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52 Lymphoedema and ChroniC SweLLing
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CAUSES OFAND RISK FACTORS
Lower limb lymphoedema – associated with several forms of
cancer, including gynaecological malignancies such as cervical,
endometrial, ovarian and vulvar cancers in women (Dessources
etal.2020), prostate and penile cancers in men and melanoma of
the lower limbs (Paskett etal.2012; National Cancer Control Programme (Ireland)2015).
Lymphoedema of the arm– associated with breast cancers that
require surgical removal of the underarm (axillary) lymph nodes,
and generally involves chemotherapy and radiotherapy (Bromham etal.2017, pp.2–3). More than 20% of women who survive
breast cancer are estimated to develop arm lymphoedema, with a
higher risk in women who need extensive surgery and are overweight or obese (DiSipio etal.2013).
Wounds– the presence of a wound is the greatest independent
risk factor for lymphoedema, while obesity and heart failure are
also important independent risk factors (Moatt etal.2019).
Cellulitis– lymphoedema is associated with a history of celluli-
tis, and the presence of lymphoedema substantially increases
the risk of cellulitis (Mortimer and Rockson2014, p.919). One
study of lymphoedema patients in southwest London found
that 29% of patients experienced at least one episode of cellulitis
over the period of one year, with 17% requiring hospitalisation
(Moatt et al.2003). A more recent Canadian study reported
that 72.06% of patients attending a wound management clinic
had suered from cellulitis (Keast et al.2019). A cost burden
of £178 million is aligned to acute hospital admissions for
lymphoedema- related cellulitis (National Lymphoedema
Partnership2019).
Risk factors that increase prevalence – cancer, increased age,
obesity, heart failure and neurological defects, particularly those
impacting mobility (Quéré etal.2019) (Table2.4).
Other risk factors– trauma such as burns, orthopaedic trauma or
surgery, abdominal surgery and long- standing skin disorders;
damage to the venous system such as varicose veins, DVT, varicose
vein stripping and chronic venous insuciency, which may
reduce lymphatic motility; and immobility, which reduces muscular function of the venous and lymphatic systems (NLP2019, p.9).
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TABLE 2.4 Risk factors forlymphoedema.
Risk factor Impact on system Cause
Obesity overload of
the system
Decreased mobility
including paralysis
Age Can occur at any time,
Cancer Cancer treatment, i.e.
Wounds Disruption of skin integrity Damage
Recurrent cellulitis Inammatory response to the
Venous
thromboembolism/
thrombosis
Chronic venous
insuciency/
venous ulceration/
venous surgeries
Dermatitis/eczema Disruption of skin integrity Damage
Scarring Disruption of skin integrity
Trauma May aect the mechanics of
Hereditary Malformation of the
Increased abdominal girth
causes pressure on the
lymphatic vessels in the
groin, reducing lymphatic
and venous return
Mobility is required for
venous and
lymphatic return
however the risk increases
with age and is linked to an
increase of co- morbidities
and decreased mobility
radiotherapy or surgery, or
obstruction from tumour
skin and subcutaneous
tissue
Obstruction in the
venous system
Reduced transit through the
venous and lymphatic
system; skin integrity may
be aecting the mechanics
and subcutaneous tissue
the lymphatic system,
including, but not exclusive
to, burns, orthopaedic
trauma or surgery,
abdominal surgery
lymphatics
Overload
Overload
Overload
Damage
Damage and/
or overload
Damage and/
or overload
Overload
and/or
damage
Damage
Damage
Intrinsic defect
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The main causes of swelling will often be a combination of several factors (Tables2.5 and2.6). Reviewing the groups that sit within
the higher- risk categories for swelling highlights that these high- risk
groups would be prevalent within a community nursing or primary
care environment.
TABLE 2.5
Overload (non- obstructive) Damage (obstructive)
Dependency Malformation of the
Venous disease Tumour obstructing lymph vessels/nodes
Paralysis Surgery
Cardiac failure Radiotherapy
Renal failure Metastatic disease
Obesity Deep vein thrombosis
Underlying causes ofswelling.
lymphatic system
Filariasis
TABLE 2.6 Common factors that will inuence andcontribute
tothe oedema.
