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Cholelythiasis
1. Cholelythiasis. Clinical picture. Methods of patient examination.
2. Complications of cholelythiasis (mechanical, infectious, degenerative).
Treatment.
3. Mechanical jaundice. Differential diagnosis. Treatment.
Cholelithiasis is the formation of gallstones, which are composed of cholesterol,
calcium salts, and bile pigments
A gallstone is a crystalline concretion formed within the gallbladder by accretion of
bile components. Presence of stones in the gallbladder is referred to as cholelithiasis
(from the Greek: chol-, "bile" + lith-, "stone" + iasis-, "process").
Characteristics and composition
Gallstones can vary in size from as small as a grain of sand to as large as a golf ball.
The gallbladder may contain a single large stone or many smaller ones. Pseudoliths,
sometime referred to as sludge, are thick secretions that may be present within the
gallbladder, either alone or in conjunction with fully formed gallstones. The clinical
presentation is similar to that of cholelithiasis. The composition of gallstones is affected
by age, diet and ethnicity. On the basis of their composition, gallstones can be divided
into the following types:
Cholesterol stones
Cholesterol stones vary in color from light-yellow to dark-green or brown and are oval
2 to 3 cm in length, often having a tiny dark central spot. To be classified as such, they
must be at least 80% cholesterol by weight (or 70%, according to the Japanese
classification system).
Pigment stones are small, dark stones made of bilirubin and calcium salts that are found
in bile. They contain less than 20% of cholesterol (or 30%, according to the Japanese
classification system).
Mixed stones
Mixed gallstones typically contain 20–80% cholesterol (or 30–70%, according to the
Japanese classification system). Other common constituents are calcium carbonate,
palmitate phosphate, bilirubin, and other bile pigments. Because of their calcium
content, they are often radiographically visible
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Cholelithiasis - Causes, Symptoms And Treatment
Diseases of the gallbladder and biliary tract are common and, in many cases, painful
conditions that may be life threatening and usually require surgery. They are generally
associated with deposition of calculi and inflammation.
Cholelithiasis is the fifth leading cause of hospitalization among adults and accounts for
90% of all gallbladder and duct diseases. Women have two to three times the incidence
as men of developing cholelithiasis. The disease may also be more prevalent in persons
who are obese, who have high cholesterol, or who are on cholesterol lowering drugs.
The prognosis is usually good with treatment unless infection occurs, in which case
prognosis depends on its severity and response to antibiotics.
In most cases, gallbladder and bile duct diseases occur during middle age. Between ages
20 and 50, they're six times more common in women, but incidence in men and women
becomes equal after age 50. Incidence rises with each succeeding decade.
Causes of Cholelithiasis
Cholelithiasis stones or calculi (gallstones) in the gallbladder. results from changes in
bile components. Gallstones are made of cholesterol, caldurn bilirubinate, or a mixture
of cholesterol and bilirubin pigment. They arise during periods of sluggishness in the
gallbladder due to pregnancy. hormonal contraceptives. diabetes mellitus. celiac
disease, cirrhosis of the liver, and pancreatitis.
One out of every 10 patients with gallstones develops Cholelithiasis, or gallstones in the
common bile duct (sometimes called common duct stones). This condition occurs when
stones pass out of the gallbladder and lodge in the hepatic and common bile ducts.
obstructing the flow of bile into the duodenum. Prognosis is good unless infection
occurs.
Cholangitis, infection of the bile duct, is commonly associated with choledocholithiasis
and may follow percutaneous transhepatic cholangiography or occlusion of
endoscopicstents. Predisposing factors may include bacterial or metabolic alteration of
bile acids. Widespread inflammation may cause fibrosis and stenosis of the common
bile duct. The prognosis for this rare condition is poor without stenting or surgery.
Cholecystitis. acute or chronic inflammation of the gallbladder. is usually associated
with a gallstone impacted in the cystic duct, causing painful distention of the
gallbladder. Cholecystitis accounts for 10% to 25% of all patients requiring gallbladder
surgery. The acute form is most common during middle age; the chronic form occurs
most commonly among the elderly. The prognosis is good with treatment.
Cholesterolosis. polyps or crystal deposits of cholesterol in the gallbladder's submucosa,
may result from bile secretions containing high concentrations of cholesterol and
insufficient bile salts. The polyps may be localized or speckle the entire gallbladder.
