56yo male has an abnormal GB identified on a CT scan. What is the approx. likelihood that he will develop GB carcinoma?
- <20%.
- Porcelain GB, w/o soft tissue thickening.
- Low risk of GB carcinoma unless there are other associated findings, e.g., a soft tissue density mass.
This patient had abdominal pain + fever & a gallstone that was seen on US.
What is the name of this sign?
Dx?
- Rim sign: hyperenhancement of the liver adjacent to a thickened, inflamed GB wall (transmural GB inflammation).
- Dx: acute chole.
- This attenuation difference is different from THAD:
- THAD is usually small, wedge-shaped and subcapsular.
- In this case, the hyperperfused area abuts the GB only.
34yo female w/abdo pain + fever, but negative Murphy.
Dx: gangrenous chole.
- Laminated, discontinuous, markedly thickened GB wall.
- Adjacent ascites, extensive inflammation & absent Murphy add weight to the Dx.
- RUQ tenderness is absent due to destruction of the nerves in the GB wall by the transmural severe inflammation.
This gallstone is in the duodenum.
- What is the name of this syndrome?
- If the gallstone travels through the small bowel, what can occur?
- Bouveret syndrome.
- Gallstone ileus.
Dx?
Caroli syndrome.
- At least one “central dot” sign & dysmorphic/fibrotic liver from repeated cycles of inflammation/infection.
- Caroli syndrome = Caroli disease + hepatic fibrosis.
- This pt had a RLQ renal xplant b/c they had ARPCKD, which gives a spectrum of abnormalities resulting in variable degrees of fibrosis & cystic anomalies of the liver & kidney.
Dilated ducts Dx
Dx?
- How to differentiate b/w choledocholithiasis and an ampullary mass (cholangio)?
- Dx: extrahepatic, ampullary cholangio.
- For a mass: enhancing, eccentric.
- More often will have marked duct dilation (like this) and jaundice.
- Will not obstruct the pancreatic duct.
- For a stone: not enhancing, shadowing, central.
- May have any attenuation on CT.
- Will have signal voids on MRCP.
- Most will show GB stones.
- Less common to have marked duct dilation or jaundice.
Biliary MR
- Sequence type normally used for MRCP.
- Optimal T2 time for MRCP.
- Advantages over ERCP-3.
- Disadvantages compared to ERCP-3
- Effect of Eovist (gadoxetic acid disodium)
- Fast spin echo
- TE 80-100ms
- Advantages of MRCP:
- See extra-luminal findings.
- Can see obstructed ducts.
- Non-invasive.
- Advantages of ERCP:
- Intervention if required.
- Can actively distend biliary system w/contrast.
- Better spatial resolution.
- Eovist:
DDx cystic hepatic lesion
- Benign:
- Simple cyst
- von Meyenberg
- biliary cystadenoma
- Caroli
- Neoplastic:
- biliary cystadenocarcinoma
- cystic, necrotic met
- Infectious:
- abscess
- echinococcal cyst
Choledochal cysts
- List the different types.
- When are most diagnosed?
- Typical Tx & rationale.
- Most common type & prevalence.
- Association w/type V/Caroli.
- Defn Caroli syndrome.
- What forms the central dot of the central dot sign?
- See types below.
- Most diagnosed in childhood.
- Tx: resection or choledochoenteric drainage & surveillance for cholangioca; 25% increased risk of cholangioca.
- Most common type = type 1 (common = fusiform common bile duct dilation); prevalence = 50% of all Todanis.
- Caroli: assoc w/polycystic kidneys.
- Syndrome = Caroli + hepatic fibrosis.
- Central dot = portal vein + hepatic artery, i.e., the other 2 components of the portal triad, as the biliary duct is dilated.
Biliary anatomical variants
- What is the standard anatomy & prevalence.
- Next most common variant & prevalence.
- Problem w/low insertion of cystic duct.
- Aberrant R posterior duct:
- To where can this drain & prevalences?
- Issue w/aberrant R posterior duct.
- Standard anatomy: 63%
- LHD: branches into segs 2 & 3.
- RHD: branches into anterior & posterior.
- Next most common variants = RP into LHD (11%) & triple confluence (RAD, RPD, LHD).
- Low insertion: surgeon may misidentify cyst duct as common duct & ligate the CBD.
- Aberrant R:
- 3 anomalous connections: to LHD (11%); CHD (6%); cystic duct (2%).
- Issue: surgeons must anastomose 2 ducts if using a R lobe liver xplant.
Bile duct infection/inflammation:
- What symptom is common to the -itis conditions and not PBC?
- Ascending cholangitis & PSC present w/fever, as they are inflammatory conditions, PBC does not.
Bile duct infection/inflammation: ascending cholangitis
Px: general cause, most common cause.
- Other causes-3
Sx: clinical triad. Clinical pentad.
US-2
CT-2
Tx-3; prognosis.
Px: general = biliary tree blockage; most common cause = choledocholithiasis.
- Others: 20% malignancy; PSC; ERCP.
Sx: Charcot’s triad (Charco = yello): jaundice, RUQ pain, fever.
- Reynold pentad: Charcot’s triad + shock + delirium.
US: thickened bile duct walls +/- debris w/in the biliary system may be present.
CT: hyperenhancement & thickening of bile duct walls often w/a CBD stone.
Tx: initially w/Abx & fluids, & if refractory, ERCP: stone removal & sphincterotomy.
- Prognosis: 50-90% mortality if severe, acute.
