[Reprinted from The Tri-State Medical Journal and Practitioner, St. Louis, May, 1897,] ELEPHANTIASIS. By A. H. Ohmann-Dumesnil, of St. Louis> ELEPHANTIASIS, Elephantiasis Arabum, or Barbadoes leg (so named on account of its frequent occurrence in the natives of those islands) is a disease of not so infrequent occurrence as some suppose. It is certainly most often observed in the tropics, but in temperate zones it is observed with sufficient frequency to render some familiarity with its appearance of more than ordinary value. This is the more important from the fact that the trouble is not limited to dark-skinned races, in which it is most frequently observed, but also occurs in Caucasians. Nearly all the cases I have had occasion to observe occurred in Caucasians. The parts whose integument is most frequently involved in the process are the leg (either one or both), the arm, the scrotum, the scrotum and penis, the labia majora and the labia minora, and the clitoris. In both the male and the female the leg is most frequently affected, and, as a result of the affec- tion of the leg, the foot participates in the trouble to quite a marked de- gree. The scrotum is next in frequency, and after this the scrotum and penis. I have seen the latter alone affected, but it is comparatively rare. On the other hand, elephantiasis of the clitoris occurs more often, and that of the labia is seen from time to time. So far as my experience goes, the affection localized to the arm is not very frequent, and in such cases the hand is invariably involved as well. Some few cases of elephantiasis of the nose, of the lip, of the ear, and of other parts, have been recorded, but they were evidently written by authors who were misled in their diagnosis by the peculiar overgrowth which had taken place, and which was rather of the nature of a hypertrophy of the underlying tissues, and not of the skin itself. Cases of acne rosacea frequently attain such enormous propor- tions as to be easily mistaken for elephantiasis, as witness the one reported by me in a former number of this Journal. The predominant characteristic of elephantiasis is a marked increase in size, accompanied by a loss of the normal contour of the part affected. Thus, a leg will show a cylindrical form as large at the ankle as at the thigh, in an old and well-developed case. In one not far developed, it will be as large just about the ankle as in the case shown in Figure 1. In an arm so affected, the forearm will be cylindrical and the arm the same, there being but slight diminution at the flexure of the elbow-joint; and in both, such an enormous size is attained that the joints become immobil- ized and the patient cannot use those limbs. The weight which the affected parts attain is something enormous. The leg will become so heavy that the patient can drag it about with difficulty only, or be entirely incapable of even doing this. When the scrotum is affected it may reach to the ground, and has been known to weigh eighty or more pounds, necessitating its being carried about in a stout sack suspended from the shoulders; and the same thing has been observed in connection with the labia. Another peculiarity is that when the scrotum alone is affected, the penis is completely hidden in the mass; and when the labia are attacked by elephantiasis, the genital cleft is no longer visible, and the urethra 2 must be looked for under the enormously enlarged mass. In the case of the disease affecting the hands or feet, the nails of the fingers and of the toes become completely hidden by the mass of hypertrophied tissue around the nail-folds. The characteristics given above are sufficient to lead to a sus- picion of the real trouble, but there are others which will make assurance doubly sure, if they be observed. When elephantiasis has attained its full proportions, or even some time before this occurs, the skin is rough, papillomatous, forming in some cases enormous folds, and is more or less pigmented. The openings of the sebaceous ducts gape wide open, permitting of a rather free escape of sebum. This latter frequently accumulates in thick, elevated crusts, sep- arated from one another as in ichthyosis sebacea, with this exception, that Fig. i. Elephantiasis of the Foot in a Man (Caucasian). fungous-like masses also show themselves, as shown in Figure 1. This accumulation of sebum does not always occur, there being in many in- stances a tense, glossy appearance of the integument, in others a rather rugous surface, the opening of the duct of each sebaceous gland being not only patulous, but surrounded by a slight elevation. In all cases the lanugo hairs disappear. There appears to be quite an amount of injury to sensation, which in some cases is totally lost. The motor power of a limb which is affected is also considerably impaired; so that, altogether, it may be stated in general terms that a sensible loss of sensory-motor power takes place in elephantiasis. The first evidence of this trouble which manifests itself is in the form of an attack of erysipelas or of dermatitis. When such a condition occurs 3 in certain individuals, the lymphatic vessels or spaces also become in- volved, and eventually a lymph stasis has declared itself. It is not usual for a single attack of erysipelas or dermatitis to bring about such a change, but it is rather the rule for a number of such to recur; and it is a well-known fact that individuals suffering from a number of such attacks are very prone to suffer from new ones. I have known a gentleman who had his right arm attacked by elephantiasis, and he told me that he had always been subject to recurring intervals of erysipelas, at more or less prolonged periods. He never looked upon the matter in a serious light, but he finally succumbed