Internal Jugular Vein Thrombosis,

Yi Lee; Waqas J. Siddiqui.

Author Information

Authors

Yi Lee1; Waqas J. Siddiqui2.

Affiliations

1 Chung Shan Medical University

2 Drexel University

Last Update: April 29, 2019.

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Introduction

The internal jugular vein (IJV) originates at the jugular foramen, tracks down to the lateral neck and ends at the brachiocephalic vein. The IJV is one of the four components of the carotid sheath, along with the common carotid artery, internal carotid artery, the vagus nerve, and the deep cervical lymph nodes. It courses medially to the sternocleidomastoid muscle at the carotid triangle. Studies have suggested significant variations in individuals, such as the IJV is anterior, lateral or anterior and lateral to the common carotid artery in the majority of the general population; whereas, for the remaining population, the IJV is medial to the common carotid artery.[1]  IJV thrombosis is the formation of thrombus located intraluminally in the IJV. Hereditary and acquired risk factors for thrombosis include intravenous drug use, factor V Leiden mutation, malignancies, hormone replacement therapy, immobilization, trauma, and pregnancy. These factors contribute to either one or more of the three components of Virchow triad: increased blood coagulation, altered blood flow (stasis) or endothelial dysfunction which lead to thrombosis.[2] The internal jugular vein is a common route used by clinicians to access the central circulation for hemodynamical monitoring and stabilization due to its accessibility and anatomic location. Intravenous catheters cause injuries to the endothelium and vein wall inflammation. The most frequently encountered site of deep vein thrombosis for centrally placed catheters is the IJV.[3]

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Etiology

A German physician, Rudolf Virchow is the first ever to coin the term of thrombosis and pulmonary embolism in 1855. The Virchow Triad refers to hypercoagulability, stasis or turbulence in blood flow and endothelial dysfunction. Despite being flushed with anticoagulants such as heparin, catheter placement in the internal jugular vein not only causes changes in the blood flow but also causes injury to the endothelium of the vein. In a retrospective review by Major KM et al. showed that the presence of a central venous catheter was a significant factor for IJV thrombosis.[3]However, there are various causes of the IJV such as Inherited hypercoagulable states, including Factor V Leiden mutation, Protein S deficiency, Protein C deficiency, Antithrombin deficiency, acquired risk factors including previous thromboembolism, malignancy, pregnancy, oral contraceptives, an intravenous drug, trauma, immobilization. Additionally, jugular vein suppurative thrombophlebitis, also known as Lemierre syndrome is suspected in patients with persistent bacteremia preceded by pharyngitis, dental infections or infectious mononucleosis. There have also been reports of surgical complications to the neck, necrotizing fasciitis, antiphospholipid syndrome, nephrotic syndrome, and severe burns.

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Epidemiology

A cross-section study suggested that the most common causes of internal jugular vein thrombosis are cancer and central venous catheter.[4] A systematic review and meta-analysis revealed 7.5 percent of patients out of 5636 cancer patients developed catheter-related venous thrombosis.[5] In examining catheter-related venous thrombosis, researchers weighed many factors such as the previous history of deep vein thrombosis, insertion site, and positioning of the catheter tip. Patients with factor V Leiden or other hypercoagulopathy were found to have a threefold increased risk of developing upper extremities thrombosis.

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Pathophysiology

The pathogenesis of venous thromboembolism (VTE) is Virchow triad. Three components of Virchow triad as below,

• Vascular endothelial injury, such as atherosclerosis, central venous catheter placement, vascular inflammation, trauma, severe burn
• Change of blood flow, such as immobility, arrhythmia, venous mechanical obstruction 
• Hypercoagulopathy, such as thrombophilia, malignancy, nephrotic syndrome, pregnancy, hormone therapy

Internal jugular vein thrombosis is most commonly due to prolonged central venous catheterization, trauma to the neck, infection, ovarian hyperstimulation syndrome (OHSS) and intravenous drug abuse.

