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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_781_Библиотеки_им_академика_М_И_Перельмана

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82 Lymphoedema and ChroniC SweLLing
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TABLE 2.20 
Untreated non- pitting chronic lymphoedema Known or suspected deep vein thrombosis Pulmonary embolism Thrombophlebitis Acute skin inammation such as cellulitis or erysipelas Uncontrolled or severe cardiac failure Pulmonary oedema Ischaemic vascular disease Active metastatic disease aecting the lymphoedematous area Oedema at the root of the aected limb or in the adjacent trunk Severe peripheral neuropathy
Source: Adapted from Lymphoedema Framework (2006), p.31.
Contraindications tointermittent pneumatic compression.
etal.2017). However, more research is needed to conrm how laser treatment works and its benets in lymphoedema (Lymphoedema Framework2006, p.51).
SURGERY
A variety of surgical options are available for lymphoedema, although access to surgery via the NHS is limited (British Lymphology Soci­ety 2022b). The options available are reconstructive microsurgery and debulking surgery. Risks associated with surgery include infec­tion, bleeding, abnormal scarring and lymphorrhoea. Surgery should only be performed by surgeons who have experience of lymphoe­dema and are specialists in the lymphatic system.
Reconstructive Microsurgery
The aim of reconstructive microsurgery is to improve the functioning of the remaining lymphatic system, improve drainage, reduce swell­ing and reduce the patient’s need for ongoing use of non- surgical treatments (British Lymphology Society2022b). One type, lymphati­covenular anastomosis (LVA), is suitable for patients with good remaining lymphatic function. It is minimally invasive. The surgery
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introduces ne channels connected to veins that enable lymphatic uid to bypass scarred or damaged tissues and return to circulation within the aected limb (Koshima etal.2003; Campisi etal.2007).
Lymph node transfer is more invasive and requires hospitalisa­tion for three to ve days (British Lymphology Society 2022b). It involves transplanting healthy lymph nodes from the neck or abdo­men, for example, to the aected area, where over time the healthy lymph nodes regenerate lymphatics by absorbing excess uid (British Lymphology Society2022b).
Debulking Surgery
If the lymphoedema is advanced, the accumulating lymphatic uid in the leg changes into fatty tissue and brous scar tissue through the action of proteins and growth factors in the uid. At this stage, recon­structive surgery is not an option because it is no longer possible to redirect the uid (Hague etal.2020).
Lymphatic liposuction removes fatty and scar tissue that has formed in the aected limb. It requires hospitalisation for two to three days due to the high risk of serious complications. It reduces limb volume and improves limb shape. Compression hosiery needs to be worn day and night indenitely after the operation to maintain good lymphatic function (Schaverien etal.2018).
PALLIATIVE CARE
Lymphoedema at the end of life may be associated with long- standing lymphoedema or with other diseases such as cancer, chronic heart failure, advanced renal or liver disease, advanced neurological disease or end- stage respiratory disease. The causes are often complex. Advanced disease may impair capillary ltration as a result of venous hypertension or hypo- albuminaemia, or impaired lymphatic drainage as a result of surgery, radiotherapy, metastatic lymphadenopathy, long- standing lymphoedema or immobility (International Lymphoe­dema Framework and Canadian International Lymphoedema Frame­work2010, p.4) (Table2.21). Immobility at the end of life is also a problem that may exacerbate oedema.
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TABLE 2.21 End- of- life diseases associated withlymphoedema.
Disease Symptoms and complications
Advanced pelvic cancer Soft and pitting oedema may aect the legs,
genitalia and lower abdomen
Neuropathic pain, bladder spasms, stulae,
haemorrhage and malodorous exudate may be part of ‘pelvic syndrome’
Lymphorrhoea is also common
Chronic heart failure Peripheral oedema
More widespread oedema and ascites can occur Immobility exacerbates the symptoms
End- stage renal disease Hypoalbuminaemia
Fluid load may become intractable with dialysis
End- stage liver disease Ascites, jaundice, hepatic encephalopathy,
bleeding from oesophageal or gastric varices
Advanced
neurological disease
End- stage
respiratory disease
Soft, pitting oedema Immobility promotes oedema In Parkinson disease, the weaker side usually
has more oedema
Extensive soft, pitting lymphoedema as a result
of immobility or cor pulmonale
Source: Adapted from International Lymphoedema Framework and Canadian Inter­national Lymphoedema Framework (2010), pp.6–7.
The prevalence of oedema at the end of life is estimated at 5–10% (International Lymphoedema Framework and Canadian Interna­tional Lymphoedema Framework2010, p.3). The patient may expe­rience oedema for an extended period before death, in some cases with limited access to specialist care (Real etal.2016). Lymphoedema of the lower limbs is a common problem in palliative care patients. In an evaluation of 63 cases aged 45–97 years from a specialist palliative care oedema service, almost 9in 10 patients had lower limb lym­phoedema (Real etal.2016). It is essential for the palliative care team to focus eorts on providing the best possible combination of eec­tive treatments to suit the individual patient and relieve pain and discomfort as much as possible.
