P15.08: Prenatal diagnosis of an extra hepatic umbilicoportosystemic shunt: impact on postnatal management

Congenital agenesis of the portal venous system (CAPVS) with extra hepatic Umbilicoportosystemic (EHUPS) shunt is an extremely rare abnormality that permits the diversion of the portal flow to the systemic circulation without perfusing the hepatic parenchyma. Therefore, toxins and nutrients absorbed by the intestines bypasses the liver and are shunted directly into the systemic circulation. Abnormal Umbilicoportosystemic shunts are direct connection between a high pressure system and low pressure system. The absence of any flow regulation (absent ductus venosus) results in increased pressure in the systemic system that may compromise fetal hemodynamic. We present a case of prenatal diagnosis of extra hepatic umbilicoportosystemic shunt which was confirmed postnatal. The prenatal diagnosis of Porto systemic shunt is important to allow close monitoring as this will impact the perinatal management and postnatal outcome. Supporting information can be found in the online version of this abstract. Fetal ultrasound features included dilated intra-abdominal segment of the umbilical vein, absent ductus venosus, absent right portal vein, and a venous connection between the left portal vein and inferior vena cava. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.