Seasonal trend in the occurrence of myelomeningocele in nigeria: a hypothesis of climate-induced oxidative stress.

PURPOSE: Myelomeningocele is the most severe birth defect compatible with long-term survival. It accounts for 5.7% of neurological surgeries in Nigeria. However, the exact cause of this neural tube defect remains unidentified. This study aims to determine if seasonal variation is a potential environmental contributor. METHOD: This study prospectively recruited 242 children diagnosed with myelomeningocele at the University of Nigeria Teaching Hospital (UNTH), Enugu, Nigeria, between January 2010 and December 2022. Our primary outcome was the seasonal occurrence of myelomeningocele, while covariates included gender, birth order, maternal folic acid supplementation (FAS), and parental age. The estimated month of conception was derived from the mother's last menstrual period (LMP), and the occurrence of myelomeningocele across the various seasons in which these babies were conceived was assessed using the Lorenz curve and the Gini coefficient. RESULTS: 242 patients were studied with a male-to-female ratio of 1.26. The majority of cases were lumbosacral (93.4%), and none of the mothers commenced FAS before conception. The highest proportion of cases (39.7%) occurred during the hottest period of the dry season (January-March), while the lowest proportion (15.7%) occurred during the early wet season (April-June). The Gini index of 0.29, and the Gini coefficient derived from 100,000 Monte Carlo simulations of 0.24, indicate a significant variation in the distribution of myelomeningocele cases across different seasons of conception. CONCLUSION: The seasonal occurrence of myelomeningocele with a peak in January-March suggests a potential association with environmental factors including oxidative stress induced by solar radiation.

"Prevalence and secular trend of neural tube defects in fetal deaths in Argentina, 1994-2019".

BACKGROUND: Fetal deaths are a major source of information on the epidemiology of neural tube defects (NTDs; anencephaly and myelomeningocele). We analyzed NTDs prevalence and secular trend using fetal death records between 1994 and 2019 in Argentina. MATERIALS AND METHODS: Data were obtained from the Department of Statistics and Information of the Ministry of Health (DEIS). Using the number of fetal deaths due to anencephaly and myelomeningocele, we estimated the proportion of all fetal deaths due to anencephaly, myelomeningocele, and NTDs (anencephaly + myelomeningocele) during pre- and post-fortification period in Argentina. We also estimated the ratio of fetal deaths due to anencephaly, myelomeningocele, and NTDs (anencephaly + myelomeningocele) to 10,000 live births. Secular trend in the outcomes was analyzed using a Poisson model and Joinpoint regression analysis. RESULTS: In the entire period analyzed, the NTD proportion on fetal deaths was 1.32. In 1994, NTDs accounted for 34.7% of congenital malformations fetal deaths (CM) and 1.7% of all fetal deaths, whereas in 2019, these percentages were 9.4% and 0.5%, respectively. NTDs present a negative secular trend (p < .05). The risk of fetal death due to anencephaly and myelomeningocele decreases between 2005 and 2019 by 67% and 51% respectively (p < .05) in comparison to the period between 1994 and 2004 before the effective fortification of wheat flour used in the food industry destined for the domestic market. DISCUSSION AND CONCLUSION: We found a significant decrease in the risk of all fetal deaths due to NTDs, particularly anencephaly, in Argentina over the study period, with most reduction observed during the mandatory flour fortification era (introduced in Argentina in 2002). The inclusion of fetal deaths in NTD surveillance, coupled or uncoupled with other pregnancy outcomes, is essential for monitoring preventive supplementation measures.

In utero myelomeningocele repair and incidence of lower urinary tract surgery. Results of a prospective study.