to an attack of erysipelas which involved the same arm and, by continuation of the process, a portion of his back. This debilitated him to such a degree that he finally succumbed. He might have continued for years in comparatively good health, as the elephantiasic growth in his arm, whilst progressive, was very slow. In the case shown in Figure 1, it had existed for some years. The patient could give no definite history, his observation having been that it had taken years to attain its present dimensions, and that he always had, and at the time of observation still suffered, great pain in the foot and leg. He merely thought that he had had some eruptions, but no sat- isfactory anamnesis could be made out. He left the hospital before any decided measures could be taken for his improvement. In the case shown in Figure 2, we have that of a woman, and it is one of more than passing interest. The disease as shown was in a woman who had borne children, and past middle age. According to her account, the trouble had gradually developed, and the size of the leg had pro- gressively increased. The first sign of the trouble was observed after an attack of phlegmasia alba dolens, which is not an uncommon sequela of pregnancy, and one which was formerly dreaded by physicians. In this case, instead of resolution setting in after the birth of the child (and, it may be stated, no active measures were taken in this respect, ending in a normal condition), a blocking up of the lymph-channels occurred. In con- sequence of this obstruction, a thickening and a hypertrophy of the skin occurred, thus constituting a true elephantiasis. At the time the patient was observed, locomotion could be performed, but with extreme difficulty, the pain in the leg was intense, an,d the strength was markedly impaired. A comparison of the affected foot and leg with the sound one, as shown in Figure 2, will readily demonstrate the extent to which the disease had progressed. The left foot is well arched, the toes well defined, and the ankle slender. The entire leg was well shaped and of a much better form than is usually found in women of that age. The right foot and leg pre- sented misshapen, enlarged outlines, and apparent lengthening of the whole extremity not actually present. A curious fact in connection with this case was that the ducts of the sebaceous glands were not yet enlarged. The skin was markedly thickened, although no roughness or rugosities were apparent. Only one fold, just above the ankle, attested to the effects of attempted locomotion, and the first impression produced would be that the case was one of a fracture. The indications at this time were that there would most probably be a continuance of the process and extension upwards, unless active measures were taken to arrest it. Like all or nearly all patients in public hospitals, she objected quite strenuously to 4 any method proposed, and accordingly left the hospital, and has been lost sight of since that time. As may be surmised from what has been said, the trouble consists essentially in an hypertrophy of the skin, more especially affecting the deeper layers. The corium suffers a marked thickening, the stratum mncosnm sharing in this, and the outermost, or stratum corneum, being also affected, somewhat in the same manner. Large lymphatic spaces and hypertrophy of the lymphatic vessels, as well as an increase in their num- ber, may be observed; all of which would tend to support the theory that the disease depends essentially upon an obstruction of lymphatic vessels. Any one who has carefully read Busey's interesting resume of articles em- Fig. 2. Elephantiasis of Right Leg in a Woman (Caucasian). braced in his work on "Obstruction of the Lymph-Channels," will be immediately struck by the truth of this explanation, not only for the origin of elephantiasis, bat also for that of many other conditions which, at first glance, have seemingly no connection with one another. The condition now being considered has no connection whatever with lymph varices, lymphangioma, or with a carious condition of dilatation of lymph spaces which I have had occasion to observe in a single instance. The question in connection with the pathogeny of elephantiasis is in regard to the ultimate caase of the peculiar obstraction of the lymph-channels. We are told by competent authority that the filaria sangziinis hominis is the true caase bringing on that condition known as filariasis, which is equally 5 held responsible for hemato-chyluria, a tropical disease of which I find no record of having been seen in this country, and of which I had occasion to note a marked case some years ago. Whatever the cause may be, there is no question whatever that, after one attack of an obstruction of the lymph-channels, the integument of the involved portion is slightly thickened and remains so. Successive attacks occur, and they invariably involve the locality where the first one appeared and also a portion of the adjacent parts. The thickening of the skin increases until the part which is affected grows to immense, even gigantic, proportions. The density of the skin keeps pace with the in- crease in size of the part which is subject to the process. I have seen and, in fact, possess pieces of skin more than one and a half inches in thickness, and these wrere derived from a case which was by no means un- usual for its size. Many interesting cases of this disease have been reported, a certain number being spurious. There are other hypertrophic processes which have been described as elephantiasis, when they really consisted of a simple connective tissue proliferation, accompanied in some instances by an exuberant infiltration of small round cells. The characteristic appear- ances of the trouble, from