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Background: Most pediatric hospitals manage patients who require external ventricular drains (EVDs) exclusively within pediatric intensive care units (PICUs) because of institutional protocols. Our institution commonly manages patients with EVDs on the neurotrauma floor (NTF). We evaluated whether this practice results in more EVD-associated complications., Methods: A retrospective cohort study at our Level 1 pediatric trauma center identified all trauma patients ≤18 years old who received an EVD in 2018-2023. Demographics, presenting characteristics, in-hospital management, and EVD management details were recorded. The primary outcome was EVD-related complication events., Results: Of the 81 patients who had EVDs placed after neurotrauma, 45 had their EVD managed exclusively in the PICU (PICU-EVD) and 36 had their EVD for some time while on the NTF (NTF-EVD). The groups were similar in sex (p = 0.87) and age (p = 0.054). PICU-EVD patients underwent fewer neurosurgeries (55.6% vs. 77.8%, p = 0.04) but spent more time on ventilators (10.6 ± 8.7 days vs. 6.4 ± 4.8, p = 0.02) and in the PICU (11.8 ± 9.0 days vs. 8.4 ± 5.9, p = 0.02). Total hospital stay was similar between groups (p = 0.44). NTF-EVD patients were on the drain longer (9.0 ± 7.4 days vs. 13.1 ± 9.1, p = 0.03), including 5.9 days on the NTF. Four EVD-related complications occurred overall: 2 accidental dislodgements and 2 cerebrospinal fluid leaks. EVD complication rates were similar on the NTF and PICU (2.2% vs. 8.3%, p = 0.21). All complications occurred late in the hospital course and were minor. A Poisson regression model comparing complication rates between PICU-only and NTF management (433 vs. 441 catheter days, respectively) found a complication rate of 6.8 per 1000 catheter days in the NTF group versus 2.3 per 1000 catheter days in the PICU-only group, yielding a rate ratio of 2.95 (95% confidence interval 0.29-30.4, p = 0.35). However, this difference was not statistically significant., Conclusion: Our center routinely discharges patients from the PICU to the NTF with EVDs in place. This practice may be associated with no increased risk or rate of EVD-related complications compared to PICU-only management., Level of Evidence: IV., Competing Interests: Conflict of interest Matthew C. Findlay owns equity in SoundPass Medical, LLC., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Responsive neurostimulation (RNS) is used off-label in pediatric patients with drug-resistant epilepsy (DRE). Our study aims to assess the safety and efficacy of RNS in pediatric and young adult patients with focal, multifocal, and generalized DRE., Methods: All patients who underwent RNS implantation at Primary Children's Hospital in Salt Lake City, UT, between December 2017 and 2022., Results: A total of 47 patients were retrospectively identified, of which 32 patients were included in the final analysis. Patients ranged in age from five to 21 years (pediatric n = 22, young adult n = 10) at the time of RNS implantation with focal (20 [63%]), multifocal (8 [25%]), and generalized (4 [12%]) DRE. Operative complications (3 [9%]) and negative side effects (6 [19%]) were minor. At the time of most recent clinic visit (mean 18.6 months, S.D. 13.9), 19 of 32 patients (59%) were responders with ≥50% reduction in seizure frequency (pediatric n = 14, young adult n = 5). The rate of responders increased with prolonged activation of RNS stimulation, reaching 71% (five of seven patients) after 24 months. Antiseizure medication was reduced in five (16%) patients, and seizure rescue medication usage was reduced in 10 (31%) patients. Quality of life improved in 15 (47%) patients., Conclusions: RNS implantation resulted in a sustained reduction in seizure frequency with minimal side effects in a majority of patients. Taken together, our data suggest that RNS is an effective and safe treatment option for focal, multifocal, and potentially generalized DRE in the pediatric and young adult population., Competing Interests: Declaration of competing interest A.S.K. is supported by the Pediatric Epilepsy Research Foundation Elterman Research Grant. None of the other authors has any conflict of interest to disclose. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. This study was approved by the institutional review board of the University of Utah and the privacy board of Intermountain Healthcare (IH), IRB 00150886. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors., (Published by Elsevier Inc.)

Introduction: The fibrous posterior atlanto-occipital membrane (PAOM) at the craniocervical junction is typically removed during decompression surgery for Chiari malformation type I (CM-I); however, its importance and ultrastructural architecture have not been investigated in children. We hypothesized that there are structural differences in the PAOM of patients with CM-I and those without., Methods: In this prospective study, blinded pathological analysis was performed on PAOM specimens from children who had surgery for CM-I and children who had surgery for posterior fossa tumors (controls). Clinical and radiographic data were collected. Statistical analysis included comparisons between the CM-I and control cohorts and correlations with imaging measures., Results: A total of 35 children (mean age at surgery 10.7 years; 94.3% white) with viable specimens for evaluation were enrolled: 24 with CM-I and 11 controls. There were no statistical demographic differences between the two cohorts. Four children had a family history of CM-I and five had a syndromic condition. The cohorts had similar measurements of tonsillar descent, syringomyelia, basion to C2, and condylar-to-C2 vertical axis (all p>0.05). The clival-axial angle was lower in patients with CM-I (138.1 vs. 149.3 degrees, p = 0.016). Morphologically, the PAOM demonstrated statistically higher proportions of disorganized architecture in patients with CM-I (75.0% vs. 36.4%, p = 0.012). There were no differences in PAOM fat, elastin, or collagen percentages overall and no differences in imaging or ultrastructural findings between male and female patients. Posterior fossa volume was lower in children with CM-I (163,234 mm3 vs. 218,305 mm3, p<0.001), a difference that persisted after normalizing for patient height (129.9 vs. 160.9, p = 0.028)., Conclusions: In patients with CM-I, the PAOM demonstrates disorganized architecture compared with that of control patients. This likely represents an anatomic adaptation in the presence of CM-I rather than a pathologic contribution., Competing Interests: The authors have declared that no competing interests exist., (Copyright: This is an open access article, free of all copyright, and may be freely reproduced, distributed, transmitted, modified, built upon, or otherwise used by anyone for any lawful purpose. The work is made available under the Creative Commons CC0 public domain dedication.)

