Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by narrowing or blockage of the internal carotid arteries in the brain, leading to the development of fragile collateral vessels resembling a “puff of smoke” on imaging. These abnormal vessels are prone to rupture or inadequate blood flow, resulting in strokes, transient ischemic attacks (TIAs), or hemorrhages. Symptoms often include recurrent headaches, seizures, weakness, cognitive decline, or speech difficulties. Moyamoya primarily affects children and young adults but can occur at any age. Early diagnosis and timely intervention are critical to prevent irreversible neurological damage and improve long-term outcomes. Management requires a multidisciplinary approach combining medical therapy, careful monitoring, and surgical revascularization techniques.
Diagnosis involves detailed neurological evaluation and advanced imaging, including MRI, MRA, and CT angiography, to assess artery stenosis, collateral vessel formation, and cerebral perfusion. Cerebral angiography is often used to confirm the diagnosis and map vascular anatomy. Functional imaging may evaluate areas at risk of ischemia. Early and accurate assessment guides treatment planning, determining whether surgical revascularization, medical management, or a combination of both is required. Timely evaluation is essential to prevent strokes and preserve neurological function.
Surgical revascularization is the mainstay treatment for Moyamoya disease, aiming to restore adequate blood flow to the brain and reduce stroke risk. Procedures include direct bypass (e.g., superficial temporal artery to middle cerebral artery) or indirect techniques (e.g., encephaloduroarteriosynangiosis), which encourage new vessel growth over time. Surgery is typically performed by experienced pediatric or adult neurosurgeons depending on the patient’s age. Minimally invasive and neuronavigation-assisted approaches enhance safety and precision. The goal is to prevent recurrent ischemic events while protecting neurological function and promoting long-term cerebral perfusion.
Postoperative recovery includes neurological monitoring, blood pressure management, and imaging to confirm improved cerebral circulation. Rehabilitation may involve physiotherapy, occupational therapy, and cognitive support depending on prior neurological deficits. Patients are advised on stroke prevention strategies and lifestyle modifications. Long-term follow-up ensures vascular stability and detects potential complications. With structured care, most patients experience improved cerebral blood flow, reduced stroke risk, and enhanced quality of life, allowing them to engage safely in daily activities.
Dr. Vamsi Krishna, Consultant Neurosurgeon at Yashoda Hospitals, Hitech City, Hyderabad, specializes in complex cerebrovascular disorders, including Moyamoya disease. His expertise in microsurgical, bypass, and minimally invasive techniques, combined with precise diagnostics and patient-centered care, ensures optimal outcomes, stroke prevention, and long-term neurological protection, making him a trusted expert in Moyamoya management.
