**8. Treatment**

Due to the lack of adequately powered, randomized clinical trials the current guidelines for the treatment of arm vein thrombosis are mainly based on small cohort studies, expert opinion or extrapolation of data derived from larger studies performed in patients with lower extremity deep vein thrombosis. Nevertheless, the eight edition of the American college of chest physicians' (ACCP) guidelines cover several important aspects of the treatment of patients with upper extremity deep vein thrombosis (Kearon et al*.*, 2008). For the initial treatment, therapeutic doses of low molecular weight heparin, unfractionated heparin or fondaparinux are recommended. Overlapping with this initial treatment, longterm anticoagulation with a vitamin K antagonist should be started and continued for a minimum of 3 months. No studies are available that have addressed the ideal duration of

Compared to lower extremity deep vein thrombosis, relatively little is known about the natural history of deep arm vein thrombosis. The follow-up of patients not receiving anticoagulant treatment was reviewed by Thomas & Zierler, who found high rates of postthrombotic syndrome (74%) and pulmonary embolism (12%) in patients treated only with physical methods (rest, heat, elevation) (Thomas & Zierler, 2005). These findings underscore the importance of a correct and fast diagnosis of upper extremity deep vein thrombosis and challenge the common view of arm vein thrombosis as a relatively harmless disease. However, even patients that are treated according to current guidelines have a significant risk of severe complications (Table 3). Compared to lower extremity deep vein thrombosis, patients with deep arm vein thrombosis present less frequently with concomitant pulmonary embolism (9% versus 30%) (Munoz et al*.*, 2008; Lechner et al*.*, 2008). However, pulmonary embolism caused by deep arm vein thrombosis can even be fatal in rare cases (Monreal et al*.*, 1994). With the exception of patients with malignancies, the recurrence rate tends to be lower than in deep vein thrombosis (Spencer et al*.*, 2007; Munoz et al*.*, 2008). However, the total mortality of patients with upper extremity and lower extremity thrombosis appears to be similar and is mainly related to the underlying disease (Spencer et al*.*, 2007; Munoz et al*.*, 2008). Contrary to common believe, post-thrombotic syndrome is not a rare complication in deep arm vein thrombosis and may lead to functional disability and significant impaired quality of life in these patients (Prandoni et al*.*, 2004; Kahn et al*.*, 2005; Vik et al*.*, 2009). Two modified versions of a validated score system for post-thrombotic syndrome in lower extremity thrombosis

(Prandoni et al*.*, 1997b) have been adapted to arm vein thrombosis (Table 4).

(7.4%)

(8.9 %)

10 / 1156 (0.87%)

141 / 610 (23%)

Table 3. Common complications of deep arm vein thrombosis.

**Complication Frequency References**

Due to the lack of adequately powered, randomized clinical trials the current guidelines for the treatment of arm vein thrombosis are mainly based on small cohort studies, expert opinion or extrapolation of data derived from larger studies performed in patients with lower extremity deep vein thrombosis. Nevertheless, the eight edition of the American college of chest physicians' (ACCP) guidelines cover several important aspects of the treatment of patients with upper extremity deep vein thrombosis (Kearon et al*.*, 2008). For the initial treatment, therapeutic doses of low molecular weight heparin, unfractionated heparin or fondaparinux are recommended. Overlapping with this initial treatment, longterm anticoagulation with a vitamin K antagonist should be started and continued for a minimum of 3 months. No studies are available that have addressed the ideal duration of

Martinelli et al. 2004, Bernardi et al. 2006, Spencer et al. 2007, Munoz et al. 2008, Isma et al. 2010

Kommareddy et al 2002, Bernardi et al. 2006, Spencer et al. 2007, Munoz et al. 2008, Lechner et al. 2008

Bernardi et al. 2006, Munoz et al. 2008

Bernardi et al. 2006

**7. Natural history and complications** 

Recurrence 78 / 1060

Pulmonary embolism 186 / 2094

Fatal pulmonary embolism

Post-thrombotic syndrome

**8. Treatment** 

anticoagulant therapy in patients with arm vein thrombosis. There is no specific recommendation in the ACCP guidelines on the treatment of cancer patients with upper extremity deep vein thrombosis. In cancer patients with deep arm vein thrombosis, the use of low molecular weight heparins instead of vitamin K antagonist as long-term treatment has been suggested in analogy to lower extremity thrombosis, but there are currently no studies supporting this approach (Shivakumar et al*.*, 2009). Although various degrees of post-thrombotic syndrome have to be expected in the long term follow up of about 1 in 4 patients with upper extremity deep vein thrombosis, the ACCP guidelines do not advocate the routine use of elastic bandages or compression sleeves for the arm, unless patients report severe symptoms like persistent edema and pain.

A number of studies have described case series of deep arm vein thrombosis treated with a variety of invasive therapeutic options, including catheter-guided thrombolysis, percutaneous angioplasty with or without venous stent insertion, surgical thrombectomy and surgical decompression of costoclavicular narrowing to correct thoracic inlet syndrome, e.g. by first rib resection (Zimmermann et al*.*, 1981; Becker et al*.*, 1983; Machleder, 1993; Urschel & Razzuk, 1998). Some investigators recommend such an invasive approach routinely e.g in patients with effort related thrombosis (Paget von Schroetter´s syndrome) (Kommareddy et al*.*, 2002). Here, the ACCP guidelines clearly do not recommend invasive procedures routinely, but only in selected patients and in specially equipped centers. It remains to be determined in adequately designed, randomized clinical trials whether these invasive procedures, which carry a substantial risk of major bleeding and other serious complications, provide a benefit compared to standard anticoagulation with optimal mechanical compression using elastic bandages.


Table 4. Two suggested modifications of the Villalta scale for the assessment of postthrombotic syndrome in deep arm vein thrombosis. Each sign or symptom is graded as 0 (absent), 1 (mild), 2 (moderate) or 3 (severe). A score of 5 or higher is classified as postthrombotic syndrome and score of 15 or higher as severe post-thrombotic syndrome.

Another point of debate is the question whether central venous catheters should be removed when a diagnosis of deep vein thrombosis has been confirmed in the respective vessel. Most experts opt against catheter removal, if the catheter is still needed and still functional. In a cohort study of 74 cancer patients with acute upper extremity thrombosis, the catheters were not removed and patients were treated for 3 months with standard anticoagulation without recurrent episodes of venous thromboembolism (Kovacs et al., 2007). If the catheter is removed, the ACCP guidelines recommend not to shorten the anticoagulation period below 3 months (Kearon et al*.*, 2008).

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