Postoperative shunt failure following hemispherectomy in pediatric patients with pre-existing hydrocephalus.

OBJECTIVE: The risk of hydrocephalus following hemispherectomy for drug resistant epilepsy (DRE) remains high. Patients with pre-existing hydrocephalus pose a postoperative challenge, as maintaining existing shunt patency is necessary but lacks a clearly defined strategy. This study examines the incidence and predictors of shunt failure in pediatric hemispherectomy patients with pre-existing ventricular shunts. METHODS: We performed a retrospective chart review at our center to identify pediatric patients diagnosed with DRE who were treated with ventricular shunt prior to their first hemispherectomy surgery. Demographic and perioperative data were obtained including shunt history, hydrocephalus etiology, epilepsy duration, surgical technique, and postoperative outcomes. Univariate analysis was performed using Fisher's exact test and Pearson correlation, with Bonferroni correction to a = 0.00625 and a = 0.01, respectively. RESULTS: Five of nineteen (26.3%) patients identified with ventriculoperitoneal shunting prior to hemispherectomy experienced postoperative shunt malfunction. All 5 of these patients underwent at least 1 shunt revision prior to hemispherectomy, with a significant association between pre- and post-hemispherectomy shunt revisions. There was no significant association between post-hemispherectomy shunt failure and valve type, intraoperative shunt alteration, postoperative external ventricular drain placement, hemispherectomy revision, lateralization of shunt relative to resection, postoperative complications, or postoperative aseptic meningitis. There was no significant correlation between number of post-hemispherectomy shunt revisions and age at shunt placement, age at hemispherectomy, epilepsy duration, or shunt duration prior to hemispherectomy. CONCLUSIONS: Earlier shunt revision surgery may portend a subsequent need for shunt revision following hemispherectomy. These findings may guide neurosurgeons in counseling patients with pre-existing ventricular shunts prior to hemispherectomy surgery.

Hemimegalencephaly: A Systematic Comparison of Functional and Anatomic Hemispherectomy for Drug-Resistant Epilepsy.

BACKGROUND AND OBJECTIVES: Hemimegalencephaly (HME) is a rare diffuse malformation of cortical development characterized by unihemispheric hypertrophy, drug-resistant epilepsy (DRE), hemiparesis, and developmental delay. Definitive treatment for HME-related DRE is hemispheric surgery through either anatomic (AH) or functional hemispherectomy (FH). This individual patient data meta-analysis assessed seizure outcomes of AH and FH for HME with pharmacoresistant epilepsy, predictors of Engel I, and efficacy of different FH approaches. METHODS: PubMed, Web of Science, and Cumulative Index to Nursing and Allied Health Literature were searched from inception to Jan 13th, 2023, for primary literature reporting seizure outcomes in >3 patients with HME receiving AH or FH. Demographics, neurophysiology findings, and Engel outcome at the last follow-up were extracted. Postsurgical seizure outcomes were compared through 2-tailed t -test and Fisher exact test. Univariate and multivariate Cox regression analyses were performed to identify independent predictors of Engel I outcome. RESULTS: Data from 145 patients were extracted from 26 studies, of which 89 underwent FH (22 vertical, 33 lateral), 47 underwent AH, and 9 received an unspecified hemispherectomy with a median last follow-up of 44.0 months (FH cohort) and 45.0 months (AH cohort). Cohorts were similar in preoperative characteristics and at the last follow-up; 77% (n = 66) of the FH cohort and 81% (n = 38) and of the AH cohort were Engel I. On multivariate analysis, only the presence of bilateral ictal electroencephalography abnormalities (hazard ratio = 11.5; P = .002) was significantly associated with faster time-to-seizure recurrence. A number-needed-to-treat analysis to prevent 1 additional case of posthemispherectomy hydrocephalus reveals that FH, compared with AH, was 3. There was no statistical significance for any differences in time-to-seizure recurrence between lateral and vertical FH approaches (hazard ratio = 2.59; P = .101). CONCLUSION: We show that hemispheric surgery is a highly effective treatment for HME-related DRE. Unilateral ictal electroencephalography changes and using the FH approach as initial surgical management may result in better outcomes due to significantly lower posthemispherectomy hydrocephalus probability. However, larger HME registries are needed to further delineate the predictors of seizure outcomes.

Current state of hemispherectomy and callosotomy for pediatric refractory epilepsy in Denmark.

