Assessment of Ventricular Size and Neurocognitive Outcomes in Children with Post-natal Closure of Myelomeningocele.

OBJECTIVE: To assess if ventricular size prior to shunting is correlated with neurodevelopmental outcomes in children with post-natal myelomeningocele closure. STUDY DESIGN: This was a retrospective review of children with post-natal surgical closure of myelomeningocele and neuropsychological testing between 2018 through 2023 at UCSF. Frontal-occipital horn ratio (FOHR) was measured immediately prior to shunt placement, or on the first study that reported ventricular stability for non-shunted patients. The primary outcome was full scale IQ (FSIQ) on the Weschler Intelligence Scale. Secondary outcomes included indices of the Weschler scale, the Global Executive Composite from the Behavior Rating Inventory of Executive Function, and the General Adaptive Composite from the Adaptive Behavior Assessment Scale. Uni- and multi-variable regression was used to determine if FOHR was correlated with neuropsychological scores. RESULTS: Forty patients met inclusion criteria; 26 (65%) had shunted hydrocephalus. Age at neuropsychological testing was 10.9+/-0.6 years. FOHR was greater in the shunted group (0.64 vs 0.51, p<0.001). There were no differences in neuropsychological results between shunted and non-shunted groups. On univariable analysis, greater FOHR was associated with lower FSIQ (p=0.025) and lower Visual Spatial Index scores (p=0.013), which remained significant on multivariable analysis after adjusting for gestational age at birth, lesion level, shunt status, and shunt revision status (p=0.049 and p=0.006, respectively). Separate analyses by shunt status revealed that these effects were driven by the shunted group. CONCLUSION: Greater FOHR prior to shunting was correlated with lower FSIQ and the VSI scores on the Weschler Intelligence Scales. Larger studies will be needed to explore further the relationship between ventricle size, hydrocephalus, and neurodevelopmental outcomes.

What is the Role of Scoliosis Surgery in Adolescents and Adults with Myelomeningocele? A Systematic Review.

BACKGROUND: Life expectancy of individuals with spina bifida has continued to improve over the past several decades. However, little is known about the longitudinal course of scoliosis in individuals with myelomeningocele (MMC), a spina bifida subtype, across their lifespan. Specifically, it is not known whether management during or after the transition years from adolescence to adulthood is associated with comorbidities in adulthood nor if these individuals benefit from scoliosis treatment later in life. QUESTIONS/PURPOSES: In this systematic review, we asked: (1) Is the risk of secondary impairments (such as bladder or bowel incontinence, decreased ambulation, and skin pressure injuries) higher among adolescents and adults with MMC and scoliosis than among those with MMC without scoliosis? (2) Is there evidence that surgical management of scoliosis is associated with improved functional outcomes in adolescents and adults with MMC? (3) Is surgical management of scoliosis associated with improved quality of life in adolescents and adults with MMC? METHODS: We performed a systematic review of articles in Medline and Embase from 2000 until February 5, 2021. Search terms such as "spinal dysraphism," "spina bifida," "meningomyelocele," and "scoliosis" were applied in diverse combinations. A total of 1429 publications were identified, and 13 were eligible for inclusion. We included original studies reporting on scoliosis among individuals older than 15 years with MMC. When available, we extracted the prevalence of MMC and scoliosis, studied population age, percentage of patients experiencing complications, functional outcomes, and overall physical function. We excluded non-English articles and those with fewer than 10 individuals with scoliosis and MMC. We used the Preferred Reporting Items for Systematic Reviews and Meta-analyses, and registered the review before data collection (PROSPERO: CRD42021236357). We conducted a quality assessment using the Methodologic Index for Nonrandomized Studies (MINORS) tool. In 13 included studies, there were 556 individuals with MMC and scoliosis. Most were retrospective case series, although a minority were retrospective/comparative studies. The mean MINORS score was 12.3 ± 1.65 (a MINORS score over 12 generally is considered good reporting quality, scores below 12 are considered at high risk of bias). RESULTS: In general, studies found that individuals with MMC and scoliosis were more likely to have secondary impairments such as bladder/bowel incontinence, decreased ambulation, and pressure injuries than were patients with MMC without scoliosis. These secondary impairments were associated with hydrocephalus and high-level MMC lesions. However, when one study evaluated mortality, the results showed that although most deceased individuals who had spina bifida had scoliosis, no association was found between the age of death and scoliosis. Among the studies evaluating functional outcomes, none supported strong functional improvement in individuals with MMC after surgery for scoliosis. No correlation between the Cobb angle and sitting balance was noted; however, the degree of pelvic obliquity and the level of motor dysfunction showed a strong correlation with scoliosis severity. There was no change in sitting pressure distributions after spinal surgery. The lesion level and scoliosis degree independently contributed to the degree of lung function impairment. Although studies reported success in correcting coronal deformity and stopping curve progression, they found no clear benefit of surgery on health-related quality of life and long-term outcomes. These studies demonstrated that the level of neurologic function, severity of hydrocephalus, and brainstem dysfunction are greater determinants of quality of life than spinal deformity. CONCLUSION: This systematic review found that adolescents and adults with MMC and scoliosis are more likely to have secondary impairments than their peers with MMC only. The best-available evidence does not support strong functional improvement or health-related quality of life enhancement after scoliosis surgery in adolescents and adults with MMC. The level of neurologic dysfunction, hydrocephalus, and brainstem dysfunction are greater determinants of quality of life. Future prospective studies should be designed to answer which individuals with MMC and scoliosis would benefit from spinal surgery. Our findings suggest that the very modest apparent benefits of surgery should cause surgeons to approach surgical recommendations in this patient population with great caution, and surgeons should counsel patients and their families that the risk of complications is high and the benefits may be small. LEVEL OF EVIDENCE: Level IV, therapeutic study.

