Management of peripheral lymphedema

INTRODUCTION — 

Lymphedema, which is the accumulation of fluid and fibroadipose tissue due to disruption of lymphatic flow for a variety of reasons (table 1), is a chronic condition that can be managed but is generally not cured. Multimodal therapy for lymphedema consists of general measures for monitoring and self-care, along with varying levels of physiotherapy and limb compression [1]. These are applicable to all stages of lymphedema, with the choice of specific management based on the clinical severity of lymphedema. Modifications of these regimens may be needed to reduce the risk of complications associated with treatment.

Conservative therapies for managing lymphedema are reviewed. The clinical manifestations and diagnosis of lymphedema and surgical treatment of lymphedema are discussed separately. (See "Clinical features, diagnosis, and staging of peripheral lymphedema" and "Surgical treatment of primary and secondary lymphedema".)

APPROACH TO MANAGEMENT — 

Lymphedema can often be difficult to treat, particularly if progression to later stages has already occurred. Left untreated, lymphedema tends to progress gradually over time and inhibits the activities of daily living [2,3]. Lifelong care, in combination with psychosocial support, is necessary to achieve optimal outcomes.

Lymphedema treatment — The mainstay of lymphedema treatment is conservative, multimodal therapy (ie, complete decongestive therapy [CDT]), which aims to limit the progression of lymphedema, reduce limb volume, and improve patient comfort, physical functioning, and quality of life [1,4]. Treatment is generally in two phases, and the intensity of treatment is based on the severity of lymphedema. (See 'Phases of treatment' below and 'Intensity of treatment' below.)

Lifelong care, in combination with psychosocial support, is necessary to achieve optimal outcomes. While these treatments do not address the underlying cause of lymphedema, they can control swelling and prevent the development of long-term sequelae such as irreversible skin changes ("elephantiasis") [5].

Among patients with lymphedema following cancer surgery, a search for concomitant disorders should be pursued when there is massive lymphedema that is refractory to usual therapies or has an onset several years after the primary surgery without apparent trauma. In particular, the recurrence of breast cancer in the axillary area or the development of lymphangiosarcoma should be excluded by the use of computed tomography or magnetic resonance imaging. (See "Clinical features, diagnosis, and staging of peripheral lymphedema", section on 'Further evaluation'.)

Phases of treatment — CDT generally consists of a two-phase treatment program that can be used for both adults and children [1,4]. Success depends, in part, on the availability of physicians, nurses, and physical therapists who are trained in these techniques and patient compliance.

●Phase 1: Treatment – The first phase includes general measures such as meticulous skin and nail care to prevent infection, therapeutic exercise (see 'General measures' below), and a massage-like technique called manual lymphatic drainage (MLD) (see 'Manual lymphatic drainage' below), followed by limb compression (see 'Compression therapy' below). Patients receive daily therapy five days per week, with volume measurements weekly to see if the patient is improving or has plateaued. The usual duration of phase 1 is two to four weeks.

●Phase 2: Maintenance – The second phase is intended to conserve and optimize the benefit attained in the first phase. In addition to continuing general measures (see 'General measures' below), it consists of compression garments worn during waking hours and self-compression bandaging at night as necessary (see 'Compression therapy' below). Exercise and self-MLD are advised if possible (see 'Simple lymphatic drainage' below). Limb circumference and volume measurements should be monitored every six months or sooner if necessary.

The effects of CDT are not permanent. Phase 2 maintenance therapy is required to prevent recurrence. Self-care, daily use of compression garments, and daily exercise are all necessary for the successful long-term management of lymphedema. Clinical measurements of girth or estimates of lymphedema volume can be used to track changes relative to baseline during and after therapy [1]. The schedule for follow-up depends upon the severity of lymphedema and treatment regimen.

Effectiveness of complete decongestive therapy — For patients with lymphedema, CDT is the mainstay of treatment. CDT (also called complex decongestive therapy, complex decongestive physiotherapy, or decongestive lymphatic therapy) refers to an empirically derived, multimodal technique that is designed to reduce the degree of lymphedema and to maintain the health of the skin and supporting structures [1].

Data to support various components of CDT come from observational studies and a handful of small, randomized trials [6-11]. Overall, these support CDT as a multimodal therapy for reducing limb volume and symptoms compared with other therapies alone, such as physical therapy, exercise, or compression therapy [1,4,6,7,12,13]. The available data also support a more intensive regimen for patients with moderate and severe lymphedema (table 2). For less severe diseases, the data are overall limited and predominantly low quality [14-18].

