1. What is Lymphoedema?

Lymphoedema is a chronic abnormal swelling of a limb due to accumulation of lymph in the subcutaneous tissues. The lymph is fluid containing proteins which develops normally in the tissues and is drained back into the circulation by specific channels, the lymphatics, which run parallel to the veins. If the lymph that is normally produced cannot be cleared adequately, swelling will ensue.

However, limb swelling is more often caused by cardiac, renal, or hepatic disease; swelling caused by venous abnormalities is far more common than lymphoedema; lymphoedema is most often caused by acquired conditions. In other words, primary [congenital] lymphoedema is rare.

2. What causes lymphoedema?

It is caused by acquired destruction of the lymphatic channels or lymph nodes [surgery, infections], or congenital absence / valvular abnormalities of the lymphatics.

Primary, congenital lymphoedema

-   Congenital aplasia or hypoplasia of the lymphatics [Milroy’s disease, is the inherited form]

-    Abnormalities of the lymphatic ducts

-  Incompetence of valves with distention [lymphangiectasis] of the lymphatic channels.

-   Infections [like filariasis, a tropical disease caused by parasites] or even common forms of cellulitis

-  Surgical damage or excision of the lymph nodes [especially after breast procedures]

-    Radiation therapy for cancer that obliterates the lymphatics

-    Invasion of the lymphatics by widespread cancer

3. When does the swelling occur in primary lymphoedema?

For primary lymphoedema, the swelling may appear either early [praecox] or late [tarda], after the 35^th year, depending on the underlying abnormality. In cases of congenital aplasia, it is evident within two years from birth. However, in most cases it appears during the second decade of life, affecting usually women.

The degree of swelling varies, and from the clinical point of view, it can be mild, that can be managed conservatively throughout life, moderate, lymphoedema of the whole limb but with healthy skin, and severe gross lymphoedema of the whole limb with or without healthy skin.

4. What can lymphoedema cause apart from the swelling?

The presence of lymph stasis may, in due course, cause scarring and induration of the subcutaneous tissues, with the skin becoming hard, stiff and scaly. The risk of superficial skin infections increases.

Apart from the cosmetic appearance, gross oedema of the limb causes a considerable handicap [weight].

Skin changes appear after years [usually in severe cases] and may consist in pigmentation, hyperkeratosis, lichenification and even ulceration.

5. How is lymphoedema diagnosed?

The progressive swelling of the limb [slow onset, but may be precipitated by minor injuries or insect bites] with tightness but no pain, accompanied by attacks of cellulitis and gradual skin hardness is characteristic.

Diffuse, long standing, hard, pitting swelling that doesn’t spare the feet with hypertrofied, lichenified skin is the picture of lymphoedema.

A simple ultrasound scan [colour flow duplex] of the veins is probably the first step, as it can detect if there is thrombosis and can also check the condition of the veins and if their valves are competent [as venous causes of swelling are far more common than lymphoedema]. If the oedema is maximal at the ankles and legs and the feet are spared, the cause is more likely to be venous.

.  Lymphoscintigram: a special isotope scanning, is probably the easiest method, done on outpatient basis.

.  Lymphangiography: it can be done at the X-ray suite or even after a small incision and cannualtion of the distal lymphatics; a dye is inserted into it, taking pictures of the lymph vessels. It is used more often in cases where surgery is under consideration.

6. How can the lymphoedema be treated?

For lymphoedema cases, there is no way to cure it but an active and enthusiastic approach can give you a great benefit. More than 95% of the patients are and should be treated conservatively, paying specific attention to the following:

.    Leg elevation [even at night]

.   Elasticated graduated compression [grade IV [50-60mmHg] elasticated compression stockings]

. Pneumatic compression, with daily or continuous night use is quite effective [usually in-hospital but can be done at home]. The best device is the one that uses timed centripetal compression.

.   Massage, in a centripetal direction.

.  Foot hygiene to prevent infection, injuries and cellulitis. This measure is essential, as infections and cellulitis deteriorate the skin and make matters worse. Patients should apply anti-fungal powder between the toes and take antibiotics early in case of infection [cellulites].

Surgical treatment

Surgery is reserved for cases with gross swelling, limitation of movement, hypertrophic skin changes, recurrent cellulites and eczema. Only a few patients [<10%] will benefit from operation, but this must not be done only for cosmetic reasons. The various bypass procedures that have been tried over the years were not shown to be effective.

In general, there are two types of excisional reducing procedures:

-    The Hommans procedure, with excision of the subcutaneous tissue and some of the redundant skin, used only in patients with intact skin, and

-   The Charles procedure, with excision of the subcutaneous tissue and skin, used in cases with scarred, hyperkeratotic or ulcerated skin. The limb is then covered by skin grafts taken from healthy areas Unfortunately, the foot skin cannot be removed and if it is affected it will remain swollen.

However, patients should know that all reducing operations are palliative, they leave scarring and further surgery may be required in the future.

The aim of surgery is to excise the subcutaneous tissue and the affected skin, in order to reduce the limb weight and give a better cosmetic appearance.