Abstract：
BACKGROUND: Vascular Ehlers-Danlos syndrome (vEDS) is a hereditary connective tissue disorder associated with an elevated risk of vascular, uterine and digestive complications. Managing pregnancy in this context can be a challenge.
OBJECTIVE: To systematically review the literature data on the complications in pregnancy associated with vEDS.
METHODS: We searched the Pubmed Medline and Embase databases for articles using the following terms \"vascular Ehlers-Danlos syndrome\" or \"vEDS\" AND \"pregnancy\".
METHODS: Women with vEDS.
METHODS: We searched the PubMed® MEDLINE® database for publications evaluating obstetric outcomes in women with vEDS.
RESULTS: A total of 121 publications were screened, with six (accounting for 412 pregnancies) included in our review. Of the women included in this sample, 30% were infertile. The miscarriage rate was 13.8% (57/412) and 8.8% of the live births were premature. Obstetric anal sphincter injuries occurred in 11.3% (23/203) of the deliveries. The maternal mortality rate per pregnancy was 5.7%.
CONCLUSIONS: Women with vEDS present an elevated risk of uterine rupture, vascular events, digestive events and death during pregnancy. Women appear to be most at risk during the peripartum period; to avoid expulsive efforts, a caesarean section should be scheduled at 37 weeks of gestation.
OBJECTIVE: To systematically review the literature data on the complications in pregnancy associated with vEDS.
METHODS: We searched the Pubmed Medline and Embase databases for articles using the following terms \"vascular Ehlers-Danlos syndrome\" or \"vEDS\" AND \"pregnancy\".
METHODS: Women with vEDS.
METHODS: We searched the PubMed® MEDLINE® database for publications evaluating obstetric outcomes in women with vEDS.
RESULTS: A total of 121 publications were screened, with six (accounting for 412 pregnancies) included in our review. Of the women included in this sample, 30% were infertile. The miscarriage rate was 13.8% (57/412) and 8.8% of the live births were premature. Obstetric anal sphincter injuries occurred in 11.3% (23/203) of the deliveries. The maternal mortality rate per pregnancy was 5.7%.
CONCLUSIONS: Women with vEDS present an elevated risk of uterine rupture, vascular events, digestive events and death during pregnancy. Women appear to be most at risk during the peripartum period; to avoid expulsive efforts, a caesarean section should be scheduled at 37 weeks of gestation.
摘要：
背景：血管Ehlers-Danlos综合征（vEDS）是一种与血管风险升高相关的遗传性结缔组织疾病，子宫和消化系统并发症。在这种情况下管理怀孕可能是一个挑战。
目的：系统回顾与vEDS相关的妊娠并发症的文献数据。
方法：我们在PubmedMedline和Embase数据库中搜索了使用以下术语“血管Ehlers-Danlos综合征”或“vEDS”和“妊娠”的文章。
方法：患有vEDS的女性。
方法：我们在PubMed®MEDLINE®数据库中搜索了评估vEDS女性产科结局的出版物。
结果：共筛选了121种出版物，我们的审查中包括6例(占412例怀孕)。在这个样本中的女性中，30%为不育。流产率为13.8%(57/412)，8.8%的活产早产。产科肛门括约肌损伤发生在11.3％（23/203）的分娩中。每次妊娠的产妇死亡率为5.7%。
结论：患有vEDS的女性子宫破裂的风险升高，血管事件,怀孕期间的消化事件和死亡。妇女在围产期似乎是最危险的；为了避免驱逐努力，剖腹产应安排在妊娠37周。
目的：系统回顾与vEDS相关的妊娠并发症的文献数据。
方法：我们在PubmedMedline和Embase数据库中搜索了使用以下术语“血管Ehlers-Danlos综合征”或“vEDS”和“妊娠”的文章。
方法：患有vEDS的女性。
方法：我们在PubMed®MEDLINE®数据库中搜索了评估vEDS女性产科结局的出版物。
结果：共筛选了121种出版物，我们的审查中包括6例(占412例怀孕)。在这个样本中的女性中，30%为不育。流产率为13.8%(57/412)，8.8%的活产早产。产科肛门括约肌损伤发生在11.3％（23/203）的分娩中。每次妊娠的产妇死亡率为5.7%。
结论：患有vEDS的女性子宫破裂的风险升高，血管事件,怀孕期间的消化事件和死亡。妇女在围产期似乎是最危险的；为了避免驱逐努力，剖腹产应安排在妊娠37周。
