Proper, timely diagnosis is crucial for patients living with lymphedema, and all wound care clinics should have a structured plan for care management. This article will educate providers on risk factors and symptoms, and will assist with making differential diagnosis for this complex patient population.
As healthcare providers, it is of utmost importance to recognize the interplay between the lymphatic system and the human body’s other systems, specifically the integumentary system. The lymphatic system is intimately involved with the venous and arterial systems. The veins and lymphatics have a similar embryological origin and anatomy. When pathological changes occur in the venous system, there are microangiopathic changes of both the vascular and lymphatic networks. Additionally, the health of the arterial, venous, and lymphatic systems is often represented and manifested in the skin, as evidenced by characteristic skin changes commonly associated with arterial, venous, and lymphatic pathologies.
Proper identification and diagnosis is crucial for patients living with lymphedema. Recognizing the predisposing risk factors, signs, and symptoms of lymphedema will assist wound care clinicians with differential diagnosis when faced with complex patient presentations. Many lymphedema patients also live with concomitant comorbidities and experience integumentary dysfunction, which may complicate medical management. Proper management involves addressing the underlying medical conditions or diseases. We must employ an integrated systems approach to manage these patients to maximize resources and improve outcomes. This article will educate providers in newly opened and existing wound care clinics on the development of an effective, comprehensive lymphedema management program. Patient case studies are also included.
PRIMARY & SECONDARY LYMPHEDEMA
The lymphatic system has been described as a nodal-centric immune-vascular system. When the system is diseased or impaired, lymphedema typically manifests. Although lymphedema is a chronic, incurable disease, it can be readily managed with the appropriate interventions. Disorders involving the lymphatic system that lead to lymphedema are 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, and/or infections.
Primary lymphedema is caused by developmental abnormalities of the lymphatic system, such as incomplete development of lymph vessels (hyper-/hypoplasia), reduced number or absent lymphatic structures, enlarged and incompetent vessels, or fibrosis of lymph vessels and nodes. Congenital lymphedema occurs within the first two years of life; lymphedema praecox (ie, Meige disease, characterized by swelling of the soft tissues in which an excessive amount of lymph has accumulated, generally in females) before age 35 and lymphedema tarda (primary lymphedema, legs are most often affected) after age 35.1 Secondary lymphedema is more prevalent than primary lymphedema. There are a number of risk factors that can contribute to the development of secondary lymphedema. Classification of causes include trauma and tissue damage, malignancies, venous disease, infection, inflammation, endocrine disease, immobility and dependency, and factitious.2 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 and fat disorders; cellulitis and wound infections; chronic wounds; orthopedic injuries; burns; scarring; immobility from advancing age or hemiparesis; congestive heart failure (CHF); and renal insufficiency.2 Factors that complicate the care of lymphedema include peripheral arterial disease, obesity, diabetes, neuropathy, limited mobility and strength, difficulties with activities of daily living (ADLs), financial constraints, depression, social isolation, lack of family support for long-term management, and other psychosocial factors.
A thorough physical examination is essential in correctly diagnosing lymphedema.3 The diagnosis of lymphedema is made in most cases by patient history, systems review, inspection, palpation, and a few select noninvasive tests such as volume or girth measurement. At present, the only clinical test that has been shown to be a reliable and valid method to diagnose lymphedema is Stemmer’s sign.4,5 The Stemmer’s sign test is performed by pinching and lifting the skin on the dorsal aspect of the base of the second toe or finger of the involved extremity. Normally, the skin in this area is thin and pliable, but as lymphedema develops and progresses, the skin thickens and becomes more fibrotic. If the skin cannot be pinched and lifted, the test yields a positive Stemmer’s sign. The sign is never falsely positive and is considered accurate to diagnose lymphedema of the extremities.6 When the skin at the base of the second toe or finger can be pinched and lifted, the sign is negative. A negative sign may be falsely negative and does not exclude lymphedema. When the sign is negative, it is appropriate to treat the edema with conventional interventions of elevation, rest, and compression. If the edema does not respond to conventional interventions, it should be monitored and regularly reassessed, as the underlying pathology may be early lymphedema without overt fibrotic changes in the tissues. A thorough history is essential to correctly identify lymphedema. The history should include:
- Onset of symptoms (swelling, “heaviness” of limb), length of time since initial onset, and the triggering event (ie, injury to the skin, sprained ankle, recent surgery or trauma), if known.
- Current and past medical history, including traumatic events and surgery. This should also include all current medications, health risk factors, coexisting problems, and family history.
- Pain and/or associated discomfort. Significant pain may suggest additional tests and measures to determine if a secondary problem exists, such as venous or arterial compromise or an underlying orthopedic condition. Most lymphedema patients report “heaviness” and associated discomfort due to the large amount of fluid in the tissues.
- Functional status and activity level. Has this impacted the ability to perform ADLs at home, work, and/or school?
- Review of social habits such as smoking, diet and nutritional habits, and physical fitness/weight-management strategies.
