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● The extrahepatic biliary tree consists of the right and left hepatic ducts, the common hepatic duct, the
cystic duct, & the common bile duct.
● As they exit the liver, the left hepatic duct is longer than the right hepatic duct & has greater
propensity for dilation due to distal obstruction.
● Shortly after, they unite to form the common hepatic duct. It is 1-4 cm long w ith a 4 mm diameter. It is
located anterior to the portal vein & lateral to the hepatic artery.
● The cystic duct is the segment immediately adjacent to the gallbladder. It possesses mucosal folds
called spiral valves of Heister; they do not have any valvular function, but makes cannulation difficult.
● The common bile duct is formed by the union of the cystic duct & common hepatic duct. It is 7-11 cm
long w ith a 5-10 mm diameter.
○ The upper third (supraduodenal portion) passes dow nw ard in the free edge of the
hepatoduodenal ligament, anterior to the portal vein & lateral to the hepatic artery.
○ The middle third (retroduodenal portion) curves behind the 1st portion of the duodenum &
diverges laterally from the portal vein & hepatic artery.
○ The low er third (pancreatic portion) curves behind or traverses through the pancreatic head.
It enters the w all of the 2nd portion of the duodenum & opens through a papilla of mucous
membrane called the ampulla of Vater.
○ Its union w ith the main pancreatic duct follow s 1 of 3 configurations:
■ 70% unite outside the duodenal w all & enter as a single duct
■ 20% unite w ithin the duodenal w all but open through the same opening, and
■ 10% exit the duodenal w all via separate openings. This is also called pancreas
divisum
○ The sphincter of Oddi is a thick coat of circular smooth muscle surrounding the CBD at the
ampulla of vater. It functions to control bile flow .
● The mucosa of the BDs is a simple columnar epithelium with mucous glands concentrated in the
CBD. The mucosa is surrounded by fibro-areolar tissue with scant smooth muscles. It does not,
however, have a distinct muscle layer.
● For its blood supply, gastroduodenal & right hepatic artery branches run along the medial & lateral
w alls (at the 3 o’clock or 9 o’clock positions).
● Its nerve supply is the same as the GB, w ith higher fiber density near the sphincter of Oddi
● The boundaries of the hepatocystic triangle of Calot are the cystic duct, common hepatic duct, &
inferior edge of the liver.
● It contains the Calot’s node, & it is also w here the cystic artery usually branches off the right hepatic
artery. This triangular space is dissected to allow the surgeon to identify, divide, and ligate the cystic
duct & artery.
● Cystic duct length & course are quite variable. They are surgically important to know because
misidentification can lead to bile duct injuries.
A. Low junction betw een the cystic duct and common hepatic duct.
B. Cystic duct adherent to the common hepatic duct.
C. High junction betw een the cystic and the common hepatic duct.
D. Cystic duct drains into right hepatic duct.
E. Long cystic duct that joins common hepatic duct behind the duodenum.
F. Absence of cystic duct.
G. Cystic duct crosses posterior to common hepatic duct and joins it anteriorly.
H. Cystic duct courses anterior to common hepatic duct and joins it posteriorly.
● Variations of the origin & course of the hepatic & cystic arteries are also common.
A. Cystic artery from right hepatic artery (80% to 90%).
B. Cystic artery off the right hepatic artery arising from the superior mesenteric artery (accessory or
replaced) (10%).
C. Tw o cystic arteries, one from the right hepatic, the other from the common hepatic artery.
D. Tw o cystic arteries, one from the right hepatic, the other from the left hepatic artery.
E. The cystic artery branching from the right hepatic artery and running anterior to the common hepatic
duct.
F. Tw o cystic arteries arising from the right hepatic artery.
● When gallbladder or extrahepatic biliary disease is suspected, CBC & liver function
tests are usually requested.
● An elevated WBC suggests the presence of infection, i.e., acute cholecystitis &
cholangitis, and
● Elevated bilirubin & ALP suggest cholestasis, i.e., cholangitis, choledolithiasis, &
strictures
● Uncomplicated or chronic cases, however, may present with normal blood tests
● Transabdominal US is the initial imaging of choice. It is non-invasive, painless,
does not use radiation, & may be used in critically ill patients.
