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Biliary Atresia
Sara M. O'Hara, MD, FAAP
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Biliary atresia (BA)
            • Definitions

              • Absent or severely deficient extrahepatic biliary tree

            IMAGING

            • General Features

              • Ultrasonographic Findings

                • MR Findings

                  • Nuclear Medicine Findings

                    • Other Modality Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          Selected References

                                          1. Cho HH et al: Ultrasonography evaluation of infants with Alagille syndrome: In comparison with biliary atresia and neonatal hepatitis. Eur J Radiol. 85(6):1045-52, 2016
                                          2. Hanquinet S et al: Acoustic radiation force impulse sonography in assessing children with biliary atresia for liver transplantation. Pediatr Radiol. 46(7):1011-6, 2016
                                          3. Lee JY et al: The value of preoperative liver biopsy in the diagnosis of extrahepatic biliary atresia: A systematic review and meta-analysis. J Pediatr Surg. 51(5):753-61, 2016
                                          4. Nguyen M et al: Liquid chromatography-mass spectroscopy as a tool in the rapid diagnosis of biliary atresia: a pilot study. J Pediatr Surg. 51(6):923-6, 2016
                                          5. Shneider BL et al: Total serum bilirubin within 3 months of hepatoportoenterostomy predicts short-term outcomes in biliary atresia. J Pediatr. 170:211-217.e2, 2016
                                          6. Zhou L et al: Ultrasound for the diagnosis of biliary atresia: a meta-analysis. AJR Am J Roentgenol. 206(5):W73-82, 2016
                                          7. Chen Y et al: Postoperative steroid therapy for biliary atresia: systematic review and meta-analysis. J Pediatr Surg. 50(9):1590-4, 2015
                                          8. Hanquinet S et al: Contribution of acoustic radiation force impulse (ARFI) elastography to the ultrasound diagnosis of biliary atresia. Pediatr Radiol. 45(10):1489-95, 2015
                                          9. Leschied JR et al: Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases. Pediatr Radiol. 45(3):366-75, 2015
                                          10. Morel B et al: Prenatal ultrasound diagnosis of cystic biliary atresia. Clin Case Rep. 3(12):1050-1, 2015
                                          11. Nio M et al: Long-term outcomes of biliary atresia with splenic malformation. J Pediatr Surg. 50(12):2124-7, 2015
                                          12. Pinto RB et al: Cirrhosis in children and adolescents: an overview. World J Hepatol. 7(3):392-405, 2015
                                          13. Shen QL et al: Assessment of liver fibrosis by Fibroscan as compared to liver biopsy in biliary atresia. World J Gastroenterol. 21(22):6931-6, 2015
                                          14. Wang KS et al: Newborn screening for biliary atresia. Pediatrics. 136(6):e1663-9, 2015
                                          15. Zhou LY et al: Optimizing the US diagnosis of biliary atresia with a modified triangular cord thickness and gallbladder classification. Radiology. 277(1):181-91, 2015
                                          16. Siles P et al: A prospective pilot study: can the biliary tree be visualized in children younger than 3 months on Magnetic Resonance Cholangiopancreatography? Pediatr Radiol. 44(9):1077-84, 2014
                                          17. Ziessman HA: Hepatobiliary scintigraphy in 2014. J Nucl Med. 55(6):967-975, 2014
                                          18. Kwatra N et al: Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center. Pediatr Radiol. 43(10):1365-75, 2013
                                          19. Aziz S et al: Pseudo gallbladder sign in biliary atresia--an imaging pitfall. Pediatr Radiol. 41(5):620-6; quiz 681-2, 2011
                                          20. Broniszczak D et al: Liver transplantation in children with biliary atresia and polysplenia syndrome. Ann Transplant. 16(1):14-7, 2011
                                          21. Chan KW et al: The outcome of laparoscopic portoenterostomy for biliary atresia in children. Pediatr Surg Int. 27(7):671-4, 2011
                                          22. Mittal V et al: Role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia in infants younger than 90 days. AJR Am J Roentgenol. 196(4):W438-45, 2011
                                          23. Rozel C et al: Imaging of biliary disorders in children. Pediatr Radiol. 41(2):208-20, 2011
                                          24. Shen O et al: Absent gallbladder on fetal ultrasound: prenatal findings and postnatal outcome. Ultrasound Obstet Gynecol. 37(6):673-7, 2011
                                          25. Raval MV et al: Trends in age for hepatoportoenterostomy in the United States. Surgery. 148(4):785-91; discussion 791-2, 2010
                                          26. Sommer S et al: Green teeth associated with neonatal hyperbilirubinemia caused by biliary atresia: review and case report. J Clin Pediatr Dent. 35(2):199-202, 2010
                                          27. Hartley JL et al: Biliary atresia. Lancet. 374(9702):1704-13, 2009
                                          28. Davenport M et al: The outcome of the older (> or =100 days) infant with biliary atresia. J Pediatr Surg. 39(4):575-81, 2004
                                          29. Poddar U et al: Ursodeoxycholic acid-augmented hepatobiliary scintigraphy in the evaluation of neonatal jaundice. J Nucl Med. 45(9):1488-92, 2004
                                          30. Weerasooriya VS et al: Hepatic fibrosis and survival in biliary atresia. J Pediatr. 144(1):123-5, 2004
                                          31. Aktas S et al: Quantitative analysis of ductus proliferation, proliferative activity, Kupffer cell proliferation and angiogenesis in differential diagnosis of biliary atresia and neonatal hepatitis. Hepatogastroenterology. 50(54):1811-3, 2003
                                          32. Kanegawa K et al: Sonographic diagnosis of biliary atresia in pediatric patients using the "triangular cord" sign versus gallbladder length and contraction. AJR Am J Roentgenol. 181(5):1387-90, 2003
                                          33. Lee HJ et al: Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology. 229(2):395-400, 2003
                                          34. Vanderdood K et al: Biliary atresia and cerebellar hypoplasia in polysplenia syndrome. Pediatr Radiol. 33(9):652-4, 2003
                                          35. Wildhaber BE et al: The Kasai portoenterostomy for biliary atresia: A review of a 27-year experience with 81 patients. J Pediatr Surg. 38(10):1480-5, 2003
                                          36. Norton KI et al: MR cholangiography in the evaluation of neonatal cholestasis: initial results. Radiology. 222(3):687-91, 2002
                                          37. Tan Kendrick AP et al: Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length. Pediatr Radiol. 30(2):69-73, 2000
                                          38. O'Hara SM: Pediatric gastrointestinal nuclear imaging. Radiol Clin North Am. 34(4):845-62, 1996
                                          39. Kasai M et al: Technique and results of operative management of biliary atresia. World J Surg. 2(5):571-9, 1978
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Biliary atresia (BA)
                                                    • Definitions

