Paget-Schroetter disease

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

Spontaneous axillary-subclavian vein thrombosis (ASVT) has been first described by Cruveilhier. Sir James Paget identified the subclavian vein thrombosis in one of his patients. Hughes created the term “Paget-Schroetter”.

There is no established system for the classification of Paget-Schroetter disease.

It is thought that Paget-Schroetter disease is a form of axillary–subclavian vein thrombosis resulted from compression of aforementioned veins. This condition is more common in athletes and/or manual labors. Some studies link Paget-Schroetter disease with anatomical defects involving the thoracic outlet.

Common causes of Paget-Schroetter disease include a repetitious activity of upper extremity, hypertrophied scalenus anterior muscle, presence of osseous exostosis, congenital deformities, and major vein catheterization.

Since early diagnosing and treatment of Paget-Schroetter disease is crucial to halt fatal complications like pulmonary embolism, it is important to differentiate it from other diseases with similar presentations and findings.

Paget-Schroetter disease usually affects young individuals. The incidence of Paget-Schroetter disease is roughly 2.03 per 100,000 people per year.Men are more commonly affected by Paget-Schroetter disease than women.

Common risk factors in the development of Paget-Schroetter include repetitive upper extremity, overdeveloped anterior scalene muscle, congenital bands between the first and second rib and thoracic outlet syndrome.

There is insufficient evidence to recommend routine screening for Paget-Schroetter disease.

The symptoms of Paget-Schroetter syndrome usually develop in the forth decade of life. Common complications include pulmonary embolism, recurrent thrombosis, superior vena cava syndrome and sepsis. Patients with early diagnosis and proper treatments usually have good prognosis.

A positive history of repetitive upper extremity movements in a patient presenting with arm discomfort and edema is suggestive of Paget-Schroetter disease.

Common physical examination findings of Paget-Schroetter disease include edema, tenderness, and dilated veins over the involved upper limb.

Paget-Schroetter disease is not diagnosed based on laboratory findings since except D-dimer, other laboratory values are commonly normal.

There are no specific chest x-ray findings associated with Paget-Schroetter disease.

Paget-Schroetter disease is commonly diagnosed with a history and physical examinations. However, imaging is usually utilized to confirm the diagnose. Duplex ultrasound is an accepted initial test and the gold standard imaging of Paget-Schroetter disease. Since this diagnostic tool is not fully appropriate to exclude the Paget-Schroetter disease, normal duplex ultrasound in a highly suspected patient requires further investigations.

Computed tomography (CT) is useful in the presence of atypical symptoms or in the setting of a normal ultrasound in a highly suspected patient.

Magnetic resonance imaging (MRI) is useful in the presence of atypical symptoms or in the setting of a normal ultrasound in a highly suspected patient.

There are no other imaging findings associated with Paget-Schroetter disease.

Contrast Venography as an invasive diagnostic tool that is usually used when non-invasive methods are indecisive or intervention is required.

An effective treatment approach with lower rate of recurrent thrombus is a combination of anti-coagulation, thrombolytic agents and surgical decompression of the thoracic outlet. Catheter-directed thrombolysis injection has proven to be effective as systemic thrombolytic agents without any worrisome systemic side effects.

Based on pathophysiology of the Paget-Schroetter disease, extrinsic compression and endothelial damage to the vein are among the underlying problems that should be addressed. While thrombolysis only treats acute symptoms, correction surgery decreases recurrence in patients with suspected subclavian vein entrapment. Correction surgery involves both thoracic outlet decompression and restoration of vein patency.

There are no established measures for the primary prevention of Paget-Schroetter disease.

There are no established measures for the secondary prevention of Paget-Schroetter disease.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

Spontaneous axillary-subclavian vein thrombosis (ASVT) has been first described by Cruveilhier. Sir James Paget identified the subclavian vein thrombosis in one of his patients. Hughes created the term “Paget-Schroetter”.

