Introduction: This study mainly discussed management strategies for the ischemic-type moyamoya disease(MMD), which were based on the clinical features and long-term outcomes after surgical revascularization.
Methods: A total of 570 patients with bilateral and unilateral MMD were identified from January 2005 to December 2012 in Beijing Tiantan Hospital. Data on clinical features, radiologic images, treatments and outcomes were reviewed.
Results: A total of 346 patients with ischemic-type MMD underwent 437 revascularization procedures and the mean follow-up period was 4.0 years. The annual postoperative stroke rate was 3.3% and the 5-year cumulative stroke rate was 14.3%. The incidence of perioperative stroke was 6.9% per procedures. Adults with severe internal carotid artery(ICA) and posterior cerebral artery (PCA) angiopathy before surgery were found to be at a higher risk of perioperative stroke; In addition, the subsequent stroke for adults occurred throughout the first 5 years after surgery. Children had better surgical outcomes with the 5-year cumulative stroke was 9.0%, and most of subsequent stroke occurred in the first 2 years after surgery. The overall mortality rate was 2.0% and stroke associated mortality was 0.9%. Moreover, 86.7% of patients experienced a significant functional improvement and 87.9% of patients had favorable outcome (mRS<3) at the last follow-up. PCA involvement (OR, 3.364; P=0.002) and age at onset (OR, 1.057; P<0.001) were highly predictive of perioperative stroke. PCA involvement (OR, 2.62; P=0.005), ICA supraclinoid segment occlusion (OR, 2.763; P=0.011) and elder age at onset (OR, 1.027; P=0.033) were predictors of unfavorable outcome at the long-term follow-up.
Conclusions: Surgical revascularization is effective at alleviating symptoms, preventing recurrent stroke and improving functional outcome in symptomatic patients with ischemic-type MMD. Active treatment, careful perioperative management and follow-up are essential to achieve favorable outcome, before patients come to ischemic presentation at elder age with severer angiopathy.
Patient Care: Although moyamoya disease has been found over 50 years, there is a scarcity of level I evidence to confirm the best management strategies for patients with ischemic-type moyamoya disease, which account for 60-80% of the whole moyamoya disease population. Our research found that surgical revascularization is effective treatment in symptomatic patients with ischemic-type moyamoya disease. More importantly, we discussed potential subgroups with distinct outcomes and specific treatment recommendations for them, based on our evidence. Our study also revealed potential predictors for surgical outcomes, which might be helpful to identify patients with a high risk of unfavorable outcome and allow individualized management strategies.
Learning Objectives: By the conclusion of this session, participants should be able to identify an effective treatment in symptomatic patients with ischemic-type moyamoya disease and may use individualized management strategies for these patients to receive favorable outcomes.