CBD Stones and Cholangitis


Common Bile Duct Stones

  1. Definitions and Pathogenesis
    1. Primary CBD Stones
      • formed within the common duct
      • earthy, crumbly, brown stones composed of calcium bilirubinate
      • associated with bile duct obstruction or stasis
      • infection, or bacterbilia, must also be present
      • bacteria such as E. coli deconjugate bilirubin diglucuronide to free bilirubin
      • free bilirubin then precipitates with calcium
      • conditions that predispose to primary CBD stones include biliary strictures, a stenotic biliary-enteric anastomosis, stenosis of the sphincter of Oddi, sclerosing cholangitis, and Asian cholangiohepatitis

    2. Secondary CBD Stones
      • migrate from the gallbladder into the common duct
      • most common type

    3. Retained CBD Stones
      • common duct stone found within 2 years of cholecystectomy

  2. Clinical Manifestations
    • occasionally, patients may be asymptomatic
    • most common presentation is right upper quadrant pain and liver function test abnormalities (↑ bilirubin, ↑ alkaline phosphatase)
    • in complicated cases, patients may present with jaundice, cholangitis, or pancreatitis
    • fever is indicative of cholangitis
    • severe epigastric pain usually results from pancreatitis

  3. Diagnosis
    1. Laboratory Tests
      • direct hyperbilirubinemia (> 4.0) is a very strong predictor of a CBD stone
      • elevations in alkaline phosphatase and transaminases are neither sensitive nor specific for CBD stones
      • amylase and lipase are poor predictors of choledocholithiasis since many patients will have passed the stone that caused the pancreatitis

    2. Ultrasound
      • lacks sensitivity for visualizing stones within the distal common duct
      • very effective at detecting gallstones and measuring the diameter of the common duct
      • a CBD < 3 mm essentially rules out a common duct stone; a CBD > 6 mm is a strong predictor of a CBD stone; a CBD > 10 mm with hyperbilirubinemia predicts choledocholithiasis 90% to 100% of the time

    3. MRCP
      • has largely replace ERCP for the diagnosis of CBD stones
      • over 90% sensitive and specific
      • if MRCP confirms a CBD stone, then a therapeutic ERCP may be performed

      MRCP of Common Duct Stone
    4. Endoscopic Ultrasound (EUS)
      • overall, has similar sensitivity and specificity as MRCP
      • may be better at detecting small stones than MRCP
      • not widely available

    5. ERCP
      • now used almost exclusively as a therapeutic procedure
      • invasive procedure with some significant complications: post-ERCP pancreatitis, bleeding, cholangitis, duodenal perforation

      ERCP of Common Duct Stone
  4. Management
    1. Preoperative Risk Assessment

      2. Preoperative Risk for a CBD Stone
    2. High-Risk Patients
      • probability of a CBD stone is > 50% (cholangitis, bilirubin > 4, CBD > 6 mm)
      • MRCP can be used to confirm the stone
      • in some situations, preceding directly to ERCP is reasonable (cholangitis)
      • preoperative ERCP followed by laparoscopic cholecystectomy is the most common treatment approach

      1. Preoperative ERCP
        • sphincterotomy and stone extraction are successful in the great majority of cases (>90%)
        • a major advantage of preop ERCP is that the surgeon learns whether there are stones present that cannot be removed by ERCP (5 or more stones, any stone > 1 cm)
        • a failed preop ERCP will necessitate a laparoscopic or open CBD exploration
        • can omit cholecystectomy in poor surgical candidates

        ERCP Sphincterotomy
    3. Intermediate-Risk Patients
      • probability of a CBD stone is 10% - 50% (bilirubin of 1.8 - 4.0, CBD > 6 mm)
      • most patients undergo an MRCP
      • if the MRCP is positive, then ERCP is done to remove the stones; if the MRCP is negative, then cholecystectomy is the next step
      • an alternative approach is to forego the preop MRCP and proceed directly to laparoscopic cholecystectomy with intraoperative cholangiogram
      • if the cholangiogram shows CBD stones, then there are several options available – which one is chosen is based on local expertise and equipment

