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  • Author or Editor: Alexander G. Weilx
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Alexander G. Weil, Aria Fallah, Shelly Wang, George M. Ibrahim, Lior M. Elkaim, Prasanna Jayakar, Ian Miller, Sanjiv Bhatia, Toba N. Niazi, and John Ragheb

OBJECTIVE

Although hemispherectomy is an effective treatment for children with intractable hemispheric epilepsy syndromes, as many as 40% of patients eventually develop seizure recurrence. The causes of seizure recurrence in these patients are incompletely understood. The authors sought to evaluate the efficacy of hemispherectomy at their center and determine whether contralateral MRI abnormalities can predict seizure recurrence.

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A retrospective review of consecutive hemispherectomies performed at Miami Children’s Hospital between January 2000 and June 2014 was performed. Time-to-event analysis was performed. The “event” was defined as any seizures following resective epilepsy surgery (not including seizures in the first postoperative week and auras). Several preoperative variables were analyzed to determine their suitability to predict seizure recurrence following surgery.

RESULTS

Sixty-nine patients (44 boys) with a mean age of 8.2 ± 5.9 years (range 0.1–20.8 years) underwent 72 hemispherectomies; 67 of these were functional hemispherectomies, while another 5 were completion of a previous functional hemispherectomy (2 completions of functional hemispherectomies, 3 anatomical hemispherectomies). The duration of epilepsy was 5.8 ± 5.5 years with 66 cases (91.7%) having daily seizures. Etiology included stroke (n = 28), malformation of cortical development (n = 11), hemimegalencephaly (n = 11), encephalitis (n = 13), and other (n = 7). Engel class I outcome was achieved in 59 (86%) and 56 (81%) patients at 1 and 2 years of follow-up, respectively. The mean time to seizure recurrence was 33.5 ± 31.1 months. In univariate analyses, the absence of contralateral abnormalities on MRI (HR 4.09, 95% CI 1.41–11.89, p = 0.009) was associated with a longer duration of seizure freedom. The presence of contralateral MRI abnormalities was associated with contralateral ictal seizures on preoperative scalp EEG (p = 0.002). Fifteen patients experienced 20 complications (20/72, 27.8%), including the development of hydrocephalus necessitating CSF diversion in 9 cases (13%), hygroma in 1, hemispheric edema in 1, aseptic meningitis in 2, postoperative hemorrhage in 2, infection in 2, ischemic stroke in 2, and blood transfusion–contracted hepatitis C in 1 case.

CONCLUSIONS

Patients with bihemispheric abnormalities, as evidenced by contralateral MRI abnormalities, have a higher risk of earlier seizure recurrence following functional hemispherectomy.

Full access

Marie-Andrée Coulombe, Lior M. Elkaim, Naif M. Alotaibi, Daniel A. Gorman, Alexander G. Weil, Aria Fallah, Suneil K. Kalia, Nir Lipsman, Andres M. Lozano, and George M. Ibrahim

OBJECTIVE

Gilles de la Tourette syndrome (GTS) is a disorder characterized by motor and vocal tics. Although by definition the onset of GTS is before age 18 years, clinical trials of deep brain stimulation (DBS) have been conducted only in adults. Using individual participant data (IPD) meta-analysis methodology, the current study investigated the safety and efficacy of DBS as a treatment for GTS in children and youth.

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A systematic review with no date or language restrictions was performed according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) statement. Three electronic databases were searched: PubMed, EMBASE, and Web of Science. From 843 articles screened, the IPD of 58 children and youth (ages 12–21 years) extracted from 21 articles were collected and analyzed. A mixed-effects univariable analysis followed by multivariable hierarchical regression was performed using change in the Yale Global Tic Severity Scale (YGTSS) score as the primary outcome and reported measures of comorbidities as secondary outcomes.

RESULTS

The authors’ results showed an average improvement of 57.5% ± 24.6% across studies on the YGTSS. They also found that comorbid depression and stimulation pulse width each correlated negatively with outcome (p < 0.05). In patients with less severe GTS, greater improvements were evident following thalamic stimulation. More than one-quarter (n = 16, 27.6%) of participants experienced side effects, the majority of which were minor.

