Filum terminale infected epidermoid cysts in pediatric age group; A case series.

Background: Epidermoid cysts are rare and account for only 1 % of primary spinal tumors. It's due to inclusion of ectodermal tissue during the third and fourth weeks of gestation. Infected epidermoid cysts are exceedingly rare with very few reports in literature. The clinical presentations include radicular symptoms, motor weakness, sphincteric disturbance, and repeated chemical meningitis. Surgery of this kind of tumor remained a challenge and of a considerable recurrence rate. Methods: This is a retrospective study reviewing the records of six children who were treated for filum terminale infected epidermoid cysts. All patients underwent microsurgery, and the surgical outcomes were studied through a follow up period of at least 24 months. Results: All children had a low back dermal sinus with purulent discharge. One child was a recurrent presentation after the previous two surgeries for evacuation of pus collection, and the other five children were operated for the first time. All the six children had an associated neurological deficit; one child presented with active central nervous system infection, and one child had a history of meningitis and admission to hospital prior to our surgery. Regular follow up revealed no recurrence in any of the six patients. The five patients, who presented with motor weakness, showed significant improvement of the motor power with regular post-operative physical therapy. Conclusion: Epidermoid cysts are rare benign lesions that may lead to significant morbidity when infected. The aim of microsurgical excision is to remove the cyst content and its capsule without inducing or increasing neurological deficit by the aid of intraoperative neuromonitoring and microsurgery techniques.

Togaram1 is expressed in the neural tube and its absence causes neural tube closure defects.

Neural tube closure defect pathomechanisms in human embryonic development are poorly understood. Here we identified spina bifida patients expressing novel variants of TOGARAM gene family. TOGARAM1 has been associated with the ciliopathy Joubert syndrome, but its connection to spina bifida and role in neural development is unknown. We show that Togaram1 is expressed in the neural tube and Togaram1 knockout mice have abnormal cilia, reduced sonic hedgehog (Shh) signalling, abnormal neural tube patterning and display neural tube closure defects. Neural stem cells from Togaram1 knockout embryos showed reduced cilia and defects in Shh signalling. Overexpression in IMCD3 and HEK293 cell of TOGARAM1 carrying the variant found in the spina bifida patient resulted in cilia defect along with reduced pericentriolar material one (PCM1), a critical constituent of centriolar satellites involved in transporting proteins towards the centrosome and primary cilia. Our results demonstrate the role of TOGARAM1 in regulating Shh signalling during early neural development that is critical for neural tube closure and elucidates potential mechanisms whereby the ciliopathy associated gene TOGARAM1 gives rise to spina bifida aperta in humans.

Influencing Factors on Independent Walking in Children With Lumbosacral Lipomas: A Retrospective Cohort Study Based on a 5-Year Untethering Series.

Background: Caregivers are deeply concerned about children achieving independent walking, and evidence-based rehabilitation support is beneficial. However, current research is confined to a single study on spina bifida aperta, leaving a gap in understanding the timing of independent walking for lumbosacral lipomas. Objectives: This study aimed to examine the factors influencing independent walking in children with lumbosacral lipomas. Design: Retrospective cohort study. Methods: This retrospective cohort study included 124 children who underwent untethering surgery for lumbosacral lipomas. The age (in months) at which the children walked independently was used as the primary endpoint, and potential influencing factors, including the type of spinal lipoma, extent of lipoma removal, magnetic resonance imaging features, congenital anomaly complications, urinary/defecation management requirements, foot/toe symptoms, and orthotic device fabrications were analyzed. Results: Multiple logistic regression analysis showed that the most significant influencing factor for delayed independent walking was the presence of systemic combined anomalies (adjusted odds ratio = 15.5, P < .001), while non-systemic malformations, such as suburethral cleft, had limited effects. A subgroup analysis of 94 patients without systemic combined anomalies showed that the presence of a malformed conus medullaris was significantly associated with delayed independent walking (P = .014). The median age of independent walking in children with Morota's classification type 2 was 14 months, which is 1 month later compared to other types, although this difference was not significant (P = .055). Conclusion: Our findings suggest that complications arising from systemic combined anomalies and the presence of malformed conus medullaris are influencing factors in delays in independent walking in children with untethered lumbosacral lipomas.

