Behavioral and brain morphological changes before and after hemispherotomy.

Hemispherotomy is an effective surgery for treating refractory epilepsy from diffuse unihemispheric lesions. To date, postsurgery neuroplastic changes supporting behavioral recovery after left or right hemispherotomy remain unclear. In the present study, we systematically investigated changes in gray matter volume (GMV) before and after surgery and further analyzed their relationships with behavioral scores in two large groups of pediatric patients with left and right hemispherotomy (29 left and 28 right). To control for the dramatic developmental effect during this stage, age-adjusted GMV within unaffected brain regions was derived voxel by voxel using a normative modeling approach with an age-matched reference cohort of 2115 healthy children. Widespread GMV increases in the contralateral cerebrum and ipsilateral cerebellum and GMV decreases in the contralateral cerebellum were consistently observed in both patient groups, but only the left hemispherotomy patients showed GMV decreases in the contralateral cingulate gyrus. Intriguingly, the GMV decrease in the contralateral cerebellum was significantly correlated with improvement in behavioral scores in the right but not the left hemispherotomy patients. Importantly, the preoperative voxelwise GMV features can be used to significantly predict postoperative behavioral scores in both patient groups. These findings indicate an important role of the contralateral cerebellum in the behavioral recovery following right hemispherotomy and highlight the predictive potential of preoperative imaging features in postoperative behavioral performance.

Development of an online calculator for the prediction of seizure freedom following pediatric hemispherectomy using the Hemispherectomy Outcome Prediction Scale (HOPS).

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

Epilepsy surgery in developmental and epileptic encephalopathies.

Developmental and epileptic encephalopathies (DEEs) present significant treatment challenges due to frequent, drug-resistant seizures and comorbidities that impact quality of life. DEEs include both developmental encephalopathy from underlying pathology and epileptic encephalopathy where seizures exacerbate cognitive and behavioral impairments. Classification by syndrome and etiology is essential for therapy and prognosis, with common syndromes like infantile epileptic spasms syndrome and Dravet syndrome having specific first-line treatments. Etiologies are predominantly genetic, structural, or combined, with targeted therapies increasingly available. Surgery aims to improve seizure control but also may improve development, if the epileptic encephalopathy can be ameliorated. Timely intervention can reduce seizures and epileptiform discharges, maximizing developmental potential and allowing reduction in antiseizure medication. In cases requiring extensive resections, new deficits may be offset by developmental gains. Studies indicate that parents are generally willing to accept some deficits for significant seizure reduction.

MRI and pathology comparisons in Rasmussen's encephalitis: a multi-institutional examination of hemispherotomy outcomes relative to imaging and histological severity.

PURPOSE: Rasmussen encephalitis (RE) is a very rare chronic neurological disorder of unilateral inflammation of the cerebral cortex. Hemispherotomy provides the best chance at achieving seizure freedom in RE patients, but with significant risks and variable long-term outcomes. The goal of this study is to utilize our multicenter pediatric cohort to characterize if differences in pathology and/or imaging characterization of RE may provide a window into post-operative seizure outcomes, which in turn could guide decision-making for parents and healthcare providers. METHODS: This multi-institutional retrospective review of medical record, imaging, and pathology samples was approved by each individual institution's review board. Data was collected from all known pediatric cases of peri-insular functional hemispherotomy from the earliest available electronic medical records. Mean follow-up time was 4.9 years. Clinical outcomes were measured by last follow-up visit using both Engel and ILAE scoring systems. Relationships between categorical and continuous variables were analyzed with Pearson correlation values. RESULTS: Twenty-seven patients met study criteria. No statistically significant correlations existed between patient imaging and pathology data. Pathology stage, MRI brain imaging stages, and a combined assessment of pathology and imaging stages showed no statistically significant correlation to post-operative seizure freedom rates. Hemispherectomy Outcome Prediction Scale scoring demonstrated seizure freedom in only 71% of patients receiving a score of 1 and 36% of patients receiving a score of 2 which were substantially lower than predicted. CONCLUSIONS: Our analysis did not find evidence for either independent or combined analysis of imaging and pathology staging being predictive for post peri-insular hemispherotomy seizure outcomes, prompting the need for other biomarkers to be explored. Our data stands in contrast to the recently proposed Hemispherectomy Outcome Prediction Scale and does not externally validate this metric for an RE cohort.

