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Journal of Orthopaedic Surgery and... Aug 2021Myelomeningocele (MMC) is the most common and severe form of spina bifida and imposes a significant burden on patients and the healthcare system. Recently, the... (Review)
Review
BACKGROUND
Myelomeningocele (MMC) is the most common and severe form of spina bifida and imposes a significant burden on patients and the healthcare system. Recently, the multidisciplinary management of MMC has become popular. Herein, we aimed to review the orthopedic management, outcomes, and complications of the of patients with MMC eyeing a multidisciplinary approach.
METHODS
We searched PubMed and EMBASE to find relevant studies published before August 2020. All studies that included clinical management of MMC patients and published earlier than 2000 were considered for review on the condition that they reported at least one orthopedic intervention and the rate of complications. We excluded review articles, case reports, case series, letters, commentaries, editorials, and conference abstracts. The primary and secondary goals of our review were to report the outcomes and complication rates of multidisciplinary management for MMC patients.
RESULTS
Twenty-six studies included data for the management of 229,791 patients with MMC and were selected. Sixteen studies reported multidisciplinary management in addition to orthopedic management. From those, 11 (42.31%) included urologic management, 13 (50%) neurosurgical management, 11 (42.31%) neurologic management, and 5 (19.23%) gastrointestinal management. All studies included postnatal operations and related management. No randomized clinical trial was found in our search.
CONCLUSION
Orthopedic approaches play a key role in MMC management by alleviating spinal deformities, particularly scoliosis, and hip, foot, and ankle complications. However, the most appropriate management, whether surgical or non-surgical, may vary for different patients, given disease severity and the age of patients.
Topics: Humans; Meningomyelocele; Orthopedics; Scoliosis
PubMed: 34389028
DOI: 10.1186/s13018-021-02643-8 -
Ultrasound in Obstetrics & Gynecology :... Jun 2020The Management of Myelomeningocele Study (MOMS) trial demonstrated the safety and efficacy of open fetal surgery for spina bifida aperta (SBA). Recently developed... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The Management of Myelomeningocele Study (MOMS) trial demonstrated the safety and efficacy of open fetal surgery for spina bifida aperta (SBA). Recently developed alternative techniques may reduce maternal risks without compromising the fetal neuroprotective effects. The aim of this systematic review was to assess the learning curve (LC) of different fetal SBA closure techniques.
METHODS
MEDLINE, Web of Science, EMBASE, Scopus and Cochrane databases and the gray literature were searched to identify relevant articles on fetal surgery for SBA, without language restriction, published between January 1980 and October 2018. Identified studies were reviewed systematically and those reporting all consecutive procedures and with postnatal follow-up ≥ 12 months were selected. Studies were included only if they reported outcome variables necessary to measure the LC, as defined by fetal safety and efficacy. Two authors independently retrieved data, assessed the quality of the studies and categorized observations into blocks of 30 patients. For meta-analysis, data were pooled using a random-effects model when heterogeneous. To measure the LC, we used two complementary methods. In the group-splitting method, competency was defined when the procedure provided results comparable to those in the MOMS trial for 12 outcome variables representing the immediate surgical outcome, short-term neonatal neuroprotection and long-term neuroprotection at ≥ 12 months of age. Then, when raw patient data were available, we performed cumulative sum analysis based on a composite binary outcome defining successful surgery. The composite outcome combined four clinically relevant variables for safety (absence of extreme preterm delivery < 30 weeks, absence of fetal death ≤ 7 days after surgery) and efficacy (reversal of hindbrain herniation and absence of any neonatal treatment of dehiscence or cerebrospinal fluid leakage at the closure site).
