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Journal of Neurosurgery. Spine Oct 2023The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism,...
OBJECTIVE
The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism, to determine the ongoing longitudinal risk for tethered cord release (TCR) among adults with myelomeningocele who had not previously undergone TCR during childhood. The authors also sought to identify the impact of lesion level, ambulation status, and prior treatments for hydrocephalus or Chiari malformations on TCR rates.
METHODS
Adults in the registry who had not previously undergone TCR during childhood were studied. This group was compared with the remaining adults in the registry. The frequency of first-time TCR and time to TCR (using Kaplan-Meier analysis) were determined independently for males and females. Cox proportional hazards analysis identified correlations between sex, best lesion level and ambulation status prior to TCR, and previous treatments for hydrocephalus and Chiari decompression.
RESULTS
Among 967 adults in the NSBPR (422 [43.6%] males and 545 [56.4%] females) who had not undergone TCR during childhood, the authors identified 47 people (4.9%) who underwent their first TCR during adulthood. This study cohort had significantly better mean functional motor levels and ambulation compared with the remaining adult cohort (both p < 0.001). The study group included 35 females (74.5%) and 12 males (25.5%); this sex distribution was significantly different in comparison with the remaining adult cohort (p = 0.016). The Kaplan-Meier curves for first TCR for females and males were significantly different (p = 0.01, log-rank test). TCR rates were correlated with sex (males had decreased risk; OR 0.31, 95% CI 0.16-0.62, p < 0.001), prior treatment for hydrocephalus (those who underwent prior treatment had decreased risk; OR 0.21, 95% CI 0.20-0.42, p < 0.001), and prior treatment for Chiari malformation (those who underwent prior treatment had greater risk; OR 3.84, 95% CI 1.50-9.88, p = 0.005).
CONCLUSIONS
Adults with myelomeningocele who escape childhood without undergoing TCR have an ongoing, albeit decreased, risk for spinal cord tethering requiring TCR. This risk is obviously not due to spinal column growth and therefore must reflect other factors such as dynamic changes in spinal cord health over time. Among people with MMC who underwent their first TCR as adults, females seemed to be overrepresented. Similar to the authors' prior childhood study, people who underwent previous Chiari decompression seemed to be overrepresented, whereas those who underwent previous treatment for hydrocephalus seem to be underrepresented. These novel findings deserve further study.
Topics: Male; Female; Adult; Humans; Meningomyelocele; Spinal Dysraphism; Neural Tube Defects; Arnold-Chiari Malformation; Hydrocephalus; Registries; Receptors, Antigen, T-Cell
PubMed: 37347622
DOI: 10.3171/2023.5.SPINE2328 -
Urology Journal Dec 2023Myelomeningocele is the most severe form of spina bifida. Management of urologic consequences of spina bifida is life long, demanding and costly for both the patient and... (Observational Study)
Observational Study
Early Onset Clean Intermittent Catheterization May Decrease Prevalence and Severity of Urinary Concentration Defects in Myelomeningocele Patients with Neurogenic Bladder: A Retrospective Cohort Study.
PURPOSE
Myelomeningocele is the most severe form of spina bifida. Management of urologic consequences of spina bifida is life long, demanding and costly for both the patient and the public health system. There is a paucity of data in the literature regarding concentration defects and their consequences on this disease. This paper aims to describe retrospectively the effect of early onset clean intermittent catheterization (CIC) in on the severity of urinary concentration defects in myelomeningocele patients with neurogenic bladder in a retrospective observational study.
MATERIALS AND METHODS
In this 10-year retrospective cohort study, children with myelomeningocele were selected with the Convenience sampling method. Demographic characteristics, polyuria index ratio (PIR) defined as 24 hour urine output of each patient divided by the maximum normal urine output of the same patient in a healthy state, and nocturnal polyuria index (NPI) were compared between early starters (< 2 years old) or late starters (≥ 2 years old) groups.
RESULTS
Seven patients who underwent cystoplasty were excluded and 130 patients (63.8% male, 5.4 ± 3.2 years old, 14.3 ± 2.83 Kg, 28.5% early onset CIC) were investigated. PIR > 1 in inset (1.7 ± 0.2 vs. 2.2 ± 0.5, P = 0.021) and outset (1.5 ± 0.32 vs. 2.5 ± 0.7, P = 0.004) were lower in early starters group than in late starters group. NPI in inset (0.2 ± 0.007 vs. 0.32 ± 0.10, P = 0.018) and outset (0.25 ± 0.15 vs. 0.42 ± 0.095, P = 0.007) were also lower in the early starters group. No further adverse events were reported during the follow-up period.
CONCLUSION
Early onset CIC is more effective than late-onset CIC in preserving the urinary ability of kidneys in myelomeningocele patients.