Prevalence of high-risk bladder categorization with prenatal and postnatal myelomeningocele repair types.

INTRODUCTION: Urologic substudies of prenatal myelomeningocele (MMC) closure have focused primarily on continence without significant clinical benefit. Fetoscopic MMC repair (FMR) is a newer form of prenatal intervention and touts added benefits to the mother, but urological outcomes have yet to be analyzed. We set out to focus on bladder safety rather than continence and examined bladder outcomes with different prenatal MMC repairs (FMR and prenatal open [POMR]) and compared bladder-risk-categorization to traditional postnatal repair (PSTNR). METHODS: An IRB-approved retrospective analysis of all patients undergoing all forms of MMC repairs with inclusion and exclusion criteria based on the MOMS trial was performed. Bladder safety assessment required initial urodynamic studies (UDS), renal bladder ultrasound (RBUS), and/or voiding cystourethrogram (VCUG) within the 1st year of life. Follow-up analyses within the cohorts required follow-up studies within 18 months after initial evaluations. Outcomes assessed included bladder-risk-categorization based on the CDC UMPIRE study (high, intermediate, and safe), hydronephrosis (HN), and vesicoureteral reflux (VUR). A single reader evaluated each UDS. RESULTS: Initial UDS in 93 patients showed that the prevalence of high-risk bladders were 35% FMR versus 36% PSTNR and 60% POMR. Follow-up UDS showed only 8% of FMR were high-risk compared to 35% POMR and 36% PSTNR. Change from initial to follow-up bladder-risk-category did not reach significance (p = .0659); however, 10% PSTNR worsened to high-risk on follow-up, compared to none in either prenatal group. Subanalysis of follow-up UDS between the prenatal cohorts also was not significant (p = .055). Only 8% of FMR worsened or stayed high-risk compared to 35% with POMR (p = .1). HN was significantly different at initial and subsequent follow up between the groups with the least in the FMR group. CONCLUSIONS: Early outcome UDS analyses demonstrated lower incidence of high-risk bladders in FMR patients with a trend toward clinically significant improvement compared to POMR in regard to all evaluated metrics. Larger, prospective, confirmatory studies are needed to further evaluate the potential benefits on FMR on bladder safety and health.

