What is Paget-Schroetter Syndrome?
Paget Schroetter syndrome (PSS), also known as effort thrombosis, is a condition that occurs when the veins in the armpit and under the collarbone become clotted or blocked due to compression in a region known as the thoracic outlet. The thoracic outlet is an area near the shoulder and neck where several nerves and blood vessels pass through to the arm. This syndrome is a form of a broader condition known as thoracic outlet syndrome (TOS), which is when any of the nerves or blood vessels in that area become compressed. The concept of this condition was first introduced in 1875 by Sir James Paget, and later, von Schroetter theorized in 1884 that the condition could be caused by repeated motion that injures the vein.
What Causes Paget-Schroetter Syndrome?
The subclavian vein is a large vein that runs close to the collarbone (clavicle), first rib, and muscles known as the anterior scalene and subclavius. Paget-Schroetter syndrome (PSS) is a condition where this vein gets squeezed and results in a blood clot due to pressure from these nearby structures.
Risk Factors and Frequency for Paget-Schroetter Syndrome
PSS, or Paget-Schroetter syndrome, is frequently seen in younger people, typically in their 20s and 30s. Men are twice as likely to get it than women. Usually, these affected individuals do not have other prominent health issues. Each year, around 1 to 2 in every 100,000 people are diagnosed with PSS. Moreover, it’s often found on the right side of the body. Comparison with other types of Thoracic Outlet Syndromes (TOS) shows that PSS is less common than neurogenic TOS but more common than arterial TOS.
- PSS mostly affects people in their 20s and 30s.
- Men are twice as likely to get it as women.
- On average, there are 1 to 2 cases per 100,000 people each year.
- PSS is usually discovered on the right side of the body.
- It is less common than neurogenic TOS but more common than arterial TOS.
- People who regularly move their arms overhead, like baseball or softball pitchers, swimmers, rowers, or weightlifters, are often affected.
Signs and Symptoms of Paget-Schroetter Syndrome
If you have Paget-Schroetter Syndrome (PSS), you might notice swelling and pain in your upper body, specifically your arm. These symptoms can appear suddenly (acute), develop over a bit of time (subacute), or persist for a long time (chronic). Often, these symptoms will show up after heavy physical activity. When a doctor checks you out, they’d likely see an enlarged, swollen arm in comparison to the other one. Your affected arm might also feel tight and look a bluish color (cyanotic). The presence of extra visible veins on your chest and around your shoulder, known as Urschel’s sign, may be another clue to this condition.
Testing for Paget-Schroetter Syndrome
One of the simplest and non-invasive tests to identify a blood clot in the upper extremities (arms and hands) is an ultrasound. This test visualizes any blockage or clot in the deep veins. A fresh blood clot will appear as a non-squeezable area on the ultrasound. The vein with the clot may look wider than usual with no blood flow in the area. If the clot is an old one, the vein might have an irregular and partially collapsed appearance. However, an ultrasound may not able to provide a full image of the central veins, which are deep-seated in the body.
If a more detailed view is needed, a Computed Tomography (CT) scan or a Magnetic Resonance Imaging (MRI) scan can be performed. These scans are useful to visualize blood clots in the entire venous system, providing a more accurate diagnosis. Yet another method is contrast venography, which is a slightly more invasive test that can show the flow of blood in the central venous system.
Alongside imaging tests, your doctor will order certain laboratory tests. A thrombophilia panel, which includes testing Protein C and S levels, antithrombin levels, as well as checking for the presence of certain genetic mutations that can cause blood clots. Another crucial test is the D-dimer levels. Elevated D-dimer levels generally indicate the presence of a blood clot, but they can also increase due to infections, inflammation, and other physiological conditions.
If you display any symptoms related to a pulmonary embolism, a condition where a blood clot is blocking blood vessels in your lungs, a CT angiogram (CTA) of the thorax area needs to be conducted. Another option is a ventilation-perfusion study, which also diagnoses a pulmonary embolism. However, CTA of the thorax area is preferred over the ventilation-perfusion study.
Treatment Options for Paget-Schroetter Syndrome
Paget-Schroetter syndrome (PSS) is a type of blood clot in a deep vein. The first step in treating this condition typically involves the use of anticoagulation therapy, by using a medicine called heparin that is given directly into the vein. This therapy is used to prevent the clot from growing or moving to another part of the body. The dose of heparin needed varies depending on the person’s weight, and adjustments will be made based on how well the body is responding to the treatment. During this time, it’s also important to keep the affected arm or leg elevated in order to lessen any swelling.
If symptoms of PSS have been present for less than two weeks, your doctor may consider additional therapies to reduce the size of the blood clot. One method is to use a catheter (a long, flexible tube) that can deliver clot-busting medication directly to the clot. Another option is to physically remove the clot using specialized tools. Both methods can help to quickly decrease the size of the clot and improve symptoms.
Once the initial clot has been dealt with, attention is turned to treating the cause of the clot. In the case of PSS, it can often be caused by compression in the thoracic outlet, an area at the base of your neck, where the vein is squashed between your collarbone and first rib. To alleviate this compression, a surgical procedure might be necessary to remove the first rib, a procedure known as decompression the thoracic outlet.
The timing and need for follow-up therapies or interventions, like additional surgery or long-term anticoagulation (clot-preventing) therapy, can vary among patients and will be determined by the doctor based on individual circumstances. The use of metal stents, or tubes inserted into the vein to keep it open, is generally discouraged due to the risk of damage or blood clot formation from constant movement in this area.
For patients with certain underlying conditions that increase their risk for clot formation, prolonged use of anticoagulation therapy might be needed. The length of time for treatment typically runs from three to six months, but the exact duration will vary depending on the individual’s situation and the doctor’s recommendations.
If clotting recurs after the initial treatment and surgery, additional clot-busting treatments and imaging studies might be needed. Long-term anticoagulation therapy is generally advised for these patients as an additional preventative measure. In some patients with PSS, the vein might become completely blocked despite surgery. In such cases, more extensive surgeries to rebuild the vein might be considered, based on the severity of symptoms.
What else can Paget-Schroetter Syndrome be?
Swelling in the upper limbs could signal several health issues. These could be problems in the lymphatic system or more widespread illnesses like kidney disease in its final stages or heart failure. The swelling can also be a sign of a serious condition known as deep vein thrombosis in the upper extremity, which could occur in patients who have tubes inserted into their blood vessels for medical treatment.
What to expect with Paget-Schroetter Syndrome
Combining clot-dissolving therapy (thrombolysis) and surgery to relieve pressure on the nerves and blood vessels in the space between the collarbone and first rib (thoracic outlet decompression) can be effective in up to 90% of patients. Using blood thinners (anticoagulation) with decompression may not be as successful as the combination of clot-dissolving therapy and decompression, but it is still more effective than using blood thinners alone.
Possible Complications When Diagnosed with Paget-Schroetter Syndrome
- Blood clots in the lungs
- Long-term complications after a deep vein thrombosis
- Repeat occurrence of blood clots
Preventing Paget-Schroetter Syndrome
It’s very important for patients to take all their prescribed medications as recommended and keep regular appointments with their healthcare providers. Making changes to certain habits or ways of living can sometimes lower the chances of blood clotting issues happening again. If a person has a condition that makes their blood clot more easily (hypercoagulable states), they should be aware that they might need to take a specific blood-thinning medication (anticoagulation) for a long time. This helps to prevent the blood clots that could be potentially harmful.
