Journal of Pediatric Rehabilitation Medicine - Volume 10, issue 3-4

Article Type: Other

DOI: 10.3233/PRM-170468

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 159-160, 2017

Article Type: Other

DOI: 10.3233/PRM-170470

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 161-, 2017

Article Type: Other

DOI: 10.3233/PRM-170472

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 163-, 2017

Authors: Brei, Timothy | Houtrow, Amy

Article Type: Other

DOI: 10.3233/PRM-170469

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 165-166, 2017

Authors: Castillo, Jonathan | Castillo, Heidi

Article Type: Research Article

Keywords: Global health, blue marble health, chronic non-communicable diseases, spina bifida, international healthcare

DOI: 10.3233/PRM-170471

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 167-168, 2017

Authors: Sawin, Kathleen J.

Article Type: Research Article

Abstract: Self-management has become a central concept in individual and family-centered care. However, there are differences in both definitions and theoretical approaches that guide clinical practice and research in this emerging science. Review of theoretical approaches can assist clinicians and researchers to identify the framework or theory that best fits their approach to practice or research question. The evidence on self-management in spina bifida since the 2003 research priorities were first established is discussed and the implications for theory-based future practice and research delineated. Resources for collaborative research and practice in self-management are provided.

Keywords: Self-management, theory, adherence

DOI: 10.3233/PRM-170461

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 169-176, 2017

Authors: Betz, Cecily L.

Article Type: Research Article

Abstract: PURPOSE: The purpose of this article is to provide perspectives pertaining to the importance of fostering comprehensive self-management competencies of AEA-SHCN, with attention directed to AEA with spina bifida based upon the Health Care Transition Research Consortium Health Care Transition model. This article is based upon the plenary presentation given at the Spina Bifida 3rd World Congress, entitled, Self-Management and Health Care Transition: Trials, Tribulations and Triumphs. METHOD: A historical perspective of healthcare transition initiatives and best practice guidelines is provided that have influenced the field of research and practice. Relatively scant attention has been directed …to the development and refinement of self-management competencies prior to the transfer of care and transition to adulthood. Against this backdrop of the predominant framework of care focused on the transfer of care, a discussion of our team’s experience with the provision of healthcare transition services and the complexity of addressing the self-management needs of the adolescents and emerging adults (AEA) with spina bifida served in the Movin’ On Up HCT program will be presented. RESULTS: Through our clinical and research healthcare transition experience, insights pertaining to self-management will be presented. CONCLUSION: The acquisition of self-management knowledge and skills is a complex process that extends far beyond learning the fundamental skills of daily condition management. Show more

Keywords: Spina bifida, health care transition, self-management

DOI: 10.3233/PRM-170446

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 177-183, 2017

Authors: Fauza, Dario O.

Article Type: Research Article

Abstract: Regenerative medicine as it applies to spina bifida is a multi-pronged endeavor involving spinal cord repair, tissue engineering and fetal regeneration, all of which can mutually overlap to variable extents. The efforts involving spinal cord repair, whether they be cell-based or not, are virtually indistinguishable from the enormous body of work related to spinal cord recovery after traumatic injury. Tissue engineering, on the other hand, can involve a variety of structures besides constructs used for covering the spina bifida defect, for example the urinary bladder, bone, muscle and skin. This brief review will not delve into any of these two …main areas, which actually can also involve fetal interventions within their respective realms, but rather be devoted to a very recent development making use of the uniquely enhanced ability of the fetus to repair, or regenerate areas of tissue damage, coined transamniotic stem cell therapy, or TRASCET. TRASCET is a still experimental therapeutic paradigm for the treatment of not only spina bifida, but also other birth defects, based on the principle of harnessing/enhancing the normal biological role of a select population of stem cells that naturally occur in the amniotic fluid, specifically amniotic fluid-derived mesenchymal stem cells (afMSCs), for therapeutic benefit. Show more

Keywords: Transamniotic stem cell therapy, TRASCET, amniotic mesenchymal stem cells, spina bifida

DOI: 10.3233/PRM-170449

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 185-188, 2017

Authors: Lupo, Philip J. | Agopian, A.J. | Castillo, Heidi | Castillo, Jonathan | Clayton, Gerald H. | Dosa, Nienke P. | Hopson, Betsy | Joseph, David B. | Rocque, Brandon G. | Walker, William O. | Wiener, John S. | Mitchell, Laura E.

