An overview of non-cancer related chronic oedema - a UK perspective

Keywords: non-cancer related chronic oedema; lymphoedema; pathophysiology; treatment strategies; compression therapy; resources.

Abstract

This paper focuses on non-cancer-related chronic oedema. The pathophysiology underlying the development of chronic oedema may be complex and a number of factors may predispose an individual to develop swelling. The charactistics, extent and problems associated with chronic oedema are discussed along with the various management stategies, which can be tailored to an individual patient's needs.

Introduction

The term lymphoedema is commonly used to refer to tissue swelling associated with primary lymphatic insufficiency or secondary damage to the lymphatic system (e.g. following cancer treatment). In most cases, the cause of oedema will have a lymphatic and venous component [1], but chronic swelling often develops as a result of venous disease and ulceration, trauma, infection, arterial or orthopaedic surgery. Consequently the phrase chronic oedema more accurately reflects the broader aetiology of this problem.

Chronic oedema is defined as swelling of greater than 3 months' duration, which does not resolve on elevation. Physical management of chronic oedema combines skin and preventative care with manual lymphatic drainage (MLD) massage, isotonic exercises and support/compression therapy using hosiery garments and compression bandages. Treatment should aim to reduce swelling, restore a natural limb shape, improve skin and tissue condition and enhance quality of life.

The lymphatic system and the formation of chronic oedema

The lymphatic system plays an important role in maintaining homeostasis, providing a 'safety valve' mechanism whereby interstitial fluid can be returned to the blood circulation. Around 700 lymph nodes are present in the body, predominantly in areas such as the neck, axilla, inguinal and abdominal areas. They form part of an extensive lymphatic system, incorporating a network of initial lymphatics that cover the skin and most internal organs. These thin-walled vessels collect fluid, proteins and various waste products from the interstitium and return them into the venous circulation via a series of lymphatic pre-collector and collector vessels, lymph nodes and larger lymphatics, such as the right and left lymphatic ducts and thoracic duct.

The forces controlling interstitial fluid volume are represented by Starling's principle[2] (Figure 1). The movement of fluid between blood capillaries and interstitial tissues is influenced by filtration and reabsorption forces, and the lymphatic system drains excess fluid from the interstitial area. Lymphatic insufficiency and changes affecting Starling's principle, therefore, have the potential to cause oedema.

In primary or secondary lymphoedema, damage or abnormalities in the lymphatic system lead to reduced lymphatic transport. Increased filtration due to cardiac failure, increased venous pressure, and increased capillary permeability due to injury, ulceration or inflammation will also result in excess fluid accumulating in the interstitial tissues. In addition, the compensatory mechanism of the lymphatic system may fail due to local damage, causing swelling. In patients with renal disease or malignant ascites, an abnormal loss of plasma proteins (hypoproteinaemia) can occur and lead to an increased interstitial fluid volume due to the decreased plasma colloid osmotic pressure.

Movement of lymph through the lymphatic system depends on a number of factors including the contraction of lymph vessels and changes in intra-thoracic and intra-abdominal pressures related to breathing. Lymphatics have a minimal capacity to contract and rely on the pulsation of adjacent blood vessels and the ability of the muscle pump to stimulate lymph flow. Reduced mobility and limb dependency will therefore further predispose an individual to oedema.

Characteristics of chronic oedema

Chronic oedema usually presents as tissue swelling most commonly developing in one or more limb(s), but often affecting adjacent areas such as the trunk, breast, head and neck or genitalia. Once fluid starts to accumulate in the tissues, a number of other changes take place. The epidermis becomes stretched, there is a proliferation of fibroblasts and collagen fibres in the local tissues, dilation of local lymphatics and increased production of inflammatory agents. In the early stages the oedema is usually 'pitting' and reduces on elevation. In time, as fluid and waste products accumulate, the tissues become hard, fibrosed, 'non-pitting' and do not respond to elevation. Skin and tissue changes occur and, as the lymphatic system has an important immunological function, an increased risk of bacterial and fungal infection is common (Box 1). Stemmer's sign (inability to pick up a fold of skin at the base of the second toe, due to thickening of the tissues/fibrosis) is a diagnostic sign for lymphoedema (Figure 2).

The extent of the problem

Primary lymphoedema is associated with an intrinsic underlying abnormality of the lymphatic system, most commonly reduction, obliteration or incompetence of lymphatic vessels and lymph nodes. This may be present from birth (lymphoedema congenita), develop between the ages of 2-35 (lymphoedema praecox) or after 35 years (lymphoedema tarda). Congenital primary lymphoedema is often referred to as Milroy's disease.

