Biliary Obstruction

Article Author:
Ethan Coucke
Article Author:
Hina Akbar
Article Author:
Arslan Kahloon
Article Editor:
Peter Lopez
Updated:
6/22/2020 5:15:58 PM
PubMed Link:
Biliary Obstruction

Introduction

Biliary obstruction refers to blockage of the bile duct system preventing bile from flowing from the liver into the intestinal tract. Bile is synthesized in the liver and transported via the bile ducts into the duodenum to assist with the metabolism of fats. Bile is a substance produced continuously by the hepatocytes consisting of bile salts, bilirubin, fats, water, and inorganic salts. Bile formed in the liver flows through the right and left hepatic ducts into the common hepatic duct. Nearly 50% 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. Biliary obstruction can occur anywhere along this path and lead to serious complications such as hepatic dysfunction, renal failure, cardiovascular impairment, nutritional deficiencies, bleeding problems, and infection.[1]

Biliary obstruction is generally referred to blockage of extrahepatic biliary system. Disruption in bile flow due to hepatic or intrahepatic biliary system is genrally referred as cholestasis. All these can present as elevated bilirubin levels and Jaundice. 

Bile obstruction is common and affects a large portion of the population around the world with significant morbidity and mortality. The most common etiology of biliary obstruction is from Choledhocholithiasis or gallstone causing extrahepatic bile duct blockage. The most serious manifestation of this is infection of bile ducts or cholangitis which can be fatal if not treated promptly. [2] 

Etiology

Biliary obstruction can divided into intrahepatic and extrahepatic etiologies. As described above, intrahepatic biliary obstruction is generally called cholestasis and not covered here in detail as is not the scope of this topic.

Intrahepatic cholestasis [3] can be from several diseases including any form of acute hepatitis (viral, drug, alcohol), drug induced liver injury, primary biliary cholangitis, primary sclerosing cholangitis and infiltrative diseases (sarcoidosis, tumors, abscess and cysts) [4]

Extrahepatic biliary obstruction which is the scope of this article can have various benign and malignant etiologies. These include Choledhocholithiasis, Choledochal cysts, Mirizzi's syndrome, benign stricturing disease (PSC, iatriogenic), neoplastic stricturing disease (cholgangiocarcinoma, pancreatic head cancer, ampullary carcinomal or adenoma) and infectious diseases (Pasrasitic cholangiopathy), inflammatory and autoimmune disease (AIDS cholangiopathy, autoimmune cholangiopathy).[5]

Epidemiology

Incidence:

Incidence of gall stone causing biliary obstruction is approximately 5 in 1000 people where 10-15 % of adult US population will develop gall stones in their lifetime [4]. 

Ethnicity[6][7][6]:

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 intermediate prevalence of cholelithiasis with 13.9% of women and 5.3% of men. The lowest prevalence of cholelithiasis is in sub-Saharan Black Africans with an overall prevalence of less than 5%. Caucasian North Americans have an overall prevalence of cholelithiasis of 16.6% and 8.6% in females and males, respectively

 Demographics[8][9][7][9][8]:

The socioeconomic and demographic diversity of biliary obstruction can be evidenced in the most common cause - in developed countries of the western world, cholelithiasis secondary to cholesterol stones in the most common cause, whereas brown pigment stones in the bile ducts are most common in Asia. Oriental cholangiohepatitis which is characterized by recurrent bacterial cholangitis, dilatation, and stricturing 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

Gender[7]:

Women are at greater risk of developing gallstones than men and hence increased risk for choledocholithiasis; likely secondary to the effect of estrogen increasing hepatic uptake of cholesterol which increases bile production

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 blockage of the bile duct system preventing bile release. 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 dilatation of 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 0.2-1.2 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 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 biliary obstruction patients, but the cause of this is unknown. Deposition of bile acid into the skin is one postulation that could account for these symptoms. Percutaneous biliary drainage was found to decrease pruritis symptoms.[10]

Histopathology

Cytology brushing and tissue biopsy to make a tissue diagnosis may be necessary for the workup for an unknown mass obstructing the biliary tract. This procedure can be performed at the time of an ERCP or by percutaneous or endoscopic ultrasound-directed biopsy.  

History and Physical

Presentation:

Biliary obstruction can have very varid presentation depending on underlying etiology. It usually presents as Jaundice with clay colored stools and dark urine. Patients can have pruritis if chronic and dependin on undrlying 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, gradual or acute onset, accompanying symptoms, family and social history is very important. 

Physical Examination

A detailed physical exam is of paramount importance. It is very important to check baseline vital signs, pallor and scleral icterus. 

