Multidisciplinary management of people with spina bifida across the lifespan.

The average worldwide prevalence of neural tube defects (NTDs) is 1.0 per 1000 births. Its development is multifactorial due to genetic and non-genetic factors. Spina bifida (SB) is one of main representatives of NTD. The spinal cord lesion level is the main determinant of the level of paralysis, numbness, and difficulties with bladder/bowel functions. Myelomeningocele prenatal repair reduces hydrocephalus and hindbrain herniation and improves motor function. The severity of hydrocephalus is associated with poorer neurodevelopmental outcomes whether operated on prenatally or after birth. People with SB tend to have a lower IQ and cognitive difficulties. Early diagnosis, proactivity, and lifelong multidisciplinary follow-up are key protective issues. Invasive urological interventions should be considered in selected patients after failure of conservative treatment. Transition to adult care should be well planned as it is challenging. Health literacy is directly associated with success at transition. Sexuality and fertility should be addressed before/during puberty. Overall, the rates of fecal and urinary continence and skin breakdown increase with age, whereas the ability to ambulate declines with age. Bowel and urinary incontinence are independent predictors of lower health-related quality of life (HRQoL) in adults with SB. Bowel incontinence has negative impact on HRQoL regardless of frequency or amount. Long-term caregiver support should be offered at diagnosis. Survival at a mean of 50 years is poor, at 32%, due to central nervous system deaths, cancer, urological disease, and sepsis. Challenges to implementation of recommended practices exist, especially in low and middle-income countries.

The urological evaluation and management of neurogenic bladder in children and adolescents-what every pediatric nephrologist needs to know.

Neurogenic bladder (NGB) is an encompassing term that includes multiple causes of bladder dysfunction linked to a congenital or acquired neurological condition that adversely impacts the innervation of the lower urinary tract. Multiple static or progressive conditions can be associated with NGB in pediatric and adolescent patients. Currently, spinal dysraphism (i.e., spina bifida) is one of the most common etiologies, which occurs in 3-4 per 10,000 live births in developed nations. Abnormal bladder dynamics can lead to kidney damage secondary to high pressures or recurrent infections, as well as urinary incontinence. The current management paradigm centers on a proactive approach to preserving kidney function and achieving continence through behavioral, pharmacological, and surgical means. This educational review highlights the key components of urological management to maximize collaboration with pediatric nephrologists.

Trajectories of self-management and independence in youth with spina bifida: Family-related predictors of growth.

The purpose of this study was to assess family-related predictors of self-management trajectories in youth with spina bifida (SB). Participants with SB completed the Adolescent/Young Adult Self-Management and Independence Scale (AMIS II) interview across four time points. Family functioning, family-related stress, and perceived family support were assessed by multiple reporters and multiple methods. Growth in AMIS II total self-management and the AMIS II subscales (Condition and Independent Living) were estimated using linear mixed effect models as a function of family factors, after controlling for socio-demographic, condition-related, and neuropsychological variables that had been found to be significant predictors of self-management in prior studies. Model fit and parsimony were assessed using Akaike's information criterion (AIC). This diverse community sample included 99 respondents aged 18-27 years old. About half were female (52.5%) and White (52.5%); 15.2% were Black, and 32.3% were Hispanic/Latino. Observed family cohesion at baseline was associated with all self-management scales at age 18 (all p < 0.05). Growth in self-management was associated with parent-reported number of family stress events. For growth in total self-management, the best model included age, race/ethnicity, family income, shunt status, lesion level, neuropsychological function, observed family cohesion, and an age-by-number of family stress events interaction effect. The study findings suggested that family factors were important predictors of self-management trajectories, even after controlling for socio-demographic, condition-related, and neuropsychological covariates. Risk and protective factors identified in families of youth with SB can inform family-focused interventions for self-management.

Improving Bowel Management in Children With Spina Bifida.

