Causes of Biliary Obstruction
Biliary obstruction results from either extrahepatic mechanical blockage of the bile ducts or intrahepatic cholestatic processes, with the most common causes being gallstones (choledocholithiasis), malignant tumors (pancreatic cancer, cholangiocarcinoma, ampullary cancer), and benign strictures. 1
Extrahepatic (Mechanical) Causes
Intrinsic Obstruction
- Choledocholithiasis is the most frequent cause of extrahepatic biliary obstruction, occurring in 7-20% of patients undergoing cholecystectomy and representing the primary etiology of acute cholangitis and gallstone pancreatitis 2, 3
- Malignant tumors causing distal biliary obstruction include pancreatic adenocarcinoma (most common), distal cholangiocarcinoma, ampullary carcinoma, and less commonly gallbladder cancer or metastatic disease 1
- Cholangiocarcinoma can present as either hilar (Klatskin tumor) or distal lesions, with the periductal-infiltrating subtype growing along bile ducts without forming a discrete mass 4
- Benign strictures may develop from chronic pancreatitis, post-surgical complications, or ischemic injury 1
Extrinsic Compression
- Pancreatic head masses from pancreatitis or pancreatic tumors commonly compress the distal common bile duct 1
- Periductal lymphadenopathy from lymphoma or metastatic disease can cause extrinsic compression 1
- Benign liver lesions in segment 4, including simple hepatic cysts as small as 3-4 cm, can compress the common hepatic duct and cause peripheral bile duct dilation with rising alkaline phosphatase 4
Intrahepatic (Cholestatic) Causes
Cholangiocellular Cholestasis
- Primary biliary cholangitis (PBC) is the major cause of small-duct biliary disease, diagnosed with high-titer antimitochondrial antibodies (≥1:40) and cholestatic enzyme profile 1
- Primary sclerosing cholangitis (PSC) causes progressive bile duct stricturing and can be missed on standard ultrasound 1
- IgG4-associated cholangitis presents with biliary strictures and requires tissue diagnosis with IgG4 immunostaining (≥10 IgG4-positive cells per high-power field) 1
- Secondary sclerosing cholangitis results from cholangiolithiasis, ischemic cholangiopathies (vasculitis, hereditary hemorrhagic telangiectasia), or infectious cholangitis in immunocompromised patients 1
Hepatocellular Causes
- Acute viral hepatitis (hepatitis A, B, C, D, E, Epstein-Barr virus) disrupts conjugated bilirubin transport 1
- Alcohol-induced liver disease and autoimmune hepatitis cause hepatic inflammation affecting bile transport 1
- Drug-induced cholestasis from medications including acetaminophen, penicillin, oral contraceptives, anabolic steroids, and chlorpromazine 1
- Sepsis-induced cholestasis disrupts hepatocellular bile transport through inflammatory cytokines and endotoxins without mechanical obstruction 4
Congenital and Developmental Causes
- Caroli disease represents the classic congenital cause of isolated intrahepatic bile duct dilation, characterized by segmental saccular or fusiform cystic areas with the pathognomonic "central dot sign" on imaging 4
- Ductal plate malformations including biliary hamartomas and Caroli syndrome 1
- Cystic fibrosis causes cholangiopathy through abnormal bile composition 1
Special Populations
Pediatric Causes
- Biliary atresia requires liver biopsy for definitive diagnosis and has best outcomes when Kasai procedure is performed before 60 days of age 5
- Metabolic diseases including α1-antitrypsin deficiency, galactosemia, tyrosinemia, and disorders of bile acid biosynthesis 1, 5
- Alagille syndrome (Jagged 1 defect) presents with syndromic paucity of bile ducts 1
Patients with Gallstone History
- Recurrent choledocholithiasis occurs in patients with prior gallstone disease, particularly those with retained stones after cholecystectomy 6, 2
- Mirizzi syndrome from impacted cystic duct stones causing extrinsic compression of the common hepatic duct 1
Patients with Pancreatitis History
- Chronic pancreatitis causes distal common bile duct strictures from fibrosis and inflammation 1
- Pancreatic pseudocysts can compress the bile duct 1
- Autoimmune pancreatitis (IgG4-related disease) causes both pancreatic and biliary strictures 1
Critical Diagnostic Pitfall
The most important initial step is distinguishing extrahepatic mechanical obstruction from intrahepatic cholestasis using abdominal ultrasound to assess for bile duct dilation, as this fundamentally determines subsequent management. 1 Ultrasound demonstrates 65-95% sensitivity for detecting biliary obstruction with 98% positive predictive value for liver parenchymal disease, though it may miss distal obstruction due to overlying bowel gas 1. If imaging does not demonstrate mechanical obstruction but clinical suspicion remains high (particularly for early pancreatic or ampullary carcinoma), repeat imaging with MRCP or endoscopic ultrasound should be performed rather than assuming intrahepatic cholestasis 1, 6.