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125 Cards in this Set

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Are minor congential anomalies of the biliary tract common or rare?
common
Are major congenital anomalies of the biliary tract common or rare?
rare
What is the clinical significance of minor congenital anomalies of the biliary tract?
These have no clinical significance unless surgery is needed-we do not want to injure the gall bladder or ducts inadvertently.
Abberant location of the gall bladder and irregular but functional biliary tract branches-->are these examples of minor or major anomalies?
Minor anomalies
5-10% of gall bladders are embedded in the liver. What type of anomaly (major or minor) is this an example of?
Abberant location-->Minor anomaly
What is a folded fundus called?
Phrygian cap
A golding over of the fundus of the gall bladder is called what?
Folded fundus or phrygian cap.
Gall bladder: agenesis, duplicated, bilobed-->these are all examples of a major or minor gall bladder anomaly?
Major anomaly
Are congenital anomalies involving location major or minor?
Minor
Are congenital anomalies involving structure major or minor?
Major
Bile duct structural anomalies are often associated with what?
Other anomalies, involving the heart, spleen, or GI tract (all the one-sided structures)
Bile Duct: Agenesis of all or part, Atresia-fetal, perinatal, Polysplneia (multiple spleens) are all examples of a minor or major anomaly?
Major anomaly
What is cholelithiasis also known as?
Gallstones
What are the 2 major types of cholelithiasis?
1.Cholesterol Stones
2.Pigment (bilirubin) stones
-Common in western, industrialized nations.
-Common in Mexican and Hispanic & Native Americans
-When the stones form individually, they are usually round, whereas when they form in large numbers simulataneously, will have a faceted surface
WHAT TYPE OF CHOLELITHIASIS?
Cholesterol Stones
Cholesterol Stones
What type of cholelithiasis?
What causes cholesterol gallstones?
1.High cholesterol or low bile salts-->crystallization of cholesterol, forming gallstones
2.Bile Stasis
What are the 2 most important factors for increased risk of high cholesterol?
Increased age, female gender (estrogen)
Changes in hormone or nervous control of BG, and starvation, can lead to what?
Bile Stasis-->Cholesterol Stones
Increased cholesterol, decreased lecithin, and decreased Na taurocholate all cause what?
Increase risk of cholesterol stone formation
What is the pathogenesis of cholesterol stone formation?
1.Increase chol
2.Decrease Lecithin
3.Decrease Na taurocholate
1.Supersaturated bile
2.Hypomotile GB (stasis)
3.Accelerated nucleation
4.Mucus hypersecretion
All of these simulatneous defects are needed for what?
Cholesterol Gallstone formation
What are the 4 simultaneous defects needed for cholesterol gallstone formation?
1.Supersaturated bile-liver hypersecretion
2.Hypomotile GB (stasis)
3.Accelerated nucleation-proteins that aid in stone formation
4.Mucus hypersecretion-acts as "scaffolding" for stone formation
What do pure cholesterol gallstones look like?
Rare, yellow, radiolucent
What do cholesterol gallstones mixed with other components looke like?
Radio-opaque (white, grey, black) but usually sill remain difficult to see on x-ray.
Chol Gallstones
What is this?
Chol Gallstones
What is this?
-Often involved with infections not seen in the US, and hence are more prevalent in non-western, developing countries.
-Most common in Asian countries
WHAT TYPE OF CHOLELITHIASIS?
Pigment (bilirubin) stones
pigment (bilirubin) stones
What type of cholesthiasis?
Why are pigment (bilirubin) stones produced?
Any disorder that favors production of unconjugated bilirubin will favor pigment stone formation.
What are the 2 main types of pigment (bilirubin)stones?
1.Brown
2.Black
Which type of pigment stone is radioluscent, which is opaque?
1.Brown: Radioluscent
2.Black: Radio-opaque
Where do brown pigment (bilirubin) stones form from?
Form in infected bile ducts
Where do black pigment (bilirubin) stones form from?
Form in sterile bile in GB
What type of pigment (bilirubin) stones are responsible for chronic intravascular hemolysis?
Black pigment (bilirubin) stones
How does the epidemiology of cholesterol stones differ from the epidemiology of pigment stones?
1.Cholesterol Stones: Increase in Wester Industrialized countries, Mexican and Native Americans
2.Pigment Stones: Increase in Non-Western/Developing countries; Asia
What is cholesterolosis?
