From the Guidelines
The treatment for an acute cerebrovascular accident (CVA) in a patient with Moyamoya disease should focus on immediate medical attention to stabilize the patient and prevent further damage, with surgical revascularization procedures considered after the acute phase to prevent recurrent strokes. Initial management includes maintaining adequate cerebral perfusion through careful blood pressure control, typically keeping systolic blood pressure between 140-160 mmHg to ensure sufficient collateral flow without risking hemorrhage, as suggested by the American Heart Association/American Stroke Association guidelines 1. Intravenous fluids should be administered to maintain euvolemia. Antiplatelet therapy with aspirin (81-325 mg daily) is often initiated, though thrombolytic agents like tPA are generally avoided due to the high risk of hemorrhage in Moyamoya patients 1. Anticoagulation with heparin or warfarin is typically contraindicated unless there's a specific indication such as atrial fibrillation. Some key points to consider in the management of Moyamoya disease include:
- Surgical revascularization procedures, including direct bypass (STA-MCA bypass) or indirect procedures (EDAS, EMS), should be considered after the acute phase to prevent recurrent strokes by improving cerebral blood flow 1.
- Supportive care including management of fever, hyperglycemia, and seizures is essential, as these factors can worsen neurological outcomes.
- Early rehabilitation should be initiated to maximize functional recovery.
- The use of direct bypass in the Japan Adult Moyamoya trial showed benefit over medical management, and is considered the most appropriate intervention for hemorrhagic patients 1. It's worth noting that there remains ongoing debate as to the optimal revascularization strategy, and most meta-analyses and their source studies have several limitations, including nonrandomized allocation of treatment, heterogeneity in age and clinical presentation of cohorts, unblinded outcome assessment, variable length of follow-up, and redundancy in the included cohorts 1.
From the Research
Treatment for Acute CVA in Moyamoya Disease
The treatment for an acute cerebrovascular accident (CVA) in a patient with Moyamoya disease involves several options, including:
- Revascularization surgery, which can be direct, indirect, or combined bypass 2, 3
- Intravenous thrombolysis, mechanical embolectomy, and intracranial stenting for hyperacute ischemic stroke 4
- Extracranial-intracranial bypass to improve cerebrovascular reactivity and clinical outcome 5
Revascularization Surgery
Revascularization surgery is considered an effective treatment for preventing recurrent ischemic stroke in patients with Moyamoya disease 2, 3. The surgery can be direct, indirect, or combined bypass, and the choice of surgery depends on the individual patient's condition. Direct and combined bypasses are more effective than indirect bypass surgery in preventing recurrent ischemic stroke 2.
Intravenous Thrombolysis and Mechanical Embolectomy
Intravenous thrombolysis and mechanical embolectomy can be used to treat hyperacute ischemic stroke in patients with Moyamoya disease 4. However, the use of these options is novel and requires further study to determine their safety and efficacy.
Extracranial-Intracranial Bypass
Extracranial-intracranial bypass can improve cerebrovascular reactivity and clinical outcome in patients with symptomatic Moyamoya vasculopathy 5. The surgery can reverse CVR impairment and is predictive of a patent extracranial-intracranial bypass.
Risks and Complications
Revascularization surgery and other treatments for Moyamoya disease carry risks and complications, including:
- Ischemic complications in the contralateral hemisphere after surgical treatment 6
- Rebleeding after surgery 3
- Perioperative hemorrhages, cortical foci of ischemia, or new white matter T2 hyperintensities 5
Key Findings
Key findings from the studies include:
- Revascularization surgery is effective in preventing recurrent ischemic stroke in patients with Moyamoya disease 2, 3
- Direct and combined bypasses are more effective than indirect bypass surgery in preventing recurrent ischemic stroke 2
- Extracranial-intracranial bypass can improve cerebrovascular reactivity and clinical outcome in patients with symptomatic Moyamoya vasculopathy 5
- Intravenous thrombolysis and mechanical embolectomy can be used to treat hyperacute ischemic stroke in patients with Moyamoya disease, but require further study 4