There are no reliable figures giving the prevalence of primary lymphoedema in the population. However, a UK-based survey carried out by the British Lymphology Society reported that 8% of 603 new referrals to 27 lymphoedema clinics over a three-month period had primary lymphoedema [3]. This is, however, likely to represent the 'tip of the iceberg' as the study also highlighted that 65% of those with primary lymphoedema waited more than five years for referral, suggesting that many go undiagnosed or misdiagnosed for long periods.

Secondary lymphoedema, developing as a result of damage or secondary changes to the lymphatic system, may be associated with a range of causes such as cancer treatment, trauma or infection. Most literature to date has focused on cancer-related swelling and information on numbers with non-cancer swelling is limited. Research in the UK has examined prevalence rates of various types of chronic oedema and early findings suggest that significant numbers may be affected, particularly in the elderly population [4].

Secondary oedema resulting from non-cancer-related causes is complex. One study investigated the causes of bilateral leg oedema in a primary care setting in the USA [5]. On clinical examination, prior to investigation, 71% of the 45 patients in this study were diagnosed with venous insufficiency while 18% were diagnosed as having congestive cardiac failure. On further investigation with ECG and Duplex scanning of the legs, the actual diagnoses were found to be cardiac disease (33%), pulmonary hypertension (42%) and venous insufficiency (22%). Other causes of leg swelling include medication, nephrotic syndrome and hypoproteinaemia. The researchers concluded that leg swelling, particularly in those over 45 years old, requires further investigation as the underlying pathology may not be clear on clinical examination.

An audit of non-cancer-related lymphoedema [6] identified oedemas associated with venous disease, trauma, infection, joint disease and orthopaedic surgery. It was noted that extensive, persistent ulceration and recurrent infections led to local lymphatic damage while inflammatory conditions further compromised an already damaged lymphatic system. A further study has reported oedema in 55% of patients with ulcerated legs and concluded that oedema may be a significant factor in ulcers which are slow to heal [7].

Problems associated with chronic oedema

Each individual with chronic oedema will experience different problems and the true impact of oedema on any one person cannot be predicted. The majority of research studies have focused on women with breast cancer-related lymphoedema of the upper limb. These indicate that increased psychological distress, anxiety, depression, altered body image, reduced confidence, difficulties with clothes and altered sensations are common [8],[9]. Individuals with non-cancer-related swelling appear to experience similar problems and patients with lower limb swelling may have particular difficulties with footwear. Patients with foot and leg swelling are often forced to resort to unsuitable, loose fitting shoes or slippers that compound their reduced mobility and, as a result, may exacerbate a dependency oedema. Skin and tissue changes in leg oedema can be severe and problems such as hyperkeratosis, lymphangioma, papillomatosis and lymphorrhoea (leakage of lymph from the skin) [10] are unsightly, distressing and further compromise skin integrity.

The management of chronic oedema

The British Lymphology Society (BLS) has produced a document that outlines four different groups within the chronic oedema population [11] (Table 1). The purpose of this document is to enable practitioners to determine the types of treatment and care required for different individuals. A 'Framework for Education', determining the educational needs of practitioners managing patients in these different groups, has also been published (see www.thebls.com).

Table 1 illustrates the specific criteria for each group and details of the treatment required. It is important to note that individuals may move between groups. For example, excess limb volume may decrease and skin fibrosis or trunk oedema resolve following treatment. This patient would then move from group 3 to group 2, the individual would no longer require specialist treatment and could be managed by a keyworker, often a community-based nurse or physiotherapist.

Table 1: Chronic oedema population and need. Source: British Lymphology Society, September 2001

