It can be thought of as a venous equivalent of thoracic outlet syndrome. Patients present with symptoms and signs of an upper limb deep vein thrombosis i. Compression of the subclavian vein when it passes through the costoclavicular space is the most favored mechanism of thrombosis. Various surrounding structures that lie in close proximity to subclavian vein, when it passes through the costoclavicular space, have been postulated to cause the compression.
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Effort thrombosis, or Paget-Schroetter Syndrome, refers to axillary-subclavian vein thrombosis associated with strenuous and repetitive activity of the upper extremities. Anatomical abnormalities at the thoracic outlet and repetitive trauma to the endothelium of the subclavian vein are key factors in its initiation and progression.
The role of hereditary and acquired thrombophilias is unclear. The pathogenesis of effort thrombosis is thus distinct from other venous thromboembolic disorders. Doppler ultrasonography is the preferred initial test, while contrast venography remains the gold standard for diagnosis. Computed tomographic venography and magnetic resonance venography are comparable to conventional venography and are being increasingly used.
Conservative management with anticoagulation alone is inadequate and leads to significant residual disability. An aggressive multimodal treatment strategy consisting of catheter-directed thrombolysis, with or without early thoracic outlet decompression, is essential for optimizing outcomes.
Despite excellent insights into its pathogenesis and advances in treatment, a significant number of patients with effort thrombosis continue to be treated suboptimally.
Hence, there is an urgent need for increasing physician awareness about risk factors, etiology and the management of this unique and relatively infrequent disorder. Effort thrombosis refers to axillary-subclavian vein thrombosis ASVT associated with strenuous and repetitive activity of the upper extremities. The earliest description of spontaneous ASVT was by Cruveilhier in , and the first elaborate account was provided by James Paget in Effort thrombosis usually follows sporting activities, such as wrestling, playing ball, gymnastics and swimming, which involve vigorous and sustained upper extremity movements.
Substantial evidence now supports the role of anatomical abnormalities involving the thoracic outlet cervical rib, congenital bands, hypertrophy of scalenus tendons and abnormal insertion of the costoclavicular ligament in the pathogenesis of effort thrombosis.
The narrow costoclavicular space leads to compression of the vein and to stasis in the flow. More importantly, it restricts the mobility of the subclavian vein, making it more susceptible to trauma from arm use. These lead to a self-perpetuating cycle of endothelial trauma, thrombosis and recanalization.
The repetitive endothelial trauma leads to intimal hyperplasia, inflammation and fibrosis, resulting in venous webs, extensive collateral formation and perivenular fibrosis. This in turn worsens the stasis and costoclavicular crowding. Effort thrombosis has therefore been rightfully categorized as a venous variant of thoracic outlet syndrome.
Normal anatomy of the thoracic outlet. Abnormal lateral insertion of the costoclavicular ligament in Paget-Schroetter syndrome. Some investigators have reported a higher frequency of factor V Leidin, Prothrombin gene mutation and other inherited thrombophilic states in patients with idiopathic UEDVT. However, others have refuted this association by demonstrating that the frequency of inherited thrombophilias in patients with effort thromboses was comparable to that of the general population.
They found that the increased frequency of concurrent thrombophilic disorders was limited to patients with idiopathic UEDVT, which was not effort related. In summary, costoclavicular crowding due to anatomical abnormalities and repetitive endothelial trauma from muscular strain are the key pathogenic factors in the initiation and progression of effort thrombosis.
Inflammation, by leading to perivenular fibrosis and adhesions, seems to play a contributory role in the perpetuation of obstruction. The role of inherited and acquired thrombophilic disorders remains unresolved and needs further investigation. Not surprisingly, effort thrombosis is more common in young and otherwise healthy men. It preferentially involves the dominant arm. Unlike those with UEDVT secondary to central venous catheters, patients with effort thrombosis are usually symptomatic.