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History and Physical

Internal jugular vein thrombosis is asymptomatic in the majority of patients. Due to its subtle manifestation, it is difficult to diagnose for clinicians. However, patients may present with typical signs for deep vein thrombosis such as erythema, tenderness, and warmth. On physical examination, swelling at the left sided neck, mandible angle or the anterior border of the sternocleidomastoid muscle can be found. Internal jugular vein thrombosis in the setting of infection as known as Lemierre syndrome (necrobacillosis), fever, headache, swelling of the neck and angle of the jaw along with trismus is another possible presentation.

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Evaluation

Laboratory studies may show an increased level of D-dimer, a degradation product of fibrin that is highly sensitive and nonspecific for venous thrombosis. Elevated levels of D-dimer present in malignancy, sepsis, recent surgery or trauma, pregnancy which often coexist with internal jugular vein thrombosis. Compression ultrasonography with Doppler is the test of choice for diagnosis with a sensitivity of 96% and a specificity of 93%.[6] While venography is the gold standard for diagnosing the IJV thrombosis, bedside ultrasound is noninvasive and rapid which may show hyperechoic thrombus within the IJV. CT scan may indicate an intraluminal filling defect in the jugular venous wall. CT scan may be superior to ultrasound due to a better assessment of veins located under soft tissues.

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Treatment / Management

The risk of bleeding requires careful assessment. Anticoagulation therapy is individualized for each patient; tools such as HAS-BLED help assess the bleeding risk in adults[7] However, no definitive index or tool can reliably predict bleeding risk in patients with internal jugular vein thrombosis. In patients with an indwelling catheter, it is crucial to remove the catheter; however, if the catheter cannot be removed for any reason, then anticoagulants should be initiated. Patients without bleeding risks should receive dual therapy with low molecular weight (LMW) heparin and warfarin, dual therapy with LMW heparin followed by direct thrombin inhibitor or factor Xa inhibitor, or monotherapy with factor Xa inhibitor. For high-risk patients like thrombophilia, some studies have suggested an INR maintained between 2.5 and 3.0; long-term warfarin therapy can also be a consideration. However, clinical studies in anticoagulation therapy for IJV thrombosis are lacking. Internal jugular vein thrombosis is often found incidentally in the majority of the patients. Hence, many patients were left untreated. Intravenous thrombolytic infusion regimens such as alteplase have been reported to be effective in IJV thrombosis. For patients diagnosed with catheter-induced IJV thrombosis, thrombolysis is not recommended as first-line therapy, as there is sparse evidence to suggest that thrombolysis leads to better outcomes than anticoagulation. Rarely do patients need surgical interventions.

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Differential Diagnosis

The clinical manifestations that accompany internal jugular vein thrombosis include erythema, swelling, and warmth along the sternocleidomastoid muscle resemble neck infections such as cellulitis. Facial pain, neck edema, a palpable cord, and neck fullness can present in some patients and may be similar to superior vena cava syndrome. A mechanical obstruction such as a lung tumor should be ruled out. 

• Mediastinitis 
• Superior vena cava syndrome
• Malignancy 

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Prognosis

In a prospective study, the mortality rate of internal jugular vein thrombosis was reported to be 44%. The mortality rate is significantly higher in patients over 75 years old, patients not treated with anticoagulation and patients with an indwelling central venous line.  In patients with underlying comorbidities such as malignancy, chronic kidney diseases, infections, and multiorgan failure, mortality is significantly higher.

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Complications

Pulmonary embolism is the most common complications in upper extremity thrombosis, followed by post-thrombotic syndrome and death.[8] Complications of IJV thrombosis are pulmonary embolism (10.3%) and post-thrombotic syndrome (41.4%).[4] In Lemierre's syndrome, without proper antibiotic management, 97 percent of cases developed septic emboli to the lung.[9]

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Deterrence and Patient Education

Internal jugular vein thrombosis is asymptomatic in most patients. Clinicians should carefully evaluate swelling, erythema, or tenderness in the angle of the jaw or the side of the neck. Complications such as pulmonary embolism although rare but can occur. Further investigations on malignancy, hypercoagulopathy or infections are necessary for recurrence prevention. Anticoagulation therapy is the treatment of choice for patients without contraindications. 

