Skip to main content

The favorable prognosis of cystic biliary atresia may be related to early surgery and mild liver pathological changes

Abstract

Objective

The objectives of this study is to compare the prognostic differences between cystic biliary atresia (CBA) and non-CBA, analyze the clinical and liver pathological differences between the two groups, and explore the possible factors that affect the native liver survival of infants with CBA after Kasai portoenterostomy (KPE).

Methods

From 2013 to 2020, 131 infants with BA were admitted to Tianjin Children’s Hospital. A total of 108 infants with BA were included after excluding those who did not undergo surgery after diagnosis (n = 23), including 12 cases of CBA and 96 cases of non-CBA. The clinical data, native liver survival and liver pathology, including liver fibrosis, bile ductular proliferation (BDP), bile plug, and portal area inflammation infiltration of the two study groups were compared.

Results

CBA accounts for 9.16% (12/131) and type I CBA accounts for 6.87% (9/131) of all types of BA. 16.7% (2/12) of CBA were detected prenatally with diagnosis of choledochal cyst (CC). The age at KPE, total bilirubin, direct bilirubin, and total bile acid levels of CBA were significantly lower than those of non-CBA (P = 0.047, P = 0.013, P = 0.009, P = 0.010, respectively). The long and wide diameters of the gallbladder were significantly larger than those of non-CBA (both P < 0.001). The 1-, 3-, and 5-year survival rates of CBA were 83.3%, 71.4%, and 71.4%, respectively, and 56.5%, 32.5%, and 29.8%, respectively, in non-CBA. The difference between the two groups was statistically significant (P = 0.031). The degree of liver fibrosis and bile plug in non-CBA was higher than that of CBA (P = 0.004, P < 0.001, respectively). There was no difference of BDP and inflammation infiltration between the two groups (P = 0.285, P = 0.243, respectively).

Conclusion

CBA is a distinct type different from non-CBA, with different pathological processes, pathological manifestations, and clinical prognosis. The favorable prognosis of CBA may be derived from the early diagnosis, early operation, and mild pathological changes.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

References

  1. 1.

    Ohi R, Chiba T, Endo N (1987) Morphologic studies of the liver and bile ducts in biliary atresia. Acta Paediatr Jpn 29(4):584–589. https://doi.org/10.1111/j.1442-200x.1987.tb02245.x

    CAS  Article  PubMed  Google Scholar 

  2. 2.

    Jiexiong F, Minju L, Hongfeng T, Weizhong G, Shaoyong Y (2003) Clinical and pathological characteristics of cystic lesions of extrahepatic bile duct in neonates. Acta Paediatr 92(10):1183–1189. https://doi.org/10.1080/08035250310005305

    CAS  Article  PubMed  Google Scholar 

  3. 3.

    Caponcelli E, Knisely AS, Davenport M (2008) Cystic biliary atresia: an etiologic and prognostic subgroup. J Pediatr Surg 43(9):1619–1624. https://doi.org/10.1016/j.jpedsurg.2007.12.058

    Article  PubMed  Google Scholar 

  4. 4.

    Bedossa P, Poynard T (1996) An algorithm for the grading of activity in chronic hepatitis C. The METAVIR cooperative study group. Hepatology 24(2):289–293. https://doi.org/10.1002/hep.510240201

    CAS  Article  PubMed  Google Scholar 

  5. 5.

    Lee WS, Looi LM (2009) Usefulness of a scoring system in the interpretation of histology in neonatal cholestasis. World J Gastroenterol 15(42):5326–5333. https://doi.org/10.3748/wjg.15.5326

    Article  PubMed  PubMed Central  Google Scholar 

  6. 6.

    El-Araby HA, Saber MA, Radwan NM, Taie DM, Adawy NM, Sira AM (2021) Temporal histopathological changes in biliary atresia: a perspective for rapid fibrosis progression. Ann Hepatol 21:100263. https://doi.org/10.1016/j.aohep.2020.09.007

    CAS  Article  PubMed  Google Scholar 

  7. 7.

    Chen G, Xue P, Zheng S, Chen L, Ma Y (2015) A pathological scoring system in the diagnosis and judgment of prognosis of biliary atresia. J Pediatr Surg 50(12):2119–2123. https://doi.org/10.1016/j.jpedsurg.2015.08.041

    Article  PubMed  Google Scholar 

  8. 8.