      1. Postoperative ERCP
        • most common option chosen
        • requires a second invasive procedure
        • there is a small chance that the endoscopist will be unable to remove the stones, necessitating a return to the operating room for an open common duct exploration
        • not likely to be successful in patients with multiple stones (>4), large stones (>1.5 cm), previous Billroth II or Roux-en-Y reconstruction, or a periampullary duodenal diverticulum

      2. Laparoscopic Common Bile Duct Exploration
        • must prove the existence of common duct stones by operative cholangiography
        • a completion cholangiogram must also be obtained

        Positive Cholangiograms
        Positive Cholangiograms

        1. Transcystic Common Duct Exploration
          • stones should be < 10 mm in size and located in the distal duct
          • initial maneuver is to try to flush the stones through the ampulla (glucagon facilitates this by dilating the sphincter of Oddi)
          • if flushing is unsuccessful, then the cystic duct will often need to be dilated with a balloon catheter in order to extract the stones
          • biliary Fogarty catheters, fluoroscopically-guided wire baskets, or a flexible choledochoscope (pediatric ureteroscope) may all be used to retrieve the stones

          Laparoscopic Transcystic CBDE
        2. Laparoscopic Choledochotomy
          • very technically demanding – requires good suturing skills
          • should not be done on small common ducts (< 7mm)
          • indications include a failed transcystic exploration, large stones, multiple stones, proximal stones
          • stones are retrieved by flushing, biliary Fogarty’s, wire baskets, or a flexible choledochoscope
          • routine T-tube drainage is not mandatory
          • if retained stones are likely, then a 14F or 16F T-tube can be placed through the choledochotomy

          Laparoscopic CBDE
      3. Open Common Duct Exploration
        • indicated if ERCP is not available or is technically not possible (Billroth II reconstruction, Roux-en-Y gastric bypass, duodenal diverticulum) and if the surgeon does not have experience in laparoscopic common duct explorations
        • additional indications include any patient who is not a candidate for laparoscopy, or who has large stones or multiple stones
        • if an impacted stone cannot be removed during a common duct exploration, then a transduodenal sphincterotomy and sphincteroplasty or choledochoduodenostomy will be necessary

        Transduodenal Sphincteroplasty
        Transduodenal Sphincterotomy and Sphincteroplasty

    4. Low-Risk Patients
      • patients with no risk factors for CBD stones still have a 10% of a common duct stone
      • these patients usually undergo laparoscopic cholecystectomy without an MRCP
      • it is up to the surgeon’s discretion whether an intraoperative cholangiogram is done
      • if a CBD stone is found on IOC, then there are several different possible strategies:

      1. Laparoscopic Common Duct Exploration
        • may attempt to flush the stones into the duodenum
        • transcystic exploration vs laparoscopic choledochotomy

      2. Open Common Duct Exploration
        • indicated if there is no endoscopist available to perform ERCP or if ERCP is unlikely to be successful

      3. Expectant Management
        • small stones (1 to 2 mm) may be managed expectantly, since they are not likely to impact or cause pancreatitis

    5. Retained Common Duct Stones
      1. Recent Laparoscopic Cholecystectomy
        • a retained stone is suspected in the patient who develops pain, fever, or jaundice in the very early postoperative period
        • need also to rule out a cystic duct leak or CBD injury
        • an ultrasound is valuable for looking for fluid collections and dilated bile ducts
        • MRCP will accurately detect a retained CBD stone
        • ERCP with sphincterotomy is used to removes the stones

      2. Recent Open Common Duct Exploration
        • retained stones will be identified on routine postoperative T-tube cholangiogram at a rate between 0 and 6.6%

        T-tube Cholangiogram with Retained Stone
        T-tube Cholangiogram with Retained Stone

