GI E2- Biliary tract disease

The pancreaticobiliary system delivers _______ to the intestinal tract.

Bile

What composes the pancreaticobiliary system?

Gallbladder, cystic duct, intrahepatic duct, hepatic duct, common bile duct, & pancreas

What composes the common bile duct?

Cystic duct + common hepatic duct

What organ is a distensible sac that concentrates and stores bile, runs behind the duodenum and ends at ampulla of Vater?

Gallbladder

Bile is formed in the ______, and modified & stored in the ________

Liver ; gallbladder and bile ducts

What emulsifies lipids (micelles) and transports wastes such as bilirubin, toxins, cholesterol, and IGs?

Bile

What AST-ALT ratio suggests alcohol injury / alcohol hepatitis?

≥ 2:1

What is a useful marker to diagnose specific liver diseases?

AST-ALT ratio

What level of AST-ALT elevations would be due to ETOH alone?

< 300 IU/L

Besides ETOH, what are other causes for elevated AST/ALT?

Viral hepatitis & drug/toxin induced (both MC), ischemia, autoimmune liver dz, acute bile duct obstruction, hepatic vein obstruction, Wilson’s disease, acetaminophen

How elevated is ALP in biliary tract obstruction?

3-10x

How elevated ALP in hepatitis and cirrhosis?

less than 3x

How elevated is ALP in pregnancy (3rd trimester)?

1.5-2.5x

What common digestive disease consists of gallstones, most often asymptomatic and found incidentally during abd sonography?

Cholelithiasis

What are the majority of gallstones/cholelithiasis?

Cholesterol

What due black pigment stones in cholelithiasis indicate?

Cirrhosis, hemolysis, biliary stasis, CF

What do brown pigment gallstones in cholelithiasis indicate?

Parasites, infection

What is the pathogenesis of cholelithiasis?

Cholesterol supersaturation of bile → destabilization of bile → stasis of bile in gallbladder

Who is cholelithiasis 2-3x more common in?

Women

What are RF for cholelithiasis in adults (> 40)?

F, obese, mexican or Native American, DM, metabolic syndrome, pregnancy, OCs, prolonged fasting, rapid wt loss, spinal cord injuries

What are RF for cholelithiasis in children?

CF, sickle cell disease

What are the 4 Fs of cholelithiasis (predisposing factors)?

Fat, forty, female, fertile

What is often a precursor of gallstones that develops during gallbladder stasis?

Biliary sludge

How does biliary sludge develop in females?

Endogenous estrogens inc biliary cholesterol secretion & cholesterol saturation of bile

How does biliary sludge develop in obesity?

Overproduction of cholesterol → hypersecretion into bile → gallstone formation

How does biliary sludge develop during pregnancy?

Impaired gallbladder emptying from progesterone + effects of estrogen → inc cholesterol hypersecretion

The following ssx are associated with what condition?

• biliary colic- steady epigastric or RUQ pain

  • radiate to R scapula or shoulder, lasts 15min-5 hrs

  • often develops after eating fatty foods

• nocturnal awakening from pain

• N, +/- V

• most will have recurrent attacks

Cholelithiasis

What causes the steady epigastric or RUQ pain (biliary colic) associated with cholelithiasis?

Stone obstructs cystic duct or common bile duct → distension of viscus → visceral pain

*stone returns back to gallbladder after attack

How is uncomplicated symptomatic gallstone disease characterized?

Episodes of biliary pain < 5 hrs

How is complicated gallstone disease characterized?

Biliary pain lasts > 5 hours + labs might indicate acute cholecystitis, acute biliary pancreatitis, or biliary obstruction

What is the preferred modality to diagnosis cholelithiasis?

Abd US → gallbladder wall thickening, cystic duct dilation

What are other dx modalities for cholelithiasis?

HIDA scan (GB contractility), CT scan, ERCP, PTC

What is a percutaneous transhepatic cholangiogram (PTC)?

Transhepatic insertion of needle into bile duct w/ injection of contrast (for those who can’t have ERCP)

What comps can occur with cholelithiasis?

Acute cholecystitis

What condition is an acute GB wall inflammation due to mechanical, chemical, or bacterial causes and is a complication of cholelithiasis?

Acute cholecystitis

What is the pathophysiology of cholecystitis?

Cystic duct obstruction w/ stone PLUS another factor → circulation & lymph drainage become impaired → mucosal ischemia & necrosis

Secondary bacterial inflammation (e. coli, klebsiella, strep, clostridium)

The following PE findings are seen in what condition?