● It is, however, operator-dependent; and is made difficult by obesity, ascites, & a
distended bowel. The retroduodenal portion of the extrahepatic bile ducts are
also not adequately visualized due to presence of bowel gas.
● It is best used in visualizing the gallbladder.
○ It is >90% sensitive & specific for gallstones, which are acoustically
dense, associated with acoustic shadows & movements with posture
change. Occasionally, sludge, appearing as low amplitude homogeneous
echoes layering on the posterior wall, are seen instead of stones. It is
also used to diagnose acute & chronic cholecystitis, the features of which
we will discuss later.
○ GB polyps are also seen on US. They are calcified, with acoustic
shadows, but do not move with changes in position.
● The extrahepatic BD are also well-visualized except for the retroduodenal
portion.
● US is also helpful in assessing for tumor invasion & flow in the portal vein.
○ Malignant features: >10 mm, sessile, solitary lesion, interval increase in
size, CT/MRI enhancement greater than the normal gallbladder wall
● Normal
● US, cholelithiasis- stones within the gallbladder (white arrows) with acoustic
shadows (black arrowheads)
● CT scan is inferior to US in diagnosing
gallstones, but is similarly sensitive in diagnosing
acute cholecystitis. Its primary use is in the
assessment of the extrahepatic biliary tree &
adjacent structures, & for evaluating alternate
causes. It is the initial imaging of choice in
suspected malignancy.
● Normal
● Axial CT scan of the abdomen, contrast-
enhanced- calcified GS within the gallbladder
(arrow)
● Axial CT scan of the abdomen- cancer of the
distal CBD; 1= PV; 2= dilated intrahepatic bile
duct; 3= dilated cystic duct & GB neck; 4= dilated
CHD; 5= bifurcation of the CHA into GDA &
proper HA; 6= dilated pancreatic duct; 7= the
splenic vein.
● Hepatobiliary scintigraphy or hepatobiliary iminodiacetic acid (HIDA) scanning is
another option for non-invasive evaluation of the liver, gallbladder, bile ducts, &
duodenum. Technetium-labeled derivatives of iminodiacetic acid are injected
intravenously, taken up by the Kupffer cells in the liver, and excreted in the bile.
Uptake by the liver is usually detected within 10 minutes, & the gallbladder, bile
ducts, & duodenum are typically visualized within 60 minutes
● Its primary use is in the diagnosis of acute cholecystitis (95% sensitive & specific),
which appears as a non-visualized gallbladder, with prompt filling of the common
bile duct & duodenum.
● It is also helpful in evaluating for ampullary obstruction, post-operative biliary leaks,
& chronic biliary dyskinesia.
● ERCP combines endoscopy & fluoroscopy for the
evaluation of pancreticobiliary pathology. Using a
side-viewing endoscope, the common bile duct
can be cannulated through the ampulla of Vater
& a cholangiogram performed using fluoroscopy.
● Its diagnostic advantages include direct
visualization of amupllary region & direct access
to distal common bile duct.
● It also has the benefit of being therapeutic at the
same time. It is rarely needed for uncomplicated gallstone
disease, but is valuable for choledocholithiasis, cholangitis,
obstructive jaundice, or biliary strictures. If CBD stones are
visualized, sphincterotomy & stone extraction may be done.
If bile strictures are found, diagnostic brushings can be
obtained.
● Notable complications include pancreatitis (which
develops 3.5% of those undergoing the
procedure), as well as bleeding, perforation, &
cholangitis
● MRCP is a special type of MRI, & is a non-invasive option for a focused
assessment of pancreaticobiliary disorders. It is 95% specific & 89% sensitive for
choledocholithiasis. In manys some center, it is the preferred imaging modality,
reserving ERCP for therapeutic purposes only.