OBJECTIVES: In individuals with spina bifida (SB), bowel incontinence is associated with lower quality of life and lower likelihood of employment. In an effort to maximize bowel continence in children and adolescents, we created a bowel management assessment and follow-up protocol in a multidisciplinary clinic. Here we report the results of this protocol using quality-improvement methodology. METHODS: Continence was defined as no unplanned bowel movements. Our protocol involved: (1) a standardized 4-item questionnaire about bowel continence and consistency; (2) if the patient was not achieving continence, an intervention starting with oral medication (stimulant and/or osmotic laxatives), and/or suppositories (glycerin or bisacodyl) followed by an escalation to trans-anal irrigation, or continence surgery; and (3) follow-up phone calls at regular intervals to monitor progress and make changes as needed. Results are summarized with descriptive statistics. RESULTS: We screened 178 eligible patients in the SB clinic. Eighty-eight agreed to participate in the bowel management program. Of those who did not participate, the majority (68/90, 76%) were already achieving continence with their bowel regimen. Of children in the program, most (68/88, 77%) had a diagnosis of meningomyelocoele. At 1 year, the proportion of patients who were bowel accident free improved to 46% (vs 22% initially, P = 0.0007). CONCLUSIONS: A standardized bowel management protocol, primarily the use of suppositories and trans-anal irrigation to achieve social continence, as well as frequent telephone follow-up, can reduce bowel incontinence in children and adolescents with SB.

Transanal irrigation is a better choice for bowel dysfunction in adults with Spina bifida: A randomised controlled trial.

AIM: To compare transanal irrigation with conservative bowel management for the treatment of bowel dysfunction in Spina bifida (SB) patients. METHODS: Patients with SB and bowel dysfunction were randomly assigned to receive either transanal irrigation or conservative bowel management. The effectiveness of the treatment was defined as a decrease of 4 points in the neurogenic bowel dysfunction (NBD) score at week 10. Data on incontinence (Cleveland scores; Jorge-Wexner [JW]) and constipation (Knowles-Eccersley-Scott Symptom Constipation Score [KESS]) were recorded at 10 and 24 weeks after inclusion. Data were analysed on an intention-to-treat basis. RESULTS: A total of 34 patients were randomised: 16 patients to conservative bowel management and 18 patients to transanal irrigation. A total of 19/31 (61%) patients improved at week 10, 13 (76%) in the transanal irrigation group versus six (43%) in the conservative group (p = 0.056). In the irrigation group, the decrease in NBD score was -6.9 (-9.9 to -4.02) versus -1.9 (-6.5 to -2.8) in the conservative group (p = 0.049 in univariate and p = 0.004 in multivariate analysis). The NBD, Cleveland (JW and KESS) and Rosenberg scores were significantly lower in the transanal irrigation group than in the conservative bowel management group at week 10. CONCLUSIONS: This prospective, randomised, controlled, multicentre study in adult patients with SB suggests that transanal irrigation may be more effective than conservative bowel management.

Self-Management in Youth With Spina Bifida: Associations With Parent Factors in the Context of a Summer Camp Intervention.

OBJECTIVE: To investigate cross-sectional and longitudinal associations between parent factors and self-management for youth with spina bifida (SB). METHODS: Participants were 89 camper-parent dyads recruited for a summer camp program for youth with SB (Myouthage = 12.2 years); 48 of these families participated across 2 years. Campers and parents completed assessments at Time 1 (pre-camp) and Time 3 (post-camp) for one or two summers. Parents reported on demographics, their own adjustment, perceptions, attitudes, and behaviors, and youth condition-related responsibility and task mastery. Youth also reported on condition-related responsibility. Hierarchical multiple regression analyses and multilevel modeling were used to examine relationships between parent factors and youth self-management. RESULTS: Parents' expectations for future goal attainment were positively associated with camper responsibility and task mastery, and these associations were moderated by camper age (only significant for older campers). When examining changes over one summer, parental expectations for the future were significantly associated with changes in campers' condition-related task mastery. When examining trajectories across summers, parental perception of child vulnerability was negatively associated with the slope of condition-related responsibility and parents' expectations for future goal attainment were positively associated with the slope of task mastery. CONCLUSIONS: Parent perceptions and behaviors may be important targets for assessment and intervention when promoting condition-related independence for youth with SB.

Spina bifida management in low- and middle-income countries - a comprehensive policy approach.

