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PloS One 2023We performed a systematic review and meta-analysis on the incidence of secondary tethered spinal cord (TSC) between prenatal and postnatal closure in patients with MMC.... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
We performed a systematic review and meta-analysis on the incidence of secondary tethered spinal cord (TSC) between prenatal and postnatal closure in patients with MMC. The objectives was to understand the incidence of secondary TSC after prenatal surgery for MMC compared to postnatal surgery for MMC.
MATERIAL AND METHODS
On May 4, 2023, a systematic search was conducted in Medline, Embase, and the Cochrane Library to gather relevant data. Primary studies focusing on repair type, lesion level, and TSC were included, while non-English or non-Dutch reports, case reports, conference abstracts, editorials, letters, comments, and animal studies were excluded. Two reviewers assessed the included studies for bias risk, following PRISMA guidelines. TSC frequency in MMC closure types was determined, and the relationship between TSC occurrence and closure technique was analyzed using relative risk and Fisher's exact test. Subgroup analysis revealed relative risk differences based on study designs and follow-up periods. A total of ten studies, involving 2,724 patients, were assessed. Among them, 2,293 patients underwent postnatal closure, while 431 received prenatal closure for the MMC defect. In the prenatal closure group, TSC occurred in 21.6% (n = 93), compared to 18.8% (n = 432) in the postnatal closure group. The relative risk (RR) of TSC in patients with prenatal MMC closure versus postnatal MMC closure was 1.145 (95%CI 0.939 to 1.398). Fisher's exact test indicated a statistically non-significant association (p = 0.106) between TSC and closure technique. When considering only RCT and controlled cohort studies, the overall RR for TSC was 1.308 (95%CI 1.007 to 1.698) with a non-significant association (p = .053). For studies focusing on children up until early puberty (maximum 12 years follow-up), the RR for tethering was 1.104 (95%CI 0.876 to 1.391), with a non-significant association (p = 0.409).
CONCLUSION AND DISCUSSION
This review found no significant increase in relative risk of TSC between prenatal and postnatal closure in MMC patients, but a trend of increased TSC in the prenatal group. More long-term data on TSC after fetal closure is needed for better counseling and outcomes in MMC.
Topics: Humans; Female; Pregnancy; Meningomyelocele; Fetus; Neurosurgical Procedures; Incidence; Spinal Cord
PubMed: 37379312
DOI: 10.1371/journal.pone.0287175 -
Clinical Orthopaedics and Related... Apr 2022Life expectancy of individuals with spina bifida has continued to improve over the past several decades. However, little is known about the longitudinal course of...
BACKGROUND
Life expectancy of individuals with spina bifida has continued to improve over the past several decades. However, little is known about the longitudinal course of scoliosis in individuals with myelomeningocele (MMC), a spina bifida subtype, across their lifespan. Specifically, it is not known whether management during or after the transition years from adolescence to adulthood is associated with comorbidities in adulthood nor if these individuals benefit from scoliosis treatment later in life.
QUESTIONS/PURPOSES
In this systematic review, we asked: (1) Is the risk of secondary impairments (such as bladder or bowel incontinence, decreased ambulation, and skin pressure injuries) higher among adolescents and adults with MMC and scoliosis than among those with MMC without scoliosis? (2) Is there evidence that surgical management of scoliosis is associated with improved functional outcomes in adolescents and adults with MMC? (3) Is surgical management of scoliosis associated with improved quality of life in adolescents and adults with MMC?
METHODS
We performed a systematic review of articles in Medline and Embase from 2000 until February 5, 2021. Search terms such as "spinal dysraphism," "spina bifida," "meningomyelocele," and "scoliosis" were applied in diverse combinations. A total of 1429 publications were identified, and 13 were eligible for inclusion. We included original studies reporting on scoliosis among individuals older than 15 years with MMC. When available, we extracted the prevalence of MMC and scoliosis, studied population age, percentage of patients experiencing complications, functional outcomes, and overall physical function. We excluded non-English articles and those with fewer than 10 individuals with scoliosis and MMC. We used the Preferred Reporting Items for Systematic Reviews and Meta-analyses, and registered the review before data collection (PROSPERO: CRD42021236357). We conducted a quality assessment using the Methodologic Index for Nonrandomized Studies (MINORS) tool. In 13 included studies, there were 556 individuals with MMC and scoliosis. Most were retrospective case series, although a minority were retrospective/comparative studies. The mean MINORS score was 12.3 ± 1.65 (a MINORS score over 12 generally is considered good reporting quality, scores below 12 are considered at high risk of bias).
