The Lymphatic System

While our understanding and recognition of the lymphatic system has considerably

improved recently, it is still one of the most scantily understood and researched

physiological systems in the body.


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 by

the Australasian Lymphology Association (ALA) 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?


The ALA reports:

  • 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 uncreated and unmanaged for an extensive period of time, 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.



References


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