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Paget-Schroetter syndrome in 21 children: outcomes after multidisciplinary care
Cameron C Trenor1, Jeremy G Fisher2, Faraz A Khan2
1Division of Hematology/Oncology, Boston Children's Hospital, Boston, MA.
Insights
Pediatric Paget-Schroetter syndrome (PSS) is successfully treated with a multidisciplinary algorithm. This approach combines anticoagulation, thrombolysis, and thoracic outlet decompression for promising early outcomes in children with PSS.
Area of Science:
- Pediatric vascular surgery
- Interventional radiology
- Hematology
Background:
- Paget-Schroetter syndrome (PSS) is a rare condition affecting children.
- Understanding its presentation, management, and outcomes is crucial for effective treatment.
Purpose of the Study:
- To review the presentation, management, and outcomes of PSS in pediatric patients.
- To propose a multidisciplinary treatment algorithm for PSS in children.
Main Methods:
- Retrospective review of 21 pediatric patients with PSS (2003-2013).
- Development of a multidisciplinary treatment algorithm based on early cases.
- Application of the algorithm to later patients, involving venography, endovascular intervention, and surgical decompression.
Main Results:
- Common symptoms included edema, discoloration, and pain.
- Thrombophilia workup identified mild abnormalities in some patients.
- The multidisciplinary algorithm involved anticoagulation, catheter-directed thrombolysis, and thoracic outlet decompression.
- Postoperative complications were noted, with transient symptom recurrence in one patient.
Conclusions:
- A multidisciplinary treatment algorithm is effective for pediatric PSS.
- The approach integrates anticoagulation, thrombolysis, and surgical decompression.
- Early outcomes for pediatric PSS treated with this algorithm are promising.
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.
Study Design:
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.
Conclusion:
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.
