Lymphedema - a lymphatic obstruction
Definition
Lymphedema, also known as lymphatic obstruction, is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system, which normally returns interstitial fluid to the thoracic duct and then the bloodstream. The condition can be inherited or can be caused by a birth defect, though it is frequently caused by cancer treatments, and by parasitic infections. Though incurable and progressive, a number of treatments can ameliorate symptoms. Tissues with lymphedema are at high risk of infection.
The Lymph system
The lymph system is a network of lymph vessels, tissues, and organs that carry lymph throughout the body. The parts of the lymph system that play a direct part in lymphedema include the following:
Lymph: A clear fluid that contains lymphocytes (white blood cells) that fight infection and the growth of tumors. Lymph also contains plasma, the watery part of the blood that carries the blood cells.
Lymph vessels: A network of thin tubes that helps lymph flow through the body and returns it to the bloodstream.
Lymph nodes: mall, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the underarm, pelvis, neck, addomen, and groin.
The sleep, thymus, tonsils, and bone marrow are also part of the lymph system but do not play a direct part in lymphedema.
Causes of Lymphedema
Lymphedema can be either primary or secondary. This means it can occur on its own (primary lymphedema) or it can be caused by another disease or condition (secondary lymphedema). Secondary lymphedema is far more common than primary lymphedema.
Surgery: Lymphedema can develop if the lymph nodes and lymph vessels are removed or cut. For instance, surgery for breast cancer may include the removal of one or more lymph nodes in the armpit to look for evidence that cancer has spread. If the remaining lymph nodes and lymph vessels can't compensate for those that have been removed, lymphedema may result in the arm.
Radiation treatment for cancer: Radiation can cause scarring and inflammation of the lymph nodes or lymph vessels, restricting flow of lymph fluid.
Cancer : If cancer cells block lymphatic vessels, lymphedema may result. For instance, a tumor growing near a lymph node or lymph vessel could become large enough to block the flow of the lymph fluid.
Infection: An infection of the lymph nodes can restrict the flow of lymph fluid and cause lymphedema. Parasites also can block lymph vessels. Infection-related lymphedema is most common in tropical and subtropical regions of the globe and is more likely to occur in developing countries.
Primary Lymphedema: Primary lymphedema is a rare, inherited condition caused by problems with the development of lymph vessels in one’s body. Specific causes of primary lymphedema include:
Milroy's disease (congenital lymphedema): This is an inherited disorder that begins in infancy and causes ones lymph nodes to form abnormally, leading to lymphedema.
Meige's disease (lymphedema praecox): This hereditary disorder often causes lymphedema in childhood or around puberty, though it can occur in your 20s or early 30s. It causes the lymph vessels to form without the valves that keep lymph fluid from flowing backward, making it difficult for ones body to properly drain the lymph fluid from your limbs.
Late-onset lymphedema (lymphedema tarda): This occurs rarely and usually begins after age 35.
Lymphedema Test and Diagnosis
To view the lymphatic system, doctor may use the following imaging techniques:
Magnetic resonance imaging (MRI): Using a magnetic field and radio waves, an MRI produces 3-D, high-resolution images. An MRI gives the doctor a better look at the tissues in the arm or leg and can also see the characteristics of lymphedema.
Computerized tomography (CT): A CT scan — also called computerized tomography, or just CT — is an X-ray technique that produces detailed, cross-sectional images of your body's structures. CT scans can reveal areas of the lymphatic system that may be blocked.
Doppler ultrasound :This variation of the conventional ultrasound looks at blood flow and pressure by bouncing high-frequency sound waves (ultrasound) off red blood cells. Ultrasound can be helpful in finding obstructions or blood clot in the veins(deep vein thrombosis).
Radionuclide imaging of your lymphatic system (lymphoscintigraphy): uring this test the patient is injected with a radioactive dye and then scanned by a machine. The resulting images show the dye moving through the lymph vessels, illustrating the blockages in lymph flow.
Lymphedema symptoms
Lymphedema symptoms include:
- Swelling of part of the arm or leg or the entire arm or leg, including the fingers or toes.
- A feeling of heaviness or tightness in the arm or leg
- Restricted range of motion in the arm or leg
- Aching or discomfort in the arm or leg
- Recurring infections in the affected limb
- Hardening and thickening of the skin on the arm or leg
The swelling caused by lymphedema ranges from mild, hardly noticeable changes in the size of the arm or leg to extreme swelling that can make it impossible to use the affected limb. If the lymphedema is caused by cancer treatment, one may not notice any swelling until months or years after treatment.
