We explored the chronologic changes in prognostic factors and clinical outcomes of Kasai portoenterostomy (KPE) for biliary atresia (BA).
Patients undergoing KPE between 1997 and 2016 were analyzed retrospectively. Ninety-two consecutive patients who underwent KPE from 1997 to 2006 (Era 1) were compared with 150 patients who underwent KPE from 2007 to 2016 (Era 2) for clinical outcomes and prognostic factors.
The jaundice clearance rate increased by 8.8% (66.7% vs. 75.5% for Eras 1 and 2, respectively, p = 0.180), and the 5-year native liver survival (NLS) rate improved slightly (62.5% vs. 64.0% for Eras 1 and 2, respectively, p = 0.617) in Era 2. The hazard ratio for age at KPE (≥ 90 days) with regard to 5-year NLS and the odds ratio for age at KPE (< 90 days) with regard to jaundice clearance were both lower in Era 2 than in Era 1 (1.95 vs. 2.25 and 2.67 vs. 5.21, respectively).
The clinical outcomes improved over a period in a single surgeon’s practice. We demonstrated that the impact of age at the time of KPE on operative outcomes became less significant over time with the increase in the single surgeon’s experience and improvement in medical treatment for BA.
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Lee KJ, Kim JW, Moon JS, Ko JS (2017) Epidemiology of biliary atresia in Korea. J Korean Med Sci 32(4):656–660. https://doi.org/10.3346/jkms.2017.32.4.656
Livesey E, Cortina Borja M, Sharif K et al (2009) Epidemiology of biliary atresia in England and Wales (1999–2006). Arch Dis Child Fetal Neonatal Ed 94(6):F451–F455. https://doi.org/10.1136/adc.2009.159780
Yoon PW, Bresee JS, Olney RS, James LM, Khoury MJ (1997) Epidemiology of biliary atresia: a population-based study. Pediatrics 99(3):376–382
Chardot C, Carton M, Spire-Bendelac N, Le Pommelet C, Golmard JL, Auvert B (1999) Epidemiology of biliary atresia in France: a national study 1986-96. J Hepatol 31(6):1006–1013
Lee S, Park H, Moon SB et al (2013) Long-term results of biliary atresia in the era of liver transplantation. Pediatr Surg Int 29(12):1297–1301. https://doi.org/10.1007/s00383-013-3366-9
Wildhaber BE (2012) Biliary atresia: 50 years after the first kasai. ISRN Surg 2012:132089. https://doi.org/10.5402/2012/132089
Shinkai M, Ohhama Y, Take H et al (2009) 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 48(4):443–450
Shin NY, Kim MJ, Lee MJ et al (2014) Transient elastography and sonography for prediction of liver fibrosis in infants with biliary atresia. J Ultrasound Med 33(5):853–864. https://doi.org/10.7863/ultra.33.5.853
Chang HK, Park YJ, Koh H et al (2009) Hepatic fibrosis scan for liver stiffness score measurement: a useful preendoscopic screening test for the detection of varices in postoperative patients with biliary atresia. J Pediatr Gastroenterol Nutr 49(3):323–328. https://doi.org/10.1097/MPG.0b013e31819de7ba
Willis AD, Miloh TA, Arnon R, Iyer KR, Suchy FJ, Kerkar N (2011) Hepatopulmonary syndrome in children—is conventional liver transplantation always needed? Clin Transplant 25(6):849–855. https://doi.org/10.1111/j.1399-0012.2010.01378.x
Laving A, Khanna A, Rubin L, Ing F, Dohil R, Lavine JE (2005) Successful liver transplantation in a child with severe portopulmonary hypertension treated with epoprostenol. J Pediatr Gastroenterol Nutr 41(4):466–468
Davenport M, Ong E, Sharif K et al (2011) Biliary atresia in England and Wales: results of centralization and new benchmark. J Pediatr Surg 46(9):1689–1694. https://doi.org/10.1016/j.jpedsurg.2011.04.013
