CHOLEDOCHAL CYST

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CHOLEDOCHAL CYST

• OCTOBER 2005

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BACKGROUND

• Congenital anomalies of the bile ducts.
• First found western literature 1723 (Vater
  and Ezler).
• First systematic description of 96 cases in
  1959.
• Classification of 5 types (Todani 1977)

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PATHOPHYSIOLOGY

• Multifunctional
• Majority of choledochal cysts have anomalous
  junction of common bile duct with pancreatic
  duct (90).
• This allows reflux of pancreatic secretions
  and enzymes into common bile duct.
• Results in inflammation and weakening of bile
  duct wall.
• Formation of choledochal cyst.

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FREQUENCY

• Rare in US and western countries.
• Reported 1 case per 2 million live births.
• Prevalent in Asia 33 cases Japan 11000.
• Females 3-41

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MORTALITY/MORBIDITY

• Infants and children pancreatitis,
  cholangitis and histological evidence of
  hepatocellular inflammation and damage.
• cholangiocarcinoma 9-28.

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CLASSIFICATION (TODANI 1977)

• Type I 80-90
• Type II
• Type III
• Type IV
• Type V

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TYPE I CHOLEDOCHAL CYST

• Dilation of entire common hapatic and common
  bile duct or segments of each.
• Sacular or fusiform.

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TYPE II CHOLEDOCHAL CYST

• Relatively isolated protrusion that project
  from common bile duct wall.
• Connected by narrow stalk.

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TYPE III CHOLEDOCHAL CYST

• Found in intraduodenal portion of the common
  bile duct (choledochocade).

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TYPE IV CHOLEDOCHAL CYST

• Multiple dilations of the intrahepatic and
  extrahepatic biliary tree.
• Large extrahepatic cyst with multiple
  intrahepatic cysts.

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TYPE V CHOLEDOCHAL CYSTS

• Dilation of intraphepatic biliary radicles.
• Numerous cysts with structures causing
  intraphepatic stone formation, obstruction and
  cholangitis.

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SYMPTOMS

• Jaundice
• Pale stools (putty colour)
• Palpable mass right upper abdominal quadrant
• Hepatomegaly
• Acute pancreatitis

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INVESTIGATIONS

• USS abdomen
• CT (93) or MRI (100) cholangiogram
• HIDA scan
• Plain AXR do not identify choledochal cysts
  well.

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TREATMENT TYPE I

• Surgery
• Complete excision with Rovx-en-y biliary end
  to side anastromosis to restore biliary
  continuity to gastrointestinal track.

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TREATMENT TYPE II

• Total excision

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TREATMENT TYPE III

• 3 cm or less endoscopic removal.
• gt3 cm surgical excised.

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TREATMENT TYPE IV

• Dilated extraphepatic
• duct removed.

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TREATMENT TYPE V

• Liver Transplantation