Sleep-related breathing disorders in infants with spina bifida repaired prenatally and postnatally.

STUDY OBJECTIVES: Advances in prenatal repair of myelomeningocele (MMC) have improved outcomes involving different organ systems. There is limited data on respiratory outcomes following prenatal surgical repair. We hypothesize there is no difference in respiratory outcomes between spina bifida (SB) patients who have undergone prenatal versus postnatal repair. METHODS: We performed a retrospective study of 46 infants <1 year with SB seen at Children's Hospital Los Angeles from 2004-2022. Demographic data, timing of closure, neonatal course, Chiari II malformation (CIIM), ventriculoperitoneal shunt (VPS), polysomnography (PSG) results, and need for supplemental oxygen were collected. Unpaired t-test and Chi-square Test were used to analyze results. RESULTS: 31/46 had prenatal repair of MMC; average age at repair was 27 weeks post-conception (PCA). Average age at postnatal repair was 37 PCA. There was no difference in age at PSG. There was no difference in CIIM presence (p=0.61). 60% of patients with postnatal repair and 23% in the prenatal group underwent VPS placement (p=0.01).There was no difference in PSG findings between the two groups: CAI (p=0.11), OAHI (p=0.64), average SpO2 baseline (p=0.91), average SpO2 nadir (p=0.17), average PETCO2 baseline (p=0.87), and average PETCO2 maximum (p=0.54). There were no significant differences in the proportion of patients on supplemental O2 (p=0.25), CSA or OSA between groups. CONCLUSIONS: Patients with SB who've undergone closure of neural tube defect have persistent central apneas, obstructive apneas, and significant hypoxemia. There were no differences in the frequency or severity of sleep-disordered breathing in those with prenatal repair versus postnatal repair.

Utility of colonic manometry in children with Hirschsprung disease.

OBJECTIVES: Abnormal motility of the residual colon has been reported in post-pull-through Hirschsprung disease (PT-HSCR) patients with persistent defecation problems. We reviewed the role of colonic manometry (CM) in the management of defecation disorders in these patients. METHODS: We retrospectively reviewed the medical record of PT-HSCR children who underwent CM for persistent symptoms of abnormal defecation. We reviewed their clinical course and its relation to CM findings. RESULTS: Thirty PT-HSCR patients underwent CM, of which five were diagnosed with transition zone pull-through and were excluded. Of the remaining 25 patients, 16 had colonic dysmotility, 8 had normal CM, and one had colonic hypermotility. In patients with dysmotility, five responded to ongoing medical management, three required surgical intervention (ileostomy), three remained symptomatic with medical management but not yet received surgical intervention, and five were lost to follow-up. In patients with normal CM, four responded to ongoing medical therapy, two required additional surgery (antegrade enema procedure), and two were lost to follow-up. The patient with hypermotility improved with adding loperamide. CONCLUSIONS: Colonic dysmotility can occur in PT-HSCR patients with persistent defecation problems. CM was helpful in delineating the degree of colonic neuromuscular dysfunction. CM results were used in conjunction with other clinical data to determine optimal management. Our findings support that medical management should first be optimized before consideration of colonic manometry and surgical interventions.

Using a mobile health app in a weight management program for overweight and obese adolescents and young adults with spina bifida.

CONTEXT: Adolescents and young adults (AYA) with spina bifida (SB) are more susceptible to obesity due to impaired mobility. There is limited access to physical activity for this population. OBJECTIVE: The primary aim of this study was to evaluate the feasibility of a mobile health app in a weight management program for AYA with SB. This was determined by measuring program adherence, active use of the app (defined as ≥3 uses per week), and user rating of the Pt Pal™ mobile app. Secondary outcomes were changes in BMI, quality of life, and health behavior, and the number of active participants over time. METHODS: Patients from the SB Clinic of a large metropolitan hospital between the ages of 11-21 years and overweight were invited to participate. The program consisted of group nutrition sessions and an individualized exercise plan using a mobile app with coaching. Outcome measures were program adherence, changes in BMI, and validated survey responses. Descriptive statistical analysis was performed. RESULTS: Fifteen participants enrolled, and ten participants completed the program. Five of the ten participants attended the nutrition sessions. The number of active app users declined after the first week. Seventy percent of participants decreased their BMI. Most participants reported the program improved their ability to exercise regularly, eat a healthier diet and feel more self-confident. Peds QL™ psychosocial health domains increased postintervention. The YRBS showed increased physical activity and less sedentary time postintervention. CONCLUSIONS: This mobile app-based weight management program with coaching implemented may not be feasible for adoption in the general population of AYA with SB; however, it was well received by some, and further testing is needed to determine how to improve feasibility. This study provides useful information to guide future programs utilizing digital health and coaching.

