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Native liver survivors of portoenterostomy for biliary atresia with excellent outcome: redefining “successful” portoenterostomy

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Abstract

Purpose

Native liver survivors (NLS) after portoenterostomy (PE) for biliary atresia (BA) with normal biomarkers defined as total bilirubin (T-Bil), aspartate aminotransferase (AST)/alanine aminotransferase (ALT) for liver function (LF), cholinesterase (ChE), platelet count (PC), and absence of portal hypertension (PHT) were reviewed to redefine “successful” PE.

Methods

92 post-PE BA patients were classified as NLS-1: normal biomarkers, PHT (−); NLS-2: at least one abnormal biomarker, PHT (−); NLS-3: normal biomarkers, PHT ( +); NLS-4: abnormal biomarkers, PHT ( +) and reviewed for a maximum 32 years.

Results

As of June 2022, 55/92 (59.8%) had received liver transplants and 37/92 (40.2%) were NLS. NLS patients were classified as excellent outcome (EO): NLS-1 (n = 10; 27.0%) or non-EO: NLS-2: (n = 8; 21.6%), NLS-3: (n = 6; 16.2%), and NLS-4: (n = 13; 35.1%). Compared with non-EO, EO had PE earlier (50.5 versus 65 days; not significant; p = 0.08), significantly earlier onset of symptoms (13 days versus 32 days; p = 0.01) and significantly shorter jaundice-clearance (JC; 34.5 days versus 56.0 days; p < 0.001). Durations of follow-up were similar: 13 years in EO, 18.5 years in NLS-2, 20 years in NLS-3, and 15 years in NLS-4.

Conclusion

Incidence of “successful” PE or EO is low and correlated with early onset of symptoms and quicker JC.

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Data Availability

The datasets generated during and/or analysed during the current study are available from the corresponding author on reasonable request.

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Authors and Affiliations

  1. Department of Pediatric Surgery, Faculty of Medicine, Juntendo University, 2-1-1 Hongo, Bunkyo-ku, Tokyo, 113-8421, Japan

    Koichi Tsuboi, Hiroko Watayo, Takafumi Tsukui, Kazuto Suda, Takanori Ochi, Geoffrey J. Lane, Hiroyuki Koga & Atsuyuki Yamataka

  2. Department of Pediatric Surgery, Juntendo Urayasu Hospital, Chiba, Japan

    Eri Abe

  3. Department of Pediatric Surgery, Juntendo Nerima Hospital, Tokyo, Japan

    Takamori Fujimoto

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  1. Koichi Tsuboi
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Contributions

KT designed the study and wrote the draft manuscript; HW, KZ, TT, TF, EA, TO, and HK collected data; GL critically revised the draft manuscript for clinical content and English expression; AY supervised the design of the study and critical revision of the manuscript for clinical content.

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Correspondence to Koichi Tsuboi.

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Tsuboi, K., Watayo, H., Tsukui, T. et al. Native liver survivors of portoenterostomy for biliary atresia with excellent outcome: redefining “successful” portoenterostomy. Pediatr Surg Int 39, 24 (2023). https://doi.org/10.1007/s00383-022-05313-w

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