-An incidental finding (no clinical significance) in which there is hypersecretion of cholesterol from liver into the GB
Normally what does the GB do will excess cholesterol?
-Normally, the mucosa of the GB will take up the excess cholesterol and convert it to esters, and then eliminate it
In cholesterolosis, where do excess esters from the liver accumulate?
Esters accumulate in the lamina propria of the mucosa.
Cholesterolosis-->With xs. cholesterol, esters accumulate in the lamina propria of the mucosa.
What does this show?
Cholesterolosis-->Mucosa with yellow flecks; "strawberry GB"
What is shown?
In cholesterolosis, what does the GB look like on dissection?
-Mucosa with "yellow flecks" flecks; "strawberry GB"
-(Microscopically forms a club-like swelling of the mucosal surface which corresponds grossly as a yellow flecked surface of the mucosa.
Cholelithiasis
Shown is a gross illustration of what?
Are patients with cholelithiasis symptomatic?
Usually not, only 1/3 of patients present with symptoms, usually PAIN
In cholelithiasis, what is pain usually associated with?
Pain is associated with the stone obstruction?
What is the consequent inflammation associated with GB obstruction in cholelithiasis called?
Cholecystitis
What is intermittent GB pain due to obstruction called in Cholelithiasis?
Colicky
In cholelithiasis, are small stones or large stones at a greater risk for obstructions?
Small stones
In cholelithiasis, what do large stones often do? What is this called?
Large stones do not often obstruct, but can erode their way out of the GB, and into the small intestine and obstruct it-->called gallstone ileus
Empyema, perforation, fistulae, inflammation of biliary tree (cholangitis) are all complications of what?
Cholelithiasis
What sized stones increase the risk of GB carcinoma?
All sized stones
What is GB inflammation called?
Cholecystitis
What is cholecystitis often associated with?
Gallstones
What is one of the most common indications for abdominal surgery?
Cholecystitis
What are the risk factor for cholecystitis similar to?
Same as those for gallstones.
What is an acute chemical irritation, inflammation of an obstructed gallbladder called?
Acute calculus cholecystitis
Blockage of neck or cystic duct-->bile cannot flow out-->bile becomes toxic lysolecithin (detergent)-->this disrupts the mucosa leading to prostaglandin release, inflammation, dysmotility, distention, and eventually decreased blood flow-->ischemia of GB tissues-->possible necrosis and serious side effects
This sequence describes the pathogenesis of what?
Acute calculus cholecystitis
RUQ pain, fever, anorexia, N/V...these are all clinical features of what?
Acute calculus cholecystitis
What does hyperbilirubinemia without jaundice indicate? And in what illness is this found?
Acute calculus cholecystitis
-Hyperbilirubinemia without jaundice
-Increased WBC's
-Increase serum alkaline phosphatase
-Previous episode(s) likely
-Surgical emergencies may happen if necrosis occurs
These are clinical symptoms of what illness?
Acute calculus cholecystitis
What is the difference b/w Acute calculus cholecystitis and Acute Acalculus cholecystitis?
1.Acute calculus cholecystitis-->with gallstones
1.Acute Acalculus cholecystitis-->withOUT gallstones
In what illness are patients severly ill with additional circumstances such as severe trauma, burns, multi-system organ failure, sepsis, postoperative state, postpartum state?
Acute Acalculus Cholecystitis
What is Acute Acalculus Cholecystitis due to?
Due to ischemia and poor perfusion of the GB
-cystic artery is an end artery, no collaterals
-essentially patients start at end point of acute calculus cholecystitis, they begin with decreased mucosal profusion
-At a much greater risk for complications
What illness can rarely be due to a bacterial infection?
Salmonella typhi, stapylococci, clostridia
Often clincial features of this illness are insidious. They are obscured by serious conditions not related to the gallbladder. We must maintain a high level of suspicion to avoid the risk of gangrene and perfusion. What illness?
Acute Acalculus Cholecystitis
Acute Cholecystitis GB-->GB is ENLARGED with thickened walls, RED-purple in color, may be BLOTCHY due to neutrophil migration to the serosa, showing a suppurative, fibrin-filled exudates
What is shown?
Wall of GB in Acute Cholecystitis. Edema, hyperemia (red). If gangrenous: green-black (necrotic) with perforation
What is shown?
-Acute Cholecystitis: Microscopioc Morpholgy
-Acute inflammatory reaction with neutrophils, edema, BV congestion
What is shown?
What is the differential diagnosis of acalculous v calculous cholecystitis?
