Biliary Obstruction - 2022
Biliary Obstruction - 2022
Biliary Obstruction - 2022
NCBI Bookshelf. A service of the National Library of Medicine, National Institutes of Health.
Biliary Obstruction
Authors
Affiliations
1 McLaren Health Care
2 University of Tennessee Health Sciences Center
3 University of Tennessee, Chattanooga
4 MSU
Objectives:
Explain the treatment options available for patients with biliary obstruction.
Describe the significance of a coordinated interprofessional team in the early evaluation and management of
patients with biliary obstruction.
Introduction
Biliary obstruction commonly refers to blockage of the bile duct system leading to impaired bile flow from the liver
into the intestinal tract. Bile is a substance that contains bile salts, bilirubin, and cholesterol and is continuously
synthesized in the liver hepatocytes. Bile is then transported via the bile ducts into the second portion of the duodenum
to assist with the metabolism of fats.[1]
Bile formed in the liver flows through the right and left hepatic ducts into the common hepatic duct. The common
hepatic duct then joins the cystic duct arising from the gallbladder to form the common bile duct. The common bile
duct then joins the pancreatic duct through its course within the head of the pancreas before opening into the duodenum
through the major papilla or ampulla. The majority of the bile flows into the gall bladder through the cystic duct, where
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it is concentrated and temporarily stored, while the rest of the bile flows through the common bile duct into the
duodenum through the sphincter of Oddi. The flow of bile into the duodenum is regulated by the release of the
hormone Cholecystokinin (CCK) from the duodenum, which controls the release of stored bile from the gallbladder
and the relaxation of the sphincter of Oddi.
Biliary obstruction is generally referred to as blockage of the extrahepatic biliary system. Biliary obstruction can occur
anywhere along this path and can lead to serious complications such as hepatic dysfunction, renal failure, nutritional
deficiencies, bleeding problems, and infections.[2]
Disruption in the bile flow due to impairment of the intrahepatic biliary system is generally referred to as cholestasis.
Cholestasis can present as abnormalities in serum hepatic enzymes, such as elevated bilirubin and alkaline phosphatase
levels, and can lead to jaundice and pruritis.
Biliary obstruction is common and affects a large portion of the world population, causing significant morbidity and
mortality. The most common etiology of biliary obstruction is choledocholithiasis or gallstones, causing extrahepatic
bile duct blockage. The most serious manifestation of this is the development of infection in the bile ducts called
cholangitis, which can be fatal if not treated promptly.[3]
Etiology
Biliary obstruction can be divided into intrahepatic and extrahepatic etiologies. As described above, intrahepatic biliary
obstruction is generally called cholestasis and is not covered here in detail as it is not the scope of this topic. Briefly,
intrahepatic cholestasis can be from several disease processes, including certain forms of hepatitis (viral infections,
alcohol), drug-induced liver injury (antibiotics, acetaminophen, anti-epileptics, anti-arrhythmic), primary biliary
cholangitis, primary sclerosing cholangitis, and infiltrative diseases (sarcoidosis, tumors, abscess, and cysts).[4][5]
Extrahepatic biliary obstruction, which is the scope of this article, can be divided into various benign and malignant
etiologies. These include choledocholithiasis (gall stones in the common bile duct), choledochal cysts (dilation/cysts of
bile ducts), Mirizzi syndrome (gall stones in cystic duct pressing on bile duct), benign structuring diseases like Primary
sclerosing cholangitis (PSC), fibrotic strictures from gall stone passage or iatrogenic strictures from bile duct
cannulation. Neoplastic stricture diseases causing biliary obstruction can include cholangiocarcinoma (bile duct
cancer), pancreatic head cancer leading to distal common bile duct stricture, and ampullary carcinoma or adenoma.
Other etiologies of biliary obstruction can include infectious diseases like parasitic cholangiopathy (Clonorchis
sinensis, Ascaris lumbricoides) causing intra- or extrahepatic obstruction to bile flow, inflammatory and autoimmune
diseases like AIDS cholangiopathy, and autoimmune cholangiopathy.[6]
Epidemiology
The incidence of gallstones causing biliary obstruction is approximately 5 in 1000 people, whereas 10% to 15% of the
adult U.S. population will develop gallstones in their lifetime.[6] Gallstones or Cholelithiasis, including gall bladder
sludge formation, is the precursor of choledocholithiasis, where gallstones pass through the cystic duct and get lodged
into the common hepatic ducts causing an obstruction. Between 10 and 15% of patients with gallstones are also noted
to have common bile duct stones at diagnosis.[7]
Women are at greater risk of developing gallstones than men and hence increased risk for choledocholithiasis. This is
likely secondary to estrogen increasing hepatic uptake of cholesterol, which increases bile production and some degree
of cholestasis.[8]
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Gallstones are the most common cause of biliary obstruction in Hispanics, Northern Europeans, and Native Americans.
