Treatment of Moyamoya Syndrome in Children with Ischaemic Presentation

Clinical Scenario

This protocol covers children under 18 years of age with Moyamoya syndrome whose presentation is predominantly ischaemic. In the paediatric population, Moyamoya disease expresses itself mainly through ischaemic events — unlike adult disease, which is also characterised by cerebral haemorrhage — making the management approach distinct from that used in adults.

Population & Key Factors

The benefit-risk balance of intervention in children with Moyamoya syndrome differs meaningfully from that in adults, warranting a dedicated protocol. Ischaemic strokes and transient ischaemic attacks (TIAs) are the defining presentations in this age group, and their pattern — single, recurrent, or with varying degrees of recovery — shapes management decisions.

Treatment Approach (Partial Overview)

The approach for this population centres on surgical revascularization — using a combined technique rather than indirect strategies alone when technically feasible. Timing of the intervention relative to any acute cerebrovascular event and management of identified precipitating factors during the waiting period are integral to the protocol.

Patient selection criteria, timing thresholds, and the approach for specific ischaemic presentations are detailed in the full structured protocol below.

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References

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 paediatric MMA patients, we suggest revascularization surgery where there is evidence of ongoing ischaemic symptoms or cerebral haemodynamic impairment.

In paediatric MMA patients with recurrent TIA or recurrent ischaemic strokes, we suggest early revascularization surgery except in case of large territorial ischaemic lesion.

In paediatric MMA patients, we suggest combined revascularization instead of indirect strategies whenever technically possible, to decrease short term risk of stroke.

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