Evaluation of multidisciplinary high-risk pregnancy clinic for myelomeningocele.

INTRODUCTION: A cross-sectional study retrospectively evaluating the perceived usefulness of attending a multi-disciplinary, roundtable, educational prenatal clinic for mothers expecting children with myelomeningocele is presented. METHODS: Mothers who currently have children with SB completed a survey which evaluated their overall preparedness, spina bifida education, delivery plans, surgical expectations, and expectations in terms of quality of life and development. Open comments were also collected. Statistical analysis was performed to identify differences between those who attended prenatal counseling and those who did not. RESULTS: Approximately half of these mothers received some form of prenatal SB counseling. Mothers who attended prenatal counseling reported that they felt more informed and prepared throughout their pregnancy, during the delivery of their child and during their initial hospital stay than mothers who did not. They reported that the roundtable discussions were beneficial, and the education they received was useful in helping them form accurate expectations and feel more at ease. CONCLUSION: This suggests that prenatal counseling and the High-Risk Pregnancy Clinic (HRPC) provides perceived utility to families and mothers and that the HRPC is an effective method of providing prenatal counseling to mothers whose unborn children have been diagnosed with myelomeningocele.

Neurosurgical Management of Myelomeningocele in Premature Infants: A Case Series.

Introduction Myelomeningocele (MMC) is the most common neural tube defect, but rarely seen in premature infants. Most centers advocate for closure of MMC within 24 hours of birth. However, this is not always possible in severely premature infants. Given the rarity of this patient population, we aimed to share our institutional experience and outcomes of severely premature infants with MMC. Methods We performed a retrospective, observational review of premature infants (≤ 32 weeks gestational age) identified through our multidisciplinary spina bifida clinic (1995-2021) and surgical logs. Descriptive statistics were compiled about this sample including timing of MMC closure and incidence of adverse events such as sepsis, CSF diversion, meningitis, and death. Results Eight patients were identified (50% male) with MMC who were born ≤ 32 weeks gestational age. Mean gestational age of the population was 27.3 weeks (SD 3.5). Median time to MMC closure was 1.5 days (IQR = 1-80.8). Five patients were taken for surgery within the recommended 48 hours of birth; 2 patients underwent significantly delayed closure (107 and 139 days); and one patient's defect epithelized without surgical intervention. Six of eight patients required permanent cerebrospinal fluid (CSF) diversion (2 patients were treated with ventriculoperitoneal shunting (VPS), three were treated with endoscopic third ventriculostomy (ETV) with choroid plexus cauterization (CPC) and 1 patient treated with ETV; mean of 3 years after birth, ranging from 1 day to 16 years). Two patients required more than one permanent CSF diversion procedure. Two patients developed sepsis (defined as meeting at least 2/4 SIRS criteria), and 2 patients had intraventricular hemorrhage (both grade III). No patients developed meningitis (defined as positive CSF cultures) prior to MMC closure. Median follow up duration was 9.7 years. During this time epoch, 3 patients died: Two before 2 years of age of causes unrelated to surgical intervention. One of the two patients with grade III IVH died within 24 hours of MMC closure. Conclusions In our institutional experience with premature infants with MMC, some patients underwent delayed MMC closure. The overall rate of meningitis, sepsis, and mortality for preterm children with MMC was similar to MMC patients born at term.

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.

First-time tethered cord release among adults with myelomeningocele: an analysis of people in the National Spina Bifida Patient Registry.

