A clinical strategy described by researchers from Texas Children's Hospital and Baylor College of Medicine, along with collaborators at Stanford University School of Medicine, aims to shorten the time to diagnosis of biliary atresia (BA), a rare infant liver disease that can rapidly progress to irreversible injury. The approach, published in World Journal of Pediatric Surgery, pairs direct or conjugated bilirubin (DB/Bc) measurements with a feeding abdominal ultrasound exam to identify infants who need urgent evaluation while reducing invasive testing in those less likely to have BA.
BA is difficult to detect because early jaundice can resemble common newborn conditions, and pale stools may not appear immediately. The disease is thought to begin before birth when extrahepatic bile ducts do not form properly, leading to bile accumulation and progressive liver injury. Infants treated with Kasai portoenterostomy (KP) before 30–45 days of life tend to have better long-term outcomes, but diagnosis is often delayed beyond 60 days. The new pathway addresses this gap by leveraging DB/Bc testing in the newborn nursery and early outpatient visits, as evidence shows DB/Bc levels can be elevated within the first 24–48 hours of life in BA infants, before clinical signs emerge. Primary care providers are also guided to test at 2–4 weeks for persistent jaundice, pale stools, or prior high DB/Bc results, consistent with American Academy of Pediatrics guidance.
The second step uses a feeding ultrasound exam for infants with high DB/Bc levels. Instead of requiring fasting, the infant feeds before or during imaging, making the duct at the hilum (DaH) easier to visualize. 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 may prompt definitive evaluation, while other findings may support continued outpatient assessment.
The authors said the strategy aims to make early BA evaluation more actionable for the entire care team, from nursery providers to specialists. They emphasized that the goal is not to replace clinical judgment but to provide clearer signals when time is critical. By sharing the pathway, they hope other centers will provide feedback and adapt useful parts into their own workflows. The potential implications include reducing diagnostic delays, addressing disparities in care by identifying risk before visual signs are missed, and making follow-up evaluation less burdensome by avoiding fasting and reducing reliance on tests requiring anesthesia. For families, earlier detection could mean faster treatment decisions and a better chance of preserving the native liver.
The review was funded by the NIH National Institute of Diabetes and Digestive and Kidney Diseases (K23DK109207), the American Association for the Study of Liver Diseases, the American Liver Foundation, and Biliary Atresia Research and Education, Inc (BARE), with additional support from philanthropic contributions. The full study is available at https://doi.org/10.1136/wjps-2025-001142.
