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OBJECTIVE Hemispherectomy is a complex surgical intervention for medically refractory epilepsy, and its surgical sequelae continue to be defined. The incidence, timing, and predictors of postoperative hydrocephalus are not well understood. Correspondingly, the aim of this study was to define the natural history of the development of hydrocephalus after hemispherectomy based on the authors’ institutional experience. METHODS The authors performed a retrospective review of their departmental database for all relevant cases between 1988 and 2018. Demographic and clinical results were abstracted and analyzed using regression analyses to identify predictors of postoperative hydrocephalus. RESULTS Of the 114 patients who satisfied selection criteria, there were 53 females (46%) and 61 males (53%) with mean ages of 2.2 and 6.5 years at first seizure and at hemispherectomy, respectively. There were 16 patients (14%) with a history of previous seizure surgery. In terms of surgery, the mean estimated blood loss was 441 ml, with a mean operative time of 7 hours, and 81 patients (71%) required intraoperative transfusions. A planned postoperative external ventricular drain (EVD) was placed in 38 patients (33%). The most common procedural complications were infection and hematoma, occurring in 7 patients (6%) each. Overall, there were 13 patients (11%) with postoperative hydrocephalus requiring permanent CSF diversion, occurring at a median of 1 year (range 0.1–5 years) after surgery. On multivariable analysis, a postoperative EVD (OR 0.12, p < 0.01) was significantly associated with a decreased likelihood of postoperative hydrocephalus, whereas previous surgery history (OR 4.32, p = 0.03) and postoperative infection complication (OR 5.14, p = 0.04) were significantly associated with increased likelihood of postoperative hydrocephalus. CONCLUSIONS Postoperative hydrocephalus mandating permanent CSF diversion following hemispherectomy can be expected in approximately 1 in 10 cases, presenting months after surgery on average. A postoperative EVD appears to reduce this likelihood, whereas postoperative infection and previous history of seizure surgery were shown to statistically increase this likelihood. These parameters should be carefully considered in the management of pediatric hemispherectomy for medically refractory epilepsy.

OBJECTIVE The success rate of endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) in the management of posthemorrhagic hydrocephalus (PHH) following intraventricular hemorrhage (IVH) in infants is not well defined. Furthermore, parameters of IVH at initial presentation have not been tested for predictive associations of ETV/CPC success in this setting. The authors sought to summarize their institutional outcomes to identify possible predictors of ETV/CPC success within this niche. METHODS A retrospective review was conducted of all ETV/CPC procedures performed at the authors’ institution for PHH between 2011 and 2021. Patients were screened against a set of selection criteria including follow-up time of at least 6 months. Associations with ETV/CPC failure were evaluated using regression and Kaplan-Meier analyses. RESULTS A total of 50 patients satisfied all criteria. There were 32 (64%) male and 18 (36%) female patients with a mean gestational birth age of 26 weeks. The presenting IVH was symmetric in 30 (60%) and asymmetric in 20 (40%) patients, and the maximum IVH grade was IV in 30 (60%) patients overall. Six months after the procedure, ETV/CPC success was seen in 18 (36%) patients and failure in 32 (64%) patients. The median overall follow-up was 42 months, at which point ETV/CPC success was observed in 11 (22%) patients and ETV/CPC failure in 39 (78%) patients. Regression analyses indicated that radiological IVH symmetry was a statistically significant predictor of ETV/CPC failure at 6 months (OR 3.46, p = 0.04) and overall (OR 5.33, p = 0.03). Overall rates of failure were 89% versus 62% (p = 0.02) when comparing symmetric versus asymmetric IVH patients, and time to failure occurred at median times of 1.4 versus 6.5 months (p = 0.03) after the initial procedure. Higher maximum IVH grade and younger age at initial ETV/CPC only trended toward increased failure rates. When the etiology component of the ETV Success Score was adjusted such that symmetric IVH was scored 0, the area under the curve for failure at 6 months increased from 0.58 to 0.69. CONCLUSIONS Overall, approximately 1 in 5 infants with PHH can expect to not require further intervention following ETV/CPC. The authors demonstrate that IVH symmetry is statistically predictive of ETV/CPC failure in this setting independent of all other parameters, where PHH infants with symmetric IVH are more likely to experience failure, and sooner, than PHH infants with asymmetric IVH. When discussing possible success rates of ETV/CPC for PHH, IVH symmetry should be considered.

OBJECTIVE Resection and disconnection surgeries for epilepsy in the pediatric demographic (patients ≤ 18 years of age) are two separate, definitive intervention options in medically refractory cases. Questions remain regarding the role of surgery when seizures persist after an initial incomplete surgery. The aim of this study was to review the contemporary literature and summarize the metadata on the outcomes of repeat surgery in this specific demographic. METHODS Searches of seven electronic databases from inception to July 2022 were conducted using PRISMA guidelines. Articles were screened using prespecified criteria. Metadata from the articles were abstracted and pooled by random-effects meta-analysis of proportions. RESULTS Eleven studies describing 12 cohorts satisfied all criteria, reporting outcomes of 170 pediatric patients with epilepsy who underwent repeat resection or disconnection surgery. Of these patients, 55% were male, and across all studies, median ages at initial and repeat surgeries were 7.2 and 9.4 years, respectively. The median follow-up duration after repeat surgery was 47.7 months. The most commonly reported etiology for epilepsy was cortical dysplasia. Overall, the estimated incidence of complete seizure freedom (Engel class I) following repeat surgery was 48% (95% CI 40%–56%, p value for heterogeneity = 0.93), and the estimated incidence of postoperative complications following repeat surgery was 25% (95% CI 12%–39%, p = 0.04). There were six cohorts each that described outcomes for repeat resection and repeat disconnection surgeries. There was no statistical difference between these two subgroups with respect to estimated incidence of complete seizure freedom (p value for interaction = 0.92), but postoperative complications were statistically more common following repeat resection (p ≤ 0.01). CONCLUSIONS For both resection and disconnection surgeries, repeat epilepsy surgery in children is likely to confer complete seizure freedom in approximately half of the patients who experience unsuccessful initial incomplete epilepsy surgery. More data are needed to elucidate the impact on efficacy based on surgical approach selection. Judicious discussion and planning between the patient, family, and a multidisciplinary team of epilepsy specialists is recommended to optimize expectations and outcomes in this setting.

OBJECTIVE Magnetic resonance–guided focused ultrasound (MRgFUS) is an incisionless procedure capable of thermoablation through the focus of multiple acoustic beams. Although MRgFUS is currently approved for the treatment of tremor in adults, its safety and feasibility profile for intracranial lesions in the pediatric and young adult population remains unknown. METHODS The long-term outcomes of a prospective single-center, single-arm trial of MRgFUS at Nicklaus Children’s Hospital in Miami, Florida, are presented. Patients 15–22 years of age with centrally located lesions were recruited, clinically consistent with WHO grade I tumors that require surgical intervention. This cohort consisted of 4 patients with hypothalamic hamartoma (HH), and 1 patient with tuberous sclerosis complex harboring a subependymal giant cell astrocytoma (SEGA). RESULTS In each case, high-intensity FUS was used to target the intracranial lesion. Real-time MRI was used to monitor the thermoablations. Primary outcomes of interest were tolerability, feasibility, and safety of FUS. The radiographic ablation volume on intra- and postoperative MRI was also assessed. All 5 patients tolerated the procedure without any complications. Successful thermoablation was achieved in 4 of the 5 cases; the calcified SEGA was undertreated due to intratumor calcification, which prevented attainment of the target ablation temperature. The HHs underwent target tissue thermoablations that led to MR signal changes at the treatment site. For the patients harboring HHs, FUS thermoablations occurred without procedure-related complications and led to improvement in seizure control or hypothalamic hyperphagia. All 5 patients were discharged home on postoperative day 1 or 2, without any readmissions. There were no cases of hemorrhage, electrolyte derangement, endocrinopathy, or new neurological deficit in this cohort. CONCLUSIONS This experience demonstrates that FUS thermoablation of centrally located brain lesions in adolescents and young adults can be performed safely and that it provides therapeutic benefit for associated symptoms.

