Management of Lymphoedema of the Lower Limbs

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Management of Lymphoedema of the Lower Limbs

Postby patoco » Sat Jun 10, 2006 12:49 pm

Management of Lymphoedema of the Lower Limbs

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Management of Lymphoedema of the Lower Limbs

Chronic swelling of the lower limb is a familiar problem in the community and is associated with a broad range of aetiological factors (Table 1). Lymphoedema is due to a reduction in the transport capacity of the lymphatic system and is characterised by persistent swelling, specific skin and tissue changes and a tendency to recurrent infection (Table 2). In practice, however, some chronic oedemas initially develop due to a non-lymphatic cause and a mixed pathogenesis may be evident (Keeley, 2000). Effective management strategies exist for lymphoedema although the literature identifies delays in the referral of patients to appropriately qualified practitioners (Sitzia et al.,1998) and highlights a lack of knowledge within primary health care teams regarding treatment measures (Logan et al., 1996). This paper outlines epidemiological and aetiological factors, discusses relevant pathophysiology and explores assessment and treatment for people with lymphoedema of the lower limb.

Epidemiology

Secondary lymphoedema due to filiarial infection transmitted by the mosquito, is the most common form of lymphoedema worldwide and occurs in tropical regions, affecting around 19 million people in India (Campisi, 1998). Studies of the prevalence of lymphoedema in the western world are limited although work is ongoing in this field (Moffatt et al., 2003). Sitzia et al. (1998) reported results from a prospective survey using a standard dataset in 603 new patient referrals to 27 lymphoedema clinics in England over a three month period. When those with breast cancer-related swelling were excluded, leg swelling was the predominant problem occurring in 212 (83 per cent) of the remaining 254 patients.

Studies of patients following inguinal lymph node dissection for cancers such as malignant melanoma have highlighted a 40 per cent incidence of leg swelling (Shaw & Rumball, 1990; Karakousis & Driscoll, 1994). A Swedish study of 54 women following cervical cancer treatment reported a 41 per cent incidence of leg oedema (Wergren-Elgström & Lidman, 1994). The incidence of leg lymphoedema following other cancers such as prostate, ovary, vulva and bladder is not known although clinical experience suggests oedema may be a significant problem in these patients.

Prasad et al. (1990) explored the prevalence of oedema in leg ulcer patients in hospital and community, identifying oedema in 55 per cent of the group, with a 77 per cent incidence in the community-based patients. A study of 58 patients with bilateral leg oedema attending a GP clinic in the USA, identified difficulties in establishing the cause of leg swelling (Blankfield et al., 1998). Patient history, findings from physical examinations and clinical impressions were compared with the results of investigations including echocardiograms, venous duplex ultrasound scans and serum albumin levels. Although the initial clinical impression was venous insufficiency (71 per cent) and congestive heart failure (18 per cent), in reality, 22 per cent had venous insufficiency, 33 per cent had a cardiac condition and 42 per cent had pulmonary hypertension. This highlights the importance of adequate recognition and understanding of the pathophysiology underlying a chronic swelling of the lower limb.

Pathophysiology

Any oedema, whatever the cause, is due to an imbalance between capillary filtration and lymph drainage. In normal circumstances, fluid moves through the blood capillary wall into the interstitial tissues (Stanton, 2000). Interstitial pressures and colloid osmotic pressures created by the plasma proteins, control fluid reabsorption back into the venous capillary. The lymphatic system functions as a one-way drainage system, transporting excess fluid, macromolecules such as proteins and fats from the interstitial tissues and 'recycling' it into the blood circulation, via a series of lymphatic vessels and lymph nodes.

volume and tissue swelling. Damage to local lymphatics also occurs in chronic venous disease, in the presence of ulceration and/or infection. The swelling essentially becomes a lymphoedema with associated characteristics such as non-pitting fibrosis and skin problems such as papillomatosis appear alongside venous changes. Quantitative lymphoscintigraphy in patients with chronic venous disease has suggested that lymphatic pathology may be an important contributing factor to the chronic venous leg ulcer (Mortimer, 1995).

Underlying pathophysiology in other chronic oedemas is often complex. Capillary permeability is influenced by factors such as inflammation, venous hypertension and congestive cardiac failure and leading to increased capillary filtration. This overwhelms the 'normal' lymphatic drainage system, resulting in oedema. Hypoproteinaemia associated with prolonged protein loss also leads to swelling (Hofman, 1998). Lymph flow is enhanced by muscular activity; immobility and dependency may therefore also predispose an individual to develop oedema.

Some patients, particularly the elderly or those with advanced cancer, have multiple problems, resulting in a chronic oedema that is not suitable for or does not readily respond to the lymphoedema treatment programme. A comprehensive assessment of the patient with leg oedema is crucial in order to establish an accurate diagnosis and provide appropriate treatment.