OBJECTIVE: To evaluate outcomes from hemispherectomy and callosotomy related to the need for anti-seizure medication (ASM), seizure frequency, and cognition. METHODS: A review of the medical charts of all Danish pediatric patients who underwent hemispherectomy or callosotomy from January 1996 to December 2019 for preoperative and postoperative ASM use, seizure frequency, and cognitive data. RESULTS: The median age of epilepsy onset was two years (interquartile range (IQR): 0.0-5.3) for the hemispherectomy patients (n = 16) and one year (IQR: 0.6-1.7) for callosotomy patients (n = 5). Median time from onset to final surgery was 3.4 years for hemispherectomy and 10.2 years for callosotomy, while the median follow-up time was 6.9 years and 9.0 years, respectively. Preoperatively, all patients had daily seizures and were treated with ≥ 2 ASM. Hemispherectomy resulted in a reduction in seizure frequency in 87.5 % of patients, with 78.6 % achieving seizure freedom. Furthermore, 81.3 % experienced a reduction in ASM use and 56.3 % stopped all ASM. Median IQ/developmental quotient (IQ/DQ) was low preoperatively (44.0 [IQR: 40.0-55.0]) and remained unchanged postoperatively (IQ change: 0.0 [IQR: -10.0-+4.0]). Callosotomy resulted in a seizure reduction of 86-99 % in four patients, and ASM could be reduced in three patients. Median IQ/DQ was 20.0 preoperatively (IQR: 20.0-30.0) and remained unchanged postoperatively (IQ change: 0.0 [IQR: 0.0]). CONCLUSION: Hemispherectomy and callosotomy result in a substantial reduction in seizure frequency and ASM use without deterioration of IQ. Extensive epilepsy surgery should be considered early in children with drug-resistant epilepsy.

The Important Role of Hemispherotomy for Rasmussen Encephalitis: Clinical and Functional Outcomes.

BACKGROUND: Rasmussen encephalitis (RE) is characterized by pharmacoresistant epilepsy and progressive neurological deficits concurrent with unilateral hemispheric atrophy. Evidence of an inflammatory autoimmune process has been extensively described in the literature; however, the precise etiology of RE is still unknown. Despite data supporting a beneficial effect of early immunosuppressive and immunomodulatory interventions, surgical disconnection of the affected hemisphere is considered the treatment of choice for these patients. The aim of this study was to report a series of children and adolescents who underwent hemispheric surgery (HS) for the treatment of RE, analyzing their clinical, electrographic, and neuroimaging features pre-operatively, as well as their postoperative status, including seizure and functional outcomes. METHODS: All patients with RE who underwent HS in the Epilepsy Surgery Center (CIREP) of the University Hospital of Ribeirão Preto Medical School, between 1995 and 2020 were retrospectively reviewed. Preoperative and postoperative analyses included gender; age at epilepsy onset; seizure semiology; seizure frequency; interictal and ictal electroencephalographic (EEG) findings; age at surgery; duration of epilepsy; surgical complications; duration of follow-up; histopathological findings; and postoperative seizure, cognitive, and functional outcomes. RESULTS: Forty-four patients were evaluated. Mean age at seizure onset and epilepsy duration was 6 years and 2.5 years, respectively. Mean age at surgery was 9 years, with an average follow-up ranging from 3 months to 23 years. All patients presented with severe epilepsy and distinct neurological abnormalities on MRI. Before HS, different degrees of abnormal intellectual performance as well as hemiparesis were seen in 86% and 90%, respectively. Histopathology examination confirmed this diagnosis in 95% patients. At the last follow-up, 68% of patients were seizure free, and 70% were classified as Engel Class I or II. Postoperatively, the cognitive status remained unchanged in 64% of patients. Likewise, the gross motor function remained unchanged in 54% of patients and 74% had functional hand ability after HS. CONCLUSIONS: Considering the progressive damage course of RE, hemispheric surgery should be offered to pediatric patients. It has manageable risks and results in good seizure outcome, and the preoperative functional status of these children is often preserved (even when the left hemisphere is involved), thus improving their quality of life.

Predictive factors of hydrocephalus development in pediatric patients undergoing hemispherectomy for intractable epilepsy.

