Lymphoedema

What is lymphoedema?

The body tissues are bathed in a colourless, watery fluid, called lymph which flows in channels called lymphatics. The lymph nodes drain the lymph channels and filter out, any foreign matter, excess protein, bacteria and other infectious organisms. These are carried in the lymph, where the lymphocytes try to break them down before they are carried by the blood and filtered out along with other body wastes.

If the lymph nodes or vessels become blocked, by a tumour or because of scarring caused by infection, radiotherapy or surgery, the lymph fluid is unable to pass along the channel. Pressure builds up in the channel and consequently the stagnant lymphatic fluid seeps into the surrounding tissues, causing swelling. This is known as lymphoedema. In India the most common cause of obstruction to the lymphatic channels is filariasis, an infection transmitted by the bite of mosquitoes and caused by a worm called W Bancrofti.

What are its causes?

Lymphoedema may occur due to the following reasons:

• Primary (lack of lymphatic vessels): There may be too few lymph vessels or if there are many, they may not pump properly, leading to lymphoedema. This type can occur early in childhood, during puberty, or later in life.
  
  
• Secondary (blocked or cut lymphatic vessels): This is more common and can occur if the lymphatic drainage is blocked or cut due to surgery, especially for cancers; radiotherapy; accidental trauma which may tear the lymphatic vessels; parasites like filarial worms which may cause inflammation and block the system.

Lymphoedema due to filarial infection is common in eastern UP, Orissa, Bihar and Tamilnadu. It often leads to severe swelling of the feet followed by gross thickening of the skin referred to as elephantiasis.

What are the symptoms?

Lymphoedema most commonly occurs in the arms, but if nodes in the groin or trunk area are involved, then it can occur in the legs or chest. The affected part may become swollen, stiff and awkward to move and painful. The skin becomes thick and dry and in some cases, broken or ulcerated.

What are the grades of lymphoedema?

Grade 1: The swelling will pit if the skin is pressed and is reduced if the limb is elevated.

Grade 2: Excess fibrous tissue may develop, so it does not pit. The limb gradually hardens.

Grade 3: The skin gets thick, with huge folds and protrusions, a condition called elephantiasis.

How is the condition diagnosed?

The condition can be easily diagnosed from history and observation in 90% of cases. In some cases however, special methods may be used, like lymphoscintigraphy (a means for measuring the lymphatic function). CT scans may be utilized to image the nodes and show oedema and fibrosis.

How can it be treated?

Lymphoedema can never be completely cured because the damage to the lymphatic vessels is irreparable. Treatment aims at reducing the swelling and preventing further build up of fluid and may include:

• Complex physical therapy (C.P.T.)
  
• Use of drugs like benzo-pyrones.
  
• Compression garments and bandaging.
  
• Intermittent pneumatic compression using a special apparatus and
  
• Massage and special exercises.
  
• A combination of all the above may be best under certain circumstances.

C.P.T. in conjunction with benzo-pyrones achieves the fastest reduction of lymphoedema. The role of drugs however remains uncertain. A course of this treatment lasts from 2 to 6 weeks, depending on the severity of the condition and the number of limbs involved. C.P.T. consists of four main parts:

• Skin care to prevent infection.
  
• A special form of massage each day, which removes the excess fluid and protein and opens unaffected lymphatics, which may drain the affected ones.
  
• Compression bandages and garments may be used to stop the reduced limb from rapidly resuming its former size.
  
• Special exercises to supplement the massage.

The benzo-pyrones aid in the removal of excess protein. They may take several months to years to show their effect but certainly help to prevent infections. The drugs commonly used are coumarin, 5,6 benzo-pyrone and Paroven.

Compression garments when used in conjunction with special exercises, help to maintain the limb size. Exercise should be done in moderation and should be gentle. In case of leg swelling, standing or sitting for long periods should be avoided. Walking on the spot and transferring the weight from the toe to the heel may help. It is very important to seek help from a specialist before starting an exercise programme.

The role of surgery is restricted to those patients who do not benefit from non-operative measures. The most common procedure performed is a lymphnode to vein anastomosis or a lymphatic vessel to vein anastomosis with the help of a microscope so that the blocked area in the lymphatic channel is bypassed. In advanced cases the only option is to remove the excess mass surgically and resurface the area with skin grafts.