RESULTS
Of 6024 search results, 17 (0.3%) studies were included, all of which had low, moderate or unclear risk of bias. Fetal SBA closure was performed using standard hysterotomy (11 studies), mini-hysterotomy (one study) or fetoscopy by either exteriorized-uterus single-layer closure (one study), percutaneous single-layer closure (three studies) or percutaneous two-layer closure (one study). Only outcomes for standard hysterotomy could be meta-analyzed. Overall, outcomes improved significantly with experience. Competency was reached after 35 consecutive cases for standard hysterotomy and was predicted to be achieved after ≥ 57 cases for mini-hysterotomy and ≥ 56 for percutaneous two-layer fetoscopy. For percutaneous and exteriorized-uterus single-layer fetoscopy, competency was not reached in the 81 and 28 cases available for analysis, respectively, and LC prediction analysis could not be performed.
CONCLUSIONS
The number of cases operated is correlated with the outcome of fetal SBA closure, and the number of operated cases required to reach competency ranges from 35 for standard hysterotomy to ≥ 56-57 for minimally invasive modifications. Our observations provide important information for institutions looking to establish a new fetal center, develop a new fetal surgery technique or train their team, and inform referring clinicians, potential patients and third parties. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.
Topics: Adult; Female; Fetoscopy; Fetus; Humans; Hysterotomy; Learning Curve; Pregnancy; Spina Bifida Cystica
PubMed: 31273862
DOI: 10.1002/uog.20389 -
Reproductive Health Oct 2022Anencephaly is a fatal congenital anomaly characterized by the absence of brain hemispheres and cranial arch. Timely preventive measures can be taken by knowing the... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Anencephaly is a fatal congenital anomaly characterized by the absence of brain hemispheres and cranial arch. Timely preventive measures can be taken by knowing the exact prevalence of this common neural tube defect; thus, carried out through systematic review and meta-analysis, the present study was conducted to determine the worldwide prevalence, incidence and mortality of anencephaly.
METHODS
Cochran's seven-step instructions were used as the guideline. Having determined the research question and inclusion and exclusion criteria, we studied MagIran, SID, Science Direct, WoS, Web of Science, Medline (PubMed), Scopus, and Google Scholar databases. Moreover, the search strategy in each database included using all possible keyword combinations with the help of "AND" and "OR" operators with no time limit to 2021. The I test was used to calculate study heterogeneity, and Begg and Mazumdar rank correlation tests were employed to assess the publication bias. Data were analyzed by Comprehensive Meta-Analysis software (Version 2).
RESULTS
In this study, the statements of Preferred Reporting Items for Systematic Reviews and Meta-Analyzes (PRISMA) were used. In the first stage, 1141 articles were found, of which 330 duplicate studies were omitted. 371 articles were deleted based on the inclusion and exclusion criteria by reviewing the title and abstract of the study. 58 articles were removed by reviewing the full text of the article because it was not relevant to the research. 360 studies with a sample size of 207,639,132 people were considered for the meta-analysis. Overall estimate of the prevalence, incidence and attenuation of anencephaly worldwide were 5.1 per ten thousand births (95% confidence interval 4.7-5.5 per ten thousand births), 8.3 per ten thousand births (95% confidence interval 5.5-9.9 per ten thousand births), 5.5 per ten thousand births (95% confidence interval 1.8-15 per ten thousand births) respectively the highest of which according to the subgroup analysis, belonged to the Australian continent with 8.6 per ten thousand births (95% confidence interval 7.7-9.5 per ten thousand births).
CONCLUSION
The overall prevalence of anencephaly in the world is significant, indicating the urgent need for preventive and treating measures.
