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Neurosurgical Focus Oct 2019Myelomeningocele (MMC), the most severe form of spina bifida, is characterized by protrusion of the meninges and spinal cord through a defect in the vertebral arches.... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Myelomeningocele (MMC), the most severe form of spina bifida, is characterized by protrusion of the meninges and spinal cord through a defect in the vertebral arches. The management and prevention of MMC-associated hydrocephalus has evolved since its initial introduction with regard to treatment of MMC defect, MMC-associated hydrocephalus treatment modality, and timing of hydrocephalus treatment.
METHODS
The Nationwide Inpatient Sample (NIS) database from the years 1998-2014 was reviewed and neonates with spina bifida and hydrocephalus status were identified. Timing of hydrocephalus treatment, delayed treatment (DT) versus simultaneous MMC repair with hydrocephalus treatment (ST), and treatment modality (ETV vs ventriculoperitoneal shunt [VPS]) were analyzed. Yearly trends were assessed with univariable logarithmic regression. Multivariable logistic regression identified correlates of inpatient shunt failure. A PRISMA systematic literature review was conducted that analyzed data from studies that investigated 1) MMC closure technique and hydrocephalus rate, 2) hydrocephalus treatment modality, and 3) timing of hydrocephalus treatment.
RESULTS
A weighted total of 10,627 inpatient MMC repairs were documented in the NIS, 8233 (77.5%) of which had documented hydrocephalus: 5876 (71.4%) were treated with VPS, 331 (4.0%) were treated with ETV, and 2026 (24.6%) remained untreated on initial inpatient stay. Treatment modality rates were stable over time; however, hydrocephalic patients in later years were less likely to receive hydrocephalus treatment during initial inpatient stay (odds ratio [OR] 0.974, p = 0.0331). The inpatient hydrocephalus treatment failure rate was higher for patients who received ETV treatment (17.5% ETV failure rate vs 7.9% VPS failure rate; p = 0.0028). Delayed hydrocephalus treatment was more prevalent in the later time period (77.9% vs 69.5%, p = 0.0287). Predictors of inpatient shunt failure included length of stay, shunt infection, jaundice, and delayed treatment. A longer time between operations increased the likelihood of inpatient shunt failure (OR 1.10, p < 0.0001). However, a meta-analysis of hydrocephalus timing studies revealed no difference between ST and DT with respect to shunt failure or infection rates.
CONCLUSIONS
From 1998 to 2014, hydrocephalus treatment has become more delayed and the number of hydrocephalic MMC patients not treated on initial inpatient stay has increased. Meta-analysis demonstrated that shunt malfunction and infection rates do not differ between delayed and simultaneous hydrocephalus treatment.
Topics: Female; Humans; Hydrocephalus; Infant, Newborn; Male; Meningomyelocele; Neuroendoscopy; Postoperative Complications; Third Ventricle; Treatment Failure; Treatment Outcome; Ventriculoperitoneal Shunt; Ventriculostomy
PubMed: 31574479
DOI: 10.3171/2019.7.FOCUS19469 -
Physical & Occupational Therapy in... 2009This systematic review provides a critical synthesis of research regarding the effects of electrical stimulation, exercise training, and motor skills training on muscle... (Review)
Review
This systematic review provides a critical synthesis of research regarding the effects of electrical stimulation, exercise training, and motor skills training on muscle strength in children with meningomyelocele. Nine databases were searched using terms related to meningomyelocele and physical therapy interventions. Of 298 potentially relevant citations, six met the inclusion criteria. Each was rated using the systematic review guidelines of the American Academy for Cerebral Palsy and Developmental Medicine. Two studies examined changes in quadriceps muscle torque following electrical stimulation, three investigated upper extremity exercise training, and one evaluated quadriceps strength after motor skills training. Although the limited evidence suggests improvements in strength when using these interventions, much of the evidence is of low methodological quality and all studies were published more than 10 years ago. Further research is needed regarding various strength-training interventions for children with meningomyelocele and the relationship between increased strength and improved activity and participation.
Topics: Child; Electric Stimulation Therapy; Humans; Meningomyelocele; Muscle, Skeletal; Physical Therapy Modalities; Resistance Training
PubMed: 19916827
DOI: 10.3109/01942630903246018 -
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 -
Pediatric Surgery International Jul 2022Heteropagus twinning (HT) is a rare anomaly. Six new cases along with a systematic review are described.
PURPOSE
Heteropagus twinning (HT) is a rare anomaly. Six new cases along with a systematic review are described.
