The impact of social determinants of health on utilization of pediatric epilepsy surgery: a systematic review.

OBJECTIVE: A minority of pediatric patients who may benefit from epilepsy surgery receive it. The reasons for this utilization gap are complex and not completely understood. Patient and caregiver social determinants of health (SDOH) may impact which patients undergo surgery and when. The authors conducted a systematic review examining SDOH and surgical intervention in children with drug-resistant epilepsy (DRE). They aimed to understand which factors influenced time to surgical program referral or receipt of epilepsy surgery among children with DRE, as well as identify areas to characterize the SDOH impacting epilepsy surgery in children and guide efforts aimed to promote health equity in epilepsy. METHODS: A systematic review was conducted using the PubMed, Embase, and Scopus databases in January 2022. Studies were analyzed by title and abstract, then full text, to identify all studies examining the impact of SDOH on utilization of epilepsy surgery. Studies meeting inclusion criteria were analyzed for SDOH examined, outcomes, and key findings. Quality was assessed using the Grading of Recommendations Assessment, Development and Evaluation system. RESULTS: Of 4545 resultant articles, 18 were included. Studies examined social, cultural, and environmental factors that contributed to SDOH impacting epilepsy surgery. Patients who underwent surgical evaluation were found to be most commonly White and privately insured and have college-educated caregivers. Five studies found differences in time to referral/surgery or rates of surgery by racial group, with most finding an increased time to referral/surgery or lower rates of surgery for those who were Hispanic and/or non-White. Four studies found that private insurance was associated with higher surgical utilization. Three studies found higher household income was related to surgical utilization. No studies examined biological, psychological, or behavioral factors that contributed to SDOH impacting epilepsy surgery. CONCLUSIONS: The authors conducted a systematic review exploring the impact of SDOH in DRE surgery utilization. They found that race, ethnicity, insurance type, caregiver educational attainment, and household income demonstrate relationships with pediatric epilepsy surgery. Further study is necessary to understand how these factors, and others not identified in this study, contribute to the low rates of utilization of epilepsy surgery and potential target areas for interventions aiming to increase equity in access to epilepsy surgery in children.

Visual outcomes after treatment of craniopharyngioma in children: A systematic review.

OBJECTIVE: Craniopharyngiomas (CP) are rare brain tumors that often result in visual impairment due to their proximity to the optic pathway. The optimal management approach to preserve visual function in these patients remains controversial. We sought to investigate visual outcomes of children with craniopharyngiomas based on treatment modality. METHODS: A systematic review was performed according to PRISMA guidelines. PubMed, Embase, and Scopus databases were searched in December 2022 for relevant articles. Articles were screened by title/abstract for relevance, then by full-text. Relevant demographic, intervention, and outcome data were extracted from included studies. RESULTS: A total of 59 studies were included, representing 2655 patients. The overall visual status (OVS) of patients receiving surgery alone was improved in 27.6% of reported outcomes, unchanged in 50.3%, and deteriorated in 22.1%. The OVS for patients receiving radiation alone was improved in 21.1%, unchanged in 42.1%, and deteriorated in 36.8%. Patients receiving surgery plus adjuvant radiotherapy had OVS improvement in 27.4%, unchanged in 63.2%, and deteriorated in 9.4%. Of those receiving intracystic bleomycin, 23.1% had improvement in OVS, 46.2% remained unchanged, and 30.8% deteriorated. Of patients receiving interferon-α, 34.8% improved, 54.5% remained unchanged, and 10.6% deteriorated. CONCLUSION: OVS most frequently remained unchanged regardless of intervention. The greatest improvement in OVS was seen in those receiving interferon-α or surgery alone. The greatest OVS deterioration was noted with radiation alone. Future standardized, randomized, large-scale studies with focused assessment of ophthalmologic findings are key to further understanding the impact different interventions have on visual outcomes in these children.

Cervical corpectomy in a pediatric patient with chondrodysplasia punctata and C5 dysplasia with spinal cord compression: illustrative case.

