Introduction to Lymphoedema

Contents

• 1. Introduction to Lymphoedema
• i. What is meant by Chronic oedema
• ii. Physiology of lymphoedema
• iii. Lymphoedema and risk of infection
• iv. Classification of lymphoedema
• v. Causes of secondary lymphoedema:
• vi. Signs and Symptoms of Lymphoedema
• vii. Staging of lymphoedema:
• viii. Incidence of lymphoedema
• ix. Risk of lymphoedema
• x. Diagnosing Lymphoedema
• 2. Management of lymphoedema
• 3. Living with lymphoedema
• 4. Prevention post Cancer Treatment

Written by Jan Simmons Macmillan Lymphoedema Project Lead, The NLCN www.nlcn.nhs.uk/ProServLymph

What is meant by lymphoedema and chronic oedema?

Chronic oedema and lymphoedema are now commonly used interchangeable terms to generally define a permanent swelling which has resulted from either a fault within or damage to the lymphatic system, or a combination of lymphatic, vascular and other causes.

Definitions:

Lymphoedema
“the accumulation of lymph in the interstitial spaces, principally in the subcutaneous fatty tissues, caused by a defect in the lymphatic system. (It is marked by an abnormal collection of excess tissue proteins, oedema, chronic inflammation and fibrosis)”
(Foldi et al 1985)

Chronic oedema
‘a broad term used to describe oedema which has been present for more than 3 months and is not relieved by elevation or bed rest and involves one or more of the following areas: limb (s), hands/feet, upper body (breast/chest, shoulder & back), lower body (buttocks, abdomen), genital (scrotal, penis, vulva), and head, neck and face. Oedema which develops as a result of a failure in the lymphatic system is referred to as lymphoedema but chronic lymphoedema may have a more complex underlying aetiology’
(Moffatt et al 2003)

Picture Right shows lymphoedema of lymphoedema of the leg following successful treatment for lymphoma – picture with kind permission from UCLH NHS Foundation Trust

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The Lymphatic System – physiology of lymphoedema:

The blood and lymphatic systems make up the body’s circulation system. These systems work together to form part of the immune system, helping to deal with infection and are also responsible for cleansing the tissues and maintaining the balance of fluids in the body.

The lymphatic system is part of the body’s circulation system and contains vessels and veins similar to the blood system. The fluid contained within the lymphatic system is clear in colour and drains in different pathways than the blood.

The main function of the lymphatic system is to support the immune system and balance fluid within the tissues and cells of the body. Lymphatic vessels drain away excess protein and water which continually leak from the blood into the interstitial spaces (between the cells of the body) This leaking is continuous and the fluid contains large molecules substances, fragments of damaged cells and foreign matter.

Lymph is pumped into and along the vessels by a combination of: the movement of adjacent muscles, contraction of the walls of the large lymphatics, the autonomic nervous system and the action of breathing. This pumping is assisted by many valves inside the vessels.

"‘Pure’ lymphoedema is caused by a failure of the lymph system to remove fluid"

On its way along the vessels, lymph is filtered in the lymph nodes (lymph glands). Lymph fluid passes through lymph nodes (filtering stations) which are mainly located in the armpits, neck, groins and abdomen. These remove foreign matter (bacteria) and start any necessary immune reactions for healing. Finally the lymph empties into the blood. The rate at which this happens varies but ranges from between 2-4 litres every 24 hours.

The key to the effective working of the lymphatic system is the constant movement of fluid away from the interstitial spaces – lymph flows in one direction only unlike the blood. Problems can occur when the lymphatic system is unable to cope with the amount of interstitial fluid present. This may occur when the lymph vessels or nodes are damaged following radiotherapy or surgery.

The accumulation of protein in the tissues causes excess fluid to enter them and the tissues to swell. The swelling decreases the oxygenation (the process by which oxygen is transported to the cells in the body by the blood) of the tissues, interferes with their normal functioning, and makes them heal more slowly than normal. This build up ‘congests’ the affected area and the protein causes a ‘honeycomb’ effect in the tissues, making it more difficult for lymph fluid to pass through. The tissues are overloaded with lymph fluid and the shape of the affected area and the skin may begin to change in appearance, texture and shape.

There are different causes of chronic oedemas, however ‘pure’ lymphoedema is caused by a failure of the lymph system to remove fluid, leading to an accumulation of protein rich fluid in the tissues. Damage to the lymphatic system that compromises its effectiveness, or an excess of interstitial fluid which the lymphatic vessels are unable to transport, can lead to lymphoedema and chronic oedemas.

