Characteristics and Outcomes of Patients With Spina Bifida in Texas by Patient Age.

INTRODUCTION: Spina bifida (SB) occurs in 3.5/10,000 live births and is associated with significant long-term neurologic and urologic morbidity. We explored the characteristics and outcomes of pediatric patients with SB and the facilities that treat them in Texas. METHODS: We retrospectively reviewed a statewide hospital inpatient discharge database (2013-2021) to identify patients aged <18 y with SB using International Classification of Diseases 9/10 codes. Patients transferred to outside hospitals were excluded to avoid double-counting. Descriptive statistics and chi-square test were performed. RESULTS: Seven thousand five hundred thirty one inpatient hospitalizations with SB were analyzed. Most SB care is provided by a few facilities. Two facilities (1%) averaged >100 SB admissions per year (33% of patients), while 15 facilities (8%) treat 10-100 patients per year (51% of patients). Most facilities (145/193, 75%) average less than one patient per year. Infants tended to be sicker (17% extreme illness severity, P < 0.001). Overall mortality is low (1%), primarily occurring in the neonatal period (8%, P < 0.001). Most admissions are associated with surgical intervention, with 63% of encounters having operating room charges with an average cost of $25,786 ± 24,884. Admissions for spinal procedures were more common among infants, whereas admissions for genitourinary procedures were more common among older patients (P < 0.001). The average length of stay was 8 ± 16 d with infants having the longest length of stay (19 ± 33, P < 0.001). CONCLUSIONS: Patients have significant long-term health needs with evolving pediatric surgical indications as they grow. Pediatric SB care is primarily provided by a small number of facilities in Texas. Longitudinal care coordination of their multidisciplinary surgical care is needed to optimize patient care.

Mortality rates, cause and risk factors in people with spina bifida, register-based study over five decades.

AIM: Care for people with spina bifida can be improved. This may be done by evaluating mortality rates and causes of death. METHODS: Between 1973 and 2021, 1735 people with spina bifida appeared in registers of the Swedish population. Survival rates and causes of death were calculated according to age and decade. RESULTS: Over almost 50 years, the prevalence of spina bifida decreased from 5.2 to 1.2 per 10 000 births. Mortality fell sharply during the first year of life, with survival rising from 75% to 94%. For children aged 2-18 years and adults, mortality rates were low and differences between decades were minimal. Causes of childhood deaths were congenital abnormalities, hydrocephalus and infections, the latter two also in adults. Adult causes also included self-inflicted injuries and substance abuse, with suicidal or unclear intent, both more common than in the general population. Bladder malignancies were also more frequent, although after reconstructive bladder surgery, mortality rates were similar. CONCLUSION: Survival in the first year of life increased in children with spina bifida, whereas there was no difference in survival rates between adults born between 1973 and 1999. For adults, proactive prevention methods regarding self-inflicted injury, substance abuse and bladder cancer are warranted.

Neonatal and infant mortality associated with spina bifida: A systematic review and meta-analysis.

OBJECTIVES: A systematic review was conducted in high-income country settings to analyse: (i) spina bifida neonatal and IMRs over time, and (ii) clinical and socio-demographic factors associated with mortality in the first year after birth in infants affected by spina bifida. DATA SOURCES: PubMed, Embase, Ovid, Web of Science, CINAHL, Scopus and the Cochrane Library were searched from 1st January, 1990 to 31st August, 2020 to review evidence. STUDY SELECTION: Population-based studies that provided data for spina bifida infant mortality and case fatality according to clinical and socio-demographical characteristics were included. Studies were excluded if they were conducted solely in tertiary centres. Spina bifida occulta or syndromal spina bifida were excluded where possible. DATA EXTRACTION AND SYNTHESIS: Independent reviewers extracted data and assessed their quality using MOOSE guideline. Pooled mortality estimates were calculated using random-effects (+/- fixed effects) models meta-analyses. Heterogeneity between studies was assessed using the Cochrane Q test and I2 statistics. Meta-regression was performed to examine the impact of year of birth cohort on spina bifida infant mortality. RESULTS: Twenty studies met the full inclusion criteria with a total study population of over 30 million liveborn infants and approximately 12,000 spina bifida-affected infants. Significant declines in spina bifida associated infant and neonatal mortality rates (e.g. 4.76% decrease in IMR per 100, 000 live births per year) and case fatality (e.g. 2.70% decrease in infant case fatality per year) were consistently observed over time. Preterm birth (RR 4.45; 2.30-8.60) and low birthweight (RR 4.77; 2.67-8.55) are the strongest risk factors associated with increased spina bifida infant case fatality. SIGNIFICANCE: Significant declines in spina bifida associated infant/neonatal mortality and case fatality were consistently observed, advances in treatment and mandatory folic acid food fortification both likely play an important role. Particular attention is warranted from clinicians caring for preterm and low birthweight babies affected by spina bifida.

