Cognitive disengagement syndrome in pediatric spina bifida.

The primary aim of this study was to characterize Cognitive Disengagement Syndrome (CDS) symptomatology in youth with spina bifida (SB). One hundred and sixty-nine patients aged 5-19 years old were drawn from clinical cases seen through a multidisciplinary outpatient SB clinic at a children's hospital between 2017 and 2019. Parent-reported CDS and inattention were measured using Penny's Sluggish Cognitive Tempo Scale and the Vanderbilt ADHD Rating Scale. Self-reported internalizing symptoms were measured with the 25-item Revised Children's Anxiety and Depression Scale (RCADS-25). We replicated Penny's proposed 3-factor structure of CDS with slow, sleepy, and daydreamer components. The slow component of CDS overlapped heavily with inattention, while the sleepy and daydreamer components were distinct from inattention and internalizing symptoms. Eighteen percent (22 of 122) of the full sample met criteria for elevated CDS, and 39% (9 of 22) of those patients did not meet criteria for elevated inattention. Diagnosis of myelomeningocele and presence of a shunt were associated with greater CDS symptoms. CDS can be measured reliably in youth with SB and can be discriminated from inattention and internalizing symptoms in this population. ADHD rating scale measures fail to identify a substantial portion of the SB population with attention-related challenges. Standard screening for CDS symptoms in SB clinics may be important to help identify clinically impairing symptoms and design targeted treatment plans.

Child, Parent, and Family Adjustment for Patients Followed in a Multidisciplinary Spina Bifida Clinic.

Objectives: To characterize child, parent, and family adjustment for patients followed in a multidisciplinary spina bifida (SB) clinic. Methods: Participants were drawn from clinical cases seen through a multidisciplinary outpatient SB clinic at a children's hospital between 2017 and 2019. Participants included 209 youth under 19 years old who were diagnosed with SB and their parents. Self-reported internalizing symptoms were measured in youth in grade 3 through 12 using the 25-item Revised Children's Anxiety and Depression Scale-25 (RCADS-25). Self- and parent-reported quality of life and family functioning were obtained using the Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales and Family Impact Modules. Results: A total of 45.7% of children and adolescents reported at-risk psychosocial functioning on the PedsQL. In contrast, only 5% of patients reported clinically elevated internalizing symptoms on the RCADS. Parents' quality of life and family functioning in the study were higher than in most studies of parents of children with other chronic health conditions, children with attention deficit-hyperactivity disorder, and healthy control samples. Conclusion: Our findings indicate that children and adolescents with SB are at risk for poor health-related quality of life (HRQOL); however, poorer HRQOL may not necessarily be associated with more severe psychiatric symptoms in this population. Examining resilience factors that may help to buffer against challenges to HRQOL will be important in informing future interventions.

Factors Associated With Ambulation and Transfer Ability: A Study From the National Spina Bifida Patient Registry.

OBJECTIVES: This study used a spina bifida electronic medical record and the National Spina Bifida Patient Registry to explore the relationship between neurosurgical/orthopedic surgeries and other variables on ambulation and transfer ability over time in individuals with spina bifida. DESIGN: This study was an analysis of longitudinal data collected within the National Spina Bifida Patient Registry and spina bifida electronic medical record. Logistic regression models were used to determine which variables were associated with ambulation/transfer ability in the myelomeningocele (MMC) and non-MMC populations. RESULTS: Longitudinal data from 806 individuals were collected. In the MMC group, decreased ambulation ability was associated with higher motor levels, tethered cord releases, spine/scoliosis surgeries, hip orthopedic surgeries, and having supplemental insurance. Increased ambulatory ability was associated with lower motor levels, tibial torsion/related surgeries, ankle/foot surgeries, being female, and being non-Hispanic/Latinx. Decreased transfer ability was associated with being Hispanic/Latinx and having higher motor levels. Lower motor level and ankle/foot surgeries were associated with increased transfer ability. No significant associations were found in the non-MMC group. CONCLUSIONS: Motor level is an important predictor of ambulation and transfer ability in MMC. Surgeries distal to the knee were associated with higher levels of function; surgeries proximal to the knee were associated with lower functional levels.

Sleep Problems and Melatonin Prescription After Concussion Among Youth Athletes.

