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Management of Lymphedema

 

Lymphoedema may manifest as swelling of one or more limbs and may include the corresponding quadrant of the trunk. Swelling may also affect other areas, eg the head and neck, breast or genitalia. Lymphoedema is the result of accumulation of fluid and other elements (eg protein) in the tissue spaces due to an imbalance between interstitial fluid production and transport (usually low output failure).  It arises from congenital malformation of the lymphatic system, or damage to lymphatic vessels and/or lymph nodes. In patients with chronic lymphoedema,large amounts of subcutaneous adipose tissue may form. Although incompletely understood, this adipocyte proliferation may explain why conservative treatment may not completely reduce the swelling and return the affected area to its usual dimensions. Lymphoedema may produce significant physical and psychological morbidity. Increased limb size can interfere with mobility and affect body image. Pain and discomfort are frequent symptoms, and increased susceptibility to acute cellulitis/erysipelas can result in frequent hospitalisation and longterm dependency on antibiotics.  Lymphoedema is a chronic condition that is not curable at present, but may be alleviated by appropriate management; if ignored, it can progress and become difficult to manage.

At birth, about one person in 6000 will develop primary lymphoedema; the overall prevalence of lymphoedema/chronic oedema has been estimated as  0.13-2%13-15. In developed countries, the main cause of lymphoedema is widely assumed to be treatment for cancer. Indeed, prevalences of 12-60% have been reported in breast cancer patients and of 28-47% in patients treated for gynaecological cancer. However, it appears that about a quarter to a half of affected patients suffer from other forms of lymphoedema, eg primary lymphoedema and lymphoedema associated with poor venous function, trauma, limb dependency or cardiac disease Effective identification of patients at risk of lymphoedema relies on awareness of the causes of lymphoedema and associated risk factors, implementation of preventive strategies, and self monitoring.

 

 

Primary lymphoedema 

 

Primary lymphoedema results from congenital abnormality or malformation of the

lymphatic system and is associated with one or a combination of the following:

  • Dysplasia—malformation of the lymphatics
  • Hypoplasia—number and/or diameter of lymph collectors is below normal
  • Hyperplasia—higher number of lymph collectors than normal and tortuous like varicose veins

Secondary lymphoedema

 

Secondary or acquired lymphoedema results from obstruction, damage or mechanical

insufficiency of the lymphatic system which may result from a number of causes.

Classification of causes of secondary lymphoedema

Trauma and tissue

damage

·         Surgical removal of lymph nodes and vessels

·         Radiotherapy to lymph nodes and vessels

·         Varicose vein surgery/harvesting

·         Other surgical procedures to the affected limb

·         Severe burns

·         Large and/or circumferential wounds

·         Significant scarring (deep or circumferential)

·         Compound fractures or extensive soft tissue injuries

Venous disease ·         Chronic venous insufficiency

·         venous ulceration

·         post-thrombotic syndrome (can occur following venous

·         Thromboembolism)

·         Intravenous drug use

Malignant disease ·         Lymph node metastases

·         Infiltrative carcinoma

·         Lymphoma

·         Pressure from large tumours (note: may be new or previously

·         Detected)

Infection  ·         Fungal

·         Bacterial— cellulitis/erysipelas, lymphadenitis

·         Parasitic—filariasis (transmitted by mosquito bites) and other

·         Insect/spider bites

·         Tuberculosis

Inflammation ·         Psoriatic arthritis

·         Rheumatoid arthritis

·         Dermatitis/eczema

·         Sarcoidosis and oro-facial granulomatosis

·         Podoconosis/lymphonoditis (silicates)

·         Pretibial myxoedema (rare endocrine disease)

Immobility/dependency ·         Severe/chronic dependency oedema

·         Morbid obesity

·         paralysis

 

Assessment

 

The medical assessment is used to diagnose lymphoedema and to identify or exclude other causes of swelling.