OBJECTIVE The aim of this study was to determine differences in complications and outcomes between posterior fossa decompression with duraplasty (PFDD) and without duraplasty (PFD) for the treatment of pediatric Chiari malformation type I (CM1) and syringomyelia (SM). METHODS The authors used retrospective and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM1-SM who received PFD or PFDD and had at least 1 year of follow-up data. Preoperative, treatment, and postoperative characteristics were recorded and compared between groups. RESULTS A total of 692 patients met the inclusion criteria for this database study. PFD was performed in 117 (16.9%) and PFDD in 575 (83.1%) patients. The mean age at surgery was 9.86 years, and the mean follow-up time was 2.73 years. There were no significant differences in presenting signs or symptoms between groups, although the preoperative syrinx size was smaller in the PFD group. The PFD group had a shorter mean operating room time (p < 0.0001), fewer patients with > 50 mL of blood loss (p = 0.04), and shorter hospital stays (p = 0.0001). There were 4 intraoperative complications, all within the PFDD group (0.7%, p > 0.99). Patients undergoing PFDD had a 6-month complication rate of 24.3%, compared with 13.7% in the PFD group (p = 0.01). There were no differences between groups for postoperative complications beyond 6 months (p = 0.33). PFD patients were more likely to require revision surgery (17.9% vs 8.3%, p = 0.002). PFDD was associated with greater improvements in headaches (89.6% vs 80.8%, p = 0.04) and back pain (86.5% vs 59.1%, p = 0.01). There were no differences between groups for improvement in neurological examination findings. PFDD was associated with greater reduction in anteroposterior syrinx size (43.7% vs 26.9%, p = 0.0001) and syrinx length (18.9% vs 5.6%, p = 0.04) compared with PFD. CONCLUSIONS PFD was associated with reduced operative time and blood loss, shorter hospital stays, and fewer postoperative complications within 6 months. However, PFDD was associated with better symptom improvement and reduction in syrinx size and lower rates of revision decompression. The two surgeries have low intraoperative complication rates and comparable complication rates beyond 6 months.

There is substantial variability in reported seizure outcome following pediatric epilepsy surgery, and lack of individualized predictive tools that could evaluate the probability of seizure freedom postsurgery. The aim of this study was to develop and validate a supervised machine learning (ML) model for predicting seizure freedom after pediatric epilepsy surgery.This is a multicenter retrospective study of children who underwent epilepsy surgery at five pediatric epilepsy centers in North America. Clinical information, diagnostic investigations, and surgical characteristics were collected, and used as features to predict seizure-free outcome 1 year after surgery. The dataset was split randomly into 80% training and 20% testing data. Thirty-five combinations of five feature sets with seven ML classifiers were assessed on the training cohort using 10-fold cross-validation for model development. The performance of the optimal combination of ML classifier and feature set was evaluated in the testing cohort, and compared with logistic regression, a classical statistical approach.Of the 801 patients included, 61.3% were seizure-free 1 year postsurgery. During model development, the best combination was XGBoost ML algorithm with five features from the univariate feature set, including number of antiseizure medications, magnetic resonance imaging lesion, age at seizure onset, video-electroencephalography concordance, and surgery type, with a mean area under the curve (AUC) of .73 (95% confidence interval [CI] = .69-.77). The combination of XGBoost and univariate feature set was then evaluated on the testing cohort and achieved an AUC of .74 (95% CI = .66-.82; sensitivity = .87, 95% CI = .81-.94; specificity = .58, 95% CI = .47-.71). The XGBoost model outperformed the logistic regression model (AUC = .72, 95% CI = .63-.80; sensitivity = .72, 95% CI = .63-.82; specificity = .66, 95% CI = .53-.77) in the testing cohort (p = .005).This study identified important features and validated an ML algorithm, XGBoost, for predicting the probability of seizure freedom after pediatric epilepsy surgery. Improved prognostication of epilepsy surgery is critical for presurgical counseling and will inform treatment decisions.

OBJECTIVE The goal of this study was to assess the social determinants that influence access and outcomes for pediatric neurosurgical care for patients with Chiari malformation type I (CM-I) and syringomyelia (SM). METHODS The authors used retro- and prospective components of the Park-Reeves Syringomyelia Research Consortium database to identify pediatric patients with CM-I and SM who received surgical treatment and had at least 1 year of follow-up data. Race, ethnicity, and insurance status were used as comparators for preoperative, treatment, and postoperative characteristics and outcomes. RESULTS A total of 637 patients met inclusion criteria, and race or ethnicity data were available for 603 (94.7%) patients. A total of 463 (76.8%) were non-Hispanic White (NHW) and 140 (23.2%) were non-White. The non-White patients were older at diagnosis (p = 0.002) and were more likely to have an individualized education plan (p < 0.01). More non-White than NHW patients presented with cerebellar and cranial nerve deficits (i.e., gait ataxia [p = 0.028], nystagmus [p = 0.002], dysconjugate gaze [p = 0.03], hearing loss [p = 0.003], gait instability [p = 0.003], tremor [p = 0.021], or dysmetria [p < 0.001]). Non-White patients had higher rates of skull malformation (p = 0.004), platybasia (p = 0.002), and basilar invagination (p = 0.036). Non-White patients were more likely to be treated at low-volume centers than at high-volume centers (38.7% vs 15.2%; p < 0.01). Non-White patients were older at the time of surgery (p = 0.001) and had longer operative times (p < 0.001), higher estimated blood loss (p < 0.001), and a longer hospital stay (p = 0.04). There were no major group differences in terms of treatments performed or complications. The majority of subjects used private insurance (440, 71.5%), whereas 175 (28.5%) were using Medicaid or self-pay. Private insurance was used in 42.2% of non-White patients compared to 79.8% of NHW patients (p < 0.01). There were no major differences in presentation, treatment, or outcome between insurance groups. In multivariate modeling, non-White patients were more likely to present at an older age after controlling for sex and insurance status (p < 0.01). Non-White and male patients had a longer duration of symptoms before reaching diagnosis (p = 0.033 and 0.004, respectively). CONCLUSIONS Socioeconomic and demographic factors appear to influence the presentation and management of patients with CM-I and SM. Race is associated with age and timing of diagnosis as well as operating room time, estimated blood loss, and length of hospital stay. This exploration of socioeconomic and demographic barriers to care will be useful in understanding how to improve access to pediatric neurosurgical care for patients with CM-I and SM.

Intracranial mixed vascular malformations (MVMs) are defined as any combination of a developmental venous anomaly (DVA), cerebral cavernous malformation (CCM), capillary telangiectasia (CTG), or arteriovenous malformation (AVM) within a single, contiguous lesion. However, most MVMs described in the literature contain only 2 pathologically discrete malformations; juxtaposition of 3 or more abnormalities in a single lesion remains exceedingly rare. We present the case of a 19-month-old female with new onset focal seizures and a 4-cm right basal ganglia lesion initially believed to be an embryonal neoplasm. She subsequently underwent gross total resection (GTR) of the lesion via a transsylvian-transinsular approach. Intraoperatively, the lesion appeared to be heterogenous and highly vascular, with areas of purplish-gray friable tissue. Pathology confirmed the lesion to be a MVM containing a CCM, CTG, and a DVA. This appears to be the first reported case of such a lesion confirmed on pathology in the literature.

BackgroundDespite the well-documented utility of responsive neurostimulation (RNS, NeuroPace) in adult epilepsy patients, literature on the use of RNS in children is limited.ObjectiveTo determine the real-world efficacy and safety of RNS in pediatric epilepsy patients.MethodsPatients 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 compared with prior literature pertaining to adult patients.ResultsOf 35 patients identified, 17 were

Minimally invasive magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has been proposed as an alternative to open epilepsy surgery, to address concerns regarding the risk of open surgery. Our primary hypothesis was that seizure freedom at 1 year after MRgLITT is noninferior to open surgery in children with drug-resistant epilepsy (DRE). The secondary hypothesis was that MRgLITT has fewer complications and shorter hospitalization than surgery. The primary objective was to compare seizure outcome of MRgLITT to open surgery in children with DRE. The secondary objective was to compare complications and length of hospitalization of the two treatments.This retrospective multicenter cohort study included children with DRE treated with MRgLITT or open surgery with 1-year follow-up. Exclusion criteria were corpus callosotomy, neurostimulation, multilobar or hemispheric surgery, and lesion with maximal dimension 60 mm. MRgLITT patients were propensity matched to open surgery patients. The primary outcome was seizure freedom at 1 year posttreatment. The difference in seizure freedom was compared using noninferiority test, with noninferiority margin of -10%. The secondary outcomes were complications and length of hospitalization.One hundred eighty-five MRgLITT patients were matched to 185 open surgery patients. Seizure freedom at 1 year follow-up was observed in 89 of 185 (48.1%) MRgLITT and 114 of 185 (61.6%) open surgery patients (difference = -13.5%, one-sided 97.5% confidence interval = -23.8% to ∞, pSeizure outcome of MRgLITT at 1 year posttreatment was inferior to open surgery. However, MRgLITT has the advantage of better safety profile and shorter hospitalization. The findings will help counsel children and parents on the benefits and risks of MRgLITT and contribute to informed decision-making on treatment options.

