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Radiol Case Rep
Radiol Case Rep
Radiology Case Reports
1930-0433
Elsevier

S1930-0433(24)00762-3
10.1016/j.radcr.2024.07.176
Case Report
Beyond an upper extremity clot: A case report of paget-schroetter syndrome
Ebrahim Mohamed Ayman MD mohdaymanebrahim@gmail.com
⁎
Adhikari Bibek MD
Wazir Hina MD
Bhattarai Hari MD
Chalise Shyam MD
Internal Medicine, Ascension Saint Joseph Hospital, Chicago, IL 60657, USA
⁎ Corresponding author. mohdaymanebrahim@gmail.com
24 8 2024
11 2024
24 8 2024
19 11 52315237
20 7 2024
29 7 2024
© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Paget-Schroetter syndrome (PSS), a rare form of deep vein thrombosis affecting the upper extremity, arises from mechanical compression of the subclavian vein at the thoracic outlet. Typically seen in young, active individuals, it manifests with acute onset of arm pain, swelling, and discoloration. Early diagnosis is crucial to prevent chronic complications such as post-thrombotic syndrome, emphasizing the importance of timely intervention and individualized treatment approaches for improved clinical outcomes. We present a case of PSS manifesting in a young adult with no significant medical history.

Keywords

Effort thrombosis
Upper extremity deep vein thrombosis
Vascular thrombosis
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pmcIntroduction

Paget-Schroetter syndrome (PSS), also known as “effort vein thrombosis,” is characterized by the formation of blood clots in the subclavian and axillary veins, often triggered by repetitive and strenuous activities involving the upper extremities, such as weightlifting, swimming, pitching, and wrestling. This condition typically affects active men in their twenties or thirties, with symptoms more commonly appearing in the dominant arm [1]. The underlying mechanism involves repeated microtrauma to the subclavian and axillary veins caused by hyperabduction or extension of the arm, exacerbated by mechanical compression from nearby muscles, bones, or ligaments. Over time, this leads to chronic scarring and eventually, blood stasis and clot formation [2]. PSS is a relatively rare condition, occurring in approximately 1 to 2 individuals per 100,000 annually, and constitutes 1% to 4% of all venous thromboembolic events [3]. We present a case of Paget-Schroetter syndrome (PSS) occurring in early adulthood in a man with an unremarkable medical history.

Case presentation

A 28-year-old Caucasian man with no significant past medical history presented to the emergency department with a worrisome 3-day history of swelling, color changes, and discomfort in his right upper extremity, which is his dominant arm. Initially attributing the symptoms to exercise-induced muscle soreness, he observed significant swelling which persisted despite rest. Engaging in regular resistance training workouts multiple times per week, he sought immediate care after the swelling failed to subside and the tingling sensation over his right arm persisted. The patient denied any recent trauma to the neck or right upper extremity and reported the absence of chest pain, shortness of breath, nausea, vomiting, diarrhea, fever, or chills. Medical history was unremarkable, and there was no family history of any clotting disorders. He leads an independent lifestyle and works in an office-based job. Smoking and tobacco use are negligible, with minimal alcohol consumption disclosed. The patient admits to using cannabis 3 times weekly but denies any illicit drug use.

Upon evaluation in the emergency department, the patient presented with a blood pressure of 132/59 mmHg, Pulse of 64, Temperature of 97.7 F, and peripheral oxygen saturation level of 98% while breathing room air. On examination, swelling and erythema are noted in the right upper extremity extending from the shoulder to the hand, with no pain noted upon palpation, as illustrated in Fig 1. Distal pulses are palpable. Chest X-ray and right shoulder X-ray yielded no significant abnormalities. A venous duplex ultrasound of the upper extremity revealed an occlusive deep vein thrombosis involving the right mid to distal subclavian, axillary, and proximal to mid brachial veins, as shown in Fig 2. Laboratory studies including complete blood count and comprehensive metabolic panel returned within normal limits. Therapeutic anticoagulation with lovenox was initiated in the emergency department, leading to subsequent admission for further management.Fig. 1 Clinical Photo demonstrating marked right upper extremity edema and redness when compared to the left side.

