( BJOG . 2024;131:1620-1629. doi: 10.1111/1471-0528.17893) The prevalence of Ehlers-Danlos syndrome (EDS) is ∼1 in 5000, and it is characterized by joint hyperlaxity, high skin elasticity, and fragile connective tissue. There are 13 known subtypes, and the most frequently occurring of these are classic EDS, hypermobile EDS, and vascular EDS (vEDS). vEDS is an autosomal dominant disease, most commonly associated with the COL3A1 mutation and less commonly with COL1A1, with an incidence ranging from 1 to 9 in 100,000, and potential for life-threatening complications. Severe complications can include spontaneous colon perforation, uterine rupture during pregnancy, severe perineal tears, arterial rupture before the age of 40, and the formation of a carotid cavernous sinus fistula, among others. Thus, pregnancy in the context of vEDS is challenging, and patients with vEDS are at higher risk of complications, especially in the peripartum period. This has led to recommendations to avoid pregnancy in patients with vEDS. This study was a systematic review of published literature surrounding pregnancy complications in patients with vEDS.
Haem et al. (2025) studied this question.