BACKGROUND: Chondrodysplasia punctata (CDP) describes skeletal dysplasia secondary to a variety of genetic underpinnings characterized by cartilaginous stippling from abnormal calcium deposition during endochondral bone formation. Approximately 20%-38% of patients with CDP have cervical spine abnormalities, resulting in stenosis and cord compression. However, approaches to management differ among patients. OBSERVATIONS: The authors present an 18-year-old male with a known history of CDP and cervical kyphosis with worsening paresthesias and increased spasticity. Imaging confirmed dysplastic C4 and C5 vertebra with focal kyphosis, bony retropulsion, spinal cord compression, and myelomalacia. To treat the stenosis and deformity, the patient underwent C4 and C5 vertebrectomies with C3 to C6 anterior fusion with resolution of symptoms. LESSONS: Despite many CDP patients having cervical deformities with spinal cord compression and associated neurological symptoms, there is a paucity of data on surgical management and outcomes. There are only scattered reports, and most authors recommend initial conservative management because of the high risk of operative morbidity and mortality secondary to comorbidities. When surgery is performed, long-term follow-up is recommended because of the high rates of progression of deformity, requiring subsequent operations. The authors hope that their experience adds to the literature describing the surgical management of cervical deformities in these patients.

Patient family engagement and partnership: Pilot survey results in assessing behavior, communication, and quality of life in children with Lennox-Gastaut syndrome and other drug-resistant epilepsy.

OBJECTIVES: Lennox-Gastaut Syndrome (LGS) and other drug-resistant epilepsy (DRE) can impact behavior, communication, and quality of life (QoL). In collaboration with community engagement efforts with the Lennox-Gastaut Syndrome Foundation (LGSF), we aimed to gain an initial snapshot of patient and family perspectives and experiences with evaluation of behavior, communication, and QoL. METHODS: A cross-sectional survey was conducted to collect self-reported information from caregivers of children with LGS and other DRE regarding their perspectives and experiences with healthcare providers' evaluation of behavior, communication, and QoL. The survey tool was developed by the study investigators in partnership with the LGS Foundation and had diffused to caregivers online by epilepsy advocacy groups including the Pediatric Epilepsy Surgery Alliance (PESA). Responses were analyzed. Descriptive statistics were calculated. The survey asked for caregiver perspectives and assessed which instruments the caregivers had previously been given for measuring these domains. RESULTS: Responses from 245 caregivers were included, with 132 (54%) caregivers of an individual with LGS and 113 (46%) caregivers of an individual with non-LGS related DRE. Respondents reported that 66% of their loved ones had undergone epilepsy-related surgery. Over 90% agreed that measuring behavior, communication, and QoL was important, but fewer than half felt that their healthcare providers evaluated these domains well. LGS caregivers largely shared non-LGS caregivers' perspectives; however, they reported more frequently that communication was not evaluated enough. Barriers to measuring these domains included a lack of good surveys (developmentally appropriate and specific to the type of epilepsy) or not receiving any survey instruments for these domains during clinic appointments. Caregivers play a crucial role for individuals with DRE, and their input is essential in identifying challenges and needs. Caregivers believe that measuring behavior, communication, and quality of life is important, and most of them feel that their loved ones are not adequately evaluated during their healthcare encounters. There is a need for appropriately scaled survey instruments to measure areas of importance for patients and caregivers, as well as incorporation of these outcomes in the healthcare discussion.

Survival and neurological outcomes after stereotactic biopsy of diffuse intrinsic pontine glioma: a systematic review.

