Encephalo-duro-arterio-synangiosis For Pediatric Moyamoya Disease:Long-term Follow-up

Objective Moyamoya disease(MMD)is a chronic occlusive cerebrovascular disease.Revascularization surgery for symptomatic MMD is considered the standard treatment for preventing further stroke.One of the peaks of incidence are children,and indirect revascularization was chosen to perform.Encephalo-duro-arterio-synangiosis(EDAS)is popular in pediatric patients as an indirect revascularization.However,there are few long-term,large-sample follow-up studies of EDAS for pediatric MMD.Our institution is one of the center for the treatment of MMD.So this study selected patients treated 10 years ago at our institution,to summarize the long-term effect of EDAS in the treatment of pediatric MMD,and investigate factors influencing clinical outcomes after EDAS.Methods A retrospective study was carried out focusing on patients with MMD,<18 years of age treated using EDAS between 2002 and 2007 at our institution.Clinical features,angiographic findings,and clinical outcomes were analyzed.The Kaplan-Meier method was used to estimate stroke risk after EDAS.Predictors of neurological outcome were assessed.Results We initially selected 115 patients,the mean age at symptom onset was 7.3±4.0 years.Familial occurrence was 11.3%.The female to male ratio was 1:1.16.The initial symptoms were transient ischemic attack(63),infarction(31),headache(16),hemorrhage(1),or else(4).The angiographic Suzuki stages were 1(3.48%),2(8.70%),3(27.83%),4(25.22%),5(26.96%),and 6(7.83%).A total of 232 EDAS procedures were performed,the postoperative complications occurred in 7 patients.Follow-up digital subtraction angiography(DSA)was performed in 63(54%)patients(at a mean of 36.5±32.9 months),and about 111 hemispheres showed obvious results.Excellent neovascularization was achieved in 65 hemispheres(57.02%),good in 26(22.81%),and fair in 20(17.54%).Neovascularization showed significant correlations with Suzuki stage and preoperative stroke(p<0.05).Clinical follow up was available in 100 patients for a mean interval of 124.4±10.5 months.The overall final clinical outcome was excellent in 67%,good in 25%,fair in 4%,and poor in 4%.Cerebrovascular events occurred in 4 patients.The annual risk of stroke was 0.33%.An independent life with no significant disability was reported by 92% of patients.Good outcome correlated with low Suzuki stage,older children and those without preoperative stroke had better clinical outcomes(p<0.05).Conclusions On the basia long-term follow up data,EDAS is an effective and safe treatment option for pediatric MMD,and can reduce the risk of subsequent neurologic events and improve the quality of life.The risk of ischemia-related complications was higher in younger patients,and older children showed better outcome.Compensation was greater with more prominent cerebral ischemia.The long-term clinical outcome largely depended on the presence and extent of preoperative stroke.