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1990 1: 1Vasc MedPS Mortimer

Investigation and management of lymphoedema

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Investigation and management of lymphoedemaPS Mortimer Consultant Dermatologist, St Georges Hospital and Royal Marsden Hospital, London

Address for correspondence: PS Mortimer, ConsultantDermatologist, St Georges Hospital, Blackshaw Rd, LondonSW17 0QT, UK.

Investigation and management oflymphoedema

Lymphoedema is a rather neglected conditionwhich, once diagnosed, is frequently dismissedbecause there is nothing that can be done forit. It is considered rare, and as a nonfatal and

harmless condition does not command the

attention or research interest that problemsrelating 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. Lymphaticinvolvement in many forms of chronic oedema

is underestimated, an attitude which stems mainlyfrom a lack of investigative techniques, and alsofrom a lack of interest in the lymphatic system.

What is lymphoedema?

Lymphoedema is defined as tissue swelling dueto a failure of lymph drainage. The chief function

of the lymphatics is the clearance of protein andother macromolecules from the tissues, with thewater content of lymph acting essentially as avehicle for the particles. Therefore, the main

abnormality in lymphoedema is the accumulationof protein, and macromolecules too large to re-

enter the blood vessels directly, in theextracellular space. The retention of fluid is

largely through osmotic forces from the trappedprotein. This underplays the role of the lymphaticas a safety valve in the prevention of oedema.1It follows that any form of oedema concerns the

lymphatic. In his textbook, well-known in its day,Aird used the expression lymphoedema simplyas a synonym for chronic oedema, and specificallystated that it was not necessarily of lymphaticorigin.2Although strictly speaking incorrect, his

point that the lymphatic is involved in all formsof oedema is true.Chronic swelling due to oedema, particularly

of the lower extremity, is a common disorderwhich 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 circulatoryproblems, e.g. venous or lymphatic disease.Oedema frequently develops from either anexcess of capillary filtrate with normal butoverloaded regional lymphatics (venoushypertension, dependency syndrome), or fromdefective lymphatics with an unaltered lymphload (lymphoedema). Chronic oedema rarelyarises solely from the failure of one system. Forexample, in a lymphoedematous limb blood flowis increased by some 30% for reasons as yetunexplained.3

3 Conversely, in chronic venousdisease of the lower limb, particularly with

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ulceration, lymphatic function can becomecompromised and a compound oedema develop.

Pathogenesis ..

Lymphoedema occurs when lymphatic channelsare either congenitally absent or becomeobstructed or obliterated. Plasma which normallyescapes from the blood stream then accumulatesin soft tissues as protein-rich oedema fluid.Lymphoedema is characterized by a decreasedrate of lymph absorption or low lymph flowfailure.44 Obstructed lymphatics occur mostcommonly at lymph node level from cancer orits treatment, i.e. surgery or radiotherapy, when

lymphatics fail to regenerate through fibrotic

irradiated tissue or surgical scars. Inflammatoryprocesses may easily cause intralumenalobliteration of lymphatic vessels due to

lymphanigitis or lymphangiothrombosis.Infections such as filiriasis or bacterial

lymphangitis and cellulitis are the major culprits,but inorganic materials such as silica can alsocause damage.When lymph formation is increased to the point

where it overwhelms the capacity of the lymphaticabsorptive apparatus, oedema also results. Thisform of lymphoedema, where there is a relativeor dynamic lymphatic insufficiency (high lymphflow failure), occurs most commonly inassociation with chronic venous disease.Increased lymph formation results from increasedcapillary filtration. Leg ulceration arising fromchronic ambulatory venous hypertension exposeslymphatics to damage from inflammation andinfection. In this way true lymphoedema andchronic venous disease can coexist.

