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.

No abstract

No abstract

Objective. The underlying factors of neovascularization after indirect bypass in the adult patients with moyamoya disease (MMD) remained unknow. The aim of this study was to explore potential predictors based on collateral characteristics for neovascularization after indirect bypass in adult MMD patients. Methods. The adult MMD patients treated by indirect bypass in a single-institution from August 2012 and January 2018 were retrospectively selected into our research. The collaterals based on cerebral angiography were classified into the following subtypes: intracerebral anastomosis, duro-cortical anastomosis, and leptomeningeal anastomosis. Neovascularization evaluation was based on Matsushima classification, with “good” collateral formation in level 2 and 3, and poor formation in level 0 and 1. Univariate and multivariate analyses were performed to identify neovascularization predictors after indirect bypass. Results. A total of 86 patients (97 hemispheres) (mean ± SD age 35.06 ± 15.09 years, range 18–61 years) were retrospectively included. Preoperative collateral circulation included intracerebral anastomosis in 49 (50.5%) cases, duro-cortical anastomosis in 19 (19.6%) cases, and leptomeningeal anastomosis in 29 (29.9%) cases, respectively. Postoperative good neovascularization was observed in 56 (57.7%) hemispheres. Multivariate analysis showed that intracerebral anastomosis (P<0.001, OR [95% CI] 2.984 [2.031-5.437]) was associated with favorable neovascularization, whereas older age (P<0.001, OR [95% CI] 0.827 [0.793-0.916]) and hemorrhagic onset (P<0.001, OR [95% CI] 0.138 [0.054–0.353]) were significantly associated with poor neovascularization. Hemispheres in the good neovascularization had lower modified Rankin scale score, and better long-term improvement than those in the poor neovascularization. Conclusions. Hemorrhagic onset and old age predict poor neovascularization after indirect bypass, while duro-cortical anastomosis and intracerebral anastomosis predict good neovascularization. Good neovascularization was associated with better long-term outcomes. The current study provides a basis for the selection of surgical procedure for MMD candidates.

No abstract