Venous obstruction of the upper extremity caused by subclavian vein valve hypertrophy

Abstract

This case report detailed a rare case of upper limb venous outflow obstruction due to primary subclavian vein valve hypertrophy in a 26-year-old male. Misdiagnosed initially, the condition was identified through advanced Doppler ultrasound and Computed Tomography Venography (CTV) Endovascular treatment using Wallstent stents effectively resolved the obstruction, preserving collateral venous circulations. This case highlights the importance of combined colour Doppler ultrasonography for diagnosing unusual venous obstructions and demonstrates the potential of endovascular treatments in managing rare venous conditions.

Venous outflow obstruction in the upper limbs is a clinically significant condition that can lead to upper limb deep vein thrombosis (DVT), accounting for only 1–2% of all DVT cases, with primary upper limb DVT or axillo-subclavian vein spontaneous thrombosis constituting ∼24% of these instances [1].

INTRODUCTION

Venous outflow obstruction in the upper limbs is a clinically significant condition that can lead to upper limb deep vein thrombosis (DVT), accounting for only 1–2% of all DVT cases, with primary upper limb DVT or axillo-subclavian vein spontaneous thrombosis constituting ∼24% of these instances [1]. This condition presents a diagnostic challenge due to its rarity and subtle symptoms. Unlike lower limb chronic venous insufficiency, which is often identified through signs observed in the Trendelenburg and Perthes tests, chronic venous insufficiency in the upper limbs frequently goes undetected [2]. While thoracic outlet syndromes are commonly considered, primary venous pathologies such as valve hypertrophy are often underestimated. This report elucidated a case of symptomatic upper limb venous outflow obstruction due to primary subclavian vein valve hypertrophy, a seldom-reported condition. It highlights the diagnostic complexities and demonstrates the effectiveness of endovascular intervention, thereby providing critical insights into the management of similar venous disorders and emphasizing the role of detailed vascular imaging and innovative endovascular stent placement.

CASE HISTORY

A 26-year-old male with a decade-long history of discomfort and swelling in the right upper limb, exacerbated in the past year, presented for evaluation. Despite earlier physiotherapy and oral administration of citrus flavonoid tablets, his condition worsened, showing no significant differences in blood pressure between limbs and no deformities in thoracic or spinal structures. A remarkable finding was noted when the patient rapidly moved his hands in a ‘washing’ motion, resulting in congestive changes and a noticeable increase in limb width and a purplish hue (Fig. 1). The upper limb Visual Analog Scale (VAS) pain score was 3 post-activity, accompanied by a sensation of heaviness. Both the Adson manoeuvre and Roos test were negative, with no history of trauma. Laboratory tests were normal. Combined colour Doppler ultrasound revealed ‘J’-shaped elongated superficial venous valves and narrowed gaps between deep venous valves in the right subclavian vein, with an increased flow speed (Video 1 and Fig. 2A and B). CT venography showed no narrowing or abnormality in the right subclavian vein and no anomalous cervical ribs (Fig. 2C). Subsequent right brachial and subclavian venography under local anaesthesia revealed significant reflux in the right subclavian vein and its branches (Fig. 2D). The hypertrophied valve of the subclavian vein was treated using overlapping Wallstent stents (1260 mm, 1290 mm), with a 12-mm balloon dilation performed at the site of subclavian vein stenosis before and after stent placement. Follow-up angiography confirmed unobstructed blood flow without reflux, and postoperative symptoms including upper limb heaviness resolved (Video 2 and Fig. 2E). Rivaroxaban (20 mg qd) was prescribed, and the patient was regularly followed up for 3 months without complaints.

Figure 1:

The right upper limb showed congestive changes with increased width compared to the opposite side. These changes persisted even after continuous fist-pumping actions.

Figure 2:

(A) Initial segment of the right subclavian vein showing elongated ‘J’-shaped superficial venous valves and pronounced spontaneous echogenicity of red blood cells dorsally. (B) Spectral display indicating a narrowed gap between deep venous valves with an accelerated flow speed of 217.2 cm/s. (C) No external stenosis observed in the right subclavian vein (indicated by arrow). (D) Significant reflux in the right subclavian vein and its branches, with tortuous, dilated communications with the internal jugular vein. A localized narrowing was seen just before the subclavian vein joined the brachiocephalic vein. (E) Post-stent implantation, blood flow in the right brachial and subclavian veins was unobstructed, with no reflux, and previously tortuous branches were no longer visible.

DISCUSSION

This case report illuminated a rare instance of upper limb venous outflow obstruction due to subclavian vein valve hypertrophy, a condition 1st noted over 50 years ago with limited subsequent reports and lacking duplex ultrasound documentation [3]. Current research emphasizes the importance of combined colour Doppler ultrasound in diagnosing venous obstructions, offering higher sensitivity than conventional ultrasound or CT venography, while compression US has optimal specificity for deep vein thrombosis (DVT) [4, 5]. However, because the subclavian vein is covered by the clavicle, it is challenging to apply direct pressure to diagnose subclavian vein thrombosis. Therefore, we used colour Doppler ultrasound, utilizing the clavicle and pleura as anatomical landmarks, to image the subclavian vein and identify any venous obstruction. Intravascular ultrasound has also shown higher sensitivity in detecting upper limb deep venous valve diseases [5].

Differentiation from ‘Venous Thoracic Outlet Syndrome’, with an incidence of 1–2/100 000, predominantly in young, active individuals like athletes and painters, was crucial [4]. The primary mechanism of this condition involves external compression of the subclavian vein, such as from the space between the clavicle and the 1st rib, or compression by the surrounding muscles of the clavicle, leading to upper limb circulatory obstruction and even upper limb DVT, and the surgical interventions for it included catheter-directed thrombolysis and thoracic outlet decompression procedures, such as 1st rib resection surgery [5]. Unlike previous treatments like rib resection, which do not benefit valve diseases, and considering the efficacy of Wallstent in superior vena cava syndrome, our approach innovatively used Wallstent stents for treatment, avoiding the risk of DVT recurrence due to endothelial damage from valve excision or repair. Primary upper limb venous obstruction diseases, particularly rare malformed subclavian vein valve, are often misdiagnosed. High-quality Doppler ultrasound plays a crucial role in accurate diagnosis, and venography is essential for treatment planning. In this case, we successfully employed a venous stent for the obstruction caused by valve hypertrophy, providing a valuable precedent for future treatment of similar conditions.

FUNDING

None declared.

Conflict of interest: none declared.

DATA AVAILABILITY

The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.

ETHICAL APPROVAL AND CONSENT TO PARTICIPATE

The study protocol conformed to the ethical guidelines of the 1975 Declaration of Helsinki, with approval granted by the Human Research Review Committee at West China Hospital, Sichuan University (Chengdu, China). The patient provided written informed consent.