Chapter I

, into whose etiology different factors probably enter.

[Illustration: FIG. 185

Elephantiasis of leg, scrotum, and penis.]

[Illustration: FIG. 186

Elephantiasis of hand, acquired.]

Of the acquired forms of elephantiasis, those seen in the tropics are nearly all expressions of filariasis. Sporadic instances are met with in colder climates, and a condition resembling it is occasionally observed for which no existing cause can be detected. Such a case is illustrated in Fig. 186, which occurred in a convict in the penitentiary in Buffalo, who had never been outside the limits of the county, and in whom no parasites could be detected. Figs. 185, 187, and 188 illustrate typical instances of elephantiasis, Fig. 188 being taken from a Klamath Indian woman in the Northwest Territory, the condition being similar to that met with in the tropics.

The worms belong to the nematoids, the adult being 0.03 or thereabouts in length, thinner than the diameter of a red corpuscle, rarely remaining long in the quiescent state. They can thus pass into the capillaries, which they may plug. The mosquito is discovered to be the medium of transportation, either directly or indirectly, through exposed drinking water, where the insect deposits her infected eggs. The adult worms outside the body may attain a length of 1 Cm. From the intestinal canal they pass into the lymph current and are carried until their progress is checked, where they establish a permanent home and breed and act as local irritants. The embryos which they produce are innocent; it is the adult and parent organisms that produce the damage. Lymph flow being thus obstructed the area previously drained by a given vessel will undergo various changes in the direction already described. In proportion, then, to the number of adult worms, and in accordance with their location, will be involvement of an entire member or of a more limited area, _e. g._, lymph scrotum (Fig. 187) or chylous hydrocele.

[Illustration: FIG. 187

Elephantiasis of scrotum.]

[Illustration: FIG. 188

Elephantiasis of vulva (Klamath Indian woman).

(Contributed by Dr. H. L. Raymond, U. S. A.)]

As yet there is no cure for filariasis; hence there is no relief for elephantiasis produced by it, except, when localized, to remove the part. In the tropical forms it is the lower part of the body which is usually involved. It begins in a limb, usually in the toes. It produces discomfort rather than actual pain, at least until such time as distention of the parts becomes unbearable. Along with lymphatic engorgement there is a peculiar liability to erysipelas, which becomes an exceedingly serious malady in tissues so saturated with lymph, and with such possibility for the propagation of germs. A milder degree of cutaneous and subcutaneous infection than is implied by the term erysipelas, as used in this work, may be called erysipeloid or cellulitis; it is quite common and frequently recurs. With each attack of this kind the condition is aggravated and the limits of the lesion extend. After a time the member becomes enlarged to a degree which disables, while the skin itself undergoes changes that alter its appearance; not only is it thickened, but there develop upon it papillomatous projections, with infiltration of the corium, that give it an unnatural appearance and feeling. Epithelial proliferation is also rapid, and is accompanied by a sort of caseous discharge which may decompose and add extremely offensive features to these cases.

The management of these cases becomes very difficult. Total disability finally succeeds inability, and patients in the last stages are often bedridden. The repeated attacks of erysipeloid should be treated with antiseptic applications and elevation of the part, but without too much compression, as germs may be forced into the circulation.

In elephantiasis of the lower extremities it has been suggested to tie the femoral arteries, hoping thereby to deprive the limb of at least a portion of its fluid supply. This may be of some avail early, but when it is done late it is likely to be followed by gangrene of the limb, from whose consequences not even amputation can save the patient.

In the tropics, especially where the external genitals are sometimes involved, extensive operations have been of great service, and among the surgeons of India reports of operations of this kind are frequent.

[Illustration: FIG. 189

Elephantiasis (“Barbadoes leg”). (E. J. Meyer.)]

