Part 180

The most usual situations for the occurrence of those changes that lead to occlusion by inflammatory adhesions are the beginning of the cystic duct, obstruction of which is of little moment, and the end of the common duct, which finally proves fatal.

The passage of a large polyangular calculus may cause such irritation, abrasion of the epithelium, and subsequent inflammatory exudation as to effect a direct union of the opposing sides of the canal. This takes place at the beginning of the cystic duct especially, since, owing to the spasm of the gall-bladder and the absence of muscular fibres in the walls of the duct, the stone crushes into, without passing through, the canal. The inflammatory exudation thus excited may close the duct. Not unfrequently the gall-bladder, full of calculi, is thus shut off from the liver permanently. In one instance the writer has seen a calculus wedged into the orifice of the cystic duct, whilst just beyond the lumen was permanently obstructed by an organized exudation. Permanent closure of the cystic duct is of far less consequence than of the common duct, and may, indeed, be a conservative condition, as in the case above mentioned, where numerous polyangular calculi may have migrated, except the closure of the passage.

The most usual point of obstruction in the course of the common duct is the intestinal end, but various processes are employed to effect it. The first in importance is catarrhal inflammation. This seems the more credible when it is remembered that to a simple catarrh of the mucous membrane is due the temporary stoppage of the duct, producing jaundice in much the largest proportion of cases. When the epithelium is detached and granulations spring up from the basement membrane, adhesions of the surfaces will readily take place, and the union may be so complete as that all traces of the duct will disappear. It is probable that in many, if not in most, of these cases the initial condition of the canal is that of simple catarrh, the more positive changes in the mucous membrane arising from peculiarities in the tissues of the individual affected, or from local injury caused by the passage of a concretion or irritation of pathological secretions of the duodenum.

Stenosis, and finally occlusion, of the common duct may arise from the cicatrization of an ulcer. Such ulcers may occur in several modes. They may result from catarrhal inflammation of a chronic type, much new connective-tissue material forming, and in the process of cicatrization, with the contraction belonging to it, the lumen of the canal is so far filled up that the passage of bile is effectually prevented. They may be produced in that state of the tissues which accompanies certain cachectic and profoundly adynamic conditions, as in severe typhoid fever. Such ulcers may also be due to the mechanical injury effected by the migration of a gall-stone. In cicatrizing, a tight stricture, impermeable to the passage of bile, may result, or the lumen of the canal be entirely obliterated. In the latter case the duct itself may disappear and leave no trace. An ulcer situated at the duodenal end of the common duct and extending into the {1084} duodenum may also in the process of healing so contract as to render the orifice impermeable to bile. The same effect may follow the cicatrization of an ulcer of the duodenum in the immediate vicinity of the orifice of the common duct.

Without the intervention of an ulcer as a means of explaining closure of the common duct, this accident may be caused by a catarrhal inflammation which effects denudation of the basement membrane, and thence union may be produced by the mere contact of the freshly-granulating surfaces. Congenital occlusion of the bile-ducts or obstruction occurring in a few days after birth, it is probable, is effected in this way, but no direct evidence of the process has thus far been offered. During intra-uterine life, as at any period in after-life, it seems necessary to the production of such changes that a peculiar constitutional state must exist; otherwise, such a result might happen to every case of catarrhal inflammation of the bile-ducts. The extent of the changes is further evidence in the same direction; for not only are the walls of the duct in permanent apposition and adhesion, but the duct degenerates into a mere fibrous cord, and in some instances is nearly, even entirely, obliterated.

The cystic or common duct--the latter to be chiefly considered--may be occluded by the retention in its lumen of some foreign body. The impaction of a biliary calculus has already been repeatedly referred to, but there are some additional points demanding consideration. The larger concretions may be stopped in the neck of the gall-bladder; those small enough to enter the canal may be arrested at its bend behind the neck, and the very entrance of the cystic duct may be blocked, as in a case examined by the writer.

