At the Clinic
The Cause of a Swollen Arm: Paget-Schrötter syndrome
Nozar Amiri, M.D.
Department of Family Medicine
University of California School of Medicine
Mr. Robinson, was a 31-year-old man who presented with a a one-week history of heaviness and swelling in his right arm. He had been seen at the ER a few days before and had been given antibiotics and NSAIDs, which helped decrease the pain. He is right-handed. He is a smoker and stated that had had some weight loss and sweating at night. He is a manual worker, involved in lifting and carrying. There was no significant family history.
On examination, the patient had dilated superficial veins across his right shoulder and biceps area and the entire right upper limb was bigger than the left. There was also a color difference - the right hand and arm duskier than the left.
Blood tests including inflammatory markers and a plain chest X-ray were normal. An ultrasound scan of his upper limb confirmed an established subclavian vein thrombosis.
Considering the unprovoked appearance of the thrombosis and the nature of his job, Paget-Schrötter syndrome was suspected and he was referred to a vascular surgeon. A subsequent thrombophilia screen* was negative, and MRI of the thoracic outlet and brachial plexus did not reveal any cervical rib or fibrous band to cause a constriction of the subclavian vein, which may have provoked the thrombosis. He was started on warfarin and the diagnosis was confirmed as Paget-Schrötter syndrome.
Venous thoracic outlet syndrome progressing to the point of axillary-subclavian vein thrombosis (ASVT), is called Paget-Schrötter syndrome or effort thrombosis which refers to primary thrombosis of the axillary and/or subclavian vein. Paget-Schrötter syndrome is an important cause of upper extremity deep vein thrombosis. It can be thought of as a venous equivalent of thoracic outlet syndrome. Paget-Schrötter syndrome is associated with strenuous and repetitive actions of the upper limbs especially forced abduction of the upper limb.(2) It is most commonly seen in young athletes such as those involved in weight-lifting, baseball pitching, softball, wrestling, swimming, hockey, tennis, martial arts, backpacking, and billiards who use repetitive shoulder-arm motions.(3,7,8,9,10,11,12)Venous compression syndromes are not commonplace in the general population and occur due to the entrapment of vein(s) in confined anatomical spaces surrounded by bony and non-bony structures.
The axillary subclavian vein passes through the tunnel formed by the clavicle and subclavius muscle anteriorly, the scalenus anticus muscle laterally, the first rib posterior-inferiorly, and the costoclavicular ligament medially. Therefore, abnormalities of any of these structures—whether congenital, traumatic, or distorted by unusual exercise or physical stress—may narrow the tunnel anatomically.
As the subclavian vein passes by the junction of the first rib and clavicle in the anterior-most part of the thoracic outlet, it is highly vulnerable to injury. In addition to compression from outside, repetitive forces in this area frequently cause fixed damage inside the vein and extrinsic scar tissue formation.
The first detailed case of this condition was described by James Paget in 1875 (6) and in 1894, von Schrötter was the first to identify vascular trauma from muscle strain as a potential etiologic factor. In 1948, Hughes coined the term Paget-Schrötter Syndrome (PSS) and published the first review.(1)
SIGNS and SYMPTOMS
Although this syndrome has always been associated with fit and active healthy young men, especially sportsmen, but its incidence in young women has increased. Patients with effort thrombosis usually present after the subclavian vein has completely thrombosed with arm heaviness, pain and swelling, more common in the dominant limb, asymmetric muscle bulk or increased postworkout swelling that does not dissipate, a bluish hue to the arm, discoloration, loss of velocity or control, arm heaviness, or a “dead arm”. Symptoms mostly occur within 24 hours of the heavy upper extremity activity, as superficial veins become prominent in the upper extremity and the neck. Our patient demonstrated Urschel's sign, or the appearance of dilated visible veins across the upper limb.
Differential diagnosis includes neurologic thoracic outlet syndrome, arterial thoracic outlet syndrome, malignant tumors of the head, neck, or arm, pancoast tumor, complex regional pain syndrome, brachial neuritis, cervical nerve root compression, quadrilateral space syndrome, and peripheral nerve compression
Doppler ultrasonography is usually sufficient to make the diagnosis. Ultrasound or venography can confirm the presence of thrombus. CT or MRI of the thoracic inlet is used to detect the cause of the extrinsic compression.
Venous contrast study showing thrombosis and flow obstruction at the thoracic outlet.
Complications of Paget-Schrötter syndrome include pulmonary embolism (4) and post-thrombotic syndrome which develops from thrombosis induced venous valvular injury leading to valvular incompetence. It can result in chronic venous insufficiency, pain, edema, and hyperpigmentation. Approximately 10 of patients with PS syndrome develop pulmonary embolus.
Nonsurgical treatment using anticoagulation only, can cause acute pulmonary embolism in 6% to 15%, and residual venous obstruction in over 75% of patients.(14) Outcomes are disappointing with the use of anticoagulation treatment as a single therapy. Symptoms persist in up to 91% of patients and up to 68% will experience permanent disability.(14,15,16,17,18)
Currently, catheter thrombolysis with subsequent first rib resection has become the treatment of choice for Paget-Schrötter syndrome.(19) Recanalization of the occluded vein, however, depends on many factors, an imprtant one of which being the time from clot formation to treatment.(15) After 10 days, the success rate of opening the vein drops to almost zero.(15, 20, 21) Approximately 50% of veins treated at 6 weeks were partially opened, and none were completely opened.(22) The size of the clot with longer thrombi is a poor prognostic indicator correlating with poor success at recanalization.(23)
Due to the poor long-term patency rate, stenting must be avoided if possible and must only be performed after surgical decompression. Otherwise, the stent may be crushed or fractured. The success of thrombolysis decreases from the time of the initial onset of symptoms.Paget-Schrötter syndrome is a good example of a condition which if treated correctly has minimal long-term sequelae but if ignored may cause significant long-term morbidity. Catheter-directed thrombolytic therapy is mostly successful if initiated within ten to 14 days of clot formation, but often unmasks an underlying lesion.
It is generally believed that decompression of the venous thoracic outlet, often by first rib excision, partial anterior scalenectomy, resection of the costoclavicular ligament, and complete external venolysis, must be done. Long-term success rates of 95 to 100% have been reported by many investigators when these guidelines have been followed.
Conflict of Interest: None.
Authorship: Author is entirely responsible for the content of this manuscript.
Requests for reprint should be addressed to Nozar Amiri, M.D., University of California School of Medicine, 900 University Ave., Riverside, CA 92507 Email: firstname.lastname@example.org
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* Thrombophilia screen tests:
- Factor V Leiden mutation
- Prothombin gene mutation
- Hyperhomocysteinaemia (fasting)
- Antiphospholipid antibody syndrome (lupus anticoagulant and anticardioplipin antibodies)
- Deficiency of antithrombin III, protein C or protein S
- High concentrations of factor VIII or XI
- Increased lipoprotein (a)