General Surgery (Board Review Series) 1st Edition

16

The Biliary Tract

Daniel P. Raymond

1. General
2. Anatomy (Figure 16-1)
3. The gallbladder is dividedinto the fundus, body, infundibulum, and neck.
4. The neck of the gallbladder

• tapers into the cystic duct, which joins with the common hepatic ductto form the common bile duct (CBD).

1. The cystic duct

• contains the spiral valves of Heister, which have little functional significance.

1. The main blood supply

• to the gallbladder is the cystic artery.

2. The cystic artery

• arises from the right hepatic artery95% of the time.
• is usually found within the triangle of Calotbounded laterally by the cystic duct, medially by the common hepatic duct, and superiorly by the liver.

3. Cystic veins

• drain directly into the liver and the right portal vein branch.

1. Innervation
2. The hepatic branch of the left (anterior) vagal trunk provides parasympathetic innervation.
3. Sympathetic fibers, including sensory fibers that mediate biliary pain, originate from T7–T10 coursing through splanchnic and celiac ganglions.
4. The biliary ducts
5. The right and left hepatic ductsform the common hepatic duct.

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• Approximately 25% of the time anterior and posterior portions of the right hepatic duct join the common hepatic duct at different points.

1. The CBD

• beginsin the hepatoduodenal ligament as part of the portal triad, and passes posterior to the first portion of the duodenum.
• joins the pancreatic duct to form a common channel leading to the intraduodenal portion called the ampulla of Vaterin 70% of individuals.

1. The sphincter of Oddi

• is a circumferential band of smooth muscle around the ampulla of the CBD and the main pancreatic duct.

1. The main arterial blood supply

• comes from branches of the gastroduodenaland right hepatic arteries. These branches run along the medial and lateral aspects of the bile duct.

Figure 16-1. Anatomy and arterial supply of the gallbladder. (Adapted with permission from Lippincott Williams & Wilkins. Gray SW, Skandalakis JE: Atlas of Surgical Anatomy for General Surgeons. Baltimore, Williams & Wilkins, 1985, p 199.)

3. Biliary anatomy

• is highly variable.

1. The right hepatic artery

• may arise from the superior mesenteric artery.

1. An anomalous left hepatic artery

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• may originate from the celiac axis and travel in the medial gastrohepatic ligament.

1. Accessory hepatic ducts

• may also be present.

1. Histology
2. There is no muscularis mucosa or submucosa

• in the gallbladder wall.

2. The epithelium

• is predominately columnarwith absorptive functions.
• Rokitansky-Aschoff sinusesare invaginations of the epithelium seen in the wall of normal gallbladders but are more abundant when inflammation is present.

3. Ducts of Luschka

• are microscopic bile ducts that drain directly from the liver into the gallbladder.
• are found in approximately 1% of the population.

1. Radiologic evaluation of the biliary tree
2. Ultrasound
3. Ultrasound is the examination of choice for initial evaluation of suspected cholelithiasis or cholecystitis.
4. Drawbacks include operator dependency and limited examination of the CBD.
5. Radionuclide scan [hepatobiliary iminodiacetic acid (HIDA) scan]
6. Intravenous (IV) administered radionuclide is

• extracted from the bloodstream by the liver.
• excreted into the bile.
• concentrated by the gallbladder.

1. Opacification of the biliary tree

• but not the gallbladdersuggests cystic duct obstruction, and therefore cholecystitis.

1. This scan is also useful for detecting

• a bile leak from a biliary tract injury.

3. Endoscopic retrograde cholangiopancreatography (ERCP)
4. Using upper endoscopy

• the ampulla of Vater is cannulated.
• contrast is injected to visualize the pancreatic and biliary tree.

1. ERCP is useful for diagnosing

• common bile duct stones.
• periampullary disease.

1. Therapeutic measures may be performed simultaneously, including

• partial division of the sphincter of Oddi (sphincterotomy).

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• stone removal.

1. Risksinclude

• bleeding.
• infection.
• perforation.
• pancreatitis(5%–10%).

4. Percutaneous transhepatic cholangiography (PTC or THC)
5. A fine needle is passed percutaneously

• through the liver parenchyma with contrast injected into the biliary system.

1. Risks associated with this procedureinclude

• bleeding.
• infection.
• sepsis.

5. Plain abdominal film

• Only 10%–15%of gallstones are radiopaque.
• Plain films may also demonstrate air in the biliary system associated with a cholecysto-enteric fistula.

6. Oral cholecystography

. Orally administered contrast

• is secreted in the bile and concentrated in the gallbladder in the absence of biliary tree obstruction.

1. Oral cholecystography is used infrequently because

• the examination takes approximately 10 hours to complete.
• the absorption of contrast is inconsistent.

7. Computed tomography (CT) scan

. CT scan is useful for

• examining the hepatic parenchyma.
• identifying mass lesions.
• detecting pathologic lymph nodes.

1. CT scans are not usedin the routine work-up of gallstones or cholecystitis.
2. Magnetic resonance cholangiopancreatography (MRCP)

• is an emerging technology that may eventually replace invasive cholangiopancreatography.
• Currently, excessive cost is the major limiting factor in the use of MRCP.

1. Physiology
2. Bile is composed predominately of

• bile salts.
• phospholipids (the majority of which is lecithin).

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• cholesterol.

1. Bile salts
2. The liver produces two primary bile salts: cholate and chenodeoxycholate.
3. These salts are conjugated

• with glycine and taurine to improve water solubility.

2. After excretion

• colonic bacteria can alter these salts to form the secondary bile salts: deoxycholateand lithocholate.

3. The primary function of bile is to

• transport lipids in micelles and vesicles.
• aid in digestion and absorption.

4. Cholesterol solubility in bile is determined

• by the relative concentration of the three main components of bile: cholesterol, bile salts, and lecithin.
• Alterations in the relative concentration of these components can result in cholesterol crystal formation.

1. Enterohepatic circulation (Figure 16-2)
2. Ninety-five percent of bile salts are

• reabsorbed in the ileum.
• extracted from portal venous blood by hepatocytes.

2. Approximately 5% of bile salts

• enter the colon where colonic bacteria can convert them to secondary bile salts.

1. Gallbladder function
2. During fasting

• the gallbladder stores and concentrates bile.

2. During a meal

• sphincter of Oddi relaxation and gallbladder contraction are coordinated by neurohumoral mechanisms.

1. Cholecystokinin (CCK)

• produced by duodenal mucosa in response to a meal is the major stimulus for these functions.

1. Vagal stimuli

• facilitate the coordinated response to CCK.
• The pylorocholecystic reflexconsists of gallbladder contraction and sphincter relaxation in response to antral distension.

1. Bilirubin
2. Breakdown of red blood cells

• leads to production of heme, which is subsequently converted to biliverdin.

1. Biliverdinis then reduced to unconjugated bilirubin.

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1. Water insoluble unconjugated bilirubinis bound to albumin in the blood.
2. Unconjugated bilirubin

• is converted to conjugated bilirubinby hepatocytes in a reaction catalyzed by glucuronyl transferase.

3. Water soluble conjugated bilirubin

• is actively secreted into the bile.

4. Jaundiced patients

• usually have a total bilirubin ≥ 2.5 mg/dL.

