LYMPH(EDEMA P R ~ C O X A N D SOME EXPERIENCES IN ITS TREATM..~NT

By JOHN WATSON, F.R.C.S.(Ed.)

From the Queen Victoria Hospital, East Grinstead

THE nature of the lymphatic derangement in this curious condition, with its insidious onset without obvious exciting course, and showing inexorable progress over the passage of many years, has never been satisfactorily established. A study of the literature conveys two main impressions of the pathology of the

condition: First, that it is carried by a mechanical block of the main lymphatic trunks of the limb at the proximal end or more centrally; the nature of the obstruction is unspecified and its site obscure. Secondly, that the condition affects the superficial lymphatic circulation only, the deep lymphatic remaining normal, a supposition engendered by the striking secondary hypertrophic changes in the veins and subcu- taneous tissues of the limb, compared with the normal appearance of the tissues beneath the deep fascia1 envelope.

In the course of treatment or surveillance of thirty-five patients with idiopathic lymphoedema at the Queen Victoria Hospital over the past few years, repeated measure- ments taken of the girth of the limbs of advanced cases conveyed an impression that the rapidity and magnitude of the changes which occur with alteration in posture were greater than would be expected from what is known of the normal rate

F~G. i and volume of lymph production. Simple tank for measurement of volume changes in Recently an endeavour has been

limbs by water displacement, made to make more accurate estima- tion of changes in limb volume by the

simple method of displacement of water by the limb in a tank constructed for the purpose (Fig. I). Fig. 2 illustrates a fairly typical case of bilateral lympheedema pr~ecox in a young man of 22 years. The lymphoedema first appeared at the age of I5 years, and a limited operation of the Kondoleon type was carried out three years ago without relief. Fig. 3 shows the rate of fluid loss from the left leg when

224

LYMPH(EDEMA PR/ECOX AND SOME EXPERIENCES IN ITS TREATMENT 225

put at complete rest, with the foot of the bed blocked and rubber compression bandages applied to the limb. There is a loss in volume of about one litre a day for the first three days ; by the eighth day the leg, although it has not regained its former size on account of tissue hypertrophy, cannot be further emptied. At this stage the not inconsiderable total of almost four litres has been lost from the limb. Severe bilateral cases may be carrying eight, ten, or more litres of excess fluid about in their lower extremities. In the case illustrated it is evident that, provided the rate of formation of lymph is not greater than about half a litre daily,

FIG. 2

Patient with lymphcedema prmcox whose limb volume changes are recorded in Figs. 3 and 4.

the limb should return to normal size by morning after a night's rest, the rate of removal at rest being adequate to dispose of this volume of daily accumulation. It is at first sight rather odd that this is not the case ; Drinker and Yoffey~(I94 I) and other workers who have investigated normal lymph flow all comment on its paucity of volume, and show how the capacity of the normal lymphatic system both for the production and the removal of lymph is low so far as volume is concerned. The normal daily production of lymph in the leg might be roughly estimated to be of the order of a few hundred millilitres at the most, and one might expect that when this patient gets up there would be a relatively slow reaccumulation of lymph over a number of days. Fig. 4 shows that nothing of the kind occurs. A considerable volume of fluid reaccumulates in a matter of hours. The steeper curve shows the reaccumulation of fluid in the first eight hours after getting up. During this period approximately two litres of fluid reaccumulate, 8oo ml. in the first hour. This alone is a greater volume of fluid collecting in one hour than has been measured from the cut end of the thoracic duct in man in a day. The second curve shows the rate of accumulation on the second day; it shows the rather

3D

226 BRITISH JOURNAL OF PLASTIC SURGERY

marked effect of posture--the patient being seated during visiting hours in the afternoon. Each successive day is begun with a greater volume of fluid in the limb than the previous day, but the rate of accumulation is progressively slower as the tissue pressure increases with the increasing distension of the tissues.

The reaccumulation of fluid at such a rate suggests two possibilities : either the presence of a grossly abnormal capillary filtration rate, with exudation of tissue fluid faster than the veins and lymphatics can dispose of it, or else, and as seems more likely, that most of the fluid accumulating is not being formed in the leg at

LITRE S

2 LITRE S

2 3 4. S 6 7 8 9 10 DAYS

FIG. 3 Reduction in volume of leg with rest

and compression

/ 2 4 6 8 10 12

HOURS

FIG. 4 Rapid rate of return of fluid into the

same limb. Steeper curve : First days with patient

mainly ambulant. Upper curve: Second day. Arrows indicate period when patient was seated.

all, but is flowing in a retrograde direction down the limb from above. It seems unlikely that such a rapid rate of fluid accumulation, even allowing for osmotic disturbances arising from the escape of protein from the vessels, could be explained by abnormal capillary permeability, especially when the condition is unilateral.

