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Lymphedema (PDQ®)
Patient VersionHealth Professional VersionEn españolLast Modified: 11/29/2007



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Overview







Management






Complications






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Management

Prevention
Treatment
        Compression garments
        Pharmacologic therapy
        Dietary management
        Pain management
        Complications
Psychosocial Considerations



Prevention

It is important to identify patients at risk for lymphedema early and to begin preventive monitoring and instruction for self-care. Inadequate nutritional status, obesity, immobility, and other medical conditions may increase the risk of developing lymphedema. The following parameters may facilitate early detection of the condition:

  • The ratio of actual to ideal weight.
  • Extremity measurements.
  • Ability to perform activities of daily living (at each physician examination).
  • History of contributing factors (e.g., edema).
  • Previous radiation therapy or surgery.
  • Concurrent medical illnesses (e.g., diabetes, hypertension, kidney or cardiac disease, or phlebitis).

Patients should also be assessed for knowledge of their disease and the potential for developing lymphedema. Deficient lymphatic drainage due to node dissection and/or radiation therapy predisposes the affected limb to serious infection. Even minor infection of the limb may lead to significant lymphedema.

Patients should understand the potential for developing lymphedema and should be instructed on limb and skin care following surgery or radiation therapy. (See the list of Considerations for Teaching Patients Prevention and Control of Lymphedema, below.) There is no empirical evidence for these or similar recommendations, though giving advice to avoid injury and infection in the affected limb seems intuitive. Lymphedema may occur as many as 30 or more years postsurgery. Breast cancer patients who comply with instruction on skin care and exercises following mastectomy show a significantly lower incidence of lymphedema.[1]

Lymphatic drainage is improved by tissue compression from muscular contractions during exercise. In exercise, muscles squeeze the soft tissue causing lymph to travel proximally to the vascular system.[2] Therefore, exercise is important in the prevention of lymphedema. Breast cancer patients should be instructed on hand and arm exercises following mastectomy. Patients who undergo operative procedures affecting pelvic lymph node drainage should be instructed in how to perform appropriate leg and ankle exercises. The physician should determine how soon the exercise is initiated following surgery. Physiatrists or therapy professionals should be consulted for a tailored program of exercises for each patient. One study of breast cancer survivors who performed twice-a-week weight training over a 6-month period did not note an increase or exacerbation of symptoms related to lymphedema.[3]

Because the recovery rate is increased when lymphedema is detected early,[2] patients should be taught to recognize the early signs of edema and to report any of the following symptoms to their doctor: feelings of tightness in the extremity; shoes that don’t fit; decreased strength; pain, aching, or heaviness; redness, swelling, or signs of infection. Rings may become tight as well, but patients are discouraged from wearing them on the side of risk.

Considerations for Teaching Patients Prevention and Control of Lymphedema

  1. Keep arm or leg elevated above the level of the heart, when possible. Avoid rapid circular movements that cause centrifugal pooling of fluid in distal parts of the limb.


  2. Clean and lubricate the skin of the extremity daily.


  3. Avoid injury and infection of the affected limb:
    • Upper extremities:
      • Use an electric razor for shaving.
      • Wear gardening and cooking gloves; thimbles for sewing.
      • Maintain good nail care; do not cut cuticles.


    • Lower extremities:
      • Wear foot coverings outdoors.
      • Keep feet clean and dry; wear cotton socks.
      • Cut toenails straight across; see a podiatrist as needed to prevent ingrown nails and infections.


    • Either upper or lower extremities:
      • Suntan gradually; use sunscreen.
      • Clean breaks in skin with soap and water, then use antibacterial ointment.
      • Use gauze wrapping instead of tape, but avoid a tourniquet effect.
      • Consult physician about rashes.
      • Avoid invasive venipuncture, including finger sticks and intravenous fluid administration, in affected extremity.
      • Avoid extreme hot or cold (e.g., heating pads or ice packs) .
      • Avoid prolonged and strenuous work with the affected extremity.




  4. Avoid constrictive pressure on the arm or leg:
    • Do not cross legs while sitting.
    • Wear loose jewelry and clothes with no constricting bands.
    • Carry handbag on opposite arm.
    • Do not use blood pressure cuffs on the vulnerable limb.
    • Do not use elastic bandages and stockings with constrictive bands.
    • Do not sit in one position without change for longer than 30 minutes.


  5. Watch for signs of infection (e.g., redness, pain, heat, swelling, fever). Call your physician immediately if signs or symptoms occur.


  6. Practice prescribed exercises, as instructed.


  7. Keep regular follow-up appointments with your physician.


  8. Closely observe all areas of the limb daily for signs of problems:
    • Measure the circumference of the arm or leg at intervals suggested by your physician/therapist at two consistent levels on the limb and report any sudden increase in size to your physician.


