Lymphoedema

What is Lymphoedema?

There are a number of reasons why tissues of the body swell, such as
trauma to the musculo-skeletal system or malfunction of the thyroid
gland, the cardiovascular system or the lymphatic system.

Oedema is the medical term for a swelling so lymphoedema is the name
given to a swelling that is due to a malfunction or abnormality of the
lymphatic system in a particular part of the body. The oedema consists
of excess tissue fluid and protein molecules, which stagnate in the
tissues. The affected area can become grossly swollen, painful, heavy,
unsightly and incapacitating.

The International Society of Lymphology (ISL) definition of lymphoedema
is as follows:

"Lymphoedema occurs when a reduced lymphatic transport capacity is
overwhelmed by a normal lymphatic load resulting in the stagnation of
protein rich fluid in the interstitial tissues".

The lymphatic transport capacity refers to the ability of the lymphatic
vessels in a particular area of the body to transport lymph to the
draining lymph nodes. This transport capacity can be reduced when, for
example in secondary lymphoedema, lymph nodes are surgically removed
in the treatment of cancer, or when a person is born with a reduced
number of lymphatic vessels, as in primary lymphoedema.

The lymphatic load refers to the amount of lymph that can be
transported through the lymphatic vessels in any given period of time.
The load is variable, being high, for example, during physical activity
and low during inactivity. However, when the lymphatic transport
capacity is normal, oedema does not develop as the lymphatic system is
able to cope with any load which is placed upon it.

The treatment of lymphoedema is designed to improve the transport
capacity and reduce the load.

The lymphatic system may be likened to a city street which, like the
lymphatic system, has a capacity and a load. The street transport
capacity refers to the ability of the street to transport vehicles along it.
This transport capacity can be reduced when, for example, there are
road works taking place or when there is an accident blocking part of the
road. The street vehicle load refers to the volume of traffic that can
travel along the road during any given period of time. This load is also
variable, being high during peak hour traffic and low at relatively quieter
periods.

Lymphoedema is divided into two basic types, primary and secondary.

The cause of primary lymphoedema is still unknown but it is thought to
be due to a birth defect involving impaired lymph vessel and/or lymph
node development. Secondary lymphoedemas are caused by some form
of trauma to the lymphatic system.

(a) Primary Lymphoedema

Primary lymphoedema may be subdivided according to two criteria.

The first classification is based on the age of the patient at the onset of
the symptoms. There are three different types - congenital, praecox and
tarda.

I. Milroy�s Disease

This describes a congenital familial lymphoedema. The oedema is
present at birth or soon after. It accounts for approximately 5-10% of all
primary lymphoedemas and usually affects the legs but can affect the
arms, face and genitalia. It is twice as common in females as in males
and may be associated with other congenital abnormalities.

II. Lymphoedema Praecox

The oedema develops in the second or third decades of life.
Lymphoedema Praecox accounts for approximately 80% of all primary
lymphoedemas, predominantly in females.

III. Lymphoedema Tarda

Lymphoedema Tarda develops after the age of 35 years, also
predominantly in females, making up approximately 10% of all primary
cases.

The onset of the oedema in Praecox and Tarda is triggered by an
initiating factor, which overloads the reduced lymphatic drainage
capacity in the limb, such as trauma or infection. The oedema begins in
the foot and ankle, progressing to involve the whole calf but rarely the
thigh, there being no further progression after the initial years of
development.

70% of primary lymphoedema patients show oedema in one leg. The
other leg may become involved as well, often years later, in the
remaining 30%.

The second classification of primary lymphoedema is dependent on the
degree of malformation of the lymphatics, there being three different
patterns. Aplasia is most frequently seen in congenital lymphoedema and
is characterised by an absence of lymphatic vessels. Hypoplasia
represents the most common pattern and is characterised by either a
decreased number of lymphatics or lymphatics which are unusually small
in calibre. The hyperplasia type shows vessels which are increased in
size and number and have valves which malfunction.

(b) Secondary Lymphoedema

Secondary lymphoedema can develop following a variety of traumas to
the lymphatic system such as wounds, surgery, malignancies or
radiotherapy.

