INTRODUCTION

Primary, "spontaneous" upper extremity deep vein thrombosis is rare and is defined as thrombosis of the deep veins draining the upper extremity due to anatomic abnormalities of the thoracic outlet causing axillosubclavian compression and subsequent thrombosis. The syndrome is appropriately termed venous thoracic outlet syndrome, but is also referred to as Paget-Schroetter syndrome, and alternatively as ‘effort’ thrombosis’ [1]. It typically presents in young, otherwise healthy individuals as sudden, severe upper extremity pain and swelling following vigorous upper extremity activity. An aggressive treatment approach that includes anticoagulation, catheter-directed thrombolysis and thoracic outlet decompression is aimed at relieving acute symptoms, and minimizing complications including recurrent thromboembolism and post-thrombotic syndrome.

The epidemiology, risk factors, pathophysiology, clinical features, diagnosis and treatment of primary (spontaneous) upper extremity venous thrombosis will be reviewed here. Catheter-induced upper extremity venous thrombosis and lower extremity deep vein thrombosis are discussed elsewhere. (See "Catheter-induced upper extremity venous thrombosis" and "Approach to the diagnosis and therapy of lower extremity deep vein thrombosis".)

UPPER EXTREMITY ANATOMY

The upper extremity veins are divided into the superficial and deep venous systems (figure 1).

Superficial veins — The main superficial veins of the upper extremity include the cephalic, basilic, median cubital, and accessory cephalic veins (figure 1). The basilic vein is a common access site for performing digital subtraction venography.

Deep veins — The deep veins of the upper extremity include the paired ulnar, radial and interosseous veins in the forearm, paired brachial veins of the upper arm, and axillary vein. The axillary vein becomes the subclavian vein at the lower border of the teres major muscle (figure 2).