CONCLUSIONS

DBS in the pediatric population may be an effective option with a moderate safety profile for treatment of GTS in carefully selected children and youth. Large, prospective studies with long-term follow-up are necessary to understand how DBS influences tic symptoms and may alter the natural course of GTS in children.

Free access

Alexander G. Weil, John Ragheb, Toba N. Niazi, and Sanjiv Bhatia

Free access

Nathan A. Shlobin, Andrew Wang, H. Westley Phillips, Han Yan, George M. Ibrahim, Lior M. Elkaim, Shuang Wang, Xiaoyan Liu, Lixin Cai, Dang K. Nguyen, Aria Fallah, and Alexander G. Weil

OBJECTIVE

The prevalence of long-term postoperative sensorimotor deficits in children undergoing perirolandic resective epilepsy surgery is unclear. The risk of developing these deficits must be weighed against the potential reduction in seizure frequency after surgery. In this study, the authors investigated the prevalence of sensorimotor deficits after resective surgery at ≥ 1 year postoperatively.

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A systematic review and individual patient data meta-analysis was conducted using PubMed, Embase, and Scopus databases. Subgroups of patients were identified and categorized according to their outcomes as follows: group A patients were denoted as seizure free with no postoperative sensorimotor deficits; group B patients experienced seizure recurrence with no deficit; group C patients were seizure free with deficits; and group D patients were not seizure free and with deficits. Rates of sensory deficits were examined in patients undergoing postcentral gyrus resection, and rates of motor deficits were aggregated in patients undergoing precentral gyrus resection.

RESULTS

造成的797篇文章从数据库中搜索es, 6 articles including 164 pediatric patients at a mean age of 7.7 ± 5.2 years with resection for drug-resistant perirolandic epilepsy were included in the study. Seizure freedom was observed in 118 (72.9%) patients at a mean follow-up of 3.4 ± 1.8 years. In total, 109 (66.5%) patients did not develop sensorimotor deficits at last follow-up, while 55 (33.5%) had permanent deficits. Ten (14.3%) of 70 patients with postcentral gyrus resection had permanent sensory deficits. Of the postcentral gyrus resection patients, 41 (58.6%) patients were included in group A, 19 (27.1%) in group B, 7 (10.0%) in group C, and 3 (4.3%) in group D. Forty (37.7%) of 106 patients with precentral resections had permanent motor deficits. Of the precentral gyrus resection patients, 50 (47.2%) patients were in group A, 16 (15.1%) in group B, 24 (22.6%) in group C, and 16 (15.1%) in group D. Patients without focal cortical dysplasia were more likely to have permanent motor deficits relative to those with focal cortical dysplasia in the precentral surgery cohort (p = 0.02).

CONCLUSIONS

总的来说,58.6%的患者癫痫发作与自由out deficit, 27.1% were not seizure free and without deficit, 10.0% were seizure free but with deficit, and 4.3% were not seizure free and with deficit. Future studies with functional and quality-of-life data, particularly for patients who experience seizure recurrence with no deficits (as in group B in the present study) and those who are seizure free with deficits (as in group C) after treatment, are necessary to guide surgical decision-making.

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Rakan Bokhari, Lior M. Elkaim, Nathan A. Shlobin, Naif M. Alotaibi, Abdulrahman J. Sabbagh, Alexander G. Weil, Saleh Baeesa, Roy W. R. Dudley, Jeffrey Atkinson, and Jean-Pierre Farmer

OBJECTIVE

Delayed cerebral ischemia (DCI) associated with vasospasm is well described in the setting of aneurysmal subarachnoid hemorrhage (SAH). In addition, DCI is very infrequently encountered in patients who have undergone resection of a brain tumor with unclear pathophysiology. The occurrence of DCI in the pediatric population is exceedingly rare, and outcomes in this population have, to the authors’ knowledge, never been systematically reviewed. Therefore, the authors present what is to their knowledge the largest series of pediatric patients with this complication and systematically reviewed the literature for individual participant data.