OBJECTIVES: To examine the effect of sleep disturbances on concussion symptom recovery and to examine the effect of melatonin prescription on symptom improvement among concussed adolescents with sleep problems. DESIGN: Longitudinal test-retest. SETTING: Sports medicine clinic. PARTICIPANTS: Patients aged 8 to 18 years, diagnosed with a concussion, evaluated within 14 days after injury, and evaluated again 15 to 35 days after injury. INDEPENDENT VARIABLES: We grouped patients based on whether they reported sleep disturbances within 14 days of injury. MAIN OUTCOME MEASURES: Outcome measures included symptom severity, headache severity, melatonin prescription, and the change in symptom severity between visits. RESULTS: Two hundred twenty-five patients were included: 36% who reported sleep problems (44% female; age = 14.4 ± 2.0 years; evaluated 7.3 ± 3.8 and 23.2 ± 5.4 days after injury) and 64% who did not (32% female; age = 14.6 ± 2.3 years; evaluated 7.2 ± 3.4 and 23.0 ± 5.3 days after injury). Those with sleep problems reported higher symptom severity than those without across the 2 visits (22.1 ± 14.3 vs 14.6 ± 12.5; P < 0.001). There was no significant difference in the change in symptom severity between visits among those who received [median = 9-point improvement; interquartile range (IQR) = 1-14] and did not (median = 9, IQR = 2-18) receive a melatonin prescription (P = 0.80). CONCLUSIONS: Sleep problems among pediatric patients within 2 weeks of concussion are associated with more severe symptoms. Melatonin prescription was not associated with faster symptom recovery.

Mobility guidelines for the care of people with spina bifida.

The Spina Bifida Association (SBA) is the organization that represents the needs of the population with spina bifida (SB). They are tasked with advocacy, education, optimizing care, and providing a social voice for those with spina bifida. In response to the tenet of optimizing care they were tasked with developing up to date clinical care guidelines which address health care needs for those impacted by spina bifida throughout their lifespan. This article will discuss the SB Mobility Healthcare Guidelines from the 2018 Spina Bifida Association's Fourth Edition of the Guidelines for the Care of People with Spina Bifida.

Early physical activity and clinical outcomes following pediatric sport-related concussion.

OBJECTIVE: The objective of the study was to evaluate the clinical outcomes among patients who did and did not report engaging in early physical activity (PA) following sport-related concussion. METHODS: We evaluated pediatric patients seen within 21 days of concussion. The independent variable was early PA engagement (since the injury and before initial clinical evaluation). Dependent variables included demographics, injury details, medical history, Health and Behavior Inventory (HBI) score, and balance, vestibular, and oculomotor function tests. RESULTS: We examined data from 575 pediatric patients: Sixty-nine (12%) reported engaging in early PA (mean age=14.3±2.4 years; 30% female). The no PA group (mean age=14.5±2.4 years; 35% female) had significantly longer symptom resolution times than the early PA group (median= 16 [interquartile range (IQR)=8-24] vs. 10.5 [IQR=4-17] days; p=0.02). When controlling for pre-existing headache history and time from injury-evaluation time, the early PA group demonstrated lower odds of reporting current headache (adjusted odds ratio=0.14; 95% CI=0.07, 0.26), and reported lower symptom frequency ratings than the no PA group (b=-5.58, 95% CI=-8.94, -2.22). CONCLUSIONS: Patients who did not engage in early PA had longer symptom duration, greater odds of post-injury headache, and greater symptoms at initial clinical evaluation. We cannot determine if patients engaged in early PA due to the lower symptom burden and higher functioning at the time of assessment, or if early PA positively affected outcomes. However, as early PA was associated with better post-injury outcomes, clinicians may consider supervised and structured early PA programs as a method to improve clinical outcomes following concussion. RELEVANCE FOR PATIENTS: Children and adolescents who were engaged in PA after concussion presented to a clinic with less severe symptoms and had symptoms that resolved sooner compared to those who did not engage in early PA after concussion.

Presence of Neck or Shoulder Pain Following Sport-Related Concussion Negatively Influences Recovery.

