DISEASES OF THE SPLEEN.
BY I. E. ATKINSON, M.D.
Morbid processes affecting the spleen have been and remain involved in great obscurity. Older writers, who were accustomed to reach their conclusions in great measure through the observation of symptoms alone, were obliged in the absence of anything like correct knowledge of anatomy, physiology, and pathology to supply from the imagination most of their theories of disease. Untrammelled by the bonds of accurate investigation and ignorant of pathological anatomy, they found no difficulty in ascribing to various parts and organs peculiar groups of symptoms, both physical and moral; and for a number of these the spleen was held responsible. We now know that many of the symptoms thus supposed to indicate splenic disease depend upon alterations in other parts of the body, and may be observed in persons possessing perfectly healthy spleens. But while we have learned that symptoms formerly supposed to depend upon splenic disorder may, in reality, have nothing to do with this organ, we still remain ignorant of many of the real symptoms of splenic disease, as well as of many of the morbid conditions that induce them. Such knowledge as we have, however, is based upon comparisons of symptomatology with dead-house revelations and the experience of the laboratory, and, while as yet imperfect, cannot fail to increase under modern methods of research.
In order to begin the study of diseases of the spleen in an intelligent manner it is manifestly necessary to have some settled ideas regarding its anatomy and physiology. No apology is needed, therefore, for the brief anatomical and physiological descriptions that follow.
The spleen is the largest of the ductless glands, and is situated in the left hypochondriac region. It is of a dark slate or bluish-gray color, and often of wrinkled appearance. It is of soft, friable structure. It rests between the stomach, diaphragm, and left kidney, and in form resembles a flattened oval. It extends from the level of the eleventh rib, beginning one or two centimeters distant from the vertebral column, downward and forward to a position about four centimeters from the point of the eleventh rib (Lushka). It is separated from the ninth, tenth, and eleventh ribs by the diaphragm. It presents two surfaces—one external and convex, facing the diaphragm; the other internal and concave, applied to the cardiac end of the stomach. The hilum divides the internal portion into two parts by a deep fissure, which marks the line of attachment of the gastro-splenic omentum. The larger and anterior part is bound to the fundus of the stomach by delicate areolar tissue, and the posterior and smaller portion to the left pillar of the diaphragm and the left suprarenal capsule. The upper portion is connected with the diaphragm by peritoneum forming a suspensory ligament. The bottom of the hilum is perforated by a number of openings for the transmission of blood-vessels, nerves, and lymphatics. The anterior border of the organ is notched and thinner than the posterior border. The pointed lower end touches the splenic flexure of the transverse colon and rests upon the costo-colic ligament. The spleen varies in size and weight within wide limits. Its average weight in adults is 250 grams, its length from 11 to 13 centimeters, and its thickness from 4 to 6 centimeters (Orth). Its volume is from 150 to 180 cubic centimeters. According to Gray, the proportionate weight of this organ to that of the whole body varies from 1:320 to 1:400, gradually diminishing until old age, when the proportion becomes as 1:700.
In the vicinity of the spleen are often found a number of small bodies similar to it in structure. These are known as accessory spleens, and are usually situated in the gastro-splenic or in the greater omentum. The attachments of the viscus are not very close, and much variation in size and position is possible.
Except at the hilum the peritoneum forms everywhere one of the coverings of the spleen. Its peculiar sheath or capsule is composed of fibro-elastic tissue of a whitish color, prolongations of which extend into the substance of the organ and form the trabeculæ that constitute its supporting framework and sheaths for blood-vessels and nerves. A close meshwork is thus created in which are contained the splenic vessels and pulp. This fibrous coat and these trabeculæ contain involuntary muscular fibres. These, with the elastic fibres, provide for the changes in size that the organ undergoes. When incised, the normal spleen presents a reddish-brown color, and its substance may be readily broken down with the finger into a pulp. This pulp consists of a mass of branched intercommunicating connective-tissue corpuscles of different sizes, within the substance of which remains of red blood-corpuscles may often be detected. The interstices of these cells are filled with blood. The very large splenic artery enters the spleen by numerous branches, ramifying within the trabecular sheaths and terminating in pencils of minute size.
The external coats of the smaller arteries are converted into lymphoid tissue, which, suddenly expanding here and there, forms the bodies known as the Malpighian follicles, which are supplied with capillary vessels, and which may often be distinguished by the naked eye as points of whitish color, sometimes attaining the size of pinheads. These small arteries end in capillaries, which, according to Müller, gradually lose their cylindrical character and emerge into a system of connective-tissue corpuscles, inosculating with the corpuscles of the splenic pulp in such a manner that the blood passes into the pulp-tissue freely, and is gradually brought to the veins by the transition of this tissue into that of the blood-vascular system. The splenic lymphatics originate in the arterial sheaths and in the trabeculæ. In the former case they accompany the blood-vessels; in the latter, they communicate with a superficial network in the corpuscle. All join at the hilum and enter the neighboring lymphatic glands. The splenic nerves are from the right and left semi-lunar ganglions and right pneumogastric nerve. They accompany the branches of the splenic artery, and have been traced deeply into the tissue of the organ.
It is perfectly established that under normal conditions the volume of the spleen may vary considerably, and especially during the act of digestion, and that this does not occur through simple engorgement of the vessels. The very important experiments of Roy show that, in cats and dogs at least, the splenic circulation does not depend upon the ordinary blood-pressure, but is carried on "chiefly, if not exclusively, by a rhythmic contraction of the muscles contained in the capsule and trabeculæ of the organ."1 This rhythmic contraction and expansion Roy observed to occur with great regularity at the rate of about sixty contractions an hour, with extremes of rapidity of rhythm of forty-six seconds for the most rapid and two minutes three seconds for the slowest. He also observed that stimulation of the central end of a cut sensory nerve, or of the medulla oblongata, or of the peripheral ends of both splanchnics and both vagi, causes a rapid contraction of the spleen. Unsatisfactory as is our knowledge of splenic physiology and of its exact relations to the maintenance of life (for that the spleen is not the seat of a peculiar and exclusive function has been demonstrated by the survival of individuals after extirpation of the organ), at present certain theories of its nature find pretty general acceptance. Thus, it is considered that in the lymphoid tissue of the blood-vessels and Malpighian corpuscles leucocytes are produced—that the cells of the splenic pulp appear to take red blood-corpuscles into their interior, where their disintegration takes place. There are not sufficient grounds for believing that in the spleen red blood-corpuscles are formed. Recent observations of Tizzoni, Crédé, and Zesas have led them to the conclusion that they are made in the spleen; but Bizzozero and others deny that this occurs except after serious hemorrhage.
1 Journal of Physiology, vol. iii., 3 and 4, p. 203.
It is impossible to detect by palpation any part of a healthy spleen. Its area may be approximately defined by percussion alone, though even by this method it is not always easy to determine its position and size. Loomis advises that the patient be placed upon his right side in order to facilitate the examination. The anterior border of the spleen is then "readily determined by the tympanitic resonance of the stomach and intestines. Inferiorly, where the organ comes into contact with the kidney, it is difficult, and often impossible, to determine its boundary. Its superior border corresponds to the line which marks the change from flatness to pulmonary resonance." The vagueness of these directions is necessitated by the difficulties of the subject, the splenic outlines being liable to frequent variations. Schuster and Mosler give excellent reasons for prosecuting the investigation with the patient in the right semi-supine position.
Acute Congestion of the Spleen.
Except within the physiological limits already referred to, acute congestion of the spleen never occurs as a primary process. Under pathological conditions it is known to take place under a great variety of circumstances, principally, however, in connection with those states of the system in which disease is supposed to depend upon some specific principle or germ. To a minor extent it is probable that splenic congestion accompanies nearly all febrile conditions, and from the border-lands of health to that highest and most intense degree of hyperæmia by which the organ acquires a volume and prominence that have caused it to be designated as acute splenic tumor, all gradations may be observed, though in many instances these may be so slight as to be incapable of recognition clinically, and are only brought to our knowledge through necroscopic examination. The congestion becomes most marked in the course of the acute specific fevers. In typhus and typhoid fevers, in small-pox, scarlatina, diphtheria, in epidemic cerebro-spinal meningitis, in acute tuberculosis, in erysipelas, puerperal fever, in conditions of blood-poisoning and in malarial fevers, more especially those of more severe type, it reaches its highest development. According to Friedreich, a form of pneumonia (differing from ordinary croupous pneumonia in its serpiginous course), acute coryza, and acute pharyngitis and tonsillitis are accompanied by enlargement of the spleen in consequence of the septic nature of these disorders. During the fever of secondary syphilis a splenic enlargement purely hyperæmic in character may sometimes be detected. Similar conditions are occasionally observed in a number of other affections. This tendency of the spleen to active congestion is to be accounted for by its peculiar anatomical structure, whereby unusual facilities for hyperæmia are afforded, more especially in the infective fevers, in the course of which the organic germs which are supposed to constitute their essential principles collect in the pulp, and by their accumulation and multiplication serve to excite a more or less intense determination of blood to the part, the organisms themselves being taken up by the leucocytes and connective-tissue corpuscles composing the pulp. We can thus account for the multitudes of these organisms to be found in the splenic pulp after various infective disorders, as in relapsing fever as observed by Ponfick, in pyæmia by Birch-Hirschfeld, and in splenic fever of animals by various observers. The less intense degrees of congestion occurring during the various specific fevers and in many simple febrile disturbances are usually so slight as not to attract attention. When the hyperæmia has been unduly prolonged, as more especially occurs as a result of chronic malarial poisoning, leucocythæmia, pseudo-leucocythæmia, or Hodgkin's disease, there is a well-pronounced tendency toward permanent structural changes and the development of hypertrophy.
