Last update and review: August 28, 2021.
A short summary.
While reviewing literature on chronic venous disease (varicose veins, etc.) and edema, we came across a quality text on classification and surgical treatment of lymphoedema in the 1950s. We reproduce the text the article below. The classification and the proposed mechanisms are of interest despite the fact that the text was written in the 1950s. There is also a description of a recent case of lymphoedema cause by heart surgery.
The conclusion of the author is very sound: edema, leg swelling, and associated inflammatory process, should be treated and brought under control at the earliest stages.
“[April 5, 1950] – DISCUSSION ON THE TREATMENT OF CHRONIC (EDEMA OF THE LEG).
Sir Archibald McIndoe, Queen Victoria Hospital, East Grinstead, Sussex:
Lisfranc in the first half of the nineteenth century treated chronic lymphoedema by scarification of the limb.
“Chronic lymphoedema still continues to resist surgical cure. In fact one may state categorically that the condition, ever since Lisfranc in the first half of the nineteenth century treated it by scarification of the limb, has proved one of the most disabling and disheartening to the patient and one of the most disappointing in its treatment to the surgeon.
Chronic lymphoedema falls broadly into two groups: (a) Parasitic, due to blockage of lymph channels by the adult forms of Filaria bancrofti or Filaria malayi. (b) Non-parasitic, due to lymphatic obstruction from other causes.
Chronic lymphoedema falls broadly into two groups: (a) Parasitic, due to blockage of lymph channels by the adult forms of Filaria bancrofti or Filaria malayi. (b) Non-parasitic, due to lymphatic obstruction from other causes. The parasitic form occurs only in tropical climates and its distribution coincides with that of filariasis. It will not be further considered here. The non-parasitic form may be met with anywhere in the world and is common throughout the British Isles. It occurs in four distinct forms:
(1) Congenital and familial lymphoedema (Milroy’s disease, 1892, 1928).
This rare disease is probably due to a congenital lymphangiectasia. Many reported cases are not true examples of the condition.
(2) Idiopathic lymphoedema.
This is commonly encountered in Great Britain in adolescent females. It has an insidious onset and a painless progress. It can produce enormous enlargement of one or both legs. Though it is said to be noninflammatory it is probable that the lymphatic damage is due to a low-grade inflammatory process for in the later stages many cases exhibit typical attacks of inflammation.
(3) Elephantiasis streptogenes nostras or inflammatory lymphoedema.
This follows repeated erysipelatous attacks often spreading from nodal points in the legs. With each attack swelling becomes more pronounced, the causative organism being either a streptococcus or a staphylococcus. The clinical course of the disease is characteristic and unmistakable.
(4) Secondary lymphoedema.
It comprises a mixed group in which the cause is usually clear. Venous insufficiency and other vascular disorders predominate. From this large group of cases idiopathic and inflammatory lymphoedema accounts for the majority of chronically swollen arms and legs in Great Britain, in which surgical aid is sought.
