title: Paget-Schroetter syndrome in 21 children: outcomes after multidisciplinary care.
abstract: OBJECTIVE
  To review the presentation, management, and outcomes of Paget-Schroetter syndrome (PSS) in children and propose a multidisciplinary treatment algorithm involving pediatric and vascular surgery, interventional radiology, and hematology.
  
  
  METHODS
  Patients with PSS presenting between 2003 and 2013 were reviewed. Demographics, symptoms, therapies, and functional outcomes were noted. Data from early patients informed the development of a multidisciplinary treatment algorithm applied to later patients.
  
  
  RESULTS
  Of 21 patients, mean ± SD age was 16 ± 1.6 years and 11 (52%) were male. Of patients with complete presentation data, common symptoms were edema (84%), discoloration (58%), and pain (58%). Thrombophilia workup revealed one heterozygote for factor V Leiden, 2 patients with factor VIII elevation and 1 patient with mildly low antithrombin. The most recent 8 patients were treated according to an algorithm developed by a multidisciplinary working group through experience with the first 13 cases. All patients underwent a venogram, endovascular intervention (including 15 receiving catheter-directed thrombolysis), and operative ipsilateral thoracic outlet decompression (first rib resection, anterior scalenectomy, and venolysis). Postoperative complications included hemothorax (2), pneumothorax (1), and recurrent thrombosis (2). Follow up duration was 12 ± 9.5 months. Symptoms recurred transiently in 1 patient.
  
  
  CONCLUSIONS
  Pediatric patients with PSS can be treated successfully using a multidisciplinary treatment algorithm including anticoagulation, catheter-directed thrombolysis, and operative decompression of the thoracic outlet. Early outcomes are promising.
authors.affiliation: Division of Hematology/Oncology, Boston Children's Hospital, Boston, MA.
authors.forename: Cameron C
authors.initials: CC
authors.lastname: Trenor
authors.affiliation: Department of Surgery, Boston Children's Hospital, Boston, MA.
authors.forename: Jeremy G
authors.initials: JG
authors.lastname: Fisher
authors.affiliation: Department of Surgery, Boston Children's Hospital, Boston, MA.
authors.forename: Faraz A
authors.initials: FA
authors.lastname: Khan
authors.affiliation: Department of Surgery, Boston Children's Hospital, Boston, MA.
authors.forename: Eric A
authors.initials: EA
authors.lastname: Sparks
authors.affiliation: Division of Hematology/Oncology, Boston Children's Hospital, Boston, MA.
authors.forename: Juliann
authors.initials: J
authors.lastname: Duzan
authors.affiliation: Division of Hematology/Oncology, Boston Children's Hospital, Boston, MA.
authors.forename: Kathy
authors.initials: K
authors.lastname: Harney
authors.affiliation: Division of Interventional Radiology, Boston Children's Hospital, Boston, MA.
authors.forename: Brian
authors.initials: B
authors.lastname: Dillon
authors.affiliation: Division of Vascular Surgery, Brigham and Women's Hospital, Boston, MA.
authors.forename: Matthew
authors.initials: M
authors.lastname: Menard
authors.affiliation: Department of Surgery, Boston Children's Hospital, Boston, MA. Electronic address: biren.modi@childrens.harvard.edu.
authors.forename: Biren P
authors.initials: BP
authors.lastname: Modi
mesh_terms: D000293:Adolescent; D000465:Algorithms; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D010348:Patient Care Team; D012189:Retrospective Studies; D016896:Treatment Outcome; D056824:Upper Extremity Deep Vein Thrombosis / Q000628:therapy*