It seems notable, therefore, that the lymph is flowing in a retrograde manner down the limb along incompetent lymphatic pathways under the influence of gravity. The rapid rate of refilling is, of course, quite the opposite of what would be expected if there were a complete block present in the proximal lymphatics, when the refilling of the limb would be a more gradual process. It is difficult to assess what is occurring in early mild cases of lymphoedema prmcox, where some lymphatic channels may still be functional, but there are strong indications that in the advanced cases the massive accumulation of quantities of fluid in the limb and the accompanying tissue hypertrophy are largely due to the reflux of lymph down dilated, incompetent, and valveless channels. In its later stages the condition seems to be one of lymphatic incompetence rather than blockage, somewhat analogous to venous incompetence.

In the uncommon group of cases in which the lymphatic accumulation is chylous, there can be no question that reflux occurs. Such a case was kindly referred for treatment by Mr Basil Hume. This patient had a long history of

L~MPH(EDEMA PRALCOX AND SOME ~XPERIENCES IN ]ITS TI~EATM~NT 227

chyluria, chylometrorrhoea, and lymph(edema of the right leg. Chronic ulceration was present on the dorsum of the foot, an unusual feature in ordinary lymph~edema. The skin of the thigh and leg was speckled with small vesicles which occasionally ruptured to leak a milky fluid. The plan was to excise the ulcerated skin of the foot and leg and replace it with a free graft, and to reduce the upper third of the leg, here replacing the original skin as a Wolfe graft. Before doing this it was thought wise to interrupt the lymphatics proximally in an attempt to control the chylous reflux, as recommended by Servelle and Deysonn (I949). This interruption was carried out below the inguinal ligament (it would probably have been better to do this in the pelvis) in order to investigate the condition of the superficial glands and lymphatics in the groin. Before operation she received a fatty meal containing Sudan 3, and this dye could not only be recovered in the urine following absorption (contrary to two normal controls) but also discoloured the vesicles on the thigh. At operation it was an impressive sight to see quantities of chyle escaping from the superficial lymphatics in the groin so that several test tubes could be filled, applying them to the wound without the use of pressure or massage ; the fluid was milky and was found to contain Sudan 3. It was interesting to see that the inguinal glands, which one might expect to form some kind of barrier against reflux, failed to provide any such protection ; in fact, no glands could be identified, having apparently disappeared in a mass of fibrosis. A wedge of inguinal tissue containing dilated lymphatics was resected and the femoral canal cleared. The chylous reflux in the thigh was temporarily arrested by this procedure but later recurred; it seems probable that a systematic occlusion of all femoral and hypogastric dilated lymphatic trunks in the pelvis would give excellent prospects of curing the reflux element in these cases.

Most cases, of course, do not show the presence of chyle in the leg but only clear lymph. Are such cases attributable to a purely local lesion of the lymphatic trunks, and are they in any way linked with the congenital cases ? One has the impression that these patients suffer from some form of inborn lymphatic insufficiency which may remain occult for many years ; when symptoms do arise gravity is a major influence. Not infrequently the disease begins in one leg and subsequently involves the other, or extends upwards to involve the genitalia or lower abdominal wall. Sometimes more than one limb is involved from the start ; Fig. 5 shows such a case in a girl of 19 years in which first an arm and subsequently a leg became affected. Another patient who came under treatment for congenital lymphoedema of the legs was born with generalised oedema of the face, arms, and legs. During infancy the swelling gradually left first his face, then his arms, finally gravitating to his legs when he began to walk. He would appear to have been born with a general insufficiency of the lymphatic system, but the final manifestations of the disease were determined by gravity.

Lympheedema is sometimes congenital, sometimes familial, and rarely both. Fig. 6 shows the family tree of one patient born with a bilateral lympheedema of the legs. This seems to be a true case of Milroy's disease, being both congenital and familial. It is apparently transmissible by both sexes and may skip a generation. In the type of case which is familial but not congenital, the condition may come on at any age but commonly seems first to arise in adolescence or early adult life. Why, if this is really a congenital lesion, is the eedema not present at birth ? It seems possible that such patients suffer from a congenital lymphatic insufficiency, either in the number of lymphatic trunks or in their size, or in the

228 BRITISH JOURNAL OF PLASTIC SURGERY

failure o f some of t hem to make connec t ion wi th the central lympha t i c c i rculat ion f rom the s tar t ; this is masked un t i l the g rowth of the tissues dra ined outstr ips

FIG. 5 Lymphoedema prmcox of both right arm and right leg in a girl of 19 years.

