What is Internal Jugular Vein Thrombosis?
The internal jugular vein (IJV) is a major blood vessel in your neck that begins at a space in your skull known as the jugular foramen, travels down the side of your neck, and ends at a vein located near your collarbone called the brachiocephalic vein. It’s one of four main structures found within a part of your neck known as the carotid sheath. The others include two major arteries that supply blood to your head and neck, a nerve that controls various body functions, and groups of lymph nodes that help your body fight off infections.
The path of the IJV varies among individuals. In most people, it is found in front or to the side of the main neck artery. However, in a few individuals, it is found medial or towards the midline of the body. IJV thrombosis refers to the formation of a blood clot inside the IJV. This can occur due to numerous risk factors. Some are inherited, such as having a specific gene mutation (known as the factor V Leiden mutation), while others are acquired or develop over time. These can include using intravenous drugs, having cancer, receiving hormone replacement therapy, being immobile for a long period, suffering trauma, or being pregnant.
These risk factors can lead to one or more components of what’s called Virchow’s triad. Named after a 19th-century German doctor, it’s a grouping of three conditions that increase the chances of a blood clot forming. These include increased blood coagulation or thickening, slowed blood flow, and problems with the lining of blood vessels.
The IJV is commonly used by doctors to monitor or stabilize a patient’s blood circulation due to its easy access and location. Sometimes, inserting intravenous catheters, which are thin tubes used to deliver fluids or medication directly into your veins, can cause injuries to the lining of the vein and inflammation. Because of that, the IJV is often where deep vein thrombosis (a blood clot in a deep vein) occurs when such catheters are used.
What Causes Internal Jugular Vein Thrombosis?
In 1855, a German doctor named Rudolf Virchow first used the words ‘thrombosis’ and ‘pulmonary embolism.’ He came up with something called the Virchow Triad, which is a set of three factors that can cause blood clots: too much clotting in the blood, slow or irregular blood flow, and damage to the veins.
Normally, anti-clotting medicines like heparin are used to prevent these clots, especially when a tube called a catheter is inserted into a vein. However, when a catheter is placed in the large vein located in the neck (the internal jugular vein), it can actually change the blood flow and damage the vein. Studying past cases, a researcher named Major KM and his team found that having a catheter was a major cause of blood clots in this vein.
Besides catheter usage, there are many potential causes of these blood clots. Some of these are inherited conditions that make the blood clot more easily, such as the Factor V Leiden mutation, Protein S deficiency, Protein C deficiency, and Antithrombin deficiency. Other risk factors that can be acquired include a history of blood clots, cancer, pregnancy, using oral contraceptives, using drugs intravenously, trauma, or being immobilized.
Furthermore, there are a few infections and conditions that can also lead to blood clots in the internal jugular vein. One is Lemierre syndrome, which is a severe throat infection typically followed by a bloodstream infection with certain types of bacteria. Other contributing conditions can include complications from neck surgery, severe skin infections called necrotizing fasciitis, a condition that can cause blood clots called antiphospholipid syndrome, a kidney disorder called nephrotic syndrome, and severe burns.
Risk Factors and Frequency for Internal Jugular Vein Thrombosis
A study found that the most frequent causes of internal jugular vein thrombosis – a blood clot within the internal jugular vein – are cancer and the use of a central venous catheter. Analyzing thousands of cancer patients, it has been found that about 7.5% of them ended up developing a blood clot related to the catheter.
Many things were considered in this study of catheter-related blood clots, such as whether the patient had a prior history of deep vein thrombosis, where the catheter was inserted, and the positioning of the catheter tip.
Also, people with factor V Leiden or other conditions that make blood clot more easily had a three times higher risk of developing a blood clot in the upper limbs.
Signs and Symptoms of Internal Jugular Vein Thrombosis
Internal jugular vein thrombosis, a condition where a blood clot forms in the neck vein, usually shows no symptoms, making it tough for doctors to diagnose. However, some people might show common signs of deep vein thrombosis such as redness, tenderness, and warmth. During a physical examination, doctors might observe a swelling on the left side of the neck, near the angle of the jaw or at the front border of the sternocleidomastoid muscle (the muscle running from behind the ear down to the collarbone). Another possible scenario is Lemierre syndrome, an infection-related condition, where symptoms like fever, headache, swelling of the neck, and jaw along with the inability to open the mouth fully (trismus) can appear.
Testing for Internal Jugular Vein Thrombosis
If you’re suspected of having venous thrombosis, a condition where a blood clot forms in your veins, your doctor may check for a substance called D-dimer in your blood. Though not specific, high levels of D-dimer in the blood are often an indicator of a clot. However, this test can also show high levels due to cancer, severe infection, recent surgery or trauma, or if you are pregnant, which are conditions that can often occur alongside internal jugular vein thrombosis, a specific type of vein clot that occurs in the neck.
