Abstract
Case reports and case series have linked umbilical vein varices (UVVs) with adverse pregnancy outcomes. Newer case reports and series suggest better perinatal outcomes in cases with an isolated UVV. The purpose of this literature review is to determine if there is commonality in management, outcomes, and association in pregnancy with UVV and fetal aneuploidy, growth restriction, demise, thrombosis, and turbulent flow. Secondly, we will review the diagnosis, pathophysiology, differential diagnosis, and incidence of UVV. A literature search was undertaken using the search engines PubMed, CINAHL, and Embase. The search terms used were "umbilical vein" AND "varix" OR "umbilical varix" AND "fetal death" OR "fetal demise" AND "guideline" AND "outcome" OR "abnormality" OR "abnormalities". There were 169 abstracts identified. We identified 392 cases of UVV from our literature review. There is a higher risk of fetal anomalies, fetal aneuploidy, and intrauterine fetal demise in pregnancies with UVV. The risk for fetal growth restriction is in the higher range of normal. Turbulence in and thrombosis of the UVV are concerning developments that influence fetal surveillance. Pregnancies with a UVV are more likely to have fetal anomalies, aneuploidy, and IUFD. We propose an antenatal assessment and management plan and recommendations for the timing of delivery. Our aim is to analyze existing literature to identify and combine case reports and case series of umbilical vein varix and analyze fetal and perinatal outcomes.