Download Management of Extremity Lymphedema

Management of Extremity Lymphedema
Christopher A. Puleo, PA-C, and Marianne Luh, BS
The Cutaneous Oncology Program at H. Lee Moffitt Cancer Center & Research Institute,
Tampa, Fla. (C.A.P.) and Venus Medical, Inc., Palm Harbor, Fla. (M.L.)
Chronic lymphedema is almost always a permanent and often progressive condition. In most cases, neither medical nor surgical means can completely relieve the effects of
lymphedema. Surgical management of chronic lymphedema has high morbidity and a success rate of only 30%, and many patients return to their presurgical limb girth within
three to four years. Nonsurgical treatment of chronic lymphedema can decrease overall lymphatic edema. Sequential gradient compression systems, which compensate for
impaired lymphatic flow, return protein-rich lymphatic fluid from the extracellular regions of the tissues back into the circulatory system where the fluid can be excreted.
Introduction
Lymphedema occurs when an interruption in the lymphatic flow results in the accumulation of protein-rich fluid in soft tissues. While lymphedema occurs most frequently in
the extremities, it also can be found in the head, neck, abdomen, lungs, and around the genitalia. When the lymphatic system is damaged or blocked, edema accumulates
over a period of time and thickens the interstitial tissue with fibrosis.
Primary lymphedema, which is associated with developmental abnormalities of the lymphatic system, may be manifested in neonates (congenital), in adolescents (praecox),
or in patients over the age of 35 years (tarda). Secondary lymphedema, an acquired loss of lymphatic patency, is the more common form of lymphedema that occurs following
infections or in patients with cancer. Secondary lymphedema occurs when the lymphatics are damaged by metastatic disease or when the lymphatic basins at risk are
surgically removed and/or radiated in a prophylactic or therapeutic manner.[1] Following the onset of secondary lymphedema, the accumulation of lymphatic fluid in the
subcutaneous tissue spaces and skin leads to a cosmetically displeasing enlargement of the extremity and overall limb heaviness and fullness that can limit activities of daily
life. Recurrent infection in the extremity can lead to the deterioration of the lymphedematous limb over time. Recent reviews[2,3] of the intergroup study of elective lymph node
dissections for melanoma show that surgical removal of the regional lymph node basins at risk induces postoperative leg lymphedema in 21.4% of patients. The incidence for
arm lymphedema is 3%.
---------------------------------------------------------Table 1. Interventions to Avoid in Patients With Lymphedema
Venipuncture
Injections
Intravenous contrast injections
Acupuncture
Liposuction
Blood pressure tests
---------------------------------------------------------Pathophysiology
The lymphatic system develops embryologically as part of the vascular system. Lymphatic fluid comprises endothelial cells, protein, water, tissue products, and foreign
particles. The lymphatic system circulates interstitial proteins and lipids back into the vascular system through lymphatic capillaries. The capillaries are valveless intradermal
vessels that drain into unidirectional-valved lymphatic vessels located at the junction between the dermis and the subcutaneous tissue. These unidirectional vessels then drain
into afferent lymphatics. The lymph is propelled by muscular movement and contraction of the larger lymphatic vessels, and the flow is controlled by valves located at 1- to 2cm intervals. The lymphatic fluid is subsequently filtered by regional lymph nodes that eventually empty into larger vessels in the venous system, primarily at the thoracic
duct. Unlike the blood circulatory system, the lymphatic system is a regional drainage system. However, lymph vessels communicate between various regions.[4]
Diagnosis, Assessment Methods, and Symptoms
Diagnosis of lymphedema of the arm or leg is established on the basis of an accurate history and a thorough physical examination. Assessment of the progression of edema
in the postoperative care of the patient can be accomplished with photography, circumferential measurements, and water displacement measurements. Each method has
inherent shortcomings, but a patient's progression or response to therapy is monitored well when these are used together.
