Pregnancy outcomes in women with Ehlers-Danlos Syndrome.

PURPOSE

Ehlers-Danlos Syndrome (EDS) is an inherited connective tissue disorder caused by abnormal collagen synthesis. Little is known about its effects on pregnancy. The purpose of this study was to evaluate the pregnancy outcomes in women with EDS.

MATERIALS AND METHODS

We conducted a population-based retrospective cohort study using the Healthcare Cost and Utilization Project Nationwide Inpatient Sample database from the United States. The study included women who delivered between 1999 and 2014. We measured the prevalence of EDS over time, and compared the baseline, obstetrical, and neonatal outcomes among women with EDS to the general obstetrical population without EDS. Unconditional logistic regression models were used to calculate the adjusted effect of EDS on maternal and neonatal outcomes.

RESULTS

The overall prevalence of EDS in pregnancy was 7 per 100,000 births, with the trend increasing over the 16 year study period ( < .0001). Women with EDS were more likely to be Caucasian, belong to a higher income quartile, and smoke. Pregnancies in women with EDS were associated with prematurity, 1.47 (1.18-1.82), cervical incompetence, 3.11 (1.99-4.85), antepartum hemorrhage, 1.71 (1.16-2.50), placenta previa, 2.26 (1.35-3.77) and maternal death, 9.04 (1.27-64.27). Pregnant women with EDS were more likely to be delivered by cesarean section, 1.55 (1.36-1.76), have longer postpartum stays (>7 days), 2.82 (2.08-3.85), and have a neonate with intra-uterine growth restriction, 1.81 (1.29-2.54).

CONCLUSIONS

EDS in pregnancy is a high-risk condition with increased maternal morbidity and mortality, as well as newborn morbidity. Consideration should be given to prematurity preventative measures and high-risk pregnancy consultation.