Kimberly D. Leaird
INTRODUCTION
This chapter will address lymphedema management and the evaluation and treatment of patients fitting the model of preferred Practice Pattern 6H: Impaired Circulation and Anthropometric Dimensions Associated with Lymphatic System Disorders.1 Knowledge of this practice pattern will enable therapists to effectively evaluate, differentiate between diagnoses, and provide appropriate intervention for patients with lymphatic system dysfunction.
This chapter provides a review of the anatomy and physiology of the lymphatic system, discussions of the stages of lymphedema, and evidence to refute and support current methods of treatment for lymphatic system disorders. It is imperative to have a basic understanding of the lymphatic system in order to deliver appropriate interventions.
PURPOSE OF THE LYMPHATIC SYSTEM
In addition to immune defense, the purpose of the lymphatic system is to drain substances that the venous blood circulation is unable to reabsorb. The lymphatic system functions like a “sweeper” to clear the interstitial space (also known as the third space or tissue space) of excess fluids, cellular debris, long-chain fatty acids (found only in the intestines), and protein molecules, otherwise known as the lymphatic loads.2 There is a continual shift of fluids within the body at the microcirculatory level of the blood capillaries. It is the responsibility of the lymphatic system to facilitate the fluid movement from the tissues back into the bloodstream—to maintain a state of homeostasis. Under normal physiological conditions, this fluid management will “maintain blood volume and eliminate chemical imbalances in the interstitial fluid.”2–5
OVERVIEW OF THE LYMPHATIC SYSTEM
Lymphatic vessels, lymph nodes, and other lymphoid organs and tissues comprise the lymphatic system. The lymph system represents an accessory route by which fluid can move from the tissue spaces into the blood. The lymph system protects the body by removing foreign material from the lymph fluid; that is, it filters lymph fluid and constantly surveys the body for the presence of foreign material. Lymph tissues contain macrophages, lymphocytes (T cells or B cells), plasma cells that produce antibodies, and reticular cells that form the lymphoid tissue stroma.3,4
The main organs of the lymph system are the lymph nodes, spleen, thymus, and tonsils. Lymph nodes appear in clusters or chains intermittently along lymphatic vessels, and their primary responsibility is to filter the lymph fluid. There are approximately 600 to 700 lymph nodes in an average-size person. A larger number of lymph nodes are strategically located in the cervical region (100–200) and in the mesentery (200). A fibrous capsule surrounds each lymph node that encloses the cortex and the medulla. The cortex of the lymph node contains mostly lymphocytes, which act in the immune response. The medulla contains macrophages, which engulf and destroy viruses, bacteria, and other foreign debris. Lymph moves quickly into the lymph node via afferent lymphatic vessels from the capsular side of the node and proceeds slowly through the chambered areas of the medulla. Lymph fluid leaves the nodes via the efferent lymphatic vessels from the hilus area of the node. There are more afferent vessels leading into the nodes than efferent vessels leaving the nodes. This causes lymph flow to almost stagnate in the lymph node, allowing time to cleanse the lymph fluid2–4 (see Fig. 22-1).
FIGURE 22-1 Lymph node. (Reprinted with permission from Shier D, Butler J, Lewis R. Hole’s Human Anatomy and Physiology. 9th ed. Boston, MA: The McGraw-Hill Companies Inc; 2002.)
The spleen provides a site for lymphocyte proliferation and immune function and destroys aged or defective red blood cells and blood-borne pathogens. The thymus is most functional during youth. Many of the thymus cells are inactive; however, some mature into T lymphocytes, which support immunity function. The tonsils and other lymph node aggregates known as Peyer patches (distributed throughout the mucous lining of the small intestines) function to prevent pathogens in the respiratory and digestive tracts from penetrating the mucous membrane lining.4,6
The lymphatic system is similar to the cardiovascular system in that it aids in the circulation of body fluids. The driving force behind the cardiovascular system is the heart muscle, which serves to propel the fluids. However, there is no “central pump” for the lymphatic system. Lymph return to the venous circulation occurs via the contraction of smooth muscles in the lymph collectors, the contraction of surrounding skeletal musculature, the pulsation of adjacent arteries and veins, and diaphragmatic breathing.2–5
The lymphatic system is not a closed system like the circulatory system, but a one-way system that starts in the interstitial spaces with initial lymph vessels (initial lymph capillaries) and ends in the venous part of the blood circulatory system. One of the most important functions of the lymphatic system is its ability to remove proteins from the tissue spaces. Reabsorption of large, protein macromolecules will not occur at the blood capillaries.2–5 The “removal of proteins from the interstitial spaces is an essential function, without which we would die in approximately 24 hours”4 (see Fig. 22-2).
FIGURE 22-2 Schematic representation of lymphatic vessels transporting fluid from the interstitial spaces to the venous system. (Reprinted with permission from Shier D, Butler J, Lewis R. Hole’s Human Anatomy and Physiology. 9th ed. Boston, MA: The McGraw-Hill Companies Inc; 2002.)
ANATOMY OF THE LYMPHATIC SYSTEM
The lymphatic system is divided into the superficial and deep systems and separated by the fascia. There are many connections between the two systems; therefore, the transport of lymph occurs from distal to proximal and, in general, from superficial to deep. The purpose of the superficial lymphatic system is to drain the lymphatic loads of the skin: water, cells, and proteins. Fat is found only in the digestive system, cisterna chyli, and thoracic duct as a lymphatic load. The deep lymphatic system drains muscles, tendons, joints, inner organs, and so forth—everything but the skin. Lymphedema presents more frequently in the superficial lymphatic system. The deep lymphatic system is often not involved because the fascia provides compression to resist swelling.2–4
Generally, there are no lymph vessels in areas without blood supply. The major exception to this is the central nervous system (CNS). Obviously, there is a blood supply to the brain and spinal cord, but there are no lymphatic vessels in the CNS to drain the lymphatic loads produced by the brain and spinal cord. There are no lymph vessels found in the nails, hair, dentin of the teeth, inside of the eyes, and bone tissue.2–4
Lymphatic Vessels
Lymphatic vessels are also referred to as lymphatics. The lymphatic vessels absorb interstitial fluid and return this fluid to the venous circulation. The lymphatics begin as initial lymph vessels and initial lymph capillaries. The initial lymph vessels represent the beginning of the one-way lymphatic system and begin “blindly” in the interstitium2–4 (see Fig. 22-3).
FIGURE 22-3 Initial lymph capillaries are microscopic, one-way vessels that begin “blindly” in the interstitial spaces. The black arrows indicate blood flow, and white arrows indicate lymph flow. (Reprinted with permission from Shier D, Butler J, Lewis R. Hole’s Human Anatomy and Physiology. 9th ed. Boston, MA: The McGraw-Hill Companies Inc; 2002.)
Initial lymph capillaries are slightly larger than blood capillaries and are composed of a single layer of endothelial cells. Many of these cells do not connect end to end as with blood capillaries but actually overlap at their junction—with the potential to create large openings. The areas of overlap resemble a “swinging flap” and create the one-way opening for fluid—particularly large molecules like proteins and the other lymphatic loads—to enter the lymphatic system2–4 (see Fig. 22-4).
FIGURE 22-4 Initial lymph capillary in the interstitium. (1) Arterial blood capillary loop, (2) venous blood capillary loop, (3) lymph capillary, (4) opening between 2 lymph capillary endothelial cells: “swinging flap,” (5) fibrocyte, (6) anchoring filament, (7) interstitial space. Small arrows indicate directional blood flow; large arrows indicate flow of interstitial fluid. (Reprinted with permission from Földi M, Kubik S. Lehrbuch der Lymphologie. 5th ed. Munich, Jena: Urban & Fischer; 2002. Copyright Urban & Fischer Verlag, Munich, Germany.)
