32: Biliary System

Question 1

Which stones, if found in the common bile duct, are considered secondary common bile duct stones?

Answer 1

Cholesterol stones and black stones

Question 2

What is the treatment for gallbladder adenocarcinoma if there is involvement beyond muscle but it is still resectable?

Answer 2

Formal resection of segments IVb and V

[UpToDate: For patients with gallbladder that extends beyond the mucosa, >T1a, the benefit of more radical surgery has been controversial. Randomized trials comparing simple cholecystectomy with radical surgery for gallbladder cancer have not been performed; all available studies are retrospective series. Some of these series, but not all, link better outcomes with more radical surgery.

In another Japanese series of 1686 resected gallbladder cancers from 172 major hospitals, survival rates were significantly better for patients undergoing radical resection compared with patients with simple cholecystectomy (three-year survival: 66% vs 14%, 5-year survival: 51% vs 6%).

At the Mayo Clinic, 22 of 40 patients undergoing potentially curative resection had a simple cholecystectomy, while the remainder had a radical procedure. Although 5-year overall survival rates were similar (33% vs 32%), median survival in patients undergoing radical resection was significantly better (3.6 vs 0.8 years), and for those with transmural extension or nodal metastasis, the only 5-year survivors were those who had undergone extended cholecystectomy.

On the other hand, in a series of 104 patients treated at Memorial Sloan-Kettering over a 12-year period, major hepatectomy, resection of adjacent organs other than the liver, and common bile duct excision increased perioperative morbidity and were not associated with better survival. The authors concluded that major hepatic resection (including excision of the common bile duct) was appropriate, when necessary, to clear disease, but not mandatory in all cases.

Recommendation - Patients with a preoperative diagnosis of potentially resectable, localized gallbladder cancer should be offered definitive resection which involves en bloc resection of the gallbladder and a margin of underlying liver (nonanatomic or anatomic resection), and resection of the regional lymph nodes or extrahepatic biliary ducts depending upon the extent of disease identified intraoperatively. A right hepatic lobectomy may be appropriate in selected patients (eg, tumor of the gallbladder neck, tumor involving the right portal triad).]

Question 3

What is the treatment for cholangitis?

Answer 3

• Fluid resuscitation
• Antibiotics
• Emergent ERCP with sphincterotomy and stone extraction (if applicable)
• If ERCP fails, place PTC tube to decompress the biliary system

[If the cause is an infected PTC tube, change the tube]

Question 4

Cystic veins drain into which vein?

Answer 4

The right branch of the portal vein

Question 5

What is caused by abnormal reflux of pancreatic enzymes during uterine development?

Answer 5

Choledochal cysts

Question 6

What percent of gallstones are radiopaque?

Answer 6

Only 10%

[Wikipedia: Cholesterol stones vary from light yellow to dark green or brown or chalk white and are oval, usually solitary, between 2 and 3 cm long, each often having a tiny, dark, central spot. To be classified as such, they must be at least 80% cholesterol by weight (or 70%, according to the Japanese- classification system). Between 35% and 90% of stones are cholesterol stones.

Bilirubin (“pigment”, “black pigment”) stones are small, dark (often appearing black), and usually numerous. They are composed primarily of bilirubin (insoluble bilirubin pigment polymer) and calcium (calcium phosphate) salts that are found in bile. They contain less than 20% of cholesterol (or 30%, according to the Japanese-classification system). Between 2% and 30% of stones are bilirubin stones.

Mixed (“Brown Pigment”) stones typically contain 20–80% cholesterol (or 30–70%, according to the Japanese- classification system). Other common constituents are calcium carbonate, palmitate phosphate, bilirubin and other bile pigments (calcium bilirubinate, calcium palmitate and calcium stearate). Because of their calcium content, they are often radiographically visible. They typically arise secondary to infection of the biliary tract which results in the release of β-glucuronidase (by injured hepatocytes and bacteria) which hydrolyzes bilirubin glucuronides and increases the amount of unconjugated bilirubin in bile. Between 4% and 20% of stones are mixed.]

Question 7

What is the approach to intraoperative common bile duct injury that is greater than 50% of the circumference of the bile duct and cannot be primarily repaired?

Answer 7

Hepaticojejunostomy or choledocojejunostomy

Question 8

What is the treatment for granular cell myoblastoma of the gallbladder?

Answer 8

Cholecystectomy

[Granular cell myoblastoma = A benign neuroectoderm tumor of the gallbladder]

Question 9

What is the first symptom of gallbladder adenocarcinoma?

Answer 9

Jaundice (bile duct invasion with obstruction)

[RUQ pain after that]

Question 10

What is the treatment for Mirizzi syndrome?

Answer 10

Cholecystectomy

[May need hepaticojejunostomy for hepatic duct stricture]

Question 11

What type of choledochal cyst is partially intrahepatic?

Answer 11

Type IV

Question 12

The cystic artery branches off of which artery?

Answer 12

Right hepatic artery

Question 13

What is the most sensitive test for cholecystitis?

Answer 13

Cholecystokinin cholescintigraphy (CCK-CS test)

[Cholescinitgraphy is a HIDA scan. It is 97% sensitive for acute cholecystitis (U/S is 95% sensitive).]

Question 14

What is the treatment for cholangiocarcinoma in the lower 1/3 of the biliary system?

Answer 14

Whipple

Question 15

What is the treatment for choledochal cysts?

Answer 15

Cyst excision with hepaticojejunostomy and cholecystectomy

[UpToDate: Patients with type I, II, or IV cysts usually undergo surgical resection of the cysts due to the significant risk of malignancy, provided they are good surgical candidates. Type I and IV cysts should be completely resected with creation of a Roux-en-Y hepatojejunostomy. Serial sections from the cyst wall should be examined by the pathologist to look for any malignant changes. Type II cysts can be treated with simple cyst excision. Type III cysts (choledochoceles) require treatment if they are symptomatic and may be managed with sphincterotomy or endoscopic resection. Treatment for type V cysts is largely supportive and is aimed at dealing with problems such as recurrent cholangitis and sepsis. Type V cysts can be difficult to manage, and some patients with type V cysts eventually require liver transplantation.]

