Diagnosing Lymphedema by Physical Examination
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How to Diagnose Lymphedema
------------------------------------------When lymphedema takes hold
KEY WORDS: lymphedema, lymphangitis, deep vein thrombosis, filariasis, lymphoscintigraphy, Kaposi-Stemmer's sign, decongestive lymphedema therapy, manual lymph drainage
This disfiguring and sometimes painful condition affects many cancer patients, and may appear months after surgery or other damage to the lymph system. Prevention and patient education are especially important for patients at risk.
Lymphedema is a condition that reflects a disheartening reality for many cancer patients—and other patients, as well. Symptoms include swelling of limbs up to twice the normal size, skin that indents and stays that way when pressed (pitting), and thickening, hardening and darkening of the skin (brawniness).1
The high protein content of the lymph that causes the swelling is an ideal environment for dermatitis. The swelling and infection can become disabling and disfiguring, causing significant physical and psychosocial impairment.
It's hard to pinpoint the precise incidence of lymphedema because of variations in its definition and measurement.1 One recent review reported that lymphedema of the arm occurs in 26% of patients undergoing breast cancer therapy.2 This is not surprising, given that the lymphatic system is the primary route for metastasis.
Lymphedema, though, is not limited to breast cancer. Individuals treated for melanomas or cancer of the ovaries, testicles, vulva, or bladder can all develop lymphedema.3 Lymphedema can also occur because of venous disease, immobility, infections, and other trauma, including an increasing number of joint replacement and venous harvesting procedures.3 As a nurse, your skill in managing lymphedema can do much to preserve the quality of life for these patients.
Normally, the lymphatic system collects excess fluid and protein from body tissues and moves them back into the bloodstream at the rate of about three liters per day.4 Lymph is propelled—moving against gravity—through the lymphatic vessels by breathing movements and skeletal muscle contractions that create a pressure gradient. A series of valves keep lymph flowing in one direction.
When the lymphatic system suffers some type of trauma, this ability to remove excess fluid is compromised; lymph accumulates in interstitial spaces, most commonly in subcutaneous fatty tissue, and thus causes swelling in the arms and legs, and, less frequently, in the trunk or genitals.5 Stagnant, protein-rich lymph causes tissue channels to increase in size and number, which reduces oxygen availability, interferes with wound healing, and creates an optimum environment for bacterial infection (lymphangitis).
Lymphedema may be either primary or secondary. Primary (congenital) lymphedema is a rare, inherited condition in which the lymph nodes are either absent or abnormal.
Secondary lymphedema results from damage to the lymphatic system—typically from surgical removal of lymph nodes (which is often necessary in cancer patients) or blockages in the lymphatic system caused by infection, cancer that has traveled to the lymph nodes, or scar tissue (e.g., from radiation therapy, an injury, or surgery to remove a tumor).1 Lymphedema may also be a side effect of the breast cancer drug tamoxifen citrate (Nolvadex), because it can cause blood clots and subsequent deep vein thrombosis (DVT) that advances to lymphedema.5
In some third world countries, the most common cause of lymphedema is filariasis, a disease caused by the parasitic worms Wucheria bancrofti, Brugia malayi, and Brugia timori, which is transmitted by mosquitoes.6,7
Secondary lymphedema may be transient or chronic.1 Transient lymphedema lasts less than six months. The skin indents when pressed and stays indented without brawniness.8 Factors that predispose patients to transient lymphedema include surgical drains that leak protein into the surgical site; inflammation following injury, radiation, or infection that leads to increased capillary permeability; limb immobility that results in decreased compression of the lymphatic system by the musculature; temporary loss of lymph vessel function due to surgery; and blockage in a vein from a clot or inflammation.1
Chronic lymphedema is difficult to reverse. It occurs as the result of tumor recurrence or migration into the lymph nodes, infection or injury to lymph vessels, long periods of immobility, or radiation therapy or surgery that damages the lymph system. Radiation therapy can damage otherwise healthy lymph nodes and vessels, which causes scar tissue to form, blocking the normal flow of lymph throughout the lymphatic system. Chronic lymphedema may also occur after previously unsuccessful management of early lymphedema or when a vein is blocked by a blood clot.
Because specific criteria for diagnosing lymphedema do not yet exist, a thorough medical history and physical exam are essential for a differential diagnosis.1
Age of onset will differentiate primary from secondary lymphedema because early onset is more likely to be due to an alteration in the gene for vascular endothelial growth factor.3,7 For a definitive diagnosis of primary lymphedema, the causes of secondary lymphedema must first be ruled out.