1. Examination of the sclera of the eyemay demonstrate jaundice in patients with a dark complexion.
2. An algorithm for the work-up of a jaundiced patient is outlined in Figure 16-3.

Figure 16-2. The enterohepatic circulation. AST = aspartate aminotransferase; ALT = alanine aminotransferase; CCK = cholecystokinin; GGT = gamma-glutamyl transaminase. (Adapted with permission from Way LW: Current Surgical Diagnosis and Treatment, 10th ed. Stamford, CT, Appleton & Lange, 1994, p 540.)

III. Calculous Biliary Disease (Table 16-1)

1. Cholelithiasis

• Approximately 10% of the population have cholelithiasis.

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• The majority of the gallstonesare asymptomatic.
• Only 30%of individuals with cholelithiasis eventually have surgery.

Figure 16-3. Evaluation of the jaundiced patient. CT = computed tomography; ERCP = endoscopic retrograde cholangiopancreatography; PTC = percutaneous transhepatic cholangiography. (Adapted with permission from O'Leary JP: The Physiologic Basis of Surgery. Baltimore, Williams & Wilkins, 1996, p 476.)

1. Cholesterol stones

• account for 75% of all gallstones in the United States.
• form almost exclusively in the gallbladder.

1. Cholesterol supersaturation

• results from alterations in bile components leading to precipitation of cholesterol crystals and subsequent stone formation.

2. Bile stasis

• also contributes to stone formation.

3. Risk factors for cholesterol stone formationinclude

• being female (women are affected 2–4 times more often than men before menopause).
• multiple pregnancies/oral contraceptive use.

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• increasing age.
• -obesity.
• rapid weight loss.
• prolonged total parenteral nutrition (TPN).

Table 16-1. Characteristics of the Various Types of Biliary Calculous Disease

Stone Type Pathogenesis Risk Factors Composition Presentation Cholesterol (most common) Altered bile components, excess cholesterol, bile stasis Female, obesity, rapid weight loss, prolonged TPN, BCPs Cholesterol crystals Biliary colic, cholelithiasis, cholecystitis, secondary ductal stones Black pigment stone Altered bilirubin solubilization, bile stasis Hemolytic disorders, prolonged TPN, cirrhosis, partial ileal resection Calcium bilirubinate, bile acids, bilirubin polymers Biliary colic, cholelithiasis, cholecystitis, secondary ductal stones Brown pigment stone Bacterial deconjugation of bilirubin, bile stasis Biliary tract infections Calcium bilirubinate, bile acids, bilirubin polymers, bacteria Choledocholithiasis, cholangitis, primary ductal stones Biliary sludge Bile stasis Situations which predispose to bile stasis such as prolonged TPN Cholesterol crystals, calcium bilirubinate, mucin gel matrix Biliary colic, incidental ultrasound finding TPN = total parenteral nutrition; BCPs = birth control pills.

1. Pigment stones
2. Twenty-five percent of gallstones

• in the United States are predominantly pigment stones.

2. Altered solubilization of bilirubin

• results in the precipitation of calcium bilirubinate and other insoluble salts.

3. Black pigment stones are found

• in patients with hemolytic disordersand cirrhosis.
• almost exclusively in the gallbladder.

1. Other risk factorsinclude

• chronic TPNtherapy.
• partial ileal resection.

1. Important factors in pathogenesisinclude

• increased bilirubin load.
• impaired hepatic function.
• bile stasis.

4. Brown pigment stones

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. In Asian populations

• these are the most common types of stones.

1. Infectionand bacterial deconjugation

• of bilirubin result in altered solubility.
• are most commonly found in the bile ducts (primary common duct stones).

1. Asymptomatic gallstones
2. Approximately 50% of gallstones

• are truly asymptomatic.
• generally do not require surgical treatment.

2. Relative indications for treatment of asymptomatic gallstonesinclude

• large stones(> 2–3 cm) secondary to increased risk of cholecystitis.
• calcified (porcelain) gallbladderdue to increased risk of malignancy.
• age over 70 years.
• diabetes.
• immunosuppression.

3. Care must be taken to recognize

• atypical complaints suggestive of gallstone disease such as dyspepsia, vague epigastric discomfort, or increased flatulence.

1. Biliary sludge

• is viscous bilecomposed of calcium bilirubinate, cholesterol crystals, and mucin gel matrix.
• is generally observed during ultrasound evaluation.
• is most likely a precursor to gallstone formation.
• may be associated with prolonged TPNuse and fasting.

1. Symptomatic Gallstone Disease (Table 16-2)
2. Biliary colic

is pain caused by

• impaction of a gallstone in the cystic duct.
• transient ductal obstruction.
• possibly, stone passage.

1. Characteristics of pain
2. Postprandial right upper quadrant (RUQ) painoften precipitated by fatty meals is characteristic, although midepigastric and right lower quadrant (RLQ) pain can occur.
3. The onset of pain isusually rapid, followed by a plateau of discomfort that lasts minutes to hours.
4. Pain often radiates to the back, inferior to the right scapula.

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2. Nausea and vomitingmay also be present.
3. The clinical manifestations often overlapwith cholecystitis, and the clinical distinction may be difficult.

Table 16-2. Clinical Characteristics of Biliary Tract Disease

Disease Pathogenesis Characteristic findings Treatment Biliary colic Transient ductal obstruction, stone passage Transient postprandial RUQ pain, nausea, vomiting Elective cholecystectomy Acute cholecystitis Cystic duct obstruction with subsequent inflammation RUQ pain, fever, nausea, vomiting, (+) Murphy's sign, leukocytosis Urgent cholecystectomy Choledocholithiasis Transient or persistent obstruction of biliary duct by a stone RUQ pain, jaundice, acholic stools, bilirubinuria, elevated alkaline phosphatase, elevated bilirubin. Signs and symptoms tend to fluctuate. Cholecystectomy plus ERCP with stone removal and sphincterotomy or Common bile duct exploration with stone removal Cholangitis Bile duct obstruction and infection of bile Fever, RUQ pain, jaundice, acholic stools, bilirubinuria, signs of sepsis (e.g., mental status changes, hypotension), leukocytosis, elevated alkaline phosphatase, elevated bilirubin, elevated liver transaminases, positive blood cultures Antibiotics plus ERCP with stone removal and sphincterotomy or PTC with stone removal and stenting or Biliary bypass (e.g. choledochojejunostomy) plus subsequent cholecystectomy RUQ = right upper quadrant; ERCP = endoscopic retrograde cholangiopancreatography; PTC = percutaneous transhepatic cholangiography; (+) = positive.

1. Acute cholecystitis

• occurs in approximately 10% of patients with symptomatic gallstones.

1. Obstruction of the cystic duct by a gallstone

• causes gallbladder distension and subsequent inflammation.

2. Presentation
3. Seventy-five percent of patients

• have had previous attacks of biliary colic.

1. The pain is

• similar to biliary colic.
• often associated with a meal, but is unremitting.
• associated with increasing abdominal tenderness.

1. Patients often complain of

• nausea.
• vomiting.

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• anorexia.

1. Physical findingsinclude

• RUQtenderness.
• guarding.
• a palpable gallbladder (30%).
• a positive Murphy's sign(inspiratory arrest during deep palpation in the RUQ).

1. Mild fever and leukocytosis

• may be present.