Objectives: Although hemispheric surgeries are among the most effective procedures for drug-resistant epilepsy (DRE) in the pediatric population, there is a large variability in seizure outcomes at the group level. A recently developed HOPS score provides individualized estimation of likelihood of seizure freedom to complement clinical judgement. The objective of this study was to develop a freely accessible online calculator that accurately predicts the probability of seizure freedom for any patient at 1-, 2-, and 5-years post-hemispherectomy., Methods: Retrospective data of all pediatric patients with DRE and seizure outcome data from the original Hemispherectomy Outcome Prediction Scale (HOPS) study were included. The primary outcome of interest was time-to-seizure recurrence. A multivariate Cox proportional-hazards regression model was developed to predict the likelihood of post-hemispheric surgery seizure freedom at three time points (1-, 2- and 5- years) based on a combination of variables identified by clinical judgment and inferential statistics predictive of the primary outcome. The final model from this study was encoded in a publicly accessible online calculator on the International Network for Epilepsy Surgery and Treatment (iNEST) website (https://hops-calculator.com/)., Results: The selected variables for inclusion in the final model included the five original HOPS variables (age at seizure onset, etiologic substrate, seizure semiology, prior non-hemispheric resective surgery, and contralateral fluorodeoxyglucose-positron emission tomography [FDG-PET] hypometabolism) and three additional variables (age at surgery, history of infantile spasms, and magnetic resonance imaging [MRI] lesion). Predictors of shorter time-to-seizure recurrence included younger age at seizure onset, prior resective surgery, generalized seizure semiology, FDG-PET hypometabolism contralateral to the side of surgery, contralateral MRI lesion, non-lesional MRI, non-stroke etiologies, and a history of infantile spasms. The area under the curve (AUC) of the final model was 73.0%., Significance: Online calculators are useful, cost-free tools that can assist physicians in risk estimation and inform joint decision-making processes with patients and families, potentially leading to greater satisfaction. Although the HOPS data was validated in the original analysis, the authors encourage external validation of this new calculator., (© 2023 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Objective: Lennox-Gastaut syndrome (LGS) is a severe form of epileptic encephalopathy, presenting during the first years of life, and is very resistant to treatment. Once medical therapy has failed, palliative surgeries such as vagus nerve stimulation (VNS) or corpus callosotomy (CC) are considered. Although CC is more effective than VNS as the primary neurosurgical treatment for LGS-associated drop attacks, there are limited data regarding the added value of CC following VNS. This study aimed to assess the effectiveness of CC preceded by VNS., Methods: This multinational, multicenter retrospective study focuses on LGS children who underwent CC before the age of 18 years, following prior VNS, which failed to achieve satisfactory seizure control. Collected data included epilepsy characteristics, surgical details, epilepsy outcomes, and complications. The primary outcome of this study was a 50% reduction in drop attacks., Results: A total of 127 cases were reviewed (80 males). The median age at epilepsy onset was 6 months (interquartile range [IQR] = 3.12-22.75). The median age at VNS surgery was 7 years (IQR = 4-10), and CC was performed at a median age of 11 years (IQR = 8.76-15). The dominant seizure type was drop attacks (tonic or atonic) in 102 patients. Eighty-six patients underwent a single-stage complete CC, and 41 an anterior callosotomy. Ten patients who did not initially have a complete CC underwent a second surgery for completion of CC due to seizure persistence. Overall, there was at least a 50% reduction in drop attacks and other seizures in 83% and 60%, respectively. Permanent morbidity occurred in 1.5%, with no mortality., Significance: CC is vital in seizure control in children with LGS in whom VNS has failed. Surgical risks are low. A complete CC has a tendency toward better effectiveness than anterior CC for some seizure types., (© 2023 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Objective: MR-guided laser interstitial thermal therapy (MRgLITT) is associated with lower seizure-free outcome but better safety profile compared to open surgery. However, the predictors of seizure freedom following MRgLITT remain uncertain. This study aimed to use machine learning to predict seizure-free outcome following MRgLITT and to identify important predictors of seizure freedom in children with drug-resistant epilepsy., Methods: This multicenter study included children treated with MRgLITT for drug-resistant epilepsy at 13 epilepsy centers. The authors used clinical data, diagnostic investigations, and ablation features to predict seizure-free outcome at 1 year post-MRgLITT. Patients from 12 centers formed the training cohort, and patients in the remaining center formed the testing cohort. Five machine learning algorithms were developed on the training data by using 10-fold cross-validation, and model performance was measured on the testing cohort. The models were developed and tested on the complete feature set. Subsequently, 3 feature selection methods were used to identify important predictors. The authors then assessed performance of the parsimonious models based on these important variables., Results: This study included 268 patients who underwent MRgLITT, of whom 44.4% had achieved seizure freedom at 1 year post-MRgLITT. A gradient-boosting machine algorithm using the complete feature set yielded the highest area under the curve (AUC) on the testing set (AUC 0.67 [95% CI 0.50-0.82], sensitivity 0.71 [95% CI 0.47-0.88], and specificity 0.66 [95% CI 0.50-0.81]). Logistic regression, random forest, support vector machine, and neural network yielded lower AUCs (0.58-0.63) compared to the gradient-boosting machine but the findings were not statistically significant (all p > 0.05). The 3 feature selection methods identified video-EEG concordance, lesion size, preoperative seizure frequency, and number of antiseizure medications as good prognostic features for predicting seizure freedom. The parsimonious models based on important features identified by univariate feature selection slightly improved model performance compared to the complete feature set., Conclusions: Understanding the predictors of seizure freedom after MRgLITT will assist with prognostication.

Blunt cerebrovascular injury (BCVI) is defined as blunt trauma to the head and neck leading to damage to the vertebral and/or carotid arteries; debate exists regarding which children are considered at high risk for BCVI and in need of angiographic/vessel imaging. We previously proposed a screening tool, the McGovern score, to identify pediatric trauma patients at high risk for BCVI, and we aim to validate the McGovern score by pooling data from multiple pediatric trauma centers. This is a multi-center, hospital-based, cohort study from all prospectively registered pediatric (<16 years of age) trauma patients who presented to the emergency department (ED) between 2003 and 2017 at six Level 1 pediatric trauma centers. The registry was retrospectively queried for patients who received a computed tomography angiogram (CTA) as a screening method for BCVI. Age, length of follow-up, mechanism of injury (MOI), arrival Glasgow Coma Scale (GCS) score, and focal neurological deficit were recorded. Radiological variables queried were the presence of a carotid canal fracture, petrous temporal bone fracture, and CT presence of infarction. Patients with BCVI were queried for mode of treatment, type of intracranial injury, artery damaged, and BCVI injury grade. The McGovern score was calculated for all patients who underwent CTA across all data groups. A total of 1012 patients underwent CTA; 72 of these patients were found to have BCVI, 51 of which were in the validation cohort. Across all data groups, the McGovern score has a >80% sensitivity (SN) and >98% negative predictive value (NPV). The McGovern score for pediatric BCVI is an effective, generalizable screening tool.

Objective: The authors of this study evaluated the safety and efficacy of stereotactic laser ablation (SLA) for the treatment of drug-resistant epilepsy (DRE) in children., Methods: Seventeen North American centers were enrolled in the study. Data for pediatric patients with DRE who had been treated with SLA between 2008 and 2018 were retrospectively reviewed., Results: A total of 225 patients, mean age 12.8 ± 5.8 years, were identified. Target-of-interest (TOI) locations included extratemporal (44.4%), temporal neocortical (8.4%), mesiotemporal (23.1%), hypothalamic (14.2%), and callosal (9.8%). Visualase and NeuroBlate SLA systems were used in 199 and 26 cases, respectively. Procedure goals included ablation (149 cases), disconnection (63), or both (13). The mean follow-up was 27 ± 20.4 months. Improvement in targeted seizure type (TST) was seen in 179 (84.0%) patients. Engel classification was reported for 167 (74.2%) patients; excluding the palliative cases, 74 (49.7%), 35 (23.5%), 10 (6.7%), and 30 (20.1%) patients had Engel class I, II, III, and IV outcomes, respectively. For patients with a follow-up ≥ 12 months, 25 (51.0%), 18 (36.7%), 3 (6.1%), and 3 (6.1%) had Engel class I, II, III, and IV outcomes, respectively. Patients with a history of pre-SLA surgery related to the TOI, a pathology of malformation of cortical development, and 2+ trajectories per TOI were more likely to experience no improvement in seizure frequency and/or to have an unfavorable outcome. A greater number of smaller thermal lesions was associated with greater improvement in TST. Thirty (13.3%) patients experienced 51 short-term complications including malpositioned catheter (3 cases), intracranial hemorrhage (2), transient neurological deficit (19), permanent neurological deficit (3), symptomatic perilesional edema (6), hydrocephalus (1), CSF leakage (1), wound infection (2), unplanned ICU stay (5), and unplanned 30-day readmission (9). The relative incidence of complications was higher in the hypothalamic target location. Target volume, number of laser trajectories, number or size of thermal lesions, or use of perioperative steroids did not have a significant effect on short-term complications., Conclusions: SLA appears to be an effective and well-tolerated treatment option for children with DRE. Large-volume prospective studies are needed to better understand the indications for treatment and demonstrate the long-term efficacy of SLA in this population.

Objective: Intrathecal baclofen (ITB) therapy is an effective treatment for spasticity and dystonia in children with cerebral palsy (CP). However, ITB pump surgery is associated with one of the highest rates of surgical site infection (SSI) in medicine, leading to significant morbidity and expense. Surgical protocols have reduced the rate of SSI in children with other CNS implants, and single-center protocols have been effective in ITB surgery in pediatrics. The authors describe the first multicenter quality improvement (QI)-driven standardized protocol for ITB pump surgery in children with CP across the Cerebral Palsy Research Network (CPRN), implemented with the aim of reducing ITB-associated SSI., Methods: SSI was defined as a culture-positive infection, ITB pump system removal for suspected infection, or wound dehiscence with exposed hardware. Each center reported historical infection rates for at least 3 years before initiating the SSI protocol (preintervention phase). After initiation of a 13-step surgical protocol, a consecutive series of 130 patients undergoing 149 surgical procedures for ITB at four CPRN tertiary pediatric neurosurgery centers were prospectively enrolled at surgery during a 2-year study period (intervention phase). QI methodology was used, including development of a key driver diagram and tracking performance using run and control charts. The primary process measure goal was documented compliance with 80% of the protocol steps, and the primary outcome measure goal was a 20% reduction in 90-day infection rate. Patient characteristics were collected from the CPRN Research Electronic Data Capture registry, including age at surgery, BMI, Gross Motor Function Classification System level, and pattern of spasticity., Results: The aggregated preintervention 90-day ITB SSI rate was 4.9% (223 procedures) between 2014 and 2017. During the intervention phase, 136 of 149 ITB surgeries performed met inclusion criteria for analysis. The mean documented compliance rate with protocol steps was 75%, and the 90-day infection rate was 4.4%, with an average of 42 days from index surgery to infection., Conclusions: This is the first multicenter QI initiative designed to reduce SSI in ITB surgery in children with CP. Ongoing enrollment and expansion of the protocol to other CPRN centers will facilitate identification of patient- and procedure-specific risk factors for SSI, and iterative plan-do-study-act cycles incorporating these data will further decrease the risk of SSI for ITB surgery in children.

