title: Clinical features, surgical treatment, and long-term outcome in pediatric patients with moyamoya disease in China.
abstract: BACKGROUND
  There was few detailed demographic and clinical data about Chinese patients with moyamoya disease. Here we describe the clinical features, surgical treatment, and long-term outcome of pediatric patients with moyamoya disease at a single institution in China.
  
  
  METHODS
  Our cohort included 288 pediatric patients with moyamoya disease. The demographic and clinical characteristics were obtained by retrospective chart review and long-term outcome was evaluated using the stroke status. Univariate and multivariate logistic regression analyses were performed to determine the risk factors for clinical outcome. The risk of subsequent stroke was determined using the Kaplan-Meier method.
  
  
  RESULTS
  The median age for the onset of symptoms was 8.0 years. The ratio of female to male patients was 1:1. Familial occurrence of moyamoya disease was 9.4%. The incidence of postoperative complications was 4.2%. Postoperative ischemic events were identified as predictors of unfavorable clinical outcome, while older age of symptom onset was associated with a favorable clinical outcome. The Kaplan-Meier estimate stroke risk was 5% in the first 2 years, and the 5-year-Kaplan-Meier risk of stroke was 9% after surgery for all patients treated with surgical revascularization. Overall, 86% of patients had an independent life with no significant disability.
  
  
  CONCLUSIONS
  This long-term survey demonstrated that most surgically treated pediatric patients with MMD maintain good outcomes. Our results indicate that an early diagnosis and active intervention before the establishment of irreversible hemodynamic change are essential to achieve a favorable clinical outcome.
authors.affiliation: Department of Neurosurgery, 307 Hospital, PLA, The Center for Cerebral Vascular Disease, PLA, Beijing, China.
authors.forename: Xiang-Yang
authors.initials: XY
authors.lastname: Bao
authors.forename: Lian
authors.initials: L
authors.lastname: Duan
authors.forename: Wei-Zhong
authors.initials: WZ
authors.lastname: Yang
authors.forename: De-Sheng
authors.initials: DS
authors.lastname: Li
authors.forename: Wei-Jian
authors.initials: WJ
authors.lastname: Sun
authors.forename: Zheng-Shan
authors.initials: ZS
authors.lastname: Zhang
authors.forename: Rui
authors.initials: R
authors.lastname: Zong
authors.forename: Cong
authors.initials: C
authors.lastname: Han
mesh_terms: D000293:Adolescent; D002533:Cerebral Angiography; D002548:Cerebral Revascularization / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D002681:China; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D018810:Magnetic Resonance Angiography; D008297:Male; D009072:Moyamoya Disease / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D012189:Retrospective Studies; D020521:Stroke / Q000209:etiology