Paget -von-Schroetter syndrome is a thrombosis that occurs in the deep arm vein, subclavian vein, or axillary vein. The disease mainly affects young adult men.
What is Paget von Schroetter Syndrome?
In medicine, Paget-von-Schroetter syndrome is also known as arm and shoulder girdle vein thrombosis. The disease was named after the British physician James Paget (1814-1899) and the Austrian physician Leopold Schroetter Ritter von Kristelli (1837-1908). Other names of the syndrome are axillary vein blockage and deep arm vein thrombosis. See gradinmath for What is CSD.
In Paget-von-Schroetter syndrome, acute thrombosis occurs in the subclavian vein (collarbone vein) or the axillary vein (armpit vein). In the case of chronic outflow disorders of the veins, doctors speak of a thoracic inlet syndrome (TIS). Paget-von-Schroetter syndrome is one of the rare diseases. This is primarily seen in young adults, who are predominantly male.
Sometimes children also get the syndrome. In most cases, Paget-von-Schroetter syndrome occurs on the right side of the body. Around two percent of all thromboses are found in the arm and shoulder girdle region.
The causes of the occurrence of Paget-von-Schroetter syndrome are different. One of the possible triggers is thoracic outlet syndrome. There is compression of the vascular nerve cord in the transition region between arm and chest. Thoracic outlet syndrome is usually caused by functional phenomena such as a narrowing between the first rib and collarbone or musculoskeletal anomalies.
It is not uncommon for Paget-von-Schroetter syndrome to appear after strenuous work such as overhead work with the arm. This can be painting walls, carrying a backpack for a long time or chopping wood. Sporting activities such as wrestling, tennis, basketball or handball sometimes result in Paget-von-Schroetter syndrome.
Another possible cause is wearing a central venous catheter. The condition usually occurs after a long period of lying in bed and after extensive infusions of hyperosmolar solutions. In some patients, the doctor cannot determine a specific cause of Paget-von-Schroetter syndrome, despite thorough examinations. The thrombosis appears spontaneously.
Symptoms, Ailments & Signs
Pain in the affected limb is a typical symptom of Paget-von-Schroetter syndrome. Swelling is also a possibility. In some cases, the arm turns reddish or bluish. Also, the surface veins are visible through mottled skin. This is a sign that bypass circuits are developing. Other conceivable complaints are feelings of tension and pressure in the armpits.
The occurrence of a pulmonary embolism, which is caused by the detachment of a thrombus (blood clot), is considered a dangerous complication. The embolism often becomes noticeable through pain in the chest and shortness of breath.
Diagnosis & course of disease
If Paget-von-Schroetter syndrome is suspected, a doctor must be consulted. This first deals with the medical history (anamnesis) of the patient and their symptoms. He also checks whether nerves and arteries are involved in the syndrome. For example, there may be reduced arterial supply, unilateral loss of strength or paresthesia.
The doctor performs various clinical tests. These include the Wright hyperabduction test and raising your arm or turning your head in a specific direction, also known as the Adson maneuver. In this way, the doctor detects a disappearing arterial pulse, which is considered an indication of thoracic outlet syndrome.
As part of the medical history, the doctor checks whether the patient does certain physical or sporting activities that can cause Paget-von-Schroetter syndrome. Duplex sonography is usually used 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 suitable imaging.
It is also important to rule out malignant neoplasms or extensive upper arm hematomas as a trigger for compression. This can sometimes be caused by lymph node metastases in the armpit. In most cases, Paget-von-Schroetter syndrome takes a benign course. Only rarely does a postthrombotic syndrome occur in the arm.
In most cases, those affected by this syndrome suffer from very severe pain and also from sensory disturbances in the affected joints and regions. Since this pain can occur not only under stress, but in many cases also at night, it also leads to sleep disorders and further to depression and other mental upsets.
The regions are clearly swollen and the skin is reddish in color. It can also lead to a feeling of tension or pressure in the armpits of the affected person. Paget-von-Schroetter syndrome also leads to a pulmonary embolism, which can be fatal for the patient. Those affected suffer 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 restricted. If the disease occurs at a young age, it can lead to various disorders in the development and growth of the patient. Treatment of Paget von Schroetter syndrome is not associated with complications. The symptoms can be alleviated with the help of medication. However, many of those affected are still dependent on a pacemaker in order to increase life expectancy.
When should you go to the doctor?
