Highlights
- •What is currently known about this topic?
- oHepatobiliary scintigraphy is a non-invasive technique used to evaluate cholestatic infants.
- oThe technical gold-standard to exclude the diagnosis of Biliary Atresia is operative exploration.
- o
- •What new information is contained in this article?
- oHepatobiliary scintigraphy compared to operative exploration for the diagnostic evaluation of cholestatic infants introduces significant time delays to biliary drainage surgery for infants with Biliary Atresia.
- o
Summary
Background
Biliary Atresia (BA), an obstructive cholangiopathy, is the most common cause of end-stage
liver disease and liver transplantation in children. Timely differentiation of BA
from other causes of neonatal jaundice remains a challenge, yet is critical to improving
outcomes.
Methods
Clinical characteristics including demographics, age at jaundice presentation, age
at hepatobiliary scintigraphy, age at surgery, severity of liver fibrosis, and native-liver
survival were reviewed in infants with hyperbilirubinemia and suspected BA for this
single center retrospective cohort study. We investigated the accuracy of hepatobiliary
scintigraphy as well as elapsed time from jaundice presentation to diagnostic intervention.
Results
BA was suspected in 234 infants. BA was identified in 17% of infants with hepatobiliary
scintigraphy and 72% of infants who underwent operative exploration without hepatobiliary
scintigraphy. Elapsed time from jaundice presentation to Kasai Portoenterostomy (KPE)
for BA patients was 2.1x longer if hepatobiliary scintigraphy was obtained (p=0.084). The mean age at KPE for this cohort was 66.8 days (n=54), with a significantly higher mean age at KPE (75.2 days) for infants who were later
listed or underwent liver transplantation (p=0.038). Histologically, the lowest liver fibrosis scores were seen in infants undergoing
KPE <30 days old and worsened significantly with increased age (p< 0.001).
Conclusion
Hepatobiliary scintigraphy compared to operative exploration for the diagnostic evaluation
of infants with suspected BA introduces significant time delays to KPE but enables
avoidance of surgery in some infants. The temporal pattern of worsening cholestatic
liver injury from BA with each day of increased age highlights the importance of intervening
as early as possible for the best prognosis.
Type of Study
Retrospective study, Level of evidence: III.
Keywords
Abbreviations:
BA (Biliary Atresia), KPE (Kasai Portoenterostomy), US (Ultrasound), HIDA (Hepatobiliary Scintigraphy), ERCP (endoscopic retrograde cholangiopancreatography), IOC (intra-operative cholangiogram), EMR (electronic medical record), NLS (native-liver survival)To read this article in full you will need to make a payment
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Article info
Publication history
Accepted:
January 5,
2023
Received in revised form:
December 26,
2022
Received:
July 20,
2022
Publication stage
In Press Accepted ManuscriptIdentification
Copyright
© 2023 Elsevier Inc. All rights reserved.