  9. Sensation may be diminished. Use the unaffected extremity to test temperatures (e.g., for bath water, cooking).


Treatment

There are two broad categories of conservative management or treatment of lymphedema: mechanical and pharmacologic. Mechanical modalities include elevation of the affected limb; manual lymphatic drainage (a form of massage that mobilizes edema fluid from distal to proximal areas and from areas of stasis to areas of healthy lymphatics); use of multilayered compression bandages and custom-fitted pressure-graded garments; and meticulous skin hygiene to prevent infection. The use of multilayered bandaging for 18 days before the use of hosiery doubled the reduction in limb volume over the next 6 months.[4] A number of these modalities have been combined in a strategy known as complex physical therapy (or complex decongestive therapy), which consists of manual lymphedema treatment, compression wrapping, individualized exercises, and skin care, followed by a maintenance program.[5-7] Complex physical therapy has been recommended by consensus panels and is an effective approach for lymphedema that is unresponsive to standard elastic compression therapy. It must be performed by a properly trained therapist.

Surgical interventions are not recommended because they are not generally successful in curing lymphedema. Several techniques that have been tried include staged excision of the skin and subcutaneous tissue with or without skin grafting and the Thompson dermal flap, which combines excision of edematous tissue with burying a shaved dermal flap to establish continuity between the superficial and deep lymphatic vessels. These methods have minimal success and high complication rates of skin necrosis, infection, and sensory difficulties.[8] The oncology patient is usually not a suitable candidate for these techniques.

Compression garments

Compression garments should always cover the entire area of edema. For example, a stocking that reaches only to the knee tends to become tight and occludes lymphatic and venous return if there is significant edema in the thigh. Extremity pumps that use intermittent sequential pneumatic compression may also be helpful in the management of the edematous limb, though many feel such pumps are ineffective and potentially counterproductive. The cuff is alternately inflated and deflated according to a controlled time cycle. This action increases fluid flow in the veins and lymphatic vessels and prevents the accumulation of residual fluid in the limb. Compression pumps should be used only under the supervision of a trained health care professional. High external pressure can damage superficial lymphatic vessels. Furthermore, when compression pumps and other techniques are used, caution should be exercised if there is a potential for residual tumor, which some theorize may be mobilized into venous or lymphatic channels.

Pharmacologic therapy

Pharmacologic therapy uses antibiotics to treat and prevent bacterial cellulitis and lymphangitis. Other drugs that have been used include diuretics, anticoagulants, pantothenic acid, pyridoxine, and hyaluronidase. These drugs have no proven therapeutic value and may cause adverse reactions.[9]

It is important to determine the specific etiology of the swelling and to treat it appropriately. Infection is a frequent sequela of edema and causes increased capillary permeability, which increases protein deposition in the tissues. If an infection is diagnosed, appropriate antibiotics should be given that are effective against gram-positive cocci and, less frequently, fungal infections. Laboratory data (e.g., complete blood cell count [CBC]) should be evaluated. Because massage and techniques to encourage drainage would be contraindicated if venous thrombosis is present, diagnostic tests may be indicated to distinguish vascular blockage from deep vein thrombosis. If thrombosis is found, anticoagulation therapy should be given.

Coumarin (Chemical Abstracts Service registry number 91-64-5; NSC 8774; systematic name 2H-1-Benzopyran-2-one, also referred to as 5,6-benzo-[a]-pyrone), is a compound that has been studied for the management of high-protein lymphedemas such as those associated with local and regional treatments for neoplastic diseases.[10,11]

In the United States, dietary supplements such as coumarin are regulated as food, not drugs. Premarket approval by the U.S. Food and Drug Administration (FDA) are not required unless specific disease prevention or treatment claims are made. Because dietary supplements are not required to be reviewed for manufacturing consistency and no specific standards for dose or purity exist, there may be considerable variation from lot to lot for all products marketed as dietary supplements.

Coumarin was formerly used in the United States as a fixative and flavoring agent in foods and as a pharmaceutical excipient. In response to investigations by coumarin manufacturers that demonstrated the compound caused liver toxicity in animals when used in amounts comparable to or greater than that appearing in human foods, it was reclassified by the FDA in 1954 as a food adulterant. Since that time, its addition to human foods has been prohibited, and importation of coumarin-containing foodstuffs from outside the United States is not permitted. Coumarin is marketed for medical use in several European countries, but its therapeutic use has not been approved in the United States or Canada.

