Epilepsy Surgery in Children with Genetic Etiologies: A Prospective Evaluation of Current Practices and Outcomes

•Surgery was effective in a majority of children with epilepsy from a genetic cause•Surgery improved seizure frequency despite diffuse or multifocal MRI or EEG findings•Seizure freedom was achieved in ⅓ of this cohort; median follow-up 11 months•>50% seizure reduction was present in ⅔ of children; median follow-up of 11 months•There is a long latency between recognizing drug resistance and referral for surgery : This study assesses current practices and outcomes of epilepsy surgery in children with a genetic etiology. It explores the pre-surgical workup, types of surgeries, and post-surgical outcomes in a broad array of disorders. : Patients ≤18 years who completed epilepsy surgery and had a known genetic etiology prior to surgical intervention were extrapolated from the Pediatric Epilepsy Research Consortium (PERC) surgery database, across 18 US centers. Data were assessed univariably by neuroimaging and EEG results, genetic group (structural gene, other gene, chromosomal), and curative intent. Outcomes were based on a modified International League Against Epilepsy (ILAE) outcome score. : Of 81 children with genetic epilepsy, 72% had daily seizures when referred for surgery evaluation, which occurred a median of 2.2 years (IQR 0.3, 5.2) after developing drug resistance. Following surgery, 68% of subjects had >50% seizure reduction, with 33% achieving seizure freedom [median follow-up 11 months (IQR 6, 17). Seizure freedom was most common in the monogenic structural group, but significant palliation was present across all groups. Presence of a single EEG focus was associated with a greater likelihood of seizure freedom (p=0.02). : There are meaningful seizure reductions following epilepsy surgery in the majority of children with a genetic etiology, even in the absence of a single structural lesion and across a broad spectrum of genetic causes. These findings highlight the need for expedited referral for epilepsy surgery and support of a broadened view of which children may benefit from epilepsy surgery, even when the intent is palliative.