Summary 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.

Lennox–Gastaut syndrome (LGS) is a severe form of childhood onset epilepsy in which patients require multiple medications and may be candidates for palliative surgical intervention. In this meta‐analysis, we sought to evaluate the impact of palliative vagus nerve stimulation (VNS), corpus callosotomy (CC), and resective surgery (RS) by analyzing their impact on seizure control, antiepileptic drug (AED) usage, quality of life (QOL), behavior, cognition, prognostic factors, and complications. A systematic search of PubMed MEDLINE, Scopus, and Cochrane Database of Systematic Reviews was performed to find articles that met the following criteria: (1) prospective/retrospective study with original data, (2) at least one LGS surgery patient aged less than 18 years, and (3) information on seizure frequency reduction (measured as percentage, Engel class, or qualitative comment). Seizures were analyzed quantitatively in a meta‐analysis of proportions and a random‐effects model, whereas other outcomes were analyzed qualitatively. Forty studies with 892 LGS patients met the selection criteria, with 19 reporting on CC, 17 on VNS, four on RS, two on RS + CC, one on CC + VNS, and one on deep brain stimulation. CC seizure reduction rate was 74.1% (95% confidence interval [CI] = 64.5%–83.7%), and VNS was 54.6% (95% CI = 42.9%–66.3%), which was significantly different (p < .001). RS seizure reduction was 88.9% (95% CI = 66.1%–99.7%). Many VNS patients reported alertness improvements, and most had no major complications. VNS was most effective for atonic/tonic seizures; higher stimulation settings correlated with better outcomes. CC patients reported moderate cognitive and QOL improvements; disconnection syndrome, transient weakness, and respiratory complications were noted. Greater callosotomy extent correlated with better outcomes. AED usage most often did not change after surgery. RS showed considerable QOL improvements for patients with localized seizure foci. In the reported literature, CC appeared to be more effective than VNS for seizure reduction. VNS may provide a similar or higher level of QOL improvement with lower aggregate risk of complications. Patient selection, anatomy, and seizure type will inform decision‐making.

OBJECTIVEInformed consent has served as a main principle of medical ethics and laws in the United States. The 1986 American Association of Neurological Surgeons Code of Ethics implied medicolegal liability for the failure to obtain informed consent without providing practical guidance regarding the application of informed consent to individual patient encounters in a medicolegal environment. Here, the authors aimed to identify baseline patient recall after discussions with neurosurgeons and their capacity to provide informed consent, describe the effects of interventions to improve patient comprehension, and elucidate the role of informed consent in malpractice litigation in neurosurgery. Their findings may guide neurosurgeons in discussions to properly inform patients and reduce the risk of litigation.METHODSA systematic review was conducted to explore informed consent within neurosurgery and its application to medicolegal liability using the PubMed, Embase, and Scopus databases. Titles and abstracts from articles identified in the search were read and selected for full-text review. Studies meeting prespecified inclusion criteria were reviewed in full and analyzed for study design, aim, population, interventions, and outcomes.RESULTSOf 1428 resultant articles, 21 were included in the review. Baseline patient recall was low, particularly for risks and alternatives of treatments, and even decreased over time. Cognitive impairment was noted as a factor limiting the ability to provide informed consent. Interventions incorporating a combination of modalities in informed consent discussions, a specialized consent form with points for neurosurgeons to check off upon discussion, interactive websites, question prompt lists, and illustrations were found to be effective in improving patient knowledge. Lack of informed consent was a common factor for malpractice litigation. Spine surgery was particularly prone to costly lawsuits. Payments were generally greater for plaintiff verdicts than for settlements.CONCLUSIONSThe application of informed consent to patient encounters is an important facet of clinical practice. Neurosurgeons have a duty to provide patients with all pertinent information to allow them to make decisions about their care. The authors examined baseline patient comprehension and capacity, interventions to improve informed consent, and malpractice litigation; it appears that determining the proper capacity to provide informed consent and considering informed consent as a process that depends on the setting are important. There is room to improve the informed consent process centered on baseline patient health literacy and understanding as well as clear communication using multiple modalities.

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.

