Understanding and Recognizing Lymphedema
If you know what to look for, you will find it.
Lymphedema is a chronic, incurable disease that can be readily managed with the appropriate interventions. It is a vascular disorder that is either due to congenital malformations of the lymphatic system (primary etiology) or related to an insult or injury to the lymphatic system (secondary etiology) such as surgery, trauma, radiation, malignancies or infections.
The clinical course of lymphedema involves a progressive swelling of the limb or areas affected due to a mechanical insufficiency of the lymphatic system. Over time, this protein-rich fluid burden may lead to various integumentary complications ranging from fibrosis and hyperkeratosis to weeping skin with or without ulceration.
The challenge is to appreciate the magnitude of those affected by both lymphedema and integumentary dysfunction. Even though millions are affected by various forms of lymphedema worldwide, the existing literature fails to fully define the population-based prevalence, health outcomes, and treatment costs of this disorder. This information is even harder to obtain or quantify for lymphedema patients with associated integumentary complications.
Although evidence-based, best-practice guidelines have been outlined in consensus documents that lymphedema is a relatively underrecognized condition in both medical and public domains. The reasons for this include a lack of public awareness of the condition, insufficient education and knowledge among health-care providers regarding its etiology and management, and limited reimbursement coverage to support lymphedema care models.1 This is further compounded by the fact that patients with integumentary dysfunction are also faced with the same limitations. Although highly interrelated, lymphedema and integumentary dysfunction have traditionally been taught, researched and even managed separately. Progress has only recently been made addressing the interdependent nature of these conditions.
A rough population prevalence rate for chronic nonhealing wounds in the United States is 2% of the general population;² this translates to roughly 7 million individuals. A conservative estimate of the cost of caring for chronic wounds exceeds $50 billion per year.3-5 The majority of chronic nonhealing wounds involves peripheral vascular disease (arterial and venous insufficiency). Estimated prevalence reports of chronic venous insufficiency vary geographically (highest reports in western countries) and range from < 1% to 40% in females and from < 1% to 17% in males.6 To gain perspective, 20-30% of patients with advanced chronic venous disease will have associated lymphatic dysfunction believed to be related to overload from the fluid burden, damage from venous hypertension and repeated infections (cellulitis).7-9 This form of lymphedema is referred to as phlebolymphedema (see Photo 1).
With respect to lymphedema, it is estimated that 3-5 million people in the United States alone suffer from this disease, yet it is likely that estimate is not truly reflective because many remain undiagnosed and/or underreported. More data are available on lymphedema secondary to various forms of cancer. The overall risk for lymphedema for all cancers is reported to be 15.5%.10 Specifically, lymphedema is reported to occur in up to 49% of breast, 20% of gynecologic, 16% of melanoma, 10% of genitourinary and 6% of head and neck cancer patients after lymph node dissection and/or radiotherapy. Additionally, 10% of patients who receive isolated axillary sentinel lymph node biopsy report subjective symptoms of lymphedema.10-17 On a global scale, the leading cause of lymphedema is due to lymphatic filariasis, a parasitic form of lymphedema spread through mosquitos. More than 1.2 billion people are at risk for contracting lymphatic filariasis (LF), which is one of 13 neglected tropical diseases and one of only six infectious diseases considered eliminable by the World Health Assembly. This disfiguring and debilitating disease affects more than 120 million people in 83 countries. (See Photos 2-3.)
To understand lymphedema, it is important to appreciate the function of the lymphatic system. In addition to immune defense, the basic purpose of the lymphatic system is to facilitate fluid movement from the interstitial tissues back to the venous circulation to help maintain a normal fluid balance. The lymphatic system has four major roles:
- Fluid in the interstitial spaces is continuously circulating. As plasma and plasma proteins escape from the small blood vessels, they are picked up by the lymphatic system and returned to the blood circulation. The lymphatic system reabsorbs/transports substances that the venous system cannot through the mechanisms of Starling’s law/ equilibrium.
- The lymphatic system acts as a safety valve for fluid overload and helps keep edema from forming. As the interstitial fluid increases, the interstitial fluid pressure increases, which causes an increase in the local lymph flow. The local lymph system can be temporarily overwhelmed by sudden local increases in the interstitial fluid and pitting edema will result (as in the case of a sprained ankle).
- The homeostasis of the extracellular environment is maintained by the lymphatic system. The lymphatic system removes excess protein molecules and waste from the interstitial fluid. The large protein molecules and fluids that cannot reenter the circulatory vessels gain entry into the blood circulation through the terminal lymphatics.
- The lymphatic system also cleanses the interstitial fluid and provides a blockade to the spread of infection or malignant cells in the lymph nodes.
