Acute Occlusion of Right Axillary to Left Jugular Collateral Vein Secondary to Paget-Schroetter Syndrome

A patient with the rare Paget-Schroetter syndrome presents a diagnostic and treatment challenge to the physician. Accounting for only 1 to 2% of all venous thrombosis cases, Paget-Schroetter syndrome has been described in multiple case reports, but optimal treatment has not been studied in prospective, randomized, controlled trials.¹ Therefore, current treatment is based largely on retrospective studies and case reports, is hardly clear cut, and remains controversial.^2,3 

 

The hallmark of Paget-Schroetter syndrome is the thrombosis of the subclavian vein at the costo-clavicular junction produced by direct damage to the endothelium of the vein by muscular strain, stretching, and/or compression. The condition typically presents as upper extremity deep venous thrombosis (DVT) in individuals who perform strenuous and repetitive tasks using their arms, such as the throwing motion of a baseball pitcher. We describe the successful endovascular treatment of a patient with a history of Paget-Schroetter syndrome who had a recurrent right upper extremity DVT due to acute thrombosis of the large right axillary to left jugular collateral vein. 

 

 

The patient is a 26-year-old male of muscular build. He works as a mechanic where he uses his dominant right arm to repetitively swing a hammer. He has a history of DVT 14 months prior, where he presented to an out-of-state hospital and was diagnosed with Paget-Schroetter syndrome. There he was treated with thrombolytics and subsequently placed on oral anticoagulation with rivaroxaban. The patient discontinued the rivaroxaban after a few months because of socioeconomic circumstances.

 

Several months after discontinuing anticoagulation therapy, he presented to his primary care physician with symptoms of right upper extremity swelling and pain that had persisted for almost one week. The venous Doppler study demonstrated acute thrombosis of the right subclavian, cephalic, axillary, and basilic veins. He was restarted on rivaroxaban; however, his symptoms persisted. Subsequently, he was referred to us for possible endovascular treatment. 

 

After a detailed discussion with the patient, it was decided to proceed with invasive venography and possible thrombolysis. A midline was placed in the right basilic vein under ultrasound guidance prior to arrival to the cath lab, which was then exchanged for 6 French Terumo sheath. Initial venography demonstrated thrombosis of the basilic vein and total occlusion of the subclavian vein with extensive collateral formation (Figure 1). Multiple attempts to cross the .035-inch wire to the right brachiocephalic vein failed, feeling like a chronic total occlusion (CTO). Switching strategies, the right common femoral vein was then accessed and a 6 French Terumo sheath was placed. Superior vena cava venography confirmed a CTO of the right subclavian vein (Figure 2). We attempted to cross the subclavian vein through the common femoral venous route, but we remained unsuccessful. 

 

Since we could not cross the CTO, and the patient was assessed to be at low risk for bleeding complications, we proceeded with endovascular thrombolysis in the visualized portion of the axillary vein to treat the extensive thrombosis. A 20 cm EkoSonic catheter (EKOS Corporation) was advanced through the right basilic vein up to the occluded segment of the subclavian vein (Figure 3). A 2 mg bolus of tissue plasminogen activator (tPA) was delivered through the catheter and an infusion of 1 mg/hour was initiated. The patient was then transferred to the intensive care unit. 

 

After the patient received 20 hours of ultrasonic-infusion therapy, he was brought back to the cath lab and the EKOS catheter was removed. Venography revealed complete thrombus resolution of the axillary vein while the subclavian vein remained occluded. The venogram also demonstrated a tortuous collateral of large caliber arising from the right axillary vein to the left jugular vein crossing the supraclavicular space and subsequently draining into the superior vena cava (Figure 4).

 

There was a significant stenosis at the intersection of the clavicle and second rib, the culprit that caused the thrombosis and subsequent closure of the collateral vein. An .035-inch Glidewire Advantage wire (Terumo) was advanced through the collateral. Venoplasty of the stenosis with a 9 x 15 mm Powerflex balloon (Cordis, a Cardinal Health company) followed by a 10 x 15 mm Powerflex balloon was performed. The final venogram demonstrated resolution of the stenosis (Figure 5). Stenting was deferred, as this segment was believed to be located at a compression site, and would likely lead to stent fracture and re-thrombosis. 

 

The patient’s symptoms were completely relieved after intervention. He was placed back on rivaroxaban and referred for surgical decompression therapy (SDT), which he ultimately refused. At his one-year follow-up, the patient remains asymptomatic and is compliant with oral anticoagulation therapy.

 

 

The uniqueness of this case is that the patient presented with upper extremity DVT, consistent with Paget-Schroetter syndrome; however, this recurrent episode was due to acute thrombosis of a large collateral, while the subclavian vein remained a CTO. While he was on oral anticoagulation therapy following his first DVT, it is presumed his subclavian vein re-thrombosed and formed a CTO. However, it is possible that he did not become symptomatic due to the extensive formation of collateral veins. Once he stopped the oral anticoagulation therapy, consequently, the stenotic axillo-jugular collateral vein developed an acute thrombosis and produced symptoms. 

 

In our case, catheter-directed therapy utilizing EKOS was safe and effective in resolving the acute thrombosis in this large collateral vein. Achieving the best outcomes in the treatment of Paget-Schoetter’s syndrome typically requires SDT to relieve the musculoskeletal compression of the vein after treating the acute thrombosis.³ Even though the subclavian vein remained a CTO, EKOS therapy was effective in opening the large collateral vein. At this point, we were able to visualize the venous stenosis and successfully treat it with balloon venoplasty. Venoplasty has shown to be an effective therapy, as demonstrated in a small study by Kreienberg et al that reported 100% patency rates at 9 years.⁵ 

 

A small, prospective study by Grommes et al demonstrated ultrasound-accelerated catheter-directed therapy as a safe and effective treatment for DVT of the lower extremities.4 With catheter-directed therapy as the initial treatment of acute thrombosis, and taking into consideration the patient’s continued debilitating symptoms, low risk for bleeding complications, and failed conservative medical therapy, we decided that EKOS therapy would be a good treatment option for this patient. EKOS ultrasound-accelerated catheter-directed therapy, as demonstrated in this case study, is an effective treatment option for upper extremity DVT and symptom relief.

 

 

Dr. Joji Varghese can be contacted at jvarghese@hendrickhealth.org. Bailey Estes can be contacted at bestes@hendrickhealth.org.