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

Dr Ajeet Kuma Yadav,Dr Ramkesh Meena,Dr Vivek Dewan,Dr Bhavika Ym,Dr Guruayyappa Ramsingh

doi:10.17511/ijpr.2016.i12.06

Abstract

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.

Highlights

Extra-hepatic portal venous obstruction (EHPVO) is a blockage to the flow of blood in portal vein before it reaches to the liver
There are strictures and dilatations in entire biliary tract and gall bladder secondary to ischemic and compressive effects of portal cavernoma formed in setting of portal hypertension which presents as clinical features of obstructive jaundice, pruritis, pain abdomen (Portal biliopathy) [4,5]
We report a case EHPVO with portal biliopathy (PTB) in a 6 month infant which has never been reported in literature [4,1113]

Summary

Introduction

Extra-hepatic portal venous obstruction (EHPVO) is a blockage to the flow of blood in portal vein before it reaches to the liver. There are strictures and dilatations in entire biliary tract and gall bladder secondary to ischemic and compressive effects of portal cavernoma formed in setting of portal hypertension which presents as clinical features of obstructive jaundice, pruritis, pain abdomen (Portal biliopathy) [4,5]. Several studies have reported that 81100% of cases of EHPVO have portal biliopathy (PTB) but only one fifth of them present as jaundice [4,6,610].

Results

Conclusion