Article Type: Research Article

Abstract: It has been estimated that 60–70% of neural tube defects (NTDs) have a genetic component, but few causative genes have been identified. The lack of information on genes associated with non-syndromic NTDs in humans is especially notable as the “genomic revolution” has led to new tools (e.g., genome-wide genotyping arrays, next-generation sequencing) that are helping to elucidate the full spectrum of genetic variation (from common to rare) contributing to complex traits, including structural birth defects. However, the application of modern genomic approaches to the study of NTDs has lagged behind that of some other common structural birth defects. This may …be due to the difficulty of assembling large study cohorts for anencephaly or spina bifida. The purpose of this review is to outline the evolution of genetic studies of NTDs, from studies of familial aggregation to candidate gene and genome-wide association studies, through whole-exome and whole-genome sequencing. Strategies for addressing gaps in NTD genetic research are also explored. Show more

Keywords: Epidemiology, genetics, human studies, neural tube defects, spina bifida

DOI: 10.3233/PRM-170456

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 189-194, 2017

Authors: Meneses, Veronica | Cruz, Natalie

Article Type: Research Article

Abstract: Children with developmental disabilities may be at greater risk for experiencing maltreatment and traumatic events, threats to their physical and psychological safety and well-being. Young children and youth with spina bifida benefit from being considered in light of this risk, and may be especially vulnerable given the complexity of their neurodevelopmental condition. A trauma-informed approach brings together evidence from the neurosciences, epidemiology and psychology to promote improved developmental, behavioral, physical and mental health status. Incorporating a trauma-informed approach strengthens a longitudinal, interdisciplinary and developmentally-oriented care model for children and youth with spina bifida. A case example further illustrates how using …a trauma-informed approach and evidence-based interventions can foster the development and well-being of youth with spina bifida who have been impacted by a traumatic event. Show more

Keywords: Children, youth, disabilities, spina bifida, maltreatment, trauma, medical trauma, risks, trauma-informed approach, evidence-based

DOI: 10.3233/PRM-170457

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 195-199, 2017

Authors: Pardo, Rosa

Article Type: Research Article

Abstract: In the last two decades in Chile events that have taken place in order to improve the prevention, management, and treatment of patients with spina bifida in Chile. Aspects of the implementation of a fortification of wheat flour with folic acid program and its evolution are described. Genetic studies performed in Chilean patients with spina bifida, improvements in coverage and medical management opportunities, as well as the creation of associations of families of patients with the condition have all evolved in Chile.

Keywords: Spina bifida, folic acid, fortification, treatment

DOI: 10.3233/PRM-170443

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 201-204, 2017

Authors: Thibadeau, Judy

Article Type: Research Article

Abstract: “The National Spina Bifida Patient Registry: Past, Present, and Future” was presented at the Spina Bifida World Congress, March 17, 2017, San Diego, California. This commentary provides a summary of registry activities including the reason for development, a description of the clinic participants and their patients who are participating, analytic works and publications. Two specific efforts that are related to the work of the registry, a urologic protocol to preserve renal function for newborns and young children, and a skin breakdown prevention bundle developed and implemented in registry clinics, are highlighted.

Keywords: Spina bifida, patient registry, interventions, outcomes

DOI: 10.3233/PRM-170463

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 205-210, 2017

Authors: Lyons, Gastón Valverde | Burek, Carol | Campmani, Liliana | De Castro, Fernanda | López, Beatriz | Sager, Cristian

Article Type: Research Article

Abstract: The purpose of this paper is to render an account of the experience of a group of healthcare providers and to propose innovative solutions for patients with Spina Bifida (SB) in Argentina. Based on our practice, patients reach specialists too late (40% already undergoing chronic kidney disease stage 1). However, several strategies were implemented in order to reverse this trend, among which were: the setting up of a professional network (via emails and Google groups), team training at different locations, videoconferences, and calling the Ministry of Health to action. Additionally, we developed a project through telemedicine to inform …patients and empower caregivers throughout Argentina. Show more

Keywords: Spina bifida, interdisciplinary care, professional network, telemedicine

DOI: 10.3233/PRM-170444

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 211-214, 2017

Authors: Martinez de Villarreal, Laura E.