Group 1: people 'at risk'	No clinical signs of oedema One or more risk factors, e.g. lymphatic abormalities, cancer treatment, chronic venous insufficiency, immobility, trauma to lymphatics	Education, information and advice Prompt referral to appropriate practitioner if required	All healthcare practitioners
Group 2: people with mild and uncomplicated oedema	Oedema with excess limb volume <20% No trunk, head or genital swelling Healthy skin Tissues soft and pitting No arterial insufficiency or malignancy	Education, information and advice Daily self maintenance programme which may include skin care, exercise, simple lymphatic drainage, hosiery garments Long-term monitoring and prompt referral to appropriate specialist practitioner if required	Practitioners liaise with keyworker who may be a nurse, physiotherapist, occupational therapist or other with appropriate education in all aspects of the maintenance programme
Group 3: people with moderate to severe, complicated oedema	Oedema with excess limb volume of >20% with one or more of the following: Trunk, head, genital oedema Distorted limb shape Skin problems Evidence of active but controlled malignancy, arterial and/or venous insufficiency, current acute inflammatory episode, lymphorrhoea	Education, information and advice Treatment plan which may include multi-layer lymphoedema bandaging, manual lymphatic drainage, isotonic exercises, skin care, hosiery garments Referral to other members of the healthcare team Transfer to maintenance programme as required	Specialist lymphoedema practitioner with appropriate education in specialist skills such as mutli-layer bandaging, manual lymphatic drainage, exercise
Group 4: people with oedema and advanced disease	Oedema associated with advanced disease Common symptoms are trunk and/or midline oedema, lymphorrhoea, tension in the tissues, impaired mobility and function, pain, infection	Education, information and advice Emphasis on optimising quality of life Daily treatment programme which may include skin care, support and positioning of the limb, appropriate exercises, simple lymphatic drainage Specialist interventions such as manual lymphatic drainage, modified multi-layer bandaging, hosiery garments, appliance to aid mobility and function	Practitioner with appropriate education in specialist skills and palliative care

Access to treatment and care

In the UK, specialist lymphoedema care is available from various centres, often based in oncology or palliative care units or through a small number of independent practitioners. The level of access to specialist treatment and care varies enormously throughout the country. Achieving the goal of seamless care and co-ordination of services through the specialist and keyworker network identified by BLS has raised many challenges and various models of care provision are currently being examined. Other European countries, such as Austria, Germany and Belgium have, for several decades, provided specialised treatment for people with chronic oedema through their health systems. A number of specialist centres, such as the Földi Clinic in Germany and the Wittlinger therapy centre in Austria, provide inpatient and outpatient treatment and it is common for patients to visit independent therapists for regular, on-going treatment, such as manual lymphatic drainage. A similar picture is seen in countries such as the USA and Australia although, again, access to treatment and care varies and is influenced by financial constraints.

Assessment and evaluation of clinical effectiveness

A detailed baseline assessment is essential. This should include circumferential measurements of the limb, which are used to calculate the volume of the total limb, proximal and distal limb segments, and excess and percentage excess volumes of an affected limb in relation to the unaffected limb. The calculation of limb volume, based on the formula for the volume of a cylinder, enables the degree of swelling to be quantified and shape distortion identified and provides the practitioner with a method to evaluate the effectiveness of treatment. In a bilateral swelling the volume of each limb may be calculated although, as yet, there is no valid and reliable way of estimating the severity of swelling where both limbs are swollen. Recently, a quality of life tool for upper and lower limb swelling has been developed and validated. This will provide future insights into the experience of chronic oedema and the impact of treatment on quality of life [12].

Treatment strategies

Physical treatment of chronic oedema comprises two phases:

• Intensive, decongestive phase (often referred to as Decongestive Lymphatic Therapy (DLT) or Complex Decongestive Therapy (CDT)

• Maintenance phase [13], [14].

Many patients, for example those in group 2 (see Table 1), do not require the intensive treatment programme. The success of the maintenance phase depends on the commitment of the patient to actively participate in their treatment.

A course of treatment may take 2-4 weeks depending on the severity of the condition. It is not uncommon for treatment to extend over time and be repeated at intervals until the goals have been achieved.

The aim of the first phase is to reduce swelling, restore a natural limb shape, improve skin and tissue condition and enhance quality of life. This is commonly achieved through the application of daily treatment consisting of skin care, manual lymphatic drainage (MLD), multi-layer lymphoedema bandaging and isotonic exercises.

Skin and preventative care

People with chronic oedema are encouraged to take meticulous care of their skin, washing and moisturising on a daily basis. The main aim is to prevent skin problems that may compromise the protective function of the skin and predispose the individual to infection. Oedematous skin is often dry and scaly; skin sensitivities and dermatitis are common and may result in further damage to local lymphatics. The need for regular use of emollients and lanolin-free creams is emphasised. Patients are also advised on other preventive care strategies such as reducing the risk of skin abrasions, cuts, insect bites and burns in and near the swollen limb. Hair removal using a wet razor should be avoided and individuals should reduce or avoid activities such as sunbathing, hot baths, air travel, venepuncture or acupuncture in the affected limb which may exacerbate the swelling. For some patients lifestyle changes may affect their ability to work and have a significant impact on family life.

