Elsevier

The Journal of Pediatrics

Volume 160, Issue 4, April 2012, Pages 638-644.e2
The Journal of Pediatrics

Original Article
Biliary Atresia in The Netherlands: Outcome of Patients Diagnosed between 1987 and 2008

https://doi.org/10.1016/j.jpeds.2011.09.061Get rights and content

Objective

To examine the outcome of biliary atresia (BA) and to identify prognostic factors using a national database.

Study design

All children born between January 1987 and December 2008 who underwent the Kasai surgical procedure for BA were retrieved from the Netherlands Study Group on Biliary Atresia Registry database. Outcomes were measured in terms of clearance of jaundice (bilirubin <1.17 g/dL, or 20 μmol/L, within 6 months after surgery) and 4-year transplant-free survival. Two cohorts, one from 1987-1997 and the other from 1998-2008, were compared. Survival rates were determined using Kaplan-Meier analysis, and prognostic factors were tested with univariate and multivariate analyses.

Results

Between January 1987 and December 2008, 214 patients underwent Kasai surgery for BA. In this series, the 4-year transplant-free survival was 46% ± 4%, and 4-year overall survival was 73% ± 3%. Clearance of jaundice, surgery within 60 days, and postoperative antibiotic prophylaxis use were independently associated with increased transplant-free survival. The yearly caseload per center (range, 0.5-2.1) was not correlated with transplant-free survival (r = 0.024; P = .73).

Conclusion

During the past 2 decades, outcome parameters have remained constant and are comparable with those reported from other Western countries, despite a relatively low annual caseload per center. Timely surgical correction and postoperative antibiotic therapy were associated with a higher transplant-free survival rate.

Section snippets

Methods

The Netherlands Study Group on Biliary Atresia Registry (NeSBAR) database is based on an ongoing joint effort of the Dutch Society for Pediatrics and the Dutch Society for Pediatric Surgeons. The registry contains data for all patients with BA treated in the 6 specialized academic centers in The Netherlands. Each center is independent and offers extensive diagnostic workup for neonatal cholestasis, primary surgical treatment (classical portoenterostomy according to the Kasai procedure or a

Results

Between January 1, 1987, and December 31, 2008, 231 infants (114 females; 49.4%) were diagnosed with BA in The Netherlands. Three patients from overseas (Dutch Antilles) had been operated on in the continental Netherlands, but these were excluded from further analysis because the first presentation and follow-up were not readily accessible for analysis. The overall incidence of BA in The Netherlands during the study period was 1 in 18 619 live births (Statistics Netherlands, http://www.cbs.nl).

Discussion

Our national results from The Netherlands are comparable with those from other Western countries (France, United Kingdom) and remarkably constant over time. Our transplant-free survival was similar to that in the previous Dutch cohort analysis.17 It is striking that although general health care has seen much development over the past 3 decades, transplant-free survival has remained constant. Overall survival has increased slightly over this period, most likely attributed to an increased

References (35)

  • K.D. Lindor et al.

    Effects of ursodeoxycholic acid on survival in patients with primary biliary cirrhosis

    Gastroenterology

    (1996)
  • R.J. Sokol et al.

    Pathogenesis and outcome of biliary atresia: current concepts

    J Pediatr Gastroenterol Nutr

    (2003)
  • D. Kelly et al.

    Current management of biliary atresia

    Arch Dis Child

    (2007)
  • M. Davenport et al.

    Biliary atresia splenic malformation syndrome: an etiologic and prognostic subgroup

    Surgery

    (1993)
  • M. Kasai et al.

    A new operation for “non-correctable” biliary atresia: portoenterostomy

    Shujutsu

    (1959)
  • R. Scheenstra et al.

    Graft fibrosis after pediatric liver transplantation: ten years of follow-up

    Hepatology

    (2009)
  • V.L. Ng et al.

    Outcomes of 5-year survivors of pediatric liver transplantation: report on 461 children from a North American multicenter registry

    Pediatrics

    (2008)
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    The authors declare no conflicts of interest.

    A list of NeSBAR members is available at www.jpeds.com (Appendix).

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