Data on the prevalence of gallstones in the United States indicate
that only about 30% of people with cholelithiasis come
to surgery. Symptoms of gallstone disease generally do not change
in severity. Each year, about 2% of patients with asymptomatic
gallstones develop symptoms, usually biliary colic rather than one
of the complications of gallstone disease. Patients with chronic
colic tend to have symptoms of the same level of severity and frequency.
The present practice of operating only on symptomatic patients, leaving
the millions without symptoms alone, seems appropriate. A question
is often raised about what to advise the asymptomatic patient found
to have gallstones during the course of unrelated studies. The presence
of either of the following portends a more serious course and should
probably serve as a reason for prophylactic cholecystectomy: (1) large
stones (> 2 cm in diameter), because they produce acute cholecystitis more
often than small stones; and (2) a calcified gallbladder, because
it so often is associated with carcinoma. However, most asymptomatic
patients have no special features. If coexistent cardiopulmonary
or other problems increase the risk of surgery, operation should
not be considered. For the average asymptomatic patient, it is not
reasonable to make a strong recommendation for cholecystectomy.
The tendency, however, is to operate on younger patients and temporize
in the elderly.
Beckingham IJ: ABC of diseases of liver, pancreas,
and biliary system. Gallstone disease. BMJ 2001;322:91.
Chronic Cholecystitis (Biliary Colic)
- Episodic abdominal pain.
- Gallstones on cholecystography or ultrasound scan.
Chronic cholecystitis is the most common form of symptomatic
gallbladder disease and is associated with gallstones in nearly
every case. In general, the term cholecystitis is applied whenever
gallstones are present regardless of the histologic appearance of
the gallbladder. Repeated minor episodes of obstruction of the cystic
duct cause intermittent biliary colic and contribute to inflammation
and subsequent scar formation. Gallbladders from symptomatic patients
with gallstones who have never had an attack of acute cholecystitis
are of two types: (1) In some, the mucosa may be slightly flattened,
but the wall is thin and unscarred and, except for the stones, appears
normal. (2) Others exhibit obvious signs of chronic inflammation,
with thickening, cellular infiltration, loss of elasticity, and
fibrosis. The clinical history in these two groups cannot always
be distinguished, and inflammatory changes may also be found in patients
with asymptomatic gallstones.
Biliary colic, the most characteristic symptom, is caused by
transient gallstone obstruction of the cystic duct. The pain usually
begins abruptly and subsides gradually, lasting for a few minutes to
several hours. The pain of biliary colic is usually steady—not
intermittent, like that of intestinal colic. In some patients, attacks
occur postprandially; in others, there is no relationship to meals.
The frequency of attacks is quite variable, ranging from nearly
continuous trouble to episodes many years apart. Nausea and vomiting
may accompany the pain.
Biliary colic is usually felt in the right upper quadrant, but
epigastric and left abdominal pain are common, and some patients
experience precordial pain. The pain may radiate around the costal
margin into the back or may be referred to the region of the scapula.
Pain on top of the shoulder is unusual and suggests direct diaphragmatic
irritation. In a severe attack, the patient usually curls up in
bed, changing position frequently in order to be more comfortable.
During an attack, there may be tenderness in the right upper
quadrant, and, rarely, the gallbladder is palpable.
Fatty food intolerance, dyspepsia, indigestion, heartburn, flatulence,
nausea, and eructations are other symptoms associated with gallstone
disease. Because they are also frequent in the general population,
their presence in any given patient may only be incidental to the
An ultrasound scan of the gallbladder should usually be the first
test. Gallstones can be demonstrated in about 95% of cases,
and a positive reading for gallstones is almost never in error.
An oral cholecystogram should be obtained if the ultrasound study
is equivocal, if the patient is a candidate for lithotripsy or ursodiol
therapy, or if symptoms are highly suggestive and an ultrasound study
has been read as normal.
About 2% of patients with gallstone disease have normal
ultrasound studies and oral cholecystograms. Therefore, if the clinical
suspicion of gallbladder disease is high and these two tests are
negative, the patient should be studied by ERCP (to opacify the
gallbladder in the search for stones) or duodenal intubation and
examination of duodenal bile for cholesterol crystals or bilirubinate
Gallbladder colic may be strongly suggested by the history, but
the clinical impression should always be verified by an ultrasound
study. Biliary colic may simulate the pain of duodenal ulcer, hiatal
hernia, pancreatitis, and myocardial infarction.
An electrocardiogram and a chest x-ray should be obtained to
investigate cardiopulmonary disease. It has been suggested that
biliary colic may sometimes aggravate cardiac disease, but angina pectoris
or an abnormal electrocardiogram should rarely be indications for cholecystectomy.
Right-sided radicular pain in the T6–T10 dermatomes
may be confused with biliary colic. Osteoarthritic spurs, vertebral lesions,
or tumors may be shown on x-rays of the spine or may be suggested
by hyperesthesia of the abdominal skin.
An upper gastrointestinal series may be indicated to search for
esophageal spasm, hiatal hernia, peptic ulcer, or gastric tumors.
In some patients, the irritable colon syndrome may be mistaken for gallbladder
discomfort. Carcinoma of the cecum or ascending colon may be overlooked
on the assumption that postprandial pain in these conditions is
due to gallstones.
Chronic cholecystitis predisposes to acute cholecystitis, common
duct stones, and adenocarcinoma of the gallbladder. The longer the
stones have been present, the higher the incidence of all of these
complications. Complications are infrequent, however, and the presence
of gallstones is not reason enough for prophylactic cholecystectomy
in a person with asymptomatic or mildly symptomatic disease.
Avoidance of offending foods may be helpful.
Cholesterol gallstones in the gallbladder can be dissolved in
some cases by chronic treatment with ursodiol, which reduces the
cholesterol saturation of bile by inhibiting cholesterol secretion.
The resulting undersaturated bile slowly dissolves the solid cholesterol
in the gallstones.
Unfortunately, bile salt therapy has marginal efficacy. The gallstones
must be small (eg, < 5 mm) and devoid of calcium (ie, nonopaque
on CT scans), and the gallbladder must opacify on oral cholecystography
(an indication of unobstructed flow of bile between bile duct and
gallbladder). About 15% of patients with gallstones are
candidates for treatment. Dissolution is achieved within 2 years
in about 50% of highly selected patients. Stones recur,
however, in 50% of cases within 5 years. In general, dissolution
therapy—alone or in conjunction with lithotripsy—is
used very rarely.
Extracorporeal shock wave lithotripsy (ESWL) involves focusing
shock waves, which pass through tissue and fluids, upon the gallstones.
The stones are fragmented by explosion of small air bubbles within interstices
of the solid material.
Lithotripsy is of little therapeutic value because the fragments
remain in the gallbladder unless they can be dissolved. Consequently,
candidates for lithotripsy must also use ursodiol therapy. Complete
elimination of gallbladder stones is attained within 9 months in
about 25% of appropriately selected patients. Because of
the many drawbacks of this form of treatment, it has not been approved
by the US Food and Drug Administration.
Cholecystectomy is indicated in most patients with symptoms.
The procedure can be scheduled at the patient’s convenience,
within weeks or months after diagnosis. Active concurrent disease
that increases the risk of surgery should be treated before operation.
In some chronically ill patients, surgery should be deferred indefinitely.
Cholecystectomy is most often performed laparoscopically, but
when the laparoscopic approach is contraindicated (eg, too many
adhesions) or unsuccessful, it may be performed through a laparotomy.
The difference consists of 4 fewer days in the hospital and fewer weeks
off work when done laparoscopically. Regardless of how it is done,
operative cholangiography is often included to look for common duct
stones. If stones are found, common duct exploration may be performed
(see section on Choledocholithiasis).
Serious complications and deaths related to the operation itself
are rare. The operative death rate is about 0.1% in patients
under age 50 and about 0.5% in patients over age 50. Most
deaths occur in patients recognized preoperatively to have increased
risks. The operation relieves symptoms in 95% of cases.
Beyer AJ 3rd, Delcore R, Cheung LY: Nonoperative
treatment of biliary tract disease. Arch Surg 1998;133:1172.
Binmoeller KF, Schafer TW: Endoscopic management of bile duct
stones. J Clin Gastroenterol 2001;32:106.
Calland JF et al: Outpatient laparoscopic cholecystectomy: patient
outcomes after implementation of a clinical pathway. Ann Surg 2001;233:704.
Fletcher DR et al: Complications of cholecystectomy: risks of
the laparoscopic approach and protective effects of operative cholangiography:
a population-based study. Ann Surg 1999;229:449.
Gadacz TR: Update on laparoscopic cholecystectomy, including
a clinical pathway. Surg Clin North Am 2000;80:1127.
Maxwell JG et al: Cholecystectomy in patients aged 80 and older.
Am J Surg 1998;176:627.
Montori A et al: Endoscopic and surgical integration in the
approach to biliary tract disease. J Clin Gastroenterol 1999;28:198.
Moonka R et al: The presentation of gallstones and results of
biliary surgery in a spinal cord injured population. Am J Surg 1999;178:246.
Sakuramoto S et al: Preoperative evaluation to predict technical
difficulties of laparoscopic cholecystectomy on the basis of histological
inflammation findings on resected gallbladder. Am J Surg 2000;179:114.
Stuart SA et al: Routine intraoperative laparoscopic cholangiography.
Am J Surg 1998;176:632.
Tocchi A et al: The need for antibiotic prophylaxis in elective
laparoscopic cholecystectomy: a prospective randomized study. Arch
Traverso LW: Risk factors for intraoperative injury during cholecystectomy:
an ounce of prevention is worth a pound of cure. Ann Surg 1999;229:458.
Yerdel MA et al: Direct trocar insertion versus Veress needle
insertion in laparoscopic cholecystectomy. Am J Surg 1999;177:247.
- Acute right upper quadrant pain and tenderness.
- Fever and leukocytosis.
- Palpable gallbladder in one third of cases.
- Nonopacified gallbladder on radionuclide excretion scan.
- Sonographic Murphy sign.
In 80% of cases, acute cholecystitis results from obstruction
of the cystic duct by a gallstone impacted in the Hartmann pouch.
