Lymphedema

Recommendations

Identify and Treat the Cause
1 Take a complete history and perform a physical examination to diagnose lymphedema and determine the etiology. Level of Evidence
Not Assessed
2 Perform lymphoscintigraphy as the investigation of choice if clinical evaluation does not establish the diagnosis. Level of Evidence
Not Assessed
3 Implement complex decongestive physiotherapy plus short-stretch compression bandaging as initial therapy. Level of Evidence
Not Assessed
4 Implement appropriate maintenance therapy using elevation, exercise and compression garments. Level of Evidence
Not Assessed
5 Manage acute exacerbations with a course of complex decongestive physiotherapy. Level of Evidence
Not Assessed
6 Consider heat therapy as a useful adjunct. Level of Evidence
Not Assessed
7 Consider surgery for refractory cases. Level of Evidence
Not Assessed


Address patient-centered Concerns
8 Provide effective patient education to support long-term self-management of lymphedema and prevent complications. Level of Evidence
Not Assessed
9 Assess / Control pain and optimize activities of daily living Level of Evidence
Not Assessed


Provide Local Wound Care
10 Assess and document the wound at regular intervals. Level of Evidence
Not Assessed
11 Optimize local wound care: debridement, inflammation / infection control, and moisture balance. Consider biopsy of appropriate active (including biologicals) & adjunctive therapies if the wound is not healing at the expected rate. Level of Evidence
Not Assessed


Provide Organizational Support
12 Consult appropriate disciplines to maximize healing (e.g. mobility and nutrition). Level of Evidence
Not Assessed


Background

Lymphedema, or accumulation of protein rich fluid in the interstitial space , usually in one or more limbs, results from congenital or acquired abnormalities in lymphatic outflow. Lymphedema is usually chronic and is classified as either primary or secondary.

Primary lymphedema usually appears spontaneously and is divided into congenital (onset at <1 year of age), precox (onset 1-35 years of age) and tarda (onset at >35 years of age).
♦ Congenital lymphedema (6-12% of primary lymphedema) may be familial and inherited through autosomal dominant or other types of transmission. Congenital lymphedema is more common in males than females. Sporadic cases are more common than hereditary lymphedema and inherited conditions associated with lymphedema, which are relatively rare. Swelling usually involves only one leg, although both legs, the genitals and even the face may also be affected. Congenital primary lymphedema may result from aplasia or hypoplasia of peripheral lymphatics, abnormalities of the lymph trunks or valvular incompetence.
♦ Lymphedema precox (77-94% of primary lymphedema) may be familial or sporadic. It is 5-10 times more common in females than males, and its frequent appearance at puberty has led to the hypothesis that estrogen may be involved. Edema is usually unilateral and generally restricted to the foot and calf.
♦ Lymphedema tarda (11% of primary lymphedema).

Secondary lymphedema, which is considerably more common than primary lymphedema, develops when lymphatic pathways are obstructed or interrupted by surgery, radiation or a disease process.
♦ Lymphatic obstruction: Therapeutic radiation causes fibrosis and increases the risk of lymphedema. Trauma may also obstruct lymphatics. Infections, such as filariasis or streptococcal lymphangitis or cellulitis, can cause inflammation and secondary lymphedema. Tumour invasion may destroy lymphatic ducts, vessels and lymph nodes. Lymphedema can accompany both rheumatoid and psoriatic arthritis. In addition, different types of chronic edema, such as venous insufficiency and lipedema, may lead to secondary lymphedema.
♦ Lymphatic interruption: Surgery for breast, cervical or prostate cancer with lymph node excision can interrupt lymphatics.

In established disease, history, physical examination, characteristic clinical presentations and ruling out other causes may allow diagnosis. It is important to determine the etiology, as treatment of the cause is an integral part of management. Established lymphedema may involve the entire lower leg with dorsal swelling, leading to an almost uniform diameter of the leg below the knee.Swelling is usually non-pitting. Numerous skin changes may be present, including thickening, woody fibrosis, hyperkeratosis, enhanced skin creases, a positive Stemmer’s sign, a verrucous warty texture, mushroom-like papules and papillomatosis. Lymphedema is rarely associated with ulceration or pain, but recurrent cellulitis is common, and malignancy may develop in the edematous limb.

Early disease and disease with a mixed etiology may require investigation. Lymphoscintigraphy, the best and least invasive diagnostic tool currently available, can differentiate between lymphedema and edema of venous origin, identify lymphatic abnormalities following arterial reconstruction, differentiate between postthrombotic disease and lymphedema, and measure response to treatment. Both early (1-hour) and delayed (2-24-hour) films may be necessary for diagnosis. The sensitivity of lymphoscintigraphy is 73-97%, and the specificity is 100%. Lymphoscintigraphy may be adequate for preoperative assessment, or lymphangiography may also be required. Other potentially useful investigations include ultrasonography, computed tomography and magnetic resonance imaging.

Continuous fluid accumulation in lymphedema can lead to progressive lymphatic damage and further edema, recurrent infection,, impaired limb function, septic or malignant complications, and significant psychologic consequences. Skin tightness, decreased range of motion, and disfigurement are important patient concerns. Patients may also fear complications, such as infection and its consequences, including sepsis, cellulitis, chronic ulceration, necrosis, gangrene, and amputation.

Lymphedema is not curable and requires lifelong treatment to manage symptoms and prevent progression.. Successful management of lymphedema is associated with improved wound healing, decreased infection and improved quality of life. Patient education about the disease and its management is critical to empower the patient to undertake lifelong self-care. No pharmaceutical agents or dietary supplements have been proven effective and safe in the management of lymphedema. The main components of treatment are skin care, compression and support, lymphatic massage and exercise.

Meticulous skin care can reduce the risk of cellulitis and lymphangitis. Good hygiene, use of a low-pH moisturizer, and prompt treatment of conditions such as tinea pedis and minor wounds are important. Antibiotics should be available to the patient for use when signs of infection are seen, and indefinite antibiotic prophylaxis should be provided to patients with recurrent infection.

Complex decongestive physiotherapy enhances lymphatic contractility, stimulates lymph flow and improves lymphatic drainage. This specialized type of massage is the standard of care for lymphedema treatment, and it is the initial therapy, in conjunction with temporary short-stretch compression bandaging. Patients may require higher compression than those with venous disease alone. Routine maintenance is comprised of elevation, exercise and use of a compression garment. Massage is used to treat exacerbations. Thermal therapy (microwave, electromagnetic irradiation or immersion in hot water) reduces leg volume and may be combined with complex decongestive physiotherapy.

Surgery, including debulking procedures, bypass surgery, prophylactic omentoplasty or lymphovenous anastamosis may be considered for individual patients.

References

Essential Publications
1 Lymphoedema Quality Indicator
 Type: Systematic review
Badger C, Seers K, Preston N, Mortimer P. Antibiotics/anti-inflammatories for reducing acute inflammatory episodes in lymphoedema of the limbs. Cochrane Database Syst Rev 2; 2004: CD003143.
Few RCTs were found addressing the use of antibiotics and anti-inflammatories to reduce acute inflammatory episodes of lymphedema. Only three studies involving 150 patients were included in the review. None used the same intervention, and thus they could not be combined. The authors were however able to conclude that wearing a compression sleeve is beneficial, especially over the long term when compared with no compression. There is a clear need for randomized trials in this area.


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