Are there different types of Lymphoedema?

Lymphoedema is divided into two main groups:

1. Primary Lymphoedema

This is lymphoedema that is due to underdevelopment of either your lymph nodes (most common form) or lymphatic vessels during embryonic development. Rarely, it may also be due to hyperplasia of lymphatic vessels. There is often, but not always, a family history of lymphoedema. In rare cases, primary lymphoedema is also associated with other genetic syndromes and symptoms.

Primary Lymphoedema (that is not related to a genetic syndrome) generally divided into three categories:

Conatal Lymphoedema.

This means that it is present or noticeable at the time of birth, and does not resolve. It is important that the child is seen by a doctor, as not all oedema or limb size difference is lymphoedema. Differences in the size of limbs may also be caused by malformation of blood vessels, or other reasons.

 


This child’s lymphoedema was evident from birth.
Primary Lymphoedema (conatal ie present at birth)

 

 

 

 

 

 

 

 Lymphoedema Preacox:

This is when lymphoedema is first noted around puberty for the first time. Usually it begins slowly, and may fluctuate on a daily basis. However with time it progresses and remains swollen. If untreated it becomes progressively worse.

Lymphoedema Tarda:

This is when the lymphoedema is first seen when a person is in their mid-thirties or later. It is not well understood why it would only present at this time, even though the underdevelopment of lymph nodes has been present since birth. Again, it usually starts slowly, and may fluctuate initially but then the swelling remains and worsens over time (if left untreated).

2. Secondary Lymphoedema

This is lymphoedema that has been caused by external injury to the lymphatic system.

Some examples include:

  • Removal of Lymph nodes for the treatment of cancer
  • Radiation of lymph nodes and vessels
  • Malignancy of the lymph nodes and/or vessels
  • Trauma and injury to the lymphatic vessels
  • Vascular or circulatory disorders
  • Filariasis (a mosquito born parasite)
  • Recurrent Infection

Removal of Lymph Nodes

Lymph nodes may be surgically removed if they are malignant, or if they carry a very high risk of malignancy. This is most often seen in surgery for breast cancer, melanoma and some other cancers. It is important to note that not everyone that has lymph nodes removed get lymphoedema. Although statistics vary considerably, it is generally accepted that in patients with breast cancer, only about 20-25% of people who have lymph nodes removed and radiation actually get lymphoedema. However if you have had lymph nodes removed you are at a high risk of developing lymphoedema. In more recent years with the advent of the sentinel node biopsy the rate of lymphoedema has reduced for patients that have no more than the sentinel node removed, and no radiation of lymph nodes. However there now appears to be a reduction in the incidence of arm lymphoedema, but an increase in breast lymphoedema. There are currently no statistics available on the incidence of lymphoedema in other cancers.

Secondary Lymphoedema following breast cancer

Secondary Lymphoedema following breast cancer

 

 

 

 

 

 

 

 

Secondary Lymphoedema affecting the whole arm and hand

Secondary Lymphoedema affecting the whole arm and hand

 

 

 

 

 

 

 

 

Lymphoedema02

Secondary Lymphoedema of the leg following removal of the inguinal (groin) lymph nodes.

 

 

 

 

 

 

 

 

Radiation Therapy

Although radiation may be a very important and life-saving treatment, it also may affect the lymph nodes and lymphatic vessels. Lymph nodes that have been radiated usually become scarred and fibrosed and are unable to perform their function adequately. The risk of developing lymphoedema is increased if lymph nodes and pathways are radiated

Malignancy involving the lymph nodes or vessels

Malignancy that invades or metastasizes to lymph nodes mostly renders the lymph node functionless, with one of the results being the development of lymphoedema. Cancer involving the lymphatic vessels ( eg lymphangiosarcoma) is fortunately very rare, but generally also results in lymphoedema in the affected area

Trauma and Surgery

Where injuries result in damage to lymphatic pathways and vessels, and if the body is unable to establish collateral (alternative) pathways lymphoedema may result. This includes tissue injuries, burns, crush injuries, some surgical procedures.

Filariasis

This is the most common cause of lymphoedema in the world. It is a mosquito born parasite that results in damage to the lymphatic vessels close to the lymph nodes. It is mostly seen in the legs, due to damage caused to lymphatic trunks near to the groin. It is endemic in certain countries, mostly in the tropical and equatorial regions in the world. The World Health Organisation has a programme to target the eradication of Filariasis.