Factors to consider Why?
Low serum albumin Without enough albumin, the body cannot keep
uid from leaking out of the blood vessels
Anaemia The low concentration of haemoglobin causes a
reduced inhibition of basal endothelium- derived
relaxing factor activity and leads to generalised
vasodilation. The consequent low blood pressure
may be the stimulus for neurohormonal
activation and salt and water retention
Hypothyroidism Hypothyroidism induces a decreased basal
metabolism and thermogenesis, an accumulation
of hyaluronic acid and a decreased renal ow, all
factors leading to water retention
Rheumatoid arthritis Lymphatic obstruction occurs by brin
degradation of inammatory products that block
the lymphatic channels. There is also a link to
drug- induced and decreased mobility for
some people
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TABLE2.6
Factors to consider Why?
Drug-
Lipoedema Lipoedema as a condition on its own does not
(Continued)
induced Many pharmacological agents modify the activity
of ion channels and other protein structures in
lymph muscle cells to disrupt the cyclic
contraction and relaxation of lymph vessels,
thereby compromising lymph ow and
predisposing to the development of
lymphoedema (Largeau etal.2021)
always present with swelling. Consideration
links additional risk factors with decreased
mobility, concurrent obesity and venous
insuciency. In addition, there is a theory that
the increased subcutaneous fat may delay the
initial lymphatic drainage routes
ASSESSMENT, DIAGNOSIS ANDSTAGING
Many patients in the community experience early or more advanced
signs of lymphoedema that can be overlooked (Moatt etal.2017,
2019; Quéré etal.2019; Nørregaard etal.2019; Gordon etal.2019).
It is therefore important to be vigilant for signs of lymphoedema
when assessing or treating patients in primary care, especially those
at high risk, for example patients with any wound. Lymphoedema is
prevalent in lower limb wounds and also has a higher prevalence in
those with post- surgical, cancer- related, arterial or vascular conditions, obesity, impaired mobilityand with diabetes. Specialist lymphoedema services should always be involved in the diagnosis of
primary lymphoedema (British Lymphology Society2016). However,
it is essential that all healthcare professionals follow the principles of
early recognition and prompt assessment.
While patients with lymphoedema may require a diagnosis from
a specialist for appropriate treatment and management, this does
not negate early- stage recognition and rst- line assessment and
management within the more generic community and primary care
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setting. Diagnosis is also important to slow the progression of the
disease and prevent the development of co- morbidities, to minimise
negative impacts on the person’s quality of life and to ameliorate the
physical and psychosocial problems associated with lymphoedema.
Diagnosis and intervention at an early stage of the disease are more
eective than once the disease has progressed to a more severe form
(British Lymphology Society2020, p.2). A late diagnosis can increase
the need for healthcare services and increase the risk of a convoluted
patient journey.
Symptoms ofLymphoedema
Swelling present for three months or longer.
Swelling that does not reduce overnight or on elevation.
Skin changes– thickened, dry, dilated lymph vessels.
Tissue changes in consistency, becoming rm/hard, may be non-
pitting.
Recurrent infections.
Simple diagnosis summary questions:
When did it start?
Was it sudden or gradual?
Is it bilateral or unilateral?
Where is the oedema? At the distal end of the limb or near the
trunk?
Have systemic causes been ruled out? (For example, sudden
acute swelling, underlying heart failure or renal impairment,
unilateral onset, underlying malignancy, DVT or infection.)
What does the skin look and feel like?
How signicant is the swelling? And how far does it extend?
Is there shape distortion?
Are any triggers noted? (For example, a new medication and sud-
den swelling, a new diagnosis, decreased mobility or a recent
traumatic wound.)
Is there a history of cellulitis or lymphorrhoea (lymph uid
leaking on the skin)?
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Diagnosis
A full assessment combining the past medical history and lymphoedema history, including family history and cellulitis history, should
be complemented by a physical examination. It should include staging consideration to exclude any systemically driven causes that have
been investigated or may require further investigation to rule them
out. The assessment aims to support a dierential diagnosis by pulling all the key components together.
