Prenatal diagnosis of biliary atresia: A case series. (August 2017)

• Record Type: Journal Article
• Authors: Shen, O.; Sela, H.Y.; Nagar, H.; Rabinowitz, R.; Jacobovich, E.; Chen, D.; Granot, E.
• Is Part Of: Early human development. Volume 111(2017)
• Page Start: 16
• Page End: 19
• Publication Date: 2017-08
• Subjects: Biliary atresia -- Prenatal diagnosis -- Ultrasound -- Gallbladder -- Triangular cord sign -- Prenatal nonvisualization of the gallbladder -- Amniotic fluid -- Digestive enzymes -- Biliary cystic malformation -- Choledochal cyst; Fetus -- Periodicals; Neonatology -- Periodicals; Prenatal influences -- Periodicals; 612.65
• DOI: 10.1016/j.earlhumdev.2017.05.005
• Languages: English
• ISSNs: 0378-3782

Abstract

Abstract: Background: Biliary atresia is a progressive disease presenting with jaundice, and is the most common indication for liver transplantation in the pediatric population. Prenatal series have yielded conflicting results concerning a possible association between BA and prenatal nonvisualization of the gallbladder. Aims: This retrospective case series was performed to assess the association between biliary atresia, prenatal nonvisualization of the gallbladder and other sonographic signs. Study design/subjects: We identified biliary atresia patients who underwent a Kasai procedure by a single pediatric surgeon and/or follow up by a single pediatric gastroenterologist. Axial plane images and/or video recordings were scrutinized for sonographic signs of biliary atresia on the second trimester anomaly scan. Outcome measures: Proportion of biliary atresia cases with prenatal sonographic signs. Results: Twenty five charts of children with biliary and high quality prenatal images were retrieved. 6/25 (24%) of cases analyzed had prenatal nonvisualization of the gallbladder or a small gallbladder on the prenatal scan. Two cases had biliary atresia splenic malformation syndrome. None of the cases had additional sonographic markers of biliary atresia. Conclusions: Our study suggests that in addition to the well-established embryonic and cystic forms, an additional type can be suspected prenatally, which is characterized by prenatal nonvisualization of the gallbladder in the second trimester. This provides additional evidence that some cases of BA are of fetal rather than perinatal onset and may have important implications for prenatal diagnosis, for counseling and for research of the disease's etiology and pathophysiology. Highlights: Biliary atresia is associated with non visualized or small gallbladder on prenatal midtrimester sonography The implication is that some cases previously thought to be related to perinatal events, are related to earlier events