Our objective was to examine the effect of current neck or shoulder pain on concussion outcomes. Variables included symptom resolution and return-to-sport time, symptom severity, amount of school missed, and sleep disturbances. Three hundred twelve patients (37% female; median age = 15.0 years; evaluated median = 9 days postinjury) reported experiencing current neck or shoulder pain at initial evaluation, and 268 did not (31% female; median age = 14.7 years; evaluated median = 8 days postinjury). Neck or shoulder pain was associated with longer symptom resolution time (ß = 6.38, 95% confidence interval [CI] = 2.44, 10.31; P = .002), more severe symptoms (ß = 7.06, 95% CI = 4.91, 9.21; P < .001), and greater odds of missing >5 days of school (adjusted odds ratio [aOR] = 1.89, 95% CI = 1.23, 2.93; P = .004), and postinjury sleep problems (aOR = 2.20, 95% CI = 1.51, 3.21; P < .001). Experiencing neck or shoulder pain during the initial postinjury clinical evaluation was associated with worsened clinical outcomes. Clinicians may consider referral to early rehabilitation following concussion among those who report neck or shoulder pain.

Survey of U.S. adults with spina bifida.

BACKGROUND: Spina Bifida (SB) is one of the most common birth defects and causes of permanent disability in the United States (US), with approximately 3.5 cases per 10,000 live births. OBJECTIVE: To identify complications associated with SB related to skin breakdown, pain, and urinary tract infections (UTIs), and to examine socio-demographic differences related to these complications. METHODS: Exploratory cross-sectional study via online of a national US convenience sample of adults with SB. RESULTS: We collected 1485 survey responses, of which 852 had complete, useable data. Skin breakdown in one or more locations during the past year was reported by 43.1%. After controlling for socio-demographic characteristics, only mobility variables remained significant predictors of skin breakdown (assistive device use OR = 3.119, 95% CI: 1.749, 5.564; using a wheelchair OR = 6.336, 95% CI: 3.442, 11.662). Pain in past seven days was reported by 46.9%. Single respondents (OR = 0.621; 95% CI: 0.419, 0.921) and those with at least a Bachelor's degree (vs high school degree or less, OR = 0.468; 95% CI: 0.283, 0.774) were less likely, and those using assistive devices were significantly more likely (OR = 1.960; 95% CI: 1.163, 3.303), to report pain. About one-third (32.7%) reported having a UTI within the past 12 months. Notably, almost half (49.6%) of respondents did not answer this question. The presence of UTIs was not significantly related to any socio-demographic characteristics assessed. CONCLUSIONS: Adults with SB in the US live with a wide range of complications which are potentially under-monitored, with predictors of complications that require further research.

Clinical predictors of symptom resolution for children and adolescents with sport-related concussion.

OBJECTIVE: The goal of this study was to determine which variables assessed during an initial clinical evaluation for concussion are independently associated with time until symptom resolution among pediatric patients. METHODS: Data collected from a prospective clinical registry of pediatric patients with concussion were analyzed. The primary outcome variable was time from injury until symptom resolution. Predictor variables assessed within 10 days after injury included preinjury factors, Health and Behavior Inventory scores, headache severity, and balance, vestibular, and oculomotor test performances. The researchers used univariate Cox proportional models to identify potential predictors of symptom resolution time and constructed a multivariate Cox proportional hazards model in which total duration of concussion symptoms remained the outcome variable. RESULTS: The sample consisted of 351 patients (33% female, mean age 14.6 ± 2.2 years, evaluated 5.6 ± 2.6 days after concussion). Univariate Cox proportional hazards models indicated that several variables were associated with a longer duration of symptoms, including headache severity (hazard ratio [HR] 0.90 [95% CI 0.85-0.96]), headache frequency (HR 0.83 [95% CI 0.71-0.96]), confusion (HR 0.79 [95% CI 0.69-0.92]), forgetfulness (HR 0.79 [95% CI 0.68-0.92]), attention difficulties (HR 0.83 [95% CI 0.72-0.96]), trouble remembering (HR 0.84 [95% CI 0.72-0.98]), getting tired often (HR 0.86 [95% CI 0.76-0.97]), getting tired easily (HR 0.86 [95% CI 0.76-0.98]), dizziness (HR 0.86 [95% CI 0.75-0.99]), and abnormal performance on the Romberg test (HR 0.59 [95% CI 0.40-0.85]). A multivariate Cox proportional hazards model indicated that an abnormal performance on the Romberg test was independently associated with a longer duration of symptoms (HR 0.65 [95% CI 0.44-0.98]; p = 0.038). CONCLUSIONS: For children and adolescents evaluated within 10 days after receiving a concussion, abnormal performance on the Romberg test was independently associated with a longer duration of symptoms during recovery. In line with findings of other recent studies investigating predictors of symptom resolution, postural stability tests may provide useful prognostic information for sports medicine clinicians.