Question 16

What is the overall 5-year survival of gallbladder adenocarcinoma?

Answer 16

5%

[UpToDate: Gallbladder cancer (GBC) is an uncommon but highly fatal malignancy; fewer than 5000 new cases are diagnosed each year in the United States. The majority are found incidentally in patients undergoing exploration for cholelithiasis; a tumor will be found in 1% to 2% of such cases. The poor prognosis associated with GBC is thought to be related to advanced stage at diagnosis, which is due both to the anatomic position of the gallbladder, and the vagueness and nonspecificity of symptoms.

Surgery is the only potentially curative treatment for gallbladder cancer. Surgical series report long-term survival rates of 85% to 100% for patients with early-stage (T1) disease. Unfortunately, fewer than 10% of symptomatic patients and only approximately 20% of patients with incidentally diagnosed gallbladder cancer have early-stage disease. The poor prognosis associated with GBC is related to the often advanced stage at diagnosis.]

Question 17

What are 2 late complications of cholangitis?

Answer 17

1. Stricture
2. Hepatic abscess

Question 18

What are 3 risks of ERCP?

Answer 18

1. Bleeding
2. Pancreatitis
3. Perforation

Question 19

What is the overall 5-year survival rate of cholangiocarcinoma?

Answer 19

20%

Question 20

What is the treatment for gallbladder adenocarcinoma if muscle is involved but nothing beyond?

Answer 20

Wedge resection of segments IVb and V

[UpToDate: Patients with stage T1b disease may benefit from a more radical approach, given that T1b tumors are associated with a higher incidence of lymph node metastases compared with T1a tumors (15% versus 2.5%). Some investigators have shown a median survival advantage of over three years for extended versus cholecystectomy alone for T1b cancers (9.85 vs 6.42 years, respectively). Extended cholecystectomy (cholecystectomy including a rim of liver tissue) should be performed for medically-fit patients who have tumors that invade the muscular layer (T1b).

The optimal approach to T1b disease is more controversial. If there is no contraindication to surgery, extended resection is reasonable for T1b gallbladder cancer. At least two retrospective studies comparing cholecystectomy alone versus extended cholecystectomy for T1b tumors found no significant difference in overall survival (with up to 87% 10-year survival). Others have reported improved survival with re-resection. Furthermore, a number of studies describe high rates of residual disease upon re-resection, with lymph node metastases in 12% to 20% and 0% to 13% with liver involvement. Two reports describe up to a 50% to 60% locoregional recurrence rate after cholecystectomy alone for T1b disease. The authors of both reports concluded that gallbladder cancer is a locally aggressive disease and even early-stage disease warrants extended resection. Finally, as noted above, decision analysis showed a median survival advantage of over three years for extended versus simple cholecystectomy (9.85 vs 6.42 years).

Question 21

What type of choledochal cyst is totally intrahepatic?

Answer 21

Type V

[Known as Caroli’s disease]

Question 22

What are 5 risk factors for cholangiocarcinoma?

Answer 22

1. C. sinensis infection
2. Ulcerative colitis
3. Choledochal cysts
4. Primary sclerosing cholangitis
5. Chronic bile duct infection

Question 23

Which bacteria produces beta-glucuronidase, deconjugating bilirubin and forming calcium bilirubinate?

Answer 23

E. Coli

Question 24

What are the 4 main causes of pigmented (black) gallstones?

Answer 24

1. Hemolytic disorders
2. Cirrhosis
3. Ileal resection (loss of bile salts)
4. Chronic TPN

[Wikipedia: Cholesterol stones vary from light yellow to dark green or brown or chalk white and are oval, usually solitary, between 2 and 3 cm long, each often having a tiny, dark, central spot. To be classified as such, they must be at least 80% cholesterol by weight (or 70%, according to the Japanese- classification system). Between 35% and 90% of stones are cholesterol stones.

Bilirubin (“pigment”, “black pigment”) stones are small, dark (often appearing black), and usually numerous. They are composed primarily of bilirubin (insoluble bilirubin pigment polymer) and calcium (calcium phosphate) salts that are found in bile. They contain less than 20% of cholesterol (or 30%, according to the Japanese-classification system). Between 2% and 30% of stones are bilirubin stones.

Mixed (“Brown Pigment”) stones typically contain 20–80% cholesterol (or 30–70%, according to the Japanese- classification system). Other common constituents are calcium carbonate, palmitate phosphate, bilirubin and other bile pigments (calcium bilirubinate, calcium palmitate and calcium stearate). Because of their calcium content, they are often radiographically visible. They typically arise secondary to infection of the biliary tract which results in the release of β-glucuronidase (by injured hepatocytes and bacteria) which hydrolyzes bilirubin glucuronides and increases the amount of unconjugated bilirubin in bile. Between 4% and 20% of stones are mixed.]

Question 25

How is hemobilia diagnosed?

Answer 25

Angiogram

Question 26

What is the treatment for biliary strictures that are due to ischemia or pancreatitis?

Answer 26

Choledochojejunostomy [Best long-term solution]

Question 27

What anatomical structures form the lateral, medial, and superior borders of the Triangle of Calot (Hepatobiliary triangle)?

Answer 27

* Lateral border: Cystic duct * Medial border: Common hepatic duct * Superior border: Liver

Question 28

Do dissolution agents such as monoctanoin work on pigmented stones?

Answer 28

No [Monoctanoin is a monoglyceride used to dissolve gallstones that are caused by cholesterol.]

