CHAPTER 32- BILIARY SYSTEM.txt

IV and V

The cystic artery branches off the R hepatic artery

It goes through the triangle of Calot (cystic duct [lateral], common bile duct [medial], liver [superior])

The Right hepatic (lateral) and retroduodenal branches of the gastroduodenal artery (medial) supply to the hepatic and common bile duct (9- and 3- o'clock positions when performing ERCP); considered longitudinal blood supply.

Cystic veins drain in to the right branch of the portal vein and into the liver

lymphatics are on the right side of the common bile duct

parasympathetic fibers are from the left (anterior) trunk of the vagus

sympathetic fibers from T7-T10 coursing through splanchnic and celiac ganglions

Gallblader has no submucosa; mucosa is columnar epithelium

Common bile duct and hepatic duct do not have peristalsis

Gallbladder normally fills by contraction of sphincter of Oddi at the ampulla of Vater

• * Morphine- contracts the sphincter of Oddi
• * Glucagon- relaxes the sphincter of Oddi

• CBD- <8mm (<10mm after cholecystectomy)
• Gallbladder wall <4mm
• Pancreatic duct <4mm

after cholecystectomy, total bile acid pools decrease

The highest concentration of CCK and secretin cells are in the duodenum

invagination of the epithelium of the wall of the gallbladder; formed from increased gallbladder pressure

biliary ducts that go directly from the liver into the gallbladder; can leak after a cholecystectomy

• Increase bile excretion:
• 1) CCK
• 2) Secretin
• 3) Vagal input

• Decrease bile excretion:
• 1) VIP
• 2) Somatostatin
• 3) Sympathetic stimulation

CCK causes constant, steady tonic contraction

• 1. Cholesterol excretion
• 2. Bilirubin excretion
• 3. Absorb fat soluble vitamins

The gallbladder forms concentrated bile by active resorption of Na and water

• Bile salt pool is 5-7g.
• It cycles 4-8 times/day

• Small amount (5-10%) of bile salts are lost in stool.
• - active resorption of conjugated bile acids occurs in the terminal ileum (50%)
• - Passive resorption of nonconjugated bile acids can occur in the small intestine (45%) and colon (5%)

postprandial emptying maximum at 2 hours (80%)

Canalicular cells (20%) and hepatocytes (80%)

The color of bile is mostly due to conjugated bilirubin

Stercobilin- the breakdown product of conjugated bilirubin in gut; gives stool brown color

Urobilin- breakdown product of conjugated bilirubin in gut; some gets reabsorbed and released in urine

1. HMG-CoA--> (HMG CoA reductase) --> Cholesterol--> (7 alpha hydroxylase) --> bile acids

2. HMG CoA reductase - rate-limiting step in cholesterol synthesis

3. Stones in obese people - overactive HMG CoA reductase

4. Stones in thin people- underactive 7-alpha-hydroxylase

• 1) occur in 10% of the population; most asymptomatic
• 2) Only 10% of gallstones are radiopaque

• Increased cholesterol insolubilization; caused by:
• 1) stasis
• 2) calcium nucleation by mucin glycoproteins
• 3) increased water resorption from gallbladder
• 4) decreased lecithin and bile acids

- found almost exclusively in the gallbladder

Most common type of stone found in US (75%)

• 1) most common worldwide, 25% of stones in US
• 2) caused by solubilization of unconjugated bilirubin with precipitation of calcium bilirubinate and insoluble salts
• 3) Dissolution agents do not work on pigmented stones (mono-octanoin)

• Can be caused by:
• 1) hemolytic disorders
• 2) cirrhosis
• 3) patients on chronic TPN
• 4) patients with ileal resection

• 2) Important factors for development of these stones:
• 1- increased bilirubin load
• 2- decreased hepatic function
• 3- bile stasis

4) almost always form in gallbladder

5) Tx: cholecystectomy

• 1) primary CBD stones, formed in ducts
• 2) Infection causing deconjugation of bilirubin
• 3) Increased in Asians
• 4) E. coli most common- produced beta-glucuronidase, which deconjugates bilirubin, causing formation of calcium bilirubinate
• 5) need to check for ampullary stenosis, duodenal diverticula, and abnormal sphincter of Oddi
• 6) most commonly form in the bile duct (are primary common bile duct stones)
• 7) Almost all patients with primary stones need a biliary drainage procedure- sphincteroplasty 90% successful
• 8) cholesterol stones and black stones found in the CBD are considered secondary common bile duct stones