Image: McIndoe operating at East Grinstead: a painting by Anna Zinkeisen, 1944
Surgical Considerations
True lymphatic edema can be produced by many agencies but in all cases the actual mechanism is the same. Drinker and Field (1933) have shown that obstruction to the main channels of lymphatic escape from the limb produces edema which can become chronic. Progressive destruction of the large lymphatics, by internal blocking, by intimal sclerosis or by external pressure, is followed by dilatation of the peripheral network of lymphatics. The valves become incompetent, the protein content of the retained fluid is high and progressive fibrosis of the waterlogged limb follows. Attacks of inflammation in the sea of lymph begin to occur even when inflammation has not been clinically evident at the onset of the disease. Thus the vicious circle is complete and the damage to the lymphatic endothelium progressive and irreversible. Eventually there comes a time when, even if the obstruction were completely relieved, the normal vis a tergo responsible for the onward flow of lymph would be quite incapable of emptying the dilated network or of overcoming the effects of gravity. The anatomy of the lytphatic system.-All tissues, except the placenta, possessing blood x essels also have lymphatic vessels-in fact the evidence suggests that the latter form a sort of accessory venous system. The lymph vessels begin in a closed capillary bed without stomata and have no communication with other body spaces. Drinker and his colleagues (1934) have shown that the vascular endothelium allows salts, electrolytes and proteins,to pass through into the tissue spaces from which proteins are differentially absorbed into the lymphatics through the lymphatic endothelium. The subepidermal layer of lymphatic capillaries is rich and freely anastomotic over fairly ill-defined focal areas, but anastomosis between these focal areas is poor or absent. These focal areas are drained by short collecting lymphatics into much’ longer, valved supra-aponeurotic trunks. These larger draining vessels are few in number, and are so polarized by valves of such strength that retrograde injection is impossible without rupture of the walls. They end in regional lymph nodes without again anastomosing with each other. The subaponeurotic deep lymphatics begin and end in the same way and little if any anastomosis takes place between the two systems. The most important barrier to the establishment of any such anastomosis is obviously the presence of fascial planes. As a result of the peculiar disposition and construction of the lymphatic system it is clear that peripheral obstruction can be fleeting in its effects while central obstruction is the almost universal requirement for the production of chronic oedema. Experimentally it is exceedingly difficult to produce chronic cedema as the oft-quoted experiment of Reichert (1926, 1930) so clearly showed, but Drinker and Field have been able to demonstrate that the extent and duration of the lympheedema depend on the nature, extent, situation and duration of the obstruction and lastly upon the ability of the individual to develop a compensatory lymph circulation or to regenerate vessels to replace those destroyed. The objects of surgical treatment ale clear when we bear in mind the pathology of the condition. (I) The retained lymphatic fluid may be drained into other channels peripherally thus reversing the lymph flow. This idea first stimulated Sampson Handley in 1908 to attempt drainage of cedema fluid into the normal tissues of the chest wall and abdomen by means of silk threads. Walther (1919) used rubber tubes and Lanz (1911) fascial strips inserted through the deep fascia into the bone marrow. Kondoleon (1912a, b) devised the operation which sought to create an anastomosis between the superficial and deep lymphatics. This operation modified by Sistrunk (1918, 1927) and Ghormley and Overton (1935)’has been widely practised. It would be freely admitted by- almost everyone with experience of it that very few patients derive lasting benefit and that not more than 25% of’patients are improved by more than 25%. Occasionally mass reduction in size confers some benefit but on the whole it is a very disappointing operation. Practically every patient, however, with chronic lympheedema has at some time or other been submitted to it. In defence of it one can say that it is the only straightforward operation yet’ described which is within the compass of the general surgeon and which offers even a modicum of hope to the patient. Improvement, if there is to be any, can only be attained by very careful post-operative elastic rubber bandaging maintained for at least one year. (2) The central obstruction may be by-passed: Gillies and Fraser in 1935 proposed the grafting of a lymphatic bearing bridge of skin from arm to leg or from one arm to the other, inserted in such a way that one end lay in the cedematous tissue of the affected limb and the other projected into the nearest available lymph drainage area. In the case of the leg, the lymphatic wick projected into the axilla, with the arm into the opposite axilla. In all cases the polarity of the lymphatics in the graft had to correspond with those in the limb. It may