Question 29

What is the risk of gallbladder cancer in a patient with porcelain gallbladder?

Answer 29

15% [These patients need a cholecystectomy]

Question 30

Thickened nodules of mucosa and muscle associated with Rokitansky-Aschoff sinuses in the gallbladder are called what?

Answer 30

Adenomyomatosis [Not premalignant and does not cause stones but can cause RUQ pain] [UpToDate: Adenomyomatosis is an abnormality of the gallbladder characterized by overgrowth of the mucosa, thickening of the muscle wall, and intramural diverticula. Despite the name, this condition does not involve any adenomatous changes in the gallbladder epithelium. While generally not considered to be a premalignant condition, adenomyomatosis is sometimes encountered in gallbladders resected for cancer, which has led to the suggestion that it is a premalignant condition. However, the association and its magnitude remain unclear. There is, however, a clear association of adenomyomatosis with cholelithiasis, particularly with the segmental type, which was reported in 89% of patients with segmental adenomyomatosis in one series. Adenomyomatosis of the gallbladder is less common than cholesterolosis in most reports, with some exceptions. In one report, for example, only 103 cases of adenomyomatosis were found in over 10,000 cholecystectomies (1%). This is compared with the much higher prevalence of cholesterolosis (9% to 26%). Adenomyomatosis is more common in women.]

Question 31

What is the treatment for brown (pigmented) gallstones which are primarily formed in the common bile duct?

Answer 31

Almost all patients with primary stones need a biliary drainage procedure [Sphincteroplasty is 90% successful]

Question 32

How does the gallbladder form concentrated bile?

Answer 32

Active resorption of NaCl and water

Question 33

What is the most common rout of bacterial infection of bile?

Answer 33

Dissemination from portal system [Not retrograde through sphincter of oddi]

Question 34

What is the treatment of acalculous cholecystitis?

Answer 34

Cholecystectomy

Question 35

What are 3 indications for immediate ERCP?

Answer 35

1. Jaundice 2. Cholangitis 3. U/S shows stone in common bile duct [Signs that a common bile duct stone is present]

Question 36

What kind of bilirubin is bound to albumin covalently, has a half-life of 18 days, and may take a while to clear after long-standing jaundice?

Answer 36

Delta bilirubin

Question 37

Which antibiotic can cause gallbladder sludging and cholestatic jaundice?

Answer 37

Ceftriaxone

Question 38

How does the gallbladder normally fill?

Answer 38

Contraction of the sphincter of Oddi at the the Ampulla of Vader

Question 39

Parasympathetic fibers to the biliary system come from where?

Answer 39

The left trunk (anterior trunk) of the vagus nerve

Question 40

What is the name of the breakdown product of conjugated bilirubin in the gut that gives stool its brown color?

Answer 40

Stercobilin

Question 41

What is the treatment for hemobilia?

Answer 41

Angioembolization [Operation if that fails]

Question 42

What is the approach to treating a patient with an anastomotic leak following transplantation or hepaticojejunostomy

Answer 42

Percutaneous drainage of fluid collection followed by ERCP with temporary stent [Leak will heal]

Question 43

Is this hepatic bile or gallbladder bile? * Na (mEq/L): 140-170 * Cl (mEq/L): 50-120 * Bile Salts (mEq/dL): 1-50 * Cholesterol (mEq/dL): 50-150

Answer 43

Hepatic bile

Question 44

Which hormone causes constant, steady, tonic contraction of the gallbladder?

Answer 44

CCK

Question 45

What are the components of Reynolds' Pentad seen in patients with cholangitis?

Answer 45

* Charcot's Triad (RUQ pain, fever, jaundice) * Mental status change * Shock [Suggests sepsis]

Question 46

What is the only treatment that can alter the outcome for a patient with primary sclerosing cholangitis?

Answer 46

Liver transplant

Question 47

Can a laparoscopic approach be used for the resection of gallbladder adenocarcinoma?

Answer 47

No [High incidence of tumor implants in trocar sites when cancer is discovered after laparoscopic cholecystectomy]

Question 48

How is cholangiocarcinoma diagnosed?

Answer 48

Magnetic resonance cholangiopancreatography (MRCP) defines the anatomy and looks for a mass

Question 49

A gallbladder polyp of what size would be worrisome for malignancy?

Answer 49

Greater than 1 cm

Question 50

What is the approach to a patient with evidence of an injury to the biliary system more than 7 days after laparoscopic cholecystectomy?

Answer 50

Hepaticojejunostomy 6-8 weeks after injury [Tissue too friable for surgery after 7 days]

Question 51

Patients undergoing which 2 procedures should have an asymptomatic cholecystectomy?

Answer 51

1. Patient undergoing liver transplant 2. Patient undergoing gastric bypass [Also Whipple procedures should include a cholecystectomy.]

Question 52

What are 3 non-iatrogenic causes of biliary strictures?

Answer 52

1. Chronic pancreatitis 2. Gallbladder cancer 3. Bile duct cancer

Question 53

What is the treatment for gallbladder adenocarcinoma if muscle is not involved?

Answer 53

Cholecystectomy [UpToDate: Simple cholecystectomy alone is felt to be adequate for patients with tumors that are limited to the lamina propria (T1a). Cure rates following simple cholecystectomy range from 73% to 100% in case series. Patients found to have incidental T1a tumors with negative margins are generally felt to be curable with the cholecystectomy that has already been performed. Re-resection for T1a tumors does not appear to provide an overall survival benefit.]

Question 54

What percent of patients undergoing cholecystectomy will have a retained common bile duct stone?

Answer 54

5% [95% of these are cleared with ERCP]

Question 55

Which medication or hormone causes the sphincter of Oddi to relax?

Answer 55

Glucagon

Question 56

What are 3 findings on cholecystokinin-cholescintigraphy that are indications for cholecystectomy?

