Skip to main content
SpringerLink
Log in

Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound

  • ESPR
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

A Correction to this article was published on 24 December 2020

This article has been updated

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9
Fig. 10
Fig. 11
Fig. 12
Fig. 13
Fig. 14
Fig. 15
Fig. 16

Similar content being viewed by others

Change history

  • 24 December 2020

    A Correction to this paper has been published: <ExternalRef><RefSource>https://doi.org/10.1007/s00247-020-04936-2</RefSource><RefTarget Address="10.1007/s00247-020-04936-2" TargetType="DOI"/></ExternalRef>

References

  1. The NS, Honein MA, Caton AR et al (2007) Risk factors for isolated biliary atresia, National Birth Defects Prevention Study, 1997–2002. Am J Med Genet A 143A:2274–2284

    CAS  PubMed  Google Scholar 

  2. Schreiber RA, Barker CC, Roberts EA et al (2007) Biliary atresia: the Canadian experience. J Pediatr 151:659–665

    PubMed  Google Scholar 

  3. McKiernan PJ, Baker AJ, Kelly DA (2000) The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 355:25–29

    CAS  PubMed  Google Scholar 

  4. Sokol RJ, Mack C (2001) Etiopathogenesis of biliary atresia. Semin Liver Dis 21:517–524

    CAS  PubMed  Google Scholar 

  5. Schwarz KB, Haber BH, Rosenthal P et al (2013) Extrahepatic anomalies in infants with biliary atresia: results of a large prospective North American multicenter study. Hepatology 58:1724–1731

    PubMed  PubMed Central  Google Scholar 

  6. Pariente D, Franchi-Abella S (2012) [Pathology of the child’s bile ducts]. EMC – Radiologie et imagerie médicale: Abdominale - Digestive 7:1–17

  7. Balistreri WF (1985) Neonatal cholestasis. J Pediatr 106:171–184

    CAS  PubMed  Google Scholar 

  8. Morotti RA, Dhanpat J (2013) Pediatric cholestatic disorders. Approach to pathologic diagnosis. Surg Pathol Clin 6:205–225

    PubMed  Google Scholar 

  9. Russo P, Magee JC, Boitnott J et al (2011) Design and validation of the biliary atresia research consortium histologic assessment system for cholestasis in infancy. Clin Gastroenterol Hepatol 9:357–362

    PubMed  PubMed Central  Google Scholar 

  10. Rastogi A, Krishnani N, Yachha SK et al (2009) Histopathological features and accuracy for diagnosing biliary atresia by prelaparotomy liver biopsy in developing countries. J Gastroenterol Hepatol 24:97–102

    PubMed  Google Scholar 

  11. Balistreri WF, Bezerra JA (2006) Whatever happened to “neonatal hepatitis”? Clin Liver Dis 10:27–53

    PubMed  Google Scholar 

  12. Chen X, Dong R, Shen Z et al (2016) Value of gamma-glutamyl transpeptidase for diagnosis of biliary atresia by correlation with age. J Pediatr Gastroenterol Nutr 63:370–373

    CAS  PubMed  Google Scholar 

  13. Di Pasquo E, Kuleva M, Rousseau A et al (2019) Outcome of non-visualization of fetal gallbladder on second-trimester US: cohort study and systematic review of literature. Ultrasound Obstet Gynecol 54:582–588

    PubMed  Google Scholar 

  14. Koukoura O, Kelesidou V, Delianidou M et al (2019) Prenatal sonographic diagnosis of biliary tract malformations. J Clin Ultrasound 47:292–297

    PubMed  Google Scholar 

  15. Shen O, Sela HY, Nagar H et al (2017) Prenatal diagnosis of biliary atresia: a case series. Early Hum Dev 11:16–19

    Google Scholar 

  16. Ruiz A, Robles A, Salva F et al (2017) Prenatal nonvisualization of the gallbladder: a diagnostic and prognostic dilemma. Fetal Diagn Ther 42:150–152

    PubMed  Google Scholar 

  17. Bardin R, Ashwal E, Davidov B et al (2016) Nonvisualization of the fetal gallbladder: can levels of gamma-glutamyl transpeptidase in amniotic fluid predict fetal prognosis? Fetal Diagn Ther 39:50–55