Differential diagnosis of lymphoedema

Unilateral limb swelling:

■ acute deep vein thrombosis

■ post-thrombotic syndrome

■ arthritis

■ Baker’s cyst

■ presence/recurrence of carcinoma

Symmetrical swelling:

■ congestive heart failure

■ chronic venous insufficiency

■ dependency or stasis oedema

■ renal dysfunction

■ hepatic dysfunction

■ hypoproteinaemia

■ hypothyroidism/myxoedema

■ drug induced (eg calcium channel blockers, steroids, non-steroidal anti-inflammatories)

■ lipoedema

 

Screening investigations

Blood tests:

■ Complete blood count (CBC)

■ urea and electrolytes (U&Es)

■ thyroid function tests (TFTs)

■ liver function tests (LFTs)

■ plasma total protein and albumin

■ fasting glucose

■ erythrocyte sedimentation rate

(ESR)/C-reactive protein (CRP)

■ B-natriuretic peptide

Urine dipstick testing, including observation

for chyluria

Ultrasound

Chest X-ray

Specific investigations

  • Ultrasound – to assess tissue characteristics, eg for skin thickening and tissue fibrosis
  • Colour doppler ultrasound26 – to exclude deep vein thrombosis and evaluate venous abnormalities
  • Lymphoscintigraphy – to identify lymphatic insufficiency in patients where the cause of the swelling is unclear, to differentiate lipoedema and lymphoedema and to evaluate potential candidates for surgery. Quantitative lymphoscintigraphy (lymphoscintigraphic function test) involves a dynamic (exercise) component in addition to the static (resting) phase and provides additional information on lymphatic transportation
  • Micro-lymphangiography using fluorescein labelled human albumin –to assess dermal lymph capillaries
  • Indirect lymphography using water soluble contrast media – to opacify initial lymphatics and peripheral lymph collectors and to differentiate lipoedema and lymphoedema
  • Ct/mri scan – to detect thickening of the skin and the characteristic honeycomb pattern produced by lymphoedema, to detect lymphatic obstruction by a tumour at the root of a limb or in the pelvis or abdomen, and to differentiate lipoedema and lymphoedema
  • Bioimpedance – to detect oedema and monitor the outcome of treatment
  • Filarial antigen card test – to detect infection with wuchereria bancrofti by testing for antibodies to the parasite in a person who has visited or is living in a lymphatic filariasis endemic area.

 

Lymphoedema staging

International Society of Lymphology (ISL) lymphoedema staging

ISL stage 0

A subclinical state where swelling is not evident despite impaired lymph transport. This stage may exist for months or years before oedema becomes evident

ISL stage I

This represents early onset of the condition where there is accumulation of tissue fluid that subsides with limb elevation. The oedema may be pitting at this stage

ISL stage II

Limb elevation alone rarely reduces swelling and pitting is manifest

ISL late stage II

There may or may not be pitting as tissue fibrosis is more evident

ISL stage III

The tissue is hard (fibrotic) and pitting is absent. Skin changes such as thickening, hyperpigmentation, increased skin folds, fat deposits and warty overgrowths develop.

Classification of severity

One method of establishing the severity of unilateral limb lymphoedema is based on the difference in the limb volume of the affected and unaffected limbs .

There is currently no formal system for the classification of the severity of bilateral limb swelling or lymphoedema of the head and neck, genitalia or trunk. The severity of lymphoedema can also be based on the physical and psychosocial impact of the condition. Factors to consider include:

■ tissue swelling – mild, moderate or severe; pitting or nonpitting

■ skin condition – thickened, warty, bumpy, blistered, lymphorrhoeic, broken or ulcerated

■ subcutaneous tissue changes –fatty/rubbery, nonpitting or hard

■ shape change – normal or distorted

■ frequency of cellulitis/erysipelas

■ associated complications of internal organs, eg pleural fluid, chylous ascites

■ movement and function – impairment of limb or general function

■ psychosocial morbidity

Severity of unilateral limb lymphoedema

  • Mild: <20% excess limb volume
  • Moderate: 20-40% excess limb volume
  • Severe: >40% excess limb volume

Assessment of swelling

The duration, location and extent of the swelling and any pitting should be recorded, along with the location of any lymphadenopathy, the quality of the skin and subcutaneous tissue, and the degree of shape distortion. Limb circumference and volume should be measured. Limb volume measurement Limb volume measurement is one of the methods used to determine the severity of the lymphoedema, the appropriate management, and the effectiveness of treatment. Typically, limb volume is measured on diagnosis, after two weeks of intensive therapy with multi-layer inelastic lymphoedema bandaging (MLLB), and at follow-up assessment.  In unilateral limb swelling, both the affected and unaffected limbs are measured. The difference in limb volume is expressed in millilitres (ml) or as a percentage. Oedema is considered present if the volume of the swollen limb is more than 10% greater than that of the contralateral unaffected limb. The dominant limb should be noted: in unaffected patients, the dominant limb can have a circumference up to 2cm greater and a volume as much as 8-9% higher than the nondominant limb. In bilateral limb oedema, the volume of both limbs is measured and used to track treatment progress.