INTRODUCTION: Globally, spina bifida (SB) occurs more often in low- and middle-income countries, where the healthcare demands are often quite challenging. Several social/societal issues and/or lack of government support makes for incomplete SB management in many areas. Clearly, neurosurgeons should be knowledgeable about initial closure techniques and the basics of SB management, but must also advocate for the patients outside our immediate scope of care. METHODS: Recently, the Comprehensive Policy Recommendations for the Management of Spina Bifida and Hydrocephalus in Low- and Middle-Income Countries (CHYSPR) and the Intersectoral Global Action Plan on Epilepsy and other Neurological Disorders (IGAP) publications emphasized the need for a more unified approach to SB care. Although both documents discuss other neurological conditions, they support SB as a congenital malformation needing attention. RESULTS: We identified several similarities for comprehensive SB care in these approaches - including education, governance, advocacy, and the need for continuum of care. Prevention was recognized as the most important aspect for SB going forward. A significant return of investment was noted, and both documents recommend more active neurosurgical involvement (i.e., folic acid fortification). CONCLUSION: A new call for holistic and comprehensive care for SB management is recognized. Neurosurgeons are called upon to use solid science to educate governments and actively participate to advocate for better care and most importantly, prevention. Folic acid fortification schemes are mandatory and neurosurgeons should advocate for global strategies.

Transitioning care for adolescents with spina bifida in the US: challenges for management.

Open spina bifida (OSB) is a common neural tube defect. Medical and surgical care involves addressing the baseline orthopedic, urologic, and neurological dysfunction as well as the changes or declines that may occur as the patient ages. Given the complexity of this disease, coordinated, multidisciplinary care involving specialists in neurosurgery, orthopedics, urology, rehabilitation and physical medicine, pediatrics, and psychology is necessary to establish and optimize baseline function. Traditionally in the US, pediatric multispecialty spina bifida clinics have provided the patient with a coordinated medical support system. Unfortunately, this coordinated, medical home has been difficult to establish during the transition from pediatric to adult care. Medical professionals must have a strong understanding of OSB to properly manage the disease and detect and prevent associated complications. In this manuscript, we (1) describe the changing needs and challenges of people living with OSB over a lifespan, (2) delineate current practices in the transition of care for people with OSB from childhood to adulthood, and (3) provide recommendations for best practices in navigating the transition process for clinicians who provide care for those afflicted with this most complex congenital abnormality of the nervous system compatible with long term survival.

Trajectories of self-management and independence in youth with spina bifida: Demographic predictors of growth.

AIM: The purpose of this study was to examine the trajectories of condition and independent living self-management in youth with spina bifida (SB). METHODS: A diverse sample of adolescents and young adults (AYAs) with SB completed the Adolescent/Young Adult Self-Management and Independence Scale (AMIS-II) across four time points. Parents reported on demographic characteristics including age, sex, race/ethnicity, and family income. Growth in self-management and its subscales (condition and independent living) were estimated using linear mixed-effect models as a function of respondents' demographics. RESULTS: This study included 99 respondents age 18 to 27 years old. About half were female (52.5%) and White (52.5%); 15.2% were Black, and about a third were Hispanic/Latino (32.3%). Eighty-seven AYAs (87.9%) had myelomeningocele. The lesion level was 31.3% sacral, 48.5% lumbar and 18.2% thoracic. A third of the families earned less than 50K. Overall, self-management growth was dependent on age, sex, and race/ethnicity, but not income. Growth in condition self-management depended on sex; only males demonstrated increasing growth ( ß Ì‚ = 0.11, p < 0.001). Black participants endorsed higher increasing total and condition self-management when compared with White ( ß Ì‚ diff = 0.17 and 0.17, respectively, both p < 0.05) and Hispanic/Latino ( ß Ì‚ diff = 0.18 and 0.21, respectively, both p = 0.02) respondents. CONCLUSION: This study provides evidence of differences in growth of self-management by demographic/social determinants of health. Possible reasons for differences are discussed. Predictors of changes in self-management behaviours over time in young adults with SB can identify subgroups in need of further study.

A multinational survey on the management of the urinary tract in newborns with spina bifida: Are we following current EAU/ESPU guidelines?

