link
Bookmarks
Biliary Atresia
Sara M. O'Hara, MD, FAAP
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Subscribe
0
9
4
0

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Definitions

              • Biliary atresia (BA): Inflammatory cholangiopathy of fetal life/infancy → progressive fibrosis & obliteration of extra- & intrahepatic bile ducts
                • Variable degrees of extrahepatic involvement

            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. Napolitano M et al: Practical approach for the diagnosis of biliary atresia on imaging, part 2: magnetic resonance cholecystopancreatography, hepatobiliary scintigraphy, percutaneous cholecysto-cholangiography, endoscopic retrograde cholangiopancreatography, percutaneous liver biopsy, risk scores and decisional flowchart. Pediatr Radiol. 51(8):1545-54, 2021
                                          2. Sandberg JK et al: Ultrasound shear wave elastography: does it add value to gray-scale ultrasound imaging in differentiating biliary atresia from other causes of neonatal jaundice? Pediatr Radiol. 51(9):1654-66, 2021
                                          3. Takimoto A et al: Postoperative intestinal obstruction in patients with biliary atresia impedes biliary excretion and results in subsequent liver transplantation. Pediatr Surg Int. 37(2):229-34, 2021
                                          4. Chen Y et al: Three-color risk stratification for improving the diagnostic accuracy for biliary atresia. Eur Radiol. 30(7):3852-61, 2020
                                          5. Di Serafino M et al: Ultrasound findings in paediatric cholestasis: how to image the patient and what to look for. J Ultrasound. 23(1):1-12, 2020
                                          6. Hattori K et al: Cyst size in fetuses with biliary cystic malformation: an exploration of the etiology of congenital biliary dilatation. Pediatr Gastroenterol Hepatol Nutr. 23(6):531-8, 2020
                                          7. Napolitano M et al: Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound. Pediatr Radiol. 51(2):314-31, 2020
                                          8. Shin HJ et al: Key imaging features for differentiating cystic biliary atresia from choledochal cyst: prenatal ultrasonography and postnatal ultrasonography and MRI. Ultrasonography. 40(2):301-11, 2020
                                          9. Dillman JR et al: Prospective assessment of ultrasound shear wave elastography for discriminating biliary atresia from other causes of neonatal cholestasis. J Pediatr. 212:60-65.e3, 2019
                                          10. Weng Z et al: Enlarged hepatic hilar lymph node: an additional ultrasonographic feature that may be helpful in the diagnosis of biliary atresia. Eur Radiol. 29(12):6699-6707, 2019
                                          11. Hwang SM et al: Early US findings of biliary atresia in infants younger than 30 days. Eur Radiol. 28(4):1771-1777, 2018
                                          12. Yang L et al: Diagnostic accuracy of serum matrix metalloproteinase-7 for biliary atresia. Hepatology. 68(6):2069-2077, 2018
                                          13. Koob M et al: The porta hepatis microcyst: an additional sonographic sign for the diagnosis of biliary atresia. Eur Radiol. 27(5):1812-1821, 2017
                                          14. Sharafinski ME Jr et al: Pediatric cholangiopathies: diseases of the gallbladder and biliary tract. Abdom Radiol (NY). 42(1):69-85, 2017
                                          15. Yoon HM et al: Diagnostic performance of sonographic features in patients with biliary atresia: a systematic review and meta-analysis. J Ultrasound Med. 36(10):2027-38, 2017
                                          16. 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
                                          17. 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
                                          18. 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
                                          19. 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
                                          20. Zhou L et al: Ultrasound for the diagnosis of biliary atresia: a meta-analysis. AJR Am J Roentgenol. 206(5):W73-82, 2016
                                          21. Leschied JR et al: Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases. Pediatr Radiol. 45(3):366-75, 2015
                                          22. Morel B et al: Prenatal ultrasound diagnosis of cystic biliary atresia. Clin Case Rep. 3(12):1050-1, 2015
                                          23. 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
                                          24. Ziessman HA: Hepatobiliary scintigraphy in 2014. J Nucl Med. 55(6):967-75, 2014
                                          25. 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
                                          26. Aziz S et al: Pseudo gallbladder sign in biliary atresia--an imaging pitfall. Pediatr Radiol. 41(5):620-6; quiz 681-2, 2011
                                          27. Rozel C et al: Imaging of biliary disorders in children. Pediatr Radiol. 41(2):208-20, 2011
                                          28. 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
                                          29. Lee HJ et al: Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology. 229(2):395-400, 2003
                                          30. Norton KI et al: MR cholangiography in the evaluation of neonatal cholestasis: initial results. Radiology. 222(3):687-91, 2002
                                          31. Kasai M et al: Technique and results of operative management of biliary atresia. World J Surg. 2(5):571-9, 1978
                                          Related Anatomy
                                          Loading...
                                          Related Differential Diagnoses
                                          Loading...
                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Definitions