The aim of palliative care is to avoid further harm and to promote comfort. Early intervention is important. Many patients
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experience additional, preventable harm and suering because intervention is not provided (Cobbe 2021; International Lymph­oedema Framework and Canadian International Lymphoedema Framework2010). It is therefore important to identify patients who require a palliative care approach at an early stage so that the neces­sary treatment regime and professional and institutional support can be put in place.
Assessment
Early assessment and intervention are essential to provide the patient with the best chance of managing symptoms well and achieving the greatest possible comfort (Lymphoedema Framework 2006). Too often patients do not present to health professionals until the lymphoedema is advanced and much more dicult to treat (Shah etal.2016).
Assessment needs to consider the patient’s priorities and goals. The cause of the lymphoedema should be diagnosed and the most appropriate treatment pathway determined that can improve symptoms. The stage of the underlying condition and the rate of progression should also be considered (International Lymphoe­dema Framework and Canadian International Lymphoedema Framework2010, p.8). It is most important to consider the symp­toms’ impact on the patient in several ways:
How the symptoms aect their physical health and mobility.Impact on the patient’s capacity to carry out daily tasks and
activities.
Impact on the patient’s family situation and the availability of
support and carers.
Presence and degree of pain.
Tools such as the Palliative Care Outcome Scale (PCOS) (Sherry and McAuley2004) or the Memorial Symptom Assessment Scale can be helpful to assess the impact of symptoms in palliative care patients (Tranmer etal.2003). LYMQOL (Keeley etal.2010) is also available to measure the specic impact of lymphoedema on the palliative patient’s quality of life.
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Management
It is likely that the palliative patient with lymphoedema will have a complex regimen combining several therapies designed to control oedema, including compression, MLD, exercises and skincare. This is sometimes referred to as complete decongestive therapy. All of these therapies need to be focused on the comfort of the palliative patient and the treatment regimen may need to be adapted (International Lymphoedema Framework and Canadian International Lymphoe­dema Framework2010, p.15). MLD may provide some symptom relief and improve comfort.
Compression Therapy
Compression therapy for the palliative patient remains a critical part of management, but provision at a therapeutic level or dose may not be suitable even in the presence of lymphorrhoea. Lymphoedema compression in a reduced dose may be more suitable for comfort dependent on the extent and site of the lymphoedema.
Exercise is important to generate muscle activity to improve the propulsion of uid through the lymphatic system. It helps avoid limb deconditioning by building muscle strength. It softens brosis and increases venous and lymphatic return. Deeper breathing activates the diaphragm to support lymphatic drainage. However, it is impor­tant to avoid overstraining in palliative patients, as trauma and inammation worsen the symptoms of lymphoedema (International Lymphoedema Framework and Canadian International Lymphoe­dema Framework2010, p.14).
Skincare in palliative patients restores hydration, counteracting the mechanical stressors of compression therapy and maintaining hygiene (International Lymphoedema Framework and Canadian International Lymphoedema Framework2010, p.14). It is an oppor­tunity to observe the skin for signs of infection and signs of risk fac­tors such as cellulitis.
Summary
Lymphoedema has a signicant prevalence within the population and has the potential to continue to increase further with the ageing population, the increase in obesity and the longer- term survivorship
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from cancer treatment. In addition, the correlation with lower limb wounds, venous insuciency and decreased mobility demonstrates the need for a system- wide approach to management. Lymphoedema baseline education for all levels of healthcare professionals and increased awareness for the public are required to support early detection and management. Through early diagnosis and interven­tion, patient harm will be avoided and the risk of an increased eco­nomic burden will be reduced, alongside a reduction in the negative impact for those who live with lymphoedema. A tiered approach to management is required, allowing for early identication and screen­ing, prompt assessment and management, and escalation to special­ist services with the ability to take a multidisciplinary approach for the most complex cases.
LIPOEDEMA
What Is Lipoedema?
Lipoedema is a poorly understood long- term condition aecting adi­pose tissue. Commonly known as fat, adipose is a loose connective tissue consisting mainly of adipocytes (fat cells). Its primary function is for storing energy, but it is also involved in endocrine function through the release of hormones. In lipoedema, the adipocytes of the subcutaneous layer (under the skin) expand and replicate in an irreg­ular way, leading to painful lumps beneath the skin. Lipoedema can be progressive and as progression occurs these lumps enlarge, lead­ing to large overhanging fat pads and signicant distortion of limb shape. In addition to causing pain, lipoedema restricts mobility, lim­its life choices and often has a profound impact on a patient’s mental health. Lipoedema almost exclusively aects women, but some cases have been reported in men. Research from Germany estimates that 11% of women and post- pubertal girls are aected (Szél etal.2014); however, the condition is under- recognised by health professionals, so the true percentage is almost certainly higher.