Complete dorsal wall agenesis of the sacral canal in a Greek population: an osteological study.

INTRODUCTION: The failure of closure of the dorsal wall of the sacral canal (SC) has been known since the eve of modern osteology, appearing in prehistoric times. Variants include partial or complete absence of the dorsal wall of the SC. SC presents a pathway for minimally invasive therapeutic and diagnostic procedures for spinal diseases and for ensuring analgesia and anesthesia in operations, including labor and genitourinary surgery.

Association between social determinants of health and select neurosurgical procedures in the National Spina Bifida Patient Registry.

OBJECTIVE: Myelomeningocele (MMC) is a lifelong condition requiring complex multidisciplinary management. Using the National Spina Bifida Patient Registry (NSBPR), the authors tested the association between sociodemographic variables and odds of undergoing neurosurgical procedures. METHODS: The authors extracted sociodemographic, clinical, and neurosurgical procedure data on participants with MMC aged ≥ 1 year who visited an NSBPR clinic between 2009 and 2020. The zip code of the participant's residence at the time of the last spina bifida clinic visit was linked to the Distressed Communities Index (DCI) tier. Multivariate models were built to identify factors associated with undergoing CSF diversion, shunt revision, tethered cord release (TCR), and Chiari decompression. RESULTS: There were 7924 participants with a median visit age of 13 years (IQR 7-20 years); 49.1% were male, 30.2% were non-Hispanic Black or Hispanic, 54.5% had public/supplemental insurance, and 16.9% were from distressed communities. CSF diversion, shunt revision, TCR, and Chiari decompression were performed in 81.8%, 47.7%, 22.9%, and 8.7% of participants, respectively. In multivariate analyses controlling for age, sex, insurance, DCI tier, lesion level, and surgical closure timing, Hispanic individuals were less likely than their non-Hispanic White counterparts to undergo shunt revision (p = 0.013). Non-Hispanic Black and Hispanic individuals were less likely to undergo TCR (p < 0.001 each) or Chiari decompression (p < 0.001 each). Compared with privately insured individuals, publicly insured individuals were more likely to undergo CSF diversion (p = 0.031). Those in distressed communities had increased odds of undergoing CSF diversion (p = 0.004) than those in prosperous communities. CONCLUSIONS: Among individuals with MMC participating in the NSBPR, there were differences in receiving neurosurgical procedures by race/ethnicity, insurance type, and DCI tier. Additional prospective studies are necessary to elucidate the reasons for these variations and their impact on long-term outcomes for this patient population in order to created targeted interventions.

Tethered cord prevalence among patients with prenatal or postnatal myelomeningocele repair.

BACKGROUND: Myelomeningocele (MMC) is a severe form of spina bifida characterized by spinal cord extrusion into a cerebrospinal fluid (CSF) filled sac which may lead to lifelong disability. Repair of these lesions have classically occurred shortly after birth, but more recently, prenatal myelomeningocele repair techniques have been elucidated. This study aimed to investigate the outcome of surgery, particularly with subsequent spinal cord tethering, in patients with prenatal myelomeningocele closure and those with postnatal repair surgery. MATERIALS AND METHODS: In this retrospective study which assessed patients from April 2002 to April 2020, the data of 18 total patients with MMC were reviewed. Nine patients from each group were included, closed prenatally or postnatally, respectively. Demographic information including age and sex, birth week, infant comorbidities, presence of dermoid or lipoma, cutaneous stigmata, total number and timing of detethering procedures, presence of Chiari malformation, and need for VPS was obtained. RESULTS: Eighteen patients including nine infants closed prenatally and nine infants with myelomeningoceles closed postnatally were reviewed. The mean age was 4 ± 3 years and 6.22 ± 2.4 in prenatal and postnatal retrospectively. There was a significant relationship between the number of detethering procedures (p-value = 0.03) and the need for a ventriculoperitoneal shunt (VPS) (p-value = 0.01) between the groups, with the prenatal closure group having lower rates of each. There was no significant difference between the groups in regard to the mean age at the detethering procedure (p = 0.4), sex (p = 0.09), birth week (p = 0.8), comorbidities (p = 0.8), presence of intraspinal dermoid or lipoma (p = 0.09), presence of cutaneous stigma (p = 0.08), Chiari (p = 0.6), fatty filum (p = 0.08), syrinx (p = 0.4), bone anomaly (p = 0.4), and spina bifida neurological scale (p = 0.66). CONCLUSION: There was a significant relationship between the two groups in terms of the number of detethering procedures, and the need for VPS. Our data represents a possible difference in the need for detethering surgeries and ventriculoperitoneal shunt placements between patients with prenatal and postnatal myelomeningocele closures.

