Abstract
Purpose
The purpose of this study was to determine perioperative risk factors for need of
liver transplantation following hepatoportoenterostomy.
Methods
A retrospective review of patients undergoing hepatoportoenterostomy for biliary atresia
at our institution from 1990 to 2016 was completed.
Results
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.
Conclusion
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.
Abbreviations:
BA (biliary atresia), BASM (Biliary atresia splenic malformation syndrome), CI (confidence interval), HPE (hepatoportoenterostomy), IQR (interquartile range)Keywords
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Article info
Publication history
Published online: March 14, 2019
Accepted:
February 21,
2019
Received:
February 14,
2019
Identification
Copyright
© 2019 Elsevier Inc. All rights reserved.
