A case of of congenital partial agenesis of the fetal portal veins

Methods A 35-year-old G3P1 patient, referred to our division of Maternal Fetal Medicine at 29 weeks of gestation due to fetal growth retardation. On the first examination, she was afebrile (36. 6’ C), and had normal blood pressure (100/70 mmHg). Past medical or family history revealed no significance and she had no drug, alcohol or tobacco use. Mild cardiomegaly, splenomegaly and dilatation of umbilical vein were found on the ultrasound scan and fetal growth was restricted for gestational age. The ductus venous was visible and accepted as normal with triphasic blood flow pattern on Doppler ultrasound. Amniotic fluid volume was within normal ranges. Fetal cardiac echocardiography was performed and normal fetal cardiac anatomy was demonstrated on multiple planes. The free cell DNA test was performed and results showed low risk for genetic abnormalities. At 38 weeks of gestation, a 1900 grams term male infant, with an APGAR score of seven at 1st minute and score of eight at 5th minute was delivered by cesarean section. Abdominal ultrasound was performed on the first day of life and it revealed splenomegaly and a dilated umbilical vein passing through the liver and then draining to vena cava inferior. The portal vein was interrupted at the portal hilum. There was no left portal vein branch and the right portal vein was observed as rudimentary. There were intrahepatic collateral veins between the umbilical and the portal vein. Therefore, patient was diagnosed with ‘Abernity Malformations’ (congenital partial agenesis of the portal veinstype 2).