Impact of hemispherotomy on quality of life and burden of caregivers in children and adolescents: a retrospective observational study.

PURPOSE: To evaluate QOL and caregiver burden of children and teenagers submitted to hemispherotomy for pharmacoresistant epilepsy, by comparing pre and post-surgical intervention data. MATERIALS AND METHODS: Retrospective analysis of pediatric patients submitted to surgical hemispherotomy before intervention (preOP) and their follow-up at 6 months (6 M PO) and 2 years (2Y PO) after surgery. QOL was evaluated through the Quality of Life in Childhood Epilepsy (QVCE-50) questionnaire and caregiver burden, through the Zarit Burden Interview (ZBI) tool. RESULTS: Twenty-two patients were included in the study. Sixteen patients (72%) were classified as Engel I at 2Y PO follow-up. QVCE-50 scale showed improvement of total QOL at 2Y PO. In relation to QVCE-50-specific domains, there was an improvement in the physical domain and in the cognitive-education a decrease in psychological and a stabilization in social/familiar domain scores. The majority of caregivers classified their burden as mild to moderate, with no PO improvement. CONCLUSIONS: Hemispherotomy represents an effective seizure control treatment, as well as it contributes to improvement of QOL, particularly in the physical domain and in spite of children's physical and cognitive limitations. However, no improvement in caregiver burden was observed, probably due to the chronic condition of these patients, which might be worsened by social issues.

Hippocampal resection during hemispherotomy: is it needed?

PURPOSE: Hemispherotomy is an effective surgery for intractable pediatric hemispheric epilepsy. Over the years, the surgical goal has shifted from a complete hemispheric resection (anatomical hemispherectomy) to a disconnective hemispherotomy (DH). Multiple techniques for DH have been described, and often, anterior temporal lobectomy (ATL, with hippocampal resection) is performed. The goal of the current study is to assess the role of hippocampal resection in DH. METHODS: We retrospectively collected all clinical data of children (< 18 years old) who underwent DH between 2001 and 2022 at two tertiary large centers. Epilepsy status and surgical outcome were compared, based on whether the hippocampus was resected (as part of an ATL) or disconnected at the amygdala and atrial segment of the fornix (with no ATL). RESULTS: A total of 86 patients (32 females) were included. The most common epilepsy etiologies were stroke (31), Rasmussen's encephalitis (16), cortical dysplasia (10), and hemimegaloencephaly (9). The mean age at surgery was 7 (± 4.9) years. The average number of anti-seizure medications (ASMs) at surgery was 3 (± 1.2). Hemispherotomy techniques included peri-insular (54), vertical (23 [19 endoscopic + 4 parasagittal]), and trans-sylvian (9). The mean follow-up was 41.5 (± 38) months. Forty-three patients had hippocampal resection, and 43 patients had a hippocampal disconnection. Both groups had similar Engel outcome scores (p = 0.53). CONCLUSIONS: Disconnective hemispherotomy is highly effective for pediatric intractable hemispheric epilepsy. Our data suggest that the inclusion of hippocampal resection does not provide additional benefit.

Intraoperative motor-evoked potential with tetanic stimulation changes pre- and post-hemispherotomy.

BACKGROUND: Careful examination of motor-evoked potential (MEP) findings is critical to the safety of intraoperative neuromonitoring during neurosurgery. We reviewed the intraoperative MEP findings in a pediatric patient who had undergone hemispherotomy for refractory epilepsy. CASE DESCRIPTION: The patient was a 4-year-and-2-month-old boy with extensive right cerebral hemisphere, drug-resistant epilepsy, left upper and lower extremity paralysis, and cognitive impairment. We examined intraoperative MEP results both before and after hemispherotomy. Post-hemispherotomy and MEPs were successfully elicited through transcranial electrical stimulation (TES) but not via direct cortical stimulation on the right side. Furthermore, TES on the right side, following hemispherotomy, led to a reduction in the MEP amplification effect resulting from tetanic stimulation of the left unilateral median and tibial nerves. Conversely, we observed the effects of MEP amplification during TES on the left side after tetanic stimulation of these nerves. Postoperatively, the patient underwent magnetic resonance imaging and electroencephalogram examinations, confirming the anatomical and electrophysiological completeness of the dissection. Notably, the seizures disappeared, and no apparent complications were observed. CONCLUSION: Collectively, our findings suggest that TES can still activate deep structures and elicit MEPs, even in cases where the corticospinal connections to the posterior limb of the internal capsule are entirely severed. Thalamo-cortical interactions may affect the MEP amplification, observed during tetanic stimulation. Injury to the corticospinal tracts of the white matter may be obscured on conventional MEP findings; however, it may be identified by MEP changes in tetanic stimulation.

