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Kasai Portoenterostomy Delays: Is a HIDA Scan Worth the Wait?

      Highlights

      • What is currently known about this topic?
        • o
          Hepatobiliary scintigraphy is a non-invasive technique used to evaluate cholestatic infants.
        • o
          The technical gold-standard to exclude the diagnosis of Biliary Atresia is operative exploration.
      • What new information is contained in this article?
        • o
          Hepatobiliary 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.

      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)
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