Disclosures: The authors report no conflicts of interest regarding the content herein.

The authors can be contacted via Dr. Sohail Khan at sohailkhan.md@gmail.com.

Thoracic outlet syndrome progressing to the point of axillosubclavian vein thrombosis, also referred to as Paget-Schroetter syndrome or effort thrombosis, is a rare entity that if treated correctly, has minimal long-term sequelae.^1-3 However, if this condition is not diagnosed and managed appropriately, it can lead to significant long-term morbidity, including pulmonary embolism and post thrombotic syndrome. The subclavian vein is highly vulnerable to injury as it passes by the junction of the first rib and clavicle in the anterior-most part of the thoracic outlet. Trauma or mechanical stress to the neck, shoulders, or upper extremities can lead to thoracic outlet syndrome by extrinsic compression, resulting in the development of  scar tissue around the vein.^4-6 Thoracic outlet syndrome is most often seen in patients who engage in repetitive motions that place the shoulder at the extreme of abduction and external rotation. We present an interesting case of venous thoracic outlet syndrome, 

where prompt clinical diagnosis and intervention lead to an excellent clinical outcome. 

Case 

A 44-year-old male with a past medical history of non-ischemic cardiomyopathy, hypertension, hyperlipidemia, chronic kidney disease, and obstructive sleep apnea, underwent implantable cardioverter defibrillator (ICD) implantation one year ago for primaryprevention. The patient’s professional work involved manual labor, moving heavy loads, and operating heavy machinery. The patient had a heavy muscular build. He presented to us with an acute onset (two days history) of left upper extremity pain, edema, and bluish discoloration. The arm was visibly swollen with mild cyanosis of his fingertips, intact pulses, and restricted flexion and extension of his arm and hands. The patient also had tingling in his left hand.

Initial ultrasound imaging showed acute venous thrombosis within the left axillary and left subclavian vein in its distal and mid aspect with patent arterial system (Figure 1). A chest x-ray showed the absence of cervical rib or ICD lead fracture (Figure 2). The ICD interrogation reported normal readings with the patient’s arm at rest and with anatomical manipulation.

The patient was taken to the cardiac catheterization lab. Using ultrasound guidance and micro puncture technique, access was achieved in the left basilic vein and a 6 French (Fr) sheath was inserted. The initial venogram showed significant acute occlusion in the left axillary and subclavian veins (Figure 3, Video 1).

The occlusion was crossed without difficulty using a 5Fr Glide catheter (Terumo) and soft Glidewire (Terumo). It was decided to perform catheter-directed thrombolysis. The EkoSonic Endovascular System (EKOS Corporation) catheter was introduced over the Glidewire and deployed across the acute thrombus in the left axillary and subclavian vein (Figure 4). Tissue plasminogen activator (tPA) at 1mg/hour was started and the patient was admitted to the intensive care unit for closer monitoring. The patient started to

 feel better, with improvement of pain and swelling in the left arm after few hours of treatment.

The patient was brought back to the catheterization lab after 16 hours of tPA treatment. The EkoSonic catheter was removed and the subsequent venogram showed minimal improvement in the venous flow. Aspiration thrombectomy was performed using theAngioJet thrombectomy system (Boston Scientific) without improvement in the flow on subsequent venogram. Balloon angioplasty of the left subclavian and left axillary vein was performed using a Charger 8mm x 40mm balloon (Boston Scientific) (Figure 5). Some resistance was felt at the level of the first rib while inflating the balloon. Again, there was no significant improvement in the flow (Video 2). At this time, we became highly suspicious that the patient was suffering from thoracic outlet syndrome, because of the partial failure of the thrombolysis therapy and the resistance felt while expanding the balloon right around the first rib. Though venous stenosis was on our differential list due to the presence of ICD leads, the patient’s effort-related thrombosis and his body habitus favored the diagnosis of venous thoracic outlet syndrome. There was also a component of neurological entrapment causing tingling and abnormal sensation in the fingertips (neurogenic thoracic

 outlet syndrome).

The case was discussed with the cardiothoracic surgeon as well as the electrophysiologist, and we decided to proceed with the thoracic outlet decompression/first rib resection. Intraoperative, the patient was found to have perivascular fibrous scar tissueformation around the left subclavian vein at the level of the first rib, most likely secondary to the chronic repeated trauma against the first rib. The patient underwent first rib resection as well as fibrous resection around the vein (circumferential venolysis). The patient recovered well, with complete resolution of his symptoms. He was discharged on coumadin for 6 months and was also enrolled in physical therapy to address postural abnormalities and muscle imbalance by relieving pressure on the thoracic outlet.

The patient was brought back to the catheterization lab for a venogram six months after his initial presentation. The venogram showed widely patent axillary and subclavian veins with brisk flow (Figure 6, Video 3).

Discussion

No prospective randomized trials concerning the treatment of effort thrombosis exist — most of the therapy is guided by retrospective reviews or expert opinions. The general consensus is if the patient has deep vein thrombosis of less than 14 days duration,catheter-directed thrombolysis followed by surgical compression is the preferred management, which was done in this case.^7,8 ICD lead-related subclavian vein thrombosis was also a possibility in this case, but the patient had the typical clinical presentation of effort-related acute thrombosis, manifested intraoperatively as fibrous scar tissue around the vein due to chronic repetitive trauma against the first rib. 

Early surgical decompression is imperative in the treatment of thoracic outlet syndrome. Angioplasty followed by stenting should be absolutely avoided. A follow-up venogram six months after the initial presentation demonstrated a patent subclavian vein, affirming thoracic outlet syndrome as the primary source of the patient’s symptoms. 

References 

1. Paget J. Clinical lectures and essays. London: Longmans, Green & Co, 1875.
2. von Schroetter L. Erkrankungen der gefasse. In: Nothnagel CWH, et al. Handbuch der Pathologie und Therapie. Wein: Holder, 1884. 
3. Hughes ES. Venous obstruction in upper extremity. Br J Surg. 1948; 36: 155-163.
4. Falconer MA, Weddell GL. Costoclavicular compression of subclavian artery and vein. Lancet. 1943; 242: 539.
5. Sampson JJ, Saunders JB, Capp CS. Compression of the subclavian vein by first rib and clavicle. Amer Heart J. 1940; 19: 292.
6. Adams JT, McEvoy RK, DeWeese JA. Primary deep venous thrombosis of upper extremity. AMA Arch Surg. 1965; 91: 29-42.
7. Doyle A, Wolford HY, Davies MG, Adams JT, Singh MJ, Saad WE, et al. Management of effort thrombosis of the subclavian vein: today’s treatment. Ann Vasc Surg. 2007; 21: 723-729.
8. Urschel HC Jr, Razzuk MA. Paget-Schroetter syndrome: what is the best management? Ann Thorac Surg. 2000; 69: 1663-1668; discussion 1668-1669.