Portosystemic Fistula Associated With Portal Varix: Prenatal Diagnosis With Successful Embolization at 2 Months of Life

Portosystemic venous malformations are rare vascular anomalies in which portal venous blood drains into the systemic circulation, bypassing the hepatic parenchyma. The estimated prevalence ranges from 1:30000 to 1:50000 live births. The prenatal diagnosis is often challenging due to its embryological variability, the use of oblique ultrasound views, and the need for a profound knowledge of the complex fetal venous system. We report a case of a vascular anomaly characterized by an abnormal connection between the portal vein and a hepatic vein through a dilated varix, which resulted in a diversion of umbilical flow to the right cardiac chambers. Diagnosis was established at 30 weeks of gestation. No associated malformations were identified. The prenatal follow up did not show any signs of cardiac failure despite hemodynamic overload. The postnatal abdominal ultrasound confirmed the fistula. Two months later, the defect was successfully occluded using a percutaneous vascular plug. This case illustrates a successful management approach for a rare portosystemic fistula associated with portal vein varix. The review of the literature of this unusual malformation emphasizes the clinical heterogeneity, the need for a multidisciplinary approach, and diversity of postnatal interventional management.