Synonyms: Paget-von Schrötter disease, axillary subclavian deep vein thrombosis (ASDVT), upper-extremity deep vein thrombosis, upper-limb deep vein thrombosis, effort-induced thrombosis

Definition

Acute swelling and pain in the upper limb due to occlusion of the axillary and/or subclavian veins by thrombus. This may occur as a a primary phenomenon or as a result of the placement of an indwelling venous catheter, thrombophilia or thoracic outlet syndrome.

Epidemiology

There is a low incidence of about 2 per 100,000 people per year.¹ Historically, axillary vein thrombosis only accounted for around 1-2% of all cases of deep vein thrombosis (DVT), but is now more common due to the growing use of central venous cannulation in a variety of medical procedures. It occurs in about 25% of patients who undergo prolonged central venous cannulation, although it is often not recognised. About 80% of primary cases occur in the dominant arm. It is also seen in young, otherwise healthy, individuals who participate in repetitive upper limb exercises.¹ One study reported that patients who had an upper limb DVT were more likely to be younger and with a lower BMI.²

Risk factors

• Vigorous and prolonged use of the upper limb in sporting/other activity (primary 'effort-induced' form)
• Upper limb trauma
• Central venous catheter, e.g. internal jugular vein, subclavian vein (probably the strongest risk factor)²
• Recent hospitalisation
• Severe infection
• Neoplasia
• Thrombophilia (a possible further increased risk if the patient has thrombophilia and is on the oral contraceptive pill)
• Anatomical abnormality of thoracic outlet
• Intravenous recreational drug use

Presentation

Symptoms

• Patients tend to present promptly due to the acute discomfort and swelling
• Pain in the axilla/around the shoulder, often aching and indistinct
• Swelling of the arm and hand
• Discolouration of the hand
• 7-20% of cases may lead to pulmonary embolism (PE) with features of pleuritic chest pain, breathlessness and haemoptysis¹

Signs

• Oedema of the arm and hand - measure the biceps/forearm diameter at a fixed distance from an anatomical landmark
• Mild-to-moderate cyanosis of the hand
• Dilated superficial collateral veins may be seen over the chest and upper arm - may be the only indicator in central venous cannulation
• Fullness in the supraclavicular fossa and even a palpable cord of thrombosed vein
• Jugular vein may be distended

Differential diagnosis³

• Superficial phlebitis
• Cellulitis
• Severe superficial bruising
• Muscular tear
• Intramuscular haemorrhage
• Lymphoedema
• Occult fracture
• Superior vena caval obstruction
• Lymphangitis
• Localised allergy
• Gas gangrene

Investigations

• Duplex ultrasonography is sensitive and specific.
• Other imaging options include angiography or MRI angiography.
• It is uncertain whether routine thrombophilic screening in patients with this condition is worthwhile. It is probably useful where it occurs idiopathically, with a family history of thrombosis or history of recurrent miscarriage or previous DVT.
• Imaging investigations to detect thoracic outlet syndrome should depend on the degree of clinical suspicion of this cause.
• In idiopathic cases one should consider investigations to look for an occult malignancy or thrombophilia.

Management

• Anticoagulation with low-molecular-weight heparin and warfarin as per lower limb DVT is the mainstay of management.⁴
• The limb should be kept elevated and warm.
• Sufficient analgesia should be prescribed.
• Catheter-directed thrombolysis has been used and minimises damage to the vessel.¹
• Percutaneous thrombectomy in combination with local thrombolysis may also be used.¹
• Early thrombolysis may reduce the incidence of long-term complications but this has not been proven in the total population of sufferers of the condition.
• Surgical thrombectomy may be needed in some cases.
• However, despite these options, the use of low-molecular-weight heparin and warfarin is also the mainstay of treatment in catheter-related DVT - this can allow the central venous catheter to remain patent and in situ.⁵

Prevention

The use of anticoagulant prophylaxis in patients who are acutely ill and those who undergo central venous catheterisation may prevent upper extremity DVT. However, the efficacy of anticoagulant prophylaxis has not been clearly evaluated at present.²

Prognosis

• About 7-20% develop pulmonary embolism (PE) and 1% die.
• Recurrent thrombosis affects between 2 and 10% of patients.
• Post-thrombotic syndrome affects around 25% (see below).⁶

Complications

• Pulmonary embolism (PE) has been detected on radiological grounds in up to 20% of patients with upper limb DVT (incidence is highest in untreated/catheter cases)
• Phlegmasia caerulea dolens (PCD) may occur rarely; there is arterial and venous compromise and a risk of gangrene
• Compartment syndrome
• Recurrent thrombosis
• Post-thrombotic syndrome - chronic upper limb pain and swelling
• Stroke following paradoxical embolisation in cases with a patent foramen ovale
• Right ventricular failure
• Thoracic duct obstruction
• Chylous pleural or pericardial effusion

Document references

1. Roche-Nagle G, Ryan R, Barry M, et al; Effort thrombosis of the upper extremity in a young sportsman: Paget-Schroetter syndrome. Br J Sports Med. 2007 Aug;41(8):540-1; discussion 541. Epub 2007 Feb 8. [abstract]
2. Spencer FA, Emery C, Lessard D, et al; Upper extremity deep vein thrombosis: a community-based perspective. Am J Med. 2007 Aug;120(8):678-84. [abstract]
3. Amir-Us-Saqlain H, Javaid A, Hashmi I, et al; Upper extremity deep vein thrombosis. J Coll Physicians Surg Pak. 2005 May;15(5):309-10. [abstract]
4. Spiezia L, Simioni P; Upper extremity deep vein thrombosis. Intern Emerg Med. 2009 Sep 26. [abstract]
5. Kovacs MJ, Kahn SR, Rodger M, et al; A pilot study of central venous catheter survival in cancer patients using low-molecular-weight heparin (dalteparin) and warfarin without catheter removal for the treatment of upper extremity deep vein thrombosis (The Catheter Study). J Thromb Haemost. 2007 Aug;5(8):1650-3. Epub 2007 May 7. [abstract]
6. Prandoni P, Bernardi E, Marchiori A, et al; The long term clinical course of acute deep vein thrombosis of the arm: prospective cohort study. BMJ. 2004 Aug 28;329(7464):484-5. Epub 2004 Jul 15.