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Internal Jugular Vein Thrombosis

Author: Dale K Mueller, MD, Clinical Associate Professor of Surgery, Section Chief, Department of Surgery, University of Illinois College of Medicine; Co-Medical Director, Thoracic Center of Excellence, Vice-Chair, Department of Cardiovascular Medicine and Surgery, OSF St Francis Medical Center; Cardiovascular and Thoracic Surgeon, HeartCare Midwest, SC
Coauthor(s): Michael J Dacey, MD, Consulting Staff, Department of Internal Medicine, Division of Critical Care, Kent County Hospital
Contributor Information and Disclosures

Updated: Sep 21, 2009

Introduction

Thrombosis of the internal jugular (IJ) vein is an underdiagnosed condition that may occur as a complication of head and neck infections, surgery, central venous access, local malignancy, polycythemia, hyperhomocysteinemia, neck massage, and intravenous drug abuse. It is also reported to occur spontaneously. IJ thrombosis itself can have serious potentially life-threatening complications that include systemic sepsis, chylothorax, papilledema, airway edema, and pulmonary embolism. The diagnosis often is very challenging and requires, first and foremost, a high degree of clinical suspicion.

History of the Procedure

At the turn of the 20th century, thrombosis of the IJ vein was a feared complication of acute oropharyngeal infection. In 1936, Lemierre described the first case series of septic thrombophlebitis of the IJ vein, often complicated by metastatic infection.¹ The diagnosis was intuitive, although before the advent of effective antibiotic therapy, little could be offered and more than 50% of patients died.

Today, with widespread use of the IJ vein for venous access, central venous catheters now are the most common underlying cause of IJ thrombosis. Of concern is a recent trend in increasing number of individuals who abuse intravenous drugs who present with IJ thrombosis secondary to repeated drug injection directly into the IJ vein. Other causes include local malignancy and head, neck, and cardiac surgery. Rare causes include polycythemia, hyperhomocysteinemia, and neck massage.

Problem

IJ vein thrombosis refers to an intraluminal thrombus occurring anywhere from the intracranial IJ vein to the junction of the IJ and the subclavian vein to form the brachiocephalic vein.

The thrombosis may become secondarily infected, producing a septic thrombophlebitis. An infected IJ thrombus caused by extension of an oropharyngeal infection is referred to as Lemierre syndrome. This has also been termed necrobacillosis or postanginal septicemia.

Frequency

Some studies suggest that the rate of thrombosis may be lower for percutaneously inserted silastic hemodialysis catheters compared to those inserted surgically. Additionally, the rate of thrombosis may be lower in patients undergoing hemodialysis compared with other critically ill patients.

The incidence of Lemierre syndrome has fallen dramatically since the use of antibiotics began in the late 1950s. However, it still occurs, particularly in underserved populations.

Recent case series describe IJ thrombosis rates of 25-30% following functional neck dissection and hemodialysis catheter placement. However, a significant percentage of affected patients have been suggested to undergo recanalization, with excellent long-term patency rates.

The frequency of IJ vein thrombosis in individuals who abuse intravenous drugs is not known, but it usually occurs in people who have been using injectable drugs for years and have exhausted all peripheral access sites.

Etiology

Thrombosis associated with central venous catheters occurs more frequently than previously believed. One study found that 66% of patients who had an IJ vein catheter in place at some time during their hospital course had either ultrasonographic or autopsy evidence of IJ thrombus. The frequency was even greater in more critically ill patients, especially those with low cardiac output or shock syndromes.

The causes of IJ thrombosis include the following:

• Central venous or Swan-Ganz catheters in the IJ vein
• Central venous or Swan-Ganz catheters in the subclavian vein
• Individuals who abuse intravenous drugs using the IJ vein for access
• Lemierre syndrome
• Deep neck infections
• Necrotizing soft tissue infections
• Neck dissection surgery complication
• Head and neck malignancy
• Distant malignancy producing hypercoagulable state
• Hypercoagulable state secondary to factor V Leiden, protein C, protein S, or antithrombin III deficiency
• Jugular bulb catheters
• Any neck surgery involving prolonged retraction of the IJ vein
• Trauma
• Association with ovulation induction with gonadotropins
• Hyperhomocysteinemia
• Neck massage
• Polycythemia
• Spontaneous causes - Often secondary to undiagnosed malignancy or hypercoagulable state