The recent emphasis on surgery as an essential yet neglected component of global health care has drawn attention to the lack of pediatric neurosurgery in low- and middle-income countries (LMICs). Most LMICs have very few neurosurgeons able or willing to care for children, leaving few access to care for readily treatable conditions, like pediatric hydrocephalus and spina bifida. Herein, we review the role of pediatric neurosurgeons to improve hydrocephalus management and medical education in LMICs. A literature search on global pediatric neurosurgery was performed using the Pubmed database from the year 2000 to October 5th, 2021. The majority of current pediatric neurosurgical efforts focus on the management of hydrocephalus. Endoscopic third ventriculostomy (ETV) with or without choroid plexus cauterization (CPC) has emerged as an effective alternative to ventriculoperitoneal (VP) shunting for CSF diversion in LMICs. Neurosurgical programs in LMICs are most successful when twinning is used to pair the program with an established center in a high-income country (HIC). Multiple international pediatric neurosurgical efforts aim to provide medical expertise, infrastructure, and surgical education to LMICs. The next step in developing sustainable surgery programs in LMICs includes establishing local training programs. Existing global pediatric neurosurgery programs are most successful when partnered to an experienced center in a HIC. A lack of funding, resources, and training programs all pose barriers to increasing the pediatric neurosurgical capacity in LMICs. Future global neurosurgery efforts may look to other neurosurgical conditions with high burdens of disease in LMICs, like epilepsy.

OBJECTIVE Posterior fossa decompression with duraplasty (PFDD) is commonly performed for Chiari I malformation (CM-I) with syringomyelia (SM). However, complication rates associated with various dural graft types are not well established. The objective of this study was to elucidate complication rates within 6 months of surgery among autograft and commonly used nonautologous grafts for pediatric patients who underwent PFDD for CM-I/SM. METHODS The Park-Reeves Syringomyelia Research Consortium database was queried for pediatric patients who had undergone PFDD for CM-I with SM. All patients had tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and ≥ 6 months of postoperative follow-up after PFDD. Complications (e.g., pseudomeningocele, CSF leak, meningitis, and hydrocephalus) and postoperative changes in syrinx size, headaches, and neck pain were compared for autograft versus nonautologous graft. RESULTS A total of 781 PFDD cases were analyzed (359 autograft, 422 nonautologous graft). Nonautologous grafts included bovine pericardium (n = 63), bovine collagen (n = 225), synthetic (n = 99), and human cadaveric allograft (n = 35). Autograft (103/359, 28.7%) had a similar overall complication rate compared to nonautologous graft (143/422, 33.9%) (p = 0.12). However, nonautologous graft was associated with significantly higher rates of pseudomeningocele (p = 0.04) and meningitis (p < 0.001). The higher rate of meningitis was influenced particularly by the higher rate of chemical meningitis (p = 0.002) versus infectious meningitis (p = 0.132). Among 4 types of nonautologous grafts, there were differences in complication rates (p = 0.02), including chemical meningitis (p = 0.01) and postoperative nausea/vomiting (p = 0.03). Allograft demonstrated the lowest complication rates overall (14.3%) and yielded significantly fewer complications compared to bovine collagen (p = 0.02) and synthetic (p = 0.003) grafts. Synthetic graft yielded higher complication rates than autograft (p = 0.01). Autograft and nonautologous graft resulted in equal improvements in syrinx size (p < 0.0001). No differences were found for postoperative changes in headaches or neck pain. CONCLUSIONS In the largest multicenter cohort to date, complication rates for dural autograft and nonautologous graft are similar after PFDD for CM-I/SM, although nonautologous graft results in higher rates of pseudomeningocele and meningitis. Rates of meningitis differ among nonautologous graft types. Autograft and nonautologous graft are equivalent for reducing syrinx size, headaches, and neck pain.

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.

BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P

Objective: We describe a multicenter experience with vagus nerve stimulator implantation in pediatric patients with drug-resistant epilepsy. Our goal was to assess vagus nerve stimulation efficacy and identify potential predictors of favorable outcome. Methods: This is a retrospective study. Inclusion criteria: ≤18 years at time of vagus nerve stimulator implantation, at least 1 year of follow-up. All patients were previously found to be unsuitable for an excisional procedure. Favorable clinical outcome and effective vagus nerve stimulation therapy were defined as seizure reduction >50%. Outcome data were reviewed at 1, 2, 3, and 5 years after vagus nerve stimulator implantation. Fisher exact test and multiple logistic regression analysis were employed. Results: Eighty-nine patients met inclusion criteria. Responder rate (seizure frequency reduction >50%) at 1-year follow-up was 25.8% (4.5% seizure-free). At last follow-up, 31.5% had a favorable outcome and 5.2% were seizure free. The only factor significantly predicting favorable outcome was time to vagus nerve stimulator implantation, with the best outcome achieved when vagus nerve stimulator implantation was performed within 3 years of seizure onset. Implantation between 3 and 5 years after epilepsy onset correlated with better long-term seizure freedom (13.3% at T5). Overall, 65.2% of patients evidenced improved quality of life at last follow-up. However, 12.4% had adverse events, but most were mild and disappeared after 3-4 months. Conclusions: Early vagus nerve stimulator implantation within 5 years of seizure onset was the only predictor of favorable clinical outcome in pediatric patients. Improved quality of life and a low incidence of significant adverse events were observed.

OBJECTIVEUncontrolled epilepsy is associated with serious deleterious effects on the neurological development of infants and has been described as “catastrophic epilepsy.” Recently, there has been increased emphasis on early surgical interventions to preserve or rescue neurodevelopmental outcomes in infants with early intractable epilepsy. The enthusiasm for early treatments is often tempered by concerns regarding the morbidity of neurosurgical procedures in very young patients. Here, the authors report outcomes following the surgical management of infants (younger than 1 year).METHODSThe authors performed a retrospective review of patients younger than 1 year of age who underwent surgery for epilepsy at Miami (Nicklaus) Children’s Hospital and Jackson Memorial Hospital between 1994 and 2018. Patient demographics, including the type of interventions, were recorded. Seizure outcomes (at last follow-up and at 1 year postoperatively) as well as complications are reported.RESULTSThirty-eight infants (median age 5.9 months) underwent a spectrum of surgical interventions, including hemispherectomy (n = 17), focal resection (n = 13), and multilobe resections (n = 8), with a mean follow-up duration of 9.1 years. Hemimegalencephaly and cortical dysplasia were the most commonly encountered pathologies. Surgery for catastrophic epilepsy resulted in complete resolution of seizures in 68% (n = 26) of patients, and 76% (n = 29) had a greater than 90% reduction in seizure frequency. Overall mortality and morbidity were 0% and 10%, respectively. The latter included infections (n = 2), infarct (n = 1), and immediate reoperation for seizures (n = 1).CONCLUSIONSSurgical intervention for catastrophic epilepsy in infants remains safe, efficacious, and durable. The authors’ work provides the longest follow-up of such a series on infants to date and compares favorably with previously published series.

OBJECTIVEAlthough hemispherectomy is an effective treatment for children with intractable hemispheric epilepsy syndromes, as many as 40% of patients eventually develop seizure recurrence. The causes of seizure recurrence in these patients are incompletely understood. The authors sought to evaluate the efficacy of hemispherectomy at their center and determine whether contralateral MRI abnormalities can predict seizure recurrence.METHODSA retrospective review of consecutive hemispherectomies performed at Miami Children’s Hospital between January 2000 and June 2014 was performed. Time-to-event analysis was performed. The “event” was defined as any seizures following resective epilepsy surgery (not including seizures in the first postoperative week and auras). Several preoperative variables were analyzed to determine their suitability to predict seizure recurrence following surgery.RESULTSSixty-nine patients (44 boys) with a mean age of 8.2 ± 5.9 years (range 0.1–20.8 years) underwent 72 hemispherectomies; 67 of these were functional hemispherectomies, while another 5 were completion of a previous functional hemispherectomy (2 completions of functional hemispherectomies, 3 anatomical hemispherectomies). The duration of epilepsy was 5.8 ± 5.5 years with 66 cases (91.7%) having daily seizures. Etiology included stroke (n = 28), malformation of cortical development (n = 11), hemimegalencephaly (n = 11), encephalitis (n = 13), and other (n = 7). Engel class I outcome was achieved in 59 (86%) and 56 (81%) patients at 1 and 2 years of follow-up, respectively. The mean time to seizure recurrence was 33.5 ± 31.1 months. In univariate analyses, the absence of contralateral abnormalities on MRI (HR 4.09, 95% CI 1.41–11.89, p = 0.009) was associated with a longer duration of seizure freedom. The presence of contralateral MRI abnormalities was associated with contralateral ictal seizures on preoperative scalp EEG (p = 0.002). Fifteen patients experienced 20 complications (20/72, 27.8%), including the development of hydrocephalus necessitating CSF diversion in 9 cases (13%), hygroma in 1, hemispheric edema in 1, aseptic meningitis in 2, postoperative hemorrhage in 2, infection in 2, ischemic stroke in 2, and blood transfusion–contracted hepatitis C in 1 case.CONCLUSIONSPatients with bihemispheric abnormalities, as evidenced by contralateral MRI abnormalities, have a higher risk of earlier seizure recurrence following functional hemispherectomy.