Predicting seizure outcomes and functional outcomes after hemispherotomy: are we any better?

INTRODUCTION: Present study attempted to analyze seizure freedom and detailed functional outcomes after functional hemispherotomy and utility of hemispherotomy outcome prediction scale (HOPS) scores in predicting outcomes. METHODS: Patients who underwent functional hemispherotomy were analyzed for clinical presentation, neuroimaging, seizure outcomes, and functional outcomes. RESULTS: A total of 76 procedures were performed on 69 patients. Mean age at the surgery was 8 ± 6.1 years. Fourteen patients were < 2 years. Age of onset epilepsy of the cohort was 2.0 ± 3.3 years. All had severe catastrophic epilepsy with multiple daily seizures. All patients had motor deficits with 36 (52%) patients had contralateral dysfunctional hand. Perinatal stroke (49%) was most common substrate followed by cortical malformations (21.7%). Eight patients had contralateral imaging abnormalities. Fifty-nine (86.76%) patients remained seizure free (Engle 1a) at 41 + -20.9 months. HOPS scores were available for 53 patients and lowest seizure outcome was 71% for HOPS score of 4. Lower HOPS scores predicted better seizure outcomes. Cortical malformations operated earlier than 2 years predicted poor seizure outcomes (66.6%). Positive functional outcomes are recorded in 80% of patients with 78% reporting improvement from the pre-surgical level. Five (7.2%) patients underwent shunt surgery. One mortality recorded. CONCLUSIONS: Hemispherotomy has excellent seizure outcomes. Early surgery in cortical malformations appears to be predictor of poorer seizure outcomes. HOPS score is a good tool to predict the seizure outcomes. Hemispherotomy is perceived to improve the Cognitive and functional performance.

Hemispherotomy in an infant with hemimegalencephaly and Ohtahara syndrome.

We represent the case of a premature twin neonate born from uncomplicated pregnancy who developed seizures at the age of 24 h. Two-dimensional ultrasound and magnetic resonance imaging revealed left-sided hemimegalencephaly. Further extensive diagnostic evaluation revealed a diagnosis of Ohtahara syndrome. Resistance of the seizures to antiepileptic therapy led to hemispherotomy that was performed at the age of 10 months. Our patient is now a 4-year-old child, walking, eating without a nasogastric tube, still with right hemiparesis and lateral strabismus but without seizures.

Not surgical technique, but etiology, contralateral MRI, prior surgery, and side of surgery determine seizure outcome after pediatric hemispherotomy.

OBJECTIVE: We aimed to assess determinants of seizure outcome following pediatric hemispherotomy in a contemporary cohort. METHODS: We retrospectively analyzed the seizure outcomes of 457 children who underwent hemispheric surgery in five European epilepsy centers between 2000 and 2016. We identified variables related to seizure outcome through multivariable regression modeling with missing data imputation and optimal group matching, and we further investigated the role of surgical technique by Bayes factor (BF) analysis. RESULTS: One hundred seventy seven children (39%) underwent vertical and 280 children (61%) underwent lateral hemispherotomy. Three hundred forty-four children (75%) achieved seizure freedom at a mean follow-up of 5.1 years (range 1 to 17.1). We identified acquired etiology other than stroke (odds ratio [OR] 4.4, 95% confidence interval (CI) 1.1-18.0), hemimegalencephaly (OR 2.8, 95% CI 1.1-7.3), contralateral magnetic resonance imaging (MRI) findings (OR 5.5, 95% CI 2.7-11.1), prior resective surgery (OR 5.0, 95% CI 1.8-14.0), and left hemispherotomy (OR 2.3, 95% CI 1.3-3.9) as significant determinants of seizure recurrence. We found no evidence of an impact of the hemispherotomy technique on seizure outcome (the BF for a model including the hemispherotomy technique over the null model was 1.1), with comparable overall major complication rates for different approaches. SIGNIFICANCE: Knowledge about the independent determinants of seizure outcome following pediatric hemispherotomy will improve the counseling of patients and families. In contrast to previous reports, we found no statistically relevant difference in seizure-freedom rates between the vertical and horizontal hemispherotomy techniques when accounting for different clinical features between groups.

