Three decades of progress: evolution of outcomes and prognostic indicators in biliary atresia management

Biliary atresia (BA) remains a challenging condition with variable outcomes following Kasai portoenterostomy (KPE). This study evaluates the predictive value of three scoring systems (ALBI, APRI, and METAVIR) for prognostication and analyzes three decades of treatment outcomes at a single institution.

Records of 96 BA patients who underwent KPE between 1990 and 2022 were retrospectively analyzed. Patients were stratified by preoperative ALBI (originally developed for HCC but applied here for its objective assessment of liver function), preoperative and postoperative APRI, and METAVIR scores. Treatment eras were divided into 1990-2000 (n = 18), 2001-2010 (n = 31), and 2011-2022 (n = 47). Clearance of jaundice was assessed at three months post-KPE, following established literature benchmarks. Kaplan-Meier curves and Log-Rank tests were used for survival analyses with a median follow-up of 5.1 years (range: 1-10 years).

The 10-year native liver survival (NLS) rate was 52.4%, with overall survival (OS) of 69.6%. Preoperative ALBI and APRI scores showed limited prognostic value (p = 0.12 and p = 0.17, respectively). However, postoperative APRI scores exceeding 1.12 at three months significantly predicted poor outcomes (p = 0.00094). METAVIR scores demonstrated poor correlation with long-term outcomes (p = 0.26). Analysis across treatment eras revealed significant improvement, with NLS increasing from 42.1 to 64.8% (p = 0.0029) and OS from 56.3 to 85.2% (p < 0.0001) between 1990 and 2000 and 2011-2022. Among the 52 patients with completed 10-year follow-up, the jaundice clearance rate at three months was 31.2%.

While preoperative scoring systems showed limited predictive ability, postoperative APRI scores at three months emerged as a powerful predictor of long-term outcomes in BA patients. Our findings suggest that patients with three-month postoperative APRI scores > 1.12 should receive more intensive monitoring and earlier consideration for transplantation. The significant improvement in outcomes across three decades demonstrates the value of accumulated institutional experience and improved clinical management protocols in this challenging disease.

Not applicable.