SYMPTOMATOLOGY.—Milder degrees of congestion do not, generally, reveal their existence by symptoms, and those of more pronounced character give for the most part signs that are vague and nearly obscured by the more prominent features of the pathological processes that occasion or accompany the splenic changes. It may happen that acute splenic tumor of considerable size may be quite painless. It has been objected, indeed, that when pain accompanies splenic enlargements it is not attributable to any sensibility of the spleen itself, but to the participation of the investing peritoneum in the morbid action or to the dragging of the enlarged organ upon the parts with which it is connected (Mosler). Patients, however, will often complain of a dull, aching pain and a sensation of weight in the left hypochondrium. Occasionally, this pain may be severe and lancinating or may extend to the shoulder. Headache and various digestive disorders—anorexia, vomiting, flatulence, and diarrhoea—may prove distressing accompaniments. Other symptoms, such as melæna, voracious appetite, vertigo, extreme anæmia with its various concomitants, etc., belong rather to conditions of protracted congestion where new formation and true hypertrophy have been developed.
It is evident that it will often be extremely difficult, and sometimes even impossible, to determine the extent to which symptoms are occasioned by the splenic congestion or by the general affection to which it owes its origin. Mosler declares that he is nearly always able to detect during the cold stage of intermittent fever a peculiar murmur over the splenic region and upward and downward in the abdominal region, which he attributes to the contraction of the splenic artery. This murmur he has not been able to perceive in chronic splenic tumors.2
2 Ziemssen's Cyclop., vol. viii. p. 468.
The normal splenic area can only be defined by percussion, and congestion to a not insignificant extent may occur without revealing itself by other symptoms than increase of the extent of percussion dulness. When the organ projects beyond the margin of the ribs and can be felt by the fingers of the examiner, it is enlarged, unless the patient is the subject of displaced or of wandering spleen. But whether the enlargement be due to hyperæmia simply or to hypertrophy can only be determined by a consideration of all the concomitant circumstances. Unless under the influence of chronic irritation or as a result of mechanical hyperæmia, congestions of the spleen are commonly of sudden development and of transitory duration. In ordinary inflammations, such as pleurisy, etc., the degree of congestion is so slight as to be unnoticeable; but as an epiphenomenon of the various specific fevers the enlargement occurs rapidly and acquires a prominent interest in many cases. Acute splenic tumor, for example, is almost of constant occurrence during the course of typhoid fever, and, according to Friedreich, its presence may be ascertained some days before the specific symptoms of the disease have declared themselves. A similar early development has been claimed for it in diphtheria and other affections. The congested spleen of typhoid fever and of relapsing fever, however, differs from that of most other acute disorders in returning to its normal dimensions much more slowly; and it is important to remember that until the splenic tumor has disappeared there is reason to believe the danger of relapse still imminent. In most cases the enlargement disappears pari passu with the disorder that occasioned it. In malarial fevers and in septic diseases the splenic tumor may acquire excessive dimensions. Acute splenic tumor, however, never attains the dimensions often encountered in chronic congestion and hypertrophy.
PATHOLOGY AND PATHOLOGICAL ANATOMY.—Simple splenic congestion presents at first no anatomical features differing from purely physiological hyperæmia. There is simply more blood in the dilated vessels and vascular spaces, and consequently in the viscus, than is usual. Very soon, however, there is hyperplasia of the cells of the pulp. Enlargement, tension of the capsule, and diminished consistency of the spleen appear. The color will depend upon the condition of the capsule, being most dark and blue when this is thinnest. In high grades of congestion the parenchyma upon section will be found distended and semi-diffluent, and after acute malarial fever (pernicious remittent fever), the organ may resemble a bag of half-liquid pulp. Softening in varying degree may be found after acute congestion from whatever cause. In the congestions due to some infective processes at least additional factors are introduced, although as yet definite knowledge of their exact pathogenetic influence has not been attained. The observations connecting minute organisms with the origin of these affections have been so elaborate, so carefully and conscientiously reported, extend over such wide and varied fields, that it is difficult to refuse to place reliance in them. It seems that in a number of affections the presence of these microscopic organisms is constant and essential, and that the splenic congestion that accompanies them is a direct result of their presence in the spleen itself. The micro-organisms will be found infesting the cells of the pulp, and, so far as we have definite knowledge, they show peculiar characteristics according to the particular infectious disease to which the patient succumbed. While the conditions in acute splenic tumor are identical with those of inflammation, and in the affections properly designated as septic, should the life of the patient have been sufficiently prolonged, may be found to have led to the formation of embolic centres with hemorrhagic infarctions and abscess, in infectious diseases not septic they do not prove equal to the production of suppuration. Where the action is acute, resolution will speedily follow the subsidence of the febrile process. But in prolonged hyperæmia new formation will be developed, and the enormous collection of leucocytes will give a reddish-gray color to the organ. This change will also be sometimes observed in the spleens of those in whom the infectious diseases have run a more protracted course.
DIAGNOSIS.—Acute splenic tumor, if at all pronounced, may usually be diagnosticated without much difficulty. The development of an enlargement in the splenic region, with pain and tenderness to pressure, during the course of any acute febrile disease will nearly always indicate splenic hyperæmia. It may sometimes be difficult to determine whether the tumor may not have existed prior to the invasion of the present malady. In such cases one must have recourse to the previous history of the patient, or, failing in this, must observe the behavior of the tumor upon the subsidence of the general affection.
PROGNOSIS.—The prognosis of acute congestion and acute splenic tumor will depend rather upon the exciting cause. When of simple origin it is of but insignificant importance. Even in specific fevers the spleen will in most instances return to its normal volume upon the establishment of convalescence. Rupture of the spleen has been known to occur in congestion from severe malarial fever, but this is a most rare accident in the absence of traumatic influences. The congestion may become chronic, and frequently does become so, in cases where the stimulus continues to exert an influence upon the spleen, as is done in chronic malarial poisoning.
TREATMENT.—The transitory hyperæmia of a brief malarial attack or of any ordinary febrile seizure will disappear with its exciting cause, and will require no special treatment. For the acute congestions of most specific fevers but little is to be done except through attention to the general condition: it is only when pain and discomfort in the splenic region are sufficient to attract the attention of the patient that measures for the relief of the congestion will be necessary. In that most common exciting cause of it, malarial fever, patients will often complain bitterly of the pain in the left hypochondrium for some time after the febrile attack has been overcome. In such cases it may be pretty safely concluded that the poisonous influence of the malaria has not been entirely overcome, and the proper employment of quinine and other derivatives of Peruvian bark, and bitter tonics, will undoubtedly prove most serviceable. In very many cases benefit may be derived from local applications. Experiment has clearly shown that the stimulation of the splenic nerves is capable of effecting a notable reduction in the bulk of the organ. Clinical experience gives similar proofs, and cold effusions, evaporating lotions, etc. will sometimes secure prompt unloading of the spleen; indeed, Mosler considers that there is danger in treating the acute splenic tumor of typhus fever of inducing unfavorable changes by the too sudden reduction of its bulk by local applications. The use of stimulating applications to the splenic region will also prove beneficial in many cases. Among the most valuable of these will be found the tincture of iodine.
Chronic Congestion and Enlargement of the Spleen.
Within narrow limits there may be simple increase in the size of the spleen from hyperæmia, without alteration of the relations between its structural parts. The common results, however, of hyperæmia of long standing are overgrowth of the elements of the reticulum, with new formation of connective tissue and hyperplasia of the pulp-tissue. This condition of chronic enlargement or hypertrophy of the spleen may develop as a result of chronic active hyperæmia or through passive or mechanical engorgement of the portal system. Chronic active hyperæmia of the spleen is in much the greater number of instances caused by chronic malarial poisoning. It also occurs as a cause or a result of leucocythæmia and of pseudo-leucocythæmia or Hodgkin's disease, and is always associated with more or less true hypertrophy of the structural elements of the organ. Enlargement from the above-mentioned causes constitutes the vast majority of those abnormalities generally designated as chronic splenic tumor. In persons living in malarious countries, and subjected for prolonged periods to the intoxicating influence, the peculiar splenic enlargement tends to become chronic. After the earlier attacks the spleen returns more or less promptly to its normal dimensions. Usually it is only after repeated attacks of intermittent or remittent fevers, and often only after exposure to the malarious influence for years, that the splenic tumor becomes established as a permanent disorder and assumes the characteristics that have secured for it the popular denomination ague-cake. Persons living in the localities referred to may develop this enlargement without ever having had unequivocal attacks of malarial fever. They will betray, however, the effects of the poisoning by malarial neuralgias and neuroses or by a well-marked periodicity in the course of simple maladies, or they will exhibit its effects by the peculiar facies and by general paludal cachexia. Under these conditions the splenic enlargement sometimes attains enormous proportions.