Background: There are limited data on the association between transport distance and outcomes in pediatric patients with severe traumatic brain injuries (sTBIs), despite children having to travel further to pediatric trauma centers (PTCs)., Objective: To assess whether distance from a PTC is associated with outcomes in children who undergo cranial surgery after sTBI., Methods: Children with sTBI who underwent craniectomy/craniotomy at our PTC between 2010 and 2019 were identified retrospectively. Of these 92 patients, 83 sustained blunt injury and underwent surgery within 24 hours. The distance from injury location to PTC was based on injury zip code and calculated as Euclidean distance. Variables associated with transport, including distance, time, and rural-urban disparity, were analyzed for correlation with poor outcome., Results: Of the 83 patients identified, 81 had injury location information. Forty patients were injured within 30 miles and 41 were injured ≥30 miles from the PTC. Injury severity and pediatric trauma scores were not significantly different between groups. Sixty-eight children (82%) had a satisfactory outcome and 10 children (12%) died. There was a nonsignificant association between distance traveled and poor outcome, even when the cohort was stratified into those with subdural hematomas and those with nonabusive injuries., Conclusions: Regardless of the distance from the PTC at which their injury occurred, most children in this cohort made a moderate to good recovery. Children injured at greater distances from the PTC did not have worse outcomes; however, studies with larger cohorts are needed to more definitively assess prehospital pediatric transport systems in this population., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Patients undergoing surgical intervention for epilepsy mapping are typically administered opioids for pain control. The use of opioids is demonstrably lower after other procedures when a minimally invasive surgery (MIS) technique is used. Our objective was to determine whether using MIS for stereoelectroencephalography (SEEG) resulted in lower opioid requirement by pediatric patients when compared with subdural grid placement after craniotomy (ECoG). A retrospective chart review was conducted to identify patients < 18 years who underwent epilepsy mapping surgery using SEEG or ECoG in 2015-2019. The hospital stay was divided into four time periods, and the total amounts of opioids (converted into morphine milligram equivalents (MMEs)) and nonsteroidal anti-inflammatory drugs (NSAIDs) and pain scores (on numerical rating scale (NRS)) were calculated for each time interval. The two groups were then compared statistically. The study included 31 patients in the SEEG group and 9 in the ECoG group. The SEEG group consumed significantly fewer opioids during the hospital stay than the ECoG group (23.6 vs. 61.7 MMEs; p = 0.041). There were also significant differences in the length of stay (6.9 vs. 12.2 days; p = 0.002), rate of complications (0% vs. 20%; p = 0.006), and total NSAIDs consumed (3,264.8 vs. 12,730.2 mg; p = 0.002). Opioid and NSAID consumption were significantly lower and hospital stays were shorter in pediatric patients who underwent epilepsy mapping via SEEG compared with ECoG. These results suggest that MIS for epilepsy mapping may decrease the overall pain medication use and expedite patient discharge., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Background: Treatment of pilocytic astrocytomas (PAs) in children can be challenging when they arise in deep midline structures because complete surgical resection may result in significant neurological injury. Laser interstitial thermal therapy (LITT) has provided an alternative treatment modality for lesions that may not be amenable to resection. However, many patients with PAs may be symptomatic from a compressive cyst associated with the PA, and LITT does not obviate the need for cystic decompression in these patients., Observations: A 12-year-old male presented with left-sided weakness. Magnetic resonance imaging (MRI) revealed an enhancing mass with a large cyst involving the right thalamus and basal ganglia. The patient underwent a reservoir placement for cyst drainage and biopsy of the mass, revealing a pilocytic astrocytoma. He then underwent LITT followed by adjuvant chemotherapy. Sixteen months after LITT, follow-up MRI of the brain revealed no tumor growth., Lessons: This is the first case to describe reservoir placement to treat the cystic portion of a pilocytic astrocytoma followed by LITT and targeted chemotherapy. Reservoir placement reduced the cyst's mass effect and resolved the patient's symptoms, allowing for treatment options beyond resection.

Case: A 7-week-old girl presented with a recurrent primitive myxoid mesenchymal tumor of infancy requiring extensive resection of lower back musculature, L3-S2 vertebral bodies, and left L5 nerve root. Reconstruction consisted of transverse rectus abdominis muscle (TRAM) flow-through to free fibular flap to reconstruct the bony defect and fill the soft-tissue void. One-year postoperative imaging revealed a well-incorporated fibula graft. At the 30-month follow-up, the patient can bear weight on the autograft while sitting upright, allowing for efficient ambulation with a wheelchair., Conclusion: TRAM flow-through to free fibular flap is an efficacious reconstructive method for a multilevel vertebral spinal defect in a pediatric patient., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B808)., (Copyright © 2022 by The Journal of Bone and Joint Surgery, Incorporated.)

Objective: Multiple studies have evaluated the use of MRI for prognostication in pediatric patients with severe traumatic brain injury (TBI) and have found a correlation between diffuse axonal injury (DAI)-type lesions and outcome. However, there remains a limited understanding about the use of MRI for prognostication after severe TBI in children who have undergone cranial surgery., Methods: Children with severe TBI who underwent craniectomy or craniotomy at Primary Children's Hospital in Salt Lake City, Utah, between 2010 and 2019 were identified retrospectively. Of these 92 patients, 43 underwent postoperative brain MRI within 4 months of surgery. Susceptibility-weighted imaging (SWI) and FLAIR sequences were used to designate areas of hemorrhagic and nonhemorrhagic cerebral lesions related to DAI. Patients were then stratified based on the location of the DAI as read by a neuroradiologist as superficial, deep, or brainstem. The location of the DAI and other variables associated with poor outcome, including Glasgow Coma Scale (GCS) score, pediatric trauma score, mechanism of injury, and time to surgery, were analyzed for correlation with poor outcome. Outcomes were reported using the King's Outcome Scale for Childhood Head Injury (KOSCHI)., Results: In the 43 children with severe TBI who underwent postoperative brain MRI, the median GCS score on arrival was 4. The most common cause of injury was falls (14 patients, 33%). The most common primary intracranial pathology was subdural hematoma in 26 patients (60%), followed by epidural hematoma in 9 (21%). Fifteen patients (35%) had cerebral herniation and 31 (72%) had evidence of contusion. Variables associated with poor outcome included cerebral herniation (r = 0.338, p = 0.027) and location of DAI (r = 0.319, p = 0.037). In a separate analysis, brainstem DAI was shown to predict poor outcome, whereas location (no, superficial, or deep DAI) did not. Logistic regression showed that brainstem DAI (OR 22.3, p = 0.020) had a higher odds ratio than cerebral herniation (OR 10.5, p = 0.044) for poor outcome. Thirty-six children (84%) had a satisfactory outcome at last follow-up; 3 (7%) children died., Conclusions: The majority of children in this series who presented with a severe TBI and underwent craniectomy or craniotomy made a satisfactory recovery. In patients in whom there is a concern for poor outcome, the location of DAI-type lesions with SWI and FLAIR may assist in prognostication. The authors' results revealed that DAI-type lesions in the brainstem and evidence of cerebral herniation may indicate a poorer prognosis; however, more studies with larger cohorts are needed to make definitive conclusions.

Objective: Advances in prenatal imaging have facilitated improvements in the fetal diagnosis of congenital anomalies. Asymmetric ventriculomegaly, interhemispheric cyst, and dysgenesis of the corpus callosum (AVID) is a constellation of congenital anomalies reported in fetal imaging. However, few data are available regarding postnatal outcomes of infants and children with a fetal diagnosis of AVID. The authors sought to report the neurodevelopmental outcomes of patients diagnosed with AVID before birth at a single institution., Methods: An institutional fetal imaging database was queried to identify cases with ventriculomegaly, interhemispheric cyst, and dysgenesis of the corpus callosum over a 10-year study period from 2000 to 2019. Overall, 41 maternal-infant dyads who met imaging criteria for AVID were identified; medical records were reviewed for prenatal variables including gestational age at birth, perinatal complications including fetal demise, and postnatal variables including demographics, mortality, hydrocephalus diagnosis and management, epilepsy, and neurodevelopmental outcomes at 2 years or the last follow-up., Results: Among 41 patients, 25 (61%) were male. A slight majority of patients (55%) were born before 36 weeks of gestational age, and 27 patients (68%) were delivered via cesarean section because their head size precluded vaginal delivery. There were 8 incidences of fetal demise, 1 pregnancy was terminated, and 32 patients were born alive. Neonatal or early infant death occurred in 5 patients. Two children died during follow-up after the neonatal period (ages 7 months and 7 years). Twenty-six children survived to at least the 2-year follow-up, all of whom required treatment for hydrocephalus. Of those 26 children, 12 (46%) had a diagnosis of epilepsy, 14 (54%) could sit independently, 4 (16%) were in mainstream school, 16 (62%) had expressive language, and 7 (28%) had near-normal development without seizures., Conclusions: Among 41 maternal-fetal dyads with AVID, a majority of children survived to the 2-year follow-up, although all developed hydrocephalus. Many continued to have seizures, but expressive language use, attendance at mainstream school, and near-normal development without seizures were not infrequent. These data are critical for prenatal counseling and to establish the natural history of a diagnosis with limited outcome data.