1. Alkaline phosphatase and bilirubin

• may also be mildly elevated although such findings may also suggest the presence of CBD obstruction.

Figure 16-4. Note thickening of the gallbladder wall (small arrows) and multiple small stones (large arrow) in this ultrasound. (Reprinted with permission from Daffner RH:Clinical Radiology: The Essentials, 2nd ed. Baltimore, Williams & Wilkins, 1999, p 329.)

3. Radiologic evaluation

. An RUQ ultrasound (Figure 16-4) may demonstrate suggestive findings, including

• gallstones.
• gallbladder wall thickening.
• sludge.
• pericholecystic fluid.
• CBD dilation suggestive of CBD obstruction.

1. Murphy's sign

• induced by the ultrasound probe with maximal tenderness over the gallbladder is suggestive of cholecystitis.

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1. Ultrasound is often

• the only test needed in the work-up of this disease.

1. A radionuclide scan

• may be useful when ultrasound findings are equivocal.
• Normal visualization of the biliary tree without visualization of the gallbladder is diagnostic of cystic duct obstruction and acute cholecystitis.

4. Treatment

. Laparoscopic cholecystectomy

• is the procedure of choice for managing uncomplicated acute cholecystitis.

2. Contraindications include hemodynamic instability.
3. Extensive previous abdominal surgery is a relative contraindication because of adhesions.
4. Open cholecystectomy

• through an RUQ incision is the preferred alternative when the laparoscopic approach cannot be performed.
• is associated with a longer hospital stay and a longer convalescence.

1. Percutaneous transhepatic placement of a cholecystostomy tube

• using CT or ultrasound guidance may be performed in patients who cannot tolerate surgery.
• When patients are able to tolerate surgery, a cholecystectomy should subsequently be performed.

1. Fluid resuscitation and perioperative antibiotics

• are other important components of therapy.
• Organisms may include Escherichia coli, Klebsiella, Enterococcus, and Enterobacterspecies.

5. Complications of acute cholecystitis

. Suppurative cholecystitis

• with frank purulence in the gallbladder may be associated with sepsis and shock and requires emergent cholecystectomy.

1. Perforation

• occurs in 3%–10% of cases.
• can result in peritonitis, abscess formation, or fistula formation.

1. Gallstone ileus

• is actually a bowel obstruction, often at the level of the ileum, caused by a gallstone that has passed through a cholecystenteric fistula.
• Obstructive symptoms with air in the biliary treemay be suggestive.

1. Emphysematous cholecystitis (gas in the gallbladder wall)

• may be produced by certain bacteria such as Clostridium perfringens.
• is seen more frequently in diabetic patients.
• Sepsis can develop rapidly and emergent cholecystectomy is required.

1. Choledocholithiasis

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• is found in 15% of patients with acute cholecystitis.

1. Acalculous cholecystitis
2. Twenty percent of cases of acute cholecystitisoccur in the absence of stones.
3. Causative factorsmay include

• bile stasis and altered bile composition.

3. Acalculous cholecystitis is most commonly associated

• with trauma and burn injuries.
• with prolonged TPN use.
• in critically ill patients.

4. Complicationsinclude

• rapid progressionto gangrenous cholecystitis. Surgical treatment with cholecystectomy is required.

1. Choledocholithiasis
2. Primary common duct stones

• are stones that form primarily in the bile ducts.
• are almost exclusively brown pigment stonesrelated to stasis and infection.

1. Secondary common duct stones

• are stones that form in the gallbladder and pass into the CBD.

1. Fifteen percent of patients with stones in the gallbladder also have common duct stones.
2. One to 2% of postcholecystectomy patients need further intervention secondary to common duct stones.
3. Presentation
4. Affected patients

• may be asymptomatic.
• However, jaundice, biliary colic, and suppurative cholangitis may be present.

2. Incomplete obstruction

• produces the classic picture ofcholedocholithiasis with fluctuating symptoms of biliary colic, jaundice, and pruritus.

3. Complete obstruction

• produces a rapidly developing biliary colic.
• can progress to cholangitis.

4. Other symptoms include

• clay-colored stools (acholic stools).
• dark urine (bilirubinuria).

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• possibly, symptoms associated with pancreatitis.

5. Physical findings include

• RUQ tenderness.
• jaundice.
• fever.
• hepatomegaly.

6. Elevated serum alkaline phosphatasemay be present.
7. Elevated serum bilirubinwith predominant elevation in direct bilirubin may be present.

• Transaminases are often normal in uncomplicated choledocholithiasis.

1. Radiologic evaluation
2. Ultrasound

• finding of gallstones with biliary duct dilatation can be diagnostic.
• The normal CBD is ~ 8 mm.

2. Subsequent cholangiography

• can characterize biliary anatomy and obstruction.
• ERCPis usually preferred over PTC because of its potential therapeutic benefit (e.g., stone removal, sphincterotomy).

1. The goals of treatment include

• stone removal.
• prevention of recurrent disease.

1. Primary common duct stones
2. ERCP with stone removal

• and sphincterotomy is appropriate treatment.

1. Surgical exploration

• of the bile duct with stone removal and sphincterotomy or choledochojejunostomy may be necessary if ERCP is unsuccessful.

2. Secondary common duct stones
3. Laparoscopic cholecystectomy

• is performed to prevent further disease.

1. Stone removal can be achieved by

• intraoperative choledochoscopy(fiberoptic camera-guided exploration).
• formal common bile duct exploration.
• postoperative ERCP, if necessary.

1. Complications
2. Acute pancreatitis

• Biliary stones account for approximately 45% of all cases of pancreatitis.

2. Hepatic abscessand biliary cirrhosis can be associated with chronic obstruction.

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3. Biliary strictures
4. Hemobilia and gallstone ileusare rare complications.
5. Cholangitis

• is an acute bacterial infectionof the biliary tract.
• is associated with bacterial contamination of bile and biliary obstruction.

1. The most common cause

of biliary obstruction

• associated with cholangitis is choledocholithiasis.

1. Other potential causes of obstruction

associated with cholangitis include

• biliary strictures (e.g., iatrogenic).
• neoplasms (rare).
• chronic pancreatitis.
• congenital cysts.
• duodenal diverticula.

1. Bile is normally sterile, but common organisms in cholangitis

include

• E. coli.
• Klebsiella.
• Enterococcus.
• Pseudomonas.
• Enterobacter.

1. Presentation
2. Classically, patients present with

• fever.
• RUQ pain.
• jaundice.
• These three signs make up Charcot's triad.

2. Charcot's triad

• plus hypotension and mental status changes (Reynolds pentad)suggests suppurative cholangitis (see Chapter 9).

3. Other symptomsthat may occur include

• bilirubinuria.
• pale acholic stools.
• tender hepatomegaly.

4. Laboratory findingsinclude

• leukocytosis.

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• elevations in alkaline phosphatase, transaminases, and bilirubin, with predominant elevation of the direct fraction.
• Blood culturesmay be positive in 40%–50% of patients.

1. Radiologic evaluation
2. Ultrasound

• will often characterize the obstruction, although cholangiogram may also be useful in some situations.

2. CT scan

• may be helpful, especially when a neoplasm or pancreatitis is suspected.

1. Treatment
2. Initial treatment requires

• fluid resuscitation.
• appropriate antibioticadministration.