Topics: Child; Humans; Male; Child, Preschool; Female; Meningomyelocele; Intermittent Urethral Catheterization; Urinary Bladder, Neurogenic; Retrospective Studies; Polyuria; Prevalence; Spinal Dysraphism; Urinary Catheterization
PubMed: 37312599
DOI: 10.22037/uj.v20i.7537 -
The Pan African Medical Journal 2023
Topics: Humans; Arnold-Chiari Malformation; Hydrocephalus; Meningomyelocele
PubMed: 37275283
DOI: 10.11604/pamj.2023.44.115.36848 -
Ultrasound in Obstetrics & Gynecology :... Nov 2023Prenatal surgery is offered for selected fetuses with open spina bifida (OSB) to improve long-term outcome. We studied the effect of fetal OSB surgery on brain...
OBJECTIVES
Prenatal surgery is offered for selected fetuses with open spina bifida (OSB) to improve long-term outcome. We studied the effect of fetal OSB surgery on brain development using advanced magnetic resonance imaging (MRI) techniques to quantify the volume, surface area and shape of cerebral structures and to analyze surface curvature by means of parameters that correspond to gyrification.
METHODS
We compared MRI data from 29 fetuses with OSB before fetal surgery (mean gestational age (GA), 23 + 3 weeks) and at 1 and 6 weeks after surgery, with that of 36 GA-matched control fetuses (GA range, 21 + 2 to 36 + 2 weeks). Automated super-resolution reconstruction provided three-dimensional isotropic volumetric brain images. Unmyelinated white matter, cerebellum and ventricles were segmented automatically and refined manually, after which volume, surface area and shape parameter (volume/surface area) were quantified. Mathematical markers (shape index (SI) and curvedness) were used to measure gyrification. Parameters were assessed according to lesion type (myelomeningocele vs myeloschisis (MS)), postoperative persistence of hindbrain herniation (HH) and the presence of supratentorial anomalies, namely partial agenesis of the corpus callosum (pACC) and heterotopia (HT).
RESULTS
Growth in ventricular volume per week and change in shape parameter per week were higher at 6 weeks after surgery in fetuses with OSB compared with controls (median, 2500.94 (interquartile range (IQR), 1689.70-3580.80) mm /week vs 708.21 (IQR, 474.50-925.00) mm /week; P < 0.001 and 0.075 (IQR, 0.047-0.112) mm/week vs 0.022 (IQR, 0.009-0.042) mm/week; P = 0.046, respectively). Ventricular volume growth increased 6 weeks after surgery in cases with pACC (P < 0.001) and those with persistent HH (P = 0.002). During that time period, the change in unmyelinated white-matter shape parameter per week was decreased in OSB fetuses compared with controls (0.056 (IQR, 0.044-0.092) mm/week vs 0.159 (IQR, 0.100-0.247) mm/week; P = 0.002), particularly in cases with persistent HH (P = 0.011), MS (P = 0.015), HT (P = 0.022), HT with corpus callosum anomaly (P = 0.017) and persistent HH with corpus callosum anomaly (P = 0.007). At 6 weeks postoperatively, despite OSB fetuses having a lower rate of change in curvedness compared with controls (0.061 (IQR, 0.040-0.093) mm /week vs 0.094 (IQR, 0.070-0.146) mm /week; P < 0.001), reversing the trend seen at 1 week after surgery (0.144 (IQR, 0.099-0.236) mm /week vs 0.072 (IQR, 0.059-0.081) mm /week; P < 0.001), gyrification, as determined using SI, appeared to be increased in OSB fetuses overall compared with controls. This observation was more prominent in fetuses with pACC and those with severe ventriculomegaly (P-value range, < 0.001 to 0.006).
CONCLUSIONS
Following fetal OSB repair, volume, shape and curvedness of ventricles and unmyelinated white matter differed significantly compared with those of normal fetuses. Morphological brain changes after fetal surgery were not limited to effects on the circulation of cerebrospinal fluid. These observations may have implications for postnatal neurocognitive outcome. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Female; Pregnancy; Humans; Spina Bifida Cystica; Meningomyelocele; Brain; Fetus; Gestational Age; Magnetic Resonance Imaging; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 37161647
DOI: 10.1002/uog.26244 -
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 -
Turkish Neurosurgery 2023To analyze the success rates of repeat endoscopic third ventriculostomy (re-ETV) procedure according to ventriculostomy orifice closure types in patients who have...
AIM
To analyze the success rates of repeat endoscopic third ventriculostomy (re-ETV) procedure according to ventriculostomy orifice closure types in patients who have undergone a second neuroendoscopic surgery for non-communicating hydrocephalus.
