Clinical Features, Surgical Treatment and Long-Term Outcome in Adult Patients with Moyamoya Disease in China

Abstract

Background: Moyamoya disease (MMD) develops mostly in Asian countries including Japan, Korea, mainland China and Taiwan. However, there are few detailed demographic and clinical data about Chinese patients with MMD. Currently, the most effective treatment in adult patients with MMD is unknown. There have only been a few small case series reporting on encephaloduroarteriosynangiosis (EDAS) in an adult population. Here we describe the clinical features, surgical treatment and long-term outcome of adults with MMD treated at a single institution in China. Methods: Our cohort included 470 adult patients with MMD. The demographic and clinical characteristics were obtained by retrospective chart review and long-term outcome was evaluated using the stroke status. The modified Rankin Scale (mRS) was used to determine the neurological functional outcome. Univariate and multivariate logistic regression analyses were performed to determine risk factors for postoperative morbidity and functional outcome. The risk of subsequent stroke was determined using the Kaplan-Meier method and Cox regression was used to determine risk factors for postoperative or subsequent strokes. Results: The median age for the onset of symptoms was 36.8 (range, 18–59) years. The ratio of female to male patients was 1:1 (231/239). Familial occurrence of MMD was 2.3%. The most common initial symptom was a cerebral ischemic event. The incidence of postoperative ischemic events or hemorrhage was 5.9% (9.8% of patients). Older age at symptom onset, posterior cerebral artery (PCA) involvement and the presence of transient ischemic attack (TIA) were identified as predictors of adverse postoperative events. The Kaplan-Meier estimate stroke risk was 10.1% in the first 2 years, and the 5-year Kaplan-Meier risk of stroke was 13% after surgery for all patients treated with surgical revascularization. Older age at symptom onset, PCA involvement and the presence of TIA were identified as predictors of postoperative or subsequent strokes. Overall, 73.2% of patients had an independent life with no significant disability, with the strongest predictor being the preoperative mRS score. Conclusion: Clinical characteristics of adult MMD in China are different from those in other Asian countries. EDAS in adult patients with MMD carries a low risk, is effective at preventing future ischemic events and improves quality of life.

Surgical outcomes following encephaloduroarteriosynangiosis in adult moyamoya disease associated with Type 2 diabetes

“…According to the multivariate logistic regression analyses, our study showed that PCA involvement in both groups with no statistically significant differences preoperatively together with the late postoperative stroke was associated with unfavorable clinical outcomes, which was similar to the previous results observed in our center. 5 We speculate that PCA stenoses may influence regional CBF and cerebrovascular reactivity and then impair cerebral perfusion, which could lead to ischemic symptoms postoperatively. However, the limited number of patients and lack of evaluation on blood flow and metabolism before and after the surgery restrict our further research.…”

“…25 There is wide controversy regarding the merits and shortcomings of the 2 surgical approaches for adults with MMD. 2,5,7,23,31 Starke et al reviewed the literature concerning the surgical treatment and concluded that both direct and indirect bypass were effective. 30 Since there is a lack of randomized trials, it was noted that the selection of the surgical modalities depended on the preference and surgical technique mastered by the cerebrovascular neurosurgeon.…”

“…5,6,11,14,32,34 Preoperative cerebral infarction, younger age at onset, involvement of the PCA, intentional hypotension, high Suzuki stage, and postoperative ischemic events have been observed to correlate with postoperative complications and outcomes. According to the multivariate logistic regression analyses, our study showed that PCA involvement in both groups with no statistically significant differences preoperatively together with the late postoperative stroke was associated with unfavorable clinical outcomes, which was similar to the previous results observed in our center.…”

“…25 Encephaloduroarteriosynangiosis (EDAS) is an indirect revascularization procedure that has been shown to be beneficial for pediatric 6,12,22 and adult populations. 2,5,7 The results of revascularization due to EDAS are associated with some factors, including age, complications, and other unknown factors.…”

Long-Term Outcomes After Combined Revascularization Surgery in Adult Moyamoya Disease

“…1 Although the pathogenesis is not yet known, revascularization surgery has shown satisfactory results in pediatric and adult MMD. [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] Indirect revascularization surgeries, such as encephaloduroarteriosynangiosis and other variants, are generally accepted as standard for pediatric MMD. 3,4,7,9,12 In adult MMD, however, there is still controversy about the effectiveness of some types of revascularization surgeries because there have been no randomized studies to compare the efficacy of surgical techniques, and several retrospective studies have been conducted with various age ranges, small numbers of patients, short-term follow-up, and no standardization of surgical techniques.…”