Vascular Medicine

http://vmj.sagepub.com/ Investigation and management of lymphoedema

PS Mortimer Vasc Med 1990 1: 1 DOI: 10.1177/1358836X9000100102 The online version of this article can be found at: http://vmj.sagepub.com/content/1/1/1

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Investigation
PS Mortimer Consultant

and management of
St

lymphoedema
Hospital, London

Dermatologist,

Georges Hospital

and

Royal

Marsden

Investigation and management of lymphoedema

which,

Lymphoedema is a rather neglected condition once diagnosed, is frequently dismissed because there is nothing that can be done for
it. It is considered rare, and as a nonfatal and harmless condition does not command the attention or research interest that problems relating to the blood circulation do, for example. Lymphoedema manifests as tissue swelling (oedema) usually of a limb. Limb oedema is, however, a common medical problem. Lymphatic involvement in many forms of chronic oedema is underestimated, an attitude which stems mainly from a lack of investigative techniques, and also from a lack of interest in the lymphatic system.

What is

lymphoedema?

Lymphoedema is defined as tissue swelling due to a failure of lymph drainage. The chief function of the lymphatics is the clearance of protein and
other macromolecules from the tissues, with the water content of lymph acting essentially as a vehicle for the particles. Therefore, the main abnormality in lymphoedema is the accumulation of protein, and macromolecules too large to reAddress for

Dermatologist,

correspondence: PS Mortimer, Consultant St Georges Hospital, Blackshaw Rd, London

SW17 0QT, UK.

the blood vessels directly, in the extracellular space. The retention of fluid is largely through osmotic forces from the trapped protein. This underplays the role of the lymphatic as a safety valve in the prevention of oedema.1 It follows that any form of oedema concerns the lymphatic. In his textbook, well-known in its day, Aird used the expression lymphoedema simply as a synonym for chronic oedema, and specifically stated that it was not necessarily of lymphatic origin.2 Although strictly speaking incorrect, his point that the lymphatic is involved in all forms of oedema is true. Chronic swelling due to oedema, particularly of the lower extremity, is a common disorder which is often the cause of much concern to the lay person as an omen of serious disease. Consideration, first, of central causes of oedema such as heart failure and hypoproteinaemia is most important, but in the majority of cases swelling will be secondary to circulatory problems, e.g. venous or lymphatic disease. Oedema frequently develops from either an excess of capillary filtrate with normal but overloaded regional lymphatics (venous hypertension, dependency syndrome), or from defective lymphatics with an unaltered lymph load (lymphoedema). Chronic oedema rarely arises solely from the failure of one system. For example, in a lymphoedematous limb blood flow is increased by some 30% for reasons as yet 3 unexplained.3 Conversely, in chronic venous disease of the lower limb, particularly with
enter
-

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lymphatic function can become compromised and a compound oedema develop.

ulceration,

Pathogenesis

..

Lymphoedema occurs when lymphatic are either congenitally absent or

channels become

obstructed or obliterated. Plasma which normally escapes from the blood stream then accumulates in soft tissues as protein-rich oedema fluid. Lymphoedema is characterized by a decreased rate of lymph absorption or low lymph flow 4 failure.4 Obstructed lymphatics occur most commonly at lymph node level from cancer or its treatment, i.e. surgery or radiotherapy, when lymphatics fail to regenerate through fibrotic irradiated tissue or surgical scars. Inflammatory processes may easily cause intralumenal obliteration of lymphatic vessels due to

common but poorly documented form of peripheral oedema results from a combination of immobility and dependency. Immobility leads to chronic lymph stasis6 which is compounded by enhanced lymph formation from venous hypertension in a dependent lower limb. This clinical syndrome is most vividly seen in infirm elderly patients confined to a chair, day and night, by heart and respiratory failure. Sneddon and Church coined the phrase armchair legs, and are from appearances indistinguishable lymphoedema (Figure 1). An alternative term is 8 lymphostasis verruciformis/verrucosis.g Chronic

oedema arises under similar circumstances in paralysed limbs and with severe arthritis, where

immobility prevails.

lymphanigitis
Infections

or

lymphangiothrombosis.

such as filiriasis or bacterial and cellulitis are the major culprits, lymphangitis but inorganic materials such as silica can also
cause

damage.

