Hemisferotomía peri-insular y Hemisferectomía funcional pediátrica para el tratamiento de la epilepsia severa refractaria al tratamiento médico: comparación de dos técnicas / Peri-insular Hemispherotomy and Pediatric Functional Hemispherectomy for the Treatment of Severe Epilepsy Refractory to Medical Treatment: Comparison of Two Techniques

Introducción. Desde que se describió por primera vez en la década de 1970, la hemisferectomía funcional ha sido una herramienta esencial en el tratamiento de la epilepsia refractaria al tratamiento médico secundaria a una enfermedad hemisférica unilateral difusa. Objetivos. Presentar nuestra experiencia con la técnica de hemisferectomía funcional (HF) y de hemisferotomía peri-insular modificada (HPI); describir además la técnica quirúrgica peri-insular, revisar los resultados después de la cirugía, y discutir las diferencias entre las técnicas en cuanto a complicaciones y resultados postoperatorios. Material y Métodos. Se realizo un estudio retrospectivo de 23 pacientes con epilepsia refractaria que fueron sometidos a hemisferectomía cerebral. Se realizó un análisis de las características clínicas, las imágenes, las técnicas quirúrgicas y los resultados postoperatorios. Analizamos tambien la técnica quirúrgica de la HPI modificada. Resultados. Entre 2000 y 2020, 23 pacientes pediátricos con convulsiones refractarias se sometieron a hemisferotomía (12 HF, 11 HPI modificada). El 91,3% de los pacientes estaban libres de crisis a los 6 meses, el 87% al año y el 78,3% en el último seguimiento. Ninguno de los 23 pacientes presentó Engel IV como resultado final. Se encontró que la HF presentó una duración quirúrgica estadísticamente más larga que la HPI (5 ± 1,5 frente a 3,83 ± 0,5 horas; p = <0,001). Como resultados generales, la esfera cognitiva mejoró en dos tercios de los pacientes (66,9%) y además demostramos una mejoría de la actividad motora en la mayoría de los pacientes, independientemente de la patología y la técnica quirúrgica. Conclusión. Cuando los pacientes se seleccionan adecuadamente, la hemisferectomía es un tratamiento eficaz para la epilepsia pediátrica resultante la marcada disfunción de un solo hemisferio cerebral, no sólo brindando al paciente una alta probabilidad de ausencia de convulsiones, sino también proporcionando una mejora en las habilidades motoras y cognitivas. En nuestro caso particular y en base al presente estudio, la HPI modificada resulta ser una técnica altamente efectiva. No solo tiene un tiempo quirúrgico más corto sino también una tasa de complicaciones muy baja

Background. Since it was first described in the 1970s, functional hemispherotomy has been an essential tool in treating disabling, medically refractory epilepsy resulting from diffuse unilateral hemispheric disease. Objectives. To report our experience with 23 patients who underwent hemispherotomy, both using the functional hemispherotomy (FH) as well as a modified peri-insular hemispherotomy (PIH) technique; to present the surgical technique, review outcomes following disconnection surgery and discuss the differences between the techniques when it comes to complications and postoperative results. Methods. A retrospective study of 23 patients with refractory seizures who underwent cerebral hemispherectomy. A thorough analysis of the clinical, imaging, surgical features and postoperative results was performed. We also review the surgical technique for the modified PIH technique. Results. Between 2000 and 2020, 23 pediatric patients with refractory seizures underwent hemispherotomy (12 FHs, 11 modified PIHs). 91.3% of patients were seizure free at 6 months, 87% at 1 year, and 78.3% at last follow-up. None of the 23 patients presented Engel IV outcome. FH was found to have statistically longer surgical duration (5 ± 1.5 vs 3.83 ± 0.5 hours; p = <0.001). Neurocognition was improved in two thirds of the patients (66.9%). Our study also shows improvement of motor activity in most of the patients, regardless of the pathology and surgical technique. Conclusion. When patients are wisely selected, the hemispherotomy procedure should be considered as a most attractive and curative treatment for children with refractory seizures, not only giving the patient a high chance of seizure freedom but also providing an improvement in motor and cognitive skills. In our case and based on the present study, the modified PIH proves to be a highly effective technique. It not only has a shorter surgical time but also a very low complication rate

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.