In lymphoedema, the transport mechanism of the lymphatic system is reduced, leading to increased interstitial fluid

Table 1: Aetiological factors in chronic swelling of the lower leg

Type Aetiology/underlying pathophysiology

Primary lymphoedema - intrinsic abnormality in the lymphatic system which may include:

- absence (aplasia) or reduction (hypoplasia) in lymphatic vessels

- fibrosis of lymph nodes

- large, dilated, incompetent lymphatics (hyperplasia)

Secondary lymphoedema - obstruction, obliteration, insufficiency in the lymphatic system due to an extrinsic cause such as:

- eg cervical, bladder, prostate cancer and malignant melanoma

- advanced malignancy infiltrating the lymphatics

- trauma to lymphatics eg injury, non-cancer surgery, burns

- infection/inflammation eg lymphangitis, lymphangiothrombosis, chronic ulceration

- filarial infection

Non-specific/ non-lymphatic oedema

- immobility, reduced function; chronic venous insufficiency dependent limb

- tumour obstruction

- hypoproteinaemia which can occur in maliganant ascites and renal disease

- congestive cardiac failure

- lipoedema

Assessment

The assessment of the person with lymphoedema includes a detailed history taking, clinical examination, baseline measurements of the limb and considerations of factors affecting the outcome of treatment. This enables a patient-centred treatment plan to be developed. Medical history includes:

- Relevant medical conditions.
- Cancer treatments/treatment complications.
- Current medications.
- History of the swelling - duration, precipitating factor/s, nature of onset.
- History of acute inflammatory episodes (AIE's) - frequency, treatment, symptoms.
- Psychosocial aspects - family/home life, occupation, leisure/exercise, views and expectations.

Time is taken to consider any factors contributing to or exacerbating the oedema such as obesity, lack of social support or occupational risks. Other aspects such as difficulties with clothing and/or footwear and psychosocial issues are also explored. Although studies are few, evidence suggests that lower limb oedema leads to significantly more pain (Sitzia & Sobrido, 1998) and may result in a poorer functional outcome and greater lifestyle changes (Lambert et al., 1984) than oedemas in other sites. In-depth interviews with six women with primary lymphoedema highlighted specific problems with depression, body image and feelings of isolation (Williams et al., 2001). An appreciation of the way in which each individual is affected by and copes with lymphoedema is important in establishing an individualised treatment plan and empowering and motivating the person towards self-care over the longer term. Clinical examination includes:

- Site/s and extent of the lymphoedema – does it affect the trunk and/or genitalia?
- Condition of the skin, underlying tissues and nails.
- Skin temperature and colour.
- Vascular changes.
- Function, range of movement, gait.

Measurements: Limb volume is calculated using the formula for the volume of a cylinder. This technique is widely used in the UK and requires skin circumferential measurements to be taken at 4cm intervals along the limb, not including the foot. The oedematous and non-oedematous limbs are compared in a unilateral oedema. This provides information on the excess volume and percentage excess volume of the distal and proximal segments and the total limb. It also enables the degree of shape distortion to be quantified (Badger, 1997).

Measurements of weight and body mass index are also recorded, as obesity is a risk factor. If compression therapy is to be used, the ankle brachial pressure index (ABPI) should be measured. However, in patients with gross swelling or severe fibrosis, the accuracy of the ABPI is in doubt and pulse oximetry may prove to be a more useful method for assessing arterial status in these patients (Bianchi & Douglas, 2002).

Table 2: Defining lymphoedema: clinical signs

- tissue swelling of > 3 months duration that does not reduce completely on elevation

- a positive Stemmer's sign (inability to pinch a fold of skin at the root of the second toe)

Dry, flaky skin
due to stretching of the tissues Hyperkeratosis
scaly, thickened skin due to a build-up of keratin (horny scale)

Fibrosisskin and subcutis become hard and non-pitting due to formation of fibrous tissue Papillomatosis
a cobblestone appearance of the skin surface due to dilation of the upper dermal lymphatics, followed by fibrosis

Lymphangio
a Proliferation of lymph vessels on the skin surface producing a wart-like appearance which may rupture and leak lymph fluid Acute inflammatory episodes infection, similar to cellulitis and often referred to as erysipelas which affects the lymphatics, the skin and underlying tissues. Red streaks along the limb indicate lymphangitis

Lymphorrhoea
Leakage of lymph from the skin in lymphoedema Others
Tinea pedis, contact dermatitis (irritant or allergic) are also common in these patients

Hyperkeratosis
scaly, thickened skin due to a build-up of keratin (horny scale

Papillomatosis
a cobblestone appearance of the skin surface due to dilation of the upper dermal lymphatics, followed by fibrosis

Acute inflammatory episodes
infection, similar to cellulitis and often referred to as erysipelas which affects the lymphatics, the skin and underlying tissues. Red streaks along the limb indicate lymphangitis

Others
Tinea pedis, contact dermatitis (irritant or allergic) are also common in these patients

Management strategies

Lymphoedema management combines four elements of treatment: skin and preventative care (Table 3), support and compression therapy, specialised massage and exercise (British Lymphology Society, 1999a). Treatment is provided as a two-phased approach described as Decongestive Lymphatic Therapy (DLT) (often referred to as intensive therapy) and maintenance therapy.

