World Journal of Pediatrics

, Volume 14, Issue 2, pp 191–196 | Cite as

Does time taken to achieve jaundice-clearance influence survival of the native liver in post-Kasai biliary atresia?

  • Hideaki Nakajima
  • Hiroyuki KogaEmail author
  • Manabu Okawada
  • Hiroki Nakamura
  • Geoffrey James Lane
  • Atsuyuki Yamataka
Original Article



We reviewed the time taken for post-portoenterostomy (PE) biliary atresia (BA) patients to obtain jaundice-clearance (total bilirubin ≤ 1.2 mg/dL; JC) post-PE to determine if JC time (JCT) is prognostic for survival of the native liver (SNL).


The subjects were 66 BA patients treated with PE at our institute between 1989, the year when liver transplantation (LTx) became available in Japan, and 2014. JCT was used to create three groups (≤ 30 days: n = 14; 31–60 days: n = 31; ≥ 61 days: n = 21). Medical records were reviewed retrospectively to evaluate: age at onset of symptoms, duration of symptoms pre-PE, age and weight at PE, serum liver function tests, incidence of cholangitis, and micro-bile duct size at PE.


Age at onset of symptoms, age and weight at PE, duration of symptoms pre-PE, and micro-bile duct size were similar for all patients in all three groups. JCT and SNL appeared to correlate because preoperative total bilirubin (7.1, 9.6, 10.2 mg/dL; P < 0.05) was significantly lower in the JCT ≤ 30 days group (P < 0.05) while there was a significant decrease in SNL (P < 0.03) and a significant increase in LTx (P < 0.01) in the JCT ≥ 61 days group. All LTx subjects who achieved JC were found to have developed cholangitis within 3 months of PE.


During the follow-up of post-PE subjects, longer JCT and cholangitis occurrence within 3 months of PE would appear to be negative prognostic factors for SNL while preoperative total bilirubin would appear to be a positive prognostic factor for SNL.


Biliary atresia Jaundice free Liver transplantation Native liver survival Portoenterostomy 


Author contributions

HN contributed to the collection of data, and wrote the first version of the paper. HK contributed to the design, data analysis of this study, and wrote the first version of the paper. MO and HN contributed to the collection of data. GJL wrote the first version of the paper. AY contributed to the design and data analysis of this study. All authors approved the final version of the manuscript.


No funding was acquired or utilized for this study.

Compliance with ethical standards

Ethical approval

This study was approved by the Juntendo University School of Medicine Institutional Review Board and complies with the Helsinki Declaration of 1975 (revised 1983).

Conflict of interest

The authors declare no conflict of interest.