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The authors conducted a retrospective review of 172 sellar and suprasellar tumors in pediatric patients who underwent surgery at the Montreal Children’s Hospital between 1999 and 2017 to identify cases of vasospasm occurring after tumor resection. Descriptive statistics, including patient characteristics, intraoperative and postoperative findings, and outcome status, were collected. A systematic review was also conducted using three databases (PubMed, Web of Science, Embase) to identify reported cases available in the literature of vasospasm after tumor resection in children and collect individual participant data on these patients for further analysis.

RESULTS

六个病人在蒙特利尔孩子的总结al were identified, with an average age of 9.5 years (range 6–15 years). The prevalence of vasospasm after tumor resection was 3.5% (6/172). Vasospasm in all 6 patients occurred after craniotomy was performed to treat a suprasellar tumor. The average interval from surgery to symptoms was 3.25 days (range 12 hours–10 days). The most common tumor etiology was craniopharyngioma, seen in 4 cases. Extensive tumor encasement of blood vessels requiring significant operative manipulation was described in all 6 patients. A rapid decrease in serum sodium (exceeding 12 mEq/L/24 hrs or below 135 mEq/L) was seen in 4 patients. On final follow-up, 3 patients were left with significant disability, and all patients had persistent deficits. A systematic review of the literature revealed a total of 10 other patients whose characteristics and treatment were compared with those of the 6 patients treated at Montreal Children’s Hospital.

CONCLUSIONS

Vasospasm after tumor resection in children and youth is likely a rare entity, with a prevalence of 3.5% in this case series. Suprasellar tumor location (particularly craniopharyngioma tumor etiology), significant encasement of blood vessels by the tumor, and postoperative hyponatremia may be predictive factors. Outcome is poor, with most patients having significant persistent neurological deficits.

Free access

Lior M. Elkaim, Farbod Niazi, Jordan J. Levett, Rakan Bokhari, Carolina Gorodetsky, Sara Breitbart, Fahad Alotaibi, Abdulelah A. Alluhaybi, Alexander G. Weil, Aria Fallah, Naif M. Alotaibi, and George M. Ibrahim

OBJECTIVE

This study aims to glean patient and caregiver perspectives surrounding deep brain stimulation (DBS) in children and youth through an analysis of patterns of social media usage.

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The authors performed a comprehensive search of the Twitter Application Programming Interface (API) database for all tweets about DBS use in children and youth, with no date restriction. Data pertaining to each tweet were extracted for analysis. Results were analyzed using qualitative and quantitative methodologies. These included thematic analysis of tweets, accounts, and descriptive statistics. Sentiment analysis of extracted tweets was also performed. A multivariable regression model was used to identify predictors of higher engagement metrics (likes, retweets, and quotes).

RESULTS

A comprehensive search of the Twitter database yielded 877 tweets from 816 unique accounts meeting study inclusion criteria. Most tweets were from patients or caregivers, researchers, or news media outlets. The most common themes among analyzed tweets were research discussing novel findings (45.2%) or personal experiences of patients or caregivers (27.4%). Sentiment analysis showed that 54.5% of tweets were positive, 35.1% were neutral, and 10.4% were negative. The presence of pictures or videos increased the tweet engagement count by an average of 10.5 (95% CI 7.3–13.6). Tweets about personal patient experiences (β = 6, 95% CI 0.95–12) and tweets tagging other accounts (β = 3.2, 95% CI 0.63–5.8) were also significantly associated with higher engagement metrics.

CONCLUSIONS

The current study is the first to assess patient and caregiver perspectives surrounding pediatric DBS through a comprehensive analysis of social media usage. Given the nascent field, social media presents an opportunity to share experiences and promote patient and healthcare professional education surrounding pediatric DBS.

Free access

Gabriel Crevier-Sorbo, Tristan Brunette-Clément, Edgard Medawar, Francois Mathieu, Benjamin R. Morgan, Laureen D. Hachem, Michael C. Dewan, Aria Fallah, Alexander G. Weil, and George M. Ibrahim

OBJECTIVE

癫痫不成比例地影响低收入和/或心肌梗死ddle-income countries (LMICs). Surgical treatments for epilepsy are potentially curative and cost-effective and may improve quality of life and reduce social stigmas. In the current study, the authors estimate the potential need for a surgical epilepsy program in Haiti by applying contemporary epilepsy surgery referral guidelines to a population of children assessed at the Clinique d’Épilepsie de Port-au-Prince (CLIDEP).