● Normal- the confluence of the right and left intrahepatic ducts to form the
common hepatic duct is seen (long thin arrow). The cystic duct (*) typically joins
the right side of the common hepatic duct to form the common bile duct (CBD)
(short arrow). The main pancreatic duct (arrowheads) drains along with the CBD
into the major duodenal papilla. An accessory pancreatic duct is present ( black
circle), draining into the minor duodenal papilla. Fluid containing structures such
as the gallbladder (GB), duodenum (D) and stomach (S) are well seen on this
T2-weighted sequence with the duodenum obscuring part of the biliary tree
● Shows early primary sclerosing cholangitis (PSC) with irregular dilatation and
strictures seen in the left sided intrahepatic ducts (arrows)
● Shows dilatation of the intrahepatic bile ducts with disconnection between the left
and right sided ducts and the common duct, due to a hilar cholangiocarcinoma
(arrow)
● Endoscopic choledochoscopy allows for direct
visualization of the biliary & pancreatic ducts,
thus increasing the effectiveness of ERCP.
● Extended mass represented as filling defect (black arrow) in the both intra-hepatic
duct, common hepatic duct, and common bile duct without dilatation of biliary
ducts. (source: https://www.researchgate.net/figure/Percutaneous-transhepatic-
cholangiography-which-was-obtained-20-days-after-follow-up-
CT_fig1_271254979)
● For the benign conditions of the gallbladder & extrahepatic biliary tree, we will first
discuss gallstone diseases.
● Gallstone disease is one of the most common afflictions of the digestive tract.
● Increased risk is associated with pregnancy, non-HDL hyperlipidemia, rapid weight
loss, Crohn’s disease, some blood disorders (HS, sickle cell disease, thalassemia),
surgeries altering normal bile regulation, & some medications (somatostatin
analogues, estrogen OCPs).
● 4 Fs: female, forty, fertile, fat
● GS form as a result of solids settling out of solution. The major organic solutes in
the bile are: bilirubin, bile salts, phospholipids, cholesterol.
● GS are classified based on their cholesterol content as cholesterol or pigment
stones.
● Cholesterol stones are more common, & account for 80% of GS in Western
countries.
● Pure cholesterol stones are uncommon, only accounting for 10%. They are often
single, large, & smooth.
● Mixed stones are more common. They are composed of at least 70% cholesterol
with bile pigments & calcium. They are often multiple, with variable size &
configuration. They are usually whitish-yellow to green or black.
● Cholesterol stones are often radiolucent.
● The primary event for their formation is supersaturation of bile with cholesterol,
most often due to cholesterol hypersecretion (either through increased intake or
dysfunctional processing of cholesterol).
● Pigment stones are more common in Asia. They are composed <20% cholesterol
and calcium bilirubinate. There are 2 types, black and brown.
● Black pigment stones are small, brittle, dark, & sometimes speculated. It results
from supersaturation of unconjugated bilirubin within the bile & subsequent
precipitation with calcium bilirubinate. Hemolytic disorders, cirrhosis, & liver
dysfunction increase the risk of their formation. Because of their high calcium
content, they are often radiopaque.
● Brown pigment stones are usually <1 cm, brownish-yellowish, soft, & mushy. They
often form secondary to bacterial infection (E. coli B-glucoronidase) & bile stasis
(parasite infection).
● (http://www.pathologyoutlines.com/topic/gallbladderchroniccholecystitis.html)
● (https://www.wikidoc.org/index.php/Chronic_cholecystitis_ultrasound)
● (https://www.thenakedscientists.com/podcasts/video-podcasts/naked-
pathology/hepato-biliary-pathology-cholelithiasis-chronic-cholecystitis)
● Acute cholecystitis is an acute inflammation of the gallbladder initiated by gallstone
obstruction of the cystic duct.
● Frequently, the gallstone is dislodged & inflammation resolves; however, 5-10%
progress to ischemia & necrosis while 15-30% are secondarily contaminated by
bacteria (most commonly E. coli, Klebsiella spp., and S. faecalis) & may lead to
gangrene, abscess, or perforation
● Patients present with a history compatible with chronic cholecystitis (with attacks of
biliary colic), but presently complain of a more severe, intractable pain. It is often
associated with fever, anorexia, nausea, & vomiting. However, elderly & diabetics
may have a subtle presentation.
