Adult Chest Surgery

Chapter 125. Thrombosis of the Subclavian Vein: Paget-Schroetter Syndrome

Paget-Schroetter syndrome is the sudden thrombosis of the subclavian vein in the setting of physical exertion of the arm. This syndrome is also known as effort thrombosis. It is the most extreme presentation of the venous form of thoracic outlet syndrome. It is a rare disease of active young people engaged in physical exertion of the arm (e.g., mechanics, rock climbers, swimmers, and weight lifters). Specifically, it is the forceful movement of pulling the extended arm from over the head down toward the torso that leads to the underlying basis of the disease: hypertrophy of the subclavius and anterior scalene muscles.

The subclavius is a small triangular muscle that originates from a groove on the undersurface of the clavicle and inserts by a short, thick tendon into the junction of the bony and cartilaginous first rib (Fig. 125-1). The subclavius muscle depresses the shoulder by drawing the clavicle downward and forward. The anterior scalene muscle arises from the transverse processes of the third to sixth cervical vertebrae and inserts onto the scalene tubercle on top of the first rib, separating the subclavian artery and vein. When an arm extended over the head is pulled down vigorously toward the torso, the anterior scalene fixes the first rib into position, anchoring it against the transverse processes of the neck. The subclavius muscle, in turn, anchors on the fixed first rib and pulls the shoulder down by displacing the clavicle.1

Figure 125-1.

 
 

A. The subclavian muscle originates from the undersurface of the clavicle and inserts into the junction of the bony and cartilaginous first rib. It lies in close proximity to the subclavian vein. B. Chronic forceful downward arm movements, such as, for example, when pitching a ball, creates the compressive pathology associated with Paget-Schroetter syndrome.

The sudden and forceful pulling down of the arm toward the torso is accomplished by the powerful contraction of the anterior scalene and subclavius muscles. The hypertrophied subclavius muscle in particular appears to be the cause of the pathologic injury. The sudden and powerful compression of the subclavian vein between these two muscles is sufficient to tear the intima of the vein, and thrombosis occurs. The clot then propagates distally toward the shoulder. Thrombolysis or anticoagulation alone may be insufficient to correct the clinical manifestations or to prevent recurrence. First rib resection with division of the two muscles prevents recurrence. The exposure given by an axillary approach to divide these muscles is inadequate and therefore not recommended. A subclavicular approach not only allows division of these muscles but also permits patch angioplasty of the vein.

DIAGNOSIS AND WORKUP

Paget-Schroetter syndrome is a clinical diagnosis confirmed by duplex ultrasound or venogram. The ipsilateral arm frequently is swollen with distended veins up to the shoulder. Range of motion of the arm is impaired as a result of pain. If not treated immediately, the patient may be left with a chronic debilitating condition that limits use of the arm. Lysis of the clot alone or anticoagulation alone is inadequate therapy. The surgeon must be prepared to divide the compressive muscles and to perform a patch angioplasty of the vein. We describe the operative approaches to affect this type of repair.

Paget-Schroetter syndrome is rare, and the average surgeon may see only a few cases across a career. J. Ernesto Molina is a cardiothoracic surgeon at the University of Minnesota who developed an interest in treatment protocols for this disease. He has educated a generation of surgeons from the University of Minnesota program, and this chapter is indebted to his work.2–5

The diagnosis of Paget-Schroetter syndrome is based on history, physical examination, and Doppler assessment of the ipsilateral axillary and subclavian veins. The history is one of recent effort of the ipsilateral arm and sudden pain. The physical examination generally reveals a swollen and functionally impaired forearm and hand. Although distention of arm veins is seen commonly, development of venous collaterals around the shoulder is a late finding and suggestive of a chronic condition. Doppler ultrasound shows venous webbing or occlusion and also can be used to rule out a subclavian artery thrombosis or aneurysm.

At the University of Minnesota in the mid-1990s, the diagnosis would be followed by the placement of a venous catheter, which would be advanced into the clot. The catheter provided access for a venogram to assess the length of the clot, as well as for local delivery of fibrinolytics. Although success was achieved with urokinase in the 1980s, this now has been supplanted by recombinant tissue plasminogen activator (Activase, alteplase, TNK-t-PA).

SURGICAL THERAPY

Acute Paget-Schroetter syndrome is approached surgically by an infraclavicular incision (Fig. 125-2). This approach allows division of the two causative muscles (anterior scalene and subclavius) and permits proximal control of the subclavian vein for patch angioplasty.

Figure 125-2.

 

Access is made through a transverse infraclavicular skin incision.

