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1.
Exp Cell Res ; 439(1): 114048, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38697275

RESUMO

Prenatal surgery for the treatment of spina bifida (myelomeningocele, MMC) significantly enhances the neurological prognosis of the patient. To ensure better protection of the spinal cord by large defects, the application of skin grafts produced with cells gained from the amniotic fluid is presently studied. In order to determine the most appropriate cells for this purpose, we tried to shed light on the extremely complex amniotic fluid cellular composition in healthy and MMC pregnancies. We exploited the potential of micro-Raman spectroscopy to analyse and characterize human amniotic fluid cells in total and putative (cKit/CD117-positive) stem cells of fetuses with MMC in comparison with amniotic fluid cells from healthy individuals, human fetal dermal fibroblasts and adult adipose derived stem cells. We found that (i) the differences between healthy and MMC amniocytes can be attributed to specific spectral regions involving collagen, lipids, sugars, tryptophan, aspartate, glutamate, and carotenoids, (ii) MMC amniotic fluid contains two particular cell populations which are absent or reduced in normal pregnancies, (iii) the cKit-negative healthy amniocyte subpopulation shares molecular features with human fetal fibroblasts. On the one hand we demonstrate a different amniotic fluid cellular composition in healthy and MMC pregnancies, on the other our work confirms micro-Raman spectroscopy to be a valuable tool for discriminating cell populations in unknown mixtures of cells.

2.
Childs Nerv Syst ; 2024 May 07.
Artigo em Inglês | MEDLINE | ID: mdl-38714605

RESUMO

INTRODUCTION: Most myelomeningocele (MMC) cases present with ventriculomegaly or hydrocephalus, yet a comprehensive volumetric assessment of MMC intracranial structures is lacking. This study aimed to provide baseline data on intracranial structural volumes immediately after birth in MMC infants who underwent repair surgeries after birth (postnatal repair). METHODS: In this retrospective single-center study, we analyzed 52 MMC infants undergoing postnatal repair, utilizing head computed tomography scans at birth for volumetric assessment. Intracranial volume (ICV), lateral ventricles volume (LVV), choroid plexus volume (CPV), and posterior cranial fossa volume (PCFV) were measured. Hydrocephalus was classified into no hydrocephalus, progressive hydrocephalus, and hydrocephalus at birth. Comparative analysis employed the Wilcoxon rank-sum test. Receiver operating characteristic (ROC) analysis discriminated cases with and without ventriculoperitoneal shunt (VPS). RESULTS: The median values were 407.50 mL for ICV, 33.18 mL for LVV, 0.67 mL for CPV, and 21.35 mL for PCFV. Thirty-seven cases (71.15%) underwent VPS. ROC analysis revealed an LVV cut-off value of 6.74 mL for discriminating cases with and without VPS. Progressive hydrocephalus showed no significant difference in ICV but significantly larger LVV compared to no hydrocephalus. Hydrocephalus at birth demonstrated statistically larger ICV and LVV compared to the other two types. CONCLUSION: Baseline volumetric data were provided, and volumetric analysis exhibited statistical differences among three hydrocephalus types. These findings enhance our understanding of intracranial volumetric changes in MMC, facilitating more objective assessments of MMC cases.

3.
Childs Nerv Syst ; 2024 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-38578480

RESUMO

PURPOSE: Patients and caregivers impacted by myelomeningocele (MMC) use online discussion board forums to create community and share information and concerns about this complex medical condition. We aim to identify the primary concerns expressed on these forums with the goal of understanding gaps in care that may merit investment of resources to improve care received by this population. METHODS: Anonymous posts from online MMC discussion boards were compiled using internet search engines. Posts were then analyzed using an adaptation of the Grounded Theory Method, a three-step system involving open, axial, and selective coding of the data by two independent researchers to identify common themes. RESULTS: Analysis of 400 posts written primarily by parents (n = 342, 85.5%) and patients (n = 45, 11.25%) yielded three overarching themes: questions surrounding quality of life, a lack of support for mothers of children with MMC, and confusion with a complex healthcare system. Many posts revealed concerns about management and well-being with MMC, including posts discussing symptoms and related conditions (n = 299, 75.75%), treatments (n = 259, 65.75%), and emotional aspects of MMC (n = 146, 36.5%). Additionally, families, especially mothers, felt a lack of support in their roles as caregivers. Finally, in 118 posts (29.5%), patients and families expressed frustration with navigating a complex healthcare system and finding specialists whose opinions they trusted. CONCLUSIONS: MMC is a complex medical condition that impacts patients and families in unique ways. Analysis of online discussion board posts identified key themes to be addressed in order to improve the healthcare experiences of those impacted by MMC.