Cholesterolosis the most common pseudotumor. isn't related to widespread
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inflammation of the mucosa or lining of the gallbladder. The prognosis is good with
surgery.
Biliary cirrhosis. ascending infection of the biliary system, sometimes follows viral
destruction of liver and duct cells. but the primary cause is unknown. This condition
usually leads to obstructive jaundice and involves the portal and periportal spaces of the
liver. It's nine times more common among women ages 40 to 60 than among men. The
prognosis is poor without liver transplantation.
Gallstone ileus results from a gallstone lodging at the terminal ileum; it's more common
in the elderly. The prognosis is good with surgery.
Postcholecystectomy syndrome commonly results from residual gal1stones or stricture
of the common bile duct. It occurs in 1 % to 5 % of all patients whose gallbladders have
been surgical1y removed and may produce right upper quadrant abdominal pain, biliary
colic, fatty food intolerance, dyspepsia. and indigestion. The prognosis is good with
selected
radiologic
procedures, endoscopic procedures, or surgery.
Acalculous cholecystitis is more common in critical1y ill patients, accounting for about
5% of cholecystitis cases. It may result from primary infection with such organisms as
Salmollella typhi. Escherichia coli, or Clostridium or from obstruction of the cystic duct
due to lymphadenopathy or a tumor. It appears that ischemia usually related to a low
cardiac output. also has a role in the pathophysiology of this disease. Signs and
symptoms of acalculous cholecystitis include unexplained sepsis, right upper quadrant
pain, fever, leukocytosis, and a palpable gallbladder.
Cholelithiasis Symptoms and Signs
Although gallbladder disease may produce no symptoms. acute cholelithiasis, acute
cholecystitis, choledocholithiasis. and cholesterolosis produce the symptoms of a classic
gallbladder attack. Attacks commonly follow meals rich in fats or may occur at night.
suddenly awakening the patient. They begin with acute abdominal pain in the right
upper quadrant that may radiate to the back. between the shoulders. or to the front of the
chest; the pain may be so severe that the patient seeks emergency department care.
Other features may include recurring fat intolerance. biliary colic. belching. flatulence,
indigestion. diaphoresis. nausea. vomiting. chills. low-grade fever. jaundice (if a stone
obstructs the common bile duct). and clay-colored stools (with choledocholithiasis).
Clinical features of cholangitis include a rise in eosinophils, jaundice, abdominal pain.
high fever. and chills; biliary drrhosis may produce jaundice, related itching, weakness,
fatigue. slight weight loss. and abdominal pain. Gallstone ileus produces signs and
symptoms of small bowel obstruction - nausea. vomiting, abdominal distention, and
absent bowel sounds if the bowel is completely obstructed. Its most telling symptom is
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intermittent recurrence of colicky pain over several days. Each of these disorders
produces its own set of complications.
Diagnosis and testing information
Differential diagnosis is essential in gallbladder and biliary tract disease because
gallbladder disease can mimic other diseases (myocardial infarction. angina.
pancreatitis. pancreatic head cancer. pneumonia, peptic ulcer, hiatal henda, esophagitis.
and gastritis). Serum amylase distinguishes gallbladder disease from pancreatitis. With
suspected heart disease. serial cardiac enzyme tests and electrocardiogram should
precede gallbladder and upper GI diagnostic tests. Tests used to diagnose gallbladder
and biliary tract disease include:
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Ultrasound reflects stones in the gallbladder with 96% accuracy. It's also
considered the primary tool for diagnosing cholelithiasis.
Percutaneous trashepatic cholangiography. done under fluoroscopic control.
distinguishes between gallbladder or bile duct disease and cancer of the
pancreatic head in patients with jaundice.
Endoscopic retrograde cholangiopancreatography (ERCP) visualizes the biliary
tree after insertion of an endoscope down the esophagus into the duodenum,
cannulation of the common bile and pancreatic ducts, and injection of contrast
medium.
HIDA scan of the gallbladder detects obstruction of the cystic duct.
Computed tomography scan, although not used routinely, helps distinguish
between obstructive and non obstructive jaundice.
Flat plate of the abdomen identifies calcified, but not cholesterol. stones with
15% accuracy.
