title: Long-term follow-up of surgically treated juvenile patients with Moyamoya disease.
abstract: OBJECTIVE
  Surgical revascularization is considered an effective treatment for juvenile patients with moyamoya disease (MMD). Yet the long-term outcome in surgically treated patients still needs to be clarified. More than 30 years have passed since the authors' department started intensively treating pediatric patients with MMD using indirect anastomosis techniques. In this study the authors surveyed the current status of these patients.
  
  
  METHODS
  Activities of daily living (ADLs) were surveyed and present clinical status was assessed based on the modified Rankin scale (mRS). Cerebrovascular events subsequent to surgical treatment were also recorded.
  
  
  RESULTS
  Since 1979, 208 patients younger than 19 years of age with MMD were surgically treated and followed up for > 3 years. Data were available on 172 patients (83%), who had been followed up for a mean of 14.3 years (range 3-32 years). Activity of daily living outcomes were as follows: 138 patients (80.2%) had mRS scores of 0-2, 29 (16.9%) a score of 3, 1 (0.6%) a score of 4, 1 (0.6%) a score of 5, and 3 (1.7%) a score of 6. Cerebrovascular events occurred 8 or more years after surgery in 6 patients (3.4%), that is, 6 hemorrhages and 3 infarctions. The cumulative risk of late-onset stroke at 10, 20, and 30 years after surgical intervention was 0.8%, 6.3%, and 10.0%, respectively.
  
  
  CONCLUSIONS
  This long-term survey demonstrated that most surgically treated pediatric patients with MMD maintain good ADL outcomes. However, a significant number of new cerebrovascular events occurred more than 10 years after the initial surgery. Additional follow-up will help to identify which events may occur during the adult years of patients treated as children.
authors.affiliation: Department of Neurosurgery, Tokyo Medical and Dental University, Bunkyo-ku, Tokyo, Japan.
authors.forename: Maki
authors.initials: M
authors.lastname: Mukawa
authors.forename: Tadashi
authors.initials: T
authors.lastname: Nariai
authors.forename: Yoshiharu
authors.initials: Y
authors.lastname: Matsushima
authors.forename: Yoji
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Motoki
authors.initials: M
authors.lastname: Inaji
authors.forename: Taketoshi
authors.initials: T
authors.lastname: Maehara
authors.forename: Masaru
authors.initials: M
authors.lastname: Aoyagi
authors.forename: Kikuo
authors.initials: K
authors.lastname: Ohno
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009072:Moyamoya Disease / Q000601:surgery*; D013997:Time Factors; D055815:Young Adult