期刊
PEDIATRIC AND DEVELOPMENTAL PATHOLOGY
卷 -, 期 -, 页码 -出版社
SAGE PUBLICATIONS INC
DOI: 10.1177/10935266231211760
关键词
fetal autopsy; agenesis of ductus venosus; absence of ductus venosus; absent ductus venosus; prenatal ultrasound; extrahepatic shunt; intrahepatic shunt; persistent right umbilical vein; single umbilical artery; embryology
This article presents two cases of ADV fetuses diagnosed through prenatal imaging studies. These cases highlight the heterogeneity of vascular anomalies and embryologic origins captured by the term ADV, as well as the misleading terminology in some ADV cases.
In fetal circulation, oxygenated blood from the placenta flows through the umbilical vein into the ductus venosus (DV), then enters the inferior vena cava, and subsequently reaches the right atrium of the heart. The DV serves as a shunt, allowing this oxygen-rich blood to bypass the liver. The absence of the DV (ADV), also known as agenesis of the DV, is a rare congenital anomaly. Without a DV, blood from the umbilical vein must follow alternative routes to the heart. In ADV cases, blood from the umbilical vein must follow 1 of 2 primary drainage patterns: either an extrahepatic shunt or an intrahepatic shunt. This report details the antenatal ultrasound and postmortem findings of 2 fetuses diagnosed with ADV by prenatal imaging studies. The first case involved a fetus with a persistent right umbilical vein connected directly to the suprahepatic IVC, accompanied by early obliteration of the left umbilical vein and true agenesis of the DV. This fetus also had additional congenital anomalies. In contrast, the second case involved a fetus with a normal left umbilical vein that entered the liver. However, despite an ultrasound diagnosis of absence of the DV, a DV was present, though markedly hypoplastic and probably minimally functional or non-functional. In this case, blood from the umbilical vein likely followed an alternate intrahepatic route through the portal and hepatic veins, before reaching the heart (intrahepatic shunt). These contrasting cases emphasize the heterogeneity of vascular anomalies and embryologic origins captured by the term ADV. Additionally, the terminology of absence or agenesis may be misleading in some purported ADV cases. Specifically, in the second case, the DV was not absent; it was markedly hypoplastic instead. This also appears to be the first reported case of a hypoplastic DV in a fetus. Both cases underscore the importance of effective collaboration and clear communication between maternal-fetal medicine specialists and pathologists.
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