Elephantiasis is most common in men; occurring in women it is not limited to the external genitals, for the writer has seen illustrations of the disease in the legs alone. In the Western Hemisphere it is met frequently in the Barbadoes, and is called _Barbadoes leg_ (Fig. 189). The principal dangers from operations on these cases pertain to the risks of hemorrhage, shock, and infection. Nothing short of amputation of limbs or ablation of the genitals is of real benefit. In all these operations the veins as well as the arteries should be ligated, and the ligatures used _en masse_, introduced with a needle. There is usually copious oozing, and drainage should be provided.

CHYLOCELE.

This term is applied to a condition also referred to as _chylous hydrocele_. It implies a collection of milky fluid in the cavity of the tunica vaginalis. Occurring in a patient known to be suffering from filariasis it may be diagnosticated before exploration. In some instances where the sac of fluid is less translucent than usual, if the candle test fail when applied, chylocele may be suspected. Careful examination of the sac may show widely opened lymph vessels or lymph spaces. It is to be distinguished from spermatocele, whose contents also are milky fluid, but rarely collecting to the same amount. Chylocele may be treated by tapping, or by open division or extirpation of the sac, exactly as recommended elsewhere for the treatment of hydrocele. (See Fig. 187.)

Chylocele is to be distinguished from _lymph scrotum_, which is a form of localized lymphangitis of mild degree rather than a circumscribed collection of chylous fluid. It presents febrile, not to say inflammatory features, and in the chronic form the skin will be frequently seen to ooze fluid closely resembling lymph, which condition is called lymphorrhagia. The scrotum rarely becomes as large as in extreme cases of dropsy, and yet may assume an uncomfortable size. This condition, like that previously mentioned, is usually associated with filariæ. It may appear, however, spontaneously, and after persisting for a long time disappear, with as little apparent reason as that which produced it. When the condition becomes unbearable ablation may be practised. (See Fig. 187.)

CHYLURIA.

The presence of chyle in the urine gives it an appearance as if emulsified oil had been mixed with it. It occurs with or without known reason. Sometimes it co-exists with lesions like lymph scrotum, etc.; at other times it seems to neither produce nor be accompanied by other disturbances. Ordinarily the urine or the blood when examined _at night_--_i. e._, the sleeping hours-will reveal the pathogenic organisms, _i. e._, filariæ. It is a condition but little influenced by treatment, which should be symptomatic in the absence of special indications.

MACROMELIA.

The more typical congenital forms of occlusion of lymph vessels produce such changes as we see, for instance, in macrochilia, where the lips and cheeks are affected; macroglossia, where the tongue is too large to be retained inside the mouth; and sometimes macrodactylia and macropodia, where the fingers and hands or toes and feet are involved (Figs. 190 and 191). It is difficult to separate some of these cases from gigantism, as already stated. The more distinctive lymphatic lesions are frequently accompanied by pigmentary, cutaneous, or papillomatous conditions, which stamp them as something more than mere expressions of disproportionate growth. The patient of Dr. Gerrish, whose condition is illustrated in Fig. 190, presented lesions which might be assigned to either of these groups. It will usually require a careful study to make a proper assignment of such cases as macromelia.

[Illustration: FIG. 190

Macromelia. (Gerrish.)]

[Illustration: FIG. 191

Macroglossia. (Neisser.)]

MACROCHEILIA.

While this condition is usually regarded as an expression of lymphangiectasis, it has been shown that it may be due to multiple adenopathy of the mucous glands in the lips. The lips are well supplied with such glands, which lie beneath mucous membrane in a mixture of more or less connective and vascular tissue. When the lips undergo marked hypertrophy in adult life, it is very likely that the affection may be explained by the hypertrophy of these collective glands, and this is particularly true when anything like nodular arrangement can be detected. A recognition of this cause will indicate the proper remedy, _i. e._, excision of the affected tissue. The writer has on more than one occasion made an elliptical incision both from the lower and upper lip and accomplished its purpose, with great improvement of appearance.

LYMPHANGIOMA.

