Urschel’s Sign in Paget-Schroetter Syndrome: Multimodality Evaluation by Extravascular and Intravascular Imaging

J INVASIVE CARDIOL 2020;32(2):E47-E48.

Key words: multimodality imaging, Paget-Schroetter syndrome, Urschel’s sign

A 52-year-old woman, employed as a janitor, presented with progressive painful swelling of the right upper limb and chest wall, particularly after exertion. Physical examination revealed Urschel’s sign with prominent superficial veins over the right shoulder and chest wall (Figure 1A).Venous duplex ultrasound suggested occlusion of the right subclavian vein, which was confirmed by invasive venogram (Figure 1B). Peripheral intravascular ultrasound (Vision PV 0.35, Philips Volcano) revealed fibrotic scarring of the occluded vein without extrinsic compression at the level of the right subclavian vein and possible thrombus extension into the right atrium (Figures 1C-1E). The intravascular findings suggested Paget-Schroetter syndrome.

Venoplasty was abandoned in view of significant thrombus extension. Contrast computed tomography (CT) venography confirmed total occlusion of the right subclavian vein with thrombus extension to the cavo-atrial junction (Figure 2). There was no obvious extrinsic compression at the level of the right subclavian vein, which was bordered by the first rib, clavicle, and the subclavius muscle/costoclavicular ligament. Vascular and Thoracic Surgery was consulted for venous bypass and clot evacuation, but declined due to high risk of surgery and recurrence. The patient was treated with oral anticoagulation and sleeve compression therapy. She could manage less physically demanding duties 2 months later. Subsequent thrombophilia work-up was unremarkable.

Clinical presentation of Paget-Schroetter syndrome varies from asymptomatic to symptoms of superior vena cava obstruction. It results from repeated compression injury to the subclavian vein by adjacent structures. Additionally, thrombophilia has been identified as a risk factor predisposing to thrombosis. In this case, the presentation with Urschel’s sign is uncommon, but potentially life-threatening. CT venography or magnetic resonance venography could offer higher anatomic detail of the adjacent structures, yet extrinsic compression could be absent in some cases. Intravascular ultrasound could be considered for better intravascular evaluation of chronic venous scarring, which could trigger the thrombosis, leading to Paget-Schroetter syndrome. Multidisciplinary imaging with extravascular and intravascular evaluation could guide proper diagnosis and treatment planning.

From the Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Hong Kong Special Administrative Region, People’s Republic of China.

Disclosure: The authors have completed and returned the ICMJE Form for Disclosure of Potential Conflicts of Interest. The authors report no conflicts of interest regarding the content herein.

The authors report that patient consent was provided for publication of the images used herein.

Manuscript accepted April 1, 2019.

Address for correspondence: Wai Kin Chi, MBChB, Division of Cardiology, Department of Medicine and Therapeutics, The Chinese University of Hong Kong, 9/F Clinical Science Building, Prince of Wales Hospital, 30-32 Ngan Shing Street, Shatin, NT, Hong Kong SAR, China. Email: chiwaikin@gmail.com