-
Transactions of the American... 1973
Topics: Child; Child, Preschool; Esotropia; Exotropia; Eye Diseases; Humans; Hydrocephalus; Infant; Meningomyelocele; Strabismus
PubMed: 10949606
DOI: No ID Found -
Ultrasound in Obstetrics & Gynecology :... Feb 2023
Topics: Pregnancy; Female; Humans; Meningomyelocele; Fetus; Fetoscopy; Prenatal Care
PubMed: 36178849
DOI: 10.1002/uog.26081 -
Pediatric Neurosurgery 2023Myelomeningocele (MMC) is a malformation resulting from the neural tube's failure to close during embryonic development, and the majority of the cases of neural tube...
INTRODUCTION
Myelomeningocele (MMC) is a malformation resulting from the neural tube's failure to close during embryonic development, and the majority of the cases of neural tube defects (NTDs) were prevalent as single location lesions along the spine; however, multiple NTDs (MNTDs) are a very rare condition. Only a few cases of MNTDs were found in the literature.
CASE PRESENTATION
We report the case of a 2-month-old male infant prenatally diagnosed with MMC, presented with two unconnected lumbar and lumbosacral epidermal, soft, dome-shaped swellings located on both sides of the midline (paravertebral) covered by intact skin. MRI revealed double MMC at the level of L4-L5, with spinal nerve roots. The patient underwent surgical repair of the defects by replacing the spinal cord and its nerve roots inside the thecal sac and recreating a covering layer around the neural structures to resemble thecal sac. The outcome was favorable, and postoperative head CT scan did not show any complication.
CONCLUSION
Our case report is considered the first from Algeria to report the condition and the first to report the occurrence of double lesions in the same spine region. MMC can be associated with neurological deficits or other congenital anomalies, thus it is necessary to thoroughly examine such patients. However, there was no antenatal folic acid deficiency in our case. We recommend antenatal care with adequate folic acid supplementation given that its deficiency during pregnancy is considered a ubiquitous risk factor for the condition. The optimal timing for surgery of MMC cases is 8 ± 5 days. Prenatal intrauterine repair of the condition provides favorable outcomes but carries high fetal and maternal risks. Surgical repair should include the sac removal, the reconstruction of the placode, and the closure of the overlying meninges. With early diagnosis and proper repair of such cases, MMC has good prognosis and favorable outcomes.
Topics: Infant; Pregnancy; Female; Humans; Male; Meningomyelocele; Neural Tube Defects; Spine; Spinal Cord; Fetus
PubMed: 37075706
DOI: 10.1159/000530726 -
World Journal of Gastroenterology Dec 2011Exciting new features have been described concerning neurogenic bowel dysfunction, including interactions between the central nervous system, the enteric nervous system,... (Review)
Review
Exciting new features have been described concerning neurogenic bowel dysfunction, including interactions between the central nervous system, the enteric nervous system, axonal injury, neuronal loss, neurotransmission of noxious and non-noxious stimuli, and the fields of gastroenterology and neurology. Patients with spinal cord injury, myelomeningocele, multiple sclerosis and Parkinson's disease present with serious upper and lower bowel dysfunctions characterized by constipation, incontinence, gastrointestinal motor dysfunction and altered visceral sensitivity. Spinal cord injury is associated with severe autonomic dysfunction, and bowel dysfunction is a major physical and psychological burden for these patients. An adult myelomeningocele patient commonly has multiple problems reflecting the multisystemic nature of the disease. Multiple sclerosis is a neurodegenerative disorder in which axonal injury, neuronal loss, and atrophy of the central nervous system can lead to permanent neurological damage and clinical disability. Parkinson's disease is a multisystem disorder involving dopaminergic, noradrenergic, serotoninergic and cholinergic systems, characterized by motor and non-motor symptoms. Parkinson's disease affects several neuronal structures outside the substantia nigra, among which is the enteric nervous system. Recent reports have shown that the lesions in the enteric nervous system occur in very early stages of the disease, even before the involvement of the central nervous system. This has led to the postulation that the enteric nervous system could be critical in the pathophysiology of Parkinson's disease, as it could represent the point of entry for a putative environmental factor to initiate the pathological process. This review covers the data related to the etiology, epidemiology, clinical expression, pathophysiology, genetic aspects, gastrointestinal motor dysfunction, visceral sensitivity, management, prevention and prognosis of neurogenic bowel dysfunction patients with these neurological diseases. Embryological, morphological and experimental studies on animal models and humans are also taken into account.
