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Effects of age at Kasai portoenterostomy on the surgical outcome: a review of the literature

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Abstract

The efficacy of early Kasai portoenterostomy has been repeatedly reported. However, the optimal age for performing this procedure remains controversial. This article reviews the literature on the age of patients at the time of Kasai portoenterostomy and its utility as a prognostic indicator. The age at the time of surgery is a known predictor of outcome; however, its exact predictive value in this context is unclear. Multicenter studies involving large volumes of data have tended to show advantages of early Kasai portoenterostomy, and there is no clear evidence to recommend any delay in the timing of surgery. At present, a reasonable strategy would be to perform a Kasai portoenterostomy as early as possible. The stool color card system has recently been implemented in Japan as part of a nationwide screening program, and it is expected to work well based on the early reports. However, efforts to identify an optimal screening system for ensuring the earliest diagnosis of biliary atresia should continue. An early diagnosis of biliary atresia is difficult, and global efforts are required to improve the early diagnosis rates.

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References

  1. Kasai M. Hepatic portoenterostomy and its modification. In: Kasai M, et al., editors. Cholestasis in infancy. Tokyo: University of Tokyo Press; 1980. p. 337–44.

    Google Scholar 

  2. Ohi R, Hanamatsu M, Mochizuki I, Chiba T, Kasai M. Progress in the treatment of biliary atresia. WJS. 1985;9:285–93.

    Article  CAS  Google Scholar 

  3. Lally KP, Kanegaye J, Matsumura M, Rosenthal P, Sinatra F, Atkinson JB. Perioperative factors affecting the outcome following repair of biliary atresia. Pediatrics. 1989;83:723–6.

    CAS  PubMed  Google Scholar 

  4. Stewart BA, Hall RJ, Karrer FM, Lilly JR. Long-term survival after Kasai’s operation for biliary atresia. Pediatr Surg Int. 1990;5:87–90.

    Article  Google Scholar 

  5. Karrer FM, Lilly JR, Stewart BA, Hall RJ. Biliary atresia registry, 1976 to 1989. J Pediatr Surg. 1990;25:1076–80.

    Article  CAS  PubMed  Google Scholar 

  6. Ohi R, Ibrahim M. Biliary atresia. Semin Pediatr Surg. 1992;1:115–24.

    CAS  PubMed  Google Scholar 

  7. 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.

    Article  CAS  PubMed  Google Scholar 

  8. Karrer FM, Hall RJ, Stewart BA, Lilly JR. Congenital biliary tract disease. Surg Clin North Am. 1990;70:1403–18.

    CAS  PubMed  Google Scholar 

  9. Davenport M, Kerkar N, Mieli-Vergani G, Mowat AP, Howard ER. Biliary atresia: the King’s College Hospital experience (1974–1995). J Pediatr Surg. 1997;32:479–85.

    Article  CAS  PubMed  Google Scholar 

  10. McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet. 2000;355:25–9.

    Article  CAS  PubMed  Google Scholar 

  11. Schoen BT, Lee H, Sullivan K, Ricketts RR. The Kasai portoenterostomy: when is it too late? J Pediatr Surg. 2001;36:97–9.

    Article  CAS  PubMed  Google Scholar 

  12. Wildhaber BE, Coran AG, Drongowski RA, Hirschl RB, Geiger JD, Lelli JL, et al. The Kasai portoenterostomy for biliary atresia: a review of a 27-year experience with 81 patients. J Pediatr Surg. 2003;38:1480–5.

    Article  PubMed  Google Scholar 

  13. 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.

    Article  PubMed  Google Scholar 

  14. Shneider BL, Brown MB, Haber B, Whitington PF, Schwarz K, Squires R, et al. Biliary Atresia Research Consortium. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr. 2006;148:467–74.

    Article  PubMed  Google Scholar 

  15. Chen G, Zheng S, Sun S, Xiao X, Ma Y, Shen W, et al. Early surgical outcomes and pathological scoring values of older infants (≥90 days old) with biliary atresia. J Pediatr Surg. 2012;47:2184–8.

    Article  PubMed  Google Scholar 

  16. Japanese biliary atresia society. Japanese biliary atresia registry 2011. Jpn J Pediatr Surg. 2013;49:277–89.

    Google Scholar 

  17. Schreiber RA, Barker CC, Roberts EA, Martin SR, Alvarez F, Smith L, et al. Canadian Pediatric Hepatology Research Group. Biliary atresia: the Canadian experience. J Pediatr. 2007;151:659–65 665.e1.

    Article  PubMed  Google Scholar 

  18. Serinet MO, Broué P, Jacquemin E, Lachaux A, Sarles J, Gottrand F, et al. Management of patients with biliary atresia in France: results of a decentralized policy 1986–2002. Hepatology. 2006;44:75–84.

    Article  PubMed  Google Scholar 

  19. Wildhaber BE, Majno P, Mayr J, Zachariou Z, Hohlfeld J, Schwoebel M, et al. Biliary atresia: Swiss national study, 1994–2004. J Pediatr Gastroenterol Nutr. 2008;46:299–307.