A detailed Abdominal Examination to examine for Right upper quadrant tendrness (Murphy's sign), hepatomegaly and splenomegaly, presence of ascites, any palpable mass, stigmata of cirrhosis are very crucial for diagnosis. 

Cardiac exam to assess for signs of congestive heart failure with JVD elevation and displacement of heart sounds can be very important to determine underlying etiology.

Similarly, detailed lung pulmonary exam to assess for pleural effusions, determining one sided or bilateral is helpful in determing etiology.

Digital Rectal Examination

Sometimes rectal exam to assess for rectal cancer can be helpful. 

Neck Exam

Asessment of Left supraclavicular lymph node and other neck lymph nodes can provide helpful information.

Evaluation

Blood tests[11][12][11]:

Work up for biliary obstrution includes routine labs and some dedicated labs including:

  • CBC
  • CMP
  • Total bilirubin
  • Fractionated bilirubin
  • Alkaline phosphatase and fractinated
  • GGT
  • viral hepatitis Serology
  • Antimitochondrial antibody levels
  • Antinuclear antibody
  • Coagulation studies
  • Tumor markers (CA19-9, CEA, AFP)

Urine test:

  • Urine bilirubin

Stool test:

  • Occult blood to rule out GI malignancy 

Radiological tests[13][14][13]:

  • Abdominal ultrasound with doppler can be a starting radiological test due to low cost, non-invasive test, and easily availablility. Based on results, an Abdominal CT Scan can be the next step. MRCP (magnetic resonance cholangiopancreatography) is a very sensitive test to assess for intra and extrahepatic bile duct abnormalities. Based on non invasive test, EUS (endoscopic ultrasound) and ERCP (endoscopic retrograde cholangiopancreatogram) can be further considered. Sometime Interventional Radiology based PTCA (percutaneous transhepatic cholangiogram) cen be useful test.

Treatment / Management

Initial management:

Initial management depends on patients clinical condition and probable etiology. If the patient is stable, most of the work can be done as out patient with regular follow up. However if patients is febrile and shwoing signs of hepatic compromise, admitting the patient for evaluation and treatment may be needed. This is needed in patients with Acute Cholangitis, Cholecystitis and Acute Liver Failure. 

Treatment[15]:

Treatment varies widely for biliary obstruction depending on the underlying cause. Some common etiologies are discusssed below

Cholelithiasis and Choledocholithiasis[16][17][16]:

  • Gallstones with CBD stones;
    • 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 sphinterrotomy with lithotripsy, choledochotomy, choledochoduodenostomy, choledochojejunostomy, or cholecystectomy

CBD stricture:

  • Benign
    • Endoscopic sphincterotomy and balloon dilatation 
    • Endoscopic prosthesis with changing at 4 to 6 weeks
    • Biliary-enteric bypass if and when surgery is required
  • Malignant
    • Endoscopic drainage with stenting
    • Percutaneous drainage with stenting
    • Palliative biliary-enteric bypass in unresectable
    • Resection of the tumor with biliary-enteric anastomosis in resectable disease

Parasites[18][19][18]

  • Medication
    • Albendazole
    • Mebendazole
    • Pyrantel pamoate
  • Surgical/Endoscopic:
    • ERCP with sphincterotomy
    • Balloon catheter with dormia basket and removal of parasites
    • Follow up at 3, 6, and 12 months
    • In the case of gall bladder invasion; cholecystectomy, CBD exploration, and T-tube placement

Choledochal cyst[20]:

  • Excision and hepaticojejunostomy

Neoplasm:

  • Advanced disease:
    • Palliation with endoscopic biliary stenting and chemoradiotherapy or photodynamic therapy
    • Percutaneous transhepatic endobiliary radiofrequency ablation along with biliary stenting
    • Duodenal stenting in ampullary carcinoma
  • Resectable disease:
    • Excision with clear margins and bilioenteric anastomosis
  • Pancreatic head carcinoma:
    • Whipple procedure/pylorus-preserving pancreaticoduodenectomy
  • Ampullary carcinoma:
    • Whipple procedure 
  • Gallbladder malignancy:
    • Cholecystectomy with liver resection and lymph node clearance

Differential Diagnosis

Benign diseases[21]:

  • Acute cholecystitis and biliary colic
  • Acute pancreatitis
  • Alcoholic hepatitis
  • Ascariasis
  • Bile duct strictures
  • Biliary trauma
  • Choledochal cysts
  • Chronic pancreatitis
  • Cirrhosis
  • Gallstones (cholelithiasis)
  • Hepatitis B
  • Hepatitis C
  • Hepatocellular adenoma
  • Mirizzi syndrome
  • Primary biliary cholangitis
  • Primary sclerosing cholangitis
  • Unconjugated hyperbilirubinemia
  • Viral hepatitis

