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Age at surgery and native liver survival in biliary atresia: a systematic review and meta-analysis

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Abstract

Biliary atresia (BA) is a childhood rare disease of the liver and bile ducts that requires prompt surgical intervention. Age at surgery is an important prognostic factor; however, controversy exists with regard to the benefit of early Kasai procedure (KP). We aimed to conduct a systematic review and meta-analysis to examine the relationship between the age at KP and native liver survival (NLS) of BA patients. We performed the electronic database search using Pubmed, EMBASE, Cochrane, and Ichushi Web and included all relevant studies published from 1968 up to May 3, 2022. Studies that examined the timing of KP at ages 30, 45, 60, 75, 90, 120, and/or 150 days were included. The outcome measures of interest were NLS rates at 5, 10, 15, 20, and 30 years post-KP and the hazard ratio or risk ratio for NLS. The quality assessment was used using the ROBINS-I tool. Among 1653 potentially eligible studies, nine articles met the inclusion criteria for the meta-analysis. Meta-analysis for hazard ratios revealed that there was a significantly faster time to liver transplantation in the group of patients who had KP at later timing as compared with earlier KP (HR = 2.12, 95% CI 1.51–2.97). The risk ratio comparing KP ≤ 30 days and KP ≥ 31 days on native liver survival was 1.22 (95% CI 1.13–1.31). The sensitivity analysis showed that comparing KP ≤ 30 days and KP 31–60 days, the risk ratio was 1.13, 95% CI 1.04–1.22.

  Conclusion: Our meta-analysis showed the importance of early diagnosis and surgical interventions ideally before 30 days of life in infants with BA on native liver survival on 5, 10, and 20 years. Therefore, effective newborn screening of BA targeting KP ≤ 30 days is needed to ensure prompt diagnosis of affected infants.

What is Known:

• Age at surgery is an important prognostic factor.

What is New:

• Our study performed an updated systematic review and meta-analysis to examine the relationship between age at Kasai procedure and native liver survival in patients with BA.

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Data availability

The data that support the findings of this study are available from the corresponding author, [EH], upon reasonable request.

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Funding

This work was supported by JSPS KAKENHI Grant Number 20K18896.

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Authors and Affiliations

  1. Comprehensive Unit for Health Economic Evidence Review and Decision Support (CHEERS), Ritsumeikan University, Kyoto, Japan

    Eri Hoshino, Kotomi Sakai & Nobuyuki Shimohata

  2. Graduate School of Public Health, St. Luke’s International University, Tokyo, Japan

    Eri Hoshino & Kevin Y. Urayama

  3. Department of Pediatric, Juntendo University, Tokyo, Japan

    Yamato Muto & Mitsuyoshi Suzuki

  4. Department of Social Medicine, National Center for Child Health and Development, Tokyo, Japan

    Kevin Y. Urayama

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  1. Eri Hoshino
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  2. Yamato Muto
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  3. Kotomi Sakai
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Contributions

Eri Hoshino designed the study, performed literature screening and analysis, drafted the manuscript, and revised the manuscript. Yamato Muto, Kotomi Sakai, and Nobuyuki Shimohata performed literature screening and data extraction and revised the manuscript. Kevin Urayama and Mitsuyoshi Suzuki performed critical revision of the manuscript. All authors have approved the final manuscript.

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Correspondence to Eri Hoshino.

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Hoshino, E., Muto, Y., Sakai, K. et al. Age at surgery and native liver survival in biliary atresia: a systematic review and meta-analysis. Eur J Pediatr 182, 2693–2704 (2023). https://doi.org/10.1007/s00431-023-04925-1

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