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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1st line – 

skin care

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

Diligent skin moisturising and regular bathing can minimise infection and dermatological changes (e.g., skin breakdown with consequent lymphorrhoea, 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 lymphoedema, 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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Plus – 

static compression bandaging or garments

Treatment recommended for ALL patients in selected patient group

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

Medical-grade garments (minimum 30 mmHg) can reduce swelling in patients with secondary lymphoedema of the arm.[68] Controlled compression therapy with garments that are progressively tightened can reduce upper extremity volume by approximately 47%.[54] Multi-layered 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 lymphoedema 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 customised 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; multi-layer 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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Plus – 

elevation

Treatment recommended for ALL patients in selected patient group

This can reduce oedema, 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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Plus – 

exercise

Treatment recommended for ALL patients in selected patient group

Exercise is not contraindicated in patients with lymphoedema 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 programmes should be carried out under the supervision of a lymphoedema consultant, with a gradual increase in intensity of exercise recommended. Exercise programmes typically incorporate both aerobic and resistance exercises.[12]​ For example, weight lifting has been shown to decrease the incidence of lymphoedema exacerbations, reduce symptoms, and increase strength in patients with secondary lymphoedema of the upper extremity.[13][80]

Water-based exercise programmes have shown some success within randomised 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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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 lymphoedema following breast cancer treatment.[37][38]​ In addition, super obesity (BMI >50 kg/m²) can cause bilateral lower extremity lymphoedema.[39][40][41]​ Although there is currently only limited evidence to support that weight loss improves lymphoedema, 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 lymphoedema is not reversible following massive weight loss.[42] Refer people with obesity to a bariatric weight-loss centre before their BMI reaches a threshold for obesity-induced lymphoedema and massive localised lymphoedema to develop.[43]

Consider – complex decongestive therapy (CDT)

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Consider – 

complex decongestive therapy (CDT)

Additional treatment recommended for SOME patients in selected patient group

A manual technique backed by long-standing experience.[47][66][67][71]​ Generally involves a two-stage treatment programme, 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 multi-layered 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 consultant (e.g., consultant lymphoedema physician) to avoid medical contraindications.[47]

CDT is resource-intensive, and requires the availability of an experienced consultant multidisciplinary team encompassing clinical lymphologists, specialist nurses, physiotherapists, 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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Consider – 

intermittent pneumatic compression

Additional 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 multi-component treatment programme 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 oedema 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 physiotherapy 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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Consider – 

psychosocial support

Additional 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 programmes and patient self-efficacy assessments, depending on service availability.[47]​ Support groups are a consideration.

Lymphoedema Support Network (UK) Opens in new window

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

Consider – pharmacotherapy for filariasis

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Consider – 

pharmacotherapy for filariasis

Additional 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 special-order manufacturers or specialist importing companies in the UK (check local guidance for availability). In the US 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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Consider – 

surgery + postoperative static compression garment

Additional 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 lymphoedema 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 utilised by surgeons in multiple countries worldwide.[10][47][94]​ Evidence suggests that it is effective for removing non-fluid components such as fat in lymphoedema, 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 radiolabelled sulfur colloid through lymphatic vasculature post-operatively.[101]

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

Physiological 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 physiological procedures are likely to lead to better outcomes in earlier stages of lymphoedema, 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.[18]​​[60][89]​​[105][106]​​[107][108][109][110][111][112][113][114][115]

Lymphatic-venous anastomosis is currently in use in many centres 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 centres, but there is a risk of developing lymphoedema 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 centres, a physiological 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 physiological and an excisional technique, is also sometimes considered for people with end-stage extremity lymphoedema.[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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