Paget-Schroetter disease overview

Paget-Schroetter disease Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Paget-Schroetter disease from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Chest X Ray

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

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

## Overview

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## Historical Perspective

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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".

## Classification

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

## Pathophysiology

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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.

## Causes

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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.

## Differentiating Paget-Schroetter disease from Other Diseases

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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.

## Epidemiology and Demographics

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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.

## Risk Factors

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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.

## Screening

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

## Natural History, Complications, and Prognosis

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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.

## Diagnosis

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### History and Symptoms

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

### Physical Examination

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

### Laboratory Findings

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

### X-ray

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

### Ultrasound

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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.

### CT scan

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

### MRI

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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.

### Other Imaging Findings

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

### Other Diagnostic Studies

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

## Treatment

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### Medical Therapy

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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.

### Surgery

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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.

### Primary Prevention

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

### Secondary Prevention

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

## References

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