From Resection to Disconnection for Seizure Control in Pediatric Epilepsy Children

Epilepsy surgery revealed dramatically improved seizure outcomes over medical therapy in drug-resistant epilepsy patients. Children with epilepsy, however, have multiple epileptic focuses which require multilobar resection for better seizure outcome. Multilobar resection has not only the several severe surgical complications, such as hydrocephalus and shunt-related craniosynostosis, due to intracranial volume reduction. Isolation method (disconnection surgery) was progressively studied over epileptic focus removal (resective surgery) for seizure control. This concept was first introduced for functional hemispherotomy, and its primary principle is to preserve the vital vascularized brain that is functionally disconnected from the contralateral healthy brain. Currently in most epilepsy centers, the predominant disconnection surgical methods, including functional hemispherotomy, are continually being refined and are showing excellent results. They allow the functional isolation of the hemisphere or multi-lobe, affected by severe epilepsy. This review describes recent findings concerning the indication, surgical technique, seizure outcome and complications in several disconnection surgeries including the functional hemispherotomy for refractory pediatric epilepsy.

From Resection to Disconnection for Seizure Control in Pediatric Epilepsy Children

Epilepsy surgery revealed dramatically improved seizure outcomes over medical therapy in drug-resistant epilepsy patients. Children with epilepsy, however, have multiple epileptic focuses which require multilobar resection for better seizure outcome. Multilobar resection has not only the several severe surgical complications, such as hydrocephalus and shunt-related craniosynostosis, due to intracranial volume reduction. Isolation method (disconnection surgery) was progressively studied over epileptic focus removal (resective surgery) for seizure control. This concept was first introduced for functional hemispherotomy, and its primary principle is to preserve the vital vascularized brain that is functionally disconnected from the contralateral healthy brain. Currently in most epilepsy centers, the predominant disconnection surgical methods, including functional hemispherotomy, are continually being refined and are showing excellent results. They allow the functional isolation of the hemisphere or multi-lobe, affected by severe epilepsy. This review describes recent findings concerning the indication, surgical technique, seizure outcome and complications in several disconnection surgeries including the functional hemispherotomy for refractory pediatric epilepsy.

Hemispherotomy for hemisphericepilepsy: outcome and early follow up for complications / 基础医学与临床

Objective To investigate the results and complications of hemispherotomy for drug resistant hemispheric epilepsy.Methods The authors reviewed 5 patients who were diagnosed as drug resistant hemispheric epilepsy and operated in the neurosurgery department of Peking Union Medical College Hospital from 2011 to 2013.All the 5 patients were underwent hemispherotomy after carefully multidisciplinary pre-operation evaluation.Results All patients tolerated the procedure well and the postoperative course was smooth.All the 5 patients didn`t have seizure in the period of following up of 46 to 69 months.Conclusions According to literatures and the authors` experience, hemispherotomy is as safe and efficient for hemispheric epilepsy as hemispherectomy.It is most important advance for hemispherectomy.The procedure of hemispherotomy is complex but not very difficult, illustrating a good prospect of application and extension.

Long-Term Outcomes of Hemispheric Disconnection in Pediatric Patients with Intractable Epilepsy

BACKGROUND AND PURPOSE: Hemispherectomy reportedly produces remarkable results in terms of seizure outcome and quality of life for medically intractable hemispheric epilepsy in children. We reviewed the neuroradiologic findings, pathologic findings, epilepsy characteristics, and clinical long-term outcomes in pediatric patients following a hemispheric disconnection. METHODS: We retrospectively studied 12 children (8 males) who underwent a hemispherectomy at Asan Medical Center between 1997 and 2005. Clinical, EEG, neuroradiological, and surgical data were collected. Long-term outcomes for seizure, motor functions, and cognitive functions were evaluated at a mean follow-up of 12.7 years (range, 7.6-16.2 years) after surgery. RESULTS: The mean age at epilepsy onset was 3.0 years (range, 0-7.6 years). The following epilepsy syndromes were identified in our cohort: focal symptomatic epilepsy (n=8), West syndrome (n=3), and Rasmussen's syndrome (n=1). Postoperative histopathology of our study patients revealed malformation of cortical development (n=7), encephalomalacia as a sequela of infarction or trauma (n=3), Sturge-Weber syndrome (n=1), and Rasmussen's encephalitis (n=1). The mean age at surgery was 6.5 years (range, 0.8-12.3 years). Anatomical or functional hemispherectomy was performed in 8 patients, and hemispherotomy was performed in 4 patients. Eight of our 12 children (66.7%) were seizure-free, but 3 patients with perioperative complications showed persistent seizure. Although all patients had preoperative hemiparesis and developmental delay, none had additional motor or cognitive deficits after surgery, and most achieved independent walking and improvement in daily activities. CONCLUSIONS: The long-term clinical outcomes of hemispherectomy in children with intractable hemispheric epilepsy are good when careful patient selection and skilled surgical approaches are applied.

