Choledochal Cyst
Choledochal Cyst
Choledochal cyst
• Cystic dilatations of the extrahepatic biliary tree
• Intrahepatic biliary ducts
Choledochal Cyst
• Females > Males– Affects females three to eight times than males
• Unknown cause– weakness of the bile duct wall and increased
pressure secondary to partial biliary obstruction are required for biliary cyst formation
Choledochal Cyst
• More than 90% of patients have an anomalous pancreaticobiliary duct junction– with the pancreatic duct joining the common bile
duct >1 cm proximal to the ampulla
Clinical Manifestations
Clinical Manifestations
• Clinical triad – abdominal pain– jaundice– palpable abdominal mass
Clinical Manifestations
NEWBORNS• usually diagnosed within
the 1st month• jaundice• passage of acholic stools• often have palpable mass
in RUQ• pain not present
OLDER CHILDREN• abdominal pain present• jaundice• occasionally palpate a
soft mass in RUQ• can develop cholangitis,
eventually leading to cirrhosis & portal hypertension
Ancillary Procedures
Ancillary Procedures
• Fetus – screening prenatal ultrasound
• Older child or adolescent– abdominal ultrasonography may reveal a cystic
structure arising from the biliary tree
• CT scan will confirm the diagnosis
Abdominal Ultrasonography
• Screening procedure of choice• Class of the cyst can be identified• But preoperatively- confirmed by CT or
MRCP\• Cystic extrahepatic mass
Abdominal Ultrasonography
Choledochal cyst: surgical perspective. Sonogram shows a large unilocular cyst under the liver. (emedicine. Besner GE )
CT Scan
• Highly accurate in diagnosing and planning surgery
• Dilated cystic mass with clearly defined walls which is separate from the gallbladder
• mass arises from or actually is the extrahepatic bile duct usually is clear from its location and its relationships to surrounding structures ,
• the wall of the cyst can appear thickened, especially if multiple episodes of inflammation and cholangitis have occurred
CT Scan
• Large Type I choledochal cyst & adjacent gallbladder
ERCP
• reserved for patients in whom confusion remains after evaluation by less-invasive imaging modalities
Choledochal cyst and associated biliary anatomy
MRCP
• more detailed depiction of the anatomy of the cyst, and its relationship to the bifurcation of the hepatic ducts and the pancreas
Choledochal cyst: surgical perspective. Magnetic resonance cholangiopancreatography shows dilated hepatic ducts and common bile duct (CBD) of a type IV cyst. (emedicine. Besner GE )
Blood test
Non-specific• status of the patient • possible complications • ↑ serum bilirubin • ↓ blood clotting (severe cholangitis / longstanding
biliary blockage)
Types of Choledochal Cysts
Types
• Type I choledochal cysts
– dilatations of the entire common hepatic and common bile ducts or of segments of each
– saccular or fusiform in configuration
Types
• Type II choledochal cysts
– isolated protrusions or diverticula that project from the common bile duct wall
– sessile or connected to the common bile duct by a narrow stalk
Types
• Type III choledochal cysts
– choledochoceles– found in the
intraduodenal portion of the common bile duct
Types
• Type IVA cysts
– multiple dilatations of the intrahepatic and extrahepatic biliary tree
– large, solitary cyst of the extrahepatic duct is accompanied by multiple cysts of the intrahepatic ducts
Types
• Type IVB choledochal cysts
– multiple dilatations that involve only the extrahepatic bile duct
Types
• Type V choledochal cysts – dilatation of the intrahepatic
biliary ducts– numerous cysts are present
with interposed strictures that predispose the patient to intrahepatic stone formation, obstruction, and cholangitis
– found in both hepatic lobes