Neonatal Kasai portoenterostomy for biliary atresia: Clinical characteristics and outcomes.

BACKGROUND & AIMS: Biliary atresia (BA) is a progressive cholangiopathy where prognosis is strongly linked to the timing of Kasai portoenterostomy (KPE). While early surgery is advocated, data specifically restricted to the neonatal period are limited. This study aims to evaluate the clinical characteristics and outcomes of infants undergoing KPE strictly within the neonatal window and to identify prognostic factors.

METHODS

We conducted a multicenter retrospective study of 56 neonates who underwent KPE between 2014 and 2022. To assess the clinical impact of neonatal timing, a comparison cohort of 164 infants who underwent KPE at 29-90 days of age was also analyzed. The primary outcomes were jaundice clearance and native liver survival (NLS).

RESULTS

The neonatal cohort achieved a jaundice clearance rate of 82.1%, which was significantly higher than the 73.2% observed in the non-neonatal comparison group (p < 0.05). NLS in the neonatal group remained excellent through 72 months. Within the neonatal cohort, earlier surgery correlated with higher clearance rates, supporting the "earlier is better" principle even in the first month of life. Exploratory analysis revealed that a preoperative biochemical profile of high Gamma-Glutamyl Transferase (GGT) and low Alanine Aminotransferase (ALT) was associated with the most favorable outcomes, with clearance rates exceeding 90%.

CONCLUSION

KPE performed in the neonatal period was associated with a higher jaundice clearance rate compared with later intervention, while no significant difference in native liver survival was demonstrated during the present follow-up. A "high-GGT/low-ALT" profile may serve as an exploratory favorable prognostic marker in neonates.

LEVEL OF EVIDENCE: Level II.