• 1) caused by obstruction of the cystic duct by a gallstones
• 2) results in gallbladder wall distention and wall inflammation
• 3) Symptoms: RUQ pain, referred pain to the right shoulder and scapula, nausea/vomiting, loss of appetite
• 4) Attacks frequently after a fatty meal and pain is persistent (unlike biliary colic)
• 5) Murphy's sign- patient resists deep inspiration with deep palpation to the RUQ secondary to pain
• 6) Alkaline phosphatase and WBC are frequently elevated
• Suppurative cholecystitis- associated with frank purulence in the gallbladder--> can be associated with sepsis & shock

• Most common organisms in cholecystitis:
• -E. coli
• -klebsiella
• -enterococcus

• Stone risk factors:
• 1) >40
• 2) female
• 3) obesity
• 4) pregnancy
• 5) rapid weight loss
• 6) vagotomy
• 7) TPN (pigmented stones)
• 8) ileal resection (pigmented stones)
• Ultrasound
• 1) 90% sensitive for picking up stones--> hyperechoic focus, posterior shadowing, movement of focus with changes in position
• 2) best initial evaluation test for jaundice or RUQ pain
• 3) Findings suggestive of acute cholecystitis- gallstones, gallbladder wall thickening (>4mm), pericholecystic fluid
• 4) Dilated CBD (>8mm) suggests CBD stone and obstruction

• HIDA Scan
• 1) technetium taken up by liver an excreted in the biliary tract
• 2) If gallbladder cannot be seen, it is secondary to cystic duct obstrution by stone--> needs cholecystectomy
• 3) If <25% of gallbaldder volume excreted after CCK over 2 hours--> biiary dyskinesia; although not totally occluded, the excretion is reduced.
• 4) 50% of these patients benefit from cholecystectomy

• 1- jaundice
• 2- cholangitis
• 3- gallstone pancreatitis
• 4- increased bilirubin (can also be due to primary liver disease)
• 5- signficantly increased AST/ALT (can also be due to primary liver disease)
• 6-stone in CBD on ultrasound.

<5% of patients undergoing cholecystectomy will have a retained CBD stone --> 95% of these cleared with ERCP

• 1- cholecystectomy
• 2- cholecystostomy tube can be placed in patients who are very ill and cannot tolerate surgery
• - when a patient is subsequently able to tolerate surgery, cholecystectomy is performed

• 1) best treatment for late common bile duct stone
• 2) sphincterotomy allows for removal of stone
• 3) grasper and other tools can then be used to remove the stone
• 4) Risks: bleeding, pancreatitis, perforation

• 1) transient cystic duct obstruction caused by passage of gallstone
• 2) resolves in 4-6 hours

• 1) most commonly occurs with previous ERCP and sphincterotomy
• 2) can also occur with cholangitis or erosion of the biliary system into the duodenum (i.e. gallstone ileus)

• 1) dissemination from portal system is usual route.
• 2) can also get retrograde infection from bacteria in duodenum
• 3) highest incidence of positive bile cultures occurs with postoperative strictures (usually E. coli, often polymicrobial)

• 1) thickened wall, RUQ pain, incresased WBCs
• 2) occurs most commonly after severe burns, prolonged TPN, trauma, or major surgery
• 3) primary pathology is bile stasis (narcotics, fasting), leading to distention and ischemia
• 4) Also have increased viscosity secondary to dehydration, ileus, transfusions
• 5) ultrasound shows sludge, gallbladder wall thickening, and pericholecystic fluid
• 6) HIDA scan is positive
• 7) Treatment: cholecystectomy, percutaneous drainage if patient is too unstable.

• 1) gas in gallbladder wall
• 2) can see on plain film
• 3) increased in diabetics; usually secondary to clostridium perfringens
• 4) Symptoms: severe, rapid-onset abdominal pain, nausea, vomiting, and sepsis
• 5) perforation more common in these patients
• 6) Tx: emergent cholecystectomy; percutaneous drainage if patient is too unstable.

• 1) fistula between gallbladder and duodenum that releases stone, causing small bowel obstruction; elderly.
• - can see pneumobilia (air in the biliary system) on plain film
• 2) Terminal Ileum- most common site of obstruction
• 3) Treatment: remove stone with enterostomy proximal to obstruction; perform cholecystectomy and fistula resection if patient can tolerate it.

