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Extra-hepatic portal venous obstruction is most common cause of portal hypertension in children. Apart from variceal formation in gastric and esophageal mucosal layer, it has been postulated that external pressure and or ischemic injury play a role in portal biliopathy. We report an unusual case of a 6 month old infant who presented to us with clinical features suggestive of extra-hepatic biliary atresia; diagnosed as EHPVO with portal biliopathy, managed surgically.

pediatric review: international journal of pediatrics research

Extra-Hepatic portal venous obstruction with portal biliopathy in infant presenting as neonatal cholestasis: a rare case report and review of literature