Treatment of Lymphedema at Super Speciality hospitals in India
Some small studies have looked at whether surgery may be helpful in cases where particularly aggressive or advanced lymphedema doesnot respond to other treatments. It is viewed as an option of last resort for severe lymphedema. Examples of surgery for lymphedema include:/p>
Liposuction :The body has a tendency to deposit fat in areas of the arm that are affected by lymphedema. By using liposuction to remove this tissue, the volume of the arm was reduced significantly. This does not cure the lymphedema, but it can get the arm down to a size the patient would then have to maintain with bandaging at first, followed by wearing a compression sleeve during the day.
Lymph node transplant or transfer surgery:In this approach, the surgeon "harvests" lymph nodes and their attached blood vessels from another area of the body — such as the abdomen or groin — and then connects them to the lymph vessels and blood vessels under the arm.
Lymphovenous anastomoses :This procedure uses microsurgery to build tiny bridges between the lymphatic vessels and the veins, so that the lymph fluid has a new pathway out of the arm. However, it hasn’t been proven effective in the small research studies available.
Non Surgical Treatment of Lymphedema
Compression Garments :Compression garments include long sleeves or stockings made to compress your arm or leg to encourage the flow of the lymph fluid out of your affected limb.
Manual Lymphatic Drainage (MLD) Manual lymphatic drainage (MLD) :sometimes called manual lymphatic therapy, uses light touch to move excess lymph and fluid out of the tissues and back into the lymphatic vessels.
Complete Decongestive Therapy (CDT) Complete decongestive therapy (CDT),also called complex decongestive therapy, is an intensive program that combines many of the different treatment approaches discussed in this section, including bandaging, compression garments, manual lymphatic drainage, exercise, self care and even MLD.
Pneumatic Pumps for LymphedemaA pneumatic pump — also called an intermittent pneumatic compression pump — is a machine that has an inflatable sleeve or vest-like garment attached to it, with multiple chambers (like balloons) that inflate one after the other to stimulate the flow of lymph in the right direction.
Laser Therapy for Lymphedema :Low-level laser therapy is used for the treatment of lymphedema. Some small studies have found that it can help reduce the volume of the arm, break down scar tissue, and increase range of motion while reducing tightness for some women. Kinesio Tape for Lymphedema Kinesio tape is a flexible tape placed on the skin to support and stabilize certain muscles and joints without restricting the body’s range of motion. The theory is that the tape can mimic the effect of manual lymphatic drainage, stimulating the movement of lymph by stretching the skin wherever it is placed.
Lymphedema and Diuretics Diuretics,also known as water pills, work to move water and salt out of the body by increasing the production of urine.
Lymphaticovenular bypass
Supermicrosurgery is defined as the anastomosis of smaller caliber vessels less than 0.8mm in diameter. Lymphaticovenular bypass is a type of lymph venous bypass, in which a super microsurgical technique is used to anastomose subdermal lymphatic vessels and adjacent venules performed through multiple small incisions using a surgical microscope. This results in more permanent improvement of lymphoedema.
Indocyanine green (ICG) fluorescent lymphography
One of challenges of lymphaticovenular bypass is identifying functional lymphatic vessels. Recently, an indocyanine green (ICG) fluorescent lymphography was developed for visualizing the lymphatic vessels. ICG is an approved water-soluble compound, and it has been used for assessing cardiac output, hepatic function, and ophthalmic angiography for decades. When ICG is bound to protein in the tissue, it emits near-infrared ray. When the injected ICG is caught and streamed by the lymphatic vessels, ICG fluorescent lymphography system enables it to detect lymphatic vessels up to 2cm in depth from the skin surface.
ICG fluorescent lymphography can visualize the lymphatic vessels in the subcutaneous tissue not only before, but also during surgery, allowing surgeons to locate a functional lymphatic vessel for the lymphaticovenular bypass prior to making a skin incision. This saves substantial operating time and may contribute to improved outcomes of the operation.
Vascularised lymph node transfer
Recent articles described transplanting composite soft tissue including lymph nodes to the lymphoedematous limb using microvascular technique. Microvascular lymph node transfer is expected to result in new lymphatic vessels sprouting from the transplanted lymph node to drain the region. However, the rationale is theoretical and there have been no definitive data showing that lymphatic vessels actually regenerate from transferred nodes and work as a lymphatic pump.
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