  1. 1.
    Hartley JL, Davenport M, Kelly DA. Biliary atresia. Lancet. 2009;374:1704–13.CrossRefPubMedGoogle Scholar
  2. 2.
    Howard ER, MacLean G, Nio M, Donaldson N, Singer J, Ohi R. Survival patterns in biliary atresia and comparison of quality of life of long-term survivors in Japan and England. J Pediatr Surg. 2001;36:892–7.CrossRefPubMedGoogle Scholar
  3. 3.
    Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O. Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology. 2005;41:366–71.CrossRefPubMedGoogle Scholar
  4. 4.
    Koga H, Wada M, Nakamura H, Miyano G, Okawada M, Lane GJ, et al. Factors influencing jaundice-free survival with the native liver in post-portoenterostomy biliary atresia patients: results from a single institution. J Pediatr Surg. 2013;48:2368–72.CrossRefPubMedGoogle Scholar
  5. 5.
    Nakamura H, Koga H, Wada M, Miyano G, Dizon R, Kato Y, et al. Reappraising the portoenterostomy procedure according to sound physiologic/anatomic principles enhances postoperative jaundice clearance in biliary atresia. Pediatr Surg Int. 2012;28:205–9.CrossRefPubMedGoogle Scholar
  6. 6.
    Ohhama Y, Shinkai M, Fujita S, Nishi T, Yamamoto H. Early prediction of long-term survival and the timing of liver transplantation after the Kasai operation. J Pediatr Surg. 2000;35:1031–4.CrossRefPubMedGoogle Scholar
  7. 7.
    Superina R, Magee JC, Brandt ML, Healey PJ, Tiao G, Ryckman F, et al. The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Ann Surg. 2011;254:577–85.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Oh M, Hobeldin M, Chen T, Thomas DW. Atkinson JB. The Kasai procedure in the treatment of biliary atresia. J Pediatr Surg. 1995;30:1077–80.CrossRefPubMedGoogle Scholar
  9. 9.
    Nio M, Wada M, Sasaki H, Tanaka H, Okamura A. Risk factors affecting late-presenting liver failure in adult patients with biliary atresia. J Pediatr Surg. 2012;47:2179–83.CrossRefPubMedGoogle Scholar
  10. 10.
    Rodeck B, Becker AC, Gratz KF, Petersen C. Early predictors of success of Kasai operation in children with biliary atresia. Eur J Pediatr Surg. 2007;7:308–12.CrossRefGoogle Scholar
  11. 11.
    Shneider BL, Brown MB, Haber B, Whitington PF, Scwarz K, Squirres R, et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr. 2006;148:467–74.CrossRefPubMedGoogle Scholar
  12. 12.
    Vazquez-Estevez J, Stewart B, Shikes RH, Hall RJ, Lilly JR. Biliary atresia: early determination of prognosis. J Pediatr Surg. 1989;24:48–50; discussion 50–1.CrossRefPubMedGoogle Scholar
  13. 13.
    Goda T, Kawahara H, Kubota A, Hirano K, Umeda S, Tani S, et al. The most reliable early predictors of outcome in patients with biliary atresia after Kasai’s operation. J Pediatr Surg. 2013;48:2373–7.CrossRefPubMedGoogle Scholar
  14. 14.
    Iwatsuki S, Shaw BW Jr, Starzl TE. Liver transplantation for biliary atresia. World J Surg. 1984;8:51–6.CrossRefPubMedPubMedCentralGoogle Scholar
  15. 15.
    Kubota A, Okada A, Fukui Y, Kawahara H, Imura K. Kamata S. Indocyanine green test is a reliable indicator of postoperative liver function in biliary atresia. J Pediatr Gastroenterol Nutr. 1993;16:61–5.CrossRefPubMedGoogle Scholar
  16. 16.
    Altman RP, Lilly JR, Greenfeld J, Weinberg A, van Leeuwen K, Flanigan L. A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers. Ann Surg. 1997;226:348–53.CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Azarow KS, Phillips MJ, Sandler AD, Hagerstrand I, Superina RA. Biliary atresia: should all patients undergo a portoenterostomy? J Pediatr Surg. 1997;32:168–72.CrossRefPubMedGoogle Scholar
  18. 18.
    Nio M, Ohi R, Miyano T, Saeki T, Shiraki K, Tanaka K, et al. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg. 2003;38:997–1000.CrossRefPubMedGoogle Scholar
  19. 19.
    Shinkai M, Ohhama Y, Take H, Kitagawa N, Kudo H, Mochizuki K, et al. Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: > 20-year experience at a children’s hospital. J Pediatr Gastroenterol Nutr. 2009;48:443–50.CrossRefPubMedGoogle Scholar
  20. 20.
    Hung PY, Chen CC, Chen WJ, Lai HS, Hsu WM, Lee PH, et al. Long-term prognosis of patients with biliary atresia: a 25 year summary. J Pediatr Gastroenterol Nutr. 2006;42:190–5.CrossRefPubMedGoogle Scholar
  21. 21.
    Bu LN, Chen HL, Ni YH, Peng S, Jeng YM, Lai HS, et al. Multiple intrahepatic biliary cysts in children with biliary atresia. J Pediatr Surg. 2002;37:1183–7.CrossRefPubMedGoogle Scholar
  22. 22.
    Wu ET, Chen HL, Ni YH, Lee PI, Hsu HY, Lai HS, et al. Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome. Pediatr Surg Int. 2001;17:390–5.CrossRefPubMedGoogle Scholar

Copyright information

© Children's Hospital, Zhejiang University School of Medicine 2018

Authors and Affiliations

  • Hideaki Nakajima
    • 1
  • Hiroyuki Koga
    • 1
    Email author
  • Manabu Okawada
    • 1
  • Hiroki Nakamura
    • 1
  • Geoffrey James Lane
    • 1
  • Atsuyuki Yamataka
    • 1
  1. 1.Department of Pediatric General and Urogenital SurgeryJuntendo University School of MedicineTokyoJapan

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