The hepatic duct is very rarely permanently occluded. As the calibre of this canal continuously enlarges downward, there is no point at which a stone is likely to be arrested; nevertheless, it occasionally happens that such an obstruction does occur. An example has occurred under the observation of the writer, but the cause was a gunshot wound of the liver.

The most usual, and for very obvious reasons the most important, of the sites where occlusion occurs is the common duct and at the termination of the duct in the small intestine, the intestinal orifice. Just behind and to the right of its orifice the duct is dilated into a fossa--the diverticulum Vateri; and here concretions of a size to pass along the common duct are stopped. It is not essential that the stone fit the canal: it may do so and prevent any bile passing into the duodenum; it may be a polyangular body, and, though wedged in, leave spaces through which more or less can slowly trickle. The symptoms will be modified accordingly. Again, the diverticulum may contain numerous concretions, which distend the canal greatly, but through the interstices of which some bile can flow.

Other foreign bodies very rarely close the intestinal end of the ductus communis; thus, for example, a cherry-seed, a plum-seed, a mass of raisin-seeds, may slip into the orifice after the passage of a gall-stone has stretched it sufficiently. A much more common cause of occlusion is an intestinal parasite, which crawls in and is fastened. The common round-worm is the most frequent offender, and much less often liver-flukes find a lodgment there.

{1085} The ductus communis choledochus may be closed by agencies acting from without. They are various, but the most common are the carcinomata. Primary cancer of the gall-bladder and gall-ducts, although not of frequent occurrence, is by no means rare. It develops in connection with the connective-tissue new formations produced by the inflammation following the migration of large calculi. A very instructive example has been examined by the writer. The patient, a woman aged forty-eight, had had numerous paroxysms of hepatic colic, and after death, which followed a protracted stage of jaundice by obstruction, a large ovoid calculus, filling the gall-bladder, was found, and an extensive organized exudation of inflammatory origin was the seat of carcinomatous disease involving the cystic and common ducts and closing the lumen of both. Cancer of the pylorus, of the duodenum, of the pancreas, of the right kidney, and of the liver itself, not unfrequently by exterior pressure permanently occlude the common duct. To this category of obstructing causes must be added enlarged lymphatic glands of the transverse fissure, large fecal accumulations, tumors of the ovaries and uterus, aneurisms of the abdominal aorta, and especially aneurism of the hepatic artery, several examples of which have been reported, and one has occurred in a case seen by the writer.

The effects of obstruction are much less important when the cystic duct is closed. The contents of the gall-bladder accumulate, constituting the condition known as dropsy of the gall-bladder. A catarrhal state of the mucous membrane is set up; the muco-pus formed mixes with the bile, and the mixture undergoes fermentative changes which further alter its character and impart to it irritating qualities, in consequence of which the mucous membrane becomes more decidedly inflamed, and a still more purulent fluid forms, so that ultimately the contents of the gall-bladder are entirely purulent, and that organ may attain to enormous size. Instead of a catarrhal inflammation leading to suppuration, the mucous membrane may pour out serum abundantly, the biliary contents and mucus disappear by absorption, and finally the gall-bladder will be moderately distended by a serous-like fluid. No further disturbance ensues, and the gall-bladder, thus shut off from participation in the hepatic functions, ceases to give trouble.