AIMS: In August 2019, the European Association of Urology (EAU) and European Society for Paediatric Urology (ESPU) published updated guidelines on the management of neurogenic bladder in children and adolescents. Our study aimed to establish whether members of the ESPU are adhering to these guidelines. METHODS: We designed a survey comprising 26 questions using SurveyMonkey®. Respondents were asked about management of neurogenic bladder at birth in newborns with spina bifida (SB), urological investigations, as well as short and long-term follow-up in their institutions. RESULTS: There were 103 respondents to the survey (754 recipients, giving a response rate of 14%) spanning 36 countries. 100% of respondents carry out a renal/bladder ultrasound at birth. Only 53% routinely commence clean intermittent catheterization soon after birth as recommended by the guidelines. Only 56% recommend anticholinergic medications after abnormal videourodynamics (VUDs). The guidelines recommend the use of continued antibiotic prophylaxis if there is evidence of vesicoureteral reflux and hostile bladder/non-conclusive results on VUDs which is followed by only 30% of providers. 63% of respondents carry out baseline VUDs at the recommended time. Seeing larger volumes of SB patients, having a formal SB protocol, having formal SB multidisciplinary clinics and working in a tertiary referral center did not make respondents more likely to adhere to guidelines. CONCLUSIONS: Our survey demonstrated that large variations from the EAU/ESPU guidelines exist in practice. The study confirms that further work is required across institutions and countries to implement these evidence-based recommendations for standardized practice.

Amniotic fluid-derived stem cell potential for therapeutic and surgical use: A review of the literature.

OBJECTIVE: Spina bifida is a debilitating neutral tube defect affecting many infants. The impact and severity of spina bifida depends on whether the patient exhibits a closed defect, spina bifida occulta, or an open defect, spina bifida aperta. Patients with spina bifida have physical and mental disabilities which merit further research into less invasive, more successful treatments. In addition to serving as protection for the growing fetus and facilitating nutrient exchange, amniotic fluid (AF) is a rich source of a mixed population of stem cells. As such, in vitro culture of AF-derived stem cells has shown promise among therapeutic and surgical applications. We present a critical evaluation of the current preclinical efforts, amniotic fluid-derived stem cell (AFSC) culture process, and the subsequent therapeutic application, with a focus on improvements for spina bifida outcomes in the pediatric patient population. METHOD: An evidence - based literature review to investigate the current literature surrounding AFSC culture and use, with an emphasis on the benefits for spina bifida treatment. RESULTS: 47 literature sources from PubMed and three studies from ClinicalTrials.gov. CONCLUSION: This review synthesizes the current literature, which shows promising data on AFSC pluripotency, as well as successful in utero coverage from AFSC - supported environments in a multitude of animal models.

Investigating the landscape and trajectory of spina bifida research in Asia: a bibliometric analysis.

BACKGROUND: Spina bifida is a type of a neural tube defect which affects 243.14 per 100,000 babies in Asia. Research articles on spina bifida have increased in the recent years. However, no study has focused on the research trends in this field in Asia. METHODS: A systematic review of literature on spina bifida in Asia was performed using the Scopus database from inception to 2020. All published studies on spina bifida conducted in or published by authors from Asia were included in our analysis. Bibliometric information was obtained from Scopus and bibliometrics diagrams were created using VOSviewer software. RESULTS: A total of 652 articles were obtained in this study. The number of publications showed an upward trend starting 2000s. The country with the greatest number of publications was Japan while All India Institute of Medical Sciences was the most productive institution in spina bifida research in Asia. The current focus of this field in Asia was prevalence of spina bifida, prenatal diagnosis, folic acid supplementation, and complications of spina bifida. Future areas of research in spina bifida include the genetic basis of neural tube defects and the use of stem cell technology as therapies for spina bifida. CONCLUSION: This is the first bibliometric analysis on spina bifida in Asia. It showed the trend and future areas of research on spina bifida in Asia. Despite the increase in scientific literature on spina bifida research, more research outputs and collaborations are needed especially in developing countries in Asia.

The impact of constant antibiotic prophylaxis in children affected by spinal dysraphism performing clean intermittent catheterization: a 2-year monocentric retrospective analysis.

