Surgical treatment of children with intractable epilepsy involves a clinical approach that recognizes the neurobiology of the developing brain, and this influences the selection of candidates and timing of surgery. The goal of surgery is to reduce epilepsy-induced cognitive disabilities by stopping seizures as soon as possible. Failure of two to three antiepilepsy drugs identifies children with therapy-resistant epilepsy who should be evaluated at pediatric epilepsy centers that include surgery. In this article, the authors discuss the most common surgically treated pediatric etiologies, including cortical dysplasia, perinatal cerebral infarcts, and mesial temporal lobe lesions; less frequent etiologies include hemimegalencephaly, Rasmussen syndrome, Sturge-Weber syndrome, tuberous sclerosis complex, Landau-Kleffner syndrome, and hypothalamic hamartomas.
Pediatric epilepsy surgeries are often extratemporal and include focal, lobar, multilobar, and hemispheric resections that are curative and corpus callosotomy and vagus nerve stimulators that are palliative. Postsurgery seizure control is obtained in 60% to 80% of children with resective surgery, and operative morbidity and mortality is less than that associated with long-term uncontrolled seizures. After successful epilepsy surgery, neurodevelopment and quality of life are significantly improved. The authors recommend that children with therapy-resistant epilepsy from focal to hemispheric pathologies be promptly referred to pediatric epilepsy centers so that those with surgical syndromes can be identified and treated to prevent catastrophic epilepsy-induced encephalopathy.
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• Epilepsy neurosurgery in children strives to enhance cognitive development through early seizure control.
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• Epilepsy neurosurgery in children is not the treatment of last resort. Children should be referred for evaluation once they fail two antiepileptic drugs.
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• The most common substrate in pediatric epilepsy surgery is cortical dysplasia, followed by tumors. Hippocampal sclerosis is less frequent in children than in adult epilepsy surgery patients.
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• Success of epilepsy neurosurgery in children depends on removing the entire histopathological substrate.
Historical note and terminology
The development of pediatric epilepsy surgery centers is relatively new in clinical practice. Neurosurgery for epilepsy was initiated in Europe in the late 1800s and predates the development of clinical EEG and all of the antiepilepsy drugs used today. The earliest patients were mostly adults, and epilepsy surgery in children was relatively rare until the pioneering work of neurosurgeon Sidney Goldring (51). With the introduction of continuous EEG-video telemetry and modern neuroimaging techniques, such as MRI, FDG-PET, and ictal SPECT, the late 1980s saw the development of multidisciplinary specialty groups involving pediatric neurologists, neurosurgeons, psychologists, and psychiatrists focused on diagnosis as well as operative and nonoperative management of children with therapy-resistant epilepsy (88). Although the initial clinical protocols mirrored those used by adult surgery programs, over the past decade the conceptual approach and surgical treatment of children with medically refractory epilepsy has undergone major evolution (53; 101).
This article will highlight the characteristics that distinguish epilepsy surgery in children and the conceptual framework for making clinical decisions in the era of modern neuroimaging and electrodiagnostics. Our goal is to show that surgery for epilepsy in the pediatric population is not the treatment of last resort. Instead, it has an important therapeutic role in the early treatment of children with surgically remedial syndromes who are at risk for epilepsy-induced cognitive and behavioral disabilities.