OBJECTIVE: The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism, to determine the ongoing longitudinal risk for tethered cord release (TCR) among adults with myelomeningocele who had not previously undergone TCR during childhood. The authors also sought to identify the impact of lesion level, ambulation status, and prior treatments for hydrocephalus or Chiari malformations on TCR rates. METHODS: Adults in the registry who had not previously undergone TCR during childhood were studied. This group was compared with the remaining adults in the registry. The frequency of first-time TCR and time to TCR (using Kaplan-Meier analysis) were determined independently for males and females. Cox proportional hazards analysis identified correlations between sex, best lesion level and ambulation status prior to TCR, and previous treatments for hydrocephalus and Chiari decompression. RESULTS: Among 967 adults in the NSBPR (422 [43.6%] males and 545 [56.4%] females) who had not undergone TCR during childhood, the authors identified 47 people (4.9%) who underwent their first TCR during adulthood. This study cohort had significantly better mean functional motor levels and ambulation compared with the remaining adult cohort (both p < 0.001). The study group included 35 females (74.5%) and 12 males (25.5%); this sex distribution was significantly different in comparison with the remaining adult cohort (p = 0.016). The Kaplan-Meier curves for first TCR for females and males were significantly different (p = 0.01, log-rank test). TCR rates were correlated with sex (males had decreased risk; OR 0.31, 95% CI 0.16-0.62, p < 0.001), prior treatment for hydrocephalus (those who underwent prior treatment had decreased risk; OR 0.21, 95% CI 0.20-0.42, p < 0.001), and prior treatment for Chiari malformation (those who underwent prior treatment had greater risk; OR 3.84, 95% CI 1.50-9.88, p = 0.005). CONCLUSIONS: Adults with myelomeningocele who escape childhood without undergoing TCR have an ongoing, albeit decreased, risk for spinal cord tethering requiring TCR. This risk is obviously not due to spinal column growth and therefore must reflect other factors such as dynamic changes in spinal cord health over time. Among people with MMC who underwent their first TCR as adults, females seemed to be overrepresented. Similar to the authors' prior childhood study, people who underwent previous Chiari decompression seemed to be overrepresented, whereas those who underwent previous treatment for hydrocephalus seem to be underrepresented. These novel findings deserve further study.

What has changed in pediatric neurosurgical care in spina bifida? A 30-year UAB/Children's of Alabama observational overview.

Spina bifida (SB) remains the most serious and most common congenital anomaly of the human nervous system that is compatible with life. The open myelomeningocele on the back is perhaps the most obvious initial problem, but the collective impact of dysraphism upon the entirety of the nervous system and innervated organs is an equal or greater longitudinal threat. As such, patients with myelomeningocele (MMC) are best managed in a multi-disciplinary clinic that brings together experienced medical, nursing, and therapy teams that provide high standards of care while studying outcomes and sharing insights and experiences. Since its inception 30 years ago, the spina bifida program at UAB/Children's of Alabama has remained dedicated to providing exemplary multi-disciplinary care for affected children and their families. During this time, there has been great change in the care landscape, but many of the neurosurgical principles and primary issues have remained the same. In utero myelomeningocele closure (IUMC) has revolutionized initial care and has favorable impact on several important co-morbidities of SB including hydrocephalus, the Chiari II malformation, and the functional level of the neurologic deficit. Hydrocephalus however is not solved by IUMC, and hydrocephalus management remains at the center of neurosurgical care in SB. Ventricular shunts were long the cornerstone of treatment for hydrocephalus, but we came to assess and incorporate endoscopic third ventriculostomy with choroid plexus coagulation (ETV-CPC). Educated and nurtured by an experienced senior mentor, we dedicated ourselves to fundamental concepts but persistently evaluated our care outcomes and evolved our protocols and paradigms for improvement. Active conversations amidst networks of treasured colleagues were central to this development and growth. While hydrocephalus support and treatment of tethered spinal cord remained our principal neurosurgical charges, we evolved to embrace a holistic perspective and approach that is reflected and captured in the Lifetime Care Plan. Our team engaged actively in important workshops and guideline initiatives and was central to the development and support of the National Spina Bifida Patient Registry. We started and developed an adult SB clinic to support our patients who aged out of pediatric care. Lessons there taught us the importance of a model of transition that emphasized personal responsibility and awareness of health and the crucial role of dedicated support over time. Support for sleep, bowel health, and personal intimate cares are important contributors to overall health and care. This paper details our growth, learning, and evolution of care provision over the past 30 years.

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.

The development of a transition medical home utilizing the individualized transition plan (ITP) model for patients with complex diseases of childhood.

BACKGROUND: Advances in medicine and technology, have enabled greater numbers of children with complex illness to survive into adulthood. Adolescents with these conditions are at high risk for adverse outcomes when transitioning to adult health care. The "Staging Transition for Every Patient" (STEP) Program was developed to systematically improve the transition from pediatric to adult healthcare. OBJECTIVE: This article details the development of the STEP program and the novel use of "Individualized Transition Plans" (ITP) in the clinic setting. METHODS: A provider needs' assessment of the existing transition services among youth with specific diagnoses was performed, a steering committee was developed that created a transition policy, and a medical home within the adult system was established with an interdisciplinary approach. The ITP focuses on 5 individualized goals, it was developed and tested with the first-year cohort of patients. RESULTS: In the initial needs assessment, 7 of 35 diagnoses were found to have an effective transition plan. The STEP program partnered with departments across the adult facility to conduct 267 interdisciplinary patient visits. In the first year, 169 new patients were seen in the clinic. The average age was 23.0 ± 4.1 years old. The ITP goals included referrals to adult specialists, advanced care planning, career and education, transition readiness, caregiver burden, and an emergency sick plan. CONCLUSION: There is a need for organized transition care for medically complex youth. The STEP program answers that need by addressing the unique needs of each patient. Individualized transition planning builds trust and addresses multiple domains of health.