Initial lymph capillaries of the skin are located just below the epidermis. These vessels are valveless and cover the entire body (excluding the previous areas identified that do not contain lymph vessels) to form a plexus. The lymph capillaries of the skin are responsible for draining an area 3 to 4 cm in circumference, which is called a lymphatic area. On the palms and soles of the feet, the lymphatic area is 1.5 to 2 cm in circumference.2,7
Endothelial cells that compose the initial lymph vessel connect to the cells and tissues in the surrounding interstitial space via semielastic anchoring filaments. If water increases in the tissues, an increase in pressure in the interstitial spaces will occur. Under normal physiological conditions, the continued water increase will cause a pull on the anchoring filaments and cause the overlapping junction areas to open. Pressure in the initial lymph capillary is lower than that in the interstitial spaces. This will cause the larger molecules of the lymphatic loads to move into the initial lymph vessels. Lymph formation occurs when the fluid from the interstitial spaces (containing the lymphatic loads) enters the initial lymph vessels.2–4
Precollectors connect the initial lymph capillaries to the lymph collectors of the superficial lymphatic system and are characterized by underdeveloped valves and muscles. The most numerous types of precollectors in the superficial system are previously described—those that connect the superficial lymph capillaries and collectors. The remainder of the precollectors provides a direct connection between the superficial lymph capillaries and the deep lymph collectors. These are called perforating precollectors—they perforate the fascia and provide a direct connection between the superficial and deep lymphatic system. Perforating precollectors are found in larger numbers in the parasternal, paravertebral, and intercostals areas.2,7
Collectors are transporting vessels that move lymph to regional lymph nodes. Collectors of the superficial lymphatic system are embedded in the superficial fatty tissue; they are above the fascia. These vessels are distinguished from precollectors in that collectors have well-developed valves (which prevent reflux and promote directional flow) and distinct wall layers (intima, media, and adventitia). The angion is the smallest functioning unit of the collector located between a proximal and a distal pair of valves. Angions are smooth muscles innervated by the sympathetic portion of the autonomic nervous system. Lymph is propelled through the collectors with the inherent contraction of each angion (every 6 seconds, or 10 times a minute at rest) in a segmental, caterpillar-type fashion called lymphangiomotoricity. The rate of contraction is determined by the volume of lymph and can increase up to 10 times above the resting rate. Transport of lymph through the collectors is supported by the contraction of surrounding skeletal muscles, external pressures, pulsation of arteries, and respiratory and thoracic pressure changes during breathing2 (see Fig. 22-5).
FIGURE 22-5 Light micrograph of valve in lymph collector (25X). (Reprinted with permission from Shier D, Butler J, Lewis R. Hole’s Human Anatomy and Physiology. 9th ed. Boston, MA: The McGraw-Hill Companies, Inc.; 2002.)
The lymph moves into the deep system and into larger collecting vessels called lymphatic trunks. Lymph from the upper extremities moves through collectors, to the regional lymph nodes (axillary lymph nodes), into their respective trunks, then into the left or right venous angle. From the lower extremities, lymph moves into the inguinal lymph nodes, pelvic nodes, and lumbar lymph nodes and then into their respective trunks. The left and right lumbar trunksconverge from the lower extremities with the gastrointestinal trunk (long-chain fatty acids return to the venous system here), which comes from the intestines. These three trunks converge to form the cisterna chyli at the T12 through L2 levels. The thoracic duct originates at the cisterna chyli and runs anterior to the vertebrae, continues slightly left of the vertebrae (at the T7 level), and returns lymph to the venous circulation at the left venous angle. The junction of the internal jugular and subclavian veins forms the venous angles on both the right and left sides of the body. The venous pressure is lower at the venous angles to allow for the return of lymph to the venous circulation. In a 24-hour period, 2 to 4 L of lymph is returned to the left venous angle by the thoracic duct, and approximately 300 mL (a 12-oz soda can) returns to the right venous angle2,5 (see Figs. 22-6 through 22-9).
FIGURE 22-6 Overview of thoracic duct and surrounding vessels. (1) left lumbar trunk; (2) right lumbar trunk; (3) cisterna chyli; (4) thoracic duct; (5) cervical part of thoracic duct; (6) esophagus; (7) trachea; (8) left venous angle; (9) right venous angle; (10) aorta; (11) azygos vein; (12) hemiazygos vein; (13) diaphram (a) medial portion, (b) Intermediate portion, (c) lateral portion; (14) right lymphatic duct; (15) superficial cervical artery. (Reprinted with permission from Földi M, Kubik S. Lehrbuch der Lymphologie, 5th ed. Munich, Jena: Urban & Fischer; 2002. Copyright Urban & Fischer Verlag, Munich, Germany.)
FIGURE 22-7 Lymphangiogram (radiograph) of the lymphatic vessels and lymph nodes of the pelvic region. (Reprinted with permission from Shier D, Butler J, Lewis R. Hole’s Human Anatomy and Physiology. 9th ed. Boston, MA: The McGraw-Hill Companies, Inc.; 2002.)
FIGURE 22-8 Schematic overview of lymphatic territories and watersheds of the upper extremity. (A) Schematic drawing. (a) Medial forearm territory with lymph vessels. (b) Radial forearm territory with lymph vessels. (c) Ulnar forearm territory with lymph vessels. (d) Medial upper arm territory with lymph vessels. (e) Dorsomedial upper arm territory with lymph vessels. (f) Lateral upper arm and shoulder territory with lymph vessels. (g) Right upper quadrant territory (anterior). (B) Cadaveric model. (1) Lymph nodes in deltopectoral groove. (2) Lymph vessels of upper arm territory (short type). (3) Supraclavicular lymph nodes. (4) Axillary lymph nodes. (5) Medial upper arm territory. (6) Anticubital lymph nodes. (Reprinted with permission from Földi M, Kubik S. Lehrbuch der Lymphologie. 5th ed. Munich, Jena: Urban & Fischer; 2002. Copyright Urban & Fischer Verlag, Munich, Germany.)
FIGURE 22-9 Schematic overview of lymphatic territories and watershed of the lower extremity. (1) Superficial inguinal lymph nodes; (2) prepubic lymph nodes; (3) lymph nodes of the penis; (4) popliteal lymph nodes; (5) plantar lymphatic plexus; (6) lymph vessels of medial sole of the foot; (7) interdigital lymph vessels; (8) lymph collectors on dorsum of foot; (9) ventromedial bundle (a) medial knee (bottleneck area); (10) dorsolateral bundle; (11) fascia; (12) adductor hiatus; (13) lymph vessels with femoral vein (deep); (14) gluteal-femoral sulcus (a) lymph vessels of gluteal area, (b) lymph vessels of gluteal area, (c) lymph vessels of the anus and scrotum, (d) lymph vessels of external genitalia, (e) lower anterior quadrant, (f) lower posterior quadrant. (Reprinted with permission from Földi M, Kubik S. Lehrbuch der Lymphologie. 5th ed. Munich, Jena: Urban & Fischer; 2002. Copyright Urban & Fischer Verlag, Munich Germany.)
MICROCIRCULATION AND THE LYMPHATIC SYSTEM
The transfer of nutrients to tissues and the removal of cellular waste products occur at the blood capillary level. The blood capillaries are located between the arterial (high-pressure) and venous (low-pressure) systems and are formed by a single layer of endothelial cells that are highly permeable to small molecules.2,4,8
The transfer of nutrients and gases and reuptake of waste products occur at the blood capillary level via a process called diffusion. Diffusion is the one-way movement of particles from a higher to a lower concentration. The hydrostatic pressure and colloid osmotic pressure (oncotic pressure) assist the diffusion process at the blood capillaries. In most areas of the body, the normal diffusion distance between the cells and the interstitial tissues is 1/10 mm. With a mere 1-cm increase in edema, the normal diffusion distance is increased by 100 times.2,8
Filtration occurs primarily at the arterial end of the blood capillaries. The driving force behind filtration is hydrostatic pressure, which normally measures 29 mmHg. The colloid osmotic pressure at the arterial end measures 20 mmHg. The hydrostatic pressure acts to push water and other nutrients from the arterial end of the blood capillaries and into the tissue spaces.2,4,8
Reabsorption occurs primarily at the venous end of the blood capillaries. The colloid osmotic force of the plasma proteins (20 mmHg) will overcome the hydrostatic pressure exerted at the venous end (14 mmHg) to cause reuptake of waste products and water into the venous blood capillaries. These pressures were first measured by Dr. Ernest Starling and are known as Starling’s law or Starling’s equilibrium. For more detailed information on Starling’s equilibrium, please refer to Chapter 16 of Guyton and Hall and to the Despopulos Color Atlas of Physiology.2,4,8
The number of blood capillaries in the human body would approximately cover the total surface area of a football field when placed end-to-end. Given this large surface area, and given that the average circulating blood volume is 4 to 6 L, there must be a mechanism to control the flow of blood into the capillaries.4,8
The precapillary sphincter is a smooth muscle located in the precapillary arterioles. Contraction of the precapillary sphincter allows less blood into the capillaries. Less blood in the blood capillaries will register a lower blood capillary pressure (BCP). Dilation of the sphincter will increase the flow of blood into the capillaries. The larger volume of blood in the capillaries will cause an increase in BCP. The higher BCP will ultimately lead to an increase in ultrafiltration. The amount of blood in the blood capillaries at any given time is due to the metabolic demand of the tissues.2,4,8
The Lymphatic Loads
Dr. Michael Földi coined the term lymphatic loads to describe the substances not reabsorbed by the venous system. The lymphatic loads include water, proteins, cells, and fat (in the digestive system). The following discussion will address each of the lymphatic loads and identify how each component becomes a lymphatic load.2,7
Water
Only a fraction of water becomes a lymphatic load. Water leaves the arterial end of the blood capillaries by filtration to supply the tissues with needed nutrients and water-soluble substances. Reabsorption returns water, solvents, lactic acid, and other cellular waste products to the venous circulation. Only 80% to 90% of the water returns to the venous blood capillaries. The 10% to 20% of water that remains in the interstitial spaces is termed the net filtrate. It is the responsibility of the lymphatic system to remove this 10% to 20%, which ultimately will become part of the lymphatic load of water. However, the total volume of the net filtrate equates much more than the 2 to 4 L of lymphatic water load returned from the interstitial space in a 24-hour period. The remaining filtrate is collected from the numerous blood capillaries at the lymph node level.2,7
Proteins
Blood is composed of roughly 52% to 62% of plasma and 38% to 48% of cells, which includes erythrocytes, leukocytes, and thrombocytes. There is approximately 3 L of plasma in an adult and 66 to 83 g/L of plasma proteins, which means there is approximately 200 g of protein molecules circulating in the plasma. Under normal physiological conditions, approximately half of the circulating proteins will leave the total blood capillary surface in 24 hours and go into the interstitial space for tissues repair, nourishment, and so forth. It is the responsibility of the lymphatic system to remove these proteins from the interstitial spaces and return them to systemic circulation.Proteins are macromolecules, and therefore physically too large to be reabsorbed at the venous blood capillaries.2–4,7
Cellular Components
All cells and macromolecular particles free in the interstitial space become a lymphatic load. Examples of these cells include cell fragments from hematomas, red and white blood cells, cancer cells, macrophages, pathogens (eg, silica dust, mites, or spores), either inhaled or ingested. All the aforementioned cells are macromolecules and the responsibility of the lymphatic system to return to the venous system.2–4,7
Long-Chained Fatty Acids
A large portion of the fat we ingest are long-chained fatty acids (LCFA), that is, composed of more than 16 carbon atoms. The LCFA enter the lymphatic system from the intestinal tract and are covered with a phospholipid coating to form a chylomicron. These chylomicrons are too large for reabsorption by the blood vessels of the small intestines and are absorbed by lymph vessels located in the intestines called chylous vessels.2–4,7
LYMPHEDEMA
Lymphedema is a high-protein edema that results from mechanical insufficiency of the lymphatic system. Lymphedema can occur anywhere in the body but occurs more frequently in the extremities. If left untreated, lymphedema can lead to significant pathological and clinical consequences for the patient.2,5
The transport capacity (TC) of the lymphatic system represents the maximum amount of lymph transported by the lymphatic system with the system working at its maximum frequency and amplitude. Lymph time volume (LTV) is the amount of lymph transported by the lymphatic system in a given unit of time. Normal values for LTV are 10% of the TC. The lymphatic load (LL) represents the normal amount of lymphatic load returning to the venous system in a 24-hour period, that is, 2 to 4 L2 (see Table 22-1).
