Adults aged 18 or older who develop Moyamoya syndrome (MMA) and present with ischaemic symptoms — transient ischaemic attack (TIA) or ischaemic stroke — without haemorrhagic features represent a distinct clinical population. The ischaemic expression of MMA predominates in adults and calls for a specific, structured management pathway.
This protocol applies to adult patients (age ≥18) with a symptomatic ischaemic presentation: either a transient ischaemic attack or an ischaemic stroke, in the absence of haemorrhagic stroke. In adults, the ischaemic form of MMA is the primary pattern, distinguishing management requirements from those of paediatric or haemorrhagic presentations.
Management centres on revascularization surgery, with preference for a direct or combined surgical technique over an indirect approach alone. A defined waiting period following the acute cerebrovascular event is observed before surgery is performed. During that interval, specific haemodynamic trigger factors must be actively avoided. Perioperative antiplatelet therapy is managed according to a structured protocol that varies depending on whether the patient is on single or dual antiplatelet therapy at the time of surgery. The complete regimen — including surgical indications, timing, and the full perioperative antiplatelet algorithm — is available in the protocol.
Patients were considered symptomatic when presenting with TIA, ischaemic or haemorrhagic stroke, headache, movement disorders or cognitive disturbances.
Indeed, the expression of the MMA is mainly ischaemic in children, whereas it is also characterised by cerebral haemorrhage in adults.
In adult MMA patients with ischaemic presentation, we suggest that revascularization surgery should be considered in case of clinical symptoms and/or imaging markers of haemodynamic impairment.
In adult MMA patients, we suggest direct/combined revascularization instead of indirect strategies for reducing risk of stroke.
In patients with MMA, we suggest waiting 6–12 weeks from an acute cerebrovascular event before performing surgery for MMA patients, to reduce the rate of postoperative complications.
In patients with MMA, we suggest avoiding trigger factors such as dehydration, fever, and hyperventilation as well as hypotension when waiting for surgery.
For patients with MMA, we suggest that, during bypass surgery continuation of antiplatelet treatment as monotherapy (aspirin) is safe.
In case of dual antiplatelet therapy (aspirin + clopidogrel or other antiplatelets), we suggest stopping clopidogrel, or the other second antiplatelet therapy, for 7 days before surgery.
However, in case of discontinuation, we suggest restarting antiplatelet therapy 1–7 days after surgery, depending on the post-surgery CT scan.
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