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42 Aetiology
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Yuksel, A., Velioglu, Y., Cayir, M.C. etal. (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 OFTHE 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 etal.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 Moriondo2011; Bazigou and Makinen2013).
Filtering and production of lymphocytes (Margaris and
Black2012).
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
44 Lymphoedema and ChroniC SweLLing
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Providing a mechanism for lipid absorption from the gut into the
circulatory system (Goswami etal.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 etal.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 Moriondo2011). 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
Morgan2007). For an overview of the key components of the anatomy of the lymphatic system, see Table2.1 and Figures2.1 and2.2.
Lymphoedema is chronic swelling owing to failure or incompetence of the lymphatic system, which results in an imbalance between
TABLE 2.1 Anatomy ofthe lymphatic system.
Precollectors Connect the initial lymphatics to the collecting
lymphatics and are partially contractile and partially
permeable like the lymphatic capillaries (Margaris
and Black2012)
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 etal.2020)
larger lymphatic vessels that drain larger regions.
Lymphatic trunks merge until the lymph enters the
two lymphatic ducts
Lymphoedema and Chronic Swelling 45
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TABLE2.1 (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 etal.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 Black2012)
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 etal.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 Michel2010)
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 etal.2020; Woodcock and Woodcock2012)
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 etal. (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 etal. (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
Lymphology2013, p.52). The result is an accumulation of lymphatic
48 Lymphoedema and ChroniC SweLLing
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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 aected. It can occur in
persons of any age.
PREVALENCE ANDKEY FACTS
Lymphoedema has a signicant impact on the population, society,
and on the wider health and social care system. England currently
spends more than £178million on admissions due to lymphoedema
(National Lymphoedema Partnership2019). 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 proles 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 (NLP2019, p.6). Moatt etal. (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 (Moatt et al. 2019, p. 151) and
increases with age (NLP2019, 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 signicant factor, with an average length of stay of 6.06
bed days (for cellulitis of limbs only) (Moatt etal.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
Lymphoedema and Chronic Swelling 49
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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 Partnership2019). 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 signicantly increases the prevalence (Appelen etal.2017;
National Lymphoedema Partnership2019; Moatt etal.2017).
When describing lymphoedema it is dened within two catego-
ries (see Table2.2):
Primary lymphoedema
Secondary 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: Signicant venous insuciency 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 dened as primary and secondary lymphoedema for simplicity, it can also be sub- divided into obstructive
and non- obstructive lymphoedema (Table2.3).
50 Lymphoedema and ChroniC SweLLing
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TABLE 2.2 Types oflymphoedema.
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
aected 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 andnon- obstructive causes oflymphoedema.
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 aected may inuence 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 Partnership2015).
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 etal. 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 inammation. Unlike supercial wounds,
surgical wound scars tend to run deeper and can extend from the
skin to bones and organs. These scars can become hard and inexible, 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 aect 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
inammatory 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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