Since secondary lymphedema tends to result from a causative event such as surgery or radiation therapy, you'll need to ask detailed questions about any past surgeries and postoperative complications, especially whether the surgery was for cancer and whether any lymph nodes were removed. Rapid onset of lymphedema may indicate tumor infiltration or a blockage of a major lymphatic pathway. The history should also document any restrictions on mobility, such as wheelchair use; trauma (especially to the legs, abdomen, or genital region); and residence in or visits to third world countries (due to risk of filariasis).
Ask patients whether they have chronic venous insufficiency, thyroid dysfunction, arthritis, or hypertension, because each of these conditions can cause venous edema. It's important to distinguish lymphedema from edema associated with venous insufficiency.5
Lymphoscintigraphy is a test that can help with that distinction. In this procedure, a small amount of radioactive protein dye is injected into the web space between the first and second digits of the affected limb; the limb is then photographed with a gamma camera to observe the dye as it moves through the lymphatic system.9 Images showing dye outside the lymphatic structures suggest edema of lymphatic origin.
Computed tomography (CT) and magnetic resonance imaging (MRI) can also be used to diagnose lymphedema.9 However, these forms of imaging, as well as lymphoscintigraphy, can be costly.
In your physical assessment, examine the skin of the affected limb(s). Dryness, increased thickness, hyperkeratosis (scaling), lymphangiomas (blisters containing lymph fluid), abnormally warm or hot temperature, unusually dark skin, and the presence of any nodules are all indicators of lymphedema.3
Papillomatosis—a cobblestone appearance of the skin—may also be present; it results from dilated and distended lymph vessels that are enveloped in fibrotic tissue.9 Look, too, for Kaposi-Stemmer's sign—the inability to pinch a fold of skin at the base of the second toe because the skin is so thickened—which points to a diagnosis of lymphedema.9
Make sure to note any wounds on the skin. Patients with lymphedema may have lymphorrhea, the weeping of lymph fluid, which may be copious enough to actually run or trickle down the leg. This symptom predisposes patients to cellulitis.
Lymphedema can cause sensations of limb heaviness and skin tightness, heat or burning, or "pins and needles." Some patients experience moderate to severe pain due to nerve compression from surrounding edematous tissue.10 Nerve compression may also decrease response to stimulation and predispose patients to injury and infections.
Your physical assessment needs to include details on the degree of swelling, and the shape and size of the limb(s) affected; the patient's height and weight (and body mass index, if available); the functionality of the limb; and arterial competency.
If the lymphedema is unilateral, quantifying the extent of swelling by comparing the size of an affected limb to a healthy one can be useful for qualifying the severity of the disease. Be aware, though, that normal anatomic variations can complicate things, and that comparison won't be possible if both limbs are affected.
If the affected limb is <20% larger than the unaffected limb, the disease is considered to be mild to moderate. Moderate to severe lymphedema is defined as an affected limb that's >20% larger than the unaffected one.11
For patients with moderate to severe lymphedema, decongestive lymphedema therapy (DLT)11 is the standard treatment. Its purpose is to help mobilize lymph and dissipate fibrosclerotic tissue. In the initial treatment phase, DLT consists of manual lymph drainage (MLD), compression, scrupulous skin hygiene to prevent infection, and exercise.12-14 This is usually followed by a maintenance program consisting of compression, skin care, and exercise. If lymphedema is mild to moderate, DLT may be bypassed in favor of maintenance therapy.
Note that although elevation is a core treatment for edema of venous origin, it doesn't appear to be as effective for treating lymphedema.15 However, elevation may be useful as an adjunct to the components of DLT, described below. Drug therapy and surgery are also discussed, but have limited use.16
• Manual lymph drainage is a light-pressure massage therapy designed to stimulate lymph flow by encouraging the opening and closing of the valves in lymph capillaries and vessels. The direction of massage is against gravity, from the distal to the proximal part of the limb. During the massage—a session generally lasts 30 – 60 minutes—the patient usually wears a light compression garment or stocking to maintain pressure on tissue and prevent the flow of lymph back into interstitial spaces.16
Ideally, this therapy should be administered by a certified lymphedema therapist. MLD should be avoided in patients with certain conditions, such as patients who have experienced cardiovascular failure or in whom DVT is suspected, because it will force more fluid toward the heart and may dislodge blood clots.16 MLD, however, doesn't pose a risk in patients with arterial compromise (e.g., no pulse in legs, poor circulation, diabetes, etc.).