● On physical examination, there may be tenderness, guarding, & a positive
Murphy’s sign (inspiratory arrest with deep palpation in the right subcostal area).
Occasionally, a palpable mass may be appreciated. Jaundice may be observed
when stones are impacted in the CBD or at the infundibulum (causing CHD
obstruction called Mirizzi’s syndrome).
● Laboratory tests usually show mild to moderate elevations of WBC, & mild
elevations of serum bilirubin, ALP, transaminases, & amylase.
● US is 70-90% sensitive & specific, with GB stones, GB wall thickening,
pericholecystic fluid, & a sonographic Murphy’s sign. In this photo, the white
arrowheads indicate the thickened gallbladder wall. White arrows show several
stones in the gallbladder, & the black arrowhead point at the acoustic shadows.
Black arrows point to trace pericholecystic fluid surrounding the gallbladder.
● HIDA scan is helpful if the diagnosis remains in question. It shows lack of GB filling
after 4 hrs, which indicates cystic duct obstruction. It is 90% sensitive when
correlated with the clinical presentation.
● According to the Evidence-Based Clinical Guidelines of the Committee on Surgical
Infections (2004?), HIDA scan is the most accurate diagnostic imaging test, but US
is a more practical initial test.
● Initial treatment includes IV fluids, broad-spectrum antibiotics, & analgesia.
○ Uncomplicated: cefazolin (1 g q8hrs), cefuroxime (1.5 g q8 hrs), cefoxitin (2
g q8hrs); alternative: FQ (400 mg q12 hrs) + metronidazole (500 mg q6hrs)
○ Complicated: cefoxitin (2 g q8hrs), ertapenem (1 g q24 hrs), AmpiSul (1.5-
3.5 g q6hrs) + gentamicin (240 mg q24 hrs; if with risk for enterococcal
infection)
○ Until 5-7 days post-op if with absence of fever >24 hrs, tolerate oral intake,
normal WBC ct with <3% bands
● Cholecystectomy is the definitive treatment. Early cholecystectomy performed
within 72 hrs is preferred due to quicker recovery times & earlier return to work.
Laparoscopy is the procedure of choice because it results in a shorter hospital stay
& a quicker convalescence.
● If patients are unfit for surgery, IV antibiotics are given & a cholecystostomy tube is
installed. Elective cholecystectomy is offered once acute inflammation resolves,
usually 6-10 weeks later.
● (https://www.ultrasoundcases.info/cholangitis-2978/)
● (https://www.youtube.com/watch?v=wfUKyDGzJCo&app=desktop)
● The following are surgical procedures performed for gallstone diseases.
● Percutaneous transhepatic cholecystostomy tubes are performed for
decompression & drainage of a distended, inflamed, hydropic, or purulent GB.
● It is usually indicated for patients with cholecystitis but is too ill to undergo
cholecystectomy.
● A pigtail catheter inserted percutaneously under ultrasound guidance, passing
through the abdominal wall, liver, & gallbladder. (Passing through the liver
minimizes the risk of uncontrolled bile leak into the peritoneal cavity.)
● Ultrasound shows 1) Dilated GB, 2) guidewire inserted (arrow), 3) 8Fr pigtail
catheter inserted over the guidewire (arrow)
● Removed when inflammation has resolved & patient’s condition has improved; but
before removal, first confirm a patent cystic duct by tube cholangiogram.
● In ERCP, 90o side viewing endoscope is used to enter the duodenum & visualize
the ampulla. The ampulla is then cannulated to allow wire & catheter access to the
biliary tree. This allows for retrograde cholangiogram, diagnostic brushings,
stenting, dilations, or fluoroscopically guided basket or balloon retrieval of CBD
stones.
● Sphincterotomy should be performed when CBD stones are present to allow for
passage of larger stones.
● Laparoscopic cholecystectomy is the treatment of choice for symptomatic
gallstones & complications of gallstone disease. It is minimally invasive, resulting
in minor pain & scarring, & early return to full activity. Absolute contraindications
are few, including hemodynamic instability, uncontrolled coagulopathy, & frank
peritonitis. Relative contraindications include severe COPD & CHF (EF< 20%)
because they may not be able to tolerate pneumoperitoneum.