Gestational Diabetes in Women with Fetal Spina Bifida Repair-Influence of Perioperative Management.

Background/Objectives: Fetal spina bifida (fSB) is the most common neural tube defect, and intrauterine repair has become a valid treatment option for selected cases. If fSB repair is offered, the ideal time for surgery is from 24 to 26 gestational weeks (GWs). The preoperative steroids for lung maturation and preoperative tocolytics that are administered are known to increase the prevalence of gestational diabetes (GD), which normally occurs in about 10-15% of all pregnant women. This study assessed the prevalence, possible influencing factors, and consequences on the course of pregnancy regarding GD in this cohort. Methods: Between 2010 and 2022, 184 fSB cases were operated. Those patients operated on after 24 0/7 GWs received steroids before surgery. All the patients received tocolysis, and an oral glucose tolerance test was performed between 26 and 28 GWs at least 7 days after steroid administration. In 2020, we established an early postoperative mobilization protocol. The perioperative management procedures of those patients with and without GD were compared to each other, and also, the patients treated according to the early mobilization protocol were compared to the remaining cohort. Results: Nineteen percent were diagnosed with GD. Corticosteroids were administered in 92%. Neither the corticoid administration nor the interval between the administration and glucose tolerance test was different in patients with or without GD. Further, 99.5% received postoperative tocolytics for at least 48 h. The women with GD had significantly longer administration of tocolytics. The length of stay (LOS) was higher in those patients with GD. The gestational age (GA) at delivery was significantly lower in the cohort with GD. In the early mobilized group, we found a significantly higher GA at delivery (37.1 GWs vs. 36.2 GWs, p = 0.009) and shorter LOS (p < 0.001), and their GD rate was lower (10% vs. 20%), although not statistically significant. Conclusions: The GD incidence in the women after fSB repair was higher than in the usual pregnant population. Early mobilization, rapid tocolytics decrease, and shorter LOS could benefit the pregnancy course after fSB repair and may decrease the risk for GD in this already high-risk cohort without increasing the risk for preterm delivery.

Memory in Spina Bifida, from Childhood to Adulthood: A Systematic Review.

Background: Spina bifida (SB) is a rare congenital disease characterized by not only physical but also neuropsychological disturbances. Among these neuropsychological impairments, memory deficits are a significant concern, as they substantially hinder aspects of crucial importance in the lives of individuals with SB such as medical needs or daily life activities. The main objective is to conduct a systematic review of the current evidence on the memory deficits in the SB population, including children, adolescents, and adults. Methods: Four databases (PubMed, SCOPUS, Web of Science, and ProQuest) were systematically screened for eligible studies. Results: The present review reveals cognitive difficulties in different memory types among individuals with SB. These deficits, identified in childhood, seem to persist into adulthood. Specifically, impairments are evident in short-term memory, working memory, and long-term memory. The neuropsychological instruments applied in the studies that were included in this systematic review vary, however, most reach the same conclusions. Conclusions: The present findings underscore the importance of incorporating cognitive assessments, particularly those focused on the memory domain, into routine childhood evaluations for individuals with SB. Early identification of these cognitive difficulties allows for the timely implementation of cognitive interventions that could leverage the inherent plasticity of the developing brain, and prevent or delay the onset of these deficits in later adulthood for people with SB, ultimately improving their functionality and quality of life.

Evidence based clinical practice guideline for follow-up care in persons with spinal cord injury.