- Past treatment and intervention history.7
Following the history, a brief systems review should be performed. This will help the healthcare provider identify other problems that may require referral to another professional, and it may assist in identifying specific tests and measures to use to complete a thorough patient assessment. Additionally, it is important to review the patient’s affect, cognition, language, and learning style to optimize the examination and intervention strategies.
Complete decongestive therapy (CDT) is considered the standard of care for lymphedema management. The objective of CDT is to achieve a reduction in limb volume and improve the integrity and quality of the skin. CDT is typically divided into two phases: an intensive phase, carried out by a trained healthcare provider, and a maintenance phase that is patient-dependent. 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. Although there is no cure for lymphedema, it is a disease that can be successfully managed while improving function, and quality of life can be achieved. Throughout both phases of CDT, patient education is paramount because self-care is critical for successful long-term outcomes.3 CDT involves the following components, which should be guided by a trained lymphedema specialist during the intensive phase and modified for the patient to continue during the maintenance phase: 1) meticulous skin and nail care; 2) manual lymph drainage (MLD); 3) short-stretch compression bandaging; 4) diaphragmatic breathing to stimulate the thoracic duct; 5) therapeutic exercises to promote lymphatic pumping under compression; and 6) over-the-counter, custom compression garments or alternative wraps once the limb has decongested. All aspects of CDT continue during the maintenance phase, with a few modifications. The maintenance phase is patient-directed and is necessary for lifelong management of lymphedema. Compression garments or alternative wraps are worn during the day, followed by short-stretch bandaging or the use of a proper compression system at night.
EDEMA VS. LYMPHEDEMA
There are numerous patients being treated in outpatient wound care clinics living with existing ulcers and edematous extremities. It is imperative to differentially diagnose the underlying cause of edema, as not all swelling is equal. Although most healthcare providers do not receive extensive training in lymphatic pathophysiology, it is important to realize that long-standing chronic venous insufficiency (CVI), with or without repeated bouts of infection, leads to fatigue and damage of the lymphatic system, often inducing a secondary lymphedema (phlebolymphedema). Roughly 10 million Americans live with lymphedema while more than 30 million Americans live with venous disease; however, according to the Society for Vascular Medicine, only 10% seek treatment. It is estimated that 80% of lower extremity ulcerations are of venous origin. Venous ulcers can be challenging to manage, as recurrence may be as high as 37%. Further, recidivism is more likely to be associated with patients who do not receive corrective surgical procedures and in those with deep venous pathology.8 Edema is a natural response to an injury/inflammation, or it can be related to various medical conditions or medications. Edema due to injury or inflammation is a protective response and an essential step that occurs to bring necessary cells to the injured area. The circulatory system helps with moving oxygenated blood and delivering water and nutrients to every cell. Once the blood becomes deoxygenated, the blood (along with waste products such as carbon dioxide) returns to the heart. Muscles in the legs help improve the venous transport efficiency to the heart. When this homeostasis is not maintained, the likelihood of accumulation of excessive fluid exists. Edema is the accumulation of excessive fluid in the tissues and is usually related to the lymphatic system being temporarily overwhelmed in its ability to maintain fluid homeostasis. Edema can develop in localized areas or over the entire body. Often, edema is typically found in the feet, ankles, legs, and/or hands, and can be classified by its location. For example, accumulation of excessive fluid in the brain is known as cerebral edema, accumulation of fluid in the abdominal cavity is known as ascites, enlargement of the legs is known as peripheral edema, enlargement of the scrotum is known as scrotal edema, and excessive fluid in the lungs is known as pulmonary edema. Cells usually contain a large amount of fluid, but outside of the cells most fluid can be found in the blood vessels and interstitial spaces. This is where accumulation of excessive fluid will be located — in the interstitial space and/or blood vessels.
To review how blood vessels play a role in the accumulation of excessive fluid, let’s review the anatomy and function of one of the key vessels — the capillary, the smallest blood vessel in the body whose main function is to allow the exchange of water, oxygen, nutrients, and other substances between the blood and the surrounding tissues. This is where these substances are delivered to the cells, and it is where waste products are removed. The capillary is composed of an arterial and venous component that is covered by a basal membrane, a semipermeable layer that allows the transport of smaller molecules. On the arterial side of the capillary, there is an arteriole sphincter that contracts or dilates to control the proper amount of fluid entering the space, allowing for filtration. On the venous side of the capillary, the pressure is less than on the arterial side and reabsorption will occur, accounting for 90% of the water being reabsorbed from the interstitial tissues. The normal distance that oxygen, nutrients, and key cells travel from the capillary to the tissue is approximately 1/10 mm. This distance is referred to as the diffusion distance. Edema increases the diffusion distance, even when not clinically visible, and can create complications as the normal diffusion distance increases, therefore compromising the local immune response as key cells, oxygen, and nutrient delivery is delayed to the cells and tissues due to the increased distance traveled. Determining the underlying cause of edema is essential and will help guide treatment, interventions, and prognosis. Normally, the hydrostatic pressure (pushing force) and colloidal osmotic pressure (pulling force) in the blood vessel will remain constant, allowing the capillaries to filter and reabsorb fluid in a homeostatic state. Alterations in vascular permeability can impact this homeostatic state, resulting in various causes of edema. For example, an increase in capillary filtration may result from an infection or damage to the capillary arteriole sphincter, as seen in patients living with radiation injuries. Another cause of edema could be related to a decrease in intravascular osmotic pressure. In conditions where this balance is disrupted (eg, hypoproteinemia) more fluid will escape into the interstitial space.
