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Paget-Schroetter syndrome.

CASE SUMMARY

A 34-year-old woman presented with a 1-day history of swelling and numbness in the right upper extremity, which she noticed after 2 days of heavy exercise. The patient, who was a smoker, had been on oral contraceptive patches for approximately 1 year. Venous ultrasound of the upper extremity showed an extensive thrombus involving the right subclavian, axillary, and basilic veins. She was started on intravenous anticoagulation but had only mild-to-moderate improvement with persistent swelling and discoloration of her right arm. Thrombolysis was subsequently performed, and much of the thrombus was disrupted. Balloon angioplasty was also performed to treat the residual mural thrombus and persistent stenosis in the axillary and subclavian veins. However, there was still an obstruction in the subclavian vein where the clavicle and first rib cross. Therefore, a stent was placed in the subclavian vein to maintain patency.

IMAGING FINDINGS

Two-dimensional and Doppler venous ultrasound of the right upper extremity showed an intraluminal filling defect within the right subclavian vein (Figure 1A) extending into the right axillary (Figure 1B) and basilic (Figure 1C) veins. These veins were noncompressible to external pressure, and no flow was visualized within these vessels on application of color Doppler.

A right upper extremity venogram showed complete thrombosis of the axillary and subclavian vein (Figure 2A). Following thrombolysis with Angiojet (Possis Medical, Inc., Minneapolis, MN) and tissue plasminogen activator (tPA), a 12-mm x 4-cm-long angioplasty balloon was used to treat the residual thrombus and stenosis (Figure 2B). Despite multiple attempts at angioplasty, large filling defects were still noted at the first rib/subclavian vein junction (Figure 2C), and, although initial improvement in flow was noted, there was complete reocclusion of this region with stasis of contrast after about 5 minutes. Therefore, the decision was made to place a stent in the region of the subclavian vein (Figure 2D) and excellent flow was noted through the system.

DIAGNOSIS

Paget-Schroetter syndrome

DISCUSSION

Axillary-subclavian vein thrombosis or "effort" throm bosis was initially described by Sir James Paget in 1875 and Von Schroetter in 1884. This clinical syndrome eventually became known as Paget-Schroetter syndrome. It is a rare disorder (2 per 100,000 patients per year) that occurs predominantly in young patients, especially males, following vigorous ex ertion or repetitive motion of the arms, producing compression of the vein. (1) Therefore, most patients are affected in their dominant arm.

The thrombosis is caused by trauma or repetitive muscular activity involving abduction or hyperabduction of the upper extremity. This position narrows the thoracic outlet and may also stretch the subclavian vein, causing intimal tears of the vein, which can stimulate the coagulation cascade. The mechanism of thrombus formation in these instances may also be caused by compression of the subclavian vein, which causes obstruction of flow. External compression of the vein may be caused by hypertrophy of the subclavian muscle tendon or anterior scalene muscle, cervical ribs, first rib, or callus of the clavicle from an old fracture. (2) Patients with hypercoagulable states are also at increased risk for Paget-Schroetter syndrome and may have more refractory thrombosis. (3)

Although most patients with PagetSchroetter syndrome have isolated vascular compression, some may also have symptoms of thoracic outlet obstruction such as paresthesias and muscle weakness. Signs of venous obstruction include pain, swelling, cyanosis, and venous collaterals. Patients often present with pain and swelling after strenuous activity, a cyanotic arm, dilated superficial veins, and tenderness to palpation over the deltopectoral groove. A cord may even be palpable in the axilla. (4)

Ultrasound is helpful as the initial imaging test, as it is noninvasive and has a high sensitivity and specificity for peripheral upper extremity venous thrombosis. However, false-negative studies can occur, as shadowing from the clavicle obscures a short segment of the subclavian vein. Although more invasive, venography is a more definitive study. It is also helpful for catheter-directed thrombolysis and angioplasty and for the assessment of response to treatment. Venography also allows for direct visualization of the clot and determination of the extent of the clot and collateral formation. (5) Magnetic resonance angiography is an accurate and noninvasive method to diagnose central venous thrombus, especially when venography is contraindicated or not possible. (6)