Neuropathology Evaluation of in Utero Correction of Myelomeningocele and Complications of Late-Onset GBS Infection.

Background: Myelomeningocele (MMC) causes significant morbidity and mortality. Efforts have been directed to correct this defect in utero. The neuropathology literature on antenatally repaired MMC and associated complications in humans is limited. Case report: A 12-day-old female, who underwent prenatal MMC repair via a two-layer closure (dural replacement patch, primary skin closure), was born at 34 weeks' gestation. Her group B streptococcus positive mother received appropriate antepartum prophylactic antibiotics. She remained stable until day 11 of life when she underwent rapid clinical deterioration. Despite aggressive intervention, she expired on day 12. Review of placental pathology showed maternal and fetal inflammatory response. Autopsy revealed Gram-positive cocci and inflammation within the basilar leptomeninges and lumbosacral region. Neural and dermal elements were present within the MMC repair. Conclusion: This case documents integration of the dermal matrix patch to neural elements, adhering the spinal cord to scar tissue, the clinical implications of which remain unclear.

Risk factors for unanticipated hospitalizations in children and youth with spina bifida at an urban children's hospital: A cross-sectional study.

BACKGROUND: Spina bifida (SB) is a condition resulting from the improper closure of the neural tube and vertebral column during fetal development. While patients' life expectancy and quality of life have improved dramatically due to medical advances, children continue to experience health-related issues that often require hospitalizations. OBJECTIVE: The association among sociodemographic and clinical characteristics with potentially preventable hospitalizations (PPH) in children and youth with myelomeningocele type SB was investigated in this cross-sectional study. METHODS: Chart reviews and data extraction were conducted on 108 children and youth, ages 1 month to 21 years, admitted for PPH in a regional children's academic medical center between May 2017 and July 2019. Sociodemographic variables included sex, age, type of insurance and ethnicity. Clinical variables included level of lesion, ambulation status, shunt dependency and selected diagnostic categories. Univariate, bivariate, and multivariate analyses were conducted to identify factors associated with PPH. RESULTS: Factors associated with PPH included being male, ages 5-18 years, low lumbar level lesions, non-ambulatory, with public insurance, Hispanic and shunt dependent. Most hospitalizations (73%) were for neurologic or urologic conditions. Factors independently associated with PPH were ethnicity for urologic conditions, being ambulatory for metabolic conditions, and age for gastroenterology conditions. CONCLUSION: Selected demographic and clinical variables were found to be associated with PPH of children and youth with myelomeningocele-type SB. The most common reasons for PPH were shunt malfunctions and urinary tract infections, consistent with other studies.

Exploratory study of the provision of academic and health-related accommodations to transition-age adolescents and emerging adults with spina bifida.

PURPOSE: The purpose of this exploratory study was to investigate the types of academic and health-related accommodations provided to adolescents and emerging adults with spina bifida aged 9-20 years. METHODS: Data were extracted from the paper and electronic records of transition-age youth enrolled in the study. Four open ended items involved content analysis. RESULTS: The most frequently identified accommodation was enrollment in special education classes in 47.7% of the charts. Other academic accommodations that were most often reported were adaptive physical education (n = 71, 39.9%), tutoring (n = 28; 15.7%), and home schooling (n = 21; 11.8%). Clean intermittent catheterization was the most frequently identified health-related accommodation provided by the school nurse/aide (n = 57; 32%).The largest percentage of requests for additional accommodations were made during the middle school grades (15; 54.8%) followed by high school (10; 32.2%). CONCLUSION: Findings demonstrated that persistent issues were identified by parents/adolescents regarding the provision of school-related accommodations. This is a relevant area for clinical practice to ensure students with special health care needs and those with spina bifida receive the academic and health-related accommodations in their Individualized Education Program/504 plans.

Percutaneous/mini-laparotomy fetoscopic repair of open spina bifida: a novel surgical technique.

