Superior vena cava syndrome (SVCS), or superior vena cava obstruction (SVCO), is a result of obstruction of the superior vena cava. It is a medical emergency and requires immediate diagnosis and treatment. It most commonly occurs by direct invasion of the superior vena cava or compression of the vessel wall by right upper lobe tumours and/or mediastinal lymphadenopathy. It can also occur as a result of thrombosis in the SVC, although this is less common.
Signs and symptoms
- Facial plethora
- Venous distension in the neck and distended veins in the upper chest and arms
- Upper limb oedema
- Light headedness|
Superior vena cava syndrome usually presents more gradually with an increase in symptoms over time as malignancies increase in size or invasiveness.
Approximately 90% of cases are associated with a cancerous tumor that is compressing the superior vena cava. Syphilis
have also been known to cause superior vena cava syndrome. SVCS can be caused by invasion or compression by a pathological process or by thrombosis in the vein itself.
Before antibiotics, untreated infections were the most common cause of SVCS. Now malignancies (in particular, lung cancer) cause most SVCS's. Common cancers that cause SVCS include bronchogenic carcinomas including small cell and non-small cell lung carcinoma, Burkitt lymphoma, lymphoblastic lymphomas, pre-T-cell lineage acute lymphoblastic leukemia (rare), and other acute leukemias.
Symptoms are usually relieved with radiation therapy within one month of treatment. However, even with treatment, 90% die within two and a half years. This relates to the cancerous causes of SVC that are 90% of the cases. The average age of onset of disease is 54 years of age.
The main techniques of diagnosing SVCS are with chest X-rays
(CXR), CT scans
, transbronchial needle aspiration
. CXRs provide the ability to show mediastinal widening and may show the presenting primary cause of SVCS. CT scans should be contrast enhanced and be taken on the neck, chest, lower abdomen and pelvis. They may also show the underlying cause and the extent to which the disease has progressed.
Several methods of treatment are available, mainly consisting of careful drug therapy and surgery. Glucocorticoids
(such as prednisone
) decrease the inflammatory response to tumor invasion and oedema surrounding the tumor. In addition, diuretics
(such as furosemide
) are used to reduce venous return to the heart which relieves the increased pressure.
- 2007 PMID 17476012 -- "Clinical practice. Superior vena cava syndrome with malignant causes." (Wilson LD, N Engl J Med. 2007 May 3;356(18):1862-9.)