Skip to main content
Log in

Early differential diagnosis methods of biliary atresia: a meta-analysis

  • Review Article
  • Published:
Pediatric Surgery International Aims and scope Submit manuscript

Abstract

Purpose

To evaluate the accuracy of early differential diagnosis methods of biliary atresia in patients with infantile cholestasis.

Methods

We searched PubMed, EMBASE and the Web of Science databases for articles evaluated the early differential diagnosis methods of biliary atresia. The methodological quality of each study was assessed with version 2 of the Quality Assessment of Diagnostic Accuracy Studies tool. Two reviewers extracted data independently. Pooled sensitivity, specificity, positive likelihood ratio (LR +), negative likelihood ratio (LR −), diagnostic odds ratio (DOR) with 95% CIs were calculated to assess each diagnosis method.

Results

A total of 38 articles were included. Summary sensitivity and specificity were 77% (95% CI 74–80%) and 93% (95% CI 91–94%), respectively, for B-US in 23 studies; 96% (95% CI 92–98%) and 58% (95% CI 51–65%), respectively, for MRCP in five studies; 87% (95% CI 82–91%) and 78% (95% CI 74–82%), respectively, for acholic stool in seven studies; 84% (95% CI 78–89%) and 97% (95% CI 97–98%), respectively, for serum liver function test in seven studies; 96% (95% CI 94–97%) and 73% (95% CI 70–76%), respectively, for hepatobiliary scintigraphy in 18 studies; 98% (95% CI 96–99%) and 93% (95% CI 89–95%), respectively, for percutaneous liver biopsy in 11 studies.

Conclusion

The accuracy rate of percutaneous liver biopsy is better than all of the noninvasive methods. Take into consideration the advantages and disadvantages of the six methods, combination of multidisciplinary noninvasive diagnosis methods is the first choice for differential diagnosis of BA from other causes of neonatal cholestasis.

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

Access this article

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

Instant access to the full article PDF.

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
Fig. 17
Fig. 18
Fig. 19

Similar content being viewed by others

References

  1. Sokol RJ, Mack C, Narkewicz MR et al (2003) Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr 37: 4–21

    Article  PubMed  Google Scholar 

  2. Wildhaber BE, Majno P, Mayr J et al (2008) Biliary atresia: Swiss national study, 1994–2004. J Pediatr Gastroenterol Nutr 46:299–307

    Article  PubMed  Google Scholar 

  3. Whiting PF, Rutjes AW, Westwood ME et al (2011) Quadas-2: a revised tool for the quality assessment of diagnostic accuracy studies. Ann Intern Med 155:529–536

    Article  PubMed  Google Scholar 

  4. Spivak W, Sarkar S, Winter D et al (1987) Diagnostic utility of hepatobiliary scintigraphy with 99mtc-disida in neonatal cholestasis. J Pediatr 110: 855–861

    Article  CAS  PubMed  Google Scholar 

  5. Tolia V, Dubois RS, Kagalwalla A, at el (1986) Comparison of radionuclear scintigraphy and liver biopsy in the evaluation of neonatal cholestasis. J Pediatr Gastroenterol Nutr 5:30–34

    Article  CAS  PubMed  Google Scholar 

  6. Cox KL, Stadalnik RC, Mcgahan JP et al (1987) Hepatobiliary scintigraphy with technetium-99m disofenin in the evaluation of neonatal cholestasis. J Pediatr Gastroenterol Nutr 6:885–891

    Article  CAS  PubMed  Google Scholar 

  7. Park WH, Choi SO, Lee HJ et al (1997) A new diagnostic approach to biliary atresia with emphasis on the ultrasonographic triangular cord sign: comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis. J Pediatr Surg 32:1555–1559

    Article  CAS  PubMed  Google Scholar 

  8. Lee CH, Wang P, Lee TT et al (2000) The significance of functioning gallbladder visualization on hepatobiliary scintigraphy in infants with persistent jaundice. J Nucl Med 41:1209–1213

    CAS  PubMed  Google Scholar 

  9. Tan KA, Phua KB, Ooi BC et al (2000) Making the diagnosis of biliary atresia using the triangular cord sign and gallbladder length. Pediatr Radiol 30:69–73

    Article  PubMed  Google Scholar 

  10. Farrant P, Meire HB, Mieli-Vergani G (2001) Improved diagnosis of extraheptic biliary atresia by high frequency ultrasound of the gall bladder. Br J Radiol 74:952–954

    Article  CAS  PubMed  Google Scholar 

  11. 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

    Article  PubMed  Google Scholar 

  12. Han SJ, Kim MJ, Han A et al (2002) Magnetic resonance cholangiography for the diagnosis of biliary atresia. J Pediatr Surg 37:599–604

    Article  PubMed  Google Scholar 

  13. Azuma T, Nakamura T, Nakahira M et al (2003) Pre-operative ultrasonographic diagnosis of biliary atresia—with reference to the presence or absence of the extrahepatic bile duct. Pediatr Surg Int 19:475–477

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  15. Visrutaratna P, Wongsawasdi L, Lerttumnongtum P et al (2003) Triangular cord sign and ultrasound features of the gall bladder in infants with biliary atresia. Austr Radio 47:252–256

    Article  Google Scholar 

  16. Ryeom HK, Choe BH, Kim JY et al (2005) Biliary atresia: feasibility of mangafodipir trisodium-enhanced MR cholangiography for evaluation. Radiology 235:250–258

    Article  PubMed  Google Scholar 

  17. Dehghani SM, Haghighat M, Imanieh MH et al (2006) Comparison of different diagnostic methods in infants with cholestasis. World J Gastroenterol 12:5893–5896