Background: Despite the well-documented utility of responsive neurostimulation (RNS, NeuroPace) in adult epilepsy patients, literature on the use of RNS in children is limited., Objective: To determine the real-world efficacy and safety of RNS in pediatric epilepsy patients., Methods: Patients with childhood-onset drug-resistant epilepsy treated with RNS were retrospectively identified at 5 pediatric centers. Reduction of disabling seizures and complications were evaluated for children (<18 yr) and young adults (>18 yr) and compared with prior literature pertaining to adult patients., Results: Of 35 patients identified, 17 were <18 yr at the time of RNS implantation, including a 3-yr-old patient. Four patients (11%) had concurrent resection. Three complications, requiring additional surgical interventions, were noted in young adults (2 infections [6%] and 1 lead fracture [3%]). No complications were noted in children. Among the 32 patients with continued therapy, 2 (6%) achieved seizure freedom, 4 (13%) achieved ≥90% seizure reduction, 13 (41%) had ≥50% reduction, 8 (25%) had <50% reduction, and 5 (16%) experienced no improvement. The average follow-up duration was 1.7 yr (median 1.8 yr, range 0.3-4.8 yr). There was no statistically significant difference for seizure reduction and complications between children and young adults in our cohort or between our cohort and the adult literature., Conclusion: These preliminary data suggest that RNS is well tolerated and an effective off-label surgical treatment of drug-resistant epilepsy in carefully selected pediatric patients as young as 3 yr of age. Data regarding long-term efficacy and safety in children will be critical to optimize patient selection., (© Congress of Neurological Surgeons 2021.)

Objective: This study was undertaken to determine whether the vertical parasagittal approach or the lateral peri-insular/peri-Sylvian approach to hemispheric surgery is the superior technique in achieving long-term seizure freedom., Methods: We conducted a post hoc subgroup analysis of the HOPS (Hemispheric Surgery Outcome Prediction Scale) study, an international, multicenter, retrospective cohort study that identified predictors of seizure freedom through logistic regression modeling. Only patients undergoing vertical parasagittal, lateral peri-insular/peri-Sylvian, or lateral trans-Sylvian hemispherotomy were included in this post hoc analysis. Differences in seizure freedom rates were assessed using a time-to-event method and calculated using the Kaplan-Meier survival method., Results: Data for 672 participants across 23 centers were collected on the specific hemispherotomy approach. Of these, 72 (10.7%) underwent vertical parasagittal hemispherotomy and 600 (89.3%) underwent lateral peri-insular/peri-Sylvian or trans-Sylvian hemispherotomy. Seizure freedom was obtained in 62.4% (95% confidence interval [CI] = 53.5%-70.2%) of the entire cohort at 10-year follow-up. Seizure freedom was 88.8% (95% CI = 78.9%-94.3%) at 1-year follow-up and persisted at 85.5% (95% CI = 74.7%-92.0%) across 5- and 10-year follow-up in the vertical subgroup. In contrast, seizure freedom decreased from 89.2% (95% CI = 86.3%-91.5%) at 1-year to 72.1% (95% CI = 66.9%-76.7%) at 5-year to 57.2% (95% CI = 46.6%-66.4%) at 10-year follow-up for the lateral subgroup. Log-rank test found that vertical hemispherotomy was associated with durable seizure-free progression compared to the lateral approach (p = .01). Patients undergoing the lateral hemispherotomy technique had a shorter time-to-seizure recurrence (hazard ratio = 2.56, 95% CI = 1.08-6.04, p = .03) and increased seizure recurrence odds (odds ratio = 3.67, 95% CI = 1.05-12.86, p = .04) compared to those undergoing the vertical hemispherotomy technique., Significance: This pilot study demonstrated more durable seizure freedom of the vertical technique compared to lateral hemispherotomy techniques. Further studies, such as prospective expertise-based observational studies or a randomized clinical trial, are required to determine whether a vertical approach to hemispheric surgery provides superior long-term seizure outcomes., (© 2021 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.)

Objective: Widespread use of modern neuroimaging has led to a surge in diagnosing pediatric brain incidentalomas. Thalamic lesions have unique characteristics such as deep location, surgical complexity, and proximity to eloquent neuronal structures. Currently, the natural course of incidental thalamic lesions is unknown. Therefore, the authors present their experience in treating such lesions., Methods: A retrospective, international multicenter study was carried out in 8 tertiary pediatric centers from 5 countries. Patients were included if they had an incidental thalamic lesion suspected of being a tumor and were diagnosed before the age of 20 years. Treatment strategy, imaging characteristics, pathology, and the outcome of operated and unoperated cases were analyzed., Results: Overall, 58 children (23 females and 35 males) with a mean age of 10.8 ± 4.0 years were included. The two most common indications for imaging were nonspecific reasons (n = 19; e.g., research and developmental delay) and headache unrelated to small thalamic lesions (n = 14). Eleven patients (19%) underwent early surgery and 47 were followed, of whom 10 underwent surgery due to radiological changes at a mean of 11.4 ± 9.5 months after diagnosis. Of the 21 patients who underwent surgery, 9 patients underwent resection and 12 underwent biopsy. The two most frequent pathologies were pilocytic astrocytoma and WHO grade II astrocytoma (n = 6 and n = 5, respectively). Three lesions were high-grade gliomas., Conclusions: The results of this study indicate that pediatric incidental thalamic lesions include both low- and high-grade tumors. Close and long-term radiological follow-up is warranted in patients who do not undergo immediate surgery, as tumor progression may occur.

Objective: Pediatric stereoelectroencephalography (SEEG) has been increasingly performed in the United States, with published literature being limited primarily to large single-center case series. The purpose of this study was to evaluate the experience of pediatric epilepsy centers, where the technique has been adopted in the last several years, via a multicenter case series studying patient demographics, outcomes, and complications., Methods: A retrospective cohort methodology was used based on the STROBE criteria. ANOVA was used to evaluate for significant differences between the means of continuous variables among centers. Dichotomous outcomes were assessed between centers using a univariate and multivariate logistic regression., Results: A total of 170 SEEG insertion procedures were included in the study from 6 different level 4 pediatric epilepsy centers. The mean patient age at time of SEEG insertion was 12.3 ± 4.7 years. There was no significant difference between the mean age at the time of SEEG insertion between centers (p = 0.3). The mean number of SEEG trajectories per patient was 11.3 ± 3.6, with significant variation between centers (p < 0.001). Epileptogenic loci were identified in 84.7% of cases (144/170). Patients in 140 cases (140/170, 82.4%) underwent a follow-up surgical intervention, with 47.1% (66/140) being seizure free at a mean follow-up of 30.6 months. An overall postoperative hemorrhage rate of 5.3% (9/170) was noted, with patients in 4 of these cases (4/170, 2.4%) experiencing a symptomatic hemorrhage and patients in 3 of these cases (3/170, 1.8%) requiring operative evacuation of the hemorrhage. There were no mortalities or long-term complications., Conclusions: As the first multicenter case series in pediatric SEEG, this study has aided in establishing normative practice patterns in the application of a novel surgical technique, provided a framework for anticipated outcomes that is generalizable and useful for patient selection, and allowed for discussion of what is an acceptable complication rate relative to the experiences of multiple institutions.

