Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

all patients

1st line – skin care

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all patients
1st line – 

skin care

All patients should be advised to maintain good skin care and avoid trauma to the skin.[12]

Diligent skin moisturizing and regular bathing can minimize infection and dermatologic changes (e.g., skin breakdown with consequent lymphorrhea, fungal growth, or ulceration; hyperkeratosis, papillomatosis, and induration).[3][18]

Patients should wear protective clothing, particularly when outdoors, and avoid walking barefoot. Even minor cuts may result in cellulitis, which can worsen lymphedema, as any remaining functional lymphatics are further damaged. Advise patients to remain attentive to any changes noticed within the at-risk limb, and, if they identify any changes, to seek medical advice.[12]

Plus – static compression bandaging or garments

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all patients
Plus – 

static compression bandaging or garments

Treatment recommended for ALL patients in selected patient group

Single or multilayered garments providing static compression are the mainstay of conservative treatment, and have been demonstrated to reduce progression of lymphedema.[1][73]

Medical-grade garments (minimum 30 mmHg) can reduce swelling in patients with secondary lymphedema of the arm.[68] Controlled compression therapy with garments that are progressively tightened can reduce upper extremity volume by approximately 47%.[54] Multilayered bandaging with joint padding is more effective than single-layered garments but reduces range of motion and can cause discomfort.

Clinical experience suggests that the choice of circular versus flat knit elastic compression is dependent upon the severity of lymphedema and on limb shape; circular knit garments are usually the first type of compression garment used for people with relatively mild swelling and more typically shaped legs, and provide better containment (i.e., the fabric is stiff and resists expansion). Flat knit garments provide less containment, and may be customized to accommodate any shape of leg.[1]​ Although static compression is effective, patient compliance is often poor because garments may be uncomfortable and cause social morbidity.

It is important to note that compression bandaging can be harmful and/or ineffective when applied incorrectly; multilayer wrapping should be carried out only following adequate professional training.[47]​ Patient education is likely to be of benefit.

Plus – elevation

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all patients
Plus – 

elevation

Treatment recommended for ALL patients in selected patient group

This can reduce edema, but improvement is minimal and temporary. One study demonstrated a 3.1% volume reduction with elevation of an affected arm at an 80° angle for 5 hours.[68]

Although elevation is often burdensome to the patients, they are encouraged to elevate the extremity whenever convenient.

Plus – exercise

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all patients
Plus – 

exercise

Treatment recommended for ALL patients in selected patient group

Exercise is not contraindicated in patients with lymphedema and has been demonstrated to be safe; furthermore, it is likely to improve associated symptoms, function, fitness, and quality of life.[47][74][75][76][77][78][79]​ Ideally, exercise programs should be carried out under the supervision of a lymphedema specialist, with a gradual increase in intensity of exercise recommended. Exercise programs typically incorporate both aerobic and resistance exercises.[12]​ For example, weight lifting has been shown to decrease the incidence of lymphedema exacerbations, reduce symptoms, and increase strength in patients with secondary lymphedema of the upper extremity.[13][80]​​

Water-based exercise programs have shown some success within randomized controlled trials, but they are not suitable for all patients (e.g., those with wounds or certain skin conditions).[47][81][82]

The International Society of Lymphology recommends that basic motion exercises of the extremities may be helpful (muscle pumping exercises), preferably performed as daily life activities (walking, using stairs over escalators, hanging clothes on the washing line rather than using the dryer).[47]

Plus – weight control

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all patients
Plus – 

weight control

Treatment recommended for ALL patients in selected patient group

Patients should maintain a normal body mass index (BMI). Obesity increases the risk of developing upper extremity lymphedema following breast cancer treatment.[37][38]​ In addition, super obesity (BMI >50 kg/m²) can cause bilateral lower extremity lymphedema.[39][40][41]​ Although there is currently only limited evidence to support that weight loss improves lymphedema, weight loss is likely to improve associated symptoms, and have other secondary benefits including improved insulin control, and improved psychosocial functioning.[47]​ Experience suggests that obesity-induced lymphedema is not reversible following massive weight loss.[42] Refer people with obesity to a bariatric weight-loss center before their BMI reaches a threshold for obesity-induced lymphedema and massive localized lymphedema to develop.[43]

Consider – complex decongestive therapy (CDT)

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all patients
Consider – 

complex decongestive therapy (CDT)

Treatment recommended for SOME patients in selected patient group

A manual technique backed by longstanding experience.[47][66][67][71]​ Generally involves a two-stage treatment program, with phase 1 involving intensive treatment, with phase 2 as a maintenance phase.[10][47]

Stage 1: Includes manual lymphatic drainage, a light massage technique, and sometimes deeper massage techniques using muscle pumping exercises, plus compression that is usually applied using multilayered bandage wrapping.[47][72]​ Frequency of treatment is usually twice daily during this phase, typically for around 14 days.[10]

Stage 2: Follows immediately after stage 1, and incorporates compression with a low-stretch elastic sticking or sleeve, skin care, continued exercise, and manual lymphatic drainage, as required.[47]​​

Prescription of long-term elastic garments is required after stages 1 and 2; this should be done by an experienced specialist (e.g., specialist lymphedema physician) to avoid medical contraindications.[47]

CDT is resource-intensive, and requires the availability of an experienced specialist multidisciplinary team encompassing clinical lymphologists, specialist nurses, physical therapists, and occupational therapists.[47]​ CDT has been demonstrated to reduce limb volume by 4% to 66%.[68]

Consider – intermittent pneumatic compression

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all patients
Consider – 

intermittent pneumatic compression

Treatment recommended for SOME patients in selected patient group

This provides an in-home, simplified treatment regimen (compared with complex decongestive therapy [CDT]) using a pneumatic pump device. In some locations, this may be the only decongestive therapy available to patients, but it can also be used as part of a multicomponent treatment program including manual decongestive therapy and compression.[1]​ Devices differ in the number of compartments that apply compression and the presence of a distal-to-proximal pressure gradient.

