Biliary Obstruction


Article Author:
Ethan Coucke


Article Editor:
Peter Lopez


Editors In Chief:
Susan Johnson
Alexandra Caley


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Trevor Nezwek
Radia Jamil
Patrick Le
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Hassam Zulfiqar
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Sarosh Vaqar
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
3/31/2019 8:15:49 PM

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]

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 gallstones which commonly manifest by dilatation of the common bile duct and jaundice. 

Etiology

Intrahepatic causes[2]:

  • Hepatocellular disease (viral hepatitis, drugs, alcohol)
  • Drug-induced cholestasis
  • Biliary cirrhosis
  • Alcoholic liver disease 
  • Space-occupying lesions (abscesses, tumors, hydatid cysts)

Extrahepatic: 

  • Intraluminal

or

  • Extraluminal

Intraluminal:

  • Gallstone disease
  • Neoplasms
  • Choledochal cyst
  • Biliary stricture (surgical trauma and intervention, tears in CBD (most common), erosion of bile duct due to stone, external abdominal injury, and pancreatitis)
  • Parasites (Ascaris lumbricoides can migrate from the intestine into the bile ducts, and their eggs cause luminal obstruction producing cholestasis)
  • Primary sclerosing cholangitis (PSC) (diffuse inflammation of bile ducts causing generalized scarring and fibrosis causing progressive obstructive jaundice over a long period)
  • AIDS-related cholangiopathy 
  • Infection
  • Parenteral feeding 
  • Mirizzi syndrome (common hepatic duct obstruction caused by extrinsic compression from a stone in the cystic duct)

Extraluminal causes:

  • Biliary tree gallstones (most common cause )
  • Neoplasm:
    • Cholangiocarcinomas (tumors originating from biliary epithelium)
    • Ampullary carcinomas 
    • Gallbladder malignancy
    • Pancreatic tumors
    • Choledochal cysts
  • Metastatic tumors and lymph node enlargement in the porta-hepatis
  • Pancreatitis due to inflammation, stricture, or pseudocyst pressure
  • Cirrhosis

Epidemiology

Incidence[3]:

  • In the United States of America, approximately 5 cases of biliary obstruction per 1000 people occur in people with gallstone disease (also the most common cause)
  • 10 to 15% (20 to 25 million) of the adult USA population will develop gallstones

Ethnicity[4][5][4]:

  • 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[6][7][5][7][6]:

  • 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[5]:

  • Women are at greater risk of developing gallstones than men; 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.[8]

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

  • Yellowing of skin and eyes
  • Pale stools
  • Dark urine
  • Pruritus
  • Right upper quadrant abdominal pain
  • Fever
  • Intractable nausea and vomiting

History

  • Age and associated comorbidities
  • Pain including site and characteristics
  • Duration of symptoms
  • Pattern of jaundice
  • Associated symptoms
  • Gastric symptoms
  • History of malignancy or jaundice
  • Known history of gallstone disease
  • History of gastrointestinal bleeding
  • History of hepatitis
  • History of biliary intervention
  • History of alcohol intake, drug use, and medications
  • History of blood transfusions
  • Family history of jaundice or malignancy

Physical Examination

  • Weight and BMI
  • Vitals
  • Signs of jaundice; yellowing of skin and icterus

Abdominal Examination

  • Palpate for hepatomegaly and splenomegaly
  • Examine for ascites
  • Palpate for a palpable mass
  • Look for signs of cirrhosis
  • Examine for palpable gallbladder (Courvoisier sign)

Digital Rectal Examination

  • Rectal shelf for rectal metastasis

Neck

  • Left supraclavicular lymph node, other neck lymph nodes

Evaluation

Blood tests[9][10][9]:

  • CBC
  • CMP
  • Total bilirubin
  • Conjugated bilirubin
  • Alkaline phosphatase
  • GGT
  • Liver enzymes
  • Serology
  • Antimitochondrial antibody levels
  • Coagulation studies
  • Lipase levels

Urine test:

  • Urine bilirubin

Stool test:

  • Occult blood to rule out GI malignancy 

Radiological tests[11][12][11]:

  • Abdominal ultrasound (initial, low cost, non-invasive, and easily available)
  • Abdominal CT Scan
  • MRCP (magnetic resonance cholangiopancreatography)
  • EUS (endoscopic ultrasound) 
  • PTCA (percutaneous transhepatic cholangiogram)

Treatment / Management

Initial management:

  • Admit patient to the hospital and depending on their clinical presentation possibly to the ICU
  • Intravenous fluid resuscitation with a goal to normalize hemodynamics and stabilize the patient
  • Many patients will be made NPO due to intractable nausea and vomiting and/or plan for procedure and will require maintenance IVF
  • Pain relief as needed with NSAIDs
  • Treat suspected or confirmed infection with broad-spectrum antibiotics, narrowing to targeted antibiotic therapy with culture and sensitivity results
  • Treat pruritus with antihistamines
  • Discontinue any medications that are known to cause cholestasis if applicable 

Treatment[13]:

  • Varies widely for biliary obstruction depending on the underlying cause

Cholelithiasis[14][15][14]:

Nonsurgical

  • Pain control with NSAIDs, reserve opioids for patients with contraindications to NSAIDs
  • Oral bile salts for 2 years ensuring normal gallbladder emptying with oral cholecystography
  • Ursodeoxycholic acid (10 mg/kg/d) for 6 months for the dissolution of cholesterol-containing stones with a high recurrence rate (50%)
  • Extracorporeal shock-wave lithotripsy if not contraindicated, this also has a high recurrence rate (70%)

Surgical

  • Biliary atresia- Kasai procedure (hepato-portoenterostomy)
  • 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 - 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[16][17][16]

  • 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[18]:

  • 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[19]:

  • 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[20]:

  • 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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Biliary Obstruction - Questions

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A 46-year-old obese female is seen in the emergency department with right upper quadrant pain, jaundice, low-grade fever, and nausea. She says that whenever she eats, she has been getting pain that is now radiating to her back. Lab work reveals mild elevation in WBC and high bilirubin levels. She is sent for an ultrasound, and the report comes back saying that she has Mirizzi syndrome. What is Mirizzi syndrome?



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What is the most common cause of biliary obstruction in North America?



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What does dark yellow fluid draining from a biliary drainage tube indicate?



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Which of the following is seen in obstructive jaundice?



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A 65-year-old female who was admitted for abdominal pain with intractable nausea and vomiting is found to have severely elevated transaminases, lipase, and bilirubin. Her abdominal ultrasound is significant for a 7 mm common bile duct without evidence of cholecystitis, cholelithiasis, or choledocholithiasis. Her laboratory values and clinical picture improve the next day significantly. The patient has no fever, tachycardia, abdominal pain, nausea, or vomiting. The patient is currently on a liquid diet and tolerating it well, asking for it to be advanced to a regular diet. Which of the following is the next best step in the management of this patient?



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Biliary Obstruction - References

References

Pavlidis ET,Pavlidis TE, Pathophysiological consequences of obstructive jaundice and perioperative management. Hepatobiliary & pancreatic diseases international : HBPD INT. 2018 Feb     [PubMed]
Siddiqui AI,Ahmad T, Biliary Atresia . 2019 Jan     [PubMed]
Naumowicz E,Białecki J,Kołomecki K, Results of treatment of patients with gallstone disease and ductal calculi by single-stage laparoscopic cholecystectomy and bile duct exploration. Wideochirurgia i inne techniki maloinwazyjne = Videosurgery and other miniinvasive techniques. 2014 Jun     [PubMed]
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