●Upper extremity lymphedema — For upper extremity lymphedema, small trials and observational studies predominantly in patients with breast cancer report reductions in limb volume with improved pain, cosmesis, and limb function [6,19-22]. Reductions in limb volume range from 28 to 70 percent. An observational study evaluated the effects of CDT completed five times a week for three weeks in 50 patients [21]. Upper extremity volumes were significantly decreased at the end of therapies compared with baseline (volume: 2943 versus 3262 cm³; excess volume: 19.12 versus 31.36 percent), and quality of life and functional indicators were also significantly improved. Notably, upper extremity volume improvement was inversely related to the duration and stage of lymphedema. The importance of nighttime compression during the reduction and maintenance phases of CDT was illustrated in a study that randomly assigned 120 female participants to daytime only versus daytime and nighttime compression [23]. A significant improvement in upper extremity volume was reported with the addition of nighttime compression. Adherence to nighttime compression was 95 percent.

●Lower extremity lymphedema — Compared with upper extremity lymphedema (predominantly breast cancer-related), fewer controlled studies have been done evaluating the treatment of lower extremity lymphedema; however, observational studies also support the effectiveness of CDT for lower extremity lymphedema compared with physical exercise or compression therapy alone [17,24-26]. CDT achieves an average volume reduction between 31 and 73 percent in patients with lower extremity lymphedema [24,27-30].

Patient compliance with treatment is important for achieving and maintaining a successful outcome. Patient compliance can be difficult due to the time commitment, lifelong need to continue treatment, limited availability of certified lymphedema therapists, expense, insurance coverage issues, and patient discomfort due to the bulkiness of compression garments and use during high temperatures [25,27,31]. As such, lymphedema can progress despite conservative treatment measures. In one study, at least 90 percent of the lymphedema reduction was maintained in compliant patients at an average follow-up of nine months, while noncompliant patients lost approximately one-third of the initial benefit [24]. The patient must be made aware that the inability to control lymphedema may lead to repeated infections, progressive skin changes, and possibly soft tissue cancer (ie, angiosarcoma) [1]. (See "Clinical features, diagnosis, and staging of peripheral lymphedema", section on 'Other manifestations'.)

Intensity of treatment — All patients with peripheral lymphedema can be managed using CDT, the type and intensity of which vary depending upon the stage of disease (table 2). For patients with moderate or severe lymphedema, we agree with recommendations from the International Society of Lymphology (ISL) and others for an intensive approach to CDT [1,4,32]. Treatments are best administered in clinics with expertise in the treatment of lymphedema. A description of the components of CDT, contraindications, effectiveness, and techniques are discussed in the sections below. (See 'Complete decongestive therapy' below.)

Our approach based on the stage of lymphedema is as follows:

●At risk for postoperative lymphedema – For all patients who are at risk for lymphedema following surgery but without clinically apparent lymphedema (ISL stage 0) (table 2), in addition to general measures (see 'General measures' below), we suggest exercise to improve mobility.

CDT may be initiated pre-emptively in at-risk surgical patients to reduce the development of lymphedema [12,14,15,18,25,33]. The potential benefits vary depending on the nature of surgical treatment and any adjuvant therapies. For patients with breast cancer at risk for lymphedema, the approach to and efficacy of prophylactic strategies to prevent lymphedema are discussed elsewhere. (See "Screening for and prevention of breast cancer-related lymphedema".)

●Mild lymphedema – For all patients with mild lymphedema (ISL stage I) (table 2), in addition to general measures (see 'General measures' below), we suggest self-directed physiotherapy (See 'Simple lymphatic drainage' below.) and compression garments. The degree of compression should be guided by the patient's vascular status and their ability to tolerate compression. (See 'Compression garments' below.)

●Moderate and severe lymphedema – For all patients with moderate-to-severe lymphedema (ISL stage II) (table 2), in addition to general measures (see 'General measures' below), we suggest an intensive approach to CDT, rather than less intense therapy, for those without specific contraindications. For patients who do not achieve anticipated volume reductions, we suggest the addition of intermittent pneumatic compression. (See 'Complete decongestive therapy' below.)

Unproven and contraindicated treatments — Pharmacologic or laser treatments are not generally used. No drug has conclusively been shown to be beneficial, and some may induce toxicity. Diuretics should not be given solely for the treatment of lymphedema because the accumulated interstitial fluid cannot be easily mobilized into the vascular space.