Article Type: Research Article

Abstract: In 1980 Smithells et al. reported that the intake of folic acid (FA) prevents the recurrence of neural tube defects (NTDs) [1]. After this and several other studies were conducted, the intake of 400 micrograms of folic acid per day, at least three months before and three months during pregnancy for prevention of NTD, was proposed [2,3,4]. Other strategies were also developed to increase folate blood levels in woman of childbearing age such as promoting the consumption of folate rich foods and food fortification (flour and most recently rice) [5]. Nevertheless, results have not been as expected due to poor consumption …of folic acid supplements [6]. As a result, in the year 2000, a novel strategy was developed in order to increase folate blood levels in Mexican women. The results of the strategy are presented, as well as, a discussion about how to personalize a program for different populations. Show more

Keywords: Folic acid, NTD, spina bifida, prevention, mexican population

DOI: 10.3233/PRM-170445

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 215-217, 2017

Authors: Castillo, Jonathan | Ostermaier, Kathryn K. | Fremion, Ellen | Collier, Talia | Zhu, Huirong | Huang, Gene O. | Tu, Duong | Castillo, Heidi

Article Type: Research Article

Abstract: PURPOSE: To describe the age of independence in intermittent self-catheterization (ISC) in a diverse patient population and identify factors associated with ISC in individuals with spina bifida. METHODS: Two hundred patients with myelomeningocele or lipomyelomeningocele, who were ⩾ 3 years of age and utilized catheterization for bladder management were included. Data regarding diagnosis, functional level of lesion, race, ethnicity, presence of shunt, method of catheterization, self-management skills, fine motor skills, and cognitive abilities were collected. RESULTS: Fifty-five percent of individuals were able to perform ISC with a mean age of …9.45 years (SD = 2.97) and 22.7% used a surgically created channel. Higher level of lesion and female gender were associated with a lower rate of ISC. Intellectual disability was present in 15% of the individuals able to perform ISC and in 40% of those not able to perform ISC (p = 0.0005). Existent self-efficacy regarding activities of daily living (i.e. dressing, bathing, skin care) were associated with ISC (p < 0.0001). CONCLUSIONS: The average age of ISC emerged as a target for culturally-appropriate educational interventions to stimulate greater early independence. Future research on factors that may foster an ‘independent spirit’ early in childhood leading to self-management are warranted. Show more

Keywords: Spina bifida, myelomeningocele, neurogenic bladder, self-catheterization, self-management

DOI: 10.3233/PRM-170447

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 219-226, 2017

Authors: Collins, Katrina

Article Type: Research Article

Abstract: PURPOSE: To inform the strategic and operational development of a community based service model at the Crann Centre, Cork, Ireland for SB children, adults, their families and providers. A needs assessment was conducted by gathering the views of multiple stakeholder perspectives within the SB community in the geographical region the Centre will serve. The intention is to create project deliverables that are responsive to the needs highlighted through this research. METHODS: The study used a multi method design with a participatory research approach to explore the needs of SB individuals, families and providers. This involved …in depth interviews, focus groups and online surveys. RESULTS: One hundred and fifty-nine respondents contributed to this qualitative needs assessment. The research established a range of psychosocial, clinical, vocational and educational issues causing ongoing difficulties for SB individuals and families. Providers highlighted supports that would benefit the social and clinical wellbeing of persons with SB. Collectively participants in the study reported that there was an absence of coordinated, continuous and comprehensive service delivery for the SB community in the region. This was amplified by geographical location of services and access to relevant supports. CONCLUSION: Consensus across stakeholders in this research pointed to the necessity for an innovative model of community based provision at the Crann Centre. This was described as offering a service with family at the core of an assets based model of practice. A key finding was the lack of importance placed on the social and emotional development of SB individuals. Traditionally participants described a singular focus on physical health through clinically defined treatment models. The desire for a social model of disability that informed health and wellbeing of SB individuals and families emerged as a prominent recommendation from the research. Show more

Keywords: Spina bifida, needs assessment, community provision, family led support

DOI: 10.3233/PRM-170448

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 227-230, 2017

Authors: Freeman, Kurt A. | Castillo, Heidi | Castillo, Jonathan | Liu, Tiebin | Schechter, Michael | Wiener, John S. | Thibadeau, Judy | Ward, Elisabeth | Brei, Timothy

