Spiegel Efrat, Nicholls-Dempsey Laura, Czuzoj-Shulman Nicholas, Abenhaim Haim Arie
Department of Obstetrics and Gynecology, Jewish General Hospital, McGill University, Montreal, QC, Canada.
Centre for Clinical Epidemiology and Community Studies, Jewish General Hospital, Montreal, QC, Canada.
J Matern Fetal Neonatal Med. 2022 May;35(9):1683-1689. doi: 10.1080/14767058.2020.1767574. Epub 2020 Jul 13.
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.
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.
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).
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.
埃勒斯-当洛综合征（EDS）是一种由胶原蛋白合成异常引起的遗传性结缔组织疾病。关于其对妊娠的影响知之甚少。本研究的目的是评估患有EDS的女性的妊娠结局。
我们使用来自美国医疗成本和利用项目全国住院样本数据库进行了一项基于人群的回顾性队列研究。该研究纳入了1999年至2014年间分娩的女性。我们测量了不同时间EDS的患病率，并将患有EDS的女性与无EDS的普通产科人群的基线、产科和新生儿结局进行了比较。使用无条件逻辑回归模型计算EDS对孕产妇和新生儿结局的调整效应。
妊娠期间EDS的总体患病率为每10万例分娩中有7例，在16年的研究期间呈上升趋势（P<0.0001）。患有EDS的女性更可能是白种人，属于较高收入四分位数，且吸烟。患有EDS的女性妊娠与早产（比值比[OR]=1.47，95%置信区间[CI]=1.18-1.82）、宫颈机能不全（OR=3.11，95%CI=1.99-4.85）、产前出血（OR=1.71，95%CI=1.16-2.50）、前置胎盘（OR=2.26，95%CI=1.35-3.77）和孕产妇死亡（OR=9.04，95%CI=1.27-64.27）相关。患有EDS的孕妇更可能通过剖宫产分娩（OR=1.55，95%CI=1.36-1.76），产后住院时间更长（>7天）（OR=2.82，95%CI=2.08-3.85），且新生儿有宫内生长受限（OR=1.81，95%CI=1.29-2.54）。
妊娠期间的EDS是一种高危情况，孕产妇发病率和死亡率以及新生儿发病率均增加。应考虑采取预防早产的措施和进行高危妊娠咨询。