The role of ethnicity and culture on functional status in children with spina bifida.

PURPOSE: Spina bifida is a common cause of pediatric disability and more prevalent in the Hispanic population. Significant health disparities exist in minority populations. Culturally adapted health interventions have been attempted in conditions such as pediatric asthma with improvement. This study aims to explore the influence of ethnicity and culture with regards to functional status and care satisfaction. METHODS: Study participants were recruited from the Children's Hospital Colorado Spinal Defects Clinic. Demographics and past medical and surgical history were obtained via chart review. A questionnaire assessed ethnicity, acculturation, self-care, mobility, bowel and bladder function, and care satisfaction. RESULTS: A total of 70 subjects with spina bifida were included in the statistical analysis. There was no difference in PEDI self-care and mobility scores between ethnicities. The Hispanic group had higher urinary incontinence rates, higher percentage with bladder accidents, and lower satisfaction with bladder management. Regarding bowel function, the Hispanic group had lower satisfaction rates and a trend towards lower bowel continence. CONCLUSIONS: Further work is needed to understand the social and cultural differences between Hispanic and Non-Hispanic children and their families that impact bowel and bladder continence and care satisfaction. Once identified, culturally sensitive interventions may be implemented that can alleviate these apparent health disparities.

Sports and disability.

Participation in recreational and competitive sports at an early age has long been touted as a positive influence on growth and development, and for fostering lifelong healthy lifestyles. The benefits of an active lifestyle include not only fitness, but the promotion of a sense of inclusion and improved self-esteem. These benefits are well documented in all populations, and their importance has been summarized in the recent Healthy People 2010 guidelines. The American Academy of Pediatrics has recently produced a summary statement on the benefits of activity for disabled children. They note that children with disabilities tend to have an overall lower level of fitness and an increased level of obesity. For this population, developing a lifelong desire to be active can be a simple means for limiting illness and much of the morbidity associated with sedentary lifestyles often associated with disability. For disabled youth, participation in disabled sports programs available nationally and internationally can be an effective means to promote such precepts. The goal of this focused review is to improve the learner's knowledge of the positive impact that active lifestyles can have on overall health in the disabled youth population and, as a result, modify their practice by incorporating recreational and competitive sport activities as part of improving overall patient care.

Pediatric spinal cord tumors and masses.

BACKGROUND/OBJECTIVE: Spinal cord tumors are a relatively rare diagnosis, accounting for 1% to 10% of all pediatric central nervous system tumors. Understanding the etiology and clinical outcomes of these tumors is therefore very important. This study presents detailed information regarding clinical presentation, histological findings, outcomes, functional assessment, and management of a series of patients with this diagnosis. METHOD: Retrospective, descriptive study. SUBJECTS: Thirty-five children with a final diagnosis of spinal cord tumor or mass, excluding dysraphism. RESULTS: Neurodevelopmental tumors (dermoid tumors, epidermoid tumors, and teratomas) were the most common tumor type (31%), followed by astrocytomas (29%) and neuroblastomas (14%). Other types included schwannomas, meningiomas, giant cell tumors, extradural cystic masses, leukemic-related masses, and masses related to neurofibromatosis. Mean age at diagnosis was 6.6 years (SD = 5.5 y) and did not vary significantly by tumor type except for children with neuroblastoma (mean = 0.4 y, SD = 0.5 y). More boys (57%) were identified in the series than girls (43%); however, there was no association between tumor type and sex. Presenting complaints of pain were noted in 57% and were localized to the back, neck, or extremities. Extremity weakness was reported as an initial presenting symptom in 46%. Three children had scoliosis as a presenting issue and 14 had gait abnormalities. Regardless of treatment modality, mobility was retained in 83% of children with or without gait aids. Neurogenic bowel and/or bladder were present in 23% of the population. CONCLUSIONS: This study corroborates other studies indicating that intramedullary tumors are the predominant form of pediatric spinal cord tumor. This population, however, presented with an unusually large number of developmental tumors, contrary to several published studies. The disparity may be the result of this institution acting as a regional referral center, thus increasing the number of this type of patient. The population is too small to make any other conjecture. The predominance of astrocytomas and neuroblastomas among those patients with poor outcomes and the prevalence of developmental tumors suggest the need for broader investigation. Although, in general, spinal cord tumors are relatively rare, this preliminary study supports the need to further evaluate associations between tumor type, presenting symptoms, treatment, and functional outcome in children with spinal cord tumors.

