Paget Schroetter Syndrome–in the Context of Work-related Injury: Case Report and Review of Literature
Received: 27-Jul-2021 / Editor assigned: 01-Jan-1970 / Reviewed: 01-Jan-1970 / Revised: 01-Jan-1970 / Accepted Date: 23-Aug-2021 / Published Date: 30-Aug-2021
Abstract
Paget-Schroetter syndrome (PSS) is a primary deep venous thrombosis of subclavian-axillary vein complex after repetitive and strenuous use of shoulders and arms. Here, we report a 24-year-old man who presented with left subclavian and axillary vein thrombosis after loading/unloading labor, diagnosed with PSS via confirmation with Doppler ultrasound, and treated with anticoagulation. By comparing our case with 44 published case reports, we also aim to analyze the patient’s characteristics, diagnostic methods, and treatment options for the disease.
Keywords: Upper extremity deep vein thrombosis; Complications; Diagnosis; Therapy; Physiopathology; Ultrasonography; Treatment outcome
Background
Paget-Schroetter syndrome (PSS) or effort thrombosis is a deep venous thrombosis (DVT) of subclavian-axillary vein complex seen in association with repeated strenuous activity of shoulders and arms such as exercises like swimming, wrestling, and gymnastics [1]. While arm exertion is commonly reported in PSS patients, it is important to note that harmless daily activities can also result in PSS [2]. As PSS is seen with activities in the upper extremities, it is often seen in physically active young individuals, especially male, and has a low incidence rate of 1-2 per 100,000 populations [2]. Symptoms include arm swelling, discomfort as well as heaviness and redness of arm, cyanosis of arm, dilated and visible veins on shoulder and upper arm known as Urschel's sign [2-4]. As it is a form of upper extremity deep vein thrombosis, PSS can result in complications including pulmonary embolism and recurrent thrombosis; therefore, it is imperative to consider possible pulmonary embolism in PSS patients [1,3,5]. Here, we report a case of a 24-year-old male with developed PSS after days of repetitive loading/ unloading labor and will compare the case with other case reports on PSS.
Case Presentation
This is a 24-year-old right-handed Hispanic male who presented to our occupational medicine clinic complaining of diffuse left upper extremity swelling and pain for the last 10 days. His symptoms occurred one week after he started working as a loader/unloader in a warehouse. He had never done any warehouse job before, and this was the first time in his life to experience these symptoms. His new job entitled constant lifting and moving of boxes from the trailer to the warehouse and vice versa. Many of these boxes were above his shoulder level. He also mentioned he felt a “knot-like” sensation at his left axilla. Examination revealed a well-appearing male with average to slightly muscular body habitus. Vital signs were normal. Generalized swelling was noted over the left upper extremity involving the arm, forearm, and hand (Figures 1 and 2). The left upper extremity also appeared to be erythematous compared to the right one with diffuse tenderness. The left arm circumference was 36 cm (right arm was 33 cm), and the left forearm circumference was 31 cm (right was 29 cm). There were conspicuous dilated veins at the upper left side of the chest (pectoral area) (positive Urschel’s sign, (Figures 3 and 4). Radial pulse was intact bilaterally, blood pressure was symmetrical in the two upper extremities and Adson’s test was negative. Shoulder range of motion was intact but he had pain with overhead activity, mainly at the axillary area. No palpable mass was identified in the axilla.
The first impression of the case was thoracic outlet syndrome, which is considered a non-industrial injury. Chest X-ray was performed and was negative for cervical rib. The patient was sent for Duplex ultrasound and CT venography scan of the chest to evaluate for possible axillary venous obstruction. The duplex ultrasound showed extensive occlusive acute deep vein thrombosis throughout the left subclavian vein and extensive non-occlusive thrombosis at the left axillary vein. The CT venography of the chest has also confirmed the findings and was negative for any pulmonary emboli. The patient was placed immediately on oral Apixaban. Comprehensive hypercoagulability workup was performed to evaluate for possible etiological agents. The patient’s D-dimer was slightly elevated to 0.66 mcg/ml (normal <0.5), screening for Factor V Leiden, antithrombin, Protein S and protein C activity came back within the normal limits. Homocysteine, tissueplasminogen activator (tPA), cardiolipin, Plasminogen Activator Inhibitor (PAI-I) level and plasminogen activity were all normal. However, Covid-19 Ig G antibodies came back positive. The patient did not recall any recent or past Covid-19 infection or Covid-19-related symptoms. At this point the diagnosis of Paget-Schroeder syndrome (or Effort-induced thrombosis) was confirmed and the patient was sent for urgent vascular surgery consultation for further evaluation and possible intervention. He was placed on restricted duty with no use of the left upper extremity.