Assessment of skin condition

The general condition of the patient’s skin and that of the affected area should be assessed for:

■ dryness

■ pigmentation

■ fragility

■ redness/pallor/cyanosis

■ warmth/coolness

■ dermatitis

■ cellulitis/erysipelas

■ fungal infection

■ hyperkeratosis

■ lymphangiectasia

■ lymphorrhoea

■ papillomatosis

■ scars, wounds and ulcers

■ lipodermatosclerosis

■ orange peel skin (peau d’orange)

■ deepened skin folds

■ Stemmer sign

Vascular assessment

The arterial vascular status of the legs of all patients with lower limb lymphoedema should be assessed. The presence of peripheral arterial occlusive disease may contraindicate compression therapy or necessitate a reduction in the level of compression used. Ankle-brachial pressure index (ABPI) provides an objective measure of the patency of the large arteries supplying blood to the foot. It is calculated from the ratio of the highest ankle systolic pressure for each limb to the highest systolic pressure in the arm. There are limitations to the test particularly in the presence of lymphoedema. Tissue thickening, hyperkeratosis or oedema may make it difficult to detect blood flow using the standard 8MHz probe. The use of a 4MHz probe and a larger size blood pressure cuff may overcome these problems. An ABPI of 1.0-1.3 is normal; an ABPI of <0.8 indicates a degree of lower limb arterial occlusive disease that precludes the use of high compression. Inability to obliterate the pulse signal during measurement or an ABPI>1.3 also indicates vascular disease.

Pain assessment

Pain has been reported to affect 50% of patients with lymphoedema, with most taking regular analgesia. Pain may be caused by:

■ inflammation

■ tissue distension

■ infection

■ ischaemia

■ lipoedema

■ nerve entrapment or neuropathy

■ complex regional pain syndrome

■ factitious swelling

■ radiation-induced fibrosis

■ cancer recurrence/progression

■ taxane chemotherapy

■ degenerative joint disease.

Effective assessment of pain requires noting the cause, nature, frequency, timing, site, severity and impact of the pain. Effective management strategies are dependent on the understanding that there are layers of pain in lymphoedema, eg:

■ procedural pain – resulting from the treatment of lymphoedema

■ incident pain – breakthrough pain caused by day to day activities

■ background pain – intermittent or continuous pain at rest.

Management

 

There are many treatment modalities available for lymphoedema management.

  • Manual lymphatic drainage (MLD)—use of specific massage techniques which mobilise the skin and stimulate the lymphatic system.
  • Self lymphatic drainage—self-administered version of MLD
  • Lymphoedema compression bandaging (LCB)—specialist bandaging technique where short-stretch bandages are applied in multiple layers in combination with other products
  • Compression garments—firm fitting custom made or ready to wear garments that are designed to provide compression to a limb or body part usually for long term management
  • Intermittent pneumatic compression (IPC)—a form of compression therapy that utilises an electrical air compression pump.
  • Exercise—specific exercises designed to enhance the efficiency of the muscle pump and increase lymph circulation.
  • Skin care—a skin care regimen involving meticulous hygiene, regular moisturising, protection of skin and early identification and management of skin infections
  • Education—verbal and written information about lymphoedema and its

 

 

LOWER LIMB LYMPHOEDEMA –INITIAL MANAGEMENT

Initial management of lower limb lymphoedema will involve psychosocial support, education, skin care, exercise/movement, elevation and management of any concomitant medical conditions, pain or discomfort. The patient’s initial management may also include:

■ compression hosiery

■ modified MLLB

■ intensive therapy.

Compression hosiery

Patients with mild lower limb lymphoedema (ISL stage I), minor pitting, no significant tissue changes, no or minimal shape distortion, or palliative needs may be suitable for initial management with compression hosiery. The pressure used should be guided by the patient’s vascular status and their ability to tolerate compression and manage the garment . Skin care, exercise/movement, elevation and SLD should be taught alongside self monitoring and proper application, removal and care of hosiery. Patients’ application/removal technique should be assessed and monitored. Patients should be reviewed four to six weeks after initial fitting, and then after three to six months if response is satisfactory. The patient should be reviewed at each garment renewal, ie approximately every three to six months.

Modified MLLB

Patients with ISL stage II or late stage II lower limb lymphoedema may be candidates for initial treatment with modified MLLB, outside an intensive therapy regimen. Modified MLLB may also be useful in controlling symptoms in patients with cancer-related lymphoedema and frail patients who have complex medical problems. Management should include skin care, exercise/movement, elevation, SLD and psychosocial support.

