Functional hemispherotomy for refractory epilepsy in pediatric patients: the right person at the right time

Abstract Introduction Vagal nerve stimulators (VNS) are a nonpharmacological treatment for patients with refractory epilepsy. The VNS can decrease seizure frequency by over 75% in 40% of pediatric patients with refractory epilepsy. An underrecognized side effect is sleep disordered breathing (SDB). The purpose of this study was to demonstrate how a sensor placed adjacent to the VNS lead can distinguish whether SDB is due to VNS discharge. Methods Five pediatric patients (ages: 5–8) with refractory epilepsy with VNS were referred to our sleep center for concern for SDB. Each patient underwent a polysomnogram (PSG) that included a standard PSG montage with a surface electrode placed adjacent to their left lateral neck to detect VNS discharge. VNS associated apnea hypopnea index (vAHI) was calculated by determining the number of hypopneas and obstructive apneas occurring during VNS discharge. Results Of the 5 patients, three met pediatric criteria for obstructive sleep apnea (OSA). Patient 1 had an obstructive AHI (oAHI) of 21.3 events/hr with a vAHI accounting for 79% of the total (16.8 events/hr), patient 2 had an oAHI of 16.6 events/hr with a vAHI accounting for 57% of the total (9.5 events/hr), and patient 3 had an oAHI of 1.9 events/hr with vAHI accounting for 68% of the total (1.3 events/hr). Because of these findings, the VNS settings of all 3 patients were changed with the goal of reducing SDB due to VNS discharge. Upon repeat PSG, patient 2 had reduced OSA with an oAHI of 3 events/hr, with no events associated with VNS discharge. The remaining 2 patients did not exhibit VNS associated SDB, however, both experienced increased respiratory rate during VNS discharge. Conclusion We demonstrated that a surface electrode adjacent to the VNS is able to temporally co-register VNS discharges and enabled us to directly correlate SDB to VNS stimulation in 3 patients with refractory epilepsy. Because of our findings, we titrated the VNS parameters in all 3 patients, with one showing resolution of VNS associated SDB on repeat PSG. We propose that an added surface electrode to detect VNS discharge be considered as standard practice in PSG studies of patients with VNS. Support (if any):

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In Vivo Evaluation of Bioprinted Cardiac Patches Composed of Cardiac-Specific Extracellular Matrix and Progenitor Cells in a Model of Pediatric Heart Failure

Biomaterials Science ◽

10.1039/d1bm01539g ◽

2021 ◽

Author(s):

Donald Bejleri ◽

Matthew Robeson ◽

Milton Brown ◽

Jervaughn Hunter ◽

Joshua Maxwell ◽

...

Keyword(s):

Heart Failure ◽

Extracellular Matrix ◽

Congenital Heart ◽

Pediatric Patients ◽

Heart Defects ◽

In Vivo Evaluation ◽

Surgical Methods ◽

Pediatric Heart Failure ◽

The Right

Pediatric patients with congenital heart defects (CHD) often present with heart failure from increased load on the right ventricle (RV) due to both surgical methods to treat CHD and the...

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Frameless robot-assisted stereoelectroencephalography for refractory epilepsy in pediatric patients: accuracy, usefulness, and technical issues

Acta Neurochirurgica ◽

10.1007/s00701-018-3720-8 ◽

2018 ◽

Vol 160 (12) ◽

pp. 2489-2500 ◽

Cited By ~ 5

Author(s):

Santiago Candela-Cantó ◽

Javier Aparicio ◽

Jordi Muchart López ◽

Pilar Baños-Carrasco ◽

Alia Ramírez-Camacho ◽

...

Keyword(s):

Pediatric Patients ◽

Refractory Epilepsy ◽

Robot Assisted ◽

Technical Issues

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Lissencephaly, abnormal genitalia and refractory epilepsy: case report of XLAG syndrome

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x2006000600027 ◽

2006 ◽

Vol 64 (4) ◽

pp. 1023-1026 ◽

Cited By ~ 2

Author(s):

Mônica Jaques Spinosa ◽

Paulo Breno Noronha Liberalesso ◽

Simone Carreiro Vieira ◽

Alaídes Susana Fojo Olmos ◽

Alfredo Löhr Júnior

Keyword(s):

Case Report ◽

Corpus Callosum ◽

Refractory Epilepsy ◽

Genetic Disorder ◽

Ductus Arteriosus ◽

Ambiguous Genitalia ◽

Second Child ◽

Neonatal Onset ◽

The Right ◽

Male Genotype

INTRODUCTION: X-linked lissencephaly with ambiguous genitalia (XLAG) is a recently described genetic disorder caused by mutation in the aristaless-related homeobox (ARX) gene (Xp22.13). Patients present with lissencephaly, agenesis of the corpus callosum, refractory epilepsy of neonatal onset, acquired microcephaly and male genotype with ambiguous genitalia. CASE REPORT: Second child born to healthy nonconsanguineous parents, presented with seizures within the first hour of life that remained refractory to phenobarbital, phenytoin and midazolam. Examination identified microcephaly, axial hypotonia, pyramidal signs and ambiguous genitalia. EEG showed disorganized background activity and seizures starting at the right midtemporal, central and occipital regions. MRI showed diffuse pachygyria, moderate thickening of the cortex, enlarged ventricles, agenesis of the corpus callosum and septum pellucidum. Karyotype showed a 46,XY genotype. Additional findings were hypercalciuria, vesicoureteral reflux, patent ductus arteriosus and chronic diarrhea.