A third process could be related to an increase in intravascular hydrostatic pressure, such as an obstruction on the venous side. This will decrease the amount of fluid that can be reabsorbed on the capillary venous side, thereby increasing the amount of fluid in the interstitial space. Damage or impairment of the lymphatic system can also result in edema. This form of edema results from a higher colloidal osmotic pressure in the interstitial space, as larger quantities of proteins are not being absorbed by the damaged system, nor is the remaining 10% of water. Proteins, being hydrophilic, will attempt to continue “pulling” a larger amount of water into the interstitial space, resulting in an increase in filtration on the capillary arterial side and a decrease in reabsorption on the capillary venous side — an imbalance in fluid homeostasis. This form of edema is referred to as lymphedema due to the excessive accumulation of protein-rich fluid.
There are two classifications of edema: high-protein and low-protein. Lymphedema is a high-protein edema that is the result of damage or absence of the normal lymphatic system. The lymphatic system is responsible for picking up and transporting proteins (large molecules) back into the venous system. When proteins do not get picked up by the lymphatic capillaries (due to some problem with the lymphatic system), they are left behind in the interstitial tissues. These proteins cause fibrosis of the tissues and, being hydrophilic, attract more water to the interstitial tissues, making the swelling or lymphedema worse. The swelling associated with lymphedema is a high-protein edema, unlike “regular” edema, which is mostly water. Regular edema will not lead to fibrosis, whereas lymphedema can lead to significant fibrotic changes in the dermal tissues. In clinical practice, an under-recognized problem is the relationship between the venous and lymphatic systems. CVI leads to venous hypertension, which results in a high filtration pressure causing increased fluid in the tissues and an increased lymphatic water load. When the lymphatic transport capacity is exceeded by the water load, a state of low-protein edema occurs. Over time, constant lymphatic hypertension causes infiltration of lymph into the perilymphatic tissues, leading to a fatigued system and fibrosclerotic changes in the tissues. This increase in protein permeability leads to further lymphatic damage (fatigue), and lymphedema (high-protein edema) becomes the underlying pathology that contributes to the formation of venous ulceration — CVI and lymphedema are interrelated!
Compression is the cornerstone intervention for most edemas and swelling. Contraindications to compression exist and include, but are not limited to, severe arterial insufficiency, certain systemic disorders, hypoproteinemia, uncompensated CHF, and acute DVT. Based on the etiology, physicians may treat edema or swelling solely with diuretics or a pneumatic pump. Diuretics and pneumatic pumps work to mobilize fluid; however, neither is able to address or manage the protein in the interstitial tissues. Both can exacerbate lymphedema as the remaining proteins, being hydrophilic, attract more water to the interstitial spaces. Further, the stagnating proteins create a pathohistological state of chronic inflammation that leads to connective tissue proliferation and fibrosclerosis. Lymphedema is best treated with CDT, a major aspect of which is the use of compression following MLD.
MLD can affect the proteins and facilitate their uptake back into the lymphatic system, where it is then routed to functional lymphatic structures. Compression following MLD will help to maintain the benefits of fluid reduction and prevent rebounding. Compression supports both the venous and lymphatic systems, and is enhanced by walking and exercise to facilitate muscle pumping. Compression for lymphedema/phlebolymphedema does require training and practice for optimal outcomes and safe application. Many types of compression are available, including multilayer bandaging systems, short-stretch bandages, elastic bandages, inelastic bandages (eg, Unna’s boot), intermittent pneumatic compression, over-the-counter and custom garments, and alternative compression wraps with various closure mechanisms. Healthcare providers must understand the etiology of the edema/swelling in order to effectively and efficiently match the compression needs to the patient.
For patients living with lymphedema/phlebolymphedema, short-stretch bandaging is essential to provide a high working pressure and low resting pressure to support lymphatic flow and proper hemodynamics.
Heather Hettrick is an associate professor in the physical therapy program at Nova Southeastern University and a faculty member at the International Lymphedema and Wound Training Institute. Her current research interests reside in game-based learning, innovations in teaching and learning, integumentary dysfunction, and the application of forensic science to wound management. Frank Aviles, Jr. is wound care service line director at Natchitoches (LA) Regional Medical Center; wound care and lymphedema instructor at the Academy of Lymphatic Studies, Sebastian, FL; physical therapist/wound care consultant at Louisiana Extended Care Hospital, Natchitoches; and physical therapist/wound care consultant at Cane River Therapy Services LLC, Natchitoches.
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