METHODS
Six cases of HT managed at two tertiary care teaching hospitals over the last 26 years are described. A PubMed search with words: Heteropagus AND/ OR parasitic twins from 2001 to 2021 hit 183 articles. 36 were added from non-PubMed sources. Finally, 120 cases including 114 from 69 articles and 6 new cases were analysed.
RESULTS
Of the new cases, 2/6 had an antenatal diagnosis. Five were males. 4 autosites had omphaloceles. Split notochord and 2 parasites attached to a single autosite were encountered. 5/6 autosites survived. On systematic review, the most frequent variant seen was rachipagus (n = 50) followed by omphalopagus (n = 46). Limbs were reported in 75 cases. Congenital heart disease was seen in 17/120(14.2%) autosites. Omphalocele and meningomyelocele were the most common extracardiac anomalies in autosites. Weight along with the anatomy and position of heteropagus twins was a better determinant of the mode of delivery than weight alone. Mortality was reported in 12 cases.
CONCLUSION
Autosites in HT generally carry a good prognosis, however, final outcome depends mainly on associated major cardiac anomalies. Meticulous antenatal assessment and preoperative planning are of paramount importance.
LEVEL OF EVIDENCE
IV.
Topics: Female; Heart Defects, Congenital; Hernia, Umbilical; Humans; Male; Pregnancy; Prenatal Diagnosis; Twins, Conjoined
PubMed: 35567628
DOI: 10.1007/s00383-022-05135-w -
The Journal of Craniofacial Surgery Oct 2023The purpose of this article was to appraise the various methods of reconstruction for meningomyelocele (MMC) defects.
PURPOSE
The purpose of this article was to appraise the various methods of reconstruction for meningomyelocele (MMC) defects.
METHODS
A systematic review of the literature was performed to evaluate all reconstructions for MMC. The method of reconstruction was categorized by: primary closure with and without fascial flaps, random pattern flaps, VY advancement flaps (VY), perforator flaps, and myocutaneous flaps. Perforator flaps were subsequently subcategorized based on the type of flap.
RESULTS
Upon systematic review, 567 articles were screened with 104 articles assessed for eligibility. Twenty-nine articles were further reviewed and included for qualitative synthesis. Two hundred seventy patients underwent MMC repair. The lowest rates of major wound complications (MWC) were associated with myocutaneous and random pattern flaps. A majority of MWC was in the lumbrosacral/sacral region (87.5% of MWC). In this region, random patterns and perforator flaps demonstrated the lowest rate of MWC (4.5, 8.1%).
CONCLUSIONS
Plastic surgery consultation should be strongly considered for MMC with defects in the lumbosacral/sacral region. Perforator flaps are excellent options for the reconstruction of these defects.
PubMed: 37622546
DOI: 10.1097/SCS.0000000000009675 -
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 -
Neurosurgery Sep 2019The incidence of spina bifida (SB) in the developing world is higher than in the United States because of malnutrition and folic acid deficiency during pregnancy....
BACKGROUND
The incidence of spina bifida (SB) in the developing world is higher than in the United States because of malnutrition and folic acid deficiency during pregnancy. Advances in technology have made prenatal repair of myelomeningocele (MM) possible.
OBJECTIVE
The objective of the guidelines are, (1) To create clinical recommendations for best practices, based on a systematic review and analysis of available literature, (2) to obtain multi-disciplinary endorsement of these guidelines from relevant organizations, and (3) to disseminate the educational content to physicians to improve the care of infants with MM.
METHODS
The Guidelines Task Force developed search terms and strategies used to search PubMed and Embase for literature published between 1966 and September 2016. Strict inclusion/exclusion criteria were used to screen abstracts and to develop a list of relevant articles for full-text review.
RESULTS
Guidelines authors aimed to systematically review the literature and make evidence based recommendations about the timing of closure after birth, hydrocephalus, the impact of prenatal closure, and the effect of prenatal closure on ambulation ability and tethered spinal cord. Evidence concerning persistent ventriculomegaly and cognitive impairment was also evaluated. Hundreds of abstracts were identified and reviewed for each of the 5 topics. A total of 14 studies met stringent inclusion criteria.
CONCLUSION
Based on a comprehensive systematic review, a total of 5 clinical practice recommendations were developed, with 1 Level I, 2 Level II and 2 Level III recommendations.The full guideline can be found at https://www.cns.org/guidelines/guidelines-spina-bifida-chapter-1.
