Paget-Schroetter Syndrome in the Young and Active
Paget-Schroetter Syndrome in the Young and Active
We report this case of effort thrombosis of the upper extremity (Paget-Schroetter syndrome) caused by hypertrophied muscles. This unusual cause of extrinsic venous compression and intimal injury leading to thrombosis was treated uniquely with good outcome. Untreated symptomatic patients can sustain long-term disability from venous obstruction resulting in significant loss of occupational productivity and quality of life. For the same reason, early catheter directed thrombolysis followed by anticoagulation and surgical intervention are recommended in much of the recent literature. Thrombolysis is the most common form of treatment, followed by surgery, if needed, after careful diagnostic approach. A majority of the literature supports a multimodal approach, but there is no definite consensus on management. This highlights the need for randomized clinical trials to guide management as well as to assess the safety and efficacy of anticoagulants commonly used and to define the optimal duration of therapy after thrombolysis.
Paget-Schroetter syndrome (effort thrombosis of upper extremity) was described by Paget in 1875 and Schroetter in 1884 independently; Huges, while reviewing his cases of spontaneous venous thrombosis, gave it its name. Upper extremity deep vein thrombosis (UEDVT) refers to thrombosis of the axillary and/or subclavian veins. They are classified as primary and secondary based on pathogenesis. Primary UEDVT is a rare disorder (2 per 100,000 persons per year, which comprises (1) Paget-Schroetter Syndrome, also known as effort thrombosis, and (2) idiopathic UEDVT. UEDVT has a potential for considerable morbidity because pulmonary embolism is present in up to a third of patients with UEDVT, and other complications, such as persistent pain and swelling, superior vena cava syndrome, and problems with vascular access, can be disabling. Secondary UEDVT develops in patients with upper extremity central venous catheters, pacemakers, or cancer and accounts for most cases of UEDVT.
Primary UEDVT refers either to effort thrombosis (also called Paget-Schroetter syndrome, or PSS) or idiopathic UEDVT. In contrast to patients with Paget-Schroetter syndrome, patients with idiopathic UEDVT have no known trigger or obvious underlying disease. Idiopathic UEDVT, however, may be associated with occult cancer. In one study, one fourth of patients presenting with idiopathic UEDVT were diagnosed with cancer (most commonly lung cancer or lymphoma) within 1 year of follow-up. Most of these cancers were discovered during the first week of hospital admission for the venous thrombosis. Young, healthy patients with PSS develop spontaneous UEDVT, usually in their dominant arm, after such strenuous activities such as rowing, wrestling, weight lifting, and baseball pitching. The heavy exertion causes microtrauma to the vascular intima and leads to activation of the coagulation cascade. Significant thrombosis may occur with repeated insults to the vein wall, especially if mechanical compression is also present. The optimal management of subclavian vein effort thrombosis remains a dilemma because outcomes after different treatment strategies are based on isolated case reports and small retrospective series. This decision should be based on the cause of thrombosis and should be individualized. We report a case of Paget-Schroetter syndrome caused by hypertrophied coracobrachialis and short head of the biceps in a very active young man.
We report this case of effort thrombosis of the upper extremity (Paget-Schroetter syndrome) caused by hypertrophied muscles. This unusual cause of extrinsic venous compression and intimal injury leading to thrombosis was treated uniquely with good outcome. Untreated symptomatic patients can sustain long-term disability from venous obstruction resulting in significant loss of occupational productivity and quality of life. For the same reason, early catheter directed thrombolysis followed by anticoagulation and surgical intervention are recommended in much of the recent literature. Thrombolysis is the most common form of treatment, followed by surgery, if needed, after careful diagnostic approach. A majority of the literature supports a multimodal approach, but there is no definite consensus on management. This highlights the need for randomized clinical trials to guide management as well as to assess the safety and efficacy of anticoagulants commonly used and to define the optimal duration of therapy after thrombolysis.
Paget-Schroetter syndrome (effort thrombosis of upper extremity) was described by Paget in 1875 and Schroetter in 1884 independently; Huges, while reviewing his cases of spontaneous venous thrombosis, gave it its name. Upper extremity deep vein thrombosis (UEDVT) refers to thrombosis of the axillary and/or subclavian veins. They are classified as primary and secondary based on pathogenesis. Primary UEDVT is a rare disorder (2 per 100,000 persons per year, which comprises (1) Paget-Schroetter Syndrome, also known as effort thrombosis, and (2) idiopathic UEDVT. UEDVT has a potential for considerable morbidity because pulmonary embolism is present in up to a third of patients with UEDVT, and other complications, such as persistent pain and swelling, superior vena cava syndrome, and problems with vascular access, can be disabling. Secondary UEDVT develops in patients with upper extremity central venous catheters, pacemakers, or cancer and accounts for most cases of UEDVT.
Primary UEDVT refers either to effort thrombosis (also called Paget-Schroetter syndrome, or PSS) or idiopathic UEDVT. In contrast to patients with Paget-Schroetter syndrome, patients with idiopathic UEDVT have no known trigger or obvious underlying disease. Idiopathic UEDVT, however, may be associated with occult cancer. In one study, one fourth of patients presenting with idiopathic UEDVT were diagnosed with cancer (most commonly lung cancer or lymphoma) within 1 year of follow-up. Most of these cancers were discovered during the first week of hospital admission for the venous thrombosis. Young, healthy patients with PSS develop spontaneous UEDVT, usually in their dominant arm, after such strenuous activities such as rowing, wrestling, weight lifting, and baseball pitching. The heavy exertion causes microtrauma to the vascular intima and leads to activation of the coagulation cascade. Significant thrombosis may occur with repeated insults to the vein wall, especially if mechanical compression is also present. The optimal management of subclavian vein effort thrombosis remains a dilemma because outcomes after different treatment strategies are based on isolated case reports and small retrospective series. This decision should be based on the cause of thrombosis and should be individualized. We report a case of Paget-Schroetter syndrome caused by hypertrophied coracobrachialis and short head of the biceps in a very active young man.