Fact sheet - Lymphoedema

This Lymphoedema fact sheet has been shared on mycarespace with the permission of the Àustralasion Lymphology Association

1. What is Lymphoedema?

Lymphoedema is the accumulation of excessive amounts of protein-rich fluid resulting in swelling of one or more regions of the body.

This is due to a mechanical failure of the lymphatic system and occurs when the demand for lymphatic drainage exceeds the capacity of the lymphatic circulation. The condition usually affects the limb(s) although it may also involve the trunk, breast, head and neck or genital area. 

The lymphatic system is a network of vessels and nodes throughout the body that transports fluid (lymph) from the body tissues back to the bloodstream. The functions of the lymphatic system are to maintain the volume and protein concentration of the extracellular fluid in the body and to assist the immune system in destroying pathogens and removing waste products from the tissues.

2. What causes lymphoedema?

Lymphoedema may arise because the lymphatic vessels or nodes have been damaged or were not formed correctly. Secondary lymphoedema is the most common type developing following damage to the lymphatic system. The damage may occur as a result of some cancer treatments including the removal of lymph nodes, following radiotherapy to lymph node groups or with the progression of malignant disease. The onset of lymphoedema may be at any time. It may occur within months of the damage or it may appear years later.

Secondary lymphoedema may also arise without a cancer diagnosis when one or more of the following conditions occur:

Trauma and tissue damage
Venous disease
Immobility and dependency
Factious – self harm
Infection such as cellulitis - see Consensus Guideline: Management of cellulitis in lymphoedema
Filariasis - Lymphatic Filariasis is a major cause of lymphoedema in the sub -tropical areas of the world. Parasitic filarial worms are transmitted through mosquito bites. The parasites lodge in the lymphatic system causing destruction of the healthy vessels and nodes, resulting in lymphoedema. More information can be found on the WHO web site:http://www.who.int/tdr/diseases-topics/lymphatic-filariasis/en/
Obesity
Primary Lymphoedema in comparison to Secondary Lymphoedema is the result of a congenital condition that affects how the lymph vessels where formed. This may result in hypoplasia of lymphatic vessels (a reduced number of lymphatic vessels), hyperplasia of lymphatic vessels (vessels that are too large to be functional) or aplasia (absence) of some part of the lymphatic system. This form may be presents at birth (congenital), develop at the onset of puberty (praecox), or not become apparent for many years into adulthood (tarda). It may be associated with other congenital abnormalities/syndromes.

Primary and secondary lymphoedema can occur together.

3. What is the incidence of lymphoedema?

The National Breast and Ovarian Cancer Centre (NBOCC) review of research evidence on Secondary Lymphoedema states “conservative estimates suggest that 20% of breast, genitourinary, gynaecological, or melanoma survivors will experience secondary lymphoedema.

More specifically the incidence of secondary lymphoedema associated with vulval cancer is estimated at 36-47%, breast cancer 20%, cervical cancer 24% and melanoma 9-29%. (1)

The incidence of lymphoedema following sentinel lymph node biopsy (SLNB) is reported to range from 4-8%. (4)

At birth, about one person in every 6000 will develop Primary Lymphoedema. (2)

4. What are the risk factors for developing lymphoedema?

The consensus document suggests that patients at risk of lymphoedema will be encountered in a wide variety of health care settings, primary, secondary and tertiary. Key risk factors identified by NBOCC for secondary lymphoedema include, the extent of surgery, lymph node dissection and radiation treatment. Other factors involve trauma, infection, increased body mass index (BMI) and immobility. Any major damage to the lymphatic system causes a life long risk of lymphoedema. For further information about identifying the patient at risk see http://www.lympho.org/mod_turbolead/upload/file/Lympho/Best_practice_20_....

For those with Primary Lymphoedema a referral to genetic counselling maybe indicated to ascertain the risk of lymphoedema.

5. What are the early warning signs of lymphoedema?

Subjectively these may include transient swelling of a limb or other region of the body. Other symptoms may include aching, heaviness, stiffness, limitation of movement, tightness or temperature changes. Clothing, jewellery or shoes may feel tighter. Lymphoedema is not usually a painful condition but some people report pain and tension in an affected limb or body part.

Clients report that the swelling associated with lymphoedema is often aggravated by heat, at the end of the day, with overuse, with sustained positions and prolonged inactivity. They report that gentle exercise, elevation, massage and compression can ease their symptoms.

Many conditions may cause these symptoms to occur and any of the changes described will need to be assessed by a doctor in order for an accurate diagnosis to be achieved.

6. How is lymphoedema diagnosed?

An accurate diagnosis is essential for appropriate therapy. This is determined from the clinical history and physical examination. Co-morbid and confounding conditions of morbid obesity, lipoedema, cardiac disease, renal disease, metabolic disorders, infection, and venous insufficiency will require thorough medical evaluation.

Lymphoedema usually has a gradual onset. However when lymphoedema has an acute onset appropriate tests to exclude, deep venous thrombosis (DVT), recurrence of cancer and infection may be necessary. Sometimes lymphoscintigraphy will be offered to confirm a clinical diagnosis of Primary Lymphoedema.

Whether primary or secondary, lymphoedema develops in stages, from mild to severe. Methods of staging are numerous and inconsistent. They ranged from three to as many as eight stages. In Australasia, the most commonly used stage scale is that adopted by The International Society of Lymphology (ISL) (3), which identifies the following stages:

Stage 0 A latent or subclinical state where swelling is not evident despite impaired lymph transport.
Stage I This represents early onset of the condition where there is an accumulation of tissue fluid with higher protein content, which subsides with limb elevation. The oedema may be pitting at this stage.
Stage II Limb elevation alone rarely reduces swelling and pitting is manifest. In later Stage II the limb may or may not pit as fat and fibrosis supervenes.
Stage III The tissue is hard (fibrotic) and pitting is absent. Skin changes such as thickening, hyperpigmentation, increased skin folds, fat deposits and warty overgrowth develop.
Stage III encompasses lymphostatic elephantiasis. At this stage, the swelling is spontaneously irreversible and usually the limb(s) is very large.
 

7. Are there any complications that can arise with lymphoedema?

Lymphoedema is understood to be a progressive disease and early intervention is recommended to minimise time and age related changes.

The swelling may progress without treatment. The skin is prone to thickening and the development of fibrosis and other secondary changes.

When the lymphatic impairment causes the lymph fluid to exceed the lymphatic system's ability to transport it, an abnormal amount of protein-rich fluid collects in the tissues of the affected area. Left untreated, this stagnant, protein-rich fluid causes tissue channels to increase in size and number, reducing the availability of oxygen. This interferes with wound healing and provides a rich culture medium for bacterial growth that can result in infections: cellulitis, lymphangitis, lymphadenitis, (in severe cases sepsis) and skin ulcers.

It is vital for lymphoedema patients to be aware of the symptoms of infection and to seek treatment at the first signs, since recurrent infections, in addition to their inherent danger, further damage the lymphatic system and set up a vicious cycle. The ALA has developed best practice guidelines in relation to cellulitis - The Management of Cellulitis in Lymphoedema available here.

Very rarely, in certain exceptionally severe cases, lymphoedema untreated over many years can lead to a form of cancer known as Lymphangiosarcoma.

8. What can be done to cure or treat lymphoedema?

Lymphoedema cannot be cured but it can be reduced and managed with appropriate intervention. The stage, location and severity of the lymphoedema together with the individual circumstances of the client will influence the most appropriate intervention. Early intervention is recommended. For further information about management and treatment options see lymphoedema management.