No specific differences except for absence or presence of stones
1.Usually associated with gallstones
2.Thought to be due to recurrent attacks of cholecystitis
3.In 1/3 of cases (E.coli, enterococci) are found in GB, but not thought to be the cause
WHAT IS THE ILLNESS?
Chronic Cholecystitis
What does the GB serosa look like in chronic cholecystitis?
Smooth serosa
What does the GB wall look like in chronic cholecystitis?
Thick, opaque, gray-white wall, NOT red
What does the GB lumen look like in chronic cholecystitis?
Clear green-yellow mucoid bile
What does the GB mucosa look like in chronic cholecystitis?
Preserved mucosa
What is a "porecelain" GB wall? And what illness is it associated with?
1.Wall is calcified (associated with cancer)
2.Chronic cholecystitis
What are hyrdops? And what illness is it associated with?
Hydrops=clear secretions into GB
Associated with chronic cholecystitis
Chronic Cholecystitis-->hydrops, clear secretions into GB
What is shown in this GB?
Chronic Cholecystitis: "Porcelain" GB-->wall is calcified (associated with cancer)
What is shown?
-Smooth serosa
-Thick, opaque grey-white wall, NOT red
-Clear, yellow mucoid bile in lumen
-preserved mucosa
-"Porcelain" GB: wall is calcified (associated with cancer)
-"Hydrops" clear secretions into GB
What illness?
Chronic Cholecystitis-Gross Morphology
-Lymphocytes, plasma cells, macrophages
-Whiteness and thickness of wall comes from fibrosis
-Rokitansky-Ashoff sinuses
WHAT ILLNESS?
Chronic-Cholecystitis-Microscopic Pathology
What are Rokitansky-Ashoff sinuses? In what illness are they found?
1.Invagintion of mucosal epithelium deep into wall of GB. Benigh reactive change that mimics invasive carcinoma.
2.Chronic cholecystitis
Chronic Cholecystitis: Microscopic Morphology
1.Lymphocytes, plasma cells, macrophages
2.Whiteness and thickness of wall comes from fibrosis
3.Rokitansky-Ashoff sinuses
What is shown?
1.Epigastric, RUQ pain, N/V
2.Recurrent attacks
3.Fatty food intolerance
CLINICAL FEATURES OF WHAT ILLNESS?
Chronic Cholecystitis
1.Bacterial superinfection-->cholangitis, sepsis
2.Perforation-->abscess
3.Rupture-->peritonitis
4.Aggravate a pre-existing illness
WHAT ARE THESE COMPLICATIONS OF?
Complications of Acute and Chronic cholecystitis
A stone in any biliary duct, although many consider it to properly refer to a stone in the common bile duct. WHAT IS THIS CALLED?
Choledocholithiasis
In choledocholithiasis, where do stones form in the:
1.west
2.asia
1.West-stones form in GB
2.Asia-stones form in ducts due to infection
In choledocholithiasis, what do symptoms depend on?
Symptoms depend on where obstruction occurs:
1.obstruction (pancreatitis)
2.Infection (acute cholangitis)
3.Concurrent cholecystitis
Bacterial infection of the bile ducts due to obstruction is called what?
Acute cholangitis.
How does bacterial infection of the bile ducts occur in acute cholangitis?
Enteric bacteria enter ducts via sphincter of oddi due to obstruction.
What bacteria is most commonly involved in acute cholangitis?
E.coli or Klebsiella (gram-bacilli)
What is ascending cholangitis?
Cholangitis that ascends into intra-hepatic duct system.
What is the classic triad of symptoms in acute cholangitis?
Fever/chills, abdominal pain, jaundice
What is the microscopic morphology of acute cholangitis?
Mural neutrophils move toward lumen
Acute cholangitis-->mural neutrophils move toward lumen
What is shown in this GB?
What is the most severe form of acute cholangitis?
Suppurative cholangitis is most severe form-->purulent bile and spesis
This congenital anomaly involves complete obstruction of the extra-hepatic biliary tree for first 3 months of life...WHAT IS IT?
Biliary atresia
What causes congential biliary atresia?
Inflammation-->obstruction-->secondary changes in extra-hepatic ducts and hepatocytes-->secondary biliary cirrhosis
-Most frequent cause of death from liver disease in childhood
-50-60% referred for liver transplants
WHAT CONGENITAL ILLNESS?
Congenital Biliary Atresia
What are the 2 main forms of congenital biliary atresia?
