title: Post-thrombotic syndrome in children: a systematic review of frequency of occurrence, validity of outcome measures, and prognostic factors.
abstract: BACKGROUND
  Post-thrombotic syndrome is a manifestation of chronic venous insufficiency following deep venous thrombosis. This systematic review was conducted to critically evaluate pediatric evidence on frequency of occurrence, validity of outcome measures, and prognostic indicators of post-thrombotic syndrome.
  
  
  METHODS
  A comprehensive literature search of original reports revealed 19 eligible studies, totaling 977 patients with upper/lower extremity deep venous thrombosis. Calculated weighted mean frequency of post-thrombotic syndrome was 26% (95% confidence interval: 23-28%) overall, and differed significantly by prospective/non-prospective analysis and use/non-use of a standardized outcome measure.
  
  
  RESULTS
  Standardized post-thrombotic syndrome outcome measures included an adaptation of the Villalta scale, the Clinical-Etiologic-Anatomic-Pathologic classification, and the Manco-Johnson instrument. Data on validity were reported only for the Manco-Johnson instrument. No publications on post-thrombotic syndrome-related quality of life outcomes were identified. Candidate prognostic factors for post-thrombotic syndrome in prospective studies included use/non-use of thrombolysis and plasma levels of factor VIII activity and D-dimer.
  
  
  CONCLUSIONS
  Given that affected children must endure chronic sequelae for many decades, it is imperative that future collaborative pediatric prospective cohort studies and trials assess as key objectives and outcomes the incidence, severity, prognostic indicators, and health impact of post-thrombotic syndrome, using validated measures.
authors.affiliation: Department of Pediatrics, Section of Hematology/Oncology/Bone Marrow Transplantation and the Mountain States Regional Hemophilia and Thrombosis Center, Univ of Colorado Denver and The Children’s Hospital, Aurora, CO 80045, USA. neil.goldenberg@ucdenver.edu
authors.forename: Neil A
authors.initials: NA
authors.lastname: Goldenberg
authors.forename: Marco P
authors.initials: MP
authors.lastname: Donadini
authors.forename: Susan R
authors.initials: SR
authors.lastname: Kahn
authors.forename: Mark
authors.initials: M
authors.lastname: Crowther
authors.forename: Gili
authors.initials: G
authors.lastname: Kenet
authors.forename: Ulrike
authors.initials: U
authors.lastname: Nowak-Göttl
authors.forename: Marilyn J
authors.initials: MJ
authors.lastname: Manco-Johnson
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D002986:Clinical Trials as Topic; D005260:Female; D006801:Humans; D008297:Male; D054070:Postthrombotic Syndrome / Q000453:epidemiology* / Q000209:etiology / Q000628:therapy; D011788:Quality of Life; D020246:Venous Thrombosis / Q000150:complications / Q000453:epidemiology* / Q000628:therapy