Fact-sheet: Choledochal cyst


Updated on 10/03/2018 at 2:38 PM

Note : 0/10

View all RADEOS cases associated with this fact-sheet

Definition

Choledochal cysts are congenital malformations of the bile ducts.

Second most common biliary tract malformation after biliary atresia.

These are cystic, saccular, or fusiform dilatations communicating with the bile ducts, most often extrahepatic.

Rare condition, more common in Asia and in females, revealed before the age of 10 years++

Clinical features

Classic triad:

  • Pain
  • Jaundice
  • Right upper quadrant abdominal mass

Complication:

  • Pancreatitis
  • Cholangitis
  • Rupture

Ultrasound

Together with MRI, allows detection of a hepatic hilar cyst.

Well-defined cystic or fusiform formation with thin walls, containing echogenic material corresponding to biliary sludge.

Located near the portal trunk, separate from the hilum, potentially communicating with a dilated common hepatic duct or an intrahepatic biliary duct

CT

Good characterization

Hepatic hilar mass, above the head of the pancreas, iso- to hypodense with a thin wall, unchanged after contrast administration. It contains sludge.

MRI

MR cholangiopancreatography (MRCP):

  • Regular, well-defined mass of variable size in continuity with the common bile duct.
  • Fluid signal, hypointense on T1, hyperintense on T2, unchanged by contrast infection

Nuclear medicine

Scintigraphy: may show uptake if the cyst communicates with the biliary tract.

Management

Surgical treatment by complete cyst resection with Roux-en-Y hepaticojejunostomy and disconnection of the common bile duct and Wirsung duct.

Classification

  • Type I: cystic dilatation of the common bile duct (the most common) Ia: dilatation of part or all of the extrahepatic bile duct with a cystic duct arising from the cyst
  • Ib: segmental dilatation of the common bile duct
  • Ic: fusiform dilatation of the common bile duct and common hepatic duct
  • II: diverticulum of the common bile duct
  • III: choledochocele (intramural dilatation of the distal common bile duct protruding into the second portion of the duodenum)
  • IV: multiple cystic dilatations IVa: intrahepatic and extrahepatic dilatations
  • IVb: multiple extrahepatic cysts
  • V: Caroli disease

Differential diagnosis

Biliary atresia with hilar cyst

Duplication cyst

Hepatic cyst: biliary, hydatid

Mesothelial cyst

Renal cyst and ureteropelvic junction syndrome

Pancreatic pseudocyst