Swelling or discoloration of the skin indicate irregularities that should be examined and clarified by a doctor. If the skin turns red or bluish, treatment is required and a doctor should be consulted. Most of the time, the symptoms occur in those affected in the arm area. A doctor should be consulted if you feel tight in your upper body or have problems with your blood circulation. If the symptoms spread to the armpits, see a doctor as soon as possible.
Feelings of pressure, sensory disturbances and 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 that require immediate action. In severe cases, the emergency services should be alerted. At the same time, ventilation of the affected person must be ensured by taking first aid measures. Since the risk group for Paget-von-Schroetter syndrome is predominantly young adult men, it is particularly necessary for these groups of people to see a doctor if symptoms occur.
In the event of sudden limitations in breathing activity, a decrease in performance and inner weakness, the person concerned needs help immediately. Disturbances in the development process of a child or adolescent as well as underdevelopment of growth should be discussed with a doctor. If there are additional psychological problems, medical help should be sought.
Treatment & Therapy
Treatment of Paget-von-Schroetter syndrome is possible in both conservative and surgical ways. As part of conservative therapy, the affected arm is elevated. In addition, the patient is given injections of heparin. If the thrombosis is resolved, coumarin derivatives are administered for a period of six months.
If the thrombosis is severe, fibrinolysis with plasminogen activators occurs in most cases. If this treatment method cannot be carried out, surgical therapy is required. In this case, for example, a removal of exotics of the first rib or of a cervical rib takes place.
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. This therapy method is not able to counteract a postthrombotic syndrome. Fibrinolysis achieves similarly good effects as operative thrombectomy.
The success rate is around 80 percent. A surgical procedure is used when there are contraindications to lysis or phlegmasia coerulea dolens. However, contraindications to surgery are also possible. These include thrombosis due to permanent intravenous implants such as pacemakers, thrombosis due to tumor compression and a weakened general state of health of the person concerned.
Outlook & Forecast
Overall, Paget-von-Schroetter syndrome has a relatively good prognosis. With appropriate treatment, the vessel opens again within a few weeks. Accompanying this, the tendency to swell the arm and the sometimes very severe pain, which can have a very negative effect on the well-being of the patient, especially in the first few days of the disease, usually decrease.
The risk of a pulmonary embolism is relatively low compared to leg and pelvic vein thrombosis. The prognosis is always based on the individual symptoms and the general condition of the patient. The attending physician also takes into account the willingness of the patient to have surgical measures performed. The procedures include the removal of cervical ribs and carry a certain risk of complications. Nevertheless, the prognosis is relatively good.
Life expectancy is not reduced if the thrombosis can be treated. Thromboses such as Paget-von-Schroetter syndrome occur again and again in high-risk patients. Close medical supervision is therefore necessary. The doctor must make the long-term prognosis in terms of the number and severity of thrombosis that will occur in the patient throughout life.
Prevention of Paget-von-Schroetter syndrome is not known. In some cases, however, certain measures such as avoiding heavy overhead work or specific sports activities can be helpful.
In most cases, those affected by Paget-von-Schroetter syndrome have very few measures and options for direct aftercare. Ideally, those affected should consult a doctor at an early stage so that other complications or a further deterioration of the symptoms do not occur. The sooner a doctor is contacted, the better the further course, so that those affected should ideally see a doctor at the first signs and symptoms of the disease.
As a rule, Paget-von-Schroetter syndrome cannot heal itself. Patients are often dependent on taking various medications even after the initial treatment. Those affected should pay attention to the correct dosage with regular intake in order to relieve the symptoms permanently and, above all, correctly.
Regular checks of the internal organs are also 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 in order 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.
You can do that yourself
Sufferers of Paget-von-Schroetter syndrome should generally avoid work that requires the arm to be raised over the head for a long period of time.
Painting the walls, cleaning windows or chopping wood are among the activities that should be avoided. The sporting activities are also to be adapted to the possibilities and needs of the body. Those affected should avoid playing sports such as handball, basketball, swimming or tennis. During these activities, the arm is raised over the shoulder and can cause pain or swelling. In addition, carrying a backpack or bag over the shoulder is not conducive to the patient’s health and should therefore be avoided.
The activities mentioned can intensify existing complaints or lead to a recurrence of health impairments. Everyday life must be restructured so that when carrying out daily tasks, care is always taken to ensure that the arm and shoulder are only exposed to low loads. At the first irregularities or if you feel unwell, you should take a break. In addition, the posture must be changed so that no complaints occur. If the usual performance decreases or if there is a tingling feeling in the shoulder or arm, the activities carried out must be stopped immediately.