• Giuseppe Mirone, Domenico Cicala, Chiara Meucci, Alessandra d'Amico, Claudia Santoro, Mario Muto, and Giuseppe Cinalli.
• Department of Pediatric Neurosurgery, Santobono-Pausilipon Children's Hospital, Naples, Italy. Electronic address: peppemirone@fastwebnet.it.
• World Neurosurg. 2019 Jul 1; 127: e843-e855.

ObjectiveMoyamoya disease (MMD) is characterized by a progressive spontaneous occlusion of distal internal carotid arteries and its main branches. It is necessary to promptly diagnose and treat this condition, especially in children, because of the high risk of stroke and consequent severe disability. In this study, we examine the role of multiple burr-hole surgery (MBHS) in the treatment of pediatric patients with MMD and quasi-MMD and the role of perfusion magnetic resonance imaging (MRI) in the surgical indication and during follow-up.MethodsWe reviewed preoperative, early postoperative, and late postoperative MRI perfusion and digital subtraction angiography images together with clinical and surgical outcomes in 10 children with MMD treated by MBHS.ResultsFourteen MBHS procedures (6 unilateral, 2 bilateral, and 2 single-setting bilateral) were performed in 10 children. Clinical and radiologic follow-up for all patients ranges from 16 months to 7 years. No ischemic events (transient ischemic attacks or stroke) occurred during the follow-up period. Postoperative digital subtraction angiography showed good revascularization around the burr-hole sites in all patients. The functional efficacy of the surgery was confirmed by dynamic susceptibility contrast perfusion MRI studies in 8 patients.ConclusionsOur data underline the good rate of success of MBHS in pediatric MMD and quasi-MMD. This technique results in good collateral revascularization, improved cerebral perfusion and excellent short and long-term symptoms control, with low perioperative risk. Postoperative changes of perfusion parameters and ivy sign MRI finding seem to show a positive correlation and mainly occur in posterior middle cerebral artery territory.Copyright © 2019. Published by Elsevier Inc.

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