Choledochal CystFrans

Definition

•Cyst of the choledocal duct•Approximately 1 in 10.000-13.000 live

births•More common in women (3-4:1)•More common in Asian (up to 1:1000) ,

especially in Japan

Etiology

• Long common channel of the terminal bile duct and pancreatic duct (more than 1 cm from the sphincter)• Reflux of the pancreatic enzyms• Possible genetic influence, common with

other anomalies (congenital hepatic fibrosis, intrahepatic biliary cysts, and polycystic kidney disease)• Possible congenital duct wall anomaly

Etiology (2)• Weakness of the wall of the bile duct (Babbitt,

1973; Bismuth, 1999; Flanigan, 1975)• Obstruction of the distal choledochus (Spitz,

1977)• Combination of obstruction and weakness

(Kato, 1974)• Reflux of pancreatic enzymes into the CBD

secondary to an anomaly of the pancreaticobiliary junction (Babbitt, 1969; Kato, 1981; Miyano, 1979; Okada, 1990; Todani, 1984, Yamashiro, 1984)

Etiology (3)

Clinical Presentation

•Jaundice •Pain in RUQ•Mass (rare)

Todani Classification• Type I - Cystic or fusiform dilatation of the CBD;

most frequent type (90-95% of the cases).• Type II - Diverticulum of the CBD, with normal size

CBD• Type III - Choledochocele, a cystic dilatation of the

distal intramural portion of the CBD, typically protruding into the second portion of the duodenum

• Type IV - Cystic or fusiform dilatation of the CBD associated with cystic, fusiform, or saccular dilatation of intrahepatic bile ducts, also termed form fruste

• Type V - Cystic, fusiform, or saccular dilatation of the intrahepatic bile ducts associated with a normal CBD; may be associated with hepatic fibrosis (referred to as Caroli disease)

Type

Radiology

TherapyType I-IV• Cystojejunostomy • Total Excision and Cystojejunostomy• Biliary reconstruction can be performed with a

Roux-en-Y hepaticojejunostomy as high as possible, near the hilum of the liver (Hata, 1993; Joseph, 1990; Kasai, 1970; Miyano, 1996)

Type V• Hepatic Lobectomy

Lilly Technique

Complications

•Cholangitis•Biliary stone formation•Anastomotic stricture•Residual debris in the intrahepatic bile

ducts•Intrahepatic bile duct dilatation•Malignancy

Case• An Elsa/ P / 6 th / 10849772 / Bedah I• MRS : 07-07-2008• Dx pre op : Kista Duktus Koledokus ( K82.8 )• Operation : Excisi Kista + Hepatikoyeyunostomi

Roux-en-Y (5-512)• Operator : dr. PW SpBA Ass : dr

WDT SpB/dr ARS SpB

Gallbladder

Choledochal Cyst

Cystic Duct

Gallbladder

Choledochal Cyst

Hepaticojejunostomy Roux-en-Y