EP10.16: Prenatal ultrasonographic features and outcomes of 19 cases of congenital intrahepatic portosystemic venous shunts

To investigate the prenatal ultrasonographic features and case characteristics of the congenital intrahepatic portosystemic venous shunt (IHPSS) diagnosed during the fetal period and analyse its prognosis. We conducted a retrospective cohort study of patients diagnosed with IHPSS between 2016 and 2021. IHPSS was defined as an abnormal connection between the fetal intrahepatic portal and the hepatic veins. In this study, 19 fetuses were identified, including 12 cases of single shunt and 7 cases of multiple shunts, with a gestational age of 33.8 ± 4.5 (range 25–40) weeks at diagnosis. In the single-shunt group, the origin position of the shunts was all from the left branch of the portal vein (LPV), whereas in the multiple-shunt group, the origin position of the shunts was from the LPV in six cases. Common concomitant intrauterine abnormalities of IHPSS include fetal growth restriction (47.4%) and fetal cardiac enlargement (21.1%). The postnatal manifestations of IHPSS include biochemical abnormalities (increased gamma-glutamyl transferase and bilirubin levels), neonatal hypoglycemia, neonatal hyperammonemia, pulmonary hypertension, multiple intrahepatic hyperechoic nodules, and cutaneous hemangiomas. Spontaneous closure of shunts occurred in ten cases, and the mean time to shunt closure was8.1 months (1–28 months). Most IHPSS found during the fetal period is located in the left branch of the portal vein, and the gestational age at diagnosis is usually in the late second or third trimester. Spontaneous closure of shunts can occur in most livebirths, and the prognosis is good.