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P05.21: Absent ductus venosus in a fetus resulting in a giant hepatic venous malformation and the Kasabach‐Merrit syndrome

We present the prenatal diagnosis in a fetus of an absent ductus venosus resulting hepatic vascular malformation and congestive heart failure. The infant was delivered at 33 weeks by cesarean section. The neonate presented with congestive heart failure and respiratory distress and with thrombocytopenia, disseminated intravascular coagulation, and hemolytic anemia as seen in the Kasabach-Merrit syndrome. The infant underwent coil embolization of the right hepatic artery and later resection of the right hepatic lobe and the gallbladder. Anatomic and hemodynamic features of the mass resembled a hemangioendothelioma, but histological analysis showed absence of endothelial cells, indicating a venous to venous malformation.