Child's Nervous System最新文献

Background: Myelomeningocele (MMC) and myeloschisis (MS) are severe neural tube defects that result in neurodevelopmental impairments and hydrocephalus due to prenatal spinal cord exposure to amniotic fluid. Fetal MMC/MS (fMMC/MS) repair has become the standard of care in appropriately selected patients, demonstrating improved outcomes, including a reduction in the need for cerebrospinal fluid (CSF) diversion compared to postnatal MMC/MS (pMMC/MS) closure. This study is a detailed analysis of the incidence, timing, and imaging predictors of hydrocephalus and CSF diversion dependence in fetal repair and postnatal closure patients in the post-MOMS trial era.

Methods: A retrospective review was conducted of MMC/MS patients treated at a single institution between 2016 and 2023. Inclusion criteria required complete prenatal and postnatal follow-up data. Imaging metrics, including prenatal atrial diameter (AD) and postnatal fronto-occipital horn ratio (FOR), were analyzed alongside head circumference (HC) growth trajectories. Statistical analyses, including Youden's index, were performed to identify predictive cutoffs for shunt dependence.

Results: Among 333 MMC/MS patients, fetal repair significantly reduced permanent CSF diversion rates compared to postnatal closure (27.8% vs. 70.1%, p < 0.01). Timing of clinical hydrocephalus onset was delayed in fetal patients (24.2 weeks vs. 2.8 weeks, p < 0.01). HC of fetal patients was highly correlated with timing of shunt dependence. AD ≥ 10 mm and postnatal FOR ≥ 0.5 were associated with higher shunt dependence (p < 0.01). Optimal cut points for predicting shunt dependence were identified by Youden's index as 14 mm for AD and 0.57 for early postnatal FOR.

Conclusion: Fetal repair of MMC/MS decreases the incidence of clinical hydrocephalus and delays its onset compared to postnatal closure. Early imaging metrics (AD and FOR) may stratify hydrocephalus risk, enabling improved prenatal counseling and postnatal care. Long-term follow-up remains crucial for early detection and management of hydrocephalus in fetal MMC/MS patients.

Purpose: In recent years, there has been a growing emphasis on studying neurosurgical diseases and access to care in LMICs. This study aims to analyze the pattern of pediatric neurosurgical disorders in our outpatient department, highlighting common conditions and patient demographics.

Methods: In this retrospective outpatient department (OPD) based study, pediatric patients with neurosurgical diagnoses between 2017 and 2023 were reviewed to characterize demographics and spectrum of disorders. Descriptive statistics were employed to summarize the data.

Results: A total of 836 patients were included in the study, with a male-to-female ratio of 1:1. The median age was 2.0 years (range, 0.4-8.9 years). Hydrocephalus was the most common disorder, accounting for 41.0% (343/836) of cases. Other congenital anomalies (18.2%), spine disorders/deformities (10.3%), and CNS tumors (10.2%) were also prevalent. Other congenital conditions included spina bifida (11.9%) and encephalocele (5.7%). Scoliosis was the most frequent spine disorder, accounting for 61.6% (53/86) of spine cases, followed by Potts disease (18.6%). For patients with location data, 68.4% (270/408) were from the Greater Accra Region, followed by 13.9% (57/408) from the Eastern region. The median travel distance was 167.4 km (interquartile range, 225-122).

Conclusion: Our institute manages varying pediatric neurosurgical disorders primarily consisting of hydrocephalus. The results suggest specific disease priorities for the pediatric population that we care for and serve as a guide for future clinical efforts.

Purpose: Despite the unique challenges presented by pediatric intracranial AVMs, little data is available on the use of GKRS in the pediatric population. Therefore, we aimed to present our experience with GKRS in treating pediatric AVMs, determining the factors that influence nidus obliteration and poor outcomes, with a focus on delayed complications.

Methods: This retrospective review examined patients ≤18 years who underwent GKRS between January 2013 and December 2021 for intracranial AVMs. Factors that predicted residual nidus and poor outcomes were analyzed. The utility of AVM size reduction at follow-up in predicting eventual nidus obliteration was tested.