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A kiskorú páciensek jogainak helyzete az erdélyi egészségügyi ellátásban

Orvosi Hetilap ◽

10.1556/650.2018.30999 ◽

2018 ◽

Vol 159 (11) ◽

pp. 423-429

Author(s):

Csanád Albert-Lőrincz

Keyword(s):

Health Services ◽

Pediatric Patients ◽

Research Process ◽

Fundamental Rights ◽

Control Group ◽

Vulnerable Children ◽

Parents And Children ◽

Communication Difficulties ◽

The Right ◽

Phd Thesis

Abstract: This study summarizes the results of the author’s PhD thesis presenting the research process and the most important findings regarding the situation of Transylvanian healthcare. Our data are based on the knowledge and compliance of pediatric patients’ rights as they are seen by healthcare specialists, parents and children. A number of 751 persons were involved in the research: 200 healthcare specialists, 200 parents, 200 children aged from 7 to 17 years who have been experiencing health services at least three times over the past year and a control group of 151 children who did not have this kind of experience. Based on our data, it was possible to highlight the major problems encountered in children’s healthcare. We found that children are treated by obsolete principles of medical practice despite the fact that the rights of pediatric patients are well known to doctors. Neither the parents nor the children themselves are sufficiently aware of their rights. Healthcare professionals are not prepared to overcome communication difficulties due to the age-specific characteristics of children, so the fundamental rights of children are just partially enforced: children are usually heard, but not properly informed, their medical decisions are usually not taken into account violating the right to self-determination. The chances to access adequate health services are lower for vulnerable children living in poverty. Orv Hetil. 2018; 159(11): 423–429.

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Single and staged laser interstitial thermal therapy ablation for cortical tubers causing refractory epilepsy in pediatric patients

Neurosurgical FOCUS ◽

10.3171/2018.6.focus18228 ◽

2018 ◽

Vol 45 (3) ◽

pp. E9 ◽

Cited By ~ 13

Author(s):

Zulma Tovar-Spinoza ◽

Robert Ziechmann ◽

Stephanie Zyck

Keyword(s):

Laser Ablation ◽

Minimally Invasive ◽

Pediatric Patients ◽

Refractory Epilepsy ◽

Neuropsychiatric Symptoms ◽

Thermal Therapy ◽

Seizure Outcome ◽

Laser Interstitial Thermal Therapy ◽

Cortical Tubers ◽

The Mean

OBJECTIVEMagnetic resonance–guided laser interstitial thermal therapy (MRgLITT) is a novel, minimally invasive treatment for the surgical treatment of epilepsy. In this paper, the authors report on clinical outcomes for a series of pediatric patients with tuberous sclerosis complex (TSC) and medication-refractory epileptogenic cortical tubers.METHODSA retrospective chart review was performed at SUNY Upstate Golisano Children’s Hospital in Syracuse, New York. The authors included all cases involving pediatric patients (< 18 years) who underwent MRgLITT for ablation of epileptogenic cortical tubers between February 2013 and November 2015.RESULTSSeven patients with cortical tubers were treated (4 female and 3 male). The patients’ average age was 6.6 years (range 2–17 years). Two patients had a single procedure, and 5 patients had staged procedures. The mean time between procedures in the staged cases was 6 months. All of the patients had a meaningful reduction in seizure frequency as reported by Engel and ILAE seizure outcome classifications, and most (71.4%) of the patients experienced a reduction in AED burden. Three of the 4 patients who presented with neuropsychiatric symptoms had some improvement in these domains after laser ablation. No perioperative complications were noted. The mean duration of follow-up was 19.3 months (range 4–49 months).CONCLUSIONSLaser ablation represents a minimally invasive alternative to resective epilepsy surgery and is an effective treatment for refractory epilepsy due to cortical tubers.