The gallbladder becomes inflamed and distended, creating abdominal
pain and tenderness. The natural history of acute cholecystitis
varies, depending on whether the obstruction becomes relieved, the
extent of secondary bacterial invasion, the age of the patient,
and the presence of other aggravating factors such as diabetes mellitus. Most
attacks resolve spontaneously without surgery or other specific
therapy, but some progress to abscess formation or free perforation
with generalized peritonitis.
The pathologic changes in the gallbladder evolve in a typical
pattern. Subserosal edema and hemorrhage and patchy mucosal necrosis
are the first changes. Later, polymorphonuclear (PMN) leukocytes
appear. The final stage involves development of fibrosis. Gangrene
and perforation may occur as early as 3 days after onset, but most
perforations occur during the second week. In cases that resolve spontaneously,
acute inflammation has largely cleared by 4 weeks, but some residual
evidence of inflammation may last for several months. About 90% of
gallbladders removed during an acute attack show chronic scarring,
although many of these patients deny having had any previous symptoms.
The cause of acute cholecystitis is still partially conjectural.
Obstruction of the cystic duct is present in most cases, but in
experimental animals, cystic duct obstruction does not result in
acute cholecystitis unless the gallbladder is filled with concentrated
bile or bile saturated with cholesterol. There is also evidence
that trauma from gallstones releases phospholipase from the mucosal
cells of the gallbladder. This is followed by conversion of lecithin
in bile to lysolecithin, which is a toxic compound that may cause
more inflammation. Bacteria appear to have a minor role in the early
stages of acute cholecystitis, even though most complications of
the disease involve suppuration.
About 20% of cases of acute cholecystitis occur in the
absence of cholelithiasis (acalculous cholecystitis). Some of these are
due to cystic duct obstruction by another process such as a malignant tumor.
Rarely, acute acalculous cholecystitis results from cystic artery
occlusion or primary bacterial infection by Escherichia
coli, clostridia, or, occasionally, Salmonella
typhi. Most cases occur in patients hospitalized with some
other illness; acute acalculous cholecystitis is particularly common
in trauma victims (civilian or military) and in patients receiving
total parenteral nutrition. Small-vessel occlusion occurs early,
and unless treatment is given promptly, the disease progresses rapidly
to gangrenous cholecystitis and septic complications, at which point
the death rate is high.
The first symptom is abdominal pain in the right upper quadrant,
sometimes associated with referred pain in the region of the right
scapula. In 75% of cases, the patient will have had previous attacks
of biliary colic, at first indistinguishable from the present illness.
However, in acute cholecystitis, the pain persists and becomes associated
with abdominal tenderness. Nausea and vomiting are present in about
half of patients, but the vomiting is rarely severe. Mild icterus
occurs in 10% of cases. The temperature usually ranges from
38 to 38.5 °C. High fever and chills are uncommon and should suggest
the possibility of complications or an incorrect diagnosis.
Right upper quadrant tenderness is present, and in about a third
of patients the gallbladder is palpable (often in a position lateral
to its normal one). Voluntary guarding during examination may prevent
detection of an enlarged gallbladder. In others, the gallbladder
is not enlarged because scarring of the wall restricts distention.
If instructed to breathe deeply during palpation in the right subcostal
region, the patient experiences accentuated tenderness and sudden
inspiratory arrest (Murphy sign).
The leukocyte count is usually elevated to 12,000–15,000/μL.
Normal counts are common, but if the count goes much above 15,000,
one should suspect complications. A mild elevation of the serum
bilirubin (in the range of 2–4 mg/dL) is common,
presumably owing to secondary inflammation of the common duct by
the contiguous gallbladder. Bilirubin values above this range would
most likely indicate the associated presence of common duct stones.
A mild increase in alkaline phosphatase may accompany the attack. Occasionally,
the serum amylase concentration transiently reaches 1000 units/dL
A plain x-ray of the abdomen may occasionally show an enlarged
gallbladder shadow. In 15% of patients, the gallstones
contain enough calcium to be seen on the plain film.
Ultrasound scans show gallstones, sludge, and thickening of the
gallbladder wall, and the ultrasonographer can determine even better
than the clinician whether the point of maximum tenderness is over
the gallbladder (ultrasonographic Murphy sign). This last finding
is often absent, however, when the gallbladder is gangrenous. Usually,
ultrasound is the only test needed to make the diagnosis of acute
If additional diagnostic information is desirable (eg, if ultrasound
is equivocal or negative), a radionuclide excretion scan (eg, HIDA
scan) should be performed. This test cannot demonstrate gallstones,
but if the gallbladder is imaged, acute cholecystitis is ruled out except
in rare cases of acalculous cholecystitis (the test is positive
in most cases of acute acalculous cholecystitis). Imaging of the
duct but not the gallbladder supports the diagnosis of acute cholecystitis.
A few false positives are seen in advanced gallstone disease without acute
inflammation and in acute biliary pancreatitis.
The differential diagnosis includes other common causes of acute
upper abdominal pain and tenderness. An acute peptic ulcer with
or without perforation might be suggested by a history of epigastric
pain relieved by food or antacids. Most cases of perforated ulcer
demonstrate free air under the diaphragm on x-ray. An emergency
upper gastrointestinal series may help.
Acute pancreatitis can be confused with acute cholecystitis,
especially if cholecystitis is accompanied by an elevated amylase
level. Furthermore, HIDA scans fail to outline the gallbladder in
most cases of acute biliary pancreatitis. Sometimes the two diseases
coexist, but pancreatitis should not be accepted as a second diagnosis
without specific findings.
Acute appendicitis in patients with a high cecum may closely
simulate acute cholecystitis.
Severe right upper quadrant pain with high fever and local tenderness
may develop in acute gonococcal perihepatitis (Fitz-Hugh-Curtis
syndrome). Clues to the proper diagnosis may be found in tenderness
in the adnexa, vaginal discharge that shows gonococci on a Gram-stained
smear, and a disparity between the patient’s high fever
and her general lack of toxicity.
The major complications of acute cholecystitis are empyema, gangrene,
In empyema (suppurative cholecystitis), the gallbladder contains
frank pus, and the patient becomes more toxic, with high spiking
fever (39–40 °C), chills, and leukocytosis greater than
15,000/μL. Parenteral antibiotics should
be given, and percutaneous cholecystostomy or cholecystectomy should
Perforation may take any of three forms: (1) localized perforation
with pericholecystic abscess, (2) free perforation with generalized
peritonitis, and (3) perforation into an adjacent hollow viscus,
with the formation of a fistula. Perforation may occur as early
as 3 days after the onset of acute cholecystitis or not until late
in the second week. The total incidence of perforation is about
Pericholecystic abscess, the most common form of perforation,
should be suspected when the signs and symptoms progress, especially when
accompanied by the appearance of a palpable mass. The patient often becomes
toxic, with fever to 39 °C and a leukocyte count above 15,000/μL,
but sometimes there is no correlation between the clinical signs
and the development of local abscess. Cholecystectomy and drainage
of the abscess can be performed safely in many of these patients,
but if the patient’s condition is unstable, percutaneous
cholecystostomy is preferable.
Free perforation occurs in only 1–2% of patients,
most often early in the disease when gangrene develops before adhesions
wall off the gallbladder. The diagnosis is made preoperatively in less
than half of cases. In some patients with localized pain, sudden
spread of pain and tenderness to other parts of the abdomen suggests
the diagnosis. Whenever it is suspected, free perforation must be
treated by emergency laparotomy. Abdominal paracentesis may be misleading
and has proved to be of little diagnostic usefulness. Cholecystectomy should
be performed if the patient’s condition will permit; otherwise,
cholecystostomy is done. The death rate depends partly on whether
the cystic duct remains obstructed or the stone becomes dislodged
after perforation. The former leads to a purulent peritonitis that
is lethal in 20% of cases. In the latter, a true bile peritonitis
ensues and over 50% of patients die. The earlier operation
is performed, the better the prognosis.
If the acutely inflamed gallbladder becomes adherent to adjacent
stomach, duodenum, or colon and necrosis develops at the site of one
of these adhesions, perforation may occur into the lumen of the
gut. The resulting decompression often allows the acute disease
to resolve. If the gallbladder stones discharge through the fistula and
if they are large enough, they may obstruct the small intestine
(gallstone ileus; see later in chapter). Rarely, patients vomit
gallstones that have entered the stomach through a cholecystogastric
fistula. In most patients, the acute attack subsides and the cholecystenteric
fistula is clinically unsuspected.
Cholecystenteric fistulas do not usually cause symptoms unless
the gallbladder is still partially obstructed by stones or scarring.
Neither oral nor intravenous cholangiograms will opacify the gallbladder
or the fistula, but the latter may be shown on upper gastrointestinal
series, where it must be differentiated from a fistula due to perforated
peptic ulcer. Malabsorption and steatorrhea have been reported in
isolated cases of cholecystocolonic fistulas. Steatorrhea in this
situation could be due either to absence of bile in the proximal
bowel following diversion into the colon or, more rarely, to excess
bacteria in the upper intestine.
Symptomatic cholecystenteric fistulas should be treated by cholecystectomy and
closure of the fistula. The majority are discovered incidentally
during cholecystectomy for symptomatic gallbladder disease.
Intravenous fluids should be given to correct dehydration and
electrolyte imbalance, and a nasogastric tube should be inserted.
For acute cholecystitis of average severity, parenteral cefazolin
(2–4 g daily) should be given. Parenteral penicillin (20
million units daily), clindamycin, and an aminoglycoside should
be given for severe disease. Single-drug therapy using imipenem
is a good alternative.
There are two schools of thought about the treatment of acute
cholecystitis. Since the disease resolves with antibiotics and supportive
care in about 60% of cases, one approach is to manage the patient
expectantly, with a plan to perform elective cholecystectomy after recovery,
reserving surgery during the acute attack for those with severe
or worsening disease. (This approach is untenable in acute acalculous
The preferred plan is to perform cholecystectomy in all patients
unless there are specific contraindications to operation (eg, serious
concomitant disease). Four controlled trials have supported this
approach with the following data: (1) the incidence of technical
complications is no greater with early surgery; (2) early surgery
reduces the total duration of illness by approximately 30 days,
length of hospitalization by 5–7 days, and direct medical
costs by several thousand dollars; and (3) the death rate is slightly
lower with early surgery because of earlier treatment for some patients
whose condition would have worsened during expectant management. Since
these trials were completed, the average case appears to have become
more severe, and the arguments against expectant management are
now even more compelling.
