ABSTRACT
Choledochal malformation (CM) is a generic term which includes different patterns of bile duct dilatation other than those associated primarily with obstruction. The older term was choledochal cyst, but this rather implies a spherical, globular appearance only seen in one subtype of CM. Surgery excision and reconstruction is indicated in most cases unless there are contraindications to anaesthesia and the CM is asymptomatic. The surgical aim is to excise the gallbladder and the entire extrahepatic biliary system down to the junction with the pancreatic duct and up to the common hepatic duct and bifurcation. There may be cases where there are stones or a distal biliary stenosis and it is not clear if there is an underlying CM. Endoscopic retrograde cholangiopancreatography may then be needed to define the diagnosis and relieve jaundice by duct clearance of stones or stent placement.
