1. Bile duct cysts in adult
H.K. Oh M.D.
Department of Surgery

3. Associated entities Bile duct cyst Choledochal cyst
Caroli’s disease(syndrome)
Anomalous pancreatico-biliary ductal union

4. History First case report : over 100 years ago
1959 Alonso-Lej : first classification of extahepatic bile duct cysts
Caroli : the entity of intrahepatic bile duct cysts
Babbit : causative role of APBDU
Todani : classification by combining Alonso-Lej classification and Caroli’s disease
Komi : definition and classification of APBDU
JSPBM ; modification

5. Classification (Todani)

6. Classification (1995)

7. Epidemiology
Prevalence : 1/ – 2million (1% of all biliary disease)
*Japanese cases : over 1/3
Prevalence by type : Ⅰ(79%) Ⅱ(3%) Ⅲ(4%) Ⅳ(13.5%) Ⅴ(0.5)
* child : similar except infrequent type Ⅳ
Female >> Male (4-5 folds)
* sex hormones in pathogenesis : not proven
Detected in adulthood : 20 –30%

9. Etiology _ APBDU

10. Etiology _ APBDU manometric study, cystic fluid analysis,
histopathologic study, experimental study
APBDU in bile duct cyst : 21-90% (not100%)
Ⅰa Ⅲ Ⅰb
Ⅰc > Ⅳb+Ⅲ > Ⅱ
Ⅳa Ⅴ
almost 100% APBDU(-)
bile duct cyst in APBDU : not 100%
experimental study
Other causes hereditary – same type in family members (Iwafuchi 90’)
aganglionosis (in distal neck of cyst) (Kusunoki 88’)

11. Komi Classification

12. Clinical features
Calculous biliary disease : fever, tenderness, jaundice
Pancreatitis(30%) : more intense pain, vomiting
Cirrhosis(15%) :hepatosplenomegaly, portal hypertension
Malignancy : mass, jaundice, cachexia
Remains asymptomatic until adulthood(20%)
* most common : jaundice, mass, pain (13-38%)
* adult vs child

13. Associated HBP pathology
Cystolithiasis
Hepaticolithiasis
Calculus cholecystitis
Pancreatitis
Cancer
Intrahepatic abscess
Liver cirrhosis

14. Associated HBP pathology
hypothesis :
bile stasis, reflux of pancreatic juice, recurrent infection
 development of secondary bile acid
(unconjugated deoxycholate, lithocholate),
activated pancreatic enzyme
 inflammation, epithelial injury
 metaplasia, cancer

15. Associated HBP pathology
1.Cystolithiasis : adult (2-72%) > child
stasis and infection - CB, BP stones
- ass. with thick, viscous bile
- anastomotic stricture of prev. cystoenterostomy 
2.Hepatolithiasis : prox. migration of intracystic stones
- stasis and infection
- typeⅣ (presence of septal stenosis in IHD (80%)
 RPC, liver abscess
3.Pancreatitis , Gallbladder disease : like cystolithiasis

16. Associated HBP pathology
4. Intrahepatic abscess : Lt. IHD > Rt.
- Lt. Intrahepatic bile duct cysts > Rt.
- angulation : Lt. main IHD > Rt
5. Cirrhosis and portal vein hypertension
: biliary cirrhosis, congenital hepatic fibrosis, PVT
: hepatosplenomegaly, variceal bleeding, ascites
: limitation of surgical resection
- pericholecochal varices with hypervascularity of
hepatoduodenal ligament
- deterioration of hepatic functional reserve

17. Associated HBP pathology
6. Cancer
- Incidence : 2.5 % ~ 30% ( general population 의 20배)
- cholangioca.(intracystic: 57%), GB ca, denocanthoma,
squamous cell ca, anaplastic ca, bile duct sarcoma, pancreatic ca.
- age related : < 1% in the 1st decade, mean : 32yrs
- incidence by type : Ⅰ,Ⅳ, > Ⅱ,Ⅲ
- location : Ⅰa, Ⅳa (intracystic cholangioca. 70%, GB ca 30%) Ⅰc : GB ca 