TABLE 22-1 Normal Lymphatic System
Under normal physiological conditions, the lymphatic system will respond to an increase in the LL with an increase in LTV. This is known as the safety factor or safety valve function. To increase LTV, the lymphatic system will increase the frequency and contraction of the collectors2,7 (see Table 22-2).
TABLE 22-2 Safety Factor or Safety Valve Function
Lymphatic System Insufficiencies
If the lymphatic system is healthy, the transport capacity is 10 times higher than the normal amount of LL (2–4 L). There is a built-in functional reserve (FR) to handle any increase in LL. In a high-volume or dynamic insufficiency, the LL exceeds the TC of the intact lymphatic system. Edema will result in this scenario and the edema can be protein rich (in response to the inflammatory response or traumatic edema) or low in protein in nature2 (see Table 22-3).
TABLE 22-3 Dynamic Insufficiency
A low-volume insufficiency or a mechanical insufficiency occurs when the TC drops below the normal amount of LL. Morphological and functional changes have occurred to the lymphatic system, which limits the ability to transport lymph fluid. The most common causes of a reduced TC are a surgical procedure involving the lymphatic system (eg, mastectomy with lymph node removal, prostectomy), radiation of lymph nodes, a congenital malformation of the lymphatic system, trauma, and chronic venous insufficiency. Once the TC is reduced, it can never be returned to its original levels—prior to disease. Swelling will result from a mechanical insufficiency, and this swelling is called lymphedema2,5 (see Table 22-4).
TABLE 22-4 Mechanical Insufficiency
Classifications of Lymphedema
Primary lymphedema results from a malformation or dysplasia of the lymphatic system that can be hereditary or congenital. With primary lymphedema, development of the lymphatic vessels has been impaired. Hypoplasia is the most common type of dysplasia. Lymph vessels/collectors that are smaller than normal, or a lower number of lymph vessels in a given area, is characteristic of a hypoplasia. Hyperplasia means the lymph collectors are larger than normal, rendering their valves incompetent, and unable to transport lymph fluid effectively. An aplasia is the defective development or absence of lymph vessels in a given area. The most common area for an aplasia is the dorsum of the foot. Inguinal node fibrosis or Kinmonth syndrome is a dysplasia of the capsule and trabeculi area of the lymph nodes. Primary lymphedema can also be associated with Klippel–Trenaunay syndrome, which is a dysplasia of blood vessels, lymph vessels, and the skeletal system2,5 (see Table 22-5).
TABLE 22-5 Primary Lymphedema9
Secondary lymphedema results from a known insult to the lymphatic system. Worldwide, secondary lymphedema is more common than primary lymphedema. Possible causes of secondary lymphedema include, in no particular order, the following:
•Radiation therapy, which can cause fibrosis to the involved tissues. Fibrosis in an area can reduce collateral circulation and inhibit the flow of lymph through tissues, for example, lymph nodes.2,5
•Trauma to a given area, which can reduce the TC below the normal level or LL, giving rise to early signs of lymphedema.10
•Iatrogenic lymphedema, which may result following diagnostic and/or therapeutic treatments. Examples are lymphedema following mastectomy with lymph node removal, hysterectomy, perpendicular cuts across collectors.9
•Lymphatic filariasis, which describes infection caused by filarial Wuchereria bancrofti. This nematode worm is transmitted by the bite of a mosquito and lives and grows in the lymphatic system—causing permanent damage. These infections are more common in subtropical and tropical regions of the world.11
•Surgery that directly involves the lymphatic system. There are numerous studies that support the fact that there is a high correlation between the degree of axillary node dissection and the degree of inguinal node dissection with higher incidences of developing lymphedema.10,12–19
•Infection, which will ultimately increase the lymphatic load by increasing blood flow and capillary permeability in the area, which creates a dynamic insufficiency and could precipitate lymphedema.2,5
•Chronic venous insufficiency, which can exacerbate or initiate the symptoms of lymphedema due to the chronic stress on the venous system.2,5,11
•Benign or malignant tumors, which can grow in the lymph vessels and clot/block the lymphatics. Tumors can also grow outside the lymphatics and cause damage to the vessels by blocking flow.2,5,11
Precipitating Factors for Lymphedema
Evidence-based research is slow in coming to the field of lymphedema management. At this time, researchers are unable to clearly identify who will develop lymphedema.2,10,20 Because of this uncertainty, clinicians should work from a list of guidelines that suggest sensible and cautious practices to promote prevention.2 Those clinicians not familiar with lymphedema, or those who do not understand its development due to the lack of evidence-based data, tend to prescribe rapid return to normal, daily activities. Rather, patients should take a gradual progression to the return of daily activities.2
In an article written for the National Lymphedema Network (NLN), Dr. Michael Földi addressed the concern of a lack of evidence-based research in the following statement: “There are cases in which anecdotal observations are in harmony with scientific fact and established knowledge. It is textbook knowledge that healthy elastic fibers are prerequisite for lymph formation and that sunshine can destroy those elastic fibers. To try to achieve an evidence-based study would be unethical.”10 During this time of ongoing research and development, clinical prudence—not dismissal of non–evidence-based information—will arm therapists with information to manage lymphedema patients. Precautions and guidelines for patients with upper and lower extremity lymphedema, and a list of certified therapists can be found on the National Lymphedema Network Web site: http://www.lymphnet.org.