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Pearls and Other Issues

Internal jugular vein thrombosis has been reported to be more common due to the widespread use of the internal jugular vein for venous access, central venous catheters. Of note, an increased number of IV drug abuses present with IJV thrombosis, nevertheless anticoagulation still is the mainstay of treatment comparing to thrombolysis or surgical intervention.

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Enhancing Healthcare Team Outcomes

Anticoagulation is the treatment of choice for patients with internal jugular vein thrombosis.[10] [Level l] Anticoagulant therapy following a diagnosis of IJV thrombosis prevents severe complications such as pulmonary embolism. The pharmacist, nurse practitioner, internist or the primary care provider can monitor the patient's anticoagulation status. These professionals need to work together in an interprofessional team approach to managing IJV thrombosis to ensure optimal clinical results for patients.

In patients with asymptomatic IJV thrombosis, anticoagulation is the recommendation, as a delay in therapy increases the risk of potentially life-threatening embolization. Anticoagulants include subcutaneous low molecular weight (LMW) heparin, subcutaneous fondaparinux, the oral factor Xa inhibitors (rivaroxaban or apixaban), or unfractionated heparin (UFH) (Level III). Anticoagulation management requires individualized customization along with clinician experience.

The duration of anticoagulation ranges from 4 to 12 weeks. It is standard to obtain an imaging study before discontinuing anticoagulation.

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Questions

To access free multiple choice questions on this topic, click here.

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References

1.

Karakitsos D, Labropoulos N, De Groot E, Patrianakos AP, Kouraklis G, Poularas J, Samonis G, Tsoutsos DA, Konstadoulakis MM, Karabinis A. Real-time ultrasound-guided catheterisation of the internal jugular vein: a prospective comparison with the landmark technique in critical care patients. Crit Care. 2006;10(6):R162. [PMC free article] [PubMed]

2.

Wolberg AS, Rosendaal FR, Weitz JI, Jaffer IH, Agnelli G, Baglin T, Mackman N. Venous thrombosis. Nat Rev Dis Primers. 2015 May 07;1:15006. [PubMed]

3.

Major KM, Bulic S, Rowe VL, Patel K, Weaver FA. Internal jugular, subclavian, and axillary deep venous thrombosis and the risk of pulmonary embolism. Vascular. 2008 Mar-Apr;16(2):73-9. [PubMed]

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Gbaguidi X, Janvresse A, Benichou J, Cailleux N, Levesque H, Marie I. Internal jugular vein thrombosis: outcome and risk factors. QJM. 2011 Mar;104(3):209-19. [PubMed]

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Saber W, Moua T, Williams EC, Verso M, Agnelli G, Couban S, Young A, De Cicco M, Biffi R, van Rooden CJ, Huisman MV, Fagnani D, Cimminiello C, Moia M, Magagnoli M, Povoski SP, Malak SF, Lee AY. Risk factors for catheter-related thrombosis (CRT) in cancer patients: a patient-level data (IPD) meta-analysis of clinical trials and prospective studies. J. Thromb. Haemost. 2011 Feb;9(2):312-9. [PMC free article] [PubMed]

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Kommareddy A, Zaroukian MH, Hassouna HI. Upper extremity deep venous thrombosis. Semin. Thromb. Hemost. 2002 Feb;28(1):89-99. [PubMed]

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Lip GY. Implications of the CHA(2)DS(2)-VASc and HAS-BLED Scores for thromboprophylaxis in atrial fibrillation. Am. J. Med. 2011 Feb;124(2):111-4. [PubMed]

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Flinterman LE, Van Der Meer FJ, Rosendaal FR, Doggen CJ. Current perspective of venous thrombosis in the upper extremity. J. Thromb. Haemost. 2008 Aug;6(8):1262-6. [PubMed]

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Golpe R, Marín B, Alonso M. Lemierre's syndrome (necrobacillosis). Postgrad Med J. 1999 Mar;75(881):141-4.[PMC free article] [PubMed]

10.

