New Biliary Atresia Guidance Released by American Academy of Pediatrics

Sanjiv Harpavat, MD, PhD

Credit: American Liver Foundation

New guidance from the American Academy of Pediatrics (AAP) is providing pediatric primary care providers with a novel strategy to facilitate the early identification of biliary atresia in infants.¹

Intended to be used between 2 and 4 weeks of life at the “By 1 month” well-child visit in the Bright Futures/AAP “Recommendations for Preventive Pediatric Health Care,” the strategy involves examining infants’ eye color, stool color, and prior laboratory results to determine whether measurement of a direct or conjugated bilirubin level is warranted.¹

A disease of the bile ducts affecting infants, biliary atresia impacts 1 in 8,000 to 1 in 18,000 live births worldwide, with symptoms typically appearing between 2 and 6 weeks after birth. The trapped bile can quickly cause liver damage and cirrhosis, eventually leading to liver failure, but can be prevented by early diagnosis and surgery.^2,3

“By identifying infants earlier, PCPs have a unique opportunity to improve outcomes and help reduce the tremendous liver transplant burden of biliary atresia,” Sanjiv Harpavat, MD, PhD, an associate professor in the department of pediatrics at Baylor College of Medicine, and colleagues wrote.¹

Guidance authors divided the strategy into 3 key steps: identifying jaundice beyond 2 weeks of life, examining stool appearance, and assessing direct or conjugated bilirubin levels.¹

According to the guidance, jaundice beyond 2 to 3 weeks of life warrants consideration of testing for direct or conjugated bilirubin levels, with authors citing joint recommendations from North American and European pediatric gastroenterology societies. Of note, other signs of biliary atresia such as hepatomegaly and splenomegaly may not be present in the early stages, underscoring the importance of assessing jaundice on physical examination.¹

Even in the absence of jaundice, guidance authors note that PCPs should proceed to Step 2 of the strategy because the infant could still have biliary atresia because jaundice may not yet be present in the early stages or may not be noticed during the examination.¹

The guidance also states that pale, gray, or white stools at any time warrant consideration of testing for direct or conjugated bilirubin levels, because lighter stools occur in biliary atresia and other liver diseases when conjugated bilirubin in bile fails to reach the intestine and color the stools.¹

Like the jaundice assessment in Step 1 of the strategy, the authors suggest that PCPs continue to Step 3, even in the absence of lighter stools, which may not occur until later stages of biliary atresia or may go unnoticed.¹

The third and final step of the strategy involves determining whether direct or conjugated bilirubin levels were previously checked. According to the guidance, a previously “high” direct or conjugated bilirubin level warrants consideration of repeat testing, with “high” defined as exceeding the laboratory’s derived reference range.¹

Authors recommend reviewing prior laboratory results from the newborn inpatient unit and/or outpatient setting, as many infants have total bilirubin levels checked and levels of direct or conjugated bilirubin measured in accordance with AAP recommendations to assess risk for bilirubin encephalopathy. They note the initial direct or conjugated bilirubin level is the most important result because it will be “high” in biliary atresia starting at birth.¹

According to the strategy, jaundiced eyes, pale stools, or a high initial laboratory result warrants consideration of testing for direct or conjugated bilirubin levels. However, the authors note a blood draw can be avoided if any prior direct or conjugated bilirubin level was normal, defined as falling within the laboratory-derived reference range, or if prior abnormal direct or conjugated bilirubin levels were equivalent or decreasing over time.¹

Upon drawing a direct or conjugated bilirubin level, the guidance asserts that a value ≥1 mg/dL warrants urgent consultation with a pediatric gastroenterologist, who may recommend additional testing, refer to an outpatient gastroenterology setting, or expedite the evaluation with an inpatient admission to the emergency department.¹

The guidance also recommends scheduling the well-child visit at 2 weeks to facilitate earlier identification of biliary atresia and help ensure that treatment with the Kasai portoenterostomy occurs before 30 days of life. Prior to this 1-month well-child visit, PCPs can also use the first 2 patient encounters in the Bright Futures/AAP periodicity schedule—the “Newborn” and “3- to 5-day” encounters—to help identify infants with biliary atresia.¹

Guidance authors recognize the development of a universal screening strategy for biliary atresia as a future goal, describing the potential utility of a stool color card program and newborn direct or conjugated bilirubin screening before hospital discharge.¹

References

1. ^{Harpavat S, Aucott SW, Karpen SJ, et al; American Academy of Pediatrics, Section on Gastroenterology, Hepatology, and Nutrition; Committee on Fetus and Newborn; Section on Surgery. Guidance for the Primary Care Provider in Identifying Infants With Biliary Atresia by 2–4 Weeks of Life: Clinical Report. Pediatrics. https://doi.org/10.1542/peds.2024-070077}
2. ^{American Liver Foundation. Biliary Atresia. July 29, 2024. Accessed February 20, 2025. https://liverfoundation.org/liver-diseases/pediatric-liver-information-center/pediatric-liver-disease/biliary-atresia/}
3. ^{Cincinnati Children’s. Biliary Atresia. April 2022. Accessed February 20, 2025. https://www.cincinnatichildrens.org/health/b/biliary-atresia}