Abstract
Background: The agenesis of the ductus venosus (DVA) is a rare condition with a variable prognosis that relies partly on the presence of associated conditions. Therefore, the prenatal evaluation should include a careful examination of fetal circulation, particularly the umbilical and portal venous malformations.Methods In this study, we describe four cases of DVA diagnosed at our institution. For each case, we access the patient’s files in order to extract the following information: gestational age, umbilical vein connection, pre-natal imaging findings, gestational age at delivery or at pregnancy termination, fetal outcomes, post-natal imaging findings, post-mortem findings and karyotype.Results From the four cases included, two were diagnosed in the first trimester and the other two cases in the second trimester of gestation. Prenatal ultrasound studies revealed an intrahepatic shunt in one case and an extrahepatic shunt in three cases. In one case, the karyotype was not performed, whereas the other three had a normal karyotype. Cardiac anomalies were found in three of the four fetuses. All but one case presented with extracardiac abnormalities. None of the cases developed hydrops. Two cases are currently alive and well, one termination of pregnancy occurred at 25 weeks of gestation and one case died at 14 days during surgical correction of a complex cardiac malformation.Conclusions Clinicians should be aware of different and important findings during the fetal examination, which can be indicative of a DVA, and, when suspected, serial revaluations should be scheduled in order to identify any malformation.
Highlights
The agenesis of the ductus venosus (DVA) is a rare condition with a variable prognosis that relies partly on the presence of associated conditions
The agenesis of the ductus venosus (DVA) is a rare anomaly first published in 1826 by Mende who reported a case of direct drainage of an umbilical vein into the right atrium during an autopsy of a stillborn infant [1]
With the widespread use of ultrasonographic techniques and their improvement over the years, a more careful examination of the fetal circulation, the umbilical and portal venous malformations, is performed prenatally which led to the increase number of DVA cases published in the literature
Summary
The agenesis of the ductus venosus (DVA) is a rare anomaly first published in 1826 by Mende who reported a case of direct drainage of an umbilical vein into the right atrium during an autopsy of a stillborn infant [1]. Paltauf in 1888, describes the second case of DVA in an infant born with severe hydrops and portal congestion without the ductus venosus. This information was only obtained in the post-mortem examination [2]. With the widespread use of ultrasonographic techniques and their improvement over the years, a more careful examination of the fetal circulation, the umbilical and portal venous malformations, is performed prenatally which led to the increase number of DVA cases published in the literature. In spite of the new and better technologies, this is still a rare condition with a reported prevalence of 1 in 2532 [7] and 1 in 556 foetuses [8], and with a variable prognosis that relies partly on the presence of associated conditions
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