What is lymphedema? Lymphedema is a chronic, manageable, medical condition caused by the abnormal accumulation of protein-rich lymphatic fluid in the interstitium, resulting in persistent and progressive swelling, chronic inflammation, and reactive fibrosis in one or more areas of the body, most often in the limbs. It can also occur in the face, neck, breast, abdomen, and genitals. The lymphatic system is a network of vessels and nodes throughout the body that transports fluid (lymph) from the body’s tissues back to the bloodstream. The functions of the lymphatic system are to maintain the volume and protein concentration of the extracellular fluid in the body and to assist the immune system in destroying pathogens and removing waste products from the tissues.
What causes lymphedema? Lymphedema may arise because the lymphatic vessels or nodes have been damaged or were not formed correctly. There are two types of lymphedema: primary and secondary.
Primary lymphedema is an inherited condition determined by genetic factors at birth. In primary lymphedema, the lymphatic vessels are either underdeveloped or functionally impaired (lymphatic dysplasia). This can affect from one to as many as four limbs and/or other parts of the body, including internal organs. It may be present at birth, but it more often develops later in life without obvious cause. Milroy’s Disease (Nonne-Milroy Syndrome) is more common in males and usually affects a child at birth or presents shortly thereafter. Meige’s Syndrome is more common in females and presents during puberty.
Secondary lymphedema means that the cause of the lymphedema is known. It is a more common problem among adults and children in the United States. It can occur following any trauma, infection or surgery that disrupts the lymphatic channels or results in the loss of lymph nodes. In the United States, lymphedema most often occurs in patients who are treated for cancer, and whose lymph systems have been damaged by surgery or radiation. However, cancer treatment is not an automatic precursor of lymphedema. While only about 20% of cancer survivors will be affected, there is no indication that the type or duration of cancer treatment has any bearing on who will ultimately get this disease. Age, type of chemotherapy, surgery, radiation, menopausal status, and early onset of lymphedema symptoms are not factors in the development of lymphedema.
One thing is clear about lymphedema: Early diagnosis is imperative to managing this condition. The earlier lymphedema is treated, the more that can be done to prevent the progression of the disease. When treated early, lymphedema can be managed successfully, but there is still no cure.
What is the incidence of lymphedema? The National Breast and Ovarian Cancer Centre’s (NBOCC) review of research evidence on Secondary Lymphedema states, “Conservative estimates suggest that 20% of breast, genitourinary, gynecological, or melanoma survivors will experience secondary lymphedema.
More specifically the incidence of secondary lymphedema associated with vulvar cancer is estimated at 36%–47%; breast cancer, 20%; cervical cancer, 24%; and melanoma, 9%–29%.(1)
The incidence of lymphedema following sentinel lymph node biopsy (SLNB) is reported to range from 4% to 8%.(4)
At birth, about one person in every 6,000 will develop Primary Lymphedema.(2)
What are the risk factors for developing lymphedema? Key risk factors for secondary lymphedema include the extent of surgery, lymph node dissection, and radiation treatment. Other factors involve trauma, infection, increased body mass index (BMI), and immobility. Any major damage to the lymphatic system causes a lifelong risk of lymphedema.
What are the early warning signs of lymphedema? Subjectively these may include transient swelling of a limb or other region of the body. Other symptoms may include aching, heaviness, stiffness, limitation of movement, tightness, or temperature changes. Clothing, jewelry, or shoes may feel tighter. Lymphedema is not usually a painful condition, but some people report pain and tension in an affected limb or body part. Clients report that the swelling associated with lymphedema is often aggravated at the end of the day by heat, overuse, sustained positions, and prolonged inactivity. They report that gentle exercise, elevation, massage, and compression can ease their symptoms. Many conditions may cause these symptoms to occur and any of the changes described here will need to be assessed by a doctor in order for an accurate diagnosis.
Characteristics of benign lymphedema are: Lymphedema is rarely painful. The onset is slow. It is pitting in early stages and starts distally. There is swelling of the dorsum of the foot “buffalo hump” and cellulitis is common. Skin changes may include Hyperkeratosis and Papillomas.
