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2 Biliary System
Cholangiocarcinoma
Prognosis Prognosis for cholangiocarcinoma is poor in general with several factors inuencing outcomes:
Lymph node involvement: Five-year survival varies signicantly by node involvement (38% with
node negative vs 10% in node positive disease). Further, increase in number of involved lymph nodes
portends worse prognosis (12% vs 28% 5-year survival in single vs multiple node metastasis)
Surgical margins: Five-year survival is signicantly better with negative margins (19–47%) vs.
involved margins (0–12%)
Vascular and perineural invasion: For intrahepatic cholangiocarcinoma, hepatic venous involvement
and lymph node metastases predict worse prognosis. The effect of lymphovascular and perineural
invasion is not entirely understood
Biomarkers: Elevation in CA 19–9 is associated with worse outcomes for distal and intrahepatic
cholangiocarcinoma
Follow-up CT scan or MRCP every 6months for 2years then annually for 5years
Research
Reference Findings
67
Valle J, Wasan H, Palmer DH, Cunningham D,
Anthoney A, Maraveyas A, Madhusudan S,
Iveson T, Hughes S, Pereira SP, Roughton M,
Bridgewater J; ABC-02 Trial Investigators.
Cisplatin plus gemcitabine versus gemcitabine
for biliary tract cancer. N Engl J Med.
2010;362(14):1273–81
The Advanced Biliary Cancer-02 (ABC-02) trial:
This systemic review compared combination gemcitabine/
cisplatin versus gemcitabine monotherapy in advanced biliary
cancers, including gallbladder cancer
As compared with gemcitabine alone, cisplatin plus
gemcitabine was associated with a signicant survival
advantage without the addition of substantial toxicity
Choledochal Cyst
Choledochal cyst
Characteristics Choledochal cysts are associated with the anomalous pancreaticobiliary junction (APBJ) in
30% of the time: Long common biliary and pancreatic channel➔pancreatic enzymes reux
into the bile duct because of the long common channel causing cystic outpouching
Choledochal cysts are associated with malignancy throughout the biliary tree
Choledochal cysts are more common in people of Asian descent and are more common in
women than in men
Presentation Right upper quadrant pain, pruritus, nausea, palpable mass, and jaundice—Most are
asymptomatic
68
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Choledochal cyst
Ultrasound, CT scan, and ERCP➔not useful in the diagnosis because lling of the dilated
biliary tree is difcult to achieve to dene the abnormal anatomy
Diagnosis
MRCP➔Diagnostic modality of choice for diagnosis of choledochocal cyst because it
creates a complete cholangiogram
Management Surgical resection is recommended given the risk of malignancy
A. H. Sohail et al.
Follow-up
Even after resection of the choledochal cyst➔patients are still at increased risk of developing
malignancy in the remainder of the biliary tree
Classication of choledochal cysts and their management
Malignancy
Type
Type I
(80–
90%)
Type II
(0–2%)
Type III Minimal Also known as a
risk Characteristics Management Figures
Most commonly
associated with
malignancy
Minimal Diverticulum off the
Fusiform dilation of the
extrahepatic bile ducts
Intrahepatic ducts are
not involved
common bile duct
choledochocele
Cystic dilation of the
common bile duct
within the wall of the
duodenum
Extrahepatic bile duct
resection followed by
Roux-en-Y
hepaticojejunostomy or
hepaticoduodenostomy
Extrahepatic bile duct
resection and
reconstruction
Endoscopic
sphincterotomy versus
transduodenal excision
and sphincteroplasty
Type IVa Minimal One extrahepatic cyst
with intrahepatic cysts
Extrahepatic bile duct
resection followed by
Roux-en-Y
hepaticojejunostomy or
hepaticoduodenostomy
Hepatic resection if
cysts are limited to one
lobe
2 Biliary System
Classication of choledochal cysts and their management
Malignancy
Type
risk Characteristics Management Figures
69
Type
IVb
Type V Most commonly
Minimal Multiple small
extrahepatic cyst
Only intrahepatic cysts
associated with
malignancy
Also known as Caroli
disease
Primary Sclerosing Cholangitis
Primary sclerosing cholangitis (PSC)
Extrahepatic bile duct
resection followed by
Roux-en-Y
hepaticojejunostomy or
hepaticoduodenostomy
Liver transplantation
Characteristics Progressive disorder
Inammation, brosis, and stricturing of medium and large ducts in the intrahepatic and
extrahepatic biliary tree➔cholestasis, brosis, and hepatic failure
More common in men than women
Association with
inammatory
bowel disease
Presentation Asymptomatic with abnormal lab test
PSC is associated with inammatory bowel disease➔majority of patients have ulcerative
colitis (prevalence of ulcerative colitis as high as 90%)
Fatigue, jaundice, and pruritus
70
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Primary sclerosing cholangitis (PSC)
