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42 Aetiology
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Yuksel, A., Velioglu, Y., Cayir, M.C. etal. (2018). Current status of arterial
revascularization for the treatment of critical limb ischemia in infrain-
guinal atherosclerotic disease. International Journal of Angiology 27 (3):
132–137. Zematitis, M., Boll, J., and Dreyer, M. (2022). Peripheral arterial disease. In:
StatPearls. Treasure Island, FL: StatPearls Publishing https://www.
ncbi.nlm.nih.gov/books/NBK430745.
CHAPTER
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2
Lymphoedema and Chronic
Swelling
CAITRIONA O’NEILL AND RHODRI HARRIS
FUNCTION OFTHE LYMPHATIC SYSTEM
The lymphatic system is considered a complement of both the circu­latory and immune systems. It keeps uid levels balanced and pro­tects the body against infections. Often referred to in lay terms as the drainage pipe of the circulatory system, unlike the circulatory system (which is closed) the lymphatic system is a blind- ended system that is responsible for the uptake of lymphatic uid from the interstitial space arising from the blood vascular system. Lymphatic uid that lls the lymph vessels is pushed around the body by a combination of contractions of the smooth muscular walls of the blood vessels and exing and relaxing of the striated muscle in the body during move­ment (Urner etal.2018).
Like the arteries that send the blood ow out to the body and the veins that return it, the lymphatic system is a system- wide transport network. It has three main functions:
Facilitating tissue uid balance and recycling of uid and pro-
teins (Negrini and Moriondo2011; Bazigou and Makinen2013).
Filtering and production of lymphocytes (Margaris and
Black2012).
Lower Limb and Leg Ulcer Assessment and Management, First Edition. Edited by Aby Mitchell, Georgina Ritchie, and Alison Hopkins. © 2024 John Wiley & Sons Ltd. Published 2024 by John Wiley & Sons Ltd.
43
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Providing a mechanism for lipid absorption from the gut into the
circulatory system (Goswami etal.2020).
The initial lymphatics or lymphatic capillaries are microscopic vessels that form web- like networks in the interstitial spaces (spaces between body organs and tissues). These capillaries branch out within the tissues and interweave around the capillary beds to take in excess uid and particles that leak from blood capillaries into the tis­sue and return it to the circulatory system. Lymphatic capillaries have a single lining called an endothelium, making them permeable to absorb uid. They are made up of overlapping sections that pre­vent uid from leaking back out again (Goswami etal.2020). Lym­phatic capillaries are attached to surrounding tissues by anchoring laments, causing a pull on the initial lymphatics as well as the tis­sues, allowing uid into them (Negrini and Moriondo2011). The ini­tial lymphatics are activated to varying degrees by the mechanical forces of surrounding anatomy such as organs, muscles and vessels, which are further enhanced by external stimulation (Gordon and Morgan2007). For an overview of the key components of the anat­omy of the lymphatic system, see Table2.1 and Figures2.1 and2.2.
Lymphoedema is chronic swelling owing to failure or incompe­tence of the lymphatic system, which results in an imbalance between
TABLE 2.1 Anatomy ofthe lymphatic system.
Precollectors Connect the initial lymphatics to the collecting
lymphatics and are partially contractile and partially permeable like the lymphatic capillaries (Margaris and Black2012)
Collecting
lymphatics
Lymph nodes Small bean- shaped structures that lter substances that
Lymphatic trunks Where the smaller lymphatic ‘branches’ converge into
Made up of valve- segregated, smooth muscle cell–lined
lymphangions that syphon uid from one section to the next
travel through the lymphatic uid. They contain lymphocytes (white blood cells) that help ght infection and disease (Goswami etal.2020)
larger lymphatic vessels that drain larger regions. Lymphatic trunks merge until the lymph enters the two lymphatic ducts
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TABLE2.1 (Continued)
Lymphatic ducts The right duct drains lymph from the upper right
quadrant of the body and the remainder of the body drains to the cisterna chyli, which then transports lymph back into the venous system via the thoracic duct. These ducts act as highways to the major venous junctions between both the left and right internal jugular and subclavian veins (Goswami etal.2020)
Lymphocytes White blood cells play a major role in immunity, ghting
pathogens. These are made in the primary lymphatic organs– the thymus and bone marrow– and develop further in the secondary lymphatic organs, including the spleen, Peyer’s patches, appendix, tonsils and lymph nodes (Margaris and Black2012)
Lymphatic uid Known as ‘lymph’, this is produced from the ltration of
the blood in the arteries and leaks from the capillaries to soak the cells of interstitial tissue. As excess uid drains from cells and tissues throughout the body, lymphatic uid collects waste products, toxins and abnormal cells (Urner etal.2018). These include lipids and vitamins from the digestive tract and molecules such as large protein molecules and cellular debris. The lymphatic system is responsible for transporting 100% of the lymphatic uid from the interstitial space back into the bloodstream. The uid is then transported alongside the waste products to the lymph nodes, the ‘ltering stations’ in which lymphocytes destroy bacteria and viruses (Levick and Michel2010)
Only 10% of the lymph uid was understood to be
returned through the lymphatic system and most was reabsorbed by the venous system. This was based on the Starling principle. Recent evidence and revision of the Starling principle have provided a greater understanding of lymph uid transport and a change in understanding that 100% of the lymphatic uid is reabsorbed at the peripheral end from the interstitial tissue. This change in the theory is based mainly on the endothelial glycocalyx, which lines the capillary wall. These structures are similar to ne hair on the inner capillary wall, which creates an exclusion whereby uid cannot go back to the venous side of the system as the glycocalyx is opposing the reabsorption (Michel etal.2020; Woodcock and Woodcock2012)
46 Lymphoedema and ChroniC SweLLing
T
Thoracic duct
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CNS
Tonsils
Lymph vessels
Lymph
nodes
Thymus
Heart
Spleen
GAL
Bone
FIGURE 2.1 Lymphatics in humans. The system consists of serially
connected networks of vessels and lymph nodes, and lymphoid organs, such as the thymus, bone marrow, spleen, gut- associated lymphoid tissue (GALT), lymph nodes and tonsils. Lymphatics play essential roles in maintaining tissue uid homoeostasis and immune surveillance and responses. CNS (central nervous system). Source: Al- Koha etal. (2017). Reproduced with permission.
