Abstract
Objective: This study aims to investigate the clinical features, long-term outcomes, and prognostic predictors of a multicenter cohort of children with moyamoya disease.Methods: A series of 303 consecutive pediatric moyamoya disease (MMD) patients were screened in the present study. The clinical characteristics were retrospectively collected, and long-term outcomes was evaluated. Furthermore, logistic regression analyses were performed to determine the prognostic predictors for the clinical outcome.Results: The mean onset age at diagnosis was 9.4 years old. The gender ratio (girl-to-boy ratio) was 1.1:1.0. Among these 303 patients, 13 patients underwent different surgical modalities in bilateral hemispheres, while eight patients failed to follow-up, and were excluded. Therefore, a total of 282 patients were analyzed. Among these patients, 17 patients underwent combined bypass (CB), 47 patients underwent direct bypass (DB), 150 patients underwent indirect bypass (IB), and 68 patients underwent conservative treatment. Furthermore, recurrent stroke events were observed in 35 patients (12.4%). The Kaplan-Meier analysis demonstrated that there was no significant difference in either ischemia or hemorrhage-free time among the different surgical modalities (P = 0.67 and 0.79, respectively). Furthermore, longer ischemia-free time was observed in the surgical group, when compared to the conservative group (P < 0.01). In addition, 82.7% (177/214) of patients who underwent surgical treatment obtained good outcomes (mRS 0-1), which were significantly higher than the rate of patients who underwent conservative treatment (52.9%, 36/68; P < 0.01). The rate of patients with improved symptoms was also significantly different (93.0 vs. 16.2%, P < 0.01). However, no significant difference was observed in the rate of good outcomes, disability, and improved symptoms among the different surgical modalities. The logistic regression analyses revealed that postoperative ischemic events were the only risk factor associated with unfavorable clinical outcome (OR:3.463; 95% CI:1.436–8.351; P < 0.01).Conclusion: CB, DB, and IB might have similar effects on long-term clinical outcome in pediatric MMD. However, surgical revascularization is superior, when compared to conservative treatment. Furthermore, postoperative ischemic events were confirmed as potential prognostic factors associated with unfavorable clinical outcome.
Highlights
Moyamoya disease (MMD) is a progressive narrowing or occlusion cerebrovascular disease characterized by bilateral stenosis of the internal carotid arteries (ICA) and the development of a compensatory network of basal collaterals [1,2,3]
The present study demonstrated that transient ischemic attack (TIA) was the most common clinical manifestation, and that bilateral MMD occurred in most pediatric patients
A large multi-center cohort analysis was presented in the present study. These present results revealed that there was no significant difference in long-term outcomes among the different surgical modalities, which strongly suggests that indirect revascularization is good enough to improve cerebral hemodynamics and reduce the incidence of subsequent ischemic events in pediatric MMD
Summary
Moyamoya disease (MMD) is a progressive narrowing or occlusion cerebrovascular disease characterized by bilateral stenosis of the internal carotid arteries (ICA) and the development of a compensatory network of basal collaterals [1,2,3]. MMD is one of the most common pediatric cerebrovascular diseases in Eastern Asian countries that causes stroke in children [4]. Surgical revascularization has been widely accepted for the treatment of MMD to prevent ischemic symptoms [6]. The optimal surgical candidates for children with MMD are not well-identified, and the objective evaluation of the efficacy of surgery and outcomes of conservative treatment is needed [8,9,10,11]. The data of 303 pediatric MMD patients from a multicenter cohort study conducted between September 2012 and May 2017 were presented. The demographics and clinical characteristics were investigated, and the effects of different surgical modalities and conservative treatment were evaluated. A risk factor analysis was conducted to evaluate the independent prognostic factors for clinical outcome
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