What is the pathology behind PSC? What is the natural history? What is the median survival?
Fibrosing inflammation Chronic progression from initial cholangitis to periportal hepatitis to septal fibrosis to bridging necrosis to cirrhosis 12 years
What are the cholangiographic features of PSC?
Band strictures (beaded appearance) Nonuniform, segmental strictures Pruned tree appearance of intrahepatic ducts Diverticular outpouching Mural irregularity
What is the feared complication with PSC? When is is suspected?
Cholcangiocarcinoma Suspect when there is a dominant stricture
What is the mechanism for PBC? What diseases is it associated with? What is the marker? What is the pathophysiology
T-cell mediated immune response RA, Sjogrens, Hashimotos Antimitochondrial antibody Small duct destruction with inflammatory cellular infiltrate
What are the cholangiographic features of PBC? Early vs late
Early - may be normal Late - Cirrhosis, crowding, tortuosity and deformity of the bile ducts. Enlarged benign reactive lymph nodes in the hepatic portal
What are the bugs related to AIDS induced cholangiopathy?
Cryptosporidium and CMV
Intrahepatic ductal strictures with papillary stenosis?
What feature is unique to AIDS cholangiopathy?
What is ascending cholangitis?
Obstruction of the biliary tree with bacterial infection of the bile.
What is a common liver finding in ascending cholangitis?
Cystic hepatic lesion communicating with biliary tree in septic patient?
Hepatic abscess complicating ascending cholangitis
What is the most common bug in ascending cholangitis?
Bile duct stones Ductal dilation and focal strictures Acute peripheral tapering Decreased arborization of intrahepatic ducts
What is the bug in oriental cholangiohepatitis
Clonorchis, Ascariasis, nutritional deficiency
What is the main difference between PSC and PBC?
PBC affects small bile ducts only
What is the target sign in relation to choledocholithiasis?
Ring of water attenuation around a stone
What is the crescent sign in relation to choledocholithiasis
Only a crescent of water surrounds the calculus
What are the 3 types of gallstone?
1) cholesterol 75% 2) pigmented 20% - crystallization of calcium bilirubinate, can be black (blood, sickle cell) or brown (bile, infection) 3) Mixed
What is Admirand's triangle
Balance of cholesterol, bile acids, and lecithin (phospholipid) in the gallbladder
What is a pseudocalculus?
Apparent filling defect within the ampulla due to sphincter of oddi spasm
What is a helpful sign to differentiate pseudocalculus from real stone?
Pseudocalculus will only have a crescent along the superior aspect that disappears with time/glucagon Real stone will have a crescent along the inferior aspect as well.
Differentiate spasm of sphincter of oddi with Papillary stenosis?
Spasm will relax over time
What is a sign of fixed narrowing of the ampulla?
Upstream dilation of both the common bile and pancreatic.
What are the associations with ampullary carcinoma?
Inflammatory stricture of the common hepatic or common bile duct due to impacted stone in the cystic duct or neck of the gallbladder.
What is the most common cause of common hepatic duct narrowing?
Multiple and recurrent papillary adenomas in the biliary tract. US - multiple solid filling defects attached to the wall of a dilated extrahepatic duct extremely rare
Are papillary adenomas premalignant?
Multiloculated cystic lesion along the biliary tree with well defined thick capsule? Seen in middle aged white women
Differentiate cystadenoma from echinococcal and hepatic abscess
Echinococcal - peripheral intracystic cysts and wall calcification and history of travel to endemic area Abscess - multiple well defined cystic masses with rim hyperenhancement and fevers/leukocytosis
What are signs of a biliary cystadenocarcinoma over cystadenoma?
Carcinoma: thick septae enhancing nodular components calcifications
What are the two types are biliary cystadenocarcinoma> which one has a better prognosis?
Those that contain ovarian stroma and those that do not Ovarian stroma favors better prognosis
What is the analogy to intraductal cholcangiocarcinoma?
Which cholangiocarcinoma has the best prognosis?
Those growing in the CBD (ie extrahepatic) have easier chance for resection
What is a klatskin tumor?
Cholangiocarcinoma involving the upper common hepatic duct
What is a limiting factor for removal of a klatskin tumor?
One hepatic lobe must be tumor free
What is the enhancement pattern of a klatskin tumor?
Hypo/isoattenuating compared to liver during arterial and portal venous phase Enhances on delayed phase
Why do scirrhous cholangiocarcinomas enhance on delayed imaging?
