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A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice
Tim Jancelewicz1, Rebecca Barmherzig1, Catherine T-S Chung2
1Division of Pediatric Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Insights
A new algorithm helps quickly rule out biliary atresia (BA) in infants with jaundice. This approach uses specific lab values and imaging to avoid unnecessary invasive tests and speed up diagnosis for cholestatic infants.
Area of Science:
- Pediatric Gastroenterology
- Neonatal Jaundice Diagnosis
- Hepatobiliary Disorders
Background:
- Neonatal cholestasis diagnosis often involves multiple tests, potentially delaying critical interventions for conditions like biliary atresia (BA).
- An optimized diagnostic pathway is needed to reduce risk, cost, and delays in identifying BA in infants.
- This study aimed to develop a rapid and accurate algorithm to exclude BA in infants presenting with cholestatic jaundice.
Purpose of the Study:
- To create an effective diagnostic algorithm for identifying infants with cholestatic jaundice who do not have biliary atresia.
- To minimize invasive procedures and reduce the likelihood of unnecessary surgeries in the diagnostic workup of neonatal cholestasis.
- To establish a reliable method for the early exclusion of BA, thereby improving patient outcomes.
Main Methods:
- Retrospective analysis of diagnostic workup in infants with and without BA, comparing those who underwent hepatobiliary iminodiacetic acid (HIDA) scans.
- Inclusion criteria focused on patients born between 2000-2010, excluding those over 100 days old at assessment.
- Sensitivity and specificity analyses were conducted on predictive variables to construct the diagnostic algorithm.
Main Results:
- The study included 45 BA patients and 167 non-BA patients.
- Key indicators for excluding BA included conjugated bilirubin <2.5mg/dL, gamma-glutamyl transpeptidase <150U/L, positive HIDA excretion, or normal percutaneous cholangiogram.
- The developed algorithm demonstrated a negative laparotomy rate ranging from 3-22% for excluding BA.
Conclusions:
- A proposed screening algorithm enables efficient exclusion of BA in infants with conjugated hyperbilirubinemia.
- The algorithm minimizes invasive testing and carries a low risk of negative laparotomy.
- Further prospective evaluation is required to confirm the algorithm's diagnostic accuracy and its impact on healthcare costs and patient outcomes.
Background:
Neonates with cholestasis may undergo many tests before biliary atresia (BA) or an alternative diagnosis is reached, and delayed intervention may worsen outcomes. An optimal diagnostic approach to reduce risk, cost, and delay has yet to be defined. The purpose of this study was to develop an algorithm that rapidly and accurately excludes BA for infants with cholestatic jaundice.
Methods:
A single-center retrospective comparison of diagnostic workup was made between cholestatic infants with BA, and those without BA who underwent hepatobiliary iminodiacetic acid (HIDA) scan during admission. Patients were born between 2000 and 2010 and those older than 100days at assessment were excluded. Sensitivity and specificity analysis of predictive variables was performed and an algorithm constructed.
Results:
There were 45 BA and 167 non-BA patients. Some variables were 100% sensitive for the exclusion of BA: conjugated bilirubin <2.5mg/dL, gamma-glutamyl transpeptidase <150U/L, excretion on HIDA, or a normal percutaneous cholangiogram. Clinical variables and ultrasound were less useful as screening tests owing to low specificity and sensitivity, respectively. Liver biopsy was 98% sensitive and 84% specific in the diagnosis of BA. An algorithm was constructed that rules out BA with a negative laparotomy rate of 3-22%.
Conclusion:
We propose a screening algorithm for infants with conjugated hyperbilirubinemia that permits efficient exclusion of BA with minimal invasive testing and with a low risk of negative laparotomy. This algorithm now requires prospective evaluation to determine its diagnostic accuracy and its ability to reduce hospital costs, patient morbidity, and time to Kasai portoenterostomy in patients with BA.