 

Intensive therapy

Intensive therapy reduces swelling by decongesting impaired lymphatic pathways, reducing lymphatic load, encouraging the development of collateral drainage routes, and stimulating the function of remaining patent routes. Intensive therapy is used in patients with ISL stage II, late stage II and stage III lower limb lymphoedema. Intensive therapy regimens use a combination of skin care, MLLB, exercise/movement and elevation. The regimen may include MLD or MLD  with IPC.  The frequency of treatment, degree of compression and type of bandaging used should be adapted according to the patient’s physical and psychosocial needs, and to the presence of venous ulceration and arterial or venous insufficiency.  Intensive therapy programmes are likely to be undertaken for a period of two to four weeks, although a maximal effect may be achieved more quickly in some patients. During this time treatment should be evaluated continuously and appropriate alterations made according to patient need and the effectiveness of the selected regimen.

Standard intensive therapy (>45mmHg)

This involves skin care, exercise/movement, elevation, MLD, and MLLB with inelastic bandages (sub-bandage pressure >45mmHg) undertaken daily.  Patients undergoing standard intensive therapy must be carefully selected and be willing and able to commit physically and emotionally to daily intensive therapy, including participation in exercise programmes.

Modified intensive therapy with high pressure (>45mmHg)

This involves skin care, exercise/movement, elevation, MLD/SLD and MLLB with inelastic bandages undertaken three times weekly.  Suitable patients are able to tolerate high levels of compression, but are unable to commit to standard intensive therapy for physical, social, psychological or economic reasons. This may include those who are elderly, obese or have poor mobility.

Modified intensive therapy with reducedpressure (15-25mmHg)

This involves skin care, exercise/movement, elevation, SLD, MLLB +/– IPC undertaken three times weekly. Patients are selected for this treatment when high levels of compression are either unsafe or difficult to tolerate. This includes those with:

■ moderate concurrent lower limb peripheral arterial occlusive disease (ABPI 0.5-0.8)50. NB Patients with ABPI <0.5 should not receive sustained compression therapy, but may benefit from special forms of IPC

■ a neurological deficit that will make sensing complications difficult

■ lipoedema/lipolymphoedema – lower levels of compression may be easier to tolerate

■ cancer requiring palliative treatment

■ co-morbidities requiring less aggressive reduction in swelling.

Intensive therapy for lymphovenous disease (35-45mmHg or 15-25mmHg)

This involves skin care, exercise/movement, elevation, and MLLB +/– IPC undertaken either daily or three times weekly. Treatment frequency will be determined by the severity of the oedema, skin condition and rate of swelling reduction. Suitable patients include those who have had deep vein thrombosis or those who have post-thrombotic syndrome, who may be at risk of developing or have existing leg ulceration. A recent review concluded that immediate ambulation with appropriate compression does not significantly increase the incidence of pulmonary embolism, produces a faster reduction of pain and swelling, and reduces the severity of post-thrombotic syndrome. MLLB may need to be modified in the presence of venous ulceration, peripheral arterial occlusive disease or immobility . IPC may be particularly useful for the many patients with venous ulceration who have poor mobility and are unable to elevate their legs

Skin Care

 

Skin problems are common in patients with lymphoedema. Swelling may produce deep skin folds where fungal and bacterial infections can develop. Chronic inflammation causes deposition of fibrin and collagen, contributing to skin thickening and firm tissue consistency. Reduced tissue compliance may further compromise lymph flow and increase the tendency to infection.  Maintenance of skin integrity and careful management of skin problems in patients with lymphoedema are important to minimise the risk of infection. The general principles of skin care  aim to preserve skin barrier function through washing and the use of emollients. Ordinary soaps, which usually contain detergents and no glycerin, should be avoided because they tend to dry the skin. Natural or pH neutral soap can be used. The perfumes and preservatives in scented products may be irritant or allergenic. In high concentrations, mineral and petrolatum based products may exacerbate dry skin conditions by occluding skin pores and preventing natural oils from surfacing. Emollients re-establish the skin’s protective lipid layer, preventing further water loss and protecting the skin from bacteria and irritants. Emollients can be bath oils, soap substitutes or moisturizers (lotions, creams and ointments). In general, ointments, which contain little or no water, are better skin hydrators than creams, which are better than lotions.

 

Intact skin

The condition of intact skin should be optimised by applying emollient at night.