Outside of specialist services, lipoedema is generally not recog­nised and, since it appears at rst glance similar to obesity or lym­phoedema, it is often misdiagnosed. Lipoedema, lymphoedema and obesity are separate conditions with dierent pathologies, although it is possible to have them concurrently. For example, a patient with
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lipoedema may also have chronic secondary swelling (lymphoe­dema) or may still have obesity even if the abnormal lipoedema fat were not present. It is therefore essential that a holistic assessment is completed to ensure that advice is appropriate for the individual patient. Table 2.22 highlights key dierences between lipoedema, lymphoedema and obesity.
How toRecognise Lipoedema inPractice
The frequent lack of recognition, particularly in primary care, often leads to patients being given unhelpful or harmful advice. Many patients report being dismissed as obese by their GP and end up pur­suing a cycle of aggressive dieting, which will not address the lipoedema. One of the key features of lipoedema is that the aected adipose tissue does not respond in the same way to conventional diet­ing as normal body fat does, so little improvement is made and symp­toms persist. The exact aetiology of lipoedema is not fully understood, but it is widely accepted that there are genetic, hormonal and inam­matory components to it. A family history of relatives with similar leg shapes is often noted, and the onset of the condition is typically seen around times of hormonal change such as puberty, childbirth or menopause (Al- Ghadban etal.2021). Concurrent inammatory con­ditions such as chronic bowel disorders and skin problems like acne and psoriasis are not uncommon for lipoedema patients. It is also estimated that around 40% of lipoedema patients have a thyroid dis­order such as hypothyroidism or Hashimoto disease (Lukowicz etal.2021).
In addition to an enlarged and distorted limb prole, the skin of lipoedematous areas often feels cool compared to unaected areas. On palpation it also usually feels soft, oppy, and may have indenta­tions and a texture like that of orange peel (also known as peau d’orange). In the early stages, a granular texture may be felt below the skin. Patients may report that the limbs feel tired, uncomfortable or tender to touch. Another common feature is being easily bruised. As lipoedema advances and the tissues expand, the size and shape of the limbs can change dramatically.
Visual examination is important because lipoedema can aect dierent parts of the body. Examples include full leg from buttocks to ankles (see Figures2.4 and2.5) or only around the hips and buttocks,
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TABLE 2.22 Key dierences between lipoedema, lymphoedema
andobesity.
Characteristic Lipoedema Lymphoedema Obesity
Sex of patient Almost always
women, rare in men
Age of onset Times of hormonal
change
Causes Possibly genetic
and/ or hormonal
Familial
history of the condition
Impact of
weight loss
Body location Legs, buttocks,
Laterality Bilateral Unilateral
Presence of
pain
Common Yes if primary,
Very little,
disproportionate if any
hips and less frequently arms
Usually painful Not directly
Men or women Men or women
Any age
depending on the cause
Primary
(genetic) or secondary (lifestyle, injury, cancer, etc.)
coincidental if occurring via secondary causes
Benecial,
proportional weight loss across the body
Any body part Any body part
or bilateral
painful
Any age
Usually lifestyle
but sometimes triggered by other conditions (e.g. Cushing disease)
Common, but
more to do with socioeconomic factors than genetics
Very benecial,
proportional weight loss
Bilateral
Not directly
painful but can impact other body parts (e.g. joint pain)
FIGURE 2.4 Type III, Stage 2lipoedema, anterior view. Note the excess
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tissue around the inner aspect of the knees.
FIGURE 2.5 Same patient as in Figure2.4, posterior view. Note the stance
and position of the knees and ankles.
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FIGURE 2.6 Close- up anterior view of ankles to demonstrate ‘cu’
phenomenonin early stages. This becomes more prominent as the condi­tion progresses.
but other presentations have been documented. The arms can also be aected, but lipoedema does not aect the feet or hands. If present below the knees it will stop suddenly at the ankles with a prominent ‘cu’ eect, and the concave spaces behind the malleoli will be lled in (see Figures 2.6 and 2.7). Fat pads usually develop around the inner part of the knees, and these steadily become larger and more cumbersome as the condition progresses. Lipoedema presents bilat­erally and the overall limb shape is usually symmetrical; however, one limb may be larger if there is secondary oedema.Figures 2.8, 2.9 and 2.10 show a more advanced case displaying many of the features described.
Venous disease is a common co- morbidity that occurs in patients who have lipoedema, with many patients displaying symptoms such as varicose veins and telangiectasia. Chapter 1 explores these and other venous conditions in more detail. Evidence suggests that lipoedema weakens connective tissue, including that of blood vessel walls, meaning that they are more susceptible to damage, which leads to venous disease (Allen et al.2020). This could also explain