INTRODUCTION: The purpose of this study was to determine the incidence of lower urinary tract surgery in patients with myelomeningocele (MMC) operated in utero as well as in the subgroup of patients with a minimum age of 5 years. METHODS: We use a previously published protocol to categorize bladder patterns and propose the treatment: high risk pattern-anticholinergics and clean intermittent catheterization (CIC); incontinent and normal patterns-clinical surveillance and underactivity pattern- CIC. Non-response to clinical treatment with maintenance of detrusor pressure higher than 40 cmH20 with worsening of hydronephrosis or recurrent urinary infection were imperative indications for surgery and urinary incontinence due to sphincter deficiency and leakage pressure below 40 cmH20 in patients aged 5 years and older were possible indications too. We identified the prevalence of urinary tract surgery and concomitant treatment of constipation and refractory fecal incontinence by convenience, the technique performed and surgery outcomes. RESULTS: A total of 122 patients are part of the prospective protocol. The first UE was performed in 119 patients (median age of 4 months). The categorization of the bladder pattern was high risk in 52.1%, incontinent in 25.2%, hypocontractile 4.2% and normal in 18.5%. Current follow-up was 29.9 months (1-99 months). Surgery was performed on 10 patients (8.4%): 3 vesicostomies, 2 surgeries to treat vesicoureteral reflux, 6 bladder augmentations, 3 ACE and a sling surgery. Considering only patients older than 5 years, we identified 65 patients and seven surgeries performed (10.8%). DISCUSSION: Most bladder reconstruction studies are case series of single institutions or cooperative services, most of which are retrospective. The prospective clinical interpretation and imaging and urodynamic exams over time allowed us prospectively to define the risk of surgery in patients with myelomeningocele operated in utero. It must be considered that the response to the initial urological treatment and even the failure that leads to the need for surgery have to be evaluated in a context of follow-up time. For this reason we have studied separately patients over 5 years-old and also the group considered to be at greatest risk for surgery, the high-risk group of the categorization already described. CONCLUSION: We found an incidence of 10.8% of surgeries in patients over 5 years-old and 12.9% for the high-risk group. This information may be useful to educate parents when stating urological treatment.

Understanding the Mothers of Children with Spina Bifida and Hydrocephalus in Tanzania.

OBJECTIVE: To identify if there are cultural, medical, educational, economic, nutritional and geographic barriers to the prevention and treatment of spina bifida and hydrocephalus. METHODS: The mothers of infants with spina bifida and hydrocephalus admitted to Muhimbilli Orthopaedic Institute, Dar Es Salaam, Tanzania, between 2013 and 2014 were asked to complete a questionnaire. A total of 299 infants were identified: 65 with myelomeningoceles, 19 with encephaloceles, and 215 with isolated hydrocephalus. The questionnaire was completed by 294 of the mothers. RESULTS: There was a high variation in the geographic origin of the mothers. Approximately 85% traveled from outside of Dar Es Salaam. The mean age was 29 (15-45) years old with a parity of 3 (1-10). The rates of consanguinity, obesity, antiepileptic medication, HIV seropositivity, and family history were 2%, 13%, 0%, 2%, and 2%, respectively. A maize-based diet was found in 84%, and only 3% of woman took folic acid supplementation, despite 61% of mothers stating that they wished to conceive another baby. Unemployment was high (77%), a low level of education was common (76% not attended any school or obtaining a primary level only), and 20% were single mothers. Hospital only was the preferred method of treatment for 94% of the mothers, and 85% of the babies were born in a hospital. CONCLUSIONS: Our study highlights some of the cultural, educational, geographic, nutritional, and economic difficulties in the prevention and management of spina bifida and hydrocephalus in Tanzania.

Awareness of spina bifida among family of affected child. A cross sectional questionnaire.

OBJECTIVES: To evaluate the awareness of spina bifida (SB), risk factors and possible preventive measures among mothers who had been following in our clinic with a spina bifida affected child. Methods: A cross-sectional questionnaire-based study with 38 mothers of SB patients who are following at SB and hydrocephalus clinic at King Fahad University Hospital, Khobar, Eastern Province, Saudi Arabia. RESULTS: Thirty-eight mother were included in this questionnaire. Most of the participants were Saudi (94.7%). Ten out of 38 women (26.3%) had used medications during pregnancy (pain medications and herbal drugs), 4 out of 38 (10.5%) had been exposed to imaging radiation, while 9 (23.7%) had experienced moderate to high grade fever (39-41°C) during pregnancy. Moreover, the majority (86.8%) of these women did not receive folic acid (FA) before pregnancy, and 42.1% of them did not have FA during their first trimester. Only one (2.6%) gave positive family history of SB while, 6 (15.8%) reported having other SB children.  Conclusion: There is a considerably low level of awareness in mothers of SB patients despite prevalence of this anomaly in the Eastern province. This necessitates an effort from health care providers to educate the community about this birth defect entity. Furthermore, genetic counseling should be encouraged especially in those who have a positive familial history for better understanding. Also, larger sample size with randomized controlled trials and larger epidemiological studies should be implemented.

