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The need for early Kasai portoenterostomy: a Western Pediatric Surgery Research Consortium study

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Abstract

Purpose

The purpose of this study was to investigate factors impacting transplant-free survival among infants with biliary atresia.

Methods

A multi-institutional, retrospective cohort study was performed at nine tertiary-level children’s hospitals in the United States. Infants who underwent Kasai portoenterostomy (KP) from January 2009 to May 2017 were identified. Clinical characteristics included age at time of KP, steroid use, surgical approach, liver pathology, and surgeon experience. Likelihood of transplant-free survival (TFS) was evaluated using logistic regression, adjusting for patient and surgeon-level factors. Secondary outcomes at 1 year included readmission, cholangitis, reoperation, mortality, and biliary clearance.

Results

Overall, 223 infants underwent KP, and 91 (40.8%) survived with their native liver. Mean age at surgery was 63.9 days (± 24.7 days). At 1 year, 78.5% experienced readmission, 56.9% developed cholangitis, 3.8% had a surgical revision, and 5 died. Biliary clearance at 3 months was achieved in 76.6%. Controlling for patient and surgeon-level factors, each additional day of age toward operation was associated with a 2% decrease in likelihood of TFS (OR 0.98, 95% CI 0.97–0.99).

Conclusion

Earlier surgical intervention by Kasai portoenterostomy at tertiary-level centers significantly increases likelihood for TFS. Policy-level interventions to facilitate early screening and surgical referral for infants with biliary atresia are warranted to improve outcomes.

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Abbreviations

BA :

Biliary atresia

HIPAA :

Health Insurance Portability and Accountability Act

KP :

Kasai portoenterostomy

OLT :

Orthotopic liver transplant

REDCap :

Research Electronic Data Capture

TFS :

Transplant-free survival

WPSRC :

Western Pediatric Surgery Research Consortium

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Funding

Dr. Kelley-Quon is supported by grant KL2TR001854 from the National Center for Advancing Translational Science (NCATS) of the U.S. National Institutes of Health. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. The remaining authors have no relevant financial or non-financial interests to disclose.

Author information

Authors and Affiliations

  1. Division of Pediatric Surgery, Children’s Hospital Los Angeles and the Keck School of Medicine, University of Southern California, 4650 Sunset Blvd. MS #100, Los Angeles, CA, 90027, USA

    Lorraine I. Kelley-Quon, Eveline Shue, Elaa Mahdi, Shadassa Ourshalimian, Michael Fenlon & Kasper Wang

  2. Department of Population and Public Health Sciences, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA

    Lorraine I. Kelley-Quon & Rita V. Burke

  3. Division of Pediatric General and Thoracic Surgery, Department of Surgery, Seattle Children’s Hospital, University of Washington School of Medicine, Seattle, WA, USA

    Caitlin Smith & Matthew Dellinger

  4. Department of Pediatric Surgery, Rady Children’s Hospital San Diego, University of California School of Medicine, San Diego, CA, USA

    Karen Kling & Romeo Ignacio

  5. Division of Pediatric Surgery, Department of Surgery, Stanford University School of Medicine, Palo Alto, CA, USA

    Stephen B. Shew

  6. Division of Surgery, Phoenix Children’s Hospital, Phoenix, AZ, USA

    Justin Lee, Benjamin Padilla & Daniel J. Ostlie

  7. Department of Surgery, University of Colorado Denver School of Medicine and Children’s Hospital of Colorado, Aurora, CO, USA

    Thomas Inge, Jonathan Roach & Ahmed I. Marwan

  8. Division of Pediatric Surgery, University of Utah and Primary Children’s Hospital, Salt Lake City, UT, USA

    Katie W. Russell

  9. Division of Pediatric Surgery, Department of Surgery, Oregon Health and Science University, Portland, OR, USA

    Elizabeth Fialkowski & Kenneth S. Azarow

  10. Division of Pediatric Surgery, University of California, San Francisco, CA, USA

    Amar Nijagal & Cecilia Im

Authors
  1. Lorraine I. Kelley-Quon
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  2. Eveline Shue
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  3. Rita V. Burke
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  4. Caitlin Smith
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  5. Karen Kling
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  6. Elaa Mahdi
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  7. Shadassa Ourshalimian
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  8. Michael Fenlon
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  9. Matthew Dellinger
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  10. Stephen B. Shew
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  11. Justin Lee
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  12. Benjamin Padilla
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  13. Thomas Inge
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  14. Jonathan Roach
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  15. Ahmed I. Marwan
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  16. Katie W. Russell
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  17. Romeo Ignacio
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  18. Elizabeth Fialkowski
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  19. Amar Nijagal
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  20. Cecilia Im
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  21. Kenneth S. Azarow
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  22. Daniel J. Ostlie
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  23. Kasper Wang
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Contributions

All authors contributed to the study conception and design. Material preparation, data collection, and analysis were performed by LK-Q, RB, EM, and SO. The first draft of the manuscript was written by LK-Q and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Lorraine I. Kelley-Quon.

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The authors have no financial relationships relevant to this article or potential competing interests to disclose.

Consent to participate

Institutional Review Board (IRB) approval was obtained independently by each member institution. All member institution IRBs approved a waiver of consent to collect medical record data.

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Kelley-Quon, L.I., Shue, E., Burke, R.V. et al. The need for early Kasai portoenterostomy: a Western Pediatric Surgery Research Consortium study. Pediatr Surg Int 38, 193–199 (2022). https://doi.org/10.1007/s00383-021-05047-1

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