Moyamoya: to cut or not to cut is not the only question. A paediatric neurologist's perspective

Abstract

The paediatric neurologist is usually the clinician who makes the diagnosis of moyamoya in children, yet most of the debate in the literature has focused on surgical management of the condition. Semantic confusion and variable use of the term among neuroradiologists continues to be unhelpful. Increasing recognition of moyamoya, for example in sickle cell disease, and the publication of clinical guidelines encouraging referral for surgical evaluation highlight the need to identify and engage with management of the condition. In practical terms, the most frequent management issues for the paediatric neurologist, other than when to refer for surgery, are headache, hypertension, and the concern of the family that other children might be affected. These issues are discussed in the context of the available literature, and areas in which there is a need for research and consensus are identified.