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 in children are similar to those in adults and include coughing, hoarseness, difficulty breathing, and chest pain. Other less common but more serious symptoms include anxiety, confusion, tiredness, headache, vision problems, a sense of fullness in the ears, and fainting.
Superior vena cava syndrome is rare in children. The cause, diagnosis, and treatment of superior vena cava syndrome in children is not the same as it is in adults. While the most common cause of superior vena cava syndrome in adults is lung cancer, in children it is non-Hodgkin's lymphoma. As with adults, a possible noncancerous cause is a blood clot associated with an intravenous catheter.
The diagnosis in children is typically made with a physical exam, medical history, and chest X-ray. If lymphoma or another cancer is suspected, a biopsy is helpful for the diagnosis, but may be too dangerous for the child. Children with superior vena cava syndrome do not tolerate general anesthesia well because of its effect on the heart and lungs.
When cancer is the cause of superior vena cava syndrome, the situation may be a medical emergency, with no time to diagnose the cancer with tissue. In most cases, the best approach is to start treatment before a definite diagnosis is made. Radiation therapy is usually used to treat the tumor. After radiation therapy, breathing may become more difficult because of the rapid swelling that narrows the windpipe. In these situations, it may be necessary to give a drug that reduces swelling.
Along with radiation, chemotherapy and steroids may be used. If the tumor does not respond, it may not be cancerous. If necessary, surgery may be performed. Surgery may include a bypass to go around the blocked part of the vein. A stent may also be placed to open the vein.
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