Adverse effects commonly associated with coumarin include mild nausea and diarrhea.[10] Liver toxicity has been reported in as many as 6% of treated patients.[12-15] Patients typically present with increased serum concentrations of hepatic transaminases, with or without coincidentally increased serum bilirubin.[16,17] Aberrant laboratory values generally resolve within a few weeks after coumarin treatment is discontinued; however, liver pathology may be progressive and fulminant despite withdrawal of the compound.[18] Long-term toxicity data are sparse for patients who have received continuous treatment for up to 2 years. The clinical toxicity of longer durations of coumarin treatment has not been investigated. Animal toxicology studies have shown that the incidence of coumarin-induced hepatotoxicity is highly variable between species.[19] Reports of hepatic toxicity in humans have led to coumarin’s removal from the market in some European countries as well as in Australia.

In one study, coumarin administered as tablets for oral use at a daily dose of 400 mg was shown to partially reverse edema fluid accumulation, to reduce the size of swollen extremities, and to decrease the discomfort associated with lymphedema.[10] However, a double-blind placebo-controlled crossover study of 140 women with lymphedema of the arm following treatment for breast cancer demonstrated that coumarin was not more effective than placebo in the treatment of lymphedema. This study also found a higher incidence (6%) of coumarin-associated hepatic toxicity and concluded that coumarin was not a safe or effective treatment for lymphedema.[11,15]

Diuretics encourage vascular fluid depletion, but they do nothing for excess protein deposits and could hasten connective tissue fibrosis.[20] Therefore, diuretics should be used with caution and only for the treatment of excess vascular fluid due to other causes.

Dietary management

The nutritional status of the patient should be evaluated and supportive measures instituted as required. Hypoalbuminemia encourages fluid to pass into interstitial tissues with excess protein and higher colloid osmotic pressure. The serum albumin level should be kept above 2.5 g/dL. Patient weight should be monitored, and patients should be encouraged to eat protein-rich foods and supplements.

Pain management

Patients with lymphedema may experience pain as a result of pressure on nerve endings or as a result of atrophy or muscle contractures during movement.[1] Following assessment, pain may be managed with nonopioid analgesics, relaxation techniques, mild-to-strong opioid analgesics, adjuvant drugs (e.g., amitriptyline), and/or transcutaneous electrical nerve stimulation (TENS). The most successful treatment, however, is reduction of the lymphedema.

Complications

Edematous tissues are less well nourished and more prone to necrosis during immobility. Therefore, patients with lymphedema should be monitored for areas of skin breakdown, especially over bony prominences.

Excess pressure on inguinal or pelvic lymphatics may indicate pelvic metastasis with subsequent interference of bladder emptying. Pressure, in conjunction with opioids, may cause problems with bowel elimination. Patient bladder and bowel status should be monitored for signs of urinary retention or constipation.

Psychosocial Considerations

There are multiple psychosocial and adjustment issues faced by cancer patients and survivors with lymphedema. Because lymphedema is disfiguring and sometimes painful and disabling, it can create problems in many aspects of functioning, e.g., psychologic, physical, and sexual. Historically, however, inadequate attention was paid to the psychosocial impact of lymphedema. Several articles have noted that women who develop lymphedema following treatment for breast cancer encounter more difficulties in each of these aspects of functioning than women who do not develop the condition after such treatment.[21-23] Additionally, because the treatments for upper extremity lymphedema can be uncomfortable, arduous, and time-consuming, the presence of psychologic difficulties can significantly interfere with treatment efforts. Upper extremity pain in women following breast cancer can have a highly complex differential diagnosis. One study has highlighted the deleterious impact of pain on quality of life and coping in patients with upper extremity lymphedema.[24] Using the European Organisation for Research and Treatment of Cancer (EORTC) Quality of Life Questionnaire C30 (QLQ-C30), a study of 744 breast cancer patients found that those with lymphedema had impaired quality of life.[25]

Another study highlighted the factors associated with psychologic distress within a group of patients who developed upper extremity lymphedema after breast cancer treatment. Risk factors for poor adjustment to the condition include poor social support, use of an avoidant and reclusive style of coping (some women avoid social situations in which their lymphedema causes a constant reminder of their cancer experience), and the presence of pain of any intensity.[21] Group and individual counseling that provides specific information about preventive measures, the role of diet and exercise, advice for selecting comfortable and flattering clothing, and emotional support can be helpful to women coping with lymphedema. (Refer to the PDQ summaries on Normal Adjustment and the Adjustment Disorders and Sexuality and Reproductive Issues for more information.)

