Paget-Schroetter syndrome, or venous thoracic outlet syndrome, has been recently described in children. This syndrome is characterized by upper extremity deep vein thrombosis after subclavian vein compression and impingement (usually the result of repeated overhead movements in athletes). In 2006, a Canadian pediatric case-series showed that anticoagulation alone has relatively poor outcomes.
But a recent study led by physician-researchers at Nationwide Children’s Hospital has shown that anticoagulation with decompression rib-resection surgery produces excellent outcomes, allowing those young athletes to return to normal activities.
The study, published in The Journal of Pediatrics, is based on the largest cohort of childhood Paget-Schroetter syndrome reported to date.
“This syndrome is rare enough that some institutions may never have seen it, and they may consider trying anticoagulation alone as a therapy,” says Riten Kumar, MD, MSc, a member of the Division of Hematology, Oncology and Blood and Marrow Transplant at Nationwide Children’s and lead author of the study. “In conjunction with earlier, smaller studies, our study shows that patients with Paget-Schroetter syndrome should be referred to a center that has the ability to offer a multipronged management strategy.”
The study considered 22 patients at Nationwide Children’s who were diagnosed with Paget-Schroetter syndrome. Median age at diagnosis was 16 years. All were treated with anticoagulation and decompression surgery, and 19 of 22 had thrombus resolution (determined by imaging).
The authors also attempted to contact those patients, sometimes years after treatment, to assess the presence or severity of post-thrombotic syndrome and to investigate health-related quality-of-life. A total of 19 of 22 patients responded to validated survey instruments, and while 14 of those reported some signs or symptoms of post-thrombotic syndrome – only 2 patients reported moderate symptoms. Patients reported excellent quality of life scores.
Additionally, the study compared patients who had initial thrombolytic therapy and those who did not. All patients underwent decompression surgery and received approximately three months of anticoagulation; ten patients (45%) also received thrombolytic therapy. Clinical and radiological outcomes were similar in both the thrombolysis and non-thrombolysis groups.
“The utility of thrombolysis in these and other pediatric thrombotic conditions remains controversial,” says Bryce Kerlin, MD, also a member of the Division of Heme/Onc/BMT at Nationwide Children’s and senior author of the study. “Many hematologists reserve thrombolysis for management of life- or limb-threatening thrombosis, but there is compelling scientific rationale that early thrombolysis may improve long-term outcomes in carefully selected cases.”
While this study was not powered or designed to settle this debate, it does question the need for upfront thrombolysis in Paget-Schroetter syndrome. A randomized controlled trial would be the ideal method to definitively resolve this question, but attempting such a study may be futile in the absence of a prolonged enrollment strategy in a multi-institutional study network due to the rarity of Paget-Schroetter syndrome, according to the authors.
Kumar R, Harsh K, Saini S, O'Brien SH, Stanek J, Warren P, Giver J, Go MR, Kerlin BA. Treatment-related outcomes in Paget-Schroetter syndrome – a cross-sectional investigation. The Journal of Pediatrics. 2018 Dec 7. [Epub ahead of print]