Gram-positive organisms that often have high-grade resistance to beta-lactam antibiotics frequently cause septic thrombophlebitis associated with central venous catheters. One study reported a 40% incidence of beta-lactam–resistant organisms with catheter-induced IJ thrombosis. Individuals who abuse intravenous drugs have a very high risk of septic thrombophlebitis caused by methicillin-resistant strains of Staphylococcus aureus.

In cases of Lemierre syndrome, anaerobic organisms often predominate. Fusobacterium species (eg, F nucleatum, F necrophorum) are anaerobic gram-negative rods that are often mistaken for Bacteroides species. F necrophorum is the most virulent and commonly isolated pathogen. Other organisms include Bacteroides and Peptostreptococcus species and Eikenella corrodens.

Pathophysiology

The classic triad predisposing to intravascular thrombosis was described first by Virchow and includes blood vessel trauma, stasis of blood flow, and a hypercoagulable state.

In the case of central venous lines, the catheter itself acts as the nidus for clot formation, despite being bonded and flushed with heparin. Additionally, the catheter tip itself may produce damage to the vessel wall and disrupt venous flow, further augmenting clot formation.

Various oropharyngeal infections, including odontogenic infections and infections of the tonsils, peritonsillar tissue, pharynx, sinuses, middle ear, and parotids, all may lead to Lemierre syndrome. The primary infection spreads to the posterior compartment of the lateral pharyngeal space, leading to thrombophlebitis of the IJ vein. The infection spreads via local tissue planes, venules, or lymphatics. Subsequent sepsis syndrome may occur, usually a week or more after the primary infection.

The factors responsible for bacterial invasion are not well understood, although bacterial toxins, primary viral infection, and smoking have been implicated.

Intravenous drug injection promotes clot formation via vascular damage, local infection, or a combination of both. Malignancy may cause IJ thrombosis via local compression and invasion and/or by producing a systemic hypercoagulable state.

Presentation

The symptoms and signs of IJ thrombosis can often be very subtle, making it easy to overlook the diagnosis. Pain and swelling at the angle of the jaw and a palpable cord beneath the sternocleidomastoid muscle both may be absent in a significant minority of patients. Once infection has set in, other objective findings may be found. Tovi et al described the following clinical manifestations in their 1991 series of patients with septic IJ thrombosis as follows:²

Clinical manifestations of IJ thrombosis occur in the following percentages of patients:

• Fever - 83% of patients
• Leukocytosis - 78% of patients
• Cervical pain - 66% of patients
• Mass or neck swelling - 72% of patients
• Cord sign - 39% of patients
• Sepsis syndrome - 39% of patients
• Pleuropulmonary complications - 28% of patients
• Superior vena cava syndrome - 11% of patients
• Chylothorax - 5% of patients
• Jugular foramen syndrome - 6% of patients
• Increased intracranial pressure with symptoms that include headache, visual disturbances, and altered sensorium - rare³

Indications

Rare indications for a superior vena cava filter are similar to those of deep venous thrombosis in the lower extremity when an upper extremity deep vein thrombosis is associated with an internal jugular thrombosis. These include a clinical setting of pulmonary embolism in which therapeutic anticoagulation has failed or is contraindicated.

Relevant Anatomy

The internal jugular (IJ) vein begins in the cranium at the conclusion of the sigmoid sinus. It exits the cranium via the jugular foramen and then courses through the anterior neck lateral to the carotid artery, covered by the sternocleidomastoid for most of its length. It concludes by joining the subclavian vein, thus forming the brachiocephalic vein.

The styloid process divides the lateral pharyngeal space into an anterior (muscular) compartment and a posterior compartment containing the carotid artery within the carotid sheath, IJ vein, cranial nerves IX-XII, and lymph nodes.

Contraindications

The contraindications to surgery are few but would include uncorrected coagulopathy and cardiac risks for the procedure that are believed to outweigh the benefits.

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