The results are far different when the obstruction occurs in the hepatic or common duct, for then the bile can no longer perform its double function of secretion and excretion--of contributing materials necessary to digestion and assimilation, and excreting substances whose removal is necessary to health. The liver continuing to functionate after closure of the duct is effected, obviously the secretion of bile continues to accumulate, and the irritation of the mucous membrane causes a catarrhal state; mucus is poured out, and serum escapes from the distended vessels. If the hepatic duct only is obstructed, the dilatation will not involve the cystic duct and gall-bladder, but as the common duct at its termination is occluded, usually the whole system of tubes will be affected by the ensuing changes. The alterations already described as occurring in the gall-bladder take place in all the hepatic ducts. The bile-elements are absorbed, and the fluid distending the whole system of hepatic tubes becomes finally a semi-transparent serum or a very thin sero-mucus, having in bulk a pale sea-green color. Although an intense jaundice {1086} coexists with the obstruction, no portion of the bile escapes into the ducts. At the beginning of the obstruction more or less bile is in the tubes, and then the fluid will have a distinct biliary character; but as it accumulates, first the bile-constituents disappear, then the mucus--which at the outset was formed freely--is absorbed, and at last only a colorless serum remains. This fluid, which has been examined chemically by Frerichs, is found to be slightly alkaline, to have only 2 per cent. of solids, and to present no trace of any biliary constituent. As the fluid accumulates the gall-bladder and ducts dilate, sometimes to an enormous extent, the fluid they contain amounting to several pints. The walls of the ducts grow thinner, and may finally give way with the pressure or from external violence, the fluid exciting an intense and quickly-fatal peritonitis. Important changes occur in the structure of the liver also. With the first retention of bile the liver conspicuously enlarges, and may indeed attain to twice its normal size, but it subsequently contracts, and may lessen in as great a degree as it had enlarged. Changes begin in the glandular structure as pressure is brought to bear on the cells by the enlarging ducts. The liver-cells become anæmic and the protoplasm cloudy, but granular and fatty degeneration does not take place. Even more important as an agency affecting the condition of the hepatic cells is the hyperplasia of the connective tissue, which ensues very promptly when an obstruction to the flow of bile arises from any cause, as has been shown by Legg and Charcot. The liver on section has a rather dark olive-green color, and is firmer in texture, owing to the increased development of the connective tissue; the cells are bile-stained and contain granules of coloring matter and crystals of bilirubin, and although they are at first not altered in outline, subsequently more or less atrophy is produced by the contraction of the newly-formed connective tissue and the pressure made by the dilated hepatic ducts.

SYMPTOMS.--The symptoms produced by occlusion of the cystic duct are not sufficiently characteristic to be diagnosticated with any certainty. When an attack of hepatic colic has slowly subsided without jaundice, and an elastic tumor, globular or pyriform in shape, has appeared from under the inferior margin of the liver in the position of the gall-bladder, dropsy of that organ may then be suspected. As paracentesis of the gall-bladder may be performed with ease, safety, and little pain, the diagnosis may be rendered more certain by the use of the exploring-trocar.

Obstruction of the hepatic or common duct is accompanied by symptoms of a very pronounced and distinctly diagnostic character. Without referring now to the antecedent symptoms or to those belonging to the obstructing cause, the complexus of disturbances following the obstruction is the subject to which our attention must be directed. The great fact dominating all other considerations is the stoppage of the bile, whether this has occurred suddenly or slowly. Jaundice begins in a few hours after the canal is blocked. At first there is yellowness of the conjunctiva, then diffused jaundice, deepening into the intensest color in two or three weeks, or, when the obstruction is sudden and complete, in a few hours. At first the color is the vivid jaundice tint, a citron or salmon or yellow-saffron hue, but this gradually loses its bright appearance, grows darker, and passes successively into a brownish, bronze-like, and ultimately a {1087} dark olive-green, which becomes the permanent color. Under some moral emotional influences there may be a sudden change to a brighter tint, lasting a few minutes, but otherwise the general dark olive-green hue persists throughout. In a few instances, after some weeks of jaundice, the abnormal coloration entirely disappears, signifying that the liver is too much damaged in its proper glandular structure to be in a condition to produce bile. Such a cessation of the jaundice is therefore of evil omen.

Pruritus, sometimes of a very intense character, accompanies the jaundice, in most cases appears with it, and in the supposed curable cases it has persisted after the cessation of the discoloration. The irritation may become intolerable, destroying all comfort, rendering sleep impossible, and so aggravating as to induce a highly nervous, hysterical state. The scratching sets up an inflammation of the skin, and presently a troublesome eczema is superadded. In some of the cases a peculiar eruption occurs on the skin and mucous membranes, entitled by Wilson xanthelasma. It has been carefully studied by Wickham Legg, who has ascertained the character of the changes occurring in the affected tissues, and also by Mr. Hutchinson. As a rule, this eruption appears after several months of jaundice, and manifests itself first on the eyelids, then on the palms of the hands, where it makes the most characteristic exhibit, and after a time on the lips and tongue. It occurs in irregular plaques of a yellowish tint slightly elevated above the general surface, and rarely assumes a tubercular form. As was shown by Hilton Fagge, xanthelasma occurs more especially in the milder cases of catarrhal icterus that had been protracted in duration, but it is also occasionally seen in the jaundice of obstruction.

