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Lymphoedema

Definition

Lymphoedema is a form of chronic oedema caused either by damage to the lymphatic system (termed ‘secondary lymphoedema’) or congenital defects in the lymphatic system (termed ‘primary lymphoedema’) (Todd [272]). This section will focus only on cancer‐related lymphoedema.

Anatomy and physiology

The lymphatic system works closely with the cardiovascular system to maintain fluid balance within the body. The cardiovascular system transports nutrients and oxygen to the body's cells via blood vessels. As the blood flows through the vessels, nutrients and water pass into the spaces between the cells, known as the interstitial spaces, to form interstitial fluid (Partsch and Moffatt [219]).

The lymphatic system moves this interstitial fluid, now known as ‘lymph’, via a network of superficial and deep lymphatic vessels forming a one‐way drainage system towards the two main ducts, the thoracic duct and the right lymphatic duct, which empty lymph back into the venous system.

Lymph drainage commences with the superficial vessels, called initial lymphatics, which are found in the connective tissue spaces. Movement of lymph in the initial lymphatics is dependent upon muscle activity and changes in tissue pressure (Partsch and Moffatt [219]). The larger, deeper lymph vessels act as collecting vessels and contain smooth muscle and valves enabling them to contract and propel lymph in a unidirectional flow. Lymph nodes are situated in groups within the larger lymph vessels and act as filters to collect and destroy bacteria and viruses (Drake et al. [94]). See Figure 27.11 for a simplified diagram of the lymphatic system.

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Figure 27.11 Simplified diagram of the lymphatic system.

The balance between tissue fluid formation and reabsorption is dependent upon pressures across the capillary wall, known as Starling's forces. Any change can affect fluid levels in the tissues and result in the appearance of oedema (Partsch and Moffatt [219]).

A reduction in the drainage routes of the lymphatic system can occur following cancer‐related treatment in lymph node areas, and oedema can appear in the adjacent limb or truncal quadrant of the body.

In summary, the lymphatic system has the following main functions:

to regulate homeostasis by returning large molecules to the circulation and draining excess fluid from the interstitium
to dispose of unwanted cellular by‐products
to defend the body from infection by absorbing micro‐organisms and generating an autoimmune response when necessary.

Related theory

Oedema and lymphoedema may be caused by a wide range of conditions, both cancer and non‐cancer related (Williams [287]). When the lymphatic system fails due to damage, obstruction or congenital abnormality, fluid cannot be drained and builds up in the interstitial spaces causing lymphoedema (Nazarko [191], Partsch and Moffatt [219]). Other causes of peripheral oedema include venous, renal or hepatic disease, obesity, lipo‐oedema and some medications (Bianchi et al. [15]). In these cases, the lymphatic system has not failed and treatment may differ (Nazarko [191]). Dependency or gravitational oedema may develop in the immobile patient when failure to activate the muscle pump causes an increase in capillary filtration and reduced lymph drainage in the dependent limb (Milne [173]).

As described previously, there are two types of lymphoedema, primary or secondary, depending on aetiology. Primary lymphoedema occurs when there is an abnormality in the lymphatic system and may be present from birth (Nazarko [191]). Secondary lymphoedema arises from external factors affecting the function of the lymphatic system. This includes treatment for cancer involving the removal or irradiation of lymph node areas and other causes which include infection, venous disease, inflammation and trauma to the lymphatic channels or vessels (International Society of Lymphology [131]).

Lymphoedema is most commonly seen in patients with cancer because of damage to lymph nodes following surgery and/or radiotherapy, but it can also occur as a result of local tumour obstruction in lymph node areas. The reported incidence of lymphoedema following cancer treatment varies widely between studies. However, in breast cancer patients, the prevalence of chronic arm oedema is reported as being between 14.9% and 29.8%, average 20% or one in five patients (DiSipio et al. [83]), and in patients treated for gynaecological cancers it is between 28% and 47% (Lymphoedema Framework [153]). Lymphoedema can also develop after treatment for other malignancies: melanoma, sarcoma, genitourinary and head and neck cancers (Fu et al. [108]). Cancer‐related lymphoedema often develops within 5 years of treatment, however lymphoedema can develop at any time after treatment and patients face a lifetime risk of developing it (Fu et al. [108]).

Lymphoedema can affect any part of the body, including the face and head, but it most commonly affects a limb. The swelling can have physical, psychological and psychosocial implications for the patient and is associated with a number of complications arising from its development (Cooper [53]). Limb heaviness may lead to impaired function, reduced mobility and musculoskeletal problems (Woods [290]). Skin and tissue changes develop with increased lymph stasis in the oedematous limb and give rise to the characteristic deepened skin folds and skin thickening. Over time, complex skin conditions can occur (Todd [272]). There is an increased risk of local and systemic infection because of poor lymph drainage, and recurrent episodes of cellulitis are common (Cooper [53]).

Evidence‐based approaches

Rationale

Compression therapy in the management of lymphoedema involves the use of compression garments, low‐stretch bandages (Fu et al. [108]), and more recently the use of adjustable wrap compression systems (Damstra and Partsch [65]). Compression therapy initiates physiological effects within the arterial, venous, lymphatic and microcirculation (Partsch and Moffatt [219]).