Objective: Drug-resistant epilepsy (DRE) during the first few months of life is challenging and necessitates aggressive treatment, including surgery. Because the most common causes of DRE in infancy are related to extensive developmental anomalies, surgery often entails extensive tissue resections or disconnection. The literature on "ultra-early" epilepsy surgery is sparse, with limited data concerning efficacy controlling the seizures, and safety. The current study's goal is to review the safety and efficacy of ultra-early epilepsy surgery performed before the age of 3 months., Methods: To achieve a large sample size and external validity, a multinational, multicenter retrospective study was performed, focusing on epilepsy surgery for infants younger than 3 months of age. Collected data included epilepsy characteristics, surgical details, epilepsy outcome, and complications., Results: Sixty-four patients underwent 69 surgeries before the age of 3 months. The most common pathologies were cortical dysplasia (28), hemimegalencephaly (17), and tubers (5). The most common procedures were hemispheric surgeries (48 procedures). Two cases were intentionally staged, and one was unexpectedly aborted. Nearly all patients received blood products. There were no perioperative deaths and no major unexpected permanent morbidities. Twenty-five percent of patients undergoing hemispheric surgeries developed hydrocephalus. Excellent epilepsy outcome (International League Against Epilepsy [ILAE] grade I) was achieved in 66% of cases over a median follow-up of 41 months (19-104 interquartile range [IQR]). The number of antiseizure medications was significantly reduced (median 2 drugs, 1-3 IQR, p < .0001). Outcome was not significantly associated with the type of surgery (hemispheric or more limited resections)., Significance: Epilepsy surgery during the first few months of life is associated with excellent seizure control, and when performed by highly experienced teams, is not associated with more permanent morbidity than surgery in older infants. Thus surgical treatment should not be postponed to treat DRE in very young infants based on their age., (© 2021 International League Against Epilepsy.)

Objective: The condylar-C2 sagittal vertical alignment (C-C2SVA) describes the relationship between the occipitoatlantal joint and C2 in patients with Chiari malformation type I (CM-I). It has been suggested that a C-C2SVA ≥ 5 mm is predictive of the need for occipitocervical fusion (OCF) or ventral brainstem decompression (VBD). The authors' objective was to validate the predictive utility of the C-C2SVA by using a large, multicenter cohort of patients., Methods: This validation study used a cohort of patients derived from the Park-Reeves Syringomyelia Research Consortium; patients < 21 years old with CM-I and syringomyelia treated from June 2011 to May 2016 were identified. The primary outcome was the need for OCF and/or VBD. After patients who required OCF and/or VBD were identified, 10 age- and sex-matched controls served as comparisons for each OCF/VBD patient. The C-C2SVA (defined as the position of a plumb line from the midpoint of the O-C1 joint relative to the posterior aspect of the C2-3 disc space), pBC2 (a line perpendicular to a line from the basion to the posteroinferior aspect of the C2 body), and clival-axial angle (CXA) were measured on sagittal MRI. The secondary outcome was the need for ≥ 2 CM-related operations., Results: Of the 206 patients identified, 20 underwent OCF/VBD and 14 underwent repeat posterior fossa decompression. A C-C2SVA ≥ 5 mm was 100% sensitive and 86% specific for requiring OCF/VBD, with a 12.6% misclassification rate, whereas CXA < 125° was 55% sensitive and 99% specific, and pBC2 ≥ 9 was 20% sensitive and 88% specific. Kaplan-Meier analysis demonstrated that there was a significantly shorter time to second decompression in children with C-C2SVA ≥ 5 mm (p = 0.0039). The mean C-C2SVA was greater (6.13 ± 1.28 vs 3.13 ± 1.95 mm, p < 0.0001), CXA was lower (126° ± 15.4° vs 145° ± 10.7°, p < 0.05), and pBC2 was similar (7.65 ± 1.79 vs 7.02 ± 1.26 mm, p = 0.31) among those who underwent OCF/VBD versus decompression only. The intraclass correlation coefficient for the continuous measurement of C-C2SVA was 0.52; the kappa value was 0.47 for the binary categorization of C-C2SVA ≥ 5 mm., Conclusions: These results validated the C-C2SVA using a large, multicenter, external cohort with 100% sensitivity, 86% specificity, and a 12.6% misclassification rate. A C-C2SVA ≥ 5 mm is highly predictive of the need for OCF/VBD in patients with CM-I. The authors recommend that this measurement be considered among the tools to identify the "high-risk" CM-I phenotype.

Objective: To develop and validate a model to predict seizure freedom in children undergoing cerebral hemispheric surgery for the treatment of drug-resistant epilepsy., Methods: We analyzed 1267 hemispheric surgeries performed in pediatric participants across 32 centers and 12 countries to identify predictors of seizure freedom at 3 months after surgery. A multivariate logistic regression model was developed based on 70% of the dataset (training set) and validated on 30% of the dataset (validation set). Missing data were handled using multiple imputation techniques., Results: Overall, 817 of 1237 (66%) hemispheric surgeries led to seizure freedom (median follow-up = 24 months), and 1050 of 1237 (85%) were seizure-free at 12 months after surgery. A simple regression model containing age at seizure onset, presence of generalized seizure semiology, presence of contralateral 18-fluoro-2-deoxyglucose-positron emission tomography hypometabolism, etiologic substrate, and previous nonhemispheric resective surgery is predictive of seizure freedom (area under the curve = .72). A Hemispheric Surgery Outcome Prediction Scale (HOPS) score was devised that can be used to predict seizure freedom., Significance: Children most likely to benefit from hemispheric surgery can be selected and counseled through the implementation of a scale derived from a multiple regression model. Importantly, children who are unlikely to experience seizure control can be spared from the complications and deficits associated with this surgery. The HOPS score is likely to help physicians in clinical decision-making., (© 2021 International League Against Epilepsy.)

Purpose: Blunt cerebrovascular injury (BCVI) is uncommon in the pediatric population. Among the management options is medical management consisting of antithrombotic therapy with either antiplatelets or anticoagulation. There is no consensus on whether administration of antiplatelets or anticoagulation is more appropriate for BCVI in children < 10 years of age. Our goal was to compare radiographic and clinical outcomes based on medical treatment modality for BCVI in children < 10 years., Methods: Clinical and radiographic data were collected retrospectively for children screened for BCVI with computed tomography angiography at 5 academic pediatric trauma centers., Results: Among 651 patients evaluated with computed tomography angiography to screen for BCVI, 17 patients aged less than 10 years were diagnosed with BCVI (7 grade I, 5 grade II, 1 grade III, 4 grade IV) and received anticoagulation or antiplatelet therapy for 18 total injuries: 11 intracranial carotid artery, 4 extracranial carotid artery, and 3 extracranial vertebral artery injuries. Eleven patients were treated with antiplatelets (10 aspirin, 1 clopidogrel) and 6 with anticoagulation (4 unfractionated heparin, 2 low-molecular-weight heparin, 1 transitioned from the former to the latter). There were no complications secondary to treatment. One patient who received anticoagulation died as a result of the traumatic injuries. In aggregate, children treated with antiplatelet therapy demonstrated healing on 52% of follow-up imaging studies versus 25% in the anticoagulation cohort., Conclusion: There were no observed differences in the rate of hemorrhagic complications between anticoagulation and antiplatelet therapy for BCVI in children < 10 years, with a nonsignificantly better rate of healing on follow-up imaging in children who underwent antiplatelet therapy; however, the study cohort was small despite including patients from 5 hospitals.

Background: Endovascular thrombectomy is a promising treatment for acute ischemic stroke in children, but outcome and technical data in pediatric patients with large-vessel occlusions are lacking., Objective: To assess technical and clinical outcomes of thrombectomy in pediatric patients., Methods: We undertook a retrospective cohort study of pediatric patients who experienced acute ischemic stroke from April 2017 to April 2019 who had immediate, 30-, and 90-d follow-up. Patients were treated with endovascular thrombectomy at 5 US pediatric tertiary care facilities. We recorded initial and postprocedural modified Thrombolysis in Cerebral Infarction (mTICI) grade ≥ 2b, initial and postprocedural Pediatric National Institutes of Health Stroke Scale (PedNIHSS) score, and pediatric modified Rankin scale (mRS) score 0 to 2 at 90 d., Results: There were 23 thrombectomies in 21 patients (mean age 11.6 ± 4.9 yr, median 11.5, range 2.1-19; 52% female). A total of 19 (83%) thrombectomies resulted in mTICI grade ≥ 2b recanalization. The median PedNIHSS score was 13 on presentation (range 4-33) and 2 (range 0-26) at discharge (mean reduction 11.3 ± 6.1). A total of 14 (66%) patients had a mRS score of 0 to 2 at 30-d follow-up; 18/21 (86%) achieved that by 90 d. The median mRS was 1 (range 0-4) at 30 d and 1 (range 0-5) at 90 d. One patient required a blood transfusion after thrombectomy., Conclusion: In this large series of pediatric patients treated with endovascular thrombectomy, successful recanalization was accomplished via a variety of approaches with excellent clinical outcomes; further prospective longitudinal study is needed., (Copyright © 2020 by the Congress of Neurological Surgeons.)

