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<p>Biliary atresia (BA) is a rare condition in newborns characterised by inflammation and obliteration of bile ducts, leading to cirrhosis. Early diagnosis and treatment through Kasai portoenterostomy are crucial for native liver survival. While diagnostic methods include clinical assessment, laboratory tests and imaging, no non-invasive test can definitively rule out BA. Therefore, laparotomy with cholangiography remains the gold standard for diagnosis. This study assesses the feasibility, safety and accuracy of endoscopic retrograde cholangiopancreatography (ERCP) for diagnosing BA in newborns. The single-center study was conducted at the University Hospital Tübingen. The study included infants with neonatal cholestasis and suspected BA scheduled for ERCP from 2011 to 2023. We identified 60 jaundiced infants with suspected BA scheduled for diagnostic ERCP with a median age of 50&#xa0;days. Endoscopy was technically feasible in 58/60 children. In 23 of 58 cases, the bile ducts could not be visualized, suggesting BA. Normal bile duct anatomy was visualized in 35/58 patients, and BA was ruled out. The sensitivity was 100%, the specificity was 92.1%, the negative predictive value was 1.0, and the positive predictive value was 0.87. We did not observe any significant complications post-ERCP (in particular, no bleeding, perforation or pancreatitis). However, in one case (1/60; 2%), protracted weaning after general anesthesia occurred.</p><p><i>Conclusions</i>: ERCP is a safe, effective and reliable tool for excluding BA in jaundiced infants. ERCP’s integration into the diagnostic algorithm can reduce the burden of surgical diagnostic procedures and enable early diagnosis and portoenterostomy, thereby improving transplant-free survival.</p><p><Table Float="No" ID="Taba"> <tgroup cols="2"> <colspec align="left" colname="c1" colnum="1" /> <colspec align="left" colname="c2" colnum="2" /> <tbody> <row> <entry align="left" nameend="c2" namest="c1"> <p><b>What is known – What is new:</b></p> <p>• <i>Biliary atresia (BA) is the leading cause of obstructive jaundice in neonates, requiring early surgical intervention for optimal outcomes.</i></p> <p>• <i>Current diagnostic approaches are multimodal but lack a definitive, non-invasive method to exclude BA.</i></p> <p>• <i>This study demonstrates that ERCP is not only technically feasible in infants as small as 2.6 kg but also highly reliable in excluding BA with 100% sensitivity, 92.1% specificity and a negative predictive value of 1.0.</i></p> <p>• <i>ERCP is a key diagnostic tool for early exclusion of BA, offering greater diagnostic precision than ultrasound, liver biopsy, or laboratory parameters. </i></p> </entry> </row> </tbody> </tgroup> </Table></p> Graphical Abstract <p></p>

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Endoscopic retrograde cholangiopancreatography as a diagnostic key tool for biliary atresia: feasibility, safety and accuracy in neonates with cholestatic liver disease

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年12月13日

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  • Steffen Hartleif,
  • Michael Esser,
  • Jörg Fuchs,
  • Martin Götz,
  • Florian Graepler,
  • Johannes Hilberath,
  • Toni Illhardt,
  • Hans-Joachim Kirschner,
  • Nisar P. Malek,
  • Andreas Manger,
  • Jürgen Schäfer,
  • Ulrike Schempf,
  • Stephan Singer,
  • Christoph Slavetinsky,
  • Dietmar Stüker,
  • Ilias Tsiflikas,
  • Dörte Wichmann,
  • Ekkehard Sturm,
  • Christoph R. Werner

摘要

Biliary atresia (BA) is a rare condition in newborns characterised by inflammation and obliteration of bile ducts, leading to cirrhosis. Early diagnosis and treatment through Kasai portoenterostomy are crucial for native liver survival. While diagnostic methods include clinical assessment, laboratory tests and imaging, no non-invasive test can definitively rule out BA. Therefore, laparotomy with cholangiography remains the gold standard for diagnosis. This study assesses the feasibility, safety and accuracy of endoscopic retrograde cholangiopancreatography (ERCP) for diagnosing BA in newborns. The single-center study was conducted at the University Hospital Tübingen. The study included infants with neonatal cholestasis and suspected BA scheduled for ERCP from 2011 to 2023. We identified 60 jaundiced infants with suspected BA scheduled for diagnostic ERCP with a median age of 50 days. Endoscopy was technically feasible in 58/60 children. In 23 of 58 cases, the bile ducts could not be visualized, suggesting BA. Normal bile duct anatomy was visualized in 35/58 patients, and BA was ruled out. The sensitivity was 100%, the specificity was 92.1%, the negative predictive value was 1.0, and the positive predictive value was 0.87. We did not observe any significant complications post-ERCP (in particular, no bleeding, perforation or pancreatitis). However, in one case (1/60; 2%), protracted weaning after general anesthesia occurred.

Conclusions: ERCP is a safe, effective and reliable tool for excluding BA in jaundiced infants. ERCP’s integration into the diagnostic algorithm can reduce the burden of surgical diagnostic procedures and enable early diagnosis and portoenterostomy, thereby improving transplant-free survival.

What is known – What is new:

• Biliary atresia (BA) is the leading cause of obstructive jaundice in neonates, requiring early surgical intervention for optimal outcomes.

• Current diagnostic approaches are multimodal but lack a definitive, non-invasive method to exclude BA.

• This study demonstrates that ERCP is not only technically feasible in infants as small as 2.6 kg but also highly reliable in excluding BA with 100% sensitivity, 92.1% specificity and a negative predictive value of 1.0.

• ERCP is a key diagnostic tool for early exclusion of BA, offering greater diagnostic precision than ultrasound, liver biopsy, or laboratory parameters.

Graphical Abstract