56: PAGET-SCHROETTER SYNDROME

PATIENT STORY

A previously healthy 22-year-old man presents with a 3-day history of right upper extremity swelling and pain. He is a competitive cross fit athlete and performs vigorous upper body weight lifting several days per week. On examination the right forearm and brachium are diffusely swollen with an overlying erythrocyanotic appearance. A venous duplex ultrasound identifies acute thrombosis within the axillary and subclavian veins. In preparation for catheter-directed thrombolysis, a right upper extremity venogram is performed. Figures 56-1A and B venographically illustrate diffuse intraluminal filling defects consistent with acute brachial and axillosubclavian deep vein thrombosis (DVT).

EPIDEMIOLOGY

• Primary effort-related thrombosis of the subclavian vein caused by compression within the thoracic outlet (Figure 56-2A).

• Provocative arm positioning includes repetitive hyperabduction and external rotation of the upper extremity or posterior and inferior shoulder rotation.

• Absence of other causes, such as extrinsic mass or indwelling catheter.

• Male-to-female ratio is approximately 2:1.¹

• Mostly in third and fourth decades of life.^1,2

• Young, healthy athletes after vigorous upper extremity exercise and in patients who do frequent overhead maneuvers (painters, construction workers, wood choppers, auto repair workers).¹

• Involves the dominant arm in 80% of cases.

ANATOMY AND PATHOPHYSIOLOGY

• The subclavian vein passes through the thoracic outlet bounded by the clavicle and subclavius muscle anteriorly, the anterior scalene muscle laterally, the first rib posterior-inferiorly, and the costoclavicular ligament medially (Figure 56-2B).

• Extrinsic compression of the subclavian vein by the first rib and the clavicle.^{1,2, and 3}

• Hypertrophy of the anterior scalene muscle and/or the subclavius muscle can decrease the size of the outlet and compress the subclavian vein.¹

• Multifactorial pathophysiology that fulfills the Virchow triad including (1) anatomic changes listed above leading to stasis; (2) hypercoagulability in association with exercise-associated stress; (3) intimal tears within the vein wall in association with repetitive shoulder-arm movement.

DIAGNOSIS

Clinical Features

• Young patient presenting with blue, heavy, swollen, painful arm.¹

• History of vigorous exercise or activity with the affected extremity, usually within the previous 24 hours.^1,2

• Dull aching pain within the affected arm and shoulder that is aggravated ...