Approach

Patients are best treated in a multidisciplinary center focusing on lymphedema, where physicians, surgeons, rehabilitation experts, and therapists are available. Aims of treatment are to maximize function and cosmesis, improve quality of life, minimize physical and psychologic morbidity, and prevent the development of infection.[47]​ Treatment begins with meticulous skin hygiene, use of emollients, and advice on prevention of skin injury.[12][18]

First-line management is conservative in nature; the manual technique “complex decongestive therapy” (CDT, also known as “complete decongestive therapy”) is often used, and is recommended according to treatment guidelines.[47][66][67]​ However, CDT requires substantial time, effort, resources, and specially trained clinical staff, and its benefit compared with compression garments, exercise, pneumatic compression, and self-massage at home is yet to be fully established.[68]​ High-quality evidence comparing conversative management strategies is currently lacking, even those with longstanding use within clinical practice, and so treatments are typically recommended based on the consensus of experts. Clinical experience suggests that, regardless of the chosen treatment strategy, earlier treatment produces better long-term outcomes for patients.[47]

Surgical intervention should be considered only when conservative therapy has failed and/or the patient continues to experience significant morbidity.[1][10]​​​ It is important to note that diuretics are not effective for the treatment of lymphedema.

Given that lymphedema is a long-term and incurable condition, holistic management incorporating psychosocial support, patient education, and training is important, and may boost compliance with self-management strategies.[47]​ Strategies employed may include brief anatomy and physiology education, training on compression treatment, skincare, self-massage, weight control, and exercise.[47]​ All people with lymphedema, and in particular children, may at times struggle to cope with intensive therapy regimens, or use of prescription garments; there is a need to tailor therapy to meet the needs and circumstances of the individual. For children, education may be delivered in camps, or during social and networking activities.[2][69][70]​ Lifelong follow-up, including physiologic and psychosocial measurements to evaluate treatment efficacy and quality of life, is recommended for all people with lymphedema, regardless of treatment type.[47]​ See Monitoring.

Conservative therapy

Skin care

  • 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).[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]

Complex decongestive therapy

  • 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]

Static compression

  • 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.

Compression garments explained
Compression garments explained

How to choose suitable compression garments for patients with lymphedema and how to measure a patient's legs for compression garments.

Elevation

  • This can help 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, patients are encouraged to elevate the extremity whenever convenient.

Exercise

  • Exercise 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 re 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]

Intermittent pneumatic compression

  • This provides an in-home, simplified treatment regimen (compared with 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%. Studies show a significant reduction in volume in patients treated with intermittent pneumatic compression and CDT, compared with CDT alone.[68]

Psychosocial support

  • 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

Weight control

  • 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 (OIL) is not reversible following massive weight loss.[42] Massive localized lymphedema (MLL) is a consequence of OIL and affects approximately 60% of people with obesity with lower-extremity dysfunction. People with a BMI >56 kg/m² have a 213 times greater odds of MLL developing versus people with a BMI ≤56 kg/m². Refer people with obesity to a bariatric weight-loss center before their BMI reaches a threshold for OIL and MLL to develop.[43]

Pharmacologic therapy: filariasis

The anthelmintic agent 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.

Surgical therapy

Proper patient selection and education is important when considering operative intervention; these procedures are not curative and require lifelong physical therapy and/or compression to maintain limb volume reduction.[47]​ 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]​​

Two major procedural categories exist: 1) excisional procedures that remove affected tissues, and 2) physiologic operations that use microsurgical techniques to repair or create new lymphatic connections, with the aim of increasing the rate of return of lymph to the blood circulatory system.​[88]​​

Evidence and guidance regarding patient selection and type and timing of intervention are lacking. As with any type of surgery, differences in surgical treatments exist between specialist centers.[47]​ Physiologic procedures 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, with excisional procedures more likely to be effective at addressing lymphedema in more advanced stages (i.e., stages 2 and 3), where excess fibro-adipose tissue has accumulated.[1]​ This is because in advanced lymphedema, physiologic procedures do not remove the excess fibro-adipose tissue, and therefore even if lymph flow is restored, limb volume can be only marginally improved.[10][90]​​​ 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]

Excisional procedures

  • 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]​​ This technique removes the adipose layer above the muscle fascia that occurs with lymphedema. 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]​​​ The procedure has been shown to achieve a limb volume reduction of up to 97%, as well as a 75% decrease in the incidence of cellulitis.[100]​​​​​ Although it is considered an excisional procedure, suction-assisted lipectomy may also be a physiologic procedure. Some patients have improved transit of radiolabeled sulfur colloid through lymphatic vasculature postoperatively.[101]​ Note that the surgical technique and follow-up are different to those of liposuction in cosmetic surgery; lifelong use of compression garments, together with specialist multidisciplinary follow-up, is essential.[47]

  • The Charles procedure involves excision of skin, subcutaneous tissue, and fascia, followed by skin grafting over the underlying muscle. Although recurrence is low, this procedure has a high morbidity rate (graft breakdown, lymph leakage, poor cosmesis) and therefore is rarely performed.[47]​​

  • Staged subcutaneous excision removes subcutaneous tissue while maintaining skin flaps for closure.[102] It requires two stages, and limb volume reduction can be as high as 80%.[18] The complication rate and cosmesis are superior to the Charles procedure.[18] In patients with severe lymphedema with significant skin excess, staged subcutaneous excision may be preferred to suction-assisted lipectomy.[10]​ However, compared with staged subcutaneous excision, suction-assisted lipectomy has improved efficacy and a much lower complication rate.[103][104]

Physiologic procedures

  • Lymphatic connections may be re-established by creating new channels, lymphatic-venous anastomosis, or transferring lymphatics to an affected area. 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 of surgical techniques include lymphangioplasty, lymph node-venous anastomosis, lymphaticovenous anastomosis, lymphatic grafting, 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]

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