OBJECTIVE: Diffuse intrinsic pontine gliomas (DIPGs) are aggressive and malignant tumors of the brainstem. Stereotactic biopsy can obtain molecular and genetic information for diagnostic and potentially therapeutic purposes. However, there is no consensus on the safety of biopsy or effect on survival. The authors aimed to characterize neurological risk associated with and the effect of stereotactic biopsy on survival among patients with DIPGs. METHODS: A systematic review was performed in accordance with PRISMA guidelines to identify all studies examining pediatric patients with DIPG who underwent stereotactic biopsy. The search strategy was deployed in PubMed, Embase, and Scopus. The quality of studies was assessed using the Grading of Recommendations, Assessment, Development and Evaluation system, and risk of bias was evaluated with the Cochrane Risk of Bias in Nonrandomized Studies-of Interventions tool. Bibliographic, demographic, clinical, and outcome data were extracted from studies meeting inclusion criteria. RESULTS: Of 2634 resultant articles, 13 were included, representing 192 patients undergoing biopsy. The weighted mean age at diagnosis was 7.5 years (range 0.5-17 years). There was an overall neurosurgical complication rate of 13.02% (25/192). The most common neurosurgical complication was cranial nerve palsy (4.2%, 8/192), of which cranial nerve VII was the most common (37.5%, 3/8). The second most common complication was perioperative hemorrhage (3.6%, 7/192), followed by hemiparesis (2.1%, 4/192), speech disorders (1.6%, 3/192) such as dysarthria and dysphasia, and movement disorders (1.0%, 2/192). Hydrocephalus was less commonly reported (0.5%, 1/192), and there were no complications relating to wound infection/dehiscence (0%, 0/192) or CSF leak (0%, 0/192). No mortality was specifically attributed to biopsy. Diagnostic yield of biopsy revealed a weighted mean of 97.4% (range 91%-100%). Of the studies reporting survival data, 37.6% (32/85) of patients died within the study follow-up period (range 2 weeks-48 months). The mean overall survival in patients undergoing biopsy was 9.73 months (SD 0.68, median 10 months, range 6-13 months). CONCLUSIONS: Children with DIPGs undergoing biopsy have mild to moderate rates of neurosurgical complications and no excessive morbidity. With reasonably acceptable surgical risk and high diagnostic yield, stereotactic biopsy of DIPGs can allow for characterization of patient-specific molecular and genetic features that may influence prognosis and the development of future therapeutic strategies.

Pediatric intraspinal arachnoid cyst: successful endoscopic fenestration. Illustrative case.

BACKGROUND: Intradural spinal arachnoid cysts (SACs) are a rare cause of spinal cord compression. Treatment is centered on decompression of the spinal cord via laminectomy or laminoplasty followed by resection or fenestration of the cyst. Although laminectomy or laminoplasty access may be needed to achieve the desired result, either procedure can be associated with more extensive surgical dissections and long-term spinal stability concerns, including postsurgical kyphosis. OBSERVATIONS: The authors present a case of a cervical intradural SAC in a 4-month-old girl presenting with symptomatic compression. The patient was treated by laminotomy and endoscopic fenestration of the SAC with resolution of symptoms and no disease progression 10 months postoperatively, when the patient was 14 months old. LESSONS: Microsurgical endoscopic fenestration of an intradural SAC can provide a less invasive means of treatment while avoiding the risks associated with more invasive approaches.

Post-Surgical Cognitive Outcomes of Moyamoya Disease: A Systematic Review.

BACKGROUND: Moyamoya disease (MMD) is an occlusive arteriopathy leading to stroke. Progressive if left untreated, revascularization surgery has become the mainstay of treatment. Although clinical and radiographic outcomes of MMD after intervention are well-characterized, cognitive outcomes in pediatric patients remain unclear. We aimed to examine postoperative cognitive outcomes in children with MMD, examine factors associated with cognitive changes after intervention, and define the effect of revascularization surgery on cognitive outcomes. METHODS: A systematic review was conducted following PRISMA guidelines searching PubMed, Embase, and Scopus databases. Articles met inclusion criteria if they studied pediatric patients undergoing revascularization surgery for MMD and examined cognitive outcomes either qualitatively or quantitatively. All data extracted from included articles was examined descriptively. RESULTS: Of 1091 resultant articles, 12 articles containing 446 patients were included. Surgery was associated with maintained or improved full scale intellectual quotient (IQ), performance IQ, perceptual IQ, memory quotient and verbal memory. However, 70% of patients had impaired cognitive function, with associated poor school performance. Improvements in cognition were associated with increased cerebral blood flow, particularly to the middle cerebral artery, due to the development of collaterals. Female sex, shorter duration from symptom onset to surgery, and surgery after age 7 were predictive of cognitive improvement. Completed ischemic stroke prior to surgery was associated with poorer cognitive outcomes. CONCLUSIONS: Although children with MMD have improved cognitive outcomes following revascularization overall, a distinct subset experience cognitive impairment. Consideration of patient-specific and treatment-related factors is important to enable proper risk stratification and inform management approaches.

Cronobacter brain abscess and refractory epilepsy in a newborn: role of epilepsy surgery. Illustrative case.