Neurosurgical training and global health education: systematic review of challenges and benefits of in-country programs in the care of neural tube defects.

OBJECTIVE: The recognition that neurosurgeons harbor great potential to advocate for the care of individuals with neural tube defects (NTDs) globally has sounded as a clear call to action; however, neurosurgical care and training in low- and middle-income countries (LMICs) present unique challenges that must be considered. The objective of this study was to systematically review publications that describe the challenges and benefits of participating in neurosurgery-related training programs in LMICs in the service of individuals with NTDs. METHODS: Using MEDLINE (PubMed), the authors conducted a systematic review of English- and Spanish-language articles published from 1974 to 2019 that describe the experiences of in-country neurosurgery-related training programs in LMICs. The inclusion criteria were as follows-1) population/exposure: US residents, US neurosurgeons, and local in-country medical staff participating in neurosurgical training programs aimed at improving healthcare for individuals with NTDs; 2) comparison: qualitative studies; and 3) outcome: description of the challenges and benefits of neurosurgical training programs. Articles meeting these criteria were assessed within a global health education conceptual framework. RESULTS: Nine articles met the inclusion criteria, with the majority of the in-country neurosurgical training programs being seen in subregions of Africa (8/9 [89%]) and one in South/Central America. US-based residents and neurosurgeons who participated in global health neurosurgical training had increased exposure to rare diseases not common in the US, were given the opportunity to work with a collaborative team to educate local healthcare professionals, and had increased exposure to neurosurgical procedures involved in treating NTDs. US neurosurgeons agreed that participating in international training improved their own clinical practices but also recognized that identifying international partners, travel expenses, and interference with their current practice are major barriers to participating in global health education. In contrast, the local medical personnel learned surgical techniques from visiting neurosurgeons, had increased exposure to intraoperative decision-making, and were given guidance to improve postoperative care. The most significant challenges identified were difficulties in local long-term retention of trained fellows and staff, deficient infrastructure, and lower compensation offered for pediatric neurosurgery in comparison to adult care. CONCLUSIONS: The challenges and benefits of international neurosurgical training programs need to be considered to effectively promote the development of neurosurgical care for individuals with NTDs in LMICs. In this global health paradigm, future work needs to investigate further the in-country professionals' perspective, as well as the related outcomes.

Evaluation and Disposition of Fetal Myelomeningocele Repair Candidates: A Large Referral Center Experience.

BACKGROUND: Fetal myelomeningocele (fMMC) repair yields superior outcomes to postnatal repair and is increasingly offered at select fetal centers. OBJECTIVES: To report the fMMC referral process from initial referral to evaluation and surgical intervention in a large fetal referral center. METHODS: We conducted a retrospective cohort study of patients referred to Texas Children's Fetal Center for fMMC between September 2013 and January 2018, reviewing the process from referral to final disposition. The stepwise evaluation included a phone interview followed by multidisciplinary consultation at our fetal center. We modified the Management of Myelomeningocele Study inclusion and exclusion criteria to allow a maternal body mass index of 35-40 on an individual basis. RESULTS: A total of 204 referrals were contacted for a phone interview; 175 (86%) pursued outpatient evaluation, and 80 (46%) of them qualified for repair. Among the eligible patients, 37 (46%) underwent fetoscopic repair, 20 (25%) underwent open repair, and 17 (21%) declined prenatal surgery. Of the 89 noneligible patients (53%) excluded upon outpatient evaluation, 64 (72%) were excluded for fetal and 17 (19%) for maternal reasons. No hindbrain herniation (16%) and maternal BMI and/or hypertension (5%) were the most common reasons for fetal and maternal exclusion, respectively. A total of 31% of our referral population underwent fetal surgery. CONCLUSIONS: A small percentage of fMMC referrals ultimately undergo prenatal surgery. Stepwise evaluation and multidisciplinary teams are key to the success of large referral programs.

Longitudinal Study of Bladder Continence in Patients with Spina Bifida in the National Spina Bifida Patient Registry.

