Chronic Swelling: Lymphedema Defined

The lymphatic system is an intricate system of vessels in the body that drains fluid – lymph – from the body back into the blood system. Lymphedema is an abnormal condition of the lymphatic system that affects both adults and children and is not always well understood in the medical community. Lymphedema is defined as a swelling of a body part that most frequently occurs in the arms or legs but may also occur in the face, neck, abdomen or elsewhere in the body.

It is important to know that lymphedema is an abnormal accumulation of protein-rich fluid in an area of the body that does not go away on its own and should not be confused with normal swelling that occurs with injury or surgery.

Lymphedema has two classifications: Primary and Secondary. Primary lymphedema occurs when the lymphatic system in body is not formed properly. This may happen at birth or later in life. The cause of primary lymphedema is unknown. Secondary lymphedema is more common and is caused by significant trauma or surgery that affects the lymphatic system.

There are several types of Primary Lymphedema that affect children including:

Milory’s Disease

A primary lymphedema that affects children at birth or shortly after and usually involves swelling in one leg but may include other areas.

Meige’s Syndrome

Another primary lymphedema that affects both legs and rarely other areas of the body. Children with Meige’s may also have fusion of their toes, two rows of eyelashes, nearsightedness, yellow colored nails, differences in their spines, hearing loss, cleft palate and other symptoms.

Distichiasis Syndrome

A rarer syndrome that typically also involves the legs and children may have two rows of eyelashes but the vessels are larger and there are more vessels in the lymphatic system of children with this diagnosis.

In addition to congenital causes of lymphedema, there are also several secondary sources of lymphedema including radiation, crush injuries, skin infections, scarring, surgical procedures and other trauma.

Treatments for lymphedema include surgical and non-surgical treatments. Non-surgical treatments include a type of massage known as manual lymph drainage (MLD), compression bandaging, exercise, and pressure garments to assist with moving the fluid and keeping the fluid from collecting in the affected area. It is important to consult with a medical professional prior to initiating these treatments as there are precautions and contraindications in some children.