Photography[5] at preoperative and postoperative visits can be useful in determining the onset and progression of lymphedema. Photographs must be taken not only from the
same focal distance, but also at the same time of day since the extent of lymphedema is dependent on time and activity. Photographs taken in the morning generally show
less edema. Afternoon photographs give a more accurate assessment of the patient's status and level of disability. Circumferential measurements using reference points to
bony landmarks also are valuable in following a patient's response to therapy or the progression of lymphedema. Relatively small increases in the diameter of the leg can
reflect a large increase in the overall volume and weight of the limb. In severe cases when most bony landmarks are obscured, the inferior border of medial malleolus and the
superior border of the patella in the leg generally can be identified; in the arm, the ulnar styloid and the tip of the olecranon are the best landmarks. A third method of
assessment, called water displacement[5] or volumetric study, measures the volume of displaced fluid after the affected limb is placed in a tank of water. Although this is the
most accurate method of documenting changes in edema, it is time consuming, and its use is limited to facilities with the required equipment.
Common complaints of chronic lymphedema include a sense of increase in size or fullness in the extremity, a corresponding heaviness, and a decrease in the functional
ability of the limb. Patients with chronic disease may experience decreases in range of motion and function (usually in the arm) secondary to interstitial fibrosis. Pain and
achiness also may occur. Lymphangiosarcoma, a rare sequela after mastectomy of long-term lymphedema, may be suspected if a local tumor or purple or bluish
discolorations become apparent.[6]
Management
Prophylaxis
Initial interventions for lymphedema are aimed at prevention. Some therapeutic interventions may aggravate lymphedema and may compromise a patient's response to
treatment (Table 1). To avoid trauma and infection of the affected limb, any injections, blood pressure measurements, and administration of intravenous medications should be
applied to an unaffected limb.
Surgical Treatment
During the last century, a number of surgical treatment plans have been used to attempt to reconstruct the lymphatic channels.[7] These treatments ranged from burying silk
and other synthetic materials in the soft tissues to mimic lymphatic channels to the more recent practices of removing the subcutaneous fat and placing a dermal flap within
the muscle to encourage superficial to deep lymphatic anastomoses.[8] A retrospective review[8,9] of the available surgical techniques shows that 30% of patients undergoing
a surgical repair had good sustained results. The overall success rate was low, and many of the patients regressed to their pretreatment girth measurements within three to
four years following the original reduction surgery.
Two categories of patients might today be considered for surgical treatment. Patients who have massive lymphedema with overlying skin breakdown are candidates for the
Charles procedure. Skin and subcutaneous tissue is removed to the level of the underlying fascia, and the extremity is covered with split thickness skin grafts. Although the
cosmetic appearance of the limb is not favorable, this procedure can allow a patient who may have been immobile to return to a normal activity routine. Moderate length
hospitalization is required and wound-healing problems can occur in the skin-grafted areas, but the risk-benefit evaluation is favorable since these patients are homebound
and/or bedridden if untreated. The second category of patients for surgical treatment consists of those who have demonstrated no further progress from optimal conservative
therapy, yet remain with significant extremity enlargement but with good skin cover. This group may benefit from excision of skin and subcutaneous tissue in a staged
approach. The inner aspect of the extremity is addressed first. In a second stage, an excision is carried out on the lateral aspect This technique can be performed with a low
morbidity and short hospitalization. To maintain the improvement that is obtained surgically, long-term compression garments are necessary, as well as all aspects of optimal
care of the affected extremity. However, neither surgery nor conservative therapy will produce long-term results without lifelong diligent care.
---------------------------------------------------------Table 2. Care Guidelines for the Patient With Lymphedema
Keep skin clean and moisturized
Elevate limb while sleeping and traveling
Wash with hypoallergenic soaps and cleaners
Use electric razors (rather than straight razors) to remove hair
Use mild detergents for clothes
Maintain a constant temperature in the home
Eat a balanced, nutritious diet
Treat infections early and thoroughly with antibiotics
Exercise (walking, swimming, prescribed isometrics)
Wear prescribed garments and or bandages
Avoid cuts, burns, and insect bites
Avoid sunburns
Wear loose-fitting clothing
Avoid heavy, traumatic, or repetitive exercises
Avoid lifting heavy objects
---------------------------------------------------------Nonsurgical Treatment
Most patients with chronic secondary lymphedema are best managed by nonsurgical measures. Patient education on activity levels and infection prophylaxis are important
factors in long-term control. Physical therapy[10] and compression garments or sequential gradient compression-type pumps are recent additions to the therapeutic
armamentarium.
