Choledocholithiasis is the presence of stones in bile ducts; the stones can form in the gallbladder or in the ducts themselves. These stones cause biliary colic, biliary obstruction, gallstone pancreatitis, or cholangitis (bile duct infection and inflammation). Cholangitis, in turn, can lead to strictures, stasis, and choledocholithiasis. Diagnosis usually requires visualization by magnetic resonance cholangiopancreatography or ERCP. Early endoscopic or surgical decompression is indicated.

Stones may be described as

• Primary stones (usually brown pigment stones), which form in the bile ducts
• Secondary stones (usually cholesterol), which form in the gallbladder but migrate to the bile ducts
• Residual stones, which are missed at the time of cholecystectomy (evident < 3 yr later)
• Recurrent stones, which develop in the ducts > 3 yr after surgery

In developed countries, > 85% of common duct stones are secondary; affected patients have additional stones located in the gallbladder. Up to 10% of patients with symptomatic gallstones also have associated common bile duct stones. After cholecystectomy, brown pigment stones may result from stasis (eg, due to a postoperative stricture) and the subsequent infection. The proportion of ductal stones that are pigmented increases with time after cholecystectomy.

Bile duct stones may pass into the duodenum asymptomatically. Biliary colic occurs when the ducts become partially obstructed. More complete obstruction causes duct dilation, jaundice, and, eventually, cholangitis (a bacterial infection). Stones that obstruct the ampulla of Vater can cause gallstone pancreatitis. Some patients (usually the elderly) present with biliary obstruction due to stones that have caused no symptoms previously.

In acute cholangitis, bile duct obstruction allows bacteria to ascend from the duodenum. Most (85%) cases result from common bile duct stones, but bile duct obstruction can result from tumors or other conditions (see Table 1: Gallbladder and Bile Duct Disorders: Causes of Bile Duct Obstruction). Common infecting organisms include gram-negative bacteria (eg, Escherichia coli , Klebsiella sp, Enterobacter sp); less common are gram-positive bacteria (eg, Enterococcus sp) and mixed anaerobes (eg, Bacteroides sp, Clostridia sp). Symptoms include abdominal pain, jaundice, and fever or chills (Charcot's triad). The abdomen is tender, and often the liver is tender and enlarged (often containing abscesses). Confusion and hypotension predict about a 50% mortality rate and high morbidity.

Table 1
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Causes of Bile Duct Obstruction Stones (common) Duct trauma due to surgery (common) Tumors Scarring due to chronic pancreatitis External compression by a cyst, a hernia of the common bile duct (choledochocele), or a pancreatic pseudocyst (rare) Extrahepatic or intrahepatic strictures due to primary sclerosing cholangitis AIDS-related cholangiopathy or cholangitis Parasitic infestation with Clonorchis sinensis or Opisthorchis viverrini Parasite migration of Ascaris lumbricoides into the common bile duct (rare)

Recurrent pyogenic cholangitis (Oriental cholangiohepatitis, hepatolithiasis) is characterized by intrahepatic brown pigment stone formation. This disorder occurs in Southeast Asia. It consists of sludge and bacterial debris in the bile ducts. Undernutrition and parasitic infestation (eg, Clonorchis sinensis, Opisthorchis viverrini) increase susceptibility. Parasitic infestation can cause obstructive jaundice with intrahepatic ductal inflammation, proximal stasis, stone formation, and cholangitis. Repeating cycles of obstruction, infection, and inflammation lead to bile duct strictures and biliary cirrhosis. The extrahepatic ducts tend to be dilated, but the intrahepatic ducts appear straight because of periductal fibrosis.

In AIDS-related cholangiopathy or cholangitis, direct cholangiography may show abnormalities similar to those in primary sclerosing cholangitis or papillary stenosis (ie, multiple strictures and dilations involving the intrahepatic and extrahepatic bile ducts). Etiology is probably infection, most likely with cytomegalovirus, Cryptosporidium sp, or microsporidia.

Diagnosis

• Liver function tests
• Ultrasonography

Common duct stones should be suspected in patients with jaundice and biliary colic. Fever and leukocytosis further suggest acute cholangitis. Elevated levels of bilirubin, alkaline phosphatase, ALT, and γ‑glutamyltransferase are consistent with extrahepatic obstruction, suggesting stones, particularly in patients with features of acute cholecystitis or cholangitis.

Ultrasonography may show stones in the gallbladder and occasionally in the common duct (less accurate). The common duct is dilated (> 6 mm in diameter if the gallbladder is intact; > 10 mm after a cholecystectomy). If the ducts are not dilated early in the presentation (eg, first day), stones have probably passed. If doubt exists, magnetic resonance cholangiopancreatography (MRCP) is highly accurate for retained stones. ERCP is done if MRCP is equivocal; it can be therapeutic as well as diagnostic. CT, though less accurate than ultrasonography, can detect liver abscesses.

For suspected acute cholangitis, CBC and blood cultures are essential. Leukocytosis is common, and aminotransferases may reach 1000 IU/L, suggesting acute hepatic necrosis, often due to microabscesses. Blood cultures guide antibiotic choice.

Treatment

• ERCP and sphincterotomy

If biliary obstruction is suspected, ERCP and sphincterotomy are necessary to remove the stone. Success rate exceeds 90%; up to 7% of patients have complications (eg, bleeding pancreatitis, infection with fibrosis and subsequent duct stricture). Laparoscopic cholecystectomy, which is not as well suited for operative cholangiography or common duct exploration, can be done electively following ERCP and sphincterotomy. Mortality and morbidity after open cholecystectomy with common duct exploration are higher. In patients at high risk of complications with cholecystectomy (eg, the elderly), sphincterotomy alone is an alternative.

Acute cholangitis is an emergency requiring aggressive supportive care and urgent removal of the stones, endoscopically or surgically. Antibiotics are given, similar to those used for acute cholecystitis (see Gallbladder and Bile Duct Disorders: Treatment) An alternative regimen for very ill patients is imipenem and ciprofloxacin, plus metronidazole to cover anaerobes.

For recurrent pyogenic cholangitis, management aims to provide supportive care (eg, broad-spectrum antibiotics), eradicate any parasites, and mechanically clear the ducts of stones and debris endoscopically (via ERCP) or surgically.

Last full review/revision December 2007 by Eldon A. Shaffer, MD