2 main forms are based on timing of duct obstruction
1.Perinatal form
2.Fetal form
Thought to occur after birth, the biliary tree is destroyed. There may be a genetic inheritance, with a viral (reovirus or rotavirus), or toxic insult. WHAT FORM OF CONGENITAL BILIARY ATRESIA?
Perinatal form
Occurs intrauterine, often along with other anomalies of organs. WHAT FORM OF CONGENITAL BILIARY ATRESIA?
Fetal form
-F>M, Asian/African Americans>Caucasian Americans
-Stools initially normal, then pale/acholic (no bile)
-Bilirubin increases
-Increae in aminotransferase and alkaline phosphatase
WHAT ILLNESS ARE THESE THE CLINCIAL FEATURES OF?
Congenital Biliary Atresia
In biliary atresia, what does an intrahepatic duct liver biopsy show?
1.Porta edema/fibrosis
2.Ductile proliferation
3.Parenchymal cholestatsis in ALL ducts
What are the features of extrahepatic bile ducts in congenital biliary atresia?
Inflammation-->fibrosis & stricture-->obstruction
What type of congential biliary atresia involves the common bile duct only and correctable surgicallY?
Type 1 congenital biliary atresia
What type of congenital biliary atresia involves the hepatic duct only and is correctable surgically?
Type 2 congenital biliary atresia
What type of congenital biliary atresia is at or above the porta hepatic? Is this correctable by surgery?
1.Type III
2.NOT correctable surgically b/c there is not patent ductal system for anastomosis
-Complications:
-->cirrhosis by 3-6 mo. age
-->death by 2 yrs age
-Treatment:
-->cure: liver transplantation with donor bile ducts
WHAT ILLNESS?
Congenital biliary atresia.
Congenital dilations of the common bile duct are called what?
Cholendocal Cysts
What are choledochoceles?
Cystic lesions protruding into duodenal lumen-->assoc. with choledocal cysts
-Most present before 10 years of age, includes:
1.duct dilations
2.diverticuli
3.choledochoceles
WHAT ILLNESS?
Choledocal Cysts
-Symptoms:
-->Jaundice, biliary pain, RUP mass
-->More common in females (3-4 times)
-Complications:
-->Most imp. is increased risk for bile duct carcinoma (for older patients)
-->Also have stone formation, stenosis, pancreatitis, and liver changes
WHAT ILLNESS?
Choledocal Cysts
a.Biliary tract tumors
b.Inflammatory polyps
c.Adenomyosis
THESE ALL HAVE WHAT IN COMMON?
These are all benign biliary tract tumors
What is adenomyosis?
-Benign Biliary Tract Tumor
-Increased number of intramural glands in hyperplastic muscularis (smooth muscle)
What are characteristics of carcinomas of GB and extra-hepatic bile ducts?
1.Both uncommon
2.Both more in older populations F>M
3.Both diagnosed at stage too late to resect surgically b/c they grow insidiously
4.Both related to chronic inflammation
-->West:associated with gallstones
-->East:associated with infections, parasitic disease
What is the most common site to have carcinoma of the GB?
Fundus, neck
Is carcinoma of the GB more common in females or males?
Females
Does carcinoma of the GB usually have an infiltrating growth pattern or an exophytic growth pattern?
Infiltrating growth pattern
What are most carcinomas of the GB?
Adenocarcinomas: may be poor or well differentiated
What are 5% of carcinoma's of the gallbladder?
Squamous Cell or Adenosquamous
-Preoperative diagnosis is uncommon-->incidental diagnosis
-In the best case scenario diagnosis should be made before tumor extends due to WHAT 2 THINGS?
1.Palpable gallbladder
2.Acute cholecystitis
What is a cholangiocarcinoma?
Carcinoma of extra-hepatic biliary tree
-Painless, progressively increasing obstructive jaundice
-Increase in serum alkaline phosphatase, aminotransferases, direct bilirubin
-Males more common than females; 1/3 have gallstones
-Increase risk with chronic inflammation: Primary sclerosing cholangitis, ulcerative colitis, choledochal cysts
WHAT CARCINOMA?
Cholangiocarcinoma-Carcinoma of extrahepatic biliary tree
-Infiltrative, malignant glands, that leads to fibrous stromal reaction causing mass to be hard
-What type of cancer?
Cholangiocarcinoma-->Adenocarcinoma
At junction of R/L hepatic ducts; slow growing, rarely metastasize
WHAT TUMOR?
Klatskin tumor