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[190] and Charcot.[191] 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.
[Footnote 190: _On the Bile, Jaundice, and Bilious Diseases_, p. 352
_et seq._]
[Footnote 191: _Leçons sur les Maladies du Foie, etc._, p. 205 _et
seq._]
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[192] xanthelasma. It has been carefully studied by Wickham
Legg,[193] who has ascertained the character of the changes occurring
in the affected tissues, and also by Mr. Hutchinson.[194] 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.
[Footnote 192: _Diseases of the Skin_, 6th ed., Lond., p. 773.]
[Footnote 193: _On the Bile, Jaundice, and Bilious Diseases_, p. 317
_et seq._]
[Footnote 194: _Medico-Chirurgical Transactions_, vol. liv. p. 171.]
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,[195] 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,[196] 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.
[Footnote 195: _Archiv der Heilkunde_, vol. xviii. p. 83.]
[Footnote 196: _Deutsches Archiv für klin. Med._, vol. xii. p. 326;
also, Harley, _op. cit._, p. 503.]
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[197] intermittent hepatic fever. Although its
character was first indicated by Monneret,[198] 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,[199]
who is rather inclined to accept Charcot's view of the pathogeny. A
remarkable case has been published by Regnard,[200] 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.
[Footnote 197: _Leçons sur les Maladies du Foie, etc._, p. 178 _et
seq._]
[Footnote 198: Cyr, _Traité de l'Affection calculeuse du Foie_, p.
193.]
[Footnote 199: _Deutsches Archiv für klin. Medicin_, vol. xxxiv. p.
529.]
[Footnote 200: _Gazette méd. de Paris_, No. 49, 1873, quoted by Wagner,
_supra_.]
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[201] 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.[202]
[Footnote 201: _Deutsches Archiv für klinische Medicin_, Band xxxiv. p.
531, 1884.]
[Footnote 202: _Leçons sur les Maladies du Foie_, p. 180.]
Suspension of work by the liver necessarily involves retention in the
blood of various excrementitious matters. The attempt of Flint[203] 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.
[Footnote 203: _The American Journal of the Medical Sciences_, 1862, p.
349 _et seq._]
By cholæmia is meant those disturbances, chiefly nervous, which are due
to the presence of biliary excrementitious matters in the blood, and
not less to the effect on nutrition of the absence of bile from the
process of digestion in the intestine. As the atrophic changes proceed
in the liver, the quantity of urea and uric acid in the urine
diminishes, and presently leucin and tyrosin appear. Amongst the means
of differential diagnosis of hepatic intermittent fever from malarial
fever Charcot mentions the quantity of urea present--in the former
greatly lessened, in the latter much increased. There is, however, a
source of fallacy here not mentioned by Charcot: that is, the
variations in the amount of urea due to destruction of the hepatic
secreting structure. It follows that as changes {1092} occur in the
kidneys, to the condition of cholæmia is superadded the derangements
belonging to uræmia.
When the occlusion has existed for some time--a variable period, partly
due to peculiarities of individual structure--there come on certain
characteristic symptoms of nervous origin: headache, hebetude of mind,
dull hearing, obscure or hazy vision, xanthopsia; somnolence and
greatly increasing stupor, leading into coma; rambling and incoherence
of mind, passing into delirium; muscular twitching, subsultus; muscular
weakness, deepening into paralysis; and finally, it may be, general
convulsions. As these derangements of the nervous system develop, a
light febrile movement supervenes, so that the whole complexus has the
typhoid type, or, as it can be more definitely expressed, the patient
thus affected lapses into the typhoid state.
COURSE, DURATION, AND TERMINATION.--Occlusion of the gall-ducts is an
essentially chronic malady in the greatest number of cases. As a rule,
the causes of obstruction operate slowly, but to this rule there are
exceptions. Permanent occlusion may take place suddenly, as when a
gall-stone is impacted immovably in the common duct, or when a
round-worm makes its way into the duct and is firmly fixed there,
incapable of further movement.
When occlusion is once effected the gradual changes occurring in the
liver lead to slow decline of the nutrition; the bile-elements
circulating in the blood poison it and set up alterations in the
structure of the kidney, and ultimately, the brain becoming affected,
the end is reached by convulsions and coma. Although permanent
occlusion, if unrelieved, terminates in death, a small proportion of
cases get well, either in consequence of giving way of the obstructing
cause or from the opening of a new route to the intestine. Thus, a
calculus lodged in the fossa of Vater may suffer such injury to its
outer shell as to yield to the action of solvents, or, suppuration
occurring around it, the stone may be loosened and forced onward, or
ulceration may open a channel into the bowel. An incurable malady
causing the occlusion, the termination in death is only a question of
time. The duration of any case must be indefinite. There are several
factors, however, whose value can be approximately estimated. When the
obstructing cause is merely local--as, for example, a gall-stone or the
cicatrix of a simple ulcer--the duration of the case is determined by
the mere effect of the suspension of the hepatic functions. As the
eliminating action of the liver and the part played by the bile in the
intestinal digestion are necessary to life, it follows that the
complete cessation of these functions must lead to death. The rate at
which decline takes place under these circumstances varies somewhat in
different subjects. Probably two years may be regarded as the maximum,
and three months the minimum, period at which death ensues when no
other pathogenetic factor intervenes.
DIAGNOSIS.--To determine the fact of occlusion is by no means
difficult: the persistent jaundice, the absence of bile in the stools,
and the appearance of the bile-elements in the urine are sufficient. It
is far different when the cause of the occlusion is to be ascertained.