The take home messages
- Upper extremity deep vein thromboses (UEDVTs) are rare
- 20% of UEDVTs are primary and are most often associated with thoracic outlet syndrome in young active men (Paget-Schroetter Syndrome) and less commonly are classified as truly idiopathic
- Secondary UEDVTs (80%) are associated with cancer, central venous catheters, pacers and thrombophilia
- Swelling and pain of the upper extremity are the most common clinical signs but the clinical exam is unreliable for diagnosis as these signs are not sensitive or specific
- Ultrasound can rule in the diagnosis of UEDVT but a negative test does not exclude it- CT venography is the gold standard and should be sought in cases with a high index of suspicion
- Less likely to have PE –the rate of clinically apparent PE at diagnosis in patients with UEDVT is about 1/3 that in patients with lower extremity DVT (LEDVT)
- UEDVT very rarely directly causes death but may be associated with poor outcomes because it is most often related to underlying disease
- Treatment remains somewhat controversial but it is most widely accepted that anticoagulation should be identical to patients with LEDVT
- Systemic or catheter directed thrombolysis is not recommended for routine cases of UEDVT but may be considered in the patient with severe symptoms and low bleeding risk
Sample case: An otherwise healthy 28-year-old woman presents to the ED with a 2-day history right shoulder pain. The patient woke with aching pain, worse with movement but not associated with trauma or overuse. Twenty-four hours before coming to hospital she noticed swelling in her right hand, significant enough to make it necessary to cut off her rings. The swelling since resolved but the pain persists. She does not report shortness of breath, chest pain or hemoptysis.
- PMH: no medical conditions, no history of DVT, no cancer, no family history of blood clotting disorders. No medications including OCP. No intravenous drug use. Twenty pack/year smoking history.
- Vital signs: stable and normal
- Physical exam: tenderness to palpation of right cervical soft tissues and abduction limited by pain. No erythema, no limb edema, normal brachial and radial pulses bilaterally
The trouble with this presentation is that soft tissue tenderness and pain on abduction are nonspecific signs that could be related to rotator cuff injury, adhesive capsulitis and any other number of shoulder pathologies. The tip off is the edema. Swelling that makes it necessary to cut off rings is a lot of swelling (remember patients don’t like cutting off rings) and even though we don’t see the edema we have to give weight to the patient’s history not just rely our objective findings to entertain the diagnosis of UEDVT in a patient with no other risk factors for this rare condition.
The patient undergoes an ultrasound that shows noncompressibility and visible intraluminal thrombus in the right axillary and subclavian veins. This seems to be a rare case of idiopathic UEDVT but it is more likely that the secondary cause has not yet been identified. She is treated with LMWH and started on vitamin K antagonists and instructed to follow up with her family doctor for further hypercoaguability risk factor assessment and work up.
Epidemiology: UEDVTs are rare and represent 1-4% of all DVTs and 18% of DVTs in hospitalized patients. The incidence of UEDVTs is on the rise as the number patients with CVCs and implantable pacer devices grows. True idiopathic DVTs are extremely rare.