In developed countries, the most common cause is surgical removal of
lymph nodes, with or without radiotherapy, in the treatment of cancer of
the breast or pelvic organs. However, with the increasing use of the
Sentinel Lymph Node (SLN) biopsy in the surgical treatment of cancer,
the incidence of post-surgical lymphoedema is likely to decrease in the
future. A sentinel lymph node is defined as the first node(s) in the
lymphatic chain to receive lymphatic drainage from a primary tumour.
Identification of the SLN allows the detection and surgical removal of the
affected nodes which clinically may have appeared normal.

In developing countries, the most common type is usually filaritic
lymphoedema caused by direct parasitic invasion of lymph nodes and
vessels following a bite by a mosquito which is carrying the filariasis
worm, Wulcheria Bancrofti. The filarial larva is deposited by the
mosquito into lymphatic vessels where it develops into an adult worm.
Months to years later, after the death of the worm, scar tissue develops
blocking the lymphatic vessels. Only mosquitos in filaritic areas such as
India, Malaysia and Thailand carry this worm. The World Health
Organisation has estimated that over 120 million people are affected by
filariasis worldwide.

Onset of Lymphoedema

There is no set period of time for the oedema to be visible, whether the
condition is primary or secondary. It can occur months or years later in
life, triggered by an incident which overloads the lymphatic system in
the limb, such as an insect bite which becomes infected, an injury or a
plane flight, even one of short duration. For weeks, months or years,
the limb, although having defective lymphatic drainage and therefore at
risk of developing lymphoedema, is able to cope adequately with any
trauma, so oedema does not develop. However, there comes a time for
many patients who are at risk when the lymphatic system in the limb is
overwhelmed and is unable to deal with the resultant acute
inflammation. Stagnation of tissue fluid, protein and debris results. This
is the start of visible lymphoedema. If the oedema is only mild, it may
disappear within a few days. It may return a short time later and stay or
it may never return again.

In secondary lymphoedema, the period between the causative incident
and the onset of the oedema is termed the latent phase. In primary
lymphoedema, the latent phase is the period between birth and the
onset of the oedema.

Grades of Lymphoedema

Lymphoedema may be classified into two grades.

Grade I oedema is soft and feels like putty. It pits on pressure, that is,
an indentation is left after finger pressure of a few seconds. The
oedema is largely reduced overnight or by elevation.

Grade II oedema is firm and feels rubbery. It does not pit on pressure, is
not reduced overnight or by elevation and shows varying degrees of
fibrosis. Fibrosis occurs as a result of chronic inflammation after the
oedema has been present for months or years, giving the limb a hard,
rubbery feel. Grade I lymphoedema is therefore usually easier to treat
than Grade II as the oedema is softer and more responsive to massage.
For this reason, it is better for the limb to be treated as soon as
possible after the onset of the oedema in order to bring it quickly under
control and stop it developing further rather than to wait and allow it to
become established before treatment is started. It is possible to have
Grades I and II in the same limb at the same time.

The clinical presentation of a lymphoedematous limb depends on the
type of lymphoedema and the grade of the condition. Subjective
symptoms can include any combination of pain, ache, heaviness,
tightness, discomfort, pins and needles, numbness, itching and burning.
Objective signs can include any combination of oedema, skin changes
such as dryness, scaliness, infection such as cellulitis, reduced joint
range of movement and restricted or reduced activities of daily living.
The amount of oedema is not always a true indication of the severity of
these symptoms. For example, a grossly swollen limb may not ache
appreciably, where as a limb which is only mildly swollen can be painful.
In addition, some patients suffer from psychological effects.

Elephantiasis is considered to be an extension of Grade II lymphoedema
and usually develops in untreated cases of primary lymphoedema and
filarial lymphoedema. It is the result of years of progressive
deterioration of the condition through chronic inflammation of the
tissues, irreversible fibrosis and recurring attacks of infection. It shows
gross oedema, loss of limb shape, thickened skin, ulcers, increase in
body weight, poor posture and reduced activity.

How is Lymphoedema Diagnosed?

The early diagnosis and treatment of lymphoedema is of paramount
importance for the short and long term future of the patient.
Early diagnosis of primary lymphoedema is not always possible. When
the oedema is evident at birth, the diagnosis should be a formality. But
the oedema can develop in later life and often for no apparent reason.
Mild, localised oedema in a limb, usually a leg, is sometimes overlooked
at first by the patient and may be initially noticed by a friend or relative.
Even when it is detected, medical opinion may not be sought due to the
patient believing that it is something minor such as a sprain, a bruise or
an insect bite. If the oedema persists and becomes worse, then the
patient may consult a medical practitioner who will include lymphoedema
as one of the possible causes of the oedema and investigate further.