Since the accumulation of protein-rich fluid creates a culture that encourages bacterial growth, infection prophylaxis is important for those patients with chronic lymphedema
who are prone to repeated infections. Patient education relating to skin care (eg, avoidance of injury, care of open wounds, and proper nail care) is necessary. The risk of
cellulitis and infection to the arm or leg correlates with the severity of the lymphedema, and each subsequent episode of infection increases the risk of bacteremia and
systemic toxicity, thereby exacerbating the lymphedematous condition. Patients are instructed on early identification of infection. The use of antibiotics at the first sign of
infection may prevent a serious cellulitis.
Not all forms of exercise are beneficial to patients with extremity lymphedema, but those that increase circulation by incorporating use of the affected limb are recommended
(eg, swimming, biking, walking, isometric exercises, and active range of motion exercises). Activities that involve heavy lifting or repetitive motion cause pooling of the fluids
are avoided. Patients with leg lymphedema should avoid high-impact aerobics and jogging, while those with lymphedema of the dominant arm should avoid sports such as
tennis and racquetball. Use of a compression garment during exercise will help to decrease pooling of fluids.
The overall treatment plan, individualized based on the patient's specific needs and deficits, generally includes instruction by a physical therapist on exercises to regain or
maintain normal range of motion and strength, as well as education regarding limb elevation, skin care precautions, massage techniques, pumping (isometric) exercises, and
soft-tissue mobility. A physical therapist not only can provide education on the mechanism of lymphedema, signs and symptoms, physical therapy goals, and treatment
options, but also can train the patient to use compression garments and to monitor sequential gradient compression-type pumps (Table 2).[4,11]
Due to communications of the lymphatic vessels between various body regions, physical therapy can aid chronic lymphedema by shunting fluid out of the compromised limb.
The physical therapy, which can be performed in a hospital or at home, is offered in two phases. The first phase, which spans a period of four weeks, is divided into four
segments. The first focuses on improving and maintaining the normal skin integrity while decreasing the risks of infection. The second involves manual lymphatic drainage, a
daily treatment that is designed to remove excess lymphatic fluid and to open collateral lymphatics, thereby allowing unaffected regions to aid the compromised regions in
draining excess lymphatic fluid. The third consists of compression bandaging to maintain and increase compartment pressure and to prevent retrograde flow of lymphatic fluid,
and the fourth entails specialized physical therapy exercises followed by lymphatic massage. The trunk is massaged first to empty the lymphatics, followed by the areas
adjacent to the compromised extremity, the central portions of the limb, and finally the distal portion of the arm or leg. In theory, this massage therapy forces the excess
lymphatic fluids into watershed regions of the body and provides access to the unaffected lymphatic collateral circulation. The second phase of physical therapy consists of
fitting the patient with specially measured compression garments.
Evaluation has shown that application of the techniques in these two phases can reduce the size of the affected limb by up to 65%.[10] In addition to the expertise of skilled
personnel, a commitment by the patient to complete the time-consuming program is required in order to realize the benefits of therapy and to maintain the achieved
improvements.
Fig 1. Multicom 500 five-chamber peristaltic gradient sequensial pressures. Shown are maximum presures per chamber during peristaltic cycle with adjustment at 80 mmHg.
Drug Therapy -Few drugs for the treatment of chronic lymphedema are currently being studied.[5,12] Venalot is a benzopyrene that breaks down the larger protein molecules
and may facilitate absorption of proteins into the vascular system at the level of the capillaries. This drug currently is not available in the United States. Diuretics give minimal
benefit in the treatment of chronic lymphedema secondary to oncologic surgery or metastatic spread of the disease, and their beneficial effects are temporary. The use of
these drugs should be reserved for early treatment of primary lymphedema.
Compression Devices - Compression pumps are being used more often in the medical management of chronic lymphedema.[13-17] While clinical massage can be effective,
constraints created by the need for experienced personnel and the time needed to perform the technique limit the availability of this treatment on a regular basis. Researchers
have focused on duplicating the beneficial effects of massage by developing mechanical and/or air compression devices.[14,17-19] The older intermittent, single-chamber,
nonsegmental compression pumps provide even pressure throughout the treated extremity. These do not provide a direction for the transfer of fluid, thereby allowing some
backflow of the lymphatic fluid. This retrograde flow, therefore, may cause increase lymphatic fluid in the proximal tissues of the limb.