Oral cholecystography, which is rarely used, shows stones in the gallbladder and
biliary duct obstruction.
Elevated icteric index, total bilirubin, urine bilirubin, and alkaline phosphatase support
the diagnosis. White blood cell count is slightly elevated during a cholecystitis attack.
Cholelithiasis treatment
Cholecystectomy (gallbladder removal) has a 99% chance of eliminating the recurrence
of cholelithiasis. Only symptomatic patients must be indicated to surgery. The lack of a
gallbladder may have no negative consequences in many people. However, there is a
portion of the population — between 10 and 15% — who develop a condition called
postcholecystectomy syndrome which may cause gastrointestinal distress and persistent
pain in the upper-right abdomen, as well as a 10% chance of developing chronic
diarrhea.
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Surgery, usually elective, is the treatment of choice for gallbladder and biliary tract
diseases and may include open or laparoscopic cholecystectomy, cholecystectomy with
operative cholangiography and, possibly, exploration of the common bile duct.
There are two surgical options for cholecystectomy:
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Open cholecystectomy: This procedure is performed via an incision into the
abdomen (laparotomy) below the right lower ribs. Recovery typically consists of
3–5 days of hospitalization, with a return to normal diet a week after release and
normal activity several weeks after release.
Laparoscopic cholecystectomy: This procedure, introduced in the 1980s, is performed
via three to four small puncture holes for a camera and instruments. Post-operative care
typically includes a same-day release or a one night hospital stay, followed by a few
days of home rest and pain medication. Laparoscopic cholecystectomy patients can, in
general, resume normal diet and light activity a week after release, with some decreased
energy level and minor residual pain continuing for a month or two. Studies have shown
that this procedure is as effective as the more invasive open cholecystectomy, provided
the stones are accurately located by cholangiogram prior to the procedure so that they
can all be removed.
Obstruction of the common bile duct with gallstones can sometimes be relieved by
endoscopic retrograde sphincterotomy (ERS) following endoscopic retrograde
cholangiopancreatography (ERCP).
Other treatments include a low-fat diet to prevent attacks and vitamin K for itching,
jaundice, and bleeding tendendes due to vitamin K deficiency. Treatment during an
acute attack may include insertion of a nasogastric tube and an I.V.line and, possibly,
antibiotic and analgesic administration. A non surgical treatment for choledocholithiasis
involves placement of a catheter through the percutaneous transhepatic
cholangiographic route. Guided by fluoroscopy, the catheter is directed toward the
stone. A basket is threaded through the catheter,opened, twirled to entrap the stone,
closed, and withdrawn. This procedure can be performed endoscopically.
Ursodiol (Actigall), which dissolves radiolucent stones, provides an alternative for
patients who are poor surgical risks or who refuse surgery. however, use of urdodiol is
limited by the need for prolonged treatment, the high incidence of adverse effects, and
the frequency of stone formation after the treatment ends.
Extra corporeal shock wave lithotrillsy (ESWL) has also been adapted for the treatment
of gallstones. ESWL Is a non surgical procedure used to ('rush stones inside the
gallbladder. Gallstones can be broken up using a procedure called extracorporeal shock
wave lithotripsy (often simply called "lithotripsy"), which is a method of concentrating
ultrasonic shock waves into the stones to break them into tiny pieces that can pass out
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of the gallbladder through the cystic duct and common bile duct into
the small intestine.
Jaundice - (jôn`dĭs, jän`–), abnormal condition in which the body fluids and tissues,
particularly the skin and eyes, take on a yellowish color as a result of an excess of
bilirubin. During the normal breakdown of old erythrocytes (red blood cells), their
hemoglobin is converted into bilirubin. Normally the bilirubin is removed from the
bloodstream by the liver, which passes from the liver into the intestines. There are
several conditions that may interrupt the elimination of bilirubin from the blood and
cause jaundice.
Prehepatic jaundice is caused by an increased content of free bilirubin circulating in
the blood, formed as a result of increased decomposition of the erythrocytes (hemolytic
jaundice), or of congenital or acquired deficiency of enzymes that participate in binding
bilirubin with glucuronic acid. Hemolytic jaundice appears in hemolytic disease of the
new-born and in poisoning with hemolytic toxins; it is characterized by increased
excretion of the products of bilirubin metabolism in the urine (urobilin) and feces
(stercobilin, which causes the saturated pigmentation of the feces). Other types of
jaundice caused by disruption in the capture and bonding of bilirubin and proceeding
without substantive affection of liver cells are physiological jaundice of the new-born,
nuclear jaundice, and juvenile jaundice.
Hepatogenic jaundice (parenchymatous jaundice) is a function of organic (infectious,
parasitic, or toxic) affection of the liver itself and is conditioned by the formation of an
anastomosis between blood and bile capillaries and also by intrahepatic stasis of bile
during inflammations of the liver. There appear, along with other symptoms of liver
affection, a saturated pigmentation of the urine and faintly colored feces.
Posthepatic, or, Mechanical biliary obstruction. Such obstruction may have a
malignant or benign origin. Choledocholithiasis is found in about 15% of patients with
gallbladder stones. The clinical presentation ranges from mild right upper quadrant pain
with minimal elevation of liver enzymes to ascending cholangitis. Ultrasonography is as
sensitive as CT for the detection of choledocholithiasis. ERCP is also highly accurate in
the diagnosis of biliary obstruction, with a sensitivity of 89% to 98% and a specificity
of 89% to 100%. ERCP can also be used for therapeutic interventions, including stone
removal and endobiliary stent placement.
Biliary obstruction may also be caused by parasitic infections (as with Ascaris
lumbricoides); on endoscopy, the organisms can sometimes be seen protruding from the
ampulla.
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Malignant causes include carcinoma of the pancreas or gallbladder, ampullary
carcinoma, and cholangiocarcinoma. ERCP facilitates procedures such as diagnostic
brushings or biopsy in cholangiocarcinoma and ampullary cancer, as well as the
placement of an endobiliary stent to temporarily relieve the obstruction. Jaundice in
PSC is typically a result of end-stage liver disease; however, occasionally a dominant
nonmalignant extrahepatic biliary stricture is the cause, and this condition is treatable
with endobiliary stent placement.
In a number of instances, a yellow coloring of the skin and other body tissues may be
caused by pigments in food (for example, the carotene contained in carrots) or
medications (acrichin).
Choledocholithiasis
If gallstones migrate into the ducts of the biliary tract, the condition is referred to as
choledocholithiasis (from the Greek: chol-, "bile" + docho-, "duct" + lith-, "stone" +
iasis-, "process").
Signs and symptoms
A positive Murphy's sign is a common finding on physical examination. Jaundice of the
skin or eyes is an important physical finding in biliary obstruction. Jaundice and/or
clay-colored stool may raise suspicion of choledocholithiasis or even gallstone
pancreatitis. If the above symptoms coincide with fever and chills, the diagnosis of
ascending cholangitis may also be considered.
Causes
While stones can frequently pass through the common bile duct (CBD) into the
duodenum, some stones may be too large to pass through the CBD and may cause an
obstruction. One risk factor for this is duodenal diverticulum.
Pathophysiology
This obstruction may lead to jaundice, elevation in alkaline phosphatase, increase in
conjugated bilirubin in the blood and increase in cholesterol in the blood. It can also
cause acute pancreatitis and ascending cholangitis.
Diagnosis
Common bile duct stone impacted at ampulla of Vater seen at time of ERCP
Choledocholithiasis (stones in common bile duct) is one of the complications of
cholelithiasis (gallstones), so the initial step is to confirm the diagnosis of cholelithiasis.
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Patients with cholelithiasis typically present with pain in the right-upper quadrant of the
abdomen with the associated symptoms of nausea and vomiting, especially after a fatty
meal. The physician can confirm the diagnosis of cholelithiasis with an abdominal
ultrasound that shows the ultrasonic shadows of the stones in the gallbladder.
The diagnosis of choledocholithiasis is suggested when the liver function blood test
shows an elevation in bilirubin. The diagnosis is confirmed with either an Magnetic
resonance cholangiopancreatography (MRCP), an ERCP, or an intraoperative
cholangiogram. If the patient must have the gallbladder removed for gallstones, the
surgeon may choose to proceed with the surgery, and obtain a cholangiogram during the
surgery. If the cholangiogram shows a stone in the bile duct, the surgeon may attempt to
treat the problem by flushing the stone into the intestine or retrieve the stone back
through the cystic duct.
