Childhood Moyamoya: Looking Back to the Future

Abstract Moyamoya is a chronic, progressive steno-occlusive arteriopathy that typically affects the anterior circulation arteries of the circle of Willis. A network of deep thalamoperforating and lenticulostriate collaterals develop to by-pass the occlusion giving rise to the characteristic angiographic "puff of smoke" appearance. Moyamoya confers a lifelong risk of stroke and neurological demise, with peak age of presentation in childhood ranging between five and 10 years. Moyamoya disease refers to patients who do not have a comorbid condition, whereas moyamoya syndrome refers to patients in whom moyamoya occurs in association with an acquired or inherited disorder such as sickle cell disease, neurofibromatosis type-1 or trisomy 21. The incidence of moyamoya disease and moyamoya syndrome demonstrates geographic and ethnic variation, with a predominance of moyamoya disease in East-Asian populations. Antiplatelet therapy and surgical revascularization procedures are the mainstay of management, as there are no available treatments to slow the progression of the arteriopathy. Future research is required to address the major gaps that remain in our understanding of the pathologic basis, optimal timing for surgery, and determinants of outcome in this high-stroke risk condition of childhood.