There are two main classifications of lymphedema: primary and secondary. Primary lymphedema is caused by developmental abnormalities of the lymphatic system, such as incomplete development of lymph vessels, reduced number or absent lymphatic structures, enlarged and incompetent vessels, or fibrosis of lymph vessels and nodes. Congenital lymphedema occurs within first two years of life, lymphedema praecox before age 35, and lymphedema tarda after age 35.18
Secondary lymphedema is more prevalent than primary lymphedema. A number of risk factors can contribute to the development of secondary lymphedema. Classification of causes include trauma and tissue damage, malignant disease, venous disease, infection, inflammation, endocrine disease, immobility and dependency, and factitious.19 Risk factors that can lead to secondary lymphedema include but are not limited to breast, head and neck, uterine and prostate cancer; chronic venous insufficiency; deep vein thrombosis (DVT); thrombophlebitis; vein stripping or harvesting; hysterectomy; orthopedic surgery; obesity; cellulitis and wound infections; chronic wounds; orthopedic injuries; burns; scarring; immobility from advancing age or hemiparesis; congestive heart failure; and renal insufficiency.19 Factors that complicate the care of a patient with lymphedema include peripheral arterial disease, obesity, diabetes, neuropathy, limited mobility and strength, difficulties with activities of daily living, financial constraints, depression, social isolation, lack of family support for long-term management, and other psychosocial factors.
The current staging system for lymphedema is rudimentary at best. Further work is needed to create a lymphedema severity score that will provide a better assessment of the complexities of each patient. The International Society of Lymphology (ISL) lymphedema staging system is as follows:
Stage 0: This is the latency stage. Risk factors and co-morbidities are present. Swelling is not yet evident despite impaired lymph transport. There are subtle changes in tissue fluid/composition and changes in subjective symptoms. Stage 0 may exist months or years before overt edema occurs (Stages I-III).
Stage I: This stage represents an early accumulation of fluid relatively high in protein content, which subsides with limb elevation. Pitting may occur. An increase in various proliferating cells may also be seen.
Stage II: In this stage, limb elevation alone rarely reduces tissue swelling, and pitting is manifest. Late in Stage II, the limb may or may not pit as excess fat and fibrosis develop.
Stage III: This stage encompasses lymphostatic elephantiasis where pitting can be absent and trophic skin changes such as papillomatosis and hyperkeratosis are present. Further deposition of fat and fibrosis and warty overgrowths may have developed.20
The ISL staging system does not reflect lymphedema severity in terms of volume of the limb. For example, within Stage III, one patient may have severe skin changes, massive enlargement of the extremity and large, overhanging lobes of hardened, fibrotic tissue. This patient may require many months of intensive treatment and more than one staff member to treat the limb, and fitting for long-term compression can be complex. In contrast, another patient may have minimal increase in limb size but with focal areas of advanced skin changes, resulting in classification of the lymphedema as Stage III.
Within each Stage 0–III, the severity of lymphedema can also be graded according to percent volume difference between limbs: minimal = <20% volume increase, moderate = 20%–40% volume increase, and severe = >40% volume increase.18 This can be calculated if only one arm or leg is affected, however, and is difficult to objectively measure in other areas of the body.
Complete decongestive therapy (CDT) is considered the standard of care for lymphedema management. The focus of CDT is to achieve a reduction in limb volume and improve the quality and integrity of the skin. CDT is typically divided into two phases: an intensive phase and a maintenance phase. The intensive phase is best performed daily (or as frequently as possible) until maximal volume reduction is achieved. Once the lymphedematous limb has plateaued and is no longer achieving a reduction in volume, the patient is transitioned into the maintenance phase, which is continued for life.
Throughout both phases of CDT, patient education is paramount because self-care is critical for successful long- term outcomes.21 Although there is no cure for lymphedema, it is a disease that can be successfully managed, improving function and quality of life.
CDT involves the following components that are guided by a trained lymphedema specialist during the intensive phase:
- meticulous skin and nail care of the affected areas
- approximately 60 minutes of manual lymph drainage (MLD), a gentle manual technique that helps redirect lymph flow
- multilayer, short-stretch compression bandaging
- diaphragmatic breathing to stimulate the thoracic duct (largest lymph vessel of the body)
- over-the-counter or custom compression garments once the limb has decongested
All aspects of CDT continue during the maintenance phase with a few modifications. The maintenance phase is also patient-directed and is necessary for lifelong management of lymphedema. Compression garments are worn during the day followed by short-stretch bandaging or the use of a proper compression system at night. Compression garments or systems can employ different closure mechanisms (such as Velcro), assisting the patient with donning and doffing and often, improving adherence with use.
Proper identification and diagnosis is essential for patients with lymphedema. Recognizing the predisposing risk factors, signs, and symptoms of lymphedema will assist with differential diagnosis when faced with complex patient presentations. Many patients with lymphedema have concomitant comorbidities, which may complicate medical management. It is imperative to appreciate all contributing factors to individualize interventions and modify treatment approaches as applicable. Chronic wounds directly or indirectly related to lymphedema are not uncommon.