According to the stage of the disease during which the examination is made the liver will be enlarged or contracted; more or less tenderness may be developed by pressure in the area occupied by the ducts, and a tumor in a position to effect compression may possibly be detected. The area of hepatic dulness will be increased in the beginning of all the cases in which the obstruction is complete, but will remain normal so long as the flow of bile persists despite the obstruction. When enlarged, the liver can be felt projecting below the inferior margin of the ribs, and with it, in most cases, the elastic globular body, the gall-bladder. The state of the hepatic secretion, and in consequence the duration of the obstruction, may be ascertained by puncture of the gall-bladder and withdrawal of some of its contents for examination. The presence of unaltered bile will indicate recent obstruction; of serum, will prove long-standing interruption of bile-production. The presence of concretions in the gall-bladder will indicate the character of the obstructing cause, and an increased amount of bile of a normal or nearly normal kind will be conclusive evidence that the obstruction is in the course of the common duct. In a fatal case of permanent occlusion examined by myself the cystic duct was closed by inflammatory adhesions and the common duct was stopped up by a calculus.

The enlarged area of hepatic dulness will, in a protracted case, not continue. The proper secreting structure, the hepatic cells, undergo atrophy, {1088} and the increased connective tissue--to the development of which enlargement of the organ is mainly due--contracts. The ultimate result is that the liver becomes sclerosed, and is distinctly smaller, the area of hepatic dulness diminishing to a greater relative extent than the area of dulness due to hypertrophic enlargement. The contraction of the liver goes on at the rate that several months are required to make the result evident on percussion and palpation. Not unfrequently, the contraction is too slight to affect the percussion note of the right hypochondrium, and then, to realize the condition of the organ, the history and rational signs must be closely studied.

Whilst the liver thus varies in size, the gall-bladder remains enlarged and projects from the under surface of the organ, elastic, globular, and distinctive. The shrinking of the liver from around it makes the impression of growing size; it may be increasing, indeed, but more frequently the enlargement is merely apparent.

Whether the liver be enlarging or diminishing in size, its functions are impaired, or indeed entirely suspended. As the digestive canal receives the bile immediately on its production, it will be best to begin with the gastro-intestinal disorders which accompany occlusion of the bile-ducts. The appetite is either wanting entirely and food is loathed, or an excessive or canine appetite is experienced. The latter belongs rather to an early stage of the disorder, and comes on after the first disturbance of the stomach belonging to the immediate effects of the occlusion. The former is the result of long-standing interference with the primary assimilation. The tongue is coated with a thick yellowish fur, which, drying, is detached in flakes, leaving the mucous membrane beneath red, raw, fissured, and easily bleeding. The taste is bitter, and the mouth has a pasty, greasy, and unclean feeling. There is much thirst, and as a rule the patient experiences a keen desire for acid drinks and for fresh fruits. The stomach is rather intolerant of food, and nausea comes on as soon as it enters the stomach. The mucus and stomach-juice accumulating over night, in the morning there is much retching and nausea until the acid and rather foul contents of the organ come up. When food is retained it causes much distress, gases of decomposition accumulate, distending the stomach and giving prominence to the epigastrium, and eructations of offensive gas, with some acid liquid, occur from time to time. Similarly, in the intestines the foods undergo decomposition instead of normal digestion; gases of putrefaction are evolved, the abdomen generally is swollen, and flatulent colic results. Very irritating fat acids are liberated by the decomposition of the fatty constituents of the food, which, with the acid products of the fermentation occurring in the starch and sugar of the diet, cause a sensation of heat and distress through the abdomen. Usually, the bowels are torpid, but in some cases the stools are relaxed, having the consistence and presenting somewhat the appearance of oatmeal porridge. They may be firm, moulded, even hard. The gas discharged and the stools are offensive, with a carrion-like odor. Sometimes decomposing articles of food can be detected in the stools by very casual inspection--always, indeed, when the examination is intimate. An excess of fat is also a characteristic of the condition induced by occlusion of the ducts, especially when the pancreatic duct is closed, as does happen in cancer of the head of the pancreas.

