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Prenatal diagnosis of Abernethy malformation by three‐dimensional ultrasonography

Abernethy malformation is an abnormal portosystemic shunt that was first described in 1793 as a postmortem finding1. Prognosis is determined primarily by the extent of the portal system development2, which classifies the malformation into one of two types: Type 1, or classical Abernethy malformation, in which portal blood is diverted completely into the inferior vena cava (IVC) with absence of the portal vein, and is associated with hepatic failure and hepatic tumors; and Type 2 in which the portal vein is intact, and thus conveys a better prognosis3. A 25-year-old primigravida was referred to our center at 23 weeks’ gestation due to detection of an abnormal blood vessel of large diameter in the fetal abdomen. On two-dimensional (2D) ultrasound examination (GE Voluson E8 Expert, GE Medical Systems, Zipf, Austria) the vessel had a monophasic Doppler waveform and direction of flow was towards the pelvis. The ductus venosus was present and the IVC was dilated. Analysis of three-dimensional (3D) color Doppler volumes with glass-body mode and four-dimensional (4D) spatiotemporal imaging correlation (STIC) showed that the aberrant vessel originated from the intrahepatic segment of the umbilical vein (UV) (Figure 1). The vessel passed above the left common iliac vein and, following a U-turn course in the fetal pelvis, anastomosed with the IVC at the level of its bifurcation (Figure 2). With 3D power Doppler rendered images we were able to conclude that the vessel originated from the intrahepatic segment of the UV, coursing sharply to the left in the fetal abdomen, anterior to the dilated IVC. Initially, on 2D ultrasound, the aberrant vessel was assumed incorrectly to originate from the IVC. This important differentiation established the diagnosis of a portosystemic shunt, and 3D power Doppler and 4D STIC rendered images enabled visual reconstruction of the complex course of the vessel in the fetal pelvis.