Introduction
A lateral neck mass is a common presenting complaint in adults, with numerous possible causes including metastatic squamous cell carcinoma, lymphoma, branchial cyst, haemangioma, sebaceous cyst, lipoma, carotid body tumour and parotid neoplasm.Reference Smith, Ellis, Bearcroft, Berman, Grant and Jani1, Reference Glosser, Pires and Feinberg2
Below, we describe a case of a painful neck swelling and laryngo-pharyngeal oedema due to an internal jugular vein (IJV) thrombosis secondary to a cardiac pacemaker.
Case report
A 67-year-old woman presented to our ENT department with a left-sided, enlarging neck swelling of three days' duration. She had dysphagia due to worsening pain, but no dyspnoea. A few days before presentation, she had developed a sore throat and dysphonia.
Neck examination revealed a 4 × 2 cm, diffuse, tender swelling in the left anterior triangle.
On flexible nasoendoscopy, a diffuse, oedematous swelling of the lateral pharyngeal wall was noted, with oedema of the ipsilateral larynx.
The patient was pyrexial with an elevated C-reactive protein (392.3 mg/l).
An initial diagnosis of lymphadenitis or abscess was made, and intravenous antibiotics and steroids were commenced.
After 48 hours of treatment, there had been no significant change in symptoms or signs, so an ultrasound scan of the neck (Figure 1) and a computed tomography (CT) scan with contrast (Figure 2) were performed.
Fig. 1 Ultrasound scan of the left side of the neck, demonstrating the thrombus.
Fig. 2 Coronal computed tomography scan with intravenous contrast, demonstrating the pacemaker, thrombosed innominate vein and collateral vein. SVC = Superior Vena Cava
The ultrasound scan (Figure 1) demonstrated a thrombus in the IJV. On the CT scan, the left innominate vein failed to opacify around the pacemaker wires after left antecubital contrast injection. The patient had had a dual chamber cardiac pacemaker inserted into the subclavian vein via a left infraclavicular approach two years previously, as treatment for heart block. The thrombosis originated around the pacemaker wires and completely occluded the left innominate vein, up to the insertion of the pacemaker in the first part of the left subclavian vein. Collaterals had developed from the first part of the subclavian vein across the mediastinum into the superior vena cava, which was of normal calibre. The left axillary vein was not involved, nor were the contralateral subclavian or internal jugular veins.
Results for antithrombin III, protein S, protein C, activated partial thromboplastin time and international normalised ratio testing were all normal.
Intravenous heparin was initiated, resulting in resolution of the neck lump within five days and improvement in the patient's symptoms, allowing her to eat and drink without discomfort.
The patient was commenced on long term warfarin. Six months later, she had experienced no further sequelae.
Discussion
Internal jugular vein thrombosis has previously been described as a cause of lateral neck swelling.Reference Albertyn and Alcock3–Reference Hadijihannas, Kesse and d'E Meredith6 No study to date has identified what proportion of neck swellings are due to this pathology, presumably due to its low incidence. Internal jugular vein thrombosis has been associated with jugular venous catheters,Reference Kroger, Gocke, Schelo, Hinrichs and Rudofsky7 surgery,Reference Leontsinis, Currie and Mannell8 intravenous drug abuse,Reference Chowdhury, Bloom, Black and al-Noury9 neoplasms of the head and neck,Reference Wing and Scheible10 infection,Reference De Sena, Rosenfeld, Santos and Keller11 parotitis,Reference Hadijihannas, Kesse and d'E Meredith6 and hypercoagulable states;Reference Martinelli, Cattaneo, Panzeri, Taioli and Mannucci12 it may also occur spontaneously. Alternatively, it may be identified following investigation of its associated complicationsReference Ascher, Salles-Cunha and Hingorani13 or during investigation of subclavian vein thrombosis.Reference Kroger, Gocke, Schelo, Hinrichs and Rudofsky7 The current report draws attention to the rare possibility of a chronic subclavian vein thrombus developing around pacemaker wires and then propagating into the IJV and presenting acutely as a lateral neck swelling, without the complications or clinical evidence associated with a subclavian vein thrombosis.
After searching the Pubmed database, we believe only two other reports have described an IJV thrombosis causing a neck swelling following catheterisation of the subclavian vein for cardiac pacing.