The following are the major factors that affect the decision
(Figure 25–8): (1) whether the diagnosis
is established; (2) the general health of the patient as modified
by coexistent disease or the present illness; and (3) signs of local
complications of acute cholecystitis. The diagnosis should be clear-cut
and the patient optimally prepared; if perforation or empyema is
suspected, emergency surgery is indicated.
Scheme for the management of acute cholecystitis.
In about 30% of cases, the diagnosis of acute cholecystitis
is established but the general condition of the patient is unsatisfactory.
If possible, surgery should be postponed in these cases until the
ancillary disease is controlled. Expectant management cannot be
rigidly adhered to, however, if the manifestations of cholecystitis
About 10% of patients require emergency treatment. These
are generally clinical situations in which the disease appears to
have become complicated or is about to. High fever (39 °C), marked leukocytosis
(> 15,000/μL), or chills suggest suppurative
progression. Acalculous acute cholecystitis should automatically
be placed in this category. When the patient’s general condition
is poor, percutaneous catheter cholecystostomy is the preferable
treatment. Patients in better overall health should be treated by
The sudden appearance of generalized abdominal pain may indicate
free perforation. Appearance of a mass while the patient is under
observation may be a sign of local perforation and abscess formation.
Changes of this sort are indications for emergency surgery.
Cholecystectomy is the preferable operation in acute cholecystitis,
and it can be performed laparoscopically in about 50% of
patients. Operative cholangiography should be performed in most
cases, and the common bile duct explored if appropriate indications
are present (see section on Choledocholithiasis). Patients with
severe acute cholecystitis who are in poor condition for emergency
cholecystectomy should be treated by percutaneous cholecystostomy.
Percutaneous cholecystostomy may also be the preferred therapy for
acute acalculous cholecystitis. A catheter inserted under ultrasound
or CT guidance is allowed to drain the gallbladder of its bile or
pus. The resulting decompression controls the acute disease, including
any local infection, but the gallstones cannot be removed. Therefore,
cholecystectomy should be performed after the patient recovers in
order to avoid recurrent attacks. Cholecystectomy is definitive therapy
in the patient with acalculous cholecystitis. At one time, cholecystostomy
was performed surgically, but most hospitals now have radiologists skilled
in the simpler percutaneous method.
The overall death rate of acute cholecystitis is about 5%.
Nearly all of the deaths are in patients over age 60 or those with diabetes
mellitus. In the older age group, secondary cardiovascular or pulmonary complications
contribute substantially to the death rate. Uncontrolled sepsis
with peritonitis and intrahepatic abscesses are the most important
local conditions responsible for death.
Common duct stones are present in about 15% of patients with
acute cholecystitis, and some of the more seriously ill patients
have simultaneous cholangitis from biliary obstruction. Acute pancreatitis
may also complicate acute cholecystitis, and the combination carries
a greater risk.
Patients who develop the suppurative forms of gallbladder disease
such as empyema or perforation are less likely to recover. Earlier
admission to the hospital and early cholecystectomy reduce the chances
of these complications.
Berber E et al: Selective use of tube cholecystostomy
with interval laparoscopic cholecystectomy in acute cholecystitis.
Arch Surg 2000;135:341.
Borzellino G et al: Emergency cholecystostomy and subsequent cholecystectomy
for acute gallstone cholecystitis in the elderly. Br J Surg 1999;86:1521.
Davis CA et al: Effective use of percutaneous cholecystostomy
in high-risk surgical patients: techniques, tube management, and
results. Arch Surg 1999;134:727.
Eldar S et al: The impact of patient delay and physician delay
on the outcome of laparoscopic cholecystectomy for acute cholecystitis.
Am J Surg 1999;178:303.
Geoghegan JG, Keane FB: Laparoscopic management of complicated
gallstone disease. Br J Surg 1999;86:145.
Greenwald JA et al: Standardization of surgeon-controlled variables:
impact on outcome in patients with acute cholecystitis. Ann Surg
Kim KH et al: Percutaneous gallbladder drainage for delayed
laparoscopic cholecystectomy in patients with acute cholecystitis.
Am J Surg 2000;179:111.
Laycock WS et al: Variation in the use of laparoscopic cholecystectomy
for elderly patients with acute cholecystitis. Arch Surg 2000;135:457.
Lillemoe KD: Surgical treatment of biliary tract infections.
Am Surg 2000;66:138.
Lobe TE: Cholelithiasis and cholecystitis in children. Semin
Pediatr Surg 2000;9:170.
Svanvik J: Laparoscopic cholecystectomy for acute cholecystitis.
Eur J Surg 2000;(Suppl 585):16.
Emphysematous cholecystitis is a rare condition in which bubbles
of gas from anaerobic infection appear in the lumen of the gallbladder,
its wall, the pericholecystic space, and, on occasion, the bile
ducts. Clostridia species are the most commonly implicated organisms,
but other gas-forming anaerobes such as E coli or
anaerobic streptococci may be found. Three times as many men as
women are affected, and 20% of patients have diabetes mellitus.
In contrast to the usual form of acute cholecystitis, the disease probably
is a bacterial infection from the earliest moment. In many cases,
the gallbladder contains no stones.
The disease begins with sudden and rapidly progressive right
upper quadrant pain. Fever and leukocytosis reach high levels quickly,
and the patient is considerably more toxic than is usually the case
in acute cholecystitis. On examination, a mass can usually be found
in the right upper quadrant.
Plain films of the abdomen show tissue emphysema outlining the
gallbladder and, in some cases, an air-fluid level in the lumen.
The clinical and x-ray pictures are characteristic enough so that
the diagnosis is usually obvious. If the changes on plain films
are equivocal, a CT scan may bring them out.
The patient should be treated with high doses of antibiotics
effective against clostridia and the other species mentioned above.
Emergency surgical treatment should follow the initial resuscitative
measures. Cholecystectomy can be safely performed in most cases,
but the most critically ill might fare better with cholecystostomy.
The types of complications are the same as in other forms of acute
cholecystitis, but illness is more severe and death rates are higher.
Danse EM, Laterre PF: Images in clinical medicine.
Emphysematous cholecystitis. N Engl J Med 1999;341:1126.
Garcia-Sancho Tellez L et al: Acute emphysematous cholecystitis. Report
of twenty cases. Hepatogastroenterology 1999;46:2144.
Zeebregts CJ et al: Percutaneous drainage of emphysematous cholecystitis associated
with pneumoperitoneum. Hepatogastroenterology 1999;46:771.
Gallstone ileus is mechanical intestinal obstruction caused by
a large gallstone lodged in the lumen. It is seen most often in
women, and the average age is about 70.
The patient usually presents with obvious small bowel obstruction,
either partial or complete. The obstructing gallstone enters the
intestine through a cholecystenteric fistula located in the duodenum, colon,
or, rarely, the stomach or jejunum. The gallbladder may contain
one or several stones, but stones that cause gallstone ileus are
almost always 2.5 cm or more in diameter. The lumen in the proximal
bowel will allow most of these large calculi to pass caudally until
the ileum is reached. Obstruction of the large intestine may follow
passage of a gallstone through a fistula at the hepatic flexure
or may occur even after the stone has traversed the entire small
In most patients, the findings on physical examination are typical
of distal small bowel obstruction. Obstruction of the duodenum or
jejunum may give a perplexing clinical picture because of the lack
of distention. Right upper quadrant tenderness and a mass may be
present in some cases, but the distended abdomen may be difficult
to examine accurately.
In addition to dilated small intestine, plain films of the abdomen
may show a radiopaque gallstone, and unless one is alert to the
possibility of gallstone ileus, the ectopic stone can be a puzzling
finding. In about 40% of cases, careful examination of
the film will reveal gas in the biliary tree, a manifestation of
the cholecystenteric fistula. When the clinical picture is unclear,
an upper gastrointestinal series should be obtained, which will demonstrate
the cholecystoduodenal fistula and verify intestinal obstruction.
The proper treatment is emergency laparotomy and removal of the
obstructing stone through a small enterotomy. The proximal intestine
must be carefully inspected for the presence of a second calculus
that might cause a postoperative recurrence. The gallbladder should be
left undisturbed at the original operation.
Once the patient has recovered, an elective cholecystectomy should
be scheduled if the patient complains of chronic gallbladder symptoms.
On this basis, interval cholecystectomy will be required in about
30% of patients. The fistula itself is rarely the source
of trouble and closes spontaneously in most patients.
The death rate of gallstone ileus remains about 20%,
largely because of the poor general condition of elderly patients
at the time of laparotomy. In many cases, the patient has developed
cardiac or pulmonary complications during a preoperative delay when
the diagnosis was unclear.
Lobo DN, Jobling JC, Balfour TW: Gallstone ileus:
diagnostic pitfalls and therapeutic successes. J Clin Gastroenterol 2000;30:72.
Scarpa F et al: Gallstone ileus: diagnostic pitfalls and therapeutic
successes. J Clin Gastroenterol 2000;30:72.
Bacterial infection of the biliary ducts always signifies biliary obstruction,
since in the absence of obstruction even heavy bacterial contamination
of the ducts fails to produce symptoms or pathologic changes. The
block to flow may be partial or, less commonly, complete. The principal
causes are choledocholithiasis, biliary stricture, and neoplasm.
Less common causes are chronic pancreatitis, ampullary stenosis,
pancreatic pseudocyst, duodenal diverticulum, congenital cyst, and
parasitic invasion. Iatrogenic cholangitis may complicate transhepatic
or T tube cholangiography. Not all obstructing lesions are followed
by cholangitis, however. For example, biliary infection develops in
only 15% of patients with neoplastic obstruction. The likelihood
of cholangitis is greatest when the obstruction occurs after the
duct has acquired a resident bacterial population.
