Visualization of intrahepatic portosystemic shunt and intra-abdominal umbilical vein varix using fetal ultrasonography with HDlive flow imaging

Excerpt

A 39-year-old Japanese multiparous woman presented to our hospital at 25 weeks of gestation for evaluation of fetal anomalies. On examining her using fetal ultrasonography (Voluson E10; GE Healthcare Japan, Tokyo, Japan), we observed fetal intra-abdominal umbilical vein varix (FIUVV), with the maximum diameter of the vein being 11 mm. At 28 weeks of gestation, an aneurysmal vascular enlargement in the right liver was first detected. In addition to conventional two-dimensional (2D) ultrasound imaging, three-dimensional (3D) HDlive flow imaging revealed that the blood flow of the portal vein was directly associated with the bifurcated hepatic vein through the intra-right liver aneurysmal lesioned portion (Fig. 1a, b). Based on these findings, we diagnosed the vascular anomaly as congenital intrahepatic portosystemic shunt (IPSS). Amniotic fluid chromosomal examination confirmed trisomy 21 in the fetus. At 35 + 5 weeks of gestation, an emergent cesarean section was performed due to a non-reassuring fetal status. A 1763-g female infant was delivered with an Apgar score of 8 and 9 at 1 and 5 min, respectively. Postnatal 2D ultrasound and CT scan indicated IPSS, similar to the prenatal diagnosis (Fig. 1c). The presence of a bifurcated hepatic vein was confirmed postnatally and was considered a vascular anomaly. During the neonatal period, no significant elevation in the galactose, total bile acid, or ammonia levels was determined on serial blood tests.

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