Decongestive Lymphatic Therapy:- Aims to reduce swelling, improve limb shape and promote skin integrity and involves:
- Skin and preventative care.
- Manual lymphatic drainage (MLD) massage.
- Multi-layer lymphoedema bandaging.
- Isotonic exercises.

Maintenance therapy:- Aims to maintain size, shape and condition of limb and involves:
- Skin and preventative care.
- Simple lymphatic drainage.
- Elastic hosiery.
- Exercises, eg walking, swimming.

Table 3: Skin and preventative care in lymphoedema
(adapted from Lymphoedema Support Network (undated) Swollen feet leaflet)
- undertake daily skin washing and moisturising
- avoid going barefoot - wear slippers or sandals at home
- avoid tight socks
- wear well-fitting shoes and keep a look out for corns or blisters
- avoid injury to the skin of the limb eg scratch, insect bite, burn, sunburn
- take care when cutting toe nails - use nail clippers
- use an electric shaver to remove hairs - not a razor
- look out for signs of fungal infection between the toes
- contact your doctor at the first signs of an acute infection
- avoid doing anything that exacerbates the swelling such as high impact sports or standing for long periods
- wear hosiery garments for flying

Table 4: Acute inflammatory episodes in lymphoedema
Look for:
- red, hot, tender limb
- sudden onset
- 'flu-like symptoms
- malaise and nausea
- local oedema

Managing the acute inflammatory episode- exclude deep venous thrombosis
- rest
- gentle elevation if possible
- stop treatment - avoid compression, manual lymphatic drainage
- increase fluid intake
- wound swab if indicated
- antibiotic therapy

DLT is a therapist-led programme usually undertaken daily over a 2-4 week period and maintenance therapy is patient-driven, requiring daily self treatment. Those suitable for DLT include patients with swelling of >20 per cent excess limb volume which may be complicated by trunk, head and neck or genital swelling, distorted limb shape and/or persistent skin problems (British Lymphology Society, 1999a). Maintenance therapy is instigated following DLT, and is the treatment of choice for individuals with mild and uncomplicated swelling.

Patients with oedema relating to advanced cancer require a similar programme of treatment although treatment goals are centred on palliation and quality of life. All elements of treatment may be used although MLD and bandaging are often modified, and treatments may be less frequent. Lymphorrhea (leakage of lymph fluid through the skin), can be successfully controlled in most patients using a short course of bandaging.

There is a developing body of evidence evaluating the efficacy of DLT and elements of lymphoedema treatment (Sitzia & Sobrido, 1997; Badger et al., 2000, Ko et al., 1998, Williams et al., 2002). However, various methods are used in these studies to measure treatment outcome and findings are not always comparable.

Skin and preventative care
Increased skin sensitivities, dry skin and a tendency to infection, due to inflammatory processes within the compromised lymphatic system are common. Daily skin care including washing and application of a non-lanolin-based cream is necessary to enhance skin integrity (Williams & Venables, 1995). Patients are advised regarding self-care and preventative actions to minimise the risk of skin problems and infection (Table 3). Referral to a lymphoedema specialist practitioner or dermatologist is required if skin conditions are persistent.

Chronic inflammation occurs in all lymphoedemas although acute infection, often referred to as cellulitis or erysipelas, has been estimated to affect around one third of people with lymphoedema

(Mortimer, 2000). At the first signs of an acute attack, lymphoedema treatment should be stopped (Table 4). A two week course of oral antibiotic therapy is usually required in order to fully eradicate the infection from the oedematous tissues. The management of recurrent infection requires prophylactic antibiotics such as Penicillin V 500mg daily although some patients do not respond to antibiotic therapy (Cefai & Lund, 2003). The problem of recurrent infection in a patient must be addressed as it exacerbates swelling and leads to a poorly controlled oedema.

Manual lymphatic drainage (MLD)

The aim of MLD is to redirect the fluid towards the healthy lymphatics. This massage provides gentle but very specific pumping movements that stretch the skin and stimulate drainage in underlying lymphatics. Deeper movements are used on the abdomen to improve drainage in the deep vessels and abdominal breathing techniques are taught.

