Prevention
        Education
        Exercise
        Other
Treatment
        Exercise
        Gradient pressure garments
        Bandages
        Manual lymphedema therapy
        Skin care
        Complex decongestive therapy
        Intermittent external pneumatic compression
        Pharmacologic therapy
        Weight loss
        Low-level laser therapy
        Surgery
Refractory Lymphedema and Complications

Prevention

Education

Ideally, prevention should begin before treatment, by educating the patient and family in a sensitive fashion about the potential risk of developing lymphedema. Proceeding in this way may diminish a breast cancer survivor’s dissatisfaction with the educational information received about lymphedema, and could serve as a foundation for making decisions and coping with lymphedema, should it develop later.[1] Patients should be taught to recognize the early signs of edema because treatment outcomes may be significantly improved if the problem is detected early.[2]

Traditionally, patients with breast cancer and axillary dissection have been warned against vigorous, repetitive, or excessive upper-body exercise because of concern about inducing lymphedema. Several reports have questioned this historical recommendation. One case series evaluated arm circumferences of 20 breast cancer patients who had a history of axillary dissection (level I–II), many of whom also received breast or axillary radiation therapy. While training and competing in the vigorous upper-body sport of dragon boat racing, none of the women showed a clinically significant difference in arm circumference after approximately 7 or 8 months.[3] As a precaution, however, the women were advised to wear compression sleeves on the affected upper extremity during exercise training.

A randomized trial examined the effects of a supervised upper- and lower-body weight training program on the incidence and symptoms of lymphedema in 45 breast cancer survivors. All patients had axillary dissection as part of their treatment, and 13 women had lymphedema at baseline. A 6-month, twice-a-week weight training program did not increase the risk of lymphedema or exacerbate symptoms.[4]

Additional research with larger samples, more rigorous designs, and more sophisticated outcome tools to measure lymphatic flow is needed to address the safety and effectiveness of exercises for women with breast cancer–related lymphedema.[5]

Generally anecdotal recommendations for taking preventive measures include the following:

Hygiene: Skin and Nail Care

• Maintain meticulous skin hygiene and nail care to prevent a portal of entry for infection which may result in cellulitis.



• Cut toenails straight across; see a podiatrist as needed to prevent ingrown nails and infections.



• Use skin moisturizers and topical antibiotic solutions after small breaks in the skin, such as paper cuts.



• Use sunscreen and suntan gradually.



• Wear cotton socks; keep feet clean and dry.



• Wear gardening and cooking gloves and use thimbles for sewing.



• Avoid going barefoot outdoors.



• Avoid blood draws (including finger sticks), vaccinations, or intravenous lines in the affected arm.



• Use the unaffected extremity to test temperatures (e.g., for bath water or cooking), as sensation may be diminished.



• Be aware of the signs of infection (fever, swelling, redness, pain, and heat) and see a physician for evaluation immediately.

Extremity Positioning

• Keep the arm or leg elevated above the level of the heart when possible.



• Avoid constrictive pressure on the affected arm or leg:
  • Do not cross legs while sitting.
  
  
  
  • Wear loose jewelry and clothes with no constricting bands.
  
  
  
  • Carry a handbag on the opposite arm.
  
  
  
  • Do not use elastic bandages and stockings with constrictive bands.
  
  
  
  • Do not sit in one position for longer than 30 minutes.
  
  
  

Avoiding the Pooling of Blood in the Involved Extremity

• Avoid rapid circular movements that cause centrifugal pooling of fluid in distal parts of the limb.



• Avoid local application of heat to the limb, which may increase blood flow.



• Do not maintain the limb in a gravity-dependent position for long periods of time.



• Avoid tight-fitting clothing or blood pressure monitoring in the affected arm, which could cause a tourniquet effect and obstruct lymph flow.

The goal of lymphedema treatment centers on controlling limb swelling and minimizing complications; the underlying lymphatic vessel interruption cannot be corrected. Because clinical trials evaluating pharmacologic measures are generally not found to be effective, nonpharmacologic measures are the mainstay of treatment, with the goal of maximizing the activities of daily living, decreasing pain, increasing range of motion, and improving function.

Nonfatiguing exercises may induce sufficient muscle contraction to move lymph into terminal lymphangioles and reduce swelling. Aerobic exercise may also increase the tone of the sympathetic nervous system, which causes the lymph collector vessels to pump more vigorously.[6]

Gradient pressure garments (also known as lymphedema sleeves or stockings) generate greater pressures distally than proximally, which enhances mobilization of edema fluid. Some patients may require custom-made sleeves to achieve an appropriate fit. The use of these garments may be especially important at high altitudes, such as during air travel, because the ambient atmospheric pressure is less than the outlet transcapillary pressure within the superficial tissues, which can lead to worsening edema.