● Conversion to open cholecystectomy may be necessary if the patient is unable to
tolerate pneumoperitoneum, an complication occurred that cannot be fixed
laparoscopically, important anatomic structures cannot be clearly identified, or no
progress is made over a set period of time.
○ Patient is typically positioned supine with the operating surgeon standing at
the patient’s left side. Pneumoperitoneum is established with carbon
dioxide.
○ A) Trocar placement. Typical access is at the supraumbilical region. A 5- or
10-mm trocar is inserted through which a 30o laparoscope is introduced.
Traditionally, 3 additional ports are then placed: 10- or 12-mm port in the
epigastrium, 5-mm port in the right MCL, & a 5-mm port in the right flank.
○ B) Through the lateral-most port, the assistant uses a locking instrument to
grasp the gallbladder fundus and retract it over the liver edge and upward
towards the patient’s right shoulder. Through the midclavicular port, the
surgeon uses a grasper to retract the gallbladder infundibulum laterally.
This exposes the proximal GB & hepatoduodenal ligament. Dissection can
then be performed through the epigastric port, utilizing a combination of
electrocautery & sharp and blunt dissection.
○ C) A critical view of safety should be obtained. Here is an Intraoperative
photo of the critical view of safety. The hepatocystic triangle has been
cleared of fat and fibrous tissue, the lower 1/3 of the GB is separated from
the liver to expose the cystic plate, & only two structures are seen entering
the gallbladder. At this point, an intraoperative cholangiogram can be
performed.
● Intraoperative cholangiogram is used for evaluating the extrahepatic bile ducts,
identifying CBD stones, or clarifying aberrant ductal anatomy. Routine vs selective
performance is still debated, but is indicated when there is history of abn liver
function tests, pancreatitis, jaundice, large ducts & small stones, dilated duct, & an
unsuccessful ERCP. Here, D) A clip is being placed on the cystic duct–gallbladder
junction. E) A small opening has been made in the cystic duct (on its anterior
surface), and a cholangiogram catheter is being inserted. It should be secured with
a clamp or a clip. A cholangiogram is then performed by injection of contrast
through the catheter during live fluoroscopic dynamic imaging. A) Ideally, it
includes filling of the right & left hepatic ducts, emptying into the duodenum, & no
visualized filling defects. B) Shows a common bile duct stone (arrow) with very little
contrast passing into the duodenum. Once the cholangiogram is completed, the
catheter is removed.
● Here, F) additional clips have been placed on the cystic duct & cystic artery. The
cystic duct has been divided, and the cystic artery is being divided.
● Finally, the gallbladder is dissected off the liver bed using electrocautery while
watching for potential abnormal posterior branches of ducts or arteries. Final
evaluation of the operative field is done. GB is then divided from its final
attachments & removed either through the epigastric or umbilical incision, often
with the aid of a retrieval bag. Any bile or blood that has accumulated during the
procedure should be cleaned away, & if stones were spilled, they should be
retrieved & removed.
● Open cholecystectomy is usually performed either as a conversion from
laparoscopic cholecystectomy or as a second procedure in patients who require
laparotomy for another reason. The same surgical principles apply for laparoscopic
& open cholecystectomies.
● CBDE is performed for management of CBD stones at the time of
cholecystectomy.
● If the stones are small, they may simply be flushed into the duodenum with saline
irrigation via the cholangiography catheter. If irrigation is unsuccessful, fluoroscopic
or endoscopic approaches are employed.
● I) Shows a transcystic basket retrieval using fluoroscopy. A) The basket has been
advanced past the stone and opened. B) The stone has been entrapped in the
basket, and together, they are removed from the cystic duct.
● II) Shows a transcystic choledochoscopy & stone removal- C) The basket has
been passed through the working channel of the scope, and the stone is entrapped
under direct vision. D) Entrapped stone. E) A view from the choledochoscope with
stone captured in basket. Alternatively, the stone may simply pushed into the
duodenum.