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Safely repositioning dual-lumen ECMO cannulas with a transfemoral lasso snare

Perfusion ◽

10.1177/0267659120969031 ◽

2020 ◽

pp. 026765912096903

Author(s):

Erika R O’Neil ◽

Ryan D Coleman ◽

Adam M Vogel ◽

Corey A Chartan ◽

Kamlesh U Kukreja

Keyword(s):

Extracorporeal Membrane Oxygenation ◽

Real Time ◽

Internal Jugular Vein ◽

Pediatric Patients ◽

Jugular Vein ◽

Limb Ischemia ◽

Venovenous Extracorporeal Membrane Oxygenation ◽

The Right ◽

Fluoroscopy Guidance ◽

Vv Ecmo

Introduction: Dual-lumen cannulas were designed to provide venovenous extracorporeal membrane oxygenation (VV ECMO) with single-vessel access. Anatomic and size considerations may make appropriate placement challenging in children. Dual-lumen cannulas are repositioned in 20–69% of pediatric patients, which can be difficult without transient discontinuation of ECMO support. Methods: We repositioned three dual-lumen ECMO cannulas introduced via the right internal jugular vein using a transfemoral snare technique under real-time ultrasound and fluoroscopy. Results: Two of three patients were supported on VV ECMO and one on veno-veno-arterial (VV-A) ECMO. Two of the three patients had their dual-lumen cannula repositioned under ultrasound and fluoroscopy guidance and one was repositioned just with ultrasound. No patient experienced a complication from the transfemoral snare technique such as femoral hematoma, hemorrhage or limb ischemia. Conclusion: We describe three patients who successfully had dual-lumen cannulas repositioned without cessation of ECMO using a transfemoral “lasso” technique.

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Seizure Surgery

10.1093/med/9780190685157.003.0027 ◽

2018 ◽

Author(s):

Caroll N. Vazquez-Colon ◽

Srijaya K. Reddy

Keyword(s):

Pediatric Patients ◽

Perioperative Management ◽

Seizure Control ◽

Refractory Epilepsy ◽

Intraoperative Neuromonitoring ◽

Anesthetic Agents ◽

Anesthetic Drugs ◽

Seizure Surgery ◽

Antiepileptic Medications

Epilepsy is a disorder of the nervous system that affects over 2 million people worldwide, with the highest incidence in children. Surgical management of a child with refractory epilepsy may result in improved seizure control and better quality of life. The perioperative management of the pediatric patient for seizure surgery presents a considerable challenge to the anesthesiologist. Primary concerns include the interactions of antiepileptic medications with anesthetic drugs, the effects of anesthetic agents and medications on intraoperative neuromonitoring, and management of seizures while under anesthesia. This chapter will focus on anesthetic concerns and management for pediatric patients presenting for seizure surgery.

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Outcome of hemispheric surgeries for refractory epilepsy in pediatric patients

Child s Nervous System ◽

10.1007/s00381-006-0212-6 ◽

2006 ◽

Vol 23 (3) ◽

pp. 321-326 ◽

Cited By ~ 37

Author(s):

Vera Cristina Terra-Bustamante ◽

Luciana Midori Inuzuka ◽

Regina Maria França Fernandes ◽

Sara Escorsi-Rosset ◽

Lauro Wichert-Ana ◽

...

Keyword(s):

Pediatric Patients ◽

Refractory Epilepsy

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Periinsular anterior quadrantotomy: technical note

Journal of Neurosurgery Pediatrics ◽

10.3171/2017.8.peds17339 ◽

2018 ◽

Vol 21 (2) ◽

pp. 124-132 ◽

Cited By ~ 3

Author(s):

Giulia Cossu ◽

Sebastien Lebon ◽

Margitta Seeck ◽

Etienne Pralong ◽

Mahmoud Messerer ◽

...

Keyword(s):

Frontal Lobe ◽

Refractory Epilepsy ◽

Focal Cortical Dysplasia ◽

Seizure Outcome ◽

Illustrative Case ◽

Neural Pathways ◽

Functional Neuroanatomy ◽

The Right ◽

Epileptogenic Foci

Refractory frontal lobe epilepsy has been traditionally treated through a frontal lobectomy. A disconnective technique may allow similar seizure outcomes while avoiding the complications associated with large brain resections. The aim of this study was to describe a new technique of selective disconnection of the frontal lobe that can be performed in cases of refractory epilepsy due to epileptogenic foci involving 1 frontal lobe (anterior to the motor cortex), with preservation of motor function. In addition to the description of the technique, an illustrative case is also presented.This disconnective procedure is divided into 4 steps: the suprainsular window, the anterior callosotomy, the intrafrontal disconnection, and the frontobasal disconnection. The functional neuroanatomy is analyzed in detail for each step of the surgery. It is important to perform cortical and subcortical electrophysiological mapping to guide this disconnective procedure and identify eloquent cortices and intact neural pathways.The authors describe the case of a 9-year-old boy who presented with refractory epilepsy due to epileptogenic foci localized to the right frontal lobe. MRI confirmed the presence of a focal cortical dysplasia of the right frontal lobe. A periinsular anterior quadrant disconnection (quadrantotomy) was performed. The postoperative period was uneventful, and the patient was in Engel seizure outcome Class I at the 3-year follow-up. A significant cognitive gain was observed during follow-up.Periinsular anterior quadrantotomy may thus represent a safe technique to efficiently treat refractory epilepsy when epileptogenic foci are localized to 1 frontal lobe while preserving residual motor functions.

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