18. Imaging
USG CT
ERCP
PTC
MRCP
IOCP
DISIDA scan

19. Imaging USG : initial investigative procedure advantage :
non-invasive, ability to imaging adjacent viscera
defining the extent of dilatation, presence of stone,
suspicion for cancer (nodularity, focal wall thickening)
disadvantage :
limited in identifying choledochocele, terminal CBD

20. Imaging Cholangiography: most important investigative procedure
advantages in defining :
- configuration and extent of cyst (type)
- stones
- filling defect suggesting cancer
- ductal stricture ( membranous, septal, prev. anastomosis)
- relationship of distal CBD to pancreatic duct
(long common channel, angle of fusion – type of APBDU)

21. Imaging ERCP : choice of cholangiography non-invasive
better visualization of an APBDU - most important
(PBDU out of sphincter of Oddi)
portal hypertension : esophageal, gastric varices
biopsy, brush cytology, stone extraction
EST (esp. typeⅢ)
PTC : prev. Roux-en-Y cystoenterostomy stricture,
tumor preventing visualization of IHD

22. Abdominal CT

23. ERCP

24. Treatment General principle 1. Definite treatment : surgical
- complete excision of all bile duct cyst and reconstruction by mucosa to mucosa bilio enterostomy
- partial excision and Roux-en-Y cystoenterostomy
2. Preoperative management
- complete cholangiographic evaluation
- control of biliary infection : antibiotics, drainage
3. Determining factors cyst type
- association of APBDU and type
- associated HBP pathology

26. Treatment (typeⅠ) Total cystectomy and Roux-en-Y hepaticojejunostomy
Reduction in cancer risk
1. Reflux(-) : potential carcinogenic effect of pancreatic secretion
2. Stasis(-) : bacterial overgrowth   production of mutagenic bile acid
3. Excision of abnormal cyst epithelium
Postop. Careful follow up
1. Recurrent cholangitis from anastomotic stricture(10-25%)
2. Cancer after excision

27. Treatment (typeⅠ) Surgical Technique
1. Initial mobilizing GB away from hilar structure
2. Dissection of post. cyst wall from hepatic a. and portal v.
3. Dissection of dist. cyst wall :
identification of panreaticobilary juction
avoidance of damage to pancreatic duct
meticulous ligation of small vessel : rebleeding

29. Treatment (typeⅠ) Several considerations
1. Another bilioenterostomies is needed
- hepaticoduodenostomy : direct endoscopic visualization
- ductoplasty, additional procedure :
co-existing biliary anomalies and anatomical variants
2. Cyst excision is not feasible
- portal hypertension, severe adhesion from recurrent inflammation, prev. drainage procedure
- Roux-en-Y choledochocystojejunostomy
+ (if rec. pancreatitis Hx(+)) : intracystic closure of base
 long- term functional result (60-70%)
 persistent cancer risk

31. Treatment (typeⅡ,Ⅲ) Type Ⅱ : limited experience
cyst excision and primary closure or T-tube insertion(depending on the size of the neck of the cyst)
Type Ⅲ :
1. Typically small cyst (<2-3cm)
transduodenal cyst excision  endoscopic sphincterotomy and cyst unroofing
* malignancy
2. Large intraduodenal choledochocele
transduodenal cyst excision
* indentification of pancreatic duct – most important
* variation : duct entering into post.cyst wall separate implantation of pancreatic duct

32. Treatment (typeⅣ) Type Ⅳb : type Ⅰ(+ Ⅲ)
Type Ⅳa : type Ⅰ+ management of intrahepatic problem
depending on :
the presence of Hilar or intrahepatic stricture, stone, abscess
localized or both lobes, cirrhosis
- ductoplasty, additional procedure
- hepatic resection of complicated segment
- transhepatic tube
- transplantation

33. Treatment (typeⅤ) 1. Hepatic resection in unilobar disease
2. Roux-en-Y bilioenterostomy in bilat. disease
: disappointing results
Initial nonoperative management
Liver trasplantation