Phlebolymphoedema

This is where there is a combination of vascular (circulation) and lymphatic causes, resulting in oedema.
This form of oedema is very common. It begins as a result of venous insufficiency, ie when the veins are unable to completely and easily pump the blood form you lower legs back up to your heart. Swelling is usually noted around the ankles and lower leg. Initially the lymphatic systems can compensate for this by increasing the amount if fluid it is draining. However, after a while, even the lymphatic system cannot compensate, becomes overloaded, and results in further swelling. In this situation there are aspects of the swelling related to circulation and aspects related to lymphatic overload, therefore it has the combination name of phlebolymphoedema. This form of oedema is very responsive to treatment.

Some patients who have had a DVT (deep vein thrombosis) they may also develop swelling in the affected limb (usually the leg, but
sometimes in the arm). This is called Post Thrombotic Syndrome and also requires treatment if it does not resolve. Manual Lymphatic Drainage Massage is delayed, but compression is initiated soon after the DVT is resolved.

Phlebolymphoedema. Phlebolymphoedema. This type of oedema is a combination of vascular and lymphatic problems.This type of oedema is a combination of vascular and lymphatic problems.

 

 

 

 

 

 

 

 


Phlebolympholymph10edema:This person has long standing, untreated oedema due originally to a vascular/ circulation disorder. Initially simple oedema or swelling will be present. However if this swelling persists untreated (ie without adequate, long-term compression and oedema management), significant skin and tissue changes result. In this case there is significant skin thickening and fibrosing (lipodermatosclerosis )resulting in the wine glass shape of the leg .Other trophic changes are also evident with discolouration and venous excema.

 

 

When Does Secondary Lymphoedema Occur? 

Not everyone who undergoes the surgical removal of lymph nodes or radiotherapy develops lymphoedema. However once the lymphatics are damaged, lymphoedema can occur at any time.

Sometimes it appears immediately after surgery, and sometimes it appears for the first time many years later. Occasionally it may occur for a few weeks and then resolve, but more often it lasts a lifetime. Once the lymphatics are damaged, sometimes only a minor incident can set it off e.g. a bee sting, carrying something heavy, sunburn etc. If lymphoedema appears for the first time many years after the original surgery/ radiation therapy, it is important to consult your doctor who will do the relevant medical tests as very late onset lymphoedema may be caused by recurrent malignancy. It is important to note that this is not the only cause of late onset lymphoedema, but should be investigated.

If left untreated lymphoedema usually worsens over time. This process is usually slow in primary lymphoedema, but can be rapid in secondary lymphoedema. However it can usually be brought under control by good treatment and an ongoing commitment to home management by the client.

What are some of the other types of swelling?

Swelling from Injuries, Surgery or Trauma

This is swelling that is a necessary part of your body’s normal injury and healing response. In these situations the lymphatic system is generally intact, or if injured has formed collateral lymphatic pathways. This swelling/ oedema mostly resolves spontaneously, but can be problematic if it lasts longer. It may also persist where the injury has damaged lymphatic vessels or pathways resulting in lymphoedema.

Other diseases

There are many diseases and condition that also present with swelling. These may include other circulation (vascular) problems, kidney disease or dysfunction, hormone and thyroid problems, cardiac problems and many others.

Medication

A side effect of some medication is fluid retention or swelling.

Lipoedema

This is a condition of abnormal lay down of fatty tissue mostly in the legs. It does have a component of oedema, but is mostly fatty tissue. It is not related to diet. In most cases the legs, hips and ankles are involved, but the foot is spared. It is also usually characterized by tenderness or pain. At times where there is a relatively large oedema component or associated lymphatic dysfunction the condition may be called a lipolymphoedema.

 

How many people get Lymphoedema?

There is a very wide range of statistics as to how many people are affected by Lymphoedema. What we do know is that it is more common than is usually realised. As detection of cancer becomes earlier, and surgical treatments advance and improve, the incidence of secondary lymphoedema is decreasing, however worldwide it is still a significant problem to many people. The reason that there are such wide-ranging statistics is partly because there are no clear, uniform standards internationally for measuring Lymphoedema. Also, since Lymphoedema may only develop years after the original surgery/treatment, often the patient is no longer in contact with their original doctor/radiologist/oncologist. Another factor is that many people don’t report their lymphoedema or believe (or may even be told by the doctor) that there is nothing that can be done for it. Sometimes doctors don’t believe that the swelling is lymphoedema (or that treatment is necessary) unless the arm/leg/breast is really big. Sometimes it is not recognised if it is in the trunk or in the breast tissue.

In 2000, the World Health Organization estimated that approximately 140 million people suffer from lymphoedema world wide.

Approximately one person in 6000 will develop primary lymphoedema (ie due to underdevelopment of lymph nodes or vessels during embryonic development).

Statistic vary considerably regarding the incidence of lymphoedema in breast cancer patients, but it is generally accepted o be between 20-30 %. In gynaecological cancer it is generally seen in between 30-40 % of patients.

Filariasis is a parasitic infection that that results in damage to lymphatic vessels, and therefore lymphoedema is a major cause of lymphoedema world-wide. It is mostly seen in areas of the world with a tropical climate.

 

What are the risk factors for getting lymphoedema?

It is very difficult to predict who will develop lymphoedema, and the relative importance of each of the risk factors is not fully understood. However it is understood that certain factors do seem to increase a person’s risk of developing lymphoedema.

The information for this risk profile was referenced form “Best Practice for the Management of  Lymphoedema”, International Consensus, London: MEP Ltd 2009

 

  • Upper Limb/ Trunk Lymphoedema
    • Surgery with axillary node (lymph nodes in the armpit) dissection, especially if the breast surgery was extensive or if many lymph nodes were removed. The exact site of surgery in relation to the lymphatic pathways also appears to be important.
    • Scar tissue and fibrosis following radiotherapy to the axilla (armpit).
    • Radiotherapy to the breast, axilla or the nodes above and below the collarbone.
    • Infection or complications with wound healing or the drain following surgery.
    • Advanced cancer
    • Obesity
    • Trauma to an arm that is at risk of developing lymphoedema (eg injections, taking blood from the “at risk” arm, taking blood pressure measurements form the “at risk” arm.
    • Some chemotherapy (eg Taxanes)
    • Air travel (if a person is already at risk)
    • Chronic skin disorders and inflammation
    • Living in or visiting an area where Filariasis is endemic.

 

  • Lower Limb Lymphoedema
    • Surgery with inguinal node dissection (ie removal of lymph nodes in the groin)
    • Radiotherapy to the pelvis
    • Recurrent infection around the site.
    • Obesity
    • Varicose vein stripping or vain harvesting
    • Family history of chronic swelling
    • Advanced cancer
    • Tumours that compress directly on lymphatic vessels
    • Some orthopaedic surgery
    • Disorders of circulation
    • Chronic skin disorders and inflammation
    • Other illnesses such as cardiac (heart) disease, kidney disease, overactive thyroid
    • Immobilisation of the leg
    • Air travel
    • Living in or visiting an area where Lymphatic Filariasis is endemic.

 

 

How is the severity of Lymphoedema graded?

Lymphoedema can be graded into different levels of severity. Without adequate treatment Lymphoedema usually progresses and worsens over time. Below is the International Society of Lymphology’s (ISL) system of grading the severity and progression of Lymphoedema. A second form of grading is often used together with the ISL’s system, and this involves measuring the size of the affected limb, and comparing it’s size to the unaffected limb

  • Grade 0

Swelling is not yet evident despite impaired lymphatic function. This stage may exist for months or years before oedema becomes evident.

  • Grade 1

Oedema is present, but mostly disappears with elevation (eg when sleeping overnight). Oedema is usually pitting (that means if you push you finger onto the affected area it leaves a dent in your skin that does not immediately resolve when you remove your finger).

  • Grade 2

Oedema is present, and does not fully resolve with elevation. It may show slight pitting, but only superficially. Fibrosis may be present.

  • Grade 3

Oedema is present all the time, and does not resolve with elevation. Tissue becomes fibrotic and hard, and pitting is absent .Skin changes begin to be noted. These may include changes in the colour of the skin, a mossy appearance of the skin, skin folds, fatty deposits and warty outgrowths.

Grade Three Lymphoedema- Note the skin changes, large skin fold around the ankle and in this case severe oedema.

Grade Three Lymphoedema- Note the skin changes, large skin fold around the ankle and in this case severe oedema.

 

 

 

 

 

 

 

 

Grade Three Lymphoedema- Skin changes are evident with disclolouration, papillomata and thickening. Note also the beginning of skin folds developing at the ankle.

Grade Three Lymphoedema- Skin changes are evident with disclolouration, papillomata and thickening. Note also the beginning of skin folds developing at the ankle.