Open spina bifida is the most common congenital anomaly of the central nervous system compatible with life. Prenatal repair of open spina bifida via open maternal-fetal surgery has been shown to improve postnatal neurologic outcomes, including reducing the need for ventriculoperitoneal shunting and improving lower neuromotor function. Fetoscopic repair of open spina bifida minimizes the maternal risks while providing similar neurosurgical outcomes to the fetus. The following 2 fetoscopic techniques are currently in use: (1) the laparotomy-assisted approach, and (2) the percutaneous approach. The laparotomy-assisted fetoscopic technique appears to be associated with a lesser risk of preterm birth than the percutaneous approach. However, the percutaneous approach avoids laparotomy and uterine exteriorization and is associated with lesser anesthesia risk and improved maternal postsurgical recovery. The purpose of this article was to describe our experience with a modified surgical approach, which we call percutaneous/mini-laparotomy fetoscopy, in which access to the uterus for one of the ports is done via a mini-laparotomy, whereas the other ports are inserted percutaneously. This technique draws on the benefits of both the laparotomy-assisted and the percutaneous techniques while minimizing their drawbacks. This surgical approach may prove invaluable in the prenatal repair of open spina bifida and other complex fetal surgical procedures.

Fetal blood gases after in utero carbon dioxide insufflation for percutaneous fetoscopic spina bifida repair.

BACKGROUND: Prenatal repair of open spina bifida via the percutaneous fetoscopic approach does not require maternal laparotomy, hysterotomy, or exteriorization of the uterus. This technique requires intrauterine partial CO2 insufflation. Limited data exist on the physiological effects of CO2 insufflation on human fetuses, with no data on open spina bifida repair performed using the entirely percutaneous fetoscopic surgical technique. OBJECTIVE: Our aim was to examine the effects of intrauterine partial CO2 insufflation on fetal blood gases after percutaneous fetoscopic open spina bifida repair. STUDY DESIGN: This was a prospective study of patients who underwent percutaneous fetoscopic open spina bifida repair from February 2019 to July 2020. Fetal cordocentesis of the umbilical vein was performed in cases with favorable access to the umbilical cord. The umbilical vein cord blood samples were obtained under ultrasound guidance immediately at the conclusion of the open spina bifida repair. Simultaneous maternal arterial blood gas samples were also obtained. The results are reported as median (range). RESULTS: Of the 20 patients who underwent percutaneous fetoscopic open spina bifida repair during the study period, 7 patients (35%) underwent fetal blood sampling. The gestational age at the time of surgery was 27.4 (24.0-27.9) weeks and the operative time was 183 (156-251) minutes. The CO2 exposure time was 122 (57-146) minutes with maximum pressure of 13.5 (12.0-15.0) mm Hg. Fetal umbilical vein results were as follows: pH 7.35 (7.30-7.39), partial pressure of O2 56.2 (47.1-99.9) mm Hg, partial pressure of CO2 43.8 (36.2-53.0) mm Hg, HCO3 23.9 (20.1-25.6) mmol/L, and base excess -2.2 (-4.5 to -0.4) mmol/L. Simultaneous maternal arterial blood gas results were as follows: pH 7.37 (7.28-7.42), partial pressure of O2 187.5 (124.4-405.2) mm Hg, partial pressure of CO2 36.6 (30.7-46.0) mm Hg, HCO3 21.3 (18.0-22.8) mmol/L and base excess -3.2 (-5.9 to -1.8) mmol/L. CONCLUSION: Despite prolonged CO2insufflation of the uterus, fetal umbilical vein pH and base excess values did not approach those associated with potentially pathologic fetal acidemia.

Quantitative Computed Tomography Assessment of Bone Deficits in Ambulatory Children and Adolescents with Spina Bifida: Importance of Puberty.

Pathologic fractures of the femur and tibia are common in youth with spina bifida (SB). These fractures may be associated with deficient bone accrual due to decreased ambulation and skeletal loading. This prospective cohort study used quantitative computed tomography (QCT) to assess three-dimensional (3D) bone properties in children and adolescents with SB. Eighty-three ambulatory youth with SB underwent QCT imaging of the tibia at up to four annual visits between ages 6 to 16 years (294 total visits averaging 3.5 visits/patient). A total of 177 controls without disability and 10 non-ambulatory youth with SB underwent imaging once. Bone geometric properties (cortical bone area, cross-sectional area, cortical thickness, cortical density, and moments of inertia) were measured at the mid-diaphysis (50% of bone length); cross-sectional area, cancellous density, and density-weighted area were measured in the proximal (13% of bone length) and distal (90% of bone length) metaphyses. Bone properties were compared between the ambulatory SB and control participants, among SB neurosegmental subgroups (sacral, low lumbar, mid lumbar and above) as a function of pubertal stage (prepubertal, pubertal, postpubertal), and considering SB type (myelomeningocele, lipomyelomeningocele) using linear mixed effects models adjusted for sex, age, height percentile, and body mass index (BMI) percentile. Only cancellous density of both metaphyses and weighted area of the proximal metaphysis differed between ambulatory children with SB and controls before puberty. However, significant deficits in all bone properties manifested during and after puberty as moderate bone growth in the SB group failed to keep pace with the large increases normally observed during puberty. The bone deficits primarily affected patients with myelomeningocele, and similar deficits were observed at all neurosegmental levels except that cancellous density was closer to normal in the sacral group. Descriptive analysis of the 10 non-ambulatory youth with SB showed greater bone deficits than ambulatory children, particularly for cancellous density in the distal metaphysis. © 2020 The Authors. JBMR Plus published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research.