4.
J Clin Sleep Med ; 2024 Apr 25.
Artigo em Inglês | MEDLINE | ID: mdl-38661675

RESUMO

STUDY OBJECTIVES: Advances in prenatal repair of myelomeningocele (MMC) have improved outcomes involving different organ systems. There is limited data on respiratory outcomes following prenatal surgical repair. We hypothesize there is no difference in respiratory outcomes between spina bifida (SB) patients who have undergone prenatal versus postnatal repair. METHODS: We performed a retrospective study of 46 infants <1 year with SB seen at Children's Hospital Los Angeles from 2004-2022. Demographic data, timing of closure, neonatal course, Chiari II malformation (CIIM), ventriculoperitoneal shunt (VPS), polysomnography (PSG) results, and need for supplemental oxygen were collected. Unpaired t-test and Chi-square Test were used to analyze results. RESULTS: 31/46 had prenatal repair of MMC; average age at repair was 27 weeks post-conception (PCA). Average age at postnatal repair was 37 PCA. There was no difference in age at PSG. There was no difference in CIIM presence (p=0.61). 60% of patients with postnatal repair and 23% in the prenatal group underwent VPS placement (p=0.01).There was no difference in PSG findings between the two groups: CAI (p=0.11), OAHI (p=0.64), average SpO2 baseline (p=0.91), average SpO2 nadir (p=0.17), average PETCO2 baseline (p=0.87), and average PETCO2 maximum (p=0.54). There were no significant differences in the proportion of patients on supplemental O2 (p=0.25), CSA or OSA between groups. CONCLUSIONS: Patients with SB who've undergone closure of neural tube defect have persistent central apneas, obstructive apneas, and significant hypoxemia. There were no differences in the frequency or severity of sleep-disordered breathing in those with prenatal repair versus postnatal repair.

5.
J Med Ultrasound ; 32(1): 21-24, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38665341

RESUMO

Prenatal diagnosis of myelomeningocele remains challenging for obstetricians, ultrasonographers, and radiologists, although the increased maternal serum alpha-fetoprotein level aids in the confirmative diagnosis. Fetal cervical myelomeningocele and meningocele are very rare and unique types of myelomeningocele. Prenatal diagnosis of cervical myelomeningocele and meningocele should include the differential diagnosis and association of many intracranial and spino-skeletal pathogenetic variants and genetic diseases, including subependymal nodular heterotopia and Klippel-Feil syndrome. In this report, a comprehensive review of fetal cervical myelomeningocele with its prenatal diagnosis and long-term outcomes is presented.

6.
Childs Nerv Syst ; 2024 Apr 22.
Artigo em Inglês | MEDLINE | ID: mdl-38644383

RESUMO

INTRODUCTION: A cross-sectional study retrospectively evaluating the perceived usefulness of attending a multi-disciplinary, roundtable, educational prenatal clinic for mothers expecting children with myelomeningocele is presented. METHODS: Mothers who currently have children with SB completed a survey which evaluated their overall preparedness, spina bifida education, delivery plans, surgical expectations, and expectations in terms of quality of life and development. Open comments were also collected. Statistical analysis was performed to identify differences between those who attended prenatal counseling and those who did not. RESULTS: Approximately half of these mothers received some form of prenatal SB counseling. Mothers who attended prenatal counseling reported that they felt more informed and prepared throughout their pregnancy, during the delivery of their child and during their initial hospital stay than mothers who did not. They reported that the roundtable discussions were beneficial, and the education they received was useful in helping them form accurate expectations and feel more at ease. CONCLUSION: This suggests that prenatal counseling and the High-Risk Pregnancy Clinic (HRPC) provides perceived utility to families and mothers and that the HRPC is an effective method of providing prenatal counseling to mothers whose unborn children have been diagnosed with myelomeningocele.

