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52 Lymphoedema and ChroniC SweLLing
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CAUSES OFAND 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 etal.2020), prostate and penile cancers in men and melanoma of the lower limbs (Paskett etal.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 etal.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 etal.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 (Moatt etal.2019).
Cellulitis– lymphoedema is associated with a history of celluli-
tis, and the presence of lymphoedema substantially increases the risk of cellulitis (Mortimer and Rockson2014, 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 (Moatt et al.2003). A more recent Canadian study reported that 72.06% of patients attending a wound management clinic had suered from cellulitis (Keast et al.2019). A cost burden of £178 million is aligned to acute hospital admissions for lymphoedema- related cellulitis (National Lymphoedema Partnership2019).
Risk factors that increase prevalence – cancer, increased age,
obesity, heart failure and neurological defects, particularly those impacting mobility (Quéré etal.2019) (Table2.4).
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 insuciency, which may reduce lymphatic motility; and immobility, which reduces muscu­lar function of the venous and lymphatic systems (NLP2019, p.9).
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TABLE 2.4 Risk factors forlymphoedema.
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 Inammatory response to the
Venous
thromboembolism/ thrombosis
Chronic venous
insuciency/ venous ulceration/
venous surgeries Dermatitis/eczema Disruption of skin integrity Damage Scarring Disruption of skin integrity
Trauma May aect 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 aecting 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 (Tables2.5 and2.6). 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 ofswelling.
lymphatic system
Filariasis
TABLE 2.6 Common factors that will inuence andcontribute
tothe 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 inammatory products that block the lymphatic channels. There is also a link to drug- induced and decreased mobility for some people
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TABLE2.6 
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 etal.2021)
always present with swelling. Consideration links additional risk factors with decreased mobility, concurrent obesity and venous insuciency. In addition, there is a theory that the increased subcutaneous fat may delay the initial lymphatic drainage routes
ASSESSMENT, DIAGNOSIS ANDSTAGING
Many patients in the community experience early or more advanced signs of lymphoedema that can be overlooked (Moatt etal.2017, 2019; Quéré etal.2019; Nørregaard etal.2019; Gordon etal.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 mobilityand with diabetes. Specialist lym­phoedema services should always be involved in the diagnosis of primary lymphoedema (British Lymphology Society2016). 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 eective than once the disease has progressed to a more severe form (British Lymphology Society2020, p.2). A late diagnosis can increase the need for healthcare services and increase the risk of a convoluted patient journey.
Symptoms ofLymphoedema
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 signicant 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 dierential diagnosis by pull­ing all the key components together.
Step1: 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 orradiation, 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 Goss2018, p.12) (Table2.7).
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 tosupport diagnosis oflymphoedema.
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 aected 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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TABLE2.7 (Continued)
Allergies Note all allergies and sensitives as this may aect
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 Chapter8.
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 etal.2022)
primary lymphoedema or to other existing medical risk factors, e.g. venous insuciency (Brouillard etal.2021; Ho etal.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 etal.2022; Burian etal.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- inammatory
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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 identied linked reason for venous insuciency and decreasing mobility, with no systemic concerns or identied medical history and gradual presentation, will lend itself to an easier diagnosis as the identication 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 signicant 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 etal.2020).
Cellulitis andLymphorrhoea 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 identication 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 etal.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 eective treatment.
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Step2: Consider Other Causes andExclusions
In addition to the screening investigations (Table2.5), consideration should be given to the presentation of the lower limb swelling.
When swelling is rst identied, it is essential that simple pre­liminary investigations are considered by the medical practitioner. This is in part to out rule any signicant systemic root cause for the swelling; see Table2.8. 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 thrombosisPost- thrombotic syndromeArthritisBaker’s cystTraumaPresence/recurrence of carcinoma
Symmetrical (bilateral) swelling
Heart failureChronic venous insuciencyDependency or stasis oedemaRenal impairmentHepatic impairmentHypoproteinaemiaHypothyroidism/myxoedemaDrug- induced (e.g. calcium channel blockers, steroids, non- steroidal
anti- inammatories)
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.
Step3: 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 Table2.10). 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 (Table2.9).
TABLE 2.9 
Full blood count (FBC)Urea and electrolytes (U&Es)Thyroid function tests (TFTs)Liver function tests (LFTs)Plasma total protein and albuminFasting glucoseErythrocyte sedimentation rate (ESR)/C- reactive protein (CRP)B- natriuretic peptideUltrasound, commonly abdominal ultrasoundVenous dopplerVenous and arterial duplexChest X- ray
Source:Adapted from Lymphoedema Framework, 2006: 31
Screening andinvestigations.
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