Bandaging involves the use of inelastic material to discourage reaccumulation of lymph by reducing capillary ultrafiltration and optimizing the efficacy of the intrinsic muscle pump. Bandaging may change an initially resistant limb to one with less edema, resulting in decreased limb volume and allowing a garment to be applied successfully.[7]

Manual lymphedema therapy (or massage therapy) involves the use of a very light superficial massage with gentle, rhythmic skin distention limited ideally to pressures of approximately 30 mmHg to 45 mmHg.[6] Manual lymphedema therapy decreases congested lymph by directing it to lymphotomes, or anatomic regions drained by a specific lymph node bed, with preserved drainage.[6] Massage begins on the unaffected lymphotome areas to direct the lymph away from the affected extremity.

Possible contraindications to massage therapy include the following:[8]

• Active cellulitis or other inflammations of the infected limb.



• Active neoplasia.



• Acute deep venous thrombosis.



• Congestive heart failure.



• Local massage over radiated soft tissue.

The goal of skin care is to minimize dermal colonization by bacteria and fungus, especially in the crevices, and hydrate the skin to control dryness and cracking.

Complex decongestive therapy is a multimodality program that consists of manual lymphedema drainage therapy, low-stretch bandaging, exercises, and skin care.[9] This approach has been recommended as a primary treatment by consensus panels and as an effective therapy for lymphedema unresponsive to standard elastic compression therapy.[10,11]

Complex decongestive therapy is divided into two successive phases. The first phase consists of intensive treatment to allow substantive reduction of lymphedema volume. The second phase consists of maintenance treatment at home. Compliance with the use of the elastic sleeve and low-stretch bandage has been found to be an important determinant of success with maintenance therapy at home.[9] Complex decongestive therapy has also been shown to improve lymphedema following groin dissection.[12] Patients should be referred to a properly trained therapist for optimal results.

Intermittent external pneumatic compression may also provide additional improvement with lymphedema management when used adjunctively with decongestive lymphatic therapy. One small randomized trial of 23 women with new breast cancer–associated lymphedema found an additional significant volume reduction when compared with manual lymphatic drainage alone (45% vs. 26%).[13] Similarly, improvements were also found in the maintenance phase of therapy. Concerns regarding the use of intermittent pneumatic compression include the optimum amount of pressure and treatment schedule and whether maintenance therapy is needed after the initial reduction in edema.[14] There is a theoretical concern that pressures higher than 60 mmHg and long-term use may actually injure lymphatic vessels.

No chronic pharmacologic therapy is recommended for patients with lymphedema. Diuretics are typically of little benefit and may promote intravascular volume depletion because the lymphedema fluid cannot be easily mobilized into the vascular space. Coumarin is associated with significant hepatotoxicity and has not been found to have any benefit in controlled trials.[15] Antibiotics should be used promptly for patients with evidence of cellulitis; intravenous use may sometimes be required for severe cellulitis, lymphangitis, or septicemia.

The results of a randomized trial have suggested that lymphedema may improve with weight loss.[16] The mechanism by which obesity may predispose to lymphedema is unclear, but proposed mechanisms include an increased risk of postoperative complications, including infection, reduced muscle pumping efficiency, and separation of lymphatic channels by subcutaneous fat.[16]

Studies suggest that low-level laser therapy may be effective in reducing lymphedema in a clinically meaningful way for some women.[17] Two cycles of laser treatment were found to be effective in reducing the volume of the affected arm, extracellular fluid, and tissue hardness in approximately one-third of patients with postmastectomy lymphedema at 3 months posttreatment.[17] Suggested rationales for laser therapy include a potential decrease in fibrosis, stimulation of macrophages and the immune system, and a possible role in encouraging lymphangiogenesis.[17]

Surgery is rarely performed on patients who have cancer-related lymphedema. The primary surgical method for treating lymphedema consists of removing the subcutaneous fat and fibrous tissue with or without creation of a dermal flap within the muscle to encourage superficial-to-deep lymphatic anastomoses. These methods have not been evaluated in prospective trials, with adequate results for only 30% of patients in one retrospective review. In addition, many patients face complications such as skin necrosis, infection, and sensory abnormalities.[18] The oncology patient is usually not a candidate for these procedures. Other surgical options include the following:

• Microsurgical lymphaticovenous anastomoses, in which the lymph is drained into the venous circulation or the lymphatic collectors above the area of lymphatic obstruction.



• Liposuction.



• Superficial lymphangiectomy.



• Fasciotomy.