OBJECTIVEFactors associated with syrinx size in pediatric patients undergoing posterior fossa decompression (PFD) or PFD with duraplasty (PFDD) for Chiari malformation type I (CM-I) with syringomyelia (SM; CM-I+SM) are not well established.METHODSUsing the Park-Reeves Syringomyelia Research Consortium registry, the authors analyzed variables associated with syrinx radiological outcomes in patients (< 20 years old at the time of surgery) with CM-I+SM undergoing PFD or PFDD. Syrinx resolution was defined as an anteroposterior (AP) diameter of ≤ 2 mm or ≤ 3 mm or a reduction in AP diameter of ≥ 50%. Syrinx regression or progression was defined using 1) change in syrinx AP diameter (≥ 1 mm), or 2) change in syrinx length (craniocaudal, ≥ 1 vertebral level). Syrinx stability was defined as a < 1-mm change in syrinx AP diameter and no change in syrinx length.RESULTSThe authors identified 380 patients with CM-I+SM who underwent PFD or PFDD. Cox proportional hazards modeling revealed younger age at surgery and PFDD as being independently associated with syrinx resolution, defined as a ≤ 2-mm or ≤ 3-mm AP diameter or ≥ 50% reduction in AP diameter. Radiological syrinx resolution was associated with improvement in headache (p < 0.005) and neck pain (p < 0.011) after PFD or PFDD. Next, PFDD (p = 0.005), scoliosis (p = 0.007), and syrinx location across multiple spinal segments (p = 0.001) were associated with syrinx diameter regression, whereas increased preoperative frontal-occipital horn ratio (FOHR; p = 0.007) and syrinx location spanning multiple spinal segments (p = 0.04) were associated with syrinx length regression. Scoliosis (HR 0.38 [95% CI 0.16–0.91], p = 0.03) and smaller syrinx diameter (5.82 ± 3.38 vs 7.86 ± 3.05 mm; HR 0.60 [95% CI 0.34–1.03], p = 0.002) were associated with syrinx diameter stability, whereas shorter preoperative syrinx length (5.75 ± 4.01 vs 9.65 ± 4.31 levels; HR 0.21 [95% CI 0.12–0.38], p = 0.0001) and smaller pB-C2 distance (6.86 ± 1.27 vs 7.18 ± 1.38 mm; HR 1.44 [95% CI 1.02–2.05], p = 0.04) were associated with syrinx length stability. Finally, younger age at surgery (8.19 ± 5.02 vs 10.29 ± 4.25 years; HR 1.89 [95% CI 1.31–3.04], p = 0.01) was associated with syrinx diameter progression, whereas increased postoperative syrinx diameter (6.73 ± 3.64 vs 3.97 ± 3.07 mm; HR 3.10 [95% CI 1.67–5.76], p = 0.003), was associated with syrinx length progression. PFD versus PFDD was not associated with syrinx progression or reoperation rate.CONCLUSIONSThese data suggest that PFDD and age are independently associated with radiological syrinx improvement, although forthcoming results from the PFDD versus PFD randomized controlled trial (NCT02669836, clinicaltrials.gov) will best answer this question.

OBJECTIVEUntreated hydrocephalus poses a significant health risk to children in the developing world. In response to this risk, global neurosurgical efforts have increasingly focused on endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) in the management of infantile hydrocephalus in low- and middle-income countries (LMICs). Here, the authors report their experience with ETV/CPC at the Hospital Bernard-Mevs/Project Medishare (HBMPM) in Port-au-Prince, Haiti.METHODSThe authors conducted a retrospective review of a series of consecutive children who had undergone ETV/CPC for hydrocephalus over a 1-year period at HBMPM. The primary outcome of interest was time to ETV/CPC failure. Univariate and multivariate analyses using a Cox proportional hazards regression were performed to identify preoperative factors that were associated with outcomes.RESULTSOf the 82 children who underwent ETV/CPC, 52.2% remained shunt free at the last follow-up (mean 6.4 months). On univariate analysis, the ETV success score (ETVSS; p = 0.002), success of the attempted ETV (p = 0.018), and bilateral CPC (p = 0.045) were associated with shunt freedom. In the multivariate models, a lower ETVSS was independently associated with a poor outcome (HR 0.072, 95% CI 0.016–0.32, p < 0.001). Two children (2.4%) died of postoperative seizures.CONCLUSIONSAs in other LMICs, ETV/CPC is an effective treatment for hydrocephalus in children in Haiti, with a low but significant risk profile. Larger multinational prospective databases may further elucidate the ideal candidate for ETV/CPC in resource-poor settings.

OBJECTIVE Many pathways to positions of leadership exist within pediatric neurological surgery. The authors sought to investigate common trends in leadership among pediatric neurosurgery fellowship directors (FDs) and describe how formalized pediatric neurosurgical training arrived at its current state. METHODS Fellowship programs were identified using the Accreditation Council for Pediatric Neurosurgery Fellowships website. Demographic, training, membership, and research information was collected via email, telephone, curricula vitae, and online searches. RESULTS The authors’ survey was sent to all 35 identified FDs, and 21 responses were received. Response data were supplemented with curricula vitae and online data prior to analysis. FDs were predominantly male, self-identified predominantly as Caucasian, and had a mean age of 53 years. The mean duration from residency graduation until FD appointment was 13.4 years. The top training programs to produce future FDs were New York University and Washington University in St. Louis (residency) and Washington University in St. Louis (fellowship). CONCLUSIONS This study characterizes the current state of pediatric neurosurgery fellowship program leadership. The data serve as an important point of reference to compare with future leadership as well as contrast with neurosurgery and other surgical disciplines in general.

IntroductionVagus nerve stimulation (VNS) is a neuromodulation therapy that can reduce the seizure burden of children with medically intractable epilepsy. Despite the widespread use of VNS to treat epilepsy, there are currently no means to preoperatively identify patients who will benefit from treatment. The objective of the present study is to determine clinical and neural network-based correlates of treatment outcome to better identify candidates for VNS therapy.Methods and analysisIn this multi-institutional North American study, children undergoing VNS and their caregivers will be prospectively recruited. All patients will have documentation of clinical history, physical and neurological examination and video electroencephalography as part of the standard clinical workup for VNS. Neuroimaging data including resting-state functional MRI, diffusion-tensor imaging and magnetoencephalography will be collected before surgery. MR-based measures will also be repeated 12 months after implantation. Outcomes of VNS, including seizure control and health-related quality of life of both patient and primary caregiver, will be prospectively measured up to 2 years postoperatively. All data will be collected electronically using Research Electronic Data Capture.Ethics and disseminationThis study was approved by the Hospital for Sick Children Research Ethics Board (REB number 1000061744). All participants, or substitute decision-makers, will provide informed consent prior to be enrolled in the study. Institutional Research Ethics Board approval will be obtained from each additional participating site prior to inclusion. This study is funded through a Canadian Institutes of Health Research grant (PJT-159561) and an investigator-initiated funding grant from LivaNova USA (Houston, TX; FF01803B IIR).

Osteosarcomas arising within the pterygomaxillary/infratemporal fossa region are rare among the pediatric population. Survival rates are most influenced by tumor resection with negative margins, which can be dependent on surgical accessibility of the tumor site. The pterygomaxillary/infratemporal fossa location poses several challenges to safe and adequate tumor resection, including proximity of the facial nerve and great vessels and scarring associated with traditional transfacial approaches. In this article, we present the case of a 6-year-old boy with an osteosarcoma of the left pterygomaxillary/infratemporal fossa region successfully managed with an “oncoplastic” approach, incorporating the use of CAD/CAM and mixed reality technologies.

