A Case Report: A Young Waiter with Paget-Schroetter Syndrome

doi:10.1016/j.jemermed.2012.07.075

The Journal of Emergency Medicine

Volume 44, Issue 3, March 2013, Pages e291-e294

https://doi.org/10.1016/j.jemermed.2012.07.075 Get rights and content

Abstract

Background

Paget-Schroetter syndrome (PSS) is a rare presentation of primary axillary subclavian vein thrombosis that classically occurs in young men with a degree of underlying thoracic outlet syndrome after a period of upper extremity exertion. The primary complication of PSS is post-thrombotic syndrome, a result of chronic venous hypertension.

Objectives

To educate Emergency Physicians on this condition to potentiate timely diagnosis and appropriate disposition.

Case Report

A 29-year-old right-handed restaurant waiter presented with 3 days of non-painful, gradual-onset right upper extremity swelling with normal vital signs. The patient's history was otherwise notable for subjective fevers and a right forearm abrasion. Upon examination, the right upper extremity was neurovascularly intact and remarkable for uniform edema and erythema extending distally from the level of the mid-humerus. The primary differential diagnoses were deep venous thrombosis (DVT) vs. soft tissue infection. Venous phase contrast computed tomography did not reveal evidence of underlying soft tissue infection and was inconclusive regarding a DVT. Ultrasound demonstrated a right subclavian vein DVT. The patient was admitted and underwent thrombolysis, venolysis, and first rib resection and initiation of warfarin.

Conclusion

PSS is a rare presentation of upper-extremity DVT occurring classically in patients without commonly recognized pro-thrombotic risk factors. PSS carries the potential of significant morbidity in the form of post-thrombotic syndrome and pulmonary embolism. Current literature suggests that optimal outcomes are achieved when treatment is initiated within 6 weeks of onset. The treatment paradigm calls for thrombolysis and, frequently, a first rib resection.

Introduction

Paget-Schroetter syndrome (PSS) is a rare presentation of primary axillary-subclavian vein thrombosis first described independently by Paget in 1875 and von Schroetter in 1884 1, 2. In 1948, Hughes coined the name “Paget-Schroetter syndrome” 3, 4. If not recognized and treated within an appropriate timeframe, PSS carries significant morbidity, primarily in the form of post-thrombotic syndrome or pulmonary embolism. It is important that Emergency Physicians be aware of PSS to avoid a missed diagnosis and a potential poor outcome.

Section snippets

Case Report

A 29-year-old right-handed restaurant waiter presented to our Emergency Department (ED) with a complaint of gradual-onset right upper-extremity redness and swelling for 3 days that extended distally from the level of his mid-humerus. The extremity was neurovascularly intact and without pain. He reported subjective fevers and noted that several days before the onset of symptoms he had sustained a superficial scratch on his right forearm from his girlfriend. Otherwise, he denied any other

Discussion

PSS, also known as “effort thrombosis,” is a rare manifestation of primary upper-extremity deep venous thrombosis, with an incidence of approximately one to two per 100,000 persons per year 5, 6, 7, 8. PSS is characterized by upper-extremity DVT resulting from repetitive trauma to the subclavian-axillary vein and typically presents after a pattern or period of upper-extremity exertion. For this reason, it is important to elicit a thorough occupational and social history, including questions

Conclusion

PSS is a rare disease. However, it is important that Emergency Physicians include PSS in their differential diagnosis of upper-extremity swelling to ensure timely diagnosis and treatment (15). PSS carries the potential for significant morbidity, most commonly in the form of post-thrombotic syndrome, and delays in treatment of >6 weeks from onset have been shown to have worse outcomes. Furthermore, it is important that Emergency Physicians be familiar with the general treatment paradigm for PSS

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There are more references available in the full text version of this article.