7.
Childs Nerv Syst ; 2024 Apr 27.
Artigo em Inglês | MEDLINE | ID: mdl-38676719

RESUMO

PURPOSE: Historically, the presence of gray matter heterotopia was a concern for adverse postnatal neurocognitive status in patients undergoing fetal closure of open spinal dysraphism. The purpose of this study was to evaluate neurodevelopmental outcomes and the onset of seizures during early childhood in patients with a prenatal diagnosis of myelomeningocele/myeloschisis (MMC) and periventricular nodular heterotopia (PVNH). METHODS: All patients evaluated at the Center for Fetal Diagnosis and Treatment with a diagnosis of MMC between June 2016 to March 2023 were identified. PVNH was determined from prenatal and/or postnatal MRI. The Bayley Scales of Infant and Toddler Development (edition III or IV) were used for neurodevelopmental assessments. Patients were screened for seizures/epilepsy. RESULTS: Of 497 patients evaluated with a prenatal diagnosis of MMC, 99 were found to have PVNH on prenatal MRI, of which 35 had confirmed PVNH on postnatal imaging. From the 497 patients, 398 initially did not exhibit heterotopia on prenatal MRI, but 47 of these then had confirmed postnatal PVNH. The presence of PVNH was not a significant risk factor for postnatal seizures in early childhood. The average neurodevelopmental scores were not significantly different among heterotopia groups for cognitive, language, and motor domains. CONCLUSION: The presence of PVNH in patients with a prenatal diagnosis of MMC does not indicate an increased risk for neurodevelopmental delay at 1 year of age. We did not demonstrate an association with seizures/epilepsy. These findings can aid clinicians in prenatal consultation regarding fetal repair of open spinal dysraphism. Long-term follow-up is required to discern the true association between PVNH seen on prenatal imaging and postnatal seizures/epilepsy and neurodevelopmental outcomes.

8.
World Neurosurg ; 2024 Apr 27.
Artigo em Inglês | MEDLINE | ID: mdl-38685347

RESUMO

OBJECTIVE: Reports on the management and survival of children with myelomeningocele defects in Bangladesh are limited. This study describes the characteristics and outcomes of these children, focusing on the timing of surgical repair and factors affecting survival. METHODS: We enrolled patients with myelomeningoceles in a case-control study on arsenic exposure and spina bifida in Bangladesh. Cases were subsequently followed at regular intervals to assess survival. Demographic, clinical, and surgical characteristics were reviewed. Univariate tests identified factors affecting survival. RESULTS: Between 2016-2022, we enrolled 272 patients with myelomeningocele. Postnatal surgical repair was performed in 63% of cases. However, surgery within 5 days after birth was infrequent (<10%) due to delayed presentation, and there was a high rate (29%) of preoperative deaths. Surgical repair significantly improved patient survival (p<0.0001). Older age at time of surgery was also associated with improved survival rates, which most likely represents that those who survived to older ages prior to surgery accommodated better with their lesions. Patients who presented with ruptured lesions had lower survival rates. CONCLUSIONS: Timely neurosurgical repair of myelomeningoceles in Bangladesh is hindered by late patient presentation, resulting in a high preoperative patient death rate. Neurosurgical intervention remains a significant predictor of survival. Increased access to neurosurgical care and education of families and non-neurosurgical providers on the need for timely surgical intervention are important for improving the survival of infants with myelomeningoceles.

