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PloS One 2012The introduction of sophisticated treatment of bladder dysfunction and hydrocephalus allows the majority of SB patients to survive into adulthood. However, no systematic... (Review)
Review
BACKGROUND
The introduction of sophisticated treatment of bladder dysfunction and hydrocephalus allows the majority of SB patients to survive into adulthood. However, no systematic review on urological outcome in adult SB patients is available and no follow-up schemes exist.
OBJECTIVES
To systematically summarize the evidence on outcome of urinary tract functioning in adult SB patients.
METHODS
A literature search in PubMed and Embase databases was done. Only papers published in the last 25 years describing patients with open SB with a mean age >18 years were included. We focused on finding differences in the treatment strategies, e.g., clean intermittent catheterization and antimuscarinic drugs versus early urinary diversion, with regard to long-term renal and bladder outcomes.
RESULTS
A total of 13 articles and 5 meeting abstracts on urinary tract status of adult SB patients were found describing a total of 1564 patients with a mean age of 26.1 years (range 3-74 years, with a few patients <18 years). All were retrospective cohort studies with relatively small and heterogeneous samples with inconsistent reporting of outcome; this precluded the pooling of data and meta-analysis. Total continence was achieved in 449/1192 (37.7%; range 8-85%) patients. Neurological level of the lesion and hydrocephalus were associated with incontinence. Renal function was studied in 1128 adult patients. In 290/1128 (25.7%; range 3-81.8%) patients some degree of renal damage was found and end-stage renal disease was seen in 12/958 (1.3%) patients. Detrusor-sphincter dyssynergy and detrusor-overactivity acted as adverse prognostic factors for the development of renal damage.
CONCLUSIONS
These findings should outline follow-up schedules for SB patients, which do not yet exist. Since renal and bladder deterioration continues beyond adolescence, follow-up of these individuals is needed. We recommend standardization in reporting the outcome of urinary tract function in adult SB patients.
Topics: Adult; Humans; Meningomyelocele; Prognosis; Risk Factors; Urinary Tract
PubMed: 23119003
DOI: 10.1371/journal.pone.0048399 -
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 -
Fetal Diagnosis and Therapy 2018The Management of Myelomeningocele (MMC) Study (MOMS) showed that prenatal repair of MMC resulted in improved neurological outcomes but was associated with high rates of... (Comparative Study)
Comparative Study Meta-Analysis Review
BACKGROUND/OBJECTIVES
The Management of Myelomeningocele (MMC) Study (MOMS) showed that prenatal repair of MMC resulted in improved neurological outcomes but was associated with high rates of obstetrical complications. This study compares outcomes of open and fetoscopic MMC repair.
DATA SOURCES
PubMed and Embase studies reporting outcomes of fetal MMC repair published since the completion of the MOMS.
RESULTS
We analyzed 11 studies and found no difference in mortality or the rate of shunt placement for hydrocephalus. Percutaneous fetoscopic repair was associated with higher rates of premature rupture of membranes (91 vs. 36%, p < 0.01) and preterm birth (96 vs. 81%, p = 0.04) compared to open repair, whereas fetoscopic repair via maternal laparotomy reduced preterm birth. The rate of dehiscence and leakage from the MMC repair site was higher after both types of fetoscopic surgery (30 vs. 7%, p < 0.01), while the rate of uterine dehiscence was higher after open repair (11 vs. 0%, p < 0.01).
DISCUSSION
Fetoscopic repair is a promising alternative to open fetal MMC repair with a lower risk of uterine dehiscence; however, fetoscopic techniques should be optimized to overcome the high rate of dehiscence and leakage at the MMC repair site. A fetoscopic approach via maternal laparotomy reduces the risk of preterm birth.
Topics: Female; Fetal Diseases; Fetoscopy; Humans; Meningomyelocele; Pregnancy
PubMed: 28910784
DOI: 10.1159/000479505 -
Ultrasound in Obstetrics & Gynecology :... Aug 2016To assess and compare the rate of procedure-related complications after intrauterine treatment of spina bifida by endoscopic surgery and by open fetal surgery. (Comparative Study)
Comparative Study Meta-Analysis Review
Procedure-related complications of open vs endoscopic fetal surgery for treatment of spina bifida in an era of intrauterine myelomeningocele repair: systematic review and meta-analysis.
OBJECTIVE
To assess and compare the rate of procedure-related complications after intrauterine treatment of spina bifida by endoscopic surgery and by open fetal surgery.
METHODS
Systematic literature searches in PubMed and SCOPUS databases were performed on 20 September 2015 to identify randomized controlled trials and observational studies on treatment of human spina bifida by endoscopic or open fetal surgery techniques. Only studies with ≥ 10 cases that were published in or after 2000 were included in the meta-analysis in order to reduce the risk of bias. Primary outcomes (complete dehiscence, focal dehiscence and/or markedly thin hysterotomy scar; preterm delivery < 34 weeks; mean gestational age at delivery) and secondary outcomes (oligohydramnios, prelabor rupture of membranes, placental abruption, chorioamnionitis and perinatal death) were assessed for both techniques. Precision of the estimated proportions was evaluated with 95% CIs. Inconsistency was assessed using the I(2) statistic.