Splenic enlargement of considerable extent may result from mechanical hyperæmia of the portal circulation from cirrhosis of the liver. It is, however, certainly not a necessary consequence of cirrhosis, since this may exist to a pronounced degree and yet the spleen remain normal—a condition that is probably favored by extensive distribution of muscular and elastic fibres to the viscus, that enable it to a great extent to regulate its own circulation. On the other hand, the spleen may be atrophied by a fibrotic contraction of its trabeculæ, the result of long-standing hyperplasia. Chronic engorgement and enlargement of the spleen may also result from mechanical obstruction to the systemic venous circulation, especially that due to insufficiency of the mitral valve, whereby obstruction to the portal circulation arises secondarily. (The ulcerative endocarditis of septic origin is associated with splenic congestion, which is, however, always of the acute active variety, and complicated for the most part with embolic abscess and hemorrhagic infarction.)
SYMPTOMATOLOGY.—Long-continued or frequently-recurring attacks of splenic hyperæmia, occurring under the stimulus of chronic malarial poisoning or of leucocythæmia or pseudo-leucocythæmia, will ultimately induce those structural changes that result in new formation. Enlargements from the two latter diseases will be more appropriately considered elsewhere. After repeated attacks of remittent or intermittent fever or other forms of malarial intoxication the symptoms of acute will gradually merge into those of chronic congestion. They will usually prevail to a more intense degree. The dragging weight of the tumor will excite pain, and may render rest upon the right side too uncomfortable to be indulged in. Hemorrhage from the stomach and bowels may occur, and at times will be excessive. The patient may be reduced to an extreme degree by the profuse and repeated losses of blood. In the intervals of the malarial attacks the temperature will be unelevated, and the pulse may be slow and irregular, though oftener feeble and rapid. All the symptoms will be, however, commingled with those from other causes. Those of malarial cachexia will sometimes be very pronounced. The pale, sallow complexion, the pallid lips, the extreme anæmia and generally unhealthy aspect, and the general symptoms accompanying such states, the history of miasmatic fevers, of characteristic neuralgias, etc., will generally be present. Oedema may be observed, but will usually be hydræmic in origin. Anomalous symptoms due to the systemic condition will be often developed when the enlargement arises from other than malarial causes.
Under the influence of the latter cause the spleen may acquire many times its normal dimensions, and may easily be felt below the border of the ribs, where its irregularly curving and notched border will serve to identify it. The tumor sometimes becomes so large that it reveals its presence by causing a bulging and asymmetry appreciable by the patient. Here, however, congestion will have been supplanted by hypertrophy. The tumor may vary greatly in size. It may fill the left part of the abdominal cavity, reaching to the pubes and distending the belly-wall with its dense enlargement, dull upon percussion, and perhaps moving within narrow limits under the hand of the examiner. This tumor may attain a size and weight many times greater than the normal. Hypertrophy once established, it may remain more or less pronounced for years, directly occasioning unimportant symptoms. It is difficult to determine the exact influence exerted by these tumors upon the duration of life.
PATHOLOGICAL ANATOMY.—In simple hypertrophy there is both hyperplasia of the pulp and of the trabecular connective tissue. The spleen is enlarged, sometimes to an extreme degree, equalling fifteen or sixteen times its normal weight.3 Such enlargement is not observed in any other form of splenic disorder, excepting in some rare cases of leucocythæmia and tumor. Its density is also increased. The capsule is thickened, and adhesions to the surrounding parts may be quite intimate. The color of the surface is darker than normal. Upon section the structure appears dense, smooth, of a dark color (from deposit of pigment), and showing to the naked eye great increase of the trabecular tissue. The pigmentation more especially observed in malarial intoxication occurs in the intervascular cords of the pulp (Rindfleisch), where it can be seen as black, flaky masses of hæmatin (the origin of melanæmia). According to Friedreich,4 there may be a circumscribed splenic hypertrophy, consisting of little points of granulation imbedded in the pulp. In ordinary diffuse hypertrophy all the elements are involved, though the trabeculæ show the greatest increase and encroach more or less upon the pulp. The Malpighian corpuscles may show little or no enlargement. The processes are indistinguishable from those of chronic inflammation. In hypertrophy from obstructed portal circulation the organ will be dark red and very full of blood. It sometimes happens that obstructive hypertrophy terminates in fibrotic contraction, when the connective tissue will be found to have almost completely crowded out the pulp.
3 Hertz, Ziemssen's Cyc., vol. ii.
4 Virchow's Archiv, xxiii., 1865; Ziemssen's Cyc., viii., Mosler.
DIAGNOSIS.—Decided enlargement will usually be recognized with but little difficulty. A tumor in the left hypochondrium, occupying and transgressing the normal splenic boundaries, will probably be of splenic origin. Occasionally enlargement may be simulated by a spleen of normal size displaced downward by intra-thoracic growths or effusions or by that remarkable abnormality known as wandering spleen. The course of the primary affection in the one case, and the free movability of the organ in the other, will suffice generally to guard against error. Rarely, the tumor may be due to cancer of the stomach, enlargement of the left kidney or of the left lobe of the liver, omental tumors, fecal accumulations in the colon, or ovarian tumors. The concomitant symptoms will suffice to distinguish cancer of the cardiac end of the stomach. Percussion will reveal the presence of subjacent gases, and palpation will detect the greater hardness of the gastric tumor. Enlargement of the left kidney may be due to cancer, abscess, or other causes, and may simulate splenic hypertrophy. The renal tumor may be traced farther backward, and will not present the characteristic outline of the spleen. The clinical history and symptoms will here, again, prevent error. Omental tumor is usually separated from the splenic region by an area of resonance. Enlarged liver may be traced toward the right side of the body, becoming more noticeable as the spleen is receded from. Fecal accumulation may closely resemble splenic tumor, as it does other abdominal enlargements. The doughy consistency of enlarged spleen may be like that of the fecal mass, but one may often permanently alter the shape of the latter by the pressure of the fingers, and in any case doubt may be dispelled by the use of purgatives. Ovarian tumors may be traced into the pelvis, as may also, for the most part, fibro-cystic and fibroid tumors of the uterus and its appendages.
On the other hand, recognition of splenic tumors may be prevented by gaseous distension of the stomach and bowels, by abdominal dropsy, diffuse or encysted, by fecal distension of the colon, and may, indeed, be impossible until these conditions have been remedied. Enlargement of the spleen from simple hyperplasia must also be distinguished from other forms of splenic enlargement—from splenitis, from lardaceous degeneration, from tumors, from leukæmia and pseudo-leukæmia, from syphilitic and tuberculous spleen, etc. In such cases the diagnosis will rather depend upon concomitant symptoms than upon the physical characters of the enlarged organ. Percussion and palpation will not seldom enable one to determine the presence of tumor (cancer), hydatids, etc. Pressure will often serve to elicit expressions of great tenderness in splenitis; enlargements with fluid contents will be revealed by fluctuation. In the greater number of cases where the enlargement is evident, but is without distinguishing characteristics, the general condition of the patient and the history of his illness will disclose its true nature. Lardaceous degeneration will have been anteceded by prolonged suppuration, by tubercle, by scrofula, or by syphilis, and will generally be associated with the same processes in other parts. Syphilitic disease may be indicated by the history of the patient, though in this case, of course, lardaceous degeneration could only with difficulty be excluded. Tubercle, rarely giving rise to an appreciable tumor, can only be conjecturally diagnosticated from the history and general condition of the patient. The condition of the blood and of the lymphatic system in leukæmia and pseudo-leukæmia will suffice to determine the nature of the splenic enlargement. The ague-cake of chronic malarial poisoning is usually accompanied by a degree of cachexia, as is shown in the earthy pallor of the complexion. This is often sufficient to enable one to discriminate between several forms of enlargement, for it differs from the intense pallor of leukæmia by its sallow hue, and is not at all like the hue of the complexion in lardaceous disease. The cancerous cachexia, it is true, may closely resemble it, but here the history and symptoms assist in avoiding mistakes.