RESULTS
In general, studies found that individuals with MMC and scoliosis were more likely to have secondary impairments such as bladder/bowel incontinence, decreased ambulation, and pressure injuries than were patients with MMC without scoliosis. These secondary impairments were associated with hydrocephalus and high-level MMC lesions. However, when one study evaluated mortality, the results showed that although most deceased individuals who had spina bifida had scoliosis, no association was found between the age of death and scoliosis. Among the studies evaluating functional outcomes, none supported strong functional improvement in individuals with MMC after surgery for scoliosis. No correlation between the Cobb angle and sitting balance was noted; however, the degree of pelvic obliquity and the level of motor dysfunction showed a strong correlation with scoliosis severity. There was no change in sitting pressure distributions after spinal surgery. The lesion level and scoliosis degree independently contributed to the degree of lung function impairment. Although studies reported success in correcting coronal deformity and stopping curve progression, they found no clear benefit of surgery on health-related quality of life and long-term outcomes. These studies demonstrated that the level of neurologic function, severity of hydrocephalus, and brainstem dysfunction are greater determinants of quality of life than spinal deformity.
CONCLUSION
This systematic review found that adolescents and adults with MMC and scoliosis are more likely to have secondary impairments than their peers with MMC only. The best-available evidence does not support strong functional improvement or health-related quality of life enhancement after scoliosis surgery in adolescents and adults with MMC. The level of neurologic dysfunction, hydrocephalus, and brainstem dysfunction are greater determinants of quality of life. Future prospective studies should be designed to answer which individuals with MMC and scoliosis would benefit from spinal surgery. Our findings suggest that the very modest apparent benefits of surgery should cause surgeons to approach surgical recommendations in this patient population with great caution, and surgeons should counsel patients and their families that the risk of complications is high and the benefits may be small.
LEVEL OF EVIDENCE
Level IV, therapeutic study.
Topics: Adolescent; Adult; Fecal Incontinence; Humans; Hydrocephalus; Meningomyelocele; Prospective Studies; Quality of Life; Retrospective Studies; Scoliosis; Spinal Dysraphism
PubMed: 34939968
DOI: 10.1097/CORR.0000000000002087 -
EClinicalMedicine Jan 2024Folic acid (pteroylmonoglutamic acid) supplements are highly effective for prevention of neural tube defects (NTD) prompting implementation of mandatory or voluntary...
BACKGROUND
Folic acid (pteroylmonoglutamic acid) supplements are highly effective for prevention of neural tube defects (NTD) prompting implementation of mandatory or voluntary folic acid fortification for prevention of NTDs. We used plasma folate levels in population studies by country and year to compare effects of folic acid fortification types (mandatory or voluntary folic acid fortification policies) on plasma folate levels, NTD prevalence and stroke mortality rates.
METHODS
We conducted systematic reviews of (i) implementation of folic acid fortification in 193 countries that were member states of the World Health Organization by country and year, and (ii) estimated population mean plasma folate levels by year and type of folic acid fortification. We identified relevant English language reports published between Jan 1, 1990 and July 31, 2023 using Google Scholar, Medline, Embase and Global Health. Eligibility criteria were observational or interventional studies with >1000 participants. Studies of pregnant women or children <15 years were excluded. Using an ecological study design, we examined the associations of folic acid fortification types with NTD prevalence (n = 108 studies) and stroke mortality rates (n = 3 countries).
FINDINGS
Among 193 countries examined up to 31 July 2023, 69 implemented mandatory folic acid fortification, 47 had voluntary fortification, but 77 had no fortification (accounting for 32%, 53% and 15% of worldwide population, respectively). Mean plasma folate levels were 36, 21 and 17 nmol/L in populations with mandatory, voluntary and no fortification, respectively (and proportions with mean folate levels >25 nmol/L were 100%, 15% and 7%, respectively). Among 75 countries with NTD prevalence, mean (95% CI) prevalence per 10,000 population were 4.19 (4.11-4.28), 7.61 (7.47-7.75) and 9.66 (9.52-9.81) with mandatory, voluntary and no folic acid fortification, respectively. However, age-standardised trends in stroke mortality rates were unaltered by the introduction of folic acid fortification.