INTRODUCTION: Spinal dysraphism (SD) is a general term used to refer to developmental abnormalities of the spine that involves many clinical conditions including myelomeningocele (MMC). In these patients, neurogenic bladder (NB) is a common and predisposing factor for renal damage; the most frequently used approach to manage this situation is based on clean intermittent catheterization (CIC) and anticholinergic drugs. Urinary tract infections (UTIs) are a significant concern for these patients, and antibiotic prophylaxis is frequently used even if it is still a debated topic of literature. The purpose of this paper is to investigate the role and the real effectiveness of antibiotic prophylaxis in the reduction of incidence of UTIs in patients with spina bifida performing CIC. METHODS: We collected data of all patients performing CIC, who did their last follow-up visit in the period between January 2019 and January 2021, followed at the children multidisciplinary Spina Bifida Center of A. Gemelli Hospital in Rome. Data collected included age at referral, gender, type of SD lesion, serum creatinine and cystatin C levels, the use of anticholinergic medications, antibiotic prophylaxis and type of prophylaxis (oral/endovesical), age of starting prophylaxis with its duration/adherence, number of CIC/day and its duration, episodes of UTIs in the 2 years prior to the last follow-up, and presence and grade of vesical-ureteric reflux (VUR) on cystourethrogram. RESULTS: A total of 121 patients with SD performing CIC was included in the study; 66 (54%) presented ≥ 1 episode of UTIs in the last two years and 55 (46%) none. During the study period, 85 (70%) patients received antibiotic prophylaxis (ABP group) and 36 (30%) did not (NABP group): no statistically significative difference in terms of UTI development was observed between the two groups (p = 0.17). We also evaluated compliance to the therapy; 71 patients (59%) took antibiotic prophylaxis constantly (CABP group) and 50 (41%) did not do antibiotic prophylaxis constantly or did not do antibiotic prophylaxis at all (NCABP group): we observed a statistically significative difference in terms of UTIs with a 2.2 times higher risk of development at least one episode of UTIs in NCABP group. CONCLUSION: In conclusion, antibiotic prophylaxis performed constantly, without interruption, is associated with a lower risk of developing urinary tract infections and consequently to develop renal failure in adulthood.

The importance of dedicated colorectal team participation in the management of spina bifida and spinal cord injury patients.

PURPOSE: In September 2020, the colorectal team of the International Center for Colorectal and Urogenital Care joined the spina bifida and spinal cord injury multidisciplinary clinic at Children's Hospital Colorado. Many important lessons were learned. METHODS: A retrospective review of patients seen in the spina bifida and spinal cord injury multidisciplinary clinic from September 2020 to May 2021 was conducted. Data collected included demographics, diagnosis, pre or post-natal repair for those with myelomeningocele, whether the patient was previously seen by the colorectal team, wheelchair usage, voluntary bowel control vs. fecal incontinence, urinary control vs. clean intermittent catheterization, characteristics of contrast enema, and our proposed intervention. RESULTS: Overall, 189 children were seen during the study period, ranging from 3 months to 20 years of age (average = 9.5 years). One hundred and two were males and 87 were females. Diagnosis included myelomeningocele (n = 153), spinal cord injury (n = 18), transverse myelitis (n = 7), sacral agenesis (n = 5), diastematomyelia (n = 2), spinal stenosis (n = 2), and tethered cord with lipoma (n = 2). Fifteen patients with myelomeningocele were repaired in-utero. One hundred and sixty patients were new to the colorectal team. Eighty-one patients were wheelchair users. One hundred and twenty-three patients suffered from fecal incontinence and needed enemas to be artificially clean for stool and thirty-eight patients had voluntary bowel movements and were clean with laxatives, suppository, or rectal stimulations. Twenty-eight patients were younger than three years of age and still in diapers. Despite a non-dilated colon on contrast enema, this population has a hypomotile colon. One hundred and twenty-eight patients required clean intermittent catheterization. CONCLUSION: Joining the spina bifida and spinal cord injury multidisciplinary clinic allowed us to better serve this population and gave us enormous satisfaction to contribute to improve the quality of life of the patients and their parents. LEVEL OF EVIDENCE: III.

[Chronic sacral neuromodulation for pelvic floor dysfunction in children with spina bifida]. / Lechenie narusheniya funktsii tazovykh organov u detei s mielodisplaziei metodom khronicheskoi sakral'noi neirostimulyatsii.