Care coordination guidelines for the care of people with spina bifida.

Care coordination is the deliberate organization of patient care activities between two or more participants (including the patient) involved in a person's care to facilitate the appropriate delivery of health care services. Organizing care involves the marshalling of personnel and other resources needed to carry out all required patient care activities. It is often managed by the exchange of information among participants responsible for different aspects of care [1]. With an estimated 85% of individuals with Spina Bifida (SB) surviving to adulthood, SB specific care coordination guidelines are warranted. Care coordination (also described as case management services) is a process that links them to services and resources in a coordinated effort to maximize their potential by providing optimal health care. However, care can be complicated due to the medical complexities of the condition and the need for multidisciplinary care, as well as economic and sociocultural barriers. It is often a shared responsibility by the multidisciplinary Spina Bifida team [2]. For this reason, the Spina Bifida Care Coordinator has the primary responsibility for overseeing the overall treatment plan for the individual with Spina Bifida[3]. Care coordination includes communication with the primary care provider in a patient's medical home. This article discusses the Spina Bifida Care Coordination Guideline from the 2018 Spina Bifida Association's Fourth Edition of the Guidelines for the Care of People with Spina Bifida and explores care coordination goals for different age groups as well as further research topics in SB care coordination.

Psychosocial outcomes for adults with spina bifida: a scoping review protocol.

OBJECTIVE: The objective of this scoping review is to explore the evidence on psychosocial needs and related outcomes for adults with spina bifida. INTRODUCTION: Individuals with spinal bifida have complex service needs that can lead to the emergence of secondary conditions and health complications, which can result in serious, life-threatening illnesses. While much is known about the biological impact of spina bifida, there is a dearth of information regarding its psychological and social impact. INCLUSION CRITERIA: This scoping review will include adults (18 years and over) with the following diagnoses: spina bifida, neural tube defects, tethered cord, myelodysplasia/myelodysplasias, diastematomyelia/diastematomyelias, meningomyelocele, terminal myelocystocele, fatty thickened filum, split cord malformation, or lipomyelomeningocele. The literature reviewed will explore the range and trend of topics reported from the time data on adults with spinal bifida were first published published to the most current time frame. This review will report on the psychosocial needs identified for adults with spinal bifida, such as access to community-based services and support needed to function independently. There will be no limits to the geographical location or setting (e.g. health care or community-based). METHODS: The following databases will be accessed for this review: Ovid MEDLINE, Embase, Cochrane Library, CINAHL, Web of Science, PsycINFO, and ERIC, with no date restrictions. Gray literature searches will be included. Potentially relevant studies will be assessed independently by two reviewers for inclusion and conflicts discussed with a third reviewer. The review narrative will be accompanied by findings presented in tabular format.

Predictors of Walking Activity in Children and Adolescents With Myelomeningocele.

OBJECTIVE: To assess the relationship between real-world walking activity in children and adolescents with myelomeningocele (MMC) and gross measures of lower extremity strength, range of motion, demographics, and medical history. DESIGN: Prospective study. SETTING: Participants recruited in outpatient clinics; data collected in a hospital-based motion analysis laboratory and in the community. PARTICIPANTS: Children and adolescents (N=52) with daily step count data available from a larger study of ambulatory children and adolescents with MMC. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE: Univariate and multivariate regression was used to assess which participant characteristics and clinical factors were related to average number of steps per day. RESULTS: Univariate analysis showed a lower number of steps per day correlated with older age, male sex, higher body mass index, higher lesion level, use of assistive devices for ambulation, history of shunt placement, more television (TV) watched per week, lower hip extension and abduction strength, knee flexion strength, and ankle dorsiflexion and plantarflexion strength, and decreased knee and hip range of motion. Only assistive device usage and hours of TV watched per week remained in the final multivariate model predicting number of steps per day. CONCLUSIONS: Walking activity in children and adolescents with MMC was best predicted by assistive device use and amount of sedentary activity. Other predictors of walking activity from univariate analysis were related to assistive device use. This information can help tailor rehabilitation efforts and educate patients and families. Interventions targeting early prevention of strength loss and contractures may be important to retain or increase walking activity in children and adolescents with MMC.