OBJECTIVE MRI is increasingly employed to assess intrauterine fetal anomalies. Central nervous system (CNS) anomalies are common structural conditions that warrant evaluation with fetal MRI and subsequent prenatal consultation with a pediatric neurosurgeon. As the use of fetal MRI increases, there is greater impetus to understand the most common CNS structural anomalies diagnosed in utero, as well as their natural histories. METHODS The authors performed a single-center retrospective review of fetal MRI evaluations performed between January 2012 and December 2020. Children who underwent both prenatal and postnatal neurosurgical evaluations of CNS anomalies were included. Specific CNS anomalies on fetal MRI, associated extra-CNS findings, and suspicion for genetic abnormality or syndromes were noted. Postnatal clinical status and interventions were assessed. RESULTS Between January 2012 and December 2020, a total of 469 fetal MRI evaluations were performed; of these, 114 maternal-fetal pairs had CNS anomalies that warranted prenatal consultation and postnatal pediatric neurosurgical follow-up. This cohort included 67 male infants (59%), with a mean ± SD follow-up of 29.8 ± 25.0 months after birth. Fetal MRI was performed at 27.3 ± 5.8 weeks of gestational age. The most frequently reported CNS abnormalities were ventriculomegaly (57%), agenesis or thinning of the corpus callosum (33%), Dandy-Walker complex (DWC) (21%), neuronal migration disorders (18%), and abnormalities of the septum pellucidum (17%). Twenty-one children (18%) required neurosurgical intervention at a mean age of 2.4 ± 3.7 months. The most common surgical conditions included myelomeningocele, moderate to severe ventriculomegaly, encephalocele, and arachnoid cyst. Corpus callosum agenesis or thinning was associated with developmental delay (p = 0.02) and systemic anomalies (p = 0.05). The majority of prenatal patients referred for DWC had Dandy-Walker variants that did not require surgical intervention. CONCLUSIONS The most common conditions for prenatal neurosurgical assessment were ventriculomegaly, corpus callosum anomaly, and DWC, whereas the most common surgical conditions were myelomeningocele, hydrocephalus, and arachnoid cyst. Only 18% of prenatal neurosurgical consultations resulted in surgical intervention during infancy. The majority of referrals for prenatal mild ventriculomegaly and DWC were not associated with developmental or surgical sequelae. Patients with corpus callosum abnormalities should be concurrently referred to a neurologist for developmental assessments.

Focused ultrasound (FUS) is a noninvasive therapeutic technology with multiple pediatric clinical applications. The ability of focused ultrasound to target tissues deep in the body without exposing children to the morbidities associated with conventional surgery, interventional procedures, or radiation offers significant advantages. In 2021, there are 10 clinical pediatric focused ultrasound studies evaluating various musculoskeletal, oncologic, neurologic, and vascular diseases of which 8 are actively recruiting and 2 are completed. Pediatric musculoskeletal applications of FUS include treatment of osteoid osteoma and bone metastases using thermal ablation and high-intensity FUS. Pediatric oncologic applications of FUS include treatment of soft tissue tumors including desmoid tumors, malignant sarcomas, and neuroblastoma with high-intensity FUS ablation alone, or in combination with targeted chemotherapy delivery. Pediatric neurologic applications include treatment of benign tumors such as hypothalamic hamartomas with thermal ablation and malignant diffuse intrinsic pontine glioma with low-intensity FUS for blood brain barrier opening and targeted drug delivery. Additionally, low-intensity FUS can be used to treat seizures. Pediatric vascular applications of FUS include treatment of arteriovenous malformations and twin-twin transfusion syndrome using ablation and vascular occlusion. FUS treatment appears safe and efficacious in pediatric populations across many subspecialties. Although there are 7 Food and Drug Administration–approved indications for adult applications of FUS, the first Food and Drug Administration approval for pediatric patients with osteoid osteoma was obtained in 2020. This review summarizes the preclinical and clinical research on focused ultrasound of potential benefit to pediatric populations.

ObjectiveThis 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.MethodsWe 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-eventmethod andcalculated using the Kaplan-Meier survival method.ResultsData for 672 participants across 23 centers were collected on the specific hemispherotomy approach. Of these, 72 (10.7%) underwent vertical parasagittalhemispherotomy 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.SignificanceThis 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 arandomized clinical trial, are required to determine whether a vertical approach to hemispheric surgery provides superior long-term seizure outcomes.

OBJECTIVE Quadrigeminal cistern arachnoid cysts (QACs) are congenital lesions that can cause pineal region compression and obstructive hydrocephalus when sufficiently large. Management of these cysts is controversial and rates of reintervention are high. Given the limited data on the management of QACs, the authors retrospectively reviewed 20 years of cases managed at their institution and performed a literature review on this topic. METHODS The authors performed a retrospective analysis of patients treated for QAC at their institution between 2001 and 2021. They also performed a literature review of studies published between 1980 and 2021 that reported at least 5 patients treated for QACs. Patient characteristics, radiographic findings, management course, and postoperative follow-up data were collected and analyzed. RESULTS A total of 12 patients treated for a QAC at the authors’ institution met the inclusion criteria for analysis. Median age was 9 months, mean cyst size was 5.1 cm, and 83% of patients had hydrocephalus. Initial treatment was endoscopic fenestration in 92% of these patients, 27% of whom had an endoscopic third ventriculostomy (ETV) performed concurrently. Reintervention was required in 42% of patients. Cases that required reintervention had a statistically significant lower median age at the initial intervention (5 months) than the cases that did not require reintervention (24.33 months; p = 0.018). There were no major complications. At a mean follow-up of 5.42 years, 83% of patients had improvement or resolution of their symptoms. A literature review revealed 7 studies that met the inclusion criteria, totaling 108 patients with a mean age of 8.8 years. Eighty-seven percent of patients had hydrocephalus at presentation. Ninety-two percent of patients were initially treated with endoscopic fenestration, 44% of whom underwent concurrent ETV. Complications occurred in 17.6% of cases, and reintervention was required in 30.6% of cases. The most frequent reason for reintervention was untreated or unresolved hydrocephalus after the initial procedure. CONCLUSIONS Endoscopic fenestration is the most common treatment for QACs. While generally safe and effective, there is a high rate of reintervention after initial treatment of QACs, which may be associated with a younger age at the first intervention. Additionally, identifying patients who require initial treatment of hydrocephalus is critically important, as the literature suggests that untreated hydrocephalus is a common cause of reintervention.

OBJECTIVEPrevious models have been utilized in other low- and middle-income countries (LMICs) to explore and assess the cost, sustainability, and effectiveness of infant hydrocephalus treatment. However, similar models have not been implemented in Haiti due to a paucity of data, epidemiology, and outcomes for hydrocephalus. Therefore, the authors utilized previously described economic modeling to estimate the annual cost and benefit of treating hydrocephalus in infants at a neurosurgery referral center, Hospital Bernard Mevs (HBM), in Port-au-Prince, Haiti.METHODSThe authors conducted a retrospective review of data obtained in all children treated for hydrocephalus at the HBM from 2008 to 2015. The raw data were pooled with previously described surgical outcomes for hydrocephalus in other LMICs. Modeling was performed to determine outcomes, neurosurgical costs, disability-adjusted life years (DALYs), and economic benefits of Haitian hydrocephalus treatment during this time frame. Standard account methodology was employed to calculate cost per procedure. Using these formulas, the net economic benefit and cost/DALY were determined for hydrocephalus treatment at HBM from 2008 to 2015.RESULTSOf the 401 patients treated during the study period, 158 (39.4%) met criteria for postinfectious hydrocephalus, 54 (13.5%) had congenital hydrocephalus, 38 (9.5%) had myelomeningocele, 19 (4.7%) had aqueductal stenosis, and 132 (33%) were not placed into a category. Overall, 317 individuals underwent surgical treatment of their hydrocephalus, averting 3077 DALYs. The total cost of the procedures was $754,000, and the cost per DALY ranged between $86 and $245. The resulting net economic benefit for neurosurgical intervention ranged from $2.5 to $5.5 million.CONCLUSIONSThis work demonstrates the substantial economic benefit of neurosurgical intervention for the treatment of pediatric hydrocephalus at a single hospital in Haiti. Based on DALYs averted, the need for additional centers offering basic neurosurgical services is apparent. A single center offering these services for several days each month was able to generate between $2.5 to $5.5 million in economic benefits, suggesting the need to develop neurosurgical capacity building in Haiti. Ultimately, prevention, screening, and early surgical treatment of these infants represent a public health and socioeconomic requisite for Haiti.

Background: fMRI of mental phenomena is quite difficult to perform because lack of patient's cooperation or because the symptoms are stable. In some exceptional cases, however, fMRI and DTI are capable to provide insights on the anatomy of organic hallucinations. Methods: In this report we describe a 14-year-old boy with a left fronto-dorsal tumor who experienced chronic complex brief, frequent and repetitive complex visual and auditory hallucinations. His clinical picture included multiple and severe social and mood problems. During a presurgical fMRI mapping the patient complained of having the visual and auditory hallucinations. A block-design FMRI paradigm was obtained from the event timecourse. Deterministic DTI of the brain was obtained seeding the lesion as ROI. The patient underwent surgery and electrocorticography of the lesional area. Results: The fMRI of the hallucinations showed activation in the left inferior frontal gyrus (IFG) and the peri-lesional area. The tractography of the tumor revealed structural aberrant connectivity to occipital and temporal areas in addition to the expected connectivity with the IFG via the aslant fasciculus and homotopic contralateral areas. Intraoperative EEG demonstrated epileptic discharges in the tumor and neighboring areas. After resection, the patient's hallucinations stopped completely. He regained his normal social life and recover his normal mood. He remained asymptomatic for 90 days. Afterwards, hallucinations reappeared but with less intensity. Conclusions: To our knowledge, this is the first reported case of combined functional and structural connectivity imaging demonstrating brain regions participating in a network involved in the generation of complex auditory and visual hallucinations.