On a different pathway, the physician may choose to proceed with ERCP before
surgery. The benefit of ERCP is that it can be utilized not just to diagnose, but also to
treat the problem. During ERCP the endoscopist may surgically widen the opening into
the bile duct and remove the stone through that opening. ERCP, however, is an invasive
procedure and has its own potential complications. Thus, if the suspicion is low, the
physician may choose to confirm the diagnosis with MRCP, a non-invasive imaging
technique, before proceeding with ERCP or surgery.
Treatment
Treatment involves removing the stone using ERCP. Typically, the gallbladder is then
removed, an operation called cholecystectomy, to prevent a future occurrence of
common bile duct obstruction or other complications
CHOLANGITIS
acute ascending cholangitis (from the Greek: chol-, "bile + ang-, "vessel" + itis-,
"inflammation"), a serious infection of the bile ducts.
Cholangitis is inflammation of the bile duct. The most common cause is a bacterial
infection, and the problem is then an ascending cholangitis. However, there are other
types of cholangitis as well.
Types of cholangitis
Ascending cholangitis
Ascending cholangitis is caused by bacteria, and causes pain, jaundice, and fever. The
bacteria usually originate from a preceding choledocholithiasis and is ascending from
the intestines. Typical bacteria is; - Aerobic: E. Coli, Klebsiella, Pseudomonas (post
ERCP), streptococcus faecalis and Enterobacter - Anaerobic: Bacteroides fragilis,
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Clostridium perfringens. Both aerobic and anaerobic infection can be present at the
same time. Gallstones obstructing the common bile duct are a risk factor for developing
ascending cholangitis.
Primary sclerosing cholangitis
Primary sclerosing cholangitis is a cholangitis with autoimmune origin. Primary
sclerosing cholangitis (PSC) is a chronic liver disease caused by progressive
inflammation and scarring of the bile ducts of the liver. The inflammation impedes the
flow of bile to the gut, which can ultimately lead to liver cirrhosis, liver failure and liver
cancer. The underlying cause of the inflammation is believed to be autoimmunity; and
more than 80% of those with PSC have ulcerative colitis. The definitive treatment is
liver transplantation.
Secondary sclerosing cholangitis
Secondary sclerosing cholangitis is a cholangitis with other causes.
Recurrent pyogenic cholangitis
Recurrent pyogenic cholangitis (a.k.a. cholangiohepatitis) is a condition that most
commonly affects humans of East Asian origins. It is characterized by recurrent bouts
of cholangitis in association with intrahepatic pigment stones and intrahepatic biliary
obstruction. It is also a common liver disease in cats.
PATHOPHYSIOLOGY
Acute inflammation of the biliary tree secondary to obstruction, complicated by
infection,and is a surgical emergency being potentially lethal
Obstruction, increased pressure, bacterial infection from duodenum, lymphatics, or
portal vein
Incomplete obstruction more common than complete obstruction
Sepsis from increased biliary tree pressure pushes bugs into portal vein
Bugs: Ecoli, Klebsiella, Proteus, Enterococcus
Etiology
CBD stone
Stricture: post op, sclerosing cholangitis
Signs and symptoms
A person with cholangitis may complain of abdominal pain (particularly in the right
upper quadrant of the abdomen), fever, rigors (uncontrollable shaking) and a feeling of
uneasiness (malaise). Some may report jaundice (yellow discoloration of the skin and
the whites of the eyes).
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Physical examination findings typically include jaundice and right upper quadrant
tenderness. Charcot's triad is a set of three common findings in cholangitis: abdominal
pain, jaundice, and fever. This was assumed in the past to be present in 50–70% of
cases, although more recently the frequency has been reported as 15–20%. Reynolds'
pentad includes the findings of Charcot's triad with the presence of septic shock and
mental confusion. This combination of symptoms indicates worsening of the condition
and the development of septicemia, and is seen less commonly still.
In the elderly, the presentation may be atypical; they may directly collapse due to
septicemia without first showing typical features. Those with an indwelling stent in the
bile duct (see below) may not develop jaundice.
Diagnosis
Blood tests
Routine blood tests show features of acute inflammation (raised white blood cell count
and elevated C-reactive protein level), and usually abnormal liver function tests (LFTs).