Topics: Animals; Disease Models, Animal; Enteric Nervous System; Humans; MEDLINE; Meningomyelocele; Multiple Sclerosis; Neurogenic Bowel; Parkinson Disease; Reflex; Spinal Cord Injuries
PubMed: 22171138
DOI: 10.3748/wjg.v17.i46.5035 -
Journal of Pediatric Orthopedics. Part B Jan 2022Myelomeningocele, characterized by extrusion of the spinal cord through a spinal canal defect, is the most common form of spina bifida, often resulting in lifelong... (Randomized Controlled Trial)
Randomized Controlled Trial
Myelomeningocele, characterized by extrusion of the spinal cord through a spinal canal defect, is the most common form of spina bifida, often resulting in lifelong disability and significant orthopaedic issues. A randomized controlled trial (RCT) has shown the efficacy of prenatal repair in decreasing the need for shunting and improving motor outcomes. However, no studies have evaluated the effects of prenatal repair on orthopaedic outcomes. The purpose of this study was to determine the rates of orthopaedic conditions in patients with prenatal and postnatal repair of myelomeningocele and compare the rates of treatment required. This study analyzes the relevant outcomes from a prospective RCT (Management of Myelomeningocele Study). Eligible women were randomized to prenatal or postnatal repair, and patients were evaluated prospectively. Outcomes of interest included rates of scoliosis, kyphosis, hip abnormality, clubfoot, tibial torsion, and leg length discrepancy (LLD) at 12 and 30 months. The need for orthopaedic intervention at the same time points was also evaluated. Statistical analyses included descriptive statistics and univariate analyses. Data for the full cohort of 183 patients were analyzed (91 prenatal, 92 postnatal). There were no differences in rates of scoliosis, kyphosis, hip abnormality, clubfoot or tibial torsion between patients treated with prenatal or postnatal repair. The rate of LLD was lower in the prenatal repair group at 12 and 30 months (7 vs. 16% at 30 months, P = 0.047). The rates of patients requiring casting or bracing were significantly lower in patients treated with prenatal repair at 12 and 30 months (78 vs. 90% at 30 months, P = 0.036). Patients treated with prenatal myelomeningocele repair may develop milder forms of orthopaedic conditions and may not require extensive orthopaedic management.
Topics: Female; Humans; Meningomyelocele; Orthopedics; Pregnancy
PubMed: 33165214
DOI: 10.1097/BPB.0000000000000827 -
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 -
The Journal of Spinal Cord Medicine Sep 2015Children with myelomeningocele (MMC) are usually subjected to multiple surgeries. However, the number and type of surgeries are not the same in every patient with MMC... (Review)
Review
INTRODUCTION
Children with myelomeningocele (MMC) are usually subjected to multiple surgeries. However, the number and type of surgeries are not the same in every patient with MMC over time. This report summarizes the surgical interventions in a cohort of several ages.
MATERIALS AND METHODS
Data on all of the patients with MMC, aged from 1 year and 10 months to 21 years and 11 months, were retrospectively reviewed at the Dona Estefânia Hospital in Lisbon, Portugal. Data were collected by chart review and individual interviews. The factors analyzed were demographics, ambulatory status, neurological level of involvement, shunt status, Arnold-Chiari malformation type II, surgical history, and occurrence of fracture. The surgical interventions were categorized as neurosurgical, orthopedic, urinary, ulcer repair and others.
RESULTS
A total of 84 alive were eligible and enrolled. The average age was 14 years and six months. A total of 59 patients received shunts (all but one ventriculoperitoneal). In the study group, the 84 patients required 663 surgeries. Neurosurgical interventions were the most frequent surgical procedure and predominated during the first 2 years of life. Surgical interventions related to shunts were the most common neurosurgical interventions. Orthopedic surgeries were more frequent in the age group 6-12 years. Urological surgeries were done mainly after 6 years of age. Surgical repair of pressure ulcers was more common after 12 years of age.
CONCLUSIONS
Our study brings to light the complexity of this condition, with multiple surgeries among patients with MMC.
Topics: Adolescent; Adult; Arnold-Chiari Malformation; Child; Child, Preschool; Female; Humans; Infant; Male; Meningomyelocele; Neurosurgical Procedures; Orthopedic Procedures; Pressure Ulcer; Retrospective Studies; Urologic Surgical Procedures; Ventriculoperitoneal Shunt; Young Adult
PubMed: 25029586
DOI: 10.1179/2045772314Y.0000000229 -
Trials Aug 2020Open spina bifida (OSB) is one of the most common congenital central nervous system defects and leads to long-term physical and cognitive disabilities. Open fetal...