By the time the patient made it to the vascular surgery clinic approximately 5 weeks from starting of the symptoms, it was deemed the thrombus is considered sub-acute and that the patient might not benefit from catheter-directed thrombolysis at that point. First rib resection was offered for the patient (based on the center’s experience) as an elective procedure. However, the patient decided that he wants to continue conservative treatment with the oral anticoagulant and the job modification.
The patient continued to have improvements in his symptoms. On the 12-week follow up the patient has significantly improved in the swelling of the affected arm and forearm (circumference decreased from 36 cm to 35 cm in the upper arm and from 31 cm to 30 cm in the forearm). Duplex ultrasound was performed and showed improvement of the left subclavian thrombosis which became non-occlusive and showed complete resolution of the axillary venous thrombosis. The patient has changed his job from warehouse worker and enrolled in school for computer programming in the hope of becoming a computer technologist in the future.
Discussion
PSS is a relatively rare condition of thrombosis of axillarysubclavian vein [2] which correlates to strenuous activities of upper libs such as swimming, handball, baseball, and bodybuilding [6,7]. It is thought that PSS is caused by micro trauma of venous intima from repetitive use of UE resulting in local coagulation [8-13].
In this review, we looked at 45 previously reported cases of PSS. Majority of the cases were male (35 males and 10 females) which corresponds to previous reviews that showed that most of PSS patients are male. Also, we found that it is more likely that right arm is affected than left arm or both as we found 26 cases involving right arm [14-30] while we found 15 cases involving left arm and 4 cases involving both arms [31-54]. While we found that the right arm is more likely to be affected by PSS than the left arm, it may have to do with the fact that the majority of the population is right-handed and so it is more likely that the right arm is used more, thereby causing such a finding in our review.
On the literature review, there is an association between PSS and repetitive hyper-abduction and/or external rotation involving shoulders; in our review, there have been association of PSS with various activities using shoulders including exercises like baseball [14,28], swimming [17], judo [19] as well as professions such as carpentry [18], photography [25], furniture lifting [27], waiter job [36], and kebab chef job [55-57]. This is consistent with what our patient had with his profession as his work involved frequent luggage loading/ unloading.
Just like our patient who had a complaint of left arm swelling and pain, in the literature review, all the published case reports of PSS demonstrate pain, swelling, and color change of affected arm as symptoms of PSS. Though coldness of the affected arm is a possible presentation of PSS, there was only one case with the symptom on our review [31]. While the clinical manifestation of PSS is one of factors to consider when making the diagnosis, the clinical symptoms of PSS have poor specificity of less than 50% [2]. Therefore, it would have to involve other diagnostic modalities to come to the diagnosis of PSS.
Ultrasound, being noninvasive and affordable, is the first test for PSS with strong sensitivity of 78-100% and specificity of 82-100% [10], in our literature review, most of the cases were diagnosed with ultrasound as well. While catheter-directed contrast venography is the most accurate diagnostic method and thus the gold standard, it is invasive and reserved for cases where upper extremity DVT was not confirmed from other tests [11]. With the incidence of pulmonary embolism (PE) in upper extremity thrombosis of 5.6%, it would also be prudent to consider CT chest angiography to excluded PE in patients with PSS [12]; in our literature review, there was also a reported case of recurrent PE in the setting of PSS (56) as well as a case of bilateral PE [57] which would raise concern of possible PE with PSS further.
