Definition & Overview

Lymphedema is a medical condition characterized by the swelling of one or more extremities due to an impaired flow of the lymph system, thereby causing a buildup of lymph fluid. Lymph fluid contains plasma and white blood cells that are distributed throughout the body through the lymph system. However, when there’s a blockage in the lymph system, flow of the lymph is interrupted, and it builds up in certain areas, like the arms or legs.

There are two forms of lymphedema; primary and secondary. Primary Lymphedema is a rare condition that is inherited and present from birth. The two most common forms of primary lymphedema are Congenital Lymphedema and Lymphedema Praecox. Secondary lymphedema is caused by injury, trauma, infections, radiation therapy, surgery, or removal of the lymph nodes as a part of cancer treatment.

Majority of lymphedema cases in the world were caused by a parasite infection called filariasis, which is transmitted through infected mosquito bites. Worms develop in the lymphatic vessels causing the lymphedema condition.

However, in the United States, radiation treatment for breast cancer, especially when performed after a breast cancer surgery, is one of the major causes of lymphedema. Unfortunately, there is no cure for lymphedema. The treatment options available for the condition are to manage the symptoms and to reduce discomfort.

Cause of Condition

Congenital malformation of the lymphatic system is what causes primary lymphedema. Some of the genes responsible for the development of the lymphatic system have a faulty structure, thus causing difficulties in carrying lymph fluids around the body. The condition is present at birth, but the symptoms may only start developing during puberty and become apparent well into adulthood. When the symptoms of primary lymphedema appear after a person is 35 years old, the condition is referred to as Meige disease or lymphedema tarda.

There are numerous causes of secondary lymphedema including trauma to the lymph nodes or lymph vessels, radiation therapy after cancer surgery, cancer cells that block the lymphatic vessels, an infection of lymph nodes, the presence of parasites in the lymphatic system, and even certain types of medicines can cause the condition.

Lymphedema develops in stages. During the first stage of the condition, swelling is less evident in the arms or legs, but the hand or foot may appear a bit “puffy”. If swelling occurs, it may be intermittent, or only appears during the night but disappears in the day. When pressure is applied to the skin over the swollen area, it will leave a small dent. Stage 1 lymphedema usually does not require any treatment and the condition will resolve on its own.

In Stage 2 lymphedema, the skin around the swollen area feels spongy. Applying pressure to the area will not leave a dent and the limbs feel hard due to tissue fibrosis. Treatment is normally required during this stage.

The most advanced stage, or stage 3 lymphedema is also referred to as lymphostatic elephantiasis. Swelling of the limb is evident as it will normally be very large. The skin is dry and scales may start to form. In some cases, fluids begin to leak or blisters form to contain the fluid. Skin infections are common due to the break in the skin. The treatment for this stage will include a reduction of fluids to reduce swelling, treating tissue fibrosis, treating the infections, and attempting to maintain limb functionality.

Key Symptoms

Swelling is the primary symptom of lymphedema. The intensity of the swelling will be dependent on the stage of the condition. For instance, swelling during stage 1 lymphedema may only be slight and will come and go. In the latter stages, swelling becomes more evident.

Apart from swelling of the limbs, lymphedema can present other symptoms such as:

In primary lymphedema, the condition is already present but the symptoms may only start developing during infancy, early childhood, or early adulthood. In some cases, the symptoms may only appear when the patient has already passed 35 years of age.

In secondary lymphedema, the symptoms can appear anytime. For instance, if the condition is caused by injury or trauma, the symptoms may be evident immediately after. If the cause of lymphedema is cancer treatment, the symptoms may appear after a few months or even years.

Who to See and Types of Treatment Available

The first person to see if you experience any of the above symptoms is your primary care physician. Your doctor will be able to perform tests and provide a complete diagnosis of the condition. If the condition is a result of cancer treatment, your doctor or oncologist will be responsible for providing treatment. Bear in mind that there is no cure for lymphedema and the treatments available will only suppress the symptoms and provide you with a bit more comfort so that you can continue with your daily activities, or at least be able to perform them a little easier.

When diagnosing lymphedema, the doctor will have you undergo several tests. First, the doctor will likely perform limb measurement tests. These will include measuring the size of the limb at different times, calculating the volume of the limb through a water displacement test, or a perometry test to measure the outline and volume of the affected limb. The next test will likely be a bioimpedance test, which involves measuring the flow of electrical currents at different parts of your body. The amount of fluids in the body will affect the flow.

Additionally, the doctor may also request several imaging tests, such as an MRI, ultrasound, CT scan, or a lymphoscintigraph.

Once the condition and stage of lymphedema have been determined, the doctor may proceed with a treatment plan using medications. Medications will include retinoid-like agents, such as acitretin and tazarotene; anthelmintics such as Albenza; topical skin products and antibiotics. Note that the medicines are used to treat the symptoms of lymphedema as there is currently no available cure for the condition.

References:

  • National Cancer Institute. https://www.cancer.gov/
  • Lymphedema – Canadian Cancer Society. https://cancer.ca/
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