O Shen1, H Y Sela2, H Nagar3, R Rabinowitz2, E Jacobovich4, D Chen5, E Granot6. 1. Department of Ob/Gyn, Shaare Zedek Medical Center, Jerusalem, Israel. Electronic address: orishenmd@gmail.com. 2. Department of Ob/Gyn, Shaare Zedek Medical Center, Jerusalem, Israel. 3. Department of Pediatric Surgery, Shaare Zedek Medical Center, Jerusalem, Israel. 4. Department of Pediatrics, Kaplan Medical Center, Rehovot, Israel. 5. Tel-Aviv Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel. 6. Department of Pediatrics, Kaplan Medical Center, Rehovot, Israel; Department of Pediatrics, Shaare Zedek Medical Center, Jerusalem, Israel.
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.
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 atresiapatients 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.
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