Introduction: While it is well-established that follow-up care programs play a crucial role in preventing and early detecting secondary health conditions (SHCs) in persons with spinal cord injury [SCI, including spina bifida (SB)], the availability of evidence-based follow-up care programs remains limited. Under the leadership of the German-speaking Medical Society for Paraplegiology (DMGP), we have developed an evidence based clinical practice guideline for follow-up care of SHCs in persons with SCI and identify research gaps. Methods: This guideline was developed in accordance with the regulations of the Association of the Scientific Medical Societies in Germany (AWMF e.V.). To ensure an evidence-based guidance, we utilized the International Classification of Functioning, Disability and Health (ICF) generic core set and ICF Core Set for individuals with SCI in long-term context as our foundational framework. We conducted a comprehensive literature review to identify existing recommendations for follow-up care and graded the level of evidence according to relevant instruments. Subsequently, we formulated recommendations and achieved consensus through a structured nominal group process involving defined steps and neutral moderation, while adhering to the criteria outlined in the German guideline development instrument (DELBI). Results: Although there is a fair number of literatures describing prevalence and severity of SHCs after SCI, the amount of literature including recommendations was low (19 for SCI and 6 for SB). Based on the current evidence on prevalence and severity of SHCs and available recommendations, a clinical practice guideline on follow-up care of most relevant SHCs was defined. The recommendations for follow-up care are described in the following chapters: (1) Nervous system; (2) (Neuropathic) pain; (3) Cardiovascular diseases; (4) Respiratory System; (5) Immunological system, vaccination and allergies; (6) Gastrointestinal tract and function; (7) Endocrinological system and nutrition; (8) Urogenital system; (9) Contraception, pregnancy, birth and postpartum care; (10) Musculoskeletal system; (11) Pressure injuries; (12) Psychological health; (13) Medication and polypharmacy. Conclusion: We could successfully establish an evidence based clinical practice guideline for follow-up care of SHCs in individuals with SCI. There is however a notable lack of high-quality recommendations for SCI follow-up care.

Simultaneous tubeless supine percutaneous nephrolithotomy and cystolitholapaxy in a patient with spina bifida.

Spina bifida is a congenital condition that often leads to significant urological complications, including an increased risk of kidney and bladder stones. Performing percutaneous nephrolithotomy (PCNL) on patients with spina bifida presents unique challenges due to the anatomical deformities. We present a case of a spina bifida patient with right staghorn stone and bladder stones in a previously augmented bladder. Simultaneous tubeless supine PCNL and cystolitholapaxy was successfully performed. This case highlights the versatility of the supine position in managing complex stones in patients with spina bifida.

Growing Up With Neurogenic Bladder: Navigating the Challenges and Controversies in Pediatric to Adult Transition and Lifelong Care: A Report From the Neurogenic Bladder Research Group (NBRG).

OBJECTIVE: Young adults with spina bifida (and other congenital neurologic diseases) have their own aspirations that may include education, employment, relationships and having children. As they move from pediatric to adult care, they must eventually transition to an adult healthcare team. The objective of this paper is to review the challenges and controversies in the transitional and adult care of people with congenital neurourological diseases. METHODS: The Research Group convened a meeting of its members and invited guests to better understand the healthcare challenges faced by these patients as they become adults. The group examined potential research opportunities focused on people with these diagnoses and themes related to their healthcare. RESULTS: Trust and clear communication are essential for effective patient transition. Ideally parents are involved in the transition to help reinforce independent self-care and responsibility. Adolescents require education about sexual health and independence, which may not be part of the core skillset of a urologist. The healthcare team must promote self-management and autonomy as early as practical. One of the major limitations is that adult care lacks the coordination of pediatric care, and patients may not have a "medical home." Multidisciplinary clinics are ideal but face logistical barriers in adult medicine. Additional barriers include limited physicians with the required specialized training. In the adult system, financial constraints are a key challenge for patients and providers. CONCLUSION: Collaboration, supported by institutions and new research, is vital for improving the neurourological care of young adults with complex diseases. TRIAL REGISTRATION: This study reports on the proceedings of a meeting, and therefore clinical trial registration was not necessary.

Application of Bioelectrical Impedance Analysis in Weight Management of Children with Spina Bifida.