The physiological processes of all types of chronic oedema/lymphoedema are not fully understood in all cases. Many people do not develop lymphoedema following cancer treatment. There are additional risk factors and circumstances are individual. For instance, it is known that the anatomy of the lymphatic system varies and this may play a role in the development of the condition following cancer treatment,

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Lymphoedema and risk of infection

Excess protein in the oedematous (swollen) area acts as a stimulus for chronic inflammation (persistent swelling and irritation in the affected area). This results in the formation of excess hardened, or fibrous, tissue. This chronic inflammation causes more blood capillaries to form and to be dilated, making the limb warm and sometimes red in appearance.

This heat, combined with the stagnant protein provides a perfect site for bacterial growth. People with chronic oedema and lymphoedema are given strict advice as to what to do in case of an infection. The immune system in the affected area cannot respond quickly and efficiently to the infection and this can quickly make the person seriously ill. It is imperative that antibiotic treatment is started immediately. This does not mean the immune system as a whole does not work, just that the area where congested swelling is present. Some people will need to be admitted to hospital for intravenous antibiotics (into a vein).

"It is imperative that antibiotic treatment is started immediately."

Fungal infections (e.g. Athlete’s Foot) are frequent, and are difficult to treat. Infection places the lymphatic vessels under more stress and can worsen the swelling as it causes further fibrosis (or hardening) of the area making lymph drainage further impaired. The person with lymphoedema is advised to take extra care of their swelling/oedema to avoid infection from mosquito bites, burns, cuts and injury. Some people with lymphoedema suffer with repeated attacks of cellulitis and fungal infections and this must be assessed by a lymphoedema practitioner and appropriate treatment started.

For ease of reading, lymphoedema will be used for the remainder of this information to represent all lymphoedemas and chronic oedema conditions.

Further information regarding symptoms and management of infection can be found in the ‘Management of Lymphoedema – Skincare’ section and guidelines for the management of infection in lymphoedema can be found at
www.thebls.com/concensus.php

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Classification and types of lymphoedema/ chronic oedema

Primary lymphoedema results from abnormalities in the lymph system due to the unusual development of the lymphatic system in utero. Lymphatic vessels may be missing or impaired and therefore unable to function in the usual way. Primary lymphoedema can be generally divided into three categories related to the approximate age when the condition develops:

• Congenital lymphoedema: swelling has been present since birth, commonly in one limb or can involve more than one area of the body
• Lymphoedema praecox: the swelling occurs before the age of 35 years, often at the age of puberty generally in the lower body or legs
• Lymphoedema tarda: the swelling occurs after the age of 35 years generally in the lower body or legs

Primary, or hereditary (familial) lymphoedema, can affect different generations within a biological family and is known to be more common to develop in women than men. Primary lymphoedema generally affects the lower body and legs but in some rarer forms of lymphoedema can affect any other or multiple parts of the body.

Lymphoedema in children is rare but has many presentations / syndromes and may affect several parts of the body. It is imperative that a thorough medical assessment and investigations are undertaken as to the cause of the swelling. Persistent swelling may be a sign of a more serious underlying problem and therefore a diagnosis of lymphoedema must be fully investigated and any other potential diagnosis eliminated. Lymphoedema in children and adolescents following cancer treatment is rare and a new recurrence of cancer must be first eliminated prior to any lymphoedema treatment commencing. There are specialist lymphoedema clinics for children in the UK, which can be found either through the British Lymphology Society’s online Directory (www.thebls.com/directory/index.php ) or by telephoning the Lymphoedema Support Network on 020 7351 4480.

Secondary lymphoedema can be caused by cancer treatment when there is damage to a previously healthy lymphatic system. Cancer surgery to remove the tumour and nearby lymph nodes can be a risk factor for developing lymphoedema in the surrounding area nearest to the tumour (For example, in breast cancer lymphoedema can develop in the arm, breast or chest wall of the affected side) If radiotherapy is given to the remaining lymph nodes which supply the surrounding area (in breast cancer this might be the lymph nodes under the arm) then this may further increase the risk. Radiotherapy can cause further damage to lymph nodes, vessels and the surrounding tissues.

"Lymphoedema is most commonly associated with breast cancer"

Lymphoedema is most commonly associated with breast cancer however occurs as commonly in people who undergo cancer treatment for gynaecological (female reproductive system), urological (penile, prostate & some bladder cancers), melanoma (a form of skin cancer) and cancers affecting the head, neck and mouth. If a person has had lymph nodes removed following cancer treatment they should be given the correct information to protect against developing lymphoedema. Many clinical nurse specialists & other health professionals, such as doctors or physiotherapists, will routinely give this information to patients.

