ABOUT LYMPHEDEMA

Lymphedema:
Assessment And Management Options

by CATHERINE COTTON, B.Sc. ,P.T.

Lymphedema is a chronic condition that affects more than 120 million people world-wide, yet it remains poorly understood and is often untreated. The key to success in managing this lifelong condition is accurate assessment, a comprehensive treatment approach and ongoing education and support for patients and their caregivers.

With very few exceptions, almost all tissues of the body have lymphatic channels. The lymphatic system represents an accessory route by which fluids and proteins can flow from the interstitial spaces into the blood. Thirty litres of fluid pass from the blood capillaries in into the interstitial spaces each day; 27 litres of this fluid pass from the interstitial spaces into the blood capillaries. If the remaining three litres were to remain in the interstitial spaces, edema would result, which, in turn, could cause tissue damage and eventual death. It is the job of the lymphatic vessels to carry these three litres of fluid, wich also contain protein molecules and large particulate matter, away from the tissue spaces to be deposited back into the bloodstream.

The lymphatic vessels begin as fingerlike capillaries between cells in the interstitial tissue. These capillaries gradually join together into large collecting vessels, which eventually converge and approach the lymph nodes. After being filtered through the lymph nodes, the lymph eventually drains into the larger lymphatic trunks. Over two-thirds of the lymph from the body drains into the large thoracic duct (the main lymphatic vessel located in the abdominal cavity) and into the Left Subclavian Vein.

Many factors can alter the absorption of lymph from the tissues. Intrinsic factors like the random contraction of the smooth muscle wall of the lymph vessels and the stretch reflex of the vessels have a direct impact on the amount of fluid moving through this system. Extrinsic factors like muscular contractions, abdominal breathing, arterial pulsations and compression of tissues by objects outside the body, will also affect the pumping action of the lymphatic vessels.

Lymphedema defined
Lymphedema is defined as an abnormal accumulation of tissue proteins, edema and chronic inflammation within an extremity. It has several causes, but in all types, there is impaired transcapillary fluid exchange and impaired transport of lymph. Lymph drainage fails to keep up with production, resulting in an accumulation of a relatively proteinrich interstitial fluid.

Lymphedema can be categorized as either primary or secondary.

Primary lymphedema: Primary lymphedema is often referred to as idiopathic (of unknown origin) and has been the least understood and most often undiagnosed or misdiagnosed form of lymphedema.
The distribution of primary lymphedema among the sexes is reported to be 87 percent in women and 13 per cent in men. It can be diagnosed at birth (Nonne-Milroy syndrome), during puberty (Meige’s syndrome, lymphedema praecox) or after the age of 35 (Meige’s syndrome, lymphedema tarda).

Sometimes the onset is gradual, but it can be sudden and appear unexpectedly. In about 70 per cent of the cases reported, it affects only one leg, usually starting in the foot, ankle, and calf. However, it can affect both legs, including the thighs, as well as the trunk, genitals, arms and face.

It is clinically recognised by the thickening of skin folds, swelling of the dorsal aspect of the foot, retromalleolar swelling, and a positive Stemmer sign. (Stemmer sign is positive when there is difficulty in lifting the thickened cutaneous skin folds at the dorsum of toes.)

Primary lymphedema can develop progressively without adequate therapy, usually during attacks that are triggered by hot weather, pregnancy, traumatic events or local infections.
Primary lymphedema can be classified according to stages (see Table 1).

Secondary lymphedema: Secondary lymphedema is an acquired form of lymphedema. It is generally caused by obstruction or interruption of the lymphatic system due to infection, malignancy, traumatic injury or scar tissue, and has been noted to be a complication following surgery and radiation for carcinoma. This secondary type can also occur as a result of parasitic infection (filariasis), found mostly in endemic areas of Southeast, India and Africa.

Treatment rationale, goals and options
It is important to note that left untreated, the collection of stagnant, proteinrich fluid in the interstitial spaces causes tissue channels to increase in size and number, reduces the amount of oxygen moving through the transport system, interferes with wound healing, and provides a culture medium for bacteria that can result in infections, all of which aggravate the edema. This chronic inflammatory condition eventually results in fibrotic lymph tissue. The fibrosis further inhibits the ability of the lymphatic vessels to clear the excess fluid, and a cycle of further lymphedema and tissue fibrosis is perpetuated.

Lymphedema produces not only physical sequelae, such as swelling, pain, decreased motor function, parasthesias, and loss of mobility, but also psychological and social problems for the affected individual. The main goals of lymphedema treatment are to reduce edema in order to retain or restore function of the affected limb and to assist in the maintenance of healthy skin and overall cosmeses (appearance) of the limb.

Improved edema control is also a means to minimise the risk of chronic and recurrent acute infections of the affected limb. Initial treatment must be complemented by conscientious follow up care and patient education regarding the condition. In fact, education is the key to successful treatment of lymphedema.

Patients who are aware of the risk factors, who are able to recognise the early signs and symptoms of lymphedema, and who know the importance of early and aggressive intervention, will be much more likely to be able to adequately control their edema.

