Abstract
INTRODUCTION: Moyamoya disease is a steno-occlusive cerebrovascular disease of unknown etiology involving the terminal portion of the internal carotid artery and the proximal portions of the anterior and middle cerebral arteries with associated collateral vascular network. When the vascular pattern is associated with a particular condition (e.g., Type 1 neurofibromatosis, Down syndrome), it is defined as moyamoya syndrome (MMS) (or quasi-moyamoya). Among different indirect bypass techniques used to prevent ischemic injury by increasing collateral blood flow to hypoperfused areas of the cortex, multiple burr holes technique is an easy and diffuse indirect revascularization approach in the treatment of moyamoya. DISCUSSION: While the effectiveness in patients with moyamoya disease was demonstrated, its role in MMS remains uncertain. In this study, we describe surgical and diagnostic implications in three pediatric cases of moyamoya sydrome unsuccessfully treated with multiple cranial burr hole technique. A critical review of the literature about the use of the surgical indirect revascularization techniques in pediatric patients was also reported.