Superior Vena Cava Syndrome

Superior vena cava syndrome (SVCS) is a process characterized by obstruction of venous return in the thoracic portion of the SVC and is associated with facial or upper extremity swelling. This condition is most commonly seen in patients with malignancy due to hypercoagulable state or in patients with intrathoracic neoplasms that compress the SVC. The diagnosis is established clinically, with imaging studies performed to identify the causal lesion. In an immunocompromised host, tuberculosis or histoplasmosis should be considered. Less common causes include goiter, fibrosis, and aortic valve replacement. Iatrogenic causes include pacer wires, catheters, and central lines. Although symptoms may have a gradual onset, they may rapidly progress to become life threatening.

Causes

More than 95% of all cases of SVCS are associated with cancers involving the upper chest. The cancers most commonly associated with SVCS are advanced lung cancers, which account for nearly 80% of all cases of SVCS, and lymphoma. Cancers that have spread (metastasized) to the chest, such as metastatic breast cancer to the chest and metastatic testicular cancer to the chest have also been shown to cause SVCS.

Other causes of SVCS include: the formation of a blood clot in the superior vena cava, enlargement of the thyroid gland, tuberculosis, and sarcoidosis.

Symptoms

• Swelling of face, upper extremities, upper chest
• Dyspnea
• Cough
• Chest pain
• Difficulty swallowing
• Hoarseness
• Stridor
• Headache
• Nasal stuffiness
• Tongue swelling
• Nausea
• Light headedness
• Can be occult

Signs

• Facial edema ++++
• Jugular venous distention +++
• Distention of thoracic veins +++
• Dyspnea +++
• Facial plethora ++
• Upper extremity edema ++
• Cyanosis ++
• Paralysis of true vocal cords
• Papilledema
• Syncope
• Horner's syndrome (rare)

Treatment

The treatment of superior vena cava syndrome is directed toward the exact underlying cause. Therefore, treatment might include radiation treatment, antibiotics, chemotherapy, clot-busting (thrombolytic) drugs, blood thinners (anticoagulation), and balloon angioplasty, and even surgery.

Other short-term treatments, aimed at reducing symptoms, include raising the patient’s head, giving corticosteriods to reduce swelling, or using diuretics to eliminate excess fluid from the body.

Prevention

SVCS may be prevented by early medical intervention to halt and/or reverse the cancer which, in a later stage, would have lead to SVCS.