Objective: The authors' objective was to better understand the anatomical load-bearing relationship between the atlantooccipital joint and the upper cervical spine and its influence on the clinical behavior of patients with Chiari malformation type I (CM-I) and craniocervical pathology., Methods: In a single-center prospective study of patients younger than 18 years with CM-I from 2015 through 2017 (mean age 9.91 years), the authors measured the occipital condyle-C2 sagittal vertebral alignment (C-C2SVA; defined as the position of a plumb line from the midpoint of the occiput (C0)-C1 joint relative to the posterior aspect of the C2-3 disc space), the pB-C2 (a line perpendicular to a line from the basion to the posteroinferior aspect of the C2 body on sagittal MRI), and the CXA (clivoaxial angle). Control data from 30 patients without CM-I (mean age 8.97 years) were used for comparison. The primary outcome was the need for anterior odontoid resection and/or occipitocervical fusion with or without odontoid reduction. The secondary outcome was the need for two or more Chiari-related operations., Results: Of the 60 consecutive patients with CM-I identified, 7 underwent anterior odontoid resection or occipitocervical fusion and 10 underwent ≥ 2 decompressive procedures. The mean C-C2SVA was greater in the overall CM-I group versus controls (3.68 vs 0.13 mm, p < 0.0001), as was the pB-C2 (7.7 vs 6.4 mm, p = 0.0092); the CXA was smaller (136° vs 148°, p < 0.0001). A C-C2SVA ≥ 5 mm was found in 35% of CM-I children and 3.3% of controls (p = 0.0006). The sensitivities and specificities for requiring ventral decompression/occipitocervical fusion were 100% and 74%, respectively, for C-C2SVA ≥ 5 mm; 71% and 94%, respectively, for CXA < 125°; and 71% and 75%, respectively, for pB-C2 ≥ 9 mm. The sensitivities and specificities for the need for ≥ 2 decompressive procedures were 60% and 70%, respectively, for C-C2SVA ≥ 5 mm; 50% and 94%, respectively, for CXA < 125°; and 60% and 76%, respectively, for pB-C2 ≥ 9 mm. The log-rank test demonstrated significant differences between C-C2SVA groups (p = 0.0007) for the primary outcome. A kappa value of 0.73 for C-C2SVA between raters indicated substantial agreement., Conclusions: A novel screening measurement for craniocervical bony relationships, the C-C2SVA, is described. A significant difference in C-C2SVA between CM-I patients and controls was found. A C-C2SVA ≥ 5 mm is highly predictive of the need for occipitocervical fusion/ventral decompression in patients with CM-I. Further validation of this screening measurement is needed.

Brainstem cavernous malformations (CMs) often have high hemorrhage rates and significant posthemorrhage morbidity. The authors present two cases in which magnetic resonance thermography-guided laser interstitial therapy was used for treatment of pontine CMs after recurrent hemorrhage. Both patients showed significant symptomatic improvement and were hemorrhage-free at 12- and 6-month follow-up, respectively. Each had radiographic evidence of lesion involution on serial follow-up imaging. These early results demonstrate this treatment modality may be technically safe; however, larger case numbers and longer follow-up are needed to demonstrate efficacy.

Objective: Prenatal imaging has several critical roles in the diagnosis and management of myelomeningocele, including specific family counseling and the selection of fetal surgery or postnatal repair. In this study, the authors compared the accuracy of fetal MRI and prenatal ultrasonography (US) in predicting the spinal lesion level and assessed the correlation between imaging findings and motor function as independently evaluated by a physical therapist (PT) after birth., Methods: A retrospective review of demographic and clinical data was performed to identify children who had been treated with postnatal myelomeningocele closure at a single institution between March 2013 and December 2018. Patients were eligible for inclusion if they had all of the following: prenatal US identifying the neural tube defect level, fetal MRI identifying the neural tube defect level, and postoperative PT evaluation identifying the motor deficit level. Statistical analysis was performed using Cohen's kappa coefficient to compare the US- and MRI-demonstrated lesion level and correlate these findings with the motor level assigned postnatally by a PT via manual muscle testing., Results: Thirty-four patients met the inclusion criteria. The mean gestational age at US was 23.0 ± 4.7 weeks, whereas the mean gestational age at MRI was 24.0 ± 4.1 weeks. The mean time from surgery to the PT evaluation was 2.9 ± 1.9 days. Prenatal US and MRI were in agreement within one spinal level in 74% of cases (25/34, k = 0.43). When comparing the US-demonstrated spinal level with the PT-assigned motor level, the two were in agreement within one level in 65% of cases (22/34, k = 0.40). When comparing MRI-demonstrated spinal level with the PT motor level, the two were in agreement within one level in 59% of cases (20/34, k = 0.37). MRI and US were within two spinal levels of the PT evaluation in 79.4% and 85.3% of cases, respectively. MRI and US agreed within two levels in 97.1% of cases. Prenatal US and MRI were equivalent when comparing the difference between the imaged level and the postnatal motor deficit level (mean level difference: 1.12 ± 1.16 vs 1.17 ± 1.11, p = 0.86)., Conclusions: Prenatal US and MRI equivalently predicted the postnatal motor deficit level in children with myelomeningocele. These data may be valuable in prenatal prognostication.

Objective: Neurosurgical management preferences related to myelomeningocele (MMC) care demonstrate significant variability. The authors sought to evaluate variability in practice patterns across a group of senior pediatric neurosurgeons. The purpose of this study was to identify the extent of variability and of consensus with regard to neurosurgical management of MMC and associated hydrocephalus, Chiari II malformation, and tethered spinal cord., Methods: A 43-question survey was distributed electronically to the members of the American Society of Pediatric Neurosurgeons (ASPN). The survey covered domains such as clinic case volume, newborn management, hydrocephalus management, transition to adulthood, clinical indications for shunt revision, Chiari II malformation decompression (C2MD), and tethered cord release (TCR). Ninety responses were received from 200 active ASPN members, for an overall response rate of 45%., Results: The majority (58%) of respondents closed 5-15 new cases of open MMC per year. Nearly all (98%) respondents perform back closure within 48 hours of birth, with the majority imbricating the placode and striving for a 3- to 4-layer closure. The most consistent indications for surgical intervention in early hydrocephalus were CSF leak from the back (92%), progressive ventricular enlargement (89%), and brainstem symptoms, including apnea/bradycardia (81%), stridor (81%), and dysphagia (81%). Eighty percent of respondents indicated that spina bifida care is delivered through multidisciplinary clinics, with neurosurgery, orthopedic surgery, urology, physical therapy, and social work as the most common disciplines included. One-third of clinics see both pediatric and adult patients, one-third offer a formal transition program to adult care, and one-third have no transition program. The vast majority of respondents offer prenatal counseling (95%), referral for in utero closure (66%), and endoscopic third ventriculostomy/choroid plexus cauterization (72%). Respondents were more willing to perform shunt revision for symptoms alone than for image changes alone. An asymptomatic broken shunt without ventricular enlargement produced responses evenly divided between observation, intervention, and further investigation. Operative shunt exploration was always performed before C2MD by 56% of respondents and performed sometimes by 40% of respondents. Symptoms of brainstem dysfunction were the strongest clinical triggers reported for C2MD, while declines in urinary continence, leg strength or sensation, or ambulation were the most consistent thresholds for TCR., Conclusions: Significant disparities exist surrounding key areas of decision making regarding treatment for patients with MMC, though there are central areas of agreement among ASPN members. Additionally, there is significant variation in the clinical management of chronic hydrocephalus, C2MD, and TCR, underscoring the need for further research into these specific areas.

Objective: Comprehensive multicenter data on the surgical treatment of pediatric cerebrovascular malformations (CVMs) in the US are lacking. The goal of this study was to identify national trends in patient demographics and assess the effect of hospital case volume on outcomes., Methods: Admissions for CVMs (1997-2012) were identified from the nationwide Kids' Inpatient Database. Admissions with and without craniotomy were reviewed separately. Patients were categorized by whether they were treated at low-, medium-, or high-volume centers (< 10, 10-40, > 40 cases/year, respectively). A generalized linear model was used to evaluate the association of hospital pediatric CVM case volume and clinical variables assessing outcomes., Results: Among the 9655 patients, 1828 underwent craniotomy and 7827 did not. Patient age and race differed in the two groups, as did the rate of private medical payers. High-volume hospitals had fewer nonroutine discharges (11.2% [high] vs 16.4% [medium] vs 22.3% [low], p = 0.0001). For admissions requiring craniotomy, total charges ($106,282 [high] vs $126,215 [medium] vs $134,978 [low], p < 0.001) and complication rates (0.09% [high] vs 0.11% [medium] vs 0.16% [low], p = 0.001) were lower in high-volume centers., Conclusions: This study revealed that further investigation may be needed regarding barriers to surgical treatment of pediatric CVMs. The authors found that surgical treatment of pediatric CVM at high-volume centers is associated with significantly fewer complications, better dispositions, and lower costs, but for noncraniotomy patients, low-volume centers had lower rates of complications and death and lower costs. These findings may support the consideration of appropriate referral of CVM patients requiring surgery or with intracranial hemorrhage toward high-volume, specialized centers.