When lymph formation is increased to the point where it overwhelms the capacity of the lymphatic absorptive apparatus, oedema also results. This form of lymphoedema, where there is a relative
or

dynamic lymphatic insufficiency (high lymph

flow failure), occurs most commonly in association with chronic venous disease. Increased lymph formation results from increased capillary filtration. Leg ulceration arising from chronic ambulatory venous hypertension exposes lymphatics to damage from inflammation and infection. In this way true lymphoedema and chronic venous disease can coexist. abnormalities in dermal Morphological lymphatics have been demonstrated in the gaiter skin of patients with incipient venous ulceration
5 (atrophie blanche).5 Lymphatics rely almost entirely on local tissue movement for lymph propulsion. Lymph and precollectors possess no smooth capillaries muscle in the vessel wall. Lymph movement into and along these smallest peripheral vessels is largely a passive process dependent on changes in local hydrostatic and osmotic pressures; it is only the larger contractile lymphatic collectors and trunks which actively pump lymph. A

Figure 1 Armchair legs. Features classical lymphoedema

indistinguishable from

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Diagnosis
Accumulation of high protein oedema in the skin results in characteristic changes, referred to as elephantiasis: enhanced skin creases, increased dermal turgor, hyperkeratosis and papillomatosis are most obvious in circumstances where dermal lymphatics are overloaded and severely obstructed. This occurs most commonly in lower limb lymphoedema and malignant infiltration of skin lymphatics, and the clinical diagnosis of lymphoedema depends almost entirely on these skin and subcutaneous tissue changes. Stemmer9 described the useful sign of thickened skin folds of the toes which prevents pinching of skin, particularly at the base of the second toe (Figure

this may be generally true, pitting is a most unreliable sign as many cases of lymphoedema will exhibit easy displacement of tissue fluid on pressure. Most forms of oedema respond to elevation and diuretics, but lymphoedema does not, except in the very early stages or when compounded by other forms of oedema. Indeed, chronic swelling that does not reduce significantly after overnight elevation is likely to be lymphatic
in

origin.
There

is one specific and characteristic complication of lymphoedema, and that is recurrent erysipelas or cellulitis. The patient feels constitutionally unwell, as if flu is starting, and within 8-24 hours redness and tenderness appear in the lymphoedematous area. Swelling invariably deteriorates, and may remain so even after the resolution of the attack. Because of the failure to isolate an organism in the majority of cases,

3). Traditionally lymphoedema is described as brawny oedema which does not readily pit. Whilst

2a
Figure 2 Cutaneous signs of lymphoedema: a) increased skin markings, b) hyperkeratosis and papillomatosis (elephantiasis)

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2b

Figure 3 Stemmers the second toe

sign.

positive sign is the inability to pinch a fold of skin (due to

increased skin

thickness)

at the base of

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5 the bacterial aetiology of all such cases has been brought into question. The term acute inflammatory episode is now considered preferable to erysipelas or cellulitis.10

Investigation

diagnosis of lymphoedema straightforward except in the

The

is

not

more

always typical

established cases. In-vivo visualization of lymphatic vessels (lymphangiography) and nodes (lymphography) using X-ray contrast medium&dquo; remains the gold standard for demonstrating lymphatic vessel abnormalities. However, the technique is invasive and difficult to perform in the presence of oedema. Only subcutaneous lymphatics as large, or larger than collectors can be opacified except in pathological circumstances when dermal backflow occurs and smaller skin lymphatics become visible. The need for more functional information rather than simply anatomical detail has seen the emergence of

quantitative lymphoscintigraphy lymphography).

