A Cellist With Paget-Schroëtter
A 21-year-old black man presented with acute onset of right arm and shoulder swelling with associated pain one day after performing 100 push-ups. He had been previously healthy and active with no significant past medical history. In particular, he had no antecedent history of shoulder trauma or deep venous thrombosis. He was right-handed and played the cello up to 6 hours a day. Physical examination on admission was notable for edema involving the right upper arm, shoulder, and infraclavicular region. Abduction of the right arm was restricted because of pain. Jugular venous pressure was not elevated. No dilated superficial or collateral veins were seen on the anterior chest wall. No pulse or neurological deficits were noted in the right arm at baseline or with attempts at Adson maneuver (neck extension, head away from affected side, and a deep breath). Duplex ultrasound of the right upper extremity revealed acute nonocclusive thrombus in a dilated axillary vein (Figure 1). The vein walls were partially compressed with external transducer pressure, and spontaneous Doppler flow was minimal with delayed augmentation on forearm compression. No cervical rib was seen on chest x-ray, and computerized tomography angiography of the chest showed no pulmonary emboli. Prothrombotic screen was negative for activated protein-C resistance, prothrombin gene mutation G20210A, protein S and C deficiencies, antithrombin III deficiency, lupus anticoagulant, anticardiolipin antibodies, and hyperhomocysteinemia.
The patient was anticoagulated with intravenous unfractionated heparin on admission. His symptoms and edema gradually lessened over the ensuing 4 days. Contrast venography was performed with the intent to perform catheter-directed thrombolysis, mechanical thrombectomy, or both. Venography revealed patent right axillary and subclavian veins with no evidence of thrombus (Figure 2). Mature collateral venous communications, however, were found between the axillary and subclavian veins. An Adson maneuver performed during venography demonstrated dynamic obstruction of the axillary vein lateral to the thoracic inlet while flow was still visualized through the venous collateral network (Figure 3). The patient was discharged on oral anticoagulation therapy. Orthopedic opinion is pending for possible musculoskeletal pathogenesis.
Paget-Schroëtter syndrome (effort-induced axillary-subclavian vein thrombosis) typically presents after vigorous activity involving arm abduction, cervical extension, and shoulder depression, especially in the presence of a mechanical abnormality at the thoracic inlet where the vein is compressed between a hypertrophied anterior scalene muscle or subclavius tendon and the first rib. It is postulated that microtrauma to the venous vessel wall activates the coagulation cascade. Repetitive venous compression can result in fibrous tissue formation that permanently constricts the vein. The rationale in favor of expeditious catheter-directed thrombolysis as the adopted therapeutic strategy is that young healthy affected individuals are at high risk (35% to 85%) of developing postthrombotic syndrome if treated with anticoagulation alone.1 Such a chronic postphlebitic state is a result of venous hypertension from persistent outflow obstruction and valve destruction leading to venous incompetence. Surgical correction of mechanical thoracic outlet obstruction, such as resection of a cervical or the first rib, is often required to prevent recurrence. Catheter-directed chemical or mechanical thrombolysis was not required in our patient because of spontaneous lysis of the thrombus and restoration of antegrade venous flow on unfractionated heparin alone.