Anchorin filaments
Lymph node
Collectin lymphatic vesse
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Lymphoedema and Chronic Swelling 47
(a)
g
Extracellular matrix
g
Afferent vessel
Lymphatic endothelial cells monolayer
Lymphatic muscle cells
Lymphangion
l
Valve
Pre-collector lymphatic vessel
Efferent vessel
Basement membrane
Initial lymphatic capillaries
(b)
Lymphatic duct
Subclavian vein
Lymphangion
Fluid movement Macromolecules and fluid Immune cells External pressure LMC contractility
FIGURE 2.2 Lymphatic vessels. (a) Initial lymphatic capillaries are
blind-
ended vessels comprising a single layer of lymphatic endothelial cells,
ounded by a minimal basement membrane without muscular
surr investment. The basal aspect of initial lymphatics are linked to the surrounding extracellular matrix by a series of anchoring laments. Pre- collecting lymphatics transfer lymph collected in initial lymphatic capillaries into collecting lymphatics. The collecting lymphatics act as connections between initial lymphatics as well as the lymph nodes, and between lymph nodes and the blood vascular circulation. The walls of collecting lymphatics are infused with layers of smooth muscle cells and unidirectional lymphatic valves in the lumen of collecting lymphatics. (b)Lymphangions are segmented chambers located between two valves that are functional propulsive units within lymphatic collecting vessels that often show an ability to contract phasically (as well as tonically). Source: Al- Koha etal. (2017). Reproduced with permission.
capillary ltration and lymphatic drainage from the interstitial space. It is essentially a problem of ‘low output’ by the lymphovascular sys­tem due to a failure in lymphatic transport (International Society of Lymphology2013, p.52). The result is an accumulation of lymphatic
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uid that cannot drain and leads to chronic swelling. It is most com­mon in the lower or upper limbs, although other areas including the head, neck, breast and genitalia can also be aected. It can occur in persons of any age.
PREVALENCE ANDKEY FACTS
Lymphoedema has a signicant impact on the population, society, and on the wider health and social care system. England currently spends more than £178million on admissions due to lymphoedema (National Lymphoedema Partnership2019). It is a common problem that can cause a serious impact on quality of life with a risk of hospi­talisation. It is important to understand the size of the problem and varying proles within a population to appreciate how the lives of people living with lower limb swelling will be restricted, including activities of daily living and psychosocial impacts. It is also necessary to understand the challenges for health services and health profes­sionals responsible for caring for people with lymphoedema.
Lymphoedema is an underestimated health problem and remains widely unrecognised (NLP2019, p.6). Moatt etal. (2017) estimated the prevalence in the United Kingdom to be close to 3.93 per 1000 population, while Thomas and Morgan (2017) found the prevalence in Wales to be as high as 6.4 per 1000 population. The incidence is higher in women (Moatt et al. 2019, p. 151) and increases with age (NLP2019, p.8). People with certain underlying conditions or medical or surgical histories have a higher risk of lym­phoedema. Inadequate management of people with lymphoedema of the leg greatly increases the risk of lower limb cellulitis, a serious sequela often requiring hospital admission, which adds greatly to the cost of health service provision. In 2011–2012, there were an esti­mated 55 000 hospital admissions for lower limb cellulitis in Eng­land, with an average length of stay of 10 days (Atkin 2016). In a study of patients admitted to acute services for cellulitis, lymphoe­dema was a signicant factor, with an average length of stay of 6.06 bed days (for cellulitis of limbs only) (Moatt etal.2017).
