Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging,
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Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound Marcello Napolitano 1 & Stéphanie Franchi-Abella 2 & Maria Beatrice Damasio 3 & Thomas A. Augdal 4 & Fred Efraim Avni 5 & Costanza Bruno 6 & Kassa Darge 7 & Damjana Ključevšek 8 & Annemieke S. Littooij 9 & Luisa Lobo 10 & Hans-Joachim Mentzel 11 & Michael Riccabona 12 & Samuel Stafrace 13,14 & Seema Toso 15 & Magdalena Maria Woźniak 16 & Gianni Di Leo 17 & Francesco Sardanelli 17,18 & Lil-Sofie Ording Müller 19 & Philippe Petit 20 Received: 6 April 2020 / Revised: 16 June 2020 / Accepted: 7 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract 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. Keywords Biliary atresia . Imaging . Infant . Magnetic resonance imaging . Recommendations . Review . Ultrasound
Introduction Biliary atresia is an important cause of obstructive jaundice in infants, causing progressive fibrosis and obliteration of extrahepatic and intrahepatic bile ducts, resulting in biliary cirrhosis in the absence of early surgery. Jaundice with pale stools and dark urine are present within the first days or weeks after birth. The prevalence of biliary atresia ranges from 1 in 5,000 to 1 in 20,000 depending on the geographic area, with the highest prevalence reported in Taiwan [
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