Superior vena cava syndrome (Mediastinal syndrome) | |
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Superior vena cava syndrome in a person with bronchogenic carcinoma. Note the swelling of his face first thing in the morning (left) and its resolution after being upright all day (right). | |
Classification and external resources | |
Specialty | cardiology |
ICD-10 | I87.1 |
ICD-9-CM | 459.2 |
DiseasesDB | 12711 |
MedlinePlus | 001097 |
eMedicine | emerg/561 |
MeSH | D013479 |
Superior vena cava syndrome (SVCS), is a group of symptoms caused by obstruction of the superior vena cava (a short, wide vessel carrying circulating blood into the heart). More than 90% of cases of superior vena cava obstruction (SVCO) are caused by cancer - most commonly bronchogenic carcinoma, typically a tumor outside the vessel compressing the vessel wall - but it can, sometimes, have a benign cause. Characteristic features are edema (swelling due to excess fluid) of the face and arms and development of swollen collateral veins on the front of the chest wall. Shortness of breath and coughing are quite common symptoms; difficulty swallowing is reported in 11% of cases, headache in 6% and stridor (a high-pitched wheeze) in 4%. The condition is rarely life-threatening, though edema of the epiglottis can make breathing difficult, and edema of the brain can cause reduced alertness, and in less than 5% of cases of SVCO, severe neurological symptoms or airway compromise are reported.
Shortness of breath is the most common symptom, followed by face or arm swelling.
Following are frequent symptoms:
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, such as bronchogenic carcinoma including small cell and non-small cell lung carcinoma, Burkitt's lymphoma, lymphoblastic lymphomas, pre-T-cell lineage acute lymphoblastic leukemia (rare), and other acute leukemias.Syphilis and tuberculosis 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, although this latter is less common (approximately 35% due to the use of intravascular devices).