Since our last Special Annual Issue dedicated to the topic of ependymoma in 2009, critical advancements have been made in the understanding of this disease which is largely confined to childhood. In ...the era of molecular profiling, the prior classification of ependymoma based on histology has become largely irrelevant, with multiple new subtypes of this disease now being described in the newest 2021 WHO CNS Tumor Classification System. Despite our advancements in understanding the underlying biology of these tumors, the mainstays of treatment—gross total surgical resection followed by confocal radiation therapy—have continued to yield the best treatment results across multiple studies and centers. Here, we provide an update on our understanding of the advancements made in tumor biology, surgical, and oncologic management of this disease. As we move into an era of more personalized medicine, it is critical to reflect on our historical understanding of different disease entities, to better understand the future directions of our treatments.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, PRFLJ, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Low-grade gliomas are the most common brain tumor of childhood, and complete resection offers a high likelihood of cure. However, in many instances, tumors may not be surgically accessible without ...substantial morbidity, particularly in regard to gliomas arising from the optic or hypothalamic regions, as well as the brainstem. When gross total resection is not feasible, alternative treatment strategies must be considered. While conventional chemotherapy and radiation therapy have long been the backbone of adjuvant therapy for low-grade glioma, emerging techniques and technologies are rapidly changing the landscape of care for patients with this disease. This article seeks to review the current and emerging modalities of treatment for pediatric low-grade glioma.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, PRFLJ, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Abstract BACKGROUND The outcomes of pediatric low-grade glioma (pLGG) are markedly influenced by accessibility to neurosurgical services. Even when clinically recommended, neurosurgical interventions ...might not be feasible when there is scarcity in qualified medical professionals and preparedness of medical facilities, which is the case in many low- and middle-income countries (LMICs). To aid cancer control planning efforts and guide policy makers and clinicians to prioritize allocation of resources, accurate neurosurgical utilization rate (NUR) estimations are crucial. This study developed a model to estimate the NUR, to facilitate the approximation of neurosurgery needs for pLGG. METHODS A decision tree model was developed to assess the NUR in HICs and LMICs. Patients were classified into five groups based on the anatomic site of the tumor. For each type of cancer, patients had three surgical options, gross total resection, subtotal resection, and biopsy. Anatomic distribution and likelihood to perform surgical resection were retrieved from the literature for both HICs and LMICs. RESULTS Findings showed discrepancy in the anatomic distribution of pLGG in HICs and LMICs. Proportion of patients with cerebral hemisphere, cerebellum, OPHG/diencephalon, brainstem, and spine tumors and their likelihood to undergo surgical intervention were 34% | 28%, 32% | 27%, 17% | 4%, 12% | 3% and 5% | 4% in HICs, and 26% | 22%, 39% | 32%, 26% | 5%, 6% | 2% and 3% | 2% in LMICs, respectively. Overall, NUR in pLGG patients was 66% in HICs and 63% in LMICs. CONCLUSION Despite the limited resources in LMICs, the overall proportion of patients recommended to undergo a surgical intervention is comparable to HICs. For this need to be met, long-term planning need to take place at the policymaking level. It is possible to estimate the NUR for all pediatric CNS tumors following the same approach to more comprehensively estimate the neurosurgical needs of this patient population globally.
The childhood brain tumor, medulloblastoma, includes four subtypes with very different prognoses. Here, we show that paracrine signals driven by mutant β-catenin in WNT-medulloblastoma, an ...essentially curable form of the disease, induce an aberrant fenestrated vasculature that permits the accumulation of high levels of intra-tumoral chemotherapy and a robust therapeutic response. In contrast, SHH-medulloblastoma, a less curable disease subtype, contains an intact blood brain barrier, rendering this tumor impermeable and resistant to chemotherapy. The medulloblastoma-endothelial cell paracrine axis can be manipulated in vivo, altering chemotherapy permeability and clinical response. Thus, medulloblastoma genotype dictates tumor vessel phenotype, explaining in part the disparate prognoses among medulloblastoma subtypes and suggesting an approach to enhance the chemoresponsiveness of other brain tumors.