With obstruction, ductal pressure rises, and bacteria proliferate
and escape into the systemic circulation via the hepatic sinusoids.
Experimentally, the incidence of positive blood cultures with ductal infection
is directly proportionate to the absolute height of the pressure
in the duct.
The symptoms of cholangitis (sometimes called the Charcot triad)
are biliary colic, jaundice, and chills and fever, though a complete
triad is present in only 70% of cases. Laboratory findings
include leukocytosis and elevated serum bilirubin and alkaline phosphatase
levels. The predominant organisms in bile (in approximately decreasing
frequency) are E coli, klebsiella, pseudomonas,
enterococci, and proteus. Bacteroides fragilis and
other anaerobes (eg, Clostridium perfringens) can
be detected in about 25% of cases, and their presence correlates
with multiple previous biliary operations (often including a biliary
enteric anastomosis), severe symptoms, and a high incidence of postoperative
suppurative complications. Anaerobes are nearly always seen in the
company of aerobes. Two species of bacteria can be cultured in about
50% of cases. Bacteremia probably occurs in most cases,
and blood cultures obtained at the appropriate time contain the
same organisms as the bile. Early in an attack, an ultrasound scan
will often give useful diagnostic information. Further workup (THC,
ERCP, etc) can proceed later after the acute manifestations are
brought under control. Cholangiography is dangerous during active
The term suppurative cholangitis has been used for
the most severe form of this disease, when manifestations of sepsis
overshadow those of hepatobiliary disease. The diagnostic pentad
of suppurative cholangitis consists of abdominal pain, jaundice,
fever and chills, mental confusion or lethargy, and shock. The diagnosis
is often missed because the signs of biliary disease are overlooked.
Most cases of cholangitis can be controlled with intravenous
antibiotics. A cephalosporin antibiotic (eg, cefazolin, cefoxitin)
is the drug of choice in the average mild to moderately severe case. If
disease is severe or progressively worsens, an aminoglycoside plus
clindamycin or metronidazole should be added to the regimen.
For patients with severe cholangitis or unremitting cholangitis
despite antibiotic therapy, the bile duct must be promptly decompressed.
Most cases of severe acute cholangitis are associated with choledocholithiasis,
where the best treatment consists of emergency endoscopic sphincterotomy.
In the uncommon case where this is unsuccessful, laparotomy is indicated
in order to decompress the bile duct. Cholangitis accompanying neoplastic
obstruction may be managed by insertion of a transhepatic drainage
catheter into the bile duct. A cholangiogram should not be obtained
because the procedure could worsen sepsis.
Urgent intervention (eg, endoscopic sphincterotomy, percutaneous
transhepatic drainage, or operative decompression) is required in
about 10% of patients with acute cholangitis. The remaining
90% are eventually treated by elective surgery or endoscopic
sphincterotomy following antibiotic therapy and a thorough diagnostic
Elsakr R et al: Antimicrobial treatment of intra-abdominal
infections. Dig Dis 1998;16:47.
Hanau LH, Steigbigel NH: Acute (ascending) cholangitis. Infect
Dis Clin North Am 2000;14:521.
Poon RT et al: Management of gallstone cholangitis in the era
of laparoscopic cholecystectomy. Arch Surg 2001;136:11.
Raraty MG, Finch M, Neoptolemos JP: Acute cholangitis and pancreatitis
secondary to common duct stones: management update. World J Surg 1998;22:1155.
- Biliary pain.
- Episodic cholangitis.
- Gallstones in gallbladder or previous cholecystectomy.
Approximately 15% of patients with stones in the gallbladder
are found to harbor calculi within the bile ducts. Common duct stones
are usually accompanied by others in the gallbladder, but in 5% of
cases, the gallbladder is empty. The number of duct stones may vary from
one to more than 100.
There are two possible origins for common duct stones. The evidence
suggests that most cholesterol stones develop within the gallbladder
and reach the duct after traversing the cystic duct. These are called
secondary stones. Pigment stones may have a similar pedigree or,
more often, develop de novo within the common duct. These are called
primary common duct stones. About 60% of common duct stones
are cholesterol stones and 40% are pigment stones. The
latter are generally associated with more severe clinical manifestations.
Patients may have one or more of the following principal clinical
findings, all of which are caused by obstruction to the flow of
bile or pancreatic juice: biliary colic, cholangitis, jaundice,
and pancreatitis (Figure 25–9).
It seems likely, however, that as many as 50% of patients
with choledocholithiasis remain asymptomatic.
The natural history of common duct stones. Of every 100
patients with gallbladder stones, 15 will have common duct stones,
which will produce the spectrum of syndromes illustrated. Note that the
individual syndromes overlap, indicating that they may appear together
in various combinations.
The common duct may dilate to 2–3 cm proximal to an
obstructing lesion, and truly huge ducts develop in patients with biliary
tumors. In choledocholithiasis or biliary stricture, the inflammatory
reaction restricts dilation, so the dilatation is less marked. Dilation
of the ductal system within the liver can also be limited by cirrhosis.
Biliary colic is the result of rapid rises in biliary pressure
whether the block is in the common duct or neck of the gallbladder.
Gradual occlusion of the duct—as in cancer—rarely
produces the same kind of pain as gallstone disease.
Choledocholithiasis may be asymptomatic or may produce sudden
toxic cholangitis, leading to a rapid demise. The seriousness of
the disease parallels the degree of obstruction, the length of time it
has been present, and the extent of secondary bacterial infection
(see earlier section on Cholangitis). Biliary colic, jaundice, or
pancreatitis may be isolated findings or may occur in any combination along
with signs of infection (cholangitis).
Biliary colic from common duct obstruction cannot be distinguished
from that caused by stones in the gallbladder. The pain is felt
in the right subcostal region, epigastrium, or even the substernal
area. Referred pain to the region of the right scapula is common.
Choledocholithiasis should be strongly suspected if intermittent
chills, fever, or jaundice accompanies biliary colic. Some patients
notice transient darkening of their urine during an attack even
though jaundice is not evident.
Pruritus is usually the result of persistent, longstanding obstruction.
The itching is more intense in warm weather when the patient perspires
and is usually worse on the extremities than on the trunk. It is much
more common with neoplastic obstruction than with gallstone obstruction.
The patient may be icteric and toxic, with high fever and chills,
or may appear to be perfectly healthy. A palpable gallbladder is
unusual in patients with obstructive jaundice from common duct stone because
the obstruction is transient and partial, and scarring of the gallbladder renders
it inelastic and nondistensible. Tenderness may be present in the
right upper quadrant but is not often as marked as in acute cholecystitis,
perforated peptic ulcer, or acute pancreatitis. Tender hepatic enlargement
In cholangitis, leukocytosis of 15,000/μL is
usual, and values above 20,000/μL are
common. A rise in serum bilirubin often appears within 24 hours
after the onset of symptoms. The absolute level usually remains
under 10 mg/dL, and most are in the range of 2–4
mg/dL. The direct fraction exceeds the indirect, but the
latter becomes elevated in most cases. Bilirubin levels do not ordinarily reach
the high values seen in malignant tumors because the obstruction
is usually incomplete and transient. In fact, fluctuating jaundice
is so characteristic of choledocholithiasis that it fairly reliably differentiates
between benign and malignant obstruction.
The serum alkaline phosphatase level usually rises and may be
the only chemical abnormality in patients without jaundice. When
the obstruction is relieved, the alkaline phosphatase and bilirubin levels
should return to normal within 1–2 weeks, with the exception
that the former may remain elevated longer if the obstruction was
Mild increases in AST and ALT are often seen with extrahepatic
obstruction of the ducts; rarely, AST levels transiently reach 1000
Radiopaque gallstones may be seen on plain abdominal films or
CT scans. Ultrasound scans will usually show gallbladder stones
and, depending on the degree of obstruction, dilatation of the bile
duct. Ultrasound and CT scans are insensitive in the search for
stones in the common duct. ERCP is indicated if the patient has had
a previous cholecystectomy. If cholecystectomy has not been performed, cholangiography
should be part of operative management. Some clinicians choose preoperative
ERCP for patients scheduled for cholecystectomy in order to clear
the common bile duct. If ERCP is not technically successful, the
surgeon will be forced to convert to open common bile duct exploration
to clear the duct of stones.
Bilirubin values above 10 mg/dL are so uncommon in choledocholithiasis
that when this finding is present, cholangiography should be performed
to rule out the possibility of neoplastic obstruction.
The workup should consider the same possibilities in differential
diagnosis as for cholecystitis.
Serum amylase levels above 500 units/dL can result from
acute pancreatitis, acute cholecystitis, or choledocholithiasis.
Other manifestations of pancreatic disease should be documented
before an unqualified diagnosis of pancreatitis is accepted.
Alcoholic cirrhosis or acute alcoholic hepatitis may present
with jaundice, right upper quadrant tenderness, and leukocytosis.
The differentiation from cholangitis may be impossible from clinical
data. A history of a recent binge suggests acute liver disease.
A percutaneous liver biopsy may be specific.
Intrahepatic cholestasis from drugs, pregnancy, chronic active
hepatitis, or primary biliary cirrhosis may be difficult to distinguish
from extrahepatic obstruction. ERCP would be appropriate to make
the distinction, particularly if other studies (eg, ultrasound scan)
failed to provide evidence of gallstone disease. If jaundice has
persisted for 4–6 weeks, a mechanical cause is probable.
Since most patients improve during this interval, persistent jaundice
should never be assumed to be the result of parenchymal disease
unless a normal cholangiogram rules out obstruction of the major
Intermittent jaundice and cholangitis after cholecystectomy are
compatible with biliary stricture, and the distinction requires ERCP.
Biliary tumors usually produce intense jaundice without biliary
colic or fever, and once it begins, the jaundice rarely remits.
Longstanding ductal infection can produce intrahepatic abscesses.
Hepatic failure or secondary biliary cirrhosis may develop in unrelieved
obstruction of long duration. Since the obstruction is usually incomplete
and intermittent, cirrhosis develops only after several years in untreated
disease. Acute pancreatitis, a fairly common complication of calculous biliary
disease, is discussed in Chapter 26. Rarely,
a stone in the common duct may erode through the ampulla, resulting in
gallstone ileus. Hemorrhage (hemobilia) is also a rare complication.