OBJECTIVE: Hemispherectomy surgery is an effective procedure for pediatric patients with intractable hemispheric epilepsy. Hydrocephalus is a well-documented complication of hemispherectomy contributing substantially to patient morbidity. Despite some clinical and operative factors demonstrating an association with hydrocephalus development, the true mechanism of disease is incompletely understood. The aim of this study was to investigate a range of clinical and surgical factors that may contribute to hydrocephalus to enhance understanding of the development of this complication and to aid the clinician in optimizing peri- and postoperative surgical management. METHODS: A retrospective chart review was conducted on all pediatric patients younger than 21 years who underwent hemispherectomy surgery at the Cleveland Clinic between 2002 and 2016. Data collected for each patient included general demographic information, neurological and surgical history, surgical technique, pathological analysis, presence and duration of perioperative CSF diversion, CSF laboratory values obtained while an external ventricular drain (EVD) was in place, length of hospital stay, postoperative aseptic meningitis, and in-hospital surgical complications (including perioperative stroke, hematoma formation, wound breakdown, and/or infection). Outcomes data included hemispherectomy revision and Engel grade at last follow-up (based on the Engel Epilepsy Surgery Outcome Scale). RESULTS: Data were collected for 204 pediatric patients who underwent hemispherectomy at the authors' institution. Twenty-eight patients (14%) developed hydrocephalus requiring CSF diversion. Of these 28 patients, 13 patients (46%) presented with hydrocephalus during the postoperative period (within 90 days), while the remaining 15 patients (54%) presented later (beyond 90 days after surgery). Multivariate analysis revealed postoperative aseptic meningitis (OR 7.0, p = 0.001), anatomical hemispherectomy surgical technique (OR 16.3 for functional/disconnective hemispherectomy and OR 7.6 for modified anatomical, p = 0.004), male sex (OR 4.2, p = 0.012), and surgical complications (OR 3.8, p = 0.031) were associated with an increased risk of hydrocephalus development, while seizure freedom (OR 0.3, p = 0.038) was associated with a decreased risk of hydrocephalus. CONCLUSIONS: Hydrocephalus remains a prominent complication following hemispherectomy, presenting both in the postoperative period and months to years after surgery. Aseptic meningitis, anatomical hemispherectomy surgical technique, male sex, and surgical complications show an association with an increased rate of hydrocephalus development while seizure freedom postsurgery is associated with a decreased risk of subsequent hydrocephalus. These findings speak to the multifactorial nature of hydrocephalus development and should be considered in the management of pediatric patients undergoing hemispherectomy for medically intractable epilepsy.

Long-term seizure outcome and mobility after surgical treatment for Rasmussen encephalitis in children: A single-center experience.

OBJECTIVE: Rasmussen Encephalitis (RE) is a rare inflammatory neurodegenerative disease associated with refractory seizures, hemiparesis, and cognitive deterioration, due to lateralized cortical atrophy. Hemispheric surgery (hemispherotomy) is the mainstay of treatment, but its unavoidable motor deficits and lack of long-term data regarding seizure outcomes can make patients and families apprehensive to undergo this procedure. The present study aimed at analyzing the results of surgical treatment for RE from a motor and epilepsy standpoint, and mitigate such concerns. METHODS: Clinical and operative data were retrospectively collected from medical records of pharmacoresistant patients treated with functional hemispherectomy at a tertiary reference center for epilepsy surgery, during a 24-year period (1996-2020). Variables such as age of epilepsy onset, seizure semiology, seizure frequency, immunomodulatory therapy, age at surgery, duration of epilepsy, surgical procedures and complications, number of medications used preoperatively and postoperatively were described and statistically analyzed. RESULTS: Forty-three (43) patients were included in this study. Mean age of epilepsy onset was 6.14 years, the average interval between epilepsy onset and hemispherotomy was 2.21 years. and the mean age at surgery was 8.28 years. Thirty patients (69.7%) were Engel I at their last follow-up, of whom 23 (56.4%) were Engel Ia, within a mean follow-up of 11.3 years. Duration of epilepsy, seizure frequency, and age at surgery, among others, did not correlate with seizure outcome, except the use of immunotherapy which led to worse outcomes (p < .05). Also, after surgery, motor functionality was significantly recovered (i.e., most patients returned to their previous status) with time. SIGNIFICANCE: This study tackled some issues regarding the surgical treatment of this disease, particularly showing that hemispherotomy is safe and leads to potentially recoverable disability of motor functions while providing high rates of effective and long-lasting seizure control; therefore, early surgical indication should be warranted once medical refractoriness has been established.

Comparison of Hemispheric Surgery Techniques for Pediatric Drug-Resistant Epilepsy: An Individual Patient Data Meta-analysis.

