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Virtual Pediatric Hospital: Paediapaedia: Choledochal Cyst Paediapaedia: Gastrointestinal Diseases

Choledochal Cyst

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Clinical Presentation:
The classic clinical triad, which is seen in 25% of patients is jaundice, abdominal pain, and a right upper quadrant mass. Fifty percent of patients present by 10 years old.

A focal dilatation of the common and hepatic bile ducts of unknown etiology. Type 1 is the most common. Type 1A is dilation of the common bile duct below the cystic duct, Type 1B is dilation of the common and hepatic ducts. Type 2 is focal eccentric dilation of the common bile duct. Type 3 is a distal dilation of the common bile duct within the wall of the duodenum, a choledochocele. Caroli's disease, which is a cystic dilation of the intra and extrahepatic biliary ducts is sometimes classified as a Type 4 but is not really related to choledochal cyst. Complications include biliary cirrhosis and portal hypertension from prolonged extrahepatic obstruction, cyst rupture, stone formation, and cancer late in life.

Not applicable

Imaging Findings:
Ultrasound shows a large cystic lesion in the porta hepatis that is distinct from the gallbladder and communicates with dilated intrahepatic biliary ducts. DISIDA demonstrates normal hepatic extratction of tracer, excretion of tracer into the biliary system, and accumulation of tracer in the cyst.


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