Bile duct infection/inflammation: PSC
Px: 1
Ex: IBD assocn; sex;
ERCP/MRCP: app; which parts of the biliary tree most affected?
How differs from HIV cholangiopathy?
Long-term complications-3
- Px: idiopathic inflammation
- Ex: assoc w/UC (the two -itis-es are together, Crohn’s doesn’t have an -itis).
- 75% of pts w/PSC have UC.
- Only 4-5% of UC pts have PSC.
- More common in males.
- ERCP/MRCP: beaded, irregular appearance of the CBD & central intrahepatic ducts.
- Mnemonic: it’s more common in males, who are larger than females; likewise, PSC affects the larger ducts.
- HIV cholangio: similar app, but HIV more commonly associated w/papillary stenosis.
- Complications: cholangio, recurrent biliary infections.
Bile duct infection/inflammation: PBC (primary biliary cholangitis, but used to be cirrhosis)
Px: which ducts affected?
Ex: who?
Sx: common initial presentation?
Sequela?
Px: unlike PSC, this affects smaller ducts (women are smaller).
Ex: middle-aged women; (mnemonic: PBC; B stands for “broads”).
Sx: pruritis, but also fatigue.
Sequela: cirrhosis.
Bile duct infection/inflammation: AIDS cholangitis/cholangiopathy
Px: 2 bugs involved.
Ix: common feature & unique; % of cases w/the unique feature.
- Px: Cryptosporidium & CMV.
- Ix: similar appearance to PSC, but w/papillary stenosis & upstream CBD dilation.
- Only 50% of cases have papillary stenosis.
Bile duct infection/inflammation: chemoTx-induced cholangiopathy/cholangitis
Px: cause.
Ix: ducts affected/spared.
- Px: caused by intra-arterial chemotherapy to treat liver mets.
- Ix:
- Affects: central ducts, i.e., CHD & R/L main duct.
- Spares: the bookending ducts, i.e., CBD & peripheral intrahepatic ducts.
Bile duct infection/inflammation: recurrent pyogenic cholangitis (Oriental cholangiohepatitis)
Px: bug involved; what does this lead to?
Ex: where?
Ix: triad.
Sequela-1
- Px: Clonorchis sinensis.
- These form pigment stones, then biliary stasis, then cholangitis.
- The fluke acts as a nidus for stone formation.
- Southeast Asia.
- Ix: pneumobilia, lamellated bile duct filling defects (hyperdense to liver parenchyma), intra- & extrahepatic bile duct dilation & strictures.
- GB calculi are usually not present.
- Lateral L lobe often affected, & may see atrophy.
- At risk for cholangiocarcinoma.
Biliary neoplasia: biliary cystadenoma
Px: cell type; does it communicate w/the biliary tree?
Ex: sex?
Ix: general appearance; how to differentiate from other similar entities.
Tx:
Sequela.
- Px; cystic structure lined by columnar or cuboidal cells found in biliary ducts; NO communication w/ the tree (like von Meyenburgs).
- Like PBC, in middle-aged women.
- Ix: large, unilocular or multilocular mass.
- Septations may mimic an echinococcal cyst.
- A thick, enhancing wall is not present, unlike abscess or necrotic mets.
- If a thick calc or solid component is present, think biliary cystadenocarcinoma.
- Tx: although benign, if resected, they can recur.
Biliary neoplasia: cholangiocarcinoma
- Most common type? Rarest?
- From where in the biliary tree do these not arise?
- What does this tumour tend to do? …and cause what 2 things?
- RFs-7.
- Most common: Klatskin; rarest: peripheral.
- Will not arise from the GB or ampulla.
- Tends to obstruct bile ducts, then cause ductal dilation and lobar atrophy.
- RFs:
- Caroli/choledochal cyst
- choledocholithiasis more than cholelithiasis
- cirrhosis
- PSC
- infections: Clonorchis sinensis; HIV, hep B/C
- toxins: thorium dioxide: not used since the 1950s; polyvinylchloride; heavy EtOH.
- FAP syndrome (familial adenomatous polyposis)
Thorium dioxide:
- This is associated w/cholangioca, but what other 2 entities as well?
- HCC
- angiosarcoma
Biliary neoplasia: GB carcinoma
- Px-1; major cell type.
- Ex-1, sex.
- Ix: 3 Ix presentations
- 3 types of tumour spread.
- Prognosis-2.
- Due to GB inflammation–any kind.
- 90% adenocarcinomas, 10% squams.
- RARE! Elderly women (4:1, F:M).
- 3 Ix appearances:
- Common: scirrhous infiltrating mass that invades through the GB wall into the liver.
- Uncommon: polypoid mass.
- Rare: mural thickening.
- Tumour spread: directly into the liver; lymphatic; hematogenous.
- Prognosis: often poor, but small polypoid lesions may undergo curative resection.
- 5-year survival 1-5%.
Biliary neoplasia: GB mets
- Ex: how common?
- Most common met.
- 2nd most common.
- Ix: US, CT, MR
- Rare: <5% of all GB cancers.
- 1st: melanoma (50-70% of cases).
- 2nd: gastric carcinoma (in Asians).
- US: hyperechoic masses w/broad base & flow.
- CT: enhancing polypoid masses (one or more), often w/associated mural thickening; enhancement will vary w/hypervascular vs. non-mets.
- MR: T1 often hyper b/c of melanoma & T2 hypo.
- Which syndrome has a higher incidence of ampullary carcinoma?
- Typical clinical presentation?
- Gardner syndrome.
- Painless jaundice.
Dx?
Dx: type 4A choledochal cyst.