Nio M, Sasaki H, Wada M, Kazama T, Nishi K, Tanaka H (2010) Impact of age at Kasai operation on short- and long-term outcomes of type III biliary atresia at a single institution. J Pediatr Surg 45(12):2361–2363. https://doi.org/10.1016/j.jpedsurg.2010.08.032
Serinet MO, Wildhaber BE, Broue P et al (2009) Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics 123(5):1280–1286. https://doi.org/10.1542/peds.2008-1949
Hahn SM, Kim S, Park KI, Han SJ, Koh H (2013) Clinical benefit of liver stiffness measurement at 3 months after Kasai hepatoportoenterostomy to predict the liver related events in biliary atresia. PLoS One 8(11):e80652. https://doi.org/10.1371/journal.pone.0080652
Joshi D, Gupta N, Samyn M, Deheragoda M, Dobbels F, Heneghan MA (2017) The management of childhood liver diseases in adulthood. J Hepatol 66(3):631–644. https://doi.org/10.1016/j.jhep.2016.11.013
Arnon R, Leshno M, Annunziato R, Florman S, Iyer K (2014) What is the optimal timing of liver transplantation for children with biliary atresia? A Markov model simulation analysis. J Pediatr Gastroenterol Nutr 59(3):398–402. https://doi.org/10.1097/MPG.0000000000000428
Lampela H, Ritvanen A, Kosola S et al (2012) National centralization of biliary atresia care to an assigned multidisciplinary team provides high-quality outcomes. Scand J Gastroenterol 47(1):99–107. https://doi.org/10.3109/00365521.2011.627446
Schreiber RA, Barker CC, Roberts EA, Martin SR, Canadian Pediatric Hepatology Research G (2010) Biliary atresia in Canada: the effect of centre caseload experience on outcome. J Pediatr Gastroenterol Nutr 51(1):61–65. https://doi.org/10.1097/MPG.0b013e3181d67e5e
Serinet MO, Broue P, Jacquemin E et al (2006) Management of patients with biliary atresia in France: results of a decentralized policy 1986–2002. Hepatology 44(1):75–84. https://doi.org/10.1002/hep.21219
Oh JT, Kim DY, Kim SC et al (2013) Biliary atresia: a Survey by the Korean Association of Pediatric Surgeons in 2011. J Korean Assoc Pediatr Surg 19(1):1–13. https://doi.org/10.13029/jkaps.2013.19.1.1
Song Z, Dong R, Shen Z, Chen G, Yang Y, Zheng S (2017) Surgical outcome and etiologic heterogeneity of infants with biliary atresia who received Kasai operation less than 60 days after birth: a retrospective study. Medicine (Baltimore) 96(26):e7267. https://doi.org/10.1097/MD.0000000000007267
Nio M, Sano N, Ishii T, Sasaki H, Hayashi Y, Ohi R (2006) Long-term outcome in type I biliary atresia. J Pediatr Surg 41(12):1973–1975. https://doi.org/10.1016/j.jpedsurg.2006.08.019
Davenport M, Tizzard SA, Underhill J, Mieli-Vergani G, Portmann B, Hadzic N (2006) The biliary atresia splenic malformation syndrome: a 28-year single-center retrospective study. J Pediatr 149(3):393–400. https://doi.org/10.1016/j.jpeds.2006.05.030
Kim JM, Kim KM, Yi NJ et al (2013) Pediatric liver transplantation outcomes in Korea. J Korean Med Sci 28(1):42–47. https://doi.org/10.3346/jkms.2013.28.1.42
Conflict of interest
The authors have no conflicts of interest or financial relationships relevant to this article to disclose.
All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. The study was approved by the institutional review board of Severance Hospital (approval number: 4-2017-1101).
A waiver of informed consent was granted because the study was determined to be minimal risk and because data are de-identified.
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Ihn, K., Na, Y., Ho, I.G. et al. A periodic comparison of the survival and prognostic factors of biliary atresia after Kasai portoenterostomy: a single-center study in Korea. Pediatr Surg Int 35, 285–292 (2019). https://doi.org/10.1007/s00383-018-04434-5
- Biliary atresia
- Kasai portoenterostomy
- Liver fibrosis
- Native liver survival