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Several treatment options have been described, and there has been some controversy as to which is the most appropriate. The conservative method includes heat, rest, and limb elevation, and may lead to symptomatic relief. However, many patients treated this way have chronic symptoms of aching and a sense of heaviness in the affected limb, muscle cramping, and even reocclusion. (7) Anticoagulation helps to maintain the patency of venous collaterals and reduces propagation of thrombus. (8) The use of thrombolytics, especially when combined with early surgical decompression, has been well documented to reduce the risk of long-term sequelae. Thrombolysis reduces damage to vascular endothelium, restores venous patency early, and reduces the risk of postthrombotic syndrome. Catheter-directed thrombolysis should be used within several weeks of the onset of symptoms, prior to thrombus organization. The catheter should be positioned as close to the thrombus as possible to prevent collateral flow of the thrombolytic away from the area. Percutaneous mechanical thrombectomy can be used in conjunction with thrombolytics and can reduce the dose and duration of thrombolytic therapy. To minimize the risk of recurrent thrombosis and long-term morbidity, surgical decompression is an important part of treatment in patients with venous compression. Such surgery usually involves resection of part of the first rib or clavicle. After surgery, venography can assess for residual stricture, which can be treated with balloon angioplasty or stenting. (9)

The most significant complications of upper extremity deep vein thrombosis (DVT) include pulmonary embolism and postthrombotic syndrome. Pulmo nary embolism can be seen in up to one third of all patients with upper extremity DVT. Postthrombotic syndrome is caused by venous hypertension from outflow obstruction and is characterized by limb swelling, pain, and possible ulceration. Other complications include superior vena cava syndrome, septic thrombophlebitis, brachial plexopathy, and thoracic duct obstruction. (9)

CONCLUSION

Paget-Schroetter syndrome is a rare disorder that may be difficult to diagnose and that typically affects young, healthy, active individuals. As the complications can be significant without prompt treatment, early detection and appropriate therapy are critical.

REFERENCES

(1.) Ellis MH, Manor Y, Witz M. Risk factors and management of patients with upper limb deep vein thrombosis. Chest. 2000;117:43-46.

(2.) Makhoul RG, Machleder HI. Developmental anomalies at the thoracic outlet: An analysis of 200 consecutive cases. J Vasc Surg. 1992;16:534-545; discussion 542-545.

(3.) Bliss S, Weinberger S, Meier M, Saint S. Clinical problem-solving. The unusual suspect. N Engl J Med. 2002;347:1876-1881.

(4.) Sheeran SR, Hallisey MJ, Murphy TP, et al. Local thrombolytic therapy as part of a multidisciplinary approach to acute axillosubclavian vein thrombosis (Paget-Schroetter Syndrome). J Vasc Interv Radiol. 1997;8:253-260.

(5.) Shah MK, Burke DT, Shah SH. Upper extremity deep vein thrombosis. South Med J. 2003;96:669-672.

(6.) Hartnell GG, Hughes LA, Finn JP, et al. Magnetic resonance angiography of the central chest veins. A new gold standard? Chest. 1995;107:1053-1057.

(7.) Medler R, McQueen DA. Effort thrombosis in a young wrestler. A case report. J Bone Joint Surg Am. 1993;75:1071-1073.

(8.) Horattas MC, Wright DJ, Fenton AH, et al. Changing concepts of deep venous thrombosis of the upper extremity: Report of a series and review of the literature. Surgery. 1988;104:561-567.

(9.) Joffe HV, Goldhaber SZ. Upper extremity deep vein thrombosis. Circulation. 2002;106:1874-1880. Prepared by Michelle Udeshi, MD, and Thomas D. Olsavsky, MD, St. Vincent's Medical Center, Department of Radiology, Bridgeport, CT.

Michelle Udeshi, MD, and Thomas D. Olsavsky, MD
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Title Annotation:RADIOLOGICAL CASE
Author:Udeshi, Michelle; Olsavsky, Thomas D.
Publication:Applied Radiology
Article Type:Case study
Geographic Code:1USA
Date:May 1, 2008
Words:1276
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