Lymphoedema

A transport disorder of the lymphatic system : Lymphoedema (swelling) occurs when lymph flow is impaired. Either damage, obstruction or absence of vessels can lead to dysfunction of the lymphatic system. This can result in the accumulation and stagnation of lymph in the tissues. Most often limbs are affected, but lymph oedema can also occur in the face, neck, abdomen or genitals. Tissues with lymphoedema are at risk of infection and susceptible to progression. Lymphoedema is a treatable and manageable condition.

Types of Lymphoedema and Causes:

1. Developmental (inherited) disturbance of the lymphatic system (primary lymphoedema
2. Acquired damage of lymphatic vessels and/or lymph nodes (secondary lymphoedema)

1. Primary Lymphoedema. 

Primary lymphoedema occurs in women more often than men. In most cases it is found on one side and is pronounced more distal. If it occurs on both sides, there is normally and asymmetry. Primary lymphoedema can be further classified according to the type of occurrence (congenital: already manifest at birth – lymphoedema praecox: occurring before the age of 35 – lymphoedema tardum: occurring after the age of 35)

2. Secondary Lymphoedema 

Secondary lymphoedema can occur following surgical removal of lymph nodes or radiation therapy in the treatment of cancer. Secondary lymphoedema can also occur as a result of the following:

• Post-operative (e.g. after plastic or venous surgery).
• Post-traumatic (e.g. trauma which leads to injury of large lymph collectors such as open fractures, burns, wounds).
• Post-inflammatory (e.g. rheumatic diseases, sinusitis, recurrent phlebitis).
• Post-infection (e.g. recurrent cellulitis, inflammation of the lymph vessels, inflammation of lymph nodes).

Stages of Lymphoedema

Lymphedema progresses through stages, and treatment intervention in early stages (stage 0 and stage I) has been shown to result in very good treatment outcomes if managed appropriately

There are four stages of lymphoedema

Stage 0  (latent  or subclinical )

In this stage the transport capacity of the lymphatic system is reduced, but the remaining lymph vessels are sufficient to manage the flow of lymph, and swelling is not visibly present.

Stage 1 (spontaneously reversible):

Tissue is still at the “pitting” stage, which means that when pressed by fingertips, the area indents and holds the indentation. Usually, upon waking in the morning, the limb(s) or affected area is normal or almost normal size.

Stage 2 (spontaneously irreversible):

The tissue now has a spongy consistency and is “non-pitting,” meaning that when pressed by fingertips, the tissue bounces back without any indentation forming). Fibrosis found in Stage 2 lymphedema marks the beginning of the hardening of the limbs and increasing size.

Stage 3 (lymphostatic elephantiasis):

At this stage the swelling is irreversible and usually the limb(s) is/are very large. The tissue is hard (fibrotic) and unresponsive; some patients consider undergoing reconstructive surgery called “debulking” at this stage.

When lymphedema remains untreated, protein-rich fluid continues to accumulate, leading to an increase of swelling and a hardening or fibrosis of the tissue. In this state, the swollen limb(s) becomes a perfect culture medium for bacteria and subsequent recurrent lymphangitis (infections). Moreover, untreated lymphedema can lead into a decrease or loss of functioning of the limb(s), skin breakdown, chronic infections and, sometimes, irreversible complications. In the most severe cases, untreated lymphedema can develop into a rare form of lymphatic cancer called Lymphangiosarcoma (most often in secondary lymphedema).

Treatment: According to the International Society of Lymphology, Combined Decongestive Therapy (CDT), also known as decongestive lymphatic therapy (DLT), is the treatment of choice for lymphoedema.

COMBINED DECONGESTIVE THERAPY

1. Manual Lymph Drainage – Patients receive Manual Lymph Drainage (MLD) to remove excess fluid and protein from the tissues. The MLD is performed to open lymphatics in the unaffected regions so these can help to drain the affected area. MLD stimulates lymphangions to increase their activity, which results in a decompression and emptying of obstructed lymphatic channels.

2. Compression Therapy – Multi-layered bandaging of the affected limb follows each MLD session. This is a precise and accurate procedure using specific bandages and interfacing materials.The bandages are applied exactly to conform to the patient’s tissues and are reapplied on a daily basis. They are short-stretch bandages that resist muscle contraction and are applied with comfortable padding underneath. The bandages help to maintain the reductions achieved with MLD and may even cause further reduction.

3. Exercise – Effective lymph flow depends on sufficient muscle and joint activity, especially if the functionality of the lymphatic system is compromised. Decongestive exercises are most effective if performed while the patient wears compression garments or bandages, which are also essential components in lymphedema management.

4. Skin Care and Hygiene – Good skin care plays an essential part in the treatment of lymphoedema. Daily skin cleansing with antibacterial washes and neutral balanced pH lotions will help to eliminate possible bacterial and fungal growth and so minimise the possibility of repeated attacks of cellulitis or lymphangitis.

5. Breathing – The lymph transport in the Thoracic Duct (Ductus Thoracicus) is mainly caused by the action of breathing. Taking into consideration that this duct transports 4 litres of lymph per day explains the importance of diaphragmatic breathing to help increase transport of lymphatic fluid.