• Spontaneous axillary-subclavian vein thrombosis (ASVT) has been first described by Cruveilhier in 1816.^[1]
• In 1875, Sir James Paget identified the subclavian vein thrombosis in one of his patients. ^[2]
• Von Schroetter was the first one who hypothesized the relation between vascular injury from muscle strain and subclavian vein thrombosis in 1894. ^[3]
• In 1949 Hughes created the term “Paget-Schroetter” and published the first review.^[4]
• The father of a patient used google to diagnose his son’s Paget-Von Schrötter syndrome by querying on the symptoms, and it resulted in one of the first descriptions of “googling for diagnosis”.^[5]

• Lee et al. used a treatment approach started with catheter-directed thrombolysis.
• J. Ernesto Molina, MD, a vascular surgeon at the University of Minnesota, has expressed the following opinion:^[6]
  1. “The currently acceptable treatment for that condition is lytic therapy followed by surgery. The patients were followed up to investigate the rate of recurrence. However, if surgery is not done, the problem will invariably recur. If patients are treated with only anticoagulants and even thrombolytics but no surgery, I expect the recurrence rate to be high.”
  2. “Most patients who suffer this condition do not have any abnormality in their coagulation mechanism” and in fact suffer from “A direct injury to the endothelium,”
  3. “Treatment for Paget-Schroetter syndrome entails the use of thrombolytics followed by surgery to decompress the thoracic inlet and widening of the vein, usually with a vein patch.”
• Molina et al. reported 5 years of 100% vein patency and complete symptom resolution in patients who treated with thrombolysis and first rib resection.^[7]

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

There is no established system for the classification of Paget-Schroetter disease.

• There is no established system for the classification of Paget-Schroetter disease.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

It is thought that Paget-Schroetter disease is a form of axillary–subclavian vein thrombosis resulted from compression of aforementioned veins. This condition is more common in athletes and/or manual labors. Some studies link Paget-Schroetter disease with anatomical defects involving the thoracic outlet.

The subclavian vein passes through a space surrounded by the first rib, the scalenus anterior muscle and the subclavius muscle and tendon.^[1]

• It is though that Paget-Schroetter disease (also known as Effort Thrombosis) is the result of axillary-subclavian vein compression and subsequent thrombosis, and is more common in athletes and/or manual labors.^[2]
• Around 60-80% of cases with Paget-Schroetter disease have a history of repetitious activity of upper extremity.^[3]
• Sports related to higher chance of developing this condition are baseball, softball, wrestling, playing ball, gymnastic, swimming, hockey, rowing, martial arts, backpacking, recreational badminton, weight training and billiards which require repetitive and constant upper extremity activities. ^[4][5]
• Repetitive overhead arm activities (e.g., auto repair and painting) have been reported in a number of cases.^[6]
• Even with normal anatomical structure the Subclavian vein can be compressed through hyper-abduction (more prominent in arm abducted over 90 degrees), extension and/or external rotation.^[7][8]
• In addition to compression, these activities can put tension on the Subclavian vein which lead to micro-trauma of the endothelium and initiate the coagulation cascade.^[9]
• More compression can be seen with hypertrophied Scalenus Anterior or Subclavius muscle.
• Increased bulk of muscles like Pectoralis minor and Subscapularis muscles and presence of osseous exostosis or fibrous bands, as well as congenital or post-trauma bone deformities can cause further compression.^[10]
• Restricted space around the Subclavian vein lead to blood flow stasis and immobilize the Subclavian vein.
• The Subclavian vein becomes more prone to endothelial trauma from arm movements when it’s mobility has been limited.^[11]
• Endothelial trauma followed by thrombosis and recanalization will result in intimal hyperplasia and inflammation, which can be more complicated by venous webs, extensive collateral formation and perivenular fibrosis.^[12]
• There is no clear connection between Effort Thrombosis and hereditary or acquired thrombophilias, however coexisting Thrombophilia can rise the rate of postoperative complications after corrective surgery. ^[13]

• No racial or genetic predisposition has been demonstrated.^[14]

Conditions associated with the Paget-Schroetter disease include:

• Anatomical abnormalities involving the thoracic outlet:
  • Paget-Schroetter disease or Effort Thrombosis is a venous variant of Thoracic Outlet Syndrome.
  • Therefore abnormalities like cervical rib, congenital bands, hypertrophy of scalenus tendons and abnormal insertion of the costoclavicular ligament are associated with this variant of Thoracic Outlet Obstruction.^[15]

• On gross pathology, the following findings can be noted:
  • Presence of cervical rib
  • Hypertrophy of scalenus tendons
  • Congenital bands and abnormal insertion of the costoclavicular ligament could be expected. ^[16]
  • Replacement of loose connective tissue that encircles the vein by dense collagen scar is seen at operation, which is resulted from trauma-induced inflammation and subsequent fibrosis. ^[17]

• No microscopic change is detected on microscopic histopathological analysis.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

Common causes of Paget-Schroetter disease include a repetitious activity of upper extremity, hypertrophied scalenus anterior muscle, presence of osseous exostosis, congenital deformities, and major vein catheterization.