BACKGROUND: Neonatal meningitis due to Cronobacter is associated with powdered infant formula. Prompt recognition of this rare but aggressive infection is critical. OBSERVATIONS: The authors report a unique case of neonatal Cronobacter meningoencephalitis complicated by brain abscess and status epilepticus, requiring surgical intervention in a preterm 4-week-old male and related to contaminated powdered infant formula. They discuss the medical and surgical management in this patient, as well as the role of epilepsy surgery in acute drug-resistant epilepsy. This is paired with a literature review examining Cronobacter infections in infants to provide a summative review of the existing literature. LESSONS: Cronobacter contamination in powdered infant formula and breast pumps is rare but can cause life-threatening infections. When evaluating patients with Cronobacter central nervous system infections, serial neuroimaging, infection control, and prompt surgical management are essential. Future studies are needed regarding the role of epilepsy surgery in the acute infectious period.

Correlating Thermodynamics with Cognitive Outcomes in Magnetic Resonance-guided Laser Interstitial Thermal Therapy (MRgLITT) of a Pediatric Hypothalamic Hamartoma.

OBJECTIVE: Magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) is a commonly used clinical method of destroying intracranial brain foci. Our objective was to correlate the thermal damage estimate transition zone with cognitive outcomes in MRgLITT of a pediatric hypothalamic hamartoma. METHODS: Uncomplicated MRgLITT was used to disconnect an 8-mm left Delalande grade II hypothalamic hamartoma (HH) revealed on neuroimaging of a 17-year-old male patient with drug-resistant epilepsy and a "gelastic +" semiology including both gelastic and tonic-clonic seizures. Despite meticulous planning, submillimetric stereotactic accuracy, and reassuring intraoperative thermography, the patient experienced transient, but profound, global amnesia. Retroactively, we applied a new iteration of thermographic software that overlays a magenta-colored transition zone (TZ) around the necrotic zone defined by the orange-pigmented thermal damage estimate (TDE). RESULTS: Clear involvement of the bilateral mesial circuits was demonstrated by the overlay of the TZ on the TDE. CONCLUSIONS: Involvement of the bilateral mesial circuits visualized with TDE and TZ could account for the neurocognitive outcomes of our patient. We highlight this case as our understanding of thermography analysis evolves, emphasizing principles of technique and trajectory planning, as well as considerations during thermablation to help inform surgical decision-making.

Thoracolumbar Scoliosis in Pediatric Patients With Loeys-Dietz Syndrome: A Case Series.

Background Loeys-Dietz syndrome (LDS) is a genetic connective tissue disorder that predominantly affects cardiovascular, skeletal, and craniofacial structures. Associated thoracolumbar scoliosis in LDS can be challenging to manage, though other etiologies of pediatric scoliosis have better-defined management guidelines. We examined our institutional experience regarding the treatment of pediatric patients with LDS and scoliosis. Methodology In this retrospective study, all patients seen at our pediatric tertiary care center from 2004 through 2018 with a diagnosis of LDS were reviewed, and those with radiographic diagnoses of scoliosis (full-length scoliosis X-rays) were included. Demographic, clinical, and radiographic parameters were collected, and management strategies were reported. Results A total of 39 LDS patients whose ages ranged between seven and 13 years were identified. A total of nine patients were radiographically diagnosed with scoliosis, but three patients were excluded due to incomplete medical records, leaving six patients. The median age at scoliosis diagnosis was 11.5 years, with a median follow-up of 51 months. Two patients were successfully managed with observation (average initial Cobb angle (CA): 14°, average final CA: 20.5°). Two were braced, one successfully (initial CA: 15°, final CA: 30°) and one with a progressive disease requiring surgery (initial CA: 40°, final CA: 58°). Of the two who were offered surgical correction, one underwent surgery with a durable correction of spinal deformity (CA: 33° to 19°). One patient underwent a recent correction of aortic root dilatation and was not a candidate for scoliosis surgery. Conclusions Principles of adolescent idiopathic scoliosis management such as bracing for CA of 20-50° and surgery for CA of >50° can be applied to LDS patients with good outcomes. This augments our understanding of the treatment algorithm for pediatric patients with LDS.

Endoscopic third ventriculostomy and biopsy of a tectal lesion using flexible neuroendoscopy and urological cup forceps: illustrative case.