Quantitative lymphoscintigraphy

(isotope

It is the essential function of the lymphatic to return to the vascular compartment extravascular molecules and colloids too large to re-enter directly. 12 The rate at which a labelled protein or colloid is removed from the interstitial tissues has therefore been regarded as an index of lymphatic function,. 13,14 Interpretation of tracer clearance by external scintillation detection in disease states in difficult and unreliable. The dynamics of lymph flow as depicted by radiocolloid uptake, and transit via lymphatic vessels can be studied using a gamma camera with a large field of view. The tracer is administered by interstitial injection, which obviates the need for direct cannulation of peripheral lymphatics. Transit times and timeactivity curves calculated from regions of interest

system

permit quantitative analysis. lymphoscintigraphy (isotope lymphography) has proved useful in the differential diagnosis of chronic limb swelling by detecting lymphatic insufficiency.S-&dquo; The main lymph drainage routes can be identified (Figure 4). Access of tracer from the injection depot to
over

nodes

Quantitative

Figure 4 Isotope lymphography:

a) normal isotope lymphogram except for collateral drainage in the left thigh. Quantification of nodal uptake gives index
of limb

lymphatic function;

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b) patient with Milroys disease, absent drainage in right leg and uptake in normal left leg;

c) patient with swollen legs previously diagnosed as

lymphoedema who was found to have normal lymph drainage and widespread venous ectasia due to EhlersDanlos Syndrome Type IV

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peripheral lymphatics is grossly impaired in the infection appears to obliterate parts of the lymphoedema with hypoplastic distal lymph network, and in advanced chronic venous disease. vessels, giving a characteristic picture at the The technique may therefore be used to confirm injection site and virtually no clearance of tracer. the clinical diagnosis of lymphoedema, and for Alternatively, proximal lymphatic obstruction the study of skin lymphatics in various disease results in retrograde lymph flow to cutaneous states. lymphatics (dermal back flow). Thus various subgroups of lymphoedema can be identified Indirect lymphography Indirect lymphography employs water-soluble without recourse to conventional lymphography, and in this way it is possible to identify subtle nonionic X-ray contrast media that can be or incipient lymphoedema and lymphatic administered via an interstitial injection, without insufficiency in cases of chronic oedema of recourse to direct access into lymphatics.19 compound origin. It is an accurate method which Iotralan or Iotasol (Schering AG, Berlin) is correlates well with the clinical severity of infused by a motor pump into the skin; 2-3mls lymphoedema, and should be considered before injected intradermally leads to considerable local X-ray lymphography in the investigation of skin distension and is not without discomfort. Dermal and subcutaneous collecting lymphatics lymphoedema. Intravital dyes, e.g. Patent Blue, used to can be visualized by X-ray using the delineate subcutaneous lymphatics prior to direct mammography film method (Figure 6). In the cannulation for X-ray lymphography, can be of presence of incompetent valves and dermal value for detecting lymphatic abnormalities; the backflow, (initial capillaries lymphatic results, however, are transitory. Increasing lymphatics) can also be seen. Characteristic interest in the microcirculation has resulted in patterns of lymphatic abnormality are recognized two new methods capable of demonstrating depending on the underlying form of oedema. 20 fluorescence microlymphangiography This technique is not suitable for lymph node lymphatics: and indirect X-ray lymphography. examination, although main lymphatic collectors and nodes are, on occasions, opacified. These techniques have revealed not only Fluorescence microlymphangiography greater morphological and functional detail of Fluorescence microlymphangiography 18 known cases of lymphoedema, but that oedema enables the superficial lymph capillary network previously attributed to other causes, e.g. venous of the skin to be seen under the vital microscope disease, may possess a significant lymphatic by means of fluorescing macromolecules (FITC- component. It is now clear that investigation of DEXTRAN, Sigma) injected subepidermally and a chronically swollen limb should, following a cleared exclusively by lymphatics (Figure 5). detailed history and examination, include some Information regarding the morphology of form of physiological venous test such as photolymphatic capillaries (initial lymphatics) and the plethysmography, strain gauge plethysmography extent of tracer propagation within the dermal or foot volumetry, as well as quantitative lymlymphatic network can be recorded on video for phoscintigraphy (isotope lymphography). analysis. It is possible to distinguish between Phlebography provides anatomical information, Milroys disease and other forms of primary not functional, and is generally unhelpful unless lymphoedema owing to the total aplasia of initial deep vein thrombosis is sought. Colour duplex lymphatics in the former. Obstructed proximal ultrasound scanning demonstrates blood flow lymphatics with intact skin capillaries result in dynamics in major arteries and veins, and the the visualization of an extensive network owing direction and velocity of blood flow within major to cutaneous reflux and horizontal flow through vessels, as well as any collateral circulation, can incompetent skin lymphatics. Lymphatics may be be readily identified. Using this noninvasive seen some distance away from the injection technique it has been possible, for example, to deposit and without obvious communication due demonstrate postural-dependent venous outflow to dermal backflow. Damaged lymphatic obstruction in cancer patients with treatmentcapillaries (microlymphangiopathy) can be induced limb swelling, which was previously observed following recurrent erysipelas, where assumed to be solely lymphoedema.