Some patients are able to effectively compensate for an increase in lymphatic loads by the regeneration of lymph vessels, utilizing alternative collateral circulation routes such as perforating precollectors, lymphovenous anastomoses, and increasing the lymph time volume of remaining collectors. Even if a patient has experienced a direct insult to the lymphatic system via surgery or some primary cause (eg, hypoplasia), the patient may not exhibit signs or symptoms of lymphedema if the lymphatic system has found a way to compensate for an increase in lymphatic loads.2,5,7
There are certain “initiating factors” that can trigger lymphedema, or place a further stress on the already impaired transport capacity. The change in cabin pressure during an airline flight coupled with inactivity may trigger the onset of lymphedema. The reduced cabin pressure may allow more fluid into the tissue spaces as a result of decreased hydrostatic pressure. Inactivity allows for venous pooling, which will eventually cause an increase in pressure at the blood capillary level, therefore increasing ultrafiltration and the lymphatic loads. For a more in-depth review of how airline travel can affect patients with lymphedema, please refer to the “Taking Flight” article in the July 7, 2003 (Vol. 14, No. 15), Advances for Physical Therapists and PT Assistants.2,21
Any fluctuation in weight gain and fluid volumes such as pregnancy, congestive heart failure, or obesity can add further stress to an impaired lymphatic system. Medications that cause sudden fluctuation in body fluids can also be detrimental.2
Active hyperemia that results from local or systemic application causes an increase in blood flow, which ultimately will increase the lymphatic load of water, and stress a compromised lymphatic system. Examples of active hyperemia include local hot pack, massage, vigorous exercise, or infection to the limb at increased risk; hot tubs/saunas, hot weather and high humidity; and sprains/strains.2–5
With a passive hyperemia, swelling may result from “some other problem,” for example, from chronic venous insufficiency (CVI) or hypoproteinemia (liver dysfunction). In CVI, the prolonged venous pooling affects venous return and eventually the blood capillary level. An increase in ultrafiltration will occur at the blood capillaries, and the lymphatic system will try to compensate with an increase in lymph time volume (as a safety factor). If the lymphatics are able to compensate, no edema will result.2
Hypoproteinemia results from a decreased colloid osmotic pressure (COP) of the plasma due to an increase in protein loss through the urine and the inability of the liver to produce adequate protein synthesis for the body’s demands. The lymphatic system will increase the lymph time volume but may become overwhelmed. Edema will initially begin as the result of a high-volume or dynamic insufficiency. This edema will be systemic in nature and begin at the scrotum or eyelids.2,5
Stages of Lymphedema
Lymphedema is a progressive condition—There is no cure for lymphedema. If not managed, lymphedema will progress.2
Stage 0 (Prestage)
Anyone who has had surgery directly affecting the lymphatic system, but does not present with swelling, is considered to be in a prestage. The best example of this is a woman who has undergone a mastectomy, but does not develop lymphedema. She is considered to be in a prestage, because the surgery (mastectomy with lymph node removal and possibly radiation to the axillary nodes) decreases the transport capacity of the lymphatic system, yet the TC remains high enough to take care of the normal amount of lymphatic loads produced.2,22
There are two “subcategories” in a prestage: lymph angiopathy and latency stage. A decline in the TC due to a congenital malformation or dysplasia (as in primary lymphedema) of the lymphatic system is known as a lymph angiopathy. Any patient who has undergone surgery directly involving the lymphatic system, for example, mastectomy with lymph node removal, hysterectomy, or trauma directly involving the lymphatic system, is considered to be in a latency stage. Again, when a patient is in a prestage, the TC remains high enough to take care of the normal amount of lymphatic load produced. The onset of lymphedema correlates to the ability of the lymphatic system to compensate for any added stress to the system, or the frequency of the occurrences that may cause a dynamic insufficiency in the limb at high risk to develop lymphedema.2
Stage I (Reversible Lymphedema)
In stage I, it is possible for the swelling that typically presents at the end of the day to recede overnight. The involved extremity is soft, and pitting is easily induced. With proper management in stage I, it is possible for the patient to expect complete reduction of the involved limb (when compared to the normal limb).2,7
Stage II (Spontaneously Irreversible Lymphedema)
Progression from stage I to stage II is primarily identified by an increase in fibrotic tissue. Pitting is more difficult to induce and the patient is at a higher risk for frequent infections due to the increased diffusion distance, that is, the increased size of the limb.2,7
Stage III (Lymphostatic Elephantiasis)
Without management, lymphedema will progress. In stage III, a further progression of skin changes occurs, for example, lymphostatic fibrosis, sclerosis, and papillomas (benign skin tumors).2 In most cases, an extreme increase in swelling develops in stage III, but this does not always occur2,7 (see Fig. 22-10).
FIGURE 22-10 Stages of lymphedema. Edema mainly located above the fascia. Increased interstitial protein concentration marked by dashes; fibrosis marked by squares.
Patients do not remain in a particular stage for a given amount of time. For example, a patient will not be in stage I for 2 months, then progress to stage II for 3 months before moving to stage III. Tissue changes, or the progression of fibrosis, remains the clinical trait that distinguishes the stages of lymphedema. Tissue changes commonly seen in the progression of lymphedema include proliferation of connective tissue cells, production of collagen fibers, an increase in fatty deposits, and fibrotic changes. These changes initially become evident at the distal end of the extremities, that is, the fingers and toes. A Stemmer sign is positive for lymphedema—the inability or difficulty in lifting the skin from the dorsum of the fingers or toes. The absence of a Stemmer sign does not exclude the presence of lymphedema. In some cases—for example, in primary lymphedema—the patient may be able to keep their shoes on, which provides compression and retards the progression of swelling.
DIAGNOSTIC TESTING
Diagnostic testing specifically for lymphedema is not routinely performed to determine the stage of lymphedema or the type of malformation present that may have caused the lymphedema. Imaging techniques are used only if there is question regarding the origin of the swelling, for example, tumor (benign or malignant), and are rarely used to determine a treatment plan or the response to a treatment. It should be noted that any diagnostic testing that involves the injection of a substance, that is a dye, or a radiographic tracer, could contribute to further destruction and compromise of the lymphatic system.2,5,7,11
•Indirect lymphangiography: A contrast medium is injected subepidermally into the dorsum of the hand or toes. Serial X-rays are taken 3 minutes after the first injection, then at 3- to 5-minute intervals. When the superficial lymphatics are visualized, the test is terminated.2,5,7,11
•Direct lymphography: An imaging medium is injected directly into the lymphatic vessels of the dorsum of the hand or foot. Patients who have had this test will often present with a perpendicular scar over the dorsum of their hand or foot. Again, serial X-rays are performed to visualize the collectors and nodes. This direct method is used to closely examine the pelvic and retroperitoneal regions; but today, less invasive measures are preferred: MRI, computed tomography (CT), and ultrasound for example.2,5,7,11
•Fluorescent microlymphography: A fluorescent microscope and video camera are used to visualize the superficial lymphatic vessels.
•Magnetic resonance imaging (MRI): This is a noninvasive method used to visualize the lymphatics. Patients are placed in a magnetic field in order to measure the “relaxation of hydrogen protons (eg, in water, fat, etc) as a function of their location.”11
EXAMINATION
As with any good evaluation, a thorough history and review of the systems are necessary for patients with lymphedema. Social history, to include favored activities and family/caregiver resources, are discussed in order to work toward discharge goals. Current job/work status assessment will determine the potential for return to work goals/criteria. Other criteria to include in the patient history are duration of swelling (years, months, or weeks); reason for swelling (testing procedures, vigorous activity, infection); other conditions present (arthritis, CVI, CHF); history of other treatments for the swelling and the outcome. It is important to identify if any diagnostic testing has been performed to identify the type of lymphedema or to rule out tumor progression or cancer reoccurrence. Integumentary review should include skin integrity, presence of scars, presence of wounds, and degree of pitting. Included in this category are evaluation for the presence of lymphatic cysts (outgrowth of lymph capillaries or collectors) and/or fistulas (abnormal connections between the skin and the superficial lymph vessels, ie, ruptured cysts), papillomas (benign skin tumors), and the presence of a Stemmer’s sign. A positive Stemmer’s sign results in the inability or difficulty lifting the skin folds at the base of the second finger or toe. However, a negative Stemmer’s sign does not exclude the presence of lymphedema.
Clinically, it is imperative to assess for the presence of adequate circulation and the appropriateness of applying compression. An ABI or ankle–brachial index is helpful to determine the presence of adequate arterial blood flow. This is a comparative value of a peripheral to a more central blood pressure that identifies arterial patency or sufficiency. A value of 1.0 signifies normal arterial flow, that is, safe for compression. A value between 0.5 and 0.8 identifies moderate arterial impairment, and a value below 0.5 identifies severe compromise of arterial blood flow. Compression is not recommended for ABI measurements in the moderate and severe ranges.2
Musculoskeletal and neuromuscular deficits may also accompany lymphedema due to the increased size and weight of the limb with chronic postural compensations to deal with the added size of the limb. Subjectively, patients will often report a feeling of heaviness, fatigue, fullness, and, initially, pain (mild aching or tightness). Benign lymphedema is not painful, but the patient may initially experience some pain from the stretching of the skin’s elastic fibers. A review of patient’s medications should also be included. The patient’s balance and the need for assistive devices are evaluated to consider the patient’s safety. Patient compliance and follow-through with the self-managementphase are imperative—Assessment of cognition level and mentation are also important.2,4
Anthropometric measurements of the involved and uninvolved limbs are necessary to have baseline comparative values. There is currently no standardization in the methods of obtaining anthropometric measurements. Several current clinically accepted methods include specified point circumferential measurements, volumetric measurements, and water displacement methods. Recent research has compared the calculation of limb volume measurements using the water displacement and circumferential measurements derived from a truncated cone formula. These results determined that either method could be used with confidence to calculate volumes of fluid, but the two methods were not interchangeable.23 Another study showed that the 6-cm frustum method for the arm and the frustum method for the hand “correlated strongly” with volume determined by water displacement, but that the two methods produced dissimilar results.24
There are limitations to all of the aforementioned methods. Water displacement methods are often time-consuming and messy; the patient may have difficulty lifting the involved limb to place it correctly in the water-holding vessel; the water-holding vessel may not be large enough to accommodate the involved limb; and finally, total limb volume measurements by circumferential measurements and water displacement methods require standardization using a measurement from the uninvolved limb. Bioelectric impedance analysis (BIA) has gained popularity for its ability to determine both intracellular and extracellular fluid content. “The reference value used in this index is that of the intracellular fluid within” the measured area. BIA can be used in calculating the volume with bilateral lymphedema. BIA appears to be more sensitive to slight increases in volume, and therefore may detect lymphedema earlier.25,26
There is no consensus in the literature on the degree of enlargement that identifies lymphedema. The most common figures are a 2- or 3-cm difference between four comparative points of the involved and uninvolved extremities: metacarpalphalangeal joints, the wrist (smallest point), 10 cm distal and 15 cm proximal to the lateral epicondyle.5,27,28 In some cases, a 2-cm difference can normally occur between the dominant and nondominant extremities.27 A 2-cm difference can be nonsignificant in larger, more muscular upper extremities, but very significant in smaller upper extremities29–32 (see Table 22-6).
