Choledochal cyst, biliary Cyst

 Introduction
 Demography
 Classification
 Pathogenesis
 Clinical features
 Diagnosis
 Associated hepatobiliary pathology
 Treatment

Introduction
 Focal / diffuse dilatation of biliary tree
 2nd M/C congenital anomoly following atresia
 Single / multiple
 Extrahepatic / intrahepatic

Demography
 Uncommon , well recognised condition
 1% of benign biliary disease
 Incidence – varies
Asian – 1:13000(Japan 1:1000)
Western – 1 :1,50,000
 Sex – females (4:1)
 Age –
1st decade – 60%
Adults – 25%

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

 Todani’ modification – 1977

 Type 1 – M/C 79%
 Type 2 – 2.6%
 Type 3 – 4%
 Type 4 – 13%
 Type 5 - <1%

Pathogenesis
 Exactly unknown
 Most are congenital
 Genetic factors
 Multiple mechanisms and theories

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

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

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

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

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

 Intermittent RUQ pain,fever, tenderness and mild jaundice

 Abdo mass – uncommon, cyst associated malignancy
suspected
 Nausea , vomiting, anorexia
 Biliuria , clay colour stools – obstructive jaundice
 15% - overt evidence of cirrhosis / hepatic fibrosis and
portal hypertension

Diagnosis
 Imaging
 Blood investigations

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

MRCP
 Noninvasive and investigation of choice
 Defines the PBMJ
 Extent of cyst
 Stones within pancreatic and biliary duct
 Polypoid filling defects – malignancy

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

Other investigation
 CBC, LFT, Amylase , coag profile
 Preoperative investigations
 CT abdomen
Assess hepatobiliary and pancreatic anatomy
Evaluate possible biliary malignancies
Metastatic disease
Vascular encasement

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

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

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

Gallbladder disease
 Acute / chronic cholecystitis/ cholilithiasis
 Denovo/ after treatment
 Gallbladder not excised during primary surgery
for bile cysts

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

Hepatobiliary malignancies
 Cholangio / adenocarcinoma – M/C
 Adenoacanthoma
 Squamous cell carcinoma
 Anaplastic carcinoma
 Bile duct sarcoma
 Hepatoma
 Pancreatic carcinoma
 GB carcinoma

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

Treatment
Principles
 Complete cholangiographic definition of extent of
cystic process and associated ductal pathology
 Control of biliary infections – antibiotics >drainage
 Long term follow up

Midline incision
Exploration to rule out
mets
Cholecystectomy and
complete cyst excisionj
Reconstruction –
mucosa to mucosa
Hepatico jejunostomy
Hepatico duodenostomy

Type I cyst
 Cholecystectomy + cyst excision +
Roux en Y hepaticojejunostomy

Type II cyst
 Cholecystectomy + Complete cyst excision
 Defect needs to be closed tranversely with or
without T tube


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

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

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

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

Advantages
 Magnified view
 Less pain
 Fast recovery
Disadvantages
 Lengthy opeartion
time
 Steep learning curve
 Costly instruments
Laparoscopic Choledochal cyst
excision

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

References
 Surgery for liver, biliary tract and pancreas – L H
BLUMGART
 MAINGOT,S abdominal operations 12ed
 SABISTON Tectbook of surgery 20th ed

Choledochal cyst, biliary Cyst

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