How is lymphedema diagnosed? An accurate diagnosis is essential for appropriate therapy. This diagnosis is determined from the clinical history and a physical examination. Co-morbid and confounding conditions of morbid obesity, lipedema, cardiac edema, congestive heart failure, renal disease, metabolic disorders, infection, malignancy, filariasis, complex regional pain syndrome, acute deep venous thrombophlebitis, and chronic venous insufficiency will require a thorough medical evaluation. Lymphedema usually has a gradual onset. However when lymphedema has an acute onset, the appropriate tests to exclude deep venous thrombosis (DVT), recurrence of cancer, and infection may be necessary.
Diagnostic Tests
Lymphography (direct lymphography): This test is radiographic imaging of lymph vessels and lymph nodes by using an intralymphatic injection of an oily contrast agent containing iodine into surgically exposed lymph vessels. This test, to a large extent, has been replaced by modern non-invasive procedures providing cross-sectional images (for example, ultrasound, computed tomography, and magnetic resonance imaging.)
Fluorescence microlymphography: This microscopic examination method is used to assess the morphology and, to certain extent, the functional status (permeability) of the initial lymphatics (cutaneous lymph capillaries). This test is done in only a few facilities because it requires very sophisticated equipment.
Lymphoscintigraphy: A quantitative lymphoscintigraphy is a non-invasive, low-risk, nuclear medical imaging method used to check the lymph system for disease. A radioactive substance that flows through the lymph ducts and can be taken up by lymph nodes is injected into the body. A scanner or probe is used to follow the movement of this substance on a computer screen. A lymphoscintigraphy can be used to find the sentinel lymph node (the first node to receive lymph from a tumor), which may then be removed and checked for tumor cells. A lymphoscintigraphy is also used to diagnose certain diseases or conditions, such as lymphoma or lymphedema.
CT scan: Computed tomography (CT) is a diagnostic procedure that uses special x-ray equipment to obtain a series of detailed cross-sectional pictures of the body. The CT computer displays these pictures as detailed images of organs, bones, and other tissues. This procedure is also called CT scanning, computerized tomography, or computerized axial tomography (CAT).
MRI lymphography: This procedure uses radio waves and a powerful magnet linked to a computer to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissues. An MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-rays. An MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. This procedure is also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.
Differential diagnosis: This diagnosis concerns diseases or conditions that resemble lymphedema.
Lipedema: This mainly affects women, usually as bilateral and symmetrical swelling from the iliac crest to the ankles. The dorsum of the foot is never affected. There is little or no pitting and no cellulitis. MLD is not affective for this condition.
Venous edema: This is characterized by brawny (brown color) and/or hemosiderin staining (rusty red color) caused by lysing (breakdown of cells) that spreads from the mid-calf to the ankle (gaiter distribution). The edema is non-pitting.
Acute deep venous thrombosis: This is characterized by the sudden, usually unilateral, onset of pain and swelling in the calf caused by a blood clot formation in the deep veins of the leg. It is potentially life-threatening because the clot could dislodge and travel to the lungs.
Malignant lymphedema: This is a form of lymphedema caused by mechanically impaired lymph flow in the lymphatics and/or lymph nodes due to malignant tumor growth. It is characterized by rapid development and starts proximally rather than distally. The skin is tight and can break down causing ulcers and non-healing open wounds. It is painful, unlike non-malignant lymphedema and can cause paralysis of the affected extremity.
Pretibial myxedema: This localized myxedema or thyroid dermopathy is an autoimmune manifestation of Graves’ disease. Lesions of thyroid dermopathy are usually asymptomatic and have only cosmetic importance. The skin is dry and orange colored. Advanced forms of dermopathy are associated with elephantiasis or thyroid acropachy. Almost all cases of thyroid dermopathy are associated with relatively severe ophthalmopathy. Localization in the pretibial area relates to mechanical factors and dependent position. When significant edema and elephantiasis are present, local compressive therapy may have added benefits. In mild cases that do not require treatment, 50% of patients achieve complete remission after several years.
Stages of Lymphedema: Whether primary or secondary, lymphedema develops in stages from mild to severe.
Latency Stage 0 (Subclinical Stage of Lymphedema): In this latent or subclinical state, swelling is not evident despite impaired lymph transport, and subjective complaints of limb heaviness are possible.
Stage I (Reversible Lymphedema): This represents the early onset of the condition where there is an accumulation of tissue fluid with higher protein content that subsides with limb elevation. The edema may be pitting at this stage. There is no fibrosis.
Stage II (Spontaneously Irreversible Lymphedema): The accumulation of protein-rich edema fluid does not reduce significantly with elevation. Pitting becomes progressively more difficult. Connective tissue proliferation (fibrosis) occurs.