Functional level of lesion scale: Validating fourteen years of research with the national spina bifida patient registry.

PURPOSE: Functional level of lesion (FLOL) is a grading of the level of neurological function in patients with myelomeningocele and other forms of spina bifida. It has been widely used as an independent variable in studies of spina bifida, but its inter-rater reliability has not previously been tested. The purpose of this study was to measure inter-rater reliability of FLOL testing and compare testing performed by a non-medically trained research associate to testing performed by a pediatric rehabilitation medicine specialist. METHODS: Children in a multi-disciplinary spina bifida clinic underwent FLOL grading by a non-medically trained research associate. On the same day, these children were also graded by a pediatric rehabilitation medicine specialist. Cohen's weighted kappa statistic was used to compare grading, with the rehabilitation medicine specialist considered the gold standard. RESULTS: A total of 71 patients participated. FLOL was graded for left and right leg for each participant, resulting in 142 measurements. Cohen's weighted kappa was κ= 0.809, with a standard error of 0.034 and 95% confidence interval 0.723-0.875, indicating substantial agreement. CONCLUSION: FLOL as measured according to the instructions of the National Spina Bifida Patient Registry by a non-medically trained researcher is a reliable method to grade lower extremity function in spina bifida.

Telemedicine and Spina Bifida Transition: A Pilot Randomized Trial.

OBJECTIVES: Transition of care is the planned movement of adolescents and young adults from pediatric to adult health care. Many studies have demonstrated the importance of an organized transition process. The purpose of this study is to determine the efficacy of a telemedicine intervention for improving transition readiness among adolescents with spina bifida. METHODS: The present study is a randomized, controlled trial, including children 14 years of age and older with myelomeningocele from a multidisciplinary spina bifida clinic. Subjects were randomized to standard care or to an intervention, consisting of video telemedicine contacts at 3, 6, and 9 months from the clinic visit. The primary outcome measure was a change in the Transition Readiness Assessment Questionnaire score from baseline to 1-year follow-up. RESULTS: Twenty-four patients were enrolled in the study and underwent randomization. The mean age at enrollment was 15.8 years. Ten patients (40%) were female, and the majority were White, non-Hispanic (67%). Despite enrolling 24 patients, only 1 patient in the telemedicine group completed any of the planned telemedicine sessions. No other participant completed any telemedicine counseling sessions. The study was stopped early for lack of participation in the intervention. In a single-group, as-treated analysis, there was no significant change in the Transition Readiness Assessment Questionnaire score between enrollment and 1-year follow-up (Δ = 0.36, P = 0.46). However, there were significant improvements in subscores for Managing Medications, Appointment Keeping, and Managing Daily Activities. CONCLUSIONS: The primary finding from this study was very low participation in a telemedicine video follow-up intervention among adolescents with myelomeningocele. Based on these results, this strategy alone is unlikely to significantly improve readiness for transition to adult care.

Time to shunt failure in children with myelomeningocele: an analysis of the National Spina Bifida Patient Registry.

