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Practical approach for the diagnosis of biliary atresia on imaging, part 2: magnetic resonance cholecystopancreatography, hepatobiliary scintigraphy, percutaneous cholecysto-cholangiography, endoscopic retrograde cholangiopancreatography, percutaneous liver biopsy, risk scores and decisional flowchart

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Abstract

We aim to present a practical approach to imaging in suspected biliary atresia, an inflammatory cholangiopathy of infancy resulting in progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts. Left untreated or with failure of the Kasai procedure, biliary atresia progresses to biliary cirrhosis, end-stage liver failure and death within the first years of life. Differentiating biliary atresia from other nonsurgical causes of neonatal cholestasis is difficult as there is no single method for diagnosing biliary atresia and clinical, laboratory and imaging features of this disease overlap with those of other causes of neonatal cholestasis. In this second part, we discuss the roles of magnetic resonance (MR) cholecystopancreatography, hepatobiliary scintigraphy, percutaneous biopsy and percutaneous cholecysto-cholangiography. Among imaging techniques, ultrasound (US) signs have a high specificity, although a normal US examination does not rule out biliary atresia. Other imaging techniques with direct opacification of the biliary tree combined with percutaneous liver biopsy have roles in equivocal cases. MR cholecystopancreatography and hepatobiliary scintigraphy are not useful for the diagnosis of biliary atresia. We propose a decisional flowchart for biliary atresia diagnosis based on US signs, including elastography, percutaneous cholecysto-cholangiography or endoscopic retrograde cholangiopancreatography and liver biopsy.

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Authors and Affiliations

  1. Department of Paediatric Radiology and Neuroradiology, V. Buzzi Children’s Hospital, 32 Castelvetro Street, 20154, Milan, Italy

    Marcello Napolitano

  2. Pediatric Radiology Department, Hôpital Bicêtre, Hôpitaux Universitaires Paris-Saclay, Assistance Publique Hôpitaux de Paris, Le Kremlin-Bicêtre, France

    Stéphanie Franchi-Abella

  3. Radiology Department, IRCCS Istituto Giannina Gaslini, Genoa, Italy

    Beatrice Maria Damasio

  4. Section of Paediatric Radiology, Department of Radiology, University Hospital of North Norway, Tromsø, Norway

    Thomas Angell Augdal

  5. Department of Pediatric Radiology, Jeanne de Flandre Hospital, CHRU de Lille, Lille Cedex, France

    Fred Efraim Avni

  6. Department of Radiology, AOUI, Verona, Italy

    Costanza Bruno

  7. Department of Radiology, The Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA

    Kassa Darge

  8. Department of Radiology, University Children’s Hospital Ljubljana, Ljubljana, Slovenia

    Damjana Ključevšek

  9. Department of Paediatric Radiology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands

    Annemieke Simone Littooij

  10. Serviço de Imagiologia Geral, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte (CHULN), Lisbon, Portugal

    Luisa Lobo

  11. Section of Pediatric Radiology, Institute of Diagnostic and Interventional Radiology, University Hospital, Jena, Germany

    Hans-Joachim Mentzel

  12. Division of Pediatric Radiology, Department of Radiology, Medical University Graz, University Hospital LKH, Graz, Austria

    Michael Riccabona

  13. Department of Diagnostic Imaging, Sidra Medicine, Doha, Qatar

    Samuel Stafrace

  14. Weill Cornell Medicine, Doha, Qatar

    Samuel Stafrace

  15. Department of Pediatric Radiology, University Hospital of Geneva, Geneva, Switzerland

    Seema Toso

  16. Department of Pediatric Radiology, Medical University of Lublin, Lublin, Poland

    Magdalena Maria Woźniak

  17. Unit of Radiology, IRCCS Policlinico San Donato, San Donato Milanese, Milan, Italy

    Giovanni Di Leo & Francesco Sardanelli

  18. Department of Biomedical Sciences for Health, Università degli Studi di Milano, Milan, Italy

    Francesco Sardanelli

  19. Division of Radiology and Nuclear Medicine, Department of Paediatric Radiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway

    Lil-Sofie Ording Müller

  20. APHM, Equipe d’Accueil 3279 - IFR 125, Service d’Imagerie Pédiatrique et Prénatale, Hôpital Timone Enfants, Aix Marseille Univ, Marseille, France

    Philippe Petit

Authors
  1. Marcello Napolitano
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  2. Stéphanie Franchi-Abella
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  8. Damjana Ključevšek
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  9. Annemieke Simone Littooij
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  10. Luisa Lobo
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  13. Samuel Stafrace
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  14. Seema Toso
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  15. Magdalena Maria Woźniak
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  16. Giovanni Di Leo
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  18. Lil-Sofie Ording Müller
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  19. Philippe Petit
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Correspondence to Marcello Napolitano.

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Napolitano, M., Franchi-Abella, S., Damasio, B.M. et al. Practical approach for the diagnosis of biliary atresia on imaging, part 2: magnetic resonance cholecystopancreatography, hepatobiliary scintigraphy, percutaneous cholecysto-cholangiography, endoscopic retrograde cholangiopancreatography, percutaneous liver biopsy, risk scores and decisional flowchart. Pediatr Radiol 51, 1545–1554 (2021). https://doi.org/10.1007/s00247-021-05034-7

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