• progressively worsening biliary pain/colic localized to RUQ

• N, V

• Murphys sign

• palpable gallbladder, peritoneal inflammation

• Triad: RUQ pain, fever, leukocytosis

Acute cholecystitis

What is the triad for acute cholecystitis?

RUQ pain, fever, leukocytosis

What sign?

• Pt exhale → place hand below costal margin on right side at MCL → pt inhale

• positive = pt stops breathing in & winces w/ a catch in breath

  • d/t inflamed gallbladder palpated as it descends → acute cholecystitis

Murphy’s sign

What will happen to acute cholecystitis if left untreated?

Inflammation → gangrene → GB wall rupture → peritonitis, septic shock, localized abscess, or cholecystoenteric fistula possible

What is the dx for acute cholecystitis?

Abd US, leukocytosis, mild elevation in AST/ALT, amylase, or bili

What is the treatment for acute cholecystitis?

NPO, NGT if V, IVF, broad spectrum abx, analgesics (toradol, opioids), chemodissolution (CDCA or UDCA), cholecystectomy

What condition is chronic inflammation of the GB wall that results from repeated attacks of acute/subacute cholecystitis OR mechanical irritation of GB mucosa by gallstones?

Chronic cholecystitis

What are possible complications of chronic cholecystitis?

Biliary sepsis, porcelain gallbladder

What condition?

• calcium salts are deposited w/in GB wall of chronically inflamed GB (comp of chronic cholecystitis)

• Dx: plain films

• Rx: cholecystectomy

Porcelain gallbladder

What does porcelain gallbladder have a high association with?

Carcinoma of gallbladder

What is an acute necroinflammatory disease of the GB, presents with NO gallstone, can cause acute cholecystitis & has a high morbidity/mortality?

Acalculous cholecystitis

Who is acalculous cholecystitis MC in?

Males over 50

What is acalculous cholecystitis associated with?

Major surgery, critical illness, burns, trauma, TPN

What is the pathogenesis of acalculous cholecystitis?

Gallbladder stasis & ischemia → local inflammatory response in GB wall → endothelial injury leading to stasis → concentration of bile salts → GB distention & eventually necrosis

*Secondary infx can occur in severe cases

How is acalculous cholecystitis diagnosed?

Abd US & CT, HIDA scan, blood cultures

What is the treatment for acalculous cholecystitis?

Abx, cholecystectomy or cholecystectomy tube

Algorithm for acute cholecystitis diagnosis

What condition is characterized by calculus in the common bile duct (CBD) & occurs in 15% of pts w/ gallstones?

Choledocholithiasis

How does choledocholithiasis form?

Stones usually originate in GB → smaller stones can progress further into duodenum

OR forms spontaneously in CBD s/p cholecystectomy

The following presentation is associated with what condition?

• MC “silent”- no sx unless obstruction

• Frequently occur in pts w/ hx of biliary colic episodes

• +/- abd tenderness

• Jaundice develops later on

Choledocholithiasis

What labs are seen in choledocholithiasis?

Initial- elevated LFTs & GGT

Later- elevated ALP & bilirubin (leads to jaundice)

How is choledocholithiasis diagnosed?

Abd US, ERCP

What diagnostic modality is highly sensitive and specific for choledocholithiasis, is completed if cholangitis or acute pancreatitis is also present, and allows for stone extraction?

ERCP

What condition is a bacterial infection superimposed over an obstructed biliary tree due to a gallstone, stricture, or neoplasm (uncommon, can develop after ERCP)?

Acute cholangitis

What is the pathophysiology of acute cholangitis?

Biliary tree obstruction → inc intraluminal pressure → bile infx → hepatic ducts → hepatic canaliculi → hepatic veins → lymph → bacteremia

What is the triad associated with acute cholangitis?

Charcot’s triad → RUQ pain, jaundice, fever w/ chills

The following labs can be seen in what condition?

• WBCs: inc w/ left shift

• Total bili: inc

• ALP: inc

• GGT: inc

• AST, ALT: mild inc

• Serum amylase & lipase: moderate inc

Acute cholangitis

What are the diagnostic modalities for acute cholangitis?

PTC, ERCP, MRCP

What is the pentad associated with acute suppurative cholangitis (presence of pus in biliary ducts)?

Reynold’s pentad → charcot’s triad + hypotension + mental confusion

If ERCP or cholecystectomy cannot be performed for acute cholangitis, what do radiologists need to do?

Percutaneous cholecystostomy → tubes placed through skin into GB for drainage

What can be used to provide road map for biliary endoscopist to define biliary anatomy prior to ERCP?