Dry skin

Dry skin may vary from slightly dry or flaky to rough and scaly . Patients may complain of itching. Emollients should be applied twice daily (including after washing) to aid rehydration. If the heels are deeply cracked, emollients and hydrocolloid dressings may help.

Hyperkeratosis

Hyperkeratosis  is caused by overproliferation of the keratin layer and produces scaly brown or grey patches. Emollients with a low water content are recommended. MLLB reduces the underlying lymphoedema and improves skin condition.

Folliculitis

Folliculitis is due to inflammation of the hair follicles. It causes a red rash with pimples or pustules, and is most commonly seen on hairy limbs. The cause is usually Staphylococcus aureus, and it may precede cellulitis/erysipelas. Swabs should be taken for culture if there is any exudate or an open wound. An antiseptic wash/lotion, eg one containing chlorhexidine and benzalkonium, should be used after washing. Emollient should be applied without being rubbed in.

Fungal infection

Fungal infection occurs in skin creases and on skin surfaces that touch. It causes moist, whitish scaling and itching, and is particularly common between the toes. It can precede the development of cellulitis/erysipelas. Skin scrapings and, if nails are affected, nail clippings should be sent for mycological examination. Treatment is with terbinafine 1% cream for up to six weeks alongside meticulous skin care. In some countries, Whitfield ointment is used as an alternative. Any sign of bacterial infection should be treated promptly.

Lymphorrhoea

Lymphorrhoea  occurs when lymph leaks from the skin surface. The patient may require medical review to determine the underlying cause, eg worsening congestive heart failure. The surrounding skin should be protected with emollient, and nonadherent absorbent dressings should be applied to the weeping skin. MLLB will reduce the underlying lymphoedema, but needs to be changed frequently to avoid maceration of the skin. Frequency of change will be determined by factors such as strikethrough and the rate of swelling reduction. In the palliative situation, light bandaging may be more appropriate.

Venous eczema

Venous eczema (also known as varicose eczema or stasis dermatitis) usually occurs on the lower legs , particularly around the ankles, and is associated with varicose veins. The skin becomes pigmented, inflamed, scaly and itchy. Treatment is with topical corticosteroids in ointment form as recommended in local guidelines, eg a potent corticosteroid such as betamethasone valerate 0.1% with clioquinol 3% for seven days followed by a mildly potent corticosteroid such as clobetasone butyrate 0.05% or betamethasone valerate 0.025%. A  non-sensitising, low water content emollient should be applied during steroid treatment. If ABPI is <0.5, the patient should be referred to a vascular surgeon.

Contact dermatitis

Contact dermatitis is the result of an allergic or irritant reaction. It usually starts at the site of contact with the causative material, but may spread. The skin becomes red, itchy and scaly, and may weep or crust. Acute episodes are treated with a potent topical corticosteroid in ointment form, eg betamethasone valerate 0.1% once or twice daily. For dermatitis unresponsive to less potent corticosteroids, treatment is with a very potent topical corticosteroid such as clobetasol propionate 0.05% once or twice daily. Treatment should continue for three to four weeks, during which time the strength of the steroid and amount applied are gradually reduced.

 

CELLULITIS/ERYSIPELAS

Patients with lymphoedema are at increased risk of acute cellulitis/erysipelas, an infection of the skin and subcutaneous tissues. The cause of most episodes is believed to be Group A β-haemolytic streptococci. It may also be caused by staphylococci or other bacteria. Good skin care reduces the likelihood of cellulitis/erysipelas, and consequently the need for antibiotics. Symptoms are variable. Episodes may come on over minutes, grumble over several weeks or be preceded by systemic upset. Symptoms include pain, swelling, warmth, redness, lymphangitis, lymphadenitis and sometimes blistering of the affected part . More severe cases have a greater degree of systemic upset, eg chills, rigor, high fever, headache and vomiting. In rare cases, these symptoms may be indicative of necrotising fasciitis. The focus of the infection may be tinea pedis (athlete’s foot), venous eczema, ulceration, ingrowing toe nails, scratches from plants or pets, or insect bites.

Criteria for hospital admission

The patient should be admitted to hospital if they show:

■ signs of septicaemia (hypotension, tachycardia, severe pyrexia, confusion or vomiting)

■ continuing or deteriorating systemic signs, with or without deteriorating local signs, after 48 hours of oral antibiotics

■ unresolving or deteriorating local signs, with or without systemic signs, despite trials of first and second line oral antibiotics.