An exploratory study of adolescents and emerging adults with spina bifida knowledge of their individual education program: Implications for health care transition planning.

PURPOSE: The purpose of this study was to explore adolescents and emerging adults (AEA) with spina bifida (SB) understanding of their Individualized Education Program (IEP) and to identify factors related to their knowledge about their own IEP. METHODS: Data were collected from January 2015 to July 2016 from 79 adolescents with SB. A ten-item questionnaire including demographics and questions specifically addressing IEPs was used. Qualitative analysis followed an iterative, emergent approach. Two experienced coders independently read and coded each of the three open-ended questions. It was determined that the responses of all three questions could feasibly be merged as the analysis of responses were similar. FINDINGS: This was a predominately 70 (88.7%) Hispanic sample of AEA with SB who ranged in age from 12 to 20 years (M= 15.3 years) consisting of 41 males and 38 females. All reported they have/had an IEP. Four major themes and eleven subthemes emerged from the analysis. Major themes were: The Barometer of How I Am Doing, Creating the Right Match for Learning, Obtaining the Assistance I Need, and Future Goals and Planning. CONCLUSIONS: Findings of this study reveal the IEP knowledge gaps and lack of lifestyle self-management skills AEA with SB reported pertaining to IEPs.

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.

Advanced skeletal maturity in children and adolescents with myelomeningocele.

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.

Fasting serum blood measures of bone and lipid metabolism in children with myelomeningocele for early detection of cardiovascular and bone fragility risk factors.

OBJECTIVE: This study examined serum levels in children with myelomeningocele to identify the prevalence of pre-clinical signs of disease. DESIGN: A prospective, cross-sectional study. SETTING: Patients were actively recruited from multidisciplinary care clinics at tertiary children's hospitals from 2010-2012. The control comparison group was recruited by word-of-mouth. PATIENTS: Twenty-eight children with myelomeningocele (93% Hispanic; 17 males; 10.0 ± 2.1 years) and 58 controls (84% Hispanic; 30 males; 10.4 ± 2.4 years) provided ≥ 8-hour fasting blood samples with concomitant dual-energy x-ray absorptiometry measurements of body fat. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: The serum analysis included a lipid panel (cholesterol, triglycerides, high-density lipoprotein, low-density lipoprotein), insulin, glucose, leptin, aspartate aminotransferase, alanine transaminase, alkaline phosphatase, albumin, creatinine, calcium, phosphatase, parathyroid hormone, and vitamin D. RESULTS: Children with myelomeningocele had higher body fat (35.2% versus 29.9%, p=0.01) and altered lipid profiles (lower high-density lipoprotein levels, 43.9 mg/dL versus 51.6 mg/dL, P = 0.03) suggesting elevated risk of metabolic syndrome. They also had a higher prevalence of vitamin D deficiency (43% versus 17%, p=0.02) and significantly lower levels of calcium (9.4 mg/dL versus 9.7 mg/dL, P = 0.003) and alkaline phosphatase (187.0 U/L versus 237.0 U/L, P = 0.003). Unexpectedly children with myelomeningocele had lower parathyroid hormone levels (14.5 pg/mL versus 18.4 pg/mL, P = 0.02) than controls despite lower calcium, vitamin D and alkaline phosphatase levels. This suggests an alteration in the sensing mechanism or response of the parathyroid gland to normal physiological stimuli in patients with myelomeningocele. CONCLUSIONS: Children with myelomeningocele have abnormal biochemical markers for cardiovascular disease, insulin resistance and bone and mineral metabolism. Early recognition and monitoring of these risk factors in patients with myelomeningocele may help prevent later complications.

Quantitative Analysis of Lower Leg Adipose Tissue Distribution in Youth with Myelomeningocele.