Functional outcomes of pediatric hemispherotomy: Impairment, activity, and medical service utilization.

OBJECTIVE: The purpose of this study was to describe the functional outcomes of hemispherotomy in a pediatric cohort, including impairments, activity limitations, utilization of therapies and medical specialist services, and subsequent surgical intervention. METHODS: We conducted a retrospective review of patients who underwent hemispherotomy at UPMC Children's Hospital of Pittsburgh from 2001 to 2021. Data on impairments, activity limitations in mobility, self-care, and communication, and use of re/habilitative therapies and medical specialist services were collected pre-operatively and at 1, 5, 10, and 15 years postoperatively. Seizure outcomes were reported via Engel classification at the longest follow-up interval and subsequent surgical procedures were documented through the latest follow-up interval. RESULTS: A total of 28 patients who underwent hemispherotomy were assessed prior to surgery, 26 at 1 year post-op, 13 at 5 years, 9 at 10 years, and 5 at 15 years. Seizure outcomes at the longest follow-up interval showed that 84.6% of patients were seizure-free. Assessment of impairments to body structure & function from baseline to 1-year post-op revealed increased impairment in 73% of patients, while most patients saw no change in impairment at 5 years (69%), 10 years (100%), and 15 years (100%) post-op compared to the previous time point. Muscle tone abnormalities (100%), hemiparesis (92%), and visual field deficits (85%) were the most frequently observed impairments in the first year following surgery. Most patients saw no change in developmental or cognitive-affective impairments at 1 (65%), 5 (85%), 10 (89%), and 15 years (80%) post-op compared to the previous time point. The only qualitative reports of decreased ability occurred in 2/26 patients whose medical records indicated decreased mobility at the 1-year mark. All further qualitative reports of ability in mobility, self-care, and communication domains indicated increases or no change in ability for all patients at each of the subsequent follow-up intervals. Exploration of the utilization of re/habilitative therapy services shows that 84% of patients received at least one therapy service at baseline, 100% at1 year, 92% at 5 years, 100% at 10 years, and 80% at 15 years post-op. Patients were followed, on average (m), by multiple medical specialist services at baseline (m = 2.58) as well as the 1- (m = 1.70), 5- (m = 2.15), 10- (m = 3.00) and 15-year (m = 3.40) follow-up intervals. Following hemispherotomy, 15 (53.6%) patients required an average of 2.21 additional surgeries. Most often required was orthopedic surgical intervention (n = 16 procedures), followed by shunt placement (n = 7) and revision (n = 14) targeting hydrocephalus. SIGNIFICANCE: This retrospective study demonstrates expected increases in impairments such as hemiparesis and visual field deficits (i.e., homonymous hemianopia) in the context of increased activity and favorable seizure outcomes for 28 pediatric patients who underwent hemispherotomy for drug-resistant epilepsy. Most patients required rehabilitative therapies prior to surgery and continued to require these services post-operatively. Reported baseline functional status, the persistence of impairments following surgery, and comorbidities among this cohort underscore the medical complexity of this patient population and the importance of multidisciplinary care both pre-and post-operatively.

An Initial Experience of Completion Hemispherotomy via Magnetic Resonance-Guided Laser Interstitial Therapy.

