Lymphedema - types, causes, symptoms, diagnosis, treatment

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Lymphedema - characteristics and types

Lymphedema is the swelling of one or more limbs that results from impaired flow of the lymphatic system.

The lymphatic system is a network of specialized vessels (lymph vessels) throughout the body to collect excess lymph fluid along with proteins, lipids, and waste products from the tissues. This fluid is then transferred to the lymph nodes, which filter waste products and contain infection-fighting cells called lymphocytes.

The excess fluid in the lymph vessels eventually returns to the bloodstream. When the lymphatic vessels are blocked or unable to drain the lymph fluid from the tissues, a local swelling (lymphedema) occurs.

Lymphedema most commonly affects one arm or leg, but may rarely affect both extremities.

Primary lymphedema is the result of anatomical abnormalities in the lymphatic vessels and is a rare hereditary disease.

Secondary lymphedema results from recognizable damage or obstruction of normally functioning vessels and lymph nodes. The most common causes of secondary lymphoedema include mechanical injuries, surgical procedures, bacterial and fungal infections, neoplastic tumors oppressing the lymphatic vessels, inflammation of the skin or lymph nodes, obesity, filariosis, chronic venous insufficiency, radiotherapy or even long-term lack of exercise, caused by e.g. . injury.

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Lymphedema - causes

There are many causes of lymphedema. It may result from congenital or acquired defects. Therefore, lymphoedema is divided into primary and secondary.

The causes of primary lymphedema

Primary lymphedema is an abnormality of the lymphatic system and usually occurs at birth, although symptoms may not appear until later in life. Depending on the age at which symptoms appear, three forms of primary lymphedema are described. Most primary lymphedema occurs with no known family history of the disease.

  1. Congenital edema (lymphoedema congenita) - appears after birth, is more common in women and accounts for approximately 10-25% of all primary lymphedema cases. A subset of people with congenital lymphedema inherit a condition known as Milroy's disease.
  2. Early lymphoedema (lymphoedema praecox) - is the most common form of primary lymphedema. It is defined as lymphedema that appears after birth and before the age of 35, with symptoms most common in adolescence. Lymphedema praecox is four times more common in women than in men.
  3. Lymphoedema tarda - Lymphedema that becomes apparent after the age of 35, is also known as Meige's disease. It is less common than hereditary lymphedema and early lymphedema.

The causes of secondary lymphedema

Secondary lymphoedema develops when a normally functioning lymphatic system is blocked or damaged. A relatively common cause is breast cancer surgery, especially when combined with radiation therapy. This causes one-sided lymphedema in the arm.

Any type of surgery that requires the removal of regional lymph nodes or lymph vessels has the potential to cause lymphedema. Surgical procedures associated with lymphedema include vein stripping, lipectomy, burn scar excision, and peripheral vascular surgery.

Injury to lymph nodes and lymph vessels leading to lymphoedema can also occur from trauma, burns, radiation, infection, pressure, inflammation (e.g. rheumatoid arthritis and eczema), or tumor invasion of the lymph nodes.

However, worldwide, filariasis is the most common cause of lymphedema. Filariasis is the direct infection of the lymph nodes by the parasite Wuchereria bancrofti. The disease is spread among humans by mosquitoes and affects millions of people in the tropics and subtropics of Asia, Africa, the Western Pacific, and parts of Central and South America.

Parasite invasion damages the lymphatic system, leading to swelling of the arms, breasts, legs and, in men, the genital area. The entire leg, arm or genital area may swell several times its normal size.

In addition, swelling and a weakened lymphatic system make it difficult for the body to fight infection. Lymphatic filamentous disease is the leading cause of permanent disability in the world.

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Lymphedema - symptoms

Lymphedema usually occurs in one or both arms or legs, depending on the extent and location of the damage. Primary lymphedema can also occur on one or both sides of the body.

Lymphedema can be mildly visible or debilitating and severe, as in filariosis, in which a limb may swell several times its normal size. For the first time, it may be noticed by the affected person as asymmetry between the arms or legs, or difficulty in fitting the body to clothing or jewelry. If the swelling becomes pronounced, fatigue from being overweight, as well as embarrassment and limited mobility, can occur.

Prolonged accumulation of fluid and protein in tissues leads to inflammation and eventually scarring of the tissues, leading to severe, tight swelling that does not form pitting edema. The skin in the affected area thickens and may take on a lumpy appearance, described as a 'peau d'orange' effect. The skin covering it may also become scaly and chapped, and secondary bacterial or fungal skin infections may develop. The affected areas may be tender and sore, and there may be a loss of mobility or flexibility.

Other symptoms that may accompany lymphedema include:

  1. redness or itching;
  2. tingling or burning pain;
  3. fever and chills;
  4. reduced joint flexibility;
  5. dull pain and a feeling of fullness in the area involved;
  6. skin rash.

