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Pediatrics Sep 2018Patients with spina bifida (SB) typically develop serious secondary conditions and undergo surgical procedures related to neurologic disorders, orthopedic abnormalities,...
BACKGROUND AND OBJECTIVES
Patients with spina bifida (SB) typically develop serious secondary conditions and undergo surgical procedures related to neurologic disorders, orthopedic abnormalities, bladder and bowel dysfunction, and skin breakdown. In this study, we describe the age distribution of common surgical procedures and health outcomes in patients with SB.
METHODS
Using serial cross-sectional data from the National Spina Bifida Patient Registry (2009-2013; = 4664), we examined surgical procedures (gastrointestinal, neurologic, orthopedic, skin, urologic, and other) and health outcomes (fecal continence, urinary continence, skin breakdown, and ambulation status) of patients with SB by age and SB type (myelomeningocele and nonmyelomeningocele).
RESULTS
All patients who were enrolled had available health outcome data, and 81.5% ( = 3801) of patients had complete surgical procedure data, which totaled 18 891 procedures across their lifetimes. Almost all procedures (91.4%) occurred among participants with myelomeningocele SB. For both types of SB, the distribution of procedures varied by age. The most frequent procedures were neurologic, with approximately half (53%) occurring in patients <1 year of age; orthopedic and urologic procedures followed in frequency but tended to occur at older ages. The health outcomes for patients with myelomeningocele SB revealed lower frequencies of positive health outcomes than those for patients with nonmyelomeningocele SB across all age groups. Overall, the rates of fecal and urinary continence and skin breakdown increased with age whereas the ability to ambulate declined with age.
CONCLUSIONS
Understanding the surgical procedures and health outcome variations by age and SB type can help clinicians and populations that are affected set expectations regarding the occurrence of these procedures and the outcomes throughout the patients' life spans.
Topics: Adolescent; Adult; Child; Child, Preschool; Cross-Sectional Studies; Female; Humans; Infant; Male; Registries; Spinal Dysraphism; Treatment Outcome; Young Adult
PubMed: 30158199
DOI: 10.1542/peds.2017-3730 -
Journal of Pediatric Rehabilitation... 2020Nutritional challenges and a lack of activity can lead to health problems across the lifespan for people with spina bifida. Children and adults with spina bifida are... (Review)
Review
Nutritional challenges and a lack of activity can lead to health problems across the lifespan for people with spina bifida. Children and adults with spina bifida are also at greater risk of being classified as overweight or obese compared to their peers without the condition. Therefore, early recognition of nutrition problems, weight management counseling, and timely referrals for evaluation and management of diet and activity can help those with spina bifida and their families achieve a healthy lifestyle. This article details the development of the Nutrition, Metabolic Syndrome and Obesity Guidelines, which are part of the 2018 Spina Bifida Association's Fourth Edition of the Guidelines for the Care of People with Spina Bifida. It discusses the identification and management of poor nutrition and prevention of obesity for children, adolescents, and adults with spina bifida and highlights areas requiring further research.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Metabolic Syndrome; Nutritional Physiological Phenomena; Obesity; Practice Guidelines as Topic; Spinal Dysraphism; Young Adult
PubMed: 33325412
DOI: 10.3233/PRM-200753 -
Journal of Rehabilitation Medicine Sep 2021Individuals with spina bifida often have cognitive impairments leading to dif-ficulties in education and daily activities. The aims of this study were to explore...
BACKGROUND AND OBJECTIVE
Individuals with spina bifida often have cognitive impairments leading to dif-ficulties in education and daily activities. The aims of this study were to explore cognitive impairments in adults with spina bifida and to consider associations between impairments, educational outcome and per-formance of daily activities, comparing individuals with and without intellectual disability.
METHODS
Data were collected on 35 adults with spina bifida via cognitive tests and Assessment of Motor and Process Skills (AMPS). Participants were divided into 3 groups: individuals without intellectual disability who completed compulsory education (NID-C); those without intellectual disability, who failed to successfully pass compulsory education (NID-F); and those with intellectual disability failed to successfully pass compulsory education (ID-F).
RESULTS
All individuals with intellectual disability failed to successfully pass compulsory education (group ID-F) and had poorer scores across almost all measures than group NID-F and significantly poorer scores than group NID-C. All except 6 individuals scored below cut-off levels for effort and safety on both AMPS motor and process scales; more significant associations were seen between the cognitive tests and the motor rather than process scale.
CONCLUSION
Cognitive impairments, irrespective of intellectual disability, impact on the performance of eve-ryday activities and on educational achievement, and thus need to be considered in assessments and inter-ventions to improve outcomes and promote independence in people with spina bifida.
Topics: Activities of Daily Living; Adult; Cognition; Humans; Neuropsychological Tests; Spinal Dysraphism
PubMed: 34448489
DOI: 10.2340/16501977-2868 -
Pain Oct 2021Chronic pain is the most commonly reported physical symptomology of cerebral palsy (CP) and spina bifida (SB) throughout the lifespan, and yet, pain is perhaps the least...