Topics: Anencephaly; Australia; Humans; Neural Tube Defects; Prevalence
PubMed: 36253858
DOI: 10.1186/s12978-022-01509-4 -
Genes Oct 2021The maternal environment during the periconceptional period influences foetal growth and development, in part, via epigenetic mechanisms moderated by one-carbon... (Review)
Review
The maternal environment during the periconceptional period influences foetal growth and development, in part, via epigenetic mechanisms moderated by one-carbon metabolic pathways. During embryonic development, one-carbon metabolism is involved in brain development and neural programming. Derangements in one-carbon metabolism increase (i) the short-term risk of embryonic neural tube-related defects and (ii) long-term childhood behaviour, cognition, and autism spectrum disorders. Here we investigate the association between maternal one-carbon metabolism and foetal and neonatal brain growth and development. Database searching resulted in 26 articles eligible for inclusion. Maternal vitamin B, vitamin B, homocysteine, and choline were not associated with foetal and/or neonatal head growth. First-trimester maternal plasma folate within the normal range (>17 nmol/L) associated with increased foetal head size and head growth, and high erythrocyte folate (1538-1813 nmol/L) with increased cerebellar growth, whereas folate deficiency (<7 nmol/L) associated with a reduced foetal brain volume. Preconceptional folic acid supplement use and specific dietary patterns (associated with increased B vitamins and low homocysteine) increased foetal head size. Although early pregnancy maternal folate appears to be the most independent predictor of foetal brain growth, there is insufficient data to confirm the link between maternal folate and offspring risks for neurodevelopmental diseases.
Topics: Brain; Carbon; Embryonic Development; Female; Fetal Development; Fetus; Folic Acid; Humans; Pregnancy; Vitamin B 12
PubMed: 34681028
DOI: 10.3390/genes12101634 -
Jornal de Pediatria 2023To map available scientific evidence about the pediatric population with spina bifida submitted to transanal irrigation to manage signs and symptoms of neurogenic bowel. (Review)
Review
OBJECTIVE
To map available scientific evidence about the pediatric population with spina bifida submitted to transanal irrigation to manage signs and symptoms of neurogenic bowel.
SOURCE OF DATA
This research was developed according to recommendations from the Joanna Briggs Institute Reviewers' Manual and the PRISMA Extension for Scoping Reviews. Searches were carried out in the databases: CINAHL, Medline/Pubmed, Scielo, Scopus, Web of Science, Embase, LILACS, Proquest, and the CAPES catalog of theses and dissertations. Quantitative and qualitative studies on the topic were included, as long as they dealt with this population. There was no predetermined time frame.
SUMMARY OF THE FINDINGS
The authors found 1.020 studies, selected 130 for close reading, and included 23 in the review, all of which had been published from 1989 to 2021. The authors mapped the characteristics of the studies, including their definitions of concepts and use of scales, criteria for the indication of transanal irrigation, training to carry out the procedure, devices and solutions used, number and frequency of transanal irrigations, health care actions, time spent, associated complications, complementary exams, adherence rate, follow-up, and outcomes, focusing on the benefits for bowel management.
CONCLUSIONS
Despite the variability of evaluation parameters and term definitions, evidence suggests that transanal irrigation is a safe and effective method to manage fecal incontinence. Studies in the field are likely to grow, using standardized scales and longitudinal follow-ups. The authors suggest further research on transanal irrigation in the pediatric population with spina bifida in the Latin American context.
Topics: Humans; Child; Neurogenic Bowel; Constipation; Therapeutic Irrigation; Fecal Incontinence; Spinal Dysraphism
PubMed: 36852756
DOI: 10.1016/j.jped.2023.02.001 -
The Journal of Urology Jun 2022We systematically reviewed the variability in definitions of kidney abnormality (KA) outcomes in individuals with spina bifida (SB). (Review)
Review
PURPOSE
We systematically reviewed the variability in definitions of kidney abnormality (KA) outcomes in individuals with spina bifida (SB).
MATERIALS AND METHODS
A systematic scoping review was conducted using MEDLINE, Embase™, Cochrane Library, CINAHL, PsycInfo®, Web of Science™ and ClinicalTrials.gov for articles from time of database inception to September 2020. No language or patient age restrictions were applied. Primary research articles involving individuals with SB where KA was assessed as an outcome were included. Means of assessing KA and defining KA severity were abstracted.