BACKGROUND AND OBJECTIVES: Hemispheric surgery effectively treats unihemispheric pediatric drug-resistant epilepsy (DRE) by resecting and/or disconnecting the epileptic hemisphere. Modifications to the original anatomic hemispherectomy have generated multiple functionally equivalent, disconnective techniques for performing hemispheric surgery, termed functional hemispherotomy. While a myriad of hemispherotomy variants exist, all of them can be categorized according to the anatomic plane they are performed in, which includes vertical approaches at or near the interhemispheric fissure and lateral approaches at or near the Sylvian fissure. This meta-analysis of individual patient data (IPD) aimed to compare seizure outcomes and complications between the hemispherotomy approaches to better characterize their relative efficacy and safety in the modern neurosurgical treatment of pediatric DRE, given emerging evidence that outcomes may differ between them. METHODS: CINAHL, Embase, PubMed, and Web of Science were searched from inception to September 9, 2020, for studies reporting IPD from pediatric patients with DRE who underwent hemispheric surgery. Outcomes of interest were seizure freedom at last follow-up, time-to-seizure recurrence, and complications including hydrocephalus, infection, and mortality. The χ2 test compared the frequency of seizure freedom and complications. Multivariable mixed-effects Cox regression controlling for predictors of seizure outcome was performed on propensity score-matched patients to compare time-to-seizure recurrence between approaches. Kaplan-Meier curves were made to visualize differences in time-to-seizure recurrence. RESULTS: Fifty-five studies reporting on 686 unique pediatric patients treated with hemispheric surgery were included for meta-analysis. Among the hemispherotomy subgroup, vertical approaches resulted in a greater proportion of seizure free patients (81.2% vs 70.7%, p = 0.014) than lateral approaches. While there were no differences in complications, lateral hemispherotomy had higher rates of revision hemispheric surgery due to incomplete disconnection and/or recurrent seizures than vertical hemispherotomy (16.3% vs 1.2%, p < 0.001). After propensity score matching, vertical hemispherotomy approaches independently conferred longer time-to-seizure recurrence than lateral hemispherotomy approaches (hazard ratio 0.44, 95% CI 0.19-0.98). DISCUSSION: Among functional hemispherotomy techniques, vertical hemispherotomy approaches confer more durable seizure freedom than lateral approaches without compromising safety. Future prospective studies are required to definitively determine whether vertical approaches are indeed superior and how it should influence clinical guidelines for performing hemispheric surgery.

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.

Efficiency of surgery on posttraumatic epilepsy: a systematic review and meta-analysis.

Posttraumatic epilepsy (PTE) accounts for approximately 20% of structural epilepsy, and surgical intervention may be a potential treatment option for these patients. Therefore, the purpose of this meta-analysis is to evaluate the effectiveness of surgical interventions for the management of PTE. Four electronic databases (Pubmed, Embase, Scopus and Cochrane library) were searched to identify studies on surgical management of PTE. Seizures reduction rate were analyzed quantitatively in a meta-analysis. Fourteen studies involving 430 PTE patients were selected for analysis, out of which 12 reported on resective surgery (RS), 2 on vagus nerve stimulation (VNS), and 2 of the 12 RS studies reported that 14 patients underwent VNS. The seizure reduction rate for surgical interventions (both RS and VNS) was 77.1% (95% confidence interval [CI]: 69.8%-83.7%) with moderate heterogeneity (I2 = 58.59%, Phetero = 0.003). Subgroup analysis based on different follow-up times revealed that the seizure reduction rate was 79.4% (95% CI: 69.1%-88.2%) within 5 years and 71.9% (95% CI: 64.5%-78.8%) beyond 5 years. The seizure reduction rate for RS alone was 79.9% (95% CI: 70.3%-88.2%) with high heterogeneity (I2 = 69.85%, Phetero = 0.001). Subgroup analysis showed that the seizure reduction rate was 77.9% (95% CI: 66%-88.1%) within 5 years and 85.6% (95% CI: 62.4%-99.2%) beyond 5 years, with 89.9% (95% CI: 79.2%-97.5%) for temporal lobectomy and 84% (95% CI: 68.2%-95.9%) for extratemporal lobectomy. The seizure reduction rate for VNS alone was 54.5% (95% CI: 31.6%-77.4%). Surgical interventions appeared to be effective for PTE patients without severe complications, RS seemed more beneficial than VNS, while temporal lobectomy is more favorable than extratemporal resection. However, further studies with long-term follow-up data are needed to better understand the relationship between VNS and PTE.