Lymphangioma has been described in the chapter on Tumors. It seems necessary to allude to but one expression of this kind in this place, _i. e._, the so-called _lymphangioma circumscriptum_. This presents as a cutaneous area dotted with vesicles, sometimes regularly, sometimes irregularly distributed, usually in annular form, seen most commonly on the upper limbs and in the region of the shoulders. The vesicles occasionally become sufficiently large to be called bullæ, while the contained bloodvessels are dilated and discolor the area involved, which may also be more or less pigmented. Here, as in elephantiasis, there is great liability to surface infection of low grade, which may perhaps be called erysipeloid. The tissues gradually become thickened and covered with scabs or warty collections of epithelium. It is met with early in life, rather than late, and is supposed to be of congenital origin. It may be distinguished from herpes by the pronounced vascular changes and by the discharge of lymph.

=Treatment.=--Treatment has been too often unsatisfactory and the trouble often re-appears after apparent recovery. If the area involved be not too large complete excision will probably prove the most satisfactory method of attack.

LYMPHANGITIS.

This term applies rather to gross and visible lesions of the larger lymphatics than to the involvement of the ultimate lymph-filled ramifications. When the smaller lymph capillaries and interspaces are involved the lesion takes the type of an erysipelas or cellulitis, but as the collected products return through the lymph channels from such an involved area they will disturb and infect the lymph vessels themselves, and this leads to what is called a lymphangitis. Formerly the term spontaneous or idiopathic was given to some of these cases. Assuming, as is done throughout this work, that there is no true inflammation that is not of microbic origin, we may expunge the term “idiopathic” and say that lymphangitis is also an expression of infection, and that the inflamed vessel represents a channel through which products of inflammation are being conveyed. Histologically the walls of these vessels become infiltrated with a coagulating exudate, which may completely occlude the vessel. The bloodvessels immediately adjoining the lymphatics also become involved, and, being engorged, give rise to the peculiar red lines or streaks which are frequently seen when cutaneous lymphatics are thus involved, this appearance being due to a _perilymphangitis_. The infected lymph passing through this channel is filtered out in the first lymph nodes with which it communicates, which themselves become thus infected; hence the rapidity with which these enlarge and break down, so that by their own sacrifice they may perhaps protect the rest of the body from serious infection. Under these circumstances suppuration and necrosis of these lymph nodes is to be regarded as a vicarious destruction on their own part.

Lymphangitis proceeds from the periphery toward the centre, and is followed by a certain amount of pain, with great soreness and sense of stiffness in the parts; the skin overlying the infected vessels becomes reddened in streaks, which indicate their course, or becomes more or less infiltrated and involved throughout in a form of infectious dermatitis. According to the virulence of the germ, and the susceptibility of the individual and his tissues, there will or will not occur suppuration. This may perhaps be averted by prompt treatment. Should deep tenderness and pain take the place of or be added to their more superficial expressions it may be inferred that the superficial lymphatics have now infected the deeper ones, and that there is greater danger of phlebitis and a generalized septic infection.

Constitutionally, at least, the expressions are those of septic intoxication, often of true septicemia or septic infection. Local appearances, increasing temperature, or accession of chills may indicate the presence of pus.

In proportion to the distance of the diseased part from the body centres the prognosis becomes more favorable. When an entire limb is involved the matter is very serious; when in the face or abdomen, still more so, the fear being of septic phlebitis and a fatal termination by a more or less typical pyemic process.

=Treatment.=--All exciting causes, including sloughing tissues, foreign bodies, pus, etc., should be thoroughly removed. Pus, when present, should be evacuated, and when its presence is suspected suitable exploration should be made. Tension should always be relieved by incision. In cases where breaking down has already begun, continuous immersion in hot water is beneficial. Nothing, however, will take the place of removal of pus or necrotic tissue, and this should be first attended to or proved to be unnecessary. In an open and sloughing wound nothing is as satisfactory as brewers’ yeast; next to this is hot water. Over an unbroken area which is simply edematous and pits on pressure, may be applied the ichthyol-mercurial ointment (10 per cent. ichthyol, 40 per cent. mercurial ointment) or the Credé silver ointment. This should not be rubbed in, but smeared freely over the surface, and then covered with oiled silk, twice daily, in acute cases. The surgeon should satisfy himself as to the presence or absence of pus; even when only suspected it is advisable to make incision early, as tissue and possibly life may thus be saved. Constitutional treatment should not be neglected. It will consist in improving elimination, maintaining nutrition, and overcoming the acute toxemia due to absorption, the toxins being best antidoted by alcohol in some palatable form, strychnine and quinine being serviceable, but not so valuable. (See chapter on Septic Infections.)

