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Archivos Argentinos de Pediatria Jun 2021A seminal study titled Management of Myelomeningocele Study, from 2011, demonstrated that prenatal myelomeningocele defect repaired before 26 weeks of gestation improved... (Review)
Review
A seminal study titled Management of Myelomeningocele Study, from 2011, demonstrated that prenatal myelomeningocele defect repaired before 26 weeks of gestation improved neurological outcomes; based on this study, fetal surgery was introduced as a standard of care alternative. Thus, prenatal myelomeningocele diagnosis within the therapeutic window became a mandatory goal; therefore, research efforts on screening strategies were intensified, especially in the first trimester. In addition, different fetal surgery techniques were developed to improve neurological outcomes and reduce maternal risks. The objective of this review is to provide an update on the advances in prenatal screening and diagnosis during the first and second trimesters, and in open and fetoscopic fetal surgery for myelomeningocele.
Topics: Female; Fetoscopy; Humans; Meningomyelocele; Pregnancy; Prenatal Care; Prenatal Diagnosis
PubMed: 34033426
DOI: 10.5546/aap.2021.eng.e215 -
Neurology India 2021Hydrocephalus is the most important co-morbidity in myelomeningocele from a neurosurgical perspective. Historically, 75-80% of patients with myelomeningocele have... (Review)
Review
Hydrocephalus is the most important co-morbidity in myelomeningocele from a neurosurgical perspective. Historically, 75-80% of patients with myelomeningocele have required treatment with a shunt but recent advances including intra-uterine myelomeningocele closure and ETV-CPC are reducing this burden. The expression of hydrocephalus differs between patients and across the life span. Hydrocephalus impacts the clinical expression of other important co-morbidities including the Chiari II malformation and tethered spinal cord. Shunt failure is often the key stress to prompt symptomatic worsening of these other conditions. Shunt failure may occur with minimal ventricular change on CT or MRI in Spina Bifida patients. Waiting for radiographic changes in symptomatic SB patients with shunts may result in hydrocephalus related fatalities. It is hypothesized but not proven that shunt failure may contribute to respiratory insufficiency and be a risk factor for sudden death in adult patients with spina bifida. Excellence in hydrocephalus management in MMC is essential for proper care, good outcomes, and quality of life for patients and families.
Topics: Adult; Arnold-Chiari Malformation; Humans; Hydrocephalus; Meningomyelocele; Quality of Life; Spinal Dysraphism
PubMed: 35102990
DOI: 10.4103/0028-3886.332247 -
Neonatal Network : NN Aug 2021Arnold-Chiari malformation (ACM), a defect that involves downward displacement of the hindbrain and herniation of the cerebellar vermis, tonsils, pons, medulla, and...
Arnold-Chiari malformation (ACM), a defect that involves downward displacement of the hindbrain and herniation of the cerebellar vermis, tonsils, pons, medulla, and fourth ventricle through the foramen magnum, is the most complex of the 4 types of Chiari malformations. Unique to the other types of Chiari malformations, approximately 95 percent of infants with ACM also present with an associated myelomeningocele (MMC), the most severe form of spina bifida. Among affected infants, those with symptomatic comorbidities incur a significantly higher morbidity and mortality risk. Prompt identification and diagnosis of ACM, as well as evidence-based postnatal and postsurgical nursing and medical care, is critical. Early surgical intervention can repair an existing MMC and restore proper cerebrospinal fluid circulation, which can dramatically improve patient outcomes and quality of life, and reduce disease and health care burden.
Topics: Arnold-Chiari Malformation; Humans; Hydrocephalus; Infant; Meningomyelocele; Quality of Life
PubMed: 34518383
DOI: 10.1891/11-T-704 -
Child's Nervous System : ChNS :... Nov 2021To investigate certain aspects of hydrocephalus in patients with myelomeningocele.
PURPOSE
To investigate certain aspects of hydrocephalus in patients with myelomeningocele.
METHODS
We retrospectively analyzed data of 1050 patients with myelomeningocele who underwent surgical treatment between June 1991 and June 2021. These patients were divided into three groups: group 1 consisted of patients who underwent surgery after the first 6 h of life, group 2 consisted of patients who underwent surgery within the first 6 h, and group 3 consisted of patients who underwent surgery during the fetal period and before 26 6/7 weeks of gestation.
RESULTS
There were 125, 590, and 335 patients in groups 1, 2, and 3, respectively. In groups 1 and 2, 593 (83%) patients developed hydrocephalus after birth and required ventriculoperitoneal shunt placement in the maternity ward, mainly within the first 4 days of life. In contrast, in group 3, 24 (7.2%) patients required surgery to treat hydrocephalus after birth. Hydrocephalus was the primary cause of mortality in groups 1 and 2, with mortality rates of 35% and 10%, respectively. In group 3, the mortality rate was 0.8% and was not related to hydrocephalus.
CONCLUSION
The onset of hydrocephalus is directly related to myelomeningocele closure in neurosurgery.