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Figure 5

Fluorescence lymphangiography. Professor A Bollinger)

Demonstrating the superficial network of cutaneous lymphatics (courtesy of

Management
is the end-stage failure of lymph is to all intents and purposes irreversible and incurable. It manifests as chronic swelling of one part of the body, and in the case of a limb this is frequently associated with swelling of the adjoining quadrant of the trunk owing to defined areas of lymph drainage. Disabilities include pain, limb heaviness, reduced mobility and impaired function, and the size and weight of some limbs result in secondary complications such as progressive musculoskeletal and joint problems. There is a small but significant risk of cutaneous malignancy developing, of which the most infamous is lymphangiosarcoma,21 but other tumours have been reported including squamous cell carcinoma,22 lymphoma,23 melanoma 24 and malignant fibrous

Lymphoedema drainage, and

histiocytoma. 25 (Kaposis sarcoma is frequently associated with lymphoedema, but the tumour usually antedates the onset of the swelling). The favoured theory for the association of chronic lymphoedema and subsequent malignancy is
altered immune surveillance in the affected region.26 This may also be the explanation for the increased incidence of bacterial and fungal infections in lymphoedematous limbs. Attacks of cellulitis or erysipelas produce debilitating constitutional upset and fever, which frequently require admission to hospital and administration of parenteral antibiotics. Such attacks are characteristically associated with lymphoedema and seem more profound than usual. The reason for this is not clear, but it may be that under normal circumstances the lymphatic system contains and disposes of any infection regionally between the portal of entry and lymph node. Obstructed

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Figure 6 Indirect lymphography. Demonstrating network of cutaneous lymphatics in a foot with lymphoedema praecox (above) and normal collectors on the healthy contralateral side (below) (courtesy of Professor H Partsch)

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10

lymphatics
severe

may dissemination of inflammation

are

possibly allow more rapid micro-organisms, leading to systemic upset often before any signs of
evident. Proof of infection is

rarely forthcoming, leading to suggestions that the terms pseudoerysipelas2 and acute inflammatory episodesl should be preferred. The approach to the treatment of lymphoedema has been relatively nihilistic. Apparently insurmountable difficulties, the lack of an effective drug therapy and a paucity of
absence of proven It must be remembered that the essential function of the lymphatic is the removal of protein and other macromolecules from the tissues. 12 Water is normally present only in sufficient quantity to act as a vehicle for the removal of these 3 materials.3 Lymph accumulation is therefore primarily the retention of protein, macromolecules and other particulate matter, with extra water being withheld due to osmotic forces. This is the fundamental reason why lymphoedema is so difficult to treat: fluid alone can be readily displaced or absorbed back into the vascular compartment, but protein can only escape via the lymphatics or be slowly broken down at site by phagocytosis. This explains why, unlike all other forms of oedema, lymphoedema does not reduce substantially with overnight elevation. Lymphoedema will invariably progress, particularly in the lower limb, unless controlled. Influences which are known to hasten this progression must be carefully avoided. Limb positioning is important at rest, as any dependent limb will tend to swell as a result of increased intravascular hydrostatic pressure. Elevation just above heart level is adequate; extreme elevation is unnecessary and probably unwise unless venous hypertension coexists. There is no evidence that the common practice of hanging a postmastectomy arm overnight from a drip stand does anything to improve drainage of highprotein oedema. On the contrary, recent experience studying blood flow dynamics with colour duplex ultrasound scanning has clearly demonstrated obstruction to venous outflow with high elevation, owing to postsurgical or radiation axillary fibrosis kinking and compressing the axillary vein.
an

research have resulted in

on

guidelines

management.