2. Subsequent definitive treatment requires

• urgent relief of the biliary obstruction.

1. ERCP with sphincterotomy or PTC

• may be used for stone removal or stent placement across an obstruction and/or biliary tree decompression.

1. Choledochojejunostomy

• may be indicated in cases of neoplasm or stricture.

1. Emergent biliary decompression

• is required in 10% of patients due to sepsis.

1. Complications include

• sepsis.
• stricture formation.
• hepatic abscess formation.

VII. Benign Biliary Strictures

• are most commonly caused by iatrogenic injuriesand primary sclerosing cholangitis (PSC).

1. Iatrogenic injuries
2. Causes of injuryinclude

• laparoscopic cholecystectomy (80%).
• excessive retraction.
• devascularization.
• fibrosis secondary to adjacent inflammatory processes (e.g., abscess).

2. Early presentation of such injuries

• is often secondary to adjacent processes (e.g., bile leak, abscess).
• may occur days to weeks after surgery.

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• Laboratory evaluation often reveals elevated serum bilirubin and alkaline phosphatase.

3. Late presentation (years after surgery)

• is due to stricture formation with signs of biliary obstruction including recurrent cholangitis, jaundice, and cirrhosis.

4. Radiologic evaluation
5. PTCis generally more useful than ERCP because it more accurately delineates the biliary tree proximal to the site of obstruction.
6. Radionuclide studies(e.g., HIDA scan) can also diagnose a bile leak.
7. Treatment

. The initial step

• often involves placement of a catheter into the proximal biliary tree using PTCto allow for decompression proximal to the stricture.

1. Appropriate drainage

• of bile collections or abscesses is also necessary.

1. If obstruction does not resolve

• with these treatment measures, surgical treatment may be needed (i.e., choledochojejunostomy or hepaticojejunostomy).

1. Balloon dilation and stenting

• are being used with increasing frequency in the management of such strictures.

1. Primary sclerosing cholangitis (PSC)

• is an idiopathic diseasecharacterized by inflammatory strictures of the intrahepatic and extrahepatic bile ducts.

1. Ulcerative colitis

• is present in approximately 60% of patients with PSC.

2. Other less commonly associated conditionsinclude

• retroperitoneal fibrosis.
• Riedel's thyroiditis.
• pancreatitis.
• diabetes mellitus.

3. Presentation
4. Insidious onset of jaundice and pruritusoften occurs in the fourth to fifth decade.
5. RUQ pain, weight loss, fever, and fatigue may also occur with exacerbation of disease with gradual progression to liver failure.
6. Acute cholangitis is rareunless biliary tract manipulation has occurred.
7. ERCPis often diagnostic

• demonstrating the characteristic beaded appearanceof multiple intrahepatic and extrahepatic strictures.

5. Treatment

. Percutaneous or endoscopic balloon dilation

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• of dominant strictures may be useful for symptomatic relief of obstruction.

1. Ursodeoxycholic acid and cholestyramine

• may relieve symptoms, including pruritus.

1. An aggressive surgical approach

• is often advocated because of the insidious progression to secondary biliary cirrhosis and eventual liver failure.

2. Liver transplantationis indicated for severe intrahepatic involvement or advanced disease.
3. Biliary bypass(e.g., choledochojejunostomy or hepaticojejunostomy) may be used for isolated extrahepatic bile duct involvement.
4. Potential complications include

• cirrhosis.
• gallstone formation.
• cholangiocarcinoma, most frequently associated with ulcerative colitis.

1. Other causes of biliary stricture formation include

• chronic pancreatitis.
• postoperative stricturing of a biliary-enteric anastomosis.

VIII. Malignant Neoplasms of the Biliary Tract

1. Gallbladder cancer
2. Epidemiology
3. Primary gallbladder cancer is rare, although it is the most common malignant lesion of the biliary tract.
4. The mean ageat diagnosis is 65

• with a 3:1 female to male ratio.

1. Seventy to 90% of casesare associated with gallstones.

• However, only 0.4% of patients with gallstones develop gallbladder cancer.

1. Calcification of the gallbladder wall (porcelain gallbladder)

• is associated with a 25%–60% risk of carcinoma.
• is an indication for cholecystectomy.

2. Pathology
3. Eighty to 90% of gallbladder cancers

• are well-differentiated adenocarcinomas.

1. The most common site of metastases

• and direct tumor spread is the liver.

1. Lymphatic spread occurs sequentially

• to the cystic duct nodes, periportal nodes, and finally to the celiac and superior mesenteric nodes.

3. Presentation
4. The diagnosis

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• is most commonly made incidentallyat the time of cholecystectomy or laparotomy (90%).

1. Ninety percent of patients

• present with metastatic disease.

1. Laboratory findings

• are related to the extent and location of the tumor.

4. CT and magnetic resonance imaging (MRI)

• are useful for characterizing the extent of disease.

5. Tumor staging

• is outlined in Table 16-3.

6. Treatment

. Stage I

• Cholecystectomyalone is potentially curative.

1. Stages II–IV

• Radical cholecystectomyis indicated. This includes cholecystectomy, wedge resection of the liver with 2–3 cm margins around the gallbladder bed, and regional lymphadenectomy.

1. Stage V

• Palliative procedures to relieve obstruction such as biliary-enteric bypass procedures are performed.

1. If the cancer is discovered after laparoscopy,

• the trocar sites must be excisedbecause of a high likelihood of tumor implantation at these sites.

7. Overall 5-year survival

• is < 5%due to the preponderance of late presentation.
• Average survival currently is 6 months after diagnosis.

Table 16-3. Staging and Treatment of Gallbladder Adenocarcinoma

Stage Description Surgical Treatment Stage I Mucosal involvement only Cholecystectomy Stage II Involvement of muscularis layer of the gallbladder Radical cholecystectomy-cholecystectomy + wedge resection of the liver around the bed of the gallbladder + regional lymphadenectomy Stage III All layers of wall involved Stage IV Cystic node involvement Stage V Distant spread including adjacent organs and periportal nodes Palliative procedures (e.g., choledochojejunostomy)

1. Cholangiocarcinoma

• is defined as cancer of the bile ducts.

1. Epidemiology
2. Cholangiocarcinoma most commonly occurs

• between 50–70years.

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• with a higher incidence in males and people of southeast Asian origin.

1. Other risk factorsinclude

• sclerosing cholangitis.
• ulcerative colitis.
• choledochal cysts.
• chronic liver infections.
• congenital hepatic fibrosis.

2. Pathology
3. Tumors may be classified

• based on location by dividing the biliary tract into thirds (Figure 16-5).
• Tumors of the upper third are called Klatskin tumors.

1. Cholangiocarcinomas

• are primarily adenocarcinomas.
• tend to spread by direct extension, frequently involving the portal vein and hepatic artery.

Figure 16-5. Distribution of cholangiocarcinomas. (Adapted with permission from Greenfield LJ, Mulholland MW, Oldham KT, et al: Surgery: Scientific Principles and Practices, 2nd ed. Philadelphia, Lippincott-Raven, 1997, p 1061.)

3. Presentation
4. The classic clinical presentation

• is painless jaundice.
• Jaundice occurs in 90% of patients and is often associated with pruritus.

1. Bilirubinuria and acholic stools

• may also be present.