OBJECTIVE: Hydrocephalus is common among children with myelomeningocele and is most frequently treated with a ventriculoperitoneal shunt (VPS). Although much is known about factors related to first shunt failure, relatively less data are available about shunt failures after the first one. The purpose of this study was to use a large data set to explore time from initial VPS placement to first shunt failure in children with myelomeningocele and to explore factors related to multiple shunt failures. METHODS: Data were obtained from the National Spina Bifida Patient Registry. Children with myelomeningocele who were enrolled within the first 5 years of life and had all lifetime shunt operations recorded in the registry were included. Kaplan-Meier survival curves were constructed to evaluate time from initial shunt placement to first shunt failure. The total number of children who experienced at least 2 shunt failures was calculated. A proportional means model was performed to calculate adjusted hazard ratios (HRs) for shunt failure on the basis of sex, race/ethnicity, lesion level, and insurance status. RESULTS: In total, 1691 children met the inclusion criteria. The median length of follow-up was 5.0 years. Fifty-five percent of patients (938 of 1691) experienced at least 1 shunt failure. The estimated median time from initial shunt placement to first failure was 2.34 years (95% confidence interval [CI] 1.91-3.08 years). Twenty-six percent of patients had at least 2 shunt failures, and 14% of patients had at least 3. Male children had higher likelihood of shunt revision (HR 1.25, 95% CI 1.09-1.44). Children of minority race/ethnicity had a lower likelihood of all shunt revisions (non-Hispanic Black children HR 0.74, 95% CI 0.55-0.98; Hispanic children HR 0.74, 95% CI 0.62-0.88; children of other ethnicities HR 0.80, 95% CI 0.62-1.03). CONCLUSIONS: Among the children with myelomeningocele, the estimated median time to shunt failure was 2.34 years. Forty-five percent of children never had shunt failure. The observed higher likelihood of shunt revisions among males and lower likelihood among children of minority race/ethnicity illustrate a possible disparity in hydrocephalus care that warrants additional study. Overall, these results provide important information that can be used to counsel parents of children with myelomeningocele about the expected course of shunted hydrocephalus.

Determination of anatomic level of myelomeningocele by prenatal ultrasound.

PURPOSE: Ultrasound is the primary method for prenatal identification of myelomeningocele and is critical to prognostication and treatment planning. No study has considered the degree of inaccuracy of prenatal US lesion level estimates and anatomic lesion level on postnatal imaging using the weighted kappa coefficient (κw), nor the impact of maternal BMI on agreement. We examined the accuracy of prenatal ultrasound lesion level estimation in a cohort of patients with myelomeningocele using κw and determined whether BMI influenced accuracy. METHODS: The study is a retrospective review including patients born 2011-2019 who had prenatal imaging and primary myelomeningocele closure at a single institution. Lesion levels from prenatal ultrasound and postnatal imaging studies were analyzed for agreement at exact level, within 1 level, and within 2 levels using κw. Maternal BMI was examined for correlation with accuracy. RESULTS: Fifty-seven patients met inclusion criteria. Mean BMI was 31.2. There was no association between maternal BMI and agreement at any level. Lesion level on prenatal ultrasound agreed with postnatal imaging to the exact level in 13 (22.8%) cases, within 1 level in 38 (66.7%) cases, and within 2 levels in 50 (87.7%) cases. Weighted kappa showed moderate agreement at exact level (κw = 0.494) and substantial agreement within 1 (κw = 0.761) and 2 levels (κw = 0.902). CONCLUSION: Weighted kappa adds confidence for clinical decision making by accounting for accuracy. Prenatal ultrasound is a reliable and accurate method of determining lesion level with near-perfect agreement to postnatal imaging within 2 spinal levels. Maternal BMI may not influence lesion level determination after initial diagnosis.

A qualitative interview study on successful pregnancies in women with spina bifida.

BACKGROUND: Improvements in antenatal medicine and surgical management for conditions associated with spina bifida such as hydrocephalus have extended the lifespan for individuals with spina bifida (SB) into adulthood. Decisions and education regarding reproductive care and pregnancies for patients with spina bifida are increasingly important. Pregnancy in these patients can be particularly challenging due to physical limitations, previous abdominal surgery for urinary or bowel management and presence of a ventriculoperitoneal shunt. To date, little research has examined the unique challenges that women with spina bifida face during pregnancy. OBJECTIVE: The purpose of this descriptive study is to characterize the successful pregnancy histories of SB women and describe how pregnancy affected their mobility as well as bladder and bowel management. STUDY DESIGN: We conducted semi-structured interviews with women followed in our adult multidisciplinary SB clinic who previously had successful pregnancies. Questions regarding perinatal issues, obstetrical complications, urinary tract infections (UTI) and neurological changes were asked. Baseline mobility, bladder and bowel management were compared with changes during and after pregnancy. RESULTS: 121 women of childbearing age were followed per year by our adult multidisciplinary spina bifida clinic between 2009 and 2016. We identified 6 women who successfully carried 8 pregnancies to term. There were no miscarriages. Four women had ventriculoperitoneal (VP) shunts. No children were born with neural tube defects. Mean age at first pregnancy was 23.5 years. Average gestational age at delivery was 37 weeks. 50% of the women had a spontaneous vaginal delivery. Five of six women intended to get pregnant; only one patient consumed folic acid regularly prior to pregnancy. Two of six women had bladder augmentation surgery, one of whom had urologic changes during pregnancy that persisted after childbirth. The other patient had a concomitant bladder neck sling procedure and did not have urologic issues during pregnancy. 50% of the patients experienced bladder-bowel dysfunction during their pregnancy. While 67% patients had full baseline ambulatory function, 4 patients had decreased mobility and required additional assistance during pregnancy. All returned to their baseline functionality afterwards. CONCLUSION: Six of our patients had eight successful pregnancies, with no children born with neural tube defects. New changes to mobility, bladder and bowel management were experienced by over half of the women during their pregnancies. Future studies should focus on the role of multidisciplinary teams in reproductive health education and perinatal management of changes to activities of daily living during pregnancy in this population.