●Anti-inflammatory agents – Preclinical reports have suggested that anti-inflammatory therapies targeting the T cell-mediated inflammatory response may help prevent lymphedema after lymphatic injury. In addition, these studies have shown promise in treating lymphedema once it has developed. One study demonstrated that topically applied tacrolimus, an inhibitor of T cell proliferation, significantly decreased the development of pathologic changes of lymphedema in a mouse model of the disease [34]. A small trial of 18 patients with unilateral breast cancer-related lymphedema showed improvements in arm swelling and lymphatic function as assessed using indocyanine green lymphangiography for those using topical tacrolimus for six months [35]. Additional research is needed to translate these findings from the bench to the bedside.

●Antithrombotic therapies – An early study reported that coumarin, which is a warfarin-like drug that may reduce high protein edema by stimulating proteolysis, was beneficial in patients with lymphedema [36]. However, a larger, carefully performed crossover study of 140 female participants found no difference between coumarin and placebo in reducing arm volume and relieving symptoms; coumarin was also associated with significant hepatotoxicity in 6 percent of patients [37]. A subsequent systematic review concluded that the poor quality of the trials prevented any conclusions from being made about the effectiveness of benzopyrones (including coumarin) in reducing limb volume, pain, or discomfort [38]. A later trial randomized 50 patients to a combination agent (coumarin/diosmin/arbutin) in addition to CDT or CDT alone [39]. Extremity volume and percent reductions in extremity volume were reduced with the addition of the combination agent.

●Low-level laser therapy — Low-level laser therapy (also known as cold laser therapy) is a photochemical treatment used for soft tissue injury, chronic pain, and wound healing that has been used alone or in combination with other therapies for the treatment of peripheral lymphedema [40-42]. Among the hypotheses to explain the possible benefits of laser therapy are the stimulation of macrophages and the immune system, a potential decrease in fibrosis, and a suggested role in encouraging lymphangiogenesis, which may stimulate available lymphatic pathways and encourage the formation of new pathways [41]. Some small randomized trials have shown a reduction in limb volume [40,43,44]; others have shown minimal or no reduction [45-47]. In a systematic review evaluating low-level laser therapy for breast cancer-related lymphedema, outcomes were not significantly improved compared with established lymphedema interventions [42].

●Diuretics – Diuretics are of little benefit in the management of chronic lymphedema and may promote the development of volume depletion. They should not be given solely for the treatment of lymphedema. When diuretics are given to treat the usual forms of peripheral edema, the initial fluid loss comes from the intravascular space. The ensuing reduction in venous, and thus intracapillary, pressure allows the edema fluid to be mobilized and the plasma volume to be maintained. However, this sequence does not occur with lymphatic obstruction.

COMPLETE DECONGESTIVE THERAPY — 

Complete decongestive therapy (CDT) is the standard approach for the initial treatment of lymphedema and combines general measures for self-care such as monitoring, meticulous skin and nail care, and therapeutic exercise with lymphatic drainage and limb compression using either multilayered padding materials and short-stretch bandages, or compression garments. The intensity of treatment is escalated for increasing severity of disease. (See 'Intensity of treatment' above.)

The effectiveness of CDT is discussed above. (See 'Effectiveness of complete decongestive therapy' above.)

Contraindications — While CDT is beneficial for treating lymphedema, the application may be associated with risks (eg, skin breakdown, fluid overload) [48]. Experts have described several possible contraindications and/or precautions to CDT, and in particular, to manual lymphatic drainage (MLD) [32,49]. Although commonly followed, these contraindications are predominantly based upon theoretical concerns, and there are few clinical data to support them. As an example, the International Society of Lymphology (ISL) consensus statement included active neoplasia in the affected limb as a contraindication, but the concept that massage promotes metastases is debated [1]. A retrospective review reported that patients with locoregional disease experienced similar benefits from CDT as those without locoregional disease, and no study has ever demonstrated that massage therapy spreads cancer [50]. (See 'Palliative care modifications' below.)

The following conditions have been listed as possible contraindications to therapy. Decisions to pursue CDT or MLD in such patients should be made with the guidance of a trained lymphologist and clinician.

●Active cellulitis, neoplasm, or other inflammation (CDT may promote the spread of infection or exacerbate symptoms).

●Moderate-to-severe heart failure (may be exacerbated by the increase in central venous volume induced by mobilization of the lymphatic fluid).

●Acute deep vein thrombosis (To avoid potential embolism that may result from dislodging of a clot).

Relative contraindications, such that patients may be treated but may warrant additional monitoring or modifications, include:

●Uncontrolled hypertension (which may be exacerbated by the increase in central venous blood volume caused by MLD and compression bandaging). In this case, the patient's cardiac functions are monitored during the treatment phase.