Early diagnosis of secondary lymphoedema should be straight forward,
particularly if the oedema follows surgery in the treatment of cancer, for
example, in an arm following a mastectomy or a leg following removal of
the uterus or cervix or a prostatectomy.

If the diagnosis is unclear and there is no obvious cause, or the extent
of the condition needs to be determined, a non-invasive nuclear medical
procedure called a lymphoscintigram is required to accurately assess the
capacity of the lymphatic system in the affected limbs.

What is the Treatment of Lymphoedema?

Various treatments have been performed over the years. These may be
classified as invasive and non-invasive.

1. Invasive Treatment

Numerous surgical procedures have been performed in the treatment of
lymphoedema over the last century. Some of the procedures have been
unsuccessful while others have been provided some relief.

The operations performed fall into three groups:

I. Procedures to resect lymphoedematous tissue
Debulking operations have been used to good effect to excise
oedematous tissue, improve function and cosmetic appearance. These
procedures are indicated when there is gross chronic lymphoedema with
fibrosis. Liposuction is now being used to effect reliable improvements
in limb shape.

II. Procedures to improve lymphatic drainage
These procedures have been used in the past to attempt to connect
lymphatic vessels to veins in the early stage of lymphoedema.

III. Procedures to reconstruct damaged lymph vessels
This micro-surgical procedure attempts to connect blocked lymphatics to
functional ones to by-pass an obstruction, but this appears not to have
been used in recent times.

2. Non-Invasive Treatment

The first recorded physical treatment regime may be traced back to
1892 when Winiwarter, a German surgeon, proposed that a combination
of meticulous cleanliness, bed rest, elevation, massage, compression
bandaging and remedial exercises should be used before surgery. This
was published in his book �Die Elephantiasis�.

Almost 100 years later, Dr Michael Foldi published his article
�Conservative treatment of lymphoedema of the limbs� in 1985
describing a treatment which he called �Complex Decongestive
Physiotherapy� consisting of manual lymph drainage (massage), skin
hygiene, compression bandaging and remedial exercises, followed by
application of suitable compression garments. This treatment has been
modified in recent times with the addition of deep breathing exercises.

This combination of modalities has proven to be the most effective
conservative non-invasive treatment worldwide. The title of the
treatment has been changed over the years by some authorities but the
components have stood the test of time. The current title usually used is
�Complex Physical Therapy� (CPT) or Complete Decongestive Therapy
(CDT).

Complex Physical Therapy (CPT)

Foldi designated the treatment of CPT as �Phase I of decongestion� and
the ongoing maintenance in the ensuring years as �Phase II of
optimisation and conservation.�

Phase I is a period of intensive daily treatment given for a variable
period of time, depending on the severity of the condition and the
progress made. Treatment reduces the oedema, improves lymphatic
drainage, softens fibrotic oedema and increases limb mobility, thereby
increasing activities of daily living and quality of life.

The components of CPT are:

1) Manual lymphatic massage with breathing exercises
2) Multi-layer bandaging
3) Skin hygiene
4) Active exercises

1) Manual lymphatic massage
Lymphatic massage is performed to assist the superficial lymphatic
drainage, reduce the oedema and soften fibrotic areas. It is given in a
methodical sequence, starting with massage of the trunk to stimulate
the central body drainage before treating the affected limb. The strokes
are slow, gentle and repetitive and are directed to the appropriate
functional lymph nodes. They may be likened to stroking a cat.

Deep breathing exercises to stimulate the deep central lymph flow
through the two largest lymphatic vessels in the body, the thoracic duct
and the right lymphatic duct, are performed in conjunction with the
lymphatic massage. Deep breathing alternately raises and lowers the
pressure in the abdomen and chest, exerting a pumping effect on all
lymphatic and blood vessels in the trunk. It has been shown that these
pressure changes propel lymph through the thoracic duct in volume
increments proportional to the rate and depth of respiration. This is
known as the respiratory pump.

Massage of the oedematous limb is commenced after stimulation of the
deep and superficial trunk lymphatic drainage. The proximal end of the
arm or leg is massaged first, stimulating the functional lymph nodes in
the armpit or groin, followed by the forearm and hand or lower leg and
foot, depending on whichever limb is affected.