Unidad de asistencia coordinada a pacientes con anomalías del tubo neural y sus familias / Coordinated care unit for patients with neural tube anomalies and their families

La Espina Bífida es una malformación multisistémica incurable y transmisible, con múltiples secuelas importantes, unas tratables y otras evitables, que afectan y limitan tanto la salud como la vida socio-laboral. El objetivo principal debe ser la prevención. La primaria, es decir la no ocurrencia, se puede realizar conociendo los posibles factores etiológicos implicados en la población. Esto es una responsabilidad de política sanitaria basada en la evidencia científica y la opinión de los expertos. La prevención secundaria es intentar disminuir las secuelas y mejorar la calidad y expectativa de vida de los nacidos con EB. En la actualidad las personas nacidas con Espina Bífida, tienen una prolongada expectativa de vida, con una serie de necesidades específicas durante toda su existencia. Son fundamentales los primeros años de vida. En la adolescencia y la adultez hay un gran riesgo si se abandona cualquier aspecto socio-sanitario a la evolución natural, dejando los controles y cuidados propios de la asistencia coordinada pediátrica, ya que la tendencia será a producirse complicaciones totalmente evitables. El paciente con Espina Bífida es fundamentalmente una persona, no una suma de patologías. Es imprescindible tener una visión holística e integral de cada persona con EB, y no como una serie de diferentes especialidades inconexas. La intervención independiente de los múltiples profesionales implicados pueden interferirse perjudicialmente, pero pueden lograr la máxima eficiencia (costo-eficacia) y calidad asistencial si lo hacen de manera continua y coordinada. Los esfuerzos realizados y los objetivos conseguidos en la edad pediátrica pueden mejorarse con la Asistencia Coordinada Multidisciplinar, pero pueden perderse si no se continúan durante toda la vida, produciéndose un deterioro evitable, en la calidad de vida y una perdida de los elevados recursos sanitarios invertidos

Bifid Spine is a multisystem malformation incurable and transmissible with a lot of important sequelae, some of them with treatment and some other avoidable. They affect and limit not only health but social-work life too.The main objective must be prevention. The primary prevention, meaning not occurrence, could be done by recognizing the possible etiologic factors that affect the population. This is a responsibility of health policies based in scientific evidences and expert's opinions. Secondary prevention would be to make efforts to decrease the consequences and improve newborns with bifid spine life`s quality and life expectancy. Actually, persons born with bifid spine, have a long-term life expectancy with specific necessities during their lives. The first two years of life are very important. During their adolescence and adult life, risk increases if any social-health aspect is abandoned giving up follow and self-care from the pediatrics coordinated assistance team, as it follows its natural evolution. It will cause totally avoidable complications. A patient with bifid spine is basically a person, not a group of diseases. It is essential to have a holistic and total look for each person with bifid spine and not consider the patient like a collection of troubles treated with different non coordinated specialities. The individual intervention of each medical doctor could damage and it could be better if they work coordinated for a long time. This strategy would allow improved efficiency (cost/efficacy) and quality of assistance.Every effort done and the objectives reached in childhood can be improved with the multidisciplinary coordinated assistance, but it can be lost if it does not continue during all life. It may cause a serious and evitable damage in life quality with loss of spent health resources

Trends in epidemiology and hospitalization utilization for myelomeningocele repair from 2000 to 2009.