RESULTS
The search identified 1080 records that were examined based on title and abstract, of which 28 full-text articles were examined completely for eligibility. Nine records were excluded because cases were also described in other studies, leaving 19 records for analysis. When comparing endoscopic vs open fetal surgery, the rate of complete dehiscence, focal dehiscence and/or markedly thin hysterotomy scar was, respectively, 1% (95% CI, 0-4%) vs 26% (95% CI, 12-42%); preterm delivery < 34 weeks was 80% (95% CI, 41-100%) vs 45% (95% CI, 38-53%); oligohydramnios was 39% (95% CI, 9-75%) vs 14% (95% CI, 7-24%); prelabor rupture of membranes was 67% (95% CI, 12-100%) vs 38% (95% CI, 26-50%); and perinatal death was 14% (95% CI, 1-38%) vs 5% (95% CI, 3-8%).
CONCLUSION
Open fetal surgery for spina bifida seems to show lower rates of procedure-related complications than does endoscopic surgery, but the rate of hysterotomy scar complications is high after open surgery. Because of the low quality of evidence, the conclusions should be interpreted with caution. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd.
Topics: Female; Fetoscopy; Humans; Infant, Newborn; Meningomyelocele; Observational Studies as Topic; Postoperative Complications; Pregnancy; Randomized Controlled Trials as Topic; Spinal Dysraphism; Treatment Outcome
PubMed: 26612040
DOI: 10.1002/uog.15830 -
International Journal of Molecular... Feb 2023Neurogenic detrusor overactivity (NDO) is a severe lower urinary tract disorder, characterized by urinary urgency, retention, and incontinence, as a result of a... (Review)
Review
Neurogenic detrusor overactivity (NDO) is a severe lower urinary tract disorder, characterized by urinary urgency, retention, and incontinence, as a result of a neurologic lesion that results in damage in neuronal pathways controlling micturition. The purpose of this review is to provide a comprehensive framework of the currently used animal models for the investigation of this disorder, focusing on the molecular mechanisms of NDO. An electronic search was performed with PubMed and Scopus for literature describing animal models of NDO used in the last 10 years. The search retrieved 648 articles, of which reviews and non-original articles were excluded. After careful selection, 51 studies were included for analysis. Spinal cord injury (SCI) was the most frequently used model to study NDO, followed by animal models of neurodegenerative disorders, meningomyelocele, and stroke. Rats were the most commonly used animal, particularly females. Most studies evaluated bladder function through urodynamic methods, with awake cystometry being particularly preferred. Several molecular mechanisms have been identified, including changes in inflammatory processes, regulation of cell survival, and neuronal receptors. In the NDO bladder, inflammatory markers, apoptosis-related factors, and ischemia- and fibrosis-related molecules were found to be upregulated. Purinergic, cholinergic, and adrenergic receptors were downregulated, as most neuronal markers. In neuronal tissue, neurotrophic factors, apoptosis-related factors, and ischemia-associated molecules are increased, as well as markers of microglial and astrocytes at lesion sites. Animal models of NDO have been crucial for understanding the pathophysiology of lower urinary tract (LUT) dysfunction. Despite the heterogeneity of animal models for NDO onset, most studies rely on traumatic SCI models rather than other NDO-driven pathologies, which may result in some issues when translating pre-clinical observations to clinical settings other than SCI.
Topics: Female; Rats; Animals; Urinary Bladder; Urinary Bladder, Neurogenic; Urinary Bladder, Overactive; Urinary Incontinence; Spinal Cord Injuries; Models, Animal; Urodynamics
PubMed: 36834694
DOI: 10.3390/ijms24043273 -
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 -
Medicina (Kaunas, Lithuania) Jul 2021: Myelomeningocele is the most severe form of spina bifida, a congenital neural tube defect arising from an incomplete neural tube closure during early development with... (Review)
Review
: Myelomeningocele is the most severe form of spina bifida, a congenital neural tube defect arising from an incomplete neural tube closure during early development with damage worsening with advancing gestational age. The Management of Myelomeningocele Study (MOMS) Trial proved that surgery performed before 26 weeks of gestation significantly improved the prognosis, significantly changing treatment paradigms. This article aims to provide a review of the changes and updates in spina bifida repair over the 10-year period following the MOMS Trial. We performed a systematic review in the PubMed and Cochrane databases as well as a hand-search of high-impact journals using the reference list of all identified articles, searching for randomized controlled trials and observational studies. We identified 27 articles published between 2011 and 2021 that fulfilled the inclusion criteria and review them in the present study. With growing experience and with the improvement of prenatal open and fetoscopic techniques, the outcome of SB-associated conditions could be improved and the risks to both the mother and the fetus reduced. A continuous follow-up of the treated infants and further randomized trials are essential to study the complications and advantages or disadvantages of any given treatment strategy.
Topics: Female; Fetus; Gestational Age; Humans; Infant; Meningomyelocele; Neural Tube Defects; Pregnancy; Spinal Dysraphism
PubMed: 34356988
DOI: 10.3390/medicina57070707 -
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 -
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 -
The Cochrane Database of Systematic... Oct 2014Spina 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... (Comparative Study)
Comparative Study Review
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.
Topics: Female; Gestational Age; Humans; Infant, Newborn; Pregnancy; Pregnancy Outcome; Premature Birth; Prenatal Care; Randomized Controlled Trials as Topic; Spinal Dysraphism
PubMed: 25348498
DOI: 10.1002/14651858.CD008825.pub2