Upper-Extremity Deep Vein and Superior Vena Cava Thrombosis
Emile R. Mohler III
Debabrata Mukherjee
Sanjay Rajagopalan
Upper-extremity deep vein thrombosis (UEDVT) is an increasingly common clinical problem with potential for considerable morbidity. UEDVT usually refers to thrombosis of the axillary and/or subclavian veins, can rarely involve the brachial vein, and may be primary or secondary (Table 23.1). The presence of UEDVT is brought to medical attention due to upperextremity pain and swelling. Extension of the clot to the brachiocephalic veins may result in a syndrome identical to the superior vena cava (SVC) syndrome. In this chapter, the approach to subclavian- axillary and brachiocephalic vein thromboses will be addressed in the first section, while the approach and management of SVC syndrome will be addressed separately in order to deal with etiologic and management features that are specific to these entities. However, it must be recognized that secondary thrombosis of the subclavian and central veins of the neck, such as due to indwelling catheters and lines, is dealt with in a very similar fashion.
CLASSIFICATION OF UPPER-EXTREMITY DEEP VEIN THROMBOSIS
UEDVT typically occurs in the axillary, subclavian, and brachiocephalic veins and is increasingly common owing to the usage of catheters and devices routed through the upper-extremity veins. UEDVT may be classified as primary or secondary (Table 23.1).
Primary Upper-Extremity Thrombosis
Primary UEDVT is characterized as idiopathic or effort thrombosis (Paget-Schroetter syndrome). Patients presenting with idiopathic UEDVT have no immediate underlying disease but may have occult or undetected cancer. The etiology is believed to be repetitive microtrauma to the vein with subsequent thrombosis. The acute thrombosis invariably occurs in an area of chronic compression and stricture of the axillosubclavian vein due to compression between the hypertrophied scalene or subclavius tendon and the first rib. The syndrome typically develops in the dominant arm after strenuous activity such as weight lifting, rowing, or baseball pitching in otherwise healthy young individuals. The repeated trauma to the vein is thought to activate the coagulation cascade, especially if a mechanical compression of the vessel is also present. Occasionally, patients with thoracic outlet obstruction [thoracic outlet syndrome (TOS), compression of the neurovascular bundle involving the brachial plexus, subclavian artery, and subclavian vein] may present with venous thrombosis.
TABLE 23.1 CAUSES OF UPPER-EXTREMITY DVT | ||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||
Secondary Thrombosis
Secondary UEDVT occurs due to intravenous catheters or pacemakers or external compression from a malignancy. Patients may present with limb swelling and discomfort but may be completely asymptomatic and only found to have deep venous thrombosis upon incidental imaging. High fever in the setting of UEDVT suggests septic thrombophlebitis, and an infectious disease evaluation is indicated to identify the infectious cause.
Central Venous Thrombosis Associated with Long-Term Central Venous Instrumentation
Central venous thrombosis is a frequent event in the presence of indwelling central venous catheters, with partial thrombosis occurring in 30 to 45% of cases and complete thrombosis in 5 to 10%. Long-term transvenous access for hemodialysis, chemotherapy, total parenteral nutrition, and transfusions likely accounts for the majority of causes of central venous occlusive disease. Central venous thrombosis rates are equal with longterm and short-term central venous catheters, with the most important determinants of clot formation being the caliber of the catheter, the use of subclavian access route, and the presence of underlying central venous stenosis. Patients undergoing hemodialysis are at a particularly increased risk for the development of central venous thrombosis, much of which is caused by multiple central venous catheterizations and possibly by a downstream effect of the ipsilateral arteriovenous fistula.
CLINICAL FEATURES
Symptoms and Signs of Upper-Extremity Deep Vein Thrombosis
Table 23.2 lists the presenting signs and symptoms of UEDVT. Axillary or subclavian vein thrombosis may sometimes be completely asymptomatic. More often, patients complain of vague shoulder or neck discomfort and arm edema. If thrombosis causes complete obstruction of the SVC, the patient may complain of arm and facial edema, head fullness, blurred vision, or shortness of breath. Patients with TOS may have pain that radiates into
the fourth and fifth digits via the medial arm and forearm, attributable to injury of the brachial plexus. Symptoms may be position dependent and worsen with hyperabduction of the shoulder or lifting. If TOS is suspected, the examiner should palpate the supraclavicular fossa for brachial plexus tenderness, inspect the hand and arm for atrophy, and perform provocative tests, such as Adson’s and Wright’s maneuvers (Table 23.2; also see Chapter 1). Patients with UEDVT may have mild cyanosis of the involved extremity, a palpable tender cord, arm and hand edema, supraclavicular fullness, jugular venous distension, and possibly dilated cutaneous collateral veins over the chest or upper arm. If a central venous catheter is present, one or multiple ports may be occluded. One should remember that the signs and symptoms of UEDVT are nonspecific and may often occur in patients with lymphedema, neoplastic compression of the blood vessels, muscle injury, or superficial vein thrombosis. Therefore, it is important
to confirm or exclude the diagnosis with objective testing, as appropriate diagnosis is crucial to management of these patients.
the fourth and fifth digits via the medial arm and forearm, attributable to injury of the brachial plexus. Symptoms may be position dependent and worsen with hyperabduction of the shoulder or lifting. If TOS is suspected, the examiner should palpate the supraclavicular fossa for brachial plexus tenderness, inspect the hand and arm for atrophy, and perform provocative tests, such as Adson’s and Wright’s maneuvers (Table 23.2; also see Chapter 1). Patients with UEDVT may have mild cyanosis of the involved extremity, a palpable tender cord, arm and hand edema, supraclavicular fullness, jugular venous distension, and possibly dilated cutaneous collateral veins over the chest or upper arm. If a central venous catheter is present, one or multiple ports may be occluded. One should remember that the signs and symptoms of UEDVT are nonspecific and may often occur in patients with lymphedema, neoplastic compression of the blood vessels, muscle injury, or superficial vein thrombosis. Therefore, it is important
to confirm or exclude the diagnosis with objective testing, as appropriate diagnosis is crucial to management of these patients.
TABLE 23.2 PRESENTING SIGNS AND SYMPTOMS OF UEDVT | ||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| ||||||||||||||||||||
DIAGNOSIS
Diagnostic Imaging
The initial diagnostic evaluation technique of choice for diagnosing UEDVT is duplex ultrasound as this technique is noninvasive with a high sensitivity and specificity. A limitation of this technique is potential acoustic shadowing from the clavicle, which may impair visualization of a short segment of the subclavian vein and result in a false-negative study. Further, extension of a clot to the SVC may be inadequately visualized. CT may enable visualization of a proximal clot. An increasingly utilized alternative to x-ray contrast venography is magnetic resonance venography (MRV), which affords an accurate method to noninvasively detect thrombosis in the central thoracic veins, such as the SVC and brachiocephalic veins. The relative advantages and disadvantages of various imaging modalities are listed in Table 23.3. Usually, x-ray contrast venography is considered the gold standard for evaluation of upper-extremity venous thrombosis, but it has limitations. These limitations include the use of iodinated contrast agent with all the associated problems, potential difficulty in cannulating the vein in an edematous arm, and exposure to radiation, especially if this occurs in pregnant women. Venography is typically done as a prelude to an intervention, such as catheter-directed thrombolysis (CDT) or venoplasty.
TABLE 23.3 IMAGING MODALITIES USED TO DIAGNOSE UEDVT WITH RELATIVE ADVANTAGES AND DISADVANTAGES | |||||||||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
|