RESULTS
Of 2,034 articles found, 274 were included in the review. Most articles were published after 1990 (63.5%) and included pediatric-only populations (0-18 years; 60.5%). KA outcomes were identified by imaging-based anatomical outcomes (84.7%), serum-based outcomes (44.9%), imaging-based functional outcomes (5.5%), urine-based outcomes (3.3%) and diagnoses of end-stage kidney disease (2.6%) or chronic kidney disease otherwise unspecified (1.8%). Hydronephrosis was the most commonly used specific outcome (64.6%, 177/274) with 19.8% (35/177) of articles defining hydronephrosis severity. Hydronephrosis was used more frequently in articles with pediatric-only populations. Creatinine and cystatin-C were used in 82.1% (101/123) and 17.9% (22/123) of articles reporting serum-based outcomes, respectively, with 32.7% and 50.0% of articles defining estimated glomerular filtration rate (GFR) severity. Serum-based outcomes were more common in articles including adults >18 years. Measured GFR was assessed in 9.9% (27/274) of articles, with 44.4% (12/27) of articles defining GFR severity.
CONCLUSIONS
Significant variability exists in how authors define KA with few specifically defining KA severity. Consensus and consistency in defining KA outcomes are needed.
Topics: Adult; Child; Female; Glomerular Filtration Rate; Humans; Hydronephrosis; Kidney; Male; Renal Insufficiency, Chronic; Spinal Dysraphism
PubMed: 35050700
DOI: 10.1097/JU.0000000000002432 -
World Neurosurgery Aug 2021This study aimed to compare the effectiveness of posterior fossa decompression (PFD), posterior fossa decompression with duraplasty (PFDD), and posterior fossa... (Comparative Study)
Comparative Study Meta-Analysis
Comparative Assessment of Three Posterior Fossa Decompression Techniques and Evaluation of the Evidence Supporting the Efficacy of Syrinx Shunting and Filum Terminale Sectioning in Chiari Malformation Type I. A Systematic Review and Network Meta-Analysis.
BACKGROUND
This study aimed to compare the effectiveness of posterior fossa decompression (PFD), posterior fossa decompression with duraplasty (PFDD), and posterior fossa decompression with resection of tonsils (PFDRT) in Chiari malformation type I (CMI). Furthermore, we aimed to evaluate the evidence supporting the efficacy of filum terminale sectioning (FTS), syringosubarachnoid shunting (SSS), and syringoperitoneal shunting (SPS) in CMI.
METHODS
PubMed, Cochrane, and Embase databases were screened for English-language studies published from inception until August 11, 2020. A total of 3593 studies were identified through the searching process. Fifteen cohort studies were included in the systematic review and network meta-analysis.
RESULTS
No studies concerning FTS, SSS, and SPS were found eligible. Therefore, only PFD, PFDD, and PFDRT were compared. PFDD showed significantly higher incidence of complications (relative risk [RR], 3.79; 95% confidence interval [CI], 2.54-5.64) cerebrospinal fluid leak (RR, 9.74; 95% CI, 2.33-40.78) and neurologic deficit (RR, 8.76; 95% CI, 2.08-36.90) than did PFD. Both PFDD and PFDRT achieved higher syringomyelia improvement (RR, 1.23, 95% CI, 1.09-1.39 and RR, 1.32, 95% CI, 1.15-1.51, respectively) and greater clinical improvement (RR, 1.24, 95% CI, 1.10-1.39 and RR, 1.24, 95% CI, 1.08-1.44, respectively) than did PFD. No differences were found between PFDD and PFDRT.
CONCLUSIONS
PFDD and PFDRT are superior to PFD, especially in patients with syringomyelia-Chiari complex, because of greater syringomyelia reduction and better clinical improvement. However, PFDD and PFDRT can be considered equally efficient. There is no evidence pleading in favor of SFT, SSS, and SPS over any PFD technique.