TABLE 22-6 Classification for Lymphedema1
The entry-level clinician may experience difficulty in differentiating lymphedema from regular edema. Table 22-7 presents clinical findings that may be used to differentiate one from the other.
TABLE 22-7 Edema Versus Lymphedema
INTERVENTIONS
Therapeutic nihilism (ie, no treatment at all) for lymphedema is deplorable, although quite common. All too often, a woman is told that she “should be thankful to be alive” and that she must “learn to live with it.” The fact that the average clinician is ill prepared to both detect and recognize early signs of lymphedema must be remedied, as data suggest that the sooner the treatment is started, the smaller the amount of treatment required to prevent further progression, and the better the ultimate result.27
Moist Heat and Cryo Modalities
CLINICAL CORRELATE
Ice, heat, hydrotherapy (hot packs), saunas, contrasts baths, and paraffin are all contraindicated for the involved limb for lymphedema management. Basic and advanced physiology identifies that vasodilation occurs with any of these modalities. This vasodilation increases blood capillary pressure and, in turn, will increase the lymphatic load of water—overloading an alreadystressed or impaired lymphatic system. Any modality that causes vasodilation to the involved limb and/or ipsilateral trunk quadrant should be avoided.2–5
Ultrasound
Because of its thermal affects, ultrasound should only be used in lymphedema management at a setting that significantly minimizes the heating effect. Suggested parameters for ultrasound are 3 MHz (50% pulsed) at 0.1–0.3 W/cm2 for 5 to 8 minutes with a dynamic head movement. This setting minimizes heating effects on the tissues that will avoid increasing blood flow in the area—avoiding the increase in the lymphatic load of water. Pulsed ultrasound at a lower intensity will produce mechanical or nonthermal effects that will soften fibrotic tissues. Ultrasound may be used for its fibrinolitic effects on lymphostatic fibrosis, but not on radiation fibrosis.2,33
Electrotherapeutic Modalities
No electrotherapeutic modalities greater than 30 Hz should be used on the involved lymphedematous extremity or in the ipsilateral adjacent quadrant. Electrotherapeutic current greater than 30 Hz will create erythema under the pads, which will increase blood flow, increase BCP, and ultimately increase the lymphatic load of water.2,4,7
Medications
Frequently, patients are given diuretics to manage lymphedema without success. The diuretics will help decrease the size of the limb for 1 to 2 days and do an effective job of removing water from the interstitial spaces. However, diuretics fail to remove proteins from the interstitial spaces; frequently there is a rapid return of swelling. Often, the involved limb is larger than it was before the patient began taking the diuretics.2,4
Topical and oral benzopyrones are a group of drugs that include flavonoids and coumarins. Flavonoids occur frequently in nature, particularly in vegetables and fruits. Studies have shown that benzopyrones may improve chronic lymphedema by stimulating macrophage proteolysis to remove excess proteins in the interstitial spaces.34,35 Benzopyrones are a standard of care in Australian and some European lymphedema management programs. Reduction of limb volumes in patients who combine benzopyrones and other lymphedema management strategies is slow—at least 6 months.34–39 In the United States, benzopyrones are not used. High liver toxicity and morbidity are associated with the use of benzopyrones. Loprinzi et al.39 performed a study that treated 140 women with 200 mg of oral coumarin or placebo twice daily for 6 months, followed by another commonly used treatment for the following 6 months. They found that coumarin was tolerated well, but there was no difference in the volumes of the involved arms and hands after 12 months of treatment. The researchers also found serologic evidence of liver toxicity in 6% of the women.2,7,34,37,39
Surgery
The best summative statement about surgical attempts to repair the lymphatic system and surgical correction for lymphedema is from Goldsmith and De Los Santos: “The large number of operations devised for improving lymphatic drainage from a chronically lymphedematous limb indicates the lack of a surgical procedure which is consistently effective.”40 Various types of surgical repairs for the lymphatic system include lympholymphatic repair, lymphovenous repair, collector replacement, and the use of enteromesenteric bridge. To date, surgical attempts to increase the transport capacity (the maximum carrying capacity) of the lymphatic system have failed. Földi asserts, “The use of prosthetic material (eg, nylon threads) with hopes of reestablishing lymph flow simply disregards the fact that the propulsive force of the lymph flow is furnished by the pulsation of the lymphangions. A blood vessel may readily be replaced by a tube because the heart pumps the blood through it, but there is no force that could propel lymph through an artificial, valveless tube.”22 Transplanted areas of tissue from the deep lymphatic system to the superficial lymphatic system are not successful in removing lymph stasis because the deep system is usually also involved with lymphedema (recall that lymph flow is from superficial to deep).22
Debulking procedures open the skin, remove all of the superficial tissues, and then close the skin via incision or graft skin over the remaining tissue. Another option for debulking is to remove the skin and the superficial tissues, and then place skin grafts over the fascia. One can only imagine the grotesque appearance of the residual limb. These operations are not as common as they were in the late 1980s and early 1990s; however, they were considered successful when the swelling was removed. This procedure does not prevent the reaccumulation of lymph fluid and does nothing to repair or improve the function of a compromised lymphatic system.2
Patients may seek help following a failed attempt at liposuction to “cure” lymphedema or debulk an involved area. Liposuction removes the superficial fatty tissues and destroys any remaining intact lymph collectors. However, because the extra fatty deposits are removed, lymphedema can still reoccur, and lymphatic microcirculation will be significantly disturbed. The long-term effects of liposuction on patients with lymphedema are still an enigma.2,7,41–43
Brorson, from Sweden, supports the use of liposuction to correct a “physical and psychological handicap” for post–breast cancer patients. He advocates the use of liposuction combined with compression over the use of compression alone to reduce the size of the involved limb. Brorson notes that liposuction does not improve lymphatic system function but does increase “skin microcirculation.”44
In a second study, Brorson and Svensson performed liposuction combined with compression on 28 patients and compared them to 28 patients who only received compression to manage their lymphedema. One-year postoperation, the liposuction group had maintained their decreased arm size results. On the 1-year postoperative follow-up, the researchers recommended that six participants (who achieved full reduction) remove their garments for 1 week. “A marked increase in arm volume was observed, which was immediately remedied by reapplying the garments.”45
Clinically, liposuction is considered a major surgery for some patients. It can be costly and very invasive. In lymphedema, the elastic fibers of the skin are destroyed, necessitating the use of compression for lifelong management of lymphedema. However, there are less invasive options with minimal to no side effects to effectively manage lymphedema.
Massage
Massage has traditionally been used to treat edema, but is not recommended to manage lymphedema. As previously mentioned, lymphedema is a high-protein edema due to the accumulation of protein in the interstitial spaces. It is the sole responsibility of the lymphatic system to remove the proteins from the interstitium. If the lymphatic system is compromised, protein removal will not occur, and a high oncotic pressure will form in the interstitial space.2–5,46 Edema of the superficial tissues can become manifest from a variety of conditions or situations: prolonged sitting or standing, insufficient venous return (incompetent valves), pregnancy, heart failure, liver disturbances in renal function, fluid and electrolyte imbalances, inflammations, and infections. Edema results when the intact lymphatic system is overwhelmed or overloaded (dynamic insufficiency), which results in an accumulation of water in the tissues.2,5,46
Massage (Greek: massain) means, “to knead” and describes the forms of “classical” or “Swedish” massage.47 Massage triggers the release of histamines from mast cells, which produce an active hyperemia in the tissues. This ultimately causes an increase in blood capillary pressure and ultrafiltration. More water accumulates in the interstitial spaces, which overloads an impaired lymphatic system. Superficial lymphatics of the skin are located in the subepidermal layer and are extremely vulnerable to high pressures from massage or trauma.46 Research has shown that a 3- to 5-minute massage affects the endothelial lining of the initial lymphatics and causes artificial cracks that develop from injury to the lymphatic wall.48
Pneumatic Compression
The use of pumps for lymphedema management and decongestion continues to be a topic of frequent discussion. Compression pumps consist of a single sleeve or a multichambered sleeve that uses rubber tubing to connect to a pump that moves compressed air into the sleeve. Single sleeves fill uniformly, where multichambered sleeves fill sequentially from distal to proximal. Newer versions of compression pumps offer “on/off” cycles with variation. Pumps do an effective job at removing water from the interstitial spaces but do nothing to remove proteins. Proteins that remain in the tissues continue to attract fibroblasts and generate new connective tissue, which creates more scar tissue.2,5,7
Disadvantages of Compression Pump Therapy for Lymphedema Management2
•Remaining/intact functioning lymph collectors may be destroyed.
