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

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

History First case report : over 100 years ago 1959 Alonso-Lej : first classification of extahepatic bile duct cysts 1958 Caroli : the entity of intrahepatic bile duct cysts 1969 Babbit : causative role of APBDU 1977 Todani : classification by combining Alonso-Lej classification and Caroli’s disease 1977 Komi : definition and classification of APBDU 1983- JSPBM ; modification

Classification (Todani)

Classification (1995)

Epidemiology Prevalence : 1/ 13000 – 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%

Etiology _ APBDU

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’)

Komi Classification

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

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

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

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

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

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 

Imaging USG CT ERCP PTC MRCP IOCP DISIDA scan

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

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)

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

Abdominal CT

ERCP

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

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

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

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

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

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

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