Lymphoedema can develop at any point following cancer treatment but has been shown to usually begin in the first 18 months following cancer treatment (Williams, Franks & Moffatt 2005) Some patients who have not had lymph nodes removed may develop the condition (for example, following surgery and radiotherapy for a bone tumour in the leg). The exact physiological process is still under debate as many factors may be in part responsible.

Some people may be more at risk than others for developing the condition but trauma and infection to the ‘at risk’ area remains the identified overall largest hazard. For lower body lymphoedema being overweight or obese may place the lymphatics under greater pressure and mean a greater risk of developing lymphoedema as the lymphatic vessels are placed under greater strain.

In practical terms patients should be given the correct preventative advice to avoid trauma to the ‘at risk’ area for example, not having blood tests or blood pressure readings taken on the arm following breast cancer treatment, or when lymph nodes have been removed from under the arm to treat melanoma (a form of skin cancer). The Lymphedema Network based in the United States has additional information regarding risk reduction:
www.lymphnet.org/pdfDocs/nlnriskreduction.pdf

Further information regarding risk factors and prevention advice for developing lymphoedema is contained in the ‘Preventative advice ‘ section.

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Causes of secondary lymphoedema:

Secondary lymphoedema & chronic oedema can also occur in several other medical conditions such as Rheumatoid Arthritis, Multiple Sclerosis or conditions which mean that a person is less mobile and spends a lot of time sitting with their feet on the floor. Trauma such as severe skin infections (cellulitis) can put the lymphatic system under pressure and there is some debate as to whether those who develop swelling as a result of these things may actually have an underlying fault in the lymphatic system, but the condition or additional trauma caused the swelling to start.

Another cause of lymphoedema which is more common in the developing world is a tropical parasite which causes widespread damage to the lymphatic system and can cause ‘elephant’ like limbs is Filariasis. This is a mosquito borne parasite which is transmitted from person to person through a mosquito bite. There are treatments available to prevent such destruction of the lymphatic system but filariasis remains a major global problem for 83 countries in the developing world affecting at least 120 million people already.. More information can be found at: www.filariasis.org/resources/lymphoedema.htm

"people living with advanced cancer.....sometimes experience chronic swelling of the legs"

Chronic oedemas can develop when there has been damage to the lymphatic and/or vascular system and can occur for many reasons. For instance, people who have trouble walking, problems with veins in their legs or other chronic conditions can develop a degree of chronic oedema. It is now commonly accepted that chronic oedema although may not be strictly ‘true’ lymphoedema in physiological terms, is a mixed form of the condition and will lead to deterioration of lymphatic function, ultimately resulting in lymph – oedema.

In addition, some people living with advanced cancer or terminal medical conditions sometimes experience chronic swelling of the legs or large areas of the body. This can be an additional distressing symptom and it may be appropriate for them to be assessed by a lymphoedema specialist who could offer treatment and advice, especially those who may experience leaking lymph fluid (lymphorrhea) in the swollen area. Treatment can be adapted to suit the individual who may be experiencing other symptoms.

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Signs and Symptoms of Lymphoedema & Chronic Oedema:

Lymphoedema can occur in any area of the body depending on the cause. If lymphoedema occurs as a result of cancer treatment the ‘at risk’ area will be where the cancer has been removed or treated and the immediate surrounding area. For example, breast cancer treatment may result in arm, breast or back swelling. Some women after gynaecological cancer treatment notice swelling in the inner upper thigh which may be lymphoedema. More commonly, however, lymphoedema presents as swelling of an arm or leg only and for many the swelling will be minimal.

The following symptoms have been described by patients and may be a sign that lymphoedema is developing:

• Feeling of tightness and heaviness in the limb – noticing that shoes, watch or clothing feels tighter around the limb
• Measurable/noticeable swelling of affected area – slow to develop (never sudden)
• Swelling does not reduce overnight after being in bed or after resting legs on a footstool
• Skin feels tight/bursting/ burning
• Constant or intermittent dull aching or pain in the affected area
• Spontaneous skin blisters and/ or leaking clear fluid from swollen area
• Often the area feels ‘wood-like’
• Skin changes such as thickening of skin, inflammation, fibrosis or in more severe cases hyperkeratosis and papillomas can occur
• A tendency towards infection in the form of cellulitis (skin infections) or fungal infection (affecting toe nails or between toes ‘athletes foot’)

(Adapted from Twycross 2000)

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Staging of lymphoedema:

Adapted from Best Practice Framework Document, Lymphoedema Framework Project.