● Once the common bile duct has been cleared of stones, the cystic duct is ligated
below the level of the ductotomy and divided, and the cholecystectomy is
completed.
● Occasionally, however, an incision into the common bile duct itself is necessary.
● III) shows a choledochotomy & stone removal- F) A small incision is made in the
common bile duct. G.) The flexible choledochoscope is then passed into the duct
for visualization & clearance of stones. The choledochotomy can be closed
primarily if the duct is very large, or over a T-tube. (T-tubes are used to prevent
strictures & leaks associated with primary closure.)
● Here, a) A standard T-tube that has been cut and modified for use in the biliary
tract. B) The T-tube is placed through a ductotomy in the common bile duct with
the defect closed over the tube.
● Additional choledochal drainage procedures may be necessary for stones or
obstructions that cannot be cleared with ERCP & sphincterotomy or CBDE.
● Drainage procedures may also be used to manage CBD strictures or for palliative
treatment of malignant obstructions in the periampullary region.
● Transduodenal sphincterotomy- incising the duodenum transversely & cutting the
sphincter of Oddi at the 11 o’clock position. The stones can then be manually
removed or simply allowed to pass through.
● Bypass procedures:
○ Choledochoduodenostomy- used for short distance bypasses; A) the distal
CBD is opened longitudinally, as is the duodenum, B) interrupted sutures
are placed between the common bile duct and the duodenum, C)
completed choledochoduodenostomy.
○ Choledochojejunostomy- used for longer distances; D) CBD & small bowel
are divided, E) a limb of jejunum is brought up in a Roux-en-Y configuration
& anastomosed to the bile duct.
○ Hepaticojejunostomy- used if the entirety of the extrahepatic biliary tree
must be bypassed; F) the entire extrahepatic biliary tree has been resected
and the reconstruction completed with a Roux-en-Y limb of jejunum, G)
percutaneous transhepatic stents are placed across hepaticojejunostomy
(optional)
● PSC is a progressive disease characterized by inflammatory strictures of the intra-
& extrahepatic biliary tree which eventually results in biliary cirrhosis. Here, a
degenerating BD entrapped in a dense, onion-skin concentric scar
● The hepatic duct bifurcation is often the most severely affected
● Primary SC has no known cause, but has been attributed to autoimmune reaction,
chronic low-grade infection, toxic reaction, & genetics; it is often associated with
other autoimmune disease (2/3 of which with ulcerative colitis)
● Secondary sclerosing cholangitis- bile duct stones, acute cholangitis, biliary
surgery, toxic agents
● Uncommon, more frequent among 30-45 yrs old males
● Symptoms include intermittent jaundice, fatigue, weight loss, pruritus, abdominal
pain
● ERCP confirms the diagnosis; multiple dilatations & strictures (beading) of the
biliary tree. MRCP & ERCP shows focal dilation in some bile ducts (bright broad
areas) & strictures in others (thinning or absence).
● Liver biopsy is done to determine the degree of hepatic fibrosis
● Clinical course is highly variable, but cyclic remissions & exacerbations are typical
○ In those with UC, the course of each disease seems independent of each
other & colectomy has no effect on PSC
○ 10-15% will develop cholangiocarcinoma at any time & does not
necessarily correlate with the extent of sclerosing cholangitis; thus, follow-
up is necessary.
● No known cure; management is supportive
○ Corticosteroids, immunosuppresants, ursodeoxycholic acid, antibiotics
○ Endoscopic/percutaneous dilation or stents may be done to relieve biliary
strictures
○ Extrahepatic biliary excision & hepaticojejunosotmy has produced
reasonable results in those without cirrhosis or significant fibrosis
○ Liver transplantation is the only option for those with advanced liver
disease; however, 10-20% recur & may require re-transplantation
● Majority of bile duct strictures are related to operative injury, most commonly during
cholecystectomy. Other causes include chronic pancreatitis, CBD stones, acute
cholangitis, cholecystolithiasis, sclerosing cholangitis, cholangiohepatitis,
structures of biliary-enteric anastomosis.