Chronic pain is the most commonly reported physical symptomology of cerebral palsy (CP) and spina bifida (SB) throughout the lifespan, and yet, pain is perhaps the least understood comorbidity in these populations. The objective of this study was to compare the prevalence and types of pain diagnosed among adults living with and without CP or SB. In this retrospective cohort study, we analyzed data from a nationwide commercial insurance claims database. Beneficiaries were included if they had an International Classification of Diseases, Ninth revision, Clinical Modification diagnosis code for CP or SB (n = 22,648). Adults without CP or SB were also included as controls (n = 931,623). Pain phenotypes (nociceptive, nociplastic, and neuropathic pain) and pain multimorbidity (≥2 conditions) were compared. We found that adults living with CP or SB had a higher prevalence of any pain disorders (55.9% vs 35.2%), nociceptive pain (44.0% vs 26.7%), nociplastic pain (26.1% vs 11.9%), neuropathic pain (9.6% vs 5.6%), and pain multimorbidity (21.1% vs 8.4%), as compared to adults without CP or SB, and differences were to a clinically meaningful extent. Adjusted odds ratios of nociceptive pain (odds ratio [OR]: 2.20; 95% confidence interval [CI]: 2.15-2.24), nociplastic pain (OR: 2.47; 95% CI: 2.41-2.53), neuropathic pain (OR: 2.71; 95% CI: 2.54-2.89), and other pain (OR: 3.92; 95% CI: 3.67-4.19) were significantly higher for adults living with CP or SB. In conclusion, adults with CP or SB have a significantly higher prevalence and odds of common peripheral, central, and neuropathic pain disorders and pain multimorbidity, as compared to adults without CP or SB.
Topics: Adult; Cerebral Palsy; Humans; Pain; Phenotype; Retrospective Studies; Spinal Dysraphism
PubMed: 34534178
DOI: 10.1097/j.pain.0000000000002240 -
Psychosocial Adjustment in Emerging Adults With and Without Spina Bifida: A 14-Year Follow-up Study.Journal of Developmental and Behavioral... Jan 2022A 14-year follow-up of youth with spina bifida (SB), as compared to a matched comparison sample without a chronic health condition, focused on psychosocial adjustment...
OBJECTIVE
A 14-year follow-up of youth with spina bifida (SB), as compared to a matched comparison sample without a chronic health condition, focused on psychosocial adjustment during emerging adulthood (ages 22-23).
METHODS
The initial cohort at time 1 consisted of 68 families with an 8-year-old or 9-year-old child with SB and a matched comparison sample of 68 typically developing children and their families. At the final assessment (time 7), participants included 56 emerging adults with SB (82% of those who participated at time 1) and 62 comparison youth (91% of those who participated at time 1) who completed a set of self-report measures.
RESULTS
Emerging adults with SB continue to lag behind across several important developmental milestones, including decision-making responsibility, involvement in social and romantic relationships, sexual intimacy, educational and vocational achievement, financial independence, and engagement in health-related behaviors. However, from a resilience perspective, these individuals also exhibited a number of strengths across the following areas: identity development, quality of social supports, and fewer problem behaviors (e.g., alcohol and other substance use). Most findings were still significant after controlling for a verbal intelligence quotient proxy.
CONCLUSION
This study highlighted vulnerabilities and strengths of individuals with SB as they enter emerging adulthood, with considerable continuity across childhood, adolescence, and emerging adulthood.
Topics: Adolescent; Adult; Child; Follow-Up Studies; Humans; Sexual Behavior; Social Behavior; Spinal Dysraphism; Substance-Related Disorders; Young Adult
PubMed: 34016827
DOI: 10.1097/DBP.0000000000000956 -
Journal of Pediatric Rehabilitation... 2020Orthopedic or musculoskeletal problems are common in individuals with spina bifida. They can affect function and mobility and, in the case of spinal deformity, affect... (Review)
Review
Orthopedic or musculoskeletal problems are common in individuals with spina bifida. They can affect function and mobility and, in the case of spinal deformity, affect pulmonary function. We discuss the current treatment guidelines developed through collaboration with the Spina Bifida Association and the Orthopedics and Mobility working group using a specific methodology previously reported [1,2]. General considerations are discussed followed by evaluation and treatment guidelines for specific age ranges. References are provided where applicable, but where data is lacking treatment guidelines fall under the umbrella of clinical consensus. This leaves "research gaps" where areas of possible future study could be considered.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Musculoskeletal Diseases; Orthopedics; Practice Guidelines as Topic; Spinal Dysraphism; Young Adult
PubMed: 33252095
DOI: 10.3233/PRM-200750 -
Pediatric and Developmental Pathology :... 2022Spina bifida (SB) is the most common neural tube defect in humans. Here, we analyzed systematically the neuropathological findings of the brain in SB cases.
INTRODUCTION
Spina bifida (SB) is the most common neural tube defect in humans. Here, we analyzed systematically the neuropathological findings of the brain in SB cases.
