Internal Jugular Vein Thrombosis

Etiology

The internal jugular vein (IJV) originates at the jugular foramen. The IJV descends through the lateral neck to join the subclavian vein, forming the brachiocephalic vein, which ultimately drains into the superior vena cava. The IJV lies within the carotid sheath alongside the carotid artery and vagus nerve, typically coursing lateral to the carotid artery and deep to the sternocleidomastoid muscle. Anatomic variation is common, with the IJV most often positioned lateral or anterolateral to the carotid artery, though it may be located medially less commonly.[2]  

Internal jugular vein thrombosis can be caused by several factors, with provoked causes being the most common. The internal jugular vein is frequently used for central venous catheterization because of its ease of access and identification with ultrasonography or anatomic landmarks. Dialysis catheters, pacemakers, and chemotherapy ports are frequently placed in or traverse the internal jugular vein and serve as a nidus for thrombus formation.[3][4] Long-term use of central venous catheters and port sites is associated with a higher risk of internal jugular vein thrombosis. Among patients in the intensive care unit, internal jugular vein thrombosis was associated with a platelet count greater than 200,000/μL, platelet transfusion, fresh-frozen plasma transfusion, vasopressor use, mechanical ventilation support, and a central line in place for more than 14 days.[5]

Other less common causes of IJVT include malignant neoplasms, surgical dissection, trauma, local infections, paraneoplastic disease, and ovarian hyperstimulation syndrome. Hypercoagulability can also cause IJVT and includes conditions such as factor V Leiden mutation, protein C deficiency, protein S deficiency, and antithrombin deficiency.[4] Adjacent ear, nose, and throat infections can cause venous inflammation, leading to IJVT. An example is Lemierre syndrome, an oropharyngeal infection that causes septic thrombophlebitis of the internal jugular vein. The most common causative bacterium in Lemierre syndrome is Fusobacterium necrophorum, which translocates to the internal jugular vein, causing thrombus formation.[6] 

Epidemiology

Upper extremity deep vein thrombosis (DVT) in general accounts for 10% of all DVTs.[4] Rates of isolated internal jugular vein DVT range from 0.5% to 3.47%.[7] 

Pathophysiology

The same disease process that causes deep vein thrombosis throughout the body underlies internal jugular vein thrombosis. Thrombosis occurs when there is vascular wall injury, stasis of blood, and changes in blood consistency that enhance coagulation. These 3 conditions together are known as the Virchow triad. Endothelial injury activates the coagulation cascade; platelet adhesion and activation then lead to the formation of a platelet plug. The platelet plug triggers activation of coagulation proteins, leading to thrombin formation. Thrombin further activates platelets and fibrinogen, triggering fibrin clot formation according to Rutherford's Vascular Surgery and Endovascular Therapy. 9th ed. 

History and Physical

Internal jugular vein thrombosis is asymptomatic in many patients and may be difficult to diagnose because of its often subtle presentation. When symptoms are present, they may resemble those of deep vein thrombosis and include neck pain, swelling, erythema, and tenderness. Patients may also report a headache or a sensation of fullness in the neck.[4]

A thorough history should assess for risk factors, including recent central venous catheter placement, malignant neoplasms, hypercoagulable states, intravenous drug use, recent surgical procedures or trauma, and recent oropharyngeal infections. On physical examination, findings may include unilateral neck swelling, tenderness, erythema, or a palpable cord along the angle of the mandible or the anterior border of the sternocleidomastoid muscle.[8] Infectious internal jugular vein thrombosis, as seen in Lemierre syndrome, may present with fever, neck swelling, jaw angle pain, and trismus, often following pharyngitis or dental infection. Signs of systemic infection, such as fever and tachycardia, may also be present.[6]

Clinicians should also assess for complications. Symptoms such as dyspnea, chest pain, or hemoptysis may suggest pulmonary embolism. Neurologic symptoms, including severe or worsening headache, visual changes, or focal deficits, may indicate extension into the cerebral venous system and warrant urgent evaluation.[4]

Evaluation

A D-dimer level, a fibrin degradation product with high sensitivity but low specificity for venous thrombosis, can indicate deep vein thrombosis. D-dimer levels, however, can be elevated in conditions such as malignant neoplasms, sepsis, recent surgical procedures, trauma, and pregnancy. To confirm the diagnosis of IJVT specifically, duplex ultrasonography is the first-line imaging modality. Duplex ultrasonography can evaluate venous flow and compressibility. A noncompressible vein without any color flow is diagnostic of DVT. Duplex ultrasonography has a sensitivity of 96% and specificity of 93%.[9] Lemierre syndrome is identified by IJV thrombosis on duplex and growth of F necrophorum on blood cultures.[6] 

Treatment / Management

When treating IJVT, bleeding risk should be assessed prior to initiating anticoagulation. The American College of Chest Physicians (CHEST) guidelines can be used to guide treatment. Patients with acute proximal deep vein thrombosis, including involvement of the internal jugular vein, should be treated with low-molecular-weight heparin, fondaparinux, or intravenous or subcutaneous unfractionated heparin in the acute setting.

For patients with indwelling catheters who no longer require them, prompt removal is recommended. In patients with persistent thrombosis after catheter removal or those with a catheter that must remain in place, anticoagulation should be continued for at least 3 months. In patients with cancer, anticoagulation is typically continued until the catheter is removed.[10] Findings from clinical studies specifically evaluating anticoagulation outcomes in IJVT are limited, because many cases are incidentally detected. Surgical intervention is rarely indicated.

For infectious etiologies, such as Lemierre syndrome, treatment involves prolonged antibiotic therapy with broad-spectrum agents, including clindamycin and ceftriaxone. Once culture results are obtained, antibiotic therapy is narrowed. No established guidance on anticoagulation exists. However, based on the literature, anticoagulation is reserved for extensive or propagating thrombus or cerebral sinus involvement.[6]

Differential Diagnosis

The clinical manifestations of IJVT, including erythema, swelling, and warmth along the sternocleidomastoid muscle, may mimic neck infections such as cellulitis. Additional findings such as facial pain, neck edema, a palpable cord, and neck fullness can resemble superior vena cava syndrome or other causes of central venous obstruction, including malignant neoplasms (eg, lung tumors). The differential diagnosis includes the following: 

• Cellulitis and other neck infections
• Deep neck space infection (eg, peritonsillar, parapharyngeal, or retropharyngeal abscess)
• Cervical lymphadenopathy (infectious or malignant)
• Superior vena cava syndrome
• Malignant neoplasms, such as a lung tumor or lymphoma
• Mediastinitis
• Carotid artery pathology, such as dissection or aneurysm
• Superficial thrombophlebitis (more localized, less severe)
• Salivary gland disorders such as parotitis or sialadenitis

Prognosis

The prognosis of IJVT is generally favorable with appropriate treatment. Outcomes depend largely on the underlying etiology, particularly the presence of malignant neoplasms or infection, as well as the development of complications. Mortality is most often related to underlying comorbid conditions rather than the thrombosis itself. Lemierre syndrome has a significantly improved prognosis with the advent of antibiotics. The mortality rate has declined to less than 5%.[6]

Complications

Complications of IJVT include pulmonary embolism (10.3%) and postthrombotic syndrome (41.4%).[11] Thrombus propagation may occur, extending into the cerebral venous sinuses or central veins and potentially leading to neurologic complications. Infectious complications are particularly important in cases of septic thrombophlebitis, such as Lemierre syndrome. Without appropriate antibiotic therapy, septic emboli to the lungs occur in up to 97% of cases.[12] Sepsis and other metastatic infections may also develop.