Topics: Female; Humans; Hydrocephalus; Meningomyelocele; Neurosurgical Procedures; Pregnancy; Retrospective Studies; Ventriculoperitoneal Shunt
PubMed: 34435215
DOI: 10.1007/s00381-021-05333-2 -
Child's Nervous System : ChNS :... Oct 2023Among fetal surgical procedures, neurosurgery stands out due to the number of cases and the possibility of developing new procedures that can be performed in the fetal... (Review)
Review
Among fetal surgical procedures, neurosurgery stands out due to the number of cases and the possibility of developing new procedures that can be performed in the fetal period. To perform fetal neurosurgical procedures, there is a need for specialized centers that have experts in the diagnosis of fetal pathologies and a highly complex obstetrics service with specialized maternal-fetal teams associated with a pediatric neurosurgery center with expertise in the diverse pathologies of the fetus and the central nervous system that offers multidisciplinary follow-up during postnatal life. Services that do not have these characteristics should refer their patients to these centers to obtain better treatment results. It is essential that the fetal neurosurgical procedure be performed by a pediatric neurosurgeon with extensive experience, as he will be responsible for monitoring these patients in the postnatal period and for several years. The objective of this manuscript is to demonstrate the diagnostic and treatment possibilities, in the fetal period, of some neurosurgical diseases such as hydrocephalus, tumors, occipital encephalocele, and myelomeningocele.
Topics: Male; Pregnancy; Female; Humans; Child; Neurosurgery; Fetus; Neurosurgical Procedures; Hydrocephalus; Meningomyelocele
PubMed: 37606832
DOI: 10.1007/s00381-023-06109-6 -
JAMA Pediatrics Apr 2021The Management of Myelomeningocele Study (MOMS), a randomized clinical trial of prenatal vs standard postnatal repair for myelomeningocele, found that prenatal repair... (Comparative Study)
Comparative Study Randomized Controlled Trial
IMPORTANCE
The Management of Myelomeningocele Study (MOMS), a randomized clinical trial of prenatal vs standard postnatal repair for myelomeningocele, found that prenatal repair reduced hydrocephalus and hindbrain herniation and improved motor function in children aged 12 to 30 months. The Management of Myelomeningocele Study Follow-up (MOMS2) was conducted in children at ages 5 to 10 years. The primary (neurocognitive) outcome has already been reported.
OBJECTIVE
To determine whether MOMS2 participants who had prenatal repair have better physical functioning than those with postnatal repair.
DESIGN, SETTING, AND PARTICIPANTS
Participants from MOMS were recruited for participation in the follow-up study, MOMS2, conducted from April 9, 2012, to April 15, 2017. For this secondary analysis of the randomized clinical trial, trained examiners without knowledge of the treatment group evaluated the physical characteristics, self-care skills, neurologic function, and mobility of the children. Physical functioning outcomes were compared between the prenatal and postnatal repair groups. MOMS2 was conducted at the same 3 clinical sites as MOMS. Home visits were conducted for families who were unable to travel to one of the clinical sites. Of the 161 children with myelomeningocele aged 5 to 10 years old enrolled in MOMS2, 154 had a physical examination and were included in the analyses.
EXPOSURES
Prenatal repair of myelomeningocele.
MAIN OUTCOMES AND MEASURES
Prespecified secondary trial outcomes of self-care skills, functional mobility, walking skills, and motor level.
RESULTS
This analysis included 78 children with postnatal repair (mean [SD] age, 7.4 [2.1] years; 50 girls [64.1%]; 69 White children [88.5%]) and 76 with prenatal repair (mean [SD] age, 7.5 [1.2] years; 43 boys [56.6%]; 70 White children [92.1%]). Children in the prenatal repair group were more competent with self-care skills (mean [SD] percentage of maximum FRESNO Scale score, 90.8% [9.6%] vs 85.5% [17.6%]) and were commonly community ambulators per the Modified Hoffer Classification (51.3% prenatal vs 23.1% postnatal; adjusted relative risk [aRR] for sex, 1.70; 95% CI, 1.23-2.34). Children with prenatal repair also performed the 10-m walk test 1 second faster (difference in medians, 1.0; 95% CI, 0.3-1.7), had better gait quality (adjusted mean difference for home distances of 5 m, 1.71; 95% CI, 1.14-2.54), and could perform higher-level mobility skills (adjusted mean difference for motor total, 5.70; 95% CI, 1.97-11.18). Children in the prenatal repair group were less likely to have a motor function level worse than their anatomic lesion level (aRR, 0.44; 95% CI, 0.25-0.77).
CONCLUSIONS AND RELEVANCE
This secondary analysis of a randomized clinical trial found that the physical functioning benefits of prenatal repair for myelomeningocele reported at age 30 months persisted into school age. These findings indicate the benefit of prenatal repair of myelomeningocele for school-aged children.
TRIAL REGISTRATION
ClinicalTrials.gov Identifier: NCT00060606.