Malignant diseases:

  • Ampullary carcinoma
  • Bile duct tumors
  • Cholangiocarcinoma
  • Gallbladder cancer
  • Gallbladder tumors
  • Pancreatic cancer
  • Primary hepatic carcinoma
  • Enlarged malignant lymph node

Prognosis

Prognosis of biliary obstruction is highly dependent on the etiology. If left untreated, it can lead to life-threatening infection. If obstruction is persistent and chronic, it is usually due to a chronic liver pathology which commonly has a poor prognosis. Most acute causes can be successfully managed with medical, surgical, and/or endoscopic treatment with full recovery. Obstruction caused by chronic liver disease and carcinoma usually has a less favorable prognosis.

Complications

Cholangitis

  • One of the most feared complications of biliary obstruction is cholangitis which is an ascending infection of the biliary tree accompanying an obstruction

Presentation

  • Charcot’s triad (right upper quadrant pain, fever, and jaundice)
  • Reynold’s pentad (Charcot's triad with mental status change and hemodynamic shock)

Laboratory evaluation:

  • Elevated white blood cell count
  • Abnormal liver testing with elevated alkaline phosphatase and gamma-glutamyl transpeptidase
  • Elevated bilirubin

Evaluation

  • Abdominal ultrasound
  • Helical CT-scan
  • MRCP

Severe[22]:

  • Hypotension requiring any vasopressor
  • Mental status change
  • PaO2/FiO2 less than 300
  • Serum creatinine greater than 2.0 mg/dL
  • INR greater than 1.5
  • Platelet count less than 100000/mm

Moderate:

  • Elevated white count greater than 12000
  • Fever greater than 39 C, or 102.2 F
  • Age older than 75
  • Hyperbilirubinemia over 5 mg/dL
  • Hypoalbuminemia

Treatment:

General:

  • Admission
  • IVF resuscitation and electrolyte replacement
  • Pain control
  • Antibiotics with activity against enteric streptococci, coliforms, and anaerobes

Low-risk community-acquired:

  • Single-agent: ertapenem or piperacillin-tazobactam
  • Combination: ceftriaxone, ciprofloxacin, or levofloxacin with metronidazole

High-risk community-acquired:

  • Single-agent: imipenem-cilastatin, meropenem, or piperacillin-tazobactam
  • Combination: cefepime or ceftazidime with metronidazole

Drainage:

  • ERCP (endoscopic retrograde cholangiopancreatography); Endoscopic removal of biliary stones with drainage of bile, often with sphincterotomy
  • PTC (percutaneous transhepatic cholangiography); Insertion of a needle into biliary tract percutaneously for drainage, removal of stones, and possible placement of the biliary stent
  • Surgical Drainage; Open or laparoscopic surgical intervention with biliary decompression, removal of the obstruction, and cholecystectomy if warranted and the patient is stable

Consultations

  • Gastroenterology
  • Radiology
  • Interventional radiology
  • Pathology
  • Oncology
  • General surgery
  • Hepatology

Deterrence and Patient Education

Biliary obstruction

  • Blockage within the biliary tract or the route bile uses to leave the liver and help metabolize fat

Most Common Cause

  • Gallbladder stones that obstruct the biliary tract

Symptoms

  • Right upper quadrant abdominal pain
  • Pain in the back under the shoulder blade
  • Nausea and vomiting
  • Jaundice (yellowing of the skin)

Testing

  • Laboratory evaluation and imaging
  • Ultrasound of the abdomen
  • Further testing will be chosen appropriately depending on the results of initial testing

Treatment

  • Treatment for biliary obstruction depends on the cause but likely will be either surgical or non-surgical
  • 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 obstruction
  • Non-Surgical may include stabilization and pain control along with medications to help dissolve any gallstones causing obstruction

Prevention of recurrence

  • Maintain a healthy body weight with proper nutrition and exercise

Enhancing Healthcare Team Outcomes

Biliary obstruction is a term encompassing many separate conditions that result in a typical clinical finding of elevated bilirubin. Patients may first present to their primary care physicians, nurse practitioner or an emergency room for treatment. The prompt initial diagnosis helps drive therapy and improve outcomes in most patients. Special attention is necessary to rule out ascending infection when patients present with findings of biliary obstruction due to the poor prognosis of ascending cholangitis and cancer. Initial laboratory and radiographic assessment will determine the proper specialty needed to treat the obstruction whether that be general surgery, gastroenterology, oncology or interventional radiology. 


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