Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound.

Napolitano M; Franchi-Abella S; Damasio MB; Augdal TA; Avni FE; Bruno C; Darge K; Ključevšek D; Littooij AS; Lobo L; Mentzel HJ; Riccabona M; Stafrace S; Toso S; Woźniak MM; Di Leo G; Sardanelli F; Ording Müller LS; Petit P

doi:10.1007/s00247-020-04840-9

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Journal article

Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound.

Napolitano M Department of Paediatric Radiology and Neuroradiology, V. Buzzi Children's Hospital, 32 Castelvetro St., 20154, Milan, Italy. marcello.napolitano1975@gmail.com.
Franchi-Abella S Pediatric Radiology Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, Le Kremlin-Bicêtre, France.
Damasio MB Radiology Department, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Augdal TA Section for Paediatric Radiology, Department of Radiology, University Hospital of North Norway, Tromsø, Norway.
Avni FE Department of Pediatric Radiology, Jeanne de Flandre Hospital, CHRU de Lille, Lille Cedex, France.
Bruno C Department of Radiology, Azienda Ospedaliera Universitaria Integrata Verona (AOUI), Verona, Italy.
Darge K Department of Radiology, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA.
Ključevšek D Department of Radiology, University Children's Hospital Ljubljana, Ljubljana, Slovenia.
Littooij AS Department of Paediatric Radiology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, The Netherlands.
Lobo L Serviço de Imagiologia Geral, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte (CHULN), Lisbon, Portugal.
Mentzel HJ Section of Pediatric Radiology, Institute of Diagnostic and Interventional Radiology, University Hospital, Jena, Germany.
Riccabona M Department of Radiology, Division of Pediatric Radiology, Medical University Graz and University Hospital LKH, Graz, Austria.
Stafrace S Department of Diagnostic Imaging, Sidra Medicine, Doha, Qatar.
Toso S Department of Pediatric Radiology, University Hospital of Geneva, Geneva, Switzerland.
Woźniak MM Department of Pediatric Radiology, Medical University of Lublin, Lublin, Poland.
Di Leo G Unit of Radiology, IRCCS Policlinico San Donato, San Donato Milanese, Milan, Italy.
Sardanelli F Unit of Radiology, IRCCS Policlinico San Donato, San Donato Milanese, Milan, Italy.
Ording Müller LS Division of Radiology and Nuclear Medicine, Department of Paediatric Radiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Petit P Aix Marseille Université, AP-HM, Equipe d'Accueil 3279 - IFR 125, Hôpital Timone Enfants, Service d'Imagerie Pédiatrique et Prénatale, Marseille, France.

2020-11-17

Published in:

Pediatric radiology. - 2020

English We 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 towards biliary cirrhosis, end-stage liver failure and death by age 3. Differentiation of biliary atresia from other nonsurgical causes of neonatal cholestasis is challenging because 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. Concerning imaging, our systematic literature review shows that ultrasonography is the main tool for pre- and neonatal diagnosis. Key prenatal features, when present, are non-visualisation of the gallbladder, cyst in the liver hilum, heterotaxy syndrome and irregular gallbladder walls. Postnatal imaging features have a very high specificity when present, but a variable sensitivity. Triangular cord sign and abnormal gallbladder have the highest sensitivity and specificity. The presence of macro- or microcyst or polysplenia syndrome is highly specific but less sensitive. The diameter of the hepatic artery and hepatic subcapsular flow are less reliable. When present in the context of acholic stools, dilated intrahepatic bile ducts rule out biliary atresia. Importantly, a normal US exam does not rule out biliary atresia. Signs of chronic hepatopathy and portal hypertension (portosystemic derivations such as patent ductus venosus, recanalised umbilical vein, splenomegaly and ascites) should be actively identified for - but are not specific for - biliary atresia.

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