Pediatric Neurosurgery最新文献

Background: "Business, like life, is all about how you make people feel. It's that simple and it's that hard" says Danny Meyer, the restauranteur and CEO of the Union Square Hospitality Group in New York. Similarly, Maya Angelou famously said that people will never forget how you made them feel, though they may forget what you said or did. In neurosurgery, we are doing two things at once: something very technical and something very human. The core thesis of this hospitality philosophy is that whereas the technical aspect of our job is critically important, it represents 49% of our success. The human aspect of our work represents 51%, ever so slightly more important.

Summary: We will explore how hospitality has impacted the practice of and a career in neurosurgery over a 35-year period, based on the principles outlined by Danny Meyer in his 2006 book Setting the Table. We will define the difference between service, the technical delivery of a product (e.g., a surgical procedure), and hospitality, how the delivery of that service makes someone feel; if someone feels you are on their side, hospitality is present. We will also define the 51% rule for hiring: one is invited onto our team based 49% on technical skill and 51% on these hospitality-related human qualities (optimistic warmth, intelligence, work ethic, empathy, self-awareness, and integrity).

Key messages: Hospitality has played a transformative role in a neurosurgery career: in developing a destination academic practice, managing complications, overcoming challenges, and in building an outstanding team. In our opinion, hospitality plays a significant role in pediatric neurosurgery, driving growth in activity and excellence. As Danny says "it takes both great service and great hospitality to rise to the top."

Introduction: Endoscopic strip craniectomy followed by molding helmet therapy for sagittal suture synostosis is thought to be most effective in infants 3-4 months of age. However, infants often present to a neurosurgeon beyond this time period. The aim of this study was to compare morphological outcomes in older infants (5-7 months of age) with younger infants, in a series of babies who were operated endoscopically for sagittal suture synostosis.

Methods: The prospectively maintained Pediatric Neurosurgery Clinical Database at BC Children's Hospital was interrogated and 81 pediatric patients who had undergone an endoscopic craniectomy between 2010 and 2021 for sagittal craniosynostosis and had follow-up of at least 1 year were identified. Eleven of these patients received surgical intervention when they were 5 months of age or older (median 5.4 months, range 5.0-6.4). Morphological indices, duration of helmet therapy, and reoperation rates were compared between the early and late intervention groups.

Results: Both the early intervention (surgery before 5 months of age, n = 70) and the late intervention (surgery at 5 months or later, n = 11) groups had comparable average preoperative cephalic index (CI) (early - 67.1 vs. late - 69.4), change in CI (early - 6.7 vs. late - 4.7), and 2-year follow-up CI (early - 73.8 vs. late - 74.1). Both groups had similar average helmeting duration (6.6 vs. 6.1 months). Of the 11 late intervention patients, none required reoperation but poor cosmetic outcome was noted in 1 patient. The early intervention group had 1 patient who required a secondary cranial vault reconstruction due to persistent scaphocephaly.

Conclusion: Infants who received an endoscopic craniectomy for sagittal suture synostosis after 5 months of age showed no statistically significant difference in morphological outcomes compared to the early intervention group.

Background: Epilepsy remains one of the most common neurological disorders in children, with approximately one-third of patients developing drug-resistant epilepsy (DRE) that may require surgical intervention. This review outlines the technical neurosurgical aspects of pediatric epilepsy surgery, focusing on resective and disconnection procedures.

Summary: Successful epilepsy surgery relies on accurate identification of the hypothesized epileptogenic zone through comprehensive presurgical evaluation including clinical assessment, seizure semiology analysis, magnetic resonance imaging, electroencephalography (EEG), and neuropsychological testing. In complex cases, additional imaging modalities such as FDG-PET, SPECT, and MEG help establish the anatomo-electric-clinical network. Invasive monitoring using subdural grids or stereotactic EEG provides critical data for surgical planning in cases with discordant findings, potential involvement of eloquent tissue, or non-lesional epilepsy. Resective surgery is the primary intervention for focal, lesional epilepsy, with seizure freedom rates varying by location and etiology (70-80% for temporal lobe and tumor-associated epilepsy; 60% for extra-temporal lobe; 51% for non-lesional cases). Anterior temporal lobectomy, a cornerstone procedure, involves careful consideration of hemisphere dominance and selective approaches to mesial structures. Extent of resection is a critical determinant of outcome, with incomplete removal of epileptogenic tissue consistently identified as the leading cause of surgical failure. Disconnection procedures include anterior and posterior quadrant disconnections, hemispherectomy, and corpus callosotomy. Anterior quadrant disconnection isolates seizure foci within the frontal lobe, whereas posterior quadrant disconnection targets the temporal, parietal, and occipital lobes. Functional hemispherotomy has largely replaced anatomic hemispherectomy because it has a lower complication profile but maintains equivalent seizure freedom rates. Corpus callosotomy primarily targets generalized seizures, especially atonic seizures leading to drop attacks.