    Muise AM, Turner D, Wine E, Kim P, Marcon M, Ling SC (2006) Biliary atresia with choledochal cyst: implications for classification. Clin Gastroenterol Hepatol 4(11):1411–1414. https://doi.org/10.1016/j.cgh.2006.07.005

    Article  PubMed  Google Scholar 

  9. 9.

    Napolitano M, Franchi-Abella S, Damasio MB, Augdal TA, Avni FE, Bruno C, Darge K, Ključevšek D, Littooij AS, Lobo L, Mentzel HJ, Riccabona M, Stafrace S, Toso S, Woźniak MM, Di Leo G, Sardanelli F, Ording Müller LS, Petit P (2021) Practical approach to imaging diagnosis of biliary atresia, part 1: prenatal ultrasound and magnetic resonance imaging, and postnatal ultrasound. Pediatr Radiol 51(2):314–331. https://doi.org/10.1007/s00247-020-04840-9

    Article  PubMed  Google Scholar 

  10. 10.

    Zhou LY, Guan BY, Li L, Xu ZF, Dai CP, Wang W, Xia HM, Xie XY (2012) Objective differential characteristics of cystic biliary atresia and choledochal cysts in neonates and young infants: sonographic findings. J Ultrasound Med 31(6):833–841. https://doi.org/10.7863/jum.2012.31.6.833

    Article  PubMed  Google Scholar 

  11. 11.

    Shin HJ, Yoon H, Han SJ, Ihn K, Koh H, Kwon JY, Lee MJ (2021) Key imaging features for differentiating cystic biliary atresia from choledochal cyst: prenatal ultrasonography and postnatal ultrasonography and MRI. Ultrasonography 40(2):301–311. https://doi.org/10.14366/usg.20061

    Article  PubMed  Google Scholar 

  12. 12.

    Zhou W, Yang Y, Yu C, Liu J, Duan X, Weng Z, Chen D, Liang Q, Fang Q, Zhou J, Ju H, Luo Z, Guo W, Ma X, Xie X, Wang R, Zhou L (2021) Ensembled deep learning model outperforms human experts in diagnosing biliary atresia from sonographic gallbladder images. Nat Commun 12(1):1259. https://doi.org/10.1038/s41467-021-21466-z

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  13. 13.

    Tanaka H, Sasaki H, Wada M, Sato T, Kazama T, Nishi K, Kudo H, Nakamura M, Nio M (2015) Postnatal management of prenatally diagnosed biliary cystic malformation. J Pediatr Surg 50(4):507–510. https://doi.org/10.1016/j.jpedsurg.2014.08.002

    Article  PubMed  Google Scholar 

  14. 14.

    Yu P, Dong N, Pan YK, Li L (2021) Ultrasonography is useful in differentiating between cystic biliary atresia and choledochal cyst. Pediatr Surg Int 37(6):731–736. https://doi.org/10.1007/s00383-021-04886-2

    Article  PubMed  Google Scholar 

  15. 15.

    Sasaki H, Nio M, Ando H, Kitagawa H, Kubota M, Suzuki T, Taguchi T, Hashimoto T (2021) Anatomical patterns of biliary atresia including hepatic radicles at the porta hepatis influence short- and long-term prognoses. J Hepatobiliary Pancreat Sci. https://doi.org/10.1002/jhbp.989

    Article  PubMed  Google Scholar 

  16. 16.

    Faure A, Hery G, Colavolpe N, Bevilacqua C, Guys JM, De Lagausie P (2015) Laparoscopic cystojejunostomy for type I cystic biliary atresia in children. J Minim Access Surg 11(4):263–266. https://doi.org/10.4103/0972-9941.158151

    Article  PubMed  PubMed Central  Google Scholar 

  17. 17.

    Sun S, Zheng S, Lu X, Chen G, Ma Y, Chen L, Dong K (2018) Clinical and pathological features of patients with biliary atresia who survived for more than 5 years with native liver. Pediatr Surg Int 34(4):381–386. https://doi.org/10.1007/s00383-018-4231-7

    Article  PubMed  Google Scholar 

  18. 18.

    Ihn K, Ho IG, Lee JH, Na Y, Lee D, Han SJ (2020) Comparison of the outcomes of biliary atresia with cystic degeneration and isolated biliary atresia: a matched-pair analysis. J Pediatr Surg 55(10):2177–2182. https://doi.org/10.1016/j.jpedsurg.2020.02.054

    Article  PubMed  Google Scholar 

  19. 19.