Patients with acute cholangitis should be treated with systemic
antibiotics and other measures as described in the preceding section;
this usually controls the attack within 24–48 hours. If
the patient’s condition worsens or if marked improvement
is not observed within 2–4 days, endoscopic sphincterotomy
or surgery and common bile duct exploration should be performed.
The typical patient presents with mild cholangitis and evidence
on ultrasound scans of gallbladder stones. Laparoscopic cholecystectomy
is indicated, and depending on the experience of the surgeon, laparoscopic
exploration of the common duct should be done if an operative cholangiogram
or laparoscopic ultrasound demonstrates the expected common duct
stones. Laparoscopic common duct exploration is usually accomplished
through the cystic duct (which may have to be dilated), but when
the common duct is enlarged (> 1.5 cm), it may be accomplished through
a choledochotomy incision, just as in open surgery. Eventually,
nearly all cases of common duct stones should be manageable by laparoscopic
techniques, but at this stage the requisite laparoscopic skills
are not available in most hospitals. If the surgeon thinks the common
duct stones cannot be removed laparoscopically, it is probably best
to remove the gallbladder laparoscopically and the common duct stones
by endoscopic sphincterotomy. If the stones cannot be removed by
sphincterotomy, a second (open) operation may be necessary.
There is also a lack of consensus regarding the importance of
operative cholangiography or ultrasound during cholecystectomy when
there are no clues suggesting stones in the duct. In such cases,
the chances of finding a stone are only 3–5%,
and some consider the effort unwarranted. On the other hand, operative
cholangiograms also provide confirmation of the biliary anatomy,
which contributes to avoidance of bile duct injuries, and the natural
history of the few overlooked stones is worrisome. Therefore, we
side with those who perform operative cholangiography liberally
in such cases.
When the common duct is explored through the cystic duct and
gallstones are removed, the cystic duct must be ligated, but a drainage
catheter is not usually left within the common duct. When the common
duct is explored through a choledochotomy (either during a laparoscopic
or open operation), a T tube is usually left in the duct, and cholangiograms
are taken a week or so postoperatively. Any residual stones discovered
on these postoperative x-rays can be extracted 4–6 weeks
later through the T tube tract.
Patients with common duct stones who have had a previous cholecystectomy are
best treated by endoscopic sphincterotomy. Using a
side-viewing duodenoscope, the ampulla is cannulated, and a 1-cm
incision is made in the sphincter with an electrocautery wire. The
opening created in the sphincter permits stones to pass from the
duct into the duodenum. Endoscopic sphincterotomy is unlikely to
be successful in patients with large stones (eg, > 2 cm), and it
is contraindicated in the presence of stenosis of the bile duct proximal
to the sphincter. Laparotomy and common duct exploration are required
in a few cases.
Stones in the intrahepatic branches of the bile duct can usually
be removed without difficulty during common duct exploration. In
some cases, however, one or more of the intrahepatic ducts have
become packed with stones, and the associated chronic inflammation
has produced stenosis of the duct near its junction with the common
hepatic duct. It is often impossible in these cases to clear the
duct of stones, and if the disease involves only one lobe (usually
the left lobe), hepatic lobectomy is indicated.
Binmoeller KF, Schafer TW: Endoscopic management
of bile duct stones. J Clin Gastroenterol 2001;32:106.
Lauter DM, Froines EJ: Laparoscopic common duct exploration
in the management of choledocholithiasis. Am J Surg 2000;179:372.
Prat F et al: Prediction of common bile duct stones by noninvasive
tests. Ann Surg 1999;229:362.
Rosenthal RJ, Rossi RL, Martin RF: Options and strategies for
the management of choledocholithiasis. World J Surg 1998;22:1125.
Soetikno RM, Montes H, Carr-Locke DL: Endoscopic management
of choledocholithiasis. J Clin Gastroenterol 1998;27:296.
Soper NJ: Intraoperative detection: intraoperative cholangiography vs.
intraoperative ultrasonography. J Gastrointest Surg 2000;4:334.
Suc B et al: Surgery vs endoscopy as primary treatment in symptomatic
patients with suspected common bile duct stones: a multicenter randomized
trial. French Associations for Surgical Research. Arch Surg 1998;133:702.
Tranter SE, Thompson MH: Potential of laparoscopic ultrasonography
as an alternative to operative cholangiography in the detection
of bile duct stones. Br J Surg 2001;88:65.
Wu JS, Dunnegan DL, Soper NJ: The utility of intracorporeal
ultrasonography for screening of the bile duct during laparoscopic
cholecystectomy. J Gastrointest Surg 1998;2:50.
Postcholecystectomy syndrome has been used to
signify the heterogeneous group of disorders affecting patients
who continue to complain of symptoms after cholecystectomy. It is
not really a syndrome, and the term is confusing.
The usual reason for incomplete relief after cholecystectomy
is that the preoperative diagnosis of chronic cholecystitis was
incorrect. The only symptom entirely characteristic of chronic cholecystitis
is biliary colic. When a calculous gallbladder is removed in the
hope that the patient will gain relief from dyspepsia, fatty food
intolerance, belching, and other symptoms, the operation may leave
the symptoms unchanged.
The presenting symptom may be dyspepsia or pain. An organic cause
for the symptoms is more likely to be discovered in patients with
severe episodic pain than in those with other complaints. Abnormal liver
function studies, jaundice, and cholangitis are other manifestations
that indicate residual biliary disease. Patients with suspicious
findings should be studied by ERCP or THC. Choledocholithiasis,
biliary stricture, and chronic pancreatitis are the most common causes
of symptoms. Evidence is accumulating to implicate sphincter of
Oddi dysmotility as a cause of pain in some patients. The diagnosis
may be possible by biliary manometry, but experience is still too
meager to justify acceptance of this entity without question. Relief
of pain may follow endoscopic sphincterotomy. Stenosis of the hepatobiliary
ampulla, a long cystic duct remnant, and neuromas have been blamed
for continued symptoms, but well-verified cases are uncommon.
Carcinoma of the gallbladder is an uncommon neoplasm that occurs
in elderly patients. It is associated with gallstones in 70% of
cases, and the risk of malignant degeneration correlates with the
length of time gallstones have been present. The tumor is twice
as common in women as in men, as one would expect from the association
Most primary tumors of the gallbladder are adenocarcinomas that
appear histologically to be scirrhous (60%), papillary (25%),
or mucoid (15%). Dissemination of the tumor occurs early
by direct invasion of the liver and hilar structures and by metastases
to the common duct lymph nodes, liver, and lungs. In an occasional
case, where carcinoma is an incidental finding after cholecystectomy for
gallstone disease, the tumor is confined to the gallbladder as a
carcinoma in situ or an early invasive lesion. Most invasive carcinomas,
however, have spread by the time of surgery, and spread is virtually
certain if the tumor has progressed to the point where it causes symptoms.
The most common presenting complaint is of right upper quadrant
pain similar to previous episodes of biliary colic but more persistent.
Obstruction of the cystic duct by tumor sometimes initiates an attack
of acute cholecystitis. Other cases present with obstructive jaundice
and, occasionally, cholangitis due to secondary involvement of the
Examination usually reveals a mass in the region of the gallbladder,
which may not be recognized as a neoplasm if the patient has acute
cholecystitis. If cholangitis is the principal symptom, a palpable gallbladder
would be an unusual finding with choledocholithiasis alone and should
suggest gallbladder carcinoma.
Oral cholecystograms almost never opacify except in patients
with small incidental cancers. CT and ultrasound scans may demonstrate
the extent of disease, but more often they show only gallstones.
The correct diagnosis is made preoperatively in only 10% of
Obstruction of the common duct may produce multiple intrahepatic
abscesses. Abscesses in or next to the tumor-laden gallbladder are
The incidence of gallbladder cancer has decreased in recent years
as the frequency of cholecystectomy has increased. It has been estimated
that one case of gallbladder cancer is prevented for every 100 cholecystectomies performed
for gallstone disease.
If a localized carcinoma of the gallbladder is recognized at
laparotomy, cholecystectomy should be performed along with en bloc
wedge resection of an adjacent 3–5 cm of normal liver and
dissection of the lymph nodes in the hepatoduodenal ligament. If
a small invasive carcinoma overlooked during cholecystectomy for
gallstone disease is later discovered by the pathologist, reoperation
is indicated to perform a wedge resection of the liver bed plus
regional lymphadenectomy. Some surgeons also recommend that the
common duct be included routinely (ie, even in the absence of gross
invasion) in the lymph node dissection for any lesion that involves
the full thickness of the gallbladder wall. In the few cases where
cancer has not penetrated the muscularis mucosae, cholecystectomy
alone should suffice. More extensive hepatectomies (eg, right lobectomy)
are not worthwhile. Lesions that invade the bile duct and produce
jaundice should be resected if possible. When not, a stent should
be inserted endoscopically or percutaneously. There is little that
surgery can offer in cases with hepatic metastases or more distant
Radiotherapy and chemotherapy are not effective palliative measures.
About 85% of patients are dead within a year after diagnosis.
The 10% of patients who presently survive more than
5 years consist of those whose carcinoma was an incidental finding
during cholecystectomy for symptomatic gallstone disease and those
in whom an aggressive resection has removed all gross tumor.
Baillie J: Tumors of the gallbladder and bile
ducts. J Clin Gastroenterol 1999;29:14.
Bismuth H, Majno PE: Hepatobiliary surgery. J Hepatol 2000;32(1 Suppl):208.
Kondo S et al: Regional and para-aortic lymphadenectomy in radical
surgery for advanced gallbladder carcinoma. Br J Surg 2000;87:418.
Mainprize KS, Gould SW, Gilbert JM: Surgical management of polypoid
lesions of the gallbladder. Br J Surg 2000;87:414.
Scott TE et al: A case-control assessment of risk factors for
gallbladder carcinoma. Dig Dis Sci 1999;44:1619.
