Alagille Syndrome Mimicking Biliary Atresia

Abstract

The clinical presentation of jaundice, dark urine, persistently acholic stools, hepatosplenomegaly and a rudimentary, non-contractile gall bladder on ultrasound in an infant raises a suspicion of biliary atresia (BA). However, Alagille syndrome (AGS) may clinically, radiologically and histopathologically mimic BA and should be suspected in infants with other extrahepatic features.We report a 2.5-month old girl with an initial suspicion of BA who was eventually found to have AGS. We highlight the clinical, biochemical and radiological features that helps one in differentiating between these two conditions.