● It may progress to complications, such as recurrent cholangitis, secondary biliary
cirrhosis, & portal hypertension.
● BS most commonly result in recurrent episodes of cholangitis, but may present
with isolated jaundice.
● Blood tests suggest cholestasis with increased bilirubin & ALP.
● US or CT scan findings include dilated bile ducts proximal to the stricture & the
level of the stenosis
● MRCP provides a more detailed anatomic information abt the location & degree of
dilatation.
● ERCP or PTC are useful if the diagnosis remains in question. Here, the arrow
points to a stricture of the common hepatic duct.
● Percutaneous or endoscopic dilatation or stent placement will provide good results
in more than half of the cases. This is a endoscopic photo of a stent in place.
● However, surgical resection & reconstruction (Roux-en-Y choledochojejunostomy
or hepaticojejunostomy) may be necessary for persistent or complex strictures.
Choledichoduodenostomy may be considered for distal strictures.
● Other benign diseases of the biliary tree include biliary dyskinesia & sphincter of
Oddi dysfunction, acalculous cholecystitis, & choledochal cysts.
● GB carcinoma is rare & is only the 6th most common GI malignancy.
● It is aggressive & usually not diagnosed until it has become advanced, with only
10-25% identified while they are localized.
● It is common in the elderly (peaking at 70 yrs old) & in females.
● The pathogenesis has not yet been fully defined, but is believed to be related to a
combination of chronic inflammation, infection, genetics, & environmental
exposures (such as tobacco & heavy metals).
● Cholelithiasis is most important risk factor (0.5% 20-year risk), especially those >3
cm, symptomatic, & cholesterol stones.
● GB polyps of >10 mm have a 25% risk, as well as solitary, sessile or rapidly
growing polyps especially if presenting with gallstones or above 50 yrs old.
Cholecystectomy or serial imaging are options.
● Porcelain gallbladder is characterized by dense circumferential calcifications that
are believed to be d/t gallstones. It carries a 10% risk of GB carcinoma.
Traditionally, it was an absolute indication for cholecystectomy, although more
recent studies suggest that observation with serial imaging may be acceptable.
● 80-90% of GB carcinoma are adenocarcinomas. 10% are papillary type & are
associated with the best outcomes because they are commonly diagnosed early
● GB carcinoma rapidly spread through lymphatics, venous drainage, or direct
parenchymal invasion.
● Lymphatic spread begins at the cystic duct node (of Calot) pericholedochal &
hilar nodes pancreatic, duodenal, periportal celiac, SMA nodes
● Venous drainage or direct invasion is towards the hepatic segments IVb & V.
● GB carcinoma presents like cholecystitis & cholelithiasis, leading to delays in
diagnosis & treatment. Jaundice, wt loss, anorexia, ascites, abd masses are less
common presenting signs.
● Laboratory findings are consistent with biliary obstruction.
● US is 70-100% sensitive & may show a thickened irregular gallbladder wall (>3
mm) with hypervascularity or a mass, tumor invasion of the liver,
lymphadenopathy, & dilated biliary tree.
● CT scan will show gallbladder mass, nodal spread, local invasion.
● MRCP is useful if there is question about local invasion & allows for complete
assessment of biliary, vascular, nodal, hepatic, & adjacent organ involvement.
● PTC or ERCP may be useful in jaundiced patents; to delineate the extent of biliary
tree involvement.
● CT- or US-guided tissue diagnosis isnot required prior to cholecystectomy if the
tumor appears resectable.
● Opened gallbladder contains a large, exophytic tumor that virtually fills the lumen.
(Robbins, 10th ed.)
● CCA is rare & also often advanced at the time of diagnosis.
● It is more frequent in 50-70 yrs olds & slightly in males than females.
● Most cases occur de novo with no identifiable risk factors. However, there is an
increased risk associated with choledochal cysts, ulcerative colitis, hepatolithiasis,
biliary-enteric anastomoses, hepatitis B and C, cirrhosis, biliary tract infections with
Clonorchis (liver flukes), chronic typhoid carriers, PSC (5-10% risk). Biliary stasis,
bile duct stones, & infection are common among these conditions.
