ABSTRACT
Congenital choledochal malformation (CCM) is a better term for the spectrum of biliary abnormalities characterized by dilatation. The incidence of CCM is not known though it is clearly much more common in East Asian countries and may even be increasing. Choledochal perforation may occur in a few, either directly into the peritoneal cavity where it can resemble acute-onset ascites or retroperitoneally tracking down the paracolic gutter and mimicking appendicitis. Endoscopic retrograde cholangiopancreatography may still be needed for diagnosis in some children with suspected common channel syndrome presenting with recurrent pancreatitis and only borderline biliary dilatation. The inspection and irrigation may be performed through a pediatric cystoscope if the common channel is wide enough. The distal choledochal cyst is clipped and divided at the level of the orifice of common channel. When the cyst is intensely inflamed and extensive pericystic adhesion is present, the dissection of cystic wall from portal vein is carried out carefully while viewing cyst internally and externally.