The function of the immune system is also impaired in scarred and swollen areas affected by lymphedema, leading to frequent infections and even a malignant tumor of the lymphatic vessels known as malignant lymphangiosarcoma (Latin lymphangiosarcoma).

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Lymphoedema - diagnosis

A careful history and physical examination are performed to rule out other causes of limb swelling, such as edema from congestive heart failure, kidney failure, blood clots, or other conditions. Often, a medical history of surgery or other conditions involving the lymph nodes will indicate the cause and allow a diagnosis of lymphedema to be made.

If the cause of the swelling is not clear, other tests may be performed to determine the cause of the swelling in the limb in question.

  1. Lymphoscintigraphy, i.e. examination for abnormalities of the lymphatic system in the flow or structure of the lymph. The patient is injected with a very small amount of a radioactive substance called a radiotracer, which travels through the area being examined. A special camera and computer above the patient create images inside his body that show any problems in the lymphatic system. The treatment is painless (although you may feel a prickle when injected) and the side effects are minimal. Radioactive material will be excreted from your body through urine.
  2. A Doppler ultrasound analyzes blood flow and pressure by reflecting high-frequency sound waves (ultrasound) off red blood cells. It can help you find obstacles and rule out other possible causes of swelling, such as blood clots.
  3. MRI (Magnetic Resonance Imaging) uses a magnet, radio waves, and a computer to take a series of detailed three-dimensional images inside the body. It can display an accurate picture of the extra fluid in the tissues.
  4. CT (Computed Tomography) uses X-rays to show detailed, cross-sectional images of our body's structures. CT also shows blockages in the lymphatic systems. Both CT and MRI show the size and number of lymph nodes, which helps determine the type of primary lymphoedema.
  5. Spectroscopic bioimpedance is a non-invasive test that measures the total amount of water in the patient's body, both extracellularly and intracellularly. Many clinics use this test in patients at risk of lymphedema by performing regular, routine assessments to check for any changes in volume. It has been shown to be able to detect lymphedema before any visible signs of swelling appear.
  6. Dye lymphography - a test performed with the use of indocyanine green (ICG - indocyanine green). The dye is injected under the skin and absorbed by the lymphatic system. A special infrared camera maps the lymphatic function. Lymphography is an imaging technique that allows you to visualize superficial lymph flow.
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Lymphedema - treatment

There is no cure for lymphedema. Treatments are aimed at reducing swelling and controlling discomfort and other symptoms.

Compression treatments can help reduce swelling and prevent scarring and other complications. Examples of compression treatments are:

  1. elastic sleeves or stockings: they must fit properly and provide gradual pressure from the end of the limb towards the torso.
  2. bandages: bandages tighter around the end of the limb and loosely wrapped towards the torso to encourage lymph drainage from the limb towards the center of the body.
  3. pneumatic compression devices: these are sleeves or stockings connected to a pump that provides sequential compression from the end of the limb towards the body. They can be used in the clinic or at home and are useful in preventing long-term scarring, but they cannot be used in everyone, such as those with congestive heart failure, deep vein thrombosis, or certain infections.
  4. Massage: Massage techniques, known as manual lymphatic drainage, may be useful for some people with lymphedema.
  5. Exercise: Your doctor or physical therapist may prescribe exercises that lightly tighten and stimulate the muscles in your arms or legs to stimulate lymph flow.

Lymph, a mixture of herbs available on Medonet Market, will help to relieve the symptoms of lymphedema.

Surgery is considered if other non-surgical treatments are not working. Not everyone is a candidate for surgery, but some symptoms can be relieved with surgery.

  1. Lymphatic bypass procedure: Lymphatic vessels and veins are connected and redirected around obstructions, allowing the lymphatic fluid to drain directly into the body's venous system. Advances in surgical techniques are now reducing risks and creating less invasive procedures.
  2. Lymph node transfer: Lymph nodes from other parts of the body are placed in areas where the lymphatic system has been damaged. This procedure helps restore a healthy lymphatic system in this region.
  3. Liposuction: Liposuction is a procedure in which fat and other tissue is removed through a small incision in the body.
  4. Debulking: This involves removing all skin, fat, and tissue from the affected area, and then placing a skin graft over that area. Only used in very advanced, severe cases.

Skin and tissue infections associated with lymphedema must be treated promptly and effectively with appropriate antibiotics to avoid spreading into the bloodstream (sepsis). Patients affected by lymphedema must constantly monitor the infection of the affected area. In the affected regions of the world, diethylcarbamazine is used to treat filariasis.