Stage III (Lymphostatic Elephantiasis): The accumulation of protein-rich edema fluid does not reduce with elevation. The edema may be non-pitting at this stage. Fibrosis and sclerosis of the tissue occur. The skin changes with warty overgrowth development (papillomas and hyperkeratosis).
Are there any complications that can arise with lymphedema? Lymphedema is understood to be a progressive disease and early intervention is recommended to minimize time and age-related changes. The swelling may progress without treatment. The skin is prone to thickening and the development of fibrosis and other secondary changes. When the lymphatic impairment causes the lymph fluid to exceed the lymphatic system’s ability to transport it, an abnormal amount of protein-rich fluid collects in the tissues of the affected area. Left untreated, this stagnant, protein-rich fluid causes tissue channels to increase in size and number, thus reducing the availability of oxygen. This interferes with wound healing and provides a rich culture medium for bacterial growth that can result in infections: cellulitis, lymphangitis, lymphadenitis, (in severe cases sepsis), and skin ulcers. It is vital for lymphedema patients to be aware of the symptoms of infection and to seek treatment at the first signs, since recurrent infections, in addition to their inherent danger, further damage the lymphatic system and set up a vicious cycle. Very rarely, in certain exceptionally severe cases, lymphedema untreated over many years can lead to a form of cancer known as lymphangiosarcoma.
Cellulitis: Cellulitis is a common skin infection caused by bacteria. Staphylococcus and streptococcus bacteria are the most common causes of cellulitis. Normal skin has many types of bacteria living on it. When there is a break in the skin, these bacteria can cause a skin infection. Skin in the infected area will become red, hot, irritated, and painful.
Erysipelas: This is an acute form of cellulitis involving the dermal lymphatics usually caused by group A Streptococcus bacteria. In the past, the face was the most common site of infection. Now it accounts for only about 20% of cases. The legs are affected in up to 80% of cases. Symptoms include blisters, fever, shaking, and chills. Skin under the sore is painful, very red, swollen, and warm and skin lesions have raised border. Antibiotics such as penicillin are used to eliminate the infection. In severe cases, antibiotics may need to be given through an IV (intravenous line).
What can be done to cure or treat lymphedema? Lymphedema cannot be cured, but it can be reduced and managed with appropriate intervention. The stage, location, and severity of the lymphedema, together with the individual circumstances of the patient, will influence the most appropriate intervention. Early intervention is very important.
Treatment for lymphedema: Treatment varies depending on the severity of the edema and the degree of fibrosis of the affected limb. Many methods such as compressive garments, bandaging/wrapping, elevation, surgery, pneumatic compression devices, or complete decongestive therapy (CDT) are utilized.
Complete decongestive therapy: CDT is one of the most common treatments for lymphedema. CDT has been referred to by several terms including complex decongestive physiotherapy, non-invasive complex lymphedematherapy (CLT), early conservative lymphedema management, complicated physiotherapeutics, manual lymphedema treatment (MLT), and multi-modal lymphedema therapy. For purposes of consistency, the term CDT will be used. There are two phases of CDT therapy.
Phase 1 (treatment phase) of CDT consists of manual lymph drainage, compression bandaging, remedial exercise, skin care, and instructions for self care.
Phase 2 (maintenance phase) of CDT consists of compression garments during the day, bandaging at night, skin and nail care, and remedial exercise.
Manual Lymph Drainage (MLD): MLD is a gentle manual treatment that improves the activity of the lymph vascular system. In lymphedema, it re-routs the lymph flow around the blocked areas into more centrally located healthy lymph vessels that drain into the venous system. As the blood passes through the kidneys, the excess fluid is filtered out and eliminated from the body through urination. This technique was pioneered by Emil Vodder in the 1930s for the treatment of chronic sinusitis and other immune disorders. MLD improves lymph production and increases the frequency of the contraction of lymph vessels, thus increasing the volume of fluid transported. MLD is a cornerstone of effective lymphedema treatment; however, in isolation, it does not provide sufficient results.
