Optimal management of upper extremity deep vein thrombosis: Is venous thoracic outlet syndrome underrecognized?

Upper extremity deep vein thrombosis (UEDVT) accounts for ~10% of all cases of DVT. In the most widely referenced general review of DVT, the American Academy of Chest Physicians essentially recommended that UEDVT be treated identically to that of lower extremity DVT, with anticoagulation the default therapy. However, the medical literature has not differentiated well between DVT in the arm vs DVT in the leg and has not emphasized the effects of the costoclavicular junction and the lack of the effect of gravity to the point at which UEDVT due to extrinsic bony compression at the costoclavicular junction is classified as "primary."

We performed a comprehensive literature review, beginning with both Medline and Google Scholar searches, in addition to collected references. Next, we manually reviewed the relevant citations within the initial reports studied. Both surgical and medical journals were explored.

It has been proposed that "effort thrombosis" should be classified as a secondary cause of UEDVT, limiting the definition of "primary" to that which is truly idiopathic. Other causes of secondary UEDVT include catheter- and pacemaker-related thrombosis (the most common cause but often asymptomatic), thrombosis related to malignancy and hypercoagulable conditions, and the rare case of thrombosis due to compression of the vein by a focal malignancy or other space-occupying lesion. In true primary UEDVT and those secondary cases in which no mechanical cause is present or can be corrected, anticoagulation remains the treatment of choice, usually for 3 months or the duration of a needed catheter. However, evidence has suggested that many cases of effort thrombosis are likely missed by a too-narrow adherence to this protocol.

Because proper treatment of effort thrombosis would decrease the long-term symptomatic status rate from 50% to almost 0% and because these are healthy patients with a long lifespan, we believe that a more aggressive attitude toward thrombolysis should be followed for any patient with a reasonable degree of suspicion for venous thoracic outlet syndrome.