Swelling and arm discomfort are the most frequent presenting problems. Not infrequently, symptoms can be nonspecific and can even mimic a muscular strain. Occasionally, minor and relatively innocuous day-to-day activities can precipitate effort thrombosis. Post-thrombotic syndrome characterized by pain, heaviness, and swelling , on the other hand, is more frequent in effort thrombosis, compared to secondary UEDVT, and is the major contributor to the morbidity associated with disease.
However, invasive nature, high cost, and the accuracy of non-invasive tests have relegated venography to the background. These tests are therefore second line and reserved for patients with high clinical probability of effort thrombosis and negative ultrasound. Though venography is not necessary for diagnosis, it is almost always done as a part of multimodal treatment strategy to deliver catheter-directed thrombolysis and plan thoracic outlet decompression surgery.
Contemporary management of effort thrombosis varies widely, and there is no broad consensus as to what constitutes the best approach. The relative rarity of the disease, paucity of awareness and lack of large randomized trails are factors contributing to this confusion. For many years patients with effort thrombosis were managed conservatively with limb elevation and anticoagulation alone.
However, subsequent long-term data demonstrated an unacceptably high incidence of residual symptoms, disability and recurrent thrombosis with this conservative strategy. Systemic fibrinolysis is superior to anticoagulation in achieving vein patency but is associated with higher rates of complications such as intracranial hemorrhage. While some authors recommend using fibrinolytics only in patients presenting within two weeks of symptom onset, others have reported fair outcomes even with a delay of about four to six weeks.
The duration of treatment and dose of these agents is not standardized and therefore varies among various institutions. Most patients require prolonged infusion of the fibrinolytic agent for catheter directed thrombolysis; average durations varying between 24—48 hours. Therapy directed at thoracic outlet decompression TOD has become an integral part in the management of effort thrombosis with the recognition of the central role of thoracic outlet obstruction.
TOD involves resection of the first rib, division of the scalenus muscles and the costoclavicular ligament using either a transaxillary or infraclavicular approach. Some investigators reserve TOD only for patients with persistent or recurrent symptoms following catheter-directed thrombolysis. Lee et al. Some suggest that the anterior, or sub-clavicular, approach is superior because it allows better exposure of the proximal subclavian and innominate veins, thereby ensuring more optimal vein repair.
Moreover, thoracic outlet decompression is a complicated surgery and should be undertaken only by experienced surgeons at centers with expertise in managing patients of effort thrombosis.
The need for a detailed discussion with patients regarding the risks and benefits of surgery cannot be overstated. So far, definite markers predicting failure of thrombolysis and need for surgery have not been identified. Surgical thrombectomy, balloon venoplasty and stenting have practically been abandoned due to the limited success, high procedural morbidity, and high rates of stent fracture.
It is reasonable to seek early surgical vascular and thoracic consultation in all young patients with UEDVT especially, in the absence of obvious precipitating factors, such as a central venous line. Finally, the need for and the duration of anticoagulation in patients treated with a combination of catheter-directed thrombolyis and TOD remains unclear. While some authors recommend no anticoagulation when good surgical results are obtained, others prefer anticoagulation for at least two to three months 12 , 36 As already mentioned, the role of inherited and acquired thrombophilic states in effort thrombosis is unclear.
Nevertheless, it is reasonable to test at least selected patients for these abnormalities as they might help predict postoperative complications, recurrence rates and determine the need for long-term anticoagulation.
It appears reasonable to recommend long-term anticoagulation in patients with coexistent thrombophilias and for those presenting late and having suboptimal surgical results. However, the efficacy of this strategy in preventing recurrent thrombosis and alleviating symptoms is unclear and needs further study. In summary, effort thrombosis is a complex and relatively infrequent disorder with a distinct pathogenesis. Most physicians unfamiliar with effort thrombosis manage it similarly to classic lower extremity DVT.
Instead, effort thrombosis is ideally managed using a multimodal approach consisting of routine catheter-directed thrombolysis, early TOD in appropriate patients and physical and occupational therapy. Long-term anticoagulation may be reasonable in patients with coexistent thrombophilia and suboptimal surgical results.
