Cardiopulmonary Syndromes (PDQ®): Supportive care - Patient Information [NCI] - Superior Vena Cava Syndrome in Children
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 the trachea (windpipe) can quickly become blocked. In adults, the windpipe is fairly stiff, but in children, it is softer and can more easily be squeezed shut. Also, a child's windpipe is narrower, so any amount of swelling can cause breathing problems. Squeezing of the trachea is called superior mediastinal syndrome (SMS). Because SVCS and SMS usually happen together in children, the two syndromes are considered to be the same.
The most common symptoms of SVCS in children are a lot like those in adults.
Common symptoms include the following:
There are other less common but more serious symptoms:
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 during use of an intravenous catheter (flexible tube used to put fluids into or take blood out of a vein) in the superior vena cava.
SVCS in children may be diagnosed and treated before a 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. Even if doctors think cancer is causing SVCS, a biopsy may not be done. This is because the lungs and heart of a child with SVCS may not be able to handle the anesthesia needed. Other imaging tests may be done to help find out if anesthesia can be safely used. In most cases, treatment will begin before a diagnosis of cancer is made.
The following treatments may be used for SVCS in children:
- Radiation therapy
Radiation therapy is usually used to treat a tumor that is blocking the vein. After radiation therapy, there may be more trouble breathing because swelling narrows the windpipe. A drug to reduce swelling may be given.
Anticancer drugs, steroids, or other drugs may be used. If the tumor does not respond, it may be benign (not cancer).
This may include surgery to bypass (go around) the blocked part of the vein or to place a stent (thin tube) to open the vein.