Differential clinical outcomes following encephaloduroarteriosynangiosis in pediatric moyamoya disease presenting with epilepsy or ischemia

Abstract

The purpose of this study was to compare clinical outcomes and surgical results after encephaloduroarteriosynangiosis (EDAS) in pediatric patients with Moyamoya disease that manifested as either ischemia or epileptic seizures. We treated 23 children who underwent EDAS; we divided the patients into either ischemic or epileptic groups according to the individuals' clinical presentation. Group Ia included those who mainly presented with cerebral ischemia in the form of preoperative transient ischemic attacks (TIA), while Group Ib presented with ischemia in the form of irreversible neurologic deficits or proven cerebral infarcts. Group II included those who presented with epileptic seizures rather than cerebral ischemia. We compared the clinical outcomes and surgical results following EDAS in the three groups. We grouped the patients into three groups according to their main preoperative clinical symptoms (Group Ia n = 10, Group Ib n = 6, and Group II n = 7). Group II, the epileptic manifestation group, tended to show more favorable clinical outcomes compared to the ischemic manifestation group, especially the severe ischemic group. However, there were no significant differences in postoperative neuroimaging and hemodynamic assessments between the groups. EDAS is a safe and effective surgical technique that prevents epileptic seizures and shows more favorable clinical outcomes when used in patients with Moyamoya disease presenting with epileptic seizures compared to cerebral ischemia.