a clinical point of view, must never be lost sight of in formulating a diagnosis. For this reason it is not well to rush to radical measures of treatment which might possibly be unnecessary. This brings us to the important subject of the treatment of the disease, which is far from being easy. It has been advocated by some authors that the use of internal remedies alone is capable of bringing about marked changes for the better, but no authenticated cases of a radical cure have yet been published, so that such suggestions may be held as belonging to that vast category of methods, the results of which have been declared not proven. Iodide of potassium and other alteratives have enjoyed a great reputation in the improvement of these cases, but competent observers have become very much discouraged at the impotence manifested by these drugs, and have completely abandoned such measures. This conclusion has become so firmly rooted in the minds of those who are competent to pass judgment on the disease and who are acquainted with its pathology, that they no longer even counsel or essay the use of drugs for the cure of elephantiasis. The only eligible methods are really those which are surgical in character. All medicinal measures having failed, an attempt was made by some to envelope an affected limb in a solid elastic rubber bandage; but this failed most signally on account of the excruciating pain produced, which would lead patients to tear the bandage off in their agony; so that a measure which, at first glance, might be deemed efficient and painless, in reality proved itself the most agonizing of any attempted. Among the more rational procedures which have been adopted with some show of success, is the ligature of the femoral artery or of the brachial artery high up in cases of elephantiasis of the leg and of the arm, respectively. The ligature of the femoral must be made in Scarpa's riangtle, so as to cut off as many large branches as possible. So far as the brachial is concerned, it should be tied as high up as possible, it being necessary at times to ligate the axillary. There are quite a number of cases reported as having been successfully treated in this manner. No 6 definite result is to be anticipated from this procedure, as most serious consequences may result, such as the effects which are apt to set in from an inadequate supply of blood. Gangrene is often the termination of this procedure, and it is very rapid, involving the entire loss of the implicated limb. This is not the only trouble which attends the procedure. Septi- cemia of a destructive character is apt to set in and, as a result, death takes place. So that, on the whole, ligation of the principal artery cannot be recommended in cases of elephantiasis. A better method might, per- haps, be the ligation of the principal lymphatic vessels; but, of course, this is entirely out of the question. Even if such could be effected it might not prove efficient, on account of the large collateral circulation and the obstruction of the lymph-channels would be still greater, if anything. A surgical method much superior to the one just mentioned, is that of amputation. The results attained by this procedure are much superior to those obtained by ligation. Prompt and good union of the tissues follows, and, beyond the deformity produced, the patient is comfortable, with little or no probability of the disease occurring anywhere else or affecting any other part. When it is the scrotum which is affected, a different mode of operation is practiced. It may be remarked that scrotal elephantiasis exists alone, no other portion being implicated. A method of decortica- tion and plastic surgery is to be followed, whereby the testicles are not only not injured, but are enclosed in a comparatively thin pouch, and the penis permitted to protrude its whole length. In those cases in which the penis is implicated, decortication maybe practiced in some, but in the ma- jority of instances very little can be done. If the weight of the organ becomes too enormous, the only recourse to be had is to amputate, either partially or completely. In the case of the labia minora, the best method is, undoubtedly, complete extirpation, the clitoris being preferably in- cluded in the operation. And the reverse holds true. If the clitoris be the part affected, amputation is best, including the labia minora in the operation. In cases of elephantiasis of a labium majus, or of both labia, thorough extirpation is the only operation which will be followed by success. One method, which is probably destined to become the one finally adopted when it is better known, is electrolysis. Excellent results have been obtained by this means, but so far the length of time required has been an inordinate one, and very few patients are willing to wait for such a long time, even to obtain a perfect result. The best result obtained by this method has been published by Silva Araujo, of Rio Janeiro. The method of procedure is to thrust the needle deep down into the tissues at frequent intervals. The strength of the current should be carefully gauged to avoid destruction of tissues instead of the changes which are brought about by the electrolytic current. In addition to this, if the current be too strong, irritation is apt to be produced, and this increases the very hypertrophic process which it is sought to alleviate or cure. Elephantiasis is a very interesting condition, its histopathology being especially of more than ordinary interest. Its study has been pursued by many, and is still being prosecuted in order to arrive at a better under- standing of the underlying conditions, and determine in this manner better and more adequate means of procuring good results without any attendant mutilation.