 Lymphedema
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Lymphedema is a swelling that is caused by an abnormal collection of lymph fluid in the affected region of tissue. Lymph fluid is an essential component of the body's lymphoid system, which is key to the body's defense against infection. The swelling caused by lymphedema occurs most often in the extremities (arms and legs) but can occur in other parts of the body. Lymphedema is generally divided into two types:
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- Primary Lymphemdema: This type occurs in children born with decreased numbers of lymphatic vessels (the small vessels that transport lymph fluid) and can affect from as many as one to four limbs and/or other parts of the body.
- Secondary or acquired lymphedema: This type of lymphedema occurs as the result of infection, radiation, or a surgerical procedure.
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In both types of lymphedema, the circulatory network of lymphatic vessels cannot efficiently transport the amount of lymph fluid, which leaves an abnormal amount of protein-rich fluid to collect in the tissues. Left untreated, this dormant, protein-rich fluid not only causes tissue spaces to increase in size and number, but also reduces oxygen availability in the transport sytem. This interferes with wound healing, and provides a culture medium for bacteria that can cause an infection called lymphangitis, i.e., inflammation of the lymphatic vessels.
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Primary lymphedema, occurs in 1 of 10,000 individuals; it can be present at birth (lympadema congenita) develop at the onset of puberty (lympedema praecox) or in adulthood (lymphadema tarda). It is unclear why the swelling sometimes does not occur until adolescence or adulthood. One theory about development of lymphedema during adolescence suggests that the transport of lymph fluid is marginally adequate at birth but decompensates with the increased demands associated with growth at the time of puberty. Primary lympedema is sometimes associated with a combined vascular anomaly such as Klippel Trenaury syndrome and Parkes Weber Syndrome.
Secondary lymphedema develops due to obstruction in of the lymphatic system that is acquired (not present at birth). The causes of acquired lymphatic obstruction include infiltration of regional lymph nodes by a tumor, operations that require removal of lymph nodes, or radiation.
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The symptoms of lymphedema are variable and depend on the severity of the condition. Symptoms in early stages include:
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- Painless swelling of a limb or other body part that is relieved with elevation
- Swelling that is soft and does not "pit" with pressure
- Tightness of the skin
- Decreased flexibility in the hand, wrist or ankle
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Symptoms during later stages can include
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- Swelling that may be painful and is not relieved with elevation
- Swelling that is thickened and doesn't "pit" with pressure
- High fever due to secondary infection
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The symptoms of lymphedema may resemble those of other conditions. Always consult your child's physician for a diagnosis.
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The doctor will want a complete medical history of your child and family's health and will perform a physical examination. In addition, the following diagnostic procedures may be used to evaluate a young child or adolescent:
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- Magnetic Resonance Imaging (MRI) - A diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body. Your child lies on a bed that moves into a cylindrical scanner. The machine takes a series of pictures of the inside of the body using a magnetic field and radiowaves. The computer enhances the pictures produced.
- Computerized Tomography scan of the affected area (Also called a CT or CAT scan) a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices) both horizontally and vertically of the body. A CT scan while your child lies on a bed that moves in a cylindrical scanner. It shows detailed images of any part of the body, including bones, muscles, fat and organs. CT scans are more detailed than general X-ray studies.
- Lymphangiogram(LAG) - an x-ray of the lymphatic channels performed after a contrast medium is injected into the lymphatic system. This test is used to determine whether there is any obstruction in the lymphatic vessels. It is rarely indicated.
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Staging is the process of determining how far the condition has progressed and how severe it is. Determining what stage your child's condition is in helps to dictate the appropriate treatment. Always consult your child's physician for information on staging. The following is a very general discription of the three stages of lymphedema:
Stage 1: During the earliest stage of lymphedema, pitting occurs when the area that is swollen is pressed by fingertips. That means that the affected area indents. Usually, the affected areas are of normal size early in the morning when your child wakes up after a night of keeping the affected areas elevated.
Stage 2: The tissue develops a spongy consistency and does not "pit" when pressure is applied. This is because of the presence of protein rich fluid incites an inflammatory thickening of the skin.
Stage 3: At this stage, the limbs become very large and the swelling is irreversible. The skin becomes more thickened, fibrous and unresponsive to compression. When lymphedema remains untreated, protein rich fluid continues to accumulate, leading to an increase of swelling and a hardening or fibrosis of the tissue. In this state, the swollen limb or limbs becomes an ideal culture medium for bacteria and subsequent recurring infection. Moreover, untreated lymphedema can lead into a decrease or loss of functioning of the limb, skin breakdown, and chronic infections.
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Specific treatment will be determined by your child's physician based on the following:
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- your child's overall health, and medical history
- the extent or stage of the condition
- your child's tolerance for specific medications, procedures, or therapies
- the expectations for the course of the disorder
- parental opinion or preference
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The initial treatment for early stages of lymphedema is aimed at preserving your child's own elastic tissue and the prevention of swelling. Your child's doctor will work with you to develop a program that includes:
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- the use of compression bandaging or compression (elastic support) garments
- elevation of the affected areas
- a massage technique to manually drain the lymph fluid from the affected areas
- special exercises
- vigilant skin hygiene and special safety precautions to prevent infections and any further damage to the lymphatic drainage system.
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If followed properly, the measures above can control lymphedema. If lymphedema is severe and cannot be effectively controlled with non-operative measures, your child's doctor might recommend a surgical procedure called serial debulking. This operation, which is performed in stages, involves excision (removal) of the tissue beneath the skin all the way to the underlying muscle.
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If early stage lymphedema is managed properly with the methods noted above, the condition can be kept under control over the long-term. There have been few reports of long-term follow up on individuals who have had surgical treatment for lymphedema, but individual reports exist of patients maintaining excellent function and contour 15 years after operation.
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