PROGNOSIS.—When the hyperplastic processes have amounted to true connective-tissue formation, a complete return to normal conditions will not occur after the removal of the stimulus. The permanence of the enlargement will be proportionate to the extent of organization of the hyperplastic elements. In ague-cake some reduction in size will follow the exhaustion of the malarial influence, though the spleen probably never ceases to be appreciable as a distinct enlargement. At the same time, the enlarged organ may not, of itself, exert any specially unfavorable effect upon its bearer. Not a few persons will live for years with it, and eventually die from other causes. It may be assumed, however, that the presence of ague-cake is indicative of profound malarial cachexia, by which the powers of life are much less resistant to unfavorable influences. It may be said, in a general way, that the larger the spleen the less favorable is the prognosis. It should be remembered that a considerable proportion of persons suffering from leucocythæmia have also suffered from chronic malarial poisoning, and that the enlarged spleen of this affection may possibly have begun its morbid course under the influence of malaria.
TREATMENT.—In passive congestion relief is often secured through the use of remedies that diminish portal engorgement or enable the heart to find compensation for a damaged mitral valve—conditions in which the splenic disorder is really an unimportant concomitant. In the enlargement that has for its cause chronic malarial intoxication cinchona and its alkaloids are preferable to all other remedies, not only in arresting the new growth otherwise progressive under the stimulus of the poison, but by neutralizing the latter and facilitating the absorption of the hyperplastic elements that have not already become converted into more highly-developed tissue. To effect these objects the agents must be given in fair doses (twenty grains of sulphate of quinia daily) until the malarial cachexia shall have been overcome—until the bulk of the enlarged spleen shall have been reduced to the smallest possible proportions. To bring about the desired result the treatment may have to be continued during several months, occasionally suspended upon the supervention of symptoms of cinchonism. A drug of deserved repute (probably through its anti-malarial influence) is arsenic. This should be given for protracted periods. Many remedies possessing anti-malarial properties have also been recommended and employed in these conditions. Eucalyptus and eucalyptol have recently been used with promising results, though the sanguine expectations of some will hardly be realized. Iron, preferably as a sulphate or as the tincture of the chloride, is invaluable in correcting the profound anæmia always present in these cases, though its influence in reducing the splenic bulk immediately is, at best, doubtful. Remedies competent to reduce hepatic and portal engorgement will often prove beneficial. Salines and vegetable cathartics may more especially be employed, but the use of mercurials, except for occasional administration, is almost universally condemned as productive of evil consequences.
Local Treatment.—The systematic application of cold by effusion or by ice-bags will at times undoubtedly reduce the size of an enlarged spleen. Alleviation will often be afforded by solutions of nitric acid to the splenic region, and counter-irritants are of occasional service, either by means of the tincture of iodine persistently employed or by blistering fluids or plasters. These, however, should be used with great caution in debilitated subjects, as gangrene has been known to follow their application. Mosler thinks that the practice of injecting tincture of iodine, carbolic acid, etc. into the substance of the spleen is sufficiently promising to justify further experiment. The continuous electric current and electrolysis have also been recently recommended as of advantage in reducing the splenic bulk. In cases of excessive enlargement, where accompanying or consequent cachexia threatens to end in death, extirpation of the spleen has been advised and practised. While the removal of the leucocythæmic spleen is so constantly followed by death that the operation cannot be considered justifiable, it seems that the spleen enlarged from other causes may sometimes be removed with safety. In the Lancet of Feb. 11, 1882, Herbert Collier tabulates all (until then) known cases of removal of the spleen for disease, 29 in number: 16 of these operations were upon leukæmic subjects, and had a fatal termination; 8 of the remaining cases recovered. Crédé5 concludes from an analysis of 30 cases of extirpation of splenic tumor that the adult spleen may be removed without detriment; that its removal causes temporary derangement of the blood-making function; and that this is compensated for by activity of the thyroid body and red marrow of the bones. As bearing further upon the question of the practicability of splenectomy, should surgical art succeed in reducing the dangers immediately dependent upon the operation, are the highly interesting experiments of Tizzoni6 and Griffini,7 wherein extirpation of the spleen in dogs was followed by reproduction of true splenic tissue.
5 Centralbl. f. d. med. Wissensch., June 23, 1883.
6 Arch. Ital. de Biologie, 1883, iii. 2, and i. 1.
7 Ibid., 1883, iii. 2.
In chronic congestion and enlargement of the spleen from malarial poisoning the removal of the patient to a non-malarious locality will always materially assist in the recovery of health.
Hemorrhagic Infarction of the Spleen.
The investigations of Virchow, and more recently of Cohnheim, into the pathogenesis and pathology of hemorrhagic infarction have afforded an easily intelligible explanation of the causes of the frequency of this morbid process in the spleen. A moment's reference to the anatomy of the splenic blood-vessels will show that the conditions most favorable to the production of hemorrhagic infarction in the presence of an exciting cause are here afforded. Instead of terminating in a capillary network with free and abundant anastomoses, the splenic arteries end in fine pencils, opening, not into capillaries leading to venous radicles, but into vascular spaces in which traces of both afferent and efferent blood-vessels gradually become effaced. These arterioles have no other vascular communications than with the small arteries, the terminal extremities of which they are. This arrangement may be very perfectly demonstrated in injected spleens where the material has been imperfectly driven through the vascular system of the organ, so that wedge-shaped areas of successful injection become sharply defined. This distribution of the blood-vessels renders the area supplied by each almost completely dependent upon it for efficient nutrition, and almost certain to become structurally altered if its lumen should become in any manner obstructed. To this arrangement of the arteries and arterioles upon one side is added, upon the other, a valveless condition of the splenic veins, whereby regurgitation may readily occur. We have here, therefore, evidently conditions most favorable to the development of hemorrhagic infarction.
The process through which hemorrhagic infarction occurs has been definitely observed by Cohnheim. The area of the distribution of the obstructed artery or arteriole, receiving no blood-supply from anastomosing branches, undergoes disintegration. The walls of the blood-vessels as far as the nearest communicating branch participate in the process of disorganization. After a while a backward movement of the blood-current begins in the nearest still pervious vessels, and is continued into the obstructed vessels, through whose disintegrating walls the blood escapes and the hemorrhagic infarction is established. The future course of the infarct depends almost entirely upon the nature of the causes that brought it about.
In the spleen, as in other organs, the causes of hemorrhagic infarction may be widely different, though an essential condition of each is that it be competent to produce plugging of the blood-vessel. The most important cause is probably ulcerative endocarditis, in the course of which minute fragments of the endocardium or of the vegetations that have formed upon it, especially of the neighborhood of the valves, in consequence of inflammation, constitute the emboli. The plugs largely consist of fibrinous matter enveloping colonies of micrococci, or they may be derived from detached portions of thrombi, or from solid particles that may have in any way gained access to the circulatory current, as from endarteritis, from atheroma, primary emboli in the pulmonary circulation, etc.
Hemorrhagic infarction has also been described by Ponfick8 as occurring in relapsing fever and originating in the veins, and not to be referred to any of the already-mentioned causes. It is thrombotic in origin, and due to some peculiarities of the morbid processes of the affection.
8 Virchow's Archiv, Bd. lx.; Mosler, Ziemssen's Cyclop., viii. p. 443.
SYMPTOMATOLOGY.—Hemorrhagic infarction of the spleen, as such, reveals its presence by no signs during life. Its importance depends almost entirely upon the nature of its exciting cause. When this is of simple origin there is hardly ever any deleterious influence exerted upon the health of the individual. That hemorrhagic infarction was present in any given case can only be ascertained by the evidences of it discoverable after death. Rarely by its presence localized inflammation and abscess may be excited. Far different, however, is the result where the embolic material has been derived from an ulcerative endocarditis or other septic centre. In this event the infarction serves surely as the starting-point for metastatic abscess.
PATHOLOGICAL ANATOMY.—Hemorrhagic infarctions of the spleen may vary in number from one to many, and in size from that of a large shot to a bulk nearly equal to that of the spleen itself. They are usually situated at the surface of the organ in a wedge-shaped distribution, the base looking toward the capsule and causing a slight projection, the apex pointing toward the deeper portions. Infarctions, however, may also occasionally occur in the central parts of the spleen. A definite wedge-shape may be destroyed by the coalescence of several neighboring infarcts.
The appearance and density of the infarction will depend very much upon its age. When recent it is of a dark-red color, of firm consistency, and of homogeneous aspect, and is surrounded by a zone of hyperæmia. As it grows older the dark color gradually fades to a paler hue, in consequence of the absorption of the color-elements of the hemorrhage, and a yellowish shade appears, from fatty degeneration of the cellular constituents. With the fading in color the infarction decreases in size; contractions and scar-formations are developed, later to become converted into bands of dense fibrous tissue. Occasionally complete fatty metamorphosis of the cellular elements may ensue and caseation of the infarct take place. The caseous mass may soften and form a cavity, or may ultimately undergo calcareous degeneration. Not very infrequently one may detect at necroscopic examinations of spleens these calcareous nodules, equal to shot or peas in size, witnesses of the bygone metamorphoses of which we are speaking. When, instead of being of simple origin, the infarct is the result of septic changes, the course is different. Coincident with or immediately preceding the hemorrhagic infarction inflammatory symptoms will develop around the embolic masses (consisting principally of fibrinous material imprisoning multitudes of micrococci), and metastatic abscess rapidly becomes established. Pus will then be commingled with the softening mass, and the microscope will reveal the swarming organisms. In the latter event the changes of the hemorrhagic infarction are much more rapid than in simple infarction, when they may be very protracted.