Article Type: Research Article

Abstract: PURPOSE: Continence is low in individuals with spina bifida, but published prevalence varies markedly across studies. The objective of this study was to examine bladder and bowel continence among patients served by multidisciplinary clinics participating in the National Spina Bifida Patient Registry and to examine whether variation in prevalence exists across clinics. METHODS: Data were obtained from patients 5 years and older from March 2009 to December 2012. Data were gathered at clinic visits using standardized definitions. RESULTS: Data from 3252 individuals were included. Only 40.8% of participants were continent of urine; 43% …were continent of stool. Bladder and bowel continence differed by spina bifida type, with those with myelomeningocele having significantly lower reported prevalence of continence than those with other forms of spina bifida. Bladder and bowel continence varied across registry sites. Adjustment based on demographic and condition-specific variables did not make substantive differences in prevalence observed. CONCLUSION: Less than half of spina bifida patients served in multidisciplinary clinics report bladder or bowel continence. Variability in prevalence was observed across clinics. Further research is needed to examine if clinic-specific variables (e.g., types of providers, types of interventions used) account for the observed variation. Show more

Keywords: Spina bifida, continence, neurogenic bladder, urologic management, bowel and bladder impairment, registry

DOI: 10.3233/PRM-170450

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 231-241, 2017

Authors: Fremion, Ellen | Morrison-Jacobus, Melissa | Castillo, Jonathan | Castillo, Heidi | Ostermaier, Kathryn

Article Type: Research Article

Abstract: Providing comprehensive transition care for adolescents and young adults with spina bifida (AYASB) requires a structured approach to addressing chronic condition management, self-management, care coordination, and health care navigation that is adaptable to the various levels of cognitive ability, physical function, and family/community environments within the population. This commentary (1) highlights AYASB transition program needs identified in the literature and within a local community, (2) analyzes advantages and limitations of published AYASB transition care models in addressing these needs, (3) demonstrates how a spina bifida (SB) transition clinic used the Chronic Care Model (CCM) to develop a comprehensive AYASB transition …program, and (4) examines the potential feasibility in adapting this model to other SB clinics. A SB-specific transition clinic based on the CCM model facilitates the complex chronic care management and transition planning for AYASB. Further study is needed to evaluate health care outcomes using the CCM for SB transition. Show more

Keywords: Spina bifida, adolescence, transition to adult care, transition clinic model, chronic care model

DOI: 10.3233/PRM-170451

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 243-247, 2017

Authors: Gonzalez, Dani O. | Cooper, Jennifer N. | McLeod, Daryl J.

Article Type: Research Article

Abstract: PURPOSE: Most patients with spina bifida require ventriculoperitoneal (VP) shunt placement. Some also require bladder augmentation, which may increase the risk of VP shunt malfunction and/or failure. The aim of this study was to assess whether bladder augmentation affects the rate of VP shunt failure in this population. METHODS: Using the Pediatric Health Information System, we studied patients with spina bifida born between 1992 and 2014 who underwent VP shunt placement. Using conditional logistic regression, we compared age- and hospital-matched patients who did and did not undergo a bladder augmentation to determine their difference in rates …of VP shunt failure. RESULTS: There were 4192 patients with spina bifida who underwent both surgical closure and VP shunt placement. Of these, 203 patients with bladder augmentation could be matched to 593 patients without bladder augmentation. VP shunt failure occurred within 2 years in 7.7% of patients, the majority of whom were in the group who underwent bladder augmentation (87%). After adjusting for confounders, undergoing bladder augmentation was independently associated with VP shunt failure (HR: 33.5, 95% CI: 13.15–85.44, p < 0.001). CONCLUSION: Bladder augmentation appears to be associated with VP shunt failure. Additional studies are necessary to better define this relationship and identify risk-reduction techniques. Show more

Keywords: Spina bifida, VP shunt, myelomeningocele, VP shunt failure, bladder augmentation, shunt infection

DOI: 10.3233/PRM-170452

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 249-255, 2017

Authors: Jiang, Ruiyang | Inouye, Brian M. | Wang, Hsin-Hsiao S. | Tejwani, Rohit | Routh, Jonathan C.