•Trunk quadrants previously not involved may fill with fluid.
•Pumps have no effect on fibrotic tissue and may worsen fibrotic areas.
•Pumps push fluid into the ipsilateral trunk quadrant, which may be congested.
•Pumps can cause genital swelling.
•Length of treatment time is long and questionable (minimum of 4 hours; some protocols suggest 8 hours).
•Patients are immobile during pump sessions.
Segers et al. investigated multichambered pumps to determine whether the pressure set on the dial was the actual pressure produced in the chamber “on the skin.”49 He found that even though the dial was set at 30, 60, 80, and 100 mmHg, respectively, the pressure applied to the skin in each chamber actually reached 54, 98, 121, and 141 mmHg.49
Boris et al.50 performed a retrospective analysis and found that 53 of 128 patients with lower-extremity lymphedema used a compression pump during their course of lymphedema management. It was not stated whether the pump was used during the initial decongestive stages of therapy, but it was noted that 23 of the 53 patients who used the pump developed genital edema, and only 2 of the 75 patients who did not receive pump therapy experienced genital edema. This study concluded that the “incidence of genital edema after pump therapy was unaffected by age, sex, grade, or duration of lymphedema; whether lymphedema was primary or secondary; whether a single or sequential pump was used; and by the pressure level applied or duration or hours per day of pump therapy. It was concluded that compressive pump therapy for lower limb lymphedema produces an unacceptably high incidence of genital edema.”50
Miranda et al.51 performed a prospective, blind study protocol with sequential intermittent pneumatic compression (SIPC), which they suggested was an accepted treatment method for lymphedema. The study evaluated 11 patients that underwent an isotope lymphography before SIPC and 48 hours following a 3-hour session of SIPC. Measuring the lower extremities at six designated points revealed that there was a significant reduction after SIPC below the knee, but not in the thigh. They concluded that “compression increased transport of lymph fluid (ie, water) without comparable transport of macromolecules (ie, protein). Alternatively, SIPC reduced lymphedema by decreasing blood capillary filtration (lymph formation) rather than by accelerating lymph return thereby restoring the balance in lymph kinetics responsible for edema in the first place.”51
Some patients who attempt to use pneumatic compression eventually realize the transitory or limited benefits and discontinue its use. A survey conducted by the Greater Boston Lymphedema Support Group in 1998 revealed that 48 (78%) of their 56 members discontinued use of the pump because (1) no further results were gained, (2) pain increased in adjacent areas to the involved limb, and (3) swelling of previously uninvolved areas began.52
At the 1993 International Congress of Lymphology, it was determined that if pumps were used at all, the adjacent trunk area and base of the involved limb should be cleared first. Specific manual techniques—manual lymph drainage—should be taught by certified instructors and specialized programs, which are listed at the end of this chapter.53
Manual Lymph Drainage with Complete Decongestive Therapy
As early as 400 and 500 BC, Hypocrates and Aristotle discussed vessels containing “white blood” or “white milky fluid.” These early findings were forgotten for nearly 2,000 years after the death of these early pioneers—Anatomical studies were considered sinful by the early Catholic Church. Nothing was documented on the lymphatic system again until the early 17th century by Caspare Asselli—an Italian anatomist—who primarily performed research on cadavers and animals. During a vivisection of a dog, Asselli discovered “cords” in the digestive system. He initially believed these cords to be nerves, but a “milky, creamlike substance escaped” from the cords when cut.2
In the 1800s, Dr. Alexander von Winiwarter, a German physician, successfully treated elephantiastic limbs with compression, elevation, and a “special massage.” Dr. von Winiwarter died in the 1890s, and his work was forgotten until Emil Vodder, PhD, MT, and his wife rediscovered the von Winiwarter techniques in the 1930s. Dr. Vodder and his wife ran a successful clinic on the French Riviera, where they treated patients with chronic colds and swellings of various origins. Treatment consisted of “intuitively manipulating” the patient’s swollen cervical lymph nodes in order to “boost their immune system.” The Vodders coined the term “manual lymph drainage” (MLD) to describe their techniques.3
In the early 1980s, Dr. Michael Földi noticed that many of his patients had swollen limbs. He heard about the success the Vodders had with their manual lymph drainage techniques and began to study the new method. Accepted treatment for elephantiastic limbs at that time was tight compression with elastic bands, which often caused sores, skin breakdown, and eventually amputation of the swollen limb. Dr. Vodder and Dr. Földi met to discuss Vodder’s new treatment but could not agree on the specific implications for the treatment. Vodder claimed his new therapy—MLD—could cure such things as hair loss and obesity. The more scientific-oriented Földi did not agree with these implications, but believed Vodder’s techniques did have a scientific basis that effectively treated the lymphatic system. Much of Dr. Földi’s work—along with that of his wife Dr. Ethel Földi—has produced significant research and laid the foundation for the advancement of MLD. Dr. Földi coined the term “complete decongestive therapy (CDT)” and realized that a combination of treatments should be employed to successfully treat lymphedema: MLD, skin care, compression, and exercise. Földi modified the manual lymph drainage techniques of Vodder to fit a more scientific profile, but to honor the work of Vodder, he refers to his techniques as “modified Vodder techniques.” The Vodder techniques and modified Vodder techniques are the more popular methods used in North America today.2
Other professionals have made significant contributions to the field of lymphedema management over the years. Drs. John Casley-Smith and Judith Casley-Smith have added research, trained lymphedema therapists, and treated patients for more than 40 years. Albert Leduc and his son Oliver Leduc have used isotopic lymphography to establish standards and “efficacy of manual techniques” and also provided instruction and patient care. Joachim E. Zuther founded the Department of Lymphology in Ulm, Germany, in 1990 and offered the first certification classes in MLD/CDT (Vodder/Földi technique) in the United States through the Academy of Lymphatic Studies, which he founded in 1994. A list of the larger schools that teach scientific-based techniques can be found in Table 22-8. Please refer to the references listed at the end of this chapter for other noted contributors.2,5
TABLE 22-8 Lymphedema Management Programs
CDT has been referred to by a variety of names from the different schools of lymphedema management that have developed over the years: complex physical therapy (Casley-Smith), combined decongestive therapy (Vodder), and complete decongestive physiotherapy or combined physiotherapy (Földi and Leduc). The 1998 American Cancer Society Workshop on Breast Cancer Treatment-Related Lymphedema included a review of the techniques from the major CDT methods (Vodder, Leduc, Casley-Smith, and Földi) and found that the “principles followed are the same for each school, the [MLD] techniques vary somewhat in terms of the degree of pressure, motion and the timing of strokes. Additionally, the Leduc technique uses low, intermittent pneumatic pressure [<40 mmHg] pumps and the Casley-Smith group uses benzopyrone medications.”5,27,54–57
Complete decongestive therapy (CDT) is a two-phased, noninvasive treatment approach, which recognizes that lymphedema also affects the ipsilateral body quadrant adjacent to the involved limb. The effects of lymphedema are most obvious in the involved limb. During phase I (treatment phase), the patient is given daily 45- to 75-minute treatments over a period of 2 to 6 weeks (depending on the severity, this time frame could be longer or shorter) that includes MLD, decongestive exercises, education regarding skin and nail care, and compression (using short-stretch bandages). In phase II (self-improvement phase), the patient works to maintain and improve the effects gained in the treatment phase by continuing the skin care, exercises, bandaging, and self-MLD techniques learned in phase I. MLD may be performed 1 to 2 times a week or on an “as-needed” basis. The self-improvement phase is lifelong—There is no cure for lymphedema. All treatment options to date are symptomatic in nature.2,5,7,27
Components of Complete Decongestive Therapy
Meticulous skin and nail care utilizes basic hygiene principles—The patient needs to bathe and keep the limb clean as well as moisturized. Sweat and sebum (an oily secretion produced by sebaceous glands) mix to form a protective layer on the skin called the acid mantle. The pH of the acid mantle ranges between 4 and 5.5 (pH of 7 is neutral; above 7 is alkaline). Normal skin is mildly acidic in order to protect it from the elements of nature (pollutants and wind) and to inhibit the growth of harmful bacteria and fungi. If the acid mantle loses its acidity, the skin becomes more prone to damage and infection. A mild, neutral soap with minimal or no fragrance should be used to clean the involved limb. Moisturizing cream or lotion with a low pH—such as Eucerin or Aquaphor (Beiersdorf, Inc)—should be used to maintain subtleness of the skin and the slightly acidic acid mantle. Because the diffusion distance of the involved limb is increased (localized swelling if the involved limb), this puts the lymphedema patient at a higher risk for infections and dryer skin.2,58
Fingernails and toenails should be monitored for nail infections and fungus. Patients with Lymphedema need to be reminded not to trim their nails too close on the involved limb. This will only serve as another possible avenue for infection due to the increased diffusion distance in the involved limb.2,7
MLD utilizes a special technique to manipulate and activate superficial lymphatic vessels, deep lymph nodes, and larger transport vessels; lympholymphatic anastomoses; perforating precollectors and lymph vasavasorum vessels (lymph vessels and nodes of larger veins). With correct pressure and timing, MLD stimulates lymph vessels to contract with greater frequency (increasing LTV or lymphangiomotoricity) and intensity. MLD techniques also reroute stagnant lymph fluid from a congested area into an adjacent body quadrant via anastomoses and then toward healthy lymphatics. The general principle of MLD is to begin centrally and then work distally toward the congested limb (ie, stimulate the cervical lymph nodes in the healthy quadrant, in order to create a suction effect on the plexus/collectors, then use modified techniques in the involved quadrant adjacent to the ipsilateral involved limb, then the involved limb). To be successful, it is essential to first decongest the involved trunk quadrant, before progressing to the involved extremity. MLD treatments should begin in the proximal body regions then progress distally.2,7,22,48,59–61
The elastic fibers of the skin are damaged in lymphedema patients. Therefore, compression using multilayered padding and short-stretch bandages is used to increase external tissue pressure to control ultrafiltration (retard swelling).62–64 Short-stretch bandages have a high working pressure and a low resting pressure. They provide resistance against working muscles but relax to avoid arterial compromise when the patient is at rest (at night). Padding and bandages are generally applied over the entire limb from distal to proximal (ie, from fingers to axilla). The padding is used to distribute pressure and create a uniform surface for the bandages to compress—to eliminate areas of increased pressure or “hot spots.” Consistent compression—during the day and night—can help the body absorb and break down lymphostatic fibrotic tissue. As a general rule, lymphedema patients require 24-hour compression2,7,22,48,59–61 (see Figs. 22-11 and 22-12).