BACKGROUND: Tectal region tumors often clinically present as obstructive hydrocephalus due to mass effect on the outflow of the third ventricle and cerebral aqueduct. Pathology in this region varies; thus, biopsy can be of great value in the management decision making. Appropriate instrumentation remains an area of interest to further advance flexible neuroendoscopic techniques and applications. OBSERVATIONS: The authors report an illustrative case using flexible neuroendoscopy through a single burr hole for simultaneous endoscopic third ventriculostomy (ETV) and tectal tumor biopsy using urological cup forceps in a 13-year-old boy who had presented with obstructive hydrocephalus. LESSONS: The authors demonstrate the feasibility of simultaneous ETV and tectal lesion biopsy via flexible neuroendoscopy to address obstructive hydrocephalus and obtain a tissue biopsy in a single-site procedure. They found that the use of flexible cup forceps designed for uroscopy is an important complement to flexible neuroendoscopy. Given the evolving applications of flexible neuroendoscopy, this has implications for instrumentation adaptation and future design.

Morning glory disc anomaly and its implications in moyamoya arteriopathy: a retrospective case series.

OBJECTIVE: Morning glory disc anomaly (MGDA), a congenital abnormality of the optic nerve, may be associated with moyamoya arteriopathy, a cerebrovascular abnormality. In this study, the authors aimed to define the temporal evolution of cerebrovascular arteriopathy in patients with MGDA to characterize a rational strategy for screening and management over time. METHODS: The records of pediatric neurosurgical patients at two academic institutions were retrospectively reviewed to identify cases of cerebral arteriopathy and MGDA, including radiographic and clinical records documenting patient outcomes of medical and surgical management. RESULTS: Thirteen cases of moyamoya syndrome (MMS) associated with MGDA were identified in 13 children aged 0.6-17 years. The pattern of arteriopathy resembled that of non-MGDA MMS, with predominantly anterior circulation involvement. The arteriopathy lateralized with the MGDA, although 3 patients also had contralateral involvement. The overall group was followed for a median of 3.2 years. Radiological biomarkers of cerebral ischemia were applied to guide surgical decisions, and more than half of the patients (7 of 13) had evidence of stroke or progression on serial imaging. Nine patients underwent revascularization surgery, and 4 were managed medically. CONCLUSIONS: Cerebral arteriopathy observed in association with MGDA resembles MMS seen in patients without MGDA and is dynamic, with progression observed over months to years and an associated risk of cerebral ischemia that indicates a role for surgical revascularization. Radiological biomarkers may augment clinical data to identify candidates for revascularization surgery.

Disparities in pediatric drug-resistant epilepsy care.

INTRODUCTION: Epilepsy affects millions of children worldwide, with 20-40% experiencing drug-resistant epilepsy (DRE) who are recommended for epilepsy surgery evaluation and may benefit from surgical management. However, many patients live with DRE for multiple years prior to surgical epilepsy referral or treatment or are never referred at all. OBJECTIVE: We aimed to describe factors associated with referral for epilepsy surgery in the USA, in order to identify disparities in DRE, characterize why they may exist, and recognize areas for improvement. METHODS: Pediatric patients diagnosed with DRE between January 1, 2004 and December 31, 2020 were identified from the Pediatric Health Information System (PHIS) Database. Patients treated with antiseizure medications (ASMs) only, ASMs plus vagus nerve stimulation (VNS), and ASMs plus cranial epilepsy surgery were studied regarding access to epilepsy surgery and disparities in care. This study used chi-square tests to determine associations between treatment time and preoperative factors. Preoperative factors studied included epilepsy treatment type, age, sex, race/ethnicity, insurance type, geographic region, patient type, epilepsy type, and presence of pediatric complex chronic conditions (PCCCs). RESULTS: A total of 18,292 patients were identified; 10,240 treated with ASMs, 5019 treated with ASMs + VNS, and 3033 treated with ASMs + cranial epilepsy surgery. Sex was not found to significantly vary among groups. There was significant variation in age, census region, race/ethnicity, patient type, presence of PCCCs, diagnosis, and insurance (p < 0.001). Those treated surgically, either with VNS or cranial epilepsy surgery, were 2 years older than those medically treated. Additionally, those medically treated were less likely to be living in the Midwest (25.46%), identified as non-Hispanic white (51.78%), have a focal/partial epilepsy diagnosis (8.74%), and be privately insured (35.82%). CONCLUSIONS: We studied a large administrative US database examining variables associated with surgical epilepsy evaluation and management. We found significant variation in treatment associated with age, US census region, race/ethnicity, patient type, presence of PCCCs, diagnosis, and health insurance type. We believe that these disparities in care are related to access and social determinants of health, and we encourage focused outreach strategies to mitigate these disparities to broaden access and improve outcomes in children in the USA with DRE.