Article Type: Research Article

Abstract: INTRODUCTION: Cost-utility analyses (CUA) are useful when the treatment conditions depend on patient preferences that are in turn dependent on health state utility value. Spina bifida (SB) is an example of such a preference-sensitive condition. Historically, the SB utility value for CUA has been gathered via a traditional face-to-face interview. However, due to funding and time constrains, utility estimation via online crowdsourcing has recently gained popularity. Our aim was to estimate the utility value for a generic SB health state using a validated online tool. METHODS: A cross-sectional survey of American adults was conducted using the …time-trade-off (TTO) method. Participants were recruited from an online crowdsourcing interface, Amazon’s Mechanical Turk (mTurk). Demographic information and prior knowledge of SB were assessed. Respondents were provided a written passage and an online video explaining SB and its potential associated comorbidities. Participants were queried on hypothetical ascending time-trades from a child-parent dyad perspective to determine the utility of a SB health state in an affected 6-year-old child. Respondents were also asked to indicate the percentage of time traded from their life in relation to their child’s. Utility estimates were then calculated and compared using bivariate and multivariate analyses. RESULTS: We obtained 503 responses (85% response rate). Mean respondent age was 34 (± 11); 247 (49%) were female; 386 (77%) were white; 189 (38%) were married, and 234 (46%) had children. Mean proportion of longevity traded by participants in the dyadic interaction was 66% (± 27) from the parent’s life. Only 51 respondents (9%) reported having “ample” prior knowledge of SB; 8 respondents (0.02%) had SB themselves. Few others had previous experience with SB or myelomeningocele either in a child (4, 1%), or friend/relative (28, 5%). Compared with a perfect health state of 1.0, we found mean utilities of 0.85 (±  0.20) for SB. CONCLUSIONS: Utility estimation for SB is feasible through crowdsourcing, and the resultant values are similar to previous estimates using traditional techniques. Subjects view the SB health state to be inferior to perfect health. Show more

Keywords: Pediatric, spina bifida, myelomeningocele, utility

DOI: 10.3233/PRM-170453

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 257-266, 2017

Authors: Jimenez-Gomez, Andres | Castillo, Heidi | Burckart, Caryssa | Castillo, Jonathan

Article Type: Research Article

Abstract: PURPOSE: Endoscopic Third Ventriculostomy (ETV) and Choroid Plexus Cautery (CPC) are low-cost, safe, and promising interventions for spina bifida-associated hydrocephalus (SBHCP). The purpose of this review was to explore and describe these efforts in Africa in order to upscale surgical training and rehabilitation services. METHODS: A PubMed search for articles on ETV and CPC as management of SBHCP in Africa was performed. Two authors appraised the results for key themes in content: indications, technique, outcomes, complications, education, and rehabilitation. RESULTS: Twenty of 47 articles identified were included for appraisal. Twelve described indications, ten …and seven outlined technique and complications, respectively, and four described predictors of operative success. Fourteen studies describe outcomes, including operative and neurodevelopmental outcomes. Only two outlined educational efforts. Half of the literature stems from a single site in Uganda; in total, only six countries were represented. No articles described significant post-operative rehabilitation services or related training. CONCLUSION: The experience of ETV and CPC in Africa is promising, however, efforts to train and empower local staff in surgical technique and methods to upscale post-operative community-based rehabilitation services remain as a key to long-term success. Show more

Keywords: Myelomeningocele, hydrocephalus, global health, neurosurgery, rehabilitation, Africa

DOI: 10.3233/PRM-170454

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 267-273, 2017

Authors: Kelly, Maryellen S. | Thibadeau, Judy | Struwe, Sara | Ramen, Lisa | Ouyang, Lijing | Routh, Jonathan

Article Type: Research Article

Abstract: PURPOSE: Recent studies have revealed that the lack of continuity in preparing patients with spina bifida to transition into adult-centered care may have detrimental health consequences. We sought to describe current practices of transitional care services offered at spina bifida clinics in the US. METHODS: Survey design followed the validated transitional care survey by the National Cystic Fibrosis center. Survey was amended for spina bifida. Face validity was completed. Survey was distributed to registered clinics via the Spina Bifida Association. Results were analyzed via descriptive means. RESULTS: Total of 34 clinics responded. Over …90 characteristics were analyzed per clinic. The concept of transition is discussed with most patients. Most clinics discuss mobility, bowel and bladder management, weight, and education plans consistently. Most do not routinely evaluate their process or discuss insurance coverage changes with patients. Only 30% communicate with the adult providers. Sexuality, pregnancy and reproductive issues are not readily discussed in most clinics. Overall clinics self-rate themselves as a 5/10 in their ability to provide services for their patients during transition. CONCLUSIONS: Characteristics of current transitional care services and formal transitional care programs at US clinics show wide variances in what is offered to patients and families. Show more

Keywords: Spina bifida, transitional care, myelomeningocele

DOI: 10.3233/PRM-170455

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 275-281, 2017

Authors: Roiz, Ronald | Mueske, Nicole M. | Van Speybroeck, Alexander | Ryan, Deirdre D. | Gilsanz, Vicente | Wren, Tishya A. L.