PURPOSE: Achieving bladder continence in individuals with spina bifida is a lifetime management goal. We investigated bladder continence status through time and factors associated with this status in patients with spina bifida. MATERIALS AND METHODS: We used National Spina Bifida Patient Registry data collected from 2009 through 2015 and applied generalized estimating equation models to analyze factors associated with bladder continence status. RESULTS: This analysis included 5,250 participants with spina bifida in a large, multi-institutional patient registry who accounted for 12,740 annual clinic visit records during the study period. At last followup mean age was 16.6 years, 22.4% of participants had undergone bladder continence surgery, 92.6% used some form of bladder management and 45.8% reported bladder continence. In a multivariable regression model the likelihood of bladder continence was significantly greater in those who were older, were female, were nonHispanic white, had a nonmyelomeningocele diagnosis, had a lower level of lesion, had a higher mobility level and had private insurance. Continence surgery history and current management were also associated with continence independent of all other factors (adjusted OR and 95% CI 1.9, 1.7-2.1 and 3.8, 3.2-4.6, respectively). The association between bladder management and continence was stronger for those with a myelomeningocele diagnosis (adjusted OR 4.6) than with nonmyelomeningocele (adjusted OR 2.8). CONCLUSIONS: In addition to demographic, social and clinical factors, surgical intervention and bladder management are significantly and independently associated with bladder continence status in individuals with spina bifida. The association between bladder management and continence is stronger in those with myelomeningocele.

Comparing binary & ordinal definitions of urinary & stool continence outcomes: Data from the National Spina Bifida Patient Registry.

INTRODUCTION: The National Spina Bifida Patient Registry (NSBPR) assesses bladder and bowel incontinence using ordinal categories, but prior NSBPR analyses employed binary classification. Our aims were to 1) perform the first NSBPR analysis of bladder and bowel incontinence as ordinal outcomes to compare to the binary definition and subject variables; 2) explore the correlation of incontinence with undergarment usage, and 3) assess incontinence status following continence surgeries. METHODS: Data from NSBPR participants' most recent clinic visit from 2013 to 2020 were analyzed. Ordinal categories of incontinence were compared to previously used binary definitions. Incontinence surgical outcomes were analyzed for those with data at least three months post-operatively. Chi-square tests evaluated associations among categorical variables. Univariate and ordinal logistic regression models were used to test associations of ordinal incontinence status with patient and condition factors. Statistical tests were 2-sided; p values < 0.05 were considered significant. RESULTS: Analysis of 7217 individuals using ordinal incontinence outcomes showed little difference from previously used binary outcomes. The final multivariable logistic regression models with ordinal multinomial outcomes showed that associations of incontinence with age, sex, race/ethnicity, health insurance, level of lesion, and continence management technique were similar to prior studies. Among those reporting never being incontinent of both bladder and bowel, 14% reported using protective undergarments. Of the 500 individuals who had bladder outlet surgery, 38% reported never being incontinent of urine. Of 1416 individuals who had appendicostomy (ACE) bowel surgery, 48% reported never being incontinent of stool. DISCUSSION: Our current analysis showed that ordinal continence outcome classification had similar continence findings as previous studies using the binary definition of continence. Expanding the binary definition of continence to include monthly episodes of incontinence did not greatly increase the proportion of continent individuals and, therefore, would have not likely made meaningful differences in continence outcomes in prior NSBPR analyses. However, it is known that even mild incontinence can affect quality of life, therefore, capturing any level of incontiennce is of clinical importance. Confirmation of the association of continence outcomes with sociodemographic, condition-related, and interventional factors with both approaches further validates previous analyses using the binary definition of continence. CONCLUSION: The previously used binary definition of bladder and bowel continence appears robust. Undergarment choice was a poor surrogate for reported incontinence. After bladder and bowel continence surgeries, 38% and 48%, respectively, reported never being incontinent.

Testing the feasibility of a National Spina Bifida Patient Registry.

