Lymphedema affects millions of Americans and hundreds of millions of people worldwide every year. It can be an incredibly painful and debilitating disease that often goes untreated for years or even decades due to a lack of awareness within the medical community. Fortunately, with the right diagnosis, many of the symptoms of lymphedema can be lessened with surgery and therapy, and patients can significantly improve their quality of life. 

What is lymphedema?

Lymphedema is a disorder of the lymphatic system. Part of the immune system, the lymphatic system is responsible for circulating lymphatic fluid — a toxic substance. Lymphedema occurs when the lymphatic fluid is blocked, often in an arm or leg, and can’t be circulated and drained properly. It begins to leak into the surrounding fat tissues and the toxic substance remains in those fat tissues, which become chronically inflamed. In more advanced lymphedema, this inflammation results in deposits of additional solids and fat in the affected area, causing the arm or leg to swell and become fibrose, or hard. These solid deposits are permanent, so no amount of diet or exercise will reduce the size of the affected arm or leg. 

What causes lymphedema?

There are two types of lymphedemas, primary and secondary lymphedema. Though they tend to look similar and require the same treatment, they are caused by different events. Primary lymphedema, also called congenital lymphedema, is much less common in the United States than secondary lymphedema. It occurs spontaneously and is a result of intrinsic problems with the lymphatic vessels. Most patients notice the swelling after a minor injury like an ankle sprain or a hormonal disturbance like pregnancy. 

Secondary lymphedema, which is far more common, can be caused by a variety of events. Worldwide, filariasis — a parasitic infection transmitted by mosquitoes — is the most common cause of lymphedema. However, filariasis is incredibly rare in developed countries. In the United States and other developed countries, secondary lymphedema is often caused by surgery (such as the removal of a lymph node, vascular surgery or knee surgery), radiation, infection, trauma (like a bike accident or burn) often as part of treatment for cancer. 

Who is most at risk for developing lymphedema?

Though men and women of all ages can suffer from lymphedema, it primarily affects women 30 to 65 years old. It is commonly found in women who have been treated for breast cancer, especially those who have had lymph nodes surgically removed or who have experienced radiation or chemotherapy.

For patients with secondary lymphedema, some notice symptoms immediately following a surgery while others don’t experience symptoms until many years later. Those with primary lymphedema may have noticed symptoms during puberty or after pregnancy but didn’t understand what they were experiencing. Because there has traditionally been a lack of awareness around lymphedema, many women do not receive the official diagnosis until later in life. 

What are the most common lymphedema symptoms?

One of the main symptoms of lymphedema is severe swelling in one arm or one leg. Though lymphedema tends to affect only one extremity, it can be present in multiple areas. The swelling can become so severe that it causes a significant loss of mobility for the patient, which makes normal day-to-day activities very difficult.

Unfortunately, patients with lymphedema are also more prone to cellulitis infections in the areas of their body affected by lymphedema. These skin infections can be incredibly dangerous if left untreated. Any knicks or bites on the affected arm or leg need to be treated with extreme care to avoid a cellulitis infection. If a patient experiences multiple cellulitis infections in one arm or leg and is also experiencing swelling, they should consider getting tested for lymphedema. 

How is lymphedema diagnosed?

To diagnose lymphedema, a specialized clinician will likely start with a physical exam and may use a diagnostic imaging test called a lymphoscintigraphy. This nuclear medicine test takes three to four hours to complete, and in addition to confirming a lymphedema diagnosis, it provides images on how and where the lymphatics drain. 

During the test, the patient receives an injection of tracer particles and can then leave the lab for a few hours while the tracer particles progress through their lymphatic system. The patient then returns to the lab for the technician to take a set of images to investigate the flow in the lymphatic channels. It’s typically recommended that the patient do the full body scan to ensure the blockage isn’t present in areas that haven’t yet become noticeably swollen. 

Why is a lymphedema diagnosis so commonly missed?

Because the disease is rare and limited lymphedema literature exists, there is a serious lack of awareness around lymphedema in the medical community. Many doctors don’t understand lymphedema and its symptoms and therefore fail to diagnose it when patients present symptoms. Very often, a physician will treat the cellulitis with antibiotics and send the patient on their way without recommending a specialist. 

And even when patients are diagnosed with lymphedema, they aren’t often sent to the right place to receive treatment. That’s due, in part, to the fact that so few experienced lymphedema surgeons in the United States. Patients are referred to physical therapists or if available, certified lymphedema therapists, who can provide temporary relief from some lymphedema symptoms, but can, over time, fail to provide more significant long-term relief. 

Treatment for lymphedema 

While there is no true cure for lymphedema, surgery can significantly improve the symptoms and effects of the disease for those with primary or secondary lymphedema. Patients suffering from lymphedema should look for a board-certified plastic surgeon who specializes in microsurgery and/or super-microsurgery to get the appropriate lymphedema surgery. 

Lymphedema surgery can significantly reduce the swelling in the affected extremities, relieving pain for the patient, decreasing infections, and increasing their mobility. Patients who receive lymphedema surgery are able to achieve a much higher quality of life post-surgery.

Dr. Jay Granzow is one of the world’s leading board-certified plastic surgeons who specializes in lymphedema treatment. Patients come from all over the country and abroad to seek treatment at the Granzow Lymphedema & Lipedema Center