Fig 1

Fig. 2 The duplex ultrasound image reveals an acute occlusive deep venous thrombosis involving the right mid to distal subclavian, axillary, and proximal to mid brachial veins. This is evidenced by the absence of color flow and venous waveform in these regions, alongside the presence of an acute thrombus.

Fig 2

Hematologist was consulted for further recommendations and management of spontaneous upper extremity DVT. Antithrombin III activity, protein C, and factor V Leiden results were nonsignificant. Due to concerns of thoracic outlet syndrome, contrast enhanced CT of the chest was performed, showing mild prominence of the right scalene muscle with no clear evidence of external compression, as shown in Fig 3. Given the extensive occlusive nature of his clots that could result in significant venous insufficiency, interventional radiology was consulted to arrange for thrombectomy. Venography revealed extensive occlusion across the right brachial, axillary, and subclavian veins, all exhibiting thrombus formation, as depicted in Fig 4. Notably, marked collateral flow was observed. With meticulous precision, the wire was advanced beyond the occluded regions. The thrombus was laced with 2 mg of TPA and allowed to dwell for 15 minutes. Then, 200 seconds of suction thrombectomy was performed with a Angiojet suction catheter. Upon observation, there was a minimal residual thrombus and severe stenosis of the subclavian vein. Therefore, angioplasty was performed on the right subclavian vein utilizing a 12 mm × 4 cm balloon (Fig. 5). Subsequent completion of venography revealed restored in-line flow in the right brachial, axillary, and subclavian veins, showcasing a significant reduction in collateral vein prominence, nearly resolving them entirely (Fig. 6). The patient reported significant improvement in his symptoms and was discharged on Eliquis for a total of 6 months duration.Fig. 3 Contrast enhanced CT of the chest, showing mild prominence of the right scalene muscle with no clear evidence of external compression.

Fig 3

Fig. 4 Contrast venogram showing near-complete occlusion of the right brachial vein, axillary vein, and subclavian vein due to thrombus, prior to intervention. Red arrows indicate marked collateral flow. The blue arrow indicates a severe narrowing of the subclavian vein.

Fig 4

Fig. 5 Following local directed thrombolysis, minimal residual thrombus with stenosis is noted at the subclavian vein (A). Angioplasty of the right subclavian vein was performed with a 12 mm × 4 cm balloon (B). Subclavian vein after angioplasty (C).

Fig 5

Fig. 6 Completion venography demonstrates in-line flow in the right brachial vein and axillary vein with near complete resolution of collateral veins.

Fig 6

Discussion

Paget Schroetter syndrome (PSS) refers to thrombosis in the axillary and subclavian veins triggered by exertion and linked to compression of the subclavian vein at the thoracic outlet. It represents the venous form of thoracic outlet syndrome (TOS), which encompasses symptoms arising from compression of the subclavian vein, subclavian artery, or brachial plexus as they traverse the thoracic outlet. This condition is often linked to vigorous and repetitive use of the upper limbs, such as wrestling, gymnastics, and swimming. These activities involve movements like retroversion, hyperabduction, and extension of the arm, which put significant strain on the axillary-subclavian vein. This strain can lead to microtrauma of the endothelium and activation of the coagulation cascade [1]. There is now significant evidence backing the influence of anatomical irregularities within the thoracic outlet, such as the presence of a cervical rib, congenital bands, hypertrophy of scalene tendons, and abnormal insertion of the costoclavicular ligament, in the development of effort thrombosis [2]. In our case, a mild prominence of the right scalene muscle without any clear evidence of external compression is observed, as shown in Fig 3. These abnormalities narrow the costoclavicular space, leading to compression of the vein and stasis of blood flow. Recurrent arm activity exacerbates this compression, causing repetitive endothelial trauma, which triggers processes like intimal hyperplasia, inflammation, and fibrosis. Over time, this can result in venous webs, extensive collateral formation, and perivenular fibrosis, worsening stasis and eventually leading to thrombus formation [2].