Cited by (9)

Paget-Schroetter syndrome in a non athlete - a case report
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Our case is unusual as the patient was not physically active. Such non-athletic patient demographics have been also described in few other case reports (involving a waiter, a mixologist and a TV cameraman) [4,5,6]. These patients have no clear thrombotic risk factors and a negative hypercoagulability workup.
A young male waiter presented with left shoulder pain, with ultrasound showing thrombosis of the left subclavian vein. After exclusion of hypercoagulability, a dynamic MRI upper limb venogram was performed, confirming Paget-Schroetter syndrome (PSS). Our case is unusual as Paget Schroetter syndrome are seldom reported in non athletes. This is also the first report to show dynamic MRI images of PSS.
Venous Thoracic Outlet Syndrome in a Young Student
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The differential diagnosis of unilateral upper extremity swelling includes soft tissue infection, upper extremity DVT, superior vena cava (SVC) syndrome, localized allergic reaction, Pancoast tumor, and lymphedema. Emergency physicians should keep VTOS on the differential of a swollen and discolored upper extremity to avoid a missed diagnosis and a potentially increased morbidity (4). Complications of untreated VTOS include chronic venous hypertension due to outflow obstruction and valve injury, pulmonary embolism, SVC syndrome, septic thrombophlebitis, thoracic duct obstruction, and brachial plexopathy (4).
Venous thoracic outlet syndrome (VTOS) results from compression and thrombosis of the axillosubclavian vein. In primary effort thrombosis, a subtype of VTOS, chronic repetitive compression injury of the axillosubclavian vein leads to scarring, stenosis, and eventually, thrombosis. This is a rare manifestation of an upper extremity deep vein thrombosis.
A 23-year-old male student without significant past medical history presented to our Emergency Department with a complaint of intermittent swelling and discoloration of his upper right arm. His symptoms had been present for the past year and had worsened over the past few weeks. Swelling was associated with overhead use of the arm. There is no family history of clotting disorders. A computed tomography angiogram of the chest with upper extremity runoff showed findings consistent with VTOS. The patient was discharged with an urgent referral to Vascular Surgery. Within 2 weeks, he underwent multiple surgical procedures and was initiated on anticoagulation.
VTOS usually presents in patients who do not have commonly recognized prothrombotic risk factors. Emergency physicians should include this diagnosis in their differential because good functional outcomes rely on early diagnosis and prompt initiation of treatment. In addition, emergency physicians must refer these patients to vascular surgeons, as most will require surgical management.
MR Imaging of Thoracic Veins
2015, Magnetic Resonance Imaging Clinics of North America
Citation Excerpt :
Less common causes are radiation fibrosis, fibrosing mediastinitis, and postoperative strictures, which occur for example in heart transplant recipients and in patients with repaired congenital heart disease. Thrombosis of the subclavian and axillary veins associated with strenuous exercise has been described as Paget–von Schroetter syndrome and occurs in otherwise healthy individuals.22 Malignant obstruction of the SVC or brachiocephalic veins is caused by encasement or invasion by adjacent tumors, such as lung cancer (most common), sarcoma, thymoma, or metastases (see Fig. 15).
Paget-Schroetter syndrome from manual handling at work: Case report and review of the literature
2022, American Journal of Industrial Medicine
Two patients with paget-schroetter syndrome that were successfully diagnosed by doppler ultrasonography: Case studies with a literature review
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Upper extremity deep vein thrombosis in a TV cameraman
2015, Occupational Medicine

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Clinical Communications: AdultsA Case Report: A Young Waiter with Paget-Schroetter Syndrome

Abstract

Background

Objectives

Case Report

Conclusion

Introduction

Section snippets

Case Report

Discussion

Conclusion

J Formos Med Assoc

Thromb Res

Ann Thorac Surg

Clinical lectures and essays

Venous obstruction in the upper extremity

Br J Surg

Paget-Schroetter syndrome

Hong Kong Med J

Clinical Communications: Adults
A Case Report: A Young Waiter with Paget-Schroetter Syndrome