%0 Journal Article
%T Vascular Ehlers-Danlos syndrome and pregnancy: A systematic review.
%A Haem T
%A Benson B
%A Dernoncourt A
%A Gondry J
%A Schmidt J
%A Foulon A
%J BJOG
%V 0
%N 0
%D 2024 Jun 26
%M 38926786
%F 7.331
%R 10.1111/1471-0528.17893
%X <B>BACKGROUND: </B>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.<BR><B>OBJECTIVE: </B>To systematically review the literature data on the complications in pregnancy associated with vEDS.<BR><B>METHODS: </B>We searched the Pubmed Medline and Embase databases for articles using the following terms "vascular Ehlers-Danlos syndrome" or "vEDS" AND "pregnancy".<BR><B>METHODS: </B>Women with vEDS.<BR><B>METHODS: </B>We searched the PubMed® MEDLINE® database for publications evaluating obstetric outcomes in women with vEDS.<BR><B>RESULTS: </B>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%.<BR><B>CONCLUSIONS: </B>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.