Scope of care in the first four years of life for individuals born with myelomeningocele: A single institution experience1.

PURPOSE: Children with myelomeningocele are known to be consumers of substantial healthcare resources, with many early hospital encounters. The purpose of this study was to survey the extent of medical and surgical care that patients with myelomeningocele receive during the first four years of life. METHODS: Clinical and demographic data were collected on newborn infants with open myelomeningocele from the Children's of Alabama Spina Bifida Web Tracker, a prospective, comprehensive spina bifida database. Additional data pertaining to all hospital admissions, surgical procedures, and clinic visits were collected from the medical record. RESULTS: One hundred and fourteen subjects with a primary diagnosis of myelomeningocele between 2004 and 2015 were included. Males slightly predominated (55%), 61.4% were Caucasian, 11% Hispanic/Latino; 28% had a mid-lumbar functional lesion level. Over the first four years of life, 688 total surgical procedures were performed (an average of 86 per child): 438 in year 1, 100 in year 2, 84 in year 3, and 66 in year 4. The mean number of hospital visits was 40.5. Total average drive time per patient over 4 years being 103.8 hours. Average number of nights spent in the hospital was 51. CONCLUSION: Children with myelomeningocele need multiple hospitalizations, surgeries, and medical encounters in the first 4 years of life. These data will be valuable when counselling new parents and prospective parents of children with this condition.

Caring for the Child with Spina Bifida.

Care for a child with spina bifida can be complex, requiring multiple specialists. Neurosurgical care centers around the initial closure or repair of the spinal defect, followed by management of hydrocephalus, symptoms of the Chiari 2 malformation, and tethered cord. This article reviews definitions and types of spina bifida, considerations surrounding the initial treatment, including fetal surgery, and the ongoing neurosurgical management of common comorbid conditions. The role of interdisciplinary care is stressed, as well as the importance of coordinated transition to adult care at an appropriate age and developmental stage.

Tethered spinal cord among individuals with myelomeningocele: an analysis of the National Spina Bifida Patient Registry.

OBJECTIVE: The aims of this study were to review the National Spina Bifida Patient Registry (NSBPR) data set to study the rates of tethered spinal cord release (TCR) among patients with myelomeningocele and variability between centers, to compare TCR rates between males and females, and to study the relationships between TCR rates and other condition-specific characteristics. METHODS: The NSBPR registry was queried to identify all patients with myelomeningocele. TCR rates were calculated over time using survival analyses; rates between centers and between males and females were compared. Cox proportional hazards models were constructed to identify relationships between TCR rates and sex, functional lesion level, ambulation status, treated hydrocephalus, and prior Chiari decompression. RESULTS: Of 6339 patients with information about their operations, 1366 (21.5%) underwent TCR, with significant variability between centers. The majority (75.8%) underwent a single TCR. The annual TCR rate was linear between birth and 13 years (1.8%/year) but declined sharply from 14 to 21 years (0.7%/year). There was no period of time at which the TCR rate accelerated. There were no significant differences in TCR rates between males and females. TCR rate was not related to functional lesion level but was lower among nonambulators compared with community ambulators (p = 0.005) and among those with treated hydrocephalus (HR 0.30, p < 0.001), and higher among those having prior Chiari decompression (HR 1.71, p < 0.001). CONCLUSIONS: These results extend the results of prior single-institution studies, demonstrate significant treatment variability between institutions, and challenge the traditional concept that tethering is related to spinal cord stretching due to spinal growth.