They incite a desmoplastic reaction
What are the two types of cholangiocarcinoma?
Intra and extrahepatic
What is the most common biliary tract anomaly?
Aberrant intrahepatic duct draining into a contralateral main or common hepatic duct.
Are biliary leaks more common with laparoscopic or open cholecystectomy?
Name 3 associations with choledochal cysts
Biliary tract stenosis/atresia Congenital hepatic fibrosis Medullary sponge kidney
What is the patholophysiology choledochal cysts
Anomalous connection between pancreatic duct and biliary system allows pancreatic secretions to weaken the wall of the duct
choledochal cyst types
1 - fusiform
2 - pedunculated
3 - at ampulla, choledochocele
4 - intra and extrahepatic
5 - intrahepatic (carolis)
What is a type 3 choledochal cyst?
What is carolis disease? is it AR or AD? How does it present radiographically? What are the associations?
What is a good differentiating feature between carolis and PSC?
Autosomal recessive disease, results in alternating regions of narrowing and saccular/fusiform dilatation within the intrahepatic ducts
Associated with renal diseases (medullary sponge kidney, polycystic kidney disease, medullary cystic disease)
PSC is rarely saccular
What are the risks with carolis disease?
100x risk of adenocarcinoma
What biliary disease does renal tubular ectasia have an association with?
How does gangrenous cholecystitis present on US? CT?
Intramural edema or sloughing of the mucosa into GB lumen
Pericholecystic fluid collection due to perforation
What bug is associated with emphysematous cholecystitis? What disease is associated and what percentage of patients have it?
How does gas appear on US?
Dirty acoustic shadowing
vs. clean shadowing such as that seen with a calculus
What are 6 causes of acalculous cholecystitis?
What is the cause of porcelain gallbladder?
Chronically obstructed cystic duct
What is the risk and percentage of that risk associated with porcelain gallbladder
25% incidence of gallbladder carcinoma
What is the wall echo shadow sign?
Seen in a gallbladder full of stones
2 echogenic lines with an intervening sonolucent line
What is milk of calcium bile?
Calcium carbonate/phosphate/bilirubinate that is highly concentrated within the GB due to cystic duct obstruction
What is gallbladder sludge?
Concentrated, echogenic bile containing particulate material consisting of cholesterol crystals and calcium bilirubinate granules
What is tumefactive sludge?
Ball of sludge, can mimic mass but wont have posterior shadowing
What makes up a cholesterol polyp?
Single layer of epithelium covering a core of cholesterol filled cells
How does metastatic melanoma present in the gallbladder?
Multiple polypoid intraluminal masses
Echogenic foci within a thickened gallbladder wall with comet down artifact suggests what? What is the pathology behind it?
Convoluted infoldings of the normal gallbladder mucosa (rokitansky aschoff sinuses) with smooth muscle proliferation
What is focal adenomyomatosis?
Can cause segmental narrowing and distort lumen with a masslike appearance
How many gallbladder carcinomas present as polypoid lesions? What is the most common manifestation? How are the differentiated from sludge on US?
Infiltrating mass arising from gallbladder fossa extending into the liver
Blood flow on doppler will be seen with cancer
What are the two types of gallbladder cancer
Schirrous - infiltrates liver from gallbladder
Polypoid - grows into GB lumen
What is the most common tumor to metastasize to the gallbladder?
What is a phrygian cap?
Congenital infolding of the gallbladder wall with no known clinical significance
Differentiate between a phrygian cap and focal adenomyomatosis?
Phrygian cap will always be in the fundus and have a THIN membrane
Adenomyomatosis will be thicker and can be located anywhere
What is the cutoff for a normal gallbladder wall thickness? What are some causes?
Cholecystitis, adenomyomatosis, hepatitis, cirrhosis, portal hypertension, hypoproteinemia, CHF, renal failure, pancreatitis
Cystic thickening of the gallbladder wall is seen with what?
Gallbladder varices should prompt a search for what?
Portal vein thrombosis
Name 5 cystic duct variations
Low insertion onto CBD
Conjoined cystic and CBD
Absent cystic duct
Insertion into RHD
Insertion at bifurcation of CHD
Name 3 intrahepatic ductal variations
Right posterior duct into the LHD
Right posterior into the CHD
Right posterior into the CHD bifurcation