A study of women with breast cancer-related lymphoedema reported a statistically significant reduction in excess limb volume and improved quality of life following MLD (Williams et al., 2002). There have been no randomised, controlled studies of the specific effects of MLD in leg swelling.

In the UK most practitioners in MLD are qualified in the Vodder, Casley-Smith, Földi and Leduc methods of MLD which are all based on the same principles. Simple lymphatic drainage (SLD), a modified version of MLD is taught to patients to be used when MLD is not available and following the DLT programme (BLS, 1999b).

Compression/support therapy

Multi-layer bandaging is used within DLT to reduce swelling and improve shape. The bandaging system used in lymphoedema is applied to the whole leg and comprises of toe bandaging, a stockinette layer next to the skin, the use of padding in the form of foam or wadding and the application of layers of short stretch bandages such as Rosidal K™ or Comprilan™.

This system provides a rigid structure around the limb and pressure occurs when the muscle expands against this rigidity (Moffatt, 2000). When the limb is moved, high working and low resting pressures are achieved and the lymphatics in the skin and underlying tissues respond to these fluctuating pressures. As oedema reduces, the bandage slackens; daily reapplication provides opportunity for reassessment of limb size and shape. Padding is particularly important to give protection to the skin and ensure a uniform cylindrical shape is provided around a poorly shaped limb (Williams, 2003). This ensures that an evenly graduated pressure profile is achieved and pressures are reduced towards the root of the limb, allowing oedema to drain in a proximal direction.

A randomised, controlled trial showed that lymphoedema patients who underwent 18 days of bandaging followed by hosiery garments had a greater reduction in excess limb volume at 24 weeks than those who had hosiery alone (Badger et al., 2000). Experience has suggested that the 4-layer venous ulcer bandaging system may lead to problems when used in people with ulceration and lymphoedema as it may result in persistent oedema of the toes, knees and upper part of the leg which exacerbates the ulcer and prevents healing (Figures 2 & 3). Some components of the lymphoedema bandaging system, such as toe bandaging and additional padding, may be incorporated into the 4-layer system but further studies may be required to address these issues and develop new systems of care for patients with ulceration and gross oedema.

On completion of DLT, a rigid hosiery garment such as the flat-bed knit is required to maintain the tissue changes which have taken place during treatment. Elastic hosiery available on FP10 is generally not suitable for these patients and difficulties are commonly experienced in accessing the correct type of garment with the community. Standard off-the-shelf garments may be used, particularly in people with a mild, uncomplicated oedema but patients with a poorly shaped limb will require made-to-measure hosiery.

Exercise

The aim of exercise is to enhance lymph flow through improved muscle activity and joint mobility. Movement may be passive or active, and an individualised exercise plan should be developed, to reflect patient need and capability. Strategies to reduce weight and improve posture and gait are also useful and the latter can be greatly improved by the fitting and wearing of comfortable, supportive shoes, once a foot oedema has resolved.

Other treatments

Diuretics are not indicated in lymphoedema although they may be useful for patients with oedema in advanced cancer to alleviate discomfort due to severe tissue swelling, particularly if venous obstruction or cardiac failure is present. The use of intermittent compression pumps for leg oedema has been associated with the development of genital oedema (Boris et al., 1998). These may be indicated in a venous oedema or used within a DLT programme when MLD is also provided.

Resources

The level of provision of lymphoedema services varies throughout the UK and is undertaken predominantly by nurses and physiotherapists. Currently there are two levels of lymphoedema practitioners; the Keyworker and Specialist Practitioner. Keyworkers provide care for people with mild and uncomplicated lymphoedema, though in practice, often treat complicated oedemas if a specialist is not available in their area. Information on local services is available from the British Lymphology Society (BLS) Lymphoedema Service Directory, through the Lymphoedema Support Network, a patient-led support and campaigning organisation and from MLD UK.

Conclusion

Lymphoedema can be successfully treated and controlled. Many oedemas have a complex and mixed aetiology, however, and clinical experience suggests that long term control of the condition is compromised, particularly in the elderly, by factors such as obesity and immobility. A venous ulcer practitioner should manage patients with chronic oedema associated with venous disease and ulceration, with support/ advice from lymphoedema services. Aspects of lymphoedema therapy may be of benefit in chronic ulceration where oedema is a significant contributory factor.

The comprehensive lymphoedema treatment programme is not suitable for or tolerated by all patients with chronic swelling. Further research is required to develop and evaluate the efficacy and cost-effectiveness of a treatment programme and bandaging system for patients with chronic ulceration and/or chronic venous disease associated with lymphoedema.

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