                                                      • Absent or severely deficient extrahepatic biliary tree

                                                    IMAGING

                                                    • General Features

                                                      • Ultrasonographic Findings

                                                        • MR Findings

                                                          • Nuclear Medicine Findings

                                                            • Other Modality Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  Selected References

                                                                                  1. Cho HH et al: Ultrasonography evaluation of infants with Alagille syndrome: In comparison with biliary atresia and neonatal hepatitis. Eur J Radiol. 85(6):1045-52, 2016
                                                                                  2. Hanquinet S et al: Acoustic radiation force impulse sonography in assessing children with biliary atresia for liver transplantation. Pediatr Radiol. 46(7):1011-6, 2016
                                                                                  3. Lee JY et al: The value of preoperative liver biopsy in the diagnosis of extrahepatic biliary atresia: A systematic review and meta-analysis. J Pediatr Surg. 51(5):753-61, 2016
                                                                                  4. Nguyen M et al: Liquid chromatography-mass spectroscopy as a tool in the rapid diagnosis of biliary atresia: a pilot study. J Pediatr Surg. 51(6):923-6, 2016
                                                                                  5. Shneider BL et al: Total serum bilirubin within 3 months of hepatoportoenterostomy predicts short-term outcomes in biliary atresia. J Pediatr. 170:211-217.e2, 2016
                                                                                  6. Zhou L et al: Ultrasound for the diagnosis of biliary atresia: a meta-analysis. AJR Am J Roentgenol. 206(5):W73-82, 2016
                                                                                  7. Chen Y et al: Postoperative steroid therapy for biliary atresia: systematic review and meta-analysis. J Pediatr Surg. 50(9):1590-4, 2015
                                                                                  8. Hanquinet S et al: Contribution of acoustic radiation force impulse (ARFI) elastography to the ultrasound diagnosis of biliary atresia. Pediatr Radiol. 45(10):1489-95, 2015
                                                                                  9. Leschied JR et al: Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases. Pediatr Radiol. 45(3):366-75, 2015
                                                                                  10. Morel B et al: Prenatal ultrasound diagnosis of cystic biliary atresia. Clin Case Rep. 3(12):1050-1, 2015
                                                                                  11. Nio M et al: Long-term outcomes of biliary atresia with splenic malformation. J Pediatr Surg. 50(12):2124-7, 2015
                                                                                  12. Pinto RB et al: Cirrhosis in children and adolescents: an overview. World J Hepatol. 7(3):392-405, 2015
                                                                                  13. Shen QL et al: Assessment of liver fibrosis by Fibroscan as compared to liver biopsy in biliary atresia. World J Gastroenterol. 21(22):6931-6, 2015
                                                                                  14. Wang KS et al: Newborn screening for biliary atresia. Pediatrics. 136(6):e1663-9, 2015
                                                                                  15. Zhou LY et al: Optimizing the US diagnosis of biliary atresia with a modified triangular cord thickness and gallbladder classification. Radiology. 277(1):181-91, 2015
                                                                                  16. Siles P et al: A prospective pilot study: can the biliary tree be visualized in children younger than 3 months on Magnetic Resonance Cholangiopancreatography? Pediatr Radiol. 44(9):1077-84, 2014
                                                                                  17. Ziessman HA: Hepatobiliary scintigraphy in 2014. J Nucl Med. 55(6):967-975, 2014
                                                                                  18. Kwatra N et al: Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center. Pediatr Radiol. 43(10):1365-75, 2013