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作者回顾了812年儿科病人records from the CLIDEP, the only pediatric epilepsy referral center in Haiti. Clinical covariates and seizure outcomes were extracted from digitized charts. Electroencephalography (EEG) and neuroimaging reports were further analyzed to determine the prevalence of focal epilepsy or surgically amenable syndromes and to assess the lesional causes of epilepsy in Haiti. Lastly, the toolsforepilepsy instrument was applied to determine the proportion of patients who met the criteria for epilepsy surgery referral.

RESULTS

Two-thirds of the patients at CLIDEP (543/812) were determined to have epilepsy based on clinical and diagnostic evaluations. Most of them (82%, 444/543) had been evaluated with interictal EEG, 88% of whom (391/444) had abnormal findings. The most common finding was a unilateral focal abnormality (32%, 125/391). Neuroimaging, a prerequisite for applying the epilepsy surgery referral criteria, had been performed in only 58 patients in the entire CLIDEP cohort, 39 of whom were eventually diagnosed with epilepsy. Two-thirds (26/39) of those patients had abnormal findings on neuroimaging. Most patients (55%, 18/33) assessed with the toolsforepilepsy application met the criteria for epilepsy surgery referral.

CONCLUSIONS

The authors’ findings suggest that many children with epilepsy in Haiti could benefit from being evaluated at a center with the capacity to perform basic brain imaging and neurosurgical treatments.

Free access

Ann Mansur, Benjamin Morgan, Alexandre Lavigne, Nicolas Phaneuf-Garand, Jocelyne Diabira, Han Yan, Unni G. Narayanan, Darcy Fehlings, Golda Milo-Manson, Blythe Dalziel, Sara Breitbart, Claude Mercier, Dominic Venne, Pierre Marois, Alexander G. Weil, Jeffrey S. Raskin, Sruthi P. Thomas, and George M. Ibrahim

OBJECTIVE

In nonambulatory children with predominantly spastic cerebral palsy (CP), the authors compared care needs, symptom burden, and complications after surgical treatment with either intrathecal baclofen (ITB) pump insertion or selective dorsal rhizotomy (SDR). The patients were treated at two Canadian centers with variability in practice pertaining to these surgical options.

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The authors performed a retrospective analysis of nonambulatory children with predominantly spastic quadriplegic or diplegic CP who underwent treatment with ITB or SDR. These two strategies were retrospectively assessed by comparing patient data from the two treatment groups for demographic characteristics, outcomes, and complications. A partial least-squares analysis was performed to identify patient phenotypes associated with outcomes.

RESULTS

Thirty patients who underwent ITB and 30 patients who underwent SDR were included for analysis. Patients in the ITB group were older and had lower baseline functional status, with greater burdens of spasticity, dystonia, pain, deformity, bladder dysfunction, and epilepsy than patients in the SDR group. In addition, children who underwent SDR had lower Gross Motor Function Classification System (GMFCS) levels and were less likely to experience complications than those who underwent ITB. However, children treated with SDR had fewer improvements in pain than children treated with ITB. A single significant latent variable explaining 88% of the variance in the data was identified.

CONCLUSIONS

Considerable baseline differences exist within this pediatric CP patient population. Factors specific to individual children must be taken into account when determining whether ITB or SDR is the appropriate treatment.

Free access

Anthony C. Wang, George M. Ibrahim, Andrew V. Poliakov, Page I. Wang, Aria Fallah, Gary W. Mathern, Robert T. Buckley, Kelly Collins, Alexander G. Weil, Hillary A. Shurtleff, Molly H. Warner, Francisco A. Perez, Dennis W. Shaw, Jason N. Wright, Russell P. Saneto, Edward J. Novotny, Amy Lee, Samuel R. Browd, and Jeffrey G. Ojemann

OBJECTIVE

大脑后运动功能的潜在损失l hemispherectomy is a common cause of anguish for patients, their families, and their physicians. The deficits these patients face are individually unique, but as a whole they provide a framework to understand the mechanisms underlying cortical reorganization of motor function. This study investigated whether preoperative functional MRI (fMRI) and diffusion tensor imaging (DTI) could predict the postoperative preservation of hand motor function.

