By Dr. Dipesh K.K General surgeon

1. DR.DIPESH

2.  The anatomy of the extrahepatic biliary tree is
highly variable.
 A thorough knowledge of this variable anatomy is
important because failure to recognize the
frequent anatomic variations may result in
significant ductal injury.
 Anomalies of the extrahepatic biliary tree may
involve the hepatic ducts, common bile duct, or
cystic duct.

3.  In 57% to 68% of patients, the right anterior and
right posterior intrahepatic ducts join, and the
right hepatic duct unites with the left hepatic
duct to form the common hepatic duct.

4.  In 12% to 18% of patients, the
right anterior, right posterior, and
left hepatic ducts unite to form
the common hepatic duct.

5.  In 8% to 20% of patients, the right
posterior and left hepatic ducts
join to form the common hepatic
duct, and the right anterior duct
joins below the union.

6.  In 4% to 7% of patients, the right
posterior duct joins the common
hepatic duct below the union of
the right anterior and the left
hepatic ducts.

7.  In 1.5% to 3% of patients, the
cystic duct joins at the union
of all the ducts or with one of
the right hepatic ducts.

8.  Ectopic drainage of a right sectoral duct into the
left hepatic ductal system .
 Right posterior sectoral duct draining into the left
hepatic ductal system.
 Right anterior sectoral duct draining into the left
hepatic ductal system).

9.  Absence of the hepatic duct confluence.
 Absence of right hepatic duct and ectopic drainage
of the right posterior duct into the cystic
duct.

10. Common Bile Duct
 Several variations of common bile duct malposition
and duplication have been described:
(1) A single duct opening into the pylorus or antrum.
(2) A single duct opening into the gastric fundus.
(3) A single duct entering the duodenum
independently of the pancreatic duct

11. (4) two separate ducts entering the duodenum.
(5) A bifurcating duct, with one branch entering the
duodenum and the other branch entering the
stomach.

12. (6) A bifurcating duct with both branches entering
the duodenum and
(7) A septate common bile duct, with two openings of
the single duct into the duodenum.

13.  In 1976, Benson and Page described five ductal
anomalies of clinical significance to the surgeon
during performance of a cholecystectomy.

14. A. Long cystic duct with low fusion with common
hepatic duct.
B. Abnormally high fusion of cystic duct with common
hepatic duct (trifurcation).
C.Accessory hepatic duct.

15. D. Cystic duct entering right hepatic duct.
E. Cholecystohepatic duct

16.  Variations in the arterial supply of the extrahepatic
Biliary tree are more common than variations in the
ductal anatomy.
 Anatomic variations of the hepatic and cystic
arteries are present in approximately 50% of
individuals.

17. Based on their anatomic dissections, Benson and Page
described following surgically important variations
In the arterial anatomy.
A. Caterpillar turn or Moynihan’s Hump:-

18.  When the right hepatic artery replaces the cystic
artery within the Calot’s triangle, and it is tortuous
and projects forwards to the right of the
(CHD),something like the hump of caterpillar back
during progression, and form U-shape loop with a
short cystic artery arising from it .

19. B. Right hepatic artery anterior to common hepatic
(or common bile) duct.
C.Cystic artery anterior to common hepatic (or
common bile) duct.

20. D. Accessory cystic artery.

21.  Choledochal cyst is a congenital cystic
dilation of any portion of intrahepatic and/or
extrahepatic biliary tree most commonly
involving the main portion of CBD.

22.  The estimated incidence of choledochal cysts in
Western countries varies between 1 in 100,000 and
1 in 150,000 individuals.
 The rate of incidence is higher in Asia and occurs
more frequently in women (M: F- 4:1).
 60% of patients present before age 10, although
choledochal cysts can present from birth to old age.

