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

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.