BACKGROUND: The purpose of this study was to describe the development and early implementation of a national spina bifida (SB) patient registry, the goal of which is to monitor the health status, clinical care, and outcomes of people with SB by collecting and analyzing patient data from comprehensive SB clinics. METHODS: Using a web-based, SB-specific electronic medical record, 10 SB clinics collected health-related information for patients diagnosed with myelomeningocele, lipomyelomeningocele, fatty filum, or meningocele. This information was compiled and de-identified for transmission to the Centers for Disease Control and Prevention (CDC) for quality control and analysis. RESULTS: A total of 2070 patients were enrolled from 2009 through 2011: 84.9% were younger than 18 years of age; 1095 were women; 64.2% were non-Hispanic white; 6.5% were non-Hispanic black or African American; and 24.2% were Hispanic or Latino. Myelomeningocele was the most common diagnosis (81.5%). CONCLUSIONS: The creation of a National Spina Bifida Patient Registry partnership between the CDC and SB clinics has been feasible. Through planned longitudinal data collection and the inclusion of additional clinics, the data generated by the registry will become more robust and representative of the population of patients attending SB clinics in the United States and will allow for the investigation of patient outcomes.

Global health opportunities within pediatric subspecialty fellowship training programs: surveying the virtual landscape.

BACKGROUND: There is growing interest in global health among medical trainees. Medical schools and residencies are responding to this trend by offering global health opportunities within their programs. Among United States (US) graduating pediatric residents, 40% choose to subspecialize after residency training. There is limited data, however, regarding global health opportunities within traditional post-residency, subspecialty fellowship training programs. The objectives of this study were to explore the availability and type of global health opportunities within Accreditation Council for Graduate Medical Education (ACGME)-accredited pediatric subspecialty fellowship training programs, as noted by their online report, and to document change in these opportunities over time. METHODS: The authors performed a systematic online review of ACGME-accredited fellowship training programs within a convenience sample of six US pediatric subspecialties. Utilizing two data sources, the American Medical Association-Fellowship and Residency Electronic Interactive Database Access (AMA-FREIDA) and individual program websites, all programs were coded for global health opportunities and opportunity types were stratified into predefined categories. Comparisons were made between 2008 and 2011 using Fisher exact test. All analyses were conducted using SAS Software v. 9.3 (SAS Institute Inc., Cary, NC). RESULTS: Of the 355 and 360 programs reviewed in 2008 and 2011 respectively, there was an increase in total number of programs listing global health opportunities on AMA-FREIDA (16% to 23%, p=0.02) and on individual program websites (8% to 16%, p=0.004). Nearly all subspecialties had an increased percentage of programs offering global health opportunities on both data sources; although only critical care experienced a significant increase (p=0.04, AMA-FREIDA). The types of opportunities differed across all subspecialties. CONCLUSIONS: Global health opportunities among ACGME-accredited pediatric subspecialty fellowship programs are limited, but increasing as noted by their online report. The availability and types of these opportunities differ by pediatric subspecialty.

International collaborations on advocacy, education, and research: Broad-spectrum thematic diversity catalyzes multidisciplinary spina bifida care.

As with the wide range in spina bifida (SB) incidence rates across nations, there is also wide variance in topics encountered by clinicians today. Both the wide variance in SB incidence rates and the wide diversity of topics to be addressed provide the backdrop for any dialogue among professionals serving this population. On the international stage, the World Congress on Spina Bifida Research and Care has been the only conference dedicated solely to research, practical challenges, and real-life solutions for those living with SB, their families, and caregivers. As a conference with a clear sense of the growing global village, the 2023 congress showcased innovative research from junior to preeminent investigators. Topical areas included urology, neurosurgery, global health, prenatal surgery, and transition to adult care amid others. Ultimately, through the dissemination of a compendium of conference abstracts, we hope that professionals will be aided and inspired to continue to improve the education, advocacy, and care among the many communities of individuals affected by SB globally.

From prenatal care to spina bifida related mortality: The lifespan is marked by transitions experienced by increasing immigrant and international populations.

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.

