BACKGROUND:In utero vascular disruptions are thought to be associated with a variety of birth defects. This study examined the descriptive epidemiology of several of those defects using data from a large birth defects registry. METHODS: Data on birth defects ascertained from pregnancies in 1996–2002 were obtained from the Texas Birth Defects Registry. Using Poisson regression, we calculated crude and adjusted associations between maternal and infant characteristics and birth defects thought to be related to vascular disruption. We repeated the analysis using isolated cases and cases occurring in mothers <20 years. RESULTS: The most commonly shared pattern was observed for plurality and five defects: large intestinal atresia (PR 3.67; CI: 1.63–7.13), renal agenesis (PR 2.05; CI: 1.55–2.65), transverse limb deficiency (PR 1.85; CI: 1.28–2.57), porencephaly (PR 5.18; CI: 2.40–9.87), and Goldenhar syndrome (PR 3.45; CI: 1.04–8.53). Hispanics had the highest prevalence of gastroschisis (PR 1.21; CI: 1.05–1.40), transverse limb deficiency (PR 1.19; CI: 1.01–1.40), microtia/anotia (PR 2.22; CI: 1.83–2.70), and Poland anomaly (PR 1.90; CI: 1.26–2.93). Male infants were at greatest risk for renal agenesis (PR 1.58; CI: 1.40–1.80), porencephaly (PR 1.66; CI: 1.03–2.72), and Poland anomaly (PR 1.52; CI: 1.05–2.21). CONCLUSIONS: Our study confirmed findings in previous studies, but also uncovered several new associations. Birth Defects Research (Part A), 2008. © 2008 Wiley-Liss, Inc.