    Article  PubMed  PubMed Central  Google Scholar 

  18. Hu Y, Huang Z, Xia L (2006) MR cholangiography and dynamic examination of duodenal fluid in the differential diagnosis between extrahepatic biliary atresia and infantile hepatitis syndrome. J Huazhong Univ Sci Technol Med Sci 26:725–727

    Article  PubMed  Google Scholar 

  19. Humphrey T, Stringer M (2007) Biliary atresia: US diagnosis. Radiology 244:845–851

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  22. Wongsawasdi L, Ukarapol N, Visrutaratna P et al (2008) Diagnostic evaluation of infantile cholestasis. J Med Assoc Thai 91:345–349

    PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  24. Poddar U, Thapa BR, Das A et al (2009) Neonatal cholestasis: differentiation of biliary atresia from neonatal hepatitis in a developing country. Acta Paediatr 98:1260–1264

    Article  PubMed  Google Scholar 

  25. Rouzrokh M, Sobhiyeh MR, Heibatollahi M (2009) The sensitvity, specificity, positive and negative predictive values of stool color test, triangular cord sign and hepatobiliary scintigraphy in diagnosis of infantile biliary atresia. Iran Red Crescent Med J 11:425–430

    Google Scholar 

  26. Yang JG, Ma DQ, Peng Y et al (2009) Comparison of different diagnostic methods for differentiating biliary atresia from idiopathic neonatal hepatitis. Clin Imaging 33:439–446

    Article  PubMed  Google Scholar 

  27. Liu SX, Huang ZH (2010) The value of radionuclide hepatobiliary scintigraphy in combination with determination of bilirubin from duodenal drainage in differential diagnosis of infantile persistent jaundice. Front Med China 4:342–345

    Article  PubMed  Google Scholar 

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

    Article  PubMed  PubMed Central  Google Scholar 

  29. Jensen MK, Biank VF, Moe DC et al (2012) HIDA, percutaneous transhepatic cholecysto-cholangiography and liver biopsy in infants with persistent jaundice: can a combination of ptcc and liver biopsy reduce unnecessary laparotomy? Pediatr Radiol 42:32–39

    Article  PubMed  Google Scholar 

  30. Elguindi 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

    Article  Google Scholar 

  31. Jiang LP, Chen YC, Ding L et al (2013) The diagnostic value of high-frequency ultrasonography in biliary atresia. Hepatobiliary Pancreat Dis Int 12:415–422

    Article  PubMed  Google Scholar 

  32. Kwatra N, Shalabyrana E, Narayanan S et al (2013) Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center. Pediatr Radiol 43:1365–1375

    Article  PubMed  Google Scholar 

  33. Boskovic A, Kitic I, Prokic D et al (2014) Predictive value of hepatic ultrasound, liver biopsy, and duodenal tube test in the diagnosis of extrahepatic biliary atresia in serbian infants. Turk J Gastroenterol 25:170–174

    Article  PubMed  Google Scholar 

  34. 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

    Article  PubMed  Google Scholar 

  35. Liu B, Cai J, Xu Y et al (2014) Three-dimensional magnetic resonance cholangiopancreatography for the diagnosis of biliary atresia in infants and neonates. Plos One 9:e88268

    Article  PubMed  PubMed Central  Google Scholar 

  36. Guan YX, Chen Q, Wan SH et al (2015) Effect of different time phases of radionuclide hepatobiliary scintigraphy on the differential diagnosis of congenital biliary atresia. Genet Mol Res 14:3862–3868

    Article  CAS  PubMed  Google Scholar 

  37. Jancelewicz T, Barmherzig R, Chung CT et al (2015) A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice. J Pediatr Surg 50:363–370

    Article  PubMed  Google Scholar 

  38. 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

    Article  PubMed  PubMed Central  Google Scholar 

  39. Brittain JM, Kvist N, Johansen LS et al (2016) Hepatobiliary scintigraphy for early diagnosis of biliary atresia. Dan Med J 63:A5253

    PubMed  Google Scholar 

  40. Mandana R, Lida S, Shoa HJ et al (2016) Diagnostic value of anti-smooth muscle antibodies and liver enzymes in differentiation of extrahepatic biliary atresia and idiopathic neonatal hepatitis. Afr J Paediatr Surg 13:63–68

    Article  Google Scholar 

  41. Shen Z, Zheng S, Dong R et al (2016) Saturation of stool color in HSV color model is a promising objective parameter for screening biliary atresia. J Pediatr Surg 51:2091–2094

    Article  PubMed  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

    Article  PubMed  Google Scholar 

  43. Sung S, Jeon TY, Yoo SY et al (2016) Incremental value of MR cholangiopancreatography in diagnosis of biliary atresia. Plos One 11:e0158132

    Article  PubMed  PubMed Central  Google Scholar 

  44. Moyer V, Freese DK, Whitington PF et al (2004) Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for pediatric gastroenterology, hepatology and nutrition. J Pediatr Gastroenterol Nutr 39:115–128

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

We thank Patrick Chung and Professor Vincent for their advices on this study.

Funding

This study was funded by the National Science Foundation of China (Grant Number 81570471) and the Tianjin Health Bureau special grant (Grant Number 14KG129).

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Jianghua Zhan.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Research involving human participants and/or animals

This article does not contain any studies with human participants or animals performed by any of the authors.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Wang, L., Yang, Y., Chen, Y. et al. Early differential diagnosis methods of biliary atresia: a meta-analysis. Pediatr Surg Int 34, 363–380 (2018). https://doi.org/10.1007/s00383-018-4229-1

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00383-018-4229-1

Keywords

Navigation