OBJECTIVEHydrocephalus is recognized as a common disabling pediatric disease afflicting infants and children disproportionately in the developing world, where access to neurosurgical care is limited and risk of perinatal infection is high. This surgical case series describes the Project Medishare Hydrocephalus Specialty Surgery (PMHSS) program experience treating hydrocephalus in Haiti between 2008 and 2015.METHODSThe authors conducted a retrospective review of all cases involving children treated for hydrocephalus within the PMHSS program in Port-au-Prince, Haiti, from 2008 through 2015. All relevant epidemiological information of children treated were prospectively collected including relevant demographics, birth history, hydrocephalus etiology, head circumference, and operative notes. All appropriate associations and statistical tests were performed using univariate and multivariate logistic regression analyses.RESULTSAmong the 401 children treated within PMHSS, postinfectious hydrocephalus (PIH) accounted for 39.4% (n = 158) of cases based on clinical, radiographic, and endoscopic findings. The majority of children with hydrocephalus in Haiti were male (54.8%, n = 197), born in the rainy season (59.7%, n = 233), and born in a coastal/inland location (43.3%, n = 61). The most common surgical intervention was endoscopic third ventriculostomy with choroid plexus cauterization (ETV/CPC) (45.7%, n = 175). Multivariate logistic regression analysis yielded coastal birth location (OR 3.76, 95% CI 1.16–12.18) as a statistically significant predictor of PIH. Increasing head circumference (adjusted OR 1.06, 95% CI 0.99–1.13) demonstrated a slight trend toward significance with the incidence of PIH.CONCLUSIONSThis information will provide the foundation for future clinical and public health studies to better understand hydrocephalus in Haiti. The 39.4% prevalence of PIH falls within observed rates in Africa as does the apparently higher prevalence for those born during the rainy season. Although PIH was the most frequent etiology seen in almost all birth locations, the potential relationship with geography noted in this series will be the focus of further research in an effort to understand the link between climate and PIH in Haiti. The ultimate goal will be to develop an appropriate public health strategy to reduce the burden of PIH on the children of Haiti.

Neurenteric cysts (NECs) of the central nervous system (CNS) are uncommon congenital entities arising from embryonal elements. Intracranial NECs in the pediatric population are rare. The authors describe the presentation, radiographic imaging, and pathologic findings of an 11-year-old boy with a right oculomotor nerve NEC. A literature review was performed to identify additional cases of pediatric intracranial NECs published in the English language, over the past 30 years (1990–2020). The authors discuss the presentation, investigations, management, and prognosis of this interesting entity. We describe an 11-year-old boy who presented to neurosurgical attention with disconjugate gaze, anisocoria, and ptosis. Magnetic resonance imaging (MRI) demonstrated a lobulated, cystic, and peripherally enhancing mass involving the right oculomotor nerve. The patient underwent pterional craniotomy for drainage of the cyst and subtotal resection of the cyst wall. The tan-colored mass was displacing the basilar artery, compressing the cerebral peduncle, and adherent to the inferior surface of the tentorium. The lesion was within the oculomotor nerve and splitting the fibers, and the cystic contents were thick and mucinous. Histopathological examination of the specimen demonstrated a thin fibrous cyst wall with scattered inflammatory cells and lined by simple columnar epithelium containing mucin. The lining cells were immunoreactive with epithelial membrane antigen (EMA) and pan-keratin AE1/AE3. The diagnosis of a NEC was rendered. A comprehensive literature review of pediatric intracranial NECs yielded 46 additional lesions published in the literature, involving the skull base, posterior fossa, cerebral convexity, and cranial nerves. NECs present with local mass effect and less commonly, with aseptic meningitis or intracystic hemorrhage. Maximal safe GTR remains the mainstay management, although cyst drainage and marsupialization, cyst shunting, and fenestration of cystic contents into the ventricle or basal cisterns have been reported with variable success. CNS NECs are rare congenital entities; although they occur less frequently in the intracranial components compared to the spine, their diagnosis and management should be considered for intracranial cystic lesions. Maximal safe GTR is the mainstay treatment and frequently yields favorable outcomes.

OBJECTIVE Infantile intracranial aneurysms are exceedingly rare. The goal of this study was to evaluate an institutional case series of infantile intracranial aneurysms, as well as those reported in the contemporary literature, to determine their demographics, presentation, management, and long-term outcome. METHODS A comprehensive literature review from 1980 to 2020 was performed to identify individual cases of intracranial aneurysms in the infantile population ≤ 2 years of age. Additional cases from the authors’ institution were identified during the same time period. An individual participant data meta-analysis (IPDMA) was performed, abiding by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Patient demographic, radiographic, and clinical information was obtained. Descriptive statistical data were recorded, and multivariate logistic regression analyses were performed. RESULTS Patient data were obtained for 133 patients from 87 articles in the literature. Ten additional patients at the authors’ institution were also identified, for a total of 143 patients included in the IPDMA. The majority (72.7%) of this cohort consisted of idiopathic aneurysms, while 13.3% were posttraumatic pseudoaneurysms, 9.8% were infectious mycotic aneurysms, and 4.2% were aneurysms associated with a systemic connective tissue disorder or vasculitis. The mean age at presentation was 6.6 months. The majority of infants (97.9%) harbored only 1 aneurysm, and hemorrhage was the most common presenting feature (78.3%). The mean aneurysm size was 14.4 mm, and giant aneurysms ≥ 25 mm comprised 12.9% of the cohort. Most aneurysms occurred in the anterior circulation (80.9%), with the middle cerebral artery (MCA) being the most commonly affected vessel (51.8%). Management strategies included open surgical aneurysm ligation (54.0%), endovascular treatment (35.0%), surgical decompression without aneurysm treatment (4.4%), and medical supportive management only (13.9%). Surgical aneurysm ligation was more commonly performed for MCA and anterior cerebral artery aneurysms (p = 0.004 and p = 0.015, respectively), while endovascular techniques were favored for basilar artery aneurysms (p = 0.042). The mean follow-up period was 29.9 months; 12.4% of the cohort died, and 67.0% had a favorable outcome (Glasgow Outcome Scale score of 5). CONCLUSIONS This study is, to the authors’ knowledge, the largest analysis of infantile intracranial aneurysms to date. The majority were idiopathic aneurysms involving the anterior circulation. Surgical and endovascular techniques yielded equally favorable outcomes in this cohort. Long-term outcomes in the infantile population compared favorably to outcomes in adults.

Objective: We describe a multicenter experience with VNS implantation in pediatric patients with epileptic encephalopathy. Our goal was to assess VNS efficacy and identify potential predictors of favorable outcome. Methods: This was a retrospective study. Inclusion criteria were: ≤18 years at the time of VNS implantation and at least one year of follow-up. All patients were non-candidates for excisional procedures. Favorable clinical outcome and effective VNS therapy were defined as seizure reduction >50%. Outcome data were reviewed at one, two, three and five years after VNS implantation. Fisher's exact test, Kaplan-Meier and multiple logistic regression analysis were employed. Results: Twenty-seven patients met inclusion criteria. Responder rate (seizure frequency reduction ≥ 50%) at one-year follow-up was 25.9%, and 15.3% at last follow-up visit. The only variable significantly predicting favorable outcome was time to VNS implantation, with the best outcome achieved when VNS implantation was performed within five years of seizure onset (overall response rate of 83.3% at one year of follow-up and 100% at five years). In total, 63% of patients evidenced improved QOL at last follow-up visit. Only one patient exited the study due to an adverse event at two years from implantation. Significance: Early VNS implantation within five years of seizure onset was the only predictor of favorable clinical outcome in pediatric patients with epileptic encephalopathy. Improved QOL and a very low incidence of adverse events were observed.

OBJECTIVE Scoliosis is common in patients with Chiari malformation type I (CM-I)–associated syringomyelia. While it is known that treatment with posterior fossa decompression (PFD) may reduce the progression of scoliosis, it is unknown if decompression with duraplasty is superior to extradural decompression. METHODS A large multicenter retrospective and prospective registry of 1257 pediatric patients with CM-I (tonsils ≥ 5 mm below the foramen magnum) and syrinx (≥ 3 mm in axial width) was reviewed for patients with scoliosis who underwent PFD with or without duraplasty. RESULTS In total, 422 patients who underwent PFD had a clinical diagnosis of scoliosis. Of these patients, 346 underwent duraplasty, 51 received extradural decompression alone, and 25 were excluded because no data were available on the type of PFD. The mean clinical follow-up was 2.6 years. Overall, there was no difference in subsequent occurrence of fusion or proportion of patients with curve progression between those with and those without a duraplasty. However, after controlling for age, sex, preoperative curve magnitude, syrinx length, syrinx width, and holocord syrinx, extradural decompression was associated with curve progression > 10°, but not increased occurrence of fusion. Older age at PFD and larger preoperative curve magnitude were independently associated with subsequent occurrence of fusion. Greater syrinx reduction after PFD of either type was associated with decreased occurrence of fusion. CONCLUSIONS In patients with CM-I, syrinx, and scoliosis undergoing PFD, there was no difference in subsequent occurrence of surgical correction of scoliosis between those receiving a duraplasty and those with an extradural decompression. However, after controlling for preoperative factors including age, syrinx characteristics, and curve magnitude, patients treated with duraplasty were less likely to have curve progression than patients treated with extradural decompression. Further study is needed to evaluate the role of duraplasty in curve stabilization after PFD.