{1089} A significant change in the color of the stools takes place. They lose their normal brownish-red tint and become yellowish or clay-colored or white, pasty, or grayish. Sometimes the stools are very dark, tar-like in color and consistence, or more thin like prune-juice, or in black scybalæ. The most usual appearance of the stools in occlusion is grayish, mush-like, and coarsely granular. The very dark hue assumed at times or in some cases signifies the presence of blood. A dark tint of the evacuations may be caused by articles of food, as a greenish hue may be due to the use of spinach; a clay-colored tint to the almost exclusive use of milk; a grayish tint to the action of bismuth; a bilious appearance to the action of rhubarb; and many others. When the occlusion is partial, although it be permanent, sufficient bile may descend into the duodenum to color the stools to the normal tint, and yet all the other signs of obstruction be present.

The bile-pigment, not having an outlet by the natural route, by the intestine, passes into the blood; all the tissues of the body and the various secretions and excretions, notably the urine, are stained by it, constituting the appearance known as jaundice or icterus. This malady has been described (see anté), but it is necessary now to give a more specialized account of those conditions due more especially to the prolonged obstruction of the biliary flow. These are a morbid state of the blood; changes in the kidneys and in the composition of the urine; a peculiar form of fever known as hepatic intermittent fever; and a group of nervous symptoms to which has been applied the term cholæmia.

It has already been shown that but little pressure is required to divert the flow of bile from the ducts backward into the blood. Changes consequently ensue in the constitution of the blood and in the action of the heart and of the vessels. The bile acids lower the heart's movements and lessen the arterial tension; hence the pulse is slower, softer, and feebler than the normal. Should fever arise, this depressing action of the bile acids is maintained; and hence, although the temperature becomes elevated, the pulse-rate does not increase correspondingly. There are exceptions to this, however, in so far that the heart and arteries are in some instances little affected, but it is probable under these circumstances that there are conditions present which induce decomposition of the bile acids.

The most important result of the action of the bile on the constitution of the blood is the hemorrhagic diathesis. Soon after the occlusion occurs in very young subjects--at a later period in adults--the occlusion having existed for many months, in some cases only near the end, the disposition to hemorrhagic extravasations and to hemorrhages manifests itself. From the surface of the mucous membranes, under the serous, in the substance of muscles, the hemorrhages occur. Epistaxis, or nasal hemorrhage, is usually the first to appear, and may be the most difficult to arrest. The gums transude blood, and wherever pressure is brought to bear on the integument ecchymoses follow. The conjunctiva may be disfigured and the eyelids swollen and blackened by extravasations, and the skin of the cheeks and nose marked by stigmata. Hæmatemesis sometimes occurs, but the extravasations into the intestinal canal more frequently--indeed, very constantly--take place in a gradual manner, and impart to the stools a dark, almost black, tar-like appearance. In the same way the urine may contain fluid blood and coagula, or it may have a merely smoky {1090} appearance from intimate admixture with the blood at the moment of secretion.

Both the bile-pigment and bile acids exert an injurious action on the kidneys. In cases of prolonged obstruction not only are the tissues of the organ stained by pigment in common with the tissues of the body, but the epithelium of the tubules, of the straight and convoluted tubes, are, according to Moebius, infiltrated with pigment. In consequence of the size and number of the masses of pigment, the tubes may become obstructed and the secretion of urine much diminished. Other changes occur, due chiefly to the action of the bile acids, according to the same authority. These alterations consist in parenchymatous degeneration. The urine contains traces of albumen in most cases, and, according to Nothnägel, always casts of the hyaline and granular varieties stained with pigment. As the alterations in the structure of the kidneys progress, fatty epithelium is cast off, and thus the tubules come finally to be much obstructed and the function of the organ seriously impaired. To cholæmia then are superadded the peculiar disturbances belonging to retention of the urinary constituents.