Topics: Child; Child, Preschool; Female; Fetal Therapies; Follow-Up Studies; Health Status Indicators; Humans; Infant, Newborn; Male; Meningomyelocele; Pregnancy; Treatment Outcome
PubMed: 33555337
DOI: 10.1001/jamapediatrics.2020.5674 -
PloS One 2021Myelomeningocele is a severe type of spina bifida, resulting from improper closure of the neural tube. This condition drastically affects the structures of the spinal... (Clinical Trial)
Clinical Trial Randomized Controlled Trial
INTRODUCTION
Myelomeningocele is a severe type of spina bifida, resulting from improper closure of the neural tube. This condition drastically affects the structures of the spinal cord resulting in deficiencies. The combination of these deficiencies results in an overall decrease in mobility and functional participation amongst this population. Physiotherapy plays an essential role in rehabilitating people with MMC. The current literature shows that resources such as photobiomodulation (PBM) may support the rehabilitation of neurological conditions. The aim of the proposed study is to evaluate the effects of photobiomodulation (PBM) combined with physical therapy on functional performance in children with low lumbosacral myelomeningocele.
MATERIALS AND METHODS
This is a protocol randomized clinical blind study, that will include 30 individuals of both sexes, aged between 5 to 8 years, diagnosed with low and sacral lumbar myelomeningocele and capable of performing the sit-to-stand task. The participants will be randomly assigned into two treatment groups: PBM + physiotherapeutic exercises and sham PBM + physiotherapeutic exercises. Irradiation will be carried out with light emitting diode (LED) at a wavelength of 850 nm, energy of 25 J per point, 50 seconds per point and a power of 200 mW. The same device will be used in the placebo group but will not emit light. Muscle activity will be assessed using a portable electromyograph (BTS Engineering) and the sit-to-stand task will be performed as a measure of functioning. Electrodes will be positioned on the lateral gastrocnemius, tibialis anterior and rectus femoris muscles. The Pediatric Evaluation of Disability Inventory will be used to assess functional independence. Quality of life will be assessed using the Child Health Questionnaire-Parent Form 50. Changes in participation will be assessed using the Participation and Environment Measure for Children and Youth. The data will be analyzed with the aid of GraphPad PRISM.
DISCUSSION
The results of this study can contribute to a better understanding of the effectiveness of PBM on functioning and quality of life in children with myelomeningocele.
CLINICAL TRIAL REGISTRATION
ClinicalTrials.gov Identifier: NCT04425330.
Topics: Child; Child, Preschool; Disability Evaluation; Exercise; Exercise Therapy; Female; Humans; Low-Level Light Therapy; Male; Meningomyelocele; Physical Functional Performance; Physical Therapy Modalities; Quadriceps Muscle; Quality of Life
PubMed: 34613973
DOI: 10.1371/journal.pone.0253963 -
The Journal of Urology Jan 2020
Topics: Humans; Meningomyelocele; Urinary Bladder
PubMed: 31644375
DOI: 10.1097/01.JU.0000605504.26263.14 -
Pediatric Clinics of North America Feb 2020Neuromuscular disorders are pathologies that can severely affect the quality of life as well as longevity of patients. The most common disorders include cerebral palsy... (Review)
Review
Neuromuscular disorders are pathologies that can severely affect the quality of life as well as longevity of patients. The most common disorders include cerebral palsy and myelodysplasia. The orthopedic manifestations of these disorders can be treated operatively or nonoperatively. Both focus on the prolongation of mobility and preservation of ambulatory capacity for patients.
Topics: Cerebral Palsy; Child; Humans; Meningomyelocele; Musculoskeletal Diseases; Neuromuscular Diseases; Spinal Dysraphism
PubMed: 31779836
DOI: 10.1016/j.pcl.2019.09.002 -
Seminars in Pediatric Surgery Aug 2019Myelomeningocele is a congenital malformation that causes a spectrum of morbidity. With the standard of care now being in utero repair, the spectrum of morbidity has... (Review)
Review
Myelomeningocele is a congenital malformation that causes a spectrum of morbidity. With the standard of care now being in utero repair, the spectrum of morbidity has changed. The purpose of this article is to review the diagnosis, workup and treatment options of fetal myelomeningocele. We also review the obstetrical, neurological, gastrointestinal, urinary, and orthopedic outcomes of the in utero myelomeningocele repair.
Topics: Arnold-Chiari Malformation; Child Development; Constipation; Female; Fetoscopy; Humans; Hydrocephalus; Hysterotomy; Infant; Infant, Newborn; Magnetic Resonance Imaging; Meningomyelocele; Neural Tube Defects; Pregnancy; Pregnancy Complications; Psychomotor Performance; Scoliosis; Ultrasonography, Prenatal; Urinary Catheterization; Ventriculoperitoneal Shunt
PubMed: 31451171
DOI: 10.1053/j.sempedsurg.2019.07.006