Our understanding and recognition of the lymphatic system has considerably
improved in recent years, but more research is needed to understand in greater detail its role in modulating inflammation and repair, how the lymphatic system is impacted in health and disease as well as what influences dysfunction and how we can identify this early to improve outcomes and management strategies.
The lymphatic system in health
The lymphatic system is a closed network making up the body’s circulatory system.
It is a one-way drainage system that executes a vital role in the returning of fluid via lymphatic vessels and lymph nodes. Fluid seeps out from the blood capillaries and into the tissue spaces, where the lymphatic system then eradicates the fluid, along with plasma proteins and cell debris, thereby maintaining homeostasis within the body. In health, the lymphatic system carries out integral duties such as; managing fluid flow throughout the body (tissue fluid equilibrium), ferrying waste from body tissues, lipid absorption, and immune cell trafficking, which is paramount in the formulation and development of an inflammatory response to terminate pathogens. As it can now be grasped, in the absence of lymphatic function, life ceases, and when there is dysregulation of the lymphatic system,
pathology results.
The lymphatic system in disease
When there is a breakdown in the lymphatic drainage system and interstitial tissues
are unable to be sufficiently drained, an accumulation of fluid and proteins ensues
and swelling of the affected area follows. When this swelling lingers for greater than
three months in duration, it is termed chronic oedema. One of the central causes of
chronic oedema is lymphoedema.
Lymphoedema is a chronic, non-curable but manageable condition and is defined as the collection of disproportionate amounts of protein-rich fluid causing swelling of one or more areas of the body. This is consequential of lymphatic system failure, when the need for lymphatic drainage surpasses the capacity of the lymphatic circulation. Lymphoedema typically affects the limb(s), though it can include the trunk, breast, head, neck or genital area.
What causes lymphoedema and what are the risk factors for developing lymphoedema?
Lymphoedema is categorised into either primary lymphoedema or secondary
lymphoedema.
1. Primary lymphoedema (genetic): Resultant of a congenital condition affecting the
development of lymph vessels. This may culminate in hypoplasia of lymphatic
vessels, hyperplasia of lymphatic vessels, or aplasia of the lymphatic system.
Primary lymphoedema can arise at birth, advance at the onset of puberty or may
remain dormant for many years until adulthood.
2. Secondary lymphoedema: Resultant of lymphatic system damage. The
destruction can befall due to cancer treatments (such as lymph node removal),
radiotherapy of lymph node groups, evolution of malignant disease, trauma,
tissue damage, venous disease, immobility and dependency, factious, infection,
filariasis or obesity.
Both primary and secondary lymphoedema can ensue collectively.
What is the incidence of lymphoedema?
1:6000 will develop primary lymphoedema
20% of breast genitourinary, gynaecological, or melanoma survivors will experience secondary lymphoedema.
36-47% of vulval cancer sufferers will acquire secondary lymphoedema
20% of breast cancer sufferers will develop secondary lymphoedema
24% of cervical cancer sufferers will foster secondary lymphoedema
9-29% of melanoma sufferers will cultivate secondary lymphoedema
4-8% will develop secondary lymphoedema following sentinel lymph node biopsy
What complications can arise with lymphoedema?
Lymphoedema is a progressive disease with a myriad of accompanying duration
dependent impediments, including:
Decreased mobility and weight gain
Fibrosis
Shape distortion, heaviness and pain of the affected area
Lymphohorrhea, where the lymph fluid escapes and collects on the skins surface
Diminished psychosocial and quality of life (QoL)
In extremely rare and severe cases, where the condition remains undetected and unmanaged for an extensive period, a form of cancer termed Lymphangiosarcoma may arise
There is also an extensive multitude of skin alterations associated with lymphoedema, these involve:
Hyperkeratosis: excessive overgrowth of the cornified epithelial layer of the skin which creates a skin thickening
Dry and flaking skin: owing to the reduction in oil secretion from the sebaceous glands
Impaired barrier which decreases the skins role in protection against bacteria and allergens, ultimately resulting in inflammation, delayed wound healing and possible infections such as, cellulitis, lymphangitis, lymphadentisis, sepsis and skin ulcers
Papillomatosis: chronic congestion of the lymphatic vessels which generates the development of wart like protrusions on the skin’s surface
Hyperpigmentation
Diagnosis and assessment of lymphoedema
An accurate diagnosis and assessment of lymphoedema is essential in ascertaining
an appropriate treatment and management plan. Identification of the disease is
achieved through a thorough and detailed patient assessment, which includes a
patient history and a physical examination. Presently, there are many assessment
techniques to aid in the assessment and diagnosis of lymphoedema, these
comprise:
Limb volume measurements
Lymphoscintigraphy: low resolution images highlighting the course of major lymphatics
MRI: visualisation of the lymphatic vessels
Lymphography: images envisaging specific lymphatics draining from an affected region
What are the initial indications of lymphoedema?
Transient swelling of a limb or other regions of the body
Limited mobility, aching, heaviness, stiffness, tightness and or temperature changes
Pain and tension in the affected area
Clothing, jewellery or shoes may feel tighter and more restrictive
Exacerbated swelling due to heat, overuse, sustained positions and extended inactivity
Positive stemmer’s sign (unable to pinch the skin together at the base of the (second toe or finger)
It is vital to note, there are an array of conditions which occupy similar indications,
therefore a thorough assessment by a specialist needs to be performed for an
accurate diagnosis.
Treatment and management of lymphoedema
While lymphoedema cannot be cured, it can be reduced and managed with the
suitable interventions. Current evidence-based and best-practice standard of care for lymphoedema is Complex Decongestive Therapy (CDT), also termed Complex
Lymphoedema Therapy (CLT). CDT comprises of four elements:
1. Skin care:
As formerly outlined, the skin becomes vulnerable in lymphoedema sufferers, hence
the promotion and preservation of the skin’s integrity is fundamental. This is done
via; daily cleansing with a soap free cleanser, meticulous drying of the skin’s surface,
and the downward application of a moisturiser in the form of emollients due to their
facilitation of a surface lipid layer to conserve moisture within the skin.
2. Exercise:
Both daily physical and breathing exercises donate to the body’s natural venous and lymphatic flow to incite the removal of interstitial fluid.
3. Manual Lymphatic Drainage (MLD):
MLD involves breathing techniques and massaging of the skins natural lymphatic
drainage system through the employment of slow and repetitive hand movements.
This stimulates the redirection of fluid from congested and oedematous areas to
healthy and functioning nodal regions.
4. Compression:
Compression requires the application of medically fitted pressure to the affected area in the form of a compression garment. This diminishes the assembly of fluid,
preserves the limb size and shape and supports and inspires lymphatic circulation.
Compression garments are accessible in a variety of styles, sizes, colours and
grades.
CDT also encompasses two phases, where phase I is an intensive treatment
program afforded by specialists, and phase II entails self-management.
The role of the Dermal Clinician in lymphoedema
Evidence has acknowledged, the effective management of lymphoedema involves a multidisciplinary and collaborative approach with the input of diverse specialists,
including Dermal Clinicians. Dermal Clinicians hold vital duties in both the early
detection and management of lymphoedema, owing to their specialisation in skin
management and integrity and their comprehensive training in MLD.
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