses (median follow-up of 22 months), all equations conferred negative eGFR changes over time (range -1.9 to -4.3 mL/min/1.73m2 per year) that were not significantly different. CONCLUSION: In emerging adults with SB, adult equations demonstrated higher median eGFRs by 22%-27% compared to analogous pediatric equations, even with Cystatin-C, and generally downstaged CKD stage classification. The same eGFR equation should be used for serial kidney function monitoring in emerging adults with SB who transition care from pediatric to adult services.

Physical performance outcome measures used in exercise interventions for adults with childhood-onset disabilities: A scoping review.

BACKGROUND: People with childhood-onset disabilities face unique physical and social challenges in adulthood. Exercise interventions may improve physical performance in children, but there is a lack of research on adults. OBJECTIVE: To describe studies that investigate exercise interventions and to evaluate the quality of physical performance outcome measures for adults with childhood-onset disabilities. METHODS: Eligible studies reported on exercise interventions for adults (ages 16+) with cerebral palsy, spina bifida, or acquired brain injuries. Only randomized controlled trials published in English from 2008 to 2019 were included. MEDLINE, CINAHL, PEDro, EMBASE, and Cochrane Central Register of Controlled Trials were searched. Two reviewers independently screened studies and abstracted data. RESULTS: This scoping review included 4 trials reporting on cerebral palsy only. Three strength training programs found significant improvements in gait, and one mixed training program found significant improvements in strength and fitness. Only two outcome measures used are valid/reliable for adults (6 Minute Walk Test and Borg-20 Grades). CONCLUSION: Certain interventions may improve physical performance, but there is a lack of research on appropriate exercise interventions and physical performance outcome measures for adults with childhood-onset disabilities. Different exercise interventions should be investigated using larger sample sizes and outcome measures should be standardized.

Integument: Guidelines for the care of people with spina bifida.

PURPOSE: Skin-related issues have a significant impact on health, activities of daily living, and quality of life among people with spina bifida. Data presented by select clinics that participate in the National Spina Bifida Patient Registry reported that 26% of individuals had a history of pressure injuries with 19% having had one in the past year. The spina bifida community lack direct guidelines on prevention of these and other skin related issues. The Integument (skin) Guidelines focus on prevention, not treatment, of existing problems. METHODS: Using a consensus building methodology, the guidelines were written by experts in spina bifida and wound care. RESULTS: The guidelines include age-grouped, evidence-based guidelines written in the context of an understanding of the whole person. They are presented in table format according to the age of the person with spina bifida. CONCLUSION: These guidelines present a standardized approach to prevention of skin-related issues in spina bifida. Discovering what results in successful minimization of skin-related issues with testing of technology or prevention strategies is the next step in protecting this vulnerable population.

Impact of COVID-19 outbreak on mental health and perceived strain among caregivers tending children with special needs.

BACKGROUND: While COVID-19 outbreak has had adverse psychological effects in children with special needs, the mental state and burden on their caregivers during this pandemic has yet to be reported. AIMS: The objectives of this study were to describe the mental health status and the change in perceived strain among caregivers during the COVID-19 outbreak. METHODS AND PROCEDURES: Two hundred sixty four caregivers completed an online survey that assessed demographics, use and perspective on tele-rehabilitation, homecare therapy, caregiver's strain and mental health. OUTCOMES AND RESULTS: The prevalence of depression, anxiety and stress symptoms were found to be 62.5 %, 20.5 % and 36.4 % respectively. A significant difference in caregiver strain (p <  0.001, effect size = 0.93) was observed during the outbreak compared to levels pre-outbreak (pre-outbreak strain was measured retrospectively). Caregivers not using tele-rehabilitation along with a perception of it being a poor medium for rehabilitation were at greater risks for poor mental health whereas a negative perception on homecare therapy were strongly associated with higher psychological symptoms and strain. CONCLUSIONS AND IMPLICATIONS: This study identified a high prevalence of depression and significant change in strain displayed by caregivers during the COVID-19 outbreak. We identified several factors associated with poor mental health and perceived strain that can be used to help safeguard caregivers.

Minimal Access vs. Open Spine Surgery in Patients With Metastatic Spinal Cord Compression - A One-Center Randomized Controlled Trial.

BACKGROUND/AIM: We conducted a randomized controlled trial to investigate whether minimally access spine surgery (MASS) is less morbid than open surgery (OS) in patients with metastatic spinal cord compression (MSCC). PATIENTS AND METHODS: A total of 49 MSCC patients were included in the trial. The outcome measures were bleeding (L), operation time (min), re-operations and prolonged wound healing. RESULTS: The median age was 67 years (range=42-85 years) and 40% were men. The peri-operative blood loss in the MASS-group was significantly lower than that in the OS-group; 0.175L vs. 0.500L, (p=0.002). The median operation time for MASS was 142 min (range=72-203 min) vs. 103 (range=59-435 min) for OS (p=0.001). There was no significant difference between the two groups concerning revision surgery or delayed wound healing. CONCLUSION: The MASS technique in MSCC patients is associated with less blood loss, but a longer operation time when compared to the OS technique.

