Standardised Transplant-Orientated Kasai Portoenterostomy in a Combined Kasai and Transplant Programme: An 11-Year...

Background: Kasai hepatoportoenterostomy (KPE) remains first-line therapy for biliary atresia(BA), yet the majority of patients will ultimately require liver transplantation. In centres where both KPE and paediatric liver transplantation are performed, KPE has been understood since the early 2000s as the first stage of a two-stage strategy, and the individual technical elements described here are established practice. What is reported is their uniform application as a single written protocol from the first case of the series, together with native liver and transplant outcomes in the resulting cohort. The technical conduct of KPE directly influences the safety and complexity of subsequent transplantation, yet operative decisions at the time of KPE have rarely been evaluated from a transplant-optimisation perspective. We describe a standardised KPE approach incorporating three technical modifications intended to preserve favourable conditions for eventual hepatic replacement and report native liver and transplant outcomes in the resulting cohort. Methods: A retrospective analysis was conducted of 74 consecutive KPE procedures performed by a single surgeon between 2014 and 2025 at Schneider Children's Medical Center, Israel. The operative approach incorporated three deliberate modifications applied uniformly from the first case: a transverse subcostal incision aligned with future transplant access, avoidance of liver mobilisation and exteriorisation, and standardisation of the Roux limb at 50 cm. Primary outcomes were native liver survival and transplant operative parameters. Results: Of the 74 patients, 39 (52.7%) maintained their native liver throughout follow-up, while 35 (47.3%) required liver transplantation. No peri-operative mortality occurred. Median age at KPE was 53 days. In the Cox model, post-KPE portal hypertension (adjusted hazard ratio (aHR) 3.63, 95% confidence interval (CI) 1.40-9.42, p = 0.008) and hepatopulmonary syndrome (adjusted HR 10.23, 95% CI 2.19-47.85, p = 0.003) were associated with eventual transplantation; complications were modelled as fixed (ever/never) covariates because onset dates were not consistently retrievable, and these estimates may be subject to immortal-time bias. Among transplanted patients, the median operative time was 8.0 h (interquartile range (IQR) 6.0-10.2) (n = 34); intraoperative blood loss was documented in 15 of 35 patients (median 300 mL (IQR 205-450)). Conclusions: A standardised, transplant-orientated KPE approach was applied uniformly across 74 consecutive cases; 52.7% maintained their native liver, and transplantation in the remainder proceeded without peri-operative mortality. This series was descriptive by design and included no comparator group that operated without these modifications; it therefore cannot establish whether the modifications influence the complexity of subsequent transplantation, and no such inference should be drawn. Post-KPE portal hypertension and hepatopulmonary syndrome are markers associated with transplant requirement and should prompt intensified surveillance. The approach represents the standardisation of existing practice rather than a new technique.