        1. T-Tube Extraction
          • T-tube should be left in place for 6 weeks to allow for maximal maturation of the T-tube tract
          • T-tube should be at least 14F in diameter
          • invasive radiologist will then remove the T-tube and insert a steerable basket through the T-tube tract into the common duct and extract the stone
          • alternatively, a flexible choledochoscope may be used
          • these procedures are ~ 90% to 95% successful in expert hands
          • main complication is perforation of the T-tube tract
          • most surgeons will choose ERCP and sphincterotomy if T-tube extraction is unsuccessful

      3. Cholecystectomy Within 2 Years
        • ERCP with sphincterotomy is the procedure of choice
        • open common duct exploration will be necessary if ERCP is unsuccessful

    6. Primary Common Duct Stones
      • arbitrarily defined as patients developing choledocholithiasis more than 2 years after cholecystectomy
      • primary stones are frequently multiple and large
      • initial investigation should be with ERCP

      1. Endoscopic Sphincterotomy
        • can be successful in treating selected patients but the recurrence rate is high

      2. Surgical Management
        1. Indications
          • 5 or more stones
          • stones > 1 cm
          • marked dilatation of the common duct
          • intrahepatic stones
          • distal stricture of the CBD (fixed biliary obstruction)
          • failure of endoscopic sphincterotomy
          • recurrence after endoscopic sphincterotomy

        2. Common Duct Exploration and T-Tube Drainage
          • not indicated in the majority of patients with primary common duct stones because of the high recurrence rate

        3. Choledochoduodenostomy
          • most patients with primary common duct stones will need a definitive common duct drainage procedure in addition to common duct exploration and stone extraction
          • choledochoduodenostomy is an easy to perform procedure
          • common bile duct must be greater than 1.5 cm in diameter
          • some patients develop ‘sump’ syndrome, where debris in the distal duct may cause pancreatitis or cholangitis

          Choledochoduodenostomy
        4. Roux-en-Y Hepaticojejunostomy
          • completely diverting (avoids ‘sump’ syndrome)
          • indicated in patients with intrahepatic stones and difficult biliary strictures
          • Roux limb may be tacked to the anterior abdominal wall and marked with clips, providing percutaneous access by interventional radiologists for later dilatations of strictures or stone extractions

          Hepaticojejunostomy

Acute Cholangitis

  1. Pathophysiology
    • requires 2 conditions: 1) the patient must have infected bile, and 2) there must be an increase in bile duct pressure from obstruction

    1. Sources of Infected Bile
      • from the duodenum via the sphincter of Oddi (sphincterotomy, choledocho-enteric anastomosis)
      • from contaminated gallstones from a chronically infected gallbladder
      • indwelling stents and tubes
      • instrumentation

    2. Increased Bile Duct Pressure
      • elevated bile duct pressure from partial or complete obstruction may lead to cholangiovenous reflux
      • bile canaliculi communicate directly with hepatic sinusoids, permitting reflux of bacteria into the venous circulation
      • common causes of biliary obstruction include choledocholithiasis, benign strictures (operative injury, sclerosing cholangitis) or malignant strictures, chronic pancreatitis, or an obstructed biliary anastomosis or stent

      Pathophysiology of Cholangitis
  2. Bacteriology
    • blood cultures are positive in 50% of patients and bile cultures are positive in 100%
    • most common aerobic organisms include E. coli, Klebsiella, Enterococcus, Enterobacter
    • most common anaerobe is Bacteroides fragilis
    • multiple organisms are isolated in 60% of patients

  3. Clinical Manifestations
    • has a varied presentation, ranging from a relatively mild illness to life-threatening septic shock
    • Charcot's classic triad consists of intermittent fever and chills, jaundice, and abdominal pain (occurs in ~ 50% of patients)
    • Reynolds' pentad adds shock and CNS depression to the classic symptoms and describes a much more fulminant form of the disease

  4. Diagnosis
    • majority of patients will have an elevated WBC
    • bilirubin, alkaline phosphatase, transaminases are elevated in 90%
    • ultrasound can detect gallstones and common duct dilatation, but it is less accurate in detecting common duct stones
    • Tokyo guidelines provide criteria for diagnosis based on clinical, laboratory, and imaging findings
    • cholangiography (ERCP or PTC) is the definitive test, but should not be done until the acute infection is under control