Treatment-wise, anticoagulation is the usual and the standard treatments for PSS; 3-6 months of anticoagulation is recommended for patients with PSS. Catheter-directed thrombolysis is indicated for patients with severe symptoms and effective within 14 days from initial presentation [2]. For long-term prevention, surgical management to decompress subclavian vein such as first rib resection can be considered [3] but the recommendations are weak [1]. In our literature review, the majority of patients just received anticoagulation and the patient in our case only received anticoagulation as his case was too late for thrombolysis and he declined any surgical option. While he had improvement in his symptoms and his left subclavian and axillary vein thrombi with follow-ups, it remains whether he would have recurrence of PSS or not.
Conclusion
While PSS is a relatively rare condition, it can result in serious complications including pulmonary embolism. Therefore, it should be considered as one of the differentials in individuals with arm swelling and pain after repetitive exertion of upper extremities. When suspecting PSS, it is imperative to exclude secondary causes of upper extremity DVT with coagulation workups and confirm presence of DVT with diagnostic modalities. Furthermore, it would be prudent to exclude pulmonary embolism with CT chest angiography given PE is one of detrimental sequelae of PSS. The management of PSS includes not only anticoagulation but also surgical intervention such as resection of first rib and thrombolysis which should be discussed with multiple disciplines including vascular surgery to have long term prophylaxis.
References
- Illig KA, Doyle AJ (2010) A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg 51: 1538-1547.
- Alla VM, Natarajan N, Kaushik M, Warrier R, Nair CK (2010) Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis. West J Emerg Med 11: 358-362.
- Urschel Jr HC, Patel AN (2008) Surgery remains the most effective treatment for Paget-Schroetter syndrome: 50 years' experience. Ann Thorac Surg 86: 254-260.
- Joffe HV, Kucher N, Tapson VF, Goldhaber SZ (2004) Upper-extremity deep vein thrombosis: a prospective registry of 592 patients. Circ 110: 1605-1611.
- Kommareddy A, Zaroukian MH, Hassouna HI (2002) Upper extremity deep venous thrombosis. Semin Thromb Haemost 28: 89-99.
- Zell L, Kindermann W, Marschall F, Scheffler P, Gross J, et al. (2001) Paget-Schroetter syndrome in sports activities--case study and literature review. Angiol 52: 337-342.
- Oktar GL, Ergul EG (2007) Paget-Schroetter syndrome. Hong Kong Med J 13: 243-245.
- Ozçakar L, Dönmez G, Yörübulut M, Aydog ST, Demirel H, et al. (2010) Paget-Schroetter syndrome forerunning the diagnoses of thoracic outlet syndrome and thrombophilia. Clin Appl Thromb Hemost 16: 351-355.
- Desjardins B, Rybicki FJ, Kim HS, Fan CM, Flamm SD, et al. (2012)  ACR Appropriateness Criteria® Suspected upper extremity deep vein thrombosis. J Am Coll Radiol 9: 613-619.
- Chin EE, Zimmerman PT, Grant EG. (2005) Sonographic evaluation of upper extremity deep venous thrombosis. J Ultrasound Med 24: 829-840.
- Owens CA, Bui JT, Knuttinen MG, Gaba RC, Carrillo TC (2010) Pulmonary embolism from upper extremity deep vein thrombosis and the role of superior vena cava filters: a review of the literature. J Vasc Interv Radiol 21: 779-787.
- Thiyagarajah K, Ellingwood L, Endres K, Hegazi A, Radford J, et al. (2019) Post-thrombotic syndrome and recurrent thromboembolism in patients with upper extremity deep vein thrombosis: A systematic review and meta-analysis. Thromb Res 174: 34-39.
- Yagi S, Mitsugi M, Sangawa T, Akaike M, Sata M (2017) Paget-Schroetter Syndrome in a Baseball Pitcher. Int Heart J, 58: 637-640.
- Sancho-González I, Bonilla-Hernández MV, Ibañez-Muñoz D, Vicente-Campos D, Chicharro JL (2017) Upper extremity deep vein thrombosis in a triathlete: Again intense endurance exercise as a thrombogenic risk. Am J Emerg Med 35: 808.e1-808.e3.
- Sanson H, Gautier V, Stansal A, Sfeir D, Franceschi C (2016) Deep venous thrombosis of the upper limb in a violin player: The "bow syndrome". J Vasc Dis 41: 396-402
- EEdo Fleta G, Torres Blanco Ã, Gómez Palonés F, Ortiz Monzón E (2016) Combined non-surgical treatment for Paget-Schröetter syndrome: a case report. J med Case Rep 10: 171.