In most cases the LFTs will be consistent with obstruction: raised bilirubin, alkaline
phosphatase and γ-glutamyl transpeptidase. In the early stages, however, pressure on
the liver cells may be the main feature and the tests will resemble those in hepatitis,
with elevations in alanine transaminase and aspartate transaminase.[1]
Blood cultures are often performed in people with fever and evidence of acute infection.
These yield the bacteria causing the infection in 36% of cases,[6] usually after 24–48
hours of incubation. Bile, too, may be sent for culture during ERCP (see below). The
most common bacteria linked to ascending cholangitis are gram-negative bacilli:
Escherichia coli (25–50%), Klebsiella (15–20%) and Enterobacter (5–10%). Of the
gram-positive cocci, Enterococcus causes 10–20%. A small proportion of cases,
especially in the elderly and those who have undergone previous surgery of the biliary
system, is due to anaerobic organisms such as Clostridium and Bacteroides. In the
developing world, cholangitis may also be caused by parasites such as Ascaris
lumbricoides and Clonorchis sinensis. In people with AIDS, a large number of
opportunistic organisms has been known to cause AIDS cholangiopathy, but the risk has
rapidly diminished since the introduction of effective AIDS treatment.[1]
Medical imaging
Cholangiogram through a nasobiliary drain showing the common bile duct in black
(diagonally from top left to bottom right in the center) with an interruption in the
contour due to a large gallstone.
Given that ascending cholangitis usually occurs in the setting of bile duct obstruction,
various forms of medical imaging may be employed to identify the site and nature of
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this obstruction. The first investigation is usually ultrasound, as this is the most easily
available.[1] Ultrasound may show dilation of the bile duct and identifies 38% of bile
duct stones; it is relatively poor at identifying stones further down the bile duct.
Ultrasound can help distinguish between cholangitis and cholecystitis (inflammation of
the gallbladder), which has similar symptoms to cholangitis but appears differently on
ultrasound. A better test is magnetic resonance cholangiopancreatography (MRCP),
which uses magnetic resonance imaging (MRI); this has a comparable sensitivity to
ERCP. Smaller stones, however, can still be missed on MRCP depending on the quality
of the hospital's facilities.
The gold standard (best possible) test for biliary obstruction is still endoscopic
retrograde cholangiopancreatography (ERCP). This involves the use of endoscopy
(passing a tube through the mouth into the esophagus, stomach and thence to the
duodenum) to pass a small cannula into the bile duct. At that point, radiocontrast is
injected to opacify the duct, and X-rays are taken to get a visual impression of the
biliary system. On the endoscopic image of the ampulla, one can sometimes see a
protuberant ampulla from an impacted gallstone in the common bile duct, or the frank
extrusion of pus from the common bile duct orifice. On the X-ray images (known as
cholangiograms), gallstones are visible as nonopacified areas in the contour of the duct.
For diagnostic purposes, ERCP has now generally been replaced by MRCP. ERCP is
only used first-line in critically ill patients in whom delay for diagnostic tests is not
acceptable; however, if the index of suspicion for cholangitis is high, an ERCP is
typically done to achieve drainage of the obstructed common bile duct.[1]
If other causes rather than gallstones are suspected (such as a tumor), computed
tomography and endoscopic ultrasound (EUS) may be performed to identify the nature
of the obstruction. EUS may be used to obtain biopsy (tissue sample) of suspicious
masses.[1] EUS may also replace diagnostic ERCP for stone disease, although this
depends on local availability.
Causes
Bile duct obstruction, which is usually present in acute cholangitis, is generally due to
gallstones. 10–30% of cases, however, are due to other causes such as benign stricturing
(narrowing of the bile duct without an underlying tumor), postoperative damage or an
altered structure of the bile ducts such as narrowing at the site of an anastomosis
(surgical connection) and various tumors (cancer of the bile duct, gallbladder cancer,
cancer of the ampulla of Vater, pancreatic cancer or cancer of the duodenum).[8]
Cholangitis may also complicate medical procedures involving the bile duct, especially
ERCP. To prevent this, it is recommended that those undergoing ERCP for any
indication receive prophylactic (preventative) antibiotics.[3]
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The presence of a permanent biliary stent (e.g. in pancreatic cancer) slightly increases
the risk of cholangitis, but stents of this type are often needed to keep the bile duct
patent under outside pressure.