9.
Int J Sports Physiol Perform ; 19(5): 510-514, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38663852

RESUMO

OBJECTIVE: To investigate whether transcranial direct-current stimulation (tDCS) optimizes the performance of a wheelchair basketball player on precision tasks. METHODS: A right-handed wheelchair basketball player (1.5 points functional class) with myelomeningocele (low lumbar level) participated in this case study. The tDCS neuromodulation protocol was applied throughout 10 interventions of 20 minutes with a current intensity of 2 mA, simultaneously with sport-specific training, 3 times a week for 4 weeks. Anodic stimulation was performed on the right cerebellar hemisphere (CB2) and cathodic stimulation in the left dorsolateral prefrontal cortex. A control participant was submitted to a sham-tDCS stimulation protocol for the same period. Functional performance was assessed before the intervention and after the 5th and 10th interventions using "pass accuracy," "free-throw shooting," and "spot shot" tests. Outcome measures were compared using percentage differences between preintervention, intermediate intervention, and postintervention values. RESULTS: There was a gradual increase in the athlete's total and average scores in all tests performed, with an overall improvement of 78% between the baseline and final assessments, while the control participant had an overall improvement of 6.5%. CONCLUSION: The tDCS protocol was effective in improving performance in precision activities in a wheelchair basketball player.


Assuntos
Desempenho Atlético , Basquetebol , Paratletas , Estimulação Transcraniana por Corrente Contínua , Cadeiras de Rodas , Adulto , Humanos , Masculino , Desempenho Atlético/fisiologia , Basquetebol/fisiologia , Destreza Motora/fisiologia , Esportes para Pessoas com Deficiência/fisiologia , Pessoa de Meia-Idade
10.
J Matern Fetal Neonatal Med ; 37(1): 2334846, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38584146

RESUMO

INTRODUCTION: Neural tube defects (NTDs) represent a spectrum of heterogeneous birth anomalies characterized by the incomplete closure of the neural tube. In Jordan, NTDs are estimated to occur in approximately one out of every 1000 live births. Timely identification of NTDs during the 18-22 weeks of gestation period offers parents various management options, including intrauterine NTD repair and termination of pregnancy (TOP). This study aims to assess and compare parental knowledge and perceptions of these management modalities between parents of affected children and those with healthy offspring. MATERIALS AND METHODS: This retrospective case-control study was conducted at Jordan University Hospital (JUH) using telephone-administered questionnaires. Categorical variables were summarized using counts and percentages, while continuous variables were analyzed using mean and standard deviation. The association between exposure variables and outcomes was explored using binary logistic regression. Data analysis was performed using SPSS for Windows version 26 (SPSS Inc., Chicago, IL). RESULTS: The study sample comprised 143 participants, with 49.7% being parents of children with NTDs. The majority of NTD cases were associated with unplanned pregnancies, lack of folic acid supplementation, and postnatal diagnosis. Concerning parental knowledge of TOP in Jordan, 86% believed it to be legally permissible in certain situations. However, there was no statistically significant difference between cases and controls regarding attitudes toward TOP. While the majority of parents with NTD-affected children (88.7%) expressed a willingness to consider intrauterine surgery, this percentage decreased significantly (to 77.6%) after receiving detailed information about the procedure's risks and benefits (p = .013). CONCLUSIONS: This study represents the first case-control investigational study in Jordan focusing on parental perspectives regarding TOP versus intrauterine repair of myelomeningocele following a diagnosis of an NTD-affected fetus. Based on our findings, we urge the implementation of a national and international surveillance program for NTDs, assessing the disease burden, facilitating resource allocation toward prevention strategies, and promoting early diagnosis initiatives either by using newly suggested diagnostic biomarkers or early Antenatal ultrasonography.


Assuntos
Ácido Fólico , Defeitos do Tubo Neural , Criança , Gravidez , Feminino , Humanos , Jordânia/epidemiologia , Estudos de Casos e Controles , Estudos Retrospectivos , Defeitos do Tubo Neural/diagnóstico , Defeitos do Tubo Neural/epidemiologia , Defeitos do Tubo Neural/terapia , Pais
11.
Urol Clin North Am ; 51(2): 187-196, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38609191

RESUMO

The health care needs children with spina bifida evolve over their lifetime; continued, regular contact with appropraitely trained, multidisciplinary providers is crucial to a patient's health and quality of life. Substantial research has been conducted to improve the transition process starting at an early age; however, there continue to be strong barriers to successful transition. This article reviews key aspects of the care of patients with spina bifida, the impact of inadequate transition to adult care, barriers to transition, and offers a potential vision for the future.


