|Year : 2014 | Volume
| Issue : 1 | Page : 12-13
The Management of Acute Upper Limb Deep Vein Thrombosis
Department of Vascular Surgery, St. Georges Vascular Institute, London SW17 0QT, United Kingdom
|Date of Web Publication||9-Oct-2014|
Department of Vascular Surgery, St. Georges Vascular Institute, London SW17 0QT,
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Loftus I. The Management of Acute Upper Limb Deep Vein Thrombosis. Indian J Vasc Endovasc Surg 2014;1:12-3
| Introduction|| |
Primary axillary/subclavian vein thrombosis, also known as Paget-Schroetter syndrome, account for around 25% of all upper limb deep vein thromboses.  Although the condition is not common, it is under-diagnosed, often presents late, and the sequelae for young, often athletic patients, are significant. The mainstay of treatment in the past has been the elevation of the affected limb, and anticoagulation, often long-term. However, a more aggressive interventional policy has been advocated, incorporating catheter-directed thrombolysis and decompression of the thoracic outlet. This has demonstrated promising results though with little long-term follow-up data in the literature. The aim of a surgical approach is to prevent re-thrombosis, and enable cessation of anticoagulation.
The keys to successful management and good clinical outcomes are early diagnosis and early intervention with lysis and decompression of the thoracic outlet. There are subgroups of patients who may not benefit from surgery, and who may actually do worse as a result of loss of collateral venous channels in the thoracic outlet from surgery. There are other risks of surgery including significant nerve damage. Effective management requires careful consideration of the imaging, and as assessment of the relative risks and benefits of a surgical approach.
| Primary Subclavian Vein Thrombosis|| |
Primary subclavian vein thrombosis is also known as Paget-Schroetter syndrome. Unfortunately, there is a paucity of good quality literature to guide us in terms of the management or outcomes from Paget-Schroetter syndrome. There is an established link with exertion of the affected upper limb, giving rise to the term "effort thrombosis." The condition has gained publicity through affecting well-known sportsmen and women.
The incidence of upper limb deep vein thrombosis is uncertain but is estimated to account for 1-4% of all deep vein thromboses.  This is almost certainly an under-estimate, as a proportion of affected individuals will be un-diagnosed. Paget-Schrotter condition probably accounts for around 25% of these, the rest being secondary to underlying aetiologies including underlying malignancy, central lines and drug abuse. 
The underlying cause of primary subclavian vein thrombosis is accepted as being related to compression of the vein as it passed through the thoracic outlet, more specifically in the narrow angle created by the costo-clavicular space. The precise anatomical problem is, however, largely undefined, with numerous anatomical abnormalities described, but little in the way of definitive descriptive studies. There is almost certainly a further causative aspect in the form of repetitive trauma causing fibrosis of the vein. Clearly any form of decompressive surgery will not reverse this, though it may slow down the degenerative process.
While it is recognised that the subclavian vein can occlude on hyper-abduction of the arm in normal individuals, it is more commonly observed in those who have experienced a spontaneous thrombosis, identified in up to 80% of contra-lateral limbs.  That said, the incidence of bilateral thrombosis is relatively low, creating a management dilemma in terms of offering surgical decompression of the opposite side. 
| Clinical Effects of Subclavian Vein Thrombosis|| |
There is a predilection for men, with an average age of around 30, and often with a preceding history of strenuous or repetitive exercise.  The dominant arm is more often affected. The presentation is the upper limb swelling of variable degrees, sometimes accompanied by pain and discolouration. The rationale for early and aggressive intervention is the long-term effects if untreated. It is now recognised that up to 90% will have persistent symptoms, especially with exertion, preventing a return to normal activity.  There is also a small but definite risk of pulmonary embolism.
| The Role of Lysis and Decompressive Surgery|| |
Effective early diagnosis relies on a high index of suspicion and rapid access to duplex ultrasound. Identification of fresh thrombus should lead to urgent venography with a view of early catheter-directed thrombolysis.
Catheter-directed lysis has largely replaced thrombectomy but will not provide good results unless combined with a surgical approach to decompress the thoracic outlet. This incorporates resection of the first rib along with the anterior and middle scalene muscles. Surgery does entail a small but significant risk of complications. These included the potential for nerve injury, in particular to the brachial plexus, the long thoracic nerve, the phrenic nerve and a risk of Horner's syndrome. Further risks include bleeding and lymph leak, especially on the left side related to damage to the thoracic duct.
There has been discussion about the optimum timing of surgery with evidence tending to suggest an early approach is beneficial.  On the other hand, surgery will not provide a useful adjunct to lysis if the result of lysis is poor. Therefore, decompression of the thoracic outlet should be mandatory in patients who have had successful lysis, possibly with the aid of venoplasty, but not in those with significant residual venous obstruction.
The success of lysis depends upon the extent of thrombus, the duration from the primary event, and the degree of underlying venous damage. While it feels inherently right to decompress the thoracic outlet whenever possible, there is little in the literature to guide the clinician in terms of patient selection. There must be a careful consideration of the effect of lysis and an examination of the postlysis imaging.
In the series of Machleder, 14% of patients had residual problems despite successful lysis and first rib resection.  Of those in whom lysis failed to provide good vein patency, 64% were symptom-free. In our series from St. Georges, 10% of patients who have undergone lysis and rib resection continue to have problems.  These are all patients with a delay of greater than 7 days between onset of symptoms and lysis. Similar results were demonstrated by Stone et al., who reported a 100% vein patency rate at 1-year, and 94% patency at 5 years, in patients who underwent lysis within 12 days of inset of symptoms followed by rib resection.  However, patients who presented late due to a delay in making the diagnosis had significantly worse outcomes. Guzzo et al. demonstrated that patients who presented with sub-acute venous thoracic outlet did not benefit from intervention.  These are largely patients who demonstrate residual thrombus, which fails to respond to lysis. Not all of these patients should be considered for mandatory rib resection.
Molina et al. presented a series of 126 cases over a 23-year period and demonstrated a 100% patency record following lysis and surgery but adopted policy of vein patching to restore vein calibre.  This is far more extensive surgery than simple decompression including rib resection, incorporating a limited transverse sternotomy. Others have adopted a policy of selected postoperative venoplasty, some advocating the use of venous stents. There is little data to support a policy of venous stenting and no good data on long-term outcomes. Neither is there any data to support a primary endovascular solution to venous obstruction.
| Summary|| |
Subclavian vein thrombosis often affects young, fit adults. The long-term sequelae of subclavian vein thrombosis and significant, often leading to disabling symptoms. The success of treatment depends on early recognition. A combined endovascular and surgical approach is beneficial, comprising catheter-directed thrombolysis and early thoracic outlet decompression including first rib resection. This is not mandatory in sub-groups of patients, namely, those without evidence of continued vein compression following lysis, and hose who present late, in particular more than 2 weeks after the initial event, who fail to respond well to lysis. There is also a group, as yet poorly defined, with residual significant venous obstruction despite lysis and venoplasty, caused by intrinsic venous fibrosis.
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