Superior Vena Cava Syndrome

The superior vena cava is a short and wide vein that carries blood into the heart. Sometimes this blood vessel can become obstructed and cause superior vena cava syndrome which encompasses a variety of symptoms. Some common characteristics of this syndrome are swelling from a build-up of fluid (edema) in the arms and face and the swelling of other veins inside the chest wall. There can also be swelling of the epiglottis that can make breathing difficult or swelling of the brain which can affect alertness and possibly cause neurological problems. Rarely the swelling can cause obstruction of the airway. Most cases of superior vena cava syndrome are caused by cancer, though it can sometimes have a benign cause.

What Is Superior Vena Cava Syndrome?

Located in the upper chest, the superior vena cava is a vein that collects the blood returning from the arms and head and sends it back into the right atrium of the heart. If a clot forms in the vena cava or if it is compressed then blood flow to the heart is blocked. This results in edema, or swelling, due to the increased pressure in the veins of the arms and face. Like all veins, the superior vena cava has thin walls and can be easily compressed from the structures surrounding it, including the heart, esophagus, trachea, and lungs.

What Are the Symptoms of Superior Vena Cava Syndrome?

Symptoms of superior vena cava syndrome are caused by a build-up of pressure in the vein above a blockage and include:

  • Headache
  • Chest pain
  • Swelling of the chest wall, arms, or face
  • Hoarseness
  • Coughing (including coughing up blood)
  • Widening of the veins in the chest and neck
  • Difficulty breathing
  • Difficulty swallowing
  • Bluish discoloration to the skin of the upper body or face

What Are the Causes of Superior Vena Cava Syndrome?

Cancer is the most common cause for superior vena cava syndrome, particularly cancer in the upper lobe of the right lung. Tumors located in the area of the upper right lobe including lymphoma can also cause the syndrome. The tumors can cause suppression of the superior vena cava, creating pressure above the site of compression. In some cases a blockage due to a blood clot in the blood vessel can cause the syndrome, as well. This can sometimes be a complication due to dialysis, pacemaker wires, and other catheters threaded through the superior vena cava. In rare cases (though it is seen in the less-developed parts of the world) superior vena cava syndrome can be caused by infections such as tuberculosis and syphilis. Sarcoidosis, a condition that causes inflammation throughout the body, can also cause the syndrome.

What Are the Treatments of Superior Vena Cava Syndrome?

Treatment for superior vena cava syndrome can depend on a number of factors, including the severity of the symptoms, the cause of the blockage causing the pressure, the patient’s prognosis, and what the patient prefers to do. Treatment for the syndrome when caused by cancer in the upper lobe of the right lung is typically done prior to chemotherapy or radiation treatment. Common treatments include:

  • Watchful waiting. If there are few symptoms and the patient who has good blood flow throughout the rest of the body will typically be monitored and may not need treatment. In many cases, treatment of the cancer caused blockage is the only treatment needed for superior vena cava syndrome.
  • Chemotherapy.Chemotherapy is typically used to treat small cell lung cancer or lymphoma so if this is the cause of superior vena cava syndrome, then treatment with chemotherapy will shrink the cancer, easing pressure and reversing the syndrome.
  • Radiation therapy.In the case where the blockage is caused by a tumor that has not responded to chemotherapy, then radiation therapy is used to treat the tumor, thus treating the cause of the syndrome.
  • Thrombolysis.Sometimes superior vena cava syndrome can be caused by a thrombus, or blood clot, that forms and partially blocks a vein. In this case a thrombolysis is performed to remove or break up the blood clot and remove the pressure.
  • Stent placement.In the case of a blocked vein, a stent may be inserted to open up the blockage. A stent is a tube-like appliance that allows blood to flow past the blockage in a blood vessel. In some cases the patient will be given an anti-coagulant or blood thinner to prevent further clot formation.
  • Surgery.When the obstruction of the superior vena cava is not caused by cancer, a surgical bypass may be performed to ease the pressure to the vein. Sometimes cancerous tumors causing the obstruction can also be removed through surgery.

Superior Vena Cava Syndrome in Children

Superior vena cava syndrome is rare in children, but it can occur with non-Hodgkin’s lymphoma or due to a blood clot caused by an intravenous catheter. Superior vena cava syndrome in children can be accompanied by a compression of the trachea called superior mediastinal syndrome and is considered the same problem. The compression of the trachea occurs in children because the trachea is far less rigid and is more likely to be squeezed shut. Symptoms are generally the same in children as in adults though additional symptoms can include tiredness, headache, confusion, anxiety, fainting, a sense of fullness in the ears, and vision problems. The situation in children when caused by cancer can be a medical emergency so treatment of the syndrome takes priority to diagnosing the cancer. However the treatment of the cancer eases the syndrome although sometimes a bypass is needed to ease the pressure.

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