    Article  PubMed  Google Scholar 

  20. Carvalho Ed, Santos JL, Silveira TR, Kieling CO, Silva LR, Porta G, et al. Grupo de Estudos em Hepatologia Pediátrica do Brasil. Biliary atresia: the Brazilian experience. J Pediatr (Rio J). 2010;86:473–9.

  21. Leonhardt J, Kuebler JF, Leute PJ, Turowski C, Becker T, Pfister ED, et al. Biliary atresia: lessons learned from the voluntary German registry. Eur J Pediatr Surg. 2011;21:82–7.

    Article  CAS  PubMed  Google Scholar 

  22. Davenport M, Ville De, de Goyet J, Stringer MD, Mieli-Vergani G, Kelly DA, McClean P, et al. Seamless management of biliary atresia in England and Wales (1999–2002). Lancet. 2004;363:1354–7.

    Article  PubMed  Google Scholar 

  23. 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.

    Article  PubMed  Google Scholar 

  24. 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.

    Article  PubMed  Google Scholar 

  25. Nio M, Sasaki H, Wada M, Kazama T, Nishi K, Tanaka H. Impact of age at Kasai operation on short- and long-term outcomes of type III biliary atresia at a single institution. J Pediatr Surg. 2010;45:2361–3.

    Article  PubMed  Google Scholar 

  26. 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.

    Article  PubMed  Google Scholar 

  27. Ibrahim M, Ohi R, Chiba T, Nio M. Indications and results of reoperation for biliary atresia. In: Ohi R, editor. Biliary Atresia. ICOM Associates INC; 1991. p. 96–100.

  28. Nio M, Sasaki H, Tanaka H, Okamura A. Redo surgery for biliary atresia. Pediatri Surg Int. 2013;29:989–93.

    Article  Google Scholar 

  29. Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop. Hepatology. 2007;46:566–81.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  30. Matsui A, Dodoriki M. Screening for biliary atresia. Lancet. 1995;345:1181.

    Article  CAS  PubMed  Google Scholar 

  31. Lien TH, Chang MH, Wu JF, Chen HL, Lee HC, Chen AC, et al. Taiwan Infant Stool Color Card Study Group. Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan. Hepatology. 2011;53:202–8.

    Article  PubMed  Google Scholar 

  32. Wildhaber BE. Screening for biliary atresia: Swiss stool color card. Hepatology. 2011;54:367–8 author reply 369.

    Article  PubMed  Google Scholar 

  33. Suzuki M, Muraji T, Obatake M, Nio M, Ito K, Suzuki K, et al. Urinary sulfated bile acid analysis for the early detection of biliary atresia in infants. Pediatr Int. 2011;53:497–500.

    Article  CAS  PubMed  Google Scholar 

  34. Powell JE, Keffler S, Kelly DA, Green A. Population screening for neonatal liver disease: potential for a community-based programme. J Med Screen. 2003;10:112–6.

    Article  CAS  PubMed  Google Scholar 

  35. Mushtaq I, Logan S, Morris M, Johnson AW, Wade AM, Kelly D, et al. Screening of newborn infants for cholestatic hepatobiliary disease with tandem mass spectrometry. BMJ 1999;21;319(7208):471–7. Erratum in: BMJ 1999;6;319(7219):1253.

  36. Gustafsson J, Alvelius G, Björkhem I, Nemeth A. Bile acid metabolism in extrahepatic biliary atresia: lithocholic acid in stored dried blood collected at neonatal screening. Ups J Med Sci. 2006;111:131–6.

    Article  PubMed  Google Scholar 

  37. Song Z, Dong R, Fan Y, Zheng S. Identification of serum protein biomarkers in biliary atresia by mass spectrometry and enzyme-linked immunosorbent assay. J Pediatr Gastroenterol Nutr. 2012;55:370–5.

    Article  CAS  PubMed  Google Scholar 

  38. Lau TT, Ho LW, Wang DA. Hepatogenesis of murine induced pluripotent stem cells in 3D micro-cavitary hydrogel system for liver regeneration. Biomaterials. 2013;34:6659–69.

    Article  CAS  PubMed  Google Scholar 

  39. Raval MV, Dzakovic A, Bentrem DJ, Reynolds M, Superina R. Trends in age for hepatoportoenterostomy in the United States. Surgery. 2010;148:785–92.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric Surgery, Tohoku University Graduate School of Medicine, 1-1 Seiryomachi, Aobaku, Sendai, 980-8574, Japan

    Masaki Nio, Motoshi Wada, Hideyuki Sasaki & Hiromu Tanaka

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  1. Masaki Nio
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  2. Motoshi Wada
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  3. Hideyuki Sasaki
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  4. Hiromu Tanaka
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Correspondence to Masaki Nio.

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Nio, M., Wada, M., Sasaki, H. et al. Effects of age at Kasai portoenterostomy on the surgical outcome: a review of the literature. Surg Today 45, 813–818 (2015). https://doi.org/10.1007/s00595-014-1024-z

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  • DOI: https://doi.org/10.1007/s00595-014-1024-z

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