Late Referral of Biliary Atresia and Poor Clinical Outcomes

Biliary atresia (BA) is a progressive cholangiopathy in infants that often necessitates liver transplantation. This study aimed to evaluate the impact of delayed referral on clinical and histopathological outcomes in BA cases.

This study was a prospective observational study conducted from January to September 2023 at Dr. Soetomo General Academic Hospital, Surabaya, Indonesia.

A total of 137 infants with cholestasis were enrolled. Of these, only 85 underwent liver biopsy, with 60 cases (70.6%) confirmed as extrahepatic cholestasis consistent with BA. Among these 60 BA-confirmed patients, 83.3% were referred after 60 days of age. Patients in the late referral group (>60 days) in all cases exhibited higher leukocyte counts, total bilirubin levels, prolonged prothrombin time, and hypoalbuminemia, reflecting a more advanced disease state. Two-phase ultrasonography detected abnormalities in 76.7% of BA-confirmed cases, supporting its potential role as a screening modality in resource-limited settings. Histopathological analysis revealed a significantly higher prevalence of liver fibrosis in patients referred after 60 days (p=0.002), indicating disease progression. Only 9 patients underwent Kasai portoenterostomy, with 8 fatalities due to ascending cholangitis.

These findings emphasize the urgent need for earlier detection and referral to improve outcomes in infants with BA.