●Diabetes mellitus (since associated vasculopathy or neuropathy may decrease the sensing of pain with improperly fitting compression garments, possibly leading to tissue injury and infection).

●Asthma (since parasympathetic activation can occur, possibly promoting an asthma attack). With these patients, the MLD treatment time should begin at approximately 20 minutes, and if no negative reactions are noted, the treatment time should be increased by 5 to 10 minutes until normal treatment times are reached [49].

●Limb paralysis (since a flaccid limb may offer insufficient resistance when compression bandages and garments are used, and any decrease in sensation may promote injury from improperly fitting compression garments).

General measures — General measures for self-care are appropriate for all patients with lymphedema and are aimed at minimizing the degree of edema and slowing the rate of progression. Although there is no scientific evidence supporting the efficacy of any of these measures, we agree with the guidelines from the ISL that suggest self-monitoring, limb elevation, maintenance of ideal body weight, avoidance of infection/injury, and avoidance of constricting garments/extremity cuffs for all patients with lymphedema regardless of severity, and for those at risk for lymphedema following surgery [1].

Self-monitoring — Patients should be taught to monitor their lymphedema. They should be counseled to promptly report changes in symptoms (eg, swelling, heaviness, pain, increased tightness of clothes that used to fit), pitting edema (if it is present), or skin condition.

Limb elevation — Simple elevation of a lymphedematous limb may reduce swelling, particularly in the early stage of lymphedema [1]. However, elevation alone is not an effective long-term therapy [51]. Patients should avoid positioning the limb in a gravity-dependent position for prolonged time periods; for lower extremity lymphedema, this includes prolonged standing, sitting, or crossing legs.

Diet and exercise — Maintaining an ideal body weight should be encouraged. Obesity may limit the effectiveness of compression garments (sleeves, stockings) and pneumatic compression and is also a contributory factor to the development of lymphedema [52,53].

Exercise and weight training are generally safe and should be allowed. Following axillary or groin lymph node dissection, exercise is safe, and exercises are generally recommended to restore the full range of motion in the affected extremity [54-67]. The National Lymphedema Network position statement for exercise recommends that individuals with lymphedema wear a properly fitted compression garment during exercise, including aerobic and resistive training, while individuals at risk for developing lymphedema may consider using a compression garment [68]. (See 'Compression garments' below.)

A large landmark trial, the Physical Activity and Lymphedema (PAL) Trial, determined the safety and efficacy of resistance exercise in those with lymphedema following breast cancer treatment [55,56]. This trial compared twice-weekly progressive weightlifting to no lifting over a one-year period (supervised for the first 13 weeks) in 141 breast cancer survivors with stable lymphedema of the arm. Weightlifting reduced the number and severity of arm and hand symptoms, increased muscular strength, and significantly reduced the incidence of lymphedema exacerbations (14 versus 29 percent). In a later systematic review, a significant reduction of lymphedema was documented by bioimpedance spectroscopy values in response to resistance exercise [69].

For patients with lower extremity lymphedema, a small study of patients with lower extremity lymphedema following cancer surgery reported a similar difference in limb volume before and after lower extremity weight training (ie, volume not exacerbated) [54]. Weight training did not affect limb volume at five months, but bench press and leg press strength increased, and there were improvements in walking distance; however, no improvement was seen in quality of life.

Avoid skin infection/injury — Patients are advised to maintain good hygiene and to keep the affected limb properly moisturized [1,4]. Patients with lymphedema have an accumulation of protein-rich fluid in the interstitial space, which can trigger inflammation, causing skin changes in patients with advanced stages of lymphedema. These changes include dry skin and decreased elasticity, making a patient more susceptible to infection and ulceration [31].

Patients should:

●Maintain meticulous skin hygiene and nail care to prevent a portal of entry for infection that may result in cellulitis.

●Use skin moisturizers and topical antibiotic solutions after small breaks in the skin as may be induced by a paper cut or abrasions, pinpricks, insect bites, or pet scratches.

●Protect exposed skin using sunscreen and wear gloves when participating in activities that could lead to skin injury.

●Protect their hands or feet depending on the location of lymphedema. Patients with upper extremity lymphedema should wear gloves while doing activities that may cause skin injury if the upper extremity is affected. Patients with lower extremity edema should always wear shoes.

●Avoid medical procedures that puncture the skin in the affected limb that might introduce infection, such as vaccination, acupuncture, phlebotomy, intravenous lines, and venography. For patients with unilateral upper extremity lymphedema, these can be performed in the contralateral arm [70].