                                                                                  19. Aziz S et al: Pseudo gallbladder sign in biliary atresia--an imaging pitfall. Pediatr Radiol. 41(5):620-6; quiz 681-2, 2011
                                                                                  20. Broniszczak D et al: Liver transplantation in children with biliary atresia and polysplenia syndrome. Ann Transplant. 16(1):14-7, 2011
                                                                                  21. Chan KW et al: The outcome of laparoscopic portoenterostomy for biliary atresia in children. Pediatr Surg Int. 27(7):671-4, 2011
                                                                                  22. Mittal V et al: Role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia in infants younger than 90 days. AJR Am J Roentgenol. 196(4):W438-45, 2011
                                                                                  23. Rozel C et al: Imaging of biliary disorders in children. Pediatr Radiol. 41(2):208-20, 2011
                                                                                  24. Shen O et al: Absent gallbladder on fetal ultrasound: prenatal findings and postnatal outcome. Ultrasound Obstet Gynecol. 37(6):673-7, 2011
                                                                                  25. Raval MV et al: Trends in age for hepatoportoenterostomy in the United States. Surgery. 148(4):785-91; discussion 791-2, 2010
                                                                                  26. Sommer S et al: Green teeth associated with neonatal hyperbilirubinemia caused by biliary atresia: review and case report. J Clin Pediatr Dent. 35(2):199-202, 2010
                                                                                  27. Hartley JL et al: Biliary atresia. Lancet. 374(9702):1704-13, 2009
                                                                                  28. Davenport M et al: The outcome of the older (> or =100 days) infant with biliary atresia. J Pediatr Surg. 39(4):575-81, 2004
                                                                                  29. Poddar U et al: Ursodeoxycholic acid-augmented hepatobiliary scintigraphy in the evaluation of neonatal jaundice. J Nucl Med. 45(9):1488-92, 2004
                                                                                  30. Weerasooriya VS et al: Hepatic fibrosis and survival in biliary atresia. J Pediatr. 144(1):123-5, 2004
                                                                                  31. Aktas S et al: Quantitative analysis of ductus proliferation, proliferative activity, Kupffer cell proliferation and angiogenesis in differential diagnosis of biliary atresia and neonatal hepatitis. Hepatogastroenterology. 50(54):1811-3, 2003
                                                                                  32. Kanegawa K et al: Sonographic diagnosis of biliary atresia in pediatric patients using the "triangular cord" sign versus gallbladder length and contraction. AJR Am J Roentgenol. 181(5):1387-90, 2003
                                                                                  33. Lee HJ et al: Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology. 229(2):395-400, 2003
                                                                                  34. Vanderdood K et al: Biliary atresia and cerebellar hypoplasia in polysplenia syndrome. Pediatr Radiol. 33(9):652-4, 2003
                                                                                  35. Wildhaber BE et al: The Kasai portoenterostomy for biliary atresia: A review of a 27-year experience with 81 patients. J Pediatr Surg. 38(10):1480-5, 2003
                                                                                  36. Norton KI et al: MR cholangiography in the evaluation of neonatal cholestasis: initial results. Radiology. 222(3):687-91, 2002
                                                                                  37. Tan Kendrick AP et al: Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length. Pediatr Radiol. 30(2):69-73, 2000
                                                                                  38. O'Hara SM: Pediatric gastrointestinal nuclear imaging. Radiol Clin North Am. 34(4):845-62, 1996
                                                                                  39. Kasai M et al: Technique and results of operative management of biliary atresia. World J Surg. 2(5):571-9, 1978