Common causes of Paget-Schroetter disease include:

• Repetitious activity of upper extremity including hyper-abduction, extension and/or external rotation.
• Restricted space around the Subclavian vein or Axillary vein which further augments the compression:
  • Hypertrophied scalenus anterior or Subclavius muscle
  • Increased bulk of muscles like Pectoralis minor and subscapularis muscles
  • Presence of osseous exostosis or fibrous bands
  • Congenital or post-trauma bone deformities
• Iatrogenic causes like major vein catheterisation^[1]
  

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

Since early diagnosing and treatment of Paget-Schroetter disease is crucial to halt fatal complications like pulmonary embolism, it is important to differentiate it from other diseases with similar presentations and findings.

• Other upper limb musculoskeletal disorders(muscle contusion and strain) and soft tissue infections(cellulitis), which also present with painful congestion and edema of the affected arm ^[1]
• Trauma
• Compressive tumors ^[2]
• Cervical radiculopathy
• Spinal stenosis
• First rib anomaly^[3]
• Upper extremity DVT^[4]
• Systemic vascular disease (eg, sickle cell trait/disease)^[5]
• Lymphedema of the upper extremity
• Localized hematoma in biceps muscle
• Superficial phlebitis
  

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

Paget-Schroetter disease usually affects young individuals. The incidence of Paget-Schroetter disease is roughly 2.03 per 100,000 people per year.Men are more commonly affected by Paget-Schroetter disease than women.

• Effort thrombosis seems to be responsible for 1% – 4% of total episodes of venous thrombosis.^[1]
• It has been estimated that the yearly incidence of Paget-Schroetter disease (or Effort Thrombosis) is approximately 3000 – 6000 cases yearly.^[2]
• A study done in Sweden concluded that the yearly incidence of Paget-Schroetter disease is roughly 2.03 per 100,000 people per year. ^[3]

• Paget-Schroetter disease usually involves young individuals and the mean age of onset is the early 30s. ^[4] 

• There is no racial predilection to Paget-Schroetter disease.

• Men are more commonly affected by Paget-Schroetter disease than women.
• The male to female ratio is approximately 2 to 1.^[5]

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

Common risk factors in the development of Paget-Schroetter include repetitive upper extremity, overdeveloped anterior scalene muscle, congenital bands between the first and second rib and thoracic outlet syndrome.

• Common risk factors in the development of Paget-Schroetter or Effort Thrombosis include:
  • Repetitive, prolonged or vigorous upper extremity movements especially, hyperabduction or external rotation of the shoulder joint.
  • Overdeveloped anterior scalene muscle
  • Rudimentary first rib^[1]
  • Presence of cervical rib^[2]
  • Congenital bands between first and second rib
  • Fracture of clavicle with callus formation^[3]
  • Apical tumor of superior sulcus of the lung (Pancoast tumor)^[4]
  • Thoracic outlet syndrome
  • Male sex^[5]

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anahita Deylamsalehi, M.D.[2]

There is insufficient evidence to recommend routine screening for Paget-Schroetter disease.

• There is insufficient evidence to recommend routine screening for Paget-Schroetter disease.

Template:WH Template:WS

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Anahita Deylamsalehi, M.D.[2]

The symptoms of Paget-Schroetter syndrome usually develop in the forth decade of life. Common complications include pulmonary embolism, recurrent thrombosis, superior vena cava syndrome and sepsis. Patients with early diagnosis and proper treatments usually have good prognosis.

• The symptoms of Paget-Schroetter syndrome usually develop in the forth decade of life, and start with symptoms such as shoulder and upper extremity pain and swelling.
• If left untreated, up to 78% of patients with Paget-Schroetter disease may progress to develop residual upper extremity venous obstruction, also continuous symptoms and permanent disability is expected in 41% to 91% and 39% to 68% of untreated patients, respectively.^[1]

• Common complications of Paget-Schroetter disease include:
  • Pulmonary embolism
  • Post-thrombotic syndrome^[2]
  • Recurrent thrombosis
  • Signs and symptoms of superior vena cava syndrome
  • Venous gangrene of upper extremity
  • Signs and symptoms of systemic sepsis secondary to infection of thrombus

• Patients with early diagnosis and proper treatments usually have good prognosis.^[3]
• Up to 90% of patients who received thrombolysis in combination with thoracic outlet decompression surgery experienced desirable outcomes.^[4]
• Without prompt and proper treatment Paget-Schroetter disease is associated with high morbidity.^[5]
• Immediate surgical intervention is related with better prognosis.^[6]

Template:WH Template:WS