The usefulness of neuronavigation in functional hemispherectomy / Aplicabilidade da neuronavegação em hemisferectomia funcional

Catastrophic epileptic encephalopathies in children comprise devastating conditions that features cerebral dysfunction in association with refractory epileptic seizures. The diagnosis is based on the clinical findings, on magnetic resonance imaging (MRI) of the brain and on electroencephalographic findings. For these conditions, surgery remains essential for attaining seizure control. We report two cases of 5-year-old girls. The first one had a diagnosis of Rasmussen’s syndrome. The second one had a large porencephalic cyst secondary to perinatal cerebral ischemia. Despite trials of anticonvulsants, both patients deteriorated, and a functional hemispherectomy guided by neuronavigation was indicated and performed, with low morbidity and excellent seizure control. The neuronavigation proved to be a valuable guidance tool in performing the functional hemispherectomy, making the disconnections more accurate, and thus decreasing the surgical time and blood loss.

Aplicabilidade da neuronavegação em hemisferectomia funcional As encefalopatias epilépticas catastróficas da infância compreendem condições graves que associam disfunção cerebral e crises epilépticas refratárias. Seu diagnóstico é firmado com base nos dados clínicos e nos achados de ressonância magnética e eletrencefalográficos. Para algumas destas condições o tratamento cirúrgico continua sendo essencial para o controle das crises. Relatamos dois casos de pacientes de 5 anos. A primeira teve diagnóstico de síndrome de Rasmussen. A segunda tinha antecedentes de encefalopatia hipóxico-isquêmica perinatal. Ambas apresentaram epilepsia parcial refratária em associação com rápida deterioração neurológica, e foram submetidas à hemisferectomia funcional com auxílio da neuronavegação, com baixa morbidade e excelente controle das crises. A neuronavegação se mostrou como uma valiosa ferramenta na realização da hemisferectomia funcional, possibilitando desconexões mais precisas, menor tempo de cirurgia e menor perda sanguínea.

Hemispherotomy in Childhood Medically Refractory Epilepsy / 대한소아신경학회지

PURPOSE: Surgical approach with medically intractable epilepsy has increased with recent advances in neurodiagnostic modalities. This study was designed to review the clinical manifestations and surgical outcome of patients who received hemipherotomy. METHODS: We performed a retrospective study of 19 patients under 15 years old who had medically intractable epilepsy and underwent hemipherotomy at Severance Children's Hospital between 2003 and 2008. RESULTS: Eleven patients(57.9%) showed Engel Class I outcome, 6 patients(31.6%) showed Engel Class II and 1 patient(5.3%) showed Engel Class III outcomes. Preoperative evaluation revealed concordance in all 19 patients(100%) on MRI, 17 patients(89.5%) on long-term EEG monitoring, 17 patients(89.5%) on PET and 13 patients(68.4%) on interictal SPECT. Malformation of cortical development was the most common etiology(4 patients, 21.5%). Other etiologies included hemimegalencephaly, Sturge-Weber syndrome, hemorrhage, infarction and schizencephaly(2 patients each, 10.5%). The most common pathologic finding was cortical dysplasia(9 patients, 49.4%). Other pathological findings included microdysgenesis(2 patients, 10.5%), gliosis(4 patients, 21.1%), leukomalacia, Lafora body and calcification in cortex(1 patient each, 5.3%). CONCLUSION: Surgical outcome of hemipherotomy in infant and children were favorable. Better evaluation through utilization of advanced neurodiagnostic modalities including EEG monitoring and neuroradiologic studies will greatly improve postsurgical outcome of hemipherotomy.