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\"An American-Chinese partnership to prevent spina bifida and related birth defects succeeded through old-fashioned field epidemiology, advocates with influence, and genuine collaboration based on personal relationships built on trust, respect, and shared proboblem-solving\"--Provided by publisher.

Background. Birth defects remain a significant source of worldwide morbidity and mortality. Strong scientific evidence shows that folic acid fortification of a region’s food supply leads to a decrease in spina bifida (a birth defect of the spine). Still, many countries around the world have yet to approve mandatory fortification through government legislation. Objectives. We sought to perform a systematic review and meta-analysis of period prevalence of spina bifida by folic acid fortification status, geographic region, and study population. Search methods. An expert research librarian used terms related to neural tube defects and epidemiology from primary research from 1985 to 2010 to search in EMBASE and MEDLINE. We searched the reference lists of included articles and key review articles identified by experts. Selection criteria. Inclusion criteria included studies in English or French reporting on prevalence published between January 1985 and December 2010 that (1) were primary research, (2) were population-based, and (3) reported a point or period prevalence estimate of spina bifida (i.e., prevalence estimate with confidence intervals or case numerator and population denominator). Two independent reviewers screened titles and abstracts for eligible articles, then 2 authors screened full texts in duplicate for final inclusion. Disagreements were resolved through consensus or a third party. Data collection and analysis. We followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses, or PRISMA, abstracting data related to case ascertainment, study population, folic acid fortification status, geographic region, and prevalence estimate independently and in duplicate. We extracted overall data and any subgroups reported by age, gender, time period, or type of spina bifida. We classified each period prevalence estimate as “mandatory” or “voluntary” folic acid fortification according to each country’s folic acid fortification status at the time data were collected (as determined by a well-recognized fortification monitoring body, Food Fortification Initiative). We determined study quality on the basis of sample representativeness, standardization of data collection and birth defect assessment, and statistical analyses. We analyzed study-level period prevalence estimates by using a random effects model (α level of < 0.05) for all meta-analyses. We stratified pooled period prevalence estimates by birth population, fortification status, and continent. Results. Of 4078 studies identified, we included 179 studies in the systematic review and 123 in a meta-analysis. In studies of live births (LBs) alone, period prevalences of spina bifida were (1) lower in geographical regions with mandatory (33.86 per 100 000 LBs) versus voluntary (48.35 per 100 000 LBs) folic acid fortification, and (2) lower in studies of LBs, stillbirths, and terminations of pregnancy in regions with mandatory (35.22 per 100 000 LBs) versus voluntary (52.29 per 100 000 LBs) fortification. In LBs, stillbirths, and terminations of pregnancy studies, the lowest pooled prevalence estimate was in North America (38.70 per 100 000). Case ascertainment, surveillance methods, and reporting varied across these population-based studies. Conclusions. Mandatory legislation enforcing folic acid fortification of the food supply lags behind the evidence, particularly in Asian and European countries. This extensive literature review shows that spina bifida is significantly more common in world regions without government legislation regulating full-coverage folic acid fortification of the food supply (i.e., Asia, Europe) and that mandatory folic acid fortification resulted in a lower prevalence of spina bifida regardless of the type of birth cohort. African data were scarce, but needed, as many African nations are beginning to adopt folic acid legislation.

Aim  The morbidity associated with osteoporosis and fractures in children and adolescents with spina bifida highlights the importance of osteoporosis prevention and treatment in these patients. The aim of this study was to examine the occurrence and pattern of bone fractures in paediatric patients with spina bifida. Method  We reviewed the data of all paediatric patients with spina bifida who were treated in our centre between 1999 and 2008. Results  One hundred and thirteen patients were included in the study (63 females, 50 males; mean age 10y 8mo, SD 4y 10mo, range 6mo–18y). The motor levels were thoracic in six, upper lumbar in 22, lower lumbar in 42, and sacral in 43 patients. Of the 113 patients, 58 (51.3%) had shunted hydrocephalus. Thirty‐six (31.8%) were non‐ambulatory (wheelchair–dependent [unable to self‐propel wheelchair] n=3, wheelchair‐independent [able to self‐propel wheelchair] n=33), 13 were partial ambulators, 61 were full ambulators, and three were below the age of walking. Forty‐five fractures were reported in 25 patients. The distal femur was the most common fracture site. Statistical analyses showed that patients with higher levels of involvement and in wheelchairs had a significantly increased risk of having a second fracture (p<0.001). Spontaneous fractures were the principal mechanism of injury, and an association was identified between fracture mechanism, type of ambulation, and lesion level: the fractures of patients with higher levels of motor functioning and those in wheelchairs were mainly pathological (p=0.01). We identified an association between risk of a second fracture, higher motor level lesion, and non‐ambulation. There was an increased risk of having a second fracture after a previous spontaneous fracture (p=0.004). Interpretation  Data in this study indicate a high prevalence of fractures in patients with spina bifida.