Hydrocephalus in Spina Bifida.

Hydrocephalus is the most important co-morbidity in myelomeningocele from a neurosurgical perspective. Historically, 75-80% of patients with myelomeningocele have required treatment with a shunt but recent advances including intra-uterine myelomeningocele closure and ETV-CPC are reducing this burden. The expression of hydrocephalus differs between patients and across the life span. Hydrocephalus impacts the clinical expression of other important co-morbidities including the Chiari II malformation and tethered spinal cord. Shunt failure is often the key stress to prompt symptomatic worsening of these other conditions. Shunt failure may occur with minimal ventricular change on CT or MRI in Spina Bifida patients. Waiting for radiographic changes in symptomatic SB patients with shunts may result in hydrocephalus related fatalities. It is hypothesized but not proven that shunt failure may contribute to respiratory insufficiency and be a risk factor for sudden death in adult patients with spina bifida. Excellence in hydrocephalus management in MMC is essential for proper care, good outcomes, and quality of life for patients and families.

Prevalence of Sleep Disordered Breathing in Children With Myelomeningocele.

BACKGROUND: Retrospective studies have shown high rates of sleep disordered breathing in children with myelomeningocele. However, most patients included in those studies underwent polysomnography because of symptoms, so the prevalence of sleep disordered breathing in this population is unknown. OBJECTIVE: To determine the prevalence of sleep disordered breathing in children with myelomeningocele using screening polysomnography. METHODS: In this cross-sectional study, all children with myelomeningocele seen in a multi-disciplinary spina bifida clinic between 2016 and 2020 were referred for polysomnography regardless of clinical symptoms. Included children had not previously undergone polysomnography. The primary outcome for this study was presence of sleep disordered breathing, defined as Apnea-Hypopnea Index (AHI, number of apnea or hypopnea events per hour of sleep) greater than 2.5. Clinical and demographic variables relevant to myelomeningocele were also prospectively collected and tested for association with presence of sleep apnea. RESULTS: A total of 117 participants underwent polysomnography (age 1 mo to 21 yr, 49% male). The majority were white, non-Hispanic. Median AHI was 1.9 (interquartile range 0.6-4.2). A total of 49 children had AHI 2.5 or greater, yielding a sleep disordered breathing prevalence of 42% (95% CI 33%-51%). In multivariable logistic regression analysis, children with more rostral neurological lesion levels had higher odds of sleep disordered breathing (OR for thoracic, mid-lumbar, and low-lumbar: 7.34, 3.70, 4.04, respectively, compared to sacral level, P = .043). CONCLUSION: Over 40% of a sample of children with myelomeningocele, who underwent screening polysomnography, had significant sleep disordered breathing. Routine screening polysomnography may be indicated in this population.

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.

Neurosurgery guidelines for the care of people with spina bifida.

Myelomeningocele (MMC) arises from an early neural developmental anomaly and results in a variety of structural abnormalities and associated functional neurologic deficits. As such, neurologic issues are central to virtually all clinical problems. Neurosurgical intervention strives to correct or improve these defects and prevent secondary complications. These interventions include closure of the open myelomeningocele and management (across the life span) of hydrocephalus, the Chiari II malformation (C2M) and tethered spinal cord (TSC). The development of pre-natal closure techniques and reports of improved outcome with in-utero closure (IUMC) have revolutionized the neurosurgical approach to myelomeningocele. Controversies remain surrounding patient selection, maternal risks, technique of IUMC (endoscopic vs. open) and long-term outcomes. However, real gains include reduced rates of hydrocephalus, modestly improved motor capabilities and reduction in C2M morbidity. For many decades, the cornerstone of treatment of hydrocephalus for many decades has been the placement and support of ventricular shunts. Endoscopic third ventriculostomy (ETV) with or without choroid plexus coagulation (ETV/CPC) is an appealing alternate strategy that avoids the morbidity and complications associated with shunts. The exact criteria for ETV-CPC candidacy and best metrics for outcome analysis remain active areas of debate and controversy. Similarly, neurosurgical management C2M, has centered upon the indications and clinical thresholds for performing posterior fossa surgical decompression. Tethered spinal cord management incorporates the diagnosis and surgical management of adhesions formed at the initial closure site, the consequent longitudinal traction related stress on the cord and the resulting neurologic signs and symptoms.