Children with myelomeningocele have a high prevalence of obesity and excess fat accumulation in their lower extremities. However, it is not known if this is subcutaneous or intramuscular fat, the latter of which has been associated with insulin resistance and metabolic disorders. This study quantified lower leg bone, muscle, and adipose tissue volume in children with myelomeningocele, classifying adipose as subcutaneous or muscle-associated. Eighty-eight children with myelomeningocele and 113 children without myelomeningocele underwent lower leg computed tomographic scans. Subcutaneous and muscle-associated adipose were classified based on location relative to the crural fascia. No differences were seen in subcutaneous adipose. Higher level disease severity was associated with increased muscle-associated adipose volume and decreased muscle volume. Bone volume tended to decrease with higher levels of involvement. Increases in lower leg adiposity in children with myelomeningocele are primarily attributable to accumulation of muscle-associated adipose, which may signify increased risk for metabolic disorders.

Movin' On Up: An Innovative Nurse-Led Interdisciplinary Health Care Transition Program.

This article provides an overview of an innovative nurse-led interdisciplinary health care transition (HCT) model of care entitled Movin' On Up for adolescents and emerging adults (AEAs) with spina bifida (SB) that was originally implemented in 2011. The components of the HCT service model include an HCT nursing specialist, who is an advanced practice nurse; interdisciplinary health care transition plans based on the individualized needs of AEAs; an interdisciplinary HCT team that meets on a weekly basis; direct HCT services provided in the weekly SB clinic; and telephonic follow-up with AEAs, families, and providers. The characteristics of this nurse-led HCT program can be described as an integrated, interdisciplinary, and comprehensive model of care based on a life span approach. To date, a total of 210 AEAs with SB, ages 10 to 20 years, have been enrolled into the program. An important feature of this HCT service model is that it is self-supporting; it generates the revenue needed for sustainability and, unlike other HCT programs, is not reliant on extramural programmatic support. Other accomplishments of Movin' On Up include the development of a transfer protocol wherein 35 AEAs with SB have been supported in their transfer to adult care; implementation of a standardized process to ensure that service referrals to community-based services for postsecondary education, employment, training, and initiation of conservatorships are made; timely performance of evaluations; close tracking of needs and outcomes of self-management knowledge and skills instruction; and attention to equipment needs prior to transfer.

Fat distribution in children and adolescents with myelomeningocele.

AIM: To evaluate fat distribution in children and adolescents with myelomeningocele using dual-energy X-ray absorptiometry (DXA). METHOD: Cross-sectional DXA measurements of the percentage of fat in the trunk, arms, legs, and whole body were compared between 82 children with myelomeningocele (45 males, 37 females; mean age 9y 8mo, SD 2y 7mo; 22 sacral, 13 low lumbar, 47 mid lumbar and above) and 119 comparison children (65 males, 54 females; mean age 10y 4mo, SD 2y 4mo). Differences in fat distribution between groups were evaluated using univariate and multivariate analyses. RESULTS: Children with myelomeningocele had higher total body fat (34% vs 31%, p=0.02) and leg fat (42% vs 35%, p<0.001) than comparison children, but no differences in trunk or arm fat after adjustment for anthropometric measures. INTERPRETATION: Children with myelomeningocele have higher than normal total body and leg fat, but only children with higher level lesions have increased trunk fat, which may be caused by greater obesity in this group. Quantifying segmental fat distribution may aid in better assessment of excess weight and, potentially, the associated health risks.

Heterogeneity of muscle fat infiltration in children with spina bifida.

Children with spina bifida have well recognized functional deficits of muscle, but little is known about the associated changes in muscle anatomy and composition. This study used water-fat magnetic resonance imaging (MRI) to measure fat infiltration in the lower extremity muscles of 11 children with myelomeningocele, the most severe form of spina bifida. MRI measurements of muscle fat fraction (FF) were compared against manual muscle test (MMT) scores for muscle strength. The FF measurements were objective and reliable with mean inter-rater differences of <2% and intraclass correlation coefficients>0.98. There was a significant inverse relationship between muscle FF and MMT scores (P ≤ 0.001). Surprisingly, however, muscles with negligible strength (MMT 0-1) exhibited a bimodal distribution of FF with one group having FF>70% and another group having FF<20%. The MRI also revealed striking heterogeneity amongst individual muscles in the same muscle group (e.g., 4% fat in one participant's lateral gastrocnemius vs. 88% in her medial gastrocnemius), as well as significant asymmetry in FF in one participant with asymmetric strength and sensation. These results suggest that quantitative water-fat MRI may serve as a biomarker for muscle degeneration which may reveal subclinical changes useful for predicting functional potential and prognosis.