A 29-year-old woman presented with recent onset, right arm swelling and pain. She gave a history of several IV infusions in that arm in late pregnancy. There was no chest pain. Nine days previously, she had delivered her second child.
On examination, she looked unwell, with a bilateral pedal oedema. Her temperature was 36.8C, with a BP of 135/97mmHg, 141/97mmHg, and no tachycardia. Her BMI was 37.
She had a previous history of gall stones and anaphylactic shock from latex allergy.
She had never taken the combined oral contraceptive pill.
The right (dominant) arm was warm, swollen and tender, but not red, and her breasts were red and oedematous. I recommended that she should go to hospital for assessment. She was assessed in her local maternity day unit that day, but discharged after a normal set of pre-eclampsia bloods.
Failing to improve, she was reviewed by her GP 10 days later. An ultrasound was requested after it was noted that her bloods were normal. The CRP was 3, WCC 6.7 (normal range 4-11x109/L), and platelets 374 (normal range 150-400x109/L).
Within the next few days, she presented four times to A&E, including twice in one day.
Blood tests showed a raised D-dimer and a Doppler ultrasound scan confirmed a below-elbow basilic vein DVT. This vein is susceptible to clot formation and drains directly into the axillary vein.
She was admitted and started on warfarin treatment, as she was not breastfeeding, with follow-up by haematology.
This was an uncommon presentation and was nearly missed by both primary and secondary care, in spite of repeated consultations. The patient was at high risk of pulmonary embolism because of her DVT.
Cases of upper extremity DVT (UEDVT) are increasing, because of greater use of central venous lines for chemotherapy, dialysis and parenteral nutrition, and for management of patients following surgery.
UEDVT can refer to thrombosis of any upper limb vein, but occurs most commonly in the axillary or subclavian veins, and can be either primary or secondary.
Primary thrombosis is rare (two per 100,000) and usually idiopathic or resulting from effort thrombosis (Paget-Schroetter syndrome). It is seen in the dominant hand of young, fit, sporty patients where strenuous activity, such as rowing or wrestling, causes repeated microtrauma to the blood vessel intima, setting off the coagulation cascade with potentially significant thrombosis. The situation is worsened if there is also mechanical compression on the veins, as occurs in thoracic outlet syndrome.
Idiopathic cases (as the name implies) occur with no apparent cause. However, occult cancer has been suggested as a trigger for some seemingly idiopathic cases. In one study, a quarter of patients were found to have lung cancer and lymphomas one year after follow-up1. Some cases of idiopathic thrombosis are linked to hypercoagulable states, as seen in patients with previous DVT, antiphospholipid antibodies, factor V Leiden thrombophilia or protein C and S deficiencies.
Secondary thrombosis is responsible for most cases of UEDVT and is found in those with incorrectly positioned central venous catheters, pacemakers, or those with cancer.
Correctly placing the catheter tip will prevent vessel wall damage and subsequent thrombosis. The catheter may also impede blood flow through the vein.
Axillary or subclavian obstruction:
- Occasionally asymptomatic
- Most patients have vague shoulder or arm discomfort
- Arm oedema
- Symptoms of superior vena cava obstruction, for example, face and arm oedema, vertigo, head fullness, blurred vision or dyspnoea
- Dilated cutaneous veins/jugular venous distention
- Extremity cyanosis
- Low-grade fever (attributable to thrombosis)
- Sinus tachycardia, especially in superior vena cava
Thoracic outlet obstruction:
- Pain radiating through the forearm to the fourth and fifth digits
- Hand weakness
- Tenderness on palpation of the supraclavicular fossa
- Positive Adson’s test (weakening of radial pulse when examiner extends affected arm with patient extending neck, and rotates the head towards the affected side)
It is important to note that the clinical features of UEDVT are non-specific. Differentials include lymphoedema, neoplastic compression of the blood vessels, muscle injury and superficial thrombophlebitis. Diagnosis should be confirmed with the following tests.
Duplex ultrasound is preferable because it is non-invasive and readily available, but it may not detect a clot inferior to the clavicle. CT scan is useful, but involves a dye and is not fully validated.
Contrast venography is useful when a high index of suspicion persists despite a normal ultrasound. It is required before and after catheter-directed thrombolysis, to monitor the progress of treatment.
Magnetic resonance is accurate and non-invasive, and provides an opportunity to assess collateral vessels and contralateral blood flow.
Treatment involves anticoagulation, usually for three to six months. Other measures include limb elevation, use of a compression arm sleeve, catheter-directed thrombolysis, suction thrombectomy, or surgical management to eradicate vein compression after thrombolysis, particularly in patients with primary UEDVT.
- Pulmonary embolism in up to one-third of cases1
- Persistent upper extremity pain and swelling
- Loss of vascular access
- Post-thrombotic syndrome in 20% causing disability
- Recurrence of symptoms
- Mortality worse than in lower limb DVT at six and 12 months2
Some oncologists give warfarin 1mg while patients are receiving chemotherapy – although the dose is insufficient to raise the INR, it can prevent thrombosis. It is not suitable for those with liver disease, taking long-term antibiotics or with poor nutrition.
Low-dose heparin with dalteparin 2500IU daily two hours before catheter insertion has been found to be effective at reducing the risk of UEDVT.3
- Dr Agomo is a GP in Borehamwood, Hertfordshire
|Key learning points|
- Joffe H, Goldhaber S. Upper-extremity deep vein thrombosis. Circulation 2002; 106: 1874-80
- Mai C, University South Florida. Presentation: Upper Extremity Deep Venous Thrombosis (UEDVT). Slides available at http://home.smh.com/sections/services-procedures/medlib/education/podcasts/documents/maiMD_12-11-09.pdf
- Monreal M, Alastrue A, Rull M et al. Upper extremity deep venous thrombosis in cancer patients with venous access devices: prophylaxis with a low molecular weight heparin (Fragmin). Thromb Haemost 1996; 75: 251-3