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.

Proper Therapy Selection Improves Epilepsy Outcomes in Patients With Multilobar Sturge-Weber Syndrome.

BACKGROUND: Hemispherectomy is an optimal treatment for patients with Sturge-Weber syndrome (SWS) affecting the whole hemisphere; however, a consensus has not been reached regarding therapeutic choices for those with involvement of two to three lobes. In this study, we compared seizure and cognitive outcomes between medical and surgical treatment groups in patients with multilobar involvement. METHODS: We evaluated 50 patients with multilobar involvement. Surgical indications included (1) antiepileptic drug (AED)-resistant seizures; (2) developmental delay; and (3) cortical atrophy. Twenty-nine patients were classified in the medical treatment group (MTG), and 21 patients were in the surgical treatment group (STG). Seizure type and frequency, SWS electroencephalography score (SWS-EEGS), and pretherapeutic and posttherapeutic SWS neurological scores (SWS-NS) were compared between groups. Median ages at the initial evaluation of the MTG and STG were 4 and 2 years, and at the final evaluation were 13 and 17 years, respectively. RESULTS: The STG had a higher incidence (76.2%) of focal to bilateral tonic-clonic seizures and status epilepticus, although no difference in SWS-EEGS. Seizure and cognitive subcategories of SWS-NS at initial evaluation were worse in the STG (P = 0.025 and P = 0.007). The seizure subcategory in MTG and STG improved after therapy (P = 0.002 and P = 0.001). Cognition was maintained in MTG and improved in STG (P = 0.002). The seizure-free rates in MTG and STG were 58.6% and 85.7%, respectively. CONCLUSIONS: Appropriate therapeutic choices improved seizure outcomes. Although patients who required surgery had more severe epilepsy and cognitive impairment, surgery improved both.

Epileptiform abnormalities in the disconnected hemisphere are common in seizure-free patients after hemispherectomy

Objective: The spectrum of EEG abnormalities in the disconnected hemisphere in seizure-free patients after hemispherectomy has not been well characterized. Methods: Fifty consecutive patients who were seizure-free following hemispheric disconnection were identified. Data on preoperative clinical, EEG and neuroimaging findings and postoperative EEG findings and antiseizure medication status were collected. Results: Forty-seven patients (94%) had functional hemispherectomy, and three (6%) had more extensive tissue resection of the posterior quadrant or frontal region along with hemispheric disconnection. Etiologies included: residual effects from vascular lesions including perinatal stroke in 35 patients, Rasmussen encephalitis in six, malformation of cortical development in seven, and Sturge-Weber syndrome in two. Pre-operative EEG showed focal epileptiform discharges in the affected hemisphere in 26 patients and in both hemispheres in 19. Eleven patients had diffuse bisynchronous epileptiform discharges. Postoperative EEGs at six to 12 months after surgery showed slowing and attenuation of physiological rhythms on the operated side in all patients. Thirty-four patients (68%) had epileptiform discharges exclusively from the operated hemisphere, six (12%) had bilateral independent epileptiform discharges, nine (18%) had no epileptiform discharges on either side, and one (2%) had epileptiform discharges from the contralateral hemisphere only. Lateralized periodic discharges from the operated hemisphere were noted in three patients (6%). EEG seizures from the operated hemisphere without clinical signs were noted in four patients (8%). After a median follow-up of three years, 30 patients (60%) were off all antiseizure medications, including 8/9 (89%) patients with no epileptiform discharges, 20/34 (59%) patients with postoperative epileptiform discharges from the operated hemisphere, and 2/7 (28%) patients with contralateral discharges. Significance: The majority of patients who are seizure-free after disconnective hemispherectomy will continue to show epileptiform discharges in the operated hemisphere. The presence of such discharges should not preclude tapering, nor prompt restarting of antiseizure medication in seizure-free patients.

Seizure Outcomes and Reoperation in Surgical Rasmussen Encephalitis Patients.