fairly be said that the results were better than with Kondoleon’s operation and that the wick actually functioned. But it would not do so in the erect posture when the lymphatic valves in the affected limbs were incompetent and the vis a tergo was insufficient to propel the retained fluid upwards. With the arm or leg elevated, however, rapid emptying, took place and the patients reported .that’ the- lymphatic” bridge fairly tingled with’ the ‘outpouring’of fluid. Those cases with ulceration of the skin were definitely benefited. The operation is, however, lengthy and difficult to the point where it should not be performed by other than plastic surgeons with a knowledge of flap transfer. (3) Total removal of the lymphatic bearing area: This idea is not new. Charles in 1912 described in detail the treatment of a case of filarial elephantiasis in which he excised all the subcutaneous tissue from the leg and covered the limb with free skin grafts. Homans (1936) and Mowlem (1948) reported success with this method. Recently in New York I saw several cases dealt with by Stephenson with encouraging results but the magnitude of the operation made it less impressive. As it seemed that there was little evidence that the existing skin cover of the limb was primarily diseased, I asked Mr. John Watson and Mr. Norman Hughes to undertake a series of cases in which after resecting all subcutaneous tissue and deep fascia down to muscle, they would replace the skin of the limb cut to a thickness approximately that of a Wolfe graft. Such a proceeding would have the advantage of greater economy of skin and less disturbance to the patient than flaying him elsewhere. They have carried this procedure out on 8 patients, 7 in the leg, 1 in the arm; 6 were idiopathic, 1 congenital and 1 filarial in origin. The operation is carried out in, two or more stages and the grafts opposed to the muscles under suitable pressure. The grafts took surprisingly well despite their large size and in only one case was a slough troublesome. The results in these cases have been impressive and the method deserves further trial. It may be that the grafts act as a kind of strong elastic stocking preventing the accumulation of further fluid. It may be that the removal of practically all the lymph-bearing tissue is curative. It is of course impossible to strip the limb completely, especially round the tendo achillis and the foot, so that one can only say that, where removal has been complete, recurrence of cedema has not taken place. In my view after considerable experience of the three methods which f have described, this is the most hopeful but it is not one which could be commended as suitable for any but a plastic surgeon. Failure with these enormous grafts might have serious consequences.
Conclusion.
The foregoing is a brief description of a plastic approach to a surgical problem which is itself the end-result of a condition which should be medically curable in the earliest stages. I look forward to the time when the inflammatory process originally responsible can be brought under control as surely as with other inflammations and that the all too common condition of chronic lymphoedema will become a rarity.”
A modern description of a rare case of lymphedema.
Lymph node excision during a surgery may result in lymphedema.
Classification of lymphedema by Onoda et al., 2016 (2).
Onoda et al., 2016 (2):
“Lymphedema is classified into two main types: secondary lymphedema accompanied by lymph node excision surgery or radiotherapy; and idiopathic lymphedema. Here we experienced a very rare case of lymphatic edema resulting from cardiac surgery that the patient underwent 50 years previously.”
“Presentation of case
A 62-year-old woman experienced progressive unilateral lower leg lymphedema for recent years. After undergoing cardiac surgery at another hospital at the age of 12 years, she gradually developed left lower leg edema. The cause of the edema was unclear and it remained untreated. Her edema symptoms gradually worsened in recent years, so she consulted the plastic surgery division of our hospital.“
“Self-massage and compression therapy using garments, were immediately started. With these treatments, the leg volume and edema symptoms reduced.”
“Fig. 2” from Onoda et al., 2016 (2), – the left lower leg after treatment.
Onoda et al., 2016 (2):
“Discusssion
Perhaps the lymphatic structures of affected individuals differ prior to disease onset. If the mechanism of lymphatic edema outbreak is elucidated, patients needing conservative and surgical therapy might be more easily identified. Knowing the outbreak mechanism of lymphatic edema would definitely ease the investigation of an unconventional case like this one. Conservative treatments for lymphedema, such as self-massage and compression therapy using garments, were immediately started. With these treatments, the leg volume and edema symptoms reduced.
Conclusion
The research on the cause of this case may be important step for elucidating the source of secondary lymphatic edema.”
Selected references:
1. ROSE SS. Treatment of chronic oedema of the leg. Proc R Soc Med. 1950 Dec;43(12):1057-8. PMID: 14808199.
2. Ononda et al. International Journal of Surgery Case Reports Volume 19, 2016, Pages 66-68.
