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