Multilayer bandaging or wrapping: This first phase of lymphedema treatment must be done by a licensed and trained physical therapist. Compression bandaging, also called wrapping, is the application of several layers of padding and short-stretch bandages to the involved areas. Short-stretch bandages are preferred over long-stretch bandages (such as those normally used to treat sprains), as the long-stretch bandages cannot produce the proper therapeutic tension necessary to safely reduce lymphedema and may, in fact, end up producing a tourniquet effect. During activity, whether exercise or daily activities, the short-stretch bandages enhance the pumping action of the lymph vessels by providing increased resistance for them to push against. This encourages lymphatic flow and helps to soften fluid-swollen areas. Bandaging prevents the re-accumulation of evacuated lymph fluid and during the Phase 1 of treatment stays in place until the next MLD session. It is important to note that compression alone does not stimulate the lymphatic system.
Therapeutic remedial exercises: Exercises are performed with bandages or compression garments in place. Exercises are slow and rhythmic with low exertion and promote an increase in lymph vessel activity and an increase in venous and lymphatic return. Exercises include diaphragmatic breathing that increases the volume of lymph fluid transported by the thoracic duct.
Skin and nail care and infection prevention: It is important to avoid injuries such as nicks and bites and burns. If injured, clean all injuries immediately. Call a physician at first sign of infection. Use a moisturizing lotion such as Eucerine.
Compression garments/elastic garments: Elastic compression garments are generally uncomfortable if worn while the limb is swollen and do nothing to correct the underlying lymphedema. Elastic compression garments are worn by persons with lymphedema on the affected limb to maintain edema reduction following complete decongestive therapy. Depending on the therapist’s discretion, a compression garment may be custom-fit or purchased in over-the-counter, standard sizes. Compression garments are meant to be worn every day to maintain edema reduction and must be replaced on a regular basis. Support garments may be the only garment of choice for patients with scrotal edema.
Sequential gradient pump therapy: Compression pump technology utilizes a multi-chambered pneumatic sleeve with overlapping cells to promote movement of lymph fluid. Pump therapy may be used in addition to other treatments such as compression bandaging and manual lymph drainage. In many cases, pump therapy may help soften fibrotic tissue and therefore potentially enable more efficient lymphatic drainage. Sequential pump therapy may also be used as a home treatment method, usually as part of a regimen involving compression garments or wrapping. In some cases, compression pumps may cause additional swelling in the lower torso and genital region when used on persons with lower extremity lymphedema.
Medications/diuretics: Diuretics usually make the lymphedema worse. Diuretics can draw off the water content of the edema while the protein molecules remain in the tissue space. These proteins continue to draw water to the edematous areas the minute the drugs lose their effectiveness. Diuretics also lead to higher concentration of proteins in the edema fluid and cause the tissue to become even more fibrotic and indurated.
Surgical treatment of lymphedema: Surgery for lymphedema is not curative, but it has been used in specific circumstances for controlling a severe condition. Circumstances where surgery may be considered are reducing the weight of the affected limb, minimizing the frequency of inflammatory attacks, improving cosmetic appearance, or fitting the limb into garments. As with all surgical procedures, the risks and benefits must be weighed against the individual needs of the patient, and the expertise of the surgical team. There are several types of surgical procedures available that have been used for lymphedema: (a) excisional operations, including debulking and liposuction; (b) tissue transfers; and (c) microsurgical lymphatic reconstruction.
Debulking: This surgery removes the hard connective tissue and any large folds of fatty tissue associated with the lymphedema-affected body part. The potential risks of this surgery include prolonged hospitalization, poor wound healing, nerve damage or loss, significant scarring, destruction of the remaining lymphatic vessels in that body part, loss of limb function, return of swelling, poor cosmetic results, and decrease in quality of life. Post-operatively, compression garments are still necessary for the maintenance of the limb and must be worn life-long due to the lymphatic scarring from these surgeries and lymphatic insufficiency.
Lymphatic liposuction: Liposuction involves the circumferential removal of fatty tissue deposits in the body part affected by long-standing lymphedema. It is generally performed under general anesthesia and involves the creation of many small incisions. Tubular suction devices are inserted into the incisions by the surgeon to break up, liquefy, and suction out the fat. Liposuction for lymphedema is similar to cosmetic liposuction. Tight bandaging is necessary to stop the bleeding after liposuction for lymphedema. Life-long compression garments are generally needed to prevent lymphedema from coming back due to the scarring of lymph vessels that can occur from the procedure. The risks of liposuction include bleeding, infection, skin loss, abnormal sensations (such as numbness, tingling, and “pins and needles” feeling), and lymphedema returning.