This routine of trunk massage and deep breathing, followed by limb
massage is repeated any number of times during one treatment session,
depending on the therapist�s treatment plan and the progress made.

There are two absolute contraindications to lymphatic massage:

i. Untreated cardiac failure
ii. Renal failure

Relative contraindications are:

i. Malignant lymphoedema
ii. Acute cellulitis

2) Multi-layer bandaging
Bandaging consists of four layers: stockinette, cotton wool bandages,
foam padding and low stretch bandages, applied in that order.
Stockinette protects the skin and absorbs perspiration. Cotton wool
bandages also protect the skin and help to produce a regular cylindrical
shape to the limb by reducing shape distortions and deep skin folds. Low
density foam pads protect potential pressure areas, reduce shape
distortions and soften areas of fibrosis. High density foam is used to
soften and reshape fibrotic areas. Low stretch bandages are used to
apply necessary compression to the limb. They have a high working
pressure and a low resting pressure providing a firm but flexible support
to the limb, causing tissue pressure variations as the muscles contract
and relax against the resistance of the bandages, thereby stimulating
the muscle pump. These bandages are applied according to the Law of
Laplace in order to provide greater pressure at the distal end of the limb
than at the proximal end. Pressure in the tissues is thereby increased,
assisting the passage of oedema fluid from the tissues into the
lymphatic vessels via the lymphatic capillaries, and lymph flow through
the lymphatic vessels to assist drainage. In addition, bandaging helps to
soften fibrosis, restore elasticity to the over-stretched skin and
re-shape the limb.

There are three absolute contraindications to multi-layer bandaging:

i. Advanced peripheral arterial insufficiency
ii. Septic phlebitis
iii. Congestive heart failure

Relative contraindications are:

i. Some advanced skin conditions
ii. Advanced peripheral neuropathy
iii. Sensory disturbances of the skin

3) Skin hygiene
The skin is the largest organ in the body and has several functions. It
supports and shapes the body, regulates body temperature, protects the
body from infection, excretes waste products, prevents tissue
dehydration and is an organ of communication.

Skin changes can be a feature of lymphoedema, particularly in the
chronic stage. These are due to poor skin hygiene, the stagnant oedema
fluid, chronic inflammation and the impaired lymphatic drainage. The
skin can become dry, hard, scaly and thickened, easily succumbing to
infection due to its poor quality.

Recurrent episodes of cellulitis are not uncommon in leg lymphoedema,
resulting in further overloading of the lymphatic system and an increase
of oedema.

Lymphoedematous limbs have an immune deficiency and are, therefore,
more susceptible to infection. Skin hygiene is therefore critical. Daily
skin care should include scrupulous inspection of the skin, application of
a moisturising lotion and treatment of any infection, abrasion or
pressure area.

4) Active Exercises
Contraction and relaxation of muscles in an oedematous limb which is
bandaged or enclosed in a compression garment cause variations in
tissue pressure and activation of the muscle pump. This promotes
lymphatic drainage, reduction of the oedema and further softening of
fibrotic oedema.

Compression Garments

At the completion of Phase 1 of CPT treatment, a suitable compression
garment is fitted to the limb, taking the place of the bandaging. The
garment applies external support which keeps the oedema under control,
assists lymphatic drainage, helps to soften any remaining fibrosis and
maintains the newly acquired shape of the limb.

Compression garments are available in a variety of different brands,
styles and compression ratings and can be fitted from stock sizes or be
custom made for irregular shaped limbs or those which do not fit into a
stock size. Correct choice is critical for the efficient functioning of the
garment and comfort of the patient. Not only must it be the correct size,
it must also be an appropriate compression rating.

Compression ratings are:

Low compression 15-25mm Hg

Medium compression 25-35mm Hg

High compression 35-45mm Hg

Very high compression > 45mm Hg

It is normal practice for two garments to be supplied for each
oedematous limb to allow time for them to be washed regularly. The
effective life of these garments is 4-6 months.

Hydrotherapy

Hydrotherapy provides an ideal medium for lymphoedema patients to
perform their exercises utilising the hydrostatic and physiological
properties of thermal immersion. Water at a temperature of 35 degrees
is pleasantly warm and is termed thermo-neutral as it has no effect on
core temperature.