Antibiotic regimens

Antibiotic regimens for cellulitis/erysipelas in lymphoedema vary according to the clinical situation . Antibiotics should be continued for at least 14 days after an acute episode has responded clinically to treatment. It may take one to two months of antibiotic treatment to achieve complete resolution.

Antibiotics ‘in case’ The risk of further attacks of cellulitis/erysipelas in lymphoedema is high. It is recommended that patients who have had an attack of cellulitis/erysipelas carry a two week supply of oral antibiotics, particularly when away from home for any length of time, eg on holiday. Patients should be advised to start antibiotics immediately when familiar symptoms of cellulitis/erysipelas arise and to seek a medical opinion as soon as possible.

Recurrent cellulitis/erysipelas

Antibiotic prophylaxis should be offered to patients who have two or more attacks of cellulitis/erysipelas per year. After two years of successful prophylaxis the antibiotics can be discontinued. However, if cellulitis/erysipelas recurs, lifelong antibiotic prophylaxis is required.  The risk of recurrent cellulitis/erysipelas can be reduced by controlling swelling, and by treating interdigital scaling, fungal infections, folliculitis, dermatitis, open wounds (including leg ulcers) and weeping lymphangiectasia.

 

Lymphatic massage

Lymphatic massage – manual lymphatic drainage (MLD) and simple lymphatic drainage (SLD) – aims to reduce swelling by encouraging lymph flow. Manual lymphatic drainage (MLD) is a gentle massage technique that is recognised as a key component of decongestive therapy. MLD aims to encourage fluid away from congested areas by increasing activity of normal lymphatics and bypassing ineffective or

obliterated lymph vessels. Although there is a wealth of clinical opinion advocating the benefits of MLD, there are little research data to conclusively support its use MLD remains a specialist skill that needs regular practice in order to maintain competence. Deep, heavy-handed massage should be avoided because it may damage tissues and exacerbate oedema by increasing capillary filtration.

Technique

A number of different techniques exist for MLD. However, there is little evidence to demonstrate which is the most effective and for what clinical indications. Essentially, MLD is a gentle massage technique that follows the lymphatic pathways. The different methods have several aspects in common:

■ performed for up to an hour daily

■ usually performed with the patient in the lying position, unless for lymphoedema of the head and neck

■ starts with deep diaphragmatic breathing

■ treats the unaffected lymph nodes and region of the body first

■ moves proximally to distally to drain the affected areas

■ movements are slow and rhythmical

■ uses gentle pressure – if the pressure is too hard it stimulates blood flow, the skin becomes red, and more fluid is encouraged to move into the tissues

■ ends with deep diaphragmatic breathing. MLD may be conducted daily (or sometimes twice daily) or three times weekly. A course of therapy may last three or more weeks, and may be repeated at intervals of three months to one year.

SIMPLE LYMPHATIC DRAINAGE

Simple lymphatic drainage (SLD) is a simplified self-administered version of MLD that patients and carers can learn and apply themselves. Ideally, all patients should be taught SLD, unless  contraindicated There is no definitive technique for SLD, but it is similar to MLD and is conducted for  10-20 minutes daily.

For SLD to be effective, the healthcare professional must ensure that:

■ the patient/carer is motivated

■ the patient/carer is sufficiently dexterous to perform SLD

■ time is allocated for initial teaching

■ teaching is progressive and enables the patient or carer to become skilled

■ written instruction is given and technique is observed

■ competence in the procedure and the patient’s ability to cope with treatment are checked regularly.

 

Massage

Intermittent pneumatic  compression

 

Although there is considerable international debate over its effectiveness in lymphoedema, intermittent pneumatic compression (IPC) is widely used. It may form part of an intensive therapy regimen or long-term management in selected patients, and may be used with caution in the palliative situation.

WHAT IS IPC?

IPC consists of an electrical air compression pump attached to an inflatable plastic garment that is placed over the affected limb. The garment is inflated and deflated cyclically for a set period, usually about  30-120 minutes. The pressure produced by the garment can be varied. Garments may be single chambered, or contain multiple chambers (usually three, five or 10) that are inflated sequentially to provide a peristaltic massaging effect along the length of the limb towards its root.  The question of whether single or multichambered devices are more effective remains open. However, multichambered devices are used most frequently and randomised controlled trials have shown them to produce a faster effect.IPC is thought to reduce oedema by decreasing capillary filtration, and therefore lymph formation, rather than by accelerating lymph return. IPC is particularly effective in nonobstructive oedemas, eg those due to immobility, venous incompetence, lymphovenous stasis or hypoproteinaemia. In obstructive lymphoedema, ie lymphoedema resulting from lymphatic vessel/node damage or lymph node resection, SLD or MLD is recommended before IPC to stimulate lymphatic flow. It is important that compression therapy with garments or bandaging is continued after IPC to prevent rapid rebound swelling.