Assuntos
Disrafismo Espinal , Cuidado Transicional , Adulto , Criança , Humanos , Qualidade de Vida , Disrafismo Espinal/terapia
12.
Braz J Anesthesiol ; 74(3): 844495, 2024 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-38521500

RESUMO

BACKGROUND: Myelomeningocele (MMC) is a neural tube defect disease. Antenatal repair of fetal MMC is an alternative to postnatal repair. Many agents can be used as tocolytics during the in utero fetal repair such as ß2-agonists and oxytocin receptor antagonists, with possible maternal and fetal repercussions. This study aims to compare maternal arterial blood gas analysis between terbutaline or atosiban, as tocolytic agents, during intrauterine MMC repair. METHODS: Retrospective cohort study. Patients were divided into two groups depending on the main tocolytic agent used during intrauterine MMC repair: atosiban (16) or terbutaline (9). Maternal arterial blood gas samples were analyzed on three moments: post induction (baseline, before the start of tocolysis), before extubation, and two hours after the end of the surgery. RESULTS: Twenty-five patients were included and assessed. Before extubation, the terbutaline group showed lower arterial pH (7.347 ± 0.05 vs. 7.396 ± 0.02 for atosiban, p = 0.006) and higher arterial lactate (28.33 ± 12.76 mg.dL-1 vs. 13.06 ± 6.35 mg.dL-1, for atosiban, p = 0.001) levels. CONCLUSIONS: Patients who received terbutaline had more acidosis and higher levels of lactate, compared to those who received atosiban, during intrauterine fetal MMC repair.

13.
Clin Neurol Neurosurg ; 239: 108214, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38503112

RESUMO

BACKGROUND AND PURPOSE: Spina bifida is the second major cause of congenital disorders and the most common central nervous system congenital malformation compatible with life primarily. Herein, we describe the short-term outcome of post-natal Myelomeningocele (MMC) surgical management and predictors of its postoperative complications and mortality. METHODS: This retrospective chart review studies the children who underwent post-natal surgical management for MMC in Namazi hospital, a tertiary referral center, in southern Iran from May 2001 to September 2020. RESULTS: 248 patients were included in this study. The mean age at the operation was 8.47 ± 8.69 days. The most common site of involvement of MMC was Lumbosacral (86%, n = 204). At the evaluation conducted prior to the operation, cerebrospinal fluid leak was observed in 7% (n=16) of the patients. Postoperatively, 5.7% of the patients expired in the 30-day follow-up after the operation (n = 14), while 24% needed readmission (n = 47). The most common complications leading to readmission were wound dehiscence (n = 10, 42%) and wound purulence (n = 6, 25%). Only the site of the lesion (p-value = 0.035) was associated with postoperative complication. After controlling for potential confounders, the site of the lesion (adjusted odds ratio = 0.146, 95% confidence interval = 0.035-0.610, p-value = 0.008) and age at surgery (adjusted odds ratio = 1.048, 95% confidence interval = 1.002-1.096, p-value = 0.041) were significantly associated with mortality CONCLUSIONS: The age of the patients at the surgery and the site of the lesion are the two factors that were associated with mortality. However, further investigations into preoperative interventions and risk factors to mitigate the risk of complications and mortality are highly encouraged.


Assuntos
Hidrocefalia , Meningomielocele , Criança , Humanos , Recém-Nascido , Meningomielocele/cirurgia , Meningomielocele/complicações , Estudos Retrospectivos , Hidrocefalia/cirurgia , Complicações Pós-Operatórias/epidemiologia , Resultado do Tratamento
14.
J Reprod Immunol ; 163: 104224, 2024 Mar 08.
Artigo em Inglês | MEDLINE | ID: mdl-38479055

