Clearance of jaundice after the modified Kasai’s operation predicts survival outcomes in patients with biliary atresia

Özet

The aim of this study was to assess the probability of survival with nativeliver (SNL) and the rate of esophageal variceal bleeding (EVB) as well as theirpotential risk factors, in patients diagnosed with Biliary Atresia (BA), whounderwent the hepaticoportoenterostomy (HPE) by retrospectively reviewingmedical records between 2007 and 2016. The subjects were classified as pooroutcomes if they died or a liver transplant (LT) was performed. A total of 73cases were enrolled. The average age at HPE was 106.2 +/- 58.5 days. Pooroutcome was observed in 27.4%, 54.8% survived with native liver and 17.8%were lost to follow-up. The principal cause of death was sepsis, followed bymassive upper GI hemorrhage. The overall 10-year SNL was 66.8%. Onlytotal bilirubin (TB)>3 mg/dL at 3, 6 months after HPE and presence ofassociated anomalies negatively affected SNL (p=0.0155, 0.0042 and 0.001,respectively). Most of the patients experienced EVB within 3 years of age,in which TB>9 mg/dL at 12 months after HPE was significantly associatedwith probability of the EVB outcome. Any interventions to improve jaundiceclearance after HPE should be strongly pursued in order to improve outcomesin BA patients, particularly in centers where liver transplantation (LT) isnot available. Surveillance esophagogastroduodenoscopy around the age of1.5 years in patients having TB>9 mg/dL may be beneficial to identify largevarices having potential fatal bleeding.