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•WNT-medulloblastoma but not other disease subtypes lack a blood brain barrier (BBB)•WNT-medulloblastoma secreted WNT antagonists block BBB formation•BBB function dictates chemotherapy exposure and response among medulloblastomas•The BBB can be manipulated in medulloblastoma by altering tumor vessel signaling
Phoenix et al. show that medulloblastoma genotype dictates tumor vessel phenotype, partially explaining disparate prognoses among subtypes. WNT-medulloblastoma paracrine signals block endothelial WNT signaling, and manipulation of the paracrine axis can alter chemotherapy permeability and response in vivo.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
INTRODUCTION: Tethered cord syndrome refers to a constellation of symptoms characterized by neurological, musculoskeletal, and urinary symptoms, caused by traction on the spinal cord. Various ...surgical techniques have been developed to address tethered cord syndrome depending on a diverse set of etiologies. Understanding perioperative complications and secondary retethering is crucial for optimizing patient outcomes and refining surgical techniques. METHODS: Retrospective review of children who underwent TCR surgery from July 2014 to April 2023. Data included demographics, perioperative characteristics, surgical techniques, and follow-up duration. Primary outcomes were 60-day postoperative complications and secondary retethering requiring repeat surgery. RESULTS: We identified 363 TCR surgeries (145 simple, 218 complex, 340 subjects). Mean follow-up was 446 days for simple TCRs and 731 days for complex TCRs. The adjusted 60-day complication rate was 3.8% (95%-CI 1.6-9.1) for simple TCRs and 11.9% (95%-CI 9.0-19.4) for complex TCRs. Lower weight, shorter surgical times, and ICU admission were associated with complications for simple TCRs. Soft tissue drains increased complications for complex TCRs. Secondary retethering rates were 1.4% for simple TCRs and 11.9% for complex TCRs. The 1-, 3-, and 5-year progression-free survival in complex cases were 94.7% (95%-CI 89.1-97.4), 77.7% (95%-CI 67.3-85.3), and 62.6% (95%-CI 46.5-75.1). Multivariate analysis revealed that prior detethering (OR 8.15, 95%-CI 2.33-28.50, p = 0.001) and usage of the operative laser (OR 10.43, 95%-CI 1.36-80.26, p = 0.024) were independently associated with secondary retethering in complex cases. CONCLUSIONS: This is the largest series examining postoperative complications and long-term secondary retethering in TCR surgery. Simple TCR surgeries demonstrated safety, rare complications, and low secondary retethering rates. Complex TCR surgeries presented higher risks for complications and secondary retethering. Modifiable risk factors, such as operative laser usage, influenced secondary retethering in complex cases.
The 2021 U.S. neurosurgery residency match interviews were conducted virtually; we surveyed applicants and interviewers to determine satisfaction with that virtual interview process. Subsequently, we ...conducted a follow-up survey to determine satisfaction with the virtual interview process after the residency match for faculty interviewers and 2022 interns.
A 22-question online faculty survey was sent to 116 U.S. neurosurgery training programs. A 26-question survey was sent to these programs for distribution to their intern classes. Data were analyzed quantitatively, including mean Likert score. Open-ended questionnaire responses were reviewed to identify themes.
Overall, 32 interns representing 20 programs and 73 faculty representing 62 programs responded. Most respondents agreed that virtual interviews were more convenient (86% faculty, 90% interns) and cost-effective (100% interns) than in-person interviews. Faculty respondents agreed or strongly agreed that virtual interviews were effective to evaluate applicants' competence as residents (44%); fewer faculty agreed or strongly agreed that virtual interviews were an effective way to evaluate candidates' fit in the program (27%). For interns, 44% agreed or strongly agreed that virtual interviews gave them a good sense of the program faculty; 75% agreed or strongly agreed they were satisfied with the process related to where they matched.
Virtual interviews offer an advantage in terms of time and cost but potentially at the expense of adequate faculty assessment of candidates' “fit” within a program's culture. Despite this, interns undergoing an all-virtual interview process report high satisfaction with the results of the residency match.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
The authors describe the unusual case of a 6-year-old boy presenting with decorticate posturing, diminished hearing, and an inability to open his eyes, despite being verbally responsive. He underwent ...a posterior interhemispheric transcallosal intervenous approach for resection of a pineal region mature teratoma, which recurred 2 years postoperatively. This video demonstrates his initial surgery and reresection, illustrating the value of this approach for more complex lesions that involve the internal cerebral veins (ICVs). At the time of recurrence, microsurgical dissection of the scarred interhemispheric fissure was required to facilitate removal of the multifocal recurrent teratoma, resulting in gross-total resection. The video can be found here: https://stream.cadmore.media/r10.3171/2021.4.FOCVID2134 .