Tissue transfers: Tissue transfers (grafts) have been attempted to bring lymph vessels into a congested area to remove excess interstitial fluid. There are few studies of the long-term effectiveness of tissue transfers for lymphedema.
Microsurgical lymphatic reconstruction: Microsurgical and supramicrosurgical (much smaller vessels) techniques have been developed to move lymph vessels to congested areas to try to improve lymphatic drainage. Surgeries involve connecting lymph vessels and veins, lymph nodes and veins, or lymph vessels to lymph vessels.
Frequently asked lymphedema questions
Do you treat all body areas? Currently, we treat only the upper extremity lymhedema that occurs primarily as the result of the breast cancer treatment.
How do I make an appointment? Call (305) 981-0609 to schedule an appointment or fill out our online form and we will contact you.
Do I need a referral/prescription for physical therapy from my doctor? Although, in the State of Florida, physical therapists have direct access to patients, most insurance companies, including Medicare, still require a prescription from the doctor. Please contact your insurance company to see if they require you to have a prescription from your doctor.
Is my insurance going to pay for my therapy? It is imperative for you to contact your insurance company to find out how much your out-of-pocket expenses will be and how many visits the company will approve for physical therapy.
How long is the physical therapy treatment? Typical treatment lasts approximately one hour.
What should I wear? For lymphedema treatment, you will most likely have to expose the entire limb that we are treating.
Will I need any supplies and who will pay for them? Yes, you will need supplies for your lymphedema treatment. Many insurance companies do not cover bandages and compression garments that are required for lymphedema treatments. It is your responsibility to contact your insurance company to find out what they will pay for. A list of supplies will be provided for you on your first visit.
What happens on my first visit? Please arrive 10 to 15 minutes prior to your appointment to fill out all the necessary paperwork. Patients who are coming in for lymphedema treatment will be examined and evaluated and provided with educational material and the list of supplies they will need to have in order for the treatment to start. No treatment will be given to you on the first visit. Most supplies required for the lymphedema treatments can be purchased at our facility or on line. We do not accept insurance payments for the lymphedema supplies. Lymphedema treatment cannot be started until the patient has acquired all the necessary supplies.
What happens on my follow-up visits? Please arrive on time. Your treatment is one hour long. If you are late, your treatment will be cut short in order to accommodate the next patient. It is you responsibility to make sure that you have all the necessary supplies. The treatment will consist of manual lymph drainage, application of compression bandaging, and instruction in remedial exercises.
How often and for how long do I need to come in for therapy? Phase 1 (treatment phase) of CDT consists of manual lymph drainage, compression bandaging, remedial exercise, skin care, and instructions in self care. It usually lasts two weeks and requires daily visits.
Who provides the treatment for me? Your initial examination and evaluation are always done by the physical therapist, who then sets up the plan of care for you. The treatment is most often provided by the licensed physical therapist assistant in accordance with the treatment plan provided by the physical therapist.
What kind of results can I expect from physical therapy? Although, results vary greatly, you can expect a significant reduction in lymphedema.
How do I know when I’m done with the physical therapy treatment? Usually by the end of two weeks once a significant reduction in lymph volume is achieved, you will be measured for a compression garment. After you are fitted with the appropriate compression garment, Phase 2 (maintenance phase) of CDT begins. This phase consists of using compression garments during the day, bandaging at night, skin and nail care, and remedial exercise.
References:
1. National Breast and Ovarian Cancer Centre. Review of research evidence on secondary lymphedema: Incidence, prevention, risk factors and treatment, NBOCC, Surry Hills, NSW, 2008.
2. Lymphedema Framework: Best Practice for the Management of Lymphedema. International Consensus. London: MEP Ltd, 2006.
Other Resources: Many resources and references were used for the above-mentioned information including:
• American Cancer Society, ACS Complete Guide to Lymphedema: Understanding & Managing Lymphedema after Cancer Treatment, ACS 2006.
• Foldi, M., Foldi, E., Kubik, S. Textbook of Lymphology, 6th Edition, Elsevier 2005.
• Zuther J. E. Lymphedema Management: The Comprehensive Guide for Practitioners. 2nd ed. New York, NY: Thieme; 2009.
• Course Manual for Manual Lymph Drainage/Complete Decongestive Therapy, Certification Training, from Norton School of Lymphatic Therapy.
• www.lymphnotes.com and www.lymphedemapeople.com have a wealth of information on lymphedema.
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