Cognitive challenges in persons with spina bifida: Bearing on urological dysfunctions?

AIM: To evaluate if adult persons with spina bifida (SB) who have urinary tract complications have cognitive difficulties that can be identified by neuropsychological tests. METHODS: All individuals with SB ≥ 18 years of age registered at a regional outpatient clinic (n = 219) were invited, of which, 154 persons were included. Neuropsychological assessment of their cognitive status was performed with Wechsler Adult Intelligence Scale®-Fourth Edition: Coding, Block design, Arithmetic's, FAS (word generation), Rey Auditory Verbal Test for learning, and delayed recall 30 min. Bladder and bowel function were assessed with questions used by the Nordic Spinal Cord Injury Registry (NSCIR) in structured interviews, by questionnaires, and by chart reviews. RESULTS: Average neuropsychological test results for this SB population was shown to be approximately 1 SD under the median for the general population. The Coding test showed significantly lower test results as compared with the whole SB group in persons with urinary tract complications, especially urinary tract infections, reduced kidney function, dependent emptying of the bladder, and the bowel and accidental bowel leakage. The Arithmetic's test showed a significant difference between subgroups in all parameters except reduced kidney function whereas the other neuropsychological tests were significantly correlated with some but not all urological parameters. CONCLUSION: We propose neuropsychological testing with primarily two tests to find those persons with SB who, due to cognitive challenges, might need extra support to minimize urological complications.

Determinants of physical activity in young wheelchair-user with spina bifida.

OBJECTIVE: To explore associations between physical activity and peak oxygen uptake (VO2peak), age, sex, and Hoffer classification in young wheelchair-users with spina bifida. DESIGN: Exploratory study. SUBJECTS: Fifty-three dutch children (age 5-19 years) with spina bifida who use a manual wheelchair. METHODS: For the dependent variable physical activity, data from 2 physical activity monitors were analysed: VitaMove data for 34 participants and Actiheart data for 36 participants. Time sedentary, time physically active, and time in moderate to vigorous physical activity were analysed. The Wheelchair Shuttle Test was used to measure VO2peak. Univariate and multivariate regression analyses were performed. Independent variables were VO2peak, age, sex, and Hoffer classification. RESULTS: Time sedentary and time physically active during a school day were influenced by age (ß=0.326/ß=-0.320) and Hoffer classification (ß=0.409/ß=-0.534) and during a weekend day by Hoffer classification (ß=0.617/ß=-0.428). Time in moderate to vigorous physical activity was influenced by Hoffer classification (ß=-0.527) during a school day and by age (ß=-0.600) during a weekend day. CONCLUSION: Older age and the inability to walk negatively influence physical activity. Sex and VO2peak were not associated with physical activity. These results imply that increasing cardiorespiratory fitness alone will not improve physical activity in young wheelchair-users with spina bifida.

Suppression of GABAergic transmission in the spinal dorsal horn induces pain-related behaviour in a chicken model of spina bifida.

Spina bifida aperta (SBA), one of the most common congenital malformations, causes various neurological disorders. Pain is a common complaint of patients with SBA. However, little is known about the neuropathology of SBA-related pain. Because loss of g-aminobutyric acid GABAergic neurons in the spinal cord dorsal horn is associated with pain, we hypothesised the existence of crosstalk between SBA-related pain and alterations in GABAergic transmission in the spinal cord. Therefore, we investigated the kinetics of GABAergic transmission in the spinal cord dorsal horn in a chicken model of SBA. Neonatal chicks with SBA exhibited various pain-like behaviours, such as an increased number of vocalisations with elevated intensity (loudness) and frequency (pitch), reduced mobility, difficulty with locomotion, and escape reactions. Furthermore, the chicks with SBA did not respond to standard toe-pinching, indicating disruption of the spinal cord sensorimotor networks. These behavioural observations were concomitant with loss of GABAergic transmission in the spinal cord dorsal horn. We also found apoptosis of GABAergic neurons in the superficial dorsal horn in the early neonatal period, although cellular abnormalisation and propagation of neuro-degenerative signals were evident at middle to advanced gestational stages. In conclusion, ablation of GABAergic neurons induced alterations in spinal cord neuronal networks, providing novel insights into the pathophysiology of SBA-related pain-like complications.