External jugular venous aneurysm: A rare case report

Abstract

Venous aneurysms of the external jugular vein are rare vascular anomalies due to the low pressure in the venous circulation i.e. the superior vena cava. A review of the literature reveals that external jugular vein aneurysms are reported rarely but the exact incidence of such aneurysms is not known. The presentation of external jugular vein aneurysms can vary widely but generally, they present as a soft cervical mass which gradually increases in size. These aneurysms pose a diagnostic and management challenge for the clinician as their clinical spectrum ranges from being asymptomatic, to cosmetic concerns or they can potentially cause thrombosis, embolism, or thrombophlebitis. Diagnosis is confirmed via Color Doppler ultrasound and computerized tomogram angiography. We present the clinical features and diagnosis of a saccular external jugular vein pseudoaneurysm in a 58-year-old female, which was most likely to be congenital and was diagnosed using computerized tomogram angiography.

Introduction

An external jugular vein aneurysm is a rare vascular abnormality of a major superficial vessel in the neck and is characterized by abnormal dilation or bulging of the external jugular vein. Because of the low pressure in the venous circulation, an aneurysm in the external jugular vein is rare and very few cases have been reported in the literature [1]. However, despite its infrequency, external jugular venous aneurysms always require attention due to their potential clinical significance. Such aneurysms can be either clinically asymptomatic and raise only cosmetic concerns or they may cause serious complications such as thrombosis within the lumen of the external jugular vein leading to pulmonary embolism or rupture of the aneurysm [2]. The diagnosis of an EJV involves a combination of clinical evaluation and imaging studies. A thorough physical examination by a clinician is generally the initial step and the presence of a pulsatile and palpable mass may raise suspicion of an aneurysm. Further investigation can be done using imaging studies which include CT scan, MRI and USG which can provide information about the anatomy, size, and location of the aneurysm. Diagnosis is confirmed using computerized tomogram angiography which provides precise information about the blood flow dynamics and vascular anatomy and involves the injection of contrast into the bloodstream and then the visualization of vessels in real-time. We report the case of a 58-year-old female who presented with swelling on the lateral aspect of the neck since childhood and the diagnosis of a congenital, saccular external jugular vein aneurysm was confirmed using computerized tomogram angiography (CTA). The relevant literature and diagnostic modalities have also been reviewed.

Case discussion

A 58-year-old female with no specific medical history came to our clinic with swelling on the right lateral aspect of the neck which had been present since childhood. The swelling had progressively increased in size over the last year with significant prominence in the last 2 months. It was associated with mild discomfort and episodes of dizziness off and on. There was no complaint of pain, dysphagia, or alteration in voice. She could not recall any relevant trauma to the neck region. There was no history of any relevant surgery or medical intervention in this area.

On physical examination, the swelling was in the right anterolateral aspect of the neck and was soft, compressible, nontender, and nonpulsatile. The overlying skin showed no discoloration, thickening, or ulceration. There was a slight increase in size on coughing and the Valsalva maneuver.

Color Doppler Ultrasound of the neck was performed by GE Voluson E6 Ultrasound machine & linear probe 11 L which revealed the true lumen of the external jugular vein of about 25 × 11 mm with a defect of about 1.8 mm causing aneurysm measuring about 26 × 3.6 mm at right anterior lateral side (Fig. 1). On Color Doppler, bidirectional flow due to the swirling of blood within true/false aneurysm giving a yin-yang sign was noted. A thrombus of around 03 mm was also noted in the pseudoaneurysm, which can alter the hemodynamics within the psuedoanuerysm and despite being small in size can dislodge and travel through the venous system. No other venous channels were seen communicating with the lesion. The flow pattern and the morphology of the right common carotid artery were normal.

Fig. 1.

Color Doppler ultrasound showing an aneurysm in the external jugular vein.

CT angiography was performed on a Toshiba Aquilion 128 slice machine. On the venous phase of CT angiography (CTA), a saccular aneurysm was visualized arising from the lower part of the lateral wall of the external jugular vein measuring around 3.4 × 1.9 cm (Fig. 2). An eccentric mural thrombus was also visualized along the upper pole of the aneurysm with a maximum thickness of around 2.5 × 1.8 cm. No arterial feeder was visualized and the rest of the arteries and veins were normally visualized and a diagnosis of external jugular vein pseudoaneurysm was confirmed.

Fig. 2.

Computerized tomogram angiography showing a saccular aneurysm arising from the external jugular vein.

Discussion

Venous aneurysms of the neck are rare as compared to arterial aneurysms due to low intravascular pressure [3]. Aneurysms of the external jugular vein can be fusiform or saccular in shape. Venous aneurysms can be of primary or secondary aetiology. Primary or congenital venous aneurysms are usually fusiform aneurysms on the right side of the neck and are true venous aneurysms because in these lesions wall is intact and these lesions are frequently diagnosed in childhood [4]. Acquired aneurysms are usually saccular and mostly present as a painless swelling. Acquired or secondary causes of venous aneurysms include trauma, chronic inflammation, degeneration, and increased pressure. Thrombosis within the aneurysm can produce pain in the swelling and symptoms secondary to the pressure effect on surrounding structures [5]. In our case, the aneurysm was saccular and was likely congenital as it was noted since childhood and no secondary cause has been documented. The characteristics of aneurysm i.e: presence of a defect in the lateral wall and presence of thrombus suggested the formation of pseudoaneurysm.

External jugular vein aneurysm usually presents as a small rounded soft painless swelling in the neck that usually gradually increases in size. If neck swelling is unilateral, nontender, and nonpulsatile that enlarges with sneezing, straining, or Valsalva manoeuvre, a venous aneurysm should be included in the differential diagnosis [6]. Differentials to consider with a lateral neck mass involve several etiologies, including tumors and cysts of the superior mediastinum, external laryngeal diverticula, lung cupola inflation, and jugular vein aneurysm [5]. Venous aneurysms are rarely included in the differentials of lateral neck masses, especially when there is no reported history of neck trauma. External jugular vein aneurysms are difficult to differentiate from internal jugular vein aneurysms by clinical assessment.

For cervical lesions, ultrasound is usually the first modality for diagnosis. Doppler US is considered the gold standard and is recommended as the first imaging technique to establish the diagnosis [7]. It can exclude vascular and nonvascular causes. CT angiography determines the exact nature, location, presence, or absence of thrombosis and the relation of other vessels with the diagnosis of the external jugular aneurysm. Magnetic resonance angiography and venography can also be used for diagnosis.

Expectant management of asymptomatic aneurysms is usually advised with regular follow-up because there are no significant associated complications like rupture, or mass effect [8,9]. However surgical or endovascular management is usually done for aesthetic or cosmetic reasons as it can prevent rare complications like thrombosis, thrombophlebitis, pulmonary thromboembolism, and rupture [8].

Conclusion

External jugular vein aneurysm should be considered in differentials of slowly growing cervical masses. Ultrasound can identify the vascular nature and further confirmation of diagnosis can be achieved by CT and MR angiography, which can also help in the planning of management. Management can be achieved via endovascular and surgical approaches and is needed in symptomatic cases and for aesthetic purposes.

Patient consent

The patient provided written, informed consent for the publication of their case.