Can Patients with Moyamoya Disease and Large-Scale Cerebral Infarction Still Undergo Surgery?
Understanding Moyamoya Disease: A Rare but Serious Condition
Moyamoya disease is a rare and progressive cerebrovascular disorder characterized by the gradual narrowing or complete blockage of major arteries at the base of the brain—specifically, the terminal portion of the internal carotid arteries and the proximal segments of the anterior and middle cerebral arteries. In some cases, the initial segment of the posterior cerebral artery may also be involved. As these vital blood vessels become obstructed, the brain attempts to compensate by forming a network of tiny, fragile collateral vessels at the base of the skull.
This abnormal vascular network, when visualized through cerebral angiography, appears as a hazy cluster of fine lines resembling puffs of smoke—hence the name "moyamoya," which means "puff of smoke" in Japanese. While this compensatory mechanism aims to maintain cerebral blood flow, it often proves insufficient and introduces new risks due to the fragility of these newly formed vessels.
The Dangers of Moyamoya: Stroke, Hemorrhage, and Seizures
Left untreated, moyamoya disease can lead to devastating neurological consequences. The most common complications include ischemic stroke, hemorrhagic stroke, seizures, and cognitive decline. When the primary arteries narrow faster than the body can develop effective collateral circulation, areas of the brain are deprived of oxygen, leading to cerebral ischemia. This can rapidly progress to large-scale infarction—commonly referred to as a massive stroke.
In addition, the delicate collateral vessels that form during the disease process are prone to rupture. Even minor fluctuations in blood pressure can trigger intracranial hemorrhage, which carries a high risk of permanent disability or death. Furthermore, abnormal electrical activity in the brain caused by chronic ischemia may result in recurrent seizures, significantly impacting a patient's quality of life.
Why Early Intervention Is Critical
Given the progressive nature of moyamoya disease and its potential for sudden, life-threatening events, early diagnosis and timely surgical intervention are crucial. Revascularization surgery is widely recognized as the most effective long-term treatment strategy. By restoring adequate blood flow to the brain, these procedures help prevent future strokes, reduce the risk of hemorrhage, and improve overall neurological function.
Surgical Options After a Major Stroke: Is It Still Possible?
Many patients and families wonder: Can surgery still be performed if a person with moyamoya disease has already suffered a large-area cerebral infarction? The answer is yes—surgery remains not only possible but highly advisable in most cases.
However, timing is essential. Immediately following a major stroke, the brain is in a vulnerable state. During this acute phase (typically the first few weeks), performing elective revascularization surgery could increase the risk of complications such as cerebral edema or reperfusion injury. Therefore, medical teams usually recommend waiting until the patient has stabilized neurologically and completed initial stroke recovery.
Optimal Timing for Surgical Revascularization
Most neurosurgeons advise delaying definitive bypass surgery for approximately 8 to 12 weeks after a major ischemic event. This window allows time for:
- Stabilization of brain tissue
- Reduction of post-stroke inflammation
- Rehabilitation and assessment of residual deficits
- Optimization of the patient's overall health
Once the patient is stable, revascularization procedures such as direct superficial temporal artery-to-middle cerebral artery (STA-MCA) bypass or indirect encephaloduroarteriosynangiosis (EDAS) can be safely performed. These surgeries aim to establish new pathways for blood flow, thereby reducing the likelihood of future strokes and supporting the recovery of compromised brain regions.
Long-Term Outlook and Prognosis
With appropriate surgical intervention and comprehensive follow-up care, many patients with moyamoya disease—even those who have experienced significant strokes—can achieve meaningful improvements in neurological function and quality of life. Early recognition of symptoms, prompt imaging, and referral to a specialized center experienced in pediatric and adult moyamoya management are key factors in achieving favorable outcomes.
In conclusion, while a large cerebral infarction poses serious challenges, it does not rule out the possibility of life-saving surgery. For individuals diagnosed with moyamoya disease, timely revascularization after stroke stabilization offers the best chance for long-term protection against further neurological damage.