OBJECTIVENeural tube defects (NTDs) are common congenital neurological defects, resulting in mortality, morbidity, and impaired quality of life for patients and caregivers. While public health interventions that increase folate consumption among women who are or plan to become pregnant are shown to reduce folate-sensitive NTDs, public health policy reflecting the scientific evidence lags behind. The authors aimed to identify the types of policies applied, associated outcomes, and impact of folate fortification and supplementation on NTDs worldwide. By identifying effective legislation, the authors aim to focus advocacy efforts to more broadly effect change, reducing the burden of NTDs in neurosurgery.METHODSA systematic review was conducted exploring folate fortification and supplementation policies using the PubMed and Scopus databases. Titles and abstracts from articles identified were read and selected for full-text review. Studies meeting inclusion criteria were reviewed in full and analyzed for study design, aim, population, interventions, and outcomes.RESULTSOf 1637 resultant articles, 54 were included. Mandatory folate fortification was effective at reducing folate-sensitive NTDs. Mandatory fortification also decreased hospitalization rates and deaths after discharge and increased 1st-year survival for infants with NTDs. Recommended folate supplementation also resulted in decreased NTDs; however, issues with compliance and adherence were a concern and impacted effectiveness. Folate fortification and/or supplementation resulted in decreased NTD prevalence, although more change was attributed to fortification. Dual policies may hold the most promise. Furthermore, reductions in NTDs were associated with significant cost savings over time.CONCLUSIONSBoth mandatory folate fortification and recommended supplementation policies were found to effectively decrease folate-sensitive NTD rates when applied. A comprehensive approach incorporating mandatory folate fortification, appropriate folate supplementation, and improved infrastructure and access to prenatal care may lead to decreased NTDs worldwide. This approach should be context-specific, emphasize education, and account for regional access to healthcare and social determinants of health. With wide implications for NTDs, associated health outcomes, quality of life of patients and caregivers, and economic impacts, policy changes can drastically improve global NTD outcomes. As caretakers of children with NTDs, the authors as neurosurgeons advocate for a comprehensive policy, the engagement of stakeholders, and a broader global impact.

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OBJECTIVE Drug-resistant epilepsy (DRE) affects many children. Vagus nerve stimulation (VNS) may improve seizure control; however, its role in children with genetic etiologies of epilepsy is not well described. The authors systematically reviewed the literature to examine the effectiveness of VNS in this cohort. METHODS In January 2021, the authors performed a systematic review of the PubMed/MEDLINE, SCOPUS/Embase, Cochrane, and Web of Science databases to investigate the impact of VNS on seizure outcomes in children with genetic etiologies of epilepsy. Primary outcomes included seizure freedom rate, ≥ 90% seizure reduction rate, and ≥ 50% seizure reduction rate. Secondary outcomes were seizure severity and quality of life (QOL), including cognitive, functional, and behavioral outcomes. A random-effects meta-analysis was performed. RESULTS The authors identified 125 articles, of which 47 with 216 nonduplicate patients were analyzed. Common diagnoses were Dravet syndrome (DS) (92/216 patients [42.6%]) and tuberous sclerosis complex (TSC) (63/216 [29.2%]). Seizure freedom was not reported in any patient with DS; the pooled proportion (95% CI) of patients with ≥ 50% seizure reduction was 41% (21%–58%). Secondary cognitive outcomes of VNS were variable in DS patients, but these patients demonstrated benefits in seizure duration and status epilepticus. In TSC patients, the pooled (95% CI) seizure freedom rate was 40% (12%–71%), ≥ 90% seizure reduction rate was 31% (8%–56%), and ≥ 50% reduction rate was 68% (48%–91%). Regarding the secondary outcomes of VNS in TSC patients, several studies reported decreased seizure severity and improved QOL outcomes. There was limited evidence regarding the use of VNS to treat patients with other genetic etiologies of epilepsy, such as mitochondrial disease, Rett syndrome, Doose syndrome, Landau-Kleffner syndrome, Aicardi syndrome, Angelman syndrome, ring chromosome 20 syndrome, and lissencephaly; variable responses were reported in a limited number of cases. CONCLUSIONS The authors conducted a systematic review of VNS outcomes in children with genetic etiologies of DRE. Among the most studied conditions, patients with TSC had substantial seizure reduction and improvements in QOL, whereas those with DS had less robust seizure reduction. Increased testing, diagnosis, and long-term follow-up studies are necessary to better characterize VNS response in these children.

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