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Objective: Malignant middle cerebral artery infarction (mMCAI) is a life-threatening condition in pediatric patients. Despite strong evidence showing decreased morbidity and mortality in adult mMCAI patients with decompressive hemicraniectomy (DCH), there is a paucity of data on the use of DCH in children with similar conditions. Here we report experience from our center and perform a systematic review of published literature on outcomes after use of DCH in pediatric mMCAI patients. Methods: By retrospective chart review, we identified 3 children with large ischemic stroke who underwent DCH for life-threatening cerebral edema. Information was obtained about patient characteristics on admission, radiological features of the stroke, surgical procedures, complications of the DCH and cranioplasty, and functional outcomes during follow-up visits. We also reviewed the current literature on DCH in pediatric stroke. Results: DCH was performed in all 3 cases after development of pupillary dilatation. All 3 children survived and were ambulatory at the time of follow-up. Review of literature identified 12 other published case series describing 26 cases of DCH in pediatric patients with ischemic stroke. Descriptive statistical analysis of these cases is presented. Published reports suggest that a good outcome is possible even in the presence of signs of herniation, low preoperative Glasgow Coma Scale score, involvement of multiple vascular territories, or longer time to surgery in pediatric ischemic stroke patients. Conclusions: The current data suggest a role for DCH in the management of cerebral edema in pediatric patients with mMCAI. Factors that help in prognostication for adult stroke patients undergoing DCH do not appear to convey similar information about the pediatric population. This highlights the urgent need for collaboration across institutes to further investigate this potentially life-saving procedure in pediatric stroke. [ABSTRACT FROM AUTHOR]