- Peki? PE, Beki? DI, Mari? NI, Ma?kovi? MA (2016) Vascular vibration injury and Paget-Shroetter syndrome. Acta Med Croatica 70: 143-146.
- Ijaopo R, Oguntolu V, DCosta D, Garnham A, Hobbs S (2016) A case of Paget-Schroetter syndrome (PSS) in a young judo tutor: a case report. J Med case Rep 10: 63.
- Thiruchelvam N, Mbuvah F, Kistangari G, Anumandla AK (2015) Upper-limb deep vein thrombosis in Paget-Schroetter syndrome. Cleveland Clin J Med 82: 658-659.
-  Lutter C, Monasterio E, Schöffl V (2015) Rock climbing-related subclavian vein thrombosis. BMJ case Rep.
- Meena M, Harish S, Kewlani JP, Gupta N, Meena VK (2015) Paget-Schroetter Syndrome. Chinese Med J 128: 2694-2695.
- Shimada T, Tounai T, Syoji T, Fukumoto Y (2015) Acute Pulmonary Embolism due to Paget-Schroetter Syndrome. Intern Med 54: 1875-1879.
- Bullock C, Johnston AM (2016) Upper extremity deep vein thrombosis in a military patient. J R Army Med Corps 162: 299-301.
- Beasley R, Braithwaite I, Evans R (2015) Upper extremity deep vein thrombosis in a TV cameraman. Occup Med 65: 337-339.
- Kondo T, Ohira Y, Ikusaka M, Terada K, Takada T (2015) Paget-Schroetter syndrome. QJM. Int J Med 108: 591.
- Stein CM, McLeod A, Devine LA (2015) Spontaneous deep vein thrombosis in the upper extremity of a 45-year-old woman. CMAJ 187: 990-993.
- Jackson SS, O'Brien MJ (2014) Case report: upper extremity deep venous thrombosis in a 19-year-old baseball player. Phys Sportsmed 42: 163-167.
- Keene DJ (2015) Upper extremity deep vein thrombosis (Paget-Schroetter syndrome) after surfing: a case report. Man Ther 20: 358-360.
- Spencer TR, Lagace RE, Waterman G (2014) Effort thrombosis (Paget-Schroetter syndrome) in a 16-year-old male. Am J case Rep 15: 333-336.
- Kellar J, Trigger C (2014) Thoracic outlet syndrome with secondary Paget Schröetter Syndrome: a rare case of effort-induced thrombosis of the upper extremity. West J Emerg Med 15: 364-365.
- Young K, Tunstall O, Mumford A (2014) Subclavian vein thrombosis in an otherwise healthy 9-year-old boy. BMJ Case Rep.
- Rainey CE, Taysom DA, Rosenthal MD (2014) Upper extremity deep venous thrombosis. J Orthop Sports Phys Ther 44:313.
- Dep A, Concannon E, Mc Hugh SM, Burke P (2013) Paget-Schrotter syndrome and complications of management. Case Rep 2013:bcr2013008858.
- Aguilar-Shea AL, Gallardo Mayo C, Sierra Santos L (2016) Regarding a case of prevention of embolism in atrial fibrillation / flutter: double antiplatelet, no please. SEMERGEN, Soc Esp Med Rural Gen 42: 195-197.
- Drakos N, Gausche-Hill M (2013) A case report: a young waiter with Paget-Schroetter syndrome. The J Emerg Med 44: e291-e294.
- Say?n A, Güngör H, Bilgin M, Ertürk U (2012) Paget-von Schrötter syndrome: upper extremity deep vein thrombosis after heavy exercise. Arch Turk Soc Cardiol 40: 354-357.
- Wong DJ, Holm TM, Dyer GS, Gates JD (2015) Late onset venous thoracic outlet syndrome following clavicle non-union fracture: A case report. Vasc 23: 183-187.
- Naeem M, Soares G, Ahn S, Murphy TP (2015) Paget-Schroetter syndrome: A review and Algorithm (WASPS-IR). Phlebology Venous Dis 30: 675-686.
