General Information about Superior Vena Cava Syndrome
Causes of Superior Vena Cava Syndrome
Diagnosis of Superior Vena Cava Syndrome
Managing Superior Vena Cava Syndrome
Social Considerations of Superior Vena Cava Syndrome
Superior Vena Cava Syndrome in Children

General Information about Superior Vena Cava Syndrome

Superior vena cava syndrome (SVCS) is a group of symptoms that occur when the superior vena cava becomes partially blocked.

The right atrium (chamber) of the heart receives blood from two major veins: the superior (upper) vena cava and the inferior (lower) vena cava.

• The superior vena cava returns blood from the upper body to the heart.
• The inferior vena cava returns the blood from the lower body to the heart.

The superior vena cava is thin-walled, and the blood is under low pressure. If a tumor forms in the chest or nearby lymph nodes become swollen (as from lymphoma), the superior vena cava can be squeezed. Blood flow slows. Complete blockage of the vein can occur. Sometimes, the other veins can become larger and take over for the superior vena cava if it is blocked, but this takes time. Superior vena cava syndrome (SVCS) is the group of symptoms that occur when this vein is partially blocked.

The location of the blocked area and how fast the blockage occurs affect the symptoms.

The symptoms will be more severe if the vein becomes blocked quickly. This is because the other veins do not have time to widen and take over the increased blood flow from the superior vena cava.

The location of the blocked area also affects how severe the symptoms will be:

• If the blockage is above where the superior and inferior vena cava veins join, other veins can become larger over time and take over the increased blood flow. The symptoms may be milder.
• If the blockage occurs below where the superior vena cava and inferior vena cava meet, the blood must be returned to the heart by the veins in the upper abdomen and the inferior vena cava, which require higher pressure. Symptoms may be more severe.

Common symptoms of SVCS include breathing problems and coughing.

The most common symptoms are these:

• Problems breathing.



• Coughing.



• Swollen face, neck, upper body, and arms.

Less common symptoms include the following:

• Hoarse voice.



• Chest pain.



• Problems swallowing and/or talking.



• Coughing up blood.



• Swollen veins in the chest or neck.



• Bluish color to the skin.



• Drooping eyelid.

Superior vena cava syndrome (SVCS) is usually caused by cancer. In adults, SVCS most commonly occurs with lung cancer or non-Hodgkin lymphoma. A tumor in the chest or swollen lymph nodes can press on the superior vena cava, blocking the blood flow. There are other less common causes for the superior vena cava to become blocked:

• A blood clot that forms as a side effect from the use of intravenous catheters (a flexible tube used to put fluids into or take blood out of a vein). A clot may also form as a side effect of pacemaker wires.



• Infection in the chest that causes affected tissues to become thick and hard.



• Other cancers, including metastatic breast cancer, metastatic germ cell tumors, colon cancer, esophageal cancer, Kaposi sarcoma, fibrous mesothelioma (cancer of the epithelium), and Hodgkin lymphoma.



• Bechet syndrome (a disease of the immune system).



• Tumors in the thymus or thyroid.



• Sarcoidosis (a disease of the lymph nodes that acts like tuberculosis).

The following tests may be done to diagnose SVCS and find the location of the blockage:

• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A CT scan of the chest will be done to diagnose SVCS.
• Venography: A procedure to x-ray veins. A contrast dye is injected into the veins to outline them on the x-rays.
• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
• Ultrasound: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.

The type of cancer can affect the type of treatment needed; for this reason, a diagnosis of suspected cancer should be made before treatment of SVCS is begun. Unless the airway is blocked or the brain is swelling, waiting to start treatment while a diagnosis is made usually presents no problem in adults. If lung cancer is suspected, a sputum sample and a biopsy may be taken.

This summary is about treatment for superior vena cava syndrome (SVCS) caused by cancer. Treatment will depend on the following:

• The type of cancer.
• The cause of the blockage.
• How severe the symptoms are.
• The prognosis (chance of recovery).
• The patient's wishes.

Treatment of SVCS may include the following:

Watchful waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment unless symptoms appear or change. A patient who has good blood flow through other veins and mild symptoms may not need treatment.