Background Spinal dysraphism is a congenital condition requiring lifelong management to prevent long-term complications. Our institution is a referral center for congenital malformations in the Indian Ocean, particularly serving Réunion Island, Mayotte, Madagascar, and the Comoros. The aim of this study was to describe a cohort of children with spinal dysraphism followed at our center. Methods This retrospective cohort study included consecutive patients diagnosed with spinal dysraphism who were born between January 1, 2006 and December 31, 2022, and followed at our institution. Data were extracted from electronic records and covered the antenatal period, delivery, initial management and follow-up. Results Over the 17 years of the study period, 121 patients were included. The annual mean incidence was 2.1/10,000 live births and remained stable throughout the study period. Among the 121 patients, 82 had a prenatal diagnosis, and 69 underwent a proactive management. Proactively managed patients originated from Réunion Island ( n  = 41), Mayotte ( n  = 12), the Comoros ( n  = 12) and Madagascar ( n  = 3). Surgical closure was performed in all patients with open defects, and a ventricular shunt was used in half of the patients. The median follow-up duration was 4.2 years. The mortality rate was 4.3%, and 42% of patients were lost to follow-up. At least one clinical review was conducted by a neurosurgeon in 85% of cases, a urologist in 71%, or an orthopedic specialist in 49%. Neurodevelopmental delay was observed in all patients who required a shunt placement. Ambulation was achieved in 39% of the patients. Conclusions The incidence of spinal dysraphism remained stable over time in our population. The monitoring of patients was not optimal, but the remote origin of some of our patients must be taken into account. Further studies should be performed to evaluate continence and long-term complications.

Neural tube defects are the major causes of fetal loss and considerable disabilities in infants. Currently, there is no significant research on the incidence of Neural tube defects in the Tigray region of Ethiopia. To determine the incidence and clinical pattern of the Neural Tube Defects. A hospital-based cross-sectional study was conducted from October 2016 to June 2017. All pregnancy outcomes were examined for any externally visible birth defects and neurological integrity by trained midwives under the supervision of senior obstetrics and gynecology and a neurosurgeon. Data were collected using a survey tool to collect maternal and newborn demographic data and a checklist developed to capture newborns with Neural Tube Defects. Data were analyzed using SPSS version 20. The prevalence of NTDs was calculated per 10,000 births. Out of the 14,903 births during the study period, a total of 195 infants were born afflicted with Neural Tube Defects. The burden of infants with anencephaly and spina bifida was 66.4 and 64.4 per 10, 000 births, respectively. The overall incidence rate of NTDs in this study was 131 per 10, 000 births of which 23% were liveborn and 77% were stillborn. The highest burden of Neural Tube Defects was observed in Adigrat Hospital from Eastern Zone of Tigray (174 per 10,000 births) and Lemlem Karl Hospital from Southern Zone of Tigray (304 per 10,000 births) compared to Kahsay Abera Hospital from Western Zone (72.8 per 10,000 births) and Sihul Hospital from North Western Zone of Tigray (69.8 per 10,000 births). Assuming that the non folic acid preventable rate should be 5 per 10,000 births, our prevalence rate is 131 per 10,000 births, and then we have a rate or an epidemic that is 26 times what it should be. This just emphasizes the urgency to implement effective programs to get all women of reproductive age to have adequate folic acid to prevent all of folic acid-preventable spina bifida and anencephaly, which would prevent 96% (125/130) of spina bifida and anencephaly in the Tigray Provence.