Morphological abnormalities in dermallymphatics have been demonstrated in the gaiterskin of patients with incipient venous ulceration(atrophie blanche).55

Lymphatics rely almost entirely on local tissuemovement for lymph propulsion. Lymphcapillaries and precollectors possess no smoothmuscle in the vessel wall. Lymph movement intoand along these smallest peripheral vessels islargely a passive process dependent on changesin local hydrostatic and osmotic pressures; it isonly the larger contractile lymphatic collectorsand trunks which actively pump lymph.A

common but poorly documented form of

peripheral oedema results from a combination of

immobility and dependency. Immobility leads tochronic lymph stasis6 which is compounded byenhanced lymph formation from venoushypertension in a

dependentlower limb. This

clinical syndrome is most vividly seen in infirmelderly patients confined to a chair, day and night,by heart and respiratory failure. Sneddon andChurch coined the phrase armchair legs, andappearances are indistinguishable fromlymphoedema (Figure 1).An alternative term islymphostasis verruciformis/verrucosis.g8 Chronicoedema arises under similar circumstances in

paralysed limbs and with severe arthritis, where

immobility prevails.

Figure 1 Armchair legs. Features indistinguishable fromclassical lymphoedema



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Diagnosis

Accumulation of high protein oedema in the skinresults in characteristic changes, referred to as

elephantiasis: enhanced skin creases, increaseddermal turgor, hyperkeratosis and papillomatosisare most obvious in circumstances where dermal

lymphatics are overloaded and severelyobstructed. This occurs most commonly in lowerlimb lymphoedema and malignant infiltration ofskin lymphatics, and the clinical diagnosis of

lymphoedema depends almost entirely on theseskin and subcutaneous tissue changes. Stemmer9described the useful sign of thickened skin foldsof the toes which prevents pinching of skin,particularly at the base of the second toe (Figure3).

Traditionally lymphoedemais described as

brawny oedema which does not readily pit. Whilst

this may be generally true, pitting is a mostunreliable sign as many cases of lymphoedemawill exhibit easy displacement of tissue fluid on

pressure. Most forms of oedema respond toelevation and diuretics, but lymphoedema doesnot, except in the very early stages or whencompounded by other forms of oedema. Indeed,chronic swelling that does not reduce significantlyafter overnight elevation is likely to be lymphaticin origin.There is one specific and characteristic

complication of lymphoedema, and that isrecurrent erysipelas or cellulitis. The patient feelsconstitutionally unwell, as if flu is starting, andwithin 8-24 hours redness and tenderness appearin the lymphoedematous area. Swelling invariablydeteriorates, and may remain so even after theresolution of the attack. Because of the failureto isolate an organism in the majority of cases,

2a

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



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2b

Figure 3 Stemmers sign.A positive sign is the inability to pinch a fold of skin (due to increased skin thickness) at the base ofthe second toe



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the bacterial aetiology of all such cases has been

brought into question. The term acuteinflammatory episode is now considered

preferable to erysipelas or cellulitis.10

Investigation

The diagnosis of lymphoedema is not alwaysstraightforward except in the more typicalestablished cases. In-vivo visualization of

lymphatic vessels (lymphangiography) and nodes(lymphography) using X-ray contrast medium&dquo;remains the gold standard for demonstratinglymphatic vessel abnormalities. However, the

technique is invasive and difficult to perform inthe presence of oedema. Only subcutaneous

lymphatics as large, or larger than collectors canbe opacified except in pathological circumstanceswhen dermal backflow occurs and smaller skin

lymphatics become visible. The need for morefunctional information rather than simplyanatomical detail has seen the emergence of

quantitative lymphoscintigraphy (isotopelymphography).

Quantitative lymphoscintigraphyIt is the essential function of the lymphatic

system to return to the vascular compartmentextravascular molecules and colloids too large tore-enter directly. 12 The rate at which a labelled

protein or colloid is removed from the interstitialtissues has therefore been regarded as an indexof lymphatic function,. 13,14 Interpretation of tracerclearance by external scintillation detection indisease states in difficult and unreliable. The

dynamics of lymph flow as depicted byradiocolloid uptake, and transit via lymphaticvessels can be studied using a gamma camerawith a large field of view. The tracer isadministered

byinterstitial

injection,which

obviates the need for direct cannulation of

peripheral lymphatics. Transit times and time-

activity curves calculated from regions of interestover nodes permit quantitative analysis.