23.  This anomaly is associated with increased
incidence of GB anomalies, biliary anomalies
( stenosis /atresia), and congenital hepatic
fibrosis

24.  Complications of choledochal cysts in adults include
- rupture with bile peritonitis
- secondary infection (cholangitis)
- biliary cirrhosis and portal hypertension
- calculus formation

25. - portal vein thrombosis
- liver abscess
- Hemorrhage and
- malignant transformation into
cholangiocarcinoma

26. TYPES OF CHOLEDOCHAL CYSTS
 The most widely accepted classification was
proposed initially in 1959 .
 It was expanded in 1977 to include intrahepatic
cysts and further refined in 2003 to incorporate the
presence of an abnormal pancreaticobiliary
junction (APBJ)

27. Type I cysts (50 to 85 percent of cysts) –
Type I cysts are characterized by cystic or
fusiform dilation of the common bile duct .

28. subcategorized as :
•Type IA – Cystic dilation of the common bile duct,
as well as part or all of the common hepatic duct
and extrahepatic portions of the left and right
hepatic ducts.
- Associated with an APBJ.

29. •Type IB – Focal, segmental dilation of an
extrahepatic bile duct (often the distal
common bile duct).
- Not associated with an APBJ.

30. •Type IC – Smooth, fusiform (as opposed to cystic)
dilation of all the extrahepatic bile ducts.
 Typically, the dilation extends from the
pancreatobiliary junction to the extrahepatic
portions of the left and right hepatic ducts.
 Associated with an APBJ.

31. •Type ID – Cystic dilation of the common duct
and cystic duct.

32.  Type II cysts (2 percent of cysts) – Type II cysts are true
diverticula of the extrahepatic bile ducts. They may
arise from any portion of the extrahepatic bile duct

33.  Type III cysts (1 to 5 percent of cysts) – Type III cysts are
cystic dilations limited to the intraduodenal portion of
the distal common bile duct and are also known as
choledochoceles. Subdivided into:-

34.  Type IIIA – The bile duct and pancreatic duct
enter the cyst, which then drains into the
duodenum at a separate orifice.
 Type IIIB – A diverticulum of the
intraduodenal common bile duct or intra-
ampullary common ductal channel.

35.  Type IV cysts (15 to 35 percent of cysts) – Type IV
cysts are defined by the presence of multiple cysts
and are subdivided based on their intrahepatic bile
duct involvement:
 Type IVA – Both intrahepatic and extrahepatic cystic
dilations.Associated with a distinct change in duct
caliber and/or a stricture at the hilum, that
differentiate it from a type IC cyst .

36.  Type IVB – Multiple extrahepatic cysts but no
intrahepatic cysts

37.  Type V cysts (20 percent of cysts) – Type V cysts are
characterized by one or more cystic dilations of the
intrahepatic ducts, without extrahepatic duct disease.also
known as Caroli disease.

38.  Type VI cysts (rare) – Isolated cystic
dilatations of the cystic duct.

39.  The classic presentation includes the triad of
1.Abdominal pain
2. Jaundice, and
3.A palpable mass
 may also present with nausea, vomiting, fever,
pruritus, and weight loss.

40.  A biliary cyst should be considered when a dilated
portion of the bile duct or ampulla is identified on
imaging.
 A high level of suspicion is required for diagnosis,
particularly for type I cysts, which may go
undiagnosed unless considered in the differential
diagnosis of patients found to have ductal dilation.

41.  Cysts are often first suspected based on the
findings from transabdominal ultrasonography in a
patient being evaluated for abdominal pain,
jaundice, or an abdominal mass.
Factors that may limit the usefulness of an
ultrasound include:-
 the presence of bowel gas, and
 limited visualization due to overlying structures.
 Ultrasound frequently misses type III cysts

42.  If a biliary cyst is suspected based on an
ultrasound, cross-sectional imaging with CT
or MRI with MRCP is typically the next step in
diagnosis

43. Advantage of CT scan:-
 CT can detect all types of biliary cysts.
 It can demonstrate continuity of the cyst with
the biliary tree.
 The relationship of the cyst to surrounding
structures and
 Evaluate for the presence of malignancy.
 It is also useful for determining the extent of
intrahepatic disease in patients with type IVA or
V cysts

44. Limitation of CT scan:-
 Its sensitivity is lower for imaging the
pancreatic duct (64 percent), and
 Its sensitivity for diagnosis of abnormal
pancreatobiliary junction is poor.