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• Anorexia and weight lossare markers of advanced disease.

1. Signsinclude

• icterus.
• hepatomegaly.
• possibly, a palpable gallbladder (Courvoisier's sign).
• possibly stigmata of cirrhosis (with advanced disease).

1. Symptoms and signs

• do not tend to fluctuate, as with choledocholithiasis.

1. Characteristic laboratory findingsinclude

• persistent elevations of bilirubin and alkaline phosphatase.

4. Radiologic evaluation
5. Cholangiography

• with PTC or ERCP is the gold standard for diagnosis.

1. A CT scan

• may further define a mass lesion and be used in staging.

1. The discovery of a focal bile duct stenosis

• in a patient without a history of biliary duct surgery is highly suggestive of a malignant process.

5. The staging of cholangiocarcinoma

• is outlined in Table 16-4.

6. Treatment

. Tumors in the lower third of the bile duct

• may require radical pancreatoduodenectomy (Whipple's operation).

1. Tumors in the middle third

• may require wide resectionand construction of a biliary-enteric anastomosis (e.g., hepaticojejunostomy).

1. Upper third lesions(Klatskin tumors)

• are often unresectable.
• If possible, a wide resectionof the mass including adjacent liver parenchyma with subsequent hepaticojejunostomy may be performed.

1. For unresectable disease

• palliative stenting is performed either percutaneously, endoscopically, or surgically.

Table 16-4. Staging of Cholangiocarcinoma

Stage I Tumor invading but not through the bile duct wall Stage II Tumor invading through the wall and involving perimuscular connective tissue Stage III Stage II plus local lymph node involvement Stage IV Tumor invading adjacent organs including liver, pancreas, duodenum, or vascular structures Stage V Distant metastatic spread

7. Prognosis

P.392

. The average patient survives

• less than 1 year after the diagnosis.

1. Overall 5-year survival

• is approximately 30%–40% after radical surgery for resectable lesions.

1. Klatskin tumors have the worst prognosis

• with an 80%–90% 5-year mortality rate owing to predominant unresectable nature at presentation.

1. Miscellaneous Biliary Pathologies
2. Congenital abnormalities
3. Biliary atresia(see BRS Surgical Specialties, Chapter 3)

• is the most common cause of persistent jaundice in the newborn.

1. Approximately 90% of people have complete obliterationof the biliary tree.
2. Therapeutic interventions include

• hepatic portoenterostomy(Kasai procedure), which is not possible with complete obliteration.
• hepatic transplantation.

2. Choledochal cyst

• is characterized by congenital segmental dilationof the extrahepatic biliary duct.
• may be related to ductal malformationthat causes inappropriate mixing of biliary and pancreatic juices, thus producing ductal wall damage.
• is more commonly seen in females.
• is associated with intrahepatic cysts(30%).

6. The classification of choledochal cysts (Types I–V) is outlined in Figure 16-6.
7. Presentation is variable.
8. Patients may present in childhood or adulthood.
9. Characteristics may include

• epigastric pain.
• fever.
• jaundice.
• cholangitis.

1. There is a significant risk of

• cholangiocarcinoma.

1. Treatment involvesexcision of the cyst, followed by biliary bypass.
2. Caroli's disease(Type V choledochal cyst)

• is characterized by congenital intrahepatic ductal dilationoften associated with stone formation and cholangitis.

P.393

• may be associated with a familial syndrome characterized by congenital hepatic fibrosisand medullary sponge kidney.

Figure 16-6. Anatomical classification of choledochal cysts. (A) Dilation of common hepatic and common bile duct, with cystic duct entering the cyst; most common type. (B)Lateral saccular cystic dilation. (C) Choledochocele represented by an intraduodenal cyst. (D) Multiple extrahepatic cysts, intrahepatic cysts, or both. (E) Single or multiple intrahepatic cysts; Caroli's disease. (Adapted with permission from Blackbourne LH: Surgical Recall. Baltimore, Williams & Wilkins, 1994, p 219.)

1. Hemobilia

• is defined as bleeding into the biliary tree.

1. Most cases in the United States

• are due to traumaor iatrogenic injury.

2. The classic presentationincludes

• biliary colic.
• jaundice.
• guaiac-positive stools.

3. Diagnosis

• often requires arteriography.

4. Treatment of persistent hemobiliamay include

• embolization.
• surgical ligation.

1. Ascariasis

• is a parasitic infection that can produce ductal obstruction.

P.394

1. Mirizzi syndrome

• is a rare cause of biliary duct obstructiondue to gallbladder pathology without common duct stone involvement.

1. A large stone within the gallbladdermay compress the CBD because of anatomic proximity.
2. Local spread of inflammationfrom the gallbladder to the bile duct may also result in ductal narrowing.

P.395

Review Test

Directions: Each of the numbered items or incomplete statements in this section is followed by answers or by completions of the statement. Select the ONE lettered answer or completion that is BEST in each case.

1. A 37-year-old woman presents 1 week after a laparoscopic cholecystectomy with complaints of fever, abdominal pain, back pain, and vomiting. Vital signs include: temperature, 38.7° C; blood pressure, 110/80 mm Hg; pulse, 120/min; respiration, 18/min. There is some tenderness in the right upper quadrant (RUQ) with voluntary guarding but no rebound tenderness or distension. Laboratory results include: white blood cells = 18,000; hematocrit = 34; platelets = 572; Na⁺130; K⁺ = 3.3; Cl^- = 95; CO₂ = 29; blood urea nitrogen = 31; creatinine = 0.9; total bilirubin = 8.1; conjugated bilirubin = 6.8; alkaline phosphatase = 700; aspartate aminotransferase (AST) = 150; alanine aminotransferase (ALT) = 209; and lipase = 40. RUQ ultrasound was performed and revealed a dilated common bile duct, no gallbladder, and no significant fluid collections. Which of the following is the most appropriate next step in the management of this patient?

(A) Antibiotics, fluid resuscitation, and observation

(B) Radionuclide hepatobiliary iminodiacetic acid (HIDA) scan

(C) Plain radiograph of the abdomen

(D) Take the patient to the operating room

(E) Endoscopic retrograde cholangiopancreatography (ERCP)

1–E. In addition to iatrogenic biliary tree injury, another complication of laparoscopic cholecystectomy is retained common duct stones, which can lead to obstruction and eventually cholangitis. Endoscopic retrograde cholangiopancreatography (ERCP) would be the examination of choice for evaluation. Not only can the common duct obstruction be identified, but therapeutic measures can be undertaken (e.g., sphincterotomy and stone extraction). If these measures are unsuccessful, surgery may be indicated but would not generally be the initial therapy. Fluid resuscitation and antibiotics are important but the key to initial therapy is to relieve the obstruction. A HIDA scan would help to identify the lesion but ERCP provides better visualization in addition to the potential therapeutic role.

2. A 54-year-old, African-American man with a history of hypertension and sickle cell trait presents with a 24-hour history of nausea, vomiting, and abdominal pain. The patient describes the pain as crampy in nature and worse after eating. His temperature is 38.4° C, blood pressure is 150/96 mm Hg, pulse 105/min, and respiration 6/min. His abdomen is tender in the right upper quadrant (RUQ) and the patient cannot take a deep breath when the examiner is palpating the RUQ. Laboratory results include: white blood cells = 15,000; hematocrit = 39; platelets = 112; total bilirubin = 1.0; conjugated bilirubin = 0.1; and alkaline phosphatase = 62. Which of the following is the most appropriate diagnostic study in the initial evaluation of this patient?