Answer 56

1. Gallbladder not seen (cystic duct likely has a stone) 2. Takes greater than 60 minutes to empty (chronic cholecystitis) 3. Ejection fraction less than 40% (biliary dyskinesia)

Question 57

Men in which decade of life typically get primary sclerosing cholangitis?

Answer 57

4th-5th decade [UpToDate: The incidence is higher in males and the median age at diagnosis was 41.]

Question 58

What is the best initial test for evaluation of jaundice or RUQ pain?

Answer 58

Ultrasound

Question 59

Common bile duct injuries most commonly occur after which operation?

Answer 59

After laparoscopic cholecystectomy

Question 60

What is the approach to a patient with nausea, vomiting, or jaundice and an ultrasound showing fluid collection following laparoscopic cholecystectomy?

Answer 60

* Percutaneous drain into the collection (Might be a bile leak) * If fluid is bilious, get ERCP Sphincterotomy and stent if due to: 1. Cystic duct remnant leak 2. Small injuries to the hepatic or common bile duct 3. Leak from a duct of Luschka [Larger lesions (ie complete duct transection) will require hepaticojejunostomy or choledochojejunostomy]

Question 61

What is the treatment for cholangiocarcinoma in the middle 1/3 of the biliary system?

Answer 61

Hepaticojejunostomy

Question 62

What are 4 ways to treat the symptoms of primary sclerosing cholangitis?

Answer 62

1. PTC tube drainage 2. Choledochojejunostomy or balloon dilatation of dominant strictures may provide some symptomatic relief 3. Cholestyramine can help with pruritus 4. Ursodeoxycholic acid can improve liver enzymes and decrease symptoms

Question 63

What kind of gallbladder stone is caused by solubilization of unconjugated bilirubin with precipitation?

Answer 63

Pigmented (Calcium bilirubinate) stones

Question 64

What kind of epithelium is the mucosa of the gallbladder?

Answer 64

Columnar epithelium

Question 65

What is the most important cause of late postoperative biliary strictures following laparoscopic cholecystectomy?

Answer 65

Ischemia

Question 66

What are the 2 most common organisms responsible for cholangitis?

Answer 66

1. E. Coli (most common) 2. Klebsiella

Question 67

What is a normal size for the common bile duct?

Answer 67

\<8mm (\<10mm after cholecystectomy) [UpToDate: Measurements of components of the hepatobiliary tree depend upon the skill of the ultrasonographer obtaining the measurements, and there is variability in terms of what is considered "normal." However, some general estimates are available regarding the expected sizes of structures in the hepatobiliary tree: * Gallbladder: The gallbladder wall should be less than or equal to 2 mm (in a distended or fasting gallbladder). Collapsed gallbladders, seen when the subject has eaten, typically appear slightly more thickened. Typically, the maximum "normal" dimension of the gallbladder is 5 X 10 cm. * Common hepatic duct: The common hepatic duct (inner wall to inner wall) is usually measured at the level of the hepatic artery. In the normal fasting state, it should be \<7 mm in patients \<60 years, and \<10 mm in patients older than 60. * Common bile duct (CBD): The normal CBD diameter increases with age and in patients who have had a cholecystectomy. In patients in their 40s, the normal mean diameter is 4 mm. The normal mean diameter then increases by 1 mm every decade, and authors have proposed that in older patients the normal upper limit of normal be set at 8.5 mm. The CBD is commonly up to 10 mm in patients who have undergone a cholecystectomy. * Liver: The span of the liver in the right midclavicular line should be less than 16 cm.]

Question 68

Which kind of gallstones are formed in the ducts and are more common in Asians?

Answer 68

Brown (pigmented stones) [Wikipedia: Cholesterol stones vary from light yellow to dark green or brown or chalk white and are oval, usually solitary, between 2 and 3 cm long, each often having a tiny, dark, central spot. To be classified as such, they must be at least 80% cholesterol by weight (or 70%, according to the Japanese- classification system). Between 35% and 90% of stones are cholesterol stones. Bilirubin ("pigment", "black pigment") stones are small, dark (often appearing black), and usually numerous. They are composed primarily of bilirubin (insoluble bilirubin pigment polymer) and calcium (calcium phosphate) salts that are found in bile. They contain less than 20% of cholesterol (or 30%, according to the Japanese-classification system). Between 2% and 30% of stones are bilirubin stones. Mixed ("Brown Pigment") stones typically contain 20–80% cholesterol (or 30–70%, according to the Japanese- classification system). Other common constituents are calcium carbonate, palmitate phosphate, bilirubin and other bile pigments (calcium bilirubinate, calcium palmitate and calcium stearate). Because of their calcium content, they are often radiographically visible. They typically arise secondary to infection of the biliary tract which results in the release of β-glucuronidase (by injured hepatocytes and bacteria) which hydrolyzes bilirubin glucuronides and increases the amount of unconjugated bilirubin in bile. Between 4% and 20% of stones are mixed.]

Question 69

What are the 3 most common organisms in cholecystitis?

Answer 69

1. E. Coli (#1) 2. Klebsiella 3. Enterococcus

Question 70

What are the 3 main causes of non-pigmented (cholesterol) gallstones?

