Pregnant women with Ehlers-Danlos syndrome (EDS) have a higher risk of experiencing a number of pregnancy and delivery complications, including premature births, placenta previa, and cervical insufficiency. than those without the disorders, a study reports.
Given these findings, the researchers suggest that physicians should consider implementing preventive measures to avoid premature deliveries, and request additional tests to ensure the safety of the mother and her unborn child.
The study, titled “Pregnancy outcomes in women with Ehlers-Danlos Syndrome,” was published in The Journal of Maternal-Fetal & Neonatal Medicine.
EDS comprises a group of connective tissue disorders caused by defects in the production of collagen or other proteins that are part of the extracellular matrix (ECM), the network that surrounds and supports cells.
Because EDS weakens connective tissue supporting the muscles, tendons, cartilage, and bones throughout the body, patients are often able to extend their joints and stretch their skin beyond normal limits. These clinical manifestations also may have an impact during pregnancy in both the mother and the developing fetus.
However, previous studies investigating the possible impact of EDS on pregnancy and delivery have yielded inconsistent findings. Some reported EDS was associated with a higher risk of complications, while others found no effect.
To clarify the potential impact of EDS on pregnancy and delivery outcomes, investigators at McGill University, in Canada, and their colleagues reviewed the medical records of 1,042 women with EDS who gave birth at a hospital in the U.S. between 1999 and 2014.
Patient records were obtained from the U.S. Healthcare Cost and Utilization Project Nationwide Inpatient Sample (HCUP-NIS) database, which contained a total of 14,513,587 records of women who were admitted for delivery.
Statistical analyses were used to compare the prevalence of pregnancy and delivery complications in women with EDS and in those from the general population (without the disease), as well as to calculate the risk of these complications in people with EDS.
The prevalence of EDS in pregnancy increased gradually over the course of the study, from a prevalence of four cases per 100,000 deliveries in 1999 to 18 cases per 100,000 deliveries in 2014. The overall prevalence was seven cases per 100,000 deliveries.
Compared with women from the general population, those with EDS were more likely to be Caucasians (52.56% vs. 87.30%), smokers (5.17% vs. 11.04%), to have private health insurance (51.97% vs. 65.83%), and to belong to a higher income socioeconomic class (22.62% vs. 30.99%).
Statistical analyses revealed that women with EDS were 3.11-times more likely to have cervical insufficiency, also known as incompetent cervix, a condition in which the tissue of the cervix — the lower part of the uterus that opens to the vagina — weakens, increasing the risk of premature birth.
They also were also found to be 2.26-times more likely to have placenta previa, a condition in which the placenta partially or completely covers the cervix, increasing the risk of severe bleeding during pregnancy and delivery.
Compared with women from the general population, those with EDS also were more likely to have a premature delivery (1.47 times), experience bleeding during pregnancy (1.71 times), deliver by cesarean section (1.55 times), and remain in the hospital for more than seven days following delivery (2.82 times).
Women with EDS also were 9.04 times more likely to die following delivery, compared with women without the disease, and 1.81-times more likely to have a baby with intra-uterine growth restriction, when the baby does not grow as expected.
Based on the results, the team concluded that “EDS in pregnancy is a high-risk condition with increased maternal morbidity and mortality, as well as newborn morbidity.”
“The results of this study merit that consideration be given to using preventative measures [for pre-term delivery] such as progesterone, cervical cerclage, and restricted activity in women with EDS. As well, growth ultrasounds in the third trimester may be warranted as these women are at higher risk of having fetal growth restriction,” the researchers concluded.
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