BACKGROUND: Rasmussen encephalitis (RE) is a rare inflammatory disease affecting one hemisphere, causing progressive neurological deficits and intractable seizures. OBJECTIVE: To report long-term seizure outcomes, reoperations, and functional outcomes in patients with RE who underwent hemispherectomy at our institution. METHODS: Retrospective review was performed for all patients with RE who had surgery between 1998 and 2020. We collected seizure history, postoperative outcomes, and functional data. Imaging was independently reviewed in a blinded fashion by 2 neurosurgeons and a neuroradiologist. RESULTS: We analyzed 30 patients with RE who underwent 35 hemispherectomies (5 reoperations). Using Kaplan-Meier analysis, seizure-freedom rate was 81.5%, 63.6%, and 55.6% at 1, 5, and 10 years after surgery, respectively. Patients with shorter duration of hemiparesis preoperatively were less likely to be seizure-free at follow-up (P = .011) and more likely to undergo reoperation (P = .004). Shorter duration of epilepsy (P = .026) and preoperative bilateral MRI abnormalities (P = .011) were associated with increased risk of reoperation. Complete disconnection of diseased hemisphere on postoperative MRI after the first operation improved seizure-freedom (P = .021) and resulted in fewer reoperations (P = .034), and reoperation resulted in seizure freedom in every case. CONCLUSION: Obtaining complete disconnection is critical for favorable seizure outcomes from hemispherectomy, and neurosurgeons should have a low threshold to reoperate in patients with RE with recurrent seizures. Rapid progression of motor deficits and bilateral MRI abnormalities may indicate a subpopulation of patients with RE with increased risk of needing reoperation. Overall, we believe that hemispherectomy is a curative surgery for the majority of patients with RE, with excellent long-term seizure outcome.

Long term outcome of functional hemispherectomy for refractory epilepsy: Experience from a single center.

BACKGROUND: Hemispherectomy has an established role as a treatment of last resort in patients with unilateral hemispheric lesions suffering from refractory epilepsy. METHODS: Seven patients were evaluated at our Epilepsy Unit. We compared the seizure outcome at 6 months, 1, 2, 5 years post-surgery, as well as at end follow-up (mean 7.1 years) using Engel classification. Reduction of antiepileptic drugs (AEDs) was also assessed utilizing equal time frames. RESULTS: The mean age of seizure onset was 5.4 years. Engel I was achieved in 5 patients at 6 months (71.4%). Engel at 1 year was predicted by the Engel at 6 months (p=0.013) with a similar number of patients being classified as Engel I outcome. Engel at 2 years was also predicted by Engel at 6 months and at 1 year (p=0.030). At end follow-up only 3 patients (42.9%) remained categorized as Engel I outcome. There was a trend toward a stability in Engel classification. All patients with developmental causes for their epilepsy experienced some deterioration of the surgical outcomes. Conversely, all patients with acquired causes were stable throughout follow-up. Seizure outcome at 6 months was worse in the patients who had post-op complications (p=0.044). Adult and pediatric populations did not differ significantly in any tested variable. CONCLUSIONS: Hemispherectomy is a valuable resource for seizure control in properly selected patients. Engel patient's evolution could be predicted at 6 months interval. Hemispherectomy could be considered a useful attitude in difficult cases.

The influencing factors and changes of cognitive function within 40 Rasmussen encephalitis patients that received a hemispherectomy.

OBJECTIVE: To evaluate the influencing factors and cognitive functional changes in Rasmussen encephalitis (RE) patients who received a hemispherectomy. METHODS: Forty RE patients underwent a hemispherectomy with at least a 2 years follow- up were included in this study . Postoperative seizure outcomes were evaluated according to the Engle classification scale. Univariate analysis and a multivariate logistic regression model in a backward fashion were used to identify the potential predictors of cognitive function. RESULTS: All 40 patients had an Engle classification outcome at a 2 years follow-up. Univariate and multivariate analyses revealed that seizure duration (OR 10.06, 95% CI 1.54-3.85, p = 0.038), age at surgery (OR 3.06, 95% CI 1.21-3.56, p = 0.043), and MRI score (OR 0.09, 95% CI 0.01-0.67 p = 0.024) are associated with postoperative cognitive outcomes respectively. Moreover,  VIQ and PIQ were negatively correlated linearly with duration of seizures and MRI score. Patients with a good VIQ and PIQ before the operation were more likely to have a better VIQ and PIQ postoperatively (p < 0.001 and p < 0.001, respectively). And, operation side is an important factor affecting cognitive function; therefore, a left hemispherectomy has a greater impact on the patient's IQ and language. CONCLUSIONS: RE patients' cognitive dysfunctions are improved after a hemispherectomy. Right-side operation can achieve better postoperative cognitive outcomes especially in VIQ and language. A shorter duration of seizures, early age at surgery, and less severe brain atrophy suggest better cognitive outcomes after a hemispherectomy.

