title: Childhood moyamoya disease and moyamoya syndrome: a pictorial review.
abstract: Moyamoya disease is an uncommon chronic cerebrovasculopathy, characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches, in association with the development of compensatory collateral vessels at the base of the brain. The etiology is unknown, and was originally considered exclusive to East Asia, with particular prevalence in Japan. Moyamoya disease is increasingly diagnosed throughout the world, and represents an important cause of childhood stroke in Western countries. In some cases, similar angiographic features are evident in children with other medical conditions, such as sickle cell disease and Down syndrome. In these instances, the term "moyamoya syndrome" is used. Diagnosing the vasculopathy, excluding possible associated conditions, and planning treatment and follow-up imaging comprise important aspects of clinical management. We review the key imaging features of childhood moyamoya disease and syndrome, present examples of its associations, and discuss new neuroradiologic methods that may be useful in management.
authors.affiliation: Leeds and West Yorkshire Radiology Academy, Leeds General Infirmary, Leeds University Teaching Hospitals, National Health Service Trust, Leeds, United Kingdom. stuart.currie@nhs.net
authors.forename: Stuart
authors.initials: S
authors.lastname: Currie
authors.forename: Ashok
authors.initials: A
authors.lastname: Raghavan
authors.forename: Ruth
authors.initials: R
authors.lastname: Batty
authors.forename: Daniel J A
authors.initials: DJ
authors.lastname: Connolly
authors.forename: Paul D
authors.initials: PD
authors.lastname: Griffiths
mesh_terms: D016893:Carotid Stenosis / Q000150:complications / Q000000981:diagnostic imaging / Q000453:epidemiology; D002533:Cerebral Angiography / Q000379:methods; D002648:Child; D002908:Chronic Disease; D003952:Diagnostic Imaging / Q000379:methods; D006801:Humans; D009072:Moyamoya Disease / Q000150:complications / Q000000981:diagnostic imaging* / Q000453:epidemiology*; D020521:Stroke / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000209:etiology; D013577:Syndrome