Predictors of Need for Liver Transplantation in Children Undergoing Hepatoportoenterostomy for Biliary Atresia



      The purpose of this study was to determine perioperative risk factors for need of liver transplantation following hepatoportoenterostomy.


      A retrospective review of patients undergoing hepatoportoenterostomy for biliary atresia at our institution from 1990 to 2016 was completed.


      A total of 81 patients were identified with a median age of 51 days (IQR: 33-68) at hepatoportoenterostomy and a median follow-up time of 5.7 years (IQR: 1-11.6). Ten-year overall survival was 93% (95% CI: 84-97). Thirty-six patients (44%) ultimately required transplantation at a median time from hepatoportoenterostomy of 8.9 months (IQR: 5.2-19). The 10-year transplant-free survival was 36% (95%CI: 24-49). Steroid use (N=42) was not associated with improved 10-yr transplant-free survival (33% vs. 38%, p=0.690). Age at hepatoportoenterostomy was not significantly associated with the need for transplantation. Multivariable logistic regression analysis demonstrated that total bilirubin >2mg/dL (OR: 97, p<0.001) and albumin < 3.5g/dL (OR: 24, p=0.027) at 3 months after surgery were independent predictors of the need for transplantation, while adjusting for age, sex, prematurity, and steroid use.


      Overall survival for children with biliary atresia is excellent, although most patients will ultimately require liver transplantation. Total bilirubin and albumin level at 3 months following hepatoportoenterostomy are predictive of the need for transplantation. Steroid use is not associated with improved outcomes.


      BA (biliary atresia), BASM (Biliary atresia splenic malformation syndrome), CI (confidence interval), HPE (hepatoportoenterostomy), IQR (interquartile range)


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