2. • Cystic dilatation of the common bile duct
(CBD) is known as choledochal cyst. It is a
fairly uncommon anomaly of the biliary tract.
• It was first described by Vater and Ezler in
1723.
• Douglas published the first complete clinical
description of the anomaly in a patient in
1853.
3. • Population prevalence estimates of
choledochal cysts range from approximately 1
case in 13,000 people to 1 case in 2 million
people.
• Choledochal cysts can occur in persons of any
age.
• 2/3 rd of the cysts are diagnosed before the
patient is aged 10 years.
4. PATHOPHYSIOLOGY
Congenital cysts may result from an unequal
proliferation of embryologic biliary epithelial
cells before bile duct cannulation is complete.
• Fetal viral ( Reo RNA )infection may also have
a role
• cyst formation may be the result of ductal
obstruction or distension during the prenatal
or neonatal period.
5. ACQUIRED
• The exact cause of choledochal cyst remains obscure.
Several theories have been postulated
1. Weakness of the wall of the bile duct
2.Obstruction of the distal CBD
3.Combination of obstruction and weakness
4.Reflux of pancreatic enzymes into the CBD secondary to
an anomaly of the pancreaticobiliary junction
7. • With APBJ, the pancreatic duct and biliary tree fuse to form
a common channel before passage through the duodenal wall;
• APBJ is seen in up to 90% of patients with choledochal cysts.
The fused duct forms a long common channel, which allows
pancreatic secretions to reflux into the biliary tree.
Because the pancreatic duct has higher secretory pressures than
the biliary tree, exocrine pancreatic secretions reflux up into the
bile duct and can inflame and damage the biliary tree resulting in
cystic degeneration.
8. • In 1977, Todani et al conducted an analysis of
ERCP and other cholangiograms and
confirmed this long common-channel
anomaly.
• All of these theories are applicable to
choledochal cyst type I, III, and IV anomalies,
but they cannot be used to explain type II and
V choledochal cysts in which the CBD is
normal
9. • Perhaps genetic factors play a role. Despite
this, the two most accepted theories are still
reflux of pancreatic enzymes into the CBD
secondary to an anomalous pancreaticobiliary
junction and obstruction of the distal CBD.
10. Type Configuration Biliary Tract Incidence Treatment
Extrahepatic Intra-
hepatic
I A Cystic
fusiform
Most or all >50 % to 75
%
Excision of
involved portion
of extrahepatic
tract + Roux-en-Y
Hepato-
jejunostomy
B Limited
C sacular Most or all
II Isolated
Diverticulum
Of CBD
5 % Excision with
closure of defect
over T-tube or
same as above
III Intraduodenal
Part of
CBD
5 % < 3 cm =
endoscopic
sphincterotomy
> 3cm = excision
via transduodenal
approach
CHOLEDOCHOCELE
TODANI(1977) CLASSIFICATION
11. Type Configuration Biliary Tract Incidence Treatment
Extrahepatic Intra-
hepatic
IV A
Multiple
Dilations
30 % Extrahepatic
Excision of
involved portion
+ Roux-en-Y
Hepato-
jejunostomy
Intrahepatic
Resection of
segment or lobe
Or
transplantation
B
V 1 %
VI Isolated Cyst of
Cystic Duct
Extremely
Rare
Cystic Duct
ligation near CBD
CAROLI DISEASE (1958)
NOT part of Todani Classification
TODANI(1977) CLASSIFICATION
Jacques Caroli
French gastroenterologist, 1902-1979
12.
13. PRESENTATION
•1. Classic triad for choledochal cysts is :
pain, jaundice, and abdominal mass.
•It is found in only a minority of children at the
time of presentation.
14. • Infants commonly presented with elevated
conjugated bilirubin (80%), failure to thrive or
an abdominal mass (30%)
15. • older than 2 years of age, abdominal pain is
the most common presenting symptom.
common presenting symptom.
• Intermittent jaundice , recurrent cholangitis
,and pancreatitis is also a common feature .
• Pancreatitis is more common with type 3 cyst .
16. Rarely, biliary cysts present with
• intraperitoneal rupture
• bleeding due to erosion into adjacent vessels
• portal hypertension
• secondary biliary cirrhosis due to prolonged
biliary obstruction and recurrent cholangitis.
• In addition, type III cysts can case gastric outlet
obstruction due to the obstruction of the
duodenal lumen or intussusception.
18. Transabdominal ultrasound
• First imaging modality used for the evaluation
• Not detect type III and type V cysts.
• sensitivity of 71 to 97 %
• Factors that may limit the usefulness of an
ultrasound include the patient's body habitus, the
presence of bowel gas, and limited visualization
due to overlying structures.
19. Computed tomography
• CT can detect all types of biliary cysts.
• Can 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.
20. CT cholangiography
has high sensitivities for visualizing the
• biliary tree (93%),
• biliary cysts (90%),
• intraductal stones (93%)
• However, its sensitivity is lower for imaging
the pancreatic duct (64 %)
22. Endoscopic ultrasound(EUS)
• EUS can demonstrate extrahepatic biliary cysts
and provide detailed images of the cyst wall
and pancreaticobiliary junction.
