Korean J Pediatr Gastroenterol Nutr.  2011 Jun;14(2):122-129. 10.5223/kjpgn.2011.14.2.122.

Early Exclusive Diagnosis of Biliary Atresia among Infants with Cholestasis

• Choe BH

• KMID: 2276531
• DOI: http://doi.org/10.5223/kjpgn.2011.14.2.122

Abstract

The persistence of jaundice beyond the first 2 weeks of life require further investigation and this can be determined if the conjugated bilirubin levels are greater than 1.5 mg/dL or greater than 20% of the total bilirubin level. There is a diverse differential diagnosis for the cause of neonatal cholestasis due to hepatobiliary disease including biliary atresia, which eventually leads to liver cirrhosis if uncorrected before 60~80 days of life. Long-established initial studies include abdominal ultrasonography, hepatobiliary scintigraphy and liver biopsy, but better diagnostic methods are needed. Promising new options are described including MRCP (magnetic resonance cholangiography), ERCP (endoscopic retrograde cholangiography), and PCC (percutaneous cholecysto-cholangiography). Though no single test can differentiate biliary atresia from other neonatal cholestasis with confidence, a combination of diagnostic methods is usually consistently beneficial. By excluding biliary atresia as early as possible, the risk of unnecessary explolaparotomy with intraoperative cholangiography is decreased. Further evaluation would be required for the diagnosis of neonatal cholestasis after excluding biliary atresia.

Keyword

Newborn; Cholestasis; Jaundice; Biliary atresia; Hepatitis; Diagnosis; Cholangiography

MeSH Terms

Biliary Atresia
Bilirubin
Biopsy
Cholangiography
Cholangiopancreatography, Endoscopic Retrograde
Cholestasis
Diagnosis, Differential
Hepatitis
Humans
Infant
Infant, Newborn
Jaundice
Liver
Liver Cirrhosis
Bilirubin

Figure

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  Fig. 1. Classification of cholestasis in infants. *NH: neonatal hepatitis; Ds: disease; UTI: urinary tract infection; PFIC: progressive familial intrahepatic cholestasis; TPN: total parental nutrition.

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  Fig. 2. Suggested approach for the exclusion of biliary atresia in infants with cholestasis. *Bil (D): direct bilirubin; IBEM: inborn error of metabolism; MS: mass spectrometry; AA: amino acid analysis; USG: ultrasonograpghy; NS: nonspecific finding; Bx: biopsy; Op: operation; BA: biliary atresia.

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