Topics: Arnold-Chiari Malformation; Cauda Equina; Cerebrospinal Fluid Shunts; Decompression, Surgical; Humans; Neurosurgical Procedures
PubMed: 34098134
DOI: 10.1016/j.wneu.2021.05.124 -
The Journal of Maternal-fetal &... Dec 2023Multiple factors associated with neural tube defects (NTDs) risk have been identified, yet there is little evidence on the possible effects of maternal stressful life... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVES
Multiple factors associated with neural tube defects (NTDs) risk have been identified, yet there is little evidence on the possible effects of maternal stressful life events. In this study, we aimed to investigate the association between stressful life events during the periconceptional period and risk of NTDs in offspring.
METHODS
Relevant literature was searched in PubMed, Springer Link, ScienceDirect, and Cochrane Library up to July 2023. The pooled odds ratio (OR) and 95% confidence interval (CI) of NTDs risk with maternal stressful life events were estimated using a random effects model. Publication bias was assessed using Egger's regression asymmetry test and Begg's rank correlation test with Begg's funnel plot.
RESULTS
Analysis results showed that mothers who experienced stressful life events during the periconceptional period were at greater risk of having NTDs offspring (OR: 1.37, 95% CI: 1.08-1.73) than those who did not. In subgroup analysis, the pooled OR was 1.37 (1.13-1.67) and 1.73 (0.36-8.32) for with and without adjusting for folic acid supplementation in each included study, while was 1.37 (1.13-1.67) and 1.64 (0.39-6.88) for exposure time of three months preconception until three months post conception and one year preconception until three months post conception, respectively.
CONCLUSIONS
This study suggests that maternal stressful life events during the periconceptional period are significantly associated with higher NTDs risk in offspring. Tailored approaches for evaluating the risk and policy of NTDs among women of childbearing age should emphasize individual stressful experiences before and during early pregnancy.
Topics: Pregnancy; Female; Humans; Neural Tube Defects; Mothers; Fertilization; Odds Ratio; Folic Acid
PubMed: 37946331
DOI: 10.1080/14767058.2023.2279021 -
Ultrasound in Obstetrics & Gynecology :... Sep 2021To compare the perinatal outcomes between pregnancies with and those without iatrogenic chorioamniotic separation (iCAS) following fetoscopic intervention. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To compare the perinatal outcomes between pregnancies with and those without iatrogenic chorioamniotic separation (iCAS) following fetoscopic intervention.
METHODS
We performed a search in PubMed, EMBASE, Scopus, Web of Science and Google Scholar from inception up to December 2020 for studies comparing perinatal outcomes between pregnancies that developed and those that did not develop iCAS after fetoscopic intervention for twin-to-twin transfusion syndrome (TTTS), open neural tube defect (ONTD) or congenital diaphragmatic hernia. A random-effects model was used to pool the mean differences (MD) or odds ratios (OR) and the corresponding 95% CI. The primary outcome was neonatal survival. Secondary outcomes included gestational age (GA) at intervention and at delivery, interval from intervention to delivery and incidence of preterm prelabor rupture of membranes (PPROM) and preterm delivery. The methodological quality of the included studies was evaluated using the Newcastle-Ottawa scale.
RESULTS
The search identified 348 records, of which seven studies (six on fetoscopic laser photocoagulation (FLP) for TTTS and one on fetoscopic repair for ONTD) assessed the perinatal outcomes of pregnancies that developed iCAS after fetoscopic intervention. Given that only one study reported on fetoscopic ONTD repair, the meta-analysis was limited to TTTS pregnancies and included six studies (total of 1881 pregnancies). Pregnancies that developed iCAS after FLP for TTTS, compared with those that did not, had significantly lower GA at the time of intervention (weeks) (MD, -1.07 (95% CI, -1.89 to -0.24); P = 0.01) and at delivery (weeks) (MD, -1.74 (95% CI, -3.13 to -0.34); P = 0.01) and significantly lower neonatal survival (OR, 0.41 (95% CI, 0.24-0.70); P = 0.001). In addition, development of iCAS after FLP for TTTS increased significantly the risk for PPROM < 34 weeks' gestation (OR, 3.98 (95% CI, 1.76-9.03); P < 0.001) and preterm delivery < 32 weeks (OR, 1.80 (95% CI, 1.16-2.80); P = 0.008).
