Choledochal Cysts

1. Pathogenesis:

• There are five types of choledochal cysts. The fifth type, known as Caroli's disease, will be discussed on the following page.



• Choledochal cysts are the most common congenital bile duct anomaly and are often associated with other gallbladder anomalies, biliary stenosis, or atresia, and congenital hepatis fibrosis.
   
• 60% present before the age of 10.
   
• Appear more often in females (3:1).
   
• Symptoms include obstructive jaundice in the neonate and right upper quadrant pain with intermittent jaundice and fever in older children and adults.
   
• Possible complications include the following:
   
• Rupture with secondary bile peritonitis.
   
• Cholangitis.
   
• Cirrhosis and portal hypertension.
   
• Calculus formation.
   
• Portal vein thrombosis.
   
• Liver abscess.
   
• Hemorrhage.
   
• Malignant transformation.
   
• Treatment involves surgical resection.
   

2. Radiographic findings:

• Type I
   
• US and CT: a cystic fluid-filled structure that communicates with the biliary system is visualized inferior to the porta hepatis.

                     

• Diagnosis is made with cholangiography showing a cystic collection of contrast material in communication with the bile duct.





• Type II:




 
• Type III: