Lymphoedema is an abnormal, chronic swelling of a body part/s caused by a collection of lymphatic fluid in the tissues below the skin. It occurs when the lymphatic system has not developed properly or has been damaged (such as by surgery or radiation). It typically occurs in the limbs (legs more so than arms), but lymph fluid can collect in the chest and abdominal cavity as well.
The lymphatic system has an important role in the circulation and it ensures fluid is returned from around each and every cell (interstitium) to the blood. It also has a vital immune role and protects the body against harmful bacteria. Lastly, the intestinal lymph has an important role in fat absorption.
Types of lymphoedema
Primary lymphoedema occurs when the lymphatic vessels or lymph nodes have not completely developed or have developed abnormally before birth. Primary lymphoedema may present at any time and is not always present from birth.
Secondary lymphoedema occurs as a result of damage to or obstruction of the normal lymphatic system, resulting in an inability of the lymphatic system to transport lymph back into the circulatory system. This may be caused by an infection of the lymph, trauma or surgery, venous insufficiency (varicose veins), a blood clot (post-thrombotic syndrome), cancer, obesity, untreated wounds or infections amongst others.
Surgical causes usually result from damage to the lymphatic channels as a side-effect of surgery or radiotherapy for cancer. This is typically seen as a complication following breast cancer surgery, melanoma and pelvic (gynaecological or prostate) surgery where the surgical lymph node removal or radiation in the armpit or groin have damaged the lymph vessels.
Destructive infection of the lymphatics typically results from infection from the roundworm Wucheria Bancrofti. It may also be from Brugia Malayi, and Brugia Timori worms. If the infection becomes chronic, lymphoedema results, known as elephantiasis
Treatment is lifelong as there is currently no cure.
Signs and Symptoms
Early / Acute Lymphoedema
- Fullness or a heavy sensation of the body part
- Painless (typically) or painful swelling
- Tightness in the skin
- Difficulty wearing clothes or shoes
Initially the main pathology is lymph fluid accumulation and this can be drained out the leg with the above techniques.
- Skin changes such as thickened, dry skin with discolouration
- Warty protrusions
- Lymphoceles (lymph leaks through little blisters)
These changes may lead to cellulitis (skin infection) and ulceration.
As the condition becomes more chronic, and if fluid has been sitting within a limb for sometime, the fluid will start to develop into fat tissue. Although decongestive therapy is necessary to continually move fluid that is constantly coming into the leg, the development of fat tissue is currently, irreversible.
Lymphoedema is a chronic condition which is progressive if left untreated. Early diagnosis and treatment will ensure the best possible outcome.
Decongestive Therapy (CDT)
This is performed by a certified lymphoedema therapist working actively with the patient and aims to reduce swelling, then maintain the optimal size and health of the limb with well-fitting, carefully selected compression garments. This is the mainstay of treatment.
It is unproven whether lymphatic treatment is absolutely necessary – in the more advanced stages of lipo-lymphoedema it certainly is. However, most patients report improvement of symptoms with this therapy.
The components of Complete Decongestive Therapy are:
- Manual lymphatic drainage therapy
- Multi-layered compression bandaging and wound care if required
- Meticulous skin and nailcare
- Compression garments
- Rehabilitative exercises to improve lymphatic function, fitness, strength and general health as required
- Self-management with the ongoing support of the treating doctor, lymphoedema therapist and the rest of the multi-disciplinary team
Compression garments and decongestive therapies are lifelong treatment.
There are centres which have compression machines (and home -based ones) which can be used as another option or, preferably, in conjugation with decongestive therapy.
Lymphoedema therapists advise on nutrition for two main reasons – weight management and to reduce fluid accumulation. Many patients with lymphoedema battle to exercise and become more sedentary with resultant weight gain. This is completely preventable and reversible. It is important for ideal weights to be maintained as excess weight will cause more swelling.
Microsurgical techniques include vascularised lymph node transplants and lymphatic-venous anastomoses with the aim of restoring some fluid drainage.
These procedures are for select patients. Outcomes with these procedures vary as there is the risk of blockage or lymphoedema in a donor site. However, if successful, they will help maintain the limb condition.
One drawback is that these will not remove the fatty tissue if it has developed. Thus the leg volume would only be reduced if there was excess fluid.
Compression is still necessary lifelong.
Liposuction is a newer surgical option to remove the fat tissue that has built up in the limb. It needs to be pre-determined what volume of fat must be removed and a strict compression protocol needs to be adhered to pre-and post- operatively. It is more intense and time consuming compared to typical liposuction as there is usually a much greater volume to remove. Thus, power-assisted liposuction is used and special cannulas designed.
Dr Hakan Broroson in Sweden pioneered this technique after proving that lymph fluid does, in fact, turn to fat tissue. (It was previously believed it was all fluid only).