Seizure and developmental outcomes after hemispherectomy in children and adolescents with intractable epilepsy
Francisco Villarejo-Ortega1, Marta García-Fernández, Concepción Fournier-Del Castillo
1Neurosurgery Service, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.
Insights
Pediatric hemispherectomy outcomes vary by cause. Early surgery improves development in some cases, but severe pre-existing issues limit gains. Pathologic substrate is key to seizure control.
Area of Science:
- Pediatric Neurosurgery
- Epileptology
- Developmental Neuroscience
Background:
- Hemispherectomy is a surgical option for intractable pediatric epilepsy.
- Understanding outcomes based on underlying pathology is crucial for patient selection and management.
Purpose of the Study:
- To review pediatric hemispherectomy cases, analyzing pathology, epilepsy features, seizure control, and developmental outcomes pre- and post-surgery.
- To identify factors influencing seizure control and developmental trajectories after hemispherectomy.
Main Methods:
- Retrospective analysis of 17 pediatric patients undergoing hemispherectomy.
- Included patients had complete pre-surgical evaluation, minimum 12-month follow-up, and post-surgical neuropsychological assessments.
- Patient groups included Rasmussen encephalitis (RE), malformations of cortical development (MCD), and vascular lesions.
Main Results:
- Excellent seizure control (Engel Class I) achieved in all RE patients and 66.7% with vascular lesions.
- Pre-surgical contralateral EEG discharges predicted worse seizure outcomes in vascular lesion group.
- Malformations of cortical development (MCD) group had the poorest seizure outcomes.
- Early age at surgery correlated with better developmental outcomes (personal-social, motor, adaptive) in vascular lesion group.
- One patient with left RE showed pre-surgical language transfer to the right hemisphere.
- Patients with MCD and vascular lesions had persistent severe global developmental delay.
Conclusions:
- Pathologic substrate is the primary determinant of seizure outcome post-hemispherectomy.
- Significant post-surgical developmental improvements in MCD and vascular lesion groups are constrained by pre-existing neuropsychological deficits and slow maturation.
- Early surgical assessment is recommended to optimize seizure control and improve long-term autonomy and socialization.
Purpose:
The aim of this study is to describe a series of pediatric hemispherectomies, reviewing pathologic substrate, epilepsy characteristics and seizure outcome as well as developmental profiles, before and after surgery, in different domains.
Methods:
Seventeen patients with full pre-surgical work-up, minimum follow-up of 12 months, and at least one post-surgical neuropsychological evaluation were selected. Three had Rasmussen encephalitis (RE), five hemispheric malformations of cortical development (MCD), and nine hemispheric vascular lesions.
Results:
At latest follow-up, all patients with RE and 66.7 % of those with vascular lesions are in Engel's class I; in the latter group, pre-surgical independent contralateral EEG discharges statistically correlated with a worse seizure outcome. Patients with MCD showed the worst seizure outcome. Pre-surgical language transfer to the right hemisphere was confirmed in a boy with left RE, operated on at 6 years of age. Patients with MCD and vascular lesions already showed severe global developmental delay before surgery, which persists afterwards. A linear correlation was found between earlier age at surgery and better outcome in personal-social, gross motor, and adaptive domains, in the vascular lesions group. The case with highest cognitive improvement had continuous spike and wave during sleep on pre-surgical EEG.
Conclusions:
Pathologic substrate was the main factor related with seizure outcome. In children with MCD and vascular lesions, although developmental progression is apparent, significant post-surgical improvements are restricted by the severity of pre-surgical neuropsychological disturbances and a slow maturation. Early surgery assessment is recommended to enhance the possibilities for a better quality of life in terms of seizure control, as well as better autonomy and socialization.
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