Neurodevelopmental Implications on Urological Self-management Among People Living With Spina Bifida: A Practical Guide for Urology Providers.

Although folic acid fortification and advances in prenatal repair have reduced Spina Bifida (SB) prevalence and the severity of comorbidities, individuals with SB remain at elevated risk for neurocognitive impairments that studies have shown can negatively impact, among other things, urological self-care. Identifying and addressing these impairments with practical interventions can meaningfully improve long-term outcomes for individuals with SB. We review neurocognitive impairments associated with SB and provide practical solutions to support improvement of long-term urological outcomes.

The impact of a health care transition clinic on spina bifida condition management and transition planning.

BACKGROUND: To better serve the growing population of individuals with spina bifida (SB) living into adulthood, pediatric SB clinics have developed structured health care transition (HCT) supports for adolescents and young adults. Evaluating the impact of structured HCT on SB-related chronic condition outcomes and transition planning goals is needed to assess such interventions. OBJECTIVE: This study explored the impact of a SB HCT Clinic on SB-related chronic condition management outcomes (e.g., reported bowel and bladder regimens and presence of pressure injury) and transition planning goals (e.g., decision-making, insurance, and transportation). METHODS: A retrospective chart review was conducted of young adults with SB who did and did not participate in an SB HCT Clinic before establishing an adult clinic to compare SB-condition outcomes and HCT planning goals between groups. Associations between demographic and clinical variables and outcomes were also assessed. RESULTS: The HCT group (n = 68) was more likely to use a bowel regimen (P < 0.01) compared to the non-HCT group (n = 94). There were no differences regarding bladder regimens or incidence of pressure injuries. For HCT planning, the groups differed regarding decision-making supports (P = 0.01). Additionally, the HCT group was more likely to use self-transportation (P = 0.01) or Medicaid transportation (P < 0.01). CONCLUSION: This single-center HCT program improved the use of a bowel regimen at the time of transfer to adult care and impacted HCT planning regarding decision-making and transportation. These initial findings support the need for further development and assessment of HCT programs for this population.

Addressing social determinants of health through customization: Quality improvement, telemedicine, and care coordination to serve immigrant families.

PURPOSE: The purpose of this project was to establish a pathway for electronic medical record (EMR) customization, utilizing quality improvement methodology, to both identify and address adverse social determinants of health (SDOH) among a diverse spina bifida (SB) population. METHODS: Starting in September 2020, the four fundamental steps were to (1) facilitate an advisory committee to safeguard the standard clinical protocols, (2) characterize barriers to implementation, (3) evaluate workflow to sustain data entry capture, and (4) manage the technology platform for seamless integration. The SB clinic was the first clinic within the enterprise to rollout the use of an adverse SDOH mitigation activity. A Spanish-speaking interpreter was scheduled for all clinics, as many families were limited in English proficiency. RESULTS: The customization of the EMR to support an efficient workflow to address SDOH was feasible in a large and diverse urban medical center. Of the 758 patients served in the clinic, a myelomeningocele diagnosis was present in 86% of individuals. While 52% of participants were female, ethnically 52% of individuals served were Latino. Many of these individuals disclosed being recent immigrants to the United States. Often immigration and asylum related issues were at the forefront of the SDOH issues addressed. CONCLUSION: Given the occurrence of adverse SDOH among individuals with SB, many of whom are new Latin-American immigrants, meaningful clinical efforts are needed to both identify and address the causes of the observed disparities. EMR customization is feasible and can identify and, through social prescriptions, address SDOH to support the provision of safe, high quality, and equitable care for vulnerable and medically complex populations at home and potentially abroad.

Age-Specific Probability of 4 Major Health Outcomes in Children with Spina Bifida.