Quantitative lymphoscintigraphy (isotopelymphography) has proved useful in thedifferential diagnosis of chronic limb swelling bydetecting lymphatic insufficiency.S-&dquo; The mainlymph drainage routes can be identified (Figure4).Access of tracer from the injection depot to

Figure 4 Isotope lymphography:a) normal isotope lymphogram except for collateral drainage

in the left thigh. Quantification of nodal uptake gives indexof limb lymphatic function;



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

c) patient with swollen legs previously diagnosed aslymphoedema who was found to have normal lymphdrainage and widespread venous ectasia due to Ehlers-Danlos Syndrome Type IV .



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peripheral lymphatics is grossly impaired in

lymphoedema with hypoplastic distal lymphvessels, giving a characteristic picture at the

injection site and virtually no clearance of tracer.

Alternatively, proximal lymphatic obstructionresults in retrograde lymph flow to cutaneous

lymphatics (dermal back flow). Thus varioussubgroups of lymphoedema can be identifiedwithout recourse to conventional lymphography,and in this way it is possible to identify subtleor incipient lymphoedema and lymphaticinsufficiency in cases of chronic oedema of

compound origin. It is an accurate method whichcorrelates well with the clinical severity of

lymphoedema, and should be considered before

X-ray lymphography in the investigation of

lymphoedema.

Intravital dyes, e.g.Patent

Blue,used to

delineate subcutaneous lymphatics prior to directcannulation for X-ray lymphography, can be ofvalue for detecting lymphatic abnormalities; the

results, however, are transitory. Increasinginterest in the microcirculation has resulted in

two new methods capable of demonstratinglymphatics: fluorescence microlymphangiographyand indirect X-ray lymphography.

Fluorescence microlymphangiography

Fluorescence microlymphangiography 18enables the superficial lymph capillary networkof the skin to be seen under the vital microscopeby means of fluorescing macromolecules (FITC-DEXTRAN, Sigma) injected subepidermally andcleared exclusively by lymphatics (Figure 5).Information regarding the morphology of

lymphatic capillaries (initial lymphatics) and theextent of tracer propagation within the dermal

lymphatic network can be recorded on video for

analysis. It is possible to distinguish between

Milroys disease and other forms of primary

lymphoedema owing to the total aplasia of initiallymphatics in the former. Obstructed proximallymphatics with intact skin capillaries result inthe visualization of an extensive network owingto cutaneous reflux and horizontal flow throughincompetent skin lymphatics. Lymphatics may beseen some distance away from the injectiondeposit and without obvious communication dueto dermal backflow. Damaged lymphaticcapillaries (microlymphangiopathy) can beobserved following recurrent erysipelas, where

the infection appears to obliterate parts of the

network, and in advanced chronic venous disease.The technique may therefore be used to confirmthe clinical diagnosis of lymphoedema, and forthe study of skin lymphatics in various diseasestates.

Indirect lymphographyIndirect lymphography employs water-soluble

nonionic X-ray contrast media that can beadministered via an interstitial injection, withoutrecourse to direct access into lymphatics.19Iotralan or Iotasol (ScheringAG, Berlin) isinfused by a motor pump into the skin; 2-3mlsinjected intradermally leads to considerable localskin distension and is not without discomfort.

Dermal and subcutaneous collecting lymphaticscan be visualized

by X-ray usingthe

mammography film method (Figure 6). In thepresence of incompetent valves and dermalbackflow, lymphatic capillaries (initiallymphatics) can also be seen. Characteristicpatterns of lymphatic abnormality are recognizeddepending on the underlying form of oedema. 20This technique is not suitable for lymph nodeexamination, although main lymphatic collectorsand nodes are, on occasions, opacified.These techniques have revealed not only

greater morphological and functional detail of

known cases of lymphoedema, but that oedemapreviously attributed to other causes, e.g. venousdisease, may possess a significant lymphaticcomponent. It is now clear that investigation ofa chronically swollen limb should, following adetailed history and examination, include someform of physiological venous test such as photo-plethysmography, strain gauge plethysmographyor foot volumetry, as well as quantitative lym-phoscintigraphy (isotope lymphography).Phlebography provides anatomical information,not functional, and is generally unhelpful unless

deep vein thrombosis is sought. Colour duplexultrasound scanning demonstrates blood flow

dynamics in major arteries and veins, and thedirection and velocity of blood flow within majorvessels, as well as any collateral circulation, canbe readily identified. Using this noninvasivetechnique it has been possible, for example, todemonstrate postural-dependent venous outflowobstruction in cancer patients with treatment-induced limb swelling, which was previouslyassumed to be solely lymphoedema.