Answer 70

1. Stasis 2. Calcium nucleation 3. Increased water reabsorption from the gallbladder [Other causes are decreased lecithin and bile salts] [Wikipedia: Cholesterol stones vary from light yellow to dark green or brown or chalk white and are oval, usually solitary, between 2 and 3 cm long, each often having a tiny, dark, central spot. To be classified as such, they must be at least 80% cholesterol by weight (or 70%, according to the Japanese- classification system). Between 35% and 90% of stones are cholesterol stones. Bilirubin ("pigment", "black pigment") stones are small, dark (often appearing black), and usually numerous. They are composed primarily of bilirubin (insoluble bilirubin pigment polymer) and calcium (calcium phosphate) salts that are found in bile. They contain less than 20% of cholesterol (or 30%, according to the Japanese-classification system). Between 2% and 30% of stones are bilirubin stones. Mixed ("Brown Pigment") stones typically contain 20–80% cholesterol (or 30–70%, according to the Japanese- classification system). Other common constituents are calcium carbonate, palmitate phosphate, bilirubin and other bile pigments (calcium bilirubinate, calcium palmitate and calcium stearate). Because of their calcium content, they are often radiographically visible. They typically arise secondary to infection of the biliary tract which results in the release of β-glucuronidase (by injured hepatocytes and bacteria) which hydrolyzes bilirubin glucuronides and increases the amount of unconjugated bilirubin in bile. Between 4% and 20% of stones are mixed.]

Question 71

What is the treatment for primary biliary cirrhosis?

Answer 71

Liver transplant

Question 72

Discovery of focal bile duct stenosis in patients without a history of biliary surgery or pancreatitis is highly suggestive of what?

Answer 72

Bile duct cancer (cholangiocarcinoma)

Question 73

What is the rate-limiting enzyme in the conversion of HMG CoA to Cholesterol?

Answer 73

HMG CoA reductase [HMG-CoA (or 3-hydroxy-3-methylglutaryl-coenzyme A) is an intermediate in the mevalonate and ketogenesis pathways. It is formed from acetyl CoA and acetoacetyl CoA by HMG-CoA synthase]

Question 74

Which 2 lab values are frequently elevated in cholecystitis?

Answer 74

1. Alkaline phosphatase 2. WBCs

Question 75

Emphysematous gallbladder disease is most commonly secondary to which bacteria?

Answer 75

Clostridium perfringens

Question 76

What is the rate-limiting enzyme in the conversion of Cholesterol into bile salts (acids)?

Answer 76

7-alpha-hydroxylase

Question 77

What is the most common type of gallstone found in the United States?

Answer 77

Non-pigmented (cholesterol) gallstones [Wikipedia: Cholesterol stones vary from light yellow to dark green or brown or chalk white and are oval, usually solitary, between 2 and 3 cm long, each often having a tiny, dark, central spot. To be classified as such, they must be at least 80% cholesterol by weight (or 70%, according to the Japanese- classification system). Between 35% and 90% of stones are cholesterol stones. Bilirubin ("pigment", "black pigment") stones are small, dark (often appearing black), and usually numerous. They are composed primarily of bilirubin (insoluble bilirubin pigment polymer) and calcium (calcium phosphate) salts that are found in bile. They contain less than 20% of cholesterol (or 30%, according to the Japanese-classification system). Between 2% and 30% of stones are bilirubin stones. Mixed ("Brown Pigment") stones typically contain 20–80% cholesterol (or 30–70%, according to the Japanese- classification system). Other common constituents are calcium carbonate, palmitate phosphate, bilirubin and other bile pigments (calcium bilirubinate, calcium palmitate and calcium stearate). Because of their calcium content, they are often radiographically visible. They typically arise secondary to infection of the biliary tract which results in the release of β-glucuronidase (by injured hepatocytes and bacteria) which hydrolyzes bilirubin glucuronides and increases the amount of unconjugated bilirubin in bile. Between 4% and 20% of stones are mixed.]

Question 78

A benign neuroectoderm tumor of the gallbladder is called what?

Answer 78

Granular cell myoblastoma

Question 79

Which 2 lab values are persistently elevated in cholangiocarcinoma?

Answer 79

1. Bilirubin 2. Alkaline phosphatase

Question 80

The color of bile is mostly due to what?

Answer 80

Conjugated bilirubin

Question 81

What is the most common type of gallstone found worldwide?

Answer 81

Pigmented stones [Wikipedia: Cholesterol stones vary from light yellow to dark green or brown or chalk white and are oval, usually solitary, between 2 and 3 cm long, each often having a tiny, dark, central spot. To be classified as such, they must be at least 80% cholesterol by weight (or 70%, according to the Japanese- classification system). Between 35% and 90% of stones are cholesterol stones. Bilirubin ("pigment", "black pigment") stones are small, dark (often appearing black), and usually numerous. They are composed primarily of bilirubin (insoluble bilirubin pigment polymer) and calcium (calcium phosphate) salts that are found in bile. They contain less than 20% of cholesterol (or 30%, according to the Japanese-classification system). Between 2% and 30% of stones are bilirubin stones. Mixed ("Brown Pigment") stones typically contain 20–80% cholesterol (or 30–70%, according to the Japanese- classification system). Other common constituents are calcium carbonate, palmitate phosphate, bilirubin and other bile pigments (calcium bilirubinate, calcium palmitate and calcium stearate). Because of their calcium content, they are often radiographically visible. They typically arise secondary to infection of the biliary tract which results in the release of β-glucuronidase (by injured hepatocytes and bacteria) which hydrolyzes bilirubin glucuronides and increases the amount of unconjugated bilirubin in bile. Between 4% and 20% of stones are mixed.]

Question 82

Hemobilia is most commonly caused by a fistula between which 2 structures?

Answer 82

Bile duct and hepatic arterial system

Question 83

Gallbladder polyps in which age group are more likely malignant?

Answer 83

People over 60 years old

Question 84

Gallstones occur in what percent of the general population?

Answer 84

10% [Vast majority are asymptomatic] [UpToDate: In the United States, the age standardized prevalence of gallbladder disease was estimated based upon a sample of more than 14,000 persons aged 20 to 74 in whom gallbladder disease was detected by the presence of gallstones or cholecystectomy on ultrasonography. The following prevalence rates were observed: * 8.6% and 16.6% among non-Hispanic white men and women, respectively * 8.9% and 26.7% among Mexican American men and women, respectively * 5.3% and 13.9% among non-Hispanic black men and women, respectively]

Question 85

When do symptoms of cholecystitis typically occur?

