For a study, researchers looked at 287 patients with biliary atresia to see if there was a correlation between the anatomy of the hepatic ducts and the choice of biliary reconstruction surgery. The Kasai hepatoportoenterostomy (Kasai) is the initial surgical procedure offered to most patients with biliary atresia. In contrast, a hepatic-cyst-jejunostomy has been reported effective in patients with the cystic form of biliary atresia. They performed an international multicenter retrospective review. About 287 patients were included, and 33 cases of cystic biliary atresia were identified. The outcomes were the serum total bilirubin level 3 months post-surgery and native liver survival at 2 years of age. The patients were categorized into 3 anatomical groups: patent intact hepatic ducts, patent hypoplastic hepatic ducts, and obliterated hepatic ducts. All 10 patients with patent intact hepatic duct group underwent hepatic-cyst-jejunostomy, and 9 experienced bile drainage and native liver survival. Among the 13 patients with hypoplastic hepatic ducts, 11 underwent the Kasai procedure, 9 had bile drainage, 2 underwent hepatic-cyst-jejunostomy, and one survived with the native liver. All of the patients who had their hepatic ducts obliterated had the Kasai procedure. Out of these patients, 5 had biliary drainage and survived with their native liver. 3 out of the 5 patients who did not have drainage needed a liver transplant. For patients with cystic biliary atresia, those with a connection between the cyst and intrahepatic bile ducts via an intact proximal hepatic duct had favorable clinical outcomes following hepatic-cyst-jejunostomy.

Source: journals.lww.com/jpgn/Abstract/2022/08000/The_Outcome_of_Patients_With_Cystic_Biliary.5.aspx