Many people are living in the community with undiagnosed lymphoedema or with unrecognised early signs of lymphoedema. Primary care and community nurses are often in a position to
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identify signs, particularly early signs. While a diagnosis requires the involvement of specialists, primary care can play an important part in recognition, referral and management after diagnosis (National Lymphoedema Partnership2019). The prevalence of lymphoedema can be characterised as follows:
6 per 1000 across a population will have chronic swelling.20% of patients following breast cancer will develop
lymphoedema.
30% of all patients will have had an episode of cellulitis prior to
diagnosis.
20–50% of those with deep vein thrombosis (DVT) will require
compression due to post- thrombotic symptoms.
Older age signicantly increases the prevalence (Appelen etal.2017;
National Lymphoedema Partnership2019; Moatt etal.2017).
When describing lymphoedema it is dened within two catego-
ries (see Table2.2):
Primary lymphoedemaSecondary lymphoedema
The majority of lymphoedema presentations that are seen within a community setting will sit within the secondary component and may have both elements of damage to the system and overload to the system. This creates a mixed picture:
Scenario 1: Signicant venous insuciency and immobility
(overload), but also DVT in one leg (damage).
Scenario 2: Surgery to the lower limb, e.g. coronary artery bypass
graft (damage) and renal impairment, decreasing mobility (overload).
Scenario 3: Surgery related to cancer requiring groin node dissec-
tion (damage), also leading to very poor mobility and not getting to bed overnight or maintaining a full night’s rest in bed (overload).
While lymphoedema is dened as primary and secondary lym­phoedema for simplicity, it can also be sub- divided into obstructive and non- obstructive lymphoedema (Table2.3).
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TABLE 2.2 Types oflymphoedema.
What has Type of lymphoedema
happened to
the system?
When does it appear? What occurs?
Primary
lymphoedema
Secondary
lymphoedema/ chronic oedema
An intrinsic defect
of the system whereby the anatomy is aected by the vessels being too small, too large or absent. There may be a genetic link for some
Damage or
overload to the lymphatic system due to an external cause such as cancer treatment, trauma, venous disease, wounds or other causes
At birth, but
commonly in adolescence or in the early years of adulthood
At any age
where there has been damage or overload
Failure of the
lymphatic system to function due to a defect whereby the vessels are too small, too large or absent and the system becomes overwhelmed
Failure of the
lymphatic system to function due to damage or overload
TABLE 2.3 Obstructive andnon- obstructive causes oflymphoedema.
Obstructive
cause
Non- obstructive
causes
Source: Adapted from Lymphoedema Framework, 2019: 31.
Damage The consequence of damage or
Overload Causes include those that are the
intrinsic defect to lymphatic vessels or lymph nodes, or lymph node removal
result of immobility, venous incompetence, lymphovenous stasis or hypoproteinaemia
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‘Chronic oedema’ is often used interchangeably with the term ‘lymphoedema’. Oedema results from an imbalance between capil­lary ltration and lymphatic drainage from the interstitial space. Although the term ‘lymphoedema’ suggests that the oedema is caused by a lymphatic abnormality, in every case of chronic oedema there will be some impairment of lymphatic drainage, either through an underlying abnormality (primary or secondary) or through lym­phatic failure as a result of the capacity of the lymphatics being over­loaded or damaged. The degree to which the lymphatics are or become aected may inuence the clinical presentation of oedema, the subcutaneous tissues and skin. Where there is an impairment of lymphatic drainage, over time the uid component of oedema may become replaced by brosis and/or adipose tissue (National Lym­phoedema Partnership2015).
Fibrosis is the thickening, hardening or scarring of tissues in the body and is part of the body’s natural healing process. It is the forma­tion of a thickened collagen bundle and sclerosis of skin lymphatic vessels (Kataru etal. 2019) and may be a result of surgery or other medical treatments such as radiation therapy, or have other causes such as injury, infection or inammation. Unlike supercial wounds, surgical wound scars tend to run deeper and can extend from the skin to bones and organs. These scars can become hard and inexi­ble, obstructing lymphatic circulation, which contributes to a form of brosis that is related to lymphoedema: lymphostatic brosis. Fibro­sis occurs in both primary and secondary lymphoedema in most areas of the body and is more frequently seen in long­phoedema. Fibrotic tissues feel very dense to the touch, are non­pliable and increase the thickness of the skin.
Treating surgical brosis can aect the process of scar harden­ing, which lessens lymphatic obstruction. Treating lymphoedema lessens lymph stasis, which lowers the development of brosclerotic brosis (Azhar et al. 2020). In the case of prolonged swelling, an inammatory process is occurring that is representative of the lym­phatic uid being chronically congested, and after a while this attracts fat cells that bind to the surrounding tissues. Over time the tissues become rmer as the lymphostatic brosis process begins, leading to rm tissue.
standing lym-
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