Sugiyama M, Atomi Y, Yamato T: Endoscopic ultrasonography for
differential diagnosis of polypoid gall bladder lesions: analysis
in surgical and follow up series. Gut 2000;46:250.
of the Bile Duct
- Intense cholestatic jaundice and pruritus.
- Anorexia and dull right upper quadrant pain.
- Dilated intrahepatic bile ducts on ultrasound or CT scan.
- Focal stricture on transhepatic or retrograde endoscopic cholangiogram.
Primary bile duct tumors are not more common in patients with
cholelithiasis, and men and women are affected with equal frequency.
Tumors appear at an average age of 60 years but may appear at any
time between 20 and 80 years of age. More young people have been
seen with this disease in recent years. Ulcerative colitis is a
common associated condition, and in occasional cases, bile duct
cancer develops in a patient with ulcerative colitis who has been
known to have sclerosing cholangitis for several years. Chronic parasitic
infestation of the bile ducts in the Orient may be responsible for
the greater incidence of bile duct tumors in that area.
Most malignant biliary tumors are adenocarcinomas located in
the hepatic or common bile duct. The histologic pattern varies from
typical adenocarcinoma to tumors composed principally of fibrous stroma
and few cells. The acellular tumors may be mistaken for benign strictures
or sclerosing cholangitis if adequate biopsies are not obtained.
About 10% are bulky papillary tumors, which tend to be
less invasive and less apt to metastasize.
At presentation, metastases are uncommon, but the tumor has often
grown into the portal vein or hepatic artery.
The illness presents with gradual onset of jaundice or pruritus.
Chills, fever, and biliary colic are usually absent, and except
for a deep discomfort in the right upper quadrant, the patient feels
well. Bilirubinuria is present from the start, and light-colored
stools are usual. Anorexia and weight loss develop insidiously with time.
Icterus is the most obvious physical finding. If the tumor is
located in the common duct, the gallbladder may distend and become
palpable in the right upper quadrant. The tumor itself is never
palpable. Patients with tumors of the hepatic duct do not develop
palpable gallbladders. Hepatomegaly is common. If obstruction is
unrelieved, the liver may eventually become cirrhotic, and splenomegaly, ascites,
or bleeding varices become secondary manifestations.
Since the duct is often completely obstructed, the serum bilirubin
is usually over 15 mg/dL. Serum alkaline phosphatase is
also increased. Fever and leukocytosis are not common, since the
bile is sterile in most cases. The stool may contain occult blood,
but this is more common with tumors of the pancreas or hepatopancreatic
ampulla than those of the bile ducts.
Ultrasound or CT scans usually detect dilated intrahepatic bile
ducts. THC or ERCP clearly depicts the lesion, and both are indicated
in most cases. THC is of greater value, since it better demonstrates
the ductal anatomy on the hepatic side of the lesion. With tumors
involving the bifurcation of the common hepatic duct (Klatskin tumors), it
is important to determine the proximal extent of the lesion (ie,
whether the first branches of the lobar ducts are also involved).
ERCP is of value with proximal tumors because if it shows concomitant
obstruction of the cystic duct, the diagnosis will most often prove
to be gallbladder cancer invading the common duct (not a primary
common duct neoplasm). The typical pattern with distal bile duct
cancers consists of stenosis of the bile duct with sparing of the pancreatic
duct. Adjacent stenoses of both ducts (the double-duct sign) indicate
primary cancer of the pancreas. MR cholangiopancreatography may
be useful if high-quality studies are available.
Occasionally, bile samples obtained at the time of THC will show
malignant cells on cytologic study, but this is not a particularly
useful test, since the diagnosis of cancer must be presumed from
the cholangiographic findings and a negative cytologic study is
unreliable. Angiography may suggest invasion of the portal vein
or encasement of the hepatic artery. False positives may occur,
The differential diagnosis must consider other causes of extrahepatic
and intrahepatic cholestatic jaundice. Choledocholithiasis is characterized
by episodes of partial obstruction, pain, and cholangitis, which
contrast with the unremitting jaundice of malignant obstruction.
Bilirubin concentrations rarely surpass 15 mg/dL and are
usually below 10 mg/dL in gallstone obstruction, whereas
bilirubin levels almost always exceed 10 mg/dL and are
usually above 15 mg/dL in neoplastic obstruction. A rapid
rise of the bilirubin level to above 15 mg/dL in a patient
with sclerosing cholangitis should suggest superimposed neoplasm.
Dilatation of the gallbladder may occur with tumors of the distal
common duct but is rare with calculous obstruction.
The combination of an enlarged gallbladder with obstructive jaundice
is usually recognized as being due to tumor. If the gallbladder
cannot be felt, primary biliary cirrhosis, drug-induced jaundice,
chronic active hepatitis, metastatic hepatic cancer, and common
duct stone must be ruled out. In general, any patient with cholestatic
jaundice of more than 2 weeks’ duration whose diagnosis
is uncertain should be studied by THC or ERCP. The finding of focal
bile duct stenosis in the absence of previous biliary surgery is
almost pathognomonic of neoplasm.
Patients without evidence of metastases or other signs of advanced
cancer (eg, ascites) are candidates for laparotomy. The 30% of
patients who do not qualify may be treated by insertion of a tube stent
into the bile duct transhepatically under radiologic control or
from the duodenum under endoscopic control. The tube is positioned
so that holes above and below the tumor reestablish flow of bile
into the duodenum. If both lobar ducts are blocked by a tumor at
the bifurcation of the common hepatic duct, it is usually necessary
to place a transhepatic tube into only one lobar duct. If the lesion
blocks the takeoff of the segmental ducts, stents are rarely beneficial.
Laparotomy is indicated in most cases, however, with the objective
of removing the tumor. Preoperative decompression of the bile duct
with a percutaneous catheter to relieve jaundice does not lower the
incidence of postoperative complications. At operation, which may
be immediately preceded by diagnostic laparoscopy, the extent of
the tumor should be determined by external examination of the bile
duct and the adjacent portal vein and hepatic artery.
Tumors of the distal common duct should be treated by radical
pancreaticoduodenectomy (Whipple procedure) if it appears that all
tumor would be removed. Secondary involvement of the portal vein
is the usual reason for unresectability of tumors in this location.
Mid common duct or low hepatic duct tumors should also be removed
if possible. If the tumor cannot be excised, bile flow should be
reestablished into the intestine by a cholecystojejunostomy or Roux-en-Y choledochojejunostomy.
The choice is based on technical considerations.
Tumors at the hilum of the liver should be resected if possible
and a Roux-en-Y hepaticojejunostomy performed. The anastomosis is
usually between hilum and bowel rather than between individual bile
ducts and bowel. A curative operation nearly always requires resection
of either the right or the left lobe of the liver and, in all cases,
the caudate lobe. Extension into the lobar and segmental ducts and
secondary involvement of the hepatic artery and portal vein are
the most common reasons for inability to resect the tumor. Subtotal
resections offer little in the way of palliation.
Postoperative radiotherapy is commonly recommended.
The average patient with adenocarcinoma of the bile duct survives
less than a year. The overall 5-year survival rate is 15%.
Following a thorough radical operation, 5-year survival is about
40%. Biliary cirrhosis, intrahepatic infection, and general
debility with terminal pneumonitis are the usual causes of death.
Palliative resections and stents may improve the length and quality
of survival in this disease even though surgical cure is uncommon.
Limited experience with liver transplantation for this disease has
been discouraging: tumor has recurred postoperatively in most patients.
Ahrendt SA, Nakeeb A, Pitt HA: Cholangiocarcinoma.
Clin Liver Dis 2001;5:191.
Burke EC et al: Hilar cholangiocarcinoma: patterns of spread,
the importance of hepatic resection for curative operation, and
a presurgical clinical staging system. Ann Surg 1998;228:385.
Chamberlain RS, Blumgart LH: Hilar cholangiocarcinoma: a review
and commentary. Ann Surg Oncol 2000;7:55.
Jarnagin WR: Cholangiocarcinoma of the extrahepatic bile ducts.
Semin Surg Oncol 2000;19:156.
Kosuge T et al: Improved surgical results for hilar cholangiocarcinoma
with procedures including major hepatic resection. Ann Surg 1999;230:663.
Lillemoe KD, Cameron JL: Surgery for hilar cholangiocarcinoma:
the Johns Hopkins approach. J Hepatobiliary Pancreat Surg 2000;7:115.
Molmenti EP et al: Hepatobiliary malignancies. Primary hepatic malignant
neoplasms. Surg Clin North Am 1999;79:43.
& Pseudotumors of the Gallbladder
Various unrelated lesions appear on the cholecystogram as projections
from the gallbladder wall. The differentiation from gallstones is
based upon observing whether a shift in position of the projections
follows changes in posture of the patient, since stones are not
fixed. Cancer should be suspected in any polypoid lesion that exceeds
1 cm in diameter.
Most of these are not true neoplasms but cholesterol polyps, a
local form of cholesterosis. Histologically, they consist of a cluster
of lipid-filled macrophages in the submucosa. They easily become
detached from the wall when the gallbladder is handled at surgery.
It is not known whether cholesterol polyps are important in the
genesis of gallstones. Some patients experience gallbladder pain,
but whether this is related to the presence of the polyps per se or
is a manifestation of functional gallbladder disease has not been
Inflammatory polyps have also been reported, but they are quite
On cholecystography, this entity presents as a slight intraluminal
convexity that is often marked by central umbilication. It is usually
found in the fundus but may occur elsewhere. It is unclear whether adenomyomatosis
is an acquired degenerative lesion or a developmental abnormality
(ie, hamartoma). The following synonyms for this lesion appear in
the literature: adenomatous hyperplasia, cholecystitis glandularis
proliferans, and diverticulosis of the gallbladder. Although the
condition is probably asymptomatic in many cases, adenomyomatosis
can cause abdominal pain. Cholecystectomy should be performed in
These appear as pedunculated adenomatous polyps, true neoplasms
that may be papillary or nonpapillary histologically. In a few cases,
they have been found in association with carcinoma in situ of the gallbladder.
Bile Duct Injuries
- Episodic cholangitis.
- Previous biliary surgery.
- Transhepatic cholangiogram often diagnostic.