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Figure 5 Fluorescence lymphangiography. Demonstrating the superficial network of cutaneous lymphatics (courtesy of

ProfessorA Bollinger)

Management

Lymphoedema is the end-stage failure of lymphdrainage, and is to all intents and purposesirreversible and incurable. It manifests as chronic

swelling of one part of the body, and in the caseof a limb this is frequently associated with

swelling of the adjoining quadrant of the trunk

owingto defined areas of

lymph drainage.Disabilities include pain, limb heaviness, reducedmobility and impaired function, and the size and

weight of some limbs result in secondarycomplications such as progressive musculoskeletaland joint problems. There is a small but

significant risk of cutaneous malignancydeveloping, of which the most infamous is lym-phangiosarcoma,21 but other tumours have been

reported including squamous cell carcinoma,22lymphoma,23 melanoma 24 and malignant fibrous

histiocytoma. 25 (Kaposis sarcoma is frequentlyassociated with lymphoedema, but the tumourusually antedates the onset of the swelling). Thefavoured theory for the association of chronic

lymphoedema and subsequent malignancy isaltered immune surveillance in the affected

region.26 This may also be the explanation forthe increased incidence of bacterial and fungalinfections in

lymphoedematouslimbs.Attacks of

cellulitis or erysipelas produce debilitatingconstitutional upset and fever, which frequentlyrequire admission to hospital and administrationof parenteral antibiotics. Such attacks are charac-

teristically associated with lymphoedema andseem more profound than usual. The reason forthis is not clear, but it may be that under normalcircumstances the lymphatic system contains anddisposes of any infection regionally between theportal of entry and lymph node. Obstructed



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



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lymphatics may possibly allow more rapiddissemination of micro-organisms, leading tosevere systemic upset often before any signs ofinflammation are evident. Proof of infection is

rarely forthcoming, leading to suggestions thatthe terms

pseudoerysipelas2and acute

inflammatory episodesl should be preferred.The approach to the treatment of

lymphoedema has been relatively nihilistic.Apparently insurmountable difficulties, the lackof an effective drug therapy and a paucity ofresearch have resulted in an absence of provenguidelines on management. It must beremembered that the essential function of the

lymphatic is the removal of protein and othermacromolecules from the tissues. 12 Water is

normally present only in sufficient quantity to

act as a vehicle for the removal of thesematerials.33 Lymph accumulation is thereforeprimarily the retention of protein, macro-molecules and other particulate matter, with extrawater being withheld due to osmotic forces. Thisis the fundamental reason why lymphoedema isso difficult to treat: fluid alone can be readilydisplaced or absorbed back into the vascular

compartment, but protein can only escape viathe lymphatics or be slowly broken down at site

by phagocytosis. This explains why, unlike allother 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. Limbpositioning is important at rest, as any dependentlimb will tend to swell as a result of increased

intravascular hydrostatic pressure. Elevation justabove heart level is adequate; extreme elevationis unnecessary and probably unwise unless venoushypertension coexists. There is no evidence thatthe common practice of hanging apostmastectomy arm overnight from a drip standdoes anything to improve drainage of high-protein oedema. On the contrary, recent

experience studying blood flow dynamics withcolour duplex ultrasound scanning has clearlydemonstrated obstruction to venous outflow with

high elevation, owing to postsurgical or radiationaxillary fibrosis kinking and compressing theaxillary vein.