Neuropsychiatric Symptoms After Brain Tumor Resection in Children and Adolescents: A Scoping Review.

BACKGROUND: Brain tumors are one of the most common solid tumors in pediatric populations, with their treatments having significant neuropsychiatric impact. OBJECTIVE: The objective of this study was to review the literature on neuropsychiatric sequelae after surgical resection of brain tumors in children and adolescents. METHODS: Using a scoping method, we reviewed empirical articles describing pediatric patients with brain tumors who underwent partial or total resection and examined major neuropsychiatric domains postoperatively over time. RESULTS: The initial search yielded 15,543 articles. After duplicate removal, abstract screening, and review, 44 articles were included. Cognitive deficits were the most widely studied outcomes and found to be associated with tumor location, operative variables, perioperative complications, treatment types, and psychosocial factors. Cerebellar mutism, or posterior fossa syndrome, commonly co-occurred with emotional and behavioral dysregulation after posterior fossa resections. Depression, anxiety, and somatization were frequently grouped together as "distress," with higher rates among pediatric patients with brain tumor than among healthy peers. Problematic school behaviors, antisocial, and attention-deficit traits were increased; however, several other behaviors (e.g., risky sexual behaviors, substance use) were equal or lower when compared to peers. Posttraumatic stress disorder was highly prevalent and often interfered with social functioning. Delirium, eating disorders, and longer-term outcomes received inadequate attention. CONCLUSION: Identifying risk factors of neuropsychiatric sequelae and their impact after pediatric brain tumor resection is important for prognostication and the development of tailored management strategies for these children and adolescents.

De novo psychosis after left temporal lobectomy: a case of forced normalization?

Forced normalization is a clinical entity defined by the appearance of psychiatric disturbance following control of epileptic seizures that were previously uncontrolled. It was first described by Landolt in 1953. The first cases described were mostly psychosis, however, subsequent work suggested that any behavioural disturbance of acute/or subacute onset concomitant with seizure control could be considered as forced normalization. We report the case of a 65-year-old, right-handed Caucasian patient who was followed in the Epilepsy Centre of Marseille, for left temporal drug-resistant epilepsy. The frequency of seizures was one seizure per month at the time before surgery. Left anterior temporal lobectomy was proposed based on presurgical evaluation. The patient remained seizure-free after surgery, but he presented with an episode of acute psychosis three months after. At this point, EEG was performed, showing rare left temporal epileptiform activity mainly provoked by hyperventilation, with breach rhythm over the left temporal surgical. The appearance of acute psychosis after cessation of epileptic seizures and reduced epileptiform activity on the EEG led us to question the forced normalization process in this case. Another hypothesis would be the effect of surgery itself, since there is an increased risk of any psychiatric disturbance unrelated to seizure cessation during the postoperative period. In conclusion, psychosis in this case could have resulted from the combination of several factors, including the effect of surgery itself and seizure cessation. This case illustrates the need for specific psychiatric care in the perioperative period in patients with epilepsy.

[Lessons learnt from 101 hemispheric pediatric epilepsy surgeries part ii: pitfalls and complications]. / Uroki 101 gemisferotomii u detei s polusharnoi simptomaticheskoi epilepsiei. Chast' II: oslozhneniya i oshibki.

OBJECTIVE: To evaluate the spectrum of pitfalls and complications after hemisherotomy basing on a retrospective study of a large consecutive pediatric cohort of patients from a single institution. MATERIAL AND METHODS: One hundred and one patients (med. age - 43 months) with refractory seizures underwent hemispherotomy. Developmental pathology was the anatomical substrate of disorder in 42 patients. The infantile post-stroke scarring and gliosis was the origin of epilepsy in the majority of 43 cases with acquired etiology. The progressive pathology (RE, S-W and TS) was the etiology in the rest of children (16 cases). The lateral periinsular technique was used to isolate the sick hemisphere in 55 patients; the vertical parasagittal approach was employed in 46 cases. Median perioperative blood loss constituted 10.5 ml/kg, but was markedly larger in kids with hemimegaly (52.8 ml/kg); 57 patients needed hemotransfusion during surgery. Median length of stay in ICU was 14.7 hours, and the length of stay in the hospital until discharge - 6.5 days. Eight patients underwent second-look surgery to complete sectioning of undercut commissural fibers. FU is known in 91 patients (med. length - 1.5 years). RESULTS: Major surgical complications with serious hemorrhage and/or surgery induced life-threatening events developed in 7 patients (one of them has died on the 5th day post-surgery for the causes of brain edema and uncontrolled hyponatremia). Various early and late infectious complications were noted in 4 cases. Ten patients experienced new not anticipated but temporary neurological deficit. Nine patients needed shunting for the causes of hydrocephalus within several first months post-hemispherotomy. Early seizure onset was associated with probability of all complications in general (p=0.02), and developmental etiology - with intraoperative bleeding and hemorrhagic complications (p=0.03). CONCLUSION: Children with developmental etiology, particularly those with hemimegalencephaly, are most challengeable in terms of perioperative hemorrhage and serious complications. Patients with relapse or persisting seizures should be evaluated for the possibility of incomplete hemispheric isolation and have good chances to become SF by re-doing hemispherotomy.