PURPOSE: Although the incidence of myelomeningocele (MMC) has declined over the past decades with folic acid supplementation and prenatal screening, neural tube defects remain the most common birth defect in the USA. A majority of affected neonates require surgical repair. To characterize US trends in the epidemiology and hospital utilization of MMC repair over the past decade, we analyzed a nationally representative database. METHODS: We queried the Healthcare Cost and Utilization Project (HCUP) Kid's Inpatient Database (KID) for all discharges with procedure code for MMC repair for the years 2000, 2003, 2006, and 2009. The cohorts from these time points were compared for their demographic and in-hospital variables. Results are reported as estimated frequencies and means with 95 % confidence intervals (CI). RESULTS: Sex, race, insurance status, family income level, and mortality of affected infants have not changed significantly over the decade. A majority of neonatal MMC repairs occur in larger hospital bed size and more specialized children's hospital centers. Of patients, 52.3 to 60 % receive VPS placement during the same admission as the primary MMC repair. Total hospital costs for the MMC hospitalizations have remained relatively stable from 42,843 dollars in 2003 to 46,749 dollars in 2009 (adjusted to 2009 dollars). CONCLUSION: Demographics of children having MMC repair have not changed significantly over the past decade, while these surgeries have become more concentrated in pediatric-specialized centers. There appears to be a plateau in public health and access advances with relatively stable cost of MMC hospital care.

PATTERNS OF NEURAL TUBE DEFECTS AT TWO TEACHING HOSPITALS IN ADDIS ABABA, ETHIOPIA A THREE YEARS RETROSPECTIVE STUDY.

BACKGROUND: Neural tube defects (NTDs), one of the most common congenital malformations, are potentially preventable cause of perinatal morbidity and mortality. OBJECTIVES: To give baseline description of NTDs and their outcome at two teaching hospitals in Addis Ababa, Ethiopia. MATERIALS AND METHODS: A retrospective cross sectional descriptive study conducted from September 2009 to August 2012. RESULTS: During the study period out of 28,961 deliveries 177 cases of NTDs were identified, giving an overall NTD prevalence of 6.1/1000. Only 12% (21/177) were diagnosed before 28 weeks of gestation. The mean gestational age at diagnosis of NTDs was 33.8 weeks (±5.5). Majority, 93.2% (165/177), had antenatal care (ANC) follow-up. Most, 72% (127/177), were diagnosed by ultrasound before delivery while 28% (50/177) were identified at the time of delivery or expulsion. Majority, 85.3% (151/177), never received folic acid supplementation. Only less than 1% (2/177) of the mothers started taking folic acid supplementation pre-conceptionally. Only a third, 33.3% (59/177), of the fetuses were born alive while only 13.6% (24/177) were discharged alive. Myelomeningocele, identified in 51.4% (91/177), was the commonest NTD in this study. CONCLUSION AND RECOMMENDATIONS: The proportion of NTDs in this study is among the highest globally reported rates. The practice of periconceptional folic acid supplementation is negligible. And although most had ANC follow-up the vast majority of NTDs were diagnosed late in the third trimester. It is, therefore, highly recommended to consider implementing national preventive strategies to reduce the prevalence of NTDs in Ethiopia.

Outcomes of incontinent ileovesicostomy in the pediatric patient.

PURPOSE: Ileovesicostomy is a reconstructive option in complex urological cases but pediatric specific outcomes are lacking. We report our results with pediatric ileovesicostomy. MATERIALS AND METHODS: We retrospectively evaluated patients younger than 18 years undergoing incontinent ileovesicostomy at Vanderbilt University. History, urinary tract management and operative course were reviewed in the electronic medical record. Particular attention was given to immediate and long-term postoperative complications. RESULTS: Nine patients underwent incontinent ileovesicostomy between 2000 and 2013 at a mean age of 10.3 years (range 1.4 to 15.5). Surgical indication was sequelae of neurogenic or nonneurogenic neurogenic bladder (such as infection or worsening hydronephrosis) in 5 patients, reversal of vesicostomy in 3 and closure of cloacal exstrophy in 1. All 9 patients were thought incapable of reliable clean intermittent catheterization due to family unwillingness, poor social support or patient refusal. Median followup was 11.5 months (mean 48.2, range 1.3 to 144.8). Immediate postoperative complications included ileus requiring total parenteral nutrition and a wound infection in 1 patient. Long-term complications included urinary tract infection in 2 patients (febrile in 1 and positive culture for foul smelling urine in 1), stomal issues in 2 and temporary urethral leakage in 1. Constipation affected 3 children in long-term followup (all with neurogenic bowel preoperatively). Postoperative creatinine was stable or improved in all patients. CONCLUSIONS: Ileovesicostomy is a viable approach in children left with few other options, particularly those who are noncompliant or physically/socially unable to handle catheterization. This operation can help keep such patients out of diapers.