Fitzgerald and LeckieReference Fitzgerald and Leckie4 described a case of IJV thrombosis presenting as a tender neck mass contralateral to the site of cardiac pacemaker placement. This patient was treated with thrombolytics and anticoagulation. In contrast, our patient's symptoms resolved fully with only anticoagulation and antibiotics.
Girard et al. Reference Girard, Reuler, Mayer, Nardone and Jendrzejewski5 described the occurrence of a cerebral venous sinus thrombosis after partial occlusion of the right IJV by a clot originating in the right subclavian vein. This led to a reduction in consciousness. In contrast, our patient had full occlusion of the left IJV, without any evidence of cerebral venous sinus thrombosis, and maintained full consciousness.
Our patient's CT scan with contrast (Figure 2) demonstrated a functioning venous collateral system; this prevented earlier presentation with arm oedema, the usual complaint associated with subclavian vein thrombosis. Such a compensatory mechanism has previously been described following cardiac pacemaker placement.Reference Stoney, Addlestone, Alford, Burrus, Frist and Thomas14
A derangement of any one element of Virchow's triad of hypercoagulability, haemodynamic changes (such as stasis or turbulence) and endothelial injury can promote thrombosis. Lemierre syndrome comprises an infected IJV thrombus due to extension of oropharyngeal infection.Reference Lemierre15 Thrombosis due to such infection is probably due to systemic hypercoaguability, venous stasis caused by inflammatory occlusion of the vessel, and endothelial damage by micro-organisms. Certainly, our patient had originally developed a sore throat. However, it is conceivable that endothelial damage could have occurred in this patient due to the long-standing presence of pacemaker wires, which themselves could have acted as a nidus for thrombus formation.Reference Hubsch, Stiglbauer and Schwaighofer16
Transvenous cardiac pacemakers are the preferred method of cardiac pacing, due to their relative ease of insertion and relatively low incidence of complications.Reference Eltrafi, Currie and Silas17 Previous studies have reported that 1.2 per cent of cardiac pacemaker insertions are complicated by some degree of symptomatic subclavian vein occlusion.Reference Lee and Chaux18 This incidence rises to between 4418 and 79 per centReference Hubsch, Stiglbauer and Schwaighofer16 when one takes into consideration subclinical thromboses evident only on radiological imaging.
It is important to recognise and treat IJV thrombosis early, in order to prevent such complications as pulmonary embolism (encountered in 0.5 per cent of patients with solely IJV thrombosis),Reference Ascher, Salles-Cunha and Hingorani13 intracranial thrombosis,Reference Girard, Reuler, Mayer, Nardone and Jendrzejewski5 septic emboli and loss of vision.Reference Gutteridge, Royle and Cockburn19 Such complications, although serious, are surprisingly rare and did not occur in our patient.
• This paper describes an unusual case of lateral neck swelling due to an internal jugular vein thrombus originating from a thrombus around cardiac pacemaker wires in the subclavian vein
• Internal jugular vein thrombosis has been associated with jugular venous catheters, surgery, intravenous drug abuse, neoplasms of the head and neck, infection, parotitis, and hypercoagulable states; it may also occur spontaneously
• The patient was treated with heparin and commenced on long term warfarin
• Clinicians should be wary of this rare cause of lateral neck swelling in patients with a cardiac pacemaker, in order to expedite the necessary investigations and treatment and help prevent potentially serious complications
First line treatment for upper extremity venous thrombosis is anticoagulation, initially with heparin and subsequently with long term warfarinisation.Reference Ascher, Salles-Cunha and Hingorani13, Reference Prescott and Tikoff20, Reference Sakakibara, Shigeta, Ishikawa, Hiramatsu, Jikuya and Onizuka21 Depending on the degree of occlusion, the patient's symptoms, the veins involved and the cause of the thrombosis, alternate treatment options are available, such as thrombolysisReference Fitzgerald and Leckie4 and superior vena cava filters.Reference Ascher, Salles-Cunha and Hingorani13 Our patient's symptoms resolved with heparinisation alone, and she was subsequently placed on long term warfarin.
When considering the differential diagnosis of a neck swelling, commoner causes than IJV thrombosis should be considered. However, as this report demonstrates, the clinician should be aware of this pathology in a patient with a cardiac pacemaker who presents with a lateral neck swelling. This will expedite the necessary investigations and treatment, and help prevent potentially serious complications.
