Ehlers-Danlos Syndrome in Pregnancy: a Review
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Jungwoo Kang, Moghees Hanif, Eushaa Mirza, Shazia Jaleel
2020
unpublished
Ehlers-Danlos syndrome (EDS) is a group of connective tissue disorders that can result in a range of complications during pregnancy. Pregnant EDS patients generally have a favourable outcome, but those with vascular EDS are more likely to suffer from severe maternal complications. Early diagnosis of EDS and subtype characterization can aid in pre-pregnancy counselling, planning of antenatal care, risk assessment of obstetric and neonatal complications, and influence both obstetric and
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... c management of these patients. This piece aims to outline the obstetric implications of classical, hypermobile, and vascular EDS, and review the current literature regarding their optimal obstetric management. TWEETABLE ABSTRACT EDS affects obstetric management, especially in vEDS. Evidence is limited, but recommendations are provided. KEYWORDS Ehlers Danlos Syndrome Medical conditions in pregnancy Genetic conditions in pregnancy FUNDING STATEMENT The authors declare no funding was provided for the publication of this manuscript. BACKGROUND Ehlers-Danlos syndrome (EDS) is the name given to a group of monogenic conditions with variable systemic manifestations that predominantly affect the skin, joints, ligaments, vasculature, and internal organs. 1 Common clinical features among different types of EDS include joint hypermobility, frequent joint dislocations, and skin hyperextensibility. Although various mutations and types of EDS exist, most forms of EDS result from a genetic mutation in collagen proteins or enzymes involved in collagen biosynthesis or organisation. 2 Inheritance is usually autosomal dominant, with de novo mutations being relatively common, and estimates of prevalence range from 1 in 20,000 to nearly 10 in 5,000. [3] [4] [5] Considering the multisystemic effects of EDS on the body, it is not surprising that the condition complicates pregnancy and delivery. A recent population-based retrospective study examining EDS in pregnancy suggests that the overall prevalence is 7 per 100,000 births on average during the period studied, with prevalence increasing every year -likely due to recent advances in EDS classification and genetic techniques. 6 In this cohort examining 1,042 pregnant patients with EDS, pregnancy in EDS was associated with intrauterine
doi:10.22541/au.159818964.40990743
fatcat:x4bfgps32vcnzlpv5pj2jdhdwq