Elevated ALP, elevated bilirubin
A. H. Sohail et al.
Diagnostic
modality
Diagnosis
Management Case Management
Ultrasound Abnormal bile ducts with
MRCP Multifocal, short strictures
ERCP
Asymptomatic
patient with PSC
with elevated LFTs
Characteristics
wall thickening and focal
dilations
(string of beads)
➔test of choice
Ursodeoxycholic acid➔Improvement in lab values but no survival
benet or change in rates of liver transplantation
Glucocorticoids➔No long-term benet
(A) Coronal HASTE abdomen MR image
without IV contrast and (B) coronal
abdomen CT image with IV contrast
demonstrate dilated intrahepatic biliary
ducts (arrows) up to 9mm
Antibiotics➔Improvement in lab values but no effect on disease
progression
Patient with PSC
and a dominant
stricture
Patient with
isolated focal
stricture at
bifurcation
Patient with PSC
and cirrhosis
Patient with PSC
with recurrence
after liver
transplantation
Life expectancy 15–20years after diagnosis without liver transplantation
Screening PSC patients need screening for gallbladder carcinoma, cholangiocarcinoma, colon cancer, and
hepatocellular carcinoma
Endoscopic therapy (dilation and stenting)
Resection and reconstruction
Liver transplantation if MELD ≥15
1/3 of those with recurrent disease require re-transplantation
2 Biliary System
Research
Reference Findings
71
Farges O, Malassagne B, Sebagh M,
Bismuth H.Primary sclerosing
cholangitis: liver transplantation or
biliary surgery. Surgery 1995;117:146
Hildebrand T, Pannicke N, Dechene
A, etal. Biliary strictures and
recurrence after liver transplantation
for primary sclerosing cholangitis: a
retrospective multicenter analysis.