BACKGROUND: Children with spina bifida (SB) face an elevated risk of obesity, which necessitates precise methods for assessing body composition to ensure effective weight management. Conventional measures like BMI are inadequate for this population because of variations in growth patterns and skeletal structure. Bioelectrical impedance analysis (BIA) is a method that offers a clearer picture of body composition, yet its use in children with SB remains underexplored. METHODS: Conducted on 57 children with SB and 28 healthy controls, with a median age of 11 years, this study evaluated anthropometrics, including BMI and BIA-derived metrics. The Hoffer's scale to assess physical activity was applied in SB children. RESULTS: Results showed that while 32% of SB patients were classified as overweight or obese based on BMI, 62% exhibited high body fat percentage via BIA. Fat-free mass, muscle and fat mass, and fat-to-muscle ratio (FMR) differed significantly compared to the reference group. Non-ambulators showed a higher median body fat mass percentage (25.9% vs. 17.8%, p = 0.01) and FMR (0.92 vs. 0.44, p = 0.003) in comparison to the community walkers. CONCLUSIONS: In SB children, BIA-measured fat mass is a better obesity indicator than BMI. Non-ambulatory, SB patients with obesity had the highest FMR values, indicating a higher risk for metabolic syndrome.

Inclusive measure development: amplifying the voices of adolescents and young adults with spina bifida in a new measure of benefit-finding and growth.

OBJECTIVE: Benefit-finding and growth is an important process across a range of medical populations. However, it has been understudied in the context of lifelong chronic conditions, such as spina bifida (SB). This study aimed to develop a new measure of benefit-finding and growth for youth with SB, confirm its factor structure, and examine its psychometric properties. METHOD: To generate items for the new measure, 20 adolescents and young adults with SB completed qualitative interviews regarding their experience of living with SB. Interviews were coded for benefits. Questionnaire items were generated from these benefits, and an expert panel refined the wording of these items. The resultant 31-item measure was shared with six of the 20 participants for feedback and then piloted among 251 youth with SB. The factor structure of the measure was confirmed and reliability and convergent validity were assessed. RESULTS: Both a one- and four-factor structure were supported. The four factors include: Life Perspectives and Priorities, Personal Characteristics and Traits, Connections and Opportunities, and Problem Solving. Higher total and factor scores represent greater benefit-finding and growth. The measure demonstrated excellent internal consistency (α = 0.95). The new measure also showed significant positive correlations with optimism, positive affect, and life satisfaction. CONCLUSIONS: This study produced a measure of benefit-finding and growth for youth with SB. Clinically, information about what youth with SB perceive to be their areas of strength and growth from their condition provides crucial insight into which factors to enhance in this population.

Lumbosacral tuft of hair as a key stigma for early detection of occult spinal dysraphism: A case report of diastematomyelia with tethered cord in a newborn.

Early detection of occult spinal dysraphism, such as diastematomyelia with tethered cord, is vital to prevent neurological damage. Though rarely diagnosed in the neonatal period, cutaneous stigmata can aid early identification. Utilizing neonatal ultrasound enables timely management and multidisciplinary intervention.

Dwelling proximity to wildfire and spina bifida risk in offspring.

OBJECTIVE: To evaluate the association between wildfire exposure in pregnancy and spina bifida risk. METHODS: This retrospective cohort study used the California Office of Statewide Health Planning and Development Linked Birth File with hospital discharge data between 2007 and 2010. The Birth File data were merged with the California Department of Forestry and Fire Protection data of the same year. Spina bifida was identified by its corresponding ICD-9 code listed on the hospital discharge of the newborn. Wildfire exposure was determined based on the zip code of the woman's home address. Pregnancy was considered exposed to wildfire if the mother lived within 15 miles of a wildfire during the pregnancy or within 30 days prior to pregnancy. RESULTS: There were 2,093,185 births and 659 cases of spina bifida between 2007 and 2010. The births were analyzed using multivariable logistic regression models and adjusted for potential confounders. Exposure to wildfire in the first trimester was associated with higher odds of spina bifida (aOR= 1.43 [1.11-1.84], p-value = 0.01). Wildfire exposure 30 days before the last menstrual period and during the second and third trimesters were not associated with higher spina bifida risk. CONCLUSION: Wildfire exposure has shown an increased risk of spina bifida during the early stages of pregnancy.

The impact of transition of Spina Bifida Care: A retrospective population-based cohort study comparing outcomes and health care costs longitudinally from adolescence into adulthood.