Article Type: Research Article

Abstract: PURPOSE: Atypical skeletal development is common in youth with myelomeningocele (MM), though the underlying reasons have not been fully elucidated. This study assessed skeletal maturity in children and adolescents with MM and examined the effects of sex, age, sexual development, ethnicity, anthropometrics and shunt status. METHODS: Forty-three males and 35 females with MM, 6–16 years old, underwent hand radiographs for bone age determination. The difference between bone age and chronological age was evaluated using Wilcoxon sign rank tests. Relationships between age discrepancy (skeletal-chronological) and participant characteristics were assessed using multiple linear regression with forward selection. …RESULTS: Overall, forty percent (31/78) of MM participants had an advanced bone age of 1 year or greater (median: 2.5 years), while 47% (37/78) were within 1 year above or below their chronological age (- 0.001 years) and 13% (10/78) were delayed by more than 1 year (- 1.4 years). Bone age was advanced compared to chronologic age in both males and females (p ⩽ 0.024). Advanced bone age was observed in early to late puberty and after maturation (p ⩽ 0.07), as well as in Hispanic participants (p = 0.003) and in those with a shunt (p = 0.0004). Advanced bone age was positively correlated with height, weight and body mass index (BMI) percentiles (p = 0.004). In multiple linear regression analysis, advanced bone age was most strongly associated with higher Tanner stage of sexual development, and higher weight, height or BMI percentile. CONCLUSIONS: Advanced skeletal maturity is common in children/adolescents with MM over 8 years of age who have reached puberty (65%), particularly those who are overweight (80%). Hormonal effects associated with adiposity and sexual maturity likely influence skeletal maturation. Clinicians may use Tanner stage and weight or BMI to gain insight into skeletal maturity. Show more

Keywords: Skeletal maturity, myelomeningocele, bone age, spina bifida, pediatrics

DOI: 10.3233/PRM-170458

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 283-293, 2017

Authors: Persson, Marika | Janeslätt, Gunnel | Peny-Dahlstrand, Marie

Article Type: Research Article

Abstract: PURPOSE: Spina bifida (SB) often results in a complex disability and can also cause cognitive dysfunction. No previous study has investigated the ability to adapt to time in children with SB. This ability is crucial for an individual’s possibility to develop autonomy in life. The purpose of this study was to investigate whether children aged 10–17 with SB have lower time-processing abilities than typically-developing children, and to describe the profile of time-processing in children with SB. METHOD: Participants comprised a consecutive sample of 21 children (drawn from a geographical cohort of 45) aged 10–17 years (mean: 14 …years, SD: 2 years); 13 were boys. The instruments used were KaTid-Y, Time-S, and Time-P. RESULTS: The children with SB had lower time-processing abilities than typically-developing children (52.4% under - 2SD), particularly difficulties to orient to and to estimate objective time, to understand time perspectives and with time planning. They also self-rated low use of strategies to adapt to time. The parents rated their children as having extensive difficulties in daily time management. CONCLUSIONS: The low time-processing ability found in children with SB is likely to be an important contributing factor to low autonomy and independence. Show more

Keywords: Myelomeningocele, time perception, time management, time orientation, executive function

DOI: 10.3233/PRM-170459

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 295-302, 2017

Authors: Routh, Jonathan C. | Joseph, David B. | Liu, Tiebin | Schechter, Michael S. | Thibadeau, Judy K. | Chad Wallis, M. | Ward, Elisabeth A. | Wiener, John S.