Step1: Medical History
A full medical history of the patient is required, including the personal
history of lymphoedema, a history of travel to areas where lariasis is
endemic, past inguinal or axillary lymphadenectomy orradiation, a history of severe obesity (body mass index [BMI] >50 kg/m2) or cellulitis.
Other important indications of lymphoedema are the site of swelling
onset and presentation of the swelling, considering whether the swelling
is distal or proximal to the trunk (Greene and Goss2018, p.12) (Table2.7).
TABLE 2.7
Past medical history Identify if there is any direct link or trigger to the
History of onset/site
of swelling
Age of onset Consider age of onset– prevalence increases with
Medical history tosupport diagnosis oflymphoedema.
presentation of the swelling, for example cancer
diagnosis, venous disease, previous surgery or a
history of deep vein thrombosis (DVT)
When did it start? Was it gradual or sudden? Was it
linked to a medical cause or was there an external
trigger, such as trauma, an insect bite or a period
of immobility? N.B. Check if there was
intermittent swelling in the past even if it was not
bothersome. What body area is aected and is
this distal or proximal, whole limb, trunk?
age linked to immobility and concurrent
co- morbidities. In cases rst line is to exclude any
cause for concern such as cancer, DVT and
infection. Once excluded, consider if this could
be a primary lymphoedema
(Continued)
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TABLE2.7 (Continued)
Allergies Note all allergies and sensitives as this may aect
the ability to manage and require consideration
with compression and skin care products
Diet and
Sleep pattern Not sleeping in bed or having a poor night’s rest will
Mobility Any decrease or alteration in mobility may impact
Lifestyle
Medication There are many drugs that can trigger swelling or
Family history Family history may link to a predisposition and
Cellulitis history and
nutritional
status
(drugs/alcohol)
lymphorrhoea
Obesity is a direct risk factor. Being underweight
and unexpected weight loss may link to general
poor health or to a direct systemic concern. Both
may link to general poor nutritional status and
ill health
link to dependency-
and contribute to swelling
Consider if there is intravenous drug use, which can
cause damage. Alcohol consumption is linked to
liver damage and associated low albumin. Both
link to poor nutritional status in some cases
have a negative impact on existing swelling. Some
of these are discussed in more detail in Chapter8.
The most commonly used drugs that can cause
oedema are calcium channel blockers, e.g.
amlodipine; nondrugs (NSAIDs), e.g. ibuprofen; corticosteroids,
e.g. prednisolone; and hormones and related
compounds, e.g. tamoxifen (Pal etal.2022)
primary lymphoedema or to other existing
medical risk factors, e.g. venous insuciency
(Brouillard etal.2021; Ho etal.2018)
Identifying cellulitis episodes and repeated episodes
can be linked directly as there is a higher lifetime
prevalence of cellulitis in those with swelling. It
also correlates to delays in therapeutic
management. Cellulitis can be as high as
37%lifetime prevalence for those with
lymphoedema (Vignes etal.2022; Burian
etal.2021). If there has been any episode of
lymphorrhoea (leaking) this will represent a high
risk of cellulitis, as the skin integrity is breached
and represents unmanaged lymphoedema
related swelling
steroidal anti- inammatory
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Lymphoedema History
History taking of the lymphoedema onset and its presentation are
required as part of a holistic assessment. Where it is noted that there has
been a sudden onset of swelling, this may relate to an underlying systemic
cause and thus require a systemic medical assessment in the rst instance.
Longer- standing gradual swelling with an identied linked reason for
venous insuciency and decreasing mobility, with no systemic concerns
or identied medical history and gradual presentation, will lend itself to
an easier diagnosis as the identication is clear. Any swelling noted that is
proximal to the trunk and unilateral would be a cause for concern and
necessitate a medical assessment to out rule any red ags, such as an
underlying malignancy not yet diagnosed or a reoccurrence, DVT or cellulitis. In addition, a family history of lymphoedema where there has
been a diagnosis of primary lymphoedema may provide a link to a primary lymphoedema, as in some cases this can be inherited. If the presentation of swelling is of sudden onset, there is signicant deterioration of
the oedema that was otherwise stable or swelling close to the trunk with
no clear identifying factors, all systemic causes should be ruled out from
a medical perspective as part of exclusion (for example cardiac failure,
renal impairment, undiagnosed malignancy or DVT) and a subsequent
specialist assessment would be required (Gasparis etal.2020).