RESUMO

INTRODUCTION: Myelomeningocele (MMC) results from incomplete closure of the neural tube, and has a complex multifactorial etiology, including an inflammatory microenvironment. OBJECTIVE: We evaluated the contribution of humoral immune response for development of inflammatory milieu. METHODS: Using public repository Gene Expression Omnibus (GEO), we retrieve dataset transcriptome from the amniotic fluid of ten fetuses with myelomeningocele and ten healthy control fetuses to found differential gene expression associated with disturbances and inflammatory signatures in MMC. The identified DEGs were submitted to enrichment, network, and matrix correlation analyses. RESULTS: Our initial analysis revealed 90 DEGs in MMC, mainly associated with signaling pathways of inflammation, including the immune modules, humoral immune response and IFN-type I signatures. Protein-protein analysis (PPI) revealed an association with three protein networks; positive regulation of B cell proliferation constituted the largest network. Matrix correlation analyses showed that MMC alters the co-expression of genes related to inflammatory processes that promote microenvironment inflammation. CONCLUSION: These results revealed an altered humoral immune response in MMC patients, contributing to an inflammatory profile and providing opportunities for identifying potential biomarkers in myelomeningocele disease.

15.
Childs Nerv Syst ; 2024 Mar 27.
Artigo em Inglês | MEDLINE | ID: mdl-38536450

RESUMO

PURPOSE: Chiari II malformation (CM-II) is a congenital malformation of the posterior fossa associated with myelomeningocele. Of the symptomatic patients, 10-33% require surgical treatment. To this date, there is not a consensus about the best surgical technique, and whether to do duroplasty. METHODS: A literature search of the PubMed database and crossed references was performed, per PRISMA guidelines. Data regarding demographic features, extent of cervicomedullary deformity, clinical presentation, surgical techniques, and clinical outcomes were extracted. Pearson's chi-squared test was applied. The p-values under 0.05 were considered statistically significant. RESULTS: Twenty studies (N = 330) were analyzed. C3 and C4 levels represented 56.4% of the lowest tonsil displacement. The most reported symptom was dysphagia/swallowing dysfunction (53.8%). Suboccipital craniectomy (SOC) and cervical spine expansion (CSE) with duroplasty were the most reported technique. Dural augmentation was performed in 57.4% of the patients. After surgery, 59.6% observed an improvement in symptoms and quality of life, 12.5% were unchanged, and 27.8% had a worsened clinical status. The mortality rate was 2.5% during the first month after surgery, and 17.4% at the last follow-up evaluation. Patients who underwent CSE presented a better clinical outcome (p = 0.002). The SOC procedure could not be correlated with symptom improvement (p = 0.06). CONCLUSION: CM-II is associated with high morbidity and mortality. An early onset symptomatic CM-II demands intervention, which provided an improvement of outcome in most patients included in this review. The best surgical technique and the exact effect of the surgical management of CM-II on mortality are not yet clear.

16.
Childs Nerv Syst ; 40(5): 1541-1569, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38459148

RESUMO

OBJECTIVES: To describe surgical treatment of 3 cases of severe and progressive thoracolumbar kyphosis in myelomeningocele and provide a systematic review of the available literature on the topic. METHODS: Medical records and pre- and post-operative imaging of 3 patients with thoracolumbar kyphosis and myelomeningocele were reviewed. A database search was performed for all manuscripts published on kyphectomy and/or surgical treatment of kyphosis in myelomeningocele. Patients' information, preoperative kyphosis angle, type of surgery, levels of surgery degrees of correction after surgery and at follow-up, and complications were reviewed for the included studies. RESULTS: Three cases underwent posterior vertebral column resection (pVCR) of 2-4 segments at the apex of the kyphosis (kyphectomy). Long instrumentation was performed with all pedicle screws constructed from the thoracic spine to the pelvis using iliac screws. According to literature review, a total of 586 children were treated for vertebral kyphosis related to myelomeningocele. At least one vertebra was excised to gain some degree of correction of the deformity. Different types of instrumentation were used over time and none of them demonstrated to be superior over the other. CONCLUSION: Surgical treatment of progressive kyphosis in myelomeningocele has evolved over the years incorporating all major advances in spinal instrumentation techniques. Certainly, the best results in terms of preservation of correction after surgery and less revision rates were obtained with long construct and screws. However, complication rate remains high with skin problems being the most common complication. The use of low-profile instrumentation remains critical for treatment of these patients.


