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Diagnosis and management of lymphoedema

VOL: 102, ISSUE: 13, PAGE NO: 47

Brenda King, MMedSci, BSc, DN, RGN, is clinical nurse specialist, tissue viability, Sheffield South West PCT

The term 'lymphoedema' describes the abnormal collection of fluid in the interstitial spaces. This accumulation of ...

The term 'lymphoedema' describes the abnormal collection of fluid in the interstitial spaces. This accumulation of relatively protein-rich fluid results from a dysfunction of the normal flow of lymph fluid. In the early stages, such a collection of fluid is called simple oedema: the area may be soft and pits easily with finger pressure and may improve when, for example, the affected limb is elevated. However, without treatment the condition becomes steadily worse, and in longstanding lymphoedema there is an accumulation of excessive interstitial fluid, which is accompanied by fibrous tissue and fat deposits (Stanton, 2000). The tissues become hard and are commonly termed 'brawny'. At this stage the oedematous area cannot be palpated, and the swelling does not reduce overnight (Fig 1). Lymphoedema is classified as being primary or secondary; it can affect the upper and lower limbs, the genitalia, the head and the trunk.

Incidence

Little is known about the epidemiology and incidence of lymphoedema. Published literature mainly refers to arm lymphoedema following breast surgery and suggests it develops in 25 to 29% of patients (Querci del Rovere et al, 2003; Mortimer et al, 1996; Kissin et al, 1986). It is likely that it develops in equal numbers of patients who have leg oedema following surgery for tumours of the pelvis or for melanomas when there is secondary spread to the lymph nodes (Serpell et al, 2003). The incidence of lymphoedema following radical vulvectomy and the dissection of lymph nodes for treatment of vulval cancer can be as high as 70% (Stehman, 1992).

Types of lymphoedema

Primary lymphoedema

This is thought to result when there has been under-development of the lymph vessels. It was previously called idiopathic lymphoedema. Primary lymphoedema is caused by a congenital lymphatic dysplasia (International Society of Lymphology, 2003).

Following lymphography studies, Kinmonth et al (1957) identified and described three different characteristics of the lymph vessels in people with lymphoedema:

- Hypoplasia - the lymph vessels are fewer in number and smaller than usual;

- Aplasia - the main collector vessels are not adequately formed;

- Hyperplasia - the main collector vessels are too large and dilated to be effective.

A number of conditions relating to primary lymphoedema include Milroy's disease (a congenital abnormality of the lymphatic vessels resulting in lymphoedema of the legs), and Klippel-Trenaunay-Weber syndrome. The latter is characterised by soft tissue and bony hypertrophy (excessive growth of the soft tissue and/or bones), venous malformations and lymphatic abnormalities.

Secondary lymphoedema

The causes of secondary lymphoedema include the following:

- Cancer, or its treatment by radiotherapy or surgery;

- Inflammation, as in inflammatory arthritis;

- Trauma, which may be accidental or surgical;

- Infection, which may result from bacterial cellulitis, tuberculosis or filarial infections caused by parasitic worms such as Wuchereria bancrofti and Onchocerca volvulus.

Diagnosis

A true lymphoedema occurs when there is a fault in the lymphatic system itself (Williams, 1997) (Box 1). It is essential that an accurate diagnosis is made as to the cause of the lymphoedema so as to rule out other causes, such as venous hypertension, cardiac failure, protein deficiency, lipoedema (abnormal deposition of subcutaneous fat), deep vein thrombosis and immobility. Doing so, will ensure that the patient receives appropriate treatment.

The diagnosis should be based on the clinical features, the patient's past medical history, and investigations.

Investigations

The diagnosis of lymphoedema using investigative techniques has been an issue of debate, as most practitioners base their decision on the patient's medical history and the clinical features. The aim of investigations is to detect the site of the malfunction in the lymphatic system or to confirm the diagnosis. Investigations include:

- Direct lymphangiography - this used to be considered the gold standard, but is used less now because of the risk of further damaging the lymphatic system where the contrast medium is injected directly into the peripheral lymphatic vessels;

- Indirect lymphangiography - this uses a radio-opaque medium that is injected into the dermal tissue;

- Fluorescence lymphangiography;

- Lymphoscintigraphy: injection of a radioactive protein or colloid into the tissues;

- Computed tomography and MRI;

- Doppler ultrasound - to assess skin thickness and also to evaluate venous incompetence or abnormalities, including deep-vein thrombosis.

The use of Doppler ultrasound to assess arterial problems is still being debated. It is used to identify patients with compromised arterial circulation before the application of compression therapy. However, it has been suggested that using a sphygmomanometer cuff around an at-risk limb can lead to the development of lymphoedema.

When gross oedema with associated brawny fibrotic skin changes is present it is highly likely that any attempt to measure the ankle brachial pressure index would give an inaccurate reading. With improved technology it is now possible to measure a toe brachial pressure index using a digital cuff and the Vascular Assist (a portable vascular laboratory) (Huntleigh Diagnostics).