Introduction. We surveyed nonretired American Academy of Pediatrics–member US pediatricians regarding common neurosurgical conditions, identifying specific areas of focus in education. Methods. Data were acquired via self-administered electronic questionnaire. Results. Of 505 total respondents, 56% reported neurology was not a required residency rotation, and 86% had diagnosed craniosynostosis, plagiocephaly, or macrocephaly. Craniosynostosis can mostly be diagnosed by physical examination alone, but almost 50% reported relying on skull X-rays. Fifty-four percent reported diagnosing ocular surface disease (OSD; with 15% to 40% not screening an infant despite well-established cutaneous markers). Seventy-four screened OSD in a patient with sacral dimple. Ninety-seven percent reported treating concussion, but nearly 25% did not manage these patients alone. Two out of 3 patients indicated head injury as most important for continuing education. Conclusion. Improved education for craniosynostosis, OSD, head injury, and concussion management are important for earlier diagnosis, management, and referral of some disorders, while decreasing resource utilization in others. These results should be used when considering pediatrician educational programs.

Magnetic Resonance-guided Laser Interstitial Thermal Therapy (MRgLITT) is an emerging minimally-invasive alternative to resective surgery for medically-intractable epilepsy. The precise lesioning effect produced by MRgLITT supplies opportunities to glean insights into epileptogenic regions and their interactions with functional brain networks. In this exploratory analysis, we sought to characterize associations between MRgLITT ablation zones and large-scale brain networks that portended seizure outcome using resting-state fMRI.Presurgical fMRI and intraoperatively volumetric structural imaging were obtained, from which the ablation volume was segmented. The network properties of the ablation volume within the brain's large-scale brain networks were characterized using graph theory and compared between children who were and were not rendered seizure-free.Of the seventeen included children, five achieved seizure freedom following MRgLITT. Greater functional connectivity of the ablation volume to canonical resting-state networks was associated with seizure-freedom (p 0.05, FDR-corrected). The ablated volume in children who subsequently became seizure-free following MRgLITT had significantly greater strength, and eigenvector centrality within the large-scale brain network.These findings provide novel insights into the interaction between epileptogenic cortex and large-scale brain networks. The association between ablation volume and resting-state networks may supply novel avenues for presurgical planning and patient stratification.

Background Medulloblastomas are common childhood central nervous system tumors that are prone to leptomeningeal spread. Intramedullary dissemination is rare with very few case reports existing in the available literature. Case Description The authors here present a case of a 14-year-old boy with Li-Fraumeni syndrome and medulloblastoma who underwent surgical resection of spinal intramedullary spread. Histopathology revealed the tumor to be anaplastic medulloblastoma, same as the intracranial lesions. Genetic testing of the metastatic deposit revealed loss of functions mutations in SUFU, NOTCH3, and TP53 and TERC amplification. An improvement in ambulatory function at short-term follow-up was noted before the patient died of disseminated disease. Conclusions Intramedullary metastasis of medulloblastoma remains a rare disease. Surgical resection might play a possible role in management in addition to radiation and chemotherapy.

When noninvasive modalities fail to adequately localize the seizure onset zone (SOZ) in children with medically refractory epilepsy, invasive interrogation with stereo-electroencephalography (SEEG) or subdural electrodes may be required. Our center utilizes SEEG for invasive monitoring in a carefully selected population of children, many of whom have seizures despite a prior surgical resection. We describe the cohort of patients who underwent SEEG in the first 5 years of its employment in our institution, almost half of which had a history of a failed epilepsy surgery.We retrospectively reviewed the records of the first 44 consecutive children who underwent SEEG at Nicklaus Children's Hospital (Miami, Florida), a large, level 4 epilepsy referral center. Patient demographic, clinical, radiographic, and electrophysiological information was collected prospectively. Student's t-test was used for sampling of means and analysis of variance (ANOVA) for evaluation of variance beyond 2 means; chi-square test of independence was used to assess the relationship between categorical variables.There were 44 patients in this cohort, of whom 17 (38.6 %) were male. The mean age of seizure onset was 6.2 years. Twenty-one patients (47.7 %) had previously failed an epilepsy surgery. Patients with a history of prior epilepsy surgery failure were older at SEEG implantation (17.6 vs. 13.7 years; p = 0.043), were more likely to have SEEG for identification of resection margins (9 vs. 4; p = 0.034), and had fewer electrodes placed (5.9 vs. 7.5; p = 0.016). No difference was seen in complication rates between groups with only 3/297 electrodes placed associated with complications, all of which were minor. Post-SEEG, 29 (65.9 %) patients underwent focal resection, 7 patients had VNS insertion, 3 underwent RNS placement, and 5 had no further intervention. The majority of patients that underwent resection in both groups experienced an improvement in seizures (Engel class I-III), reported by 13/15 (86.7 %) in those naive to surgery and 10/14 (71.4 %) in those with prior surgical failure. Seizure-freedom was much lower in those with prior epilepsy surgery, seen in only 4/14 (28.6 %) versus 8/15 (53.3 %).Our data supports current literature on SEEG as a safe and effective method of electrophysiological evaluation in children naive to surgery and adds that it is a safe technique in children with a history of failed epilepsy surgery. There was no difference in complication rates, which were1 % in both groups. A favorable outcome was seen in the majority of patients in both groups; the seizure freedom rate, however, was much lower in those with prior epilepsy surgery.

OBJECTIVECombined endoscopic third ventriculostomy (ETC) and choroid plexus cauterization (CPC)—ETV/CPC— is being investigated to increase the rate of shunt independence in infants with hydrocephalus. The degree of CPC necessary to achieve improved rates of shunt independence is currently unknown.METHODSUsing data from a single-center, retrospective, observational cohort study involving patients who underwent ETV/CPC for treatment of infantile hydrocephalus, comparative statistical analyses were performed to detect a difference in need for subsequent CSF diversion procedure in patients undergoing partial CPC (describes unilateral CPC or bilateral CPC that only extended from the foramen of Monro [FM] to the atrium on one side) or subtotal CPC (describes CPC extending from the FM to the posterior temporal horn bilaterally) using a rigid neuroendoscope. Propensity scores for extent of CPC were calculated using age and etiology. Propensity scores were used to perform 1) case-matching comparisons and 2) Cox multivariable regression, adjusting for propensity score in the unmatched cohort. Cox multivariable regression adjusting for age and etiology, but not propensity score was also performed as a third statistical technique.RESULTSEighty-four patients who underwent ETV/CPC had sufficient data to be included in the analysis. Subtotal CPC was performed in 58 patients (69%) and partial CPC in 26 (31%). The ETV/CPC success rates at 6 and 12 months, respectively, were 49% and 41% for patients undergoing subtotal CPC and 35% and 31% for those undergoing partial CPC. Cox multivariate regression in a 48-patient cohort case-matched by propensity score demonstrated no added effect of increased extent of CPC on ETV/CPC survival (HR 0.868, 95% CI 0.422–1.789, p = 0.702). Cox multivariate regression including all patients, with adjustment for propensity score, demonstrated no effect of extent of CPC on ETV/CPC survival (HR 0.845, 95% CI 0.462–1.548, p = 0.586). Cox multivariate regression including all patients, with adjustment for age and etiology, but not propensity score, demonstrated no effect of extent of CPC on ETV/CPC survival (HR 0.908, 95% CI 0.495–1.664, p = 0.755).CONCLUSIONSUsing multiple comparative statistical analyses, no difference in need for subsequent CSF diversion procedure was detected between patients in this cohort who underwent partial versus subtotal CPC. Further investigation regarding whether there is truly no difference between partial versus subtotal extent of CPC in larger patient populations and whether further gain in CPC success can be achieved with complete CPC is warranted.