                                                      • Biliary atresia (BA): Inflammatory cholangiopathy of fetal life/infancy → progressive fibrosis & obliteration of extra- & intrahepatic bile ducts
                                                        • Variable degrees of extrahepatic involvement

                                                    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. Napolitano M et al: Practical approach for the diagnosis of biliary atresia on imaging, part 2: magnetic resonance cholecystopancreatography, hepatobiliary scintigraphy, percutaneous cholecysto-cholangiography, endoscopic retrograde cholangiopancreatography, percutaneous liver biopsy, risk scores and decisional flowchart. Pediatr Radiol. 51(8):1545-54, 2021
                                                                                  2. Sandberg JK et al: Ultrasound shear wave elastography: does it add value to gray-scale ultrasound imaging in differentiating biliary atresia from other causes of neonatal jaundice? Pediatr Radiol. 51(9):1654-66, 2021
                                                                                  3. Takimoto A et al: Postoperative intestinal obstruction in patients with biliary atresia impedes biliary excretion and results in subsequent liver transplantation. Pediatr Surg Int. 37(2):229-34, 2021
                                                                                  4. Chen Y et al: Three-color risk stratification for improving the diagnostic accuracy for biliary atresia. Eur Radiol. 30(7):3852-61, 2020
                                                                                  5. Di Serafino M et al: Ultrasound findings in paediatric cholestasis: how to image the patient and what to look for. J Ultrasound. 23(1):1-12, 2020
                                                                                  6. Hattori K et al: Cyst size in fetuses with biliary cystic malformation: an exploration of the etiology of congenital biliary dilatation. Pediatr Gastroenterol Hepatol Nutr. 23(6):531-8, 2020
                                                                                  7. Napolitano M et al: Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound. Pediatr Radiol. 51(2):314-31, 2020
                                                                                  8. Shin HJ et al: Key imaging features for differentiating cystic biliary atresia from choledochal cyst: prenatal ultrasonography and postnatal ultrasonography and MRI. Ultrasonography. 40(2):301-11, 2020
                                                                                  9. Dillman JR et al: Prospective assessment of ultrasound shear wave elastography for discriminating biliary atresia from other causes of neonatal cholestasis. J Pediatr. 212:60-65.e3, 2019
                                                                                  10. Weng Z et al: Enlarged hepatic hilar lymph node: an additional ultrasonographic feature that may be helpful in the diagnosis of biliary atresia. Eur Radiol. 29(12):6699-6707, 2019
                                                                                  11. Hwang SM et al: Early US findings of biliary atresia in infants younger than 30 days. Eur Radiol. 28(4):1771-1777, 2018
                                                                                  12. Yang L et al: Diagnostic accuracy of serum matrix metalloproteinase-7 for biliary atresia. Hepatology. 68(6):2069-2077, 2018
                                                                                  13. Koob M et al: The porta hepatis microcyst: an additional sonographic sign for the diagnosis of biliary atresia. Eur Radiol. 27(5):1812-1821, 2017
                                                                                  14. Sharafinski ME Jr et al: Pediatric cholangiopathies: diseases of the gallbladder and biliary tract. Abdom Radiol (NY). 42(1):69-85, 2017
                                                                                  15. Yoon HM et al: Diagnostic performance of sonographic features in patients with biliary atresia: a systematic review and meta-analysis. J Ultrasound Med. 36(10):2027-38, 2017
                                                                                  16. 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
                                                                                  17. 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
                                                                                  18. 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
                                                                                  19. 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
                                                                                  20. Zhou L et al: Ultrasound for the diagnosis of biliary atresia: a meta-analysis. AJR Am J Roentgenol. 206(5):W73-82, 2016
                                                                                  21. Leschied JR et al: Shear wave elastography helps differentiate biliary atresia from other neonatal/infantile liver diseases. Pediatr Radiol. 45(3):366-75, 2015
                                                                                  22. Morel B et al: Prenatal ultrasound diagnosis of cystic biliary atresia. Clin Case Rep. 3(12):1050-1, 2015
                                                                                  23. 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
                                                                                  24. Ziessman HA: Hepatobiliary scintigraphy in 2014. J Nucl Med. 55(6):967-75, 2014
                                                                                  25. 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
                                                                                  26. Aziz S et al: Pseudo gallbladder sign in biliary atresia--an imaging pitfall. Pediatr Radiol. 41(5):620-6; quiz 681-2, 2011
                                                                                  27. Rozel C et al: Imaging of biliary disorders in children. Pediatr Radiol. 41(2):208-20, 2011
                                                                                  28. 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
                                                                                  29. Lee HJ et al: Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology. 229(2):395-400, 2003
                                                                                  30. Norton KI et al: MR cholangiography in the evaluation of neonatal cholestasis: initial results. Radiology. 222(3):687-91, 2002
                                                                                  31. Kasai M et al: Technique and results of operative management of biliary atresia. World J Surg. 2(5):571-9, 1978