Article Type: Research Article

Abstract: PURPOSE: Optimal management of neurogenic bowel in patients with spina bifida (SB) remains controversial. Surgical interventions may be utilized to treat constipation and provide fecal continence, but their use may vary among SB treatment centers. METHODS: We queried the National Spina Bifida Patient Registry (NSBPR) to identify patients who underwent surgical interventions for neurogenic bowel. We abstracted demographic characteristics, SB type, functional level, concurrent bladder surgery, mobility, and NSBPR clinics to determine whether any of these factors were associated with interventions for management of neurogenic bowel. Multivariable logistic regression with adjustment for selection bias was performed. …RESULTS: We identified 5,528 patients with SB enrolled in the 2009-14 NSBPR. Of these, 1,088 (19.7%) underwent procedures for neurogenic bowel, including 957 (17.3%) ACE/cecostomy tube and 155 (2.8%) ileostomy/colostomy patients. Procedures were more likely in patients who were older, white, non-ambulatory, with higher-level lesion, with myelomeningocele lesion, with private health insurance (all p < 0.001), and female (p = 0.006). On multivariable analysis, NSBPR clinic, older age (both p < 0.001), race (p = 0.002), mobility status (p = 0.011), higher lesion level (p < 0.001), private insurance (p = 0.002) and female sex (p = 0.015) were associated with increased odds of surgery. CONCLUSIONS: There is significant variation in rates of procedures to manage neurogenic bowel among NSBPR clinics. In addition to SB-related factors such as mobility status and lesion type/level, non-SB-related factors such as patient age, sex, race and treating center are also associated with the likelihood of undergoing neurogenic bowel intervention. Show more

Keywords: Pediatrics, spina bifida, neurogenic bowel, clinical care variation

DOI: 10.3233/PRM-170460

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 303-312, 2017

Authors: Szymanski, Konrad M. | Hensel, Devon J. | Wiener, John S. | Whittam, Benjamin | Cain, Mark P. | Misseri, Rosalia

Article Type: Research Article

Abstract: PURPOSE: Sexuality has received little attention in spina bifida (SB) care. The aim of this study was to assess sexual identity and orientation in adults with SB. METHODS: An international online survey to adults with SB was administered over 10-months (recruitment: SB clinics, SB organizations via social media). Collected data included demographics, sexual identity and orientation. Non-parametric tests were used for analysis. RESULTS: Median age of 77 men and 119 women was 35 years old (52.0% shunted, 48.5% community ambulators, 42.3% outside United States). Most commonly, men identified as male (96.1%), while 1.3% …each described themselves as female, transgender and other. All women reporting sexual identity identified as female (99.2%), 0.8% not providing an answer. Most men reported heterosexual orientation (89.6%), followed by gay (7.8%) and bisexual (2.6%). Most women reported heterosexual orientation (84.9%), followed by bisexual (10.4%), gay/lesbian (2.5%), asexual (0.8%) and other (1.7%). CONCLUSION: As in the general population, sexual identity typically coincides with biological gender. Sexual orientation of adults with SB mirrors the general population. Due to self-selection, these findings likely do not reflect exact prevalence in the SB population. Show more

Keywords: Sexuality, spinal dysraphism, adult, neurogenic urinary bladder

DOI: 10.3233/PRM-170462

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 313-317, 2017

Authors: Timberlake, Matthew D. | Kern, Adam J. | Adams, Richard | Walker, Candice | Schlomer, Bruce J. | Jacobs, Micah A.

Article Type: Research Article

Abstract: PURPOSE: Report urologic outcomes among newborns with spinal dysraphism managed within an expectant clean intermittent catheterization (CIC) program. METHODS: Newborns were followed clinically and with serial ultrasound (US). Urodynamics (UD) and dimercaptosuccinic acid (DMSA) renal scan were obtained at 3–6 months, 1 year, 3 years, then as needed. Patients with initial evaluation after 6 months were excluded. RESULTS: Median follow-up was 3.2 years. 11/102 began catheterization for continence (median 4.0 years) and 47/102 did not start CIC. Of these, 2/58 developed a DMSA abnormality. 44/102 began CIC early, often for elevated storage pressures and febrile …urinary tract infection (UTI). Of these, 20/44 developed a DMSA abnormality including 9 who had abnormality detected prior to starting CIC. Being on CIC or starting immediately upon recognition of new hydronephrosis, reflux, elevated filling pressures, or febrile UTI was associated with lower chance of DMSA abnormalities (4/17, 24%) compared to delaying CIC (16/27, 60%) (p = 0.03). CONCLUSIONS: CIC can be deferred until continence in select infants with a low risk of significant DMSA abnormality. However, immediate initiation of CIC upon recognition of risk factors is recommended as this was associated with fewer DMSA abnormalities than delaying CIC. Recommendations for expectantly-managed patients include close follow-up, serial US and UD, and prompt initiation of CIC upon recognition of new hydronephrosis, reflux, elevated storage pressures, or febrile UTIs. Show more

Keywords: Spinal dysraphism, spina bifida, newborn management, clean intermittent catheterization, expectant management, DMSA abnormalities, urodynamics, renal outcomes urinary tract infection

DOI: 10.3233/PRM-170464

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 319-325, 2017

Authors: Huang, Gene O. | Belfort, Michael A. | Whitehead, William E. | Olutoye, Oluyinka O. | Castillo, Jonathan | Castillo, Heidi | Ostermaier, Kathryn K. | Koh, Chester J. | Tu, Duong D.

