Fig. 4

Schema of the disease process together with possible etiologies. Biliary perforation secondary to increased luminal pressure in the biliary cyst due to possible obstruction by protein plugs in the distal end of the common bile duct at 81 days old (Panel a). The resultant decrease in intraluminal pressure, together with adequate drainage of the common bile duct and spontaneous removal of the protein plugs contributes to healing the site of perforation (Panel b). On the hepatic side, the perihilar bile ducts and common hepatic duct become progressively narrower, marking the beginning of development of biliary atresia. Finally, the perihilar bile ducts become completely obliterated and the distal side of the common bile duct becomes narrower, as was observed during the surgery at 120 days old (Panel c)