INTRODUCTION: In carefully selected patients with medically refractory epilepsy, disconnective hemispherotomy can result in significant seizure freedom; however, incomplete disconnection can result in ongoing seizures and poses a significant challenge. Completion hemispherotomy provides an opportunity to finish the disconnection. We describe the use of magnetic resonance-guided laser interstitial thermal ablation (MRgLITT) for completion hemispherotomy. METHODS: Patients treated with completion hemispherotomy using MRgLITT at our institution were identified. Procedural and seizure outcomes were evaluated retrospectively. RESULTS: Five patients (3 males) underwent six MRgLITT procedures (one child treated twice) for completion hemispherotomy at a median age of 6 years (range 1.8-12.9). Two children had hemimegalencephaly, two had Rasmussen encephalitis, and one had polymicrogyria. All five children had persistent seizures likely secondary to incomplete disconnection after their functional hemispherotomy. The mean time from open hemispherotomy to MRgLITT was 569.5 ± 272.4 days (median 424, range 342-1,095). One patient underwent stereoelectroencephalography before MRgLITT. The mean number of ablation targets was 2.3 ± 0.47 (median 2, range 2-3). The mean length of the procedure was 373 min ± 68.9 (median 374, range 246-475). Four of the five patients were afforded improvement in their neurocognitive functioning and speech performance after ablation, with mean daily seizure frequency at 1 year of 1.03 ± 1.98 (median 0, range 0-5). Two patients achieved Engel Class I outcomes at 1 year after ablation, one was Engel Class III, and two were Engel Class IV. The mean follow-up time was 646.8 ± 179.5 days (median 634, range 384-918). No MRgLITT-related complications occurred. Delayed retreatment (>1 year) occurred in three patients: one child underwent redo ablation and two underwent anatomic hemispherectomy. CONCLUSION: We have demonstrated the feasibility of a minimally invasive approach for completion hemispherotomy using MRgLITT. Delayed retreatment was needed in three patients; thus, further study of this technique with comparison to other surgical techniques is warranted.

Effective Epilepsy Surgery for Post-Traumatic West Syndrome Following Abusive Head Trauma.

West syndrome, an infantile developmental and epileptic encephalopathy with a deleterious impact on long-term development, requires early treatment to minimize developmental abnormality; in such cases, epilepsy surgery should be considered a powerful therapeutic option. We describe a 10-month-old female admitted with West syndrome associated with a hemispheric lesion following abusive head trauma. Her seizures were suppressed by hemispherotomy at 12 months of age, leading to developmental improvement. Surgical treatment of West syndrome following traumatic brain injury has not been reported previously but is worth considering as a treatment option, depending on patient age and brain plasticity.

Peri-Insular Hemispherotomy: A Systematic Review and Institutional Experience.

INTRODUCTION: Peri-insular hemispherotomy (PIH) is a hemispheric separation technique under the broader hemispherotomy group, a surgical treatment for patients with intractable epilepsy. Hemispherotomy techniques such as the PIH, vertical parasagittal hemispherotomy (VPH), and modified-lateral hemispherotomy are commonly assessed together, despite significant differences in anatomical approach and patient selection. We aim to describe patient selection, outcomes, and complications of PIH in its own right. METHODS: A systematic review of the literature, in accordance with the Preferred Reporting Items of Systematic Reviews and Meta-Analyses (PRISMA) guidelines, was conducted, with searches of the PubMed and Embase databases. A local series including patients receiving PIH and followed up at the Queensland Children's Hospital between 2014 and 2020 was included. RESULTS: Systematic review of the literature identified 393 patients from 13 eligible studies. Engel class 1 outcomes occurred in 82.4% of patients, while 8.6% developed post-operative hydrocephalus. Hydrocephalus was most common in the youngest patient cohorts. Developmental pathology was present in 114 (40.8%) patients, who had fewer Engel 1 outcomes compared to those with acquired pathology (69.1% vs. 83.7%, p = 0.0167). The local series included 13 patients, 11/13 (84.6%) had Engel class 1 seizure outcomes. Post-operative hydrocephalus occurred in 2 patients (15.4%), and 10/13 (76.9%) patients had worsened neurological deficit. CONCLUSION: PIH delivers Engel 1 outcomes for over 4 in 5 patients selected for this procedure, greater than described in combined hemispherectomy analyses. It is an effective technique in patients with developmental and acquired pathologies, despite general preference of VPH in this patient group. Finally, very young patients may have significant seizure and cognitive benefits from PIH; however, hydrocephalus is most common in this group warranting careful risk-benefit assessment. This review delivers a dedicated PIH outcomes analysis to inform clinical and patient decision-making.

A simulation study to investigate the use of concentric tube robots for epilepsy surgery.

PURPOSE: Patients with pharmacoresistant refractory epilepsy may require epilepsy surgery to prevent future seizure occurrences. Conventional surgery consists of a large craniotomy with straight rigid tools with associated outcomes of morbidity, large tissue resections, and long post-operative recovery times. Concentric tube robots have recently been developed as a promising application to neurosurgery due to their nonlinear form and small diameter. The authors present a concept study to explore the feasibility of performing minimally invasive hemispherotomy with concentric tube robots. METHODS: A model simulation was used to achieve the optimal design and surgical path planning parameters of the concentric tube robot for corpus callosotomy and temporal lobectomy. A single medial burr hole was chosen to access the lateral ventricles for both white matter disconnections. RESULTS: The concentric tube robot was able to accurately reach the designated surgical paths on the corpus callosum and the temporal lobe. CONCLUSION: In a model simulation, the authors demonstrated the feasibility of performing corpus callosotomy and temporal lobectomy using concentric tube robots. Further advancements in the technology may increase the applicability of this technique for epilepsy surgery to better patient outcomes.