References

  1. Getz DH: The primary, secondary, and tertiary nursing interventions of lymphedema. Cancer Nurs 8 (3): 177-84, 1985.  [PUBMED Abstract]

  2. Markowski J, Wilcox JP, Helm PA: Lymphedema incidence after specific postmastectomy therapy. Arch Phys Med Rehabil 62 (9): 449-52, 1981.  [PUBMED Abstract]

  3. Ahmed RL, Thomas W, Yee D, et al.: Randomized controlled trial of weight training and lymphedema in breast cancer survivors. J Clin Oncol 24 (18): 2765-72, 2006.  [PUBMED Abstract]

  4. Badger CM, Peacock JL, Mortimer PS: A randomized, controlled, parallel-group clinical trial comparing multilayer bandaging followed by hosiery versus hosiery alone in the treatment of patients with lymphedema of the limb. Cancer 88 (12): 2832-7, 2000.  [PUBMED Abstract]

  5. Casley-Smith JR, Casley-Smith JR: Modern treatment of lymphoedema. I. Complex physical therapy: the first 200 Australian limbs. Australas J Dermatol 33 (2): 61-8, 1992.  [PUBMED Abstract]

  6. Boris M, Weindorf S, Lasinkski S: Persistence of lymphedema reduction after noninvasive complex lymphedema therapy. Oncology (Huntingt) 11 (1): 99-109; discussion 110, 113-4, 1997.  [PUBMED Abstract]

  7. Daane S, Poltoratszy P, Rockwell WB: Postmastectomy lymphedema management: evolution of the complex decongestive therapy technique. Ann Plast Surg 40 (2): 128-34, 1998.  [PUBMED Abstract]

  8. Savage RC: The surgical management of lymphedema. Surg Gynecol Obstet 160 (3): 283-90, 1985.  [PUBMED Abstract]

  9. Brennan MJ: Lymphedema following the surgical treatment of breast cancer: a review of pathophysiology and treatment. J Pain Symptom Manage 7 (2): 110-6, 1992.  [PUBMED Abstract]

  10. Casley-Smith JR, Morgan RG, Piller NB: Treatment of lymphedema of the arms and legs with 5,6-benzo-[alpha]-pyrone. N Engl J Med 329 (16): 1158-63, 1993.  [PUBMED Abstract]

  11. Loprinzi CL, Kugler JW, Sloan JA, et al.: Lack of effect of coumarin in women with lymphedema after treatment for breast cancer. N Engl J Med 340 (5): 346-50, 1999.  [PUBMED Abstract]

  12. Casley-Smith JR, Casley-Smith JR: Frequency of coumarin hepatotoxicity. Med J Aust 162 (7): 391, 1995.  [PUBMED Abstract]

  13. Beinssen AP: Possible coumarin hepatotoxicity. Med J Aust 161 (11-12): 725, 1994 Dec 5-19.  [PUBMED Abstract]

  14. Cox D, O'Kennedy R, Thornes RD: The rarity of liver toxicity in patients treated with coumarin (1,2-benzopyrone). Hum Toxicol 8 (6): 501-6, 1989.  [PUBMED Abstract]

  15. Loprinzi CL, Sloan J, Kugler J: Coumarin-induced hepatotoxicity. J Clin Oncol 15 (9): 3167-8, 1997.  [PUBMED Abstract]

  16. Morrison L, Welsby PD: Side-effects of coumarin. Postgrad Med J 71 (841): 701, 1995.  [PUBMED Abstract]

  17. Faurschou P: Toxic hepatitis due to benzo-pyrone. Hum Toxicol 1 (2): 149-50, 1982.  [PUBMED Abstract]

  18. Bassett ML, Dahlstrom JE: Liver failure while taking coumarin. Med J Aust 163 (2): 106, 1995.  [PUBMED Abstract]

  19. Fentem JH, Fry JR: Species differences in the metabolism and hepatotoxicity of coumarin. Comp Biochem Physiol C 104 (1): 1-8, 1993.  [PUBMED Abstract]

  20. Földi E, Földi M, Weissleder H: Conservative treatment of lymphoedema of the limbs. Angiology 36 (3): 171-80, 1985.  [PUBMED Abstract]

  21. Passik SD, Newman ML, Brennan M, et al.: Predictors of psychological distress, sexual dysfunction and physical functioning among women with upper extremity lymphedema related to breast cancer. Psychooncology 4 (4): 255-63, 1995. 

  22. Maunsell E, Brisson J, Deschênes L: Arm problems and psychological distress after surgery for breast cancer. Can J Surg 36 (4): 315-20, 1993.  [PUBMED Abstract]

  23. Tobin MB, Lacey HJ, Meyer L, et al.: The psychological morbidity of breast cancer-related arm swelling. Psychological morbidity of lymphoedema. Cancer 72 (11): 3248-52, 1993.  [PUBMED Abstract]

  24. Newman ML, Brennan M, Passik S: Lymphedema complicated by pain and psychological distress: a case with complex treatment needs. J Pain Symptom Manage 12 (6): 376-9, 1996.  [PUBMED Abstract]

  25. Kwan W, Jackson J, Weir LM, et al.: Chronic arm morbidity after curative breast cancer treatment: prevalence and impact on quality of life. J Clin Oncol 20 (20): 4242-8, 2002.  [PUBMED Abstract]

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