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 as blockage of the extrahepatic biliary system. Disruption in bile flow due to the hepatic or intrahepatic biliary system is referred to as cholestasis. All these can present as elevated bilirubin levels and jaundice.
Bile obstruction is common and affects a large portion of 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 the infection of bile ducts or cholangitis, which can be fatal if not treated promptly.[2]
Biliary obstruction can be 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 chol[estasis 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).[3][4]
Extrahepatic biliary obstruction, which is the scope of this article, can have various benign and malignant etiologies. These include choledocholithiasis, choledochal cysts, Mirizzi syndrome, benign stricture disease (PSC, iatrogenic), neoplastic stricturing disease (cholangiocarcinoma, pancreatic head cancer, ampullary carcinomal or adenoma), and infectious diseases (parasitic cholangiopathy), inflammatory and autoimmune disease (AIDS cholangiopathy, autoimmune cholangiopathy).[5]
Incidence
The incidence of gallstones causing biliary obstruction is approximately 5 in 1000 people, where 10% to 15% of the adult U.S. population will develop gallstones in their lifetime [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 an 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%. White North Americans have an overall prevalence of cholelithiasis of 16.6% and 8.6% in females and males, respectively.
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. Recurrent pyogenic cholangiohepatitis, previously known as oriental cholangiohepatitis, 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. This is likely secondary to the effect of estrogen increasing hepatic uptake of cholesterol, which increases bile production.
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 endoscopic retrograde cholangiopancreatography (ERCP) or by percutaneous or endoscopic ultrasound-directed biopsy.
Presentation
Biliary obstruction can have very varied presentation depending on underlying etiology. It usually presents as jaundice with clay-colored stools and dark urine. Patients can have pruritis if chronic and 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, 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 tenderness (Murphy's sign), hepatomegaly and splenomegaly, presence of ascites, any palpable mass, stigmata of cirrhosis are 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 detailed lung pulmonary exam to assess for pleural effusions, determining one-sided or bilateral is helpful in determining etiology.
Digital Rectal Examination
Sometimes rectal exam to assess for rectal cancer can be helpful.
Neck Exam
Assessment of the left supraclavicular lymph node and other neck lymph nodes can provide helpful information.
Work up for biliary obstruction includes routine labs and some dedicated labs, including:
Urine Test
Stool Test
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 up. However, if the patient is febrile and showing 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 discussed below.
Cholelithiasis and Choledocholithiasis[16][17]
CBD Stricture
Choledochal Cyst[20]
Neoplasm
Benign Diseases[21]
Malignant Diseases
The prognosis of biliary obstruction is highly dependent on the etiology. If left untreated, it can lead to life-threatening infections. If 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 carcinoma usually has a less favorable prognosis.
Cholangitis
Presentation
Laboratory Evaluation
Evaluation
Severe[22]
Moderate
Treatment
General
Low-risk Community-acquired
High-risk Community-acquired
Drainage
Biliary Obstruction
Most Common Cause
Symptoms
Testing
Treatment
Prevention of Recurrence
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 practitioners, or an emergency department 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 general surgery, gastroenterology, oncology, or interventional radiology.
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