Prevention of myelomeningocele: African perspectives.

BACKGROUND: The prevention of neural tube defects (NTDs) is now an issue of major public health concern. Myelomeningocele, the most common NTD, often results in severe disabilities and may be life threatening. In Africa, there is little awareness of the NTDs, and most African countries lag behind in the global efforts at preventing them. PURPOSE: Following a review of global literature, we discuss the burden of myelomeningocele on the society and the current state of its prevention with an emphasis on Africa within the global context. The realization that folate supplementation reduces the occurrence of NTDs by as much as 85 % offers an opportunity to promote global reduction in the incidence of NTDs through (1) effective folate supplementation strategies combined with (2) secondary prevention using prenatal detection and termination of NTD pregnancies within respective national legal frameworks. We call attention to the poor state of understanding of NTDs in Africa and the alarming rarity of policies to prevent the condition in the continent. CONCLUSIONS: The understanding of the contributions of folate deficiency to the causation of NTDs has enabled appropriate, though still inadequate, preventive measures to be taken in several countries. We call on African governments and the international community to rapidly promote policies aimed at making fortification of wheat (and or other substitute staple foods) with folic acid universally available.

Decreased incidence of myelomeningocele at birth: effect of folic acid recommendations or prenatal diagnostics?

PURPOSE: In Denmark, prevention to reduce the spina bifida birth rate has focused on two areas: folic acid supplementation (1997) and changes in the national ultrasonography screening programme (2004). Myelomeningocele (MMC) is the most severe malformation among the spina bifidas. Taking into consideration the potential negative effect of high-dose folic acid consumption, we found a need to look into the effectiveness of these two strategies in our complete MMC population. METHODS: All spina bifida patients born in the western part of Denmark are differentiated into proper subgroups based on MR imaging, giving us a unique chance to study a true MMC population. The total number of the group of MMC children since 1983 is 121. One hundred and eight (89%) parents answered a questionnaire. RESULTS: Following the changes in the prenatal ultrasonography screening programme in 2004, a significant decline of 60% live birth MMC per year was noted, incidence rate ratio (IRR) = 40% (22-73%), p = 0.3%. We found no change in MMC birth rate after introduction of folic acid supplementation, IRR = 121% (81-181%), p = 36%. CONCLUSION: Our findings demonstrate no effect of folic acid recommendation due to lack of compliance among women of reproductive age in Denmark. However, we found an improved early detection rate of prenatal MMC by high-quality ultrasonography. Subsequent early termination of pregnancy has led to a significant reduction of birth rate of babies with MMC.

Frequencies of congenital anomalies among newborns admitted in nursery of Ayub Teaching Hospital Abbottabad, Pakistan.

BACKGROUND: Congenital anomalies play a significant role in perinatal and neonatal morbidity and mortality. The frequency of these congenital anomalies varies in different populations. Objective of this study was to find out the frequencies of congenital anomalies admitted in nursery of Ayub Teaching Hospital, Abbottabad. METHODS: In this descriptive, cross-sectional study all patients admitted in NICU from October 2009 to January 2010 were included. The patients were examined for major and minor congenital anomalies. The observations were recorded in tabulated form. RESULTS: A total of 2,360 patients were admitted in NICU during the study period. One hundred patients were noted to have congenital anomalies. The most frequent anomalies involved the central nervous system (31%). Meningomyelocele was the commonest defect (71%, 22 out of 31 cases of CNS defects), among these males were more (77%, 17 out of 22 of meningomyelocele cases) than females (14 out of 31). These were followed by patients born with congenital heart defects (16%). Patients with urogenital anomalies (6%) were all male except for one who had ambiguous genitalia. CONCLUSIONS: Cases of meningomyelocele were the commonest presenting congenital anomaly. More stress should be laid on the role of peri-conceptional vitamin supplementation like folic acid for the primary prevention of congenital defects.