Answer 85

After a fatty meal

Question 86

Cholangitis is usually caused by what?

Answer 86

Obstruction of the bile duct [Can also be caused by indwelling tubes, biliary strictures, neoplasm, choledochal cysts, duodenal diverticula]

Question 87

What is the treatment for gallbladder polyps?

Answer 87

Cholecystectomy

Question 88

Do the common bile duct and common hepatic duct have peristalsis?

Answer 88

No

Question 89

Which segment(s) of the liver does gallbladder adenocarcinoma invade first?

Answer 89

Segments IV and V [First nodes are the cystic duct nodes on the right side]

Question 90

Which cells secrete 20% of bile?

Answer 90

Bile canalicular cells [Other 80% is secreted by the hepatocytes.]

Question 91

Where does active resorption of conjugated bile salts occur?

Answer 91

Terminal ileum [50% of resorption occurs here]

Question 92

What is the treatment for cholangiocarcinoma in the upper 1/3 of the biliary system (Klatskin tumor which occur at the confluence of the right and left hepatic bile ducts)?

Answer 92

Lobectomy and stenting of contralateral bile duct if localized to either the right or left lobe Palliative stenting if unresectable disease [Upper 1/3 has worst prognosis and is usually unresectable]

Question 93

What is the treatment for a type V choledochal cyst?

Answer 93

Partial liver resection or liver transplant [Type V cyst is totally intrahepatic]

Question 94

Which 3 medical conditions are associated with primary sclerosing cholangitis?

Answer 94

1. Ulcerative colitis 2. Pancreatitis 3. Diabetes [UpToDate: Ulcerative colitis has been reported in 25% to 90% of patients with PSC.]

Question 95

What type of choledochal cyst is characterized by fusiform or saccular dilatation of extrahepatic ducts?

Answer 95

Type I [Most common type]

Question 96

What is Mirizzi syndrome?

Answer 96

Compression of the common hepatic duct by a stone in the gallbladder infundibulum or from inflammation arising from the gallbladder or cystic duct and causing common hepatic duct stricture

Question 97

What is characterized by multiple strictures throughout the hepatic ducts?

Answer 97

Primary Sclerosing Cholangitis

Question 98

Fistula formation between the gallbladder and duodenum can result in what?

Answer 98

Gallstone ileus [More common in elderly]

Question 99

What percent of choledochal cysts are extrahepatic?

Answer 99

90%

Question 100

How does one work up a suspected biliary stricture?

Answer 100

* Magnetic resonance cholangiopancreatography (MRCP) defines the anatomy and looks for a mass (cancer) * If cancer is not ruled out with MRCP, need ERCP with brush biopsies

Question 101

Biliary stricture without a history of pancreatitis or biliary surgery is what until proven otherwise?

Answer 101

Cancer

Question 102

What is the treatment for adenomyomatosis?

Answer 102

Cholecystectomy [Adenomyomatosis = A thickened nodule of mucosa and muscle associated with Rokitansky-Aschoff sinus] [UpToDate: Adenomyomatosis is an abnormality of the gallbladder characterized by overgrowth of the mucosa, thickening of the muscle wall, and intramural diverticula. Despite the name, this condition does not involve any adenomatous changes in the gallbladder epithelium. While generally not considered to be a premalignant condition, adenomyomatosis is sometimes encountered in gallbladders resected for cancer, which has led to the suggestion that it is a premalignant condition. However, the association and its magnitude remain unclear. There is, however, a clear association of adenomyomatosis with cholelithiasis, particularly with the segmental type, which was reported in 89% of patients with segmental adenomyomatosis in one series. Adenomyomatosis of the gallbladder is less common than cholesterolosis in most reports, with some exceptions. In one report, for example, only 103 cases of adenomyomatosis were found in over 10,000 cholecystectomies (1%). This is compared with the much higher prevalence of cholesterolosis (9% to 26%). Adenomyomatosis is more common in women.]

Question 103

What is suppurative cholecystitis?

Answer 103

Frank purulence in the gallbladder that can be associated with sepsis and shock

Question 104

Will HIDA scan be positive in acalculous cholecystitis?

Answer 104

Yes

Question 105

What are 8 risk factors for gallstones?

Answer 105

1. Age greater than 40 2. Female 3. Obesity 4. Pregnancy 5. Rapid weight loss 6. Vagotomy 7. TPN (pigmented stones) 8. Ileal resection (pigmented stones)

Question 106

What are speckled cholesterol deposits on the gallbladder wall called?

Answer 106

Cholesterolosis

Question 107

What is the most common site of metastasis of gallbladder adenocarcinoma?

Answer 107

Liver

Question 108

Is this hepatic bile or gallbladder bile? * Na (mEq/L): 225-350 * Cl (mEq/L): 1-10 * Bile Salts (mEq/dL): 250-350 * Cholesterol (mEq/dL): 300-700

Answer 108

Gallbladder bile

Question 109

What is the associated risk of cholangiocarcinoma in a patient with a choledochal cyst?

Answer 109

15% cancer risk

Question 110

What is the most common site of obstruction in gallstone ileus?

Answer 110

Terminal ileum

Question 111

Epithelial invaginations in the gallbladder wall that are formed from increased gallbladder pressures are called what?

Answer 111

Rokitansky-Aschoff sinuses

Question 112

What test should be performed to work up a patient with nausea, vomiting, or jaundice following laparoscopic cholecystectomy?

Answer 112

Ultrasound to look for fluid collection [If fluid collection is present, may be bile leak -\> percutaneous drain into the collection -\> If fluid is bilious, get ERCP]

Question 113

What is the approach to a patient with nausea, vomiting, or jaundice, an ultrasound showing fluid collection, and percutaneous drainage of bilious fluid following laparoscopic cholecystectomy?