Benign biliary injuries and strictures are caused by surgical
trauma in about 95% of cases. The remainder result from external
abdominal trauma or, rarely, from erosion of the duct by a gallstone. Prevention
of injury to the duct depends on a combination of technical skill,
experience, and a thorough knowledge of the normal anatomy and its
variations in the hilum of the liver. The number of bile duct injuries
has risen sharply in the past few years along with the shift from
open to laparoscopic cholecystectomy.
The most common lesion consists of excision of a segment of the
common duct as a result of mistaking it for the cystic duct. Partial
transection, occlusion with metal clips, injury to the right hepatic duct,
and leakage from the cystic duct are other examples. A full discussion
of how these injuries occur and how they can be prevented is beyond
the scope of this text.
A clean incision of the duct without additional damage is best
managed by opening the abdomen and suturing the incision with fine
absorbable suture material.
Manifestations of injury to the duct may or may not be evident
in the postoperative period. Following laparoscopic surgery, bile
ascites, manifested by abdominal distention, bloating, and pain plus
mild jaundice, is the usual presentation, since the duct is usually
open to the abdomen. The symptoms are relatively mild and may for
a time be thought to represent only ileus until a worsening picture
requires further investigation.
Injuries following open cholecystectomy more often present with
intermittent cholangitis or jaundice as a consequence of a biliary
stricture. The first clear-cut symptoms may not be evident for weeks or
months after surgery.
Findings are not distinctive. Bile ascites produces abdominal
distention and ileus and, rarely, true bile peritonitis with toxicity.
The right upper quadrant may be tender but usually is not. Jaundice
is usually present during an attack of cholangitis.
The serum alkaline phosphatase concentration is elevated in cases
of stricture. The serum bilirubin fluctuates in relation to symptoms
but usually remains well below 10 mg/dL.
Blood cultures are usually positive during acute cholangitis.
Bile ascites can be suspected on ultrasound or CT scan. Fluid
should be aspirated, and if it is bile, the diagnosis is clear.
THC and ERCP are necessary to depict the anatomy. After laparoscopic cholecystectomy,
the most common pattern is a blocked (by a metal clip) lower duct
and an upper duct draining freely into the abdomen. With a stricture,
the findings most often consist of focal narrowing of the common
hepatic duct within 2 cm of the bifurcation and mild to moderate
dilatation of the intrahepatic ducts.
Choledocholithiasis is the condition that most often must be
differentiated from biliary stricture because the clinical and laboratory
findings can be identical. A history of trauma to the duct would
point toward stricture as the more likely diagnosis. The final distinction
must often await radiologic or surgical findings. THC or ERCP should
Other causes of cholestatic jaundice may have to be ruled out
in some cases.
Complications develop quickly if the leak is not controlled.
Bile peritonitis and abscesses may form. With stricture, persistent
cholangitis may progress to multiple intrahepatic abscesses and
a septic death.
Bile duct injuries should be surgically repaired in all but a
few patients who are likely to improve with a nonoperative approach.
Excision of the damaged duct and Roux-en-Y hepaticojejunostomy is indicated
for most acute and chronic injuries. The entire biliary tree must
be outlined by cholangiograms preoperatively. The key to success
is the thoroughness of the dissection and the ability ultimately to
suture healthy duct to healthy bowel. This, in turn, depends on
the experience of the surgeon with this particular operation.
When a definitive repair is technically impossible, the stricture
may be dilated with a transhepatic balloon-tipped catheter. This
is particularly applicable to patients with portal hypertension,
whose hepatic hilum contains numerous venous collaterals that make
The death rate from biliary injuries is about 5%, and
severe illness is frequent. If the stricture is not repaired, episodic cholangitis
and secondary liver disease are inevitable.
Surgical correction of the stricture should be successful in
about 90% of cases. Experience at centers with a special interest
in this problem indicates that good results can be obtained even
if several previous attempts did not relieve the obstruction. There
is essentially no place for liver transplantation in this disease.
Nealon WH, Urrutia F: Long-term follow-up after
bilioenteric anastomosis for benign bile duct stricture. Ann Surg 1996;223:639.
Savader SJ et al: Laparoscopic cholecystectomy-related bile
duct injuries: a health and financial disaster. Ann Surg 1997;225:268.
Strasberg SM, Eagon CJ, Drebin JA: The “hidden cystic
duct” syndrome and the infundibular technique of laparoscopic cholecystectomy:
the danger of the false infundibulum. J Am Coll Surg 2000;191:661.
Strasberg SM, Hertl M, Soper NJ: An analysis of the problem
of biliary injury during laparoscopic cholecystectomy. J Am Coll
Strasberg SM, Picus DD, Drebin JA: Results of a new strategy
for reconstruction of biliary injuries having an isolated right-sided
component. J Gastrointest Surg 2001;5:266.
Yeh TS et al: Value of magnetic resonance cholangiopancreatography
in demonstrating major bile duct injuries following laparoscopic
cholecystectomy. Br J Surg 1999;86:181.
of Bile Duct Obstruction
About 30% of congenital choledochal cysts produce their
first symptoms in adults, usually presenting with jaundice, cholangitis,
and a right upper quadrant mass. Diagnosis can be made by THC or ERCP.
The optimal surgical procedure is excision of the cyst and construction
of a Roux-en-Y hepaticojejunostomy. If this is not technically possible
or if the patient’s condition will not permit a prolonged operation,
the cyst should be emptied of precipitated biliary sludge and a
cystenteric anastomosis constructed. Congenital cysts of the biliary
tree have a high incidence of malignant degeneration, which is another
argument for excision rather than drainage.
Vercruysse R, Van den Bossche MR: Choledochal
cyst in adults. Acta Chir Belg 1998;98:220.
Watanatittan S, Niramis R: Choledochal cyst: review of 74 pediatric
cases. J Med Assoc Thai 1998;81:586.
Caroli disease, another form of congenital cystic disease, consists
of saccular intrahepatic dilatation of the ducts. In some cases,
the biliary abnormality is an isolated finding, but more often it
is associated with congenital hepatic fibrosis and medullary sponge
kidney. The latter patients often present in childhood or as young
adults with complications of portal hypertension. Others have cholangitis and
obstructive jaundice as initial manifestations. There is no definitive
surgical solution to the problem except in rare cases with isolated
involvement of one hepatic lobe, where lobectomy is curative. Intermittent
antibiotic therapy for cholangitis is the usual regimen.
Hara H et al: Surgical treatment for congenital
biliary dilatation, with or without intrahepatic bile duct dilatation.
Parada LA et al: Clonal chromosomal abnormalities in congenital
bile duct dilatation (Caroli’s disease). Gut 1999;45:780.
Waechter FL et al: The role of liver transplantation in patients
with Caroli’s disease. Hepatogastroenterology 2001;48:672.
Hemobilia presents with the triad of biliary colic, obstructive
jaundice, and occult or gross intestinal bleeding. Most cases in
Western cultures follow several weeks after hepatic trauma with
bleeding from an intrahepatic branch of the hepatic artery into
a duct. It is seen with less frequency now, because the general principles
of management of hepatic trauma are better understood. In the Orient,
hemobilia usually follows ductal parasitism (Ascaris lumbricoides)
or Oriental cholangiohepatitis. Other causes are hepatic neoplasms,
rupture of a hepatic artery aneurysm, hepatic abscess, and choledocholithiasis.
The diagnosis may be suspected from a technetium-99m-labeled red
blood cell scan, but an arteriogram is usually required for diagnosis
and planning of therapy. Sometimes the bleeding can be stopped by
embolizing the lesion with stainless steel coils, Gelfoam, or autologous
blood clot infused through a catheter selectively positioned in
the hepatic artery. If this is unsuccessful, either direct ligation of
the bleeding point in the liver or proximal ligation of an upstream
branch of the hepatic artery in the hilum is required.
Pancreatitis can cause obstruction of the intrapancreatic portion
of the bile duct by inflammatory swelling, encasement with scar,
or compression by a pseudocyst. The patient may present with painless jaundice
or cholangitis. Occasionally, a distended gallbladder can be felt
on abdominal examination. Differentiation from choledocholithiasis
and secondary acute pancreatitis depends on biliary x-rays or surgical
exploration if the jaundice persists. Jaundice due to inflammation
alone rarely lasts more than 2 weeks; persistent jaundice following
an attack of acute pancreatitis suggests the development of a pseudocyst,
underlying chronic pancreatitis with obstruction by fibrosis, or
even an obstructing neoplasm.
Biliary obstruction from chronic pancreatitis may have few or
no clinical manifestations. Jaundice is usually present, but the
average peak bilirubin level is only 4–5 mg/dL.
Some patients with functionally significant stenosis have persistently
elevated alkaline phosphatase levels as the only abnormality; when
surgical decompression of the bile duct is not performed, these
patients often develop secondary biliary cirrhosis within a year
or so. Diagnosis of stricture is made by ERCP, which shows a long stenosis
of the intrapancreatic portion of the duct, proximal dilatation,
and either a gradual or abrupt tapering of the lumen at the pancreatic
border, occasionally accompanied by ductal angulation. If cholangiograms
show stenosis and if alkaline phosphatase or bilirubin levels remain
more than twice normal for longer than 2 months, the stenosis is
functionally significant and unlikely to resolve and requires surgical
correction. Choledochoduodenostomy is done in most cases. Cholecystoduodenostomy
is unreliable because the cystic duct is often too narrow to provide
continued biliary decompression.
Patients with obstructive jaundice and pseudocyst usually respond
to surgical drainage of the pseudocyst. However, occasionally they
do not respond, because chronic scarring—not the cyst—is
the cause of obstruction. Procedures to drain both the bile duct
and the pseudocyst are indicated if operative cholangiograms demonstrate
persistent bile duct obstruction after the cyst has been decompressed.
Stenosis of the hepatopancreatic ampulla (ampullary stenosis)
has been implicated as a cause of pain and other manifestations
of ampullary obstruction and is often considered as a cause of postcholecystectomy
complaints. Some cases are idiopathic, whereas others may be the
result of trauma from gallstones. If the patient has secondary manifestations
of biliary obstruction (eg, jaundice, increased alkaline phosphatase
concentration, cholangitis) in the absence of gallstones or some
other obstructing lesion, and cholangiography shows dilatation of
the common duct, ampullary stenosis is a plausible explanation.
