Treatment of Moyamoya Syndrome in Adults with Intracerebral Haemorrhage
Clinical Scenario
This protocol applies to adult patients (aged 18 years or older) with Moyamoya syndrome who have experienced a haemorrhagic event — intracerebral, intraventricular, or subarachnoid haemorrhage — rather than an ischaemic-only course. Cerebral haemorrhage in adults represents a distinct and clinically significant expression of Moyamoya disease, contrasting with the predominantly ischaemic presentation seen in children. Eligible patients are functionally independent and have a confirmed history of such haemorrhage within the preceding 12 months.
Approach — partial overview
Management in this setting involves consideration of revascularization surgery under specific haemodynamic and anatomical conditions. A defined interval is observed between the acute haemorrhagic event and any surgical intervention, during which particular clinical risk factors are actively avoided. The full protocol specifies the exact indications, timing criteria, and perioperative precautions.
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.
- Adult patients functionally independent, aged between 16 and 65 years and with a history of intracerebral, intraventricular or subarachnoid haemorrhage that occurred within 12 months before inclusion were eligible.
- In adult MMA patients with haemorrhagic presentation, we recommend revascularization surgery (evidence only for direct STA-MCA bypass) in case of cerebral haemodynamic impairment and presence of choroidal collaterals.
- 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.
View source ↗