Moyamoya Disease: Symptoms, Treatment Options, and Post-Surgical Care Guidelines

Understanding Moyamoya Disease and Its Rising Prevalence

Moyamoya disease is a rare but increasingly recognized cerebrovascular disorder characterized by the progressive narrowing and eventual occlusion of the internal carotid arteries and their major branches, including the anterior and middle cerebral arteries. This condition leads to the development of a fragile network of tiny collateral blood vessels at the base of the brain, which appear "smoky" or hazy on imaging—hence the name "moyamoya," derived from the Japanese term meaning "puff of smoke." These abnormal vessels are prone to rupture or fail to deliver sufficient blood flow, resulting in either ischemic strokes or intracranial hemorrhages.

Common Clinical Manifestations

Patients with moyamoya disease often present with neurological symptoms stemming from compromised cerebral circulation. In children, transient ischemic attacks (TIAs) or ischemic strokes are more common, often triggered by hyperventilation or physical exertion. Adults, on the other hand, may experience either ischemic events or hemorrhagic strokes due to the rupture of fragile collateral vessels. Additional symptoms can include seizures, cognitive decline, headaches, and motor deficits such as weakness or paralysis on one side of the body. Early diagnosis through MRI, MRA, or cerebral angiography is critical for timely intervention.

Surgical Interventions and Revascularization Techniques

First described by Japanese neurologists, moyamoya disease has no definitive cure, but surgical revascularization remains the cornerstone of long-term management. The primary goal is to restore adequate blood supply to the brain and prevent future stroke events. One widely used approach is direct bypass surgery, such as the superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis, which provides immediate improvement in blood flow.

Indirect Revascularization Methods

In addition to direct bypass, indirect revascularization techniques are frequently employed, especially in pediatric cases. These include encephaloduroarteriosynangiosis (EDAS), where the superficial temporal artery is placed in contact with the brain surface to encourage new vessel growth, and encephalomyosynangiosis (EMS), which involves placing the temporal muscle directly onto the brain to stimulate vascularization. These procedures promote the formation of new collateral pathways over time and are considered safe and effective, with many major neurosurgical centers across the United States and Europe offering these advanced treatments.

Postoperative Care and Recovery Strategies

Successful outcomes following moyamoya surgery depend not only on the procedure itself but also on meticulous postoperative management. Patients should adhere to specific care protocols to minimize complications and support recovery.

Key Post-Surgery Recommendations

Blood pressure regulation is essential, as patients with moyamoya have delicate cerebral hemodynamics. Hypertension can lead to hyperperfusion syndrome—a potentially dangerous surge in brain blood flow—while hypotension may result in cerebral ischemia. Maintaining blood pressure within a narrow, physician-recommended range is crucial during the recovery phase.

Controlled volume expansion may be advised to enhance cerebral perfusion, particularly in the early postoperative period. Intravenous fluids are often administered under close monitoring to optimize blood flow without increasing the risk of edema or hemorrhage.

Delaying anticoagulant and antiplatelet therapy is another critical precaution. While these medications are commonly used after vascular procedures, they are typically avoided in the initial weeks after moyamoya surgery due to the elevated risk of intracranial bleeding from fragile neovessels.

Early mobilization is strongly encouraged. Getting out of bed and engaging in light physical activity as soon as medically safe promotes circulation, reduces the risk of deep vein thrombosis, and supports overall neurological recovery. Physical and occupational therapy may be integrated into the rehabilitation plan to improve functional outcomes.

Long-Term Outlook and Monitoring

With appropriate surgical intervention and comprehensive follow-up care, many patients experience significant symptom relief and a reduced risk of future stroke. Regular imaging studies and clinical evaluations are recommended to monitor vascular changes and ensure sustained cerebral perfusion. Raising awareness among healthcare providers and improving access to specialized neurovascular centers remain key to enhancing patient outcomes worldwide.