CONCLUSIONS
iCAS is a common complication after FLP for TTTS. In patients undergoing FLP for TTTS, iCAS develops more often with earlier GA at intervention and is associated with earlier GA at delivery, higher risk of PPROM < 34 weeks' gestation and preterm delivery < 32 weeks and lower neonatal survival. Given the limitations of this meta-analysis and lack of literature reporting on other types of fetoscopic intervention, the presented findings should be interpreted with caution and should not be generalized to fetoscopic procedures used to treat other fetal conditions. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Adult; Delivery, Obstetric; Female; Fetal Membranes, Premature Rupture; Fetofetal Transfusion; Fetoscopy; Gestational Age; Hernias, Diaphragmatic, Congenital; Humans; Iatrogenic Disease; Incidence; Infant, Newborn; Neural Tube Defects; Odds Ratio; Postoperative Complications; Pregnancy; Pregnancy Outcome; Pregnancy, Twin; Premature Birth
PubMed: 33428299
DOI: 10.1002/uog.23588 -
Frontiers in Pharmacology 2022Hyperemesis gravidarum is a serious pregnancy complication that affects approximately 1% of pregnancies worldwide. To determine whether the use of ondansetron during...
Hyperemesis gravidarum is a serious pregnancy complication that affects approximately 1% of pregnancies worldwide. To determine whether the use of ondansetron during pregnancy is associated with abnormal pregnancy outcomes. PubMed, Cochrane Library, CINAHL, Embase, CNKI, CBM, WANFANG, and ClinicalTrials.gov were searched for citations published in any language from inception to 15 December 2021. Eligible studies included any observational study. Odds ratio (OR) and 95% confidence interval (CI) were used as indicators to examine the association between ondansetron and abnormal pregnancy outcomes. Twenty articles from 1,558 citations were included. Our preliminary analysis showed that compared with the unexposed group, the use of ondansetron during pregnancy may be associated with an increased incidence of cardiac defects (OR = 1.06, 95% CI: 1.01-1.10), neural tube defects (OR = 1.12, 95% CI: 1.05-1.18), and chest cleft (OR = 1.21, 95% CI: 1.07-1.37). Further sensitivity analysis showed no significant association between ondansetron and cardiac defects (OR = 1.15,95% CI: 0.94-1.40) or neural tube defects (OR = 0.87,95% CI: 0.46-1.66). When controversial studies were eliminated, the results for the chest defects disappeared. Simultaneously, we found that the use of ondansetron was associated with a reduced incidence of miscarriage (OR = 0.53, 95% CI: 0.31-0.89). Ondansetron was not associated with orofacial clefts (OR = 1.09,95% CI: 0.95-1.25), spinal limb defects (OR = 1.14,95% CI: 0.89-1.46), urinary tract deformities (OR = 1.06,95% CI: 0.97-1.15), any congenital malformations (OR = 1.03,95% CI: 0.98-1.09), stillbirth (OR = 0.97,95% CI: 0.83-1.15), preterm birth (OR = 1.22,95% CI: 0.80-1.85), neonatal asphyxia (OR = 1.05,95% CI: 0.72-1.54), or neonatal development (OR = 1.18,95% CI: 0.96-1.44) in our primary analysis. In our analysis, using ondansetron during pregnancy was not associated with abnormal pregnancy outcomes. Although our study did not find sufficient evidence of ondansetron and adverse pregnancy outcomes, future studies including the exposure period and dose of ondansetron, as well as controlling for disease status, may be useful to truly elucidate the potential risks and benefits of ondansetron.
PubMed: 36120333
DOI: 10.3389/fphar.2022.951072