Flexible neuroendoscopy for endoscopic third ventriculostomy and fourth ventricular arachnoid cyst fenestration in an infant.

Arachnoid cysts of the fourth ventricle are rarely reported. Management options include CSF diversion, cyst fenestration, or cyst excision. Fenestration can be done via open microsurgical technique or endoscopically with or without simultaneous third ventriculostomy; and both rigid and flexible endoscopy have been used successfully. However, application of this treatment modality in pediatric patients is not well described. Therefore, to their knowledge, the authors report the first successful treatment of a fourth ventricular arachnoid cyst with a single frontal burr hole entry point for third ventriculostomy and fourth ventricular arachnoid cyst fenestration performed using flexible neuroendoscopy. The patient was a 13-month-old boy presenting with progressive macrocephaly. The authors review their technique, discuss special considerations when using this approach, and include an annotated intraoperative video for demonstration to help instruct and guide management. The authors demonstrate with an example that a single frontal burr hole entry point for flexible endoscopic third ventriculostomy and navigation through a dilated cerebral aqueduct for fourth ventricular arachnoid cyst fenestration is a viable treatment for symptomatic fourth ventricular arachnoid cysts in children.

Pediatric Vagus Nerve Stimulation: Case Series Outcomes and Future Directions.

BACKGROUND: Vagus nerve stimulation (VNS) is a neuromodulatory procedure most extensively studied as an adjunct to medically refractory epilepsy. Despite widespread adoption and decades of clinical experience, clinical predictors of response to VNS remain unclear. OBJECTIVE: To evaluate a retrospective cohort of pediatric patients undergoing VNS at our institution to better understand who may benefit from VNS and identify factors which may predict response to VNS. METHODS: We conducted a retrospective cohort study examining pediatric patients undergoing VNS over nearly a 20-year span at a single institution. Presurgical evaluation, including demographics, clinical history, and diagnostic electroencephalogram, and imaging findings were examined. Primary outcomes included VNS response. RESULTS: Two hundred ninety-seven subjects were studied. The mean age at surgery was 10.1 (SD = 4.9, range = 0.8-25.3) years; length of follow-up was a mean of 4.6 years (SD = 3.5, median = 3.9 years, range 1 day-16.1 years). There was no association between demographic factors, epilepsy etiology, or genetic basis and VNS outcomes. There was an association between reduction in main seizure type with positive MRI finding. Of all MRI findings analyzed, brain atrophy was significantly associated with worse VNS outcomes, whereas dysplastic hippocampus and chronic periventricular leukomalacia findings were found to be associated with improved outcomes. Increased seizure semiology variability and seizure type were also associated with improved seizure outcomes. CONCLUSION: Predicting response to VNS remains difficult, leading to incompletely realized benefits and suboptimal resource utilization. Specific MRI findings and increased seizure semiology variability and type can help guide clinical decision making and patient counseling.

Germline genetic mutations in pediatric cerebrovascular anomalies: a multidisciplinary approach to screening, testing, and management.