Acute inflammatory episodes, sometimes referred to as 'cellulitis' are relatively common in people with chronic oedema and should be promptly treated with antibiotics, especially where systemic symptoms are present. The use of phenoxymethyl penicillin (Penicillin V) or co-amoxiclav (Augmentin) is recommended over a 14-day course and may be substituted by erythromycin if the patient is allergic to penicillin [15]. Some patients experience recurrent infection particularly if the acute infection is not adequately treated. A persistent sub-acute or recurrent infection will lead to poor treatment results and a poorly controlled oedema. In this instance, the use of prophylactic antibiotics such as phenoxymethyl penicillin (Penicillin V) is justified and may be required for up to 1 year or beyond. Topical antibiotics are not recommended and patients with persistent infection or skin changes should be referred to a specialist lymphoedema practitioner.

Support and compression

The main aims of support and compression therapy in chronic oedema are to reduce swelling and maintain limb size and normal shape by preventing oedema from re-accumulating in the tissues and enhancing lymph and venous flow in the limb. The bandaging system used in chronic oedema differs from that used to treat venous disease and ulceration in the following ways:

• It is common for the whole limb to be bandaged, including the digits, as they may be particularly prone to swelling

• Padding, either in the form of wadding or different types of foam is used in specific ways to improve limb shape and provide a graduated sub-bandage pressure profile that encourages movement of fluid toward the root of the limb. For example, if there are deep folds around the ankle, it is important to pad out any indentations to provide a cylindrical shape along the length of the limb. In addition, foam may be used to protect vulnerable areas such as the Achilles tendon; a denser type of foam is used to provide pressure in specific areas such as around the malleolus to improve drainage; small chips or various shapes of denser foam can also be made into pads, which are applied under the wadding, and will provide a massaging effect that helps to reduce fibrosis

• The multi-layer system utilises short-stretch (inelastic) bandages that have low-extensibility and provide rigidity and a greater variation between working and resting pressures - features that enhance the function of the lymphatics

• The bandage system is usually renewed daily follwing MLD treatment to accommodate the changing shape caused by oedema displacement.

Although not all patients will require bandaging, there is evidence that a better outcome is achieved when bandages are used prior to the fitting of hosiery garments [16].

Hosiery garments are a long-term management strategy and for many people with chronic oedema, provide the means to controlling the oedema on a day-to-day basis. Elastic garments intended for venous problems are suitable for some individuals with chronic oedema although most need a full limb garment; many require the flatbed knit garments, which provide greater rigidity. In the UK, many patients are fitted with standard, off-the-shelf garments. However, custom-made garments are increasingly being used following intensive treatment or if the limb is poorly shaped. Any form of support or compression therapy is contraindicated if there is an acute infection or arterial insufficiency or when there is a recent history of thrombosis. Doppler studies are recommended although there are questions over the reliability of results where the limb is grossly oedematous and pulse oximetry may offer a better alternative.

Manual lymphatic drainage (MLD) and self lymphatic drainage (SLD)

MLD is a type of massage, developed in the 1930s by Dr Emil Vodder, a physical therapist. It has been adapted by others who recognised its importance in chronic oedema management [13],[17],[18]. The aim of MLD is to utilise skin lymphatic drainage routes to redirect fluid away from oedematous areas, towards healthy lymphatics. It is a gentle type of massage which uses light pressures and very specific movements to stimulate lymph flow and open up the collateral lymphatics. In the UK it is common for practitioners to teach patients a simplified form of MLD, called simple lymphatic drainage (SLD). This enables patients to continue their care independently and is useful in maintaining the patency of collateral lymphatics following the intensive course of DLT.

Exercise

The main aim of exercise in chronic oedema is to maximise mobility and flexibility to enhance lymph and venous flow. Physiotherapy and occupational therapy referrals may be required. Exercise programmes should be individualised and designed to fit in with the needs and lifestyle of the patient. Walking, swimming and cycling can be useful for those who are fit and movements can be modified for individuals who are frail or have limited mobility.

Conclusion

Chronic oedema is a complex condition, providing many challenges for patients and healthcare professionals. Effective control of the swelling and associated problems, however, can be achieved. Practitioners require specific expertise in order to develop, implement and evaluate a treatment plan based on the individual's needs and, where possible, patients should be referred to a lymphoedema practitioner for assessment and appropriate treatment.

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