Hydrops fetalis due to agenesis of the ductus venosus: new hepatic histological features.

We describe the clinical course and autopsy findings of a male infant with hydrops fetalis due to agenesis of the ductus venosus. Fetal echocardiography at 27 weeks in gestation demonstrated hydrops fetalis due to unknown causes. The baby was born at 28 weeks in gestation by emergency caesarean section because of preeclampsia and progressive hydrop fetalis but died immediately at birth. The umbilical vein catheter ran an unusual course: left renal vein and inferior vena cava were opacified after postmortem injection of radiopaque dye into the umbilical vein. The autopsy demonstrated agenesis of the ductus venosus without extrahepatic umbilical venous drainage. The type without extrahepatic venous drainage is rare but shows a favorable outcome in general. However, our findings illustrate that in addition to sinusoidal dilatation, some cases may induce significant medial hypertrophy of portal veins, leading to hydrops fetalis and neonatal demise.