TYPE 1B ABERNETHY MALFORMATION IN AN ADOLESCENT MALE: A CASE REPORT

Abstract

Abernethy malformation or congenital extrahepatic portosystemic shunt is a rare genetic disorder characterised by bypass of portal venous blood into the systemic circulation. This rare vascular anomaly is classified into two types based on the pattern of vascular shunting if left untreated, it can lead to severe complications. We report the case of 17 y old male patient admitted in CCU with complaints of shortness of breath and chest pain for 1 w with history of chest fullness and breathlessness. On evaluation, Lab parameters revealed pancytopenia and altered liver profile. CECT of abdomen showed multiple collaterals venous channels with large splenorenal shunt consistent with portal hypertension these findings confirmed a diagnosis of type 1b Abernethy malformation. Patient was managed conservatively with symptomatic medical treatment, no surgical or interventional radiological procedure was performed, after few days patient cardiac parameters were stable with no significant improvement in overall disease condition, discharged with follow-up for further management.