Selective Use of Endoscopic Retrograde Cholangiopancreatography in the Diagnosis of Biliary Atresia in Infants Younger Than 100 Days

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Objectives: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.Patients and 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.

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