Cellulitis andLymphorrhoea History
Capturing a detailed history of cellulitis episodes alongside any hospital
admissions linked to the onset of the condition will aid in consideration of the diagnosis and management plan; new or repeated episodes
demonstrate delayed identication or sub- therapeutic management.
Lymphorrhoea represents the most critical component of unmanaged
lymphoedema. Patients who are leaking will have a higher risk of
cellulitis infection (skin integrity is breached and the oedema is not controlled). Similar to cellulitis, unmanaged lymphorrhoea also directly
correlates with late intervention or assessment or with sub- therapeutic
management. Approximately 10% of palliative patients will be symptomatic with lymphorrhoea due to the overwhelming uid retention as
part of the end- of- life process (Real etal.2016). Repeated infection due
to poorly managed oedema is resource intensive in nursing time, General Practitioner (GP) time, dressings and admissions; these healthcare
costs can be avoided with eective treatment.
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Step2: Consider Other Causes andExclusions
In addition to the screening investigations (Table2.5), consideration
should be given to the presentation of the lower limb swelling.
When swelling is rst identied, it is essential that simple preliminary investigations are considered by the medical practitioner.
This is in part to out rule any signicant systemic root cause for the
swelling; see Table2.8. It is important to be alert for possible signs of
the development of systemic problems that require urgent investigation. The critical concerns would be to out rule venous thrombosis,
cancer diagnosis or reoccurrence.
Red ags to observe are:
Pain– persistent or newly presented pain.
Persistent neuropathic pain.
Unusual presentations, sudden swelling or swelling proximal to
the trunk with sudden onset and unresponsive.
TABLE 2.8
Unilateral (single limb) swelling
Acute deep vein thrombosis
Post- thrombotic syndrome
Arthritis
Baker’s cyst
Trauma
Presence/recurrence of carcinoma
Symmetrical (bilateral) swelling
Heart failure
Chronic venous insuciency
Dependency or stasis oedema
Renal impairment
Hepatic impairment
Hypoproteinaemia
Hypothyroidism/myxoedema
Drug- induced (e.g. calcium channel blockers, steroids, non- steroidal
anti- inammatories)
Lipoedema
a
Presence or recurrence of carcinoma requires direct referral to the appropriate
oncology service.
Unilateral or bilateral lower limb swelling.
a
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Extensive congestion swelling, which is congested, rm and
unresponsive to compression.
New venous swelling that is congested with associated venous con-
gestion and visible telangiectasia (thread veins) or discoloration.
Step3: Clinical Examination
Observe and record a description of the presentation of the limb and
the site of the swelling. Inspect subcutaneous tissue by sight and
palpation. Record a history of skin changes such as skin folds, dry skin,
hyperkeratosis, papillomatosis or peau d’orange. With permission,
take photographic images for the patient record. Gather context from
the patient on the skin changes, the impact of these and the symptoms reported. Clinicians should look for asymmetries in the condition of the skin and signs of congestion, for example changes in skin
colour or ‘staining’, giving particular attention to skin changes such
as peau d’orange, papilloma or brosis (see later Table2.10). Localised
proximal swelling to the trunk or discoloration such as a red- bluish
colour of the skin with the appearance of venous congestion should
be considered a red ag for the presence of cancer or DVT.
A wide variety of investigations may be considered as part of
exclusion considering systemic causes and to support diagnoses
(Table2.9).
TABLE 2.9
Full blood count (FBC)
Urea and electrolytes (U&Es)
Thyroid function tests (TFTs)
Liver function tests (LFTs)
Plasma total protein and albumin
Fasting glucose
Erythrocyte sedimentation rate (ESR)/C- reactive protein (CRP)
B- natriuretic peptide
Ultrasound, commonly abdominal ultrasound
Venous doppler
Venous and arterial duplex
Chest X- ray
Source:Adapted from Lymphoedema Framework, 2006: 31
Screening andinvestigations.
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