Article Type: Research Article

Abstract: PURPOSE: Prenatal repair of myelomeningocele (MMC) via hysterotomy has demonstrated neurosurgical and motor benefits, when compared to postnatal repairs. Urologic benefits, however, remain to be seen. The purpose of this study was to review early postnatal bladder function in patients undergoing a novel endoscopic approach for MMC repair using an exteriorized uterus. METHODS: A prospective urologic assessment of patients undergoing fetoscopic MMC repair and receiving subsequent care at our facility, was performed. Patients were managed and urodynamic studies risk-stratified according to the Centers for Disease Control and Prevention Urologic and Renal Protocol for the Newborn and …Young Child with Spina Bifida. RESULTS: Fetoscopic MMC repair was performed in 14 patients. No patients had hydronephrosis or bladder thickening at birth. Detrusor overactivity was observed in nine (64.3%) patients. Impaired compliance was seen in eight (57.1%) patients. No patients had a detrusor leak point pressure of > 40 cm H 2 O or evidence of detrusor sphincter dyssynergia. Three (21.4%) patients had vesicoureteral reflux, seven (50.0%) had an open bladder neck, and none had trabeculated bladders. CONCLUSION: In this early experience with fetoscopic MMC repair, postnatal bladder function does not appear to be any worse than that of previously reported prenatal or postnatal closures. Show more

Keywords: Neural tube defects, spinal dysraphism, neurogenic urinary bladder, urodynamics

DOI: 10.3233/PRM-170465

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 327-333, 2017

Authors: Wiener, John S. | Suson, Kristina D. | Castillo, Jonathan | Routh, Jonathan C. | Tanaka, Stacy | Liu, Tiebin | Ward, Elisabeth | Thibadeau, Judy | Joseph, David | Registry, National Spina Bifida Patient

Article Type: Research Article

Abstract: INTRODUCTION: Advances in care have allowed most children with spina bifida (SB) to live to adulthood. The majority have neuropathic bowel dysfunction (NBD), resulting in constipation, incontinence, and diminished quality of life. We sought to 1) describe contemporary NBD management and continence outcomes of adults with SB; 2) describe differences from younger patients; and 3) assess for association with socio-economic factors. METHODS: We analyzed data on NBD management and continence from the National Spina Bifida Patient Registry (NSBPR). Patients were segregated into young children (5–11 years), adolescents (12–19 years), and adults (20 years and older). A strict …definition of continence was utilized. Statistical analysis compared cohorts by gender, ethnicity, SB type, lesion level, insurance status, educational attainment, employment status, and continence. RESULTS: A total of 5209 SB patients were included, of whom 1370 (26.3%) were adults. Management and continence varied by age and SB type. Oral medication use did not differ between groups (5.2–6.6%). Suppositories and rectal enemas were used only by 11.5% of adults, which was significantly less than among school-aged children. Antegrade enemas were used by 17.7% of adults which was significantly less than among adolescents (27.2%). Adults were more likely to use digital stimulation or disimpaction or have undergone a colostomy. Bowel continence was reported by 58.3% of overall adult cohort: 55.6% of adults with myelomeningocele and 74.9% with non-myelomeningocele. Bowel continence was significantly associated with employment (p = 0.0002), private insurance (p = 0.0098), non-myelomeningocele type of SB (p = 0.0216) and educational attainment (p = 0.0324) on univariate analysis but only with employment on multivariable logistic regression (p = 0.0027). CONCLUSIONS: Bowel management techniques differed between adults and younger patients with SB. Bowel continence was reported by over half of SB adults and was associated with socio-economic factors. Show more

Keywords: Neurogenic bowel, spina bifida, fecal incontinence

DOI: 10.3233/PRM-170466

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 335-343, 2017

Article Type: Other

DOI: 10.3233/PRM-170467

Citation: Journal of Pediatric Rehabilitation Medicine, vol. 10, no. 3-4, pp. 345-346, 2017