The diagnosis and management of Paget-Schroetter syndrome (primary effort thrombosis) in the emergency department: A case report : Medicine: Case Reports and Study Protocols

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Clinical Case Report

The diagnosis and management of Paget-Schroetter syndrome (primary effort thrombosis) in the emergency department

A case report

Jacob, Vinitha MD, PhDa,b,∗; Daley, James MDb; Coughlin, Ryan F. MDb

Author Information
Medicine Case Reports and Study Protocols: June 2021 - Volume 2 - Issue 6 - p e0106
doi: 10.1097/MD9.0000000000000106
  • Open

Abstract

1 Introduction

Primary effort thrombosis or Paget-Schroetter syndrome is relatively rare, making its diagnosis particularly challenging in the emergency department. This syndrome refers to a type of venous thrombosis that occurs in the subclavian vein, typically produced by repetitive use of the affected extremity.[1] Importantly, management of this syndrome is markedly different from that of the much more frequently encountered lower extremity deep venous thrombosis (DVT). This article will present a “classic” case of an uncommon diagnosis, review the literature, and provide practice guidance for emergency medical management.

2 Case presentation

A 40-year-old right-hand-dominant male bodybuilder presented for evaluation of right upper extremity swelling. The patient was in his usual state of health until three days before presentation, when he noted diffuse right upper extremity edema and plethora after working out at the gym. He denied cough, chest pain, shortness of breath, trauma, or any other symptoms in other extremities. His medical and social history was significant for regular injection use of testosterone as well as an active 30 pack-year smoking history. He denied any illicit intravenous drug use. The rest of his medical and surgical histories were non-contributory. Vital signs were within normal limits. Physical examination revealed a well appearing male in no acute distress with hypertrophied musculature, particularly in the upper extremity, shoulder, and trapezius muscles consistent with his history of bodybuilding. There was rubor and edema of his right upper extremity, with intact distal pulses, motor function, and sensation. His fingers were warm and well perfused with normal capillary refill. There was full passive and active range of motion. Dilated veins around the shoulder (Urschel's sign) were not observed. No facial plethora nor swelling was noted.

The differential diagnoses included DVT, superficial thrombophlebitis, muscle tear, superior vena cava (SVC) syndrome, and thoracic outlet obstruction e.g. due to Pancoast tumor. Based on the history and physical exam, we felt that cellulitis, arterial thromboembolism, and necrotizing fasciitis were unlikely to be present.

A complete blood count and basic metabolic panel were obtained and were within normal limits. Total creatine kinase was elevated at 352 U/L (Reference range: 11-204 U/L). A duplex venous ultrasound of the right upper extremity was then obtained, which showed an occlusive thrombus in the medial and mid subclavian vein (Fig. 1). A nonocclusive thrombus was identified in the right lateral subclavian vein. No demonstrable thrombus was found in the internal jugular, axillary or brachial vein. A chest X-ray was normal; no cervical rib was noted.

F1
Figure 1:
Ultrasound image shows a thrombus in the right subclavian vein (upper half of image, box). No flow is appreciated on doppler ultrasound (bottom half of image).

Upon discovery of a DVT of the right upper extremity, a heparin drip was started, and the vascular surgery service was consulted from the emergency department. Shortly thereafter, the patient was consented for a venogram, admitted to the vascular surgery step-down unit for thrombolysis and first rib resection.

After admission to the vascular surgery step down unit, the patient underwent successful catheter directed thrombolysis. He returned to the operating room the following day for termination of thrombolysis and venoplasty of the right subclavian vein. A follow up venogram performed in the operating room demonstrated patency of his right subclavian vein. The patient opted to forgo first rib resection, which was recommended by the vascular team. Neurovascular checks were intact and improved throughout his hospital course. He was discharged home with improved symptoms on oral rivaroxaban on hospital day 2. He was counseled on smoking cessation as well as on stopping heavy lifting for the next few months. He was advised that a recurrence of DVT was almost certain.