Prevention of inflammation General measures regarding limb care are important, and the prevention of infection is critical: one attack of cellulitis/lymphangitis may not only make the patient very ill, but lead to further deterioration in limb swelling which may not recover. Care of the skin, good hygiene, control of fungal infection between digits and good antisepsis following abrasions and minor wounds are as important as in diabetes. Recurrent inflammatory episodes (cellulitis) can be very debilitating and must be prevented. Prophylactic phenoxymethyl penicillin 500mg daily is considered more effective than the administration of high-dose penicillins at the time of an attack, although there are no clinical trials to support this. Circumstances provoking inflammation such as sunburn, insect bites and trauma from gardening, cooking etc. should be avoided as far as possible, as should prolonged isometric muscular contractions, e.g. carrying shopping. Diuretics remain the most commonly used treatment for lymphoedema because, to most doctors, oedema is an indication for such drugs. Diuretics alone have very little benefit in lymphoedema simply because their main action

limit capillary filtration by reducing blood volume. Indeed, improvement from diuretics suggests the predominant cause of the oedema is not lymphatic. Nevertheless, their use in oedema of mixed origin or in combination with other therapy may be helpful. The benzo-pyrones, e.g. oxerutins (Paroven®), a group of drugs related to coumarin, have been advocated for use in lymphoedema, although they are licensed for venous disorders. Recent work has shown objectively their effect in normalizing increasing capillary permeability,28 and a trial has demonstrated benefit in lymphoedema, although this needs substantiating.29
to

is

circulating

Surgery

Lymphoedema
surgical

is

considered

a as

surgical
yet,
no

condition, but unfortunately there is,

solution. Until recently all operations have aimed simply to reduce limb size.3o Advances in microvascular techniques and the introduction of lymph node-venous shunt and lymphovenous anastomosis operations3l-32 have proved of temporary benefit. Debulking of tissue in the grossest limbs may at times still be

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11

performed,

but

improvements

in

medical

treatment make this less necessary.

Physical therapy

Specific physical therapy aims essentially to lymph formation and improve lymph drainage through existing lymphatics and collateral routes by applying normal physiological processes which stimulate lymph flow. Peripheral lymph flow depends upon two factors: first, and flow absorption through lymph noncontractile lymphatic vessels, and secondly, lymph propulsion through contractile lymph
control
ensure unidirectional flow. The first step depends on changes in local tissue pressure produced by external pressure,33 skin surface massage, 34,35 arterial pulsation,36 passive movements,37,38 and muscular activity.39 The second step involving flow in contractile lymph vessels depends on the volume of fluid entering each lymphatic segment or lymphangion. The stimulus to lymphatic contractions is the filling and distention of vessels4; the characteristics of these contractions have some similarity to those of the heart. 41 Muscular activity and exercise produce the greatest lymph propulsion. Obstruction to main lymphatic drainage routes should result in the opening up of collateral routes, as is known to happen in venous obstruction. However, without sufficient vis a tergo collateral drainage may not satisfactorily occur. The principle of physical therapy depends upon performing procedures which would normally enhance lymph flow and therefore maximize remaining lymph transport capacity. It is claimed swelling can often be reduced dramatically with this therapy, but there is little objective published evidence. 42

vessels; valves

External support External support is the cornerstone of medical treatment for several reasons:

