Lymphoedema

What is Lymphoedema?

Lymphoedema is the gradual swelling of a limb as a result of the accumulation of tissue fluid (lymphatic fluid), due to the progressive failure of lymphatic system. There are two types of lymphoedema : Primary lymphoedema and secondary lymphoedema.

Primary Lymphoedema

This is a rare disorder, occurring in about 1 in 100 000 people. Females are affected 2 to 5 times more often than males. Rarely, it may run in families, but it usually occurs sporadically and the cause is unknown. There are three ages groups which may be affected :

  • Birth to 2 years : This disorder is known as Milroy’s disease and is due to failure of lymphatic development—these patients are born without lymphatic vessels.
  • Puberty and early twenties : This disorder is known as Meige’s disease and is due to abnormally small lymphatic vessels, which work during childhood, but become obliterated during puberty possibly due to oestrogen.
  • Later thirties : This disorder is known as lymphoedema tarda and is due to enlarged and incompetent lymphatic vessels.

Seconday Lymphoedema

This is due to another underlying medical or surgical condition such as malignancy, surgery, radiation therapy, infection or other trauma.

What does lymphoedema look like?

Initially there is only oedema (swelling due to fluid) which begins distally and progresses proximally and is worse on standing. There are three stages of lymphoedema, depending and the degree of tissue change – as classified by the International Society for Lymphology:

Stage 1 (Spontaneously reversible)

There is fluid accumulation in the tissues, which reduces at night.  The swelling is soft and pitting (when pressed by a finger the area indents and holds the indentation), and there are few skin changes present.

Stage 2 (Spontaneously irreversible)

The swelling becomes increasingly hard and scarred and thus pitting is no longer as obvious. Infections become frequent and the skin deteriorates, becoming more dry and scaly. Over a period of time, the limb gradually increases in size as scar tissue and abnormal fat enlargement occurs.

Stage 3 (Lymphostatic elephantiasis)

The limb becomes seriously deformed as volume increases dramatically; there is further deterioration in the skin and nails with gross scarring and frequent cellulitis (infections) and sinus tracts. Ulceration may also occur.

How is the diagnosis of lymphoedema made?

Clinical diagnosis is often sufficient. Duplex Ultrasound, MRI and CT as well as lymphoscintigraphy and lymphangiogaphy may be used to confirm the diagnosis where some doubt exists. The need for biopsy is rare.

What treatment is available?

  1. Dermatological Care

The goal of skin and nail care is to avoid bacterial and fungal growth and prevent infections. Skin care treatment involves using a low-pH, alcohol and fragrance free moisturizer to supply moisture to the dry skin, to improve skin suppleness. Nail care involves not cutting the cuticles back to avoid skin damage, as this could serve as an entrance for bacteria. Antibiotic use is controversial. In the early stages it can be given to prevent further damage, but once sinus tract formation occurs, it is of limited use.

  1. Physiotherapy

Lymphoedema can be treated using “Complete Decongestive Therapy (CDT)” – this is made up of Manual Lymph Drainage (MLD), Compression Therapy and Decongestive Exercises. This is the mainstay of treatment.

Manual Lymph Drainage is a gentle massage treatment that improves the activity of the lymph-vascular system. It re-routes the lymph flow around the blocked areas into more centrally located healthy lymph vessels. To avoid re-accumulation of fluid that has been cleared during the drainage session it is necessary to apply compression therapy to the affected area after and between MLD treatments. This is achieved with the use of bandages, padding materials and compression garments. Decongestive exercises are use to improve lymph circulation. Optimal joint range and muscle strength are also important. Finally, increased thoracic duct drainage through diaphragmatic breathing exercises is encouraged.

  1. Medical Therapy

Unless a specific bacterial or parasitic infection is being treated most of the medication used is of little value. Benzopyrones (Coumarin, Oxerutin, Cyclo-3, Troxerutin) have been unable to show any significant improvement over baseline. Flavonoids have minimal reports and diuretics are of no help.

  1. Surgical Treatment

Surgical treatment consists of 2 approaches: Debulking operations and Bypass procedures. While there are numerous debulking procedures, in my hands suction lipectomy (liposuction) is the only procedure to produce good results. Liposuction achieves reasonable results with much improved cosmesis. All patients should get post-operative compression. The Charles procedure is a radical operation that has, in my experience, not given my patients satisfactory results and I no longer perform this procedure.

There are numerous bypass procedures, but unless the abnormal lymphatics occur over a relatively short distance, with normal lymphatics on either end, the bypass procedures are difficult to justify. The use of a bypass procedure in primary lymphoedema is, in my opinion, unwarranted.