Novel treatment immediately after myelomeningocele repair applying low-level laser therapy in newborns: a pilot study.

BACKGROUND/AIMS: The use of low-level laser therapy (LLLT) in neurosurgery is still hardly disseminated and there are situations in which the effects of this therapeutic tool would be extremely relevant in this medical field. The aim of the present study is to analyze the effect of LLLT on tissue repair after the corrective surgical incision in neonates with myelomeningocele, in an attempt to diminish the incidence of postoperative dehiscences following surgical repair performed immediately after birth. MATERIALS AND METHODS: Prospective pilot study with 13 patients submitted to surgery at birth who received adjuvant treatment with LLLT (group A). A diode laser CW, λ = 685 nm, p = 21 mW, was applied punctually along the surgical incision, with 0.19 J delivered per point, accounting for a total of 4-10 J delivered energy per patient, according to the surgical wound area and then compared with the results obtained in 23 patients who underwent surgery without laser therapy (group B). RESULTS: This pilot study disclosed a significant decline in dehiscences of the surgical wounds in neonates who were submitted to LLLT (7.69 vs. 17.39%). CONCLUSION: This new adjuvant therapeutic modality with LLLT aided the healing of surgical wounds, preventing morbidities, as well as shortening the period of hospital stay, which implies a reduction of costs for patients and for the institution.

Impact of folic acid food fortification on the birth prevalence of lipomyelomeningocele in Canada.

BACKGROUND: Recent studies reported no reduction in the frequency of lipomeningomyelocele (LMMC) in Hawaii and Nova Scotia after the implementation of a folic acid food fortification policy in 1998, while a marked reduction in the prevalence of other NTDs was observed. This study was performed to assess the prevalence of LMMC in Canada in relation to the timing of food fortification. METHODS: The study population included livebirths, stillbirths, and terminations of pregnancies because of fetal anomaly to women residing in seven Canadian provinces, from 1993 to 2002. In each province, the ascertainment of NTD cases relied on multiple sources, and in addition all medical charts were reviewed. The study period was divided into pre-, partial, and full fortification periods, based on results of red cell folate tests published in the literature. RESULTS: A total of 86 LMMC cases were recorded among approximately 1.9 million live births. The average birth prevalence rate was 0.05/1,000, ranging from a minimum of 0.01/1,000 in 2002 to a maximum of 0.08/1,000 in 1999. There was statistical heterogeneity between years (p = .01), but no pattern compatible with a decrease following fortification. Comparing the full fortification period with the prefortification period, there was a slight but not statistically significant decrease in LMMC birth prevalence. CONCLUSIONS: LMMC seems to be pathogenically distinct from myelomeningocele and more studies are needed to understand the embryologic mechanisms leading to this condition, and the environmental and genetic factors involved in its etiology.

Pattern and factors affecting management outcome of spina bifida cystica in Ile-Ife, Nigeria.

BACKGROUND AND OBJECTIVE: There is paucity of data on the pattern and factors affecting the management outcome of patients with spina bifida cystica in the Ife-Ijesa zone, Nigeria. This study was designed to address this research question. METHOD: One hundred and six consecutive cases of spina bifida cystica who presented in our hospital from January 1990 to December 2004 were reviewed. We obtained information on sociodemographic factors, medical history and management as well as clinical outcome. SPSS was used to analyze the data. RESULT: Males constituted 54.7% and females 45.2% of cases. Mortality was high in those presented after the 4th week of life (p = 0.04). The malformation occurred in the lumbar and lumbosacral regions in 77.4%. Myelomeningocele was the most common type (86.8%). Hydrocephalus was recorded in 53.8% of patients. Surgical closure was done for 91.5% of the patients. About 77% of all the patients were discharged while 22.7% died. This was significantly related to age at presentation (p = 0.04) and infection before surgery (p = 0.045). Postoperative complications were more frequent in patients with ruptured lesions (p = 0.025), a larger size of defect (p = 0.028) and a lower birth weight (p = 0.006). CONCLUSION: Myelomeningocele is the most common type of spina bifida cystica in our environment. Late presentation and preoperative infection are associated with high mortality in our patients.

