Paget-Schroetter syndrome or effort-related upper extremity deep vein thrombosis is a rare condition that usually afflicts young healthy individuals, most commonly males. The cause is multifactorial but almost always involves extrinsic compression of the subclavian vein at the thoracic inlet, causing venous stenosis from repetitive trauma. The diagnosis of this condition may be difficult, and its delay may contribute to potential complications including thrombosis progression, pulmonary embolism, thrombosis recurrence, and post-thrombotic syndrome. Similarly, the best therapeutic option has not been established and in the lack of evidence-based guidelines, treatment may be extremely challenging especially in children, in whom long-term complications can be particularly disabling.

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