45. Advantage of MRI/MRCP
 it does not use ionizing radiation
 may demonstrate an APBJ, and
 can assess for an obstructing lesion within
the biliary tree or pancreas.

46. FIG. show (MRCP) images
of the distal common bile
duct with a choledochocele
bulging into the duodenum.
The lower images show the
corresponding endoscopic
views obtained with
forward- (left lower panel)
and side-viewing (right
lower panel) endoscopes.

47. Limitation of MRCP :-
 MRCP is less sensitive than ERCP for
excluding obstruction.

48. Following cross-sectional imaging, some patients
will require additional testing:
 Types I and IVA cysts: MRCP is an effective imaging
technique for diagnosis,
- If concern remains for an obstruction following
MRCP an ERCP or EUS should be performed.

49. 2. FIG.CT scan of TYPE 1 Choledochal cyst obtained after injection of
contrast material through the biliary catheter shows a choledochal cyst
(straight arrow) containing contrast material and air adjacent to the
duodenum (curved arrow)

50. Fig. —A.
MR cholangiopancreatography
shows large type 1 bile duct cyst of
extrahepatic bile duct (arrows).
Right and left hepatic ducts are
slightly distended(open arrows).
Gallbladder (GB) and duodenum (D)
are noted.
Fig._B. Percutaneous transhepatic
cholangiogram of same patient
shows large type 1 bile duct cyst of
extrahepatic bile duct containing
contrast material (arrows). Slightly
distended left and right hepatic
ducts are noted (open arrows).
Percutaneous needle (Perc),
gallbladder (GB), and duodenum
(D) are noted.

52. Advantage of ERCP
 provide direct visualization of the ampulla as well
as the peri-ampullary bile duct and pancreatic
duct and
 can evaluate for evidence of obstruction at the
proximal end of the cyst (due to malignancy).
 Is the gold standard for preoperative diagnosis of
an abnormal pancreatobiliary junction.

53. Disadvantage of ERCP
 ERCP may miss malignant strictures in the
proximal portion of a type I cyst, unless
attempts are made to cannulate and fill the
intrahepatic bile ducts and biliary confluence.
 Complications of ERCP include cholangitis and
pancreatitis

54. 2. Types II and IVB cysts:
If it is unclear whether the cyst communicates
with the biliary tree after cross-sectional
imaging, confirmation may be obtained by
hepatobiliary scintigraphy, ERCP, or EUS.
 EUS may be particularly helpful for
distinguishing a pancreatic head cyst from a Type
II biliary cyst .

55. Fig.type II biliary cyst.
(a) ERCP image of a 46 yr
women shows a type II
BILIARY CYST (Diverticulum)
Arising from the medial
aspect of the
suprapancreatic common
bile duct (solid arrow).there
is a 15-mm stone within the
diverticulum (open arrow).
(b) CT scan shows the
diverticulum (arrow)
between the pancreas and
duodenum.

56. 3.Type III cysts:
ERCP can confirm the presence of a type III cyst
and permits endoscopic therapy and cyst mucosal
biopsy during the same examination.

57. Fig. —A .MRCP image reveals oval-
shaped high-signal-intensity contour
(arrows) of distal common bile duct
(CBD) protruding into duodenum (D),
which represents choledochocele.
Pancreatic duct (P) is noted.
FIG.B. Axial T2-weighted image shows
fluid-filled structure (arrows)
protruding into duodenum (D).
Pancreas (P) is noted.

58. Fig. — ERCP image shows contrast-filled and oval-shaped structure (arrows)
of distal common bile duct (CBD) protruding into duodenum (D), which
represents choledochocele .