Kearon C, Akl EA, Ornelas J, Blaivas A, Jimenez D, Bounameaux H, Huisman M, King CS, Morris TA, Sood N, Stevens SM, Vintch JRE, Wells P, Woller SC, Moores L. Antithrombotic Therapy for VTE Disease: CHEST Guideline and Expert Panel Report. Chest. 2016 Feb;149(2):315-352. [PubMed]

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Bookshelf ID: NBK541111PMID: 31082155

Internal jugular vein thrombosis (IJVT) is a serious event with a potentially fatal outcome. Complications include pulmonary embolism, sepsis with septic emboli to different organs and tissues as well as intracranial propagation of the thrombus with cerebral edema. As any thrombosis, IJVT is precipitated by Virchow's triad: endothelial damage, alteration of blood flow and hypercoagulability. The history and examination in patients with an IJVT may be vague and misleading. Patients may present with a painful swelling of the neck but they may also be absolutely asymptomatic. Imaging procedures frequently used to diagnose an IJVT include sonography with color-coded duplex sonography, computed tomography, magnetic resonance imaging as well as magnetic resonance venography. Up to date, there is no standardized treatment regimen for patients with an IJVT. PATIENTS AND METHODS: This retrospective study includes all ten patients with an IJVT who were seen at our department between January 2000 and January 2004. There were six female and four male patients. The average age was 49.7 years, ranging from 28 to 79 years. RESULTS: In five cases, the thrombosis was associated with a malignant tumor. In four patients, it was caused by a deep neck space infection and in one case the IJVT was due to cervical, intravenous drug abuse. Two patients were found to be pregnant (one tumor patient and one patient with a deep neck space infection). In all cases, a ten day treatment regimen with intravenous antibiotics and anticoagulant therapy was initiated. Oral or subcutaneous anticoagulation was continued for six weeks to six months. No complications were seen in any patient. In three patients a revascularization of the affected vessel could be demonstrated with color-coded duplex sonography six months after the initial presentation. CONCLUSIONS: Thrombosis of the IJV is probably underdiagnosed. Since the clinical presentation may be vague or misleading, a high degree of suspicion is required to make the diagnosis. The potential complications such as pulmonary embolism or intracranial propagation of the thrombus may be fatal. Whenever the thrombosis is not caused by an inflammatory process, a malignant tumor should be excluded. We recommend a therapy with intravenous antibiotics as well as a systemic anticoagulation. Ligation or resection of the internal jugular vein is reserved for patients who develop complications despite adequate medical therapy. PMID: 15538664 DOI: 10.1055/s-2004-825749

Thrombosis of the internal jugular vein in the ENT-department — Prevalence, causes and therapy: A retrospective analysis Publication date: August 2019 Source: Auris Nasus Larynx, Volume 46, Issue 4 Author(s): Janina Hahn, Melanie Nordmann-Kleiner, Thomas K. Hoffmann, Jens Greve Abstract Objective Less than 5% of deep vein thrombosis is due to thrombosis of the internal jugular vein. Genetic, malignant or inflammatory underlying diseases as well as insertion of venous catheters can be responsible for this pathology. Due to its rare occurrence, it is difficult to find systematic research about thrombosis of the internal jugular vein. Methods We performed a systematic analysis of present patient data from our ENT department with the electronic patient record considering the period from 2012-2017. Search terms were “thrombosis” and “jugular internal vein”. We identified 41 patients with the requested diagnosis and performed further analysis of the cases. Internal jugular vein thrombosis was diagnosed in all patients using Duplex sonography and/or CT/MR angiography. Results Paraneoplastic thrombosis was found in 22/41 patients (54%), in 15 of the 22 (68%), the tumor was located in the ENT region. Two out of seven (29%) of the patients with tumor entities outside the head and neck region had thrombosis of the internal jugular vein as the first symptom of the disease. Another 14/41 patients (34%) had underlying inflammatory diseases – mostly streptococci-associated – for example a cervical abscess. In two patients, insertion of a central-venous catheter was causal, in three patients we could not find any reason for the development of thrombosis. Conclusion To diagnose the rare and often asymptomatic thrombosis of the internal jugular vein, ultrasound of the cervical region should always include vascular imaging. Thrombosis of the internal jugular vein results mostly paraneoplastic or due to inflammation/abscess. It can be the first symptom of a malignant primary disease and always requires detailed diagnostic clarification. Level of evidence 4.