Perfil epidemiológico de mortalidade por espinha bífida / Epidemiological profile of mortality from Spina Bifida

Objetivo: Avaliar o perfil epidemiológico nacional de mortalidade por espinha bífida. Métodos: Trata-se de estudo observacional, descritivo, de série temporal, a partir de dados obtidos da plataforma eletrônica do Departamento de Informática do Sistema Único de Saúde entre os anos de 2005 e 2015. Resultados: Nesse período, as proporções de óbitos infantis relacionados à espinha bífida diminuíram. As Regiões Sul e Sudeste mantiveramse abaixo da média nacional, e as demais permaneceram acima, corroborando o maior apoio técnico nas regiões consideradas referências em saúde no país. As maiores diminuições proporcionais nos coeficientes de mortalidade infantil ocorreram no Nordeste e no Centro-Oeste, de 351,55 a 155,56 e de 809,52 a 290,32, respectivamente. Isso pôde ser justificado por essas duas regiões apresentarem maiores proporções de óbitos. Conclusão: Com a atenuação de outras causas de mortalidade infantil, as malformações evidenciaram-se. O acompanhamento pré-natal, a adoção de estilo de vida saudável pelas gestantes e a prevenção dos fatores de risco para defeitos de fechamento do tubo neural, sobretudo pela suplementação com ácido fólico, merecem destaque na redução do número de óbitos infantis e na perpetuação da vida.

Objective: To evaluate the national epidemiological profile of mortality from Spina Bifida. Methods: This is an observational, descriptive study of a time series, based on data obtained from the electronic platform of the Department of Informatics of the Unified Health System (DATASUS) between 2005 and 2015. Results: In this period, the proportions of infantile deaths related to spina bifida decreased. The Southern and Southeastern regions remained below the national average, while the others remained above, resulting in a greater technical support from the regions considered health references in the country. The largest proportional decreases in infant mortality coefficients took place in the Northeast and Midwest, from 351.55 to 155.56, and from 809.52 to 290.32, respectively. This can be justified by the fact that these two regions have higher proportions of deaths. Conclusion: Because of the attenuation of other causes of infant mortality, the malformations were evidenced. Prenatal follow-up, the adoption of a healthy lifestyle by pregnant women, and the prevention of risk factors for neural tube defects, especially through folic acid supplementation, shall be highlighted for the reduction in the number of infant deaths, and for perpetuation of life

Growing up with spina bifida: bridging the gaps in the transition of care from childhood to adulthood.

Spina bifida is the most common nonchromosomal birth defect, resulting in permanent disability of multiple organ systems, yet compatible with long-term survival. Important advances across various disciplines have now improved survival among the spina bifida population. Although the majority of individuals living with spina bifida are now adults, there are few publications in the neurosurgical literature regarding the care of adults with spina bifida, associated medical conditions, surgical interventions, and long-term complications. The major goals for transitioning adult patients with spina bifida are preservation of function and promotion of independence as well as general overall health. Nevertheless, many gaps exist in our knowledge and understanding of the complex needs of this aging patient population. The goal of this paper was to provide a comprehensive updated review of the literature regarding the challenges and considerations involved in the transitional care to adulthood for patients with spina bifida. Unique to this review, the authors provide a first-hand personal communication and interview with an adult patient with spina bifida that discusses many of these challenges with transition.

Analysis of Mortality among Neonates and Children with Spina Bifida: An International Registry-Based Study, 2001-2012.