●Avoid exposure to temperature extremes, which may increase the risk of injury to the tissue in the lymphedematous limb. In addition, lymphedema may be exacerbated in patients who use saunas, steam baths, or hot tubs.

Patients should seek medical attention if they observe any abnormalities on their affected limb or suspect that an infection may be present. All episodes of cellulitis should be treated with antibiotics that have adequate coverage for gram-positive cocci. Severe cellulitis, lymphangitis, and bacteremia require intravenous antibiotics. If patients experience three or more episodes of cellulitis in a year, an extended period of oral antibiotic therapy may be necessary. (See "Acute cellulitis and erysipelas in adults: Treatment".)

Avoid limb constriction — Recommendations to avoid limb constriction (eg, tight-fitting clothing, blood pressure cuffs) are pervasive but are based on limited scientific evidence [71]. Those who support limb constriction as a risk factor for developing lymphedema hypothesize that constriction (no matter how brief) can increase pressure in the limb, increasing lymph production and potentially leading to stenosis and fibrosis of the lymphatic vessels. On the other hand, others have challenged recommendations to avoid limb constriction, observing that limb compression is commonly used in the management of lymphedema.

For patients with lymphedema or significant risk factors for its development (eg, axillary node dissection), we support a recommendation to measure blood pressure in the contralateral arm, particularly in any setting in which blood pressure is being closely monitored (eg, in an intensive care unit, recovery room, or during procedures). This recommendation is based upon several factors. Blood pressure measurement in an enlarged extremity will be inaccurate if the cuff is not properly sized and the cuff causes a high-pressure focal compression (in contrast to pneumatic compression devices used for lymphedema treatment, for which the compression is applied sequentially) [72,73]. Among the rare patients who have undergone bilateral axillary lymph node dissection, routine blood pressure measurements can be obtained in the lower extremity. If the patient has had bilateral axillary lymph node dissection and there is no option of obtaining blood pressure measurements in the lower extremity, then a manual (but not automatic) blood pressure cuff can be used intermittently but inflated to only just above the expected level of systolic blood pressure [72]. (See "Hypertension in adults: Blood pressure measurement and diagnosis", section on 'Other methods'.)

Patients who do not have lymphedema or who have undergone sentinel lymph node biopsy rather than a full axillary lymph node dissection may have blood pressure measurements taken from either extremity. One study prospectively investigated the association between factors thought to increase the risk for lymphedema in 632 patients treated for breast cancer and screened routinely for lymphedema [74]. On multivariate analysis, there was no significant association between increases in arm volume and blood pressure readings. Significant factors included body mass index ≥25, axillary lymph node dissection, regional lymph node irradiation, and cellulitis. There was no association between blood pressure readings on the ipsilateral arm and cellulitis.

Compression therapy — Compression therapy, often combined with MLD, is used in the initial stages of lymphedema management. External compression is used with the aim of decreasing interstitial fluid production and reducing excess lymph fluid in the affected extremity. Different methods of compression include multilayer short-stretch compression bandages, compression garments, and pneumatic compression devices.

Compression therapy (bandaging, garments) is effective for reducing limb lymphedema volume; however, the benefits of compression therapy largely depend on patient compliance. Reported percentage reductions with compression garments or compression bandaging among various etiologies of lymphedema range from 17 to 60 percent [75,76]. For symptoms such as pain and heaviness, 60 to 80 percent of participants report feeling better regardless of which treatment they received [77-82]. Initial limb compression uses multilayered padding materials and short-stretch (also called low-stretch) bandages. Maintenance therapy uses custom compression garments worn during waking hours. It is important to note that compression bandages or garments may lead to the onset or progression of lymphedema if they are not properly applied or fitted.

Compression therapy is also an important component of management before and after surgery. Compression therapy is used preoperatively to maximize limb volume reduction and improve surgical outcomes. Many patients are also required to continue compression therapy following surgical intervention to maintain long-term improvements. (See "Surgical treatment of primary and secondary lymphedema", section on 'Postoperative management'.)

Compression bandaging — External compression is achieved with multilayered padding materials and short-stretch (also called low-stretch) bandages that are applied repeatedly [32,83-85]. For moderate-to-severe lymphedema, compression bandaging is applied to the affected limb after MLD and worn around the clock during the treatment phase (phase 1) of CDT [32]. (See 'Phases of treatment' above.)