Contraindications to IPC

■ Untreated nonpitting chronic lymphoedema

■ Known or suspected deep vein thrombosis

■ Pulmonary embolism

■ Thrombophlebitis

■ Acute inflammation of the skin, eg cellulitis/erysipelas

■ Uncontrolled/severe cardiac failure

■ Pulmonary oedema

■ Ischaemic vascular disease

■ Active metastatic disease affecting oedematous region

■ Oedema at the root of the affected limb or truncal oedema

■ Severe peripheral neuropathy

GUIDELINES FOR USE

Consensus on the pressures suitable forIPC in lymphoedema is lacking. Careful surveillance is required to ensure that the correct technique and pressures are applied. Pressures should be adjusted according to patient tolerance and response to treatment. In general:

■ pressures of 30-60mmHg are advised

■ lower pressures are advised in palliative care, eg 20-30mmHg

■ a duration and frequency of 30 minutes to two hours daily is recommended.

IPC may exacerbate or cause congestion or a ring of fibrosis at the noncompressed root of a treated limb if the lymphatics in the root of the limb have not been cleared. IPC of the lower limbs may precipitate genital oedema. IPC is not recommended if there is oedema at the root of the limb or in the adjacent trunk.

 

Multi-layer inelastic lymphoedema bandaging

 

Multi-layer lymphoedema bandaging (MLLB) is a key element of intensive therapy regimens. For some patients it may also form part of their transition, long-term or palliative management.

MLLB uses inelastic bandages that have low extensibility and that produce high working pressures and lower resting pressures , ie they create peak pressures that produce a massaging effect and stimulate lymph flow. In certain situations , elastic bandages may be used instead. Elastic bandages produce sustained compression with smaller variations during movement

USES FOR MLLB

As well as reducing oedema, MLLB:

■ restores shape to the limb/affected area

■ reduces skin changes such as hyperkeratosis and papillomatosis

■ supports overstretched inelastic skin

■ eliminates lymphorrhoea

■ softens subcutaneous tissues.

MLLB is indicated when skin changes are marked or limb distortion and skin folds preclude compression garments

Contraindications to MLLB

■ Severe arterial insufficiency (ABPI <0.5), although modified MLLB with reduced pressures can be used under close supervision

■ Uncontrolled heart failure

■ Severe peripheral neuropathy

Achieving the desired pressure

The pressure produced by a compression

bandage can be predicted according to

Laplace’s Law

Laplace’s Law

P =      T x N x 4630

C x W

P =  sub-bandage pressure (mmHg)

T =  bandage tension (kilograms force – kgf)

N =  number of layers

C =  limb circumference (cm)

W =bandage width (cm). This law shows that sub-bandage pressure will:

■ rise with increasing bandage tension and number of bandage layers

■ decrease with increasing limb circumference and bandage width.

In practice, therefore, Laplace’s Law shows that for a larger limb requiring high levels of compression, the desired pressure may be achieved by increasing the number of bandage layers applied and increasing the tension used during application.

Frequency of MLLB system change

As yet, there is no empirical evidence to indicate how frequency of bandage change affects speed of oedema reduction or final outcome. Clinical experience recommends that MLLB systems should be changed daily for the first seven days. This will minimize bandage slippage and ensure that sub-bandage pressure is maintained as swelling reduces. According to therapy regimen and wound/skincare requirements, it may then be possible to reduce the frequency of change to two to three times per week. Continence issues may also influence the frequency of change.

Use of elastic bandaging

In some situations, the inelastic bandages used in MLLB may be replaced with a multi-layer elastic bandage regimen. The stiffness produced by the combination of layers and the inclusion of a cohesive elastic bandage produces high working pressures. However, the resting pressure is higher than with inelastic systems.  The sustained resting pressure produced by high stiffness elastic bandage systems may be useful when:

■ the patient is immobile

■ the ankle joint is fixed, ie the calf muscle pump cannot be used

■ the patient has venous ulceration and lymphatic disease

■ the patient has proven venous disease

■ large volume loss is expected, ie to increase time worn.