However, the diagnosis is more often proposed as a reason for upper abdominal
pain without these more objective findings. Ampullary dysfunction
is postulated in these cases.
Sphincter of Oddi dysfunction may be the cause of biliary-like
pain and is often considered in patients who remain uncomfortable
after cholecystectomy. The pathogenesis of the symptoms is thought to
be similar to that of esophageal dysmotility and the irritable bowel
syndrome. The patients typically experience severe, intermittent
upper abdominal pain that lasts for 1–3 hours, sometimes
following a meal.
Residual gallstone and pancreatic disease must first be ruled
out. Ampullary dysfunction can then be diagnosed by sphincter of
Oddi manometry. Patients are placed in one of three groups depending
on the presence of three objective manifestations of biliary obstruction:
abnormal liver function tests, prolonged (> 45 minutes) common bile duct
emptying of contrast media after ERCP; and a common duct greater
than 12 mm in diameter. Patients in group I have all three findings;
patients in group II have one or two findings; and patients in group
III have none of the findings. Group I patients are thought to have enough
evidence of disease that sphincterotomy should be performed without manometry.
Group I patients have abnormal motility so rarely that they should
not be considered further for sphincterotomy. Thus, motility studies
are most often of value in determining which of the group II patients
will improve after sphincterotomy.
The abnormalities sought on the motility studies include an elevated
(> 40 mm Hg) basal sphincter pressure and a paradoxic rise in sphincter
pressure in response to CCK. The former is most reliable. About
50% of group II patients have elevated sphincter pressures,
and these are the ones who benefit from sphincterotomy.
A scintigraphic test may be just as accurate. The patient is
given a bolus of CCK followed by technetium-99m diisopropyl iminodiacetic
acid (99mTc-DISIDA). Gamma camera images of the liver and
bile duct are obtained for 60 minutes. A scoring system (score:
0–12) is based on the rate of passage of the imaging agent
past various relevant points (eg, appearance and clearance through
the liver, bile duct, and bowel). The normal range is 0–5;
abnormal is 6–12.
Sphincter of Oddi dysfunction is an uncommon explanation for
abdominal pain, and it is appropriate to remain skeptical unless
the objective findings of biliary obstruction are clear-cut. In
well-selected cases, however, endoscopic sphincterotomy is truly
Chen JW, Saccone GT, Toouli J: Sphincter of Oddi
dysfunction and acute pancreatitis. Gut 1998;43:305.
Rosenblatt ML et al: Comparison of sphincter of Oddi manometry,
fatty meal sonography, and hepatobiliary scintigraphy in the diagnosis
of sphincter of Oddi dysfunction. Gastrointest Endosc 2001;54:697.
Silverman WB et al: Hybrid classification of sphincter of Oddi
dysfunction based on simplified Milwaukee criteria: effect of marginal
serum liver and pancreas test elevations. Dig Dis Sci 2001;46:278.
Thomas PD et al: Use of (99m)Tc-DISIDA biliary scanning with
provocation for the detection of elevated sphincter of
Oddi basal pressure. Gut 2000;46:838.
Toouli J et al: Manometry based randomized trial
of endoscopic sphincterotomy for sphincter of Oddi dysfunction.
Duodenal diverticula usually arise on the medial aspect of the
duodenum within 2 cm of the orifice of the bile duct, and in some
individuals the duct empties directly into a diverticulum. Even
in the latter circumstance, duodenal diverticula are usually innocuous.
Occasionally, distortion of the duct entrance or obstruction by
enterolith formation in the diverticulum produces symptoms. Either
choledochoduodenostomy or Roux-en-Y choledochojejunostomy is usually
a safer method of reestablishing biliary drainage than attempts
to excise the diverticulum and reimplant the duct.
When the worms invade the duct from the duodenum, ascariasis
can produce symptoms of ductal obstruction. Air may sometimes be
seen within the ducts on plain films. Antibiotics should be used until
cholangitis is controlled, and anthelmintic therapy (mebendazole,
albendazole, or pyrantel pamoate) should then be given. The acute
symptoms usually subside with antibiotics, but if they do not, endoscopic
sphincterotomy should be performed and attempts made to extricate
the worms. If this is unsuccessful and the patient remains acutely
ill, the duct should be emptied surgically.
Cholangitis (Oriental Cholangiohepatitis)
Oriental cholangiohepatitis is a type of chronic recurrent cholangitis
prevalent in coastal areas from Japan to Southeast Asia. In Hong
Kong, it is the third-most common indication for emergency laparotomy
and the most frequent type of biliary disease. The disease is currently thought
to result from chronic portal bacteremia, with portal phlebitis
antedating the biliary disease. E coli causes secondary
infection of the bile ducts, which initiates pigment stone formation
within the ducts.
Biliary obstruction from the stones gives rise to recurrent cholangitis,
which, unlike gallstone disease in Western countries, may be unaccompanied
by gallbladder stones. The gallbladder is usually distended during
an attack and may contain pus.
Chronic recurrent infection often leads to biliary strictures
and hepatic abscess formation. The strictures are usually located in
the intrahepatic bile ducts, and for some unknown reason, the left
lobe of the liver is more severely involved. Intrahepatic gallstones
are common, and their surgical removal may be difficult or impossible.
Acute abdominal pain, chills, and high fever are usually present,
and jaundice develops in about half of cases. Right upper quadrant
tenderness is usually marked, and in about 80% of cases the
gallbladder is palpable. ERCP or THC is the best way to study the
biliary tree and can help in determining the need for surgery and
the type of procedure.
Systemic antibiotics should be given for acute cholangitis. Surgical
treatment consists of cholecystectomy, common duct exploration,
and removal of stones. Sphincteroplasty should also be performed
to allow any residual or recurrent stones to escape from the duct.
A Roux-en-Y choledochojejunostomy is indicated for patients with
strictures, markedly dilated ducts (eg, > 3 cm), or recurrent disease
after a previous sphincteroplasty. The results of surgery are good
in 80% of patients. Chronic intrahepatic stones and infection,
which often involve only one lobe, may require hepatic lobectomy.
Although many patients are cured, prolonged illness from repeated
infection is almost unavoidable once strictures have appeared or
the intrahepatic ducts have become packed with stones.
Cosenza CA et al: Current management of recurrent
pyogenic cholangitis. Am Surg 1999;65:939.
Harris HW et al: Recurrent pyogenic cholangitis. Am J Surg 1998;176:34.
Kim M et al: MR imaging findings in recurrent pyogenic cholangitis.
AJR Am J Roentgenol 1999;173:1545.
Park MS et al: Recurrent pyogenic cholangitis: comparison between
MR cholangiography and direct cholangiography. Radiology 2001;220:677.
Sclerosing cholangitis is a rare chronic disease of unknown cause
characterized by nonbacterial inflammatory narrowing of the bile
ducts. About 60% of cases occur in patients with ulcerative
colitis, and sclerosing cholangitis develops in about 5% of
patients with that disorder. Other less commonly associated conditions
are thyroiditis, retroperitoneal fibrosis, and mediastinal fibrosis.
The disease chiefly affects men 20–50 years of age. In
most cases, the entire biliary tree is affected by the inflammatory
process, which causes irregular partial obliteration of the lumen
of the ducts. The narrowing may be confined, however, to the intrahepatic
or extrahepatic ducts, though it is almost never so short as to
resemble a posttraumatic or focal malignant stricture. The woody-hard
duct walls contain increased collagen and lymphoid elements and
are thickened at the expense of the lumen.
The clinical onset usually consists of the gradual appearance
of mild jaundice and pruritus. Symptoms of bacterial cholangitis
(eg, fever and chills) are uncommon in the absence of previous biliary
surgery. Laboratory findings are typical of cholestasis. The total
serum bilirubin averages about 4 mg/dL and rarely exceeds
10 mg/dL. ERCP is usually diagnostic, demonstrating ductal stenoses
and irregularity, which often gives a beaded appearance. Liver biopsy may
show pericholangitis and bile stasis, but the changes are nonspecific.
The complications of sclerosing cholangitis include gallstone
disease and adenocarcinoma of the bile duct. The latter is most
common in patients with ulcerative colitis. Furthermore, patients
with ulcerative colitis and sclerosing cholangitis appear to be
at greater risk for colonic mucosal dysplasia and colon cancer than
those with ulcerative colitis not associated with sclerosing cholangitis.
Ursodiol (ursodeoxycholic acid), 10 mg/kg/d,
improves liver function tests and symptoms. Cholestyramine will
give relief from pruritus. Percutaneous transhepatic balloon dilatation
can be of value to treat dominant strictures. In cases where the
disease is largely confined to the distal extrahepatic duct and the
proximal ducts are dilated, a Roux-en-Y hepaticojejunostomy
may be indicated. For patients with severe intrahepatic involvement,
hepatic transplantation should be considered.
The natural history of sclerosing cholangitis is one of chronicity
and unpredictable severity. Some patients seem to obtain nearly
complete remission after treatment, but this is not common. Bacterial
cholangitis may develop after operation if adequate drainage has
not been established. In these cases, antibiotics will be required
at intervals. Most patients experience the gradual evolution of
secondary biliary cirrhosis after many years of mild to moderate
jaundice and pruritus. Liver transplantation is indicated when the
disease becomes advanced. The results are good.
Kim WR et al: A revised natural history model for primary sclerosing
cholangitis. Mayo Clin Proc 2000;75:688.
Kubicka S et al: K-ras mutations in the bile of patients with
primary sclerosing cholangitis. Gut 2001;48:403.
Ryder SD, Beckingham IJ: ABC of diseases of liver, pancreas,
and biliary system. Other causes of parenchymal liver disease. BMJ 2001;322:290.
van Hoogstraten HJ et al: Ursodeoxycholic acid therapy for primary sclerosing
cholangitis: results of a 2-year randomized controlled trial to
evaluate single versus multiple daily doses. J Hepatol 1998;29:417.