BACKGROUND: Medical advancements have resulted in better survival and life expectancy among those with spina bifida, but a significantly increased risk of perinatal and postnatal mortality for individuals with spina bifida remains. OBJECTIVES: To examine stillbirth and infant and child mortality among those affected by spina bifida using data from multiple countries. METHODS: We conducted an observational study, using data from 24 population- and hospital-based surveillance registries in 18 countries contributing as members of the International Clearinghouse for Birth Defects Surveillance and Research (ICBDSR). Cases of spina bifida that resulted in livebirths or stillbirths from 20 weeks' gestation or elective termination of pregnancy for fetal anomaly (ETOPFA) were included. Among liveborn spina bifida cases, we calculated mortality at different ages as number of deaths among liveborn cases divided by total number of liveborn cases with spina bifida. As a secondary outcome measure, we estimated the prevalence of spina bifida per 10 000 total births. The 95% confidence interval for the prevalence estimate was estimated using the Poisson approximation of binomial distribution. RESULTS: Between years 2001 and 2012, the overall first-week mortality proportion was 6.9% (95% CI 6.3, 7.7) and was lower in programmes operating in countries with policies that allowed ETOPFA compared with their counterparts (5.9% vs. 8.4%). The majority of first-week mortality occurred on the first day of life. In programmes where information on long-term mortality was available through linkage to administrative databases, survival at 5 years of age was 90%-96% in Europe, and 86%-96% in North America. CONCLUSIONS: Our multi-country study showed a high proportion of stillbirth and infant and child deaths among those with spina bifida. Effective folic acid interventions could prevent many cases of spina bifida, thereby preventing associated childhood morbidity and mortality.

Mortality by mode of delivery among infants with spina bifida in Texas.

BACKGROUND: It is hypothesized that cesarean delivery may reduce mortality among infants with spina bifida (e.g., by reducing trauma to the open lesion); however, few studies have assessed this relationship. METHODS: We used the Texas Birth Defects Registry to identify neonates with spina bifida born between 1999 and 2014. The mode of delivery (main exposure) was abstracted from each subject's birth certificate. The vital status (main outcome) was determined based on the presence or absence of a death certificate. When a death certificate was present, survival time was calculated by subtracting the date of birth from the date of death. We then conducted multivariable Cox proportional hazards regression to estimate the adjusted hazard ratio between cesarean delivery and death prior to 29 days. We adjusted for maternal race/ethnicity, maternal education, gestational age/birthweight, and breech presentation. This analysis was repeated for death prior to 365 days. RESULTS: We analyzed 1,983 nonsyndromic, liveborn neonates with spina bifida, and 68% of these neonates were delivered by cesarean. After adjusting for potential confounders, the adjusted hazard ratio [aHR] for death prior to 29 days was 0.77 (95% confidence interval [CI] 0.49, 1.21) and the aHR for death prior to 365 days was 0.93 (95% CI 0.63, 1.38) comparing infants delivered by cesarean to those delivered vaginally. CONCLUSIONS: Despite a lack of strong prior epidemiologic evidence, cesarean rates for neonates with spina bifida were high. Further investigations of the relationship between mode of delivery and infant outcomes, including mortality, complications, and long-term prognosis, are warranted.

Survival of infants with spina bifida and the role of maternal prepregnancy body mass index.

OBJECTIVE: To investigate first-year survival of infants born with spina bifida, and examine the association of maternal prepregnancy body mass index (BMI) with infant mortality. METHODS: This is a retrospective cohort study of 1,533 liveborn infants with nonsyndromic spina bifida with estimated dates of delivery from 1998 to 2011 whose mothers were eligible for the National Birth Defects Prevention Study (NBDPS). NBDPS data were linked to death records to conduct survival analyses. Kaplan-Meier survival functions estimated mortality risk over the first year of life. Cox proportional hazards models estimated hazard ratios (HRs) for maternal prepregnancy BMI categorized as underweight (<18.5), normal (18.5-24.9), overweight (25-29.9), and obese (≥30). RESULTS: Infant mortality risk among infants with spina bifida was (4.4% [3.52, 5.60%]). Infants with multiple co-occurring defects, very preterm delivery, multiple gestation, high-level spina bifida lesions, or non-Hispanic Black mothers had an elevated risk of infant mortality. Maternal prepregnancy underweight and obesity were associated with higher infant mortality (15.7% [7.20, 32.30%] and 5.82% [3.60, 9.35%], respectively). Adjusted HR estimates showed underweight and obese mothers had greater hazard of infant mortality compared to normal weight mothers (HR: 4.5 [1.08, 16.72] and 2.6 [1.36, 8.02], respectively). CONCLUSION: The overall risk of infant mortality for infants born with spina bifida was lower than most previously reported estimates. Infants born with spina bifida to mothers who were underweight or obese prepregnancy were at higher risk of infant mortality. This study provides additional evidence of the importance of healthy maternal weight prior to pregnancy.