(A) Radionuclide hepatobiliary iminodiacetic acid (HIDA) scan

(B) Plain films of the abdomen

(C) RUQ ultrasound

(D) Computed tomography (CT) scan of the abdomen

(E) Magnetic resonance cholangiopancreatography (MRCP)

2–C. This clinical picture is consistent with acute cholecystitis. Right upper quadrant (RUQ) ultrasound is the examination of choice for initial evaluation of a patient with possible cholecystitis. Plain films of the abdomen are rarely useful in this situation because only 5% of gallstones appear on such radiographs. A HIDA scan may be used when ultrasound findings are inconclusive; however, it should not be performed as an initial study. Magnetic resonance cholangiopancreatography (MRCP) provides excellent visualization of the biliary tree but it is still limited by cost and inconvenience. Computed tomography (CT) scans are generally not useful in the straightforward evaluation of cholecystitis.

3. A 62-year-old, white woman with a history of hypertension, hypercholesterolemia, and peptic ulcer disease presents to the clinic after a routine laparoscopic cholecystectomy. Surgical pathology reveals a 3 mm × 3 mm × 4 mm adenocarcinoma in the wall of the gallbladder that extends into but not through the muscularis layer. A chest radiograph and abdominal computed tomography (CT) scan show no evidence of metastases. Which of the following is the appropriate treatment for this patient's disease?

(A) The patient has been treated appropriately and no further surgery is necessary

(B) Perform a choledochojejunostomy as a palliative measure for relief of potential obstructive symptoms

(C) Perform a wedge resection of the liver around the gallbladder and a regional lymphadenectomy

(D) Perform a pancreaticoduodenectomy to obtain wide margins around the gallbladder

(E) Perform a wedge resection of the liver around the gallbladder, a regional lymphadenectomy, and excision of all trocar sites

3–E. The appropriate treatment for this gallbladder cancer is a radical cholecystectomy. The patient has already had her gallbladder removed and therefore needs to have the procedure completed with a liver resection with 2–3 cm margins around the gallbladder and a regional lymphadenectomy. In addition, the laparoscopic trocar sites must also be excised to prevent tumor seeding. Wider resections such as a Whipple's operation or palliative procedures are not indicated at this time.

4. A 68-year-old, Vietnamese man with a history of hypertension, asthma, and arthritis presents with complaints of pruritus that has gradually worsened over the past 3 weeks. Most recently he also reports some nausea and vomiting. He reports having lost approximately 15 pounds in the past month and attributes this to his nausea and vomiting. On physical examination his temperature is 37.6° C, blood pressure is 160/90 mm Hg, pulse is 87/min, and respirations are 13/min. He is a thin male in no acute distress. Laboratory results include: white blood cells = 8.2; hematocrit = 42; platelets = 208; Na⁺= 132; K⁺ = 4.0; blood urea nitrogen = 32; creatinine = 0.7; total bilirubin = 12.0; conjugated bilirubin = 10.8; alkaline phosphatase = 812; amylase = 35; lipase = 60; aspartate aminotransferase (AST) = 212; and alanine aminotransferase (ALT) = 230. A right upper quadrant (RUQ) ultrasound was performed before the patient's referral and reveals no gallstones, gallbladder wall thickening, or common duct dilation. There is, however, intrahepatic ductal dilation. Which of the following is the most likely diagnosis?

(A) Cholangitis

(B) Cholangiocarcinoma

(C) Pancreatitis

(D) Pancreatic cancer

(E) Choledocholithiasis

4–B. The presentation of a patient with painless jaundice and no history of abdominal surgery is consistent with either pancreatic cancer or cholangiocarcinoma. The finding of intrahepatic ductal dilation suggests obstruction of the biliary system above the common duct and therefore makes pancreatic cancer less likely. Cholangiocarcinoma and more specifically, tumors involving the upper third of the biliary tree (Klatskin tumors), is the most likely etiology of these findings. Cholangitis often produces a fever and this patient is not febrile.

5. A 42-year-old man with a history of diabetes mellitus and inflammatory bowel disease presents because of a 3-day history of worsening itching. The patient denies any fever, chills, or abdominal pain but does report two episodes of vomiting. The patient also claims to have lost 12 pounds in the last 2 months. After further questioning, the patient reports having had several similar episodes in the past that resolved spontaneously. On physical examination he is jaundiced. Rectal examination is trace positive for blood. Laboratory results include: white blood cell count = 10.0; hematocrit = 33; platelets = 256; blood urea nitrogen = 28; creatinine = 1.1; total bilirubin = 9.0; conjugated bilirubin = 7.6; alkaline phosphatase = 1000; aspartate aminotransferase (AST) = 78; alanine aminotransferase (ALT) = 152; prothrombin time (PT) = 12.2; and partial thromboplastin time (PTT) = 21.8. Which of the following is the most likely diagnosis?

(A) Primary sclerosing cholangitis (PSC)

(B) Choledocholithiasis

(C) Biliary colic

(D) Cholangiocarcinoma

(E) Cholangitis

5–A. This is a characteristic presentation of primary sclerosing cholangitis (PSC). The patient is relatively young and therefore malignancy is less likely. Furthermore, he describes a history of fluctuating symptoms, which is characteristic of PSC and not of cholangiocarcinoma. The lack of pain makes the diagnosis of biliary colic and choledocholithiasis less likely. Of concern is the patient's anemia, guaiac-positive stools, and weight loss. This may be due to colon cancer, which is an increased risk in patients with PSC and ulcerative colitis.

6. A 75-year-old woman has been in the intensive care unit for the past 3 weeks because of a 20% burn to her back and left arm. Her course has been complicated by adult respiratory distress syndrome (ARDS) and a non-Q wave myocardial infarction. Her cardiovascular status is currently tenuous. Over the past week the patient's white blood cell count has been rising and she grimaces with palpation of the abdomen. Her current white blood cell count is 18,000 with a left shift. Her other laboratory results are unremarkable except for a hematocrit of 29. Current vital signs include: temperature, 38.4° C; blood pressure, 88/48 mm Hg; pulse, 120/min; and respiration, 26/min. Abdominal films are unremarkable. A right upper quadrant (RUQ) ultrasound was performed and revealed a distended gallbladder, thickened gallbladder wall, pericholecystic fluid, no gallstones, and normal common bile ducts. Which of the following is most appropriate in the initial management of this patient?

(A) Endoscopic retrograde cholangiopancreatography (ERCP) with stone removal

(B) Emergent open cholecystectomy

(C) Cholecystostomy

(D) Laparoscopic cholecystectomy

(E) Intravenous (IV) antibiotics and observation

6–C. Acalculous cholecystitis characteristically affects critically ill patients. An appropriate management strategy in an unstable patient with cholecystitis is cholecystostomy that involves percutaneous placement of a catheter in the gallbladder to provide for drainage until the patient is stable enough to tolerate formal cholecystectomy. With simple antibiotics and observation serious morbidity and mortality can affect up to 60% of these patients. On the other hand, the patient is not well enough to tolerate the stress of surgery, so a temporizing measure is needed. Endoscopic retrograde cholangiopancreatography (ERCP) is not indicated in the setting of cholecystitis.