    PubMed  Google Scholar 

  18. Berger Y, Superina RA, Zbar A et al (2015) A case series of congenital hepatic hilar cyst: recommendations for diagnosis and management. Isr Med Assoc J 17:32–36

    PubMed  Google Scholar 

  19. Morel B, Kolanska K, Dhombres F et al (2015) Prenatal ultrasound diagnosis of cystic biliary atresia. Clin Case Rep 3:1050–1051

    PubMed  PubMed Central  Google Scholar 

  20. Cong X, Sun X, Liu S (2015) Evaluation and screening ultrasonic signs in the diagnosis of fetal biliary cystic malformation. J Matern Fetal Neonatal Med 28:2100–2105

    PubMed  Google Scholar 

  21. Nori M, Venkateshwarlu J, Vijaysekhar, Prasad GR (2013) Extrahepatic biliary atresia with choledochal cyst: prenatal MRI predicted and post natally confirmed: a case report. Indian J Radiol Imaging 23:238–242

    PubMed  PubMed Central  Google Scholar 

  22. Shen O, Rabinowitz R, Yagel S, Gal M (2011) Absent gallbladder on fetal ultrasound: prenatal findings and postnatal outcome. Ultrasound Obstet Gynecol 37:673–677

    CAS  PubMed  Google Scholar 

  23. Boughanim M, Benachi A, Dreux S et al (2008) Nonvisualization of the fetal gallbladder by second-trimester US scan: strategy of clinical management based on four examples. Prenat Diagn 28:46–48

    PubMed  Google Scholar 

  24. Park WH, Choi SO, Lee HJ (1999) The ultrasonographic ‘triangular cord’ coupled with gallbladder images in the diagnostic prediction of biliary atresia from infantile intrahepatic cholestasis. J Pediatr Surg 34:1706–1710

    CAS  PubMed  Google Scholar 

  25. Kotb MA, Kotb A, Sheba MF et al (2001) Evaluation of the triangular cord sign in the diagnosis of biliary atresia. Pediatrics 108:416–420

    CAS  PubMed  Google Scholar 

  26. Tan Kendrick AP, Phua KB, Ooi BC, Tan CE (2003) Biliary atresia: making the diagnosis by the gallbladder ghost triad. Pediatr Radiol 33:311–315

    PubMed  Google Scholar 

  27. Visrutaratna P, Wongsawasdi L, Lerttumnongtum P et al (2003) Triangular cord sign and US features of the gall bladder in infants with biliary atresia. Australas Radiol 47:252–256

    PubMed  Google Scholar 

  28. Kanegawa K, Akasaka Y, Kitamura E et al (2003) Sonographic diagnosis of biliary atresia in pediatric patients using the “triangular cord” sign versus gallbladder length and contraction. AJR Am J Roentgenol 181:1387–1390

    PubMed  Google Scholar 

  29. Lee HJ, Lee SM, Park WH, Choi SO (2003) Objective criteria of triangular cord sign in biliary atresia on US scans. Radiology 229:395–400

    PubMed  Google Scholar 

  30. Humphrey TM, Stringer MD (2007) Biliary atresia: US diagnosis. Radiology 244:845–851

    PubMed  Google Scholar 

  31. Kim WS, Cheon J-E, Youn BJ et al (2007) Hepatic arterial diameter measured with US: adjunct for US diagnosis of biliary atresia. Radiology 245:549–555

    PubMed  Google Scholar 

  32. Takamizawa S, Zaima A, Muraji T et al (2007) Can biliary atresia be diagnosed by ultrasonography alone? J Pediatr Surg 42:2093–2096

    PubMed  Google Scholar 

  33. Lee MS, Kim MJ, Lee MJ et al (2009) Biliary atresia: color Doppler US findings in neonates and infants. Radiology 252:282–289

    PubMed  Google Scholar 

  34. Imanieh MH, Dehghani SM, Bagheri MH et al (2010) Triangular cord sign in detection of biliary atresia: is it a valuable sign? Dig Dis Sci 55:172–175

    PubMed  Google Scholar 

  35. Sun Y, Zheng S, Qian Q (2011) Ultrasonographic evaluation in the differential diagnosis of biliary atresia and infantile hepatitis syndrome. Pediatr Surg Int 27:675–679