Increasing awareness among primary care and emergency physicians will ensure early recognition, timely thrombolysis, and prompt referral to a thoracic or vascular surgeon. Future research should focus on defining the benefit of thrombolytic therapy in patients presenting late, identifying factors that predict failure of thrombolysis and need for surgery. Other avenues for research include assessment of the need for and duration of anticoagulation following TOD, and cost benefit analysis of the various treatment strategies.
Conflicts of Interest: By the WestJEM article submission agreement, all authors are required to disclose all affiliations, funding sources, and financial or management relationships that could be perceived as potential sources of bias.
The authors disclosed none. National Center for Biotechnology Information , U. West J Emerg Med. Venkata M. Chandra K. Author information Article notes Copyright and License information Disclaimer.
Email: ude. This article has been cited by other articles in PMC. Abstract Effort thrombosis, or Paget-Schroetter Syndrome, refers to axillary-subclavian vein thrombosis associated with strenuous and repetitive activity of the upper extremities.
Open in a separate window. Figure 1. Figure 2. Figure 3. Diagnostic and management algorithm for patients with suspected effort thrombosis. TOD, thoracic outlet decompression. Cruveilhier LJB. Doctoral thesis. Paget J. Clinical Lectures and Essays. London: Longman Green; Vienna, Austria: Holder; Hughes ESR. Hughes ES. Surg Gynecol Obstet. Upper extremity deep venous thrombosis. Semin Thromb Hemost. Upper extremity deep vein thrombosis: a community-based perspective.
Am J Med. Upper-extremity deep vein thrombosis: a prospective registry of patients. Central venous thrombosis: an early and frequent complication in cancer patients bearing long-term silastic catheters.
Paget-Schroetter Syndrome: Review of Pathogenesis and Treatment of Effort Thrombosis
Venous thromboembolic disease VTED in the upper extremities is an infrequent entity, although its incidence has increased in relation to the use of central venous catheters. Its etiology can be primary idiopathic, spontaneous, due to effort or traumatic or secondary related to tumours, central venous catheters, etc. We present a case of primary venous thrombosis of the upper right extremity, also called the Paget-Schroetter syndrome. The clinical picture usually consists of pain in the affected extremity, frequently accompanied by edema and collateral circulation. Echography-Doppler frequently presents false negatives, and it is recommendable to carry out CAT, due to its greater specificity and for evaluating the neighbouring structures, although flebography continues to be the cardinal test for diagnosing this picture. There is no unanimity of opinion concerning treatment, and it is recommendable that this should be individualised in accordance with the characteristics of each case.
[Paget-Schroetter Síndrome Associated With Hyperhomocsyteinemia]
Paget—Schroetter disease , is a form of upper extremity deep vein thrombosis DVT , a medical condition in which blood clots form in the deep veins of the arms. The condition is relatively rare. The syndrome also became known as "effort-induced thrombosis" in the s,  as it has been reported to occur after vigorous activity,  though it can also occur due to anatomic abnormality such as clavicle impingement  or spontaneously. It may develop as a sequela of thoracic outlet syndrome. It is differentiated from secondary causes of upper extremity thrombosis caused by intravascular catheters.
Effort thrombosis, or Paget-Schroetter Syndrome, refers to axillary-subclavian vein thrombosis associated with strenuous and repetitive activity of the upper extremities. Anatomical abnormalities at the thoracic outlet and repetitive trauma to the endothelium of the subclavian vein are key factors in its initiation and progression. The role of hereditary and acquired thrombophilias is unclear. The pathogenesis of effort thrombosis is thus distinct from other venous thromboembolic disorders. Doppler ultrasonography is the preferred initial test, while contrast venography remains the gold standard for diagnosis. Computed tomographic venography and magnetic resonance venography are comparable to conventional venography and are being increasingly used.
Archivos de Bronconeumologia http: www. Other types of articles such as reviews, editorials, special articles, clinical reports, and letters to the Editor are also published in the Journal. It is a monthly Journal that publishes a total of 12 issues, which contain these types of articles to different extents. All manuscripts are sent to peer-review and handled by the Editor or an Associate Editor from the team.