One of the most interesting complications which arises during the existence of obstruction of the bile-ducts is the form of fever entitled by Charcot intermittent hepatic fever. Although its character was first indicated by Monneret, we owe the present conception of its nature and its more accurate clinical history to Charcot. It does not occur in all cases. As has already been pointed out, the passage of a gall-stone may develop a latent malarial infection or a febrile movement comparable to that caused by the passage of a catheter, and known as urethral fever. Charcot supposes that true intermittent hepatic fever is septicæmic in character, and can therefore arise only in those cases accompanied by an angiocholitis of the suppurative variety--such, for example, as that which follows the passage of calculi. Illustrative cases of this fever, one of them confirmed by an autopsy, have been recently reported by E. Wagner, who is rather inclined to accept Charcot's view of the pathogeny. A remarkable case has been published by Regnard, in which the angiocholitis was induced by the extension of echinococcus cysts into the common duct. Whilst there are some objections to Charcot's theory, on the whole it is probably true that this intermittent hepatic fever is produced by the absorption from the inflamed surface of the ducts of a noxious material there produced. It may be likened to the fever which can be caused by the injection of putrid pus into the veins of animals.

Intermittent hepatic fever, as its name implies, is a paroxysmal fever, having a striking resemblance to malarial fever, but differs from it in less regularity of recurrence, in the fact that urea is below the normal amount instead of increased, and in the effect of quinine, which in the case of malarial fever is curative, but not curative in hepatic fever. The paroxysms are sometimes quotidian, rarely double quotidian, tertian, quartan, and even longer, and in the same case all of these varieties may occur; on {1091} the other hand, there may be entire regularity of the seizures. The severity of the chill, the maximum temperature, and the amount of sweating vary within considerable limits; there may be merely a slight sense of chilliness or a severe rigor; the temperature may rise to 101° or to 104° F., and there may be a gentle moisture or a profuse sweat. There does not seem to be any relation between the extent and severity of the local mischief and the systemic condition.

The period of onset of intermittent hepatic fever, and its duration and mode of termination, are by no means readily determined. Cyr fixes on the paroxysms of colic as the beginning, but he obviously confounds the chill and fever caused by the passage of a calculus with the true intermittent hepatic fever. In a carefully-observed case, the facts confirmed by an autopsy, E. Wagner gives the clinical history of a typical example of this malady: Gall-stones were found in the duodenum, in the common and cystic ducts, but the most important one was a polyangular stone obstructing the hepatic duct. There was an ulcer with thickened margin at the entrance to the gall-bladder, and the mucous membrane of the common duct near the intestinal orifice had a smooth, cicatricial aspect of recent origin, indicating inflammatory ulceration. The conditions favorable to the production of a morbid material of a kind to induce septicæmic fever were therefore present. The onset of fever occurred ten days after the last seizure, time being thus afforded for the local changes necessary. The duration of the fever in this case was five months, but the existence of pulmonary phthisis with cavities will explain this apparently protracted hepatic intermittent fever. The duration of the disease in its usual form is uncertain, and ranges between a week and two months, or even three months, according to Charcot.

Suspension of work by the liver necessarily involves retention in the blood of various excrementitious matters. The attempt of Flint to establish the doctrine of cholesteræmia has not been supported by the evidence of contemporary or subsequent physiologists. This theory denies to the other constituents of the bile any morbific action, and concentrates those disturbances known as cholæmia on the effects of cholesterin. As uræmia signifies not merely the presence of urea in the blood, but of all of the toxic substances excreted by the kidneys, so the word cholæmia comprehends all the constituents of bile having power to derange the organism by their presence in the blood.