- Higuchi R, Miyawaki M, Yasuga Y, Tomobuchi A, Shigyo H (2019) Paget-Schroetter syndrome accompanied by pulmonary thromboembolism: A case report. J Cardiol Cases 19: 93-96.
- Chu AS, Harkness J, Witmer CM (2017) Spontaneous Subclavian Vein Thrombosis in a Healthy Adolescent Cheerleader: A Case of Paget-Schroetter Syndrome. Pediatr Emerg Care 33: e92-e94.
- Lawless SM, Samson R (2017) Urschel's Sign in Paget Schroetter Syndrome. Am J Med 130: e537.
- Fenando A, Mujer M, Rai MP, Alratroot A (2018) Paget-Schroetter syndrome. Case Rep 2018:bcr-2018-227754.
- Kaczynski J, Sathiananthan J (2017) Paget-Schroetter syndrome complicated by an incidental pulmonary embolism. BMJ Case Rep.
- Norinsky AB, Espinosa J, Kianmajd M, DiLeonardo F (2016) Painless acrocyanosis: Paget-Schroetter syndrome secondary to thoracic outlet obstruction from muscle hypertrophy. Am J Emerg Med 34: 1323.e1-1323.e3
- Wadhawan A, Laage Gaupp FM, Sista AK (2014) Automatic implantable cardiac defibrillator implantation may precipitate effort-induced thrombosis in young athletes: a case report and literature review. Clin Imaging 38: 510-514.
- Shiva C, Saini M (2015) Paget-von Schroetter Syndrome: Upper Extremity Deep Vein Thrombosis after Continuous Lifting of Heavy Weight. J Assoc Phys India 63: 84-85.
- Ringhouse B, Jackson C (2017) Bringing to Light Symptoms and Treatments of Effort Thrombosis (Paget-Schroetter Syndrome) in the Military Population, a Case Study. Mil Med 182: e1826-e1829.
- DeLisa LC, Hensley CP, Jackson S (2017) Diagnosis of Paget-Schroetter Syndrome/Primary Effort Thrombosis in a Recreational Weight Lifter. Phys Ther 97: 13-19.
- Ryan JW, Murphy A, Wrafter S, Ramiah V (2016) Paget Schroetter syndrome imaged in multiple modalities and successfully treated with pharmacomechanical thrombectomy. BMJ Case Rep 2016: 2016218238.
- Noto T, Hashimoto G, Takagi T, Awaya T, Araki T, et al. (2017) Paget-Schroetter Syndrome Resulting from Thoracic Outlet Syndrome and KAATSU Training. Intern Med 56: 2595-2601.
- Weaver LA, Kanter CR, Costantino TG (2019) Effort Thrombosis Provoked by Saxophone Performance. J Emerg Med 56: 323-326.
- O'Keefe S, Carmody KA (2013) Paget-Schroetter syndrome diagnosed by bedside emergency physician performed ultrasound: a case report. J Emerg Med 45: 74-77.
- Huang CY, Wu YH, Yeh IJ, Chen YY, Kung FY (2018) Spontaneous bilateral subclavian vein thrombosis in a 40-year-old man: A case report. Med 97: e0327.
- Sahashi Y, Naito J, Kawasaki M (2019) Recurrent pulmonary embolism related with Paget-Schroetter syndrome: a case report. Eur Heart J Case Rep 3: ytz118.
- Glavich G, Gourley J, Fong V (2017) Paget-Schroetter syndrome with bilateral pulmonary emboli. Radiol Case Rep 13: 28-31.
- Aytekin E, Dogan YP, Okur SC, Burnaz O, Caglar NS (2015) Differential diagnosis of a rare case of upper limb pain: Paget-Schroetter syndrome in a doner kebab chef. J Phys Ther Sci 27: 3333-3335.
Citation: Heo J, Medani K, Juma H (2021) Paget Schroetter Syndrome – in the Context of Work-related Injury: Case Report and Review of Literature. Occup Med Health Aff 9.364.
Copyright: © 2021 Heo J, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Share This Article
Recommended Journals
Open Access Journals
Article Usage
- Total views: 2731
- [From(publication date): 0-2021 - Dec 05, 2024]
- Breakdown by view type
- HTML page views: 1638
- PDF downloads: 1093