=Chronic Lymphangitis.=--Chronic lymphangitis is seen in connection with the slower infections, tuberculosis--syphilis, filariasis, etc. Here the lymph vessels are not involved so much as the lymph nodes. Chronic lymphangitis does not occur without a toxic or infectious process behind it.

LYMPH NODES.

For the surgeon’s purpose, at least, he may assume that lymph nodes are never enlarged except in the presence of toxemic or infectious processes. The role which they play as filters of the fluid returning through the lymph vessels subjects them to daily possibilities of contamination. They may be acutely infected and actually break down by a phlegmonous process, or their lesions may be very slow, chronic, and intractable. The lymph nodes, like the leukocytes, are among our best friends; they serve as guardians at the various portals of the system, excluding, sometimes at the risk of their own existence, various deleterious elements.

The term “lymph gland” should be expunged from medical terminology, the node having, so far as known, no secretion nor any title to be considered a gland. This would mean abandonment also of the expression “lymphadenitis,” and so the writer would prefer to use the expressions lymphitis, lymphangitis, etc., which at least do not imply a wrong conception of the process. The morbid activity which the lymph nodes present will be an expression of the general virulence of the whole process which has produced it. To a tender enlargement, in acute cases, there will succeed rapid swelling, with pain and soreness commensurate with the density of the surrounding tissues and the degree of tension thus produced. The result is essentially an abscess, or multiple abscess, which necessitates prompt treatment by free incision, evacuation, and drainage, as does any other abscess. It is as often necessary to use a curette as a knife, and when so-called specific features are present, as in chancroidal bubo, a strong antiseptic should also be used. Under these conditions the collection of lymph nodes in the axilla or in the groin may become involved in multiple abscess, and it is then good practice to make a complete cleaning out of these regions. The ultimate effect of such extirpation is beneficial, and the patient does not seem to suffer from the loss of the involved lymph nodes; indeed, it is probable that new ones form to replace those which are destroyed.

The _chronic affections of the lymphatics_ which come under the surgeon’s care are expressions of tuberculosis, syphilis, gonorrhea, cancer, or of some of the other less frequent surgical diseases. In every one of these instances the disease has assumed constitutional proportions, and the lymph-node involvement will be general. The ultimate fate of these affected nodes will differ with the different diseases; in _tuberculosis_ they sometimes suppurate by secondary infection, and they frequently caseate, or remain enlarged for indefinite periods, often throughout life. Around them will be found an area of infiltration which produces firm adhesions and frequently makes their extirpation very difficult. The lymph vessels which connect the various nodes will also be involved in a similar process, which adds to the difficulty of operation. In many cases these involved nodes can be felt where they cannot be attacked--for example, in tabes mesenterica. If, under suitable climatic and constitutional conditions, it be possible to favorably affect other tuberculous conditions, these expressions of the disease may also subside or at least cease to trouble.[29]

[29] At date of going to press I do not feel justified in lauding too highly the work done by numerous workers with the _opsonins_. Justice to what has been done with and claimed for them demands, however, their extensive trial, and suspension of any judgment not as yet favorable.

_Syphilis of the lymph nodes_ has already been considered, as well as the frequency, nay, the certainty, with which the lymphatics become involved in this disease. So true is this that any general lymphatic involvement which cannot be accounted for in some other manner is usually attributable to syphilis. The condition of the lymphatics may be considered a fair index as to the success and effect of antisyphilitic treatment, for if, under such treatment, these enlargements subside completely it may be regarded as eminently successful. On the other hand, it is not felt by many that it is safe to discontinue treatment in the presence of these enlargements. Syphilitic enlargements may, moreover, undergo secondary infection, either acute or chronic, _i. e._, may suppurate or become tuberculous. In _gonorrheal bubo_ the pus which the lymph-node abscesses contain will often be found almost a pure culture of the gonococcus, thus illustrating the specificity of this kind of infection.