Descriptive epidemiology of lipomyelomeningocele, Hawaii, 1986-2001.

BACKGROUND: The epidemiology of neural tube defects (NTDs) may depend on the type of the defect and its location. There is little epidemiologic information on lipomyelomeningocele, a type of NTD. The objective of this investigation was to describe the epidemiology of lipomyelomeningocele. METHODS: Cases were obtained from a Hawaii birth defect registry and consisted of all infants delivered during 1986-2001 with lipomyelomeningocele. Lipomyelomeningocele rates per 10,000 births were calculated for various factors. RESULTS: A total of 17 cases of lipomyelomeningocele were identified, for a total rate of 0.57. When the lipomyelomeningocele rate was examined with respect to the time period of folic acid fortification of cereal grains in the United States, the rate was 0.57 during 1986-1996 (prefortification), 0.57 during 1997-1998 (voluntary fortification), and 0.58 during 1999-2001 (mandatory fortification). A total of 11 (64.7%) of the cases were female. Lipomyelomeningocele rates were higher among infants born to mothers in younger and older age groups. The rate was highest among Pacific Islanders, followed by Filipinos and Far East Asians, and lowest among whites. Rates were greater with lower birth weight and gestational age and among multiple births. CONCLUSIONS: Lipomyelomeningocele rates were not found to be affected by folic acid fortification. The majority of cases were female. Other demographic and clinical factors appear to be associated with lipomyelomeningocele risk. None of the differences in rates between the various subgroups were statistically significant, and due to the small number of cases, the 95% confidence interval (CI) ranges were wide. However, the findings suggest possible patterns of occurrence. Additional studies involving larger numbers of cases are recommended.

Ineffectiveness of dietary folic acid supplementation on the incidence of lipomyelomeningocele: pathogenetic implications.

OBJECT: Periconceptual folic acid supplementation is effective in myelomeningocele prevention. The relationship between folic acid and lipomyelomeningocele (LMM) and the overall incidence of this occult form of spina bifida has never been studied. The objectives of this study were to determine the impact of dietary folic acid supplementation on the incidence of LMM and to measure its overall incidence. METHODS: In a retrospective population-based study the authors calculated the incidence of LMM in Nova Scotia between 1985 and 2001. Because of changes in public policy during this period, there are three intervals defined in relation to the treatment of the food supply with folic acid: 1) prior to folic acid fortification (1985-1994); 2) postsupplementation but prefortification (1995-1998); and 3) postfortification. The overall incidence of LMM in Nova Scotia between 1985 and 2001 was 16 per 100,000 live births or one case per 6121 live births. Its incidence between 1985 and 1994 was 15 per 100,000 live births, and between 1995 and 1998 it was 12 per 100.000 live births (relative risk [RR] 0.82, 95% confidence interval [CI] 0.31-2.22; p = 0.7). Between 1999 and 2001, the incidence of LMM was 29 per 100,000 live births, which was not significantly different from that between 1995 and 1998 (RR 2.41. 95% CI 0.79-7.36; p = 0.11) or between 1985 and 1994 (RR 1.98, 95% CI 0.86-4.56; p = 0.1). CONCLUSIONS: The overall incidence of LMM between 1985 and 2001 in Nova Scotia was 16 per 100,000 live births and has not been reduced by dietary folic acid supplementation. This finding provides epidemiological evidence that the embryogenesis of LMM is fundamentally different from that of myelomeningocele.

Epidemiology, etiologic factors, and prenatal diagnosis of open spinal dysraphism.

The caudal neural tube closes late in the first month after fertilization and failure of it results in myelomeningocele. Epidemiologic studies have shown differences in prevalence at birth based on ethnic-racial backgrounds and geography. Etiologic factors include the drug valproic acid or carbamazepine. Periconceptional folic acid supplementation appears to decrease the prevalence of neural tube defects. Numerous modalities allow for prenatal diagnosis of myelomeningocele. A cesarean section, before rupture of amniotic membranes and onset of labor, decreases the degree of paralysis.