The correct answer is: D. Consult vascular specialists for catheter-based venography, catheter-based thrombolysis, and thoracic outlet decompression.

This patient has developed an axillary-subclavian vein thrombosis (ASVT) (Figure 1) and should undergo a venogram with catheter-directed thrombolytic therapy.

Figure 1

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Figure 1

Also known as Effort Thrombosis or Paget-Schroetter Syndrome, ASVT is a rare entity that typically affects young, healthy individuals. Primarily affecting the subclavian vein at the costoclavicular junction, it preferentially involves the dominant arm.^1-3

The pathophysiology is thought to be related to repetitive trauma of the upper extremity in the setting of an underlying anatomical abnormality at the thoracic outlet.³ As such, ASVT is thought to be a manifestation of venous thoracic outlet syndrome (VTOS), specifically related to a restricted anterior portion of the thoracic outlet region.^2,3 Wrestling, basketball, gymnastics, and swimming have all been described as activities with repetitive upper extremity movements which can lead to ASVT,^1,4 though non-athletes with frequent overhead activities such as painting and auto mechanics can also develop ASVT.³

In patients with VTOS, progressive fibrosis and focal stenosis can occur when the subclavian vein is repetitively compressed between the first rib, clavicle, anterior scalene muscle, subclavius muscle, and the costoclavicular ligament.^2,3 Compression of the vein leads to stasis and restricted mobility of the subclavian vein, leading to a cycle of endothelial trauma, thrombosis and recanalization with subsequent intimal hyperplasia, inflammation, and fibrosis.^1,2,5,6

Classically, individuals with ASVT present with sudden onset of arm discomfort and swelling, though they can also present with a constellation of symptoms known as Urschel's sign, which includes arm heaviness, erythema, and cyanosis of the affected upper extremity.³ If the occlusion is chronic, dilated visible superficial veins across the upper arm, chest wall, and shoulder may develop.^3,7,8

Initial evaluation involves Doppler ultrasonography, but is limited by the position of the clavicle, depth of the vein, expanded collateral veins which may be mistaken for the subclavian vein, and is highly dependent on a skilled sonographer.³ Increasingly, computed tomography and magnetic resonance imaging venography are being utilized to identify and characterize ASVTs, although they should be protocoled appropriately to image the venous phase.^5,9 Contrast venography is considered the gold standard, as it provides higher spatial and temporal resolution of collateral venous anatomy as well as the extent of thrombosis.^1,5,9

The management of ASVT has evolved over time, noting that leaving an ASVT untreated results in significant morbidity in most patients.³ Treatment with anticoagulation alone is also insufficient, leading to residual disability in the form of persistent symptoms due to chronic venous obstruction or even the development of acute pulmonary emboli,^3,10-13 and is therefore not recommended.^1,3,14 The mainstay of management is catheter-directed thrombolysis with subsequent thoracic outlet decompression.¹ The earlier catheter-directed thrombolysis is performed, the more likely the procedure is to be successful, nearing 100% if initiated within a few days (and less than ten days) of symptom onset.³ Without subsequent thoracic outlet decompression, recurrent ASVT occurs in up to a third of patients,^3,14 though there is significant controversy regarding the need for decompression. Decompression most commonly involves resection of the first rib with good long-term outcomes.^14,15 A majority of experts agree that decompression is necessary to prevent recurrence and that early decompression has been shown to be effective and safe.^3,14-17

Our patient with an ASVT had successful recanalization of his axillary and subclavian veins by catheter-directed thrombolytic therapy. Two weeks after his hospitalization, he underwent successful first right rib resection with anterior scalenectomy and venolysis. Five weeks after his presentation, he is doing well with complete resolution of his right upper extremity swelling and discomfort. A duplex at that time revealed laminar flow in the right axillary vein without evidence of thrombus (Figure 2). He has resumed more vigorous activities, but has not yet returned to rock climbing. Preventative contralateral evaluation and intervention was discussed, but not pursued due to lack of evidence.

Figure 2

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Figure 2

References

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