The extent to which the lymphatics are involved in cases of cancer will often be the guide for the surgeon in advising removal or the reverse. The principal advance in the modern operative surgery of cancer has come through a better working knowledge of the area of lymph distribution of given regions. All cancerous lymph nodes which can be reached should be extirpated. If others can be discovered which are beyond reach it raises a doubt whether the operation should be performed. At all events, in these cases it should be represented as a temporary rather than an absolutely curative resort, not only because this is true, but because the surgeon may need to protect himself against charges which may be made later by disappointed patients.

The advisability of removing diseased lymph nodes is often a matter for serious discussion. There is little to justify their removal when the exciting cause cannot also be taken with them. It is a mistake to operate on nodes in the neck and leave diseased teeth through which the infection may be spread. So, too, it is a mistake to operate on nodes which may prove to be syphilitic. In many instances, then, it is best to apply the therapeutic test. In cancerous disease it can rarely be advisable to remove lymph nodes alone except for purely temporary purposes, as to check hemorrhage, remove breaking-down material, or something of the kind. In the neck, groin, or axilla the operation is not to be lightly undertaken, for it is made extremely difficult by adhesion of the surrounding structures. The surgeon should be prepared then for careful dissection, which should be made with a not too sharp knife, and he should be ready to sew up a rent in the jugular vein or tie it, as it and its large branches are frequently so displaced and obscured as to be injured, even by the most careful operator.

HODGKIN’S DISEASE.

This is one of many names applied to a condition whose most conspicuous characteristics are a progressive anemia, with enlargement of lymph nodes, as well as usually of the spleen, with secondary or metastatic growths in the viscera, bone-marrow, and elsewhere. That its etiology hitherto has been considered obscure and that its clinical characteristics vary in different cases may be shown by a partial list of the names by which it has been previously known: _lymphadenoma_, _malignant lymphoma_, _infective lymphoma_, _progressive glandular hypertrophy_, _lymphosarcoma_, and _pseudoleukocythemia_. To the writer’s mind, if the disease is to be known by any other name rather than that of the one who first described it, it might be called malignant lymphomatosis, as its tendency is downward, in which sense it is malignant in an almost hopeless degree.

The changes which occur in the blood are at first in the direction of simple anemia, followed by marked reduction in the number of red cells, with poverty of hemoglobin and increase in the number of leukocytes. In the anemia of extreme cases the red cells may be reduced 1,000,000 per Cm., while the leukocytes, especially the polynuclear forms, may be numbered by the hundreds of thousands. In one case recently under my observation the leukocytes amounted to about 300,000 when treatment was begun. (See chapter on the Blood.) It is a disease of early rather than of later life, and occurs more often in males than in females. The most pronounced objective changes occur in the lymph nodes, which enlarge steadily, the swellings thus formed being hard or soft according to the rapidity of the disease. The swellings thus formed will appear conspicuously in the neck and will be noted also in the axilla and the groin. Careful examination will show that every lymph node in the body which is accessible is involved in the course of the disease. Sometimes the tumors become so large as to cause serious pressure, and when in the neck perhaps to require tracheotomy to prevent suffocation. Fig. 192 illustrates a case under the writer’s observation, in which he had to resort to this emergency measure. The microscopic picture of this enlargement is that of hyperplasia of the tissues composing the lymph nodes, while the lymphoid cells are multiplied in number.[30]

[30] Pathologists have long suspected that Hodgkin’s disease and sarcoma have, at least, certain features in common if they are not more or less actually associated in character. Yamasaki has recently reported several cases of typical Hodgkin’s disease without any suspicion of tuberculosis, in which there were unmistakable sarcomatous formations in various parts of the body, especially in the viscera, and he believes, as do others, that the affection which begins as Hodgkin’s disease may later assume the characteristics of a general sarcomatosis.