INTERPRETATION
There is substantial heterogeneity in folic acid fortification policies worldwide where folic acid fortification are associated with 50-100% higher population mean plasma folate levels and 25-50% lower NTD prevalence compared with no fortification. Many thousand NTD pregnancies could be prevented yearly if all countries implemented mandatory folic acid fortification. Further trials of folic acid for stroke prevention are required in countries without effective folic acid fortification policies.
FUNDING
Medical Research Council (UK) and British Heart Foundation.
PubMed: 38169713
DOI: 10.1016/j.eclinm.2023.102366 -
European Archives of... Apr 2024Clinical presentations encompass respiratory, feeding issues, nasopharyngeal mass, meningitis, CSF leakage, craniofacial anomalies, and endocrine problems. Surgery is... (Meta-Analysis)
Meta-Analysis Review
PURPOSE
Clinical presentations encompass respiratory, feeding issues, nasopharyngeal mass, meningitis, CSF leakage, craniofacial anomalies, and endocrine problems. Surgery is the primary treatment, transitioning from frontal craniotomy to endoscopic methods, offering improved outcomes. Yet, more studies are needed. A comprehensive review on trans-sellar trans-sphenoidal encephalocele (TSTSE) is missing. Our study aims to fill this gap, offering a comprehensive perspective for physicians.
METHODS
This review adhered to the PRISMA guideline. Eligible studies focused on human subjects, specifically trans-sellar encephaloceles, and provided comprehensive treatment details. English language articles published up to April 11th, 2023, were considered. Two trained researchers conducted article screening using consistent criteria. Data extraction covered various aspects, including clinical presentation, surgical methods, and outcomes, with results presented descriptively in two tables. Due to the rarity of this congenital anomaly, meta-analysis and publication bias assessment were not feasible. Data extraction was independently conducted by two reviewers, with subsequent cross-verification.
RESULTS
A total of 36 patients were identified from 14 studies, the most frequently observed clinical presentation was dyspnea (41.67%) and the most frequently observed accompanying anomaly was cleft lip/palate (55.56%). CT and MRI were adopted in nearly all the cases, and trans-nasal approach was the most often used surgical approach (57.14%) with the 'soft material combination' the most commonly used method for cranial base repairment (35.71%). A total of two deaths occurred and diabetes insipidus was the most common perioperative complication which occurred in six surgery patients (21.43%).
CONCLUSION
TSTSE predominantly affects males and presents with dyspnea, visual deficits, pituitary insufficiency, and cranial base-related symptoms. Early diagnosis is critical, with advanced imaging playing a key role. Endocrine assessment is vital for hormone management. Surgery offers symptom relief but entails risks, including reported fatalities and complications. The choice between surgery and conservative management requires careful deliberation. The trans-nasal approach is favored for its reduced trauma, yet further research is necessary to validate this preference.
Topics: Male; Humans; Encephalocele; Cleft Lip; Cleft Palate; Prognosis; Dyspnea
PubMed: 38189965
DOI: 10.1007/s00405-023-08355-5 -
PloS One 2024Congenital anomalies represent a significant contributor to infant mortality, morbidity, and enduring disability. With this in mind, the present investigation endeavour... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Congenital anomalies represent a significant contributor to infant mortality, morbidity, and enduring disability. With this in mind, the present investigation endeavour to ascertain the pooled prevalence of congenital anomalies and associated determinants among neonates in Ethiopia.
METHOD
PubMed, Google Scholar, CINAHAL, Hinari, and Global Health databases were systematically searched. Joanna Briggs Institute (JBI) assessment checklist was used to assess quality of included studies. Data were extracted from database and exported to stataMP-17 for analysis. Pooled prevalence was determined using DerSimonian-Laird random effects model. The degree of heterogeneity and Publication bias were assessed using I2 statistics and Eggers test, respectively. Study protocol was registered under PROSPERO ID CRD42021229140.
