New Clinical Pathway Aims to Accelerate Biliary Atresia Diagnosis in Infants

A new clinical strategy described by researchers from Texas Children's Hospital and Baylor College of Medicine, in collaboration with Stanford University School of Medicine, aims to reduce diagnostic delays for biliary atresia (BA), a rare infant liver disease that can rapidly progress to irreversible liver injury. Published in World Journal of Pediatric Surgery (DOI: 10.1136/wjps-2025-001142), the review details a two-step pathway that pairs direct or conjugated bilirubin (DB/Bc) measurements with a feeding abdominal ultrasound exam. The approach is designed to help clinicians identify infants who need urgent evaluation while reducing unnecessary invasive testing.

Biliary atresia is difficult to detect early because initial jaundice can mimic common newborn conditions, and pale stools may not appear immediately. The disease is thought to begin before birth due to improper formation of extrahepatic bile ducts. After birth, bile accumulates in the liver, causing progressive injury and increasing the likelihood of liver transplantation. Studies have shown that infants treated with Kasai portoenterostomy (KP) before 30–45 days of life have better long-term outcomes, but diagnosis is often delayed beyond 60 days.

The first step of the proposed pathway involves DB/Bc measurements in the newborn nursery and during early outpatient visits. Evidence cited in the review indicates that DB/Bc levels can be elevated within the first 24–48 hours of life in infants with BA, before clear clinical signs or other liver injury markers appear. Primary care providers are guided to test DB/Bc at 2–4 weeks for infants with persistent jaundice, pale stools, or a previous high DB/Bc result, consistent with American Academy of Pediatrics guidance. The second step is a feeding abdominal ultrasound exam for infants with high DB/Bc levels. Instead of requiring fasting, 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. In the workflow, an MxE greater than 4.0 mm or an absent DaH raises concern for BA and may prompt definitive evaluation, while other findings support continued outpatient assessment.

The authors emphasize that the strategy is designed to make early BA evaluation more actionable for the entire care team, from nursery providers and primary care physicians to radiologists, hepatologists, and surgeons. They note that the aim is not to replace specialists' judgment but to provide clearer signals at the moment when time matters most. By sharing the pathway, they hope other centers will provide feedback, test the approach in different practice settings, and adapt useful parts into their own workflows.

The potential implications of the pathway are broad. Universal newborn DB/Bc screening could reduce delays and may also help address disparities in diagnosis by identifying risk before visual signs are missed or misread. The feeding ultrasound approach could make follow-up evaluation less burdensome by avoiding fasting and potentially reducing reliance on tests that require anesthesia or invasive procedures. For families, earlier detection could mean faster treatment decisions and a better chance of preserving the native liver. Future studies will need to evaluate implementation, cost-effectiveness, and performance across multiple centers and healthcare systems.