Introduction: Transition from multidisciplinary paediatric to adult care results in a significant change in the intensity of care provided to patients with spina bifida (SB). Objective: To compare planned and unplanned health care utilization and costs of care of transitioning SB patients. Study Design: Through a retrospective population-based cohort study, patients with SB born in Ontario between 1988 and 1999 were studied using routinely collected data analysed at ICES. Patients were followed longitudinally from age 16 to 22 years with comparisons made between the paediatric baseline year (age 16) to the first 3 years of adulthood (ages 19 to 21). A composite primary outcome of unplanned emergency room (ER) visits, hospitalizations and emergency surgical procedures, was compared before and after transition. Secondary outcomes included these variables individually and estimated health care costs. Results: In the 299 patients, no significant differences were identified in the composite primary outcome between periods (P = 0.09). Unplanned ER visits (P = 0.004) and emergency surgical procedures (P = 0.02) increased after transition. Despite this, the estimated individual total cost of care declined after transition (P = 0.03). Further, multivariable analysis identified rural residence (RR 1.78, confidence interval [CI] 1.30 to 2.44) and previous urologic surgery (RR 1.91, CI 1.41 to 2.57) as significant predictors of increased need for emergent care and health care costs, respectively. Conclusion: Patients with SB in Ontario have higher rates of ER usage and unplanned surgery after the transition to adult care. While this does not drive an increase in health care costs, improvements in the care of transitioned SB patients, particularly rural patients and those with prior urologic surgery, may decrease the requirement for acute care.

Infection control in schools for learners with spina bifida: A scoping review.

Background: Despite the widespread prevalence of spina bifida and its impact on individual learners, there is limited information on how infection control is managed in the school environment. Objective: This scoping review was conducted to map the evidence on infection control programmes at schools for learners with spina bifida (LSB) globally. Methods: This review followed the Joanna Briggs Institute methodology for the scoping review. A database search was conducted on an internet browser search on MEDLINE, PUBMED, EBSCOhost, Google Scholar, ERIC and Google search engines. Results: A total of five articles met the inclusion criteria. Most of the schools in the study did not have special facilities or running water to accommodate basic infection control procedures. Conclusion: Collaboration between policymakers, specialists of spina bifida and infection control in the health sector, the Association of Spina Bifida, specialists in spina bifida under the Department of Education and researchers is needed to improve the lives of LSB through infection control. Contribution: The study will contribute to the improvement of training of staff working with LSB and the need for more research.

Use of 'stacked' dermal template: Biodegradable temporising matrix to close a large myelomeningocele defect in a newborn.