Newer devices provide sequential compression. Such machines force compressed air into a sleeve that fits over the affected limb. There are standard and gradient sequential
systems. The standard sequential compression system without calibrated gradient pressure is a multichamber pump that delivers the compression at the same pressure in
each garment section from distal to proximal tissues.[17] The peristaltic sequential gradient compression system more closely mimics normal extremity pressure changes.
The pressures delivered by the sequential gradient system differ by approximately 10 mmHg between each chamber. The higher pressures are delivered to the distal chamber,
with each chamber having approximately 10 mmHg less pressure than the preceding chamber (Fig 1).
For increased efficiency, the delivery of lymphedema therapy must be not only physiologically compatible with the lymph system, but also powerful enough to imitate the
rhythmic motion of the skeletal muscles in order to transfer the excess lymphatic fluid in a distal-to-proximal fashion. This system provides the best results when the patient
is recumbent (for treatment of a leg) or when the limb is elevated (for treatment of an arm), since these positions minimize the effects of gravity on venous flow.
Initial studies[13] with compression pumps employed a series of overlapping cell compartments that applied a sequential pattern of compression to the affected limb. This
recreated the physiologic "milking" action of the lymphatics within the extremity. The pressures used in this pump ranged to 110 mmHg in the arms and to 150 mmHg in the
legs. A controlled site trial[18] of this type of pump followed 24 patients for six months following treatment and demonstrated that even after one treatment with the device,
approximately half of the patients maintained most of their reduction over several months. The best results were seen in a patient who had access to a pump and used it on
an intermittent basis (three times per week) over the follow-up period of six months. An overall reduction in girth and heaviness was seen in each limb without cutaneous,
neurologic, or muscular complications.
Success requires treatment for a minimum of one hour each session, and lower pressures for longer periods of time are more effective than higher pressures for shorter
periods of time. Patients are instructed to wear their fitted compression garments and to elevate the limb whenever possible.
Fig 2. Sequential compression systems consist of an air compression pump, sequential pneumatic garments, and air hoses that connect the pump to the individual
compartments of the garment. This therapy can be self- administered at home.
The most advanced sequential compression systems consist of air compression pumps, sequential pneumatic garments, and air hoses that connect the pump to individual
compartments of the garment. The system provides continuous pneumatic compression to gently massage the arm or leg. Once connected, the garment will inflate
peristaltically up the limb: the first chamber inflates and holds, followed by the second chamber, and when the third chamber inflates, the first one deflates. This sequential
compression continues up the limb to provide maximum therapeutic pressures while relieving unnecessary pressure on tissues behind the wave of compression. This therapy
can be self-administered at home (Fig 2).
Contraindications to the use of gradient sequential compression devices include massive edema of the extremity secondary to congestive heart failure, concurrent neurologic
symptoms, ischemic vessel disease or severe arteriosclerosis, deformity of the limb, metastatic disease in the involved extremity, and skin changes (eg, dermatitis,
gangrene, recent skin grafts, and especially cellulitis and deep-vein thrombosis).[5]
Conclusions
The oncologist often is faced with the problematic management of chronic lymphedema of the arm or leg. Until recent years, this condition had been neglected due to poor
understanding of the causative and abnormal physiology behind the condition. Consequently, most patients were either undertreated or completely untreated. This resulted in
a lifelong struggle for many patients that eventually led to crippling and disabling consequences. In the past, patients were told that this condition was something they had to
live with.
Knowledge of the physiology and pathophysiology of lymphedema is helpful to understand the rationale of available prophylactic and therapeutic approaches. The degree of
edema is established prior to initiating short- or long-term care. Surgical intervention is useful for some patients, but the current standard of care is conservative medical
management aimed at minimizing existing edema while controlling the formation of new edema. Patient education includes instruction in exercises, elevation of the arm or
leg, and infection prophylaxis, as well as activities to avoid.[20]
Physicians, nurses, physical therapists, and occupational therapists all have active roles in the care of chronic lymphedema. External compression therapy using peristaltic
sequential gradient compression devices assist control. This treatment can be given intermittently at home. A realistic approach to long-term care, coupled with therapeutic
and emotional support, can maximize the quality of life of patients with chronic lymphedema.
References
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