Short-stretch bandages apply pressure during movement, but not at rest [32]. The pressure induced by muscle contraction within the bandage appears to reduce lymphedema via mechanical stimulation of the smooth muscle of the lymphatic vessels, resulting in increased lymph flow. Multilayered bandages are more effective than compression therapy alone for initial volume reduction. A minimum of two layers of bandages is most effective. A trial randomly assigned 90 patients with unilateral lymphedema to multilayered short-stretch bandaging for 18 days, followed by elastic hosiery for 24 weeks or elastic hosiery alone for 24 weeks [83]. Combined therapy was approximately twice as effective in reducing limb volume (31 versus 16 percent at 24 weeks). Multilayered bandages may also soften the edematous tissue, thereby increasing the efficacy of MLD. Compression bandaging also provides the greatest volume reduction in the early stages of lower limb lymphedema [31].

A possible alternative to standard compression bandaging is Kinesio tape (also called K-tape), which is made of a highly elastic woven fabric that stretches only along its longitudinal axis and has been used for rehabilitation after sports injuries [86-88]. A randomized trial of 41 patients with breast cancer and unilateral lymphedema of the arm reported similar efficacy for K-tape and short-stretch bandages but better patient acceptance of K-tape, manifested by less difficulty in usage and increased comfort and convenience [87].

Compression garments — Once swelling has reached its nadir, customized compression garments (lymphedema compression sleeve and gauntlet or stocking) are used in the maintenance phase of lymphedema treatment (phase 2 of CDT) to effectively prevent fluid reaccumulation [89,90]. (See 'Phases of treatment' above.)

The use of compression garments during exercise is discussed above. (See 'Diet and exercise' above.)

Fitted elastic knit two-way low-stretch compression garments generate greater pressures distally than proximally, thereby promoting mobilization of the edema fluid [32,91]. Compression garments deliver 20 to 50+ mmHg of pressure [1,32]. The highest level of compression tolerated by the patient is likely to be the most beneficial [1]. For the upper extremity, a compression handpiece, either a glove or a gauntlet, is necessary when wearing a compression sleeve to manage swelling in the hand. Similarly, in the lower extremity, the foot is an integral part of compression hosiery.

A prescription is necessary to obtain compression garments, and these garments need to be provided by a fitter with appropriate expertise. When correctly fitted and worn properly, compression garments can help reduce limb swelling [92]; however, poorly fitted garments can be restrictive and can exacerbate lymphedema. If off-the-shelf garments do not provide a proper fit, custom-made garments will be necessary. However, as fluid shifts occur, even custom-made garments may no longer fit. Garments should be replaced every three to six months, or sooner if they lose elasticity.

Compression garments are typically worn during waking hours, with compression bandaging at night, if necessary [32]. Nighttime compression garments consist of padding materials with compression applied over the padding with short-stretch bandages, elastic sleeves, or Velcro bandages.

Whether the use of compression sleeves during air flight for upper extremity lymphedema is of benefit is debated. In theory, lymphedema may be exacerbated at high altitudes or during air travel since the ambient atmosphere pressure is less than the relative outlet transcapillary pressure within the superficial tissues; however, studies suggest that the risk of precipitating or worsening lymphedema during air travel is very low [32,93-96]. For longer durations, air travel, compression garments, exercises, and self-massage may all be helpful.

Intermittent pneumatic compression — Intermittent pneumatic compression (IPC; also called sequential pneumatic compression) is another method of compression therapy that plays an important role in the movement of lymph fluid and is typically used for those with more severe lymphedema [97-101]. IPC is widely used in the treatment of lower extremity lymphedema.

IPC devices use a plastic sleeve or stocking that is intermittently inflated over the affected limb. Most pneumatic compression pumps sequentially inflate a series of chambers in a distal-to-proximal direction. Some pumps permit adjustment of the amount of pressure in a particular chamber; however, for the management of patients with lymphedema, the ideal pressure for the pump is not known. Some investigators have suggested that a pressure greater than 60 mmHg may injure lymphatic vessels. After using IPC, patients wear a low-stretch elastic sleeve or stocking to maintain edema reduction [1].

IPC is usually applied daily, five times per week; however, the optimal duration of IPC is also unknown. Among the various studies, sessions have varied in length (90 minutes to as long as six hours) and duration (two to three days to four weeks) [98,102-104].

IPC may be most effective as an adjunct to a multimodality lymphedema treatment. Clinical experience has shown that IPC may be an alternative maintenance program for lymphedema patients who have difficulty in performing self-MLD due to weakness, fatigue, or range of motion deficits. It may also be beneficial for lymphedema patients who are unable to use compression bandaging or garments due to mobility limitations or allergies to the materials used in these compression products.