MATERIAL AND METHODS
The study included 74 patients who underwent re-ETV procedure due to dysfunctional ventriculostomy orifice. Ventriculostomy closure patterns are classified into three types: Type-1 is defined as the complete closure of the orifice with non-transparent gliosis or scar tissue. Type-2 represents the closure or narrowing of the orifice by newly formed translucent membranes. Type-3 pattern is defined as the blockage of CSF flow due to newly formed reactive membranes in the basal cisterns, with an intact ventriculostomy orifice.
RESULTS
The frequency of the ventriculostomy closure patterns was found as follows. Type-1: 17 cases (22.97%); Type-2: 30 cases (40.54%); and Type-3: 27 cases (36.48%). The success rate of the re-ETV procedure according to closure types was 23.52% in Type-1 cases, 46.66% in Type-2 cases, and 37.03% in Type-3 cases. A significantly higher rate of Type-1 closure pattern was observed in the myelomeningocele associated hydrocephalus cases (p < 0.01).
CONCLUSION
In cases where ETV failure occurs, an endoscopic exploration with reopening of the ventriculostomy orifice is a preferable treatment option. Therefore, identifying patients who may benefit from the re-ETV procedure is essential. Type-1 closure pattern was observed to have a higher frequency in cases where hydrocephalus was associated with myelomeningocele, and the success rate of re-ETV seems to be lower in those cases.
Topics: Humans; Ventriculostomy; Meningomyelocele; Third Ventricle; Retrospective Studies; Hydrocephalus
PubMed: 36951029
DOI: 10.5137/1019-5149.JTN.41786-22.1 -
Turkish Journal of Medical Sciences Feb 2023The study aims to evaluate the neurodevelopmental outcomes of neonates with myelomeningocele (MMC) operated in the postnatal period.
BACKGROUND
The study aims to evaluate the neurodevelopmental outcomes of neonates with myelomeningocele (MMC) operated in the postnatal period.
METHODS
This is a prospective follow-up study in a tertiary neonatal intensive care unit. Neurodevelopmental outcomes of term neonates operated for MMC and healthy term newborns were compared with the Bayley Scales of Infant and Toddler Development -Third Edition (BSID III) at 12-18 months.
RESULTS
A total of 57 cases were included in the study (patient group = 27; control group = 30). Demographic data between the groups were similar. Cognitive, linguistic, and motor composite scores of the patient group were lower than those of the control group (p < 0.001). In the patient group, those who underwent ventriculoperitoneal shunt had lower cognitive, language and motor scores than those without shunt (p < 0.05). The cognitive, linguistic, and motor composite scores in the patient group who underwent surgery before 72 h were better than those who underwent surgery after 72 h.
DISCUSSION
In our study, it was found that the neurodevelopmental prognosis of MMC cases requiring ventriculoperitoneal shunt in the postnatal period was significantly worse than those without shunt. It is the first study in which the neurodevelopment of patients with MMC who were operated in the postnatal period was evaluated with BSID III evaluated and delays in all areas were shown in cases with MMC compared to normal cases. Better neurodevelopmental outcomes in patients operated in the first 72 h suggest that early surgery will improve neurodevelopmental outcomes in patients with MMC.
Topics: Infant; Humans; Infant, Newborn; Meningomyelocele; Follow-Up Studies; Prospective Studies; Ventriculoperitoneal Shunt
PubMed: 36945937
DOI: 10.55730/1300-0144.5561 -
Medical Archives (Sarajevo, Bosnia and... Feb 2023Bleeding Worldwide, approximately 300,000 infants are born annually with neural tube defects (NTDs), which carry a high risk of morbidity and mortality.
BACKGROUND
Bleeding Worldwide, approximately 300,000 infants are born annually with neural tube defects (NTDs), which carry a high risk of morbidity and mortality.
OBJECTIVE
The aim of the study was to describe the experience with NTD patients born at a tertiary academic center.
METHODS
A retrospective record review of all neonates with NTD admitted to the neonatal intensive care unit over six years.
RESULTS
Out of the 39 patients identified, 32 (82.1%) were diagnosed antenatally. Most NTD cases were of the myelomeningocele 26 (66.7%) type. The most common site of the myelomeningocele was lumbar, and the thoracolumbar site had the worst prognosis.
CONCLUSION
Early detection of the disease allows better planning of delivery and treatment decisions. Nevertheless, understanding the magnitude of the problem necessitates adopting public health prevention strategies for better outcomes.
Topics: Female; Humans; Infant, Newborn; Pregnancy; Meningomyelocele; Neural Tube Defects; Patient Discharge; Prenatal Diagnosis; Retrospective Studies; Tertiary Care Centers; Intensive Care Units, Neonatal
PubMed: 36919133
DOI: 10.5455/medarh.2023.77.40-43 -
The Pan African Medical Journal 2022
Topics: Humans; Meningomyelocele
PubMed: 36879634
DOI: 10.11604/pamj.2022.43.171.38082 -
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