No evidence of thrombosis was found during the patient's last known follow up ultrasound which was performed slightly over a year after his initial presentation. At his last known follow up visit at approximately the same time, the patient reported that he had been doing physical therapy exercises which had been helping him. He had also returned to lifting weights. He could go several weeks without feeling any symptoms and then took rest if he felt “tightness” in his arm. He continued to take oral rivaroxaban and had not experienced any symptoms as similar or as intense as when he first presented with primary effort thrombosis.

3 Discussion

Primary effort thrombosis or Paget-Schroetter syndrome is rare, with an annual incidence between 1 and 2 per 100,000 people, even at regional referral centers.[1] The mean age at presentation is in the 30 s, and the male-to-female predominance is 2:1.[1] The dominant arm is more likely to be affected.[1,2] As in the above case, the “classic” presentation of Paget-Schroetter syndrome, therefore, is that of a young, athletic male who reports pain and swelling of the upper extremity after a period of repetitive activity. Repetitive activity results in inflammation and fibrosis surrounding the subclavian vein, which passes next to the intersection of the clavicle and first rib.[1,3] The vein thus becomes less mobile relative to the surrounding structures.[1] Congenital abnormalities such as the presence of a cervical rib, as well as acquired abnormalities such as hypertrophied subclavius muscles due to repetitive lifting in this case, is also thought to contribute to the development of this syndrome.

Primary effort thrombosis is a subtype of venous thoracic outlet syndrome (TOS). TOS can be subdivided into three distinct categories: neurogenic (involving the brachial plexus), venous (involving the subclavian vein), and arterial (involving the subclavian artery).[1] Neurogenic TOS is by far the most common, representing approximately 95% of cases of TOS.[4] This variant of TOS can manifest as thenar wasting along with paresthesia of the fingers and hand. Tumors of the lung apex, such as Pancoast tumor, can cause brachial plexus compression and present in this manner. Venous TOS accounts for 3–5% of cases, while arterial TOS accounts for 1% to 2%.[4] TOS of the blood vessels presents with varying degrees of pain and swelling; arterial TOS can additionally cause weak or absent pulses. When the clot burden is extensive, the SVC is compressed which results in facial swelling, plethora, and jugular venous distention (SVC syndrome). Venous thoracic outlet syndromes are further divided into three separate categories: positional venous obstruction, secondary subclavian vein thrombosis (catheter or dialysis associated) and primary effort thrombosis, the subject of this report. Secondary subclavian vein thrombosis accounts for the majority of cases of venous TOS.

Diagnosis of effort thrombosis is typically done with duplex venous ultrasound, which is non-invasive and has reasonable sensitivity (78%–100%) and specificity (82%–100%).[1,5] There have been recent reports that demonstrate that emergency-physician-performed bedside ultrasound can be effective at detecting upper extremity thrombus.[3,6] When ultrasound is inconclusive and when a high clinical index of suspicion remains, computed tomography venography or magnetic resonance venography can also be employed which have higher sensitivity/specificity. Venography, though invasive, is the gold standard in diagnosis.[2]

Similar to lower extremity DVT, treatment of effort thrombosis generally involves the immediate initiation of anticoagulation. However, morbidity with anticoagulation alone is significant and can involve post thrombotic syndrome (chronic venous insufficiency) or pulmonary embolism.[7,8] One study showed that only one out of six patients treated with anticoagulation alone experienced resolution of the thrombus.[9] Furthermore, acute pulmonary embolism occurs in a significant percentage of cases treated with anticoagulation alone.[1,10] Thus, catheter directed thrombolysis is now part of the initial array of treatment options offered to the patient. The success of this approach is nearly 100% if initiated within days of symptom onset but diminishes the longer the symptoms persist.[11] One study reported that the success rate of thrombolysis was just 29% if started 2–12 weeks after symptom onset.[11,12] Given the significant deterioration in efficacy of thrombolysis over time, it is crucial that effort thrombosis is recognized at the initial presentation.