OBJECTIVE: This study aimed to estimate the age-specific probability of 4 health outcomes in a large registry of individuals with spina bifida (SB). METHODS: The association between age and 4 health outcomes was examined in individuals with myelomeningocele (MMC, n = 5627) and non-myelomeningocele (NMMC, n = 1442) from the National Spina Bifida Patient Registry. Sixteen age categories were created, 1 for each year between the ages of 5 and 19 years and 1 for those aged 20 years or older. Generalized linear models were used to calculate the adjusted probability and 95% prediction intervals of each outcome for each age category, adjusting for sex and race/ethnicity. RESULTS: For the MMC and NMMC groups, the adjusted coefficients for the correlation between age and the probability of each outcome were -0.933 and -0.657 for bladder incontinence, -0.922 and -0.773 for bowel incontinence, 0.942 and 0.382 for skin breakdown, and 0.809 and 0.619 for lack of ambulation, respectively. CONCLUSION: In individuals with SB, age is inversely associated with the probability of bladder and bowel incontinence and directly associated with the probability of skin breakdown and lack of ambulation. The estimated age-specific probabilities of each outcome can help SB clinicians estimate the expected proportion of patients with the outcome at specific ages and explain the probability of the occurrence of these outcomes to patients and their families.

Spina Bifida Global Learning Collaborative: Educating the next generation of clinicians, researchers, and advocates.

PURPOSE: This project aimed to launch an international learning community to guide the development of a spina bifida (SB) curriculum for global health trainees. METHODS: Using a descriptive study design, a convenience sample of SB curricula were identified in 2022-23 by members of the Spina Bifida World Congress Outreach Committee and evaluated during a series of monthly Zoom calls to discuss SB education in a global health context. Participants included (1) leadership from the ReachAnother Foundation, (2) invited panelists from the Spina Bifida World Congress Global Health Symposium, and (3) global health students and preceptors. Education initiatives in Ethiopia, Sweden, Argentina, Ecuador, and the United States were evaluated vis-à-vis format and content. RESULTS: All of the education initiatives referenced the framework of the World Health Organization International Classification of Functioning, Disability and Health. Formats varied and included both virtual and interactive workshops, print materials, videos, and guides for small group discussion. Content addressed four domains: Folate Prevention, Neurosurgical Training, After Care, and Data Collection. A multidisciplinary approach, partnerships with families, and workforce pipeline training were identified as guiding themes for educating the next generation of SB researchers and clinicians in global health settings. CONCLUSION: The Spina Bifida Global Learning Collaborative is a transnational group of advocates, clinicians, and investigators whose mission is the advancement of SB-related global health education. Lessons learned from the collaborative are being leveraged to develop a global health curriculum for learners, which may improve services for individuals with SB around the globe.

Causes of death among people with myelomeningocele: A multi-institutional 47-year retrospective study.

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%). For 261 shunted individuals, leading COD were neurological (21%) and pulmonary (17%), and leading MOD were infections (34%, including shunt infections: 4%) and non-infectious shunt malfunctions (14%). For 32 unshunted individuals, leading COD were pulmonary (34%) and cardiovascular (13%), and leading MOD were infections (38%) and non-infectious pulmonary (16%). COD and MOD varied by shunt status and age (p < = 0.04), not ambulation or birthyear (p > = 0.16). Urology-related deaths (urosepsis, renal failure, hematuria, bladder perforation/cancer: 10%) were more likely in females (p = 0.01), independent of age, shunt, or ambulatory status (p > = 0.40). COD/MOD were independent of bladder augmentation (p = >0.11). Unexplained deaths while asleep (4%) were independent of age, shunt status, and epilepsy (p >= 0.47). CONCLUSION: COD varied by shunt status. Leading MOD were infectious. Urology-related deaths (10%) were independent of shunt status; 26% of COD were unknown. Life-long multidisciplinary care and accurate mortality documentation are needed.

From autism to zoom®: Spina bifida advocacy, care, education, and research in a changing word.

