Moyamoya syndrome
ICD-10 I67.5 · ICD-11 8B22.B

Treatment of Moyamoya Syndrome in Adults (Age ≥18) Without Cerebrovascular Symptoms

This protocol covers the management of Moyamoya syndrome in adult patients aged 18 years or older who are asymptomatic — presenting without active cerebrovascular symptoms and without a haemorrhagic event.

Clinical scenario: Adult (age ≥18) with confirmed Moyamoya syndrome — asymptomatic, no cerebrovascular symptoms, no haemorrhagic presentation.

In asymptomatic adults with Moyamoya syndrome, the threshold for surgical intervention versus conservative management is guided by specific cerebral haemodynamic and imaging criteria. When surgery is indicated, the selection of revascularisation strategy directly influences outcomes.

Treatment approach (partial summary)

When surgical intervention is indicated, the preferred approach involves a direct or combined revascularisation technique rather than indirect revascularisation alone. The timing of surgery relative to any prior cerebrovascular event, and management of physiological conditions in the waiting period, are central elements of the protocol.

The full decision criteria, surgical algorithm, and perioperative management details are in the complete protocol.

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References

DOI: 10.1177/23969873221144089

In adult MMA asymptomatic patients, we suggest considering conservative treatment except in patients with both cerebral haemodynamic impairment and silent ischaemic lesions in the same cerebral region.

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.

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