BACKGROUND
Open spina bifida (OSB) is one of the most common congenital central nervous system defects and leads to long-term physical and cognitive disabilities. Open fetal surgery for OSB improves neurological outcomes and reduces the need for ventriculoperitoneal shunting, compared to postnatal surgery, but is associated with a significant risk of prematurity and maternal morbidity. Fetoscopic surgery comes with less maternal morbidity, yet the question remains whether the procedure is neuroprotective and reduces prematurity. Comparison of outcomes between different treatment options is challenging due to inconsistent outcome reporting. We aim to develop and disseminate a core outcome set (COS) for fetal OSB, to ensure that outcomes relevant to all stakeholders are collected and reported in a standardised fashion in future studies.
METHODS
The COS will be developed using a validated Delphi methodology. A systematic literature review will be performed to identify outcomes previously reported for prenatally diagnosed OSB. We will assess maternal (primary and subsequent pregnancies), fetal, neonatal and childhood outcomes until adolescence. In a second phase, we will conduct semi-structured interviews with stakeholders, to ensure representation of additional relevant outcomes that may not have been reported in the literature. We will include patients and parents, as well as health professionals involved in the care of these pregnancies and children (fetal medicine specialists, fetal surgeons, neonatologists/paediatricians and allied health). Subsequently, an international group of key stakeholders will rate the importance of the identified outcomes using three sequential online rounds of a modified Delphi Survey. Final agreement on outcomes to be included in the COS, their definition and measurement will be achieved through a face-to-face consensus meeting with all stakeholder groups. Dissemination of the final COS will be ensured through different media and relevant societies.
DISCUSSION
Development and implementation of a COS for fetal OSB will ensure consistent outcome reporting in future clinical trials, systematic reviews and clinical practice guidelines. This will lead to higher quality research, better evidence-based clinical practice and ultimately improved maternal, fetal and long-term childhood outcomes.
TRIAL REGISTRATION
International Prospective Register of Systematic Reviews (PROSPERO) CRD42018104880 . Registered on December 5, 2018. Core Outcome Measures in Effectiveness Trials (COMET): 1187.
Topics: Adolescent; Child; Consensus; Delphi Technique; Female; Humans; Infant, Newborn; Meningomyelocele; Outcome Assessment, Health Care; Pregnancy; Research Design; Spina Bifida Cystica; Systematic Reviews as Topic; Treatment Outcome
PubMed: 32825852
DOI: 10.1186/s13063-020-04668-6 -
The Western Journal of Medicine Oct 1974
Topics: Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Meningomyelocele; Pregnancy; Spinal Dysraphism
PubMed: 4608838
DOI: No ID Found -
World Neurosurgery Jan 2021Spina bifida disproportionally affects low-and-middle-income countries. We describe myelomeningocele surgical outcomes in Zambia and predictors of postoperative...
OBJECTIVE
Spina bifida disproportionally affects low-and-middle-income countries. We describe myelomeningocele surgical outcomes in Zambia and predictors of postoperative complications and mortality.
METHODS
This 2-center retrospective cohort study includes children who underwent surgical treatment for myelomeningocele in Lusaka, Zambia from 2017 to 2019. Primary outcomes included mortality and 30-day postoperative complications.
RESULTS
Seventy-five patients were identified. Median age at first neurosurgical evaluation was 9 days (interquartile range [IQR], 6-21) and at surgery was 21 days (IQR 15-36). Lumbosacral myelomeningocele was most common (73%, n = 54). At first preoperative evaluation, 28% of the neural tube defects were deemed infected (n = 21), and 30% were leaking cerebrospinal fluid (n = 21). Postoperatively, 7% of patients died (n = 5), whereas 31% experienced a complication (n = 23). Most common complications included wound dehiscence (n = 10, 42%) and wound purulence (n = 6, 25%). Median follow-up duration was 41 days (IQR, 6-128). On univariable analysis, mortality was significantly associated with shorter follow-up duration (5 days [IQR, 2-7] vs. 46 days [IQR, 12-132]; P = 0.02) and any complication (P < 0.001). No variable was significantly associated with postoperative complication; however, 2 variables that notably neared significance were preoperative infection of the lesion (P = 0.05) and longer surgical delay (P = 0.06).
CONCLUSIONS
Most patients born with myelomeningocele in Zambia present for first neurosurgical evaluation after 1 week of age. Preoperative infection of the lesion and postoperative complications are relatively common, and complications are a significant predictor of postoperative mortality. Further investigation into preoperative efforts to mitigate risk of postoperative complications and mortality is warranted.
Topics: Female; Humans; Infant; Infant, Newborn; Male; Meningomyelocele; Neurosurgical Procedures; Postoperative Complications; Retrospective Studies; Treatment Outcome; Zambia
PubMed: 33091647
DOI: 10.1016/j.wneu.2020.10.069