 

The patient is positioned supine with the ipsilateral arm placed on an armboard at an angle of about 60 degrees from the torso. The clavicle runs lateral to the sternum for the first third of its length, angles posteriorly for the second third, and curves back to a transverse course for the lateral third. The transverse infraclavicular incision starts at the edge of the transition between the medial and middle thirds of the clavicle. It is extended over the deltopectoral groove of the pectoralis major muscle about 2 cm inferior to the midsection of the clavicle. The fibers of the pectoralis major muscle are divided in the direction of their course, and the first rib is identified (Fig. 125-3).

Figure 125-3.

 

The pectoralis major muscle fibers are separated, exposing the first rib.

 

The subclavius muscle is identified in the medial aspect of the incision, inserting onto the top surface of the most medial extent of the first rib and passing to insert posteriorly onto the median third of the clavicle (Fig. 125-4). It is frequently hypertrophied in patients with Paget-Schroetter syndrome. The subclavius muscle is divided off its insertion into the top of the first rib, reflected laterally, and then excised. A web of the subclavian vein is frequently observed beneath the subclavius muscle.

Figure 125-4.

 

The subclavian muscle is identified, divided off its insertion into the top surface of the medial extent of the first rib, and excised. The costoclavicular ligament is divided.

 

The intercostal attachments to the undersurface of the first rib are divided from as far medial to as far lateral as possible, often extending beneath the subclavian vessels and brachial plexus (Fig. 125-5). The pleura is bluntly pushed away from the undersurface of the first rib. The medial end of the first rib is divided as close to the sternum as possible, taking care not to injure the internal mammary vessels.

Figure 125-5.

 

The subclavian artery is retracted to reveal the intercostal muscle, which is divided from the undersurface of the first rib.

 

The first rib then is displaced caudally with downward traction on the broad surface of the rib (Fig. 125-6). This stretches the anterior scalene muscle into view posterior to the subclavian vein. This muscle is divided from its attachment into the scalene tubercle of the midsection of the first rib. The rib then is displaced caudally even more readily. A rib cutter is used to divide the rib posteriorly at the level of the subclavian artery (Fig. 125-7).

Figure 125-6.

 

The first rib is depressed to expose and divide the anterior scalene muscle, which is attached to the scalene tubercle of the first rib.

 

Figure 125-7.

 

The rib is divided by using the right-angled rib cutter.

To implant a patch on the vein, vascular clamps are used to obtain both proximal and distal control. The web is incised longitudinally, and a diamond-shaped vein patch is used to enlarge the area of stenosis. Saphenous vein harvested from the upper thigh is used for this patch. If a long segment of the vein is stenosed, a long vein patch is constructed using the proximal saphenous vein with its larger diameter.

POSTOPERATIVE CARE

Postoperative anticoagulation is implemented for a total of 8 weeks with warfarin (Coumadin) and clopidogrel (Plavix). Although dextran and low-molecular-weight heparin are used in the early postoperative period, it is discontinued as the international normalization ratio level reaches the therapeutic range, which is optimally maintained between 2 and 3. To ensure the effectiveness of the operation, a duplex ultrasound examination is obtained the next day. If significant stenosis persists, placement of an endovascular stent may be necessary.

SUMMARY

Paget-Schroetter syndrome is an acute thrombosis of the subclavian vein often related to exertional physical activity of the arm. It affects mostly young, healthy people and requires emergency care. The standard treatment entails immediate thrombolytic therapy by catheter-directed infusion followed by surgery. The operation involves decompression of the thoracic inlet and reestablishment of the normal caliber and flow of the vein. By following this approach, one can prevent the chronic sequelae of total arm vein obstruction and the consequent permanent disability of the patient.

EDITOR'S COMMENT

Despite advancements in the field of interventional radiology and the development of stents, thrombolysis followed by prompt surgical repair remains the treatment of choice for Paget-Schroetter syndrome.

–LZ

REFERENCES

1. Gray H: Anatomy, Descriptive and Surgical, 15th ed. New York, Bounty Books, 1977.

2. Molina JE: Surgery for effort thrombosis of the subclavian vein. J Thorac Cardiovasc Surg 103:341–6, 1992.[PubMed: 1735999]

3. Molina JE: Operative technique of first rib resection via subclavicular approach. Vasc Surg 27:667–72, 1993. 

4. Molina JE: Approach to the confluence of the subclavian and internal jugular veins without claviculectomy. Semin Vasc Surg 13:10–9, 2000.[PubMed: 10743884]

5. Molina JE, Hunter DW, Dietz CA: Paget-Schroetter syndrome treated with thrombolytics and immediate surgery. J Vasc Surg 45:328–34, 2007.[PubMed: 17264012]



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