Northern Native Americans have the highest reported rates of cholelithiasis at 64% for females and 29% for males.
Asian and African Americans have an intermediate prevalence of cholelithiasis, with 13.9% in women and 5.3% in
men. The lowest prevalence of cholelithiasis is in sub-Saharan Black Africa, with an overall prevalence of less than
5%. White race North Americans have an overall prevalence of cholelithiasis of 16.6% and 8.6% in females and males,
respectively.[9][8]
In developed countries, choledocholithiasis secondary to cholesterol stones is the most common cause of biliary
obstruction, whereas brown pigment stones from infectious etiologies or hemolysis are the most common etiology in
Asia. Other etiologies like recurrent pyogenic cholangiohepatitis are characterized by recurrent bacterial cholangitis,
dilatation, and stricture of the biliary tree, the presence of calculi within the intrahepatic bile ducts, and an increased
risk for cholangiocarcinoma is common in Asia but rare in the west. Gallbladder malignancy is more common in
Central and South America, Central and Eastern Europe, the northern Indian subcontinent, and East Asia.[10][11][8]
Pathophysiology
1. Catabolism of hemoglobin is a normal function that releases heme molecules, which then converts to biliverdin,
a precursor to bilirubin. Biliverdin then transforms into unconjugated bilirubin within the
reticuloendothelial system. Being that unconjugated bilirubin is lipid-soluble, the renal system does not eliminate
it. Unconjugated bilirubin is bound in serum to albumin or exists as free unbound bilirubin. Unbound bilirubin is
taken up by hepatocytes and converted to conjugated bilirubin, which is water-soluble. Conjugated bilirubin
becomes part of the bile, which is secreted from the hepatocytes in the liver.
2. Bile formed in the liver flows through the right and left hepatic ducts into the common hepatic duct. Fifty percent
of the bile flows into the cystic duct and is then stored in the gallbladder, with the rest of the bile flowing through
the common bile duct and converging and flowing through the main pancreatic duct in the head of the pancreas
to empty into the duodenum through the sphincter of Oddi.
3. Biliary obstruction refers to obstruction in the biliary system, leading to blockage of bile flow into the small
intestine. Biliary obstruction can occur anywhere along the path from the liver to the intestinal tract. Biliary
obstruction is common and affects a large portion of the population around the world, with significant morbidity
and mortality. Gallstones are the most prevalent cause of biliary obstruction; they commonly manifest by
dilatating the common bile duct and jaundice.
4. Jaundice, a physical exam finding of yellowish discoloration of the skin, conjunctiva, and/or mucous
membranes, is a consequence of obstruction leading to bile stasis and buildup of conjugated bilirubin in the
blood.
5. Normal total serum bilirubin values are 0.2 to 1.2 mg/dL. Jaundice is evident clinically at the level of 3 mg/dl.
6. Normal urine contains no bilirubin; however, in patients with obstructive jaundice, conjugated bilirubin is
excreted in the urine, giving it a dark color. Urinary bilirubin is detectable at a lower level of bilirubin than that
needed to cause clinical jaundice.
7. The inability of bilirubin to reach the intestinal tract gives pale color to stools.
8. Pruritus is common in patients with biliary obstruction, but the cause of this is unknown. The deposition of bile
acid into the skin is one postulation that could account for these symptoms. Percutaneous biliary drainage was
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Histopathology
Intrahepatic cholestasis can be diagnosed with a percutaneous or transjugular liver biopsy and is not covered in detail
under this topic.
Extrahepatic biliary obstruction can also lead to chronic changes to liver histology over prolonged obstruction of bile
ducts. However, the etiology of biliary obstruction can be determined through histology from brush cytology or tissue
biopsy samples obtained during various procedures like endoscopic retrograde cholangiopancreatography
(ERCP), percutaneous transhepatic cholangiogram (PTC), or endoscopic ultrasound (EUS) directed biopsy. Sometimes
a tissue sample is imperative for accurate diagnosis, especially for the workup for stricture of the biliary tract noted on
non-invasive imaging.
Biliary obstruction can have very varied presentations depending on the underlying etiology. It usually presents as
jaundice with clay-colored or acholic stools and dark urine. Patients can have pruritis with chronic biliary obstruction.
