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

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Background and Purpose Hemispherectomy reportedly produces remarkable results interms of seizure outcome and quality of life for medically intractable hemispheric epilepsy inchildren. 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 cognitivefunctions 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 followingepilepsy 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 ofinfarction 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 achievedindependent 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.

Abstract

Background and Purpose Hemispherectomy reportedly produces remarkable results interms of seizure outcome and quality of life for medically intractable hemispheric epilepsy inchildren. 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 cognitivefunctions 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 followingepilepsy 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 ofinfarction 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 achievedindependent 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.

참고문헌 (23)

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