title: Hemorrhagic moyamoya disease in children: clinical features and surgical outcome.
abstract: OBJECTIVE
  The clinical presentation of moyamoya disease (MMD) typically includes cerebral ischemia in children and intracranial hemorrhage in adults. Because of its rarity, the benefit of surgery in the hemorrhagic type of pediatric MMD has not been clearly established. The purpose of this study was to delineate the clinical features and surgical outcome of hemorrhagic MMD in children.
  
  
  METHODS
  Thirteen patients (six boys and seven girls with a combined mean age of 11.1 years and a range of 4-18 years) were retrospectively confirmed as having MMD with intracranial hemorrhage between 1988 and 2009. All of the patients underwent indirect bypass surgery that followed a relatively uniform scheme at a single institution. We retrospectively reviewed their clinical features and surgical outcome. The mean time of follow-up was 50.3 months (with a range of 3-99 months).
  
  
  RESULTS
  Six of the patients presented with intracerebral hemorrhage (ICH), and six others presented with intraventricular hemorrhage (IVH); the remaining patient presented with both ICH and IVH. Preoperative angiography demonstrated that the patients were in various stages of the disease, but the majority of the patients (i.e., 16 of the 26 hemispheres) were in stage 3 with abundant moyamoya vessels (MMV). Three of four patients who presented with a history of prior hemorrhage or infarct exhibited a poor clinical outcome. Five patients with good revascularization had a decreased degree of MMV in their follow-up angiogram and showed good clinical outcome. Recurrent bleeding occurred after surgery in one patient.
  
  
  CONCLUSIONS
  Revascularization surgery may have a role for prevention of rebleeding and cerebral infarction in pediatric MMD patients who present with intracranial hemorrhage.
authors.affiliation: Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, Republic of Korea.
authors.forename: Jun Hyong
authors.initials: JH
authors.lastname: Ahn
authors.forename: Kyu-Chang
authors.initials: KC
authors.lastname: Wang
authors.forename: Ji Hoon
authors.initials: JH
authors.lastname: Phi
authors.forename: Ji Yeoun
authors.initials: JY
authors.lastname: Lee
authors.forename: Byung-Kyu
authors.initials: BK
authors.lastname: Cho
authors.forename: In-One
authors.initials: IO
authors.lastname: Kim
authors.forename: Seung-Ki
authors.initials: SK
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D001921:Brain / Q000098:blood supply / Q000473:pathology; D002533:Cerebral Angiography; D002548:Cerebral Revascularization / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D020300:Intracranial Hemorrhages / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D008297:Male; D009072:Moyamoya Disease / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D012189:Retrospective Studies; D016896:Treatment Outcome