METHODS
79 cases with SB aperta (SBA) and 6 cases with SB occulta (SBO) autopsied at the Charité Neuropathology from 1974 to 2000 were re-evaluated retrospectively. For this, case files and spinal cord as well as brain sections were studied.
RESULTS
While no brain malformations were detected in SBO cases, 95% of SBA cases had brain malformations. Main brain anomalies identified were hydrocephalus (71%), Chiari II malformation (36%), heterotopia (34%), other cerebellar anomalies (36%), gyrification defects (33%), and ependymal denudation (29%). Hydrocephalus was observed as early as gestational week 17 and was highly associated to Chiari II and ependymal denudation. In 55% SBA was accompanied by further anomalies not primarily affecting the CNS.
CONCLUSION
We confirm using neuropathologic methods brain malformations in most SBA but none in SBO cases. In addition to our previous radiologic study, we now demonstrate the high prevalence of cerebellar malformations and cerebral heterotopias in SBA. The early detection of hydrocephalus and Chiari II malformation in fetuses raises the question whether these arise parallel rather than in strict temporal sequence.
Topics: Arnold-Chiari Malformation; Humans; Hydrocephalus; Nervous System Malformations; Retrospective Studies; Spinal Dysraphism
PubMed: 34614376
DOI: 10.1177/10935266211040500 -
Topics in Spinal Cord Injury... 2016To identify differences in the diagnosis and treatment of attention deficit/hyperactivity disorder (ADHD) between typically developing children and children with spina...
To identify differences in the diagnosis and treatment of attention deficit/hyperactivity disorder (ADHD) between typically developing children and children with spina bifida. Sixty-eight children with spina bifida and 68 demographically matched, typically developing children participated in a larger, longitudinal study. Rates of maternal, paternal, and teacher reports of attention problems, as well as rates of maternal reports of ADHD diagnosis, diagnosing provider, pharmaceutical treatment, mental health treatment, and academic accommodations were obtained at 5 time points over a period of 8 years and were compared across groups. Children with spina bifida were more likely to have an ADHD diagnosis and attention problems. Attention problems and ADHD diagnoses were first reported at earlier time points for children with spina bifida than typically developing children. Among children with ADHD or attention problems, children with spina bifida were more likely to be treated with medication, but they were just as likely to use mental health services and receive resource services at school. Children with spina bifida were diagnosed with ADHD and identified as having attention problems more frequently and at an earlier age. This finding could be due to earlier symptom development, greater parental awareness, or more contact with providers. Among those with ADHD or attention problems, stimulant medication was more likely to be prescribed to children with spina bifida, despite research that suggests it may not be as beneficial for them. Further research on the effectiveness of ADHD pharmacological treatment for children with spina bifida is recommended.
Topics: Attention; Attention Deficit Disorder with Hyperactivity; Case-Control Studies; Central Nervous System Stimulants; Child; Female; Humans; Longitudinal Studies; Male; Spinal Dysraphism
PubMed: 29339866
DOI: 10.1310/sci2204-253 -
Journal of Pediatric Rehabilitation... 2023Whether it is for collaboration on folic acid fortification or the standardization of care efforts concerning neurogenic bowel dysfunction, a global forum on neural tube...
Whether it is for collaboration on folic acid fortification or the standardization of care efforts concerning neurogenic bowel dysfunction, a global forum on neural tube defects related issues is needed. Propitiously, the 2023 Spina Bifida World Congress sponsored by the Spina Bifida Association (SBA) was a catalyst for transnational dialog in the field of spina bifida (SB) research. Concurrently, the Journal of Pediatric Rehabilitation Medicine (JPRM) provides a platform for both international research as well as numerous clinical and educational projects, such as The Lifespan Bowel Management Protocol, and social interventions taught through the American Academy of Pediatrics' Spina Bifida Transition ECHO. Through this open access issue, work by colleagues in Ethiopia, the Nordic countries, and Switzerland, as well as among other transnational populations is highlighted. The development of the Spina Bifida Global Learning Collaborative is also showcased, representing a training initiative across four continents. Correspondingly in this issue, JPRM published an update to the Transition Guidelines for the Care of People with Spina Bifida. The clinical guidelines are a product of the SBA Collaborative Care Network cooperative agreement with the National Center on Birth Defects and Developmental Disabilities in the Centers for Disease Control and Prevention. While colleagues across the globe remain committed to native, immigrant, and displaced populations of individuals affected by SB, JPRM will continue to distribute premier research in multidisciplinary care, education, and advocacy.
Topics: Pregnancy; Female; Humans; Child; United States; Prenatal Care; Longevity; Spinal Dysraphism; Neural Tube Defects; Emigrants and Immigrants
PubMed: 38160377
DOI: 10.3233/PRM-239020 -
Fetal Diagnosis and Therapy 2015
Topics: Animals; Fetal Diseases; Fetal Therapies; Humans; Prenatal Diagnosis; Spinal Dysraphism
PubMed: 25721029
DOI: 10.1159/000375329