Answer 113

ERCP Sphincterotomy and stent if due to: 1. Leak from a duct of Luschka 2. Small injuries to the hepatic or common bile duct 3. Cystic duct remnant leak [Larger lesions (ie complete duct transection) will require hepaticojejunostomy or choledochojejunostomy]

Question 114

Where does cholecystitis frequently cause referred pain?

Answer 114

Right shoulder and scapula [UpToDate: Visceral pain can be caused by obstruction of any hollow viscus or injury to another pain-sensitive visceral structure such as the visceral pleura, hepatic capsule, or peritoneum. These syndromes are particularly common in patients with gastrointestinal and gynecologic malignancies. Hepatic distention syndrome — Pain-sensitive structures in the region of the liver include the hepatic capsule, vessels, and biliary tract. Stretching of the hepatic capsule by a primary hepatoma or intrahepatic metastases can cause chronic cancer pain, which is commonly described as dull, right sided subcostal pain. If the superior aspect of the capsule is involved, diaphragmatic irritation may lead to referred pain to the top of the ipsilateral shoulder. Pain also may result from injury or invasion of the porta hepatis, with or without biliary duct obstruction. This pain may be referred to the ipsilateral scapular region.]

Question 115

What is the normal size of the pancreatic duct?

Answer 115

Less than 4mm [UpToDate: Several studies have evaluated the pancreas with EUS in populations of patients without clinical features or obvious risk factors for chronic pancreatitis such as those undergoing nonpancreatic tumor staging, evaluation of submucosal tumors or portal hypertension. However, whether these studies truly reflected findings in a population of patients with a "normal" pancreas is unclear. In a report of 20 patients, the pancreatic parenchyma was described as having a homogeneous, fine, granular appearance with smooth margins. The pancreatic duct diameter was 1.9 mm on average (range 1.5-2.4 mm). In another series of 25 patients, the pancreatic parenchyma was described as being homogeneous and finely reticulated without evidence of side branch ectasia. A ventral anlage (echogenic difference between the ventral and dorsal pancreas) was seen in 68% of patients. No cysts or stones were described. The main pancreatic duct was uniformly tubular in shape with anechoic walls and a mean diameter (in the pancreatic body at the portal vein confluence) of 1.7 mm (range 1-3 mm). Only two patients had a duct diameter of 3 mm. Side branches were visible in 8 of 25 patients. A third report focused on a group of healthy volunteers without a prior history of abdominal pain or alcohol abuse. The pancreatic parenchyma was described as being uniform and more echogenic than liver. A ventral anlage was detected in 45%. No cysts were seen. The main pancreatic duct diameter was 2.4 mm (range 0.8-3.6) in the head, 1.8 mm (0.9-3.0) in the body, and 1.2 mm (range, 0.5-2.0) in the tail. Side branches were visible but narrow (mean diameter 0.8 mm, head; 0.5 mm, body; 0.3 mm, tail).]

Question 116

Lymphatics are on which side of the common bile duct?

Answer 116

Right side

Question 117

What are the early and late symptoms of cholangiocarcinoma?

Answer 117

* Early: Painless jaundice * Late: Weight loss, pruritus

Question 118

What is the name of the breakdown product of conjugated bilirubin that gets released in the urine and gives urine its yellow color?

Answer 118

Urobilin [Conjugated bilirubin is broken down in gut -\> Reabsorbed -\> Converted to urobilinogen -\> converted to urobilin]

Question 119

What is the treatment for emphysematous gallbladder disease?

Answer 119

Emergent cholecystectomy

Question 120

Which cells secrete 80% of bile?

Answer 120

Hepatocytes [Other 20% is secreted by the bile canalicular cells.]

Question 121

What is the primary pathophysiology of acalculous cholecystitis?

Answer 121

Bile stasis (from narcotics and fasting) leading to distention and ischemia [Also have increased viscosity from dehydration, ileus, transfusions]

Question 122

What is the likely cause of shock that occurs within the first 24 hours after laparoscopic cholecystectomy?

Answer 122

Hemorrhagic shock from clip that fell off cystic artery

Question 123

When is postprandial gallbladder emptying at its maximum?

Answer 123

2 hours after meals

Question 124

Which tissue layer is absent in the gallbladder?

Answer 124

Submucosa

Question 125

What is the treatment for gallstone ileus?

Answer 125

1. Remove stone through enterotomy proximal to obstruction 2. Cholecystectomy and fistula resection if patient can tolerate it (leave fistula if patient is old and frail)

Question 126

What is the sensitivity of ultrasound for detecting gallstones?

Answer 126

95%

Question 127

Which 3 signals increase bile excretion?

Answer 127

1. CCK 2. Secretin 3. Acetylcholine (vagal input)

Question 128

Where are the highest concentrations of CCK and secretin cells located?

Answer 128

Duodenum

Question 129

Which 2 signals decrease bile excretion?

Answer 129

1. Somatostatin 2. Sympathetic stimulation

Question 130

What are the 2 most common causes of hemobilia?

Answer 130

1. Trauma 2. Percutaneous instrumentation to liver

Question 131

Do patients with primary biliary cirrhosis have an increased risk of cancer?

Answer 131

No

Question 132

Where does passive resorption of nonconjugated bile salts occur?

Answer 132

1. Small intestine (45% of resorption) 2. Colon (5% of resorption) [The other 50% of resorption is actively resorbed in the terminal ileum.]

Question 133

What are the components of Charcot's Triad seen in patients with cholangitis?

Answer 133

1. RUQ pain 2. Fever 3. Jaundice

Question 134

Sympathetic fibers to the biliary system come from where?

Answer 134

T7-T10 (splanchnic and celiac ganglions)

Question 135

Which anatomical triangle contains the cystic artery?