OBJECTIVE: To estimate the effectiveness of chronic sacral neurostimulation for neurogenic lower urinary tract dysfunction in children with spina bifida. MATERIAL AND METHODS: Eight patients with spina bifida and neurogenic lower urinary tract dysfunction underwent surgical treatment. Three patients had detrusor-sphincter dyssynergia and urinary incontinence. Five patients had detrusor overactivity and urine retention. Conservative treatment was failed in all cases. We implanted quadripolar test electrode on the S3 root through Tuohy needle under fluoroscopic control. Test stimulation was ineffective in three children with detrusor overactivity. Five patients with positive response to stimulation underwent implantation of chronic neurostimulation system. We estimated the efficacy of neurostimulation considering symptoms of neurogenic lower urinary tract dysfunction, urinary diary, PAD test and complex urodynamic examination data. RESULTS: Positive clinical effects were observed in 3 patients with detrusor-sphincter dyssynergia and 2 patients with detrusor overactivity. Clinical effect included improvement in urine leakage and higher bladder volume. In patients with urinary retention, we revealed voluntary urination and less number of daily catheterization procedures. In one patient, clinical effect of chronic stimulation regressed within 3 month after surgery. CONCLUSION: Preliminary results of chronic sacral neuromodulation confirmed its advisability in children with neurogenic lower urinary tract dysfunction. Patients with detrusor-sphincter dyssynergia had better results compared to those with detrusor overactivity.

Trends in Procedures to Initiate Renal Replacement Therapy among People Living with Spina Bifida.

PURPOSE: Given the increasing prevalence of chronic kidney disease in people with spina bifida, we sought to determine if this is associated with an increase in end stage kidney disease. We examined population based data to measure the frequency of procedures to establish renal replacement therapy-a marker for end stage kidney disease-among patients with spina bifida. MATERIALS AND METHODS: We used the Healthcare Cost and Utilization Project State Inpatient Database and State Ambulatory Surgery and Services Database from Florida, Kentucky, Maryland and New York (2000 to 2014), which include encounter level data. With a diagnosis code based algorithm we identified all procedural encounters made by patients with spina bifida. We determined the percentage of these encounters that were for facilitating renal replacement therapy (ie arteriovenous anastomosis, renal transplantation). We assessed for changes over time in this percentage with the Cochran-Armitage trend test. Bivariate analysis was performed using chi-square test. RESULTS: Of all procedures performed on patients with spina bifida over this time the proportion of procedures performed to establish renal replacement therapy significantly decreased in both the inpatient and outpatient settings (p=0.042 and p <0.001, respectively). People with spina bifida undergoing procedures to establish renal replacement therapy were, on average, young adults (mean age 34.5 and 36.0 years) with a high prevalence hypertension (75.8% of inpatients, 68.6% of outpatients). CONCLUSIONS: The frequency of surgeries to initiate renal replacement therapy among people with spina bifida undergoing procedures is low and is not increasing. This highlights the importance of consistent care throughout adolescence and young adulthood, and hypertension screening.

Long-term outcomes of artificial urinary sphincter in female patients with spina bifida.

AIMS: To report the long-term functional outcomes of artificial urinary sphincter (AUS) implantation in female patients with spinal dysraphism and stress urinary incontinence (SUI) related to intrinsic sphincter deficiency (ISD). METHODS: The charts of all spina bifida female patients with SUI due to ISD who underwent AUS (AMS 800) implantation between 2005 and 2019 at three academic departments of urology were retrospectively reviewed. Reoperation was defined as either revision or explantation of the AUS device. Reoperation-free survival of the AUS device was estimated using the Kaplan-Meier method. Continence status as per patients' subjective assessment was categorized as follows: complete continence (no pads), improved continence, unchanged SUI or worsened SUI. RESULTS: Twenty-three patients were included, 69.6% were self-catheterizing. The median follow-up was 14 years. Median time to first reoperation was 10 years. Survival rates without reoperation were 85.9%, 41.8%, 34.6%, and 20.9% at 5, 10, 15, 20 years, respectively. Survival rates without AUS explantation were 90.7%, 66.3%, 55.2%, and 41.4% at 5, 10, 15, 20 years, respectively. None of the patients who underwent device explantation had a new AUS implanted. The only predictive factor of reoperation-free survival was the type of spinal dysraphism (hazards ratio = 3.60 for closed vs. open dysraphism; p = .04). At last follow-up, 17 of the 23 patients were fully continent (73.9%). CONCLUSION: AUS in female patients with spina bifida may be associated with satisfactory long-term functional outcomes and a high reoperation rate. The median time to first reoperation was similar to what is reported in the male AUS literature (10 years).

