Surgery remains the most effective treatment for Paget-Schroetter syndrome: 50 years' experience

Background. Significant improvements were made in the diagnosis and management of Paget-Schroetter syndrome (thrombosis of the axillary-subclavian vein) secondary to thoracic outlet syndrome during the past 50 years. The diagnosis has often been extremely difficult. Methods. Multiple approaches both in diagnosis and therapy have been tried during the years. After recognizing that the underlying pathologic process resulted from an abnormal insertion of the costoclavicular ligament laterally on the first rib, along with hypertrophy of the scalenus anticus muscle, 506 of 626 extremities have been managed by thrombolytic therapy followed by prompt transaxillary resection of the first rib. These patients have been followed up from 1 to 32 years (average of 7.2 years +/- 1.0 standard deviation). Results. Four hundred eighty-six patients (96%) improved. Because the pathophysiology is not well understood, many venograms suggest intraluminal disease rather than external compression. Therefore, attempts at opening the narrowed vein with intraarterial techniques do not work. Use of percutaneous venous angioplasty with stents have all occluded in our experience, making further management difficult. Venous bypass grafts fail because of low venous pressure. Conclusions. Recognition that an abnormal congenital lateral insertion of the costoclavicular ligament on the first rib causes venous occlusion in Paget-Schroetter syndrome has led to acute thrombolysis, followed by prompt first rib resection, as the ideal management.