Superior vena cava syndrome Classification and external resources
Diagram showing the superior vena cava, the affected vein in SVCS.
ICD-10	I87.1
ICD-9	459.2
DiseasesDB	12711
MedlinePlus	001097
eMedicine	emerg/561
MeSH	D013479

Superior vena cava syndrome (SVCS), or superior vena cava obstruction (SVCO), is usually the result of the direct obstruction of the superior vena cava by malignancies- such as compression of the vessel wall by right upper lobe tumors and/or mediastinal lymphadenopathy. The most common malignancies that cause SVCS is non small cell lung cancer and small-cell lung cancer. It can also occur as a result of thrombosis in the SVC, although this is less common (approximately 35% due to the use of intravascular devices).¹ It is often clinically striking but very seldomly requires emergency intervention. Clinical symptoms may develop over several weeks or longer. Increased venous pressure leads to edema of the head, neck, and arms and may turn the patient a shade of blue with cyanosis. Other common symptoms include distention in the neck and chest veins and flushing. Edema may impede the function of the larynx or pharynx resulting in cough, dyspnea, dysphagia, and stridors. Cerebral edema may lead to mental status changes such as confusion, coma, or migraines. Cerebral edema is rare, but if it occurs it may be fatal. ¹²

Signs and symptoms

• Dyspnea¹
• Headache¹
• Facial plethora¹
• Venous distension in the neck and distended veins in the upper chest and arms¹
• Upper limb oedema¹
• Lightheadedness|³
• Cough³

Superior vena cava syndrome usually presents more gradually with an increase in symptoms over time as malignancies increase in size or invasiveness.³

Etiology

Approximately 90% of cases are associated with a cancerous tumor that is compressing the superior vena cava.³ Syphilis and tuberculosis have also been known to cause superior vena cava syndrome.³ SVCS can be caused by invasion or compression by a pathological process or by thrombosis in the vein itself.³

Before antibiotics, untreated infections were the most common cause of SVCS. Now malignancies (in particular, lung cancer) cause most SVCS's.³ 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.³

Prognosis

Symptoms are usually relieved with radiation therapy within one month of treatment.³ However, even with treatment, 90% die within two and a half years.³ 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.³

Diagnosis

The main techniques of diagnosing SVCS are with chest X-rays (CXR), CT scans, transbronchial needle aspiration at bronchoscopy and mediastinoscopy.¹ CXRs provide the ability to show mediastinal widening and may show the presenting primary cause of SVCS.¹ CT scans should be contrast enhanced and be taken on the neck, chest, lower abdomen and pelvis.¹ They may also show the underlying cause and the extent to which the disease has progressed.¹

Treatment

Several methods of treatment are available, mainly consisting of careful drug therapy and surgery.³ Glucocorticoids (such as prednisone or methylprednisolone) decrease the inflammatory response to tumor invasion and oedema surrounding the tumor.³ In addition, diuretics (such as furosemide) are used to reduce venous return to the heart which relieves the increased pressure.³

See also

• Pemberton's sign

References

• Wilson LD, Detterbeck FC, Yahalom J (May 2007). "Clinical practice. Superior vena cava syndrome with malignant causes". N Engl J Med. 356 (18): 1862–9. doi:10.1056/NEJMcp067190. PMID 17476012. 

External links

• SVC syndrome - CT images - Toshiba-Europe.com.