• unlike transabdominal U/S, it is not limited by
body habitus, bowel gas, or overlying
structures.
23. Intraductal ultrasound (IDUS)
• has been used for the diagnosis of early
malignant changes in a biliary cyst .
• This technique is likely to be more sensitive
than direct cholangiography for detecting
early malignancy in the cyst wall.
24. Hepatobiliary scintigraphy
• using radio-labeled dyes : technetium-99m-labeled hepatic
iminodiacetic acid (HIDA), which is selectively taken-up by
hepatocytes and excreted into the bile.
• HIDA scanning is useful for extrahepatic cysts, with a
sensitivity up to 100% for type I cysts. However, it is
inadequate at visualizing the intrahepatic bile ducts
• HIDA scanning may also be useful in cases of cyst rupture
26. Cholangiography
• Direct cholangiography (whether intraoperative,
percutaneous, or endoscopic) has a sensitivity of up to 100
percent for diagnosing biliary cysts and previously was a
commonly obtained test.
• can identify abnormal pancreatobiliary junction, and filling
defects due to stones or malignancy.
• Increase risk of cholangitis and pancreatitis. [ Patients
with cystic disease are greater risk for these complications ]
28. Magnetic resonance
cholangiopancreatography [MRCP]
• Does not have the risks of cholangitis and
pancreatitis as direct cholangiography
• Sensitivity 73 - 100 %.
• less sensitive than direct cholangiography for
excluding obstruction.
• The data are variable with regard to its ability
to diagnose an abnormal pancreatobiliary
junction. [46-75%]
30. TREATMENT
• If pt presents with pancreatitis /cholangitis
should be treated supportively before
definitive operative management.
• In choledochal cyst pb maljunction are high
risk for pancreatitis .chance of panceatitis
increase with ercp and ampulary stenting.
31. • The treatment of choice for choledochal cysts
is complete excision of the cyst with
construction of a biliary-enteric anastomosis
to restore continuity with the gastrointestinal
tract.
• partial resection of the cyst and internal
drainage procedures expose patients to
increased risks of cholangitis, pancreatitis,
and cholangiocarcinoma.
32. • Type 1 : KOCHER maneuver to explore distal
portion of cyst .
• Type 1(B) : Extend distally to entrance of the
cbd into pancrease . Goal is to excise
intrapancreatic portion of cyst without
injuring pancreatic duct or long common duct
.
33. • Type I
• cysts are treated by complete surgical excision, cholecystectomy,
• and Roux-en-Y hepaticojejunostomy. The proximal extent of
• resection should continue to the nondilated biliary tree and may
• require anastomosis to the left and right hepatic ducts. If there is
• substantial pericyst fibrosis, an intramural plane can be developed
• to excise the entire epithelium while leaving the fibrotic outer cyst
• wall in place. The distal duct is oversewn, with care taken not to
• injure the pancreatic duct.
34. • Kocher incision made
• Mobilisation of Transverse Colon from hepatic Flexure
• Retrograde dissection of GB from liver bed
Retrograde
Dissected
GB
Cystic Duct
Choledochal
Cyst
CBD
35. • Choledochal Cyst dissected away from portal vein and
hepatic artery ( Lilly Technique?)
• Cyst wall opened till common hepatic duct junction
36. • Hepatic ducts washed with normal saline
• Hepatic ducts patency confirmed with bougies
Left
Hepatic
Duct
Right
Hepatic
Duct
37. • Roux-en-Y (french: rōō'ěn-wī')
Hepatojejunostomy done
(retrocolic isoperistaltic functional side-to-side)
Cesar Roux
Swiss Surgeon (1857-1934),
(Performed 1st successful excision of
pheochromocytoma in 1926)
(End-to-side)
(End-to-side OR
Side-to-side)
38. • Type 2 : simple cyst excision along with
cholecystectomy .close the defect transversely
, which reduce the cbd stricture.
• Type 3: endoscopic sphincterotomy is
benificial .
39. • Type 3 : pt may benefit from endoscopy
sphincterotomy .
• Surgical resection is via transverse
duodenotomy in second or 3 rd part .
Duodenotomy allow both biliary and
pancreatic duct to be identify individually.
• After resection of cyst both pancreatic and
bile duct mucosa are sutured individually to
duodenal mucosa .
40. • Type 4 : 4a and 4b are managed in similarly to
type 1 cyst .
• Type 5 : if confined to single lobe resection of
involved parenchyma.
• In bilobar absence of cirrhosis or malignancy
roux –en- y hepaticojejunostomy with bilateral
trans transhepatic silastic stent may be
indicated to improve biliary drainage.
41. • Patient with carolis disease and liver failure
may warrant liver transplantation .
• Summary : patient with holedochal cyst
require long term surveillance for recurrent
cholangitis , intrahepatic stone , pancreatitis
,postoperative biliary strictures and
malignancy.
