Moyamoya Disease treatment options in adults
Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by the narrowing of arteries at the base of the brain, particularly the internal carotid arteries and their branches. This constriction leads to the development of a network of tiny blood vessels that attempt to compensate for reduced blood flow, forming a “puff of smoke” appearance on imaging studies—hence the name “moyamoya,” which means “hazy” or “puff of smoke” in Japanese. While often diagnosed in children, moyamoya can also affect adults, presenting a unique set of challenges and treatment considerations.
In adults, the clinical presentation of moyamoya disease can differ from that in children. Adults may experience transient ischemic attacks (TIAs), strokes, headaches, seizures, or cognitive disturbances. The risk of hemorrhage is also higher in adult patients due to fragile abnormal vessels that can rupture. Managing moyamoya in adults requires a comprehensive approach that aims to restore adequate cerebral blood flow, prevent strokes, and address symptoms.
The cornerstone of treatment for moyamoya disease in adults is surgical intervention, which primarily involves revascularization procedures. These procedures aim to bypass the narrowed or occluded arteries, providing an alternative route for blood flow to the brain tissue at risk. There are two main types of surgical options: direct bypass and indirect bypass techniques.
Direct bypass procedures, such as superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis, involve directly connecting a scalp artery to a brain artery. This provides immediate improvement in blood flow, making it particularly effective for adult patients with significant ischemic symptoms. However, the success of direct bypass depends on the surgeon’s expertise and the patient’s vascular anatomy.
Indirect bypass techniques, like encephaloduroarteriosynangiosis (EDAS) or encephalomyosynangiosis (EMS), involve placing vascular-rich tissues—such as scalp arteries or muscles—around the brain surface. Over time, new blood vessels grow from these tissues into the brain, gradually improving cerebral perfusion. These procedures are often combined with direct methods to maximize revascularization, especially in adults who may have less pliable vessels or complex disease anatomy.
Medical management plays a supportive role, especially in cases where surgery is contraindicated or as an adjunct to surgical treatment. This includes controlling risk factors like hypertension, smoking cessation, and antiplatelet therapy to reduce the risk of stroke. Antiplatelet agents, such as aspirin, are commonly prescribed to prevent blood clots that could exacerbate ischemic events.
While surgical revascularization remains the definitive treatment, ongoing follow-up with neuroimaging is essential to monitor the success of the procedure and detect any new or residual vascular abnormalities. Rehabilitation therapies may also be necessary for patients who have suffered strokes or neurological deficits.
In summary, treatment options for adults with moyamoya disease focus on restoring and improving cerebral blood flow through surgical revascularization, complemented by medical management to minimize stroke risk. Early diagnosis and intervention are crucial to prevent irreversible neurological damage and improve quality of life.
