Vascular Ehlers-Danlos syndrome and pregnancy: A systematic review.

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.

OBJECTIVES

To systematically review the literature data on the complications in pregnancy associated with vEDS.

SEARCH STRATEGY: We searched the Pubmed Medline and Embase databases for articles using the following terms "vascular Ehlers-Danlos syndrome" or "vEDS" AND "pregnancy".

SELECTION CRITERIA: Women with vEDS.

DATA COLLECTION AND ANALYSIS: We searched the PubMed® MEDLINE® database for publications evaluating obstetric outcomes in women with vEDS.

MAIN

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.