Qian-Nan Wang, Xiang-Yang Bao, Yong Zhang, Qian Zhang, De-Sheng Li and Lian Duan
The objective of this study was to investigate long-term outcomes after encephaloduroarteriosynangiosis (EDAS) for the treatment of hemorrhagic moyamoya disease (MMD) and identify the risk factors for recurrent hemorrhages.
The authors retrospectively reviewed 95 patients with hemorrhagic MMD who were treated with EDAS at 307th Hospital PLA. Clinical features, angiographic findings, and clinical outcomes were investigated. Rebleeding incidences were compared between anterior or posterior hemorrhagic sites. Kaplan-Meier survival analysis and Cox proportional hazards regression models were used to estimate rebleeding risks after EDAS.
The average age at symptom onset was 37.1 years (range 20–54 years) for adult patients. The ratio of female to male patients was 1.16:1. In 61 of 95 hemorrhagic hemispheres (64.2%), the anterior choroidal artery (AChA) or posterior communicating artery (PCoA) was extremely dilated, with extensive branches beyond the choroidal fissure, which only occurred in 28 of 86 nonhemorrhagic hemispheres (32.6%). Fifty-seven incidences were classified as anterior hemorrhages and 38 as posterior. Sixteen of 95 patients (16.8%) suffered cerebral rebleeding after a median follow-up duration of 8.5 years. The annual rebleeding rate was 2.2% per person per year. The incidence rate was higher for the posterior group than for the anterior group, but this difference was not statistically significant (p > 0.05). Cox regression analysis revealed that the age of symptom onset (OR 1.075, 95% CI 1.008–1.147, p = 0.028) was a predictor of rebleeding strokes.
Through long-term follow up, EDAS proved beneficial for patients with hemorrhagic MMD. Dilation of the AChA-PCoA is associated with the initial hemorrhage of MMD, and rebleeding is age-related. Patients with hemorrhagic MMD should undergo follow-up over the course of their lives, even when neurological status is excellent.
Yong Zhang, Xiang-Yang Bao, Lian Duan, Wei-Zhong Yang, De-Sheng Li, Zheng-Shan Zhang, Cong Han, Feng Zhao, Qian Zhang and Qian-Nan Wang
The object of this study was to summarize the long-term effect of encephaloduroarteriosynangiosis (EDAS) for the treatment of pediatric moyamoya disease (MMD) and to investigate factors influencing the clinical outcomes of EDAS.
Clinical features, angiographic findings, and clinical outcomes were analyzed among MMD patients younger than 18 years who had been treated with EDAS between 2002 and 2007 at the authors’ institution. The Kaplan-Meier method was used to estimate stroke risk after EDAS. Predictors of neurological outcome were assessed.
One hundred fifteen patients were identified. The mean age at symptom onset was 7.3 ± 4.0 years. The incidence of familial MMD was 11.3%. The female/male ratio was 1:1.16. A total of 232 EDAS procedures were performed, and the incidence of postoperative complications was 3%. Postoperative digital subtraction angiography was performed in 54% of the patients, and about 80% of the hemispheres showed good or excellent results. Neovascularization showed significant correlations with delay time (from symptom onset to first operation), Suzuki stage, and preoperative stroke (all p < 0.05). Clinical follow-up was available in 100 patients with a mean follow-up of 124.4 ± 10.5 months. Ten-year cumulative survival was 96.5% after surgery, and the risk of stroke was 0.33%/person-year. An independent life with no significant disability was reported by 92% of the patients. A good outcome correlated with a low Suzuki stage (p = 0.001). Older children and those without preoperative stroke had better clinical outcomes (p < 0.05).
On the basis of long-term follow-up data, the authors concluded that EDAS is a safe and effective treatment for pediatric MMD, can reduce the risk of subsequent neurological events, and can improve quality of life. The risk of ischemia-related complications was higher in younger patients, and older children showed better outcomes. Compensation was greater with more prominent cerebral ischemia. The long-term clinical outcome largely depended on the presence and extent of preoperative stroke.
Bin Ren, Zheng-Shan Zhang, Wei-Wei Liu, Xiang-Yang Bao, De-Sheng Li, Cong Han, Peng Xian, Feng Zhao, Hui Wang, Hai Wang and Lian Duan
Debate exists regarding the merits and shortcomings of an indirect bypass procedure for treating adult patients with moyamoya disease (MMD). Considerable variation in neovascularization occurs among different organs in patients with diabetes mellitus. Here, the effect of encephaloduroarteriosynangiosis on MMD associated with Type 2 diabetes mellitus (T2DM) is evaluated.
A retrospective and 1:2 matched case-control study was conducted in moyamoya patients with or without T2DM (n = 180). Postoperative collateral formations were graded according to the Modified Collateral Grading System that originated from the Matsushima Angiographic Stage Classification. Neurological function outcomes before and after the operation were evaluated according to the modified Rankin Scale. Univariate and multivariate logistic regression analyses were performed to determine the risk factors for clinical outcome.
There was no statistically significant difference in the constituent ratios of initial symptom and preoperative Suzuki stage between patients with and without T2DM. Progression of angiopathy around the circle of Willis was postoperatively observed in bilateral internal carotid arteries in both groups. Patients with T2DM had a higher postoperative Suzuki stage (p < 0.01) and more frequent development of collateral angiogenesis germinating from the external carotid after indirect revascularization procedures in the surgical cerebral hemisphere (82.7% vs 72.2%; p < 0.05). The extent of postoperative collateral formation in patients with diabetes mellitus was significantly higher (p < 0.01). Postoperative clinical improvement in the diabetes group was more common after revascularization procedures (p < 0.05), and the diabetes group had lower modified Rankin Scale scores (p < 0.05) in comparison with the nondiabetes group. Late postoperative stroke and posterior cerebral artery involvement were identified as predictors of unfavorable clinical outcome in both groups, while T2DM was associated with a favorable clinical outcome.
Encephaloduroarteriosynangiosis is an efficacious treatment for adult patients with MMD. Patients with T2DM could achieve better collateral circulation and clinical improvement following surgery.