Guideline for the Evaluation of Cholestatic Jaundice in Infants
Publication Date: January 1, 2017
Last Updated: March 14, 2022
Recommendations
EVALUATION OF THE JAUNDICED INFANT
1. Any formula-fed infant noted to be jaundiced after 2 weeks of age should be evaluated for cholestasis with measurement of total and conjugated (direct) serum bilirubin. (Category IA)
Depending upon local practice, breast-fed babies that appear otherwise well may be followed clinically until 3 weeks of age, at which time if they appear icteric should then undergo serum evaluation of total and conjugated (direct) serum bilirubin.
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2. Measurements of serum bilirubin should always be fractionated into unconjugated (indirect) or conjugated (direct) hyperbilirubinemia (Category IA)
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3. Conjugated (direct) hyperbilirubinemia (>1.0 mg/dL, 17 μmol/L) is considered pathological and warrants diagnostic evaluation. (Category IA)
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PHYSICAL EXAMINATION
4. A thorough physical examination is crucial to the proper evaluation of the jaundiced infant. Attention to hepatomegaly, splenomegaly, and ill appearance warrants special considerations. (Category IA)
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5. Direct visualization of stool pigment is a key aspect of a complete evaluation of the jaundiced infant. (Category IA)
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LABORATORY EVALUATION
6. The abdominal ultrasound is useful in excluding choledochal cyst or gallstone disease causing extrahepatic bile duct obstruction. It may demonstrate an absent or abnormal gallbladder, or other features suggestive, but not diagnostic, of BA. (Category IA)
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7. Limited specificity precludes the use of the HBS scan as a stand-alone test in making a definitive diagnosis of BA. (Category IB)
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Definitively demonstrated bile flow by selective use of HBS may be of value in excluding BA. (Category IB)
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8. Limited specificity of MRCP, ERCP, PTCC provides a limited role in the general guidance to caregivers toward diagnosing BA in the present era. ()
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9. In the hands of an experienced pediatric pathologist, histopathological findings of bile duct proliferation, bile plugs, and fibrosis in an appropriately timed liver biopsy is the most supportive test in the evaluation of the infant with protracted conjugated hyperbilirubinemia. (Category IB)
Diseases other than BA that cause cholestasis can be determined via histologic examination of the liver.
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INTRAOPERATIVE CHOLANGIOGRAM
10. Evaluation by intraoperative cholangiogram and histological examination of the duct remnant is considered the gold standard to diagnose biliary atresia. (Category IA)
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Recommendation Grading
Disclaimer
Overview
Title
Guideline for the Evaluation of Cholestatic Jaundice in Infants
Authoring Organization
Consensus and Physician Experts
Publication Month/Year
January 1, 2017
Last Updated Month/Year
August 2, 2023
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Inclusion Criteria
Female, Male, Infant
Health Care Settings
Ambulatory, Hospital, Outpatient
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Assessment and screening, Diagnosis, Management, Treatment
Diseases/Conditions (MeSH)
D007567 - Jaundice, Neonatal
Keywords
biliary atresia, hepatoportoenterostomy, Kasai, neonatal cholestasis, neonatal jaundice, radionuclide scan