59. Fig. A Sonogram shows
aneurysmal dilation of
extrahepatic bile duct
(arrows). Dotted line
between arrows represents
measurement line of
sonogram.
FIG.
cholangiopancreatography
shows marked dilation of
extrahepatic bile duct (solid
arrows) and left main
intrahepatic duct (open
arrow).

60. 4.Type V cysts:
Typically, the diagnosis is established when
imaging studies demonstrate
 Intrahepatic bile duct ectasia and
 Irregular, cystic dilation of the large proximal
intrahepatic bile ducts with a normal
common bile duct.

61. Fig. —70-year-old woman
with jaundice.MR
cholangiopancreatography
shows multiple cystic
dilations of intrahepatic bile
ducts (arrows) consistent
with Caroli’s disease.
Common bile
duct (CBD), duodenum (D),
and pancreatic duct (P) are
noted.
B. Coronal T2-weighted
image shows multiple fluid-
containing foci (arrowheads)
in papillae of kidneys,
indicating renal tubular
ectasia (medullary sponge
kidney). Saccular
dilations of intrahepatic bile
ducts (arrows) are noted.

62. FIG.Magnetic resonance cholangiography in an infant with Caroli's disease. There is biliary ectasia throughout the
liver and saccular dilatation of bile ducts in the left lobe (arrow). The kidneys show typical changes of autosomal
recessive polycystic kidney disease with massive enlargement due to fusiform dilatation of the collecting ducts.

63.  The approach to management of patients with
biliary cysts depends on the cyst type.
 Patients with type I, II, or IV cysts usually undergo
surgical resection of the cysts due to the significant
risk of malignancy.

64. 1. Type I and IV cysts :-
-Should be completely resected with creation of a
Roux-en-Y hepatojejunostomy.
-Serial sections from the cyst wall should be
examined by the pathologist to look for any
malignant changes.
2. Type II cysts :-
-Can be treated with simple cyst excision.
-Cysts with complicated presentations (including
jaundice or malignancy in the cyst) may require
more extensive resection.

65. 3. Type III cysts (choledochoceles) :-
Require treatment if they are symptomatic and
may be managed with sphincterotomy or
endoscopic resection in the absence of neoplasia.

66. 4.TYPE V CYSTS :-
 Is largely supportive and is aimed at dealing
with problems such as recurrent cholangitis
and sepsis.
 Type V cysts can be difficult to manage, and
some patients with type V cysts eventually
require liver transplantation.

67. An anomalous pancreaticobiliary junction also
known as
 Anomalous junction of the pancreatic and biliary
ducts [AJPBD] or
 Anomalous pancreaticobiliary union [APBU]
 can be demonstrated in virtually all patients with
type 1 biliary cysts

68.  Anomalous junction of the pancreaticobiliary
ductal system has been reported in association
with
-cholangiocarcinoma,
-gallbladder carcinoma
-Choledocholithiasis, and
-Chronic pancreatitis.
 In this entity, pancreatic secretions can reflux into
the common bile duct and can result in the
development of a bile duct cyst (type l).

69.  It is characterized by a junction of the bile duct
and pancreatic duct outside the duodenal wall
with a long common duct channel.

70.  In children, the maximum length of the common
channel in healthy neonates and infants younger
than one year is 3 mm.
 It increases with age to a maximum of 5 mm in
children and adolescents between 13 and 15
years of age .
 In adults, a common channel longer than 8 mm is
considered abnormal.

71.  Three types of anomalous ductal union have
been described.
 All three types are characterized by a long
common channel, and this is dilated in the
first two types (Kimura classification)

72.  Type B-P – Common bile
duct joining the main
pancreatic duct.
 Type P-B – Pancreatic
duct joining the common
bile duct; this type is
more likely to be
associated with
recurrent pancreatitis
than the B-P type.
 Long Y type – A long
common channel,
without common bile
duct dilatation

73. FIG.ERCP
showing a
choledochal
cyst, type IV-A,
in a 12-year-old
female. Note
an
anomalous panc
reaticobiliary
union, type "BP"
(arrow).