A number of studies have evaluated the role of IPC in patients with lymphedema following breast cancer treatment [2,8,44,81,103-108]. In a systematic review of IPC [109], among three trials that reported volume reductions [8,104,106], there were no significant differences between routine management of lymphedema with and without the use of IPC. In contrast, other evidence suggests IPC may still be an effective adjunct. One of these trials randomly assigned 23 patients with unilateral previously untreated lymphedema to CDT alone or with CDT with adjunctive IPC (30 minutes daily for 10 days) [104]. A significantly greater reduction in limb volume during initial treatment was seen for combined therapy compared with CDT alone (45 versus 26 percent). A second arm of the trial evaluated the efficacy of maintenance IPC therapy (self-administered 60 minutes daily) added to CDT in 27 patients with unilateral breast cancer-associated lymphedema who had previously been treated with CDT [104]. At 6 to 12 months, the fall in mean limb volume occurred with combined therapy compared with an increase with CDT alone (-90 versus +33 mL), a difference that was statistically significant.

Physiotherapy

Simple lymphatic drainage — Simple (self) MLD is a commonly taught self-help maneuver for those with preclinical or mild lymphedema [110,111]. Lymphatic drainage may also be promoted by gentle therapeutic exercise of the affected limb. When possible, patients and caregivers should be instructed on self-MLD techniques [1,32].

Manual lymphatic drainage — The treatment or intensive phase of CDT typically includes MLD. MLD, which is a massage-like technique that is performed by specially trained physical therapists, is an important component for achieving successful results with CDT [1,16,32]. MLD enhances the filling of the cutaneous lymphatics and improves the dilation and contraction of the lymphatic vessels [90]. It appears to offer an additional benefit to compression therapy for reducing swelling, particularly for those with mild-to-moderate lymphedema [78,112]. However, MLD alone is not recommended for attempting to achieve volume reduction [25]. (See 'Lymphedema treatment' above.)

There are some concerns that MLD may dislodge and promote the spread of tumor cells among those with secondary lymphedema related to cancer [1,50]. These concerns and other contraindications to MLD as a component of CDT are discussed above. (See 'Contraindications' above.)

Light pressure is used to mobilize edema fluid from distal to proximal areas to enhance the filling of the cutaneous lymph vessels, promote dilation and contractility of lymphatic conduits, and recruit watershed pathways for lymph flow. Upon completion of MLD, compression bandaging/garments are applied immediately following MLD after the completion of a series of exercises to increase lymphatic flow [31]. The patients should continue compression therapy during waking hours and, as necessary, perform self-compression bandaging of the limb at night. (See 'Compression therapy' above.)

Palliative care modifications — The goals of palliative treatment of patients with cancer-related lymphedema include comfort, support, relief of symptoms, maintenance of function, and integration of caregivers into patient care [113]. Goals of care and treatment plans must be flexible, conforming to the ever-changing needs of the patient in the palliative setting. (See "Overview of comprehensive patient assessment in palliative care".)

While CDT is beneficial for extremity lymphedema, its application may be associated with risk (eg, skin breakdown, fluid overload) when used in the context of metastatic cancer, liver and heart failure, neuropathy, and peripheral artery disease, all of which are common in palliative care populations [48,114]. Further, CDT may also adversely affect well-being by being an overly burdensome treatment. However, one small review demonstrated improvements in limb volume, skin quality, and lymphedema-related quality of life for CDT in palliative cancer patients [115]. Therefore, the benefits and burdens of a full program need to be assessed to determine whether it may meet patient needs or represent an unduly burdensome aspect of care at the end of life.

Modifications may be necessary for patients in palliative care who are candidates for CDT to reduce complications. Examples of this include [48,113,116]:

●Modifying multiple-layer bandaging technique (lower pressure, fewer layers, frequent reapplication for lymphorrhea).

●Use of extra padding and skin protective materials for patients with impaired arterial circulation or sensation.

●Transitioning between compression garments and multiple-layer bandaging.

●Shortened bandaging durations between skin checks.

●Modifying the frequency and pressure of MLD.

●Decreasing the pressure of IPC to no more than 30 mmHg.

●Using exercises to promote blood flow during bandaging (eg, repetition of ankle pumps or circles), especially in patients with arterial insufficiency.

For patients with heart failure or chronic kidney disease, positioning adjustments might be needed (eg, not elevating the involved extremity, as it might reduce perfusion) [48]. In addition, initial bandaging should be done on a limited portion of the involved extremity to evaluate the impact more proximally, which can guide the extent of bandaging in future treatment. Patients and caregivers should be educated to identify dyspnea and/or abdominal bloating, which may reflect an adverse effect of fluid redistribution.