FIGURE 22-11 Lymphedema bandaging of an upper extremity. (Reprinted with permission from Lohmann and Rauscher, Inc., Topeka, Kansas.)
FIGURE 22-12 Lymphedema bandaging of a lower extremity. (Reprinted with permission from Lohmann and Rauscher, Inc., Topeka, Kansas.)
It is recommended that patients perform decongestive exercises with compression in place. Appropriate exercises can be any simple active exercise that addresses each joint to maintain range of motion and activate the muscle and joint pumps. Decongestive exercises improve the flow of lymph to the venous angle; deep breathing improves transport of lymph via the thoracic duct (the thoracic duct goes through the diaphragm at the aortic hiatus), helps to maintain normal hydrostatic pressure, varies tissue pressure, and prevents reaccumulation of lymph fluid. Of course, exercise helps to increase the patient’s level of fitness, and improves self-perception, and increases their tolerance to daily activities. Patient constraints and ability levels govern exercise prescription.2,7
Physiologically, exercise increases active hyperemia, which in turn increases blood capillary pressure, ultrafiltration, and the lymphatic load of water. Under normal physiological conditions, an intact lymphatic system can handle this increase without difficulty. Vigorous exercise may trigger the onset of lymphedema or exacerbate current symptoms for patients with an impaired lymphatic system. How much exercise is too much? How can one patient return to playing golf and another patient play three rounds of nine holes and experience lymphedema? These questions remain unanswered. The lack of evidence-based research regarding specific exercise prescription for patients at a higher risk to develop lymphedema symptoms is due to the inability of clinicians to accurately predict which patients will develop lymphedema. Therefore, exercise prescription is based on the patient’s fitness level and tolerance to activity. Even a more physically fit individual should be progressed through graded exercise programs (beginning at a lower intensity level) that focus on smooth, concentric activities with light resistance. Prudent clinicians and therapists will educate their patients about risk factors and precautions in an attempt to prevent against the onset of lymphedema. These precautions may be too conservative for some, but clinicians should be in a “conservative or prevention” mode, which may prevent the onset of lymphedema. This is not to say that certain patients should not be progressed above a minimal to moderate fitness level. Some studies refute the need to adhere to precautions, and support this claim by referencing data on participants who have performed strenuous activities and have not developed lymphedema or an exacerbation of symptoms.70 This may be the case, but clinical prudence and precaution should take precedence until it can be determined who will develop lymphedema. Progression of exercise and appropriate exercise prescription should be issues determined by clinicians knowledgeable about the physiological aspects of exercise and the response of a lymphedematous limb to exercise.2,5,7,28,70
Phase I of treatment ends when measurements of the involved limb(s) plateau. In phase II, or the self-improvement phase, all components of phase I are continued. Patients adhere to the principles of skin and nail care. Once the limb size has plateaued, the patient can be measured for compression garments. (Juzo and Beiersdorf-Jobst are the largest manufacturers of lymphedema garments.) Garments are made of a special weave of fabric that prevent reaccumulation of fluid by increasing external tissue pressure to accommodate for destroyed elastic fibers of the involved limb(s). Compression should be maintained during the day with garments and at night with bandages and padding. Patients who have no lymphostatic fibrotic tissue may not be required to wear compression bandages at night.2,7
Compression garments are divided into compression classes (CC) I–IV. Generally, an uncomplicated arm should require a CC II, and a leg should require a CC III. Of course, there will be exceptions. An older individual with an arthritic hand or no assistance at home may not be able to donn a CC II for the arm or a CC III for the leg, and a lower class should be utilized to achieve some degree of compression2 (see Figs. 22-13 to 22-15).
FIGURE 22-13 Example of lower-extremity pantyhose with compression classes. (Reprinted with permission from Juzo USA, Cuyahoga Falls, Ohio.)
FIGURE 22-14 Example of upper extremity garment. (Reprinted with permission from Juzo USA, Cuyahoga Falls, Ohio.)
FIGURE 22-15 Example of hand garment. (Reprinted with permission from Juzo USA, Cuyahoga Falls, Ohio.)
The involved limb should be monitored for response to exercise. It is important for the patient to recognize the signs and symptoms of an exacerbation (increase in swelling) and adjust their activity level appropriately2,7 (see Table 22-9).
TABLE 22-9 Exercise Risk Classifications
The patient can be taught basic self-MLD strokes to promote contraction of the lymph angions. It is imperative the patient learn the correct depth of the strokes—a light skin stretch is used versus a kneading motion as in traditional massage. Performing self-MLD should not take the place of exercises or bandaging.2,7
Continuation of the home exercise program helps to maintain the benefits of MLD, stimulates contraction of the lymph collectors, maintains joint range of motion, and increases the patient’s tolerance to activity. Exercise for 10 to 15 minutes, 2 times a day, with a few simple exercises is more beneficial than no exercise.2
It is recommended that a certified lymphedema therapist perform complete decongestive therapy. For certification, a therapist must complete a minimum of 135 hours of training, to include anatomy and physiology of the lymphatic system, bandaging techniques, and the correct manual techniques and principles of treatment. Specialized training is required to properly apply the manual lymph drainage techniques and the short-stretch bandages. Adequate education for lymphedema management cannot be accomplished in 4 days, a weekend, or even 7 days. A prevalent amount of anecdotal evidence has shown CDT to be successful for more than 50 years. Evidence-based literature is slowly accumulating to support the efficacy of this new intervention for patients with lymphedema.
CASE STUDY
Patient/Client Diagnosis Classification
In order to include patients in Pattern 6H of the Guide, a thorough history and review of the systems are necessary to identify risk factors and pathological or pathophysiological impairment of the lymphatic system. Lymphatic system impairment can result from surgical interventions directly involving the lymphatic system, trauma, filariasis, infection of lymph collectors (lymphangitis) or lymph nodes (lymphadenitis), complex regional pain syndrome, postradiation, frequent bouts of cellulitis (particularly in an immune compromised limb), and trauma. Regardless of the precipitating incident, lymphedema results from an abnormal accumulation of protein-rich edema due to the inability of the lymphatic system to remove this protein-rich swelling. Patients with severe impairments or multiple complicating factors are not excluded from the pattern; however, the frequency of visits and duration of care may require modification.1,2,5,7
Not all swellings or edemas are classified as lymphedema. Examination findings may support exclusion from this pattern, or the physical therapist may determine the patient/client may be managed more appropriately through (1) classification into another pattern, (2) classification in both Pattern 6H and another pattern, and (3) educated and objective inclusion of selective portions of Pattern 6H to appropriately manage the condition.1
Edema resulting from congestive heart failure (CHF) may require classification into a different pattern.