OBJECTIVE Research on pediatric abusive head trauma (AHT) has largely focused on clinical presentation and management. The authors sought to review a single-institution experience from a public ...health perspective to gain a better understanding of the local population affected, determine overall incidence and seasonal trends, and provide details on the initial hospitalization, including extent of injuries, neurosurgical interventions, and hospital charges. METHODS All cases of AHT involving patients who presented to Le Bonheur Children's Hospital (LBCH) from 2009 through 2014 were identified. AHT was defined as skull fracture or intracranial hemorrhage in a child under the age of 5 years with a suspicious mechanism or evidence of other intentional injuries, such as retinal hemorrhages, old or new fractures, or soft-tissue bruising. Injuries were categorized as Grade I (skull fracture only), Grade II (intracranial hemorrhage or edema not requiring surgical intervention), or Grade III (intracranial hemorrhage requiring intervention or death due to brain injury). RESULTS Two hundred thirteen AHT cases were identified. The demographics of the study population are similar to those reported in the literature: the majority of the patients involved were 6 months of age or younger (55%), male (61%), African American (47%), and publicly insured (82%). One hundred one neurosurgical procedures were performed in 58 children, with the most common being bur hole placement for treatment of subdural collections (25%) and decompressive hemicraniectomy (22%). The annual incidence rate rose from 2009 (19.6 cases per 100,000 in the population under 5 years of age) to 2014 (47.4 cases per 100,000) and showed seasonal peaks in January, July, and October (6-year average single-month incidence, respectively, 24.7, 21.7, and 24.7 per 100,000). The total hospital charges were $13,014,584, with a median cost of $27,939. Treatment costs for children who required surgical intervention (i.e., those with Grade III) were up to 10 times those of children with less severe injuries. CONCLUSIONS In the authors' local population, victims of AHT are overwhelmingly infants, are more often male than female, and are disproportionately from lower socioeconomic ranks. The incidence is increasing and initial hospitalization charges are substantial and variable. The authors introduce a simple 3-tiered injury classification scheme that adequately stratifies length of hospital stay and cost.
Robot-assisted stereoelectroencephalography (sEEG) is frequently employed to localize epileptogenic zones in patients with medically refractory epilepsy (MRE). Its methodology is well described in ...adults, but less so in children. Given the limited information available on pediatric applications, the objective is to describe the unique technical challenges and considerations of sEEG in the pediatric population. In this report, we describe our institutional experience with the technical aspects of robot-assisted sEEG in an exclusively pediatric epilepsy surgery unit, focusing on pre-, intra-, and post-operative nuances that are particular to the pediatric population. The pediatric population presents several unique challenges in sEEG, including reduced skull thickness relative to adults, incomplete neurologic development, and often special behavioral considerations. Pre-operative selection of putative epileptogenic zones requires careful multidisciplinary decision-making. Intraoperative attention to nuances in positioning, clamp selection, registration, and electrode placement are necessary. Activity considerations and electrode migration and removal are key post-operative considerations. Robot-assisted sEEG is a valuable tool in the armamentarium of techniques to characterize MRE. However, special considerations must be given to the pediatric population to optimize safety and efficacy.
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EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PNG, PRFLJ, SAZU, SBCE, SBJE, SBMB, SBNM, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Surgical management of pediatric patients with nonlesional, drug-resistant epilepsy, including patients with Lennox-Gastaut syndrome (LGS), remains a challenge given the lack of resective targets in ...most patients and shows seizure freedom rates <50% at 5 years. The efficacy of deep brain stimulation (DBS) is less certain in children than in adults. This study examined clinical and seizure outcomes for pediatric patients with LGS undergoing DBS targeting of the centromedian thalamic nuclei (CMTN).
An institutional review board–approved retrospective analysis was performed of patients aged ≤19 years with clinical diagnosis of LGS undergoing bilateral DBS placement to the CMTN from 2020 to 2021 by a single surgeon.
Four females and 2 males aged 6–19 years were identified. Before surgery, each child experienced at least 6 years of refractory seizures; 4 children had experienced seizures since infancy. All took antiseizure medications at the time of surgery. Five children had previous placement of a vagus nerve stimulator and 2 had a previous corpus callosotomy. The mean length of stay after DBS was 2 days. No children experienced adverse neurologic effects from implantation; the mean follow-up time was 16.3 months. Four patients had >60% reduction in seizure frequency after surgery, 1 patient experienced 10% reduction, and 1 patient showed no change. No children reported worsening seizure symptoms after surgery.
Our study contributes to the sparse literature describing CMTN DBS for children with drug-resistant epilepsy from LGS. Our results suggest that CMTN DBS is a safe and effective therapeutic modality that should be considered as an alternative or adjuvant therapy for this challenging patient population. Further studies with larger patient populations are warranted.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
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