Pathogenesis
Bile is produced by the liver, and serves to eliminate cholesterol and bilirubin from the
body, as well as emulsifying of fats to make them more soluble in water and aid in their
digestion. Bile is formed in the liver by hepatocytes (liver cells) and excreted into the
common hepatic duct. Part of the bile is stored in the gall bladder because of back
pressure (exerted by the sphincter of Oddi), and may be released at time of digestion.
The gall bladder also concentrates the bile by absorbing water and dissolved salts from
it. All bile reaches the duodenum (first part of the small intestine) through the common
bile duct and the ampulla of Vater. The sphincter of Oddi, located at the junction of the
ampulla of Vater and the duodenum, is a circular muscle that controls the release of
both bile and pancreatic secretions into the digestive tract.
The biliary tree is normally relatively free of bacteria because of certain protective
mechanisms. The sphincter of Oddi acts as a mechanical barrier. The biliary system
normally has low pressure (8 to 12 cmH2O)[9] and allows bile to flow freely through.
The continuous forward flow of the bile in the duct flushes bacteria, if present, into the
duodenum, and does not allow establishment of an infection. The constitution of bile—
bile salts and immunoglobulin[2] secreted by the epithelium of the bile duct also has a
protective role.
Bacterial contamination alone in absence of obstruction does not usually result in
cholangitis. However increased pressure within the biliary system (above 20 cmH2O)
resulting from obstruction in the bile duct widens spaces between the cells lining the
duct, bringing bacterially contaminated bile in contact with the blood stream. It also
adversely affects the function of Kupffer cells, which are specialized macrophage cells
that assist in preventing bacteria from entering the biliary system. Finally, increased
biliary pressure decreases production of IgA immunoglobulins in the bile. This results
in bacteremia (bacteria in the blood stream) and gives rise to the systemic inflammatory
response syndrome (SIRS) comprising fever (often with rigors), tachycardia, increased
respiratory rate and increased white blood cell count; SIRS in the presence of suspected
or confirmed infection is called sepsis.[1] Biliary obstruction itself disadvantages the
immune system and impairs its capability to fight infection, by impairing the function
of certain immune system cells (neutrophil granulocytes) and modifying the levels of
immune hormones (cytokines).
In ascending cholangitis, it is assumed that organisms migrate backwards up the bile
duct as a result of partial obstruction and decreased function of the sphincter of Oddi. [1]
Other theories about the origin of the bacteria, such as through the portal vein or
transmigration from the colon, are considered less likely.
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Treatment
Fluids and antibiotics
Cholangitis requires admission to hospital. Intravenous fluids are administered,
especially if the blood pressure is low, and antibiotics are commenced. Empirical
treatment with broad-spectrum antibiotics is usually necessary until it is known for
certain which pathogen is causing the infection, and to which antibiotics it is sensitive.
Combinations of penicillins and aminoglycosides are widely used, although
ciprofloxacin has been shown to be effective in most cases, and may be preferred to
aminoglycosides because of fewer side effects. Metronidazole is often added to
specifically treat the anaerobic pathogens, especially in those who are very ill or at risk
of anaerobic infections. Antibiotics are continued for 7–10 days. Drugs that increase the
blood pressure (vasopressors) may also be required to counter the low blood pressure.[2]
Endoscopy
The definitive treatment for cholangitis is relief of the underlying biliary obstruction.[1]
This is usually deferred until 24–48 hours after admission, when the patient is stable
and has shown some improvement with antibiotics, but may need to happen as an
emergency in case of ongoing deterioration despite adequate treatment, or if antibiotics
are not effective in reducing the signs of infection (which happens in 15% of cases).
Endoscopic retrograde cholangiopancreatography (ERCP) is the most common
approach in unblocking the bile duct. This involves endoscopy (passing a fiberoptic
tube through the stomach into the duodenum), identification of the ampulla of Vater and
insertion of a small tube into the bile duct. A sphincterotomy (making a cut in the
sphincter of Oddi) is typically done to ease the flow of bile from the duct and to allow
insertion of instruments to extract gallstones that are obstructing the common bile duct;
alternatively or additionally, the common bile duct orifice can be dilated with a balloon.
