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

• Published in: Pediatric radiology 2021-07, Vol.51 (8), p.1545-1554
• Main Authors: Napolitano, Marcello, Franchi-Abella, Stéphanie, Damasio, Beatrice Maria, Augdal, Thomas Angell, Avni, Fred Efraim, Bruno, Costanza, Darge, Kassa, Ključevšek, Damjana, Littooij, Annemieke Simone, Lobo, Luisa, Mentzel, Hans-Joachim, Riccabona, Michael, Stafrace, Samuel, Toso, Seema, Woźniak, Magdalena Maria, Di Leo, Giovanni, Sardanelli, Francesco, Ording Müller, Lil-Sofie, Petit, Philippe
• Format: Article
• Language: English
• Subjects: Bile ducts; Biliary atresia; Biopsy; Cholestasis; Cirrhosis; Diagnosis; Endoscopy; Espr; Fibrosis; Flow charts; Gallbladder diseases; Imaging; Imaging techniques; Inflammation; Liver; Liver cirrhosis; Liver diseases; Magnetic resonance; Magnetic resonance imaging; Medical diagnosis; Medical imaging; Medicine; Medicine & Public Health; Neonates; Neuroradiology; Nuclear Medicine; Oncology; Pediatrics; Radiology; Scintigraphy; Ultrasonic testing; Ultrasound
• ISSN: 0301-0449; 1432-1998
• DOI: 10.1007/s00247-021-05034-7

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.