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