Stones may be removed either by direct suction or by using various instruments,
including balloons and baskets to trawl the bile duct in order to pull stones into the
duodenum. Obstructions that are caused by larger stones may require the use of an
instrument known as a mechanical lithotriptor in order to crush the stone prior to
removal. Obstructing stones that are too large to be removed or broken mechanically by
ERCP may be managed by extracorporeal shock wave lithotripsy. This technique uses
acoustic shock waves administered outside the body to break down the stones. [14] An
alternative technique to remove very large obstructing stones is electrohydraulic
lithotripsy, where a small endoscope known as a cholangioscope is inserted by ERCP to
directly visualize the stone. A probe uses electricity to generate shock waves that break
down the obstructing stone. Rarely, surgical exploration of the common bile duct
(termed choledochotomy), which can be performed with laparoscopy, is required to
remove the stone.
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Narrowed areas may be bridged by a stent, a hollow tube that keeps the duct open.
Removable plastic stents are used in uncomplicated gallstone disease, while permanent
self-expanding metal stents with a longer lifespan are used if the obstruction is due to
pressure from a tumor such as pancreatic cancer. A nasobiliary drain may be left
behind; this is a plastic tube that passes from the bile duct through the stomach and the
nose and allows continuous drainage of bile into a receptible. It is similar to a
nasogastric tube, but passes into the common bile duct directly, and allows for serial xray cholangiograms to be done to identify the improvement of the obstruction. The
decision on which of the aforementioned treatments to apply is generally based on the
severity of the obstruction, findings on other imaging studies, and whether the patient
has improved with antibiotic treatment. Certain treatments may be unsafe if blood
clotting is impaired, as the risk of bleeding (especially from sphincterotomy) is
increased in the use of medication such as clopidogrel (which inhibits platelet
aggregation) or if the prothrombin time is significantly prolonged. For a prolonged
prothrombin time, vitamin K or fresh frozen plasma may be administered to reduce
bleeding risk.
It may be difficult to obtain endoscopic access to the obstruction located higher
(proximal) up in the biliary system, or when it is due to a stricture in the priorly
performed anastomosis between the bile duct (surgically joining) with the duodenum or
jejunum.[2] When this happens, percutaneous transhepatic cholangiography (PTC) may
be needed to relieve pressure. This involves identifying the bile duct by ultrasound and
then passing a tube through the skin (percutaneous).[3] PTC is generally performed by
radiologists. PTC has potential complications, so occasionally further attempts at ERCP
by more experienced doctors are preferred.
Continual contamination of bile duct by indwelling stents (as may occur in chronic
conditions like tumor of the head of pancreas) requires monitoring by repeated
radiologic tests and changing of the stents.
Cholecystectomy
Not all gallstones implicated in ascending cholangitis actually originate from the
gallbladder, but cholecystectomy (surgical removal of the gallbladder) is generally
recommended in people who have been treated for cholangitis due to gallstone disease.
This is typically delayed until all symptoms have resolved and ERCP or MRCP have
confirmed that the bile duct is clear of gallstones.[1][2][3] Those who do not undergo
cholecystectomy have an increased risk of recurrent biliary pain, jaundice, further
episodes of cholangitis, and need for further ERCP or related procedures; the risk of
death is also significantly increased.
Prognosis
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Acute cholangitis carries a significant risk of death, the leading cause being irreversible
shock with multiple organ failure (a possible complication of severe infections).[8]
Improvements in diagnosis and treatment have led to a reduction in mortality: before
1980, the mortality rate was greater than 50%, but after 1980 it was 10–30%.[8] Patients
with signs of multiple organ failure are likely to die unless they undergo early biliary
drainage and treatment with systemic antibiotics. Other causes of death following
severe cholangitis include heart failure and pneumonia.
Risk factors indicating an increased risk of death include older age, female gender, a
history of liver cirrhosis, biliary narrowing due to cancer, acute renal failure and the
presence of liver abscesses. Complications following severe cholangitis include renal
failure, respiratory failure (inability of the respiratory system to oxygenate blood and/or
eliminate carbon dioxide), cardiac arrythmia, wound infection, pneumonia,
gastrointestinal bleeding and myocardial ischemia (lack of blood flow to the heart,
leading to heart attacks).
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