Reductions in child mortality by preventing spina bifida and anencephaly: Implications in achieving Target 3.2 of the Sustainable Development Goals in developing countries.

BACKGROUND: There is an opportunity to reduce child mortality by preventing folic acid-preventable spina bifida and anencephaly (FAP SBA) in developing countries. We estimated reductions in FAP SBA-associated child mortality in 69 countries with an immediate potential for mandatory fortification of wheat flour. METHODS: Using data from multiple sources, we estimated the percent reductions in neonatal, infant, and under-five mortality that would have occurred by preventing FAP SBA; and the contributions of these reductions toward each country's Sustainable Development Goals (SDG) for child mortality reduction. We used the combined prevalence of spina bifida and anencephaly in selected countries before fortification, and estimated preventable child mortality associated with FAP SBA, assuming 0.5 per 1,000 live births as minimum achievable prevalence from mandatory fortification. RESULTS: Annually, 56,785 live births with FAP SBA occurred in the 69 countries examined. Of these, about 49,680 (87%) would have resulted in deaths under age 5 years, and are preventable through mandatory folic acid fortification. On average, compared to current rates, prevention of FAP SBA would have reduced the neonatal, infant, and under-five mortality by 19% (95% uncertainty interval [UI]: 16-24%), 15% (UI: 13-17%), and 14%, (95% UI: 13-17%), respectively. Prevention of FAP SBA seemed to contribute toward achieving SDG on neonatal and under-five mortality in developing countries. CONCLUSIONS: Prevention of FAP SBA will lead to notable and immediate reductions in child mortality. Many countries have an opportunity to effectively move toward child mortality-related SDG targets with existing milling infrastructure for food fortification.

Total prevention of folic acid-preventable spina bifida and anencephaly would reduce child mortality in India: Implications in achieving Target 3.2 of the Sustainable Development Goals.

BACKGROUND: The potential to reduce child mortality by preventing folic acid-preventable spina bifida and anencephaly (FAP SBA) is inadequately appreciated. To quantify possible reduction in FAP SBA-associated child mortality in low- and middle-income countries, we conducted an analysis to demonstrate in India, a country with more than 25 million births and 1.2 million under-five deaths each year, the decrease in neonatal, infant, and under-five mortality that would occur through total prevention of FAP SBA. METHODS: We estimated the percent reductions in neonatal, infant, and under-five mortality that would have occurred in India in 2015 had all of FAP SBA been prevented. We also estimated the contributions of these reductions toward India's Sustainable Development Goals on child mortality indicators. We considered the overall prevalence of spina bifida and anencephaly in India as 5 per 1,000 live births, of which 90% were preventable with effective folic acid intervention. RESULTS: In the year 2015, folic acid interventions would have prevented about 116,070 cases of FAP SBA and 101,565 under-five deaths associated with FAP SBA. Prevention of FAP SBA would have reduced annually, neonatal, infant, and under-five mortality by 10.2%, 8.9%, and 8.3%, respectively. These reductions would have contributed 18.5% and 17.2% to the reductions in neonatal and under-five mortality, respectively, needed by India to achieve its 2030 Sustainable Developmental Goal Target 3.2 addressing preventable child mortality. CONCLUSIONS: Total prevention of FAP SBA clearly has a significant potential for immediate reductions in neonatal, infant, and under-five mortality in India, and similarly other countries.

The Evolution of Spina Bifida Treatment Through a Biomedical Ethics Lens.