● CCA may arise anywhere along the biliary tree. 50% are located at the hepatic
bifurcation (Klatskin tumors), 40% occur more distally, 10% occur intrahepatically.
● Klatskin (hilar) tumors are further classified using the Bismuth-Corlette
classification based on their anatomic location.
● Type I involves the CHD only
● Type II involves the bifurcation without secondary intrahepatic ducts
● Type IIIa & IIIb extend into the right & left secondary intrahepatic ducts,
respectively
● And type IV involves both the right & left intrahepatic ducts
● The most common initial presentation is painless jaundice. Aside from this, PE is
usually normal.
● Blood tests are consistent with cholestasis.
● CA 19-9 is a useful tumor marker, with 98% sensitivity at serum levels >129 U/mL.
CA 125 & CEA are also elevated, but are less specific (other GI & gynecologic
malignancies).
● US or CT scan are the initial imaging of choice, which can provide an outline of the
biliary anatomy, estimate level of obstruction, evaluate portal vein patency, &
screen for nearby LAD. Prehilar tumors are suggested by dilatation of the
intrahepatic biliary tree but a normal/collapsed GB & extrahepatic ducts distal to
the tumor, while distal tumors are suggested by dilatation of extra- & intrahepatic
bile ducts & gallbladder
○ The US is of a CHD CCA showing dilated ducts & tumor obstructing the
common hepatic duct (arrow). The walls of the bile ducts adjacent to the
obstruction are thickened by tumor infiltration (arrowheads).
● ERCP or PTC can provide a more detailed evaluation of the biliary anatomy. This
ERCP is of the same patient showing a CBD of normal size, as is the cystic duct
(arrow), but a dilated proximal biliary tree. The gallbladder is not visualized
because of tumor obstructing its neck (arrowheads).
● Current techniques for tissue diagnosis (FNAB, biliary brushings) have been
shown to have a low sensitivity (between 15-60%), & are thus not usually done.
● The 2 most commonly used staging systems for perihilar CCA include the
American Joint Committee on Cancer/Union for International Cancer
Control (AJCC/UICC) & the Memorial Sloan-Kettering Cancer Center (MSKCC)
staging systems. However, the MSKCC staging system failed to stratify resectable
from unresectable patients with sufficient accuracy. The AJCC/UICC is currently
the only staging system for distal CCA.
● Surgical excision is the only potentially curative treatment. If the tumor is potentially
resectable, diagnostic laparoscopy should be offered.
○ Distal BD tumors are often resectable but may require a Whipple
procedure. The 5-yr survival rate resectable distal CCA is 30-50%. For
unresectable distal BD tumors, Roux-en-Y hepaticojejunostomy,
cholecystectomy, & gastrojejunostomy are performed to prevent gastric
outlet obstruction.
○ Klatskin types I or II without vascular involvement are managed with local
tumor excision, portal LAD, cholecystectomy, CBDE, & bilateral Roux-en-Y
hepaticojejunostomies
○ Klatskin type III is additionally managed with right/left hepatic lobectomy &
possible caudate lobectomy. The 5-yr survival rate for resectable perihilar
carcinoma is 10-40%. This surgical resected specimen is of a type IIIb
Klatskin tumor.. Stump of the right hepatic duct is free from tumor
spreading.
○ Klatskin type IV are considered unresectable, & cholecystectomy & biliary
bypass are performed. Liver transplantation is their only curative option.
● Best outcomes for Klatskin tumors are seen with neoadjuvant chemoradiation
followed by liver transplant; however, only few patients qualify.
● If the tumor is unresectable at presentation, non-operative biliary decompression
can be performed. Endoscopic placement of expandable metal stents is often the
preferred approach. There is no proven role yet for adjuvant chemotherapy or
radiation, but palliative chemotherapy may be offered, typically with gemcitabine &
cisplatin, but with low response rates. Of those presenting with unresectable
disease, the median survival is 5-8 mos. The most common COD are secondary to
hepatic failure & cholangitis. Liver transplantation increases the 5-yr survival rate
up to 68%.