The following may be used to relieve symptoms and keep the patient comfortable:

• Keeping the upper body raised higher than the lower body.
• Corticosteroids (drugs that reduce swelling).
• Diuretics (drugs that make excess fluid pass from the body in urine). Patients taking diuretics are closely monitored because these drugs can cause dehydration (loss of too much fluid from the body).

Radiation therapy

If the blockage of the superior vena cava is caused by a tumor that is not sensitive to chemotherapy, radiation therapy may be given. Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

Chemotherapy

Chemotherapy is the usual treatment for tumors that respond to anticancer drugs, including small cell lung cancer and lymphoma. This treatment would not be changed for patients who have SVCS. Chemotherapy uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping the cells from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy).

Thrombolysis

SVCS may occur when a thrombus (blood clot) forms in a partially blocked vein. Thrombolysis is a method used to break up and remove blood clots. This may done using a drug put directly into the clot, through a catheter, or by a thrombectomy (the use of a device inserted into the vein).

Stent placement

A stent may be used to open up the blocked vein. A stent is a tube-like device that is inserted into the blocked area of a vein to allow blood to pass through. This helps most patients. Patients may also receive an anticoagulant to keep more blood clots from forming.

Surgery

Surgery to bypass (go around) the blocked part of the vein is sometimes used for cancer patients, but is used more often for patients without cancer.

Superior vena cava syndrome is serious and the symptoms can be upsetting to the patient and family. It is important that patients and family members receive information about the causes of superior vena cava syndrome and how to treat it. This can help relieve anxiety over symptoms such as swelling, trouble swallowing, coughing, and hoarseness.

When a patient has chosen not to receive aggressive treatment because of terminal cancer, palliative treatment can help keep the patient comfortable by relieving symptoms. Patients and family members can be taught how to provide palliative care to relieve symptoms and improve quality of life.

Superior vena cava syndrome in a child is a serious medical emergency because the child's windpipe can become blocked.

Superior vena cava syndrome (SVCS) in children can be life threatening. This is because blockage of the child's trachea (windpipe) can quickly occur along with SVCS. In adults, the windpipe is fairly hard, but in children, it is softer and can more easily be squeezed shut. Also, the diameter of a child's windpipe is smaller, so any amount of swelling can cause breathing problems. Squeezing of the trachea is called superior mediastinal syndrome (SMS). Because SVCS and SMS often occur together in children, the two syndromes are considered to be the same.

The most common symptoms of SVCS in children are similar to those in adults.

Common symptoms include the following:

• Coughing.
• Hoarseness.
• Problems breathing.
• Chest pain.

There are other less common but more serious symptoms:

• Fainting.
• Anxiety.
• Confusion.
• Tiredness.
• Headache.
• Vision problems.
• A sense of fullness in the ears.

The causes, diagnosis, and treatment of SVCS in children are not the same as in adults.

The most common cause of SVCS in children is non-Hodgkin lymphoma.

SVCS in children is rare; the most common cause is non-Hodgkin lymphoma. As in adults, SVCS may also be caused by a blood clot that forms as a side effect of using an intravenous catheter.

SVCS in children may be diagnosed and treated before a definite diagnosis of cancer is made.

A physical exam, chest x-ray, and medical history are usually all that are needed to diagnose superior vena cava syndrome in children. If cancer is suspected, a biopsy is not done unless the lungs and heart of the child with SVCS are able to handle the anesthesia needed. Other imaging tests may be done to help determine if anesthesia can be safely used. In most cases, treatment will begin before a definite diagnosis of cancer is made.

It is important that treatment begins right away.

The following treatments may be used for SVCS in children:

• Radiation therapy

  Radiation therapy is usually used to treat a tumor that is causing the blocked vein. After radiation therapy, breathing may become more difficult because swelling narrows the windpipe. A drug to reduce swelling may be given.




• Drugs

  Anticancer drugs, steroids, and/or other drugs may be used. If the tumor does not respond, it may be benign (not cancer).




• Surgery

  This may include surgery to bypass (go around) the blocked part of the vein or to place a stent to open the vein.

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