The treatment of lymphedema can be difficult, costly and time consuming. It is important that each patient understand that multiple modalities and an interdisciplinary approach are needed to provide the most beneficial results. Unfortunately, the quality of the data supporting the various treatment options in the management of lymphedema is inconsistent. Much information has been obtained by uncontrolled, nonrandomized trials or by anecdotal experience. Still, it behoves us to examine what treatment methods are available and what they have to offer our patients.

Skin care: Much can be done in the early stage of lymphedema to prevent skin problems. The skin must be kept supple, spotlessly clean, and in good general health. Patients are instructed to use a mild hypoallergenic soap daily and to carefully dry all body parts particularly between digits and body crevices. Care in cutting nails and maintaining healthy cuticles will help to avoid distal infection sites. Use of a moisturising lotion, one without fragrance, that has a low pH and contains alpha hydroxy, will help to prevent skin form becoming cracked and hardened. Skin integrity must be preserved, as the breakage of the skin in the affected quadrant could lead to the development of infection. Problem of bacterial infection should dealt with immediately when they occur, as they can worsen the lymphedema and can be life threatening. Early administration of systemic antibiotics and bed rest with elevation of the affected boyd part can reduce the effects of the infection.

Elevation: Elevation is among the first interventions generally recommended by physicians. It has been suggested that elevation of extremity reduces intravascular hydrostatic pressure, thereby decreasing those intravascular forces responsible in part for the production of lymph. However, there is no data on the efficacy of elevation in the treatment of lymphedema, and continuous elevation of a swollen limb does not promote functional use. Elevation has shown to be of limited use during the early onset of lymphedema; however, in later stages, elevation has little effect. Lymphedema is not only the presence of excess fluid in the tissues, but also the accumulation of proteins within the interstitial spaces. Protein movement is not affected by the elevation of the affected limb, and it increased concentration in the tissues will effectively increase the return flow of fluid into the interstitium.

Compression therapy: Compression is defined as the application of any external pressure to the limb. Its purpose is twofold: to reduce edema formation and to assist in the removal of excess lymph fluid already present in the limb. The two most common ways to achieve this are through sequential pneumatic compression devices and compression garments. (See Compression therapy for lymphedema Table 2)

Manual Lymph Drainage (MLD): Manual lymphatic therapy is a specialised form of gentle massage that has been demonstrated to stimulate and direct lymphatic flow, thereby decreasing the edema and fibrous changes of the involved extremity. The massage is based on the concept of emptying the truncal regions first to provide an empty reservoir for the peripheral edema; then the massage is extended distally. A major part of the rationale of the massage is to force lymph gently and slowly across the lymphatic watersheds (divisions between different lymphatic drainage areas), dilating the collateral vessels and stimulating alternative drainage patterns. The other function of the massage is to move tissue fluid into the lymphatics of the limb, removing excess protein from the tissues and preventing the formation of fibrotic tissue.

The characteristic light strokes (less than 30 mmHg pressure) used in lymphatic massage are always in the direction of desired lymph flow. This pressure is sufficient to cause the skin to stretch, but not enough to cause pain or redness. The hand rotates spirally into and out of the tissue with rising and filling pressure, similar to the action of the heart with its systole and diastole. The length of the treatment course depends on the needs of the individual patient, and will vary with the severity of the lymphedema. The typical time spent on massage on a consecutive daily basis can range from 40 to 90 minutes. Most initial treatment sessions last from 3-4 weeks, and patient follow-up is based on the overall deem reduction and tendency of the tissues to refill. Reduction of deem is much easier to achieve if treatment commenced as soon as the limb shows signs of swelling, when tissues are still soft, not unduly stretched, and excess fibrosis has not formed.

Bandaging of the affected limb with low-stretch bandages (combined with appropriate padding) follows each massage session. These bandages are applied with greater pressure distally, achieved by the number of layers and overlap of the bandages, and enhance the effect of muscular activity upon the clearance of lymphatic fluid from the limb.

Exercise: Exercise is an essential part of the treatment program for lymphedema. Data support the benefits of exercise in increasing the uptake of fluid by the initial lymphatics and enhance the pumping of the collecting lymphatics. The deep breathing pattern that accompanies exercise enhances the pumping in the thoracic duct; lymph flow improves with the reduction in the intrathoracic pressure associated with inspiration. Combinations of flexibility, aerobic training, and strengthening in combination with the use of compression bandaging or garment have produced significant benefits for patients with lymphedema. It is important that any exercise program be gradually progressed to avoid sprain and strain injuries. A slow progression will also allow the individual to monitor the affected or at risk limb for any sensation of aching or fullness that could indicate an overwhelming of the lymphatic system.

Conclusion
Ongoing studies continue to assess the efficacy of the various methods available to treat lymphedema. Based on current literature and practice, it is recommended that a comprehensive treatment regimen be used to provide functional, cosmetic and emotional support to individuals with lympedema. Unless there is widespread recognition and appropriate assessment of lymphedema by health care professionals, there will continue to be conflicting treatment information. Further research is needed to continue to critically evaluate current and innovative treatment methods to enable health care professionals to provide the most appropriate care for our patient population.·

By Catherine Cotton, BSc PT, CDT.
C. Cotton is Patient Care Director, Orthopaedics & Rehabilitation,
at the Scarborough Hospital, Scarborough, ON.


Treatment should provide functional, cosmetic
and emotional support.

Exercise is an essential part of the
treatment program for lymphedema.