7. A 56-year-old woman with a history of hypertension and gout presents with a 3-day history of worsening abdominal pain, back pain, nausea, and vomiting. The patient claims she has had a history of crampy abdominal pain after eating for almost 1 year but most recently she has been suffering from constant pain. Her temperature is 36.7° C, blood pressure is 168/85 mm Hg, and pulse is 92/min. She is tender to palpation in the midepigastric region and right upper quadrant (RUQ). Laboratory results include: white blood cell count = 10,000; hematocrit = 42; platelets = 210; Na⁺= 138; K⁺ = 3.4; Cl^- = 96; CO₂ = 29; blood urea nitrogen = 34; creatinine = 1.3; total bilirubin = 1.2; conjugated bilirubin = 0.3; aspartate aminotransferase (AST) = 50; alanine aminotransferase (ALT) = 80; alkaline phosphatase = 210; amylase = 400; and lipase = 566. Abdominal films are consistent with an ileus. RUQ ultrasound reveals sludge, no wall thickening, no pericholecystic fluid, and a normal bile duct. Which of the following is the most likely cause of this patient's pathology?

(A) Obstruction of the cystic duct by a gallstone

(B) A malignancy obstructing the common hepatic duct

(C) Idiopathic inflammatory strictures of the bile ducts

(D) Passage of a gallstone through the ampulla of Vater

(E) Obstruction and bacterial infection of the biliary tree

7–D. The most common cause of pancreatitis in the United States is gallstone disease, including passage of a gallstone. Evidence for this includes the long history of biliary colic, which suggests calculous biliary disease. Furthermore, the elevation in alkaline phosphatase suggests recent irritation to the bile ducts, such as that caused by stone passage. In addition, the right upper quadrant (RUQ) ultrasound findings include the precursor to gallstones in the gallbladder as well as a normal duct, which weighs against other diagnoses, including primary sclerosing cholangitis (PSC), cholangitis, and cholangiocarcinoma. There was also no evidence on ultrasound of active biliary tree obstruction.

8. A 54-year-old, white woman presents with a 48-hour history of worsening crampy abdominal pain. Her pain is exacerbated by eating and is associated with nausea and vomiting. Her temperature is 38.7° C, pulse is 101/min, blood pressure is 130/82 mm Hg, and respiration is 19/min. Her abdomen is tender in the right upper quadrant (RUQ) and she cannot take a deep breath when the examiner palpates in the RUQ. She has no peritoneal signs. Laboratory results include: white blood cells = 16,000; hematocrit = 38; platelets = 316; Na⁺= 134; K⁺ = 3.6; Cl^- = 101; CO₂ = 22; blood urea nitrogen = 21; creatinine = 1.0; total bilirubin = 2.0; conjugated bilirubin = 0.8; alkaline phosphatase = 67; aspartate aminotransferase (AST) = 62; alanine aminotransferase (ALT) = 61; and amylase < 30. RUQ ultrasound reveals a distended gallbladder with pericholecystic fluid and multiple echogenic foci in the gallbladder. The common duct appears normal. Which of the following is the most appropriate next step in the management of this patient?

(A) Exploratory laparotomy

(B) Computed tomography (CT) scan of the abdomen

(C) Laparoscopic cholecystectomy

(D) Cholangiopancreatography

(E) Hepatobiliary iminodiacetic acid (HIDA) scan

8–C. The history of colicky pain worsened by eating is classic for cholecystitis. On physical examination the patient has a positive Murphy's sign. Her laboratory tests show evidence of infection but her normal bilirubin and alkaline phosphatase suggest no common duct involvement. The right upper quadrant (RUQ) ultrasound visualizes stones in the gallbladder. The next step for this patient is a cholecystectomy to remove her gallbladder. Further diagnostic tests, such as a HIDA or computed tomography (CT) scan, are not necessary because of the clear evidence of cholecystitis. Endoscopic retrograde cholangiopancreatography (ERCP) is not necessary because there is no suggestion of common duct pathology.

9. A 68-year-old, diabetic, hypertensive, African-American man was found to have gallstones on an abdominal computed tomography (CT) scan performed as part of a metastatic work-up. The man denies any symptoms except for occasional heartburn, which is treated medically. His vitals signs include: temperature, 37.1° C; blood pressure, 160/90 mm Hg; pulse, 87/min; and respiration, 12/min. His abdomen is soft, nontender, and nondistended. He has normoactive bowel sounds. Which of the following is an appropriate statement regarding the management of this patient?

(A) The patient must have a cholecystectomy

(B) This patient is at no increased risk of developing cholecystitis, versus a diabetic patient without gallstones

(C) If the patient had vague abdominal pain, a laparoscopic cholecystectomy may be helpful

(D) If the patient develops symptoms suggestive of cholecystitis, a CT scan should be repeated

(E) A laparoscopic cholecystectomy for acute cholecystitis is contraindicated in this patient because of the diabetes

9–C. Surgery for asymptomatic gallstones is not absolutely indicated. The patient's age and diabetes may lead many surgeons to perform a cholecystectomy, but these are relative indications. On the other hand, a cholecystectomy could help this patient, especially if there is a history of vague complaints. A right upper quadrant (RUQ) ultrasound would be the test of choice for initial evaluation if symptoms suggestive of cholecystitis were to develop, not a computed tomography (CT) scan. A laparoscopic approach is not contraindicated because of diabetes. The presence of gallstones in any population is associated with an increased risk of cholecystitis versus those without stones.

10. A 4-year-old boy was in his usual state of good health until approximately 1 month ago when he developed cholangitis. As part of the work-up an endoscopic retrograde cholangiopancreatography (ERCP) was performed and revealed segmental dilation of the common bile duct. A computed tomography (CT) scan was also performed and revealed several intrahepatic cysts, the segmental dilation of the common duct, and no masses. Which of the following is the most appropriate recommendation for this patient?

(A) This is a normal anatomic variant and no surgery is indicated

(B) The patient should have a repeat ERCP to perform a sphincterotomy

(C) The patient should have a cholecystectomy to prevent gallbladder involvement

(D) The patient should have the region of segmental dilation removed followed by biliary reconstruction

(E) The patient should have the dilated region excised, the head of the pancreas removed, and then a biliary bypass

10–D. The most likely diagnosis in this case is a choledochal cyst. There is an increased risk of cholangiocarcinoma associated with this congenital abnormality, therefore the recommendation is to have the cyst excised and the biliary tree reconstructed, often with a Roux-en-Y choledochojejunostomy or hepaticojejunostomy. There is no pathology in the pancreas, thus the head of the pancreas should not be removed with the cyst. Additionally, a sphincterotomy would not relieve the obstruction in this patient.