Prevention of inflammation

General measures regarding limb care are

important, and the prevention of infection iscritical: one attack of cellulitis/lymphangitis maynot only make the patient very ill, but lead tofurther deterioration in limb swelling which maynot recover. Care of the skin, good hygiene,control of fungal infection between digits and

good antisepsis following abrasions and minorwounds are as important as in diabetes. Recurrent

inflammatory episodes (cellulitis) can be verydebilitating and must be prevented. Prophylacticphenoxymethyl penicillin 500mg daily isconsidered more effective than the administration

of high-dose penicillins at the time of an attack,although there are no clinical trials to supportthis. Circumstances provoking inflammation such

as sunburn, insect bites and trauma fromgardening, cooking etc. should be avoided as faras possible, as should prolonged isometricmuscular contractions, e.g. carrying shopping.

Diuretics remain the most commonly usedtreatment for lymphoedema because, to mostdoctors, oedema is an indication for such drugs.Diuretics alone have very little benefit in

lymphoedema simply because their main actionis to limit capillary filtration by reducingcirculating blood volume. Indeed, improvementfrom diuretics suggests the predominant cause of

the oedema is not lymphatic. Nevertheless, theiruse in oedema of mixed origin or in combinationwith other therapy may be helpful.The benzo-pyrones, e.g. oxerutins (Paroven),

a group of drugs related to coumarin, have beenadvocated for use in lymphoedema, although theyare licensed for venous disorders. Recent workhas shown objectively their effect in normalizingincreasing capillary permeability,28 and a trial hasdemonstrated benefit in lymphoedema, althoughthis needs substantiating.29

SurgeryLymphoedema is considered a surgical

condition, but unfortunately there is, as yet, no

surgical solution. Until recently all operationshave aimed simply to reduce limb size.3oAdvances in microvascular techniques and theintroduction of lymph node-venous shunt andlymphovenous anastomosis operations3l-32 haveproved of temporary benefit. Debulking of tissuein the grossest limbs may at times still be



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performed, but improvements in medicaltreatment make this less necessary.

Physical therapySpecific physical therapy aims essentially to

control

lymphformation and

improve lymphdrainage through existing lymphatics andcollateral routes by applying normal physiologicalprocesses which stimulate lymph flow. Peripherallymph flow depends upon two factors: first,lymph absorption and flow throughnoncontractile lymphatic vessels, and secondly,lymph propulsion through contractile lymphvessels; valves ensure unidirectional flow. Thefirst step depends on changes in local tissuepressure produced by external pressure,33 skinsurface massage, 34,35 arterial pulsation,36 passive

movements,37,38 and muscular activity.39 Thesecond step involving flow in contractile lymphvessels depends on the volume of fluid enteringeach lymphatic segment or lymphangion. Thestimulus to lymphatic contractions is the fillingand distention of vessels4; the characteristics of

these contractions have some similarity to thoseof the heart. 41 Muscular activity and exercise

produce the greatest lymph propulsion.Obstruction to main lymphatic drainage routesshould result in the opening up of collateralroutes, as is known to happen in venous

obstruction. However, without sufficient vis atergo collateral drainage may not satisfactorilyoccur. The principle of physical therapy dependsupon performing procedures which would

normally enhance lymph flow and thereforemaximize remaining lymph transport capacity. Itis claimed swelling can often be reduced

dramatically with this therapy, but there is little

objective published evidence. 42

External supportExternal support is the cornerstone of good

medical treatment for several reasons:

1) Limiting blood capillary filtration by raisinginterstitial pressure.

2) Opposing tissue expansions.3) Improving striated muscle pump efficiency.

Elastic hosiery exerts a controlling externalpressure and serves as a containment garment tomaintain limb size. In rare instances

improvements can be achieved as a result of a

more efficient muscle pump enhancing lymphdrainage.Agarment must be well fitting andcomfortable, otherwise patient compliance willbe poor; equally, a torniquet effect may beproduced particularly if it rolls down or is foldedover.