    PubMed  Google Scholar 

  36. Aziz S, Wild Y, Rosenthal P, Goldstein RB (2011) Pseudo gallbladder sign in biliary atresia — an imaging pitfall. Pediatr Radiol 41:620–626

    PubMed  PubMed Central  Google Scholar 

  37. Mittal V, Saxena AK, Sodhi KS et al (2011) Role of abdominal sonography in the preoperative diagnosis of extrahepatic biliary atresia in infants younger than 90 days. AJR Am J Roentgenol 196:W438–W445

    PubMed  Google Scholar 

  38. El-Guindi MA, Sira MM, Konsowa HA et al (2013) Value of hepatic subcapsular flow by color Doppler ultrasonography in the diagnosis of biliary atresia. J Gastroenterol Hepatol 28:867–872

    PubMed  Google Scholar 

  39. El-Guindi MA, Sira MM, Sira AM et al (2014) Design and validation of a diagnostic score for biliary atresia. J Hepatol 61:116–123

    PubMed  Google Scholar 

  40. Hanquinet S, Courvoisier DS, Rougemont AL et al (2015) Contribution of acoustic radiation force impulse (ARFI) elastography to the US diagnosis of biliary atresia. Pediatr Radiol 45:1489–1495

    PubMed  Google Scholar 

  41. Lee SM, Cheon JE, Choi YH et al (2015) Ultrasonographic diagnosis of biliary atresia based on a decision-making tree model. Korean J Radiol 16:1364–1372

    PubMed  PubMed Central  Google Scholar 

  42. Ağın M, Tümgör G, Alkan M et al (2016) Clues to the diagnosis of biliary atresia in neonatal cholestasis. Turk J Gastroenterol 27:37–41

    PubMed  Google Scholar 

  43. Koob M, Pariente D, Habes D et al (2017) The porta hepatis microcyst: an additional sonographic sign for the diagnosis of biliary atresia. Eur Radiol 27:1812–1821

    PubMed  Google Scholar 

  44. Choi SO, Park WH, Lee HJ, Woo SK (1996) Triangular cord: a sonographic finding applicable in the diagnosis of biliary atresia. J Pediatr Surg 31:363–366

    CAS  PubMed  Google Scholar 

  45. Hwang SM, Jeon TY, Yoo SY et al (2018) US findings of biliary atresia in infants younger than 30 days. Eur Radiol 28:1771–1777

    PubMed  Google Scholar 

  46. Caponcelli E, Knisely AS, Davenport M (2008) Cystic biliary atresia: an etiologic and prognostic subgroup. J Pediatr Surg 43:1619–1624

  47. Kim MJ, Park YN, Han SJ et al (2000) Biliary atresia in neonates and infants: triangular area of high signal intensity in the porta hepatis at T2-weighted MR cholangiography with US and histopathologic correlation. Radiology 215:395–401

    CAS  PubMed  Google Scholar 

  48. Zhou LY, Jiang H, Shan QY et al (2017) Liver stiffness measurements with supersonic shear wave elastography in the diagnosis of biliary atresia: a comparative study with grey-scale US. Eur Radiol 27:3474–3484

    PubMed  Google Scholar 

  49. Wang X, Qian L, Jia L et al (2016) Utility of shear wave elastography for differentiating biliary atresia from infantile hepatitis syndrome. J Ultrasound Med 35:1475–1479

  50. Duan X, Peng Y, Liu W et al (2019) Does supersonic shear wave elastography help differentiate biliary atresia from other causes of cholestatic hepatitis in infants less than 90 days old? Compared with grey-scale US. Biomed Res Int 2019:9036362

    PubMed  PubMed Central  Google Scholar 

  51. Dillman JR, DiPaola FW, Smith SJ et al (2019) Prospective assessment of ultrasound shear wave elastography for discriminating biliary atresia from other causes of neonatal cholestasis. J Pediatr 212:60–65

    PubMed  Google Scholar 

  52. Zhou L, Shan Q, Tian W et al (2016) Ultrasound for the diagnosis of biliary atresia: a meta-analysis. AJR Am J Roentgenol 206:W73–W82

    PubMed  Google Scholar 

  53. Yoon HM, Suh CH, Kim JR et al (2017) Diagnostic performance of sonographic features in patients with biliary atresia: a systematic review and meta-analysis. J Ultrasound Med 36:2027–2038