Background Spinal dysraphism (SD) requires lifelong multidisciplinary follow-up to prevent complications and optimize functional outcomes. In geographically fragmented healthcare systems, continuity of care may be challenging, particularly when patients cross regional or national borders to access specialized services. This study aimed to describe care trajectories and identify factors associated with optimal follow-up among children with SD managed in the southwestern Indian Ocean region. Methods We conducted a retrospective cohort study including children with SD who received active postnatal surgical management in the regional referral center of Reunion Island. Maternal, perinatal, clinical, and geographic characteristics were collected from hospital records. Optimal follow-up was defined according to the institutional multidisciplinary follow-up protocol. Results A total of 92 mother–infant dyads were included. Most pregnancies originated in Reunion Island (62.0%), followed by Mayotte (18.5%), the Comoros (16.3%), and Madagascar (3.3%). Prenatal diagnosis was established in 47.8% of pregnancies but varied substantially across territories. Surgical closure of the spinal defect was performed at a median age of 3 days, and ventriculoperitoneal shunt placement was required in 44.6% of patients. Overall, 39 patients (42.4%) were lost to follow-up. Optimal follow-up was achieved in only 13 patients (14.1%). Geographic factors were strongly associated with follow-up outcomes. Patients residing in Reunion Island were significantly more likely to achieve optimal follow-up than those living outside the island ( p  = 0.04). In contrast, clinical characteristics—including lesion level, surgical timing, ventricular shunt placement, and functional outcomes—were not associated with follow-up status. Conclusion Continuity of care for children with SD in the southwestern Indian Ocean region is strongly influenced by geographic and health system factors. Despite successful initial surgical management, long-term follow-up remains limited for many patients living outside Reunion Island. Strengthening regional healthcare coordination and cross-border care pathways may help reduce disparities in access to long-term multidisciplinary care.

Purpose Early recognition is crucial for occult spinal dysraphism associated with congenital spinal deformities. There is limited literature available on its occurrence in congenital scoliosis and kyphosis in the Indian population. Methods Our study involved a retrospective review of 247 children who presented at a single centre. We analyzed their demographics and clinical and radiological findings, which included the type of deformity, its location, vertebral anomaly, Cobb angle, and MRI findings. The deformities were categorized as congenital scoliosis or congenital kyphosis with failure of formation, failure of segmentation, or both. Results A total of 247 cases were examined (congenital scoliosis—229, congenital kyphosis—18). The average age was seven years (range 0.8 to 19 years, SD 4.6). The mean Cobb angle at presentation in the congenital scoliosis group was 49.4° (range 8 to 145°, SD 23.77) for those with abnormal MRI and 42.45° (range 5 to 97°, SD 20.09) for those with normal MRI. For the congenital kyphosis group, the mean K angle at presentation was 47.7° (range 14 to 110°, SD 33.33) for those with abnormal MRI and 47.36° (range 15 to 70°, SD 16.63) for those with normal MRI. Abnormal MRI results were observed in 130 of the patients (congenital scoliosis—53.7%, congenital kyphosis—38.8%). The highest incidence of abnormal MRI findings was observed in the failure of segmentation (66.6%) and mixed (65%) types. Deformities in the dorsal region had the highest incidence (61.9%). The most common dysraphism instances were diastematomyelia and tethered cord. There was a significant correlation between type of deformity and presence of dysraphism. Conclusion This is the largest case series of congenital scoliosis and kyphosis reported from India. We found a high incidence of occult spinal dysraphism as compared to other published series. Occult spinal dysraphism is more common in the thoracic region. Diastematomyelia followed by tethered cord was the most common anomaly observed. We recommend MRI screening of whole spine and craniovertebral junction.