A vital part of lymphedema management is compression, which limits ultrafiltration and increases reabsorption. One should consider the added volume return to the heart and the integrity of the heart wall with compression and CHF patients. Patients with lymphedema may have comorbidities, for example, CHF that accompany lymphatic insufficiencies. A physician who understands the principles and components of lymphedema management should medically clear these patients.1,2,7
The swelling from lipedema is the result of an abnormal accumulation of adipose tissue. Lipedema is more common in women and may be caused by a genetic or hormonal component (specifics are still not known). There is a decrease in the transport capacity of the lymphatic system because the collectors must “cork-screw” and wind their way through adipose tissue versus running directly toward their regional lymph nodes. Over time, with the further proliferation of adipose tissue and increase of the lymphatic loads, further stress is placed on the lymphatic system that further decreases transport capacity. Initially, a full lymphedema management program may not be appropriate for these patients with lipedema (depending on the severity, because lymphedema can develop over lipedema; ie, lipolymphedema). A patient with lipedema can be effectively managed with compression that supports the venous return and the lymphatic system.2,7
Description of the Case
Rebecca is a 36-year-old, obese (5 ft 7.5 in.; 350 lb), white female who reports a long-standing, progressive history of increased swelling of her bilateral lower extremities. The swelling has gradually increased over the years. Her attempts to resolve the swelling with rest and elevation have failed.
History of Present Illness/Past Medical History
The patient presents to therapy and reports that she has experienced swelling in both lower legs (below the knees) since the age of 13. She has wrapped her lower legs since that time with ace bandages (long-stretch bandages) in an attempt to prevent progression of the swelling. As a child, she was active and always enjoyed movement activities, but continued to experience a gradual increase in swelling of her lower extremities, which seemed to worsen without compression. Rebecca reports no history of CHF, diabetes, or deep vein thrombosis.
Rebecca states that over the last 3 to 4 years, she has experienced problems with chronic infections in her legs (left more than right). A small scratch from the family cat transformed into cellulitis for Rebecca 4 or 5 times over the last 2 years. She continues to wrap her lower legs but has “given up” with her thighs. A cat scratch on her left thigh 2 years ago resulted in her leg “weeping clear fluid for days.” She went to her primary care physician at that time for help and suggestions without success. The weeping eventually stopped after 5 days. Following that incident, Rebecca noticed that lobes began to develop on her thighs. Both thighs have lobes at the medial aspects (left 50 lb; right 30 lb), and she reports difficulty finding appropriately sized clothing. One year ago, she noticed a lump at her left medial thigh. Her physician at that time suggested that it may be a fibrotic lymph node, and should be removed (she did not report any unusual swelling or soreness). After the removal of the benign, fibrotic lymph node, the lobes and her thighs further increased in size. Rebecca also states she has had a nonhealing wound on the medial aspect of her left lower leg for more than 3 years. She was prescribed wound care treatments at a local wound care center—3 times a week for 4- to 6-week intervals for more than a year. Rebecca expressed frustration in regard to her wound care experience, in that the wound did not heal. Rebecca is excited about her referral to physical therapy for lymphedema management and willingly accepts and agrees with her plan of care.
Examination
Social History/Family/Caregiver Resources
Rebecca has a degree in accounting and plans to continue working while undergoing treatment. She performs mainly deskwork, but can move about during the day as needed, and feels her employer will work with her to allow time for physical therapy appointments. Rebecca lives with her sister’s family in an extended family situation. She is active with church activities and attends school- and church-sponsored activities with her niece and nephew. If she needs help with bandaging or donning garments, Rebecca feels her family will provide the necessary assistance.
Other Criteria
As previously mentioned, Rebecca has experienced swelling below the knee since age 13. The swelling has gradually progressed since that time. She experienced an increase in swelling following episodes of cellulitis and surgical removal of a benign, fibrotic lymph node on the left medial thigh.
General Health
Rebecca is obese and reports chronic back and hip pain from lifting her heavy lower extremities. A chronic leg wound is present on the left lower leg. Blood pressure is 138/85 mmHg, and she is not currently taking blood pressure medications. Rebecca has no history of diabetes. She is able to care for herself and is oriented to person, place, time, and situation. She does not smoke or drink.
Integumentary Review
A chronic venous wound on the medial aspect of the left lower leg has been present for 3 years. No odor or drainage is noted, with good granulation tissue in the wound bed. Prior treatment for this wound included wet-to-dry dressing at a local wound care facility for almost 1 year. Rebecca has no other skin infections in the large skin folds or nail fungus. Pitting is difficult to induce at the distal lower extremities. Lymphostatic fibrosis is present—The skin is hard/firm to touch. Hemosiderin staining is present on both lower extremities below the knees, indicating a venous involvement (CVI). The Stemmer’s sign is positive bilaterally.
Musculoskeletal/Neuromuscular Deficits
Rebecca has fair lower back range of motion, but is only able to achieve 70 degrees of left-hip active flexion and 20 degrees of left-hip abduction due to the weight of her left lower extremity and the ensuing pain with active range of motion. Strength measures 4+/5 for the upper extremities, 4+/5 for the right lower extremity, and 4/5 for the left lower extremity.
Pain
Rebecca reports a 5 for low back pain and an 8 for pain using the Visual Analog Scale. This pain subsides with rest and elevation of the lower extremities.
Medications
The only medication reported at this time is a diuretic for the swelling in Rebecca’s legs. She has taken this medication for the last 6 months but experienced an increase in the size of her leg.
Balance and the Need for Assistive Devices
Rebecca demonstrates good dynamic and static sitting and standing balance. She does not use an assistive device for gait but reports she is always careful when stepping into the tub, negotiating stairs, and so forth.
Current Conditions/Chief Complaints
Rebecca is anxious to begin her course of treatment of lymphedema management. She understands the involvement and compliance she will need to gain optimal benefits and feels certain she will have support from work and family members at home. Rebecca’s goals are to be able to decrease her back and hip pain, increase her mobility into and out of the tub/shower, fit into normal clothing without making alterations, and decrease the size of her legs.
Circulation
Capillary refill assessed at the nail beds of the toes is normal. Pedal pulses are normal. The ankle–brachial index measures one.
Anthropometric Measurements
This was documented using girth measurements every 4 cm to the groin (see Table 22-10).
TABLE 22-10 Rebecca’s Before and After Anthropometric Measurements
Evaluation Summary
On the basis of the objective data and patient history, Rebecca can be classified as a primary lymphedema (lymphedema precox), in stage II or early stage III. She presents with a venous wound at the medial aspect of her left leg, and pitting is difficult to induce below the knees. The Stemmer’s sign is positive at the skinfold of the second toe.
Treatment Summary
Because of the amount of lymphostatic fibrosis and the size of her lower extremities, Rebecca will require an extended treatment time. Two times a day would be preferred; however, insurance in the United States usually will not cover this (at least not in this case). In Europe, Rebecca would be able to receive treatment for her lymphedema in an inpatient setting, with at least two sessions of treatments a day by qualified manual lymph drainage therapists. Rebecca received MLD/CDT daily visits for 6 to 8 weeks. The continuous 6- to 8-week sessions continued for 5 months with 2- sometimes 3-week breaks between the sessions. Even during breaks from CDT, Rebecca continued to bandage herself independently and perform her home exercise program. A decrease in the volume of the left and right leg can be appreciated from the girth measurements every 4 cm up the leg beginning at the malleolus. When measurements plateaued, Rebecca was measured for a full pantyhose compression garment with closed toes. She was thankful for the garment, because this meant she would be able to wear the garment during the day, and only bandaged at night. Rebecca was required to bandage at night because of the amount of lymphostatic fibrosis present. She also required consistent compression to hold back the reaccumulation of fluid. With constant compression, the body is able to absorb scar tissue. Rebecca was not diagnosed with lymphedema when she was younger. This diagnosis would have been beneficial. She could have adhered to certain precautions for patients with lymphedema, and received education regarding the benefits of compression, skin care in combination with MLD. Tables 22-11 and 22-12 summarize Rebecca’s physical therapy management while on program.
TABLE 22-11 Summary of Rebecca’s Treatment Interventions
TABLE 22-12 Rebecca’s Home Exercise Program
Limits of Our Knowledge
The field of lymphedema management has significantly evolved in the United States over the last 10 years. Clinically, a major limitation is that the signs and symptoms of lymphedema are not managed early enough. Unfortunately, patients with lymphedema are told they have to live with the swelling or the swelling is a side effect of surgery and it should resolve.
There has been much anecdotal success with CDT. For some individuals, this is not satisfactory, and a resounding cry for more evidence-based research has evolved. More clinical research and evidence-based research are needed to support the efficacy of MDL/CDT and to prove that the inclusion of all components of CDT provides a more effective treatment than does just one component, which is, exercise or compression. Other areas worthy of investigation include patient well-being and financial issues.
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