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Choledochal Cyst

Frans

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

Therapy
Type 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

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