Paget-Shreter syndrome( disease): treatment, diagnosis, symptoms

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Who is sick more often? The statistics are unambiguous: the elderly and people who lead an unhealthy lifestyle living in an unfavorable ecological situation. It would seem that it is enough to observe a healthy life-style, and you will be healthy. But this is erroneous. The disease can overtake and athletes, and some of the diseases in general can be called professional for this group of people. The latter includes the Paget-Shreter syndrome.

Features of the disease

The name of the syndrome was in honor of James Paget, who first suggested that pain symptoms and swelling can cause vein thrombosis, and also in honor of Leopold von Schreter, who linked the signs of the disease with thrombosis of the axillary and subclavian veins.

Paget-Shreter syndrome is a rare disease, so it is not fully understood. In the world medical practice there were only 900 cases of this pathology. Statistics based on these cases show that men are more prone to the syndrome and are sick 4 times more often.

An interesting observation is that the disease affects people involved in sports and physical labor. The average age of patients is 20-40 years.

Classification and forms

Several classifications of the state are distinguished. Thus, in 1970, in the distribution of puffiness, R. Cadenat identified 2 forms:

• Partial. Edema affects only the limb.
• Common. Edema from the extremity passes to the chest and area under the clavicle.

The same scientist identified 2 forms of severity: light and heavy, when progress of the condition leads to disability. E. Loelsen in 1969 identified 2 forms of the disease, depending on the nature of the course: thrombotic and non-thrombotic.

In Russia, the classification of A. Pytel and B. Sokolovsky is common, dividing the syndrome into acute and chronic. Later, in 1971, V. Prikupets expanded the classification, singling out 3 forms of acute stage:

• Lung. The pressure in the veins is up to 300 mm of water column.
• Average. The pressure rises up to 800 mm of water column.
• Heavy, when the pressure can reach 1300 mm of water column.

It is noteworthy that the symptoms of the disease in the acute stage are constant, while their intensity rises and reaches a maximum of 3 days. The duration of this stage is usually up to 3 weeks.

The chronic stage is characterized by moderate hypertension of the veins in the limbs, however, the lack of blood outflow is clearly visible under physical exertion.

Reasons for the occurrence of

The exact cause is for certain unknown. At the moment there are 3 theories explaining the reasons for the appearance of the Paget-Shreter syn: traumatic, neurogenic and infectious.

1. The most popular among scientists is a traumatic theory linking pathology and traumatism of subclavian or axillary veins due to physical effort. When a person exerts a certain effort, the vein is damaged, so a clot forms in it. To a single opinion of the primary location of the gap, scientists have not come. So, Scientist J. Sampson suggested that the syndrome can be caused by the fact that the crevice under the first rib and clavicle narrows, which compresses the vein. It is also not possible to compile a list of actions that could lead to damage, as there are a lot of them. It can be and constant actions, made by the person almost every day.
2. Cottalorda J. in 1932, during surgery, found that narrowing of the subclavian vein accompanies the cervical sympathetic nodes. This episode gave rise to the development of a neurogenic theory, the essence of which lies in the fact that the body has a pathological process in the tissues, and this breaks the vasomotor balance.
3. The infectious theory is based on a nonspecific infection and is partly confirmed by the symptomatology of the syndrome: high fever, leukocytosis and increased erythrocyte sedimentation rate.

Symptoms of

Usually, the symptoms of Paget-Shreter's disease appear after physical exertion and are expressed in:

1. puffiness;
2. cyanosis( blue limbs);
3. voltage;
4. increased fatigue;
5. dyspnea;
6. widening of the subcutaneous veins;
7. elevated temperature;

During the acute stage, patients experience acute pain of constrictive nature, as well as:

1. sensation of raspiraniya;
2. sensation of cold;
3. tingling and a feeling of heaviness in the neck;

Most patients have hyperesthesia( hypersensitivity), muscle hypotension of the hands. Periodic and tendon reflexes initially increase, and then decrease.

The following section will tell you about the diagnosis of the Paget-Shreter syndrome.

Diagnosis

First of all, the doctor must collect a history of complaints and family, as well as conduct a physical examination, which can differentiate the disease from others. On examination, cyanosis and puffiness are detected, which are the main signs of the disease. After this, the patient is sent for examination:

• Ultrasound dopplerography or duplex study confirming the diagnosis.
• Phlebography, which allows to assess the localization and distribution of thrombosis, the degree of development of collateral vessels.
• X-ray, which determines the bone causes of the syndrome.

If there is an MRI device in the population of the patient, it is useful to perform the NMR.This study allows you to determine the exact location of the thrombus and whether other veins are involved in the process.

Treatment of Paget-Shreter syndrome

The treatment method is chosen depending on the form of the disease. The basis of treatment is a conservative drug regimen.

More details on some of the methods of treatment for deep vein thrombosis will be described by the specialist in the following video:

Therapeutic

Therapeutic method consists in observing a stationary regime if the patient has an acute stage of the disease.

Therapeutic technique also affects the postoperative period. So, after the patient is discharged, he is recommended elastic bandaging of the hand.

Medicated

The basis of drug treatment is antithrombotic therapy. As the drugs included in this therapy, appoint:

• Fibrinolytic like fibrinolysin, streptases, and so on. It is shown in the first days( up to 5 days) to conduct a lysis of the thrombus.
• Anticoagulant of direct action, for example, heparin.
• Anticoagulant of indirect action such as warfarin, phenylin and others.
• Antiaggregants: trental, tiklid, etc.

After treatment, the patient should periodically resort to conservative medication to prevent relapse.

Operation

Surgical intervention is prescribed in the event that a conservative one has not helped or the patient has a severe violation of the outflow of blood, manifested by limited work capacity. The patient can undergo such operations:

• Thrombectomy. Optimal conduct it in the first 72 hours of the disease. The operation allows you to restore blood flow.
• Autovenous bypass. It is characterized by low traumatism in comparison with other operations, as the muscle and vein do not overlap. As a shunt, transplants are used, which are made from a large subcutaneous femoral vein.

Other reconstructive vascular surgery did not have any popularity.

Prevention of

disease No specific prevention of Paget-Shreter syn-syndrome has been developed. It is recommended to follow the general preventive measures:

• To pass a preventive examination every 6 months.
• Strengthen immunity.
• Take a multivitamin.
• Refuse bad habits.
• Ensure that the food is balanced.
• Restrict physical activity to moderate.

It is also important to treat emerging infectious diseases in time and strictly follow the prescription of doctors.

Complications of

Paget-Shreter syndrome is rarely complicated. In the absence of adequate treatment, diseases associated with circulatory disorders, for example, chronic heart failure and arrhythmia, can develop. The most dangerous complications are pulmonary embolism and venous gangrene, which lead to death. However, their cases are extremely rare.

Forecast

The prognosis for all stages is positive: Pajeta-Shreter's syndrome does not pose a threat to life. However, the patient does not completely recover, and negative symptoms are observed even after the cure. Also, the disease can lead to disability.

The following video will describe in more detail how to provide for the development of thrombosis, including Paget-Shreter syndrome: