Diagnosis and management of superior vena cava syndrome

Background: Superior vena cava (SVC) syndrome, first described in 1757 by William Hunter (1), refers to a constellation of clinical symptoms caused by obstruction of the SVC. This obstruction is nearly always (>85%) attributable to advanced malignancy (2, 3), most commonly lung cancer. In fact, SVC syndrome affects 3% to 4% of patients with bronchogenic cancer (4). Other primary thoracic malignancies, lymphoma and metastatic disease (particularly from breast and testicular primaries) have also been implicated in SVC syndrome either secondary to extrinsic compression of the SVC or due to direct tumor invasion (2). Benign causes of SVC syndrome include venous stenoses, thrombosis (secondary to vascular access catheters and invasive monitoring devices), extrinsic compression from thoracic aortic aneurysms and mediastinal fibrosis from granulomatous disease (5). The diagnosis of SVC syndrome is initially made clinically. SVC syndrome is characterized by congestion and swelling of the face and upper thorax, with distended superficial chest veins. Other associated symptoms include dyspnea, hoarseness, dysphagia, severe headache and cognitive dysfunction (6, 7). The most severe complications of SVC syndrome include glottic edema and venous thrombosis in the central nervous system (venous stroke). Contrast-enhanced computed tomography (CT) of the chest with vascular reconstruction images should be obtained in these patients, as it can both confirm the site of SVC obstruction as well as delineate the cause of the obstruction (8). Alternatively, magnetic resonance imaging (MRI) can be obtained in those patients with contraindications to CT. The gold standard for diagnosing SVC syndrome is venography.