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Predictors of Need for Liver Transplantation in Children Undergoing Hepatoportoenterostomy for Biliary Atresia

      Abstract

      Purpose

      The purpose of this study was to determine perioperative risk factors for need of liver transplantation following hepatoportoenterostomy.

      Methods

      A retrospective review of patients undergoing hepatoportoenterostomy for biliary atresia at our institution from 1990 to 2016 was completed.

      Results

      A total of 81 patients were identified with a median age of 51 days (IQR: 33-68) at hepatoportoenterostomy and a median follow-up time of 5.7 years (IQR: 1-11.6). Ten-year overall survival was 93% (95% CI: 84-97). Thirty-six patients (44%) ultimately required transplantation at a median time from hepatoportoenterostomy of 8.9 months (IQR: 5.2-19). The 10-year transplant-free survival was 36% (95%CI: 24-49). Steroid use (N=42) was not associated with improved 10-yr transplant-free survival (33% vs. 38%, p=0.690). Age at hepatoportoenterostomy was not significantly associated with the need for transplantation. Multivariable logistic regression analysis demonstrated that total bilirubin >2mg/dL (OR: 97, p<0.001) and albumin < 3.5g/dL (OR: 24, p=0.027) at 3 months after surgery were independent predictors of the need for transplantation, while adjusting for age, sex, prematurity, and steroid use.

      Conclusion

      Overall survival for children with biliary atresia is excellent, although most patients will ultimately require liver transplantation. Total bilirubin and albumin level at 3 months following hepatoportoenterostomy are predictive of the need for transplantation. Steroid use is not associated with improved outcomes.

      Abbreviations:

      BA (biliary atresia), BASM (Biliary atresia splenic malformation syndrome), CI (confidence interval), HPE (hepatoportoenterostomy), IQR (interquartile range)

      Keywords

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      References

        • Hopkins P.C.
        • Yazigi N.
        • Nylund C.M.
        Incidence of Biliary Atresia and Timing of Hepatoportoenterostomy in the United States.
        J Pediatr. 2017; 187: 253-257https://doi.org/10.1016/j.jpeds.2017.05.006
        • Bassett M.D.
        • Murray K.F.
        Biliary atresia: recent progress.
        J Clin Gastroenterol. 2008; 42: 720-729https://doi.org/10.1097/MCG.0b013e3181646730
        • Feldman A.G.
        • Mack C.L.
        Biliary Atresia: Clinical Lessons Learned.
        J Pediatr Gastroenterol Nutr. 2015; 61: 167-175https://doi.org/10.1097/MPG.0000000000000755
        • Altman R.P.
        • Lilly J.R.
        • Greenfeld J.
        • et al.
        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: 348-353-355
      1. Kim WR, Lake JR, Smith JM, Skeans MA, Schladt DP, Edwards EB, et al. OPTN/SRTR 2015 Annual Data Report: Liver. Am J Transplant Off J Am Soc Transplant Am Soc Transpl Surg 2017;17 Suppl 1:174–251. doi:https://doi.org/10.1111/ajt.14126.

        • Serinet M.-O.
        • Wildhaber B.E.
        • Broué P.
        • et al.
        Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening.
        Pediatrics. 2009; 123: 1280-1286https://doi.org/10.1542/peds.2008-1949
        • Wong K.K.Y.
        • Chung P.H.Y.
        • Chan I.H.Y.
        • et al.
        Performing Kasai portoenterostomy beyond 60 days of life is not necessarily associated with a worse outcome.
        J Pediatr Gastroenterol Nutr. 2010; 51: 631-634https://doi.org/10.1097/MPG.0b013e3181e8e194
        • Hung P.-Y.
        • Chen C.-C.
        • Chen W.-J.
        • et al.
        Long-term prognosis of patients with biliary atresia: a 25 year summary.
        J Pediatr Gastroenterol Nutr. 2006; 42: 190-195https://doi.org/10.1097/01.mpg.0000189339.92891.64
        • Nightingale S.
        • Stormon M.O.
        • O’Loughlin E.V.
        • et al.
        Early Posthepatoportoenterostomy Predictors of Native Liver Survival in Biliary Atresia.
        J Pediatr Gastroenterol Nutr. 2017; 64: 203-209https://doi.org/10.1097/MPG.0000000000001289
      2. Shneider BL, Magee JC, Karpen SJ, Rand EB, Narkewicz MR, Bass LM, et al. Total Serum Bilirubin within 3 Months of Hepatoportoenterostomy Predicts Short-Term Outcomes in Biliary Atresia. J Pediatr 2016;170:211-217-2. doi:https://doi.org/10.1016/j.jpeds.2015.11.058.

        • Shneider B.L.
        • Brown M.B.
        • Haber B.
        • et al.
        A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000.
        J Pediatr. 2006; 148: 467-474https://doi.org/10.1016/j.jpeds.2005.12.054
        • Yoon P.W.
        • Bresee J.S.
        • Olney R.S.
        • et al.
        Epidemiology of biliary atresia: a population-based study.
        Pediatrics. 1997; 99: 376-382
        • Nio M.
        • Ohi R.
        • Miyano T.
        • 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
        • Kasai M.
        • Suzuki H.
        • Ohashi E.
        • et al.
        Technique and results of operative management of biliary atresia.
        World J Surg. 1978; 2: 571-579
        • Schoen B.T.
        • Lee H.
        • Sullivan K.
        • et al.
        The Kasai portoenterostomy: when is it too late?.
        J Pediatr Surg. 2001; 36: 97-99https://doi.org/10.1053/jpsu.2001.20020
        • Goda T.
        • Kawahara H.
        • Kubota A.
        • et al.
        The most reliable early predictors of outcome in patients with biliary atresia after Kasai’s operation.
        J Pediatr Surg. 2013; 48: 2373-2377https://doi.org/10.1016/j.jpedsurg.2013.08.009
        • Meyers R.L.
        • Book L.S.
        • O’Gorman M.A.
        • et al.
        High-dose steroids, ursodeoxycholic acid, and chronic intravenous antibiotics improve bile flow after Kasai procedure in infants with biliary atresia.
        J Pediatr Surg. 2003; 38: 406-411https://doi.org/10.1053/jpsu.2003.50069
        • Davenport M.
        • Stringer M.D.
        • Tizzard S.A.
        • et al.
        Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia.
        Hepatol Baltim Md. 2007; 46: 1821-1827https://doi.org/10.1002/hep.21873
        • Bezerra J.A.
        • Spino C.
        • Magee J.C.
        • et al.
        Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial.
        JAMA. 2014; 311: 1750-1759https://doi.org/10.1001/jama.2014.2623
      3. Japanese Biliary Atresia Society, Nio M, Muraji T. Multicenter randomized trial of postoperative corticosteroid therapy for biliary atresia. Pediatr Surg Int 2013;29:1091–5. doi:10.1007/s00383-013-3377-6.

        • Zhang M.-Z.
        • Xun P.-C.
        • He K.
        • et al.
        Adjuvant steroid treatment following Kasai portoenterostomy and clinical outcomes of biliary atresia patients: an updated meta-analysis.
        World J Pediatr WJP. 2017; 13: 20-26https://doi.org/10.1007/s12519-016-0052-8