Static compression garments should be worn to maintain edema reduction following external compression with the pneumatic device.

The technique is well tolerated and associated with a significant reduction in limb girth, quality of life, reduction in the risk of cellulitis, and reduced healthcare costs (primarily associated with reduced incidences of cellulitis as well as reduced use of physical therapy and occupational therapy).[83][84][85]

Pneumatic compression reduces limb volume by 37% to 69%.[118] Studies show a significant reduction in volume in patients treated with intermittent pneumatic compression and CDT, compared with CDT alone.[68] 

Consider – psychosocial support

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all patients
Consider – 

psychosocial support

Treatment recommended for SOME patients in selected patient group

Frequency of infection, pain, poor skin quality, and reduced limb function may affect quality of life.[86] In addition, patients may experience distress associated with poorly fitting clothing and concerns about their physical appearance. Therefore, providing ongoing psychosocial support helps to improve overall patient well-being. Options include quality of life assessment-improvement programs and patient self-efficacy assessments, depending on service availability.[47]​ Support groups are a consideration.

National Lymphedema Network: patient support groups (US) Opens in new window

Consider – pharmacotherapy for filariasis

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all patients
Consider – 

pharmacotherapy for filariasis

Treatment recommended for SOME patients in selected patient group

Diethylcarbamazine is the preferred drug to destroy the microfilariae associated with lymphatic filariasis. It is available only from the US Centers for Disease Control and Prevention (CDC).

Albendazole and ivermectin have also proved beneficial.​[87]

Consultation with an infectious diseases specialist is recommended to manage the drug regimen, as dose recommendations and treatment courses vary.

Primary options

diethylcarbamazine: consult specialist for guidance on dose

Secondary options

ivermectin: consult specialist for guidance on dose

OR

albendazole: consult specialist for guidance on dose

Consider – surgery + postoperative static compression garment

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all patients
Consider – 

surgery + postoperative static compression garment

Treatment recommended for SOME patients in selected patient group

Indications for surgical treatment include failure of conservative treatment and significant morbidity, including loss of function, recurrent infections, and severe psychosocial morbidity.[1][3][18]​​

Excisional techniques remove affected tissues; clinical experience suggests that they are likely to be more effective at addressing lymphedema in more advanced stages (i.e., stages 2 and 3), where excess fibro-adipose tissue has accumulated.[1]​ Suction-assisted lipectomy, similar to the concept of liposuction in cosmetic surgery, is being increasingly utilized by surgeons in multiple countries worldwide.[10][47][94]​ Evidence suggests that it is effective for removing nonfluid components such as fat in lymphedema, and is associated with increases in quality of life; potential risks include venous thromboembolism, fat embolism, and fluid overload.[54][94][95][96][97][98]​​​[99]​ Some patients have improved transit of radiolabeled sulfur colloid through lymphatic vasculature postoperatively.[101]

Other excisional procedures include the Charles procedure (which, due to a high morbidity rate, is rarely performed) and staged subcutaneous excision.[47]

Physiologic procedures use microsurgical techniques to re-establish lymphatic connections by creating new channels, lymph-venous anastomosis, or transferring lymphatics to an affected area. They are generally considered less predictable compared with excisional procedures that remove affected tissue.[89]​ Clinical experience suggests that physiologic procedures are likely to lead to better outcomes in earlier stages of lymphedema, before adipose deposition and fibrosis occurs.[1]​ Judicious use of imaging tools is required both preoperatively as part of planning of surgery, and postoperatively to ensure short- and longer-term efficacy.[47]

Examples include lymphangioplasty, lymph node-venous anastomosis, lymphaticovenous anastomosis, lymphatic grafting, or pedicled flap transposition, and free-tissue transfer.[60][89]​​[105][106]​​[107][108][109][110][111][112][113][114][115]

Lymphatic-venous anastomosis is currently in use in many centers around the world, and is backed by evidence confirming long-term patency of 25 years and more.[47][114][115]​ Lymph node transfer operations have also demonstrated efficacy and are gaining in popularity within many centers, but there is a risk of developing lymphedema at the lymph node donor site; careful selection of the donor site is therefore required.[10][47][116][117]

Surgical procedures are not curative and require lifelong compression with a static compression garment to maintain limb volume reduction.

In some centers, a physiologic technique using microsurgery may be combined with an excisional procedure (e.g., suction-assisted lipectomy) with the aim of decreasing the need for continual compression postoperatively.[47]​ Combination surgical treatment, which combines both a physiologic and an excisional technique, is also sometimes considered for people with end-stage extremity lymphedema.[10][91][92][93]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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