OBJECTIVE: Genetic alterations are increasingly recognized as etiologic factors linked to the pathogenesis and development of cerebrovascular anomalies. Their identification allows for advanced screening and targeted therapeutic approaches. The authors aimed to describe the role of a collaborative approach to care and genetic testing in pediatric patients with neurovascular anomalies, with the objectives of identifying what genetic testing recommendations were made, the yield of genetic testing, and the implications for familial screening and management at present and in the future. METHODS: The authors performed a descriptive retrospective cohort study examining pediatric patients genetically screened through the Pediatric Neurovascular Program of a single treatment center. Patients 18 years of age and younger with neurovascular anomalies, diagnosed radiographically or histopathologically, were evaluated for germline genetic testing. Patient demographic data and germline genetic testing and recommendation, clinical, treatment, and outcome data were collected and analyzed. RESULTS: Sixty patients were included; 29 (47.5%) were female. The mean age at consultation was 11.0 ± 4.9 years. Diagnoses included cerebral arteriovenous malformations (AVMs) (n = 23), cerebral cavernous malformations (n = 19), non-neurofibromatosis/non-sickle cell moyamoya (n = 8), diffuse cerebral proliferative angiopathy, and megalencephaly-capillary malformation. Of the 56 patients recommended to have genetic testing, 40 completed it. Genetic alterations were found in 13 (23%) patients. Four patients with AVMs had RASA1, GDF2, and ACVRL1 mutations. Four patients with cavernous malformations had Krit1 mutations. One with moyamoya disease had an RNF213 mutation. Three patients with megalencephaly-capillary malformation had PIK3CA mutations, and 1 patient with a cavernous sinus lesion had an MED12 mutation. The majority of AVM patients were treated surgically. Patients with diffuse cerebral proliferative angiopathy were treated medically with sirolimus. At-risk relatives of 3 patients positive for genetic anomalies had also been tested. CONCLUSIONS: This study demonstrates a role for exploring genetic alterations in the identification and treatment of pediatric neurovascular disease pathogenesis. Germline genetic mutations were found in almost one-quarter of the patients screened in this study, results that helped to identify medically targeted treatment modalities for some pediatric neurovascular patients. Insight into the genetic etiology of vascular anomalies may provide broader clinical implications for risk assessment, family screening, follow-up surveillance, and medical management.

Vagus nerve stimulator revision in pediatric epilepsy patients: a technical note and case series.

INTRODUCTION: Vagus nerve stimulation (VNS) is an adjunctive treatment in children with intractable epilepsy. When lead replacement becomes necessary, the old leads are often truncated and retained and new leads are implanted at a newly exposed segment of the nerve. Direct lead removal and replacement are infrequently described, with outcomes poorly characterized. We aimed to describe our experience with feasibility of VNS lead removal and replacement in pediatric patients. METHODS: Retrospective review examined 14 patients, at a single, tertiary-care, children's hospital, who underwent surgery to replace VNS leads, with complete removal of the existing lead from the vagus nerve and placement of a new lead on the same segment of the vagus nerve, via blunt and sharp dissection without use of electrocautery. Preoperative characteristics, stimulation parameters, and outcomes were collected. RESULTS: Mean age at initial VNS placement was 7.6 years (SD 3.5, range 4.5-13.4). Most common etiologies of epilepsy were genetic (5, 36%) and cryptogenic (4, 29%). Lead replacement was performed at a mean of 6.0 years (SD 3.8, range 2.1-11.7) following initial VNS placement. Reasons for revision included VNS lead breakage or malfunction. There were no perioperative complications, including surgical site infection, voice changes, dysphagia, or new deficits postoperatively. Stimulation parameters after replacement surgery at last follow-up were similar compared to preoperatively, with final stimulation parameters ranging from 0.25 mA higher to 1.5 mA lower to maintain baseline seizure control. The mean length of follow-up was 7.9 years (SD 3.5, range 3.1-13.7). CONCLUSION: Removal and replacement of VNS leads are feasible and can be safely performed in children. Further characterization of surgical technique, associated risk, impact on stimulation parameters, and long-term outcomes are needed to inform best practices in VNS revision.

Firearm-Related Pediatric Head Trauma: A Scoping Review.