Splenitis.
Although it is impossible to separate acute splenic tumor and chronic splenic enlargement from the processes of inflammation, for practical purposes it is convenient to consider as splenitis only those morbid conditions in which the tendency is toward suppuration. Simple idiopathic splenitis is undoubtedly very rare, and, although formerly its symptoms were described with great detail, most recent writers are content to acknowledge an almost complete ignorance of them. Indeed, splenic abscess is often detected after death when it had not even been suspected during life.
Diffuse Splenic Abscess.—In the rare cases of idiopathic splenic inflammation the exciting cause will commonly have been a fall or blow or other violence by which the spleen has been injured, or it may have followed chronic malarial poisoning or an extension of inflammation from the capsule or neighboring parts.
SYMPTOMATOLOGY.—The rarity of this affection makes accurate description of its symptoms almost impossible. Mosler has never seen it. The descriptions of it are based, at best, upon observation of but few cases. Its onset may be sudden; more commonly it is insidious, the patient complaining of weight and dull pain in the left hypochondrium, irradiating to the left shoulder. The presence of pain depends upon the participation of the capsule in the process. Cough and dyspnoea may be present, and febrile phenomena are constantly to be observed. Vomiting, want of appetite, furred tongue, etc. will be noticeable. After a time a tumor will be detected that will, at first, almost certainly show no sign of fluctuation. Coincidently with the development of the tumor a small degree of ascites and of anasarca of the lower extremities may appear.
Up to this point the presence of abscess may only be conjectured, and indeed throughout its entire course it usually escapes positive identification. Fluctuation may, however, be detected, and from its location and concomitant symptoms may reasonably be ascribed to a splenic abscess. The fluctuating tumor has been known to fill the whole abdominal cavity from epigastrium to pubes. Grisolle reports such a case where the tumor presented the appearance of ascites. In this form of splenic abscess the progress is generally insidious. Under symptoms of hectic fever, wasting, etc. the physical signs of splenic enlargement are gradually manifested until the presence of fluid may be determined. After months of suffering the patient will expire from exhaustion or from the consequences of rupture of the abscess into the abdominal or pleural cavity or into the lungs, lighting up rapidly-fatal inflammation; or, discharging into the bowel or stomach or through the abdominal wall, the abscess may temporarily improve and allow a short prolongation of a wretched life. In the event, however, of an escape of pus from the body, as through the abdominal wall, bowel, etc., recovery is possible in a very small proportion of cases. Wardell has seen such a case discharging through the abdominal parietes. Zweifel has met a similar condition. Nasse has known of recovery after the pus had been expectorated, and Webb, after discharge into the intestine. Occasionally, splenic abscess may become encapsulated and undergo caseous metamorphosis, when it may become inactive, ultimately cicatrize, or become calcareous.
PATHOLOGICAL ANATOMY.—In abscess of the spleen, when of small size, a non-metastatic origin may be recognized by the absence of micrococci from its purulent contents and of concomitant signs of blood-poisoning. When not spontaneously arrested, these abscesses attain a size not equalled by metastatic splenic abscess. The splenic substance will then be reduced to a semi-fluid or fluid mass of reddish pus enclosed within a pyogenic membrane. In extreme cases all traces of true splenic pulp and trabeculæ will be obliterated; but when the inflammatory action is less intense the trabeculæ will extend in all directions through the abscess cavity. The capsule, thickened and indurated, will have formed adhesions or will have entirely disappeared before the advancing wall of the abscess.
Embolic Abscess.
It has been shown that hemorrhagic infarction in the spleen is the result of an embolic obstruction of the splenic blood-vessels. If the embolus be simply a detached portion of an aseptic clot or fibrinous vegetation or of atheromatous degeneration, the subsequent changes will be those characteristic of the involution of hemorrhagic infarction. Under exceptional circumstances and from not understood causes inflammation and abscess may follow. These, however, are to be reckoned among the rare results of simple hemorrhagic infarction of the spleen. Altogether more frequently the embolus is derived from the ulcerative endocarditis of septic origin or from other septic centre, and consists of congeries of micro-organisms, themselves the infecting agents or the vehicles of the poison that lights up the characteristic morbid processes. A colony of these micro-organisms, lodged in and occluding splenic arteries, by the irritation of their presence and by their multiplication excite the inflammatory processes that accompany and follow the hemorrhagic infarction. Embolic splenic abscess is, then, nearly constantly a secondary result of conditions of blood-poisoning, and as such can only play a subservient part in the train of pathological events in which all parts reached by the blood-supply may be engaged. The vascular distribution in the spleen is such as to afford exceptionally favorable opportunities for the development of metastatic abscesses, and in a large proportion of spleens of those who have died from blood-poisoning they will be detected. They are rarely present without the appearance of similar changes in other organs, and there is, therefore, but little difficulty in attributing them to their true cause.
SYMPTOMATOLOGY.—Unless inflammation of the splenic capsule be excited, these abscesses give rise to no pain, neither do they (except rarely) produce discoverable splenic enlargement as distinct from the general splenic enlargement always present in septic fever. Their course is usually brief, in consequence of the usually acute course of the disease that occasions them. When, in chronic pyæmia, splenic embolic abscess may develop more slowly, exceptionally palpable fluctuating tumor becomes manifest.
Fever, with all the accompanying phenomena of blood-poisoning, is present in these cases, and commonly masks any splenic alteration that might otherwise become apparent. Embolic abscess should always be suspected in blood-poisoning, though in most cases its detection could have but little influence in determining treatment.
PATHOLOGICAL ANATOMY.—Embolic abscess may develop from a hemorrhagic infarction, in which case the necrotic central mass is surrounded by a zone of inflammation which rapidly converts the whole area into a broken-down, reddish, purulent, semi-fluid matter. If the abscess supervene without the occurrence of hemorrhagic infarction, its situation is still nearly always peripheral, the wedge-shaped embolic area pointing toward the centre. It varies in size from that of a pinhead to that of a pea and larger. It consists of a necrotic centre composed of pus-cells and detritus, a surrounding mass of exudation, and a circumscribing border of hyperæmia. Microscopic examination will usually reveal swarms of micrococci. In the progress of the abscess the whole mass becomes converted into a grumous brownish fluid. The peritoneum rarely participates in the activity of the inflammation, but may form deposits of lymph over the seats of the abscesses.
Mosler9 summarizes Ponfick's description of a peculiar splenic inflammatory process resulting from relapsing fever. It differs from ordinary embolic abscess in being limited to the splenic venous system. It may equal two-thirds of the entire spleen in bulk. It resembles in appearance embolic abscess, but the arteries remain pervious. These abscesses may heal or may enlarge and peritonitis may be excited. The possibility of their originating in a venous thrombosis is entertained by Ponfick. A peculiar inflammatory condition of the follicular tissue of the spleen has also been described by Ponfick as a result of relapsing fever.
9 Ziemssen's Cyclop., vol. iii.
DIAGNOSIS.—The diagnosis of splenic abscess presents very often great difficulties, and is frequently quite impossible. In ordinary embolic abscess a diagnosis cannot be made with certainty. The existence of pyæmia with enlargement and pain would make it probable that splenic abscess had formed. In the larger abscesses of malarial, traumatic, or unknown origin the detection of a fluctuating tumor in the region of the spleen will suggest its true cause, but examination of the contents will alone clear up the diagnosis between the real disease and hydatid tumors, nephritic and perinephritic accumulations of fluids, etc. Even where the contents of the cavity are purulent, it will often be impossible to decide upon their splenic origin unless in the event of portions of the splenic tissue escaping at the orifice of the abscess. In cases of constant and increasing pain and tenderness in the splenic region, with enlargement, associated with general failure of health, splenic abscess may be suspected, and an exploratory puncture with the aspirating-needle may not only be justifiable, but imperatively called for. In all cases it must be remembered that splenic abscess of this character is a most rare disease.
PROGNOSIS.—Splenic abscess usually terminates fatally. The life-destroying influence, however, is not exerted through the spleen itself, for this may be converted into a simple bag of semi-fluid contents, with complete destruction of all its tissue, and yet danger is to be apprehended only from the effects of suppuration or of rupture into the closed cavities or from peritonitis, etc. Of itself, embolic abscess rarely excites alarming symptoms, because, being usually of septic origin, the stress of the general condition is thrown more upon the whole body, or upon a number of its parts, of which the spleen is not the most important.
TREATMENT.—Treatment should be directed more toward prophylaxis than toward cure. In those congestions and hyperplasias that may result in abscess the remedies indicated for these conditions should be actively employed. The application of ice to the splenic region, of counter-irritants, the use of local bloodletting, the unloading of the intestinal circulation by saline purgatives, the proper employment of quinine, etc. in chronic malarial poisoning, seasonably adapted, may prevent the formation of abscess. In the event of fluctuation declaring itself, evacuation under antiseptic precautions should be practised; ordinarily, the most effective general treatment is that directed against the primary disease.