Compression ultrasonography is the preferred method for diagnosing this condition, which uses sound waves to produce images of the inside of the body. This test is particularly good for this purpose, catching 96% of cases and being accurate 93% of the time. Another benefit is that it’s quick and doesn’t require any invasive procedures. This method is able to show if there’s a clot in your neck vein.
Though venography, an x-ray examination of the veins, is the ultimate test for diagnosing IJV thrombosis, a non-invasive and quick bedside ultrasound is commonly used. A CT scan, is another option and it can show a clot within the wall of a vein in the neck. CT scans might be better than ultrasounds for examining veins that are located under soft tissues – these create more detailed images.
Treatment Options for Internal Jugular Vein Thrombosis
It is vital to carefully assess the risk of bleeding when dealing with blood clot in the internal jugular vein (a large vein in the neck), as blood thinning treatments can potentially increase this risk. Tools such as HAS-BLED are used to help doctors assess this risk in adults. However, there is no full-proof method that predicts these risks perfectly.
If the patient has a catheter (a small, flexible tube) inserted, the doctors’ first step would usually be to remove it. If for some reason this isn’t possible, medications to prevent the blood from clotting (anticoagulants) should be started. The type of anticoagulant therapy is determined on a case-by-case basis.
Patients who aren’t at high risk of bleeding might be given a combination of anticoagulant drugs like heparin and warfarin or a combination of heparin followed by drugs like direct thrombin inhibitors or factor Xa inhibitors. Alternatively, they could be given factor Xa inhibitors alone.
Higher risk patients, such as those with blood clotting disorders, might benefit from a carefully controlled long-term treatment with warfarin. Here, the goal is to keep the patient’s International Normalized Ratio (a measure of blood clotting) between 2.5 and 3.0. However, it’s important to note that there’s a lack of extensive research specifically on treating internal jugular vein blood clots with anticoagulants.
Internal jugular vein blood clots often go unnoticed until they cause a noticeable problem. Thus, many patients may not receive treatment specifically for this.
Infusions of clot-dissolving drugs like alteplase have found to be effective, but generally aren’t recommended as the first-line of treatment when the clot is caused by a catheter. Current research doesn’t clearly show that these drugs improve outcomes compared to anticoagulant treatments.
In rare cases, a surgical procedure might be necessary to manage the clot.
What else can Internal Jugular Vein Thrombosis be?
Internal jugular vein thrombosis, which is a blood clot in a vein in the neck, can show up with various symptoms. These might include redness, swelling, and a warm feeling along a major neck muscle (the sternocleidomastoid). Such symptoms can make it look like a neck infection, like cellulitis. Other possible symptoms include facial pain, neck swelling, a hard lump that can be felt, and a full feeling in the neck. These can be similar to those that someone with superior vena cava syndrome might experience. Superior vena cava syndrome is a condition where blood flow gets blocked to the heart. Sometimes, these symptoms may also suggest a mechanical obstruction in the body, like a lung tumor.
These symptoms should not be ignored as they could indicate other serious issues like:
- Mediastinitis, which is an infection in the space between your lungs
- Superior vena cava syndrome, a blockage of blood flow to the heart
- A type of cancer, known as malignancy
What to expect with Internal Jugular Vein Thrombosis
In a forward-looking study, the death rate was found to be 44% for a condition called internal jugular vein thrombosis, which is a blood clot in a major vein in the neck. This rate is significantly higher in patients over 75 years old, those not treated with blood-thinning drugs (anticoagulation), and those with a central venous line, which is a tube placed into a large vein in your neck, chest, or groin to give medications or fluids.
For patients who already suffer from other illnesses, like cancer, chronic kidney diseases, infections, or failure of multiple organs, the death rate is significantly higher.
Possible Complications When Diagnosed with Internal Jugular Vein Thrombosis
The primary complication of upper extremity thrombosis, which is a blood clot in the arm, is a lung clot, also known as a pulmonary embolism. This can be followed by post-thrombotic syndrome, a long-term problem that can cause pain, swelling, and skin changes in the affected area, and even death. Complications related to a clot in the internal jugular vein (IJV thrombosis) include pulmonary embolism, occurring in about 10.3% of cases, and post-thrombotic syndrome, affecting 41.4% of patients. In the case of Lemierre’s syndrome, if not properly treated with antibiotics, 97 percent may develop an infection that travels (septic emboli) to the lungs.
Common Complications:
- Pulmonary embolism
- Post-thrombotic syndrome
- Death
- Septic emboli to the lung in Lemierre’s syndrome
Preventing Internal Jugular Vein Thrombosis
A blood clot in the internal jugular vein, a large vein on the side of the neck, often doesn’t cause noticeable symptoms in most people. However, healthcare providers should pay close attention to any signs of swelling, redness, or tenderness around the corner of the jaw or the side of the neck. Even though they are uncommon, serious complications like a lung blood clot can sometimes happen. To prevent these clots from happening again, it’s crucial that we do more tests to check for cancer, conditions that cause blood clots, or infections. The preferred treatment for patients who don’t have any reasons not to use it is a type of medication that prevents blood clots, known as anticoagulation therapy.