Key messages: Recent advances include minimally invasive techniques (such as laser ablation) and neuromodulation approaches (such as responsive neurostimulation and deep brain stimulation). Future directions will likely incorporate higher resolution imaging technologies and artificial intelligence driven signal processing to optimize outcomes and offer personalized treatment strategies that improve seizure control and quality of life for children with DRE.

Background: Destructive surgical treatment for drug-resistant epilepsy may lead to complete seizure relief. In cases where a surgical cure is unavailable, epilepsy surgery can still alleviate seizure burden by decreasing reliance on medication, decreasing the severity or frequency of seizures, or decreasing the frequency of status epilepticus.

Summary: In this article, we discuss the role of palliative epilepsy surgical techniques in pediatric patients, relevant syndromes characterized by refractory epilepsy, and pertinent procedures and considerations. We review both destructive and neuromodulatory therapies, the current evidence supporting their use, and discuss future indications and expansion of techniques.

Key message: Our expanding epilepsy surgery armamentarium has the potential to provide significant palliative therapy for our pediatric patients and significantly improve their quality of life.

Background: In pediatric patients with drug-resistant epilepsy, successful localization of the seizure onset zone (SOZ) is critical to surgical planning and outcome prognostication. SOZ localization requires the identification of seizures captured through intracranial electroencephalography (iEEG), necessitating prolonged hospital stays for invasive monitoring and two-stage surgical procedures at minimum. Localization of SOZ in automated fashion using only interictal data would enable a substantial reduction in the time required for pre-resection iEEG recording. Using iEEG to model brain functional connectivity (FC) is an approach that has shown significant potential.

Summary: We conduct a literature review on directed FC methods and their use in preoperative SOZ localization. Granger Causality, an approach originally used to analyze economic time series, has evolved into multiple similar methodologies for directed FC graph creation. Several modalities show strong correlations between electrodes with specific FC patterns and SOZs, but there is no current tool that can reliably predict SOZs from interictal iEEG data.

Key messages: Multiple studies show a pattern of increased inward FC in electrodes located in the SOZ during interictal periods, with reversed information flow during seizures, suggesting the increased inward flow toward the SOZ may represent inhibitory pathways, which, when absent, lead to a more epileptogenic state. Further analysis of the changes in directed FC across longer periods may help elucidate how to select optimal segments for localization.

Introduction: Surgical removal of trigonal intraventricular tumors presents significant challenges. These tumors are primarily supplied by branches of the choroidal artery group arising from the inferior side. To access the vascular pedicles, the shortest and most direct surgical corridor to the trigone is generally preferred, making the transcortical transparietal approach a common choice. However, this approach carries a high risk of damaging critical subcortical white matter tracts. Injury to these tracts can result in language deficits (on the left side), spatial neglect syndrome (on the right side), as well as sensory disturbances and visual field deficits. To minimize disruption to these white matter tracts, the high parietal approach is often recommended. However, this technique presents its own challenges, particularly in achieving early control of arterial feeders, which becomes even more critical in larger trigonal tumors.

Case presentation: We present the case of a 13-year-old boy with a large trigonal intraventricular tumor. Given his lack of significant symptoms and his young age, our surgical strategy focused on maximizing the preservation of his neurological function. We developed a novel combined transtemporal and high parietal approach. The small transtemporal approach allows early devascularization, followed by the high parietal approach, which facilitates tumor resection while preserving the vital white matter tracts along the lateral wall of the trigone.

Conclusion: We propose a novel combined technique that offers a balance between optimal tumor resection and functional preservation, particularly in cases of large and hypervascularized trigonal tumors.

Introduction: Thalamic strokes are uncommon in children and often arise from underlying conditions such as coagulopathy. Their clinical and radiological resemblance to neoplasms - with shared symptoms and overlapping MRI features - makes accurate diagnosis particularly challenging.

Case presentation: A 2-year-old boy with trisomy 21 (Down syndrome) presented with 5 days of altered mentation and fussiness, nausea, vomiting, and seizures. CT head demonstrated a right thalamic edematous lesion. MRI showed a lesion with imaging features suggestive of a thalamic neoplasm that was ultimately found to be an infarct secondary to right internal cerebral vein thrombus. The patient was treated with heparin and returned to his clinical baseline.

Conclusion: Given their predisposition to coagulopathy, patients with Down syndrome require meticulous imaging evaluation. CT venogram can be an essential part of workup for this patient population.