Perisplenitis.
Inflammation of the splenic capsule is a more common affection than clinical observation would lead one to suppose. It consists of a more or less localized splenic peritonitis, and its lesions are often found at the necropsy when its existence had not been suspected during life.
ETIOLOGY.—Its commonest cause is the extension of inflammation from neighboring parts. Chronic ulcer of the stomach may be the origin of chronic perisplenitis, leading to the formation of dense inflammatory deposits. Persons who have long suffered from miasmatic poisoning frequently develop strong adhesions between the spleen and diaphragm. And from the same cause the spleen may become closely adherent to the neighboring viscera. Chronic enteritis, perinephritic inflammation, and the like may excite it. It has been shown that the pain in splenic affections is nearly always due to the capsulitis present; and it is probable that much pain in the splenic region, stitches in the side, etc. are really the results of this inflammation. It can only be conjectured that in given cases one has to do with perisplenitis. Almost all that is known about it comes from the dead-house.
PATHOLOGICAL ANATOMY.—The simplest post-mortem signs of bygone perisplenitis are the unusually dense fibrous adhesions between the spleen and surrounding parts. These may vary within wide limits. Exceptionally, the spleen will be found intimately adherent to surrounding parts throughout, and can only be separated from them by tearing it away. Under these circumstances, mostly in chronic malarial subjects, the capsule will be uniformly much thickened and sac-like. The splenic tissue may be reduced to a tarry, semi-fluid pulp that oozes through the lacerated walls. Sometimes the capsule of the spleen will show localized thickenings of dense cartilage-like consistency, usually on the convex surface. According to Wilks and Moxon (p. 487), "section shows them to be laminated parallel to the surface, and the microscope reveals a fibrinous structure, the fibres being arranged in dense areolated lamellæ." The same authors consider these to be among the most decisive evidences of chronic alcoholism. They may become calcified (Orth). It is not unlikely that they may often be the effects of syphilis. They undoubtedly often occur in syphilitic subjects. The interest attaching to them is entirely a pathological one, as the affection is never detected during life, and as they probably exert no influence whatever upon the duration of life or even upon the well-being of their bearer.
Lardaceous Spleen.
The spleen is more liable to lardaceous or amyloid disease than any other organ of the body. And, although in the further course of the degeneration other organs and tissues inevitably become implicated (unless the patient die of some intercurrent affection), the spleen may in the earlier stages be alone involved. In 58 cases of lardaceous disease compiled from the records of the London Hospital, the spleen was the only organ in which the degeneration was detected in 28 cases, while it remained unaffected in only 10 cases.10
10 Turner, Transactions Patholog. Soc. Lond., vol. xxx.
The tendency of lardaceous disease toward generalization shows that it is under systemic and not local influence, though whether this influence is exerted in depositing preformed albuminoid material in the affected parts (infiltration), or in bringing about a special alteration in situ (degeneration), is even yet not definitely decided. Upon the one hand, the infiltration theory is upheld by Rindfleisch, Billroth, and others, while Fehr, Kyber, Cohnheim, and others consider it to be a result of tissue-metamorphosis. Cohnheim concludes that the infiltration theory could only be accepted upon the presumption that the lardaceous material is not a soluble but a corpuscular substance, or that it is only deposited in consequence of some acquired predisposition of the part. He regards the process as a local degeneration due to general causes in which the lardaceous material is derived from the pre-existing albumen of the tissues. According to Virchow and Kyber, there is brought to the tissue whose nutrition is somehow lowered a substance, between which and a malarial substance formed in loco an intimate combination occurs, the result being lardaceous material (Ziegler).
This form of degeneration involves the spleen in one or both of two ways. It may appear as scattered points throughout the splenic substance, corresponding to the Malpighian bodies and presenting a resemblance to grains of boiled sago, or in a diffused manner, constituting true lardaceous spleen, in which the entire organ appears to be involved.
ETIOLOGY.—As in lardaceous disease of other parts, by far the most common causes of its development in the spleen are prolonged suppuration, especially of bone, the suppurative processes of phthisis pulmonalis and of scrofulosis. The next most frequent causative influence is syphilis, whether accompanied by prolonged suppuration or by the cachexia so often observed in this disorder. Chronic malarial poisoning, chronic diarrhoea, chronic alcoholism, and occasionally the less-rapidly fatal malignant new growths, may induce the degeneration. Exceptionally, it has been observed where no other general disturbance of nutrition had existed. The various causes of lardaceous degeneration have in common one feature, chronicity, though Mosler quotes from Cohnheim instances where lardaceous spleen was discovered in one case five months after joint injuries had been received, and in another four months after a compound fracture of the right leg.
SYMPTOMATOLOGY.—Lardaceous disease of the spleen is usually associated with similar disease of other organs—the liver, kidneys, stomach, intestines, heart, etc.—and its symptoms are so frequently accompanied by those of the affection that has given origin to it that it must always be difficult to distinguish them as attributable to the condition of the spleen itself. Profound anæmia with an appearance of cachexia is always present in advanced cases. Milder cases may reveal themselves by no signs. The symptoms arising from other parts implicated in the degeneration may completely mask those depending upon the spleen. When the stomach is involved, vomiting and hæmatemesis even to a fatal termination may occur, or uncontrollable diarrhoea from intestinal changes may supervene. Splenic enlargement is not unusually accompanied by enlargement of the liver. Ascites, however, is always a rare accompaniment.
Rarely, the spleen attains enormous size, and may then occasion sensations of weight and tension, and occasionally acute pain from implication of the capsule in inflammatory action. When the organ can be felt through the abdominal walls it will generally be hardened, painless, and with its boundaries much thicker and rounder than normal.
PATHOLOGICAL ANATOMY.—As has been already remarked, lardaceous disease of the spleen is observed in two forms. In both the spleen is enlarged and hardened. Its structure presents a tough, waxy consistence, and the organ has entirely lost its friability. In sago spleen, light-brown or grayish waxy bodies are scattered throughout the splenic structure. The pulp may remain quite healthy, or it may also be involved. These sago-like bodies correspond to the enlarged and lardaceous Malpighian corpuscles, and stand out with some prominence from the general surface. They may vary in size from that of a pinhead to that of a small pea. The color of the spleen may shade from a pale fawn color to a reddish-brown. In many cases where the parenchyma is involved there will be exhibited scattered areas of semi-transparent, wax-like material.
In the diffusely lardaceous spleen the organ is enlarged throughout, pitting to pressure, and upon section presenting a waxy, semi-translucent appearance, usually of a reddish-gray, but sometimes of a deep-red, color. Instead of a pulpy, easily broken-down condition of the splenic parenchyma, there will be found a dense tissue that can be cut into tough, glistening slices. Minor degrees of the change cannot be readily detected by the unaided eye, and even in advanced cases the judgment will often be at fault. Under these, and in fact under all circumstances a correct conclusion as to the nature of a given change can only be reached after the employment of reagents that exert peculiar influences over the lardaceous material. The action of iodine upon this material is quite characteristic. If a watery solution of iodine with iodide of potassium be applied to the cut surface of the suspected organ, the normal portions will be stained a yellowish color, while those parts that have undergone lardaceous degeneration will assume a rich mahogany red or brown, which will become violet or purple upon addition of sulphuric acid. This latter reaction is not constant, and may usually be omitted. Cornil has recently proposed as a test a solution of methyl-aniline-violet, which possesses the property of staining lardaceous matter red, while ordinary tissues will be stained a deep, bright blue. This reaction possesses the advantage of being permanent and very delicate, and on that account preferable for microscopic examination of specimens. According to Cohnheim, this reagent enables one to distinguish commencing lardaceous change.
In lardaceous disease of the Malpighian corpuscles the alteration will be found to begin in the arterial twig to which the corpuscle is attached, soon extending to the entire tissue of the corpuscle, which it causes to enlarge considerably. When the splenic pulp is attacked it is said to be the vessels of the pulp that are first involved. It is held by most pathologists (Virchow, Kyber, etc.) that the change is chiefly seated in the muscular coat of the small arteries, but that the intima is also very frequently affected, and that occasionally all the coats are involved. Thence the degeneration spreads to the cells and nuclei of the splenic tissue. Later investigations, however, seem to make it probable that the lardaceous degeneration is mostly limited to the connective-tissue trabeculæ and walls of the venous sinuses; that the pulp-cells are for the most part not implicated, but that they disappear in consequence of the pressure of the ever-increasing lardaceous material and the consequent anæmia (Cohnheim, Ziegler).
DIAGNOSIS.—This will depend more upon the history and concomitant symptoms and general condition of the patient than upon any positive evidence to be gained by special reference to the spleen. In a patient predisposed to lardaceous degeneration by any of the influences enumerated above the presumption in favor of splenic lardaceous disease is strong if, in addition to splenic enlargement, there is evidence of hypertrophy of the liver and albuminuria, indications of the participation of other organs in the process, and an anæmic and cachectic appearance of the individual always observed in advanced degrees of the degeneration.
