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An Overview of Pediatric and Primary Lymphedema

October 20, 2019

What is the lymphatic system? The lymphatic system is transporting system, with a fluid called lymph that moves through the whole body via small specialized vessels, the lymphatic vessels. It has an important function in protecting us from viruses, bacteria, fungi and all sorts of toxic molecules that could make us sick. The lymphatic system is abundant everywhere in our body where we are in contact with the environment (skin, gut with food, chest with air, genitals and urinary tract). Eventually, after having taken up organisms and substances, the lymphatic fluid will be transported through the lymph vessels to lymph nodes that destroy viruses and bacteria or toxic molecules and then produce antibodies that help the body to protect itself (immune system). What does the lymphatic system look like? The lymph nodes are present at many places but are also grouped at particular parts of our body: in the neck, next to the trachea, in the armpits, near the lungs, in the abdominal area, in the pelvic area, and in the groin. The lymph is finally transported into the veins mostly at the left super clavicular region. What is lymphedema? Lymphedema is a chronic swelling of one or more parts of the body because the lymphatic drainage system is not able to work properly. There are many reasons for this, of which important ones are that the lymphatic system has been damaged, is overloaded, or has not developed properly. The last one is the case in the so-called primary lymphedema. Lymphatic fluid builds up and this will cause a chronic swelling. Sometimes there is a genetic link, especially in primary lymphedema, and the lymphedema may run in families. Lymphedema in children (pediatric lymphedema) can be primary lymphedema, but it can also occur in children when lymphatics have been damaged after treatment of cancer or after filariasis, which is very common in some areas of the world. Therefore, lymphedema should best be seen as a symptom that has many causes. How can lymphedema be diagnosed? Lymphedema has specific features such as pitting of the skin when pressed firmly. There can be a specific sign, that it is not possible to lift the skin of the second toe (called positive Stemmer sign). The function of the lymphatics can be visualized by lymphoscintigraphy. Sometimes this can help to find the cause of the lymphedema. In lymphoscintography a small amount of liquid is injected between the toes (when the swelling occurs in the legs) or between the fingers (when the swelling occurs in the arms). One follows the movements of the liquid, including the uptake in the lymph vessels, by making several pictures in the course of time, and this makes the lymphatic system visible. Furthermore, the uptake in the lymph nodes can be measured. The outcome of the test can vary considerably depending on the physical activities of a patient. A good standardized protocol is mandatory. The lymphatic system can also be made visible using Magnetic Resonance Imaging – MRI, which even allows visualization of the lymphatic vessels themselves. It also relies on the imaging of lymph accumulation in the tissues (with MRI). It is nowadays also possible with MRI to see the lymphatic vessels themselves. How can lymphedema be treated? Lymphedema cannot be cured completely; medications usually do not help to reduce the swelling. But often the lymphedema can be reduced by other treatments. Treatment is divided in two stages: the initial treatment phase (often 8-12 weeks) followed by a maintenance phase. Both phases have their specific therapeutic modalities. Treatment is based on a dedicated individual program, taking someone’s limitations and possibilities into account. Factors known to worsen the lymphedema, such as lack of physical activity and being overweight, should be addressed as well. Patients with lymphedema should wear compression: they can wear sleeves, stockings, socks, or bandages on their affected limb in order to reduce the swelling or to prevent the swelling from increasing. These garments need to be worn all the time, especially during exercise. Patients often need to learn how to adapt their compression to their needs. Exercise is important for lymphedema patients since the muscles act as pumps themselves and stimulate the lymphatics to function. This works particularly well when wearing compression garments when exercising. Typically, a program is set up to increase gradually the intensity of the exercise (the graded activity method). Another helpful therapy is manual lymphatic drainage (MLD). Usually it is applied in combination with compression. MLD is a very mild massage of the affected limb, which stimulates lymph drainage. Especially in the initial phase of treatment it can be helpful. MLD is performed by specialized physical therapists, but one can often learn oneself how to perform MLD and then treat oneself. In a limited number of patients, surgery can be an option. This is only performed in a small number of highly specialized clinics, and positive results can often not (yet) be guaranteed. Even after surgery compression treatments remain almost invariably needed. The right choice for therapy asks for an overview of all possibilities and insight into the characteristics and situation of a patient, and it is useful to visit a lymphedema center to discuss the options. What are the complications of lymphedema? Cellulitis is an infection in the swollen area. It can make a patient feel very unwell, with fever, vomiting and pain in the affected limb. The affected area can become warm and red. Rapid medical consultation is important. In case of cellulitis, antibiotics will be prescribed. If lymphedema of the extremities is involved in further defects of the lymphatic system, ascitis or pleural effusion can be present. Here, the lymph can be found in the abdominal or pleural cavity. Respiratory distress and abdominal pain can result. Drainages can be necessary and a diet with MCT fat is prescribed. For more information, look at our website:

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