A newly described clinical strategy from Texas Children's Hospital and Baylor College of Medicine aims to shorten diagnostic delays for biliary atresia (BA), a rare infant liver disease that can rapidly progress to irreversible injury. Published in the World Journal of Pediatric Surgery (DOI: 10.1136/wjps-2025-001142), the review outlines a practical pathway pairing direct or conjugated bilirubin (DB/Bc) measurements with a feeding abdominal ultrasound exam to identify infants needing urgent evaluation while reducing unnecessary invasive testing.
Biliary atresia occurs when extrahepatic bile ducts fail to form properly, leading to bile buildup and progressive liver injury. Early treatment with Kasai portoenterostomy (KP) before 30–45 days of life offers the best chance of delaying or avoiding liver transplantation, yet many infants are diagnosed after 60 days. The disease is difficult to detect because early jaundice can mimic common newborn conditions, and pale stools may not appear immediately.
The proposed pathway begins with DB/Bc testing in the newborn nursery and early outpatient visits. Evidence suggests DB/Bc levels can be elevated within the first 24–48 hours of life in infants with BA, before other clinical signs emerge. Primary care providers are guided to test at 2–4 weeks for infants with persistent jaundice, pale stools, or previous high DB/Bc results, aligning with American Academy of Pediatrics recommendations.
For infants with high DB/Bc levels, a feeding abdominal ultrasound is performed instead of a traditional fasting exam. The infant feeds before or during imaging, which can make the duct at the hilum (DaH) easier to visualize. The exam also measures maximum echogenicity (MxE) near the right portal vein. An MxE greater than 4.0 mm or an absent DaH raises concern for BA and may prompt definitive evaluation.
The authors emphasize that the strategy is designed to make early BA evaluation more actionable for the entire care team, from nursery providers to specialists. By sharing the pathway, they hope other centers will provide feedback and adapt useful components. Potential implications include universal newborn DB/Bc screening to reduce diagnostic delays and address disparities, as well as a less burdensome follow-up process for families, avoiding fasting and potentially reducing reliance on tests requiring anesthesia.
Future studies will need to evaluate implementation, cost-effectiveness, and performance across multiple centers. The research was funded by the NIH National Institute of Diabetes and Digestive and Kidney Diseases, the American Association for the Study of Liver Diseases, the American Liver Foundation, and Biliary Atresia Research and Education, Inc., among others.