good

efficient muscle pump enhancing lymph A garment must be well fitting and comfortable, otherwise patient compliance will be poor; equally, a torniquet effect may be produced particularly if it rolls down or is folded over. Strong hosiery exerting pressures > 30mmHg is usually required, and it is often worth the extra effort and expense of starting with lower compression in order to allow time for the patient to become accustomed to the fitting and wearing of these garments. Lymphoedema invariably requires the highest compression strength (> 40mmHg) hosiery, and double hoses may need to be worn on some occasions to maintain control. Close collaboration is advised between clinician, fitter and surgical appliance officer. Most garments last six months before renewal is necessary, and it is wise to provide two pairs, one to wear and one for the wash. External support may also be provided by bandages, but usually only in a palliative care situation or where hosiery cannot be managed. Bandages are most often used for compression therapy in limb reduction. The technique of compression bandaging is based on the principle of applying a strong, nonelastic bandage as firmly as possible; this allows a high pressure during muscular contraction but low pressure at rest. Elastic bandages such as blue-line continue to compress at rest and this allows too much stretch. Nonelasticated bandages, e.g. Comprilang (Biersdorf) and Secureforte,@ (Johnson & Johnson) possesses sufficient give to mould to the shape of the limb but do not yield, and therefore act as a firm outer collar during muscular contraction. The use of foam underneath distributes pressure evenly, thereby minimizing constricting bands, and the positioning of rubber pads helps iron out pockets of swelling such as collect on the dorsum of the foot or the back of the hand. Multiple layers of
more

drainage.

1)
2) 3)

Limiting blood capillary filtration by raising interstitial pressure. Opposing tissue expansions. Improving striated muscle pump efficiency.

bandage serve (Figure 7).

Massage

to

generate higher compression

Elastic hosiery exerts a controlling external pressure and serves as a containment garment to In rare instances maintain limb size. improvements can be achieved as a result of a

Tissue movement is a stimulus to lymph flow, and by creating the correct incentive and guidance for lymph flow there should be sufficient reserve transport capacity, even in an obstructed system, to allow drainage by collateral routes, as occurs in the venous system. Optimal isotonic exercise

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15 in the presence of external support enhances lymph pumping, as it does in venous return in chronic venous insufficiency. If performed correctly, skin surface massage encourages the movement of lymph through the intercommunicating network of skin and subcutaneous lymphatics to normally drained areas. Truncal and facial lymphoedema can only be influenced by massage, as external support or compression are impractical. A reduction in swelling and congestion at the truncal root of a limb will allow freer drainage from that limb via skin and subcutaneous collaterals to normally draining lymph node areas, thus bypassing the obstruction. This is the principle of manual lymphatic drainage practised in many parts of Europe.46 Pneumatic compression theory Pneumatic compression therapy is widely employed.44 Consisting of an inflatable sleeve or boot connected to a motor driven air pump, these machines reduce limb volume by displacement of fluid and help soften a limb by disrupting tissue fibrosis.45 If external support is not fitted immediately following compression therapy, a limb readily reswells. The first machines possessed a single chamber boot or sleeve (Flowtron, Jobst), and discomfort from sustained pressure was often a disadvantage. The Lymphapress (Huntleigh Technology) consists of a multichamber garment, each chamber filling consecutively to displace fluid proximally. More recent machines, e.g. Talley Multicom (Talley Medical Equipment Ltd) provide sequential filling of a ten-chamber inflatable garment, thus delivering a ripple effect up the limb. The multichamber machines appear more effective than the standard single chamber intermittent pumps.46 Care must be taken with pneumatic compression to ensure that fluid is not forcibly displaced from the limb to the adjoining quadrant of the trunk, where new swelling may develop and

heated to an ambient temperature of 80-90C for hour daily for 10 days. After each treatment an elastic bandage was applied. Up to five (10day) courses of treatment were administered, and results from limb circumference measurements revealed more than 50% resolution of swelling in over 60% of patients. Despite examples of improved lymph drainage demonstrated by lymphangiography and lymphoscintigraphy, the most likely mechanism for the success is denaturing of extracellular protein and improved phagocytosis with enhanced resorption of material directly into the blood stream. Microwave heating has now superseded the infrared oven and appears of equal benefit. 48 Clearly this form of treatment needs more careful evaluation. It is difficult to understand how the patients tolerate the high temperatures used. Success of lymphoedema treatment must depend on the permanent control of swelling and unfortunately, there are few, if any, reliable long-term results.
one

Midline

lymphoedema

persist.
Heat treatment A novel treatment involving heat and bandages has recently been reported from China, in a study of over 1000 patients with chronic lymphoedema of the extremities. 47 Using an electro controlled infrared heating chamber, the affected limb was