Assuntos
Cifose , Meningomielocele , Fusão Vertebral , Criança , Humanos , Meningomielocele/cirurgia , Resultado do Tratamento , Fusão Vertebral/métodos , Estudos Retrospectivos , Cifose/cirurgia
17.
Childs Nerv Syst ; 40(5): 1571-1575, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38514517

RESUMO

The intrauterine repair of myelomeningocele presents certain advantages and has gained widespread acceptance. It significantly reduces the incidence of Chiari-2 anomalies and hydrocephalus, and it is thought to enhance the neurologic outcome. Nevertheless, several issues remain unsettled and there are no negligible disadvantages. After working with patients with myelomeningocele for 30 years, I thought about how we currently treat them. There are ethical, organizational, neurological, obstetrical, and postnatal aspects worth discussing.


Assuntos
Malformação de Arnold-Chiari , Hidrocefalia , Meningomielocele , Humanos , Meningomielocele/cirurgia , Malformação de Arnold-Chiari/complicações , Hidrocefalia/cirurgia , Incidência
18.
J Neurosurg Case Lessons ; 7(10)2024 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-38437675

RESUMO

BACKGROUND: Anterior cervicothoracic myelomeningoceles are a rare pathology. In reported cases, treatment has included shunting, isolated resection and repair without deformity correction, or isolated deformity correction without meningocele repair. The authors describe a pediatric patient with an anterior cervicothoracic myelomeningocele presenting with progressive neurological decline, who underwent simultaneous treatment of the myelomeningocele to detether the spinal cord and achieve major correction of the scoliotic deformity. OBSERVATIONS: A 15-year-old girl was born with C7-T1-T2 hemivertebrae and anterior cervical myelomeningocele at C7-T1. She developed progressive cervical thoracic scoliosis, left hemiparesis initially, and additional right hemiparesis eventually. She underwent surgical repair via C7, T1, and T2 corpectomies with intradural detethering of the spinal cord. The scoliosis was treated with C7-T2 Ponte osteotomies and C2-T5 posterior fixation, followed by anterior reconstruction with a titanium cage and anterior plate from C6 to T3. The myelomeningocele was adequately treated with good correction of the patient's deformity. The patient had postoperative improvement in her strength and solid arthrodesis on postoperative imaging. LESSONS: The authors describe the successful treatment of an anterior cervicothoracic myelomeningocele and associated scoliosis in a child. This is a unique report of a combined strategy to achieve both deformity correction and detethering of the spinal cord.

19.
J Neurosurg Case Lessons ; 7(10)2024 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-38437685

RESUMO

BACKGROUND: Myelomeningocele (MMC) is the most serious form of spina bifida, a congenital defect in neural tube development. Defect closure in a patient with an extremely low birth weight presents unique challenges and risks; lower birth weight is associated with multiple organ system concerns, homeostasis is difficult, and local tissue is underdeveloped. To the authors' knowledge, the present case is the lowest reported weight (490 g) for a neonate with postnatal MMC repair. OBSERVATIONS: A preterm male with a prenatally diagnosed lumbosacral MMC and associated Chiari malformation type II was born at 23 weeks 1 day to a 29-year-old mother, gravidity 6 parity 4. The patient was medically stabilized and underwent MMC closure on day of life 5. His weight was 490 g at the time of this repair, and he did not have any surgical complications. At age 16 months, he underwent endoscopic third ventriculostomy with choroid plexus cauterization; he has not required any further hydrocephalus treatments since the last follow-up at 30 months of age. LESSONS: To the authors' knowledge, this case is the lowest birth weight ex utero MMC closure reported in the literature. Challenges of prematurity and size required appropriate preoperative stabilization, careful hemostasis and temperature regulation, and meticulous surgical technique.

20.
Radiol Case Rep ; 19(5): 1872-1875, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38434787

RESUMO

Chiari malformations are structural defects in the cerebellum that are characterized by the downward displacement of one or both cerebellar tonsils through the foramen magnum. A case report of a female neonate with clinical features of Chiari II malformation is presented. The diagnosis of this condition was made through a combination of clinical and radiological features. In this case, the diagnosis was confirmed by a noncontrast computed tomography (CT) scan of the brain and multiplanar brain magnetic resonance imaging (MRI).

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