Moyamoya Disease and Anesthesia Considerations
Moyamoya Disease and Anesthesia Considerations Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by the narrowing or occlusion of the terminal portions of the internal carotid arteries and their main branches. This narrowing leads to the development of a complex network of fragile collateral vessels that attempt to compensate for reduced blood flow to the brain. The name “moyamoya,” Japanese for “puff of smoke,” describes the hazy appearance of these collateral vessels on imaging studies. Patients typically present with symptoms such as transient ischemic attacks, strokes, or headaches, often at a young age, though it can occur at any age.
Managing anesthesia in patients with moyamoya disease requires careful planning and meticulous intraoperative management due to their unique cerebrovascular physiology. The primary concern is maintaining adequate cerebral perfusion while avoiding fluctuations that could precipitate ischemic or hemorrhagic events. Since the disease involves compromised blood flow, any factors that increase intracranial pressure (ICP), decrease cerebral blood flow, or cause blood pressure swings must be minimized. Moyamoya Disease and Anesthesia Considerations
Preoperative assessment should include detailed neurological evaluation and imaging studies like MRI or cerebral angiography to determine the extent of arterial stenosis and collateral circulation. Additionally, baseline blood pressure, oxygenation status, and neurological function should be documented. It is also critical to review any history of previous strokes, seizures, or neurological deficits which could influence anesthetic management.
During anesthesia, maintaining hemodynamic stability is paramount. This involves avoiding hypotension, which can exacerbate cerebral ischemia, and hypertensive episodes that may increase the risk of hemorrhage. Blood pressure should be kept within a range that ensures sufficient cerebral perfusion without stressing fragile collateral vessels. The use of invasive blood pressure monitoring may be indicated in complex cases or lengthy procedures.
Moyamoya Disease and Anesthesia Considerations Choice of anesthetic agents should aim for minimal impact on cerebral vasculature. Volatile anesthetics like isoflurane or sevoflurane are commonly used, but their vasodilatory effects must be carefully titrated. Intravenous agents such as propofol can provide stable anesthesia with neuroprotective properties. Maintaining adequate oxyge

nation and normocapnia is vital because both hypoxia and hypercapnia can cause cerebral vasodilation, potentially increasing ICP or disrupting fragile collateral circulation.
Monitoring cerebral status intraoperatively can be enhanced with techniques like near-infrared spectroscopy (NIRS) or transcranial Doppler ultrasound, which provide real-time feedback on cerebral blood flow. Avoiding sudden changes in body temperature, glucose levels, and acid-base status also contributes to optimal cerebral protection. Moyamoya Disease and Anesthesia Considerations
Moyamoya Disease and Anesthesia Considerations Postoperative care involves close neurological monitoring for any signs of ischemia or hemorrhage. Maintaining stable blood pressure, ensuring adequate hydration, and preventing hypoxia are crucial in the immediate postoperative period. In some cases, therapies such as antiplatelet agents or anticoagulation may be continued or initiated to reduce the risk of thrombotic events, but these decisions are made in coordination with neurology or neurosurgery teams.
In conclusion, anesthesia management in patients with moyamoya disease demands a nuanced understanding of cerebrovascular physiology and vigilant intraoperative monitoring. Tailoring anesthetic techniques to maintain cerebral perfusion while minimizing risks can significantly influence surgical outcomes and patient safety. Moyamoya Disease and Anesthesia Considerations