Long-Term Patient-Reported Outcomes of Visual Field Defects and Compensatory Mechanisms in Patients After Cerebral Hemispherectomy.

BACKGROUND: In cases of intractable epilepsy resistant to drug therapy, hemispherectomy is often the only treatment option to mitigate seizures; however, the true long-term subjective visual outcomes are relatively unexplored. In this study, we sought to determine and characterize patient-reported visual function years after hemispherectomy. METHODS: This was an observational study conducted on a large cohort of children with seizure disorder treated with cerebral hemispherectomy. An online survey was sent to parents with questions to assess subjective visual function with a variety of questions from presence of visual field defects after hemispherectomy, to improvement over time, compensatory mechanisms used, and development of strabismus. RESULTS: This survey was emailed to 248 parents of previously evaluated children who agreed to be re-surveyed, 48 (20%) of which responded. The average age at hemispherectomy was approximately 5 (±4) years, and the average time after hemispherectomy was 7 (±5) years. Thirty-nine patients (81%) were seizure-free after 1 surgery and 85% (n = 41) were seizure-free after ≥1 surgeries. Thirty-four (71%) experienced a visual field defect after surgery, but 25 (52%) experienced subjective improvement over time. Thirty-eight (79%) used compensatory mechanisms, such as head tilting, with 16 (33%) patients experiencing subjective improvement over time. Twenty-seven (56%) patients experienced a decrease in visual acuity after surgery with 12 (25%) experiencing subjective improvement over time. CONCLUSION: In a large cohort examining patient-reported visual outcomes years after hemispherectomy, most patients experienced strabismus and/or visual field defects. However, more than half reported improvements and compensatory mechanisms (exotropic strabismus and ipsilateral esotropic strabismus) over time, presumably to enhance visual field function. By exploring subjective visual and cognitive function, this paper uniquely characterizes patient-reported improvements over time, and provides motivation for larger longitudinal studies using more quantitative measures of visual function and improvement after hemispherectomy.

Language after childhood hemispherectomy: A systematic review.

OBJECTIVE: To conduct a systematic review on language outcomes after left and right hemispherectomy in childhood, a surgical procedure that involves removing or disconnecting a cerebral hemisphere. METHODS: We searched MEDLINE, Embase, and PsycInfo for articles published between January 1, 1988, and May 16, 2019. We included (1) all types of observational studies; (2) studies in which hemispherectomy was performed before age 18 years; and (3) studies with standardized scores measuring receptive vocabulary, expressive vocabulary, sentence comprehension, and/or sentence production. We calculated mean z scores after left and right hemispherectomy in the whole group and within etiology-specific subgroups. RESULTS: Our search identified 1,096 studies, of which 17 were eligible. The cohort added up to 205 individuals (62% left hemispherectomy) assessed 1 to 15 years after surgery. In the left surgery group, all language skills were impaired (z scores <-1.5) except sentence comprehension. In the right surgery group, language performance was in the borderline range (z scores ∼ -1.5). Children with cortical dysplasia showed the worst outcomes irrespective of surgery side (z scores <-2.5). Individuals with left vascular etiology and right-sided Rasmussen syndrome showed the best outcomes. CONCLUSION: Evidence based on the largest patient cohort to date (205 participants) suggests that the risk of language impairment after hemispherectomy is high, with few exceptions. Etiology plays a major role in postsurgical plasticity. We recommend specialist evaluation of language skills soon after surgery to identify intervention targets. Large-scale studies examining outcomes in consecutive cases are still needed.