• 1) most commonly occur after laparoscopic cholecystectomy
• 2) intraoperative cholangiography does not prevent injuries; may limit severity; increased early diagnosis of injury
• 3) in 10% of patients, the right posterior duct (from segement 6 or 7) enters the common bile duct seperately
• 4) Risk of injury with cholecystectomy (confused for cystic duct)
• 5) if >2mm, will need to open and perform hepaticojejunostomy

if less than 50% the circumference of the common bile duct, can probably perform primary repair; in all other cases, will likely need hepaticojejunostomy or choledochojejunostomy

• 1) Ultrasound to look for fluid collection
• -if fluid collection present, may be bile leak--> percutaneous drain into the collection
• - if fluid is bilious, get ERCP --> sphincterotomy and stent if due to cystic duct remnant leak, small injuries to hepatic or common bile duct, or a leak from duct of Luschka.
• - larger lesions (i.e. complete duct transection) will require hepaticojejunostomy or choledochojejunostomy
• - if fluid collection not present and the hepatic ducts are dilated, likely have a completely transected common bile duct.

with ERCP and stents

• 1) Fluid resuscitation and stabilize patient
• 2) May be due to complete transection of the CBD and cholangitis--> get ultrasound to look for dilated intrahepatic ducts or fluid collections
• 3) If no fluid collections but bile ducts are dilated --> get ERCP and try to stent the strictured area
• 4) If that fails, place a PTC tube

• 1) Most commonly occur after laparoscopic cholecystectomy
• 2) Ischemia is the most important cause of late postoperative biliary strictures
• 3) Can also be caused by chronic pancreatitis or stricture of a biliary enteric anastomosis
• 4) Diagnosis- ERCP will show stricture; U/S will likely show dilated hepatic ducts
• 5) Symptoms: sepsis, cholangitis, jaundice
• 6) Treatment:
• - ERCP with sphincterotomy and possible stent placement to decompress; PTC tube if that fails
• - for lesions that cause early symptoms (<7days)- hepaticojejunostomy
• - for lesions that cause later symptoms (>7days)- hepaticojejunostomy 6-8 weeks after injury
• - acute injuries are unlikely to be treated sufficiently with ERCP, sphincterotomy, and stent (need to make sure these late injuries do not represent Ca- get brushings)

• 1) fistula between bile duct and hepatic arterial system (most commonly)
• 2) patients classically present with UGI bleed, jaundice, and RUQ pain
• 3) Most commonly occurs with trauma (50% of all cases), infections, primary gallstones, aneurysms, and tumors
• 4) Diagnosis: angiogram
• 5) Treatment: angiogram and embolization first; operation if that fails.

• 1) rare; most common cancer of the biliary tract
• 2) four times more common than bile duct Ca; most have stones
• 3) Liver is the most common site of metastasis
• 4) Porcelain gallbladder- risk of gallbladder Ca (10-20%) --> these patients need cholecystectomy
• 5) 1st spreads to segments IV and V; 1st nodes are the cystic duct nodes (right side)
• 6) Symptoms: jaundice 1st, then RUQ pain
• 7) If limited to mucosa (stage I), cholecystectomy is all that is needed
• - this scenario usually occurs as an incidental finding following laparoscopic cholecystectomy
• 8) If into the muscle (stage II), need wide resection around liver bed at segments IV and V (2-3 cm margins), regional lymphadenectomy, including portal triad; may need whipple, lobectomy, or resection of the CBD.
• -90% of patients present with stage IV disease
• 9) High incidence of tumor implants in trocar sites when discovered after laparoscopic cholecystectomy
• 10) laparoscopic approach contraindicated for gallbladder Ca
• 11) 5%- 5year survival overall

1) occurs in elderly males

• 2) risk factors:
• 1- C. sinensis infection
• 2- typhoid
• 3- ulcerative colitis
• 4- choledochal cysts
• 5- sclerosing cholangitis
• 6- congenital hepatic fibrosis
• 7- chronic bile duct infection

• 3) Symptoms:
• early- painless jaundice most common; can also get cholangitis
• late- weight loss, anemia, pruritis

4) Persistent increase in bilirubin and alkaline phosphatase

5) Diagnosis: ERCP 1st, MRI may help define the lesion (these tumors can be hard to find)

6) Invades contiguous structures early

7) discovery of a focal bile duct stenosis in patients without a history of biliary surgery or pancreatitis is highly suggestive of bile duct Ca

• 8) Klatskin tumors-
• upper 1/3
• - most common type, worst pronosis, usually unresectable
• Middle 1/3- hepaticojejunostomy
• Lower 1/3- whipple
• 9) Palliative stenting for unresectable disease
• 10) overall 5yr survival rate- 20%