Spina bifida is a neurodevelopmental disorder that results in a broad range of disability. Over the last few decades, there have been significant advances in diagnosis and treatment of this condition, which have raised concerns regarding how clinicians prognosticate the extent of disability, determine quality of life, and use that information to make treatment recommendations. From the selective treatment of neonates in the 1970s, to the advent of maternal-fetal surgery today, the issues that have been raised surrounding spina bifida intervention invoke principles of medical bioethics such as beneficence and nonmaleficence, while also highlighting how quality of life judgments may drive care decisions. Such changes in treatment norms are also illustrative of how disability is viewed both within the medical community and by society at large. An examination of the changes in spina bifida treatment provides a model through which to understand how ethically complex decisions regarding care for children with disabilities has evolved, and the challenges faced when medical information is combined with value-based judgments to guide medical decision making.

Scorecard for spina bifida research, prevention, and policy - A development process.

Spina bifida is a serious and largely preventable neural tube birth defect and an important cause of mortality and lifelong disability. The People and Organizations United for Spina Bifida and Hydrocephalus (PUSH!) Global Alliance was formed in 2014 to provide a common platform for various organizations worldwide to raise the visibility of spina bifida and hydrocephalus. In its formative phase, the alliance recognized that in order to accelerate surveillance, prevention, and care for these conditions, there was a need to provide an evidence-based assessment of how nations are performing in specific areas. In this paper, we describe the impetus for, and the process of, developing country-level scorecards for spina bifida surveillance, prevention and care. The PUSH! Executive Committee formulated a comprehensive list of six actionable indicators measuring availability of published studies on population-based folate studies; surveillance of prevalence and mortality; prevention-based policies; access to care; and quality of life associated with spina bifida. Rubrics were developed to score each country on the aforementioned indicators. Country scores were pooled across each indicator and the composite scores ranged between zero and three if there was a need for improvement, four and five if they were in good standing, or six for an excellent status. The scorecard included country-specific recommendations assimilated from the literature and published guidelines to aid policy makers in accelerating surveillance and prevention, and improving the care and quality of life indicators. For comparison, country-level scorecards were grouped by WHO-regions. Score cards were made available publicly through the website "www.pu-sh.org".

Skin Ulcers and Mortality Among Adolescents and Young Adults With Spina Bifida in South Carolina During 2000-2010.

The authors investigated 48 deaths (7% death rate) among 690 adolescents and young adults with spina bifida in South Carolina during 2000-2010. The authors used Medicaid and other administrative data and a retrospective cohort design that included people with spina bifida identified using ICD-9 codes. Cox regression models with time-dependent and time-invariant covariates, and Kaplan-Meier survival curves were constructed. The authors found that 21.4% of the study group had a skin ulcer during the study period and individuals with skin ulcers had significantly higher mortality than those without ulcers (P < .0001). People who had their first skin ulcer during adolescence had higher mortality than those who had the first skin ulcer during young adulthood (P = .0002; hazard ratio = 10.70, 95% confidence interval for hazard ratio: 3.01, 38.00) and those without skin ulcers, controlling for other covariates. This study showed that age at which individuals first had a skin ulcer was associated with mortality.

Mortality after bladder augmentation in children with spina bifida.

PURPOSE: Renal failure has been a leading cause of death for children with spina bifida. Although improvements in management have increased survival, current data on mortality are sparse. Bladder augmentation, a modern intervention to preserve renal function, carries risks of morbidity and mortality. We determined long-term mortality and causes of death in patients with spina bifida treated with bladder augmentation. MATERIALS AND METHODS: We retrospectively reviewed the records of patients with spina bifida who underwent bladder augmentation between 1979 and 2013. Those born before 1972 or older than 21 years at augmentation were excluded. Demographic and surgical data were collected. Outcomes were obtained from medical records, death records and the Social Security Death Index. Fisher exact and Wilcoxon rank-sum tests and Kaplan-Meier plots were used for analysis. RESULTS: Of 888 patients in our bladder reconstruction database 369 with spina bifida met inclusion criteria. Median followup was 10.8 years. A total of 28 deaths (7.6%) occurred. The leading causes of mortality were nonurological infections (ventriculoperitoneal shunt related, decubitus ulcer fasciitis, etc) and pulmonary disease. Two patients (0.5%) died of renal failure. No patient died of malignancy or bladder perforation. Patients with a ventriculoperitoneal shunt had a higher mortality rate than those without a shunt (8.9% vs 1.5%, p = 0.04). CONCLUSIONS: Previously reported mortality rates of 50% to 60% in patients with spina bifida do not appear to apply in children who have undergone bladder augmentation. On long-term followup leading causes of death in patients with spina bifida after bladder augmentation were nonurological infections rather than complications associated with augmentation or renal failure.