Background: Myelomeningocele is a severe and complex congenital malformation of the central nervous system. Failure of neural tube closure at around four weeks of gestation results in an open communication between the neural placode and the external environment with varied functional impairment. Surgery is usually required. Objectives: The primary goals of surgical management are to preserve neural function and minimise infection. Reconstruction is dependent upon the site and size of the defect as well as the quality of the surrounding soft tissues. Surgeons may employ a range of reconstructive techniques in order to achieve closure. Skin substitutes, also known as dermal regeneration templates, have also been utilised. Discussion: In our unit, we use NovoSorb Biodegradable Temporising Matrix to reconstruct full-thickness skin and soft tissue defects. It is a synthetic, biodegradable, dermal regeneration template, composed of polyurethane foam bonded to a transparent sealing membrane and typically requires a two stage reconstruction. Integration and vascularisation take approximately three weeks. After this time, the recipient wound bed is suitable for split thickness skin grafting. A further benefit of dermal regeneration templates is the possibility of 'stacking' layers, which serves to increase the thickness of the final construct and to minimise overall contour defects. The authors present the case of a one-day-old full-term neonate with a large lumbosacral myelomeningocele that was successfully managed with staged, stacked NovoSorb Biodegradable Temporising Matrix and split thickness skin grafting. The authors believe this is the first case in which a 'stacked' dermal regeneration templates has been used to achieve healing of a primary myelomeningocele defect. Lay Summary: Background: NovoSorb Biodegradable Temporising Matrix (BTM) is a dermal regeneration template (DRT) and is used to reconstruct wounds following full-thickness skin and soft tissue loss resulting from burn injury, trauma, infection or surgery. It is composed of 2-millimetre thick, synthetic, biodegradable polyurethane foam bonded to a transparent (non-biodegradable) sealing membrane. Like all DRTs, it acts as a scaffold for cellular integration and vascularisation to eventually form a 'neo-dermis'. This is usually apparent from around three weeks. A second stage procedure can then be performed, with removal of the outer sealing membrane and split thickness skin grafting of the vascularised layer.Objectives: Myelomeningocele is a severe and complex congenital malformation of the central nervous system and forms the group of anomalies commonly referred to as neural tube defects (NTDs). Neural tube closure usually occurs at around four weeks of gestation and failure to do so, results in an open communication between the neural placode and the external environment. The degree of functional impairment varies but can include: lower limb paralysis; sensory loss; bladder and bowel dysfunction. In order to preserve neural function and minimise the risk of infection, surgery is usually required to close the defect. Reconstruction is varied and is dependent upon the site and size of the defect as well as the quality of the surrounding soft tissues. The use of local flaps has the potential complication of skin necrosis. Muscle based flaps may be debilitating and limit future functionality and worsen postural development. We were presented with a one-day-old neonate with a large lumbosacral myelomeningocele. A DRT (NovoSorb BTM) was selected as the primary reconstruction. Firstly, selection provided relatively low risk, with minimal morbidity and preserved the full complement of flap based reconstructive options for a later stage should instrumentation be required. Secondly, NovoSorb BTM conferred a robust seal over the dural repair with no demonstrable cerebrospinal fluid leak. Thirdly, the ability to add layers ('stack') of NovoSorb BTM in stages, once integration and vascularisation of the previous layer is complete, allows reconstruction of deeper contour defects.Discussion: We have illustrated the successful use of NovoSorb BTM as a DRT to achieve closure of a large lumbosacral myelomeningocele without complication and with longstanding stability. We believe this technique provides reconstructive teams with an alternative option that is effective, safe and reproducible and which spares local tissues for future elective reconstructive procedures, should they be required.

Neurosurgical Advocacy in the Prevention of Neural Tube Defects: Impacting Global Fortification Policies Through Leadership, Collaboration, and Stakeholder Engagement.

The G4 Alliance and its member organizations formed a delegation that participated in the 76th World Health Assembly (WHA) in 2023, which unanimously adopted the resolution to address micronutrient deficiencies through safe, effective food fortification to prevent congenital disorders such as spina bifida and anencephaly, the first neurosurgery-led resolution since the founding of the World Health Organization. The WHA included other resolutions and side events by the G4 Alliance and other organizations relevant to neurosurgery. An opportunity exists for neurosurgeons to harness the momentum from this resolution to promote initiatives to prevent neurosurgical disease or expand access to neurosurgical care.

Associations between exposure to extreme ambient heat and neural tube defects in Georgia, USA: A population-based case-control study.

INTRODUCTION: The association between extreme ambient heat exposures during pregnancy and neural tube defects (NTDs) in offspring remains unclear. This study sought to estimate the association between exposure to extreme ambient heat during periconception and NTDs. METHODS: This population-based case-control study in Georgia, USA (1994-2017) included 825 isolated NTD cases (473 anencephaly, 352 spina bifida) and 3,300 controls matched 1:4 on county of residence and time period of delivery. Daily ambient temperature data were linked to fetal death and birth records by county of residence. Extreme ambient heat exposure was defined as the number of consecutive days the daily apparent temperature exceeded the county-specific 95th percentile (derived over 1980-2010) during an eight-week periconception period. We calculated adjusted odds ratios (aORs) and 95% confidence intervals (CI) using conditional logistic regression models adjusted for maternal age, education, and ethnicity and month and year of last menstrual period. RESULTS: The aORs for NTDs were 1.09 (95% CI 1.01, 1.17), 1.18 (95% CI 1.03, 1.36), and 1.29 (95% CI 1.04, 1.58) for exposure to 1-2, 3-5, and 6 or more consecutive days with apparent ambient temperatures exceeding the county-specific 95th percentile during periconception, respectively, compared to no days of extreme ambient heat exposure. Weekly analysis of extreme heat exposure indicated consistently elevated odds of offspring NTDs during periconception. These results were largely driven by spina bifida cases. CONCLUSIONS: Our results highlight potential health threats posed by increasing global average temperatures for pregnant people with implications for increased risk of neural tube defects in their offspring.