PROGNOSIS.—The prognosis is almost always unfavorable, not so much on account of the splenic condition as from the general depreciation of the powers of life. The disorder being progressive, the tendency is toward death by complications resulting from degenerations of other organs. And yet it seems quite probable that mild grades of lardaceous degeneration may be entirely recovered from occasionally; but this will be almost invariably in cases where the spleen alone is implicated. At all events, when not advanced it may be long held in abeyance. The duration of the disease generally is indefinite and may cover a space of years.
TREATMENT.—The treatment of lardaceous degeneration of the spleen will consist rather in combating its exciting causes than in efforts directed toward the condition of the spleen itself. It may, however, be possible to effect some good by resorting to remedies supposed to be useful in subduing ordinary splenic enlargement.
Echinococcus of the Spleen.
Echinococci invade the human body in the United States far less frequently than in many other countries, where the canine race occupies much closer relations with man (as in Iceland). The echinococci are the larval forms of Tænia echinococcus, a tape-worm of minute size inhabiting the intestinal tract of the genus Canis, more especially that of the dog. The ova of the tæniæ are voided in countless numbers in the feces of their hosts. Still unhatched or in an embryonic form, they are thence conveyed through the medium of water or otherwise to the stomach of man, whence the embryos (scolices) escape into the tissues and develop into ordinary hydatid cysts. Rare as is this affection in the human body, it is relatively extremely uncommon as implicating the spleen, and recorded instances of its occurrence are not numerous. Hydatids of the spleen may coexist with those of other parts, and in occasional instances are said to be secondary to these. They are commonly encountered about the middle period of life, and appear to affect the sexes in equal proportions.
In cases of multiple hydatid cysts in different parts of the body it has been asserted, upon the one hand, that a single older cyst serves as the parent cyst, germs from which become transplanted in other localities through the blood. This view receives some support from the fact that one cyst, usually seated in the liver, is commonly much larger than the others. An objection to its universal acceptance, however, as pointed out by Budd, is that it is very difficult to imagine that a germ from a larger cyst can travel through the portal vein, against the current, toward the spleen, mesentery, etc., to form a secondary cyst. On the other hand, it seems likely that an individual exposed to infection by the echinococcus would be liable to ingest many scolices at one time or on repeated occasions, and that the differences in development depend upon varying degrees of assimilative power on the part of the parasite and of the conditions of its environment.
SYMPTOMATOLOGY.—Whether echinococcus of the spleen will betray symptoms of its presence depends upon varied circumstances. Small cysts, certainly, may occasion no signs, subjective or objective. Cysts may even attain very large dimensions without exciting discomfort to their bearer, and may consequently escape detection. Pain may precede the appearance of a tumor, but will be irregular and paroxysmal, increasing in severity with the growth of the cyst. The most constant annoyance, however, is that occasioned by the size and weight of the enlargement. The patient may detect its presence accidentally, or his attention may first be directed to it by his medical attendant. He may give a history of its growth during a number of years without its having occasioned more than passing uneasiness.
The tumor may exceptionally attain a large size, nearly filling the left side of the abdominal cavity. It may encroach upon the area of the thoracic cavity. Upon examination, the tumor, when of sufficient size, will be rounded, not resembling the appearance of a simply enlarged spleen. Fluctuation will be detected, and occasionally the peculiar hydatid thrill, upon the diagnostic importance of which great stress has been placed. This, however, is a very inconstant sign, and in the majority of cases is not to be discovered. Frerichs only found it where the sac was not tense and contained other vesicles. A peritoneal friction sound may sometimes be detected by the ear placed over the region of the tumor. These cysts differ from other fluctuating tumors in being of very slow growth, remaining almost without change for years, and in exciting no constitutional reaction, unless, as is quite possible, inflammation of the sac is developed, when rigors, hectic, and other symptoms indicative of suppurative inflammation will be observed. Pressure of the tumor upon the stomach may excite anorexia, vomiting, epigastric uneasiness, and gastric catarrh. If the pressure is exerted upon the portal vein or vena cava, dropsy may result; if upon the bowel, constipation may be produced.
It is possible for the development of the cyst to be arrested through the death of the echinococcus. This may occur if it is of small size. Its walls may then become calcareous, and the mass will cease to exert any injurious influence upon the host. In other cases, as a result of inflammation, rupture will take place, and the contents of the cyst, with the characteristic formations, will escape into the peritoneal, pericardial, or pleural cavities, or into the alimentary tract, the urinary passages, or even into the vena cava; or they may be discharged through the body-wall. In any of these events a fatal termination is almost inevitable. Rupture may also occur in an unaltered cyst from any sudden or excessive violence. Death will usually speedily ensue from collapse or as a result of inflammation of the peritoneum. Finally, complete recovery will sometimes be secured through treatment.
DIAGNOSIS.—Echinococcus of the spleen presents no characteristic symptoms. When the tumor is small and escapes observation, or when the fluid nature of its contents cannot be recognized, its existence cannot be determined. In larger tumors the hydatid thrill will, when present, assist the observer, and the presence of fluctuation will of course serve to exclude all solid enlargements of the spleen from consideration. Abscess will differ in its shorter course, its rapid increase in size, and its inflammatory symptoms, the general condition contrasting with the excellent condition of health usually observed in simple hydatid tumor. The diagnosis will become greatly obscured in the event of inflammation of the cyst. Certainty can only be attained through an exploratory puncture and examination of the contents of the cysts. These will consist of a clear, non-albuminous fluid, rich in sodium chloride, and revealing the echinococcus scolices and hooks and membranous shreds when examined under the microscope. Doubt may arise where inflammatory changes have made the fluid albuminous and where the scolices and hooklets have been destroyed or do not accompany the escaped fluid.
MORBID ANATOMY.—The spleen may be almost destroyed by the hydatid cysts, which, usually single, may exist in large numbers. According to Wardell,11 "they are seldom found in the pulp, usually in the gastro-splenic epiploon or in the cysts constituted of the serous investment." The cysts consist of a thick fibrous investment and an inner parenchymatous layer, from which the little heads develop in tiny vesicles. Compound systems, one enclosed within the other, are thus formed, varying from the size of a pea to that of a marble, and even very much larger. The cysts may undergo atheromatous or calcareous degeneration. In these cases the echinococci are destroyed, and the mass becomes encapsulated in a calcareous envelope and remains quiescent. The microscope will reveal the remains of the echinococci, even after long periods. Where rupture has taken place the rent in the cyst will have allowed characteristic matters to escape into the communicating parts, where they may be detected.
11 Reynolds's System of Medicine, vol. v.
The PROGNOSIS of echinococcus of the spleen is always serious, usually most unfavorable. The best results are observed in those cases where, the cyst being small, spontaneous arrest of development has occurred. Puncture of the cyst and partial evacuation of its contents, when practicable, increase what would otherwise be almost hopeless chances of ultimate recovery in cysts of moderate and large size.
TREATMENT.—The only treatment that promises good results is the evacuation of the cyst fluid. Murchison recommends the removal of the fluid with a very small trocar, whereby the admission of air into the cavity is avoided. The withdrawal of the fluid is sufficient to destroy the life of the parasite, and in favorable cases to secure the degenerative changes of which mention has been made. The adoption of antiseptic precautions will undoubtedly increase the chances of recovery. Unfortunately, a certain number of cases will run into suppuration, when all the dangers of suppurating cavities have to be encountered, and must be treated in the usual way. Various injections into the cyst-cavity have been recommended, but they do not seem to afford better results than simple evacuation. These will probably most successfully be employed in cases where the cyst has formed inflammatory adhesions to the skin, which may be effected through the external application of caustic agents capable of exciting inflammatory changes in the deeper parts (Vienna paste, etc.). Injections may be then made through incisions carried into the cyst, without danger of exposing the peritoneal cavity. Internal medication, except for general purposes, has no efficacy in the treatment of these tumors.
Syphilis of the Spleen.
The spleen is not very frequently affected by syphilis. Nevertheless, this viscus may become the seat of syphilitic disorder during either its early or late phases. It has even been asserted by Weil that the spleen may become enlarged in the interval between the appearance of the primary sore and the first cutaneous eruption. Whatever changes the spleen may undergo during the course of early syphilis are essentially of the simple congestive type, and are comparable to the acute splenic enlargements of the ordinary specific fevers; certainly, no essentially syphilitic changes can be detected at this stage. In fact, throughout the whole secondary period the splenic derangement is of the nature of simple hyperplasia. In the later stages of syphilis there is a more permanent enlargement of the spleen, due to a chronic interstitial inflammation that should be distinguished from that very much more common result of old syphilis, lardaceous degeneration. The histological characteristics of these enlargements are not known to differ essentially from the simple chronic enlargements of the spleen already considered.