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Lymphedema - complications

Common complications of lymphoedema are inflammation of the skin and connective tissues (cellulitis) and lymphangitis (Latin lymphangitis). Deep vein thrombosis (the formation of blood clots in deeper veins) is also a known complication of lymphedema. Further complications of lymphedema include functional impairment in the affected area and cosmetic issues.

People with chronic, long-term lymphoedema for more than 10 years have a 10% chance of developing cancer of the lymphatic vessels known as lymphangiosarcoma. The cancer begins as a reddish or purplish lump visible on the skin and spreads rapidly. It is an aggressive tumor that is treated by amputation of a diseased limb. Even with treatment, the prognosis is poor - less than 10% of patients survive after 5 years.

It's worth noting that lymphedema can affect appearance, and this in turn can have a psychological effect, especially in people living with cancer. Lymphedema increases the risk of developing depression.

See also: Acute lymphangitis

Lymphedema - exercise

People with lymphedema are encouraged to lead a healthy lifestyle, including regular exercise and exercise. However, in some cases, you may need specialized help to help you exercise safely and effectively.

Studies have shown that women at risk of lymphedema after breast cancer surgery will not be at greater risk of lymphedema in their arm if they perform gentle lifting exercises. Exercise like this, researchers say, can reduce the risk of lymphedema.

The types of exercise that may be beneficial include those that:

  1. increase flexibility;
  2. they practice stretching;
  3. they build strength.

Aerobic exercise that focuses on the upper body, helps with weight loss, and encourages deep breathing is also recommended.

If there is any heaviness or change in shape, texture, or other alteration of the limb, it should be observed. This may mean that your current level of exercise is too high.

Experts believe that muscles act as a pump during exercise, pumping lymph to the areas where it's needed.

However, there is not enough evidence yet to support any particular type of exercise for lymphedema. Women who have had breast cancer surgery are advised to seek help from a specialist physical therapist or other health care professional to help them gradually increase their physical activity.

Lymphedema - proper diet

Lymphedema is better controlled with good eating habits. Our lymphatic system works more efficiently thanks to better nutrients from minimally processed, natural foods (fruits, vegetables, whole grains). Eating healthy brings us closer to our ideal weight, which is an important factor in reducing the symptoms of the disease. It makes us feel better overall.

Healthy eating for lymphedema includes the following rules.

  1. Reduce the consumption of foods high in salt and fat.
  2. Include at least 2 to 4 servings of fruit and 3 to 5 servings of vegetables in your daily meal plan.
  3. Eating a variety of foods to get all the nutrients you need.
  4. Use the information on the packaging labels to make the best choices for a healthy lifestyle.
  5. Eating high-fiber foods such as whole wheat bread, cereals, pasta, rice, fresh fruit and vegetables.
  6. Drinking plenty of water - Eight glasses of 240 ml of water per day are recommended.
  7. Maintaining an ideal body weight. A registered dietitian or doctor can help you calculate your ideal body weight for this situation and we can measure your BMI.
  8. Avoiding alcoholic beverages.

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Lymphedema - prevention

Primary lymphedema cannot be prevented, but measures can be taken to reduce the risk of developing lymphedema if there is a risk of secondary lymphedema, for example after cancer surgery or radiation therapy.

The following steps can help reduce the risk of developing lymphedema in people at risk of secondary lymphedema.

  1. Protect your arm or leg. Avoid injuring the affected limb. Wounds, scrapes, and burns can trigger infection. Protect yourself from sharp objects. For example, shave with an electric razor, wear gloves when you work in the garden or cook, and use a thimble when sewing. If possible, avoid medical procedures such as blood sampling and vaccinations for the affected limb.
  2. Let your limbs rest. After cancer treatment, exercise and stretching are encouraged. However, avoid strenuous activity until you recover from surgery or radiation.
  3. Avoid warm sensations on the arm or leg. Do not apply ice or warm compresses to the affected limb. Also protect the diseased limb from extreme cold.
  4. Raise your arm or leg. If possible, raise the affected limb above the level of the heart.
  5. Sit down correctly. To keep your legs flowing, try to practice good posture by keeping your feet flat on the floor and avoid crossing your legs. Try not to sit for more than 30 minutes.
  6. Avoid tight clothes. Avoid anything that might press on your arm or leg, such as tight-fitting clothing and, in the case of the upper arm, blood pressure cuffs. Ask to measure your blood pressure on the other arm.
  7. Keep your arm or leg clean. Make skin and nail care a priority. Inspect the skin of your arm or leg daily for changes or cracks in the skin that could lead to infection. Don't go barefoot. Always apply sunscreen (SPF 30 or higher) when going outside. Keep your skin scrupulously clean. Thoroughly dry the skin (including between the fingers and toes). Apply the lotion to the surrounding skin, but not between your toes. Wash your hands frequently with soap and warm water, especially before preparing meals, and after using the bathroom or touching soiled bed linen or clothes.

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