Midline lymphoedema such as that occurring on the face or involving the genitalia is fortunately rare. Presumably this is because bilateral lymph drainage routes operate and, although welldefined anatomical lymph drainage areas exist, cross-flow from one region to another can occur including across the midline. Facial lymphoedema develops in circumstances where skin or subcutaneous lymphatics fail. Inflammatory processes such as acne rosacea or erysipelas are usually responsible (Figure 8), and treatment is extremely difficult, even after the cause has been brought under control. Swelling frequently concentrates around the eyes, and closed palpebral fissures on waking can be distressing. Patients may need to rise early, sit upright for an hour or so and practise frequent blinking before normal vision is restored. Sleeping with the head of the bed raised may limit the overnight accumulation of oedema, and gentle massage to lymph node areas may siphon away much of the fluid. Drug therapy is ineffective and external compression of course impractical. Surgery is the treatment of choice. Lymphoedema of the genitalia can be equally difficult, particularly in the male. It arises most commonly from pelvic or bilateral inguinal surgery or radiotherapy. Lax tissues permit gross

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16

Figure 8 Facial lymphoedema secondary to chronic which damages cutaneous lymphatics

rosacea - a

chronic

inflammatory disorder of the pilo

sebaceous units

swelling. Surgery, if feasible, can be helpful, otherwise palliative care with a good scrotal support is the only other option.
Lymphangioma
Dilation of the upper dermal lymphatics to an extent where they visibly bulge on the skin surface is referred to as lymphangioma. Obstruction of

alongside. Cautery or laser therapy followed by sustained external pressure during the healing phase to seal the skin surface can prove successful treatment but relapse is common.
Volume measurements Close monitoring of limb volumes is necessary for the assessment of treatment progress. While subjective improvement is important for the patient, objective measurement of swelling must be performed if new, or even existing treatment regimes are to be evaluated properly. Water displacement has been considered the gold standard for limb volume measurement, but it is cumbersome, and calculation of volume by multiple circumferential measurements has proved more reliable and convenient. From a fixed point such as the heel a distance is measured to what is considered the ankle (or in the upper limb a distance is measured from the tip of the middle finger to the wrist). From the ankle or

deep dermal or subcutaneous collecting lymphatics due to a congenital abnormality is termed lymphangioma circumscriptum .49 Dilatation of surface lymphatics can also occur from
subcutaneous fibrosis
in

lymphoedema secondary radiotherapy. Such changes, although clinically and histologically indistinguishable from lymphangioma circumscriptum, are best referred to as acquired lymphangioma50,51 (Figure 9). Such lesions release lymph and serve as a portal of entry for infection. Piecemeal excisions only result in further lymph blisters appearing
to

association with or surgery

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17

-..

-..-...

_._--~----_._--~-~~-,.__._-,

Figure

Acquired lymphangioma

on

the shaft of the

penis secondary

to

inguinal

and

pelvic

surgery and

radiotherapy

wrist marks are drawn every 4cms up the limb, and at each 4cm point the circumference is measured. By calculating the volume for each 4cm segment according to

the values, the volume of the limb from wrist to shoulder (or ankle to hip) is obtained. The method achieved good reproducibility when performed by more than one individual. Assessment of truncal swelling is more difficult. Measurement of pitting by tonometry has been used,52 but its value is uncertain. Future progress in the investigation and management of lymphoedema will depend entirely on a greater research input. A fuller understanding of the pathophysiology of lymphoedema is necessary before more effective and
as a

totalling cylinder

implemented. Most cases of lymphoedema secondary to cancer therapy should be preventable. This relies on identifying those individuals at risk by measuring deteriorating lymph drainage function before swelling occurs. This should become possible as methods of investigation improve. The expectation with current medical therapy is at least to prevent progression of swelling and at best to reduce limb size to that approaching normal. Successful treatment depends on high motivation and perseverence on the part of the patient, and then long-term reductions in limb volume can be maintained (Figure 9).
Acknowledgements
The author wishes to thank Caroline Badger (Clinical Nurse Specialist in lymphoedema at the Royal Marsden Hospital) for her help in the preparation of this article.

treatment can be

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18

10a

10b Figure 10 Successful treatment by physical therapy of lower limb lymphoedema with maintenance of hosiery one year later a) before treatment; b) one year later

limb size

by elastic

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19

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