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Thirteen independent reviewers analyzed sensorimotor fMRI and colored fractional anisotropy (CoFA)–DTI maps in 25 patients undergoing functional hemispherectomy for treatment of intractable seizures. Pre- and postoperative gross hand motor function were categorized and correlated with fMRI and DTI findings, specifically, abnormally located motor activation on fMRI and corticospinal tract atrophy on DTI.

RESULTS

Normal sensorimotor cortical activation on preoperative fMRI was significantly associated with severe decline in postoperative motor function, demonstrating 92.9% sensitivity (95% CI 0.661–0.998) and 100% specificity (95% CI 0.715–1.00). Bilaterally robust, symmetric corticospinal tracts on CoFA-DTI maps were significantly associated with severe postoperative motor decline, demonstrating 85.7% sensitivity (95% CI 0.572–0.982) and 100% specificity (95% CI 0.715–1.00). Interpreting the fMR images, the reviewers achieved a Fleiss’ kappa coefficient (κ) for interrater agreement of κ = 0.69, indicating good agreement (p < 0.01). When interpreting the CoFA-DTI maps, the reviewers achieved κ = 0.64, again indicating good agreement (p < 0.01).

CONCLUSIONS

Functional hemispherectomy offers a high potential for seizure freedom without debilitating functional deficits in certain instances. Patients likely to retain preoperative motor function can be identified prior to hemispherectomy, where fMRI or DTI suggests that cortical reorganization of motor function has occurred prior to the operation.

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Han Yan, Taylor J. Abel, Naif M. Alotaibi, Melanie Anderson, Toba N. Niazi, Alexander G. Weil, Aria Fallah, John H. Phillips, Christopher R. Forrest, Abhaya V. Kulkarni, James M. Drake, and George M. Ibrahim

OBJECTIVE

In this systematic review and meta-analysis the authors aimed to directly compare open surgical and endoscope-assisted techniques for the treatment of sagittal craniosynostosis, focusing on the outcomes of blood loss, transfusion rate, length of stay, operating time, complication rate, cost, and cosmetic outcome.

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A literature search was performed in compliance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Relevant articles were identified from 3 electronic databases (MEDLINE, EMBASE, and CENTRAL [Cochrane Central Register of Controlled Trials]) from their inception to August 2017. The quality of methodology and bias risk were assessed using the Effective Public Health Practice Project Quality Assessment Tool for Quantitative Studies. Effect estimates between groups were calculated as standardized mean differences with 95% CIs. Random and fixed effects models were used to estimate the overall effect.

RESULTS

Of 316 screened records, 10 met the inclusion criteria, of which 3 were included in the meta-analysis. These studies reported on 303 patients treated endoscopically and 385 patients treated with open surgery. Endoscopic surgery was associated with lower estimated blood loss (p < 0.001), shorter length of stay (p < 0.001), and shorter operating time (p < 0.001). From the literature review of the 10 studies, transfusion rates for endoscopic procedures were consistently lower, with significant differences in 4 of 6 studies; the cost was lower, with differences ranging from $11,603 to $31,744 in 3 of 3 studies; and the cosmetic outcomes were equivocal (p > 0.05) in 3 of 3 studies. Finally, endoscopic techniques demonstrated complication rates similar to or lower than those of open surgery in 8 of 8 studies.

CONCLUSIONS

Endoscopic procedures are associated with lower estimated blood loss, operating time, and days in hospital. Future long-term prospective registries may establish advantages with respect to complications and cost, with equivalent cosmetic outcomes. Larger studies evaluating patient- or parent-reported satisfaction and optimal timing of intervention as well as heterogeneity in outcomes are indicated.

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