Primary deep vein thrombosis of the upper limbs
Primary deep vein thrombosis of the upper limbs (DVTUL) is defined as a thrombosis of the deep veins draining the upper limbs (axillary and subclavian) due to an underlying anatomical abnormality at the thoracic outlet causing compression or repeated venous injuries.
This condition is also called thoracic outlet syndrome (TOS), Paget-Schroetter syndrome, and “effort” thrombosis.
It represents one of the many causes of central thoracic venous obstruction (CTVO) and is estimated to account for between 1 and 4% of all thrombotic CTVO cases. With secondary causes of thrombosis related to central venous cannulation (e.g., central catheter, pacemaker) or prothrombotic states (e.g., thrombophilia, cancer).
Risk factors
Anatomical abnormalities of the thoracic outlet can be congenital or acquired.
Congenital abnormalities include cervical ribs, supernumerary muscles, abnormal tendon insertions, or abnormal muscle or tendon bands.
Acquired abnormalities include bone overgrowth due to bone fracture (usually of the clavicle), or hypertrophy of the anterior scalene or subclavian muscles, often related to repetitive lifting of loads.
Clinical presentation of Deep Vein Thrombosis
Primary deep vein thrombosis of the upper limbs generally manifests in young individuals. Otherwise healthy in the form of sudden and severe arm edema.
Although more commonly associated with deep vein thrombosis of the lower limbs. Pulmonary embolism related to deep vein thrombosis of the upper limbs (primary and secondary) occurs in 4 to 10% of patients.
The clinician should have a high index of suspicion for this disorder when a young patient without triggering factors presents with signs of venous thrombosis. Of the upper limbs, or a pulmonary embolism in the absence of lower limb symptoms.
Diagnosis of Deep Vein Thrombosis
The diagnosis of upper limb venous outlet obstruction (i.e., deep vein thrombosis or venous stenosis). Can be suspected based on clinical presentation but must be confirmed by imaging, usually first by ultrasound.
D-dimer testing is useful for ruling out thrombosis as an etiology but will not exclude compression/venous stenosis without thrombosis as a source of symptoms.
Once the diagnosis of venous outlet obstruction is established. A primary etiology should be sought to identify the underlying anatomical abnormality causing the obstruction.
We perform a standard chest X-ray for all patients to identify any obvious bone abnormalities.
More advanced imaging may be necessary to demonstrate abnormal muscle attachments. Or dynamic venous compression for atypical presentations.
Comprehensive etiological evaluation may include an MRI or CT scan of the shoulder and neck. As well as venous angiography to identify underlying anatomical abnormalities.
Once the cause of the obstruction is identified, appropriate treatment can be determined. Which may include thrombolysis, thrombectomy, angioplasty, or decompressive surgery.
Source: UpToDate