Axillary and subclavian vein thrombosis • LITFL • FFS

Axillary and subclavian vein thrombosis is an important diagnosis to make. The incidence of pulmonary embolism (PE) and long-term sequelae is high in untreated cases. It is associated with PE in 6–30% of cases and therefore requires anticoagulation.

Thrombosis can be primary (Paget-Schroetter syndrome / effort thrombosis / spontaneous upper extremity venous thrombosis) or secondary to identifiable causes.

Routine anticoagulation is recommended in all cases. For young, fit patients, specialist advice regarding thrombolysis, imaging, and surgery may significantly improve outcomes. Early referral to vascular surgery is recommended when the cause is uncertain.

History

Paget-Schroetter syndrome was first described by Sir James Paget (1875) and later by Leopold Von Schrötter (1884).
Paget-Schroetter syndrome and also known as “effort” or “spontaneous” upper extremity venous thrombosis.

Epidemiology

Incidence has increased due to catheter use in patients with cancer and chronic conditions.

Pathophysiology

Causes

1. Primary / Spontaneous Thrombosis:

Often effort-induced in predisposed individuals
Strenuous effort with the arm in the overhead or hyper-abducted position is thought to be a risk factor, particularly in younger elite sports people.
Implicated sports include cricket, tennis, wrestling, weight-lifting, water polo, gymnastics, baseball, and wood chopping

2. Secondary Causes:

Iatrogenic: PICC lines, transvenous pacemakers, post-mastectomy, radiotherapy.
IV drug use
Thoracic Inlet Anomalies: Cervical ribs, congenital fibromuscular bands, anomalous subclavius or anterior scalene muscles
Prothrombotic States: Malignancy, hematological disorders.
Venous stenosis from previous scarring.

Complications

Pulmonary embolism (up to 30%).
Chronic recurrent pulmonary embolism → pulmonary hypertension and right sided heart failure.
Chronic venous insufficiency (swelling, pain, SVC syndrome).

Clinical Features

History

Recent trauma or strenuous arm use
History of thromboembolism
OCP use in women
Symptoms of PE (chest pain, SOB)
Venous cannulation history

Examination

Non-pitting upper limb edema
Cyanosis
Collateral vein dilation (Urschel’s sign)
Raised hand test – persistent venous distension
Signs of SVC obstruction (if extensive)
Gradual symptom resolution over months (collateral formation)
Normal arterial exam

Differential Diagnoses

Lymphedema
Trauma

Investigations

Bloods: FBE, U&Es, glucose, coags, D-dimer (to exclude thrombosis as aetiology)
CXR: Look for malignancy, cervical ribs, other obstructive lesions
Ultrasound: Color Doppler for initial assessment
CT Chest/Venography: Confirm diagnosis, assess thoracic inlet, rule out PE
MRI/MRA: Alternative if contrast contraindicated

Management

General Principles

Aggressive early intervention can restore venous patency, especially if thrombolysis is performed within 2 weeks of symptom onset. Late decompression may still benefit select cases.

Treatment Options

An aggressive approach that includes a combination of thrombolysis and thoracic outlet decompression with or without venoplasty (percutaneous, open) appears to improve long-term outcomes in patients with primary upper extremity deep vein thrombosis, particularly those with acute, moderate-to-severe symptoms

1. Anticoagulation

All patients: therapeutic clexane + warfarin (or DOAC under haematology supervision)
Duration: At least 3 months

2. Thrombolysis

Consider for effort thrombosis < 2 weeks with extensive disease
Not useful in chronic thrombosis
Initiate IV heparin pending vascular review

3. Surgery

Indications: Contraindication to thrombolysis, recurrent thrombosis, recurrent PE, anatomical causes
Procedures: Embolectomy, angioplasty, stenting, rib/fibromuscular band resection