FOLLOW-UP

Frequency — The frequency of follow-up depends on the severity of lymphedema and the intensity of complete decongestive therapy (CDT). For patients with mild lymphedema, ongoing monitoring every three to six months and as needed should be sufficient to address patient concerns. For moderate and severe lymphedema, follow-up may be as frequent as weekly during the treatment phase or increasing periods between visits during the early maintenance phase.

Referral for surgery — Referral for surgical evaluation is appropriate for any patient being treated for lymphedema (ISL stage I through III) (table 2) if the desired limb volume reduction has not been achieved with conservative management, the patient is experiencing complications or if the patient is motivated to pursue other avenues for treatment. Earlier intervention prior to tissue fibrosis and severe adipose deposition is more effective for treating lower extremity lymphedema. (See "Surgical treatment of primary and secondary lymphedema", section on 'Reasons for referral'.)

Other indications for referral include:

●Localized primary lesions (including microcystic and macroscopic lymphatic malformations)

●Limitation of function (eg, mobility, contracture)

●Leakage of lymph into body cavities, organs, or externally

●Deformity or disfigurement

●Pain or diminished quality of life, including emotional or psychosocial distress

Lymphedema can be surgically treated using physiologic interventions or reductive techniques. Physiologic interventions designed to restore lymphatic circulation, including lymph node transplantation and lymphovenous bypass, have shown promising results, particularly in patients with early-stage lymphedema. Reductive (excisional) procedures that aim to remove fibrofatty tissues deposited in lymphedematous limbs may be useful for patients with late-stage lymphedema.

SOCIETY GUIDELINE LINKS — 

Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Lymphedema".)

INFORMATION FOR PATIENTS — 

UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)

●Basics topics (see "Patient education: Lymphedema (The Basics)")

●Beyond the Basics topics (see "Patient education: Lymphedema after cancer surgery (Beyond the Basics)")

SUMMARY AND RECOMMENDATIONS

●Lymphedema treatment – Complete decongestive therapy (ie, CDT) is the mainstay of treatment and is aimed at improving patient comfort and reducing limb volume. CDT consists of general measures for monitoring and self-care, compression therapy, and physiotherapy to reduce limb volume. General measures are aimed at minimizing the degree of edema and slowing the rate of progression. They include self-monitoring, limb elevation, diet and exercise, and avoiding exacerbating factors. The degree of compression should be guided by the patient's vascular status and their ability to tolerate compression. (See 'Approach to management' above and 'General measures' above.)

●Complete decongestive therapy – All patients with peripheral lymphedema are managed using CDT, the type and intensity of which vary depending upon the stage of lymphedema (table 2). (See 'Complete decongestive therapy' above and 'Intensity of treatment' above.)

•At risk for postoperative lymphedema – For all patients at risk for lymphedema following surgery but without clinically apparent lymphedema (International Society of Lymphology [ISL] stage 0), we initiate general measures, which include exercise to improve mobility. (See 'General measures' above.)

•Mild lymphedema – For all patients with mild lymphedema (ISL stage I), in addition to general measures (see 'General measures' above), we suggest initiating compression therapy and self-directed physiotherapy (ie, simple lymphatic drainage) rather than more intensive CDT (Grade 2C). (See 'Compression therapy' above and 'Simple lymphatic drainage' above.)

•Moderate or severe lymphedema – For all patients with moderate or severe lymphedema (ISL stage II), in addition to general measures and compression therapy (see 'General measures' above and 'Compression therapy' above), we suggest escalating to, or initiating, manual lymphatic drainage (MLD) to intensify CDT (Grade 2B). For patients who do not achieve anticipated volume reductions, we suggest the addition of IPC (Grade 2C). (See 'Effectiveness of complete decongestive therapy' above and 'Intermittent pneumatic compression' above and 'Manual lymphatic drainage' above.)

●Other treatments – Pharmacologic or other treatments are not generally used. No drug has conclusively been shown to be beneficial. In particular, diuretics should not be given because edema fluid cannot be easily mobilized into the vascular space. (See 'Unproven and contraindicated treatments' above.)

●Surgical referral – Any patient can be referred for surgical evaluation for either physiologic treatments (eg, lymph node transfer, lymphovenous bypass) or reductive procedures if clinical goals have not been achieved with conservative management or they would like to pursue other avenues for treatment. (See 'Referral for surgery' above and "Surgical treatment of primary and secondary lymphedema".)

ACKNOWLEDGMENTS — 

The UpToDate editorial staff acknowledges Tammy Mondry, DPT, MSRS, CLT-LANA, and Emile R Mohler, III, MD, who contributed to earlier versions of this topic review.