    Takahashi Y, Matsuura T, Saeki I, Zaizen Y, Taguchi T (2009) Excellent long-term outcome of hepaticojejunostomy for biliary atresia with a hilar cyst. J Pediatr Surg 44(12):2312–2315. https://doi.org/10.1016/j.jpedsurg.2009.07.051

    Article  PubMed  Google Scholar 

  20. 20.

    Shan QY, Liu BX, Zhong ZH, Chen HD, Guo Y, Xie XY, Zhou WY, Jiang H, Zhou LY (2021) The prognosis of type III biliary atresia with hilar cyst. Indian J Pediatr 88(7):650–655. https://doi.org/10.1007/s12098-020-03561-z

    Article  PubMed  Google Scholar 

  21. 21.

    Rhu J, Jung SM, Choe YH, Seo JM, Lee SK (2012) PELD score and age as a prognostic index of biliary atresia patients undergoing Kasai portoenterostomy. Pediatr Surg Int 28(4):385–391. https://doi.org/10.1007/s00383-012-3060-3

    Article  PubMed  Google Scholar 

  22. 22.

    Wong KK, Chung PH, Chan IH, Lan LC, Tam PK (2010) Performing Kasai portoenterostomy beyond 60 days of life is not necessarily associated with a worse outcome. J Pediatr Gastroenterol Nutr 51(5):631–634. https://doi.org/10.1097/MPG.0b013e3181e8e194

    CAS  Article  PubMed  Google Scholar 

  23. 23.

    Chen G, Zheng S, Sun S, Xiao X, Ma Y, Shen W, Chen L, Song Z (2012) Early surgical outcomes and pathological scoring values of older infants (≥ 90 d old) with biliary atresia. J Pediatr Surg 47(12):2184–2188. https://doi.org/10.1016/j.jpedsurg.2012.09.002

    Article  PubMed  Google Scholar 

  24. 24.

    Ge L, Zhan J, Gao W, Zhao S, Xu X, Dou R (2020) Relevant factors for early liver transplantation after Kasai portoenterostomy. BMC Pediatr 20(1):484. https://doi.org/10.1186/s12887-020-02355-8

    Article  PubMed  PubMed Central  Google Scholar 

  25. 25.

    Mashima M, Tanaka H, Numoto A, Kubo H, Shimono R, Kusaka T, Itoh S, Hata T (2013) Antenatal three-dimensional sonographic features of fetal biliary atresia. J Med Ultrason (2001) 40(3):279–281. https://doi.org/10.1007/s10396-012-0425-0

    Article  Google Scholar 

  26. 26.

    Mahalik SK, Mitra S, Patra S, Das K (2019) Cystic biliary atresia or atretic choledochal cyst: a continuum in infantile obstructive cholangiopathy. Fetal Pediatr Pathol 38(6):477–483. https://doi.org/10.1080/15513815.2019.1627621

    CAS  Article  PubMed  Google Scholar 

  27. 27.

    Masumoto K, Kai H, Oka Y, Otake R, Yoshizato T, Miyamoto S, Hirose S, Hamasaki M, Nabeshima K, Iwasaki A (2011) A case of cystic biliary atresia with an antenatally detected cyst: the possibility of changing from a correctable type with a cystic lesion (I cyst) to an uncorrectable one (IIId). Pediatr Surg Int 27(1):99–102. https://doi.org/10.1007/s00383-010-2715-1

    Article  PubMed  Google Scholar 

  28. 28.

    Fujishiro J, Iwanaka T, Arai M, Kawashima H, Kudou S, Imaizumi S, Hirukawa S, Inasaka O (2005) Disappearing cyst of the hepatic hilum in uncorrectable biliary atresia. Pediatr Surg Int 21(2):116–118. https://doi.org/10.1007/s00383-004-1352-y

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

This study was funded by Tianjin Health Bureau special grant (14KG129), Xinjiang Uygur Autonomous Region Science Foundation Projects (2019D01A12), and Hospital-level project of Tianjin Children’s Hospital (Y2020002).

Author information

Affiliations

Authors

Corresponding author

Correspondence to Zhan Jianghua.

Ethics declarations

Conflict of interest

No authors have a conflict of interest or financial ties to disclose.

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

Verify currency and authenticity via CrossMark

Cite this article

Qipeng, Z., Fang, Y., Yilin, Z. et al. The favorable prognosis of cystic biliary atresia may be related to early surgery and mild liver pathological changes. Pediatr Surg Int (2021). https://doi.org/10.1007/s00383-021-05030-w

Download citation

Keywords

  • Biliary atresia
  • Cyst
  • Native liver survival
  • Hepatic fibrosis