Results: We treated 101 pediatric patients for AVMs with a mean age of 14.2 ± 2.8 years. The mean dose delivered was 22.7 ± 2.4 Gy. After a mean follow-up of 41.4 months, 78 AVMs (77.8%) were obliterated. Residual nidus was associated with prior embolization (HR 4.953; 95% CI 1.343, 18.268; p = 0.016) and age ≥14 years (HR 5.920; 95% CI 1.559, 22.480; p = 0.009) while presentation with bleed (HR 0.178; 95% CI 0.05, 0.631; p = 0.008) was protective. Reduction in size of a nidus during early follow-up closely correlated with eventual obliteration, with increasing accuracy at 6-month, 12-month, and 24-month follow-up (67.6%, 80.2%, and 86.5% respectively). Twelve patients developed perilesional edema, while one patient each developed a chronic encapsulated hematoma (CEH), cyst formation, and rebleed. Dose > 22 Gy (HR 25.641; 95% CI 2.257, 250; p = 0.009) and volume ≥ 3 cc (HR 7.189; 95% CI 1.176, 43.945; p = 0.033) predicted poor outcomes on multivariate analysis.

Conclusions: GKRS delivers a high rate of AVM nidus obliteration in the pediatric population, with prior embolization, older age, and unruptured presentation associated with residual nidus. The incidence of delayed complications, although low, warrants regular surveillance in the pediatric population due to their greater life expectancy.

This systematic review investigates the management of sterile abdominal pseudocysts (APCs), a complication associated with ventriculoperitoneal shunts (VPS). Management options for sterile APCs include repositioning and externalization, but there remains no consensus on which management technique is superior in terms of outcomes in recurrence or overall complication rates. Therefore, a comparison of outcomes between shunt externalization and repositioning was conducted. A systematic review was done through PRISMA guidelines, and a search of multiple databases, including Medline and Embase, was conducted from the date of inception until 2023. The search results demonstrated 382 articles. Of the 382 articles, 252 were unique, and 43 articles were included in the analysis after full-text analysis. The results of our analysis indicate there is no significant difference in pseudocyst recurrence and overall complications between externalization and repositioning of the distal shunt catheter. The rate of pseudocyst recurrence for studies with a follow-up of 6 or more months was 25% and 24.1% for repositioning and externalization, respectively (p = 0.99). The overall complication rate for studies with a follow-up of 6 or more months was 44.4% and 34.5% for repositioning and externalization, respectively (p = 0.3861). Although our analysis did not demonstrate a significant difference between the two approaches, further work that includes prospective studies, longer follow-up periods, and larger sample sizes is needed to establish this.

A 10-year-old male with juvenile idiopathic scoliosis underwent an anterior vertebral body tether (VBT) and two disc releases to correct his 72° thoracolumbar curvature. To the best of our knowledge, this procedure has not been previously documented. The patient trialed bracing prior to presenting to our institution, but his curve continued to progress. In an effort to maintain truncal flexibility and preserve growth, the patient and his family elected for a VBT. A preoperative MRI was unremarkable, and the patient had no underlying medical conditions. The operation was complicated by undersized and severely rotated vertebrae. The curvature failed to adequately correct with increasing cord tension. Two disc releases were performed at the apex of the curve: T8-T9 and T9-T10. The VBT cord spanned T5-L3. At the patient's 3-year post-operative appointment, his major cobb angle measured 22° and showed no evidence of fusion.

Purpose: To evaluate indications, techniques, nuances, and outcomes of posterior cranial vault distraction (PCVD) in children with craniosynostoses.

Methods: We performed clinical assessments, multidimensional CT, MRI brain rapid protocol, ophthalmological evaluation, sleep study, and nasal endoscopy (if indicated). Detailed data was collected in Excel. Customized craniotomy (supratorcular or subtorcular), distraction vectors, strategic barrel staving on stenosed bones (other than lambdoid), and ~ 2 cm relief craniectomy for venous decompression were employed. Additional procedures were performed for the frontal and midface aspects during distractor removal based on functional needs.