Introduction In Ethiopia approximately 3,200,000 babies are born annually and 41.09 per 10,000 live births are affected by spina bifida. Hydrocephalus (HCP) is another common pediatric neurosurgical condition with studies in Ethiopia showing the most common etiology is post spina bifida closure. The out-of-pocket expense (OOPE) and indirect expense of patients treated surgically for spina bifida and hydrocephalus during the first year of life were assessed. Methods A prospective hospital-based study was done on patients treated surgically for spina bifida and HCP in two university-affiliated hospitals, between April 1st, 2022, and April 1st, 2023. Data on direct and indirect expenses were collected during inpatient care and follow-up. Catastrophic health expenditure (CHE) was assessed, defined as total expenditure exceeding 10% of the total annual household expenditure. Result A total of 245 patients were eligible for analysis. The median annual total expenditure of households for treatment was ETB 11,510.00 with ETB 5700.00 being indirect expenditure. Forty-nine percent of the households suffered CHE. In multivariate analysis, the factors which were found to have a statistically significant association with CHE were the hospital where the patient received the treatment, the household’s wealth quintile, the place of residency, and pre-admission duration of stay. Conclusion Our study revealed a high CHE in households with spina bifida and HCP. We recommend working on primary prevention of spina bifida, expanding surgical services regionally to minimize costs associated with travel for surgical care, and reducing pre-admission duration of stay by improving evaluation and investigations at outpatient clinics.

Globally, 180 million young people aged 10-24 live with a physical or mental health disability. Their rights to sexual and reproductive health have been denied often overshadowed by the societal negative knowledge, beliefs, and attitudes. This study sough to explore sexual and reproductive health challenges among adolescents and young people with spina bifida and hydrocephalus disability in Uganda. This was an exploratory community based cross-sectional qualitative study among adolescents in Uganda. We conducted 60 semi structured interviews, 30 with Adolescents and young people with spina bifida and hydrocephalus and 30 with caregivers between March 2021 and February 2022 to explore their sexual and reproductive health challenges. Through thematic analysis, we identified common themes across the interviews regarding their challenges. Participants reported a number of sexual and reproductive health challenges including sexual violence and abuse, incontinence and stigma, Inability to feel sexually empowered, other adolescents are coerced to take birth control methods without their informed decision. Negative Socio-cultural beliefs, lack of and inaccessible sexual reproductive health information and poor menstrual health management. Adolescents and young people with spina bifida experience a number of sexual and reproductive challenges that are not given attention and often overshadowed by the negative knowledge, beliefs, attitudes, and practices within the society. Therefore, there is need to develop and implement programs and awareness campaigns aimed for the empowerment of individuals with SB to seek knowledge and skill building regarding sexual and reproductive health.

ObjectivesTo determine the baseline trends in the total birth prevalence of neural tube defects (NTDs) in England (2000–2019) to enable the impact of folic acid fortification of non-wholemeal wheat flour to be monitored.DesignPopulation-based, observational study using congenital anomaly (CA) registration data for England curated by the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS).SettingRegions of England with active registration in the time period.ParticipantsBabies that were liveborn or stillborn and pregnancies that resulted in a termination of pregnancy or a late miscarriage (20–23 weeks’ gestation) with an NTD.Main outcome measuresTotal birth prevalence of anencephaly, spina bifida and all NTDs in England. Poisson regression analysis was used to evaluate time trends with regional register as a random effect. The progress of national registration across England was assessed.ResultsThere were 4541 NTD pregnancies out of 3 637 842 births in England; 1982 anencephaly and 2127 spina bifida. NTD prevalence was 12.5 (95% CI 12.1 to 12.9) per 10 000 total births. NTD prevalence per 10 000 total births was significantly higher in 2015–2019 (13.6, 95% CI 12.9 to 14.4) compared with 2010–2014 (12.1, 95% CI 11.7 to 12.5). An increasing trend in NTDs overall was detected (incidence rate ratio (IRR) 1.01, 1.00 to 1.02), although further analysis determined this effect was confined to 2015–2019 (compared against 2000–2004, IRR 1.14, 1.04 to 1.24). The birth prevalence of anencephaly reflected this pattern. The prevalence of spina bifida remained relatively stable over time.ConclusionsBaseline NTD prevalence for England has been established. National and standardised CA registration is in place, facilitating the systematic and consistent monitoring of pre-fortification and post-fortification NTD trends and evaluating the impact of fortification on NTD prevalence.