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Hemorrhagic Moyamoya Disease : A Recent Update
Miki Fujimura1, Teiji Tominaga2
1Department of Neurosurgery, Kohnan Hospital, Sendai, Japan.
Abstract:
Moyamoya disease (MMD) is a progressive cerebrovascular disease with unknown etiology, characterized by bilateral stenoocclusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network formation at the base of the brain. MMD has an intrinsic nature to convert the vascular supply for the brain from internal carotid (IC) system to the external carotid (EC) system, as indicated by Suzuki's angiographic staging. Insufficiency of this 'IC-EC conversion system' could result not only in cerebral ischemia, but also in intracranial hemorrhage from inadequate collateral anastomosis, both of which represent the clinical manifestation of MMD. Surgical revascularization prevents cerebral ischemic attack by improving cerebral blood flow, and recent evidence further suggests that extracranial-intracranial bypass could powerfully reduce the risk of re-bleeding in MMD patients with posterior hemorrhage, who were known to have extremely high re-bleeding risk. Although the exact mechanism underlying the hemorrhagic presentation in MMD is undetermined, most recent angiographic analysis revealed the characteristic angio-architecture related to high re-bleeding risk, such as the extension and dilatation of choroidal collaterals and posterior cerebral artery involvement. We sought to update the current management strategy for hemorrhagic MMD, including the outcome of surgical revascularization for hemorrhagic MMD in our institute. Further investigations will clarify the optimal surgical strategy to prevent hemorrhagic manifestation in patients with MMD.
Insights
Moyamoya disease (MMD) management is updated, showing surgical revascularization effectively reduces re-bleeding risk in hemorrhagic MMD patients. Further research aims to optimize surgical strategies for preventing hemorrhagic MMD.
Area of Science:
- Neurology
- Neurosurgery
- Vascular Neurology
Background:
- Moyamoya disease (MMD) is a progressive cerebrovascular disorder characterized by internal carotid artery stenosis and abnormal basal brain vasculature.
- MMD involves a shift in cerebral blood supply from the internal carotid (IC) to the external carotid (EC) system, which can lead to ischemic or hemorrhagic events.
- Hemorrhagic MMD presents a high risk of re-bleeding, often associated with specific angiographic features like dilated choroidal collaterals and posterior cerebral artery involvement.
Purpose of the Study:
- To update the current management strategies for hemorrhagic Moyamoya disease.
- To evaluate the outcomes of surgical revascularization in patients with hemorrhagic MMD.
- To identify optimal surgical approaches for preventing hemorrhagic manifestations in MMD.
Main Methods:
- Review of current literature and clinical data on Moyamoya disease management.
- Analysis of angiographic findings associated with hemorrhagic MMD and re-bleeding risk.
- Assessment of surgical revascularization outcomes, specifically extracranial-intracranial bypass, in hemorrhagic MMD patients.
Main Results:
- Surgical revascularization, particularly EC-IC bypass, is effective in improving cerebral blood flow and reducing ischemic events in MMD.
- Evidence suggests extracranial-intracranial bypass significantly lowers the re-bleeding risk in MMD patients with posterior hemorrhage.
- Specific angio-architectures, including extensive choroidal collaterals and posterior cerebral artery involvement, are linked to increased re-bleeding risk in hemorrhagic MMD.
Conclusions:
- Surgical revascularization is a crucial component in managing both ischemic and hemorrhagic Moyamoya disease.
- Extracranial-intracranial bypass shows promise in mitigating the high re-bleeding risk associated with hemorrhagic MMD.
- Further investigation is needed to refine surgical strategies for optimal prevention of hemorrhagic events in MMD patients.
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