Objective: Large experiences with the treatment of pediatric arteriovenous malformations (AVMs) remain relatively rare, with limited data on presentation, treatment, and long-term functional outcomes. Because of the expected long lifespan of children, caregivers are especially interested in outcome measures that assess quality of life. The authors' intention was to describe the long-term functional outcomes of pediatric patients who undergo AVM surgery and to identify predictors of sustained neurological deficits., Methods: The authors analyzed a 21-year retrospective cohort of pediatric patients with intracranial AVMs treated with microsurgery at two institutions. The primary outcome was a persistent neurological deficit at last follow-up. Secondary outcome measures included modified Rankin Scale (mRS) score and independent living., Results: Overall, 97 patients (mean age 11.1 ± 4.5 years; 56% female) were treated surgically for intracranial AVMs (mean follow-up 77.5 months). Sixty-four patients (66%) presented with hemorrhage, and 45 patients (46%) had neurological deficits at presentation. Radiologically, 39% of lesions were Spetzler-Martin grade II. Thirty-seven patients (38%) with persistent neurological deficits at last follow-up were compared with those without deficits; there were no differences in patient age, presenting Glasgow Coma Scale score, AVM size, surgical blood loss, or duration of follow-up. Multivariate analysis demonstrated that a focal neurological deficit on presentation, AVM size > 3 cm, and lesions in eloquent cortex were independent predictors of persistent neurological deficits at long-term follow-up. Overall, 92% of the children had an mRS score ≤ 2 on long-term follow-up., Conclusions: Pediatric patients with AVMs treated with microsurgical resection have good functional and radiological outcomes. There is a high rate (38%) of persistent neurological deficits, which were independently predicted by preoperative deficits, AVMs > 3 cm, and lesions located in eloquent cortex. This information can be useful in counseling families on the likelihood of long-term neurological deficits after cerebral AVM surgery.

Background: The use of intraoperative MRI (iMRI) during treatment of gliomas may increase extent of resection (EOR), decrease need for early reoperation, and increase progression-free and overall survival, but has not been fully validated, particularly in the pediatric population., Objective: To assess the accuracy of iMRI to identify residual tumor in pediatric patients with glioma and determine the effect of iMRI on decisions for resection, complication rates, and other outcomes., Methods: We retrospectively analyzed a multicenter database of pediatric patients (age ≤ 18 years) who underwent resection of pathologically confirmed gliomas., Results: We identified 314 patients (mean age 9.7 ± 4.6 years) with mean follow-up of 48.3 ± 33.6 months (range 0.03-182.07 months) who underwent surgery with iMRI. There were 201 (64.0%) WHO grade I tumors, 57 (18.2%) grade II, 24 (7.6%) grade III, 9 (2.9%) grade IV, and 23 (7.3%) not classified. Among 280 patients who underwent resection using iMRI, 131 (46.8%) had some residual tumor and underwent additional resection after the first iMRI. Of the 33 tissue specimens sent for pathological analysis after iMRI, 29 (87.9%) showed positive tumor pathology. Gross total resection was identified in 156 patients (55.7%), but this was limited by 69 (24.6%) patients with unknown EOR., Conclusions: Analysis of the largest multicenter database of pediatric gliomas resected using iMRI demonstrated additional tumor resection in a substantial portion of cases. However, determining the impact of iMRI on EOR and outcomes remains challenging because iMRI use varies among providers nationally. Continued refinement of iMRI techniques for use in pediatric patients with glioma may improve outcomes.

OBJECTIVEThere remains uncertainty regarding the appropriate level of care and need for repeating neuroimaging among children with mild traumatic brain injury (mTBI) complicated by intracranial injury (ICI). This study's objective was to investigate physician practice patterns and decision-making processes for these patients in order to identify knowledge gaps and highlight avenues for future investigation.METHODSThe authors surveyed residents, fellows, and attending physicians from the following pediatric specialties: emergency medicine; general surgery; neurosurgery; and critical care. Participants came from 10 institutions in the United States and an email list maintained by the Canadian Neurosurgical Society. The survey asked respondents to indicate management preferences for and experiences with children with mTBI complicated by ICI, focusing on an exemplar clinical vignette of a 7-year-old girl with a Glasgow Coma Scale score of 15 and a 5-mm subdural hematoma without midline shift after a fall down stairs.RESULTSThe response rate was 52% (n = 536). Overall, 326 (61%) respondents indicated they would recommend ICU admission for the child in the vignette. However, only 62 (12%) agreed/strongly agreed that this child was at high risk of neurological decline. Half of respondents (45%; n = 243) indicated they would order a planned follow-up CT (29%; n = 155) or MRI scan (19%; n = 102), though only 64 (12%) agreed/strongly agreed that repeat neuroimaging would influence their management. Common factors that increased the likelihood of ICU admission included presence of a focal neurological deficit (95%; n = 508 endorsed), midline shift (90%; n = 480) or an epidural hematoma (88%; n = 471). However, 42% (n = 225) indicated they would admit all children with mTBI and ICI to the ICU. Notably, 27% (n = 143) of respondents indicated they had seen one or more children with mTBI and intracranial hemorrhage demonstrate a rapid neurological decline when admitted to a general ward in the last year, and 13% (n = 71) had witnessed this outcome at least twice in the past year.CONCLUSIONSMany physicians endorse ICU admission and repeat neuroimaging for pediatric mTBI with ICI, despite uncertainty regarding the clinical utility of those decisions. These results, combined with evidence that existing practice may provide insufficient monitoring to some high-risk children, emphasize the need for validated decision tools to aid the management of these patients.

Objective: Single-pulse electrical stimulation (SPES) of intracranial electrodes evokes responses that may help identify the seizure onset zone (SOZ); however, lack of automation and response variability has limited clinical adoption of this technique. We evaluated whether automated delivery of low-current SPES could evoke delayed high-frequency suppression (DHFS) of ongoing electrocorticography (ECoG) signals that, when combined with objective analytic techniques, may provide a reliable marker of this zone., Methods: Low-current SPES (1-ms, 3.5-mA biphasic pulses) was delivered to 652 electrodes across 10 patients undergoing ECoG for seizure focus localization. DHFS was measured by calculating the normalized trial-averaged time-frequency power (70-250 Hz) 0.4-1 sec post-stimulation. Electrodes that evoked suppression when stimulated or recorded suppression when stimulation was nearby were used to estimate the SOZ., Results: The estimated SOZ significantly identified the clinical SOZ in 6 of 10 patients (5 of 7 temporal foci) with a false-positive rate of 0-0.06. Stimulation required <2 h, was undetectable by patients, and did not induce seizures or after-discharges., Conclusions: We show that DHFS provides accurate estimates of the clinical SOZ in patients with refractory epilepsy., Significance: This approach may increase the safety, speed, and reproducibility of SOZ identification while reducing cost, subjectivity, and patient discomfort., (Copyright © 2018 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

Magnetic resonance imaging-guided stereotactic laser ablation of intracranial targets, including brain tumors, has expanded dramatically over the past decade, but there have been few reports of complications, especially those occurring in a delayed fashion. Laser ablation of subependymal giant cell astrocytomas (SEGAs) is an attractive alternative to maintenance immunotherapy in some children with tuberous sclerosis complex (TSC); however, the effect of treatment on disease progression and the nature and frequency of potential complications remains largely unknown. The authors report the case of a 5-year-old boy with TSC who underwent stereotactic laser ablation of a SEGA at the right foramen of Monro on 2 separate occasions. After the second ablation, immediate posttreatment MRI revealed gadolinium extravasation from the tumor into the lateral ventricle. Nine months later, the patient presented with papilledema and delayed obstructive hydrocephalus secondary to intraventricular adhesions causing a trapped right lateral ventricle. This was successfully treated with endoscopic septostomy. The authors discuss the potential cause and clinical management of a delayed complication not previously reported after a relatively novel surgical therapy.

Anterior two-thirds corpus callosotomy is a common palliative surgical intervention most commonly employed in patients with atonic or drop seizures. Recently, stereotactic laser ablation of the corpus callosum without a craniotomy has shown promise in achieving similar outcomes with fewer side effects and shorter hospitalizations. The authors demonstrate ablation of the anterior two-thirds corpus callosum in a patient with Lennox-Gastaut syndrome and drug-resistant drop seizures. Technical nuances of laser ablation with 3 laser fibers are described. Postoperatively, the patient showed a significant reduction in seizure frequency and severity over a 9-month follow-up period. The video can be found here: https://youtu.be/3-mMq5-PLiM .

