Choledochal Cyst

 Possibly congenital malformation






Clinical Issues




• Usually presents in childhood before age 10
image 75% of patients are female

image Very common in Asian populations, particularly in Japan

• Jaundice, abdominal pain, mass are common findings

• Presentation
image Classic presentation (minority of patients) is RUQ mass, intermittent abdominal pain, jaundice

image Infants usually present with jaundice
– Infants at particular risk for chronic low-grade biliary obstruction leading to cirrhosis

• Increased risk of carcinoma (usually adenocarcinoma)


Imaging




• Cholangiography is definitive diagnostic procedure


Macroscopic




• Todani classification
image Type I (segmental or diffuse fusiform dilatation of common bile duct) is most common

• Range from few cm to > 15 cm


Microscopic




• Thickened, fibrotic cyst wall
image Epithelial lining may be intact or damaged, attenuated, or absent altogether

image Inflammation often present

• Liver biopsy specimen shows nonspecific changes of acute or chronic biliary obstruction

image
Cholangiogram
Anteroposterior radiograph during percutaneous cholangiogram shows a fusiform dilatation of the common bile duct with rapid change in caliber at the sphincter of Oddi, confirming type I choledochal cyst.


image
Todani Classification
The Todani classification includes types I (dilated common duct), II (diverticulum), III (choledochocele), IVa (extrahepatic cysts and cystic dilatation of intrahepatic ducts), IVb (multiple extrahepatic cysts), and V (multiple intrahepatic cysts).

image
Gross Resection Specimen
This resection shows a large saccular choledochal cyst image on the right of the photograph.

image
Cyst Wall
This section of a choledochal cyst wall has intact epithelium with underlying mural fibrosis and acute and chronic inflammation.


TERMINOLOGY


Definitions




• Cystic dilatation of biliary tract, usually extrahepatic


ETIOLOGY/PATHOGENESIS


Unknown




• Possible congenital malformation
image Majority of patients have abnormal pancreaticobiliary junction

– Long common channel between distal common bile duct and pancreatic duct

– May allow pancreatic secretions to reflux into bile ducts, possibly causing damage and eventual dilatation

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Choledochal Cyst

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