Compression reduces oedema by (Partsch [221], Partsch and Moffatt [219]):

increasing lymphatic drainage from oedematous tissues to non‐oedematous tissues
reducing the formation of excess tissue fluid by reducing capillary filtration and decreasing lymphatic load
containing the tissues of the swollen limb and promoting the maintenance of a normal shape to the limb
increasing blood flow in the microcirculation, initiating a softening of fibrotic tissue and maintaining skin integrity
maximizing the effect of the muscle pump.

Compression therapy influences the principles of Starling's hypothesis by increasing local tissue pressure, counteracting capillary fluid filtration and enhancing lymph reabsorption (Partsch and Moffatt [219]).

The type of material from which the bandages or garments are made can determine the degree of pressure exerted on the tissues below (Partsch [221]), although the level of pressure achieved is also dependent upon a complex combination of other factors including the size and shape of the limb and the activity of the wearer (Partsch and Mortimer [220]).

The characteristics of compression garments, bandages and adjustable wrap compression systems have been summarized by the acronym PLaCE (Partsch and Moffatt [219], Partsch and Mortimer [220]) (see Box 27.7).

Box 27.7

PLaCE: characteristics of compression garments, bandages and adjustable wrap compression systems

Pressure. This is mainly determined by the manual force with which the bandage is applied, and is not determined by the bandage itself.
Layers. Bandages are applied with some overlap, therefore even one bandage will be multilayer. A single compression arm sleeve will be single layer.
Components. A bandaging application will be comprised of different components (e.g. liner and padding as well as the textile components of the bandage itself).
Elastic property/stiffness. Compression bandages and garments are categorized according to their elastic property, traditionally as elastic (long stretch) and inelastic (short stretch/low stretch). Elastic compression garments (also called round knit) are seam free, made from finer fabric and suitable for managing mild, uncomplicated swelling, where skin is intact and there is no limb distortion (Todd [273]). Inelastic bandages, garments or adjustable wrap systems produce a more ridged structure around the limb which does not yield. This produces high pressure peaks when muscles are contracted, but tolerable lower resting pressure. These are referred to as compression systems with a high static stiffness index (SSI). Compression with high SSI will not yield during exercise leading to a massaging effect and oedema reduction (Elwell [98], Partsch and Moffatt [219], Partsch and Mortimer [220]).

Indications and contraindications

Indications for the most suitable type of compression therapy will depend on the patient and the extent of their swelling and will be made after full assessment, see text on bandaging and compression garments.

Principles of care

Compression therapy in the management of lymphoedema can be approached in two phases: the intensive phase and the maintenance phase (Tidhar et al. [270]).

The intensive phase

The intensive phase of treatment is a short period of therapist‐led treatment, usually planned over a 2‐ to 3‐week period, in which specific aims are identified, discussed and agreed between the patient and the therapist. Depending upon the oedema present, however, the treatment may be planned over a longer or shorter period. During the intensive or reduction phase of treatment, short‐stretch, inelastic bandages are applied to the swollen limb each day and left in place for a period of 23 hours as part of a multilayer system to provide a semi‐rigid encasement to the limb (Muldoon [179]). Alternatively, other bandaging systems (generally comprising two layers – a foam layer and an adhesive top layer) are applied twice weekly (Moffatt et al. [177]). This aspect of treatment is combined with other elements of treatment including (International Lymphoedema Framework [130], Tidhar et al. [270]):

a skincare regimen to minimize the risks of infection and optimize skin condition
specific exercises to promote lymph drainage and maintain joint mobility
information, support and advice on self‐management
manual lymphatic drainage (MLD) administered by a specially trained therapist to stimulate lymph drainage by moving fluid to an area with functioning lymphatics.

The maintenance phase

During the maintenance phase, the concept of self‐care is promoted to encourage the patient to become independent in the long‐term management and control of their swelling. Compression garments suitable for the nature and extent of swelling are selected and there is now a wide range of styles depending on the individual needs and preferences of patients. Co‐morbidities, mobility and dexterity limitations will also have to be considered in garment selection (Elwell [99]). Compression garments are worn for a period each day and the therapist evaluates progress at regular intervals initially to ensure that the garments remain appropriate and that any problems can be identified at an early stage (Linnitt [146]). Once the swelling is stable and maximum reduction has been achieved, the patient is encouraged to commence long‐term control through self‐supported management. This aspect of treatment is also combined with other elements including:

a skincare regimen to minimize the risks of infection and optimize skin condition
specific exercises to promote lymph drainage and maintain joint mobility
simple lymphatic drainage (SLD), a simplified version of MLD taught to the patient by a therapist to stimulate normal draining lymphatics (Todd [272]).

Not all patients will follow both phases of treatment, and many may only need to follow the maintenance phase. The decision concerning the most appropriate phase of treatment for the patient should be made by a skilled therapist taking the patient's wishes into consideration.