In paediatric patients under 18, Moyamoya syndrome presents predominantly with ischaemic events rather than the haemorrhagic strokes more commonly seen in adults. This age-specific pattern is clinically important: the benefit-risk balance of available therapies is substantially different in children, and management guidance is developed separately for this population.
This protocol applies to children (under 18 years of age) with Moyamoya syndrome whose disease course is mainly ischaemic. Because the paediatric and adult presentations differ fundamentally — ischaemic predominance in children versus a mixed ischaemic-haemorrhagic picture in adults — treatment selection must account for the child's specific risk profile.
For children with non-haemorrhagic Moyamoya syndrome, the evidence supports long-term antiplatelet therapy as the first-line strategy to reduce stroke risk. Which specific agent is chosen, and how it is used, is detailed in the full structured protocol.
DOI: 10.1177/23969873221144089
Indeed, the expression of the MMA is mainly ischaemic in children, whereas it is also characterised by cerebral haemorrhage in adults.
For PICO5 and PICO6 the adult population was differentiated from the paediatric population as the key factors affecting the benefit-risk balance differ in these two situations.
In patients with non-haemorrhagic MMA, we suggest the use of long-term antiplatelet therapy to reduce the risk of embolic strokes without an increase in haemorrhagic strokes.
Evaluating the type of antiplatelet drugs, the most commonly used agents in the studies included in this analysis were aspirin, clopidogrel or cilostazol as monotherapy, with only a minority of patients receiving dual antiplatelet therapy.
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