    PubMed  Google Scholar 

  54. Fawaz R, Baumann U, Ekong U et al (2017) Guideline for the evaluation of cholestatic jaundice in infants: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric [sic] Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr 64:154–168

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Paediatric Radiology and Neuroradiology, V. Buzzi Children’s Hospital, 32 Castelvetro St., 20154, Milan, Italy

    Marcello Napolitano

  2. Pediatric Radiology Department, Hôpital Bicêtre, Hôpitaux Universitaire Paris-Sud, Assistance Publique Hôpitaux de Paris, Le Kremlin-Bicêtre, France

    Stéphanie Franchi-Abella

  3. Radiology Department, IRCCS Istituto Giannina Gaslini, Genoa, Italy

    Maria Beatrice Damasio

  4. Section for Paediatric Radiology, Department of Radiology, University Hospital of North Norway, Tromsø, Norway

    Thomas A. Augdal

  5. Department of Pediatric Radiology, Jeanne de Flandre Hospital, CHRU de Lille, Lille Cedex, France

    Fred Efraim Avni

  6. Department of Radiology, Azienda Ospedaliera Universitaria Integrata Verona (AOUI), Verona, Italy

    Costanza Bruno

  7. Department of Radiology, The Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA

    Kassa Darge

  8. Department of Radiology, University Children’s Hospital Ljubljana, Ljubljana, Slovenia

    Damjana Ključevšek

  9. Department of Paediatric Radiology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, The Netherlands

    Annemieke S. Littooij

  10. Serviço de Imagiologia Geral, Hospital de Santa Maria - Centro Hospitalar Universitário Lisboa Norte (CHULN), Lisbon, Portugal

    Luisa Lobo

  11. Section of Pediatric Radiology, Institute of Diagnostic and Interventional Radiology, University Hospital, Jena, Germany

    Hans-Joachim Mentzel

  12. Department of Radiology, Division of Pediatric Radiology, Medical University Graz and University Hospital LKH, Graz, Austria

    Michael Riccabona

  13. Department of Diagnostic Imaging, Sidra Medicine, Doha, Qatar

    Samuel Stafrace

  14. Weill Cornell Medicine, Doha, Qatar

    Samuel Stafrace

  15. Department of Pediatric Radiology, University Hospital of Geneva, Geneva, Switzerland

    Seema Toso

  16. Department of Pediatric Radiology, Medical University of Lublin, Lublin, Poland

    Magdalena Maria Woźniak

  17. Unit of Radiology, IRCCS Policlinico San Donato, San Donato Milanese, Milan, Italy

    Gianni Di Leo & Francesco Sardanelli

  18. Department of Biomedical Sciences for Health, Università degli Studi di Milano, Milan, Italy

    Francesco Sardanelli

  19. Division of Radiology and Nuclear Medicine, Department of Paediatric Radiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway

    Lil-Sofie Ording Müller

  20. Aix Marseille Université, AP-HM, Equipe d’Accueil 3279 - IFR 125, Hôpital Timone Enfants, Service d’Imagerie Pédiatrique et Prénatale, Marseille, France

    Philippe Petit

Authors
  1. Marcello Napolitano
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Stéphanie Franchi-Abella
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Maria Beatrice Damasio
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Thomas A. Augdal
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Fred Efraim Avni
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Costanza Bruno
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Kassa Darge
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Damjana Ključevšek
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Annemieke S. Littooij
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Luisa Lobo
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Hans-Joachim Mentzel
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. Michael Riccabona
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Samuel Stafrace
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. Seema Toso
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. Magdalena Maria Woźniak
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. Gianni Di Leo
    View author publications

    You can also search for this author in PubMed Google Scholar

  17. Francesco Sardanelli
    View author publications

    You can also search for this author in PubMed Google Scholar

  18. Lil-Sofie Ording Müller
    View author publications

    You can also search for this author in PubMed Google Scholar

  19. Philippe Petit
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Marcello Napolitano.

Ethics declarations

Conflicts of interest

None

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Napolitano, M., Franchi-Abella, S., Damasio, M.B. et al. Practical approach to imaging diagnosis of biliary atresia, Part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound. Pediatr Radiol 51, 314–331 (2021). https://doi.org/10.1007/s00247-020-04840-9

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-020-04840-9

Keywords

Search

Navigation