Treatment

Lymphoedema is a chronic condition. Treatment is aimed at reducing the oedema and restoring the equilibrium in the interstitium by encouraging lymph fluid to flow through collateral vessels (Foldi et al, 1985), so minimising complications and preventing further oedema. The principles of treatment are based on four important cornerstones of management:

- Skin care;

- Compression;

- Lymphatic drainage;

- Exercise.

Patient education is fundamental in underpinning the treatment and enabling success. It should focus on self-management and include patient empowerment, choice and the development of the expert patient.

Skin care

Meticulous skin care is one of the most important aspects in the management of lymphoedema. The aims are to reduce the risk of infection and maintain the skin in a healthy condition.

Skin care should include the following:

- Daily inspection;

- Paying attention to hygiene;

- Moisturisation.

Many patients have repeat acute inflammatory episodes (AIE), a term used to describe infection and cellulitis, but good skin care will reduce this risk. Predisposing factors for the development of AIE include skin injury, lymphorrhoea, fungal infection and poor skin condition.

Regular inspection should be made of the colour and condition of the skin and of the condition of the nails; patients should also check they have no problems with folliculitis, and check for the presence of scratches, blisters, papules or plaques. Feeling their skin to check that its temperature is not raised is also important, as heat may indicate infection.

Hygiene measures should include paying particular attention to skinfolds, which may be macerated and made worse by creams.

The use of emollients such as aqueous cream should be encouraged. Patients should be advised to apply the moisturiser at bedtime; if they apply it to their limb in the morning it may cause problems when they put on their compression garment (Veitch, 1993). Other advice to give to patients that is related to skin care includes:

- Wear protective footwear at all times if leg lymphoedema is the problem;

- Wear gloves when washing up or gardening if arm lymphoedema is the problem;

- Take care when using sewing needles;

- Avoid insect bites; use a repellent but treat any bites promptly;

- Seek immediate advice about any cuts, scratches or grazes or if the limb becomes more hot or swollen;

- Do not allow injections to be given to the affected limb or blood to be taken from it;

- Protect the limb from direct sun.

Compression hosiery

Todd (2000) suggests managing lymphoedema by containment, which he defines as enclosing, restricting and keeping under control, thereby preventing the development of oedema. In cases of mild oedema, hosiery can be applied straightaway. This assumes that the limb oedema is confined to the limb, and that the limb is a normal shape (British Lymphology Society, 1999).

Care should be taken during the assessment to eliminate patients for whom compression is contraindicated, such as those with arterial disease, thrombosis, acute inflammatory episodes or allergies.

It is essential that accurate limb measurements are taken by a suitably trained practitioner; failure to do so could result, at the least, in ineffective management and at the worst, to trauma to the underlying skin and tissues. There is a range of hosiery available on the Drug Tariff. Garments can also be made to measure, and include items such as mittens for oedematous hands. It is essential that the hosiery is worn every day.

Patients should be encouraged to have a routine of removing any garment at night and reapplying it first thing in the morning. Hosiery must be renewed on a regular basis as, over time, it stretches and can be ineffective. Sometimes, patients using compression garments need to have periods of intensive bandaging at regular intervals as part of their maintenance programme.

Multi-layer bandaging

For many patients, their oedema needs to be reduced with intensive therapy using a multilayer bandage system before they are fitted for hosiery garments. This involves using short-stretch bandages that should be applied only by practitioners who have had appropriate training and practice (Todd, 2000).

Short-stretch bandages are inelastic and made of 100% cotton. They provide high working pressures on exercise and low resting pressures.

The bandages are used in conjunction with wool and foam padding. This protects the limb, achieves optimum shape and avoids the bandages slipping.

Most of the oedema will be reduced during the first few days of bandaging, although it may be continued for two to three weeks before the maintenance period with compression hosiery.

During this period of intensive management, manual lymphatic drainage is also advocated.

Manual lymphatic drainage

This is a form of massage that assists in the removal of interstitial fluid away from congested areas. It differs significantly from standard massage techniques and it is important that a trained therapist performs it (Leduc and Leduc, 2000). This type of drainage can be an effective treatment for managing lymphoedema without using compression. In certain cases of lymphoedema, bandaging cannot be used, especially when it is associated with head and neck problems.

Patients and relatives can be taught the principles of simple lymphatic drainage, which is a simplified form of the manual technique.

Exercise

Lack of movement contributes significantly to the development of dependent oedema. Exercise is important for the propulsion of lymph (Mortimer, 1995) and therefore in the treatment of lymphoedema, but published evidence to support it is scarce. An exercise programme can be helpful in reducing lymphoedema, but it must be tailored to meet the individual's needs, taking into consideration the severity of the lymphoedema, the patient's age and physical condition.

During periods of exercise it is essential that compression (either hosiery or bandages) is used.

Conclusion

Lymphoedema management is a specialist area, but all healthcare professionals can make a contribution to managing patients successfully. Nurses working in primary care can support patients in maintaining good skin care and in monitoring the effectiveness of the hosiery during the maintenance period.

Most important is early recognition of the development of oedema and of the need for prompt referral for assessment and diagnosis.

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