  5. Management
    • principles of treatment are 1) resuscitation, 2) antibiotics, and 3) biliary drainage
    • 85% of patients will improve with resuscitation and antibiotics alone

    1. Resuscitation
      • IV fluids
      • may need inotropic support and/or intubation
      • ICU monitoring
      • correct coagulopathy

    2. Antibiotics
      • initially need broad spectrum coverage against gram-negative, gram-positive (enterococcus), and anaerobes
      • Zosyn has good penetration into bile

    3. Biliary Drainage
      • patients who do not respond quickly to antibiotics will require emergent biliary drainage
      • relapse after initial response also requires drainage

      1. ERCP
        • most useful for patients with distal obstructions
        • sphincterotomy will manage most common duct stones
        • benign strictures can be dilated or stented
        • occluded biliary stents can be replaced
        • if a coagulopathy precludes an invasive procedure like a sphincterotomy, then a nasobiliary drain can be placed as a temporizing measure

      2. PTC
        • best option for proximal benign or malignant strictures
        • fallback option if ERCP is not available or is unsuccessful
        • also necessary if altered anatomy precludes ERCP: Billroth 2, Roux-en-Y reconstruction, large duodenal diverticulum, complete obstruction or transection of the CBD
        • does not require sedation or airway control, so may be the better choice in an unstable patient

        Percutaneous Transhepatic Cholangiography
      3. Surgical Drainage
        • used only if ERCP or PTC is unavailable or unsuccessful
        • operative mortality may be as high as 40% in unstable patients
        • emergency procedure is choledochotomy and placement of a large T-tube (16F)
        • formal CBD exploration, sphincteroplasty, biliary bypass are contraindicated in unstable patients

Sclerosing Cholangitis

  1. Pathophysiology
    • characterized by inflammatory strictures of the intrahepatic and extrahepatic biliary tree
    • progressive disease that eventually results in biliary cirrhosis
    • has no known cause, but is associated with ulcerative colitis in the majority of cases
    • likely an immune-mediated disorder with genetic influences
    • 10% to 20% will develop cholangiocarcinoma

  2. Clinical Manifestation
    • mean age at presentation is 30 to 45
    • men are affected twice as often as women
    • symptoms include intermittent jaundice, pruritis, fatigue, weight loss, and abdominal pain
    • cyclic exacerbations and remissions are typical
    • colectomy for ulcerative colitis does not affect the course of sclerosing cholangitis
    • most patients die of hepatic failure 10 to 18 years after diagnosis

  3. Diagnosis
    • alkaline phosphatase is usually elevated
    • Total bilirubin is normal in 60% of cases
    • MRC reveals beading of the intrahepatic and extrahepatic bile ducts
    • ERCP and PTC may also be used to make the diagnosis

    Sclerosing Cholangitis - MRCP
  4. Management
    • no known curative treatment
    • goal of treatment is to slow progression of disease and reduce the risks for cancer and end-stage liver disease
    • strictures can be dilated or stented to give short term improvement
    • resection of the extrahepatic biliary tree and bifurcation can provide reasonable results in patients without cirrhosis or hepatic fibrosis
    • liver transplantation is the only option in patients with cirrhosis, with 5-year survival as high as 85%; however, the recurrence rate is 10% to 20%, and may require retransplantation







References

  1. Sabiston, 20th ed., pgs 1494 - 1497
  2. Schwartz, 10th ed., pgs 1321 - 1331
  3. Cameron, 11th ed., pgs 391 - 395, 395 - 399, 409 - 413
  4. Cameron, 13th ed., pgs 453 – 456, 457 - 462
  5. UpToDate. Choledocholithiasis: Clinical Manifestations, Diagnosis, and Management. Mustafa A. Arain, MD, Martin L. Freeman, MD, Nabeel Azeem, MD. Mar 02, 2020. Pgs 1 – 31
  6. UpToDate. Surgical Common Bile Duct Exploration. Flavio G. Rocha. Aug 08, 2018. Pgs 1 – 32