Results: Thirty-seven patients (ages 4-204 months, mean 32.94 months) underwent PCVD. Supratorcular PCVD in 8, subtorcular in 29. The distraction vector was posterior-horizontal in 28 cases and posterior-inferior in 9. Strategic barrel staving was used in 8 cases, and venous decompression in 24. Initial assessments showed satisfactory clinical and radiological outcomes. Long-term follow-up indicated seven of 11 patients with hydrocephalus required a ventriculoperitoneal shunt, and two needed additional PCVD procedures due to symptom recurrence. Average intracranial volume increased by 186 ± 42.67 cm³ (18 patients), and the average distraction achieved was 21 ± 2.64 mm (37 patients). Additional procedures at the time of distractor removal included fronto-facial or monobloc advancement (n = 3), isolated fronto-orbital advancement and remodeling (n = 13), and midface distraction for airway issues (monobloc advancement, n = 3; isolated midface, n = 9). Nine patients underwent all three procedures in sequence.

Conclusion: PCVD is an accepted surgical strategy for craniosynostosis with posterior calvarial involvement. Our technical modifications aim to enhance functional and aesthetic outcomes without increasing morbidity.

Background: Pediatric patients with drug-resistant epilepsy and normal preoperative MRIs present significant challenges in surgical planning. Advanced diagnostic techniques, including PET, SPECT, and intraoperative ECOG, are used to localize seizure foci, but their high cost and limited availability pose challenges, especially in low-resource settings. This study aims to evaluate the outcomes of resective epilepsy surgery in these cases and assess the role of advanced imaging in a middle-income country.

Methods: This retrospective cohort study included 12 pediatric patients (mean age 10.21 years) with normal preoperative 3 T MRI who underwent resective epilepsy surgery or functional hemispherectomy between 2007 and 2021 at two centers in Medellín, Colombia. Demographic, clinical, and surgical data were collected, including the use of advanced imaging techniques (PET, SPECT) and intraoperative ECOG. Seizure outcomes were assessed using the Engel Epilepsy Surgery Outcome Scale.

Results: Of the 12 patients, 10 underwent extratemporal resections, and 2 underwent temporal lobe surgery. Seven patients had advanced imaging, and 5 were evaluated with intraoperative ECOG. At 2-year follow-up, 83.3% of patients who underwent resective surgery achieved favorable outcomes (Engel Classes I and II). Temporal lobe resections had a higher rate of seizure freedom (50%) compared to extratemporal resections (30%), although the difference was not statistically significant (p = 0.47). Reoperations due to seizure recurrence were required in 30% of extratemporal resections (p = 0.02). Complications were minimal, with three superficial wound infections. Histopathology revealed cortical dysplasia in 33.3% of cases.

Conclusion: Epilepsy surgery in pediatric patients with normal MRIs can yield favorable outcomes, especially with temporal lobe resections. Advanced imaging improves localization but remains costly, highlighting the need for cost-effective surgical strategies in resource-limited settings.

Introduction: Adamantinomatous craniopharyngioma (ACP) is a benign epithelial tumor of the sellar and suprasellar region, and in children, it usually presents with cysts and calcifications.

Methods: In this study, ACP samples were collected, and after enzymatic digestion of the calcified component of these tumors, which were placed in culture to isolate possible cellular components, in vitro and in vivo viability analysis and characterization were performed.

Results: ACP-calcified component cells cultured in vitro were established showing the doubling time in 2 days, confirmed by the 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay. Other analyses were carried out, such as ultrastructural characterization and the growth of ACP-calcified component cells in xenograft animal model by MRI monitoring. Immunocytochemistry detected cytokeratin-7 expression in the cytomembrane and cytoplasm of ACP-calcified component cells, confirming that the tumor mass established in the xenograft animal model comes from calcified component of ACP patients' cell cultures which were squamous epithelial cells.

Conclusions: Thus, our results suggest that the calcifications present in ACPs have the capacity to generate a tumor, and we could only consider complete tumor excision when all calcifications have been removed. Therefore, these ACP-calcified component cell cultures may be important to study possible targets for drug therapies and help understand the progression mechanisms of this tumor and better control its spread.