Strong hosiery exertingpressures

> 30mmHg is usually required, and it is oftenworth the extra effort and expense of startingwith lower compression in order to allow timefor the patient to become accustomed to thefitting and wearing of these garments. Lymph-oedema invariably requires the highest com-pression strength (> 40mmHg) hosiery, anddouble hoses may need to be worn on some

occasions to maintain control. Close collaborationis advised between clinician, fitter and surgicalappliance officer. Most garments last six months

before renewal is necessary, and it is wise to pro-vide two pairs, one to wear and one for the wash.External support may also be provided by

bandages, but usually only in a palliative caresituation or where hosiery cannot be managed.Bandages are most often used for compressiontherapy in limb reduction. The technique ofcompression bandaging is based on the principleof applying a strong, nonelastic bandage as firmlyas possible; this allows a high pressure duringmuscular 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 tothe shape of the limb but do not yield, andtherefore act as a firm outer collar duringmuscular contraction. The use of foamunderneath distributes pressure evenly, therebyminimizing constricting bands, and thepositioning of rubber pads helps iron out pocketsof swelling such as collect on the dorsum of thefoot or the back of the hand. Multiple layers of

bandage serve to generate higher compression(Figure 7).

MassageTissue movement is a stimulus to lymph flow,

and by creating the correct incentive and guidancefor lymph flow there should be sufficient reservetransport capacity, even in an obstructed system,to allow drainage by collateral routes, as occursin the venous system. Optimal isotonic exercise



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in the presence of external support enhances

lymph pumping, as it does in venous return inchronic venous insufficiency. If performedcorrectly, skin surface massage encourages themovement of lymph through the intercom-municating network of skin and subcutaneous

lymphatics to normally drained areas. Truncaland facial lymphoedema can only be influencedby massage, as external support or compressionare impractical.Areduction in swelling andcongestion at the truncal root of a limb will allowfreer drainage from that limb via skin andsubcutaneous collaterals to normally draininglymph node areas, thus bypassing the obstruction.This is the principle of manual lymphatic drainagepractised in many parts of Europe.46

Pneumatic

compression theoryPneumatic compression therapy is widelyemployed.44 Consisting of an inflatable sleeve orboot connected to a motor driven air pump, thesemachines reduce limb volume by displacementof fluid and help soften a limb by disruptingtissue fibrosis.45 If external support is notfitted immediately following compressiontherapy, a limb readily reswells. The firstmachines possessed a single chamber boot orsleeve (Flowtron, Jobst), and discomfort fromsustained pressure was often a disadvantage.

The Lymphapress (Huntleigh Technology)consists of a multichamber garment, eachchamber filling consecutively to displace fluidproximally. More recent machines, e.g. TalleyMulticom (Talley Medical Equipment Ltd)provide sequential filling of a ten-chamberinflatable garment, thus delivering a rippleeffect up the limb. The multichamber machines

appear more effective than the standard

single chamber intermittent pumps.46 Caremust be taken with pneumatic compression toensure that fluid is not forcibly displaced

from the limb to the adjoining quadrant of thetrunk, where new swelling may develop andpersist.

Heat treatment

Anovel treatment involving heat and bandageshas recently been reported from China, in a studyof over 1000 patients with chronic lymphoedemaof the extremities. 47 Using an electro controlledinfrared heating chamber, the affected limb was

heated to an ambient temperature of 80-90C forone hour daily for 10 days.After each treatmentan elastic bandage was applied. Up to five (10-day) courses of treatment were administered, andresults from limb circumference measurements

revealed more than 50% resolution of swellingin over 60% of patients. Despite examples ofimproved lymph drainage demonstrated by lym-phangiography and lymphoscintigraphy, the mostlikely mechanism for the success is denaturing ofextracellular protein and improved phagocytosiswith enhanced resorption of material directly intothe blood stream. Microwave heating has nowsuperseded the infrared oven and appears ofequal benefit. 48 Clearly this form of treatmentneeds more careful evaluation. It is difficult tounderstand how the patients tolerate the high

temperaturesused. Success of

lymphoedematreatment must depend on the permanent controlof swelling and unfortunately, there are few, ifany, reliable long-term results.