OBJECTIVE Selective dorsal rhizotomy (SDR) via limited laminectomy is an effective treatment of lower-extremity spasticity in the pediatric population. Children with spasticity are also at risk for neuromuscular scoliosis; however, specific risk factors for progressive spinal deformity requiring posterior spinal fusion (PSF) after SDR are unknown. The authors' goal was to identify potential risk factors. METHODS The authors performed a retrospective cohort study of patients who underwent SDR via limited laminectomy between 2003 and 2014 and who had at least 1 year of follow-up. They analyzed demographic, clinical, and radiographic variables to elucidate risk factors for progressive neuromuscular scoliosis. The primary outcome was need for PSF. RESULTS One hundred thirty-four patients underwent SDR and had at least 12 months of follow-up (mean 65 months); 48 patients (36%) had detailed pre- and postoperative radiographic data available. The mean age at surgery was 10 years (SD 5.1 years). Eighty-four patients (63%) were ambulatory before SDR, 109 (82%) underwent a single-level laminectomy, and a mean of 53% of the dorsal rootlets from L-1 to S-1 were sectioned. Fifteen patients (11.2%) subsequently required PSF for progressive deformity. Nonambulatory status (p < 0.001) and a preoperative Cobb angle > 30° (p = 0.003) were significantly associated with PSF on univariate analysis, but no statistically significant correlation was found with any clinical or radiographic variable and PSF after SDR on multivariate regression analysis. CONCLUSIONS Patients with preoperative nonambulatory status and Cobb angle > 30° may be at risk for progressive spinal deformity requiring PSF after SDR. These are well-known risk factors for progressive deformity in children with spasticity in general. Although our analysis suggests SDR via limited laminectomy may not significantly accelerate the development of neuromuscular scoliosis, further case-control studies are critical to elucidate the impact of SDR on spinal deformity.

Background: Blunt cerebrovascular injury is uncommon in the pediatric population; penetrating cerebrovascular injuries are even rarer and are thus poorly understood., Objective: To describe the diagnosis and management of penetrating cerebrovascular injuries and describe outcomes of available treatment modalities., Methods: Clinical and radiographic data were collected retrospectively from a multicenter trauma registry for children screened for cerebrovascular injury during 2003 to 2013 at 4 academic pediatric trauma centers., Results: Among 645 pediatric patients evaluated with computed tomography angiography with blunt cerebrovascular injury, 130 also had a penetrating trauma indication. Seven penetrating cerebrovascular injuries were diagnosed in 7 male patients (mean age 12.4 years, range 12-18 years). Focal neurological deficit and concomitant intracranial injury were each seen in 2 patients. There were 2 intracranial carotid artery injuries, 4 extracranial carotid artery injuries, and 1 vertebral artery injury. The majority of injuries were higher than grade I (5/7; 71%): 2 were grade I, 1 grade II, 2 grade III, and 2 grade IV. The 2 patients with grade III injuries required open surgery, and 1 patient with a grade IV injury underwent endovascular treatment. Two patients suffered immediate stroke secondary to the penetrating cerebrovascular injury. There were no delayed neurological deficits from the penetrating injuries, and no patients died as a result of the injuries., Conclusion: This is the largest series of penetrating cerebrovascular trauma in the pediatric literature. Although rare, penetrating cerebrovascular injuries can be high-grade injuries that require urgent recognition and may require aggressive endovascular and/or open surgery for treatment., (Copyright © 2017 by the Congress of Neurological Surgeons)

Among the 1% of children affected by epilepsy, failure of pharmacological therapy and early age of seizure onset can lead to worse long-term cognitive outcomes, mental health disorders and impaired functional status. Surgical management often improves functional and cognitive outcomes in children with medically refractory epilepsy, especially when seizure remission is achieved. However, surgery remains underused in children with drug-resistant epilepsy, creating a large treatment gap. Several recent innovations have led to considerable improvement in surgical technique, including the recent development of minimally invasive diagnostic and therapeutic techniques such as stereotactic EEG, transcranial magnetic stimulation, MRI-guided laser ablation, as well as novel paradigms of neurostimulation. This article discusses the current landscape of surgical innovation in the management of paediatric epilepsy, leading to a paradigm shift towards minimally invasive therapy and closing the treatment gap in children suffering from drug-resistant seizures., Competing Interests: Competing interests: None declared., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Blunt cerebrovascular injury in children is an uncommon occurrence that if missed and left untreated can result in devastating long-term neurologic consequences. Diagnosis can be readily obtained by a computed tomographic angiogram of the head and neck. If confirmed, treatment with antithrombotic therapy dramatically reduces the risk of a cerebrovascular accident. The difficulty lies in determining which child should be screened for such an injury. Several institutions have come up with criteria for screening. In this article, we review the nuances of the cerebrovascular system and its resulting injury. We present recent literature on the subject in an attempt to add clarity to this challenging situation., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Risk factors for blunt cerebrovascular injury (BCVI) may differ between children and adults, suggesting that children at low risk for BCVI after trauma receive unnecessary computed tomography angiography (CTA) and high-dose radiation. We previously developed a score for predicting pediatric BCVI based on retrospective cohort analysis. Our objective is to externally validate this prediction score with a retrospective multi-institutional cohort. We included patients who underwent CTA for traumatic cranial injury at four pediatric Level I trauma centers. Each patient in the validation cohort was scored using the "Utah Score" and classified as high or low risk. Before analysis, we defined a misclassification rate <25% as validating the Utah Score. Six hundred forty-five patients (mean age 8.6 ± 5.4 years; 63.4% males) underwent screening for BCVI via CTA. The validation cohort was 411 patients from three sites compared with the training cohort of 234 patients. Twenty-two BCVIs (5.4%) were identified in the validation cohort. The Utah Score was significantly associated with BCVIs in the validation cohort (odds ratio 8.1 [3.3, 19.8], p < 0.001) and discriminated well in the validation cohort (area under the curve 72%). When the Utah Score was applied to the validation cohort, the sensitivity was 59%, specificity was 85%, positive predictive value was 18%, and negative predictive value was 97%. The Utah Score misclassified 16.6% of patients in the validation cohort. The Utah Score for predicting BCVI in pediatric trauma patients was validated with a low misclassification rate using a large, independent, multicenter cohort. Its implementation in the clinical setting may reduce the use of CTA in low-risk patients., Competing Interests: Author Disclosure Statement No competing financial interests exist.

The authors report the case of a previously healthy 6-month-old girl who presented with right arm and leg stiffening consistent with seizure activity. An initial CT scan of the head demonstrated acute subarachnoid hemorrhage in the basal cisterns extending into the left sylvian fissure. Computed tomography angiography demonstrated a 7 × 6 × 5-mm saccular aneurysm of the inferior M 2 division of the left middle cerebral artery. The patient underwent left craniotomy and microsurgical clip ligation with wrapping of the aneurysm neck because the vessel appeared circumferentially dysplastic in the region of the aneurysm. Postoperative angiography demonstrated a small remnant, sluggish distal flow, but no significant cerebral vasospasm. Fifty-five days after the initial aneurysm rupture, the patient presented again with an acute intraparenchymal hemorrhage of the left anterior temporal lobe. Angiogram revealed a circumferentially dysplastic superior division of the M 2 branch, with a new 5 × 4-mm saccular aneurysm distinct from the first, with 2 smaller aneurysms distal to the new ruptured aneurysm. Endovascular parent vessel occlusion with Onyx was performed. Genetic testing revealed a mutation of the MYH11. To the authors' knowledge, this is the first report of rapid de novo aneurysm formation in an infant with an MYH11 mutation. The authors review the patient's clinical presentation and management and comprehensively review the literature on this topic.

Epilepsy is a common disease in the pediatric population, and the majority of cases are controlled with medications and lifestyle modification. For the children whose seizures are pharmacoresistant, continued epileptic activity can have a severely detrimental impact on cognitive development. Early referral of children with drug-resistant seizures to a pediatric epilepsy surgery center for evaluation is critical to achieving optimal patient outcomes. There are several components to a thorough presurgical evaluation, including a detailed medical history and physical examination, noninvasive testing including electroencephalogram, magnetic resonance imaging (MRI) of the brain, and often metabolic imaging. When necessary, invasive diagnostic testing using intracranial monitoring can be used. The identification of an epileptic focus may allow resection or disconnection from normal brain structures, with the ultimate goal of complete seizure remission. Additional operative measures can decrease seizure frequency and/or intensity if a clear epileptic focus cannot be identified. In this review, we will discuss the nuances of presurgical evaluation and decision-making in the management of children with drug-resistant epilepsy (DRE)., Competing Interests: The authors have no conflicts of interest to declare.