Characteristics and survival of patients with end stage renal disease and spina bifida in the United States renal data system.

PURPOSE: We describe the characteristics, treatments and survival of patients with spina bifida in whom end stage renal disease developed from 2004 through 2008 in the United States Renal Data System. MATERIALS AND METHODS: We used ICD-9-CM code 741.* to identify individuals with spina bifida using hospital inpatient data from 1977 to 2010, and physician and facility claims from 2004 to 2008. We constructed a 5:1 comparison group of patients with end stage renal disease without spina bifida matched by age at first end stage renal disease service, gender and race/ethnicity. We assessed the risk of mortality and of renal transplantation while on dialysis using multivariate cause specific proportional hazards survival analysis. We also compared survival after the first renal transplant from the first end stage renal disease service to August 2011. RESULTS: We identified 439 patients with end stage renal disease and spina bifida in whom end stage renal disease developed at an average younger age than in patients without spina bifida (41 vs 62 years, p <0.001) and in whom urological issues were the most common primary cause of end stage renal disease. Compared to patients with end stage renal disease without spina bifida those who had spina bifida showed a similar mortality hazard on dialysis and after transplantation. However, patients with end stage renal disease without spina bifida were more likely to undergo renal transplantation than patients with spina bifida (HR 1.51, 95% CI 1.13-2.03). Hospitalizations related to urinary tract infections were positively associated with the risk of death on dialysis in patients with end stage renal disease and spina bifida (HR 1.42, 95% CI 1.33-1.53). CONCLUSIONS: Spina bifida was not associated with increased mortality in patients with end stage renal disease on dialysis or after renal transplantation. Proper urological and bladder management is imperative in patients with spina bifida, particularly in adults.

Prenatal versus postnatal repair procedures for spina bifida for improving infant and maternal outcomes.

BACKGROUND: Spina bifida is a fetal neural tube defect (NTD), which may be diagnosed in utero and is compatible with life postnatally, albeit often with significant disability and morbidity. Although postnatal repair is possible, with increasing in utero diagnosis with ultrasound, the condition has been treated during pregnancy (prenatal repair) with the aim of decreased morbidity for the child. The procedure that is performed during pregnancy does have potential morbidities for the mother, as it involves maternal surgery to access the fetus. OBJECTIVES: To compare the effects of prenatal versus postnatal repair and different types of repair of spina bifida on perinatal mortality and morbidity, longer term infant outcomes and maternal morbidity. SEARCH METHODS: We searched the Cochrane Pregnancy and Childbirth Group's Trials Register (31 July 2014). SELECTION CRITERIA: All published, unpublished, and ongoing randomised controlled trials comparing prenatal and postnatal repair of meningomyelocele for fetuses with spina bifida and different types of prenatal repair. DATA COLLECTION AND ANALYSIS: Two review authors independently evaluated trials for inclusion and methodological quality without consideration of their results according to the stated eligibility criteria and extracted data. MAIN RESULTS: Our search strategy identified six reports for potential inclusion. Of those, we included one trial (four reports) involving 158 women, which was at low risk of bias.The one included trial examined the effect of prenatal repair versus postnatal repair. For the primary infant outcome of neonatal mortality, there was no clear evidence of a difference identified for prenatal versus postnatal repair (one study, 158 infants, risk ratio (RR) 0.51, 95% confidence interval (CI) 0.05 to 5.54), however event rates were uncommon and so the analysis is likely to be underpowered to detect differences.Prenatal repair was associated with an earlier gestational age at birth (one study, 158 infants, mean difference (MD) -3.20 weeks, 95% CI -3.93 to -2.47) and a corresponding increase in both the risk of preterm birth before 37 weeks (one study, 158 infants, RR 5.30, 95% CI 3.11 to 9.04) and preterm birth before 34 weeks (one study, 158 infants, RR 9.23, 95% CI 3.45 to 24.71). Prenatal repair was associated with a reduction in shunt dependent hydrocephalus and moderate to severe hindbrain herniation. For women, prenatal repair was associated with increased preterm ruptured membranes (one study, 158 women, RR 6.15, 95% CI 2.75 to 13.78), although there was no clear evidence of difference in the risk of chorioamnionitis or blood transfusion, although again, event rates were uncommon.A number of this review's secondary infant and maternal outcomes were not reported. For the infant: days of hospital admission; survival to discharge; stillbirth; need for further surgery (e.g. skin grafting); neurogenic bladder dysfunction; childhood/infant quality of life. For the mother: admission to intensive care; women's emotional wellbeing and satisfaction with care. AUTHORS' CONCLUSIONS: This review is based one small well-conducted study. There is insufficient evidence to recommend drawing firm conclusions on the benefits or harms of prenatal repair as an intervention for fetuses with spina bifida. Current evidence is limited by the small number of pregnancies that have been included in the single conducted randomised trial to date.