Even with anticoagulation and thrombolysis, re-thrombosis can occur in as many as a third of patients.[12] Due to the high risk of re-thrombosis, it is standard practice that definitive treatment of Paget-Schroetter disease involves decompression surgery, although there have not been any randomized controlled trials.[13] Definitive management generally involves first rib resection in those without other anatomic abnormalities. Patients who were aggressively treated with this approach (thrombolysis and decompressive surgery) reported quicker resolution of symptoms and return to work than those treated with anticoagulation alone.[12,14] The duration of anticoagulation and even the need for anticoagulation itself is debated among those treated with successful thrombolysis and decompressive surgery. Due to the importance of surgical intervention, it is important that vascular and/or other ancillary services are consulted from the emergency department.

4 Conclusion

Paget-Schroetter syndrome is rare and classically occurs in young, male, otherwise healthy athletic patients. Unlike the treatment of lower extremity DVT, management of Paget-Schroetter syndrome involves thrombolysis and likely decompressive surgery in addition to anticoagulation. Since treatment is time sensitive and because the therapeutic options are distinct from lower extremity DVT, it is critical that the emergency physician be familiar with the treatment algorithm for this particular entity and involve the appropriate specialists in an expeditious manner.

Author contributions

Conceptualization: Vinitha Jacob, Ryan Coughlin.

Data curation: Vinitha Jacob.

Supervision: Vinitha Jacob, Ryan Coughlin.

Writing – original draft: Vinitha Jacob.

Writing – review & editing: Vinitha Jacob, Ryan Coughlin, James Daley.

References

[1]. Illig KA, Doyle AJ. A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg 2010;51:1538–47.
[2]. Hughes MJ, D’Agostino JC. Upper extremity deep venous thrombosis: a case report and review of current diagnostic/therapeutic modalities. Am J Emerg Med 1994;12:631–5.
[3]. Norinsky AB, Espinosa J, Kianmajd M, et al. Painless acrocyanosis: Paget-Schroetter syndrome secondary to thoracic outlet obstruction from muscle hypertrophy. Am J Emerg Med 2016;34:1323.
[4]. Jones MR, Prabhakar A, Viswanath O, et al. Thoracic outlet syndrome: a comprehensive review of pathophysiology, diagnosis, and treatment. Pain Ther 2019;8:5–18.
[5]. Chin EE, Zimmerman PT, Grant EG. Sonographic evaluation of upper extremity deep venous thrombosis. J Ultrasound Med 2005;24:829–38.
[6]. Weaver LA, Kanter CR, Costantino TG. Effort thrombosis provoked by saxophone performance. J Emerg Med 2019;56:323–6.
[7]. Alla VM, Natarajan N, Kaushik M, et al. Paget-schroetter syndrome: review of pathogenesis and treatment of effort thrombosis. West J Emerg Med 2010;11:358–62.
[8]. Drakos N, Gausche-Hill M. A case report: a young waiter with Paget-Schroetter syndrome. J Emerg Med 2013;44:e291–4.
[9]. AbuRahma AF, Sadler D, Stuart P, et al. Conventional versus therapy in spontaneous (effort) axillary-subclavian veinthrombosis. Am J Surg 1991;161:459–65.
[10]. Hingorani A, Ascher E, Lorenson E, et al. Upper extremity deep venous thrombosis and its impact on morbidity and mortality rates in a hospital-based population. J Vasc Surg 1997;26:853–60.
[11]. Molina JE, Hunter DW, Dietz CA. Paget-Schroetter syndrome treated with thrombolytics and immediate surgery. J Vasc Surg 2007;45:328–34.
[12]. Hangge P, Rotellini-Coltvet L, Deipolyi AR, et al. Paget-Schroetter syndrome: treatment of venous thrombosis and outcomes. Cardiovasc Diagn Ther 2017;7 (Suppl 3):S285–90.
[13]. Naeem M, Soares G, Ahn S, et al. Paget-Schroetter syndrome: a review and Algorithm (WASPS-IR). Phlebology 2015;30:675–86.
[14]. Urschel HC, Razzuk MA. Paget-Schroetter syndrome: what is the best management? Ann Thorac Surg 2000;69:1663–8.
Keywords:

deep venous thrombosis; emergency medicine providers; paget-schroetter syndrome; subclavian vein; thoracic outlet syndrome

Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.