It is only toward the end of the secondary period, and during tertiary syphilis and in inherited syphilis, that products essentially syphilitic can be recognized. Gummy infiltrations and tumors of the spleen have been observed by a few writers—not, however, clinically, but for the most part in the dead-house. These tumors are found scattered throughout the substance of the organ, but most commonly near its surface. They vary in number within not very wide limits, and in size from that of a pinhead to that of a pea or larger. They may be sharply circumscribed (but not encapsulated) or more diffused. The portions of the spleen affected become changed by the syphilitic material into grayish-red, homogeneous masses in recent cases. At a later stage they are "gray or grayish-yellow, homogeneous, somewhat dry, tough, almost cheesy."12 The spleen under these circumstances is, as a whole, somewhat enlarged.13 Gummy tumors of the spleen may be confounded with tubercle and old hemorrhagic infarction.
12 Wagner, Mosler, Ziemssen's Cyclop., vol. viii. p. 485, Am. ed.
13 Gold, Viertelj. f. Derm. und Syph., 1880, p. 463.
There is a form of circumscribed enlargement from new growth that is sometimes observed in the spleens of syphilitics, and which is probably of syphilitic origin, producing changes similar to certain forms already described as a variety of perisplenitis. It is situated at the surface of the spleen, and consists of hard whitish or pale-yellow plates but slightly elevated above the normal level, but of considerable superficial extent. When incised, these plates remind one of cartilage.
Splenic enlargements are common in the subjects of inherited syphilis. According to Cornil, infants syphilitic by inheritance have very frequently enlarged spleens, the capsule being inflamed and thickened and the splenic tissue abnormally hard. The organ may thus become sufficiently enlarged to be detected by palpation. Sée considers that enlargement of the spleen is present in one-fourth of all cases of inherited syphilis, and Haslund reports splenic enlargement in 58 of 154 necropsies of such infants.
The clinical signs of syphilitic spleen are almost beyond recognition, if indeed they can be said to exist. Circumstances of growth, etc. may excite the suspicion that a given splenic tumor may be syphilitic. Jullien, it is true, describes symptoms of splenic syphilis, but his views do not seem to be well founded.
TREATMENT.—In recent enlargements therapeutics may effect much in reducing the tumor, and the facility with which its reduction is effected will afford a valuable indication of the success of treatment. Gummy tumors are probably within the reach of antisyphilitic treatment, and it is not unlikely that some of the shrunken, indurated areas often detected post-mortem, and usually ascribed to infarctions, are in reality due to the cicatricial remnants of old gummata. Chronic diffuse splenic enlargements of syphilitic origin are but little influenced by treatment.
Rupture of the Spleen.
The peculiar texture of the spleen renders it especially liable to rupture—more so than any of the other abdominal viscera. By far the most common cause of splenic rupture is external violence from blows, kicks, falls, squeezing force, and wounds incised or punctured. It may be the direct result of the injury, or the rent may be made by the penetration of broken ribs or of foreign bodies. The rupture may even occur spontaneously from causes located within the organ itself. It has been previously observed that in the enlargement accompanying the acute infectious fevers, malarial fever, etc., while the distension of the capsule renders the spleen tense and elastic, section through its substance will often reveal a semi-diffluent condition, the exact nature of which is not well understood, but which undoubtedly originates in excessive vascularity. This occurs especially in malarial fever and typhus. Rupture may here take place spontaneously, or, as is commonly the case, a very slight degree of violence is sufficient to produce it: a wrench, the effort to preserve a disturbed equilibrium, an otherwise insignificant blow, may determine the lesion. Pregnancy and the puerperal state may be the predisposing causes to the accident, and vomiting has been known to produce it. It has also been known to follow the softening and breaking down of a hemorrhagic infarction or the rupture of varices and aneurism. The normal spleen is only with the greatest rarity subjected to a degree of violence sufficient to rupture it, while in countries where enlargement of the spleen is of common occurrence, as from malaria, the accident occurs more frequently.
SYMPTOMATOLOGY AND COURSE.—When the rupture is of traumatic origin there may be no marks of external violence: the symptoms usually are those that follow sudden and great losses of blood, faintness, pallor following intense pain in the splenic region, frequency and weakness of pulse, sighing, coldness of the extremities, and the rapidly developing signs of profound prostration. A fatal termination usually quickly follows the rupture. Where the hemorrhage is not immediately great the patient may not succumb at once, but may live for hours, even days—nay, may even recover, as has occurred in the experience of some observers. Wilks and Moxon saw a case of splenic laceration where five ounces of laminated clot in process of absorption were found lying upon the spleen, death having occurred eighteen days after the accident in consequence of abscess of the brain. In cases where rupture has taken place, perhaps from very slight violence, in a spleen enlarged and softened from disease, the above-mentioned symptoms may have been preceded by pain and a sense of weariness in the splenic region, and by distinguishable enlargement of the organ.
PATHOLOGICAL ANATOMY.—Except in injuries caused by the penetration of foreign bodies or fractured ribs the rupture will usually be linear, and either straight, curved, angular, or stellate. If the rupture have occurred spontaneously it will probably be single, but in the event of its following violence it will most often have resulted at several places. In cases of traumatic splenic rupture in persons suffering from chronic malarial poisoning, Konaraloff14 invariably found the rents in the lower portion of the organ, the greater ones on the outer surface, the smaller ones mostly on the inner surface near the hilum. They were widely gaping and deep. In ruptures consequent upon disease alone or slight violence to a diseased organ the spleen will usually be found enlarged, sometimes to three or four times its normal volume, with its substance softened and of a cherry-red color. In such cases signs of bruising or injury to the integument will not usually be discoverable. Splenic hemorrhage has been known to occur from the rupture of varices and aneurism, in which case characteristic appearances have been found after death. After death from rupture of the spleen the abdominal cavity will be more or less filled with blood, dark and coagulated. Though the contrary has been held, it is doubtful if multiple rupture of the spleen can be regarded as certainly indicative of a traumatic origin.
14 Lond. Med. Rec., No. 97, 1883, p. 259.
Tubercle of the Spleen.
Tubercle not unfrequently attacks the spleen, but only as secondary to general tuberculosis. Wilks and Moxon indeed think the larger nodules of tubercle may be primary, but there seems to be no evidence in support of this opinion. As a symptom of general tuberculosis, splenic enlargement from congestion, simply and quite without any specific deposit, is observed as a form of acute splenic tumor. It is at the later stages of general tuberculosis that distinct deposits of tubercle are formed in the spleen, and these are consequently almost always crude. They are generally scattered throughout the pulp, and, according to Billroth, they but rarely affect the Malpighian corpuscles. They are of very small size, and may be present in great numbers; their color is grayish and they are translucent, and only the largest show the yellow tinge of commencing fatty degeneration. According to Orth, they are not always easily distinguishable from the Malpighian bodies. Occasionally, and especially in scrofulous children, larger nodules are formed by the confluence of several tubercles that may equal a pea in size and present numerous yellow points of caseation.
It is usually impossible to diagnosticate the existence of splenic tubercle during life. After death the general splenic tissue will be darkened from hyperæmia and the tubercles surrounded by a vascular halo. When incised the tubercles will stand out from the tissue in which they are imbedded, unlike the Malpighian bodies, and when exposed to a stream of water the latter will disappear, while the tubercles will remain unaffected.
Tumors of the Spleen.
The spleen is very rarely invaded by new growths other than those already mentioned, and then almost exclusively either from direct extension from other parts or by metastasis. In pseudo-leukæmia or Hodgkin's disease the spleen is usually enlarged by a hyperplastic process quite like that of leukæmia. In that variety of this disease that has been called lympho-sarcoma, in which the spleen is invaded subsequently to the implication of the lymphatic glands, especially those of the cervical region, the Malpighian follicles may become enlarged, and even attain the size of walnuts. They contain spindle-cells and connective tissue. The trabeculæ likewise participate in the enlargement. Apart from the hyperplastic follicles thus occurring and also seen in leukæmia, small-pox, scarlatina, etc., lymphoma has been observed by Virchow, Weichselbaum, and others. The tumors consist of bright grayish-red or reddish, not sharply defined, nodules projecting from the dark-red mass of the spleen. Primary sarcoma is said to have been observed in the spleen, but malignant tumors of this organ are usually secondary growths, and even thus occurring are exceedingly rare. They are soft and very rapidly-growing sarcomata and carcinomata. As a rule, they depend upon malignant disease of the liver or abdominal glands through metastasis or by extension of growth. They sometimes grow with almost incredible rapidity. The symptoms are very obscure, and the presence of the malignant infiltration cannot be detected unless hard nodulated masses are formed, which become perceptible through the abdominal wall, as in hepatic cancer. The prognosis is always bad, and depends generally upon the existence of splenic cancer only in so far as this indicates the dissemination of the primary affection and becomes the forerunner of the cancerous cachexia. Fibroma and angioma have also been encountered in the spleen: they are both exceedingly rare. The latter has been known as a pulsating tumor (Langhans).