BACKGROUND: Firearm-related injury is a significant cause of morbidity and mortality in pediatric populations. Despite a disproportionate role in the most morbid outcomes in both traumatic brain injury and firearm-related injury populations, firearm-related traumatic brain injury (frTBI) is an understudied epidemiological entity. There is need to increase understanding and promote interventions that reduce this burden of disease. OBJECTIVE: To assess the evidence characterizing pediatric frTBI to highlight trends and gaps regarding burden of disease and interventions to reduce frTBI. METHODS: We conducted a scoping review under Preferred Reporting Items for Systematic Reviews and Meta-Analyses-Scoping Review (PRISMA-ScR) guidelines on peer-reviewed studies across 5 databases (Medline OVID, EMBASE, Web of Science Legal Collection, PsychINFO, and Academic Search Complete). English studies examining pediatric frTBI epidemiology, prevention, and/or social or legal policy advocacy were included. Articles were excluded if they more generally discussed pediatric firearm-related injury without specific analysis of frTBI. RESULTS: Six studies satisfied inclusion criteria after screening and full-text assessment. Limited studies specifically addressed the burden of disease caused by frTBI. There was an increased risk for both injury and death from frTBI in men, preteenage and teenage youths, minorities, and individuals in firearm-owning households. Further study is required to ascertain if suggested methods of targeted patient screening, firearm-injury prevention counseling, and advocacy of safety-oriented policy tangibly affect rates or outcomes of pediatric frTBI. CONCLUSION: By understanding published epidemiological data and areas of intervention shown to reduce frTBIs, neurosurgeons can become further engaged in public health and prevention rather than strictly treatment after injury.

Case Report: Dual Target Deep Brain Stimulation With Externalized Programming for Post-traumatic Complex Movement Disorder.

Introduction: Movement disorders can be common, persistent, and debilitating sequelae of severe traumatic brain injury. Post-traumatic movement disorders are usually complex in nature, involving multiple phenomenological manifestations, and can be difficult to control with medical management alone. Deep brain stimulation (DBS) has been used to treat these challenging cases, but distorted brain anatomy secondary to trauma can complicate effective targeting. In such cases, use of diffusion tractography imaging and inpatient testing with externalized DBS leads can be beneficial in optimizing outcomes. Case Description: We present the case of a 42-year-old man with severe, disabling post-traumatic tremor who underwent bilateral, dual target DBS to the globus pallidus internus (GPi) and a combined ventral intermediate nucleus of the thalamus (Vim)/dentato-rubro-thalamic tracts (DRTT) target. DRTT fiber tracts were reconstructed preoperatively to assist in surgical targeting given the patient's distorted anatomy. Externalization and survey of the four leads extra-operatively with inpatient testing allowed for internalization of the leads that demonstrated benefit. Six months after surgery, the patient's tremor and dystonic burden had decreased by 67% in the performance sub-score of The Essential Tremor Rating Scale (TETRAS). Conclusion: A patient-tailored approach including target selection guided by individualized anatomy and tractography as well as extra-operative externalized lead interrogation was shown to be effective in optimizing clinical outcome in a patient with refractory post-traumatic tremor.

Time-Out and Its Role in Neurosurgery.

BACKGROUND: Safety checklists have improved surgical outcomes; however, much of the literature comes from general surgery. OBJECTIVE: To identify the role of time-outs in neurosurgery, understand neurosurgeons' attitudes toward time-out, and highlight areas for improvement. METHODS: A cross-sectional study using a 15-item survey to evaluate how time-outs were performed across 5 hospitals affiliated with a single neurosurgery training program. RESULTS: Surveys were sent to 51 neurosurgical faculty, fellows, and residents across 5 hospitals with a 72.5% response rate. At all hospitals, surgeons, anesthesiologists, registered nurses, and circulators were involved in time-outs. Although all required time-out before incision, there was no consensus regarding the precise timing of time-out, in policy or in practice. Overall, respondents believed the existing time-out was adequate for neurosurgical procedures (H1: 17, 65.4%; H2: 19, 86.4%; H3: 14, 70.0%; H4: 20, 80.0%; and H5: 18, 78.3%). Of the respondents, 97.2% believed time-out made surgery safe, 94.6% agreed that time-outs reduce the risk of wrong-side or wrong-level neurosurgery, and 17 (45.9%) saw a role for a neurosurgery-specific safety checklist. Pragmatic challenges (n = 20, 54.1%) and individual beliefs and attitudes (n = 20, 54.1%) were common barriers to implementation of standardized time-outs. CONCLUSION: Multidisciplinary time-outs have become standard of care in neurosurgery. Despite proximity and overlapping personnel, there is considerable variability between hospitals in the practice of time-outs. This lack of uniformity, allowed for by flexible World Health Organization guidelines, may reflect the origins of surgical time-outs in general surgery, rather than neurosurgery, underscoring the potential for time-out optimization with neurosurgery-specific considerations.