A newly described clinical strategy from Texas Children's Hospital and Baylor College of Medicine offers a practical approach to diagnosing biliary atresia (BA) earlier, potentially improving long-term outcomes for affected infants. Published in the World Journal of Pediatric Surgery (DOI: 10.1136/wjps-2025-001142), the pathway combines direct or conjugated bilirubin (DB/Bc) measurements with a feeding abdominal ultrasound exam to identify infants who need urgent evaluation for BA while reducing unnecessary invasive testing.
Biliary atresia is a rare condition where the bile ducts outside the liver are blocked or absent, leading to bile accumulation and progressive liver damage. Early treatment with Kasai portoenterostomy (KP), ideally before 30–45 days of life, offers the best chance of preserving the native liver and delaying or avoiding transplantation. However, diagnosis is often delayed beyond 60 days due to the subtlety of early signs, such as jaundice that can be mistaken for common newborn issues.
The proposed pathway begins with DB/Bc measurements in the newborn nursery and during early outpatient visits. Evidence suggests that 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 encouraged to test DB/Bc at 2–4 weeks for infants with persistent jaundice, pale stools, or previous high levels, following American Academy of Pediatrics guidance. Infants with elevated DB/Bc then undergo a feeding abdominal ultrasound, where the infant feeds before or during imaging to enhance visualization of the duct at the hilum (DaH). 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, prompting definitive evaluation, while other findings support continued outpatient monitoring.
The approach aims to make early BA evaluation more actionable for the entire care team, from nursery providers to specialists. By using DB/Bc screening and a feeding ultrasound that avoids fasting, the pathway could reduce the burden on families and healthcare systems. The authors hope that sharing this strategy will encourage feedback and adaptation in other practice settings. Future studies are needed to evaluate implementation, cost-effectiveness, and performance across diverse centers.
This research was supported 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), along with philanthropic contributions. The work was published in World Journal of Pediatric Surgery, an open-access, peer-reviewed journal indexed in PubMed and other databases.