Patients depending on underlying etiology, can have right upper quadrant abdominal pain, fever, nausea and vomiting,
and weight loss. The onset of these symptoms can be variable ranging from acute development of symptoms to over
months.
A careful history of symptom duration, if gradual or acute onset, accompanying symptoms like weight loss, loss of
appetite, nausea/vomiting is important. Similarly, any right-sided abdominal pain, along with severity and radiation, is
important to know. History of diarrhea, hematochezia, and upper GI bleeding symptoms is also relevant to diagnosing
the underlying process. It is imperative to know any pertinent personal and family history like bile duct or pancreatic
malignancy, inflammatory bowel disease, or primary liver disease. A careful review of social history related to current
and former smoking, alcohol use, and drug abuse is important. Similarly, travel history to parasitic endemic regions is
of value in determining the etiology of biliary obstruction. A careful review of medication history can sometimes be of
great significance in determining intrahepatic cholestasis vs. extrahepatic biliary obstruction.
Physical Examination
A detailed and thorough physical exam is of paramount importance. It is imperative to check baseline vital signs,
especially checking for fever and elevated heart rate. A general physical exam to assess for any distress, pallor, skin
jaundice, scleral icterus, palmer erythema, and signs of malnutrition is important. A detailed abdominal examination to
assess for right upper quadrant tenderness (Murphy's sign), hepatomegaly and splenomegaly, presence of ascites, any
palpable mass, and stigmata of cirrhosis (caput-medusae) is very crucial for diagnosis. A cardiac exam to assess for
signs of congestive heart failure with jugular venous distension and displacement of heart sounds can be very important
to determine underlying etiology. Similarly, a pulmonary exam to assess for pleural effusions, determining whether
one-sided or bilateral, helps determine etiology. Assessment for lymphadenopathy in left supraclavicular lymph nodes
and other neck lymph nodes can provide helpful information. Looking for lower extremity edema is also important.
Evaluation
Blood Tests [13] [14]
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Workup for biliary obstruction includes routine labs and some dedicated labs, including:
Fractionated bilirubin
Gamma-glutamyltransferase
Antinuclear antibody
Coagulation studies
Urine Test
Urine bilirubin
Stool Test
Radiological and Endoscopic tests - Non-invasive and invasive radiologic tests can help diagnose the underlying
etiology of biliary obstruction.[15][16]
Abdominal ultrasound with Doppler can be a starting radiological test due to its low cost, non-invasive test, and
easy availability. Ultrasound is a good test to evaluate gallstones, dilations of extrahepatic bile ducts, and assess
for patency of blood vessels using doppler.
Based on the ultrasound results, an abdominal CT scan can be the next step. This can help assess for pancreatic
head lesions and liver lesions and rule out other intra-abdominal processes.
Nuclear medicine scans like the HIDA scan can assess for cystic duct obstruction.
MRCP (magnetic resonance cholangiopancreatography) is a very sensitive test to assess further intra- and
extrahepatic bile duct abnormalities like cholangiocarcinoma, strictures, or bile duct stones.
Endoscopic tests like EUS (endoscopic ultrasound) with needle aspiration and ERCP with cytology and biopsies
can be further considered based on noninvasive testing results. ERCP can be both diagnostic and therapeutic in
cases of biliary obstruction caused by gallstones.
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Treatment / Management
Initial Management
Initial management depends on the patient's clinical condition and probable etiology. If the patient is stable, most of the
work can be done as an outpatient with regular follow-ups in the gastroenterology clinic. However, if the patient is
febrile and showing signs of sepsis and hepatic dysfunction, admitting the patient for evaluation and treatment may be
necessary. This is needed in patients with acute cholangitis, cholecystitis, and acute liver failure.
Treatment [17]
Treatment varies widely depending on the etiology of biliary obstruction. Some common etiologies and board
management are discussed below.
CBD size less than 1.5cm with small stones- ERCP and sphincterotomy with cholecystectomy
CBD size more than 1.5cm with large stones - ERCP and sphincterotomy with lithotripsy,
choledochotomy, choledochoduodenostomy, choledochojejunostomy, or cholecystectomy
Benign
Malignant
Medication
Albendazole
Mebendazole
Pyrantel pamoate
Surgical/endoscopic:
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In the case of gallbladder invasion, cholecystectomy, CBD exploration, and T-tube placement
Neoplasm [24]
Advanced disease
Resectable disease
Ampullary carcinoma
Whipple procedure
Gallbladder malignancy
Differential Diagnosis
The differential diagnosis for biliary obstruction is very broad, ranging from benign disease to malignant diseases, as
mentioned below.