Answer 135

The triangle of Calot (Hepatobiliary triangle)

Question 136

Progressive fibrosis of intrahepatic and extrahepatic bile ducts in a patient with primary sclerosing cholangitis eventually results in what?

Answer 136

Portal hypertension and hepatic failure

Question 137

Intraoperative common bile duct injury can be primarily repaired if the injury is less than what percent of the circumference of the common bile duct?

Answer 137

Less than 50% [In all other cases, will likely need hepaticojejunostomy or choledocojejunostomy]

Question 138

Which 3 lab values, if persistently high for greater than 24 hours, is an indication for pre-op ERCP?

Answer 138

1. AST or ALT (greater than 200) 2. Bilirubin (greater than 4) 3. Amylase or lipase (greater than 1,000)

Question 139

What is the likely cause of shock that occurs more than 24 hours after laparoscopic cholecystectomy?

Answer 139

Septic shock from accidental clip on common bile duct with subsequent cholangitis

Question 140

Air in the biliary system most commonly occurs after what?

Answer 140

ERCP and sphincterotomy [Can also occur with cholangitis or erosion of the biliary system into the duodenum]

Question 141

In which portion of the biliary system does cholangiocarcinoma most commonly occur?

Answer 141

Upper 1/3 [Worst prognosis and usually unresectable] [UpToDate: Biliary tract cancers were traditionally divided into cancers of the gallbladder, the extrahepatic ducts, and the ampulla of Vater, while intrahepatic tumors of the bile system were classified as primary liver cancers. More recently, the term cholangiocarcinoma has been used to refer to bile duct cancers arising in the intrahepatic, perihilar, or distal (extrahepatic) biliary tree, exclusive of the gallbladder or ampulla of Vater. Intrahepatic cholangiocarcinomas originate from small intrahepatic ductules (termed peripheral cholangiocarcinomas) or large intrahepatic ducts proximal to the bifurcation of the right and left hepatic ducts. The extrahepatic bile ducts are divided into perihilar (including the confluence itself) and distal segments, with the transition occurring at the point where the common bile duct lies posterior to the duodenum distal to the insertion of the cystic duct into the common bile duct. In general, perihilar disease represents about 50%; distal disease, 40%; and intrahepatic disease, less than 10% of cholangiocarcinoma cases.]

Question 142

Patients with primary biliary cirrhosis test positive for which antibody?

Answer 142

Antimitochondrial antibodies [UpToDate: Antimitochondrial antibodies (AMA) are the serologic hallmark of PBC. They are present in approximately 95% of patients with PBC. Occasionally, AMA are detected in patients with no other features suggestive of PBC. Many of these patients will eventually go on to develop features of PBC. Antinuclear antibodies (ANA) are found in up to 70% of patients with PBC. A variety of staining patterns may be present. Two immunofluorescence patterns are considered "PBC-specific": the multiple nuclear dots pattern (target antigen, Sp100) and the rim-like/membranous pattern (target antigens, gp210, nucleoporin p62, and the lamin B receptor). Other antibodies such as anticentromere, anti-SSA/Ro, and anti-dsDNA antibodies can be also found in PBC. ANA have clinical significance in PBC for two reasons. First, their presence can cause confusion with autoimmune hepatitis or autoimmune hepatitis/PBC overlap. Second, some suggest that ANA may be associated with more rapid progression of disease and a poorer prognosis. However, the strength of this association and the implications for management are uncertain. For example, patients with PBC who are AMA-negative and have a positive ANA, a disease often called autoimmune cholangitis but more appropriately AMA-negative PBC, have the same outcomes as those who are AMA-positive and ANA-negative.]

Question 143

What is the normal thickness of the gallbladder wall?

Answer 143

Less than 4mm [UpToDate: Gallbladder wall thickening (greater than 4 to 5 mm) is indicative of cholecystitis. Measurements of components of the hepatobiliary tree depend upon the skill of the ultrasonographer obtaining the measurements, and there is variability in terms of what is considered "normal." However, some general estimates are available regarding the expected sizes of structures in the hepatobiliary tree: Gallbladder: The gallbladder wall should be less than or equal to 2 mm (in a distended or fasting gallbladder). Collapsed gallbladders, seen when the subject has eaten, typically appear slightly more thickened. Typically, the maximum "normal" dimension of the gallbladder is 5 X 10 cm. Common hepatic duct: The common hepatic duct (inner wall to inner wall) is usually measured at the level of the hepatic artery. In the normal fasting state, it should be \<7 mm in patients \<60 years, and \<10 mm in patients older than 60. Common bile duct (CBD): The normal CBD diameter increases with age and in patients who have had a cholecystectomy. In patients in their 40s, the normal mean diameter is 4 mm. The normal mean diameter then increases by 1 mm every decade, and authors have proposed that in older patients the normal upper limit of normal be set at 8.5 mm. The CBD is commonly up to 10 mm in patients who have undergone a cholecystectomy. Liver: The span of the liver in the right midclavicular line should be less than 16 cm.]

Question 144

What is the approach to a patient with evidence of an injury to the biliary system within 7 days of laparoscopic cholecystectomy?

Answer 144

Take back to the OR for hepaticojejunostomy [After 7 days, must wait 6-8 weeks before takeback for hepaticojejunostomy.]

Question 145

Biliary ducts that lie in the gallbladder fossa and can leak after cholecystectomy are called what?

Answer 145

Ducts of Luschka

Question 146

What is Murphy's sign?

Answer 146

Patient resists deep inspiration with deep palpation to the RUQ because of pain

Question 147

What is the most common cancer of the biliary tract?

Answer 147

Gallbladder adenocarcinoma [4x more common than bile duct cancer, but still rare]

Question 148

Which medication or hormone causes the sphincter of Oddi to contract?

Answer 148

Morphine