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