Paget von Schroetter syndrome is a thrombosis that occurs in the deep brachial vein, subclavian vein, or axillary vein. The condition predominantly affects young adult males.
What is Paget von Schroetter syndrome?
In medicine, Paget-von-Schroetter syndrome is also known as arm and shoulder girdle vein thrombosis. The condition was named after the British physician James Paget (1814-1899) and the Austrian physician Leopold Schroetter Ritter von Kristelli (1837-1908). Other names for the syndrome include axillary vein blockage and deep arm vein thrombosis. In Paget von Schroetter syndrome, acute thrombosis occurs in the subclavian vein (subclavian vein) or axillary vein (axillary vein). In cases of chronic venous outflow obstruction, physicians refer to the condition as thoracic inlet syndrome (TIS). Paget-von-Schroetter syndrome is one of the rare diseases. Thus, it primarily manifests itself in young adults, who are predominantly men. Sometimes children also suffer from the syndrome. In most cases, Paget von Schroetter syndrome occurs on the right side of the body. About two percent of all thromboses are found in the arm and shoulder girdle region.
Causes
The causes for the occurrence of Paget von Schroetter syndrome vary. Among others, thoracic outlet syndrome is a possible trigger. In this case, there is a compression of the vascular nerve cord in the transition region between the arm and the chest. Thoracic outlet syndrome is usually caused by functional phenomena such as a narrowing between the first rib and the collarbone or musculoskeletal anomalies. It is also not uncommon for Paget-von-Schroetter syndrome to present after strenuous work such as overhead work with the arm. This may involve painting walls, carrying a backpack for long periods of time, or chopping wood. Sports activities such as wrestling, tennis, basketball or handball also sometimes result in Paget-von-Schroetter syndrome. Another possible cause is wearing a central venous catheter. In this case, the condition usually occurs after prolonged periods of immobilization as well as after extensive infusions of hyperosmolar solutions. In some patients, however, the physician is unable to determine a specific cause of Paget von Schroetter syndrome despite thorough examinations. In such cases, the thrombosis presents spontaneously.
Symptoms, complaints, and signs
Pain in the affected limb is considered a typical symptom of Paget von Schroetter syndrome. Likewise, swelling is within the realm of possibility. In some cases, the arm becomes reddish or bluish in color. In addition, the surface veins are visible through blotchy skin. This is the sign that bypass circulation is developing. Other conceivable complaints are feelings of tension as well as feelings of pressure in the armpit. A dangerous complication is pulmonary embolism, which occurs when a thrombus (blood clot) breaks loose. The embolism is often noticeable by pain in the chest and shortness of breath.
Diagnosis and course of the disease
If Paget von Schroetter syndrome is suspected, a physician must be consulted. This first deals with the medical history (anamnesis) of the patient as well as his complaints. He also checks whether nerves and arteries are involved in the syndrome. For example, there may be decreased arterial supply, loss of strength on one side, or paresthesia. The physician performs several clinical tests. These include Wright’s hyperabduction test and raising the arm or turning the head in a certain direction, which is also called the Adson maneuver. In this way, the physician detects a disappearing arterial pulse, which is considered an indication of thoracic outlet syndrome. As part of the medical history, the physician checks whether the patient engages in certain physical or athletic activities that can cause Paget-von-Schroetter syndrome. Duplex ultrasonography is usually performed to confirm the diagnosis. In some cases, phlebography (venography) may also be required. If there is a compression-related genesis, it is important to ensure appropriate imaging. It is also important to rule out malignant neoplasms or extensive upper arm hematomas as triggers of compression. For example, compression can sometimes be caused by lymph node metastases to the axilla.In most cases, Paget von Schroetter syndrome takes a favorable course. Only rarely does the arm develop postthrombotic syndrome.
Complications
In most cases, those affected by this syndrome suffer from very severe pain and further also from sensory disturbances in the affected joints and regions. Since this pain can occur not only during exertion, but in many cases also at night, this also results in sleep complaints and further in depression and other psychological upsets. The regions are clearly swollen and the skin is reddish in color. There may also be a feeling of tension or pressure in the armpits of the affected person. Paget von Schroetter syndrome also leads to pulmonary embolism, which can be fatal for the patient. In this case, the affected person suffers from shortness of breath and relatively severe pain that occurs in the chest. Due to Paget-von-Schroetter syndrome, the patient’s quality of life is significantly reduced and limited. If the disease occurs at a young age, it can cause various disturbances in the patient’s development and growth. Treatment of Paget-von-Schroetter syndrome is not associated with complications. The symptoms can be alleviated with the help of medication. Furthermore, however, many affected patients are dependent on a pacemaker to increase their life expectancy.
When should you see a doctor?
