Paget–Schroetter disease (also known as venous thoracic outlet syndrome) is a form of upper extremity

deep vein thrombosis Deep vein thrombosis (DVT) is a type of venous thrombosis involving the formation of a blood clot in a deep vein, most commonly in the legs or pelvis. A minority of DVTs occur in the arms. Symptoms can include pain, swelling, redness, and enla ...

(DVT), a medical condition in which

blood clot A thrombus (plural thrombi), colloquially called a blood clot, is the final product of the blood coagulation step in hemostasis. There are two components to a thrombus: aggregated platelets and red blood cells that form a plug, and a mesh of c ...

s form in the deep

vein Veins are blood vessels in humans and most other animals that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are the pulmonary and umbilical veins, both of which carry oxygenated b ...

s of the

arm In human anatomy, the arm refers to the upper limb in common usage, although academically the term specifically means the upper arm between the glenohumeral joint (shoulder joint) and the elbow joint. The distal part of the upper limb between the ...

s. These DVTs typically occur in the axillary and/or

subclavian vein The subclavian vein is a paired large vein, one on either side of the body, that is responsible for draining blood from the upper extremities, allowing this blood to return to the heart. The left subclavian vein plays a key role in the absorption ...

Signs and symptoms

The condition is relatively rare. It usually presents in young and otherwise healthy patients, and also occurs more often in males than females. The syndrome also became known as "effort-induced thrombosis" in the 1960s, as it has been reported to occur after vigorous activity, though it can also occur due to anatomic abnormality such as clavicle impingement or spontaneously. It may develop as a sequela of thoracic outlet syndrome. It is differentiated from secondary causes of upper extremity thrombosis caused by intravascular catheters. Paget–Schroetter syndrome was described once for a

viola The viola ( , also , ) is a string instrument that is bow (music), bowed, plucked, or played with varying techniques. Slightly larger than a violin, it has a lower and deeper sound. Since the 18th century, it has been the middle or alto voice of ...

player who suddenly increased practice time 10-fold, creating enough repetitive pressure against the brachiocephalic and external jugular veins to cause thrombosis. Symptoms may include sudden onset of pain, warmth, redness, blueness and swelling in the arm. Diagnosis is usually confirmed with an ultrasound. These DVTs have the potential to cause a

pulmonary embolism Pulmonary embolism (PE) is a blockage of an pulmonary artery, artery in the lungs by a substance that has moved from elsewhere in the body through the bloodstream (embolism). Symptoms of a PE may include dyspnea, shortness of breath, chest pain p ...

Diagnosis

Duplex ultrasonography MR Venography

Prevention

Prevention of Paget–Schroetter disease can be accomplished by gradual increases in activity and by avoiding strenuous upper extremity activity.

Treatment

The traditional treatment for thrombosis is the same as for a lower extremity DVT, and involves systemic

anticoagulation Anticoagulants, commonly known as blood thinners, are chemical substances that prevent or reduce coagulation of blood, prolonging the clotting time. Some of them occur naturally in blood-eating animals such as leeches and mosquitoes, where the ...

to prevent a pulmonary embolus. Some have also recommended thrombolysis with catheter directed

alteplase Alteplase (t-PA), a biosynthetic form of human tissue-type plasminogen activator (t-PA), is a thrombolytic medication, used to treat acute ischemic stroke, acute ST elevation myocardial infarction, ST-elevation myocardial infarction (a type of h ...

or mechanical thrombectomy with a large bore catheter and manual aspiration providing definitive intervention with an endovascular approach. If there is thoracic outlet syndrome or other anatomical cause then surgery can be considered to correct the underlying defect.

History

The condition is named after two men.

James Paget Sir James Paget, 1st Baronet FRS HFRSE (11 January 1814 – 30 December 1899) (, rhymes with "gadget") was an English surgeon and pathologist who is best remembered for naming Paget's disease and who is considered, together with Rudolf Virc ...

first proposed the idea of venous thrombosis causing upper extremity pain and swelling, and

Leopold von Schrötter Leopold Schrötter Ritter von Kristelli, (name often given as Leopold von Schrötter in medical literature) (February 5, 1837 – April 22, 1908) was an Austrian internist and laryngologist born in Graz. He was the son of chemist Anton Schröt ...

later linked the clinical syndrome to thrombosis of the axillary and subclavian veins.L. von Schrötter. Erkrankungen der Gefässe. Nothnagel’s Handbuch der speciellen Pathologie und Therapie, 1901. Volume XV, II. Theil, II. Hälfte: Erkrankungen der Venen. Wien, Hölder, 1899: 533–535.

References

External links

{{DEFAULTSORT:Paget-Schroetter disease Vascular diseases Diseases of veins, lymphatic vessels and lymph nodes