RESULT
A total of 18 studies with 519,327 participants were included in the study. Pooled prevalence of congenital anomalies in Ethiopia was 2% (95% CI: 0.02, 0.03%). Among affected newborns neural tube defect (48%) was the most common congenital anomaly in Ethiopia, followed by orofacial cleft (19%). Risk factors such as alcohol consumption (pooled OR: 2.28, 95% CI: 1.54, 3.38), lack of folic acid supplement (pooled OR: 2.83, 95% CI: 1.09-7.36), medication during pregnancy (pooled OR: 2.58, 95% CI: 1.03-6.47), khat (Catha edulis) chewing (pooled OR: 2.44, 95% CI: 1.61-3.71), exposure to pesticides (pooled OR: 4.45, 95% CI: 2.44-8.09) and maternal illness (pooled OR:1.79, 95% CI: 1.03, 3.10) had statistically significant association with congenital anomalies in Ethiopia.
CONCLUSION
In this review, prevalence of congenital anomalies in Ethiopia was high with considerable regional variation. The most common type of congenital anomaly in Ethiopia was neural tube defects, followed by oro-facial cleft. Alcohol consumption, inadequate intake of folic acid, khat chewing, maternal diseases, exposure to pesticides, and use of medication during pregnancy were identified as potential contributors to congenital abnormalities in Ethiopia.
Topics: Humans; Ethiopia; Prevalence; Congenital Abnormalities; Female; Risk Factors; Pregnancy; Infant, Newborn; Neural Tube Defects
PubMed: 38687732
DOI: 10.1371/journal.pone.0302393 -
Roczniki Panstwowego Zakladu Higieny 2023Folic acid (folacin, B9) is a vitamin that performs many very important functions in the human body, and its inadequate level - deficiency as well as excess, may...
Folic acid (folacin, B9) is a vitamin that performs many very important functions in the human body, and its inadequate level - deficiency as well as excess, may contribute to an increased risk of developing many disease processes. The aim of this study was to analyze the available scientific literature on folic acid and its impact on human health. A systematic review of the studies, published until November 2022, was made on the basis of searching bibliographic databases such as: PubMed, Elsevier and Google Scholar. The following keywords and combinations were used: folic acid, folate, folic acid supplementation, folate deficiency. Folic acid, thanks to its high biological activity, has a direct and indirect effect on the metabolism of the human body cells. It plays a very important role, among others in the prevention of neural tube defects and megaloblastic anemia, the proper functioning of the nervous system, as well as reducing the risk of developing certain cancers. Currently, the important role of folic acid in maintaining the proper functioning of the immune system is also emphasized, which is of particular importance both in the prevention and in the situation of SARS-CoV-2 (COVID-19) infection. The effects of deficiency and excess of vitamin B9 may turn out to be dangerous to health and even life. There is a need for nutritional and health education of the society regarding the importance of folic acid for human health, due to the presence of large deficiencies in the population, which is particularly important for some social groups, such as, for example, women of procreation age, pregnant or breastfeeding, people with a nutrient malabsorption, and people who smoke or abuse alcohol.
Topics: Pregnancy; Humans; Female; Folic Acid; COVID-19; SARS-CoV-2; Vitamins; Breast Feeding
PubMed: 37309846
DOI: 10.32394/rpzh.2023.0252 -
Prenatal Diagnosis Feb 2021We performed a systematic review to summarize the efficacy and safety of in utero stem cells application in preclinical models with myelomeningocele (MMC). (Meta-Analysis)
Meta-Analysis
OBJECTIVE
We performed a systematic review to summarize the efficacy and safety of in utero stem cells application in preclinical models with myelomeningocele (MMC).
METHODS
The study was registered with PROSPERO (CRD42019160399). We searched MEDLINE, Embase, Web of Science, Scopus and CENTRAL for publications articles on stem cell therapy in animal fetuses with MMC until May 2020. Publication quality was assessed by the SYRCLE's tool. Meta-analyses were pooled if studies were done in the same animal model providing similar type of stem cell used and outcome measurements. Narrative synthesis was performed for studies that could not be pooled.
RESULTS
Nineteen and seven studies were included in narrative and quantitative syntheses, respectively. Most used mesenchymal stem cells (MSCs) and primarily involved ovine and rodent models. Both intra-amniotic injection of allogeneic amniotic fluid (AF)-MSCs in rat MMC model and the application of human placental (P)-MSCs to the spinal cord during fetal surgery in MMC ovine model did not compromise fetal survival rates at term (rat model, relative risk [RR] 1.03, 95% CI 0.92-1.16; ovine model, RR 0.94, 95% CI 0.78-1.13). A single intra-amniotic injection of allogeneic AF-MSCs into rat MMC model was associated with a higher rate of complete defect coverage compared to saline injection (RR 16.35, 95% CI 3.27-81.79). The incorporation of human P-MSCs as a therapeutic adjunct to fetal surgery in the ovine MMC model significantly improved sheep locomotor rating scale after birth (mean difference 5.18, 95% CI 3.36-6.99).