• Compression serves the dual purpose of optimizing the action of the muscles as lymph pumps and preventing further swelling. Regardless of whether patients are receiving initial treatment phase DLT or maintenance therapy, arterial competence needs to be assessed with a Doppler ultrasound prior to using compression.
Before deciding to compress the affected limb, several other factors need to be considered, as well. Compression can exacerbate poor skin condition, DVT, heart conditions, inflammation, and cellulitis; it may also increase the risk of infection.16 At the same time, the lymphedema itself creates a breeding ground for infection—which is a compelling reason to use compression.
Studies have demonstrated that multilayer systems reduce lymphedema more effectively than single-layer compression garments when used for the initial phase of treatment.17 Multilayered compression systems consist of three or four layers of bandaging and are used in the first two to three weeks of the initial phase of treatment.3 Multilayer bandage systems can be left on for seven days at a time before changing or are changed two to three times a week if wound care is required.18
Pneumatic compression pumps consist of a simple, multichamber sleeve that fits over the affected limb. The chambers alternately inflate and deflate according to a timed cycle, causing a peristaltic motion on the swollen tissue. Their use to control lymphedema, however, is controversial.15
Compression garments—such as stockings, sleeves, and gloves—should only be used in maintenance therapy.3 They are available off the shelf in small, medium, and large sizes, or they can be custom-made for the patient. Garments are rated to deliver 30 – 60 mm Hg of pressure.19 Patients wear the garments for most of the day and remove them at night to inspect, clean, and moisturize the skin. Check to see if your patient has an allergy to garment components, such as latex.
• Fastidious skin care is a critical component of lymphedema treatment. The high protein content of lymph fluid predisposes patients to infections in the lymphedematous limb.20 A daily routine should be established in which the affected limb is inspected for wounds, cleaned, and moisturized. The routine may be altered to every two or three days, or weekly in patients who could benefit from continuous multilayer compression and whose skin is in relatively good condition. Careful inspection is especially important if there is a sensory neuropathy present.
After cleansing the skin with a mild hypoallergenic soap and water, the patient should apply an emollient, preferably in the evening, for rehydration overnight. Lotions containing lanolin or fragrances should be avoided because they can cause sensitization with a resulting dermatitis. In addition, avoid petrolatum-based lotions, since they may damage compression garments. The toes and feet should also be inspected regularly for fungal infection, which is very common in patients who have lymphedema.
• Exercise can help promote lymph flow and increase protein reabsorption as a result of muscle contraction.9,21 The exercise, however, should be gentle and suited to the individual's abilities and fitness level. Vigorous sports are not recommended due to the possibility of trauma.17
• Drug therapy—other than antibiotics to treat infections caused by lymphedema—has not been very useful in treating lymphedema directly. Diuretics help to purge vascular fluid, but they're ineffective in clearing out excess protein and could cause connective tissue fibrosis.22 Therefore, diuretics shouldn't be used unless there is evidence of excessive venous fluid.22
Until the lymphedema can be resolved, pain can be managed with non-opioid analgesics, relaxation techniques, mild to strong opioid analgesics, adjuvant medications—e.g., amitriptyline HCl (Elavil, Endep, others), or transcutaneous electrical nerve stimulation.1
• Surgery is reserved for grossly edematous limbs to de-bulk some of the tissue. The results have been varied, however, and conservative treatment is still required to manage the remaining lymphedema.22 Furthermore, surgical interventions on any lymphedematous limb can present severe and chronic wound healing problems.
In lymphedema, two additional dimensions of nursing care should not be overlooked: patient education and psychosocial health. Patient education is critical both for the prevention and management of lymphedema—especially for cancer patients.23
Clinicians need to educate patients about the risk of developing lymphedema after surgery or radiation therapy, how to recognize lymphedema symptoms early, and how to manage the condition. You will need to advise patients, for instance, to wear loose-fitting clothing, not to cross their legs while sitting, and to avoid sitting in one position for more than 30 minutes.
It's also extremely important to assess the psychosocial effects of lymphedema. The condition can cause major disfigurement and disability—an obstacle that cancer patients or survivors are often ill-equipped to overcome.