Population-based study to determine mortality in spina bifida: New York State Congenital Malformations Registry, 1983 to 2006.

BACKGROUND: The lifetime risk of death among individuals with spina bifida is 10-times higher compared with the general population. A population-based analysis on cause-specific mortality among individuals spina bifida is lacking. METHODS: Using statewide, population-based New York Congenital Malformations Registry, we examined all births between years 1983 and 2006, and identified 1988 births with spina bifida and 10,951 births with congenital hypertrophic pyloric stenosis (CHPS). We linked registry records to birth and death files from vital records, and determined age- and cause-specific mortality for isolated and multiple spina bifida, and compared the findings with the less fatal CHPS. RESULTS: Mortality in spina bifida is significantly high compared with CHPS (16.9% vs. 0.96%, respectively). The probability of survival in spina bifida was lower compared with CHPS. A majority of the deaths in spina bifida occurred in infants within the first year of birth; however, an increased risk of death persisted in young adulthood for both isolated and multiple cases of spina bifida. The common causes of death in children with spina bifida were hydrocephalus, infections, cardiac anomalies, pneumonia, and pulmonary embolism; while infections, heart or kidney failure, injuries and neoplasms contributed to deaths in adults. CONCLUSION: We conclude that mortality in spina bifida is a large concern, and individuals living with the defect require improved clinical care for lethal medical complications. Primary prevention of spina bifida through mandatory folic acid fortification remains as the best strategy to reduce both disability and mortality associated with this defect across the world.

Improved survival among children with spina bifida in the United States.

OBJECTIVE: To evaluate trends in survival among children with spina bifida by race/ethnicity and possible prognostic factors in 10 regions of the United States. STUDY DESIGN: A retrospective cohort study was conducted of 5165 infants with spina bifida born during 1979-2003, identified by 10 birth defects registries in the United States. Survival probabilities and adjusted hazard ratios were estimated for race/ethnicity and other characteristics using the Cox proportional hazard model. RESULTS: During the study period, the 1-year survival probability among infants with spina bifida showed improvements for whites (from 88% to 96%), blacks (from 79% to 88%), and Hispanics (from 88% to 93%). The impact of race/ethnicity on survival varied by birth weight, which was the strongest predictor of survival through age 8. There was little racial/ethnic variation in survival among children born of very low birth weight. Among children born of low birth weight, the increased risk of mortality to Hispanics was approximately 4-6 times that of whites. The black-white disparity was greatest among children born of normal birth weight. Congenital heart defects did not affect the risk of mortality among very low birth weight children but increased the risk of mortality 4-fold among children born of normal birth weight. CONCLUSIONS: The survival of infants born with spina bifida has improved; however, improvements in survival varied by race/ethnicity, and blacks and Hispanics continued to have poorer survival than whites in the most recent birth cohort from 1998-2002. Further studies are warranted to elucidate possible reasons for the observed differences in survival.