C-sections may reduce risks for pregnant women with vEDS: Study
Patients at high risk of uterine rupture, blood vessel issues, death
Women with vascular Ehlers-Danlos syndrome (vEDS) are at high risk of uterine rupture, issues in blood vessels, and death during pregnancy, particularly in the period around delivery.
“To avoid expulsive efforts [active pushing], a caesarean section should be scheduled at 37 weeks of gestation,” researchers wrote.
The study, “Vascular Ehlers-Danlos syndrome and pregnancy: A systematic review,” was published in the BJOG: An International Journal of Obstetrics and Gynaecology.
vEDS is a type of Ehlers-Danlos syndrome (EDS), a group of genetic diseases characterized by weakness in the connective tissue that holds tissues together. This EDS subtype is marked by thin, easily bruised skin and fragile arteries, muscles, and internal organs. vEDS is generally considered the most severe form of EDS due to a high risk of serious bleeding.
Managing pregnancy in women with vEDS poses challenges
Managing pregnancy in women with vEDS is challenging, because they are at a higher risk of complications, particularly during the perinatal period, or around the time of delivery. However, few studies have been published about mortality and morbidity rates in pregnant women with vEDS.
Researchers in France addressed this knowledge gap by looking at 31 studies, including six observational studies that covered a total of 412 pregnancies, and 25 case reports.
One study showed the proportion of women with infertility was significantly lower among vEDS patients (30%) than in those with hypermobile EDS (47.6%), the most common disease type.
However, the proportion of women whose infertility was related to an unspecified uterine anomaly was higher in vEDS patients (22.2%) than in those with hEDS ( 2.9%) or classical EDS (9.7%). Similar to vEDS, classical EDS is caused by mutations in genes responsible for producing collagen, an important protein in conjunctive tissue.
During the first trimester of pregnancy, the rate of early spontaneous miscarriage ranged from 5.5% to 46.2%, with no significant differences between EDS types reported in one study. Among the total 412 pregnancies in the observational studies, the babies were born at full term in 75.2% and prematurely (earlier than 37 weeks) in 7.3%.
In one study that reported the method of delivery in 99 women, nearly two-thirds of the babies were born by vaginal delivery whereas about one-third were born by cesarean section (65.7% vs. 34.4%). Most cesarean sections, also known as C-sections, had been scheduled.
Major life-threatening complications, including vascular dissection (tear) and uterine or intestinal rupture without death, were more likely after delivery (3%) than during pregnancy (1.8%) or delivery (0.6%).
Overall rate of maternal death from 5 studies was 5.7%
The overall rate of maternal death from five studies was 5.7%, corresponding to 22 women, and occurred more frequently during the perinatal period, particularly during delivery (3.1%). In most cases, maternal death was caused by a blood vessel rupture or dissection (63.6%), and less frequently by ruptures in the uterus (31.8%) or in other organs (4.6%).
Nine maternal deaths were reported in the case reports, related to a blood vessel rupture or dissection. At the time of death, none of these women had been diagnosed with vEDS.
A total of 16 patients, most with a family history suggestive of vEDS, were diagnosed before delivery. Ten had a scheduled C-section and five gave birth by vaginal delivery. One woman died six days after vaginal delivery due to a rupture of a kidney artery.
“It is therefore essential to ask patients about their family history at the start of pregnancy, so as not to miss a possible diagnosis of vEDS,” the team wrote.
Also, as women with vEDS are at risk of death or major complications during or after delivery, clinicians might “systematically prescribe a cesarean section before labour for pregnant women with vEDS,” the researchers added.
Such an approach was mentioned in case reports. In some, the risk of severe or fatal complications made clinicians schedule a C-section, sometimes before 37 weeks of gestation. “We note that none of the women who underwent a scheduled caesarean section died,” the investigators wrote.
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