FIG. 6 Family tree of a case of Milroy's disease.

X-patient. [] female. 0 -male. Dark quadrant =limb swollen at birth.

the available capacity of the lymph channels; mild lymphcedema then occurs, and sometimes first begins after some slight injury such as an ankle sprain, a bruise, or an insect bite, or any injury which temporarily increases the capillary filtration

L Y M P H ( E D E M A PR21E, C O X A N D S O M E E X P E R I E N C E S I N I T S T R E A T M E N T 2 2 9

rate. Finally, more massive enlargement occurs as lymphatic incompetence allows reflux of lymph.

There has been much discussion regarding the means by which lymph is propelled proximally along the normal main lymphatic ducts. It seems significant that the ducts are relatively narrow in character, constant in calibre, not enlarging as they pass proximally, contain valves which project in the lumen, and both muscle and elastic tissue disposed in circular fashion in their walls. When injected with dye the entire trunk distends and fills with great rapidity, giving the familiar beaded appearance, as though it had previously been empty; it discharges its contents in a unidirectional manner very much more gradually. It does not seem unreasonable to suppose that the muscle and elastic tissue in the duct wall, stretched by distension with fluid, exert a pressure higher than the general surrounding tissue pressure on the contents, sufficient to propel the lymph up to and through the regional gland, the valves ensuring propulsion in the correct direction. I f this is true it is evident that constant dilatation will upset completely the valvular mechanism, no forward flow will occur, and a progressive stagnation must result. It is to be hoped that X-ray lymphangiography (Kinmonth, 1954) will provide the answer to a number of these questions.

Turning to the question of treatment, most operative procedures to-day are designed with two aims in view : to remove the lymph-sodden and hypertrophied subcutaneous tissues in which the fluid mainly accumulates, and to provide an inelastic skin support so that an early rise in tissue pressure in the limb determines a proximal disposal of the lymph. It is the author's practice (Watson, 1953) to remove the subcutaneous tissues and deep fascia from knee to toes in two stages, raising the skin at Wolfe-graft thickness, removing all fat, and returning it to the muscle layer as a graft (Fig. 7)- The thin flaps retain some circulation from the dermal and subdermal plexuses, as can be seen from their return of colour on release of the limb tourniquet, but do not survive on this limited supply alone, adhering mainly as free grafts; there seems to be no lack of arterial supply but an insufficient venous drainage. A two-stage operation, though tedious, does ensure that if skin should slough such necrosis is unlikely to occur over the subcutaneous surface of the tibia or over the tendo achillis. Skin occasionally sloughs marginally along the lateral suture line or near it, more commonly in the middle-aged patient than in the young. Even narrow areas of loss are usually free grafted in order to avoid delay in healing and the rather objectionable hypertrophic scar which results. The skin retains some sensitivity to pain and temperature, which is sufficient to avoid accidental burning from a hot-water bottle. Since first carried out in 1948, this method has in twenty-two personal cases of lymphoedema preecox provided reasonably satisfactory palliative treatment, and the results seem to be permanent over a number of years. The skin is thick and durable. At the beginning (Fig. 8) the leg appears overattenuated, but during the following months regains some of its normal contour ; the right-hand half of the photograph shows the result four years later. I f one re-operates on such a case, for example, to clear the pads of oedematous tissue which tends to protrude from beneath the Achilles tendon at the ankle, the skin is found still firmly adherent to the muscle layers, and the expansion which has occurred since operation is apparently due to a swelling of the deeper structures, an indication of the involvement of the deep lymphatics as well as the superficial in these cases. Recently a patient with a severe lymphoedema pr~ecox was seen who required for

230 BRITISH JOURNAL OF PLASTIC SURGERY

subastragaloid fusion a bone graft which was removed from the tibia ; clear fluid was observed exuding freely from the marrow cavity o f the bone ; regardless of

A B

FIG. 7 Routine method of treatment.

A, Pre-operative condition. B, Elevation of flaps, excision of

subcutaneous tissue and deep fascia, and return of flaps after excision of redundant skin and perforation.

C, Six months later.

C

whether or not bone carries lymphatics, fluid retention was certainly present in t h e deepest structures of the limb.

Trea tment by excision and application of free-grafted skin from elsewhere has been carried out on a limited number of severe cases in which the method outlined'above was considered unsuitable because o f gross skin changes, or multiple scarring from previous operations. T h e late results have on the whole been unsatisfactory, being marred by the recurrence o f papillomatosis on the skin in those cases for which free grafting was adopted in an at tempt to improve the

LYMPH(EDEMA PRPECOX AND SOME EXPERIENCES IN ITS TREATMENT 231

A B FIG. 8

A, Immediate post-operative appearance showing gross overattenuafion of limb. B, Same case four years later showing slight increase in girth.

