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Pregnancy outcomes in women with Ehlers-Danlos Syndrome

Spiegel E, Nicholls-Dempsey L, Czuzoj-Shulman N, Abenhaim HA

The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians, 2022

Abstract

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 (p 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.

How strong is the evidence?

Evidence Hub reads this as a cohort study, done in people. Its evidence level is 3 of 5 (Moderate · trial / cohort / secondary analysis). Trials without a large randomized design, cohorts and secondary analyses give moderate evidence.

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