Comparison of the real-world effectiveness of vertical versus lateral functional hemispherotomy techniques for pediatric drug-resistant epilepsy: A post hoc analysis of the HOPS study.

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

Functional cognitive and language outcomes after cerebral hemispherectomy for hemimegalencephaly.

OBJECTIVE: Hemispheric surgeries are an effective treatment option to control seizures for children with hemimegalencephaly (HME); however, not enough is known about their cognitive outcomes. This study aimed to delineate the cognitive and language outcomes after hemispherectomy for HME and identify the clinical characteristics associated with cognition and language. METHODS: Data came from the Global Pediatric Epilepsy Surgery Registry, a patient-driven web-based registry for epilepsy surgery. We focused on children's functional status, assessed through parent-reports of cognitive and language skills. Parents also reported on their satisfaction with surgery, their child's quality of life, and various demographic, clinical, and surgery characteristics. RESULTS: Parents of 45 children (40% female) participated. Children were aged 2.6 (SD 6.5) months at seizure onset, 10.8 (SD 12.7) months at hemispherectomy, and 8.7 (SD 4.8) years at follow-up, at which point 68% were seizure-free. We found that at follow-up, 43% had average or mildly impaired cognition, 26% could speak age appropriately, and 21% had satisfactory reading skills. A total of 55%, 43%, and 17% of children first babbled, spoke their first words, and started speaking in sentences at an age-appropriate period, respectively. Children who had undergone a right hemisphere resection and those who were older at epilepsy onset were more likely to have better cognitive and language outcomes. SIGNIFICANCE: Children with HME have delayed language milestones and continue to require significant language and literacy support long-term after cerebral hemispherectomy.

Epilepsy surgery in infants up to 3 months of age: Safety, feasibility, and outcomes: A multicenter, multinational study.

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.

Hemispherotomy for Epilepsy: The Procedure Evolution and Outcome.

Hemispherectomy is a unique epilepsy surgery procedure that has undergone significant modification and evolution since Dandy's early description. This procedure is mainly indicated to treat early childhood and infancy medically intractable epilepsy. Various epileptic syndromes have been treated with this procedure, including hemimegalencephaly (HME), Rasmussen's encephalitis, Sturge-Weber syndrome (SWS), perinatal stroke, and hemispheric cortical dysplasia. In terms of seizure reduction, hemispherectomy remains one of the most successful epilepsy surgery procedures. The modification of this procedure over many years has resulted in lower mortality and morbidity rates. HME might increase morbidity and lower the success rate. Future studies should identify the predictors of outcomes based on the pathology and the type of hemispherectomy. Here, based on a literature review, we discuss the evolution of hemispherectomy techniques and their outcomes and complications.

Hemispherotomy for pediatric epilepsy: a systematic review and critical analysis.

PURPOSE: Several variations of functional disconnection surgery have been described for the treatment of lateralized, hemispheric, drug-resistant epilepsy in children. The purpose of this study is to investigate the existing literature regarding patient selection, approach, and outcomes after hemispherotomy. METHODS: A systematic review of the English literature through February 2019 was performed in accordance with the PRISMA statement. The articles were classified by level of evidence and summarized in an evidentiary table. Seizure outcomes, functional outcomes, surgical techniques, complications, and patient selection were critically analyzed. RESULTS: A total of 173 papers were reviewed, of which 37 met criteria of inclusion and exclusion. Thirteen studies were classified as level III evidence, the remaining reached level IV. Vertical and lateral hemispherotomy achieve similar rates of seizure freedom and functional outcomes, though parasagittal and interhemispheric approaches may have shorter operative times and less blood loss. Etiology, bilateral MRI abnormalities, and nonlateralizing EEG did not predict worse seizure or functional outcomes. CONCLUSIONS: Both vertical and lateral hemispherotomy approaches result in durable, reproducible benefits to epilepsy severity and functional status in appropriately selected pediatric patients.