VP10.11: Aorta‐porto‐umbilical Vein Shunt in Association with Pulmonary Sequestration

A 31-year-old woman (gravida 2, para 1) presented at 30 weeks' gestation to the emergency room following the onset of left lower abdomen pain. The pregnancy had been uncomplicated. Ultrasonography at the time showed polyhydramnios and hydroptic changes, in addiction to an extralobar pulmonary sequestration on the left side of the thorax. Absent end-diastolic flow was seen in the umbilical artery. The woman was admitted for induction of fetal lung maturation and subsequent ultrasonography evidenced an abdominal arteriovenous shunt, compatible with a systemic aorta-porto-umbilical shunt with biphasic arterial flow (image 1). The ductus venosus was patent. A Caesarean section was performed the following day at 30 weeks and 6 days, resulting in a 2000 gr baby girl with an Apgar score of 1/2/3. The newborn died following the onset of persistent respiratory distress. Congenital hepatic arteriovenous fistulae are very rare (<1:100,000 live births). (1-3) Since Moore et al. categorised in three groups the abnormalities of the intra-abdominal umbilical vein, there have been reports upon congenital defects associated with aorta-porto-umbilical vein fistulas, although it mostly occurs isolated. (1, 2, 4) Zhou et al. described two cases of fetuses with such abnormality in association with left-sided inferior vena cava and there have also been reports of association with duodenal atresia and trisomy 21. (1, 2) To our knowledge, this is the first description of an aorta-porto-umbilical vein fistula associated with a pulmonary sequestration. Supporting information can be found in the online version of this abstract Supporting Information Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.