OBJECTIVEEndoscopic third ventriculostomy (ETV)/choroid plexus cauterization (CPC) has become an increasingly common technique for the treatment of infant hydrocephalus. Both flexible and rigid neuroendoscopy can be used, with little empirical evidence directly comparing the two. Therefore, the authors used a propensity score–matched cohort and survival analysis to assess the comparative efficacy of flexible and rigid neuroendoscopy.METHODSIndividual data were collected through retrospective review of infants younger than 2 years of age, treated at 1 of 2 hospitals: 1) Boston Children's Hospital, exclusively utilizing flexible neuroendoscopy, and 2) Nicklaus Children's Hospital-Jackson Memorial Hospital, exclusively utilizing rigid neuroendoscopy. Patient characteristics and postoperative outcomes were assessed. A propensity score model was developed to balance patient characteristics in the case mix.RESULTSA propensity score model for neuroendoscope type was developed with 5 independent variables: chronological age, sex, hydrocephalus etiology, prior CSF diversion, and prepontine scarring. Propensity score decile-adjusted and 1-to-1 nearest-neighbor matching analysis revealed that compared with flexible neuroendoscopy, rigid neuroendoscopy had an ETV/CPC failure odds ratio (OR) of 1.43 (p = 0.31) and 1.31 (p = 0.47), respectively, compared with an unadjusted OR of 2.40 (p = 0.034). Furthermore, in a Cox regression analysis controlled by propensity score, rigid neuroendoscopy had a hazard ratio (HR) of 1.10 (p = 0.70), compared with an unadjusted HR of 1.61 (p = 0.031).CONCLUSIONSAlthough unadjusted analysis suggested worse ETV/CPC outcomes for infants treated by rigid neuroendoscopy, much of the difference could be attributed to the case mix and other predictors of outcome. A larger sample observational study or randomized controlled trials are required to provide evidence-based guidelines on ETV/CPC technique.

The appropriate imaging for pediatric patients being evaluated for suspected physical abuse depends on the age of the child, the presence of neurologic signs and symptoms, evidence of thoracic or abdominopelvic injuries, and whether the injuries are discrepant with the clinical history. The clinical presentations reviewed consider these factors and provide evidence-based consensus recommendations by the ACR Appropriateness Criteria ® Expert Panel on Pediatric Imaging.

ESES is a developmental epileptic disorder directly responsible for progressive encephalopathy and neurocognitive regression. The natural history, indications for surgical intervention, and predictors for favorable seizure and neuropsychological outcome remain unclear. We performed a retrospective review of children who underwent resective or disconnective surgery for ESES between January 2009 and July 2016 at a large tertiary pediatric center. Information on the patients' demographics, seizure semiology, radiographic and electrographic findings, and surgical management was collected. The primary outcome was seizure freedom at last follow-up visit, and secondary outcomes were neuropsychological improvement and electrographic ESES resolution. We identified 11 children who underwent surgery for ESES. The mean ages were 3.2 years for seizure onset, 7.1 years for formal ESES diagnosis, and 9.4 years for surgery. Seizure etiologies included cortical malformations (four patients), encephalomalacia and gliosis from prior hemorrhage or tumor resections (three patients), developmental porencephaly (one patient), and Rasmussen's encephalitis (one patient); the etiology was unknown in two children. Preoperatively, nine children had motor deficits, seven had speech and language delay, and three had visual field defects. All children had seizures and neuropsychological regression prior to surgical consideration. Focal cortical resections were performed in seven children, and hemispherectomies in four. Post-operatively, nine children experienced decreased seizure frequency, eight had neuropsychological improvement, and nine had resolution of electrographic ESES. Patients with poor surgical outcomes had more significant pre-operative comorbidities, in addition to bilateral ESES activity. In this case series, surgery for a carefully selected group of children with ESES is safe and feasible, yielding rates of seizure freedom and neuropsychological improvement that compare favorably with previous reports for antiepileptic drugs, benzodiazepines, and steroids. As we gain greater understanding into the management of ESES, surgery is an increasingly useful tool for patients with mild or moderate neurodevelopmental delay, focal epileptogenic foci, and hemi-ESES electrographic findings.

Background: A subset of pediatric epilepsy patients suffer from epilepsy syndromes related to multi-lobar or hemispheric epileptogenic networks and diseases. Most of these patients are refractory to anti-seizure drug treatment, resulting in persistent disabling seizures, neurocognitive delay and impaired quality of life. In well-selected patients, hemispheric surgery is associated with excellent seizure freedom rates and relatively low complication rates. Nevertheless, 30% of patients develop seizure recurrence after cerebral hemispheric surgery. The purpose of this study was 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 1237 hemispheric surgeries performed in pediatric patients across 31 centers and 12 countries to identify predictors of seizure freedom at 12 months after surgery. A multivariate logistic regression model was developed based on the entire dataset and its performance was evaluated based on cross-validation. Missing data was handled using multiple imputation techniques. Findings: Overall, 817/1237 (66%) hemispheric surgeries led to seizure freedom (median follow-up of 24 months), and 1050/1237 (85%) were seizure free at 12 months after surgery. The 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 non-hemispheric resective surgery were predictive of seizure freedom (AUC 0·72). A practical Hemispheric Surgery Outcome Prediction Scale (HOPS) score was devised based on these results that can be used to predict seizure freedom. Interpretation: Children most likely to benefit from hemispheric surgery can be selected and counselled 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. Funding Statement: The authors stated: "None." Declaration of Interests: None of the authors has any conflict of interest to disclose. None of the authors have financial relationships relevant to this article. Gary W Mathern MD is partly supported by the Davies/Crandall Endowed Chair for epilepsy Research at UCLA. Dr. Phillip Pearl receives research support from the NIH, NSF, and Boston Healthcare Associates; royalty payments from Elsevier, Springer Pubs, and UpToDate; has served as a consultant for Agilis Biotherapeutics and GLG Health Care Council; and is Associate Editor for the Journal of Child Neurology and on the editorial boards of Annals Neurology, Epilepsia, Future Neurology, Music and Medicine, and Neurology. Dr. Jeffrey Ojemann is a shareholder of Therma Neurosciences, a grant recipient of the NIH/NSF, and serves on the advisory board for the Epilepsy Foundation of the Northwest. The views expressed in this article are not the official positions of any authors’ affiliated institutions. Ethics Approval Statement: The organizing center received IRB approval for this study and each contributing center obtained research ethics approval locally.

Treatment-resistant epilepsy is a common and debilitating neurological condition, for which neurosurgical cure is possible. Despite undergoing nearly identical ablation procedures however, individuals with treatment-resistant epilepsy frequently exhibit heterogeneous outcomes. We hypothesized that treatment response may be related to the brain regions to which MR-guided laser ablation volumes are functionally connected. To test this, we mapped the resting-state functional connectivity of surgical ablations that either resulted in seizure freedom (N = 11) or did not result in seizure freedom (N = 16) in over 1,000 normative connectomes. There was no difference seizure outcome with respect to the anatomical location of the ablations, and very little overlap between ablation areas was identified using the Dice Index. Ablations that did not result in seizure-freedom were preferentially connected to a number of cortical and subcortical regions, as well as multiple canonical resting-state networks. In contrast, ablations that led to seizure-freedom were more functionally connected to prefrontal cortices. Here, we demonstrate that underlying normative neural circuitry may in part explain heterogenous outcomes following ablation procedures in different brain regions. These findings may ultimately inform target selection for ablative epilepsy surgery based on normative intrinsic connectivity of the targeted volume.

BACKGROUND Methotrexate has been used for intrathecal administration in leukemia as well as embryonal CNS tumors in children. Concerns about neurologic side effects including leukoencephalopathy, demyelination, and seizures have limited the use of methotrexate following exposure to focal radiation. OBJECTIVE To evaluate and determine safety of Intraventricular administration of Methotrexate in pediatric patients with recurrent malignant brain tumors along with systemic Topotecan and Cyclophosphamide after exposure to prior radiation therapy. DESIGN/METHOD: Patients with recurrent cerebellar embryonal tumors after standard treatment that included radiation were enrolled on this IRB approved phase 2 study. An Ommaya reservoir was inserted in the lateral ventricle and used to administer 4 daily doses of methotrexate (2 mg/dose) along with (Topotecan [0.75mg/m2/day] and Cyclophosphamide [250 mg/m2/day]). A neurological evaluation was performed at baseline and daily during the intraventricular administration of the Methotrexate, this evaluation was repeated prior to each subsequent cycle and at completion of the protocol. RESULTS Three patients (age range 3–20) received 2–3 cycles of intra-Ommaya Methotrexate and Topotecan/Cyclophosphamide. No MRI demyelination or white matter changes were seen after completion of the intraventricular Methotrexate therapy. None of the patients enrolled on this trial had adverse effects related to the therapy regimen received. Clinical neurological status was unchanged during the entire course of the treatment and upon completion of the scheduled therapy. CONCLUSION Intraventricular administration of daily low dose Methotrexate is well tolerated in children with recurrent embryonal CNS tumors who had prior exposure to radiation.