Selective use of endoscopic retrograde cholangiopancreatography in the diagnosis of biliary atresia in infants younger than 100 days.

Objective: We investigated the role and safety of endoscopic retrograde cholangiopancreatography (ERCP) in diagnosing biliary atresia (BA) in prolonged neonatal cholestasis, when standard workup was inconclusive.

Methods: We reviewed notes of 48 cholestatic infants younger than 100 days undergoing ERCP from 1997 to 2007.

Results: Amongst approximately 3300 infants evaluated for liver disease during the study, 224 (6.8%) were diagnosed with BA. Forty-eight children underwent ERCP. Findings at liver biopsy (n=47) included nonspecific cholestasis (n=19, 40%), giant-cell hepatitis (n=12, 26%), "large bile duct obstruction" (n=9, 19%) in the presence of pigmented stools, and mixed cholestatic/hepatitic features (n=7, 15%). ERCP demonstrated a patent biliary tree in 20 infants (42%). BA was confirmed at exploratory laparotomy in all 3 infants (6%) in whom cannulation failed. The remaining 25 infants (52%) also proceeded to exploratory laparotomy, in which BA was confirmed in 22 (46%). Amongst the 20 children in whom ERCP ruled out BA, 8 (17%) had normal biliary anatomy, whilst 12 (25%) had an abnormal biliary tree, including 6 (12.5%) with neonatal sclerosing cholangitis. After ERCP none developed clinical pancreatitis or peritonitis.

Conclusions: ERCP is a safe procedure for diagnosing BA even in the smallest infants with high positive and negative predictive values.