• Jun T Park, Guadalupe Fernandez-Baca Vaca, Rachel B Tangen, Mark L Cohen, and Jonathan P Miller.
• Section of Pediatric Epilepsy, Department of Pediatrics, Rainbow Babies & Children's Hospital, Cleveland, Ohio, USA; Epilepsy Center, Neurological Institute, Department of Neurology, University Hospitals of Cleveland Medical Center, Cleveland, Ohio, USA; Case Western Reserve University School of Medicine, Cleveland, Ohio, USA. Electronic address: jun.park@uhhospitals.org.
• World Neurosurg. 2018 Aug 1; 116: 268-273.

BackgroundCatastrophic epilepsy results in severe neurodevelopmental delay in infants because of frequent and/or long seizures. Therefore, consideration of early epilepsy surgery is essential for neurodevelopmental outcome. Once an infant with catastrophic seizures is identified as a surgical candidate, it is important that the surgical plan be carefully defined based on detailed presurgical evidence to minimize surgical complications in this age group.Case DescriptionWe present 2 infants with catastrophic epilepsy, epileptic spasms, and bihemispheric electroencephalographic abnormalities who underwent one-stage disconnection surgery based on a sound hypothesis of the epileptogenic zone. Each patient underwent an extensive noninvasive presurgical investigation followed by stereotactic disconnection leucotomy in a single stage. After the 2 children were followed for 24-36 months. A seizure reduction by at least 90% (Engel class I) was achieved in both cases with subsequent improvement in neurodevelopmental progress. There were no perioperative complications. Both patients had widespread cortical dysplasia on pathologic evaluation.ConclusionsCareful consideration of the noninvasive presurgical workup can identify focal onset even in the presence of catastrophic epilepsy with widespread bilateral abnormalities. Single-stage lobar leucotomy for disconnection of the epileptogenic zone can lead to excellent outcome in these patients.Copyright © 2018 Elsevier Inc. All rights reserved.

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