CONCLUSIONS
Stem cell application during prenatal period in preclinical animal models is safe and effective.
Topics: Animals; Chi-Square Distribution; Female; Fetus; Meningomyelocele; Pregnancy; Rats; Sheep; Stem Cell Transplantation
PubMed: 33427329
DOI: 10.1002/pd.5887 -
Nutrients Apr 2024(1) Background: Fortifying maize and wheat flours with folic acid has effectively reduced neural tube defect-affected births. However, maize and wheat flours may not be...
(1) Background: Fortifying maize and wheat flours with folic acid has effectively reduced neural tube defect-affected births. However, maize and wheat flours may not be widely consumed in all countries; further reduction in neural tube defect-affected births could benefit from the identification of alternative food vehicles. We aimed to use dietary intake or apparent consumption data to determine alternative food vehicles for large-scale fortification with folic acid in low-income and lower-middle-income countries (LILMICs) and identify current research related to examining the technological feasibility of fortifying alternative foods with folic acid. (2) Methods: We identified 81 LILMICs, defined by the World Bank's (WB) 2018 income classifications. To identify dietary intake or apparent consumption, we reviewed WB's Microdata Library and Global Health Data Exchange for national surveys from 1997-2018. We reviewed survey reports for dietary intake or apparent consumption data and analyzed survey datasets for population coverage of foods. We defined alternative food vehicles as those that may cover/be consumed by ≥30% of the population or households; cereal grains (maize and wheat flours and rice) were included as an alternative food vehicle if a country did not have existing mandatory fortification legislation. To identify current research on fortification with folic acid in foods other than cereal grains, we conducted a systematic review of published literature and unpublished theses, and screened for foods or food products. (3) Results: We extracted or analyzed data from 18 national surveys and countries. The alternative foods most represented in the surveys were oil ( = 16), sugar ( = 16), and salt ( = 14). The coverage of oil ranged from 33.2 to 95.7%, sugar from 32.2 to 98.4%, and salt from 49.8 to 99.9%. We found 34 eligible studies describing research on alternative foods. The most studied alternative foods for fortification with folic acid were dairy products ( = 10), salt ( = 6), and various fruit juices ( = 5). (4) Conclusions: Because of their high coverage, oil, sugar, and salt emerge as potential alternative foods for large-scale fortification with folic acid. However, except for salt, there are limited or no studies examining the technological feasibility of fortifying these foods with folic acid.
Topics: Folic Acid; Food, Fortified; Humans; Neural Tube Defects; Triticum; Edible Grain; Flour; Zea mays; Developing Countries
PubMed: 38732559
DOI: 10.3390/nu16091312 -
European Journal of Physical and... Aug 2022Spinal dysraphism (SD) or spina bifida (SB) is a congenital deformity that results from embryonic neural tube closure failure during fetal development. This...
Evidence-based position paper of the UEMS PRM on the role of Physical and Rehabilitation Medicine (PRM) physician in the management of children and adults with spinal dysraphism.
Spinal dysraphism (SD) or spina bifida (SB) is a congenital deformity that results from embryonic neural tube closure failure during fetal development. This evidence-based position paper represents the official position of the European Union through the UEMS PRM Section. This paper aims to evaluate the role of the physical and rehabilitation medicine (PRM) physician and PRM practice for children and adults with spinal dysraphism. A systematic literature review and a consensus procedure involved all European countries delegates represented in the UEMS PRM section through a Delphi process. The systematic literature review is reported together with thirty-two recommendations resulting from the Delphi procedure. The professional role of the PRM physician requires specific expertise in the treatment of patients with SD to plan, lead and monitor the rehabilitation process in an interdisciplinary setting and to participate in the assessment of the needs of these patients in the transitional phase from childhood to adulthood, with particular attention to the activity limitation and participation restriction.
Topics: Adolescent; Child; Europe; European Union; Humans; Physical and Rehabilitation Medicine; Physicians; Spinal Dysraphism; Young Adult
PubMed: 35575455
DOI: 10.23736/S1973-9087.22.07536-0