If lymphedema is massive and refractory to treatment, or has an onset several years after the primary surgery without obvious trauma, a search for other etiologies should be undertaken. Of particular importance is exclusion of the recurrence of tumor or the development of lymphangiosarcoma, which should be excluded with computed tomography or magnetic resonance imaging. The complication of lymphangiosarcoma is classically seen in the postmastectomy lymphedematous arm (Stewart-Treves syndrome). The mean time between mastectomy and lymphangiosarcoma is 10.2 years, with a median survival of 1.3 years. Clinically, the lesions of lymphangiosarcoma may initially appear as blue-red or purple with a macular or papular shape in the skin. Multiple lesions are common; subcutaneous nodules may appear and should be carefully evaluated in the patient who has chronic lymphedema.[19]

References

1. Ridner SH: Pretreatment lymphedema education and identified educational resources in breast cancer patients. Patient Educ Couns 61 (1): 72-9, 2006.  [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. Harris SR, Niesen-Vertommen SL: Challenging the myth of exercise-induced lymphedema following breast cancer: a series of case reports. J Surg Oncol 74 (2): 95-8; discussion 98-9, 2000.  [PUBMED Abstract]
   
   
4. 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]
   
   
5. Bicego D, Brown K, Ruddick M, et al.: Exercise for women with or at risk for breast cancer-related lymphedema. Phys Ther 86 (10): 1398-405, 2006.  [PUBMED Abstract]
   
   
6. Petrek JA: Commentary: prospective trial of complete decongestive therapy for upper extremity lymphedema after breast cancer therapy. Cancer J 10 (1): 17-9, 2004. 
   
   
7. 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]
   
   
8. The diagnosis and treatment of peripheral lymphedema. Consensus document of the International Society of Lymphology Executive Committee. Lymphology 28 (3): 113-7, 1995.  [PUBMED Abstract]
   
   
9. Vignes S, Porcher R, Champagne A, et al.: Predictive factors of response to intensive decongestive physiotherapy in upper limb lymphedema after breast cancer treatment: a cohort study. Breast Cancer Res Treat 98 (1): 1-6, 2006.  [PUBMED Abstract]
   
   
10. Didem K, Ufuk YS, Serdar S, et al.: The comparison of two different physiotherapy methods in treatment of lymphedema after breast surgery. Breast Cancer Res Treat 93 (1): 49-54, 2005.  [PUBMED Abstract]
    
    
11. Koul R, Dufan T, Russell C, et al.: Efficacy of complete decongestive therapy and manual lymphatic drainage on treatment-related lymphedema in breast cancer. Int J Radiat Oncol Biol Phys 67 (3): 841-6, 2007.  [PUBMED Abstract]
    
    
12. Hinrichs CS, Gibbs JF, Driscoll D, et al.: The effectiveness of complete decongestive physiotherapy for the treatment of lymphedema following groin dissection for melanoma. J Surg Oncol 85 (4): 187-92, 2004.  [PUBMED Abstract]
    
    
13. Szuba A, Achalu R, Rockson SG: Decongestive lymphatic therapy for patients with breast carcinoma-associated lymphedema. A randomized, prospective study of a role for adjunctive intermittent pneumatic compression. Cancer 95 (11): 2260-7, 2002.  [PUBMED Abstract]
    
    
14. Dini D, Del Mastro L, Gozza A, et al.: The role of pneumatic compression in the treatment of postmastectomy lymphedema. A randomized phase III study. Ann Oncol 9 (2): 187-90, 1998.  [PUBMED Abstract]
    
    
15. Loprinzi CL, Barton DL, Jatoi A, et al.: Symptom control trials: a 20-year experience. J Support Oncol 5 (3): 119-25, 128, 2007.  [PUBMED Abstract]
    
    
16. Shaw C, Mortimer P, Judd PA: Randomized controlled trial comparing a low-fat diet with a weight-reduction diet in breast cancer-related lymphedema. Cancer 109 (10): 1949-56, 2007.  [PUBMED Abstract]
    
    
17. Carati CJ, Anderson SN, Gannon BJ, et al.: Treatment of postmastectomy lymphedema with low-level laser therapy: a double blind, placebo-controlled trial. Cancer 98 (6): 1114-22, 2003.  [PUBMED Abstract]
    
    
18. Chilvers AS, Kinmonth JB: Operations for lymphoedema of the lower limbs. A study of the results in 108 operations utilizing vascularized dermal flaps. J Cardiovasc Surg (Torino) 16 (2): 115-9, 1975 Mar-Apr.  [PUBMED Abstract]
    
    
19. Tomita K, Yokogawa A, Oda Y, et al.: Lymphangiosarcoma in postmastectomy lymphedema (Stewart-Treves syndrome): ultrastructural and immunohistologic characteristics. J Surg Oncol 38 (4): 275-82, 1988.  [PUBMED Abstract]