Liver Transpl 2016;22:42
This retrospective study found that liver transplantation to be superior to
biliary surgical procedures for patients with PSC
This multicenter study analyzed a large patient cohort with a long
follow-up in order to evaluate the incidence of biliary strictures and
recurrent PSC after liver transplantation. Biliary stricture was diagnosed
in 36% and recurrent PSC was diagnosed in 20% of patients after liver
transplantation
Anatomy
Anterior view
Cantlie’s line
Posterior view
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Surface anatomy
Liver
3
AmirH.Sohail, JenniferWhittington,
andChristopherWolfgang
Anterior surface of the liver: Made up of segments 5, 8,
4a/b, 2, and 3
Right
posterior
section
Right lobe
Right
anterior
section
VIII
Left
medial
section
IVa
Left lobe
lateral
section
Left
II
VII
IVb
V
VI
Cantlie’s line
A. H. Sohail (*)
General Surgery Residency, NYU Long Island
School of Medicine, NYU Langone—Long Island
Hospital, Mineola, NY, USA
e-mail: amir.sohail@nyulangone.org
J. Whittington
Department of Surgery, NYU Long Island School of
Medicine, NYU Langone—Long Island Hospital,
Mineola, NY, USA
e-mail: Jennifer.whittington@nyulangone.org
III
Umbilical
fissure
Posterior aspect of the liver is composed of segments 7,
6, and 1
VIII
Right lobe
Right
anterior
section
VII
Right
posterior
section
Left
lateral
section
II
Left lobe
Left
medial
section
I
III
IV
Umbilical fissure
V
C. Wolfgang
Department of Surgery, NYU Grossman School of
Medicine, New York, NY, USA
Division of Hepatobiliary Surgery, NYU Langone
Hospital, New York, NY, USA
e-mail: Christopher.Wolfgang@nyulangone.org
VI
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2023
H. Ajouz et al. (eds.), The ABSITE Blueprints, https://doi.org/10.1007/978-3-031-32643-1_3
73
74
Middle hepatic vein
artery
General anatomy
A. H. Sohail et al.
Liver receives 75% of its blood supply via the portal
venous system and 25% via the hepatic artery
All liver segments, except segment I, are drained by
Right hepatic vein
VII
VIII
Left hepatic vein
II
the three hepatic veins (right, middle, left)➔which
drain into the IVC
IV
I
III
Segment I (caudate lobe) drains directly to
retrohepatic vena cava through the short hepatic veins
Biliary system typically follows branches of the portal
VI
vein and hepatic artery
V
Hepatic
duct
Hepatic
Portal
vein
Inferior
vena cava
Sections
Brisbane 2000 system divides the liver into two hemilivers, four sections (anterior, posterior, medial, and lateral)
and nine segments (I, II, III, IVa, IVb, V, VI, VII, VIII)
Couinaud segmental scheme divides the liver into eight segments
Middle hepatic vein (along Cantlie line) separates the right and the left hepatic lobes
Right hepatic vein separates the right anterior from the right posterior section
Umbilical ssure separates the left medial section from the left lateral section
Hepatic vein anatomy
Three hepatic veins drain the liver directly into IVC
Middle and left hepatic veins often form a common trunk before entering the IVC
Right hepatic vein: Provides drainage for all right segments and is the sole venous drainage for the right posterior
section (segments VI and VII)
Middle hepatic vein: The right and the middle hepatic veins drain the right anterior section (segments V and VIII).
Segment IV is primarily drained by the middle hepatic vein
Left hepatic vein: Drains segments II and III
c
Artery
3 Liver
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Hepatic arterial anatomy
Provides approximately 25% of hepatic blood
ow
Right hepatic artery passes posterior to the
common hepatic duct and gives off the cystic
artery at the triangle of Calot
75
Proper hepatic artery splits into right and left
hepatic arteries at hilum
40% aberrant anatomy
Most common aberrant anatomy: Right hepatic
artery arising from SMA; left hepatic artery
from left gastric artery
Accessory or replaced right hepatic artery
passes through the portacaval space and ascends
to the right lobe along the posterolateral aspect
of the common bile duct
A pulsatile structure on the most lateral aspect
of the porta during a Pringle maneuver identies
this anomaly
Hepatic
Artery
Proper
Right
Gastric
Artery
Gastroduoenal
Artery
Right hepatic
Replaced
Artery
Common Hepatic
Splenic
artery
Left
gastric
artery
Celia
Tr unk
76
VI
Ligamentum
VI
II
Portal vein anatomy
A. H. Sohail et al.
Provides 75% of hepatic blood inow with
poorly oxygenated but well-nourished blood
Portal vein divides into left and right portal
veins
Run in a horizontal plane dividing liver section
in superior and inferior segments
Left branch continues into the umbilical ssure
and provides inow to segments II, III, and IV
Right branch forms the anterior and posterior
sectoral branches supplying the right segments
Normal portal venous pressure: 5–10mmHg
Hepatic venous pressure gradient
(HVPG)=portal venous pressure—hepatic
venous pressure or RA pressure (normal:
1–5mmHg)
Bile ducts
Branches into the left and right main hepatic
ducts
Anterior and posterior on right
Segments 2, 3 and 4 on left
VII
VIII
V
V
Right Posterior
Right Portal
VIII
Right
Anterior
Right Anterior
Duct
IV
Umbilical
portion LPV
Left Portal
Main Portal
I
Ligamentum
Venosum
Te res
Transverse
portion LPV
I
III
II
III
Anatomic variations seen in up to 30% of
patients
Intrahepatic bile ducts course alongside the
portal vein and hepatic artery branches forming
the portal pedicles
I
VI
Right Posterior
Duct
Right Hepatic
Duct
IV
Left Hepatic
Duct
Bile ducts are supplied with oxygenated blood
from the hepatic arterial system
3 Liver
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Anatomy of portal triad
Porta hepatis is the area under the liver by
segment 4b/5 where the portal triad enters the
liver
77
Conguration in portal triad: Portal vein
posteriorly, CBD anterolateral, hepatic artery
anteromedial
Transverse scanning of portal triad➔Mickey
mouse view
Liver Disease
Budd-Chiari syndrome
Denition Results from obstruction of hepatic veins
Mechanical factors and hypercoagulable states
Hematologic abnormalities (including myeloproliferative disorders and polycythemia vera)➔most
Causes
frequent causes of Budd-Chiari syndrome in North America
Oral contraceptives, collagen vascular diseases, and disorders of the coagulation cascade (protein C
or protein S deciency), venous membranes, tumors, trauma➔rare causes of Budd-Chiari
syndrome
Common Hepatic Duct
Right
Hepatic Artery
Left
Hepatic Artery
Portal
Vein
Proper
Hepatic Artery
Presentation ranges from insidious to fulminant (depending on the speed and extent of the
Presentation
Diagnosis
obstructive process)
Most common manifestation: Abdominal distention with ascites
Duplex ultrasonography and angiography of the inferior vena cava and hepatic veins
Hematologic evaluation, coagulation studies, and liver biopsy➔to clarify the contributing factors
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