11. A 49-year-old, obese man presents to the emergency room with a 24-hour history of fever, chills, and abdominal pain. He describes crampy pain in the midepigastric region with radiation to the right shoulder. He also reports some associated nausea and vomiting. His vital signs include: temperature, 38.9° C; blood pressure, 156/96 mm Hg; pulse, 99/min; and respiration, 16/min. His sclera are anicteric, his heart is regular, and his lungs are clear to auscultation except for occasional wheezes. Laboratory results include: white blood cells = 14,000; hematocrit = 42; platelets = 221; Na⁺= 132; K⁺ = 3.3; Cl = 100; CO₂ = 21; blood urea nitrogen = 20; creatinine = 1.5; glucose = 266; total bilirubin = 1.2; conjugated bilirubin = 0.1; alkaline phosphatase = 242; and amylase = 30. A right upper quadrant (RUQ) ultrasound was attempted but because of the patient's size the gallbladder could not be adequately visualized. Which of the following is the most appropriate next step in the management of this patient?

(A) Endoscopic retrograde cholangiopancreatography (ERCP)

(B) Hepatobiliary iminodiacetic acid (HIDA) scan

(C) Oral cholecystogram

(D) Percutaneous transhepatic cholangiography

(E) The patient should be taken to the operating room

11–B. This patient has a presentation that is highly suggestive of acute cholecystitis. When a right upper quadrant (RUQ) ultrasound is equivocal, the next test of choice is nuclear imaging of the biliary tree, such as a HIDA scan. An oral cholecystogram can give the same information as the HIDA scan but it is much more time-consuming and the reliability is lower owing to the patient's vomiting. Cholangiography is not indicated before the HIDA scan but may be necessary if common duct obstruction is diagnosed. Surgery may be necessary, but not before confirming the diagnosis in this setting.

12. A 71-year-old, white man with a history of diabetes mellitus, coronary artery bypass surgery, and a 80 pack-year smoking history presents to the office complaining of severe itching that is not improved with home remedies. He denies fever, chills, or abdominal pain. On physical examination he is a thin, jaundiced, elderly man in no acute distress. Vital signs include: temperature, 36.8° C; pulse, 67/min; blood pressure, 145/67 mm Hg; respiration, 13/min. Laboratory results include: white blood cells = 8000; hematocrit = 32; platelets = 201; Na⁺= 134; K⁺ = 3.6; Cl^- = 101; CO₂ = 24; blood urea nitrogen = 31; creatinine = 1.6; glucose = 278; total bilirubin = 11.1; conjugated bilirubin = 8.9; alkaline phosphatase = 1002; and amylase = 100. A computed tomography (CT) scan is performed and reveals dilated intrahepatic ducts but no visualization of the extrahepatic ducts because of a 2 cm × 2 cm × 3 cm mass that appears to involve the common hepatic duct and cystic duct. There is no obvious lymphadenopathy. Which of the following is the most appropriate next step in the management of this patient?

(A) Computed tomography (CT) scan of the chest and a bone scan

(B) Percutaneous transhepatic cholangiography (PTC)

(C) Exploratory laparotomy

(D) Biliary ultrasound

(E) Radical pancreaticoduodenectomy

12–B. This patient is presenting with obstructive jaundice from a mass involving the common hepatic duct and the cystic duct. This is most likely due to gallbladder cancer with extension down the cystic duct but may also be due to cholangiocarcinoma. The next step in evaluation would be cholangiography. Percutaneous transhepatic cholangiography (PTC) is preferred because it can characterize the extent of involvement of the proximal biliary tree, which is important for surgical planning. In addition, a drain may be left in the intrahepatic bile ducts or a stent may be placed to decompress the dilated biliary system. After this has been performed clinical staging and surgical planning can be accomplished. Although computed tomography (CT) may further define a mass lesion and be used in staging, it is not as effective as PTC at outlining the degree of biliary tree involvement. Exploratory laparotomy is not indicated in this setting.

Directions: Each set of matching questions in this section consists of a list of four to twenty-six lettered options followed by several numbered items. For each numbered item, select the appropriate lettered option(s). Each lettered option may be selected once, more than once, or not at all.

Questions 13–15

1. Distended gallbladder with thickened wall and pericholecystic fluid. Multiple echogenic foci in the gallbladder. Normal common bile duct and liver.
2. Normal gallbladder except for a single echogenic foci. Common bile duct distended. Normal liver.
3. Normal gallbladder. Extrahepatic bile ducts not visualized. Intrahepatic ducts distended.
4. Normal gallbladder. Normal liver and bile ducts.
5. Focal stricture of the distal common bile duct with proximal dilation.

Match the right upper quadrant (RUQ) ultrasound result most consistent with the clinical presentation.

13. A 56-year-old, white man with a history of tobacco abuse, hypertension, recent unintentional 80-lb weight loss, and squamous cell cancer of the lung presents with a 24-hour history of right upper quadrant (RUQ) abdominal pain, nausea, and vomiting. He has had similar pains over the past 2 weeks, mostly after eating, but they had resolved spontaneously. The patient denies fever and chills. His temperature is 36.7° C. Physical examination findings are significant for scleral icterus and RUQ tenderness to palpation. Significant laboratory findings include white blood cells = 7800, total bilirubin = 6.8, conjugated bilirubin = 5.6, and alkaline phosphatase = 670. (SELECT 1 RESULT)

13-B. This patient is presenting with choledocholithiasis most likely related to cholesterol stones formed in the gallbladder caused by his rapid weight loss. Therefore one would expect to see common duct distension caused by the cholesterol stone. In addition, one might expect to find evidence of stone formation in the gallbladder, such as another stone or sludge.

14. A 53-year-old, morbidly obese, white woman with a history of diabetes presents with a 36-hour history of worsening crampy midepigastric and right upper quadrant (RUQ) abdominal pain. She claims the pain was initially bad only after meals but has since become constant. She also complains of nausea, vomiting, and fever. Her temperature is 38.5° C. Her physical examination is significant for anicteric sclera, RUQ pain, and inspiratory arrest with deep palpation in the RUQ. Significant laboratory findings include white blood cells = 14,000, K⁺= 3.3, Cl^- = 96, CO₂ = 30, total bilirubin = 1.1, and alkaline phosphatase = 64. (SELECT 1 RESULT)

14-A. This patient is presenting with classic acute cholecystitis. Characteristic findings of acute cholecystitis on right upper quadrant (RUQ) ultrasound could reveal gallstones, a distended gallbladder, pericholecystic fluid, and thickening of the gallbladder wall.

15. A 58-year-old, diabetic, African-American man with a history of hypertension and hypercholesterolemia presents with a 48-hour history of progressively worsening constant right upper quadrant (RUQ) pain, fever, nausea and vomiting. Vital signs include: temperature, 39.1° C; pulse, 120/min; blood pressure, 100/59 mm Hg; respiration, 21/min. Physical examination is significant for icteric sclera and RUQ tenderness. Significant laboratory findings include white blood cells = 17,000; alkaline phosphatase = 670; total bilirubin = 7.8; conjugated bilirubin = 6.5; aspartate aminotransferase (AST) = 350; alanine aminotransferase (ALT) = 390; and amylase = 45. (SELECT 1 RESULT)

15-B. This patient is presenting with cholangitis. Characteristic findings include fever; right upper quadrant (RUQ) pain; icteric sclera (Charcot's triad); and elevated bilirubin, alkaline phosphatase, and transaminases. The RUQ ultrasound should show evidence of biliary obstruction, the dilated ducts. The ultrasound picture of cholangitis and choledocholithiasis are very similar but the clinical picture can easily separate the two. The most likely cause of cholangitis in this setting is choledocholithiasis.