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, such as
thymomaIn medicine , thymoma is a tumor originating from the thymus. It consists of any type of thymic epithelial cell as well as lymphocytes that are usually abundant and probably not neoplastic. Thymoma usually is benign, and frequently encapsulated; when occasionally malignant, then it is invasive:...
and/or mediastinal lymphadenopathy. The most common malignancies that cause SVCS is bronchogenic carcinoma. It can also occur as a result of
thrombosisThrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot, because the first step in repairing it is to prevent loss of blood...
in the SVC, although this is less common (approximately 35% due to the use of intravascular devices).
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, such as
thymomaIn medicine , thymoma is a tumor originating from the thymus. It consists of any type of thymic epithelial cell as well as lymphocytes that are usually abundant and probably not neoplastic. Thymoma usually is benign, and frequently encapsulated; when occasionally malignant, then it is invasive:...
and/or mediastinal lymphadenopathy. The most common malignancies that cause SVCS is bronchogenic carcinoma. It can also occur as a result of
thrombosisThrombosis is the formation of a blood clot inside a blood vessel, obstructing the flow of blood through the circulatory system. When a blood vessel is injured, the body uses platelets and fibrin to form a blood clot, because the first step in repairing it is to prevent loss of blood...
in the SVC, although this is less common (approximately 35% due to the use of intravascular devices). SVC thrombosis may occur secondary to
polycythemia veraPolycythemia vera is a blood disorder in which the bone marrow makes too many red blood cells. Polycythemia vera may also result in the overproduction of white blood cells and platelets...
. 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.
Signs and symptoms
- Dyspnea
Dyspnea or dyspnoea , from Latin dyspnoea, from Greek dyspnoia from dyspnoos, shortness of breath) or shortness of breath is a debilitating symptom that is the experience of unpleasant or uncomfortable respiratory sensations...
- Headache
In medicine a headache or cephalalgia is a symptom of a number of different conditions of the head. Some of the causes are benign while others are medical emergencies.There are a number of different classification systems for headaches...
- Facial edema
Edema or oedema , formerly known as dropsy or hydropsy, is an abnormal accumulation of fluid beneath the skin, or in one or more cavities of the body...
- Venous distension in the neck and distended veins in the upper chest and arms
- Upper limb edema
- Lightheadedness
- Cough
Superior vena cava syndrome usually presents more gradually with an increase in symptoms over time as malignancies increase in size or invasiveness.
Etiology
Approximately 90% of cases are associated with a cancerous tumor that is compressing the superior vena cava.
SyphilisSyphilis is a sexually transmitted disease caused by the spirochetal bacterium Treponema pallidum subspecies pallidum. The route of transmission of syphilis is almost always through sexual contact, although there are examples of congenital syphilis via transmission from mother to child in utero.The...
and
tuberculosisTuberculosis is a common and often deadly infectious disease caused by mycobacteria...
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
antibioticIn common usage, an antibiotic is a substance or compound that kills or inhibits the growth of bacteria...
s, untreated infections were the most common cause of SVCS. Now
malignanciesCancer is a class of diseases in which a group of cells display uncontrolled growth , invasion , and sometimes metastasis...
(in particular,
lung cancerLung cancer is a disease of uncontrolled cell growth in tissues of the lung. This growth may lead to metastasis, which is the invasion of adjacent tissue and infiltration beyond the lungs. The vast majority of primary lung cancers are carcinomas of the lung, derived from epithelial cells...
) 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 leukemiaAcute lymphoblastic leukemia , is a form of leukemia, or cancer of the white blood cells characterized by excess lymphoblasts.Malignant, immature white blood cells continuously multiply and are overproduced in the bone marrow. ALL causes damage and death by crowding out normal cells in the bone...
(rare), and other acute leukemias.
Prognosis
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.
Diagnosis
The main techniques of diagnosing SVCS are with chest X-rays (CXR),
CT scansComputed tomography is a medical imaging method employing tomography created by computer processing. Digital geometry processing is used to generate a three-dimensional image of the inside of an object from a large series of two-dimensional X-ray images taken around a single axis of rotation.CT...
, transbronchial needle
aspirationAspiration may refer to:*Aspiration , the release of a strong burst of air after some obstruents*Naturally-aspirated engine, an internal combustion engine that relies on atmospheric pressure for air intake**Supercharger.**Turbocharger....
at
bronchoscopyBronchoscopy is a technique of visualizing the inside of the airways for diagnostic and therapeutic purposes. An instrument is inserted into the airways, usually through the nose or mouth, or occasionally through a tracheostomy. This allows the practitioner to examine the patient's airways for...
and
mediastinoscopyMediastinoscopy is a surgical procedure that enables visualization of the contents of the mediastinum, usually for the purpose of obtaining a biopsy...
. 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.
Treatment
Several methods of treatment are available, mainly consisting of careful drug therapy and surgery. Glucocorticoids (such as
prednisonePrednisone is a synthetic corticosteroid drug that is particularly effective as an immunosuppressant, and affects virtually all of the immune system. It is used to treat certain inflammatory diseases and cancers, but has significant adverse effects. It is usually taken orally but can be delivered...
or
methylprednisoloneMethylprednisolone is a synthetic glucocorticoid drug. It is sold in the USA and Canada under the brand names Phocenta, Medrol, Solu-Medrol and Cadista. It is also available as a generic drug....
) decrease the inflammatory response to tumor invasion and oedema surrounding the tumor. In addition, diuretics (such as
furosemideFurosemide or frusemide is a loop diuretic used in the treatment of congestive heart failure and edema. It is most commonly marketed by Sanofi-Aventis under the brand name Lasix...
) are used to reduce venous return to the heart which relieves the increased pressure.