The gravitational pressure in a body of water increases by 1mm Hg for
each 13.6 millimetres of water depth. This pressure results from the
weight of the water. For each 30cm of water depth, therefore, 22mm Hg
pressure (300mm x 13.6 = 22.05) is exerted on a standing immobile
body such that, at a depth of 1.2 metres, 88mm Hg pressure is exerted
on the ankles. This pressure is approximately double the pressure which
is exerted on the ankle by a compression stocking. Blood is thereby
forced back to the heart from the lower legs and oedema fluid passes
from the tissue spaces into the vascular and lymphatic systems. This
compressive effect is increased when the muscle pump effect is
activated by the performance of active exercises.

Compression Pumps
Compression therapy using various forms of pneumatic pumps has been
a popular form of treatment for lymphoedema for many years,
particularly for mild cases when the oedema is still soft.

Pumps which have multi-compartmental sleeves are more successful as
the compartments are activated in sequence so have a better pumping
affect. Uni-compartment sleeves are less effective. The use of a
compression pump is not recommended unless it is preceded by
measures to stimulate the central trunk lymphatic drainage as is done
when performing manual lymphatic massage.

Laser Therapy
The word LASER is an acronym for �light amplification by stimulated
emission of radiation�

Low level laser therapy (LLLT) is used in the treatment of lymphoedema
producing low intensity irradiation, causing biological effects in the
tissues, not heating. It is claimed that LLLT stimulates the regeneration
of lymphatics, increases the contractibility of lymphatics and stimulates
changes in the fibre content of oedematous tissues, thereby softening
hard fibrotic oedema. Laser also usually provides dramatic relief in the
treatment of cording in the axilla and inner aspect of the arm
particularly when combined with graduated stretching exercises. These
cord-like bands, which sometimes develop post-surgically in the
treatment of breast cancer can cause pain and discomfort when they are
stretched inadvertently on certain shoulder movements.

The most common lasers used in the treatment of lymphoedema are:

Gallium-Arsenide infrared laser, wavelength of 904 nanometres,
penetration of 5cm and power of 14mw. The beam is pulsed and is
invisible.
6.5mw Helium Neon laser, wavelength of 632.8 nanometres, penetration
of 0.8 mm and power of 10mw. The beam is red and visible.

Drug Therapy
The use of two groups of drugs has been advocated in the treatment of
lymphoedema.

I. Diuretics
Diuretics are recognised as being of use in the treatment of generalised
low-protein oedemas which have an elevated sodium content in the
body, such as congestive heart failure, renal disease and hypertension.
Their administration in high protein oedemas such as lymphoedema is
sometimes beneficial for a short time in those with mild, soft oedema
but is contra-indicated in the long term due to the potential to induce
fluid and electrolyte imbalance in the body, increased protein
concentration and resultant increase in fibrosis.

It has been shown that any improvement gained when used in the
treatment of lymphoedema is achieved by reduction of the water content
of the oedema which is excreted in the urine rather than removal of the
protein molecules.

II. II. Benzo-pyrones
It has been shown that the benzo-pyrone group of drugs break down the
large protein molecules present in the oedema so assist in its reduction.
However, registration of the benzo-pyrone drug Coumarin (Lodema) was
cancelled in Australia in 1996 by the Department of Health and Family
Services due to the increasing number of reported cases of hepatoxicity
received by the Australian Drug Evaluation Committee.

Phase II of CPT

This starts at the completion of Phase I when the oedema has been
reduced and the affected limb has been fitted with a compression
garment. It is normal practice for two garments to be supplied for each
oedematous limb, allowing time for them to be washed regularly.

Phase II is the continuous period of self-management by the patient in
the ensuing years along with supervision, assistance and possible
intermittent treatment from the therapist.

Patients are usually reviewed by their therapist every three months to
check on the progress of the condition, the circumferential
measurements of the oedematous limb and supply of new garments as
and when required. The effective life of compression garments is usually
4-6 months, after which time they have lost their elasticity and are
consequently no longer providing adequate support to the limb,
sometimes allowing some oedema to return.

During Phase II, lymphoedema patients are usually required to wear
some form of compression on the affected limb daily, and sometimes at
night, in order to keep the oedema under control. In theory, the
oedematous limb does not need the same compression at night as it
does during the day, due to the reduced effect of gravity while lying
down. Patients can, therefore, usually wear a garment of a lesser
compression at night to keep the oedema under control or no garment if
the oedema is only mild.