74. Fig. —49-year-old woman with pancreatitis.
A. MR cholangiopancreatography shows 2.4-cm-long common channel (double arrows) distal to union
of distal common bile duct (CBD) and pancreatic duct (P). Major papilla (open arrow) and duodenum
(D) are noted. Mid common bile duct is focally dilated, which is consistent with type 1 bile duct cyst
(Cyst).
B. Schematic of A. The double arrows represent long common channel from orifice of papilla (open
arrow) to union of distal common bile duct (CBD) and pancreatic duct (P). Mid common bile duct is
focally dilated, which is consistent with type 1 bile duct cyst (Cyst). Duodenum is noted (D).

75.  In situs inversus, the bile ducts are reversed
right to left—as are the other abdominal
organs (“mirror image”) .
 In situs ambiguous with polysplenia, the
stomach and multiple small spleens usually
lie in the right upper quadrant of the
abdomen, and the gallbladder and the
liver lie close to midline .

76. A. MR
cholangiopancreatography
shows situs inversus with
gallbladder (GB), common
bile duct (CBD), and second
portion of duodenum (D) in
left upper quadrant, and
pancreatic duct (P)
coursing toward right. High
signal intensity focus in
right upper quadrant (S)
represents fluid in stomach.
 B. Axial CT shows
gallbladder (GB) and liver
(L) located in left upper
abdomen, and stomach (S)
and spleen (Sp) in right
upper abdomen. Splenic
vein (v) is noted.

77.  Fig. 13—42-year-old
woman withpolysplenia
syndrome. Axial CT scan
shows two spleens (Sp)
to right of midline.
Gallbladder (GB) is near
midline and stomach (S)
is on right. Pancreas is
foreshortened (P).

78.  Normally, the right and left hepatic ducts converge
at the porta hepatis to form the common hepatic
duct.
 The right hepatic duct has two major branches, a
ventrocranial or anterior branch and a dorsocaudal
or posterior branch.
 The left hepatic duct also has two major branches,
medial and lateral.

79.  Congenital abnormalities or variations of the
biliary ducts are common, with a reported
prevalence of approximately 18.5% (3).
 Most common biiary ductal anomaly is an
anomalous right hepatic duct emptying into
the common hepatic duct or cystic duct.
 Seen in 4%-5% of cholangiograms

80.  A severed anomalous hepatic duct can result
in biliary fistula formation and bile leakage.
 Anomalous ducts arising from the left lobe
are rare and are usually of no clinical
significance.

81. Figure 18. Right hepatic duct anomalies.
(a) ERCP image of a 59-year-old man reveals an aberrant right hepatic duct
inserting at the junctionof the cystic duct and common hepatic duct
(arrow).
(b) Intraoperative cholangiogram of the same patient reveals an accessory
right hepatic duct inserting atthe junction of the cystic duct and
common hepatic duct (arrow).

82. FIG. Intraoperative cholangiogram of a 22-year-old woman reveals an
aberrant right hepatic duct inserting into the cystic duct. Note the
proximity of the aberrant right hepatic duct to the severed cystic duct
(arrow).

83.  It is important to recognize an anomalous right
hepatic duct at cholangiography because of the risk
of injury (inadvertent ligation or severing) during
cholecystectomy.

84. FIG.(21b) percutaneous transhepatic cholangiogram reveals complete
obstruction of the right hepatic ductal system. Note the surgical clips at the
point of obstruction (arrow). In this case, the cystic duct entered the right
hepatic duct, which was inadvertently ligated during cholecystectomy.

85.  Biliary atresia (BA) is a progressive, idiopathic,
fibro-obliterative disease of the extrahepatic
biliary tree that presents with biliary obstruction
exclusively in the neonatal period.
 The overall incidence is low (approximately 1 in
10,000 to 20,000 live births).
 BA is the most common cause of neonatal jaundice
for which surgery is indicated and the most
common indication for liver transplantation in
children.