Midline lymphoedemaMidline lymphoedema such as that occurring

on the face or involving the genitalia is fortunatelyrare. Presumably this is because bilateral lymphdrainage routes operate and, although well-defined anatomical lymph drainage areas exist,cross-flow from one region to another can occur

includingacross the

midline. Facial lymphoedemadevelops in circumstances where skin orsubcutaneous lymphatics fail. Inflammatoryprocesses such as acne rosacea or erysipelas areusually responsible (Figure 8), and treatment isextremely difficult, even after the cause has beenbrought under control. Swelling frequentlyconcentrates around the eyes, and closed

palpebral fissures on waking can be distressing.Patients may need to rise early, sit upright foran hour or so and practise frequent blinkingbefore normal vision is restored. Sleeping with

the head of the bed raised may limit the overnightaccumulation of oedema, and gentle massage tolymph node areas may siphon away much of thefluid. Drug therapy is ineffective and externalcompression of course impractical. Surgery is thetreatment of choice.

Lymphoedema of the genitalia can be equallydifficult, particularly in the male. It arises mostcommonly from pelvic or bilateral inguinalsurgery or radiotherapy. Lax tissues permit gross



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Figure 8 Facial lymphoedema secondary to chronic rosacea - a chronic inflammatory disorder of the pilo sebaceous unitswhich damages cutaneous lymphatics

swelling. Surgery, if feasible, can be helpful,otherwise palliative care with a good scrotalsupport is the only other option.

LymphangiomaDilation of the upper dermal lymphatics to an

extent where they visibly bulge on the skin surfaceis referred to as lymphangioma. Obstruction of

deep dermal or subcutaneous collectinglymphatics due to a congenital abnormality istermed lymphangioma circumscriptum .49 Dila-

tation of surface lymphatics can also occur fromsubcutaneous fibrosis in association with

lymphoedema secondary to surgery orradiotherapy. Such changes, although clinicallyand histologically indistinguishable from

lymphangioma circumscriptum, are best referredto as acquired lymphangioma50,51 (Figure 9). Suchlesions release lymph and serve as a portal ofentry for infection. Piecemeal excisions onlyresult in further lymph blisters appearing

alongside. Cautery or laser therapy followed bysustained external pressure during the healingphase to seal the skin surface can provesuccessful treatment but relapse is common.

Volume measurements

Close monitoring of limb volumes is necessaryfor the assessment of treatment progress. While

subjective improvement is important for thepatient, objective measurement of swelling mustbe performed if new, or even existing treatment

regimes are to be evaluated properly. Waterdisplacement has been considered the goldstandard for limb volume measurement, but it is

cumbersome, and calculation of volume bymultiple circumferential measurements has

proved more reliable and convenient. From afixed point such as the heel a distance is measuredto what is considered the ankle (or in the upperlimb a distance is measured from the tip of themiddle finger to the wrist). From the ankle or



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-..

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

Figure 9 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 ismeasured. By calculating the volume for each4cm segment according to

and totalling the values, the volume of the limbas a cylinder from wrist to shoulder (or ankle tohip) is obtained. The method achieved good re-

producibility when performed by more than oneindividual.

Assessment of truncal swelling is more difficult.Measurement of pitting by tonometry has beenused,52 but its value is uncertain.Future progress in the investigation and

management of lymphoedema will dependentirely on a greater research input.Afullerunderstanding of the pathophysiology oflymphoedema is necessary before more effective

treatment can be implemented. Most cases oflymphoedema secondary to cancer therapy shouldbe preventable. This relies on identifying thoseindividuals at risk by measuring deterioratinglymph drainage function before swelling occurs.This should become possible as methods ofinvestigation improve. The expectation withcurrent medical therapy is at least to preventprogression of swelling and at best to reduce limbsize to that approaching normal. Successfultreatment

dependson

highmotivation and

perseverence on the part of the patient, and thenlong-term reductions in limb volume can bemaintained (Figure 9).

AcknowledgementsThe author wishes to thank Caroline Badger

(Clinical Nurse Specialist in lymphoedema at theRoyal Marsden Hospital) for her help in thepreparation of this article.



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10a

10b

Figure 10 Successful treatment by physical therapy of lower limb lymphoedema with maintenance of limb size by elastic

hosiery one year later a) before treatment; b) one year later



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