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Endoscopic retrograde cholangiography in the diagnosis of biliary malformations in infants
M B Heyman1, H A Shapiro, M M Thaler
1Department of Pediatrics, University of California Children's Medical Center, San Francisco.
Insights
Endoscopic retrograde cholangiography (ERC) aids in diagnosing infantile biliary issues. This study shows ERC can visualize infant biliary passages, helping differentiate malformations from parenchymal diseases in persistent cholestasis.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Diagnostic Imaging
Background:
- Differentiating infantile biliary malformations from parenchymal liver diseases poses diagnostic challenges.
- Persistent cryptogenic cholestasis in infants requires precise biliary system evaluation.
Purpose of the Study:
- To assess the utility of endoscopic retrograde cholangiography (ERC) using a pediatric side-viewing endoscope for visualizing extrahepatic biliary passages in infants.
- To determine if ERC can aid in diagnosing biliary malformations in infants with persistent cholestasis.
Main Methods:
- Endoscopic retrograde cholangiography (ERC) was performed in 12 infants with persistent cryptogenic cholestasis.
- Visualization of extrahepatic bile ducts was attempted.
- Results were correlated with surgical findings and liver biopsies where applicable.
Main Results:
- Extrahepatic bile ducts were visualized in 4 of 12 infants.
- Complete visualization of the biliary system in one infant excluded biliary atresia and choledochal cyst, with intrahepatic biliary hypoplasia suspected.
- Six of eight infants with non-visualized extrahepatic ducts were diagnosed with biliary atresia at laparotomy; two had neonatal hepatitis.
Conclusions:
- Endoscopic retrograde cholangiography (ERC) can visualize infant biliary passages, aiding diagnosis.
- ERC may serve as a valuable alternative to operative cholangiography in select infants with persistent cholestasis and acholic stools.
Abstract:
The differentiation of infantile biliary malformations from primary parenchymal diseases is difficult. The recent development of a pediatric side-viewing endoscope (PJF Endoscope; Olympus Corporation of America) provided an opportunity to investigate the usefulness of endoscopic retrograde cholangiography (ERC) for precise visualization of the extrahepatic biliary passages in infants with persistent cryptogenic cholestasis. ERC was performed in 12 patients, with visualization of the existing extrahepatic bile ducts in 4. The entire biliary system was visualized in one, excluding extrahepatic biliary atresia and choledochal cyst. The reduced caliber of the intrahepatic bile ducts and histological observations in a percutaneous liver biopsy supported the diagnosis of intrahepatic biliary hypoplasia in this case. An intact hepatic portochole cystostomy was documented in one, although the intrahepatic biliary system was not delineated. Atresia of the hepatic bile ducts proximal to the gallbladder was documented in two. Of the eight patients in whom extrahepatic bile ducts were not visualized by ERC, six had extrahepatic biliary atresia confirmed at exploratory laparotomy. The papilla of Vater could not be located in four of these six infants. The remaining two had neonatal hepatitis. ERC may offer a useful alternative to operative cholangiography in selected infants with persistent cholestasis and acholic stools.
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