86.  The disease is classified according to the
level of most proximal biliary obstruction.
1) Biliary atresia type 1 (about 5% of cases):-
Has luminal patency down to the common
bile duct and proximal cystic biliary duct.

87. 2)Biliary atresia type 2 (about 2%):-
Has patency to the level of the common
hepatic duct,

88. 3)Biliary atresia type 3 (>90%):-
Where the most proximal part of the
extrahepatic biliary tract within the porta
hepatis is entirely solid.

89.  BA Result from a number of different etiologies.
Three groups are defined clinically:
1. Biliary atresia splenic malformation syndrome
a. First-trimester onset
b. Other anomalies, including polysplenia, asplenia,
situs inversus, preduodenal portal vein, and others
2. Cystic biliary atresia
a. First- or second-trimester onset
b. May be detected on antenatal ultrasound

90. 3. Isolated biliary atresia
a. Onset unclear (no clinical evidence) but
possibly perinatal
b. May have underlying viral etiology
i. Direct, such as cytomegalovirus
ii. Indirect, such as an alloimmune attack
following a viral “trigger”

92. 1.Jaundice
2.Pale stool
3.Dark urine
4.Coagulopathy
5.Failure to thrive
6.Hepatosplenomegaly and ascites-
(Late (>3 months of age) signs suggestive of cirrhosis)

93.  Laboratory studies

94.  Ultrasound
1.Echogenic fibrous tissue anterior to the
portal vein: triangular cord sign
2.larger hepatic arterial caliber
3.gallbladder ghost
-atretic gallbladder, length less than 19 mm
-irregular or lobular contour
-lack of smooth/complete echogenic mucosal lining with an indistinct wall

95. Hepatobiliary scintigraphy
 if scintigraphy demonstrates definite excretion of
the tracer from the liver to the small bowel,
patency is established and BA is very unlikely.
 In BA excretion of tracer is unlikely to occur .

96. Liver biopsy
 liver biopsy is perform in suspected BA for two
reasons.
 One purpose is to identify histologic changes
consistent with obstruction that warrant surgical
exploration.
 The other is to differentiate BA from other
causes of intrahepatic cholestasis, which would
not need surgical exploration.

97. fig.Pathologic changes in biliary atresia: a
marked degree of fibrosis, bile duct
proliferation, and portal inflammation.

98. Cholangiogram
 If the above steps in the evaluation support the
diagnosis of BA, the infant should be taken to
the operating room.
 The first step is an intraoperative
cholangiogram, which is the gold standard in the
diagnosis of BA.
 Fig. Intraoperative cholangiogram
demonstrates filling of the cyst and
mildly dilated intrahepatic ducts
but no communication with the
duodenum.

100. Surgery
 Portoenterostomy (first described by the
Japanese surgeon Morio Kasai in the 1950s)
and liver transplantation remain the
cornerstones of treatment of children with
biliary atresia.

101. This operation is undertaken in the attempt
to restore bile flow from the liver to the
proximal small bowel.
 For this procedure, a Roux-en-Y loop of
bowel is created by the surgeon and directly
anastomosed to the hilum of the liver,
following excision of the biliary remnant and
portal fibrous plate.

102. 1. The liver should be fully mobilized by
dividing the falciform,coronary, and
triangular ligaments such that the organ can
then be everted outside of the abdominal
cavity

103. 2.Mobilization of gallbladder and division of
distal common bile duct.

104.  3. Elevation of biliary remnants and separation
from vascular structures at level of porta hepatis
(portal vein confluence, right hepatic
arterties,and, to a lesser extent, left hepatic
arteries)

105.  4. Transection of portal plate from umbilical
“point” to around the division of right-sided
(anterior and posterior) portal pedicles.

106. 5. Portoenterostomy with 40- to 45-cm
retrocolic Roux loop.