Acute pancreatitis
Alcoholic hepatitis
Ascariasis
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Choledochal cysts
Chronic pancreatitis
Mirizzi syndrome
Malignant Diseases
Gallbladder cancer
Pancreatic cancer
Prognosis
The prognosis of biliary obstruction is highly dependent on the etiology. If left untreated, it can lead to life-threatening
infection and sepsis. If the obstruction is persistent and chronic, it is usually due to a chronic liver pathology, which
commonly has a poor prognosis. Most acute cases can be successfully managed with medical, surgical, and/or
endoscopic treatment with full recovery. Obstruction caused by chronic liver disease and malignant etiology usually
has a less favorable prognosis.
Complications
Acute Cholangitis
One of the most feared complications of biliary obstruction is acute ascending cholangitis, which is an infection of the
biliary tree due to obstruction, which can quickly lead to systemic sepsis and resultant multi-organ failure.
Acute cholangitis can present as Charcot’s triad (right upper quadrant pain, fever, and jaundice) or Reynold’s pentad
(Charcot's triad with mental status change and hemodynamic shock). Important and helpful laboratory evaluation
includes elevated White blood cell count with leukocytosis, Abnormal liver testing with elevated bilirubin levels,
alkaline phosphatase, and gamma-glutamyl transpeptidase. Elevation of bilirubin is generally in the form of conjugated
hyperbilirubinemia.
Important diagnostic tests include abdominal ultrasound, CT scan of the abdomen, and MRCP.
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Treatment
General Principles
Initial management for acute cholangitis requires admission to the hospital for IVF resuscitation and electrolyte
replacement, pain control, and antibiotics with activity against enteric streptococci, coliforms, and anaerobes are
generally needed.
Drainage ProceduresMost patients with cholangitis will eventually require one of the following:
Endoscopic removal of biliary stones with drainage of bile, often with sphincterotomy, PTC (percutaneous
transhepatic cholangiography)
Insertion of a needle into the biliary tract percutaneously for drainage, removal of stones, and possible placement
of the biliary stent or surgical drainage
Open or laparoscopic surgical intervention with biliary decompression, removal of the obstruction, and
cholecystectomy if warranted and the patient is stable
Consultations
Biliary obstruction can result from various etiologies, which could be complex and considered difficult to access
anatomy; its management may require a multi-specialty approach, including:
Radiology
Interventional radiology
Pathology
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Oncology
Blockage within the biliary tract leads to obstruction of bile flow to the small intestine.
Symptoms
Testing
Further testing will be chosen appropriately depending on the results of the initial testing.
Treatment
Treatment for biliary obstruction depends on the cause but likely will be either surgical or non-surgical,
including endoscopic.
Surgical may include cholecystectomy to remove the gallbladder if the cause is gallstones, along with ERCP
with sphincterotomy to remove any gallstones from the biliary tract causing an obstruction.
Non-surgical may include stabilization and pain control along with medications to help dissolve any gallstones
causing an obstruction.
Patients should eat a low-fat, high-fiber diet. They should avoid fried foods, canned fish, processed meats, full-
fat dairy products, processed baked goods, fast food, and most packaged snack foods. whole fruits and
vegetables high in vitamin C, like kiwi and broccoli. citrus fruits, like orange and grapefruit, may be beneficial.
Prevention of Recurrence
Biliary obstruction encompasses many separate conditions that result in a typical clinical finding of elevated bilirubin.
Patients may first present to their primary care physicians, nurse practitioners, or an emergency department for
treatment. A prompt initial diagnosis helps drive therapy and improve outcomes in most patients. Special attention is
necessary for timely diagnosis of infection of the bile ducts where patients present with fever, sepsis, and biliary
obstruction findings, which can lead to poor prognosis. Initial laboratory and radiographic assessment will determine
the proper specialty needed to treat the obstruction, whether general surgery, gastroenterology, oncology, or
interventional radiology.
Management of biliary obstruction requires the efforts of an interprofessional healthcare team comprised of clinicians
(including PAs and NPs), specialists, nurses, and pharmacists, all collaborating and communicating across disciplinary
boundaries to achieve improved patient outcomes. [Level 5]
Review Questions
Access free multiple choice questions on this topic.
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Disclosure: Ethan Coucke declares no relevant financial relationships with ineligible companies.
Disclosure: Hina Akbar declares no relevant financial relationships with ineligible companies.
Disclosure: Arslan Kahloon declares no relevant financial relationships with ineligible companies.
Disclosure: Peter Lopez declares no relevant financial relationships with ineligible companies.
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