A comprehensive review of Paget-Schroetter syndrome

Venous thoracic outlet syndrome progressing to the point of axilosubclavian vein thrombosis, variously referred to as Paget-Schroetter syndrome or effort thrombosis, is a classic example of an entity which if treated correctly has minimal long-term sequelae but if ignored is associated with significant long-term morbidity. The subclavian vein is highly vulnerable to injury as it passes by the junction of the first rib and clavicle in the anterior-most part of the thoracic outlet. In addition to extrinsic compression, repetitive forces in this area frequently lead to fixed intrinsic damage and extrinsic scar tissue formation. Once primary thrombosis is recognized, catheter-directed thrombolytic therapy is usually successful if initiated within ten to 14 days of clot formation, but often unmasks an underlying lesion. The vast majority of investigators believe that decompression of the venous thoracic outlet, usually by means of first rib excision, partial anterior scalenectomy, resection of the costoclavicular ligament, and thorough external venolysis, is necessary, although opinion is less uniform as to the need for and method of treatment of the venous lesion itself. Using this algorithm, long-term success rates of 95 to 100% have been reported by many investigators. This review, in addition to discussing the overall treatment algorithm in more detail, attempts to point out controversies that still exist and research directions, both clinical and basic, that need to be pursued. Prospective randomized trials addressing this entity are surprisingly lacking, and although there is consensus based on experience, it may be necessary to step back and rigorously explore several aspects of this entity.