[Illustration: FIG. 192

Hodgkin’s disease.]

Less conspicuous but equally distinctive changes occur in the spleen in four-fifths of the cases, it becoming enormously enlarged and occupying the left half of the abdominal cavity, being universally enlarged and preserving its original outlines. This splenic enlargement sometimes is simply an hypertrophy, but in many instances the spleen itself will be occupied by tumors, _i. e._, lymphomas, which are scattered through it and cause part of its enormous dimensions. Late in the disease the liver also becomes enlarged and lymphomas are also scattered throughout its substance. The same lymphomatous or adenoid tissue may be met with in many other parts of the body, the bone-marrow, the alimentary canal, the ductless glands, kidney, lung, etc.

Hodgkin’s disease is doubtless closely related to other varieties of leukemia and to Banti’s disease, or splenic anemia, all of which should be regarded as expressions of an infection by organisms not yet clearly described, although their better recognition and identification are clearly foreshadowed in work now under way. Death comes as the result of the exhaustion and poisoning of a terminal infection, save when it is produced earlier by absolute starvation or suffocation. To run its entire course an average case consumes from eighteen months to two and a half years.

=Diagnosis.=--So far as diagnosis is concerned the microscope will serve a certain purpose even early in the disease, enabling one to recognize an increasing anemia and leukocytosis, but not until perceptible enlargement of lymph nodes and of the spleen is found can the diagnosis be made absolutely certain. One has to distinguish mainly between those forms of leukemia in which lymphomatous changes are not conspicuous, cachexia of cancer and syphilis, and the condition of lymphosarcoma, as it has been called by some, in which there is the involvement of the lymph nodes without the characteristic blood changes met with in Hodgkin’s disease. In splenomegaly we may have enormous enlargement of the spleen without the marked involvement of the lymph nodes. From lymphatic tuberculosis it is to be distinguished by absence of fever, the tendency to universal involvement of the lymphoid tissues in all parts of the body, and the absence of suppuration and caseation which occur so distinctively in tuberculous disease.

=Treatment.=--Few drugs are of much or any avail in this disease. Of them all nothing compares with arsenic, which should be given for a long period and pushed to the physiological limit. The formula which was given in the chapter on Cancer will serve in the treatment of Hodgkin’s disease (p. 296, note). Next to this, and especially in patients with enlargement of the spleen, the _x_-rays are the most effective. In one case much of this character, in which I began active treatment by both methods, I saw in forty-eight hours a diminution of 100,000 leukocytes. This did not persist, however, for the proportion was later somewhat increased, but the immediate effect as well as the benefit were very pronounced. All the affected regions may be exposed to the x-rays, which should be used with great care.

TUMORS OF THE LYMPHATICS.

The term _lymphoma_ has been indiscriminately ascribed to various enlargements of the lymph nodes and lymphoid tissue throughout the body, so much so as to have really lost its significance. If by the term is meant simply a tumor of a lymph node it will usually fall under the proper classification as being a granuloma, a syphiloma, a carcinoma, etc. If by the term is meant a general involvement of lymph tissue throughout the body, such as is seen in status lymphaticus, then it would be best to use some other term. Finally it may be questioned whether there is any distinctly marked lymphoma, _i. e._, a tumor of true lymphatic structure, which is not of infectious origin. The term _lymphosarcoma_ is still in use and probably will not be expunged until our notions of pathology are clearer. The expression _lymphadenoma_ should be discarded. Multiple malignant lymphoma, as stated above, is but another name for the condition ordinarily described as Hodgkin’s disease. That sarcoma and endothelioma may arise in the lymph nodes is universally conceded, although as primary neoplasms in these localities they are rare. Not much can be said, then, that is distinctive about lymphoma.

In a general way, it may be said of any tumors of the lymph nodes that if isolated or not too multiple they should be extirpated.

##