Proper management involves addressing the underlying medical conditions or diseases that may be the likely cause of ulceration, while simultaneously addressing skin and nail care and providing proper complete decongestive therapy, modified according to the patient’s specific needs, clinical presentation, and functional impairments. Referral to a certified lymphedema therapist and/or trained physician specialist is of utmost importance. (See Photo 4, which depicts lower-extremity lymphedema before and after a few months of CDT.)
- Stout N, Weiss R, Feldman, J, Stewart B, Amer J, Cormier J, Shi Y. A systematic review of care delivery models and economic analysis in lymphedema: Health Policy Impact 2004-2011. Lymphology. 2013; 46:27-41.
- Sen CK, Gordillo GM, Roy S, et al. Human skin wounds: a major and snowballing threat to public health and the economy. Wound Repair Regen. 2009; 17(6):763–771.
- Kuhn BA, Coulter SJ. Balancing ulcer cost and quality equation. Nurs Econ. 1992; 10(5):353–359.
- Hess CT. Putting the squeeze on venous ulcers. Nursing. 2004; 34(Suppl):8–13
- Driver VR, Fabbi M, Lavery LA, Gibbons G. The costs of diabetic foot: the economic case for the limb salvage team. J Am Podiatr Med Assoc. 2010; 100(5):335-341.
- Beebe-Dimmer J, Pfeifer J, Engle J, Schottenfeld D. The epidemiology of chronic venous insufficiency and varicose veins. Ann of Epidemiology. 2005; 15(3):175-184.
- Bull R, Gane J, Evans J, Joseph A, Mortimer P. Abnormal lymph drainage in patients with chronic venous leg ulcers. J Am Acad Dermatol 1993; 28:585-590.
- Collins P, Villavicencio J, Abreu S, Gomez E, Coffey J, Connaway C, et al. Abnormalities of lymphatic drainage in lower extremities: a lymphoscintigraphy study. J Vasc Surg 1989; 9:145-152.
- Gloviczki P, Calcagno D, Schriger A, Pairolero P, Cherry K, Hallett J, et al. Noninvasive evaluation of the swollen extremity; experiences with 190 lymphoscintigraphic examinations. J Vasc Surg 1989; 9:683-689.
- Cormier JN, Askew RL, Mungovan KS, et al. Lymphedema beyond breast cancer: a systematic review and meta-analysis of cancer-related secondary lymphedema. Cancer. 2010; 116:5138–5149.
- Schook CC, Mulliken JB, Fishman SJ, Grant FD, Zurakowski D, Greene AK. Primary lymphedema: clinical features and management in 138 pediatric patients. Plast Reconstr Surg. 2011; 127:2419–2431.
- Kim JH, Choi JH, Ki EY, et al. Incidence and risk factors of lower-extremity lymphedema after radical surgery with or without adjuvant radiotherapy in patients with FIGO stage I to stage IIA cervical cancer. Int J Gynecol Cancer. 2012; 22:686–691.
- Petrek JA, Senie RT, Peters M, Rosen PP. Lymphedema in a cohort of breast carcinoma survivors 20 years after diagnosis. Cancer. 2001; 92:1368–1377.
- Wilke LG, McCall LM, Posther KE, et al. Surgical complications associated with sentinel lymph node biopsy: results from a prospective international cooperative group trial. Ann Surg Oncol. 2006; 13:491-500.
- Chang SB, Askew RL, Xing Y, et al. Prospective assessment of postoperative complications and associated costs following inguinal lymph node dissection (ILND) in melanoma patients. Ann Surg Oncol. 2010; 17:2764–2772.
- Hunt KK, Askew R, Cormier JN. Measuring lymphedema in patients with breast cancer: go with the flow? Breast Cancer Res Treat. 2009; 117:559–560.
- Disipio T, Rye S, Newman B, Hayes S. Incidence of unilateral arm lymphoedema after breast cancer: a systematic review and meta-analysis. Lancet Oncol. 2013; 14:500–515.
- Zuther JE, Norton S. Lymphedema Management: The Comprehensive Guide for Practitioners. 3rd Edition. New York: Thieme; 2013.
- Framework L. Best practice for the management of lymphoedema. International Consensus. London: MEP Ltd. 2006:3-52.
- ISL. The diagnosis and treatment of peripheral lymphedema: 2013 consensus document of the international society of lymphology. Lymphology. 2013; 46(1):1-11.
- Lasinski B, Thrift, K, Squire D, Austin M, Smith K, Wanchai A, et al. A systematic review of the evidence for Complete Decongestive Therapy in the treatment of lymphedema from 2004 to 2011. Amer Acad Phys Med Rehab. 2012; 4: 580-601.