A newly described clinical pathway from Texas Children's Hospital and Baylor College of Medicine aims to shorten the diagnostic delay for biliary atresia (BA), a rare infant liver disease where early intervention is critical. Published in World Journal of Pediatric Surgery (DOI: 10.1136/wjps-2025-001142), the strategy pairs direct or conjugated bilirubin (DB/Bc) measurements with a feeding abdominal ultrasound exam to identify infants needing urgent evaluation while minimizing unnecessary invasive tests.
Biliary atresia, which occurs when extrahepatic bile ducts fail to form properly, leads to bile accumulation and progressive liver injury if untreated. The Kasai portoenterostomy (KP) surgery offers the best chance of preserving native liver function when performed before 30–45 days of life, yet many infants are diagnosed after 60 days. The new pathway addresses this by integrating DB/Bc testing in the newborn nursery and at early outpatient visits, as elevated levels can appear within 24–48 hours of birth, before clinical signs emerge. Primary care providers are guided to test at 2–4 weeks for persistent jaundice or pale stools, aligning with American Academy of Pediatrics recommendations.
The second component is a feeding ultrasound, where infants feed before or during imaging to better visualize the duct at the hilum (DaH). The exam also measures maximum echogenicity (MxE) near the right portal vein. In the proposed workflow, an MxE greater than 4.0 mm or an absent DaH raises concern for BA and prompts definitive evaluation, while other findings support continued outpatient assessment. This approach eliminates the need for fasting and may reduce reliance on tests requiring anesthesia or invasive procedures.
The authors emphasize that the strategy is designed to make early BA evaluation actionable for the entire care team, from nursery providers to specialists. By sharing the pathway, they hope to gather feedback and encourage adaptation in diverse practice settings. The implications extend beyond clinical efficiency: universal newborn DB/Bc screening could help address diagnostic disparities by identifying risk before visual signs are missed or misread. For families, earlier detection could mean faster treatment decisions and a better chance of preserving the native liver.
Future research will need to evaluate implementation, cost-effectiveness, and performance across multiple centers and healthcare systems. The study was supported 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.