Pediatric sport-related concussion: a review of the clinical management of an oft-neglected population.

Athletic concussion is a growing focus of attention for pediatricians. Although numerous literature reviews and clinical guidelines are now available pertaining to athletic concussion, few have focused on the pediatric athlete in particular. Sport-related concussions occur relatively frequently in children and adolescents, and primary health care providers are often responsible for coordinating clinical management. Here we summarize the scientific literature pertinent to the care of young athletes. We examine how concussion affects younger and older athletes differently at biomechanical, pathophysiological, neurobehavioral, and contextual levels. We also discuss important issues in clinical management, including preparticipation assessment, concussion evaluation and recovery tracking, and when and how to return pediatric athletes to play sports. We also briefly cover non-sport-related interventions (eg, school support). With proper management, most children and adolescents sustaining a sport-related concussion can be expected to recover fully.

Botulinum toxin type a in the treatment of children with congenital muscular torticollis.

This is a retrospective case series describing the use of botulinum toxin type A in the treatment of children with congenital muscular torticollis who fail to progress with conservative management. A total of 27 children with congenital muscular torticollis, 6-18 mos of age, received 30 botulinum toxin type A injections into their sternocleidomastoid or upper trapezius muscle, or both, at a pediatric tertiary care center between 1995 and 2001. Three children received repeat injections. Twenty of 27 children (74%) had improved cervical rotation or head tilt after the injections, and 2 of 27 (7%) experienced transient adverse events, specifically, mild dysphagia and neck weakness. This series suggests that botulinum toxin type A may be a safe and effective treatment option for children with congenital muscular torticollis who are unresponsive to a traditional regimen of physical therapy and a home program. A prospective, randomized controlled trial is necessary to definitively assess the role of botulinum toxin type A in this population.

A survey of chronic pain in the pediatric spinal cord injury population.

BACKGROUND/OBJECTIVE: Although several reports characterize pain associated with adult-onset spinal cord injury (SCI), little is known about the incidence and etiology of pain associated with pediatric-onset SCI. The purpose of this study was to assess the incidence and type of chronic pain in the pediatric SCI population and to evaluate the resulting impact on activities of daily living (ADLs). METHODS: Patients attending clinic in a pediatric tertiary care facility in a major metropolitan area having sustained an SCI during childhood were asked to participate. Participants were assessed using the Adolescent Pediatric Pain Tool and the Lansky Play Performance Scale, 2 standardized assessments of pain and function, respectively. In addition, a questionnaire designed to characterize the population and the types of pain reported was administered. RESULTS: Thirty-one participants were enrolled with an age range at the time of injury from 5 months to 18 years. Of this population, 65% reported chronic pain. The pain reports were classified as either nocioceptive (48%) or neuropathic (19%). Interference with ADLs and play was present in only 1 participant. CONCLUSION: Based on these pilot data, pain associated with a pediatric-onset SCI is common. The incidence of pain in this study approached the reported incidence of chronic pain in adult-onset SCI. In the present population, nocioceptive pain was more common than was neuropathic pain. These data suggest that although common, chronic pain associated with childhood SCI has a significantly smaller impact on daily activities than that reported in the literature for adult-onset SCI.

Aging with a disability.

UNLABELLED: This focused review highlights important issues in the care of persons who are aging with a disability. It is part of the study guide on geriatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation. This article specifically focuses on significant medical and rehabilitation issues pertinent to persons with cerebral palsy, spina bifida, postpoliomyelitis syndrome, and selected other neurologic and neuromuscular diseases. In addition to normal physiologic aging, people with these conditions often experience secondary complications and accelerated impairments because of aging itself. These complications are described, and monitoring strategies and treatment are recommended. OVERALL ARTICLE OBJECTIVE: To summarize issues in the care of persons aging with a disability.

Exercise and sports for children who have disabilities.

This article focuses on the exercise needs of children who have disabilities, how these needs differ from able-bodied children, and what medical concerns are relevant for a given disability. The information presented also discusses some preventative options related to individual sports and a discussion of various organized recreational and competitive opportunities available both nationally and internationally. In addition, a listing of major disabled sports organizations is provided for reference.