Topics: Child; Female; Humans; Infant; Meningomyelocele; Pregnancy; United States
PubMed: 31418038
DOI: 10.1093/neuros/nyz261 -
Neurourology and Urodynamics Jan 2022The purpose of this systematic review was to investigate and synthesize the effects (benefits and harms) of electrical stimulation (EE), alone or in association with...
AIMS
The purpose of this systematic review was to investigate and synthesize the effects (benefits and harms) of electrical stimulation (EE), alone or in association with other interventions, compared with sham and other interventions, for the treatment of neurogenic bladder dysfunction in myelomeningocele.
METHODS
This systematic review was conducted following the methodological recommendations of the Cochrane Handbook for Systematic Reviews of Interventions and registered at PROSPERO (CRD42020200425). A search was performed in the following electronic databases: MEDLINE, Cochrane Central Register of Controlled Trials, EMBASE, LILACS, and PEDro. Randomized clinical trials (RCTs) that assessed any EE in children diagnosed with myelomeningocele and neurogenic bladder and/or urinary incontinence were included and reported.
RESULTS
When comparing EE versus sham groups, some estimated effects showed a wide confidence interval, probably due to the small sample size of the included studies. This indicates an imprecision in these findings. Regarding the safety of this intervention and safety of the lower urinary tract, no adverse events resulting from EE were reported. All the included studies have evaluated the efficacy of EE compared with sham, but different EE parameters and electrode positions among studies make it impossible to perform a meta-analysis.
CONCLUSIONS
Based on very low certainty evidence, the findings of this systematic review suggested no difference between EE and sham to improve urinary incontinence in children with myelomeningocele. However, the small sample size and the imprecision arising from the wide confidence intervals must be considered. Future RCTs following a rigorous methodology, as recommended by the CONSORT statement, should be conducted to support the use of this intervention in clinical practice.
Topics: Child; Electric Stimulation; Humans; Meningomyelocele; Randomized Controlled Trials as Topic; Urinary Bladder, Neurogenic
PubMed: 34524699
DOI: 10.1002/nau.24792 -
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 -
Neurosurgical Focus Oct 2019Comparing prenatal and postnatal surgical repair techniques for myelomeningocele (MMC), in utero fetal surgery has increasingly gained acceptance and is considered by...
OBJECTIVE
Comparing prenatal and postnatal surgical repair techniques for myelomeningocele (MMC), in utero fetal surgery has increasingly gained acceptance and is considered by many specialized centers the first choice of treatment. Despite its benefits, as demonstrated in the Management of Myelomeningocele Study (MOMS), including reduced need for CSF shunting in neonates and improved motor outcomes at 30 months, there is still an ongoing debate on fetal and maternal risks associated with the procedure. Prenatal open hysterotomy, fetoscopic MMC repair techniques, and subsequent delivery by cesarean section are associated with maternal complications. The aim of this systematic review is to assess the available literature on maternal and obstetric complication rates and perinatal maternal outcomes related to fetal MMC repair.
METHODS
The authors identified references for inclusion in this review by searching PubMed and MEDLINE, with restrictions to English language, case series, case reports, clinical trials, controlled clinical trials, meta-analyses, randomized controlled trials, reviews, and systematic reviews. The rate of maternal and obstetric complications was analyzed based on studies focusing on this issue and presenting clear results on the matter.
RESULTS
Of 1264 articles screened, 36 were included in this systemic review, whereof 11 were eligible for data analysis and comparison. The average overall rate of maternal and obstetric complications corresponds to 78.6%. The majority of the described events are obstetric complications, varying from chorioamniotic membrane separation in 65.6% of cases, oligohydramnios in 13.0% of cases, placental abruption in 5.0% of cases, spontaneous or preterm premature membrane rupture in 42.0% of cases, and early preterm delivery in 11.3% of cases due to uterine dehiscence, occurring in 0.9% of cases. The most common medical complications are development of pulmonary edema occurring in 2.8%, gestational diabetes in 3.7%, gestational hypertension/preeclampsia in 3.7%, and need for blood transfusions in 3.2% of cases. Limitations of the review arise from the lack of data in the current literature, with maternal and obstetric complications being underreported.
CONCLUSIONS
Although the efforts of further advancement of intrauterine prenatal MMC repair aim to increase neonatal outcomes, maternal health hazard will continue to be an issue of crucial importance and further studies are required.
Topics: Adult; Cesarean Section; Female; Fetal Membranes, Premature Rupture; Fetoscopy; Humans; Infant, Newborn; Meningomyelocele; Neurosurgical Procedures; Pregnancy; Premature Birth
PubMed: 31574465
DOI: 10.3171/2019.7.FOCUS19470