 

Wrapping

 

 

Compression garments

 

The main use of compression garments is in the long-term management of lymphoedema, usually following a period of intensive therapy. Compression garments are also used for prophylaxis or as part of initial treatment. They may provide the only form of compression used, or form part of a regimen that includes other types of compression. Some patients wear garments during waking hours only, for exercise only, or up to 24 hours per day.

COMPRESSION GARMENT CONSTRUCTION

Compression garments can be categorized according to method of fabric manufacture:

■ Circular knit garments – the material is continuously knitted on a cylinder and has no seam, and is used mainly to make ready to wear garments. Garments are shaped by varying stitch height and yarn tension. Circular knitgarments may be thinner and more cosmetically acceptable than flat knit garments.

■ Flat knit garments – the material is firmer and thicker than that of circular knit garments. Garments are knitted as a flat piece that is shaped by adding or removing needles. The flat piece is then joined by a seam to form the garment. Most custom made garments are made from flat knit material.

 

Criteria indicating patient suitability for compression garments

■ Good dexterity

■ Intact, resilient skin

■ No or minimal shape distortion

■ Absent or minimal pitting oedema

■ Swelling that can be contained by compression garments

■ Concordant and motivated

■ Ability to tolerate and manage hosiery (+/- carer support)

■ Ability to monitor skin condition and engage in prevention strategies

■ Symptom-based management/palliative needs

Contraindications to compression garments

■ Arterial insufficiency – ABPI <0.5 in the lower limb

■ Acute cardiac failure

■ Extreme shape distortion

■ Very deep skin folds

■ Lymphorrhoea, or other weeping skin condition

■ Extensive ulceration

■ Severe peripheral neuropathy

Exercise/movement and elevation

 

Exercise/movement are common rehabilitative interventions used to reduce oedema. At present, there is little evidence to indicate which types, intensities and frequencies of exercise may be safely used in the management of lymphoedema.

EFFECTS OF EXERCISE/MOVEMENT

 

Exercise improves muscular strength, cardiovascular function, psychological wellbeing and functional capacity. Gentle resistance exercise stimulates muscle pumps and increases lymph flow; aerobic exercise increases intra-abdominal pressure, which facilitates pumping of the thoracic duct. Combinations of flexibility, resistance and aerobic exercise may be beneficial in controlling lymphoedema. Elevation of the affected limb, ideally to just above the level of the heart, is often advised to reduce swelling. It is thought that elevation acts by maximising venous drainage and by decreasing capillary pressure and lymph production.

 Surgery

Surgical treatment of lymphoedema can be divided into three main categories:

■ surgical reduction

■ procedures that bypass lymphatic obstructions

■ liposuction.

Potential indications for surgery in lymphoedema

■ Severe deformity or marked disability due to swelling

■ Removal of redundant tissue after successful conservative therapy

■ Proximal lymphatic obstruction with patent distal lymphatics

■ Lymphocutaneous fistulae andmegalymphatics

■ Eyelid and external genital lymphoedema

■ Lack of response to compression therapy

■ Recurrent cellulitis/erysipelas

■ Intractable pain

■ Lymphangiosarcoma

SURGICAL REDUCTION

Surgical reduction (sometimes also known as debulking surgery) aims to remove excess subcutaneous tissue and skin, and may be useful in the symptomatic treatment of severe lymphoedema. However, the postsurgical morbidity of reduction operations may be considerable. In some cases, surgical reduction may be considered for lymphoedema of the eyelid or genitalia.

RESTORING LYMPH FLOW

Some surgical techniques aim to restore lymphatic function through lymphovenous anastomoses and lymphatic or venous vessel grafting, or lymph node transplantation. Anastomosis of lymph vessels to the venous system may be attempted in patients with proximal lymphatic obstruction and patent distal lymphatics, and produces better results at earlier stages of lymphostatic disease. Lymphatic grafting and lymph node transplantation require microsurgical techniques, and show promising results in carefully selected patients.

LIPOSUCTION

In patients with chronic lymphoedema, adipocyte proliferation (which may be related to an inflammatory process) may mean that conservative treatment or microsurgery do not completely resolve limb enlargement.  Liposuction has been performed on patients with long-standing breast cancer related lymphoedema. It removes excess fat tissue and is considered only if the limb has not responded to standard conservative therapy. Liposuction does not correct inadequate lymph drainage and is not indicated when pitting is present. Where concordance with compression garments after treatment is high, results have been maintained. Liposuction has also been used for primary and secondary leg lymphoedema with promising results.