Introduction: There are no predictive outcome scales that have been validated in pediatric patients with brain tumors. An index can help identify children with increased risk for negative postoperative results. The Johns Hopkins Adjusted Clinical Groups (JHACG) frailty and the Elixhauser Comorbidity Index (ECI) have been used independently in adult brain tumor patients to identify patients who are at an increased risk for detrimental outcomes. We investigated whether JHACG and ECI can better predict hospital length of stay (LOS), nonroutine discharge, and 1-year readmission in pediatric patients undergoing craniotomy for primary brain tumors.

Methods: The Nationwide Readmissions Database was queried for pediatric brain tumor resections between 2016 and 2019. In total, 237 and 1,235 patients with benign and malignant tumors were identified, respectively. Frailty, ECI, and Frailty+ECI were assessed as predictors using generalized linear mixed-effects models. Receiver operating characteristic curves evaluated predictive performance.

Results: Frailty+ECI, frailty, and ECI scores similarly predicted hospital LOS, nonroutine discharge, and 1-year readmission in the benign tumor cohort. In the malignant cohort, Frailty+ECI (area under the curve [AUC] 0.895) outperformed frailty alone (AUC 0.742, p = 0.001) but performed similar to ECI score alone (AUC 0.893, p = 0.438) in predicting hospital LOS. Concerning nonroutine discharge prediction, Frailty+ECI (AUC 0.871) also outperformed frailty alone (AUC 0.744, p = 0.04) while performing similarly to ECI score alone (AUC 0.869, p = 0.871). All indices performed in a similar way to predict 1-year readmission in this cohort.

Conclusion: Our study showed that Frailty+ECI demonstrated a robust ability to predict hospital LOS and nonroutine discharge disposition in pediatric patients undergoing malignant brain tumor resection. These findings suggest that combining these indices may improve the prediction of postoperative outcomes in this population. While further studies are warranted, these findings can be used as a risk assessment index to coordinate care plans with the patient and their family after an operation.

Introduction: Hemispherotomy is an effective treatment for children with drug-resistant epilepsy (DRE). While hemispherotomy techniques and indications have evolved, access remains predominantly constrained to high-resource settings.

Methods: We performed a retrospective analysis of children who underwent hemispherotomy from 2011 to 2023 by a hybrid team, including local Panamanian and US neurologists, neurosurgeons, and EEG technicians and analyzed surgical, epilepsy, and quality of life (QoL) parameters. Follow-up data were collected according to the International Consortium for Health Outcomes Measurement (ICHOM) guidelines for children with epilepsy.

Results: Twenty-three children underwent hemispherotomy. The median age at surgery was 10 years (range 2-20). The median follow-up time was 6 years (range 1-13). The etiology of DRE included malformations of cortical development in 14 children (60.8%), including 8 (34.8%) with schizencephaly, and secondary causes in 9 children (39.1%). Seizure frequency improved for all 23 children (100%): Engel I was achieved in 15 children (65.2%), Engel II (26.1%) in six children, and Engel III (8.7%) in two children. Patients with seizure freedom had significantly fewer preoperative seizures per day than patients with seizure recurrence. Complications occurred in six children (26.1%): 2 wound infections, 2 meningitis, 1 femoral vein thrombosis, and 1 wound hematoma with return to OR. There were no perioperative mortality and no postoperative hydrocephalus or CSF diversion. QoL-related outcomes were available for 16 children: 16/16 (100%) reported that the surgery was a worthwhile and repeatable choice, 14 (87.5%) reported improved cognitive function, the median QOLCE-16 score was 62.5 ± 21.

Conclusion: Hemispherotomy for DRE in selected children is a safe and effective surgery in a public children's hospital in a low-resource setting. At last follow-up, the majority of children were seizure-free, and all children had decreased seizure frequency. Families reported improved cognitive function, improved QoL and high satisfaction with their decision to pursue this surgery.

Background: Drug-resistant epilepsy is a debilitating condition that afflicts individuals across all demographics, including children. The only recourse for many of these individuals is neurosurgery to reduce seizure burden, by either resecting or ablating the cerebral source or modulating it with a stimulator device. In either case, a thorough presurgical evaluation is required to identify brain regions of interest and construct an appropriate surgical plan. The scope of this evaluation has grown rapidly over the years as new and refined techniques have emerged. The aim of this article was to condense the most salient points regarding investigational tools used commonly in this process and provide a framework from which epilepsy management providers can tailor their own epilepsy surgery pathway.

Summary: This article will discuss criteria to identify appropriate candidates for epilepsy surgery, as well as various techniques that are used to localize seizure onset, interictally active areas, dysfunctional regions, and eloquent cortex. Topics reviewed include neuroimaging (MRI, PET, SPECT), electrophysiology (EEG and MEG), and functional mapping procedures (fMRI, TMS, neuropsychologic evaluation, intracarotid amobarbital test).

Key messages: A comprehensive, multimodal presurgical evaluation including imaging, electrophysiology, and functional mapping is essential to establish the bounds of the epileptogenic zone in relation to eloquent cortex.