A B C FIG. 9

A, Lymphcedema prmcox with warty papillomatosis and infolding of skin. B, Post-operative appearance after replacement of skin and subcutaneous tissue

with free grafts. C, Poor result two years later.

232 BRITISH JOURNAL OF PLASTIC SURGERY

quality of the skin, and by the recurrence of swelling with a tendency to lymphatic leakage and consequent eczema in other cases. Fig. 9 illustrates such a case in which free grafting was carried out on account of the presence of patches of warty papillomatosis on both legs. The grafts took without difficulty, but the ultimate result is very poor, with recurrence of skin changes and stretching of the graft by oedema two years later. Other cases after a lapse of four to five years have shown

FIG. IO

A, Papillomatosis of toes, with formation of deep crypts. B, Result of surgical removal.

various complications, including recurrence of swelling, leakage of lymph with consequent chronic dermatitis, and papillomatosis over the surface of the graft. These unsatisfactory results are almost certainly due to the fact that the skin, taken from elsewhere on the body, was cut at ordinary split-thickness level of I5/2o thousandths. Such a thin graft is insufficient to support the high internal pressure occurring within these limbs. The highly satisfactory results reported by Gibson and Tough (I954) were by contrast obtained with the use of local skin cut with the inclusion of all, or nearly all, the dermis. The unsuccessful cases described above illustrate that simple excision and grafting with split-thickness free grafts of the usual thickness does not provide an answer to the problems of treatment of advanced cases.

LYMPH(EDEMA PR~..COX AND SOME EXPERIENCES IN ITS TREATMENT 233

Not infrequently the level of the oedema is sufficiently high to involve the genitalia and lower abdominal wall. In women the development of elephantiasis of the vulva can be a source of considerable disability, with massive swelling, warty excrescences, constant discharge, chafing and excoriation, painful inflammatory episodes, and associated marital difficulties. The maximum impact is upon the labia minora, which sometimes become grossly enlarged and form pedunculated masses; the labia majora and vaginal wall are also involved. It is well worth while removing the redundant tissues surgically, even if the relief afforded lasts for only a few years. Through two lateral elliptical incisions the whole of the labia minora, the greater part of the labia majora, and the underlying hypertrophied and lymph-sodden tissue are excised, and the vaginal lining is sutured to the surrounding skin; a self-retaining catheter is advisable during the first few post-operative days. It has been found that great relief of symptoms is obtained from a reducing operation of this nature.

Gross papillomatosis on the dorsum of the toes is a complication presenting a problem in treatment. These outgrowths may extend to an inch in height, and may prevent the fitting of normal shoes. Deep clefts are present between the papillomatous masses, and these ale not only difficult to keep clean but also commonly harbour pathogenic fungi. It seems likely that the fungi are secondary invaders occupying an ideal habitat rather than the cause of the papillomatosis as has been suggested ; it is, in any case, impossible to treat a fungus infection satisfactorily in the depths of the crypts between the outgrowths. This condition (Fig. IO) may be treated rather as one might deal with a rhinophymatous nose; the papillomata are pared down with a knife blade until almost the whole thickness of the skin of the toes has been excised. It is remarkable how thick a layer of tissue can be excised and rapid healing still occur. Usually the fungus infection is no longer evident after elimination of the crypts, but if still present is accessible to treatment. It is unlikely that a permanent result can be achieved, but the resulting interstitial scarring which results in the skin discourages rapid recurrence of the papillomatous masses.

SUMMARY

I. The nature of the pathological lesion in lymph~dema pr~ecox is discussed, with particular reference to lymph reflux and lymphatic incompetence.

2. The influence of gravity as the determining factor in the site of manifestation of the disease is stressed rather than local mechanical obstruction.

3. Various methods of palliative treatment are discussed.

I am indebted to Mr Gordon Clemetson for the photographs.

REFERENCES

DRINKER, C. K., and YOI~FEY, J. M. (I94I). " Lymphatics, Lympb, and Lymphoid Tissue." Cambridge, Mass. : Harvard University Press.

GIBSON, T., and TOUGH, 1. S. (1954). Brit. J. plast. Surg., 7, 195. KINMONTH, J. B. (I954). Ann. R. Coll. Surg. Engl., xS, 3oo. SERVELLE, M., and DEYSONr¢ (1949). Arch. Mal. Ceeur, 42, 1181. WATSOr~, J. (1953). Brit. ft. Surg., 4I , 31.