The Transition to Adult Health Care in Youth With Spina Bifida: Theory, Measurement, and Interventions.

PURPOSE: This article focuses on the transition to adult health care in youth with spina bifida (SB) from the perspective of theory, measurement, and interventions. METHODS: The purpose of this article is to discuss (a) a theory of linkages between the transfer of medical responsibility from parent to child and the transition from pediatric to adult health care, as mediated by transition readiness; (b) measurement issues in the study of self-management and the transition to adult health care; and (c) U.S.-based and international interventions focused on the transition to adult health care in young adults with SB. FINDINGS: Individuals with SB must adhere to a complex multicomponent treatment regimen while at the same time managing a unique array of cognitive and psychosocial challenges and comorbidities that hinder self-management, medical adherence, and the transition to adult health care. Moreover, such youth endure multiple transitions to adult health care (e.g., in the areas of urology, orthopedics, neurosurgery, and primary care) that may unfold across different time frames. Finally, three transition-related constructs need to be assessed, namely, transition readiness, transition completion, and transition success. CONCLUSIONS: SB provides an important exemplar that highlights the complexities of conducting research on the transition to adult health care in youth with chronic health conditions. Many transition trajectories are possible, depending on the functioning level of the child and a host of other factors. Also, no single transition pathway is optimal for all patients with SB. CLINICAL RELEVANCE: The success of the process by which a child with SB transitions from pediatric to adult health care can have life-sustaining implications for the patient.

Discrepancies in Educational Needs for Transition in Adolescents and Young Adults with Spina Bifida in South Korea: Use of the Borich Needs Assessment Model.

PURPOSE: Transition is an important goal for ensuring that adolescents and young adults (AYAs) with spina bifida (SB) lead autonomous lives. This study aimed to identify the educational needs of AYAs with SB based on the discrepancies between perceived importance and proficiency levels during the transition process. DESIGN AND METHODS: A cross-sectional study was conducted through face-to-face and online surveys from Jan-Dec 2020 of AYAs aged 13-25 years who had previously been diagnosed with SB. The survey consisted of 37 transition-related questions, of which 11 pertained to healthcare environments and 26 pertained to transition education needs SPSS and Excel were used for statistical analysis. Transition educational needs were analyzed by the Borich Needs Assessment Model. Higher the mean weighted discrepancy scores, lower the proficiency as compared to the perceived importance, indicating that the educational needs were high. RESULTS: Overall, 108 responses were analyzed, and 56 (51.9%) AYAs were diagnosed with lipomyelomeningocele. The highest ranked educational needs were for "Health insurance system", "SB related urinary system diseases management", "SB related nervous system symptoms", and "Self-catheterization management". "The demands for 'SB related work life", "Urinary incontinence management", and "Constipation management" were significantly higher in young adults than adolescents. CONCLUSIONS: During the transition process, activities perceived as important by AYAs with SB may differ from the activities that they can actually perform proficiently. It is important to assess their needs based on these discrepancies. PRACTICE IMPLICATIONS: Transition education programs are needed that consider the individual educational needs and developmental stage-specific characteristics of AYAs with SB.

Pediatric Spinal Cord Diseases.

Spinal cord diseases in pediatric patients are highly variable in terms of presentation, pathology, and prognosis. Not only do they differ with respect to each other but so too with their adult equivalents. Some of the most common diseases are autoimmune (ie, multiple sclerosis, acute disseminated encephalomyelitis, and acute transverse myelitis), congenital (ie, dysraphism with spina bifida, split cord malformation, and tethered cord syndrome), tumor (ie, juvenile pilocytic astrocytoma, ependymoma, and hem-angioblastoma), and vascular (ie, cavernous malformations, arteriovenous malformations, and dural arteriovenous fistulas) in nature. These each require their own niche treatment paradigm and prognosis. Furthermore, presentation of different spinal cord diseases in children can be difficult to discern without epidemiologic and imaging data. Interpretation of these data is crucial to facilitating a timely and accurate diagnosis. Correspondingly, the aim of this review was to highlight the most pertinent features of the most common spinal cord diseases in the pediatric population.