Paget Schroetter syndrome primarily affects young, healthy men, while commonly manifesting in the dominant arm with noticeable symptoms like swelling and discomfort [1,2]. Other signs include arm heaviness, redness, and dilated visible veins [4]. Symptoms often arise suddenly due to sports-related activities, although even minor daily tasks can trigger them [2]. Complications include pulmonary embolism and debilitating post-thrombotic syndrome as they pose significant health concerns. While some studies suggest a lower pulmonary embolism incidence compared to other deep venous thrombosis (DVT) types, the risk remains substantial [5]. In a study involving a cohort of 432 patients with confirmed upper extremity deep vein thrombosis (UEDVT), the study found that 15% of the patients developed PTS at the 2-year follow-up. PTS was defined by the presence of symptoms such as heaviness, pain, paresthesia, functional limitation, and pruritus, along with signs like edema, tenderness, skin induration, venous dilation, redness, and skin discoloration. According to this study, the presence of residual thrombosis on ultrasound following UEDVT is linked to a four-fold higher risk of developing PTS [6]. Post-thrombotic syndrome contributes significantly to morbidity as it underlies the upper extremity. Given its impact on active individuals, even minor residual disability is noteworthy.

The symptoms and signs of UEDVT lack specificity, with less than half of those with suggestive symptoms actually confirmed to have DVT. Confirmatory tests are therefore crucial following a presumptive clinical diagnosis [4]. Compression ultrasonography with color Doppler is currently the preferred initial test due to its ease, availability, portability, and cost-effectiveness in evaluating suspected UEDVT [7]. Although contrast venography has long been considered the gold standard, its invasive nature, high cost, and the reliability of noninvasive tests have now restricted its use primarily to prethrombectomy evaluations. Though not always essential for diagnosis, venography is often performed as part of a multimodal treatment approach to facilitate catheter-directed thrombolysis and plan thoracic outlet decompression surgery [4,5]. Alternative modalities such as radionuclide, magnetic resonance, and computed tomographic venography offer superior diagnostic capabilities compared to ultrasonography, with magnetic resonance venography showing the highest sensitivity (100%) and specificity (97%) [8]. However, its higher cost and limited availability pose limitations. Computed tomography venography, though widely available, carries risks associated with contrast administration. These modalities are typically reserved for patients with a high clinical probability of effort thrombosis and negative ultrasound results.

The contemporary management of effort thrombosis lacks a standardized approach due to its rarity, limited awareness, and absence of large-scale trials. Historically, conservative management with limb elevation and anticoagulation alone proved insufficient, leading to high rates of residual symptoms and recurrent thrombosis. Consequently, more aggressive treatment strategies have emerged, including systemic fibrinolysis, local catheter-directed thrombolysis, and thoracic outlet decompression (TOD) [9]. Early recognition and intervention are crucial, with thrombolysis showing decreased efficacy over time from symptom onset. For optimal outcomes, starting therapeutic anticoagulation for at least 5 days, followed by venography and catheter-directed thrombolysis within 2 weeks of symptom onset, is considered optimal as early catheter-directed thrombolysis shows success rates ranging from 75% to 84% [10]. TOD, involving first rib resection and muscle and ligament division, has become integral, either as an adjunct to thrombolysis or as primary therapy in select candidates. Debate persists over the timing and approach of TOD, with proponents of early surgery citing better outcomes in terms of symptom resolution and thrombosis recurrence. However, surgical risks, such as pneumothorax and arterial injury, necessitate careful patient selection and discussion [4]. In addition, there is no consensus regarding the optimal duration of anticoagulation for PSS. According to the 2016 CHEST Guideline and Expert Panel Report on antithrombotic therapy for VTE disease, a 3-month treatment course is recommended after any upper extremity DVT, irrespective of thrombolytic interventions [11].