MRCP

How would acute cholangitis appear on MRCP/ERCP?

Generalized dilatation of intrahepatic bile ducts to level of hilum

What condition is pancreatic inflammation due to the passage of stones through the CBD during acute cholecystitis or in patients with choledocholithisis?

Biliary pancreatitis

What condition is an autoimmune destruction of intrahepatic bile ducts & cholestasis?

Primary biliary cholangitis (PBC)

Who is PBC MC in?

Middle aged women

The inflammation & fibrosis associated with PBC can lead to what?

Portal HTN and eventual cirrhosis (in 10-12 yrs)

What is the pathogenesis of PBC?

Antimitochondrial antibodies (AMA) destroy intrahepatic bile ducts; possible role with genetics & environmental triggers, viruses or bacteria

What is the onset of PBC?

Insidious- often asx and found incidentally w/ inc LFTs on annual labs

The following symptoms are associated with what condition?

• Fatigue

• dry eyes & mouth

• pruritus / excoriations- severe, local, or diffuse

• unexplained RUQ discomfort- hepatomegaly

• xanthelasma- yellow plaques around eyes

  • d/t dec LDL receptors in damaged hepatocytes

• late finding → jaundice

PBC

What is the dx for PBC?

Cholestatic LFT pattern → 3-4x inc ALP

Positive AMA, positive ANA, liver bx (dz not uniform throughout)

What is the first line and only proven therapy for PBC?

Ursodiol (URSO) → decreases ALP

What are additional treatment options for PBC?

Cholestyramine for pruritus, Osteoporosis agents (Ca, Vit D), vaccines, hepatology referral, no alcohol

What is the only effective treatment for end stage PBC?

Liver transplant

What are possible complications of PBC?

Portal HTN, edema, ascites, esophageal or gastric varices, splenomegaly, hepatic encephalopathy, osteoporosis & fractures, cholelithiasis, steatorrhea

What condition is a progressive, inflammatory, sclerosis & obliterative disease of the extra hepatic bile ducts, intrahepatic bile ducts, or both?

Primary sclerosing cholangitis (PSC)

Can the progression of primary sclerosing cholangitis (PSC) be halted?

No

Who is primary sclerosing cholangitis MC in?

Men 20-50 y/o

What is primary sclerosing cholangitis (PSC) often associated with?

UC (check in pts w/ IBD + persistent & unexplained elevated ALP)

What is the median survival rate of PSC?

12 yrs

What does PSC ultimately lead to?

Biliary obstruction, secondary biliary cirrhosis, hepatic failure, portal HTN w/ bleeding varices

What are PSC patients at an increased risk for developing?

Cholelithiasis, choledocholithiasis, cholangitis, & cholangiocarcinoma

The following ssx are associated with what condition?

• often asx

• if sx- fatigue & pruritus are common

• RUQ pain

• progressive jaundice

• anorexia, indigestion

• acute cholangitis

PSC

The following diagnostic workup is for what condition?

• cholestatic LFT pattern → 4-10x inc ALP

• liver bx → fibrous obliteration of connective tissue in onion skin pattern

• fibroscan

• MRCP (preferred) & ERCP→ narrowing & beading of bile ducts

PSC

Which is noninvasive and has no risk of pancreatitis or cholangitis?

MRCP

What is the tx for PSC?

Ursodiol (URSO), cholestyramine for pruritus, ERCP (to distinguish from PBC), liver transplant if advanced

What procedure?

• endoscope placed into bile duct → IV contrast outlines duct/pancreas & XRs are taken

• Therapeutic for:

  • sphincterotomy to remove stone

  • stent placed for drainage

ERCP

What is a noninvasive procedure that uses a powerful magnetic field, radio frequency pulses, and a computer to produce detailed pictures of hepatobiliary & pancreatic systems?

MRCP

PBC vs PSC

In what population is there an increased incidence of gallbladder carcinoma?

Elderly women

What are most gallbladder carcinomas?

Adenocarcinomas

What ist he 5th MC GI malignancy and has a poor prognosis?

GB carcinoma

What RF are associated with GB carcinoma?

Hx chronic cholecystitis, porcelain gallbladder** (calcification of GB itself)

The following ssx are associated with what condition?

• early → usually asx, incidental finding

• advanced → RUQ pain & mass, wt loss, malaise, jaundice

• dx w/ radiologic imaging

GB carcinoma

What is the treatment for GB cancer?

Cholecystectomy, wedge resection, LAD w/ large tumors