Swelling or discoloration of the skin appearance indicates irregularities that should be examined and clarified by a physician. If there is a reddish or bluish coloration of the skin, there is a need for treatment and a doctor should be consulted. In most cases, the complaints of those affected occur in the area of the arm. If there is a feeling of tension in the upper part of the body or disturbances in the blood circulation, a doctor should be consulted. If the symptoms spread to under the armpits, a doctor should be consulted as soon as possible. Pressure sensations, sensory disturbances as well as hypersensitivity to touch are signs of an existing health impairment and should be examined. Chest pain and shortness of breath are among the acute symptoms of Paget von Schroetter syndrome requiring immediate action. In severe cases, the emergency medical services should be alerted. At the same time, ventilation of the affected person should be ensured by taking first aid measures. Since the risk group for Paget-von-Schroetter syndrome mainly includes young adult males, it is particularly necessary for these groups of persons to consult a doctor if symptoms occur. In case of suddenly occurring restrictions of respiratory activity, a decrease in performance as well as internal weakness, the affected person needs immediate help. Disturbances in the developmental process of a child or adolescent as well as underdevelopment of growth should be discussed with a doctor. If additional psychological problems occur, medical help should be sought.
Treatment and therapy
Treatment of Paget-von-Schroetter syndrome is possible in both conservative and surgical ways. As part of conservative therapy, elevation of the affected arm takes place. In addition, the patient is given injections of heparin. If the thrombosis can be resolved, coumarin derivatives are administered for a period of six months. If the thrombosis is severe, fibrinolysis with plasminogen activators is performed in most cases. If this treatment procedure cannot be performed, surgical therapy is required. This involves, for example, removal of exotoses of the first rib or of a cervical rib. The most important therapeutic goals in Paget von Schroetter syndrome are to avoid postthrombotic syndrome and pulmonary embolism. In most cases, conservative treatment is only used in exceptional cases. Thus, this method of therapy is not able to counteract a post-thrombotic syndrome. Fibrinolysis achieves similarly good effects as surgical thromectomy. Thus, the success rate is about 80 percent. A surgical procedure is used if there are contraindications to lysis or phlegmasia coerulea dolens. However, contraindications to surgery are also possible.These include thrombosis due to intravenous permanent implants such as pacemakers, thrombosis due to tumor compression, and weakened general health of the affected individual.
Outlook and prognosis
Overall, Paget von Schroetter syndrome has a relatively good prognosis. With appropriate treatment, the vessel reopens within a few weeks. Accompanying this, the tendency of the arm to swell and the sometimes very severe pain, which can have a very negative effect on the patient’s well-being, especially in the first few days of the disease, usually decrease. The risk of pulmonary embolism is relatively low compared with leg and pelvic vein thrombosis. The prognosis is always based on the patient’s individual symptoms and general condition. The treating physician also considers the patient’s willingness to have surgical procedures performed. The procedures include removal of neck ribs and carry some risk of complications. Nevertheless, the prognosis is relatively good. Life expectancy is not reduced, insofar as the thrombosis is treatable. Thromboses such as Paget von Schroetter syndrome recur in high-risk patients. Therefore, close medical monitoring is necessary. The physician must establish the long-term prognosis in terms of the number and severity of thromboses that will occur in the patient during his or her lifetime.
Prevention
There is no known prevention of Paget von Schroetter syndrome. However, in some cases, certain measures such as avoiding heavy overhead work or specific sports activities may be helpful.
Follow-up
In Paget von Schroetter syndrome, very few measures and options for direct aftercare are available to affected individuals in most cases. However, affected individuals should ideally see a doctor early to avoid other complications or further worsening of symptoms. The earlier a doctor is contacted, the better the further course is usually, so that affected persons should ideally see a doctor at the first signs and symptoms of the disease. Self-cure of Paget-von-Schroetter syndrome usually cannot occur. Patients are often dependent on taking various medications even after initial treatment. Patients should pay attention to a correct dosage with regular intake in order to alleviate the symptoms permanently and, above all, correctly. Likewise, regular checks of the internal organs are very important in order to monitor the condition and detect damage at an early stage. Furthermore, sufferers of this disease should pay attention to a healthy lifestyle with a healthy diet to alleviate the symptoms. In some cases, the syndrome reduces the life expectancy of those affected, although no general prediction can be made about this.
Here’s what you can do yourself
Sufferers of Paget-von-Schroetter syndrome should generally avoid work that requires the arm to be above the head for an extended period of time. Painting walls, cleaning windows or chopping wood are among the activities that should be avoided. Sports activities should also be adapted to the body’s capabilities and needs. Affected individuals should avoid playing sports such as handball, basketball, swimming or tennis. These activities involve more upward movement of the arm over the shoulder and can trigger pain or swelling. In addition, carrying a backpack or bag over the shoulder is not beneficial to the patient’s health and should be avoided. The aforementioned activities may exacerbate existing symptoms or lead to a recurrence of adverse health effects. Everyday life should be restructured so that care is taken at all times in the performance of daily tasks to ensure that the arm and shoulder are subjected to only minor stresses. At the first signs of irregularity or discomfort, a period of rest should be taken. In addition, the posture should be changed so that no discomfort occurs. If the usual performance decreases or there is a tingling sensation in the shoulder or arm, the activities performed should be stopped immediately.