There are different stages or classifications of lymphoedema depending on how much swelling is present and the condition of the underlying tissues and skin. The majority of lymphoedema is described as ‘mild’ to ‘moderate’ and can be managed without any need for intensive treatment. More severe presentations can be classified as ‘moderate’ to ‘severe’ and these severe cases may often be referred to as elephantiasis.
Further information on staging and all care aspects of lymphoedema can found in the document entitled: Best Practice Framework Document, Lymphoedema Framework Project at the publishers website: www.mepltd.co.uk/oneoffsdetail.html?p=bpml

"approximately 20% of people treated for the high risk cancers may develop lymphoedema"

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The extent of the problem: Incidence of lymphoedema

A recent study (Moffatt et al 2003) found that there were at least 100,000 people in the UK living with lymphoedema and chronic oedema. When compared to the incidence of leg ulcers in the UK which is 75,000 people, the authors have successfully highlighted that lymphoedema is a common and unrecognized problem.

It should be noted that approximately 20% of people treated for the high risk cancers (listed below) may develop lymphoedema (National Breast and Ovarian Cancer Review 2008) and it is not clear why some people develop lymphoedema rather than others. Following treatment for cancer the incidence of lymphoedema has been varies and some suggested percentages are below:

• Breast cancer: 6 – 49% (Swenson et al 2002)
• Gynecological cancers (vulval, cervical & ovary): 18 – 41% (Sneddon & Lewis 2007)
• Melanoma (skin cancer): up to 41% (Wrightson et al 2003)
• Prostate cancer: up to 66% (more common in those living with cancer – Williams, Franks & Moffatt 2005)
• Bladder cancer: 13 – 20% (Williams, Franks & Moffatt 2005)
• Bone and soft tissue sarcomas: 20 – 30% (Williams, Franks & Moffatt 2005) (NB. This figures represent patients who have undergone radiotherapy high dose treatments)

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Sentinel lymph node biopsy and cancer surgery: risk of lymphoedema

In cancer surgery lymph nodes are frequently removed at the time of surgery. The sentinel lymph nodes are the lymph nodes which are nearest or directly drain the fluid nearest to where the cancer tumour has been found. These lymph nodes, instead of all the lymph nodes are removed and tested for cancer cells. If no cancer is found then no further lymph nodes need to be removed. If cancer is found then further surgery may be required to remove more lymph nodes. The technique of testing the sentinel lymph nodes greatly reduced the risk of lymphoedema and other side effects of surgery and is key to identifying cancer spread.

Reassuringly rates of lymphoedema are much lower if only the sentinel lymph nodes are removed as opposed to many or all of the lymph nodes (often referred to as complete/total/axillary or inguinal lymph node dissection)

Continuing research is underway for a better picture of the long term risk of lymphoedema following sentinel lymph node biopsy. The risk appears to be lower following sentinel lymph node biopsy in the axilla (armpit) region than for patients needing the same procedure in the groin (inguinal) lymph nodes. Groin lymph nodes are deeper in the body and when removed surgically require deeper and larger incisions causing more damage to the lymphatics in the region and a larger number of lymph nodes may need to be removed in some cancers.

There are additional factors that may affect the patients’ risk for developing lymphoedema. Pre operative and preventative advice following surgery regardless of which lymph node removal technique is used should be made widely available for all patients.

Further information regarding the risk and self care for cancer patients is in the Prevention advice section.

"In rare cases.....a scan called Lymphoscintigraphy is required."

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Diagnosing Lymphoedema

People who have suspected lymphoedema or chronic oedema will require a thorough assessment. This includes information from the patient regarding the history of swelling; past medical history of any illnesses, accidents, operations and medications; what makes the swelling worse/better together with a physical examination of the affected and surrounding area.

Generally there is no need for any tests to diagnose lymphoedema. The information gained from the in depth assessment and physical examination will be sufficient for an accurate diagnosis in adults.

In some cases however, blood tests may be necessary to identify any other contributing factors, and if the person with swelling has been treated for cancer further scans or details may be required from the patient’s cancer specialist.

In rare cases, and where a cause cannot be readily identified, a scan called Lymphoscintigraphy is required. This test is not routine for the majority of people with lymphoedema. It involves the injection of a dye into lymphatic vessels of the affected area (in leg oedema between the toes, in arm oedema the fingers). The scan then traces the lymphatic pathways and the dye moving through the vessels to highlight any abnormality such as missing or incompetent lymphatic vessels or nodes.

DISLCIAMER: this information has been written for the public and health professionals who have an interest in lymphoedema from a variety of reliable sources which are in the public domain. The author is not responsible for how the information is interpreted or used.