title: Pial synangiosis for moyamoya syndrome in children with sickle cell anemia: a comprehensive review of reported cases.
abstract: OBJECTIVE
  Pediatric patients with sickle cell anemia (SCA) carry a significant risk of developing moyamoya syndrome (MMS) and brain ischemia. The authors sought to review the safety and efficacy of pial synangiosis in the treatment of MMS in children with SCA by performing a comprehensive review of all previously reported cases in the literature.
  
  
  METHODS
  The authors retrospectively reviewed the clinical and radiographic records in 17 pediatric patients with SCA treated at the Morgan Stanley Children's Hospital of New York (MSCHONY) who developed radiological evidence of MMS and underwent pial synangiosis between 1996 and 2012. The authors then added any additional reported cases of pial synangiosis for this population in the literature for a combined analysis of clinical and radiographic outcomes.
  
  
  RESULTS
  The combined data consisted of 48 pial synangiosis procedures performed in 30 patients. Of these, 27 patients (90%) presented with seizure, stroke, or transient ischemic attack, whereas 3 (10%) were referred after transcranial Doppler screening. At the time of surgery, the median age was 12 years. Thirteen patients (43%) suffered an ischemic stroke while on chronic transfusion therapy. Long-term follow-up imaging (MR angiography or catheter angiography) at a mean of 25 months postoperatively was available in 39 (81%) treated hemispheres. In 34 (87%) of those hemispheres there were demonstrable collateral vessels on imaging. There were 4 neurological events in 1590 cumulative months of follow-up, or 1 event per 33 patient-years. In the patients in whom complete data were available (MSCHONY series, n = 17), the postoperative stroke rate was reduced more than 6-fold from the preoperative rate (p = 0.0003).
  
  
  CONCLUSIONS
  Pial synangiosis in patients with SCA, MMS, and brain ischemia appears to be a safe and effective treatment option. Transcranial Doppler and/or MRI screening in asymptomatic patients with SCA is recommended for the diagnosis of MMS.
authors.affiliation: Department of Neurological Surgery, Columbia University Medical Center, New York, New York; and.
authors.forename: Benjamin C
authors.initials: BC
authors.lastname: Kennedy
authors.forename: Michael M
authors.initials: MM
authors.lastname: McDowell
authors.forename: Peter H
authors.initials: PH
authors.lastname: Yang
authors.forename: Caroline M
authors.initials: CM
authors.lastname: Wilson
authors.forename: Sida
authors.initials: S
authors.lastname: Li
authors.forename: Todd C
authors.initials: TC
authors.lastname: Hankinson
authors.forename: Neil A
authors.initials: NA
authors.lastname: Feldstein
authors.forename: Richard C E
authors.initials: RC
authors.lastname: Anderson
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000503:physiopathology; D002545:Brain Ischemia / Q000601:surgery; D002533:Cerebral Angiography; D002550:Cerebral Veins / Q000601:surgery*; D002648:Child; D015331:Cohort Studies; D005260:Female; D006801:Humans; D002546:Ischemic Attack, Transient / Q000209:etiology / Q000601:surgery; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D009072:Moyamoya Disease / Q000000981:diagnostic imaging / Q000601:surgery*; D019635:Neurosurgical Procedures / Q000009:adverse effects / Q000379:methods*; D011183:Postoperative Complications / Q000453:epidemiology; D020521:Stroke / Q000209:etiology / Q000601:surgery; D016896:Treatment Outcome; D055815:Young Adult