5. Classfication
First – Alonso-Lez 1959
Type 1 – fusiform/saccular dilatation of CHD/CBD
Type 2 – supraduodenal diverticulam of CBD/CHD
Type 3 – intraduodenal diverticulam of distal CHD /
Choledococele
(not included intrahepatic cysts)
Caroli – 1958 described multiple intrahepatic cysts
9. Defect in maturation with ductal plate
formation
Primarily to Caroli disease
AR>AD
Ductal plate – development of intrahepatic progenitor cells
that are in contact with mesenchyme of portal vein and
then remodelled into mature duct
Defective remodelling – inflammation and ulceration of
biliary epithelium of large bile ducts – segmentally dilated –
cysts
10. Bile duct obstruction / distension in
prenatal / neonatal period
Stricture/ web / sphincter of oddi dysfunction
Distal biliary obstruction
Reflux of pancreatic juice into bile dict
Chronic inflammation and increased bile duct
pressure - dilatation
11. Pancreaticobiliary maljunction
M/C proposed theory
57-96% of choledochal cyst
Extramural junction of pancreatic and bile duct beyond the
sphincter
Long common channel outside sphincter >1.86cm(nomal
<0.46cm)
Reflux of pancretic juice – biliary cyst
12. Others
Incomplete / partial pancreatic divisum
Oligoganglionosis of neck of cyst (Hirshsprungs of colon)
Congenital biliary cysts may be diagnosed prenatally and
can be associated with biliary atresia .
Fetal viral infection may also have a role; reovirus RNA has
been isolated from biliary tissue of neonates with infantile
biliary obstruction and choledochal cysts
13. Clinical features
Vast variety of symptoms
Classical triad – female child
Jaundice
Abdo pain
RUQ mass
Infants – conjugated hyperbilrubinemia(70%) ,
faolure to thrive, abdominal mass(30%)
Adults – mimics calculous cholecystitis
16. Imaging
USG abdomen
m/c first imaging
Noninvasive , accurate for type 1 and type 5 disease
Limited to choledococeles
Type 1
Irregular hypoechoic
segmental dilated
extrahepatic bile duct
Caroli disease
Multiple cysts adjacent to
intrahepatic bile ducts
17. MRCP
Noninvasive and investigation of choice
Defines the PBMJ
Extent of cyst
Stones within pancreatic and biliary duct
Polypoid filling defects – malignancy
18.
19. ERCP
Invasive
Focussed view of PBJ through ampulla
Extraction of intracystic stones
Biopsy / brush cytology of intracystic mass
Visualise stomach and esophagus – portal
hypertension
Endoscopic papillotomy for type 3 cyst
PTC
Proximal anatomy
Obstruction to ERCP
20. Other investigation
CBC, LFT, Amylase , coag profile
Preoperative investigations
CT abdomen
Assess hepatobiliary and pancreatic anatomy
Evaluate possible biliary malignancies
Metastatic disease
Vascular encasement
21.
22. Associated hepatobiliary conditions
1. Cystolithiasis
2. Hepaticolithiasis
3. Calculous cholecystitis
4. Pancreatitis
5. Cholangiocarcinoma
6. Intrahepatic abscess
7. Cirrhosis with portal hypertension
8. Spontaneous perforation and bleeding from
erosion of adjacent vessels
23. Cystolithiasis
Most frequent
2-72% prevalence in adults
Soft earthy pigmented stones
Viscous bile - forming bile duct / cyst casts
Follows stricture at previous cystoenterotomies / cyst
remnant in head of pancreas(incomplete excision )
Cause recurrent pancreatitis
24. Hepaticolithiasis
Follows complete or partial stricture at
cystoenteric anastomosis
Proximal migration of intracystic stones
>80% of type 4 – membranous / septal stenosis
of major lobar bile duct
25. Gallbladder disease
Acute / chronic cholecystitis/ cholilithiasis
Denovo/ after treatment
Gallbladder not excised during primary surgery
for bile cysts
26. Pancreatitis
2-70% prevalence in adults
Mild and acute, often relapsing presentation
Cause
Anomolous PBJ Type II
Cysto/cholelithiasis
Mucus by metastatic epithelium of cyst
28. Cholangiocarcinoma
26% incidence
Age related with cysts disease
Early 2nd decade – 2.3% risk
30-40yrs – 14.6%
Older age – 75%
Type I, IV and V.
Hyperplasia – dysplasia – carcinoma sequence
Bile stasis and intrabiliary carcinogens
29. Treatment
Principles
Complete cholangiographic definition of extent of
cystic process and associated ductal pathology
Control of biliary infections – antibiotics >drainage
Long term follow up
30. Midline incision
Exploration to rule out
mets
Cholecystectomy and
complete cyst excisionj
Reconstruction –
mucosa to mucosa
Hepatico jejunostomy
Hepatico duodenostomy
31.
32.
33. Type I cyst
Cholecystectomy + cyst excision +
Roux en Y hepaticojejunostomy
35. Type II cyst
Cholecystectomy + Complete cyst excision
Defect needs to be closed tranversely with or
without T tube
36. Type III cyst
Unusual and lower overall rate of malignancy
Surgical excision is uncommon
ERCP with unroofing of choledochocele and
sphincterotomy of CBD
Surgery – difficult sphincterotomy / suspecting
malignancy
Transverse dudodenotomy thro 2nd or 3rd part of
duodenum
37. Type IV cyst
Cyst excision of extra hepatic cyst and Roux En Y
Hepaticojejunostomy (as type I)
IVA cysts with complicated intrahepatic bile ducts
Hilar/intrahepatic bile duct strictures
Intrahepatic stones/ abscess
Requires abnormal hepatic segment resection
IVA cysts with intraheptic cysts of both lobes liver/
cirrhosis --Transhepatic stents needs to be placed
38. Caroli’s disease
Depends on extent and presence of congenital hepatic
fibrosis , cirrhosis, and portal hypertension.
m/c localised to left ductal system
Limited to one lobe –
Without concurrent cirrhosis / fibrosis , hepatic resection
with Roux en Y cholangiojejunostomy
If not feasible – Roux en Y intrahepatic
cholangiojejunostomy to intrahepatic cyst
39. Diffuse Carolis disease
Recurrent cholangitis ,portal hypertension with
variceal bleeding and liver failure
Long term medical therapy with antibiotics,
analgesics and litholytic agents may improve
Long term transhepatic stents – recurrent
cholangitis
Hepatic transplant is the treatment of choice
40. Advantages
Magnified view
Less pain
Fast recovery
Disadvantages
Lengthy opeartion
time
Steep learning curve
Costly instruments
Laparoscopic Choledochal cyst
excision
41.
42. Summary
Choledochal cyst disease is uncommon
Most common in children , increasing in adult population
Diagnosis by cross sectional imaging promarily CT and
MRCP
Risk of malignancy in untreated patients
Majority cases can be treated with Cholecystectomy ,
cyst excision and biliary reconstruction
Long term followup surveillance for cancer cholangitis
,intrahepatic stones are necessary
43. References
Surgery for liver, biliary tract and pancreas – L H
BLUMGART
MAINGOT,S abdominal operations 12ed
SABISTON Tectbook of surgery 20th ed
Editor's Notes
In extrahepatic cyst 1 and 4a
Stenosis of major heatic ducts should be sought in hepaticolothiasis patients
Complicates further intrahepatic dilatation / abscess
Denovo leading to incidental discovery of bile duct cysts / can evolove after treatment of bileduct cyst
Type 1 - major pancreatic duct joins CBD
Type 2 – major CBD joins pancreatic duct