Whereas legislation mandates for folic acid fortification have been implemented throughout many nations, divergent neural tube defects (NTDs) prevalence rates still remain among the world's populations. In North America, the prevalence estimate is 39 infants per 100,000 live births. Open spina bifida (SB), also known as myelomeningocele, remains the most complex congenital abnormality of the central nervous system compatible with long term survival; this recognized complexity gives rise to emerging comorbidities and interventions. For example, increasing autism spectrum disorder rates have been reported among individuals with SB utilizing a 31,220 subject population-based birth cohort. Along with new clinical observations, telecommunication platforms such as Zoom® have evolved as clinical and investigational tools. Historically, society meetings, research conferences, and journals have provided opportunities for professional development and dissemination of up-to-date materials. The Journal of Pediatric Rehabilitation Medicine (JPRM) has arisen as an open-access global platform for the dissemination of SB-related inquiry. The journal has also highlighted the research presented at the Spina Bifida Association's previous Spina Bifida World Congresses. At the last congress, which was held in 2017, twenty-three countries were represented; this number is expected to grow by the next convocation in 2023. This congress will provide an opportunity for health care professionals from around the globe to present a broad array of research topics and build collaborations. Concurrently, the JPRM will continue as an open-access platform for SB advocacy, care, education, and investigation, across our fast changing world for the international SB community well into the future.

Team Approach: The Management of Adolescents and Adults with Scoliosis and Spina Bifida.

¼: Globally, the prevalence of myelomeningocele, the most common subtype of spina bifida, is 0.2 to 6.5 per 1,000 live births. In the U.S., adults account for >67% of the overall population with spina bifida. ¼: With an estimated prevalence of up to 50%, scoliosis is one of the most common and severe orthopaedic conditions in patients with myelomeningocele. ¼: The variable effects that scoliosis can have on an individual, the comorbidities associated with progressive scoliosis, and the risks associated with spine surgery call for a strong partnership and care coordination between medical and surgical teams to deliver a patient-centered approach. ¼: A coordinated, structured, planned, and incremental team approach can help individuals achieve the overall goals of functionality and independence, as well as successful transition to adulthood. ¼: Teams should consider a patient's social determinants of health (e.g., poverty or language barriers) and the effect of scoliosis on quality of life before proceeding with spinal deformity correction.

Association between age of starting clean intermittent catheterization and current urinary continence in individuals with myelomeningocele.

INTRODUCTION: Patients with myelomeningocele often use clean intermittent catheterization (CIC) for renal preservation and to promote urinary continence. While starting CIC at an early age is associated with better renal outcomes, the impact of age of CIC initiation on continence outcomes has not been examined. OBJECTIVE: To examine whether earlier CIC initiation is associated with higher likelihood of current urinary continence for patients with myelomeningocele. STUDY DESIGN: Data of patients aged ≥5 years at last visit were obtained from 35 spina bifida clinics participating in the National Spina Bifida Patient Registry from 2013 to 2018. Sociodemographic characteristics, disease characteristics, and current bladder management strategies were collected. Via univariate and multiple logistic regression models, the latter conducted controlling for all variables associated with current continent status, associations between continence and sociodemographic factors, condition characteristics, and age CIC began (<3 years of age, 3-5 years, 6-11 years, ≥12 years) were analyzed. RESULTS: Data from 3510 individuals were included (mean age at last visit = 17.0 years, range 5.0-88.7). The sample was evenly distributed by sex (52% female); most individuals were non-Hispanic White (62.6%). The majority of patients (55.2%) started CIC before age 3 years. Continence varied markedly across those who never started CIC (0.6% of patients were continent) and those who started at any age (range 35.3-38.5%). Among those who started CIC, the magnitude of the association was not proportional to age CIC was started. Compared with those who started CIC at age 12 or older, estimated adjusted odds ratio of being continent ranged from 1.04 (6-11 years, 95% CI, 0.72-1.52) to 1.25 (<3 years, 95% CI, 0.89-1.76). DISCUSSION: Although CIC may be positively associated with achieving urinary continence in individuals with myelomeningocele, we could not demonstrate that younger age at CIC initiation increased the likelihood of achieving this goal. Limitations include lack of data on reason for starting CIC, urodynamic data, and the observational nature of data collection. CONCLUSIONS: Further study is needed addressing limitations of the current investigation to determine if urinary continence outcomes are influenced by the age of starting CIC among patients with myelomeningocele.