Risk Factors: Most UEDVTs are not idiopathic and are associated with specific secondary causes or thoracic outlet syndrome. The pathogenesis of UEDVTs, like LEDVTs, is related to Virchow’s triad of vascular injury, stasis and hypercoaguability. Factors that affect any of these three domains will contribute to the risk of clot formation. Important factors in UEDVT include but are not limited to:
- Presence of a CVC: approximately 55% of all UEDVTs are in the setting of a CVC (vascular injury)
- Implantable pacer device: 5% higher risk of UEDVT (vascular injury and stasis associated with underlying condition)
- Cancer: more common in patients with CVC, radiotherapy to or external compression by tumour of the SVC (hypercoaguability)
- Thrombophilia or previous VTE: implicated in 10-62% of non-CVC related UEDVT (hypercoaguability)
- Repetitive hyperabduction (ie baseball pitcher, weight lifter) and overdeveloped anterior scalene muscles leads to thoracic outlet syndrome and direct injury to the SVC (stasis and hypercoaguability)
- Cervical rib or clavicular fracture are contributors to thoracic outlet syndrome (stasis)
Presentation: Up to 66% of all UEDVT patients are asymptomatic. The most common presentation in primary UEDVT is limb swelling and of patients with edema, approximately 40% will also report pain, parasthesias or heaviness. Patients with CVC related UEDVT often present with an inability to draw blood from the catheter and are more often asymptomatic as the clots develop insidiously and are less likely to be occlusive. Despite subtle clinical signs one should hold a high index of suspicion for UEDVT in patients with CVCs. Bottom line-The physical exam has poor sensitivity and specificity (50%) for detecting and diagnosing UEDVT.
Diagnosis: Currently there are no clinical decision rules that in combination with d-dimer can be used to rule out UEDVT without imaging. Everybody gets a compression ultrasound but index of suspicion governs how patients with a negative result are worked up.
- Positive ultrasound = confirms UEDVT
- Negative ultrasound
- Low index of suspicionà serial ultrasounds/STOP= rules out UEDVT.
- High Index of suspicion (cancer, CVC, pacer, thrombophilia, obvious signs without alternate diagnosis)à CT venographyà confirms or rules out UEDVT
Treatment: Historically, conservative treatment of UEDVT with heat, rest and arm elevation was the common approach. No randomized trials evaluating the best treatment for UEDVTs exist but using evidence derived from observational studies the most recent ACCP Guidelines, 9th Ed suggest full anticoagulation for three months with DVTs that include the axillary or more proximal veins. The evidence to support this approach seems moderate at best and studies published as recently as 2012 (Levy et al.) continue to support conservative management. Moderate evidence is not great but it is all that we have to go on.
Thrombolysis should be avoided in all patients but those with severe symptoms and low risk of bleeding. In patients with CVC related UEDVT the catheter can be removed or kept in place with anticoagulation. Other management strategies include SVC filters, thrombectomy and surgical decompression of the thoracic outlet. For full treatment overview see this review of the ACCP Guidelines- http://pulmccm.org/2012/review-articles/accp-guidelines-for-diagnosis-and-management-of-dvt-pe-vte-9th-edition-review/ or the Engelberger review.
Outcome: If left untreated approximately 5% of patients will experience PE, 20% post thrombotic syndrome and 8% will have recurrence. Preventing these negatives outcomes is the impetus for anticoagulation in all patients with UEDVT.
Compared to LEDVT, UEDVT is:
- Less common– because the upper extremities have relatively higher venous flow rates; less gravitational related stasis; fewer venous valves; smaller surface area and higher fibrinolytic activity than the lower extremities
- More likely to be secondary– in patients with UEDVT underlying causes of hypercoaguability need to be chased down!!!
- Less likely to cause PE-but it still can…
- Treated similarly but with less evidence for the approach to management
Marshall, P. S., & Cain, H. (2010). Upper Extremity Deep Vein Thrombosis. Clinics in chest medicine, 31(4), 783-797.
Levy, M. M., Albuquerque, F., & Pfeifer, J. D. (2012). Low Incidence of Pulmonary Embolism Associated With Upper-Extremity Deep Venous Thrombosis. Annals of Vascular Surgery.
Gaffar, M. (2005). Upper extremity deep vein thrombosis. Hosp Physician, 6, 29-34.
Lee, J. A., Zierler, B. K., & Zierler, R. E. (2012). The Risk Factors and Clinical Outcomes of Upper Extremity Deep Vein Thrombosis. Vascular and endovascular surgery, 46(2), 139-144.
Engelberger, R. P., & Kucher, N. (2012). Management of Deep Vein Thrombosis of the Upper Extremity. Circulation, 126(6), 768-773.