Patients who experience pain from the condition report higher levels of body image disturbance and decreased sexual drive.24 The added burden of laborious, time-consuming treatments for lymphedema can further assail the patient's already fragile psyche. Psychosocial issues may be addressed by group or individual counseling, along with practical information on diet and exercise, and advice for picking comfortable and flattering clothes.
Your efforts to care for patients who have—or are at risk for—lymphedema can do much good. That's true not only when it comes to patients' physical recovery, but when it comes to their psychological well-being, as well.
1. National Cancer Institute. "Lymphedema." 2002. www.cancer.gov/cancerinfo/pdq/supportivecare/lymphedema/HealthProfessional (1 June 2003).
2. Erickson, V. S., Pearson, M. L., et al. (2001). Arm edema in breast cancer patients. J Natl Cancer Inst, 93(2), 96.
3. Burt, J., & White, G. (1999) Lymphedema. Alameda, CA: Hunter House Publishers.
4. Thibodeau, G. A., & Patton, K. T. (2003). Anatomy and physiology (5th ed). St. Louis: Mosby.
5. National Lymphedema Network. "Lymphedema: A brief overview." www.lymphnet.org/whatis.html (21 Apr. 2003).
6. Wilson, W., & Sande, M. A. (2001) Current diagnosis and treatment in infectious diseases. New York: McGraw-Hill.
7. Board, J., & Harlow, W. (2002). Lymphedema: Classification, signs, symptoms and diagnoses. Br J Nurs, 11(6), 391.
8. Brennan, M. J. (1992). Lymphedema following surgical treatment of breast cancer: A review of pathophysiology and treatment. J Pain Symptom Manage, 7(2), 110.
9. Mortimer, P. S. (2000). Swollen lower limb—2: Lymphedema. BMJ, 320(7248) 1527.
10. Getz, D. H. (1985). The primary, secondary, and tertiary nursing interventions of lymphedema. Cancer Nurs, 8(3), 177.
11. Lund, E. (2000). Exploring the use of CircAid legging in the management of lymphoedema. Int J Palliative Nurs, 6(8), 384.
12. Casley-Smith, J. R., Casley-Smith, J. R. (1992). Modern treatment of lymphoedema. I. Complex physical therapy: The first 200 Australian limbs. Australas J Dermatol, 33(2), 61.
13. Boris, M., Weindorf, S., & Lasinkski, S. (1997). Persistence of lymphedema reduction after noninvasive complex lymphedema therapy. Oncology, 11(1), 113.
14. Daane, S, Poltoratszy, P., & Rockwell, W. B. (1998). Postmastectomy lymphedema management: Evolution of the complex decongestive therapy technique. Ann Plast Surg, 40(2), 128.
15. Brennan, M. J., & Miller, L. T. (1998). Overview of treatment options and review of the current role and use of compression garments, intermittent pumps and exercise in the management of lymphedema. Cancer, 83(12), 2822.
16. Board, J., & Harlow, W. (2002). Lymphoedema 3: The available treatments for lymphoedema. Br J Nurs, 11(7), 447.
17. Badger, C. M., Peacock, J. L., & Mortimer, P. S. (2000). A randomized, controlled parallel-group clinical trial comparing multilayer bandaging followed by hosiery vs hosiery alone in the treatment of patients with lymphedema of the limb. Cancer, 88(12), 2835.
18. Kloth, L. C., & McCulloch, J. M. (Eds.). (2001). Wound healing: Alternatives in management. Philadelphia: F. A. Davis.
19. Jeffs, E. (1998). Management of chronic edema. J Wound Care, 7(9), 3.
20. Cotton, C. (2003). Lymphedema: Assessment and management options. Rehab and Community Care, 12(1), 24.
21. Cohen, S. R., Payne, D. K., & Tunkel, R. S. (2001). Lymphedema: Strategies for management. Cancer, 92(4), 983.
22. Moseley, A., & Piller, N. (2002). The assessment and care of the patient with secondary limb lymphoedema. Aust Nurs J, 10(Suppl. 2), 1.
23. Passik, S. D., & MacDonald, M. V. (1998). Psychosocial aspects of upper extremity lymphedema in women treated for breast carcinoma. Cancer, 83(12), 2818.
24. Newman, M. L., Brennan, M., & Passik, S. (1996). J Pain Symptom Manage, 12(6), 376.
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