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Preoperative risk factors for the early failure of the Kasai portoenterostomy in patients with biliary atresia

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Abstract

Purpose

Biliary atresia is managed surgically by the Kasai portoenterostomy (KP). It has been reported by some groups that the outcomes of patients who have an early failed KP requiring a liver transplant (LTx) within the first year of life are worse than the outcomes of patients who undergo a primary LTx. The aim of this study was to identify preoperative parameters that could help predict what patients are at risk for the early failure of the procedure.

Materials and methods

We conducted a retrospective chart review of all patients who underwent a KP between January 2008 and May 2018. The following preoperative parameters were analyzed: age at KP, anatomical variant of the biliary atresia, degree of liver fibrosis, CMV status, and PELD score. The main outcome of the study was the early failure of the KP (EF-K), which was defined as the need for LTx before 1 year of age, or BA-related death before 1 year of age. Second, we analyzed the risk factors associated with death without LTx within the first year of life.

Results

A total of 58 patients were included in the analysis. The native liver survival (NLS) was 56.5% and 48% at 1 and 5 years post KP, respectively. Overall survival (OS) was 79% and 76% at 1 and 5 years post KP, respectively. Early failure of KP occurred in 23 (39.7%) patients. OS in this group was 47% and 40% at 1 and 5 years, respectively. On the contrary, the OS of the remaining 35 (60.3%) patients was 100% at 1 and 5 years (P < 0.0001). When we compared all preoperative parameters, the only predictor of EF-K was the PELD score. When we analyzed the cases in the EF-K group who died without LTx, we found that the significant predictors were the cystic variant, a degree of liver fibrosis >4, and the PELD score. Nevertheless, on multivariate analysis, only PELD score was found as a statistically significant variable.

Conclusion

Due to bad prognosis found in EF-K patients, we believe that it could be reasonable to offer them a primary LTx. PELD score was found to be the strongest preoperative parameter that allows predicting which patient will likely have an early failed KP. Further prospective and multicenter studies are needed to reinforce these results.

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Abbreviations

BA:

Biliary atresia

KP:

Kasai portoenterostomy

LTx:

Liver transplant

EF-K:

Early failed Kasai

NLS:

Native liver survival

OS:

Overall survival

PELD:

Pediatric end-stage liver disease

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Funding

The authors did not receive support from any organization for the submitted work. The authors have no relevant financial or non-financial interests to disclose.

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Authors and Affiliations

  1. Division of General Surgery, J. P. Garrahan Hospital, Combate de los Pozos 1881, C 1245 AAM, Buenos Aires, Argentina

    Mauro Ariel Capparelli, Victor Hugo Ayarzabal, Horacio Alberto Questa & Marcelo Eugenio Barrenechea

  2. Division of Liver Transplant, J. P. Garrahan Hospital, Buenos Aires, Argentina

    Esteban Tomas Halac, Maria Julia Minetto & Guillermo Cervio

Authors
  1. Mauro Ariel Capparelli
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  2. Victor Hugo Ayarzabal
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  3. Esteban Tomas Halac
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  4. Horacio Alberto Questa
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  5. Maria Julia Minetto
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  6. Guillermo Cervio
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  7. Marcelo Eugenio Barrenechea
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Contributions

Study conception and design: CM. Data acquisition: CM. Analysis and data interpretation: HE, MJ. Drafting of the manuscript: CM, HE, MJ. Critical revision: AV, QH, CG, BM.

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Correspondence to Mauro Ariel Capparelli.

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Capparelli, M.A., Ayarzabal, V.H., Halac, E.T. et al. Preoperative risk factors for the early failure of the Kasai portoenterostomy in patients with biliary atresia. Pediatr Surg Int 37, 1183–1189 (2021). https://doi.org/10.1007/s00383-021-04933-y

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  • DOI: https://doi.org/10.1007/s00383-021-04933-y

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