Conclusion

Effort thrombosis presents a complex and relatively rare condition with a unique underlying cause. Often, physicians not well-versed in this disorder tend to treat it similarly to typical lower extremity deep vein thrombosis (DVT). However, the optimal management of effort thrombosis involves a comprehensive approach. This includes assessing the need for regular catheter-directed thrombolysis, early thoracic outlet decompression (TOD) in suitable candidates, and engagement in physical and occupational therapy. For individuals with both thrombophilia and unsatisfactory surgical outcomes, long-term anticoagulation may be considered. Raising awareness among primary care and emergency physicians is crucial for timely identification, initiation of thrombolysis, and referral to thoracic or vascular surgeons. Future research should concentrate on determining the effectiveness of thrombolytic therapy in cases of delayed presentation, identifying predictive factors for thrombolysis failure necessitating surgery, assessing the necessity and duration of anticoagulation post-TOD, and conducting cost-benefit analyses of various treatment approaches.

Patient consent

A written informed consent for the publication of this case was obtained from the patient. The patient has agreed to the sharing of relevant clinical information and images for educational and research purposes.

Acknowledgments: All authors have declared that no financial support was received from any organization for the submitted work.

Competing Interests: All authors have declared that they have no financial relationships at present or within the previous 3 years with any organizations that might have an interest in the submitted work. All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

All authors have reviewed the final version to be published and agreed to be accountable for all aspects of the work.
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References

1 Alla VM Natarajan N Kaushik M Warrier R Nair CK. Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis West J Emerg Med 11 4 2010 358 362 21079709
2 Zell L Kindermann W Marschall F Scheffler P Gross J Buchter A. Paget-Schroetter syndrome in sports activities: case study and literature review Angiology 52 5 2001 337 342 10.1177/000331970105200507 11386385
3 Hangge P Rotellini-Coltvet L Deipolyi AR Albadawi H Oklu R. Paget-Schroetter syndrome: treatment of venous thrombosis and outcomes Cardiovasc Diagn Ther 7 Suppl 3 2017 S285 S290 10.21037/cdt.2017.08.15 29399532
4 Urschel HC Patel AN. Surgery remains the most effective treatment for Paget-Schroetter syndrome: 50 years' experience [published correction appears in Ann Thorac Surg. 2008 Nov;86(5):1726] Ann Thorac Surg 86 1 2008 254 260 10.1016/j.athoracsur.2008.03.021 18573433
5 Elman EE Kahn SR. The post-thrombotic syndrome after upper extremity deep venous thrombosis in adults: a systematic review Thromb Res 117 6 2006 609 614 10.1016/j.thromres.2005.05.029 16002126
6 Prandoni P Bernardi E Marchiori A The long term clinical course of acute deep vein thrombosis of the arm: prospective cohort study BMJ 329 7464 2004 484 485 10.1136/bmj.38167.684444.3A 15256419
7 Mustafa BO Rathbun SW Whitsett TL Raskob GE. Sensitivity and specificity of ultrasonography in the diagnosis of upper extremity deep vein thrombosis: a systematic review Arch Intern Med 162 4 2002 401 404 10.1001/archinte.162.4.401 11863471
8 Chang YC Su CT Yang PC Wang TC Chiu LC Hsu JC. Magnetic resonance angiography in the diagnosis of thoracic venous obstruction J Formos Med Assoc 97 1 1998 38 43 9481063
9 Urschel HC Razzuk MA. Paget-Schroetter syndrome: what is the best management? Ann Thorac Surg 69 6 2000 1663 1669 10.1016/s0003-4975(00)01151-6 10892903
10 Moore R Wei Lum Y Venous thoracic outlet syndrome Vasc Med 20 2 2015 182 189 10.1177/1358863X14568704 25832605
11 Kearon C Akl EA Ornelas J Antithrombotic Therapy for VTE Disease: CHEST Guideline and Expert Panel Report [published correction appears in Chest. 2016;150(4):988. doi: 10.1016/j.chest.2016.08.1442] Chest 149 2 2016 315 352 10.1016/j.chest.2015.11.026 26867832