• 1) female gender, asia, japan
• 2) 90% extrahepatic; 15% cancer risk
• 3) Older patients have episodic pain, fever, jaundice, cholangitis
• 4) most are type I- fusiform or saccular dilation of extrahepatic ducts (very dilated)
• 5) infants can have symptoms similar to biliary atresia
• 6) possibly caused by abnomal reflux of pancreatic enzymes during development secondary to bad angle of insertion
• 7) occurs during uterine development
• 8) Treatment: cyst excision with hepaticojejunostomy and cholecystectomy
• 9) Type IV cysts are partially intrahepatic and Type V (Caroli's disease) are totally intrahepatic--> will need partial liver resection

• 1) men 4-5th decade
• 2) can be associated with:
• - retroperitoneal fibrosis
• - Riedel's thyroiditis
• - ulcerative colitis
• - DM
• 3) Symptoms: fatigue, fluctuating jaundice, pruritis, weight loss, RUQ pain
• -pruritis caused by bile acids
• 4) Diagnosis: ERCP- multiple strictures and dilatations (beaded appearance)

• 5) antimitochondrial antibodies
• 6) Bacterial cholangitis unusual unless biliary tract manipulation has occured
• 7) Does not get better after colon resection for ulcerative colitis
• 8) Leads to portal HTN and hepatic failure (scarring and patching with progressive fibrosis of intrahepatic and extrahepatic ducts
• 9) Can have isolated intrahepatic or extrahepatic duct inflammation and fibrosis
• 10) complications: cirrhosis, cholangiocarcinoma
• 11) Treatment: transplant needed long term for most; PTC tube drainage, choledochojejunostomy may be effective for some; balloon dilatation of dominant strictures may provide some symptomatic relief
• - cholestyramine- can decrease pruritis symptoms (decrease bile acids)
• - UDCA (urodeoxycholic acid)- can decrease symptoms (decrease bile acids) and improve liver enzymes

• 1) women; medium-sized hepatic ducts
• 2) Cholestasis--> cirrhosis--> portal hypertension
• 3) symptoms-
• -fatigue
• -pruritis
• -jaundice
• -xanthomas
• 4)antimitochondrial antibodies
• 5) No increased risk for cancer
• 6) Treatment: transplantation

• 1) Charcot's triad-
• 1- RUQ pain
• 2- fever
• 3- jaundice

2)Reynold's pentad- charcot's triad plus mental status changes and shock

3) Most common organisms- 1) E.coli 2) klebsiella

4) Cholovenous reflux occurs at 20mmHg pressure --> systemic bacteremia

5) Diagnosis: AST/ALT, bilirubin, alkaline phosphatase, and WBC often increase

6) Ultrasound- CBD will be dilated (>8 mm, >10mm after cholecystectomy) on ultrasound if due to obstruction of the biliary system

7) Stricture and hepatic abscess are late complications of cholangitis

8) Renal failure- #1 serious complication, related to sepsis

9) Gallstones are most common etiology

• 10) Other causes-
• 1- biliary strictures (iatrogenic)
• 2- neoplasm
• 3- chronic pancreatitis
• 4- congential choledocal cysts
• 5- duodenal diverticula

10) Treatment: fluid resuscitation, antibiotics

11) Emergent ERCP with sphincterotomy and stone extraction; if ERCP fails, go to PTC tube

12) If PTC tube is infected- change the PTC tube

1) Asia, recurrent cholangitis from primary CBD stones

• 2) Caused by:
• 1- C. sinesis
• 2- A. lumbridoides
• 3- T. trichiura
• 4- E.coli

3) Treatment: hepaticojejunostomy and antiparasitic medications

1) early (1st 24hrs)- hemorrhagic shock from clip that fell off cystic artery

2) Late (after 1st 24hrs)- septic shock from accidental clip on CBD with subsequent cholangitis

• ^{thickened nodule of mucosa and muscle associated with Rokitansky-Aschoff sinus}
• -not premaligannt; does not cause stones, can cause RUQ pain

Treatment: cholecystectomy

• 1) benign neuroectoderm tumor of the gallbladder
• 2) can occur in biliary tract with signs of cholecystitis
• 3) Treatment: cholecystectomy

speckled cholesterol deposits on gallbladder wall

• 1) >1cm worry about malignancy
• 2) polyps in patients >60 years more likely malignant
• 3) Treatment: cholecystectomy

• 1) bound to albumin covalently
• 2) half life of 18 days
• 3) may take a while to clear after long-standing jaundice

compression of the common hepatic duct by a stone in the infundibulum of the gallbladder or inflammation arising from the gallbladder or cystic duct extending to the contiguous hepatic duct, causing stricture and hepatic duct obstruction

can cause gallbladder sludge and cholestatic jaundice

in patients undergoing liver TXP or gastric bypass